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Biomedical subjects

J M Guys

Publications and source records attributed to J M Guys.

At least 37 records · Page 2Linked to original sources
PubMed1999

Exclusion of the Sonic Hedgehog gene as responsible for Currarino syndrome and anorectal malformations with sacral hypodevelopment.

Anorectal malformations (ARMs) are common congenital anomalies that account for 1:4 digestive malformations. ARM patients show different degrees of sacral hypodevelopment while the hemisacrum is characteristic of the Currarino syndrome (CS). Cases of CS present an association of ARM, hemisacrum and presacral mass. A gene responsible for CS has recently been mapped in 7q36. Among the genes localized in this critical region, sonic hedgehog (SHH) was thought to represent a candidate gene for CS as well as for ARM with different levels of sacral hypodevelopment according to its role in the differentiation of midline mesoderm. By linkage analysis we confirmed the critical region in one large family with recurrence of CS. In addition, the screening of SHH in 7 CS and in 15 sporadic ARM patients with sacral hypodevelopment allowed us to exclude its role in the pathogenesis of these disorders.

Anal Canal↗
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

Use of polydimethylsiloxane for endoscopic treatment of neurogenic urinary incontinence in children.

PURPOSE: We report on the injection of polydimethylsiloxane for endoscopic treatment of urinary incontinence in children with neurogenic bladder and determine the optimal criteria for patient selection. MATERIALS AND METHODS: We have treated 17 boys and 16 girls since 1995. The etiology of incontinence was spina bifida in 24 cases. Previous surgery was performed in 18 patients, including bladder neck reconstruction in 15 and bladder augmentation in 9. Mean patient age at injection was 13 years (range 7 to 17). We administered 1, 2 and 3 injections in 21, 11 and 1 patients, respectively. Mean volume at each injection was 3.2 cc. Mean interval between injections was 6 months (range 3 to 15). In all cases injection was done transurethrally. RESULTS: Followup ranged from 6 to 41 months (median 16). A total of 11 patients (33.3%) are dry (continence for greater than 4 hours and no urinary pad use during the day) and 8 (24.2%) are improved (continence for 2 to 3 hours and minimal pad use). Results are poor in 14 cases. Overall previous bladder neck surgery or preoperative detrusor hyperactivity did not influence results. Good results were mainly associated with female gender (47.4% of girls versus 10.5% of boys achieved cure). CONCLUSIONS: Injection of polydimethylsiloxane at the bladder neck resulted in continence in 33% of neurogenic bladder cases. Better results occurred in girls and injection did not compromise other surgical procedures. Polydimethylsiloxane seems more suitable than bovine collagen due to potential problems with biological product use.

Adolescent↗
PubMed1999

[Contralateral transinguinal laparoscopy in unilateral inguinal hernia].

BACKGROUND: Indication for surgical exploration of the contralateral groin during unilateral herniorraphy in children is the subject of a worldwide debate. Routine exploration based on the likelihood of a patent processus vaginalis (PV) according to age, gender or side to some extent leads to unnecessary procedures, while routine abstention may leave a peritoneal sac, likely to later induce a symptomatic hernia in about 10% of cases. METHODS AND PATIENTS: One hundred and twenty-five children aged from 1 month to 15 years underwent transinguinal laparoscopic assessment of the contralateral groin, using a 3 mm trocar and a 70 degrees telescope gently introduced through the exposed PV. Surgical exploration was performed only in those patients who exhibited a patent PV, and in patients where the groin was poorly visualized due to technical problems. RESULTS: Regardless of age, contralateral surgery was not considered in 88 (70%) of the 125 children. Among the 37 patients that were operated upon, eight had a negative exploration due to an erroneous endoscopic evaluation. Surgery was avoided in 35 (56%) of the 62 infants aged less than 2 years, including nine of the 13 prematures who were previously routinely operated upon. Conversely, in the 63 older patients who were readily spared from surgical exploration, the videoscopic evaluation allowed appropriate selection for contralateral surgery in six. CONCLUSION: A routine policy, either of surgery or observation, is no longer indicated as a quick, safe and cost-effective method is available to detect a patent PV. The transinguinal laparoscopy is safe and could be easily performed by surgeons already skilled in pediatric herniorraphy. Therefore, the videoscopic transinguinal contralateral evaluation is worth being promoted to ensure an appropriate surgery tailored to the anatomical features.

Adolescent↗
PubMed1999

Sacrococcygeal teratoma with an intradural and extramedullary extension in a neonate: case report.

OBJECTIVE AND IMPORTANCE: The intradural extension of a sacrococcygeal teratoma is rare and has been reported previously in only two cases. We present a case of a sacrococcygeal teratoma with an intradural and extramedullary extension. CLINICAL PRESENTATION: The patient was a full-term female infant, born with a sacral mass. Ultrasonography, computed tomography, and magnetic resonance imaging showed an intraspinal extension. INTERVENTION: The operation confirmed the extension of the mass up to the T4 level, and total tumor removal was achieved. The pathological examination showed a mature teratoma. CONCLUSION: The postoperative course was uneventful, and at the 3-year follow-up examination, the patient had experienced normal growth without deficit in the lower limbs but had a neurogenic bladder. Magnetic resonance imaging disclosed no tumor recurrence. This case represents the third case in the literature of an intradural extension of a sacrococcygeal teratoma and the first one with an extramedullary component so extensive.

Dura Mater↗
PubMed1999

[Abdominal cystic lymphangiomas in children. Clinical, diagnostic and therapeutic aspects: apropos of 21 cases].

Twenty-one abdominal cystic lymphangiomas were observed in paediatric patients during a 15-year period, in 11 boys and 10 girls. Diagnosis was prenatal in 2 cases; the mean age of the other 19 children was 4.7 years (range: 3 months-8 years). Tumours were intraperitoneal in 16 cases and retroperitoneal in 5 cases. Symptoms were variable: abdominal pain in 15 cases, palpable tumour in 6 cases (excluding the two cases of prenatal diagnosis). Complications included obstruction in 7 cases (including 3 by volvulus), infection in 6 cases, and intracystic haemorrhage in 3 cases. Abdominal ultrasonography correctly established the diagnosis in all children. Surgical treatment included 20 complete resections and one incomplete resection, including 6 with bowel resections. With a follow-up ranging from 6 months to 10 years, one recurrence occurred and was successfully reoperated. Intraabdominal cystic lymphangioma in childhood is a rare tumour with a variable presentation. An accurate diagnosis can be established by abdominal ultrasound. Complete resection should be performed whenever possible.

Abdominal Neoplasms↗
PubMed1999

[Use of polydimethylsiloxane in the endoscopic treatment of urinary incontinence of neurologic origin in children].

AIM: We report the use of polydimethylsiloxane (PDS-Macroplastique) in endoscopic treatment of urinary incontinence in children with neurogenic bladder and try to determine optimal criteria for patient selection. METHODS: Forty four children (19 males, 25 females) have been treated since 1995. Aetiology was mainly spina bifida (n = 36). Previous surgery had been performed in 26 patients, including bladder neck reconstruction in 21 cases and bladder augmentation in 15 cases. Mean age at injection was 13 years (7 to 17). Only one injection was performed in 27 patients, two injections in 4 cases, and three and more injections in 3 cases. Mean volume at each injection was 3.6 cc. Mean interval between two injections was 6 months (3-15 m). All injections were performed transurethrally. RESULTS: Follow-up ranged from 6 to 41 months (median: 23). Fifteen patients (34%) are dry (continence > 4 hours, no urinary pad during the day) and 11 (25%) are improved (continence from 2 to 3 hours, minimal pad). Eighteen patients obtained poor results. In the entire series previous bladder neck surgery or preoperative detrusor hyperactivity did not interfere with the results. The only difference concerns the sex-ratio: the good results were mainly seen in females: 44% of girls are cured versus 21% of boys. CONCLUSION: Injection of PDS in the bladder neck achieve the goal of continence in 34% of the cases in neurogenic bladder. Better results are seen in girls. Injection does not compromise other surgical procedures. The use of PDS seems more suitable than bovine collagen in view of the potential problems of prions.

Adolescent↗
PubMed1998

Antenatal rupture of a diverticular rectal duplication with neonatal perineal fistulization.

A cystic pelvic malformation was found in a fetus on antenatal sonography (US) at 26 weeks of gestational age that was no longer present 3 weeks later on control US. The male child presented at birth with a right-sided perineal mass that fistulized with meconial drainage. A radiopaque enema showed a low posterior rectal fistula filling a poorly delineated pouch. Surgery performed through a posterior sagittal approach allowed identification and closure of the fistula and pouch drainage. The diagnosis of a diverticular rectal duplication was considered, although no intestinal lining was observed macroscopically or histologically. The child's anorectal function was normal after a 20-month follow-up. Labeling of the malformation and embryological hypotheses are discussed since the case does not fulfill all the criteria of an intestinal duplication. Surgical techniques are discussed, with an emphasis on the sagittal posterior approach.

Diverticulum↗
PubMed1998

Functional outcome and specific complications of gastrocystoplasty for failed bladder exstrophy closure.

PURPOSE: The encouraging initial results of gastrocystoplasty led us to perform it for failed bladder exstrophy closure. We assess the functional outcome of the augmented bladder and evaluate complications related directly to use of the stomach in this specific group of children. MATERIALS AND METHODS: We performed gastrocystoplasty in 22 children an average of 9.5 years old with a small, poorly compliant bladder after staged reconstruction of bladder exstrophy failed. Followup ranged from 6 months to 6 years (mean 3 years). RESULTS: Complete urinary continence was achieved in 14 children (64%). Voiding via the urethra was possible in 13 patients (60%) but post-voiding residual urine was significant in 12. Bladder capacity increased from a mean of 77 to 270 ml. Bladder capacity decreased during followup in 3 children, requiring repeat augmentation. Six children had isolated dysuria and 2 had dysuria with hematuria. Perforation of the gastric patch and a bleeding gastric ulcer occurred in 1 patient each. CONCLUSIONS: The disadvantages of gastrocystoplasty outnumber its advantages after failed bladder exstrophy closure. Urethral sensation makes dysuria a major discomfort. Safety is not optimal, since perforation may occur. Voiding is not efficient because gastrocystoplasty provides continence only when it is associated with intermittent catheterization. Bladder capacity is insufficiently augmented and inconsistent during followup. We believe that the use of gastrocystoplasty in cases of failed bladder exstrophy closure should be reconsidered.

Adolescent↗
PubMed1997

[Persistent urination disorders after treatment of posterior urethral valve: incidence and semiology].

Micturition disorders were studied retrospectively in a series of 165 children over a period of 15 years. Among patients without lesions of the upper tract at the time of diagnosis (group A, n = 131), 18 (14%) had persistent nocturnal and diurnal enuresis: one urethral stenosis and six vesicoureteric reflux required surgery; three experienced persistent pollakiuria and enuresis. Among patients with upper urinary tract damage at the time of diagnosis (group B, n = 34), seven presented with recurrent urinary tract infection, five with nocturnal and diurnal enuresis and three with urolithiasis. In the long term, only 52% of them had normal renal function and two were successfully transplanted. Micturition disorders following treatment of posterior urethral valves are frequent and usually related to the so-called valve bladder syndrome. Iatrogenic complications and mortality rate have dramatically decreased during the recent years but long-term renal function impairment remains the most critical problem.

Child↗
PubMed1997

A transvesical approach to müllerian duct remnants.

Surgical extirpation is the treatment of choice for symptomatic müllerian duct remnants (prostatic utricle, PU), and several surgical approaches have been described for the treatment of this pathology. A group of 11 patients with symptomatic PU were observed and treated. Associated anomalies included proximal or penoscrotal hypospadias in all patients and cryptorchidism in 9 (81.8%). In all cases the PU needed surgical correction, as the patients had recurring symptomatology. Surgery was carried out transvesically in 10 (91%) cases and in 1 a perineal approach was used. There were no surgical complications, and at follow-up all patients showed complete resolution of the symptoms. We believe the transvesical approach, compared to other techniques, is more advantageous in the treatment of this pathology, as it permits excellent exposure, ease of surgery, good reconstruction, and good functional results with no sequelae.

Child, Preschool↗
PubMed1996

Weaning children from tube to oral feeding.

Most of the children seen by specialists in neonatalogy, neuropaediatrics or neurosurgery do not have any problems in starting with oral feeding after a period of tube feeding lasting between 15 and 20 days. Children who have been tube fed for a longer period, however, can find it very difficult or even impossible to re-establish oral feeding when they have sufficiently recovered from their underlying problem. To cope with this situation we propose a procedure based on the afferentation or re-afferentation of the oropharyngeal cavity by sensory stimulations and by re-establishment of the biological clock (circadian rhythm) by applying these stimulations during tube feeding at regular hours. In 19 children who showed difficulties oral feeding became possible a short time after such a procedure had been applied. If the principles of swallowing neurophysiology and the biological rhythm are respected, this procedure, which also involves a contribution from the family, leads to quicker oral feeding and shorter stay in hospital.

Adolescent↗
PubMed1996

Acute presentation of congenital diaphragmatic hernia past the neonatal period: a life threatening emergency.

PURPOSE: Major gastric distension in the left hemithorax can threaten life in patients with congenital diaphragmatic hernia (CDH) presenting after the neonatal period. After presentation of two pediatric cases, guidelines for the optimal care of these patients are given. CLINICAL FEATURES: Both children had respiratory and cardio-circulatory compromise on arrival. The diagnosis of late presenting CDH was made and the severity of symptoms was related to a voluminous distension of an intrathoracic stomach. Successful placement of an naso-gastric tube in the first patient, lead to a rapid clinical improvement, allowing surgical repair. In the second patient, oro- or naso-gastric decompression was not possible, and while the lungs were mechanically ventilated and the patient was prepared for surgery, a sudden cardiocirculatory arrest was managed by external chest compressions and rescuscitation drugs. Transthoracic percutaneous decompression of the stomach was the sole treatment allowing spontaneous cardiac activity to reappear, and haemodynamic condition to normalize. However, the child died from brain death after this episode. CONCLUSION: Gastric decompression is the key for the treatment of patients with CDH who present respiratory and/or cardiocirculatory distress due to the intrathoracic distension of the stomach. If an oro- or naso-gastric decompression is not possible, then radiologically directed percutaneous decompression under local anesthesia is required. After decompression, the patient is prepared for surgery, with particular emphasis on fluids infusion, in order to correct the frequently associated hypovolaemia.

Acute Disease↗
PubMed1996

Artificial urinary sphincter implantation for neurogenic bladder: a multi-institutional study in 107 children.

OBJECTIVES: To evaluate the outcome of implantation of the AMS 800 artificial urinary sphincter in children with neurogenic bladder. PATIENTS AND METHODS: The records of 107 children (74 boys and 33 girls) treated for neurogenic urinary incontinence by implantation of the AMS 800 artificial sphincter between 1983 and 1993 were reviewed retrospectively. The underlying cause of incontinence in 92 patients was spina bifida, the mean age at implantation was 13.7 years and 30 patients had undergone previous bladder neck surgery. Implantation was carried out in combination with another surgical procedure in 24.3% of patients. The mean follow-up was 61 months (minimum 12). RESULTS: The mean operational life of the sphincter was 56 months. No deaths occurred but removal of the artificial sphincter was necessary in 20 patients (19%). Surgical revision was not required in 44 patients (41%) but at least one revision was performed in 63 and more than one in 42. There were 21 mechanical failures, 40 surgical complications and 39 cases involving changes in the dynamics of bladder function. Of the 87 children (81%) in whom the device is still in place, 72 are completely continent (83%), 10 are slightly wet, and five are incontinent. The overall success rate was 77%. CONCLUSIONS: The results emphasize that for successful implantation of an artificial urinary sphincter in children, the pre-operative bladder capacity must be sufficient and previous surgery should not have been performed on the bladder neck, the site of choice for implantation in children. Sphincterotomy is not always necessary in paediatric patients. Children and parents should be informed of the high complication rate and the need for long-term follow-up.

Adolescent↗
PubMed1994

Pediatric endoscopic surgery: pride and prejudice.

82 endoscopic surgical procedures (abdominal: 77; thoracic: 5) were performed by the same surgeon on 75 children aged from 1 month to 17 years (median 8.1 years) during the two-year period from January 1991 to December 1992. Due to the multispecialization of the Children's Hospital, a variety of pathologies were explored or treated with appendectomy accounting for 33% (27/82). There were no perioperative deaths. Three major complications occurred (1 post-appendectomy peritonitis, 1 hemorrhage during splenectomy and 1 post-operative occlusion). 14 patients required conversion to open surgery. Indications for endoscopic exploration of advanced lesions, prospective indications, and policy when confronted with a healthy appendix are discussed.

Abdomen↗
PubMed1994

Tracheomalacia caused by arterial compression in esophageal atresia.

After surgical repair of esophageal atresia (EA), any persistent respiratory distress unexplained by operative complications should lead to performance of tracheoscopy to evaluate the possibility of tracheomalacia. In a review of 46 cases of EA repair, 12 patients developed severe airway problems, manifested mainly by acute apneic episodes. These were related to an anterior extrinsic tracheal compression from vascular structures: the innominate artery alone in 8 patients, and in combination with the aortic arch in 4 patients. Aortopexy, evaluated by intraoperative tracheoscopy, provided good results in 10 patients with a mean follow-up of 2 years. After EA repair, respiratory symptoms, often related to feeding, should be recognized as a possible indication of severe compression of the trachea by the great vessels. The analysis of endoscopic features should allow consideration of patients as candidates for aortopexy, as long as they have resolved their symptoms after conservative therapy, and after investigation of all other possible sources of pulmonary problems. Gastroesophageal reflux must be recognized (7 cases in the series), but does not contraindicate aortopexy if the latter is deemed necessary. In selected cases, the relief of compression, controlled by intraoperative endoscopy, is considered a key component of a comprehensive program to treat children after EA repair.

Aorta, Thoracic↗