Digestive tract symptoms in congenital langerhans cell histiocytosis: a fatal condition in an illness usually considered benign.

INTRODUCTION: Congenital Langerhans cell histiocytosis is usually limited to cutaneous lesions and has a good prognosis. In rare cases of gut involvement, mortality is high and early and aggressive treatment essential. MATERIALS AND METHODS: We report a case of histiocytosis in a newborn with bowel involvement, and performed a literature review of 13 similar cases worldwide documented between 1973 and 2008. RESULTS: Skin eruptions are usually the initial symptoms at birth. Bloody stools or protein-losing enteropathy are the first signs of bowel involvement that appear mostly in the first 4 weeks of life. Risk organs (hematopoietic system, liver, spleen) are often affected in the newborns with intestinal Langerhans cell histiocytosis. Prognosis is usually poor, with 78.5% mortality. CONCLUSIONS: Even if histiocytosis in a neonate appears limited to autoinvoluting skin lesions, it is important to exclude all other organ involvement, including the bowel and stomach, as early treatment is vital.

Splenic haemorrhage in a newborn as the first manifestation of wandering spleen syndrome.

Wandering spleen is an unusual condition in children and is even more rarely diagnosed in the neonatal period. A case of splenic haemorrhage after dystocic birth in a newborn is reported. Before surgery, results of imaging studies were suggestive of a ruptured spleen. On laparotomy, a big haematoma surrounding a wandering spleen was found. Haemorrhage aroused from short splenic arteria. Haemostasia and splenopexy were performed. The spleen proved later to be viable. The authors speculate that the haemorrhage was the first manifestation of the wandering spleen.