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Biomedical subjects

Jacob C Langer

Publications and source records attributed to Jacob C Langer.

48 records · Page 3Linked to original sources

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↗

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↗

Incidence of medical error and adverse outcomes on a pediatric general surgery service.

BACKGROUND/PURPOSE: The Institute of Medicine has identified medical error as a leading cause of death and injury, with deaths resulting from medical error exceeding those caused by motor vehicle collisions, breast cancer, or AIDS. The authors examined the incidence and sources of medical error in relation to adverse events on a pediatric general surgery service. METHODS: All intensive care unit (ICU) and ward admissions to 2 staff pediatric general surgeons during a 1-month period were identified prospectively and in-patient care was reviewed daily by a 3-person panel consisting of a staff surgeon, a surgical fellow, and a nonmedical observer. Medical errors, identified through daily patient encounters, nursing rounds, medical rounds, and chart examinations, were evaluated based on type, hospital setting, personnel involved, and outcome. Adverse outcomes were evaluated based on type and contributing factors. RESULTS: Our study group included 64 patients. A total of 108 errors were identified; 28% of these errors resulted in adverse outcomes. One or more medical errors were identified in the care of two thirds of patients (43 of 64), with medical error contributing to adverse outcomes in one third of patients (21 of 64). Errors occurred most frequently in communication, postoperative monitoring and care, and diagnosis, with errors in postoperative care and diagnosis having the highest likelihood of resulting in an adverse outcome. Seventy-four adverse outcomes were identified in 31 patients; 35 (47%) of these outcomes, occurring in 21 patients, were attributable to medical error. The most common adverse outcomes identified were additional nonoperative procedures, of which, 92% resulted from medical error. There were no deaths. CONCLUSIONS: Medical error occurs in more than one half of hospital admissions on a general pediatric surgery service and contributes to a substantial number of adverse outcomes.

Adolescent↗

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↗

One-stage transanal Soave pullthrough for Hirschsprung disease: a multicenter experience with 141 children.

BACKGROUND: The surgical management of Hirschsprung's disease (HD) has evolved from the original 3-stage approach to the recent introduction of minimal-access single-stage techniques. We reviewed the early results of the transanal Soave pullthrough from 6 of the original centers to use it. METHODS: The clinical course of all children with HD undergoing a 1-stage transanal Soave pullthrough between 1995 and 2002 were reviewed. Children with a preliminary stoma or total colonic disease were excluded. RESULTS: There were 141 patients. Mean time between diagnosis and surgery was 32 days, and mean age at surgery was 146 days. Sixty-six (47%) underwent surgery in the first month of life. Forty-seven (33%) had the pathologic transition zone documented laparoscopically or through a small umbilical incision before beginning the anal dissection. Mean blood loss was 16 mL, and no patients required transfusion. Mean time to full feeding was 36 hours, mean postoperative hospital stay was 3.4 days, and 87 patients (62%) required only acetaminophen for pain. Early postoperative complications included perianal excoriation (11%), enterocolitis (6%), and stricture (4%). One patient died of congenital cardiac disease. Mean follow-up was 20 months; 81% had normal bowel function for age, 18% had minor problems, and 1% had major problems. Two patients required a second operation (twisted pullthrough, and residual aganglionosis). One patient developed postoperative adhesive bowel obstruction. CONCLUSION: To date, this report represents the largest series of patients undergoing the 1-stage transanal Soave pullthrough. This approach is safe, permits early feeding, causes minimal pain, facilitates early discharge, and presents a low rate of complications.

Child↗

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↗

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↗

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↗

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↗

[Medico-surgical consensus for management of children with gastroesophageal reflux Acapulco, Mexico 2002].

UNLABELLED: Gastroesophageal reflux (GER) is a common disease in children less one year old. It is present around 10% of unselected infant population. 40-50% have abnormal 24 h pH monitoring. An early diagnosis and treatment should be done in order to avoid complications. AIM: To establish the consensus for the diagnosis and treatment of children with GER, to rule out similar diseases avoid the use of unnecessary drugs and the secondary side effects as well as unnecessary surgery. METHOD: The consensus was done with the participation of general pediatricians, pediatrics gastroenterologist, pediatric surgeons, radiologist and endoscopist. An initial paper was done by pediatric surgeon and pediatric gastroenterologist who submitted to the rest of participants. Second stage: the paper was review through E-mail for all participants who send their suggestions and modifications. A new paper was done and discussed by medical and surgery area. During the Congress of Pediatric Surgery, in an open session was discuss again with the participation of the main authors and all the audience present. Finally, a paper was done and review for the main authors.

Child↗