Workhorse Free Functional Muscle Transfer Techniques for Smile Reanimation in Children with Congenital Facial Palsy: Case Report and Systematic Review of the Literature.

BACKGROUND: Pediatric facial palsy represents a rare multifactorial entity. Facial reanimation restores smiling, thus boosting self-confidence and social integration of the affected children. The purpose of this paper is to present a systematic review of microsurgical workhorse free functional muscle transfer procedures with emphasis on the long-term functional, aesthetic, and psychosocial outcomes. MATERIALS AND METHODS: We performed a literature search of the PubMed database from 1995 to 2019 using the following search strategy: "facial paralysis"[Title/Abstract] OR "facial palsy"[Title]. We used as limits: full text, English language, age younger than 18 years, and humans. Two independent reviewers performed the online screening process using Covidence. Forty articles met the inclusion criteria. The protocol was aligned with the PRISMA statement (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) and was registered at the International Prospective Register of Systematic Reviews (PROSPERO, CRD42019150112) of the National Institute for Health Research. RESULTS: Free functional muscle transfer procedures include mainly segmental gracilis, latissimus dorsi, and pectoralis minor muscle transfer. Facial reanimation procedures with the use of the cross-face nerve graft (CFNG) or masseteric nerve result in almost symmetric smiles. The transplanted muscle grows harmoniously along with the craniofacial skeleton. Muscle function and aesthetic outcomes improve over time. All children presented improved self-esteem, oral commissure opening, facial animation, and speech. CONCLUSIONS: A two-stage CFNG plus an FFMT may restore a spontaneous emotive smile in pediatric facial palsy patients. Superior results of children FFMT compared to adults FFMT are probably attributed to greater brain plasticity.

Contralateral Depressor Labii Inferioris Chemodenervation for Congenital Unilateral Lower Lip Palsy.

INTRODUCTION: Congenital unilateral lower lip palsy - also known as asymmetric crying facies - is isolated asymmetry of the lower lip unilaterally. It is characterized by isolated lower lip asymmetry during smiling and speech. Although etiology is unknown, depressor labii inferioris (DLI) weakness is hold responsible. AIM: Purpose of this study was to evaluate the effectiveness of contralateral depressor labii inferioris botulinum toxin injection on patients' concern levels and patient satisfaction. Ten units of botulinum toxin A injection was carried out to the healthy contralateral side. METHODOLOGY: Eleven patients were treated. Patients' pretreatment and posttreatment concern regarding asymmetry during speech and smiling was evaluated with a questionnaire. Patients' perception of treatment satisfaction was also evaluated with a questionnaire. RESULTS: Mean score related to concern about asymmetric appearance during smiling decreased from 1.6 ±â€Š0.8 to 0.5 ±â€Š0.5. Mean score related to concern about asymmetric appearance during speech decreased from 1.6 ±â€Š0.5 to 0.4 ±â€Š0.5. Eleven out of 11 patients reported improvement with speech whereas 10 out of 11 patients reported improvement with smiling. No weakness about oral competence was reported. CONCLUSION: Most congenital unilateral lower lip palsy patients are concerned regarding their asymmetric appearance while smiling or speaking. Chemodenervation of the contralateral DLI muscle reduces concern levels and has high patient satisfaction. Chemodenervation of the contralateral healthy DLI muscle is a valid, practical treatment option.

[Congenital facial palsy].

Congenital facial palsy is unilateral or bilateral facial nerve palsy at birth due to genetic or different pathogenic factors. It can be divided into syndromic type and non-syndromic type according to its accompanying symptom. The pathogeny and symptom of each type are different, in part with genetic heterogeneity. Congenital facial palsy cannot recover spontaneously. Different types of congenital facial palsy have different treatment schemes. The treatment is significant to the improvement of life quality and physical and mental development of children with congenital facial palsy.

Congenital asymmetric crying facies syndrome: A case report.

INTRODUCTION: Congenital asymmetric crying facies (ACF) in newborns is a rare condition usually caused by unilateral agenesis or hypoplasia of the depressor anguli oris muscle on one side of the mouth (symmetric face at rest and asymmetric face while crying), which is often accompanied with other malformations. CASE REPORT: We present a case of a female newborn with nonconsanguineous ethnic Han Chinese parents who presented with 37 minutes of breathlessness and asymmetrical face when crying. A thorough physical examination had been conducted. The patient was diagnosed with aspiration pneumonia and congenital ACF syndrome, accompanied with congenital bilateral anophthalmia, left homolateral auricle dysplasia, malformation in the left-hand thumb, patent ductus arteriosus (PDA), and patent foramen ovale (PFO) and tracheoesophageal fistula. The patient's mother underwent routine fetal sonogram at 25 weeks gestation, which showed major anatomical anomalies in the eyes of the fetus. The mother chose to pregnancy until vaginal delivery. This case is unique because congenital bilateral anophthalmia has not been reported in such patients before. CONCLUSION: Careful physical examination of newborns and genetic testing are important for early diagnosis of neonatal asymmetric crying facies (NACF), especially if ACF is present. Early determination of the etiology and future screenings are very important for the management of this condition. The lower lip on the affected side looks thinner because of the lack of the muscle agenesis, so the use of ultrasound to observe facial muscles and electrodiagnostic testing could be helpful for the differential diagnosis of NACF from congenital facial nerve dysplasia.

Toxina botulínica para tratamento de síndrome do Choro Assimétrico: relato de caso / Botulinum toxin in the treatment of asymmetric crying face syndrome: a case report

Síndrome do choro assimétrico é uma condição congênita secundária à hipoplasia ou ausência do músculo depressor do ângulo da boca. Trata-se de uma condição não tão incomum que pode cursar com assimetria facial ao chorar e sorrir, além de poder estar associadas a outras malformações congênitas. Crianças com essa deformidade podem sofrer dificuldades psicossociais e introversão. O arsenal terapêutico dessa condição já foi estudado e discutido na literatura com ênfase em abordagens cirúrgicas e invasivas. Relatamos aqui um caso de uma criança de 9 anos com essa síndrome, tratada, de forma menos invasiva, com toxina botulínica, com um bom resultado e satisfação.

Asymmetric crying face syndrome is a congenital condition secondary to hypoplasia or absence of the depressor muscle at the mouth angle. It is a common condition that presents with facial asymmetry while crying and smiling and may be associated with other congenital malformations. Children with this deformity may experience psychosocial difficulties and introversion. The therapeutic arsenal of this condition has already been studied and discussed in the literature with an emphasis on surgical and invasive approaches. We report here a case of a 9-year-old child with this syndrome, treated less invasively with botulinum toxin, with good result and satisfaction.

An exome sequencing study of Moebius syndrome including atypical cases reveals an individual with CFEOM3A and a TUBB3 mutation.

Moebius syndrome is characterized by congenital unilateral or bilateral facial and abducens nerve palsies (sixth and seventh cranial nerves) causing facial weakness, feeding difficulties, and restricted ocular movements. Abnormalities of the chest wall such as Poland anomaly and variable limb defects are frequently associated with this syndrome. Most cases are isolated; however, rare families with autosomal dominant transmission with incomplete penetrance and variable expressivity have been described. The genetic basis of this condition remains unknown. In a cohort study of nine individuals suspected to have Moebius syndrome (six typical, three atypical), we performed whole-exome sequencing to try to identify a commonly mutated gene. Although no such gene was identified and we did not find mutations in PLXND1 and REV3L, we found a de novo heterozygous mutation, p.E410K, in the gene encoding tubulin beta 3 class III (TUBB3), in an individual with atypical Moebius syndrome. This individual was diagnosed with near-complete ophthalmoplegia, agenesis of the corpus callosum, and absence of the septum pellucidum. No substantial limb abnormalities were noted. Mutations in TUBB3 have been associated with complex cortical dysplasia and other brain malformations and congenital fibrosis of extraocular muscles type 3A (CFEOM3A). Our report highlights the overlap of genetic etiology and clinical differences between CFEOM and Moebius syndrome and describes our approach to identifying candidate genes for typical and atypical Moebius syndrome.

Lengthening Temporalis Myoplasty: Virtual Animation-Assisted Technical Video.

Lengthening temporalis myoplasty is a well-established procedure for dynamic palliative reanimation of the lip in facial palsy sequelae. The particularity of this technique is that the entire temporal muscle is transferred from the coronoid process to the upper half of the lip without interposition of aponeurotic tissue. To date, no video describing the technique was available. This is the first video describing the entire procedure, from preoperative markings through postoperative rehabilitation. In the video presented herein, the authors craft virtual three-dimensional animations in addition to a live operation on a patient performed by Daniel Labbé, who first described this technique 20 years ago.

Facial animation with gracilis muscle transplant reinnervated via cross-face graft: Does it change patients' quality of life?

PURPOSE: Gracilis muscle reinnervated by the contralateral facial nerve via cross-graft technique is nowadays considered to be a first-line procedure for facial animation in unilateral palsies. Despite the wide number of papers published analyzing technical aspects, refinements, functional results, and cosmetic outcomes, only a few authors have focused their publications on the patient's perspective and impact on QOL of these procedures. MATERIAL AND METHODS: Changes in quality of life in 42 patients treated with gracilis muscle transplant reinnervated via cross-face graft were analyzed through a comparison of preoperative and postoperative items on the Facial Disability Index questionnaire. Statistical evaluation with a paired t-test was performed concerning overall results and specific items modifications. RESULTS: Overall improvement of QOL was found to be highly significant (p = 0.001). Mouth and eye functions were the most improved (p = 0.001), whereas isolation (p = 0.004) and feeling calm and peaceful (p = 0.001) were the most improved among the social functions. CONCLUSIONS: Facial animation with gracilis neuromuscular transplantation reinnervated with contralateral healthy facial nerve via a cross-graft procedure has been demonstrated to be a safe and reliable procedure in the treatment of congenital or established facial palsies. Our results on quality of life impact support that these operations are not only reliable and safe but also of primary relevance to patients' everyday lives.

Ophthalmic profile and systemic features of pediatric facial nerve palsy.

BACKGROUND: Facial nerve palsy (FNP) occurs less frequently in children as compared to adults but most cases are secondary to an identifiable cause. These children may have a variety of ocular and systemic features associated with the palsy and need detailed ophthalmic and systemic evaluation. METHODS: This was a retrospective chart review of all the cases of FNP below the age of 16 years, presenting to a tertiary ophthalmic hospital over the period of 9 years, from January 2000 to December 2008. RESULTS: A total of 22 patients were included in the study. The average age at presentation was 6.08 years (range, 4 months to 16 years). Only one patient (4.54%) had bilateral FNP and 21 cases (95.45%) had unilateral FNP. Seventeen patients (77.27%) had congenital palsy and of these, five patients had a syndromic association, three had birth trauma and nine patients had idiopathic palsy. Five patients (22.72%) had an acquired palsy, of these, two had a traumatic cause and one patient each had neoplastic origin of the palsy, iatrogenic palsy after surgery for hemangioma and idiopathic palsy. Three patients had ipsilateral sixth nerve palsy, two children were diagnosed to have Moebius syndrome, one child had an ipsilateral Duane's syndrome with ipsilateral hearing loss. Corneal involvement was seen in eight patients (36.36%). Amblyopia was seen in ten patients (45.45%). Neuroimaging studies showed evidence of trauma, posterior fossa cysts, pontine gliosis and neoplasms such as a chloroma. Systemic associations included hemifacial macrosomia, oculovertebral malformations, Dandy Walker syndrome, Moebius syndrome and cerebral palsy CONCLUSIONS: FNP in children can have a number of underlying causes, some of which may be life threatening. It can also result in serious ocular complications including corneal perforation and severe amblyopia. These children require a multifaceted approach to their care.

Lengthening temporalis myoplasty and facial paralysis from birth.

INTRODUCTION: Congenital facial paralysis (FP) is present from birth. It can produce major esthetic and functional disorders. It can be from two different etiologies: developmental and acquired. There is no curative treatment for congenital FP and the aim for the plastic surgeon is to restore a smile as symmetrical and as dynamic as possible. For this, two opposite techniques can be used: muscular free flaps and locoregional flaps whose lengthening temporalis myoplasty. MATERIEL AND METHODS: We report our series of 34 congenital FP patients who were operated by lengthening temporalis myoplasty (LTM). We divided the patients into three categories: acquired FP (11 cases), isolated developmental FP (13 cases), and syndromic developmental FP (10 cases). The evaluation of the smile is based on the quality of the commissural course compared to the healthy side and the spontaneity of it. RESULTS: In the acquired FP group, 100% obtained a spontaneous smile with a postoperative delay of 9.5 months, 12 of the 13 cases of isolated developmental FP (92.7%) after 7.3 months postoperatively, and finally, in the 10 cases of syndromic FP, nine (90%) had a spontaneous smile after 9.7 months. DISCUSSION: Muscular free flaps continue to be the gold standard for the reanimation of smile on the FP. To our knowledge, no articles comparing smile restoration using free flap and smile restoration using LTM exist. A comparison of the success rates from different studies shows that both these techniques yield good results and can be used for smile restoration in FP. This technique is faster and easier than a free flap and has a same result, which is why we consider this technique as a reference on smile reanimation in FP.

The incidence of facial vessel agenesis in patients with syndromic congenital facial palsy.

BACKGROUND: Congenital facial palsy can result in significant disfigurement. A potential treatment option is free functional muscle transfer to reanimate the face. For this to be possible, a suitable recipient artery and vein must be present in the affected hemiface. In this study, the authors aim to identify whether patients with syndromic congenital facial palsy have a higher rate of facial vessel agenesis than those with isolated congenital facial palsy. METHODS: Patients were identified between November of 2006 and October of 2013. Patients were stratified into two groups: those with syndromic congenital facial palsy and those with isolated congenital facial palsy. The presence or absence of facial vessels was determined intraoperatively. RESULTS: Forty-seven eligible patients were included in the study. Those with syndromic congenital facial palsy were significantly more likely to have an absent facial vein than patients with isolated congenital facial palsy (p = 0.015). There was a strong trend toward those with syndromic facial palsy lacking a facial artery (p = 0.08). Subgroup analysis of patients with Möbius syndrome revealed that these patients were significantly more likely to have facial artery agenesis than those with isolated congenital facial palsy (p = 0.03). CONCLUSIONS: Facial vessel agenesis is significantly more common in patients with syndromic congenital facial palsy compared with those with isolated congenital facial palsy. This must be considered in the preoperative planning for facial reanimation with free functional muscle transfer. The operating surgeon should consider vascular studies of the affected hemiface before undertaking the procedure.

Influence of congenital facial nerve palsy on craniofacial growth in craniofacial microsomia.

Facial muscles are of major importance in human craniofacial growth and development. The purpose of our study was to investigate whether congenital facial nerve palsy influences craniofacial growth in craniofacial microsomia. Fifty-one patients with unilateral craniofacial microsomia and no history of craniofacial skeletal surgery whose radiographs were taken after craniofacial growth was complete were included in this study. These patients were divided into groups in which the facial nerve was involved or uninvolved. The authors evaluated a total of seven measurement items to analyze the midface and mandibular asymmetry. Twenty patients had facial nerve involvement, and 31 had no involvement. None of the measurement items revealed any significant differences between the facial nerve-involved group and the uninvolved group within the same modified Pruzansky grade. There was no correlation between the type of facial nerve involvement and the measurement items. In relationships among the measurement items within each group, maxillary asymmetry was indirectly correlated with mandibular asymmetry or midline deviation through the occlusal plane angle in the uninvolved groups. However, in the facial nerve-involved group, the relationships disappeared. When the correlations in the facial nerve-involved group were compared with those of the uninvolved group, the relationships in the uninvolved group appeared more significant than in the facial nerve-involved group. The loss of relationships between the upper and lower jaw in the facial nerve-involved group might have been caused by subtle changes, which occur in midfacial bones and in the mandible due to facial nerve palsy. The main limitation of our study is that aside from facial nerve palsy, craniofacial microsomia has many factors that can influence craniofacial growth, such as hypoplasia of the mandibular condyle and soft tissue deficiencies.

Dynamic facial reanimation with orthodromic temporalis tendon transfer in children.

IMPORTANCE: To our knowledge, orthodromic temporalis tendon transfer (OTTT) for dynamic facial reanimation has not been described for use in children. OBSERVATIONS: Three pediatric patients with permanent facial paralysis underwent OTTT using our modified technique between August 30, 2010, and January 23, 2012. Outcomes were assessed by the surgeons, patients, and patient families, with the longest follow-up period being 13 months after surgery. Two patients were 4 years old at the time of surgery, and the third patient was 17 years old. All underwent upper eyelid gold weight placement and OTTT. The hospital length of stay was 1 to 2 nights. By the first postoperative visit, all patients exhibited improved symmetry at rest, creation of a melolabial crease, and voluntary movement of the oral commissure and smile production without physical therapy. CONCLUSIONS AND RELEVANCE: The OTTT for dynamic facial reanimation in children seems to be safe and effective. To our knowledge, this is the first report of the use of this procedure in pediatric patients. This procedure has an advantage over free muscle transfer procedures in achieving immediate dynamic reanimation in a single-stage surgical procedure, without significant additional donor site morbidity. This is an important procedure in our armamentarium for treating pediatric facial paralysis.

Lateral facial cleft associated with accessory mandible having teeth, absent parotid gland and peripheral facial weakness.

Transverse facial cleft is a very rare malformation. The Tessier no. 7 cleft is a lateral facial cleft which emanates from oral cavity and extends towards the tragus, involving both soft tissue and skeletal components. Here, we present a case having transverse facial cleft, accessory mandible having teeth, absent parotid gland and ipsilateral peripheral facial nerve weakness. After surgical repair of the cleft in 2-month of age, improvement of the facial nerve function was detected in 3-year of age. Resection of the accessory mandible was planned in 5-6 years of age.

Rehabilitation of congenital facial palsy with temporalis flap--case series and literature review.

BACKGROUND: Congenital facial paralysis is a rare disorder with an incidence of less than 1 in 1000 live births. Several surgical rehabilitation techniques have been described, such as free flaps or transpositions of the temporalis muscle. We report our experience of transposition of the temporalis muscle in this indication. MATERIALS AND METHODS: Retrospective monocentric study between January 2005 and December 2009. RESULTS: Five children (mean age at the surgery: 7.3 years, range 6-13) presenting with a congenital facial paralysis were treated by a temporalis muscle transposition during the considered period. An etiological assessment was achieved by carrying out a petrous bone CT, a cerebral and petrous MRI, and audiometric evaluation. Four patients out of 5 (80%) showed a satisfactory and stable functional outcome over time (average follow-up: 26 months). CONCLUSION: Temporalis muscle transposition is a single-stage procedure, easily adapted for the pediatric condition of congenital facial paralysis. It is our opinion that this procedure needs to be carried out at the end of the pre-school period.

Successful salvage surgery after treatment failures with cross graft and free muscle transplant in facial reanimation.

BACKGROUND: The microneurovascular transfer of a free-muscle transplant is the procedure of choice for facial animation, It is characterized by low morbidity in both adult and paediatric patients. In spite of the improvements in microsurgical techniques, failures due to absent revascularization or reinnervation of the transplanted muscle or infections causing flap necrosis are observed. We propose a second surgical procedure based on the gracilis muscle transplant reinnervated by the masseteric nerve as a solution for these cases. METHODS: We analyzed and report on two patients treated in our department after the failure of a previous cross-facial nerve graft and free muscle transplant. They were treated with a new facial reanimation using the contralateral gracilis muscle and the masseteric nerve as the donor nerve. RESULTS AND DISCUSSION: We did not observe any postoperative complications, and all of the flaps survived. Reinnervation and contraction of the muscle appeared 3-4 months postoperatively, with good functional and aesthetic results. CONCLUSIONS: This technique is a one-step procedure characterized by reliable flap harvesting, low donor site morbidity and good activity of the masseteric nerve. We consider it as a good option for treatment of facial animation failures.

Experience with developmental facial paralysis: Part I. Diagnosis and associated stigmata.

BACKGROUND: This study is a thorough literature review of the clinical presentation and evaluation of developmental facial paralysis, with a systematic description of the various stigmata and associated anomalies. It is hoped that this approach will facilitate the differentiation of developmental facial paralysis from other causes of facial paralysis present at birth. METHODS: Forty-two cases of developmental facial paralysis were identified in a retrospective clinical review (1980 to 2010); 34 were children (80.95 percent; age, 8±6 years) and eight were adults (19.05 percent; age, 27±12 years). Thirty-one patients had simple developmental paralysis, and two patients had developmental unilateral lower lip palsy. There were nine patients with associated anomalies or craniofacial syndromes. Five of these patients had multiple cranial nerve deficits. RESULTS: Analysis of the various stigmata revealed significant correlation between the presence of developmental facial paralysis and amblyopia, hypoplastic facial nerve on imaging or surgical exploration, lower alar atresia, and skin changes (i.e., acne), but not the ear abnormalities. CONCLUSIONS: Early targeted screening and diagnosis, with prompt specialized treatment, improves the physical and emotional development of children with developmental facial paralysis and reduces the prevalence of amblyopia and other sequelae of the condition, thus facilitating reintegration among their peers. Given the dramatic presentation of this condition, accurate and reliable guidelines are necessary to facilitate early diagnosis, initiate appropriate therapy, and provide support and counseling to the family.