American Journal of Medical Genetics 40:126-127 (1991)
Letter to the Editor
Adams-Oliver Syndrome Associated With Congenital Heart Defect: Not a Coincidence
To the Editor: The recent article by Santos et al.  on aplasia
cutis congenita associated with congenital heart defect prompts us to report the following case.
The patient, a boy, was the first child of healthy, unrelated parents (father 42 years, mother 32 years). He was born at 35 weeks of gestation with a birth weight of 1560 g. He had an hairless area without underlying scalp defect (Fig. 1). The left hand was normal but on the right hand the distal phalanges were short (Fig. 2): On the feet, all the toes were hypoplastic (Fig. 3). In addition the child had a ventricular septa1 defect (4 mm) shown by echocardiography.
Adams-Oliver syndrome is a variable condition pri- marily affecting scalp and limb development but other anomalies have been reported occasionally. Congenital heart defect was present in 5 patients and the present case (Table I). As reported by Santos et al. (1989) we think that congenital heart defect can be a component manifestation of the Adams-Oliver syndrome and is not a coincidence.
Fig. 2. Right hand with short distal phalanges.
Fig. 1. Parietal hairless area.
Received for publication July 20,1990; revision received August 25, 1990.
Address reprint request to Albert David, Departement de Pedi- atrie, Centre Hospitalier Bgional et Universitaire, 44035 Nantes Cedex 01, France.
Fig. 3. Distal defects of all toes
0 1991 Wiley-Liss, Inc.
Letter to the Editor 127
TABLE I. Adams-Oliver Syndrome With Congenital Heart Defect
Paltzik and Sybert Aiello r19851 rig851
Sex M M Age (months) Newborn 14 Scalp defect + + + Distal limb -
anomalies Congenital Ventricular Ventricular
heart defect septal de- septal fect and defect pulmonary stenosis (dysplastic pulmonary valve)
Toriello et al. 
M 4 + t
Pulmonary vein steno- sis and pul- monary artery hy- pertension
Kiister et al. 
+ Tetralogy of
Santos et al. [19891
F Newborn + -
Aortic coarc- tation and ventricular septal defect
Our case - M Newborn + t
Ventricular septal defect
Toriello HV, Graff RG, Florentine MF, Lacina S, Moore WD (1988): Scalp and limb defects with cutis marmorata telangiectatica con- genita: Adams-Oliver syndrome? Am J Med Genet 29:269-276.
REFERENCES Kuster W, Lenz W, Kaariainen H, Majewski F (1988): Congenital scalp
defects with distal limb anomalies (Adams-Oliver syndrome): Re- port of ten cases and review of the literature. Am J Med Genet 31:99-115.
Paltzik RM, Aiello AM (1985): Aplasia cutis congenita associated with valvular heart disease. Cutis 757-58.
Santos H, Cordeiro I, Menezes I (1989): Aplasia cutis congenita associ- ated with congenital heart defect, not a coincidence? Am J Med Genet 34:614-615.
Sybert VP (1985): Aplasia cutis congenita: A report of 12 families and review of the literature. Pediatr Dermatol 3:l-14.
Albert David Jean-Christophe Roze Valerie Melon-David Department of Pediatrics, Centre Hospitalier Mgional et Universitaire, Nantes, France