Congenital diaphragmatic hernias (CDHs) are due to failure of the development of the diaphragm, which leads to displacement of the abdominal component into thorax, causing respiratory distress. It is a rare condition affecting 1 in 3000 live births¹. Recurrences after surgery have been reported in 3% of the cases² and have been associated with different risk factors.

We report the case of a male patient with a recurrent diaphragmatic hernia associated with intestinal malrotation 22 years after the surgical repair of a CDH.

A 22-year-old male patient with a history of CDH treated with surgery on the second day of life without perioperative or postoperative complications sought medical care due to class I dyspnea and gastroesophegeal reflux which appeared four months before consultation. The chest X-ray showed a radiolucent image encompassing 35% of the left hemithorax with displacement of the mediastinum and left lung towards the right hemithorax. The diagnosis of left diaphragmatic hernia was made, and a contrast-enhanced computed tomography (CT) scan of the abdomen was performed (Fig. 1A) to plan the surgical management. A left diaphragmatic hernia was found with a hernia sac containing the spleen, splenic flexure, tail of pancreas and left adrenal gland, associated with atelectasis of the ipsilateral lung base and displacement of the mediastinum towards the right hemithorax.

Figure 1 A. Contrast-enhanced CT scan of the abdomen. Left diaphragmatic hernia and hernia sac containing the spleen, colon, tail of pancreas and left adrenal gland. B. Posteroanterior chest X-ray on postopera tive day 1 with the chest tube placed in the pleural space of the left lung 

The patient underwent laparoscopic repair of the left diaphragmatic defect via a left subcostal incision. The hernia was identified with a size of 12 cm × 12 cm and the abdominal organs were visualized. After the abdomen was accessed and intestinal malrotation was observed, the hernia sac was reduced. A Sepramesh®- like double layer mesh was placed and sutured with Prolene 2-0 U-stitches using the parachute technique. The back of the mesh was fixed to the rib cage and was then closed with continuous Vicryl 1-0 suture (Fig. 2). We performed the Ladd procedure, which consists of appendectomy and releasing the Ladd’s bands; the small intestine is folded into the right side of the abdomen, and the colon is placed on the left side. A Blake-like drain was left in the surgical bed and 24-Fr Argyle® chest tube was placed in the pleural space of the left lung (Fig. 1B).

Figure 2 Diaphragm after mesh repair. Sepramesh-like mesh (short white arrow). Diaphragm (long black arrow). Liver (short black arrow). 

The patient presented tachypnea and tachycardia during the first 3 postoperative days without respiratory or hemodynamic involvement. He evolved with favorable outcome, and on postoperative day 6 both tubes were removed when fluid output from the pleural space and Blake drainage were < 120 cm³ and < 20 cm³, respectively. The patient was discharged one day later and was followed up at the outpatient clinic without complications three months after surgery.

Congenital diaphragmatic hernias are due to failure of the development of the diaphragm, which leads to displacement of the abdominal organs into the thorax. There are two types of CDH: Morgagni hernia (anterior defect) and Bochdalek hernia (posterolateral defect), the most common. This condition occurs in 1 out of 3000 live births¹, is suspected due to the presence of respiratory distress in the first hours after birth and is confirmed by chest x-ray and CT scan. Adults are usually asymptomatic and CDH is often an incidental finding in imaging tests; symptoms as abdominal pain, respiratory distress, palpitations, pneumonia, or gastroesophageal reflux may occur.

The definite treatment consists of reducing the abdominal organs and performing the surgical repair of the diaphragmatic defect by means of primary closure, prosthetic patch or abdominal muscle flaps in the case of type C or D diaphragmatic defect, according to the International Congenital Diaphragmatic Hernia Study Group², or in case of increased intra-abdominal tension, which may compromise the thoracic function. Surgical repair should be performed in patients with or without symptoms. Thoracotomy, laparotomy, thoracoscopy and laparoscopy are valid options for treating this condition; the technique is chosen depending on the clinical presentation. An open approach is preferred in emergency surgeries and a minimally invasive approach is chosen in elective procedures³. Laparoscopy presents lower rate of complications (9%), absence of mortality and short postoperative stay; however, the open technique is preferred in difficult cases, with large hernia volume and in the presence of dense adhesions³.

The incidence of recurrent CDH is between 5 and 65%⁴; specifically, the incidence of early or “in-hospital” recurrence of CDH is 3%². The main risk factors for early recurrence include repair of the diaphragmatic defect with synthetic patch or abdominal wall patch and prolonged postoperative length of stay⁵; the latter reflects the severity of the patient’s condition influenced by nutritional status and other comorbidities.

Diaphragmatic hernias in adults can be classified according to their etiology in congenital and acquired (hiatal or traumatic) which are the most common.

Congenital diaphragmatic hernia is uncommon in adults with an incidence of 0.17%, particularly on the left side³; the prevalence of recurrence is unknown as it is usually asymptomatic.

The association of congenital diaphragmatic hernia with intestinal malrotation is also uncommon. Intestinal malrotation is caused by an aberration of embryological development during intestinal rotation and fixation⁶. In adults, as intestinal malrotation is usually asymptomatic, its real incidence is not known, and hinders the diagnosis, which is usually made incidentally through an X-ray or during surgical exploration for other reasons. The Ladd procedure is the treatment of choice and was performed in our patient. The procedure involves detorsion of the bowel, surgical division of peritoneal bands, reorientation of the small bowel and large bowel, widening of the small intestine’s mesentery and performing an appendectomy.

The incidence of CDH recurrence is 3% with a median time to recurrence of 78 days after surgical repair of the defect²: after this period, recurrence is rare as in the case here described, so the actual recurrence in adults is not known.

Bochdalek hernia repair can be performed through a transthoracic or transabdominal approach. The transabdominal approach may be preferred in cases of intestinal obstruction or strangulation, or in the presence of other abdominal condition, as in this case report⁷.