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Biomedical subjects

R L Telander

Publications and source records attributed to R L Telander.

At least 19 recordsLinked to original sources

Review of head and neck lesions in infancy and childhood.

Common congenital and infectious lesions of the head and neck in infancy and childhood have been discussed. Those that are present at birth, asymptomatic, and frequently cystic are clearly benign lesions that require operative management for the potential complications of enlargement and infection. Solid lesions, particularly those of the lymph nodes, must be differentiated from neoplasms, particularly Hodgkin's disease and non-Hodgkin's lymphoma. Suppurative lymphadenitis is obvious from its local inflammatory signs, but the subacute and chronic adenopathies may be difficult to distinguish from neoplasia. An attitude that unless the benign nature of a lesion is clearly demonstrated, early excisional biopsy for histopathologic evaluation must be the rule will avoid delays in diagnosing those neoplastic lesions that can be treated successfully if managed early and aggressively.

Branchioma

Giant epignathus. A case report.

We report the successful treatment of a 2.8 kg female infant born with a giant epignathus, and we present our current prenatal and neonatal recommendations for managing this problem. We recommend that the delivery be done by cesarean section, that an adjacent operating room be ready for the baby, and that a neonatologist, anesthesiologist, and pediatric surgeon be standing by. The term epignathus now commonly applies to a teratoma protruding from a newborn's mouth. The survival of these otherwise normal children has been very low. We discuss the perinatal, neonatal, and operative care necessary to improve survival, as evidenced by the case presented.

Biomarkers, Tumor

The surgical management of children with ulcerative colitis. The old vs. the new.

The authors' experience with children who have chronic ulcerative colitis was reviewed to compare their current surgical approach (ileoanal anastomosis) with earlier methods of management. Between 1960 and 1984, 137 children with chronic ulcerative colitis underwent surgery (mean duration of follow-up, 7.1 years). In 91 patients, the procedures were a total proctocolectomy with ileostomy or Kock pouch (66) or a lesser colectomy with either an ileostomy (16) or an ileorectal anastomosis (9) (group I). Forty-six patients underwent an ileoanal anastomosis procedure (group II). Children in group I were more likely to have significant preoperative loss of weight, a debilitated condition, and malnutrition. Urgent or emergency surgical intervention was required in 25 percent of patients in group I but in only 4 percent of patients in group II. Trends included 1) a younger age at operation in group II, 2) a higher mortality in group I (7.7 percent) than group II (0 percent), and 3) a higher perioperative mortality with emergency operations (23 percent) than elective procedures (1.6 percent). In group I, 98 percent of patients had an abdominal ostomy, but no patients in group II had an abdominal ostomy. The children with an ileoanal anastomosis had an average of 4.8 stools during waking hours and 1.3 stools each night. On the basis of this experience, the authors recommend use of the ileoanal anastomosis procedure in the surgical treatment of chronic ulcerative colitis in children.

Adolescent

Carcinoid tumor of the appendix in the first two decades of life.

Carcinoid tumor of the appendix is the most common neoplasm of the gastrointestinal tract in childhood and adolescence. Sufficient long-term follow-up data after surgical treatment are not currently available for patients diagnosed during the first two decades of life. From 1936 to 1988, 23 patients were observed at this institution with histologically confirmed carcinoid tumors involving the vermiform appendix. In contrast to the adult experience, in which the tumor is most commonly encountered as the result of an incidental appendectomy, 18 of these patients presented with signs and symptoms of an acute abdomen directing the surgeon to the appendix. In the other five cases, surgery was performed for other reasons. Median age at presentation was 13.0 years (range, 6 to 20 years). Fourteen patients were female, nine were male. Simple appendectomy was the initial procedure for all patients. Tumor size ranged from "microscopic" to 2.5 cm in largest dimension. Three patients subsequently underwent right hemicolectomy, and one patient had removal of a residual appendiceal stump, but no residual or metastatic tumor tissue was found in any of the resected specimens. Nineteen patients underwent simple appendectomy alone. Eighteen available specimens were reviewed at the time of this study for confirmation of histology and degree of invasion. The tumor invaded to the serosa in nine of 23 (39%). The mesoappendix or periappendiceal fat was involved in seven of 23 (30%). Vessel invasion was not noted in any specimen. Our median follow-up time was very long, being 26 years (range, 9 months to 51 years). No patient has had evidence of recurrent or metastatic disease.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent

Evaluation of children with multiple endocrine neoplasia type IIb following thyroidectomy.

Medullary thyroid carcinoma (MTC) develops in all patients with multiple endocrine neoplasia type IIb (MEN IIb), a rare syndrome that either occurs sporadically or is inherited in an autosomal dominant pattern. The MTC in patients with MEN IIb has been reported to be biologically aggressive with onset at a young age and rapid progression as evidenced by widespread metastases and death, frequently in the teenage years. Seven children, aged 2 to 11 years (mean, 7 years), from three kindreds with MEN IIb were evaluated for evidence of tumor recurrence 3 to 10 years following thyroidectomy. In one child, age 11, a thyroid mass was palpable preoperatively. However, in the remaining six children (aged 2 to 10 years), the diagnosis of MTC was established by an increased concentration of plasma calcitonin (CT), either basally or following pentagastrin (Pg) stimulation. All patients underwent total thyroidectomy with removal of central lymph nodes from the neck. At the time of surgery, six children were found to have bilateral macroscopic MTC, five without and one with cervical metastases. One child (age 2 years) had C-cell hyperplasia, a premalignant precursor of MTC. Currently, five of the seven children are without evidence of recurrent disease clinically and have normal plasma CT levels (less than 0.3 ng/mL) following calcium (Ca) and Pg stimulation 3, 3, 10, 10, and 10 years after thyroidectomy. Two of the seven children have biochemical evidence of residual MTC.(ABSTRACT TRUNCATED AT 250 WORDS)

Biomarkers, Tumor

Pediatric urological complications with intraoperative radiation therapy.

Intraoperative radiotherapy with variable energy electrons has been used as a supplemental boost to treat 6 children with locally advanced retroperitoneal malignancies. Of the patients 4 had treatment-related injuries to portions of the urinary tract within the intraoperative and external radiation fields. Three patients had significant renal impairment requiring surgical correction. The pathogenesis and management of treatment-induced injury to the urinary tract are discussed.

Adolescent

Long-term follow-up of the ileoanal anastomosis in children and young adults.

The purpose of this study was to carry out a long-term study of the ileoanal anastomosis (IAA) in children and young adults, comparing the straight IAA to the J pouch. One hundred twenty-one young people who had undergone IAA were studied, with 114 available for long-term follow-up. One hundred one were 18 years and under. Forty-nine patients had a straight IAA and 72 had a J-pouch reservoir. There were no deaths. After surgery, three children had intraabdominal sepsis and one had pelvic sepsis, but it did not lead to excision of the IAA. The mean stool frequency in all 114 patients was 5.0 +/- 2.5 per day and 1.2 +/- 1.1 at night. The mean number of stools for the straight IAA was 6 per day and 2.1 at night. The mean number of stools for the straight IAA with balloon dilations was 5.8 per day and 1.2 at night, and for the J pouch it was 4 per day and 1 at night. Patients with both the J pouch and straight IAA had good to excellent sensation, with patients with the J pouch always able to distinguish flatus from stool in 87% of patients and almost always in 13%. Daytime continence was very good in both groups. Moderate nighttime loss of stool occurred in 10 patients, 6 with a straight IAA and 4 with a J pouch. Ninety-five percent of the 114 patients were satisfied or very satisfied, with most children with a J pouch very satisfied. The J pouch remains the procedure of choice in young people.

Adolescent

Exercise cardiorespiratory function in adolescents with pectus excavatum. Observations before and after operation.

We studied 12 children with pectus excavatum (mean age, 13.8 years) using pulmonary function and exercise testing. Eight patients had surgical repair and were studied before and after repair. Four patients did not have repair and were tested twice. The degree of chest wall deformity in the two groups was similar. All patients reached 86% or more of their maximal predicted heart rate with exercise. Total lung capacity decreased 8% (p less than 0.01) after operation. In the nonoperated control group, total lung capacity was similar during both studies. The ratio of ventilation at maximal exercise to maximal voluntary ventilation was 68% +/- 12% before and 66% +/- 9% after operation in the operated group and 69% +/- 18% and 79% +/- 30% at two studies in the nonoperated group (nonsignificant changes). Maximal oxygen uptake was 36.1 +/- 4.4 ml/kg/min preoperatively and 38.1 +/- 8.1 postoperatively and 41.2 +/- 7.3 ml/kg/min and 43.0 +/- 6.9 on two studies in the nonoperated group (nonsignificant changes). Cardiac output and stroke volume increased appropriately with exercise both before and after operation. Operation had no physiologically significant effect on the cardiorespiratory response to exercise. Slight changes in cardiorespiratory function occurred in both operated and nonoperated subjects. These results emphasize the importance of studying patients before and after operation and of using a control group.

Adolescent

Medullary carcinoma in children. Results of early detection and surgery.

Early diagnosis and surgical treatment of medullary carcinoma of the thyroid (MCT) in children is essential to decrease the likelihood of metastatic spread. To determine the optimal timing of screening and surgery, we reviewed all children younger than 16 years old who were undergoing surgical treatment. From 1970 to 1988, 33 children 6 months old to 15.9 years old (mean, 9 years old) with MCT or C-cell hyperplasia or both underwent total thyroidectomy. Follow-up was 4 months to 15 years after surgery (mean, 9 years). Six of 31 patients presented with a neck mass. Basal immunoreactive calcitonin level was increased in 3 of 7 patients with C-cell hyperplasia alone, in 10 of 18 patients with nonmetastatic MCT, and in all 5 patients with metastatic MCT. At the time of surgery, 7 patients had C-cell hyperplasia, 19 patients had MCT without metastasis, and 7 patients had MCT with metastases. All children with metastasis were more than 12 years old. Medullary carcinoma of the thyroid was seen as early as age 6 months in a child with multiple endocrine neoplasia type IIb (MEN-IIb) and age 3 years in a child with MEN-IIa. At follow-up, 25 patients were free of disease and 8 had had recurrent disease. Two of the 8 patients with MEN-IIb had died. All 6 children with a neck mass at presentation had recurrent disease. Of 27 patients diagnosed by screening, 25 had no evident disease and 2 had persistent disease. Thus, in children with MEN-II syndromes, screening studies are highly effective in detecting MCT. All children with MEN-IIb should be studied shortly after birth, and those with MEN-IIa should be studied by 1 year old.

Adolescent

Clinical outcome in children with acute cerebellar encephalopathy and neuroblastoma.

Acute cerebellar encephalopathy (ACE)--ataxia often associated with opsoclonus, polymyoclonus, and irritability--may be associated with neuroblastoma and should be suspected in a child who presents with ACE. The survival in ten children with ACE associated with neuroblastoma was 100%. Most of the tumors were ganglioneuroblastomas. The abdomen was the most common location for the tumor in this study although it also can be found in the mediastinum. All patients with this syndrome had a localized tumor. With ACTH therapy, ACE may resolve early postoperatively, but it tends to recur in most patients, continuing for as long as 3 years after the initial operation. Treatment with ACTH or prednisone modifies and rapidly clears the symptoms during the acute episode of recurrent ACE as well as helps to resolve subsequent neurologic sequelae. Significant neurologic sequelae persisted in seven of ten patients and included deficits in cognition or intellect, hyperactivity, impulsivity, emotional lability, and mild motor deficits. Seizures were seen in one patient.

Abdominal Neoplasms

Distribution, quantitation, and origin of immunoreactive neuropeptide Y in the human gastrointestinal tract.

A radioimmunoassay for measurement of immunoreactive neuropeptide Y has been developed using antiserum from a rabbit (221) immunized with porcine neuropeptide Y. Antibody 221 has been characterized for both sensitivity and specificity. To determine the distribution of neuropeptide Y in the human gastrointestinal tract, fresh tissue specimens were separated by microdissection into the muscularis externa and the mucosa-submucosa. To examine the origin of neuropeptide Y in human colon, specimens of aganglionic and ganglionic colon were obtained from patients with Hirschsprung's disease. Immunoreactive neuropeptide Y in human gut was present in highest concentrations in the muscularis externa of the stomach and in lowest concentrations in the muscularis externa of the ileum and descending colon. Neuropeptide Y in the stomach was present in higher concentrations in the muscularis externa than in the mucosa-submucosa, but in the descending colon there were lower concentrations of neuropeptide Y in the muscularis externa than in the mucosa-submucosa. In Hirschsprung's disease, concentrations of neuropeptide Y were increased in aganglionic colon in both the muscularis externa and the mucosa-submucosa, compared to corresponding layers from proximal ganglionic colon. Extracts of the gastric muscularis externa and the colonic mucosa-submucosa were separated by C18 reverse-phase high-performance liquid chromatography. One major immunoreactive species was identified by radioimmunoassay which eluted in a position similar to synthetic human neuropeptide Y. These results demonstrated both regional and layer differences in concentrations of neuropeptide Y in human gut. Increased concentrations of neuropeptide Y in aganglionic colon from Hirschsprung's disease most likely result from enlargement of neuropeptide Y-containing extrinsic nerve fibers in both the mucosa-submucosa and the muscularis externa.

Animals

Localization of pancreatic insulinoma: comparison of pre- and intraoperative US with CT and angiography.

Methods of preoperative radiologic localization of insulinoma were compared in 52 patients, 44 of whom had solitary tumors. Examinations performed in these 44 patients were preoperative ultrasonography (US) in 28, angiography in 26, and computed tomography in 23. Prospective sensitivities were 61%, 54%, and 30%, respectively. Imaging sensitivities were lower for the eight patients with multiple insulinomas. In 28 of the 44 patients, intraoperative US was performed without the examiner being aware of the surgical findings. The sensitivity was 84%. Four insulinomas were not palpable but were visualized sonographically. The combined sensitivity of intraoperative US and surgical palpation for detecting solitary insulinomas was 100%. High-frequency intraoperative US is valuable for detecting occult solitary insulinomas and considerably useful for determining the proximity of insulinomas to the pancreatic and bile ducts.

Adenoma, Islet Cell

Results of early thyroidectomy for medullary thyroid carcinoma in children with multiple endocrine neoplasia type 2.

Children with multiple endocrine neoplasia type 2 (MEN2) often develop medullary carcinoma of the thyroid (MCT) or its precursor, C-cell hyperplasia. Survival results are improved if malignancy is diagnosed early from the results of plasma immunoreactive calcitonin (iCT) measurement. The effect of early detection and thyroidectomy in children with MEN2 syndrome was determined by reviewing the experience between 1975 and 1985. Seventeen children with MEN2 who were 12 years old or younger underwent a total thyroidectomy for MCT or C-cell hyperplasia. iCT was measured in all patients preoperatively and postoperatively. Of the 17 children, 14 (82%) had MEN2a and 3 (18%) had MEN2b. There were 14 (82%) female and three (18%) male patients; their mean age was 6.97 years (range 1.5 to 12 years). In all patients, the diagnosis of MCT was made from initial elevated levels of iCT after stimulation with pentagastrin. Three patients had clinical evidence of disease preoperatively. All patients underwent a total thyroidectomy and lymph nodes were removed from the central zone; a neck dissection was performed in the three with clinically obvious disease. MCT with C-cell hyperplasia was found in 11 children and C-cell hyperplasia alone in six. Of the 11 with carcinoma, eight had bilateral disease and three unilateral. Six children had bilateral C-cell hyperplasia. All 17 children were alive and feeling well at the time of this report; however, three had evidence of metastatic disease according to iCT measurements. None of the children had recurrent nerve injuries; one had evidence of hypoparathyroidism.(ABSTRACT TRUNCATED AT 250 WORDS)

Calcitonin

Intraoperative ultrasonography of the pancreas in children.

In children, lesions of the pancreas often are small, and precise localization is required for optimal surgical management. We have used newer-generation real-time ultrasonography of the pancreas intraoperatively in seven children. Five of these children had hypoglycemia, hyperinsulinemia, and insulinomas; one had a persistent small pancreatic pseudocyst with a disrupted secondary duct, and one had familial pancreatitis with a remarkably enlarged duct and a stone. Two of the five with adenomas had multiple endocrine neoplasia syndrome I (MEN I syndrome); in them, ultrasonography localized several adenomas preoperatively and several additional adenomas intraoperatively. This allowed a 90% pancreatectomy with enucleation of small adenomas in the remaining head. One child had a nonpalpable insulinoma deep in the head of the pancreas; intraoperative ultrasonography localized the lesion and permitted successful enucleation. Another child with a small pseudocyst and a disrupted secondary pancreatic duct ultimately required surgical drainage; intraoperative ultrasonography of the inflammatory mass immediately localized the small pseudocyst and thus decreased the operative time. The child with familial pancreatitis appeared to require a surgical drainage procedure; however, intraoperative ultrasonography demonstrated that the stone had passed spontaneously just prior to operation and the duct size had returned to normal, eliminating the need for the drainage procedure at that time.

Adenoma, Islet Cell

Endocrine disorders of the pancreas and adrenal cortex in pediatric patients.

Hyperinsulinemia, a common cause of persistent hypoglycemia in infants and children, can result in permanent damage to the central nervous system. Thus, early diagnosis and treatment are important. The typical clinical manifestation of hyperinsulinemic hypoglycemia is symptomatic hypoglycemia that responds poorly to medical therapy. Affected infants may have tremors, jitteriness, apnea, cyanosis, or seizures. If initial medical therapy (frequent feedings, large amounts of intravenously administered glucose, diazoxide, and glucocorticoids) fails to stabilize plasma glucose levels, subtotal pancreatectomy is indicated. This surgical intervention does not completely correct the hypoglycemia in all patients, but it effectively reduces the severity of the condition. Surgical treatment of pediatric patients with Cushing's syndrome or aldosteronoma has also been effective. In our experience, survival of patients with adrenocortical adenomas has been 100%, whereas only five of nine children with adrenocortical carcinomas survived, and four of the five were younger than 10 years of age.

Adrenal Cortex Neoplasms

Benign pancreatic insulinoma: preoperative and intraoperative sonographic localization.

Twenty-nine patients with surgically proved benign pancreatic insulinoma were studied by preoperative or intraoperative sonography. Twenty-five patients had solitary pancreatic tumors; four had multiple tumors. Six of the patients with solitary insulinomas and one of the patients with multiple insulinomas had undergone previous unsuccessful exploration. Preoperative sonography was performed in 24 patients with solitary insulinomas, and 15 (63%) were localized. Intraoperative sonography was performed in 22 patients with solitary insulinomas, and 19 (86%) were visualized without having been previously located by palpation. Four of these visible solitary tumors (18%) were not detected by palpation at surgery. All the solitary insulinomas were detected with the combination of palpation and intraoperative sonography. In each of the six patients with solitary insulinoma who had undergone previous surgery, the tumor was visible with intraoperative sonography, which also demonstrated nonpalpable insulinomas in two of the four patients with multiple tumors. Preoperative real-time sonography is a sensitive, noninvasive, inexpensive method for localization of insulinoma. Intraoperative high-frequency sonography is a highly sensitive method for the detection of insulinoma. Intraoperative sonography is also valuable to determine the relationship of the insulinoma to pancreatic and bile ducts and thereby facilitate safe enucleation.

Adenoma, Islet Cell