eMedicine Specialties > Pediatrics: General Medicine > Gastroenterology

Hirschsprung Disease: Follow-up

Author: Holly L Neville, MD, Assistant Professor of Clinical Surgery, Division of Pediatric Surgery, University of Miami Miller School of Medicine
Contributor Information and Disclosures

Updated: Nov 17, 2008

Follow-up

Further Outpatient Care

  • Prior to surgical intervention in patients with Hirschsprung disease, perform close follow-up care to be sure the colon is adequately decompressed and that signs or symptoms of enterocolitis do not develop. Teach the family techniques of decompression and rectal irrigation because these therapies aid in decreasing colonic dilation in preparation for surgery.
  • Preoperatively, counsel the family as to the available surgical options. If the child is to undergo a staged procedure or have a permanent ostomy, provide preliminary instruction about ostomy care to the family.
  • Postoperatively, patients need close follow-up care to assess healing as well as a screen for potential complications (eg, stricture formation). Outpatient dilations may be necessary to alleviate strictures and should be expected in patients who undergo a single-stage pull-through procedure in the newborn period.

Complications

  • Postoperative complications may include intermittent fecal soiling and incontinence, anastomotic leak, stricture formation, intestinal obstruction, and enterocolitis.

Prognosis

  • The outcome in infants and children with Hirschsprung disease is generally quite good. Most children obtain fecal continence and control. However, children with Down syndrome may be expected to have lower rates of continence, and some authors support placement of a permanent ostomy.

Patient Education

  • Alert patients and their families to potential preoperative and postoperative complications of Hirschsprung disease. When applicable, teach patients and their families how to care for an ostomy.

Miscellaneous

Medicolegal Pitfalls

  • The diagnosis is confirmed based on the histologic examination. In institutions that do not have a pediatric pathologist, the slides should be sent out for an expert opinion prior to proceeding with surgical intervention.
  • Parents of infants with Hirschsprung disease must be carefully counseled as to the risks and benefits of enterocolitis both preoperatively and postoperatively. The mortality rate associated with untreated enterocolitis is high yet preventable with proper parental education and immediate recognition and therapy.

Special Concerns

  • Consider the diagnosis of Hirschsprung disease in any neonate without passage of meconium in the first 48 hours of life. Also consider this disease in infants and children with chronic constipation that is refractory to treatment.
  • Enterocolitis is a potentially fatal complication that occurs in 10-30% of children with Hirschsprung disease and requires rapid diagnosis and institution of intravenous hydration, intestinal decompression, rectal washouts, and broad-spectrum antibiotics. Although enterocolitis most commonly occurs in the preoperative period, it may also occur postoperatively, which requires the same treatment.
  • Infants who lack ganglion cells throughout their entire colons are said to have total aganglionosis coli. Early diagnosis and prompt surgical care are essential to prevent serious complications as enterocolitis. In some infants, the distal small bowel may be affected as well. Confirm diagnosis by surgical exploration and multiple biopsies. Ileostomy at the level of normally innervated bowel may be lifesaving.
 
Acknowledgments

The authors and editors of eMedicine gratefully acknowledge the contributions of previous author Charles Cox Jr, MD, to the development and writing of this article.



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Multimedia: Hirschsprung Disease
References
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Further Reading

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Keywords

Hirschsprung disease, Hirschsprung's disease, Hirschsprung enterocolitis, Hirschsprung's enterocolitis, congenital aganglionosis, congenital megacolon, megacolon congenitum, Hirschsprung's disease, enterocolitis, abdominal distention, outflow incontinence, transmural intestinal necrosis, intestinal perforation, neonatal meconium plug syndrome, multiple endocrine neoplasia, MEN, Waardenburg syndrome, congenital deafness, malrotation, gastric diverticulum, intestinal atresia

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Contributor Information and Disclosures

Author

Holly L Neville, MD, Assistant Professor of Clinical Surgery, Division of Pediatric Surgery, University of Miami Miller School of Medicine
Holly L Neville, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Surgeons, American Pediatric Surgical Association, and Association of Women Surgeons
Disclosure: Nothing to disclose.

Medical Editor

Hisham Nazer, MB, BCh, FRCP, DCh, DTM&H, Professor of Pediatrics, Consultant in Pediatric Gastroenterology, Hepatology and Clinical Nutrition, Bushnaq Medical Centre, University of Jordan
Hisham Nazer, MB, BCh, FRCP, DCh, DTM&H is a member of the following medical societies: Royal College of Paediatrics and Child Health, Royal College of Physicians, Royal College of Surgeons in Ireland, Royal College of Surgeons of Edinburgh, and Royal Society of Tropical Medicine and Hygiene
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from broker recommendation; Avanir Pharma Stock Investment from broker recommendation

CME Editor

Steven M Schwarz, MD, FAAP, FACN, AGAF, Professor of Pediatrics, State University of New York, Downstate Medical Center College of Medicine; Distinguished Lecturer, New York Medical College, School of Public Health
Steven M Schwarz, MD, FAAP, FACN, AGAF is a member of the following medical societies: American Academy of Pediatrics, American College of Nutrition, American College of Physician Executives, American Gastroenterological Association, American Pediatric Society, Gastroenterology Research Group, New York Academy of Medicine, North American Society for Pediatric Gastroenterology and Nutrition, and Society for Pediatric Research
Disclosure: TAP Pharmaceuticals Honoraria Speaking and teaching; Curemark, LLC Consulting fee Board membership

Chief Editor

Carmen Cuffari, MD, Associate Professor, Department of Pediatrics, Division of Gastroenterology/Nutrition, Johns Hopkins University School of Medicine
Carmen Cuffari, MD is a member of the following medical societies: American College of Gastroenterology, American Gastroenterological Association, North American Society for Pediatric Gastroenterology, Hepatology and Nutrition, and Royal College of Physicians and Surgeons of Canada
Disclosure: Nothing to disclose.

 
 
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