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Aim: Acute appendicitis is a common surgical emergency in children, yet it poses diagnostic challenges in preschool children due to atypical presentation. This case-control study aims to evaluate the distinct characteristics of acute appendicitis in preschool compared to school-aged children., Methods: Children under 5 years and a control group of children aged 5-10 years, operated on due to acute appendicitis at our institution during 2009-2022 were included. Data on demographics, clinical presentation, laboratory results, imaging, surgical procedure, pathology, bacteriology, antibiotic treatment, length of stay and outcomes were collected retrospectively., Results: A total of 184 preschool and 187 school-aged children were included. Preschool children presented less frequently with abdominal pain but more often with vomiting, diarrhoea and fever. Preschool children had lower rates of leucocytosis but higher thrombocytosis and C reactive protein. Younger patients were more likely to undergo both chest and abdominal X-rays and be admitted to a paediatric department before diagnosing appendicitis. Complicated appendicitis, requiring increased use of intra-operative drain placement, and conversions to open procedures were more common in preschool children, with higher rates of ICU admission and surgical complications. Polymicrobial positive cultures and positive cultures for Bacteroides were more common in preschool children., Conclusions: This study highlights the critical need for increased awareness among healthcare providers regarding the diagnostic challenges posed by atypical presentations of acute appendicitis in preschool children. Despite comprehensive evaluation, diagnosis in this age group may be difficult, emphasising the significance of recognising these presentation patterns to improve diagnostic accuracy and prompt management., (© 2024 Paediatrics and Child Health Division (The Royal Australasian College of Physicians).)

Purpose:  All types of cloacal malformations may be associated with anatomic variations of the external genitalia, including hypoplasia of the labia minora and enlarged clitoris; these variations could be even higher in posterior cloacas (PCs). If a careful physical examination is not performed, patients may be misdiagnosed with ambiguous genitalia (AG), leading to subsequent unnecessary testing, surgeries, or even wrong gender assignment. The aim was to analyze data of patients with PC within the ARM-Net registry, focusing on the description of the genitalia, gender assignment, and its consequences. Additionally, we investigated the presence of AG diagnosis in utero or at birth in patients with PC in the literature., Methods:  The ARM-Net registry was scanned for PC cases and data on diagnosis were collected. A systematic literature search was conducted using the PubMed, EMbase, and Web-of-Science databases. Descriptive statistics was used to report data., Results:  Nine patients with PC were identified in the ARM-Net registry. Five patients (55%) were diagnosed with AG, two (22%) were assigned as males and only two patients were correctly assigned as females and diagnosed with PC with respective variations of external genitalia. All patients diagnosed with AG had extensive blood testing including karyotype and hormonal studies. One of the patients who was diagnosed as a male, had surgery for pelvic cystic mass removal, which ultimately led to unaware salpingo-oophorectomy, hysterectomy, and vaginectomy. In the literature we identified 60 patients, 14 (23%) with AG, 1 with clitorolabial transposition and 1 with undeveloped vulva and vagina; 4 patients had normal anatomy. In 40 (67%) patients the anatomy of genitalia was not mentioned., Conclusion:  Patients with PC are at high risk of being diagnosed with AG or even assigned the wrong gender at birth. In our series two patients were assigned as males, and consequently one of them underwent a highly mutilating surgery. A thorough physical examination together with a high index of suspicion and laboratory workup are mandatory to identify these variations, avoiding further investigations, unnecessary surgeries, and parental stress., Competing Interests: None declared., (Thieme. All rights reserved.)

Background: Cloacal malformation represents the rarest and most complex congenital anorectal malformation in females and is characterized by the convergence of urinary, gynecological, and intestinal systems within a single common channel. Three-dimensional computed tomography reconstruction (3D CT cloacagram) has emerged as a valuable method for anatomical assessment and preoperative planning., Objectives: To evaluate our experience with 3D CT cloacagram and assess its results., Methods: This retrospective case series included all patients with cloacal malformation who underwent preoperative 3D CT cloacagram at a single institution during 2019-2023. Collected data included patient characteristics, timing of the 3D CT cloacagram, results of the 3D CT cloacagram, comparison with endoscopic results, surgical procedures, and postoperative outcome., Results: Six patients with cloacal malformation were included in this study, including two with posterior cloaca. The median common channel length on 3D CT cloacagram was 24.5 mm (range 9-48 mm) and the median urethral length was 15.5 mm (range 13-24 mm). The surgical approach involved a combined abdominoperineal approach in three patients and posterior sagittal anorectal vaginal urethral plasty in one patient. Two patients were awaiting surgical reconstruction at the time of publication., Conclusions: Our implementation of 3D cloacagram has facilitated precise measurements of both the urethra and common channel lengths, two key factors in formulating surgical strategies for cloacal reconstruction. Moreover, this technique has markedly improved our capacity for surgical planning contributing to colorectal, gynecological, and urological perspectives.

Introduction: Patients with Hirschsprung disease (HD) can experience soiling after their pull-through. We evaluated the use of antegrade continence enema (ACE) for this patient population and investigated the timing and factors associated with getting the patient clean., Methods: We performed a single-institution retrospective review (from January 2020 to January 2023) of patients with HD and prior pull-through who had persistent soiling, failed laxative treatment or rectal enemas, and were treated with antegrade enemas. The primary outcome was time to become "clean of stool" which was defined as at least one bowel movement per day, no more than one stool accident per week, and no significant stool burden radiographically. Kaplan-Meier survival analysis was performed, and univariate cox proportional hazard regression was used to assess factors associated with time to continence., Results: Thirty patients who met the criteria underwent ACE creation at a median age of 6.5 y (interquartile range 5.3-9.8) with a median follow-up time of 11.5 mo (interquartile range 5.6-16.5). Most patients became clean by 4 mo (13 of 20, 65%) with similar results at 1-y follow-up (16 of 21, 76%). The median time to becoming clean was 4.3 mo (95% confidence interval: 1.7-15.0. Patients with hypermotility were more likely to continue to soil at 1 y (80% versus 13%, P = 0.01). There were no additional factors significantly associated with time to cleanliness., Conclusions: ACE is a useful modality for HD patients with soiling. Most became clean of stool in 4 mo. The presence of hypermotility was associated with a higher incidence of persistent soiling at 1 y., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Background: Most congenital pulmonary airway malformations (CPAMs) are detected antenatally. The majority of newborns are asymptomatic. Patients are prone to subsequent respiratory complications and to a lesser extent malignant transformation remains concerning. In Israel, until 2013, pediatric surgeries were performed by thoracotomy. To minimize its morbidity, we introduced thoracoscopy using a mentorship approach. We present our experience with thoracoscopic resections coordinated by the mentorship of a pediatric worldwide leader in his field and compare our results with resections performed by thoracotomy., Materials and Methods: A retrospective review of records of children operated between 2013 and 2020 was conducted. Data were compared using t -test for quantitative variables., Results: Fifty patients were operated by thoracoscopy with a median age of 4 years, a thoracoscopic lobectomy performed in 68%. There was no conversion with a median length of stay (LOS) of 3½ days. Thirty patients were operated by thoracotomy by a thoracic surgeon with a median age of 3.5 years. A lobectomy was performed in 87% with a median LOS of 7 days., Conclusions: Thoracoscopic lobectomy is a technically demanding procedure with a long learning curve, strongly related to the low volume of cases. The role of a mentorship program in acquiring those surgical skills is crucial through standardization of the technique applied and supervised by the mentor. Early thoracoscopy for congenital pulmonary lesions at an early age can be achieved with a low conversion rate and minimal complications creating a change in the paradigm of practice when considering surgery for CPAM in Israel., Competing Interests: There are no conflicts of interest., (Copyright: © 2023 Journal of Indian Association of Pediatric Surgeons.)

Purpose: To assess the number, characteristics, and functional short-, and midterm outcomes of patients with rectal atresia (RA) and stenosis (RS) in the ARM-Net registry., Methods: Patients with RA/RS were retrieved from the ARM-Net registry. Patient characteristics, associated anomalies, surgical approach, and functional bowel outcomes at 1 and 5-year follow-up were assessed., Results: The ARM-Net registry included 2619 patients, of whom 36 (1.3%) had RA/RS. Median age at follow-up was 7.0 years (IQR 2.3-9.0). Twenty-three patients (63.9%, RA n = 13, RS n = 10) had additional anomalies. PSARP was the most performed reconstructive surgery for both RA (n = 9) and RS (n = 6) patients. At 1-year follow-up, 11/24 patients with known data (45.8%, RA n = 5, RS n = 6) were constipated, of whom 9 required stool softeners and/or laxatives. At 5-year follow-up, 8/9 patients with known data (88.9%, RA n = 4, RS n = 4) were constipated, all requiring laxatives and/or enema., Conclusion: RA and RS are rare types of ARM, representing 1.3% of patients in the ARM-Net registry. Additional anomalies were present in majority of patients. Different surgical approaches were performed as reconstructive treatment, with constipation occurring in 46% and 89% of the patients at 1 and 5-year follow-up. However, accurate evaluation of long-term functional outcomes remains challenging., (© 2023. The Author(s).)

Background: Ileo-colic resection (ICR) is an important therapeutic option for Crohn's disease (CD) patients. There are limited updated data of clinical and endoscopic post-operative recurrence (POR) in pediatric patients with CD for the long run. We aimed to determine recurrence rates following ICR over an extended period of time and asses its risk factors., Methods: This is a single-center retrospective review of 35 patients with CD between the ages of 6 and 17.9 years who required ICR between 2003 and 2021 at Schneider Children Medical Center of Israel. Medical charts were reviewed at different time-points post-ICR., Results: Clinical recurrence following ICR was demonstrated in only 11.4% and 28.6% (n = 4, n = 10) in the first two and five years-much lower rates than what was reported so far. We found no specific risk factor that correlated with clinical recurrence, although patients that were treated with early prophylaxis of anti-TNF medications following ICR tend to have less recurrence., Conclusions: We found lower POR following ICR, especially in the first years after surgery-which can be attributed to close surveillance and early medical treatment. Such surveillance seems to improve recurrence rates in the first years following ICR., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Introduction:  To investigate the current experience of the ARM-Net Consortium in the management of epididymo-orchitis (EO) in patients with anorectal malformations (ARMs), and to identify specific risk factors and the need for urological care involvement., Materials and Methods:  We retrospectively collected data of EO in patients with ARM between 2015 and 2019. Data on urological aspects, ARM type, surgical approach, associated anomalies, diagnosis, and treatment of EO were collected and analyzed., Results:  Twenty-nine patients were reported by 12 centers. Twenty-six patients with EO (90%) had ARM with a rectourinary fistula. Median age at first EO was 2 years (range: 15 days-27 years). Twenty patients (69%) experienced multiple EO, and 60% of recurrences were ipsilateral. Associated urological anomalies included vesicoureteral reflux (48%), urethral anomalies (41%), neurogenic bladder (41%), and ectopic vas (10%). A positive urine culture during EO was present in 69%. EO was treated with antibiotics (90%), limiting surgical exploration to 14%. Prevention of recurrences included surgery (bulking agents 15%, vasectomy 15%, and orchiectomy 5%) and antibiotic prophylaxis (20%)., Conclusion:  Urologists may encounter patients with EO in ARM patients, frequently with positive urine culture. An appropriate urologic work-up for most ARM patients is necessary to identify and treat underlying risk factors. A practical scheme for the work-up is suggested for a close collaboration between pediatric surgeons and urologists., Competing Interests: None declared., (Thieme. All rights reserved.)

Aim: Ingestion of multiple magnets is increasing these last 15 years in children. They have resulted in numerous reports of serious gastrointestinal complications such as bowel obstruction, ischaemia, necrosis, perforation and fistula formation and even led to death. The increasing number of world-wide reports of complications secondary to magnet ingestion and a frequently delayed diagnosis point to a lack of awareness about these risks among medical care-givers in our country and parents in general., Methods: We reviewed retrospectively all cases of multiple magnet ingestion that required a gastro-intestinal or surgical procedure for removal from 2009 to 2020., Results: Five children underwent gastroscopy removal and three colonoscopy removal of the magnets. Five patients required surgical (laparotomy or laparoscopy) removal of multiple magnets with intestinal perforations., Conclusions: We propose an updated management algorithm for multiple magnet ingestion to highlight awareness among primary physicians and parents of the presenting circumstances and symptoms as well as the potential complications associated with multiple magnet ingestion., (© 2022 Paediatrics and Child Health Division (The Royal Australasian College of Physicians).)

Objectives: The sacral ratio (SR) was described as a postnatal X-ray-based method to detect sacral abnormalities and predict functional prognosis for fecal continence in children with anorectal malformations (ARMs). The present study aimed to describe a novel method of assessing sonographic fetal sacral ratio (f-SR) in a normal population of fetuses., Methods: Sixty three-dimensional (3D) ultrasound reconstruction images of the sacrum obtained from routine low-risk scans performed between 21 and 26 weeks of gestation served for measurement. The f-SR was calculated in a coronal view as the ratio between lines drawn at the upper and lower levels of the iliac bone and the 5th sacral vertebra. Bland-Altman plots assessed the inter- and intrareader variabilities of measurements., Results: The f-SR in the normal population of fetuses was 0.913 (±0.094). During the study period, three cases with ARM were examined and had a mean f-SR of 0.55. There was good repeatability of measurements and between readers' agreement., Conclusions: The present study introduces a novel prenatal sonographic f-SR that can be reliably calculated on prenatal 3D ultrasound with good reliability and reproducibility. Future research will identify the clinical significance of f-SR abnormalities in ARM and their long-term impact on continence., (© 2021 American Institute of Ultrasound in Medicine.)

In the past, an anteriorly located anus was often misdiagnosed and treated as an anorectal malformation (ARM) with a perineal fistula (PF). The paper aims to define the criteria for a normal anus, an anterior anus (AA) as an anatomic variant, and milder types of ARM such as congenital anal stenosis (CAS) and PF. An extensive literature search was performed by a working group of the ARM-Net Consortium concerning the subject "Normal Anus, AA, and mild ARM". A consensus on definitions, clinical characteristics, diagnostic management, and treatment modalities was established, and a diagnostic algorithm was proposed. The algorithm enables pediatricians, midwives, gynecologists, and surgeons to make a timely correct diagnosis of any abnormally looking anus and initiate further management if needed. Thus, the routine physical inspection of a newborn should include the inspection of the anus and define its position, relation to the external sphincter, and caliber. A correct diagnosis and use of the presented terminology will avoid misclassifications and allow the initiation of correct management. This will provide a reliable comparison of different therapeutic management and outcomes of these patient cohorts in the future.

Aim: Percutaneous endoscopic gastrostomy (PEG) and surgical gastrostomy (SG) are common procedures in children with neurological impairment (NI) with swallowing difficulties. Pulmonary aspirations are a major concern and performing concomitant or delayed fundoplication is still controversial, especially among these patients. The aim of our study was to review our experience with fundoplication performed concomitantly with gastrostomy or later and to evaluate patient outcomes., Methods: This is a retrospective, cohort study including all paediatric patients who underwent SG or PEG with or without Nissen fundoplication at Schneider Children's Medical Center of Israel between the years 2007 and 2018. Patients' clinical and surgical data were recorded and analysed., Results: Between 2007 and 2018, 345 patients underwent SG or PEG. Of these, 89 patients underwent fundoplication. Of the patients who underwent PEG/SG, 158 (45.8%) were neurologically impaired. Most of the patients who underwent fundoplication (n = 69, 77.5%) were NI patients (P = 0.0001). NI patients with refractory seizures showed almost no improvement in terms of relief of gastro-oesophageal reflux disease symptoms following fundoplication (P = 0.0001) compared to NI patients without refractory seizures., Conclusion: Our findings suggest that in NI patients a concomitant fundoplication is not mandatory and is not efficacious in preventing gastro-oesophageal reflux disease in patients with refractory seizures., (© 2021 Paediatrics and Child Health Division (The Royal Australasian College of Physicians).)

Background: Pancreatic trauma is uncommon in pediatric patients and presents diagnostic and therapeutic challenges. While non-operative management (NOM) of minor pancreatic injuries is well accepted, the management of major pancreatic injuries remains controversial., Objectives: To evaluate management strategies for major blunt pancreatic injury in children., Methods: Data were retrospectively collected for all children treated for grade III or higher pancreatic injury due to blunt abdominal trauma from 1992 to 2015 at two medical centers. Data included demographics, mechanism of injury, laboratory and imaging studies, management strategy, clinical course, operative findings, and outcome., Results: The cohort included seven boys and four girls aged 4-15 years old (median 9). Six patients had associated abdominal (mainly liver, n=3) injuries. The main mechanism of injury was bicycle (handlebar) trauma (n=6). Five patients had grade III injury and six had grade IV. The highest mean amylase level was recorded at 48 hours after injury (1418 U/L). Management strategies included conservative (n=5) and operative treatment (n=6): distal (n=3) and central (n=1) pancreatectomy, drainage only (n=2) based on the computed tomography findings and patient hemodynamic stability. Pseudocyst developed in all NOM patients (n=5) and two OM cases, and one patient developed a pancreatic fistula. There were no differences in average length of hospital stay., Conclusions: NOM of high-grade blunt pancreatic injury in children may pose a higher risk of pseudocyst formation than OM, with a similar hospitalization time. However, pseudocyst is a relatively benign complication with a high rate of spontaneous resolution with no need for surgical intervention.

Acute appendicitis is the most common cause of acute abdominal pathology in children. However, other rare non-inflammatory non-neoplastic disorders involving the appendix may manifest as surgical emergencies. This study aimed to describe these atypical entities and present representative cases. The database of a tertiary children's medical center was reviewed for all the patients aged 0-18 years who underwent urgent appendectomy between June 2014 and December 2019, for rare disorders of the appendix unrelated to inflammatory or neoplastic processes. Of 1367 patients who underwent appendectomy, 1345 were operated urgently or emergently. Of these, six, all males, mean age 32.6 months (range 0.7-76), underwent appendectomy for rare surgical complications that involved the appendix. These included torsion of the appendix (2), a strangulated internal hernia through an appendicular ring (1) or through a mesoappendix gap (1), an incarcerated appendix in an acute hernia sac (1), and appendiceal intussusception (1). In all cases, the role of the appendix in the pathologic process was unexpected and came as a surprise to the surgeon. During a median follow-up of 4.2 months (range 1-8 months), one patient underwent relaparotomy for small bowel obstruction 4 weeks after the original procedure.Conclusion: The appendix in children can be the source of rare pathological disorders that present as surgical emergencies. Familiarity with these entities may aid in achieving accurate preoperative diagnosis and contribute to surgical team orientation on exploratory laparotomy. However, correct diagnosis is often only established during timely surgical intervention.

In this review, the care of children with a previously repaired anorectal malformation is explored. We know that the surgical care of children with anorectal malformations is complex; however, despite an increased understanding of the congenital anomaly and significant technical advances in the operative repair, many of these children continue to have poor functional outcomes. In this article we focus on the common surgical complications, discuss typical presentations, consider appropriate investigations, and review the risks and benefits of revisional surgery in those patients that are 'not doing well' following their primary reconstruction., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Objective: To review the Anorectal Malformation Network experience with perineal groove (PG) focusing on its clinical characteristics and management., Study Design: Data on patients with PG managed at 10 participating Anorectal Malformation Network centers in 1999-2019 were collected retrospectively by questionnaire., Results: The cohort included 66 patients (65 females) of median age 1.4 months at diagnosis. The leading referral diagnosis was anal fissure (n = 20 [30.3%]): 23 patients (34.8%) had anorectal malformations. Expectant management was practiced in 47 patients (71.2%). Eight (17%) were eventually operated for local complications. The median time to surgery was 14 months (range, 3.0-48.6 months), and the median age at surgery was 18.3 months (range, 4.8-58.0 months). In the 35 patients available for follow-up of the remaining 39 managed expectantly, 23 (65.7%) showed complete or near-complete self-epithelization by a mean age 15.3 months (range, 1-72 months) and 4 (11.4%) showed partial self-epithelization by a mean age 21 months (range, 3-48 months). Eight patients showed no resolution (5 were followed for ≤3 months). Nineteen patients (28.7%) were primarily treated with surgery. In total, 27 patients were operated. Dehiscence occurred in 3 of 27 operated patients (11.1%)., Conclusions: PG seems to be an underestimated anomaly, frequently associated with anorectal malformations. Most cases heal spontaneously; therefore, expectant management is recommended. When associated with anorectal malformations requiring reconstruction, PG should be excised in conjunction with the anorectoplasty., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Objectives: Infantile hypertrophic pyloric stenosis (IHPS) is potentially life threatening. The etiology of IHPS remains unknown and many risk factors have been reported. We aimed to assess the prevalence of known risk factors and investigate maternal nutrition and habits as possible additional risk factors for IHPS., Methods: This case-control study includes mothers of infants diagnosed with IHPS and control mothers of infants, age 2 to 11 months, hospitalized in the pediatric department due to other conditions. Cases of IHPS were identified by review of all infants diagnosed with IHPS and operated upon in 2010 to 2016 at 2 major hospitals in central Israel. Data regarding potential risk factors were collected via questionnaires in both study groups., Results: Sixty-six cases and 67 controls were included in the study. Maternal omega 3 supplement consumption during pregnancy was significantly less common among cases of IHPS as compared with controls (P = 0.031). Consumption of omega 3 supplement was defined as consumption of at least 1 to 2 per week during the pregnancy period. Following adjustment for known risk factors, including male sex and maternal smoking, maternal omega 3 supplement consumption remained associated with a significantly lower risk of developing IHPS (odds ratio = 0.303, 95% confidence interval 0.111-0.828, P = 0.02)., Conclusions: Maternal omega 3 supplement consumption during pregnancy was associated with a significantly reduced risk of IHPS. Further studies are needed to support these results and investigate possible mechanisms of the effect of omega 3.

Background: Perianal abscess is a common surgical condition in daily pediatric practice. Management is a subject of controversy and a variety of approaches are practiced. While the most frequent approach is drainage with/without fistulotomy, the superiority of this approach and the place of conservative approach has not been established. The aim of this study was to evaluate the outcomes of conservative approach in selected cases of perianal abscesses in infants., Methods: Data of 19 patients aged <24 months treated conservatively for perianal abscess at a tertiary hospital in 2014-2018 were retrospectively reviewed., Results: Criteria for a conservative approach were: spontaneous drainage into the anal canal (n = 8) or perianal skin (n = 4), and phlegmonous infiltrate with fluid collection detected on ultrasound (n = 7). Mean age at symptom onset was 8.4 months. Twelve patients were managed for the first time. Previous care in seven patients included 1-4 drainage procedures (n = 4), spontaneous drainage (n = 1) and antibiotics (n = 2). Five patients were on oral antibiotics at presentation. After diagnosis, 18 patients received i.v. antibiotics and one, oral antibiotics. Three patients (15.7%) ultimately required surgical drainage; two were lost to follow up. During follow up (mean, 22.4 months) four patients (28.5%) had a single recurrent episode; abscess in three (managed conservatively in two and surgically in one) and fistula-in-ano in one patient that healed spontaneously. Thus, surgical intervention was prevented in 13/17 patients (76.4%) available for follow up., Conclusions: Perianal abscess in infants is amenable to conservative management in selected cases. Avoiding surgical intervention is advantageous, especially given the high recurrence rate., (© 2019 Japan Pediatric Society.)

Aim: Perineal hemangiomas rarely occur in patients with anorectal malformations (ARMs), but they can pose a significant challenge and warrant special attention. Surgical incision of posterior sagittal anorectoplasty (PSARP) may involve the hemangioma site resulting in hemorrhage, damage to blood supply, leading to complications and adversely affecting outcome. The aim of this study was to review the experience of the ARM-Net Consortium in the management of perineal hemangioma associated with ARM and evaluate treatment strategies., Materials and Methods: Data on all patients with ARM and a perineal hemangioma located in the planes of the PSARP dissection who were managed at participating ARM-Net centers were collected retrospectively by questionnaire, as follows: ARM type, hemangioma distribution and penetration, imaging findings, medical/surgical management, timing of definitive repair, complications and outcome., Results: Ten patients from eight centers were included. Three patients each had a rectobulbar or rectovestibular fistula, 2 had a rectoperineal fistula, and one had a rectoprostatic fistula; in one patient, the hemangioma was too disfiguring to determine malformation type. Mean follow-up time was 36.6 months (median 29 months). Colostomies were performed before definitive repair in 8 patients. Five patients received systemic beta-blockers before PSARP: 3 were operated uneventfully following partial/complete involution of the hemangioma, and 2 are awaiting surgery. The two patients with rectoperineal fistula were managed expectantly. The remaining 3 patients underwent surgery with no preoperative medical treatment, and all had complications: mislocated neoanus in three and complete perineal dehiscence in one., Conclusion: Attempting PSARP in the presence of a perineal hemangioma may lead to complications and adversely affect outcome. This study confirms the benefits of beta blocker treatment before surgical reconstruction., Level of Evidence: Treatment study, level III., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Emergency splenectomy is rarely performed since a widespread consensus exists towards conservative management of splenic injury. However, in selected conditions, mainly hematological, there is a role for emergency or urgent splenectomy. This study aims to retrospectively review these cases and discuss outcome in relation to the pre-existing splenic pathologies. Between 2000 and 2015, 12 patients, five girls, and seven boys, with a median age of six years (3 months-13.11 years), underwent emergency or urgent splenectomy for non-traumatic conditions. All patients had major associated disorders; mainly hematological (11 cases) including hemolytic anemia with pancytopenia (1), sickle cell anemia (1), AML (1), ALL (2), CML (1), T cell lymphoma (1), Burkitt lymphoma (1), and ITP (3). One patient had a microvillous inclusion disease. Indications for splenectomy included diffuse resistant splenic abscesses (4), intracranial hemorrhage (4) or hypersplenism (3) with refractory thrombocytopenia, and spontaneous splenic rapture (1). Nine patients improved following surgery but three died, owing to massive intracranial hemorrhage (1) and severe respiratory failure (2) despite aggressive management.Conclusions: Rarely, an emergency splenectomy is required in complex settings, mostly refractory hematological conditions, in a deteriorating patient when all other measurements have failed. A multidisciplinary team approach is mandatory in the treatment of these complex cases. What is known • Conservative treatment is advised for splenic injury. • Many hematological disorders are responsible of splenic pathology. What is new • Emergency splenectomy in children for reasons other than trauma is a treatment of last resort that should be performed in a multidisciplinary context. • The outcome of emergency splenectomy in children for reasons other than trauma depends on the underlying medical condition.

Introduction:  Acute appendicitis is the most common emergency condition in pediatric surgery. Historically, a triple-antibiotic therapy consisted of ampicillin, gentamicin, and clindamycin has been used postoperatively for perforated appendicitis. According to recently published trials, dual therapy consists of ceftriaxone and metronidazole only, offers a more efficient and cost-effective antibiotic management compared with triple therapy. Based on these results, our department applied dual antibiotic therapy for children with perforated appendicitis that underwent appendectomy from 2009 and forth., Aim:  The aim of our study was to compare postoperative outcomes between patients treated with triple therapy (ampicillin, gentamicin, and metronidazole) (group A) versus dual therapy (ceftriaxone and metronidazole) (group B)., Methods:  Clinical and laboratory data were retrospectively collected by review of the medical records for all children who underwent appendectomy for the perforated appendix at the Schneider Children's Medical Center of Israel, a tertiary pediatric care center between 2007 and 2011. Children with perforated appendicitis received antibiotic therapy in accordance with the hospital's guidelines that were valid at the time the surgery took place. In the first period (years 2007-2009) (group A) a triad of ampicillin, gentamicin, and metronidazole and the second period (2009-2011) (group B) dual therapy consists of ceftriaxone and metronidazole. The two groups were compared for outcome and complications, such as wound infections, changing of antibiotic therapy, and length of stay., Results:  During the study period 1,203 patients underwent an appendectomy. Of these, 175 patients were diagnosed with perforated appendix and were treated with postoperative antibiotic's regimen. Group A and group B consisted of 89 and 86 patients, respectively. The two groups were not different significantly in terms of demographic data, length of stay, or readmission rates. However, more rates of wound infection and changing of antibiotic therapy were seen in group B, although not statistically significant ( p  = 0.064)., Conclusion:  Dual antibiotic therapy for perforated appendicitis is a cost-effective and efficient mode of therapy compared with triple-antibiotic's regimen. However, prospective studies are required to determine whether this policy is associated with higher rates of wound infections and change in antibiotic therapy., Competing Interests: None., (Georg Thieme Verlag KG Stuttgart · New York.)

Background: Wandering spleen is a rare entity that may pose a surgical emergency following torsion of the splenic vessels, mainly because of a delayed diagnosis. Complications after surgery for wandering spleen may necessitate emergency treatment., Objectives: To describe the clinical course and treatment for children who underwent emergency surgeries for wandering spleen at a tertiary pediatric medical center over a 21 year period and to indicate the pitfalls in diagnosis and treatment as reflected by our experience and in the literature., Methods: The database of a tertiary pediatric medical center was searched retrospectively for all children who underwent emergency treatment for wandering spleen between 1996 and 2017. Data were collected from the medical files. The relevant literature was reviewed., Results: Of ten patients who underwent surgery for wandering spleen during the study period, five underwent seven emergency surgeries. One patient underwent surgery immediately at initial presentation. In the other four, surgical treatment was delayed either due to misdiagnosis or for repeated imaging studies to confirm the diagnosis. Emergency laparotomy revealed an ischemic spleen in all patients; splenectomy was performed in two and the spleen was preserved in three. Four of the seven emergency operations were performed as the primary surgery and three were performed to treat complications., Conclusions: Wandering spleen should ideally be treated on an elective or semi-elective basis. Surgical delays could be partially minimized by a high index of suspicion at diagnosis and by eliminating unnecessary and time-consuming repeated imaging studies.

Introduction:  Appendiceal intussusception is a rare condition in children characterized by an invagination of the appendix into the cecum to various degrees. The treatment is appendectomy; however since symptoms are not specific, clinical diagnosis is challenging and frequently only intraoperative. We present a series of five patients with appendiceal intussusception and discuss features that may direct the pediatric surgeon to achieve early recognition and provide optimal treatment., Materials and Methods:  The database of a tertiary medical center was retrospectively reviewed for all patients treated for appendiceal intussusception during the period from January 1995 to January 2016. Data collected by chart review included demographics, clinical characteristics, imaging studies, surgical technique, and outcome. The findings were analyzed by descriptive statistics., Results:  This series included five patients (two females and three males) with ages ranging between 27 and 42 months (mean: 35.2). Patients presented with intermittent abdominal pain (IAP, all five patients), alternate vomiting (three of five patients), alternate diarrhea (two of five patients), fever (two of five patients), and rectal bleeding (one of five patients). The average length of symptoms was 22.6 days. Eighteen diagnostic studies were performed, including abdominal ultrasound for all patients, barium enema for three patients with secondary ileocolic intussusception, and abdominal computed tomography (CT) for one patient. The average number of studies per patient was 3.6. In surgery, the appendiceal intussusception was found to be complete in four patients, whereas it was partial in the remaining patient. In all patients, appendectomy was performed with resection of a small rim of cecal wall due to marked congestion and edema in an attempt to decrease recurrence., Conclusion:  The mainstay of clinical presentation is intermittent abdominal pain while patients may be completely asymptomatic between attacks. Appendiceal intussusception may act as a leading point to ileocolic intussusception and is frequently concealed by it. The treatment is appendectomy. Both pediatric surgeons and radiologists should be aware of this occurrence to provide adequate management and avoid complications., Competing Interests: Conflict of Interest: None., (Georg Thieme Verlag KG Stuttgart · New York.)

Background: Point-of-care ultrasound (POCUS) is becoming a common tool for routine use in emergency medicine, anesthesiology and intensive care for diagnostic and interventional purposes. When a portable ultrasound device became available for the department of Pediatric and Adolescent Surgery at the Schneider's Children Medical Center of Israel, we added POCUS assessments to the physician's daily rounds. POCUS is performed by pediatric surgeons trained in basic ultrasonography skills. Starting September 2015 all POCUS examinations were documented., Objectives: To describe the current use, diagnostic and therapeutic impacts of POCUS in a department of pediatric and adolescent surgery., Methods: We conducted an observational study of all the documented POCUS procedures performed during a half-year period. Data regarding patient condition and the POCUS procedures were collected, as well as data on the use of other diagnostic modalities, mainly formal ultrasound exams (by radiologists) and computed tomography scans and their correlation with the POCUS assessment., Results: Fifty-one POCUS exams were performed during the study period, most of which served to define the presence and resolution of a collection - intraabdominal (34%) and subcutaneous (31%). Despite a high rate for formal diagnostic studies (65%), probably due to a relative lack of confidence of surgeons performing the POCUS exams during this initial period, most results (92%) were compatible., Conclusions: The ability and availability to perform multiple POCUS exams by the attending physician proved to be a valuable aide to the classical physical and laboratory examinations of surgical patients, and we predict its increasing use in quotidian practice.

Background: Rectobladder neck fistula is the highest and most complex anorectal malformation in boys and the only one that requires an abdominal approach, open or laparoscopic, for repair. The aim of this study was to describe the unique characteristics of rectobladder neck fistulas that warrant special attention and to describe the associated anatomic variants in the genitourinary tract., Methods: The database of a tertiary medical center was retrospectively reviewed for all patients treated for rectobladder neck fistula, by our team in 1980-2011. Data on surgical history, associated and functional defects, treatment and outcome were collected by chart review., Results: The study group included 111 patients. The most common anatomic urologic defect was a single kidney in 37 patients (33.3%) and the most common functional urologic defect was vesicoureteral reflux in 40 patients (36%), including 11/37 patients with a single kidney (29.7%). Of the 40 patients who underwent cystoscopy, 16 (40%) had a higher than normal location of the verumontanum. Follow-up ranged from 2 to 290months (median 59). Urinary continence was achieved in 40 of the 61 patients (65.5%) for whom data were available, and fecal continence was achieved in 9 of the 69 patients (13%) for whom data were available. A sacral ratio of 0.4 or less was associated with lower rates of urinary control (23%) and fecal control (0%), relative to higher ratios. Twenty stomas (18%) were found to be located too distally, limiting the availability of the bowel for a pull through., Conclusions: Rectobladder neck fistula carries a poor prognosis for bowel control and is associated with a high rate of urinary malformations that require long-term care. Pediatric surgeons need to be aware of these complications in order to provide proper treatment and parental counseling. Intra-vesical verumontanum is found in a surprisingly high percentage of patients. The combination of a single kidney with vesicoureteral reflux is common and should be closely followed to avoid renal deterioration. Special attention should be given to colostomy construction to avoid complications and unnecessary procedures. A sacral ratio of 0.4 or less is an indicator of poor fecal and urinary control., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Purpose: To present our experience in abdominal transplantations to manage unresectable abdominal neoplasms in children and to describe the role of extensive surgeries in such cases., Methods: This is a retrospective study of 22 abdominal transplantations in 21 patients for abdominal tumors over 16 years. Transplantation techniques included liver transplant (LT), multivisceral transplant (MVTx), and intestinal autotransplant (IA). Follow-up intervals ranged from 0.3 to 168 months (median 20 months)., Results: LT alone was performed in 15 patients for primary malignant (11) and benign (4) liver tumors. Pathological classification included HB hepatoblastoma (6), HCC hepatocellular cancer (3), hepatic epithelioid hemangioendothelioma HEH (1), angiosarcoma (1), benign vascular tumors (3), and adenoma (1). IA was performed in four patients for lesions involving the root of the mesentery; tumors of the head of pancreas (3) and mesenteric hemangioma (1). MVTx was performed in 2 patients for malignancies; pancreaticoblastoma (1), recurrent hepatoblastoma (1), and in one patient as a rescue procedure after IA failure. Four of the eleven patients who underwent LT for malignant liver tumor had metastatic disease at presentation. Six of them died of recurrent neoplasm (3), transplant-related complications (2), and underlying disease (1). All LT patients who had benign tumors are alive with functioning grafts. All IA patients survived and are on an oral diet, with one patient requiring TPN supplementation. One of the three patients who underwent MVTx died of metastatic disease., Conclusions: Allo/auto transplantation for abdominal tumors is a valuable modality when conventional treatments fail or are not feasible.

Introduction: Anorectal injuries in children are not frequently reported and their management is challenging. This report reviews the experience in managing this type of injuries in two medical centers over 20 years., Methods: An institutional database search for patients who were treated for anorectal injuries between 1994 and 2015 was undertaken. Twenty cases were located and medical records reviewed. This study was conducted with institutional review board approval (#572-14)., Results: There were 6 girls and 14 boys with ages ranging between 1 and 15 years (mean 7 years). Eleven patients sustained penetrating trauma, while nine sustained blunt trauma. The mechanism of injury was variable and associated injuries were more common in blunt trauma. Most common presenting symptoms were rectal bleeding (n = 12) and anal pain (n = 11), followed by abdominal pain in six patients. Eighteen anorectal injuries were extraperitoneal and two intraperitoneal. Among patients with extraperitoneal injuries, 12/18 were managed by primary repair with (6) or without (6) fecal diversion and 2/18 by wound irrigation and drainage with fecal diversion and delayed repair. Four patients had superficial anal and perineal injuries that were irrigated and left to heal by secondary intention. Two patients with intraperitoneal rectal injuries underwent primary repair with fecal diversion. Follow-up period ranged from 2 weeks to 8 years (mean 2 years). There were three cases of wound infection, one case of suture line leak requiring reoperation and one case of vesicorectal fistula in a patient with combined trauma of the rectum and urinary bladder. There was no mortality. Fecal continence was preserved in all patients available for follow-up evaluation., Conclusions: Primary repair of the perineal wound and anal sphincters can be performed safely in most cases given hemodynamic stability. Fecal diversion should be saved for cases with severe perineal involvement or cases with substantial associated injuries and concern of gross contamination.

During a 5-year period 50 children, aged 2 months to 15 years, underwent Nissen fundoplication for the management of severe gastroesophageal reflux and were then followed for from 3 months to 5 years. There were no immediate postoperative deaths. 8 patients (16%) died of serious underlying medical conditions during follow-up. The operation was successful in 86.3% in relation to indications for surgery. Postoperative complications specific for fundoplication occurred in 27 (54%) and other complications in 11 (22%). Of those with complications, 71% were treated successfully. The rest developed dumping syndrome which was diagnosed 1 month to 4 years after operation. They were treated with carbohydrate restriction and a special diet, the results of which will be evaluated later. Although Nissen fundoplication is very successful in resolving the indications for surgery, the high rate of postoperative complications demands re-evaluation of the indications for the operation in children.