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Rowland Payne Syndrome – Diagnosing A Rare Clinical Triad with Major Implications

Rowland Payne Syndrome | 1 February 2025

Rowland Payne Syndrome (RPS) is a rare but clinically significant neurological syndrome that presents as a distinct triad of nerve dysfunction. Though uncommon, its recognition is critical because it frequently points to serious underlying conditions, including malignancies in the neck or chest.

Named after Dr. Rowland Payne, who first described the syndrome in 1981, RPS consists of three hallmark findings—Horner syndrome, vocal cord paralysis, and hemidiaphragm paralysis—all on the same side of the body. Understanding and diagnosing RPS early is crucial in directing investigations toward potentially life-threatening causes.

In this post, we’ll explore how clinicians identify RPS, differentiate it from other conditions, and utilize modern diagnostic tools to confirm the syndrome and uncover its etiology.

Overview: What is Rowland Payne Syndrome?

Rowland Payne Syndrome refers to a neuroanatomical triad involving:

  1. Horner syndrome – damage to the cervical sympathetic chain.

  2. Vocal cord palsy – injury to the recurrent laryngeal nerve.

  3. Hemidiaphragm paralysis – dysfunction of the phrenic nerve.

All three nerves course through the lower neck and thoracic inlet, a compact anatomical region vulnerable to compressive, infiltrative, infectious, or traumatic lesions. RPS is not a primary disease but a clinical signpost indicating a deeper pathological process.

When to Suspect Rowland Payne Syndrome

Clinical Clues

Because each of the three components may present subtly or even asymptomatically, a high index of suspicion is needed. The diagnosis typically begins with a constellation of symptoms, which may include:

  • Drooping eyelid and small pupil on one side (Horner syndrome)

  • Hoarseness or breathy voice (vocal cord palsy)

  • Unexplained shortness of breath, especially lying down (diaphragm paralysis)

  • Shoulder or neck pain, sometimes radiating

Often, patients may report only one or two of these symptoms. It is up to the clinician to piece them together. A thorough neurological and head and neck exam is the first step in uncovering the triad.

Step-by-Step Diagnostic Approach

1. History and Physical Examination

A detailed history provides important context:

  • Recent surgery or trauma? Especially to the neck or chest?

  • History of cancer? Especially breast, lung, or thyroid malignancy?

  • Systemic symptoms? Like weight loss, night sweats, or fever?

The physical exam should look for:

  • Ptosis, miosis, and anhidrosis (Horner syndrome)

  • Vocal changes or weak cough

  • Asymmetric chest movement or diminished breath sounds (suggestive of diaphragm weakness)

Subtle or chronic signs may be missed without careful inspection. A focused cranial nerve and respiratory exam can be invaluable.

2. Confirming Horner Syndrome

Horner syndrome is often the first diagnostic clue in RPS. Classic signs include:

  • Mild ptosis (drooping of the upper eyelid)

  • Miosis (constricted pupil)

  • Anhidrosis (lack of sweating) on the same side of the face

Pharmacologic testing can help confirm Horner syndrome if findings are subtle:

  • Apraclonidine eye drops reverse ptosis and miosis in affected eyes due to denervation hypersensitivity.

  • Cocaine drops fail to dilate the affected pupil in Horner syndrome.

These simple eye drop tests can be performed in an ophthalmology or neurology clinic and offer high diagnostic value.

3. Vocal Cord Evaluation

When vocal changes such as hoarseness or a weak voice are present, clinicians will often order:

  • Flexible laryngoscopy – a small camera passed through the nose or mouth to visualize vocal cord movement directly.

  • Findings may show unilateral paralysis or reduced motion of one vocal fold.

Recurrent laryngeal nerve palsy is often painless, and vocal symptoms may go unreported unless specifically asked about. Therefore, vocal cord examination is essential when RPS is suspected.

4. Assessing Diaphragm Function

Diaphragm paralysis may not cause obvious symptoms unless severe or bilateral. Common signs include:

  • Dyspnea (especially when lying flat)

  • Reduced breath sounds at lung bases

Diagnostic tools include:

  • Chest X-ray – may show elevation of the affected hemidiaphragm.

  • Fluoroscopy (“sniff test”) – used to observe paradoxical diaphragm motion.

  • Ultrasound – a noninvasive way to assess diaphragm movement.

  • Pulmonary function testing – may reveal reduced lung capacity in supine position.

If phrenic nerve involvement is suspected, these modalities help confirm the final piece of the RPS triad.

5. Imaging Studies: Finding the Cause

Once RPS is clinically suspected, imaging is the cornerstone of diagnosing the underlying cause. The goal is to evaluate the entire oculosympathetic pathway from brain to chest and identify structural lesions compressing or infiltrating key nerves.

Key imaging modalities include:

a. MRI of Brain, Neck, and Cervical Spine

  • To assess for lesions along the central sympathetic chain.

  • Rules out central causes of Horner syndrome (e.g., brainstem stroke, tumor).

b. CT or MRI of the Neck and Thoracic Inlet

  • Looks for masses, lymphadenopathy, or abscesses compressing the cervical sympathetic chain, recurrent laryngeal, or phrenic nerves.

  • Often reveals tumors of the thyroid, lung apex, or mediastinum.

c. Chest Imaging (X-ray, CT Chest)

  • Identifies apical lung tumors (e.g., Pancoast tumor), elevated hemidiaphragm, or lymph node enlargement.

  • May show tracheal deviation or loss of lung volume on the affected side.

In many cases, cross-sectional imaging (CT or MRI with contrast) provides definitive evidence of the lesion causing the triad.

6. Additional Diagnostic Tests

Depending on the findings, further evaluations may be needed:

  • Fine-needle aspiration (FNA) or biopsy of a mass for histologic diagnosis

  • PET scan to identify metastasis in suspected malignancy

  • Bloodwork if infection or systemic illness is suspected

  • Electromyography (EMG) of the diaphragm in uncertain or subclinical cases

Differential Diagnosis

Each element of the RPS triad has its own differential diagnosis. When all three are present, the focus narrows, but clinicians must rule out:

  • Carotid dissection – can mimic Horner syndrome

  • Thyroid surgery complications – can injure recurrent laryngeal or phrenic nerves

  • Central lesions (e.g., brainstem stroke, syringomyelia) – for isolated nerve palsies

  • Guillain-Barré Syndrome – for bilateral diaphragm paralysis

  • Neuroinfectious conditions – such as tuberculosis or viral neuropathy

Ultimately, the full triad together remains highly suggestive of a lesion near the thoracic inlet or lower neck, most often a mass or tumor.

Case-Based Example

A 71-year-old woman presents with mild ptosis, shoulder pain, and hoarseness two weeks after abdominal surgery. Imaging reveals an elevated left diaphragm and a mass in the left neck compressing the carotid artery and invading surrounding tissues.

A biopsy confirms anaplastic thyroid carcinoma—a classic etiology of Rowland Payne Syndrome. The diagnosis allowed for rapid treatment initiation and appropriate palliative care planning.

This case illustrates the importance of a thorough diagnostic approach in patients with non-specific yet subtle findings.

Final Thoughts: Why Early Diagnosis Matters

Rowland Payne Syndrome may be rare, but its diagnostic value is immense. When recognized early, RPS can prompt timely imaging, uncovering malignancies, infections, or injuries that would otherwise progress unnoticed.

Key takeaways for clinicians:

  • Always correlate Horner syndrome, voice changes, and diaphragm findings—don’t evaluate them in isolation.

  • Utilize flexible laryngoscopy, chest imaging, and pharmacologic pupil tests to confirm each component.

  • Follow up with cross-sectional imaging to locate the lesion and plan management.

For patients, unexplained changes in voice, breathing, or eyelid function warrant prompt evaluation—these seemingly unrelated symptoms may be telling a much larger story.

Do you or someone you know experience persistent hoarseness, eyelid drooping, or breathing difficulty?
Consult with your healthcare provider. Early recognition of patterns like RPS can be life-saving.