Appreciation of Unique Anatomical Variations During a Laparoscopic Repair of a Hiatus Hernia in an Adult Patient with Heterotaxy Syndrome Allows a Safe Outcome

Abstract

Heterotaxy in Greek literally means a different arrangement in space. Heterotaxy syndrome is a rare congenital condition that occurs from failure of intra-abdominal and/or intrathoracic organs to rotate correctly during embryologic development, resulting in abnormal arrangement of the organs across the left-right axis of the body [1]. It has two broad subcategories which include polysplenia and asplenia [1]. As most of patients remain asymptomatic; the prevalence of this anomaly remains underestimated, although one study has suggested that 1 in every 2500 people may have heterotaxy syndrome with polysplenia [2]. This condition is mostly detected incidentally in the adults during multi-detector computed tomography scanning for diagnosis of other pathologically related or unrelated complaints as in this reported case [3,4]. Herein authors report unusual anatomical findings that posed many operative challenges during a laparoscopic repair of a hiatus hernia in an adult patient with heterotaxy syndrome. Preoperative radiological evaluation and intra-operative appreciation of the anomalies with considering an alternative feasible technique allowed authors to achieve a safe outcome. Case Report