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 Table of Contents  
CASE REPORT
Year : 2022  |  Volume : 2  |  Issue : 3  |  Page : 763-766

Parinaud's oculoglandular syndrome: A diagnostic dilemma


Department of Ophthalmology, Faculty of Medicine, University of Malaya, Kuala Lumpur, Malaysia

Date of Submission29-Jan-2022
Date of Acceptance23-Mar-2022
Date of Web Publication16-Jul-2022

Correspondence Address:
Dr. Goh Chon Han
Ophthalmology Clinic, Department of Ophthalmology, Pusat Perubatan Universiti Malaya, Lembah Pantai, 59100, Kuala Lumpur
Malaysia
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijo.IJO_263_22

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  Abstract 


Parinaud's oculoglandular syndrome (POGS) is a rare condition characterized by unilateral granulomatous conjunctivitis and ipsilateral regional lymphadenopathy. Among all infectious agents, cat scratch disease (CSD), which is caused by Bartonella henselae, remains the commonest etiology agent of POGS. We present a case report of a 13-year-old boy who came to a tertiary center in Malaysia with left granulomatous conjunctivitis and subsequent necrotizing lesions involving left upper eyelid, left paranasal region, and left preauricular lymph nodes. With a poor initial treatment response and confusing investigation results, this interesting case describes the diagnostic dilemma in differentiating the etiological agents of POGS.

Keywords: Bartonella henselae, caseating granulomatous lymphadenitis, cat scratch disease, Chlamydia conjunctivitis, granulomatous conjunctivitis, Parinaud's oculoglandular syndrome, Mycobacterium Tuberculosis


How to cite this article:
Han GC, Run NH, Jalaluddin JB. Parinaud's oculoglandular syndrome: A diagnostic dilemma. Indian J Ophthalmol Case Rep 2022;2:763-6

How to cite this URL:
Han GC, Run NH, Jalaluddin JB. Parinaud's oculoglandular syndrome: A diagnostic dilemma. Indian J Ophthalmol Case Rep [serial online] 2022 [cited 2022 Aug 13];2:763-6. Available from: https://www.ijoreports.in/text.asp?2022/2/3/763/351144



Parinaud oculoglandular syndrome (POGS) is a rare condition characterized by unilateral granulomatous conjunctivitis with surrounding follicles and ipsilateral regional lymphadenopathy.[1],[2] It is often associated with ipsilateral preauricular, submandibular, or cervical necrotizing lymphadenitis with or without stellate abscesses.[1],[2],[3]

Among all infectious agents, cat scratch disease (CSD), which is caused by Bartonella henselae, a gram-negative fastidious bacillus, remains the commonest etiological agent of POGS.[2] The incidence of CSD is quoted to be between 2.4 and 7/100,000/year.[3] Lymphadenopathy, fever, malaise, anorexia, and fatigue are the commonest presentations of CSD.[1],[2],[3] Ocular manifestations of CSD include neuroretinitis, papillitis, optic neuritis, retinochoroiditis, and POGS. Only 5%–10% of CSD cases are reported to be POGS.[2],[3] Treponema pallidum, Francisella tularensis, sporotrichosis, Mycobacterium tuberculosis and Chlamydia trachomatis acute infection are the other less-common causes of POGS.[1],[3]

Due to the low incidence of the condition and scarcity of local literature on this topic, POGS is often misdiagnosed and missed on the initial diagnostic workups.

We present a case report of POGS from Hospital Pakar Sultanah Fatimah Muar, a tertiary center in Southern Peninsular Malaysia, highlighting its etiological and diagnostic challenges.


  Case Report Top


A 13-year-old healthy Malay boy presented with a history of persistent left eye redness and foreign body sensations for the past two weeks. He had a few domestic kittens at home, but denied clear history of being scratched or bitten by them.

Initial examinations revealed left eye preseptal swellings with minimal diffuse conjunctival injections. Numerous nontender giant papillae–nodular swellings were noted at the upper palpebral conjunctiva [Figure 1]. After a week, his condition worsened with episodes of fever, new superior eyelid, and paranasal skin lesions [Figure 2]. Concurrently, there were ipsilateral suppurative preauricular [Figure 3] and postauricular lymphadenitis. His visions remained unaffected. Other examinations were normal.
Figure 1: Left eyelid swelling with numerous nontender, giant papillae–nodular elevations were noted at left upper palpebral conjunctiva

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Figure 2: Left paranasal lesion developed concurrently with left superior eyelid lesions. These lesions were erythematous and raised. These lesions were found to have well-defined margins and central irregular ulcerations

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Figure 3: Firm ipsilateral suppurative preauricular lymphadenitis with overlying ulceration was noted. It was tender upon palpation

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Blood investigations revealed mild leukocytosis with neutrophilia, elevated erythrocyte sedimentation rate (ESR), and raised C-reactive protein (CRP). However, his chest radiography was normal. Tuberculin skin test (Mantoux test) was normal (0 mm).

He showed no response to empirical intravenous ceftriaxone to antibiotic regime. Conjunctival swab gram staining showed no organism. Multiple conjunctival swab samplings examined after Giemsa staining failed to demonstrate Chlamydia spp. inclusion body. Conjunctival swab and lymph node pus cultures were negative. Fine needle aspiration and cytology (FNAC) of preauricular lymph node showed evidence of granulomatous inflammations. A conjunctival biopsy was done and sent for tuberculosis polymerase chain reaction (PCR) testing. However, M. tuberculosis complex was not detected. Syphilis screening tests were negative as well.

While awaiting for the result of further investigations, oral doxycycline 100 mg BD was empirically initiated. To our surprise, his condition remained static after a 6-week course of doxycycline and azithromycin, despite both C. trachomatis and B. henselae testing positive during the course of disease (Chlamydia inclusion body was detected via immunofluorescent antibody assay (IFA) testing; Bartonella serology revealed Bartonella IgG of 1:128, no IgM was detected).

Subsequently, left palpebral conjunctival mass and left paranasal lesion incisional biopsies were done. Evidence of caseating granulomatous inflammations were noted during microscopic examination of the samples [Figure 4] and [Figure 5]. After multidisciplinary discussions were made, anti-tuberculous regimen was started. Finally, he attained complete resolution [Figure 6] within two months of anti-tuberculous regimen (OD dosing of isoniazid 75 mg, rifampicin 150 mg, ethambutol 275 mg, and pyrazinamide 400 mg). He completed his maintenance regimen of anti-tuberculous drugs without further recurrence.
Figure 4: Hematoxylin and eosin (H&E) stained section of left conjunctival mass and paranasal tissues incisonal biopsy samples (Magnification x 5): multiple fragments of fibrous tissues with foci of granulomata foci of granulomata (arrow). Central caseating necrosis was seen in some granulomata

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Figure 5: Hematoxylin and eosin (H&E) stained section of left conjunctival mass and paranasal tissues incisonal biopsy samples (Magnification x 10): multiple fragments of fibrous tissues with foci of granulomata composed of collection of epithelioid histiocyte (H) and surrounded by small mature lymphocytes (L)

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Figure 6: Resolution of lymphadenitis and periorbital lesions within two months of anti-tuberculous regimen (note the healing incisional biopsy wounds over left upper eyelid and paranasal region)

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  Discussion Top


This is a case of diagnostic dilemma among three causal agents of POGS, namely, B. henselae, C. trachomatis, and M. tuberculosis.

There is no single gold standard diagnostic criterion utilized in the literature till date.[4] This case fulfilled all the four diagnostic criteria mentioned by Margileth et al. [Table 1].[5] This patient had positive history of contact with cat, negative tuberculin skin test, sterile pus aspirated from node, positive IFA (IgG = 1:128), and positive histological findings.
Table 1: Diagnostic criteria of cat scratch disease as described by Margileth et al

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In acute B. hensalae infection, antibody to Bartonella IgM is elevated, but it is often short-lived and may not always be present.[4],[5] Contrary to this diagnostic criterion, a few studies suggested the value of B. hensalae IFA IgG titer of 1:64 or less does not indicate active infection, titers 1:64 to 1:256 suggest possible infection, and titers >1:256 suggest acute infection.[6] Therefore, the IgG level in this case is not high enough to indicate active infection. In the context of disease progression and recovery, his condition was not self-limiting, as described in other journals about POGS caused by B. hensalae infection.[1],[2],[3] He did not respond to azithromycin and doxycycline, as recommended in the guidelines.[2],[3]

Inclusion conjunctivitis is another differential diagnosis. This is an oculogenital infection caused by the serological variant D-K of C. trachomatis.[1] It could manifest similarly with unilateral red eye, marked hyperemia, papillary hypertrophy, follicular conjunctivitis, and a tender preauricular lymphadenopathy.[1] Chlamydia urethritis could be asymptomatic in male patients.[1] Similar to Bartonella spp., this intracellular fastidious bacterium is also difficult to isolate in culture. This diagnosis was in favor as the conjunctival swab immunofluorescent antibody assay (IFA) testing was positive for Chlamydia. However, the absence of sexual exposure together with unidentifiable basophilic intracytoplasmic inclusion body in conjunctival swabs (with Giemsa staining) are making this diagnosis doubtful. He also did not respond to the recommended azithromycin and doxycycline regimen for Chlamydia POGS, as reported in other journals.[3],[7]

POGS secondary to conjunctiva tuberculosis could be the best-fitting diagnosis, especially in Malaysia, a tuberculosis-endemic country. This case presented similarly with hypertrophic granulomatous subclassification of conjunctiva tuberculosis, as described by Eyre et al.[8] The typical caseating granulomatous inflammatory features together with failure to respond to conventional antibiotics made this etiological agent favorable. Given the fact that he responded well to anti-tuberculous regimen, we believe this could be POGS secondary to M. tuberculosis infection. This diagnosis is often challenging as extrapulmonary tuberculosis always manifest with low baterial load.[9] This explains the reason why this causal agent was not detected despite using better diagnostic tests (e.g., conjunctival PCR for M. tuberculosis and conjunctival culture with Ogawa culture medium for acid-fast bacilli). The window period between infection and positive skin test reactivity in tuberculin skin test (Mantoux test) is 8–10 weeks after exposure.[10] Hence, this truly explains the reason why this test was negative in the early course of disease.


  Conclusion Top


In conclusion, it is crucial to recognize the common presentations of this uncommon clinical syndrome. However, the commonest etiological agents (B. hensalae) may not always be the causal agent in every POGS case. High index of suspicion of other etiological agents with well-targeted individualized treatment for every patient is important.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Kanski JJ, Bowling B. Clinical Ophthalmology: A Systematic Approach. 7th ed. Philadelphia: Elsevier/Saunders; 2011. p. 162, 137-9.  Back to cited text no. 1
    
2.
Yanoff M, Duker JS. Fourth Edition Ophthalmology. Elsevier Saunders; 2014. Chapter 4.6 Conjunctivitis: Infectious and Non infectious. International Edition ISBN 978-1-4557-3983-7. p. 188.  Back to cited text no. 2
    
3.
Arjmand P, Yan P, O'Connor MD. Parinaud oculoglandular syndrome 2015: Review of the literature and update on diagnosis and management. J Clin Exp Ophthalmol 2015;6:2.  Back to cited text no. 3
    
4.
Florin TA, Zaoutis TE, Zaoutis LB. Beyond cat scratch disease: Widening spectrum of Bartonella henselae infection. Pediatrics 2008;121:e1413-25.  Back to cited text no. 4
    
5.
Margileth AM. Recent advances in diagnosis and treatment of cat scratch disease. Curr Infect Dis Rep 2000;2:141-6.  Back to cited text no. 5
    
6.
Mabra D, Yeh S, Shantha JG. Ocular manifestations of bartonellosis. Curr Opin Ophthalmol 2018;29:582-7.  Back to cited text no. 6
    
7.
Malamos P, Georgalas I, Rallis K, Andrianopoulos K, Georgopoulos G, Theodossiadis P, et al. Evaluation of single-dose azithromycin versus standard azithromycin/doxycycline treatment and clinical assessment of regression course in patients with adult inclusion conjunctivitis. Curr Eye Res 2013;38:1198-206.  Back to cited text no. 7
    
8.
Eyre JWH. Tuberculosis of the conjunctiva: Its etiology, pathology and diagnosis. Lancet 1912;1:1319-28.  Back to cited text no. 8
    
9.
Salas D, Murthy S, Champ C, Hawksworth N. Primary tuberculosis of the conjunctiva. Eye 2001;15:674-6.  Back to cited text no. 9
    
10.
National Tuberculosis Controllers Association, Centers for Disease Control and Prevention Guidelines for the investigation of contacts of persons with infectious tuberculosis. Recommendations from the National Tuberculosis Controllers Association and CDC. MMWR Recomm Rep 2005;54:1-47.  Back to cited text no. 10
    


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