ORIGINAL ARTICLE Morgagni’s Hernia in Infants and Children A. Latif Al-Arfaj From the Division of Paediatric Surgery, Department of Surgery, College of Medicine and Medical Sciences, King Faisal University, Dammam, Saudi Arabia Eur J Surg 1998; 164: 275–279 ABSTRACT Objective: To report my experience of Morgagni’s hernia in 4 infants and 1 child. Design: Retrospective study. Setting: Teaching hospital, Saudi Arabia. Subjects: 4 infants and 1 child with Morgagni’s hernias, one of which was recurrent. Interventions: Repair through an abdominal incision. Main outcome measures: Presentation, morbidity, and recurrence. Results: 4 of the 5 cases presented with respiratory distress or pulmonary infection, or both; 1 presented with failure to thrive as well. 3 patients had associated anomalies (hypertrophic pyloric stenosis, malrotation of the gut, and left inguinal hernia; Down syndrome; and multiple Mongolian spots and umbilical hernia). The hernia was recognised on chest radiograph in all cases, and confirmed by barium studies in 3. All the hernias were repaired through an abdominal incision. There were no deaths and no early complications, but late complications included a recurrence and incisional hernia in 1 patient and midgut volvulus caused by associated anomalies in another. Conclusion: Outcome is usually influenced by associated anomalies and the seriousness of the effects of the hernia. Timely diagnosis and repair can improve outcome. Key words: foramen of Morgagni hernia, retrosternal hernia, parasternal hernia, anterior diaphragmatic hernia. INTRODUCTION Morgagni’s hernia, also known as anterior diaphragmatic, parasternal, or retrosternal hernia, is generally thought to result from a congenital defect in the fusion of the anterior portion of the diaphragmatic anlage to the ribs and sternum, or in the fusion of pars costalis to pars sternalis of the diaphragmatic anlage on the right or left, or bilaterally. It can also result from a combination of the two. It is usually diagnosed in adults as an incidental asymptomatic finding; 90% are on the right, 8% on the left, and 2% bilateral. By contrast it is uncommon in infants and children (4, 6, 14), and often causes symptoms. We report 4 infants and one child with Morgagni’s hernias treated in our department during the last 15 years (1981–95). PATIENTS AND METHODS The medical records of all infants and children who presented with hernias through the foramen of Morgagni treated at the King Fahad Hospital of the King Faisal University, Al-Khobar, between 1981 and 1995 inclusive were reviewed (Table I). All patients underwent elective repair through an abdominal incision. The defects varied between 5 and 10 cm in diameter, three were unilateral and two 1998 Scandinavian University Press. ISSN 1102–4151 bilateral. There was always a hernial sac separated from the underlying pleura and pericardium by a thin layer of loose connective tissue and fat which facilitated dissection. The dissection can safely be carried out if it starts close to the edge of the hernial opening and develops gradually towards the centre of the sac. The sac was empty at the time of operation in one case and filled with varying lengths of colon in three. The omentum was adherent to the hernial sac in one case, and the left lobe of the liver was among the contents in another. The reduction of the hepatic portion was facilitated by introducing the index finger of the right hand into the hernial sac from the left lateral aspect of the hernial opening, hooking it around the posterior margin of the liver, and pushing the lobe downwards in a tilting motion. At the same time the anterior portion of the lobe was gently pushed up with the left hand. This manoeuvre freed the liver lobe from the margin of the diaphragmatic defect which was embedded all round in a groove in the hepatic tissue. Repeated simple traction had failed to reduce it. There were no immediate postoperative complications, but there were a recurrent hernia after the first repair and an incisional hernia after the second operation in one patient with recurrent pulmonary infection and feeding problems. Another patient developed a Eur J Surg 164 276 A. Latif Al-Arfaj Table I. Clinical details of five children with Morgagni’s hernia. All were born at full term by normal vaginal delivery Age (months) Case Sex 1 Male 5 2 Male* 12 3 Male** 9 4 Male 5 Female 28 8 Siblings Symptoms 3-normal Radiological Findings Recurrent chest infections; Retrosternal gas on chest feeding problems; failure film to thrive Not known Incidental finding Retrosternal gas on chest film; caecum-splenic flexure retrosternal on barium study Not known Recurrent chest infections; Retrosternal gas and respiratory distress; pneumonic infiltration on subcostal retraction. chest film; retrosternal colon on barium study. 1-normal Recurrent respiratory Chest film at 40 days distress within normal limits; chest film at 12 months retrosternal gas; gut retrosternal on barium study 2-normal Recurrent chest infections; Retrosternal gas on chest respiratory distress; film; retrosternal subcostal retraction transverse colon on barium study. Associated Anomalies Sickle cell anaemia Hypertrophic pyloric stenosis; left inguinal hernia. Malrotation of gut. Down syndrome; band of Ladd. Malrotation of gut. None Umbilical hernia; Mongolian spots * Pyloromyotomy and repair of left inguinal hernia at 40 days of age. ** Morgagni’s hernia previously repaired when 5 months old. midgut volvulus as result of associated malrotation and malfixation. These complications developed after 4 months in the former and 3 years in the latter case. DISCUSSION The incidence of Morgagni’s hernia in infants and children is low. It has been reported to be as frequent as 4/91 (4%), 3/66 (5%), 1/77 (1%), and 4/74 (5%) in four series of congenital diaphragmatic hernias excluding oesophageal hiatal hernias (5, 8, 12, 13). Our incidence was 5/57 (9%). Most Morgagni’s hernias are right sided (90%) (6, 14). There was no female preponderance as reported in adults series, and most series report equal sex distribution (3, 12). Four of our five patients were boys, and the hernia was bilateral in two. Most of Morgagni’s hernias in adults are asymptomatic or cause only vague epigastric distress or gastrointestinal symptoms. They therefore remain undetected until they are discovered as anterior cardiophrenic masses or air fluid levels in the retrosternal space on chest radiographs obtained for other reasons (1, 6, 12). This is in contrast to infants and young children who present with respiratory distress or pulmonary infection or both (as in four of our five) and less frequently with gastrointestinal symptoms such as cough, choking, and Eur J Surg 164 vomiting after feeding (12, 14). The latter symptoms were so pronounced in one of our patients that he failed to thrive. Our observations confirm those of others that in infancy and early childhood only a few patients are symptom-free (12, 14). The severity of symptoms varies from one series to another (3, 9, 12, 14). The respiratory distress in patients with Morgagni’s hernia is similar to that seen with Bochdalek’s hernia at a similar age (12). However, the pulmonary hypoplasia usually associated with Bochdalek’s hernia was reported in only two cases (3). We could not identify such an association clinically, nor could we find a correlation between the size of the hernia and the severity of the respiratory distress. The respiratory distress is most probably the result of pain arising at the hernial orifice or sac combined with reflex vagal-mediated bronchospasm, and not the result of displacement of lung tissue by the hernial contents, which was minimal in our series. Physical examination does not contribute to the diagnosis of Morgagni’s hernia because of the lack of physical signs except the respiratory distress and pulmonary infection found in some cases. The diagnosis is made radiologically. The anteroposterior and lateral chest radiographs often show a gas shadow or air-fluid level in the retrosternal space or right Morgagni’s hernia in infants and children 277 Fig. 2. Barium enema examination confirmed the diagnosis of Morgagni’s hernia showing the malrotation. Fig. 1. (A) Antero posterior and (B) lateral chest radiograph showing retrosternal gas shadow. hemithorax (1, 9, 12) (Fig. 1). However, a chest film may initially look normal despite the presence of a retrosternal defect; the abdominal viscera probably herniate into the chest first when trauma or increased intra-abdominal pressure displace viscera through the defect, which often happens in adults (11). This may explain the delay in diagnosing one of our cases. Suspicious findings are confirmed by barium enema or barium meal examination; the contrast study may also disclose a pre-existing malrotation (Fig. 2). The presence of omentum or a solid organ such as liver or spleen in the hernia may render the differential diag- nosis difficult and require more sophisticated procedures like ultrasonography, radionuclide scanning, or computed tomography (9, 12). If these are not conclusive, right and left angiocardiography, inferior venacavography, or coeliac angiography may be necessary (10). The reported incidence of associated congenital anomalies is variable (3, 5, 9, 12); three of our patients were affected. Some authors consider that the timing of repair of Morgagni’s hernia or the decision about whether to operate at all is a matter of individual preference (3, 4, 6), but we (like others) think that repair is indicated as soon as the condition is diagnosed unless there is a contraindication (12, 14). Morgagni’s hernia is at risk of incarceration, volvulus, or strangulation (9, 14). Furthermore, it can lead to severe respiratory compromise (3, 12, 14) or recurrent pulmonary infection (3). The postoperative improvement of these Eur J Surg 164 278 A. Latif Al-Arfaj symptoms (as noted in our series) is a further strong argument for the repair. Occasional herniation of abdominal viscera into the pericardium can result in death from cardiac tamponade (7), which can also be avoided by timely repair of the hernia. Most surgeons choose the transabdominal approach because it enables the surgeon to deal with other preexisting disease in the abdominal cavity (1, 3, 9, 14). This approach was of advantage in two of our cases with malrotation, one of whom had appendicectomy alone, and one appendicectomy with division of the band of Ladd. In one of the two cases hypertrophic pyloric stenosis was diagnosed and treated when he was a neonate, before the diagnosis of the retrosternal hernia; we could find no previous report of such an association. Advocates of the transabdominal approach report that the reduction of abdominal contents is always possible and if there is reduced volume in the abdominal cavity a ventral hernia can be created. Apart from being easy to do, the transabdominal approach also permits repair of bilateral Morgagni’s hernias—there were two in our series. A child has been reported to have undergone successful transthoracic repair of a right Morgagni’s hernia, only to die postoperatively of a strangulated unrecognised left Morgagni’s hernia (2). The transthoracic route has been recommended because it should give more accurate vision and facilitate dissection of adhesions in and around the hernial sac as well as making identification of the phrenic nerve easier. It should also make the reduction of incarcerated viscera and hepatic lobe safer (12). I have described a new manoeuvre to reduce the liver lobe safely through the transabdominal approach. Postoperative complications have rarely been described. However, a few cases of unexplained pneumopericardium were reported with mostly lethal consequences (12, 14). The postoperative course is often influenced by associated anomalies such as congenital heart disease (12), malrotation or other diseases, rather than by the hernia itself. We think that the postoperative outcome is adversely influenced by the chronicity of effects of the hernia such as recurrent pulmonary infection and feeding problems (as in one of our cases). Timely diagnosis and repair can contribute appreciably to the final outcome. REFERENCES 1. Anderson KD. Congenital diaphragmatic hernia. In: Welch KJ, Randoph JG, Ravitch MM, O’Neill JA Jr. and Rowe MI, eds. Pediatric surgery Chicago: Year Book Medical, 1986: 589–601. Eur J Surg 164 2. Bentley G, Lister J. Retrosternal hernia. Surgery 1965; 57: 567–575. 3. Berman L, Stringer D, Ein SH, Shandling B. The latepresenting pediatric Morgagni hernia: a benign condition. J Pediatr Surg 1989; 24: 970–972. 4. Bingham JAW. Hernia through congenital diaphragmatic defects. Br J Surg 1959; 201: 1–15. 5. Bonham-Carter RE, Waterston DJ, Aberdeen E. Hernia and eventration of the diaphragm in childhood. Lancet 1962; i: 656–659. 6. Comer TP, Clagett OT. Surgical treatment of hernia of the foramen of Morgagni. J Thorac Cardiovasc Surg 1966; 62: 461–468. 7. de Fonseca JMB, Davies MRQ, Bolton KD. Congenital hydropericardium associated with the herniation of part of the liver into the pericardial sac. J Pediatr Surg 1987; 22: 851–853. 8. Gross RE Congenital hernia of the diaphgram in: Gross RE, ed. Surgery of infancy and childhood. Philadelphia: WB Saunders, 1953: 428–444. 9. Kimmelstiel FM, Holgersen LO, Hilfer C. Retrosternal (Morgagni) hernia with small bowel obstruction secondary to a Richter’s incarceration. J Pediatr Surg 1987; 22: 998–1000. 10. Korobkin MT, Miller SW, Delorimer AA, et al. Hepatic herniation though the Morgagni foramen. Am J Dis Child 1973; 126: 217–219. 11. MacDougall JT, Abbott AC, Goodhand TK. Herniation though congenital diaphragmatic defects in adults. Can J Surg 1963; 6: 301–315. 12. Pokorny WJ, McGill CW, Harberg FJ. Morgagni hernias during infancy-presentation and associated anomalies. J Pediatr Surg 1984; 19: 394–397. 13. Snyder Jr WH, Greamey Jr EM. Congenital diaphragmatic hernia: 77 consecutive cases. Surgery 1965; 57: 576–588. 14. Thomas GG, Clitherow NR. Herniation though the foramen of Morgagni in children. Br J Surg 1977; 64: 215–217. RÉSUMÉ But: Rapporter mon expérience des hernies de Morgani chez 4 nouveaux-nés et un enfant. Type d’étude: Rétrospective. Provenance: Hôpital universitaire, Arabie Saoudite. Patients: Quatre nouveaux-nés et un enfant ayant une hernie de Morgani dont une récidivée. Méthodes: Cure chirurgicale par voie abdominale. Résultats: Quatre des 5 cas présentaient une défaillance respiratoire ou une infection pulmonaire, ou les deux; un présentait un retard de croissance. Trois patients avaient des anomalies associées (sténose hypertrophique du pylore, malrotation intestinale et hernie inguinale gauche; syndrome de Down; différents traits de mongolisme et hernie inguinale). Toutes les hernies ont été réparées par une voie d’abord abdominale. Il n’y a eu aucun décès et aucune complication précoce, mais des complications tardives à type de récidive et d’éventration chez un patient et de volvulus du grêle en rapport avec d’autres anomalies chez un autre. Conclusions: L’évolution est habituellement influencée par les anomalies associées et la gravité des conséquences de la hernie. Un diagnostic et une réparation effectués à temps peuvent améliorer l’évolution. Morgagni’s hernia in infants and children ZUSAMMENFASSUNG Ziel: Der Bericht meiner Erfahrung mit der Morgagnischen Hernie bei 4 Säuglingen und 1 Kind. Studienanordnung: Retrospektive Studie. Studienort: Lehrkrankenhaus, Saudi Arabien. Patienten: 4 Säuglinge und 1 Kind mit Morgagnischer Hernie, von denen eins rezidivierend war. Interventionen und Versorgung durch eine abdominelle Inzision. Endpunkte: Präsentation, Morbidität und Rezidiv. Ergebnisse: 4 der 5 Fälle zeigten Atembeschwerden, eine pulmonale Infektion oder beides; 1 Patient zeigte Entwicklungsstörungen. 3 Patienten zeigten assoziierte Anomalien (hypertrophe Pylorusstenose, Malrotation des Darms und linksseitige Inguinalhernie; Down-Syndrom, multiple mongolische Flecken und Umbilikalhernie). Die Hernie wurde auf einer Thoraxröntgenaufnahme in allen Fällen erkannt und durch Bariumstudien in 3 Fällen bestätigt. Alle Hernien wurden versorgt durch abdominelle Inzision. Es zeigte sich keine Letalität und keine frühen Komplikationen, doch die Spätkomplikationen beinhalteten ein Rezidiv und eine Narbenhernie bei einem Patienten und ein Volvulus verursacht durch assoziierte Anomalien in einem anderen Fall. Schlußfolgerungen: Das Ergebnis ist meist durch assoziierte Anomalien sowie die Ausdehnung der Effekte der Hernie beeinflußt. Zeitgemäße Diagnose und Versorgung können das Endergebnis verbessern. HTPUJVÉ Wtkm% Cjj,otybt gthcjyfkmyjuj jgsnf ktxtybz Morgagni’s uhs; e 4 yjdjhj;ltyys[ b 1 ht,tyrf& {fhfrnth bccktljdfybz% Htnhjcgtrnbdyjt bccktljdfybt& Rkbybrf% Ext,ysq ujcgbnfkm^ Cfeljdcrfz Fhfdbz& Gfwbtyns% 4 yjdjhj;ltyys[ b 1 ht,tyjr c Morgagni’s uhs;tq^ jlyf bp rjnjhs[ ,skf htwblbdyjq& 279 Vtnjls% Uthybjgkfcnbrf xthtp f,ljvbyfkmysq ljcneg& Pflfxb bccktljdfybz% Bpextybt jckj;ytybq b htwblbdjd& Htpekmnfns% 4 bp 5 ckexftd hjlbkbcm c htcgbhfnj- hysv lbcnhtcc-cbylhjvjv bkb ktujxyjq byatrwbtq^ bkb ;t b ntv b lheubv& E lde[ gfwbtynjd bvtkb vtcnj cjgencnde.obt fyfvfkbb (ubgthnhjabxtcrbq cntyjp ds[jlyjuj jnltkf ;tkelrf^ vfkmhjnfwbz rbirb b gf[jdfz uhs;f cktdf^ cbylhjv Lfeyf^ vyj;tcndtyyst vjyujkjblyst gznyf b gegjxyfz uhs;f)& Uhs;f ,skf hfcgjpyfyf ghb htynutyjdcrjv bccktljdfybb uhelyjq rktnrb^ dj dct[ ckexfz[ gjlndth;ltyf lfxtq ,fhbz& Dct uhs;b ,skb jgthbhjdfys xthtp f,ljvbyfkmysq ljcneg^ yt ,skj jnvtxtyj ktnfkmys[ bc[jljd b hfyyb[ gjcktjgthfwbjyys[ jckj;ytybq& Jlyfrj^ d rfxtcndt gjplyb[ jckj;ytybq bvtk vtcnj htwblbd b gjcktjgthfwbjyyfz dtynhfkmyfz uhs;f e jlyjuj bp gfwbtynjd b pfdjhjn njyrjq rbirb e lheujuj& Dsdjls% Htpekmnfns ktxtybz j,sxyj cdzpfys c cjgencnde.obvb fyfvfkbzvb^ f nfr;t 'aatrnfvb cfvjq uhs;b& Cdjtdhtvtyysq lbfuyjp b ktxtybt gjpdjkz.n ekexibnm htpekmnfns& Submitted October 31, 1996; submitted after revision March 5, 1997; accepted March 13, 1997 Address for correspondence: A. Latif Al-Arfaj, M.D. Thogbah P.O. Box 20325 Al-Khobar 31952 Saudi Arabia Tel: ‡966 898 4317 Fax: ‡966 864 5972 Eur J Surg 164