Rowland Payne Syndrome: Signs, Symptoms and Clinical Relevance

Rowland Payne Syndrome | 2 May 2025

In the landscape of rare neurological syndromes, Rowland Payne Syndrome (RPS) stands out as a triad of clinical signs that often point to a more serious underlying condition. Though infrequent in occurrence, recognizing this constellation of symptoms can provide clinicians with critical clues that influence both diagnostic and therapeutic decisions.

Named after Dr. Rowland Payne, who first described the syndrome in 1981, RPS involves the simultaneous impairment of three nerves: the cervical sympathetic chain, the recurrent laryngeal nerve, and the phrenic nerve. The result is a characteristic trio of:

Horner Syndrome
Vocal cord palsy
Hemidiaphragm paralysis

While each of these signs can occur independently in various clinical contexts, their coexistence—especially when ipsilateral—should prompt further investigation into possible compressive or infiltrative lesions in the neck or thoracic inlet.

What is Rowland Payne Syndrome?

Rowland Payne Syndrome is a neuroanatomical syndrome defined by dysfunction of three specific nerve structures, typically on the same side of the body:

Horner Syndrome indicates involvement of the cervical sympathetic pathway.
Vocal cord palsy suggests recurrent laryngeal nerve compromise.
Hemidiaphragm paralysis reflects impairment of the phrenic nerve.

This syndrome is rare but often significant in that it points to either malignant tumors or inflammatory lesions in the cervicothoracic region—especially where these three nerves are anatomically close together.

Anatomy Behind the Syndrome

The pathophysiology of RPS is best understood through the anatomical proximity of the involved nerves:

The cervical sympathetic chain, which controls pupil dilation and eyelid elevation, runs near the vertebral bodies and carotid sheath.
The recurrent laryngeal nerve, a branch of the vagus nerve, loops around the subclavian artery on the right and aortic arch on the left, ascending back to innervate the vocal cords.
The phrenic nerve travels along the anterior scalene muscle, descending into the thoracic cavity to supply the diaphragm.

At the level of the thoracic inlet, particularly around the C6–T1 vertebrae, these nerves are closely clustered. A lesion in this region can simultaneously affect all three, leading to the full clinical picture of Rowland Payne Syndrome.

Signs and Symptoms

1. Horner Syndrome

This component is often the most recognizable, yet the most subtle:

Ptosis (drooping of the upper eyelid)
Miosis (constriction of the pupil)
Anhidrosis (loss of sweating on the affected side of the face)
Occasionally, enophthalmos (sunken appearance of the eye)

Because Horner syndrome can be difficult to appreciate in well-lit environments or in individuals with mild symptoms, pharmacological testing (such as with apraclonidine or cocaine drops) is sometimes used to confirm the diagnosis.

2. Vocal Cord Palsy

Involvement of the recurrent laryngeal nerve results in:

Hoarseness or breathy voice
Weak or ineffective cough
Voice fatigue with speaking
Aspiration, in severe cases

Flexible laryngoscopy can be used to visualize impaired vocal cord movement, often confirming unilateral paralysis.

3. Hemidiaphragm Paralysis

Phrenic nerve dysfunction may be silent, especially if the patient has good pulmonary reserve, but signs can include:

Shortness of breath, particularly when lying flat
Paradoxical movement of the diaphragm on fluoroscopy
Elevated hemidiaphragm on chest X-ray

This component is often discovered incidentally on imaging, unless the dysfunction is bilateral or severe.

Associated Symptoms

Because RPS often arises from deeper pathologies, other symptoms may accompany the core triad:

Ipsilateral shoulder or neck pain (suggesting local tumor or inflammatory infiltration)
Weight loss or fatigue (in systemic illnesses like cancer or infection)
Neurological deficits such as numbness or weakness in the arm (when neighboring brachial plexus structures are involved)

Common Causes of Rowland Payne Syndrome

Although rare, RPS is most often associated with underlying malignancies, especially:

Breast cancer
Lung cancer (particularly Pancoast tumors at the lung apex)
Thyroid carcinoma, especially anaplastic variants
Neuroblastoma in pediatric populations

Non-malignant causes include:

Infectious lymphadenitis (e.g., tuberculosis)
Trauma or birth injury in neonates
Iatrogenic injury, such as from interscalene nerve blocks or neck surgery

Notably, in infants and children, mechanical stretching during forceps-assisted delivery has been implicated in rare neonatal cases.

Diagnosis

Clinical Evaluation

A thorough history and physical exam remain the cornerstone:

Ask about recent trauma, surgery, or malignancy history.
Observe for ptosis, pupil asymmetry, hoarseness, and breathing difficulty.

Imaging

To investigate the underlying cause and localize the lesion:

MRI and/or CT of the brain, neck, and thorax
Chest X-ray to assess diaphragm position
Neck ultrasound or CT angiography, especially when evaluating masses

These modalities help assess whether a structural lesion is compressing the nerves and guide any biopsy or surgical intervention.

Additional Testing

Flexible laryngoscopy for vocal cord evaluation
Pharmacologic pupil testing for Horner confirmation
Electromyography (EMG) of the diaphragm in uncertain cases

Differential Diagnosis

Because each component of the triad can exist in isolation, it’s critical to distinguish RPS from other conditions:

Horner Syndrome alone can result from carotid dissection, cluster headaches, or brainstem stroke.
Vocal cord paralysis may follow thyroid surgery, viral infections, or vagal nerve injury.
Phrenic nerve palsy may occur with trauma, spinal cord disease, or viral neuritis.

However, when all three components occur together, particularly on the same side, RPS should be high on the list of considerations.

Management and Prognosis

Treatment

Management focuses on the underlying cause:

Malignant tumors may require surgery, chemotherapy, or radiation.
Infectious etiologies are treated with appropriate antimicrobial therapy.
Traumatic cases may resolve spontaneously or benefit from nerve rehabilitation strategies.

Supportive Care

Speech therapy for voice rehabilitation
Breathing exercises or CPAP in cases of diaphragm weakness
Surgical interventions, like vocal cord medialization or diaphragm pacing, in severe cases

Prognosis

The outlook for Rowland Payne Syndrome largely depends on its underlying etiology. For malignancy-related RPS, the syndrome may reflect advanced local invasion, often indicating a poorer prognosis. However, benign or traumatic cases can improve significantly with time and appropriate therapy.

Clinical Case in Context

Consider a patient presenting with:

Left eyelid drooping and constricted pupil
New onset hoarseness
Chest imaging showing elevated left hemidiaphragm

Such a constellation should prompt imaging of the neck and thoracic inlet. In one case, a thyroid mass compressing the carotid artery and surrounding nerves was discovered to be anaplastic thyroid carcinoma, exemplifying how RPS can serve as an early warning system for serious disease.

Final Thoughts

Rowland Payne Syndrome may be rare, but its recognition is clinically powerful. The convergence of Horner syndrome, vocal cord paralysis, and hemidiaphragm dysfunction should alert clinicians to the possibility of a compressive lesion in the lower neck or upper chest.

Early diagnosis not only aids in symptom management but can also uncover life-threatening pathologies like cancer. Awareness of this triad—and the anatomical relationships underpinning it—equips healthcare providers to take swift, targeted action.

Have you encountered symptoms that could suggest a neurological or compressive syndrome?
Reach out to your healthcare provider or a neurologist for further evaluation. Early recognition can make all the difference with Rowland Payne Syndrome.

Rowland Payne Syndrome