Hasan Meirizal, Muhammad Hilmi, Cein C Rayhan, Ahmad Husein, Baskara Anak Agung Ngurah Nata
Department of Orthopedic and Traumatology, Sardjito General Hospital / Faculty of Medicine, Public Health and Nursing, Universitas Gadjah Mada, Yogyakarta, Indonesia.
Division of Pediatric Orthopedic, Department of Orthopedic and Traumatology, Sardjito General Hospital / Faculty of Medicine, Public Health and Nursing, Universitas Gadjah Mada, Yogyakarta, Indonesia.
Orthop Res Rev. 2023 Mar 24;15:47-57. doi: 10.2147/ORR.S392024. eCollection 2023.
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.
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.
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.
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.
腘窝翼状胬肉综合征（PPS）是一种罕见的常染色体显性疾病，可导致膝关节固定性屈曲畸形。腘窝蹼状瘢痕及周围软组织缩短会限制患侧肢体的功能，除非进行手术矫正。我们报告了在我院接诊的1例小儿PPS病例。
一名10个月大的男孩因先天性左膝关节异常屈曲、双侧隐睾及左足并趾前来就诊。可见左侧腘窝翼状胬肉从臀部延伸至跟骨，伴有膝关节固定性屈曲挛缩及马蹄足畸形。血管造影CT扫描显示血管解剖结构正常；因此，实施了多次Z成形术及纤维束带切除术。在腘窝水平暴露坐骨神经干，在显微镜下从远端残端切除束状节段并缝合至近端残端，使坐骨神经延长约7厘米。未报告术后并发症。患者2岁时进行了多次肌腱和软组织重建，以矫正左足内收和马蹄畸形。
腘窝翼状胬肉的手术矫正需要分阶段技术来处理缩短的结构。在我们的病例中，实施了多次Z成形术，并切除纤维束带直至其基部，同时仔细考虑其下方的神经血管束。对于因坐骨神经缩短导致膝关节伸展困难的单侧腘窝翼状胬肉，可考虑采用束状移位技术延长坐骨神经。该手术导致神经传导障碍的不良后果可能是多因素的。不过，现有的足部畸形，包括一定程度的马蹄内翻足，可通过多次软组织重建和适当的康复治疗来达到预期效果。
多次软组织手术取得了可接受的功能结果。然而，神经移植手术仍然是一项具有挑战性的任务。需要进一步研究探索优化腘窝翼状胬肉神经移植手术的技术。
