Language：English   Publishing type：Research paper (scientific journal)  

BACKGROUNDS: Iris nodules are frequently noted as clinical manifestations of neurofibromatosis type 1 but the other intraocular manifestations are rare. The purpose of this study is to present a patient with a phthisic eye who underwent enucleation for a cosmetic reason after 15-year follow-up and also to review 14 patients with enucleation described in the literature. CASE PRESENTATION: A 17-year-old man with neurofibromatosis type 1 from infancy underwent the enucleation of phthisic left eye and also had the resection of eyelid subcutaneous mass lesions on the left side for a cosmetic reason. He had undergone four-time preceding surgeries for eyelid and orbital mass reduction on the left side in childhood and had developed total retinal detachment 10 years previously. Pathologically, the enucleated eye showed massive retinal gliosis positive for both S-100 and glial fibrillary acidic protein (GFAP) in the area with involvement of the detached retinal neuronal layer, together with a more fibrotic lesion along the choroid which were, in contrast, negative for both S-100 and GFAP. The choroid, ciliary body, and iris did not show apparent neurofibroma while episcleral neurofibroma was present. LITERATURE REVIEW: In review of enucleated eyes of 14 patients in the literature, buphthalmic eyes with early-onset glaucoma on the unilateral side was clinically diagnosed in 9 patients who frequently showed varying extent of hemifacial neurofibromatosis which involved the eyelid and orbit on the same side. Pathologically, neurofibromas in varying extent were found in the choroid of 12 patients. One patient showed choroidal malignant melanoma on the left side and fusiform enlargement of the optic nerve on the right side suspected of optic nerve glioma. The phthisic eye in another patient showed massive retinal gliosis similar to the present patient. CONCLUSIONS: In summary of the 15 patients with neurofibromatosis type 1, including the present patient, buphthalmic or phthisic eyes with no vision were enucleated for cosmetic reasons and showed choroidal neurofibroma in most patients and massive retinal gliosis in two patients including the present patient.

DOI： 10.1186/s12886-024-03604-5

PubMed

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Language：Japanese   Publisher：(一社)日本小児神経外科学会  

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Publishing type：Research paper (scientific journal)   Publisher：Japan Neurosurgical Society  

DOI： 10.2176/nmc.oa.2021-0208

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Language：English   Publishing type：Research paper (scientific journal)  

We herein introduce horizontal vector analysis, a simple method for assessing cranial morphology based on measurement of the head’s horizontal plane, and use this method to establish normal cranial morphology in Japanese children Computed tomography scans taken in 2010-2019 in healthy Japanese children aged ≤ 6 years. The two measurement planes were parallel to the orbitomeatal plane: namely, a plane passing through the dorsum sellae (DS) and the plane superior to that with the maximal area (Max plane). A protractor was used to circumferentially measure the lengths from the central point to the outer surface of the skull. A total of 487 images were extracted. The distances between the DS and Max planes were consistently almost 30 mm for each age group, so we fixed the Max plane as the plane 30 mm superior to the DS plane. Finally, we established datasets of normal values for each age group and sex. Using these norms, perioperative evaluation of various cranial deformities could be performed more easily and circumstantially.

DOI： 10.18926/AMO/64038

Scopus

PubMed

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：Journal of Neurosurgery Publishing Group (JNSPG)  

The multidirectional cranial distraction osteogenesis (MCDO) procedure, which uses an external distraction device, enables tailor-made distraction in an arbitrary direction, eliminating the disadvantage of unidirectional distraction with an internal distraction device. Multiple-suture synostosis cases for syndromic craniosynostosis patients are better indicated for this procedure. Here the authors describe seven cases in which the MCDO procedure was used to treat syndromic craniosynostosis. In each case, the MCDO procedure and postoperative distraction, with reference to midsagittal vector analysis of normal morphology in Japanese children, resulted in morphological improvement.

The video can be found here: https://vimeo.com/519006555

DOI： 10.3171/2021.1.focvid20116

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Other Link： https://thejns.org/downloadpdf/journals/neurosurg-focus-video/4/2/article-pV17.xml

Authorship：Lead author   Language：Japanese   Publishing type：Article, review, commentary, editorial, etc. (scientific journal)  

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Authorship：Lead author   Language：Japanese   Publishing type：Article, review, commentary, editorial, etc. (scientific journal)  

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Language：Japanese   Publisher：(一社)日本小児神経外科学会  

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Language：Japanese   Publisher：(一社)日本小児神経外科学会  

MCDO法で治療した頭蓋骨縫合早期癒合症の11症例の分析を行い、MCDO法を選択しうる症例・治療タイミングについて検討した。MCDO法は様々な方向に延長が可能であり、複数縫合の癒合例はよい適応とされるが、両側冠状縫合の早期癒合例や矢状縫合の早期癒合例といった単一縫合癒合の例でも、よりよい頭蓋形態を作る上で有用だった。Syndromic craniosynostosisでは、長期経過を考慮した治療計画を立てる必要があるが、MCDOフレームに延長に必要なだけの余裕があれば、児童期でもMCDO法による形態修復は検討に値すると考えられた。(著者抄録)

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Language：Japanese   Publisher：(一社)日本頭蓋顎顔面外科学会  

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Event date： 2023.11.9 - 2023.11.10

Language：Japanese   Presentation type：Symposium, workshop panel (nominated)  

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Event date： 2023.4.26 - 2023.4.28

Language：Japanese   Presentation type：Oral presentation (general)  

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Event date： 2023.3.4

Language：Japanese   Presentation type：Symposium, workshop panel (nominated)  

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