Sarcoidosis presenting as multilobular limbal corneal nodules.

PURPOSE: To review reported external ocular manifestations of sarcoidosis and to present bilateral, multilobular, nodular, limbal, corneal nodules as being a unique manifestation of the disease. PATIENTS AND METHODS: A 16-year-old Saudi girl presented with bilateral, multilobular, solid, limbal nodules, with a vascular supply from the conjunctival vessel, and associated membraneous conjunctivitis and healed trachoma. The Schirmer's test revealed less than 2 mm in both eyes with tear meniscus less than 2 mm. Biopsy of an associated palpebral conjunctival nodule was performed, in addition to a gallium scan, chest X-ray, and a serum angiotensin-converting enzyme (SACE) level. RESULTS: The culture showed beta-hemolytic streptococci. Gallium scan showed intake by both lacrimal glands. Her chest X-ray results were normal, as was the SACE level. Biopsy of the excised conjunctival nodule disclosed a noncaseating granulomatous reaction with epithelioid and giant cells, and chronic inflammatory cell infiltrate confirming a diagnosis of sarcoidosis. CONCLUSION: A multilobular, nodular, perilimbal mass as a unique manifestation of sarcoidosis is presented. A streptococcal membraneous conjunctivitis and healed trachoma superimposed. A review of sarcoidosis of the external eye is included.

Ocular sarcoidosis in Kuwait with a review of literature.

OBJECTIVE: To study 18 cases of sarcoidosis, the hall mark of which was uveitis. SETTING: Referrals from peripheral eye clinics. PATIENTS: 18 patients with bilateral almost symmetric uveitis, negative Mantoux test and positive gallium scan were enrolled in the study. Preliminary bronchoscopy and bronchial lavage were not done due to social habits. RESULTS: Age ranged between 7-48 years with median 15 and mean 19 years. Although the patients were multinational, yet all of them were residents of Kuwait. A father and son were affected within 8 months period. 78% were strictly ocular, associated pulmonary and salivary gland affection 11% each. Sole anterior uveitis was found in 28%, associated with intermediate uveitis in 55% and with posterior in 16.6% of cases. Clinically detectable dry eyes associated 33% of cases. All our patients developed glaucoma which resolved with treatment of uveitis in 88% of them. Gallium uptake of the eye balls was found in 22%, of the lacrimal glands (panda sign) in 67%, and of the salivary glands or chest 11% each. Positive biopsy was found in 72%, 22% of which was conjunctival. Chest X-ray and SACE were positive in 11%. 61% had hypergammaglobulinaemia and all had negative ANA and RF. CONCLUSIONS: (1) 61% of ocular sarcoidosis presented below sarcoid age. (2) Multinationality together with father's and son's affection indicate a climatic or environmental insult in an already predisposed person. (3) Routine chest X-ray and SACE may not be adequate for diagnosis of ocular sarcoidosis. Gallium should be done in suspected cases. (4) Follow-up for prospects is emphasized.

Congenital alacrima without associated manifestations (AD). An affected father and son.

The authors report a Kuwaiti Arab family in which the father and one of his two sons have severe hypolacrimation with blotchy staining of the cornea and punctate staining of the interpalpebral bulbar conjunctiva by fluorescein and Rose Bengal. Pharmacologic testing together with biochemical analysis and systemic examinations and investigations suggest an isolated dysfunction of lacrimation. The authors' small Arab family differs from the only other recorded pedigree (Irish) in which all five affected members in four generations also had atopy.

Central pulverulent (Coppock) cataracts. A sibship of two Arab females with full-cousin normal parents.

Two Arab (Saudi) sisters are described each with bilateral typical central pulverulent (powdery) or Coppock cataracts. As their unaffected parents are first cousins, the heredity is probably autosomal recessive, unlike the autosomal dominant heredity of the vast majority of previously described cases in the literature. Chromosomes were normal in all four individuals. There are no other children in the family. Both children and both parents were phenotypically Fy a - b+, reasonably common in Arab populations, so that any linkage to the Duffy blood group is neither supported nor refuted.

Herpetic stromal keratitis in congenital dysgammaglobulinemia. Case report.

The clinical picture and laboratory findings of a case of secondary herpes simplex virus type 2 in a patient with congenital dysgammaglobulinemia who was followed for 26 months is described. Local combined therapy of acyclovir and Decadron (dexamethasone) 0.1% was given for management for 14 months. The clinical and immunopathologic findings of our patient match the results described by other investigators in experimental animals.

A syndrome of multiple fundal anomalies in siblings with microcephaly without mental retardation.

Chorioretinal and retinal anomalies, among others, have been described in association with true autosomal recessive microcephaly. Accompanying hypertrophy of retinal pigment epithelium has been described in association with Gardner's syndrome. We present the cases of three siblings (two boys and one girl) with true autosomal recessive microcephaly without mental retardation and without associated systemic anomalies who showed hypertrophy of the retinal pigment epithelium similar to that described in Gardner's syndrome. In the boys, these spots were located on a background of fine variations in pigment, with scattered depigmented atrophic areas revealing the sclera in the peripheral and midperipheral fundus. Superadded white spots were located in front of the retinal vessels. The younger boy also had discrete patches of chorioretinal atrophy of 0.5 to 1 disc diameter. The girl, in addition to the hyperpigmented spots, had a diffuse area of chorioretinal atrophy. The anomalies described above suggest that congenital hypertrophy of the retinal pigment epithelium is not unique to Gardner's syndrome.

Aniridia, ectopia lentis, abnormal upper incisors and mental retardation--an autosomal recessive syndrome.

A 13-year-old boy presented with bilateral aniridia, ectopia lentis, two palatally placed upper incisors which may be supernumerary teeth or may be malpositioned lateral incisors, and mental retardation. The cause is very probably a single autosomal recessive gene defect although the mental state may have a separate, genetic cause. Since his parents, who are normal, are second cousins and his father's and his mother's parents are also full cousins, the coefficient of inbreeding is 1/64 which is only 25% of the coefficient for full cousinhood of parents alone (1/16). No biochemical correlate has been identified.

Etiologic role of infectious agents in uveitis.

A prospective clinical and serological study of 120 consecutive patients with anterior uveitis, 15 with posterior uveitis, and 110 healthy volunteers was carried out. It appeared from our findings that infections such as syphilis and brucellosis should be suspected in non-responsive cases of anterior uveitis. Furthermore, a highly significant frequency (41%) of seropositive HSV antibodies in our patients would suggest the possibility of its role in the etiology of uveitis. In addition, specific treatment in presumptive intraocular infections would lead to marked improvement in terms of intensity and duration of the eye inflammatory process. The results also indicate that Toxoplasmosis is almost invariably associated with posterior focal uveitis.

Ocular injuries from carbonated soft drink bottle explosions.

Sixteen cases of ocular injuries serious enough to require admission to Ibn-Sina Hospital, Kuwait, Arabian Gulf, due to explosion of glass bottles of carbonated soft drinks are reported over a period of 14 months from the beginning of July 1981 to the end of August 1982. Prevalence was much greater in the summer months and among children. Explosions of bottles without prior agitation occurred in 11 cases (68.7%). High environmental temperature and defective bottles were the most important predisposing factors. Preventive measures we suggest are better standards for manufacturers, more careful inspection of returnable bottles to detect defective ones, a separate detailed warning label on all bottles, and health education especially of school children about this and other risks of serious injury to the eyes and other parts of the body.