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One-Stage Correction for Blepharophimosis Syndrome

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One-Stage Correction for Blepharophimosis Syndrome Eye (2008) 22, 380–388 & 2008 Nature Publishing Group All rights reserved 0950-222X/08 $30.00 www.nature.com/eye CLINICAL STUDY One-stage S-Y Wu, L Ma, Y-J Tsai and JZ-C Kuo correction for blepharophimosis syndrome Abstract Introduction Purpose To classify the severity of Blepharophimosis-ptosis-epicanthus inversus blepharophimosis, describe associated (BPES) syndrome is a rare congenital disorder features and their effects on the incidence of and occurs either as an autosomal dominant amblyopia and to recommend guidelines for trait or sporadically.1–6 In 1971, Kohn and surgical treatment and management of surgical Romano2 described the tetrad of this complications. syndrome, which includes blepharophimosis, Methods The case records of 23 patients with blepharoptosis, epicanthus inversus, and blepharophimosis syndrome were examined telecanthus. It is also associated with ocular, retrospectively. Patients’ photographs and lacrimal, nasal, and auricular anomalies.4–6 measurements were reviewed to analyse the There are two subtypes of this syndrome severity of blepharophimosis, surgical proposed by Zlotogora et al:3 type I is more techniques undertaken, surgical outcomes, prevalent and affected female subjects are and complications. Statistical analyses were infertile and type II is transmitted both in performed using paired-sample t-tests to affected male and female subjects.3 evaluate the surgical outcome and Spearman Blepharophimosis syndrome (BPES) has a correlation to examine the influence of great impact on a patient’s functional status and blepharophimosis on the interpalpebral may cause poor visual development.7 Although fissure height (PFH). previous studies describe the demography and Results Eighteen out of 23 (78%) patients aetiology of children with blepharophimosis underwent one-stage surgery before the age syndrome,1–6,8–11 there is a little information in of 5 years. About 31% of these patients the ophthalmic literature regarding the severity had amblyopia. Only two patients had a of the syndrome, surgical outcomes, and blepharophimosis ratio greater than 1.5 as complications.12–17 Our study focuses on a poor result. Two out of 18 (11%) patients with blepharophimosis syndrome cohort undergoing PFHs more than 2 mm needed a repeat Department of one-stage surgical correction in a tertiary Ophthalmology, Division of operation, but all five (100%) patients with referral oculoplastic centre. Orbital and Ophthalmic s less than 2 mm (very severe ptosis) needed Plastic Surgery, Chang Gung repeat operations. University College of Conclusions The one-stage corrective Methods Medicine, Chang Gung procedure provided acceptable results Memorial Hospital, This is a retrospective, interventional case series both in function and cosmesis. However, Kewi-Shan, Taoyuan, report. Medical records of all patients who patients with very severe ptosis required Taiwan, ROC underwent one-stage surgical correction for multiple stages of reconstruction for ptosis blepharophimosis syndrome at Chang-Gung Correspondence: L Ma, correction at an earlier age, after which Memorial Hospital between 1 January 1984 and Tel: 886 3 3281200 correction of telecanthus and small ext 8666; 31 December 2004 were reviewed. The study horizontal palpebral fissure length followed Fax: 886 3 3287798. complied with the policies of the institutional at an older age. E-mail: [email protected] review board, and permission was granted to Eye (2008) 22, 380–388; doi:10.1038/sj.eye.6702644; conduct the study. Data regarding visual acuity, Received: 21 May 2006 published online 17 November 2006 refraction, results of orthoptic examinations, Accepted in revised form: 1 October 2006 vertical interpalpebral fissure height (IPFH), Published online: Keywords: blepharophimosis; ptosis; epicanthus horizontal palpebral fissure length (HPFL), 17 November 2006 inversus; telecanthus; amblyopia inner intercanthal distance (IICD) were Correction of blepharophimosis S-Y Wu et al 381 recorded and analysed. Amblyopia was defined as the The skin was marked with the modified ‘Mustarde best refracted visual acuity of 20/40 or less in the affected technique’ figure for correcting epicanthus inversus, and eye and two lines of difference on the Snellen chart.7,18 with lid crease lines for levator resection or frontalis The severity of ptosis was classified as mild, moderate or suspension.16,17 For Mustarde technique, the desired final severe, based on the criteria described by Freuh.19 We position of the canthus in most patients was also defined very severe ptosis as less than 2 mm of approximately half the distance from the centre of the palpebral aperture. Levator function was measured as pupil to the centre of the nasal bridge. As the IICD of the maximum lid excursion from maximal down gaze to Asian children is nearly 28 mm,21,22 we used 24 mm as up gaze, with frontal function abolished. This was the desired intercanthal distance for all patients during recorded as poor if it was less than 4 mm, as fair if it was the operation (Figure 1a), allowing for overcorrection. between 4 and 8 mm and good if it was more than 8 mm. Our first step was to perform a lateral canthotomy of According to the normal range for IICD and 5 mm to elongate the narrow horizontal palpebral fissure. interorbital distance in the Chinese population,20–22 the Then, we sutured the conjunctiva to the eyelid raw edge ratio between the IICD and HPFL was approximately with polyglactin 6–0 (Vicryl) to prevent adhesion 1–1.2. We defined a ratio from 1.3 to 1.5 as mild (Figure 1b). The modified Mustarde flaps were incised blepharophimosis, 1.5–1.8 as moderate blepharophimosis and the fibrous tissue beneath the fold was removed. The and greater than 1.8 as severe blepharophimosis. All entire medial canthal area was mobilised by making a patients had the same senior surgeon (Dr Lih Ma) and vertical incision into the periosteum just medial to the surgery was performed with the patient under general anterior lacrimal crest. The medial canthal tendon was anaesthesia. The criteria for surgical results for clearly identified. An anchorage hole for the wire was blepharophimosis was classified as good if the ratio located superior to the level of medial canthal tendon between the IICD and HPFL was less than 1.3, as and carefully prepared with an otology burr (Figure 1c). suboptimal if the ratio was between 1.3 and 1.5, as poor if The wire passer required a 19-G needle. The needle on the ratio was more than 1.5. The surgical outcome for the wire suture imbricated the temporal aspect of the ptosis was judged as good, moderate, or poor, based on medial canthal tendon. The 2–0 surgical wire was passed the criteria described by Manners.23 A poor outcome to the contralateral side by a curved-angle needle. The often led to the decision to reoperate. contralateral medial canthal tendon was imbricated with the wire and tightening of the suture pulled both tendons toward the midline. The wires were then twisted over the Surgical technique intervening bone. The cut ends were placed inside the One-stage surgical correction consisted of lateral contralateral hole so there was less wire under the canthotomy, medial canthoplasty, transnasal wiring palpebral skin surface. The surgical wire was secured to for medial canthal tendon shortening, and frontalis two sides of medial canthal tendon so we could adjust suspension using donor fascia lata or levator resection. the distance between two sides (Figure 1d). The skin of Figure 1 Operative procedure. (a) Skin markings. (b) Lateral canthotomy and suturing the conjunctiva to the eyelid cutting edge with polyglactin 6–0 (Vicryl) to prevent adhesion. (c) Skin hooks gently retract the flaps to expose the medial canthal structures. Fibrous tissues are exposed and excised. An anchorage hole for the wire is located superior to the level of medial canthal tendon. (d) The surgical wire is secured to two sides of the medial canthal tendon so as to adjust the distance between two sides. (e) Dissection of the levator aponeurosis from the septum and conjunctiva to show Whitnall’s ligament. Placement of a double-armed, 4–0 Vicryl suture at the tarsus through the aponeurosis above Whitnall’s ligament. (f) Removing the redundant levator aponeurosis. Eye Correction of blepharophimosis S-Y Wu et al 382 medial commissure was sutured to the deep tissue and and five (29%) patients had amblyopia. Pertinent data are desired canthal skin point with 4–0 Vicryl. The skin flaps shown in Table 1. were trimmed and sewn into position with a 6–0 Vicryl Patient age at surgery ranged from 16 months to suture. Our methods emphasised not only the design of 20 years (mean, 5.1 years). Eighteen of 23 (78%) patients incision, but also firm and symmetrical attachments to underwent one-stage surgery before the age of 5 years. shorten the lengthened medial canthal tendon to its The minimal postoperative observation period was insertion. All of these procedures were performed 6 months, and the maximum postoperative follow-up was depending on the degree of severity of blepharophimosis 228 months (mean, 50.43 months). Seventeen patients syndrome. (74%) with severe blepharophimosis underwent lateral The surgical approach to congenital ptosis is based on canthotomy, transnasal wiring, medial canthoplasty, and the amount of levator function. In all cases, the distance ptosis surgeries in one stage (Table 2). The surgical time between the upper lid margin and the papillary reflex for frontalis suspension group was 165–315 min (mean, was 1 mm or less, with poor levator function of 4 mm or 230 min) and only medial canthoplasty and frontalis sling less, requiring frontalis suspension as the operation of was 165 min. The mean surgical time for the levator choice. A double rhomboid technique with freeze-dried resection group was 258 min (range, 250–270 min). The human cadaveric fascia was used in almost all patients.24 mean IICD improved from 38.17 mm preoperatively to Only five patients underwent external maximal levator 31.52 mm postoperatively, with a mean difference of resection.
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