Sarah Bouchard - Academia.edu (original) (raw)

Papers by Sarah Bouchard

Research paper thumbnail of It is all about form and function: a new approach to giant neonatal sacrococcygeal teratomas

Pediatric Surgery International, Apr 18, 2023

Research paper thumbnail of Perianal rhabdomyosarcoma presenting 21 months after hamartoma excision

Pediatric Surgery International, Mar 20, 2012

Research paper thumbnail of Tu-be or not tu-be? Is routine endotracheal intubation necessary for successful bedside reduction and primary closure of gastroschisis?

Journal of Pediatric Surgery, 2022

BACKGROUND Wide practice variation exists in the management of gastroschisis. Routine endotrachea... more BACKGROUND Wide practice variation exists in the management of gastroschisis. Routine endotracheal intubation for bedside closure may lead to longer duration of mechanical ventilation. METHODS The Canadian Association of Pediatric Surgery Network gastroschisis dataset was queried for all patients undergoing attempted bedside reduction and closure. Patients with evidence of intestinal necrosis or perforation were excluded. A propensity score analysis was used to compare the rate of successful primary repair and post-operative outcomes between intubated and non-intubated patients. RESULTS In propensity score matched analysis, the successful primary repair rate did not reach statistical significance between patients who were intubated for attempted bedside closure and those who were not intubated (Odds Ratio: 2.18, 95% Confidence Interval: 0.79, 6.03). Intubated patients experienced 3.02 more ventilator days than patients who were not intubated at the time of initial attempted closure. Other post-operative parameters were similar between both groups. CONCLUSIONS It is reasonable to attempt primary bedside gastroschisis closure without intubation in otherwise healthy infants.

Research paper thumbnail of Les volumineuses tumeurs rénales nécrotiques et hémorragiques ne sont pas toutes malignes même en pédiatrie : à propos d’un cas chez un enfant de 7 mois

Annales de Pathologie, 2017

Research paper thumbnail of Implementing a standardized gastroschisis protocol significantly increases the rate of primary sutureless closure without compromising closure success or early clinical outcomes

Journal of Pediatric Surgery, 2021

PURPOSE Standardized protocols have been shown to improve outcomes in several pediatric surgical ... more PURPOSE Standardized protocols have been shown to improve outcomes in several pediatric surgical conditions. We implemented a multi-disciplinary gastroschisis practice bundle at our institution in 2013. We sought to evaluate its impact on closure type and early clinical outcomes. METHODS We performed a retrospective review of uncomplicated gastroschisis patients treated at our institution between 2008-2019. Patients were divided into two groups: pre- and post-protocol implementation. Multivariate logistic regression was used to compare closure location, method, and success. RESULTS Neonates (pre-implementation n = 53, post-implementation n = 43) were similar across baseline variables. Successful immediate closure rates were comparable (75.5% vs. 72.1%, p = 0.71). The proportion of bedside closures increased significantly after protocol implementation (35.3% vs. 95.4%, p < 0.01), as did the proportion of sutureless closures (32.5% vs. 71.0%, p < 0.01). Median postoperative mechanical ventilation decreased significantly (4 days IQR [3, 5] vs. 2 days IQR [1, 3], p < 0.01). Postoperative complications and duration of parenteral nutrition were equivalent. After controlling for potential confounding, infants in the post-implementation group had a 44.0 times higher odds of undergoing bedside closure (95% CI: 9.0, 215.2, p < 0.01) and a 7.7 times higher odds of undergoing sutureless closure (95% CI: 2.3, 25.1, p < 0.01). CONCLUSIONS Implementing a standardized gastroschisis protocol significantly increased the proportion of immediate bedside sutureless closures and decreased the duration of mechanical ventilation, without increasing postoperative complications. Level of Evidence III Type of Study Retrospective comparative study.

Research paper thumbnail of Prenatal prediction of survival in congenital diaphragmatic hernia: An audit of postnatal outcomes

Journal of Pediatric Surgery, 2019

Research paper thumbnail of Predicting Perinatal Outcome from Prenatal Ultrasound Characteristics in Pregnancies Complicated by Gastroschisis

Fetal diagnosis and therapy, Jan 2, 2015

The objective of the study was to establish the predictive value of prenatal ultrasound markers f... more The objective of the study was to establish the predictive value of prenatal ultrasound markers for complex gastroschisis (GS) in the first 10 days of life. In this retrospective cohort study over 11 years (2000-2011) of 117 GS cases, the following prenatal ultrasound signs were analyzed at the last second- and third-trimester ultrasounds: intrauterine growth restriction, intra-abdominal bowel dilatation (IABD) adjusted for gestational age, extra-abdominal bowel dilatation (EABD) ≥25 mm, stomach dilatation, stomach herniation, perturbed mesenteric circulation, absence of bowel lumen and echogenic dilated bowel loops (EDBL). Among 114 live births, 16 newborns had complex GS (14.0%). Death was seen in 16 cases (13.7%): 3 intrauterine fetal deaths, 9 complex GS and 4 simple GS. Second-trimester markers had limited predictive value. Third-trimester IABD, EABD, EDBL, absence of intestinal lumen and perturbed mesenteric circulation were statistically associated with complex GS and death. ...

Research paper thumbnail of Efficient Transduction of Liver and Muscle after in Utero Injection of Lentiviral Vectors with Different Pseudotypes

Research paper thumbnail of The gastroschisis prognostic score: reliable outcome prediction in gastroschisis

Journal of Pediatric Surgery, 2012

Background/Purpose: Disease-specific outcome predictors are required for gastroschisis. We derive... more Background/Purpose: Disease-specific outcome predictors are required for gastroschisis. We derived and validated a gastroschisis prognostic score (GPS) based on bowel appearance after birth. Methods: Visual scoring of bowel matting, necrosis, atresia, and perforation generated a novel gastroschisis bowel injury score recorded in a national database. Reweighting of score components by regression analysis led to assessments of model calibration and goodness of fit. The GPS was validated in subsequent cases. Results: Records from 225 infants were used for model derivation. Only intestinal necrosis independently predicted mortality by regression analysis (odds ratio, 11.5; 95% confidence interval, 4.2-31.4). Model recalibration identified that a GPS of 4 or more predicted mortality in 75% of nonsurvivors and 99% of survivors (P = .0001). A GPS of 2 or more demonstrated significantly worse survival outcomes compared with scores of 0 or 1 (length of stay: P = .011, days to first enteral feed: P = .013, days on total parenteral nutrition: P = .006). Model validation with 184 new patients yielded continued high-quality discrimination of outcomes. The GPS demonstrated "near-perfect" interobserver reliability between 2 surgeons (κ ≥ 0.86).

Research paper thumbnail of Canadian Pediatric Surgical Network: a population-based pediatric surgery network and database for analyzing surgical birth defects. The first 100 cases of gastroschisis

Journal of Pediatric Surgery, 2008

Research paper thumbnail of Pediatric surgeons' activities and future plans

Journal of Pediatric Surgery, 1997

Research paper thumbnail of Expression of neuropeptides in normal and abnormal appendices

Journal of Pediatric Surgery, 2001

Increased neuroproliferation in the appendix associated with an increase in substance P (SP), vas... more Increased neuroproliferation in the appendix associated with an increase in substance P (SP), vasoactive intestinal polypeptide (VIP), and growth-associated protein-43 (GAP-43) has been documented in appendices of adults with acute right lower quadrant (RLQ) abdominal pain and absence of gross or histologic signs of appendiceal inflammation. The authors tested whether these findings were present in children with RLQ pain and a normal appendix. Immunohistochemistry staining of paraffin-embedded appendices was performed with GAP-43, VIP, and SP. The positive control group included appendices with acute inflammation (group I, n = 5); the negative control group included appendices removed incidentally (group II, n = 5); and the experimental group included appendices from children suspected to have acute appendicitis without histologic signs of inflammation (group III, n = 9). Group I: VIP was strongly expressed in the nerve plexuses. The lamina propria and muscularis showed absent or minimal VIP expression. SP staining was strong in all plexuses and was moderate to strong in the muscularis. SP expression in the epithelium and lamina propria was difficult to quantify secondary to inflammation. Group II: VIP expression was essentially undetectable in the epithelium, lamina propria, and muscularis, and was moderate in the nerve plexuses. Mild SP staining was detected in the nerve plexuses of most specimens, and absent to mild staining was found in the epithelium and muscularis. However, one specimen strongly expressed SP in all layers. Group III: VIP expression was moderate to strong in the lamina propria and muscularis of nearly all specimens, and strong expression was found in all nerve plexuses. All but one specimen strongly expressed SP in plexuses. There was moderate to strong expression of SP in the epithelium, lamina propria, and muscularis in over 50% of specimens. The immunostaining for GAP-43 was very weak and nonspecific and did not help discriminate between the 3 study groups. Increased neuroproliferation in the lamina propria and muscularis was evident in patients with abdominal pain and normal appendices compared with appendices removed incidentally. The VIP and SP expression in these patients was similar or higher than that observed in patients with acute inflammation on histology.

Research paper thumbnail of Pulmonary lymphangiectasia revisited

Journal of Pediatric Surgery, 2000

Pulmonary lymphangiectasia (PL) is a rare, poorly documented disease characterized by abnormal pu... more Pulmonary lymphangiectasia (PL) is a rare, poorly documented disease characterized by abnormal pulmonary lymphatics. Although case reports are published, little is known about survivors past the neonatal period. Methods: This is a retrospective review of histologically proven PL in fetuses, infants, and long term survivors since 1965. Results: Eleven children (8 boys, 3 girls) and 8 aborted fetuses (7 male, 1 female) were identified. The fetuses weighed 463.4 g (177 to 681 g). Six were aborted between 19 to 24 weeks of gestation for multiple malformations or anencephaly, and 2 spontaneously aborted: one with PL only, the other with twin-twin transfusion syndrome. Clinical PL was diagnosed between 0 and 11 months of age. Six children died (2 neonatal, 4 within 10 days), 5 survived. Two deaths occurred after cardiac surgery. Among survivors, the symptomatology and frequency of admissions diminished over time. Symptoms included progressive respiratory distress, chronic cough, recurrent pneumonia, bronchial asthma, and choking. One child with bilateral chylothorax was later diagnosed with Noonan syndrome; 2 patients had minor cardiac malformations. Rapid deterioration occurred with mild respiratory infections with only supportive treatment available. Chest x-ray showed marked hyperinflation with interstitial infiltrate. Conclusions: This is the first long-term study of primary PL and will help counsel parents. Although fatal in the neonatal period, survival is possible if diagnosed past the neonatal period and improvement is expected.

Research paper thumbnail of Correction of hindbrain herniation and anatomy of the vermis after in utero repair of myelomeningocele in sheep

Journal of Pediatric Surgery, 2003

In utero repair of myelomeningocele (MMC) in humans spares distal neurologic function, reverses t... more In utero repair of myelomeningocele (MMC) in humans spares distal neurologic function, reverses the hindbrain herniation component of the Arnold-Chiari II malformation (ACM), and reduces the rate of postnatal shunt placement. The authors hypothesized that extravasation of cerebrospinal fluid (CSF) from the lumbar spinal cord results in herniation. This hypothesis was tested by assessing the impact of a spinal cord myelotomy on hindbrain anatomy in fetal sheep. A MMC lesion was created surgically in 34 fetal sheep at 75 days&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;#39; gestation by excision of the L1-L5 lamina, the exposed dura, and surrounding tissues. A lumbar level myelotomy was performed in 28 of the 34 fetuses to open the central canal of the spinal cord to enhance egress of CSF through the MMC defect and potentially induce hindbrain herniation. At 102 days&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;#39; gestation, a repair of the MMC lesion was performed in 14 fetuses with a myelotomy. Fetuses underwent autopsy at 102, 114, 120, or 140 days&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;#39; gestation. Control animals underwent 2 unrelated fetal surgical procedures at approximately 70 and 110 days&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;#39; gestation. The incidence of hindbrain herniation, ventricular size, biparietal diameter, brain weight, and brain anatomy were compared between the different animal groups. After MMC creation, significant cerebellar tonsillar herniation was observed in 85% of fetuses that underwent creation of a myelotomy; none of the lambs without a myelotomy (n = 6) had hindbrain herniation. At autopsy, cerebellar tonsillar herniation was present at the time of MMC repair (102 days&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;#39; gestation), 2 weeks after MMC repair, but was reversed 3 weeks post-MMC repair. At birth, tonsillar herniation was absent, and hindbrain anatomy was restored in 88% of the fetuses with a myelotomy that underwent fetal MMC repair. No significant differences in brain weight and ventricular size was observed between animals with and without MMC repair. Adding a myelotomy to the sheep model of MMC leads to hindbrain herniation that is similar to that observed in the human ACM. These experiments support the hypothesis that leakage of CSF through the exposed central canal alters the normal CSF hydrodynamics, resulting in cerebellar tonsillar herniation. Fetal MMC repair reverses hindbrain herniation and restores gross anatomy of the vermis.

Research paper thumbnail of A contemporary evaluation of pulmonary function in children undergoing lung resection in infancy

Journal of Pediatric Surgery, 2011

Background/Purpose: The management of asymptomatic congenital lung lesions is controversial. Some... more Background/Purpose: The management of asymptomatic congenital lung lesions is controversial. Some centers recommend resection in infancy, and others prefer observation. Our objective was to evaluate the pulmonary function of children who underwent lung resection at 12 months or younger. We hypothesized that these children would not have a significant reduction in pulmonary function when compared with norms for age. Methods: All patients at 2 tertiary-care children's hospitals who underwent lung resection at 12 months or younger and are currently older than 5 years were identified and prospectively recruited. Pulmonary function testing was standardized in all patients. Results: Fourteen children were tested prospectively, whereas results were available for another 5 children. Four children were excluded for inability to perform pulmonary function testing (n = 2) or for preexisting pulmonary hypoplasia/syndrome (n = 2). Pulmonary function testing values were considered normal if they were more than 80% of predicted. Forced vital capacity was normal in 14 (93%) of 15 children, and forced expiratory volume in 1 second was normal in 13 (86%) of 15 children. Diffusion capacity and respiratory muscle strength were normal in all children tested. Conclusions: Most children undergoing lung resection in infancy will have normal pulmonary function tests, supporting our philosophy of early, elective resection of congenital lung lesions.

Research paper thumbnail of The EXIT procedure: Experience and outcome in 31 cases

Journal of Pediatric Surgery, 2002

The EXIT (ex utero intrapartum treatment) procedure, although initially designed for reversal of ... more The EXIT (ex utero intrapartum treatment) procedure, although initially designed for reversal of tracheal occlusion in fetuses with congenital diaphragmatic hernias (CDH), has been adapted to treat a variety of fetal conditions. A retrospective chart review of all consecutive EXIT procedures since 1996 was conducted. Thirty-one women underwent the EXIT procedure, with an average maternal age of 29 years (range, 20 to 38), and average gestational age of 34 weeks (range, 29 to 40). The indication was airway obstruction from fetal neck mass in 13, and reversal of tracheal occlusion from in utero clipping in 13. Singular indications included an EXIT-to-ECMO (extracorporeal membrane oxygenation) procedure for a fetus with CDH and a cardiac defect (n = 1), congenital high airway obstruction syndrome (CHAOS, n = 1), resection of a very large congenital cystic adenomatoid malformation of the lung (CCAM) on uteroplacental bypass (n = 1), unilateral pulmonary agenesis (n = 1), and thoracoomphalopagus conjoined twins. The mean duration on uteroplacental bypass (from uterine incision to umbilical cord clamping) was 30.3 plus minus 14.7 minutes (range, 8 to 66). No fetus experienced hemodynamic instability during uteroplacental bypass as recorded by fetal heart rate (FHR), pulse oximeter, and fetal echocardiography, except for one instance of reversible bradycardia from umbilical cord compression. The mean FHR and fetal saturation were 153.0 plus minus 38.5 beats per minute and 71.2% plus minus 19.9%, respectively. Five fetuses required a tracheostomy. Only 1 death occurred during an EXIT procedure because of inability to secure the airway secondary to extensive involvement by a lymphangioma. The average cord pH and pCO(2) were, respectively, 7.20 plus minus 0.11 and 63.2 plus minus 14.6. Two maternal complications occurred: bleeding from a hysterotomy site and dehiscence of an old hysterotomy scar noticed at a subsequent cesarean section. The average maternal blood loss was 848.3 plus minus 574.1 mL. The EXIT procedure was used successfully to ensure uteroplacental gas exchange and fetal hemodynamic stability during a variety of surgical procedures performed to secure the fetal airway or ensure successful transition to postnatal environment.

Research paper thumbnail of Megarectum after surgery for anorectal malformations

Journal of Pediatric Surgery, 2010

Megarectum complicating surgery for anorectal malformation (ARM) has implications for long-term c... more Megarectum complicating surgery for anorectal malformation (ARM) has implications for long-term continence. Factors influencing continence and defecation include intact rectal reservoir, innervation/proprioception of the anorectal muscle complex, functioning anorectal inhibitory reflex (AIR), and intact perception at the anal margin. We studied outcomes after surgery for ARM with emphasis on megarectum; particularly as to whether altered rectal proprioception from anatomic sacrococcygeal anomalies affect incidence. We also assessed whether an abnormal AIR could trigger passive rectal dilatation without mechanical obstruction. Methods: Eighty six infants (53 male) with ARM over 20 years were included. Demographics, surgical history, pathology, defecation patterns, imaging, manometry, and morbidity were analyzed. Incidence of sacrococcygeal malformations in children with and without megarectum was compared using Fisher exact test. Manometry results were evaluated for integrity of AIR and correlated to megarectum occurrence. Results: There were 23 high/intermediate and 63 low ARMs. Fourteen (16%) developed a megarectum: 6 of 23 in high and 8 of 63 in low anomalies (P = .33). Twelve patients underwent megarectum resection at a median of 2.6 years (7 months to 10 years); 2 received bowel management protocols. Fifty-seven percent (8/14) of children with and 7% (5/72) without megarectum had sacrovertebral anomalies (P = .0001). Patients with preoperative manometry (n = 5) demonstrated an intact AIR. Colonic manometry demonstrated hyperactive colons (n = 2). Constipation was the predominant preoperative symptom; 3 patients suffered from incontinence after resection. All the specimens showed normal innervation and thickened muscularis on pathology. Conclusions: Sacral anomalies, which are more prevalent in children who developed megarectum, may result in abnormal rectal proprioception contributing to this pathology. Innervation anomalies may coexist, although preoperative manometries showed normal AIRs. Rectal dysmotility may lead to stool retention with subsequent dilatation, and patients who underwent colonic manometry had diffuse colonic hypermotility. Further physiologic and cellular studies are needed to elucidate the causes of this significant complication after surgical ARM repair in the absence of obstruction.

Research paper thumbnail of Gastroschisis closure—does method really matter?

Journal of Pediatric Surgery, 2008

Research paper thumbnail of Long-term transgene expression in cardiac and skeletal muscle following fetal administration of adenoviral or adeno-associated viral vectors in mice

The Journal of Gene Medicine, 2003

In utero gene transfer may provide advantages for the correction of congenital genetic disorders.... more In utero gene transfer may provide advantages for the correction of congenital genetic disorders. In the present study we compare the ability of adenovirus (AdCMVLacZ), and two serotypes of adeno-associated virus (AAVCMVLacZ serotypes 2 and 2/5), to target cardiac and skeletal muscle after prenatal systemic or intramuscular injection in mice and assess the immune response to the vectors. Day 14 gestation fetal mice underwent direct intraperitoneal or intramuscular injection of AdCMVLacZ, and AAVCMVLacZ serotypes 2 and 2/5 vectors. Tissues were processed for beta-galactosidase expression in frozen or high-resolution thin plastic sections at early and late time points. Neutralizing antibodies to Ad and AAV were analyzed in separate fetal experimental and neonatal or adult control groups after administration and re-administration of the vectors. A single injection of each vector in utero resulted in sustained expression of beta-galactosidase transgene in skeletal and cardiac muscle. Transgene expression was detected for the length of the study, i.e. 86, 58, and 31 weeks after birth for AdCMVLacZ, and AAVCMVLacZ serotypes 2 and 2/5, respectively. High-level expression in the myocardium was observed independent of the vector or route of administration. Neutralizing antibody responses to AAV and Ad antigens were reduced and long-term expression in muscle was not ablated on postnatal re-administration of vector. Sustained, high-level cardiac and skeletal muscle transgene expression can be obtained after prenatal gene transfer with each of these vectors. The potential for immune response to viral antigens is altered, but not entirely ablated after in utero exposure.

Research paper thumbnail of Chondroid Cystic Malformation of the Lung With Trisomy 8 Mosaicism: A New Cystic Lung Malformation

American Journal of Surgical Pathology, 2008

Neonatal cystic disorders of the lungs are a heterogeneous malformative group including giant lob... more Neonatal cystic disorders of the lungs are a heterogeneous malformative group including giant lobar hyperinflation, congenital pulmonary airway malformations, intralobar pulmonary sequestration, and bronchogenic cyst. Here, we describe a giant cystic pulmonary malformation in a 5-year-old girl, morphologically characterized by a highly disorganized proliferation of numerous cartilage islands, abundant mesenchymal tissue with abundant adipose differentiation, and epithelium-lined cysts. Cytogenetic analysis revealed an isolated trisomy 8, as the sole karyotype anomaly, a finding further confirmed by a whole-genome single nucleotide polymorphism array genotyping. The trisomy 8 was observed by fluorescent in situ hybridization within the malformation, and also in adjacent pulmonary parenchyma. A search of the literature revealed only 2 cases having similarities with the present case, but bearing different names. We believe that this lesion differs from congenital pulmonary airway malformations and from adult-type pulmonary hamartomas. We propose for this malformative mass the name &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;quot;chondroid cystic malformation of the lung.&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;quot;

Research paper thumbnail of It is all about form and function: a new approach to giant neonatal sacrococcygeal teratomas

Pediatric Surgery International, Apr 18, 2023

Research paper thumbnail of Perianal rhabdomyosarcoma presenting 21 months after hamartoma excision

Pediatric Surgery International, Mar 20, 2012

Research paper thumbnail of Tu-be or not tu-be? Is routine endotracheal intubation necessary for successful bedside reduction and primary closure of gastroschisis?

Journal of Pediatric Surgery, 2022

BACKGROUND Wide practice variation exists in the management of gastroschisis. Routine endotrachea... more BACKGROUND Wide practice variation exists in the management of gastroschisis. Routine endotracheal intubation for bedside closure may lead to longer duration of mechanical ventilation. METHODS The Canadian Association of Pediatric Surgery Network gastroschisis dataset was queried for all patients undergoing attempted bedside reduction and closure. Patients with evidence of intestinal necrosis or perforation were excluded. A propensity score analysis was used to compare the rate of successful primary repair and post-operative outcomes between intubated and non-intubated patients. RESULTS In propensity score matched analysis, the successful primary repair rate did not reach statistical significance between patients who were intubated for attempted bedside closure and those who were not intubated (Odds Ratio: 2.18, 95% Confidence Interval: 0.79, 6.03). Intubated patients experienced 3.02 more ventilator days than patients who were not intubated at the time of initial attempted closure. Other post-operative parameters were similar between both groups. CONCLUSIONS It is reasonable to attempt primary bedside gastroschisis closure without intubation in otherwise healthy infants.

Research paper thumbnail of Les volumineuses tumeurs rénales nécrotiques et hémorragiques ne sont pas toutes malignes même en pédiatrie : à propos d’un cas chez un enfant de 7 mois

Annales de Pathologie, 2017

Research paper thumbnail of Implementing a standardized gastroschisis protocol significantly increases the rate of primary sutureless closure without compromising closure success or early clinical outcomes

Journal of Pediatric Surgery, 2021

PURPOSE Standardized protocols have been shown to improve outcomes in several pediatric surgical ... more PURPOSE Standardized protocols have been shown to improve outcomes in several pediatric surgical conditions. We implemented a multi-disciplinary gastroschisis practice bundle at our institution in 2013. We sought to evaluate its impact on closure type and early clinical outcomes. METHODS We performed a retrospective review of uncomplicated gastroschisis patients treated at our institution between 2008-2019. Patients were divided into two groups: pre- and post-protocol implementation. Multivariate logistic regression was used to compare closure location, method, and success. RESULTS Neonates (pre-implementation n = 53, post-implementation n = 43) were similar across baseline variables. Successful immediate closure rates were comparable (75.5% vs. 72.1%, p = 0.71). The proportion of bedside closures increased significantly after protocol implementation (35.3% vs. 95.4%, p < 0.01), as did the proportion of sutureless closures (32.5% vs. 71.0%, p < 0.01). Median postoperative mechanical ventilation decreased significantly (4 days IQR [3, 5] vs. 2 days IQR [1, 3], p < 0.01). Postoperative complications and duration of parenteral nutrition were equivalent. After controlling for potential confounding, infants in the post-implementation group had a 44.0 times higher odds of undergoing bedside closure (95% CI: 9.0, 215.2, p < 0.01) and a 7.7 times higher odds of undergoing sutureless closure (95% CI: 2.3, 25.1, p < 0.01). CONCLUSIONS Implementing a standardized gastroschisis protocol significantly increased the proportion of immediate bedside sutureless closures and decreased the duration of mechanical ventilation, without increasing postoperative complications. Level of Evidence III Type of Study Retrospective comparative study.

Research paper thumbnail of Prenatal prediction of survival in congenital diaphragmatic hernia: An audit of postnatal outcomes

Journal of Pediatric Surgery, 2019

Research paper thumbnail of Predicting Perinatal Outcome from Prenatal Ultrasound Characteristics in Pregnancies Complicated by Gastroschisis

Fetal diagnosis and therapy, Jan 2, 2015

The objective of the study was to establish the predictive value of prenatal ultrasound markers f... more The objective of the study was to establish the predictive value of prenatal ultrasound markers for complex gastroschisis (GS) in the first 10 days of life. In this retrospective cohort study over 11 years (2000-2011) of 117 GS cases, the following prenatal ultrasound signs were analyzed at the last second- and third-trimester ultrasounds: intrauterine growth restriction, intra-abdominal bowel dilatation (IABD) adjusted for gestational age, extra-abdominal bowel dilatation (EABD) ≥25 mm, stomach dilatation, stomach herniation, perturbed mesenteric circulation, absence of bowel lumen and echogenic dilated bowel loops (EDBL). Among 114 live births, 16 newborns had complex GS (14.0%). Death was seen in 16 cases (13.7%): 3 intrauterine fetal deaths, 9 complex GS and 4 simple GS. Second-trimester markers had limited predictive value. Third-trimester IABD, EABD, EDBL, absence of intestinal lumen and perturbed mesenteric circulation were statistically associated with complex GS and death. ...

Research paper thumbnail of Efficient Transduction of Liver and Muscle after in Utero Injection of Lentiviral Vectors with Different Pseudotypes

Research paper thumbnail of The gastroschisis prognostic score: reliable outcome prediction in gastroschisis

Journal of Pediatric Surgery, 2012

Background/Purpose: Disease-specific outcome predictors are required for gastroschisis. We derive... more Background/Purpose: Disease-specific outcome predictors are required for gastroschisis. We derived and validated a gastroschisis prognostic score (GPS) based on bowel appearance after birth. Methods: Visual scoring of bowel matting, necrosis, atresia, and perforation generated a novel gastroschisis bowel injury score recorded in a national database. Reweighting of score components by regression analysis led to assessments of model calibration and goodness of fit. The GPS was validated in subsequent cases. Results: Records from 225 infants were used for model derivation. Only intestinal necrosis independently predicted mortality by regression analysis (odds ratio, 11.5; 95% confidence interval, 4.2-31.4). Model recalibration identified that a GPS of 4 or more predicted mortality in 75% of nonsurvivors and 99% of survivors (P = .0001). A GPS of 2 or more demonstrated significantly worse survival outcomes compared with scores of 0 or 1 (length of stay: P = .011, days to first enteral feed: P = .013, days on total parenteral nutrition: P = .006). Model validation with 184 new patients yielded continued high-quality discrimination of outcomes. The GPS demonstrated "near-perfect" interobserver reliability between 2 surgeons (κ ≥ 0.86).

Research paper thumbnail of Canadian Pediatric Surgical Network: a population-based pediatric surgery network and database for analyzing surgical birth defects. The first 100 cases of gastroschisis

Journal of Pediatric Surgery, 2008

Research paper thumbnail of Pediatric surgeons' activities and future plans

Journal of Pediatric Surgery, 1997

Research paper thumbnail of Expression of neuropeptides in normal and abnormal appendices

Journal of Pediatric Surgery, 2001

Increased neuroproliferation in the appendix associated with an increase in substance P (SP), vas... more Increased neuroproliferation in the appendix associated with an increase in substance P (SP), vasoactive intestinal polypeptide (VIP), and growth-associated protein-43 (GAP-43) has been documented in appendices of adults with acute right lower quadrant (RLQ) abdominal pain and absence of gross or histologic signs of appendiceal inflammation. The authors tested whether these findings were present in children with RLQ pain and a normal appendix. Immunohistochemistry staining of paraffin-embedded appendices was performed with GAP-43, VIP, and SP. The positive control group included appendices with acute inflammation (group I, n = 5); the negative control group included appendices removed incidentally (group II, n = 5); and the experimental group included appendices from children suspected to have acute appendicitis without histologic signs of inflammation (group III, n = 9). Group I: VIP was strongly expressed in the nerve plexuses. The lamina propria and muscularis showed absent or minimal VIP expression. SP staining was strong in all plexuses and was moderate to strong in the muscularis. SP expression in the epithelium and lamina propria was difficult to quantify secondary to inflammation. Group II: VIP expression was essentially undetectable in the epithelium, lamina propria, and muscularis, and was moderate in the nerve plexuses. Mild SP staining was detected in the nerve plexuses of most specimens, and absent to mild staining was found in the epithelium and muscularis. However, one specimen strongly expressed SP in all layers. Group III: VIP expression was moderate to strong in the lamina propria and muscularis of nearly all specimens, and strong expression was found in all nerve plexuses. All but one specimen strongly expressed SP in plexuses. There was moderate to strong expression of SP in the epithelium, lamina propria, and muscularis in over 50% of specimens. The immunostaining for GAP-43 was very weak and nonspecific and did not help discriminate between the 3 study groups. Increased neuroproliferation in the lamina propria and muscularis was evident in patients with abdominal pain and normal appendices compared with appendices removed incidentally. The VIP and SP expression in these patients was similar or higher than that observed in patients with acute inflammation on histology.

Research paper thumbnail of Pulmonary lymphangiectasia revisited

Journal of Pediatric Surgery, 2000

Pulmonary lymphangiectasia (PL) is a rare, poorly documented disease characterized by abnormal pu... more Pulmonary lymphangiectasia (PL) is a rare, poorly documented disease characterized by abnormal pulmonary lymphatics. Although case reports are published, little is known about survivors past the neonatal period. Methods: This is a retrospective review of histologically proven PL in fetuses, infants, and long term survivors since 1965. Results: Eleven children (8 boys, 3 girls) and 8 aborted fetuses (7 male, 1 female) were identified. The fetuses weighed 463.4 g (177 to 681 g). Six were aborted between 19 to 24 weeks of gestation for multiple malformations or anencephaly, and 2 spontaneously aborted: one with PL only, the other with twin-twin transfusion syndrome. Clinical PL was diagnosed between 0 and 11 months of age. Six children died (2 neonatal, 4 within 10 days), 5 survived. Two deaths occurred after cardiac surgery. Among survivors, the symptomatology and frequency of admissions diminished over time. Symptoms included progressive respiratory distress, chronic cough, recurrent pneumonia, bronchial asthma, and choking. One child with bilateral chylothorax was later diagnosed with Noonan syndrome; 2 patients had minor cardiac malformations. Rapid deterioration occurred with mild respiratory infections with only supportive treatment available. Chest x-ray showed marked hyperinflation with interstitial infiltrate. Conclusions: This is the first long-term study of primary PL and will help counsel parents. Although fatal in the neonatal period, survival is possible if diagnosed past the neonatal period and improvement is expected.

Research paper thumbnail of Correction of hindbrain herniation and anatomy of the vermis after in utero repair of myelomeningocele in sheep

Journal of Pediatric Surgery, 2003

In utero repair of myelomeningocele (MMC) in humans spares distal neurologic function, reverses t... more In utero repair of myelomeningocele (MMC) in humans spares distal neurologic function, reverses the hindbrain herniation component of the Arnold-Chiari II malformation (ACM), and reduces the rate of postnatal shunt placement. The authors hypothesized that extravasation of cerebrospinal fluid (CSF) from the lumbar spinal cord results in herniation. This hypothesis was tested by assessing the impact of a spinal cord myelotomy on hindbrain anatomy in fetal sheep. A MMC lesion was created surgically in 34 fetal sheep at 75 days&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;#39; gestation by excision of the L1-L5 lamina, the exposed dura, and surrounding tissues. A lumbar level myelotomy was performed in 28 of the 34 fetuses to open the central canal of the spinal cord to enhance egress of CSF through the MMC defect and potentially induce hindbrain herniation. At 102 days&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;#39; gestation, a repair of the MMC lesion was performed in 14 fetuses with a myelotomy. Fetuses underwent autopsy at 102, 114, 120, or 140 days&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;#39; gestation. Control animals underwent 2 unrelated fetal surgical procedures at approximately 70 and 110 days&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;#39; gestation. The incidence of hindbrain herniation, ventricular size, biparietal diameter, brain weight, and brain anatomy were compared between the different animal groups. After MMC creation, significant cerebellar tonsillar herniation was observed in 85% of fetuses that underwent creation of a myelotomy; none of the lambs without a myelotomy (n = 6) had hindbrain herniation. At autopsy, cerebellar tonsillar herniation was present at the time of MMC repair (102 days&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;#39; gestation), 2 weeks after MMC repair, but was reversed 3 weeks post-MMC repair. At birth, tonsillar herniation was absent, and hindbrain anatomy was restored in 88% of the fetuses with a myelotomy that underwent fetal MMC repair. No significant differences in brain weight and ventricular size was observed between animals with and without MMC repair. Adding a myelotomy to the sheep model of MMC leads to hindbrain herniation that is similar to that observed in the human ACM. These experiments support the hypothesis that leakage of CSF through the exposed central canal alters the normal CSF hydrodynamics, resulting in cerebellar tonsillar herniation. Fetal MMC repair reverses hindbrain herniation and restores gross anatomy of the vermis.

Research paper thumbnail of A contemporary evaluation of pulmonary function in children undergoing lung resection in infancy

Journal of Pediatric Surgery, 2011

Background/Purpose: The management of asymptomatic congenital lung lesions is controversial. Some... more Background/Purpose: The management of asymptomatic congenital lung lesions is controversial. Some centers recommend resection in infancy, and others prefer observation. Our objective was to evaluate the pulmonary function of children who underwent lung resection at 12 months or younger. We hypothesized that these children would not have a significant reduction in pulmonary function when compared with norms for age. Methods: All patients at 2 tertiary-care children's hospitals who underwent lung resection at 12 months or younger and are currently older than 5 years were identified and prospectively recruited. Pulmonary function testing was standardized in all patients. Results: Fourteen children were tested prospectively, whereas results were available for another 5 children. Four children were excluded for inability to perform pulmonary function testing (n = 2) or for preexisting pulmonary hypoplasia/syndrome (n = 2). Pulmonary function testing values were considered normal if they were more than 80% of predicted. Forced vital capacity was normal in 14 (93%) of 15 children, and forced expiratory volume in 1 second was normal in 13 (86%) of 15 children. Diffusion capacity and respiratory muscle strength were normal in all children tested. Conclusions: Most children undergoing lung resection in infancy will have normal pulmonary function tests, supporting our philosophy of early, elective resection of congenital lung lesions.

Research paper thumbnail of The EXIT procedure: Experience and outcome in 31 cases

Journal of Pediatric Surgery, 2002

The EXIT (ex utero intrapartum treatment) procedure, although initially designed for reversal of ... more The EXIT (ex utero intrapartum treatment) procedure, although initially designed for reversal of tracheal occlusion in fetuses with congenital diaphragmatic hernias (CDH), has been adapted to treat a variety of fetal conditions. A retrospective chart review of all consecutive EXIT procedures since 1996 was conducted. Thirty-one women underwent the EXIT procedure, with an average maternal age of 29 years (range, 20 to 38), and average gestational age of 34 weeks (range, 29 to 40). The indication was airway obstruction from fetal neck mass in 13, and reversal of tracheal occlusion from in utero clipping in 13. Singular indications included an EXIT-to-ECMO (extracorporeal membrane oxygenation) procedure for a fetus with CDH and a cardiac defect (n = 1), congenital high airway obstruction syndrome (CHAOS, n = 1), resection of a very large congenital cystic adenomatoid malformation of the lung (CCAM) on uteroplacental bypass (n = 1), unilateral pulmonary agenesis (n = 1), and thoracoomphalopagus conjoined twins. The mean duration on uteroplacental bypass (from uterine incision to umbilical cord clamping) was 30.3 plus minus 14.7 minutes (range, 8 to 66). No fetus experienced hemodynamic instability during uteroplacental bypass as recorded by fetal heart rate (FHR), pulse oximeter, and fetal echocardiography, except for one instance of reversible bradycardia from umbilical cord compression. The mean FHR and fetal saturation were 153.0 plus minus 38.5 beats per minute and 71.2% plus minus 19.9%, respectively. Five fetuses required a tracheostomy. Only 1 death occurred during an EXIT procedure because of inability to secure the airway secondary to extensive involvement by a lymphangioma. The average cord pH and pCO(2) were, respectively, 7.20 plus minus 0.11 and 63.2 plus minus 14.6. Two maternal complications occurred: bleeding from a hysterotomy site and dehiscence of an old hysterotomy scar noticed at a subsequent cesarean section. The average maternal blood loss was 848.3 plus minus 574.1 mL. The EXIT procedure was used successfully to ensure uteroplacental gas exchange and fetal hemodynamic stability during a variety of surgical procedures performed to secure the fetal airway or ensure successful transition to postnatal environment.

Research paper thumbnail of Megarectum after surgery for anorectal malformations

Journal of Pediatric Surgery, 2010

Megarectum complicating surgery for anorectal malformation (ARM) has implications for long-term c... more Megarectum complicating surgery for anorectal malformation (ARM) has implications for long-term continence. Factors influencing continence and defecation include intact rectal reservoir, innervation/proprioception of the anorectal muscle complex, functioning anorectal inhibitory reflex (AIR), and intact perception at the anal margin. We studied outcomes after surgery for ARM with emphasis on megarectum; particularly as to whether altered rectal proprioception from anatomic sacrococcygeal anomalies affect incidence. We also assessed whether an abnormal AIR could trigger passive rectal dilatation without mechanical obstruction. Methods: Eighty six infants (53 male) with ARM over 20 years were included. Demographics, surgical history, pathology, defecation patterns, imaging, manometry, and morbidity were analyzed. Incidence of sacrococcygeal malformations in children with and without megarectum was compared using Fisher exact test. Manometry results were evaluated for integrity of AIR and correlated to megarectum occurrence. Results: There were 23 high/intermediate and 63 low ARMs. Fourteen (16%) developed a megarectum: 6 of 23 in high and 8 of 63 in low anomalies (P = .33). Twelve patients underwent megarectum resection at a median of 2.6 years (7 months to 10 years); 2 received bowel management protocols. Fifty-seven percent (8/14) of children with and 7% (5/72) without megarectum had sacrovertebral anomalies (P = .0001). Patients with preoperative manometry (n = 5) demonstrated an intact AIR. Colonic manometry demonstrated hyperactive colons (n = 2). Constipation was the predominant preoperative symptom; 3 patients suffered from incontinence after resection. All the specimens showed normal innervation and thickened muscularis on pathology. Conclusions: Sacral anomalies, which are more prevalent in children who developed megarectum, may result in abnormal rectal proprioception contributing to this pathology. Innervation anomalies may coexist, although preoperative manometries showed normal AIRs. Rectal dysmotility may lead to stool retention with subsequent dilatation, and patients who underwent colonic manometry had diffuse colonic hypermotility. Further physiologic and cellular studies are needed to elucidate the causes of this significant complication after surgical ARM repair in the absence of obstruction.

Research paper thumbnail of Gastroschisis closure—does method really matter?

Journal of Pediatric Surgery, 2008

Research paper thumbnail of Long-term transgene expression in cardiac and skeletal muscle following fetal administration of adenoviral or adeno-associated viral vectors in mice

The Journal of Gene Medicine, 2003

In utero gene transfer may provide advantages for the correction of congenital genetic disorders.... more In utero gene transfer may provide advantages for the correction of congenital genetic disorders. In the present study we compare the ability of adenovirus (AdCMVLacZ), and two serotypes of adeno-associated virus (AAVCMVLacZ serotypes 2 and 2/5), to target cardiac and skeletal muscle after prenatal systemic or intramuscular injection in mice and assess the immune response to the vectors. Day 14 gestation fetal mice underwent direct intraperitoneal or intramuscular injection of AdCMVLacZ, and AAVCMVLacZ serotypes 2 and 2/5 vectors. Tissues were processed for beta-galactosidase expression in frozen or high-resolution thin plastic sections at early and late time points. Neutralizing antibodies to Ad and AAV were analyzed in separate fetal experimental and neonatal or adult control groups after administration and re-administration of the vectors. A single injection of each vector in utero resulted in sustained expression of beta-galactosidase transgene in skeletal and cardiac muscle. Transgene expression was detected for the length of the study, i.e. 86, 58, and 31 weeks after birth for AdCMVLacZ, and AAVCMVLacZ serotypes 2 and 2/5, respectively. High-level expression in the myocardium was observed independent of the vector or route of administration. Neutralizing antibody responses to AAV and Ad antigens were reduced and long-term expression in muscle was not ablated on postnatal re-administration of vector. Sustained, high-level cardiac and skeletal muscle transgene expression can be obtained after prenatal gene transfer with each of these vectors. The potential for immune response to viral antigens is altered, but not entirely ablated after in utero exposure.

Research paper thumbnail of Chondroid Cystic Malformation of the Lung With Trisomy 8 Mosaicism: A New Cystic Lung Malformation

American Journal of Surgical Pathology, 2008

Neonatal cystic disorders of the lungs are a heterogeneous malformative group including giant lob... more Neonatal cystic disorders of the lungs are a heterogeneous malformative group including giant lobar hyperinflation, congenital pulmonary airway malformations, intralobar pulmonary sequestration, and bronchogenic cyst. Here, we describe a giant cystic pulmonary malformation in a 5-year-old girl, morphologically characterized by a highly disorganized proliferation of numerous cartilage islands, abundant mesenchymal tissue with abundant adipose differentiation, and epithelium-lined cysts. Cytogenetic analysis revealed an isolated trisomy 8, as the sole karyotype anomaly, a finding further confirmed by a whole-genome single nucleotide polymorphism array genotyping. The trisomy 8 was observed by fluorescent in situ hybridization within the malformation, and also in adjacent pulmonary parenchyma. A search of the literature revealed only 2 cases having similarities with the present case, but bearing different names. We believe that this lesion differs from congenital pulmonary airway malformations and from adult-type pulmonary hamartomas. We propose for this malformative mass the name &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;quot;chondroid cystic malformation of the lung.&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;quot;