A rare triad: complicated meconium ileus with type 3b jejunal atresia and antenatal mid jejunal perforation complicated by late-onset sepsis induced hemophagocytic lymphohistiocytosis

Vollständiger Abstract

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Complicated meconium ileus (MI) involving mid jejunal perforation with congenital adhesions bands with type IIIB jejunal atresia (apple-peel deformity) is a high-risk surgical emergency. While surgical management is often the primary focus, the postoperative course can be derailed by systemic inflammatory syndromes. We report a rare case of a 35-week neonate with antenatal perforation and Type IIIB atresia who, following successful surgical repair, developed late-onset culture-proven sepsis. This subsequently triggered hemophagocytic lymphohistiocytosis (HLH), a life-threatening hyperinflammatory condition. Through aggressive multidisciplinary management, the neonate survived and is currently a healthy two-year-old. This case emphasizes the need for early recognition of HLH in neonatal sepsis cases that fail to respond to standard therapy.