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Jacob C Langer

Publications and source records attributed to Jacob C Langer.

At least 19 recordsLinked to original sources
PubMed2005

Desmoplastic nested spindle cell tumor of liver: report of four cases of a proposed new entity.

We describe a distinctive tumor of the liver in four children composed of nested spindled and epithelioid cells with extensive desmoplasia that we have termed "desmoplastic nested spindle cell tumor of the liver." All four patients were previously healthy. One patient had a presumptive diagnosis of hepatic hemangioma 11 years prior to presentation. Grossly, the tumors were well circumscribed, lobular white masses, ranging from 2.8 to 15 cm in diameter. These tumors were characterized by the presence of cohesive nests of plump, bland spindle cells arranged in short fascicles with an accompanying desmoplastic stroma. Epithelioid areas ranging from palisading epithelioid cells at the periphery of some nests to pseudoglandular and polygonal cells with intercellular bridges were invariably present. Mitotic activity was low. Calcification and ossification were present. Non-neoplastic bile ducts and hepatic elements were seen both within and surrounding the tumor cell nests. Each tumor displayed cytoplasmic reactivity for vimentin, pan-cytokeratin, CD57, and nuclear staining for WT1. Neuroendocrine markers were negative. Ultrastructurally, the tumor cells showed focally well-developed cell junctions, basal lamina, and few cytoplasmic organelles. All tumors were confined to the liver and were resected without complication. Two patients received postoperative adjuvant therapy for presumed hepatoblastoma. The patients are doing well without recurrence at 7.5 years, 7 years, 5 years, and 8 months post-surgery. The morphologic appearance and immunohistochemical profile of these lesions are unique in our experience and represent a new category of pediatric liver tumor.

Adolescent↗
PubMed2004

Perinatally diagnosed asymptomatic congenital cystic adenomatoid malformation: to resect or not?

BACKGROUND: Management of asymptomatic congenital cystic adenomatoid malformation (CCAM) is controversial. The natural history of untreated asymptomatic CCAM is unknown, although most surgeons recommend resection of these lesions to prevent future infection. The aim of this study was to determine the relative surgical risk of resection compared with the risk of observation for these patients. METHODS: A retrospective review of hospital records between 1996 and 2002 in a tertiary care pediatric referral center was conducted. All perinatally (prenatal or neonatal) diagnosed CCAMs were included. In addition, patients presenting with late diagnosis of CCAM were also reviewed. RESULTS: Forty-eight children had CCAM diagnosed perinatally. Thirteen of these were symptomatic and required surgery within 6 months; these were excluded from the analysis. Of the 35 asymptomatic infants, 6 were operated on electively before 6 months of age (median age, 4.5 months). The other 29 asymptomatic infants were followed up for more than 6 months. Of these, 9 remained asymptomatic and were eventually operated on electively (median age, 13 months). Three (10%) had CCAM infections at 7, 8, and 11 months of age and required resection. The remaining 17 children have not undergone resection and are still asymptomatic (median follow-up, 3 years). An additional 12 patients presented with a late diagnosis of CCAM. All of these presented with complications (infection or pneumothorax) and underwent resection (median age, 6 years). Overall, the complication rate after resection of an asymptomatic CCAM was not significantly different from those of resected CCAM that had already developed infection or pneumothorax (P =.64). CONCLUSIONS: Ten percent of perinatally diagnosed asymptomatic patients had 5 complications requiring surgery during follow-up. The true incidence is probably higher given the relatively short follow-up in our series. Morbidity after resection of a complicated CCAM was not statistically significantly higher than after elective resection for an asymptomatic CCAM. Although conservative management of asymptomatic CCAM may be warranted, a more extended period of follow-up is necessary before this approach can be recommended.

Cystic Adenomatoid Malformation of Lung, Congenita↗
PubMed2004

Ovarian torsion in children: is oophorectomy necessary?

BACKGROUND: Most pediatric surgeons perform oophorectomy in girls presenting with ovarian torsion in which the ovary appears necrotic. However, the adult gynecology literature suggests that many ovaries can be treated by detorsion alone. METHODS: All children with ovarian torsion on the gynecology and general surgery services between 1988 and 2002 were reviewed. RESULTS: There were 36 torsions in 34 children. Seventeen underwent detorsion with or without ovarian cystectomy, and 19 had oophorectomy (mean age 10 years in both groups). Torsion was suspected preoperatively in 94% of the detorsion cases and in 47% of the oophorectomy patients. Median time from presentation to surgery was significantly lower in the detorsion than the oophorectomy group (median 14 v 27 hours; P =.04). Postoperative complications and length of stay were similar between the 2 groups. Despite the ovary being judged intraoperatively as moderately to severely ischemic in 53% of the detorsion cases, follow-up sonogram or ovarian biopsy available in 14 of the 17 cases showed normal ovary with follicular development in each case. CONCLUSIONS: Simple detorsion was not accompanied by an increase in morbidity, and all patients studied had functioning ovarian tissue on follow-up despite the surgeon's assessment of the degree of ovarian ischemia. Detorsion is the procedure of choice for most cases of ovarian torsion in children.

Adolescent↗
PubMed2004

Reliability of intraoperative frozen sections in the management of Hirschsprung's disease.

BACKGROUND: The use of a 1-stage pull-through for Hirschsprung's disease (HD) is dependent on accurate identification of the normally innervated bowel on intraoperative frozen sections (IOFS). The authors wished to determine the incidence and sources of error during this process. METHODS: All HD patients undergoing IOFS over a 15-year period were reviewed. RESULTS: Three hundred four patients underwent a total of 700 IOFS. In 9 cases (3%), there was discrepancy between IOFS and permanent sections. Two of these were false-positive (ganglion cells incorrectly believed to be present at IOFS); both required a second operation as a result of the error. Seven were false-negative (presence of ganglion cells not recognized at IOFS); none required a subsequent operation, but 2 had a significantly more extensive colonic resection than was necessary. Responsible factors included sampling from the transition zone, freezing artifact, and misinterpretation of ganglion cells in very young patients owing to pathologist inexperience. There was significant variability in the error rate among the 11 pathologists. However, the numbers were too small for statistical analysis to determine whether there was a correlation between the rate of errors and the volume of cases done or years of experience. CONCLUSIONS: Error in reading of IOFS is rare but can have significant repercussions in patient care. Multiple factors, including technical issues and pathologist experience, may have a role in contributing to these errors.

Adolescent↗
PubMed2004

Persistent obstructive symptoms after surgery for Hirschsprung's disease: development of a diagnostic and therapeutic algorithm.

BACKGROUND: Although most children with Hirschsprung's disease (HD) do well after pull-through surgery, some continue to have persistent obstructive symptoms that may lead to significant morbidity. The author's goal was to develop an organized algorithm for the workup and ultimate management of these symptoms. METHODS: All children referred to the author with severe obstructive symptoms after a pull-through for HD were evaluated using an algorithm based on 5 potential etiologies, and appropriate therapeutic interventions were instituted. RESULTS: A total of 49 children were evaluated over 7 years. Mechanical obstruction was found using rectal examination and barium enema in 7, of which, 2 had resection of a Duhamel spur, and 5 had a repeat pull-through (after failed dilatation). Rectal biopsy results showed aganglionosis in 10, of whom, 8 underwent repeat pull-through, and 2 refused further surgery. Abnormal intestinal motility or intestinal neuronal dysplasia was found proximal to the aganglionic segment in 10 children using colonic and small bowel manometry or laparoscopic biopsies; 4 of these had additional colon resected and repeat pull-through, and 6 were treated with a bowel management routine, cecostomy, or stoma formation. Internal sphincter achalasia was felt to be the primary cause of symptoms in 14, and all were treated with intrasphincteric botulinum toxin. Eight were found not to fall into any of the above groups and were treated medically for presumed functional megacolon; 2 of these ultimately had a colostomy. CONCLUSIONS: An organized approach to the diagnosis and management of obstructive symptoms in a child after a pull-through for HD permits accurate diagnosis and effective treatment in the majority of cases.

Algorithms↗
PubMed2004

Image-guided percutaneous gastrostomy in neonates with esophageal atresia.

BACKGROUND: Neonates with esophageal atresia may require a gastrostomy before definitive repair. Most surgeons do this procedure using the Stamm technique through a laparotomy. The authors describe a new technique for percutaneous placement of a gastrostomy in these infants and report their preliminary results. METHODS: For children with esophageal atresia and a tracheoesophageal fistula (TEF), the air-filled stomach is localized fluoroscopically and accessed using a standard percutaneous technique. For those with pure esophageal atresia, a transhepatic needle is used to instil air into the stomach. Once the stomach is distended, the gastrostomy tube is inserted under fluoroscopy. RESULTS: Fourteen neonates with esophageal atresia had a percutaneous gastrostomy tube placed. Eleven had esophageal atresia and a TEF, and 3 had pure esophageal atresia with a gasless abdomen requiring the transhepatic approach. There were no intraoperative or major postoperative complications, but there were 5 minor early postoperative complications and 2 minor late postoperative complications. CONCLUSIONS: Percutaneous gastrostomy insertion is a safe technique for neonates with esophageal atresia and can be used even in children with pure esophageal atresia who have a gasless abdomen. This technique does not require laparotomy and appears to be associated with a low rate of complications.

Esophageal Atresia↗
PubMed2004

Errors and adverse outcomes on a surgical service: what is the role of residents?

PURPOSE: The extensive changeover in residents that occurs every July in university-affiliated hospitals has been postulated to result in impaired delivery of patient care as new house staff are less experienced and unfamiliar with hospital-specific systems (the "July phenomenon"). To assess the impact of this process on patient safety, we examined the incidence and sources of medical error and adverse outcomes on a pediatric general surgery service during the final month of an academic year and the first month of the subsequent academic year. MATERIALS AND METHODS: All admissions to two pediatric surgeons during June and July 2002 were prospectively followed. The attending surgeon, a surgical fellow, and a medical student reviewed in-patient care daily. Errors committed by doctors, nurses, and allied health workers were identified through daily patient encounters, nursing rounds, medical rounds, and chart audit. Adverse outcomes were evaluated based on type and contributing factors, including involvement of residents. To correct for variations in patient volume, the incidence of errors and adverse outcomes were expressed as a percentage of total patient days. RESULTS: The error rate was 46/643 patient days (7.1%) in June, and 58/776 patient days (7.5%) in July (P = 0.9). Resident error accounted for 52.2% of errors in June and 39.7% of errors in July (P = 0.28). There was no significant difference in the adverse outcome rates (5% versus 6.7%, P = 0.21) or incidence of error-related adverse outcomes (10.8% versus 22.4%, P = 0.2) between June and July. Most errors were made by the on-call resident. CONCLUSION: Resident changeover at the completion of an academic year did not result in an increased number of medical errors or adverse outcomes, indicating that effective systems are in place to prevent the "July phenomenon."

Adolescent↗
PubMed2004

Mechanisms of pediatric trauma deaths in Canada and the United States: the role of firearms.

BACKGROUND: This study aimed to determine whether firearms are a more prevalent cause of pediatric death in the United States than in Canada. METHODS: All pediatric trauma deaths from 1991 to 1996 in Ontario and Missouri were reviewed. Socioeconomic data were compiled for the two jurisdictions. RESULTS: During the period reviewed, there were 1,146 pediatric trauma deaths in Ontario (10.4 per 100,000 population) and 1,782 in Missouri (32.4 per 100,000 population). Firearm injuries accounted for 19% of the trauma deaths in Missouri and 0.5% of such deaths in Ontario. Overall, a child was 100 times more likely to die of firearm injury in Missouri (6 per 100,000 population) than in Ontario (0.06 per 100,000 population). The incidences of violent acts unrelated to firearms were similar between the two groups. Both populations were similar in terms of socioeconomic and education parameters, but differed in their rates for guns carried. CONCLUSION: The significantly higher death rate from firearm injuries in Missouri likely reflects differing gun control attitudes and legislation, and provides a rationale for prevention and future investigation.

Child↗
PubMed2004

Does pediatric surgical specialty training affect outcome after Ramstedt pyloromyotomy? A population-based study.

OBJECTIVE: Ramstedt pyloromyotomy is a common operation in infants and is often done by general surgeons. We wished to determine whether there are any differences in outcome when this procedure is done by subspecialist pediatric general surgeons as compared with general surgeons. METHODS: All Ramstedt pyloromyotomies in the province of Ontario between 1993 and 2000 were reviewed. Children with complex medical conditions or prematurity were excluded. Cases done by general surgeons were compared with those done by pediatric surgeons, specifically examining hospital stay and complications. RESULTS: Of 1777 eligible infants, 67.9% were operated on by pediatric surgeons and 32.1% by general surgeons. Total and postoperative lengths of stay were longer in the general surgeon group compared with the pediatric surgeons (4.31 vs 3.50 days for length of stay; 2.95 vs 2.25 days for postoperative length of stay). The general surgeons had a higher overall complication rate (4.18% vs 2.58%). The incidence of duodenal perforation among general surgeons was almost 4 times that of pediatric surgeons (relative risk: 3.65; 95% confidence interval: 1.43-9.32). Of the 4 infants who required repeat surgery because of an incomplete pyloromyotomy, all were originally operated on by a general surgeon. Analysis of the effect of surgeon volume on outcomes suggested that higher volume resulted in better outcome in both groups. CONCLUSION: Subspecialist pediatric general surgeons achieve superior outcomes for children who undergo Ramstedt pyloromyotomy.

Clinical Competence↗
PubMed2003

Pediatric pulmonary carcinoid: a case report and review of the literature.

We report on the youngest known patient with a pulmonary carcinoid, a rare tumor in children. We review the management of and prognosis for this tumor. Diagnosis can be delayed because of low clinical suspicion and the many ways in which these tumors can present. Treatment is surgical and geared towards complete resection while sparing healthy lung parenchyma. Prognosis is generally good, but careful long-term follow-up is needed for potential recurrences or associated tumors.

Antineoplastic Agents, Hormonal↗
PubMed2003

Abdominal wall defects.

The frequent use of prenatal diagnostic techniques including ultrasound and maternal serum alpha-fetoprotein has increasingly led to detection of abdominal wall defects before birth. This prenatal detection creates the opportunity to influence neonatal outcome by alteration in management of pregnancy or delivery. The optimal management of an individual fetus depends on careful prenatal assessment of the abdominal wall defect, combined with experience and knowledge of the natural history for that particular lesion. A multidisciplinary approach to the fetus can improve neonatal outcome. Careful assessment for other structural anomalies and karyotype analysis should be performed. Delivery at a high-risk perinatal center should be encouraged. Currently, there is no convincing evidence to support routine cesarean section for most abdominal wall defects.

Abdominal Muscles↗
PubMed2003

Acute appendicitis with fulminant necrotizing fasciitis in a neonate.

The authors report on a neonate who presented with acute appendicitis and who went on to have fatal necrotizing fasciitis. Although recognized in adults, this rare combination of conditions has not been reported previously in the neonate and requires rapid recognition with aggressive medical and surgical intervention.

Acute Disease↗
PubMed2003

Can 'long-gap' esophageal atresia be safely managed at home while awaiting anastomosis?

BACKGROUND: Neonates with "long gap" esophageal atresia (EA) are often managed with gastrostomy and tube drainage of the proximal pouch for a number of months while awaiting definitive repair. Because of the risk of aspiration and need for complex nursing care, most remain hospitalized during this time. However, prolonged hospitalization utilizes scarce resources and may be difficult for many families. METHODS: The authors report on 5 patients who were treated successfully at home while awaiting esophageal anastomosis. RESULTS: Four patients had pure EA (one had a duodenal atresia), and one had a distal fistula. Gestational ages ranged from 31 to 41 weeks. All had a gastrostomy within days of birth. Age at definitive repair ranged from 6 to 12 months. Time at home while awaiting anastomosis ranged from 42 to 113 days. Care at home included nursing care, suction equipment and training, gastrostomy feeding, and ability to perform cardiopulmonary resuscitation. The only complications noted while at home consisted of ear infection in one patient and recurrent upper respiratory tract infections in another patient. CONCLUSIONS: Selected patients with long gap esophageal atresia can be treated safely at home while awaiting esophageal anastomosis. Success of this approach depends on a motivated, reliable family, and adequate support from community health care providers.

Anastomosis, Surgical↗
PubMed2003

Cut it out: Managing hepatic abscesses in patients with chronic granulomatous disease.

BACKGROUND: Hepatic abscesses develop in patients with chronic granulomatous disease (CGD) because the liver is a site of constant bacterial challenge. The authors investigated the roles of drainage and hepatic resection in the management of liver abscesses in CGD patients. METHODS: Medical records of CGD patients with hepatic abscesses from 1990 to 2001 were reviewed. RESULTS: There were 6 patients. Mean age of initial abscess was 7.2 years (range, 3 weeks to 18.9 years). All abscesses involved the right lobe of the liver (2 single, 4 multiple). All patients received appropriate antibiotics. Four patients were treated with one to 6 drainage procedures over one to 4 admissions before ultimately undergoing resection. The other 2 patients underwent primary resection without preliminary drainage. Of the 6 resections, 4 were nonanatomic, and 2 were anatomic. There was one major postoperative complication (bleeding) requiring reoperation. There were no recurrences after resection (mean follow-up 4.3 yr). Mean total days in hospital for the treatment of liver abscess was 49 in the preliminary drainage group and 8.5 in the primary resection group. Three patients required admission into the intensive care unit, one after a drainage procedure and 2 after resection. CONCLUSIONS: For CGD patients with hepatic abscesses, drainage procedures are associated with recurrence and prolonged hospitalization. Primary hepatic resection removing all involved tissue is safe and definitive for the management of this problem.

Adolescent↗