• Title/Summary/Keyword: Congenital facial deformity

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Oral findings and dental management of a patient with Moebius syndrome: a case report

  • Lee, Eunkyoung;Kim, Youngjin;Kim, Hyunjung;Nam, Soonhyeun
    • The Journal of Korea Assosiation for Disability and Oral Health
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    • v.10 no.2
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    • pp.101-105
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    • 2014
  • Moebius syndrome is a rare, congenital neurological disease involving facial paralysis and limitation of eye movements. It results from maldevelopment of the sixth and seventh cranial nerves. Dental features of this syndrome include micrognathia, microstomia, tongue deformity, cleft palate, hypoplasia of the teeth, and congenital missing teeth. A 7-year-old female with Moebius syndrome was referred from a local dental clinic for caries treatment. She presented with facial paralysis and microstomia. Oral findings included multiple caries with enamel hypoplasia, congenital missing teeth, and tongue deformity. Dental treatments including restorative and preventive procedures were performed. Oral findings and management aspects of Moebius syndrome for this case are discussed. Early evaluation and multidisciplinary care are needed for children with Moebius syndrome.

Multidirectional Vector Excision Leads to Better Outcomes than Traditional Elliptical Excision of Facial Congenital Melanocytic Nevus

  • Oh, Seung Il;Lee, Yoon Ho
    • Archives of Plastic Surgery
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    • v.40 no.5
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    • pp.570-574
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    • 2013
  • Background The elliptical excision is the standard method of removing benign skin lesions, such as congenital melanocytic nevi. This technique allows for primary closure, with little to no dog-ear deformity, but may sacrifice normal tissue adjacent to the lesion, resulting in scars which are unnecessarily long. This study was designed to compare the predicted results of elliptical excision with those resulting from our excision technique. Methods Eighty-two patients with congenital melanocytic nevus on the face were prospectively studied. Each lesion was examined and an optimal ellipse was designed and marked on the skin. After an incision on one side of the nevus margin, subcutaneous undermining was performed in the appropriate direction. The skin flap was pulled up and approximated along several vectors to minimize the occurrence of dog-ear deformity. Results Overall, the final wound length was 21.1% shorter than that achieved by elliptical excision. Only 8.5% of the patients required dog-ear repair. There was no significant distortion of critical facial structures. All of the scars were deemed aesthetically acceptable based on their Patient and Observer Scar Assessment Scale scores. Conclusions When compared to elliptical excision, our technique appears to minimize dogear deformity and decrease the final wound length. This technique should be considered an alternative method for excision of facial nevi.

Reconstruction of the Acquired Facial Deformity due to Cancrum Oris Sequelae (괴저성 구내염 후유증에 의한 후천성 안면 변형의 재건)

  • Moon, Jae Won;Lee, Seung Chan;Cheon, Ji Seon;Yang, Jeong Yeol
    • Archives of Plastic Surgery
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    • v.33 no.3
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    • pp.359-366
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    • 2006
  • Acquired facial deformities following cancrum oris sequelae manifested variably according to the nature of tissue necrosis. In cases that tissue loss extends over a wide area of the face, or the tissue nature is different due to congenital facial cleft, it is difficult to reconstruct with a single operation. As cancrum oris has virtually disappeared from our country, clinical report of reconstruction is also rare. We report 5 cases of facial deformities following cancrum oris sequelae. Since 1988, five adult patients(4 female and 1 male) were treated by authors. These patients, with an age ranged from 47 to 58 years, all suffered from acquired facial cleft such as facial mutilation, asymmetry. The stages of operation were from 1 to maximum of 5 operations. All surgeries achieved satisfactory results after a long-term follow-up. But one surgery in the case of palatal mucosal flap for the coverage of reconstructed maxilla alveolar bone resulted tissue sloughness, followed by osteomyelitis. Those were debrided and discarded. In conclusion, all sites of deformities were positioned around one of the oral commissures. In their past medical history, they have been suffering from measles, typhoid fever and unknown febrile illness. We diagnosed the acquired facial deformity following cancrum oris sequelae. The reconstruction of acquired facial deformity following cancrum oris sequelae were difficult due to extensive multiple tissue defects. Therefore multiple staged operations were inevitable. The authors reconstructed 5 cases of simple and complex form of facial deformity with minimum staged operations. All patients were satisfied functionally and cosmetically.

Clinical Experiences on the Treatment of Congenital Cutis Aplasia and Craniosynostosis (선천성 두피결손증 및 두개골 조기유합증의 치험 5례)

  • Lee, Kyung-Ho
    • Journal of Yeungnam Medical Science
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    • v.10 no.2
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    • pp.493-505
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    • 1993
  • The congenital cutis aplasia mainly occurs in head, sometimes involving the skull and dura mater. It's cause and the rate of falling ill are not known yet, it is the disease that rate of death is high by the infection, such as, the injured vascular hemorrhange of meningitis. Craniosynostosis is the disease the appears the skull as well as the facial deformity with growing, has from the developmental difficiency, visual distibance, motor disturbance, convulsion to the neurologic impairment of mental retardation, and accompanies the each characteristic deformity following the suture fused. Satisfactory results was achieved by local flap surgery and conservative treatment on the infant, diagnosed as the congenital cutis aplasia, case 1 Also successful treatment experiences of craniosynostosis(oxycephaly, brachycephaly, trigonocephaly, cloverleaf deformity) through the frontal bone advancement and the barrel stave asteotomy, were reviewed & pursues and investigates the intracranial volume of before and after of surgery, and then reports with the literature investigation.

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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.

임상가를 위한 특집 2 - 선천성 기형환자에서의 악교정수술 적용과 수술 증례 분석

  • Jung, Hwi-Dong;Jung, Young-Soo
    • The Journal of the Korean dental association
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    • v.50 no.11
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    • pp.670-676
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    • 2012
  • Distraction osteogenesis and orthognathic surgery are the widely used surgical methods for treating hemifacial microsomia and cleft lip and palate, the representative forms of congenital deformity. Distraction osteogenesis is an outstanding treatment of choice when more traction is needed than what can be achieved by general orthognathic surgery. However, the stability of distraction osteogenesis has not yet been established, and in most of the cases, additional orthognathic surgery is mandatory. Moreover, the difficulty in precise control of the traction directions is another disadvantage of distraction osteogenesis. Therefore, it would be desirable not to conduct distraction osteogenesis when the patient is suitable for an orthognathic surgery. Also, distraction osteogenesis should be recognized as an accessorial method of treatment, and be used restrictively.

Correction of Bilateral Tessier No. 2, 3, and 12 Facial Cleft with Anopthalmia

  • Moon, Seong-Yong;Kim, Seong-Gon;Park, Young-Ju;Park, Young-Wook
    • Maxillofacial Plastic and Reconstructive Surgery
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    • v.35 no.4
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    • pp.243-247
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    • 2013
  • Oblique facial cleft is a rare congenital deformity. Its incidence has been reported as 0.24% of all reported cases of facial cleft. We report on a patient who had a left-sided oblique facial cleft with anopthamia, including lip and palate, nose alar base, and medial canthus. The patient also had a right-sided oblique facial cleft, which included lip and palate, nose alar base, medial canthus, and upper eye brow. Primary closure of the facial cleft was performed using multiple Z-plasty after excision of scar tissue.

A Case Report of Noonan Syndrome with Mental Retardation and Attention-Deficit Hyperactivity Disorder (정신지체와 주의력결핍 과잉행동장애를 보이는 Noonan 증후군 1예)

  • Kim, Won-Woo;Shim, Se-Hoon
    • Journal of the Korean Academy of Child and Adolescent Psychiatry
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    • v.23 no.1
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    • pp.31-35
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    • 2012
  • Noonan syndrome is characterized by short stature, typical facial dysmorphology, and congenital heart defects. The main facial features of Noonan syndrome are hypertelorism with down-slanting palpebral fissures, ptosis, and low-set posteriorly-rotated ears with a thickened helix. The cardiovascular defects most commonly associated with this condition are pulmonary stenosis and hypertrophic cardiomyopathy. Other associated features are webbed neck, chest deformity, mild intellectual deficit, cryptorchidism, poor feeding in infancy, bleeding tendency, and lymphatic dysplasias. The patient is a 10-year-old boy. He had experienced repeated febrile convulsions. He had typical facial features, a short stature, chest deformity, cryptorchidism, vesicoureteral reflux, and mental retardation. His language and motor development were delayed. When he went to school, it was difficult for him to pay attention, follow directions, and organize tasks. He also displayed behavior such as squirming, leaving his seat in class, and running around inappropriately. Clinical observation is important for the diagnosis, so we report a patient who was diagnosed with Noonan syndrome, mental retardation, and attention-deficit hyperactivity disorder.

CASE REPORTS OF CONGENITAL DUPLICATED MAXILLA (선천성 상악골 중복기형의 치험례)

  • Lee, Beak-Soo;Choi, Hyun-Jung;Ryu, Dong-Mok;Oh, Jung-Hwan
    • Journal of the Korean Association of Oral and Maxillofacial Surgeons
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    • v.30 no.5
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    • pp.434-437
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    • 2004
  • Neural crest cells have embriologically important role for the development and growth in oral and maxillofacial region. If the early hereditary defect occurs or environmental factors affect these cells diminutive mesenchymal cells are disabled to make neural plate and decreased proliferation of mesenchymal cells result in hypoplastic development of neural crest. As a result, this brings about severe facial malformations such as various located facial clefts and/or loss or duplication of facial structure. These are two cases of accessory maxilla and zygomatic deformity with and without facial cleft.

A Study of Facial Deformity in the Patient with Bilateral Cleft Lip before the Primary Cheiolplasty (양측성 구순열 환자의 안모 변형에 대한 연구)

  • Yoon Bo-Keun;Soh Byung-Soo;Baik Jin-Ah;Shin Hyo-Keun
    • Korean Journal of Cleft Lip And Palate
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    • v.4 no.2
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    • pp.51-68
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    • 2001
  • Midfacial hypoplasia in patients with clefts of the lip and palate is considered to be the result of congenital dysmorphogenesis. And cleft lip and palate developes facial deformity, jaw abnormality, speech problem, which is most frequent hereditary deformity in maxillofacial region. So cleft lip and palate is characterized by midface deformity which shaws maxillary anterior nasal septal deviation and deformity. Our study describes congenital correlates of midfacial hypoplasia by examining the displacement of a normal complement of parts, a triangular tissue deficiency low on the lip border on the columellar side, and a linear deficiency and displacement in the line of the bilateral cleft lip. 15 patients with bilateral cleft lip and palate were taken impression before operation, but the patient who had other abnormalities and complications were excluded. Average age is 3.4 months and they were classified into both complete, both incomplete and complete & incomplete group. The obtained results were as follows 1. There were no differences on intercanthal width and canthal width between each of the groups. 2. Both complete group had longer lateral ala length than both incomplete group, but there were no differences between both complete group and complete side of com. & incom. group and both incomplete group and incomplete side of com. & incom. group. 3. Columella length was greater in both incomplete group than in both complete group, but there was no difference between both complete group and complete side of com. & incom. group and both incomplete group and incomplete side of com. & incom. group. 4. Both complete group had longer ala width & ala base width than both incomplete group had. But there were no differences between both complete group and complete side of com. & incom. group and both incomplete group and incomplete side of com. & incom. group. 5. There were no differences between each of the groups on upper lip length, but nose/mouth width ratio was greater in both complete group than in both incomplete group. 6. Pronasale(pm), subnasle(sn), la~rale superioris(ls), stomion(sto) points were located around the central vertical line of face but deviated to incomplete side in com. & incom. group. 7. Nasal tip protrusion was greater in both incomplete group and com. & incom. group than both complete group, but there was no difference between both incomplete group and com. & incom. group.

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