Vomiting - Case 3-6: 10-Month-Old Girl
Vomiting - Case 3-6: 10-Month-Old Girl: Excerpt from Pediatric Complaints and Diagnostic Dilemmas
I. History of Present Illness
A 10-month-old girl presented with a 1-day history of vomiting and fever to 38.3°C. The emesis was nonbloody and nonbilious. She had a history of constipation
and failure to thrive starting at 4 months of age. She had had no recent
changes in her stooling pattern of once a week. Stooling was painful, but there
was no blood or mucus. Her mother used prune juice, Karo syrup, and laxatives
to aid the patient
's bowel movements. Her mother also claimed that her daughter had always had a
distended abdomen.
II. Past Medical History
The patient had a history of constipation, failure to thrive (she was growing at
the 50th percentile until 4 months of age), anemia, hypotonia, and
developmental delay.
III. Physical Examination
T, 38.7°C; RR, 56/min; HR, 150 bpm; BP, 92/50 mm Hg
Weight, 6.2 kg; length, 66 cm; head circumference, 40.5 cm—all significantly less than the 5th percentile for age
General examination revealed a pale, crying infant with a distended abdomen. Her
heart and lungs were normal, but her abdomen was distended and tender, with
hypoactive bowel sounds and a palpable mass in the right lower quadrant. There
was no hepatomegaly or splenomegaly. Her neurologic examination was notable for
general hypotonia.
IV. Diagnostic Studies
Laboratory evaluation revealed 25,000 WBCs/mm3, with 81% segmented neutrophils, 10% lymphocytes, and 6% monocytes. The
hemoglobin was 8.7 g/dL with hypochromia, occasional schistocytes, and burr
cells. The mean corpuscular volume (MCV) was 74.6 fL, the red blood cell
distribution width index (RDW) was 23.2; and the reticulocyte count was 2.2%.
The platelet count was 649,000 cells/mm
3. Serum chemistry results were as follows: sodium, 137 mEq/L; potassium, 4.9
mEq/L; chloride, 115 mEq/L; bicarbonate, 18 mEq/L; BUN, 3 mg/dL; creatinine,
0.2 mg/dL; glucose, 98 mg/dL; alkaline phosphatase, 77 U/L; total bilirubin,
0.8 mg/dL; ALT, 98 U/L; and AST, 156 U/L.
V. Course of Illness
The chest radiograph was normal. An abdominal radiograph revealed findings that
led to an immediate therapeutic procedure (Fig. 3-5).
Discussion: Case 3-6
I. Differential Diagnosis
Vomiting in a child with a distended, tender abdomen; a palpable mass in the
right lower quadrant; and an abdominal radiograph revealing a large
pneumoperitoneum is considered a ruptured hollow viscus until proved otherwise
and requires immediate surgical exploration. Once the perforation has been
identified and resected, the cause of the perforation must be discovered and
corrected.
Vomiting in an infant with a history of chronic constipation could be caused by
mechanical obstruction resulting from tumors, malrotation with volvulus,
congenital bands, strictures, or duplications. Intestinal problems that can
cause constipation include neuropathies, myopathies, and Hirschsprung
's disease. Extraintestinal problems include endocrine disorders, hypothyroidism,
hypokalemia, hypercalcemia, infant botulism, certain medications, familial
dysautonomia, and spinal cord lesions. Finally, functional disorders caused by
a slow transit time or by dietary abnormalities can also cause constipation.
II. Diagnosis
The abdominal radiograph showed a moderate amount of free air in the abdomen,
suggesting bowel perforation (see Fig. 3-5). The patient was taken to the
operating room, where a large rush of air was felt when the abdominal cavity
was entered. A massively dilated colon was discovered, and an 18-cm segment of
the colon was resected after a 3-mm perforation was found in the sigmoid colon.
Pathologic review of the resected segment confirmed the diagnosis of
Hirschsprung
's disease.
III. Incidence and Epidemiology of Hirschsprung's Disease
Hirschsprung initially reported this condition in 1888, but it was not until the
1920s that the absence of ganglion cells in the distal gastrointestinal tract
was discovered. Hirschsprung
's disease has an incidence of 1 in 5,000 live births, with a male predominance
of almost 4:1. Ninety percent of cases occur in full-term infants, and it is
the most common cause of lower intestinal obstruction in neonates. A family
history is noted in 7%, and if the cecum is involved the proportion rises to
21%. Hirschsprung
's disease occurs in up to 10% of patients with Down syndrome and is associated
with other conditions such as Smith-Lemli-Opitz syndrome, Waardenburg syndrome,
congenital deafness, Laurence-Moon-Biedl-Bardet syndrome, and Ondine
's curse.
Hirschsprung's disease is caused by the failed cephalocaudal migration of neural crest cells
during the 5th through 12th week of gestation. The absence of neurons begins at
the anus and extends proximally. This defect is limited to the rectal and
sigmoid area in 75% of patients and includes the total colon in only 8%.
Absence of these neural crest cells interrupts the inhibitory parasympathetic
nerves in the myenteric plexus, thereby inhibiting relaxation and causing
unopposed contraction.
IV. Clinical Presentation
The clinical presentation of patients with Hirschsprung's disease depends on the length of the aganglionic segment. Ninety-four percent
of all patients with Hirschsprung
's disease fail to pass meconium within the first 24 hours of life. Other
symptoms include constipation (93%), vomiting (64%), diarrhea (25%), rectal
fissures with bleeding (5%), and bowel perforation (3%). Abdominal distention
occurs in 83%, and rectal examination reveals an empty rectal vault in 60%
(especially with long-segment Hirschsprung
's disease) or rectal impaction in 7% (especially with short-segment Hirschsprung's disease).
V. Diagnostic Approach
Only 15% of patients are diagnosed in the first month of life, 65% by 3 months,
and 80% by 1 year of age; almost 10% are diagnosed after 3 years of age. Early
diagnosis is associated with a decreased incidence of enterocolitis and
mortality.
Abdominal radiographs. Plain abdominal radiographs show a paucity of air in the distal rectum.
Barium enema. Barium enema is diagnostic in 80% of cases but may be inconclusive or falsely
negative in cases of short-segment disease and in patients who are younger than
1 month of age. The colon must be unprepared; in inconclusive cases, follow-up
films 24 hours later that show retained barium suggest the disease.
Anal manometry. If barium enema is inconclusive, anal manometry can rule out Hirschsprung's disease by the demonstration of normal relaxation.
Rectal biopsy. The most accurate diagnosis is acetylcholinesterase staining of a suction rectal
biopsy specimen that shows thick acetylcholinesterase-positive nerve fibers in
the muscularis mucosa. This finding is pathognomonic for Hirschsprung
's disease. If the diagnosis is still in doubt, the demonstration of
aganglionosis in the myenteric and submucosal plexuses of full-thickness biopsy
specimens identifies 98% of all cases.
VI. Treatment
Medical treatment with fluids and antibiotics, if necessary, is used to
stabilize the patient before surgery. Surgical treatment includes resection of
the aganglionic segment, with the creation of a stoma until 6 to 12 months of
age. Ninety percent of patients return to a normal stooling pattern, and the
remaining 10% have a continuing problem with either constipation or
incontinence. Death occurs in one third of those patients presenting with
enterocolitis.
VII. References
1. Curran TJ, Raffensperger JG. Laparoscopic endorectal pull-through: a
comparison with the open procedure.
J Pediatr Surg 1996;31:1155–1157.
2. Milla PJ. Hirschsprung disease and other neuropathies. In: Rudolph CD,
Rudolph AM, eds.
Rudolph's pediatrics, 21st ed. New York: McGraw-Hill, 2003:1461–1463.
3. Sherman JO, Snyder ME, Weitzman JJ, et al. A 4-year multinational
retrospective study of 880 Swenson procedures.
J Pediatr Surg 1989;24:833–838.
4. Swenson O. Hirschsprung's disease: a review. Pediatrics 2002;109:914–918.
Book Source Details
- Book Title: Pediatric Complaints and Diagnostic Dilemmas
- Author(s): Samir S Shah MD; Stephen Ludwig MD
- Year of Publication: 2003
- Copyright Details: Pediatric Complaints and Diagnostic Dilemmas, Copyright © 2003 Lippincott Williams & Wilkins.
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