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Biomedical subjects

Jean-Martin Laberge

Publications and source records attributed to Jean-Martin Laberge.

At least 19 recordsLinked to original sources

Management and outcome of patients with combined vaginal septum, bifid uterus, and ipsilateral renal agenesis (Herlyn-Werner-Wunderlich syndrome).

OBJECTIVE: Herlyn-Werner-Wunderlich syndrome (HWWS) is a rare müllerian anomaly consisting of uterine didelphy, hemivaginal septum, and ipsilateral renal agenesis. The purpose of this study was to evaluate the natural history and outcome of patients with HWWS. METHODS: With ethics review board approval, all patients with uterine/vaginal anomalies were reviewed between 1982 and 2004. Patients with cloacal and/or anorectal anomalies were excluded. Presenting symptoms, preoperative investigations, operative management, and long-term follow-up were assessed. RESULTS: Of 80 patients identified with uterine/vaginal anomalies, 12 had HWWS. Median age at presentation was 13 years. Most patients (11/12) in this series presented with either abdominal pain and/or pelvic masses. Two patients had intra-abdominal abscesses. Seven patients were menstruating at presentation with 4 of these patients having dysmenorrhea. Symptom duration ranged from 0.5 to 12 months. Diagnosis was confirmed by ultrasound (n = 11), computed tomographic scan (n = 3), and/or magnetic resonance imaging (n = 2). Operative management included vaginal septectomy and drainage of the hematocolpos/hematometrocolpos. One patient required salpingectomy for pyosalpinx. Follow-up ultrasounds revealed no recurrent collections. Median follow-up was 3 years (2 months to 16 years). Eleven patients were asymptomatic after treatment. One patient complained of irregular menses. CONCLUSION: This is one of the largest reviews of HWWS in pediatric patients to date. Good long-term outcome occurs after vaginal septectomy. This diagnosis should be suspected in females with a pelvic mass and ipsilateral renal agenesis.

Abnormalities, Multiple↗

The role of laparotomy for intestinal perforation in very low birth weight infants.

BACKGROUND/PURPOSE: The management of intestinal perforation in very low birth weight (VLBW) infants (less than 1500 g) is controversial. Current practice favors peritoneal drainage (PD) with or without a delayed laparotomy over primary laparotomy (PL). We compared the outcomes of PD +/- delayed laparotomy vs PL in VLBW infants using the Score for Neonatal Acute Physiology with Perinatal Extension (SNAPPE-II) as a validated predictor of mortality. METHODS: A retrospective analysis (1998-2003) of VLBW infants with intestinal perforation at 2 pediatric centers was undertaken. Data retrieval included neonatal demographics and parameters for SNAPPE-II calculation. The primary end point was 30-day mortality. Other outcome measures included in-hospital mortality, days fasting, days to extubation, and length of stay. Statistical analysis was performed with either Student's t test or chi2 analysis. Subgroup and multivariate analyses were also performed. P values < .05 were considered significant. RESULTS: Fifty-two neonates (25 PD, 27 PL) were reviewed. Overall, 10 (19.2%) infants died. Observed 30-day mortality rates in PD and PL groups were 32% and 7.4% (P = .028), respectively. Average SNAPPE-II scores for PD (42.5 +/- 20.8) and PL (25.1 +/- 14.6) groups yielded predicted mortality rates of 15.7% and 4.9% (P = .001), respectively. PD group 30-day mortality far exceeded the rate predicted by the SNAPPE-II score. Days fasting (13.7 vs 20.4; P = .0001), days to extubation (26.7 vs 51.5; P = .014), and length of stay (56.1 vs 83.6; P = .031) all favored the PL group despite incorporating SNAPPE-II score as a covariate into the multivariate analysis. Of the 25 patients receiving drainage, 9 underwent PD alone (SNAPPE-II = 46.6 +/- 27.9), whereas 16 patients underwent delayed laparotomy (SNAPPE-II = 37.8 +/- 17.6). The PD-only group had a greatly elevated mortality rate (77.8% vs 15.7% predicted), whereas the delayed laparotomy group had a reduced mortality rate (6.3% vs 9.3% predicted). CONCLUSION: Our data suggest that laparotomy, either alone or after PD, provides an improved outcome in VLBW infants with intestinal perforation. PD should be used as a temporizing measure until laparotomy can be performed.

Drainage↗

Type 1 pleuropulmonary blastoma in a 3-year-old male with a cystic lung mass.

Pleuropulmonary blastoma (PPB) is a rare and aggressive intrathoracic malignant tumor that can be associated with cystic lung lesions in children. This neoplasm is histologically characterized by primitive blastoma and a malignant mesenchymal stroma. The authors describe a 3-year-old boy who presented with a history of fever and cough. Radiological imaging demonstrated a large cystic lesion replacing the left lower lobe. The patient underwent thoracoscopic resection of the lesion. Interestingly, the histopathology demonstrated a type 1 PPB. Type 1 lesions are usually observed in young infants, whereas older infants and children tend to present with type 2 or 3 PPB, which carry a poorer prognosis and higher risk of recurrence. Thus, the presence of large or peripherally based lung cysts should raise the suspicion of PPB. Resection is warranted for all such lesions.

Antineoplastic Agents↗

Foregut duplications: is there an advantage to thoracoscopic resection?

PURPOSE: Thoracoscopy has an expanding role in the treatment of FD cysts (bronchogenic cysts and esophageal duplications). We examined this trend in our patients and reviewed our overall experience. METHODS: All charts of children undergoing surgery for foregut duplications (FDs) in 2 pediatric hospitals between 1992 and 2003 were retrospectively reviewed. Data gathered included age, weight, symptoms, diagnostic tests, operative technique, postoperative course, complications, and outcome. RESULTS: There were 39 children, with FD resected by thoracotomy in 21 patients, thoracoscopy in 11 patients (no conversions to open), cervical incision in 6 patients, and laparotomy in 1 patient for an FD near the gastroesophageal junction. Diagnosis was made by antenatal ultrasound in 7 cases. Four of these neonates had tachypnea or cough, and the rest were asymptomatic. Seventy-five percent of patients diagnosed postnatally presented with respiratory symptoms. Excision of isolated FD (without lung resection) was compared between those who had a thoracotomy (n = 16) vs thoracoscopy (n = 11). The age, weight, operating time, and anesthesia time were not different between the 2 groups. However, the thoracoscopy group had significantly fewer chest tube days (1.6 vs 3.3 days) and a shorter hospital stay (2.6 vs 6.6 days). Intraoperative complications consisted of tracheal injury in 3 patients (2 thoracotomy, 1 thoracoscopy) and esophageal mucosal injury in 2 patients (both thoracotomy), which were all recognized and repaired. CONCLUSION: Foregut duplications may present in a variety of ways and locations. Thoracoscopy is advantageous for isolated intrathoracic FDs.

Adolescent↗

Postnatal pulmonary distension for the treatment of pulmonary hypoplasia: pilot study in the neonatal piglet model.

BACKGROUND: Accelerated lung growth has previously been demonstrated after fetal tracheal occlusion. The purpose of this study was to determine if short-term perfluorocarbon (PFC) distension could increase lung growth postnatally in neonatal piglets. METHODS: Eleven piglets aged 5 to 8 days were divided into 3 groups: (a) controls (n = 4), (b) PFC x 6 hours (n = 3), and (c) PFC x 12 hours (n = 4). A right posterolateral thoracotomy was performed and a pressure-monitoring catheter was placed in the posterior segment of the right upper lobe. Perfluorocarbon was infused and a mean intrabronchial pressure of 12 mm Hg (range, 5-21 mm Hg) was maintained. The control piglets also had a thoracotomy with right upper lobe bronchus dissection without ligation or PFC distension. All piglets were injected with [3H]-thymidine 3 hours before killing. Both right and left posterior segments of each upper lobe were analyzed for their respective amount of total DNA by fluorometry. Rates of DNA synthesis for each segment were determined by precipitating incorporated [3H]-thymidine with 5% trichloroacetic acid and reporting this value by the total amount of DNA. The differential lung DNA synthesis rate was calculated as (right posterior segment/left posterior segment) x 100. Statistical analysis consisted of 1-way analysis of variance and Student's t tests with significance at P < or = .05. RESULTS: Heart rate, mean arterial pressure, temperature, oxygen saturation, pH, PCO2 , and PO2 were similar in all 3 groups. Lung DNA synthesis was nearly doubled in the PFC x 6 hours group compared with controls (302% vs 165%, P = .05). Animals in the PFC x 12 hours group experienced a 261% increase (P = NS). CONCLUSION: Short-term PFC distension in neonatal piglets resulted in increased DNA synthesis within 6 hours presumably because of stretch-induced mechanisms.

Animals↗

Asymptomatic congenital lung malformations.

Congenital lung malformations are often discovered incidentally on routine prenatal sonography or postnatal imaging. Lesions such as congenital cystic adenomatoid malformations (CCAM), sequestrations, bronchogenic cysts and congenital lobar emphysema may be asymptomatic at birth or at the time of discovery later in life. Some authors advocate simple observation because of the lack of data on the incidence of long-term complications. However, there are very few described cases where CCAM and intralobar sequestration have remained asymptomatic throughout life; complications eventually develop in virtually all patients. The most common complication is pneumonia, which may respond poorly to medical treatment. Other complications include the development of malignancies (carcinomas and pleuropulmonary blastomas), pneumothorax and hemoptysis or hemothorax. Since lung resection will be required sooner or later for CCAM, intralobar sequestration and intrapulmonary bronchogenic cysts it is best not to wait for complications to occur. For patients diagnosed prenatally, we recommend surgery at 3 to 6 months of life at the latest, so that compensatory lung growth can occur. At this age the postoperative course is usually smooth and long-term follow-up has shown normal respiratory function. Mediastinal bronchogenic cysts also tend to become symptomatic and elective resection is recommended. On the other hand, asymptomatic congenital lobar emphysema may regress spontaneously and observation is warranted. The management of small noncommunicating extralobar sequestrations is more controversial; it is known that these lesions can remain asymptomatic throughout life but complications may develop and they are sometimes difficult to differentiate from neuroblastoma.

Bronchogenic Cyst↗

Effect of temporary tracheal occlusion on the endothelin system in experimental cases of diaphragmatic hernia.

Previously, the authors have shown that tracheal occlusion (TO) partially reverses the onset of congenital diaphragmatic hernia (CDH)-induced pulmonary hypertension (PH) and abnormal pulmonary vascular development whereas release of the occlusion (TR) abolishes these clinical benefits. As a consequence of their mitogenic and vasoactive properties, the authors hypothesize that the expression of endothelin (ET)-1 and ET receptor (ETA) genes is increased in lungs of CDH lambs, and that this increase is abolished partially in CDH + TO but not in CDH + TO + TR. A surgical left-sided CDH was created in fetal lambs at 80 days of gestation (gd), followed by TO at 108 gd, and by TR at 129 gd. Four groups were compared: CDH, CDH + TO, CDH + TO + TR, and nonoperated controls (C). Assessment of mRNA expression by Northern blot showed significantly lower ET-1 and ETA levels in the CDH group than in the CDH + TO +/- TR groups (P < .05). Endothelin protein expression levels were lower in CDH +/- TO +/- TR groups when compared with controls for airways and vessels (P < .05) with the exception of endothelial cells. In contrast, ETA protein expression levels were higher in CDH +/- TO +/- TR groups compared with controls for airways and blood vessels smooth muscles (P < .05). These results suggest that involvement of the endothelin system in the pulmonary hypertension associated with CDH is limited. However, the endothelin system appears to be modulated during development.

Animals↗

Fetal tracheal occlusion in lambs with congenital diaphragmatic hernia: role of exogenous surfactant at birth.

Fetal tracheal occlusion (TO) has been used to reverse the lung hypoplasia associated with congenital diaphragmatic hernia (CDH). However, TO has a detrimental effect on type II pneumocyte function and surfactant production. Previously, we have shown that in surgically created CDH lambs, TO improved markedly the response to resuscitation even though the lungs remain surfactant deficient. The goal of this investigation was to assess the effects of exogenous surfactant administered at birth to CDH lambs with or without fetal TO during 8 h of resuscitation. Lambs were divided into five groups: CDH, CDH+surfactant (SURF), CDH+TO, CDH+TO+SURF, and nonoperated controls. A left-sided CDH was created in fetal lambs at 80 d gestation. TO was performed at 108 d, and the lambs were delivered by hysterotomy at 136 d. Bovine lipid extract surfactant was administered before the first breath and again at 4 h of life. All CDH+SURF lambs, but only three of five CDH lambs, survived up to 8 h. When compared with the corresponding nonsurfactant-treated group, surfactant-treated CDH and CDH+TO lambs did not demonstrate improved alveolar-arterial oxygen gradients, pH, or Pco(2). In fact, in the CDH+TO group, surfactant treatment significantly worsened ventilation efficiency as measured by the ventilation efficiency index. The observed improvement in pulmonary compliance secondary to surfactant treatment was not significant. This investigation demonstrates that prophylactic surfactant treatment at birth does not improve gas exchange or ventilation efficiency in CDH lambs with or without TO.

Animals↗

Day surgery for thyroglossal duct cyst excision: a safe alternative.

The goal of this study was to assess which factors affect length of stay in patients operated on for a thyroglossal duct cyst (TDC) and whether day surgery is a safe alternative to a postoperative admission. All charts of patients with TDC excisions at one children's hospital from 1995 to 2001 were reviewed retrospectively. Chi-square tests and multiple logistic regression were used as statistical tests, with p<0.05 considered significant. One hundred children with a mean age of 6 years were operated on for TDC; 46% had day-surgery, with a median length of stay (LOS) of 4 h, and the other 54% were admitted overnight after TDC excision, with a median LOS of 24 h. Factors that significantly prolonged LOS were drain placement, start of surgery after 1 p.m., and surgery being performed by the ENT surgical service rather than the pediatric general surgery service. There was only one hospital readmission for a patient with day surgery at 36 h postoperation for wound hematoma that resolved with nonoperative treatment. Day surgery is safe for routine TDC excisions. There must be a shift in surgeons' behavior if the trend towards an increase in outpatient services for TDC excisions is to continue.

Ambulatory Surgical Procedures↗

Routine cesarean delivery does not improve the outcome of infants with gastroschisis.

BACKGROUND/PURPOSE: The optimal mode of delivery for infants with gastroschisis is controversial. The authors compared the outcomes of infants with gastroschisis born vaginally (VD) or by cesarean section (CS). METHODS: A retrospective analysis of infants with gastroschisis born between 1990 and 2000 was performed. Assessment included patient demographics, respiratory distress, method of closure, number of surgeries, presence of atresia, feeding parameters, parenteral nutrition days (TPN), time to full feeding (FPO), mortality, and length of stay (LOS). Subgroup analyses were performed for those infants requiring cesarean section for fetal distress. Student's t test/analysis of variance (ANOVA) or chi2/Fisher's Exact tests were used for statistical analysis. Logistic and linear regression analyses were also performed. RESULTS: One hundred thirteen patients were studied (82 VD and 31 CS). No statistical difference existed between the VD and CS groups for perinatal complications, method of closure, number of surgeries (1.6 each), TPN (40.6 v 46.0 days), FPO (40.4 v 47.1 days), mortality (9.7 v 6.5%) and LOS (53.4 v 61.7 days). CS was associated with increased stenosis (25.8 v 4.9%; P =.003), gastrointestinal dysfunction (25.8 v 11.0%; P =.049), and respiratory distress (16.1 v 3.7%; P =.035). Many of these differences did not persist when infants undergoing CS for fetal distress were excluded from the analysis. However, regression analysis identified CS as an independent risk factor for the development of respiratory distress at birth (odds ratio, 7.11; CI, 1.06 to 47.7), with a trend to increased gastrointestinal dysfunction (odds ratio, 4.35; CI, 0.77 to 24.61). CONCLUSIONS: The routine use of CS for infants with gastroschisis is not supported by our results because equivalent outcomes were observed with both modes of delivery. CS may be a necessary intervention for fetal distress.

Cesarean Section↗

The significance of intrauterine growth restriction is different from prematurity for the outcome of infants with gastroschisis.

BACKGROUND/PURPOSE: Recent reviews of gastroschisis identify prematurity and low birth weight as predictors of morbidity and mortality. The authors compared the outcomes of intrauterine growth-restricted infants (IUGR) with gastroschisis to those without growth restriction because IUGR is different from prematurity. METHODS: A retrospective analysis was performed for infants born with gastroschisis between 1990 and 2000 at 2 pediatric hospitals. Patients were segregated into 3 groups based on birth weight corrected for gestational age: group 1 (IUGR, 25th percentile). Patient demographics, method of closure, number of surgeries, presence of atresia, and time to full enteral feedings (FPO days) were assessed. Mortality rate, length of stay (LOS), and readmission rates were also compared. Analysis of variance (ANOVA)/Student's t test and Fisher's. Exact tests were used for statistical analysis (P <.05 significant). Regression analysis was also performed. RESULTS: One hundred thirteen patients were included (group 1 = 17; group 2 = 43; group 3 = 53). Overall, infants with IUGR had similar outcomes to non-IUGR infants, including FPO and total parenteral nutrition (TPN) days, LOS, readmission, and mortality rates. The method of closure did not affect outcome. Infants with atresia had significantly increased times to full feeding (95 v 34 days; P =.034), more surgeries (2.7 v 1.4; P =.002), and longer LOS (106 v 48 days; P =.011). Infants born at less than 37 weeks' gestation had significantly increased fasting (NPO) days (28 v 18 days; P =.005) and longer LOS (65 v 37 days; P =.006) when compared with infants born at greater than 37 weeks. Logistic regression analysis identified the presence of atresia as an independent risk factor for gastrointestinal dysfunction and the need for prolonged TPN. Prematurity also adversely affected these same parameters, although it did not reach statistical significance. CONCLUSIONS: Although infants with gastroschisis are generally small for gestational age, the outcomes of growth-restricted infants are similar to those of other infants. The type of closure does not affect outcome, regardless of birth weight. The presence of atresia or prematurity does lead to longer times for full feeding and LOS. Therefore, routine premature delivery of infants with gastroschisis should not be advocated, even in the context of IUGR.

Adult↗

Lung function in lambs with diaphragmatic hernia after reversible fetal tracheal occlusion.

BACKGROUND/PURPOSE: Short-duration resuscitation (< or =4 hours) of lambs with diaphragmatic hernia treated in utero with tracheal occlusion have shown improved lung function compared with untreated diaphragmatic hernia. This may be a transient phenomenon in the treated diaphragmatic hernia lambs because of surfactant deficiency. Our objective was to analyze the effect of fetal tracheal occlusion with or without release of the occlusion 1 week before delivery on pulmonary function during a longer period of resuscitation (8 hours) in the diaphragmatic hernia lamb model. METHODS: Four groups were compared: diaphragmatic hernia (n = 5), diaphragmatic hernia and tracheal occlusion until delivery (n = 5), diaphragmatic hernia and tracheal occlusion with release of the occlusion 1 week before delivery (n = 5), and normal controls (n = 4). RESULTS: Despite persistently decreased surfactant levels, diaphragmatic hernia lambs treated with tracheal occlusion had normal-sized lungs with marked improvement in lung function and gas exchange over 8 hours when compared with untreated lambs with diaphragmatic hernia. Release of the tracheal occlusion 1 week before delivery added no benefit. CONCLUSIONS: It appears that surfactant-independent mechanisms such as pulmonary growth and structural changes are of foremost importance in relating to improved compliance, oxygenation, and ventilation of diaphragmatic hernia lambs treated with tracheal occlusion.

Animals↗

The management of asymptomatic congenital lung malformations.

Congenital Pulmonary Airway Malformation (CPAM) and lung sequestration are often discovered on routine prenatal sonography. While some lesions may be large and cause serious complications in the fetus or newborn, many will be asymptomatic at birth. Some authors advocate simple observation because of the lack of data on the incidence of long-term complications. However, there are very few described cases where CPAM and intralobar sequestration (ILS) have remained asymptomatic throughout life; complications eventually develop in virtually all patients. The most common complication is pneumonia, which may respond poorly to medical treatment. Other complications include the development of malignancies (carcinomas and pleuropulmonary blastomas), pneumothorax and hemoptysis or hemothorax. Since lung resection will be required sooner or later for CPAM and ILS, it is best not to wait for complications to occur. We recommend surgery at 3 to 6 months of life at the latest, to allow compensatory lung growth to occur. At this age the postoperative course is usually smooth and long-term follow- up has shown normal respiratory function. The management of small non- communicating extralobar sequestrations is more controversial; it is known that these can remain asymptomatic throughout life but complications may develop and they are sometimes difficult to differentiate from neuroblastoma.

Cystic Adenomatoid Malformation of Lung, Congenita↗

Appendicitis in children: a ten-year update of therapeutic recommendations.

BACKGROUND/PURPOSE: In 1990, the authors reported excellent outcomes using a standard protocol to treat pediatric appendicitis. This protocol has been simplified further and a large retrospective review was conducted to assess current outcomes. METHODS: All patients treated for presumed appendicitis between April 1997 and December 1999 were reviewed. All patients received preoperative gentamicin and clindamycin. Patients with complicated appendicitis received postoperative ampicillin, gentamicin, and clindamycin or metronidazole. All wounds were closed primarily without drains. Patients with complicated appendicitis were discharged when their ileus resolved, they remained afebrile for 24 hours, and had a normal leukocyte count. RESULTS: A total of 648 patients were reviewed. A total of 9.4% of appendices were pathologically normal, 55.6% were simple acute, 15.7% were gangrenous, and 19.3% were perforated. Hospital stay was 2.21 +/- 2.04 days for normal, 1.39 +/-.89 for simple acute, 2.97 +/- 1.25 for gangrenous, and 6.31 +/- 3.51 days for perforated appendices. There were no wound infections in patients with normal or simple acute appendices. Two minor intraabdominal infections (0.56%) occurred in patients with simple appendicitis. Patients with complicated appendicitis (gangrenous or perforated) had wound infection and intraabdominal infection rates of 2.6% and 4.4%, respectively. CONCLUSIONS: The authors' current protocol results in reasonable hospital stays and good outcomes. It serves as an evidence-based standard of care for the treatment of pediatric appendicitis.

Abdominal Abscess↗

Pediatric spontaneous pneumothorax.

The aim of this study was to review pediatric spontaneous pneumothorax and describe diagnostic and therapeutic approaches and to review our institutional experience with spontaneous pneumothorax. A medline search using keywords pneumothorax and pediatric in both French and English from 1988 through May 2002 was performed, and pertinent papers regarding the natural history, diagnosis, imaging, and therapy were reviewed. A 10-year chart review of spontaneous pneumothorax recording data on demographics, diagnostic imaging, therapeutic approach, and outcomes was performed. Only 4 case series and one review article were produced by the medline search involving exclusively pediatric spontaneous pneumothrax. One hundred ninety-six other articles involved adult series with a minority of patients under 18 years of age. A subset of articles from this adult-based group were examined to assist in formulating recommendations for diagnosis, imaging, treatment, and surgical management of pediatric spontaneous pneumothorax. Few articles on this topic report randomized studies in the adult literature and none in the pediatric literature. The chart review yielded 53 cases of spontaneous pneumothorax in 40 patients with important data presented in the current review. Although few articles are able to provide best-evidence information, the diagnosis, imaging, treatment, and surgical management of patients with primary spontaneous pneumothorax is achieved with good results. Stronger data specific to pediatric patients are needed.

Adolescent↗