1/22. Sprengels deformity: anaesthesia management.A 28 years old lady presented with Sprengels deformity and hemivertebrae for Fothergills surgery. Clinically there were no anomalies of the nervous, renal or the cardiovascular systems. She had a short neck and score on modified Mallapati test was grade 2. She was successfully anaesthetised using injection propofol as a total intravenous anaesthetic agent after adequate premedication with injection midazolam and injection pentazocine. Patient had an uneventful intraoperative and postoperative course.- - - - - - - - - - ranking = 1keywords = anaesthesia (Clic here for more details about this article) |
2/22. Intraspinal haematoma following lumbar epidural anaesthesia in a neonate.A neonate with chromosomal 9 abnormality and omphalocele received a lumbar epidural catheter after laparotomy. Several attempts were needed to establish this catheter. Bleeding occurred from the operative wound after surgery. Using an epidural infusion with ropivacaine 0.1% for 48 h postoperative pain relief was sufficient. Four days after epidural catheter removal, dysfunction of the sacral parasympathetic nerves was noted. Motor and sensor function of the lower limbs were unaffected. magnetic resonance imaging showed a localized intraspinal haematoma in the lower lumbar region.- - - - - - - - - - ranking = 1keywords = anaesthesia (Clic here for more details about this article) |
3/22. 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.- - - - - - - - - - ranking = 0.5keywords = anaesthesia (Clic here for more details about this article) |
4/22. 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.- - - - - - - - - - ranking = 0.25keywords = anaesthesia (Clic here for more details about this article) |
5/22. 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.- - - - - - - - - - ranking = 0.25keywords = anaesthesia (Clic here for more details about this article) |
6/22. 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.- - - - - - - - - - ranking = 1keywords = anaesthesia (Clic here for more details about this article) |
7/22. scoliosis in Steinert syndrome: a case report.BACKGROUND CONTEXT: Steinert syndrome is described as an autosomal dominant condition characterized by progressive muscular wasting, myotonia, musculoskeletal manifestations and rare spinal defects. Little is reported about spinal deformity associated with this syndrome. PURPOSE: We present a patient with Steinert syndrome complicated by scoliosis. In the literature on muscular dystrophy, other than Duchenne, little mention is given to the problem of scoliosis in general and its treatment in particular. STUDY DESIGN: A case report of a patient with Steinert syndrome associated with thoracic scoliosis and hypokyphosis is presented. methods: A 17-year-old boy presented with King type II right thoracic scoliosis (T5-T11, Cobb angle of 40 degrees) and hypokyphosis--10 degrees. He was treated with posterior stabilization and instrumentation at level T3-L2 with a postoperative correction of the scoliotic curve to 20 degrees. Histopathologic examination of the muscles confirmed the diagnosis of Steinert myotonic dystrophy. RESULTS: At 30-month follow-up, the patient was clinically pain free and well balanced. Plain radiographs showed solid spine fusion with no loss of deformity correction. CONCLUSIONS: scoliosis in Steinert syndrome shares the characteristic of an arthrogrypotic neuromuscular curve and demands the extensive soft tissue release for optimal surgical correction. Intraoperative observations included profound tissue bleeding, abnormally tough soft tissues and a difficult recovery from anaesthesia.- - - - - - - - - - ranking = 0.25keywords = anaesthesia (Clic here for more details about this article) |
8/22. Anaesthesia for proteus syndrome.A 14-year-old boy with proteus syndrome presented for orthopaedic surgery to his legs. No report in the literature exists on anaesthesia for this condition. Our patients posed airway problems that were managed by tracheal intubation under sedation using a fibreoptic bronchoscope. Anaesthesia was induced with thiopentone and maintained with nitrous oxide and isoflurane in oxygen. His lungs were ventilated mechanically throughout surgery, which was uneventful.- - - - - - - - - - ranking = 0.25keywords = anaesthesia (Clic here for more details about this article) |
9/22. Congenital corneal anaesthesia and the MURCS association: a case report.Bilateral corneal anaesthesia and reduced sensation in the distribution of the ophthalmic division of the fifth cranial nerve associated with neuroparalytic keratitis and reduced vision is described in a 26-year-old woman with absence of the fallopian tubes, uterus, and upper vagina, a single pelvic kidney, and cervico-thoracic vertebral anomalies (the MURCS association). Other features of the disorder also present were short stature, facial asymmetry, micrognathia, and cleft palate. The neuroparalytic keratitis did not respond to tear substitutes and bilateral lateral tarsorrhaphies were eventually needed. This is the first report of congenital corneal anaesthesia in a case of MURCS.- - - - - - - - - - ranking = 1.5keywords = anaesthesia (Clic here for more details about this article) |
10/22. The anaesthetic management of an infant with frontometaphyseal dysplasia (Gorlin-Cohen syndrome).We describe an infant with frontometaphyseal dysplasia, who presented to us twice for anaesthesia for orthopaedic surgery. These patients have facial asymmetry, mandibular hypoplasia, bradycardia, restrictive lung disease, primary pulmonary hypertension, skeletal abnormalities and difficult endotracheal intubation. The patient also showed laryngeal stridor because of laryngomalacia, vocal cord paralysis and subglottic stenosis. light premedication along with atropine, ECG and blood pressure monitoring, gradual inhalational induction and intubation of the spontaneously breathing patient, careful positioning and postoperative CPAP are recommended.- - - - - - - - - - ranking = 0.25keywords = anaesthesia (Clic here for more details about this article) |
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