Associated malformations in Morgagni hernia

Abstract

Morgagni hernia (MH) is a congenital herniation of the abdominal organs through the subcostosternal portion of the diaphragm. It is a rare type of diaphragmatic hernia that has been associated with other congenital anomalies. The purpose of this clinical review was to determine the incidence of associated anomalies in patients with MH at a major pediatric referral center. The medical records of all patients with the diagnosis of MH were reviewed retrospectively between 1983 and 2006. The age, sex, side of herniation, presenting symptoms and signs, associated anomalies, radiologic features, intraoperative findings, postoperative course, complications, and mortality were recorded. There were 11 males and 5 females. The average age of the patients was 34.5 months (range 2 months-14 years). Twelve patients (75%) had at least one associated congenital malformation and eight (66.6%) of them had multiple anomalies. Six (37.5%) patients had a chromosomal anomaly, Down's syndrome in five and Turner syndrome in one. MH has a high incidence of associated congenital malformations.

Bilateral morgagni hernia in a case of Weill-Marchesani syndrome-a rare association

“…6 Berman et al treated only 18 cases of CMH over a period of 40 years and Pokorny et al reported only 4 cases of CMH over a period of 25 years. 8,9 Cigdem et al treated 16 cases of CMH over a period of 23 years. 9 Giovanni Battista Morgagni, an Italian anatomist, first described the herniation of abdominal contents through the sternochondral triangles in 1769 based on cadaver observation.…”

“…8,9 Cigdem et al treated 16 cases of CMH over a period of 23 years. 9 Giovanni Battista Morgagni, an Italian anatomist, first described the herniation of abdominal contents through the sternochondral triangles in 1769 based on cadaver observation. 10 Later, in 1828, Larrey described a surgical approach to the pericardial sac through these same triangles.…”

“…17 Cigden et al in a series of 16 patients with CMH seen over a period of 23 years reported a 31.25% incidence of Down's syndrome. 9 Other rare clinical associations of Morgagni hernia are pulmonary hypoplasia, gastric volvulus, rotational abnormalities and midgut volvulus, hypoplasia of the left ventricle and bilateral renal hypertrophy.…”

Laparoscopic repair of Morgagni hernia in the adults: Report of two cases

“…[13,14] The genetic component in the penetrance of MH is further confirmed by the fact that MH is closely associated with Down's syndrome (Trisomy 21). [15,16] These genetic components in the etiology strongly suggest a fusion defect between diaphragmatic muscle fibers and the costochondral arc. [15] Other than genetic factors, mechanical factors such as increased intra-abdominal pressure due to various reasons such as trauma have been reported.…”

“…[15,16] These genetic components in the etiology strongly suggest a fusion defect between diaphragmatic muscle fibers and the costochondral arc. [15] Other than genetic factors, mechanical factors such as increased intra-abdominal pressure due to various reasons such as trauma have been reported. In a multicenter study performed by Al-Salem et al fortyeight infants had MH and 4 were due to acquired etiologies causing increased intra-abdominal pressure such as trauma, ventricul operitoneal shunts, beta thalassemia causing massive splenomegaly, and thus increasing intraabdominal pressure.…”