Acquired rectovaginal fistula.

Nine girls presented with an acquired rectovaginal fistula shortly after birth. All mothers tested were seropositive for HIV. It is suggested that an acquired rectovaginal fistula is an early manifestation of HIV infection in girls.     

Graft-versus-host disease and phimosis

Graft-versus-host disease rarely causes genitourinary problems. We report a case of pathological phimosis in a child secondary to chronic graft-versus-host disease.     

High incidence of upper gastrointestinal tract involvement in children with Crohn disease

This retrospective study of Crohn disease in 230 children and adolescents with a mean age of 12.5 years at the time of diagnosis and an average follow-up of 6.6 years showed that 30% had lesions of the esophagus, stomach, and duodenum. Three patients had Crohn disease isolated to the upper gastrointestinal tract. The 169 patients with both small and large bowel disease were at greater risk (33%, P less than .05) of having upper gastrointestinal lesions than the 37 with isolated small bowel disease and the 21 with disease limited to the colon and/or rectum. An aggregate of symptoms and signs more likely present in those with upper gastrointestinal involvement included: dysphagia, pain when eating, nausea and/or vomiting, and aphthous lesions of the mouth. Furthermore, weight loss was more severe and hypoalbuminemia more frequent. Because upper gastrointestinal series x-ray studies failed to detect upper gastrointestinal lesions in 13 patients of 69 of those with upper gastrointestinal disease, endoscopy should be considered in all children and adolescents in whom a diagnosis of Crohn disease is entertained. Endoscopy and biopsy of the upper gastrointestinal tract should be done in any patient with symptoms suggestive of proximal involvement. Finally, in view of the fact that endoscopy established the diagnosis of Crohn disease in five patients previously thought to have chronic ulcerative colitis, the procedure should routinely be performed in all patients with chronic ulcerative colitis or indeterminate colitis before surgery is performed.     

Fatal polyarteritis nodosa with gastrointestinal involvement in a child

Polyarteritis nodosa (PAN) is a necrotizing angiitis that predominantly affects small- and medium-sized arteries. Polyarteritis nodosa occurs rarely during childhood. Boys and girls seem to be equally affected, with a peak at the age of 10 years. Rarely, severe and fatal gastrointestinal involvement is seen in PAN. Here, we report a 15-year-old boy with PAN, who had gastrointestinal involvement with multiple aneurysms of the hepatic and superior mesenteric arteries. This involvement could be demonstrated with conventional angiography and gastrointestinal bleeding scintigraphy. The progression of the symptoms and the decrease in the size of the aneurysms were noted after combination treatment with cyclophosphamide and prednisolone, but there was severe bleeding from small bowel, and it was taken under control by resection of jejunum. However, the patient died because of sepsis. In conclusion, severe gastrointestinal involvement in PAN is usually fatal despite aggressive therapy, as is the case in our patient.     

Autologous buccal mucosa graft for repair of recurrent rectovaginal fistula

Post-operative pediatric rectovaginal fistulas are rare, can be challenging to repair, and often recur. The versatility, ease of accessibility, vascularization, and likeness to native vaginal tissues make autologous buccal mucosal grafts a novel tissue substitute for the repair of a recurrent rectovaginal fistula after the surgical repair of anorectal malformations.     

Graft-versus-host disease in rhabdomyosarcoma following transfusion with nonirradiated blood products

Graft-versus-host disease is a complication of transfusion of nonirradiated blood products into an immunocompromised patient, and it has been reported in hematologic and lymphoid malignancies as well as in neuroblastoma in children. We report the first case of acute graft-versus-host disease in a child undergoing chemotherapy for a rhabdomyosarcoma, who received transfusions during an intercurrent illness. The graft-versus-host disease was accompanied by an allogeneic lymphoid graft and was diagnosed on the basis of HLA studies on the patient's and her family's circulating lymphocytes and confirmed by HLA typing of the patient's skin fibroblasts.     

Erdheim-Chester disease with multisystem involvement in a 4-year-old

Erdheim-Chester disease is a rare form of non-Langerhans histiocytosis of unknown origin occurring mainly in adults. It is extremely rare in children. We report a case of a 4-year-old boy with Erdheim-Chester disease that initially presented as hemifacial palsy and bone pain with multisystem involvement. We describe radiographic findings of bones that show characteristic bilateral symmetrical osteosclerosis with atypical osteolytic lesions in addition to CT findings for pulmonary involvement and MR findings for intracranial lesions.     

Multicentric Castleman's disease in a child with subpectoral involvement

Castleman's disease is a benign lymphoproliferative disorder characterised by enlarged hyperplastic lymph nodes. It is rare in children and usually presents as localised disease. Subpectoral involvement has not been previously described in multicentric Castleman's disease in children. We present the CT, US and Doppler US findings of hyaline-vascular type multicentric Castleman's disease in a 5 year-old-boy with masses in the left subpectoral region and supraclavicular and axillary lymphadenopathy.     

Multiorgan involvement in a pediatric patient with hydatid disease

Human Echinococcus infection still remains an important health problem in endemic regions. Herein, we report a 5-year-old boy with hydatid disease who has spleen, lung, kidney and liver involvement simultaneously. To our knowledge, there is no pediatric case with hydatid disease in the literature reporting simultaneous involvement of spleen, kidney, liver and lungs as in our case.     

Laparoscopic assisted endorectal pull-through with posterior sagittal approach to the repair of postoperative rectourethral and rectovaginal fistula

Rectourethral or rectovaginal fistula is a troublesome complication after anorectal surgery. The pelvic and perineal dissection may be difficult because of severe fibrosis adhesion around the fistula. The authors applied a novel technique: a combined laparoscopic assisted abdominal and posterior sagittal approach (PSA) to perform the redo surgery. Three boys and two girls (3–13 years old): case 1 had rectovaginal fistula after rectal dialation and modified Swenson’s procedure; case 2 had rectovestibular fistula after twice perineal anorectoplasty; case 3 had rectourethral fistula after twice anorectoplasty; case 4 was imperforate anus with Hirschsprung’s disease and rectourethral fistula that had been misdiagnosed; case 5 had rectourethral fistula after abdominoperineoanoplasty and Mollard procedure and posterior sagittal anorectoplasty. Laparoscopic assisted abdominal dissection was done first to mobilize the colon as far as the mid pelvis, and the normal colon was marked with a suture. The lower pelvic dissection was performed through the posterior sagittal route, the proximal rectum was mobilized and servered, the distal rectum was left undisected, endorectal mucosectomy with electric ablation was performed, then the fistula was closed from inside the rectum, and the stump of the colon was pulled through the rectum, the stump and the dentate line were anastomosed extraanally. Colostomy was done in case 2 and case 5. The postoperative follow-up showed no recurrent fistula, and all patients had attained normal voluntary bowel actions, but one child had infrequent minor soiling. Laparoscopic assisted endorectal pull-through of the intact colon can offer precise dissection, minimal abdominal injure, and spare troublesome mobilization of the fistula, and can prevent the recurrent of fistula. Posterior sagittal approach provides a direct repair of the fistula and anastomosis.     

Griscelli disease with cerebral involvement

A 9-month-old Turkish boy was diagnosed as having Griscelli disease (Chediak-Higashi-like syndrome). Clinical signs consisted of silver-grey hair and a relatively light skin colour, recurrent episodes of fever, with or without detectable infections, increasing hepatosplenomegaly, hypotonia and motor retardation. Laboratory studies showed pancytopenia of varying degree but neither inclusion bodies nor vacuoles were seen in his leucocytes. Serum immunoglobulin levels were normal except for a IgG2 deficiency. In the mixed lymphocyte reaction the stimulation capacity of the leucocytes was decreased. Microscopic examination of his hair and electron-microscopic examination of a skin biopsy further confirmed the diagnosis. Shortly before the diagnosis was made, the child developed cerebral symptoms with hemiparesis and convulsions. A CT scan suggested cell infiltration of the brain. A few weeks later the boy died of an infection.     

Basal ganglia involvement in a child with cat-scratch disease

Catch-scratch disease (CSD) is a self-limiting zoonotic illness. Encephalopathy is the most common neurologic manifestation of CSD. Neuroimaging is usually normal with occasional abnormalities reported in children involving the cerebral cortex and thalamus but not previously described in the basal ganglia. Here we report a 7-year-old boy with CSD encephalopathy who developed choreoathetosis with high intensity changes in basal ganglia on magnetic resonance imaging scan.     

Upper gastrointestinal and ultrasound examinations of gastric antral involvement in chronic granulomatous disease

Two brothers with chronic granulomatous disease of childhood are presented with circumferential gastric antral thickening demonstrated on sonography. Barium studies and sonography correlated well in one brother. The antral changes can resolve quickly without antibiotic therapy.     

Primary aortoesophageal fistula : Presenting as massive upper gastrointestinal hemorrhage

Primary aortoesophageal fistula is a rare cause of upper gastrointestinal bleeding. A six-year-old boy presented with massive upper gastrointestinal hemorrhage. Endoscopy revealed a submucosal bulge in the esophagus with an ulcer and clot at the top. Lateral skiagram of the chest showed a posterior mediastinal mass. CT scan of the chest revealed a ruptured aortic aneurysm into the oesophagus, confirmed the diagnosis. The patient succumbed to the illness before he could be subjected to definitive treatment.     

Severe gastrointestinal involvement in children with the acquired immunodeficiency syndrome

Five children with the acquired immunodeficiency syndrome (AIDS) and unusual gastrointestinal disease are described. Two children presented with malnutrition, abdominal distention, and diarrhea. One was found to have moderately severe villus atrophy on jejunal biopsy and was initially thought to have celiac disease. Jejunal biopsy from the second child revealed infiltration of the mucosa with acid-fast bacilli-laden macrophages. A third child suffered recurrent abdominal pain, progressive weight loss, diarrhea, and severe gastrointestinal hemorrhage secondary to infection with cytomegalovirus. Pseudomembranous necrotizing jejunitis associated with overgrowth of Klebsiella pneumoniae in the duodenal fluid occurred in one patient. The fifth child presented in the newborn period with Serratia marcescens cholecystitis. Gastrointestinal disease in children with AIDS may be due to idiopathic villus atrophy and bacterial or opportunistic infection.     

Functional gastrointestinal obstruction in a child with chronic granulomatous disease

A child with chronic granulomatous disease developed an antral-pyloric obstruction, followed a month later by a postbulbar duodenal obstruction. At both areas, there was no evidence of an anatomical lesion, and some improvement in the passage of barium was observed following glucagon and metoclopramide administration. Presumably, symptoms have resulted from a functional disturbance of gastrointestinal motility.     

The involvement of the gastrointestinal tract in posttransplant lymphoproliferative disease in pediatric liver transplantation

BACKGROUND: Posttransplant lymphoproliferative disease (PTLD) is a serious complication associated with the use of immunosuppression after transplantation. In a retrospective study the clinical features of PTLD located primarily in the gastrointestinal tract were analyzed. METHODS: Three hundred ninety-two consecutive pediatric patients who underwent orthotopic liver transplantation (OLT) during a 13-year period with a survival of more than 6 months were reviewed. Two immunosuppression protocols were used: cyclosporin A, or tacrolimus-based primary therapy. Twenty-nine randomly selected liver transplant recipients without PTLD were used for comparison of signs and symptoms of gastrointestinal PTLD. RESULTS: Among the 30 patients identified with PTLD, 9 had gastrointestinal PTLD. The overall incidence density of PTLD was 1.8 per 100 patient-years (30/392). Nine patients (30%) had involvement of the gastrointestinal tract, whereas 7 (23%) had the gastrointestinal tract as the only involved site. When compared with a cohort of liver transplant recipients without PTLD, only gastrointestinal bleeding, weight loss, hypoalbuminemia, and protein-losing enteropathy were signs most likely associated with gastrointestinal PTLD. Hypoalbuminemia was the most sensitive sign of gastrointestinal PTLD. The lower tract (ileum and colon) was the most common site of involvement. CONCLUSIONS: gastrointestinal involvement is common and occurs in 30% of all patients with PTLD. It may be the only affected organ in a subgroup of patients. Hypoalbuminemia, gastrointestinal bleeding, and weight loss are features that are characteristic of gastrointestinal PTLD. Patients with aggressive gastrointestinal signs and symptoms should undergo upper and lower gastrointestinal tract endoscopy with biopsy, to establish the diagnosis.