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Biomedical subjects

R L Telander

Publications and source records attributed to R L Telander.

At least 55 records · Page 3Linked to original sources

Pheochromocytoma in the pediatric age group: current status.

Pheochromocytoma is an unusual tumor in the pediatric age group. Several aspects serve to differentiate children with pheochromocytoma from their adult counterparts. Children have fewer malignant tumors, more extraadrenal tumors, and greater bilaterality and multiplicity of tumor. Similarly, they have an increased incidence of multiple endocrine neoplasia (MEN) and familial disease. The records of 16 children (age 17 years and younger) with pheochromocytoma were evaluated. One newborn died at 36 hours of age, and a 3-year-old died four days after exploratory laparotomy. Of the 14 survivors (with an average follow-up of 8 years), three had manifestations of multiple endocrine neoplasia type 2 and two had familial pheochromocytoma only. One patient had malignant paragangliomas. Three patients had bilateral tumors; one of these had MEN. Four patients underwent operation before the use of alpha- and beta-adrenergic blockade was routine at our institution. An additional patient received blockade in preparation for the removal of the pheochromocytoma but was not prepared for preoperative angiography. Hypertensive complications were encountered in four of these five children; in one, this complication was fatal. There were no intraoperative complications in the 12 patients who had received appropriate blocking agents. Eleven of the 14 survivors are currently normotensive without the need for antihypertensive medication. Two patients have medically controlled hypertension, and one continues to have severe hypertension. An overview of this series underscores the characteristic features of pheochromocytoma in the pediatric age group. Angiography has been replaced by high-resolution computed tomography for localization.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

Surgical implications of Klippel-Trenaunay syndrome.

Between January 1956 and July 1981, 40 patients with Klippel-Trenaunay syndrome were seen at the Mayo Clinic. Twenty male and 20 female patients presented with the classic triad of soft tissue and bony hypertrophy of the extremity, hemangioma, and varicosity without evidence of functional arteriovenous fistulae. The lower extremity was involved in 38 patients (95%), the upper extremity in six patients (15%). In four of these cases (10%), both the upper and lower extremities were affected. The disease was unilateral in 34 patients (85%), bilateral in five cases (12.5%), and crossed-bilateral in one case (2.5%). Surgery was done in 13 patients (32.5%), nine of whom were operated on at the Mayo Clinic and four of whom had had previous surgery elsewhere. Excision and stripping of varices were performed in three patients; of these three, a deterioration of symptoms was observed in one, but the procedure was beneficial in the other two. Partial varicectomy was performed in four cases, while resection of the angioma was attempted in eight cases, with good results in three cases of small angiomas. Femoral and tibial epiphysiodesis stopped the overgrowth and produced an excellent result in one case. In four cases of epiphysiodesis at the foot level and two cases of derotational tibial osteotomy, moderate improvement was achieved. The indication for vascular or orthopedic surgery should be carefully considered in each patient who has this syndrome. One patient (2.5%) died of a severe form of the disease, but the relatively benign course is documented by the 21 patients (52.5%) who are free of complaints without any treatment or with elastic support only.

Adolescent↗

Treatment of defects of the anterior abdominal wall in newborns.

Primary closure of an omphalocele or gastroschisis may cause respiratory compromise in the neonate. Some authors recommend primary closure of the defect with prolonged respiratory support because of dissatisfaction with staged visceral reduction and use of a Silastic pouch. Our experience with use of a Silastic pouch from 1975 to 1982 was reviewed. Twenty-three newborns with major defects of the abdominal wall (14 omphaloceles and 9 gastroschisis anomalies) were surgically treated, and only one death occurred. The mean birth weight of the infants was 2,927 g; nine of them were premature. Seven infants had major associated anomalies. The goal of the surgical procedure was closure of the abdominal wall without compromise of the cardiorespiratory status. During the operation, muscle relaxants were avoided and the infants breathed spontaneously. If progressive visceral reduction caused tachypnea (rate of more than 70/min) or hemodynamic instability, a Silastic pouch was constructed. Ten patients were treated with primary fascial closure, and 13 were treated with a Silastic prosthesis. The neonates with the prostheses required three to eight reductions, and the prostheses were in place for 4 to 22 days. No patient had wound dehiscence, wound infection, or an intestinal fistula. The one death occurred in an infant with trisomy 18 syndrome and multiple anomalies. Thus, the Silastic pouch was effective when the defect could not be closed primarily without respiratory compromise.

Critical Care↗

Primary mediastinal tumors in children.

Mediastinal masses in children constitute a heterogeneous group of malignant and benign neoplasms. The majority of malignant tumors in our series of 188 children with a mediastinal mass were Hodgkin's and non-Hodgkin's lymphoma (87 patients) arising in the anterior and middle mediastinum. Ganglioneuroma (17) arising in the posterior mediastinum made up the bulk of benign tumors (52). Infants less than 2 yr old presented with symptoms of tracheal compression, whereas older children had fewer symptoms but a far greater likelihood of having a malignancy. In the assessment of patients with mediastinal tumors, the posteroanterior and lateral chest roentgenograms were most often diagnostic, and computed tomography of the chest provided the most information concerning preoperative resectability. The excellent survival of patients with stage I and II Hodgkin's disease reflects the radiosensitivity of the tumor; in our series of 33 patients, survival was not increased by radical resection. Unlike lymphomatous tumors, neurogenic masses should be completely excised. When initial exploration reveals unresectability, biopsy followed by radiation therapy and second-look excision of the tumor can be accomplished. In patients with primary mediastinal sarcomas, total resection should be carried out if possible. In our series, adjuvant radiation therapy or chemotherapy was effective in only 1 of 15 children with sarcoma. Surgical treatment of all mediastinal masses except lymphoma should be complete excision. In cases of suspected lymphoma, cervical or supraclavicular node biopsy can yield adequate tissue for diagnosis.

Adolescent↗

Physiologic aspects of continence after colectomy, mucosal proctectomy, and endorectal ileo-anal anastomosis.

We examined the physiology of continence in 12 patients at least four months after colectomy, mucosal proctectomy, and endorectal ileo-anal anastomosis for ulcerative colitis and familial polyposis. The mean fecal output (+/-SEM) was 598 +/- 60 gm, passed as 12 +/- 4 movements/24 hr, of which 4 +/- 1 were passed at night. The patients were generally continent during the day and could distinguish gas from stool, but 11 of 12 leaked stools at night. Anal sphincter resting pressures (71 +/- 8 cm H2O) and squeeze pressures (171 +/- 15 cm H2O) of patients were similar to those of ten healthy controls (P greater than 0.05), although the rectal inhibitory reflex was absent in the patients. After operation, the distal bowel had a pressure-volume curve of greater slope (0.15 +/- 0.05 ml/cm H2O) than it had in controls (0.07 +/- 0.01 ml/cm H2O, P less than 0.05) and a lesser maximum capacity (patients, 248 +/- 31 ml; controls, 406 +/- 26 ml; P less than 0.05). The greater the capacity of the neorectum, the fewer was the number of bowel movements/day (r = 0.91, P less than 0.001). We concluded that the operation preserved the anal sphincter, although it decreased the capacity and compliance of the distal bowel and impaired continence.

Adult↗

Endorectal ileoanal anastomosis.

In 1933, Nissen performed the first ileoanal anastomosis. Since then, ileoanal anastomosis has enjoyed only periodic popularity because numerous postoperative problems, primarily as a result of infection and incontinence, plagued the procedure. In recent reports, however, better postoperative results have been detailed which stem not only from improved surgical technique and better selection of patients but also from increased understanding of the physiologic mechanisms by which fecal continence is achieved. At operation, meticulous hemostasis and asepsis, accurate en bloc mucosal dissection and effective drainage each contribute to good functional results and the minimization of complications. In addition, patients who manifest evidence of dysfunction of anal sphincters, who are elderly or obese or who have Crohn's disease should not be candidates for ileoanal anastomosis. Finally, if the anal sphincters are intact, reliable ileal reservoir function, achieved by passive dilation, balloon distention or surgical construction of a pouch, may be the most important determinate of excellent clinical results after ileoanal anastomosis.

Adult↗

Colectomy with rectal mucosectomy and ileoanal anastomosis in young patients. Its use for ulcerative colitis and familial polyposis.

Twenty-five children and young adults underwent colectomy with rectal mucosectomy and ileoanal anastomosis for chronic ulcerative colitis or familial polyposis. Follow-up ranged from three to 35 months. A simpler and shorter modification of the operation was used in 12 patients. Balloon catheter dilations were performed in nine patients before closure of the ileostomy to begin enlargement of the neorectum; 24 patients underwent closure of their temporary ileostomy. All patients are alive and well and participate full time in school or work. Two patients had mechanical bowel obstruction; on critical retrospective review, one patient was found to have Crohn's disease that necessitated removal of the rectal cuff. The clinical result was considered excellent in 11 patients, good in seven, fair in three, and poor in three. A gradual decrease in frequency of stooling was observed as the neorectum enlarged during the first year postoperatively. All patients had very good anal sphincter tone with voluntary rectal continence. Although some soiling and leakage occurred, especially during sleeping, these complications decreased with time.

Adolescent↗

Early development of the neorectum by balloon dilations after ileoanal anastomosis.

Recently, young people with chronic ulcerative colitis and familial polyposis have been undergoing colectomy with rectal mucosectomy and ileoanal anastomosis with encouraging clinical results. However, during the early period after closing the temporary ileostomy, some patients have frequent stools, which decrease with time as the terminal ileum dilates and becomes a reservoir. To enhance the early development of the neorectal reservoir and to minimize the frequent stooling, we instituted balloon dilations of the neorectum before ileostomy closure. A group of 16 patients not undergoing balloon dilations were compared with a similar group of 13 patients who had balloon dilations. Patients undergoing balloon dilations were observed to have an increase of 40.6% per month in the measured volume of the neorectum, along with a decrease of approximately 40% in the mean number of stools at 3 mo and 34% at 6 mo, with a very acceptable clinical result.

Adolescent↗

Aggressive thoracotomy for pulmonary metastatic osteogenic sarcoma in children and young adolescents.

During a 6-yr period, 57 children and young adults less than 21 yr of age underwent 111 thoracotomies for pulmonary metastatic osteogenic sarcoma. Follow-up after the initial thoracotomy averaged 36.8 no and ranged from 1 to 78 mo. Twenty-eight patients (49%) underwent more than one thoracotomy--the number of multiple thoracotomies averaged 2.9 and ranged from two to eight. Twenty-six of the 57 patients are currently alive, and 25 of these are free of disease. Median survival was 36 mo. Actuarial survival curves demonstrated a 5-yr survival of approximately 40%. Seventy-one percent of patients who had a tumor-free interval of greater than 1 yr are currently alive, compared with 39% of patients who had a tumor-free interval of less than 1 yr. Patients with disease confined to one lobe at the initial thoracotomy had a better survival (64%) than patients with diffuse or bilateral disease (41%). An aggressive surgical approach toward osteogenic sarcoma with pulmonary metastasis thus appears to be justified.

Actuarial Analysis↗

Surgical treatment of ulcerative colitis in children.

Our finding in 100 patients indicate that elective surgical treatment of ulcerative colitis in children has a low mortality rate in contrast to emergency surgery (2.3% versus 23%). Morbidity associated with these procedures is moderate and appears to be decreasing (13%), as shown by the comparison between the experience in the 1960s and that in the 1970s. Results with the newer surgical procedures have been encouraging; 60% of patients with Brooke ileostomy have been very satisfied as compared with 90% of patients with Kock pouch and of those with rectal mucosectomy with ileoanal anastomosis. Ileorectostomy was not a satisfactory procedure in this series. Additionally, after surgical treatment, the median height percentile increased from the thirty-ninth to fifty-seventh percentile. Finally, and important, 96% of the patients in this series reported that their general health at follow-up was good to excellent and that they had minimal limitation in their activities.

Adolescent↗

Limited thoracotomy in the pediatric patient.

Thirty-one children underwent 33 limited thoracotomies between 1973 and 1979. Adequate tissue for diagnosis was obtained in each case, and subsequent therapy was influenced in 91%. The preoperative diagnosis was confirmed in 61% and corrected in 36%. One death may have been hastened by the procedure, but all others were related to the underlying disease process. There were very few complications, the most common being pneumothorax. The routine use of a chest tube postoperatively seems to reduce the frequency of pneumothoraces. The average duration of the operation was 50 minutes. We believe that limited thoracotomy is a safe and rapid method of obtaining lung tissue and almost always ensures adequate tissue for diagnosis.

Adolescent↗

Management of stages I to III Hodgkin disease in children.

Thirty-seven children, ages 4 through 16 years, presented with clinical stages I, II, or III Hodgkin disease. In nine (24%) patients, laparotomy and splenectomy resulted in a pathologic stage that varied from the clinical stage. Of 36 patients with pathologic stages I, II, and III, 26 have been followed for more than two years from diagnosis. Pathologic stages I and IIA disease were found in 21 patients, and 19 received radiation therapy alone (usually mantle-field), with 90% disease-free survival and 95% overall survival (median follow-up 46 months). Five patients had stage IIB disease; two had progression of disease while received combined modality therapy. Of ten patients with stage III disease, five have had relapses and five have remained in complete remission. All relapses occurred in patients receiving either irradiation or chemotherapy but not both. This experience supports extended-field irradiation alone as adequate treatment for stages 1 and IIA Hodgkin disease in children, but suggests that for stages IIB and III, single modality treatment is not adequate.

Adolescent↗

Extra-adrenal intrathoracic functioning paraganglioma (pheochromocytoma) in childhood.

Extra-adrenal pheochromocytomas are more common in children (30%) than in adults (10%). Of the extra-adrenal sites, the intrathoracic site is the most rare. A 15-year-old boy had a pheochromocytoma successfully removed from the left paraspinal region of his chest. Four years before removal, he had radiation therapy of 3,500 rads to the tumor because of its apparent nonresectability. After therapy, there was transient clinical and biochemical improvement. Preoperative angiography and computed tomography helped define the anatomy of the tumor vessels and the relationship to the thoracic aorta of the tumor. These studies also aided in discounting any other site for the pheochromocytoma, either adrenal or extramedullary. A slow-growing pulmonary metastatic lesion was subsequently identified and successfully excised.

Adrenal Gland Neoplasms↗