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Biomedical subjects

J M Guys

Publications and source records attributed to J M Guys.

At least 19 recordsLinked to original sources
PubMed2006

Quality of life and continence in patients with spina bifida.

PURPOSE: Spina bifida (SB) is the most common congenital cause of incontinence in childhood. This study attempts to determine the relationships between urinary/faecal incontinence, methods of management, and Health Related Quality of Life (HRQoL) in people with SB. PATIENTS AND METHOD: A total of 460 patients (300 adults and 160 adolescents) from six centres in France have taken part in this cross-sectional study. Clinical outcome measures included walking ability, urinary/faecal continence, and medical management. HRQoL was assessed using the SF36 in adults and the VSP in adolescents and their parents. Univariate and multivariate analysis was used to determine the relationships between clinical parameters and HRQoL. RESULTS: HRQoL were significantly lower than in the general population. Adult women had significantly lower scores than men, and adolescent females had significantly lower scores for psychological well being. We did not found strong relationship between incontinence and HRQoL in this population. Moreover patients surgically managed for urinary/fecal incontinence did not show significantly higher scores of HRQoL. CONCLUSION: Using generic HRQoL measures, urinary/faecal incontinence and their medical management may not play a determinant role in HRQoL of persons with SB. However many other factors affect HRQoL in these patients. A longitudinal study design is recommended to assess whether incontinence management is associated with improved HRQoL.

Adolescent↗
PubMed2006

[Neurogenic bladder in children: basic principles in diagnosis and treatment].

The diagnosis of neurogenic bladder can be easy in myelomeningocele and much more difficult in occult dysraphia or medical etiologies. Careful clinical examinations and urodynamic investigations are mandatory for the diagnosis and the follow up of affected patients. Clinico-anatomical correlations are poor. If urinary leak is the first apparent symptom, preservation of the upper urinary tract is the main goal of the surgeon. If natural history of the neurogenic bladder is destruction of the detrusor and paralysis of the trigona, obstructive uropathy is the main physiological concern. Urinary leak must be integrated in the global context of the bladder function in order to determine urinary incontinence type. Ideal micturition is voluntary, must be complete, and needs the synergistic action of a reservoir with a good capacity, a normal compliance, and adequate sphincter outlet resistances. Continence is obtained by balancing these functions, and associating medical treatment and surgery is necessary. Bladder intermittent catheterization is the clue to obtain in most of the cases complete evacuation of the bladder and protection of the upper urinary tract. Increasing bladder capacity is achieved more often by augmentation cystoplasty (colon, ileus, stomach and ureter can be used). Autoplasty at the beginning, artificial tissue engineering will be the future. Augmentation of the bladder outlet resistances need surgical reconstruction (young dees, Pipi-salles procedures...) or uretral and bladder neck suspensions, artificial urinary sphincters, endoscopic injections of bulking agents. All these techniques can be proposed and combined according to the patient's gender, age and social environment. Continent cystostomy allows obtaining continence in difficult cases and after unsuccessful surgery of the bladder neck. Other techniques are under evaluation and sacral neuromodulation give at the moment some promising results. Managing neurogenic bladder must not be considered only in urological terms: orthopedic troubles, digestive and sexual disorders must not be forgotten in order to obtain at least an "acceptable social life".

Child↗
PubMed2006

A multicenter evaluation of urinary incontinence management and outcome in spina bifida.

PURPOSE: We describe urinary continence management and outcome in patients with spina bifida to identify the procedures that are most successful. MATERIALS AND METHODS: In a multicenter retrospective cohort study medical charts were studied. At the same time in a cross-sectional survey sociodemographic characteristics, orthopedic features and urinary continence were described based on the frequency of leakage from the viewpoint of patients or close relatives using a Likert scale of 5 items, namely 1-leakage permanent to 5-leakage never. RESULTS: A total of 421 patients were included, of whom 191 (45%) had been medically treated with a normal voiding pattern according to the patient viewpoint in 21%, clean intermittent catheterization in 61% and no specific bladder emptying method in 18%. The mean leakage score +/- SD was 2.74 +/- 1.55. On the other hand, 230 patients (55%) were surgically treated. Except for 23 patients who underwent noncontinent urinary diversion 207 were considered for treatment and continence description. The mean leakage score was 3.45 +/- 1.60. An artificial urinary sphincter in male and females, and a sling or Kropp technique in females were satisfactory when bladder enlargement was not required. In cases of bladder augmentation without continent diversion an artificial urinary sphincter in males and a bladder neck sling or cinch, Kropp and Young-Dees procedures in females have provided the best results. In cases of bladder enlargement with continent urinary diversion bladder neck closure or a wrap have provided the best results whatever the patient sex. CONCLUSIONS: Many factors may influence the choice of a technique, such as patient sex, bladder characteristics or orthopedic conditions. However, since to our knowledge no randomized, controlled study has been yet performed, definitive conclusions on the best way to achieve urinary continence in patients with spina bifida cannot be established.

Adolescent↗
PubMed2006

A multicentre study of the management of disorders of defecation in patients with spina bifida.

Patients with spinal dysraphism may have severe constipation and faecal incontinence. The impact of antegrade colonic enema (ACE) in the management of patients with spina bifida (SB) is analysed. In a multicentre cross-sectional study, constipation, faecal incontinence and faecal management were described. Cases surgically treated were identified. Data were collected from 423 patients, of whom 230 did not use any manoeuvre or laxatives to assist evacuation. Conventional treatment was used in 193 patients, including digital extraction in 39%, retrograde enema in 21% and oral laxatives in 52%. For intractable constipation and overflow of faecal incontinence, 47 patients were treated with ACE, of whom 41 used the method at a mean time of interview of 4.1 +/- 1.9 years after ACE operation; six abandoned ACE for conventional management. With ACE, faecal continence was significantly improved compared with conventional management, and neither retrograde rectal enema nor digital extraction were required. The conduit was fashioned to the right colon in 32 cases and to the left colon in nine cases. This study provides information on a multicentre experience in bowel management in SB patients. Whatever the technique used, ACE has improved faecal status compared with conventional therapy.

Adolescent↗
PubMed2005

[Cystic intrathoracic hemolymphangioma: a rare differential diagnosis of acute bronchiolitis in an infant].

Hemolymphangioma is a benign malformation of the lymphatic vessels. Cervical localisation is the most frequent. Mediastinal localisation is rare, but may be life-threatening because of airway compression. We report on a four-month-old boy who presented, in a context of epidemics, with clinical signs of acute bronchiolitis requiring mechanical ventilation for several days. Chest X-rays showed an important mediastinal mass with airways compression. Diagnosis was evoked on CT-scan aspects and confirmed by histology. Surgery allowed complete resolution, but dysphonia and oropharyngeal disorders persisted for several months. This rare congenital malformation is reviewed.

Acute Disease↗
PubMed2004

[Neonatal localized neuroblastoma: 52 cases treated from 1990 to 1999].

UNLABELLED: Neuroblastoma is the most frequent tumor observed in the newborn. The aim of this study was to review clinical features, treatment and outcome of newborns diagnosed with a localized neuroblastoma. POPULATION AND METHODS: Data from 52 cases treated according to the NBL 90 and 94 protocols between 1990 and 1999 in 18 French centers of pediatric oncology were analyzed. RESULTS: The median age at diagnosis was 12 days (range 0-28) with antenatal detection in 14 patients (27%). Tumor location was abdominal in 40 patients (adrenal in 20 of the 40), thoracic in eight, pelvic in three, and cervical in one. N-myc amplification was observed in one out of 40 evaluable cases. The size of the primary tumor was less than 5 cm in 25 cases, between 5 and 10 cm in 25 and more than 10 cm in two. Dumbbell tumor was observed in seven, of whom five had neurological deficit. One child died from hemorrhage after fine needle biopsy during diagnostic procedure. Primary surgical resection was attempted in 37 infants, of whom two died of surgery related complications and three had nephrectomy. Tumor was deemed as unresectable in 14 patients, and primary chemotherapy was given followed by surgical excision in 12. One of them died a few days after the beginning of chemotherapy. As a whole, continuous complete remission was achieved in 48 children, four of them after relapse. Overall survival was 92% with a median follow-up of 46 months (0-113 months). CONCLUSION: The excellent prognosis of localized NB in neonates needs very restrictive surgical indications, with well-established anatomic and imaging criteria. Indeed, chemotherapy based on weight and managed by expert teams should allow to perform surgical excision in safer conditions for unresectable tumors.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed2004

Sacral neuromodulation for neurogenic bladder dysfunction in children.

PURPOSE: We report a prospective randomized controlled study to evaluate the possible benefits of sacral neuromodulation (SNM) for the management of neurogenic bladder dysfunction in children. MATERIALS AND METHODS: We included in the study subjects between the ages of 5 and 21 years presenting with urinary incontinence due to neurogenic bladder. Before study inclusion all previous treatment was discontinued. Patients were randomly divided into the control group treated conventionally and the implant group treated with SNM. Treatment outcome was compared every 3 months for a minimum of 12 months based on clinical examination, voiding diary and urodynamic evaluation. RESULTS: A total of 26 boys and 16 girls with a mean age of 11.9 years were enrolled in this study. There were 21 patients in each group. The main underlying etiology was spina bifida. The 2 groups were comparable with regard to sex, age and urodynamic variables. Total disappearance of urinary leakage was observed in 1 patient who underwent implantation but he still required intermittent catheterization. Comparison of urodynamic variables disclosed no significant statistical difference except with regard to functional bladder capacity which was better in the control group and the leak point pressure which was better in the implant group (p <0.05). Evaluation of interindividual variations in the implant group revealed significant improvement in compliance and functional bladder capacity at 6 and 9 months but not at 12 months. Nine patients in the implant group reported improvement in intestinal transit, 5 total disappearance of urinary infection and 6 persistent sensation of a full bladder. No patient in the control group reported any improvement. Revision surgery was required in 3 cases due to lead migration, faulty connection and wound infection. CONCLUSIONS: SNM is a promising new therapeutic modality. Implant placement is minimally invasive, nondestructive and reversible. Use in children with neurogenic bladder has not been previously reported. Although some improvement was noted in patients treated with SNM, the difference with the control group was not significant. A larger multicenter study is warranted to evaluate SNM.

Adolescent↗
PubMed2003

The cholecystocolic bypass with jejunal interposition graft for bile acid depletion in bile and portal blood in guinea pigs.

Ileal bypass and cholecystostomy are used in children with selected cholestatic diseases to lower the bile acid (BA) levels in bile and blood and improve outcome. The efficacy of a cholecystocolic bypass in achieving the same goals was investigated in guinea pigs. In the study group (n=7), a cholecystocolic bypass was performed using a jejunal graft interposed between the gallbladder and the cecum. In the control group (n=5), a cholecystojejunal bypass was performed with a similar graft implanted in the proximal jejunum. Total BA concentration was measured in bile and portal blood at surgery (D0) and 30 days later (D30) by pooling the concentrations of 7 individual BA. D30/D0 BA ratios were compared. All animals developed normally without clinical symptoms. A 76% reduction in the bile T-BA levels was observed in both groups (p<0.05). A 80% decrease of T-BA levels was observed in portal vein in study group (p<0.05), suggesting that ileal bile flow and BA ileal reabsorption were highly impaired. No change in portal vein BA levels was observed in control group. Cholecystocolic bypass led to a significant loss of bile acids in guinea pigs and might be considered for bile diversion in pediatric patients with selected cholestatic diseases.

Anastomosis, Surgical↗
PubMed2003

Localized cervical neuroblastoma: prevention of surgical complications.

OBJECTIVES: The purpose of this study focused on cervical neuroblastoma (NB) was to assess the prognosis, define the most suitable methods of investigation, and evaluate risk factors for complications following primary surgery. METHODS: Between 1990 and 1999, we conducted two consecutive prospective multicentric studies (NBL90 and NBL94) on localized NB. Because the first study (1990-1994) found surgery-related morbidity and mortality, several surgical risk factors (i.e. adhesion to major vessels, size, friability, and dumb bell tumor) were defined and used prospectively as criteria of resectability in the second study (1994-1999). RESULTS: Of 617 cases included in the two studies, 43 involved cervical NB including 17 cervicothoracic tumors. With a median follow-up of 4 years, overall survival and event-free survival rates were 91 and 81%, respectively with no significant difference between cervical or cervicothoracic NB. Seventeen patients were included in the second study; surgery was used as the first line treatment in 11. Full pre-operative work-up was performed in eight patients, demonstrating one or more risk factors in three. The remaining three patients underwent emergency surgery with no pre-operative work-up or only ultrasound: two developed serious complications. All three patients presenting documented risk factors developed post-operative complications versus only two of the eight patients who presented no risk factor (n = 5) or were inadequately evaluated (n = 3) (P = 0.06). None of the five patients in whom full work-up demonstrated no risk factor had post-operative complications (P = 0.02). CONCLUSIONS: Cervical neuroblastoma has a favorable prognosis. Surgery is the treatment of choice but there is a risk of complications. Appropriate pre-operative work-up is mandatory to evaluate resectability. The surgical risk factors defined for our second study seem to be significant predictors of post-operative complications.

Female↗
PubMed2002

[Antenatal diagnosis of ovarian cyst: perinatal management].

More than 85% of prenatal ovarian cysts have a follicular or luteal origin. Their natural history is a spontaneous involution. However, adnexal torsion resulting in the loss of the ovary can occur at any time of the evolution. Pre and postnatal changes in the sonographic aspects allow to choose the appropriate therapeutical indications: conservative approach, laparoscopic surgery, or percutaneous aspiration. Prenatal aspiration must be avoided. The last prenatal sonographic examination should be performed as close as possible from the end of the gestation, and the first postnatal echography within the first 24 hours of life. Surgery of prenatal complicated cysts has to be planned a few days after birth. Uncomplicated prenatal cysts will be treated depending on size and echographic patterns at birth.

Adult↗
PubMed2002

Value of electrophysiological assessment after surgical treatment of spinal dysraphism.

PURPOSE: This study was conducted in children treated surgically for spinal dysraphism between 1994 and 2000 to determine the value of electrophysiological parameters for followup in comparison with clinical, radiological and urodynamic findings. MATERIALS AND METHODS: Electrophysiological examination was performed based on electromyographic detection of perineal muscle activity with evaluation of the sacral reflexes and measurement of lower limb and perineal evoked potentials. Clinical and radiological assessment consisted of physical examination, kidney ultrasonography and retrograde cystouretrography. Urodynamic assessment included determination of urethral pressure profile, bladder activity, compliance, voiding pressure and sphincter synergy. A total of 45 children were included in the study. In 28 cases electrophysiological assessment was performed routinely in 20 children (group 1) before and after (6 months and annually thereafter) surgical treatment (mean followup 30 months) and in 8 children (group 2) only after surgical treatment (mean followup 65 months). Electrophysiological findings were correlated with the urodynamic and clinical findings. Sensitivity, specificity and positive and negative predictive values were calculated. In 17 children (group 3) electrophysiological assessment was performed only once either before or after surgery. RESULTS: Electrophysiological assessment with combined electromyographic detection and perineal evoked potentials was highly sensitive in predicting urodynamic impairment, particularly vesico-sphincter dyssynergia (sensitivity 100%) or bladder dysmotility (sensitivity 86%). Specificity of perineal evoked potentials for detecting urodynamic dysfunction was 90%. Electromyographic detection and perineal evoked potentials also demonstrated satisfactory sensitivity in confirming urological impairment (sensitivity 79%, negative predictive value 90%). CONCLUSIONS: Electromyographic detection and perineal evoked potentials are valuable for assessment of patients after surgical treatment of spinal dysraphism. Because these techniques are minimally invasive and highly reproducible, they are suitable for baseline assessment in most cases (tethered cord, lipoma, syringomyelic cavities). Urodynamic assessment should be reserved for patients at high risk for secondary neurogenic bladder dysfunction such as myelomeningocele or sacral agenesis.

Child↗
PubMed2001

An experimental model of gastroschisis using fetoendoscopy: preliminary results and technical considerations.

BACKGROUND: The aim of this study was to show the feasibility of an experimental model of gastroschisis using fetoendoscopic surgery on sheep fetuses, and also to perform amnioinfusion until delivery using an in-dwelling intraamniotic catheter. METHODS: We analyzed the data from 18 pregnant ewes having 26 fetuses, which underwent surgery at 80 days of gestation (full term, 145 days). The fetendo technique was used to create a gastroschisis in 15 fetuses. The fetal abdominal wall was opened on the left side of the cord using scissors. The omentum and the intestinal loops were eviscerated using atraumatic forceps. Eleven fetuses were used as a control group. Twenty-one fetuses underwent amnioinfusion; a simple exteriorized catheter was used in seven cases and an intraamniotic catheter with an implantable port was used in the other 14. All ewes and fetuses were killed at the end of the experiment by an intravenous injection of pentotal; thereafter, the fetuses underwent necropsy. RESULTS: Twelve fetuses died and 14 survived (53.8%); seven of the 15 that underwent gastroschisis survived (46.7%). An amniotic infection occurred in nine fetuses (34.6%); of these, six died and three that were administered antibiotics survived. CONCLUSIONS: Our experience shows that this experimental model of gastroschisis is feasible and reproducible, and that a repeated amnioinfusion can be performed with an in-dwelling catheter in pregnant ewes. The use of an implantable port is safer than a simple exteriorized catheter.

Animals↗
PubMed2001

Endoscopic treatment of urinary incontinence: long-term evaluation of the results.

PURPOSE: We report on the use of polydimethylsiloxane for endoscopic treatment of urinary incontinence in children with neurogenic bladder and discuss our results to determine optimal criteria for patient selection. MATERIALS AND METHODS: A total of 44 children (19 males) have been treated endoscopically for urinary incontinence since 1995. Etiology was spina bifida in 35 cases. Previous surgery had been performed on 24 patients, including bladder neck reconstruction with (17) or without bladder augmentation. Mean patient age at injection was 13 years (range 7 to 17). A single transurethral injection was given in 23 cases, 2 in 17 and 3 or more in 4. Mean volume at each injection was 3.5 cc and for each patient the total volume injected was 5.7 cc. Mean delay between 2 injections was 6 months (range 3 to 15). RESULTS: Followup ranged from 6 to 53 months (median 28). Of the patients 15 (34%) are dry (continent greater than 4 hours, no urinary pad during the day), 11 (25%) are improved (continent 2 to 3 hours, occasional pad) and 18 had poor results. In the entire series only gender and preoperative hyperactivity influenced the results, as the best results were achieved in females with a stable bladder (44% of girls versus 21% of boys were cured). Good results persisted at 12-month followup in patients treated with only 1 injection (until 36 months for older patients) and after the last of 2 injections. Of the patients treated with 3 or more injections 1 was dry at 12-month followup and treatment failed in 3. CONCLUSIONS: Injection of polydimethylsiloxane at the bladder neck achieved continence in 34% of neurogenic bladder cases. Results were better in girls with a stable bladder. Results deteriorated in the first 12 months of followup. No more than 3 injections are advised if a satisfactory result is not achieved.

Adolescent↗
PubMed2000

Genetic homogeneity of the urofacial (Ochoa) syndrome confirmed in a new French family.

The urofacial syndrome (UFS) or Ochoa syndrome has been reported as a rare autosomal recessive disorder comprising a uropathy and facial abnormalities. The gene was mapped on chromosome region 10q23-q24. We report the first European cases of UFS. Haplotype analyses in our French family were compared with those previously described in patients from Columbia and America (literature data). The results are compatible with the same localization of the critical region and favor the hypothesis of genetic homogeneity.

Adolescent↗
PubMed2000

Chronic renal failure and portal hypertension--is portosystemic shunt indicated?

We report two girls with histories of recessive polycystic kidney disease. Both were on maintenance hemodialysis. They had undergone surgical distal portocaval shunt because of portal hypertension. Later, bilateral nephrectomy was performed, and they presented with hepatic encephalopathy (HE) and evolution towards irreversible hepatic coma and death. Portosystemic shunt is the treatment of choice of portal hypertension. The kidney plays a pivotal role in ammonia disposal during portosystemic shunt. Thus, we stress the risk of HE after portosystemic shunt followed by bilateral nephrectomy in patients with end-stage renal failure and suggest that combined liver-kidney transplantation should be considered.

Child↗
PubMed2000

Inappropriate liver transplantation in a child with Alpers-Huttenlocher syndrome misdiagnosed as valproate-induced acute liver failure.

A 3-yr-old boy received valproic acid (VPA) for recurrent seizures. He developed coma and acute liver failure that were attributed to VPA toxicity, and underwent emergency orthotopic liver transplantation (OLTx). Despite good graft function, his neurological state worsened and led to death a few months later. The diagnosis of Alpers-Huttenlocher Syndrome (AHS) was suspected, subsequently to liver Tx, in view of ongoing neurologic deterioration and magnetic resonance imaging (MRI) findings. The syndrome, recessively inherited, associates brain degeneration with liver failure, and is now considered a mitochondrial disease. Enzyme activity deficiencies of the respiratory chain were identified in muscle mitochondria, as well as morphologic abnormalities of mitochondria in the explanted liver. Guidelines for diagnosis are presented, in order to differentiate the liver failure in AHS from that induced by genuine VPA toxicity. It is recommended to avoid liver Tx in patients with AHS given the fatal neurological course of the disease.

Anticonvulsants↗
PubMed2000

Delayed cure of an omphalocele requiring abdominosternoplasty, right hepatectomy and partial splenectomy.

A large ventral hernia resulting from the primary treatment of an omphalocele according to the Gross technique was repaired at age 16. The girl presented with extra-abdominal development of the liver and the spleen along with hypotrophy of the abdominal and thoracic cavities. The operation included enlargement sternoplasty, liver and spleen-size reduction and prosthetic abdominal closure. The cosmetic and functional results are good and stable on 7-year follow-up. Surgical issues and blood transfusion policy are discussed. A multi-disciplinary pediatric surgical approach is advocated.

Female↗