Focal dermal hypoplasia accompanying to Adams-Oliver syndrome

Key Findings & Data:Our case had parietal alopecia with scalp defect, hypopiastic fingers and toes nail abnormalities, periventriculary calcifications on cranial CT, atrophic plaque associated with focal dermal hypoplasia.

💡

Conclusion & Impact:This case is presented because focal dermal hypoplasia accompanied with Adams-Oliver syndrome was not previously reported.

🔬Design:Research Article

📍Scope:Comprehensive Sample

⚙️Variables:Domain Parameters

Abstract

Adams-Oliver syndrome is a condition with scalp defects, limb and cardiovâsculer system abnormalities. Our case had parietal alopecia with scalp defect, hypopiastic fingers and toes nail abnormalities, periventriculary calcifications on cranial CT, atrophic plaque associated with focal dermal hypoplasia. This case is presented because focal dermal hypoplasia accompanied with Adams-Oliver syndrome was not previously reported.

Özet

Adams-Oliver sendromu doğumsal kafa derisi defektleri, bazen duraya kadar ulaşan derin kapanma defektleri, ekstremite ve kardiovasküler sistem anomalilerinin görüldüğü bir sendromdur. Pariyetal alopesi ortasında deri defekti, el ve ayak parmaklarında hipoplazi, tırnak anomalileri, kranial BT'sinde periventrikuler kalsifikasyon ve batında fokal dermal hipoplazi ile uyumlu atrofik plak olan olgu literatürde fokal dermal hipoplazinin eşlik ettiği Adams-Oliver sendromuna rastlanılmadığı için sunulmuştur.

🔖

Cite This Article

ALTINCIK, A., AKDİLLİ, A., TÜRKMEN, M., ÇOPCU, E., USLU, M., ULUCAN, H. (2022). Focal dermal hypoplasia accompanying to Adams-Oliver syndrome. Ege Pediatri Bülteni, Cilt: 13 - Sayı: 2, 115–118. https://ajindex.com/articles/495730

📄 Full Text PDF Viewer

Open in New Tab ↗

↗New Tab

📄

PDF görüntüleyici hazırlanıyor...

References (1)

htpp://www,ncbi.nlm.nih.gov/entrez/dispomirn.cgi?id=100300. 2.Scribanu, N, Temtamy, SA. Syndrome of aplasia cutis congenita with terminal transverse defects of limbs. J Pediatr 1975; 87: 79-82. 3.Kuster W, Lenz W, Kaariainen H, Majewski F. Congenital scalp defects with distal limb anomalies (Adams-Oliver syndrome); report of ten cases and review of the literature. Am J Med Genet 1988;31: 99-115. 4.Toriello HV, Graff RG, Florentine MF, Lacina S, Moore WD. Scalp and limb defects with cutis marmorata telangiectatica congenita: Adams- Oliver Syndrome ? Am J Med Genet 1988; 29: 269-276. 5.Fereira-Da-Silva L, Leal F, Santos GC, Videira Amaral JM, Feijoo MJ. Clinical evidence of vascular abnormalities at birth in Adams-Oliver syndrome: report of two further cases. Am J Med Genet 2000; 94: 75-76. 6.Piazza AJ, Dwain B, Augosto S. A case of Adams-Oliver syndrome with associated brain and pulmonary involvement. Am J Medic Genet 2004; 130: 172-175. 7.Zapata HH, Sletten LJ, Pierpont ME. Congenital cardiac malformations in Adams-Oliver Syndrome. Clin Genet 1995; 47: 80-84. 8.Swartz EN, Sanatani S, Sandor GG, Schreiber RA. Vascular abnormalities in Adams-Oliver Syndrome: Cause or effect ? Am J Med Genet 1999; 82: 49-52. 9.Caksen H, Kurtoğlu S. A case of Adams-Oliver syndrome associated with acrania, microcephaly, hemiplegia, epilepsy and mental retardation. Acta Neural Belg 2000; 100: 252-55. 10.Qomes LB, Castro J, Matos M, Nunes A, Furtado J, Barrueco MC. Lesions of central nervous system in Adams-Oliver's syndome. Acta Med Port 2001; 14: 89-94. 11.Romani J, Puig L, Aznar Q, Demestre X, Altirriba O, Alomar A, Adams-Oliver syndrome with.unusual central nervous system alterations. Pediatr Dermatol 1998; 15: 48-50. 12.Patel MS, Taylor GP, Bharya S, Al-Sanna'a N, Adatia I, Chitayat D, Suzanne Lewis ME, Human DQ. Abnormal pericyte recruitment as a cause for pulmonary hypertension in Adams-Oliver Syndrome. Am J Med Genet 2004; 129: 294-298.