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Biomedical subjects

R C Shamberger

Publications and source records attributed to R C Shamberger.

At least 55 records · Page 3Linked to original sources
PubMed1995

Association of imperforate anus with occult spinal dysraphism.

Neurological deficits may occur in some children with imperforate anus produced by unrecognized spinal cord anomalies amenable to neurosurgical correction. Although this association has been well described, its frequency has not been defined. The authors prospectively studied 45 infants with imperforate anus from 1985 to 1992 to establish the frequency of this association. Eighteen infants (40%) had high imperforate anus, 24 had low imperforate anus (53%), and 3 had anal stenosis (7%). All underwent radiographic spinal cord imaging in early infancy, which consisted of radiographs (45), ultrasound (26), magnetic resonance imaging (29), computed tomography (1), and myelography (1). Thirty-three percent of the patients with imperforate anus had bony sacral abnormalities. Eight infants were discovered to have spinal cord abnormalities. Two of those infants had obvious myelomeningocele at birth, and 6 infants (14%) had occult spinal dysraphism consisting of tethered cord with or without associated lipoma of the cord. There was no correlation between the presence of tethered cord and the gender of the infants, the type of malformation (high versus low imperforate anus), or the presence of sacral dysplasia (Fisher's exact test). Five of the infants who were found to have tethered cord underwent neurosurgical intervention and repair of the defect, and 1 infant was followed up clinically. The mean follow-up time of all the infants was 2.5 +/- 0.9 (+/- SD) years. No neurological sequelae occurred in the infants who had surgical correction. The frequency of occult spinal dysraphism with imperforate anus was 14%. It was independent of the type of imperforate anus or the coexistence of bony sacral anomalies.(ABSTRACT TRUNCATED AT 250 WORDS)

Anus Diseases↗
PubMed1995

Splenic preservation in the management of splenic epidermoid cysts in children.

Splenic epidermoid cysts are a rare entity. The traditional treatment was splenectomy, performed to prevent infection, hemorrhage, or rupture of the cyst. The authors reviewed their series of 19 children with congenital splenic cysts from 1914 to 1993 to assess the change in management of these lesions. The age of time of presentation ranged from newborn to 17 years (median, 12.0 years) The male:female ratio was 1.0:1.1. Five cysts were found incidentally at the time of autopsy. The cysts were 0.5 to 24 cm in diameter (median, 12.0 cm). Only cysts greater than 8 cm presented with clinical symptoms: 64% with an abdominal mass and/or 78% with abdominal pain. One patient presented with a left varicocele. There were no complications related to the cysts. A variety of radiographic studies were performed, but ultrasonography provided all required diagnostic information. All patients treated before 1971 (n = 9) underwent splenectomy, and five patients treated since 1983 had hemisplenectomy or cystectomy. Postoperative spleen scans (obtained in three of these patients) and platelet counts showed preservation of splenic function. Pathological examination showed epidermoid cysts in all cases. There were no postoperative complications. In conclusion, in this series of epidermoid splenic cysts in children, (1) the cysts presented with an abdominal mass and/or abdominal pain only if they were greater than 8 cm in size, and there were no complications owing to the cysts; (2) ultrasonography was the most cost-effective and least invasive method of evaluation; and (3) splenic preservation by hemisplenectomy or cystectomy, used to avoid the long-term risks of splenectomy, proved to be a safe, easy procedure, with maintenance of splenic function.

Adolescent↗
PubMed1995

Prospective evaluation by computed tomography and pulmonary function tests of children with mediastinal masses.

BACKGROUND: Our ability to predict respiratory compromise during general anesthesia in a child with an anterior mediastinal mass is limited. Two prior reports have found a correlation between adequacy of ventilation during general anesthesia and the tracheal cross-sectional area obtained from computed tomograms (computed tomography [CT] scans). These and other reports have suggested that pulmonary function tests may provide additional information regarding anesthetic risks, but no studies have evaluated the extent of respiratory compromise in children with an anterior mediastinal mass. METHODS: We prospectively evaluated 31 children with mediastinal masses before 34 surgical procedures. At each evaluation the tracheal area (as a percent of the predicted area on the basis of age and gender) was determined by CT. Pulmonary function tests were performed in the sitting and supine positions. The eleven children with either a tracheal area or peak expiratory flow rate (PEFR) of less than 50% of predicted received only a local anesthetic; the majority of children above these levels (17 of 22) received a general anesthetic. RESULTS: Eleven of 31 patients had significant pulmonary restriction as defined by total lung capacity of less than 75% of predicted. Eight patients had a PEFR in the supine position of less than 50% of predicted. PEFR was lower in the supine than the upright position in all patients (median value of decrease, 12%). In 28 of 34 evaluations the child had a tracheal area greater than 50% of predicted, a criterion proposed for safe utilization of general anesthesia. This latter guideline, however, did not identify all patients with significant impairment of pulmonary function; five patients had a PEFR of less than 50% of predicted but tracheal areas of greater than 50% of predicted. All children were administered anesthetics uneventfully with these guidelines. CONCLUSIONS: Although the tracheal area can be accurately measured with the CT scan, this does not identify all children with mediastinal masses and abnormal pulmonary function. A large mass may produce significant restrictive impairment and hence reduction in PEFR by the intrathoracic volume it occupies and yet not cause tracheal compression. It may also reduce the PEFR by narrowing the bronchi distal to the carina. Currently no CT standards exist for measuring bronchial areas in children. Our study did not evaluate whether impaired pulmonary function as measured by PEFR would be predictive of respiratory collapse during general anesthesia because all were excluded and operated on under local anesthesia. General anesthesia was well tolerated in children with tracheal area and PEFR greater than 50% of predicted. Pulmonary function tests in children with anterior mediastinal masses may add valuable information to the anatomic evaluation obtained by CT scan.

Adolescent↗
PubMed1995

Interposed jejunal segment with nipple valve to prevent reflux in biliary reconstruction.

BACKGROUND: Resection is the accepted management of a choledochal cyst. However, the debate continues regarding the optimal method of biliary reconstruction. The Roux-en-Y limb is used most frequently, but concerns have been raised about this method due to associated peptic ulcer disease, cholangitis, and poor growth. A method of reconstruction using an interposed segment of jejunum with a nipple valve placed between the common bile duct and the duodenum has been proposed. STUDY DESIGN: We have reviewed a series of 12 children requiring biliary reconstruction for choledochal cyst (11 children) and biliary stricture (one child). All had reconstruction with a nipple valve, and ten had an interposed segment of jejunum. RESULTS: All of the children are alive and have had follow-up evaluation from six months to 8.5 years (median of three years). Sequential examinations with ultrasound and biliary excretion scans have shown no evidence of obstruction, and liver function tests have remained normal. Three children have had cholangitis. One child had a brief episode in the perioperative period. The second child had cholangitis 16 months postoperatively, and the third child had multiple episodes of cholangitis. These latter two children were unique. One had Alonso-Lej type IV choledochal cyst with intrahepatic dilatation, which persisted after reconstruction. The other had a prior diversion with a Roux-en-Y limb from the gallbladder after resection of a choledochal cyst and had multiple episodes of cholangitis before reconstruction. These episodes are now controlled with chronic antibiotic suppression. Postoperative complications were limited to two episodes of obstruction of the small bowel requiring lysis of adhesions. No child has had peptic ulcer disease. These children have grown well after reconstruction, except for three with multiple anomalies or chronic pancreatitis. CONCLUSIONS: Biliary reconstruction with a jejunal interposition containing a nipple valve can be performed safely with a low incidence of complications. It offers a more physiologic method of reconstruction and a low incidence of postoperative cholangitis.

Adolescent↗
PubMed1994

Recent experience with Wilms' tumor: 1978-1991.

BACKGROUND: The treatment of Wilms' tumor has undergone major advances in the past four decades. Current therapy is based on both the stage and pathology of the tumor. We have reviewed our recent experience with this tumor to assess the results of treatment on protocols that generally avoid the use of doxorubicin. METHODS: Between January 1978 and December 1991 we treated 114 children with renal tumors. Ninety-one (80%) had favorable histology Wilms' tumor and 23 (20%), unfavorable histology tumors (13 anaplastic Wilms' tumors, four clear cell tumors of the kidney, and six sarcomatous tumors with rhabdoid elements). Chemotherapy was divided into two eras: 1978-1982 (vincristine and actinomycin-D) and 1983-1991 [vincristine and actinomycin-D for all patients, and cyclophosphamide for those with favorable histology and metastatic disease (n = 13) and all patients with anaplastic histology (n = 13)]. Four patients with clear cell or rhabdoid/sarcomatous tumor, three of whom are disease-free, were treated with a five-drug regimen (vincristine, doxorubin, cyclophosphamide, platinum, and VP-16). Approximately two thirds of the patients received megavoltage radiotherapy to the tumor bed. Mean abdominal radiation doses from 1978 to 1982 were slightly higher than those used from 1983 to 1991 (2,597 +/- 782 cGy vs. 2,039 +/- 524 cGy, respectively). RESULTS: No isolated local failures were observed in any favorable histology patient who received radiotherapy. Among the 91 patients with favorable histology, there was no statistically significant difference in event-free survival irrespective of stage. Outcome for patients with anaplastic or clear cell variants was not different from that for those patients with favorable histology, but there were only small numbers in these groups for comparison. Only children with a rhabdoid/sarcomatous variant demonstrated survival that was different from all others (p = 0.00). Our previously reported patients with stage I tumors (< 550 g) (n = 11) who were treated by nephrectomy only continue to have an excellent outcome (survival 100%). CONCLUSIONS: Wilms' tumors remain highly curable. Some unfavorable histology tumors require intensive therapy. Others such as our stage I tumors may be best observed without adjuvant therapy to avoid the toxicity of treatment.

Child↗
PubMed1994

The importance of staging laparotomy in pediatric Hodgkin's disease.

The findings of 247 pediatric patients who presented with supradiaphragmatic Hodgkin's disease and underwent staging laparotomies between April 1969 and December 1991 were reviewed to assess the importance of the staging laparotomy in pediatric Hodgkin's disease. A change in stage occurred in 25% of the cases reviewed. Fifty of the 202 (25%) clinical stage (CS) I or II patients were upstaged to pathological stage (PS) III or IV, and 12 of the 45 (27%) clinical stage III or IV patients were downstaged to pathological stage I or II. Possible risk factors for positive surgical staging, including gender, age, presence or absence of B symptoms, extent of involvement above the diaphragm, and histological type, were used to define subgroups of patients. Three statistically significant subgroups of patients with less than a 10% chance of restaging were identified. These groups included CS I and II patients with lymphocyte-predominant histology, CS I females, and CS III and IV females with nonlymphocyte predominant histology. These subgroups represent 24% of the cohort. Because CS is an accurate predictor of PS in these groups, treatment could be based solely on CS. The impact of radiographic imaging techniques on correctly predicting pathological stage was assessed. The rates of restaging for individuals with lymphangiography or computed axial tomography were not statistically different from those of patients without these radiographic studies. Therefore, abdominal imaging is not a substitute for surgical staging. No mortality and 2.8% morbidity occurred from staging laparotomy. Postsplenectomy sepsis and small bowel obstruction were the most common complications. Ninety-six percent of upstaged patients had splenic involvement, and 54% had positive nodal involvement.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1994

Effect of Golytely on serum electrolytes and hydration status of infants.

Golytely (GLP) is a commonly used oral preparation for infants and children undergoing intestinal surgery. Although the composition of GLP, a nonabsorbable electrolyte lavage solution, is designed to avoid electrolyte shifts and dehydration, systematic evaluation of the effects of this preparation on the hydration status and serum electrolytes of infants has not been performed. The authors evaluated 48 children (0 to 24 months of age) who received GLP. Serum electrolytes and blood urea nitrogen measurements were obtained before and after GLP administration. During GLP administration all the patients were allowed to drink clear fluids, including Rehydralyte in unrestricted quantities. Only two received intravenous fluids during the preparation. Statistical evaluation of the shifts in electrolytes were performed by the Students paired t test. Although the shift in serum electrolytes may be statistically significant, with a decrease in potassium and chloride and an increase in CO2, these changes were not clinically significant.

Child, Preschool↗
PubMed1994

Size disparity in pediatric lung transplantation.

Donor scarcity and the limitations of organ preservation dictate that a wide size range be used to maximize effective donor utilization in pulmonary transplantation. Problems of size disparity are magnified in the pediatric population, where relevant dimensions vary considerably by age. There have been 10 pediatric lung recipients (7 bilateral, 2 single, 1 heart-lung) at our institution since 1991. The age range was 3 to 26 years (mean, 13.7), and the weight range was 15 to 57 kg (mean, 33.6). Diagnoses included cystic fibrosis (5), pulmonary fibrosis (2), pulmonary hypertension (2), and arteriovenous malformation (1). The donor-to-recipient weight ratio ranged from 0.45 to 1.9, and the donor-to-recipient thoracic height ratio ranged from 0.6 to 1.6. Lobar resection and delayed chest closure was required in one case and was successful. Cardiopulmonary bypass was used electively in seven cases. No reexplorations for bleeding were necessary. Two of the three hospital deaths resulted from right ventricular failure, judged to be secondary to excessive pulmonary artery pressures intraoperatively associated with small donor allografts. Elective bypass had not been used. Inhaled nitric oxide (6 patients) and/or extracorporeal membrane oxygenation (2 patients) were used for temporary postoperative support. The hospital survival rate was 70% (7/10). One late death occurred 14 months postoperatively and was caused by obliterative bronchiolitis. The authors conclude that size disparity is a significant problem in pediatric lung transplantation. However, with elective use of cardiopulmonary bypass and aggressive postoperative support, a broad size range can be used.

Adolescent↗
PubMed1994

Malignant small round cell tumor (Ewing's-PNET) of the chest wall in children.

From 1976 to 1989 the authors treated 21 infants and children with malignant tumors of the chest wall. Fifteen were classified as Askin's tumors or Ewing's sarcoma, which we now consider as a single entity of primitive neuroectodermal origin. They are infrequent but highly aggressive tumors that involve the chest wall in children. Five patients presented with metastatic disease; despite chemotherapy and radiotherapy, all succumbed to progressive disease. Ten patients with localized disease received combined modality therapy including surgical resection (three after initial chemotherapy), radiotherapy, and chemotherapy. Surgery involved resection of the mass and up to three ribs, with prosthetic mesh reconstruction in one patient. Six of the 10 patients with localized disease are continuously disease-free 3.5 to 9 years (median, 5 years) following diagnosis. No patient had local recurrence. Of the four whose treatment failed, one died (free of disease) from complications after resection of an extensive primary tumor. In the second patient, acute monocytic leukemia developed shortly after relapse in a distant bone site. The patient died during induction for the leukemia. In the other two patients, hilar and carinal lymph node relapse occurred 68 and 80 months after initial treatment. One of the patients is considered in second remission (now 105 months later) after further chemotherapy (Adria-VAC) and radiation; the other succumbed to secondary relapse 17 months after second remission was achieved through chemotherapy, radiation, and surgery. Initial percutaneous needle biopsy of the large lesions has provided adequate material for histological, immunohistochemical, cytogenetic and ultrastructural diagnosis, and permitted initial chemotherapy before proceeding to surgical resection.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1994

Anorectal function in children after ileoanal pull-through.

Mucosal proctectomy and ileoanal pull-through is increasingly used in children requiring total colectomy for ulcerative colitis or familial polyposis. Excellent continence can be achieved with this procedure, and it avoids proctocolectomy and permanent ileostomy. We have evaluated prospectively anorectal function in nine consecutively treated children who underwent ileoanal pull-through. Patients were 8 to 17.5 years of age (median, 11.3 years) at the time of surgery; seven had ulcerative colitis, and two had familial polyposis. Anorectal evaluation was performed before colectomy and ileoanal pull-through, following ileoanal pullthrough, after rectal training, and then at yearly intervals. A biofeedback "rectal training" program was instituted 6 weeks after ileoanal pull-through and a contrast study documenting integrity of the pouch. The program consisted of an initial biofeedback session with the motility unit, followed by daily instillations, through a catheter, of progressively larger volumes of water (from 1 to 6 oz, increasing 1 oz per week) into the ileal pouch. Patients were instructed to retain the water and participate in normal activities after the instillation. This protocol acclimated the patient to sensing distension of the pouch and using the sphincters. The follow-up period ranges from 1 to 4.5 years (median, 2.2 years). All patients are continent by day and night. One patient has nocturnal incontinence with episodes of pouchitis. Stool frequency is three to eight movements per day (median, four), with none at night. Preoperative resting rectal sphincter pressures averaged 74.3 +/- 23.1 mm Hg (mean +/- standard deviation), and a maximum squeeze pressure was 93.9 +/- 25.3 mm Hg.(ABSTRACT TRUNCATED AT 250 WORDS)

Adenomatous Polyposis Coli↗
PubMed1994

Chest wall tumors in infants and children.

Chest wall tumors are infrequent in infants and children, but a high proportion of these tumors are malignant. They present most frequently as a palpable mass, and less frequently with pain or respiratory distress. Radiographic evaluation should include chest radiographs followed by computed tomographic (CT) scan. In most cases an initial incisional biopsy is performed because of the significant risk of malignancy. The most frequent tumors are the malignant small round cell tumors (Ewing's sarcoma/primitive neuroectodermal tumor [PNET] family) followed by rhabdomyosarcoma, osteosarcoma, chondrosarcoma, and a spectrum of other sarcomas. Initial treatment with chemotherapy, particularly for the malignant small round cell tumors and osteosarcoma, may facilitate resection by decreasing the size of the tumor as well as its vascularity and friability. Cure requires successful local control and adjuvant chemotherapy and is particularly difficult to achieve in children presenting with metastases.

Child↗
PubMed1994

Long-term nutritional and metabolic consequences of pancreaticoduodenectomy in children.

BACKGROUND: The long-term nutritional and metabolic consequences of pancreaticoduodenectomy in children are unknown. METHODS: Five children were evaluated in a clinical research center 2.5 to 10 years after pancreaticoduodenectomy to assess their nutritional status based on patterns of growth and to assess their gastrointestinal function. Investigation included vitamin levels, a bentiromide study, and serum immunoreactive trypsinogen levels to evaluate pancreatic function and a d-xylose absorption and a radionuclide gastric emptying scan for intestinal absorption and motility. RESULTS: Children were able to grow after pancreaticoduodenectomy. Three remained in low percentile groups for height/weight ratio, and two were near or above normal. Low normal levels of the fat-soluble vitamins were present. Very low levels of pancreatic function were found based on the bentiromide and trypsinogen studies, whereas intestinal absorption of d-xylose was normal except for one patient with extremely rapid gastric emptying. CONCLUSIONS: After pancreaticoduodenectomy children can grow and develop normally if given adequate levels of oral pancreatic supplements to replace the severely decreased level of endogenous pancreatic enzymes after operation. Routine supplementation of the fat-soluble vitamins should be considered.

4-Aminobenzoic Acid↗
PubMed1993

Posterior mediastinal masses.

Solid mediastinal masses in infancy and childhood occur most frequently in the posterior mediastinum. From 1972 to 1989, 63 patients presented with a posterior mediastinal mass. The median age at diagnosis was 6 years (range, 1 day to 26 years). Thirty patients were female. Forty-five percent of the patients presented with respiratory symptoms or chest pain; 13% had neurologic symptoms, one half of which were related to spinal cord compression; and 5% had a palpable mass. In 32% of patients the mass was an incidental finding. The tumors were of neurogenic origin in 89% of patients, of which neuroblastoma was the most common. Of all patients with posterior mediastinal masses, 60% had malignant tumors. Median follow-up for 62 of 63 evaluable patients was 45 months (range, 1 to 289 months). One patient was lost to follow-up. Of the 62 patients followed, 84% are alive and free of disease. All but 4 of the 32 patients with neuroblastoma are alive and free of disease with a median follow-up of 73 months (range, 7 to 289 months). Patients with neuroblastoma who were diagnosed in the first year of life had a significantly better survival pattern than those presenting after the first year. There were seven deaths in the series: four from neuroblastoma, two from primitive neuroectodermal tumor, and one from malignant schwannoma. Preoperative diagnostic evaluation of a posterior mediastinal mass should include posteroanterior and lateral chest roentgenograms, and either CT or MRI of the chest and abdomen to assess the extent of the mass.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1993

Perioperative management for cholecystectomy in sickle cell disease.

Perioperative complications of surgical procedures are frequently encountered in patients with sickle cell disease. We have reviewed our series of patients with hemoglobinopathies who underwent cholecystectomy from 1978 to 1991 to evaluate their perioperative management and clinical outcome. Twenty-two children with major sickle hemoglobinopathy underwent cholecystectomy for symptomatic cholelithiasis. All 22 were transfused to achieve a hemoglobin (Hgb) level greater than 9 g/dL and hemoglobin S (HbS) less than 37%. Fourteen underwent immediate preoperative automated red cell exchange (ARCE). The median preexchange Hgb of these patients was 8.1 g/dL (range, 6.8 to 10.5). Their median HbS was 84% (range, 53% to 97%). These patients underwent placement of an apheresis catheter under local anesthesia followed by red cell exchange. The median volume of packed red blood cells (PRBC) exchanged was 28.1 mL/kg (range, 13.8 to 58.7). The median HbS after exchange was 21% (range, 16% to 37%) and the median Hgb was 10.6 g/dL (range, 6.5 to 16.7). Eight other patients underwent sequential transfusion (3 after an exchange for an acute pulmonary vasoocclusive crisis). These patients had been prepared over an interval of 2 to 8 weeks preoperatively and had received a median of 26.9 mL PRBC/kg (range, 12.8 to 95). Following sequential transfusion the median Hgb was 11.8 g/dL (range, 9 to 15.7) and the median HbS was 19% (range, 5% to 32%) at the time of surgery. All patients received extended antigen matched blood. Complications of preoperative transfusion were minor and included two febrile-/allergic reactions and one mild superficial catheter-induced phlebitis.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1993

Abdominal tumors in infancy and childhood.

Detection of an abdominal mass in a child is an alarming finding for both parent and practitioner. Diagnostic studies including ultrasonography, computed tomography, and magnetic resonance imaging provide an accurate diagnosis in most cases. The current treatment of Wilms' tumor results in an 85% to 90% survival. Hepatoblastoma and neuroblastoma often present in an advanced stage and require preoperative chemotherapy before resection is attempted. Improved survival for children with these tumors awaits better detection, chemotherapy, and understanding of the biology of their growth.

Abdominal Neoplasms↗
PubMed1993

Neuroectodermal differentiation of the gastrointestinal tumors in the Carney triad. An ultrastructural and immunohistochemical study.

A 16-year-old female with mediastinal paraganglioma and multicentric gastroduodenal "leiomyoblastomas" was thought to have an incomplete form of the Carney triad. The histologic, ultrastructural, and immunohistochemical findings of the gastroduodenal tumors revealed features of neuroectodermal differentiation. The architecture of the smallest duodenal tumors suggested an origin from the myenteric autonomic ganglia.

Adolescent↗
PubMed1993

Wilms tumor and nephroblastomatosis: imaging characteristics at gadolinium-enhanced MR imaging.

Twelve children with Wilms tumors were prospectively evaluated with 1.5-T magnetic resonance (MR) imaging. Spin-echo images were obtained before and after administration of gadopentetate dimeglumine. Nine of the 12 children had histologically proved nephroblastomatosis or nephrogenic rests involving a total of 14 kidneys. MR imaging depicted involvement in eight of these kidneys, including five of five kidneys with lesions contralateral to the primary Wilms tumor. Overall sensitivity of detection of nephrogenic rests was 43% on nonenhanced images and 58% when gadolinium-enhanced images were added. Nephrogenic rests admixed with Wilms tumors less than 4 mm in diameter were not depicted at MR imaging. On gadolinium-enhanced T1-weighted images, Wilms tumors and hyperplastic were hypointense to normal renal tissue. On T2-weighted images, Wilms tumors and hyperplastic nephrogenic rests were iso- or slightly hyperintense to renal cortex, while sclerotic nephrogenic rests were hypointense to renal cortex. On all images, including gadolinium-enhanced T1-weighted images, the signal intensity of Wilms tumors was inhomogeneous, while the signal intensity of nephrogenic rests was homogeneous.

Child↗