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.

Perforación esofágica yatrogénica en el período neonatal / Istrogenic oesophageal perforation in the newborn period: report on two cases

La perforación esofágica de origen yatrogénico es rara en períodoneonatal. Se presentan dos casos, uno de ellos en un neonato atérmino y el otro en un prematuro. En ambos se referían maniobrasprevias de sondaje o de intubación endotraqueal como probablemecanismo de la lesión. La sintomatología inicial de uno delos casos fue similar a la de la atresia esofágica, y posteriormentese observó la situación anómala de una sonda nasogástrica en elmediastino. El otro cursó con un síndrome de dificultad respiratoriarelacionada con su prematuridad y complicada con un neumotórax.La sospecha se confirmó por el hallazgo accidental del extremode una sonda en el espacio pleural. En ambos casos, sedecidió efectuar una pauta conservadora con tratamiento antibióticode amplio espectro y nutrición parenteral total. La evoluciónfue favorable, sin secuelas aparentes. Se revisa esta entidad poniendoénfasis en aspectos sobre el diagnóstico y en la recomendación actual de considerar una terapéutica conservadora, y reservarel abordaje quirúrgico para los pocos casos que presentan unaevolución desfavorable(AU)

Iatrogenic oesophageal perforations are very rare in the newbornperiod. Herein, we report two cases occurring in a term and in apremature infant. In both cases, the probable mechanism of injurywas the orogastric or endotracheal tubes that both infants required.The initial symptoms in one of the cases mimicked those of oesophagealatresia, and imaging studies revealed the distal end ofthe nasogastric tube in the mediastinum. The second baby suffereda respiratory distress syndrome related to his prematurity, whichwas complicated by a pneumothorax. The suspicion of oesophagealperforation was confirmed after imaging revealed the distal end ofa orogastric tube in the pleural space. In both cases, a conservativetreatment was followed, consisting of broad-spectrum antibioticsand exclusive parenteral nutrition. Both cases evolved satisfactorily,with no sequelae. In this article, we provide an update on this entity,with special emphasis on early diagnosis and the current recommendationsbased on conservative management and avoidanceof surgery except on cases with adverse outcome(AU)

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.