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At least 181 records · Page 10Linked to original sources
PubMed1986

Radioulnar synostosis following an isolated fracture of the ulnar shaft. A case report.

In the case of a 19-year-old man, overuse of his nondominant forearm resulted in a radioulnar synostosis. Despite the persistence of the synostosis, the patient adapted to loss of forearm rotation. No further treatment was necessary. Isolated fractures of the ulnar shaft are slow in healing. Several authors observed that early function may be beneficial. However, excessive activity causing motion at the fracture site may cause subperiosteal hemorrhage and soft tissue trauma and may stimulate exuberant callus formation. Fractures of the ulnar shaft, even undisplaced, need to be immobilized to prevent overuse.

Adult↗
PubMed1984

Post-traumatic radioulnar synostosis. A report of two cases.

In two cases of post-traumatic radioulnar synostosis occurring in a father and his adult son, the bony bridge was excised and a Silastic sheet interposed between the forearm bones. Both patients benefitted and had improved function of the hand following the procedure, although the range of forearm rotation restored was limited. The question is raised as to whether trauma triggers the expression of a latent familial tendency to synostosis of the radius and ulna in these cases.

Adult↗
PubMed1980

[Ventral vertebral synostosis in adolescents (author's transl)].

6 cases of anterior vertebral synostosis in juvenile patients are described. All cases were observed in girls in the age of 11-16 years and together with rapidly developing round back deformity. In 4 patients the synostosis was seen at the first examination already in 2 patients only later. A special form of this deformity is described and therapeutic measures are discussed.

Adolescent↗
PubMed1993

[Pathomorphogenesis of radio-ulnar synostosis in fractures of forearm bone shafts].

Pathomorphogenesis of radioulnar synostosis which occurred in 4 out of 174 patients treated because of recent and inveterate fractures as well as pseudoarthroses within shafts of forearm bones has been presented. Fractures of both radius and ulna, located at the same level within proximal half, inveterate or neglected, after severe or multiple trauma are at the highest risk of developing radioulnar synostosis.

Adult↗
PubMed1994

Genetic nosology and counseling of humeroradial synostosis.

From published cases of humeroradial synostoses three entities can be delineated: AD ankylosis of the elbow as a part of a systemic disorder causing multiple synostoses (symphalangism), AR humeroradial synostosis with dysgenesis of the ulna (and fibula?) but without oligodactyly as part of a syndromal disorder, non-germinal uni- or bilateral humeroradial synostosis as part of ulnar malformation and oligodactyly. Three new observations are considered examples of the latter entity.

Ectromelia↗
PubMed1997

Autosomal dominant and sporadic radio-ulnar synostosis.

We report on seven cases of congenital radio-ulnar synostosis (RUS). Five were found in the same family and two were sporadic. In six the synostosis was bilateral and consistently involved the proximal end of the radius and ulna. In the familial cases the anomaly was inherited as an autosomal dominant trait and was associated with a Dubois sign and relative shortness of metacarpals number 4 and 5 in two patients, and of number 2 in another patient, and of all phalanges of the 5th fingers. These observations suggest involvement of an ulnar developmental field. RUS does not seem to be rare in the Sicilian population.

Adult↗
PubMed1997

Congenital metacarpal synostosis treated by longitudinal osteotomy and placement of a silicone wedge.

Three cases of congenital metacarpal synostosis are reported. Two had congenital synostosis between the fourth and fifth metacarpal, and one between the third and fourth metacarpal. Both hands were involved in the former cases, while only one unilateral hand was involved in the latter case. An abnormal abduction position of the finger was noted in early infancy, and this gradually progressed as the patients grew. Operations were performed between the ages of three to eleven years. Fused metacarpal bases were longitudinally osteotomized with a chisel, and a silicone wedge was inserted between the metacarpal bases. After the operation, the hand was immobilized with a plaster cast for four weeks. The follow-up periods range from two to sixteen years. The postoperative function and appearance of the hand were remarkably improved.

Child↗
PubMed2006

Hypertropia associated with superolateral translation of the superior rectus muscle pulley in unilateral coronal synostosis.

OBJECTIVE: To explore the mechanisms underlying the hypertropia associated with unilateral coronal synostosis. METHODS: In 13 patients with unilateral coronal synostosis, we measured gaze-dependent binocular alignment before and after strabismus surgery, assessed the superior rectus muscle (SRM) pulley using computed tomography, and simulated posterior displacement of the trochlea and superolateral displacement of the SRM pulley. RESULTS: All the patients had an ipsilateral hypertropia in primary gaze (3-30 diopters) that increased in contralateral gaze and decreased in ipsilateral gaze and that simulated an inferior oblique muscle overaction. Strabismus surgery fully or partially corrected the hypertropia in only 7 of 11 patients. High-resolution computed tomography demonstrated that the pulley of the SRM relative to the inferior rectus muscle was translated 0 to 11.0 mm laterally and up to 2.7 mm vertically. Lateral translation (up to 10 mm) alone or combined with vertical translation (up to 5 mm) of the SRM pulley in the simulated model produced a hypertropia with lateral incomitance. Posterior translation (15 mm) of the trochlea did not induce a significant hypertropia. CONCLUSION: Superolateral translation of the SRM pulley creates an imbalance of muscle pulling forces that better accounts for the hypertropia than posterior displacement of the trochlea.

Child↗
PubMed1999

Three-dimensional morphological analysis of isolated metopic synostosis.

Morphological differences were quantified in three-dimensions among individuals with untreated isolated metopic synostosis and between those individuals and similar aged-matched normal dry skulls to test two hypotheses: first, that the dysmorphology is a self-correcting condition; and second, that a lack of vertical growth of the skull produces this dysmorphology. Three-dimensional (3D) coordinates were recorded for 22 craniofacial landmarks from CT scans of 15 metopic patients, ranging from 5- to 32-months-old, and of four normal dry skulls, ranging in age from 6- to 36-months-old. The patient population was diagnosed with isolated metopic synostosis at The Johns Hopkins Medical Institutions in Baltimore, Maryland or Children's Hospital in St. Louis, Missouri. Comparisons between the metopic age groups indicate that the trigonocephalic phenotype worsens through time. Between 5 and 14 months, the neurocranium displays an increase in vertical growth. This was followed by a lack of vertical growth between 14 and 32 months. The face displays a lack of vertical growth from 5 to 14 months and an increase in vertical growth after 14 months. Comparisons between the metopic age groups and the normal skulls indicate that the trigonocephalic head is taller superoinferiorly and longer anteroposteriorly. Relative to the normal phenotype, the inferior temporal region in the metopic phenotype is narrow. These findings enabled the rejection of both hypotheses and localized form differences between normal and metopic phenotypes. Based on these results, we suggest that the trigonocephalic phenotype worsens with age and the amount of vertical growth that produces the trigonocephalic phenotype varies throughout growth with respect to location within the skull and age.

Age Factors↗
PubMed1995

Recurrent lambdoid synostosis within two families.

We report on 2 families with multiple members who have proven or suspected lambdoid craniosynostosis. In one family the lambdoid suture was unilaterally involved in one sib, and bilaterally in the other. In the second family the propositus had unilateral lambdoid synostosis and his twin sisters each had bilateral lambdoid synostosis. Both families had another distant relative, a maternal grandmother in the first and paternal uncle in the second, who were also reported to have posterior plagiocephaly. We report these families as evidence for genetic transmission of a craniosynostotic trait which has only been rarely reported previously.

Brain↗
PubMed2006

Prenatal diagnosis of achondroplasia presenting with multiple-suture synostosis: a novel association.

OBJECTIVE: We report an atypical case of a fetus presenting with a combined achondroplasia and multiple craniosynostosis phenotype. METHODS: Sonographic monitoring in conjunction with molecular genetic analysis was performed in a 32-gestational weeks fetus. RESULTS: Sonographic findings were consistent with a diagnosis of achondroplasia associated with multiple-suture synostosis. The most common G380R FGFR3 achondroplasia mutation was detected. CONCLUSION: The most common achondroplasia mutation should be considered for prenatal DNA testing in cases with ultrasound findings of achondroplasia and multiple-suture synostosis. This is crucial for the genetic counselling and perinatal management of the fetus.

Achondroplasia↗
PubMed1996

Procedure of lateral canthal advancement for the treatment of coronal synostosis.

The procedure of lateral canthal advancement has been used at the Hospital for Sick Children, Toronto, since 1973. The patients treated by this technique between 1973 and 1993 consisted of 41 with unilateral coronal synostosis, 13 with trigonocephaly and 29 with craniofacial dysostosis. The results have been excellent in patients with unilateral coronal synostosis and trigonocephaly. Furthermore, 17 of the infants with craniofacial dysostosis needed no further treatment after their initial operation.

Craniofacial Dysostosis↗
PubMed1998

[Standardized occipital advancement. A new method for therapy of lambda suture synostosis].

Lambdoid synostosis can manifest unilaterally, bilaterally or in combination with other craniosynostoses. Using the concept of fronto-orbital advancement we developed occipital advancement in order to correct unilateral or bilateral lambdoid synostosis. The process involves striped transversal osteotomy, removal, remodelling and advancement of the occipital region. It allows precise, reproducible and predictable positioning of the segments. Artificial sutures are created as a result of the osteotomy. The remodelling leads to a well-proportioned skull shape and advancement to an increase in the intracranial volume. This article presents the operative method and the results of 14 patients.

Cephalometry↗
PubMed2003

[Typical features of craniofacial growth of the FGFR3-associated coronal synostosis syndrome (so-called Muenke craniosynostosis)].

BACKGROUND: The FGFR3-associated coronal synostosis syndrome (Muenke craniosynostosis) is caused by a point mutation (C749G) on the FGFR3 gene resulting in a Pro250Arg substitution. METHODS: To characterize this malformation, the neuro- and viscerocranium were analyzed by axial CT scans of the skull and cephalometric radiographs of up to 13 affected children before and in part after fronto-orbital advancement. RESULTS: Preoperative analysis of the intracranial volume of four patients showed a mean decrease of 3.6%, indicating a compensatory growth pattern of the skull in cases of coronal synostosis. The typical brachycephaly could be verified by the significant shortening of the skull length of 13.2% on average and by the significant reduction of the anterior cranial base length of maximal 5.9% on average. The anterior part of the skull was characterized by a significant mean increase of the intercoronal distance of 8.6%, which indicates a compensatory transversal growth in this malformation. The widened bilateral interorbital and anterior interorbital distances were increased by 7.3 or 9.0%, respectively, confirming a hypertelorism typical for this syndrome. The "frontal bossing" frequently found in brachycephaly was characterized by the preoperatively increased sagittal extension of the forehead (about 112.9% above the norm) and by the increased height of the frontal prominence (about 47.8% above the norm). Following surgery, both variables defining the morphology of the forehead were reduced and appeared to be constant throughout the follow-up. Hypoplasia of the midface described by Muenke et al. (1997) was confirmed in the present study only by the significant reduction of the sagittal length of the maxillary base, which was decreased by 6.8%. CONCLUSION: In accordance with the current literature, the skull configuration described for Muenke craniosynostosis shows similarities with the Saethre-Chotzen syndrome.

Amino Acid Substitution↗
PubMed2004

Correction of sagittal synostosis using a modified Pi method.

The authors' technique for the treatment of sagittal synostosis, which involves total cranial vault reconstruction and is termed the Pi procedure, is a safe, effective method for correcting the deformities associated with sagittal synostosis. It provides an immediate correction that does not require any further manipulations to the skull, such as a molding helmet. The technique addresses all the aspects of the deformity. It increases the width as well as the central height of the skull and decreases the length of the skull to produce a rounder cranial vault.

Child, Preschool↗
PubMed2003

[Clinico-radiological study of the skull deformation in the plagiocephaly without synostosis].

UNLABELLED: The aim of this study was to analyze the mechanism of the skull deformation observed in frontal and occipital plagiocephaly without synostosis. POPULATION AND METHODS: From 1987 to 2001, 96 plagiocephalies without synostosis, 72 males and 24 females, were examined. There were 72 Frontal Plagiocephalies (FP) and 24 Occipital Plagiocephalies (OP). The clinical examination of the neck muscles was performed to find out a torticolis with or without retraction. All patients underwent a 3D CT scan of the skull to exclude a craniostenosis. Cephalic Index (CI) was also calculated. RESULTS: The FP had a torticollis in 89.3% of cases associated with a muscle retraction (54.8%), a tonus asymmetry (16.6%), or in association (28.6%). The skull had a parallelepiped shape and the greater axis was oblique. The CI was 0.85. The OP had a torticollis in 50% of case associated with a retraction (28.6%), a tonus asymmetry (57.1%), or the association (14.3%). The skull had a square shape with an occipital flat and the CI was 0.95. DISCUSSION: The FP and OP are two deformations whose mechanism is different. The FP corresponds to a three plans skull asymmetry secondary to muscle traction on the base of the skull, and the OP corresponds to a posterior localized asymmetry of the skull secondary to compression. CONCLUSION: The traction and/or compression deformation start in utero or in the newborn period and the supine position is an aggravating positional factor.

Anthropometry↗
PubMed2002

Preliminary results of standardized occipital advancement in the treatment of lambdoid synostosis.

INTRODUCTION: Lambdoid synostosis can be found unilaterally, bilaterally or in combination with other forms of craniosynostosis. Based on the concept of frontoorbital advancement, we used the occipital advancement in order to correct unilateral or bilateral lambdoid synostosis. METHODS: The standardized technique consists of transverse osteotomies, removal, remodelling and advancement of the occipital region. RESULTS: Standardized occipital advancement was performed in 21 patients at a multidisciplinary craniofacial centre. The surgery was carried out for patients between 5 and 28 months of age. Aesthetically satisfactory skull shape and normalization of the intracranial pressure could be achieved. A major complication in the form of a life-threatening intraoperative haemorrhage occurred in one case. Other complications like infections have not been experienced. CONCLUSION: Standardized occipital advancement allows precise, reproducible and predictable positioning of the segments. Artificial 'sutures' are created as a result of the osteotomy. Remodelling leads to a well-proportioned skull shape and posterior advancement leads to an increase in intracranial volume.

Blood Loss, Surgical↗