Cases reported "Achondroplasia"

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1/7. Combined spinal epidural anaesthesia for vesico-vaginal fistula repair in an achondroplastic dwarf.

    A 33-year-old achondroplastic female was scheduled to undergo vesico-vaginal fistula repair by the abdominoperineal route. Preoperative examination suggested a difficult airway so a combined spinal epidural technique was used. Subarachnoid block (sensory loss to T6) was established using 0.5% hyperbaric bupivacaine 1 ml. Anaesthesia was prolonged with incremental doses of epidural bupivacaine 0.5% (total 10 ml) and postoperative analgesia was provided with epidural morphine boluses.
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keywords = anaesthesia
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2/7. Anaesthesia for dwarfs and other patients of pathological small stature.

    Sixty-nine anaesthetics were administered to 29 patients of pathological proportionate and disproportionate small stature. The anaesthetic course in most cases was uncomplicated. The few complications noted were similar in type and severity to those found in normal size patients undergoing similar anaesthesia and operative procedures. Achondroplastic dwarfs often develop neurological problems due to their bony deformities. General anaesthesia should be given preferential consideration in these patients. Non-achondroplastic dwarfs may have an associated odontoid dysplasia and if the neck is placed in flexion there is a potential risk of spinal cord damage. Tube size for proportionately small children is best estimated from body weight. No definite recommendations concerning proper tybe size in dwarfs can be given on the basis of the findings in the study.
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ranking = 0.5
keywords = anaesthesia
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3/7. Epidural anaesthesia for caesarean section in an achondroplastic dwarf.

    This report describes the anaesthetic management of an 18-yr-old achondroplastic dwarf who presented for elective Caesarean section. Epidural anaesthesia was performed without technical difficulty using 8 ml carbonated lidocaine 2% with epinephrine 1:200,000. Although the skeletal abnormalities of achondroplasia have been cited as contraindications to the use of epidural anaesthesia, clinical experience does not support this contention. Previous reports have described technical difficulties in these patients, such as dural puncture and inability to advance the catheter into the epidural space, but no serious complications resulted and epidural anaesthesia was successful on subsequent attempts. The existing literature on the anaesthetic management of achondroplasia for Caesarean section is reviewed and considerations are presented concerning the choice of local anaesthetic, the epidural test dose, and dose titration.
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ranking = 1.75
keywords = anaesthesia
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4/7. pregnancy in an achondroplastic dwarf: a case report.

    achondroplasia is a rare disorder occurring 1 in 1 5,000 to 1 in 40,000 live births. It is, however, the commonest cause of short-limbed dwarfism. It is a genetic disorder and inherited as an autosomal dominant trait but most cases (80%) are due to mutations of fibroblast growth factor receptor 3 (FGFR3). These individuals have normal mental and sexual development, and life span may be normal. Certain gynaecological problems like infertility, menorrhagia, dysmenorrhoea, leiomyomata and early menopause are more common in these patients. Information regarding obstetric behaviour in achondroplastic females is scarce in literature. However, problems such as pre-eclampsia, polyhydramnios, respiratory compromise, contracted pelvis necessitating lower section caesarean section, prematurity and foetal wastage, etc, have been reported. General anaesthesia is preferred to regional anaesthesia because of the spinal abnormalities. There is increased neonatal mortality due to hydrocephalus and thoracic cage abnormality. Such a patient is considered high risk in terms of anaesthesia and obstetric outcome and there is enough room for prenatal counselling and diagnosis. Here a case of achondroplasia with pregnancy is reported. The patient, an achondroplastic dwarf presented with 30 weeks pregnancy. She was prenatally screened with ultrasonography to rule out affection in baby. She had a caesarean section for contracted pelvis.
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ranking = 0.75
keywords = anaesthesia
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5/7. Anaesthesia in a gravid achondroplastic dwarf.

    A patient with achondroplasia presented for elective Caesarean section under epidural anaesthesia. A block from C5 to S4 developed over 20 minutes after 12 ml plain bupivacaine 0.5%. This case serves to highlight the difficulties of regional anaesthesia in the gravid achondroplastic dwarf.
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ranking = 0.5
keywords = anaesthesia
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6/7. Extradural anaesthesia for caesarean section in achondroplasia.

    We describe the successful management of a 26-yr-old achondroplastic dwarf undergoing elective Caesarean section under extradural anaesthesia. The patient had marked thoracolumbar kyphoscoliosis and clinical features which suggested that tracheal intubation would prove difficult. Block sufficient for surgery required only 5 ml of 0.5% bupivacaine and, apart from an initial unilateral block and mild intraoperative hypotension, her perioperative course was uneventful.
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ranking = 1.25
keywords = anaesthesia
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7/7. Epidural anaesthesia for caesarean section in an achondroplastic dwarf.

    We describe the anaesthetic management of a parturient with achondroplasia presenting for Caesarean section under epidural anaesthesia. A block extending from T4 to S4 was established over 25 min using a total of 12 ml of 2.0% lidocaine (lignocaine) with epinephrine (adrenaline) 1:200,000 and fentanyl 37.5 micrograms. Apart from mild discomfort during peritoneal incision, her perioperative course was uneventful. achondroplasia is reviewed and the anaesthetic implications of the condition are discussed.
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ranking = 1.25
keywords = anaesthesia
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