[Medical progress--exemplified by endocrine surgery].
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Biomedical subjects
Publications and source records attributed to R Roka.
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Graft-dependent recurrence was observed 24 and 27 months after total parathyroidectomy and immediate autotransplantation in 2 out of 35 haemodialized patients (6%) with reactive (renal) hyperparathyroidism. In order to normalize the altered parathyroid metabolism in these 2 patients 7 reductions of the graft have been required so far in the patients and cervico-mediastinal reexploration was necessary as an additional procedure in one of the patients. Histological examination of the more or less enlarged fragments showed nearly the same architecture as the original glands used for grafting. Proliferating chief cell nodules with mitoses and signs of expansive, but never invasive growth were seen. Reviewing the literature, 38 authors describe 61 graft-dependent recurrences in 783 patients (7.8%) since 1975. Our own experiences and those in literature are discussed with respect to diagnosis, differential diagnosis, localization and histology of the graft-dependent recurrence.
Serum calcium determination of 11,588 hospitalized patients during a twelve-month period revealed hypercalcaemia in 74 (0.64%). Further clinical and biochemical investigation established primary hyperparathyroidism in 20 of them (27%), and in 15 (20%) a malignant tumour (with bone metastases in six) as the cause of the hypercalcaemia. Rarer causes were found in 11 patients (15%): diuretic medication (5), lithium treatment (3), immobilization (2) or hyperthyroidism (1). In the remaining 28 patients (38%) no cause of the hypercalcaemia could be established with certainty. In at least six patients, however, there were clinical pointers towards hyperparathyroidism in the absence of unequivocal biochemical findings. Leaving out of account borderline cases, one must reckon on a prevalence of hyperparathyroidism in 0.17% of an unselected group of hospitalized patients. Parathyroid hyperfunction must always be considered in the differential diagnosis because of its manifold clinical presentation.
During total parathyroidectomy and autotransplantation 140 enlarged glands were removed in 35 hemodialyzed patients (normocalcemic: n = 14; hypercalcemic: n = 21). The cross-sections of all glands were classified intraoperatively. Diffuse hyperplastic (type 1) and nodular hyperplastic (type 2) glands could be distinguished. Using a stereo-magnifier (magnification: x 10 -x 16), type 1a- (stromal fat cells!) and type 1b- glands (without stromal fat cells!) could be differentiated. Those areas were also found between the nodules of type 2-glands. Significantly, nodular hyperplastic glands predominated in hypercalcemic patients (chi 2-Test: p less than 0.001). The colour of the nodules on the cross-sections of type 2-glands correlated with the predominating cell type ("dark": nodule of oxyphile cells; "medium": nodule of chief cells; "light": nodule of 'degenerating' oxyphile cells). As sign of proliferation the mitotic index was elevated (greater than 1:10,000) in type 1b-glands, in type 1b-like areas and in nodules of type 2-glands. These areas should not be used for autotransplantation.
The PTH secretion was studied in fragments of 83 (reactive) hyperplastic parathyroids (type 1 a: n = 24; type 1 b: n = 20, type 2: n = 39) simulating hypo- (0.3 mmol Ca++ = maximal PTH secretion), normo- (0.9 and 1.2 mmol Ca++) and hypercalcemia (3.0 mmol Ca++ = basic PTH secretion, maximal suppressibility) in vitro. 56 out of 83 glands (67%) were suppressible (PTH secretion decreased under 50% of maximal secretion) with no significant difference in suppressibility of diffuse and nodular hyperplasia (50% and 74%, respectively). Differentiating diffuse hyperplastic glands in those with (type 1 a) and without (type 1 b) stroma fat cells, 92% of type 1 a glands and only 25% of type 1 b glands were suppressible (Fischer's test: p less than 0.001). Separated (oxyphilic and/or chief cell) nodules, type 1 b- and type 1 a-like areas (both localized between nodules) of types 2 glands were suppressible in 18%, 17% and 91%, respectively (Fischer's test: p less than 0.001). Thus fragments of type 1 a regions should be autotransplanted for preference. Type 1 b regions or nodules should not be used for grafting.
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In order to investigate the relation between catecholamine output and haemodynamics, 13 patients aged between 16 and 62 years, who were operated for active pheochromocytoma, were investigated. Patients had general anesthesia employing muscle relaxants, artificial ventilation with nitrous oxide/oxygen and predominantly intravenous analgetics. Before surgery 9 patients had increased norepinephrine blood levels and only 6 patients increased epinephrine levels. Intraoperatively an increase in pulse rate and blood pressure was found, although alpha- and beta-blockers were employed. At this time there was a massively increased level of epinephrine and norepinephrine, both however decreasing at the conclusion of surgery going along with a normalisation of cardial parameters. Comparing norepinephrine to cardiovascular parameters, the best correlation could be found between norepinephrine and mean arterial pressure, followed by total catecholamines and mean arterial pressure. Heart rate showed a smaller correlation coefficient to norepinephrine, followed by norepinephrine and total peripheral resistance. It is concluded that invasive haemodynamic monitoring helps to differentiate between haemodynamic disturbances and to improve the cardiovascular management, although direct relations between blood catecholamine levels and circular vascular parameters could not be found.
Primary aldosteronism-characterized by hypertension, hypokalemia and metabolic alkalosis--is caused by benign adenomata or bilateral adrenal hyperplasia in most cases. Aldosterone producing carcinomata of the adrenal cortex are very rare tumors. As the histological classification is difficult, the diagnosis is often drawn from tumor size, very high levels of plasma aldosterone, severe hypokalemia and malignant behaviour. The prognosis is very poor: Overall median and 5 year survival rate from diagnosis of adrenocortical carcinoma are 14 months and 24%.
A 39-year-old patient with an epiglottic carcinoma stage T4N2M0 was treated initially by radiotherapy, but later underwent laryngectomy for irradiation necrosis. A pharyngeal fistula occurred in the irradiated area. After resection of the damaged skin reconstruction was performed by a double skin paddle myocutaneous pectoralis major flap. A recurrence of the fistula in the same area was closed by invagination of the flap. Three years later a massive scar stenosis developed in the reconstructed hypopharynx, so that the patient was unable to swallow. This required a total resection of the scar tissue which extended from the entrance of the hypopharynx to the upper oesophageal sphincter. The gap was bridged by a free microvascular jejunal graft. To date 6 months have elapsed since this operation and the patient is symptom free.
The long-term clinical results of parathyroidectomy for primary hyperparathyroidism were analyzed in 176 patients. Preoperatively 152 patients (86%) showed classic symptoms, 15 (9%) were classified as minimally symptomatic (only hypertension, diffuse osteopenia or manifestations of the hypercalcemic syndrome), and nine (5%) were asymptomatic. All patients were normocalcemic at follow-up, but renal, skeletal or gastrointestinal symptoms were eliminated in only 88-91%. Preoperatively impaired renal function and hypertension were seldom improved by parathyroidectomy. Deterioration of renal function and hypertension occurred only in the symptomatic and minimally symptomatic patients, in whom the course was varied and unpredictable. During observation periods up to 22 years, 7% of the patients died of acute/chronic renal failure or consequences of hypertension. Death from uremia was more common when there was also skeletal involvement. Acute pancreatitis could occur without preoperative symptoms, but other gastrointestinal disorders responded to normalization of parathyroid metabolism. Multiple bone lesions responded functionally and lacked prognostic significance. Hypercalcemic syndrome was rapidly and lastingly relieved by parathyroidectomy.
The long-term clinical courses of 212 "cured" (normocalcemic) patients were analyzed for 1 to 25 years (mean, 6.8 +/- 5.4 years). Preoperatively, 181 patients (85%) were classified as having typical symptoms, 22 patients (11%) as having minimal symptoms, and nine patients (4%) as having no symptoms of primary hyperparathyroidism (PHP). Although the formation of urinary calculi was stopped in 91% of patients, deteriorated renal function and hypertension were seen in patients with symptoms (14% and 8%, respectively) and patients with minimal symptoms of PHP (6% and 15%, respectively). Renal function changes and hypertension were unpredictable despite normalization of the hyperactive parathyroid metabolism and had decisive results: 7% of the patients died of uremia or of the consequences of hypertension (stroke). Large, multiple bone lesions healed functionally and were of no prognostic significance. In the majority of patients with symptoms of PHP, gastrointestinal manifestations healed postoperatively, but two patients who had no preoperative gastrointestinal complaints died of acute pancreatitis. Almost all symptoms of the hypercalcemia syndrome disappeared immediately and permanently in patients with symptoms and patients with minimal symptoms of PHP. Neither deterioration of renal function nor elevation of blood pressure were observed postoperatively in "cured" patients who showed no symptoms of PHP preoperatively. Even in these patients, immediate surgical treatment may have avoided the complications of chronic renal failure or hypertension. As soon as organic manifestations, even in mild form, have been established, it seems impossible to predict the course and to prevent an unfavorable clinical outcome.
Total parathyroidectomy, immediate heterotopic autotransplantation and cryopreservation of parathyroid tissue was introduced in the surgical treatment of drug-resistant renal hyperparathyroidism (rHPT) in 1980 and was performed in 36 patients. During 34 initial bilateral cervical explorations including transcervical thymectomy 142 glands were removed (orthotopic localization: 84%; heterotopic localization: 16%). Supernumerary glands were found in 6 patients (18%; thymus: 3; cervical fatty tissue: 4). The elevated parathyroid metabolism was successfully corrected, in all, but one patient in whom only three glands were removed. Biochemical and clinical follow-up examinations showed a restitution of the symptoms in 88% 3 to 79 months postoperatively. Graft-dependent recurrence of rHPT was diagnosed in one patient. Normocalcemia was reestablished by partially excising the grafted tissue under local anesthesia. Graft-dependent hypoparathyroidism was suspected in one patient because of low levels of serum calcium and parathyroid hormone. Late graft failure was not observed during the follow-up period. None of the patients required delayed autotransplantation of cryopreserved tissue.
Depending on their symptomatology 152 cured (i.e., normocalcemic) patients with surgically proven primary hyperparathyroidism (pHPT) showed typical symptoms preoperatively. Besides hypercalcemia and elevated parathyroid hormone levels, 15 patients suffered only from hypertension and/or diffuse osteoporosis and/or complaints caused by the hypercalcemic syndrome (oligosymptomatic patients). Nine patients had no complaints (asymptomatic patients). The long-term clinical course of all patients was analyzed up to 22 years. Although the formation of urinary calculi was stopped in 94% of cases, a deterioration of renal function and hypertension was seen in symptomatic (12.5% and 9.2%, respectively) and oligosymptomatic patients (6.7% and 13.3%, respectively). Renal function and hypertension were unpredictable despite normalization of the hyperactive parathyroid metabolism and were of decisive prognostic significance; 6% died of acute or chronic renal failure, or of the consequences of hypertension. Multiple bone lesions, even large, healed functionally and were of no prognostic significance. In the majority of symptomatic patients gastrointestinal manifestations held postoperatively, but two patients died of acute pancreatitis without gastrointestinal complaints preoperatively. Almost all symptoms of the hypercalcemic syndrome disappeared immediately and permanently in symptomatic and oligosymptomatic patients. No deterioration of renal function and no elevation of blood pressure was observed in cured asymptomatic patients postoperatively. Immediate surgical treatment even in asymptomatic patients may have avoided complications of chronic renal failure or of hypertension. As soon as organic manifestations, even in a mild form, have been established, it seems impossible to predict the course and to prevent an unfavorable clinical outcome.
We report 10 patients undergoing pharyngo-laryngectomy in whom the pharyngeal defect was reconstructed with a free jejunal graft. This method is technically demanding. The advantages are excellent healing due to the excellent blood supply of the edges of the graft, and the almost unlimited supply of jejunum. The use of this method is justified by the immediate restoration of deglutition.
A new method of surgical rehabilitation of the voice is being presented. A shunt is introduced between the trachea and the hypopharynx or the entrance to the oesophagus, by the use of a jejunal graft which is sutured isoperistaltically. By microsurgical anastomoses the jejunum is revascularized by the cervical vessels. Seven patients, having undergone total laryngectomy because of carcinoma of the larynx, have been treated by this procedure. In one case a venous thrombosis developed and the graft had to be resected. The other patients regained a voice, able to meet all requirements of daily life. The voice shows a remarkable low phonatory pressure, a good modulation capacity and a phonatic duration of up to 20 seconds.
A total of 45 patients have received surgical treatment for distant metastases in 41 follicular and four papillary carcinomas. Fifty-four metastatic lesions were removed. In the majority of cases (n = 25, 46%), surgical intervention was indicated on the basis of oncologic data (reduced administration of radioiodine). Sixteen patients (30%) underwent surgery to relieve pain, and 13 other patients (24%) had surgical treatment of pathologic fracture. At the time of surgery, 29 patients (64%) had only one resectable metastasis, while 16 patients (36%) had further nonresectable metastases (six in the bone, 10 in the bones and lungs). In the course of 53 operations, metastases were resected from bone in 46 cases, from the lungs and greater omentum in two cases, and from the skin, suprarenal gland, pleura, and intra-abdominal lymph node in one case each. A total of 25 metastases (17 bone, eight soft tissue) could be removed by resection. In 16 patients, the resulting bone defect was filled with bone cement after resection of the metastases. Osteosynthesis was necessary in another six cases, while seven required the implantation of an endoprosthesis. Thirty-eight patients died between 1 and 136 months after surgical treatment. Twenty-six (58%) died of their primary disease after an average 49.3 months, seven (15%) died with their carcinomas of other causes after an average of 12 months, and five (11%) died intercurrently after an average of 16 months. Seven patients (15%) are still alive after 12 to 264 months (average, 99.3 months); four of them are without recurrence and three have metastases. Five of these patients exhibit normal activity, while the activity of the other two is limited by the progress of the carcinoma or as a result of surgical treatment. The estimated cumulative survival rate (Kaplan-Meier) was 44.8 +/- 11.2% for 5 years and 32.7 +/- 11.0% for 10 years after removal of a solitary metastasis. Analysis of these patients shows that the surgical removal of resectable metastases can be a valuable complement to nuclear medical therapy. The complicated surgical treatment of metastases is justified by the favorable effect it has on prognosis and on the patient's quality of life.
The jejunal graft with microsurgical vascular anastomoses is now recognised as one of the most reliable methods for reconstructing large and various defects of the upper aero-digestive tract. After resection of T4-tumours and reconstruction of phonation, deglutition and aesthetics 50% of our patients could be resocialized within the first postoperative month.
After laryngopharyngectomy and reconstruction of the upper digestive tract, a free jejunal graft can be anastomosed in an isoperistaltic direction end-to-end to the trachea and the hypopharynx in order to form a shunt for vocal rehabilitation. Following placement of the shunt, no special care is required nor does any patient show any aspiration. Because of a low-flow resistance of the shunt, patients so treated possess long-lasting phonation with loud and modulated voices.