Situs inversus totalis in combination with hiatal hernia and hereditary hemolytic anemia in a 4-year-old child: A case report

Situs inversus totalis is an extremely rare anomaly in which the organs of the chest and abdominal cavity lie in the opposite direction. With complete transposition, clinical manifestations may be absent, and diagnostic and therapeutic problems may arise in the presence of other malformations or diseases. A 4-year-old girl was admitted for treatment in the hematology department of the National Medical Center of the Republic of Tajikistan, with complaints of nausea, periodic vomiting, lethargy, lack of appetite, malaise, and skin pallor. Owing to the ineffectiveness of conservative therapy, surgical treatment was recommended, i.e., splenectomy. During the preoperative examination, radiography and radiopaque examination of the organs of the gastrointestinal tract with barium sulfate revealed that the fundus and body of the stomach were located in the left half of the chest, and the heart was symmetrically deployed to the right. An ultrasound examination revealed signs of displacement of the liver to the left half of the abdomen, the spleen to the right, and the heart in the right half of the chest. The diagnosis was hiatal hernia on the left, situs inversus totalis, hereditary hemolytic anemia, fermentopathy, and G-6-PD deficiency. Laparoscopic surgery was performed, which included splenectomy, elimination of a hiatal hernia, chiatoplasty, and esophagogastrofundoplication with the creation of a Nissen cuff. The girl was discharged on day 10. On control examination after 6 and 12 months, she had no complaints and has grown and developed according to age. This clinical case is the first in the Russian literature to describe the treatment of a child with a hiatal hernia against the background of complete transposition of internal organs. This case highlights the need for imaging techniques for any unclear symptoms, especially in children with hereditary diseases. Minimally invasive intervention could contribute to a favorable outcome.