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At least 1,063 records · Page 59Linked to original sources
PubMed1995

Transcranial Doppler analysis of cerebral hemodynamics in primary craniosynostosis: study in progress.

BACKGROUND: Regional elevations in intracranial pressure (ICP) have been monitored adjacent to areas of cranial stenosis by some authors [9,19,20]; however, noninvasive techniques such as transcranial Doppler (TCD) would be preferable if increased ICP could be detected just as reliably. METHODS: TCD examinations of basal cerebral arteries were performed in 31 children with skull deformities. Skull X rays and computed tomography (CT) scans or magnetic resonance imaging (MRI) were analyzed for degree of cranial vault abnormality, as well as for presence or absence of intracranial pathology. Neurodevelopmental examination and clinical signs of increased ICP, if any, were recorded. RESULTS: The overall incidence of increased resistivity indices (RI) was 70%. Patients with asymmetric skull deformities (plagiocephaly) or sagittal synostosis (scaphocephaly) were statistically more prone to exhibit increased RI compared to previously reported prevalences. CONCLUSIONS: Because TCD is a convenient, rapid, accurate, and noninvasive method by which to gauge ICP, it should be considered in the preliminary work-up of a patient with craniosynostosis.

Craniosynostoses↗
PubMed1990

Congenital hand anomalies in Japan: a family study.

During the 20-year period from 1968 to 1987, 1024 patients with congenital anomalies of the hand from 1000 Japanese families were examined in our clinic. Of these patients, 204 had relatives with limb anomalies. While postaxial polydactyly showed a 33% level of familial recurrence, symphalangism and Kirner's syndrome showed about 23% recurrence, syndactyly 18%, radioulnar synostosis 9%, radial ray deficiency 8%, cleft hand 7%, and duplicated thumb 5%. None of the relatives had ulnar deficiency or symbrachydactyly.

Hand Deformities, Congenital↗
PubMedDate not supplied

Identification of the cranium of W.A. Mozart.

In 1801 at the cemetery in Vienna, Austria, the skull of W.A. Mozart was exhumed (La Chronique Médicale, 13 (1906) 423), and now it has been examined for identification. The osteometrical and osteological findings correspond with the available data of W.A. Mozart. Superimposition gives evidence that craniofacial distinctiveness of the cranium is consistent with the portrait. Additional individual particularities caused by the premature synostosis of the metopic suture (PSMS) and a bone lesion are described.

Austria↗
PubMed1989

Spontaneous intercostal pulmonary hernia with subsegmental incarceration.

A case of acute intercostal pulmonary herniation due to vigorous coughing secondary to chronic bronchitis is reported in a 70-year-old male. Protruding pleura-covered lung tissue was found bulging through an intercostal space defect between the left midaxillary line and the infrasternal costochondral arch. A hernial sac consisting of parietal pleura and atrophic intercostal muscle confined a "sliding pouch" for two pulmonary segments of which one presented a demarcation zone of temporary incarceration interpreted as an entrapment of lung tissue between two ribs. A fracture gap was discovered affecting the anterior synostosis between ribs 7 and 8. Treatment was accomplished by anterior fixation of the ribs and by the basic principles of hernia repair between adjacent ribs. A case report and a brief survey of aetiological and anatomical classification is presented.

Aged↗
PubMed2005

A variant of Cenani-Lenz syndactyly (CLS): review of the literature and attempt of classification.

An oligodactylous variant of Cenani-Lenz syndactyly and its surgical treatment is presented. The deformity is believed to be of autosomal recessive inheritance and caused by a disordered axial and longitudinal differentiation of the upper and lower extremities. The classical form is mainly characterised by a complete syndactyly of the hands. Malformations may also affect the bones of the forearm and, to a lesser extent, the lower limbs. We analysed clinical features and compared them with those previously described. According to our research of literature and our clinical findings there seem to exist two grossly different clinical phenotypes: spoon hand type and oligodactyly type. Typical constant clinical features such as carpal, metacarpal and digital synostoses, disorganisation of carpal bones, reduction of digital rays and syndactyly of toes are found in the reported cases. Inconstant features such as radio-ulnar synostosis, brachymesomelia, metatarsal synostoses and reduction of metatarsal rays may be present. In our case, successful bilateral digital ray individualisation and tendon transfers were performed to construct a grip function of the grossly deformed hands.

Fingers↗
PubMed2005

Prevalence of rib anomalies in normal Caucasian children and childhood cancer patients.

PURPOSE: To evaluate the prevalence of abnormalities of rib development in normal Caucasian children and patients with childhood cancer. MATERIALS AND METHODS: Chest radiographs of 881 Caucasian pediatric controls and 906 childhood cancer patients were reviewed, and independently scored by four blinded observers, using strict definitions. Prevalences of 6 major rib anomaly categories in controls were compared to their prevalence in the total group of childhood cancer patients, and the 12 individual larger tumor groups using Chi-square tests. RESULTS: Values in the control population were generated for the occurrence of six major rib anomaly categories; cervical rib anomalies were present in 6.1% of controls, aplasia of 12th ribs in 6.6%, lumbar ribs in 0.9%, bifurcations in 0.7%, synostosis-bridging in 0.3%, and segmentations were not found. The overall prevalence of total rib anomalies in cases and controls was equal (14.9% and 14.2%, respectively). Cervical rib anomalies were found significantly more often in cases (8.6%) compared to controls (p-value=0.047), three groups accounting for this higher prevalence: 12.1% of acute lymphoblastic leukemia patients (p=0.011), 18.2% of astrocytoma patients (p=0.023), and 14.7% of germ cell tumor patients (p=0.046) had a cervical rib anomaly. CONCLUSION: Prevalence figures for the presence and type of rib anomalies in a large group of normal Caucasian children were generated. In childhood cancer patients a significantly higher prevalence of cervical rib anomalies was demonstrated in patients with acute lymphoblastic leukemia, astrocytoma, and germ cell tumors.

Adolescent↗
PubMed2005

Treatment of infected defect pseudoarthrosis of the tibia by in situ fibular transfer in children.

The aim of this study was to determine the effectiveness of in situ fibular transfer in the treatment of infected defect pseudoarthrosis in childhood. Four cases with infected defect pseudoarthrosis of the tibia were treated with debridement, sequestrectomy and two-staged ipsilateral fibular transfer. The mean age of the patients was 7.5 (2-11) years. Mean follow-up period was 9.5 (6-13) years. In all cases infection was resolved and tibia-fibular synostosis was achieved. The diameter of the transferred fibula increased significantly with regard to the unaffected fibula. None of the patients had limitation of joint motion or shortening more than 1cm. This technique is a good method for treatment of infected defect pseudoarthrosis of the tibia.

Child↗
PubMed2004

Bone lengthening for congenital differences of the hands and digits in children.

PURPOSE: We investigated the growth of bones after lengthening to evaluate the effectiveness and usefulness of bone lengthening in congenital hand deficiencies. METHODS: Bone lengthening was performed to treat congenital hand deficiencies including hypoplastic thumb, hypoplasia of the small finger, cleft hand, metacarpal synostosis, brachymetacarpia, symbrachydactyly, and amniotic band syndrome. A total of 15 patients (1-13 years old) underwent lengthening of 23 bones. The average of distraction was 10 mm. The mean follow-up period was 59 months. The length of the bones at follow-up evaluation was measured and then compared with the length of the bones immediately after lengthening. The bone growth after lengthening, the percentage of growth after lengthening, and the growth rate were compared among diagnostic groups. RESULTS: In the cases involving older patients the bone length changed little and the growth plates of these patients closed naturally soon after lengthening. In contrast, in the cases in which the patients were younger than 7 years old the lengthened bones grew continuously. The amount of bone growth was different for each disease in the younger patients. In patients with amniotic band syndrome the lengthened bones grew at the same rate as the bones in the hand. Other diagnoses such as symbrachydactyly had a different growth pattern in the hand. CONCLUSIONS: We conclude that bone lengthening is a reliable method for the correction of congenital differences of the hand. The indications for this procedure must be determined while considering the relationship between the specific disease and subsequent bone growth.

Adolescent↗
PubMed2006

Radial shift of the ulnar fingers: a new technique for special cases of longitudinal central deficiency.

Treatment of central longitudinal deficiencies is well defined, with different techniques established for the classical clefts. However, none of these techniques is easily applied to the treatment of very deep clefts accompanied by a significant divergence of the metacarpal bones. In such cases, the results of current techniques are disappointing. We propose a new technique of "Translocation in the Radial direction of the Ulnar Finger(s)" (TRUF) by intra-carpal osteotomy. The results are illustrated in three clinical cases. The TRUF operation allowed closing of the cleft, alignment of the metacarpal bones and preservation of carpometacarpal mobility. When necessary, a metacarpal synostosis may be treated at the same procedure.

Bone Transplantation↗
PubMed2004

The effects of craniosynostosis on the brain with respect to intracranial pressure.

Intracranial pressure (ICP) and skull volume are intricately related. Craniosynostosis alters skull volume, and the many forms of craniosynostosis complicate the relationship to ICP even further. Patients with single-suture synostosis are less likely to experience elevated ICP than patients in whom multiple sutures, craniofacial syndromes, or both are involved. Among patients with more than one suture involved, the multifactorial mechanisms underlying elevated ICP include cephalocranial disproportion and venous outflow obstruction. Direct monitoring of ICP for at least 24 hours can aid in the diagnosis and decision making process. The management of craniosynostotic patients is diverse and necessitates a long-term plan for follow-up.

Brain↗
PubMed2004

Cognitive impact of craniosynostosis.

This article reviews the data on the effects of synostosis on cognitive development and the role of surgical intervention in ameliorating these effects. The literature on children with single-suture craniosynostosis, treated both surgically and conservatively, is reviewed. The evidence for possible pathophysiological mechanisms by which brain restriction might affect cognitive development is also reviewed. Although children with single-suture isolated craniosynostosis may be at risk of developmental delay, learning disability, or both, especially with regard to speech or language skills, available testing methodologies provide no evidence of an association between surgical intervention and ultimate intellectual outcome. Despite the controversies about the role of surgery in addressing the cognitive sequelae of simple craniosynostosis, in the absence of definitive data, the indications for surgery include correction of deformity and minimization of cognitive sequelae. The relative risks of performing surgery early or late must be determined on an individual basis, balancing potential cosmetic and cognitive benefits against the heightened risk of reoperation or perioperative morbidity.

Child↗
PubMedDate not supplied

[Hearing loss in Townes-Brocks syndrome].

In nineteen seventy two Towns and Brocks reported on a family in which the father and seven children showed anal atresia, triphalangeal thumb, metatarsal synostosis, extra fingers, neurosensorial deafness and ear abnormalities. The main traits were those related to hand, ear and anus alterations, transmitted under a dominant autosomal pattern. In this paper we present several members of the same family with similar alterations who finally were diagnosed of Townes-Brocks syndrome.

Abnormalities, Multiple↗
PubMed1985

Basilar multiplane cephalometric analysis.

This article presents a method of cephalometric tracing and analysis using the basilar view cephalogram and discusses its role in diagnosis and treatment planning. Landmarks and structures found in each of three separate basilar planes are defined and instructions for tracings are presented. The analysis is applied to the study of orbital hypertelorism, craniofacial synostosis, and hemicraniofacial microsomia. The multiplane tracing technique is demonstrated to provide a three-dimensional concept of deformities in the craniofacial skeleton. A method to determine an anteroposterior midline construct from structures in the cranial base is described. As is practiced with the lateral cephalogram, presurgical tracings of the basilar film may be manipulated to simulate the skeletal changes anticipated in surgery.

Cephalometry↗
PubMed1997

Craniofrontonasal dysplasia.

A series of 10 patients with craniofrontonasal dysplasia presenting to the Oxford Craniofacial Unit since 1983 is presented. In addition to the well-described combination of coronal synostosis and frontonasal dysplasia, 9 patients had very characteristic dry, curly or frizzy hair. All the patients were female. Recognition of the syndrome is important for genetic counselling, although the precise mode of genetic transmission is unclear with females predominating and males being less severely affected. Surgical correction was in two stages: early frontal advancement followed by correction of hypertelorism when the child became aware of the deformity. Four patients had their craniosynostosis treated in the Oxford Craniofacial Unit. Three patients had previously had frontal remodelling elsewhere. Nine patients had surgery for hypertelorism. The preferred technique for hypertelorism correction was facial bipartition. Following hypertelorism correction, the excess skin was allowed to redrape and subsequently dealt with by medial canthoplasties, thus avoiding a midline scar. Careful attention to the primary frontal advancement procedure is important to avoid complications following difficult dissection of the frontal bone flap at the time of hypertelorism correction.

Abnormalities, Multiple↗
PubMed2001

Treatment of diaphyseal fractures of the forearm using the Point Contact Fixator (PC-Fix): results of 387 fractures of a prospective multicentric study (PC-Fix II).

Fractures of the forearm are considered to be a very good indication for plating. Conventional plating is still technically demanding. Extensive animal studies show that there is potential for improvement. The method consists of using an internal fixator that minimizes implant contact to the bone to reduce biological damage. Furthermore, foregoing the need for precise reduction and interfragmental compression makes it easier to use and, therefore, it should be safer. The goal of the study was to observe handling qualities, healing, and complications. For this purpose the study was designed to yield a very high follow-up in clinics with adequate experience and with a number of different surgeons performing the internal fixation. The study was designed as a prospective multicentre investigation in 16 trauma centres worldwide. To test the new approach to internal fixation, diaphyseal fractures of the forearm were stabilized surgically with the PC-Fix (Point-Contact Fixator) system, namely, 387 fractured bones involving 277 forearms in 272 patients. Internal fixation was performed using 387 PC-Fixators. 21% of the fractures were open and 25% occurred in polytraumatized patients. The rate of follow-up was 97%. 355 fractured bones healed uneventfully within four months. Overall, there were 32 complications before bone union occurred; 27 of these bones required re-operation (27 of 387, 7%). All of them subsequently healed without further complications. There were 15 delayed or non-unions and seven implant loosenings, two of which were associated with infection. Two superficial (0.6%) and two deep infections (0.6%) occurred in 306 closed fractures. Deep infection developed in one of 81 osteosyntheses of open fractures (1.2%). Other complications included one synostosis, fractures with the implant in situ with adequate trauma involving three bones, and two secondary correction procedures for postoperative malalignment. There were seven refractures occurring at a mean of three weeks after the removal of 150 PC-Fixators (4.7%). This study demonstrated that the technique of using an internal fixator is a simple one for the fixation of forearm fractures, resulting in shorter duration of surgery with a low rate of complications compared with the data reported in the literature on conventional techniques for forearm fracture stabilization.

Adolescent↗
PubMed1977

A heritable syndrome of craniosynostosis, short thin hair, dental abnormalities, and short limbs: cranioectodermal dysplasia.

Five children are reported with dolichocephaly (with sagittal suture synostosis in three), sparse, slow-growing, fine hair, epicanthal folds, hypodontia and/or microdontia, short span, brachydactyly and brachypodia, and narrow thoraces. Radiologic abnormalities were noted chiefly in the skull, chest, and limbs. Intelligence was normal. The pattern of inheritance is compatible with an autosomal recessive trait.

Abnormalities, Multiple↗
PubMed1977

Subtotal neonatal calvariectomy for severe craniosynostosis.

This report sets forth an example of a new mode of management of severe craniosynostosis in the neonate: subtotal calvariectomy. An infant with synostosis of sagittal, coronal, and lambdoidal sutures who had signs of increased intracranial pressure with impending neurologic complications was operated on at 13 days of age. Wide excision of the bony calvarium from the underlying dura was accomplished from the supraorbital ridge to near the foramen magnum; the entire area of the coronal sutures was included. Regeneration of the calvarium occurred over two to 12 weeks and was accompanied by the presence of "sutures" in a usual location. These procedures have allowed for normal brain growth without reoperation up to the current age of two years. Neurologic and intellectual performance has been satisfactory, and cosmetic results appear to be preferable to those obtained by more limited surgery. The observations suggest that this approach may permit better craniofacila growth by minimizing secondary deformities in growth of the cranial base.

Child, Preschool↗
PubMed2000

Syndromes involving craniosynostosis and midface hypoplasia.

This article reviews a number of well-known syndromes involving craniofacial synostosis and associated midface deficiencies. Syndromes discussed include Apert's, Crouzon's, Saethre-Chotzen, and Carpenter's. Clinical characteristics and genetic defects are discussed. A general approach to surgical management is outlined.

Acrocephalosyndactylia↗