Goldenhar's Syndrome (oculo-auriculo-vertebral dysplasia) is a wide spectrum of congenital anomalies that involves structures arising from the first and second branchial arches. In this report, a case of a male infant, with the features of hemi facial microsomia, anotia, vertebral anomalies, congenital facial nerve palsy and lagophthalmos is described. Although the syndrome itself is not uncommon, the presence of congenital facial nerve palsy, which has been reported in rare cases, prompted this case report.
*Facial Nerve ; Facial Paralysis/*complications/congenital ; Goldenhar Syndrome/*complications ; Human ; Infant ; Male ; Scoliosis/complications/congenital/radiography

The tear trough deformity and the palpebromalar groove are characteristic appearances in midface aging. The aim of this study is to suggest the direction of aging process in midface by sampling survey in the Koreans. Sampling survey was conducted to 480 subjects who had preoperative pictures of the midface in Koreans. The excluding criteria of the subjects were with prior oculoplastic surgery, facial bone fracture, facial palsy, previous rejuvenation procedure, Grave's oculopathy and some medical history affecting eyelid position. Prevalence of tear trough deformity and palpebromalar groove in each subject, and age-related change in prevalence were analyzed. Prevalence of the tear trough deformity and the palpebromalar groove was 57.92% and 44.58%, respectively. Prevalence of tear trough deformity was higher than that of palpebromalar groove in both males and females(Chi-Square test, p<0.05). The tear trough deformity and the palpebromalar groove showed a strong positive correlation with age. All the subjects with the palpebromalar grooves had the tear trough deformities. From the results of survey, it suggested that the aging process of midface of the Korean, from medial to lateral direction, differed from that in westerns.
Aging ; Congenital Abnormalities ; Eyelids ; Facial Bones ; Facial Paralysis ; Humans ; Male ; Prevalence ; Rejuvenation

Melkersson-Rosenthal syndrome is a rare condition and clinical diagnosis was established on the basis of the triad: facial or lip edema, peripheral facial palsy and scrotal or plicated tongue. Labial swelling(Cheilitis granulomatosa) is the most common feature of this syndrome complex. The lips may enlarge up to three times their normal size, resulting in aesthetic deformity and functional disability. The condition appears to be a granulomatous disorder causing edema and inflammation of the soft tissues of the face, lips, oral cavity and particularly, the facial nerve. Traditional medical interventions are only marginally successful in treating this syndrome. We report the case of a young man with cheilitis granulomatosa as a manifestation of Melkersson-Rosenthal syndrome successfully treated by transverse and vertical star-shaped resection with a transmodiolar labial suspension suture at mouth angle. Our result shows symmetry of the lip, a normalized anterior projection, dimensional harmony between upper and lower lip and no disfiguring cicatrices.
Congenital Abnormalities ; Diagnosis ; Edema ; Facial Nerve ; Facial Paralysis ; Inflammation ; Lip ; Melkersson-Rosenthal Syndrome* ; Mouth ; Sutures ; Tongue

A female infant was admitted to the hospital due to perioral cyanosis two hours after birth. The infant was born at the gestational age of 35 weeks by cesarean section with a birth weight of 2 400 g. Physical examination revealed wry mouth to the left side while crying, small auricles, and high palatal arch; fibrolaryngoscopy suggested bilateral vocal cord paralysis; echocardiography suggested ventricular septal defect; single nucleotide polymorphism testing showed 22q11.21 microdeletion. Therefore, the infant was given a definite diagnosis of asymmetric crying facies syndrome accompanied by 22q11.21 microdeletion. After 8-month follow-up, the infant still had asymmetric crying facies with presence of growth retardation.
Cesarean Section ; Crying ; Facial Paralysis ; Female ; Heart Defects, Congenital ; Humans ; Infant ; Pregnancy ; Vocal Cord Paralysis

OBJECTIVES: The clinical features of various facial deformities in Korean leprosy patients were evaluated according to the type of leprosy. METHODS: One hundred ninety six patients with leprosy were examined for various facial deformities using a nasal speculum, endoscope, and digital camera. The frequency and severity of external nasal deformities and septal perforations were evaluated according to the type of leprosy. Eye deformities, ear deformities, and facial palsy were also assessed. RESULTS: Seventy-one patients (36.2%) displayed external nasal deformities: 28 minimal contractures, three cartilage contractures, two bony-cartilage contractures, and 38 skin defects. The external nasal deformity and severe form deformity in lepromatous types were more frequent compared to other types (P<0.05 for each variable). Twenty-three patients (9%) displayed septal perforations, among whom 11 had cartilaginous perforations and 12 had bony-cartilaginous perforations. The frequency of septal and bony-cartilaginous perforations did not differ significantly between the types of leprosy (P>0.05 for each variable). Sixty-one patients (31.1%) had eye deformities and 19 patients (9.7%) had facial nerve palsy, common in the borderline type. No cases of ear deformities were observed. CONCLUSION: Korean patients had characteristic deformities according to the type of leprosy. They were different from those seen in the prior analyses of Caucasian populations.
Cartilage ; Congenital Abnormalities ; Contracture ; Ear ; Endoscopes ; Eye ; Facial Nerve ; Facial Paralysis ; Humans ; Leprosy ; Paralysis ; Skin ; Surgical Instruments

Facial paralysis is a devastating problem, since such a patient can neither make facial expressions nor enjoy an active social relationship. If it persists for a long time, facial muscles will have irreversible changes and deformities. The goal in the treatment of facial paralysis is to achieve a normal appearance at rest and reconstruct a natural, symmetrical smile. One of the most distressing figures in a facial palsy patient is oral deformity. There have been numerous corrective methods of reanimating the paralyzed lips. Recently the one-stage muscle transplantation, innervated by the contralateral facial nerve, has been introduced to solve this distressing problem. From March 1998 to December 1998, 3 patients(2 males and 1 female), who ranged in age from 17 to 33 years, underwent one-stage reanimation surgery of paralyzed lips using rectus abdominis muscle neurovascular free flap. All patients had unilateral facial paralysis caused by tumor resection. The rectus abdominis muscle with long intercostal nerve and pedicle vessels(deep inferior epigastric vessels) was elevated and transferred to the preoperatively designed subcutaneous pocket. The upper end of the muscle was fixed to the zygoma and parotid fascia, and the lower end was fixed to the mouth corner, upper and lower lip. The intercostal nerve was repaired to the non-paralyzed buccal branch of the facial nerve through the subcutaneous tunnel of the upper lip, and the vascular pedicle of the muscle flap was anastomosed to the paralyzed side of the facial vessels. The onset of muscle function was reported in all the patients. Five months after the surgery, nerve conduction study and EMG showed compound motor action potential(CMAP) and motor unit action potential(MUAP) of the transferred muscle respectively. Nine months after the surgery, we observed volitional muscle contraction. The power of contraction increased constantly for 2 years after the surgery.
Congenital Abnormalities ; Facial Expression ; Facial Muscles ; Facial Nerve ; Facial Paralysis ; Fascia ; Free Tissue Flaps* ; Humans ; Intercostal Nerves ; Lip* ; Male ; Mouth ; Muscle Contraction ; Neural Conduction ; Rectus Abdominis* ; Zygoma

BACKGROUND: Damage of the facial nerve, especially buccal and marginal mandibular branches, causes facial palsy and paralytic deformity of the lips such as lower lip ectropion, lip drooping, deviation of the mouth, inability of closing mouth, and the loss of the nasolabial folds. OBJECTIVE: To find out the best surgical method to correct paralytic deformity of the lips. METHODS: We reviewed retrospectively the various surgical treatment modalities of the paralytic deformity of the lips performed in The Institute for Leprosy Research for 13 years. RESULTS: One hundred and twenty five surgical procedures were performed in 80 patients with Hansen's disease. Of these, the static method, dermal suspension flap, was done in 15 cases, the dynamic methods were done in 64 cases, and others were done in 46 cases. All patients showed postoperatively clinical improvement above the average. Two patients received reoperation or additional procedures due to relapse of the condition. CONCLUSIONS: The dynamic method using tensor fascia lata was superior to other treatment modalities.
Congenital Abnormalities ; Ectropion ; Facial Nerve ; Facial Paralysis ; Fascia Lata ; Humans ; Leprosy ; Lip ; Mouth ; Recurrence ; Reoperation ; Retrospective Studies

There is no method of single surgical treatment for facial paralysis, because of its variable condition and variable patient's demand. We usually have been performed the free vascularized gracilis muscle transfer to achieve facial reanimation in cases of the complete facial paralysis. However. most of the patients who visit outpatient room are incomplete facial paralysis. In these cases, we performed the several auxiliary operations for the correction of a drooping eyebrow, paralyzed eyelids, paralysis of the lower lip, and for similar paralytic deformities. To correct a drooping eyebrow, we performed simple eyebrow lifting and upper blepharoplasty. Gold plate implantations and Kuhnt-Szymanowski's method were performed for the lagophthalmos and ectropions individually. To correct a paralyzed lower lip, tensor fascia lata graft was performed with the face lifting. The authors also discuss their operative methods and results of various surgical methods for treating facial paralysis.
Blepharoplasty ; Congenital Abnormalities ; Ectropion ; Eyebrows ; Eyelids ; Facial Paralysis* ; Fascia Lata ; Humans ; Lifting ; Lip ; Outpatients ; Paralysis ; Rhytidoplasty ; Transplants

Asymmetric crying facies are caused by unilateral weakness of the depressor muscles of lower lip, which is most obvious during crying and is associated with other congenital anomalies, especially congenital heart disease. This sign results from partial paralysis of facial nerve, specifically the ramus marginalis mandibulae, or congenital agenesis of the depressor anguli oris muscle (DAOM). We report two patients with congenital hypoplasia or absence of DAOM proved by electromyography, who have not cardiac anomalies.
Crying* ; Electromyography ; Facial Nerve ; Facies* ; Heart Defects, Congenital ; Humans ; Lip ; Muscles ; Paralysis

Paralytic lagophthalmos and ectropion by leprosy are serious complications of facial paralysis, which may lead to exposure keratitis, corneal ulceration, and further lead to blindness. In 1995 and 1997, we reported in this journal on the surgical treatment of 38 patients and 98 patients suffering with paralytic lagophthalmos and ectropion. In the first report of 1995, for lid closing I(Ahn) performed the method of traditional surgery such as temporal muscle transfer, medial and lateral canthoplasty as well as gold implant. In the 2nd report of 1997, we(Ahn and Park) presented the results of our combination treatment that changed the design and weight of the gold plate inserted in upper lid, and the medial canthoplasty and horizontal shortening in lower lid. Combination treatment provided for near normal eye closure and aesthetically pleasing appearance without the drawbacks associated with other methods such as eye clinching in concert with mouth closure, donor site deformities resulting from temporalis muscle transfer, and over exposure of carbuncle due to stretching effects of lateral canthoplasty. We have now found that raising the level of the lower lid margin to the sclera is important in concealing the scleral show due to drooping of the lower lid. We grafted conchal cartilage in a 5 x 35 mm sized band, which was fixed at the medial and lateral canthal area in 57 patients during the recent 3 years. We also added the ancillary procedure of the horizontal shortening in cases of highly atonic lower lid. We have noted that gold implantation in the upper eyelid and cartilage graft in the lower eyelid, with optional horizontal shortening, successfully corrected the lagophthalmos and ectropion due to facial nerve palsy.
Blindness ; Carbuncle ; Cartilage* ; Congenital Abnormalities ; Corneal Ulcer ; Ectropion* ; Eyelids ; Facial Nerve ; Facial Paralysis ; Humans ; Keratitis ; Leprosy ; Mouth ; Paralysis ; Sclera ; Temporal Muscle ; Tissue Donors ; Transplants