A girl with sternal malformation/vascular dysplasia association

Abstract

Sternal malformation/vascular dysplasia association is a rare congenital dysmorphology, which has not yet been reported in Korea. Its typical clinical features include a sternal cleft covered with atrophic skin, a median abdominal raphe extending from the sternal defect to the umbilicus, and cutaneous craniofacial hemangiomata. We report a case of a full-term newborn who presented with no anomalies at birth, except for a skin defect over the sternum and a supraumbilical raphe. Multiple hemangiomas appeared subsequently on her chin and upper chest wall, and respiratory distress due to subglottic hemangioma developed during the first 2 months of life. Her symptoms were controlled with oral prednisolone administration. No respiratory distress have recurred during the 3-year follow-up period.

Keywords: Hemangioma; Malformation; Sternum.

Fig. 1

At birth: (A) the patient's chest wall and supraumbilical raphe extending from the umbilicus to the low sternal boarder; (B) the midline depression of the chest wall, with the parchment-like overlying skin.

Fig. 2

At 7 weeks of age: (A) the multiple cutaneous hemangiomas that developed subsequently on her chin and upper sternal area and (B) the persistent supraumbilical raphe and skin defect.

Fig. 3

(A) Coronal reconstruction of the chest computed tomographic (CT) scan showing hemangiomas at the right lateroposterior aspect of the infraglottic airway with airway narrowing (arrow). (B) Three-dimensional reconstruction of the chest CT scan depicting the complete sternal defect with split sternum bones.