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At least 217 records · Page 12Linked to original sources
PubMed1996

Radiographic findings in Shprintzen-Goldberg syndrome.

We report the case of a Japanese boy whose dysmorphic features were consistent with those of Shprintzen-Goldberg syndrome. The radiological features were characterized by late-onset craniosynostosis, arachnodactyly, undermodeling of short tubular bones, mildly undermodeled and slightly bowed long bones, twisted ribs and tall vertebral bodies with elongated neural arches. Apart from the craniosynostosis, these skeletal changes resembled those of frontometaphyseal dysplasia, a well-known craniotubular dysplasia. Shprintzen-Goldberg syndrome also shares many clinical features with frontometaphyseal dysplasia.

Abnormalities, Multiple↗
PubMed1980

Mesomelic dysplasia with short ulna, long fibula, brachymetacarpy, and micrognathia. Clinical and radiological differential diagnostic features.

A girl with a "new" variant of mesomelic dysplasia is reported. The disorder is characterized by mesomelic brachymely, especially of the arms, a short ulna, relatively long fibula, brachymetacarpy, minor symmetrical changes at the hands, developmental arrest of the spine, contractures, and micrognathia. The clinical and radiological differential diagnostic features are discussed.

Bone Diseases, Developmental↗
PubMed1986

Dysphagia and micrognathia in a patient with juvenile rheumatoid arthritis.

This report describes the case of a 35-year-old man with juvenile rheumatoid arthritis. The patient experienced swallowing difficulties that were initially thought to be a result of the disease affecting the cervical spine. A maxillofacial examination, however, showed a total loss of the mandibular condyles, short rami and bodies, and a retrusion of the jaw. This was considered to be the main cause of the dysphagia, and not a glossopharyngeal nerve compression. The surgical-orthodontic management of these difficult cases is discussed.

Adult↗
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↗
PubMed1984

Hydrolethalus syndrome in consecutive African siblings.

Hydrolethalus syndrome may comprise mainly hydrocephalus, polydactyly, micrognathia, congenital cardiac and respiratory anomalies and uniform lethality. It was recently described in Finland, with a suggestion that it might be one of the "Finnish" diseases [4]. We report two cases of this syndrome in consecutive siblings of a Nigerian couple. In addition, we describe associated healing fractures of the long bones, a feature not mentioned in the original report, apparently because those cases were not subjected to post-partum radiography.

Adult↗
PubMed2000

[Mandibular hypoplasia. Growth induction by osteochondral transplants or callus distraction?].

In order to restore function and esthetics in mandibular hypoplasia, transplantation of osteochondral grafts and the distraction osteogenesis technique are used alternatively. In a retrospective study the indication, growth and complication rate of osteochondral graft and distraction osteogenesis cases in mandibular hypoplasia were compared. In total, 12 patients (4-14 years old; 7 female, 5 male) with different grades of mandibular hypoplasia received osteochondral grafts (7 costochondral, 5 iliac crest; observation period 4-9 years). In 5 patients (10-12 years old; 1 female, 4 male), distraction osteogenesis was performed (observation period 10 months to 3 1/2 years). In the results, according to the literature, the range of growth after osteochondral grafting varied considerably, approximate normal growth was noticed in only two cases. On the other hand, all patients treated by distraction osteogenesis showed a stable increase of bone length as expected. After osteochondral grafts, adequate growth is to be expected in the minority of cases and it does not seem predictable in individual cases. Therefore, the indication for osteochondral grafts should be restricted to severe hypoplasia, where the local amount of bone does not allow the use of a distraction device, and to cases where support of the mandibula at the skull base is necessary. In all other cases of mandibular hypoplasia in children, distraction osteogenesis should be discussed as the primary option, even if in the long term a second surgical procedure should be necessary.

Adolescent↗
PubMed2000

[CT-assisted volumetry and densitometry of distraction osteogenesis lengthened mandibular areas].

Gradual callus distraction in the correction of hypoplastic areas in the viscerocranial skeleton is an established method due to its efficacy. Whereas functional and esthetical improvements can be seen easily, it is impossible to get the increase of the bony mass right using the distractor scale or by clinical investigation. Only in animal studies can volumetric and densitometric measurements be done directly. By permission of the local ethics commission, in a clinical study, CT data of 24 children (12 unilateral and bilateral hypoplasias each) taken before and after mandibular distraction osteogenesis were used to get information on the amount of created bone and the grade of ossification of the callus at the time of the distractor removal. The CT data were prepared for computer evaluation using software which had been especially developed for this investigation. The quality of the investigation was influenced by disturbed data acquisition (insufficient sedation, Gantry deviation, different axial cuts). Therefore, the estimation of the real increase of bone was not possible, but all in all an increase of the volume could be seen. Using a grey scale analysis, the grade of new bone ossification was over 90% at the time of distractor removal. In our opinion, these results argue against the use of CT scans for the validation of a mandibular distraction. The estimation of the volume is very imprecise, the grade of ossification of the new bone very constant.

Absorptiometry, Photon↗
PubMed2003

Transversal palatal expansion using a palatal distractor.

METHOD AND RESULTS: The method and first results of transversal expansion with a palatal distractor in adolescents and adults with transverse maxillary deficiencies are presented. In ten patients with a mean age of 25.8 years, a newly developed distractor was applied for bone-borne expansion of the two halves of the maxilla following osteotomy of the lateral walls of the maxillary sinuses and the midpalatal suture. After a 3-week distraction period, mean changes of 8.8 mm in intercanine distance (ICD), 8.6 mm in anterior dental arch width (ADA), and 8.3 mm in posterior dental arch width (PDA) were registered. 6 months after the subsequent multibracket appliance therapy, these values were found to be largely constant. CONCLUSION: Because of the short treatment period, the absence of relapses, and the handling simplicity for the patient, this method is recommended for clinical application.

Adolescent↗
PubMed2000

Mandibulo-acral dysplasia.

We report on a 7 year-old-girl with mandibulo-acral dysplasia. When she was 3 years of age it mimicked scleroderma because of skin atrophy and later on a Hutchinson-Gilford progeria syndrome (HGP). Acro-mandibular dysplasia was diagnosed because of facial hypoplasia and mandibular hypoplasia. The bilateral proximal mid-humeral notch seen in this case is unusual.

Abnormalities, Multiple↗
PubMed2003

Pierre-Robin syndrome associated with Chiari type I malformation.

CASE REPORT: Pierre-Robin syndrome (PRS) is a rare congenital malformation that shows severe micrognathia and cleft soft palate. A 15-year-old boy who was admitted with occipital headache and gait disturbance was diagnosed with PRS. Radiological evaluation revealed severe herniation of the cerebellar tonsil and multiple craniovertebral osseous anomalies. We carried out foramen magnum decompression (FMD) with duroplasty. Postoperative MRI showed the cerebellar tonsil was freed from strangulation. RESULT AND CONCLUSION: This patient presented with the common finding between PRS and Chiari type I on the embryological aspect by illustrating pathophysiology of the Chiari I malformation. To our knowledge, this is the first reported case of PRS associated with Chiari malformation type I.

Adolescent↗
PubMed2006

[CT assisted mandibular osteodistraction with reconstruction of the temporomandibular joint and two piece maxilla].

BACKGROUND: The possibilities for computer assisted planning and its surgical conversion with stereolithographic templates are shown for complex maxillofacial surgical interventions. CASE REPORT: We describe the case of a patient with extreme micrognathia due to an ankylosis of the temporomandibular joint. In addition to the exact determination of the osteotomy lines on the mandibular angle and the segment osteotomy of the maxilla, vectors of the bidirectional osteodistraction were also predictable. CONCLUSION: In comparison with the conventional procedures, computer assisted planning achieved a much higher degree of precision and safety with reduced complication rates. The CMF module is a component of the SimPlant software which enables the precise planning of osteotomies and osteodistractions.

Adult↗
PubMed2006

Experience with the transpalatal distractor in congenital deformities.

The transpalatal distractor is a bone-borne device that should eliminate negative orthodontic side-effects of tooth-borne devices. The literature contains reports of several possible complications of the transpalatal distractor. In this retrospective study its use was evaluated in ten patients with various congenital craniofacial anomalies, including clefts. During placement of the transpalatal distractor it was noted that in extremely narrow maxillae the palatal bone is very thin, which makes the initial placement difficult and therefore less primary stability can be obtained. There is often a thick palatal mucosa, which makes placement of the abutment plates difficult and leaves hardly any space for the module itself. Finally, in patients with mental retardation it is difficult to exchange modules and re-fix abutment plates. In our group of ten patients with congenital deformities a 60% complication rate was observed. The transpalatal distractor seems not to be the ideal device for use in widening the maxilla in cases with congenital deformities.

Adolescent↗