Delhi Journal of Ophthalmology

Centurion Syndrome- Rarity Revisited

Bijnya Birajita Panda1, Jyotsna Sharma2
1Department of Ophthalmology, All India Institute of Medical Sciences, Bhubaneswar, Odisha, India.
2Department of Ophthalmology, Srirama Chandra Bhanja (S.C.B) Medical college, Cuttack, Odisha, India.

Corresponding Author:

Bijnya Birajita Panda 
Assistant professor
Department of Ophthalmology 
All India Institute of Medical Sciences, Bhubaneswar, Odisha, India.
E-mail: bigyan_panda@yahoo.co.in

Received: 02-JUN-2021

Accepted: 04-MAY-2022

Published Online: 25-JUN-2022

DOI:http://dx.doi.org/10.7869/djo.762

Abstract
Centurion syndrome is a rare cause of unexplained epiphora which often goes unrecognized due to a lack of awareness among ophthalmologists. This entity should be considered in the differential diagnosis of causes of epiphora which can be managed surgically. This article is a short review of the available literature on etiopathogenesis, clinical features, diagnosis, and current management of Centurion syndrome. 

Keywords :Epiphora, Centurion Syndrome, Medial Canthal Repositioning

Introduction 
Centurion syndrome is a rare idiopathic disorder characterised by abnormal anterior insertion of medial canthal tendon along with a prominent nasal bridge and malposition of the medial part of lid away from the globe displacing the lacrimal puncta out of lacus lacrimalis, resulting in epiphora. It was first described by Sullivan, Welham and Collins in 1993 in an article based on a series of thirteen patients with unexplained epiphora in whom a peculiar anterior insertion of medial canthal tendon (MCT) along with a high nasal bridge was observed. They described these patients as having ‘Centurion syndrome’ owing to the nasal structural similarity between their patients to those of Roman centurions.1 Few studies have been published regarding the diagnosis and management of this clinical entity.2-6 The exact insertion of anterior limb of MCT to the anterior lacrimal crest could be anterior, normal or maldeveloped and can be visualized on magnetic resonance imaging 7 The techniques described in literature for surgical correction are disinsertion/release of anterior limb of medial canthal tendon followed by suturing of the MCT stump to the anterior lacrimal crest. This can be accompanied by adjunctive procedures like canthoplasty, retractor plication and dacryocystorhinostomy. The purpose of this review is to summarize the clinical features which clinch to the diagnosis of this rare condition and further analyse the various surgical techniques utilized to treat this condition.



Material and Methods
An online search was made using the key words “centurion syndrome eyelid” on Google scholar, PubMed and Medline. 

Results
A total of 11 articles were retrieved which comprised of 5 case series and 6 case reports. Two articles were excluded considring linguistic barriers of other than English language. A total of 53 cases were analysed on the basis of clinical features, clinical tests performed, surgical procedure and their outcomes and summarized in (Table 1).



Discussion
Various ocular abnormalities may lead to epiphora including hyper lacrimation, eyelid abnormalities, lacrimal outflow obstruction. In this literature review we have described Centurion Syndrome which is a rare cause of epiphora and often goes undetected. It was first published as a series of 13 case reports in 1993 by Timothy J. Sullivan et al. Based on review of medical records of those 13 patients, they reached to the conclusion that epiphora in them was caused due to abnormal anterior insertion of medial canthal tendon which causes inferior punctum to displace anteriorly out of lacus lacrimalis and named the disorder as Centurion Syndrome due to similarity in nasal structure of their patients and the description of Roman Centurions. Two theories have been proposed for pathogenesis- one theory as proposed by Sullivan et al is the abnormal anterior insertion of medial canthal tendon, which has been confirmed based on pre and postoperative (i.e., after performing medial canthal tendon release) findings of lacrimal scintillography and the other theory is based on a more complex anatomic disparity between orbit and its contents.1

It is important to look for medial canthal tendon abnormality in patients presenting with epiphora. This disorder is more common in males (89.8%). Age at presentation of Centurion syndrome cases may vary from 1st decade to 4thdecade of life mean age being around 21 years of age. Age of onset varied in different case reports as Sullivan et al noted it to be between 3-10 years of age whereas majority of other case reports mentioned epiphora becoming apparent in 2nd decade of life. However, Sullivan et al mentioned that the symptom aggravated in their patients during puberty.1 The reason being growth of mid facial structures especially frontal process of maxilla and nasal bone during puberty which draws medial canthal tendon further forwards. Epiphora is mostly bilateral, in this review we found that 95% of cases whose laterality is mentioned are reported to be bilateral. Sullivan et al however reported that 15% cases may be unilateral. Prominent nasal bridge is an important factor aiding in diagnosis but its absence doesn’t exclude the diagnosis. Beak sign is the inferior angulation of medial canthus which can be looked for in suspected cases. Other causes of epiphora have to be ruled out by excluding history of discharge, pain over lacrimal sac area, any ocular trauma or surgery and by performing tests like ROPLAS, syringing or lacrimal passage irrigation, probing of puncta, Jones Primary Dye test. Deep set eyes or enophthalmos has been reported to be associated in 25.7% of cases in this review which contributes to the pathogenesis of disease by pulling the globe further inwards away from the lid by causing relative retro displacement of globe. Sullivan et al however didn’t find enophthalmos in their cases and concluded that it only has a minor role to play in the pathogenesis, if at all any.
Tests like Fluorescein dye disappearance test which shows delayed emptying of dye (i.e., persistence of dye beyond 3 minutes), tear film height which is raised, and lacrimal scintillography which shows functional block at medial canthus with hold up of activity before entry into the sac, are performed to help in finalising the diagnosis.

Treatment options include disinsertion of anterior limb of medial canthal tendon with or without posterior plication of the tendon stump with periosteum of lacrimal fossa just posterior to anterior lacrimal crest. Decision to do posterior plication can be made on table during surgery depending on whether adequate retro displacement of lid to globe has been achieved by anterior MCT release alone or not. Similarly, anterior MCT release can be supplemented with conjunctivoplasty to achieve adequate lid globe apposition and align the punctum with lacus lacrimalis. It can also be combined with punctoplasty. This surgery has been proven to be effective in almost all cases without enophthalmos except for one case reported by Huerva et al6 in which status of enophthalmos was not mentioned. However, it has been reported to be ineffective in patients having enophthalmos as seen in 4 case reports by Ma'luf et al4 where epiphora persisted after anterior MCT release and in 4 cases mentioned by Murthy et al who also reported 4 of their cases having enophthalmos.

Anterior MCT release along with medial eyelid retractor plication has been effective in both the cases it was performed, one of them having enophthalmos suggesting it as the preferred surgery for Centurion syndrome cases with enophthalmos. Bilateral lateral tarsal strip procedure done in a case having concomitant anterior displacement of lateral lid margin by Sullivan showed successful result.1 Post op follow up should be done up to a minimum period of 6 months to assess the outcome of surgery subjectively by grading epiphora and objectively by Fluorescein dye disappearance test. Post op lacrimal scintillography can also be done and compared with the pre op findings.

Conclusion
Diagnosis of Centurion Syndrome should be kept in mind while dealing with patients complaining of epiphora. A typical patient is a male in 2nd to 4th decade of life complaining of bilateral epiphora which affects his daily studies or work with the symptom becoming apparent during puberty having a prominent nasal bridge, deep set eyes, forward displacement of punctum out of tear lake and loss of lid globe apposition mostly involving medial side is likely to have Centurion syndrome. General ophthalmologists should be aware of this condition, recognizing and referring them to specialized personnel at appropriate time for surgical correction.

References
  1. Sullivan TJ, Welham RA, Collin JR. Centurion syndrome. Idiopathic anterior displacement of the medial canthus. Ophthalmology. 1993. ;100(3):328-333. 
  2. Sujatha Y, Sathish S, Stewart WB. Centurion syndrome and its surgical management. Ophthalmic Plastic and Reconstructive Surgery. 1999; 15:243-244. 
  3. Murthy R, Honavar SG, Naik M, Menon V, Bhaduri A, Das S. Centurion syndrome: clinical presentation and surgical outcome. Orbit. 2009;28(5):269-74. 
  4. Ma'luf RN, Bashshur ZF, Noureddin BN. Anterior canthal tendon release for correction of the centurion syndrome. Ophthalmic Plastic and Reconstructive Surgery. 2003 Nov;19: 446-448. 
  5. Chang JH, O'Donnell BA. Medial canthal tendon release and lower eyelid retractor plication for Centurion syndrome. Clin Exp Ophthalmol. 2006; 34:284-5.
  6. Huerva V, Sánchez MC, Canto LM. Surgical management of Centurion syndrome. Can J Ophthalmol. 2007 Dec;42 :879-80.
  7. Alam MS, Tongbram A, Noronha OV. Magnetic Resonance Imaging Features of Medial Canthal Tendon in Centurion Syndrome. Curr Eye Res. 2021 Apr;46(4):592-595. doi: 10.1080/02713683.2020.1817492. Epub 2020 Sep 7. PMID: 32857945.

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Panda BB, Sharma JCenturion Syndrome- Rarity Revisited.DJO 2022;32:25-27

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Panda BB, Sharma JCenturion Syndrome- Rarity Revisited.DJO [serial online] 2022[cited 2022 Aug 7];32:25-27. Available from: https://www.djo.org.in/articles/32/4/Centurion-Syndrome-Rarity-Revisited.html