GASTROINTESTINAL SYSTEM BLOCK

CASE 4B

Ronald Chrisbianto Gani 405090223 Faculty of Medicine 2009 Tarumanagara University

HIRSCHSPRUNG DISEASE (CONGENITAL AGANGLIONIC MEGACOLON)

HIRSCHPRUNG DISEASE
Is caused by abnormal inervation of the bowel. Most common cause of lower intestinal obstruction in neonates Male : Female = 4 : 1 Increased familial incidence Associated with another congenital defects, Down, Smith-Lemli-Opitz, Waardenburg, cartilage-hair hypoplasia, Ondine curse Associated with microcephaly, mental retardation, abnormal facies, autism, cleft palate, hydrocephalus, micrognathia
Nelson Textbook of Pediatrics 18th ed

PATHOLOGY
Result of an absence of ganglion cells in the bowel wall, extending proximally and continuously from the anus for variable distance Usually sporadic, dominant and recessive patterns of inheritance have been demonstrated in family groups Aganglionic segment is limited to the rectosigmoid in 75% cases. In 10% cases,entire colon lacks of ganglion Histologically, theres an absence of Meissner and Auerbach Plexus and hypertrophied nerve bundle
Nelson Textbook of Pediatrics 18th ed

CLINICAL MANIFESTATION
Delayed passage of meconium at birth FTT with hypoproteinemia (less common) Failure to pass stool dilatation of proximal bowel and abdominal distention. Stasis allow proliferation of bacteria can lead to enterocolitis Increasing difficulty with the passage of stool Large fecal mass is palpable in left lower abdomen Rectum is empty of feces The stools consist of small pellets, or fluid consistency Rectal examination demonstrated normal anal tone followed by explosive discharge of foul-smelling feces and gas

Nelson Textbook of Pediatrics 18th ed

DIAGNOSIS
Rectal Manometry : measures the pressure of internal anal sphincter, high accuracy but difficult to perform in young infants Rectal suction biopsy : evaluating the precense of ganglion cells Radiographic : presence of transitional zone after 1-2 week of age 24 hrs delayed film (barium)
Nelson Textbook of Pediatrics 18th ed

HIRSCHPRUNG DISEASE

Nelson Textbook of Pediatrics 18th ed

HIRSCHPRUNG DISEASE

Nelson Textbook of Pediatrics 18th ed

TREATMENT
Operative Intervention 3 basic surgical
Swenson : excise the aganglionic segment and anastomose the normal proximal bowel to the rectum 1-2cm above the dentate line Duhamel : procedure to create neorectum Boley : Endorectal pull through procedure

Nelson Textbook of Pediatrics 18th ed

PROGNOSIS
Generally satisfactory Postoperative problems include reccurent enterocolitis, stricture, prolapse, perianal abscess, fecal soiling.

Nelson Textbook of Pediatrics 18th ed