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PubMed1999

Unilateral coronal synostosis treated by internal forehead distraction.

A 1-year-old infant with left hemicoronal synostosis was treated by distraction osteogenesis of the craniofacial skeleton using an internal distraction device. Surgery was performed through a coronal incision. The frontal bone and upper half of both orbits were first osteotomized en bloc after minimal epidural dissection of the supraorbital area and no epidural dissection around the coronal osteotomy site. The lateral one fourth of the frontal bone, including the right lateral half of the orbit, was left intact. The internal distraction device was fixed in the left temporal area. A 0.5-mm per day rate of distraction was performed up to an elongation of 17 mm after a 5-day latency period. The distraction device was removed after a consolidation period of 2 months. The results obtained were satisfactory, with symmetry of the forehead, orbit, and nose achieved without complications. The merits of this procedure are no extradural dead space after the operation (which prevents infection), shortened operative time, reduced blood loss, filling in the bone gap created by advancement with new bone, acceptable cosmesis by the parents during distraction, and no fixation device left after the second operation.

Craniosynostoses↗
PubMed2002

Surgical treatment of aplasia cutis congenita of the scalp associated with bilateral coronal synostosis.

Aplasia cutis congenita is a rare condition characterized by the congenital absence of skin. The authors report a case of aplasia cutis congenita of the scalp associated with bilateral coronal synostosis. Simultaneous fronto-orbital advancement and skull reconstruction for large defect at the fontanelle were performed in the initial operation. A tissue expander made it possible to resect most of the hairless scar and totally cover the reconstructed defect and skull. During the second stage, the residual hairless scar was completely covered with hair-bearing scalp by tissue expansion and the residual skull defects were successfully reconstructed with split calvarial bone grafting.

Alopecia↗
PubMed2002

Molding helmet therapy in the management of sagittal synostosis.

The current surgical treatment of sagittal craniosynostosis often fails to produce a normal cephalic index; however, recent reports of early endoscopic suture release and postoperative helmet therapy promise improved cranial symmetry. The role of helmet therapy is critical in this approach, but there are few reports on the use of helmet therapy after craniosynostosis surgery. The authors present a retrospective review comparing the results of surgery alone versus surgery and postoperative banding in treating children diagnosed with sagittal synostosis. Cephalic index (CI) measurements and the divergence of the CI from the norm (DFN) were used to compare the banded and the non-banded groups. Included in the study were 21 children with sagittal craniosynostosis of whom 6 were treated with surgery alone and 15 were treated with surgery and postoperative banding. Preoperatively, both groups were significantly different from the norm (non-banded, P < 0.05; banded, P < 0.01), while postoperatively they were not significantly different from the norm. Statistically significant changes were present in the CIs of the banded group in the preoperative-follow-up interval (P < 0.01), while no significant changes occurred in the non-banded group during this same interval. Statistically significant changes in the DFN occurred in the preoperative-postoperative and preoperative-follow-up intervals of the banded group; however, these changes were not significant in the non-banded group during the same intervals. Correction toward a normal CI was seen in the banded group throughout the course of treatment, while this trend was not present in the non-banded group. Therefore, molding helmet therapy maintains the operative correction obtained and promotes more normal cranial growth patterns.

Cephalometry↗
PubMed1986

Experimental unilateral coronal synostosis in rabbits.

Immobilization of the coronal suture was produced unilaterally in 9-day-old rabbits to determine its effect on subsequent craniofacial development. The suture was immobilized unilaterally by the topical application of methylcyanoacrylate adhesive. Subsequent growth effects on the cranial vault, base, and facial skeleton were assessed by serial radiographic cephalometry. Unilateral coronal suture immobilization resulted in significantly decreased bone growth at the coronal suture (mean 0.95 mm +/- 0.35 SE) when compared to sham-treated control animals (mean 5.06 mm +/- 0.20 SE). Frontonasal suture bone growth contralateral to the immobilized half of the coronal suture, however, was significantly increased. The anterior cranial base became significantly shortened, and orbital asymmetry developed. The pattern of induced abnormalities simulates unilateral coronal synostosis in humans.

Animals↗
PubMed1988

Lambdoid synostosis: surgical considerations.

Premature closure of the lambdoid suture may result in abnormalities in skull shape not only in the parieto-occipital region, but also in the frontal region and at the vertex of the skull. Although most of these abnormalities are mild, some constitute significant abnormalities in shape and therefore require specific surgical correction. In this report, we describe our approach from five patients we have treated and followed for 6 to 24 months with abnormalities characteristic of unilateral and bilateral lambdoid synostosis. We describe our approach to the simultaneous correction of frontal, occipital, and abnormal vertical height skeletal abnormalities in one operative procedure. To date, no negative neurologic sequelae have resulted from this operative approach, but significant improvement in skull form has been appreciated.

Craniosynostoses↗
PubMed2005

Correction of sagittal synostosis using foreshortening and lateral expansion of the cranium activated by gravity: surgical technique and postoperative evolution.

BACKGROUND: The authors present a technique for correction of sagittal synostosis addressing the transverse constriction and providing foreshortening of the skull without the use of hardware. The design of the osteotomies combined with strict postoperative supine positioning allows foreshortening of the skull, with concomitant expansion. METHODS: Twenty-two patients were operated on using the foreshortening and lateral expansion activated by gravity technique. Postoperative follow-up included clinical evaluation and three-dimensional computed tomographic scanning at 4 to 6 weeks, 1 year, and yearly afterward. These scans were analyzed using three-dimensional surface analysis software, according to a protocol the authors describe in this article. RESULTS: The cephalic index improved from 66.4 preoperatively to 74.5 at 1 year postoperatively (normal, 72 to 87). Transverse growth continued to be the dominant vector of growth up to 1 year (until ossification-bridging of the vertex craniectomy). Beyond 1 year, the main area of transverse growth was overlying the temporal suture, producing a distinctive temporal bossing in patients. The parietal areas showed mini-mal growth afterward. Growth was mainly occipital between 1 and 3 years of age, and frontal afterward, up to 5 years of age. A low frontal bossing also occurred in some of our patients and resolved spontaneously. CONCLUSIONS: The authors have described a technique for correction of scaphocephaly that avoids the use of hardware, with acceptable operative times and transfusion needs. Postoperative improvement in shape is satisfactory. Analysis of the three-dimensional scans of these patients shows abnormal skull growth patterns up to 5 years of age.

Cephalometry↗
PubMed2006

Changes in anthropometric values of paired craniofacial measurements of patients with right coronal synostosis.

Quantitative changes of the 5 paired (right and left) projective linear measurements of the craniofacial complex were analyzed by anthropometric methods in 26 patients (9 males and 17 females) with right coronal synostosis, 16 in infancy (0-5 and 6-12 months) and 10 in early childhood (1-10 years). Measurements were taken from 3 regions: the supraorbital rim length of the head (t-g), the length (en-ex) and height (ps-pi) of the eye fissures in orbits, and the width (pra-pa) and length (sa-sba) of the ears. None of the patients had undergone corrective surgery.Anthropometric analysis of patients in infancy showed a large percentage of subnormal supraorbital rim lengths (t-g) on the right side of the head, with a higher frequency in females (90.9%) than in males (80.0%). Eye fissure lengths (en-ex) were subnormal more often in males than in females on both sides, while heights were generally normal bilaterally in both sexes (72.7-100% of cases). Ears of normal width (pra-pa) and length (sa-sba) were more frequent in females. In males, the frequency of subnormal ear widths and lengths surpassed those in females. The most remarkable changes between infancy and early childhood were noted in the orbital region, in which subnormal eye fissure lengths became bilaterally normal in both sexes.

Anthropometry↗
PubMed2005

The bowstring canthal advancement: a new technique to correct the flattened supraorbital rim in unilateral coronal synostosis.

Patients with unilateral coronal synostosis characteristically have a recessed and flattened supraorbital rim ipsilateral to the fused suture. Despite lateral canthal advancement procedures to correct these anomalies, patients often have a persistent flattened and recessed supraorbital rim after surgery. Current procedures address the pathologic features of the orbital rim only partially by advancing forward a deformed supraorbital rim without correcting the abnormal flattening of the normal rim curvature. The authors describe a technique modification of the supraorbital rim advancement procedure that addresses not only the lack of anterior positioning of it, but also its flattened contour: the bowstring canthal advancement.

Craniosynostoses↗
PubMed2005

Anterior ischemic optic neuropathy complicating cranial vault reconstruction for sagittal synostosis in a child.

The authors report a case of postoperative visual loss in a pediatric patient who underwent an uneventful cranial vault reconstruction. A 5-year-old boy underwent total cranial vault reconstruction for nonsyndromic sagittal synostosis. He was positioned prone for 6 hours and then supine for the final 2 hours of surgery. Controlled hypotension was used to maintain the systolic arterial pressure at 80 mmHg to 90 mmHg. The intraoperative hematocrit was 23% to 31%. In the immediate postoperative period, both pupils were noted to be reactive to light. After an uneventful night in the intensive care unit, he was transferred to the ward. The eyelids were edematous on postoperative day 1, and visual examination was no longer possible. He was discharged home on postoperative day 4, and readmitted 2 days later with bilateral blindness. The pupils were nonreactive, and the optic discs were pale and edematous. Computed tomography and magnetic resonance imaging of the head revealed no abnormality. Intravenous steroid therapy was initiated, and the patient was taken to the operating room for re-exploration; the intracranial pressure was found to be normal. The presumed diagnosis was bilateral infarctions of the anterior optic nerves. Visual acuity 14 months after surgery was less than 3/200 in the right eye and 20/20-2 in the left, indicating significant recovery. Pediatric patients undergoing cranial vault reconstruction are at risk for postoperative visual loss because of ischemic optic nerve injury. Prolonged duration in the prone position and significant blood loss may be risk factors.

Blindness↗
PubMed2005

Relationship of brain and skull in pre- and postoperative sagittal synostosis.

Models of vertebrate skull evolution stress the coordinated developmental relationship between the skull and the brain that it houses. This study investigates the relationship between altered skull morphology and brain morphology in premature fusion of the cranial sagittal suture (isolated sagittal synostosis; ISS), a condition associated with dysmorphology of both neurocranium and brain. Although the skull displays a more normal shape following reconstructive cranial vault surgery, effects of this surgery on the brain have not been investigated. Landmark coordinate data were collected from three-dimensional magnetic resonance imaging reconstructions of the brain in a sample of ISS patients and an age-matched unaffected cohort. These data were analysed using Euclidean distance matrix analysis (EDMA). Results show that the brain in ISS is dysmorphic preoperatively, displaying a posteriorly directed neural expansion that does not 'worsen' with growth. Postoperatively, the brain in ISS displays a more globular shape overall as compared with the preoperative morphology, but differs from normal in its subcortical morphology. These results show that the ISS brain is altered following neurocranial surgery, but does not more closely approximate that of unaffected individuals. This suggests that although the brain is affected by manipulation of the skull, it retains a growth pattern that is, at least in part, independent of the skull.

Brain↗
PubMed1984

Premature cranial synostosis in X-linked hypophosphatemic rickets: possible precipitation by 1-alpha-OH-cholecalciferol intoxication.

A child suffering from X-linked hypophosphatemic rickets developed vitamin D intoxication under treatment with 1-alpha-OH-cholecalciferol (1(OH)D3) and phosphorus. Beside the usual findings in this condition he showed precocious synostosis of the skull with signs of raised intracranial pressure. In view of earlier reports of coincidence of craniostenosis and X-linked hypophosphatemic rickets, we conclude that the possibility exists that intoxication with 1(OH)D3 has been the precipitating factor. In addition we found hypersensitivity to 1(OH)D3 2 months after cessation of treatment, and normal levels of calcitriol (1,25(OH)2D3) at the same time.

Cranial Sutures↗
PubMed2005

Pre- and postoperative developmental attainment in sagittal synostosis.

AIMS: To investigate whether sagittal synostosis (SS) has consequences for children's mental and psychomotor development, and whether surgery has any impact on this. METHODS: The study involved 28 children with SS who underwent corrective surgery at a mean age of 8.0 (SD 7.16) months, and 28 normal controls. All the children with SS were assessed pre- and postoperatively using the Griffiths Mental Development Scales. The controls were assessed on one occasion, at an age matched with individuals in the patient group at the time of the preoperative assessment. A further control group consisted of 13 children with SS, who had received developmental assessment on two or more occasions without surgical intervention. RESULTS: The data indicated that children with SS have significantly poorer gross locomotor function than the normal controls. Following surgical intervention the deficit was shown to have resolved; consistent with this a lesser improvement in eye-hand coordination and performance skills was shown. Overall developmental attainment also improved postoperatively. The children with SS who did not receive surgery did not show any improvement in development. CONCLUSIONS: The study shows improved developmental attainment following surgical correction of SS, which may therefore be more than a cosmetic procedure.

Analysis of Variance↗
PubMed1977

A new syndrome of cleft palate associated with coloboma, hypospadias, deafness, short stature, and radial synostosis.

A new syndrome characterized by cleft palate, coloboma, hypospadias, deafness, short stature, and radial synostosis has been described. The family history suggests either an autosomal dominant mode of inheritance with limited expression in females or X-linkage. Other syndromes with similar phenotypes and modes of inheritance are discussed. The need for accurate and complete family histories in cases involving cleft palate and cleft lip/palate is discussed in relation to genetic counselling and recurrent risk estimates.

Abnormalities, Multiple↗
PubMed2003

A late result of an early operation for unilateral coronal synostosis.

An infant underwent strip craniectomy for unilateral coronal synostosis in 1967. This report presents the cosmetic, functional and psychosocial outcome at 34 years of age. Although aggressive, reconstructive treatment of the various forms of single-suture craniosynostosis is the current standard of care, neither the natural history of these conditions nor the long-term results of treatment have been documented systematically.

Adult↗
PubMed1994

Occipital reduction-biparietal widening technique for correction of sagittal synostosis.

In the older infant with a marked skull deformation from sagittal synostosis a midline craniectomy will not produce an optimal cosmetic result as the brain grows at a much reduced rate and cannot provide the impetus for passive correction of the deformity. An operative technique is described whereby the occipital prominence is reduced, the biparietal diameter widened, and the height of the calvarium lowered at the vertex. Although the frontal bossing is not directly addressed the changes in the remainder of the calvarium render the bossing of little cosmetic relevance and has not been a long-term problem in the group of patients treated with this technique. The amount of manipulation, operative time, and blood loss with the procedure is comparable to others reported. This technique does not have the potential to raise intracranial pressure if one takes care to avoid undue compression on the major dural venous sinuses. There have been no significant bony defects and no patient has required reoperation. The cosmetic results have been excellent.

Cephalometry↗
PubMed1996

Occipital plagiocephaly: deformation or lambdoid synostosis? I. Morphometric analysis and results of unilateral lambdoid craniectomy.

Between 1987 and 1992, 30 infants aged 1.4-13 months (mean 7.3 months) underwent unilateral lambdoid strip craniectomy at the Children's Hospital of Buffalo for occipital plagiocephaly. Males outnumbered females (22:8) and right-sided occipital flattening was significantly more common than left-sided flattening (25:5). The deformity was noticed at an average age of 3.2 months; 16% of the infants had an asymmetry at birth. Positional preferences (a distinct tendency to lie preferentially on the back, in most cases with the head turned to the ipsilateral side) were described in 79% of infants for whom this information was available, and torticollis was present in 10%. Pre- and post-operative CT scans were analyzed using several morphometric measurements. Asymmetries were measured between the flattened and contralateral sides, both posteriorly and anteriorly, using a translucent grid placed over the CT slice showing maximum asymmetry. The average maximum asymmetry between the flattened and contralateral sides was 24% posteriorly and 16% anteriorly. Significant improvements were seen postoperatively, with both anterior and posterior asymmetries improving by an average of one third (p < 0.05). However, when compared with CT scans from a control group of infants without synostosis, the operated group showed persistent and significant asymmetries postoperatively. The morphometric measurements described allow an objective and reproducible means of assessing the results of various treatments for this disorder. The improvements following unilateral lambdoid craniectomy are difficult to interpret in isolation; we suggest that future efforts be directed toward similarly assessing the results of both nonoperative treatments such as positional changes and molding helmets, and more aggressive surgical treatments that have been advocated for this disorder.

Craniofacial Abnormalities↗