Morgagni-Larrey parasternal diaphragmatic hernia in the adult.

With a prevalence of 0.3-0.5/1000 births, congenital diaphragmatic hernia (CDH) remains a serious, poorly understood abnormality with a high mortality rate that cannot always be effectively managed. Its reported frequency in Spain is 0.69%00 with a yearly decreasing trend of 0.10%00 during the period 1980-2006. Up to 5% of cases are incidentally identified in adults undergoing studies for other reasons.We report the case of a 74-year-old woman with vomiting for three months due to parasternal diaphragmatic hernia of Morgagni-Larrey (retrochondrosternal, retrocostoxyphoid, retrosternal, subcostal, substernal or subcostosternal hernia), which allowed us to report an update on this condition in the adult, and on thoracoabdominal diaphragm morphogenesis. It is in the embryology of the diaphragm where an explanation may be found for some morphological changes and clinical manifestations, even though a number of uncertainties remain. We also analyze the extent of controversy persisting on some aspects of surgical treatment (access routes, mesh use, hernial sac reduction). Overall, minimally invasive techniques predominate. We consider laparoscopy the approach of choice for adult patients with parasternal hernia eligible for surgery.

Hernia diafragmática paraesternal de Morgagni-Larrey en adulto / Morgagni-Larrey parasternal diaphragmatic hernia in the adult

Con una prevalencia de 0,3-0,5/1.000 nacimientos, la hernia diafragmática congénita (HDC) sigue siendo una anomalía grave, no bien entendida, alta mortalidad y tratamiento no siempre efectivo. En España se ha informado de una frecuencia del 0,69%oo con una tendencia decreciente en el periodo 1980-2006 del 0,10%oo por año. No obstante, hasta un 5% se diagnostican en adultos durante la realización de un reconocimiento por otra causa. Presentamos un cuadro de vómitos de tres meses de evolución en una mujer de 74 años por hernia diafragmática paraesternal de Morgagni-Larrey (retrocondroesternal, retrocostoxifoidea, retroesternal, subcostal, subesternal o subcostoesternal), que nos ha permitido realizar una actualización de esta patología en adultos y de la morfogénesis del diafragma toracoabdominal. Es en la embriología del diafragma donde encontramos explicación de algunas de sus alteraciones morfológicas y características clínicas, si bien persisten aspectos confusos de la misma. También analizamos el grado de controversia que persiste en algunos aspectos de su tratamiento quirúrgico (vías de acceso, uso o no de mallas y reducción o no del saco herniario). Por lo general priman las técnicas mínimamente invasivas. Consideramos el abordaje laparoscópico como de elección en pacientes adultos con hernia paraesternal candidatos a la cirugía(AU)

With a prevalence of 0.3-0.5/1000 births, congenital diaphragmatic hernia (CDH) remains a serious, poorly understood abnormality with a high mortality rate that cannot always be effectively managed. Its reported frequency in Spain is 0.69%oo with a yearly decreasing trend of 0.10%oo during the period 1980-2006. Up to 5% of cases are incidentally identified in adults undergoing studies for other reasons. We report the case of a 74-year-old woman with vomiting for three months due to parasternal diaphragmatic hernia of Morgagni-Larrey (retrochondrosternal, retrocostoxyphoid, retrosternal, subcostal, substernal or subcostosternal hernia), which allowed us to report an update on this condition in the adult, and on thoracoabdominal diaphragm morphogenesis. It is in the embryology of the diaphragm where an explanation may be found for some morphological changes and clinical manifestations, even though a number of uncertainties remain. We also analyze the extent of controversy persisting on some aspects of surgical treatment (access routes, mesh use, hernial sac reduction). Overall, minimally invasive techniques predominate. We consider laparoscopy the approach of choice for adult patients with parasternal hernia eligible for surgery(AU)