CASE
A 3-month-old male baby born 3710 g at 38th-week from the first pregnancy of a 38-year-old mother was followed up in another center for 17 days due to respiratory distress. The baby was then referred to the neonatal intensive care unit due to the persistence of flexion contracture in the right hand, kyphoscoliosis, and respiratory distress. As a result of the examinations performed in the neonatal intensive care unit, he was operated on with a pre-diagnosis of hiatal hernia. In postnatal radiological examinations, spinal MRI revealed that the C5 vertebral corpus could not be clearly identified. In this localization, the anterior defect at the level of the C5 vertebra and the areas associated with the lung parenchymal areas in the localization adjacent to this area were observed with air-fluid leveling. This area was thought to be related to the spinal cord. Thorax CT showed that mediastinal compartments were displaced to the left. In the medial of the right hemidiaphragm, a 2 cm suspicious appearance in the diaphragm posteriorly and herniation of the intestinal loops to the right hemidiaphragm at this level was detected.
In operation, no hiatal hernia was observed, and it was terminated with appendectomy only. A 3-month-old baby with multiple congenital anomalies (adduction-pronation and flexion in the right arm, defect at the anterior of the C5 cervical vertebra, and anterior cervical meningomyelocele appearance) and receiving intra-hood oxygen support was transferred to our pediatric palliative care center due to congenital malformation and maternal compliance.
On physical examination, his general condition was moderate, he was conscious, and his hydration was good. His weight was 3160gr (10-50p), height 52 cm (90p), head circumference 36cm (90p), apex beat 149 min, respiratory rate 52/min, fever 36.6°C (axillary), blood pressure 80/62 mmHg, preductal Spo2 97%, and postductal Spo2 98% (in room air). There was respiratory distress. There were no pathological findings in cardiovascular, gastrointestinal and genitourinary system examinations, apart from an incision scar in the midline of the abdomen.
Due to the persistence of the cystic appearance in the chest radiography (Figure 1), advanced radiological examinations and fluoroscopy were performed. Fluoroscopy showed malrotation resulting in herniation of the jejunal loops from the medial center of the right hemidiaphragm to the right hemithorax from the hiatus level and gastroesophageal reflux. The MRI reported an enteric duplication cyst extending transdiaphragmically to the right hemithorax from the hiatus level and accompanying meningomyelocele tissue observed at the cervical level, hypoplasia/agenesis in the right hemithorax apex level, and a pre-diagnosis of the fused neurenteric cyst was suggested.
The case, was consulted again with pediatric surgery and operated again with an experienced surgeon and chairman of the department. The cystic lesion adhering to the wall at the apex of the right hemithorax was removed. In pathological examination, a small intestinal (jejunal) duplication cyst with a length of 5 cm and a diameter of 1.5 cm was reported. On the 10th postoperative day, he was discharged to his home.