Scribd Logo
Upload

Welcome to Scribd!

  • 16 views

    Mwima Ongaro Vincent

    Uploaded by

    Malueth Angui
    1. Hirschsprung's disease is a developmental disorder of the enteric nervous system where there is an absence of intramural ganglion cells in parts of the colon. 2. It most commonly presents in neonates with symptoms like failure to pass meconium, abdominal distension, and vomiting. 3. Diagnosis involves tests like rectal biopsy and barium enema to determine the extent of the aganglionic segment. 4. Treatment involves supportive care and definitive surgery like Swenson's procedure or Duhamel operation to remove the aganglionic segment.

    Copyright:

    © All Rights Reserved
    Download as PPTX, PDF, TXT or read online from Scribd

    Mwima Ongaro Vincent

    Uploaded by

    Malueth Angui
    16 views17 pages
    1. Hirschsprung's disease is a developmental disorder of the enteric nervous system where there is an absence of intramural ganglion cells in parts of the colon. 2. It most commonly presents in neonates with symptoms like failure to pass meconium, abdominal distension, and vomiting. 3. Diagnosis involves tests like rectal biopsy and barium enema to determine the extent of the aganglionic segment. 4. Treatment involves supportive care and definitive surgery like Swenson's procedure or Duhamel operation to remove the aganglionic segment.

    Original Title

    Mwima Ongaro Vincent.pptx

    Copyright

    © © All Rights Reserved

    Available Formats

    PPTX, PDF, TXT or read online from Scribd

    Did you find this document useful?

    Is this content inappropriate?

    1. Hirschsprung's disease is a developmental disorder of the enteric nervous system where there is an absence of intramural ganglion cells in parts of the colon. 2. It most commonly presents in neonates with symptoms like failure to pass meconium, abdominal distension, and vomiting. 3. Diagnosis involves tests like rectal biopsy and barium enema to determine the extent of the aganglionic segment. 4. Treatment involves supportive care and definitive surgery like Swenson's procedure or Duhamel operation to remove the aganglionic segment.

    Copyright:

    © All Rights Reserved
    Download as PPTX, PDF, TXT or read online from Scribd
    Download as pptx, pdf, or txt
    16 views17 pages

    Mwima Ongaro Vincent

    Uploaded by

    Malueth Angui
    1. Hirschsprung's disease is a developmental disorder of the enteric nervous system where there is an absence of intramural ganglion cells in parts of the colon. 2. It most commonly presents in neonates with symptoms like failure to pass meconium, abdominal distension, and vomiting. 3. Diagnosis involves tests like rectal biopsy and barium enema to determine the extent of the aganglionic segment. 4. Treatment involves supportive care and definitive surgery like Swenson's procedure or Duhamel operation to remove the aganglionic segment.

    Copyright:

    © All Rights Reserved
    Download as PPTX, PDF, TXT or read online from Scribd
    Download as pptx, pdf, or txt
    of 17

    Mwima Ongaro Vincent

    TM226-1899/2014
    Introduction
    • Known as Congenital Aganglionic Megacolon or
    Aganglionic Megacolon
    • A developmental disorder of ENS; absence of
    intramural ganglion cells
    • Commonest cause of IO in neonates
    • Types;- short segment(75%), Long Segment(15%),
    Total colonic(10%)
    • Has 3 zones;-aganglionic, transitional &
    hypertrophied segments
    Introduction
    • Described by Ruysch in 1691
    • Popularized by Hirchsprung in 1886
    • Whitehouse & Kernohan- mid 20th century
    Epidiemology
    • 1 in 1500-7000 newborns annually
    • M:F=4:1;2:1(long segment)
    • Racial distribution is similar, though roughly 3X
    more in Asian Americans
    • Uncommon in premature infants
    • Median age of diagnosis: 2-6 months
    Associated anomalies
    • Down’s syndrome
    • Neurocristopathy syndromes
    • Waardenburg-Shah syndrome
    • Yemenite deaf-blind syndrome
    • Piebaldism
    • MEN2
    • CCHS (Ondine’s curse)
    Embryology & Etiology
    • 5th week-upper alimentary tract
    • 7th week- intestine, 12th week in the
    colon(myenteric first then Meissner’s plexus)
    Possible etiology
    • Arrest of aborral neural crest cells migration
    • Harsh environment(apoptosis, failure of
    proliferation, improper proliferation)
    Pathophysiology
    • In neonatal period the intestine is normal
    • The proximal portion hypertrophies
    • Taeniae disappear and the longitudinal muscle layer
    completely surrounds the colon
    • The distal portion is aganglionic
    • Marked increase in nerve fibers extending into sub-
    mucosa
    • Unbalanced smooth muscle contractility,
    uncoordinated peristalsis and functional obstruction
    Clinical manifestations
    Newborns
    • Failure to pass meconium within 48 hours
    • Abdominal distension
    • Bilious vomiting
    • Failure to thrive
    • Fever and bloody diarrhea
    • Enterocolitis
    Older children
    • Failure to gain weight
    • Malnutrition
    • Chronic progressive constipation
    • Recurrent fecal impaction
    • History of pencil thin stools
    • Abdominal distension
    • Ribbon like/fluid/pellet form stools
    Diagnosis
    • History
    • Physical examination
    • Plain x-ray- shows intestinal obstruction
    • Rectal biopsy- definitive
    • Barium enema- shows extent and zones
    • Anorectal manometry- the rectoanl reflex is
    absent
    Differentials
    • Meconium plug syndrome
    • Small left colon syndrome
    • Distal ileal atresia
    • Cystic fibrosis(meconium ileus)
    • Low imperforate anus
    • Prematurity
    • Hypothyroidism
    • Neonatal sepsis
    • Currarino triad
    Treatment
    Supportive
    • Manage fluid and electrolyte imbalance
    • Antibiotics(Ampicillin, Gentamicin & Metronidazole)
    Definitive treatment
    Principles
    • Decompression and improving the nutrition
    • Establish the extent of disease
    • Definitive surgery
    Common surgical procedures
    • Swenson’s procedure
    • Duhamel operation
    • Soave’s mucosectomy
    • Ileo-anal anastomosis
    • Anal-rectal myectomy
    Complications
    • Constipation
    • Recurrent enterocolitis
    • Stricture
    • Prolapse
    • Perianal abcess
    • Fecal soiling
    Prognosis
    • >90% of the patients report satisfactory results
    • 1% may have debilitating incontinence requiring
    permanent colostomy
    • Total conic aganglionosis has poorer outcomes,
    33% of patients experience persistence
    incontinence, 14% requiring permanent ileostomy
    • Association with other abnormalities/syndromes
    has poor clinical outcomes

    You might also like