RSUD MAKASSAR

Hirschsprung Disease
 Case Report
A 5- months old male admitted to General
Pediatric the University Hospital Center of Tirana
with diagnosis of admission Acute Diarrhea. The
child A.B has 2 days with bilious emesis,
abdominal distention, loss of appetite, dimness,
subfebrile temperature. In physical examination
he appeared ill. The history of life the mother
refered that child has passed meconium 48
hours after births. The child was born premature
with cesarean secion, birth weight 3200gr.
 Case report
On physical examination showed a markedly
distended abdomen, his facies was pale and
suffered. Laboratory investigation on admission
revealed a blood cell count WBC 5500 (45 %
lymphocytes, 7% monocytes, 48% granulocytes
), RBC 4 540000, hemoglobin level 9.6 g dl,
hemotocrit value 36.5 %, plated count 460000,
total protein 5.1g dl, kalium value 3.1mmol l,
chloride value 112mmol l.
 Case report
Abdominal ultrasonography showed dilatation
of descendent colon, sigma and rectum. Plain
abdominal radiography showed a dilatation
descedent and ascedent colon. Barium enema
showed the aganglionic distal segment was
narrow with distended normal ganglionic bowel
above it. We looked a transition zone, delayed
evacuation 24 h
 Case report
In the examination of rectum that make gastro-
hepotologic pediatric showed rectal ampulla
was empty. We consulted with pediatric surgeon
and decided to make rectal biopsy that result no
ganglion cells. The patient was started antibiotic
treatment with cephalosporin for prevent
complication of HD. He treated with rehydration
therapy.
 Sign and symptoms
Typically, the most obvious sign of Hirschsprung's
disease is a newborn's failure to have a bowel
movement within 48 hours after 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
A plain abdominal radiograph may show a
dilated small bowel or proximal colon
Contrast enema radiographs of the colon
commonly are normal for the first three
months of life and indefinitely in patients with
total colonic disease.
 diagnosis
After the dilation process begins, the diseased
portion of the colon will appear normal and the
more proximal colon will be dilated. A
transition zone (the point where the normal
bowel becomes aganglionic) may be visible on a
contrast enema radiograph
 diagnosis
Anal manometry (balloon distention of the
rectum) demonstrates the absence of internal
anal sphincter relaxation upon rectal
distention
 Treatment
Hirschsprung's disease is treated with surgery to
bypass the part of the colon that has no nerve
(ganglia) cells. The lining of the diseased part of
the colon is stripped away, and normal colon is
pulled through the colon from the inside and
attached to the anus. This is usually done using
minimally invasive (laparoscopic) methods,
operating through the anus.
 Treatment
In children who are very ill, surgery may be done
in two steps. First, the abnormal portion of the
colon is removed and the top, healthy portion of
the colon is connected to a small hole (ostomy)
the surgeon creates in the child's abdomen.
Stool then leaves the body through the ostomy
into a bag that attaches to the end of the
intestine that protrudes through the hole in the
abdomen. This allows time for the lower part of
the colon to heal.