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Jacob C Langer

Publications and source records attributed to Jacob C Langer.

12 recordsLinked to original sources
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

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↗
PubMed2003

Image-guided percutaneous approach is superior to the thoracoscopic approach in the diagnosis of pulmonary nodules in children.

BACKGROUND/PURPOSE: Image-guided, percutaneous techniques are increasingly used in diagnosis of pulmonary disease in children. The aim of this study was to determine the diagnostic accuracy and clinical outcomes of thoracoscopic versus percutaneous lung biopsy in children. METHODS: Sixty-three consecutive patients from January 1996 to December 2000 who had a thoracoscopic lung biopsy, a percutaneous ultrasound scan, or computed tomography (CT)-guided lung biopsy for well-defined and ill-defined lesions were analyzed. RESULTS: Twenty-eight patients had a thoracoscopic lung biopsy (TLB), and 35 patients had a percutaneous image-guided lung biopsy (PLB). Age ranged from 6 months to 17 years (median, 8 years). There was no significant difference between groups with regard to age, depth of lung nodule biopsied, or prebiopsy diagnoses. Seventeen patients (60%) of TLB and 23 (65%) of PLB had well-defined pulmonary nodules suspicious for malignancy at the time of biopsy. Adequate tissue for pathologic diagnosis was obtained in 28 (100%) of TLB versus 26 (80%) of PLB patients. However, 8 (28%) thoracoscopic cases needed to be converted to an open procedure. In 3 (8.5%) PLB cases the percutaneous biopsy was insufficient, and a thoracoscopic or open biopsy was required. The median hospital stay was 3 days for TLB and 4 to 6 hours for PLB (P =.023). There were no complications in the PLB group. Five (18%) of TLB patients suffered a persistent air leak treated with continued chest tube drainage, and one patient died of other causes with a persistent air leak. CONCLUSIONS: Percutaneous lung biopsy has a significantly shorter hospital stay and a lower complication rate than thoracoscopic lung biopsy. The authors propose that the percutaneous technique should be considered as the initial approach for children with pulmonary nodules.

Adolescent↗
PubMed2003

The failed fundoplication.

Fundoplication is the standard surgical approach to gastroesophageal reflux (GER) in a child. Although successful in many patients, there is a significant risk of complications and failure, especially in high-risk patients such as those with certain types of associated anomalies, diffuse motility disorders, chronic pulmonary disease, neurologic impairment, and young infants. Fundoplication failure can take the form of persistent reflux-related symptoms, symptoms that are caused by complications of the surgery, or anatomic problems such a para-esophageal hernia or migration of the wrap into the mediastinum. The most effective strategy for treatment of the child undergoing fundoplication is to prevent failure by careful patient selection, individualization of the operation based on the patient's anatomy and physiology, and meticulous attention to the technical details of the operation. Options for the child with a failed fundoplication include medical management, jejunal feeding using a percutaneous tube or a Roux-en-Y jejunostomy, revision of the fundoplication, or esophagogastric dissociation. If the fundoplication is to be revised, the same principles of patient selection, individualization of the operation, and attention to technique must be used to optimize the chance of success. The primary goal in the treatment of GER is to improve quality of life for the patient and the family.

Child↗
PubMed2002

Chronic constipation due to Hirschsprung's disease and desmosis coli in a family.

Five members of a family are described, all of whom suffered from chronic constipation and megacolon. Detailed clinical and histologic evaluation of each member revealed that two individuals have histologic evidence of desmosis coli and three have Hirschsprung's disease, one of whom also has desmosis coli. The latter combination has never been described before, either in a family or in a single patient. Genetic studies of the family did not reveal an increase in the number of shared markers for the RET proto-oncogene, suggesting that this previously undescribed familial association is likely not caused by a mutation in the RET gene, but by other genetic abnormalities.

Chronic Disease↗
PubMed2002

Feasibility of partial nephrectomy for Wilms' tumor in children with Beckwith-Wiedemann syndrome who have been screened with abdominal ultrasonography.

BACKGROUND: Children with Beckwith-Wiedemann syndrome (BWS), a congenital syndrome associated with Wilms' tumor commonly are screened with abdominal sonography resulting in detection of tumor at a lower stage. Wilms' tumors have been traditionally treated with complete nephrectomy; however, smaller tumors are amenable to nephron-sparing surgery. Because Wilms' tumors may be metachronous and nonmalignant disease may compromise renal function in BWS, nephron-sparing approaches may be desirable as the first option. METHODS: Seven patients with BWS and Wilms' tumor underwent nephrectomy. The preoperative computed tomography (CT) or ultrasound scan were evaluated by a pediatric surgeon to assess whether partial nephrectomy would have been feasible. The determining criteria included tumor involving one third or less of the kidney and no involvement of either hilar or vascular structures. RESULTS: Seven patients underwent complete nephrectomies. The remaining patient, who had undergone a left nephrectomy before the initiation of screening had salvage chemotherapy after biopsy results showed right kidney involvement with Wilms' tumor. CONCLUSIONS: Nephron-sparing surgery is reasonable to consider in children with Beckwith-Wiedemann syndrome who are screened at intervals of 4 months or less. The relative benefits of partial nephrectomy for children with Wilms' tumor-predisposing conditions only can be assessed in the setting of a cooperative clinical trial.

Beckwith-Wiedemann Syndrome↗
PubMed2002

Management of late-stage parapneumonic empyema.

PURPOSE: Despite the reported value of early video-assisted thoracoscopic surgery (VATS) for empyema, many children are still referred to the surgeon late in the disease process. The authors wished to determine the optimal management strategy for this group of children. METHODS: Medical records of all children (n = 70) from 1990 to 2000 with late-presenting empyema (stage II or III) were reviewed. Patients were grouped as (G1) successful management with chest tube (CT), (G2) surgery after initial CT, (G3) thoracentesis followed by surgery, and (G4) surgery alone. RESULTS: There were no significant differences with respect to age, gender, pleural cultures or fluid analysis. Fifty-one (73%) patients required surgical intervention. Treatment using CT (G1, G2) or thoracentesis (G3) was associated with prolonged length of stay (LOS) when compared with surgery alone (G4; 12 v 8 days). For G2, G3, and G4, rapid clinical improvement and early discharge (6 days) was seen after surgery. For all surgery groups (G2, G3, G4), video-assisted thoracoscopic surgery (n = 19) was associated with a longer postoperative fever (4 v 2 days; P <.05), but a shorter total LOS (12 v 15 days; P <.05) when compared with open decortication (n = 32). CONCLUSIONS: Over 70% of children with late presenting empyema required surgery, including more than half of the children who received initial chest tube drainage. Delay in surgery was associated with more procedures, more radiographs, and an increased LOS. Despite later intervention, patients undergoing surgery as an initial approach had the shortest length of stay. Early surgical intervention is indicated for most children referred with established empyema.

Adolescent↗
PubMed2002

Fundoplication and gastrostomy versus image-guided gastrojejunal tube for enteral feeding in neurologically impaired children with gastroesophageal reflux.

BACKGROUND: Neurologically impaired children with gastroesophageal reflux (GER) usually are treated with a fundoplication and gastrostomy (FG); however, this approach is associated with a high rate of complications and morbidity. The authors evaluated the image-guided gastrojejunal tube (GJ) as an alternative approach for this group of patients. METHODS: A retrospective review of 111 neurologically impaired patients with gastroesophageal reflux was performed. Patients underwent either FG (n = 63) or GJ (n = 48). All FGs were performed using an open technique by a pediatric surgeon, and all GJ tubes were placed by an interventional radiologist. RESULTS: The 2 groups were similar with respect to diagnosis, age, sex and indication for feeding tube. Patients in the GJ group were followed up for an average of 3.11 years, and those in the FG group for 5.71 years. The groups did not differ statistically with respect to most complications (bleeding, peritonitis, aspiration pneumonia, recurrent gastroesophageal reflux [GER], wound infection, failure to thrive, and death), subsequent GER related admissions, or cost. Children in the GJ group were more likely to continue taking antireflux medication after the procedure (P <.05). Also, there was a trend for GJ patients to have an increased incidence of bowel obstruction or intussusception (20.8% v 7.9%). Of the FG patients 36.5% experienced retching, and 12.7% experienced dysphagia. Eighty-five percent of patients in the GJ group experienced GJ tube-specific complications (breakage, blockage, dislodgment), and GJ tube manipulations were required an average of 1.68 times per year follow-up. Nine patients (14.3%) in the FG group had wrap failure, with 7 (11.1%) of these children requiring repeat fundoplication. In the GJ group, 8.3% of patients went on to require a fundoplication for persistent problems. A total of 14.5% of GJ patients had their tube removed by the end of the follow-up period because they no longer needed the tube for feeding. CONCLUSIONS: Image-guided gastrojejunal tubes are a reasonable alternative to fundoplication and gastrostomy for neurologically impaired children with GER. The majority can be inserted without general anesthesia. This technique failed in only 8.3% patients, and they subsequently required fundoplication. A total of 14.5% of GJ patients showed some spontaneous improvement and had their feeding tube removed. Each approach, however, still is associated with a significant complication rate. A randomized prospective study comparing these 2 approaches is needed.

Canada↗