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BRIEF REPORT |
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Year : 2007 | Volume
: 55
| Issue : 1 | Page : 57-59 |
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Unusual dendritic keratitis
Nikhil S Gokhale1, Alpa J Dherai2, Haresh Desai2, TF Ashavaid2
1 Gokhale Eye Hospital and Eyebank, Anant Building, Gokhale Road, Dadar West, Mumbai - 400 028, India 2 Dept. of Laboratory Medicine, PD Hinduja National Hospital and Medical Research Center, Veer Savarkar Marg, Mahim, Mumbai - 400 016, India
Date of Submission | 26-Jul-2005 |
Date of Acceptance | 29-Jan-2006 |
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Correspondence Address: Nikhil S Gokhale Gokhale Eye Hospital, Anant Bldg, Gokhale Road South, Dadar West, Mumbai, India
Source of Support: None, Conflict of Interest: None | Check |
DOI: 10.4103/0301-4738.29497
Bilateral pseudo-dendritic keratitis in infancy can be due to tyrosinemia, a rare metabolic disorder. Ocular involvement may be the earliest presenting manifestation of this disease. Early diagnosis is essential because dietary modifications can result in complete reversal of the manifestations of this disorder. This disease must be suspected in all cases of non-responsive dendritic keratitis in the pediatric age group, especially if it is associated with cutaneous lesions such as patmoplantar keratosis. Serum tyrosine levels must be done in these cases. Keywords: Dendrite, keratitis, phenylalanine, pseudo-dendrite, tyrosine, tyrosinemia.
How to cite this article: Gokhale NS, Dherai AJ, Desai H, Ashavaid T F. Unusual dendritic keratitis. Indian J Ophthalmol 2007;55:57-9 |
Corneal dendrites are a common finding in herpetic keratitis and usually respond well to antivirals. However, one must consider pseudo-dendritic keratitis especially if dendrites are bilateral and not responding to antiviral therapy. Tyrosinemia is a rare metabolic disorder, which can present in infancy as bilateral pseudo-dendrites. Ocular involvement may be the earliest presenting manifestation of this disease. An early diagnosis is important as timely dietary changes can reverse changes, stop progression of the disease and avoid neurological impairment. We report a case of bilateral pseudo-dendritic keratitis in an infant, which was initially treated as a viral keratitis but showed no improvement and was subsequently diagnosed as tyrosinemia.
Case Report | | |
A five-month-old infant presented to us with symptoms of photophobia and corneal haze noticed by the parents since two months of age. The child was initially treated with antibiotic drops for about two months with no improvement. Subsequently, the child was examined under anesthesia and a diagnosis of bilateral dendritic keratitis was made. The child was put on acyclovir eye ointment five times a day in both eyes. After two weeks a repeat examination showed no improvement and the dendritic pattern persisted in both eyes. The child was brought to us for a second opinion. A repeat examination was done under anesthesia. On examination both the eyes were quiet with no congestion or discharge, both corneas had pseudo-dendrites, which showed staining with fluorescein [Figure - 1][Figure - 2]. Subtle differences from a classical viral dendrite could be appreciated only under very high magnification. The dendritic pattern was formed by multiple white opacities and there were small gaps between these opacities. Opacities under high magnification appeared to have a crystalline appearance. Corneal esthesiometry could not be done. The rest of the ocular examination was within normal limits.
The presence of bilateral pseudo-dendrites and a history of a lack of response to acyclovir, made us suspect the possibility of tyrosinemia Type II. The parents were advised to have a biochemistry workup for inherited metabolic diseases and also have a pediatric evaluation.
Urine metabolic screen and plasma amino acid profile were done. The urine tested positive for nitrosonaphthol test (for tyrosine) and ferric chloride test (for oxo acids and detection of phenylketonuria). The plasma amino acid profile was done by high performance liquid chromatography. The tyrosine levels were 1474 nmoles/ml (Ref. range 22-108 nmoles/ml). The test was repeated after 15 days and the levels obtained were 1276 nmoles/ml. The child's liver and renal functions were within normal limits. No systemic problems were found on routine pediatric consultation. The child was diagnosed with tyrosinemia Type II and referred to a nutritionist and a pediatrician specializing in metabolic disorders for further dietary advice and guidance. The child will require long-term multidisciplinary management and frequent monitoring of tyrosine levels. Unfortunately, tyrosine and phenylalanine-free food products are not available in India and needed to be imported. General guidelines to avoid dietary proteins containing these amino acids have been given by the dietician. After dietary modifications the tyrosine levels were 83 nmoles/ml and the child's photophobia and corneal haze has completely regressed.
Discussion | | |
Tyrosine is an organic amino acid precursor in the metabolic pathways of amines, which include thyroid hormones and the neurotransmitters epinephrine, norepinephrine, dopamine and tyramine. Elevated levels of serum tyrosine can occur in transient neonatal tyrosinemia, tyrosinemia Type I (hepatorenal tyrosinemia), tyrosinemia Type II (oculocutaneous tyrosinemia, Riecner-Hanhart syndrome) and a rare disorder tyrosinemia Type III (may be asymptomatic or associated with mental retardation).[1] Hepatorenal disease is not seen in Type II and corneal changes are not seen in Type I.[2] Tyrosinemia Type II is due to a deficiency of the enzyme tyrosine aminotransferase.[3] It is characterized by eye lesions in about 75% of the cases, skin disease (80%) or neurological complications (60%) or any combination of these.[3] Serum tyrosine levels are 2.5 to 25 times higher than normal.[2] Ocular involvement may be the earliest presenting manifestation of this disease. Keratoconjunctivitis with photophobia may appear before the patient is two weeks of age.[4],[5] The corneal opacities are bilateral superficial punctate crystalline deposits that may assume a dendritiform pattern leading to an ulcerated keratitis and a mistaken diagnosis of herpes simplex keratitis.[6],[7],[8] Peripheral vascularization, irregular hazy epithelium and corneal haze develop over time. The ocular findings may appear months before the hyperkeratotic skin lesions on the hands and feet (palmo-plantar keratosis). Variable mental retardation, nystagmus, strabismus, conjunctival thickening and cataract have also been reported in association with tyrosinemia Type II. Treatment with dietary restriction of phenylalanine and tyrosine results in complete reversal of both ocular and dermatologic abnormalities.[5],[6],[7]
The case presented to us as a nonresponding herpetic keratitis, however, because the dendrites had an atypical appearance, we considered a differential diagnosis of tyrosinemia Type II. This disease must be suspected in all cases of non-responsive dendritic keratitis in the pediatric age group, esspecially if it is associated with cutaneous lesions such as patmoplantar keratosis.[6] An early diagnosis is important as timely dietary changes can reverse oculo-cutaneous changes, stop progression of the disease and avoid neurological impairment. Early initiation of dietary changes in infancy is most effective in preventing cognitive impairment.[7]
References | | |
1. | Kvittingen EA, Holme E. Disorders of tyrosine metabolism. Inborn metabolic diseases. In : Fernandes J, Saudubray JM, van den Berghe G, editors. 3rd ed. Springer: 2000. p 187-94. |
2. | Burns RP. Tyrosinemia. In : Gold DH, Weingeist TA, editors. The eye in systemic disease. JB Lippincott: Philadelphia; 1990. p. 327-9. |
3. | Buist NR, Kennaway N, Fellman JG. Tyrosinemia type II: Hepatic cytosol tyrosine amino - transferase deficiency. In : Bickel H, Wachtel U, editors. Inherited diseases of aminoacid metabolism. George Thieme: Stuttgart; 1985. p. 203-35. |
4. | Burns RP, Gipson IK, Murray MJ. Keratopathy in tyrosinemia. Birth Defects 1976;12:169. |
5. | Roussat B, Fournier F, Besson D, Godde-Joly D. A propos de deux cas de tyrosinose de type II (syndrome de Richner-Hanhart). Bull Soc Ophthalmol Fr 1988;88:751. [ PUBMED] |
6. | Benatiya AI, Bouayed MA, Touiza E, Daoudi K, Bhalil S, Elmesbahi I, et al . Tyrosinemia type II - Case report. Bull Soc Belge Ophtalmol 2005;296:57-61. |
7. | Macsai MS, Schwartz TL, Hummel MB, Mulhern MG, Rootman D. Tyrosinemia type II: Nine cases of ocular signs and symptoms. Am J Ophthalmol 2001;132:522-7. [ PUBMED] [ FULLTEXT] |
8. | Charlton KH, Binder PS, Wozinak L, Digby DJ. Pseudodendritic keratitis and systemic tyrosinemia. Ophthalmology 1981;88: 355-60. |
[Figure - 1], [Figure - 2]
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