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Biomedical subjects

J M Laberge

Publications and source records attributed to J M Laberge.

At least 73 records · Page 4Linked to original sources

Apnea threshold and breathing rhythmicity in newborn lambs.

This study was designed to determine the effect of the removal of chemical stimuli on breathing rhythmicity in awake newborn lambs; it was also designed to define the chemical threshold below which breathing would stop [arterial PCO2 (PaCO2) apnea threshold]. We used a technique of graded extracorporeal CO2 removal with apneic oxygenation in three groups of animals according to age and carotid body (CB) integrity: < 2 days, CB intact (n = 5); 12 days, CB intact (n = 7); and 12 days, CB denervated (CBD; n = 5). In all animals, whatever their age and CB status, suppression of the chemical drive resulted in sustained apnea. The study, performed at four constant levels of oxygenation (hyperoxia, normoxia, moderate hypoxia, and severe hypoxia), allowed precise determination of the PaCO2 apnea threshold. We found that this PaCO2 apnea threshold depended on the degree of postnatal maturation (it was higher in the younger lambs), the level of arterial oxygenation (it was lowered by hypoxia), and CB status (it was higher in CBD animals). Moreover, we found that the 12-day-old CBD lambs breathe at a level of PaCO2 set close to the point of apnea.(ABSTRACT TRUNCATED AT 250 WORDS)

Animals↗

[Biliary complications in pediatric liver transplantation].

Biliary tract complications are reported in 15% to 20% of orthotopic liver transplantations (OLT). Since 1986, 55 OLT were done in 50 children with a mean age and weight of 5.6 years and 18.8 kg respectively. There were 28 (51%) reduced liver grafts (RLG) and 27 (49%) whole liver grafts (WLG). Since starting using RLG in 1988, 70% of transplantations have been RLG. Choledochocholedochostomy with a T-tube (CC) or choledochojejunostomy (CJ) were done in 25 (45%) and 30 (55%) cases, respectively. The overall mortality was 19% with one death related to biliary problems. There were 14 biliary tract complications (25%) in 12 patients including 7 leaks, 6 obstructions and one intrahepatic biloma. Leaks leading to bile peritonitis were managed with simple suture and drainage and were related to the T-tube (4, to the Roux-en-Y loop (2) and the transection margin of a RLG (1). Obstruction was documented in 6 cases, none of which were associated with hepatic artery thrombosis (HAT). Stenosis after CC reconstruction (3) required conversion to CJ. Two patients had revision of CJ because of kinking of the common bile duct and an anastomotic stricture 46 months after OLT. One patient developed a vanishing bile duct syndrome 4 months post-transplant and died while waiting for retransplantation. One patient had multiple episodes of cholangitis after HAT and was retransplanted. The rate of biliary complications was not influenced by neither the type of graft (RLG : 25% vs WLG : 25,9%) nor the type of biliary reconstruction(CC : 28% vs CJ : 23%).(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

A new prognostic classification for esophageal atresia.

BACKGROUND: Waterston's risk categories have been extensively used in the past for the classification and management of neonates with esophageal atresia. Advances in neonatal care have affected the prognostic usefulness of the Waterston classification. The purpose of this study is to formulate a new classification of risk factors that would more accurately predict outcome. METHODS: We retrospectively reviewed 95 consecutive cases of esophageal atresia and/or tracheoesophageal fistula treated at the Montreal Children's Hospital. Fifteen patients were in the Waterston class A, with a 6.7% mortality; 35 patients, in class B with a 5.7% mortality; and 45 patients, in class C with a 26.7% mortality. Logistic regression analysis of the influence of each risk factor (weight, pulmonary status, and severity of associated anomalies) on mortality was performed. RESULTS: Birth weight was not found to independently influence mortality. Only severe pulmonary dysfunction with preoperative ventilator dependence and severe associated anomalies had a prognostic influence. We therefore revised the classification to include only significant factors. The new high-risk class II consists of patients with either life-threatening anomalies or both major anomalies and ventilator dependence; the low-risk class I includes all other patients. These criteria radically changed the stratification of both the number of cases and the mortality among classes: the 82 patients in class I had a 7.3% mortality; the 13 patients in class II had a 69.2% mortality. Logistic regression analysis confirmed the validity of this new classification by showing displacement of individual variables by the revised classification but not by Waterston's. CONCLUSIONS: This new "Montreal classification" can simplify and improve the stratification of patients with esophageal atresia. It also reflects the more favorable outcome of low birth weight neonates.

Abnormalities, Multiple↗

Congenital urethral fistula with normal anus: a report of two cases.

Congenital rectourethral or anourethral fistulae without imperforate anus in males are rare, representing less than 1% of anorectal malformations. We report our experience with two males with "N type" urethral fistulae. One, a 5-year-old boy, presented with recurrent urinary tract infections (UTIs) and passage of urine per anus. Investigations included a voiding cystourethrogram (VCUG), which demonstrated a fistula from the urethra to the anus. On physical examination, a small perianal opening was noted just outside the anus, which drained a small amount of urine after voiding. The fistula was excised via a perineal approach. The second patient is a 5-year-old boy with a long history of recurrent UTI requiring multiple hospitalizations since the newborn period. Chronic renal failure developed as a complication of repeated urinary tract infections. Investigations showed a single hydronephrotic pelvic kidney and a small bladder. He underwent numerous diagnostic and reconstructive procedures including cystoscopy and augmentation cystoplasty. Recurrent infections continued and an N type anourethral fistula was eventually diagnosed. The fistula was located between the anal canal and the membranous urethra. An anterior perineal approach was also used. Both fistulae were easily located, and reconstructive surgery of the urethra was not required. Postoperative VCUGs in both patients were normal. They have been free of infection with normal urinary continence since resection of the fistula. Congenital N type anourethral fistulae are rare, but should be considered in cases of recurrent urinary tract infections. The diagnosis may be missed by endoscopic procedures, but VCUG should demonstrate the fistulous tract.

Child, Preschool↗

Cystic neuroblastoma.

In the newborn, cystic masses of the adrenal gland are unusual findings and most are secondary to hemorrhage. We present a patient with a clinical history typical for adrenal hemorrhage who was found to have a thick walled cystic adrenal mass on both physical and ultrasound examination. During evaluation the mass decreased in size, but vanillylmandelic acid (VMA) and homovanillic acid (HVA) levels were elevated. Laparotomy and left adrenalectomy confirmed the diagnosis of a cystic neuroblastoma. Adrenal cyst, adrenal abscess, and cystic neuroblastoma are all rare entities in the newborn. We recommend that all cystic masses of the adrenal be evaluated by urinary VMA and HVA and that the possibility of cystic neuroblastoma be kept in mind when an adrenal cystic mass is followed nonoperatively.

Adrenal Gland Diseases↗

Diaphragmatic injury in children.

Injury to the diaphragm is rare in children. From 1972 to 1990, 13 children were treated for diaphragmatic injury at Ste-Justine and Montreal Children's hospitals. There were seven boys and six girls, ranging in age from 1 to 15 years (average, 7.5 years). Eight patients sustained penetrating trauma and five patients sustained blunt trauma. Nine patients had associated injuries, most commonly involving the liver. All thirteen patients underwent exploratory laparotomy with repair of the diaphragm. There were two deaths, both unrelated to the diaphragmatic trauma. All surviving patients recovered without sequelae. One patient underwent cosmetic repair of a chest wall deformity 9 years after injury. Diaphragmatic injury must be considered in any child suffering blunt or penetrating thoracoabdominal trauma. Because of the increased compliance of the thoracic cage in children, rupture of the diaphragm can occur without signs of external injury. Morbidity and mortality can be minimized by a high index of suspicion, prompt recognition, and surgical repair of even the smallest diaphragmatic injury.

Adolescent↗

Extensive upper aerodigestive tract anomalies in 'VACTERL' Association.

Extensive upper aerodigestive tract anomalies are rare in newborns. When they occur, they are often accompanied by other congenital malformations. We present a case of a newborn who presented to the Montreal (Quebec) Children's Hospital with severe tracheoesophageal abnormalities, as part of vertebral, anal, cardiac, tracheal, esophageal, renal, and limb anomalies (VACTERL) association. In spite of surgical intervention, the patient finally succumbed to other congenital abnormalities. Management of infants with such conditions usually involve complex reconstructive efforts, while prognoses remain poor due to other congenital defects and cardiopulmonary complications.

Abnormalities, Multiple↗

Laparoscopic cholecystectomy: a treatment option for gallbladder disease in children.

Laparoscopic cholecystectomy was performed safely in two children ages 7 and 15 years. Special considerations were required in the younger child because of the discrepancy between the length of the instruments and the size of the peritoneal cavity. Both children remained in hospital less than 24 hours and were able to resume normal diet and activity quickly. Pediatric surgeons with special training in laparoscopic surgery may want to use this treatment modality for children with biliary tract disease.

Adolescent↗

Gracilis muscle transposition for anal incontinence in children: long-term follow-up.

We report a series of 7 patients, aged 6.5 to 19 years (mean, 12.9), who have been treated for uncontrollable fecal incontinence since 1976: 5 had imperforate anus and multiple subsequent operative procedures, 1 had a low myelomeningocele with bi-sphincteric incontinence, and 1 had a traumatic destruction of the sphincter apparatus. A modified Pickrell procedure was performed, with the gracilis muscle transposed subcutaneously, without constructing a pulley through the median raphe as originally described. All patients were evaluated by anorectal manometry preoperatively and post-operatively. They were followed-up for a period of 0.5 to 12.5 years (mean, 4.4). All patients were continent at follow-up, with a normal defecation pattern and no enemas required. None of the patients had evidence of fibrosis of the muscle or anal canal, and tension in the transposed muscle was maintained. Voluntary contractions remain efficient in all cases. Age was thought to be an important factor: personal motivation and compliance with physiotherapy, essential for a good outcome, is unlikely to be present in the younger child. We conclude that the gracilis sling procedure is an excellent long-term alternative for total fecal incontinence when time and other therapeutic measures have failed.

Adolescent↗

Familial biliary atresia in three siblings including twins.

We report a North American Indian family of five children in which dizygotic twin sisters and a third sibling had biliary atresia. This is in contrast to many reports of discordant biliary atresia in twins. Added to 29 previously documented cases of familial biliary atresia, these three cases support the theory that both genetic and acquired factors play a role in the pathogenesis of this disease.

Biliary Atresia↗

Infectious complications of pediatric liver transplantation.

Twenty-five pediatric orthotopic liver transplantations (OLTs) performed in 22 patients at Sainte-Justine Hospital were reviewed for infections complications. One patient died within 12 hours posttransplantation and is excluded. The patients had an average age of 6.1 years (range, 1.25 to 19 years) and an average weight of 20.4 kg (range, 11 to 55 kg). Two patients (9%) were cytomegalovirus (CMV) seropositive and 9 of 19 patients (48%) were Epstein-Barr virus (EBV) seropositive preoperatively. Five of the donors (20%) were CMV seropositive. The most common indications for OLT were biliary atresia (8) and tyrosinemia (7). There were 4 deaths, for an overall mortality rate of 19%. In 3 patients, deaths were related to infection (CMV hepatitis and duodenitis with aortoduodenal fistula, adult respiratory distress syndrome [ARDS] with Streptococcus viridans pneumonia, Escherichia coli cholangitis with progressive hepatic failure). Fifteen patients (72%) had 41 major infections, most of them bacterial, during the first month posttransplantation. These include pneumonia (25%), line sepsis (17%), cholangitis (14%), and tracheitis (14%). There was only one major viral infection, a CMV hepatitis that occurred in the first month posttransplantation. Three patients had fungal infections (8%) associated with hepatic artery thrombosis and recurrent cholangitis. All three patients required retransplantation. There was only one protozoal infection (Pneumocystis carinii pneumonia) causing life-threatening respiratory failure, from which patient recovered without sequelae. Infection still remains a serious complication of OLT. Bacterial infection is common and is usually associated with technical complications. The low rate of CMV infection is related to low incidence of CMV in the donor pool and the minimal use of strong immunosuppressants.

Adolescent↗

Congenital adenomatoid malformation of the lung: current management and prognosis.

A retrospective 10-year review of congenital adenomatoid malformation (CAM) included 10 cases diagnosed in utero by ultrasound and 13 cases that presented postnatally. Two prenatally diagnosed cases were aborted because of associated lethal anomalies. All remaining patients underwent resection. Up to one third of all cases, whether diagnosed prenatally or postnatally, were asymptomatic. Resection is recommended to avoid respiratory distress, infection, or associated malignancy. There were 5 nonsurvivors, including 2 therapeutic abortions and 3 who died postoperatively. All had either polyhydramnios or ascites. All patients who died postoperatively had a respiratory arrest at birth and underwent immediate lobectomy. All died on the first day of life after a brief period and were found to have associated pulmonary hypoplasia. One had undergone a prenatal transthoracic cyst aspiration at 34 weeks gestation in an attempt to allow lung growth and prevent premature labor. Prenatal ultrasound findings suggestive of poor prognosis included polyhydramnios, ascites, mediastinal shift, and noncystic type III CAM. However, there is a spectrum of severity of CAM. The lesion can either regress and be asymptomatic at birth, or it can progress to cause either fetal death from hydrops or neonatal death caused by associated pulmonary hypoplasia. These findings should be considered in prenatal counseling for CAM.

Cystic Adenomatoid Malformation of Lung, Congenita↗

A more than 25-year experience with end-to-end versus end-to-side repair for esophageal atresia.

End-to-end anastomosis has become the standard technique for repair of esophageal atresia with distal tracheoesophageal fistula. End-to-side anastomosis with ligation of the fistula in continuity has also been advocated. For over 25 years both methods have been used in this hospital. The purpose of this report is to compare retrospectively the results obtained with these two techniques. Between 1962 and 1988, 111 neonates underwent repair of esophageal atresia and distal tracheoesophageal fistula. 74 patients had an end-to-end repair and 37 had end-to-side anastomosis with fistula ligation, according to the surgeons' preference. There were no significant differences between the two groups with respect to preoperative variables and Waterston classification. There was a significantly increased incidence of fistula recanalization (22% v 3%, P = .003) and mortality directly related to the esophageal atresia (16% v 3%, P = .03) in the end-to-side group versus the end-to-end group. The end-to-end group had a significantly higher incidence of gastroesophageal reflux. Logistic regression analysis showed that the end-to-side repair was associated with a greater overall mortality. We conclude that the end-to-end repair appears to be a safer procedure than the end-to-side technique.

Analysis of Variance↗

Distal congenital esophageal stenosis associated with esophageal atresia.

A series of six patients with congenital esophageal stenosis associated with esophageal atresia (EA) and distal tracheoesophageal fistula is presented. Three patients required only repeated dilatations, and have had good results. Two patients required limited resections of the distal esophagus, with excellent results. One patient died following a Heller myotomy. Tracheobronchial rests were present in the distal esophagus in the latter three patients. Diagnosis of congenital distal esophageal stenosis following repair of EA requires a high index of suspicion and a careful review of previous esophagrams. It is important to exclude anastomotic stricture and stenosis associated with gastroesophageal reflux. This requires barium esophagram, esophagoscopy with biopsy, and esophageal pH monitoring. Once a congenital basis for distal esophageal stenosis is suspected, management consists of dilatation by bouginage followed by balloon dilatation. Resection is reserved for persistent stenoses from tracheobronchial rests, which usually do not respond to dilatations.

Biopsy↗

Management of complex ovarian cysts presenting in the first year of life.

With more frequent antenatal and postnatal diagnosis, the management of ovarian cysts has become somewhat controversial. Management protocols for simple ovarian cysts have been proposed. The purpose of this study was to establish a management protocol for complex ovarian cysts presenting antenatally and in the first year of life. We reviewed the records of nine infants who underwent surgical treatment for ovarian cysts over a 10-year period (1980 through 1989). Antenatal ultrasound performed between 24 and 34 weeks of gestation showed ovarian cysts in six infants. All six infants had complex cysts ranging in size from 3 to 10 cm on postnatal ultrasound. Exploration with oophorectomy or salpingooophorectomy was carried out at 2 days to 3 months of age for ovarian torsion. Ovarian cysts measuring up to 7 cm were diagnosed postnatally in three infants from 1 day to 7 months of age with ultrasound confirmation of complex, cystic intraabdominal masses. All patients underwent salpingooophorectomy, two for tuboovarian torsion and the third patient for a juvenile granulosa cell tumor. We recommend that all complex ovarian cysts, regardless of size, be surgically removed because they represent torsion, neoplasm, or alternate diagnoses, and removal can prevent possible complications.

Diagnosis, Differential↗

The use of Glisson's capsule flap in reduced orthotopic hepatic transplantation.

The technique of graft reduction has increased the donor pool for children in need of orthotopic hepatic transplantation. However, this technique has been associated with increased perioperative blood loss. Further refinements, such as covering the cut surface of the donor liver [corrected] with a flap of Glisson's capsule, may help reduce this complication.

Blood Loss, Surgical↗