Surgical Management of Sirenomelia: A Case Study.

Background: Sirenomelia is a rare congenital condition characterized by fusion of the lower limbs. Patients with sirenomelia generally do not survive long after birth because the condition is associated with multisystem organ dysfunction due to developmental anomalies. Considering the low incidence and few cases surviving the neonatal period, there is minimal understanding regarding the surgical management of sirenomelia. We present a unique case of an infant born with type 1 sirenomelia, absence of external genitalia, presence of a cloaca, absence of the bladder, and presence of an imperforate and vestigial anus, who not only survived the birth process, but, at the age of 11 months, was determined to be a candidate for surgical separation of the lower extremities. Methods: This case was approached much like a dorsal rectangular flap syndactyly release. Large Z-plasty flaps were designed and raised, and the soft tissue within the skin bridge was meticulously dissected to preserve anatomy and to provide adequate skin flaps without perineal skin grafting. A quadrangular flap was designed to reconstruct the perineum and produce a neo-vulva using de-epithelialization. Results: Successful lower extremity separation was achieved. There were no major postoperative complications. The patient progressed with lower extremity function, and eventually achieved independent ambulation. Conclusions: Management of sirenomelia is incredibly challenging, and data to guide surgical management are limited. This report details our approach to a successful lower extremity separation, repair, and neo-vulvar reconstruction in a case of type I sirenomelia.

Contralateral C7 nerve transfer for severe pediatric brachial plexus injuries: donor site morbidity.

BACKGROUND: Pediatric brachial plexus injuries (BPI) can have a devastating impact on upper extremity function. With localized lesions, nerve grafting and transfers are well-described. However, reconstruction of pan-plexus (C5-T1) injuries (PPI) requires donor nerves outside of the brachial plexus. The cross C7 (CC7) nerve transfer extended with sural nerve grafts to the contralateral recipient nerve offers the advantage of supplying robust donor axons. Though controversial in the West, CC7 transfer is routine in many Asian centers. We present a case series of pediatric patients who underwent CC7 transfer for BPI. Our objective was to catalog donor site morbidity incurred by transferring the C7 nerve root. METHODS: This retrospective study was approved by the Institutional Review Board of our university. INCLUSION CRITERIA: patients under 18 years old that underwent CC7 nerve transfer for BPI at our health system between 2021 and 2022. A chart review was completed to collect demographic and outcomes data. RESULTS: Three patients underwent a complete CC7 transfer between 2021 and 2022 for BPI reconstruction. All patients underwent concomitant additional nerve transfers. Post-operative donor site sensory deficits were minimal and transient in all but one patient, who reported mild but persistent paresthesia of the donor side hand with movement of recipient side digits; however, no patients suffered donor site motor deficits (Table 1). CONCLUSIONS: We conclude that CC7 nerve transfer is a safe surgical option to provide additional donor motor axons for PPI in pediatric patients.

Power-Optimizing Repair for Distal Biceps Tendon Rupture: Stronger and Safer.

Purpose: Many approaches have been described to accomplish tendon reattachment to the radial tuberosity in a distal biceps tendon rupture, with significant success, but each is associated with potential postoperative complications, including posterior interosseous nerve (PIN) injury. To date, there has been no consensus on the best approach to the repair. The purpose of this study was to evaluate the supination strength and the distance of drill exit points from the PIN in a power-optimizing distal biceps repair method and compare the findings with those of a traditional anterior approach endobutton repair method. Methods: Cadaveric arms were dissected to allow for distal biceps tendon excision from its anatomic footprint. Each arm was repaired twice, first with the power-optimizing repair using an anterior single-incision approach with an ulnar drilling angle and biceps tendon radial tuberosity wraparound anatomic footprint attachment, then with the traditional anterior endobutton repair. Following each repair, the arm was mounted on a custom-built testing apparatus, and the supination torque was measured from 3 orientations. The PIN was then located posteriorly, and its distance from each repair exit hole was measured. Results: Five cadaveric arms, each with both the repairs, were included in the study. On average, the power-optimizing repair generated an 82%, 22%, and 13% greater supination torque than the traditional anterior endobutton repair in 45° supination, neutral, and 45° pronation orientations, respectively. On average, the power-optimizing repair produced drill hole exit points farther from the PIN (23 mm) than the traditional anterior endobutton repair (14 mm). Conclusions: The power-optimizing repair provides a significantly greater supination torque and produces a drill hole exit point significantly farther from the PIN than the traditional anterior endobutton approach. Type of study/level of evidence: Therapeutic III.