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PubMed1999

Positron emission tomography studies confirm the need for early surgical intervention in patients with single-suture craniosynostosis.

Craniosynostosis, the premature fusion of one or more cranial sutures, may occur in isolation or in association with a syndromic constellation. Multiple-suture synostosis has consistently been associated with brain compression and increased intracranial pressure, and frequently decreased cognitive development. Single-suture craniosynostosis, however, has been thought by some to be an aesthetic problem with infrequent consequences on brain function and development. Some studies have disputed this concept and have argued a correlation between single-suture craniosynostosis and abnormalities in development. The purpose of this study was to determine, using an objective radiographic tool, positron emission tomography scans, if patients with single-suture craniosynostosis had any abnormalities in cerebral glucose metabolism that would indicate changes in local brain function. A total of 10 children with single-suture craniosynostosis, eight males and two females, ranging in age from 0.1 to 3.2 years, were enrolled in this prospective study approved by the Internal Review Board. Six of the children had sagittal synostosis, three had metopic synostosis, and one had coronal craniosynostosis. Each of the patients had preoperative positron emission tomography scans performed 1 to 5 weeks before cranial reconstructive surgery and postoperative scans at 6 to 12 weeks after surgery. Surgical treatment consisted of cranial vault remodeling in eight of the children and strip craniectomy with cranial expansion in two of the children. After surgery, the two scans were compared qualitatively and quantitatively by a single radiologist. The results demonstrated variable regional increases and decreases in local post-operative cerebral glucose metabolism. However, in the posterior occipital region, the area of visual development and visual spatial coordination, there was a consistent postoperative increase in all 10 patients. Maximum glucose metabolic rate was increased up to 30.2% with a mean of 9.9%, and average glucose metabolic rates demonstrated a maximum increase of up to 18.8%. The results of this study indicate cerebral glucose metabolism consistently increases in the posterior occipital cortex after surgical release of single-suture craniosynostosis. Future developmental studies are being performed to examine the functional consequences of these metabolic changes.

Age Factors↗
PubMed1999

Increased intracranial pressure after coronal suturectomy in craniosynostotic rabbits.

It has been suggested that the complications associated with intracranial hypertension in craniosynostotic infants may be managed with surgical release of the synostosed sutures. However, both postoperative increases and decreases in intracranial pressure (ICP) have been reported in heterogeneous samples of infants with syndromic and nonsyndromic craniosynostoses. The present study was designed to describe longitudinal changes in ICP in a homogeneous sample of rabbits with uncorrected and corrected familial coronal suture synostosis and compare them with age-matched normal control rabbits. Fifty-three rabbits were divided into three groups: normal rabbits (n = 28), rabbits with uncorrected bilateral coronal suture synostosis (n = 9), and rabbits with bilateral coronal suture synostosis with coronal suturectomy at 25 days of age (n = 16). ICP was measured at 25 and 42 days of age using a Codman epidural microtransducer. Results revealed that rabbits with uncorrected craniosynostosis had significantly (P < 0.05) higher ICP at 25 days of age than normal control rabbits by approximately 86%. However, by 42 days of age, ICP in normal rabbits increased by 75%, whereas ICP in rabbits with uncorrected craniosynostosis decreased by 69% over the same time. Synostotic rabbits with coronal suturectomy showed a 50% decrease in ICP immediately after surgical release and then followed the normal, age-related ICP pattern, which significantly increased by 75% at 42 days of age. Results suggest that, in the rabbit model, the postsuturectomy rise in ICP may simply be normal, age-related changes, although a longer follow-up will be needed to determine the recurrence of pathological ICP. Possible multifactorial explanations for intracranial decompression and compensation in the craniosynostotic rabbit model are also discussed.

Analysis of Variance↗
PubMed1990

Delayed and progressive multiple suture craniosynostosis.

A considerable amount of information is available on various types of craniosynostoses. The patient exhibiting single suture synostosis that progresses to involve multiple sutures is distinctly uncommon, as is the patient exhibiting delayed synostosis involving all of the calvarial sutures. We report a group of 11 such patients with progressive and delayed holocalvarial synostosis. Most patients exhibited features of raised intracranial pressure or developmental delay, and in all patients symptoms were relieved after surgery. The diagnostic and therapeutic implications of this type of presentation in craniosynostosis are discussed.

Child↗
PubMed2005

Early development of infants with untreated metopic craniosynostosis.

BACKGROUND: The purpose of this archival descriptive study was to examine the associations among craniosynostosis, perinatal risk factors or complications, and early cognitive development in infants diagnosed with craniosynostosis before they underwent surgery, to provide a greater empirical basis for surgical decision making and other early interventions. Specifically, it was hypothesized that there would be a greater number of infants with developmental delays than seen in the normative population. Furthermore, it was hypothesized that greater severity of synostosis would be correlated with lower cognitive and developmental scores. The secondary purpose of this study was to examine specific developmental domains such as language and motor skills in children with metopic craniosynostosis. METHODS: The research subjects for this retrospective cross-sectional study were a consecutive series of 22 infants diagnosed with metopic synostosis: 86 percent male, ages 3.6 to 25.3 months (mean +/- SD, 10.6 +/- 6.4 months). Mean gestational age was 38.8 (2.3) months, mean birth weight was 107.7 ounces, and 48.1 percent subsequently had craniovault reconstruction. Severity of craniosynostosis was assessed by a plastic surgeon (Buchman) and a neurosurgeon (Muraszko) and was confirmed radiographically by an independent rater (Angobaldo). Cognitive status was assessed with the Bayley Scales of Infant Development, Second Edition, at a mean age 11.6 (4.8) months. RESULTS: The mean Mental Development Index score for the sample was 96.0 (14.5), falling within the average range. Severity of synostosis was not significantly correlated with the overall Mental Development Index score. The mean language quotient for the sample was 77.3 (21.1). CONCLUSIONS: Despite suspicions that increased severity of deformity in infants with metopic craniosynostosis correlates with decreased cognitive and motor development, no such associations could be demonstrated. The results of this study did suggest, however, that children with isolated metopic craniosynostosis might show specific developmental delays in language acquisition. Finally, there were no significant correlations between cognitive development and prenatal risk factors.

Child, Preschool↗
PubMed2006

Sagittal craniosynostosis: surgical outcomes and long-term growth.

BACKGROUND: Numerous publications have examined technique-specific results in treating sagittal synostosis, but there are scant data examining long-term outcomes. This retrospective study was designed to evaluate a large series of surgically repaired children with sagittal craniosynostosis and to examine long-term cranial growth following correction. METHODS: From 1990 to 2003, 132 children with sagittal synostosis were evaluated and 89 surgically treated (single-stage posterior remodeling) patients were retrospectively reviewed (43 were excluded for multisutural involvement, incomplete records, or nonsurgical treatment). Long-term growth was assessed from anthropologic measurements taken from 3 to 11 years postoperatively (mean, 4.7 years). RESULTS: The surgical age ranged from 2 months to 5 years (mean, 8 months), and the hospital stay was less than 3 days. There were no deaths, major complications, infections, or repeated remodeling procedures performed. Postoperatively, the cephalic index was overcorrected. Subsequent growth of the cranial breadth and length was less than predicted. Moreover, growth was more deficient in cranial breadth than length, resulting in a regression of the cephalic index over time (p < 0.01). Frontal breadth improved immediately postoperatively but did not subsequently narrow over time. Head circumference also showed a statistically significant diminished capacity for growth (p < 0.001). CONCLUSIONS: This study demonstrated abnormal skull growth in children following the surgical correction of scaphocephaly. On the basis of these findings, surgeons treating sagittal synostosis may wish to expand their goals of treatment beyond normalization to an overcorrection of the abnormal skull shape.

Child, Preschool↗
PubMed2001

Antley-Bixler syndrome, description of two new cases and review of the literature.

OBJECTIVE: Antley-Bixler syndrome (ABS) is a rare disorder characterized by multiple malformations of cartilage and bone including multisynostotic osteodysgenesis, midface hypoplasia, choanal atresia or stenosis, femoral bowing, neonatal fractures and multiple joint contractures and, occasionally, urogenital, gastrointestinal or cardiac defects. Since the first report in 1975, at least 34 cases (including this report) have been described. We present 2 cases of congenital hydrocephalus, suggesting the cause of craniosynostosis and midface hypoplasia is associated with ABS. CLINICAL PRESENTATION: The first case was a 1-day-old female with Arnold-Chiari malformation, multiple cranial synostosis, obstructive hydrocephalus and radioulnahumeral synostosis. Ventriculoperitoneal (V-P) shunting was performed when she was 7 days old. She died 42 days later due to cardiopulmonary failure. The second case was a 2-month-old female with bilateral coronal synostosis, obstructive hydrocephalus and brachycephaly. V-P shunting was done soon after her admission, and bilateral lateral canthal advancement with the floating forehead procedure was performed 1 month later. She is still alive with good development. DISCUSSION: The main anomalies of the ABS can be divided into 4 categories: craniofacial, skeletal, extremity and urogenital anomalies. They may be the result of mutation in the fibroblast growth factor receptor 2 (Ser351Cys) gene, which was confirmed in our case 2. Craniosynostosis combined with hydrocephalus created congenital increased intracranial pressure (IICP). Early V-P shunt implantation and surgical release of the closed suture and lateral canthal advancement should be done as soon as possible, ideally when the patient is younger than 3 months. CONCLUSION: Early correction of craniosynostosis was feasible and safe for one of our patients. We offer our experience in the treatment of hydrocephalus and correction of craniosynostosis to relieve IICP.

Abnormalities, Multiple↗
PubMed1995

Transplanted demineralized bone graft in cranial reconstructive surgery.

Surgical reconstruction of cranial deformities and synostosis is occasionally accompanied by incomplete bone growth to cover all areas of cranial vault that have been exposed in the correction. The restrictive nature of some forms of synostosis require more bone in the repair than is available using the child's natural skull for autogenous bone cranioplasty. Rib and iliac crest autografts have been used with success. These grafts must be harvested form a remote site with increased morbidity. A split-thickness skull autograft is the cranioplasty material of choice but children under the age of 6 years may lack the skull thickness needed to use this technique. Perforated demineralized bone matrix has been transplanted in 46 operations in 42 patients from 1990 to 1995 for repair of residual skull defects in children having previously undergone craniofacial repairs, for primary reconstruction of the cranial vault for patients with synostosis and for repair of skull defects resulting from trauma and skull tumor excisions. The vast majority of grafts have resulted in complete closure of the defect, providing a matrix for new bone formation. These patients are presented. Surgical techniques of cranial defect repair with perforated demineralized bone matrix are discussed.

Adolescent↗
PubMed1987

Hereditary bovine syndactyly: diagnosis in bovine fetuses.

Diagnostic guidelines were established for progeny testing of hereditary bovine syndactyly. Through the use of superovulation and embryo transfer, 139 fetuses were recovered at 50 to 77 days gestation. Normal (+/+, +/sy) and syndactylous (sy/sy) anatomy of Holstein fetuses was defined, and the accuracy of macroscopic versus microscopic limb diagnosis was assessed. Chondrification and ossification differences between normal (+/+, +/sy) and syndactylous (sy/sy) fetuses were only age-related. Normal (+/+, +/sy) fetal limbs differed from normal (+/+, +/sy) adult bovine limbs in two ways. Fetal metacarpal and metatarsal III and IV bones were not fused, and fetal metacarpal and metatarsal II and V bones often extended up to three-fourths the length of metacarpal and metatarsal III and IV bones. In syndactylous (sy/sy) fetuses, synostosis asymmetries occurred within and between fetal limbs, and between fetuses, representing variable gene expressivity. Synostosis pattern within limbs did not correspond with those of the adult bovine; the second phalangeal pair was synostotic most frequently in the fetus, followed by the first, and then the third pair. Synostosis patterns between fetal limbs agreed with those of the adult; there was a right-left and front-rear limb gradient. Partial synostoses occurred sporadically in all three paired phalanges. Those of the first and third pair always involved the tip closest to the second phalangeal pair. A unique example of variable gene expressivity occurred in one syndactylous fetus. Both front limbs were syndactylous, while both rear limbs were normal grossly. Microscopically the right rear limb was normal while the left rear limb consisted of closely apposed phalangeal blastemata without coalescence.(ABSTRACT TRUNCATED AT 250 WORDS)

Animals↗
PubMed1997

Upper airway obstruction and raised intracranial pressure in children with craniosynostosis.

In children with craniosynostosis, raised intracranial pressure (ICP) and upper airway obstruction (UAO) are both common features. However, potential interactions between UAO and ICP during sleep are poorly understood. The aim of the present study was to compare the levels of ICP during sleep between a group of patients with syndromic craniosynostosis (with facial involvement and consequent UAO) and a group of control patients with isolated unicoronal synostosis (with no facial involvement and normal upper airways). Polygraphic cardiorespiratory sleep studies with continuous monitoring of ICP were performed during unsedated sleep in 13 children with syndromic craniosynostosis and 7 control patients with isolated unicoronal synostosis only. In the syndromic group, UAO was present in 11 out of 13 patients, with 8 out of 13 having frank obstructive sleep apnoea. In contrast, none of the control patients showed signs of UAO during sleep. There was no evidence of central apnoeas in any of the patients studied. Clinical histories taken from parents tended to underestimate the severity of the respiratory problems. Elevated ICP was seen in 10 of the 13 syndromic patients, with borderline raised ICP in the remaining three cases. In contrast, raised ICP was seen in only 3 of the 7 control patients, with borderline raised levels in 2 of the 7. For both patient groups, ICP was higher during active sleep compared to quiet sleep. Multiple regression analysis showed that ICP during active sleep was dependent upon disease severity (unicoronal/ multiple synostosis) and to the baseline ICP level during quiet sleep. Both raised ICP and airway obstruction were more apparent during active sleep. There was a significant correlation between severity of UAO and increased ICP in active sleep. We conclude that obstructive respiratory problems are frequent in the syndromic patients, and can be severe in a large proportion of cases; intracranial hypertension is also frequent in this group. Further studies are required to investigate the possibility of a causal relationship between upper airway obstruction and raised intracranial pressure.

Child↗
PubMed2004

Differential diagnosis of the trapezoid-shaped head.

OBJECTIVE: To recognize several conditions that result in a trapezoid head shape and review and contrast their various physical findings. METHODS: A detailed review of all patients seen in the Craniofacial Clinic at the Children's Hospital and Regional Medical Center in Seattle, Washington, over a 10-year period from 1991 to 2001, with the diagnosis of craniosynostosis and plagiocephaly was performed. During this period, 690 patients had a surgical correction of craniosynostosis, and 1537 patients had posterior plagiocephaly. RESULTS AND CONCLUSIONS: The shape of the head when viewed from the vertex position in an axial plane can be a significant diagnostic aid when evaluating a patient with plagiocephaly. Positional molding causes the vast majority of plagiocephaly. This deformational change results in a parallelogram-shaped head. A much more rare cause of plagiocephaly is lambdoid synostosis. With premature fusion of one of the lambdoid sutures, the head takes on a very characteristic trapezoid shape when viewed from the vertex. Unilateral coronal synostosis that occurs on the same side as either posterior positional molding or unilateral lambdoid synostosis will also result in the trapezoid head shape. Furthermore, on the rare occasion when anterior and posterior deformational plagiocephaly occurs on the same side, the trapezoid head shape may be the consequence. The choice of appropriate treatment modality requires systematic evaluation of the patient with a trapezoid-shaped head to determine the etiology of the deformation.

Craniosynostoses↗
PubMed2001

The effect of neurocranial surgery on basicranial morphology in isolated sagittal craniosynostosis.

OBJECTIVE: Isolated sagittal craniosynostosis produces a scaphocephalic neurocranium associated with abnormal basicranial morphology, providing additional evidence of the developmental relationship of the neurocranium and basicranium. Corrective surgical procedures vary, but the immediate impact of the surgical procedure is restricted to the neurocranium. This study addresses the secondary effects of neurocranial surgery on the cranial base. DESIGN: Three-dimensional (3-D) computed tomography (CT) scans were obtained for preoperative (n = 25) and postoperative (n = 12) patients with isolated sagittal synostosis. Landmark data from 14 landmarks on and around the cranial base were collected from 3-D CT reconstructions and analyzed using Euclidean distance matrix analysis. Subsamples of age-matched patients were used to identify basicranial differences in pre- and postoperative patients and to compare postoperative growth patterns identified in longitudinal data with preoperative growth patterns characterized in cross-sectional data. RESULTS: Statistically significant differences (p < or = 0.10) were found in the morphology of the cranial base in preoperative and postoperative patients. The relative positions of the landmarks nasion, right asterion, and left asterion are similar in preoperative and postoperative patients. However, the position of these landmarks relative to the cranial base is different in the two groups, being positioned relatively more anteriorly in postoperative patients. In addition, we found that the cranial base angle, on average, neither increases nor decreases in the first postoperative year. These morphological differences are associated with divergent growth trajectories in the operated and unoperated cranial base. CONCLUSION: Regardless of specific procedure, neurocranial surgery in sagittal synostosis patients affects growth patterns of the cranial base. The lack of change in the postoperative cranial base angle suggests that neurocranial surgery alleviates the occipital rotation and decreased cranial base angle described in the sagittal synostosis basicranium.

Case-Control Studies↗
PubMed1998

Monteggia fractures in adults.

The records concerning ten consecutive years of experience with Monteggia fractures in adult patients at a level-one trauma center were retrospectively reviewed. Forty-eight patients who had been followed for a minimum of two years (average, 6.5 years; range, two to fourteen years) were identified. There were twenty-five women and twenty-three men, and the average age was fifty-two years (range, eighteen to eighty-eight years). According to the classification of Bado, there were seven type-I, thirty-eight type-II, one type-III, and two type-IV injuries. Twenty-six patients (68 percent) who had a Bado type-II fracture had an associated fracture of the radial head; ten of these patients also had a fracture of the coronoid process as a single large fragment. The ulna was fixed with a tension band-wire construct supplemented with screws in three patients (all of whom had a Bado type-II fracture). An ulnar diaphyseal fracture was fixed with an intramedullary Steinmann pin in one patient. The remaining patients had fixation with a plate and screws. The fracture of the radial head was treated with either complete or partial excision of the fragments in twelve patients (with replacement with a silicone prosthesis in two), open reduction and internal fixation in ten patients, and no intervention in four patients. Nine patients, all of whom had a Bado type-II fracture, needed a reoperation within three months after the initial operation; five had revision of a loose ulnar fixation device, three had resection of the radial head, and one had removal of a wire that had migrated from the radial head into the elbow articulation. Other important complications included proximal radioulnar synostosis in three patients, ulnar malunion in three, posterolateral rotatory instability of the ulnohumeral joint in one, and instability of the distal radioulnar joint in one. At the most recent follow-up examination, which was performed after all of the reoperations and reconstructive procedures had been done, the average score according to the system of Broberg and Morrey was 86 points (range, 15 to 100 points). The result was excellent for eighteen patients, good for twenty-two, fair for two, and poor for six. Six of the eight patients who had an unsatisfactory (fair or poor) result had had a Bado type-II fracture with a concomitant fracture of the radial head. These unsatisfactory results were related to a malunited fracture of the coronoid process in two patients, a proximal radioulnar synostosis in one, a malunited fracture of the coronoid process and a proximal radioulnar synostosis in one, a malunion of the ulna in one, and painfully restricted rotation of the forearm after operative fixation of a comminuted fracture of the radial head in one. The other two unsatisfactory results were in a patient who had had a Bado type-I fracture and in one who had had a Bado type-IV fracture. The results of the present series are much better than those reported in most earlier studies, suggesting that stable anatomical fixation of the ulnar fracture (including associated fracture fragments of the coronoid process) with a plate and screws inserted with use of current techniques of fixation leads to a satisfactory result in most adults who have a Monteggia fracture. The posterior (Bado type-II) fracture is the most common type of Monteggia fracture in adults. Problems with the elbow related to fractures of the coronoid process and the radial head, which are common with Bado type-II Monteggia fractures, remain the most challenging elements in the treatment of these injuries.

Female↗
PubMed2002

Changes in orbital volume during childhood in cases of craniosynostosis.

OBJECT: Controversy remains concerning the timing of frontoorbital advancement (FOA) surgery performed for craniosynostosis. Reduced orbital volume and degree of exorbitism are often cited as reasons for early surgical intervention. To date, however, little attention has been given to orbital volume and its changes during the first few years of life as an indicator of orbital growth in children with craniosynostosis. Knowledge of orbital volume and growth patterns in individuals with craniosynostosis and those with normal cranial structures will enable surgeons to refine both the type and timing of surgical intervention required, thus obtaining the optimum outcome for their patients. METHODS: Using the procedure of segmentation, orbital volumes in 50 children with various forms of craniosynostosis were measured on preoperative computerized tomography scans. Changes in average volume that occur with increasing age were calculated and compared with a model of normal orbital volume growth. At presentation the children with craniosynostosis ranged in age from 1 to 29 months, with 82% of them within the 1st year of life. Several interesting observations emerged from this study. Excluding patients with unilateral coronal synostosis, there was no difference between orbital volumes measured on the right and left sides, allowing mean orbital volume measurements to be used for comparative purposes. Although children with craniosynostosis begin life with significantly smaller orbital volumes, overall normal mean volumes for both sexes are attained by 13 months of age, with volumes approaching normal by 6 months of age in male infants and by 8 months of age in female infants. Changes in orbital volume associated with age generally appear to be similar in most forms of craniosynostosis. There appears to be no significant difference in changes in orbital volume between children with syndromic or nonsyndromic forms of bicoronal synostosis. Orbital volume is significantly reduced on the ipsilateral affected side in cases of unicoronal synostosis in comparison with the contalateral side, but it is not significantly lower than that of normal. Finally, FOA surgery appears to restore normal growth of orbital volume. CONCLUSIONS: The results of this study indicate that the underlying mechanism leading to craniosynostosis and restriction of orbital volume "burns out" and begins to lose its major effects within the first few months of life. It would appear that FOA surgery should be delayed until the end of the second half of the 1st year of life, thus maximizing the effects of accelerated normal orbital growth and reducing the risks of relapse.

Aging↗
PubMed2002

A novel fibroblast growth factor receptor 2 mutation in Crouzon syndrome associated with Chiari type I malformation and syringomyelia.

OBJECT: It has been reported that due to premature synostosis of the lambdoid suture in the first 24 months of life, more than 70% of patients with Crouzon syndrome concurrently suffer from chronic tonsillar herniation (Chiari Type I malformation) and some (20%) associated syringomyelia. The goal of the present study was to examine mutations in the fibroblast growth factor receptor (FGFR) genes in Crouzon syndrome and its related conditions. METHODS: Five patients were studied: three with Crouzon syndrome (one sporadic and two familial), one with sporadic Chiari I with syringomyelia, and one with unilateral lambdoid synostosis. Deoxyribonucleic acid was screened for FGFR1-3 mutations by using single-strand conformational polymorphism and subsequent direct sequencing. Two types of missense mutations were detected in the FGFR2 gene, Cys342Trp (1205, TGC --> TGG) in a patient with sporadic Crouzon syndrome and Tyr281Cys (1021, TAC --> TGC) in two siblings (brother and sister) with familial Crouzon syndrome, respectively. The former has been reported only in sporadic cases but the latter has not previously been identified. A polymorphism in the FGFR3 gene, Asn294Asn (882, AAT --> AAC), was also detected in three patients. No mutation was found in the patient with sporadic Chiari I with syringomyelia. CONCLUSIONS: The FGFR2 missense mutation was detected in Crouzon syndrome but not in sporadic Chiari I with syringomyelia or lambdoid synostosis. A novel FGFR2 mutation, Tyr281Cys, was found in familial Crouzon syndrome with Chiari I and syringomyelia. It may be informative to look for this in patients with Crouzon syndrome and associated syringomyelia.

Adolescent↗
PubMed2004

Endoscopy-assisted wide-vertex craniectomy, barrel stave osteotomies, and postoperative helmet molding therapy in the management of sagittal suture craniosynostosis.

OBJECT: Endoscopic techniques were introduced 7 years ago for the surgical management of patients with sagittal synostosis. In this study of 139 patients with sagittal synostosis, the authors assessed the efficacy, safety, complications, and outcomes after performing endoscopy-assisted wide-vertex craniectomies with bitemporal and biparietal barrel stave osteotomies. METHODS: The sample population consisted of a total of 99 boys and 40 girls who ranged in age from 0.4 to 9.2 months (mean 3.6 months). Two small incisions were made near the lambda and vertex. Using endoscopic visualization, wide-vertex craniectomies with bilateral temporal and parietal barrel stave osteotomies were performed. Postoperative treatment included custom-made surlyn cranial orthotic devices for cranial reshaping and maintenance. The mean craniectomy width was 5.4 cm and the length was 10 cm. The overall blood transfusion rate was 9% (two intraoperative and 12 postoperative transfusions). The mean estimated blood loss was 29 ml (range 5-150 ml). The mean preoperative hematocrit was 32%, whereas the postoperative level was 27%. One hundred thirty-two patients were discharged the morning following surgery. The majority of patients did not experience facial swelling, and none suffered postoperative fevers. Anthropometric cephalic index measurements indicated that excellent results were obtained in 87% of the patients (cephalic index > 75); good results in 8.7% (cephalic index 70-75); and poor results in 4.3% (cephalic index > 70). There were no cases of intraoperative death, infection, hemorrhage, or venous sinus injury. CONCLUSIONS: Analysis of the results indicates that use of the aforedescribed procedure in the early treatment of infants with sagittal synostosis provides excellent outcomes and that the morbidity rate is lower than that associated with traditional cranial vault reconstruction. Detailed anthropometric and radiographic analyses demonstrated that with adequate helmet therapy in our patients normocephaly was achieved and maintained without the need for secondary operations.

Blood Loss, Surgical↗
PubMed2004

Nonsynostotic scaphocephaly: the so-called sticky sagittal suture.

OBJECT: Scaphocephaly is a common craniofacial abnormality that results from craniosynostosis of the sagittal suture. The authors have treated a group of infants who presented with nonsynostotic scaphocephaly, or a so-called sticky sagittal suture. The purpose of this study was to describe these patients with nonsynostotic scaphocephaly, the natural history of the disease, and its treatment. METHODS: At the University of Texas-Houston Medical School between 1996 and 2002, nine patients presented with nonsynostotic scaphocephaly. When the abnormality in patients was diagnosed prior to 12 months of age, the majority (seven of eight) were successfully managed by molding helmet therapy. The only child in this group in whom this therapy failed to correct the deformity was noted on repeated computerized tomography scanning to have true sagittal synostosis, which required surgical correction. One child with nonsynostotic scaphocephaly, who presented after 1 year of age, required surgical correction followed by postoperative molding helmet therapy. CONCLUSIONS: Patients with nonsynostotic scaphocephaly appear to have sagittal synostosis. If treated early (at < 12 months of age), head shape in these patients can be normalized by molding helmet therapy. Surgery is reserved for older patients (> 12 months of age) or those with sagittal synostosis.

Cranial Sutures↗
PubMed2005

Tibialization of the fibula: a viable option to salvage limbs with extensive scarring and gap nonunions of the tibia.

I retrospectively reviewed 21 patients who had tibialization of the fibula for infected nonunions with scarring of soft tissues. Most of the patients had unsuccessfully had other operations to restore continuity of the tibia before this treatment. The prerequisites were adequate vascularity, an intact sensate sole, and intact fibula. Proximal site tibiofibular synostosis was done in all patients. Three patients required a supplementary procedure at the proximal tibiofibular junction because of screws cutting out. Distal tibiofibular synostosis was done as a second-stage procedure in a majority of the patients 3 to 6 weeks after the proximal procedure. Protected weightbearing was recommended for 4 to 8 months. The transplanted fibula hypertrophied and approached the diameter of the tibia (or double the size of original fibula) in 2-3 years. Tibialization of the fibula is a safe, nondestructive, salvage procedure for treating difficult infected nonunions of the tibia. It is a simple technique that can be done in hospitals with a moderate infrastructure. Despite scarring, shortening, and limitation of knee and ankle motion, the patients were satisfied to be able to take part in normal daily activities on their own. After the success of synostosis, all patients engaged in activities of daily living and during the followup of 4-14 years none developed stress fracture of the tibialized fibula.

Adolescent↗
PubMedDate not supplied

Operatory indications and licence of craniostenosis.

On average 0.04% of the population is affected by craniostenosis. It is believed there are anatomo-clinical forms at moderate risk and others at high risk. The neurosurgical indications are very different according to various Authors. The forms at high risk which are caused by an isolated synostosis of the coronal system with brachicephalia are either associated to other synostosis (oxicephalia) or to facial synostosis should always be operated, with various techniques, within the first 6 months of life. Basing on the experience of 3 cases of craniostenosis, deliberately not operated on, followed and controlled for over 20 years from the decision of not to operate, all in excellent general condition both neurological and psychiatric, the Authors consider and discuss the criteria of surgical indications with particular regard to clinical objectivity (implicity disregarding a precise value of the craniometric data).

Adolescent↗