A 9-year-old male child who has a history of muscular dystrophy was referred to our centre for signs of a diaphragmatic hernia. Three months before coming to the hospital he developed multiple episodes of vomiting, which was non-projectile, non-bilious, intermittent, and accompanied by a gradual worsening of diffuse abdominal pain associated with a history of dyspnoea at rest. There was no history of hematemesis. The patient had no significant birth history. The mother noticed delayed developmental milestones in motor tasks without any delay in speech or cognitive development. The patient had trouble sitting up and later standing up. There was no diurnal variation in muscle weakness and no difficulty in fine motor skills. There was no history of abnormality in bladder and bowel movements.
He was evaluated at a secondary care hospital where on examination he was afebrile, tachypnoeic, heart rate of 110/ min and SpO2 of 94% on room air. There was no abdominal rigidity or guarding and the bowel sounds were normal. An ultrasound abdomen was performed which showed splenomegaly measuring 12.8 cm with minimal ascitic fluid. A chest X-ray
(Fig. 1) was taken which showed a large mediastinal shift to the right and a radiolucent shadow in the left thorax was misdiagnosed as pneumothorax for which he was admitted, and an intercostal drain was placed. The patient's symptoms worsened, and he developed significant continuous vomiting, melena, obstipation and guarding. Further evaluation done by a Computer tomography (CT) scan (Fig. 2) revealed, large left-sided diaphragmatic hernia with contralateral mediastinal shifting and complete collapse of the left lung with patchy opacities in the right lung. Hence the patient was shifted to our institute for further management.
On examination, pulse was 120/min, regular, low volume. Blood pressure was 78/50 mm Hg in the left upper limb in semi propped position at the level of the heart. Abdominal examination revealed a flat abdomen with normal bowel sounds. The respiratory system showed marked decreased breath sounds on the left side with abdomino-thoracic respiration with a rate of 35/min using accessory muscles of respiration. Cardiovascular examination showed a shifted cardiac apex on the 5th intercostal space 1 inch lateral to the left border of the sternum. Initial stabilization was done with nil per oral status, large bore intravenous cannula was placed.
All the necessary blood investigations were sent. ECG was done which showed sinus tachycardia without any conduction abnormalities. Trans thoracic echocardiography showed normal systolic and diastolic function and normal chamber size and filling, suggesting the absence of cardiomyopathy.
The patient was taken up for semi-emergency surgery with adequate blood products in hand under high-risk consent. Standard American Society of Anaesthesiology (ASA) monitoring and invasive hemodynamic monitors were used for monitoring. After preoxygenation, induction was done intravenously with Midazolam 0.15 mg/kg, Fentanyl 2mcg/kg, and Thiopentone in the sleeping dose. Intubation was done with Inj. Rocuronium 0.5mg/kg. The inhalational anaesthesia and nitrous oxide were avoided in view of muscular dystrophy and gut distension which could further jeopardize patient oxygenation. So, the patient was maintained with the Oxygen, and air mixture along with a maintenance dose of fentanyl and atracurium.
The patient was placed in the right lateral decubitus position and the left hemithorax was approached through left thoracotomy. A large rent was present in the anterior left hemi diaphragm with very thin edges. The stomach was the major content in the hemi thorax with a compressed left lung. After decompressing the abdominal contents and closing the diaphragmatic rent, an attempt was made to recruit the left lung manually under vision and only partial recruitment was achieved so it was decided to electively ventilate the patient in the post-operative period. Later the patient was extubated on post-operative day 1. The patient is followed up for 5 months without any complaints.