1/5. Anaesthesia in a child with autosomal recessive omodysplasia.A child with a recently described form of dwarfism, autosomal recessive omodysplasia, presented for general anaesthesia for dental conservation. Despite the patient's dysmorphic features and craniofacial and mandibular deformities, no significant anaesthetic problems were encountered. Anaesthetic implications of the condition are reviewed.- - - - - - - - - - ranking = 1keywords = anaesthesia (Clic here for more details about this article) |
2/5. 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.- - - - - - - - - - ranking = 2keywords = anaesthesia (Clic here for more details about this article) |
3/5. 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.- - - - - - - - - - ranking = 7keywords = anaesthesia (Clic here for more details about this article) |
4/5. The dentofacial features of Sanjad-Sakati syndrome: a case report.Sanjad-Sakati syndrome is an autosomal recessive disorder that was first reported by Sanjad et al. in 1988. It is characterized by congenital hypoparathyroidism, severe growth failure and dysmorphic features. The clinical features include deep set eyes, microcephaly, thin lips, depressed nasal bridge with beaked nose, external ear anomalies and learning difficulties. This report presents the case of a 4-year-old patient who was referred to our dental clinic because of pain in her mouth and poor dental health. Oral findings included micrognathic mandible and maxilla, microdontia, enamel hypoplasia as well as severely decayed teeth. Treatment was carried out under general anaesthesia to extract the most severely affected teeth and restore those which could be conserved. It was concluded that these patients have special dental needs; early diagnosis of the affected children is therefore important in order to commence preventive dental therapy and carry out appropriate dental treatment at the optimum time.- - - - - - - - - - ranking = 1keywords = anaesthesia (Clic here for more details about this article) |
5/5. 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.- - - - - - - - - - ranking = 3keywords = anaesthesia (Clic here for more details about this article) |