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[Multiple paraganglioma: careful with surgery!].

INTRODUCTION: Extraadrenal paragangliomas are exceptional tumors. They prevail the carotid, jugulotympanic, and vagal ones. They are frequently multiple and its treatment is controversial in view of the fact that bilateral removal can go with severe morbidity. The case of a patient with bilateral paraganglioma and postoperative baroreflex dysfunction with severe arterial hypertension and hypotension episodes is presented. CLINICAL OBSERVATION: A 23-year-old woman with a diagnosis of left carotid and right vagal paraganglioma by TC and angiography. In January 1999 the left carotid paraganglioma was operated. The patient showed dysphonia, dysphagia, and lingual dysmotility in the postoperative course, with spontaneous improvement after some months. In December 1999, after the removal of the right vagal paraganglioma, the same complications appeared and hypertension crises (230/140), associated with headache, dizziness, and rash, and alternating with severe hypotension episodes (70/50). Blood biochemistry and the levels of cortisol, thyroid hormones, catecholamines, and metabolites were normal. Imaging techniques discarded tumor at another level and the registry of the ambulatory monitoring of blood pressure (AMBP) confirmed an important pressure lability. The neurophysiological study of the autonomous nervous system demonstrated the failure of the fast regulation mechanisms of the blood pressure. With the diagnosis of baroreceptors dysfunction and paralyses of cranial nerves IX, X and XII a treatment with clonidine was started with poor tolerability and incomplete response. DISCUSSION: This case illustrates the treatment difficulties of paragangliomas, especially when they are bilateral, and in which the surgery can go with severe morbidity. Baroreflex dysfunction should be entertained in the differential diagnosis of the extreme pressure lability.

Adult↗

Effect of diabetes mellitus on sympathetic neuronal regeneration studied in the model of transplant reinnervation.

UNLABELLED: The transplanted heart is initially denervated but undergoes subsequent sympathetic reinnervation. It thus provides a unique model for studying regeneration as a specific component of autonomic nerve biology. The aim of this study was to determine the effect of diabetes mellitus on the regenerational capacity of sympathetic neurons using molecule-targeted PET. METHODS: Twenty-two nonrejecting, otherwise healthy cardiac transplant recipients underwent PET with the (11)C-labeled physiologic neurotransmitter epinephrine at 4.0 +/- 3.3 y after surgery. Sympathetic reinnervation was defined as regional restoration of epinephrine retention to values within normal limits. RESULTS: Reinnervation was observed in 8 of 12 patients with no evidence of diabetes and in 6 of 10 patients with a long-term history of diabetes mellitus. The regional extent of reinnervation (4.7% +/- 5.3% of left ventricle vs. 19.1% +/- 20.6% for nondiabetic recipients, P = 0.04) and the regeneration rate (0.8% +/- 1.0% of left ventricle per year vs. 8.0% +/- 10.1% for nondiabetic recipients, P = 0.04) were significantly reduced in diabetic subjects. In a multivariate model, diabetes mellitus was an independent determinant of allograft reinnervation. Finally, the reappearance of innervation was found to correlate with an improved chronotropic and inotropic response to stress in a standardized, symptom-limited exercise test including radionuclide angiography. CONCLUSION: The regenerational capacity of the sympathetic nervous system of the heart is reduced, but not abolished, by diabetes mellitus. This study on cardiac transplant recipients further supports a general link between impaired glucose handling and cardiac autonomic nerve function.

Catecholamines↗

Clinical applications of pupillography.

The development of personal computer-based infrared video instruments has allowed pupillography to enter the clinical arena. Measuring pupil diameter for refractive surgery, distinguishing Horner syndrome from physiologic anisocoria, quantifying the relative afferent pupillary defect, and plotting visual fields by means of graded pupil constriction to focal light stimuli are recent applications in ophthalmology. Pupillography has also been used to determine sleepiness and autonomic effects of new pharmaceuticals.

Diagnosis, Computer-Assisted↗

Lateral ligament: its anatomy and clinical importance.

Since Miles proposed abdominoperineal excision as a radical surgery for rectal cancer in 1908, surgeons have recognized the lateral ligament in the pararectal space of their patients and attached clinical importance to it, although anatomists did not describe any such configuration in cadavers. By analyzing an experience of 421 lower rectal cancer cases at the Cancer Institute Hospital in Tokyo, discussion of the lateral ligament was focused on its relationship to the fascial arrangements in the pelvis, the pelvic autonomic nervous system, and the lymphatic drainage of the rectum. The lateral ligament is not an anatomical term, but a clinical or surgical one. It exists in a living pelvis as a condensation of connective tissue around the middle rectal artery and is divided into two segments by the inferior hypogastric nerve plexus inside it and the visceral endopelvic fascia around it. The lateral ligament is a pathway of blood vessels and nerve fibers toward the rectum and lymphatic vessels from the lower rectum toward the iliac lymph nodes. Therefore, the lateral ligament plays a critical role in surgery for lower rectal cancer in two respects: the anatomic extent of resection for curing rectal cancer, and the preservation of sexual function.

Collateral Ligaments↗

Influence of the autonomic nervous system on calcium homeostasis in the rat.

The local surgical manipulation of sympathetic and parasympathetic nerves innervating the thyroid-parathyroid territory was employed to search for the existence of a peripheral neuroendocrine link controlling parathyroid hormone (PTH) and calcitonin (CT) release. From 8 to 24 h after superior cervical ganglionectomy (SCGx), at the time of wallerian degeneration of thyroid-parathyroid sympathetic nerve terminals, an alpha-adrenergic inhibition, together with a minor beta-adrenergic stimulation, of hypercalcemia-induced CT release, and an alpha-adrenoceptor inhibition of hypocalcemia-induced PTH release were found. In chronically SCGx rats PTH response to EDTA was slower, and after CaCl2 injection, serum calcium attained higher levels in face of normal CT levels. SCGx blocked the PTH increase found in sham-operated rats stressed by a subcutaneous injection of turpentine oil, but did not affect the greater response to EDTA. The higher hypocalcemia seen after turpentine oil was no longer observed in SCGx rats. The effects of turpentine oil stress on calcium and CT responses to a bolus injection of CaCl2 persisted in rats subjected to SCGx 14 days earlier. Interruption of thyroid-parathyroid parasympathetic input conveyed by the thyroid nerves (TN) and the inferior laryngeal nerves (ILN) caused a fall in total serum calcium, an increase of PTH levels and a decrease of CT levels, when measured 10 days after surgery. Greater responses of serum CT and PTH were detected in TN-sectioned, and in TN- or ILN-sectioned rats, respectively. Physiological concentrations of CT decreased, and those of PTH increased, in vitro cholinergic activity in rat SCG, measured as specific choline uptake, and acetylcholine synthesis and release. The results indicate that cervical autonomic nerves constitute a pathway through which the brain modulates calcium homeostasis.

Animals↗

Pseudoobstruction of the colon.

Pseudoobstruction of the colon is a specific variety of adynamic ileus. Its characteristic clinical presentation is severe cramping lower abdominal pain, a massively distended abdomen, and a characteristic x-ray picture. Thirty-five patients with this disease complex have been reviewed, and their surgical and medical therapy is discussed. Guidelines for continued medical versus surgical intervention are suggested. Two patients underwent decompression with the colonoscope. Its use and a possible hazard of the procedure are discussed. For those patients who develop pseudoobstruction after trauma or surgery, a pathophysiologic explanation is offered. The possible role of prostaglandin abnormality in the genesis of pseudoobstruction is also discussed.

Adult↗

Difficulty in differentiating thyrotropin secreting pituitary microadenoma from pituitary-selective thyroid hormone resistance accompanied by pituitary incidentaloma.

A 33-year-old woman with inappropriate secretion of TSH and a 2-mm pituitary microadenoma is described. She had a high serum free T4 concentration (31 pmol/L) with an inappropriately nonsuppressible serum TSH concentration (0.93 mU/L). The alpha/TSH molar ratio was 2.3 and magnetic resonance imaging with gadolinium enhancement identified an area of low signal intensity in the left lateral pituitary gland. However, TSH secretion was not completely autonomous. There was a significant response to exogenous TRH stimulation and suppression by T3 administration. Therefore, it was difficult to rule out a nonfunctioning pituitary adenoma with concomitant pituitary selective thyroid hormone resistance syndrome. A 2-mm microadenoma was excised via transsphenoidal surgery. The tumor cells were immunoreactive to antisera to alpha-subunit and minimally immunoreactive to antisera to TSHbeta. The patient's thyroid function normalized after surgery without medication. Because the adenoma could become large and intractable if the patient was treated inadequately, early diagnosis and treatment are important in patients with TSH secreting adenomas.

Adenoma↗

Alcohol withdrawal in the surgical patient: prevention and treatment.

In the literature on AWS, there is repeated emphasis on performing a thorough preanesthesia assessment in patients with suspected chronic alcohol use. Because these patients are difficult to diagnose and to treat in surgical settings if complications arise, a multimodal approach is highly recommended (86). Ideally, AWS should be prevented by adequate prophylaxis. If AWS develops after surgery or trauma, immediate therapy is required. The symptoms of AWS can be controlled using the combination of a benzodiazepine (in Europe, also chlormethiazole) with haloperidol or clonidine. The drug regimens must be individualized and symptom-oriented to treat hallucinations and autonomic signs. Dosages are generally larger than those in detoxification units. Other approaches to modulate the neuroendocrine-immune axis in patients with an increased risk of postoperative infectious complications look promising but await controlled trials.

Alcohol-Related Disorders↗

[Clinical and pathogenetic features of neurotrophic corneal disorders in diabetes].

The corneal neurotrophic status was evaluated in 110 patients (220 eyes) with diabetes mellitus and in 20 normal controls (40 eyes). Corneal abnormalities (gerontoxon, limb vascularization, punctate keratopathy, endothelial dystrophy, relapsing erosion, corneal ulcers) were detected in 73.6% diabetics. Corneal changes depended on the type, duration, and compensation of diabetes mellitus. Based on the diagnostic markers (threshold sensitivity of the cornea, microcirculatory status, bulbar conjunctiva status, level of lacrimal production, cellular composition of the limb smears impressions, lacrimal levels of catecholamines, hydroperoxides, and lipids), three types of diabetic neuropathy of the cornea were distinguished: vegetative, sensory, and vegetosensory. Corneal changes were associated with signs of diabetic neuropathy in 55.6% cases and with autonomic nervous disorders of the cardiovascular system in 46.9% cases, which evidences a generalized involvement. The detected neurotrophic features of the cornea should be borne in mind when choosing pathogenetic therapy for diabetics with corneal complications and preparing the patients to ocular surgery.

Adult↗

[The external pudendal veins. An anatomo-clinical study, their treatment by ambulatory phlebectomy (Muller method)].

Forty-one cases of E.P. varices (bilateral in 3 cases) were identified after examining 1371 patient histories followed up between 1985 and 1989 inclusive, i.e., 2.99 percent. Analysis of the history, clinical signs and Doppler and arguments based on the practice of ambulatory phlebectomy of the thighs made it possible to establish an accurate anatomical description of these somewhat poorly understood venous territories: simple or complex origin, principal or secondary saphenous ending and non-saphenous endings, supramelic, para-public, labial and perineal connections, extensions into other territories, defining major EP varicoses. Physical-pathological investigation can establish the frequently autonomous nature of these EP varicoses. The high incidence of primary reflux, their wide distribution along the varicose axes prolonged over 1 to 3 segments of the limbs, characterize the 37 cases of major EP varicosis. Therapeutic analysis reveals: the inadequacy of surgery and sclerosis in severe cases, the efficacy of ambulatory phlebectomy (A.P.) AP presents specific characteristics of method and bandaging related to the quality of the skin and venous tissue and the EP site. Thirty APs were carried out with very good anatomical, functional and aesthetic results.

Adult↗

Post-traumatic reflex sympathetic dystrophy.

This report presents a brief review and an illustrative case summary of post-traumatic reflex sympathetic dystrophy. The manuscript is concerned with reviewing updated methods of properly diagnosing and treating such a complex condition. The case presented involves a young, healthy male patient, who after undergoing foot surgery, developed signs and symptoms of post-traumatic reflex sympathetic dystrophy.

Adult↗

[Present place of lumbar sympathectomy in arteritis of the lower limbs (author's transl)].

A study of 75 lumbar sympathectomy helped the authors in specifying the indications of this operation for arteritis of the lower limbs. Lumbar sympathectomy is based on precise anatomical facts : para-vertebral sympathetic chain is usual but a collateral chain must be searched if the latter appears too thin. Lumbar sympathectomy can be performed alone or in association with reconstructive procedures. It is used alone in old people for its antalgic and moderately functional effect in accordance with their level of activity. Also it is used alone in cases of gangrene localized or pending or in the absence of a runoff vasculature. It is associated to repair if one wants to improve an incompetent peripheral perfusion. The authors record 79% of favourable results of which 52% were maintained when lumbar sympathectomy was used alone. In patients where direct vascular surgery is added, favorable results account for 82% of which 78% long lasting.

Aged↗

[Anesthesia for a child with congenital sensory neuropathy with anhydrosis].

We gave anesthesia twice to a 4-year-old boy with congenital sensory neuropathy with anhydrosis. At the first surgery, anesthesia was induced with midazolam and maintained with nitrous oxide, oxygen and sevoflurane 0.5-0.8% under mask breathing. Surgery was performed without any trouble but the patient vomited postoperatively for three days. Next time, anesthesia was induced and maintained with propofol under mask. The patient often moved during surgery, and therefore, we changed from propofol to oxygen and sevoflurane 1.0-1.5% anesthesia. Nitrous oxide was not used. After the surgery, no vomiting occurred.

Anesthesia, Inhalation↗

Autonomous beta-endorphin secretion from the pituitary neurointermediate lobe: in vivo studies.

The existence of independent control mechanisms of beta-endorphin (beta-EP) secretion from the anterior (AP) and intermediate (NIL) pituitary lobes is now ascertained. The aim of this study was to evaluate the effect of surgical separation from the hypothalamus of the two pituitary lobes on beta-EP secretion. Two experimental models of surgical hypothalamo-pituitary disconnection were used: 1) rats with ablation of the medial basal hypothalamus (MBH); 2) rats bearing two entire ectopic pituitaries or two anterior pituitaries (APs) only, transplanted under the kidney capsule. In rats with MBH-ablation plasma beta-EP levels were significantly higher than in sham-operated controls. Plasma beta-EP levels increased in rats transplanted with entire pituitaries 3 days after surgery and were still elevated after 1 week. In rats transplanted with APs only, no significant beta-EP changes in plasma were evident. In both experimental conditions no significant difference was present in beta-LPH plasma levels. Concentrations of beta-EP in the ectopic NILs decreased gradually after transplantation. In all these results indicate that that NIL but not the AP is capable, when is disconnected from the hypothalamus, or secreting autonomously beta-EP.

Animals↗

Ultrastructural changes of human cardiac atrial nerve endings in diabetes mellitus.

BACKGROUND: Autonomic neuropathy resulting from long-term diabetes mellitus may affect heart innervation. However, so far diabetes induced morphological changes of cardiac nerves are not well-known. In this study human cardiac atrial tissue from diabetic patients was analysed by electron microscopy for structural alterations as a result of diabetic neuropathy. METHODS: In coronary bypass surgery, an edge of the right auricle was routinely resected for reason of extracorporal circulation. Thin cardiac tissue sections of 100 nm were studied by electron microscopy. Atrial tissue samples were collected from 5 patients with long-standing diabetes (for at least 8 years) and compared to atrial tissue samples from 5 patients without diabetes, equally undergoing coronary bypass surgery. RESULTS: In all atria-free nerve endings with unmyelinized, axons were observed. Cross sections of 479 axons from diabetic patients were compared to 419 axons of nondiabetic patients. The number of altered axons was significantly higher in cardiac tissue of diabetic patients (32%) in comparison to normal subjects (17%). In diabetic patients, 20% of the intra-axonal mitochondria were condensed or hydropic, whereas in nondiabetic patients only 4% of the mitochondria were altered. Membrane fragments were present in 21% of the axons in atria of diabetic patients compared to 10% in nondiabetic subjects. Only in cardiac axons from diabetic patients there were lamellar bodies, dissolved axoplasma and junctions between neighbouring axons in a minor number. Few vacuoles were present in axons of both groups. CONCLUSION: In myocardial atrial-free nerve fibre bundles of diabetic patients, the amount of degenerative changes was higher in comparison to atrial cardiac tissue from nondiabetic subjects. These morphological alterations may indicate manifestation of diabetic neuropathy and might contribute to the impairment of autonomic neural control affecting the heart in long-standing diabetes mellitus.

Aged↗

Diaphragmatic shortening after thoracic surgery in humans. Effects of mechanical ventilation and thoracic epidural anesthesia.

BACKGROUND: Diaphragmatic function is believed to be inhibited after thoracic surgery and may be improved by thoracic epidural anesthesia. METHODS: Diaphragmatic function after a thoracotomy was monitored by implanting one pair of sonomicrometry crystals and two electromyogram (EMG) electrodes on the costal diaphragm of six patients undergoing an elective pulmonary resection. Crystals and EMG electrodes remained in place for 12-24 h. RESULTS: During mechanical ventilation, costal diaphragmatic length (as a percent of rest length; %LFRC) decreased passively as tidal volume (VT) increased (%LFRC = 2.81 + 1.12 x 10(-2) VT (ml), r = 0.99). During spontaneous ventilation, the costal shortening (2.1 +/- 2.3 %LFRC) was less than during mechanical ventilation (7.9 +/- 3.0 %LFRC, P < 0.05) at the same VT. Comparing spontaneous ventilation before and 30 min after thoracic epidural anesthesia, there were increases of VT (390 +/- 78 to 555 +/- 75 ml), vital capacity (1.37 +/- 0.16 to 1.68 +/- 0.21 l), and esophageal (-8.5 +/- 1.5 to -10.6 +/- 1.7 cmH2O), gastric (-0.7 +/- 0.8 to +0.8 +/- 0.8 cmH2O), and transdiaphragmatic (7.7 +/- 1.5 to 11.5 +/- 1.9 cmH2O) pressures, but diaphragmatic EMG and shortening fraction remained constant. In three of six patients, epidural anesthesia produced paradoxical segment lengthening upon inspiration. CONCLUSIONS: Thoracotomy and pulmonary resection produce a marked reduction of active diaphragmatic shortening, which is not reversed by thoracic epidural anesthesia despite improvement of other indices of respiratory function.

Adenocarcinoma↗