有范德武德综合征家族史的腘窝翼状胬肉综合征:病例报告。

Jiten Kumar Mishra, Shamendra Anand Sahu, Aparajita Saha, Abi Sindhuja, Jalaz Joezer Rahmi, Abhijith Valsalan
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引用次数: 0

摘要

腘窝翼状胬肉综合征(PPS)是一种罕见的遗传性疾病,涉及面部、四肢和生殖器。腘窝翼状胬肉是与该综合征相关的最突出的残疾畸形。腘窝翼状胬肉是一条挛缩带,从跗骨结节延伸到小腿骨。它是一种致密的纤维带,很难通过手术矫正。我们报告了一例腘窝翼状胬肉患者,她的另外两名家庭成员被诊断为范德沃德综合征。由于神经血管和周围软组织缩短,单阶段手术松解无效。手术松解的多模式治疗和家长的积极配合可能有助于这些患儿的康复。
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Popliteal Pterygium Syndrome with a Family History of Van der Woude Syndrome: A Case Report.

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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