Clinical Vignette Poster Session II
Friday, November 13, 2009
12:15pm – 2:15pm
cv 25 IS THERE A ROLE FOR UPPER ENDOSCOPY IN THE EVALUATION OF FAILURE TO THRIVE?
Stuart Berezin, Michael S. Halata, Howard E. Bostwick. Pediatrics, NY Medical College, Valhalla, NY
Fifteen patients with poor weight gain, 5 months to 2 years of age, were evaluated for failure to thrive
due to inadequate caloric intake based on dietary history. All patients had weight for length less than 5%
and experienced a significant decrease in weight percentile on the growth curve. None had
gastrointestinal symptoms of vomiting, diarrhea, constipation or dysphagia. Evaluation included CBC,
total protein, albumin, ALT, AST, BUN, creatinine, T4, TSH and electrolytes. Patients over 10 months had
lab testing for celiac disease. Abdominal ultrasound was performed in eight patients and upper
gastrointestinal series in four patients. Evaluations were normal for all patients, except for two with
positive celiac disease serology.
All patients had an upper endoscopy with biopsies of the duodenum, stomach and esophagus. Seven of
fifteen patients were under one year of age. Two had biopsy‐confirmed eosinophilic esophagitis (> 100
eosinophils/high power field (HPF)). Two patients had normal appearing endoscopies, but had
histological evidence of reflux esophagitis (5‐7 eosinophils/HPF). One patient had a duodenal bulbar
ulcer. Two patients had normal endoscopies.
Eight of fifteen were over 1 year of age and also had upper endoscopies. One had eosinophilic
esophagitis (> 30 eosinophils/HPF). This patient had previously been treated with omeprazole for 2
months. Two patients had biopsy‐proven celiac disease that confirmed their abnormal celiac serology.
Five patients had normal endoscopies.
In this young population of failure to thrive patients, upper endoscopic examinations with biopsies were
useful in obtaining a diagnosis in more than 50% of those examined. Upper endoscopy is therefore a
useful evaluation in children under 2 years of age with failure to thrive due to inadequate caloric intake
and who do not exhibit overt gastrointestinal symptoms. In children under 1 year of age eosinophilic
and reflux esophagitis were the most common diagnosis; in children over one year of age, celiac disease
was the most common diagnosis in this patient population.
cv 26 ENDOSCOPIC REMOVAL OF A LARGE GASTRIC TRICHOBEZOAR IN A PEDIATRIC PATIENT
Ahmet Aybar, Anca M. Safta. Pediatrics, University of Maryland, Baltimore, MD
A trichobezoar is an accumulation of swallowed hair in the stomach that fails to pass through the
intestines. The incidence is greater among the mentally retarded or emotionally disturbed children.
Endoscopic removal is usually not successful and can cause severe complications. Surgical or
laparoscopic removal is the preferred method. Many endoscopic techniques have been described for
breaking up the trichobezoar. We report a young patient with a large trichobezoar which was removed
using hot biopsy forceps and electrocautery snare.
A 5 ½ year‐old girl presented to the Pediatric ED with symptoms of small bowel obstruction. Exploratory
laparotomy revealed a trichobezoar in the ileum and was removed with resection of the distal ileum.
She had persistent nausea and vomiting with poor appetite for 6 weeks. A follow up CT of the abdomen
and UGI study confirmed a large residual trichobezoar in the stomach extending through duodenal bulb.
Endoscopic removal was attempted to avoid a second operation. Initially, the retrieval of the intact
bezoar through the lower esophageal sphincter failed. Subsequently the bezoar was broken into 13
smaller pieces using hot biopsy forceps and snare via ERBE™ and completely removed. After the
procedure, mucosal abrasions and small superficial mucosal burns noted in the greater curvature and
treated with a course of lansoprazole and sucralfate. She was symptom‐free at follow up.
Trichobezoar is a rare condition in which swallowed hair accumulates in the stomach. It is indigestible
and slippery in character, can not be propulsed distally and may cause small bowel obstruction.
Surgery is recommended for the removal of large bezoars. Endoscopic removal is very seldom successful
due to a distal tail extending into small bowel or imbedding of hair in the gastric mucosa. We describe a
safe and successful endoscopic removal of a large gastric trichobezoar without immediate
complications. To our knowledge this is the first published report in the English literature removing a
large trichobezoar endoscopically by cutting into small pieces using electrocautery.
cv 27 RELATIONSHIP BETWEEN BMI AND REFLUX ESOPHAGITIS(GERD) IN CHILDREN Radha Nathan,
Nidhi Rawal. Pediatrics, Brookdale University Hospital and Medical Center, Brooklyn, NY
BACKGROUND: Obesity is a rising public health concern, because of various co‐morbidities. Studies in
adults have shown a positive correlation between obesity and GERD. However, data in children have
shown conflicting results.
AIM: To evaluate the relationship between BMI and GERD in children.
METHODS: In a retrospective case control study, we identified patients between ages 3‐18 years, from
Jan’06 to Feb’08, with histological evidence of GERD. Charts were reviewed for age, sex, weight, height
and symptoms. BMI was calculated and BMI percentiles were obtained using CDC growth charts. We
defined overweight as BMI between the 85th and 95th percentiles and obese as >or equal to the 95th
percentile. Exclusion criteria included patients with neurological conditions, IBD, prematurity and
age<3years. Pediatric patients seen in the Well Child Clinic with no history of reflux symptoms, served as
controls. Cases and controls were age and gender matched. Information was obtained for 47 matched
pairs. Demographic data were collected. Analysis was performed with STATA software using logistic
regression and t test.
RESULTS: About 48.9% of cases (GERD +) were overweight/obese compared to 25.7% of controls (GERD
‐). The percentage of overweight and obese children in the control group corresponded closely to the
population norm as per the CDC data. When regressed, GERD correlated well with BMI percentiles
(OR=1.97, P=0.008) and female gender (OR=2.3, P value=0.026). No statistically significant relationship
was found between GERD and age/simple BMI value.
CONCLUSION: We report that children with BMI>85%ile have twice the risk of having GERD, as
compared to the normal weight population. Prevalence is significantly higher in females.
Variables Cases(47)GERD+ Controls(47)GERD‐
Weight(kg) 15.3‐127.8 10.3‐110
BMI(kg/m ) 12.6‐48 Mean=24.1±8.06 14‐37.9 Mean=22.04±6.3
BMIpercentiles <85th%ile 85‐95th%ile ‐ 24(51.06%) 8(17.02%)P=0.008 ‐ 35(74.4%) 6(12.7%)
>95th%ile 15(31.9%) 6(12.7%)
cv 28 ARGON PLASMA COAGULATION FOR TREATMENT OF RADIATION‐INDUCED HEMORRHAGIC
GASTRITIS KATTAYOUN KORDY, MD, BRADLEY BARTH, MD, MPH UNIVERSITY OF TEXAS
SOUTHWESTERN MEDICAL CENTER AT DALLAS, CHILDREN’S MEDICAL CENTER
Kattayoun Kordy, Bradley Barth. University of Texas Southwestern Medical Center at Dallas, Children's
Medical Center Dallas, Dallas, TX
Argon plasma coagulation (APC) is a non‐contact, through the scope, monopolar, electrocoagulation
technique that has potential benefit in children requiring endoscopic therapy for GI bleeding. It is
especially well suited for patients who are at high risk for perforation or with diffuse lesions, including
those undergoing chemotherapy. Case Report: We describe a 4 year old boy with history of biliary
rhabdomyoscarcoma who recently completed a 5 week course of radiation therapy. He presented with
transfusion dependent upper GI bleeding secondary to severe radiation‐induced hemorrhagic gastritis.
Several multimodal endoscopic therapies failed to control his chronic GI bleeding, including epinephrine
injections and endoscopic clip placement. Due to the diffuse nature of the lesion, APC was applied to
80%‐90% of circumferential bleeding in the antrum with successful achievement of hemostasis. One
week follow‐up endoscopy revealed improved gastritis limited to the antrum with some areas of oozing
that was again treated with APC. Subsequent follow‐up endoscopy revealed complete resolution of GI
bleeding without evidence of ulceration, and a significant drop in transfusion requirement post‐APC
treatment. Discussion: To our knowledge, this is the first reported case of APC in the management of GI
bleeding secondary to radiation induced hemorrhagic gastritis in a pediatric patient. Data is limited on
APC in children with yet unknown long term effects. However, the non‐contact nature of this therapy,
along with minimal depth of penetration make it ideally suited for this indication.
1. Kahn K, Scharzenberg S, Sharp H, Weisdorf‐Schindele S. Argon plasma coagulation: Clinical experience
in pediatric patients. Gastrointestinal Endoscopy 2003; 57 (1): 110‐112.
2. Watson J, Bennett M, Griffin S, Matthewson K. The tissue effect of argon plasma coagulation on
esophageal and gastric mucosa. Gastrointestinal Endoscopy 2000; 52(3):342‐5.
cv 29 GASTRIC ADENOCARCINOMA IN A 14 YEAR‐OLD WITH HISTORY OF GIARDIA AND CMV
INFECTIONS. Jeffrey H. Ho, Marvin E. Ament. Division of Pediatric GI, Hepatology and Nutrition, UCLA,
Los Angeles, CA
We describe a case of a 14 year old male with history of chronic abdominal pain and intermittent
vomiting since two years of age. Family history is remarkable for members with gastric adenocarcinoma.
At age two, patient began vomiting and was reported to have hypoalbuminemia and protein losing
enteropathy. Endoscopy was normal but stool studies showed presence of Giardia. At age four, he
presented with persistent vomiting. Endoscopy grossly showed antral ulcers. Biopsies showed severe
gastritis, intestinal metaplasia and CMV intranuclear inclusions. Immunodeficiency work‐up showed
reversal of CD4 and CD8 lymphocytes. He was then treated with Ganciclovir with resolution of
At age 14, he was evaluated for chronic abdominal pain. Endoscopy showed a friable and necrotic
appearing gastric mass arising from mid‐body of the greater curvature. Biopsies showed high grade
dysplasia. Patient underwent endoscopic ultrasound and the tumor appeared to penetrate through
certain areas of muscularis propria. Patient then underwent subtotal gastrectomy with resection of
gastric tumor, regional D2 lymphadenectomy and Roux‐en‐Y gastrojejunostomy. Frozen section analysis
showed clear margins and benign reactive lymph nodes. Pathology revealed a 6.2 cm tubular/intestinal
type gastric adenocarcinoma that was moderately differentiated. Tumor invaded into superficial
submucosa and 76 resected lymph notes were negative for metastatic carcinoma. Tumor was classified
as a Stage IA malignancy or T1N0M0. Interestingly, EBV EBER was positive in dysplasia but negative in
adjacent normal mucosa.
There have been limited studies on standardized treatment protocols for pediatric gastric
adenocarcinoma, therefore approach was made to treat our patient as an adult. Patient received
adjuvant chemotherapy with 11 courses of 5‐FU over six months. At one year post‐surgery, surveillance
endoscopies with biopsies and imaging showed no recurrence of tumor or any evidence of metastatic
cv 30 GASTROCOLIC FISTULA AFTER PERCUTANEOUS ENDOSCOPIC GASTROSTOMY (PEG)
K. Nguyen1, P. De Angelis2, N. Gupta3, A. S. Day3, J. Teitelbaum4, J. Dias5, A. Bautista6, C. Wilson7, R. Gill1,
J. Xu1, S. Schwarz1, W. Treem1. 1SUNY Downstate Medical Center, Brooklyn, NY; 2Ospedale Pediatrico
Bambino Gesù, Rome, Italy; 3Sydney Children's Hospital, Randwick, NSW, Australia; 4Children's Hospital
at Monmouth Medical Center, Long Branch, NJ; 5Hospital de S. João, Porto, Portugal; 6University Hospital
Santiago de Compostela, Galicia, Spain; 7Lucille Packard Children's Hospital, Palo Alto, CA
Introduction:Gastrocolic fistulas (GCF) are a known complication of PEG placement in children but have
only been described in case reports.Aims:We determined signs/symptoms, predisposing factors, natural
history, and management strategies for GCF after PEG in a series of children.Methods:A questionnaire
was designed and distributed to physicians who cared for pts with GCF after PEG.Each physician
performed a retrospective chart review. No specific pt identifiers were provided.Consent waiver was
obtained from SUNY Downstate IRB.Results:12 pts were included.7 had cerebral palsy, 3 of whom had
scoliosis.7 had previous abdominal surgery.The number of PEGs/year by each physician ranged from < 5
and up to 40.Finger indentation/stomach insufflation alone was used in 11 cases.In 1 case, fluoroscopy
was also used.The time from PEG to diagnosis of GCF ranged from <1 wk to >6 mo.The most common
symptoms of GCF were feculent vomiting, diarrhea, and weight loss.Diagnosis was made by contrast
study in 10, by EGD in 1, and at surgery in 1. GCF spontaneously closed in 4, was surgically closed in
6,and was endoscopically clipped in 1 pt.3 pts had repeat PEG and 7 had G‐tube replacement by
surgery.Modifications to PEG after GCF included adoption of safe track technique,fluoroscopy
w/transverse colon opacification,reduced stomach insufflation,or increased referrals to interventional
radiology.Conclusions:GCF can present in the immediate post‐procedure period and as long as 10
months after PEG.Previous abdominal surgery is a key risk factor for GCF.Surgical closure or
spontaneous closure was used in the majority of cases.The varied number of PEGs/year suggests that
increased experience with PEG does not necessarily mitigate the risk for the development of GCF.
cv 31 COIN RETRIEVAL AND EOSINOPHILIC ESOPHAGITIS
Nicole Jordan1, Aeri Moon2. 1Pediatric Gastroenterology, NYPH Weill Cornell Medical Center, NY, NY;
Pediatric Gastroenterology, NYPH Weill Medical College of Cornell University, NY, NY
Coins are the most commonly ingested foreign body in children, and spontaneous passage occurs in up
to a third. Of those lodged in the esophagus, approximately 10‐20% lodge in the mid‐esophagus.
Retained esophageal coins occur most often in those who are small or with underlying esophageal
pathology. Eosinophilic esophagitis (EE) is a chronic inflammatory condition of the esophagus. Although
esophageal food impaction is a fairly common presentation of EE in adolescents, there are no published
case reports on non‐food‐related foreign body impactions in children as an initial clinical manifestation
of EE. This is the first case report of EE diagnosed in a child who underwent an
esophagogastroduodenoscopy (EGD) for retrieval of a coin. Case: A four year‐old healthy male
presented to the emergency department after having swallowed a coin the night prior. He was
asymptomatic and a chest x‐ray showed a radiopaque foreign body within the esophagus. He underwent
an EGD in which a coin was found in the mid‐esophagus. Gross endoscopic inspection noted linear
exudates of the esophagus. Three‐level esophageal biopsies were taken and histologically reported as
active esophagitis with eosinophilic infiltrates. The patient was placed on Prevacid. A repeat EGD was
performed 3 months later which confirmed the diagnosis of EE whereby a swallowed topical steroid was
added. Conclusion: In young children, EE is reported to present most often as dysphagia, pain and
emesis, and in adolescents, as food impaction requiring endoscopic removal. This is not the first case in
our institution where an EGD was performed on a child for coin retrieval in which the child was
subsequently diagnosed with EE. This case illustrates two points: 1) presentation of a non‐food‐related
foreign body impaction may be the first manifestation of EE in an otherwise normal child without
symptoms of dysphagia or food impaction; therefore 2) it should be considered that all children
undergoing an EGD for foreign body retrieval have biopsies taken when the foreign body is impacted in
cv 32 RAPUNZEL SYNDROME: NOT JUST A FAIRY TALE
Ryan K. Brislin1, Samantha Cook1, Bonnie Beaver2, Yoram Elitsur1. 1Pediatrics, Marshall, Huntington, WV;
Surgery, Marshall, Huntington, WV
Rampion (campanula rapunculus) is a pot‐erb plan, characterized by its long stems and white hair. The
plan was the main subject of the Grimms' brothers famous fairy tale, Rapunzel. Rapunzel syndrome was
later described as a gastric trichobezoar that extended into the small intestine. Rapunzel syndrome is
rarely described in the USA.
Case report: A 12 year old female presented with intermittent and sharp abdominal pain with emesis,
decreased appetite, early satiety, and weight loss. The patient reported history of trichotillomania, but
denies hair swallowing.
Physical examination : A diffuse abdominal pain with hyperactive bowel sounds. A mobile firm mass
(approx. 15cm x 8cm) in the epigastric region was palpated. Abdominal CT scan showed a gastric bezoar
that extended as far as the proximal jejunum and intestinal edema. Initial laboratory investigation
showed normal CBC and electrolytes. A prliminary diagnosis of trichobezoar and partial bowel
obstruction was made. An upper endoscopy documented the presence of a gastric trichobezoar
occupying the entire gastric cavity. The hair was visualized exiting the pylorus and extending beyond the
duodenal bulb, suggesting the diagnosis of Rapunzel syndrome. Surgical laparotomy revealed a firm
trichobezoar occupying the entire gastric cavity. After removing the gastric portion, the distal portion of
the bezoar was located 70 cm beyond the ligament of Treitz, well into the jejunum. A longitudinal
incision was made and the removal of the remaining portion of the trichobezoar was performed. As a
result of hair entrapment, a partial intestinal obstruction was present and was relieved. The patient
tolerated the procedure uneventfully and was discharged at day 6. Surgical follow up was uneventful.
The patient was referred for psychiatric evaluation. After few therapeutic sessions the patient was
discharged in excellent condition. At 1.5 year post surgery, the patient is asymptomatic and his
trichotillomania is under control.
Conclusion : Rapunzel syndrome is a rare complication of trichotillomania that requires surgical therapy.
cv 33 GASTRIC POLYPS IN MENKES DISEASE ASSOCIATED WITH GASTROINTESTINAL BLEEDING AND
Melissa Kennedy, Rose Graham. Gastroenterology, Hepatology, and Nutrition, Childrens Hospital of
Philadelphia, Philadelphia, PA
A 19 month old male with Menkes Disease was admitted to the intensive care unit with
metapneumovirus bronchiolitis and pnueomococcal bacteremia. Admission history and laboratory
studies revealed severe anemia and a recent history of melena and intermittent hematemesis. The
patient was treated with intravenous pantoprazole, antibiotic therapy, and supportive respiratory care.
Endoscopy was postponed pending improvement in respiratory status as bleeding was manageable with
supportive therapy. One week after admission, the patient developed acute hematemesis. Upper
endoscopy revealed a large polypoid mass with numerous large fronds obstructing the pyloric outlet. As
this could not be managed endoscopically, the procedure was converted to a surgical case, when the
lesion was noted to be emanating from the gastric antrum. Antrectomy with a Bilroth Type 1
reconstruction was performed to surgically remove the mass. Microscopic pathology revealed
hyperplastic gastric foveolar epithelium with edema, inflammation and areas of ulceration. Menkes
Disease is an X‐linked recessive disorder of impaired copper membrane transport secondary to
mutations in the ATP7A gene. Depleted serum copper levels lead to copper dependent enzyme
deficiencies causing most of the clinical features of Menkes Disease. Deficiency in lysyl oxidase, a copper
dependent enzyme required in the first step of collagen cross linkage, results in connective tissue
fragility and predisposition towards mucosal redundancy including polypoid masses in the
gastrointestinal tract. Hypertrophic polyp formation is more common at the pyloric outlet which is
exposed to persistent localized pressure during peristalsis. These lesions are more prone to bleeding
secondary to the underlying vascular abnormalities associated with Menkes Disease. Gastric polyps in
Menkes Disease have been reported infrequently in the literature but are likely an underappreciated
clinical feature of Menkes and may lead to gastrointestinal bleeding or obstruction.
cv 34 17‐YEAR‐OLD IMMUNOCOMPETENT MALE WITH HERPES SIMPLEX VIRUS ESOPHAGITIS;
COULD THIS BE PRECIPITATED BY ESOPHAGEAL EOSINOPHILIA?
M. Samer Ammar. Pediatric, SIU School of Medicine, Springfield, IL
Introduction: Herpes simplex virus (HSV) may cause esophagitis in immunocompromised patients and
seldom in immunocompetent ones. In immunocompetent patients, risk factors are yet to be identified.
In this patient, follow up histological examination of esophageal biopsies supported the diagnosis of
Case report: 17‐year‐old male with history of intermittent dysphagia for years, developed odynophagia
2‐3 days before he was evaluated by pediatric gastroenterologist. Mucosal irregularity was seen on the
contrast esophogram. Patient underwent an Esophagogastroduodenoscopy (EGD) with biopsies.
Grossly, esophageal mucosa appeared thickened with blunted vasculature and whitish, nonwashable
adherent materials. Fungal culture of esophageal brushing was negative. Distal esophageal biopsies had
extensive ulceration with focal squamous mucosa demonstrating viropathic changes. Proximal
esophageal biopsies demonstrated focal findings similar to those in the distal. The immuno stain for HSV
was positive in distal and proximal esophageal biopsies confirming the diagnosis of HSV related
esophagitis. GMS stain for fungal organisms was negative. Patient was then determined to be
immunocompetent. He was treated with proton pump inhibitor and acyclovir, intravenously initially,
followed by oral administration with improvement in symptoms. Patient underwent EGD with biopsies
about 3.5 weeks and 6 months from the first endoscopic evaluation. The diagnosis of eosinophilic
esophagitis was confirmed.
Conclusion: HSV may cause esophagitis in immunocompetent patients. Esophageal eosinophilia was the
only identifiable risk factor in this patient. Esophageal eosinophilia may be a risk factor for HSV
cv 35 CHRONIC COUGH CAN IT BE HELICOBACTER PYLORI
Vaibhav Goyal, Rima Jibaly. Pediatrics, Hurley Medical Center, Flint,, MI
Chronic cough is defined as cough present for more than 4 weeks.Asthma,allergic rhinitis,foreign body
ingestion and sinusitis are few of the common causes of chronic cough.Only one study looked at H.pylori
as a possible etiology of chronic cough in adult patients.No similar publication was identified in the
literature in the pediatrics age group.We are presenting a child with persistent cough who was found to
have H.Pylori gastritis. His symptoms resolved after he was treated for H. Pylori.
Case:13 year old child with a history of asthma came to the clinic complaining of cough since past three
month.It was affecting his sleep and daily activities.He had been in and out of the asthma clinic where
his medications were adjusted with no relief.PPI were added for possible reflux with no
improvement.He was referred to the ENT specialist where a direct laryngoscopy showed erythema of
larynx. GERD was suspected and he was referred to the GI clinic because of failure of reflux
treatment.His chief complaint included cough with no abdominal pain, dyspepsia or vomiting.His exam
revealed epigastric tenderness.GI endoscopy was done which showed: decreased vascularity of the
oesophagus but no lesions were present.Stomach was erythematous with red streaks especially in the
antrum. The esophageal biopsy was normal,but the Gastric antral biopsy revealed gastritis and
Helicobacter pyori organisms were identified.Patient was treated with amocixillin and clarithromycin for
2 wks and prevacid for 4 weeks. Cough subsided within next couple of days after starting the treatment.
Patient came for follow up one month after treatment and was asymptomatic.His last coughing was
before initiating the treatment.
Conclusion:Our case supports the possible association between H pylori and persistent cough.It was
suggested by Petar Rouev et al that it may be a result of the pro inflammatory nature of the bacteria.The
possibility of reflux as contributing to the cough can not be completely ruled out in this patient. We
suggest keeping H. pylori in the differential of patients with persistent cough after the common causes
have been ruled out.
cv 36 ANTRAL POLYPS IN A CHILD WITH HYPOPROTEINEMIA, ANEMIA AND ABDOMINAL PAIN
Yilda Alvarado, Iona Monteiro. Pediatrics, UMDNJ‐NJMS, Newark, NJ
12 year old male was initially seen in ‘02 with generalized swelling, abdominal pain and blood‐streaked
vomiting. On exam he had macrocephaly and generalized edema. Lab studies showed anemia and
hypoproteinemia. Esophagogastroduodenoscopy (EGD) showed few pedunculated antral polyps.
Pathology: inflammatory/hamartomatous polyp. Colonoscopy: no polyps. Small bowel series:
questionable filling defects. MRI of head was normal. He was lost to follow‐up till ‘09, when he came in
with abdominal pain, hypoproteinemia and anemia. EGD revealed marked increase in polyps. Pathology
revealed hyperplastic polyps. His pain decreased post polypectomy.
Hyperplastic polyps are the commonest polyps in the stomach occuring in either gender and common in
the 7th decade of life1. They are small solitary antral lesions, usually asymptomatic but can present with
dyspepsia, abdominal pain or gastrointestinal (GI) bleeding and anemia1. Pathogenesis is unknown, but
may occur with chronic gastritis ‐ autoimmune or H. pylori2. They can cause GI blood loss in older
patients3. Removal of the polyps using endoscopic or surgical methods is required for resolution of the
blood loss together with iron therapy3.
In children there are very few case reports of hyperplastic polyps causing hypoproteinemia, anemia4 or
gastric outlet obstruction5. We feel that this child's pain was likely secondary to obstruction at the
pylorus as it improved post polypectomy.
1. Gastric hyperplastic polyps. A Review. R Jain, P Chetty. Dig Dis Sci, Nov 27, 2008 (E pub)
2. Hyperplastic polyps of the stomach: Associations with histologic patterns of gastritis and gastric
atrophy. Abraham SC et al. Am J Surg Pathol, April 2001:25 (4) 500‐7
3. Hyperplastic polyps of the gastric antrum in patients with GI blood loss. M. Al‐Haddad et al. Dig Dis
Sci. Jan 2007:52 (1):105‐9
4. Juvenile polyposis of the stomach: clinicopathological features and its malignant potential. Hizawa K.
et al. J Clin Pathol Sep1997;50(9):771‐4
5. Prolapsed hyperplastic gastric polyp causing gastric outlet obstruction, hypergastrinemia, and
hematemesis in an infant. Brooks GS et al. J Pediatr Surg Dec 1992:27(12):1537‐8
cv 37 DELAYED SPONDYLODISCITIS AFTER BUTTON‐BATTERY INGESTION
Rupinder K. Gill, J. Amodio*, K. Nguyen, J. Xu, S. Schwarz, W. R. Treem. Pediatric Gastroenterology and
Radiology*, Children's Hospital at SUNY Downstate Medical Center, Brooklyn, NY
Ingestion of a button‐battery requires immediate endoscopic removal to avoid tissue necrosis,
perforation, TE fistula, or death. We report a case complicated by spondylodiscitis, an unusual
complication of button‐battery ingestion. Case report: A 14‐mo. old female presented with 4 wk history
of persistent cough and a normal physical exam. A CXR revealed a round battery in the mid‐esophagus.
The battery was removed by rigid endoscopy with only mild irritation of the esophagus reported. A
barium swallow (BS) performed two days later showed a focal area of dilatation in the thoracic
esophagus and a scalloped appearance of the posterior wall of the esophagus. There was no evidence of
perforation or fistualization. The patient began oral feeds and was discharged home. Six weeks later the
patient returned with torticollis but no fever or dysphagia. The CBC was normal with an elevated ESR
(76) and CRP (12). MRI of the neck showed spondylodiscitis with erosion of the inferior endplate of T1
and the superior endplate of T2 vertebral bodies; with abnormal contrast enhancement of the T1 and T2
intervertebral disc and prevertebral tissue; and with extension into the posterior wall of the esophagus
at the level of the previous button battery. Repeat BS showed a filling defect in the proximal esophagus
without evidence of contrast leak. An upper endoscopy revealed a smooth, movable, polypoid lesion.
The endoscope was easily passed beyond the lesion into the distal esophagus. Biopsy of the polyp
showed granulation tissue. The patient was treated with IV antibiotics for 6 weeks and the torticollis
resolved. Blood cultures obtained prior to IV antibiotics were negative and ESR and CRP returned to
normal with treatment. Conclusion: We present a case of button‐battery ingestion complicated by
spondylodiscitis 6 weeks later in the absence of esophageal necrosis, perforation, or fistualization at the
time of battery removal. The onset of torticollis weeks after removal of a button battery should prompt
imaging for this complication.
cv 38 RECURRENCE OF RAPUNZEL SYNDROME
Sheela Raikar1, Prateek Wali2, Seema Khan2,1. 1Pediatrics, Thomas Jefferson University, Philadelphia, PA;
Pediatric Gastroenterology, Alfred I. duPont Hospital for Children, Wilmington, DE
We report a case of a 12‐year‐old girl presenting with a three day history of severe, intermittent
abdominal pain and vomiting. The pain was localized to the epigastric area. It was worse with meals and
ambulation. She had one episode of diarrhea. Her history was negative for fever, back pain, dysuria or
dymenorrhea. She reported similar symptoms three months earlier that resolved spontaneously. She
had a history of depression. She had a history of prior trichobezoar and laparotomy at age four. On
physical exam, she appeared in discomfort, had short cropped hair and was wearing a wig. Her
abdominal exam was positive for generalized tenderness and guarding, otherwise unremarkable. CT of
the abdomen revealed a mass in the stomach extending through the pylorus, into the proximal small
bowel. An upper endoscopy revealed a large trichobezoar extending from the LES to the third part of the
duodenum. Multiple attempts at endoscopic removal failed. She underwent laparotomy with removal of
an extensive trichobezoar. She had an uncomplicated post‐operative course and was discharged home
with psychiatric follow‐up.
Trichobezoars result secondary to ingestion of hair with associated trichotillomania. Rapunzel syndrome
is a rare complication of trichobezoar formation resulting in a mass extending through the pylorus into
the small bowel. This mainly affects girls and has been linked to psychiatric conditions. The human body
is unable to digest hair, leading to a ball formation in the stomach. Complications include FTT, chronic
anemia, ulcer formation, perforation, pancreatitis, intussusception, and intestinal obstruction. Extent of
the bezoar may be determined by CT or MRI. Endoscopic removal is generally unsuccessful due to large
dimensions and firmness of the bezoar. Endoscopic fragmentation can lead to distal small bowel
obstruction. If a large bezoar is present (greater than 20cm), surgical removal is required. Open
laparotomy or laparoscopy remains the standard of treatment. After removal, psychiatric evaluation is
essential to prevent recurrence of trichobezoar.
cv38a DIEULAFOY LESION IN A CHILD
A.N. Nasir, C.M. Wilhelm, J.N. Udall, Pediatrics, WVUHSC‐Charleston, Charleston, WV;J.A. Levien,
Medicine, WVUHSC‐Charleston, Charleston, WV;W.P. Tomlison, Medicine/Pediatrics, WVUHSC‐
Charleston, Charleston, WV.
Dieulafoy lesions are caused by a large tortuous artery that erodes as it approximates the mucosa of the
GI tract leading to massive bleeding.The patient is a 7 year old boy who had a chronic fever and cough.
He was treated with no improvement. A chest x‐ray showed a right lung pneumonia and large pleural
effusion. The boy was hospitalized. The hemoglobin in gm% and hematocrit in % (H/H) were 11.3/32.3.
A right‐sided chest tube was placed. On hospital day 2 he vomited 45 ml of blood. His H/H dropped to
7.4/21.7.He was transfused 2 units of PRBCs. I.V. pantoprazole and p.o. sucralfate were started. He was
stable until day 8, when he had a 40 ml hematemesis. His H/H dropped from 10.3/29.5 to 8.5/25.3.
Three units of PRBCs were given. At upper endoscopy there were clots of blood in the stomach but no
active bleeding. Two ulcers approximately 3 cm apart in the duodenal bulb were noted. One had a white
escar base (healing) and the other was covered with clot (recent bleed). There was no active bleeding.
Following endoscopy octreotide and pantoprazole drips were started. A day later blood was aspirated
from his NG tube, and his hemoglobin dropped from 9.0 to 6.8. He was returned to the OR for upper
endoscopy. At endoscopy, a small clean based ulcer crater was found (healing) in the duodenal bulb. In
the duodenal sweep there was a moderate sized blood clot. The clot was removed and a large blood
vessel which had recently bled was noted. The area was injected with 2.5 ml of 1:10,000 epinephrine
and cauterized.The boy remained stable with no signs of active bleeding during the rest of his hospital
stay. However, he did require 4 more units of PRBCs. He had a third upper endoscopy just prior to
discharge on hospital day 21. There was no active bleeding and healing of both duodenal ulcers was
evident. Biopsies from the gastric antrum were negative for H. pylori, and a fasting serum gastrin level
was normal. The H/H at discharge was 12.6/36.8. In outpatient clinic 2 months later the H/H was
14.2/42.0. This case illustrates the success of endoscopy in the diagnosis and treatment of a duodenal
Dieulafoy lesion in a 7 year old boy.
cv 39 AN UNUSUAL CASE OF GASTROINTESTINAL BLEEDING
Kristin N. Fiorino1, Brian Lestini2, Asim Maqbool1. 1Gastroenterology, The Children's Hospital of
Philadelphia, Philadelphia, PA; 2Oncology, The Children's Hospital of Philadelphia, Philadelphia, PA
A previously healthy 10‐year‐old African American boy presented with a 3 day history of fever,
headaches, and periumbilical abdominal pain, with the development of nonbloody, nonbilious emesis
and melena 24 hours prior to presentation. No history of ingestion, trauma, travel, use of NSAIDs, or sick
contacts; family history was negative for inflammatory bowel disease, peptic ulcer disease, and
polyposis syndromes. Upon initial examination, HR 126, BP 116/56; he was afebrile and dehydrated. No
oral lesions, rashes, joint swelling/effusions, or perirectal lesions. Abdomen was soft, with epigastric
tenderness, no hepatosplenomegaly/masses. Stool was hemoccult positive. Laboratory studies included
hemoglobin 4 g/dL, MCV 80.1 fL, sedimentation rate 42 mm/hr, lipase 202 U/L, BUN 12 mg/dL,
creatinine 0.8 mg/dL. Infectious stool studies and abdominal x‐ray were negative. Within 8 hours, a
palpable fullness was felt in the epigastric and suprapubic regions which rapidly progressed to a
discrete, dense 10 cm firm mass. CT revealed a well‐defined, vascular 8 cm retroperitoneal mass near
the pancreas, compressing the duodenum with near complete effacement of the inferior vena cava,
with arterial branches from the gastroduodenal and superior mesenteric arteries and the infrarenal
aorta. Histopathologic evaluations of tissue suggested a neuroendocrine tumor, specifically
paraganglioma. Neo‐adjuvant chemotherapy was followed by surgical exploration and eventual
resection. A malignant ulcer eroded into the third part of the duodenum, the primary source of the
bleeding. Follow‐up imaging revealed no residual mass or MIBG‐avid disease. The patient remains
disease‐free two years post‐resection. Paragangliomas are rare tumors of neuroendocrine origin with
the potential for malignant progression. They may initially grow insidiously and lack biochemical
secretion, and therefore may present with nonspecific symptoms dependent upon the site of origin.
Abdominal paragangliomas are not typically associated with GI bleeding, unless invading the intestinal
cv 40 FURTHER EVIDENCE FOR EPCAM AS THE GENE FOR CONGENITAL TUFTING ENTEROPATHY
Tiffany D. Schaible1, Mamata Sivagnanam2, Robert H. Byrd3, Milton J. Finegold3, Reka Szigeti3, Nina
Tatevian3, Sarangarajan Ranganathan4, Richard Kellermayer1
Section of Pediatric Gastroenterology, Hepatology and Nutrition, Department of Pediatrics, Baylor
College of Medicine, Texas Children's Hospital, Houston, TX; 2Division of Gastroenterology, Hepatology
and Nutrition, Department of Pediatrics, University of California San Diego, Rady Children's Hospital, San
Diego, CA; 3Department of Pathology, Baylor College of Medicine, Texas Children's Hospital, Houston, TX;
Children's Hospital of Pittsburgh, Pittsburgh, PA
Congenital tufting enteropathy (CTE) is a rare, but severe disorder resulting in early postnatal diarrhea.
This entity has been recently shown to associate with mutations in the gene encoding epithelial cell
adhesion molecule (EpCAM). However, this association was conclusively made in only 4 individuals thus
far. We present an ethnically distinct patient with CTE affected by the first nonsense mutation described
in EpCAM. Fluorescent immunohistochemistry of duodenal tissue from the patient, demonstrated lack
of EpCAM staining. Our results underscore EpCAM as the gene for congenital tufting enteropathy.
cv 41 DAIRY ALLERGY AS POTENTIAL CAUSE OF RECURRENT INTUSSUSCEPTION
Crystal M. Knight1, Laura Finn2, David Suskind1, Ghassan Wahbeh1. 1Gastroenterology, Seattle Children's
Hospital, Seattle, WA; 2Pathology, Seattle Children's Hospital, Seattle, WA
A 4 year old female presented with abdominal cramps and subsequently demonstrated ileocolic
intussusception. Within 48 hours after air enema reduction, intussusception recurred twice with
successful nonoperative reduction. Contrast enema studies showed residual ileocecal valve edema
(figures 1, 2). A nuclear scan for Meckel’s diverticulum was normal.
Milk protein allergy had been diagnosed in infancy due to vomiting and diarrhea, and later peanut
allergy presented with facial rash and swelling. At age 1 year she had ileocolonic intussusception which
was reduced by air enema. She was asymptomatic since on a dairy and peanut free diet. Milk was
reintroduced 3 days prior to the latest episode.
Colonoscopy (done to assess for lead points) was normal except for prominent edema and erythema in
the terminal ileum. Capsule endoscopy revealed lymphoid hyperplasia in the distal ileum (figure 3).
Histologic exam showed focal neutrophilic inflammation, reactive epithelium and moderate mucosal
eosinophilia, raising the possibility of allergic enteritis (figure 4). She received oral steroids and was kept
dairy free. After 1 year of follow up, she remained without symptoms.
Intussusception is the second most common cause of bowel obstruction in children, most commonly in
the ileocecal region. The majority occur in children under one year of age. In children age > 1 year, a
pathologic lead point is not uncommon. There are no prior reports of allergic enterocolitis association
with intussusception. Given this temporal association to the reintroduction of dairy and the biopsy
findings, as well as the resolution after treatment with steroids and resumption of a dairy‐free diet, we
propose dairy allergy to be a plausible cause of intussusception in our patient. Allergic enterocolitis
should be considered as a trigger of recurrent intussusception in the future, particularly in children with
prior known allergies.
cv 42 HERMANSKY PUDLAK SYNDROME; A PEDIATRIC CASE WITH SEVERE COLITIS AND GOOD
RESPONSE TO INFLIXIMAB
Lina M. Felipez, Ranjana Gokhale, Barbara Kirschner, Stefano Guandalini. Pediatric Gastroenterology,
University of Chicago Hospitals, Chicago, IL
Hermansky Pudlak Syndrome (HPS) is a rare autosomal recessive disorder characterized by tyrosine
positive oculocutaneous albinism, bleeding diathesis resulting from platelet dysfunction and ceroid
deposition within the reticuloendothelial system. HPS results from 1 of at least 7 different gene
mutations. It has been associated with fatal pulmonary fibrosis and severe colitis. The colitis has been
associated mostly with HPS 1 and HPS 4 genotypes and is severe and poorly responsive to medical
We report a case of a pediatric patient with HPS type 1 with clinical, endoscopic and histologic features
of Crohn's disease, refractory to medical treatment with azathioprine, sulfasalazine, flagyl and steroids.
The patient was started on infliximab infusions with good response and quiescent colitis but later
developed perianal fistulas. IBD serology was positive for ASCA IgA, Anti‐OMPC IgA and PANCA. These
observations suggest there are many similarities between granulomatous colitis in HPS and Crohn's
disease. The pathogenesis of granulomatous colits has been attributed to the lysosomal accumulation of
ceroid lipofusion but this relationship is speculative. The findings of severe colitis at presentation and
perianal fistulization refractory to infliximab suggest that HPS is a severe form of inflammatory bowel
disease that may need aggresive treatment comparable to severe Cronh's disease.
cv 43 COLLAGENOUS GASTROENTEROCOLITIS CAUSING PREOTEIN LOOSING ENTEROPATHY IN A
Osama F. Almadhoun, Megan Gabel, Thomas Rossi. Pediatric GI, University of Rochester Medical Ctr,
Collagenous Colitis (CC) is a cause of watery diarrhea in the elderly, but it is rarely reported in children.
Protein losing enteropathy (PLE) in association with this disease has never been reported in children. We
report a 15 month old boy who presented with severe diarrhea, diffuse edema, and hypoalbuminemia.
Further testing revealed PLE associated with collagenous colitis and enteritis.
Our patient is a 15 month old who presented with progressive peripheral edema for two weeks. Parents
reported that the child had 4‐5 explosive diarrheal bowel movements per day for approximately 4 weeks
prior to presentation in addition to vomiting after almost every meal. His physical exam was significant
for facial, upper limb, and lower limb edema. Albumin level was 2.4, and total protein 3.6.Stool for alpha
one antitrypsin was >1.33,consistent with protein losing enteropathy.Celiac panel, EBV and CMV
profile,stool for bacterial and viral culture, were all negative.Upper endoscopy showed edematous
antral and duodenal mucosa.The rectal and sigmoid mucosa appeared normal on flexible
sigmoidoscopy.Biopsies showed wide subepithelial band of collagen typical of collagenous
gastroenterocolitis.The patient was started on oral budesonide 3 mg per day for 5 days but he continued
with diarrhea and protein loss requiring parenteral replacement. Methylprednisolone was then added at
2 mg/kg/day. A marked improvement of his symptoms was noticed, and gradually he tolerated oral
intake and was weaned off TPN.
Collagenous Colitis is a rare entity in Pediatrics. While there are 12 reported pediatric cases of CC none
of them were found to have small intestinal involvement. This is a novel description of severe protein
losing enteropathy as the presenting feature of collegenous gastroenterocolitis in child. Our patient was
treated according to modified guidelines established for adults and has had a favorable response.
cv 44 ULCERATIVE DUODENITIS IN A CHILD WITH CELIAC DISEASE
Richard L. Mones1, Geraldine O. Mercer2. 1Pediatric Gastoenterology/Nutrition, Goryeb Children's
Hospital, Morristown, NJ; 2Dept. of Pathology, Morristown Memorial Hospital, Morristown, NJ
A 15 month old boy was seen because of a 2 month history of weight loss, vomiting, irritability and
abdominal distension. His family history was non‐contributory.He had been having one to two formed
bowel movements per day. The review of systems was otherwise non‐contributory. An UGI series,
abdominal ultrasound, CBC, metabolic panel, TSH and T4, CRP, food allergy panel and celiac screen were
all normal. Stool for infectious agents were negative. On exam, he was a thin, irritable, sallow appearing
toddler with a distended abdomen. An UGI endoscopy was performed. There were multiple ulcers of the
duodenal bulb and the second part of the duodenum. Biopsies of the duodenum showed ulceration of
the mucosa, total villous atrophy, marked glandular hyperplasia and increased numbers of IELs. On the
day following the endoscopy, the repeat celiac screen was received and was as follows: Anti‐Human
Tissue Transglutaminase IgA >100U/ML, Anti‐Endomysial IgA Ab. positive, Anti‐Giadin IgG Ab 49.3U/ml
and the Anti‐Gliadin IgA Ab was 21.9U/ml. The patient was treated with a brief course of oral
corticosteroids and a gluten free diet. He is happy, totally asymptomatic and thriving.
Ulceration of the duodenum is a rare finding in children with celiac disease. The first description of
ulcerative duodenitis in children with celiac disease was reported by Eltumi et.al.(1)
This case also illustrates the great variability of results when screening for celiac disease.
1.Eltumi M, Brueton MJ, Francis N. Ulceration of the Small Intestine in Children with Celiac Disease. Gut.
TTG IgA Ab <3U/ml 9/08/2008 EGD/Biopsy 11/18/2008 TTG IgA>100U/ml
EMA not done EMA +
Gliadan IgA Abs. <3U/ml Gliadin IgA Abs. 21.9U/ml (<5 WNL)
cv 45 HEMATURIA & BLADDER GRANULOMA WITHOUT ENTEROVESICULAR FISTULA AS
PRESENTATION OF CROHN'S DISEASE
Kara M. Sullivan1, Aseem R. Shukla2, Glenn R. Gourley1. 1Pediatric Gastroenterology, University of
Minnesota, Minneapolis, MN; 2Pediatric Urology, University of Minnesota, Minneapolis, MN
13‐year old male referred to pediatric GI for evaluation of elevated liver enzymes. His AST and ALT had
been in the 300‐450 range, with normal bilirubin and alkaline phosphatase levels for four months. His
past medical history included an 18‐month history of hematuria and dysuria. Previous evaluations by
pediatric urology and nephrology had revealed hematuria with >150 rbc/hpf, 50 wbc/hpf, normal
calcium and protein to creatinine ratios, multiple sterile urine cultures, a normal renal ultrasound,
normal CT scan of chest, abdomen and pelvis, normal CRP, CBC, ESR, ANA, and electrolytes. Hepatic
panel six months prior to referral was normal. Bladder biopsy showed inflammatory changes with
foreign body reaction and noncaseating granulomas. Evaluation for tuberculosis, chronic granulomatous
disease, and sarcoidosis were negative. Urine culture and PCR for viruses, Chlamydia and gonorrhea
were negative. Viral hepatitis testing was negative. His ANCA was positive at 1:80 (reference range
He reported occasional abdominal discomfort when eating dairy products and having 4‐5 loose stools
daily. There was a family history of rheumatoid arthritis in his grandfather and a cousin with an
undefined autoimmune disease. His medications included pyridium 100 mg three times daily for bladder
pain, and daily Tylenol and ibuprofen. IBD serology was consistent with ulcerative colitis. His liver
enzymes returned to normal with discontinuing his medications. Endoscopy revealed an ulcer in the
duodenum, but was otherwise grossly normal. Biopsies revealed granulomatous inflammation in the
stomach, duodenum, terminal ileum and colon consistent with Crohn’s disease. He was treated for
Crohn’s disease with steroids, 5‐ASA and 6‐MP with resolution of his symptoms and hematuria, gross
and microscopic. While extraintestinal manifestations are common in IBD, granuloma without fistula is
very rare, especially in a patient without a previous diagnosis of Crohn’s disease.
cv 46 BEDSIDE SUCTION RECTAL BIOPSY FOR GRAFT‐VS‐HOST DISEASE IN CHILDREN
Robert P. Dillard, Ashok Raj, Alexandra C. Cheerva. Pediatrics, University of Louisville, Louisville, KY
Background. Graft‐vs‐host disease (GVHD) is often a major complication of hematopoietic stem cell
transplantation (BMT) in children. Clinical criteria can be inadequate for accurate diagnosis and tissue
biopsy of the intestinal tract is required. These seriously ill patients are at increased risk for procedural
complications and routinely are emotionally exhausted. Our aim was to evaluate our experience with
bedside suction rectal biopsy (SRB) to determine if it could establish the diagnosis of GVHD using
minimal or no sedation. Others found rectal biopsy useful. However, this is the first report of bedside
suction rectal biopsy as the initial test.
Methods. A retrospective chart review was done of children who underwent bedside SRB for suspected
GVHD following allogenic BMT at the Kosair Children’s Hospital in Louisville, KY. from August 2006 to
Results. Seven children required tissue biopsy to differentiate GVHD from other diagnoses. Six had
bedside SRB and the diagnosis of GVHD was established from histology in 4 cases. One had a negative
biopsy and no further evidence of GVHD. One had a negative SRB but subsequent endoscopic biopsies
were positive. One had mild GVHD, evidence of infectious proctitis with repeat stool culture positive for
an enteric pathogen. No complications occurred. One had minimal midazolam for anxiety, one a small
dose of fentanyl by patient controlled analgesia and one distraction therapy. The remainder agreed to
no intervention following assurance about the ease and painlessness of the procedure. No colon
preparation was required and tissue was obtained within hours of gastroenterology service
consultation. All patients and family members were relieved and pleased with the rapidity of diagnosis
and ease of the procedure.
Conclusions. We conclude that, in children, bedside SRB should be the initial test for suspected GVHD. It
provides a readily available, safe and rapid method to obtain tissue. Potential complications from
anesthesia and more invasive procedures are avoided as well as emotional trauma to already anxious
children and their families.
cv 47 CELIAC DISEASE DIAGNOSED IN A PEDIATRIC PATIENT WITH HIRSCHSPRUNG DISEASE
Alexandra N. Menchise, Adria A. Condino, Michael J. Wilsey. University of South Florida College of
Medicine, Tampa, FL
Hirschsprung disease is a disorder of neural crest migration characterized by intestinal aganglionosis
along a variable segment of the gastrointestinal tract. It is a complex disorder associated with several
syndromes. Celiac disease is an autoimmune enteropathy characterized by a dietary intolerance to
gluten proteins. Celiac disease can mimic Hirschsprung disease when it presents with constipation and
megacolon. We present the case of celiac disease diagnosed in a pediatric patient with Hirschsprung
disease. CASE REPORT: A five year old Caucasian male with history of delayed meconium passage (>48
hours) was diagnosed with Hirschprung disease during infancy by contrast barium enema and full‐
thickness rectal biopsy. Patient underwent a primary pull‐through procedure with resection of the
rectum, sigmoid colon, and part of the descending colon and primary coloanal anastomosis without
colostomy at seven months of age. Gross pathology and histology confirmed proximal megacolon, recto‐
sigmoid transition zone and aganglionosis of the rectum. The patient did well postoperatively until 18
months of age when he developed progressive constipation, abdominal distention and recurrent fecal
retention, which was only partially responsive to laxative therapy and rectal irrigation. Celiac testing at
age three revealed elevated TTG IgG (>100 U/mL) and IgA ( >100 U/ml) levels and he was homozygous
for HLA‐DQB1*0201 allele. Duodenal biopsies showed severe villous atrophy and crypt hyperplasia
consistent with celiac disease. The patient symptomatically improved with a gluten‐free diet, but later
required surgical revision of his pull through at age four. Currently, the patient is maintained on laxative
therapy and rectal irrigation as needed. CONCLUSIONS: To the authors’ knowledge, this is the first case
of celiac disease diagnosed in a pediatric patient with concurrent Hirschsprung disease. Further research
is warranted to establish whether an association exists between celiac disease and Hirschsprung disease
or if celiac disease can complicate and delay the diagnosis of Hirschsprung disease.
cv 48 OCCULT COLONIC DUPLICATION: A CASE REPORT
Andrea C. Hernandez Troya1, Souheil Gebara1, David A. Bloom2, Winston K. Chan3. 1Department of
Pediatrics, Beaumont Children’s Hospital, Royal Oak, MI; 2Department of Pediatric Radiology, Beaumont
Children’s Hospital, Royal Oak, MI; 3Department of Pediatric Surgery, Beaumont Children’s Hospital,
Royal Oak, MI
Gastrointestinal (GI) duplication is uncommon and has a variety of clinical presentations. Colonic
duplication (CD) is even less common, occurring in 4‐18% of all cases of GI duplication. We report the
case of a 33 week GA female, born with neonatal bowel obstruction. The initial contrast enema (CE)
demonstrated a microcolon and possible ileal atresia (IA). No other anatomic issues were identified. An
IA was found at surgery, with resection and primary reanastamosis. At 6 weeks of age, the patient
developed recurrent episodes of diarrhea and abdominal distention, suggestive of bacterial overgrowth,
perhaps in relation to ileal stricture, dysmotility and prestenotic small bowel ectasia. An upper GI was
performed at 5 months of age, demonstrating a dilated loop of bowel on the scout radiograph that
eventually filled with barium. It extended into the pelvis, paralleling the rectosigmoid. Its exact etiology
was unclear, so the patient underwent a CE one week later. The scout image showed retained contrast
in the same dilated loop as seen by prior upper GI. During the CE this loop filled further with contrast,
extended deep into the pelvis, appeared to be colonic in origin, and was blind ending at its distal extent.
At laparotomy, a CD was found with a communication between native colon and the duplication at the
level of the splenic flexure. The CD was resected without complication. Since then, the patient has been
well, with resolution of all GI symptoms. To our knowledge, no case of a communicating CD that
presented in a similar, delayed fashion, with an initial negative CE and surgery has been reported. We
hypothesize that the communication between the CD and native colon was missed at CE due to
meconium and other thick secretions obstructing the fistula, or that the size of the microcolon and
adjacent dilated small bowel led to occlusion of the connection. Duplication must always be considered
in patients with symptoms of bacterial overgrowth.
cv 49 HEPATIC PELIOSIS AND GANULOMATOUS HEPATITIS IN A CHILD WITH CROHN'S DISEASE
Chuan‐Hao Lin. Children Hospital LA, Los Angeles, CA
Background: Increases in childhood Crohn's disease have paralleled overall population trends.
Sulfasalazine and ‐ASA have been used as active and preventive treatment for Crohn's disease.
Azathioprine (AZ)/6 MP, given as the immunomodulatory drugs, have become a mainstay of the
management of pediatric Crohn's disease. However, there are potential adverse effects of both
OBJECTIVE: We report a pediatric Crohn's disease patient treated with Sulfasalazine and 6 MP. The
patient developed adverse effects of both medications with hepatic peliosis and granulomatous
DESIGN/METHODS: A 9 years old female presented with bloody diarrhea was diagnosed with Crohn's
disease by endoscopic findings. She was initially treated with Prednisone, Sulfasalazine and 6 MP with
improvement. The prednisone was tapered after 6 months treatment. She was found to have mild
hepatomegaly and minimally elevated liver enzymes (ALT:68) after 14 months of treatment with both
medications. A CT scan showed hepatic peliosis. 6 MP was discontinued after CT scan finding.
RESULTS: Two months after discontinuation of 6 MP, a repeat CT scan showed resolution of hepatic
peliosis. A liver biopsy was performed one month after discontinuation of 6 MP. The liver biopsy showed
extensive granulomatous hepatitis and no micro‐organisms were found. Sulfasalazine was discontinued.
Asacol was introducted as the only medication for treatment of her Crohn's disease. Prednisone was
added again 4 months later due to active terminal iletis. CONCLUSIONS: 1)Potential adverse effects of 6
MP in the treatment of pediatric Crohn's disease include hepatic peliosis. 2) Granulomatous hepatitis
can occur in pediatric Crohn's disease treated with Sulfasalazine. 3) Hepatobiliary involvement of
pediatric Crohn's disease includes granulomatous hepatitis and fibrosis. 4) Early detection of
hepatobiliary lesions using CT scan and/or liver biopsy is indicated with pediatric Crohn's disease with
hepatomegaly and/or mildly elevated liver enzymes.