Frank DiPaola1, Andrew T Trout2,3, Ashley E Walther4, Anita Gupta5, Rachel Sheridan5, Kathleen M Campbell6, Greg Tiao7, Jorge A Bezerra6, Kevin E Bove5, Manish Patel2,3, Jaimie D Nathan7. 1. Division of Pediatric Gastroenterology, Hepatology, and Nutrition, C.S. Mott Children's Hospital, MPB D5200, 1500 East Medical Center Drive, SPC 5718, Ann Arbor, MI, 48109-5718, USA. dipaolaf@med.umich.edu. 2. Department of Radiology, Cincinnati Children's Hospital Medical Center, MLC 5031, 3333 Burnet Avenue, Cincinnati, OH, 45229, USA. 3. Department of Radiology, University of Cincinnati Medical Center, Cincinnati, OH, USA. 4. Division of Pediatric Surgery, Children's Hospital Los Angeles, 4650 Sunset Blvd, MS #100, Los Angeles, CA, 90027, USA. 5. Department of Pathology and Laboratory Medicine, Cincinnati Children's Hospital Medical Center, MLC 1035, 3333 Burnet Avenue, Cincinnati, OH, 45229, USA. 6. Division of Gastroenterology, Hepatology, and Nutrition, Cincinnati Children's Hospital Medical Center, MLC 2010, 3333 Burnet Avenue, Cincinnati, OH, 45229, USA. 7. Division of Pediatric General and Thoracic Surgery, Cincinnati Children's Hospital Medical Center, MLC 2023, 3333 Burnet Avenue, Cincinnati, OH, 45229, USA.
Abstract
BACKGROUND: Congenital portosystemic shunt (CPSS) is a rare malformation in which splanchnic venous flow bypasses the liver. CPSS is associated with other congenital anomalies and syndromes and can be associated with life-threatening complications. CPSS and their management remain underreported in the literature. Here, we review the clinical characteristics, management, and outcomes of a cohort of children and young adults with CPSS from two pediatric centers. METHODS: Cases of CPSS from Cincinnati Children's Hospital Medical Center and C.S. Mott Children's Hospital were reviewed to define CPSS anatomy, associated anomalies, complications, interventions, and outcomes. The imaging features and histopathology of liver lesions were characterized in detail. RESULTS: A total of 11 cases were identified. Median age was 10 years (range 0-26); 8 (73%) cases were female. Associated anomalies included six patients with heterotaxy (55%), five patients with congenital heart disease (45%), three patients with Turner syndrome (27%), and two patients with omphalocele, exstrophy, imperforate anus, spinal defects (OEIS) complex (18%). Eight (73%) cases had hyperammonemia ± encephalopathy. A 4-month-old presented with hepatopulmonary syndrome, and 12-year-old presented with pulmonary hypertension. Eight patients (73%) had liver lesions including five with premalignant adenomas and three with well-differentiated hepatocellular carcinoma (HCC). Four children underwent successful CPSS occlusion/ligation. Three children underwent liver transplant (2) or resection (1) for HCC without recurrence at extended follow-up. CONCLUSIONS: CPSS is associated with multiple anomalies (heterotaxy, congenital heart disease) and syndromes (Turner syndrome). CPSS liver lesions should be very carefully evaluated due to risk of premalignant adenomas and HCC. Serious complications of CPSS can occur at a young age but can be managed endovascularly or with open surgery.
BACKGROUND: Congenital portosystemic shunt (CPSS) is a rare malformation in which splanchnic venous flow bypasses the liver. CPSS is associated with other congenital anomalies and syndromes and can be associated with life-threatening complications. CPSS and their management remain underreported in the literature. Here, we review the clinical characteristics, management, and outcomes of a cohort of children and young adults with CPSS from two pediatric centers. METHODS: Cases of CPSS from Cincinnati Children's Hospital Medical Center and C.S. Mott Children's Hospital were reviewed to define CPSS anatomy, associated anomalies, complications, interventions, and outcomes. The imaging features and histopathology of liver lesions were characterized in detail. RESULTS: A total of 11 cases were identified. Median age was 10 years (range 0-26); 8 (73%) cases were female. Associated anomalies included six patients with heterotaxy (55%), five patients with congenital heart disease (45%), three patients with Turner syndrome (27%), and two patients with omphalocele, exstrophy, imperforate anus, spinal defects (OEIS) complex (18%). Eight (73%) cases had hyperammonemia ± encephalopathy. A 4-month-old presented with hepatopulmonary syndrome, and 12-year-old presented with pulmonary hypertension. Eight patients (73%) had liver lesions including five with premalignant adenomas and three with well-differentiated hepatocellular carcinoma (HCC). Four children underwent successful CPSS occlusion/ligation. Three children underwent liver transplant (2) or resection (1) for HCC without recurrence at extended follow-up. CONCLUSIONS: CPSS is associated with multiple anomalies (heterotaxy, congenital heart disease) and syndromes (Turner syndrome). CPSS liver lesions should be very carefully evaluated due to risk of premalignant adenomas and HCC. Serious complications of CPSS can occur at a young age but can be managed endovascularly or with open surgery.
Authors: Christiane Sokollik; Robert H J Bandsma; Juan C Gana; Meta van den Heuvel; Simon C Ling Journal: J Pediatr Gastroenterol Nutr Date: 2013-06 Impact factor: 2.839
Authors: Atessa Bahadori; Beatrice Kuhlmann; Dominique Debray; Stephanie Franchi-Abella; Julie Wacker; Maurice Beghetti; Barbara E Wildhaber; Valérie Anne McLin Journal: Children (Basel) Date: 2022-02-11