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Management of a complex abdominal wall malformation in a newborn: a rare case report
Syrine Laribi 1, Myriam Ben Fredj 1, Samia Belhssan 1, Afef Toumi 1, Sawsen Chakroun 2, Maha Ben Mansour 2, Amine Ksia 1, Mongi Mekki 1, Mohsen Belghith 1, Lassaad Sahnoun 1
1 Pediatric surgery department, Fattouma Bourguiba Hospital, Monastir, Tunisia
2 Pediatric anaesthesia and intensive care department, Fattouma Bourguiba Hospital, Monastir, Tunisia
Introduction:
Aplasia cutis congenita (ACC) is a rare congenital disorder characterized by localized or widespread absence of skin, sometimes involving deeper tissues. Its etiology is multifactorial and remains incompletely understood. Frieden classified ACC into nine subtypes; type IV, associated with embryologic malformations, is among the rarest and least described in the literature.
Case Presentation:
We report the case of a full-term male newborn delivered via cesarean section due to maternal congenital cataract. Antenatal ultrasound revealed a 4×3.5 cm abdominal wall defect suggestive of an omphalocele. Postnatal examination showed a minor omphalocele surrounded by translucent erythematous skin lesions, consistent with ACC. Additional findings included pubic depression, a megameatus, epithelialized skin defects on the dorsal penis and left thigh, as well as oligodactyly and syndactyly of the right limbs. Imaging (abdominal, transfontanellar, renal ultrasounds, and echocardiography) revealed no internal anomalies. Cutaneous biopsy confirmed the diagnosis of ACC. Conservative local treatment with Silvaderma and antiseptics led to complete epithelialization within two months.
At 6 month of age, the patient underwent a complete repair of the omphalocele. This involved a single-stage aponeurotic closure of the defect, an abdominal skin plasty, and an omphaloplasty using an inverted rectangular lateral skin flap with preserving its vascularization. He is scheduled for a plasty to address pelvic depression caused by adipose tissue aplasia, as well as a plasty of the dorsal aspect of the penis.
Conclusion:
This case represents a rare presentation of type IV ACC associated with omphalocele and limb anomalies, but no internal malformations. Conservative treatment proved effective for skin healing, and staged surgery addressed residual anatomical defects. The prognosis remains favorable in the absence of systemic involvement.