Chilaiditi syndrome in pediatric patients - Symptomatic hepa
Chilaiditi syndrome, first described by Viennese radiologist Dr. Chilaiditi in 1910, is noted to be an extremely rare disorder associated with various symptoms including nausea, vomiting, abdominal pain, constipation, and respiratory distress. The condition is recognized radiologically by the presence of the hepato-diaphragmatic interposition of the intestine, called the Chilaiditi sign. Chilaiditi sign can be confused radiologically with other conditions such as pneumoperitoneum and subdiaphragmatic abscess. The cause of Chilaiditi syndrome is currently unknown but may include intestinal, diaphragmatic, or hepatic factors.

A 12-year-old male was admitted to the pediatric intensive care unit due to severe respiratory distress. Prior to this admission, he experienced persistent cough, dyspnea, nausea, and chest pain for over two months. He was prescribed antibiotics, nebulizations, and pain medication; however, there were no improvements in his respiratory symptoms. The patient has a history of asthma, gastroesophageal reflux disease, constipation, and a prior diagnosis of Chilaiditi syndrome. The diagnosis of Chilaiditi syndrome was made two years prior to this admission when the patient presented with a one-week history of right upper quadrant pain, nausea, and vomiting. There was no history of recent weight loss. An abdominal computerized tomography (CT) showed constipation and colonic interposition between the liver and the diaphragm with the displacement of the liver. Constipation was initially managed with a routine bowel cleansing protocol and a daily stool softener; however, intermittent episodes of abdominal pain persisted.

A chest X-ray revealed that the transverse colon was above the liver. On the first hospital admission day, a kidney, ureter, and bladder X-ray (KUB) showed a significant amount of fecal material and air-filled colonic loops which were slightly dilated and reaching the right hemidiaphragm. He subsequently received a bowel-cleaning regimen with GoLytely®. A follow-up KUB on the second hospital admission day showed the resolution of fecal retention or constipation. However, the patient continued to complain of tachypnea and right upper quadrant pain. Because of his persistent respiratory and abdominal symptoms, and due to the lack of significant improvement, surgery was consulted. The patient underwent laparoscopic colopexy and peritoneal abrasion of the diaphragm and liver. Significant intraoperative findings included a redundant transverse colon, no evidence of volvulus or adhesions in the upper abdomen, a relatively small right liver lobe (noncirrhotic), and a large gap between the liver and the anterior chest wall and diaphragm. His respiratory distress and abdominal pain resolved completely post-operatively and the patient was discharged with a maintenance stool softener regimen, colonic stimulant, and adequate dietary fiber. At the one-month follow-up after surgery, the patient reported regular bowel movements and no recurrence of his respiratory distress. He reported some mild intermittent episodes of right upper quadrant abdominal pain but never required emergency care or any interventions since the surgery.