Rowland Payne Syndrome
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DISEASE ENTITY
Rowland Payne syndrome
Disease
Rowland Payne syndrome (RPS) refers to a triad of Horner syndrome, vocal cord palsy, and hemidiaphragm paralysis due to involvement of the ipsilateral cervical sympathetic plexus, recurrent laryngeal nerve, and phrenic nerve, respectively.[1] This triad is classic for RPS but the complete triad is not necessary for the diagnosis.[2] The main symptoms and signs of RPS include hoarseness, dysphagia, dysaesthesia and pain in the ipsilateral shoulder. The syndrome was named after Dr. Rowland Payne who first described the condition in a report of three cases in 1981.[1]
Epidemiology
RPS is a rare condition with only a few case reports and series published in the literature since its initial description.[2] RPS is typically seen in patients with underlying malignant tumors, but a few reports have described nonmalignant causes (e.g., trauma and infectious lymphadenopathy).[2][3][4] Future epidemiologic studies of incidence and prevalence would be beneficial as awareness and reporting of the syndrome increases.
Etiology
The most commonly reported causes of RPS are neoplasms of the breast and lung but other reported neoplasms include neuroblastoma and anaplastic thyroid carcinoma.[1][2][5][6][7][8] These malignant masses are suggested to compress or infiltrate the mediastinal, thoracic inlet, and/or neck regions. In these instances of underlying malignant lesions, symptoms and signs do not need to be caused by a single mass. Multiple separate lesions can collectively contribute to the clinical picture of Roland Payne syndrome.[5]
Other less commonly reported etiologies include underlying infection, including tuberculous lymphadenitis and empyema thoracis.[4][7] Although the mechanisms are not fully ascertained, infectious lymphadenopathy of the cervical region can often mimic a malignant mass, and extensive inflammation and infection of structures surrounding the nerves (e.g., lung, pleura, first rib, soft tissue) have been proposed mechanisms to explain the atypical manifestations of RPS in these settings.[4][7]
RPS can also occur in the absence of structural lesions. A case of neonatal RPS was reported immediately following forceps-assisted delivery without evidence of structural lesion on imaging.[3] In this case, excessive stretching and manual maneuvering presumably caused a mechanical shearing injury of the nerves located in the lower neck and supraclavicular fossa.[3] Overall, RPS can occur secondary to a variety of underlying etiologies that involve the superficial and deep structures of the neck to upper thoracic anatomic region.
Pathophysiology
The anatomy of the cervical neck and upper thoracic region provides clues to localization of pathology and correlation with clinical presentations in RPS. The recurrent laryngeal nerves are in close proximity to the sympathetic chain and the phrenic nerve at the root.[1] At the level of the sixth cervical vertebra, the sympathetic, vagus, and phrenic nerves are found almost adjacent to each other in a small area posterior to the carotid sheath, anterolateral to the anterior scalene muscle, anteromedial to the jugular chain lymph nodes, and anterior to the fifth cervical nerve.[1] This area is surrounded by the scalene muscles, first rib, and dome of the lung. This anatomical localization is also consistent with the initially described cases of metastatic breast and lung cancers causing infiltrative lesions in these areas visualized on post-mortem evaluation.[1][5]
RPS can occur at other levels than the sixth cervical vertebra.[6] In these cases, the area of pathology is presumed to be located more caudally in the lower neck or thoracic inlet. In cases involving the thoracic inlet, large lesions have been noted to expand to reach the sympathetic, vagus, and phrenic nerves.[6] In a case of mechanical injury to the lower neck, the right laryngeal nerve looping underneath the right subclavian artery, right phrenic nerve crossing the right subclavian artery, and right ansa subclavius of the sympathetic chain also passing under the subclavian artery may be involved.[3] RPS may have a right side predominance due to the anatomic asymmetry of the recurrent laryngeal, phrenic, and sympathetic nerves in relation to the subclavian arteries on the right.[3]
Diagnosis
Symptoms and Signs
The ophthalmic finding in RPS is the Horner syndrome. This typically manifests as unilateral ipsilateral mild ptosis, upside down ptosis anhidrosis, and miosis.[9] The findings may be subtle as lesion of the sympathetically innervated Muller muscle only produces a mild ptosis, the anhidrosis is difficult to notice in temperature-controlled environments, and the miosis is more pronounced in dark (i.e., difficult to appreciate in daily activities).[9] Pharmacological tests using topical apraclonidine and cocaine may be used to confirm the presence of a Horner syndrome.[9] Vocal cord palsy presents with hoarseness of the voice. Patients typically do not manifest symptoms from hemidiaphragm paralysis, but it can be seen on imaging such as chest X-ray.[2][3][5][10]
Other manifestations may include pain, particularly in the ipsilateral shoulder region, dysphagia, and dysaesthesia.[5][6] Depending on the underlying etiology and involved neighboring structures, the syndrome can also present with other concomitant deficits. Involvement of the lower brachial plexus trunks can present with Klumpke palsy, and involvement of the pulmonary superior sulcus can present with Pancoast syndrome.[1]
Related symptoms of the possible underlying etiology are also important in the evaluation. These may include symptoms of malignancy (e.g., weight loss, progressive fatigue), evidence of infection, and history of trauma.
Diagnostic procedures
The preferred imaging of RPS is the same as the Horner imaging protocol encompassing the entire oculosympathetic axis. This might include Magnetic resonance imaging (MRI) brain, cervical spine (including upper thoracic) with Computed tomography angiography (CTA) or / MR angiography (MRA) of the neck, in order to visualize the entire length of the oculosympathetic pathway as well as the surrounding structures that may be involved.[9][11] A flexible laryngoscopy can aid in the evaluation vocal cord paralysis, and X-ray of the chest can be used to visualize hemidiaphragm elevation.[3] Additional testing for underlying etiology, such as biopsy of neoplastic mass, is also warranted.
Differential diagnosis
The differential diagnoses of RPS includes other causes of Horner syndrome, vocal cord palsy, and hemidiaphragm paralysis.
- Iatrogenic injury (e.g., surgical trauma)
- Vascular (e.g., carotid artery thrombosis or dissection, jugular venous ectasia)
- Intracranial pathology (e.g., neoplasm, vascular lesion, malformation, infection)
- Demyelinating disorders (e.g., multiple sclerosis)
- Inherited congenital
- Spinal cord pathology (e.g., myelitis, syringomyelia)
- Cervical disc herniation
- Brachial plexus injury
- Pneumothorax
- Migraine or other headaches
- Herpes zoster
- Raeder paratrigeminal syndrome
- Temporal arteritis
- Iatrogenic injury (e.g., surgical trauma)
- Vascular (e.g., carotid artery thrombosis or dissection, jugular venous ectasia)
- Intracranial pathology (e.g., neoplasm, vascular lesion, malformation, infection)
- Demyelinating disorders (e.g., multiple sclerosis)
- Inherited congenital
- Spinal cord pathology (e.g., myelitis, syringomyelia)
- Cervical disc herniation
- Brachial plexus injury
- Pneumothorax
- Migraine or other headaches
- Herpes zoster
- Raeder paratrigeminal syndrome
- Temporal arteritis
Hemidiaphragm paralysis:[10]
- Iatrogenic injury (e.g., surgical trauma)
- Vascular (e.g., carotid artery thrombosis or dissection, jugular venous ectasia)
- Intracranial pathology (e.g., neoplasm, vascular lesion, malformation, infection)
- Demyelinating disorders (e.g., multiple sclerosis)
- Inherited congenital
- Spinal cord pathology (e.g., myelitis, syringomyelia)
- Cervical disc herniation
- Brachial plexus injury
- Pneumothorax
- Migraine or other headaches
- Herpes zoster
- Raeder paratrigeminal syndrome
- Temporal arteritis
MANAGEMENT
Treatment
Definitive treatment of RPS involves targeted therapy of the underlying pathology.
Prognosis
The prognosis of RPS depends on the underlying etiology.
SUMMARY
RPS consists of a triad of ipsilateral Horner syndrome, vocal cord palsy, and hemidiaphragm paralysis. Imaging of the entire oculosympathetic pathway is necessary to exclude structural causes of RPS. The management and prognosis for RPS depend upon the underlying etiology (e.g., neoplasm). Clinicians should be aware of RPS and the localizing value of the clinical triad.
REFERENCES
- ↑ 1.0 1.1 1.2 1.3 1.4 1.5 1.6 Payne CM. Newly recognized syndrome in the neck: Horner's syndrome with ipsilateral vocal cord and phrenic nerve palsies. J R Soc Med. Nov 1981;74(11):814-8.
- ↑ 2.0 2.1 2.2 2.3 2.4 Sierra-Hidalgo F, Aragón Revilla E. Rowland payne syndrome. Neurologia (Engl Ed). Mar 12 2021;Síndrome de Rowland Payne. doi:10.1016/j.nrl.2021.02.001
- ↑ 3.0 3.1 3.2 3.3 3.4 3.5 3.6 Murone A-JB, Kawasaki A. Rowland Payne syndrome in a neonate as a consequence of birth trauma. Case Reports in Perinatal Medicine. 2015;4(1):77-78. doi:doi:10.1515/crpm-2014-0011
- ↑ 4.0 4.1 4.2 Bhaskar G, Lodha R, Kabra SK. Unusual complications of empyema thoracis: Diaphragmatic palsy and Horner's syndrome. The Indian Journal of Pediatrics. 2006/10/01 2006;73(10):941-943. doi:10.1007/BF02859293
- ↑ 5.0 5.1 5.2 5.3 5.4 Amin R. Horner's syndrome with ipsilateral vocal cord and phrenic nerve palsies. Postgrad Med J. Feb 1984;60(700):140-2. doi:10.1136/pgmj.60.700.140
- ↑ 6.0 6.1 6.2 6.3 Vaghadia H, Spittle M. Newly recognized syndrome in the neck. J R Soc Med. Sep 1983;76(9):799. doi:10.1177/014107688307600924
- ↑ 7.0 7.1 7.2 Ismail MH, Hodkinson HJ, Tuling B. Horner's syndrome and tuberculosis. A case report. S Afr Med J. Dec 3 1988;74(11):586-7.
- ↑ Kapoor V, Lodha R, Agarwala S. Superior mediastinal syndrome with Rowland-Payne syndrome: an unusual presentation of cervico-mediastinal neuroblastoma. Pediatr Blood Cancer. Mar 2005;44(3):280-2. doi:10.1002/pbc.20198
- ↑ 9.0 9.1 9.2 9.3 Maamouri R, Ferchichi M, Houmane Y, Gharbi Z, Cheour M. Neuro-Ophthalmological Manifestations of Horner's Syndrome: Current Perspectives. Eye Brain. 2023;15:91-100. doi:10.2147/eb.S389630
- ↑ 10.0 10.1 Dubé BP, Dres M. Diaphragm Dysfunction: Diagnostic Approaches and Management Strategies. J Clin Med. Dec 5 2016;5(12)doi:10.3390/jcm5120113
- ↑ Davagnanam I, Fraser CL, Miszkiel K, Daniel CS, Plant GT. Adult Horner's syndrome: a combined clinical, pharmacological, and imaging algorithm. Eye (Lond). Mar 2013;27(3):291-8. doi:10.1038/eye.2012.281
- ↑ Khan Z, Bollu PC. Horner Syndrome. StatPearls. StatPearls Publishing. Copyright © 2023, StatPearls Publishing LLC.; 2023.
- ↑ Martin TJ. Horner Syndrome: A Clinical Review. ACS Chem Neurosci. Feb 21 2018;9(2):177-186. doi:10.1021/acschemneuro.7b00405
- ↑ Seyed Toutounchi SJ, Eydi M, Golzari SE, Ghaffari MR, Parvizian N. Vocal cord paralysis and its etiologies: a prospective study. J Cardiovasc Thorac Res. 2014;6(1):47-50. doi:10.5681/jcvtr.2014.009
- ↑ Williamson AJ, Shermetaro C. Unilateral Vocal Cord Paralysis. [Updated 2022 Aug 19]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2023 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK535420/.
