gastrointestinal manifestations in a child with the wrinkly skin syndrome
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esophageal cyst. EUS showed multiple peri-esophageal cysts with apparentcommunication. Further imaging with MRI of the chest showed a tubularstructure with its cephalad extent noted at a level 2-3 cm above the clavicleand its caudad extent identified at L1-2. To clarify further, she was broughtback for fluoroscopy and this structure was injected with radiocontrast andTc99m-macroaggregated albumin (MAA) with EUS guidance. The fluo-roscopy showed a tubular tortuous structure from the upper abdomen to theregion of the left subclavian vein consistent with a diffusely dilated thoracicduct. Pulmonary perfusion scan with Tc99m-MAA did not show traceruptake in the lungs also consistent obstruction.Conclusion: Diffuse dilation of the thoracic duct has not been previousreported. Although outlet obstruction due to a mass is considered in ourpatient, the imaging studies have not yet revealed any mass. At this point,our patient has not developed any new complaints and has had no lymphed-ema. We are closely following this patient with serial imaging studies toevaluate for new masses.
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COMPLICATIONS OF GASTRIC SURGERY FOR MORBIDOBESITYYashma Patel, M.D., Anshuman Jyoti, M.D.,Lakshmanasamy Somasundaram, M.D., Yatin R. Patel, M.D.,Raman M. Patel, M.D., FACG* Lancaster, CA.
We present a case of full thickness erosion of the polypropylene mesh intothe gastric pouch, 13 years after vertical banded gastroplasty causingrecurrent abdominal pain, nausea, and vomiting. A 72 year-old Caucasianfemale with a history of vertical banded gastroplasty in 1985 for morbidobesity and gastric bypass surgery in 1990, presented with recurrent ab-dominal pain, nausea, vomiting and weight loss of several weeks duration.An EGD was preformed to evaluate for possible complications whichshowed black polypropylene mesh. It appeared to be part of the bandedgastroplasty material eroding through the wall into the stomach causingpartial obstruction. The patient was advised to have surgical removal of themesh which she failed to follow for fear of weight gain. One and half yearslater, she presented with the same symptoms. EGD found worsening of thepolypropylene mesh into the stomach so she agreed for surgery. At the timeof surgery, the polypropylene Dacron band was found to have eroded atleast one third of its circumference into the lumen of the banded area of thestomach. The Dacron patch was transected in one spot and removed in toto.Post operatively the patient did very well and has continued to do well untiltoday. The common indications for endoscopy in the postopertative bari-atric patient include evaluation of the symptoms, the management ofcomplications, and evaluation of failure of weight loss. Nausea and vom-iting are fairly common in these patients. Some of the frequently seencomplications could involve stomal stenosis, stomal ulceration, and erosionof banding material causing ulceration and obstruction. Several of thesecomplications will require endoscopic and surgical evaluation.
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GASTROINTESTINAL MANIFESTATIONS IN A CHILD WITHTHE WRINKLY SKIN SYNDROMELeonel Rodriguez, M.D., Rajendra Setty, M.D.,Richard Rosencrantz, M.D.*, Alan Shanske, M.D. Children’s Hospitalat Montefiore, Bronx, NY.
The wrinkly skin syndrome (WSS) is characterized by excessive wrinklingof the skin on the dorsum of the hands, feet and trunk, decreased skinelasticity, an increased number of palmar and plantar creases, craniofacialanomalies, musculoskeletal abnormalities, neurological involvement andmental retardation. We present a child with WSS and prominent gastroin-testinal abnormalities. A male patient presented in the newborn period withphysical findings of wrinkled skin over the trunk, abdomen and extremities.He was also was noted to have both an umbilical and an indirect right
inguinal hernia. Severe gastroesophageal reflux, oropharyngeal dysphagia,esophageal dysmotility and fluoroscopy-proven aspiration resulted in poorfeeding which required gastrostomy tube placement and a Nissen fundo-plication. His first year of life was complicated by recurring episodes ofabdominal distention, bilious vomiting and mild constipation. An upper GIseries with small bowel follow through demonstrated dilated small bowelloops that implied the presence of a distal intestinal obstruction. Explor-atory laparotomy revealed a cecal malrotation, which was corrected with-out resection. His upper obstructive abdominal symptoms markedly im-proved following the surgical procedure. However, signs and symptoms ofconstipation gradually worsened. A repeat UGI showed normal smallbowel morphology and transit time, however, there was moderate right-sided colonic distention with the presence of abundant stool in the distalcolon. Histology obtained by suction rectal biopsy, demonstrated the ab-sence of ganglion cells in the submucosa and also showed increasedacetylcholinesterase stained fibers consistent with a diagnosis of Hir-schprung’s Disease (HD). To our knowledge, this is the first report of apatient with this specific cluster of gastrointestinal complications in WSS.Recognizing an association between WSS and HD may be important, asWSS could find new classification as a form of syndromic HD.
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LYMPHOMA IN A PATIENT RECEIVING PEG INTERFERONAND RIBAVARIN THERAPY FOR HEPATITIS C VIRUSINFECTIONVikas Khurana, M.D.*, Prasanth Gogineni, M.D., Sami Bahna, M.D.Louisiana State University Health Sciences Center and Overton BrooksVA Medical Center, Shreveport, LA and Louisiana State UniversityHealth Sciences Center, Shreveport, LA.
Introduction: There are an estimated 3.9 million people with HCV infec-tion in the United States. Currently, pegylated interferon (PEG-IFN) incombination with ribavirin has been found to be the most effective therapy.PEG-IFN is a relatively new treatment modality and as such many sideeffects may still be unknown.Case Report: A 50 year-old African American man was admitted formultiple episodes of syncope. His past history revealed Non-Hodgkin’sLymphoma in remission and Hepatitis C virus infection genotype 1a. Thelatter was recently treated with Ribavirin and PEG-IFN Alfa 2a, which wasdiscontinued at 44 weeks, 4 weeks before completion of therapy due tofatigue and anemia. Physical exam revealed moderate rubbery anteriorcervical adenopathy and hepatosplenomegaly. CT scan of abdomen andpelvis revealed hepatosplenomegaly and inguinal and pelvic lymphadenop-athy. Lymph node biopsy showed diffuse infiltration by large lymphoidcells; large, lobulated vesicular tumor nuclei; numerous mitotic cells,consistent with lymphoma. Bone marrow biopsy showed active hemato-poiesis, increased number of immature large cells either immature myeloidor erythroid precursors or lymphoma cells. While he was in the hospital, thepatient developed acute dyspnea and severe respiratory distress that neces-sitated assisted ventilation, IV fluids and IV vasopressors. Patient initiallyimproved, but with in few days he deteriorated rapidly and died.Discussion: Interferon therapy is known to cause IFN alpha gene expres-sion, which can lead to lymphocytic proliferation and has been linked to thedevelopment of cutaneous sarcoidosis in some patients with chronic hep-atitis C. PEG-IFN with ribavirin therapy may be the cause of the relapse ofhis lymphoma. Lymphoma has not been previously reported as a side effectof PEG-IFN treatment but our case suggests that possibility.Conclusion: PEG-IFN is now the standard treatment for hepatitis C bothfor its effectiveness and ease of weekly administration, but it is a strongimmuno-modulator with side effects that are still not completely known.The development of lymphoma in our patient may be a complication ofPEG-IFN and is worth further investigation.
S191AJG – September, Suppl., 2003 Abstracts