Abdominal Pain - Case 7-2: 5-Year-Old Girl
I. History of Present Illness
A 5-year-old girl was well until 2 days before presentation, when she developed
emesis and fever. On the day of presentation, she had two bouts of nonbloody,
nonbilious emesis and continued to have fever as high as 39.4
°C. The patient pointed to the periumbilical area when describing her pain. Her
parents also reported that she had had ear pain and a sore throat for the past
3 days. They denied cough, dysuria, and frequency. She had had a good appetite
and no weight loss. The parents reported that about 6 months ago the patient
had an episode of abdominal pain. Her primary care physician reportedly felt
stool in the abdomen and started her on prune juice. which she had stopped
using regularly.
II. Past Medical History
Birth history was normal, with no complications at delivery or birth. She had
had mild asthma but no hospitalizations. Three years earlier, she was exposed
to tuberculosis and had a positive tuberculin skin test. She was treated with
isoniazid for 9 months.
III. Physical Examination
T, 39°C; RR, 24/min; HR, 119 bpm; BP, 106/65 mm Hg
Weight, 22.9 kg (70th percentile); height, 120 cm (70th percentile)
Physical examination revealed an alert, well-nourished, and interactive child.
There was no conjunctival pallor. The tonsils were 2+ bilaterally, with mild
erythema of the posterior pharynx. Shotty cervical lymphadenopathy was present,
with enlarged superior cervical lymph nodes that were mobile and nontender. The
lungs were clear, and there was a I/IV systolic ejection murmur at the left
lower sternal border. The abdominal examination revealed normal bowel sounds.
On palpation, the abdomen was nontender, but a firm mass was felt in the
periumbilical region and left upper quadrant. The mass had sharp borders, was
approximately 6
× 4 cm, and was slightly mobile. Rectal examination revealed good rectal tone. The
rectal vault was full of stool, which was negative for occult blood. She was a
Tanner I female with no inguinal lymphadenopathy. Her neurologic examination
was normal.
IV. Diagnostic Studies
Laboratory analysis revealed 11,500 WBCs/mm3, with 2% band forms, 62% segmented neutrophils, 24% lymphocytes, and 9%
monocytes. The hemoglobin was 14.3 g/dL, and the platelet count was 251,000/mm
3. Electrolytes, blood urea nitrogen, creatinine, calcium, magnesium, and
phosphorus were normal. Liver function tests were normal. The uric acid
concentration was 5.3 mg/dL, and the lactate dehydrogenase concentration was
747 U/L. The abdominal radiograph revealed a large amount of stool.
V. Course of Illness
A Fleet enema was given with good results, but the mass was still palpable.
Abdominal MRI suggested a diagnostic category (Fig. 7-2). Biopsy of the mass
confirmed the diagnosis.
Discussion: Case 7-2
I. Differential Diagnosis
The finding of an abdominal mass in a child is important and that can be
attributed to causes as varied as bladder distention and life-threatening
malignancies. The age of the patient, history, physical examination, and
specific laboratory and imaging studies are crucial to arriving at the correct
diagnosis. Children younger than 5 years of age are the group in which
abdominal masses are most commonly identified, and about 60% of abdominal
masses that are identified in childhood by physical examination are secondary
to organomegaly.
In neonates, retroperitoneal masses that arise from the genitourinary system are
the most common type; causes include hydronephrosis, multicystic/polycystic
kidneys, mesoblastic nephroma, and renal vein thrombosis, which is seen in
infants of diabetic mothers and in those with severe hydration. Other possible
causes include pelvic masses such as an ovarian cyst or hydrometrocolpos, which
manifests with a suprapubic mass and vomiting as a result of hydronephrosis
from obstruction of the ureters. GI causes include intestinal duplication,
malrotation, and sacrococcygeal teratoma. Bladder distention, often as a
consequence of posterior urethral valves, can also be common in the neonatal
period. Hydronephrosis and multicystic/polycystic disease account for as many
as 75% of the abdominal masses in neonates.
In infants, the most common malignant solid tumor is neuroblastoma. In
childhood, Wilms tumor is the most common childhood abdominal malignancy. The
classic presentation is that of an asymptomatic child with an abdominal mass
noted by a parent while bathing the child. More than half of Wilms tumors
manifest before 5 years of age. Neuroblastomas that arise in the abdomen often
cross the midline, and more than half are seen within the first 2 years of
life. The tumor can produce catecholamines and therefore can be clinically
associated with tachycardia, hypertension, and skin flushing. The variability
of the site of the primary tumor makes the clinical presentation variable, but
constitutional symptoms such as fever and weight loss often occur. Other
retroperitoneal masses seen in infants in children include rhabdomyosarcoma,
lymphoma, Ewing
's sarcoma, and germ cell neoplasm. There are several liver lesions that can
cause abdominal masses in this age group, including benign solid tumors,
malignant tumors, vascular lesions, and cystic hepatobiliary disease. In
addition, lesions of the stomach (carcinoma, leiomyosarcoma, fibrosarcoma),
small bowel (duplication, Meckel diverticulum, lymphoma), colon (fecal mass is
also common), and omentum can cause abdominal masses in this age group.
Many of the causes of abdominal masses in infants and children also apply to
adolescents, but there are some diagnoses that are more common (pelvic masses
in particular). Hematocolpos may not be clinically evident until menarche.
Ovarian cystic lesions are common, and more than 85% are benign, with teratomas
as the most common lesion of this type. Malignant ovarian lesions include germ
cell tumors, dysgerminomas, choriocarcinomas, and gonadoblastomas. In the
retroperitoneal region, renal cell carcinoma occurs most commonly at 14 years
of age and manifests with flank pain and hematuria.
Physical examination is the most important aspect of early detection of
abdominal masses in children. A recent study showed that the majority of
malignant abdominal masses in children could be palpated on initial
examination.
II. Diagnosis
MRI of the abdomen (Fig. 7-2) revealed a 6 × 4.5 cm, multiloculated mass arising from the left adrenal gland. There were no
other retroperitoneal masses. During recovery, the biopsy of the tumor revealed
ganglioneuroma. A metaiodobenzylguanidine (MIBG) scan was negative, confirming
that the tumor was entirely a ganglioneuroma. A chemotherapy protocol was begun
to reduce the size of the mass before resection.
The diagnosis is adrenal ganglioneuroma.
III. Incidence and Epidemiology
There is a spectrum of neuroblastoma tumors that includes ganglioneuromas,
neuroblastomas, and ganglioneuroblastomas that arise from neural crest cells.
Unlike neuroblastomas, ganglioneuromas are benign and differentiated tumors.
Although the incidence of ganglioneuromas is not known, they are most common in
children and young adults. They usually are found in the posterior mediastinum
and retroperitoneum, and most often arise from the adrenal medulla. Much like
pheochromocytomas, which are tumors of chromaffin cells of the adrenal medulla
and adrenergic ganglia, adrenal ganglioneuromas can secrete epinephrine and
norepinephrine, giving rise to endocrinologic symptoms. There have been reports
of malignant transformation of ganglioneuromas to neuroblastoma and of mixed
tumors with pheochromocytoma.
IV. Clinical Presentation
Apart from the mass effect of any abdominal mass, ganglioneuromas can secrete
catecholamines and can cause the paraneoplastic syndrome seen with
pheochromocytomas. Hypertension is the most concerning sign, and symptoms can
include perspiration, tremor, nausea, vomiting, diarrhea, and other
manifestations of Cushing syndrome. Because ganglioneuromas can be associated
with neurofibromatosis type I or with von Recklinghausen
's disease, clinical manifestations of this disease (e.g., axillary freckling,
caf
é-au-lait spots) may be noted.
V. Diagnostic Approach
Evaluation of an adrenal mass should include studies to detect ganglioneuroma,
pheochromocytoma, and neuroblastoma.
Abdominal imaging. The initial study is usually an abdominal radiograph to exclude GI obstruction.
Some clinicians next obtain an ultrasound study to determine the organ or
origin and identify cysts, hemorrhage, and calcification. Compute tomography
(CT) or MRI may be used in lieu of ultrasound. The adrenal gland can be clearly
visualized by CT in 95% of patients. In distinguishing pheochromocytoma from
other adrenal masses, MRI has a positive predictive value of 83% and a negative
predictive value approaching 100%. Additional imaging of the head, spine, or
chest may be indicated to exclude metastatic disease.
Complete blood count (CBC). Pancytopenia indicates bone marrow involvement due to malignancy. Isolated
anemia suggests either chronic illness or hemorrhage into the mass.
Electrolytes, calcium, phosphorus, uric acid, and lactate dehydrogenase. Abnormalities in these studies are seen with tumor lysis syndrome.
Urine homovanillic acid (HVA) and vanillymandelic acid (VMA). In a patient with an adrenal mass, a spot-check of the urine for HVA and VMA
should be obtained to detect neuroblastoma or pheochromocytoma.
Other studies. Plasma concentrations of normetanephrines or metanephrines above the upper
reference limits (4 and 2.5 times normal, respectively) indicate a
pheochromocytoma with 100% specificity.
VI. Treatment
Treatment of an abdominal ganglioneuroma depends on the patient's clinical manifestations. In general, resection is curative. If the patient has
endocrinologic manifestations, they should be stabilized medically before
resection.
VII. References
1. Tunnessen WW. Abdominal masses. In: Tunnessen WW, ed. Signs and symptoms in pediatrics, 3rd ed. Philadelphia: Lippincott Williams & Wilkins, 1999:484–488.
2. Squires RH. Abdominal masses. In: Walker WA, Durie PR, Hamilton JR, et al.,
eds.
Pediatric gastrointestinal disease, 3rd ed. Hamilton, Ontario: BC Decker, 2000:150–163.
3. Golden CB, Feusner JH. Malignant abdominal masses in children: quick guide
to evaluation and diagnosis.
Pediatr Clin North Am 2002;49:1369–1392.
4. Stratakis CA, Chrousos GP. Endocrine tumors. In: Pizzo PA, Poplack DG, eds. Principles and practice of pediatric oncology, 3rd ed. Philadelphia: Lippincott–Raven Publishers, 1997:947–976.
5. Pacak K, Linehan WM, Eisenhoffer G, et al. Recent advances in genetics,
diagnosis, localization, and treatment of pheochromocytoma.
Ann Intern Med 2001;134:315–329.
6. Celik V, Unal G, Ozgultekin R, et al. Adrenal ganglioneuroma. Br J Surg 1996;83:263.
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 Lower abdominal pain
» Next page: Abdominal Pain - Case 7-3: 11-Year-Old Girl (Pediatric Complaints and Diagnostic Dilemmas)
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