The boy who lost his smile: facioscapulohumeral dystrophy in the head and neck.

BACKGROUND: Facioscapulohumeral dystrophy is the third most common muscular dystrophy and the one most likely to present primarily in the head and neck. METHODS: In this report, we present a case of a young child with subtle progressive bilateral facial weakness whose workup ultimately led to this diagnosis. Paralysis in this disorder is secondary to worsening muscle atrophy, which typically progresses in a cephalad to caudad direction. Despite facial paralysis being a key and early component of this illness, no prior descriptions in the otolaryngology literature exist. DISCUSSION: The case described is unusual in that the patient initially presented to a community otolaryngologist. In addition to workup, the disease characteristics, head and neck manifestations, and prognosis are discussed.

A comparison of facial nerve grading systems.

PURPOSE: This study aimed to compare a computerized tool to standard objective clinical scales for global and zone-specific assessment of facial nerve function. METHODS: This was a retrospective review of 77 patients with facial paralysis who underwent facial videography. Videos were independently scored by 3 facial nerve specialists using the House-Brackmann Scales (HBI and HBII). Digital scoring was performed with Facogram software. Scores were recorded and compared using intraclass and Pearson (r) correlations. RESULTS: Interobserver correlation was high with HBII, with overall scores in excellent agreement (intraclass correlation range, 0.78-0.95; P ≤ 0.0001). There were strong correlations between Facogram and HBII (r ≥ 0.67, P ≤ 0.0001) and strong intraobserver correlations between HBI and HBII (r ≥ 0.71, P ≤ 0.0001). The HBII required more clinician time [mean (SD), 72 (21) seconds per case], compared with Facogram, which did not require any clinician time. CONCLUSIONS: An automated, zone-specific facial analysis tool can eliminate clinician subjectivity and allow standardized assessment of facial paralysis.

Worldwide Testing of the eFACE Facial Nerve Clinician-Graded Scale.

BACKGROUND: The electronic, clinician-graded facial function scale (eFACE) is a potentially useful tool for assessing facial function. Beneficial features include its digital nature, use of visual analogue scales, and provision of graphic outputs and scores. The authors introduced the instrument to experienced facial nerve clinicians for feedback, and examined the effect of viewing a video tutorial on score agreement. METHODS: Videos of 30 patients with facial palsy were embedded in an Apple eFACE application. Facial nerve clinicians were invited to perform eFACE video rating and tutorial observation. Participants downloaded the application, viewed the clips, and applied the scoring. They then viewed the tutorial and rescored the clips. Analysis of mean, standard deviation, and confidence interval were performed. Values were compared before and after tutorial viewing, and against scores obtained by an experienced eFACE user. RESULTS: eFACE feedback was positive; participants reported eagerness to apply the instrument in clinical practice. Standard deviation decreased significantly in only two of the 16 categories after tutorial viewing. Subscores for static, dynamic, and synkinesis all demonstrated stable standard deviations, suggesting that the instrument is intuitive. Participants achieved posttutorial scores closer to the experienced eFACE user in 14 of 16 scores, although only a single score, nasolabial fold orientation with smiling, achieved statistically significant improvement. CONCLUSIONS: The eFACE may be a suitable, cross-platform, digital instrument for facial function assessment, and was well received by facial nerve experts. Tutorial viewing does not appear to be necessary to achieve agreement, although it does mildly improve agreement between occasional and frequent eFACE users.

Masseteric nerve use in facial reanimation.

PURPOSE OF REVIEW: To review the growing literature on the use of the masseteric nerve in facial reanimation, from direct neurotization, to 'baby-sitter' techniques, to its use in powering neuromuscular free tissue transfer. We focus on the indications for the different uses based on the timing of the facial paralysis and other patient factors. RECENT FINDINGS: The use of the masseteric nerve in facial reanimation is gaining widespread acceptance for use in an expanding number of clinical scenarios. Surgeon's experience and preference as well as patient selection are very important factors in choosing the appropriate surgical use of this nerve. SUMMARY: Facial reanimation surgery is a difficult challenge for any reconstructive surgeon. The use of the masseteric nerve branch to reanimate the face is gaining popularity. Its versatility, anatomical location, relative ease of dissection, low morbidity, and high potential for motor neural input make it an excellent option for many different reanimating techniques. The appropriate nerve use should be based on the type of facial paralysis, its timing, and patient factors such as age, prognosis, and desires. Understanding the benefits and potential drawbacks of utilizing this nerve represents an essential piece of knowledge for the facial reanimation surgeon.

Color Doppler ultrasound: effective monitoring of the buried free flap in facial reanimation.

OBJECTIVE: The gracilis muscle free flap has become a reliable means for smile reanimation for patients with facial paralysis. Because it is a buried flap, it presents a postoperative monitoring challenge. We sought to evaluate our experience with color Doppler ultrasound in the monitoring of gracilis free flap viability in the immediate postoperative setting. STUDY DESIGN: Case series with planned data collection. SETTING: Tertiary medical center. METHODS: Patients with facial paralysis treated with gracilis muscle free flap for smile reanimation performed between March 2009 and November 2010 were evaluated by color Doppler ultrasound and included in the study. Our experience with the use of the color Doppler ultrasound to monitor the gracilis muscle flap is presented. RESULTS: Forty-six patients were identified. In all cases, color Doppler ultrasound was used postoperatively to assess flow through the vascular pedicle. Outcomes included an early flap survival rate of 100%, with no instances of equivocal or absent flow on either the arterial or venous side. Color Doppler ultrasound provided important objective information regarding muscle perfusion postoperatively in several instances of equivocal postoperative perfusion of the flap. CONCLUSION: Color Doppler ultrasound is a safe, noninvasive method that can be performed serially to evaluate a buried free flap. We have had success in verifying normal arterial and venous flow through the pedicle using this method of monitoring of the gracilis muscle free flap during facial reanimation, and in 3 instances, it eliminated the need for wound exploration to verify appropriate muscle perfusion.

Surgical site infections after gracilis free flap reconstruction for facial paralysis.

Compared with other free tissue transfer procedures, the gracilis free muscle transfer (GFMT) for facial reanimation is unique in that the recipient site is typically uninvolved by malignancy or infection. In this study, the authors examined the incidence, bacteriology, and outcomes of surgical site infection (SSI) after gracilis free muscle transfer for facial reanimation. From 2003 to 2011, 105 patients underwent 107 GFMT operations, with 6 SSIs. All cases of infection occurred in patients receiving clindamycin, levofloxacin, and/or cefazolin perioperatively. None of the patients who received ampicillin-sulbactam developed an SSI. Surgical site cultures grew oral flora, including α-hemolytic streptococci, Haemophilus parainfluenzae, Fusobacterium and Neisseria species, and coagulase-negative staphylococci. Notably, there were no methicillin-resistant Staphylococcus aureus (MRSA) infections. These data may have implications for the optimal perioperative antibiotic choice in facial reanimation cases. Further study is needed to determine the ideal antibiotic regimen for this category of free flap surgeries.

Platysmectomy: an effective intervention for facial synkinesis and hypertonicity.

OBJECTIVES: To describe a procedure to permanently address platysmal synkinesis and hypertonicity and to report changes in quality of life associated with platysmectomy using the Facial Clinimetric Evaluation instrument. METHODS: Chemodenervation significantly relieves platysmal synkinesis in almost all patients with significant face and neck synkinesis associated with dynamic facial expressions. We recently began to offer platysmectomy as part of a permanent solution to chronic superficial torticollis-like neck symptoms. For a 10-month period, 24 patients underwent the procedure, and preoperative and postoperative Facial Clinimetric Evaluation data were obtained from 21 patients (88%). RESULTS: In 19 patients, platysmectomy was performed using local anesthesia without sedation. In the remaining 5 patients, platysmectomy was performed using general anesthesia concurrent with free gracilis transfer for smile reanimation. No intraoperative or postoperative complications occurred. Overall, the patients' quality of life significantly improved after platysmectomy (P = .02). CONCLUSION: Platysmectomy is straightforward and seems effective in treating neck synkinesis associated with chronic hypertonic platysmal activity.

Surgical treatment of the periocular complex and improvement of quality of life in patients with facial paralysis.

OBJECTIVE: A devastating sequela of facial paralysis is the inability to close the eye. The resulting loss of corneal protection can potentially lead to severe consequences. Eyelid weight placement, lower eyelid suspension, and brow ptosis correction are frequently performed to protect the eye. We sought to measure and report the change in quality of life (QOL) after surgical treatment of the periocular complex, using the validated Facial Clinimetric Evaluation (FaCE) QOL instrument. METHODS: From March 2009 to May 2010, 49 patients presenting to the Facial Nerve Center with paralytic lagophthalmos requiring intervention were treated with static periocular reanimation. Thirty-seven of the patients completed preoperative and postoperative FaCE surveys. RESULTS: Overall QOL, measured by the FaCE instrument, significantly improved following static periocular treatment. Mean FaCE scores increased from 44.1 to 52.7 (P < .001). Patients also reported a significant decrease in the amount of time their eye felt dry, irritated, or scratchy (P < .001). The amount of artificial tears and/or ointment also significantly decreased (P = .03). There were 2 cases of localized cellulitis with 1 eyelid weight extrusion. CONCLUSIONS: We report the first series of postoperative QOL changes following static periocular treatment for paralytic lagophthalmos. Patients report a notable improvement in periocular comfort and overall QOL.

Free gracilis transfer for smile in children: the Massachusetts Eye and Ear Infirmary Experience in excursion and quality-of-life changes.

BACKGROUND: Free muscle transfer for facial reanimation has become the standard of care in recent decades and is now the cornerstone intervention for dynamic smile reanimation. We sought to quantify smile excursion and quality-of-life (QOL) changes in our pediatric free gracilis recipients following reanimation. METHODS: We quantified gracilis muscle excursion in 17 pediatric patients undergoing 19 consecutive pediatric free gracilis transplantation operations, using our validated SMILE program, as an objective measure of functional outcome. These were compared against excursion measured the same way in a cohort of 17 adults with 19 free gracilis operations. In addition, we prospectively evaluated QOL outcomes in these children using the Facial Clinimetric Evaluation (FaCE) instrument. RESULTS: The mean gracilis excursion in our pediatric free gracilis recipients was 8.8 mm ± 5.0 mm, which matched adult results, but with fewer complete failures of less than 2-mm excursion, with 2 (11%) and 4 (21%), respectively. Quality-of-life measures indicated statistically significant improvements following dynamic smile reanimation (P = .01). CONCLUSIONS: Dynamic facial reanimation using free gracilis transfer in children has an acceptable success rate, yields improved commissure excursion, and improves QOL in the pediatric population. It should be considered first-line therapy for children with lack of a meaningful smile secondary to facial paralysis.

Good correlation between original and modified House Brackmann facial grading systems.

OBJECTIVES/HYPOTHESIS: Subjective scales of facial function were plagued with reporting variations until the House-Brackmann scale was described in 1985. Despite its utility, weaknesses were identified, including noninclusion of synkinesis phenomena and insensitivity to segmental weakness. Therefore, the scale was recently revised to address these weaknesses. The objective of this investigation was to determine agreement between the original and the updated House-Brackmann scales. STUDY DESIGN: Prospective clinical trial. METHODS: Fifty consecutive new facial paralysis patients underwent standardized facial videography while performing facial movements. Video clips were scored by three independent facial nerve clinicians. The time it took to produce a score for each method was tracked. Interobserver correlations were calculated, and comparisons were made between scores by using the original and modified House-Brackmann scales. RESULTS: : Interobserver correlation was high for both House-Brackmann scales. Overall scores were in excellent agreement (difference of 0.1 ± 0.5, no statistical difference), although the modified scale took substantially longer to calculate (120 seconds ± 20 seconds vs. 30 seconds ± 10 seconds, P < .001). CONCLUSIONS: We found substantial grading correlation between the original and the newly modified House-Brackmann scales. Because of specific zonal and synkinesis scoring, the modified scale took longer to score.

Transoral resection for squamous cell carcinoma of the base of the tongue.

OBJECTIVE: To review the oncologic and functional outcomes of patients with squamous cell carcinoma of the base of the tongue who underwent transoral tumor resection and neck dissection with or without postoperative adjuvant therapy. DESIGN: Retrospective medical record review. SETTING: Tertiary referral center. PATIENTS: All patients undergoing transoral resection of squamous cell carcinoma on the base of the tongue as part of their primary treatment from January 1, 1996, through January 31, 2005. MAIN OUTCOME MEASURES: We analyzed overall survival, disease-specific survival, local control, and locoregional control rates using the Kaplan-Meier method. Speech and swallowing function and treatment-related morbidity were also analyzed. RESULTS: A total of 20 patients underwent transoral resection. Four patients had surgery only, 12 had surgery and radiotherapy, and 4 had surgery and chemoradiotherapy. One patient had stage II disease, 3 had stage III disease, and 16 had stage IVA disease. The Kaplan-Meier overall survival rate was 90.0%, and the disease-specific survival rate was 94.7% at 2 years, with a mean follow-up of 3.7 years. Median hospital stay was 4.7 days. Patients who received a tracheostomy underwent decannulation with a median tracheostomy time of 5.5 days. Seven of 9 patients who received a percutaneous endoscopic gastrostomy tube had it removed. Three patients developed local recurrence, there were no regional recurrences, and 2 patients developed distant metastasis. CONCLUSIONS: Transoral resection of squamous cell carcinoma of the base of the tongue with postoperative adjuvant therapy provided excellent local and regional control and minimized morbidity. Transoral resection is a reasonable treatment option for patients with oropharyngeal squamous cell carcinoma, resulting in very low overall loss of organ function in properly selected patients.

Scapula osteocutaneous free flap reconstruction of the head and neck: impact of flap choice on surgical and medical complications.

BACKGROUND: The scapula osteocutaneous free flap is frequently used to reconstruct complex head and neck defects given its tissue versatility. Because of minimal atherosclerotic changes in its vascular pedicle, this flap also may be used as a second choice when other osseous flaps are not available because of vascular disease at a preferred donor site. METHODS: We performed a retrospective chart review evaluating flap outcome as well as surgical and medical complications based upon the flap choice. RESULTS: The flap survival rate was 97%. The surgical complication rate was similar for the 21 first-choice flaps (57.1%) and the 12 second-choice flaps (41.7%; p = .481). However, patients having second-choice flaps had a higher rate of medical complications (66.7%) than those with first-choice flaps (28.6%; p = .066). Age and the presence of comorbidities were associated with increased medical complications. All patients with comorbidities that had a second-choice flap experienced medical complications, with most being severe. CONCLUSIONS: The scapula osteocutaneous free flap has a high success rate in head and neck reconstruction. Surgical complications occur frequently regardless of whether the flap is used as a first or second choice. However, medical complications are more frequent and severe in patients undergoing second-choice flaps.