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Malignant hyperthermia (MH) is a potentially fatal disorder triggered by volatile anesthetics or succinylcholine, inducing a hypermetabolic crisis in susceptible patients. The caffeine-halothane contracture test (CHCT) remains a gold standard for MH detection. The authors describe a pregnant patient with a history of exertional rhabdomyolysis, who required urgent MH screening for administration of MH-triggering anesthetics. This anesthetic plan allowed for an in utero myelomeningocele repair for a 24-week-old fetus. A multi-institutional team coordinated the urgent CHCT and repair with a favorable outcome., Competing Interests: The authors declare no Conflicts of Interest., (Copyright © 2024 International Anesthesia Research Society.)

Background: This study aims to present lateral sacral artery perforator (LSAP) flaps as a new option for myelomeningocele reconstruction and to discuss their advantages and disadvantages., Methods: Eleven patients were included in the study, and reconstruction was performed with 22 LSAP flaps. Patients were evaluated in age, gender, birth weight, kyphosis status, defect localization and size, flap size, operation time, amount of bleeding, and postoperative complications., Results: Sixteen flaps were raised from the first and 6 from the second LSA. The average time for soft tissue reconstruction was 57 minutes. The mean blood loss was 11.1 mL/kg. No cerebrospinal fluid leakage was detected in any patient. In 1 of the 22 flaps, venous congestion did not completely resolved and resulted in partial necrosis secondary to venous insufficiency. In 2 patients, minimal wound dehiscence was detected in the distal part of the flap. No wound infections, hematomas, donor site complications, or seromas were observed in any patient., Conclusions: Considering that myelomeningoceles are often located in the lumbar region, we think that LSAP flaps will be a new option among other flaps. Additionally, if other flaps are used in the neonatal period, it may be a good alternative for pressure sore reconstruction in the kyphotic area., Competing Interests: Conflicts of interest and sources of funding: none declared., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

Objective: This integrative literature review aims to discuss the benefits and limitations of postnatal surgery to correct myelomeningocele using the placenta as an autologous graft used on the lesion; in addition, it seeks to highlight the placental properties and the benefits and indications of surgery., Methods: For this production, the PRISMA criteria were used. PubMed was used as a database on October 19, 2023, and three searches were made, all using the words "myelomeningocele" and "surgery" varying only between "amnion," "placenta," and "chorion" as the third word. A total of 91 articles were found, and after analysis of duplicates and inclusion and exclusion criteria, only 11 articles were used in this systematic review., Results: Properties of the amniotic membrane were observed, such as anti-inflammatory, stimulation of native tissue growth, regenerating action by the secretion of neutrophil factors, promotion of epithelialization, inhibition of fibrosis and healing, and antibacterial effect. It is observed that there are benefits in using the placenta as an autograft and there are contradictions between the periods of surgery., Conclusion: Due to its properties that accelerate healing, the absence of the possibility of rejection, and its easy access make this choice more frequently adopted. We are confident in saying that the effectiveness of the amniotic membrane is widely reliable., Competing Interests: Declarations. Conflict of interest: The authors declare no competing interests., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Terminal myelocystocele (TMC) is a rare form of spinal dysraphism which arises due to aberration in the secondary neurulation process involving the caudal cell mass. Terminal myelocystocele has been defined by Pang et al. based on essential and non-essential features. One of the non-essential features includes non dysraphic lipomas which do not tether to the neural placode. We are presenting two cases which meets all the essential criteria outlined by Pang et al. for TMC but also show the presence of a lipomatous component tethering to the neural placode, similar to a dysraphic lipoma. Through this article, we want to showcase a subset which represents "true" terminal lipomyelocystocele (TLMC), bridging the spectrum of spinal dysraphism between TMC and lipomyelomeningocele (LMM)., Competing Interests: Declarations. Competing interests: The authors declare no competing interests. Conflict of interest: None, (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Introduction: Since the Management of Myelomeningocele Study (MOMS) publication in 2011 we have designed a prospective protocol for surveillance of neurogenic bladder according to the Leal da Cruz categorization system (J Urol, 2015) that guides us on treatment. Our institution was the precursor of in-utero myelomeningocele (MMC) in Latin America, so our database was biased mostly for patients that underwent fetal surgery for MMC closure. We have demonstrated that in-utero MMC closure does not improve bladder function in opposition to the data from the urological branch of the MOMS study (10), but our control group was based on a historical cohort, before the onset of in-utero MMC repair in our division (15) Since 2018, we have identified an increasing number of referrals of postnatal operated MMC patients to our group just for urological follow up. We decided then to start this prospective protocol for all neurogenic patients and provide a contemporary database to record differences in early bladder function, presence of hydronephrosis and vesicoureteral reflux, treatment, initial outcome and indication of surgery among three mains groups (in-utero MMC repair, post-natal repair and miscellaneous other cases of neurogenic bladder). We want to present preliminary data of this cohort in the period of time 2018 to 2023 (5 years). There was not an exclusion criteria, all patients with neuropathic bladder were included in this study., Materials and Methods: We evaluated our database of all neurogenic bladder (NB) patients aged <1 year who started urological treatment in our institution from 2018. We evaluated diagnosis, age at first visit, clinical data, prevalence of hydronephrosis and (vesicoureteral reflux) VUR, bladder pattern according to the Leal da Cruz categorization system (1), treatment, time of follow-up, number of clinical visits and (urodynamic evaluation) UE performed, final bladder status and surgeries performed., Results: We identified 43 were aged <1 year with a mean age of 4.5 months (median 3.5) at first urological appointment. Diagnosis was myelomeningocele in 33 patients and miscellaneous in 10. From the MMC group, 24 were operated in-utero and 9 post-natal. The initial bladder pattern in the whole group showed 23 (53.5%) high risk, 11 normal (25.6%), 5 underactive bladder (11.6%) and 4 incontinent (9.3%). Mean follow-up was 24 months, mean age at last UE (cases with minimum of 2): 37 months, mean UE per patient: 2. At present, 28 patients perform clean intermittent catheterization (CIC), 23 with anticholinergics and 15 are only under surveillance. VUR was seen in 11/43 cases: 25.6%. Urological surgery has been performed in 4 patients: bladder augmentation in 3 and vesicostomy in 1. Bladder categorization for high-risk and normal patterns were respectively 62.5% and 25% for in utero and 44.4% and 22.2% for postnatal repair and detailed statistical analysis did not present statistical significance of in utero and postnatal groups., Conclusion: Our study can conclude in a contemporary prospective study the findings of elevated incidence of high-risk patterns irrespectively of in-utero or post-natal repair (62.5% and 44.4%). We acknowledge that even though this is not new information and besides the still limited follow up, this cohort is one of the very few that follow and compare in one single institution with comparable UE for in-utero and post-natal MMC repair population, having also a miscellaneous group of NB as a pictorial report of also a similar initial distribution of bladder patterns. We plan to report in the future in a larger cohort the continuation of this study., (Copyright © 2024. Published by Elsevier Ltd.)

Background: The aim of this study was to assess the safety and efficacy of human umbilical cord mesenchymal stromal cells (hUC-MSCs) patch used as an adjuvant therapy in fetal myelomeningocele (MMC) surgery in the ovine model., Methods: hUC-MSCs were isolated from human umbilical cords (UC) using the explant method, cultured and characterized. hUC-MSCs were then embedded in a fibrin patch. MMC were surgically created at 75 days of gestation and repaired at 89 days of gestation in sheep fetuses. Two groups were compared: the hUC-MSCs group in which MMC was repaired using a cellular patch and the control group, in which MMC was repaired using an acellular patch. Safety was evaluated by clinical ewes' monitoring during gestation, and clinical and histological examinations of lambs after birth. Efficacy was assessed by clinical neurological evaluation at 2 and 24 h of life using the sheep locomotor rating scale and by histological analyses., Results: Among the 17 operated lambs, nine were born alive: six in the hUC-MSCs group and three in the control group. Overall fetal loss was 47% (8/17) without differences between the two groups. No fever was reported in ewes. No tumors were detected in clinical and histological examinations in the lambs. At 24 h of life, mean Sheep Locomotor Rating score was higher in the hUC-MSCs group than in the control group: 15.0 versus 2.0 (p = 0.07). Histological analyses showed a higher large neurons density in the hUC-MSCs group in comparison with the control group: 9.9 versus 6.3/mm 2 of gray matter (p = 0.04). Lambs in the hUC-MSCs group had lower fibrosis around the spinal cord and at the level of the MMC scar: 70.9 versus 253.7 μm (p = 0.10) and 691.3 versus 1684.4 μm (p = 0,18), respectively., Conclusions: Ovine fetal repair of MMC using human UC-MSCs seems to be an effective and safe procedure., Competing Interests: Declarations. Ethics approval and consent to participate: The study was approved the 6th of July, 2020, by the French National Committee on Animal Research (APAFIS#25589-2020052810367317 v4). Title: “Myéloméningocèle: évaluation du bénéfice d’un patch de cellules souches mésenchymateuses dans le traitement anténatal”. Women gave written consent for the use of their umbilical cord for research. Consent for publication: All authors confirm their consent for publication. Competing interests: The authors declare that they have no competing interests. Disclosure of AI: The authors declare that they have not used Artificial Intelligence in this study., (© 2024. The Author(s).)

Objective: Objective: Myelomeningocele (MMC) is the most severe type of spina bifida, with an incidence of 1.87 per 10,000 live births in Taiwan. Exposure of the lesion to amniotic fluid exacerbates neurological outcomes, while fetal surgery for MMC repair, now a routine practice, improves postnatal outcomes. However, Asian women and their families often find it difficult to accept prenatal defects, leading nearly all pregnancies with fetal MMC to opt for termination without considering fetal surgery., Materials and Methods: In Taiwan's first approved trial of fetoscopic MMC repair, we prospectively recruited 15 cases from 2020 to 2023. Final diagnoses were confirmed using MRI and ultrasound. The medical team provided non-direct consultations to discuss possible outcomes of fetal surgery with family members. For those opting for fetal surgery, we offered total percutaneous fetoscopic MMC repair., Results: Over 30 months, 14 of 15 cases (93%) chose to terminate their pregnancies between 18 and 26 weeks of gestational age. Decision factors included potential disabilities, morbidities, economic, social, and psychological aspects. Despite supportive groups in the country, the termination rate remained high among the Chinese population. One out of the 15 cases underwent fetal surgery successfully, resulting in a 30-month-old child without motor function delays, able to walk and run naturally., Conclusion: We initiated the first fetoscopic MMC repair in Taiwan with promising outcomes, though we faced a high termination rate here and similar situation in other Asian countries. Continuous social education through media could play a crucial role in changing perceptions and increasing acceptance of fetal surgery., Competing Interests: Conflicts of interest All authors declare no conflicts of interest regarding this study., (Copyright © 2024. Published by Elsevier B.V.)

Objective: Despite advances, myelomeningocele remains a major cause of mortality and disability. This study aims to analyze factors influencing mortality and suggest ways to reduce it., Methods: We reviewed 173 patients who underwent surgery for myelomeningocele from January 2010 to December 2021. Variables affecting mortality were categorized into patient-related and indirectly related factors. Data were collected through patient file reviews and phone interviews with parents., Results: Mortality is influenced by multiple factors: female gender (3.2-fold increase), paraplegia (3.1-fold increase), absence of tethered cord release surgery (9.4-fold increase), scoliosis (4.2-fold increase), and renal failure (5.28-fold increase). Defect size and father's education level also significantly impact mortality. The overall mortality rate was 20.8 %, with hydrocephalus being the leading cause., Conclusion: Mortality and disability in myelomeningocele patients remain high, with over 50 % of deaths being preventable. Effective management could significantly improve mortality rates. Long-term studies are crucial for advancing research in this field., Competing Interests: Declaration of Competing Interest None., (Copyright © 2024 Elsevier B.V. All rights reserved.)

Background: Myelomeningocele (MMC) is a severe form of spina bifida characterized by spinal cord extrusion into a cerebrospinal fluid (CSF) filled sac which may lead to lifelong disability. Repair of these lesions have classically occurred shortly after birth, but more recently, prenatal myelomeningocele repair techniques have been elucidated. This study aimed to investigate the outcome of surgery, particularly with subsequent spinal cord tethering, in patients with prenatal myelomeningocele closure and those with postnatal repair surgery., Materials and Methods: In this retrospective study which assessed patients from April 2002 to April 2020, the data of 18 total patients with MMC were reviewed. Nine patients from each group were included, closed prenatally or postnatally, respectively. Demographic information including age and sex, birth week, infant comorbidities, presence of dermoid or lipoma, cutaneous stigmata, total number and timing of detethering procedures, presence of Chiari malformation, and need for VPS was obtained., Results: Eighteen patients including nine infants closed prenatally and nine infants with myelomeningoceles closed postnatally were reviewed. The mean age was 4 ± 3 years and 6.22 ± 2.4 in prenatal and postnatal retrospectively. There was a significant relationship between the number of detethering procedures (p-value = 0.03) and the need for a ventriculoperitoneal shunt (VPS) (p-value = 0.01) between the groups, with the prenatal closure group having lower rates of each. There was no significant difference between the groups in regard to the mean age at the detethering procedure (p = 0.4), sex (p = 0.09), birth week (p = 0.8), comorbidities (p = 0.8), presence of intraspinal dermoid or lipoma (p = 0.09), presence of cutaneous stigma (p = 0.08), Chiari (p = 0.6), fatty filum (p = 0.08), syrinx (p = 0.4), bone anomaly (p = 0.4), and spina bifida neurological scale (p = 0.66)., Conclusion: There was a significant relationship between the two groups in terms of the number of detethering procedures, and the need for VPS. Our data represents a possible difference in the need for detethering surgeries and ventriculoperitoneal shunt placements between patients with prenatal and postnatal myelomeningocele closures., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: Spontaneous regression of Chiari malformation type 2 (CM2) is observed rarely, as CM2 is associated with meningomyelocele (MMC) that is surgically repaired either pre- or early postnatally. While the radiological regression of CM2 occurs frequently following prenatal repair of MMC, it has been reported in only a few studies after postnatal repair., Methods: From the consecutive series of children with postnatally repaired MMC, we reviewed the clinical and radiological data regarding CM2, particularly its regression either spontaneously or following CSF diversion., Results: Eighteen children underwent postnatal repair of MMC between February 2011 and April 2023. CM2 was present in 16 (89%), and hydrocephalus in 15 children (83%), requiring shunting in 14 of them. During the mean clinical observation time (from birth to April 2023) of 59 ± 51 months, three children with CM2 (19%) underwent 1-2 foramen magnum decompressions (FMD), five children (28%) 1-4 surgical untethering procedures and 13 children with shunted hydrocephalus (93%) 1-5 shunt revisions. Out of sixteen children with CM2, we observed regression of CM2 on MRI in only one case (6%) during the mean radiological follow-up (from birth to the last MRI taken) of 49 ± 51 months., Conclusion: In our experience, spontaneous regression of CM2 in children with postnatally repaired MMC occurs quite rarely. Pathophysiological mechanisms behind the development of CM2 in children with MMC remain unclear, but our observation supports the hypothesis of an association between the downward displacement of the hindbrain and the low intraspinal pressure secondary to CSF leakage in children born with MMC., (© 2024. The Author(s).)

Purpose: Myelomeningocele (MMC) is a prevalent neural tube closure defect often associated with hydrocephalus, necessitating surgical intervention in a significant proportion of cases. While ventriculoperitoneal shunting (VPS) has been a standard treatment approach, endoscopic third ventriculostomy (ETV) has emerged as a promising alternative. However, factors influencing the success of ETV in MMC patients remain uncertain. This retrospective observational study aimed to identify clinical and radiological factors correlating with a higher success rate of ETV in MMC patients., Methods: Medical records of MMC patients who underwent ETV at a tertiary care center between 2015 and 2021 were reviewed. Demographic, clinical, and radiological data were analyzed. ETV success was defined as the absence of further hydrocephalus treatment during follow-up., Results: Of 131 MMC patients, 21 met inclusion criteria and underwent ETV. The overall success rate of ETV was 57.1%, with a six-month success rate of 61.9%. Age ≤ 6 months was significantly associated with lower ETV success (25%) compared to older patients (76.9%) (OR: 0.1; 95% CI 0.005-2.006; p = 0.019). Radiological factors, including posterior fossa dimensions and linear indices, did not exhibit statistically significant associations with ETV success., Conclusion: Age emerged as a significant factor affecting ETV success in MMC patients, with younger patients exhibiting lower success rates. Radiological variables did not significantly influence ETV outcomes in this study. Identifying predictors of ETV success in MMC patients is crucial for optimizing treatment strategies and improving patient outcomes., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Introduction: Myelomeningocele (MMC) is the most common neural tube defect, but rarely seen in premature infants. Most centers advocate for closure of MMC within 24 h of birth. However, this is not always possible in severely premature infants. Given the rarity of this patient population, we aimed to share our institutional experience and outcomes of severely premature infants with MMC., Methods: We performed a retrospective, observational review of premature infants (≤ 32 weeks gestational age) identified through our multidisciplinary spina bifida clinic (1995-2021) and surgical logs. Descriptive statistics were compiled about this sample including timing of MMC closure and incidence of adverse events such as sepsis, CSF diversion, meningitis, and death., Results: Eight patients were identified (50% male) with MMC who were born ≤ 32 weeks gestational age. Mean gestational age of the population was 27.3 weeks (SD 3.5). Median time to MMC closure was 1.5 days (IQR = 1-80.8). Five patients were taken for surgery within the recommended 48 h of birth; 2 patients underwent significantly delayed closure (107 and 139 days); and one patient's defect epithelized without surgical intervention. Six of eight patients required permanent cerebrospinal fluid (CSF) diversion (2 patients were treated with ventriculoperitoneal shunting (VPS), three were treated with endoscopic third ventriculostomy (ETV) with choroid plexus cauterization (CPC) and 1 patient treated with ETV; mean of 3 years after birth, ranging from 1 day to 16 years). Two patients required more than one permanent CSF diversion procedure. Two patients developed sepsis (defined as meeting at least 2/4 SIRS criteria). In both cases of sepsis, patients developed signs and symptoms more than 72 h after birth. Notably, both instances of sepsis occurred unrelated to operative intervention as they occurred before permanent MMC closure. Two patients had intraventricular hemorrhage (both grade III). No patients developed meningitis (defined as positive CSF cultures) prior to MMC closure. Median follow up duration was 9.7 years. During this time epoch, 3 patients died: Two before 2 years of age of causes unrelated to surgical intervention. One of the two patients with grade III IVH died within 24 h of MMC closure., Conclusions: In our institutional experience with premature infants with MMC, some patients underwent delayed MMC closure. The overall rate of meningitis, sepsis, and mortality for preterm children with MMC was similar to MMC patients born at term., (© 2024. The Author(s).)

Objectives: Enhanced recovery after surgery (ERAS) protocols are multimodal evidence-based care plans that have been adopted for multiple surgical procedures to promote faster and better patient recovery and shorter hospitalization. This study aimed to explore whether worldwide fetal therapy centers offering prenatal myelomeningocele repair implement the ERAS principles and to provide recommendations for improved perioperative management of patients., Methods: In this survey study, a total of 53 fetal therapy centers offering prenatal surgery for open spina bifida were identified and invited to complete a digital questionnaire covering their pre-, intra- and postoperative management. An overall score was calculated per center based on compliance with 20 key ERAS principles, extrapolated from ERAS guidelines for Cesarean section, gynecological oncology and colorectal surgery. Each item was awarded a score of 1 or 0, depending, respectively, on whether the center did or did not comply with that principle, with a maximum score of 20., Results: The questionnaire was completed by 46 centers in 17 countries (response rate, 87%). In total, 22 (48%) centers performed exclusively open fetal surgery (laparotomy and hysterotomy), whereas 14 (30%) offered both open and fetoscopic procedures and 10 (22%) used only fetoscopy. The perioperative management of patients undergoing fetoscopic and open surgery was very similar. The median ERAS score was 12 (range, 8-17), with a mean ± SD of 12.5 ± 2.4. Center compliance was the highest for the use of regional anesthesia (98%), avoidance of bowel preparation (96%) and thromboprophylaxis (96%), while the lowest compliance was observed for preoperative carbohydrate loading (15%), a 2-h fasting period for clear fluids (20%), postoperative nausea and vomiting prevention (33%) and early feeding (35%). ERAS scores were similar in centers with a short (2-5 days), medium (6-10 days) and long (≥ 11 days) hospital stay (mean ± SD, 12.9 ± 2.4, 12.1 ± 2.0 and 10.3 ± 3.2, respectively, P = 0.15). Furthermore, there was no significant association between ERAS score and surgical technique or case volume., Conclusions: The perioperative management of fetal spina bifida surgery is highly variable across fetal therapy centers worldwide. Standardized protocols integrating ERAS principles may improve patient recovery, reduce maternal morbidity and shorten the hospital stay after fetal spina bifida surgery. © 2024 The Author(s). Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology., (© 2024 The Author(s). Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology.)

Objective: To assess if ventricular size before shunting is correlated with neurodevelopmental outcomes in children with postnatal myelomeningocele closure., Study Design: This retrospective review included children with postnatal surgical closure of myelomeningocele and neuropsychological testing between 2018 and 2023 at the University of California, San Francisco. Frontal-occipital horn ratio (FOHR) was measured immediately before shunt placement or on the first study that reported ventricular stability for nonshunted patients. The primary outcome was full scale IQ (FSIQ) on the Weschler Intelligence Scale. Secondary outcomes included indices of the Weschler scale, the Global Executive Composite from the Behavior Rating Inventory of Executive Function, and the general adaptive composite from the Adaptive Behavior Assessment Scale. Univariable and multivariable regression was used to determine if FOHR was correlated with neuropsychological scores., Results: Forty patients met the inclusion criteria; 26 (65%) had shunted hydrocephalus. Age at neuropsychological testing was 10.9 ± 0.6 years. FOHR was greater in the shunted group (0.64 vs 0.51; P < .001). There were no differences in neuropsychological results between shunted and nonshunted groups. On univariable analysis, greater FOHR was associated with lower FSIQ (P = .025) and lower Visual Spatial Index scores (P = .013), which remained significant on multivariable analysis after adjusting for gestational age at birth, lesion level, shunt status, and shunt revision status (P = .049 and P = .006, respectively). Separate analyses by shunt status revealed that these effects were driven by the shunted group., Conclusions: Greater FOHR before shunting was correlated with lower FSIQ and the Visual Spatial Index scores on the Weschler Intelligence Scales. Larger studies are needed to explore further the relationship between ventricle size, hydrocephalus, and neurodevelopmental outcomes., Competing Interests: Declaration of Competing Interest The authors declare no conflicts of interest. Funding: There was no funding for this study., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Objective: Myelomeningocele (MMC) is a lifelong condition requiring complex multidisciplinary management. Using the National Spina Bifida Patient Registry (NSBPR), the authors tested the association between sociodemographic variables and odds of undergoing neurosurgical procedures., Methods: The authors extracted sociodemographic, clinical, and neurosurgical procedure data on participants with MMC aged ≥ 1 year who visited an NSBPR clinic between 2009 and 2020. The zip code of the participant's residence at the time of the last spina bifida clinic visit was linked to the Distressed Communities Index (DCI) tier. Multivariate models were built to identify factors associated with undergoing CSF diversion, shunt revision, tethered cord release (TCR), and Chiari decompression., Results: There were 7924 participants with a median visit age of 13 years (IQR 7-20 years); 49.1% were male, 30.2% were non-Hispanic Black or Hispanic, 54.5% had public/supplemental insurance, and 16.9% were from distressed communities. CSF diversion, shunt revision, TCR, and Chiari decompression were performed in 81.8%, 47.7%, 22.9%, and 8.7% of participants, respectively. In multivariate analyses controlling for age, sex, insurance, DCI tier, lesion level, and surgical closure timing, Hispanic individuals were less likely than their non-Hispanic White counterparts to undergo shunt revision (p = 0.013). Non-Hispanic Black and Hispanic individuals were less likely to undergo TCR (p < 0.001 each) or Chiari decompression (p < 0.001 each). Compared with privately insured individuals, publicly insured individuals were more likely to undergo CSF diversion (p = 0.031). Those in distressed communities had increased odds of undergoing CSF diversion (p = 0.004) than those in prosperous communities., Conclusions: Among individuals with MMC participating in the NSBPR, there were differences in receiving neurosurgical procedures by race/ethnicity, insurance type, and DCI tier. Additional prospective studies are necessary to elucidate the reasons for these variations and their impact on long-term outcomes for this patient population in order to created targeted interventions.

Study Objectives: Advances in prenatal repair of myelomeningocele have improved outcomes involving different organ systems. There are limited data on respiratory outcomes following prenatal surgical repair. We hypothesize there is no difference in respiratory outcomes between patients with spina bifida who have undergone prenatal vs postnatal repair., Methods: We performed a retrospective study of 46 infants < 1 year with spina bifida seen at Children's Hospital Los Angeles from 2004-2022. Demographic data, timing of closure, neonatal course, Chiari II malformation, ventriculoperitoneal shunt, polysomnography results, and need for supplemental oxygen were collected. Unpaired t test and χ 2 test were used to analyze results., Results: A total of 31/46 had prenatal repair of myelomeningocele; average age at repair was 27 weeks postconception. Average age at postnatal repair was 37 weeks postconception. There was no difference in age at polysomnography. There was no difference in Chiari II malformation presence ( P = .61). Sixty pecent of patients with postnatal repair and 23% in the prenatal group underwent ventriculoperitoneal shunt placement ( P = .01). There was no difference in polysomnography findings between the 2 groups: central apnea index ( P = .11), obstructive apnea-hypopnea index ( P = .64), average oxygen saturation baseline ( P = .91), average oxygen saturation nadir ( P = .17), average end-tidal carbon dioxide baseline ( P = .87), and average end-tidal carbon dioxide maximum ( P = .54). There were no significant differences in the proportion of patients on supplemental oxygen ( P = .25), central sleep apnea or obstructive sleep apnea between groups., Conclusions: Patients with spina bifida who have undergone closure of neural tube defect have persistent central apneas, obstructive apneas, and significant hypoxemia. There were no differences in the frequency or severity of sleep-disordered breathing in those with prenatal repair vs postnatal repair., Citation: Stark KG, Wang RY, Smith KA. Sleep-related breathing disorders in infants with spina bifida repaired prenatally and postnatally. J Clin Sleep Med . 2024;20(10):1579-1583., (© 2024 American Academy of Sleep Medicine.)

Objective: Compared with postnatal repair, prenatal myelomeningocele (MMC) repair is associated with improved motor function and decreased need for cerebrospinal fluid (CSF) diversion. It is unknown how prenatal surgery alters neuroanatomical structures identifiable on magnetic resonance imaging (MRI). The purpose of this study was to use MRI to compare neurodevelopmental anatomy in patients undergoing fetal MMC repair compared with those undergoing postnatal repair., Methods: This was a retrospective review of neonates who underwent prenatal or postnatal MMC repair at our institution between 2016 and 2021. Imaging data, including prenatal ultrasound and pre- and postnatal MRI examinations, if available, were retrieved. We analyzed anatomical findings characteristically seen on MRI of the neuroaxis in patients with MMC and compared imaging findings between patients with prenatal vs postnatal MMC repair., Results: The study population included 61 patients who underwent surgical repair for MMC during the 6-year study period, of whom 25 underwent prenatal repair and 36 postnatal repair. CSF diversion was required in 24% of the prenatally repaired cohort vs 67% of the postnatally repaired cohort (P = 0.001). On postnatal MRI, a syrinx was present in 13% of the prenatally repaired cohort vs 42% in the postnatally repaired cohort (P = 0.02). Postnatal corpus callosal (CC) morphology was abnormal in 54% of the prenatally repaired cohort vs 53% of the postnatally repaired cohort (P = 0.92), while falx morphology was normal in 92% of the prenatally repaired cohort vs 34% of the postnatally repaired cohort (P < 0.001). On postnatal MRI, patients in the prenatally repaired cohort had a shorter tentorium-to-foramen-magnum distance compared with those in the postnatally repaired cohort (mean, 18.43 mm vs 22.42 mm; P = 0.01), a larger foramen magnum diameter (mean, 22.87 mm vs 18.94 mm; P < 0.001) and a smaller degree of hindbrain herniation (mean, 1.53 mm vs 8.72 mm; P < 0.001). The cerebral aqueduct was patent in 79% of the prenatally repaired cohort vs 100% of the postnatally repaired cohort on postnatal MRI (P = 0.008). Between the two cohorts, at postnatal MRI there was no significant difference in the presence of gray-matter heterotopia, presence of the septum pellucidum or size of the massa intermedia., Conclusions: We report variations in developmental neuroanatomy in patients with MMC, including rates of CC dysgenesis, gray-matter heterotopia and additional cranial and spinal MRI findings. Compared to postnatal surgery, prenatal surgery is associated with changes to infratentorial anatomy, with minimal effect on supratentorial brain development. This information will be useful in counseling parents affected by fetal MMC and in understanding how prenatal repair of MMC affects brain development. © 2024 International Society of Ultrasound in Obstetrics and Gynecology., (© 2024 International Society of Ultrasound in Obstetrics and Gynecology.)

Introduction: Myelomeningocele (MMC) is a prevalent form of neural tube defect. Despite advancements in treatment, MMC still poses significant health risks, including complications leading to chronic disability and mortality. Identifying prognostic risk factors for early outcomes is crucial for tailored intervention strategies., Methods: This prospective study involved newborns and infants diagnosed with MMC who underwent surgery between 2020 and 2023 at Urmia University of Medical Sciences. Demographic data and surgical outcomes were collected, and participants were followed up for six months. Statistical analyses were conducted using descriptive statistics, Chi-Square, and independent t-test., Results: The study included 29 MMC cases, with an incidence rate of 1.4 per 10,000 live births. Lesions were predominantly located in the lumbar spine. Although mortality rates appeared to increase with ascending lesion sites, this trend was not statistically significant. Short-term outcomes revealed high morbidity and mortality rates, with neurological deficits being the most prevalent complication. Multivariable analysis identified head circumference as a significant predictor of adverse outcomes (IRR = 1.37, 95% CI = 1.02 to 1.86, p = 0.04). Furthermore, an increase in birth weight was associated with a reduction in the incidence of requiring a ventriculoperitoneal shunt (IRR = 0.99, 95% CI = 0.998 to 0.999, p = 0.02)., Conclusion: This prospective study highlights prognostic risk factors for early outcomes in MMC patients, emphasizing the need for personalized intervention strategies. By addressing modifiable risk factors and implementing targeted interventions, healthcare providers can strive to improve outcomes and enhance the quality of life for MMC patients., (© 2024. The Author(s).)

Introduction: Most myelomeningocele (MMC) cases present with ventriculomegaly or hydrocephalus, yet a comprehensive volumetric assessment of MMC intracranial structures is lacking. This study aimed to provide baseline data on intracranial structural volumes immediately after birth in MMC infants who underwent repair surgeries after birth (postnatal repair)., Methods: In this retrospective single-center study, we analyzed 52 MMC infants undergoing postnatal repair, utilizing head computed tomography scans at birth for volumetric assessment. Intracranial volume (ICV), lateral ventricles volume (LVV), choroid plexus volume (CPV), and posterior cranial fossa volume (PCFV) were measured. Hydrocephalus was classified into no hydrocephalus, progressive hydrocephalus, and hydrocephalus at birth. Comparative analysis employed the Wilcoxon rank-sum test. Receiver operating characteristic (ROC) analysis discriminated cases with and without ventriculoperitoneal shunt (VPS)., Results: The median values were 407.50 mL for ICV, 33.18 mL for LVV, 0.67 mL for CPV, and 21.35 mL for PCFV. Thirty-seven cases (71.15%) underwent VPS. ROC analysis revealed an LVV cut-off value of 6.74 mL for discriminating cases with and without VPS. Progressive hydrocephalus showed no significant difference in ICV but significantly larger LVV compared to no hydrocephalus. Hydrocephalus at birth demonstrated statistically larger ICV and LVV compared to the other two types., Conclusion: Baseline volumetric data were provided, and volumetric analysis exhibited statistical differences among three hydrocephalus types. These findings enhance our understanding of intracranial volumetric changes in MMC, facilitating more objective assessments of MMC cases., (© 2024. The Author(s).)

Objective: Approximately 70%-80% of children born with myelomeningocele develop hydrocephalus and need CSF diversion, commonly a ventriculoperitoneal shunt (VPS) placement. The optimal timing of surgery is not known, but many centers delay VPS placement and perform it in a separate surgery to avoid shunt complications, mainly shunt contamination and infection. This systematic review and meta-analysis aimed to compare shunt-related complications between populations with VPS surgery performed either simultaneously with myelomeningocele closure or with delay., Methods: The authors searched MEDLINE (PubMed), Scopus, Web of Science, Cochrane Central Register of Controlled Trials, and Cochrane Database of Systematic Reviews databases on December 15, 2022, and November 11, 2023, using a predefined search strategy. Randomized and nonrandomized trials of neonates undergoing postnatal myelomeningocele closure and VPS placement before the age of 29 days were included. Cases with prenatal myelomeningocele closure and hydrocephalus treatment other than a VPS were excluded. All studies were screened and assessed by two independent reviewers. The authors performed a meta-analysis pooling risk ratios (RRs) with a 95% CI using the random-effects model. The quality of studies was assessed using the Newcastle-Ottawa Scale., Results: After screening and a full-text review of 2099 database search results, 12 studies with a total number of 4894 patients were included. All studies were nonrandomized studies. Only 2 studies were ranked as good quality on the Newcastle-Ottawa Scale. No statistically significant differences were found between simultaneous and delayed shunt insertion in terms of shunt infection (RR 0.77, 95% CI 0.41-1.42); shunt revision (RR 0.49, 95% CI 0.19-1.30); overall mortality (RR 0.87, 95% CI 0.09-8.57); wound CSF leak (RR 0.20, 95% CI 0.03-1.23); or myelomeningocele wound dehiscence (RR 0.52, 95% CI 0.07-3.71). In the subgroup analysis of studies conducted in high-income countries, simultaneous shunting was superior to delayed shunting concerning shunt infection (RR 0.49, 95% CI 0.31-0.78) and shunt revision (RR 0.30, 95% CI 0.09-0.95)., Conclusions: This systematic review and meta-analysis found no statistically significant differences in shunt-related complications between simultaneous and delayed VPS surgery in myelomeningocele-related hydrocephalus in neonates. The current literature does not support the common practice of delayed shunting or its alternative, simultaneous shunting.

A mature cystic teratoma is a mass with heterogeneous appearance, consisting of adult tissue with two or three layers: endoderm, mesoderm, and ectoderm. It is a rare, benign transformation of somatic tissue most commonly found in the sacrococcygeal region and may resemble an uncomplicated spina bifida on prenatal ultrasonography. In this case report, we describe a female newborn with an extremely rare mature cystic teratoma in the thoracolumbar region. She presented prenatally with a preliminary diagnosis of meningomyelocele, diastematomyelia, and Chiari II malformation and a possible teratoma. However, a mass containing solid glandular tissues and bony calcifications approximately 3 × 4 cm in size was observed in the thoracolumbar region upon birth. During surgical resection, no nerve roots were found in the associated meningocele. The patient retained full lower body function postoperatively following surgical excision of the thecal sac and teratoma., (© 2024. The Author(s).)

Purpose: Historically, the presence of gray matter heterotopia was a concern for adverse postnatal neurocognitive status in patients undergoing fetal closure of open spinal dysraphism. The purpose of this study was to evaluate neurodevelopmental outcomes and the onset of seizures during early childhood in patients with a prenatal diagnosis of myelomeningocele/myeloschisis (MMC) and periventricular nodular heterotopia (PVNH)., Methods: All patients evaluated at the Center for Fetal Diagnosis and Treatment with a diagnosis of MMC between June 2016 to March 2023 were identified. PVNH was determined from prenatal and/or postnatal MRI. The Bayley Scales of Infant and Toddler Development (edition III or IV) were used for neurodevelopmental assessments. Patients were screened for seizures/epilepsy., Results: Of 497 patients evaluated with a prenatal diagnosis of MMC, 99 were found to have PVNH on prenatal MRI, of which 35 had confirmed PVNH on postnatal imaging. From the 497 patients, 398 initially did not exhibit heterotopia on prenatal MRI, but 47 of these then had confirmed postnatal PVNH. The presence of PVNH was not a significant risk factor for postnatal seizures in early childhood. The average neurodevelopmental scores were not significantly different among heterotopia groups for cognitive, language, and motor domains., Conclusion: The presence of PVNH in patients with a prenatal diagnosis of MMC does not indicate an increased risk for neurodevelopmental delay at 1 year of age. We did not demonstrate an association with seizures/epilepsy. These findings can aid clinicians in prenatal consultation regarding fetal repair of open spinal dysraphism. Long-term follow-up is required to discern the true association between PVNH seen on prenatal imaging and postnatal seizures/epilepsy and neurodevelopmental outcomes., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Meningomyelocele is a common congenital condition and its reconstruction poses a challenge for surgeons. The dorsal intercostal artery (DICA) flap offers a one-stage tension-free closure with adequate results. This study, spanning from January 2019 to September 2022, analyses the outcome of nine DICA flaps for meningomyelocele reconstruction, where the average size of the DICA flap was 6.8 x 4.6 cm for an average defect of 6.33 x 4 cm. Notably, no post-operative blood transfusion was required, nor any complications occurred except for one patient's septic shock-related death. Two had post-operative cerebrospinal fluid (CSF) leak, repaired primarily with one requiring VP shunt. Based on our experience, the DICA flap, with its consistent anatomy, is a reliable option for the reconstruction of meningomyelocele defects.

To discern the efficacy of simultaneous versus delayed VPS surgery in managing hydrocephalus linked with MMC repair: The debate over the concurrent or deferred placement of ventriculoperitoneal shunts (VPS) during myelomeningocele (MMC) repair in hydrocephalic neonates necessitates a nuanced evaluation of associated risks and benefits. While VPS placement can mitigate cerebrospinal fluid (CSF) leaks and minimize wound dehiscence post-MMC repair, it concurrently introduces potential hazards such as infections and shunt-related malfunctions. This prospective cohort study focused on144 newborns with spinal myelomeningocele and hydrocephalus. Divided into two groups based on the timing of dysraphism repair and VPS placement, 101 children underwent concurrent procedures, while 43 received deferred VPS insertion post-MMC closure. Female patients constituted 60% of the cohort, with lumbar lesions being predominant. The median age for MMC closure was three days. Analysis revealed that the deferred insertion group exhibited higher rates of shunt malfunctions, CSF leaks, and wound dehiscence compared to the concurrent insertion group. Although indications hinted at a potential increase in shunt infections in the immediate insertion group, statistical significance was lacking. The study established a statistically significant association between the timing of shunt insertion during MMC repair and specific outcomes, such as CSF leaks and wound dehiscence. The findings suggest that concurrent shunt insertion during MMC repair may reduce the incidence of these complications compared to deferred insertion. However, no substantial differences emerged in terms of shunt infection and malfunction, emphasizing the persistent challenges associated with these major complications., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Objective: Reports on the management and survival of children with myelomeningocele defects in Bangladesh are limited. This study describes the characteristics and outcomes of these children, focusing on the timing of surgical repair and factors affecting survival., Methods: We enrolled patients with myelomeningoceles in a case-control study on arsenic exposure and spina bifida in Bangladesh. Cases were subsequently followed at regular intervals to assess survival. Demographic, clinical, and surgical characteristics were reviewed. Univariate tests identified factors affecting survival., Results: Between 2016 and 2022, we enrolled 272 patients with myelomeningocele. Postnatal surgical repair was performed in 63% of cases. However, surgery within 5 days after birth was infrequent (<10%) due to delayed presentation, and there was a high rate (29%) of preoperative deaths. Surgical repair significantly improved patient survival (P < 0.0001). Older age at time of surgery was also associated with improved survival rates, which most likely represents that those who survived to older ages prior to surgery accommodated better with their lesions. Patients who presented with ruptured lesions had lower survival rates., Conclusions: Timely neurosurgical repair of myelomeningoceles in Bangladesh is hindered by late patient presentation, resulting in a high preoperative patient death rate. Neurosurgical intervention remains a significant predictor of survival. Increased access to neurosurgical care and education of families and non-neurosurgical providers on the need for timely surgical intervention are important for improving the survival of infants with myelomeningoceles., (Copyright © 2024 Elsevier Inc. All rights reserved.)

After myelomeningocele (MMC) repair, a secondary tethered spinal cord occurs in almost all patients. The tethered spinal cord may result in progressive neurological deterioration and walking disability. This retrospective cohort study aimed to highlight the walking recovery one year after tethered cord release and its relation to the preoperative conus level. We reviewed the medical records at our university hospital from January 2014 to December 2022. The patients who underwent spinal cord untethering following lumbosacral MMC repair were included. We assessed the walking recovery one year after cord release using the modified Benzel scale. Thirty-seven patients met our selection criteria. There were 19 girls (51.4%) and 18 boys (48.6%). Their mean age at presentation was 8.6 years. The preoperative conus vertebral levels ranged between L4 and S3. One year after spinal cord release, 37.8% of the patients regained their walking ability. All the patients whose preoperative conus level was at S2 or S3 regained their walking ability. In contrast, all the patients with preoperative conus levels at L4 or L5 didn't regain their ability to walk. One-third (33.3%) of patients whose conus was at the S1 level regained their walking ability one year after cord release. One year after tethered cord release, 37.8% of the patients regained their walking ability. We found that the walking recovery was statistically associated with the preoperative conus level. A multicenter prospective study is required to support the results of this study., (© 2024. The Author(s).)

Objective: The aim of this study was to compare clinical and craniometric outcomes of patients treated for hydrocephalus following fetal myelomeningocele repair (fMMR) via a ventriculoperitoneal shunt (VPS) or endoscopic third ventriculostomy with choroid plexus cauterization (ETV/CPC)., Methods: This was a retrospective cohort study of children who were treated for hydrocephalus following fMMR via VPS or ETV with or without CPC (ETV ± CPC) at Vanderbilt between 2012 and 2021. The primary outcomes were treatment failure and time to failure (TTF). Secondary outcomes included changes in hydrocephalus metrics (fronto-occipital horn ratio [FOHR] and head circumference measurements) and healthcare resource utilization (number of hospital admissions, clinic visits, and neuroimaging findings)., Results: Among 88 patients who underwent fMMR, 37 (42%) required permanent CSF diversion, of whom 19 received treatment at the authors' institution. Twelve patients underwent ETV ± CPC, and 7 underwent VPS placement at a median corrected age of 23 weeks versus 1 week (p = 0.002). The preoperative median head circumference percentiles and z-scores for patients in the ETV ± CPC cohort were similar to those of the VPS cohort (percentiles: 98.5 vs 94.0, p = 0.064; z-scores: 2.32 vs 1.60, p = 0.111). There was no difference in preoperative median FOHR measurements between the two cohorts (0.57 vs 0.59, p = 0.53). At 6 months postoperatively, the median head circumference percentile and z-score for the ETV ± CPC cohort remained similar between the two cohorts (percentiles: 98.0 vs 67.5, p = 0.315; z-scores: 2.12 vs 0.52, p = 0.307). There was no difference in the change in FOHR (-0.06 vs -0.09, p = 0.37) and change in head circumference percentile (-1.33 vs -28.6, p = 0.058) between the cohorts 6 months after the index CSF diversion procedure. One patient in the ETV ± CPC cohort experienced a seizure and a nonoperative subdural hemorrhage postoperatively; no other complications were observed. Six of the 7 patients in the VPS cohort required shunt revision with a median TTF of 9.8 months while 2 of the 12 ETV ± CPC patients required a repeat ETV at a median of 17.5 months (86% vs 17%, p = 0.013). The median number of hydrocephalus-related hospital readmissions was significantly lower in the ETV ± CPC cohort than in the VPS cohort (0 vs 1, p = 0.006). The ETV ± CPC cohort had fewer CT scans (0 vs 2, p = 0.004) and radiographs (0 vs 2, p < 0.001) than the VPS cohort., Conclusions: In a single-center cohort, hydrocephalic fMMR patients treated via ETV ± CPC remained shunt free, while a majority of patients receiving an upfront shunt required revision. This is the first study comparing ETV ± CPC with VPS in the fMMR hydrocephalus population. While larger, multicenter studies are needed, these results suggest that ETV/CPC may be a preferred means of CSF diversion following fMMR.

Objective: The aim of this study was to understand the dynamics of families with children with myelomeningocele undergoing intrauterine fetal surgery., Methods: A retrospective cohort pilot study was carried out with 11 mothers of children who had undergone intrauterine myelomeningocele repair. Participants in this study responded to an electronic questionnaire (via Google Forms), developed by the study authors, that consisted of 22 multiple-choice questions, of which 17 were closed-ended and 5 had a standardized format., Results: The mean (± standard deviation) of the mothers' age was 37.6 (± 3.5) years. The median of gestational age at delivery and birthweight were 34.9 (range, 33 to 36.1) weeks and 2,300 (range, 1,950 to 2,763) g, respectively. The majority of mothers were white (81.8%), had university degree (81.8%), were Catholic (63.6%), and were married (100%). The majority of mothers rated their relationship with their husband, family, and friends as excellent (54.5, 72.7, and 54.5%, respectively). All 11 mothers reported that the newborn with myelomeningocele was born <37 weeks gestation and the birthweight most often<2,500 g. Approximately 64% of the mothers reported that their child required adaptations or had special needs, of which walking aids (50%) and bladder control (50%) were the most common ones., Conclusion: Telemedicine proved to be a useful tool in the long-term follow-up of children who underwent intrauterine surgery to correct myelomeningocele.

Background: Myelocele is a rare form of open spina bifida. Surgical repair is recommended prenatally or in the first 48 h. In some cases, the repair may be delayed, and specific surgical factors need to be considered., Method: We give a brief overview of the surgical anatomy, followed by a description of the surgical repair of a thoracolumbar Myelocele in an 11-month-old child., Conclusion: Surgical repair of the Myelocele stabilizes the neurological status, prevents local and central nervous system infections. The understanding of Myelocele anatomy enables its removal while preserving as much healthy tissue as possible and restoring normal anatomy., (© 2024. The Author(s).)

Background: Neural tube defects (NTDs) account for the largest proportion of congenital anomalies of the central nervous system and result from failure of the neural tube to close spontaneously between the 3rd and 4th weeks of in utero development. Prognosis and treatment outcome depends on the nature and the pattern of the defect. The nature of treatment outcomes and its pattern associated with grave prognosis is not well known in the study area., Objective: The aim of study was to determine the patterns and short term neurosurgical management outcomes of newborns with neural tube defects admitted at Felege Hiwot Specialized Hospital., Methods: Institutional based retrospective cross-sectional study among neonates, who were admitted at Felege Hiwot Specialized Hospital with neural tube defects from January 1 st to December, 30 th , 2018 was conducted. All Charts of Neonates with confirmed diagnosis of neural tube defects were included as part of the study. Trained data collectors (medical interns) supervised by trained supervisors (general practitioners) collected the data using a pretested data extraction format. Data were coded, entered and analyzed using SPSS version 23 software. Frequency and cross tabulations were used to summarize descriptive statistics of data, and tables and graphs were used for data presentation., Result: About 109 patients had complete documentation and imaging confirmed neural tube defects. Myelomeningocele was the commonest pattern 70 (64.2%). Thoracolumbar spine was the commonest site of presentation 49(45%). The most common associated impairment was hydrocephalus 37(33.9%). Forty-five (41.1%) had multiple complications. The mortality rate was 7.3%, 44% were discharged with sequalae and 36.7% were discharged without impairment. The significant causes of death were infection 66.7% and Chiari crisis 33.3%., Conclusion: Myelomeningocele was the most frequent clinical pattern of neural tube defect and thoracolumbar spine was the commonest site. Isolated neural tube defect was the commonest finding. There were multiple complications after surgery accompanied with meningitis and hydrocephalus. The mortality rate among neonates with neural tube defects was considerably high. The commonest causes of death were infection and Chiari crisis., (© 2024. The Author(s).)

Background: Myelomeningocele or spina bifida is an open neural tube defect that is characterized by protrusion of the meninges and the spinal cord through a deformity in the vertebral arch and spinous process. Myelomeningocele of post-natal tissue is well described; however, pre-natal tissue of this defect has no known previous histologic characterization. We compared the histology of different forms of pre-natal myelomeningocele and post-natal myelomeningocele tissue obtained via prenatal intrauterine and postnatal surgical repairs. Methods: Pre-and post-natal tissues from spina bifida repair surgeries were obtained from lipomyelomeningocele, myeloschisis, and myelomeningocele spina bifida defects. Tissue samples were processed for H&E and immunohistochemical staining (KRT14 and p63) to assess epidermal and dermal development. Results: Prenatal skin near the defect site develops with normal epidermal, dermal, and adnexal structures. Within the grossly cystic specimens, histology shows highly dense fibrous connective tissue with complete absence of a normal epidermal development with a lack of p63 and KRT14 expression. Conclusion: Tissues harvested from prenatal and postnatal spina bifida repair surgeries appear as normal skin near the defect site. However, cystic tissues consist of highly dense fibrous connective tissue with complete absence of normal epidermal development.

Background: Myelomeningocele (MMC) is a neural tube defect disease. Antenatal repair of fetal MMC is an alternative to postnatal repair. Many agents can be used as tocolytics during the in utero fetal repair such as β2-agonists and oxytocin receptor antagonists, with possible maternal and fetal repercussions. This study aims to compare maternal arterial blood gas analysis between terbutaline or atosiban, as tocolytic agents, during intrauterine MMC repair., Methods: Retrospective cohort study. Patients were divided into two groups depending on the main tocolytic agent used during intrauterine MMC repair: atosiban (16) or terbutaline (9). Maternal arterial blood gas samples were analyzed on three moments: post induction (baseline, before the start of tocolysis), before extubation, and two hours after the end of the surgery., Results: Twenty-five patients were included and assessed. Before extubation, the terbutaline group showed lower arterial pH (7.347 ± 0.05 vs. 7.396 ± 0.02 for atosiban, p = 0.006) and higher arterial lactate (28.33 ± 12.76 mg.dL -1 vs. 13.06 ± 6.35 mg.dL -1 , for atosiban, p = 0.001) levels., Conclusions: Patients who received terbutaline had more acidosis and higher levels of lactate, compared to those who received atosiban, during intrauterine fetal MMC repair., Competing Interests: Conflicts of competing interest The authors declare no conflicts of interest., (Copyright © 2024 Sociedade Brasileira de Anestesiologia. Published by Elsevier España S.L.U. All rights reserved.)

Objective: To investigate the effects of an adjuvant allogenic umbilical cord mesenchymal stromal cell (UC-MSC) patch applied during fetal surgery on motor and sphincter function in the ovine MMC model., Design: MMC defects were surgically created at 75 days of gestation and repaired 14 days later., Population: Ovine MMC model: fetal lambs., Methods: We compared lambs that received a UC-MSC patch with a control group of lambs that received an acellular patch., Main Outcome Measures: Clinical neurological assessment was performed at 2 and 24 hours of life and included determination of the Sheep Locomotor Rating scale (SLR), which has been validated in the ovine MMC model. Electrophysical examinations, spine scans and histological analyses were also performed., Results: Of the 13 operated lambs, nine were born alive: five had of these had received a UC-MSC patch and four an acellular patch. At 24 hours of life, lambs in the UC-MSC group had a significantly higher score (14 versus 5, P = 0.04). Amyotrophy was significantly more common in the control group (75% versus 0%, P = 0.02). All the lambs in the control group and none of those in the UC-MSC group were incontinent. No significant differences were observed between the UC-MSC and control groups in terms of the presence of spontaneous EMG activity, nerve conduction or spinal evoked potentials. In the microscopic examination, lambs in the UC-MSC group had less fibrosis between the spinal cord and the dermis (mean thickness, 453 versus 3921 μm, P = 0.03) and around the spinal cord (mean thickness, 47 versus 158 μm, P < 0.001). Examination of the spinal cord in the area of the MMC defect showed a higher large neuron density in the UC-MSC group (14.5 versus 5.6 neurons/mm 2 , P < 0.001). No tumours were observed., Conclusions: Fetal repair of MMC using UC-MSC patches improves motor and sphincter function as well as spinal preservation and reduction of fibrosis., (© 2023 John Wiley & Sons Ltd.)

To show a modified placement of the navigation reference frame in posterior corrective fusion of spinal deformity with myelomeningocele. This was a retrospective, single-surgeon case series, and IRB-approved study. Six consecutive patients (one male and five females) who were diagnosed with spinal deformity with myelomeningocele underwent posterior corrective fusion surgery from the upper thoracic spine to the pelvis with preoperative computed tomography navigation (pCTN). At the level of the spina bifida, where posterior elements such as the spinous process were missing, the reference frame of the pCTN was placed on the flipped lamina or pedicles, and a pedicle screw (PS) or iliac screw (IS) was inserted. Screw deviation was investigated by using postoperative CT. A total of 55 screws were placed at the spina bifida level and pelvis. Of these, 12 ISs were placed on each side in each case. The screws placed using the pCTN were not reinserted or removed intraoperatively or postoperatively. However, only one PS was found to have perforated the spinal canal on postoperative CT but was left in place because it caused no neurological problem. By modifying the placement of the reference frame, such as placing it on the flipped lamina or pedicles, pCTN could be used even at the levels of the spina bifida, where the posterior elements are missing, to accurately place PSs and various types of ISs., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

The intrauterine repair of myelomeningocele presents certain advantages and has gained widespread acceptance. It significantly reduces the incidence of Chiari-2 anomalies and hydrocephalus, and it is thought to enhance the neurologic outcome. Nevertheless, several issues remain unsettled and there are no negligible disadvantages. After working with patients with myelomeningocele for 30 years, I thought about how we currently treat them. There are ethical, organizational, neurological, obstetrical, and postnatal aspects worth discussing., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Objective: The objective of our study was to examine factors associated with distance to care for first surgical repair among infants with myelomeningocele in California., Study Design: A total of 677 eligible cases with complete geocoded data were identified for birth years 2006 to 2012 using data from the California Perinatal Quality Care Collaborative linked to hospital and vital records. The median distance from home to birth hospital among eligible infants was 9 miles, and from birth hospital to repair hospital was 15 miles. We limited our analysis to infants who lived close to the birth hospital, creating two study groups to examine transfer distance patterns: "lived close and had a short transfer" (i.e., lived <9 miles from birth hospital and traveled <15 miles from birth hospital to repair hospital; n  = 92), and "lived close and had a long transfer" (i.e., lived <9 miles from birth hospital and traveled ≥15 miles from birth hospital to repair hospital; n  = 96). Log-binomial regression was used to estimate crude and adjusted risk ratios (aRRs and 95% confidence intervals (CIs). Selected maternal, infant, and birth hospital characteristics were compared between the two groups., Results: We found that low birth weight (aRR = 1.44; 95% CI = 1.04, 1.99) and preterm birth (aRR = 1.41; 95% CI = 1.01, 1.97) were positively associated, whereas initiating prenatal care early in the first trimester was inversely associated (aRR = 0.64; 95% CI = 0.46, 0.89) with transferring a longer distance (≥15 miles) from birth hospital to repair hospital. No significant associations were noted by maternal race-ethnicity, socioeconomic indicators, or the level of hospital care at the birth hospital., Conclusion: Our study identified selected infant factors associated with the distance to access surgical care for infants with myelomeningocele who had to transfer from birth hospital to repair hospital. Distance-based barriers to care should be identified and optimized when planning deliveries of at-risk infants in other populations., Key Points: · Low birth weight predicted long hospital transfer distance.. · Preterm birth was associated with transfer distance.. · Prenatal care was associated with transfer distance.., Competing Interests: None declared., (Thieme. All rights reserved.)

Myelomeningocele (MMC) is a congenital defect of the spine characterised by meningeal and spinal cord protrusion through the open vertebral arches. This defect causes progressive prenatal damage of the spinal cord, leading to lifelong handicap. Although mid-trimester surgical repair may reduce part of the handicap, an earlier and less invasive approach would further improve the prognosis, possibly minimising maternal and foetal risks. Several studies have proposed an alternative approach to surgical repair by covering the defect with a patch and protecting the exposed neural tissue. Our study aims to elaborate on a waterproof and biodegradable bioactive patch for MMC prenatal foetal repair. We developed a double-layer patch that can provide a waterproof coverage for the spinal cord, with a bioactive side, conducive to cell proliferation, and an antiadhesive side to avoid its attachment to the medulla., (© 2023 The Wound Healing Society.)

Objective: Hydrocephalus is a neurological disorder with an incidence of 80-125 per 100,000 births in the United States. The most common treatment, ventricular shunting, has a failure rate of up to 85% within 10 years of placement. The authors aimed to analyze the association between ventricular catheter (VC) tissue obstructions and shunt malfunction for each hydrocephalus etiology., Methods: Patient information was collected from 5 hospitals and entered into a REDCap (Research Electronic Data Capture) database by hydrocephalus etiology. The hardware samples were fixed, and each VC tip drainage hole was classified by tissue obstruction after macroscopic analysis. Shunt malfunction data, including shunt revision rate, time to failure, and age at surgery, were correlated with the degree of tissue obstruction in VCs for each etiology., Results: Posthemorrhagic hydrocephalus was the most common etiology (48.9% of total cases). Proximal catheter obstruction was the most frequent cause of hardware removal (90.4%). Myelomeningocele (44% ± 29%), other congenital etiologies (48% ± 40%), hydrocephalus with brain tumors (45% ± 35%), and posthemorrhagic hydrocephalus (41% ± 35%) showed tissue aggregates in more than 40% of the VC holes. A total of 76.8% of samples removed because of symptoms of obstruction showed cellular or tissue aggregates. No conclusive etiological associations were detected when correlating the percentage of holes with tissue for each VC and age at surgery, shunt revision rates, or time between shunt implantation and removal., Conclusions: The proximal VC obstruction was accompanied by tissue aggregates in 76.8% of cases. However, the presence of tissue in the VC did not seem to be associated with hydrocephalus etiology.

Purpose: To investigate differences in sociodemographic characteristics and short-term outcomes between patients undergoing prenatal versus postnatal myelomeningocele repair., Methods: Patients who underwent myelomeningocele repair at our institution were stratified based on prenatal or postnatal timing of repair. Baseline characteristics and outcomes were compared. Multivariate analysis was performed to identify whether prenatal repair was a predictor of outcomes independent of socioeconomic measures., Results: 49 patients underwent postnatal repair, and 30 underwent prenatal repair. Patients who underwent prenatal repair were more likely to have private insurance (73.3% vs. 42.9%, p = 0.03) and live farther from the hospital where they received their repair (251.5 ± 447.4 vs. 72.5 ± 205.6 miles, p = 0.02). Patients who underwent prenatal repair had shorter hospital stays (14.3 ± 22.7 days vs. 25.3 ± 20.1 days, p = 0.03), fewer complications (13.8% vs. 42.9%, p = 0.01), fewer 30-day ED visits (0.0% vs. 34.0%, p < 0.001), lower CSF diversion rates (13.8% vs. 38.8%, p = 0.02), and better functional status at 3-months (13.3% vs. 57.1% delayed, p = 0.009), 6-months (20.0% vs. 56.7% delayed, p = 0.03), and 1-year (29.4% vs. 70.6% delayed, p = 0.007). On multivariate analysis, prenatal repair was an independent predictor of inpatient complication (OR(95%CI): 0.19(0.05-0.75), p = 0.02) and 3-month (OR(95%CI): 0.14(0.03-0.80) p = 0.03), 6-month (OR(95%CI): 0.12(0.02-0.73), p = 0.02), and 1-year (OR(95%CI): 0.19(0.05-0.80), p = 0.02) functional status., Conclusion: Prenatal repair for myelomeningocele is associated with better outcomes and developmental functional status. However, patients receiving prenatal closure are more likely to have private health insurance and live farther from the hospital, suggesting potential barriers to care., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Aim: We aimed to evaluate the occurrence of, and risk factors for precocious and early puberty in a retrospective cohort study of girls with shunted infantile hydrocephalus., Methods: The study population comprised 82 girls with infantile hydrocephalus, born between 1980 and 2002, and treated with a ventriculoperitoneal shunt. Data were available for 39 girls with myelomeningocele and 34 without. Medical records were analysed regarding clinical data and timing of puberty. Precocious and early puberty was defined as the appearance of pubertal signs before 8 years and 0 months and 8 years and 9 months, respectively., Results: Median age at last admission was 15.8 years (range 10.0-18.0). In total, 15 girls (21%) had precocious puberty, and another 21 (29%) had early puberty. Three or more shunt revisions had been performed in 26/36 girls with early or precocious puberty and in 3/37 girls without (p = 0.01). The number of shunt revisions correlated negatively with age at the start of puberty in the girls with myelomeningocele (Spearman's correlation coefficient = -0.512, p = 0.001)., Conclusion: Girls with shunted infantile hydrocephalus have a high risk of precocious or early puberty. Repeated shunt revisions seemed to be associated with early puberty., (© 2024 The Authors. Acta Paediatrica published by John Wiley & Sons Ltd on behalf of Foundation Acta Paediatrica.)

Abstract: Tethered cord syndrome is a neurological disorder closely associated with congenital spinal dysraphism. Aberrant dorsal nerve roots may be one of the possible and relatively rare tethering pathologies, especially in the complex form of occult spinal dysraphism such as caudal regression syndrome or split cord malformation. We report an illustrative case of caudal regression syndrome with spinal cord tethering due to a combination of a contiguous bundle of an aberrant dorsal nerve root, and a dorsal-type lipomyelomeningocele, with a thickened filum terminale treated with microneurosurgical untethering., (Copyright © 2024 Copyright: © 2024 Nigerian Journal of Clinical Practice.)

Background and Purpose: Spina bifida is the second major cause of congenital disorders and the most common central nervous system congenital malformation compatible with life primarily. Herein, we describe the short-term outcome of post-natal Myelomeningocele (MMC) surgical management and predictors of its postoperative complications and mortality., Methods: This retrospective chart review studies the children who underwent post-natal surgical management for MMC in Namazi hospital, a tertiary referral center, in southern Iran from May 2001 to September 2020., Results: 248 patients were included in this study. The mean age at the operation was 8.47 ± 8.69 days. The most common site of involvement of MMC was Lumbosacral (86%, n = 204). At the evaluation conducted prior to the operation, cerebrospinal fluid leak was observed in 7% (n=16) of the patients. Postoperatively, 5.7% of the patients expired in the 30-day follow-up after the operation (n = 14), while 24% needed readmission (n = 47). The most common complications leading to readmission were wound dehiscence (n = 10, 42%) and wound purulence (n = 6, 25%). Only the site of the lesion (p-value = 0.035) was associated with postoperative complication. After controlling for potential confounders, the site of the lesion (adjusted odds ratio = 0.146, 95% confidence interval = 0.035-0.610, p-value = 0.008) and age at surgery (adjusted odds ratio = 1.048, 95% confidence interval = 1.002-1.096, p-value = 0.041) were significantly associated with mortality CONCLUSIONS: The age of the patients at the surgery and the site of the lesion are the two factors that were associated with mortality. However, further investigations into preoperative interventions and risk factors to mitigate the risk of complications and mortality are highly encouraged., Competing Interests: Declaration of Competing Interest The authors have no relevant financial or non-financial interests to disclose., (Copyright © 2024 Elsevier B.V. All rights reserved.)

Background: The current convention for treatment of children with myelomeningocele (MMC) is timely surgical intervention combined with long-term follow-up by a multidisciplinary specialized team. This study aims to investigate the outcomes of MMC patients treated at Namazi Hospital., Methods: All children presenting to Namazi Hospital with myelomeningocele between May 2001 and August 2020 were eligible for this study. For those with a documented telephone number, follow-up phone surveys with the patient's caregivers, on top of the review of the medical documents were carried out to assess mortality, morbidities, and the functional outcome of the care provided to them., Results: A total of 125 patients were studied (62 females). All of the patients were followed up for a mean duration of 6.28 years (range 1-23 years). The majority were located in the lumbosacral area. All of the patients underwent postnatal surgical intervention for MMC in Namazi Hospital. Mean age at surgery was 9.51 days. There were statistically significant differences between urinary and bowel incontinence and presence of scoliosis, MMT grading of the lower limbs, school attendance, number of readmissions, and requirement of laminectomy at the initial surgical intervention., Conclusions: This study is the first to characterize the long-term outcomes of MMC patients in Iran. This study illustrates that there is a great need for improved access to and coordination of care in antenatal, perioperative, and long-term stages to improve morbidity and mortality., (© 2024. The Author(s).)

Objective: Although hydrocephalus rates have decreased with intrauterine surgery for myelomeningocele (MMC), 40%-85% of children with MMC still go on to develop hydrocephalus. Prenatal ventricle size is known to be associated with later development of hydrocephalus; however, it is not known how prediction measures or timing of hydrocephalus treatment differ between pre- and postnatal surgery for MMC. The goal of this study was to determine anatomical, clinical, and radiological characteristics that are associated with the need for and timing of hydrocephalus treatment in patients with MMC., Methods: The authors retrospectively identified patients from Barnes Jewish Hospital or St. Louis Children's Hospital between 2016 and 2021 who were diagnosed with MMC prenatally and underwent either pre- or postnatal repair. Imaging, clinical, and demographic data were examined longitudinally between treatment groups and hydrocephalus outcomes., Results: Fifty-eight patients were included (27 females, 46.6%), with a mean gestational age at birth of 36.8 weeks. Twenty-three patients (39.7%) underwent prenatal surgery. For the overall cohort, the ventricle size at prenatal ultrasound (HR 1.175, 95% CI 1.071-1.290), frontal-occipital horn ratio (FOHR) at birth > 0.50 (HR 3.603, 95% CI 1.488-8.720), and mean rate of change in head circumference (HC) in the first 90 days after birth (> 0.10 cm/day: HR 12.973, 95% CI 4.262-39.486) were identified as predictors of hydrocephalus treatment. The factors associated with hydrocephalus in the prenatal cohort were FOHR at birth > 0.50 (HR 27.828, 95% CI 2.980-259.846) and the rate of change in HC (> 0.10 cm/day: HR 39.414, 95% CI 2.035-763.262). The factors associated with hydrocephalus in the postnatal cohort were prenatal ventricle size (HR 1.126, 95% CI 1.017-1.246) and the mean rate of change in HC (> 0.10 cm/day: HR 24.202, 95% CI 5.119-114.431). FOHR (r = -0.499, p = 0.008) and birth HC (-0.409, p = 0.028) were correlated with time to hydrocephalus across both cohorts. For patients who underwent treatment for hydrocephalus, those in the prenatal surgery group were significantly more likely to develop hydrocephalus after 3 months than those treated with postnatal surgery, although the overall rate of hydrocephalus was significantly higher in the postnatal surgery group (p = 0.018)., Conclusions: Clinical and imaging factors associated with hydrocephalus treatment differ between those receiving pre- versus postnatal MMC repair, and while the overall rate of hydrocephalus is lower, those undergoing prenatal repair are more likely to develop hydrocephalus after 3 months of age. This has implications for clinical follow-up timing for patients treated prenatally, who may live at a distance from the treatment site.

Objective: The need for permanent CSF diversion is lower in patients who have undergone prenatal surgery for myelomeningocele (MMC) than in those who have undergone postnatal closure. Differences in brain development and head growth between treatment groups are not known, particularly for those who do not require surgical treatment for hydrocephalus. The objective of this study was to determine differences in head growth and to generate MMC-specific head circumference (HC) growth curves for patients who underwent either prenatal or postnatal surgery., Methods: The authors retrospectively identified patients from St. Louis Children's Hospital who were treated for MMC between 2016 and 2021. HC data were obtained from birth until the most recent follow-up or hydrocephalus treatment. Nonlinear least-squares regression analysis was performed to fit the data into four models: two-term power, Gompertz, West ontogenetic, and Weibull. Subsequently, the curves were assessed for their utility in predicting hydrocephalus treatment., Results: Sixty-one patients (29 females [47.5%], 25 [41%] underwent prenatal surgery, mean gestational age at birth 36.6 weeks) were included in the study. The Weibull model best fit the HC data (prenatal adjusted R2 = 0.95, postnatal adjusted R2 = 0.95), while the Gompertz model had the worst fit (prenatal adjusted R2 = 0.56, postnatal adjusted R2 = 0.39) across both cohorts. Prenatal MMC repair patients had significantly larger HC measurements than their postnatal repair counterparts. The 50th percentile of the Weibull curve was determined as a useful threshold for hydrocephalus treatment: children with HC measurements that crossed and remained above this threshold were significantly more likely to have hydrocephalus treatment regardless of time of MMC repair (prenatal relative risk [RR] 10.0 [95% CI 1.424-70.220], sensitivity 85.7% [95% CI 0.499-0.984], and specificity 82.4% [95% CI 0.600-0.948]; postnatal RR 4.750 [95% CI 1.341-16.822], sensitivity 90.5% [95% CI 0.728-0.980], and specificity 75.0% [95% CI 0.471-0.924]). The HC growth curves of the MMC patients treated prenatally were significantly larger than the WHO HC curves (p < 0.001)., Conclusions: The Weibull model was identified as the HC growth curve with the best fit for MMC patients and serves as a useful predictor of hydrocephalus treatment. For MMC patients with hydrocephalus, prenatal repair patients fit the model well but postnatal repair patients did not, potentially indicating different mechanisms of hydrocephalus development. Those treated prenatally had significantly larger HC measurements compared with both the general population and those treated postnatally. Further study is needed to understand the long-term cognitive outcomes and optimal management of clinically asymptomatic patients with large HC measurements who were treated prenatally for MMC.

Objective: Among patients with a history of prior lipomyelomeningocele repair, an association between increased lumbosacral angle (LSA) and cord retethering has been described. The authors sought to build a predictive algorithm to determine which complex tethered cord patients will develop the symptoms of spinal cord retethering after initial surgical repair with a focus on spinopelvic parameters., Methods: An electronic medical record database was reviewed to identify patients with complex tethered cord (e.g., lipomyelomeningocele, lipomyeloschisis, myelocystocele) who underwent detethering before 12 months of age between January 1, 2008, and June 30, 2022. Descriptive statistics were used to characterize the patient population. The Caret package in R was used to develop a machine learning model that predicted symptom development by using spinopelvic parameters., Results: A total of 72 patients were identified (28/72 [38.9%] were male). The most commonly observed dysraphism was lipomyelomeningocele (41/72 [56.9%]). The mean ± SD age at index MRI was 2.1 ± 2.2 months, at which time 87.5% of patients (63/72) were asymptomatic. The mean ± SD lumbar lordosis at the time of index MRI was 23.8° ± 11.1°, LSA was 36.5° ± 12.3°, sacral inclination was 30.4° ± 11.3°, and sacral slope was 23.0° ± 10.5°. Overall, 39.6% (25/63) of previously asymptomatic patients developed new symptoms during the mean ± SD follow-up period of 44.9 ± 47.2 months. In the recursive partitioning model, patients whose LSA increased at a rate ≥ 5.84°/year remained asymptomatic, whereas those with slower rates of LSA change experienced neurological decline (sensitivity 77.5%, specificity 84.9%, positive predictive value 88.9%, and negative predictive value 70.9%)., Conclusions: This is the first study to build a machine learning algorithm to predict symptom development of spinal cord retethering after initial surgical repair. The authors found that, after initial surgery, patients who demonstrate a slower rate of LSA change per year may be at risk of developing neurological symptoms.

The keystone flap has become an established option that has been utilized recently to close significant myelomeningocele defects. Although the keystone flaps can be adapted to repair myelomeningocele defects of different sizes, the shape of the defect is also an essential factor to consider. In this study, the authors proposed an algorithmic approach for reconstructing significant meningomyelocele defects utilizing different types of keystone flaps based on the shape of the resulting defect. From September 2016 to March 2022, the authors retrospectively reviewed the medical records of 28 patients with meningomyelocele who underwent repair with the keystone flaps. The mean age was 28 days (3 days to 4.5 months). Twenty-one meningomyelocele defects were in the lumbosacral (75%) and 7 in the thoracolumbar regions (25%). The defect sizes ranged from 3.5×5 cm (area, 13.4 cm 2 ) to 9×7 cm (area, 49.5 cm 2 ), with an average of 26.2 cm 2 . Ten meningomyelocele defects were a vertically oriented oval shape, 16 defects were a transversely oriented oval shape, and 2 defects were circular. In addition, unilateral keystone flap was used in 7.1% of the patients (2 patients), bilateral keystone flap in 28.6% (8 patients), and rotation advancement keystone flaps in 64.3% (18 patients). According to these findings, an algorithm was created based on the defect shape. This algorithmic approach is used as a decision-making guide to select the correct type of keystone flaps to repair myelomeningocele according to the defect shape., Competing Interests: The authors report no conflicts of interest., (Copyright © 2023 by Mutaz B. Habal, MD.)