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Biomedical subjects

L R Scherer

Publications and source records attributed to L R Scherer.

53 records · Page 3Linked to original sources

The spectrum of biliary tract disorders in infants and children. Experience with 300 cases.

OBJECTIVES: To assess the results of surgical treatment of infants and children with biliary tract disease. DESIGN: The records of children with biliary tract disorders requiring surgical intervention were reviewed retrospectively. Diagnosis, age, sex, clinical presentation, treatment, and outcome were evaluated. SETTING: A large pediatric referral facility. PATIENTS: A total of 300 patients treated from 1972 through 1993 were evaluated, including 102 with biliary atresia, 29 with choledochal cyst, and 169 with cholelithiasis. Hepatic portoenterostomy was performed in 87 patients with biliary atresia, and biopsy alone was performed in 15. Twenty girls and nine boys, 50% of whom were younger than 3 years, had choledochal cyst. Operative management included cyst excision and hepatojejunostomy in 25 patients, cyst jejunostomy in two patients, cystduodenostomy in one patient, and choledochocele excision in one patient. Gallstones were observed in 106 girls and 63 boys; 28 were aged 0 to 5 years, 31 were aged 6 to 10 years, and 110 were aged 11 to 18 years. Open cholecystectomy was performed in 143 patients, and laparoscopic cholecystectomy was performed in 26 patients. MAIN OUTCOME MEASURES: Principal outcomes examined were surgical morbidity and mortality. RESULTS: Hepatic portoenterostomy was successful in 28 (32%) of 87 patients with biliary atresia (all younger than 3 months), and 23 showed improvement following hepatic portoenterostomy; hepatic portoenterostomy failed in 36 patients. Twenty-eight (82.3%) of 34 patients survived liver transplantation. Overall survival was 71.5%. Survival of patients with choledochal cyst was 96.4% (28/29). Cholecystectomy was successful in all 169 patients. There were eight complications and one death (0.59% mortality). CONCLUSIONS: Survival in patients with biliary atresia (71%) has improved with hepatic portoenterostomy complemented by liver transplantation. Hepatic portoenterostomy is the procedure of choice in infants younger than 3 months. An improved outlook for patients with choledochal cyst can be expected after cyst excision and hepatojejunostomy. Gallstones are relatively common in children. Both open and laparoscopic cholecystectomy are safe and effective procedures in children.

Adolescent↗

Mediastinal tumors in children: experience with 196 cases.

BACKGROUND: Mediastinal masses are relatively common in infants and children. These lesions are often neoplastic in origin and have a high risk of malignancy. METHODS: This report concerns 196 infants and children with mediastinal tumors. Fifty-five cases (28%) were benign, and 141 (72%) were malignant. Diagnosis included Hodgkin's disease (47), neuroblastoma (46), non-Hodgkin's lymphoma (37), teratoma (18), ganglioneuroma (14), cystic hygroma (11), Schwannoma (five), germ-cell tumors (three), lipoma (three), thymic tumor (three), malignant histiocytosis (two), neurofibroma (two), mesenchymal sarcoma (one), rhabdomyosarcoma (one), peripheral neuroectodermal tumor (one), hamartoma (one), and hemangioma (one). Diagnoses were usually made by assessing the patient's age, radiologic evidence of tumor location, the presence of calcium in the tumor, and the presence of tumor markers (alpha-fetoprotein, vanillmandelic acid, human chorionic gonadotropin). Diagnoses were verified by histologic evaluation. Resection was the only treatment for benign tumors. Biopsy and chemotherapy (and/or radiation) were employed for lymphoid tumors, and resection and adjuvant therapy were used for other solid malignancies. RESULTS: Survival was achieved in 53 of 55 (96.3%) patients with benign tumors and 105 of 141 (74.4%) patients with malignant tumors. CONCLUSIONS: Seventy-two percent of mediastinal tumors in this study were malignant. Early diagnosis followed by biopsy and chemotherapy for lymphoid tumors or resection of nonlymphoid tumors along with aggressive adjuvant therapy result in high survival rates (74.4%). Children with benign tumors almost always survive (96.3%) after resection.

Adolescent↗

Gastrointestinal tumors in children: an analysis of 39 cases.

BACKGROUND: Gastrointestinal tumors are relatively uncommon in infants and children, and the histologic diagnoses differ from those seen in an adult practice. Furthermore, the clinical presentation of such tumors is quite variable. METHODS: We reviewed the records of 39 pediatric patients who had been treated for tumors of the alimentary tract at our hospital over the past 20 years. The symptoms, physical findings, treatments, and outcomes are tabulated and discussed. RESULTS: Non-Hodgkin's lymphoma was the predominant diagnosis. It was found in 22 children, and the survival rate was 60%. Other malignant tumors found in this pediatric series included colorectal carcinoma in four patients and gastric leiomyosarcoma in one. The outcomes of these patients were poor; four of the children have died, and one girl remains alive with extensive disease. Benign lesions included neurogenic tumors (n = 5), inflammatory pseudotumors (n = 3), hemangiomas (n = 2), teratoma and carcinoid (n = 1 each). These tumors were ultimately cured, with one exception, after surgical resection. CONCLUSIONS: Gastrointestinal tumors in children cover a broad spectrum of benign and malignant varieties and stem from conditions that differ significantly from those observed in adults. While treatment varies according to diagnosis, most patients respond best to complete resection of the primary tumor.

Adolescent↗

Criteria for safe cost-effective pediatric trauma triage: prehospital evaluation and distribution of injured children.

In an effort to maximize staff utilization, all pediatric trauma patients were triaged by emergency room personnel to one of two tiers, based on information reported by prehospital providers over radiotelephones. A total of 952 patients less than 15 years of age were evaluated during a 1-year period. The triage criteria had a sensitivity of 86% in predicting which trauma patients would require operating room and/or pediatric intensive care, while maintaining a specificity of 90%. Fifteen patients died; however, by TRISS methodology there were no unexpected deaths and four unexpected survivors. All eventual deaths were initially captured from field data by the severely injured triage criteria. The study data suggest that physician-controlled two-tiered field triage criteria can safely serve to maximize staff utilization in the emergency room.

Child↗

The complex nature of type A (long-gap) esophageal atresia.

BACKGROUND: The management of neonates with long-gap esophageal atresia without a fistula (type A) is complex. Options for esophageal reconstruction include use of the native esophagus or replacement with colon, stomach, or small bowel. The purpose of this study was to evaluate the long-term outcome of children variously treated with this defect. METHODS: The course of 23 infants with type A esophageal atresia were retrospectively reviewed. Diagnosis was achieved by plain radiographs and endoscopy. The mean gestational age was 33.9 weeks, and the birth weight was 1.87 kilograms. RESULTS: Two of nine infants with serious associated defects died early, and one infant with brain damage was not reconstructed. Initial treatment consisted of gastrostomy and proximal pouch suction (n = 10) or cervical esophagostomy (n = 10). Reconstructive procedures were performed in 20 infants, including colon interposition in eight infants, reversed gastric tube in two infants, and delayed primary esophageal repair (often with myotomy) in 10 infants. Secondary procedures were required for three infants with failed colons, one infant with a failed gastric tube, and one infant with a native esophageal disruption. Long-term satisfaction was 90% with esophagus, 63% with colon, and 50% with stomach. CONCLUSIONS: When possible, native esophageal reconstruction is the procedure of choice rather than esophageal replacement.

Anastomosis, Surgical↗

Inguinal hernia and umbilical anomalies.

Inguinal hernias and umbilical anomalies remain the most common congenital anomalies. The loss of testis, ovary, or a portion of bowel from an irreducible hernia and the infectious complications of umbilical anomalies continue to be a threat to infants and young children. This article reviews the embryology, clinical features, and treatments of these anomalies and discusses some of the unusual and special considerations of these children.

Child↗

Vascularity of tumors in children: evaluation with color Doppler imaging.

Twenty-one tumors in 20 children were evaluated with duplex and color Doppler imaging to determine the value of the technique in assessing the origin and pattern of vascular supply and the degree of neovascularity. The origin of the vascular supply was detected correctly in 12 of 13 tumors that were subsequently resected. In five children, this aided in determining the organ from which the tumor originated, and in one child, it established the presence of a tumor by showing blood flow in the center of a suspected abscess. In 18 tumors, color Doppler imaging showed the pattern of the vascular supply. Eleven had a peripheral pattern, and seven had a central, branching pattern. Although individual tumor types appeared to have characteristic patterns of vascular supply, these were not specific enough to aid in making a specific diagnosis. When the degree of intratumor neovascularity was graded on the basis of the findings on color Doppler imaging, it agreed with the results of histologic evaluation in 16 of 19 tumors. In one tumor, neovascularity was overestimated, and in two, it was markedly underestimated. Our experience suggests that color Doppler imaging is useful in detecting the origin and pattern of vascular supply and the degree of intratumoral blood flow in a variety of solid tumors in children.

Adolescent↗

Bowel visualization during indium-111-labelled diethylene triamine penta-acetic acid cisternography due to massive cerebrospinal fluid leak. Case report and review of the literature.

We report a case of massive cerebrospinal fluid (CSF) leakage where the tracer injected intra-thecally for radionuclide cisternography was later visualized in the bowel as well as the nasopharynx. We discuss the potential implications of this finding in patients with CSF leaks. A brief review of the diagnosis of CSF leaks is included.

Brain↗

The ontogeny of the gut-associated lymphoid tissue in short bowel syndrome.

Newborns suffering from short bowel syndrome (SBS) after massive intestinal resection have numerous infectious complications, which may be due to immunoincompetence from the loss of gut-associated lymphoid tissue (GALT). This study examines the ontogeny of GALT in the rat with SBS. A total of 36 3-week-old rats were divided into two groups: I, sham operated (C, n = 16); and II (SBS, n = 20), with a 50% resection of small intestine with jejunoileostomy. At 4, 5, 6, and 10 weeks of age the animals were sacrificed and the GALT was assessed by video analysis and immunoperoxidase monoclonal antibodies, OX8 (CD8), W-3/25 (CD4), and MARA-2 (IgA). The data were expressed as positive staining lymphocytes per 10(4) microns2 (mean +/- SD). table; see text In the sham group there was an increase in all lymphocyte subsets over time. In the SBS group there was a rapid fall in OX8 and W-3/25 T-lymphocytes by 10 weeks, with no increase in IgA plasma cells at 6 and 10 weeks. This study demonstrates that in the massive bowel resection in the suckling rat decreases the T- and B-lymphocyte populations in the GALT. This lack of development may underlie the associated infectious complications and malabsorption in SBS.

Animals↗

Neonatal diagnosis of a presacral mass in the presence of congenital anal stenosis and partial sacral agenesis.

The simultaneous presentation of clinically symptomatic anal anomalies and roentgenographically demonstrated sacral dysgenesis should alert the pediatric surgeon to investigate for the presence of a presacral malformation. We report on such a case to illustrate a new radiographic technique that facilitates diagnosis and management of complex congenital malformations. A 1-day-old white boy presented with anal stenosis, a scimitar-shaped sacrum, and large anterior and posterior meningoceles. In addition, a distinct presacral tumor--a teratoma--was identified. These malformations were identified utilizing metrizamide myelography and three-dimensional reconstruction computed tomography (CT) scanning. The meningoceles and a tethered cord were successfully corrected utilizing a posterior approach. A diverting colostomy was performed and subsequently taken down. Two years postoperatively, the patient continues to do well. This case demonstrates that this triad of anomalies (presacral mass, sacral dysgenesis, and anorectal malformation), once considered, can be safely detected with modern radiologic techniques and can be expeditiously corrected during infancy before further deterioration occurs.

Abnormalities, Multiple↗

Outcome of pectus excavatum in patients with Marfan syndrome and in the general population.

We reviewed the records of 28 patients with Marfan syndrome and 30 age-matched control patients with presumed isolated pectus excavatum to determine the outcome of surgical repair of the pectus deformity in Marfan syndrome. One third of the patients with Marfan syndrome underwent repair of the pectus excavatum before diagnosis. Of the 30 patients with "isolated" pectus excavatum, 17 had findings by history or physical examination, such as mitral valve prolapse, scoliosis, or a relative with pectus excavatum, suggestive of an underlying disorder of connective tissue. Pectus excavatum of more than moderate severity recurred in 11 of 28 patients with Marfan syndrome and was associated with young age at initial surgery and lack of temporary internal stabilization of the chest after surgery. Only two of the control patients had recurrence of the defect; one of these patients had findings suggestive of an underlying heritable disorder of connective tissue. We conclude that pectus excavatum may indicate the presence of an underlying heritable disorder of connective tissue such as the Marfan syndrome. In patients with Marfan syndrome, and possibly other inherited connective tissue disorders, surgical repair should be delayed if possible until skeletal maturity is nearly complete and should employ internal stabilization.

Adolescent↗

Evolving management of pectus excavatum based on a single institutional experience of 664 patients.

Most pediatricians and family physicians believe that children with pectus excavatum require surgery only for cosmetic indications and then only in teenagers. We believe pectus excavatum should be repaired in childhood (1) to relieve structural compression of the chest and allow normal growth of the thorax; (2) to prevent pulmonary and cardiac dysfunction in teenagers and adults; and (3) to obviate the cosmetic impact that may cause a child to avoid sports and gymnastics. Preoperative CT scans now help select those children who need repair to prevent progressive deformities. Pulmonary function studies during vigorous exercise can document respiratory dysfunction in teenagers. These features are reversible if repair is completed before the pubertal growth spurt. The ideal age for repair is 4 to 6 years, which permits enough emotional maturity for a positive hospital experience and avoids later psychological effects. Repair at an earlier age has no operative advantages. Our operative technique consists of the removal of three to four overgrown costal cartilages, repositioning of the sternum with a transverse osteotomy, and internal support using the child's lowest normal ribs, avoiding any prosthetic support. To prevent recurrence in teenagers, we add a temporary bar beneath the sternum to prevent depression of the sternum from the weight of the chest-wall muscle mass. Six hundred sixty-four patients have been followed for 1 to 40 years; 95% have excellent long-term results and only 5% have mild to moderate recurrences. Our current techniques of patient selection and surgery will be presented.

Adolescent↗

Surgical management of children and young adults with Marfan syndrome and pectus excavatum.

Significant chest wall deformities occur in two thirds of children with Marfan syndrome (MS). The symptoms, physical findings, and surgical outcome of 11 patients with MS and severe pectus excavatum who required operative repair were reviewed. The diagnosis of MS was made before the pectus repair in six patients, at the time of evaluation of pectus in two patients, and after the repair in three patients. Symptoms included dyspnea upon exertion, tachypnea, and chest pain. Physical findings included aortic root enlargement or valvular disease, mitral valve disease, ligamentous disease, congestive heart failure, and ocular disease. All patients had severe pectus deformities with a narrow anteroposterior diameter in the midline, as well as a broad chest defect that extended bilaterally to the midclavicular line. The heart was shifted into the left side of the chest in all patients. A Ravitch-type pectus repair with a stainless steel substernal strut was used in eight patients, with one patient suffering a late recurrence; in three patients no strut was used, and all three had recurrence. There were no postoperative complications. In the postoperative follow-up of seven patients, symptomatic improvement of cardiopulmonary performance was noted. Four of the patients required subsequent open heart surgery, including replacement of the aortic valve in one patient, and composite grafts of the ascending aorta in three patients. Postoperative cardiac arrest was the only major open heart complication. All four patients recovered and did well after surgery, showing significant cardiac and pulmonary functional improvement.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

Multiple esophageal duplication cysts.

During thoracic exploration for resection of an esophageal duplication cyst, a second such cyst was encountered and removed. Although the association of esophageal duplication cysts with other intestinal duplications has been described, to our knowledge this is the first reported case of multiple esophageal duplication cysts.

Esophageal Cyst↗

Surgical treatment of total colonic aganglionosis: efficacy of aganglionic patch enteroplasty in the rat.

This study evaluates ileo endorectal pull-through (IEP) with and without an aganglionic colon patch in an experimental rat model for the surgical treatment of total colonic aganglionosis (TCA). Animals were randomly assigned to Group 1 (no patch, No. = 6), Group 2 (right colon patch, No. = 8), Group 3 (transverse colon patch, No. = 7), Group 4 (left colon patch; No. = 6), sham operation (SH, No. = 6), and unoperated controls (UC, No. = 10). Change in percent body weight at 4 weeks after operation was -30.9 +/- 3.68% in Group 1, +5.1 +/- 1.67% in Group 2, -3.4 +/- 3.96% in Group 3, -1.8 +/- 4.17% in Group 4, and +12.7 +/- 1.54% in SH (Group 1 v other groups: P less than .001). Restoration of initial body weight occurred in 100% in Group 2 and SH (8/8 and 6/6, respectively), 50% in Group 4 (3/6), 42.9% in Group 3 (3/7), and 0% in Group 1 (0/6). Transit time (stomach to anus) was significantly shorter in Group 1. All rats in patched groups had a prolonged transit time compared with Group 1. Manometric studies in IEP rats showed favorable anal canal pressures, which were slightly lower than SH and UC. Water and Na+ absorption were significantly greater in patched groups. Rats with a right colon patch (Group 2) showed slightly greater absorption at 4 weeks. These data suggest that an aganglionic colon patch may be an important adjunct in the surgical treatment of TCA.

Animals↗

The effect of polycythemia and hyperviscosity on bowel ischemia.

Recent clinical reports suggest that newborn infants with polycythemia and other causes of hyperviscosity may be at risk for developing necrotizing enterocolitis (NEC). This study evaluates the relationship of polycythemia and increased blood viscosity on survival and bowel integrity and an ischemic bowel model in rats. Ninety-nine weanling Sprague Dawley rats underwent one-minute occlusion of the superior mesenteric artery. Animals were divided into five treatment groups. Group 1 (n = 40) ischemic controls had a mean hematocrit (HCT) of 44.3 +/- 3.3%. Group 2 (n = 19) rats were transfused with whole blood from donor rats followed by a dose of lasix (2mg/kg) intravenously (IV) 20 hours prior to ischemic injury, raising the HCT to 53.6 +/- 3.2%. Group 3 (n = 19) animals were transfused with whole blood X 2 and given lasix at 20 and 4 hours before operation, raising the HCT to 63.0 +/- 1.6%. Group 4 (n = 16) (HCT 68.6 +/- 2.55%) and group 5 (n = 15) rats (HCT 71.6 +/- 2.07%) were prepared with multiple blood transfusions and given lasix as group 3 animals to achieve those hematocrit levels. Animals were kept in individual cages and fed rat chow and water ad libitum. Survival, length of survival, and evidence of bowel perforation or necrosis were recorded at seven days following the ischemic insult. Blood viscosity was determined in each group. Survival at one week was 65% in group 1, 63% in group 2, and 63% in group 3.(ABSTRACT TRUNCATED AT 250 WORDS)

Animals↗

Staphylococcus epidermidis sepsis in pediatric patients: clinical and therapeutic considerations.

This report concerns 60 children with documented Staphylococcus epidermidis sepsis. There were 34 boys and 26 girls, ages 2 weeks to 15 years. The primary diagnosis included malignancy (13), congenital (13) or acquired (11) gastrointestinal disorders, prematurity (7), cardiac defect (5), hydrocephalus (2) and miscellaneous (9). Clinical presentation included fever (54), tachycardia (15), lethargy (20), hypotension (8), irritability (6), increased gastric residuals (6) and apnea/bradycardia (3). A documented source of sepsis was noted in 56 patients, including percutaneous central venous catheters (23), Broviac catheters (17), umbilical arterial catheters (6), wound (3), V-P shunt (2), cardiac defect (2), cholangitis (1), chest tube (1) and peripheral arterial line (1). There were six sepsis-related deaths, four in premature infants. Two of six infected subclavian catheters were treated successfully with vancomycin. Infection was successfully cleared in 20 of 23 infected Broviac catheters with vancomycin through the line. However, six were eventually removed for tract infection (1), persistent fever (2), and Candida sp. infection (3). Although once considered a non-pathogenic skin contaminant, S. epidermidis has emerged as a serious pathogen in hospitalized, immunosuppressed, premature and malnourished pediatric patients. Indwelling catheters enhance the likelihood of infection in these patients. Aggressive antimicrobial therapy is vital in this potentially lethal infection. Vancomycin proved efficacious in this series.

Adolescent↗