Correspondence REFERENCES 1. El Kehdy J, Abbas O, Rubeiz M. A review of Parry Romberg syndrome. J Am Acad Dermatol. 2012;67:769-84. 2. Asher S, Berg B. Progressive hemifacial atrophy: report of three cases, including one observed over 43 years, and computed tomographic findings. Arch Neurol. 1982;39:44-6. 3. Delaire J, Lumineau JP, Mercier J, et al. Romberg’s syndrome: progressive facial hemiatrophy. Rev Stomatol Chir Maxillofac. 1983;84:313-21. 4. Resende LA, Dal Pai V, Alves A. Experimental study of progressive facial hemiatrophy: effects of cervical sympathectomy in animals. Rev Neurol (Paris). 1991;147:609-11. 5. O’Flynn S, Kinirons M. Parry-Romberg syndrome: a report of the dental findings in a child followed up for 9 years. Int J Paediatr Dent. 2006;16:297-301. 6. de Vasconcelos Carvalho M, do Nascimento GJ, Andrade E, et al. Association of aesthetic and orthodontic treatment in ParryRomberg syndrome. J Craniofac Surg. 2010;21:436-9. 7. Amaral TN, Marques Neto JF, Lapa AT, et al. Neurologic involvement in scleroderma en coup de sabre. Autoimmune Dis. 2012;2012:719685. 8. Claudy AL, Segault D, Rousset H. Moulin G. Facial hemiatrophy, homolateral cervical linear scleroderma and thyroid disease. Ann Dermatol Venereol. 1992;119:543-5. 9. Ruhin B, Bennaceur S, Verecke F, et al. Progressive hemifacial atrophy in the young patient: physiopathologic hypotheses, diagnosis and therapy. Rev Stomatol Chir Maxillofac. 2000;101:287-97. 10. Pichiecchio A, Uggetti C, Grazia Egitto M, et al. Parry-Romberg syndrome with migraine and intracranial aneurysm. Neurology. 2002;59:606-8. 11. Ford JG, Busbee B, Reed JW, et al. Hemifacial atrophy and primary corneal endothelial failure. Arch Ophthalmol. 1998;116:1246-8. 12. Auvinet C, Glacet-Bernard A, Coscas G, et al. Parry-Romberg progressive facial hemiatrophy and localized scleroderma: nosologic and pathogenic problems. J Fr Ophtalmol. 1989;12:169-73. 13. Lapresle J, Desi M. Scleroderma with progressive facial hemiatrophy and atrophy of the other side of the body. Rev Neurol (Paris). 1982;138:815-25. 14. Tollefson MM, Witman PM. En coup de sabre morphea and ParryRomberg syndrome: a retrospective review of 54 patients. J Am Acad Dermatol. 2007;56:257-63. 15. Jun JH, Kim HY, Jung HJ, et al. Parry-Romberg syndrome with en coup de sabre. Ann Dermatol. 2011;23:342-7. 16. Wartenburg R. Progressive facial hemiatrophy: report of 3 cases. Arch Neurol Psych. 1945;54:75. 17. Banks TL, Sugar HS. Ocular manifestations of facial hemiatrophy. Bull Mt. Sinai Hosp. 1963;11:83. 18. Mendonca J, Viana SL, Freitas F, Lima G. Late-onset progressive facial hemiatrophy (Parry-Romberg syndrome). J Postgrad Med. 2005;51:135-6. 19. Stone J. Parry-Romberg syndrome: a global survey of 205 patients using the Internet. Neurology. 2003;61:674-6.

Bilateral retinal detachment: a clue to diagnosis of HELLP syndrome HELLP syndrome is characterized by hypertension, elevated liver enzymes, and low platelets, and is a life-threatening complication during pregnancy, both for mother and fetus. Bilateral, serous, nonrhegmatogenous retinal detachment (RD) is a rare complication of pre-eclampsia/eclampsia in pregnancy. We report on the clinical course and outcome in a patient in whom sudden loss of vision from bilateral exudative RD was the presenting feature of HELLP syndrome. A 20-year-old primigravida presented to us with painless, rapid loss of vision of 3 days’ duration, associated with

20. Galanopoulos A, McNab AA. Hemifacial atrophy: an unusual cause of upper eyelid retraction. Ophthal Plast Reconstr Surg. 1995;11: 278-80. 21. Aracena T, Roca FP, Barragan M. Progressive hemifacial atrophy (Parry-Romberg syndrome): report of two cases. Ann Ophthalmol. 1979;11:953-8. 22. Ong K, Billson FA, Pathirana DS, Clifton-Bligh P. A case of progressive hemifacial atrophy with uveitis and retinal vasculitis. Aust N Z J Ophthalmol. 1991;19:295-8. 23. Bandello F, Rosa N, Ghisolfi F, et al. New findings in the ParryRomberg syndrome: a case report. Eur J Ophthalmol. 2002;12: 556-8. 24. Zamir E, Chowers I, Banin E, et al. Neurotrophic corneal endothelial failure complicating acute Horner syndrome. Ophthalmology. 1999;106:1692-6. 25. Grayson M, Pieroni D. Progressive facial hemiatrophy with bullous and band-shaped keratopathy. Am J Ophthalmol. 1970;70: 42-4. 26. Segal P, Jablonska S, Mrzyglod S. Ocular changes in linear scleroderma. Am J Ophthalmol. 1961;51:807-13. 27. Hung S, Rutar T, Lin S, et al. Severe hypotony associated with ParryRomberg syndrome. Ophthalmic Surg Lasers Imaging. 2010;9:1-3. 28. Ousterhout DK. Correction of enophthalmos in progressive hemifacial atrophy: a case report. Ophthal Plast Reconstr Surg. 1996;12: 240-4. 29. Miller MT, Spencer MA. Progressive hemifacial atrophy: a natural history study. Trans Am Ophthalmol Soc. 1995;93:203-15. 30. Nasser O, Greiner K, Amer R. Unilateral optic atrophy preceding Coats disease in a girl with Parry-Romberg syndrome. Eur J Ophthalmol. 2010;20:221-3. 31. Park DH, Kim IT. Patient with Parry-Romberg syndrome complicated by Coats’ syndrome. Jpn J Ophthalmol. 2008;52:520-2. 32. Karim A, Laghmari M, Ibrahimy W, et al. Neuroretinitis, ParryRomberg syndrome, and scleroderma. J Fr Ophtalmol. 2005;28: 866-70. 33. Derex L, Isnard H, Revol M. Progressive facial hemiatrophy with multiple benign tumors and hamartomas. Neuropediatrics. 1995;26: 306-9. 34. Sharma DC, Parihar PS, Kumawat DC, et al. Duane’s retraction syndrome with facial hemiatrophy (a case report). J Postgrad Med. 1990;36:51-3. 35. Garcher C, Humbert P, Bron A, et al. Optic neuropathy and ParryRomberg syndrome: apropos of a case. J Fr Ophtalmol. 1990;13: 557-61. 36. Aynaci FM, Sen Y, Erdol H, et al. Parry-Romberg syndrome associated with Adie’s pupil and radiologic findings. Pediatr Neurol. 2001;25:416-8. Can J Ophthalmol 2014;49:e2–e5 0008-4182/14/$-see front matter & 2014 Canadian Ophthalmological Society. Published by Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.jcjo.2013.09.010

headache and vomiting at 28 weeks of gestation. There was no history of any loss of consciousness, seizures, or weakness of limbs. Her antenatal visits at a local nursing home had been irregular, and there were no prior records of blood pressure examination. Ophthalmologic evaluation revealed a visual acuity of only perception of light in both eyes. Funduscopy showed exudative RD in both eyes (Fig. 1A, 1B). Optical coherence tomography of the retinal layers revealed neurosensory detachment from underlying retinal pigment epithelium and intraretinal cystic changes characteristic of RD in both eyes (Fig. 2A, 2B). On physical examination, she was conscious and well oriented in time, place, and person. Her pulse was 98, blood CAN J OPHTHALMOL — VOL. 49, NO. 1, FEBRUARY 2014

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Fig. 1 — A and B, Clinical photograph demonstrating bilateral exudative retinal detachment at presentation with minimal vascular and optic disc changes. C and D, Complete resolution of the detachment after delivery.

pressure was 140/100 mm Hg, and respiratory and cardiovascular systems were normal. On obstetrical examination, uterus was enlarged to 26 weeks’ size, relaxed and fetal heart was regular. Ultrasonography showed single live growth restricted fetus with severe oligohydramnios, no congenital malformation, and estimated fetal weight of 700 g. Investigations revealed hemoglobin 8.6 g%, platelet count 28,000/mm3, total leucocyte count 12,000/mm3, blood urea 64 mg%, serum creatinine 1.2 mg%, total bilirubin 0.8 mg%, aspartate aminotransferase 53 units/L, alanine aminotransferase 51 units/L, alkaline phosphatase 239 units/L, serum lactate dehydrogenase 2186 IU, and albuminuria 3þ. Coagulation profile was normal. A diagnosis of HELLP syndrome with severe Intrauterine growth retardation (IUGR) with bilateral exudative RD complicating severe pre-eclampsia was made. The patient was started on oral labetalol to control blood pressure along with magnesium sulphate infusion (Zuspan regimen) as anticonvulsant. She could receive only a single injection of betamethasone (12 mg) for fetal lung maturity as decision to immediately terminate the pregnancy in view of HELLP syndrome and severe IUGR was taken. Labour was induced as the patient’s relatives refused caesarean section in view of the poor fetal prognosis. The ophthalmologists were

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consulted before allowing a trial of labour in view of bilateral RD. The patient delivered a stillborn 767-g female fetus. She was transfused 5 units of platelets because of the low platelet count. Postdelivery, her blood pressure gradually settled and the biochemistry returned to normal within 5 days. There was significant improvement in visual acuity 2 days postdelivery. One month postdelivery, her visual acuity was 6/6 and 6/18. Funduscopy was normal except for a small, solitary intraretinal hemorrhage in the right eye. Resolution of detachment was seen both clinically and on optical coherence tomographic imaging (Fig. 1C, 1D). The latter, however, demonstrated minimal residual subretinal fluid limited to subfoveal region in both eyes (Fig. 2C, 2D). Pre-eclampsia is a hypertensive disorder of pregnancy with multiorgan involvement and in which visual system is affected in 30% to 100% cases.1,2 In patients with acute loss of vision, the causes may include such retinal vascular changes as retinal arteriolar spasm, retinal hemorrhage, serous RD, and cortical lesions.3–5 Exudative RD is observed in 1% to 2% patients with severe pre-eclampsia and 10% of patients with eclampsia.6 De Gracia and Ortega-Paz6 have reported 7 times greater risk for development of RD in women with HELLP syndrome as compared with healthy pregnant females.7 RD is usually

Correspondence

Fig. 2 — A and B, Optical coherence tomography (OCT) images at presentation showing detachment of neurosensory layer from the underlying retinal pigment epithelium (white arrow) and intraretinal cystoid changes (red arrow). C and D, OCT images 4 weeks later showing spontaneous resolution of the retinal detachment with minimal residual subretinal fluid (red arrowhead).

seen in cases of pre-eclampsia with severe hypertension, although it has also been reported in mild hypertension. Although the pathophysiology of RD is not clear, it has been proposed that terminal arteriolar spasm leads to choroidal ischemia and ischemic injury to the retinal pigment epithelium, thus breaking the blood–retinal barrier.8 Other proposed mechanisms include disseminated intravascular coagulation due to secretory products from placenta and combination of severe hypertension, microangiopathic hemolysis, and hypoalbuminemia in choroidal vessels that results in focal ischemia leading to RD.7 Management of exudative RD involves treatment of the underlying cause, which in our patient was control of hypertension and immediate delivery. Prophylactic anticonvulsants were administered, and immediate delivery was induced in view of severe pre-eclampsia. No guidelines are available in the literature regarding the preferred mode of delivery in pre-eclampsia complicated by RD. However, various series indicate higher use of caesarean deliveries as compared with vaginal deliveries.7 This may be because of the need for immediate termination of pregnancy in view of severe pre-eclampsia/eclampsia. Severe pre-eclampsia may be associated with fetal growth restriction caused by placental insufficiency and may result in asphyxia and intrauterine death. In our patient, the relatively poor prognosis of the very small fetus and the requirement of

intensive neonatal care postdelivery were explained to the patient’s relatives. They refused caesarean section and intrauterine death resulted because of severe fetal hypoxia during the course of vaginal delivery. Most women with RD show complete recovery within 2 to 12 weeks from delivery and surgical intervention is generally not required in these cases.8 Our patient recovered to a major degree spontaneously within a week. In conclusion, RD complicating severe pre-eclampsia and HELLP syndrome is a rare complication and requires prompt management for survival of both mother and child, and also to prevent permanent visual disability. Varun Gogia, Sumedha Sharma, Dipika Deka, Vatsala Dadhwal, Pradeep Venkatesh All India Institute of Medical Sciences, New Delhi, India Correspondence to: Varun Gogia, MD: [email protected] REFERENCES 1. Sibai BM. The HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets): much ado about nothing? Am J Obstet Gynecol. 1990;162:311-6. 2. Ober RR. Pregnancy-induced hypertension (pre-eclampsiaeclampsia). In: Ryan SJ, editor. Retina, Vol. 2. St. Louis, Mo.: CV Mosby; 1994: 1393-403. CAN J OPHTHALMOL — VOL. 49, NO. 1, FEBRUARY 2014

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Correspondence 3. Tung CF, Peng YC, Chen GH, Chow WK, Yang DY, Hu WH. Hemolysis, elevated liver enzymes and low platelet count (HELLP) syndrome with acute cortical blindness. Chin Med J (Taipei). 2001;64:482-5. 4. Cunningham FG, Fernandez CO, Hernandez C. Blindness associated with preeclampsia and eclampsia Am J Obstet Gynecol. 1995;172:1291-8. 5. Sreckovic SB, Janicijevic MA, Stefanovic IB, et al. Bilateral retinal detachment in a case of preeclampsia. Bosn J Basic Med Sci. 2011;11:129-31. 6. Vigil-De Gracia P, Ortega-Paz L. Retinal detachment in association with preeclampsia, eclampsia and HELLP syndrome. Int J Gynaecol Obstet. 2011;114:223-5.

7. Mohan AK, Al-Senawi RN, Mathew M, Ganesh A, Al-Mujaini A. Exudative retinal detachment in eclampsia. A management dilemma. Saudi Med J. 2009;30:302-3. 8. Sánchez Vicente JL, Ruiz Aragón J, Nanwani K, et al. Retinal detachment in preeclampsia and HELLP syndrome. Arch Soc Esp Oftalmol. 2003;78:335-8.

A rare case of ectopic cilia

Topical antibiotic (lomefloxacin, 0.3%) and steroid (loteprednol, 0.5%) were administered after excision. No recurrence was observed 3 months after excision. Wiegmann described the first case of ectopic cilia in 1936, but only 18 cases have been reported in humans.2,3 The origin of ectopic cilia is not known. It was previously hypothesized to originate as a result of meibomian substitution, but embryologic origin has been suggested by MacQuillan et al.4,5 Also, family history can be important for some cases.3 Hase et al.6 reported that ectopic cilia in the palpebral conjunctiva may be acquired. They described that ectopic cilia are associated with crypts of Henle and chronic inflammation. Two distinct types of ectopic cilia have been reported in the literature: the anterior type (cilia protrude from the anterior surface of the tarsal plate) and the posterior type (cilia protrude from the posterior surface).4,7 The posterior type presents as a single eyelash below the upper or inferior conjunctiva. We report a patient with ectopic cilia of the posterior surface similar to those cases reported by Hase et al.6 Ectopic cilia has been associated with some pathologies such as congenital distichiasis, a complex choristoma, cutaneous features of atopic eczema in the periorbital region, and preseptal cellulitis.8 No other pathology was observed in any patient. Surgical removal is the preferred treatment for ectopic cilia.9 Recurrence has been reported in 1 patient 5 months after excision, which may have been caused by an incomplete excision.10 Recurrence was not observed 3 months after excision. Inferior palpebral ectopic cilia is a rare abnormality in humans. To our knowledge, this is the second reported case in the inferior palpebral conjunctiva.

Ectopic cilia is a rare anomaly of the lashes. Eyelashes are normally found at the eyelid margin, but rare anomalies such as cilial row duplication, agenesis, and ectopic placement can be seen.1 Ectopic cilia is the most common anomaly in developmental disorders. We report a case of ectopic cilia in the inferior palpebral conjunctiva. A 22-year-old female was admitted to our clinic with moderate itching, hyperemia, and irritation of the left eye for 1 month. She had no medical history of any ocular surgery or trauma. There was no family history. Her best corrected visual acuity was 10/10 OU. The intraocular pressure was normal. Biomicroscopic examination demonstrated an eyelash located in the palpebral conjunctiva of the lower lid, conjunctival hyperemia, and mild superficial punctuate keratopathy in the left eye (Fig. 1). The anterior segment examination of the right eye was normal. The fundus examination was normal in both eyes. Ectopic cilia was diagnosed by clinical findings. It was excised including conjunctiva and tarsal plate tissue under local anaesthesia.

Can J Ophthalmol 2014;49:e5–e8 0008-4182/14/$-see front matter & 2014 Canadian Ophthalmological Society. Published by Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.jcjo.2013.09.009

Erdem Dinc, * Ozlem Yildirim † *

Elbistan State Hospital, Ophthalmology Clinic, Kahramanmaras;†Mersin University, Mersin, Turkey Fig. 1 — Ectopic cilia in the palpebral conjunctiva and hyperemia.

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Correspondence to: Erdem Dinc, MD: [email protected]

Bilateral retinal detachment: a clue to diagnosis of HELLP syndrome.

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