AI Article Synopsis
- Popliteal pterygium syndrome (PPS) is a rare genetic condition affecting the face, limbs, and genital areas, characterized mainly by a restrictive band known as popliteal pterygium.
- This band creates a contracture that runs from the ischial tuberosity to the calcaneum, making surgical correction challenging due to its dense fibrous nature.
- The report discusses a case where a PPS patient has family members diagnosed with Van der Woude syndrome, highlighting that a combination of surgery and strong parental support is necessary for effective rehabilitation since single-stage surgeries are often unsuccessful.
Article Abstract
Popliteal pterygium syndrome (PPS) is a rare inherited disorder involving the face, limbs, and genitalia. The most prominent and handicapping deformity associated with this syndrome is Popliteal pterygium. Popliteal pterygium is a contracture band that extends from ischial tuberosity to calcaneum. It is a dense fibrous band difficult to correct surgically. We report a case of PPS with her two other family members diagnosed with Van der Woude syndrome. Single-stage surgical release is ineffective due to shortened neurovascular and surrounding soft tissues. Multimodality treatment with surgical release and motivated parents may help these children to rehabilitate.
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Source |
|---|---|
| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC11412588 | PMC |
| http://dx.doi.org/10.4103/jwas.jwas_173_23 | DOI Listing |
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