Poor Weight Gain - Case 6-4: 5-Day-Old Boy
I. History of Present Illness
A 5-day-old Asian boy was brought to his pediatrician for poor feeding. Compared
with his siblings, his parents believed that he had been a poor feeder since
birth. He was taking only one-half ounce every 2 to 3 hours. The mother had
initially breast-fed the baby, but she had begun supplementing with formula
because of jaundice. In the clinic, the child was noted to have respiratory
distress and was believed to have a cardiac murmur. He was referred to the
emergency department for further evaluation. In the review of symptoms, there
were no fevers or vomiting. The urine and stool patterns were normal. There was
decreased activity but no diaphoresis with feeds, cyanosis, or abnormal
movements. There was no rash. The infant had been in contact with a 2-year-old
sibling who had emesis and gastroenteritis.
II. Past Medical History
Prenatal care had been good, and a prenatal ultrasound was normal. The infant
was born by spontaneous vaginal delivery at 39 weeks
' gestation, with a birth weight of 3,100 g. The mother was positive for group B Streptococcus and received intrapartum penicillin. The infant received antibiotics empirically
for the first 48 hours of life due to tachypnea but was discharged home with
clinical improvement after cultures were negative. The family history was
remarkable for leukemia in the maternal grandmother. The infant lived with his
mother, father, and a 2-year-old sibling. The maternal grandfather died
unexpectedly 2 days before the child
's hospital admission.
III. Physical Examination
T, 36.1°C; RR, 44/min; HR, 168 bpm; BP in right arm, 74/44 mm Hg; BP in left arm, 85/53
mm Hg; BP in right leg, 77/57 mm Hg; BP in left leg, 77/53 mm Hg; SpO
2, 98% room air
Weight, 2.9 kg
In general, the infant was awake, crying, and vigorous. The anterior fontanel
was open and flat. There were no dysmorphic facial features. There were
ecchymoses surrounding both eyes. The tympanic membranes were normal in
appearance and mobility. There was no rhinorrhea. The mucosa was pink and
moist, and the oropharynx was clear. There were mild intercostal retractions,
but the lungs were clear to auscultation. A IV//VI systolic murmur was noted at
the sternal border and radiated to the back. S1 and S2 were normal, but an S3
gallop was noted. The point of maximal impulse was displaced slightly to the
left. The abdomen was soft, and the liver edge was palpable 5 cm below the
right costal margin. The spleen was not palpable. There were no palpable
abdominal masses. The skin was jaundiced to the waist. Distal pulses were
normal and symmetric. The neurologic examination was normal.
IV. Diagnostic Studies
The chest radiograph revealed cardiomegaly and increased interstitial edema but
no discrete infiltrates. The complete blood count demonstrated the following:
15,500 WBCs/mm
3 (73% segmented neutrophils, 3% eosinophils, 13% lymphocytes, and 11%
monocytes); hemoglobin, 21.5 g/dL; and 140,000 platelets/mm
3. Serum electrolyte findings were as follows: sodium, 140 mEq/L; potassium, 3.9
mEq/L; chloride, 105 mEq/L; and bicarbonate, 15 mEq/L. Tests of liver function
were also abnormal: albumin, 3.0 mg/dL; total bilirubin, 17.5 mg/dL, ALT, 54
IU/L; AST, 163 IU/L; and
γ-glutamyltransferase (GGT), 700 U/L. The PTT was 28 seconds.
The electrocardiogram showed diffuse T-wave abnormalities but normal voltage.
Echocardiography showed a diffusely dilated heart with a particularly large
left ventricle. The shortening fraction was 22%. The heart was otherwise
structurally normal. Lumbar puncture revealed 17 WBCs and 39 red blood cells
per cubic millimeter; the protein and glucose concentrations were normal. No
bacteria were seen on Gram staining of the cerebrospinal fluid (CSF). A herpes
simplex virus PCR study from the CSF was negative.
V. Course of Illness
On admission, he was believed to have congestive heart failure and was treated
with digoxin and furosemide. Initially, cardiomyopathy and myocarditis were
considered the most likely causes. Viral infections (coxsackievirus,
enteroviruses, TORCH infections), bacterial infections, and metabolic storage
diseases were all considered. The infant was initially treated with ampicillin,
cefotaxime, and acyclovir until those studies returned negative. A head
ultrasound study was normal; specifically, no cerebral arteriovenous
malformation was visualized. He continued to have mild elevation in his liver
function tests, and an abdominal ultrasound examination was performed on the
second hospital day. It was remarkable for several masses of increased
echogenicity involving most of the right lobe of the liver.
Discussion: Case 6-4
I. Differential Diagnosis
This child presented in heart failure as the cause of his growth failure. The
cause of heart failure may be cardiac or noncardiac, as shown in Table 6-9.
Table 6-10 shows the differential diagnosis of a liver mass in a child.
II. Diagnosis
CT of the abdomen to further evaluate the hepatic lesions revealed large
heterogeneous lesions with enhancements in the periphery. The hepatic artery
was slightly dilated, which may have been the cause of the significant shunting
into the lesion. Magnetic resonance imaging (MRI) of the liver was then
performed to obtain better detail of the liver process. On MRI, the patient was
believed to have a mass lesion rather than an arteriovenous malformation. He
underwent open biopsy of the liver mass and Broviac catheter placement. On
biopsy and special stains, the mass was believed to be most consistent with a
hemangioendothelioma.
The findings in this case resulted from a large hemangioendothelioma or
cavernous hemangioma.
These lesions can cause congestive heart failure of the high-output type.
III. Incidence and Epidemiology
Liver tumors are rare in children, and approximately 30% are benign
(nonmalignant). Benign liver tumors may be classified into five groups: (a)
tumor-like epithelial lesions (e.g., focal nodular hyperplasia); (b) epithelial
tumors; (c) cysts and mesenchymal lesions (e.g., cystic mesenchymal
hamartomas); (d) benign teratomas; and (e) mesenchymal tumors (e.g.,
hemangiomas, hemangioendotheliomas). Of these benign lesions,
hemangioendothelioma is the most common. Hemangioendotheliomas are soft tissue
tumors that demonstrate endothelial proliferation. Like cutaneous hemangiomas,
these hepatic tumors increase in size during the first year of life and then
undergo involution over a period of several months to years.
IV. Clinical Presentation
Most affected children (85%) present in the first 6 months of life. Before tumor
involution, findings may include a palpable liver mass, jaundice, weight loss,
anorexia, and fever. Life-threatening complications include rupture with
hemorrhage, anemia, obstructive jaundice, and, as in this case, congestive
heart failure. Many children have associated cutaneous hemangiomas,
particularly if there are multiple hepatic lesions. Similar lesions may be
found in the trachea, lungs, gastrointestinal tract, spleen, and pancreas.
V. Diagnostic Approaches
The diagnostic approach is based first on ultrasound examination, followed by CT
or MRI with contrast. Measurements of
α-fetoproteins (AFP) and liver enzymes are helpful. A young child with multiple
lesions and normal AFP most likely has hemangioendothelioma. An older child
with elevated AFP most likely has hepatocellular carcinoma.
Complete blood count. Anemia is noted in 50% of patients. Thrombocytopenia, when present, may range
from mild to severe.
Liver function tests. AST is frequently elevated. High bilirubin levels are usually caused by
obstructive jaundice.
Abdominal ultrasound. Hemangioendotheliomas are usually hypoechoic with well-defined margins. Doppler
flow studies reveal high-flow velocity. Differentiation from arteriovenous
malformations may be difficult by ultrasonography.
Abdominal computed tomography. On CT, a low-attenuation mass with calcification is seen in approximately 40% of
cases. Multifocal lesions are less likely to demonstrate calcification. These
lesions usually have peripheral enhancement with central hypoattenuation due to
central infarction or hemorrhage in larger lesions.
Abdominal magnetic resonance imaging. On MRI, large lesions are heterogeneous, with occasional high signal on
T1-weighted imaging suggesting central hemorrhage.
Other studies. Biopsy is often required to distinguish these tumors from other hepatic masses.
VI. Treatment
A hemangioendothelioma may be resected if it is singular and uncomplicated by
thrombocytopenia. Nonoperative treatment with prednisone or interferon-
α, or both, is the treatment of choice. Cyclophosphamide has also been used, as
has radiotherapy. Liver transplantation may be needed.
VII. References
1. Reis-Filho JS, Paiva ME, Lopes JM. Congenital composite hemangioendothelioma:
case report and reappraisal of the hemangioendothelioma spectrum.
J Cutaneous Pathol 2002;29:226–231.
2. Lu CC, Ko SF, Liang CD, et al. Infantile hepatic hemangioendothelioma
presenting as early heart failure: report of two cases.
Chang Gun Med J 2002;25:405–410.
3. Zenge JP, Fenton L, Lovell MA, et al. Case report: infantile
hemangioendothelioma.
Curr Opin Pediatr 2002;14:99–102.
4. Ayling RM, Davenport M, Hadzic N, et al. Hepatic hemangioendothelioma
associated with production of humoral thyrotropin-like factor.
J Pediatr 2001;138:932–935.
5. Davenport M, Hansen L, Heaton ND, et al. Hemangioendothelioma of the liver
in infants.
J Pediatr Surg 1995;30:44–48.
6. d'Annibale, Piovanello P, Carlini P, et al. Epithelioid hemangioendothelioma of
the liver: case report and review of the literature.
Transplant Proc 2002;34:1248–1251.
Pictures
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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Copyright Details: Pediatric Complaints and Diagnostic Dilemmas, Copyright © 2008 Williams & Wilkins.
More About Causes of Weight gain
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