PubMed Health⌕ Search

Biomedical subjects

B Bachy

Publications and source records attributed to B Bachy.

At least 19 recordsLinked to original sources

Secondary localisation of an intra-thoracic benign mesenchymoma in the fossa poplitea: a rare paediatric case.

Thymomas are tumours that rarely occur in children, are almost invariably benign, and are usually discovered incidentally in the anterior mediastinum on chest X-rays. Whereas in adults these tumours are often associated with myasthenia gravis and other autoimmune diseases, this occurrence is very rare in the paediatric population. Multiple localisation and/or extra-thoracic recurrence of thymomas in children also appears to be exceptional with no reported cases in the English literature. We report one rare paediatric case.

Adolescent↗

[Early spontaneous ileal perforations in preterm infants: report of 4 cases].

An ileal perforation occurred shortly after birth in 4 very premature newborns. Diagnosis was made on an abdominal distension with a pneumoperitoneum on X-ray. There were no biological, radiological nor histological signs of necrotizing enterocolitis. There were no digestive short- or long-term complications. According to the few authors who described this syndrome, there are some risk factors, but they were not clearly involved in our cases. Ileal perforation in the absence of signs of necrotizing enterocolitis is rarely reported but should be well known because of its good prognosis.

Diagnosis, Differential↗

[Laparoscopic surgery for undescended testicles].

PURPOSE: Impalpable and undescended testis is a very common preoccupation for specialized pediatric surgery teams. The laparoscopy as the single most accurate modality for diagnosis and localization of impalpable undescended gonad is well established by all authors. However the therapeutic attitude and the technique are still a matter of debate. MATERIAL AND METHODS: A series of 122 boys presenting an impalpable and undescended testis was studied. All the children beneficiated of a primary laparoscopic exploration. During this exploration, when a testis was found, the clamping of the spermatic vessel was made and the final descent took place few months later. RESULTS: In our study the testis was found in 60 cases and the spermatic vessel's clamping assured the descent of a well vascularized testis in about 80% of cases. CONCLUSION: Clamping the spermatic vessel during the laparoscopic exploration assured, thanks to the development of a supply, a good vascularisation of the testis during its second step descent. Our retrospective study has showed the good reliability of laparoscopic surgery as primary treatment in front of impalpable undescended testicles in comparison to the one-step classical orchiopexy.

Adolescent↗

[Management of cleft lip and palate in university hospital of Rouen].

Cleft lips and cleft palates are managed in the department of Pediatric surgery in Rouen for the last 30 years. From the antenatal diagnosis, the parents got in touch with the surgeon who will coordinate this management. Around thirty new patients are treated every year. The chronology of the treatment is of "classic" manner. The cleft lip is repaired at about 3 weeks of age and the palatoplasty is performed after the age of 1 year. In view to maintain the intimacy of the consultation we did not institute multidisciplinary consultations. The other members of the interdisciplinary team will intervene during the follow up depending on the form of the cleft and the encountered problems. The information and the files circulate freely and are discussed together.

Academic Medical Centers↗

Type 1-primary cutaneous meningioma of the scalp.

Type 1-primary cutaneous meningioma is a rare and often clinically unsuspected lesion of the scalp, forehead or paravertebral region which occurs at birth or during childhood. The pathogenesis of these lesions still remains uncertain. Several authors have emphasized that type 1-cutaneous meningiomas are not real tumors but sequestrated meningoceles or heterotopic meningeal nodules of the skin. Nevertheless, the search for an intracranial or intravertebral connection should be carried out. We describe the clinical and pathological features of a congenital type 1-meningioma of the vertex. No cranial defect or intracranial tumor was found. However, the cutaneous lesion was directly linked to a large cranial vein. The purpose of this study was to illustrate this rare lesion and to indicate the possible surgical risks and the pathological characteristics.

Child↗

Epididymitis in children: is further investigation necessary after the first episode?

OBJECTIVE: The aim of this study was to evaluate the usefulness of investigating underlying urinary tract pathology after the first episode of acute epididymitis in children with no prior urological history, and also to assess the possible predictive factors of urological disease at the time of diagnosis. METHODS: Children with acute epididymitis were studied retrospectively over a period of 8 years. Diagnosis was made either using ultrasonography or surgical exploration. Renal ultrasonography and voiding cystourethrography were performed 1 month after epididymitis. RESULTS: These tests were carried out in 38 children who revealed 7 anomalies (18%). Only 1 patient received further surgery : endoscopic treatment of a ureterocele. We also studied 3 predictive factors: age <2 years; recurrence, and urine bacteria. None could be associated with the presence of urinary tract pathology. CONCLUSION: The authors suggest that, when there is no previous urological anomaly and absence of bacteriuria, routine screening for epididymitis should be carried out following the second episode.

Acute Disease↗

[Intestinal volvulus after enterocystoplasty: report of three cases in children].

Enterocystoplasty complications related to the presence of intestinal mucous presence in the bladder are well known and well reported in the literature. Mechanical problems in the intestinal tract due to using gut for bladder augmentation are not as well recognised. We analyzed three cases of children treated by ileocystoplasty who presented an intestinal volvulus in two cases and a cecal volvulus in one case around the vascular pedicle of the intestinal patch used for bladder augmentation.

Adolescent↗

Tracheobronchial ruptures from blunt thoracic trauma in children.

BACKGROUND/PURPOSE: Tracheobronchial ruptures in blunt thoracic trauma in children are rare. The aim of this study was to suggest the means of an early diagnosis and a conservative management as often as possible. METHODS: Sixteen cases of tracheobronchial ruptures by blunt thoracic trauma were observed over 26 years in 9 regional pediatric centers. RESULTS: There were 12 boys and 4 girls, from ages 1 hour to 17 years. Nine children presented with associated lesions. Fibroscopy established the following diagnosis: 8 tracheal wounds and 8 bronchial wounds. Six children were operated on within 18 hours (on average) after installation of a thoracic drainage. Two lobectomies, 3 ideal tracheal sutures, and 1 bronchial suture were performed. Seven children were treated exclusively by thoracic drainage. Two of them were intubated through the lesion, leading to a transitory endoprothesis accompanied or not by an external thoracic drainage. One infant recovered spontaneously. There were no deaths in this series. Two recurrent postoperative nerve injuries were noted, one of which was a transitory spontaneously resolutive scar bud and one a granuloma treated by laser. Three times, a stenosis occurred after a conservative management. Two were operated on. CONCLUSIONS: Tracheobronchial ruptures in children are rare. An early fibroscopy holds an important place in the approach of this pathology. Treatment is variable, based on thoracic lesions, their tolerance by the child, and associated lesions. Surgery is not the only therapy because conservative treatment by simple thoracic drainage or lesion intubation has proved effective.

Adolescent↗

Results of the Gil Vernet procedure in preventing contralateral reflux in unilateral ureteric reflux.

OBJECTIVE: To evaluate whether contralateral meatal advancement based on the technique described by Gil Vernet decreases the risk of postoperative contralateral reflux, which may occur after a unilateral reimplantation. PATIENTS AND METHODS: From January 1986 to 1997, 321 reimplantations were performed for unilateral vesico-ureteric reflux (VUR) using the Cohen procedure. In cases where the contralateral meatus was symmetrical or had a pathological appearance, preventive contralateral surgery with meatal advancement was performed. RESULTS: Ureteric reimplantation was exclusively performed unilaterally in 254 patients and in 67 a contralateral meatal advancement was performed. There were 29 cases of contralateral reflux at the 4-month follow-up. In nine patients contralateral reimplantation was necessary for persistent symptomatic VUR, the reflux resolved spontaneously in 14 and a radiological examination was necessary in six. Reflux also appeared on the Gil Vernet side in only 6% of patients; there were no clinical symptoms and the outcome was favourable. CONCLUSION: The advancement of the meatus using the Gil Vernet procedure is simple, with no surgical complications. We suggest that this technique constitutes a useful surgical alternative in the prevention of contralateral reflux.

Child↗

[Extrarenal nodular nephrogenic blastema in the inguinal canal: report of two cases].

Two observations of extrarenal nephrogenic nodule in inguinal canal are reported. We underline the difficulties in differential diagnostic with true Wilms' tumor, and in sparing these pediatric cases from more aggressive treatment. The main morphological arguments in favor of non-tumoral nephrogenic nodular remnants are: the size of the lesion, the association with a congenital inguinal hernia suggesting a congenital abnormality, and the organoid organization of blastemic tissue surrounding the cavities lined with "hobnail" epithelium evoking a mesonephrogenic origin.

Abdominal Neoplasms↗

[US-guided percutaneous drainage of an infected epidermoid cyst of the spleen in a child].

Epidermoid cyst of the spleen is a rare entity (2.5% of all splenic cysts) and rarely becomes complicated by hemorrhage, rupture or infection. Classically, management consisted of total or partial splenectomy. We report the case of an 8-year-old boy presenting with a splenic abscess complicating an epidermoid cyst. Percutaneous drainage of the abscess was performed under sonographic guidance and completed by intravenous antibiotic therapy. Six weeks later, laparoscopic surgery was performed and the splenic parenchyma could be preserved.

Abscess↗

[Focal dermal hypoplasia: description of three cases].

BACKGROUND: Focal dermal hypoplasia syndrome is mainly defined by the association of abnormalities of extremities, atrophy and linear hyperpigmentation of the skin, localized deposits of superficial fat, anomalies of the eyes and of the nails. Neonates are often small for their age. CASE REPORTS: Three sporadic cases are reported. Mental delay and omphalocele were observed in the first case. The neurological development was subnormal in the second and an unusual monodactyly was seen in the third. CONCLUSION: Most cases are sporadic, but in family cases, an X-linked dominant factor is likely. When a first affected offspring is observed, skin examination and X-ray should be carried out in parents to evaluate the risk of recurrence in their children. As the gene site has not yet been determined, antenatal diagnosis should be suspected on echography when fetal growth delay is associated to distal limb and/or ocular anomalies.

Child↗

Mutation of the RET ligand, neurturin, supports multigenic inheritance in Hirschsprung disease.

Hirschsprung disease (HSCR) is a frequent neurocristopathy characterized by the absence of submucosal and myenteric plexuses in a variable length of the gastrointestinal tract. Pedigrees and segregation analyses suggested the involvement of one or several dominant genes with low penetrance in HSCR. Considering that RET and glial cell line-derived neurotrophic factor (GDNF) mutations have been reported in the disease, we regarded the other RET ligand, neurturin (NTN), as an attractive candidate gene, especially as it shares large homologies with GDNF. Here, we report on the finding of a heterozygous missense NTN mutation in a large non-consanguineous family including four children affected with a severe aganglionosis phenotype extending up to the small intestine. Interestingly, it appears that the NTN mutation reported here is not sufficient to cause HSCR, and this multiplex family also segregates a RET mutation. This cascade of independent and additive genetic events fits well with the multigenic pattern of inheritance expected in HSCR, and further support the role of RET ligands in development of the enteric nervous system.

Amino Acid Sequence↗

[The value of examination and treatment using laparoscopy in non-palpable testes: apropos of a series of 48 cases].

OBJECTIVES: Laparoscopy now constitutes the reference technique for the diagnosis and treatment of cryptorchid testes. We report our experience over the last three years (1993-1996). MATERIAL AND METHODS: 48 strictly impalable testes were investigated in 46 boys between the ages of 11 months and 14.5 years (mean age: 40 months). The intraperitoneal investigation assessed both deep inguinal regions looking for gonads, vas deferens and pedicles. Intra-abdominal gonads were ligated and their pedicle was sectioned laparoscopically allowing transinguinal descent 6 months later according to the Fowler-Stephens technique. RESULTS: We found 21 cases of typical antenatal torsion, including one bilateral case (pedicle and vas deferens present, but gonad absent), one case of total unilateral agenesis and 3 cases of incomplete agenesis (only the vas deferens was detected) and performed three resections of the gonadal rest for histological examination. The first-stage of cryptorchid testis descent was performed in 20 cases, by laparoscopy in 19 cases (1 failure of insufflation). Definitive descent was possible in 13 cases, with early onset of atrophy in only one case. CONCLUSION: Laparoscopy is therefore a simple technique, allowing a definitive diagnosis and two-stage descent without increasing the risk of testicular atrophy.

Adolescent↗

Idiopathic varicocele in children and adolescents--which therapeutic choice?

61 children were studied and treated between January 1986 and September 1993 for idiopathic varicocele. The aim of our study was to evaluate the advantages and disadvantages of the different techniques and to show the progression to a greater efficacy. The mean age at the time of therapy was 14 years, ranging from 7 to 16 years. All children presenting with pain or testicular asymmetry were treated. Four asymptomatic children were followed for 2 years before treatment. 36 children were treated by surgical ligature via the inguinal approach; 8 with a resection of the varicose veins as far as the tunica vaginalis. 14 children were treated by percutaneous sclerotherapy including 1 patient following unsuccessful classical surgical treatment. 12 children were treated by surgical inguinal ligature associated with peroperative phlebography and thrombosis. 56 children were reviewed postoperatively over a period which varied from 2 months to 4 years (5 lost to follow-up). For the 36 classical ligatures: 25 good results, but 9 hydroceles (5 out 8 varicose resections): 70% good results. 7 failures and 4 lost to follow-up. For the 14 percutaneous sclerotherapy: 4 technical failures (impossibility to catheterize the spermatic vein): 10 good results. For the 12 ligatures with peroperative phlebography and thrombosis: 11 good results and 1 lost to follow-up. All the above procedures were carried out at our out-patient clinic. The therapeutic choice will therefore have to take into consideration a procedure which produces the lowest morbidity rate and proves to be the most effective. The association of surgical ligature, phlebography and thrombosis meets these requirements.

Adolescent↗

[Mesenteric lipoblastoma with changes in chromosome 8: use of cytogenetics in the diagnosis of adipocytic tumors in children].

Lipoblastoma is a rare type of benign tumor occurring in infants. We report a case of mesenteric lipoblastoma with histologic, electron microscopic and cytogenetic studies. The microscopic features of this tumor including lipoblastic proliferation and prominent immature capillary beds were typical of lipoblastoma. Cytogenetic study showed a karyotype 46,XX, inv (8) (p 21.1; q 24.2). We discuss the usefulness of cytogenetic study associated to fluorescent in situ hybridization, in the diagnosis of the lipoblastic tumors, i.e. myoxoid liposarcoma and lipoblastoma.

Chromosome Aberrations↗