• Archives of Plastic Surgery
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• v.32 no.2
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• pp.175-182
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• 2005

Skull base tumors have been determined inoperable because it is difficult to accurately diagnose the extent of the involvement and to approach and excise the tumor safely. However, recently, the advent of sophisticated diagnostic tools such as computed tomography and magnetic resonance imaging as well as the craniofacial and neurosurgical advanced techniques enabled an accurate determination of operative plans and safe approach for tumor excision. Resection of these tumors may sometimes result in massive and complex extirpation defects that are not amenable to local tissue closure. The purpose of this study is to analyze experiences of skull base reconstruction and to evaluate long term survival rate and complications. All cranial base reconstructions performed from July 1993 to September 2000 at Department of Plastic and Reconstructive Surgery of the Seoul National University Hospital were observed. The medical records were reviewed and analysed to assess the location of defects, reconstruction method, existence of the dural repair, history of preoperative radiotherapy and chemotherapy, complications and causes of death of the expired patients. There were 12 cases in region II, 8 cases in region I and 1 case in region III according to the Irish classification of skull base. Cranioplasty was performed in 4 patients with a bone graft and microvascular free tissue transfer was selected in 17 patients to reconstruct the cranial base and/or mid-facial defects. Among them, 11 cases were reconstructed with a rectus abdominis musculocutaneous free flap, 2 with a latissimus dorsi muscluocutaneous free flap, 1 with a fibular osteocutaneous free flap, 2 with a scapular osteocutaneous free flap, and 1 with a forearm fasciocutaneous free flap, respectively. During over 3 years follow-up, 5 patients were expired and 8 lesions were relapsed. Infection(3 cases) and partial flap loss(2 cases) were the main complications and multiorgan failure(3 cases) by cancer metastasis and sepsis(2 cases) were causes of death. Statistically 4-years survival rate was 68%. A large complex defects were successfully reconstructed by one-stage operation and, the functional results were also satisfactory with acceptable survival rates.

• Archives of Plastic Surgery
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• v.43 no.2
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• pp.204-209
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• 2016

Temporal hollowing occurs to varying degrees after pterional craniotomy. The most common cause of temporal hollowing is a bony defect of the pterional and temporal regions due to the resection of the sphenoid ridge and temporal squama for adequate exposure without overhang. The augmentation of such bony defects is important in preventing craniofacial deformities and postoperative hollowness. Temporal cranioplasty has been performed using a range of materials, such as acrylics, porous polyethylene, bone cement, titanium, muscle flaps, and prosthetic dermis. These methods are limited by the risk of damage to adjacent tissue and infection, a prolonged preparation phase, the possibility of reabsorption, and cost inefficiency. We have developed a method of temporal augmentation using a calvarial onlay graft as a single-stage neurosurgical reconstructive operation in patients requiring craniotomy. In this report, we describe the surgical details and review our institutional outcomes. The patients were divided into pterional craniotomy and onlay graft groups. Clinical temporal hollowing was assessed using a visual analog scale (VAS). Temporal soft tissue thickness was measured on preoperative and postoperative computed tomography (CT) studies. Both the VAS and CT-based assessments were compared between the groups. Our review indicated that the use of an onlay graft was associated with a lower VAS score and left-right discrepancy in the temporal contour than were observed in patients undergoing pterional craniotomy without an onlay graft.

• Archives of Craniofacial Surgery
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• v.17 no.4
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• pp.211-217
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• 2016

Background: Nonsyndromic craniosynostosis is a relatively common craniofacial anomaly and various techniques were introduced to achieve its operative goals. Authors found that by using smaller bone fragments than that used in conventional cranioplasty, sufficiently rigid bone union and effective regeneration capacity could be achieved with better postoperative outcome, only if their stable fixation was ensured. Methods: Through bicoronal incisional approach, involved synostotic cranial bone together with its surrounding areas were removed. The resected bone flap was split into as many pieces as possible. The extent of this 'multi-split osteotomy' depends on the degree of dysmorphology, expectative volume increment after surgery and probable dead space caused by bony gap between bone segments. Rigid interosseous fixation was performed with variable types of absorbable plate and screw. In all cases, the pre-operational three-dimensional computed tomography (3D CT) was checked and brain CT was taken immediately after the surgery. Also about 12 months after the operation, 3D CT was checked again to see postoperative morphology improvement, bone union, regeneration and intracranial volume change. Results: The bony gaps seen in the immediate postoperative brain CT were all improved as seen in the 3D CT after 12 months from the surgery. No small bone fragment resorption was observed. Brain volume increase was found to be made gradually, leaving no case of remaining epidural dead space. Conclusion: We conclude that it is meaningful in presenting a new possibility to be applied to not only nonsyndromic craniosynostosis but also other reconstructive cranial vault surgeries.

• Journal of Korean Neurosurgical Society
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• v.46 no.4
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• pp.340-345
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• 2009

Objective : We investigated the locations of compressing vessels in hemifacial spasm. To approach compression sites, we described and evaluated the efficacy of the infranuchal infrafloccular (INIF) approach. Methods : A retrospective review of 31 consecutive patients who underwent microvascular decompression (MVD) through INIF with a minimum follow-up of 1 year was performed. Along the intracranial facial nerve, we classified the compression sites into the transitional zone (TRZ), the central nervous system (CNS) segment and the peripheral nervous system (PNS) segment. The INIF approach was used to inspect the CNS segment and the TRZ. Subdural patch graft technique was used in order to achieve watertight dural closure. The cranioplasty was performed using polymethylmethacrylate. The outcome and procedure-related morbidities were evaluated. Results : Twenty-nine patients (93%) showed complete disappearance of spasm. In two patients, the spasm was resolved gradually in 2 and 4 weeks, respectively. Late recurrence was noted in one patient (3%). The TRZ has been identified as the only compression site in 19 cases (61.3%), both the TRZ and CNS segment in 11 (35.5%) and the CNS segment only in 1 (3.2%). There was no patient having a compressing vessel in the PNS segment. Infection as a result of cerebrospinal fluid leak occurred in one patient (3%). Delayed transient facial weakness occurred in one patient. Conclusion : The TRZ and the CNS segment were more vulnerable area to the compression of vessels. We suggest that surgical avenue with the INIF approach provides early identification of this area.

• Journal of Genetic Medicine
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• v.13 no.1
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• pp.31-35
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• 2016

Antley-Bixler syndrome (ABS) is a rare form of syndromic craniosynostosis with additional systemic synostosis, including radiohumeral or radioulnar synostosis. Another characteristic feature of ABS is mid-facial hypoplasia that leads to airway narrowing after birth. ABS is associated with mutations in the FGFR2 and POR genes. Patients with POR mutations present with either skeletal manifestations or congenital adrenal hyperplasia with ambiguous genitalia. We report here two cases of ABS caused by mutations in FGFR2 and POR. Although the patients had craniosynostosis and radiohumeral synostosis in common and cranioplasty was performed in both cases, the male with POR mutations showed an elevated level of $17{\alpha}$-hydroxyprogesterone during newborn screening and was diagnosed with congenital adrenal hyperplasia by adrenocorticotropic hormone stimulation. This patient has been treated with hydrocortisone and fludrocortisone. He had no ambiguous genitalia but had bilateral cryptorchidism. On the other hand, the female with the FGFR2 mutation showed severe clinical manifestations: upper airway narrowing leading to tracheostomy, kyphosis of the cervical spine, and coccyx deformity. ABS shows locus heterogeneity, and mutations in two different genes can cause similar craniofacial and skeletal phenotypes. Because the long-term outcomes and inheritance patterns of the disease differ markedly, depending on the causative mutation, early molecular genetic testing is helpful.

• Archives of Reconstructive Microsurgery
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• v.22 no.1
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• pp.29-32
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• 2013

Purpose: Reconstruction of scalp and calvarial defects should provide both aesthetic and functional aspects. The inelastic nature of the scalp and previous surgery or radiation preclude the use of primary closure or a local flap. With development of microsurgical technique, a free tissue transfer is a good option. We use the latissimus dorsi myocutaneous free flap for reconstruction. Materials and Methods: A review of all latissimus dorsi free flap reconstructions performed in nine patients from 2009 to 2012 was conducted. There were six males and three females, ranging in age from seven to 69 years, and nine different regions, including five temporal regions, two occipital regions, and two frontoparietal regions. The flaps ranged in size from $9.0{\times}10.0cm$ to $14.0{\times}15.0cm$. Recipient vessels available for microanastomosis were most often the superficial temporal vessels and two patients had anastomoses to the external carotid artery and internal jugular vein. Results: All flaps survived postoperatively. With a median follow-up period of 14 months, no major complications were noted. However, two patients developed minor wound dehiscence, and a hematoma was observed in one patient. Conclusion: We performed the latissimus dorsi myocutaneous free flap reconstruction, which is one of the most popular reconstructive methods. The latissimus dorsi myocutaneous free flap reconstruction has been proven successful in our patients with satisfactory results. During the long term follow-up period, even though depressions were observed on the defect area in some patients, they were treated successfully with cranioplasty. Therefore, we recommend the latissimus dorsi myocutaneous free flap for reconstruction of scalp and calvarial defects.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.19 no.1
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• pp.76-81
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• 2016

Carnitine palmitoyltransferase 1A (CPT1A) is an enzyme functioning in mitochondrial fatty acid oxidation (FAO) of the liver. Patients with CPT1A deficiency have impaired mitochondrial FAO and display hypoketotic hypoglycemia and hepatic encephalopathy as typical manifestations. In this report, we present a case of CPT1A deficiency presenting jaundice as the first manifestation. A 1.9 years old boy showed jaundice and elevated levels of free and total carnitine were observed. From direct sequencing analysis of CPT1A, two novel mutations, c.1163+1G>A and c.1393G>A (p.Gly465Arg), were identified. At the age of 2.2 years, hypoglycemia, tachycardia, and altered mental status developed just after cranioplasty for craniosynostosis. High glucose infusion rate was required for recovery of his vital signs and mentality. Diet rich in high carbohydrate, low fat and inclusion of medium chain triglyceride oil resulted in improvement in cholestatic hepatitis and since then the boy has shown normal growth velocity and developmental milestones to date.

• Archives of Plastic Surgery
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• v.49 no.2
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• pp.174-183
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• 2022

Management of traumatic skull base fractures and associated complications pose a unique reconstructive challenge. The goals of skull base reconstruction include structural support for the brain and orbit, separation of the central nervous system from the aerodigestive tract, volume to decrease dead space, and restoration of the three-dimensional appearance of the face and cranium with bone and soft tissues. An open bicoronal approach is the most commonly used technique for craniofacial disassembly of the bifrontal region, with evacuation of intracranial hemorrhage and dural repair performed prior to reconstruction. Depending on the defect size and underlying patient and operative factors, reconstruction may involve bony reconstruction using autografts, allografts, or prosthetics in addition to soft tissue reconstruction using vascularized local or distant tissues. The vast majority of traumatic anterior cranial fossa (ACF) injuries resulting in smaller defects of the cranial base itself can be successfully reconstructed using local pedicled pericranial or galeal flaps. Compared with historical nonvascularized ACF reconstructive options, vascularized reconstruction using pericranial and/or galeal flaps has decreased the rate of cerebrospinal fluid (CSF) leak from 25 to 6.5%. We review the existing literature on this uncommon entity and present our case series of n = 6 patients undergoing traumatic reconstruction of the ACF at an urban Level 1 trauma center from 2016 to 2018. There were no postoperative CSF leaks, mucoceles, episodes of meningitis, or deaths during the study follow-up period. In conclusion, use of pericranial, galeal, and free flaps, as indicated, can provide reliable and durable reconstruction of a wide variety of injuries.

• Archives of Plastic Surgery
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• v.49 no.6
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• pp.704-709
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• 2022

Background The scar alopecia after cranioplasty (SAC) may decrease the patient's quality of life. We have treated SAC using follicular unit extraction (FUE). The aim of this study was to discuss that efficacy of FUE and how much hair follicular unit (FU) should be transplanted intraoperatively for the treatment of SAC. Methods We treated 10 patients (4 men and 6 women) who had SAC using FUE. Results The average age, alopecia size, and intraoperative hair density on the graft area were 29.8 ± 12.1 years, 29.8 ± 44.5 cm², and 34.6 ± 11.8 FU/cm², respectively. One year postoperatively, the average hair survival rate on the graft area was 66.3 ± 6.1%. Hair appearance was rated as good in six, fair in three, and poor in one. Among patients whose 1-year postoperative hair density was ≥ 20 FU/cm², five of six patients achieved good results. However, among patients whose 1-year postoperative hair density was < 20 FU/cm², all four patients achieved fair or poor results. The postoperative hair density was significantly higher in patients whose 1-year postoperative hair density was ≥ 20 FU/cm² than in patients whose 1-year postoperative hair density was< 20 FU/cm². The rate of achieving fair or poor results was significantly higher if the postoperative hair density was < 20 FU/cm² than if it was ≥ 20 FU/cm² (p = 0.047). Conclusions FU excision is useful for the treatment of scar alopecia after craniotomy. Our results suggest that the 1-year postoperative hair density should exceed 20 FU/cm² to achieve good outcomes.

• Journal of Veterinary Clinics
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• v.31 no.1
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• pp.46-50
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• 2014

A 7-year-old spayed female Welsh corgi presented with a mass of the skull. The mass was diagnosed as multilobular bone tumor and surgically removed. To treat a large bone defect after the tumor removal, custom-made artificial bone fabricated by a 3-dimensional ink-jet printer was implanted in the defect. Follow-up computed tomography evaluation was performed for 4.3 years. The implant was well integrated with the skull and had covered the large bone defect during the follow-up period. Gradual degradation of the implant began 6 weeks after surgery. It provides an additional option for the treatment of large bone defect.