A Neglected Case of Popliteal Pterygium Syndrome Presenting in Adulthood

Shilpi, Karmakar; Brijesh, Mishra

doi:10.23937/2474-3682/1510159

Clin Med Img Lib

2020

DOI: 10.23937/2474-3682/1510159

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A Neglected Case of Popliteal Pterygium Syndrome Presenting in Adulthood

Karmakar Shilpi¹,

Mishra Brijesh²

Abstract: Popliteal Pterygium Syndrome (PPS) is autosomal dominant syndrome with incomplete penetrance and variable expressivity. We report a case of 18-year-old female, who had repair of cleft of lip and palate in childhood, and now presented with paramedian bilateral lower lip pits. Examination revealed her to be a case of sporadic PPS. A comprehensive plan of management was formed, but the patient was lost to follow-up. We report the case for the purpose of educating the clinicians of this rare syndrome and highlight… Show more

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Orthopedic Surgical Management of Complicated Congenital Popliteal Pterygium Syndrome: A Case Report

Hasan¹,

Muhammad²,

Cein³

et al. 2023

ORR

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Introduction Popliteal pterygium syndrome (PPS) is a rare autosomal-dominant condition that causes fixed flexion deformity of the knee. The popliteal webbing and shortening of the surrounding soft tissue could limit the functionality of the affected limb unless it is surgically corrected. We reported a case of PPS in a pediatric patient encountered in our hospital. Case A 10-month-old boy came with a congenital abnormally flexed left knee with bilateral undescended testis and syndactyly of the left foot. The left popliteal pterygium extending from the buttock to the calcaneus was observed, with an associated fixed flexion contracture of the knee and equine position of the ankle. Normal vascular anatomy was seen in the angiographic CT scan; therefore, multiple Z-plasty and fibrotic band excision were performed. The sciatic trunk was exposed on the popliteal level, and the fascicular segment was excised from the distal stump and sutured to the proximal stump under the microscope to extend the sciatic nerve for approximately 7 cm. No postoperative complications were reported. Multiple tendons and soft tissue reconstruction were performed when the patient was 2-year-old to correct the adductus and equine deformity of the left foot. Discussion Surgical correction for popliteal pterygium demands staged techniques to deal with the shortened structure. In our case, multiple Z-plasty were performed, and the fibrotic band was excised until its base with meticulous consideration of the underlying neurovascular bundle. Fascicular shifting technique for sciatic nerve lengthening can be considered in unilateral popliteal pterygium with difficulty extending the knee due to shortened sciatic nerve. The unfavorable outcome of nerve conduction disturbance resulting from the procedure may be multifactorial. Still, the existing foot deformity, including a certain degree of pes equinovarus could be treated by multiple soft tissue reconstructions and adequate rehabilitation to achieve the desired outcome. Conclusion Multiple soft tissue procedures resulted in acceptable functional outcomes. However, the nerve grafting procedure is still a challenging task. Further study is required to explore the technique in optimizing the nerve grafting procedure for popliteal pterygium.

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Orthopedic Surgical Management of Complicated Congenital Popliteal Pterygium Syndrome: A Case Report

Hasan¹,

Muhammad²,

Cein³

et al. 2023

ORR

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show abstract

Pterygium Popliteal Syndrome Concerning a Case in the Pediatric Surgery Department of the Donka National Hospital (Conakry CHU)

Touré¹

2022

OJPed

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A Neglected Case of Popliteal Pterygium Syndrome Presenting in Adulthood

Cited by 2 publications

References 3 publications

Orthopedic Surgical Management of Complicated Congenital Popliteal Pterygium Syndrome: A Case Report

Orthopedic Surgical Management of Complicated Congenital Popliteal Pterygium Syndrome: A Case Report

Pterygium Popliteal Syndrome Concerning a Case in the Pediatric Surgery Department of the Donka National Hospital (Conakry CHU)

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