PubMed Health⌕ Search

SEARCH · PubMed Health

Results for “MANDIBULOFACIAL DYSOSTOSIS”

Explore indexed PubMed citations for clinical trials, systematic reviews and public health research. Read source abstracts and follow each citation to its original PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 559 records · Page 31Linked to original sources
PubMed1989

Liver transplantation for hepatoblastoma in a child with congenital absence of the portal vein.

Congenital absence of the portal vein with systemic visceral venous return in a 8-year-old girl with oculoauriculovertebral dysplasia (Goldenhar Syndrome) had been previously reported following its discovery during preoperative evaluation of a liver mass which was diagnosed as focal nodular hyperplasia after open biopsy. Subsequently, an enlarging diffuse hepatic neoplasm developed with associated elevated alpha feto-protein levels. Repeat biopsy and imaging showed the tumor to be a hepatoblastoma involving both lobes of the liver. The patient was treated by hepatic resection and orthotopic liver transplantation and is doing well at 18 months follow-up.

Abnormalities, Multiple↗
PubMed1996

Mandibular distraction osteogenesis as first step in the early treatment of severe dysgnathia in childhood.

The sole orthodontic treatment of severe dysgnathias in childhood often leads to unsatisfactory results. On the other hand, standard surgical procedures are very difficult and due to their high risks not practicable in early childhood. The distraction osteogenesis enables us to correct hypoplastic mandibles, so that secondary malformations of the midfacial complex can be avoided. During the operation the hypoplastic site of the mandible is osteotomized behind the last visible tooth bud and a bidirectional distractor is inserted. Following the principles of Ilizarov the new callus is lengthened gradually until the required length of the mandible has been achieved. Out of a total sample of 27 patients 3 case reports of young children are presented. The new surgical concept describes new treatment perspectives.

Adolescent↗
PubMed1982

Congenital cardiac, pulmonary, and vascular malformations in oculoauriculovertebral dysplasia.

Three cases of oculoauriculovertebral dysplasia (OAV) with associated cardiac, pulmonary, and facial malformations are described. One patient had a rare condition: isolation of the left innominate artery. All 3 had pulmonary anomalies ranging from minor (incomplete lobulation) to major (hypoplasia of the left lung). When pulmonary hypoplasia occurs, it is usually on the same side of the body as the facial anomalies. Forty-five reported cases of OAV with associated cardiac, vascular, and pulmonary abnormalities are reviewed. Tetralogy of Fallot and ventricular septal defect account for half of the cardiac malformations. In the remaining cases, a wide variety of cardiovascular malformations are present. It is suggested that the facial malformations in OAV may result from a perfusion abnormality. An isolated left innominate artery (as in one of our cases) could produce abnormal vascular supply to the external carotid artery and the facial structures that it supplies.

Arteriovenous Malformations↗
PubMed1990

Respiratory inductance plethysmography and pulse oximetry in the assessment of upper airway patency in a child with Goldenhar's syndrome.

The anaesthetic management of a child with Goldenhar's syndrome and upper airway dysmorphology is presented. She had a history of severe dyspnoea due to deterioration of cor pulmonale caused by upper airway obstruction. The patency of the upper airway and oxygenation were evaluated during the perioperative period with respiratory inductive plethysmography (RIP) and pulse oximetry, which did not show severe upper airway obstruction or oxygen saturation below 80 per cent. Tracheal intubation was performed under inhalational anaesthesia with spontaneous breathing. This case suggests that RIP and pulse oximetry may be useful monitoring devices in the anaesthetic management of patients with upper airway problems as in Goldenhar's syndrome.

Anesthesia, Inhalation↗
PubMed1991

Anaesthesia for Treacher Collins syndrome using a laryngeal mask airway.

Patients with Treacher Collins syndrome pose a serious problem to anaesthetists in maintaining their airway because of retrognathia. Two patients with Treacher Collins syndrome undergoing tympanoplasty are reported in whom a laryngeal mask was used in place of an endotracheal tube for airway maintenance.

Anesthesia, Inhalation↗
PubMed1986

Anaesthesia for Treacher Collins and Pierre Robin syndromes: a report of three cases.

We present three patients with Treacher Collins or Pierre Robin syndromes who had historical and physical evidence of airway obstruction, difficulty feeding, and sleep disturbances. These preoperative findings correlated with difficult airway management intraoperatively. Based on this experience, we recommend that children with obstructive symptoms have laryngoscopy prior to anaesthetic induction. If the glottic opening is visualized, inhalational induction can proceed. If the glottic structures cannot be visualized, then the anaesthetist must choose between awake oral or nasal intubation, elective tracheostomy, or fiberoptic intubation. In all cases, a tracheostomy tray should be ready and a surgeon experienced in paediatric tracheostomy should be in attendance. After intubation, anaesthesia is best maintained with oxygen and a potent inhalational agent. Extubation should only be done with the patient fully awake and with emergency airway equipment immediately available. Postoperatively, these patients should be transferred to an intermediate care area or intensive care unit where they can be observed closely since delayed complications of airway obstruction are common in this group of patients.

Anesthesia↗