Download Treatment for Hirschsprung`s Disease

Treatment for Hirschsprung
Disease
to treat the complications of
unrecognized or untreated
Hirschsprung disease
to institute temporary
measures until definitive
reconstructive surgery can
take place
to manage bowel function
after reconstructive
surgery
General Goals for Treatment
• Management of complications of recognized aganglionosis is directed
toward:
– reestablishing normal fluid and electrolyte balance
– preventing bowel overdistension (with possible perforation)
– managing complications, such as sepsis
• Institute temporary measures until definitive reconstructive surgery can
take place
• Manage bowel function after reconstructive surgery.
• Cornerstones of initial medical management
– intravenous hydration
– nasogastric decompression
– as indicated, administration of intravenous antibiotics
• Cardiac evaluation and genetic testing may be warranted
Surgery
• Traditionally, treatment also includes creating a
diverting colostomy at the time of diagnosis, and,
once the child grows and weighs more than 10
kg, the definitive repair is performed.
– But, now, definitive treatment should be done ASAP
• Definitive treatment: Swenson, Duhamel, and
Soave procedures.
– Clean the colon before repair
• Laparoscopic pull through procedures
Definitive Treatments
• Swenson procedure
– The aganglionic segment is resected down to the sigmoid colon and
the remaining rectum, and an oblique anastomosis is performed
between the normal colon and the low rectum.
• Duhamel procedure
– Key points are that a retrorectal approach is used and a significant
portion of aganglionic rectum is retained.
– The aganglionic bowel is resected down to the rectum, and the rectum
is oversewn. The proximal bowel is then brought through the
retrorectal space (between the rectum and sacrum), and an end-toside anastomosis is performed on the remaining rectum.
• Soave (endorectal) procedure
– The Soave procedure consists of removing the mucosa and submucosa
of the rectum and pulling the ganglionic bowel through the
aganglionic muscular cuff of the rectum.
Others
• Transanal pull-through
• Stem cell transplantation
Diet and Activity
• NPO before the operation
• Institute tube feeding or formula/breast milk
once bowel function resumes.
• High-fiber diets and diets containing fresh fruits
and vegetables may optimize postoperative
bowel function in certain patients.
• Limit physical activity for about 6 weeks to allow
the wound to heal properly
Continuing Pharmacotherapy
• Goals
– eradicate infection
– reduce morbidity
– prevent complications.
• Antibiotics
– empiric antimicrobial therapy: comprehensive and
cover all likely pathogens
– selection should be guided by blood culture
sensitivity whenever feasible.
Follow-up and Counseling
• Monitor for postoperative complications and offering
counseling and self-help resources to the patient’s
family
• Stress the importance of a high-fiber diet
• constipation and bowel stasis are thought to increase the risk of
enterocolitis.
• Consider the conditions that are associated with
Hirschsprung’s disease
• Counsel parents on the risk of the disease in the
patient’s siblings - genetics
• Introduce to support groups
Complications and Prognosis
• Most patients treated for Hirschsprung’s disease do
not have complications
• However,
• >10%, constipation
• <1%, fecal incontinence
• enterocolitis (17-50%)and colonic rupture- most serious
complications
– enterocolitis- most commonly by intestinal obstruction and residual
aganglionic bowel.
• Ultra-short segment Hirschsprung has the best
prognosis.
• Total colonic aganglionosis is the most severe type.
Poor prognosis.