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Objective: Coverage of upper and lower extremity wounds with exposed vital structures such as tendon and bone is reliant on pedicled and free flaps. However, a population of patients with medical comorbidities or other social issues may not be suitable for flap coverage. We present the first in patient clinical experience in the US with MatriDerm (Billerbeck, Germany) for treatment of difficult extremity wounds, all with exposed bone or tendon. Method: MatriDerm dermal matrix has been popular in Europe for coverage of wounds. However, MatriDerm only received full approval for use by the US Food and Drug Administration (FDA) in 2021. Here we review our clinical experience. Results: Locations (n=11) treated included forearm (n=1), hand (n=4), leg (n=3), and feet (n=3). Vital structures in wounds exposed included bone (n=3), bone and tendon (n=1), and tendon (n=7). Mean area of the wound treated was 59.2cm2 (range: 2 to 230). In our series, eight out of 11 wounds healed, with MatriDerm only (n=6) or following delayed skin grafting (n=2). Mean time to healing in the patients treated only with MatriDerm was 49 days (range 7 to 84). Mean time to split-thickness skin grafting in the remaining two patients was 44.5 days (range 32 to 57). Conclusion: MatriDerm has potential for treatment of complex extremity wounds, which would otherwise require flap coverage, in patients who are not good candidates for flap surgery.

Thoracic outlet syndrome (TOS) is a rare condition (1-3 per 100,000) caused by neurovascular compression at the thoracic outlet and presents with arm pain and swelling, arm fatigue, paresthesias, weakness, and discoloration of the hand. TOS can be classified as neurogenic, arterial, or venous based on the compressed structure(s). Patients develop TOS secondary to congenital abnormalities such as cervical ribs or fibrous bands originating from a cervical rib leading to an objectively verifiable form of TOS. However, the diagnosis of TOS is often made in the presence of symptoms with physical examination findings (disputed TOS). TOS is not a diagnosis of exclusion, and there should be evidence for a physical anomaly that can be corrected. In patients with an identifiable narrowing of the thoracic outlet and/or symptoms with a high probability of thoracic outlet neurovascular compression, diagnosis of TOS can be established through history, a physical examination maneuvers, and imaging. Neck trauma or repeated work stress can cause scalene muscle scaring or dislodging of a congenital cervical rib that can compress the brachial plexus. Nonsurgical treatment includes anti-inflammatory medication, weight loss, physical therapy/strengthening exercises, and botulinum toxin injections. The most common surgical treatments include brachial plexus decompression, neurolysis, and scalenotomy with or without first rib resection. Patients undergoing surgical treatment for TOS should be seen postoperatively to begin passive/assisted mobilization of the shoulder. By 8 weeks postoperatively, patients can begin resistance strength training. Surgical treatment complications include injury to the subclavian vessels potentially leading to exsanguination and death, brachial plexus injury, hemothorax, and pneumothorax. In this review, we outline the diagnostic tests and treatment options for TOS to better guide clinicians in recognizing and treating vascular TOS and objectively verifiable forms of neurogenic TOS.

To assess the prognostic capabilities of various diagnostic modalities for childhood brachial plexus injuries (BPIs) and brachial plexus birth injury (BPBI) and postneonatal BPI.In this single-center retrospective cross-sectional study, we examined children with BPIs diagnosed or confirmed by electrodiagnostic studies between 2013 and 2020, and compared the prognostic value of various components of the electrophysiologic findings, magnetic resonance imaging (MRI) data, and the Active Movement Scale (AMS). We developed scoring systems for electrodiagnostic studies and MRI findings, including various components of nerve conduction studies and electromyography (EMG) for electrodiagnostic studies.We identified 21 children (10 females and 11 males) aged 8 days to 21 years (mean 8y 6.95mo) who had a total of 30 electrodiagnostic studies, 14 brachial plexus MRI studies, and 10 surgical procedures. Among the diagnostic modalities assessed, brachial plexus MRI scores, EMG denervation scores, and mean total EMG scores were the most valuable in predicting surgical versus non-surgical outcomes. Correspondingly, a combined MRI/mean total EMG score provided prognostic value.Brachial plexus MRI scores and specific electrodiagnostic scores provide the most accurate prognostic information for children with BPI. Our grading scales can assist a multidisciplinary team in quantifying results of these studies and determining prognosis in this setting.A new scoring system to quantify results of electrodiagnostic and magnetic resonance imaging (MRI) studies is presented. Severity of denervation has good prognostic value for childhood brachial plexus injuries (BPIs). Composite electromyography scores have good prognostic value for childhood BPIs. Brachial plexus MRI has good prognostic value for childhood BPIs.

This review discusses the anatomy and biomechanics of the thumb carpometacarpal (CMC) joint. This articulation between the trapezium and first metacarpal is integral for opposition and other complex movements necessary for pinch and grasp maneuvers. Fortunately, this joint is well equipped to handle the extreme forces imposed by these movements, as it is stabilized by an elaborate arrangement of ligaments and muscles. Without this stability, thumb subluxation would occur with loading during pinch and grasp, and human prehension would be impossible. Understanding the interactions occurring within this joint is essential for adequately treating pathology arising in this crucial joint.

Little is known regarding risk factors specific to pediatric upper extremity firearm injuries. The purpose of this study is to evaluate pediatric patients treated for these injuries to identify at-risk populations and recurring mechanisms of injury. A 20-year retrospective review was conducted. Patients 17 years of age and younger, with upper extremity injuries related to a firearm, were included. Analysis involved Fisher’s exact and Chi-square tests. One hundred and eighty patients were included. The mean age was 12.04 ± 4.3 years. Most included patients were male (85%). Interestingly, females were more frequently victims of assault (P = 0.03), and males were more frequently injured due to accidental discharge (P

The profunda artery perforator flap has numerous advantages. For coverage of extremity wounds, however, traditional subfascial harvest results in a thick flap, which routinely requires secondary thinning. The author describes his approach and experience with thin and superthin profunda artery perforator flaps for extremity reconstruction. With preoperative perforator localization via computed tomography angiogram, color Doppler, and handheld Doppler, the author used a single dominant perforator for extremity reconstruction in 10 consecutive patients. The mean age of patients who had a thin or superthin profunda artery perforator flap for extremity reconstruction was 41.2 ± 21.3 years. Flaps were used to cover wounds in the upper ( n = 3) and lower ( n = 7) extremities. Mean flap thickness was 0.7 ± 0.2 cm. Mean flap artery diameter was 16 ± 3 mm; mean flap vein diameter was 21 ± 6 mm. Mean pedicle length was 6.8 ± 1.2 cm. The difference between the actual measured intraoperative proximal-distal distance and the preoperative computed tomography angiogram measurement for each patient was calculated, with a mean difference of 1.2 ± 0.6 cm. Advantages of the profunda artery perforator flap include a concealed donor site, large perforator caliber, long pedicle length, and a straightforward perforator dissection. Intraoperative measurement of the proximal-distal distance of the dominant perforator fell within 2 cm of the preoperative computed tomography angiogram measurement in all cases. The thin and superthin profunda artery perforator flaps provide new reconstructive options, particularly suited for use in the extremities.Therapeutic, IV.

BACKGROUND The free anterior branch split latissimus dorsi flap has a reliable anatomy and advantages over the traditional latissimus dorsi flap. By preserving the posterior branch of the thoracodorsal nerve, morbidity at the donor site is reduced, preserving shoulder strength. METHODOLOGY The purpose of this article is to review our experience with the split latissimus flap, describe our surgical technique, and finally review representative cases of reconstruction in different anatomical regions. RESULTS From April 2017 to October 2020, 39 free split latissimus flaps were performed at a single center. Flaps were performed for coverage in the upper extremity (n = 2), lower extremity (n = 32), and head and neck (n = 5). Flap success rate was 97.4%. Mean dimensions of the flap were 17.0 × 8.3 cm, with a mean area of 145 cm2. CONCLUSIONS The flap has a broad application and can be utilized in many different reconstructive scenarios including for coverage of defects in the extremities, trunk, and head and neck.

Previous reports suggest that a complex microbiome exists within the female human breast that might contribute to breast cancer etiology. The purpose of this pilot study was to assess the variation in microbiota composition by breast side (left versus right) within individual women and compare the microbiota of normal and breast tumor tissue between women. We aimed to determine whether microbiota composition differs between these groups and whether certain bacterial taxa may be associated with breast tumors. Bilateral normal breast tissue samples (n = 36) were collected from ten women who received routine mammoplasty procedures. Archived breast tumor samples (n = 10) were obtained from a biorepository. DNA was extracted, amplified, and sequenced. Microbiota data were analyzed using QIIME and RStudio. The most abundant phyla in both tumor and normal tissues were Bacteroidetes, Firmicutes, Proteobacteria, and Actinobacteria. There were statistically significant differences in the relative abundance of various bacterial taxa between groups. Alpha diversity (Simpson’s index) was significantly higher in normal compared to tumor samples (0.968 vs. 0.957, p = 0.022). Based on unweighted UniFrac measures, breast tumor samples clustered distinctly from normal samples (R2 = 0.130; p = 0.01). Microbiota composition in normal samples clustered within women (R2 = 0.394; p = 0.01) and by breast side (left or right) within a woman (R2 = 0.189; p = 0.03). Significant differences in diversity between tumor and normal tissue and in composition between women and between breasts of the same woman were identified. These results warrant further research to investigate the relationship between microbiota and breast cancer.

The latissimus dorsi flap is a workhorse for reconstruction. However, flap harvest has been variably reported to result in donor-site morbidity. The aim of this study was to compare donor-site morbidity following harvest of a split latissimus dorsi flap, preserving the anterior branch of the thoracodorsal nerve, and a traditional nerve-sacrificing full latissimus dorsi flap.Patients who underwent split or full latissimus dorsi flaps between July of 2017 and August of 2020 at a single center were recalled for assessment. Donor-site morbidity in the shoulder was evaluated through the Disabilities of the Arm, Shoulder and Hand questionnaire; the Shoulder Pain and Disability Index; and the American Shoulder and Elbow Surgeons questionnaire. Medical Research Council strength grading was also performed.A total of 22 patients in the split latissimus dorsi cohort and 22 patients in the full latissimus dorsi cohort were recalled. Patient-reported outcomes as assessed through the Disabilities of the Arm, Shoulder and Hand questionnaire; Shoulder Pain and Disability Index; and American Shoulder and Elbow Surgeons questionnaire scores revealed statistically greater (p0.05) donor-site morbidity associated with the traditional compared to split latissimus dorsi flap. Seven patients in the full latissimus dorsi cohort had less than Medical Research Council grade 5 power at the shoulder, whereas all patients in the split latissimus dorsi cohort demonstrated full power at the shoulder.Traditional full latissimus dorsi flaps were found to result in greater donor-site morbidity compared to thoracodorsal nerve-preserving split latissimus dorsi flaps. Split latissimus dorsi flaps may be beneficial in preserving donor-site function and strength.Therapeutic, III.

We report a case of myofibroma encasing the ulnar nerve on the medial aspect of the left arm with motor and sensory deficit secondary to compression. Initially, the tumour appeared to be a benign peripheral nerve sheath tumour based on preoperative imaging, with clinical examination positive for left hand clawing and a positive Wartenberg’s and Froment’s sign. However, intraoperative dissection demonstrated that the mass did not originate from the ulnar nerve proper, lowering suspicion for a peripheral nerve sheath tumour. Histopathological analysis showed spindle cell neoplasm, consistent with myofibroma. The patient underwent hand occupational therapy subsequently, with improvement of grip strength from 5 lb to 12 lb by 4 months postoperatively and resolution of clawing of the hand postoperatively. We discuss differentiating features for this rare occurrence of solitary adult myofibroma, where the final diagnosis was only made after formal histopathological analysis.

Peripheral nerve injury (PNI) is a common source of pain and disability in patients. While many patients are affected by PNI, peripheral nerve surgery advancements in the lower extremity have lagged behind the upper extremity. Subsequently, principles that have demonstrated success in the upper extremity have been implemented in the lower extremity. Interventions with recent advances include the advent of novel nerve transfers in the lower extremity and using stem cells and electrical stimulation (ES) for nerve regeneration. This article focuses on advances in nerve transfers for lower extremity PNI and provides details on the basic science and clinical applications of newer interventions.

Diagnosis of trigger sites for migraine surgery relies on history to detect a constellation of symptoms and secondarily, nerve blocks, imaging studies, and Doppler probe examination. The scratch collapse test (SCT) has been described for localization of compressive neuropathies in the upper and lower extremities. In this study, we hypothesized that the SCT could also be used to diagnose trigger sites for surgical planning in migraine surgery. Eleven consecutive patients presenting for migraine surgery, and four patients presenting with recurrent headaches and secondary trigger sites after initial successful migraine surgery were examined with the SCT using a standard protocol to assess involvement of bilateral sites I, II, IV, V, and VI, with the carpal tunnel as a negative control. The SCT was positive bilaterally at sites I, II, IV, V, and VI for all patients presenting primarily for migraine surgery, regardless of trigger sites localized by history and other secondary modalities. The SCT, however, correlated with secondary trigger sites localized through history and examination for patients presenting with recurrent migraine headaches after previous primary surgery. The SCT is not reliable for localization of trigger sites in patients presenting primarily for migraine surgery. This likely relates to central sensitization of migraine headaches, leading to global cutaneous allodynia in the head and neck. In patients with recurrent migraine headaches, abrogation of central sensitization following the initial surgery allows diagnosis of secondary trigger sites through the SCT.

Purpose Nerve transfers for peripheral nerve injuries can result in variable outcomes. We investigated the neuroprotective effect of epineurial lidocaine injection in the donor nerve prior to transection, with the hypothesis that proximal axon loss would be decreased with consequent increased neuroregeneration and functional recovery. Methods A rat sciatic nerve model was used with 4 intervention groups: (1) lidocaine; (2) lidocaine/calcium gluconate (CG); (3) CG; or (4) saline (control). Behavioral testing and qualitative and quantitative histological evaluation was performed at 8 and 12 weeks. Histological assays included transmission electron microscopy, retrograde fluorogold labeling, and whole mount immunostaining. Results Functional assessments through the sciatic functional index and Basso, Beattie, and Bresnahan scale showed a statistically significant increase in recovery at 8 and 12 weeks with lidocaine treatment. Significantly higher axonal counts were obtained in the lidocaine-treated groups. Fragmentation and increased myelin damage was present in the CG and saline groups. Retrograde fluorogold labeling showed a statistically significant increase in the number of L4-6 dorsal root ganglion neurons in the lidocaine-treated groups. Whole mount immunostaining identified extension of the axonal growth cone past the nerve coaptation site in lidocaine-treated groups, but not in CG and saline groups. Conclusions Our results suggest that epineurial lidocaine injection prior to donor nerve transection for nerve transfer has a neuroprotective effect, resulting in increased proximal axon counts and improved functional recovery. Clinical relevance These findings may have direct clinical application because epineurial lidocaine can be used in surgery as a simple and inexpensive intervention for promoting improved clinical outcomes after nerve transfer.

BACKGROUND Diabetes mellitus is a well-known risk factor for infection after trigger finger (TF) injection and/or release. However, the effect of preoperative hypoglycemia before TF injection or release is currently unknown. The purpose of this study is to determine the effects of preoperative hypoglycemia on infection incidence after TF injection or release. METHODOLOGY A retrospective cohort review between 2007 and 2015 was conducted using a national private payer database within the PearlDiver Supercomputer. Preoperative, fasting, glucose levels were collected for each patient, and these ranged from 20 to 219 mg/dL. Surgical site infection (SSI) rates were determined using International Classification of Diseases, Ninth Revision codes. RESULTS The query of the PearlDiver database returned 153,479 TF injections, of which 3479 (2.27%) and 6276 (4.09%) had infections within 90 days and 1 year after procedure, respectively. There were 70,290 TF releases identified, with 1887 (2.68%) SSIs captured within 3 months after surgery and 3144 (4.47%) within 1 year after surgery. There was a statistically significant increase in SSI rates in patients with hypoglycemia within 90-day (P = 0.006) and 1-year (P < 0.001) time intervals post-TF injection. Likewise, a statistically significant increase in SSI rate in patients with hypoglycemia undergoing TF release within 1 year after release was seen (P = 0.003). CONCLUSIONS Hypoglycemia before TF injection or release increases the risk for SSI. Tight glycemic control may be warranted to mitigate this risk. Further studies are needed to investigate the effect of hypoglycemia as an independent risk factor for SSI.

Traditional subfascial harvest of the profunda artery perforator (PAP) flap results in a thick flap, which routinely requires secondary thinning. The aim of this study was to characterize the suprafascial perforator anatomy of PAPs on preoperative computed tomography angiography (CTA) and correlate radiological findings with our clinical experience in extremity reconstruction.Suprafascial PAP anatomy was reviewed in CTAs in 159 thighs. Findings on CTA were correlated with intraoperative findings in a cohort of patients who had a PAP flap for extremity reconstruction.Two main perforator patterns, a "T" (superficial bifurcation) and "Y" (deeper bifurcation) were identified. The ratio between the total skin thickness and the distance from the skin to the perforator bifurcation point and the total skin thickness was higher in the "T" perforator pattern. A dominant "T" perforator (n = 97) was more common than "Y" (n = 62). A dominant "T" perforator was more common in women and with higher body mass index (BMI). In the clinical study, we found a positive correlation between the skin thickness of the bifurcation point of a dominant "T" perforator and the thickness of the superficial fascia where a thin PAP flap is elevated.A dominant "T" perforator on preoperative CTA accurately predicts thickness of a PAP flap elevated at the level of the superficial fascia.

Background We report our findings from an anatomical study on harvest of a vascularized scapular bone graft from a supine position. A clinical case is presented to illustrate the operative approach. Methods Twenty cadaveric hemibody specimens were dissected in the supine position. Outcomes of interest included the characterization of anatomical variants and measurements of pedicle length. Specific measurements included distance from the origin of the subscapular artery (at the axillary artery) to the branch point of the angular artery from the thoracodorsal artery or serratus branch and the length of the angular branch proper. Results There are five reported anatomic variations regarding the origin of the angular branch of the thoracodorsal artery. In our cadaveric cohort only four known types were seen, and an entirely new variant was encountered. Six cadaveric dissections exhibited a type 3 configuration, six were type 1, four were type 2, three were type 4, and one was a previously unreported variant we termed a type 6, with multiple angular artery branches originating from the posterior branch of the thoracodorsal. The mean distance between the origin of the subscapular artery and the takeoff of the angular branch was 6.3 ± 2.0 cm. The mean length of the angular branch was 3.7 ± 1.4 cm. Conclusions Supine positioning for harvest of a vascularized bone graft obviates the need for an intraoperative position change and allows reconstruction of bone defects in the hand and upper extremity within a single surgical field.

Nonsyndromic congenital mitten hand with thumb and index finger syndactyly is rare. Reconstruction of this condition is challenging, requiring repositioning of the thumb and creation of a wide and deep first webspace. The aim of this paper is to describe the characteristics of patients with this condition and also to describe our surgical technique.We describe our technique in 16 consecutive children utilizing complete mobilization of the first metacarpal and thumb as an island flap on the neurovascular bundle, with a rotational osteotomy to reposition the thumb in pronation. In addition, a dorsal M flap was used to reconstruct the first webspace. Patients in this series also had varying degrees of symbrachydactyly with hypoplastic or aplastic phalanges of the central digits.There were 9 male and 7 female patients, with a mean age of 24.4 ± 22.4 months at the time of surgery. Mean follow-up was 19.1 ± 18.3 months. All reconstructions healed well. The mean thumb web distance in the affected hands was 4.2 cm (range 3.5 to 5). All reconstructed thumbs had a good opposition. Two children initially had mild hypertrophic scarring, which resolved with pressure gloves. No other complications were observed.One-stage reconstruction of the thumb and first webspace can be effectively achieved with our technique of complete mobilization of the thumb unit, rotational osteotomy, and a dorsal interdigitating M flap.

BACKGROUND The free split latissimus dorsi flap for lower-extremity reconstruction has some advantages over the traditional latissimus dorsi flap. The flap is harvested with the patient in the supine position and is associated with minimal morbidity as the function of the remaining latissimus dorsi muscle is preserved through the posterior division of the thoracodorsal nerve. METHODS A consecutive single-surgeon 5-year series of free split latissimus dorsi muscle flaps for lower-extremity reconstruction (n = 42) was evaluated. Donor site morbidity was evaluated through assessment of the strength of the remaining latissimus dorsi at least 1 month after surgery. Shoulder function was evaluated postoperatively using the Disabilities of the Arm, Shoulder and Hand (DASH) score, American Shoulder and Elbow Surgeons (ASES) score, and Shoulder Pain and Disability Index (SPADI). RESULTS The mean age of the 42 patients was 40.7 years. The mean length and width of the flaps were 17.9 cm and 8.6 cm. The majority (71%) of the wounds were due to acute trauma. Of the 42 flap procedures performed, 95% (40) were successful. Assessment of remaining latissimus dorsi strength at least 1 month postoperatively, during 3 activities, showed a Medical Research Council (MRC) grade of 5 in all patients. The mean and median scores were 6.4 and 0 according to the DASH, 6.0/6.4 and 0/0 on the SPADI pain/disability scales, and 90.7 and 100 on the ASES. CONCLUSIONS The free split latissimus dorsi flap is a large reliable muscle flap with negligible donor site morbidity that is particularly advantageous for lower-extremity resurfacing following trauma. LEVEL OF EVIDENCE Therapeutic Level IV. See Instructions for Authors for a complete description of levels of evidence.

Aim: Targeted muscle reinnervation (TMR) is a procedure pioneered to improve control of myoelectric prostheses and was fortuitously found to improve postamputation pain by transferring residual nerve ends from an amputated limb to reinnervate motor nerve units in denervated muscles. This study sought to perform a systematic review of the literature regarding the postamputation pain-related outcomes following TMR. Methods: PubMed database was queried using the key term “targeted muscle reinnervation”. Articles were chosen based on the following criteria: (1) clinical studies on TMR; (2) greater than one subject; (3) studies were case-controls, comparative cohort analyses, controlled trials, or randomized controlled trials; and (4) studies included one or more outcomes of interest: prosthetic use and functionality, improvement or persistence of pain, indications, complications, donor nerves, and technical aspects of TMR. Results: Overall, 9 studies including 101 upper extremity and 252 lower extremity nerve transfers were analyzed, with nerve transfer type, amputation location, and specific neurotizations reported. Four studies assessed the efficacy of TMR in addressing phantom limb pain (PLP) and residual limb pain (RLP), with 3 out of 4 studies reporting significant improvements in PROMIS (Patient Reported Outcome Measurement Information System) scores in TMR subjects compared to controls. Five additional studies did not analyze PROMIS scores but reported subjective improvements in pain outcomes. Conclusion: Included studies demonstrated TMR had lower maximal pain and pain intensity, behavior and interference compared to the standard of care. Secondary TMR used to treat patients with established painful neuromas also reported improvement in pain compared to baseline.

Nerve transfers for peripheral nerve injuries have become increasingly popular over the past two decades. While techniques for ulnar nerve repair have been well-documented, more recent techniques for median and radial nerve branch reinnervation are still being explored. This review describes the outcomes of common and emerging techniques for reinnervation of the distal branches of the median and radial nerves.

We report the results of ten consecutive patients who had correction of an elbow flexion contracture of greater than 30° in brachial plexus birth injury using a modified Outerbridge-Kashiwagi procedure. All patients had minimum 23-month follow-up. Pre- and post-operative elbow range of motion and DASH scores were recorded in all patients. The operative technique for the procedure and post-operative course is discussed. Surgery was supplemented by botulinum toxin injection into the biceps brachii muscle in most cases. The average age at surgery was 14 years 10 months. The initial plexus lesion was global in eight patients and upper in two. Pre-operative flexion contractures averaged 51° (range 35 to 60) and post-operative averaged 21° (range 15 to 30). Of these patients, one had no change in active flexion, four had loss of active flexion, and five had gain of active flexion. All ten patients were satisfied with their results and stated that they would recommend the procedure to other patients.Level of evidence: IV

Background Tissue expansion, while a mainstay of reconstruction for pediatric cutaneous lesions, has significant complication rates. The authors review the complications in a single-surgeon series of tissue expansion to identify risk factors for complications and guide subsequent therapy so that reconstructive goals in patients can be met irrespective of intervening complications. Methods A retrospective chart review was conducted of all pediatric patients who underwent tissue expansion performed by the senior author (A.K.G.) over a 12-year period. In total, 282 expanders were placed in 94 patients. Results A total of 65 complications occurred in 39 of 94 patients (41.5 percent), involving 65 of the 282 expanders (23.0 percent) placed. Major complications that required expander removal included exposure (n = 11), rupture (n = 15), and migration (n = 11). The most frequent minor complications, which did not require immediate expander removal, included migration (n = 13) and port malfunction (n = 9). The majority of expanders were placed in the scalp (n = 114), followed by the torso (n = 100), face and neck (n = 52), and the extremities (n = 16). Serial expansion beyond the second round resulted in a marked increase in complications. Despite complications, tissue expansion in the majority of patients could be salvaged, and a satisfactory outcome was achieved. Conclusions Families must be made aware that approximately one-third of patients may have a complication requiring additional surgery or modification of the initial reconstructive plan. However, these complications need not preclude attainment of reconstructive goals. Clinical question/level of evidence Therapeutic, IV.

Objective: The present study investigates the efficacy of performing a “palate rerepair” utilizing a double-opposing z-palatoplasty (DOZ) following primary Furlow palatoplasty. Design: Retrospective study. Setting: Tertiary referral academic center for craniofacial surgery. Patients: 15 consecutive patients who presented with velopharyngeal insufficiency (VPI) after primary Furlow palatoplasty. Main Outcome Measures: All subjects were evaluated using the perceptual speech assessment (PSA) scale. Criteria for inclusion in the study were (1) velopharyngeal gap size on phonation of 7 mm or less and (2) lateral wall motion at least 40% normal. Results: Mean PSA score was 7.13 + 3.31 (range 3-13) preoperatively, and decreased to 1.80 + 2.83 (range 0-11; P < .001 vs baseline) 3 months or more after surgery. A sphincter pharyngoplasty was performed as a tertiary procedure in 2 patients due to persistent nasal air emission. There was no symptomatic airway compromise following secondary or tertiary management in all patients. Presence of a cleft lip, lateral wall motion, and velopharyngeal gap size did not impact outcomes. Conclusions: We have demonstrated that a DOZ performed secondarily is anatomically possible following Furlow palatoplasty and can restore function of the levator muscle despite significant scarring following primary repair. This approach respects anatomic principles of palatoplasty without eliminating the possibility for extrapalatal procedures should velopharyngeal competence not be achieved.

Highlights • Myoepithelial carcinoma of the vulva and epithelioid sarcoma has overlapping histological, immunohistochemical and genetic features. • Similarities between two diagnoses in aggressive vulvar cancer should be noted as a sarcoma-based chemotherapy regimen should be considered. • We recommend immediate surgical resection with bilateral superficial and deep inguinal lymph node dissections in these aggressive cancers. • Identification of SMARCB1-deficiency trials of targeted therapies such as EZH2 inhibitors which show promise in halting further progression., Introduction Myoepithelial carcinoma and proximal-type epithelioid sarcoma of the vulva are two rare malignancies with known aggressive behavior. In addition to a similar clinical course, these two disease entities also have significant histologic and pathologic overlap. Given the rarity of these malignancies, there is limited literature on the appropriate treatment regimen. Nevertheless, there is a consensus that early surgical resection is beneficial in both cases. Presentation We present a case of a patient who was initially diagnosed with myoepithelial carcinoma of the vulva with a differential later expanded to include possible epithelioid sarcoma. Discussion We demonstrate the importance in early identification of a SMARCB1 deficiency. Additionally, we suggest an appropriate treatment regimen for these patients going forward. Specifically, we encourage consideration of bilateral superficial and deep inguinal lymphadenectomies. Furthermore, sarcoma based chemotherapy regimens in the appropriate clinical setting may be beneficial in treating SMARCB1 deficient tumors. Additionally, there are now clinical trials involving EZH2 Inhibitors which may offer benefit for similar patients going forward. Conclusion This case highlights the difficulty in making a definitive diagnosis, and the importance in identifying a SMARCB1 deficiency as it will affect treatment options and may allow for enrollment in ongoing clinical trials.