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Laparoscopic repair of esophageal hiatal hernia for infant with asplenia syndrome : A case report Terutaka TANIMOTO 1 , Takuo NODA 1 , Hiroshi NOSO 1 1Department of Pediatric Surgery, Okayama University Hospital Keyword: 無脾症候群 , 食道裂孔ヘルニア , 対称肝 pp.176-183
Published Date 2022/7/15
DOI https://doi.org/10.11477/mf.4426200992
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 This report describes the case of a 4-month-old girl with hiatal hernia complicated by heterotaxy with asplenia. She was born at 33 weeks of gestation and weighed 1821g. The diagnosis of heterotaxy with

asplenia and congenital cardiovascular malformations, including single ventricle with single atrium, common atrioventricular valve, total anomalous pulmonary venous connection was made postnatally. The symptom of vomiting appeared around the age of 1 month and she was referred to our department. The upper gastrointestinal series revealed right-sided stomach, hiatal hernia, and severe gastrointestinal reflux. Laparoscopic repair of the hiatal hernia was performed at 4 months old when she weighed over 4kg.

 We inserted the trocar in the reversed position compared to the typical case because of her unique morphology; the stomach presented in the right side, the liver was symmetric, and the hepatoduodenal ligament and gallbladder were located in the midline of the body. The Nathanson liver retractor was introduced from the right upper quadrant of the abdomen to elevate the symmetric liver. The right-sided stomach, which had prolapsed into the mediastinum, was repositioned. After suturing of the esophageal hiatus, Toupet fundoplication was performed. The postoperative course was uneventful.

 Accurate understanding of the unique anatomy of asplenia and the technique to develop the surgical field properly enables the performance of laparoscopic repair of the hiatal hernia safely in infants with asplenia.


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電子版ISSN 2186-6643 印刷版ISSN 1344-6703 日本内視鏡外科学会

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