Zimmermann-Laband Syndrome: Vaka Raporu

Year 2025, Volume: 4 Issue: 1, 15 - 20, 29.01.2025

Gürkan Raşit Bayar , Kerim Ortakoğlu , H. Aykut Özyiğit

Abstract

Zimmerman-Laband Sendromu (ZLS), gingival fibromatozis, kraniofasiyal anormaliler ve uzuv deformiteleri ile karakterize edilen nadir bir genetik hastalıktır. Bu vaka raporu, ZLS'nin klasik ve özgün özellikleriyle başvuran 21 yaşında bir erkek hastayı tanımlamaktadır. Hasta, anterior dişlerin sürmemesi nedeniyle sevk edilmiştir. Klinik muayene, gingival fibromatozsiz, hipoplastik tırnaklar ve geniş, düz bir burun ile uyumluydu. Ek bulgular arasında doğuştan gelen kıvrımlı parmaklar ve anterior open-bite yer alıyordu. Radyografik değerlendirme, birden fazla gömülü ve süpernümerer dişi ortaya koymuştur. Hastanın çürük ve süpernümerer dişleri çekilmiştir. Sürmeyen dişler ve anterior open-bite için ortodontik ve cerrahi müdahaleler planlanmıştır. Bu rapor, ZLS'nin fenotipik spektrumunu genişleterek, daha önce bildirilmeyen kıvrımlı parmaklar ve çekiç parmaklar gibi bulguları tanımlamaktadır. ZLS hastalarında sonuçların iyileştirilmesi için kapsamlı bir değerlendirme ve multidisipliner yaklaşım gereklidir.

Keywords

Zimmermann-Laband Sendromu, gingival fibromatozis, anterior open-bite, gömülü diş, konjenital anomaliler

Zimmermann-Laband Syndrome: A Case Report

Abstract

Zimmerman-Laband Syndrome (ZLS) is a rare genetic disorder characterized by gingival fibromatosis, craniofacial abnormalities, and limb deformities. This case report describes a 21-year-old male presenting with classical and unique features of ZLS. A male patient was referred for failure of anterior teeth eruption. Clinical examination revealed gingival fibromatosis, hypoplastic toenails, and a broad, flat nose. Additional findings included congenital curly toes and an anterior open bite. Radiographic evaluation identified multiple impacted and supernumerary teeth. The patient underwent extraction of carious and supernumerary teeth. Orthodontic and surgical interventions were planned for managing unerupted teeth and anterior open bite. This report expands the phenotypic spectrum of ZLS, describing previously unreported findings such as curly toes and hammer toes. Comprehensive evaluation and interdisciplinary management are essential for optimizing outcomes in ZLS patients.

Keywords

Zimmerman-Laband syndrome, gingival fibromatosis, anterior open bite, impacted teeth, congenital anomalies

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