Jaw Abnormalities

颌骨异常
  • 文章类型: Case Reports
    背景:本病例报告在出生36周时健康的男婴中表现出罕见的先天性异常。婴儿被诊断为先天性上颌关节粘连,ectrodactyly,和强直舌肌综合征(ASS)。
    方法:无法完全张开嘴,喂养挑战,和腭裂.婴儿在出生时通过面罩成功的正压通气稳定,并通过喂养胃造口术开始肠内喂养。
    结果:诊断测试显示中线腭裂,发育不良的下巴,持续性异位缝合,和中线的骨融合。
    方法:沿中线切开骨刺,并在操作后实现2厘米的张口。病人正在随访中,未来的治疗计划包括在12个月时进行腭裂矫正,并根据生长轨迹进行潜在的额下颌和下颌前移。
    此病例强调了处理多种先天性异常的复杂性以及对个性化治疗计划的需求。
    BACKGROUND: This case report presents a rare combination of congenital anomalies in an otherwise healthy male infant born at 36 weeks. The infant was diagnosed with congenital maxillomandibular synechia, ectrodactyly, and ankyloglossia superior syndrome (ASS).
    METHODS: Inability to open the mouth completely, feeding challenges, and a cleft palate. The infant was stabilized through successful positive pressure ventilation via a face mask at birth and enteral feeding was initiated via a feeding gastrostomy.
    RESULTS: Diagnostic tests revealed a midline palatal cleft, hypoplastic jaws, persistent metopic suture, and a bony fusion at the midline.
    METHODS: Sectioning of the bony spur along the midline and achieving a mouth opening of 2 cm post-manipulation. The patient is under follow-up, with future treatment plans including cleft palate correction at 12 months and potential frontomandibular and lower jaw advancement depending on growth trajectories.
    UNASSIGNED: This case underscores the complexity of managing multiple congenital anomalies and the need for individualized treatment plans.
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  • 文章类型: Case Reports
    先天性上颌下颌融合术是一种罕见的疾病。上颌下颌融合术通常在出生后立即发现,因为新生儿无法张口或正常进食。融合缺陷可以呈现广泛的严重程度,从粘膜带(粘连)到完全骨性融合(合颌)。
    我们报告一例3天大的男性新生儿先天性牙槽粘连伴后裂。新生儿是在多学科方法的帮助下进行管理的。
    牙龈融合是一种非常罕见的先天性异常。纤维带的早期分裂不仅可以有效进食,而且可以防止面部畸形的发展。然而,这个过程的麻醉可能是具有挑战性的。
    UNASSIGNED: Congenital maxillomandibular fusion is a rare disorder. Maxillomandibular fusion is usually discovered immediately after birth because the newborn is unable to open mouth or feed normally. The fusion defects can present with a wide range of severity, ranging from mucosal band (synechiae) to complete bony fusion (syngnathia).
    UNASSIGNED: We report a case of congenital alveolar synechiae with posterior cleft palate in a 3-day-old male neonate. The newborn was managed with the help of a multi-disciplinary approach.
    UNASSIGNED: Fusion of the gums is a very rare congenital anomaly. Early division of fibrous bands not only allows effective feeding but also prevents the development of facial deformities. However, anesthesia for this procedure can be challenging.
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  • 文章类型: Journal Article
    目的:单侧颞下颌关节强直伴颌骨畸形(UTMJAJD)可能需要同时进行全关节假体(TJP)重建,矢状裂支(SSRO),还有LeFortI截骨术.这项研究的目的是评估接受这些手术治疗的患者的预后。
    方法:选择2016年至2018年诊断为UTMJAJD的患者进行研究。在强直释放后,在强直侧使用TJP,在对侧使用SSRO进行下颌骨先行手术。最后进行了LeFortI截骨术,有或没有进行生殖器成形术。最大门牙开口(MIO),面部对称,并比较了颌骨和髁的稳定性,手术后,在后续行动中。
    结果:7名患者被纳入研究。他们的平均下巴偏差为9.5±4.2mm,上颌斜度为5.1±3.0°。手术后,颌骨畸形明显改善,下巴偏差校正7.6±4.1mm(p=0.015),前进5.9±2.5mm(p=0.006)。平均随访26.6±17.1个月,MIO从11.4±9.3显著增加到35.7±2.6(p=0.000)。咬合稳定,下颌位置或旋转无明显变化(p>0.05)。随访期间未见明显髁突吸收。
    结论:同时TJP重建,SSRO,LeFortI型截骨术是治疗UTMJAJD的可靠有效方法。
    OBJECTIVE: Unilateral temporomandibular joint ankylosis with jaw deformity (UTMJAJD) may require simultaneous total joint prosthesis (TJP) reconstruction, sagittal split ramus (SSRO), and Le Fort I osteotomies. The purpose of this study was to evaluate outcomes in patients treated with these procedures.
    METHODS: Patients diagnosed UTMJAJD between 2016 and 2018 were selected for the study. Mandible-first procedure was performed after ankylosis release with TJP on the ankylosed side and SSRO on the contralateral side. Le Fort I osteotomy with and without genioplasty was lastly performed. Maximal incisor opening (MIO), facial symmetry, and jaw and condyle stability were compared before, after operation, and during follow-ups.
    RESULTS: Seven patients were included in the study. Their average chin deviation was 9.5 ± 4.2 mm, and maxillary cant was 5.1 ± 3.0°. After operation, jaw deformity significantly improved, with chin deviation corrected 7.6 ± 4.1 mm (p = 0.015) and advanced 5.9 ± 2.5 mm (p = 0.006). After an average follow-up of 26.6 ± 17.1 months, MIO significantly increased from 11.4 ± 9.3 to 35.7 ± 2.6 mm (p = 0.000). The occlusion was stable with no significant positional or rotational changes of the jaw (p > 0.05). There was no obvious condylar resorption during follow-ups.
    CONCLUSIONS: Simultaneous TJP reconstruction, SSRO, and Le Fort I osteotomy are reliable and effective methods for the treatment of UTMJAJD.
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  • 文章类型: Journal Article
    未经批准:确定治疗MarcusGunn眨眼综合征(MGJWS)患者的基本原理。
    UNASSIGNED:回顾性回顾38例MGJWS患者,转诊至一家高等教育机构。临床数据包括视力,眼运动性,下巴眨眼的一侧,有无上睑下垂,提升机功能,临床照片,和管理。由高级外科医生(FQL)对32例患者进行了定制手术。
    UNASSIGNED:无下垂或轻度下垂的病例采用保守治疗。在中度上睑下垂和可忽略的综合症的情况下,提肌前移(LA)是成功的,但导致了更明显的综合症。严重上睑下垂患者的提肌切除术(LR)与上睑复发率高有关。在接受单或双侧提上肌切除术(LE)和双侧额肌悬吊(FS)或单侧额肌皮瓣(FF)的所有患者中,下垂均得到了充分纠正。所有接受LE治疗的患者的颌骨眨眼均消失,但在后期有三例复发。在相关的内斜视或下斜视的情况下,同时进行斜视手术。
    UNASSIGNED:中度上眼睑可以用LA矫正,但是成功与提上肌功能无关,术后联合运动变得更加明显。在严重上睑下垂时,LR显示不可预测的结果。如果有严重的下垂和严重的联合运动,建议使用单侧或双侧LE和双侧FS;单侧FF是拒绝双侧治疗的患者的替代方案,因为美容结果通常比单侧FS后好。
    UNASSIGNED: To identify a rationale for treatment of patients with Marcus Gunn jaw winking syndrome (MGJWS).
    UNASSIGNED: Retrospective review of 38 consecutive patients with MGJWS referred to a single tertiary institution. Clinical data included visual acuity, ocular motility, side of jaw-wink, presence or absence of ptosis, levator function, clinical photographs, and management undertaken. Thirty-two patients were operated on with customized surgery by a senior surgeon (FQL).
    UNASSIGNED: Cases with no ptosis or mild ptosis were managed conservatively. Levator advancement (LA) was successful in case of moderate ptosis and negligible synkynesis but resulted in a more evident synkinesis. Levator resection (LR) in patients with severe ptosis was associated with high rate of ptosis recurrence. Ptosis was adequately corrected in all patients submitted to uni- or bilateral levator excision (LE) and bilateral frontalis suspension (FS) or unilateral frontalis flap (FF). Jaw winking resolved in all patients submitted to LE but recurred in three cases at a later stage. Strabismus surgery was performed simultaneously in case of associated esotropia or hypotropia.
    UNASSIGNED: Moderate ptosis can be corrected with LA, but success is not related to levator function and synkinesis becomes more evident postoperatively. In severe ptosis, LR showed unpredictable results. In case of severe ptosis and severe synkinesis, uni- or bilateral LE and bilateral FS are recommended; unilateral FF is an alternative in patients who refuse bilateral treatment, as the cosmetic outcome is usually better than after unilateral FS.
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  • 文章类型: Case Reports
    腭裂-外侧粘连综合征(CPLSS)是一种极为罕见的先天性畸形综合征,病因不明。其特征是腭裂和口内外侧粘连,将腭的自由边界连接到口腔底。我们报道了一个女性新生儿的病例,因与多个口内外侧粘连相关的唇裂和腭裂而导致的吸乳困难而入院。切除粘连,允许口服喂养。腭裂-外侧粘连综合征是一种特殊综合征,因为文献中仅报道了17例。可以分离或更频繁地与其他先天性异常例如唇裂和/或腭裂相关联。这些粘连会导致功能缺陷,尤其是在呼吸道和呼吸道,语言障碍或复发性中耳炎。虽然是例外,医生必须了解这种畸形实体,才能建立适应良好的治疗方案。
    Cleft palate-lateral synechiae syndrome (CPLSS) is an extremely rare congenital malformation syndrome with undetermined etiology, characterized by a cleft palate and lateral intraoral synechiae linking the free borders of the palate to the mouth floor. We report a case of a female neonate, admitted for suckling difficulties with a cleft lip and palate associated to multiple lateral intraoral synechiae. Resection of the synechiae allowed oral feeding. Cleft palate-lateral synechiae syndrome is an exceptional syndrome as only seventeen cases have been reported in the literature. Synechiae can be isolated or more frequently in association with other congenital anomalies such as cleft lip and/or palate. These synechiae can cause functional deficits, especially in the respiratory and feeding tracts, language disorders or recurrent otitis. Although it is exceptional, this malformative entity must be known by medical practitioners in order to set up a well-adapted therapeutic protocol.
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  • 文章类型: Case Reports
    凹痕(DI)通常会引起冠部和根部的异形变化。该病例报告介绍了上颌侧切牙的复杂3a型DI,该切牙具有唇齿尖和根分叉。尽管舌齿尖在上颌侧切牙中很常见,唇齿尖头是罕见的。上颌侧切牙中的辅助pal根也不常见。文献中没有描述涉及所有三种变化的这种情况。根据舒尔茨和布兰德的说法,DI被归类为B4型,因为它涉及纸浆和根的分裂。DI由正行MTA管理,对腭根进行了放射切除术和牙周再生。与DI相关的唇爪可导致早期牙周/牙髓受累。类型3DI可以影响具有明显扩张和分裂的根。应在3a型DI中仔细检测其他pal根,并借助CBCT进行管理。
    Dens invaginatus (DI) often causes dysmorphic changes in both the crown and root. This case report presents a complicated type 3a DI in a maxillary lateral incisor with labial talon cusp and root bifurcation. Although lingual talon cusp is common in maxillary lateral incisor, labial talon cusp is rare. An auxiliary palatal root in maxillary lateral incisor is also unusual. No such case involving all three variations has been described in the literature. The DI was classified as type B4 according to Schulze and Brand, as it involved division of pulp and root. DI was managed by orthograde MTA, radisectomy and periodontal regeneration was done for the palatal root. Labial talon associated with DI and can lead to early periodontal/pulpal involvement. Type 3 DI can affect the root with marked dilatation and division. Additional palatal root should be carefully detected in type 3a DI and managed with the aid of CBCT.
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  • 文章类型: Case Reports
    计划对一名10个月大的女孩进行手术融合分离,该女孩患有四花综合征和先天性上颌下颌融合术。这种情况下的麻醉管理相当具有挑战性。标准监测仍适用于患者的四肢。怀疑静脉进入是困难的,但在紧急情况下插管以提供复苏之前必须坚决需要。连接麻醉回路与鼻咽气道是首选技术,因为它的好处,如保持自发通气,提供吸入麻醉剂,以及监测氧合和通气。重要的是,处理此类复杂案件的基石是多学科方法和团队合作。
    A 10-month-old girl who had tetra-amelia syndrome and congenital maxillomandibular fusion (syngnathia) was scheduled for the surgical fusion separation. Anesthetic management for this case was considerably challenging. Standard monitoring was still applied to the patient\'s extremities. IV access was suspected to be difficult but firmly needed before intubation to provide resuscitation during an emergency. Connecting anesthetic circuit with nasopharyngeal airway was the preferred technique due to its benefits such as maintaining spontaneous ventilation, providing inhaled anesthetic, as well as monitoring oxygenation and ventilation. Importantly, the cornerstones for handling such complicated cases are multidisciplinary approach and teamwork.
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  • 文章类型: Review
    先天性颌骨融合(合颌)是一种罕见的面部畸形,病因不明。这种疾病的严重程度可能随软组织粘连和骨融合而变化。可以是前路融合,单侧或双侧融合,完全融合。这些患者的主要问题是气道维护和喂养困难,术后最常见的并发症是骨融合复发。这里,我们报道了一个年轻的男性患者,患有骨性同座症,涉及下颌骨的上升支和身体与上颌复合体的双侧融合。我们通过计算机辅助的术前计划进行了骨隔离,并使用了插入的颞皮瓣固定了伤口表面,以防止骨重新融合。
    Congenital fusion of the jaws (syngnathia) is a rare facial malformation with an unknown etiology. This disease may vary in severity with adhesion of soft tissue and bony fusion. It can be anterior fusion, unilateral or bilateral fusion, and complete fusion. The main problem of these patients is the difficulty of airway maintenance and feeding, and the most common postoperative complication is the relapse of bony fusion. Here, we report a young male patient with bony syngnathia, involving bilateral fusion of the ascending ramus and body of the mandible with the maxillary complex. We performed bone isolation by computer-assisted preoperative planning and used an insertional temporalis flap to fix the wound surface to prevent refusion of bone.
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  • 文章类型: Case Reports
    Gorham病是一种罕见的疾病,其特征是骨的进行性骨溶解最终导致骨的完全消失。病因不明,疾病主要累及骨盆,肱骨,和轴向骨架。因为它不寻常,模棱两可的呈现和罕见的发生,这种疾病无法被识别,并且经常被其他疾病所掩盖。这种疾病的诊断是通过排除。迄今为止,据报道约有50例涉及颌面部的Gorham病,其中大多数涉及下颌骨。仅在4例中记录了上颌骨的唯一受累。我们报告了一名68岁的男性患者的上颌骨Gorham病的特殊情况,该患者患有慢性疼痛并伪装成牙源性感染。
    Gorham\'s disease is a rare condition characterized by progressive osteolysis of bone ultimately resulting in the total disappearance of bone. The etiology is unknown and the disease predominantly affects the pelvis, humerus, and axial skeleton. Because of its unusual, ambiguous presentation and rare occurrence, the disorder goes unrecognized and is often masqueraded by other disorders. The diagnosis of this disorder is by exclusion. About 50 cases of Gorham\'s disease involving the maxillofacial region are reported to date and most of them involve the mandible. Exclusive involvement of maxilla is documented in only 4 cases. We report an exceptional case of Gorham\'s disease of the maxilla in a 68-year-old male patient presenting with chronic pain and masquerading as an odontogenic infection.
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  • 文章类型: Case Reports
    岩尖是金字塔形结构,由于解剖位置困难,难以检查。岩尖的病变可以通过手术治疗,也可以是偶然的病变,这是保守管理的。岩尖头颅囊肿(PAC)是由于颞骨Meckel洞穴中的疝引起的囊性病变。双侧PAC是一种非常罕见的现象,迄今为止文献中仅报道了21例。我们在这里介绍一个双边PAC的案例,他出现了头痛,并得到了保守的管理。
    The petrous apex is a pyramidal-shaped structure which is difficult to examine due to difficult anatomical location. Lesions in the petrous apex can be managed surgically or they can be incidental lesions, which are managed conservatively. Petrous apex cephaloceles (PAC) are the cystic lesion due to herniation in the Meckel\'s cave of temporal bone. Bilateral PAC is a very rare phenomenon with only 21 cases reported in the literature so far. We present here a case of bilateral PAC, who presented with headache and was managed conservatively.
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