Centurion Syndrome

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Disease Entity

Centurion syndrome is a rare developmental anomaly of the medial canthal region characterized by persistent epiphora despite a patent lacrimal drainage system. The condition results from abnormal medial eyelid anatomy, leading to anterior displacement of the lacrimal puncta away from the lacus lacrimalis and subsequent impairment of tear drainage. Patients typically present during adolescence or early adulthood with chronic unilateral or bilateral tearing and characteristic craniofacial features.¹

Disease

First described by Sullivan et al. in 1993, Centurion syndrome derives its name from the resemblance of affected individuals to Roman centurions, who were traditionally depicted with a prominent nasal bridge.¹,2 The condition is frequently underrecognized and may be mistaken for nasolacrimal duct obstruction, functional epiphora, or punctal disorders. Awareness of this entity is important because the lacrimal drainage apparatus is usually anatomically patent, and simple surgical correction of the medial canthal abnormality often results in complete resolution of symptoms.¹⁻⁴

Etiology

The exact etiology remains incompletely understood.¹ Initially, Centurion syndrome was attributed to abnormal anterior insertion of the anterior limb of the medial canthal tendon, resulting in anterior displacement of the medial eyelid and puncta.¹However, magnetic resonance imaging studies by Alam et al. demonstrated that anterior insertion of the MCT is not universally present. Some patients exhibit normal insertion, while others demonstrate developmental abnormalities of the tendon, suggesting a more complex pathogenesis.³

Associated facial features frequently include:

  • Prominent nasal bridge
  • Deep-set eyes
  • Synophrys
  • Sharp medial canthal angulation

These findings support a developmental craniofacial basis for the disorder.¹,³

Epidemiology

Centurion syndrome is an uncommon disorder with relatively few cases reported in the literature. The true prevalence remains unknown owing to underdiagnosis and misclassification. Most reported patients are post-pubertal males, although the condition may occur in either sex.¹,⁴

The condition is typically bilateral but may be asymmetric. Symptoms usually become more apparent during adolescence when facial skeletal development accentuates the underlying anatomical abnormality.¹

Relevant Anatomy

The medial canthal tendon (MCT) is a key structure responsible for maintaining normal eyelid position and facilitating lacrimal drainage.

The MCT divides into:

  • Anterior limb
  • Posterior limb

The anterior limb itself consists of two lamellae:

  1. The anterior lamella, formed by the tendon of the pretarsal orbicularis muscle.
  2. The posterior lamella, formed by musculotendinous fibers from the preseptal and orbital orbicularis muscle.³

The normal relationship between the puncta, medial canthus, and lacus lacrimalis allows efficient tear drainage through the lacrimal pump mechanism. Any alteration in this anatomical arrangement can impair tear entry into the drainage system despite normal canaliculi, sac, and nasolacrimal duct anatomy.

Pathophysiology

The hallmark abnormality is anterior displacement of the medial eyelid from the globe.

As a result:

Prominent nasal bridge and/or medial canthal tendon abnormality

Anterior rotation of the medial eyelid

Punctum displaced away from the lacus lacrimalis

Failure of effective tear entry

Functional obstruction despite a patent drainage system

Constant epiphora

The lacrimal drainage system itself remains anatomically patent, explaining the normal findings on syringing.¹,⁴

Murthy et al. described a characteristic sharp inferior angulation of the medial canthal tendon, termed the "beak sign," which may contribute to punctal malposition and tear drainage dysfunction.⁴

Diagnosis

Centurion syndrome

History

Important historical features include:

  • Adolescent or young adult onset
  • Chronic bilateral tearing
  • Lack of recurrent infections
  • Previous unsuccessful treatment with lubricants, antibiotics, or antihistamines

Physical Examination

Particular attention should be paid to:

  • Medial canthal anatomy
  • Punctal position
  • Nasal bridge prominence
  • Facial morphology

Observation from above often demonstrates anterior displacement of the puncta away from the lacus lacrimalis.

Symptoms

The condition most frequently affects post-pubertal males and typically presents with bilateral involvement, although unilateral cases have been described and asymmetry between the two eyes is not uncommon.¹˒⁴ Patients typically present with constant, longstanding epiphora that persists despite repeated medical treatment.

Unlike infective lacrimal disorders, discharge, pain, redness, and recurrent dacryocystitis are typically absent.

Signs

Characteristic examination findings include:

Facial Features

  • Prominent nasal bridge
  • Deep-set eyes
  • Synophrys
  • Sharp medial canthal contour

Eyelid Findings

  • Medial ectropion-like appearance
  • Anterior displacement of puncta
  • Medial eyelid not apposed to globe
  • Loss of normal punctal position within tear lake

Lacrimal Findings

  • Increased tear meniscus height
  • Positive fluorescein dye disappearance test (FDDT)
  • Patent syringing
  • Negative ROPLAS
  • Normal punctal openings

These findings are highly suggestive of Centurion syndrome.¹,⁴

Diagnostic Procedures

Fluorescein Dye Disappearance Test

FDDT is typically positive because tears fail to enter the drainage system efficiently despite anatomical patency.¹

Lacrimal Syringing

Syringing usually demonstrates free flow into the nose suggestive of patent drainage apparatus. This finding helps distinguish Centurion syndrome from nasolacrimal duct obstruction.

Imaging

Imaging is not routinely required. Alam et al. demonstrated that MRI can evaluate:

  • Medial canthal tendon anatomy
  • Tendon insertion
  • Relationship between tendon and surrounding soft tissues

MRI findings challenge the traditional theory that all cases result from anterior tendon insertion.³

Differential Diagnosis

Differential Diagnosis

Congenital Nasolacrimal Duct Obstruction [Nasolacrimal Duct Obstruction, Congenital]],

Feature Centurion Syndrome Congenital NLDO
Age Adolescents Infants
Syringing Patent Obstructed
Punctal position Abnormal Normal
Facial features Present Absent
FDDT Positive Positive

Punctal Stenosis

Punctal stenosis presents with narrowing or occlusion of the punctal opening, whereas punctal size is usually normal in Centurion syndrome.⁶ [[Punctal Stenosis]]

Punctal atresia

Congenital absence of imperforate punctum with epiphora since birth.

Canaliculitis

Features favoring canaliculitis include:

  • Pouting punctum
  • Expressible concretions
  • Discharge
  • Chronic unilateral conjunctivitis

These findings are absent in Centurion syndrome.⁷

Chronic Dacryocystitis

Unlike Centurion syndrome:

  • ROPLAS may be positive
  • Syringing may show obstruction
  • Recurrent infection is common

Medial Ectropion

Medial ectropion generally affects older patients and is associated with eyelid laxity rather than characteristic craniofacial abnormalities.⁸

Telecanthus:

Increased intercanthal distance without punctal malposition or functional epiphora. [[Telecanthus ]]

Management

Medical therapy is generally ineffective because the underlying problem is anatomical.¹

Patients often receive multiple courses of topical medications before the correct diagnosis is established.

Surgery

The goal of surgery is restoration of normal punctal position and apposition to the tear lake.

Medial Canthal Tendon Release

This remains the most commonly reported procedure.¹,⁴The procedure releases abnormal tension exerted by the medial canthal tendon, allowing the medial eyelid to reposition closer to the globe.

Reported outcomes are generally excellent with significant reduction or complete resolution of epiphora.⁴

Posterior Plication of MCT Stump- Posterior repositioning may improve punctal alignment in selected cases.⁴

Medial Conjunctivoplasty- Can be performed when conjunctival factors contribute to punctal malposition.⁴

Lower Lid Retractor Plication- May be useful in selected patients with associated lower eyelid malposition.⁴

Punctoplasty- Reserved for cases with concurrent punctal abnormalities.

Prognosis

Published reports demonstrate favorable outcomes following surgical correction.¹,⁴Murthy et al. reported significant symptomatic improvement following correction of medial canthal tendon abnormalities.⁴ The prognosis is excellent when the diagnosis is recognized and appropriate surgical intervention is performed. Most patients experience substantial improvement in symptoms and quality of life.¹,⁴

Failure to recognize the condition may lead to unnecessary investigations and lacrimal drainage procedures.

References

  1. Sullivan TJ, Welham RA, Collin JR. Centurion syndrome. Ophthalmology. 1993;100:328-333.
  2. Kumar GS. Centurion syndrome. Int J Sci Res. 2015;4:89.
  3. Alam MS, Tongbram A, Noronha OV. Magnetic resonance imaging features of medial canthal tendon in centurion syndrome. Curr Eye Res. 2021;46:592-595.
  4. Murthy R, Honavar SG, Naik M, Menon V, Bhaduri A, Das S. Centurion syndrome: Clinical presentation and surgical outcome. Orbit. 2009;28:269-274.
  5. Ali MJ. Principles and Practice of Lacrimal Surgery. 2nd ed.
  6. Nesi FA, Lisman RD, Levine MR. Smith and Nesi's Ophthalmic Plastic and Reconstructive Surgery.
  7. Ali MJ. Canaliculitis. Springer.
  8. Dortzbach RK. Ophthalmic Plastic Surgery: Prevention and Management of Complications.
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