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Biomedical subjects

J M Laberge

Publications and source records attributed to J M Laberge.

At least 91 records · Page 5Linked to original sources

Reversal of intrapulmonary shunting in cirrhosis after liver transplantation demonstrated by perfusion lung scan.

A young girl with biliary atresia leading to cirrhosis developed respiratory complications with hypoxemia. Intrapulmonary shunting was diagnosed with a 99mTc-MAA perfusion lung scan, which showed marked systemic activity. The shunting resolved after liver transplantation. The perfusion lung scan offers an efficient method to screen patients with cirrhosis in whom intrapulmonary shunts are suspected and to follow their progress.

Child↗

Appendicitis in children: current therapeutic recommendations.

Wound infection is the most common source of morbidity in appendicitis. Most recent pediatric series use protocols of preoperative antibiotics with aerobic and anaerobic coverage, intraoperative lavage, no peritoneal or wound drains, and continuation of antibiotics postoperatively with complicated appendicitis. There still remains controversy concerning skin closure and the duration of antibiotic therapy. We report the results of a prospective protocol followed over 2 years with 420 children. The protocol was designed to determine whether the skin could be closed primarily in all patients undergoing appendectomy. Preoperatively all patients received triple antibiotics (ampicillin, gentamicin, and clindamycin) that were continued postoperatively for two doses if there was a normal appendix or simple acute appendicitis, for at least 3 days with gangrenous appendicitis, and at least 5 days with perforated appendicitis. Antibiotics were continued if the patient remained febrile or had a white count greater than 10,000. No drains were used and the skin was closed primarily. The overall infectious complication rate was 1.0% (4/420). Among those with a normal appendix or simple acute appendicitis there were no infectious complications. Among those with gangrenous or perforated appendicitis there were 1.7% wound infections (2/117) and 1.7% intraabdominal abscesses (2/117). Duration of hospitalization was 2.1 days (range, 1 to 5 days) after simple acute appendicitis and 6.9 days (range, 3 to 40 days) after gangrenous or perforated appendicitis. These results set new standards in terms of wound management, infectious complications, and length of hospital stay.

Adolescent↗

Unusual patterns of congenital neck masses in children.

Congenital neck masses--thyroglossal duct cysts (TDC) and branchial cleft cysts (BCC)--are frequently encountered in any pediatric surgical practice. While their diagnosis is usually straightforward, unusual or combined presentations may occur. We report eleven cases of unusual patterns of congenital neck masses in children. Two patients underwent resection of a BCC with a sinus tract extending through the carotid bifurcation. Both patients subsequently presented with a new mass near the previous scar, which was thought to be a recurrence but was found at surgery to have TDCs with sinus tracts extending through the hyoid bone. In one patient, a lateral neck mass (presumed to be a BCC) was found at surgery to be a TDC. In all three cases the diagnoses were confirmed histologically. Eight patients presented with a solitary thyroid nodule. Six of these had intrathyroid branchial cleft remnants, and two had intrathyroid TDCs. The diagnosis became apparent at operation in six patients, while in two it was made by the pathologist after hemithyroidectomy. Ages at presentation ranged from 16 months to 14 years. The embryology of these neck structures is closely related. It should not come as a surprise that errors in their development may occur, at times paralleling the occurrence of intrathyroid location of parathyroid glands. The possibility of an embryologic rest in the neck should therefore be kept in mind with all clinically evident neck masses. TDCs and BCCs may coexist in the same patient. The histologic differentiation may be difficult in the presence of inflammation, but differences in structure are often characteristic.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

Pediatric liver transplantation: the Montreal experience.

The introduction of cyclosporine A in 1980 greatly improved the survival of children with end-stage liver disease undergoing orthotopic liver transplantation. The average 1-year survival rate following hepatic transplantation increased from 30% in 1963 to 70% in 1980. This report summarizes the initial experience of two pediatric hospitals in Montreal--Hôpital Ste-Justine and Montreal Children's Hospital. Since December 1985, 13 orthotopic liver transplantations have been performed in 11 patients: six females and five males. The median age was 18 months (range, 13 months to 17 years) and the median weight was 10 kg (range, 8.5 to 38 kg). The indications for transplantation were biliary atresia (5 patients), tyrosinemia (2 patients), biliary hypoplasia (1 patient), Amerindian cirrhosis (1 patient), Crigler-Najjar syndrome, type I (1 patient), and fulminant non-A, non-B hepatitis with grade IV encephalopathy (1 patient). Immunosuppression was ensured by cyclosporine A, azathioprine, and steroids. The function of 11 grafts was immediate following revascularization of the grafts. One graft had delayed function due to preservation injury, and one had primary nonfunction. Surgical complications included bile peritonitis (1), ruptured aneurysm of an aortic conduit (1), and thrombosis of the hepatic artery necessitating retransplantation (2). Three of our patients died, one from postoperative cerebral edema, one of primary nonfunction of the graft, and one of ruptured aneurysm of an aortic conduit. Our overall survival rate was 72% with a follow-up of 3 to 32 months. Pediatric liver transplantation can now provide successful treatment and cure of liver diseases considered, until recently, dismal and hopeless.

Adolescent↗

Malignant small-cell tumor of the thoracopulmonary region: 'Askin tumor'.

Malignant small-cell tumor of the thoracopulmonary region (MSCT) is an uncommon neoplasm in children. We describe five cases diagnosed since 1981 that fulfill the criteria put forth by Askin et al. Surgery was performed for diagnosis or therapy in all patients. Two patients underwent open lung biopsy, only because of tumor extent. The other three had chest wall resections. All patients received radiotherapy and chemotherapy. Three patients presenting initially with extensive disease died at intervals of 2.5 to 7 months after diagnosis. Two patients are alive and disease-free at 16 and 24 months postdiagnosis. All five cases were reviewed for standard histology and differential immunohistochemistry. Electronmicroscopy and tissue cultures were done in 3 of the 5 patients. All five patients were neuron-specific enolase-positive. MSCT is an exclusion diagnosis not always readily made. Clinical course and pathologic expertise may point to the correct diagnosis.

Adolescent↗

Congenital anal fistula with normal anus.

Three patients with a congenital anovestibular/perineal fistula were treated at the Montreal Children's Hospital. Two females (one of East Indian and the other of Japanese origin) had anovestibular fistulae that became symptomatic in the first few months of life. The third patient, a boy of Korean descent, presented at 9 months of age with a chronically draining perineal fistula. During surgery, a small fistula tract was easily dissected out and excised. Microscopic examination showed a well-preserved rectal mucosa throughout the tract. Most male patients described to date had anourethral fistulae, often accompanied by other major anomalies such as esophageal atresia or renal malformations. We believe our patient is the first male to be described with a congenital perineal fistula; this suggests that some fistula-in-ano in male infants may be due to a congenital sinus that secondarily becomes infected and drains to the skin.

Anus Diseases↗

Primary fetal hydrothorax: natural history and management.

Primary fetal hydrothorax presents a wide spectrum of severity ranging from small, harmless effusions, to life-threatening thoracic compression. To define natural history and management, we reviewed 32 cases seen at two large perinatal centers from 1980 to 1987. Spontaneous resolution of the effusions was seen in three fetuses, all of whom survived. Three fetuses were electively terminated. The overall mortality was 53%. In the 24 untreated fetuses, sex and the presence of polyhydramnios did not influence mortality, but hydrops, gestational age less than 35 weeks at delivery, and bilateral effusions were associated with a poor prognosis. Five fetuses underwent in utero decompression. In four, thoracentesis was performed, with rapid reaccumulation of the effusion. All four died from pulmonary insufficiency. In the fifth fetus, a thoracoamniotic shunt permanently decompressed the effusion, with resolution of the hydrops, and delivery of a normal viable infant. We conclude that (1) primary fetal hydrothorax may resolve or progress to hydrops, necessitating close follow-up with ultrasound; (2) pulmonary hypoplasia as a result of undrained large pleural effusions may result in neonatal mortality; (3) the gestational age at both diagnosis and delivery, the development of hydrops, and bilaterality of effusions are important prognostic predictors; and (4) the fetus with large effusions and hydrops has a poor prognosis, and thoracic decompression with a thoracoamniotic shunt may prove life saving.

Female↗

Pathogenic Escherichia coli: a new etiology for acute ileitis in children.

Acute ileitis in children is frequently diagnosed at the time of laparotomy for acute abdominal pain and is usually due to inflammatory bowel disease, Yersinia, or Campylobacter infections. We report a case in which a pathogenic strain of Escherichia coli was responsible for the disease. The diagnosis, microbiology, and epidemiology are discussed.

Abdomen, Acute↗

[Fixation anomaly of the duodenum. Apropos of 4 cases].

"Z"--shaped duodenum and "roller - coaster" duodenum are synonyms for malfixation of the duodenum. This rare malformation has been described recently. We report 4 cases (ages 3 months-9 years) with: --intermittent abdominal pain; --bilious vomiting; --failure to thrive. Radiologic examination of the upper gastrointestinal tract revealed a malformation of the distal duodenum (D2-D3) which presented a "Z" or "M" form and under fluoroscopy, this portion was very mobile, causing intermittent obstruction when it became kinked. All attempts of medical management have failed. At surgery, all the bands were cut, giving the duodenum a normal shape. The symptoms disappeared after surgery. Follow-up between 4 months and 3 years showed the patients were completely asymptomatic.

Child↗

Multiple-flap anoplasty in the treatment of rectal prolapse after pull-through operations for imperforate anus.

Rectal prolapse is a frequent complication after pull-through operations for high imperforate anus. Mucosal prolapse causes soiling, occasional bleeding, and pain. Simple resection of the redundant mucosa is unsatisfactory and leads to frequent recurrences or strictures. In 1982, Millard and Rowe reported a technique designed to correct rectal prolapse using two perineal flaps, thus providing a skin-lined anal canal. We have operated on two patients using the same technique. A three-flap anoplasty was used in nine other patients. With an average follow-up of 13 months, none of out patients presented recurrence of the prolapse or a significant stenosis. This procedure is safe and physiologically sound. The skin-lined anal canal provides some sensation where it is lacking. The functional and esthetic results are gratifying and we are now using the three-flap anoplasty as a primary procedure in the correction of high imperforate anus.

Adolescent↗

Pulmonary barotrauma in congenital diaphragmatic hernia: experimental study in lambs.

A left diaphragmatic hernia was created surgically in 20 fetal lambs between 93 and 110 days of gestation. Ten animals were alive with defects at cesarean section near term (135 to 140 days). These animals and two controls were submitted to various transpulmonary pressure gradients (inspiratory pressure minus pleural pressure). Hemodynamic and ventilatory studies were performed after the correction of the hernia. Morphometric analysis of the lung was carried out in all cases. The results showed a highly significant linear correlation between the transpulmonary pressure gradient employed and the pulmonary interstitial emphysema found at morphometry. Our data suggest that using low ventilatory pressures and not draining the pleural cavity results in less trauma to both lungs and may prevent one of the components of the pulmonary hypertension so often seen in newborns with congenital diaphragmatic hernia.

Animals↗

Bilateral Wilms' tumors: changing concepts in management.

Bilaterality is uncommon in Wilms' tumor, being present in 4% to 8% of the cases. We report the combined experience of two children's hospitals in one city over a 20-year period. We encountered nine cases of synchronous bilateral nephroblastoma (National Wilms' Tumor Study 3, stage V). Age at diagnosis ranged from 9 to 41 months (mean 23 months). There were five girls and four boys. Associated findings include nephroblastomatosis in three cases (33%), one of which also had a familial history; undescended testis in two cases; and minor anomalies in two other cases. Surgical treatment consisted of unilateral nephrectomy with contralateral partial nephrectomy or tumorectomy in six cases, nephrectomy with contralateral biopsy only in two cases, and the other patient had bilateral biopsies initially, followed at a later date by partial nephrectomy on one side. All patients received chemotherapy; actinomycin D (AMD) only was used in the oldest case, vincristine and AMD in five cases, to which was added cyclophosphamide in one case and adriamycin in two. Seven patients received radiation therapy. Seven out of the nine patients survived more than 2 years (77%); five are well, off chemotherapy, with no evidence of disease from 4 to 11 years after diagnosis. Two patients suffered from chronic renal failure and one died from complications after renal transplantation more than 19 years after diagnosis. The two patients who died from their disease presented with more advanced tumor. Therefore, the agressiveness of multimodal therapy can be tailored according to stage and histology, and effective chemotherapy allows maximal preservation of renal parenchyma in patients with stage I and II tumors.

Child, Preschool↗