Congenital horizontal tarsal kink: clinical characteristics from a large series.

PURPOSE: To describe the clinical characteristics of congenital horizontal tarsal kink. METHODS: Retrospective clinical series derived from a literature review and mail survey. Characteristics included sex, race, association with systemic diseases, interval between birth and diagnosis, laterality, presence of corneal ulcer and causative infectious agent, treatment, and visual outcome. RESULTS: Twenty-five cases were reviewed. The typical patient is a white male diagnosed at 7.2 weeks of age. Right-sided and bilateral cases were most common. Ulcers were present in 12/24 patients; most were culture negative. Various methods of surgical repair were successful. Visual outcomes, in general, were favorable although amblyopia developed in 4/15 patients. CONCLUSIONS: Congenital horizontal tarsal kink is rare and its cause is unknown. A poor visual outcome usually results from a corneal ulcer scar and a delay in diagnosis. Amblyopia not related to corneal opacification can also occur.     

A simple correction for congenital tarsal kink associated with distichiasis.

Congenital tarsal kink is a rare condition. The folded edge of the upper tarsus, or the inturned lashes, may traumatize the cornea causing ulceration. We describe a case of unilateral upper eyelid horizontal tarsal kink associated with distichiasis and congenital corneal ulceration. Several different surgical treatment options have been previously alluded to in the literature including lamellar tarsoplasty, full-thickness eyelid fracture, and rotation and excision of the kink with tarsal reapposition. Repair by means of eyelid everting sutures via an anterior lamellar approach is presented as a novel technique to correct this congenital anomaly.     

Severe congenital horizontal tarsal kink without levator aponeurosis disinsertion

A newborn presented with congenital severe tarsal kink of the upper eyelids not caused by levator aponeurosis disinsertion. A bilateral anterior lamellar repositioning procedure was performed to correct the tarsal kink. Levator aponeurosis disinsertion was not observed as an intraoperative finding. The tarsal kink disappeared, and eyelid positions remained stable during a follow-up period of 1 year. Our case indicates that severe congenital tarsal kink may develop without levator aponeurosis disinsertion, and anterior lamellar repositioning technique is a simple and effective method of treatment.     

Trisomy 13: a rare case of congenital tarsal kink

We describe the management of the eyelid anomaly associated with Patau syndrome. Trisomy 13 is the genotype of the syndrome's phenotype. The eyelid anomaly was a tarsal kink, a congenital malformation of the tarsus that causes entropion. A 2-month-old white girl presented with unilateral upper eyelid entropion and central corneal ulceration. To correct this condition, two 6-0 polyglactin sutures were passed through the gray line of the upper and lower eyelids and tied. Correction of the entropion and improvement in the corneal condition were achieved after surgery. No recurrence of the entropion or corneal ulceration was noted after 2 months of follow-up. This simple technique, which corrected the eyelid malposition, providing an excellent cosmetic result without incision of the tarsus, has been previously reported in other cases of tarsal kink but not in a patient with Patau syndrome.     

探讨倾斜水平直肌止端在V型外斜中的应用

目的 探讨水平肌的斜行重置对外斜视V征的治疗效果.方法 对11例不合并斜肌异常或斜肌亢进+1的外斜视V征患者经水平肌的斜行重置治疗后,观察其术前与术后的眼位、眼肌功能、双眼视功能等.结果 所有患者V征消失,双眼球运动无障碍,无旋转不适,5例有双眼视功能改善.结论 选择合适的病例(不合并斜肌异常或斜肌亢进+1的V征),水平肌的斜行重置矫正V征是有效的术式.     

Epikeratophakia: the surgical correction of aphakia. Update: 1982

Sixty-five epikeratophakia procedures have been performed in 63 patients; visual acuity data have been tabulated on 31 patients with 4 to 30 months follow-up. Patients with more than a year of follow-up showed stable keratometry readings. Early patients achieved 70% of the predicted dioptric correction; more recent patients have achieved 87% with improvements in the lathing procedure, tissue handling, and surgical technique. Visual acuities improve with time. At any given time after surgery, acuities measured with a hard contact lens are better than those measured with spectacles; the decrease in spectacle acuity is probably a result of irregular refraction at the graft surface. The gap between contact lens and spectacle acuity decreases with time. A number of patients achieve postoperative visual acuities better than their preoperative acuities, and most achieve final spectacle acuities within a line or two of their preoperative acuities.     

Congenital ptosis. A new surgical procedure

The technique described takes existing anatomic conditions and residual function into account. No resection is required. Results after four years are very good.     

Congenital ptosis: results of surgical management

PURPOSE: To determine the visual and cosmetic outcome following the surgical correction of isolated congenital ptosis. METHODS: A retrospective review of the outcome of isolated congenital ptosis corrected under the supervision of one surgeon at The Children's Hospital, Camperdown, between January 1983 and January 1993 was examined. Some 65 patients with 80 involved eyes were identified; 30 eyes underwent a levator resection procedure, 40 eyes underwent a brow suspension using donor stored fascia lata, and in 10 eyes a brow suspension was performed using mersilene mesh. RESULTS: In 78 eyes of 63 patients, a good cosmetic result was achieved. In two patients (two eyes) a poor cosmetic result was achieved. These two patients refused further surgery following an undercorrection of their initial ptosis. The recurrence rates for the primary procedures were 16.7% for levator resection procedures, 35% for brow suspension procedures using donor fascia lata, and 30% for brow suspension surgery using mersilene mesh. Some 35.3% of eyes following mersilene slings required further surgery for granulomas and exposed mersilene mesh compared with 6% having similar complications with stored fascia lata. Nine patients (11.25%) had reduced visual acuity (one line or more on the Snellen chart or its equivalent with the other tests used) on the operated side. Only one patient was found to have significant astigmatism. CONCLUSIONS: An acceptable cosmetic result was achieved with one operation in 75.3% of cases. In 20.8% of cases a second operation was required and in 3.9% of cases three or more operations were required. This series supports the view that where possible, levator resection is the preferred form of surgery to correct congenital ptosis. When the levator function is inadequate, brow suspension is performed. The use of donor fascia lata resulted in a good cosmetic appearance with a low occurrence of surgical side effects. Amblyopia, when strictly defined, occurred in 11.25% of eyes despite early surgery for severe cases and intensive amblyopia therapy. Management requires repeated follow up for early detection and introduction of occlusion therapy or surgical ptosis correction.     

Congenital ptosis: results of surgical management

Purpose: To determine the visual and cosmetic outcome following the surgical correction of isolated congenital ptosis. Methods: A retrospective review of the outcome of isolated congenital ptosis corrected under the supervision of one surgeon at The Children's Hospital, Camperdown, between January 1983 and January 1993 was examined. Some 65 patients with 80 involved eyes were identified; 30 eyes underwent a levator resection procedure, 40 eyes underwent a brow suspension using donor stored fascia lata, and in 10 eyes a brow suspension was performed using mersilene mesh. Results: In 78 eyes of 63 patients, a good cosmetic result was achieved. In two patients (two eyes) a poor cosmetic result was achieved. These two patients refused further surgery following an undercorrection of their initial ptosis. The recurrence rates for the primary procedures were 16.7% for levator resection procedures, 35% for brow suspension procedures using donor fascia lata, and 30% for brow suspension surgery using mersilene mesh. Some 35.3% of eyes following mersilene slings required further surgery for granulomas and exposed mersilene mesh compared with 6% having similar complications with stored fascia lata. Nine patients (11.25%) had reduced visual acuity (one line or more on the Snellen chart or its equivalent with the other tests used) on the operated side. Only one patient was found to have significant astigmatism. Conclusions: An acceptable cosmetic result was achieved with one operation in 75.3% of cases. In 20.8% of cases a second operation was required and in 3.9% of cases three or more operations were required. This series supports the view that where possible, levator resection is the preferred form of surgery to correct congenital ptosis. When the levator function is inadequate, brow suspension is performed. The use of donor fascia lata resulted in a good cosmetic appearance with a low occurrence of surgical side effects. Amblyopia, when strictly defined, occurred in 11.25% of eyes despite early surgery for severe cases and intensive amblyopia therapy. Management requires repeated follow up for early detection and introduction of occlusion therapy or surgical ptosis correction.     

The effects of surgical technique and the radius of the eye on correction for horizontal strabismus

A theory is developed that considers the effects of surgical technique and the radius of the eye on the values for the surgical amount that correct horizontal strabismus. By replacing the biologic system of the eye and the extraocular muscles with an idealized model eye, equations are derived that relate to the surgical amount. Using these equations, it is possible to show that small variations in the radius of the eyeball can have a significant effect on the surgical amount. It is also shown that for a recession, variations in the distance between the point where the sutures are tied on the muscle, before disinserting it, and the original insertion point of the muscle can have a significant effect on the final outcome of the surgery.     

Binocularity following surgical correction of strabismus in adults.

PURPOSE: This is a retrospective study to determine the pre- and postoperative binocular status of adults who have undergone surgical correction of strabismus. METHODS: A list of all consecutive adult patients who underwent surgery for strabismus between June 1990 and December 2001 (surgery by MBM) was compiled and their medical charts were reviewed. Patients who had at least 6 weeks postoperative follow-up were included if their charts were available for review and if information on binocularity was recorded. Binocularity was measured by the Titmus stereo test (Stereo Optical Co., Inc., Chicago), and/or the Worth 4-Dot test for distance (Scioto Medical Products, Powell, OH [discontinued]), and/or the Worth 4-Dot near test (Gulden Ophthalmics, Inc., Elkin Park, PA). Improvements or decreases in stereo acuity were noted 6 weeks postoperatively and at the final office visit. Prism management was incorporated pre- and/or postoperatively in some of our patients. A total of 112 patients underwent surgery for strabismus. Of these, 72 patients, aged 16 to 80 years, were included; 24 were excluded as their charts did not contain any information on stereo acuity 6 weeks postoperatively or were lost to follow-up after surgery, and 16 were excluded because their charts were unavailable from storage. RESULTS: The mean follow-up for the study patients was 16.5 months (range 6 weeks to 79 months). Overall, 30 of the 72 patients improved in binocular function (42%), 38 remained the same (53%), and 4 decreased (5%). If you exclude those patients with 40 sec of stereo acuity preoperatively whose stereopsis, by definition, could not improve further, and look only at the 59 surgical patients who could improve, then 51% improved. A variety of surgical procedures was performed, the most common being a bilateral medial rectus recession. Eleven patients required a second surgical procedure and two required a third surgical procedure. CONCLUSION: The benefits of surgical correction of strabismus in adults include improvement in binocular function as seen in 51% of the patients in the study.     

Stability of one-stage adjustable suture for the correction of horizontal strabismus.

One-stage adjustable suture for strabismus correction, with the whole operation done under topical anaesthesia and adjustment done on the table, was performed on 45 consecutive patients. The stability of the post-adjustment result was studied by comparing the post-adjustment deviation on the operating table to that at six weeks and three months after operation. The stability was comparable to that following the usual two-stage adjustable suture. The original angle of deviation and the fusion status were found to have no bearing on the stability of the procedure.     

Congenital simple hamartoma of the retinal pigment epithelium: a study of five cases

PURPOSE: To report 5 patients with presumed congenital, simple hamartoma of the retinal pigment epithelium (RPE). DESIGN: Retrospective, observational small case series. PARTICIPANTS: Five patients with a black macular tumor of the RPE. MAIN OUTCOME MEASURES: Initial tumor features, initial associated fundus features, tumor course on follow-up, and visual acuity outcome. RESULTS: In all 5 cases, the tumor involved the macula and the mean distance to the foveola was 0.4 mm (median, 0.2 mm; range, 0.2-1.2 mm). In all cases, the tumor was darkly pigmented, with full-thickness retinal and RPE involvement and minimal protrusion into the vitreous cavity. The mean ultrasonographic tumor thickness was 1.6 mm (median, 1.4 mm; range, 1.1-2.5 mm). Associated features included minimally dilated retinal feeding artery and draining vein (100%), surrounding mild retinal traction (80%), retinal exudation (20%), and vitreous pigmented cells (20%). None of the lesions had associated subretinal fluid, macular edema, or macular hole. Visual acuity was 20/20 in 3 cases and slightly decreased in the other 2 cases because of foveal traction. The findings remained stable in 3 patients who had adequate follow-up. We chose to classify this lesion as congenital simple hamartoma of the RPE to differentiate it from combined hamartoma of the retina and RPE, congenital hypertrophy of the RPE, adenoma or adenocarcinoma of the RPE, and acquired hyperplasia of the RPE, which are different clinical entities. CONCLUSIONS: Congenital hamartoma of the RPE is typically a black, full-thickness retinal mass, often adjacent to the foveola. Despite such location, the visual acuity was affected minimally.     

Congenital cystic eye: recurrence after initial surgical removal

Congenital cystic eye arises from failure of invagination of the primary optic vesicle. The cyst lacks the usual intraocular structures and contains only abortive derivatives of the anterior and posterior portions of the primary optic vesicle. Because the cystic eye usually occupies a large portion of the orbit and distends the upper eyelid, surgical excision is often carried out. We report a case of congenital cystic eye in which an initial attempt at excision was followed in three months by recurrence of the cyst in the orbit. A second excision appears to have eliminated the cyst since no recurrence has become evident in the ensuing two years. The pathology of the cyst from both excision specimens suggests that the pigmented cells from the posterior portion of the cyst were not included in the first resection. Based on this experience, we conclude that every effort should be made to excise all of the congenital cystic eye when surgical removal is undertaken.