Sternal Malformation: Vascular Dysplasia Complex
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《交互式心脏血管和胸部手术》
Department of Pediatrics, Government Medical College and New Civil Hospital, Surat, Gujarat, India
A 18-month-old female child presented with capillary hemangioma on lower lip and chin with midline skin dimple on chest. The manubrium sterni was absent. A hollow space bulged out when the child cried (Fig. 1). The child was otherwise normal. Chest X-ray confirmed absence of manubrium sterni, widening of medial clavicular ends. Rest of the skeletal survey, laryngoscopy, ultra-sonography of thorax and abdomen, echo-cardiography and other routine investigations were normal.
Fig. 1. Capillary hemangioma over lower lip and chin are seen. In this crying photograph, the soft tissue is seen to bulge over the area of absent manubrium sternum.
This combination of anomalies represent rare sternal malformation vascular dysplasia complex. The spectrum of defects in various combinations include midline ventral defects, supraumbilical raphe, micrognathia ophthal-mic, brain and cardiac defects. Etiopatho-genesis and inheritance are uncertain. Diagnostic evaluation for serous internal vascular and cardiac anomalies is necessary.
Early surgical repair of sternal defects is indicated even in asymptomatic child because it may render the major vessels and heart vulnerable to injury. Hemangiomas can be treated by standard management.(Sangita S. Trivedi,)
A 18-month-old female child presented with capillary hemangioma on lower lip and chin with midline skin dimple on chest. The manubrium sterni was absent. A hollow space bulged out when the child cried (Fig. 1). The child was otherwise normal. Chest X-ray confirmed absence of manubrium sterni, widening of medial clavicular ends. Rest of the skeletal survey, laryngoscopy, ultra-sonography of thorax and abdomen, echo-cardiography and other routine investigations were normal.
Fig. 1. Capillary hemangioma over lower lip and chin are seen. In this crying photograph, the soft tissue is seen to bulge over the area of absent manubrium sternum.
This combination of anomalies represent rare sternal malformation vascular dysplasia complex. The spectrum of defects in various combinations include midline ventral defects, supraumbilical raphe, micrognathia ophthal-mic, brain and cardiac defects. Etiopatho-genesis and inheritance are uncertain. Diagnostic evaluation for serous internal vascular and cardiac anomalies is necessary.
Early surgical repair of sternal defects is indicated even in asymptomatic child because it may render the major vessels and heart vulnerable to injury. Hemangiomas can be treated by standard management.(Sangita S. Trivedi,)