Anaesthetic management of a patient with Leigh’s syndrome

Purpose

Leigh’s syndrome, a progressive neurodegenerative disorder of infancy and childhood, is clinically charactenzed mainly by developmental delay, nervous system dysfunction and respiratory abnormalities such as aspiration, wheezing, breathing difficulties, gasping, hypoventilation and apnoea. Acute exacerbation and respiratory failure may follow surgery, general anaesthesia or intercurrent illnesses. Hyperlactataemia is variably present. Histopathological findings include necrosis, vascular proliferation, astrocytosis and demyelination of several brain areas. We present a 30-month-old patient with Leigh’s syndrome anaesthetized for extracorporeal shockwave lithotripsy, and describe the anaesthetic considerations.

Clinical features

Leigh’s syndrome was diagnosed at five months of age based on failure to thrive, lethargy, hypotonicity, choreo-athetosis and lactic acidaemia, with basal ganglia hypodense areas demonstrated by brain computerized tomographic scan. Muscle pyruvate dehydrogenase complex and NADH-coenzyme Q oxidoreductase activity were 25% and 13% of control. No preoperative respiratory symptoms or signs were present. Preoperative fasting lasted two hours and gastric aspiration was negative. Anaesthesia was induced with ketamine and midazolam im, and N₂O in oxygen, and maintained with propofol and N₂O. No volatile anaesthetics were used. Intravenous fluids given were 1/2 normal saline and glucose 5% administered. Besides laryngospasm dunng anaesthetic induction, relieved by sublingual succinylcholine injection, the perianaesthetic course was uneventful. The lungs were mechanically ventilated and lithotripsy was performed. No adverse sequelae have occurred, and the patient was discharged one day later.

Conclusion

Perioperative management of patients with Leigh’s syndrome requires cautious attention to the metabolic, neurological and respiratory aspects of the disease, and appropriate selection of anaesthetic drugs.     

Anaesthetic management of a child with Bartter’s syndrome

We report the anaesthetic management of an eight-year-old asthmatic boy with Bartter’s syndrome who had bilateral orchidopexy with caudal epidural analgesia. Bartter’s syndrome is a rare congenital disorder characterized by hypokalaemic hypochloraemic metabolic alkalosis, hyperaldosteronism, hyperreninaemia and hyperplasia of the juxtaglomerular apparatus of the kidneys. Characteristically, although these patients are normotensive they may be hypovolaemic. They may have unstable baroreceptor responses and show marked resistance to vasopressors. Hence, fluid, acid-base and electrolyte imbalances along with haemodynamic instability pose particular problems in their anaesthetic management. Previous case reports have described the management of these patients with general anaesthesia, our patient had his orchidopexy with caudal epidural analgesia using plain bupivacaine 0.5%. The patient was haemodynamically stable throughout surgery and was comfortable with caudal analgesia as the sole anaesthetic. Hypovalaemia, acid-base status and electrolyte imbalance were treated before instituting caudal epidural analgesia. We present this case report which describes the anaesthetic considerations in the light of the pathophysiology of Bartter’s syndrome.     

Anaesthetic anagement of Miller’s syndrome

Miller's syndrome is a rare congenital disorder with facial features similar to that of Treacher-Collins syndrome. This report details the anaesthetic management of an infant during multiple surgical procedures, beginning with pylormyotomy at one month of age. Airway management was difficult because of severe micrognathia and was accomplished using an awake intubation with a conventional straight blade modified for continuous administration of oxygen ("oxyscope"). Due to recurrent upper airway obstruction and the anticipated need for multiple surgical procedures in the first years of life, a tracheostomy was placed. Because of the multiple airway, orthopaedic, and nutritional difficulties, it is important that a prospective, multidisciplinary approach be used in these patients' care. Consideration should be given to early tracheostomy for airway maintenance.     

Anesthetic management of Guillain-Barré syndrome in pregnancy

We report the case of a 23-year-old woman who was diagnosed with an axonal type of Guillain-Barré syndrome at 16 weeks' gestation. The patient had severe motor loss but she was treated effectively with intravenous immunoglobulin, and she underwent cesarean delivery with epidural anesthesia at full term.     

What’s new in management of bone metastases?

Metastases are the most common malignancy in bone. In patients with bone metastases, especially if a limited expected survival, the indications for surgical treatment are limited, immediate pain relief and improvement in the functional status are important, and complications of treatments are unwanted. Novel medical treatments can offer an effective palliative option in these patients. Advances in interventional radiology and surgery have led to the development of less invasive techniques with the aim to achieve the same clinical results with less surgical morbidity. These include embolization, electrochemotherapy, magnetic resonance imaging-guided high-intensity focused ultrasound, and thermal ablation. Less invasive techniques combine the advantages of less invasive procedures including decreased blood loss, earlier functional recovery and initiation of adjuvant medical therapies and seem to be both effective in pain relief and local tumor control.

     

Surgical management of Crohn’s disease

Introduction

Crohn’s disease is an inflammatory bowel disease that can affect the entire gastrointestinal tract. It is chronic and incurable, and the mainstay of therapy is medical management with surgical intervention as complications arise. Surgery is required in approximately 70% of patients with Crohn’s disease. Because repeat interventions are often needed, these patients may benefit from bowel-sparing techniques and minimally invasive approaches. Various bowel-sparing techniques, including strictureplasty, can be applied to reduce the risk of short-bowel syndrome.

Methods

A review of the available literature using the PubMed search engine was undertaken to compile data on the surgical treatment of Crohn’s disease.

Results and conclusion

Data support the use of laparoscopy in treating Crohn’s disease, although the potential technical challenges in these settings mandate appropriate prerequisite surgical expertise.     

The surgical management of obesity in young women: consideration of the mother’s and baby’s health before, during, and after pregnancy

Background  Maternal obesity has a substantial associated morbidity and mortality affecting both mother and child. This has a major impact on provision of care due to increased requirements for both medical and surgical management of the consequences that follow obesity in pregnancy. Methods  A review examined the English language literature on Medline databases describing the effect of obesity on pregnancy and outcomes of pregnancy after bariatric surgery. Guidelines from the National Institute for Clinical Excellence yielded information on selection criteria for fertility treatment and bariatric surgery. The World Health Organization definitions of overweight and obesity were adhered to throughout this review. Results  The level of clinical and morbid obesity has shown a dramatic increase in women of childbearing age, with far-reaching consequences for both their own health and that of their offspring. Obese women require a substantial amount of additional clinical care beyond that offered to women of normal weight due to the wide range of medical and obstetric complications they experience. Recent evidence suggests that obesity may be implicated in approximately one-third of maternal deaths. The consensus of the literature is that the best way to reduce obesity-associated morbidity is by weight reduction before pregnancy. Where behavioral and medical interventions fail, the most successful method is bariatric surgery. The effect of surgery on the outcome of subsequent pregnancies indicate improved chances of normal pregnancy, delivery, and healthy babies. Conclusions  Bariatric surgery is a safe and effective method of weight loss for morbidly obese women of childbearing age, with favorable outcomes for pregnancies after surgery.     

The role of diagnostic block in the management of Morton’s neuroma

Objective

To determine the outcome of surgical excision of Morton’s neuroma after a local anesthetic diagnostic block into the neuroma has relieved symptoms.

Design

A cohort study.

Setting

A university affiliated hospital.

Patients

A sequential series of 37 patients who underwent 41 excisions with at least 2 years’ follow-up. Seven patients had undergone repeat excision of a neuroma, and 34 primary excisions were performed. Surgery was performed by a specialist in orthopedic surgery of the foot and ankle.

Intervention

Excision of the Morton’s neuroma after a positive diagnostic block.

Main outcome measures

Grade of symptoms at follow-up done by independent review on a 4-point scale.

Results

Of 41 procedures, 11 had an unfavourable outcome: 4 procedures were graded 3, and 7 procedures were graded 4. Eight (24%) of the 34 primary procedures were reported as failures, and 3 (43%) of the 7 revision procedures were reported as failures. Most patients reported poor results owing to persistent pain.

Conclusions

Diagnostic blocks do not improve the results of surgery for excision of Morton’s neuroma and are not recommended. Because failure rates are greater than 20%, surgery for Morton’s neuroma should only be offered after a full course of nonoperative management.     

Endoscopic total extraperitoneal management of Amyand’s hernia

Presence of an appendix in a hernial sac (Amyands hernia) is a rare entity, still rarer is an inflamed appendix within the hernial sac. An endoscopic total extraperitoneal repair of a right-sided incarcerated inguinal hernia is described. The inguinal hernial sac contained an inflamed appendix. An associated small incisional hernia at the lower end of a midline suprapubic scar for a Millins prostatectomy was simultaneously repaired.     

Peri-operative management of patients with Parkinson’s disease

Patients with Parkinson’s disease are at higher risk of peri-operative medical and surgical complications. Multidisciplinary management, early recognition of potential complications, specialised care of medications and intra-operative protection of the vulnerable brain are all important aspects of the peri-operative management of patients with Parkinson’s disease. Advances in continuous dopaminergic treatment, development of a peri-operative Parkinson’s disease pathway and application of telemedicine are starting to play a role in improving peri-operative care. Management of patients with advanced Parkinson’s disease is also evolving, with potential for incorporation of integrated care and changes in the anaesthetic management for deep brain stimulation surgery. There are new methods for localisation of target nuclei and increasing insight on the effects of anaesthetic drugs on microelectrode recordings and clinical outcomes. Parkinson’s disease is a progressive disease, but management is improving with better peri-operative care for patients.     

Anaesthetic considerations in down’s syndrome: experience with 100 patients and a review of the literature

Down’s Syndrome (Trisomy 21, T21) occurs in approximately 0.15 percent of live births. In addition to the stigmata of the syndrome, other congenital defects are frequently found in these patients. Cardiac lesions are particularly prominent. To determine the complications associated with anaesthesia and surgery we examined the records of 100 consecutive patients (58 males, 42 females) who underwent surgery with general anaesthesia during a two year period, from March 1978-March 1980. In addition to the cardiac lesions, the low birth weight of Trisomy 21 infants, increased suceptibility to infections, atlanto-occipital dislocation, and reduced central nervous system catecholamine levels might be expected to result in an increased incidence of complications. This study of 100 patients with Trisomy 21 (T21) indicates that the incidence of complications is low. However, the anaesthetist must understand the pathophysiology of T21 in order to provide optimal anaesthetic care.     

A systematic review of the management of hangman’s fractures

During the past 30 years various treatment protocols for hangmans fractures have been attempted. In order to guide the management of hangmans fractures, different classifications have been introduced. However, opinions on operative or nonoperative treatment have not yet been solidified. To evaluate both conservative and operative management of hangmans fractures in the published literature and to provide appropriate guidelines for treatment of hangmans fractures, a systematic review of the literature regarding the management of hangmans fractures was performed. An English literature search from January 1966 to January 2004 was completed with reference to treatment of hangmans fractures. The classification for treatment guidance from the literature was also reviewed. Regarding a primary therapy for hangmans fractures, there were 20 papers (62.5%) that advocated for a conservative treatment and 11 of the remaining 12 papers suggested that conservative treatment was suitable for some stable fractures. The classification of Effendi et al. modified by Levine and Edwards was used widely. Most hangmans fractures could be managed successfully with traction and external immobilization, especially in Effendi Type I, Type II and Levine-Edwards Type II fractures. It is necessary for Levine-Edwards Type IIa and III fractures to be treated with rigid immobilization. Only for some stable Type I and Levine-Edwards Type II injuries, nonrigid external fixation alone was sufficient. Rigid immobilization alone was necessary for most cases. Surgical stabilization is recommended in unstable cases when there is the possibility of later instability, such as Levine-Edwards Type IIa and III fractures with significant dislocation. The classification system proposed by Effendi et al. and modified by Levine and Edwards provided a clinically reasonable guideline for successful management of hangmans fractures.     

Anaesthetic considerations in the child with Menkes’ syndrome

The author presents and discusses the anaesthetic implications of a four-month-old infant with Menkes' syndrome who required tracheostomy. Menkes' syndrome is an X-linked recessive disorder of copper absorption and metabolism. Defective processing of copper results in abnormalities of several enzyme systems leading to severe dysfunction of multiple organ systems. Due to the progressive nature of this disorder and its severe effects on several different organ systems, most importantly the central nervous system, these children frequently require anaesthetic care during imaging procedures such as MRI or during various surgical operations. The high prevalence of seizure disorders, gastroesophageal reflux with the risk of aspiration, and airway complications related to poor pharyngeal muscle control are of concern to the anaesthetist. In addition, defective collagen formation, similar to that seen in Ehlers-Danlos syndrome, may be present. Identification of these associated conditions during the preoperative examination will guide the selection of appropriate, safe anaesthetic care for these children.     

Treatment on Nature’s lap: Use of herbal products in the management of hyperglycemia

Diabetes mellitus (DM) is characterized by persistently elevated blood glucose concentration that lead to multisystem complications. There are about 400 medicinal plants cited to have a beneficial effect on DM. We must choose products wisely based on data derived from scientific studies. However, a major obstacle in the amalgamation of herbal medicine in modern medical practices is the lack of clinical data on its safety, efficacy and drug interaction. Trials of these herbal products often underreport the side effects and other crucial intervention steps deviating from the standards set by Consolidated Standards of Reporting Trials. Due to a lack of knowledge of the active compounds present in most herbal medicines, product standardization is difficult. Cost-effectiveness is another issue that needs to be kept in mind. In this mini-review, we focus on the anti-hyperglycemic effect of herbal products that are commonly used, along with the concerns stated above.