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Y Aigrain

Publications and source records attributed to Y Aigrain.

At least 55 records · Page 3Linked to original sources
PubMed2000

Thoracic pheochromocytoma revealed by ventricular tachycardia. Clinical case and review of the literature.

A case of thoracic extra-adrenal pheochromocytoma was observed in a 13-year-old girl. The initial clinical symptoms consisted of her being unwell after effort over a period of 3 years. The diagnosis was suggested after a recorded episode of paroxysmal ventricular tachycardia with concomitant hypertension, which reproduced the characteristic symptoms. Biochemical analysis confirmed hypersecretion of catecholamines and morphological examinations showed the presence of an isolated left median thoracic tumor. One year after surgical excision, the patient remained completely asymptomatic, in spite of resuming intense sporting activity. The diagnosis of ectopic pheochromocytoma should be considered as a possible etiology of cardiac rhythm disturbances or hypertension in children. Thorough biochemical and radiological examination including MIBG radioisotope scanning should be carried out systematically before surgical excision; pheochromocytomas in children are frequently extra-adrenal (30%), multifocal (50%) or associated with multiple endocrine neoplasms (NEM).

Adolescent↗
PubMed2000

How accurate is the prenatal diagnosis of abnormal genitalia?

PURPOSE: The prenatal diagnosis of abnormal genitalia may have a major impact on prenatal counseling and postnatal outcome. We studied the accuracy and clinical implications of the prenatal diagnosis of abnormal genitalia. MATERIALS AND METHODS: Between 1991 and 1999 the prenatal and/or postnatal diagnosis of abnormal genitalia in 53 cases was made at our institution. All cases were prenatally assessed at our Obstetrics and Fetal Medicine Department. Outcome was confirmed postnatally or by a fetopathologist in the case of pregnancy termination. RESULTS: A genital anomaly was prenatally diagnosed in 43 cases and was accurate in 34, while in 9 cases anomalies were absent at birth. In 10 cases ambiguous genitalia were not detected prenatally. The primary anomalies suspected were male pseudohermaphroditism in 19 cases and female pseudohermaphroditism in 12, including 2 cases of congenital adrenal hyperplasia. Male pseudohermaphroditism was detected prenatally in 17 cases and diagnosis was confirmed at birth. Female pseudohermaphroditism was detected prenatally in 12 cases and only 5 were confirmed and the anomaly was discovered at birth in 6. The prognosis was highly altered when many malformations or aneuploidy was associated with ambiguous genitalia. Of the 15 patients with many malformations only 3 survived, and pregnancy was terminated in 3 of 4 cases of aneuploidy. CONCLUSIONS: When pseudohermaphroditism was detected in a male fetus by an experienced ultrasonographer at a tertiary center the prenatal diagnosis was accurate in 100% of cases. The prenatal diagnosis was less accurate (46% correct) in a female fetus.

Disorders of Sex Development↗
PubMed2000

Laparoscopic retroperitoneal nephrectomy in high risk children.

PURPOSE: Nephrectomy may be indicated in children with end stage renal disease before transplantation. We studied the feasibility and results of nephrectomy performed via a retroperitoneal laparoscopic approach in these high risk children. MATERIALS AND METHODS: We performed 12 nephrectomies in 9 children with end stage renal disease and a mean age of 7 years (range 7 months to 13 years) through a 3 trocar retroperitoneal laparoscopic approach. Cases were classified as American Society of Anesthesiologists grade III and presented with end stage renal disease, hypertension, thrombocytopenia and/or the nephrotic syndrome. The renal artery and vein were ligated separately with endocorporeal knots and clips. Mean size of the kidney was 8 cm. (range 5 to 12). Bilateral nephrectomy was performed simultaneously in 2 patients 7 and 12 months old, respectively. Cardiorespiratory changes related to retroperitoneal gas insufflation were assessed prospectively. To compare laparoscopic versus open nephrectomy we retrospectively analyzed the data of 12 open nephrectomies performed in 9 children with similar nephrological indications. RESULTS: The procedure was feasible in all cases without conversion to open surgery, and no intraoperative incident occurred. Mean operative time of laparoscopic nephrectomy was 2 hours (range 1 hour 20 minutes to 3 hours 10 minutes). After retroperitoneal carbon dioxide insufflation systolic arterial pressure and end-tidal carbon dioxide were significantly increased without the need for specific measure to correct these modifications. Hemodialysis began 1 day postoperatively and feeding began 2 days postoperatively. Mean hospital stay was 5.2 days (range 3 to 7). The comparative study of the open nephrectomy group showed no significant difference in mean operating time (p = 0.07), and hospital stay was significantly shorter for the laparoscopic group (p <0.001). CONCLUSIONS: Retroperitoneal laparoscopic nephrectomy is safe and feasible for high risk children. The relatively long operating time is necessary for hemostasis in these children at risk for hemorrhagic complications.

Adolescent↗
PubMed2000

ET(A)-receptor blockade and ET(B)-receptor stimulation in experimental congenital diaphragmatic hernia.

The aim of this study was to assess the role of nitric oxide (NO) and endothelin (ET)-1 in the pathophysiology of persistent pulmonary hypertension of the newborn in fetal lambs with a surgically created congenital diaphragmatic hernia (CDH). The pulmonary vascular response to various agonists and antagonists was assessed in vivo between 128 and 132 days gestation. Age-matched fetal lambs served as control animals. Control and CDH lambs had similar pulmonary vasodilator responses to acetylcholine, sodium nitroprusside, zaprinast, and dipyridamole. The ET(A)-receptor antagonist BQ-123 caused a significantly greater pulmonary vasodilatation in CDH than in control animals. The ET(B)-receptor agonist sarafotoxin 6c induced a biphasic response, with a sustained pulmonary vasoconstriction after a transient pulmonary vasodilatation that was not seen in CDH animals. We conclude that the NO signaling pathway in vivo is intact in experimental CDH. In contrast, ET(A)-receptor blockade and ET(B)-receptor stimulation significantly differed in CDH animals compared with control animals. Imbalance of ET-1-receptor activation favoring pulmonary vasoconstriction rather than altered NO-mediated pulmonary vasodilatation is likely to account for persistent pulmonary hypertension of the newborn in fetal lambs with a surgically created CDH.

Acetylcholine↗
PubMed1999

Laparoscopic Duhamel procedure. Management of 30 cases.

BACKGROUND: Between February 1995 and June 1998, 30 laparoscopic Duhamel pull-through procedures were performed in our department. METHODS: Our main aim was to prove the feasibility of the laparoscopic abdominal Duhamel procedure for different localizations of Hirschsprung disease. We used one camera port and three working ports. The sigmoid colon and posterior rectum were mobilized laparoscopically. A standard posterior colo-anal anastomosis was fashioned and a stapler was used for the anterior anastomosis. The top of the rectum was then closed by endo stapler under laparoscopic vision. RESULTS: Thirty patients underwent laparoscopic surgery for this procedure. Three laparoscopic procedures were converted because of technical difficulties. The operative time was 100-330 mn. Oral feeding was started at a mean postoperative time of 2.5 days. Mean postoperative hospitalization was 9 days. Early postoperative complications included 1 anastomotic leak, 1 retrorectal abscess, 2 urinary infections, and 1 evisceration (after conversion). No enterocolitis or enterocolitis-like symptoms were noted. All patients now have daily spontaneous bowel movements. CONCLUSION: The laparoscopic Duhamel procedure can be performed safely, giving good results.

Anastomosis, Surgical↗
PubMed1999

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↗
PubMed1999

Up-to-date evolution of small bowel transplantation in children with intestinal failure.

PURPOSE: The aim of the authors was to report an up-to-date review of their experience with 26 intestinal transplantations in children since 1987. METHODS: A retrospective study was conducted of 26 patients with a mean age of 5 years (range, 0.3 to 14 years). Three groups were isolated. In group A (1987 to 1990), seven patients received nine isolated intestinal transplants for short bowel syndrome. Immunosuppression therapy consisted of cyclosporine, aziathioprine, and corticosteroids. In group B (1994-current), nine patients received nine isolated intestinal transplants for short bowel syndrom (n = 2), intestinal pseudoobstruction (n = 2), neonatal intractable diarrhea (n = 3), and Hirschsprung' disease (n = 1); hepatic biopsy results showed weak cholestasis or fibrosis. In group C (1994-current), 10 patients received 10 combined liver-small bowel transplants for short bowel syndrome (n = 3), neonatal intractable diarrhea (n = 4), and Hirschsprung' disease (n = 3); hepatic cirrhosis related to total parenteral nutrition (TPN) was shown in all cases. Groups B and C received immunosupressive treatment consisting of tacrolimus, aziathioprine, and corticosteroids. Posttransplant follow-up included intestinal biopsies of the small bowel twice a week and more frequently or combined with liver biopsy if rejection was suspected. RESULTS: Overall patient survival (PS) and graft survival (GS) are 61% (16 of 26) and 50% (13 of 26), respectively. In group A, severe intestinal allograft rejection occurred in six patients leading to graft removal (GS, 11%). Five patients died of TPN complications after graft removal (PS, 28%). One survivor is off TPN, and one currently is waiting for a second graft. In group B, six patients survived (PS, 66%). Causes of death include hepatic failure (n = 1), renal and liver failure (n = 1), and systemic infection (n = 1). Severe intestinal allograft rejection occurred in five patients, which neccessitated aggressive immunosuppression (antilymphocyte serum) leading to an incomplete functional recovery of the graft. Only two patients currently are off TPN. In group C, eight patients survived (PS, 80%) all of which are currently off TPN. One patient died during the procedure, and one died of severe systemic infection. Intestinal graft rejection occurred in six patients; rejection of the liver allograft occurred in five patients, yet all rejections were weak and successfully treated by corticosteroids (GS, 80%). CONCLUSIONS: Intestinal transplantation is a valid therapeutic option for children with definitive intestinal failure and not only for short bowel syndrome. Tacrolimus improves graft and patient survival (group A v group B). The lower severity of graft rejection in combined liver-small bowel transplantation improves functional results of intestinal transplantation in children without additional mortality or morbidity (group B vgroup C).

Adolescent↗
PubMed1999

Cystography after the Cohen ureterovesical reimplantation: is it necessary at a training center?

PURPOSE: Reimplantation by the Cohen procedure has a low rate of recurrent reflux, although postoperative cystography is done routinely at most centers. According to the French training program for pediatric surgery and urology residents, reimplantation is the main pediatric urology procedure performed during residency. We determine whether it is necessary to perform postoperative cystography routinely and whether the fact that the procedure is done by a junior surgeon modifies management. MATERIALS AND METHODS: A total of 268 children with primary vesicoureteral reflux underwent ureteral reimplantation by the Cohen transtrigonal technique. Bilateral reimplantation was done in 97% of the cases. Reimplantation was performed by a surgery resident assisted by a clinical fellow or senior consultant surgeon in 37% of the cases. Routine cystography and renal ultrasound were done in all patients postoperatively. Followup ranged from 6 months to 5 years (mean 10 months). RESULTS: In 2 children (0.7%) with recurrent reflux surgery was not performed by a resident. One of the 2 children had asymptomatic persistent reflux and no further surgery was done. In the other child postoperative cystography was normal at 6 months. One year later she had acute pyelonephritis with recurrent unilateral reflux and underwent repeat reimplantation. CONCLUSIONS: Routine cystography is not necessary after bilateral Cohen reimplantation. Reflux recurrence is low even at a training center where surgery may be performed by junior surgeons.

Child↗
PubMed1999

Outcome of posterior urethral valves: to what extent is it improved by prenatal diagnosis?

PURPOSE: To assess the impact of prenatal diagnosis and evaluation on the outcome of posterior urethral valves we studied all cases of valves detected prenatally, including cases of pregnancy termination due to posterior urethral valves. MATERIALS AND METHODS: Between 1989 and 1996, 30 neonates with prenatally detected posterior urethral valves were treated at our hospital. The prenatal parameters analyzed were age of gestation at diagnosis, ultrasonographic appearance of renal parenchyma and amniotic fluid volume. Fetal urine was analyzed in 9 cases. We reviewed the outcome of 10 neonates treated for posterior urethral valves which were not diagnosed prenatally during the same period. RESULTS: Of the 30 neonatal survivors 6 (20%) had renal failure, including end stage renal disease in 2, after a mean followup of 4 years. Renal failure developed in 2 of 5 cases detected before 24 weeks of gestation, in 1 of 6 with oligohydramnios and in 2 of 5 with abnormal parenchymal renal ultrasound. Normal parenchymal ultrasound and amniotic volume could not predict for good outcome. Renal failure developed in 2 of 7 cases predicted by fetal urinalysis as good prognosis and in 1 of 2 cases predicted as poor prognosis. Pregnancy was terminated for posterior urethral valves in 5 cases based on prenatal criteria of severe renal impairment. Considering these cases as poor outcome, the rate of poor prognosis increased from 20 to 31%. Among the 10 neonates without a prenatal diagnosis of posterior urethral valves renal failure developed in 2 (20%), including end stage renal disease in 1. CONCLUSIONS: When negative parameters were absent and/or fetal urine predicted good outcome there were no cases of end stage renal disease in early infancy, which was a significant help in parent counseling. The predictive value of the currently available prenatal parameters needs to be updated with larger series specifically dealing with posterior urethral valves. According to the current data, the outcome of posterior urethral valves is not yet significantly improved by prenatal diagnosis.

Female↗
PubMed1999

[Posterior approach to the bladder for implantation of the 800 AMS artificial sphincter in children and adolescents: techniques and results in eight patients].

Blind dissection of the bladder neck during implantation of an artificial urinary sphincter (AMS 800) may lead to injury of either the bladder, genital tract or even the rectum. Significant bleeding may occur. A posterior approach to the bladder neck allowing visual control of the anatomical structures is described. From November 1995 to February 1998, 8 adolescents (12-19 years old) underwent AMS 800 artificial urinary sphincter implantation for the treatment of severe incontinence associated with neurogen bladder; one patient had had a previous augmentation. Three had a simultaneous ileocystoplasty and another patient had a simultaneous bilateral extravesical ureteric reimplant. The procedure consisted of separation of the bladder from the peritoneum, allowing the development of a dissection plane between the rectum and genital tracts posteriorly and the ureters and bladder neck anteriorly; the dissection is extended to the base of the prostate. The endopelvic fascia is then incised laterally and on both sides the neurovascular bundles are perforated under visual control and the cuff is positioned safely around the bladder neck, above the prostate and in front of the genital tract. The bladder was opened only in the case of associated ileocystoplasty, thus avoiding prolonged bladder drainage. The mean operating time was 2.5 hours and the blood loss never exceeded 300 cc. This route was not found to be convenient in the case of the patient with a previous augmentation; 7 sphincters function normally with a follow-up of 18 to 44 months; one was never activated and the patient is dry under CIC. This route for exposure of the bladder neck allows visual control of the anatomical structures, accurate positioning of the cuff, avoids bladder opening and reduces bleeding. It can be used for other procedures such as bladder neck suspension or Müllerian cavity removal.

Adolescent↗
PubMed1999

[Congenital malformations of the abdominal wall].

Congenital malformations of the abdominal wall consist of a combination of a parietal opening and visceral abnormalities. The most frequent are omphalocele and laparoschisis. Embryological analysis of the formation of the abdominal wall distinguishes omphalocele, due to absence of differentiation of the lateral folds of the embryo, from laparoschisis, due to absence of differentiation of the mesenchyma at one point of the somatopleural lining. Clinically, omphalocele consists of an opening of the abdominal wall, lined by a double layer (peritoneum and external layer of the amniotic membrane), which may rupture before birth. Laparoschisis is a true antenatal hernia lying to one side of the umbilical cord. The other abnormalities of the abdominal wall are less common: muscle and purely cutaneous aplasia. The treatment of omphaloceles requires multidisplinary management. Several surgical techniques are used, mainly primary closure and rectomyoplasty. Primary closure of the abdominal wall is only possible in one-half or one-third of cases.

Abdominal Muscles↗
PubMed1998

[Bulky superinfected tinea capitis of the scalp. Treatment by surgical resection and reconstruction by cutaneous expansion].

BACKGROUND: Tinea capitis are usually cured by medical treatment. We present an unusual case which required a surgical treatment. CASE REPORT: A bulky infected kerion ignored for several weeks extended to a large part of the scalp of a 4-year-old girl. Antibiotics and antifungal agents were ineffective so that a total resection was necessary. The loss of substance was covered by a cutaneous graft; the large alopecia was secondarily repaired by a cutaneous expansion. Evolution has been followed for the past 10 years. CONCLUSION: It is exceptional that surgical treatment should be required for this condition.

Alopecia↗
PubMed1998

[Iterative intestinal intussusception and appendiceal mucocele in an infant with mucoviscidosis].

UNLABELLED: Cystic fibrosis is a common and potentially life-threatening hereditary disease which can affect numerous organs, particularly the digestive tract. CASE REPORT: A 4.5-year-old boy exhibited two little known clinical manifestations: an appendiceal mucocele and repeated intussusceptions. In spite of an appendectomy, intussusception relapsed and an ileocolic resection was necessary 2 years later. DISCUSSION: Appendiceal diseases in cystic fibrosis represent a large spectrum, ie, distention on the appendiceal lumen, engorged with sticky mucous matter, which becomes an appendiceal mucocele, peritonitis with an appendiceal perforation due to delayed diagnosis since acute appendicitis is difficult to diagnose in these patients. Intussusception is rarely observed in cystic fibrosis. CONCLUSION: Appendiceal mucocele could be a cause of intussusception. If an appendectomy is performed, resection of a part of the cecum, around the appendix, could be useful in preventing again mucocele formation.

Appendectomy↗
PubMed1998

[Left aortic arch--right descending aorta--right ductus arteriosus (encircling aortic arch). A rare malformation of the aortic arches].

UNLABELLED: Abnormalities of the aortic arch which are responsible for tracheobronchial compression are well known. This case demonstrates the value of magnetic resonance imaging (MRI) for diagnosis and suggests that recurrent respiratory symptoms should evoke these abnormalities in infancy. CASE REPORT: The authors report a very rare malformation of the aortic arch formed by encircling aortic arch, with left aortic arch, right descending aorta and right ligamentum arteriosum. It was revealed by airway disorders due to the compression of tracheobronchial axis by the ligamentum arteriosum. Section of the ligamentum permitted suppression of obstruction. The diagnosis of these abnormalities is usually established by means of oesophagogram, tracheobronchial endoscopy, angiography and MRI. CONCLUSION: In this case, MRI gave a better picture than angiography. Sections of the ligamentum arteriosum are sometimes ineffective when compression is due to the aorta itself. Aortic uncrossing, a more complex operation, is then necessary.

Abnormalities, Multiple↗
PubMed1998

[Hypoplasia of the penis: etiologic diagnosis and results of treatment with delayed-action testosterone].

BACKGROUND: Results of long-term testosterone treatment of congenital hypoplasia of the penis are sparse; the aim of this work was to evaluate these results according to age at onset of treatment, the presence or absence of hypospadias, and its eventual adverse effect upon growth and bone maturation. PATIENTS AND METHODS: Sixty-six children with congenital hypoplasia of the penis, isolated (n = 31) or associated with a hypospadias (n = 35), were evaluated between 1 day and 16 years of age. The prevalence (40% of the cases) of the different malformations and/or syndromes associated with congenital hypoplasia and its severity were not different whether the penis was isolated or associated with hypospadias (male-pseudohermaphroditism). The effect of the testosterone heptylate (IM) was evaluated in 40 children treated before 10 years of age. RESULTS: The penis' increase in length was more important in the children with isolated micropenis (+2.1 +/- 0.8 SDS) than those with hypospadias (+1.3 +/- 1.2 SDS) but, in all cases, its increase was better when the treatment was started during the neonatal period, which corresponded to those with the most severe forms of micropenis. A mean regression of the penile volume of -0.7 +/- 0.8 SDS was noticed after the end of each stimulation. No long-term secondary effects related to treatment were seen on later growth and bone-maturation. CONCLUSION: Penile length at the last follow-up was not associated with total dose of testosterone but with its length at the first evaluation time (r = 0.52; P = 0.002).

Adolescent↗