• Journal of Korean Neurosurgical Society
• /
• 제59권3호
• /
• pp.204-213
• /
• 2016

Syndromic craniosynostosis has severe cranial stenosis and deformity, combined with hypoplastic maxillary bone and other developmental skeletal lesions. Among these various lesions, upper air way obstruction by hypoplastic maxillary bone could be the first life-threatening condition after birth. Aggressive cranial vault expansion for severely deformed cranial vaults due to multiple synostoses is necessary even in infancy, to normalize the intracranial pressure. Fronto-orbital advancement (FOA) is recommended for patients with hypoplastic anterior part of cranium induced by bicoronal and/or metopic synostoses, and posterior cranial vault expansion is recommended for those with flattening of the posterior part of the cranium by lambdoid synostosis. Although sufficient spontaneous reshaping of the cranium can be expected by expansive cranioplasty, keeping the cranial bone flap expanded sufficiently is often difficult when the initial expansion is performed during infancy. So far distraction osteogenesis (DO) is the only method to make it possible and to provide low rates of re-expansion of the cranial vault. DO is quite beneficial for both FOA and posterior cranial vault expansion, compared with the conventional methods. Associated hydrocephalus and chronic tonsillar herniation due to lambdoid synostosis can be surgically treatable. Abnormal venous drainages from the intracranial space and air way obstruction should be always considered at any surgical procedures. Neurosurgeons have to know well about the managements not only of the deformed cranial vault and the associated brain lesions but also of other multiple skeletal lesions associated with syndromic craniosynostosis, to improve treatment outcome.

• 대한두개안면성형외과학회지
• /
• 제15권2호
• /
• pp.47-52
• /
• 2014

Background: Management of positional plagiocephaly by wearing a cranial molding helmet has become a matter of growing medical interest. Some research studies reported that starting helmet therapy early (age 5 to 6 months) is important and leads to a significantly better outcome in a shorter treatment time. The aim of the present study was to evaluate the effectiveness of cranial remodeling treatment with wearing helmet for older infants (${\geq}18$ months). Methods: We conducted a retrospective study of 27 infants with positional plagiocephaly without synostosis, who were started from 2008 to 2012. Every child underwent a computerized tomography (CT) before starting helmet therapy to exclude synostosis of the cranial sutures and had CT performed once again after satisfactory completion of therapy. Anthropometric measurements were taken on using spreading calipers in every child. The treatment effect was compared using cranial vault asymmetry (CVA) and the cranial vault asymmetry index (CVAI), which were obtained from diagonal measurements before and after therapy. Results: The discrepancy of CVA and CVAI of all the patients significantly decreased after cranial molding helmet treatment in older infants (${\geq}18$ months) 7.6 mm from 15.6 mm to 8 mm and 4.51% from 9.42% to 4.91%. Six patients had confirmed successful outcome, and all subjects were good compliance patients. The treatment lasted an average of 16.4 months, was well tolerated, and had no complication. Additionally, the rate of the successful treatment (final CVA ${\leq}5mm$) significantly decreased when the wearing time per was shorter. Conclusion: This study showed that treatment by cranial remodeling orthosis was effective if the patient could wear the helmet longer and treatment duration was somewhat longer than in younger patients, well tolerated in older infants and had no morbidity. This therapeutic option is available and indicated in these older infants before other cranial remodeling surgery.

• Journal of Korean Neurosurgical Society
• /
• 제59권3호
• /
• pp.192-196
• /
• 2016

Understanding the development of a skull deformity requires an understanding of the normal morphogenesis of the cranium. Craniosynostosis is the premature, pathologic ossification of one or more cranial sutures leading to skull deformities. A review of the English medical literature using textbooks and standard search engines was performed to gather information about the prenatal development and growth of the cranial vault of the neurocranium. A process of morphogenic sequencing begins during prenatal development and growth, continues postnatally, and contributes to the basis for the differential manner of growth of cranial vault bones. This improved knowledge might facilitate comprehension of the pathophysiology of craniosynostosis.

• Archives of Plastic Surgery
• /
• 제38권3호
• /
• pp.222-227
• /
• 2011

Purpose: Autogenous particulate bone grafting is a type of autogenous bone graft that consists of small particles of cortical and cancellous bone. Autogenous particulate bone grafting has been used for calvarial bone defect after calvarial defect of craniosynostosis and prevention of temporal depression after fronto-orbital advancement. The results were followed up and studied for effectiveness of autogenous calvarial particulate bone grafting. Methods: Cranial vault remodeling and fronto-orbital advancement was performed for six craniosynostosis patient from August 2005 to October 2007. Autogenous particulate bone grafting was harvested from endocortex of separated cranial vault and if insufficient, from extocortex of occipital region using Hudson brace & D'Errico craniotomy bit and was grafted on the calvarial bone defect of cranial vault and temporal hollow. Fibrin glues were added to the harvested particulated bone for adherence and shaping of paticles. Results: Autogenous particulate bone grafting was followed-up at least longer than I year. The calvarial bony defects following primary cranial remodeling were successfully covered and postoperative temporal depressions after fronto-orbital advancement were also well prevented by grafted particulated bone. Conclusion: Autogenous calvarial particulate bone graft can be harvested in infants and young children with minimal donor site morbidity. It effectively heals cranial defects in children and during fronto-orbital advancement reduces the prevalence of osseous defects independent of patient age. It's easy and effective method of reconstruction of calvarial defect.

• 대한두개안면성형외과학회지
• /
• 제15권3호
• /
• pp.109-116
• /
• 2014

Background: Infants with deformational plagiocephaly (DP) usually present with cranial vault deformities as well as facial asymmetry. The purpose of this study was to use three-dimensional anthropometric data to evaluate the influence of cranial deformities on facial asymmetry. Methods: We analyzed three-dimensional computed tomography data for infants with DP (n=48) and without DP (n=30, control). Using 16 landmarks and 3 reference planes, 22 distance parameters and 2 angular parameters were compared. This cephalometric assessment focused on asymmetry of the orbits, nose, ears, maxilla, and mandible. We then assessed the correlation between 23 of the measurements and cranial vault asymmetry (CVA) for statistical significance using relative differences and correlation analysis. Results: With the exception of few orbital asymmetry variables, most measurements indicated that the facial asymmetry was greater in infants with DP. Mandibular and nasal asymmetry was correlated highly with severity of CVA. Shortening of the ipsilateral mandibular body was particularly significant. There was no significant deformity in the maxilla or ear. Conclusion: This study demonstrated that the cranial vault deformity in DP is associated with facial asymmetry. Compared with the control group, the infants with DP were found to have prominent asymmetry of the nose and mandible.

• Journal of Korean Neurosurgical Society
• /
• 제59권3호
• /
• pp.233-241
• /
• 2016

In this review, we discuss in detail our current procedure for treating craniosynostosis using multidirectional cranial distraction osteogenesis (MCDO). The MCDO method allows all phenotypes of skull deformity to be reshaped by distraction osteogenesis, except in patients who are 5 months of age or younger and patients with posterior cranial vault problems. We report the results of clinical data of 36 children with craniosynostosis who underwent MCDO between 2005 and 2014 in our institute. This method has the following benefits, such as a high flexibility of reshaping, shorter treatment period and less invasive secondary intervention. We also discuss the other distraction osteogenesis techniques that are used to treat craniosynostosis and compare them with MCDO. The preferred procedure for correction of craniosynostosis may depend on the patient's age, the extent of deformity, and the extent of correction achievable by surgery. We can arrange the combinations of various methods according to the advantage and disadvantage of each technique.

• Archives of Plastic Surgery
• /
• 제32권1호
• /
• pp.29-36
• /
• 2005

Craniosynostosis is the premature fusion of one or more sutures of either cranial vault or base. Fused sutures may impede normal growth of the calvaria, leading to characteristic skull deformities; Morphological craniosynostosis is classified descriptively. Being craniosynostosis uncorrected the deformity progresses continuously and causes an increase of intracranial pressure. The surgical involvement aims at the expansion of intracranial space as well as satisfactory achievement of craniofacial shape. Early surgical correction in infancy prevents the deformity from the further progression and possible associated complication of high intracranial pressure. A long period of follow-up is essential to asses the outcome of an effectiveness of the surgery. measurement of intracranial volume has been concerned in medical personnel and anthropologists for many years. A reliable and accurate measurements of the intracranial volume facilitates to make a diagnosis and treatment of craniosynostosis. Pre-and postoperative change of intracranial volume was evaluated with 3D CT scanning in 12 cases of craniosynostosis who underwent frontal advancement and total cranial vault remodeling. Increased intracranial volume is attributed to surgical release of craniosynostosis and natural growth. We conceive that the intracranial volume is significantly increased after surgical correction of fused cranial sutures and along with natural growing. A procedure of frontal advancement and total cranial vault remodeling is very useful to correct such a deformity as craniosynostosis. And also 2 cases out of five mentally retarded patients improved remarkably and Forehead retrusion or temporal depression followed in another two cases.

• 한국임상수의학회지
• /
• 제17권2호
• /
• pp.470-474
• /
• 2000

A Shih Tzu Puppy had clinical onset of anotexia crying and progression of neurological sings when enlargement of the cranial vault at 1 month old and died after showing clinical signs during 2 months period. Radiological and pathological examinations were performed. Radiological findings were homogeneous appearance of the calvaria with cortical thinning, loss of the normal convolutional skull markings and persistent fontanelles. Grossly enlargement of the cranial vault thinning of the bone and defective closure of the fontanelles were also observed. The entire subcortical area of the cerebral hemispheres with severe, dilatation of ventricles and cerebrospinal fluid(CSF) wits absent. There was parenchyma atrophy affecting chiefly in the white mater and the cerebral cortices, axon degeneration and necrosis and gitter cell infiltration in the whiter matter and the subependymal area. Mononuclear perivacular cuffing in the cerebrum and the pons was shown. Based on the radiological, gross and histopathological findings, this case was believed to have congenital hydrocephalus with nonsuppurative encephahitis. Possible etiology on the case is also discussed.

• 대한두개안면성형외과학회지
• /
• 제23권2호
• /
• pp.59-63
• /
• 2022

Background: Deformational plagiocephaly is usually managed conservatively, as it tends to improve over time and with the use of conservative measures. However, before the year 2017 we operated on patients with severe plagiocephaly and neurological symptoms at the Helsinki Cleft Palate and Craniofacial Center. Methods: Of the 20 infants with severe deformational plagiocephaly and neurological symptoms referred to us between 2014 and 2016, 10 underwent cranioplasty open reshaping of the posterior cranial vault. The parents of the last 10 patients were given information on the natural history of the condition and the patients were followed up with an outpatient protocol. The aim of this study was to gain information on the brain electrophysiology and recovery of patients after total cranial vault reconstruction by measuring the electroencephalogram (EEG) somatosensory evoked potentials (SEP; median nerve). Results: Of the 10 participants in the operation arm, six had abnormal SEP at least on the affected cerebral hemisphere and all SEPs were recorded as normal when controlled postoperatively. In the follow-up arm, eight out of 10 participants had abnormal SEP at the age of approximately 24 months, and all had normalized SEPs at control visits. Conclusion: Our data suggest that cranioplasty open reshaping of the posterior cranial vault did not affect abnormal SEP-EEG recordings. We have abandoned the operations in deformational plagiocephaly patients due to findings suggesting that expanding cranioplasty is not beneficial for brain function in this patient group.

• Archives of Plastic Surgery
• /
• 제33권2호
• /
• pp.237-240
• /
• 2006

The simultaneous correction of the hypertelorism and exophthalmos combined with craniosynostosis is very rarely performed operative procedures in the world. The craniosynostosis is the congenital anomaly that designates premature fusion of one or more sutures in either cranial vault or cranial base. Hypertelorism is not a distinct clinical syndrome in itself, but is a physical finding secondary to facial and cranial maldevelopment and it is defined as a increase in the distance between the medial orbital walls. Exophthalmos can occur following the decrease in the size of the orbit in patients with developmental skeletal disorders such as craniofacial synostosis. The authors experienced 9-year-old male patient, who has complex cranio-facial abnormality. The craniosynostosis was oxycephaly type and primary fronto-orbital advancement surgery had been performed in other hospital. The abnormal cranial vault combined with hypertelorism and exophthalmos due to maldeveloped both orbital walls. Surgical correction was obtained by various cranio-fronto-orbital remodeling technique such as calvarial bone craniotomy, fronto-orbital advancement, paramedian resection, medial canthopexy, Tessier-Wolfe three wall orbital expansions. We achieved a quite satisfactory result both functionally and aesthetically in a complex cranio-facial deformity patient by combination and modification of previously developed various cranio-facial plasty technique and hereby report the case with brief discussion and review of literature.