Biomedical subjects
Publications and source records attributed to J M Laberge.
A 2-year-old boy presented with bowel obstruction 5 days after repair of a recurrent epigastric hernia. Pre-operative imaging implicated intussusception as a cause of the obstruction. At operation a gangrenous transverse colocolic intussusception was resected en masse. No clear lead point was seen. Recovery was uneventful.
BACKGROUND/PURPOSE: The aim of this study was to review the presentations of gastrointestinal duplication (GID) and to assess the influence of prenatal diagnosis on treatment. METHODS: Retrospective review of all GID at 2 pediatric hospitals from 1980-2002 was conducted. RESULTS: Seventy-three patients (M43:F30) were identified: 21 neonates, 28 infants (1 to 24 months), 15 children (1 to 10 years), 9 adolescents (>/=11 years). GID location by frequency was ileum (31.5%), ileocaecal valve (30.2%), duodenum (9.6%), stomach (8.2%), jejunum (8.2%), colon (6.8%), and rectum (5.5%). In neonates and infants, vomiting and distension were the most common presentations. Volvulus, caused by a duplication, occurred in 23.8% of neonates and caused the death of one neonate. Intussusception was identified in 10.9% of patients. In older children and adolescents, pain and vomiting were the most common associations. Six of these patients were being treated for Crohn's disease, with the diagnosis of duplication made at laparotomy. Eighteen patients had a prenatal diagnosis by ultrasound scan, with 77.2% of these asymptomatic after birth. Most prenatal diagnoses occurred after 1991 (77.8%). When comparing an earlier period (1980 to 1991; 29 patients) with the current (1992 to 2002; 44 patients), a greater proportion of the latter patients were asymptomatic (36.4 v 13.8%) and had a lower incidence of complications (volvulus/intussusception). CONCLUSIONS: GID can lead to life-threatening complications. Prenatal diagnosis should lead to expeditious postnatal investigation and treatment before the onset of symptoms or complications. GID in older children can mimic Crohn's disease. Laparoscopy/laparotomy should be considered in patients with atypical Crohn's disease or when the diagnosis of an intraabdominal mass is unclear.
BACKGROUND/PURPOSE: In normal lungs, fetal tracheal occlusion (TO) induces lung growth but decreases the number of type II cells; this is remedied if TO is released (TR) before delivery. In the current study, the effects of TO with or without TR on pulmonary structure and surfactant were assessed in the ovine model in which lung hypoplasia was induced by creation of a diaphragmatic hernia (CDH). METHODS: A left-sided CDH was created in fetal lambs at 80 days gestation; TO was done at 108 days; and TR at 129 days. All ewes were given 1 dose of glucocorticoids at 135 days. At 136 days, the fetus was delivered. Lung weight to body weight ratio, mean terminal bronchiole density, type II cell density, bronchoalveolar lavage fluid (BAL) phosphatidylcholine (PC), BAL surfactant protein A (SP-A) and B (SP-B), and lung tissue SP-A and SP-B were assessed in CDH, CDH with TO, CDH with TO and TR, and controls. RESULTS: CDH lungs were hypoplastic and structurally immature, but had increased type II cell density. TO with or without TR caused lung growth with normalization of lung parenchymal architecture and type II cell density. Although the BAL SP-A and BAL SP-B were similar in all 4 groups, the BAL PC was low in CDH with or without TO or TR. Also, lung tissue SP-B levels were low in CDH with or without TO or TR. However, lung tissue SP-A levels were normal in CDH, but low in CDH with TO with or without TR. CONCLUSIONS: Despite the finding that lung morphology was improved in CDH with TO with or without TR animals, surfactant content and composition remained abnormal. Although surfactant secreted early by the fetus into alveolar spaces contained normal levels of BAL SP-A and BAL SP-B, the low levels of BAL PC and low lung tissue stores of SP-B indicate that these experimental lambs may experience respiratory insufficiency soon after birth. This implies that prophylactic surfactant at birth might be beneficial for CDH.
PURPOSE: The aim of this study was to review the indications, success rate, and complications of endoscopic retrograde cholangiopancreatography (ERCP) in the pediatric age group. METHODS: From 1990 to 1999, 21 ERCP procedures were attempted in 20 patients. They consisted of 8 boys and 12 girls whose age ranged from 4 to 17 years (mean, 11.3 years). Fourteen were performed under deep sedation (mean age, 12.8 years), and 7 were done under general anesthesia (mean age, 7.6 years). All ERCP procedures were performed by experienced adult endoscopists. RESULTS: The indication for ERCP was biliary in 15 patients. Eleven had suspected choledocholithiasis by either ultrasound scan, intraoperative cholangiogram or magnetic resonance imaging (MRI). In 6 cases, the ERCP was done for pancreatic pathology. In 11 patients, the ERCP was diagnostic only, and in 10 a therapeutic procedure was done. The overall success rate was 90.5%. Post-ERCP complications consisted of 6 episodes of pancreatitis (28.5%), 4 of which followed a therapeutic procedure, and 1 episode of bleeding. Pancreatitis resolved 2 to 6 days post-ERCP. The patients underwent follow-up between 2 and 56 months after the ERCP (mean, 11 months). CONCLUSIONS: The authors conclude that even in experienced hands, ERCP in the pediatric population has a much higher complication rate than in adults (33.3%). We recommend that very specific indications be met before subjecting a pediatric patient to an endoscopic retrograde cholangiopancreatography.
PURPOSE: Congenital diaphragmatic hernia (CDH) is associated with thickened pulmonary arteries (PA) contributing to pulmonary hypertension. In the current study, the effects of antenatal glucocorticoids and reversible tracheal occlusion (TO) on PA structure were assessed in a hypoplastic lung model. METHODS: A left-sided CDH was created in fetal lambs at 80 days gestation, TO at 108 days, and release of the occlusion (TR) at 129 days. All were given 1 dose of maternal glucocorticoids at 135 days. At 136 days (term, 145 days), the fetus was delivered by cesarian section. CDH (n = 7), CDH + TO (n = 6), CDH + TO + TR (n = 6), and unoperated twin controls (n = 16) were compared. Outcome measurements were (1) lung growth, represented by lung weight to body weight ratio (LW/BW), (2) lung structural maturation, which is inversely proportional to mean terminal bronchiole density (MTBD), (3) PA medial and adventitial areas (square micrometers), (4) lung capillary load, which is the ratio of vessel surface area (SA) to tissue SA ratio. RESULTS: CDH lungs were hypoplastic with a low LW/BW and high MTBD. The small PAs (<75 microm) of CDH had an increased medial area, indicating increased muscle mass and an increased adventitial area. CDH + TO +/- TR increased LW/BW and achieved normal structural lung maturity with a low MTBD. Only CDH + TO thinned the PA medial area closer to control values. The adventitial area remained thick in CDH +/- TO +/- TR when compared with controls. All 4 groups had similar capillary load. CONCLUSIONS: TO may be especially important for PA remodeling in the latter part of gestation, because TR 1 week before delivery prevents thinning of the small PAs in CDH. The shaping achieved by TO in terms of lung growth, structural maturity, and pulmonary artery medial area thinning may prove beneficial in lessening the severity of the associated pulmonary hypertension in CDH.
PURPOSE: Abdominal sonography has gained popularity in establishing the diagnosis of appendicitis in children with equivocal clinical presentations. However, no clear outcome benefits have been demonstrated to date. The authors conducted a retrospective study to compare the characteristics and outcomes of patients undergoing appendectomy after clinical evaluation only with those undergoing the procedure after sonography. METHODS: The charts of 454 consecutive patients undergoing appendectomy for acute appendicitis between January 1, 1998 and December 4, 1999 were reviewed. Patients operated on after clinical evaluation only were compared with patients operated on after abdominal sonography. RESULTS: Forty-two percent of patients (n = 191) constituted the sonography group. When compared with the clinical group, these patients had higher prevalence of female gender (52% v 38%; P =.004), longer symptom duration (2.2 +/- 2.5 v 1.6 +/- 1.6 days; P =.003), higher incidence of preoperative in-patient observation (19% v 4%; P <.001), longer duration between evaluation and operation (8.0 +/- 3.9 v 4.9 +/- 2.9 hours; P <.001), higher incidence of normal appendices on pathologic examination (13% v 6%; P =.006), and higher incidence of postoperative abscesses or phlegmons (4.4% v 1.2%; P =.04). The groups did not differ significantly in age, hospital stay, incidence of complicated appendicitis, or incidence of wound infection. CONCLUSIONS: Patients undergoing sonography before appendectomy have a longer delay before operation, a higher rate of misdiagnosis, and more postoperative complications. Limiting sonography to truly equivocal cases and using it early in the diagnostic workup may improve outcomes in this group of patients.
PURPOSE: The goal of this study was to identify the proportion of sequestrations that were atypical or associated with other entities, such as congenital cystic adenomatoid malformations, communicating bronchopulmonary foregut malformations, bronchogenic cyst, and scimitar syndrome. METHODS: All charts of patients with pulmonary sequestration admitted at 2 children's hospitals from 1982 to July 1999 were reviewed retrospectively. The authors included all anomalies with a systemic arterial supply or without bronchial connection. RESULTS: Only 22 of the 39 patients (56%) had a classic isolated extralobar or intralobar sequestration, whereas the others presented with a spectrum of anomalies. Of the 13 cases diagnosed prenatally, 85% were asymptomatic at birth. In contrast, 26 cases diagnosed postnatally were all symptomatic, with those patients less than 2 weeks old presenting with various degrees of respiratory distress, and those older than 2 weeks old presenting with respiratory infections. The correct diagnosis was made preoperatively in 59% of cases. Only 4 patients did not undergo resection of their lesion, of which, 1 underwent interventional radiology with embolization of the anomalous arterial supply. Follow-up issues of importance included pneumonia, asthma, gastroesophageal reflux, and pectus excavatum. CONCLUSIONS: Sequestrations represent a spectrum of anomalies that overlap with other lung lesions. To facilitate management, they should be described according to their (1) connection to the tracheobronchial tree, (2) visceral pleura, (3) arterial supply, (4) venous drainage, (5) foregut communication, (6) histology, (7) mixed/multiple lesions, and (8) whether there are associated anomalies. Surgeons should be aware that approximately 50% of sequestrations could be atypical or associated with other anomalies. This should be kept in mind when weighing the benefits of resection versus conservative management of pulmonary sequestrations.
Retroperitoneal epidermoid cysts are rare. The authors report a case of an 11-year-old boy with an asymptomatic subdiaphragmatic cyst, which was found incidentally during an investigation for hypertension. At laparoscopy, the cyst was densely adherent to the diaphragm, resulting in a pneumothorax during dissection. Nevertheless, the excision and the diaphragmatic repair could be completed laparoscopically without complication. Microscopic examination showed an epidermoid cyst. No similar case has been reported in the literature.
PURPOSE: We sought to assess the role of endovascular techniques in the management of perigraft flow (endoleak) after endovascular repair of an abdominal aortic aneurysm. METHOD: We performed endovascular repair of abdominal aortic aneurysm in 114 patients, using a variety of Gianturco Z-stent-based prostheses. Results were evaluated with contrast-enhanced computed tomography (CT) at 3 days, 3 months, 6 months, 12 months, and every year after the operation. An endoleak that occurred 3 days after operation led to repeat CT scanning at 2 weeks, followed by angiography and attempted endovascular treatment. RESULTS: Endoleak was seen on the first postoperative CT scan in 21 (18%) patients and was still present at 2 weeks in 14 (12%). On the basis of angiographic localization of the inflow, the endoleak was pure type I in 3 cases, pure type II in 9, and mixed-pattern in 2. Of the 5 type I endoleaks, 3 were proximal and 2 were distal. All five resolved after endovascular implantation of additional stent-grafts, stents, and embolization coils. Although inferior mesenteric artery embolization was successful in 6 of 7 cases and lumbar embolization was successful in 4 of 7, only 1 of 11 primary type II endoleaks was shown to be resolved on CT scanning. There were no type III or type IV endoleaks (through the stent-graft). Endoleak was associated with aneurysm dilation two cases. In both cases, the aneurysm diameter stabilized after coil embolization of the inferior mesenteric artery. There were two secondary (delayed) endoleaks; one type I and one type II. The secondary type I endoleak and the associated aneurysm rupture were treated by use of an additional stent-graft. The secondary type II endoleak was not treated. CONCLUSIONS: Type I endoleaks represent a persistent risk of aneurysm rupture and should be treated promptly by endovascular means. Type II leaks are less dangerous and more difficult to treat, but coil embolization of feeding arteries may be warranted when leakage is associated with aneurysm enlargement.
Tracheal occlusion (TO) in fetal lambs induces pulmonary hyperplasia but has negative effects on type II cells. The purpose of this study was to determine whether antenatal steroids could reverse the adverse effects of TO on lung maturation in fetal lambs. Sixteen time-dated pregnant ewes (term, 145 d) and 24 of their fetuses were divided into six groups: 1) TO at 117 d gestation; 2) TO at 117 d with a single maternal intramuscular injection of 0.5 mg/kg betamethasone 24 h before delivery; 3) TO at 117 d and release of the occlusion 2 d before delivery; 4) TO and release of the occlusion with maternal steroids; 5) unoperated controls without antenatal steroid treatment; and 6) unoperated controls, littermates of groups 1-4, treated with antenatal steroids. All fetuses were killed at 137 d gestation. Outcome measurements consisted of lung weight-to-body weight ratio; lung morphometry determined by mean terminal bronchial density; and assessment of type II pneumocytes by in situ hybridization to the mRNA of surfactant proteins B and C. Lung weight-to-body weight ratio and mean terminal bronchial density were significantly different among groups with TO and controls, indicating increased lung growth and structural maturation. The density of type II pneumocytes was markedly decreased by TO. Release 2 d before sacrifice significantly increased the density and surfactant activity of type II pneumocytes, but to levels still far from controls. Steroids alone had an effect similar to release. An additive effect was noted with steroids and 2-d release resulting in type II cell density comparable to controls. After fetal TO, a single maternal intramuscular dose of 0.5 mg/kg of betamethasone 24 h before delivery allows partial recuperation of the type II pneumocytes, an effect that is potentiated by 2-d release.
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Tubular duplication of the recto-sigmoid colon is a rare entity. Associated anomalies including fistulae to the genitourinary tract may be found. A baby girl was found to have duplication of the recto-sigmoid colon, anomalies of sacral vertebra from S1 to S5, and solitary right kidney. The septum of this duplication was divided using staplers. Because of a history of stool coming from the vagina, a meticulous examination perioperatively was performed, but no fistula could be found. Further extensive investigation failed to show any fistula. At the age of 10 she was operated on for a tethered cord. At age 14, she experienced passage of a small amount of liquid stool per vaginum. A recto-vaginal fistula was found. Via a posterior sagittal incision, the fistula was closed by a transrectal approach. She remained asymptomatic for 16 months until the fistula recurred. Using a perineal approach, a very short fistula between the vagina and the rectum was closed. The closure was reinforced by a vaginal flap. Four months later, she remains without signs of recurrence.
PURPOSE: The aim of this study was to better define the mode of presentation, rate of volvulus, and surgical findings in children younger than 2 versus older than 2 years of age with malrotation. METHODS: The authors reviewed the charts of all patients with malrotation admitted to their hospital between January 1980 and December 1998, excluding patients having malrotation as a secondary finding. RESULTS: An upper gastrointestinal series was done in 90 patients (6% falsely negative) and a barium enema in 20 patients (40% read as normal). Fifty-eight patients had 114 associated congenital anomalies. Volvulus was found at the time of surgery in 28 patients, 5 of whom were older than 2 years. Three presented with acute symptoms and 2 with chronic symptoms. Surgery was performed by laparotomy in 103 patients and by laparoscopy in 3. Mean length of stay was 13.6 days. Mean follow-up was 19 months. Death occurred in 4 patients; postoperative bowel obstruction was seen in 3 patients (only 1 required surgery). CONCLUSIONS: Children with malrotation who are older than 2 years old have a significant risk of volvulus that is difficult to predict radiologically. They require surgical attention even if asymptomatic. Laparoscopy allows evaluation of the base of the mesentery and completion of the Ladd's procedure.
The authors report on an infant who had a multiloculated cystic lesion located in segment IV of the liver, consistent with Caroli's disease diagnosed, by routine prenatal ultrasound at 25 weeks' gestation, and confirmed by hepatobiliary HIDA scan and computed tomography soon after birth. Because there was no sign of biliary obstruction, the patient was observed initially, with gradual regression of the cysts noted by serial sonograms. Caroli's disease in older children and adults often is associated with recurrent cholangitis and cirrhosis, mandating resection when the disease is unilobar. However, the natural history of Caroli's disease diagnosed in utero is unclear, and a period of observation appears warranted in the asymptomatic patient.
Prenatal ultrasound (US) permits in utero diagnosis of sacrococcygeal teratoma (SCT), follow-up of tumor size, and the early identification of complications, allowing for a more timely and appropriate delivery. The recommended management of large SCTs is delivery by cesarean section (CS) to prevent dystocia, tumor rupture, hemorrhage, and death. However, even delivery by CS can be difficult, necessitating a large hysterotomy that adds to maternal morbidity. The authors report two cases of cystic SCTs in which prenatal percutaneous drainage allowed for an uncomplicated vaginal delivery. In the first case, a large unilocular cystic SCT was diagnosed at 31 weeks' gestation on prenatal US. The fetal presentation was breech, and the mass was steadily increasing in size, preventing spontaneous version. At 37 5/7 weeks, the cyst was percutaneously drained under US guidance allowing for successful external version. Repeat drainage just before induction of labor permitted a successful vaginal delivery. In the second case, the cystic SCT was percutaneously drained just before induction of labor at full term, again allowing for an uncomplicated vaginal delivery. Prenatal percutaneous needle drainage of cystic SCTs offers an alternative to CS that results in decreased risks for both mother and fetus.
BACKGROUND/PURPOSE: The management of lymphangioma in children is challenging because complete resection is difficult to achieve in some cases, and recurrences are common. The authors reviewed their experience to assess the risk factors for recurrence and the role of nonoperative treatment. METHODS: A retrospective study over a period of 25 years was carried out. One hundred eighty-six patients with 191 lesions (five patients with de novo lesions in different sites) were treated. There were 98 boys and 88 girls. The average age at diagnosis was 3.3 years (range, fetal life to 17 years) and the average size 8 cm in diameter. Histocytological confirmation was obtained in all patients. The involved sites were head and neck, 89 patients (48%); trunk and extremities, 78 patients (42%); internal or visceral locations (eg, abdominal and thorax), 19 patients (10%). The treatment consisted of macroscopically complete excision in 145 patients (150 lesions, of which five were recurrences in different sites), partial excision in 10 patients, aspiration in five patients, laser excision in 10 patients, biopsy only in four patients, drainage and biopsy in two patients, and injection of sclerosing agents in 10 patients. RESULTS: There were 54 recurrences; 44 underwent excision (five of them more than once), and five regressed spontaneously on follow-up. Five other recurrences were stable and not progressing. Recurrences, (defined as clinically obvious disease), were found to be 100% after aspiration, 100% after injection, 40% after incomplete excision, 40% after laser excision, and 17% after macroscopically complete excision. The recurrence rate in the last group was the highest in the head (33%), the least in the internal locations (0%), and intermediate for the cervical location (13%). There were no significant differences, in terms of outcome, between those who had their surgery immediately at the time of diagnosis (n = 101) and those who had delayed surgery (n = 85). CONCLUSIONS: There were fewer recurrences after macroscopically complete excision. Aspiration and injection had the highest recurrence rate. Risk factors for recurrence included location, size, and complexity of lesions. A period of observation may be useful for infants to facilitate complete excision. In the present series, spontaneous regression was infrequent and was seen more often with recurrent lesions.
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