Endgames Case Report

Hoarseness in a 79 year old woman

BMJ 2013; 346 doi: http://dx.doi.org/10.1136/bmj.f2305 (Published 25 April 2013) Cite this as: BMJ 2013;346:f2305
  1. Thomas Jacques, core surgical trainee,
  2. Tarik Abed, otolaryngology registrar,
  3. Sunil Sharma, otolaryngology registrar,
  4. Jonathan Philpott, consultant head and neck surgeon
  1. 1Department of Otolaryngology, Southend University Hospital, Westcliff on Sea SS0 0RY, UK
  1. Correspondence to: T Jacques Thomas.Jacques{at}southend.nhs.uk

A 79 year old woman was referred to our ear, nose, and throat outpatient clinic with a history of hoarse voice. This symptom had been present for around three months. It initially fluctuated in severity but eventually became constant. There was no associated pain, weight loss, cough, dysphagia, odynophagia, or other upper airway symptoms. She had not recently had surgery or experienced trauma. Her medical history was of chronic obstructive pulmonary disease, and she was an ex-smoker.

There were no abnormalities on head and neck examination. Flexible laryngoscopy showed no mucosal lesions in the nasopharynx, oropharynx, or hypopharynx. The left vocal cord was normal in appearance but fixed in the paramedian position. Contrast enhanced computed tomography from the skull base to the diaphragm was performed. This showed a large saccular aneurysm of the distal part of the aortic arch, near the origin of the left subclavian artery, measuring 4.5 cm in diameter (figure).

Figure1

Sagittal contrast enhanced computed tomogram showing a saccular aneurysm of the distal aortic arch, measuring 4.5 cm in diameter

Questions

  • 1 What is the differential diagnosis of hoarseness?

  • 2 How did the abnormality seen on the computed tomogram lead to the patient’s symptoms?

  • 3 How can the problems be managed?

Answers

1 What is the differential diagnosis of hoarseness?

Short answer

Hoarseness (dysphonia) can be caused by infections such as viral laryngitis, benign laryngeal lesions, laryngeal cancer, vocal cord paralysis, gastro-oesophageal reflux disease, neurological disease, hypothyroidism, and functional voice disorders.

Long answer

Hoarseness (dysphonia) may be acute or chronic (lasting more than three weeks) and has a wide variety of causes. The most common acute cause is localised infection, usually self limiting laryngitis in association with a viral upper respiratory tract infection. It may also be present in tonsillitis, candidiasis, or less commonly in life threatening epiglottitis or supraglottitis. Patients with hoarseness that persists for more than three weeks, or that is present intermittently for more than 12 weeks despite treatment, must be assessed urgently (under the two week wait pathway) with laryngoscopy to rule out cancer.

Any mass or lesion arising on or near the vocal cord may lead to a hoarse voice through interruption of the normal mucosal wave that leads to voice production.1 Benign laryngeal lesions include vocal cord polyps, which are typically unilateral, and nodules, which are bilateral. Both phenomena are often related to voice abuse in young children, singers, or other patients who have to speak loudly, such as teachers. Benign mucous retention cysts are also common and lead to a breathy voice. Reinke’s oedema refers to a baggy sac-like swelling of the vocal cord, which leads to a deep and husky voice. This is usually seen in smokers and patients with gastro-oesophageal reflux disease or hypothyroidism. Benign papillomas may cause recurrent hoarseness in adults and children and are associated with infection with human papillomavirus subtypes 6 and 11.2

Laryngeal cancer is most commonly squamous cell carcinoma, which is strongly associated with smoking and excessive alcohol intake.3 It often presents with persistent hoarseness due to direct involvement or fixation of the vocal cord. It may also present with persistent cough, systemic symptoms of cancer, cervical lymphadenopathy, or referred otalgia.

Vocal cord paralysis leads to hoarseness and can sometimes cause aspiration pneumonia because of the loss of airway protection. It is caused by disease of the recurrent laryngeal nerve, which supplies most of the intrinsic muscles of the larynx. It is often idiopathic but may be caused by a lesion at any point in the nerve’s anatomical course. Systemic neurological diseases such as stroke, multiple sclerosis, and Parkinson’s disease are also potential causes of vocal cord paralysis and hoarseness, although other features are usually present.

Hypothyroidism and gastro-oesophageal reflux disease are commonly associated with hoarseness and may lead to visible changes in the larynx, although medical treatment is not always effective.4

2 How did the abnormality seen on the computed tomogram lead to the patient’s symptoms?

Short answer

The formation of an aneurysm (or pseudoaneurysm) of the aortic arch caused injury to the left recurrent laryngeal nerve, leading to vocal cord paralysis. This nerve passes around the aortic arch before ascending in the tracheo-oesophageal groove to supply the intrinsic muscles of the larynx.

Long answer

The recurrent laryngeal nerve is a branch of the vagus nerve (X) and supplies all of the intrinsic muscles of the larynx, with the exception of the cricothyroid muscle, which lengthens the vocal cords and is supplied by the external branch of the superior laryngeal nerve. The course of the recurrent laryngeal nerve is circuitous and varies between sides. The right recurrent laryngeal nerve is shorter; after arising from the right vagus at the level of T1-2, it passes posteriorly around the right subclavian artery, before ascending in the tracheo-oesophageal groove towards the larynx. In 0.5-1% of the general population the right nerve may be non-recurrent, passing medially from the vagus to the larynx. The left recurrent laryngeal nerve arises from the vagus nerve in the thorax and winds around the aortic arch, posterior to the ligamentum arteriosum, before passing superiorly towards the larynx.5 Both nerves are intimately related to the lung apices, the oesophagus, and the posteromedial aspect of the thyroid lobes. They enter the larynx at the level of the cricothyroid joint.

Patients with recurrent laryngeal nerve palsy with no apparent cause—such as proximity to thyroid surgery—are typically investigated with a computed tomogram that encompasses the full course of the nerve.6 Magnetic resonance imaging may also be performed, particularly if disease of the skull base is suspected or if an initial computed tomogram shows no abnormality.

A lesion at any point in the course of the recurrent laryngeal nerve may result in vocal cord paralysis. Recurrent laryngeal nerve palsy may be congenital (presenting in neonatal life with a weak cry or stridor if bilateral) or acquired. The condition is idiopathic in around a third of cases. About a third of cases are caused by trauma during surgery, most commonly to the thyroid gland7; the nerve is also vulnerable during carotid endarterectomy and other procedures involving the carotid sheath or the cervical spine. Around a third of such palsies are caused by cancer, most often of the lung: recurrent laryngeal nerve palsy due to lymph node infiltration at the lung hilum is more common than the well known apical Pancoast’s tumour. Other malignant causes include oesophageal cancer and thyroid cancer. The nerve may also be impinged by tumours of the skull base or nasopharynx, near its exit through the jugular foramen. A minority of cases are caused by vascular lesions or tuberculosis. Investigation in cases of new onset vocal cord paralysis is therefore primarily aimed at excluding cancer.

Risk factors for aneurysm of the aortic arch are similar to those for aortic aneurysm elsewhere: age, hypertension, atherosclerosis, and, more rarely, connective tissue disorders such as Marfan’s syndrome and infections such as syphilis. Pseudoaneurysm may result from trauma.8

In this case, the development of the aneurysm probably impinged on the left recurrent laryngeal nerve as it passes around the aortic arch in the thorax. This would result in a traction neuropraxia, and over time, complete paralysis of the left vocal cord.

3 How can the problems be managed?

Short answer

Aortic aneurysm may be managed conservatively or with endovascular, open, or hybrid repair. The symptoms of vocal cord palsy may improve with time owing to compensation from the contralateral vocal cord; this process can be aided by speech therapy. Injections or surgical techniques are used to medialise the affected vocal cord when compensation is not sufficient.

Long answer

The patient’s aortic arch aneurysm presents a risk of growth and rupture. Management of an aortic aneurysm must take into account the patient’s wishes and overall health status. Patients can be managed conservatively (with serial imaging) or with endovascular, open, or hybrid repair.9 Management of the aortic arch aneurysm in itself would be unlikely to improve the patient’s presenting symptom of hoarseness.

Hoarseness, dysphagia, and aspiration due to recurrent laryngeal nerve palsy may improve spontaneously over time because of compensation from the contralateral vocal cord, which medialises gradually to close the glottic gap. Patients are typically referred for speech and language therapy to help with this process and to provide the patient with strategies to improve voice quality and swallowing safety.10

Patients who do not improve over a period of active monitoring and speech therapy (typically three months) may benefit from a procedure to move the paralysed vocal cord towards the midline, allowing the cords to meet during phonation and swallowing. This may be performed under local or general anaesthesia, either by a transoral endoscopic technique or an external approach. Materials such as collagen, bioplastic, Teflon, or fat may be injected into the vocal fold; this is most often performed under general anaesthesia and may require revision.11 Laryngeal framework surgery (Isshiki type 1 thyroplasty) allows medialisation of a paralysed cord in an awake patient; this permits real time assessment of the voice and has longer lasting results.12

Patient outcome

Our patient remained well and was referred to a consultant vascular surgeon. An endovascular repair of her aortic arch aneurysm is planned. Speech and language therapy is ongoing.

Notes

Cite this as: BMJ 2013;346:f2305

Footnotes

  • Competing interests: All authors have completed the ICMJE uniform disclosure form at www.icmje.org/coi_disclosure.pdf (available on request from the corresponding author) and declare: no support from any organisation for the submitted work; no financial relationships with any organisations that might have an interest in the submitted work in the previous three years; no other relationships or activities that could appear to have influenced the submitted work.

  • Provenance and peer review: Not commissioned; externally peer reviewed.

  • Patient consent obtained.

References