Tuesday, June 4, 2019
Unilateral Peters Anomaly in a Nigerian Child
Unilateral Peters Anomaly in a Nigerian small fryCase lineAbstract10-week-old female infant presented in our clinic in March, 2013 with score of small right universe since birth. There is associated history of whitish speck in the affected eye.The child is a crossroad of term pregnancy which was uneventful. M separate denied any history of febrile illness during pregnancy. Nil history of maternal rashes, nil comprehend or visual challenges, nil engross of any drug apart from routine ante-natal haematinics. No history of use of traditional medicine in the cover of her pregnancy. The patient role is the second child of her parents. There is no similar occurrence in the other sibling or in any other division of the monogamous family. optic examination revealed right microphthalmos, microcornea and cornea opacity with pannus. The whole termination of the cornea was affected. An assessment of right Peters anomaly was made. The optic ultrasound make revealed that the right g lobe measured 16.1mm in its AP diameter and the left(p) globe measured 16.9 mm. There was cataract in the right eye and the introductory chamber was absent with adherence of the iris diaphragm to the cornea. Patient was to be placed on cosmetic contact lens in future when she would be old enough to manage it.Key talking to Cornea opacity, microphthalmos, unilateral, Nigerian, child INTRODUCTIONPeters anomaly is a form of congenital corneal opacity. It is associated with malformation of the frontal segment of the eye. 1 The aetiology of the condition is non clear. The well-nigh likely causes include genetic, infectious, traumatic and toxic factors. 1Peters anomaly is a rare form of anterior segment dysgenesis in which ab design cleavage of the anterior chamber occurs. The condition whitethorn involve the central or entire cornea. The leucoma is associated with irido- corneal adherences at the area of the leucoma. In the inherited form, mutations involve the PAX6 gene.2 Peter s anomaly was expound by Peters about one hundred years ago. 3It is a rare condition world wide, however the incidence rate is unknown.The condition does not strike racial or sexual predilection. A study done by Bhandari et al. Showed equal distribution in both sexes. 4 There are devil types of Peters anomaly based on the state of the lens namely type 1 and type 2. In type 1, the lens may or may not be cataractous but the lens does not adhere to the cornea. In type 2, the lens is cataractous and adheres to the cornea. 5 Type 1 is usually unilateral plot of land type 2 is bilateral in 60% of cases. The cornea is usually clear at the periphery and vascularisation is not common however there may be sclerization of the limbus. Case HistoryA 10-week-old female infant presented in our clinic in March,2013 with history of small right globe since birth. The mother noticed patient to have small right globe since few hours after birth. There is associated history of whitish speck in the a ffected eye. The child is a product of term pregnancy, which was uneventful. Mother denied any history of febrile illness during pregnancy. Nil history of maternal rashes, nil hearing or visual challenges, nil use of any drugs apart from routine ante-natal haematinics, nil ingestion of native concoction during pregnancy. She received routine antenatal Tetanous Toxoid vaccination. She neither smoked cigarette nor drank alcohol. She did not chew kola either. Child was delivered by spontaneous vagina delivery, vertex presentation. She cried spontaneously immediately after birth and the umbilical cord was cut using uninventive cord scissors. She had received immunization according to National Programme on Immunization schedule. She had also been achieving all the normal developmental milestones. There is no history of any febrile illness since birth. She is the second child of her parents. There is no similar occurrence in the other sibling or in the family.Ocular examination revealed right microphthalmos, microcornea and cornea opacity with pannus. General and systemic examination did not reveal any other abnormality.An assessment of right Peters anomaly was made. The nature of this condition was explained to patients parents with counselling on how to raise the patient. Consent was obtained from the mother and clinical photograph of the condition was taken. The ocular ultrasonography done revealed that the right globe measured 16.1mm in its AP diameter and the left globe measured 16.9 mm. There was cataract in the right eye and the anterior chamber was absent. The left eye was sonographically normal. It is our belief that patient may benefit from the use of cosmetic contact lens in future. DiscussionThe early presentation of the patient is sort of commendable. This may be related to the rarity of the ocular condition and the fact that the parents were enlightened.Most cases of Peters anomaly are sporadic however the inherited ones could be of autosomal recessi ve and dominant modes of inheritance. It is associated with abnormalities of chromosome 4. 6 We could not establish family history of similar presentation in the family of this patient. The other child of the parents was also said to be normal and in stable condition. Based on this fact, it is our considered opinion that the case may actually be a sporadic one. Peters anomaly is often an isolated ocular defect but there may be associated ocular and systemic anomalies. However further examination of the patient did not reveal any other systemic abnormality. This may be related to the fact that the condition was unilateral as bilateral Peters anomaly is more often associated with systemic anomalies. The globe was spy to be small and this was confirmed with the aid of ultrasonography. The associated anterior segment anomalies with Peters anomaly include glaucoma, anterior polar cataract, cornea plana, sclerocornea, microphthalmos, colobomata as well as mesodermal dysgenesis of the ant erior chamber angle and iris. 7 Peters anomaly may be associated with systemic anomalies like limb deformities, craniofacial anomalies, cleft lip and palate as well as genitourinary defects. 8-10Treatment of Peters anomaly is challenging and unsatisfactory. The outcome of corneal transplantation is not rewarding due to post operative complications like graft failure, cataract, retinene detachment and glaucoma.11 Alternative treatment measures include rotation of corneal autograft, atropinisation of the pupil most especially if the visual axis is spared and optical iridectomy.The involvement of the whole extent of the cornea and the microphthalmic globe are associated with hapless prognosis for visual recovery. Affectation of only one eye is also risk factor for amblyopia. The patient was not adequate for corneal grafting in view of the vascularisation of the cornea. Optical iridectomy or atropinisation was also not considered as the whole extent of the cornea was affected. In vie w of the poor prognosis coupled with the cosmetic challenge, the patient was to be placed on cosmetic contact lens when she is old enough to manage contact lens. remainderWe conclude that prompt presentation and treatment of patients with Peters anomaly is desirable. There is need to create more awareness about the condition among health care providers and the canaille in view of the fact that it is rare. Multidisciplinary approach should be adopted in the management of affected patients most especially those with associated systemic anomalies. Figure 1 Child with right eye Peters anomaly
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