Intrinsic Malformations of the Anorectum

Fetal experiments by Pickard et al. [23] revealed that an intestinal obstruction evolving in early fetal life might cause secondary alterations in the plexus of the intestinal wall. According to our own studies in 1994 and 1996, aganglionosis, hypoganglionosis, intestinal neuronal dysplasia (IND), desmosis, and deficiency of interstitial cells of Cajal may occur [24-26]. We also observed a case with duplication of the plexus, and another where the internal anal sphincter consisted of longitudinal muscle fibers of the rectum instead of, as usual, the circular muscle layer (Fig. 25.8).

Martuciello et al. [27] examined 100 suction rectal biopsy samples from the fistulae, and at 6, 4, 3, and 2 cm distance to the opening of the fistula in 22 patients with ARM. They detected innervation disorders of the rectal wall in 81.8% of the biopsy samples. All female patients with vestibular fistulae exhibited dysganglionosis. Aganglionosis was found in 18% of all patients.

Masumoto et al. [26] described anomalies of enteric neurons, intestinal pacemaker cells, and smooth muscle in human intestinal atresia. They observed a hypoplasia of the myenteric ganglia and a marked reduction of intramuscular nerve fibers, including nitrergic neurons, in the dilated proximal segment of intestinal atresia. C-kit-positive cells were localized around the myenteric plexus, but were rarely found within the muscularis propria in the proximal segment. A reduced staining intensity for monoclonal antibodies to a-smooth muscle actin was observed mainly in the hypertrophic circular muscle layer of

Intramural Nerve Plexus

the proximal segment. These data suggest that anomalies of the intramural nerve plexus are less important for chronic constipation than is malformation or damage to the extramural cholinergic nerve fibers. In addition, biopsy samples of the rectal wall in 25 patients with severe postoperative constipation investigated by Holschneider et al. revealed no correlation between histomorphological findings and the clinical symptoms. Only four out of nine patients with agan-glionosis and two out of eight children with hypogan-glionosis suffered from severe chronic constipation. However, all patients without histological abnormalities showed normal bowel evacuations [7,13]. Therefore, aganglionosis, hypoganglionosis, and IND of the most distal part of the rectal pouch and in the fistula may represent a normal situation.

Absent Ganglion Cells

Fig. 25.8 Muscular and neuronal abnormalities of the blind pouch. A Absent ganglion cells of the internal anal sphincter, short hypoganglionic segment, and oral normalization of the myenteric plexus (arrow). B Higher magnification of the section shown in A. C The submoucus plexus (arrow) showing hypoganglionosis with a strongly reduced number of ganglia and ganglion cells inside the smaller ganglia. D Enlarged and heterotopic ganglia in the circular muscle layer of the rectal pouch (intestinal neuronal dysplasia; arrow). E Two completely doubled mesenteric plexus in the muscle wall of the rectal pouch (arrows)

Fig. 25.8 Muscular and neuronal abnormalities of the blind pouch. A Absent ganglion cells of the internal anal sphincter, short hypoganglionic segment, and oral normalization of the myenteric plexus (arrow). B Higher magnification of the section shown in A. C The submoucus plexus (arrow) showing hypoganglionosis with a strongly reduced number of ganglia and ganglion cells inside the smaller ganglia. D Enlarged and heterotopic ganglia in the circular muscle layer of the rectal pouch (intestinal neuronal dysplasia; arrow). E Two completely doubled mesenteric plexus in the muscle wall of the rectal pouch (arrows)

Constipation Prescription

Constipation Prescription

Did you ever think feeling angry and irritable could be a symptom of constipation? A horrible fullness and pressing sharp pains against the bladders can’t help but affect your mood. Sometimes you just want everyone to leave you alone and sleep to escape the pain. It is virtually impossible to be constipated and keep a sunny disposition. Follow the steps in this guide to alleviate constipation and lead a happier healthy life.

Get My Free Ebook


Post a comment