Trachoma

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Disease Entity

Disease

Trachoma is the most common infectious cause of blindness in the world caused by recurrent ocular surface infection and secondary scarring by the bacterial organism Chlamydia trachomatis. Repeat infection with this organism leads to conjunctival inflammation and scarring, trichiasis, and ultimately blinding corneal opacification and thus a leading cause of preventible blindness.

Epidemiology

The World Health Organization (WHO) reports trachoma is endemic to more than 50 countries, with most blinding trachoma in Africa with cases still being reported in the Middle East, north west India and parts of Southeast Asia as well the b. An estimated 21 million people are living with active trachoma and another 2.2 million people are blind or severely visually impaired. Furthermore, 7.3 million people suffer from trichiasis and are at risk for developing blindness.

Epidemiologic studies have shown active trachoma is most common in young children with the prevalence of active trachoma decreasing with age. The long-term sequelae of trachoma infection, including scarring, trichiasis and corneal opacification in adults relates to their exposure to active trachoma when they were young. The gender distrubtion of active trachoma is similar; however, scarring and trichiasis are more common in women than men because women are more likely to care for young children and have longer exposure to the disease.

Pathogenesis

The Organism:

Trachoma is caused by the bacterium Chlamydia trachomatis, which was first discovered by Prof. Feifan Tang and Xiaolou Zhang in 1956. Four ocular serotypes of Chalmydia trachomatis: A, B, Ba and C have been implicated. Infection with genital serotypes D to K can cause isolated episodes of ophthalmia neonatorum in infants or inclusion conjunctivitis in adults and do not generally lead to blindness.

Pathophysiology:

Blindness from trachoma is due to recurrent episodes of active infection over months to years. The initial infection is confined to the conjunctival epithelium and triggers an immune response presenting as conjunctival follicles. Repeated infections with subsequent inflammatory responses results in tissue destruction with fibrosis and contraction, cicatricial entropion with trichiasis and lash corneal touch resulting in corneal erosions, ulcerations, scarring and vascularisation resulting in corneal opacification and resultant blindness.

Risk Factors

Risk factors for trachoma include things that favor transmission of the organism. These include Endemic geographical regions: parts of North Africa, Middle East, North west India and parts of Southeast Asia. Limited environmental and social hygiene resources eg. Inadequate water supply with less water availabe to use for facial cleansing, breeding and transmission of flies that may help spread disease Poor facial hygiene: secretions around the eye attracts flies that are physical vectors for C. trachomatis tranmission & shared personal objects Overcrowded Living Conditions: close contact, especially between children sleeping in same bed, enables exchange of secretion.


Grading

The WHO grading system: [1]

Trachomatous Inflammation (TF) The presence of 5 or more follicles (>0.5 mm) in the upper tarsal conjunctiva Trachomatous Inflammation (TI) Inflammatory thickening of the tarsal conjunctiva that obscures more than half of the deep normal vessels Trachomatous Scarring (TS) The presence of scarring in the tarsal conjunctiva Trachomatous Trichiasis (TT) At least one lash rubs on the eyeball Corneal Opacity Easily visible corneal opacity over the pupil


Clinical Findings

The clinical manifestations of trachoma can be divided into findings associated with active disease, those associated with repeat infections and late stages with scarring and residual abnormalities.

Active Disease:

  • Follicular Conjunctivitis: Follicles are dome-shaped collections of lympoid cells. The center of the follicle is avascular and has blood vessels that surround the round base. These appear as yellow-white elevations and are prominent on the everted upper eyelid in trachoma. Large conjunctival follicles located at the upper margin of the cornea may leave depressions known as 'Herbert's pits' which are pathognomonic for trachoma.
  • Papillary Hypertrophy: engorgement of small vessels with edema that can obscure deep tarsal vessels
  • Corneal Pannus: vascularization of the upper cornea is usually a late feature from chronic corneal erosions and inflammation.


Repeat Infection and Inflammation:

  • Conjunctival Scarring: White bands or lines that represent fibrosis
  • Cicatricial Entropion and Trichiasis: Fibrotic scar tissue contraction causes the eyelid margin and eyelashes turning inward and lashes touching the ocular surface - cornea and conjunctiva.
  • Corneal Opacification and vascularisation: secondary to repeat corneal irritation from inturned eyelashes

Diagnosis

Trachoma is a clinical diagnosis based on the above mentioned clinical findings. PCR testing developed for urogenital infection is available but typically utilized for research purposes only. Some of the available diagnostic tools include: cytologic tests (Giemsa stain or direct fluorescent antibodies), different cell cultures and enzyme immunoassay methods. These three methods have been surpassed in both sensitivity and specificity by nucleic acid amplification tests (NAATs). Currently, there is insufficient evidence to support the use of NAATs for national elimination programs

Histopathology

Histopathological studies of active inflammatory trachoma are characterized by diffuse mixed inflammatory cell infiltrate of the conjunctiva, mild to moderate epithelial hyperplasia and lymphoid follicles in the stroma; the latter being a clinical and pathological hallmark of trachoma. In cicatricial trachoma, the conjunctiva may display a chronic inflammatory infiltrate, mostly marked in the substantia propria with predominantly lymphocytes. Conjunctival epithelium may show squamous metaplasia or atrophy with multiple denuded areas. Underneath this epithelium, stroma may be replaced with thick, compact avascular scar tissue. [2] [3]

Symptoms

Most individuals are asymptomatic or have mild symptoms depending on the level of inflammation. Symptoms, if present, are similar to those seen in any chronic conjunctivitis and include redness, discomfort, tearing, photophobia and muco-purulent discharge.

Management

The World Health Organization recommends the 'SAFE' strategy for the management of trachoma. This strategy was developed in 1997 by The Alliance for the Global Elimination of Blinding Trachoma by the year 2020 (GET 2020) and utilizes a 4 step approach:

  • S: Surgery for Trichiasis
  • A: Antibiotics for C. trachomatis infection
  • F: Facial cleanliness
  • E: Environmental change to improve sanitation and increase access to clean water.

Prevention is ideally the best strategy which includes hygiene, public education coupled with increased awareness.

Studies have shown that with rapid improvement in overall health, hygiene and environmental status of societies and nations, there has been a significant reduction in spread and prevalence of the disease even in endemic regions.

Recognition with adoption of good hygienic practices, improvement in overall general health, reduced social crowding all have great impact. Mass antibiotic therapy has also been used in endemic countries with significant gains noted. however with antibiotic resistance also being recognised.

Medical management begins with recognition with clinical and/or laboratory confirmation. New or recent onset active infective/inflammatory phase of the disease should be teated medically with appropriate and early use of antibiotics. Historically prolonged treatment with tetracycline group of drugs with topical application was considered. More recently single dose oral antibiotics - Azithromycin and even topical Azithromycin used twice daily for 3 days has been reported to be as effective (Diab MM, Allen RC, Gawdat TI, Saif AS. Trachoma elimination, approaching 2020. Curr Opin Ophthalmol. 2018 Sep;29(5):451-457. doi: 10.1097/ICU.0000000000000504. PMID: 29965850. Evans JR, Solomon AW, Kumar R, Perez Á, Singh BP, Srivastava RM, Harding-Esch E. Antibiotics for trachoma. Cochrane Database Syst Rev. 2019 Sep 26;9(9):CD001860. doi: 10.1002/14651858.CD001860.pub4. PMID: 31554017; PMCID: PMC6760986.) Supportive treatment include lid and ocular surface hygiene, aggressive lubrication and antibiotics for secondary infections.

Surgical management is reserved for late and advanced cases after maximum medical ocular surface management. This may include simple measures such as those that redirect eyelashes and eyelid margin away from the cornea. Various tarsal fracture techniques with lid margin rotation have been described although with recurrences when performed inadequately or with untreated active infections. For recurrent cases, additional procedures including posterior lamellar augmentation may also be considered.


Summary

Trachoma is one of the still prevalent and leading causes of preventible corneal blindness around the world especially in endemic regions. The WHO target of eradition by 2020 has only been met partially, especially in those regions with active eradication activities and implementation with sustain public health efforts. Simple antibiotic therapy in early stages, mass antibiotic programs and reconstructive surgery may help prevent and manage patients better.

Additional Resources

References

  1. WHO/Department of control of neglected tropical diseases. Trachoma simplified grading card SAFE documents. World Health Organization (WHO), 1987. http://www.who.int/trachoma/resources/SAFE_documents/en/ Accessed 10 October 2017
  2. Hu VH, Holland MJ, Burton MJ. Trachoma: protective and pathogenic ocular immune responses to Chlamydia trachomatis. PLoS neglected tropical diseases. 2013; 7(2), e2020.
  3. Keenan JD, Lietman TM. Chlamydial infections. In Cornea. 4th ed. Elsevier Mosby. 2016; 503–507.