Clinical Review & Education

JAMA Surgery Clinical Challenge

An Unusual Hernia Devin S. Zarkowsky, MD; Peter B. Cornell, NP; Frederic Eckhauser, MD

Figure 1. Intraoperative view.

A 68-year-old woman presented 1 year after noticing a flank bulge that developed following a forceful coughing episode. Her medical history included gastroesophageal reflux disease, irritable bowel syndrome, hypothyroidism, hypertension, asthma, and obesity. On palpation, the mass extended Quiz at jamasurgery.com superiorly to the costal margins and inferiorly to the anterior-superior iliac spine; no fascial edge was palpable medially. A computed tomographic (CT) scan demonstrated abdominal contents—including liver, ascending colon, and omental fat— protruding through a 12-cm defect. Weight loss was counseled and 8 weeks allowed for optimization before exploration. On incision, we encountered this view (Figure 1).

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WHAT WOULD YOU DO NEXT?

A. Video-assisted thoracic surgery reduction B. Transabdominal laparoscopic reduction and mesh placement C. Video-assisted thoracic surgery reduction with primary closure D. Right thoracotomy with primary closure and mesh placement

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Clinical Review & Education JAMA Surgery Clinical Challenge

Diagnosis Morgagni and chest wall hernias

What To Do Next D. Right thoracotomy with primary closure and mesh placement This patient’s hernia proved a diagnostic odyssey; the right 10th rib near the ipsilateral kidney’s lower pole was not appreciated preoperatively (Figure 2A). Repair proceeded through an open incision, as is our preference for a presumed flank hernia, particularly one containing multiple abdominal organs. We encountered pleura with a sac herniating posterior to the sternum, leading to the diagnosis of combined Morgagni and chest wall hernias. Consultation with our thoracic colleagues prompted sac inversion and diaphragm plication with nonabsorbable monofilament suture in a figure-of-8 fashion without entering the abdomen. Bioprosthetic mesh reinforced the intrapleural space between the ninth and 10th ribs, followed by polypropylene mesh onlay extending 8 to 10 cm beyond the fascial closure in all directions and binding with fibrin sealant (Figure 2B). Drains were placed between the bioprosthetic and hernia closure and subcutaneously, then vacuum dressing. She was discharged on postoperative day 12. At follow-up 2, 5, and 6 weeks later, she reported gradual to full function. Her incision healed well and the final drain was removed at week 6. At 6 weeks, she had a small right pleural effusion but no recurrence.

Discussion Giovanni Battista Morgagni described a diaphragm laceration “independent of any wounding instrument… from the ensiform cartilage and the neighbouring fibres. …the stomach was found in the right cavity, and the freedom from lungs indicated that this was an instance of malformation” in The Seats and Causes of Diseases,1 published in 1769 and translated by Cooke in 1824. Other translations suggest Morgagni transcribed a conversation about his eponymous diaphragmatic defect related by a Roman surgeon.2 A

Congenital diaphragmatic hernias affect 1 in 3000 live births and demonstrate a postnatal survival rate of 50% to 75%.3 Defective rhabdomyoblast migration is the causative factor.4 Primary hepatic herniation, followed by the liver’s return to the abdominal cavity and ingress into the sac of more freely mobile abdominal contents or insufficient posthepatic mesenchymal plate development are hypothesized to cause congenital diaphragmatic hernias.5,6 Horton et al7 reviewed 295 patients and found most defects (91%) were rightsided; the balance were either left-sided (5%) or bilateral (4%). Clinical presentation varies. Vague abdominal symptoms were reported in some series, whereas others cited pulmonary and pain or pressure symptoms.4,7 Obstruction, dysphagia, bleeding, and gastroesophageal reflux disease may also be present. Horton et al7 found 72% of patients had symptoms. Physical examination is nonspecific. Chest radiography is ordered in 93% of cases, followed by CT scan in 47%.7 Reported CT scan sensitivity and specificity were 78% and 100% for left-sided defects vs 50% and 100% for right-sided defects, respectively.8 Horton et al7 noted that 40 of 195 published cases reported changing diagnosis during surgery. Isolated Morgagni hernias are amenable to multiple approaches, with successes published in all 8 permutations. Surgeons chose thoracotomy in 49% of the published cases. This is potentially problematic given the 9% reported rate of left-sided hernias combined with a 78% sensitivityofCTscanforleft-sideddefects.Surgeonshavereportedlaparoscopic and thoracoscopic approaches.7,9 Mesh placement occurred in 7% of the open cases vs 64% of endoscopic cases.7 Garriboli et al10 tracked 22 children, finding recurrences in 2 treated thoracoscopically; 1 received mesh. Laparoscopic primary closure resulted in recurrence in5of11childreninanotherseries;reinterventionwithmeshplacement in 2 resolved the reherniation. Although the large review by Horton et al7 reported 1 recurrence from an open procedure, no distinction between primary closure and mesh placement was noted.

B

Figure 2. A, Preoperative computed tomographic scan. B, Mesh placement.

ARTICLE INFORMATION Author Affiliations: Department of Surgery, Johns Hopkins Hospital, Baltimore, Maryland. Corresponding Author: Devin S. Zarkowsky, MD, Department of Surgery, Johns Hopkins Hospital, 600 N Wolfe St, Blalock 658, Baltimore, MD 21287 ([email protected]). Section Editor: Jonathan R. Hiatt, MD. Published Online: July 16, 2014. doi:10.1001/jamasurg.2013.4851. Conflict of Interest Disclosures: None reported. REFERENCES 1. Morgagni GB. In: Cooke W, ed. The Seats and Causes of Diseases: Investigated by Anatomy. Boston, MA: Wells and Lilly; 1824. 990

2. McBride CA, Beasley SW. Morgagni’s hernia: believing is seeing. ANZ J Surg. 2008;78(9):739-744. 3. Logan JW, Rice HE, Goldberg RN, Cotten CM. Congenital diaphragmatic hernia. J Perinatol. 2007; 27(9):535-549. 4. Nasr A, Fecteau A. Foramen of Morgagni hernia. Thorac Surg Clin. 2009;19(4):463-468. 5. Mayer S, Metzger R, Kluth D. The embryology of the diaphragm. Semin Pediatr Surg. 2011;20(3):161-169. 6. Iritani I. Experimental study on embryogenesis of congenital diaphragmatic hernia. Anat Embryol (Berl). 1984;169(2):133-139.

8. Schumacher L, Gilbert S. Congenital diaphragmatic hernia in the adult. Thorac Surg Clin. 2009;19(4):469-472. 9. Yamaguchi S, Marshall MB. Outpatient laparoscopic repair of a Morgagni hernia. Surg Innov. 2012;20(6):np38-39. 10. Garriboli M, Bishay M, Kiely EM, et al. Recurrence rate of Morgagni diaphragmatic hernia following laparoscopic repair. Pediatr Surg Int. 2013; 29(2):185-189.

7. Horton JD, Hofmann LJ, Hetz SP. Presentation and management of Morgagni hernias in adults. Surg Endosc. 2008;22(6):1413-1420.

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An unusual hernia.

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