Emrah DOĞAN, Süha GÜL, Neşat ÇULLU, Marwa MOULİNE DOĞAN

Inkomplet fibular hemimelia’ya eşlik eden tarsal koalisyon, pes planus, ball-socket ayak bileği deformitesi olgusu

Fibular hemimelia (FH), hafif fibular hipoplazi’den fibular aplaziye kadar uzanan klinik spektrumu olan bir konjenital hastalıktır. Kanıtlanmış bir genetik faktör yoktur. Tarsal koalisyon, ulnar hemimelia, amelia, sindaktili, kardiyak anomaliler, renal anomaliler ve çeşitli ekstremite anomalileri anomaliye eşlik edebilir. On dört yaşında kız olguda sağda tek taraflı ve parsiyel tipte FH mevcuttu. Tibia kavisli (eğimli), kısa ve ekstemiteler arasında uzunluk bakımından uyumsuzluk mevcuttu. Osseöz formda tarsal koalisyon deformiteye eşlik etmekteydi. Fibula distal parçası mevcuttu. Aynı taraf ayak bileğinde ball-socket deformitesi saptandı. Kalkaneal eğim açısı 1200 idi. Bulgular pes planus ile uyumluydu.

Anahtar Kelimeler:

Fibular hemimelia, Tarsal koalisyon, Fibular hipoplazi

Case of incomplete fibular hemimelia with tarsal coalition, pes planus, ball and socket ankle

Fibular hemimelia (FH) is a congenital disease with a clinical spectrum ranging from mild fibular hypoplasia to fibular aplasia. There is no proven genetic factor. Some anomalies can accompany FH such as tarsal coalition, ulnar hemimelia, amelia, syndactyly, several extremity anomalies, renal anomalies and cardiac anomalies. Our case is about unilateral and incomplete type of right-side FH in a 14 years old female patient. Tibia was curved (bowing) and short. Disparity of measure with left lower extremity was monitored. Tarsal coalition in osseous form, tibial curve anomaly and small bone part placed in fibula distal region compatible with FH, were visualized. There was curved joint form in the same ankle with hemimelia compatible with ball and socket ankle deformity. Calcaneal inclination angle was 120. The findings were compatible with pes planus.

Keywords:

Fibular hemimelia, Tarsal coalition, Fibular hypoplasia,

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