• Title/Summary/Keyword: Craniofacial anomalies

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Split orthodontic airway plate: An innovation to the utilization method of conventional orthodontic airway plate for neonates with Robin sequence

  • Choo, HyeRan;Kim, Seong-Hun;Ahn, Hyo-Won;Poets, Christian F.;Chung, Kyu-Rhim
    • The korean journal of orthodontics
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    • v.52 no.4
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    • pp.308-312
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    • 2022
  • Since the emergence of neonatal infant orthodontics for treatments of cleft lip and palate with or without Robin sequence (RS) in Europe in the 1950s, advancements in design and scope of its application have been remarkable. As the first institution to adopt orthodontic airway plate (OAP) treatment in the United States in 2019, we saw a need for innovation of the original design to streamline the most labor-intensive and time-consuming aspects of OAP utilization. A solution is introduced using a systematic split expansion mechanism to re-size the OAP periodically to accommodate the neonate's maxillary growth. To date, seven RS patients have received this modified treatment protocol at our institution. Each patient completed full treatment using only one OAP. This innovative utilization method is aptly named the split orthodontic airway plate (S-OAP). Details of the S-OAP and its modifications from conventional OAP are reported.

Cartilage tissue engineering for craniofacial reconstruction

  • Kim, Min-Sook;Kim, Hyung-Kyu;Kim, Deok-Woo
    • Archives of Plastic Surgery
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    • v.47 no.5
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    • pp.392-403
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    • 2020
  • Severe cartilage defects and congenital anomalies affect millions of people and involve considerable medical expenses. Tissue engineering offers many advantages over conventional treatments, as therapy can be tailored to specific defects using abundant bioengineered resources. This article introduces the basic concepts of cartilage tissue engineering and reviews recent progress in the field, with a focus on craniofacial reconstruction and facial aesthetics. The basic concepts of tissue engineering consist of cells, scaffolds, and stimuli. Generally, the cartilage tissue engineering process includes the following steps: harvesting autologous chondrogenic cells, cell expansion, redifferentiation, in vitro incubation with a scaffold, and transfer to patients. Despite the promising prospects of cartilage tissue engineering, problems and challenges still exist due to certain limitations. The limited proliferation of chondrocytes and their tendency to dedifferentiate necessitate further developments in stem cell technology and chondrocyte molecular biology. Progress should be made in designing fully biocompatible scaffolds with a minimal immune response to regenerate tissue effectively

Modified two flap palatoplasty in asymptomatic transsphenoidal encephalocele: a case report

  • Richardson, Sunil;Khandeparker, Rakshit Vijay;Raghuvaram, Ajit Kumar;Mohan, Ram
    • Journal of the Korean Association of Oral and Maxillofacial Surgeons
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    • v.44 no.2
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    • pp.86-90
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    • 2018
  • About one-third of patients with transsphenoidal basal encephaloceles have associated congenital anomalies, including cleft palate. Moreover, they are often plagued by symptomatic exacerbations in the form of upper respiratory obstructions, cerebrospinal fluid leaks, meningitis, etc., with few patients being asymptomatic. We herein present a rare asymptomatic case of transsphenoidal basal encephalocele in an 18-month-old child with cleft palate and highlight a modified version of two-flap palatoplasty.

Pituitary Adenoma Accompanying Sphenoidal Meningoencephalocele with Clival Extension

  • Kim, Tae-Joon;Kim, Choong-Hyun;Cheong, Jin-Hwan;Kim, Jae-Min
    • Journal of Korean Neurosurgical Society
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    • v.38 no.3
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    • pp.242-244
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    • 2005
  • Encephaloceles are known as rare craniofacial developmental anomalies which are herniation of cranial contents through a skull defect. Sphenoidal meningoencephalocele represents an important cause of reversible visual loss. We report a rare case of pituitary adenoma accompanying sphenoidal meningoencephalocele with clival extension. Although the definitive diagnosis of cystic lesions in the sellar region before surgery is difficult, accurate diagnosis of these lesions is important to determine the type of treatment and predict prognostic outcome.

Congenital unilateral hypoplasia of depressor anguli oris muscle in adult

  • Oh, Suk Joon
    • Archives of Craniofacial Surgery
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    • v.20 no.4
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    • pp.265-269
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    • 2019
  • Congenital hypoplasia of the depressor anguli oris muscle is a rare cause of asymmetrical crying facies in newborns. The clinical manifestations range from mild to severe asymmetry and may persist up to adulthood. In the current case, the patient did not exhibit other congenital anomalies or paralysis of other branches of the facial nerve. This adult patient presented with severe asymmetrical lower lip deformity during full mouth opening since birth. A chromosomal study for the detection of 22q gene deletion yielded negative results. The electromyography findings of the lower lip were insignificant. Depressor labii inferioris muscle resection was not effective, but bidirectional (horizontal and vertical) fascia lata grafting improved the aesthetic appearance of the asymmetrical lower lip. The patient showed improved lower lip symmetry during full mouth opening at 1 year after the surgery. Therefore, the details of this rare case are reported herein.

A modified presurgical alveolar molding technique for treatment of cleft in Down syndrome

  • Gonca, Merve;Ozel, Mehmet Birol
    • The korean journal of orthodontics
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    • v.51 no.6
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    • pp.428-434
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    • 2021
  • Craniofacial clefts are extremely rare deformities. Tessier's classification is a widely accepted system that is based on clinical, radiographical, and surgical observations. The Tessier No. 0 cleft most commonly affects the upper lip, nose, and palate. This case presentation aims to report the outcome of a modified presurgical alveolar molding (PAM) appliance used in the treatment of an infant with Tessier No. 0 cleft as an alternate approach to mold such defects before surgery. The modified PAM appliance consisted of reciprocal parts connected by a helix. The segments were approximated by stripping the appliance at the midline in a V-shaped manner and the force was exerted by the extraoral elastics. The procedure gave results in 8 weeks, which may be regarded as a reasonable duration. The anterior cleft gap, which was 13 mm before the treatment, was reduced to 3 mm after the treatment by using modified PAM appliance. On a 21-month follow-up period, oral reshaping was regarded successful due to stability of the improved oral mold.

Cervical chondrocutaneous remnant: a case report

  • Park, Dae Hwan;Lee, June Key;Baik, Bong Soo;Yang, Wan Suk;Kim, Sun Young
    • Archives of Craniofacial Surgery
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    • v.23 no.5
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    • pp.241-245
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    • 2022
  • Cervical chondrocutaneous branchial remnants are very rare congenital lesions of the lateral neck; thus, our knowledge of this condition derives almost entirely from occasional case reports in the literature. They are thought to originate from the branchial arches and, therefore, can be found anywhere on the pathway along which those branchial arches migrate during embryogenesis. We report the case of a 5-year-old girl presenting with a cervical chondrocutaneous branchial remnant on the right lateral neck that had existed since birth, with no other anomalies.

Clinical and radiographic characteristics of pycnodysostosis: A systematic review

  • Amanda Katarinny Goes Gonzaga;Carla Samily de Oliveira Costa;Hannah Gil de Farias Morais;Brazm da Fonseca Neto;Leao Pereira Pinto;Wagner Ranier Maciel Dantas;Patricia Teixeira de Oliveira;Daniela Pita de Melo
    • Imaging Science in Dentistry
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    • v.54 no.1
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    • pp.13-24
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    • 2024
  • Purpose: Pycnodysostosis (PYCD), an autosomal recessive syndrome, is characterized by an imbalance in bone remodeling that produces various clinical and radiographic craniofacial manifestations. This review represents a systematic examination of these manifestations, as well as oral features associated with PYCD. Materials and Methods: A systematic review was conducted across 8 databases from February to March 2023. The search strategy focused on studies reporting cases of PYCD that examined the clinical and radiographic craniofacial and oral characteristics associated with this syndrome. Results: The review included 84 studies, encompassing a total of 179 cases of PYCD. More than half of the patients were female (55.3%), and the mean age was 14.7 years. Parental consanguinity was reported in 51.4% of the cases. The most common craniofacial clinical manifestation was a prominent nose, observed in 57.5% of cases. Radiographically, the most frequently reported craniofacial characteristics included the presence of an obtuse mandibular angle (84.3%) and frontal cranial bosses(82.1%). Clinical and radiographic examinations revealed oral alterations, with micrognathia present in 62.6% of patients and malocclusion in 59.2%. Among dental anomalies, tooth agenesis was the most commonly reported, affecting 15.6% of patients. Conclusion: Understanding the clinical and radiographic craniofacial features of PYCD is crucial for dental professionals. This knowledge enables these clinicians to devise effective treatment plans and improve patient quality of life.

A Case Report of Congenital Eyelid Defect without Any Other Deformities (다른 기형을 동반하지 않은 선천성 상안검 결손증 1례 보고)

  • Moon, Jae Won;Yang, Jeong Yeol
    • Archives of Craniofacial Surgery
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    • v.9 no.2
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    • pp.90-92
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    • 2008
  • Purpose: Congenital upper eyelid defect is rare anomaly whether it is compared with syndromic anomaly or not. It has many clinical manifestation in the extent, location. Many operation procedures such as simple closure, semicircular rotation flap, Cutler Beard procedure, lower lid rotation flap, etc can be used to reconstruct eyelid defects. We intend to introduce a simple, congenital eyelid defect which was not compared with syndromical anomaly, ophthalmic complication. Methods: Our experience is a case of 19 years old female who had a upper eyelid coloboma without any other anomalies. we could not find any skeletal deformity in orbital CT scan. she had no ophthalmic problem. we reconstructed the defect with bilateral marginal flap after deepithelization of supramarginal area and tarsal reposition. Results: There were no visible deformity of lid lining. postoperative scar was favorable. satisfactory results were obtained in cosmetic and functional aspects. Conclusion: Upper lid coloboma without other anomalies is rare. we obtained satisfactory outcome as treated this rare case with marginal flap advancement.

Characterization of phenotypes and predominant skeletodental patterns in pre-adolescent patients with Pierre-Robin sequence

  • Yang, Il-Hyung;Chung, Jee Hyeok;Lee, Hyeok Joon;Cho, Il-Sik;Choi, Jin-Young;Lee, Jong-Ho;Kim, Sukwha;Baek, Seung-Hak
    • The korean journal of orthodontics
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    • v.51 no.5
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    • pp.337-345
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    • 2021
  • Objective: To investigate the phenotypes and predominant skeletodental pattern in pre-adolescent patients with Pierre-Robin sequence (PRS). Methods: The samples consisted of 26 Korean pre-adolescent PRS patients (11 boys and 15 girls; mean age at the investigation, 9.20 years) treated at the Department of Orthodontics, Seoul National University Dental Hospital between 1998 and 2019. Dental phenotypes, oral manifestation, cephalometric variables, and associated anomalies were investigated and statistically analyzed. Results: Congenitally missing teeth (CMT) were found in 34.6% of the patients (n = 9/26, 20 teeth, 2.22 teeth per patient) with 55.5% (n = 5/9) exhibiting bilaterally symmetric missing pattern. The mandibular incisors were the most common CMT (n = 11/20). Predominant skeletodental patterns included Class II relationship (57.7%), posteriorly positioned maxilla (76.9%) and mandible (92.3%), hyper-divergent pattern (92.3%), high gonial angle (65.4%), small mandibular body length to anterior cranial base ratio (65.4%), linguoversion of the maxillary incisors (76.9%), and linguoversion of the mandibular incisors (80.8%). Incomplete cleft palate (CP) of hard palate with complete CP of soft palate (61.5%) was the most frequently observed, followed by complete CP of hard and soft palate (19.2%) and CP of soft palate (19.2%) (p < 0.05). However, CP severity did not show a significant correlation with any cephalometric variables except incisor mandibular plane angle (p < 0.05). Five craniofacial and 15 extra-craniofacial anomalies were observed (53.8% patients); this implicated the need of routine screening. Conclusions: The results might provide primary data for individualized diagnosis and treatment planning for pre-adolescent PRS patients despite a single institution-based data.