Abstract A case report of aplasia cutis congenita of the vertex of the scalp associated with symbrachydactyly of both feet is presented. The Adams-Oliver syndrome, which is also known as Type 2 aplasia cutis congenita, is aplasia cutis congenita of the midline scalp seen together with congenital limb anomalies. Although vari- ous limb anomalies linked to aplasia cutis congenita have been described in the literature, we were not able to find any report of bilateral symbrachydactyly of the feet related to aplasia cutis congenita. Thus, this case seems to represent an unreported form of the Adams- Oliver syndrome. Etiology, associated anomalies, treat- ment and complications of Adams-Oliver syndrome are reviewed. Keywords Aplasia cutis · Symbrachydactyly · Adams-Oliver syndrome Introduction Symbrachydactyly is a congenital anomaly of the hands and/or feet, which presents as short fingers that are fused together [21]. The condition is usually confined to the central portion of the affected hand or foot [21], it is a common congenital anomaly in the hands but seldom affects the feet [4, 24]. Hand and foot symbrachydactyly may occur together but this is a unusual combination [5,6]. Isolated symbrachydactyly of the foot is a rare congenital anomaly and bilateral feet involvement is even more uncommon [7, 24]. Aplasia cutis congenita is a rare disease, which may involve any site on the body but usually includes the ver- tex with scalp and even skull involvement [30]. It may occur in isolation or with other congenital malformations [1,18]. A combination of various degrees of terminal transverse limb defects and short fingers or toes accom- panying aplasia cutis congenita of the scalp is known as Adams-Oliver syndrome [1, 17, 20]. Multiple hereditary patterns have been described for this condition (usually an autosomal dominant inheritance) and sporadic cases have also been reported [17, 19]. Other malformations, such as central nervous system anomalies, may be seen in Adams-Oliver syndrome [19, 20]. We report, to the best of our knowledge, probably the first case in the literature with bilateral symbrachydactyly of feet associated with aplasia cutis congenita of scalp [1, 8, 10, 11, 13, 20, 26, 28, 29]. This case seems to be a rare and possibly unreported presentation of Adams- Oliver syndrome. Case report A 4-year-old boy with an absence of hair in a localized area of scalp and abnormalities in the toes of both feet was referred for treatment. An examination revealed that he had localized alopecia extending from the frontal area to the vertex in the midline, mea- suring 15 cm in length and approximately 8 cm in width (Fig. 1a,b). The lesion was scar tissue, which was heterogeneous in appearance with hypo- and hyperpigmented areas. The hair was scarce and found mostly at periphery of the alopecic area. In addi- tion, there was hypoplasia of the second, third and fourth toes of the right foot. The right second and third web spaces were not present, which resulted in syndactyly between the second and third and the third and fourth digits (Fig. 2a,b). There was hypo- plasia of the left first, second, third and fourth toes (Fig. 2a,b). This resulted in syndactyly between the second and third and the third and fourth toes, respectively. The child was otherwise healthy. Oral, dental, ophthalmologic and neurological examinations were unremarkable, and normal intelligence and development were noted. U. Koçer ( ✉ ) Mes ¸rutiyet Cad. 17/12, 06640 Kzılıay, Ankara, Turkey e-mail: u_kocer@hotmail.com Tel.: +90-312-4175535, Fax: +90-312-4255633 U. Koçer · H.M. Aksoy · Y.Ö. Tiftikciog ˘lu · F. Bingül Plastic and Reconstructive Surgery Clinic, Ankara Training and Research Hospital, Ankara, Turkey D. Ertoy Department of Pathology, Hacettepe University School of Medicine, Ankara, Turkey G. Bükülmez Department of Dermatology, Hacettepe University School of Medicine, Ankara, Turkey Eur J Plast Surg (2001) 24:310–314 DOI 10.1007/s00238-001-0307-y CASE REPORT U. Koçer · H. M. Aksoy · Y. Ö. Tiftikciog ˘lu · F. Bingül D. Ertoy · G. Bükülmez Coexistence of aplasia cutis congenita of the scalp with symbrachydactyly of bilateral feet: an unreported form of Adams-Oliver syndrome Received: 29 March 2001 / Accepted: 14 August 2001 / Published online: 7 November 2001 © Springer-Verlag 2001