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.