Hirschsprung Disease

Subject: Child Health Nursing

Overview

It is characterized by a congenital lack of parasympathetic ganglionic nerve cells from the muscle wall of the digestive tract, commonly at the distal ends of the colon, resulting in functional blockage with proximal colon dilatation and hypertrophy. Congenital an aganglionic megacolon is another name for it. The precise reason is unknown, but potential possibilities include a child with drowning syndrome, a congenital abnormality, and congenital cardiac disease. The signs and symptoms of Hirschsprung's disease differ depending on the severity of the disorder. Signs and symptoms usually develop immediately after delivery, but they might appear later in life as well. In a youngster, treatment may be symptomatic, such as isotonic enema, stool softener, and low residue diet. If the youngster has enterocolitis, resuscitation and care with intravenous fluid and antibiotics are required. In some circumstances, an emergency colostomy may be required.

It is characterized by a congenital lack of parasympathetic ganglionic nerve cells from the muscle wall of the digestive tract, commonly at the distal ends of the colon, resulting in functional blockage with proximal colon dilatation and hypertrophy. Congenital aganglionic megacolon is another name for it.

Causes

  • Unknown
  • Congenital defect (an arrest in embryonic development affecting the migration of parasympathetic nerve of the intestine, occurring prior to the 12th week of gestation.)
  • A child with Drown syndrome and congenital heart disease.
  • Some cases- may have a hereditary and familial tendency.

Clinical Presentation

The signs and symptoms of Hirschsprung's disease differ depending on the severity of the disorder. Signs and symptoms usually develop immediately after delivery, but they might appear later in life as well. The most noticeable symptom of Hirschsprung's disease is a newborn's failure to have a bowel movement within 48 hours of birth.

Other signs and symptoms in newborns may include:

  • Swollen belly
  • Vomiting, including vomiting a green or brown substance
  • Constipation or gas, which might make a newborn fussy
  • Diarrhea

In older children, signs and symptoms can include:

  • Swollen belly
  • Chronic constipation
  • Gas
  • Failure to gain weight
  • Fatigue

Diagnosis

  • Physical examination and history
  • Abdominal x-ray will reveal collection of gasses, fluid
  • Barium enema x-ray shows megacolon
  • Anorectal manometry: for this procedure balloon is inflated inside the rectum.
  • Rectal/colonic tissue biopsy reveals presence or absence of nerve cells. It is a most definitive test for HD.

Management

Management depends on age and manifestation of the infant and children.

In infant/older children

  • In a youngster, treatment may be symptomatic, such as isotonic enema, stool softener, and low residue diet.
  • If the youngster has enterocolitis, resuscitation and care with intravenous fluid and antibiotics are required. In some circumstances, an emergency colostomy may be required.
  • Surgical reconstruction.

In newborn

  • Fluid and electrolyte balance establishment
  • Refraining from drinking oral fluids
  • Surgical management is the specific treatment that comprises

Stage of surgery in Newborn

  • After the diagnosis is confirmed, a first colostomy is performed in the normal colon above the dilated portion.
  • Anastomosis surgery is performed 9-12 months later (child weight 6.8-9kg), which includes the excision of the aganglionic section. The typical ganglionic colon is pulled above the internal sphincter, and the components are anastomosed.
  • Colostomy closure is performed around 3 months after the second stage surgery or once the repaired section has established function.
  • The youngster may undergo bowel training therapy following surgery.

Nursing Management

  • Observe the passage of meconium, as well as bowel patterns such as constipation, stool features, and abdominal distension in newborns.
  • Examine the frequency and characteristics of stools in infants and children.
  • Avoid taking the infant's or children's temperature rectally.
  • Give the child any prescribed medication, such as antibiotic stool softener.
  • Assess and treat any respiratory problems caused by abdominal distention.
  • Observe and measure belly circumference on a regular basis.
  • Provide assistance to the child and his or her parents.
  • Assist with symptom management prior to surgery, including a low fiber, high calorie, high protein diet.
  • Nursing management also includes pre- and post-operative care at various stages of surgery.

Preoperative care

  • Assessment of child's health condition
  • Written consent from parents
  • Physical preparations: Emptying the bowel with repeated saline enemas and use of systemic antibiotics and colon irrigations
  • Insertion of nasogastric tube to prevent abdominal distension
  • Instilled antibiotics solution through the nasogastric tube to minimize infection
  • Vitals monitoring
  • Maintain intake output record
  • Regular measurement of abdominal girth
  • Preoperative medications; Painkillers, antibiotics, others
  • Improving breathing patterns
  • Regular vitals monitoring
  • Close assessment of respiratory patency i.e. watches for rapid, shallow respiration, cyanosis, abdominal distension and retraction
  • Elevate head and chest of baby and make comfortable position
  • Administer oxygen as ordered to support respiratory status.
  • Provide adequate nutrition
  • Obtain a dietary history, food habits, offer small frequent feeding and maintain nil per orally as needed before and after surgery
  • Administration of intravenous fluid and maintain intake output chart
  • Psychological preparation: In a case of an older child, give a clear explanation in a simple way to prevent anxiety, fear, and confusion regarding disease and treatment process. Prepare parents for surgery. For this, the nurse should assess parents previous knowledge regarding disease and its treatment.

Postoperative care

  • Assessment of child's general condition
  • Monitor vital signs and record properly
  • Maintain airway patency
  • Prevent from aspiration
  • Assess wound site and drainage tube if present for bleeding leakage
  • Change dressing using sterile technique
  • Prevent contamination from diaper; change diaper below dressing frequently
  • Prevent perineal and anal excoriation thorough cleaning and using ointment after the infant soils.
  • Follow careful hand washing technique
  • Report signs of wound infection
  • Encourage frequent coughing and deep breathing exercises to maintain respiratory status as well as to minimize respiratory complications in older children
  • Allow the baby to cry for short periods to prevent atelectasis
  • Change baby's position frequently to increase circulation and allow for aeration of all lung areas
  • Maintain patency of nasogastric tube postoperatively
  • Assess bowel sound regularly with measurement of abdominal girth
  • Maintain nil per orally until bowel sound return and the bowel is ready for feeding
  • Provide oral hygiene frequently and begin oral feeding as ordered
  • Monitor for proper functioning of colostomy if present
  • Note drainage from colostomy, its color, amount, composition
  • Note abdominal distension
  • Measure fluid loss from colostomy
  • Increased tenderness, irritability, vomiting, raised temperature
  • Supporting parents
  • Acknowledge that even a temporary colostomy will be a difficult procedure to accept and learn to manage
  • Supports parents when teaching them to care for colostomy
  • Involve the during changing dressing immediately after surgery
  • Reassure parents that colostomy will not cause delay in the child's normal growth development
  • Encourage parents to express their fear and anxieties: Anticipating future surgery for resection may be confusing and frightening
  • Teach parents about care of the child at home
  • Provide teaching on colostomy care
  • Colostomy pouch change, prevention, and treatment of skin excoriation around the stoma by keeping dry and clean
  • Dressing must be checked frequently for
  • Dressing and collection bags may not adhere well or stay in place, skin breakdown, and elimination patterns
  • Use appropriate size of ostomy equipment
  • Does colostomy works properly or not
  • Encourage care of the ostomy as part of normal activities of daily living
  • Involve the entire family in teaching to enhance acceptance of body change of the child
  • Procedure needs to be thoroughly understood and practiced, including preparation of skin, application of collecting appliance, care of appliance and control of order
  • Signs of stoma complications that include ribbons like stool, diarrhea, failure to evacuate stool or flatus, bleeding etc
  • Dilatation of stoma with finger allow the parents to learn and practice this procedure long before the child is to be discharge
  • Emphasis the importance of treating the child as normally as possible to prevent from behavior problems
  • Teach on: Nutrition, general growth, and development immunization, and follow up visit.

 
Things to remember
  • It is characterized by a congenital lack of parasympathetic ganglionic nerve cells from the muscle wall of the digestive tract, commonly at the distal ends of the colon, resulting in functional blockage with proximal colon dilatation and hypertrophy.
  • Congenital aganglionic megacolon is another name for it.
  • The precise cause is unknown, but other possibilities include a child with drowning syndrome, a congenital defect, and congenital heart disease.
  • The signs and symptoms of Hirschsprung's disease differ depending on the severity of the disorder. Signs and symptoms usually develop immediately after delivery, but they might appear later in life as well.
  • In a youngster, treatment may be symptomatic, such as isotonic enema, stool softener, and low residue diet.
  • If the youngster has enterocolitis, resuscitation and care with intravenous fluid and antibiotics are required. In some circumstances, an emergency colostomy may be required.
Videos for Hirschsprung Disease
Hirschsprung Disease
Questions and Answers

Management:

The age and symptoms of the infant and child will determine the management strategy.

In infant/older children:

  • A child with minor symptoms may just need symptomatic treatment, such as an isotonic enema, a stool softener, and a low residue diet.
  • Rapid resuscitation, intravenous fluid management, and antibiotics if the youngster has enterocolitis. In some circumstances, a colostomy may be required.
  • Surgical repairment.

In newborn:

  • Creation of a fluid and electrolyte balance.
  • Denying oral hydration.

Stage of surgery in Newborn:

  • An first colostomy is performed in the healthy colon above the dilated portion following diagnostic confirmation.
  • Aganglionic segment excision is done during anastomosis surgery, which is performed 9–12 months after the child's birth (child weight: 6.8–9 kg). the anastomosis of the components and pulling of the normal ganglionic colon above the internal sphincter.
  • Colostomy closure is carried out 3 months following the second stage of surgery or after the function of the repaired component has been established.
  • The youngster could need therapy for bowel training after surgery.

Nursing management:

  • Keep an eye out for meconium transit as well as bowel patterns like constipation, stool features, and abdominal distension in newborns.
  • Analyze the frequency and characteristics of an infant's or child's feces.
  • Do not rectally take the infant's or kids' temperature.
  • Give the child any prescribed medication, such as an antibiotic stool softener.
  • Evaluate and treat any breathing problems caused by abdominal distention.
  • periodically check in on and gauge abdominal circumference
  • Support the child's and the parents' needs.
  • Help with symptom management before to surgery, including a low-fiber, high-calorie, and high-protein diet.
  • During a distinct stage of surgery, nursing management also involves pre- and postoperative care.

Preoperative care:

  • Before and after surgery, make sure the infant or toddler is getting enough food and water.
  • Prepare both the parent and the child for various procedures and treatments.
  • Saline irrigations, systemic antibiotics, and colonic antibiotic irrigations should be used as directed to prepare the intestine for surgery. But there is no need to get the bowels ready for operation in newborns.
  • Preoperative treatment includes getting lab results and consent.

Postoperative care:

  • During the initial postoperative phase, keep the infant NPO.
  • Nasogastric drainage is included in the monitor I/O chart.
  • I/V fluid first, then oral fluid once bowel function has been established.
  • Treating the wound.
  • The provision of ostomy care includes skin care, application of the collecting appliances, appliance care, odor control, and monitoring for complications such as persistent diarrhea, stools with a ribbon-like appearance, bleeding, prolapse, etc.
  • Family education on in-home care
  • Describe the process and medication, such as enemas and stool softeners.
  • Instructions for colostomy dietary management include avoiding raw vegetables and fruits, whole grains, and following a low residue or low fiber diet.
  • Include them in the care process by explaining ostomy care.

A functional obstruction with proximal colon hypertrophy and dilation results from the congenital lack of parasympathetic ganglionic nerve cells from within the muscle wall of the intestinal tract, typically at the distal ends of the colon. The term "congenital aganglionic megacolon" is also used.

The submucosal (Meissner) plexus, the myenteric (Auerbach) plexus, and the smaller mucosal plexus are the three nerve plexuses that innervate the intestine. All of these plexuses are intricately connected and play a role in regulating blood flow as well as absorption, secretion, motility, and other bowel functions.

Intrinsic neurons are principally in charge of normal motility. Due to the intricate reflexive architecture of the enteric nervous system, bowel function remains adequate in the absence of external signals (ENS). The ENS is frequently referred to as the "second brain" because of this. Enteric ganglia regulate the contraction and relaxation of intestinal smooth muscle. Nitric oxide and other enteric neurotransmitters play a major role in the majority of enteric nerve activation's ability to relax muscles. Cholinergic and adrenergic fibers are found in extrinsic neuronal afferents to the ENS. While adrenergic fibers primarily cause inhibition, cholinergic fibers often cause contraction.

Hirschsprung disease patients lack the myenteric and submucosal plexuses. Aganglionosis extends proximally for a varying distance and always affects the anus. Control of the intestinal smooth muscle is primarily extrinsic in the absence of ENS responses. The cholinergic and adrenergic systems are 2-3 times more active than the normal gut. It is believed that an increase in smooth muscle tone results from the adrenergic (excitatory) system dominating the cholinergic (inhibitory) system. The diminished intrinsic enteric relaxing impulses leave the heightened muscular tone unchallenged. As a result of this phenomenon, there is an obstruction that prevents movement and an imbalance in the contractility of the smooth muscles.

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