Original article
Down's syndrome as a factor in the diagnosis, management, and outcome in patients of Morgagni hernia

https://doi.org/10.1016/j.jpedsurg.2010.10.001Get rights and content

Abstract

Purpose

The purpose was to study the impact of Down's syndrome (DS) in the diagnosis, management, and outcomes of patients with Morgagni hernia (MH).

Methods

Twenty-two (22) patients with MH treated at a tertiary center were retrospectively studied for history, findings, associated anomalies, referral diagnoses, hospital admissions, radiological procedures for diagnosis, age at which operated on, operative procedure, complications, and recurrences. Eleven did not have DS (group 1); 11 others had associated DS (group 2). The ages at operation were compared in the 2 groups using the Mann-Whitney test. More than 3 hospital admissions for symptoms and signs relevant to MH before a diagnosis of MH were considered a “delayed diagnosis.”

Results

Twenty-two patients (7 females, 15 males) aged 3 months to 10 years were seen. They presented with respiratory distress (n = 16), with vomiting (n = 5), with intestinal obstruction (n = 1), by serendipity (n = 2), and with recurrence from another hospital (n = 1). The mean age of group 1 was 14.5 months, and that of group 2 was 29.18 months; the difference was not significant (P = .621). Nine patients of group 2 were “delayed diagnosis” compared with 2 in group 1. Both delays from group 1 had severe associated anomalies. All patients underwent operative correction (17 open and 5 laparoscopic repairs). Two had recurrences, one operated on by the open method by us and another laparoscopically by the Lima technique at another center. Both had DS. Both were reoperated on by the open method.

Conclusions

The diagnosis of MH may be strikingly delayed when associated with DS or other severe congenital anomalies. Morgagni hernia should be strongly considered in patients with DS admitted repeatedly for chest infections. Chest x-rays in 2 planes may avoid misdiagnosis of MH. Both open and laparoscopic methods have proven satisfactory as operative treatment of MH. Recurrences were seen in patients with DS, which may be corrected by laparotomy or laparoscopically. We feel that resecting the sac and approximating the posterior lip of the defect to the anterior abdominal wall, whether in open or laparoscopic methods, may give stronger repairs, which may avoid recurrence.

Section snippets

Patients and methods

All patients treated with a diagnosis of MH at Asir Central Hospital between January 1994 and December 2008 were studied. Asir Central Hospital serves the southern region of Saudi Arabia and is a tertiary care center and teaching hospital. Patients were divided into 2 groups: those with (group 2) and those without DS (group 1). Their records were retrospectively analyzed for demographics, presenting features, referral diagnoses, other associated congenital anomalies, investigations, and

Results

The demography, associated anomalies, and referral diagnoses of the 2 groups are displayed in the Table 1. The mean age was 14.5 months (median, 12 months) for the group without DS and 29.18 months (median, 12 months) for those with DS. Comparison of ages of the 2 groups by the Mann-Whitney test yielded nonsignificant differences (P = .621). Overall, there were 14 males and 8 females, with more males in the DS group. In both groups, the majority presented with respiratory tract symptoms. In the

Discussion

The large number of DS patients in this series presented us with a unique opportunity of comparing patients with MHs who have DS with those who do not. The age at which patients from both groups came to surgery was not significantly different in the 2 groups. However, the number of patients with “delayed diagnosis” was notably different. Delays were defined by the authors as more than 3 hospital admissions for symptoms and signs pertinent to MH but without its diagnosis. Nine patients in the DS

References (19)

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