[Neonatal purpura fulminans without sepsis due to a severe congenital protein C deficiency]. / Purpura fulminans néonatal sans sepsis par déficit congénital sévère en protéine C.

Severe congenital protein C deficiency is a rare life-threatening coagulopathy. In the early hours of life, the neonate presents with extensive purpura fulminans and substantial skin necrosis contrasting with a preserved general state and a negative infectious exam. Disseminated intravascular coagulation sets in secondarily. Prenatal outset of thrombotic events is a rare situation that worsens the prognosis, especially protein C replacement in utero is not available. We report a case of a male newborn of consanguineous parents who were asymptomatic carriers of heterozygous protein C deficiency. This infant presented prenatal ventricular hemorrhage with hydrocephalus and rapidly extensive postnatal skin necrosis that was not regressive in spite of fresh frozen plasma administrated after 24h of life. Prenatal diagnosis, early recognition, and urgent therapy with protein C replacement and anticoagulant treatment are crucial to improve the prognosis, avoid further damage after delivery, and prevent the devastating consequences of severe protein C deficiency.

Trans-mesenteric hernia in infants: report of two cases.

Internal hernias are rare causes of intestinal obstruction in children. Trans-mesenteric hernia remains the most common form. We report transmesenteric hernia in a neonate and infant presented with intestinal obstruction.

[Bile duct dilatation associated with pancreatic heterotopia: a case report]. / Hétérotopie pancréatique associée a une dilatation kystique du cholédoque. À propos d'un cas.

Pancreatic heterotopia or aberrant pancreas is a rare congenital anomaly, usually asymptomatic. Its diagnosis is difficult. We report an original observation in an 8-month-old infant, operated in the neonatal period for esophageal atresia type III in the context of VACTERL syndrome, cystic dilatation of the bile duct, and pancreatic heterotopia.

Trichobezoar with small bowel obstruction in children: two cases report.

A trichobezoar is a mass of cumulated hair within the gastrointestinal tract. Stomach is the common site of occurrence. Intestinal obstruction due to trichobezoar is extremely rare. The authors report two cases of a trichobezoar obstructing the terminal ileum in one and the jejunum in another.