aganglionosis

aganglionosis

 [ah-gang″gle-on-o´sis]
congenital absence of parasympathetic ganglion cells, such as in congenital megacolon.

a·gan·gli·o·no·sis

(ā-gang'glē-ō-nō'sis),
The state of being without ganglia; for example, absence of ganglion cells from the myenteric plexus as a characteristic of congenital megacolon.
[G. ā- priv. + ganglion + -osis, condition]

aganglionosis

/agan·gli·on·o·sis/ (a-gang″gle-on-o´sis) congenital absence of parasympathetic ganglion cells.

aganglionosis

[əgang′lē·ənō′sis]
Etymology: Gk, a, not, gagglion, knot, osis, condition
an absence of parasympathetic ganglion cells in the myenteric plexus, a diagnostic sign of congenital megacolon.

a·gan·gli·o·no·sis

(ā-gang'glē-ŏ-nō'sis)
The state of being without ganglia; e.g., absence of ganglion cells from the myenteric plexus as a characteristic of congenital megacolon.
[G. ā- priv. + ganglion + -osis, condition]

aganglionosis, agangliosis

congenital absence of parasympathetic ganglion cells.

colonic aganglionosis
congenital defect manifested by stenosis, but patency, of the colon, an absence of ganglia of the myenteric plexus and an almost complete absence of skin pigment in white foals resulting from matings between overo horses. The foal develops colic at birth and dies after about 48 hours. See also atresia. Occurs also in some spotted mutant strains of mice. Called also congenital megacolon, aganglionic megacolon.
References in periodicals archive ?
In retrospect the resected distal ileum and cecum were analyzed and showed absence of ganglion cells, supporting the diagnosis of total aganglionosis.
Total aganglionosis associated with ileal atresia and malrotation
Waardenburg syndrome (WS) and Hirshsprung disease are examples of these neurocristopathies and patients suffer symptoms ranging from sensorineural hearing loss and pigmentation defects to intestinal aganglionosis.
In conclusion, calretinin provides a very reliable adjunctive test in the evaluation of RSBs for HD and, in our opinion, should be routinely used as an additional tool, similar to presence of hypertrophic nerves or AChE histochemistry, particularly because surgeons are often looking for an additional corroborative histologic finding to aganglionosis before undertaking surgery.
The loss of calretinin expression indicates aganglionosis in Hirschsprung's disease.
Discussion includes characteristics of classical and ultrashort Hirschsprung's disease; total intestinal aganglionosis and hypoganglionosis; new diseases like intestinal neuronal dysplasia, desmosis coli, leiomyopathy, architectural malformation, and stretching lesions of muscularis propria; and atrophic desmosis.
Luke Biggs aged 9 from Oxford has a total bowel aganglionosis which meant he could not eat until a bowel operation 18 months ago.
The grandmother (I2) carries the mutation but does not display a HSCR phenotype, suggesting that RET haploinsufficiency alone is necessary but not sufficient to cause aganglionosis.
An ileocolectomy specimen was received, and representative sequential sections of the total length were submitted for histologic examination, which confirmed the diagnosis of distal aganglionosis up to the hepatic flexure (Figure 3, a).
The HSCR phenotype is variable and can be classified into two groups: short-segment aganglionosis (SSA; 80% of cases), which includes patients with aganglionosis as far as the rectosigmoid junction; and long-segment aganglionosis (LSA; 20% of cases), which includes patients with aganglionosis beyond the rectosigmoid junction (1).
Three-dimensional morphology of gut innervation in total intestinal aganglionosis using whole-mount preparation.
Resected specimens of colon were obtained at the time of pull-through operation from 12 patients with HD, aged 21 days to 7 years; 10 patients had classic rectosigmoid HD, and 2 patients had total aganglionosis of the colon.