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We report four members of a Lebanese Druze family with the syndrome of lens dislocation, spontaneous filtering blebs, anterior segment abnormalities, and a distinctive facial appearance. The constellation of clinical abnormalities in these patients is not suggestive of the Marfan syndrome or other connective tissue disorders associated with ectopia lentis. We previously described this syndrome in another presumably unrelated and highly inbred Druze family from the mountains of Lebanon. We postulated autosomal recessive inheritance in a pseudo-dominant pedigree. A few isolated reports of similar cases are scattered in the world literature. We now confirm that this is a distinct autosomal recessive syndrome whose gene mutation is enriched in the Lebanese Druze community.

Purpose: To report the ultrasound biomicroscopy (UBM) and surgical findings in a subject with a syndrome of ectopia lentis, spontaneous filtering blebs, and craniofacial dysmorphism (Traboulsi syndrome). Methods: Case report, using a 40-MHz UBM wide-field anterior segment scan and anterior segment optical coherence tomography (OCT). Results: A 16-year-old orphan girl presented with visual loss to the level of 6/60 (20/200) bilaterally. She had a central corneal opacification with retrocorneal fibrosis. The anterior chamber was flat with a very poorly dilating pupil. The lens was central in location. Perilimbal conjunctival blebs were bilateral with an intraocular pressure of 8 mm Hg. UBM and anterior segment OCT revealed chronic apposition of the iris to the cornea with angle closure, delineation of the bleb tract and rarefaction of the zonules. The girl had abnormal facial features (a beaked nose and long face) with normal chromosomal studies, negative fluorescent in situ hybridization study for velocardiofacial syndrome and an absence of signs suggesting Marfan syndrome. Under general anesthesia, attempts at deepening the anterior chamber with sodium hyaluronate 3% led to a spontaneous dislocation of the lens into the anterior chamber, facilitating its aspiration. Deepening of the angle was found after lens removal. Retrocorneal fibrosis persisted after surgery, but the bleb height decreased. Best corrected visual acuity did not improve from the preoperative level beyond 6/60 (20/200) because of central retrocorneal fibrosis. Conclusions: Early surgical removal of the lens is necessary in this syndrome to avoid irreversible corneal and trabecular meshwork damage in chronic apposition of the iris to the cornea. UBM can help in the delineation of the bleb tract and document resolution of angle closure after surgery.

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SYNOPSIS Human 0 erythrocytes, treated with 1% formalin and subsequently exposed simultaneously to hydatid protein antigen and 0-1% chromic chloride, acquire the property of agglutinating on a slide when brought in contact with specific antiserum. This constitutes the basis of the slide haemagglutination test, an easy, quick, sensitive, and specific procedure, useful in the diagnosis of hydatid disease.In previous communications (Mamo and Dakroub, 1974;Mamo et al, 1975) we described a diagnostic method based on the principle of binding hydatid antigen to human erythrocytes with the use of chromic chloride. When mixed on a slide with specific antiserum the sensitized cells produced a visible haemagglutination. Comparison of the slide haemagglutination (SHA) test with the tube method of Garabedian et al (1957) showed that the two procedures are equally sensitive and specific. We present the results of a correlative study of the SHA, tube haemagglutination (THA), complement-fixation (CF), and indirect fluorescent (IFA) tests in the diagnosis of patients with hydatidosis. Materials and Methods BLOOD SAMPLES Serum samples were obtained from: 1. 57 patients with hydatid disease; of these, 32 had hepatic and 25 pulmonary cysts; 2. 24 postoperative patients with no demonstrable cysts one year after surgery; 3. 100 controls; of these 50 were healthy adults and the rest were patients admitted to the hospital for miscellaneous conditions other than hydatid disease. All samples were stored at -20°C. PROCEDURESSlide Haemagglutination (SHA) Test This followed the method of Gold and Fudenberg (1967) with a number of modifications. Fresh human 0 Rh positive or negative blood, in Alsever's solution, was washed three times in 09% saline, and the erythrocytes were packed by centrifugation.Received for publication 15 July 1975. Five drops of the packed cells were next suspended in 10 ml of 1 % formalin in saline and left at room temperature for 10 minutes. The erythrocytes were washed twice with saline and re-packed. Five drops of saline were added to the cells to make up a 50% suspension. This was added to 2-5 ml of potent hydatid fluid antigen in a flask and, immediately after, 1 ml of 0-1 % freshly prepared chromic chloride solution in saline (CrCI3, 6H20-MW 266 45-BDH) was added. The mixture was gently shaken for exactly 4 minutes, after which the erythrocytes were washed twice in saline and packed. Fifteen drops of saline were added to the cells to constitute a 25 % suspension. The sensitized cells retained their potency for about a week. Variations in the potency of the sensitized cells were at times observed with erythrocytes obtained from different donors. This necessitated testing multiple samples of blood.Serum dilutions of 1:10, 1:100, and 1:500 in saline were prepared in tubes. One drop of each dilution was added to a properly labelled area on a slide. One drop of the sensitized cell suspension was added to each dilution of the test sera and mixed with a wooden applicator. One drop of serum at 1:10, mixed with norma...