Chronic postoperative constipation is present in 7 to 8% of the operated cases. Postoperative enterocolitis, a severe manifestation, is present in the 10–20% of operated patients. Epidemiology
According to a 1984 study conducted in Maryland, Hirschsprungs disease appears in 18.6 per 100,000 live births. In Japan, it occurs at a similar rate of about one in 5,000 births (20 per 100,000). It is more common in male than female (4.32:1) and in white rather than nonwhite. Nine percent of the Hirschsprung cases were also diagnosed as having Down syndrome. Most cases are diagnosed before the patient is 10 years of age. History
The first report of Hirschsprungs disease dates to 1691, when it was described by Dutch anatomist Frederik Ruysch. However, the disease is named after Harald Hirschsprung, the Danish physician who first described two infants who died of this disorder in 1888.Hirschsprungs disease is a congenital disorder of the colon in which certain nerve cells, known as ganglion cells, are absent, causing chronic constipation. In patients with Hirschsprung disease, both myenteric and submucosal plexuses are absent. A barium enema is the mainstay of diagnosis of Hirschsprungs, though a rectal biopsy showing the lack of ganglion cells is the only certain method of diagnosis.The first publication on an important genetic discovery of the disease was from Martucciello Giuseppe et al. in 1992. The authors described a case of a patient with total colonic aganglionosis associated with a 46, XX, del 10 (q11.21 q21.2) karyotype.
Duration and aggressiveness of offloading (non-weight-bearing vs. weight-bearing, non-removable vs. removable device) should be guided by clinical assessment of healing of neuropathic arthropathy based on edema, erythema, and skin temperature changes. It can take six to nine months for the edema and erythema of the affected joint to recede. Outcome
Outcomes vary depending on the location of the disease, the degree of damage to the joint, and whether surgical repair was necessary. Average healing times vary from 55 to 97 days, depending on location. Up to one to two years may be required for complete healing. Further reading
Neuropathic osteoarthropathy by Monica Bhargava, M.D., University of Washington Department of Radiology
John R. Crockarell; Daugherty, Kay; Jones, Linda Winstead; Frederick M. Azar; Beaty, James H; James H. Calandruccio; Peter G. Carnesale; Kevin B. Cleveland; Andrew H. Crenshaw (2003). Campbells Operative Orthopedics (10th ed.). Saint Louis, MO: C.V. Mosby. ISBN 0-323-01248-5. Gupta R (November 1993). "A short history of neuropathic arthropathy". Clinical Orthopaedics and Related Research (296): 43–9. PMID 8222448. Sommer TC, Lee TH (November 2001). "Charcot foot: the diagnostic dilemma". American Family Physician. 64 (9): 1591–8. PMID 11730314. References


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