Recognition and management of phaeochromocytoma

Phaeochromocytomas and paragangliomas (PPGL) are catecholamine-secreting neuroendocrine tumours. These tumours may be identified incidentally, as part of a work-up for multiple endocrine neoplasia or following haemodynamic surges during unrelated procedures. Advances in preoperative management and improved management of intraoperative haemodynamic instability have significantly reduced surgical mortality from around 40% to less than 3%. Surgery is the definitive treatment in most cases and laparoscopic resection where possible is associated with improved outcomes. Anaesthetic management of PPGL cases represents a unique haemodynamic challenge both before, during and after tumour resection. In this article we describe the physiology of these tumours, their diagnosis, preoperative optimization methods, intraoperative anaesthetic management and management of postoperative complications.     

Recognition and management of phaeochromocytoma and paraganglioma

Phaeochromocytomas and paragangliomas (PPGL) are catecholamine-secreting neuroendocrine tumours arising from the chromaffin cells in the adrenal medulla. These tumours may be identified incidentally, as part of a work-up for multiple endocrine neoplasia or following haemodynamic surges during unrelated procedures. Advances in perioperative management and improved management of intraoperative haemodynamic instability have significantly reduced surgical mortality from around 40% to less than 3%. Surgery is the definitive treatment in most cases and laparoscopic resection where possible is associated with improved outcomes. Anaesthetic management of PPGL cases represents a unique haemodynamic challenge both before and after tumour resection. In this article we describe the physiology of these tumours, their diagnosis, preoperative optimization methods, intraoperative anaesthetic management and management of postoperative complications.     

Endocrine tumours of the pancreas

Endocrine tumours of the pancreas, also known as islet cell tumours, are neuroendocrine tumours arising from the pancreatic islets. Pancreatic endocrine tumours are uncommon and no endocrine surgeon has vast experience with them. Patients with these rare tumours present either with a specific syndrome or with symptoms related to the malignant nature of the tumours. In addition, an increasing number of ‘pancreatic incidentalomas’ with no obvious function have been detected by abdominal computerized tomography. Diagnosis relies on a high index of clinical suspicion and is based on the demonstration of elevated serum levels of the secretory products concerned. Therapeutic strategy depends on the exact localization of the primary tumor and the presence or absence of metastasis, and is designed to control the tumoral process as well as ameliorate the syndrome associated with it. The present review highlights the clinical presentation, diagnosis, localization and therapeutic principles in the management of pancreatic endocrine tumours.     

Pituitary disease and anaesthesia

Patients with pituitary gland tumours comprise a significant proportion of the neurosurgical population, making transphenoidal pituitary surgery a relatively common procedure. These patients present unique anaesthetic challenges resulting from hormone hypersecretion, pituitary hypofunction or tumour mass effect. To understand this pathophysiology requires knowledge of normal pituitary anatomy and physiology. Respiratory and cardiovascular sequelae of pituitary tumours also pose anaesthetic challenges. We aim to review the key principles involved in the preoperative assessment, intraoperative management and postoperative care of these patients.     

Anaesthetic management of a patient with a large neck mass

Large thyroid masses present several potential difficulties for anaesthetists. These include distortion of the airway, endocrine disturbances, and metastatic effects. A typical case is presented and the anaesthetic management and considerations are described.     

Pituitary disease and anaesthesia

Patients with pituitary gland tumours comprise a significant proportion of the neurosurgical population, making transphenoidal pituitary surgery a relatively common procedure. These patients present unique anaesthetic challenges resulting from hormone hypersecretion, pituitary hypofunction or tumour mass effect. To understand this pathophysiology requires knowledge of normal pituitary anatomy and physiology. Respiratory and cardiovascular sequelae of pituitary tumours also pose anaesthetic challenges. We aim to review the key principles involved in the preoperative assessment, intraoperative management and postoperative care of these patients.     

Cryosurgery in the management of rectal tumours.

Ten patients with rectal tumours were treated by cryosurgery. Good palliation without local complications was achieved in the cases of rectal carcinoma and a complete cure followed treatment of benign rectal tumours. Low rectal carcinomas with clinical evidence of dissemination are ideally suited for cryosurgery and complications are minimal. Cryosurgery is a simple, safe procedure which can be performed without a general anaesthetic, and this study indicates that it has a place in the management of rectal tumours.     

Anasthesia and mediastinal masses A case report and review of the literature

A case in which a mediastinal tumour caused complications including airway obstruction unrelieved by intubation during inhalational induction is described. Other case reports are reviewed and the anaesthetic management of patients with mediastinal tumours is discussed.     

Transtemporal-transchoroidal approach and its transamygdala extension to the posterior chiasmatic cistern and diencephalo-mesencephalic lesions

The aim of this report is to illustrate the successful surgical management of five patients with suprasellar retrochiasmatic and diencephalo-mesencephalic tumours, using a trans-amygdala extension of the trans-temporal/trans-choroidal fissure approach. The procedure is described for five surgically treated patients with such lesions that includes three retrochiasmatic craniopharyngiomas, one hypothalamic hamartoma, and one pilocytic astrocytoma in the left crus cerebri. The management procedure included computed tomography scan (CT scan) and magnetic resonance imaging (MRI). The trans-temporal/trans-choroidal fissure approach enabled us to perform total tumour resection in four patients and a subtotal resection in one. Some technical aspects and pitfalls of the procedure are discussed. This method creates optimum conditions for a radical excision of various suprasellar retrochiasmatic and diencephalo-mesencephalic tumours without mortality and only minimum morbidity. However, no single surgical approach can provide access to the entire variety of tumours located in the suprasellar retrochiasmatic and diencephalo-mesencepalic region. Surgical approaches must be tailored to the site, type of lesion, and its extensions. This method is only another surgical option. Its successful use requires a familiarity with the anatomy of these regions and an understanding of its specificity for a radical excision of some selected tumours, as well as its limitations to access others types of lesions. Although, it seems effective, this approach needs to be evaluated by further experience, owing to the small number of patients reported in this series.     

Maxillary hypoplasia with unsuspected choanal atresia

Maxillary hypoplasia can pose considerable anaesthetic problems in securing the airway. The presence of concomitant, unsuspected choanal stenosis complicated the anaesthetic management of a 20-day-old baby for tarsorrhaphy.     

High frequency jet ventilation via a tracheoscope for endobronchial laser surgery

Palliative surgery of malignant endobronchial tumours is associated with various anaesthetic risks. A technique is described using a neodymium:yttrium-aluminium-garnet (Nd:YAG) laser in combination with a special tracheoscope and high frequency jet ventilation (HFJV). The purpose of this technique is to provide separation of anaesthetic and surgical manipulations, good conditions of ventilation and easy access to the lungs for the surgeon. We present our experience with 14 adult patients in whom large endobronchial neoplasms were resected using this technique under intravenous anaesthesia and muscle relaxation. Careful perioperative monitoring and a good outcome allowed us to conclude that this set-up may contribute to improving efficiency and safety of endoscopic resections of endobronchial and/or endotracheal tumours.     

Anaesthetic management of awake craniotomy

This review article presents a detailed analysis of patients' management for awake craniotomy, at the light of the available data in the literature and the authors' experience. Indications of this type of surgery are discussed as well as anaesthetic management itself, from preoperative assessment of the patient to peroperative concerns. Anaesthetic strategy, choice of anaesthetic agents, anaesthetic technique, and management of the airway and possible complications are discussed. The authors emphasize the tricky aspect of the procedure, the necessity of rigorous patient selection and good preparation. They emphasize the need for controlled studies to validate the proposed techniques.     

Rare testicular tumors. Apropos of 15 cases

In a series of 141 patients treated over the last ten years for testicular tumour, 15 presented lesions considered to be rare: 9 Leydig cell tumours, 3 epidermoid cysts, 1 leiomyoma, 1 primary testicular lymphoma and one testicular localisation of a known leukaemia. The clinical, endocrine and histological features of these different tumours are reviewed on the basis of the present series and a wider discussion of other rare testicular tumours. Leydig cell tumours, epidermoid cysts, mature benign teratomas and testicular lymphomas are the most frequent. Leiomyomas, metastatic tumours and connective tissue tumours are exceptional. Sertoli cell tumours are rare and are similar to Leydig cell tumours in that they raise problems concerning their possible endocrine activity and the evaluation of their malignant potential. The general therapeutic rule of radical orchidectomy for any testicular tumour is still valid.     

Surgical management of large desmoid tumour of the anterior abdominal wall

Desmoid tumours are uncommon. They are locally invasive and incomplete excision leads to recurrence, which can pose a significant management challenge. Patients therefore require effective treatment, which essentially entails tumour excision with a clear surgical margin. The resulting wide defect may lead to difficulty in closure of the anterior abdominal wall. We report our experience in treating large desmoid tumours of the anterior abdominal wall. Between January 2000 and December 2001, three patients with large desmoid tumour of the anterior abdominal wall were treated with wide excision, which included a 3-cm margin of uninvolved tissues. This led to a considerable abdominal wall defect. The peritoneal defect was closed as a separate layer, though under considerable tension, while the abdominal wall musculature defect was closed with a polypropylene mesh. All three patients recovered well with no immediate or late postoperative morbidity. Follow-up until December 2006 has not revealed any tumour recurrence or hernia development. Wide excision of an anterior abdominal wall desmoid tumour with a clear margin of 3 cm including the peritoneum should be considered when managing such tumours. Closure under tension of the peritoneum did not seem to produce any morbidity.     

Tracheomalacia and bronchomalacia in children: pathophysiology,assessment, treatment and anaesthesia management

Tracheomalacia and bronchomalacia are becoming increasingly well recognized. Although pathologically benign conditions, they are responsible for considerable morbidity, occasional mortality and significant difficulties in the operating theatre and intensive care unit. We performed an extensive literature search to identify causal associations, methods of clinical and investigative assessment, treatment modalities and anaesthetic experience with these conditions.     

Tumours of the glomus jugulare

Two cases are reported using a two stage procedure for the excision of glomus jugulare tumours. The anaesthetic problems which this may produce are discussed, and recommendations are made regarding the anaesthetic conduct for this procedure. A two stage, combined procedure is suggested to reduce anaesthetic morbidity.     

HELLP syndrome and the anaesthetist

Four pregnant patients are described who had varying signs of pre-eclampsia plus haemolysis, elevated liver enzymes and a low platelet count. Two of the patients presented without hypertension and all posed considerable diagnostic difficulties, with problems in clinical management. Pregnancy induced hypertension is only one manifestation of a much more diverse pathophysiological process. Anaesthetists need to be aware of these other pregnancy related disorders in order to avoid diagnostic pitfalls and to enable them to provide safely the appropriate general and regional anaesthetic techniques.     

Jet ventilation for the excision of vocal cord polyps in a pregnant patient

The anaesthetic management of patients presenting with laryngeal tumours and airway obstruction is difficult. We present the case of a pregnant woman at 30 weeks gestation who underwent surgical removal of two vocal cord polyps under general anaesthesia using jet ventilation     

Anaesthetic management of a pregnant woman with carcinoid disease

Carcinoid tumours are neuroendocrine in origin and release vasoactive substances. Carcinoid tumours may be associated with carcinoid syndrome in 2–5% of patients and result in haemodynamic instability, bronchospasm, volume and electrolyte imbalance, and hyperglycaemia. We present the anaesthetic management of a 29-year-old parturient with metastatic carcinoid tumour. Although our patient did not ultimately develop carcinoid syndrome during the peripartum period, it was important that we used a multidisciplinary team approach, with close monitoring of her antenatal progress, and planned epidural analgesia for labour and delivery.     

Synchronous lymph node and liver metastasis in a well differentiated gastric endocrine tumour type I

Gastric endocrine tumours constitute less than 1% of all (neuro-)endocrine tumours and less than 2% of all gastrointestinal malignancies. They are classified into three groups: well-differentiated endocrine tumours, well-differentiated endocrine carcinoma, poorly-differentiated endocrine carcinoma. We report the rare case of a patient with a metastasising gastric endocrine tumour that histologically revealed all signs of a well-differentiated endocrine tumour.