Successful use of VV ECMO in managing negative pressure pulmonary edema.

The indications for extracorporeal membrane oxygenation (ECMO) are expanding. Postobstructive pulmonary edema, also known as negative pressure pulmonary edema, can result in severe respiratory compromise and acute respiratory distress syndrome. We present a case of a 26-year-old female with laryngeal papillomatosis and laryngospasm after direct laryngoscopy, who developed severe NPPE refractory to mechanical ventilator support, which was successfully treated with veno-venous ECMO.

Main branch of ACN-to-RLN for management of laryngospasm due to unilateral vocal cord paralysis.

OBJECTIVES/HYPOTHESIS: This study explored the feasibility and efficiency of main branch of ansa cervicalis nerve (ACN)-to-recurrent laryngeal nerve (RLN) anastomosis for management of paroxysmal laryngospasm due to unilateral vocal cord paralysis (UVCP). METHODS: Thirteen patients who underwent main branch of ACN-to-RLN anastomosis for management of paroxysmal laryngospasm due to UVCP were enrolled in the present study. Multidimensional assessments, including videostroboscopy, voice assessment, and laryngeal electromyography (LEMG), were performed preoperatively and postoperatively. RESULTS: This series was limited to UVCP with iatrogenic causes, including thyroidectomy, cervical spine surgery, and thoracic surgery. After main branch of ACN-to-RLN anastomosis, all cases showed significant airway improvement, and laryngospasm was completely abolished in 92.3% (12 of 13) of cases. Videostroboscopy showed that the bulging and paradoxical adduction of the affected vocal cord during a sniff were abolished immediately after operation, and there was no significant difference in vocal fold position or glottal closure before versus after the operation. LEMG showed that the postoperative recruitment and amplitude of voluntary motor unit potential in the affected thyroarytenoid muscle during a sniff were significantly decreased compared to preoperative values, and postoperative recruitment showed significant improvement during phonation compared to that preoperatively. Voice assessment showed that there were no significant differences in overall grade, roughness, breathiness, jitter (local), shimmer (local), noise-to-harmonics ratio, or maximum phonation time after the operation compared to the preoperative values. CONCLUSIONS: Main branch of ACN-to-RLN anastomosis could have long-lasting efficacy in the management of paroxysmal laryngospasm due to UVCP, with no apparent compromise of voice quality. LEVEL OF EVIDENCE: 4 Laryngoscope, 130:2412-2419, 2020.

Concurrent Glossopharyngeal Neuralgia and Hemi-Laryngopharyngeal Spasm (HeLPS): A Case Report and a Review of the Literature.

BACKGROUND AND IMPORTANCE: Hemi-laryngopharyngeal spasm (HeLPS) has been recently described but is not yet widely recognized. Patients describe intermittent coughing and choking and can be cured following microvascular decompression of their Xth cranial nerve. This case report and literature review highlight that HeLPS can co-occur with glossopharyngeal neuralgia (GN) and has been previously described (but not recognized) in the neurosurgical literature. CLINICAL PRESENTATION: A patient with GN and additional symptoms compatible with HeLPS is presented. The patient reported left-sided, intermittent, swallow-induced, severe electrical pain radiating from her ear to her throat (GN). She also reported intermittent severe coughing, throat contractions causing a sense of suffocation, and dysphonia (HeLPS). All her symptoms resolved following a left microvascular decompression of a loop of the posterior inferior cerebellar artery that was pulsating against both the IXth and Xth cranial nerves. A review of the senior author's database revealed another patient with this combination of symptoms. An international literature review found 27 patients have been previously described with symptoms of GN and the additional (but not recognized at the time) symptoms of HeLPS. CONCLUSION: This review highlights that patients with symptoms compatible with HeLPS have been reported since 1926 in at least 4 languages. This additional evidence supports the growing recognition that HeLPS is another neurovascular compression syndrome. Patients with HeLPS continue to be misdiagnosed as conversion disorder. The increased recognition of this new medical condition will require neurosurgical treatment and should alleviate the suffering of these patients.

Episodic hemilaryngopharyngeal spasm (HELPS) syndrome: case report of a surgically treatable novel neuropathy.

The authors describe a novel cranial neuropathy manifesting with life-threatening episodic hemilaryngopharyngeal spasm (HELPS). A 50-year-old woman presented with a 4-year history of intermittent throat contractions, escalating to life-threatening respiratory distress. Botulinum toxin injections into her right vocal cord reduced the severity of her spasms, but the episodes continued to occur. MRI demonstrated a possible neurovascular conflict involving the cranial nerve IX-X complex and the posterior inferior cerebellar artery. Microvascular decompression of the upper rootlets of the vagal nerve eliminated her HELPS without complication. The authors propose a mechanism of HELPS implicating isolated involvement of the upper motor rootlets of the vagus nerve.

Effect of Botulinum Toxin and Surgery among Spasmodic Dysphonia Patients.

Objective The effect of botulinum toxin among patients with adductor spasmodic dysphonia (AdSD) is temporary. To optimize long-term treatment outcome, other therapy options should be evaluated. Alternative treatment options for AdSD comprise several surgical treatments, such as thyroarytenoid myotomy, thyroplasty, selective laryngeal adductor denervation-reinnervation, laryngeal nerve crush, and recurrent laryngeal nerve resection. Here, we present the first systematic review comparing the effect of botulinum toxin with surgical treatment among patients diagnosed with AdSD. Data Sources MEDLINE (PubMed), EMBASE, and the Cochrane Library. Methods Articles were reviewed by 2 independent authors, and data were compiled in tables for analysis of the objective outcome (voice expert evaluation after voice recording), the subjective outcome (patient self-assessment scores), and voice-related quality of life (Voice Health Index scores). Results No clinical trials comparing both treatment modalities were identified. Single-armed studies evaluated either the effect of botulinum toxin or surgical treatment. Thirteen studies reported outcomes after botulinum toxin treatment (n = 419), and 9 studies reported outcomes after surgical treatment (n = 585 patients). A positive effect of bilateral botulinum toxin injections was found for the objective voice outcome, subjective voice outcome, and quality of life. The duration of the beneficial effect ranged from 15 to 18 weeks. Surgical treatment had an overall positive effect on objective voice improvement, subjective voice improvement, and quality of live. Conclusion No preference for one treatment could be demonstrated. Prospective clinical trials comparing treatment modalities are recommended to delineate the optimal outcomes by direct comparison.

Surgery or botulinum toxin for adductor spasmodic dysphonia: a comparative study.

OBJECTIVES: Currently, botulinum toxin (Botox) injection is the standard of treatment for adductor spasmodic dysphonia (ADSD). We sought to compare the outcome of selective laryngeal adductor denervation-reinnervation (SLAD-R) surgery for ADSD to that of Botox injections. METHODS: Patient-oriented measures (VHI-10) and objective single-blinded gradings of digital voice recordings were utilized as outcome measures. The surgical cohort, recruited by retrospective patient selection, consisted of 77 patients with a mean follow-up time of 7.54 +/- 2.55 years (range, 2.2 to 14.2 years). The injection cohort, recruited prospectively, included 28 patients with a mean follow-up time of 46.37 +/- 5.51 days (range, 36 to 54 days). RESULTS: As measured by the VHI-10, the surgical patients had significantly improved voice handicap outcome scores (mean, 14.4 +/- 13.6) as compared to the patients who had Botox injection (mean, 26.5 +/- 12.1; p = 0.001). Aside from VHI-10 item 2, the surgical group demonstrated significantly improved voice-related function on each VHI-10 component (p = 0.01). Within the injection subgroup, 88% agreed that Botox successfully treats their ADSD, yet only 63% agreed that Botox improves their speech consistently. Within the surgical subgroup, 82% would recommend this surgery to others, and 78% agreed that their voice was actually better after surgery than after Botox. Objective voice ratings demonstrated similar levels of breathiness and overall voice quality in the treatment subgroups. CONCLUSIONS: When indicated, the SLAD-R surgery for ADSD demonstrates outcomes equal to or superior to those of the current standard of Botox injections.

Transoral resection of short segment Zenker's diverticulum and cricopharyngeal myotomy: an alternative minimally invasive approach.

OBJECTIVES/HYPOTHESIS: Transoral treatment of cricopharyngeal bar and small Zenker's diverticulum remains a challenge. We propose a new transoral approach for transoral cricopharyngeal myotomy (TOCPM) for patients with cricopharyngeal spasm and pharyngeal bar, and for transoral resection of diverticula (TORD) with cricopharyngeal myotomy in the patient with small (<3 cm) Zenker's diverticulum. METHODS: A retrospective review was conducted of 45 patients with cricopharyngeal spasm (21) and Zenker's diverticulum (24), where 14 patients were considered suitable for TORD and TOCPM. TOCPM used the Weerda laryngoscope (Karl Storz, Tuttlingen, Germany) to expose the cricopharyngeal bar using a microscope; the mucosa is cut and then the muscle is transected using monopolar cautery. A 0 degrees endoscope is inserted through the incision to ensure complete myotomy. Incision closure is by interrupted 4-0 Vicryl sutures (Ethicon Inc., Somerville, NJ) and fibrin glue. For the TORD procedure, the diverticular sac is everted and then resected using scissors. Through the sac opening, the TOCPM is completed. The sac opening is then closed as described in TOCPM. The patients are kept without food for 24 hours, followed by feeding and discharge. Modified barium swallow (MBS) evaluated functional results. RESULTS: Fourteen patients underwent TOCPM (eight), and TOCPM+TORD (six). There was one case of TOCPM that was aborted due to excessive bleeding, which prevented full myotomy. The rest did well. All were discharged the next day. Two poor results from the TOCPM and TOCPM+TORD group were due to poor esophageal motility. The remainder of patients had resolution of dysphagia and normalized MBS. No patient developed stricture or complications. CONCLUSIONS: Short segment Zenker's diverticulum and cricopharyngeal bar can now be addressed completely with a transoral approach. Because there is complete closure of the mucosal incision, prolonged hospitalization can be avoided.

Cervical osteophytes: a cause of potentially life-threatening laryngeal spasms. Case report.

This 80-year-old woman presented with acute breathing difficulty during neck flexion when cyanosis also developed. The only potential causes were detected on cervical magnetic resonance imaging: two large anterior cervical osteophytes compressing the retropharyngeal space. Excision of these osteophytes resulted in resolution of the symptoms.

A model of neuronopathic Gaucher disease.

Gaucher disease (GD) is a lysosomal disorder involving the accumulation of glucocerebroside in the liver, spleen, bones and brain. Some patients exhibit only systemic disease (type I), but others have additional neurological signs which may lead to rapid neurodegeneration in infancy (type II) or take a more intermediate course (type III). Types II and III are collectively known as neuronopathic Gaucher disease (NGD). Systemic disease can now be treated by enzyme replacement therapy (ERT), but its efficacy in NGD is limited. Two infants who presented with bulbar palsy and failure to thrive were enzymatically diagnosed at 8 months with NGD. They were started on high-dose ERT (120 IU/kg every 2 weeks). Both underwent serial oculomotor assessment and an audiological battery, including visual reinforcement audiometry, otoacoustic emissions, and the auditory brain stem response (ABR). Biochemical markers showed an incomplete systemic response to ERT, but neurological deterioration was relentless, leading to death at 16 and 25 months. Oculomotor testing revealed a complete absence of saccadic eye movements and progressive bilateral sixth nerve palsy in one. Audiological assessment revealed progressive deterioration of ABRs, but with normal peripheral hearing and otoacoustic emissions. Both infants showed neurological deterioration in spite of high-dose ERT. The audiological findings suggested a loss of inner hair cell pathway function with preserved outer hair function, similar to what is seen in auditory neuropathy. The unusual pattern of audiological and oculomotor abnormalities is consistent with an excitotoxic mechanism predisposing nerve cells to glucocerebroside toxicity. Such excitotoxic damage may be amenable to direct therapeutic intervention.

Apnea and the elongated uvula.

Four cases of respiratory distress and apnea associated with an elongated uvula are presented. In all cases, the uvula was found to intermittently fall onto the epiglottis and vocal cords. In all four patients, resection of the uvula led to resolution of all airway symptoms. It is hypothesized that the uvula, touching the vocal cords, caused intermittent laryngospasm and subsequent symptoms of cough, airway obstruction, and cyanosis. The anatomic reasons for such phenomenon are discussed.

Sleep-related laryngeal obstruction presenting as snoring or sleep apnea.

The difference between snoring (with or without sleep apnea) and laryngeal stridor resulting from laryngeal dysfunction may not be readily apparent. Two cases of Shy-Drager syndrome and one undiagnosed case in which laryngeal dysfunction was exacerbated by sleep are reported. Such dysfunction might create life-threatening situations for which emergency tracheostomy should be considered. The importance of differentiating stridor from snoring is discussed.

Respiratory distress after recurrent laryngeal nerve sectioning for adductor spastic dysphonia.

Adductor spastic dysphonia is a voice sign associated with various neurologic and psychologic disorders. Treatment of spastic dysphonia in selected patients is unilateral recurrent laryngeal nerve sectioning. Except for voice change or, in some patients, return of phonatory spasticity, there have been no long-term sequelae or complications of this treatment. We describe three patients with adductor spastic dysphonia who underwent recurrent laryngeal nerve sectioning and who, 3 to 38 months later, suffered respiratory distress that required tracheostomy. The respiratory distress in all three patients was due to episodic jerky vocal cord hyperadductions that caused stridor during inspiration and expiration. These repetitive laryngospasms during respiration and phonation were progressive. Two patients needed an arytenoidectomy to achieve a useful voice, and all three required a permanent tracheostomy to alleviate inspiratory laryngeal obstruction.

Recurrent laryngeal nerve section in the treatment of spastic dysphonia.

Spastic dysphonia is a severe vocal disability in which the patient speaks with hyperadducted vocal folds. The resulting abnormality is characterized by excessively low pitch, vocal tremor, laryngospasms, and strain-strangle voice quality. Until recently the disorder was regarded as psychogenic and treated unsuccessfully with speech therapy and psychotherapy. New evidence supports the theory that the etiology is neurologic, requiring management designed to alter neurophysiologic function. Section of the recurrent laryngeal nerve provides immediate results and a new voice virtually free of the previous unpleasant characteristics.

Recurrent layngeal nerve section for spasmodic dysphonia.

Spasmodic dysphonia is a severe disorder of phonation accompanied by extreme tension of the entire phonatory system. The expressive functions of speech such as laughter, singing, and whispering are much less affected if at all. Psychotherapy, speech therapy, stimulant and psychotropic drugs, hypnotism and acupuncture have all been tried as treatment without success. In 1976, Dedo reported 34 patients who were managed with recurrent laryngeal nerve section for spasmodic dysphonia. All of these patients had marked improvement in voice with relief of spasticity. Twenty-two patients with documented spasmodic dysphonia present for at least one year have been managed at the Cleveland Clinic since Dedo's report. None of them had any improvement with conventional voice therapy and were subjected, therefore, to recurrent laryngeal nerve section.

Laryngeal nerve crush for spastic dysphonia.

Four patients with spastic dysphonia refractory to speech and phychiatric therapy were treated by crushing the recurrent laryngeal nerve. Vocal cord paralysis was produced in all patients. Vocal spasticity subsided in all patients. Vocal cord motion returned in four to six months. Three of four patients remained free of spasticity for a minimum of 24 months.