Congenital Macrostomia Research Articles

Surgical repair for congenital macrostomia using three small triangular flaps: Long-term follow-up and outcomes

Surgical repair for congenital macrostomia using three small triangular flaps: Long-term follow-up and outcomes

Correction of congenital macrostomia by double reversing Z-plasty: A technical note

Congenital macrostomia is a rare congenital deformity that consists of an enlargement of the commissure of the mouth. The malformation may be unilateral or bilateral and has a polymorphic presentation. Various surgical techniques have been described to correct macrostomia, with only a few cases illustrating the expected results. The surgical repair must consider both esthetic as well as functional impacts for the patient. We here propose a technical note to refine and provide additional information for good achievement of “Double Reversing Z-Plasty” for correction of macrostomia. Our case series also reports good long-term functional and esthetic results obtained with this technique, especially in case of a minor cleft.

Surgical Correction of Congenital Macrostomia with Double Z-Plasty technique: A Case Report

Abstract: Introduction: Congenital macrostomia is a relatively rare congenital facial anomaly that can occur either alone or in association with other facial anomalies and present either unilaterally or bilaterally. Purpose: To report the management of macrostomia with good functional and aesthetic results. Case report: A 3-months-old baby girl with widening at right corner of the lip since birth. Case management: Surgical correction was done with double Z-plasty to correct the macrostomia. Conclusion: Surgical correction of macrostomia in infant patient should be done promptly in young age to provide good psychological impacts on both child and family. Macrostomia reconstruction with double Z-plasty technique is the simplest one and easy to yields good results in both functional and aesthetics aspects. In the present case, there was minimal scar and no lateral deviation of the commissure after 2 weeks follow up.

Congenital macrostomia management in children in a country with limited resources: A case series.

• Congenital macrostomia is an uncommon facial malformation in children. • The choice of the surgical procedure should be based on functional, aesthetic results and the surgeon's experience. • Even in underdeveloped countries, Z-commissuroplasty could be performed with good results. • Further research should focus on long-term follow-up of large series, regardless of the type of surgical procedure.

Surgical Correction of Congenital Macrostomia in Cattle Calf

Surgical Correction of Congenital Macrostomia in Cattle Calf

Surgical correction of unilateral and bilateral congenital macrostomia: a case report and literature review

A Revista Brasileira de Cirurgia Plástica é o órgão oficial de divulgação da Sociedade Brasileira de Cirurgia Plástica (SBCP), tem como objetivo registrar a produção científica em Cirurgia Plástica, fomentar o estudo, aperfeiçoamento e atualização dos profissionais da especialidade.

Bilateral transverse facial clefts (congenital macrostomia) - A case report

We describe a very rare case of bilateral congenital macrostomia in a three month old female infant with an unusual association of brachycephaly and other dysmorphic features as a result of ingestion of an alcohol based herbal preparation, Sulphadoxine-Pyrimethamine and probably supplemental doses of Vitamin E in the early first trimester of pregnancy. This case report emphasizes the need for maternal education and support of maternal health care.

Asyndromic Bilateral Transverse Facial Cleft

Bilateral transverse facial cleft, Tessier no 7 also known as bilateral congenital macrostomia are very rare clefts. We present an eight months old female with bilateral transverse facial cleft, third child of three siblings in a monogamous setting born to parents of Delta origin who resides in effurun Delta state of Nigeria. Mother ingested postinor several times in an attempt to abort pregnancy. Pregnant women should be encouraged to have adequate folic acid diet or supplements.

Transverse facial cleft: A series of 17 cases.

Introduction:Transverse facial cleft (Tessier type 7) or congenital macrostomia is a rare congenital anomaly seldom occurring alone and is frequently associated with deformities of the structures developing from the first and second branchial arches. The reported incidence of No. 7 cleft varies from 1 in 60,000 to 1 in 300,000 live births.Material and Methods:Seventeen patients of transeverse facial cleft who presented to us in last 5 years were included in the study. Their history regarding familial and environmental predispositions was recorded. The cases were analysed on basis of sex, laterality, severity, associated anomalies and were graded according to severity. They were operated by z plasty technique and were followed up for 2 years to look for effectiveness of the technique and its complications.Result:Out of the seventeen patients of transverse cleft, none had familial predilection or any environmental etiology like antenatal radiological exposure or intake of drugs of teratogenic potential. Most of the patients (9/17) were associated with hemifacial microsomia and 1 patient was associated with Treacher Colin's Syndrome. Out of the 6 cases of Grade I clefts, 4 were isolated transverse clefts and of the 10 patients of Grade II clefts, 7 were associated with hemifacial microsomia. We encountered only one case of Grade III Transverse Cleft which was not only associated with hemifacial microsomia but also had cardiac anomaly. Out of the 17 cases, 15 were operated and in most of them the outcome was satisfactory.

Refined simple line closure for macrostomia repair: Designing a mucosal triangular flap on the commissure region

Congenital macrostomia is a relatively rare deformity. A number of different methods for its correction have been reported in the past. Here, we report our refined method of correcting macrostomia. Our method is characterized by creation of a small triangular mucosal flap on the cleft region of the lower red lip where the tissue appears identical to the normal commissure. Adequate advancement of this flap into the mouth along with proper reconstruction of muscle continuity and simple line closure of the skin enable correction of macrostomia with a natural-looking commissure in a symmetric position with an acceptable scar.

A susceptibility locus on 1p32–1p34 for congenital macrostomia in a Chinese family and identification of a novel PTCH2 mutation

Macrostomia, (horizontal facial cleft, lateral facial cleft, transversefacialcleft)isararecongenitalmalformation.Onoccasion,itcanbea sign in certain syndromes, such as Goldenhar syndrome orTreacher–Collin syndrome [McCarthy and West, 1977]. Rarely,it occurs as an isolated malformation. The cause has remainedunknown.We studied autosomal dominantly inherited macrosomia in alarge family of Chinese Han origin (Fig. 1A and Table I). Linkageandhaplotypeanalysisidentifiedthemacrostomia-associatedlocusbetween markers D1S193 and D1S2652 on 1p32–1p34; the maxi-mum LOD score at D1S2797 was 4.18 (q¼0.00, Table II). At thislocus, we identified a heterozygous mutation in exon 11(NC_000001.9) of PTCH2 (1423G!A, resulting in Val471Ile inthe 4th transmembrane domain) (NP_003729) in all patients withmacrosomia (Fig. 1B). No mutation was found in family memberswholackedmacrosomiaandwefoundnoPTCH2mutationsin520unrelated controls.Although over-expression of SMO induced GLI-dependentluciferase activity, co-expression of wild-type PTCH2 inhibitedSMO-induced activity significantly, confirming the inhibitoryfunction of PTCH2 in the SHH signaling network (Fig. 1C). Incontrast, co-expression of PTCH2 Val471Ile did not inhibit SMO-inducedluciferaseactivity.Over-expressionofPTCH2significantlyinhibited cell proliferation, but over-expression of PTCH2 Va-l471Ile did not appear to have a major effect on the cell growth rate(Fig. 1D). Western blot analysis confirmed that the expression levelof PTCH2 and PTCH2 Val471Ile is similar (Fig. 1E).The hedgehog signaling network has been reviewed extensivelyelsewhere [Cohen, 2003]. PTCH2 encodes a 1204-amino acidtransmembrane protein with about a 54% overall identity toPTCH1 [Motoyama et al., 1998; Smyth et al., 1999] and a 90%identity to Ptch2 in mice [Carpenter et al., 1998]. In the absence ofSonic Hedgehog (SHH) stimulation, PTCH2, like PTCH1, canmaintain the hedgehog signaling pathway in an inactive state byinhibiting SMO. When SHH binds to PTCH2, the inhibitory effectof PTCH2 on SMO is removed, resulting in activation of SMO,downstreamsignaling,andupregulationoftargetgenes,suchastheGLI family proteins [Hahn et al., 1996; Young et al., 2000; Chenetal., 2002;Vokesand McMahon, 2004;Hutchinetal.,2005]. SHH

Vermilion Square Flap for Correction of Bilateral Macrostomia-A Case Report

Congenital bilateral macrostomia, a transverse facial cleft, is a rare deformity of the mouth; when it occurs, it is usually unilateral. Many procedures have been developed for correction of macrostomia and most of them use the noncleft side to mark the reconstructed commissural position. In cases of bilateral forms, the surgeon must define the commissural position without a normal side as a reference. Most of the reported procedures place the scars at the mouth angle or at the lower lip, which result in an unnatural appearance. We report a successful correction for a 4-month-old baby girl with bilateral macrostomia using vermilion square flap technique. The scar is placed at the upper lip. At one month follow-up, the oral commissures are symmetric, the scars are inconspicuous, and the overall balance of facial contour and lip is excellent.

An Isolated Bilateral Pure Macrostomia in a 2-Year-Old Girl

Congenital macrostomia is a lateral orofacial cleft between the maxillary and mandibular components of the first branchial arch. Bilateral macrostomia is a rare entity. To date, 17 cases have been reported in the literature, with more than 50% isolated. We present a case of bilateral transverse facial cleft (macrostomia) in a 2-year-old girl. It was not associated with any other anomaly. The cleft involved only the soft tissues of the face. Hence, it was a pure macrostomia. She was the third female child of the family, with 2 elder sisters normal. Nutritional deficiency during intrauterine life and/or advanced maternal age can be causative factors. Transverse facial cleft was closed by Z-plasty incision, with good functional and aesthetic result.

Myoplasty for Congenital Macrostomia

To describe the different myoplasty techniques that could be used for limited commissural reconstruction. Twelve cases of congenital macrostomia are reported, with different cleft lengths and termination sites. For each case, an orbicular myoplasty was performed, and in the case of extension to the area of the tragus or tonsillar pillars, a masseteric myoplasty or pharyngoplasty was performed. Functional and aesthetic results were analyzed. Functional results were excellent, with normal phonation, facial expression, and deglutition in the case of posterior extension. Aesthetic results were good, with only two cases of skin fasciculation during facial movement. Myoplasty in macrostomia could be limited to an orbicular reorientation in the case of a short cleft or can include a masseteric myoplasty or pharnygoplasty should the cleft extend further. Analyzing 90 reported cases of congenital macrostomia in the world literature, an important point has emerged. In some cases, the cleft could continue sagittally to the tonsillar pillars or laterally, distal to the anterior border of the masseter, to the region of the tragus. Repair in these cases requires reconstruction of the tonsillar pillars and masseteric repair in addition to orbicular removal. No reports in the world literature have referred to these other myoplasties that could be necessary, even if such pathology is very rare. In addition, no classification of congenital macrostomia was found in the world literature. We therefore propose a surgical classification of macrostomia relative to the nature of myoplasty required.

Emergency Airway Management using a Laryngeal Mask Airway (LMA) Following Extubation in an Infant with a Congenital Facial Anomaly - A case report -

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.

Macrostomie congénitale bilatérale

Congenital macrostomia, a transverse facial cleft, is a rare deformity of the mouth, which can occur alone or in association with other deformities. We report a case of congenital bilateral macrostomia. We discuss the difficulties of plastic surgery in this pathology. Congenital macrostomia in a one-year-old girl compromised feeding. The patient presented an isolated bilateral transverse facial cleft. Surgery associated a suture of the orbicularis oris muscle and a cutaneous W plasty. At twelve months, follow-up has been uneventful. Congenital macrostomia is most commonly associated with others anomalies. Many procedures are described regarding surgical correction of macrostomia. Reconstruction of the integrity of the oral sphincter associated with W plasty usually gives the best results.

Bilateral Macrostomia as an Isolated Pathology

Congenital macrostomia is a lateral orofacial cleft between the maxillary and mandibular components of the first branchial arch. Bilateral macrostomia is a poorly characterized malformation, with only 14 cases reported in the literature. The purpose of this study was to compare our experience with the world literature. A retrospective analysis of 20 cases of bilateral congenital macrostomia was conducted; 6 cases were drawn from 2 maxillofacial surgery units and 14 cases from the world literature. Cases of bilateral congenital macrostomia were compared with cases of unilateral forms using a review of the literature post-1954. Among the six cases identified from the two maxillofacial surgery units, three were treated with linear sutures and three with Z-plasty. Subsequent aesthetic and functional results were analyzed. Compared to unilateral forms, bilateral macrostomia is more often isolated without ear or skin deformities. Moreover, there are a greater proportion of larger defects among cases with bilateral macrostomia when compared to unilateral macrostomia. Alimentation, phonation, and mouth opening were always normal. The two sides were always symmetric. Only one case presented with the complication of skin contractions during lip movement. The etiopathogenesis of bilateral macrostomia is unclear. Although over 50% of the reported cases of bilateral macrostomia are isolated, this condition presents a therapeutic challenge. In the case of bilateral forms, the surgeon must define the commissure position without a normal side. Repair thus requires extraoral landmarks and normal measurements.

Étude épidémiologique et clinique des macrostomies. À propos d'une série de dix observations

Introduction. – Macrostomia or lateral cleft or commissural cleft or transverse cleft is a rare facial cleft in comparaison with standard cleft lip and palate. Method. – A retrospective study of 10 cases of congenital macrostomia is realised. For each case a retrospective study with epidemiologic and clinic features was realised. Results and Discussion. – From these clinical considerations, a new classification with direct surgical consequences is proposed.

Straight line closure of congenital macrostomia

ABSTRACTThe results of patients operated on by Nepal Cleft Lip and Palate Association (NECLAPA) surgeons for congenital macrostomia were prospectively studied between January 2000 and December 2002. There were four males and three females with a median age of 10 years. Three had an associated branchial arch syndrome. In all patients an overlapping repair of orbicularis oris was done. Six patients had a straight line closure with excellent cosmetic results and one a Z-plasty with a more obvious scar. All had a normal appearing commissure. Overlapping orbicularis repair with straight line skin closure for this rare congenital anomaly is recommended.

Bilateral macrostomia as an isolated deformity and its repair with a modified technique

A 3-year-old boy with bilateral congenital macrostomia as an isolated clinical entity is presented. Although this is a rare deformity, it causes functional and cosmetic problems which are difficult to correct. The skin, muscle, and mucosal components of the deformity were separately repaired using a modification of Kaplan's technique.