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Biomedical subjects

C Mahatumarat

Publications and source records attributed to C Mahatumarat.

5 recordsLinked to original sources

Mandibular distraction osteogenesis in unilateral craniofacial microsomia: preliminary report.

At present, Ilizarov's distraction principle becomes applicable in craniofacial surgery. We would like to present a report of mandibular lengthening by distraction osteogenesis that has been performed in 4 Thai children with unilateral craniofacial microsomia in King Chulalongkorn Memorial hospital from 1996 to 1997. The distraction process was composed of latency, distraction, and consolidation phases. After the latency period, the distraction was performed by a patient's family member at home at the rate of 1 millimeter per day. Facial asymmetry and malocclusion were improved in all cases after the process was completed. No complication was experienced. No relapse or complication was detected after a mean follow-up period of 99.5 weeks. However, more cases and longer follow-up are needed before any conclusion can be made.

Adolescent↗

A case of Pfeiffer syndrome type 1 with an A344P mutation in the FGFR2 gene.

Pfeiffer syndrome, an autosomal dominant disorder, consists of craniosynostosis, broadening of the thumbs and great toes, and partial soft tissue syndactyly of the hands and feet. Three clinical subtypes have been classified mainly for the purpose of genetic counseling. Mutations in FGFR1 and FGFR2 are known to be associated with the syndrome. However, the correlation between genotype and phenotype is not well defined. Only one patient with Pfeiffer syndrome with no other clinical information has been reported to have had an A344P mutation of the FGFR2. Here we report a Thai male patient with sporadic Pfeiffer syndrome type 1 with impaired intelligence (IQ = 77). Mutation analysis revealed A344P in FGFR2. Identification of the clinical features and molecular defects in more patients is required to better correlate the genotype and phenotype of this complex syndrome.

Acrocephalosyndactylia↗

Spontaneous closure of bony defect in a frontoethmoidal encephalomeningocele patient.

The frontoethmoidal encephalomeningocele (FEEM) is a congenital herniation of meninges and brain tissue through the skull bony defect at the foramen cecum. The size of the defect may vary from a few millimeters to many. Those patients with a small defect may not always require a risky operation during childhood. We report on an infant whose bony defect has closed spontaneously with definite clinical evidence. It is proved that the skull defect and brain herniation are able to heal naturally, and this affirms an existence of the abortive subtype of FEE. Conservative treatment may be considered in those with a small bony defect, and surgery can be considered later when it is required.

Child↗

One-stage extracranial repair and reconstruction for frontoethmoidal encephalomeningocele: a new simple technique.

Forty-five patients born with frontoethmoidal encephalomeningoceles were treated using the craniofacial technique of one-stage extracranial repair and reconstruction. The operation begins with a bicoronal scalp flap, involving frontonasosuperomedial orbital wall osteotomy, reduction of the interorbital distance by nasal bone segment removal, hernial sac amputation and dural repair, medial orbital wall mobilization, medial canthopexy, and rib augmentation rhinoplasty. The result was very satisfactory. This new method has undoubtedly contributed in a major way to the improved results in frontoethmoidal encephalomeningocele treatment. We believe that the main advantages of this technique are that it offers a simple procedure for simultaneous correction of both soft tissue and bony deformities. The direct and external access to the neck of the hernial sac renders more secure dural repair with almost negligible cerebrospinal fluid leakage and eventually none of the postoperative brain sequelae. The postoperative course is less eventful and requires a shorter hospital stay than previous procedures.

Adult↗

Hemifacial microsomia: a multisystem classification.

Variability of deformities in hemifacial microsomia has precluded the general acceptance of any classification based on one reference organ. We present a review of hemifacial microsomia classifications and propose a TNM-style multisystem classification. This alphanumeric coding system, SAT, provides cohesion to existing hemifacial microsomia classifications. The acronym SAT is derived as follows: S = skeletal, A = auricle, and T = soft tissue. There are five levels of skeletal deformity (S1 through S5), four levels of auricular deformity (A0 through A3), and three levels of soft-tissue deformity (T1 through T3). Hence a patient with minimal deformity would be classified S1A0T1, whereas a patient with the most severe deformity would be S5A3T3.

Ear, External↗