TITLE:
Pterygium Popliteal Syndrome Concerning a Case in the Pediatric Surgery Department of the Donka National Hospital (Conakry CHU)
AUTHORS:
Balla Keita, Mamadou Alpha Toure, Mohamed Lamie Sacko, Mamadou Madiou Barry, Mamadou Karamba Kaba, Daniel Agbo-Panzo
KEYWORDS:
Popliteal Pterygium, Congenital Malformation, Genetic Disease, Pediatric Surgery, Multidisciplinary Team
JOURNAL NAME:
Open Journal of Pediatrics,
Vol.12 No.1,
March
2,
2022
ABSTRACT: Introduction: Popliteal pterygium syndrome is a rare birth defect, combining
craniofacial, genitourinary and musculoskeletal abnormalities. It is an
autosomal dominant disease caused by a mutation in the IRF6 gene. We report in
this observation the 1st Guinean case corrected by the surgical
method as well as a review of the literature for a diagnostic and therapeutic
approach. Patient and observation: We present the case of a 7-day old
male newborn weighing 2700 g who was received for bilateral cleft lip and palate, lower lip fossa or
sinuses, bilateral popliteal pterygium, and triangular skin fold above the
hallux. The patient underwent several surgical procedures aimed at correcting
these abnormalities. The correction of the pterygium of the lower limbs was
ensured by excision of the fibrous band, the tenoplasty in z of the calcaneal tendon on the right side and the skin
plasty in z in series then immobilized by plaster splints. The immediate
postoperative follow-up was straightforward. Conclusion: Popliteal
pterygium syndrome is a rare congenital malformation, the diagnosis is
primarily clinical. Early soft tissue lengthening surgery and serial z-skin
plasty provide better correction of the knee pterygium. Correct correction of
facial abnormalities gives the child a better appearance. The
management of this syndrome is multidisciplinary.