Congenital hernia of Morgagni in infants and children.

BACKGROUND

Congenital Morgagni hernia (CMH) is very rare, comprising 3% to 5% of all types of congenital diaphragmatic hernia; and very little is written about it. Commonly, it is diagnosed during childhood but can remain asymptomatic till adulthood. In the pediatric age group, the presentation is usually vague and nonspecific leading to delay in diagnosis.

PATIENTS AND METHODS

The medical records of all patients with the diagnosis of CMH treated at our hospital were retrospectively reviewed for age at diagnosis, sex, presenting symptoms, associated anomalies, diagnosis, operative findings, treatment, and outcome.

RESULTS

During an 18-year period (January 1983 through December 2001), 20 cases with the diagnosis of CMH were treated at our hospital. There were 15 males and 5 females. Their age at time of diagnosis ranged from 28 days to 9 years (mean, 16 months), and most of them (65%) were younger than 1 year. Sixteen of them (80%) presented with recurrent chest infection, which most of them had had since early infancy. In 3 patients, Morgagni hernia was discovered after blunt abdominal trauma in one, after insertion of ventriculoperitoneal shunt in another, and as a result of liver enlargement after splenectomy for beta-thalassemia major in the third. Of the 20 patients, 9 had right-sided Morgagni hernia, 5 had left-sided, and 6 had bilateral hernias. In 4 of those with bilateral hernia, the diagnosis of bilaterality was made at the time of surgery. Associated anomalies were seen in 14 (70%) of our patients. Five (25%) had congenital heart disease, 4 (20%) had malrotation of bowel, and 3 (15%) had Down's syndrome. All our patients were operated on transabdominally (14 upper midline, 5 upper transverse) except for one who had a right thoracotomy. In all 3 was a hernial sac, which was excised, and the defect was repaired using nonabsorbable sutures. The contents of the hernial sac included colon, small intestines, part of the liver, and omentum, with the colon being the most common (80%). There was no mortality, and postoperatively all patients did well. One of our patients required postoperative ventilation for 5 days because of associated lung hypoplasia.

CONCLUSIONS

Congenital Morgagni hernia is very rare. The rarity, as well as the vague and nonspecific presentations, contributes to the delay in diagnosis. Commonly, the presentation in the pediatric age group is that of recurrent chest infection and rarely with gastrointestinal symptoms. Physicians caring for these patients should be aware of this, and a high index of suspicion is recommended to obviate delay in diagnosis with its associated morbidity. We advocate surgical repair even in asymptomatic patients. This is to obviate the risk of strangulation and colonic perforation. We also advocate a transabdominal approach via either an upper midline or an upper transverse incision. This allows easy reduction and inspection of contents, allows access and repair of bilateral hernias, and corrects an associated malrotation if present.