Moshfeghi TE, Merchant TE, Pratt CB. Multiagent chemotherapy as neoadjuvant treat- ment for multifocal intraocular retinoblastoma. Ophthalmology. 2001;108:2106-2115. 9. Madreperla SA, Hungerford JL, Cooling RJ, Sullivan P, Gregor Z. Repair of late retinal de- tachment after successful treatment of retinoblastoma. Retina. 2000;20:28-32. 10. Baumal CR, Shields CL, Shields JA, Tasman WS. Surgical repair of rhegmatogenous retinal de- tachment after treatment for retinoblastoma. Ophthalmology. 1998;105:2134-2139. 11. Honavar SG, Shields CL, Shields JA, Demirci H, Naduvilath TJ. Intraocular surgery after treat- ment of retinoblastoma. Arch Ophthalmol. 2001; 119:1613-1621. 12. Portellos M, Buckley EG. Cataract surgery and intraocular lens implantation in patients with retinoblastoma. Arch Ophthalmol. 1998;116: 449-452. Idiopathic CD4  Lymphocytopenia and Sjogren Syndrome Idiopathic CD4  lymphocytopenia (ICL) is a rare syndrome that is marked by a CD4  count that is less than 300 cells/mm 3 without human immunodeficiency virus infection. 1 Its course differs from that of AIDS in that although patients with this dis- order may develop opportunistic in- fections, the majority of them re- main stable. No transmissible agent has been implicated in the pathogen- esis of ICL. The ocular manifesta- tions of ICL have only rarely been de- scribed, 2 and there are no reports of ICL in ophthalmology literature. We report the case of a patient with ICL and Sjogren syndrome. Report of a Case. A 52-year-old woman was referred to the ophthal- mology department because of a sev- eral-year history of burning and sting- ing in both eyes. Her medical history was significant for ICL, with 5 CD4  counts during 6 years ranging from 93 to 253, despite 3 negative human immunodeficiency virus test re- sults. Additionally, assays for Epstein- Barr virus, cytomegalovirus, and hu- man herpesviruses 6 and 8 were all negative. At the time, her visual acu- ity was 20/20 OD and 20/25 OS. The patient had marked superficial punc- tate keratitis and abundant mucus production in both eyes, and as a re- sult, she began a course of applying artificial tears to both eyes every 2 hours with only minimal relief. During the ensuing months, a bandage contact lens was placed over the patient’s left eye, but it failed to relieve her symptoms. Schirmer test- ing with topical anesthesia showed 6 mm of tearing in the right eye and 5.5 mm in the left. Subsequent bi- lateral inferior punctual plug place- ment provided some relief, but her symptoms and superficial punctate keratitis persisted. In addition to the aggressive use of artificial tears, other modalities (corticosteroid eye drops and systemic doxycycline adminis- tration) were employed, but the pa- tient’s condition did not improve. A diagnosis of Sjogren syndrome was confirmed after testing showed a Sjogren syndrome antigen anti- body level of 13.1 (range, 0-4.9 U/mL). The patient then began a course of cevimeline hydrochloride (30 mg by mouth 3 times a day), and her symptoms improved consider- ably. Furthermore, her superficial punctate keratitis diminished appre- ciably. She remains stable and com- fortable on this regimen with the use of artificial tears 4 times per day. Comment. Idiopathic CD4  lym- phocytopenia is a rare disorder of CD4  lymphocytopenia without hu- man immunodeficiency virus infec- tion. The ophthalmic sequelae of this syndrome have not yet been eluci- dated. In this report, we describe the characteristics and clinical courses of a patient with ICL and Sjogren syndrome. The underlying pathophysiology of ICL results from apoptosis of CD4  cells, 3 with subsequent limitations on the repertoire of the T-cell population. 4 Autoimmune processes such as Sjo- gren syndrome may result from restric- tion of T-cell diversity, which may lead to a subsequent decrease in immune surveillance. This scenario would al- low autoantibodies that may otherwise be cleared from systemic circulation to flourish. Kirtava et al 2 found an in- creased prevalence of ICL among pa- tients with Sjogren syndrome. Insummary,bothophthalmologists and internists should be aware of the connection between Sjogren syndrome and patients with ICL. Further evalu- ation is necessary to determine other ocular manifestations of ICL. Financial Disclosure: None. Correspondence: Dr Chu, Doc- tor’s Office Center, Sixth Floor, 90 Bergen St, Newark, NJ 07103 (chuda @umdnj.edu). 1. Ho DD, Cao Y, Zhu T, et al. Idiopathic CD4  T- lymphocytopenia: immunodeficiency without evidence of HIV infection. N Engl J Med. 1993; 328:380-385. 2. Kirtava Z, Blomberg J, Bredberg A, et al. CD4  T-lymphocytopenia without HIV infection: in- crease prevalence among patients with primary Sjogren’s syndrome. Clin Exp Rheumatol. 1995; 13:609-616. 3. Laurence J, Mitra D, Steiner M, et al. Apoptotic depletion of CD4  T-cells in idiopathic CD4  T-lymphocytopenia. J Clin Invest. 1996;97:672- 680. 4. Signorini S, Pirovano S, Fiorentini S, et al. Re- striction of T-cell receptor repertoires in idio- pathic CD4  lymphocytopenia. Br J Haematol. 2000;110:434-437. Advanced Keratomalacia With Descemetocele in an Infant With Cystic Fibrosis Xerophthalmia refers to the spec- trum of ocular manifestations of vi- tamin A deficiency. It represents the leading cause of childhood blind- ness worldwide but is uncommon in industrialized countries, 1 where xe- rophthalmia is more often the re- sult of malabsorption than malnu- trition due to poverty. Cystic fibrosis (CF) is an autosomal recessive dis- ease with hyperviscosity of mucus secretions causing chronic pulmo- nary changes and pancreatic insuf- ficiency. Anderson 2 was the first to note the association between xe- rophthalmia and CF, now thought to be due to fat malabsorption re- sulting in fat-soluble vitamin defi- ciency. Advanced xerophthalmia has been reported as an initial sign of CF. 3,4 A recent review article 5 sum- marized the ocular findings of CF to include xerophthalmia, tear film ab- normalities, papilledema, and nyc- talopia. To our knowledge, this is the first clinicopathologic report of kera- tomalacia with a descemetocele re- quiring keratoplasty as the initial manifestation of CF. Report of a Case. A 5-month-old girl from Juarez, Mexico, was admitted to a hospital in Las Cruces, NM, with Edward J. Wladis, MD Rajendra Kapila, MD David S. Chu, MD (REPRINTED) ARCH OPHTHALMOL / VOL 123, JULY 2005 WWW.ARCHOPHTHALMOL.COM 1012 ©2005 American Medical Association. 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