New perspective for the management of near-total or total intestinal aganglionosis in infants

Intestinal transplantation for total intestinal aganglionosis: a series of 12 consecutive children

“…In addition, those patients used to undergo multiple surgical procedures to improve intestinal absorption capacity, which usually failed. Apart from our preliminary report of 3 cases [8], the only relevant study was reported by Sharif et al [7], involving 5 patients, 4 of whom received combined liver and intestinal graft. Four (80%) of the patients were alive after a median follow-up of 32 months.…”

“…Twelve patients had TIA with a median age of 54 months (range, 10-144 months) and median weight being 21.6 kg (range, 12-43 kg). Most underwent several prior surgical procedures, with a median number of 5.75 laparotomies (range, [3][4][5][6][7][8][9][10][11]. The mean residual length of ganglionic jejunum at the time of transplantation was 32.2 cm (range, 5-110 cm).…”

“…None of the surgical procedures so far used for SBS and TIA, such as reversed loops, Kimura's procedure, longitudinal myomectomy, and intestinal lengthening as Bianchi's procedure, have provided a sufficient improvement in intestinal absorption to allow patients to be weaned from PN [2][3][4][5]. Management of these patients is thus challenging, making intestinal transplantation (ITx) the logical alternative treatment [6][7][8]. Irreversible IF associated with early onset of long-term PN may lead to liver impairment, potentially requiring combined liver-ITx (LITx).…”

“…Irreversible IF associated with early onset of long-term PN may lead to liver impairment, potentially requiring combined liver-ITx (LITx). Only small series of ITx in patients with normal innervated small intestine segment ranging from 15 to 50 cm have been reported [7]. We recently reported the first results of ITx, including the right colon, in 3 patients with TIA [8].…”

Waardenburg syndrome with extended aganglionosis: report of 3 new cases

“…One characteristic is the length of the aganglionic segment. Total colonic (ileocecal) aganglionosis and long segment (up to the splenic flexure) aganglionosis have been observed in 60.4% and 14%, respectively, where the location of caliber change was described, whereas rectosigmoid aganglionosis has been reported at a rate of 23.2% and ultrashort aganglionosis at 2.3% [4,[10][11][12][13][14][15][16]. All of our cases had EA.…”