Video-assisted thoracoscopic sympathectomy for Harlequin syndrome.

Harlequin syndrome is an exceedingly rare condition, characterized by unilateral facial flushing and hyperhidrosis. Postulated to be dysregulated sympathetic nervous system stimulation of the dermal vasculature and blood vessels of the face. There is no clear unifying pathological cause. Due to its heterogeneity and rarity, very little is known about the treatment of it. Hereafter, we describe our experience in successfully curing right-sided Harlequin syndrome through video-assisted thoracoscopic sympathectomy.

Approach to the Patient: Perioperative Management of the Patient with Pheochromocytoma or Sympathetic Paraganglioma.

Pheochromocytomas and sympathetic paraganglioma (PPGL) are rare chromaffin cell tumors originating in the adrenal medulla and sympathetic paraganglia, respectively, which share the capacity to synthesize and release catecholamines. The incidence of PPGL has increased in recent years. Surgical resection is the only curative treatment for PPGL. Management of patients with PPGL is complex and should be done by a specialized multidisciplinary team in centers with broad expertise. Surgical resection of a PPGL is a high-risk procedure for which optimal pretreatment with antihypertensive drugs is required in combination with state-of-the-art surgical procedures and anesthesiological techniques. In this article we discuss the underlying evidence and the pros and cons of presurgical medical preparation. Finally, the areas of uncertainty and controversies in this field are addressed.

Resection of hip heterotrophic ossification leads to resolution of autonomic nervous system dysfunction in a patient with spinal Charcot arthropathy: a case report.

INTRODUCTION: Patients with complete spinal cord injury (SCI) may develop concurrent sequalae that interact and share symptoms; thus, a careful approach to diagnosis and management of new symptoms is crucial. CASE PRESENTATION: A patient with prior T4 complete SCI presented with progressive autonomic nervous system (ANS) dysfunction. The initial differential diagnosis included syringomyelia and lumbar Charcot arthropathy. He had comorbid heterotopic ossification (HO) of the left hip. Surprisingly, his autonomic symptoms resolved following resection of the HO. In hindsight, loss of motion through the hip caused by HO may have led to hinging through a previously asymptomatic lumbar Charcot joint, causing dysautonomia. DISCUSSION: ANS dysfunction is a disabling sequela of complete SCI and has a broad differential diagnosis. Hip immobility may be an indirect and overlooked cause due to the mechanical relationship between the hip and the lumbar spine.

Otitis Media: Long-Term Effect on Central Auditory Nervous System.

OBJECTIVES: To analyze the central auditory nervous system function through behavioral and electrophysiological tests in children with a history of otitis media and subsequent bilateral tubes placement surgery. METHODS: The participants were divided into two groups between eight and 14 years old: control group (CG) consisted of 40 children with no history of otitis media; experimental group (EG) consisted of 50 children with documented history of otitis media and undertook a surgery for bilateral tubes placement. All children completed audiological evaluation (audiometry, speech audiometry, and immittance audiometry), behavioral evaluation (tests: dichotic digits, synthetic sentence identification with ipsilateral competing message, gaps-in-noise, frequency pattern), and electrophysiological evaluation (Auditory Brainstem Response, ABR, Frequency Following Response, FFR (verbal), and Long Latency Auditory Evoked Potential, LLAEP). RESULTS: The EG group showed significantly poorer performance (p<0.001) than the CG for all auditory abilities studied. The results revealed significant latency delays and reduced amplitude (p<0.05) of waves III and V for ABR; significant latency delay was seen of potentials P2, N2, and P300 for LLAEP; significant latency delays and reduced amplitude (p<0.05) were observed for FFR in children with a history of otitis media. CONCLUSION: The results demonstrate negative effect of otitis media in the auditory abilities and electrophysiological measures in children with a history of otitis media.

Histological characteristics of eosinophilic myenteric ganglionitis: an under-recognised cause of chronic intestinal pseudo-obstruction.

Eosinophilic myenteric ganglionitis (EMG) is characterised by eosinophilic infiltration of the myenteric plexus. EMG has been rarely reported as a cause of chronic intestinal pseudo-obstruction (CIPO), and its histopathological features are not fully elucidated. We analysed seven patients with CIPO. Three of them were diagnosed with EMG and four patients were categorised as non-EMG. Clinicopathological features were similar in both groups. These features included subtle to mild lymphocytic infiltration at the myenteric ganglia/muscularis propria, loss of myenteric ganglions and interstitial cells of Cajal (ICC), and no significant findings in the mucosa. The exceptions were moderate to severe degree of eosinophilic infiltration at the myenteric ganglia/muscularis propria in EMG. Functional gastrointestinal obstruction may be associated with inflammatory cell infiltration at the myenteric ganglia/muscularis propria, leading to subsequent hypoganglionosis and deficiency of ICC in EMG. Pathologists and clinicians should be aware of this distinction during differential diagnosis of patients with CIPO.

Carotid Body Ablation: a New Target to Address Central Autonomic Dysfunction.

PURPOSE OF REVIEW: An abnormal heightened carotid body (CB) chemoreflex, which produces autonomic dysfunction and sympathetic overactivation, is the common hallmark of obstructive sleep apnea (OSA), resistant hypertension, systolic heart failure (HF), and cardiometabolic diseases. Accordingly, it has been proposed that the elimination of the CB chemosensory input to the brainstem may reduce the autonomic and cardiorespiratory alterations in sympathetic-associated diseases in humans. RECENT FINDINGS: A growing body of evidence obtained in preclinical animal models support that an enhanced CB discharge produces sympathetic hyperactivity, baroreflex sensitivity and heart rate variability impairment, breathing instability, hypertension, and insulin resistance. The elimination CB chemosensory input reduces the sympathetic hyperactivity, the elevated arterial blood pressure in OSA and hypertensive models, abolishes breathing instability and improves animal survival in HF models, and restores insulin tolerance in metabolic models. These results highlight the role played by the enhanced CB drive in the progression of sympathetic-related diseases and support the proposal that the surgical ablation of the CB is useful to restore the autonomic balance and normal cardiorespiratory function in humans. Accordingly, the CB ablation has been used in pilot human studies as a therapeutic treatment for resistant hypertension and HF-induced sympathetic hyperactivity. In this review, I will discuss the supporting evidence for a crucial contribution of the CB in the central autonomic dysfunction and the pros and cons of the CB ablation as a therapy to revert autonomic overactivation. The CB ablation could be a useful method to reverse the enhanced chemoreflex in HF and severe hypertension, but caution is required before extensive use of bilateral CB ablation, which abolished ventilatory responses to hypoxia and may impair baroreceptor function.

Alternative approach to autonomic instability of very severe tetanus: stellate ganglion block / Abordagem alternativa para instabilidade autonômica no tétano muito grave: bloqueio do gânglio estrelado

Abstract Tetanus is an acute and deadly disease caused by Clostridium tetani. A 60-year-old male came to hospital after he injured his thumb with a knife. Ten days later, he returned to hospital with abdominal spasms. He was vaccinated against tetanus and referred to intensive care unit. As he had sudden difficulty in respiration, he was entubated. Midazolam, magnesium and esmolol infusion were started. Next day, muscle spasms progressed all over his body. Midazolam infusion was replaced with propofol and vecuronium. At the third day, morphine infusion was added. At the 16th day, dexmedetomidine infusion was started. At the 20th day, ultrasound guided stellate ganglion block was performed to denervate sympathetic activity. The block was performed three times in a 10 days period. At the 30th, the patient recovered from very severe tetanus. The mainstay of tetanus treatment is adequate sedation. Neuroaxial blocks were proved to be effective for the control of sympathetic overactivity in recent years. Circulatory collapse remains to be the major cause of death. The mechanism is unclear but altered myocardial function is thought to be related to changeable catecholamine levels. The effect of stellate ganglion block on sympathetic and parasympathetic control of heart has been studied since the beginning of 1980s. Recently Scanlon et al. reported they treated a patient with medically refractory ventricular arrhythmias by ultrasound guided bilateral stellate ganglion block. In conclusion, stellate ganglion block can be an alternative method when the autonomic storm cannot be controlled with medical agents.

Resumo O tétano é uma doença aguda e fatal causada por Clostridium tetani. Um homem de 60 anos deu entrada em nosso hospital depois de ferir o polegar com uma faca. Após dez dias, deu entrada no hospital com espasmos abdominais; foi vacinado contra tétano e enviado para a unidade de terapia intensiva. Como apresentava dificuldade súbita na respiração, foi intubado. Foi iniciada uma infusão de midazolam, magnésio e esmolol. No dia seguinte, os espasmos musculares progrediram para o corpo todo. A infusão de midazolam foi substituída por propofol e vecurônio. No terceiro dia, foi adicionada morfina à infusão. No 16º dia, foi iniciada uma infusão de dexmedetomidina. No 20º dia, o bloqueio do gânglio estrelado guiado por ultrassom foi realizado para dessensibilizar a atividade simpática. O bloqueio foi feito três vezes em dez dias. No 30º dia, o paciente recuperou-se de um tétano muito grave. A base do tratamento de tétano é a sedação adequada. Nos últimos anos, os bloqueios neuraxiais provaram ser eficazes para o controle da hiperatividade simpática. O colapso circulatório continua a ser a principal causa de morte. O mecanismo não está claro, mas se acredita que a função alterada do miocárdio esteja relacionada com os níveis de catecolaminas mutáveis. O efeito do bloqueio do gânglio estrelado sobre o controle simpático e parassimpático do coração tem sido estudado desde o início da década de 1980. Recentemente, Scanlon et al. relataram o tratamento de um paciente com arritmia ventricular refratária a medicamentos com bloqueio bilateral do gânglio estrelado guiado por ultrassom. Em conclusão, o bloqueio do gânglio estrelado pode ser um método opcional quando a tempestade autonômica não pode ser controlada com agentes medicamentosos.

Modulation of renal sympathetic innervation: recent insights beyond blood pressure control.

Renal afferent and efferent sympathetic nerves are involved in the regulation of blood pressure and have a pathophysiological role in hypertension. Additionally, several conditions that frequently coexist with hypertension, such as heart failure, obstructive sleep apnea, atrial fibrillation, renal dysfunction, and metabolic syndrome, demonstrate enhanced sympathetic activity. Renal denervation (RDN) is an approach to reduce renal and whole body sympathetic activation. Experimental models indicate that RDN has the potential to lower blood pressure and prevent cardio-renal remodeling in chronic diseases associated with enhanced sympathetic activation. Studies have shown that RDN can reduce blood pressure in drug-naïve hypertensive patients and in hypertensive patients under drug treatment. Beyond its effects on blood pressure, sympathetic modulation by RDN has been shown to have profound effects on cardiac electrophysiology and cardiac arrhythmogenesis. RDN can display anti-arrhythmic effects in a variety of animal models for atrial fibrillation and ventricular arrhythmias. The first non-randomized studies demonstrate that RDN may promote the maintenance of sinus rhythm following catheter ablation in patients with atrial fibrillation. Registry data point towards a beneficial effect of RDN to prevent ventricular arrhythmias in patients with heart failure and electrical storm. Further large randomized placebo-controlled trials are needed to confirm the antihypertensive and anti-arrhythmic effects of RDN. Here, we will review the current literature on anti-arrhythmic effects of RDN with the focus on atrial fibrillation and ventricular arrhythmias. We will discuss new insights from preclinical and clinical mechanistic studies and possible clinical implications of RDN.

[Alternative approach to autonomic instability of very severe tetanus: stellate ganglion block]. / Abordagem alternativa para instabilidade autonômica no tétano muito grave: bloqueio do gânglio estrelado.

Tetanus is an acute and deadly disease caused by Clostridium tetani. A 60-year-old male came to hospital after he injured his thumb with a knife. Ten days later, he returned to hospital with abdominal spasms. He was vaccinated against tetanus and referred to intensive care unit. As he had sudden difficulty in respiration, he was entubated. Midazolam, magnesium and esmolol infusion were started. Next day, muscle spasms progressed all over his body. Midazolam infusion was replaced with propofol and vecuronium. At the third day, morphine infusion was added. At the 16th day, dexmedetomidine infusion was started. At the 20th day, ultrasound guided stellate ganglion block was performed to denervate sympathetic activity. The block was performed three times in a 10 days period. At the 30th the patient recovered from very severe tetanus. The mainstay of tetanus treatment is adequate sedation. Neuroaxial blocks were proved to be effective for the control of sympathetic overactivity in recent years. Circulatory collapse remains to be the major cause of death. The mechanism is unclear but altered myocardial function is thought to be related to changeable catecholamine levels. The effect of stellate ganglion block on sympathetic and parasympathetic control of heart has been studied since the beginning of 1980s. Recently Scanlon et al. reported they treated a patient with medically refractory ventricular arrhythmias by ultrasound guided bilateral stellate ganglion block. In conclusion, stellate ganglion block can be an alternative method when the autonomic storm cannot be controlled with medical agents.

Extracranial schwannoma in the carotid space: A retrospective review of 91 cases.

BACKGROUND: Schwannomas of the vagus nerve and cervical sympathetic nerve are rare; hence, only limited information exists regarding their diagnosis and clinical management. METHODS: We conducted a retrospective review of the clinical features, imaging studies, and treatment results of patients with schwannoma of the vagus nerve and schwannoma of the sympathetic nerve. RESULTS: Of 91 patients, 91% (n = 83) were preoperatively diagnosed with schwannoma tumors. Using the hyoid bone as an anatomic landmark, the location of the schwannoma of the vagus nerve in the carotid space was significantly different to the location of schwannoma of the sympathetic nerve (p = .003). Although 52 of the 76 patients followed up (68%) had postoperative nerve weaknesses, 13 patients (50%) and 14 patients (53.8%), respectively, fully recovered from schwannoma of the vagus nerve and schwannoma of the sympathetic nerve. CONCLUSION: In the carotid space, schwannomas of the vagus nerve are usually located below the hyoid bone, whereas schwannomas of the sympathetic nerve more commonly arise from the suprahyoid compartment. Accurate preoperative diagnosis and the intracapsular enucleation surgical approach decreased the incidence of postoperative morbidity. © 2016 Wiley Periodicals, Head Neck 39: 42-47, 2017.

Sympathetic Chain Schwannoma Resembling Carotid Body Tumour.

Schwannomas are rare, benign nerve sheath tumours of parapharyngeal space. Differential diagnosis should include salivary gland tumours, paragangliomas, neurofibromas, and metastatic lymph nodes. The tumours may arise from vagus nerve and cervical sympathetic chain (CSC). Diagnosis is usually made by imaging techniques: contrast CT, magnetic resonance imaging (MRI), and magnetic resonance angiography (MRA). Fine needle aspiration cytology (FNAC) is useful diagnostic procedure but poor results are seen in neurogenic tumours. Rarely, a vascular CSC schwannoma at the level of carotid arteries bifurcation may mimic carotid body tumour (CBT) on imaging techniques, especially if they are vascular, causing splaying of internal and external carotid arteries. Clinically patient was asymptomatic except for a pulsatile swelling in neck for 5 years. The presented case resembled CBTclinically, on ultrasound and on imaging techniques causing splaying of carotid arteries. FNAC was inconclusive and was always hemorrhagic. During operation, it was found to be CSC schwannoma just posterior to carotid body. CSC was sacrificed and patient developed Horner syndrome postoperatively.

[Features of vegetative dysfunction development in patients with cholelithiasis before and after cholecystectomy].

UNLABELLED: The aim of the present study was a comprehensive study of the features autonomic nervous system in cholelithiasis before and after cholecystectomy. MATERIALS AND METHODS: 88 patients aged 40 to 60 years. 55 patients with cholelithiasis before and after laparoscopic cholecystectomy (CE). Control group consisted of 33 patients of similar age and gender. To investigate the function of the autonomic nervous system were evaluated themes complaint history, physical examination data, and used less Tod mathematical analysis of cardiac rhythm by Baevsky RM using the author's computer-related programs "Korveg" with the definition of heart rate variability and table--Solovevoj Wayne. RESULTS AND CONCLUSIONS: The study of autonomic provision in rest and during exercise were increased sympathetic activity, exceeding those in the control group. Studies indicate a tendency to sympathicotonia patients with gall stones before and after cholecystectomy, which is enhanced adaptive compensatory mechanisms to maintain homeostasis in the body. Identify logical connections between clinical and autonomic indicators will predict flow pattern cholelithiasis before and after cholecystectomy, as well as pick individual therapy for each patient taking into account the autonomic features that can be widely used in practical medicine--not.

Gastrointestinal autonomic nerve tumour of jejunum presenting as a perforated mass.

Gastrointestinal autonomic nerve tumour (GANT) is a rare mesenchymal neoplasm of the gastrointestinal tract arising from the neural plexus of the intestinal wall. Herein, we present a 70-year-old male patient presenting with a clinical picture of acute abdomen. Examination of the specimen obtained from the small bowel by means of complete resection revealed a relatively soft submucosal mass measuring 4.5 x 3 cm in size with spindle morphology and high mitotic activity (> 10 mitoses per 50 high-power fields). The tumour cells were strong positive for c-kit (CD117), S-100 protein and glial fibrillary acidic protein (GFAP), but did not harbour mutations in the c-kit and PDGFR genes. The diagnosis was based on light microscopy and immunohistochemical verification. We started tyrosine kinase inhibitor 400 mg/day. The patient is currently alive without metastasis at 28 months postoperatively. He is under close follow-up and survival data of the patient will be presented in the later studies.

Autonomic nervous system activity in diabetic and healthy obese female subjects and the effect of distinct weight loss strategies.

OBJECTIVE: Obesity and type 2 diabetes mellitus (T2DM) are reported to be associated with relative overactivity of the sympathetic nervous system (SNS), which is reversible by weight loss. However, direct effects of weight loss by calorie restriction vs Roux-en-Y gastric bypass (RYGB) on SNS overactivity were not studied in parallel. This study compared the effects of RYGB vs restrictive weight loss in obese patients with normal glucose tolerance (NGT) and with T2DM on SNS function as measured by heart rate variability (HRV). DESIGN AND METHODS: Lean (n=12), obese NGT (n=27) and T2DM (n=27) subjects were included in this study. Weight reduction in NGT subjects was achieved by gastric banding (GB) or RYGB and in T2DM subjects by RYGB or high-protein very-low-calorie diet (VLCD). HRV analysis was performed and blood samples were taken at baseline, 3 weeks and 3 months after intervention. RESULTS: At baseline, T2DM subjects showed SNS overactivity and NGT subjects showed similar, but non-significant, findings when compared with lean controls. Weight loss after 3 weeks was comparable in all treatment groups, whereas after 3 months, weight loss was most in VLCD and RYGB subjects. RYGB and VLCD treatment reduced SNS activity within 3 weeks in T2DM patients. After 3 months, restoration to normal autonomic nervous system activity was evident for all groups, except for the NGT-GB group. CONCLUSION: We can conclude that SNS overactivity is more pronounced in obese T2DM subjects when compared with NGT subjects. Reduction of SNS overactivity coincides with weight loss with the time-course of reduction dependent on the type of intervention. Surgery or caloric restriction may transiently induce SNS overactivity but do not prevent a direct restoration of sympathovagal balance.

Long-term results of endoscopic sympathetic block using the Lin-Telaranta classification.

BACKGROUND: Endoscopic thoracic sympathectomy has been used successfully in the treatment of blushing, excessive sweating, and social phobia. However, the adverse effects of endoscopic thoracic sympathectomy are more severe and frequent than the adverse effects of endoscopic sympathetic block (ESB). The use of different blocking levels for different indications in ESB according to the Lin-Telaranta classification further decreases the postoperative adverse effects. However, there are few data on the long-term results of ESB performed using the Lin-Telaranta classification. METHODS: Ninety-five patients (55 men, 40 women) were interviewed by before the surgery using our routine questionnaire, and the same questionnaire was answered postoperatively by the patients. In addition, a long-term follow-up questionnaire was sent to all patients whose address was known. Forty-seven patients (24 men, 23 women) answered to this questionnaire. The Davidson brief social phobia scale and the Liebowitz quality of life scale were used. Patients were divided to 3 categories: category 1, patients with sweating problems; category 2, patients with blushing; and category 3, and patients with symptoms other than sweating or blushing. RESULTS: Among patients in category 1, social phobia decreased from 12.43 to 6.71 (p = 0.004), in category 2 from 13.97 to 7.69 (p < 0.001), and in category 3 from 13.18 to 9.64 (p = 0.007) during long-term follow-up. Among patients with severe sweating problems preoperatively, sweating decreased from 2.50 to 1.29 (p = 0.003) among patients in category 1 and from 1.86 to 1.16 (p < 0.001) among patients in category 2. Among patients with unbearable blushing, blushing decreased from 4 to 1.80 (p < 0.001). CONCLUSIONS: Patients got a clear help from ESB performed using the Lin-Telaranta classification to treat blushing, excessive sweating, and social phobia with and without physical symptoms. In addition, compensatory sweating increased only slightly.

Loss of nerve fibers in the oviduct isthmus in women with hydrosalpinx.

Apart from the abnormalities of tubal anatomy, the main concern linked to infertility is impaired tubal motility associated with hydrosalpinx, which is thought to be controlled by hormones and nerves. The objective of this study was to determine the distribution of nerve fibers in the oviduct isthmus in women with and without hydrosalpinx. Histological sections of the oviduct isthmus tissue were obtained from 18 women undergoing salpingectomy for hydrosalpinx, and from 15 women undergoing hysterectomy and salpingectomy for benign gynecologic diseases. The tissues were immunohistochemically stained for protein gene product (PGP) 9.5, protein S100, neuropeptide tyrosine (NPY), and vasoactive intestinal peptide (VIP) to reveal all nerve fibers, as well as sympathetic and parasympathetic nerve fibers, in the oviduct isthmus. We detected the presence of PGP9.5, S100, VIP, and NPY-immunoreactive nerve fibers in the oviduct isthmus in all study subjects. However, the densities of PGP9.5, S100, VIP, and NPY-immunoreactive nerve fibers in the oviduct isthmus were all significantly decreased in women with hydrosalpinx compared with those in women without hydrosalpinx (P<0.01). Our results suggest that reduced nerve fibers in the oviduct isthmus in women with hydrosalpinx compared with women without hydrosalpinx may have an important function in the mechanism of hydrosalpinx-associated infertility.