Ectopic antral teeth: diagnosis and management implications.
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Adult↗
Biomedical subjects
J A Morkel
Publications and source records attributed to J A Morkel.
Crouzonodermoskeletal syndrome.
Crouzon craniostenosis [MIM 123500], is identified on the basis of the additional phenotypical manifestations of acanthosis nigricans, vertebral changes and cementomas of the jaws. Choanal atresia and hydrocephalus are other features. The molecular defect in CDSS is a point mutation in the FGFR3 gene on chromosome 4p, whereas, the mutation in the Crouzon syndrome is in the FGFR2 gene at 10q25.3-26. An affected girl aged 2 years presented at the UWC dental genetics unit with a prior diagnosis of Crouzon syndrome. Choanal atresia had necessitated a permanent tracheostomy, and hydrocephalus was managed by a shunt operation. Clinical examination revealed acanthosis nigricans in the axilliary regions, a diagnosis confirmed by a biopsy of the lesion. Eruption of the primary dentition was delayed with only six out of twenty teeth present. Radiographic examination conducted shortly after birth revealed the presence of several tooth buds in both the maxillae and the mandible. The delayed eruption of the teeth will be of significance in future orthodontic and maxillofacial measures for the improvement of the patient's facial Crouzonodermoskeletal syndrome (CDSS) was separated from the classical appearance. Molecular investigations in the girl and her parents are underway. If the specific mutation in FGFR3 is observed, a positive diagnosis of CDSS will be confirmed and the status of her parents and other family members will be determined. On this basis, appropriate genetic management can be offered to the kindred.
Acanthosis Nigricans↗
Anesthesia for temporomandibular arthroplasty in a quadriplegic patient: a case report.
A 41-year-old male quadriplegic patient with bilateral TMJ ankylosis was admitted for surgery under general anesthesia. Preoperative tests showed abnormal lung function and a low total serum calcium level. Premedication consisted of diazepam 10 mg orally and glycopyrrolate 0.2 mg intramuscularly 90 minutes before the start of the operation. Because of an inability to open the mouth more than 0.5 cm, a nasal fiberoptic endoscopic intubation was done under sedation and local anesthesia. For maintenance of anesthesia the patient received nitrous oxide 50% in oxygen, halothane, and alfentanil. No muscle relaxant was administered. The 2-hour operation was completed uneventfully and the patient needed no ventilatory support afterward. Major anesthetic problems of spinal injury patients can be compared to those with cervical cord transection. The stage that the condition has reached determines the dominant anesthetic problems. The chronic stage (greater than 3 months postinjury, as in this patient) is usually characterized by sympathetic overactivity. Anesthetic problems during this stage may include risk of hyperkalemia from succinylcholine, cardiovascular instability and autonomic hyperreflexia, impaired thermoregulation, anemia, chronic infections, risk of hypercalcemia, and alveolar hypoventilation. None of these problems was encountered in this patient. A surprising finding was that the low serum calcium concentration did not influence the anesthetic outcome.
Adult↗