OBJECTIVE: To report a case series of Tessier 3, 4, 7 and combined 4,7 craniofacial clefts, their clinical presentations, surgical approaches and outcomes in light of the current literature.
METHODS:
Design: Case series
Setting: Tertiary Government Hospital
Subjects: Five patients
RESULTS: Five  patients aged 3 to 14-years-old with Tessier 3, 4 (2  cases), 7  and combined 4,7 were included in this study: Tessier 3 - medial orbitomaxillary cleft extending through the bony skeleton traversing obliquely across the lacrimal groove, Tessier 4 - median orbitomaxillary cleft traversing  vertically  through  the  inferior  eyelid,  infraorbital rim and  orbital  floor extending  to the lip between the philtral crest and the oral commissure  (2 cases), Tessier 7 - macrostomia and cleft oral commissure and combined Tessier 4 and 7, combining features described above. Four underwent 2- or 3-stage surgeries while one declined.
CONCLUSION: Five  craniofacial  clefts  were  presented.  Because  of  the  varying  patterns of craniofacial  deformities,  a  series of surgical  procedures,  tailor-made  for  each  individual  were performed  on  four. Otolaryngologists who perform maxillofacial and cosmetic surgery  should have good background knowledge about craniofacial defects and be familiar with the  surgical approaches at their disposal to yield favorable results that are appropriate to their local contexts.

Human ; Male ; Adolescent ; Child ; Child Preschool ; General Surgery ; Macrostomia ; Surgery, Plastic ; Lip ; Otolaryngologists ; Craniofacial Abnormalities ; Eyelids ; Orbit

Treacher Collins syndrome (TCS) is the most common and well known mandibulofacial dysostosis with characteristic clinical features including downward slanting of palpebral fissures, coloboma of the lower eyelid, hypoplastic zygomatic arches, micrognathia, macrostomia, microtia, and other deformities of the ears. TCS is caused by mutations in at least 3 genes involved in pre-rRNA transcription: TCOF1, POLR1D and POLR1C. We experienced a 1-day-old female infant with characteristic clinical features of TCS. A novel, heterozygotic mutation within the TCOF1 gene (c.3874_3875insG, p.Ala1292Glyfs*30) was identified to cause a premature stop codon.
Codon, Nonsense ; Coloboma ; Congenital Abnormalities ; Ear ; Exons* ; Eyelids ; Female ; Humans ; Infant ; Infant, Newborn* ; Macrostomia ; Mandibulofacial Dysostosis* ; RNA Precursors ; Zygoma

PURPOSE: A Tessier classification number 7 cleft is an uncommon malformation that results from a failure of mesenchymal fusion within the maxillary and mandibular prominences of the 1st pharyngeal arch. Many operative techniques of the number 7 cleft repair have been proposed to restore function and improve aesthetics. Fifteen patients underwent repair of a number 7 cleft over 13 years by a modification of the surgical Technique, and an appraisal of the operative outcome is reported herein. METHODS: A retrospective review was conducted involving 15 patients with number 7 clefts who underwent surgery from 1996 to 2009. The changes in surgical technique included skin closure, attachment of the orbicularis oris muscle, and position of the repaired commissure; the changes were analysed with a review of the medical records and the outcomes of surgery were analysed via photographs. Specifically, the technique of skin closure was changed from the a Z-plasty to a linear closure, the orbicularis oris muscle overlapped attachment was replaced by a side-to-side approximation with horizontal mattress sutures, and the position of the repaired commissure was changed from 1mm laterally to 1mm medially in reference to the non-cleft side. RESULTS: A Z-plasty caused additional cutaneous scarring, an overlapped attachment of the orbicularis oris muscle caused a thick oral commissure, and the repaired commissure migrated to the lateral side, so a 1mm, laterally-positioned commissure caused asymmetry. The altered procedure included a linear skin closure, a side-to-side orbicularis oris muscle approximation, and a 1mm, medially-positioned commissure, which together resulted in a good outcome. CONCLUSION: The altered procedure for repair of a number 7 cleft as described herein, yields a short scar, no functional problems with the orbicularis oris muscle, a thin oral commissure, and symmetry of the repaired commissure.
Branchial Region ; Cicatrix ; Esthetics ; Humans ; Macrostomia ; Medical Records ; Muscles ; Retrospective Studies ; Skin ; Sutures

A female infant (4 months-old) with Goldenhar syndrome was scheduled for cheiloplasty to treat a transverse facial cleft and congenital macrostomia. There was no past history of difficulty during feeding or airway obstruction. Following induction of anesthesia using an inhalational anesthetic technique, conventional oro-tracheal intubation was possible. However, following extubation of the endotracheal tube she developed an upper airway obstruction. Her lungs could not be ventilated using a facial mask and oxygen saturation was decreased. A #1 laryngeal mask airway (LMA) was inserted immediately, which allowed us to ventilate her lungs and restore the oxygen saturation. Here we describe the use of a LMA for emergency airway management in an infant.
Airway Management ; Airway Obstruction ; Anesthesia ; Emergencies ; Female ; Goldenhar Syndrome ; Humans ; Infant ; Intubation ; Laryngeal Masks ; Lung ; Macrostomia ; Masks ; Oxygen

OBJECTIVE: To explore the role of vrk1 gene in two Chinese pedigrees of the first and second branchial arch syndrome. METHOD: Sixty members in 2 Chinese pedigrees were recruited. The exon 2 -13 were analyzed by polymerase chain reaction and direct sequencing. RESULT: We found a new SNP in proband of Shandong pedigree. CONCLUSION vrk1 gene mutation can be excluded in 2 Chinese pedigrees of the first and second branchial arch syndrome.
Asian Continental Ancestry Group ; genetics ; Branchial Region ; Exons ; Female ; Humans ; Intracellular Signaling Peptides and Proteins ; genetics ; Macrostomia ; genetics ; Male ; Mutation ; Pedigree ; Protein-Serine-Threonine Kinases ; genetics ; Syndrome

Macrostomia, or lateral facial cleft, is a rare congenital deformity that occurs alone or in combination with other anomalies as a result of a failure of proper fusion of the maxillary and mandibular processes during the fourth week of embryonic life. Although many surgical techniques have been suggested to correct this deformity, the procedures are controversial and no gold standard has yet been established. The purpose of these procedures includes formation of symmetric commissure, restoration of the abnormally positioned orbicularis oris muscles, and an inconspicuous scar. A natural-looking oral commissure is not easily achieved because the commissure is not just a corner but a continuous vermilion web. The purpose of this study is to achieve a more natural-looking commissure using natural anatomical landmarks different from other studies and to divide three anatomical units of the commissure for the proportional surgical repair of congenital macrostomia. Surgical methods include commissuroplasty using mucosal triangular flap, myoplasty of the disarranged orbicularis oris muscle and closure of the cheek cleft with a small Z-plasty. From March, 1999 to Feb, 2003, a total of 12 patients(4 males, 8 females) with congenital macrostomia have been operated. We obtained satisfactory functional and esthetic outcomes in all patients by using these techniques after a long period follow-up. We think the most important aspect of macrostomia repair is a proportional anatomical repair using natural landmarks.
Cheek ; Cicatrix ; Congenital Abnormalities ; Follow-Up Studies ; Humans ; Macrostomia* ; Male ; Muscles

Macrostomia, also called a transverse or lateral facial cleft, is a relatively rare malformation. Although many surgical procedures have been introduced, and no gold standard has yet been established. Moreover, most papers published in Korea were based on the results of the research conducted on the very limited number of patients, and for this reason its findings do not offer sufficient clinical reliability. We devised a modified commissuroplasty as follows: First, new commissure was placed 1 or 2mm inside when compared with the opposite side so that the commissure may not look longer than usual because of the scar on the side. Second, z-plasty of about 5mm was performed on the nasolabial fold to prevent the displacement of the new commissure on its lower part and avoid a continuation of a scar with the medial flap placed upward. We treated 32 cases of macrostomia from August 1, 1998 to July 1, 2002. We obtained relatively satisfactory clinical results by using this modified commissuroplasty. Based on our experience, we intend to present a clinical analysis and an operation technique of our own derived from the classic commissuroplasty, so that we may contribute to the diagnosis and treatment of the patients in the future.
Cicatrix ; Diagnosis ; Humans ; Korea ; Macrostomia* ; Nasolabial Fold

Congenital macrostomia is a result of defective union between the mandibular and maxillary processes and it is a rare deformity seen in every 100 to 300 facial clefts. Ohnizuka1`classified macrostomia into two groups as congenital and posttraumatic. We experienced two cases of acquired macrostomia due to NOMA sequelae(58/F:Lt & 51/F:Rt) and one case of congenital macrostomia (3 months/M:Rt). Many plastic surgeons have developed surgical procedures for repair of this congenital macrostomia. Among them, McCarthy6,11 described the classic commissuroplasty. We could repaired 1 case of congenital macrostomia and two cases of acquired macrostomia due to NOMA sequelae using modified technique of McCarthy,s classic commissuroplasty. McCarthy described new oral commissure 2-3mm laterally for prevention of postoperative contraction, orbicularis oris muscle transposition to restore labial function and a z- plasty cutaneous closure. But some author raise an objection to new oral commissure 2-3mm laterally, and they made new oral commissure at same distance of opposite side normal commissure. And so, we designed the new oral commissure moved 1mm laterally comparing to original commissuroplasty in a congenital case for the prevention of displacement. In cases of acquired macrostomia due to NOMA sequelae, we reconstructed new oral commissure like congenital case, moved 1mm laterally. Orbicularis oris muscle transposition could not be possible because of destruction of muscle, adhesion and atrophy. And so we dissected muscle and just sutured side by side. Acquired macrostomia following NOMA sequelae manifsted facial deformity variably, and reconstruction of the facial deformity is difficult by using simple approach. Other variable reconstructive procedures were needed with commissuroplasty as like Washio flap, rotation advancement flap, bone graft and free radial forarm flap, etc. Postoperative results were relatively good. We propose that macrostomia due to NOMA sequelae must add to Ohnizuka classification of acquired macrostomia.
Atrophy ; Classification ; Congenital Abnormalities ; Macrostomia* ; Noma ; Transplants

The goals of the reduction mammoplasty are to reduce the volume of the breast, to create aesthetic shape that is stable over time, to maintain blood supply and innervation to the nipple-areolar complex, and to make fine limited scars. There are 3 rationales in our reduction mammoplasty. To reduce the scar, we perform the periareolar incision. To make effective reduction of the breast volume, and to preserve blood supply and innervation to the nipple-areolar complex, we use a central or an inferior pedicle technique. To prevent areolar widening, we use a purse-string suture. We performed the periareolar reduction mammoplasty to 36 breasts in 18 patients from Jul. 1998 to Jun. 2000. The mean follow up period was 8 months. The mean age was 41 and mean resection amount was 420 gm per breast. Most patients satisfied with their fine periareolar scars, adequate size of breasts and the innervation of the nipple-areolar complex. We applied this procedure to all kinds of macrostomia. The greatest advantage of the periareolar reduction mammoplasty is the inconspicuous limited scar. Other advantages over conventional technique include preservation of sensitivity to the nipple-areolar complex and shorter operative time. As disadvantages, 10 breasts(28%) showed areolar widening. In 8 of 10 breasts with areolar widening, purse-string suture was not applied in the skin flap margin of the outer circle and reoperation was executed to reduce the areolar size by excision of the widened areola. The application of the purse-string suture was carried out in 6 breasts. Two breasts with purse-string suture showed areolar widening possibly due to loosening of the purse-string suture knot. There were persistent periareolar wrinkles in 4 breasts and poor sensitivity to the nipple-areolar complex in 6 breasts in which more than 500 gm of breast tissue per breast was resected. Periareolar reduction mammoplasty is optimal for patients who require reduction of lesser than 500 grams per breast. In the severe macromastia with or without ptosis, inverted T-incision is preferable to the periareolar incision, and periareolar incision can be modified by adding wedge resection of the outer excess skin flap inferiorly which results in a periareolar and vertical scar below the nipple-areolar complex.
Breast ; Cicatrix ; Female ; Follow-Up Studies ; Humans ; Macrostomia ; Mammaplasty* ; Operative Time ; Reoperation ; Skin ; Sutures

Macrostomia is a relatively rare malformation. There have been many surgical methods for the correction of macrostomia. The old method, a simple straight line closure , did not include reconstruction of the orbicularis oris muscle. Postoperatively, a depressed scar and severe contraction on the cheek were found, especially with animation. As a result, many authors have known that reconstruction of the orbicularis oris muscle was very important to avoid a depressed scar for the correction of macrostomia. The tendency toward lateral displacement of the reconstructed commissure has been attributed to linear scar contraction, and Z-plasty is advocated to prevent this deformity. However, we found that the Z-plasty scar may be conspicuous when the patient smiles. So we performed reconstruction of the orbicularis oris muscle to avoid a depressed scar, and straight line closure to avoid a conspicuous scar. In order to prevent linear scar contracture due to straight line closure, we overcorrected the new commissure. We treated 6 cases of macrostomia form May 1, 1996 to April 30, 1999 using straight line closure and reconstruction of the orbicularis oris muscle. Periods of follow-up were from 12 months to 35 months, with an average of 20,2 months. Every patient was analyzed clinically by ordinary scale method and anthropometrically by the ratio of abnormal distances to normal distances between cheilion and crista philtri. In 3 clinical assessments : symmetry of the commissure was excellent: degree of the scar at rest was good: and degree of depression with animation was excellent. The anthropometrical ratio was 1:1.05. Despite the fact that the linear scars were perpendicular to the minimal skin tension lines, the scars were inconspicuous. The muscle repair provided reconstruction to the modiolus and gave a natural appearance to the commissure, however it did not seem to provide sufficient bulkiness around the commissure. The straight line skin closure and repair of the orbicularis oris muscle provide effective functional and aesthetic reconstruction.
Cheek ; Cicatrix ; Congenital Abnormalities ; Contracture ; Depression ; Follow-Up Studies ; Humans ; Macrostomia* ; Skin