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Torstein R Meling

Publications and source records attributed to Torstein R Meling.

4 recordsLinked to original sources
PubMed2004

[Complex craniofacial synostoses].

BACKGROUND: Complex craniofacial synostosis is a group of rare genetic disorders characterized by premature closure of the sutures in the craniofacial skeleton and which to varying degrees affects the extremities. MATERIAL AND METHODS: On the basis of relevant literature, we present a review of syndromal craniofacial synostosis. RESULTS: Phenotypically, the complex craniofacial syndromes have many similarities. Synostosis of the sutures of the cranial vault can result in a variety of skull deformations, depending on the sutures involved, the sequence of premature closure, and the time of closure. Synostosis of the sutures in the skull base and facial skeleton leads to shallow orbits, exophthalmus, hypertelorism, midface retrusion, and prognathia. INTERPRETATION: Precise diagnosis of complex craniofacial syndromes may be difficult solely on the basis of a clinical examination. However, several of the most common syndromes are caused by mutations in genes that code for fibroblast growth-factor receptors. Children with a suspected complex craniofacial syndrome should be referred to genetic testing.

Acrocephalosyndactylia↗
PubMed2004

Monobloc distraction osteogenesis in pediatric patients with severe syndromal craniosynostosis.

The management of the hypoplastic midface in syndromic craniosynostosis remains a great challenge. Frequently, patients have to be operated on numerous times to achieve a satisfactory end result, partially because of the limited skeletal advancement possible when using traditional surgical techniques. During the last decade, however, methods for gradual midfacial distraction have been presented, whereby greater advancements can be obtained. We present four children aged 17 months to 15 years with severe syndromal craniosynostosis in need of midface advancements because of severe respiratory obstruction or severe exophthalmos. These patients were complex cases with several previous craniofacial surgeries (mean of three times, range of two to six times) that yielded insufficient skeletal advancements. They were operated on with gradual monobloc advancements using the Modular Internal Distraction System. The mean length of operations was 370 minutes (range: 240-455 minutes), and the mean amount of perioperative blood transfusion needed was 1,300 ml (range: 280-2,700 ml) or 66.9 ml/kg (range: 31.1-94.9 ml/kg). The patient with the greatest number of previous operations also had the longest operation time as well as the most blood loss. The average midface advancement obtained was 25 mm (range: 20-30 mm), resulting in cessation or a significant decrease of preoperative respiratory problems, reduced exophthalmos, and improved facial profile. Apart from a local infection in one patient with a connective tissue disorder and several previous wound infections, no major postoperative complications were recorded. Distraction osteogenesis has become a versatile and safe technique that allows for large advancements of the midface.

Adolescent↗
PubMed2002

[Occult spinal dysraphism].

BACKGROUND: Spinal dysraphism is a group of congenital anomalies of the spine characterized by a midline defect affecting the nervous tissue and its bony and soft tissue coverings. Closed spinal dysraphism, spina bifida occulta, refers to skin-covered lesions. However, there are a number of cutaneous stigmata or other physical signs that serve as indicators of an underlying dysraphic malformation of the lower spinal canal. MATERIAL AND METHODS: Based upon relevant literature, we present a review of occult spina bifida. RESULTS AND INTERPRETATION: The cutaneous stigmata that may indicate an underlying dysraphism are particularly hairy patches, subcutaneous lipomas, capillary hemangiomas, dorsal dermal sinuses and sacral cutaneous pits. The clinical examination of a child with lumbosacral cutaneous stigmata should focus on neurological signs and symptoms, urological disturbances and orthopaedic deformities in the lower limbs. Spinal MRI is the imaging modality of choice in children presenting with features consistent with occult spinal dysraphism. Children with occult spinal dysraphism should be referred to a neurosurgical unit.

Child↗