Therapeutic Strategy for Gastrointestinal Disorders in Children With Asplenia Syndrome or Polysplenia Syndrome

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  • Okumura Kenji
    Department of Pediatric Surgery, Kumamoto City Hospital
  • Yamamoto Hirotoshi
    Department of Pediatric Surgery, Kumamoto City Hospital Department of Pediatric Surgery, Kumamoto University Hospital

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  • 無脾症候群および多脾症候群に合併した小児外科疾患の治療戦略
  • ムヒショウコウグン オヨビ タヒショウコウグン ニ ガッペイ シタ ショウニ ゲカ シッカン ノ チリョウ センリャク

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<p>Purpose: The purpose of this study was to review the therapeutic strategy for gastrointestinal disorders in children with asplenia syndrome (AS) or polysplenia syndrome (PS).</p><p>Methods: We retrospectively reviewed the medical records of 44 consecutive infants (32 AS and 12 PS) treated at our hospitals from January 2000 to December 2015. The analysis focused on gestational age, birth weight, complicated cardiac anomalies, complicated gastrointestinal disorders, and prognosis.</p><p>Results: Twenty-eight AS (87.5%) patients had a single ventricle. Among the AS patients, 14 had intestinal malformations (43.7%), and 2 patients with intestinal malrotation, 3 patients with gastric volvulus, and 1 patient each with necrotizing enterocolitis, congenital duodenal atresia, and esophageal hiatal hernia underwent surgery. Six PS (50%) patients showed the absence of the inferior vena cava. There was only 1 PS patient (8.3%) with intestinal malrotation with congenital duodenal atresia and he underwent surgery. The overall survival rates of PS and AS patients were 46.9% and 77.5%, respectively.</p><p>Conclusions: Patients with AS or PS can have severe cardiac anomalies. All patients should be investigated for intestinal rotational abnormalities. AS or PS patients who require abdominal surgery should be adequately managed for each cardiac anomaly.</p>

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