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Journal of Stomatology, Oral and... Jun 2022Macrostomia or lateral cleft lip is a rare congenital deformity. In this article we describe a surgical technique of macrostomia repair developed. The objective of this...
BACKGROUND
Macrostomia or lateral cleft lip is a rare congenital deformity. In this article we describe a surgical technique of macrostomia repair developed. The objective of this article is to assess the results of our surgical technique and to validate a method for macrostomia surgical result evaluation.
METHODS
We included retrospectively patients with unilateral and bilateral macrostomia, operated from 1995 to 2014 in our department. First part of the study was a satisfaction questionnaire completed by patients. The second part was subjective evaluation of frontal photography (closed mouth, wide open and smiling) by surgeons and lay people with a questionnaire. Both group completed a second questionnaire within one to six months.
RESULTS
Eighteen patients answered the questionnaire. The satisfaction for all patients were considered as very good for 38.9% (n = 7) of patients and good for 44.4% (n = 8). 21 patients were photographed, 5 isolated macrostomia, 13 macrostomia with minor facial asymmetry and 3 with a major asymmetry. Surgeons evaluated the result as very good for isolated macrostomia and good for syndromic macrostomia. Layperson evaluated the result as good in isolated macrostomia and macrostomia with minor facial asymmetry and average with major facial asymmetry. P < 0.0001. The evolution of the results between medical and non-medical assessors in our two questionnaires, were non-significant.
CONCLUSION
In this study, we propose a new methodology to assess commissuroplasty surgical results, with a 3 type of evaluator: patients, surgeons and laypeople. We present a simple surgical technique, that allows good results in syndromic and isolated macrostomia.
Topics: Cleft Lip; Esthetics; Facial Asymmetry; Humans; Macrostomia; Retrospective Studies
PubMed: 34224921
DOI: 10.1016/j.jormas.2021.06.018 -
The Journal of Craniofacial Surgery Sep 2018Macrostomia (Tessier's 7 cleft) is a rare congenital lip deformity. Macrostomia can occur unilateral or bilateral, isolated or associated with other syndromes. Isolated...
Macrostomia (Tessier's 7 cleft) is a rare congenital lip deformity. Macrostomia can occur unilateral or bilateral, isolated or associated with other syndromes. Isolated bilateral macrostomia is exceedingly rare with only a few cases reported to date. The authors report 6 cases of isolated bilateral macrostomia surgically repaired in 4-layered approaches. The traditional method was improved and the result obtained was satisfactory after longest follow-up of 3 years. The technique is easy to imitate, simple in design, aesthetically and functionally corrects the deformity.
Topics: Child, Preschool; Female; Humans; Infant; Macrostomia; Male; Mouth Mucosa; Oral Surgical Procedures; Plastic Surgery Procedures; Surgical Flaps
PubMed: 29742578
DOI: 10.1097/SCS.0000000000004621 -
American Journal of Medical Genetics.... Aug 2016Barber-Say syndrome (BSS) and Ablepharon-Macrostomia syndrome (AMS) are congenital malformation syndromes caused by heterozygous mutations in TWIST2. Here we provide a... (Meta-Analysis)
Meta-Analysis Review
Barber-Say syndrome (BSS) and Ablepharon-Macrostomia syndrome (AMS) are congenital malformation syndromes caused by heterozygous mutations in TWIST2. Here we provide a critical review of all patients published with these syndromes. We excluded several earlier reports due to misdiagnosis or insufficient data for reliable confirmation of the diagnosis. There remain 16 reliably diagnosed individuals with BSS and 16 with AMS. Major facial characteristics present in both entities, albeit often in differing frequencies, are excessive facial creases, hypertelorism, underdevelopment of the anterior part of the eyelids (anterior lamella), ectropion, broad nasal ridge and tip, thick and flaring alae nasi, protruding maxilla, wide mouth, thin upper vermillion, and attached ear lobes. In BSS a remarkable extension of the columella on the philtrum can be seen, and in both the medial parts of the cheeks bulge towards the corners of the mouth (cheek pads). Scalp hair is sparse in AMS only, but sparse eyebrows and eyelashes occur in both entities, and general hypertrichosis occurs in BSS. We compare these characteristics with those in Setleis syndrome which can also be caused by TWIST2 mutations. The resemblance between the three syndromes is considerable, and likely differences seem larger than they actually are due to insufficiently complete evaluation for all characteristics of the three entities in the past. It is likely that with time it can be concluded that BSS. AMS and Setleis syndrome form a continuum. © 2016 Wiley Periodicals, Inc.
Topics: Abnormalities, Multiple; Eye Abnormalities; Eyelid Diseases; Facies; Genetic Association Studies; Genotype; Hirsutism; Humans; Hypertelorism; Hypertrichosis; Macrostomia; Mutation; Phenotype; Skin Abnormalities; Twist-Related Protein 2
PubMed: 27196381
DOI: 10.1002/ajmg.a.37757 -
Clinics in Perinatology Dec 2018Infants may be born with external ear deformities or malformations that can present a diagnostic clue to an affiliated syndrome while also presenting the possibility of... (Review)
Review
Infants may be born with external ear deformities or malformations that can present a diagnostic clue to an affiliated syndrome while also presenting the possibility of surgical intervention. Microtia is a malformation of the ear that is associated with other craniofacial or systemic anomalies in 50% of cases. Surgical correction of microtia and associated facial anomalies is complex and must be integrated thoughtfully into the overall care plan for children with complex medical needs. Familiarity with types of ear and facial anomalies and their association with more global concerns can allow perinatal practitioners to better serve their patients.
Topics: Abnormalities, Multiple; Congenital Microtia; Craniofacial Abnormalities; Female; Follow-Up Studies; Goldenhar Syndrome; Humans; Infant, Newborn; Male; Quality of Life; Plastic Surgery Procedures; Risk Assessment; Treatment Outcome
PubMed: 30396412
DOI: 10.1016/j.clp.2018.07.007 -
Reviews in Endocrine & Metabolic... Sep 2016Male fertility can be affected by a variety of organs diseases, including the skin. Several genodermatoses affect the skin and several other organs including the male... (Review)
Review
Male fertility can be affected by a variety of organs diseases, including the skin. Several genodermatoses affect the skin and several other organs including the male reproductive system, commonly in the form of cryptorchidism and hypogonadism. The most relevant syndromes are associated with dyschromias, such as deSanctis-Cacchione, poikiloderma congenital, LEOPARD, and H syndrome; others with ichthyosis, such as Rud, and trichothiodystrophy; or a group of unrelated genodermatoses, such as ablepharon macrostomia, Coffin-Siris, Gorlin-Goltz, and Werner. Acquired skin diseases may also affect male fertility usually in the form of orchitis or epididymal obstruction or androgen antagonists. These include infections (leprosy and HIV), autoimmune (erythema nodosum leprosum), granulomatous (sarcoidosis, Langerhans cell histiocytosis), nutritional deficiency (zinc), and malignancy. Several therapeutics of skin diseases are notorious for their effects on male fertility, most notably are the cytotoxic drugs (methotrexate), irradiation, and antiandrogens (spironolactone, finasteride). Although the prevalence of these skin diseases is low, the associated male infertility represents a challenge due to the difficulty of its management. Clinical management of the skin diseases should include consideration of their effects not only on the diseases but also on the male reproductive system.
Topics: Humans; Infertility, Male; Male; Skin Diseases
PubMed: 27342409
DOI: 10.1007/s11154-016-9368-x -
Facial Plastic Surgery & Aesthetic... 2022Most of the characteristic facial features of patients with unilateral macrostomia are attributed to the malformation of commissure muscles. This study aimed to...
Most of the characteristic facial features of patients with unilateral macrostomia are attributed to the malformation of commissure muscles. This study aimed to evaluate a modified surgical treatment for such patients that focuses on both appearance and symmetry. Twenty-seven patients with macrostomia underwent surgery using the proposed method. Facial measurements were analyzed preoperatively, 1 week postoperatively, and during a long-term follow-up using statistical software. The overall length ratio of the healthy and affected sides of the vermillion preoperatively, 1 week postoperatively, and during the long-term follow-up was 1:1.61, 1:1.01, and 1:1.00, respectively (all, > 0.05). The overall angle between the pupil line and the commissure line was 9.90° preoperatively, 2.34° postoperatively, and 3.31° during the long-term follow-up. There was no statistically significant difference in the covering relation of the upper and lower lips between the affected and healthy sides postoperatively ( > 0.05). 3dMD Dynamic Surface Imaging System (3dMD, Atlanta, GA, USA) showed a symmetrical three-dimensional commissure structure during long-term follow-up measurements. The symmetry and appearance of patients with macrostomia commissure significantly improved following this modified surgical method.
Topics: Humans; Lip; Macrostomia; Muscles; Plastic Surgery Procedures; Surgical Flaps
PubMed: 35020489
DOI: 10.1089/fpsam.2021.0084 -
The Journal of Craniofacial Surgery May 2021Unilateral oral macrostomia can present in isolation or conjunction with other craniofacial abnormalities. Common associations include cartilaginous tags and accessory...
Unilateral oral macrostomia can present in isolation or conjunction with other craniofacial abnormalities. Common associations include cartilaginous tags and accessory tragi, while the facial nerve is rarely involved. Our work is the first of its kind to report depressor anguli oris paralysis in the setting of oral macrostomia. The authors present 2 cases of unilateral oral macrostomia, with and without contralateral pre-operative depressor anguli oris paralysis, to highlight this often overlooked finding. Furthermore, these cases illustrate the impact of depressor anguli oris paralysis on postoperative outcomes and patient expectations. Depressor anguli oris function can be detected preoperatively and therefore should be weaved into surgical decision-making and used to manage expectations for symmetric facial animation following repair. Further work is required to evaluate the long-term benefits of electromyography and botulinum toxin injections as diagnostic and therapeutic modalities for DAO paralysis.
Topics: Face; Facial Muscles; Facial Nerve; Facial Paralysis; Humans; Macrostomia
PubMed: 33229990
DOI: 10.1097/SCS.0000000000007255 -
The Journal of Craniofacial Surgery May 2021In principle, reconstruction in macrostomia requires symmetry and accurate positioning of the newly reconstructed commissure. The proper position of the new commissure...
In principle, reconstruction in macrostomia requires symmetry and accurate positioning of the newly reconstructed commissure. The proper position of the new commissure can be determined by several methods. In the determination of the new commissure of bilateral cases, generally the average length of the lips or the distance from anatomical landmarks other than the lips, such as the pupils or tragi, has been used. A new approach was reported by Tse et al in 2018: the anatomic approximation approach. Macrostomia repair refers to anatomical landmarks in the lips to determine the new commissure. The authors performed surgery using this anatomic approximation approach for bilateral macrostomia and achieved the good results.
Topics: Hair; Humans; Lip; Macrostomia; Pupil; Scalp
PubMed: 33252523
DOI: 10.1097/SCS.0000000000007273 -
International Journal of Surgery Case... Dec 2023Macrostomia is a congenital deformity found in Tessier no. 7 facial clefts defined as an enlargement of the mouth at the oral commissure. Several techniques are...
INTRODUCTION AND IMPORTANCE
Macrostomia is a congenital deformity found in Tessier no. 7 facial clefts defined as an enlargement of the mouth at the oral commissure. Several techniques are described in literature to achieve optimal functional and aesthetic results, with varying results and surgeon preferences. In this case series we report surgical repair of macrostomia with a vermillion square flap method for the oral commissure combined with either Z-plasty or W-plasty closure for the skin.
CASES PRESENTATION
A retrospective case analysis of 12 patients with macrostomia operated over the past 7 years at our plastic surgery division was performed (by two different operators; 11 cases by A.S. and 1 case by R.S.). Clinical features of the patients were analyzed through photography documentation, and patient description such as age of operation, operation technique, and complications were obtained through patient records. Macrostomia was corrected with a vermillion square flap method for commissure, overlapping muscle closure, along with either Z-plasty or W-plasty closure for the skin. Quality of lip commissure position, symmetry, thickness of vermillion, and scar result were recorded.
CLINICAL DISCUSSION
In all twelve patients repaired with the overlapping muscle closure and square flap, the lip commissures were formed with satisfactory shape, position, and thickness with no commissure contracture during the follow up period. The Z-plasty was a simpler method compared to the W-plasty, and resulted in comparable scars. One patient (adult with hemifacial macrostomia and W-plasty skin closure) underwent revision surgery for more accurate symmetry and position of the oral commissure.
CONCLUSION
There are many varieties of surgical repair for macrostomia, and each method should be adjusted and combined according to each patient. Overall, macrostomia repair with this technique combination produced satisfactory aesthetic and functional results in all twelve patients. Z-plasty for skin closure after muscle and vermillion closure was a simpler technique and resulted in comparable scars than W-pasty closure in this case series.
PubMed: 37956496
DOI: 10.1016/j.ijscr.2023.109023