Functional intestinal obstruction associated with a
variety of pathologic conditions. Colonic motility disorder associated with
characteristic histochemical changes of the bowel wall.
Chronic Idiopathic Pseudoobstruction; Deficiency of
Argyrophil Myenteric Plexus; Intestinal Neuronal Dysplasia (IND) Type A;
Neuronal Intestinal Dysplasia.
Fewer than 20 patients have been reported.
Sporadic, autosomal dominant, and autosomal
Wide spectrum of pathologic disorders: (1)
neuronal intestinal dysplasia (NID) type A: hypoplasia or aplasia of the
intestinal sympathetic innervation; (2) NID type B: hyperplasia of the
submucosal parasympathetic plexus; and (3) other diseases: degenerative
processes involving the myenteric plexus, combinations of NID B and
aganglionosis (Hirschsprung), or hypoganglionosis. This group of disorders
results in ineffective intestinal propulsion with various degrees of
aperistalsis and clinical manifestations.
Clinical course. NID A: Younger than 6 months with bloody
stools and spastic diarrhea (in cases with immaturity of the sympathetic
innervation: complete cessation of the ulcerative colitis after temporary
colostomy). NID B: Severe constipation, subileus, sometimes attacks of or
persistent malabsorption (treatment usually conservative). Although NID B
appears clinically similar to Hirschsprung disease, there is no
aganglionosis of intramural plexuses. NID B may be associated with
intestinal malrotation, short bowel syndrome, ileal stenosis, colonic
atresia, or multiple endocrine neoplasias type II (MEN IIA and IIB). Some
infants with pyloric hypertrophy have an associated functional intestinal
obstruction with small intestine malrotation and short bowel syndrome.
Chronic neuropathic intestinal pseudoobstruction with ophthalmoplegia,
ptosis, hearing loss, and severe sensory and motor peripheral neuropathy
also described. Radiographic examination, electromanometry, and transit time
studies do not show pathognomonic criteria for specific neuronal intestinal
manifestations. Biopsies with histoimmunologic and histochemical analysis of
the involved areas give the final diagnosis.
As described under Diagnosis: wide spectrum of recurrent
episodes of intestinal obstruction in the absence of a mechanical blockage
of the lumen; occasional history of prenatal oligohydramnios; and failure to
thrive (some patients may be so malnourished that they require total
parenteral nutrition with its potential adverse effects). Consider the
possibility of associated gastrointestinal, endocrine, and neurologic
Obtain full history of
gastrointestinal symptoms, failure to thrive, and associated endocrine or
neurologic diseases. Evaluate patient's intravascular volume status:
clinically, plasma electrolytes, coagulation profile, and hemoglobin level. If on total parenteral
nutrition, obtain albumin, glucose, phosphate, calcium, and magnesium
levels, liver function tests. In rare cases of associated MEN IIA (medullary thyroid
carcinoma, parathyroid adenoma, pheochromocytoma) or MEN IIB (medullary thyroid
carcinoma, mucosal neuromas [tongue, buccal mucosa, lips, conjunctivae,
pheochromocytoma]): request endocrine consultation.
Optimization of fluid status most
recommended prior to anesthesia. Rapid-sequence induction in the presence of
intestinal obstruction or pseudoobstruction (use nondepolarizing muscle
relaxants if associated myopathy); intraoperative blood glucose levels if on
total parenteral nutrition. Expect prolonged postoperative mechanical
ventilation if associated peripheral neuropathy affecting respiratory
muscles or in presence of muscular hypotonia with MEN IIB. If MEN IIA or IIB associated
with NID B: continue antihypertensive ...