Human herpes virus-6 encephalitis in a paediatric bone marrow recipient: successful treatment with pharmacokinetic monitoring and high doses of ganciclovir.
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Antiviral Agents↗
Biomedical subjects
T Basset
Publications and source records attributed to T Basset.
[Gastric trichobezoar in an adolescent presenting with anemia: ultrasound and MRI findings].
Trichobezoars are rare and composed of ingested hair or fibers. Diagnosis is usually made by plain radiographs and barium meal. The usefulness of computed tomography has been reported in the preoperative period. In the pediatric population the authors propose, based on a recent case report, a non invasive and non irradiating protocol using sonography and T1W and T2W MR imaging.
Adolescent↗
Long-term results of bladder neck reconstruction for incontinence in children with classical bladder exstrophy or incontinent epispadias.
OBJECTIVE: To review the long-term results of bladder neck reconstruction (BNR) in patients with classical bladder exstrophy or epispadias, and to review the concept of continence surgery in these two groups, stressing the difficulty in finding an adequate balance between urine storage (which implies high outlet resistance and low storage pressure) and complete bladder emptying (which implies low outlet resistance and a transient increase in bladder pressure); surgery cannot achieve 'continence' (which implies active mechanisms) but only 'dryness' (which implies passive mechanisms). PATIENTS AND METHODS: Eighty patients with classical bladder exstrophy (52 male, 28 female) and 25 with incontinent epispadias (17 male, 18 female) had their bladder neck reconstructed after a Young-Dees-Leadbetter procedure, subsequently modified by Mollard. The treatment is detailed and results reviewed after a mean follow-up of 11 years. All patients were treated and followed in the same institution. RESULTS: In the exstrophy group, 36 (45%) patients presented with a dry interval of > 3 h, with urethral emptying after one BNR; 52 (65%) presented with recurrent urinary tract infections, 19 (24%) with urinary stones, 21 (26%) with dilated upper urinary tracts, 13 (16%) with bladder perforations and one with an adenocarcinoma of the bladder. Thirty-eight patients (48%) required further surgery; 51% of all patients required an endoscopic procedure within 3 months after the BNR and 26% had endoscopic procedures for late (> 3 months) urine retention. In the epispadias group, 13 (52%) patients presented with a dry interval of > 3 h with urethral emptying after one BNR; 12 (48%) had recurrent urinary tract infections, five (20%) upper tract dilatation, two (8%) bladder stones, one (4%) bladder perforation and one an adenocarcinoma of the bowels after a ureterosigmoidostomy. Ten (40%) children required further surgery. CONCLUSION: We compared the present results for continence with those in other published series; most complications encountered were related to the obstructive pattern of bladder emptying and the abnormal bladder urodynamic behaviour caused by BNR. We consider that BNR is unpredictable and the roles of the other factors in urinary continence are discussed. Alternative procedures are detailed. The concept of continence surgery in exstrophy and incontinent epispadias is reviewed, stressing the importance of favouring bladder development and limiting obstructive patterns of bladder emptying that cause severe and recurrent complications.
Bladder Exstrophy↗
Mesenteric hernia: a rare cause of intestinal obstruction in children.
A 3-year-old boy presented with small bowel obstruction resulting from incarceration of the distal ileum in a mesentric defect. Attempted laparoscopy was unsuccessful. The hernia was reduced successfully at laparotomy.
Child, Preschool↗
Does nephroblastomatosis influence the natural history and relapse rate in Wilms' tumour? A single centre experience over 11 years.
The presence of multifocal or diffuse nephrogenic rests (NRs) in one or both kidneys is termed nephroblastomatosis (Nbm). Nbm may be a predisposing factor for Wilms' tumour (WT). The aim of this retrospective study was to evaluate the impact of Nbm on the outcome of WT in children. We assessed the outcome of 81 children with Wilms tumours and practical implications of Nbm in the treatment and follow-up. All the pathology slides have been reviewed in 1997. 63 had WT without Nbm (group A) and 18 had WT associated with Nbm (group B). There was no statistical difference between the two groups according to the age at diagnosis and histology. Clinical abnormalities were more frequent in group B (33 versus 8%). There was no statistical difference between the percentage of stage IV in both groups, but bilaterality (stage V) was present only in the group B. Relapse was observed in 20/81 patients (25%): 11 (17%) in group A and 9 (50%) in group B. Mean delay of relapse was longer (25 months) in group B than in group A (10 months). For the whole population, with a median follow-up of 9 years, the event-free survival (EFS) and the overall survival (OS) probabilities were respectively 74%+/-10 and 83%+/-9 at 120 months. The difference in EFS between groups A (82+/-9%) and B (38%+/-29) was significant (P=0.004). The discovery of Nbm in the non-tumoral part of the kidney with WT can be an adverse factor and in particular favours the subsequent development of a new Wilms tumour. It justifies separate follow-up guidelines.
Adolescent↗
Epstein-Barr virus-related lymphoproliferative disease complicating childhood acute lymphoblastic leukemia: no recurrence after unrelated donor bone marrow transplantation.
A 16-year-old boy in complete remission of ALL, undergoing oral maintenance therapy, developed intestinal perforation related to EBV-associated lymphoproliferative disease (LPD). He was successfully managed with surgical resection, acyclovir, immunoglobulins and discontinuation of maintenance therapy. Leukemic marrow relapse occurred 3 months later, treated by polychemotherapy followed by unmanipulated BMT from a matched unrelated EBV seropositive donor. Donor lymphocytes were infused twice after transplant because of delayed immunologic recovery and severe CMV colitis. This was followed by acute GVHD requiring prolonged immunosuppressive treatment. Despite intensive and prolonged immunosuppression, recurrence of LPD was not observed. Following EBV-related LPD, allogeneic BMT can be performed if indicated. Selection of an EBV seropositive donor is of major importance for the prevention of LPD recurrence as the recipient may be protected by passive transfer of EBV-specific cytotoxic T cells.
Adolescent↗
Male epispadias: experience with 45 cases.
PURPOSE: We evaluate the long-term results of surgery for isolated (without exstrophy) male epispadias. MATERIALS AND METHODS: A total of 45 male patients with isolated epispadias were treated and followed at Debrousse Hospital between 1971 and 1993, 8 in whom repairs performed elsewhere had failed and 14 had total urinary incontinence. Incontinence was treated with a modified Young-Dees reconstruction of the bladder neck and the ureters were reimplanted in all cases. Long-term followup was available in 18 cases of a Cantwell-Young-Gross, 4 Duplay, 7 full thickness skin graft, 1 bladder graft and 10 Cantwell-Ransley urethroplasties. Penile reconstruction was performed using a 2-stage procedure in 8 patients (freeing of the corpora followed by urethroplasty), and an isolated urethroplasty in 13. All other patients underwent a single stage repair combining dissection of the penis and urethroplasty. RESULTS: All patients were reassessed clinically. Of 13 incontinent patients followed for 1 to 20 years 11 acquired continence, 1 remains incontinent and 1 underwent a repeat procedure recently. Urethroplasty was immediately satisfactory in 66% of the cases, minor complications developed in 25% and serious complications (especially free graft urethroplasty) developed in 10%. Assessment of genital cosmesis is subjective but it was considered acceptable in most cases. All 29 patients with long-term followup had erections, 24 performed regular sexual intercourse, 17 had normal ejaculations and 4 fathered children. CONCLUSIONS: The principles described by Cantwell-Young and Gross, and modified by Ransley, have considerably improved penile reconstruction. Free graft and Duplay urethroplasties provided unacceptable results in our series. Our results of surgery for incontinence associated with isolated male epispadias (84%) were better than for male exstrophy (63%).
Adolescent↗
[Acute gastric distension necessitating gastrostomy after anesthetic induction for surgical correction of type III esophageal atresia].
IPPV during anaesthesia for management of oesophageal atresia with tracheo-oesophageal fistula (TOF) can cause gastric insufflation. We report such a complication in a one-day-old newborn, who developed, 15 min after induction, a distension of the abdomen, hypoxia and bracdycardia. An emergency gastrostomy was performed. His status improved rapidly and surgery could be completed. TOF was located at the carina and had a large calibre. To avoid gastric distension in such cases, the tip of the tube is located just proximal to the carina, but distal to the fistula to prevent intubation of the latter. Difficulties are due to position of the fistula (carina, main bronchi) or its large bore. Gastric distension carries a risk of regurgitation and inhalation of gastric contents, elevation of hemidiaphragm and lung compression, decreased tidal volume, decreased venous return, cardiovascular collapse and cardiac arrest. When insufflation peak pressures are low, gastrostomy is benefitful, as in our case, as the tidal volume loss through the stomach is acceptable. In case of high insufflation pressures because of co-existing lung disease, gastrostomy is better avoided, as most if not all the tidal volume may be lost through the stomach.
Anesthesia, Inhalation↗
MR cholangiography in neonates and infants: feasibility and preliminary applications.
OBJECTIVE: The objective of the study was to evaluate the accuracy of MR cholangiography in showing the biliary tree in neonates and infants and to show preliminary applications in a pediatric population. SUBJECTS AND METHODS: MR cholangiography was performed with T2-weighted turbo spin-echo sequences (8000/300 [TR/TE], six or eight excitations). The study included seven patients with suspected bile duct disorders and 10 control individuals. RESULTS: In the control group, the extrahepatic bile ducts (EHBDs) were revealed by MR cholangiography in all patients. In one of the five patients with neonatal cholestasis, biliary atresia was excluded by identifying the entire EHBD on MR cholangiograms. In the four other patients with neonatal cholestasis, biliary atresia was suspected because the EHBD could not be seen on MR cholangiography. The diagnosis was confirmed in three of these four patients, in whom the MR finding was associated with significant periportal thickening. In the remaining patient, in whom periportal thickening was not seen, sclerosing cholangitis was diagnosed on biopsy. In the sixth patient with neonatal cholestasis, who had received a liver transplant, MR cholangiography showed biliary dilatation above the biliary-digestive anastomosis. In the seventh patient, a neonate with a prenatal diagnosis of hepatic cyst, MR cholangiography showed a choledochal cyst. CONCLUSION: MR cholangiography may become an important diagnostic tool in the detection of biliary atresia, congenital choledochal dilatation, and biliary complications in hepatic transplantation for pediatric patients.
Bile Duct Diseases↗
Juvenile granulosa cell tumor of the ovary in infants: a clinicopathologic study of three cases and review of the literature.
The clinical and pathological features of three cases of juvenile granulosa cell tumors occurring in infants were studied. Precocious pseudopuberty developed in two patients and acute abdominal symptoms related to the rupture of the tumor developed in one. Surgery was the only treatment in each case and no adjuvant therapy was delivered. No patient experienced relapse. Histological examination showed a predominantly diffuse pattern with prominent luteinization. Call-Exner bodies were absent. Two tumors had multilocular thin walled cysts containing large amounts of estradiol, the third one contained rudimentary microfollicles. The prognosis of juvenile granulosa cell tumors in infancy appears more favorable than those occurring in older patients. No case of tumor recurrence has been reported in infancy so far. Surgery appears to be the state-of-the-art treatment of these tumors and additional therapy (chemotherapy or radiotherapy) must be discussed with caution, even in advanced stages.
Female↗
Female epispadias.
PURPOSE: We assessed the incidence, anatomical features and treatment of female epispadias. MATERIALS AND METHODS: We studied 10 patients followed from 1 to 18 years. Four were operated on in 2 stages: first, cervicoplasty with original technique, and second, reconstruction of genitalia and distal urethra. Two had only a cervicoplasty. The last 4 patients were operated on in 1 stage according to Hendren's technique but with a simplified cervicoplasty. RESULTS: Eight of 10 patients are totally continent, 1 has some small nocturnal leakage, and 1 is incontinent but the interval of dryness is increasing and is longer than 1 hour. Concerning external genitalia, the result is good but not perfect. CONCLUSIONS: Female epispadias is most often complete with total incontinence. The aim of treatment is to reconstruct the anatomy in 1 stage. Treatment is easier and the results are better than in cases of female exstrophy or male incontinent epispadias.
Adolescent↗
Abdominal inflammatory myofibroblastic tumors in children: report of an appendiceal case and review of the literature.
Inflammatory myofibroblastic tumors (IMT) are rare, benign lesions. They mimic, clinically and radiologically, malignant tumors-especially sarcoma. IMT most often are seen in the lung of young adults, but there have been reports of these tumors occurring in children, in various sites. The authors report a case of appendiceal IMT arising in a 15-year-old boy previously treated for inherited distal renal tubular acidosis. He was admitted because of anorexia, weight loss, and a low-grade fever. The ultrasound study showed bilateral hydroureteronephrosis, with a poorly functioning right kidney noted on intravenous urography, and a retrovesical soft-tissue mass. Laboratory studies showed renal insufficiency, an elevated white blood cell count, anemia, thrombocytosis, and hypergammaglobulinemia. During laparotomy, a pelvic appendiceal mass was discovered, appendectomy was performed, and the pathological diagnosis of inflammatory myofibroblastic tumor was made. Three months later the boy was well, and the biological, sonographic, and urographic results had returned to normal. A review of 74 cases of abdominal IMT occurring in childhood emphasizes the importance of pathological differentiation of these lesions from malignancy, with early total excision whenever possible. Long-term follow-up is necessary to detect local recurrence, which may develop many years later, and to confirm the allegedly benign nature of these tumors.
Adolescent↗
[Unusual relapse for neuroblastoma. Case of an intracranial relapse].
The usual sites of initial metastatic deposits in neuroblastoma are osteo-medullary. With modern therapies including megatherapy and hematopoietic rescue, prolonged survival is obtained. However, unusual metastatic sites are more and more often described during the prolonged evolution of these patients such as brain metastases. A case of isolated intracerebral metastatic relapse is reported here in a patient who had received 4 months before a megatherapy in first complete remission. Pathogeny and therapeutical implications are discussed.
Adrenal Gland Neoplasms↗
[Current treatment of hypospadias].
The aim of this work was to discuss general ideas concerning the factors which should be taken into account for the treatment of hypospadia including: anatomy, preoperative work-up, analysis of the anatomic type, patient status, search for an underlying more complex malformation. Precise indications for per and post-operative care should provide good results. Two major ideas predomination in these recommendations: operate early (at about 2 years) in a single procedure for both anatomic and psychological reasons. This latter point is particularly important. Operative technique is illustrated and described together with complications and their management.
Humans↗
[A recto-sigmoid lesion due to trans-anal aspiration. Apropos of a case].
A three year old boy presented a transanal evisceration of the rectum after sitting over a non covered drain site of a swimming pool. Peritonitis led to laparatomy 24 hours later. The high rectum was found to be necrotic and perforated. A protecting sigmoïd loop colostomy was performed with out any rectocolic resection. Five months later, resection of the fibrosed high rectum and end to end anastomosis between sigmoïd and low rectum was done. The colorectal function was quickly restored. Continence is now normal with a follow-up of 18 months. Pathogenesis of transanal suction may be as follows: relaxation of perineal muscles and anal sphincter during sitting position permit transanal evisceration by suction. Such lesions are rare (less than 10 cases reported). The therapeutic choice, between initial reparation and drainage associated, with protecting colostomy is discussed.
Anal Canal↗
[Acute intestinal intussusception: ultrasonic diagnosis and air insufflation].
Sixty-five children with intussusception were diagnosed by ultrasonography. Colonic air insufflation was the principle form of treatment. The diagnosis value of ultrasound was 100% and the success rate of reduction by air insufflation was 93%, after intrarectal injection of midazolam (0.3 mg/kg). The device for insufflation was simple, original and manual, with permanent control of intrarectal pressure. No complications were observed.
Acute Disease↗
[Pygomelus. A single monster or a double monster?].
With an anatomical case and a clinical case, the authors describe the pygomelus, a very rare monstruosity. Legend and reality are often mixed in the description of literature. It is difficult to classify this monstruosity either in the simple monster or in the twin monsters.
Abnormalities, Severe Teratoid↗