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Lymphaticovenular anastomosis for Morbihan disease: a case report. 淋巴小静脉吻合术治疗Morbihan病1例
Q2 Medicine Pub Date : 2023-06-01 DOI: 10.7181/acfs.2022.01067
Jung Hyun Hong, Changryul Claud Yi, Jae Woo Lee, Yong Chan Bae, Ryuck Seong Kim, Joo Hyoung Kim

Morbihan disease (MD) is a very rare condition characterized by rosaceous or erythematous lymphedema on the upper twothirds of the face. A definitive management strategy for MD is lacking, and treatment is challenging. Herein, we present a case of persistent bilateral eyelid edema treated by lymphaticovenular anastomosis (LVA) and lymph node-vein bypass surgery. The patient experienced persistent bilateral eyelid edema. Indocyanine green lymphography was performed, and the diagnosis of bilateral facial lymphedema was confirmed. On the right side, a preauricular lymphatic vessel was anastomosed to a vein. On the left side, lymphostomy on the preauricular lymph node was done, with anastomosis to the transected proximal end of the concomitant vein of the transverse facial artery. Furthermore, a preauricular lymphatic vessel was anastomosed to a vein. Eyelid edema decreased and progressively improved on both sides. The outcome of this case suggests that LVA and lymph node-vein bypass surgery are appropriate for treating persistent eyelid edema related to MD.

莫比罕病(MD)是一种非常罕见的疾病,其特征是面部上三分之二的红斑性或红斑性淋巴水肿。缺乏明确的MD管理策略,治疗具有挑战性。在此,我们报告一例用淋巴小静脉吻合术和淋巴结静脉搭桥手术治疗持续性双眼睑水肿的病例。患者双侧眼睑持续水肿。行吲哚菁绿淋巴造影,确诊为双侧面部淋巴水肿。右侧耳前淋巴管与静脉吻合。左侧耳前淋巴结行淋巴造口术,吻合面横动脉伴静脉近端。此外,耳前淋巴管与静脉吻合。双侧眼睑水肿减轻并逐渐改善。本病例的结果提示LVA和淋巴结静脉搭桥手术是治疗MD相关的持续性眼睑水肿的合适方法。
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引用次数: 0
A comprehensive review of surgical techniques in unilateral cleft lip repair. 单侧唇裂修复手术技术的全面回顾。
Q2 Medicine Pub Date : 2023-06-01 Epub Date: 2023-06-20 DOI: 10.7181/acfs.2023.00268
Tae-Suk Oh, Young Chul Kim

Unilateral cleft lip is a common congenital anomaly that affects the appearance and function of the upper lip and nose. Surgical repair of cleft lip aims to restore the normal anatomy and functionality of the affected structures. In recent years, several advances have been made in the field of cleft lip repair, including new surgical techniques and approaches. This comprehensive review discusses the surgical management of patients with unilateral cleft lip and palate and provides step-by-step instructions for the surgical procedures.

单侧唇裂是一种常见的先天性畸形,会影响上唇和鼻子的外观和功能。唇裂手术修复的目的是恢复受影响结构的正常解剖和功能。近年来,唇裂修复领域取得了多项进展,包括新的手术技术和方法。本综述讨论了单侧唇腭裂患者的手术治疗,并提供了手术过程的逐步指导。
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引用次数: 0
Periorbital cutaneous angiomyolipoma: a case report 眼眶周围皮肤血管平滑肌脂肪瘤1例
Q2 Medicine Pub Date : 2023-04-20 DOI: 10.7181/acfs.acfs.2022.01088
Young Jun Kim, Min Hyub Choi, Ji Seon Cheon, Woo Young Choi
Angiomyolipomas are usually found in the kidneys of patients with tuberous sclerosis. They occur less frequently in organs such as the liver, the oral cavity, the nasal cavity, the heart, the large intestines, and the lungs. Angiomyolipomas of the skin are extremely rare, and cutaneous angiomyolipomas generally occur on the elbow, the ends of digits, the ear, and the glabella. Herein we present a rare case of angiomyolipoma occurring on the face—specifically, the right upper eyelid. We propose that upper eyelid angiomyolipoma is a hamartomatous, rather than neoplastic, lesion. Although angiomyolipoma in the periocular area is rare, it should be considered in the differential diagnosis of clinically benign masses. and regular follow-up is warranted.
血管平滑肌脂肪瘤通常见于结节性硬化症患者的肾脏。它们在肝脏、口腔、鼻腔、心脏、大肠和肺部等器官中发生的频率较低。皮肤血管平滑肌脂肪瘤极为罕见,皮肤血管平滑肌脂肪瘤通常发生在肘部、指端、耳部和眉间。在此我们提出一个罕见的病例血管平滑肌脂肪瘤发生在脸上,特别是,右上眼睑。我们认为上眼睑血管平滑肌脂肪瘤是一种错构瘤,而不是肿瘤。虽然眼周血管平滑肌脂肪瘤是罕见的,但在临床良性肿块的鉴别诊断中应予以考虑。定期跟进是必要的。
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引用次数: 0
Micropipette tip intubation in rats as a replacement for conventional endotracheal tube intubation. 微管尖端插管在大鼠中替代传统气管插管。
Q2 Medicine Pub Date : 2023-04-01 DOI: 10.7181/acfs.2022.01081
Myung-Good Kim, Eong-Ho Ryu, Dong Min Lee, Tae-Seo Park, Ji-An Choi

Endotracheal intubation is often necessary in the course of animal experiments, especially in craniofacial surgery. However, endotracheal intubation can be a major burden in this context. The authors performed simple and cost-saving method using a 200 μL yellow micropipette tip, and the success of this method was demonstrated by X-ray and autopsy. We used a total of 30 rats. After the rats were fixed with a plaster, the trachea and vocal cords were visualized with the tongue pulled back. Under direct visualization of the vocal cords, a curving micropipette tip was advanced into the trachea. This method can be learned quickly and applied successfully by general experimenters. We successfully intubated all 30 rats without any complications. The success rate of micropipette tip intubation was 100%. This procedure was performed by one experimenter within 2 to 3 minutes after induction of anesthesia. We demonstrated its superiority by X-ray and autopsy. Herein, we describe endotracheal intubation of rats using micropipette tips. To the best of our knowledge, this method is novel and represents the simplest and most efficient means of intubation in rats, providing an alternative to conventional endotracheal intubation.

在动物实验中,尤其是颅面外科手术中,气管插管是必不可少的。然而,在这种情况下,气管插管可能是一个主要负担。用200 μL的黄色微移液针尖进行了简单、节省成本的方法,并通过x线和尸体解剖证明了该方法的成功。我们总共使用了30只大鼠。用石膏固定大鼠后,将舌头向后拉,观察气管和声带。在直接观察声带的情况下,将一根弯曲的微移液管尖端插入气管。该方法可以被一般实验人员快速学习并成功应用。30只大鼠插管成功,无并发症。微管尖端插管成功率为100%。该程序由一名实验者在麻醉诱导后2至3分钟内完成。我们通过x光和尸检证明了它的优越性。在这里,我们描述了使用微吸管尖端的大鼠气管插管。据我们所知,这种方法新颖,是最简单、最有效的大鼠插管方法,是传统气管内插管的替代方法。
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引用次数: 0
Surgical treatment of postauricular hidradenitis suppurativa with delayed diagnosis: a case report and literature review. 延迟诊断的耳后化脓性扁桃体炎的手术治疗:病例报告和文献综述。
Q2 Medicine Pub Date : 2023-04-01 Epub Date: 2023-04-20 DOI: 10.7181/acfs.2023.00115
Inho Kang, Gyu Yong Jung, Min Jun Yong, Yujin Ahn, Joon Ho Lee

Hidradenitis suppurativa (HS) is a chronic inflammatory condition that is difficult to diagnose, with a period of 10.0± 9.6 years from symptom onset to diagnosis. A 32-year-old Asian man presented with bilateral postauricular abscesses that first appeared 5 years previously. Despite several incisions and drainage, the symptoms only temporarily improved and continued to recur. On physical examination, chronic scars and sinus tracts were observed around the lesion. Postauricular HS was diagnosed, and surgical treatment was performed. We performed a wide excision and reconstructed the defect using a posterior auricular artery perforator-based keystone flap. Histological examination confirmed the diagnosis of HS. The reconstruction was successful, and there was no recurrence for 2 years after surgery. HS is difficult to diagnose without specific attention. Although the postauricular region is not a typical site of HS, it can occur in this area. Therefore, if a patient presents with recurrent abscesses in the postauricular region, HS should be considered. Additionally, if HS is diagnosed in the postauricular region, wide excision with reconstruction using a posterior auricular artery perforator-based keystone flap can lead to a favorable outcome.

化脓性扁桃体炎(HS)是一种难以诊断的慢性炎症,从症状出现到确诊需要 10.0±9.6 年的时间。一名 32 岁的亚洲男子在 5 年前首次出现双侧耳后脓肿。虽经多次切开引流,但症状仅暂时好转,且不断复发。体格检查发现,病灶周围有慢性疤痕和窦道。耳后 HS 被确诊,并进行了手术治疗。我们进行了大范围切除,并使用耳后动脉穿孔基底匙形皮瓣重建了缺损。组织学检查证实了 HS 的诊断。重建手术很成功,术后两年没有复发。如果不特别注意,很难诊断出 HS。虽然耳后区域不是HS的典型部位,但它也可能发生在这一区域。因此,如果患者耳后区反复出现脓肿,则应考虑为 HS。此外,如果确诊耳后区为 HS,则应进行广泛切除,并使用耳后动脉穿孔基底瓣进行重建,这样可以获得良好的治疗效果。
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引用次数: 0
Malignant melanoma associated with a plaque-type blue nevus of the cheek: a case report. 与脸颊斑块型蓝痣相关的恶性黑色素瘤:病例报告。
Q2 Medicine Pub Date : 2023-04-01 Epub Date: 2023-04-20 DOI: 10.7181/acfs.2023.00024
Yoon Kyu Chung, Min-Seok Kim, Jin Yong Shin, Nae-Ho Lee, Ae Ri An, Si-Gyun Roh

Blue nevi, which are characterized by collections of pigment-producing melanocytes in the dermis, have a variety of clinicopathological characteristics. Plaque-type blue nevus (PTBN) is a variant of blue nevi. PTBN presents at birth or arises in early childhood, and it shows a combination of the features found in common blue nevus and cellular blue nevus. It is typically found on the dorsal surface of the hands and feet or on the head and neck, and it is usually benign and stable over time. However, reports have occasionally described malignant melanomas developing in or associated with a PTBN. Malignant blue nevi are most commonly found on the scalp. We report the case of an 88-year-old woman with a malignant melanoma associated with a PTBN of the cheek.

蓝痣的特征是真皮层中产生色素的黑素细胞聚集,其临床病理特征多种多样。斑块型蓝痣(PTBN)是蓝痣的一种变异型。斑块型蓝痣是蓝痣的变异型,在出生时出现或在幼儿期出现,它综合了普通蓝痣和细胞型蓝痣的特征。它通常出现在手足背侧或头颈部,通常是良性的,而且随着时间的推移会逐渐稳定。不过,偶尔也有报告描述在 PTBN 中发生或与 PTBN 相关的恶性黑色素瘤。恶性蓝痣最常见于头皮。我们报告了一例 88 岁女性的恶性黑色素瘤病例,该病例与脸颊的 PTBN 相关。
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引用次数: 0
Orbital wall restoration with primary bone fragments in complex orbital fractures. 复杂眶壁骨折一期骨碎片修复。
Q2 Medicine Pub Date : 2023-04-01 DOI: 10.7181/acfs.2022.01116
Jong Hyun Park, Dong Hee Kang, Hong Bae Jeon, Hyonsurk Kim

Background: Complex orbital fractures are impure orbital fractures because they involve the orbital walls and mid-facial bones. The author reported an orbital wall restoration technique in which the primary orbital wall fragments were restored to their prior position in complex orbital fractures in 2020. As a follow-up to a previous preliminary study, this study retrospectively reviewed the surgical results of complex orbital wall fractures over a 4-year period and compared the surgical outcomes by dividing them into groups with and without balloon restoration.

Methods: Data of 939 patients with facial bone fractures between August 2018 and August 2022 were reviewed. Of these, 154 had complex orbital fractures. Among them, 44 and 110 underwent reduction with and without the balloon technique respectively. Pre- and postoperative Naugle exophthalmometer (Good-Lite Co.) scales were evaluated. The orbital volume and orbital volume ratio were calculated from preoperative and 6 months postoperative computed tomography images.

Results: Among 154 patients with complex orbital fractures, 44 patients underwent restoration with the balloon technique, and 110 patients underwent restoration without it. The Naugle scale did not differ significantly between the two groups, but the orbital volume ratio significantly decreased by 3.32% and 2.39% in groups with and without the balloon technique and the difference in OVR was significantly greater in patients in the balloon restoration group compared with the control group. Postoperative balloon rupture occurred in six out of 44 cases (13.64%). None of the six patients with balloon rupture showed significant enophthalmos at 6 months of follow-up.

Conclusion: The balloon rupture rate was 13.64% (6/44 cases) with marginal screw fixation, blunt screws, and extra protection with a resorbable foam dressing. Furthermore, we restored the orbital wall with primary orbital fragments using balloon support in complex orbital wall fractures.

背景:复杂的眶骨折是不纯粹的眶骨折,因为它累及眶壁和面中骨。作者报告了一种眶壁修复技术,在2020年将复杂眶骨折的初级眶壁碎片恢复到原来的位置。作为先前初步研究的随访,本研究回顾性回顾了4年来复杂眶壁骨折的手术结果,并将手术结果分为有球囊修复组和无球囊修复组进行比较。方法:回顾性分析2018年8月至2022年8月939例面部骨折患者的资料。其中,154例有复杂的眼眶骨折。其中44例和110例分别采用球囊复位和不采用球囊复位。对术前和术后诺格眼差计(Good-Lite Co.)量表进行评估。根据术前和术后6个月的计算机断层图像计算眼眶体积和眼眶体积比。结果:154例复杂眶骨折患者中,44例采用球囊技术复位,110例未采用球囊技术复位。两组间的Naugle评分差异无统计学意义,但球囊修复组与未球囊修复组眼眶体积比分别显著降低3.32%和2.39%,球囊修复组患者的OVR与对照组相比差异显著较大。44例术后球囊破裂6例(13.64%)。随访6个月,6例球囊破裂患者均无明显内陷。结论:采用边缘螺钉固定,钝螺钉固定,并用可吸收泡沫敷料进行额外保护,球囊破裂率为13.64%(6/44例)。此外,在复杂的眶壁骨折中,我们使用球囊支架修复眶壁。
{"title":"Orbital wall restoration with primary bone fragments in complex orbital fractures.","authors":"Jong Hyun Park,&nbsp;Dong Hee Kang,&nbsp;Hong Bae Jeon,&nbsp;Hyonsurk Kim","doi":"10.7181/acfs.2022.01116","DOIUrl":"https://doi.org/10.7181/acfs.2022.01116","url":null,"abstract":"<p><strong>Background: </strong>Complex orbital fractures are impure orbital fractures because they involve the orbital walls and mid-facial bones. The author reported an orbital wall restoration technique in which the primary orbital wall fragments were restored to their prior position in complex orbital fractures in 2020. As a follow-up to a previous preliminary study, this study retrospectively reviewed the surgical results of complex orbital wall fractures over a 4-year period and compared the surgical outcomes by dividing them into groups with and without balloon restoration.</p><p><strong>Methods: </strong>Data of 939 patients with facial bone fractures between August 2018 and August 2022 were reviewed. Of these, 154 had complex orbital fractures. Among them, 44 and 110 underwent reduction with and without the balloon technique respectively. Pre- and postoperative Naugle exophthalmometer (Good-Lite Co.) scales were evaluated. The orbital volume and orbital volume ratio were calculated from preoperative and 6 months postoperative computed tomography images.</p><p><strong>Results: </strong>Among 154 patients with complex orbital fractures, 44 patients underwent restoration with the balloon technique, and 110 patients underwent restoration without it. The Naugle scale did not differ significantly between the two groups, but the orbital volume ratio significantly decreased by 3.32% and 2.39% in groups with and without the balloon technique and the difference in OVR was significantly greater in patients in the balloon restoration group compared with the control group. Postoperative balloon rupture occurred in six out of 44 cases (13.64%). None of the six patients with balloon rupture showed significant enophthalmos at 6 months of follow-up.</p><p><strong>Conclusion: </strong>The balloon rupture rate was 13.64% (6/44 cases) with marginal screw fixation, blunt screws, and extra protection with a resorbable foam dressing. Furthermore, we restored the orbital wall with primary orbital fragments using balloon support in complex orbital wall fractures.</p>","PeriodicalId":52238,"journal":{"name":"Archives of Craniofacial Surgery","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/48/ed/acfs-2022-01116.PMC10165238.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9437964","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 1
Periorbital cutaneous angiomyolipoma: a case report. 眼眶周围皮肤血管平滑肌脂肪瘤1例。
Q2 Medicine Pub Date : 2023-04-01 DOI: 10.7181/acfs.2022.01088
Young Jun Kim, Min Hyub Choi, Ji Seon Cheon, Woo Young Choi

Angiomyolipomas are usually found in the kidneys of patients with tuberous sclerosis. They occur less frequently in organs such as the liver, the oral cavity, the nasal cavity, the heart, the large intestines, and the lungs. Angiomyolipomas of the skin are extremely rare, and cutaneous angiomyolipomas generally occur on the elbow, the ends of digits, the ear, and the glabella. Herein we present a rare case of angiomyolipoma occurring on the face-specifically, the right upper eyelid. We propose that upper eyelid angiomyolipoma is a hamartomatous, rather than neoplastic, lesion. Although angiomyolipoma in the periocular area is rare, it should be considered in the differential diagnosis of clinically benign masses. and regular follow-up is warranted.

血管平滑肌脂肪瘤通常见于结节性硬化症患者的肾脏。它们在肝脏、口腔、鼻腔、心脏、大肠和肺部等器官中发生的频率较低。皮肤血管平滑肌脂肪瘤极为罕见,皮肤血管平滑肌脂肪瘤通常发生在肘部、指端、耳部和眉间。在此我们提出一个罕见的病例血管平滑肌脂肪瘤发生在脸上,特别是,右上眼睑。我们认为上眼睑血管平滑肌脂肪瘤是一种错构瘤,而不是肿瘤。虽然眼周血管平滑肌脂肪瘤是罕见的,但在临床良性肿块的鉴别诊断中应予以考虑。定期跟进是必要的。
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引用次数: 0
Long-term outcomes after core extirpation of fibrous dysplasia of the zygomaticomaxillary region. 颧腋区纤维发育不良取芯术后的远期疗效。
Q2 Medicine Pub Date : 2023-04-01 DOI: 10.7181/acfs.2023.00038
Joseph Kyu-Hyung Park, Se Yeon Lee, Jong-Ho Kim, Baek-Kyu Kim

Background: Fibrous dysplasia (FD) is a localized bone disorder in which fibro-osseous tissue replaces the normal bone structure. Patients with craniofacial FD often present with gradual swelling, deformity, and compromised vision or hearing. We previously introduced "the core extirpation method," a novel surgical technique that is minimally invasive like traditional bone shaving but has longer-lasting effects. This study presents the long-term outcomes of our core extirpation method.

Methods: We conducted a retrospective analysis of patients who underwent core extirpation for FD of the zygomaticomaxillary region from 2012 through 2021. Computed tomography (CT) scans were performed 6 to 12 months before the operation, immediately before and after the operation, and during follow-up visits. We performed all operations using the upper gingivobuccal approach, and we extirpated the core of the lesion while preserving the cortical structures of the zygoma and the maxilla to maintain symmetrical facial contour.

Results: In 12 patients with lesions in the growth phase, anteroposterior/mediolateral (AP/ML) length discrepancies and the volume increased between preoperative and immediate postoperative CT scans. All patients' immediate postoperative AP/ML discrepancies were stable up to 12-17 months postoperatively. Postoperative volume showed continuous lesion growth; the median volume growth rate was 0.61 cc per month.

Conclusion: In this article, we present our experiences managing FD using the minimally invasive core extirpation technique, which entails small expected blood loss and can be performed as day surgery. It provides similar cosmetic outcomes as traditional bone shaving but with longer-lasting results. Although there are some limitations with the study's retrospective nature and small sample size, our 4-year follow-up results show promising results of the core extirpation method in well-indicated patients.

背景:纤维性发育不良(FD)是一种局部骨疾病,纤维骨组织取代了正常的骨结构。颅面FD患者通常表现为逐渐肿胀、畸形和视力或听力受损。我们之前介绍过“核心去除法”,这是一种新的手术技术,像传统的骨刮除一样是微创的,但效果更持久。本研究展示了我们的核心剔除方法的长期结果。方法:我们对2012年至2021年接受颧腋窝区FD核心摘除的患者进行了回顾性分析。术前6 ~ 12个月、术前、术后及随访期间分别进行CT扫描。所有手术均采用上龈颊入路,切除病灶核心,同时保留颧骨和上颌骨皮质结构,保持面部轮廓对称。结果:12例病变处于生长期的患者,术前和术后CT扫描显示正前方/中外侧(AP/ML)长度差异和体积增加。所有患者术后即刻AP/ML差异在术后12-17个月保持稳定。术后体积呈病变持续增长;中位数体积增长率为每月0.61立方厘米。结论:在这篇文章中,我们介绍了我们使用微创核心摘除技术治疗FD的经验,该技术需要较小的预期出血量,可以作为日间手术进行。它提供了与传统剃骨术相似的美容效果,但效果更持久。虽然该研究的回顾性和小样本量存在一些局限性,但我们4年的随访结果显示,在适应症良好的患者中,核心摘除方法的效果很好。
{"title":"Long-term outcomes after core extirpation of fibrous dysplasia of the zygomaticomaxillary region.","authors":"Joseph Kyu-Hyung Park,&nbsp;Se Yeon Lee,&nbsp;Jong-Ho Kim,&nbsp;Baek-Kyu Kim","doi":"10.7181/acfs.2023.00038","DOIUrl":"https://doi.org/10.7181/acfs.2023.00038","url":null,"abstract":"<p><strong>Background: </strong>Fibrous dysplasia (FD) is a localized bone disorder in which fibro-osseous tissue replaces the normal bone structure. Patients with craniofacial FD often present with gradual swelling, deformity, and compromised vision or hearing. We previously introduced \"the core extirpation method,\" a novel surgical technique that is minimally invasive like traditional bone shaving but has longer-lasting effects. This study presents the long-term outcomes of our core extirpation method.</p><p><strong>Methods: </strong>We conducted a retrospective analysis of patients who underwent core extirpation for FD of the zygomaticomaxillary region from 2012 through 2021. Computed tomography (CT) scans were performed 6 to 12 months before the operation, immediately before and after the operation, and during follow-up visits. We performed all operations using the upper gingivobuccal approach, and we extirpated the core of the lesion while preserving the cortical structures of the zygoma and the maxilla to maintain symmetrical facial contour.</p><p><strong>Results: </strong>In 12 patients with lesions in the growth phase, anteroposterior/mediolateral (AP/ML) length discrepancies and the volume increased between preoperative and immediate postoperative CT scans. All patients' immediate postoperative AP/ML discrepancies were stable up to 12-17 months postoperatively. Postoperative volume showed continuous lesion growth; the median volume growth rate was 0.61 cc per month.</p><p><strong>Conclusion: </strong>In this article, we present our experiences managing FD using the minimally invasive core extirpation technique, which entails small expected blood loss and can be performed as day surgery. It provides similar cosmetic outcomes as traditional bone shaving but with longer-lasting results. Although there are some limitations with the study's retrospective nature and small sample size, our 4-year follow-up results show promising results of the core extirpation method in well-indicated patients.</p>","PeriodicalId":52238,"journal":{"name":"Archives of Craniofacial Surgery","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/b0/03/acfs-2023-00038.PMC10165236.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9431266","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 1
Current concepts of craniofacial fibrous dysplasia: pathophysiology and treatment. 颅面纤维结构不良的当前概念:病理生理学和治疗。
Q2 Medicine Pub Date : 2023-04-01 DOI: 10.7181/acfs.2023.00101
Dong Yeon Kim

Fibrous dysplasia is an uncommon genetic disorder in which bone is replaced by immature bone and fibrous tissue, manifesting as slowgrowing lesions. Sporadic post-zygotic activating mutations in GNAS gene result in dysregulated GαS-protein signaling and elevation of cyclic adenosine monophosphate in affected tissues. This condition has a broad clinical spectrum, ranging from insignificant solitary lesions to severe disease. The craniofacial area is the most common site of fibrous dysplasia, and nine out of 10 patients with fibrous dysplasia affecting the craniofacial bones present before the age of 5. Surgery is the mainstay of treatment, but the technique varies according to the location and severity of the lesion and associated symptoms. The timing and indications of surgery should be carefully chosen with multidisciplinary consultations and a patient-specific approach.

纤维发育不良是一种罕见的遗传性疾病,其中骨骼被未成熟的骨骼和纤维组织所取代,表现为生长缓慢的病变。GNAS基因的偶发性合子后激活突变导致g α s蛋白信号传导失调,影响组织中环磷酸腺苷水平升高。这种疾病具有广泛的临床范围,从轻微的孤立病变到严重的疾病。颅面区是纤维性发育不良最常见的部位,10个患者中有9个在5岁之前出现影响颅面骨的纤维性发育不良。手术是治疗的主要方法,但技术根据病变的位置和严重程度以及相关症状而有所不同。手术的时机和适应症应仔细选择多学科咨询和患者具体的方法。
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引用次数: 2
期刊
Archives of Craniofacial Surgery
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