Diffuse infantile haemangiomatosis: clinicopathological features and management problems in five fatal cases

Abstract

The clinicopathological features of five fatal cases of diffuse haemangiomatosis presenting in neonatal life or early infancy are presented. The infants all had multiple skin haemangiomas as well as deep-seated lesions in many different tissues that caused protean clinical manifestations and management problems. Because the outlook may be improved by early diagnosis and application of new modes of treatment, any infant with multiple cutaneous haemangiomas should be closely assessed for possible visceral involvement. Development of hepatomegaly, high-output cardiac failure, unexplained anaemia or thrombocytopenia in these infants should immediately suggest disseminated disease. Early recognition with implementation of steroid and/or antiangiogenic therapy, embolization and/or surgery is essential to improve the chances of survival.

Angiogenesis and angiogenesis inhibitors in paediatric diseases

“…In the future, they may be replaced by angiostatic steroids, which are devoid of glucocorticoid action [9]. Systemic forms of haemangiomas, the haemangiomatoses, present considerable therapeutic problems with a high mortality rate, and steroids may be ineffective [4]. In these cases, interferon may be useful, as has been demonstrated in a case of pulmonary haemangiomatosis [50].…”

Pathology of Pediatric Gastrointestinal and Liver Disease

“…Cavernous vascular malformations of the colon have been described in children with Klippel-Trenaunay syndrome (Ghahremani et al 1976 ), and the gastrointestinal tract, most commonly the small bowel, may be involved by the blue rubber bleb nevus syndrome (Browne et al 1983 ) (Fig. Diffuse infantile hemangiomatosis can affect the gastrointestinal tract as well as other visceral sites (Byard et al 1991 ). This syndrome is associated with cutaneous vascular malformations and may present with severe gastrointestinal bleeding.…”

Unusual causes of fatal upper aerodigestive tract obstruction in wild bottlenose dolphins (Tursiops aduncus)

“…Infectious causes of upper airway obstruction may be localized, or part of a generalized illness such as infectious mononucleosis [4,5]. Neoplastic lesions may be benign, as in upper airway hemangiomatosis, or malignant as in squamous cell carcinoma of the larynx [6]. The essential feature is that the tumor masses produce a critical reduction in luminal diameter of the airway.…”