Adams Oliver Syndrome: Our One-Year Experience

Yıl 2025, Cilt: 20 Sayı: 3, 223 - 228

Aydın Bozkaya , Salih Davutoğlu

https://doi.org/10.17517/ksutfd.1415076

Öz

Adams-Oliver syndrome, although rare, is a congenital disease commonly characterized by aplasia cutis congenita and terminal limb defects. While small lesions often heal spontaneously, larger lesions may be associated with common fatal anomalies in the gastrointestinal, cardiopulmonary, genitourinary, and central nervous systems
In this paper, we aimed to remind this syndrome, which affects many systems, and to emphasize the importance of the multidisciplinary approach in its treatment. The criteria for Adams-Oliver syndrome have been evaluated in three cases according to the textbooks and the current literature.
In case one, aplasia cutis, nail hypoplasia in bilateral toes, polydactyly, and kutis marmaratus in the right upper extremity, in case two, aplasia cutis, kutis marmaratus, and intrauterine intestinal perforation, and in case three, aplasia cutis, kutis marmaratus, and cystic encephalomalasic changes on cranial MRI have been detected.
Adams-Oliver syndrome is a rare syndrome with various clinical presentations. Hence, in cases presenting with dysmorphic findings and Adams-Oliver syndrome, all systems should be examined. After diagnosis, follow-up and treatment should be performed by the relevant branches with a multidisciplinary approach.

Anahtar Kelimeler

Adams-Oliver sydrome , Aplasia cutis congenita , Terminal limb defects , Hypoplastic phalanges , Extremity malformations

Adams Oliver Sendromu: Bir Yıllık Deneyimimiz

Öz

Adams-Oliver sendromu nadir olmasına rağmen sıklıkla aplaziya kutis konjenita ve terminal ekstremite defektleri ile karakterize konjenital bir hastalıktır. Küçük lezyonlar sıklıkla kendiliğinden iyileşirken, daha büyük lezyonlar gastrointestinal, kardiyopulmoner, genitoüriner ve merkezi sinir sistemindeki yaygın ölümcül anomalilerle ilişkili olabilir.
Bu yazıda birçok sistemi etkileyen bu sendromu hatırlatmayı ve tedavisinde multidisipliner yaklaşımın önemini vurgulamayı amaçladık. Adams-Oliver sendromunun kriterleri ders kitaplarına ve güncel literatüre göre üç olguda değerlendirildi.
Birinci olguda sağ üst ekstremitede aplaziya kutis, iki taraf ayak parmaklarında tırnak hipoplazisi, polidaktili ve kutis marmaratus, ikinci olguda aplaziya kutis, kutis marmaratus ve intrauterin bağırsak perforasyonu ve üçüncü olguda aplaziya kutis, kutis marmaratus ve Kranial MR'da kistik ensefalomalazik değişiklikler tespit edildi.
Adams-Oliver sendromu çeşitli klinik bulgularla seyreden nadir bir sendromdur. Bu nedenle dismorfik bulgular ve Adams-Oliver sendromu ile başvuran olgularda tüm sistemlerin incelenmesi gerekmektedir. Tanı konulduktan sonra takip ve tedavi ilgili branşlar tarafından multidisipliner yaklaşımla yapılmalıdır.

Anahtar Kelimeler

Adams-Oliver sendromu , Aplasia kutis konjenita , Terminal ekstremite defektleri , Hipoplastik falanjlar , Ekstremite malformasyonları

Kaynakça

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