Syndromic craniosynostosis caused by a novel missense variant in MAP4K4: Expanding the genotype-phenotype relationship in RASopathies.

RASopathies represent a distinct class of neurodevelopmental syndromes caused by germline variants in the Ras/MAPK pathways. Recently, a novel disease-gene association was implicated in MAPK kinase kinase kinase 4 (MAP4K4), which regulates the upstream signals of the MAPK pathways. However, to our knowledge, only two studies have reported the genotype-phenotype relationships in the MAP4K4-related disorder. This study reports on a Korean boy harboring a novel de novo missense variant in MAP4K4 (NM_001242559:c.569G>T, p.Gly190Val), revealed by trio exome sequencing, and located in the hotspot of the protein kinase domain. The patient exhibited various clinical features, including craniofacial dysmorphism, language delay, congenital heart defects, genitourinary anomalies, and sagittal craniosynostosis. Our study expands the phenotypic association of the MAP4K4-related disorder to include syndromic craniosynostosis, thereby providing further insights into the role of the RAS/MAPK pathways in the development of premature fusion of calvarial sutures.

Applicative Factors of Helmet Molding Therapy in Late-diagnosed Positional Plagiocephaly.

BACKGROUND: Although the benefits of helmet therapy for positional plagiocephaly are strongly correlated with age, the effective period remains controversial. However, most physicians agree that effective results can be obtained in patients within the age of 6 months. Owing to the characteristics of positional plagiocephaly in Koreans, many Korean patients have delayed diagnosis, and because this results in delayed onset of the helmet therapy, the outcomes remain largely underevaluated. In the management of late-diagnosed positional plagiocephaly, we aimed to determine the factors affecting the effective application of helmet therapy. METHODS: We recruited 39 consecutive patients with positional plagiocephaly who received helmet therapy and completed the treatment between December 2008 and June 2016. The ages at initiation and completion of treatment, duration of daily use, initial and final absolute diagonal differences, cephalic index, and cranial vault asymmetry index (CVAI) were analysed using data retrospectively collected from the patients' medical records. RESULTS: We identified 12 patients with late-diagnosed positional plagiocephaly, of whom 83.33% were effectively treated. The effective change in CVAI (%) was affected by age at treatment initiation (P = 0.001), initial absolute diagonal distance differences (P < 0.001), and initial CVAI (P < 0.001). Up to 9 months, a gradual change of at least 1% CVAI was attained. Treatment initiation at ages < 5.5 months was beneficial. Even at a later age, patients with an initial absolute diagonal distance difference of > 13.50 mm and initial CVAI of > 11.03% could receive effective helmet therapy. CONCLUSION: The efficacy of helmet therapy in late-diagnosed patients can be predicted on the basis of not only age at treatment initiation, but also initial absolute diagonal distance differences and initial CVAI. We anticipate that even patients with late-diagnosed positional plagiocephaly can expect better helmet therapy outcomes.

Effects of Tranexamic Acid Based on its Population Pharmacokinetics in Pediatric Patients Undergoing Distraction Osteogenesis for Craniosynostosis: Rotational Thromboelastometry (ROTEMTM) Analysis.

Background: Distraction osteogenesis for craniosynostosis is associated with significant hemorrhage. Additionally, patients usually require several transfusions. Tranexamic acid (TXA) is effective for reducing blood loss and the need for transfusions during surgeries. However, the significance of TXA infusion has not been thoroughly described yet. Methods: Forty-eight children undergoing distraction osteogenesis for craniosynostosis were administered intraoperative TXA infusion (loading dose of 10 mg/kg for 15 min, followed by continuous infusion at 5 mg/kg/h throughout surgery; n = 23) or normal saline (control, n = 25). Rotational thromboelastometry (ROTEMTM) was conducted to monitor changes in coagulation perioperatively. Results: Blood loss during surgery was significantly lower in the TXA-treated group than it was in the control group (81 vs. 116 mL/kg, P = 0.003). Furthermore, significantly fewer transfusions of red blood cells and fresh frozen plasma were required in the TXA group. In the control group, clotting time during the postoperative period was longer than it was during the preoperative period. Similarly, clot strength was weaker during the postoperative period. D-dimer levels dramatically increased in the control group compared with the TXA group after surgery. The duration of mechanical ventilation and the number of postoperative respiratory-related complications were significantly greater in the control group than they were in the TXA group. Conclusions: TXA infusion based on population pharmacokinetic analysis is effective in reducing blood loss and the need for transfusions during the surgical treatment of craniosynostosis. It can also prevent the increase in D-dimer levels without affecting systemic hemostasis.

Scaffold Free Bone Regeneration Using Platelet-Rich Fibrin in Calvarial Defect Model.

Bone regeneration is a complex process influenced by various physiological factors. Platelet-rich plasma (PRP) contains many growth factors and has shown osteogenic effects. The PRP is usually activated before use. However, the authors showed that nonactivated PRP (nPRP) and activated PRP have comparable osteogenic effects in the previous study. Generally, a scaffold has been needed for the application of PRP in the cranial defect model. In this study, the authors aimed to compare the performance of scaffold free platelet-rich fibrin (PRF) to nPRP as an adjuvant for bone regeneration.Twenty-four New Zealand White rabbits were randomly allocated into 3 groups: control, nPRP, and PRF. A 15 × 15 mm defect was created on each rabbit's cranium. Acellular collagen sponges (Gelfoam) were placed on the defects of the control group, Gelfoam with nPRP was used for the nPRP group, and PRF membrane was directly applied for the PRF group. nPRP and PRF were obtained from each subject's peripheral blood. Sixteen weeks later, the volume of regenerated bone was measured using 3-dimensional computed tomography. The surface area was measured via autopsy, and the samples were then obtained for histological analysis.Bone regeneration in the experimental groups was significantly greater than that in the control group. There were no significant differences in the area of regeneration or histological characteristics between the nPRP and PRF groups.In the calvarial defect of the rabbits, the use of PRF and scaffolded PRP showed comparable bone regeneration effects, which suggested that PRF might be a therapeutic alternative for bone grafts.

Altered cerebrospinal fluid dynamics in neurofibromatosis type l: severe arachnoid thickening in patients with neurofibromatosis type 1 may cause abnormal CSF dynamic.

INTRODUCTION: The object of this study is to understand abnormal dynamic of cerebrospinal fluid (CSF) in patients with neurofibromatosis type 1 (NF1), which may cause temporal lobe herniation and bulging of temporal fossa. METHODS: Four patients, three females and one male, with NF1 were studied retrospectively. They presented with a similar craniofacial deformity, which consisted of pulsatile exophthalmos, an enlarged bony orbit, dysplasia of the sphenoid wing with the presence of a herniation of the temporal lobe into the orbit, and a bulging temporal fossa. RESULTS AND DISCUSSION: Surgical exploration demonstrated abnormally thickened arachnoid membrane in one case. Protruding temporal lobe, which was one of the main symptoms in NF1 patients, could be stopped by control of intracranial pressure (ICP) via programmable ventriculoperitoneal shunt (VPS) or extra ventricle drainage implantation. The dense fibrosis of the arachnoid membrane and consequent altered hemispheric CSF dynamics may cause symptoms including pulsatile exophthalmos and consequent worsening of vision, prolapse of the temporal lobe, and enlargement of the temporal fossa. This finding may not present with general features of hydrocephalus, so that delays in diagnosis often result. CONCLUSION: For the NF1 patients with cranio-orbito-temporal deformities, prior to any surgical reconstruction, control of increased ICP (IICP) should be primarily considered.

Craniofacial Fibrous Dysplasia: Retrospective Study on the Relationship Between the Tumor Volume Changes and the Circulating Serum Calcitonin and Serum Alkaline Phosphatase.

BACKGROUND: The purpose of this study was to determine the usefulness of serum alkaline phosphatase (ALP) and calcitonin in the follow-up of tumor volume changes in patients with craniofacial fibrous dysplasia. METHODS: Twenty patients with isolated craniofacial fibrous dysplasia were included, who met our criteria for long-term follow-up. Three-dimensional computed tomography scans were obtained, and tumor segmentation was performed. The tumor volume was measured preoperatively, immediate postoperative and during long-term follow-up. Serum ALP and calcitonin levels were obtained at the same times to assess their correlation with tumor volumes. RESULTS: Preoperative calcitonin levels were correlated with the presence of tumor (P = 0.0442), whereas ALP levels were not (P = 0.1125). There were no significant associations between tumor volume and ALP or calcitonin levels in the preoperative or postoperative periods. During long-term follow-up, serum ALP was significantly associated with tumor recurrence (P = 0.0096), but serum calcitonin was not (P = 0.4760). However, serum levels of ALP did not reflect the tumor volume changes. CONCLUSIONS: Serum ALP may be useful as a laboratory test for follow-up of patients with isolated craniofacial fibrous dysplasia. However, it cannot represent the tumor's volume changes and 3-dimensional computed tomography scans with tumor volume measurement are mandatory for detecting significant volume changes during follow-up. Investigation of the serum calcitonin in the preoperative period is also recommended on a large scale because it was related to the presence of the tumor.

Cervical Spine Deformity in Long-Standing, Untreated Congenital Muscular Torticollis.

BACKGROUND: Congenital muscular torticollis (CMT) is a benign condition. With early diagnosis and appropriate management, it can be cured completely, leaving no residual deformity. However, long-standing, untreated CMT can lead to permanent craniofacial deformities and asymmetry. METHODS: Four adult patients presented to the author with long-standing, untreated CMT. Initial clinical assessment demonstrated tightness of the sternocleidomastoid muscle on the affected side. Investigation of cervical spine using 3-dimensional computed tomography scans with cervical segmentation allowed a 3-dimensional module to be separately created for each vertebra to detect any anatomical changes. RESULTS: A change in the axis of the vertebral column was noted when compared to that of the skull. Also, there were apparent anatomical changes affecting the vertebrae, which were most noticeable at the level of the atlas and axis vertebrae. These changes decreased gradually till reaching the seventh cervical vertebra, which appeared to be normal in all patients. The changes in the atlas vertebra were mostly due to its intimate relation with the skull base. The changes of the axis were the most significant, affecting mainly the superior articular facet, the lamina, and the body. CONCLUSIONS: There were seemingly permanent changes along the cervical spine region in the adult patients with long-standing, untreated CMT in the form of bending and rotation deformities that might result in residual torticollis postoperatively.

Computer-assisted shape descriptors for skull morphology in craniosynostosis.

PURPOSE: Our aim was to develop a novel method for characterizing common skull deformities with high sensitivity and specificity, based on two-dimensional (2D) shape descriptors in computed tomography (CT) images. METHODS: Between 2003 and 2014, 44 normal subjects and 39 infants with craniosynostosis (sagittal, 29; bicoronal, 10) enrolled for analysis. Mean age overall was 16 months (range, 1-120 months), with a male:female ratio of 56:29. Two reference planes, sagittal (S-plane: through top of lateral ventricle) and coronal (C-plane: at maximum dimension of fourth ventricle), were utilized to formulate three 2D shape descriptors (cranial index [CI], cranial radius index [CR], and cranial extreme spot index [CES]), which were then applied to S- and C-plane target images of both groups. RESULTS: In infants with sagittal craniosynostosis, CI in S-plane (S-CI) usually was <1.0 (mean, 0.78; range, 0.67-0.95), with CR consistently at 3 and a characteristic CES pattern of two discrete hot spots oriented diagonally. In the bicoronal craniosynostosis subset, CI was >1.0 (mean 1.11; range, 1.04-1.25), with CR at -3 and a CES pattern of four discrete diagonally oriented hot spots. Scatter plots underscored the highly intuitive joint performance of CI and CES in distinguishing normal and deformed states. Altogether, these novel 2D shape descriptors enabled effective discrimination of sagittal and bicoronal skull deformities. CONCLUSIONS: Newly developed 2D shape descriptors for cranial CT imaging enabled recognition of common skull deformities with statistical significance, perhaps providing impetus for automated CT-based diagnosis of craniosynostosis.

Decorin-expressing adenovirus decreases collagen synthesis and upregulates MMP expression in keloid fibroblasts and keloid spheroids.

Decorin is a natural transforming growth factor-ß1 (TGF-ß1) antagonist. Reduced decorin synthesis is associated with dermal scarring, and increased decorin expression appears to reduce scar tissue formation. To investigate the therapeutic potential of decorin for keloids, human dermal fibroblasts (HDFs) and keloid-derived fibroblasts (KFs) were transduced with decorin-expressing adenovirus (dE1-RGD/GFP/DCN), and we examined the therapeutic potential of decorin-expressing Ad for treating pathologic skin fibrosis. Decorin expression was examined by immunofluorescence assay on keloid tissues. HDFs and KFs were transduced with dE1-RGD/GFP/DCN or control virus, and protein levels of decorin, epidermal growth factor receptor (EGFR) and secreted TGF-ß1 were assessed by Western blotting and ELISA. And type I and III collagen, and matrix metalloproteinase-1 (MMP-1) and matrix metalloproteinase-3 (MMP-3) mRNA levels were measured by real-time RT-PCR. Additionally, we immunohistochemically investigated the expression levels of the major extracellular matrix (ECM) proteins in keloid spheroids transduced with dE1-RGD/GFP/DCN. Lower decorin expression was observed in the keloid region compared to adjacent normal tissues. After treatment with dE1-RGD/GFP/DCN, secreted TGF-ß1 and EGFR protein expressions were decreased in TGF-ß1-treated HDFs and KFs. Also, type I and III collagen mRNA levels were decreased, and the expression of MMP-1 and MMP-3 mRNA was strongly upregulated. In addition, the expression of type I and III collagen, fibronectin and elastin was significantly reduced in dE1-RGD/GFP/DCN-transduced keloid spheroids. These results support the utility of decorin-expressing adenovirus to reduce collagen synthesis in KFs and keloid spheroid, which may be highly beneficial in treating keloids.

Expansion and compression distraction osteogenesis based on volumetric and neurodevelopmental analysis in sagittal craniosynostosis.

PURPOSE: Distraction osteogenesis (DO) is a less daunting procedure than extensive cranial vault remodeling and has been used to correct sagittal craniosynostosis. The purposes of this study are to describe DO in combination with expansion and compression procedures and to report analytic results based on the cranial index (CI), volumetric measurement, and neurodevelopmental tests. METHODS: Between June 2002 and May 2013, 32 patients with non-syndromic sagittal synostosis who had undergone antero-posterior compression with bitemporal expansion were recruited. Circumferential baseline, mid-sagittal, and bicoronal craniotomies were performed in addition to four-quadrant bone flap procedures via distraction with dural attachment. CI, intracranial volume (ICV), head circumference (HC), and neurodevelopmental outcomes using the Bayley Scales of Infant Development-II (BSID-II) scoring system were analyzed. RESULTS: Dolichocephalic CI (68.85 ± 3.61) was altered to mesocephalic CI (78.28 ± 3.74) postoperatively. Preoperative ICV and HC determinations were within the normal range for the majority of participants (normal ICV 87.5%, n = 28; normal HC 75%, n = 24). ICV and HC did not show significant changes with postoperative measurements and were maintained within normal ranges. Regarding neurodevelopment, both the mental and psychomotor developmental indices (MDI, PDI) demonstrated significant improvement (MDI, 88.69 ± 17.38 to 96.23 ± 21.05; PDI, 91.38 ± 16.31 to 100 ± 11.51; p < 0.05). CONCLUSIONS: Symmetric sagittal synostosis can be treated comprehensively through DO in combination with expansion and compression procedures. ICV and HC measurements were useful for designing surgical and postoperative distraction strategies. Achievement of mesocephalic CI and neurodevelopmental improvement validated morphological and functional effectiveness.

Three-dimensional analysis of cranial and facial asymmetry after helmet therapy for positional plagiocephaly.

PURPOSE: Helmet therapy is a non-surgical option for treating positional plagiocephaly, and its effectiveness has been validated by various researches. In addition to cranial flattening and asymmetry, ipsilateral prominence of the mid-face and relative anterior transposition of the ipsilateral ear is also common. Hence, we investigated the impact of helmet therapy on mid-facial asymmetry. METHODS: Ninety-nine patients diagnosed with positional plagiocephaly and treated by helmet therapy between September 2005 and July 2012 were enrolled. Therapy was initiated at various ages: group I, <6 months (n = 35); group II, 6 months to 1 year (n = 43); group III, >1 year (n = 21). A cranial vault asymmetry index was measured at the levels of the inferior orbital rim (CVAIIOR-MF) and superior orbital rim (CVAISOR-LC) and midway from the superior orbital rim to the vertex (CVAIMID-UC). Anterior transposition of the ipsilateral ear was verified by measuring the distance (D EAR) between the actual position of the ear and its expected position relative to the contralateral ear. All variables were compared before and after helmet therapy and were categorized by age at treatment initiation. RESULTS: CVAIIOR-MF and CVAISOR-LC were lower in all three age groups after helmet therapy, confirming therapeutic efficacy. CVAIMID-UC (upper level cranial asymmetry) and D EAR (mid-facial soft tissue asymmetry) also improved significantly in groups I and II which were younger than 1 year old. CONCLUSION: In positional plagiocephaly, helmet therapy is effective in correcting both cranial and mid-facial asymmetries. Outcomes were optimal in patients <1 year of age, but older patients also showed some improvement.

Transport distraction osteogenesis with recombinant human bone morphogenic protein-2 for large calvarial defect reconstruction.

BACKGROUND: Transport distraction osteogenesis (TDO) has been used in attempts to treat large calvarial defects but has, until now, lacked consistency and reliability. To achieve sufficient bone formation, the effect of TDO was compared to the effect of TDO combined with recombinant human bone morphogenic protein-2 (rhBMP-2). METHODS: Fourteen dogs were divided into 2 groups; 6 animals in the control group received TDO only, and 8 received TDO combined with rhBMP-2. A calvarial defect 33 × 35 mm in size was generated, and the drug-delivering internal distractor was applied. After a 5-day latency period, distraction with rhBMP-2 at 10 µg/day was initiated at a rate of 2 mm/day. This was followed by a consolidation period of 3 months, after which areas of osteogenesis and strength were measured and histologic examinations were conducted. RESULTS: The average area of osteogenesis was higher in the experimental group (P < 0.01). Regenerated bone of the experimental group showed increased strength (P < 0.05). Histological examination showed typical mature bone in the experimental group. Prominent osteoblastic rimming was observed in the bone marrow of the experimental group. CONCLUSIONS: TDO with an internal distraction device delivering rhBMP-2 can enhance bone regeneration of large calvarial defects in a dog model. These results suggest the potential for human clinical testing of TDO combined with rhBMP-2.

Association of cranial base suture/synchondrosis fusion with severity of increased intracranial pressure in Crouzon syndrome.

This study investigated how the fusion states of the cranial base is related to the degree of increased intracranial pressure (ICP) in patients with Crouzon syndrome. This retrospective cohort study enrolled patients who were diagnosed with Crouzon syndrome between May 2007 and April 2022. We categorized the patients into three groups: A, B, and C, according to the severity of increased ICP and the number of cranial vault remodeling procedures for corrective operation. The preoperative fusion states of the cranial base sutures/synchondroses were examined using facial bone computed tomography and compared between groups. Overall, 22 patients were included in Groups A, B, and C, including 8, 7, and 7 patients, respectively. The preoperative average grades of the total cranial base suture/synchondrosis fusion appeared to significantly increase with severity, except for the frontoethmoidal suture, which showed the opposite tendency. In the subgroup analysis, frontosphenoidal, sphenoparietal, sphenosquamosal, parietomastoid, and occipitomastoid suture and petro-occipital synchondrosis were associated with earlier fusion in the more severe group. Premature closure of the cranial base sutures/synchodroses seems to be associated with increased ICP severity in patients with Crouzon syndrome. Precise evaluation of minor sutures/synchondroses at the first visit might help build subsequent operative plans and predict disease prognosis.

A novel three-dimensional model system for keloid study: organotypic multicellular scar model.

We developed a three-dimensional organotypic multicellular spheroid scar model to mimic the microenvironment of human keloid tissues. Keloid tissues were cultured for 7 days. Changes in total cellularity and apoptotic index in the primary keloid spheroid cultures were evaluated histologically and with a TUNEL assay, respectively. The expression profiles of transforming growth factor-ß (TGF-ß), collagen I, collagen III, elastin, fibronectin, matrix metalloproteinase-2, and matrix metalloproteinase-9 were examined with immunohistochemistry. In addition, these expression profiles were investigated after treating primary keloid spheroids with triamcinolone acetonide. Cell viability and morphology of ex vivo cultured keloid spheroids were maintained, and the apoptotic index did not increase for up to 1 week in culture. Keloid spheroids cultivated ex vivo retained the major characteristics of keloids, such as high levels of collagen I and TGF-ß expression for up to 7 days. The biological activity of keloids responding to TGF-ß was also maintained during ex vivo culture. Moreover, ex vivo triamcinolone acetonide treatment of cultivated keloid spheroids significantly reduced collagen I, collagen III, elastin, and fibronectin expression levels, in accordance with clinical observations. The three-dimensional organotypic multicellular spheroid keloid culture will allow investigators to study keloid pathogenesis and test potential keloid therapeutic agents.

Cranial compression using distractors in reverse fashion as an alternative method for correcting scaphocephaly in older patients.

BACKGROUND: Intraoperative calvarial contraction of conventional calvarial remodeling is more difficult in scaphocephaly patients who are 1 year or older in age. In our current study, gradual cranial compression with a distractor was used to correct scaphocephaly in this older age group and the surgical results were quantitatively analyzed. METHODS: Gradual cranial compression was used to treat 7 sagittal craniosynostosis patients. The mean age was 20.9 months (range, 12-32 months) and the mean follow-up period was 65 months (range, 3-81 months). Computed tomography was used to calculate the cephalic index (CI; equal to the maximum width of the head/maximum length of the head ×100), and the ratios of these indices at 3 different time periods (before, immediately after, and at 1 year postoperatively) were evaluated. RESULTS: An average cranial compression of 18 mm was found to be possible with an average CI increase from 67.9 to 73.5. The CI ratio at 1 year after surgery was found to be the highest, showing a 9.8% increase. CONCLUSION: Sagittal craniosynostosis patients are less easy to treat with conventional calvarial remodeling surgery if they are older than 1 year. Gradual cranial vault compression with distractors can be another option in these cases.

Eyelid dynamics and supratarsal crease appearance after double eyelid surgery.

BACKGROUND: Although double eyelid surgery is one of the most common cosmetic surgeries among Asians, there are few reports to confirm the physiology of eye blinking after such surgeries. This study analyzed eyelid dynamics and supratarsal crease appearance after double eyelid surgery using a high-speed digital camera to provide precise movement detection. METHODS: Twenty healthy volunteers and 15 patients who underwent double eyelid surgery with tarsodermal fixation were studied. Using the slow-motion replay setting of a high-speed digital camera, the patients' spontaneous eye blinking was analyzed for (1) blink duration, (2) peak eyelid velocity, (3) blink rate, and (4) supratarsal crease appearance. RESULTS: After double eyelid surgery, there were no significant differences in blink duration, peak eyelid velocity, or blink rate compared with the control group. Regarding supratarsal crease appearance, dynamic creases were created in 6 of 15 patients, whereas 9 patients showed static creases. CONCLUSIONS: Double eyelid surgery with tarsodermal fixation does not alter lid dynamics of spontaneous eye blinking. Furthermore, the tarsodermal fixation method, which is known to create a static crease, could also lead to the development of a dynamic crease.

Improved scar appearance with combined use of silicone gel and vitamin C for Asian patients: a comparative case series.

BACKGROUND: In Asians, facial scars, even fine surgical scars, often can be conspicuous and uncomfortable. The authors used a topical silicone gel containing vitamin C on facial scars for the purpose of making the scar less distinct. METHODS: The study enrolled 80 patients. For the experimental group, the topical silicone gel mixture containing vitamin C was applied from the time of stitch removal to 6 months after the operation. The control group did not undergo any adjunctive treatment. Each participant was evaluated using the modified Vancouver Scar Scale (VSS) as well as erythema and melanin indices by spectrophotometer. RESULTS: With the modified VSS, the experimental group showed a significant decrease in scar elevation (p = 0.026) and erythema (p = 0.025). The hypo- or hyperpigmentation of the scars was more normalized in the experimental group. In the measured results via spectrophotometer, the experimental group showed a significant decrease in the melanin index (p = 0.045). The erythema index showed a statistically significant difference between the time of stitch removal and 6 months after the operation in the experiment group only. CONCLUSIONS: Topical use of silicone gel containing vitamin C has the effect of improving the appearance of fine surgical scars in Asian facial skin. LEVEL OF EVIDENCE IV: This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors www.springer.com/00266 .

Endoscopic-assisted surgical correction for congenital muscular torticollis.

BACKGROUND: Various surgical approaches have been advocated to release, lengthen, or excise a segment of the sternocleidomastoid (SCM) muscle for the treatment of congenital muscular torticollis (CMT). However, all those conventional methods make noticeable scars on the clavicle or neck area. We proposed the endoscopic-assisted postauricular approach for the treatment of CMT that provides good functional and cosmetic outcomes and less learning curve. METHODS: Endoscopic-assisted release of the SCM muscle was performed in 15 children. Ages at operation ranged from 9 months to 6 years, with a mean of 17.6 months. The sternal and clavicular attachments of the SCM muscle were dissected and resected at 1-cm segments near to the insertion area, through the endoscopic view. To prevent relapse, neck splint was applied after operation for 6 months. RESULTS: The mean operative time was 40 minutes. During the procedure, great auricular nerve and spinal accessory nerve were preserved well and there were no complications. Follow-up for 6 months in all patients showed complete muscular release and satisfactory correction with no recurrence. The neck scars were not visible after the procedure. The incision scar was well obscured on the posterior auricular sulcus. CONCLUSIONS: Endoscopic correction for the CMT is a feasible and easy-to-learn procedure. This method is a safe and effective procedure that provides good functional and cosmetic outcomes.

Osteoconductivity of porous polyethylene in human skull.

PURPOSE: Recently, biomaterials have been generally used in reconstruction of a bony defect or augmentation of the facial skeleton. Medpor implants in vivo in animal models showed both soft tissue and bony ingrowth into its pores and have been widely accepted to have an osteoconduction activity. However, in an in vivo study in humans, there was no definite evidence of bony ingrowth into the pores of Medpor. This study examined the osteoconductivity of Medpor in human vivo. METHODS: We gained a total of 24 Medpor blocks when removing a distraction device in 11 patients with craniosynostosis. The Medpor blocks were used for secure placement of the distraction device. The blocks were taken out after distraction and consolidation periods. The surface of Medpor in contact with the bone was histologically examined to confirm the osteogenic activity. RESULTS: There was no evidence of osteoconduction in all 24 specimens. The mean total duration of implantation was 2.5 months. CONCLUSIONS: In human vivo, implantation of a porous polyethylene implant is thought to have no osteogenetic effect through osteoconductive activity even in young children.