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1/55. Anaesthetic management of two paediatric patients with Hecht-Beals syndrome.

    We undertook the anaesthetic management of two children with Hecht-Beals syndrome for orthopaedic surgery under general anaesthesia. Both patients had arachnodactyly, kyphoscoliosis, and multiple congenital joint contractures, but limited mandible excursion was not obvious preoperatively in either, although mental retardation made it difficult for them to cooperate with mouth examination. They had no apparent difficulties with their mouths in daily activities. The anaesthesia records of one patient showed that intubation had been difficult in an earlier procedure. The other patient also had a history of difficult intubation, with slight tearing of the corners of her mouth during an intubation procedure. During slow induction of general anaesthesia with sevoflurane, face mask ventilation was easily performed. We attempted to visualize the larynx under anaesthesia with muscle relaxation, but we were unsuccessful because of the limited mouth opening. After several trials, blind oral intubations were fortunately successful in both patients. There were no postoperative problems with the airway.
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2/55. The dental management of a child with leopard syndrome.

    This report describes the dental management of a child with leopard syndrome who presented with multiple grossly carious primary teeth. comprehensive dental care was carried out under general anaesthesia.
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ranking = 0.25
keywords = anaesthesia
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3/55. Post-apopletic trigeminal trophic syndrome.

    Trigeminal trophic syndrome is an uncommon clinical entity in which cutaneous trophic ulceration develops with continuous manipulation of trigeminal dermatomes. patients spontaneously refer picking, rubbing and/or scratching at the affected areas because of hypo-anaesthesia, paraesthesia and/or pain following damage of the sensory trigeminal fibres or nuclei. We herein describe a patient who developed the syndrome as a sequela of brain stem infarction. diagnosis by scrape cytology in ruling-out basal cell carcinoma and other ulcerative skin diseases is discussed and the importance of neurological examination in disclosing hemi-anaesthesia of trigeminal dermatome(s) is emphasized.
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ranking = 0.5
keywords = anaesthesia
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4/55. Undiagnosed cardiomyopathy in a neonate: significance of low oxygen saturation during anaesthesia.

    A case study is described of a 7-day-old full term baby with bilateral congenital cataracts who underwent surgical removal of both cataracts 2 days apart. Problems with oxygen saturation during and after the first anaesthetic prompted further investigation that revealed a non-obstructive hypertrophic cardiomyopathy. The significance and possible causes of low oxygen saturation in a previously healthy neonate during anaesthesia are discussed. The likely diagnosis of Sengers syndrome, and the evaluation of asymptomatic babies with cardiac pathology are discussed.
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ranking = 1.25
keywords = anaesthesia
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5/55. Anaesthetic management of splenectomy in Evan's syndrome during pregnancy with pregnancy induced hypertension.

    The management of idiopathic thrombocytopenic purpura (ITP) during pregnancy, especially with ongoing bleeding diathesis, has not been highlighted sufficiently in the literature. Aortocaval compression and reduction in uteroplacental circulation resulting in foetal hypoxia and acidosis, Mendelson's syndrome due to gravid uterus, trauma to airway with resultant haemorrhage and aspiration into lungs, compromised airway due to short neck, anasarca and heavy breast, limitation in using invasive monitoring and regional anaesthesia and uncontrolled bleeding leading to placental hypoperfusion and foetal hypoxia are some of the important risks. In the present case report, anaesthetic management for splenectomy during pregnancy complicated with pregnancy induced hypertension and bleeding diathesis secondary to ITP is described with reference to above risks.
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ranking = 0.25
keywords = anaesthesia
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6/55. Bilateral massive choroidal hemorrhage secondary to Glanzmann's syndrome.

    Characteristics of intraocular bleeding and its management in association with blood dyscrasias are discussed. We present a patient with massive bilateral choroidal hemorrhage secondary to Glanzmann's syndrome. magnetic resonance imaging and ultrasonographic findings were ordered. During the clinical course, bilateral intravitreal hemorrhage and tractional retinal detachment occurred. Left pars plana vitrectomy was performed under general anaesthesia. The procedure was unsuccessful because of intraoperative uncontrolled bleeding.
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ranking = 0.25
keywords = anaesthesia
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7/55. Two cases of medically and surgically intractable SUNCT: a reason for caution and an argument for a central mechanism.

    We report two cases of SUNCT that demonstrate the medically and surgically refractory nature of this disorder and support the hypothesis that the causative 'lesion' lies within the central nervous system. After both patients had failed medical therapies, the first underwent a glycerol rhizotomy, gammaknife radiosurgery and microvascular decompression of the trigeminal nerve. The second patient underwent gammaknife radiosurgery of the trigeminal root exit zone and two microvascular decompression surgeries. Neither patient benefited from these procedures. Currently, the first patient suffers from anaesthesia dolorosa and the second patient from unilateral deafness, chronic vertigo and dysequilibrium as a result of surgical trauma. These cases of SUNCT highlight the uncertainty regarding the role of surgery given the potential for significant morbidity. These cases also suggest that SUNCT originates and may be maintained from within the CNS and this central locus explains why SUNCT is not typically amenable to interventions aimed at the peripheral portion of the trigeminal nerve.
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ranking = 0.25
keywords = anaesthesia
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8/55. Intra-ureteric capsaicin in loin pain haematuria syndrome: efficacy and complications.

    OBJECTIVE: To evaluate the safety and efficacy of intra-ureteric capsaicin for loin pain haematuria syndrome (LPHS). patients AND methods: In an open prospective pilot study, four middle-aged patients (three women and one man) with LPHS resistant to therapies such as splanchnic nerve block, psychological treatment or renal autotransplantation (one) were assessed. An intra-ureteric infusion of capsaicin (30 mg/100 mL of 30% alcohol in saline) for 30 min with bladder irrigation was administered under general anaesthesia, with a subsequent intravenous patient-controlled narcotic analgesic pump for pain control. Double-concentration capsaicin was used for second infusions, if necessary when the response to the earlier infusion was inadequate or incomplete. RESULTS: The first patient had experienced reduced pain levels for the first 3 months only, with no benefit from the subsequent treatments with higher doses of capsaicin (60 mg). The second patient with recurrent pain in an autotransplanted kidney had no benefit from either a 30 or 60 mg capsaicin infusion a month apart, but developed a fibrotic stricture at the transplant pelvi-ureteric junction, requiring pyelocystoplasty. The third patient with concurrent depression had no benefit from a 30-mg infusion of capsaicin. The fourth patient experienced no pain relief from a 30 mg infusion of capsaicin but developed proteinuria secondary to mesangial proliferative glomerulonephritis, ureteric inflammation needing stenting within 7 days of treatment and subsequently nephrectomy for a nonfunctioning kidney at 3 months. CONCLUSION: Intra-ureteric capsaicin was neither effective nor safe in LPHS; the contribution of the alcohol diluent cannot be excluded.
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ranking = 0.25
keywords = anaesthesia
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9/55. Anaesthetic management in McKusick-Kaufman syndrome.

    The cardinal features of McKusick-Kaufman syndrome (MKS) are polydactyly and hydrometrocolpos. Sometimes, this abdominopelvic mass may restrict pulmonary function and decrease the oxygenation of the newborn. We present a case of MKS and review the clinical features and appropriate anaesthetic management. A 45-day-old girl, weighing 4450 g, born at term, presented to our Paediatric Surgery Department with abdominal distention, diarrhoea and vomiting, which started on the 15th day of life. On physical examination, peripheral cyanosis, tachycardia and tachypnoea were found. A prominent mass was palpated in the lower abdomen. There was vaginal atresia and a sixth digit was found on her left foot. There was a large cystic mass extending from the pelvis to the level of L1 displacing the diaphragm, and bilateral hydroureteronephrosis were seen on plain abdominal X-ray, ultrasonography and computerized tomography. In the preanaesthetic assessment, the patient was evaluated for multiple congenital anomalies. Our management of anaesthesia, for a neonate with severe hydrometrocolpos compressing the diaphragm, consisted of gastric decompression and preoxygenation before tracheal intubation, routine and airway pressure monitoring, periodic analyses of blood gases and maintenance of anaesthesia with a low concentration of volatile agent, together with an opioid.
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ranking = 0.5
keywords = anaesthesia
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10/55. Anaesthetic implications of costello syndrome.

    costello syndrome is a disorder that primarily involves ectodermal tissues and is characterized by mental and growth retardation, distinctive coarse facies, redundant skin (neck, palms and soles), and papillomata (perioral, nasal and anal). Of primary concern to anaesthesiologists are potential airway difficulties related a short neck, macroglossia, hypertrophied tonsillar and supraglottic tissues, laryngeal papillomata and choanal atresia. A significant percentage of patients also have cardiac involvement which may manifest as congenital heart defects, arrhythmias, valvular dysfunction, or hypertrophic cardiomyopathy. central nervous system involvement includes developmental delay and seizure disorders while endocrine abnormalities have been reported including hypothalamic-pituitary dysfunction resulting in hypopituitarism, hypothyroidism, and hypoadrenalism. The authors present a 2-year old child with costello syndrome who required anaesthesia for direct laryngoscopy, rigid bronchoscopy, bilateral pressure equalization tubes and tonsillectomy. The perioperative implications of the syndrome are discussed.
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ranking = 0.25
keywords = anaesthesia
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