Morgagni Hernia in Children: Nine Cases and a Review of the Literature

Abstract

Brief ReportsMorgagni Hernia in Children: Nine Cases and a Review of the Literature Mahmoud Machmouchi, MD, DESC Nabil Jaber, and MD, FAAP Jihad NaamaniMB, ChB Mahmoud Machmouchi Address reprint requests and correspondence to Prof. Machmouchi: Department of Pediatric Surgery, Alhada Armed Forces Hospital, P.O. Box 1347, Taif, Saudi Arabia. From the Departments of Pediatrics, Pediatric Surgery, and Emergency Medicine, Alhada Armed Forces Hospital, Taif, Saudi Arabia Search for more papers by this author , Nabil Jaber From the Departments of Pediatrics, Pediatric Surgery, and Emergency Medicine, Alhada Armed Forces Hospital, Taif, Saudi Arabia Search for more papers by this author , and Jihad Naamani From the Departments of Pediatrics, Pediatric Surgery, and Emergency Medicine, Alhada Armed Forces Hospital, Taif, Saudi Arabia Search for more papers by this author Published Online:1 Jan 2000https://doi.org/10.5144/0256-4947.2000.63SectionsPDF ToolsAdd to favoritesDownload citationTrack citations ShareShare onFacebookTwitterLinked InRedditEmail AboutIntroductionRetrosternal hernia is much less common than congenital posterolateral diaphragmatic hernia, and is a benign diaphragmatic defect, apart from associated trisomy or severe cardiac malformations. It is embryologically related to a congenital developmental anomaly or a defect between the sternal and costal origin of the diaphragm. This defect was first described by Morgagni in 17611 and by Larrey in 18292 and has many names, though it is most commonly referred to as Morgagni hernia.PATIENTS AND METHODSThe charts of nine patients who underwent surgical repair from January 1989 to December 1998 were reviewed. The diagnosis of retroxyphoidal hernia was obtained for all patients by gastroscopy of the upper gastrointestinal tract. Details obtained during examination included age at diagnosis, sex, preoperative signs and symptoms, associated malformations, and results of x-ray studies. The diagnosis of Morgagni hernia was achieved in all cases by barium swallow, and the follow-up was based on the clinical picture and the chest x-ray performed every two months postoperatively for six months, and yearly thereafter. The follow-up ranged from six months to five years.RESULTSOver the 10-year study period, nine cases of Morgagni hernia were operated upon. They comprised five males and four females. The mean age at diagnosis was 16 months. Six patients were below the age of one year, and one patient was nine years old. Three cases were firstborn children. There was no relevant family history in seven cases, however, two of the cases were the offspring of a consanguineous marriage. All patients were delivered full-term after uneventful gestation.The patients were admitted with complaints of chronic cough and repeated chest infections in four cases, and two cases of post-feeding epigastric painful crisis with vomiting. In three patients, the Morgagni hernia was missed on the anteroposterior chest x-ray due to its small size, however, the lateral views were always conclusive, even in very small-sized hernias. The final diagnosis was obtained by barium swallow (Figures 1 and 2).Figure 1. Lateral view of a small-sized hernia.Download FigureFigure 2. A huge hernia discovered at the age of nine months.Download FigureThe left hepatic lobe, or part of it, was herniated into the sac in eight cases, in which one case represented mild abnormal segmentation. The stomach, spleen, part of the small intestine and the cecum were herniated together in one case, nevertheless, part of the transverse colon was always present in the hernia.Associated malformations were found in four cases: one with Down's syndrome, one with a small ventricular septal defect (VSD) that resolved spontaneously, one with malrotation of the gut, and the last one with bilateral undescended testes.The defects were repaired using the abdominal approach, and the procedure consisted of a simple reduction of the hernial content and direct closure of the hernia with non-absorbable sutures without sac resection or drainage. The diaphragmatic defect was on the right side in four children, on bilateral sides in two, and on the left side in the other three cases.There were no postoperative complications or mortality in these cases and the remaining sac resolved completely before the end of the fourth month. No recurrence was noticed, and only one child continued to have mild pseudo-asthmatic bronchitis. The follow-up ranged between six months to seven years, with a mean of three years.DISCUSSIONThe foramen of Morgagni is a gap or potential gap lying between the sternal and costal attachments of the diaphragm transmitting the internal mammary artery. A retrosternal hernia is called a parasternal hernia or hernia through the foramen of Morgagni, and arises from a congenital developmental anomaly or defect between the sternal and costal origin of the diaphragm. Unlike Cantrell's pentalogy defect, the parasternal hernias are associated with a hernial sac.1–3Males are usually affected more than females,4,5 and the etiology is not known. A number of reports of multiple family members or twins having retrosternal hernias raised the possibility that Morgagni hernia may result from an inherited defect,6 occurring in a ratio of about 1:10 of congenital posterolateral diaphragmatic hernias. The lack of awareness of the delayed presentation of Morgagni hernia in children with uncharacteristic thoracic or abdominal symptoms leads to an undesirable time delay before surgery.5,7Mild to severe symptoms may develop, with the most common symptoms and signs being respiratory, mimicking pulmonary infection, chronic cough, choking, and shortness of breath or respiratory distress.8–12 Gastrointestinal symptoms with abdominal pain, nausea and vomiting may be from compression of the bowel, but incarceration of the intestine in Morgagni hernia is unusual, with few reported cases producing obstruction,13–15 and those mainly in adults.A wide variety of associated anomalies are present, particularly trisomy 21,5,6,16 heart defect, mainly ventricular septal defect (VSD),17 malrotation of the gut,8,18 cryptorchidia, pyeloureteral stenosis and diaphragmatic relaxation.5The conventional chest x-ray, stressing the importance of lateral films, is the most diagnostic procedure of diaphragmatic hernia, and a gastrointestinal series should confirm the diagnosis. However, other radiological exploration such as CT scan, MR or pneumoperitoneography should be reserved for difficult cases.5,8,19Morgagni hernias appear more frequently on the right side than either the left side or bilaterally. The most common viscera herniated are portions of the liver (without constant abnormal segmentation), small and large bowels, spleen and stomach.1,5Surgical repair is required in all cases, and may be performed by the abdominal or transthoracic approach, whether by open or laparoscopic surgery.20–22 The postoperative course is usually uneventful, and life-threatening complications are usually related to delayed diagnosis or to an inadequate surgical procedure. The recurrence is no more than 5%.7,11Morgagni hernia is a rare congenital malformation, and surgical treatment should be attempted before the appearance of complications. The resection of the hernia sac is not imperative. 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