Biomedical subjects
Publications and source records attributed to J M Laberge.
Primary aldosteronism in children under 16 years of age is usually due to adrenal hyperplasia. We could find only seven cases in the literature where it was caused by a solitary adrenal adenoma; all but one were female. We describe the case of a 10-year-old Caucasian boy in whom a unilateral adrenal adenoma was demonstrated by high resolution computed tomography (CT) alone. The literature is reviewed.
Diffuse retroperitoneal soilage following rectal laceration with a blunt object resulted in significant morbidity despite prompt recognition, parenteral antibiotic administration, and early surgical intervention. A septic postoperative course was complicated by perforation of the third portion of the duodenum and a diffuse suppurative peritonitis with multiple intra-abdominal abscesses. Closure of the incision at reexploration was facilitated by sewing Marlex to the fascia while a centrally located nylon zipper allowed access to the peritoneal cavity for further explorations and eventual ablation of the septic process.
Maternal safety and future reproduction potential are paramount considerations in fetal surgery. In a series of 102 fetal surgical procedures on time-dated pregnant monkeys, we examined the maternal complications, the potential for future pregnancy, and the effects of uterine staples used for hysterotomy and uterine closure. There were significant maternal complications associated with the development of the present techniques for fetal surgery including 3 maternal deaths, 5 uterine ruptures, and 5 cases of wound infection of dehiscence. All were preventable with knowledge gained from this experience. Compared with a control unoperated breeding colony, fetal surgery did not interfere with subsequent reproductive capacity in animals that had uterine closure with absorbable sutures. However, metal staple hysterotomy resulted in a markedly decreased fertility rate probably related to exposure of the endometrial cavity to a permanent foreign body. Although serious maternal complications occurred in this series, many are avoidable as we improve both our obstetric management and our anesthetic and surgical techniques. Prenatal intervention carries considerable maternal risk and should not be attempted clinically under any but the most rigorous conditions.
During the 15 years from 1970 to 1984, 38 infants, all with cystic fibrosis, were treated for meconium ileus at The Montreal Children's Hospital and Ste-Justine Hospital for Children. Thirteen patients (34%) had complicated meconium ileus that included 7 perforations (2 colon, 5 ileum), 4 volvulus, and 2 atresia with meconium pseudocyst. In this group, various operations were done: resection with primary anastomosis for atresia, or resection with enterostomy for peritonitis or volvulus. One died shortly after surgery. Of 25 patients with uncomplicated meconium ileus (66%), one died shortly after arrival from respiratory distress, leaving 24 patients available for study. Gastrografin enema was attempted on 20 patients with eight successes (40%). Of the remaining 16 patients with unresolved meconium ileus, nine were treated with laparotomy and ileostomy, and one with laparotomy and T-tube irrigation. Six patients were treated by laparotomy and enterotomy for irrigation with N-acetylcysteine and evacuation by Fogarty catheter, a technique not widely used. No one succumbed in this group. This latter method of management is recommended for patients with simple uncomplicated meconium ileus.
Sacrococcygeal teratoma (SCT) is being diagnosed before birth with increasing frequency. We were recently consulted about management of a 22-week fetus with SCT and reviewed our experience (6 cases) and the literature. We found that most fetal SCT present from 22 to 34 weeks gestation with a uterus enlarged by the tumor and/or associated polyhydramnios; although the American Academy of Pediatrics Surgical Section clinical classification is an important prognostic indicator in neonatal SCT, it does not appear to predict outcome in fetal SCT; associated chromosomal abnormalities or life threatening anomalies are rare; presentation after 30-weeks gestation is a relatively good prognostic sign with fetal survival, after planned cesarean delivery, in 6 of 8 cases; and hydrops and/or placentomegaly in association with fetal SCT predicts fetal demise soon after diagnosis with 7 of 7 cases dying in utero.
Deep vein thrombophlebitis (DVT), unrelated to intravenous catheters, is unusual in children. During the period between 1970 to 1984, 15 cases of spontaneous DVT were seen at The Montreal Children's Hospital. The female to male ratio was 2:1. Age ranged from 10 to 17 years. Venogram was positive in the 14 patients in whom it was done. Significant factors were related to the DVT in 12 patients: oral contraceptives, pelvic fracture, Ewing's sarcoma and chemotherapy, brain tumor, nephrotic syndrome, and ulcerative colitis (2, one of whom developed the DVT 3 months before the onset of gastrointestinal symptoms). The sites of thrombophlebitis encountered were left iliofemoral vein, right iliofemoral, right tibial, and left subclavian. Three patients developed pulmonary emboli, which were recurrent in two and required iliac vein ligation or inferior vena cava clipping. Treatment usually consisted of intravenous heparin followed by oral anticoagulants for 3 to 6 months. Two patients received streptokinase with good results. All the patients recovered. With a limited follow-up (6 months to 5 years), we have not seen any postphlebitic sequelae.
Between 1971 and 1985, 97 patients born with posterolateral diaphragmatic hernia (CDH) were treated in three different centers in Quebec and Montreal (Canada). A retrospective study of these cases shows that with appropriate measures the results can be much less gloomy than they used to be. The series is analysed in order to determine the effect of post-operative thoracic drainage, the effect of the high frequency low volume mechanical ventilation as well as the effect of the serious associated conditions present in a significant number of these patients. Our results indicate that the ipsilateral thoracic drainage has a clear deleterious effect when used systematically++. Out of 52 such patients, 17 survived (32.7%) and 35 died (67.3%); while out of 43 patients who received no thoracic drainage, 25 survived (58.1%) and 18 died (42.8%). This is statistically significant CHI2: (p less than 0.005). If the series is analysed after elimination of the cases with severe associated conditions and taking into account the advent of the high frequency low pressure mechanical ventilation, the figures are even more significant. Fifty-seven patients (57) fall in this category; 28 patients received an ipsilateral thoracic drainage and 29 did not. In the first group 11 survived (39.2%) and 17 died (60.7%) while in the no drainage group 22 survived (75.8%) and 7 died (24.1%). This is statistically significant (p less than 0.005). We believe that with simple measures, it is possible to prevent the return to fetal circulation in a significant number of patients born with CDH and to clearly increase to overall survival rate.
Selecting appropriate management for the fetus with obstructive uropathy depends on our ability to accurately assess the severity of existing renal damage and to predict the potential for recovery of renal function if the obstruction is relieved. Diagnosis and treatment would be markedly enhanced by a simple, safe, quantitative fetal renal function test. To answer the question of whether endogenous fetal creatinine clearance (CrC) is an accurate measure of glomerular filtration rate (GFR) in the obstructed fetal kidney, we compared fetal CrC to a standard test for GFR--iothalamate clearance (IC). Six fetal lambs underwent unilateral ureteral ligation at 60 to 63 days gestation (term = 145 days). The contralateral unobstructed kidneys served as a control. At a second operation at 113 to 120 days, renal function was measured by hourly split urine collections for determination of CrC, IC, and fractional sodium excretion on each side over a 4-hr study period. There was excellent correlation of CrC with IC in all kidneys (r = 0.997, P less than 0.001, y = 1.14 x). Compared to the control side, the obstructed fetal kidneys had significantly decreased GFR and abnormal tubular function with marked sodium loss.(ABSTRACT TRUNCATED AT 250 WORDS)
Successful preservation of small bowel by cryobiologic techniques would increase the feasibility of intestinal transplants. Immunosuppression by Cyclosporin A (CyA) has also increased interest in intestinal transplantation. We have investigated the effect of cryopreservation and immunosuppression in fetal rat intestinal transplantation. Segments of fetal bowel implanted isogeneically into the paravertebral gutter of young rats were found to grow in a high percentage of animals (53% to 100%). Segments frozen to -20 degrees C or -40 degrees C at two rates of cooling, grew isogeneically (50% to 89%), demonstrating the feasibility of cryopreservation. Histologic examination of this bowel showed preservation of structure. When these segments were cooled and implanted allogeneically, no immunosuppressive effect was found. Segments protected by daily CyA administration grew. No synergistic effect was seen by associating CyA and cryopreservation. These experiments suggest the possibility of creating fetal small bowel long-term banking.
Using a percutaneous approach, 18 of 20 staghorn calculi were completely removed from the upper urinary tract. The initial nephrostomy was performed in the radiology department with local anesthesia. Tract dilatation and stone removal ensued on the next day in the operating room under general anesthesia. Minor complications, including postoperative temperature spikes and pyelonephritis, occurred in seven patients. Two major complications, urosepsis and hemorrhage requiring transfusion, were encountered.
Recent widespread interest in a new approach to imperforate anus is ample testimony to the difficulty of the classical operations and to the unsatisfactory results. In the early seventies, Mollard began to use an anterior perineal approach to high imperforate anus as an alternative to the sacrococcygeal pull-through. It entails a semicircular retroscrotal incision allowing direct access and visualization of the urethra and puborectalis sling. Since 1976 we have switched to the anterior perineal approach. We now have experience with 30 patients, 27 males and 3 females. Two of the females had complicated cloacal anomalies, and three teenage patients were reoperated for incontinence following previous unsuccessful pull-throughs. Of the 27 primary operations, there were 17 with an uneventful early postoperative course. There were two major and eight moderate or minor complications. There was no mortality. A 3-year follow-up is available in 14 patients. The results are good in 8, fair in 4 and poor in 2. The advantages of this technique are threefold: it allows clear visualization and precise identification of the puborectalis sling; the dissection proceeds parallel to and with constant vision of the urethra, avoiding injury to it; and it obviates the need to change the position of the patient during the procedure. This operation is easier and safer than the sacral approach, it avoids cutting through the puborectalis sling as recently described, and, finally, it respects the criteria established by Stephens.
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UNLABELLED: Congenital cystic adenomatoid malformation of the lung (CCAM) is diagnosed by prenatal ultrasonography with an increasing frequency but controversy persists as to its prognosis and prenatal management. METHOD: A multi-institutional study of cases of CCAM diagnosed antenatally identified by ultrasonographers and by a review of hospital charts. RESULTS: We obtained 48 cases from five centers. We estimate the incidence of CCAM at 1:25,000 to 1:35,000 pregnancies. The incidence of voluntary abortions was 15% (7/48), of spontaneous abortions 2% (1/41) and of postnatal death 10% (4/40). One of the postnatal deaths was from trisomy 18. Of the 7 aborted fetuses, 2 had multiple malformations and 1 had severe hydrops and oligohydramnios; the other 4 had a large mass with mediastinal displacement but without hydrops. When pregnancy was allowed to continue, 56% of the lesions regressed spontaneously, even though one third of these had initial progression. In 17 cases (42%) the mediastinal shift corrected itself, sometimes by simple growth of the fetus but most often by a decrease in the size of the lung mass. In 1 fetus, repeated needle decompressions followed by double-pigtail catheter drainage of large cysts allowed regression of hydrops. Despite this, neonatal death occurred from pulmonary hypoplasia. CONCLUSION: CCAM can lead to fetal or neonatal demise from hydrops, lung hypoplasia, prematurity or severe associated malformations, but has a good prognosis in the majority of cases.
With the advent of modern neonatology and the survival of most premature infants, necrotizing enterocolitis of the newborn (NEC) has become a relatively frequent illness. NEC, although affecting mainly premies, may still be found in any infant, even full term ones. We therefore believe that it is important for all physicians to become somewhat familiar with this entity. The pathogenesis of NEC is comprised of several variables: mesenteric ischemia, gastrointestinal immaturity, enteral feedings and even possibly infection. A diagnosis of NEC is based on a combination of clinical and radiological grounds. On radiographs, pneumatosis intestinalis and air in the portal vein are of special significance. NEC is classified in three broad categories: suspected NEC, definite NEC and advanced NEC. The treatment is either medical or surgical, depending on the severity and the evolution of the disease. It is important to emphasize that any infant who is deteriorating deserves very tight clinical and radiological follow-up. This follow-up should take place in a center where pediatric surgeons are ready to intervene rapidly should there be a need. Even if in some cases NEC is very severe, sometimes fatal, approximately 85% of infants suffering from it survive and among them more than 70% do so without any long term sequelae.