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Moderator:
JOE RUTLEDGE
Children's Hospital
Seattle, WA
Panelists:
CYNTHIA KAPLAN, State University of New York, Stony Brook, NY
STEPHEN QUALMAN, Children's Hospital, Columbus, OH
LAURA FINN, Children's Hospital and Medical Center, Seattle, WA
MIGUEL REYES, Yale University School of Medicine, New Haven, CT
DAVID WALKER, University of Texas at Galveston, Galveston, TX
NOTE: Slides and protocols will also be available to participants for study in the microscope room (Superior Room) in advance of the evening session.
Clinical histories are printed below.
Click on the slide images for a larger view.
| Case 1 - | Stephen J. Qualman, M.D., Children's Hospital, Columbus, Ohio |
A nine-year-old girl who presented with a mass in the posterior cranial fossa underwent suboccipital
decompression and C1 laminectomy with tumor resection (Fig. 1). Rectal bleeding developed five-and-a-half
months after brain resection and chemotherapy,. Two colon biopsies (Fig. 2) were collected during
proctoscopy, which revealed a friable, granular and exudative mucosa. The clinical impression at this point
was one of possible pseudomembranous colitis. Stools were sent for bacterial and viral culture. The
patient was maintained on Flagyl therapy.
Nine years later, the patient presented again with rectal bleeding (hemoglobin decreased to 8 g/DL) with
history of intervening stroke and recurrent bleeding thought secondary to hemorrhoids. Upper endoscopy and
colonoscopy now revealed multiple nodules (Fig. 3 - gastric fundus and Figs. 4 & 5 -colon).
| Case 2 - | Miguel Reyes-Múgica, Yale University School of Medicine, New Haven, CT |
A 3 year-old African-American boy presented with limping due to a thigh mass of recent appearance. The
lesion was initially painless and non-mobile, without erythema or swelling. There was no fever or other
constitutional symptom. Four days before admission, a second tumor appeared on the left parietal region.
The leg mass became erythematous and painful, and fever of 39.4°C ensued. The relevant past medical history
includes three soft tissue nodules removed from the buttock on the third day of life, one of which is shown.
- the patient's father had an identical soft tissue lesion surgically removed as a newborn (Figs. 1 & 2).
In addition, there were frequent and recurrent infections including meningitis and pneumonias, and failure
to thrive. At two years of age the patient developed an intra-abdominal lymphangioma which showed
histological evidence of infection with multiple granulomata. At the time of admission the patient was at
the 5th percentile for height and below the 3rd percentile for weight. A CT scan revealed a large soft
tissue lesion in the scalp, eroding the parietal bone, and several small lesions involving the brain
parenchyma. In addition, a large mass was found involving the posterior aspect of the right thorax. These
findings were interpreted as suggestive of metastatic disease, possibly Ewing sarcoma or neuroblastoma.
Pertinent lab results include normal levels of immunoglobulins and negative HIV testing. The leg and
parietal lesions were surgically removed and drained. A representative sample from the leg is shown on
Figures 3 & 4.
| Case 3 - | David H. Walker, University of Texas, Galveston, TX |
This previously healthy, developmentally normal, 7 month old male child from south Texas had a febrile
illness with coryza three weeks before admission and was treated with antibiotics for otitis media 10 days
before admission. He was admitted to the intensive care unit with cough, pulse 146/min, respirations
50/min, temperature 97.4°F, clear lungs, and hepatosplenomegaly. Admission laboratory data included WBC
22,200/ul, hemoglobin 9.2 g/dl, serum sodium 125 mMol/l, and pO2 76 mmHg. Chest radiographs showed
cardiomegaly and increased pulmonary vascular markings, ECHO showed pericardial effusion and decreased
myocardial contractility, and EKG showed accelerated junctional rhythm.
Treatment included multiple antibiotics, pericardiocentesis, and creation of a pericardial window. The
hospital course was characterized by progressive cardiac failure and death. Figures are myocardium and
figure 4 is a Giemsa stain.
| Case 4 - | Laura Finn, University of Washington and Children=s Hospital and Regional Medical Center, Seattle, WA |
This AGA female infant (XX karyotype) was delivered vaginally to a 19-year-old G1P1 mother at 34 weeks
gestation with Apgars of 71 and 95. The placenta was large and the infant was noted to have widely spaced
cranial sutures and large anterior and posterior fontanelles. She was discharged in good condition on day
12 but presented at 1 month of age with abdominal distension and respiratory distress. Peritoneal fluid and
blood cultures grew Streptococcus pneumoniae. Evaluation revealed hypoalbunemia and nephrotic range
proteinuria (3.37grams/24hr); complement levels were normal; BUN and Cr were 3mg/dl and 0.3mg/dl,
respectively. The kidney size by ultrasound was the upper limit of normal and there was diffuse bilateral
echogenicity. Multiple bouts of sepsis and peritonitis ensued. She was unresponsive to medical management
and underwent a nephrectomy at 4 months and 7.5 months of age.
| Case 5 - | Cynthia Kaplan, SUNY-Stony Brook, Stony Brook, NY |
These twins were born at 26 weeks gestation to a 20 year old 0+ woman with preterm labor. The placenta was
diamniotic monochorionic with a large artery-to-artery anastomosis. Cords were labeled with 1 and 2 clamps
and both inserted eccentrically into their respective portions of the disk. There was substantial
disruption of the maternal surfaces of both, with approximate weights of the portions referable to "1" and
"2" as 160 and 120 grams respectively.
Baby A weighed 936 grams with initial hematocrit of 52%. In addition to respiratory distress, the infant
had thrombocytopenia the first few days of life. A grade II intraventricular hemorrhage had largely
resolved by discharge at 2 months of age. Baby B was 894 grams with initial hematocrit of 44%. In addition
to respiratory distress, neutropenia was present the first few days of life. A grade II intraventricular
hemorrhage had partially resolved by discharge at 2 months. Figures 1 and 2 are from the placenta of Baby A
and Figures 3 and 4 from that of Baby B. Figure 5 is dividing membrane.
For answers, text and references for all cases, please click here.
Opinions stated and/or conclusions reached in this syllabus are the
responsibility
of the authors and are not necessarily endorsed by the
Academy.
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