Gastrointestinal bleeding in a child associated with type Abernethy II malformation: а case report

Gastrointestinal bleeding (GIB) is a significant and frequently encountered clinical problem in pediatric practice. One rare etiologic factor is Abernethy malformation, in which lower gastrointestinal bleeding has been reported as the primary presenting symptom in 8.1 % of cases. Abernethy malformation is an extremely rare congenital anomaly of the portal venous system, characterized by diverse clinical manifestations that may be difficult to recognize in early childhood. The aim of this report is to describe the clinical case of an 8-yearold boy with Abernethy malformation and to inform clinicians about the diagnostic strategies and management considerations for this condition. A retrospective review of medical records was performed for a patient treated at the Children’s Republican Clinical Hospital of the Ministry of Health of the Republic of Tatarstan (DRKB MZRT) and the Russian Children’s Clinical Hospital, a branch of the Pirogov Russian National Research Medical University (RDKB, RNIMU). The child had been followed by a pediatric surgeon since the age of three for complaints of hematochezia. Local therapy with methyluracil suppositories was initiated following the diagnosis of an anal fissure. During preoperative evaluation for elective adenoidectomy, laboratory testing revealed thrombocytopenia (platelet counts ranging from 57–103 × 10⁹/L). The patient was admitted to the Department of Hematology and Immunology at DRKB for further evaluation. Subsequent diagnostic investigations resulted in the diagnosis of small intestinal angiodysplasia (ileal angioectasias) complicated by gastrointestinal bleeding; moderate anemia secondary to chronic blood loss; and secondary (consumptive) thrombocytopenia. The patient was then referred to the RDKB (RNIMU) for advanced diagnostic workup. The following studies were performed: duplex ultrasonography of the portal venous system, contrast-enhanced abdominal computed tomography, and direct portal venography. Portal venography demonstrated a markedly dilated inferior mesenteric vein (up to 12 mm) draining via a tortuous portosystemic communication into the right internal iliac vein. At the federal center, the patient was diagnosed with Abernethy malformation, Type II. Laparoscopic ligation of the congenital portocaval shunt was performed in the Department of Pediatric Surgery at DRKB. Given the anticipated hemodynamic changes within the portal venous system postoperatively, anticoagulant therapy was administered. The child was discharged in stable condition and continues to receive follow-up care with hematology and vascular surgery specialists at the RDKB in Moscow. This case highlights the importance of recognizing Abernethy malformation as a rare cause of gastrointestinal bleeding in children and underscores key considerations in its clinical presentation, diagnostic evaluation, and surgical management. The report aims to increase awareness among pediatric surgeons, radiologists, and pediatricians regarding this uncommon but clinically significant anomaly.

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