Treatment of Hirschsprung’s Disease in Children

Treatment of Hirschsprung’s disease in children is performed by pediatric specialists at Herzliya Medical Center in Israel. The most advanced methods of treatment for Hirschsprung’s disease in Israel are utilized and paired with highly effective postoperative treatment for rehabilitation, allowing patients to return to a normal lifestyle within a short period.

What is Hirschsprung’s Disease?

Healthy bowel functions from the wavelike contraction of the smooth muscle – known as peristalsis. Ongoing peristalsis promotes intestinal health and absorption of fluid and nutrients; a necessary process in conjunction with the evacuation of feces. Hirschsprung’s disease is characterized by a congenital defect in the structure of nerve endings (lack of nerve ganglia) in the smooth muscles of the colon, which disturbs motor function. Motility disorders can lead to severe constipation, this may result in complete intestinal obstruction, along with other complications that may be life-threatening. The disease is hereditary and associated with the presence of other genetic mutations. Hirschsprung’s disease predominantly occurs in males and often observed among patients suffering from other hereditary diseases.

Forms of Hirschsprung’s Disease

Various forms of Hirschsprung’s disease depend on the location of the intestinal lesion, removal of a short segment of the colon. In the colon, distal portions, such as the rectum and sigmoid are most affected. The removal of the short segment of the colon is known to be the most commonly diagnosed form of Hirschsprung’s disease (75% of patients).

• In contrast, removal of a long segment of the colon is considered a relatively rare form of the disease, as there are no nerve endings in most of the large intestine, particularly in proximal, ascending, descending, and transverse colon
• Total removal the colon, as well as end portions of the small intestine,  are considered severe forms of the disease, leading to complications observed in early stages

Depending on the clinical course established, experts distinguish each patient’s development of Hirschsprung’s disease into two categories: compensated (stable) and decompensated (complicated)

Symptoms of Hirschsprung’s Disease in children

Symptoms of Hirschsprung’s disease are observed in infancy and early childhood. For 80% of patients, symptoms of the disease develop during the first weeks and months of life. In rarer cases, children are diagnosed much later (6-10 years). Symptoms of Hirschsprung’s disease are characterized by:

• Severe constipation
• Bloating
• Nausea
• Vomiting
• Malnutrition
• Delayed growth and development
• Disruption of water and electrolyte balance
• Development of secondary disorders, onset by ongoing impairments in the absorption of vital elements

Disease severity is clinically diagnosed thorough evaluation of the health of the colon, timely diagnosis, and the extent of symptomatic treatment that has been conducted. Severe cases of Hirschsprung’s disease have led to the development of toxic megacolon, sepsis (blood poisoning), and intestinal obstruction. Hirschsprung’s disease patients exhibiting such complications require emergency medical intervention.

Diagnosis for Hirschsprung’s Disease at HMC

At HMC in Israel, various diagnostic tools are used to screen for Hirschsprung’s disease as early as possible:

• Radiography and tomography – used for imaging of the intestine to determine the location and severity of the lesion
• Anorectal manometry – measures pressure in the rectum to evaluate the overall functioning and health status of the intestine
• A biopsy is used to conduct the precise, microscopic examination. For infants and young children, the least invasive techniques are used to aspire samples for biopsy from the mucous membrane of the colon. Endoscopic and surgical biopsy is carried out as the diagnostic process continues.

Hirschsprung’s Disease Treatment at HMC

Symptomatic therapy, including adherence to a strict diet, is often paired with the use of laxatives and cleansing enemas, but provide only a temporary effect. The vast majority of patients require surgical treatment.

Surgical Procedures for Hirschsprung’s disease involves the removal of the affected area of ​​the colon. The procedure is performed in one or two stages:

• One-stage surgery for Hirschsprung’s disease in Israel by intervention to internally connect (via anastomosis) sections of healthy bowel
• Two-stage surgery includes a colostomy (artificial stoma) that allows for the removal of feces from the body into a pouch. After a few months, an additional procedure is performed to preserve intestinal functional capacity by removal of the colon where ganglion cells are absent and closing the stoma initially created.

In the Department of Pediatrics at Herzliya Medical Center in Israel, several surgical options for treatment of Hirschsprung’s disease are made available to patients. Services are delivered to patients by a multidisciplinary team of pediatric professionals that work to provide comprehensive care aimed at full restoration of bowel function and to prevent complications. Early treatment that is timely and effective provides patients with the opportunity to achieve favorable prognosis among many cases of the disease.