Shy-Drager syndrome A review and a description of the anaesthetic management

Autonomic failure in patients with the Shy-Drager syndrome may produce cardiovascular instability during anaesthesia and surgery. The syndrome is reviewed and the anaesthetic management of a case is described. The choice between general and regional anaesthesia seems to be less important than adequate cardiovascular monitoring and the maintenance of blood pressure with intravenous fluids. Sympathomimetic drugs, if used at all, should be administered in very dilute solutions to avoid hypertension from denervation hypersensitivity. In the postoperative period, symptoms from orthostatic hypotension may be severe and their control requires prolonged postural training, by elevation of the head of the bed, and therapy with 9-alpha-fludrocortisone.     

Anaesthetic management of a parturient with the postural orthostatic tachycardia syndrome: a case report

Postural orthostatic tachycardia syndrome encompasses a groupof disorders characterized by orthostatic intolerance. We describethe anaesthetic management of analgesia for labour and of Caesareansection in a parturient suffering from this disorder. Worseningof her symptoms during pregnancy was managed with an increasein the dose of ß-blockers taken by the patient. Epiduralanalgesia was instigated early to attenuate the stress of labourand avoid consequent triggering of a tachycardic response. Slowtitration of epidural analgesia and anaesthesia after an adequatefluid preload was undertaken to minimize hypotension and subsequenttachycardia. Neuraxial opioid, combined with non-steroidal anti-inflammatorydrugs and bilateral iliohypogastric and ilioinguinal nerve blockswere used to optimize postoperative analgesia.     

Anaesthesia and the Kearns-Sayre syndrome

A six-month-old infant presented for anaesthesia with, unbeknown to us, some of the manifestations of the Kearns-Sayre Syndrome. This syndrome is one of the clinical presentations in patients with mitochondral myopathy. The anaesthetic related events and aspects of mitochondral myopathy are described and caveats are suggested for the management of anaesthesia for patients with this type of myopathy.     

Difficult intubation during rapid sequence induction in a parturient with Ehlers-Danlos syndrome, hypermobility type

There have been several reports of resistance to local anaesthetic agents in women with Ehlers-Danlos syndrome, hypermobility type, also known as Ehlers-Danlos syndrome Type III. General anaesthesia with rapid sequence induction was performed for caesarean section due to prolonged second stage of labour, but intubation proved to be difficult. We propose that intubation difficulty probably arose from collapse of fibro-elastic tissues and adjoining C-shaped cartilages of the trachea with appropriately applied cricoid pressure. We found no other case reports of difficult intubation in patients with Ehlers-Danlos syndrome, hypermobility type. There are reports of cervical spine instability and temporomandibular joint dysfunction in patients with this syndrome suggesting a potential for difficult airway management. Additional anaesthetic problems associated with Ehlers-Danlos syndrome involve patient positioning and vascular access.     

Anaesthesia in a child with Patau's syndrome

A 5-year-old girl who had Patau's syndrome associated with double outlet right ventricle and pulmonary stenosis required general anaesthesia for the formation of a modified Blalock-Taussig shunt.     

Anaesthesia for an adolescent with mitochondrial encephalomyopathy-lactic acidosis-stroke-like episodes syndrome

Anaesthesia for patients with mitochondrial disorders occurs with regularity, either for a diagnostic or therapeutic procedure associated with the syndrome or for unassociated surgery. We report the case of a 17-year-old boy with mitochondrial encephalomyopathy-lactic acidosis-stroke-like episodes (MELAS) syndrome who underwent a laparotomy for fundoplication. The potential hazards of anaesthesia for this patient, and the steps taken to avoid them, are discussed.     

Anaesthetic management of a child with Pallister-Killian syndrome

Pallister-Killian syndrome is characterized by tetrasomy of the short arm of chromosome 12p, which produces mental retardation of varying degrees and dysmorphic characteristics. We describe anaesthesia in a 2-year-old child affected by this syndrome who underwent surgery for orchidopexy. Anaesthetic consisted of an inhalation mixture of O2, N2O and sevoflurane, together with an inguinal block with ropivacaine and administration of alfentanil plus ketorolac. Tracheal intubation was uneventful. No complications of any type were observed.     

Tumour lysis syndrome during anaesthesia

We describe a child who developed acidosis, hyperkalaemia and cyanosis while emerging from an otherwise uneventful anaesthetic. A diagnosis of tumour lysis syndrome was suspected and confirmed biochemically. Although this syndrome is usually associated with chemotherapy and has been described to occur spontaneously, it has not previously been reported as being associated with anaesthesia.     

ACE inhibition can improve orthostatic proteinuria associated with nutcracker syndrome

Left renal vein entrapment syndrome (nutcracker syndrome) was documented by magnetic resonance angiography (MRA) as a cause of orthostatic proteinuria in a 14-year-old girl female adolescent. Because of continuous proteinuria we performed a left renal biopsy which showed moderate mesangial hypercellularity. Her overt orthostatic proteinuria disappeared after a treatment of angiotensin-converting enzyme (ACE) inhibition. Nutcracker syndrome remains a rare but important cause of elevated protein excretion, which can induce mesangial changes and be improved by ACE inhibitor treatment.     

Anaesthetic management of Osler-Weber-Rendu syndrome with coexisting congenital methaemoglobinaemia

A 9-year-old cyanosed child suffering from Osler-Weber-Rendu syndrome with bilateral pulmonary arteriovenous malformations (PAVMs) was posted for cerebral angiography under general anaesthesia. Careful preanaesthetic evaluation led to the diagnosis of coexisting congenital methaemoglobinaemia. There is no previous report of Osler-Weber-Rendu syndrome coexisting with congenital methaemoglobinaemia. This report emphasizes that a second contributory cause of cyanosis must be suspected and meticulously looked for if the symptomatology in a patient cannot be explained by a single established diagnosis. Positive-pressure ventilation was associated with reduction in arterial oxygenation despite an increasing inspired oxygen concentration, which returned to preanaesthetic levels only after extubation and resumption of spontaneous respiration.     

Anaesthetic management of a patient with Liddle's syndrome for emergency caesarean hysterectomy

We describe the anaesthetic management of a patient with Liddle’s syndrome during caesarean section and emergency hysterectomy for placenta accreta associated with significant intrapartum haemorrhage. Liddle’s syndrome is a rare autosomal dominant disorder characterised by early onset arterial hypertension and hypokalaemic metabolic alkalosis. Additional issues were the presence of short stature, limb hypertonicity and preeclampsia. Initial management with a low-dose combined spinal-epidural technique was subsequently converted to general anaesthesia due to patient discomfort. The management of Liddle’s syndrome in the setting of neuraxial and general anaesthesia in a patient undergoing caesarean section is discussed.     

Anaesthesiological considerations in patients with Sneddon's syndrome

Sneddon's syndrome is a rare disease with strong gender prevalence of females. This syndrome is characterized by livedo racemosa and cerebrovascular lesions. Since no specific test is available, the clinical differentiation from other disorders with similar symptomatology may raise difficulties. The cerebral involvement includes strokes with cases of more than one ischaemic event having been reported. Associations with convulsions, heart valve disease, systemic hypertension, and renal impairment have been described. We report the case of a 5-year-old boy who was anaesthesized for dental surgery. Due to the fact that 50% of Sneddon's syndrome patients develop mental retardation, even minor procedures require general anaesthesia. A review of the literature is added and specific anaesthesiological aspects of the perioperative care of Sneddon's syndrome are discussed.     

Long QT interval syndrome with increased QT dispersion

This case report describes the anaesthetic management of a patient with sporadic-type long QT interval syndrome (LQTS), and increased QT dispersion, who presented for removal of an ovarian cyst. Beta adrenergic blockade and adequate depth of anaesthesia for successful management is emphasized. The Successful use of epidural administration of lignocaine and opioids in addition to general anaesthesia is described.     

Anaesthesia in a child with Sotos syndrome

Sotos syndrome is a rare condition characterized by typical facies, early accelerated growth, large body size, developmental delay and congenital heart defects. Reports of anaesthetic management of these children are very rare. We report a case of general anaesthesia in a 2(1/2)-year-old boy with this condition, undergoing inguinal hernia repair. The child had a marked developmental delay, hypotonia and mitral regurgitation. The key points in the management of anaesthesia in Sotos syndrome are discussed.     

Anaesthesia for cholecystectomy in two non-parturients with Eisenmenger's syndrome

Eisenmenger's syndrome consists of high pulmonary vascular resistance with reversed or bidirectional shunt at aortopulmonary, ventricular or atrial level. We describe the anaesthetic management of two adult females with Eisenmenger's syndrome admitted for laparoscopic cholecystectomy. One patient suffered post-operative complications, but the other case was uncomplicated. We used sevoflurane and total intravenous anaesthesia to provide general anaesthesia. Both techniques were tolerated.     

Difficult airway in a patient with Marshall-Smith syndrome

Marshall-Smith syndrome is a rare clinical disorder characterized by accelerated bone maturation, dysmorphic facial features, airway abnormalities and death in early infancy because of respiratory complications. Although patients with Marshall-Smith syndrome have several features with potential anaesthetic problems, previous reports about anaesthetic management of these patients do not exist. We present a case, in which severe hypoxia developed rapidly after routine anaesthesia induction in an eight-month-old male infant with this syndrome. After several unsuccessful attempts the airway was finally secured by blind oral intubation. After 2 weeks, laryngeal anatomy was examined with fibreoptic laryngoscopy which revealed significant laryngomalacia. Laryngoscopy was performed without problems with ketamine anaesthesia and spontaneous breathing. The possibility of a compromised airway should always be borne in mind when anaesthetizing patients with Marshall-Smith syndrome. Anaesthesia maintaining spontaneous breathing is safest for children with this syndrome. If tracheal intubation or muscle relaxation is required, precautions are needed to maintain a patent airway. Muscle relaxants should possibly be avoided before intubation.     

Pre-treatment with somatostatin in the anaesthetic management of a patient with carcinoid syndrome

Carcinoid syndrome produces flushing, bronchoconstriction and gastrointestinal hypermotility secondary to serotonin, histamine, bradykinin and prostaglandin release. A variety of drugs, foods and anaesthetic agents may provoke this syndrome. Under anaesthesia, the flushing produced may be associated with acute hypotension and cardiovascular collapse; this phenomenon is called a carcinoid crisis. Recently, somatostatin analogue has been used successfully to treat intraoperative carcinoid crisis. In this report, we present a 66-year-old lady with carcinoid syndrome who was pre-treated with 50 micrograms somatostatin analogue IV and IM prior to surgical manipulation. The anaesthetic course was relatively uneventful and the patient did well postoperatively.     

Anaesthesia for a child with Walker-Warburg syndrome

Walker-Warburg syndrome (WWS) is a rare lethal autosomal recessive disorder manifested by characteristic central nervous system and eye malformations. We have not come across reports of general anaesthesia in a child with WWS in the English literature. We report a case of general anaesthesia in a 12-month-old male child with WWS. The child also had bilateral cleft lip, cleft palate, urogenital malformation and hydronephrosis. Despite many potential anaesthesia concerns, anaesthesia was uneventful in this child.     

General anaesthesia for lobectomy in an 8-year-old child with Kartagener's syndrome

Kartagener's syndrome (KGS) is an autosomal recessive disorder characterized by the clinical triad of bronchiectasis, sinusitis and dextrocardia (situs inversus). Reports of general anaesthesia in a child with KGS are rare in medical literature. We describe the case of a young boy who underwent lobectomy under general anaesthesia using a single lumen tube. Anaesthetic implications of this syndrome are mentioned. Key points in the management of anaesthesia in KGS are discussed.     

Spontaneous mesenteric hemorrhage associated with ehlers-danlos syndrome

The vascular type of Ehlers-Danlos syndrome is a genetic disorder of connective tissue and is frequently associated with catastrophic arterial complications. Its surgical treatment is extremely difficult because of the fragility of vessels. This article describes three patients with vascular type of Ehlers-Danlos syndrome who developed mesenteric hemorrhage due to spontaneous arterial rupture. The clinical and molecular characteristics of the disease are briefly reviewed.