Paul H. Parker

Solitary rectal ulcer: a rare cause of gastrointestinal bleeding in an adolescent with hemophilia A.

Solitary rectal ulcer syndrome (SRUS) is a rarely reported condition in children. The typical presentation is one of anorectal pain with passage of blood and mucus per rectum in the setting of defecation abnormalities. Diagnosis is made via endoscopy and biopsy. Solitary rectal ulcer syndrome alone is usually a benign condition; however, significant morbidity can occur if complicated by underlying disease states. We report an adolescent with hemophilia A and SRUS who presented with a rectal bleed that required blood transfusion.

Medulloblastoma in association with the Coffin-Siris syndrome

An 8-year old patient with Coffin-Siris syndrome is presented, who was found to have a medulloblastoma during the evaluation of apnea and ventilator dependency. Although several cases of Dandy Walker cysts and one case of brain-stem heterotopia have been described in this rare syndrome, this is the first report of medulloblastoma in a patient with Coffin-Siris syndrome. Possible pathogenetic mechanisms are briefly discussed.

The inflammatory polyp-fold complex in children.

The inflammatory polyp–fold complex (IPFC) is an uncommon endoscopic or radiologic finding in children. In this complex, an inflammatory polyp at the gastroesophageal junction is present, often in continuity with a prominent gastric fold. Histologically, there is an inflammatory infiltrate in otherwise benign gastric and esophageal mucosa. We report four cases of IPFC in children, all associated with reflux esophagitis. In two patients who underwent repeat endoscopy, acid suppression therapy led to a decrease in the size of the polyp and histologic improvement of esophagitis. Four case studies in children with IPFC are presented, followed by a literature review of this endoscopic finding as it applies to children.

Bullous Pemphigoid Complicating Crohn's Disease in a Child:

was extremely thin with multiple cutaneous lesions. There was a mixture of hyperpigmented scars; open, ulcerated lesions; and clear vesicles and bullae, most prominent over the upper chest and extensor surface of the hands, legs, and feet. The oral cavity showed ulcerations on the tongue, gingiva, and buccal mucosa (Figure 1). Findings from heart and lung examination were normal. The abdomen was scaphoid, non-

Digital Desquamation—A New Finding in Perianal Streptococcal Dermatitis

as a perianal rash accompanied by perianal itching, pain, and/or rectal bleeding. The clinical features are being continually expanded as this disorder becomes more widely recognized. We report a young boy with perianal streptococcal dermatitis who developed desquamation of his fingers concomitant with treatment and resolution of his perianal complaints. While digital desquamation is known to follow scarlet fever and streptococcal pharyngitis, we report the association of digital desquamation with perianal streptococcal dermatitis, expanding upon the cutaneous findings of this disease. Patient Report

Colonic chicken skin mucosa: association with juvenile polyps in children

OBJECTIVES:Chicken skin mucosa is a newly described endoscopic finding associated with colonic neoplasms in adults. Chicken skin mucosa was sought in children with juvenile polyps to determine the prevalence, endoscopic features, and location. An alternative theory is proposed for the pathogenic mechanism of this finding.METHODS:Children having colonoscopy and polypectomy were prospectively evaluated for the presence of chicken skin mucosa. The location of the polyps was determined at colonoscopy; the size of removed polyps was measured during processing of samples in pathology. Biopsies from colonic chicken skin mucosa were stained with hematoxylin and eosin and mucicarmine.RESULTS:Over a 1-yr period, 27 juvenile polyps were removed from 15 children at colonoscopy. Eleven of 15 children (73%) were found to have polyps with chicken skin mucosa; overall, 43% of the polyps had associated chicken skin mucosa. Chicken skin mucosa–positive polyps were larger than chicken skin mucosa–negative polyps and were only found in the rectosigmoid colon. Lipid-laden macrophages were found in all samples of chicken skin mucosa tested.CONCLUSIONS:Chicken skin mucosa is a common finding in children with juvenile polyps. It probably is the result of local mucosal trauma, rather than a preneoplastic lesion.