Management of Hirschsprung's disease ? an alternate approach

Congenital aganglionic megacolon in Nigerian adults: Two case reports and review of the literature

“…Hirschsprung's Disease (HD) is characteristically manifested at birth and diagnosis is made in the neonatal period, in a majority of cases. [7,13,[15][16][17][18] The most common accepted theory of the etiology of Hirschsprung's disease is that there is a defect in the craniocaudal migration of neuroblasts originating from the neural crest, which occurs during the first 12 weeks of gestation, and other the mechanisms proposed include defects in the differentiation of neuroblasts in the ganglion cells and accelerated ganglion cell destruction in the intestine [6,7] More than eight genetic mutations have been identified with the disease. [8,9] The RET proto-oncogene is most affected and accounts for 50% of the familial and 20% of the sporadic cases.…”

“…[7] The late presentation of Hirschsprung's disease is very rare in advanced countries. [13,20,21] However it is still common in our environment, probably because of lack of adequate perinatal care in our rural setting, where a majority of the populace live. [22] Approximately 75-80% of the patients with Hirschsprung's disease have a transition zone in the rectosigmoid area, but 2-3% of Hirschsprung disease patients have the so-called 'ultra-short segment' or zonal segment Hirschsprung's disease.…”

“…The choice of the operative approach depends on identifying the distal most segment of the bowel with ganglion cells, which is frequently suggested by the contrast enema and then confirmed by biopsy with frozen section examination, accomplished in the operating room [13] , but due to lack of facilities, we adopted less than ideal techniques for the management of Hirschsprung's disease. [7,13,15] Technical approaches different from Swenson's original pull-through procedure have been developed and further modified to the present development of the laparoscopic approach, for the surgical treatment of Hirschsprung's disease. [1,3,16,18,19,[27][28][29] Both patients had short or zonal segment of aganglionosis close to the anal canal, hence, low anterior resection (state) was done.…”

“…[6][7][8][9][10][11][12] The presentation of Hirschsprung's disease is not common in adults because it is a congenital anomaly, and therefore, most often presents early in life, for surgical intervention. [7,13,14] We report two cases in Nigerian adults with review of the literature.…”