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Anterior capsulotomy of dislocated lenses in Marchesani syndrome using a Nd:YAG laser
Anterior capsulotomy of dislocated lenses in Marchesani syndrome using a Nd:YAG laser Paul M. Woodward, M. D. Middletown, Ohio ABSTRACT A method of bilateral lens removal in a patient with Marchesani syndrome is described. The patient had nasally dislocated spherical lenses, shallow anterior chambers, brachymorphism, systolic heart murmur, and joint anomalies. Anterior capsulotomies were performed using a mode-locked Nd:YAG laser. The lenses were removed by irrigation-aspiration without complications.
Fig. 1.
(Woodward) Right eye in patient with Marchesani syndrome, showing microspherophakia with nasal ectopia. Zonules on temporal side are practically nonexistent.
Fig. 2.
(Woodward) Left eye of same patient.
Fig. 3.
(Woodward) Serpiginous hyperpigmented lesions near macula of left eye.
Key Words: anterior capsulotomy, bilateral lens removal, Marchesani syndrome, Nd:YAG laser
CASE REPORT A 28-year-old white male who had been followed for myopia and dislocated lenses since age five years was seen because of blurred vision in his left eye of two months duration. Besides the blurred vision, he had been experiencing blurry spells with each eye for the previous year or longer. From age five years until the present, his refractive error changed from -9.00 -3.00 x 10 to -21.00 -2.00 x 10 in his right eye and from -9.00 -3.00 x 170 to -21.00 -2.00 x 170 in his left eye. With these corrections, his visual acuity was 20/20 in the right eye and 20/25 in the left eye. Ocular examination revealed a pupil diameter of 2.5 mm, blue irides, iridodonesis, shallow anterior chambers, and nasal dislocation of small, spherical lenses (Figures 1 and 2). Intraocular pressures (lOPs) were 18 mm Hg by Goldmann applanation. In the left eye, there was a serpiginous, hyperpigmented fundus lesion superonasal and inferior to the fovea and a depigmented area superotemporal to the fovea (Figure 3). The right fundus was normal. Gonioscopy showed a critically narrow chamber angle that deepened when mydriatic (2~% phenylephrine hydrochloride) drops were used to dilate the pupils to 7~ mm. The width of the angle appeared normal after dilating the pupils.
Reprint requests to Paul M. Woodward, M.D., 500 South Breiel Boulevard, Middletown, Ohio 45042.
AM INTRA-OCULAR IMPLANT SOC J-VOL 10, SPRING 1984
215
After dilation the lenses were noted to be clear, spherical, and dislocated nasally. The vitreous face was intact and only vestiges of the zonules were visible. Axial length measurement (A-Scan) was 24.45 mm in the right eye and 24.47 mm in the left eye. Keratometry measured 44.75 by 49.50 diopters (D), steep meridian at axis 100 degrees for the right eye; 44.00 by 48.75 D, steep meridian at axis 80 degrees for the left eye. General physical examination revealed the patient's height to be 63 inches. He was noted to be quite muscular although his work rarely required lifting and he did not exercise (Figure 4). His hands were spadelike with short stubby fingers and enlarged knuckles. His elbows could not be extended beyond 20 degrees. He was noted to walk on his toes because of restriction of flexion of the ankle joints. He had a loud systolic murmur.
Marchesani syndrome made lens removal desirable ifit could be safely performed. The axial length measurements and dioptric power of his corneas indicated that the ultimate refractive error in each eye would be +6.75 D if his lenses were simply removed. This seemed preferable to the highly myopic correction he was wearing. Vitreous loss followed by postoperative complications are frequent after an anterior approach to dislocated lens removal. Anterior capsulotomy using a mode-locked (dye) Nd:YAG laser followed in a few hours by irrigation-aspiration of the lens was chosen as the least traumatic, safest procedure. I used a Meditec N d: YAG laser; at the time it was used on this patient, it had an emission at a fixed quantity of 3.7 mJ. I operated on the left eye first. Ninety minutes prior to laser anterior capsulotomy the patient was given 25 mg of indomethacin (Indocin@), 125 mg acetazolamide (Diamox®), and 125 mg of methylprednisolone ~Solu-Medrol®). A drop of %% timolol (Timoptic ) was also instilled in the left eye. The pupil was widely dilated with 10% phenylephrine (Neosynephrine®) and 1% cyclopentolate (Cyclogyl®), four doses of each at 15 minute intervals. The laser was focused to produce optical breakdown at or just under the lens capsule for a circular anterior capsulotomy (Figure 5). I was careful to leave no visible
Fig. 5. (Woodward) Left eye immediately after anterior capsulotomy performed with a mode-locked Nd:YAG laser. Fig. 4.
(Woodward) Patient with Marchesani syndrome, illustrating brachymorphia characteristics.
The patient's condition was diagnosed as Marchesani syndrome with nasal dislocation of spherical lenses, refractive myopia, impending pupillary block glaucoma, and serpiginous retinopathy. The history of blurred vision, the critically narrow chamber angles as well as the notoriously high incidence of pupillary block glaucoma in patients with 216
intact bridges of capsule and to make laser sites no closer than 2% mm to the iris. One hundred fifty-two laser emissions were used to perform the capsulotomy. One hour after the laser procedure was completed, the lOP was 16 mm Hg by Goldmann applanation. Three hours after the anterior caps ulotomy the irrigation-aspiration procedure was performed under local anesthesia.
AM INTRA-OCULAR IMPLANT SOC J-VOL 10, SPRING 1984
A small peritomy was performed. A 2%-mm diamond knife incision was made. The anterior chamber was entered with a bent 25-gauge needle to check the completeness of the capsulotomy. The freeQ piece of anterior capsule was removed with KelmanMcPherson forceps. There were still some adhesions between the capsule and the underlying cortex so we had to be careful to remove this piece of capsule without tearing it and without injuring the remaining zonules or hyaloid face. The McIntyre irrigation-aspiration system was used to remove the lens material, which was soft and easy to aspirate. Low flow was used. A peripheral iridectomy was performed. There were no operative complications. One month later I performed a similar procedure on the right eye. Again there was no vitreous loss; however, there was some difficulty with wound closure. Despite low lOP, the iris tended to £rolapse into the wound. Sodium hyaluronate (Healon ) had to be used to keep the iris back even after a peripheral iridectomy was performed. I used the Cavitron 8000 unit on the right eye. The soft lens nucleus was removed with the Kelman phacoemulsifier-aspirator using irrigation-aspiration only (no ultrasound). The 0.3-mm irrigation-aspiration tip was used for cortex removal. Irrigationaspiration "minimum" (25 inches of water) was the only aspiration force used and the irrigation bottle was kept low. Both methods worked well for gentle removal of the lens material. The patient has been followed for four months for the left eye and three months for the right eye. In each eye the visual acuity is 20/20 and the lOP is 17 mm Hg by Goldmann applanation tonometry. The anterior chambers are deep and the angles are wide open. The refractive error is +7.50 -l.50 x 10 in the right eye and +7.50 -l.25 x 170 in the left eye. DISCUSSION Marchesani1 classified a syndrome first described by Weil12 as a congenital, systemic affection of mesodermal tissue. He related it to its counterpart, the Marfan-Achard syndrome. The Marchesani syndrome is a hyperplastic (brachymorphic) form of a systemic disorder, whereas the Marfan syndrome is a hypoplastic (dolichomorphic) form. Both syndromes display dislocation of the crystalline lens .1,3 Individuals affected by the Marchesani syndrome (spherophakia-brachymorphia) are short and have stubby hands with thick fingers, knobby knuckles, and enlarged interphalangeal joints. Range of motion in their fingers and joints is limited in extension and flexion. The lens in the Marchesani syndrome is dislocated,
spherical, and small. It is most commonly dislocated inferiorly or nasally but may be dislocated in any direction. The spherical shape of the lens usually leads to lenticular myopia that tends to be progressive and may be severe. Removal of the lens usually results in a typical aphakic refractive error. Glaucoma is common in this syndrome and may be secondary to pupillary block or peripheral anterior synechia caused by the dislocated lens, or may be due to an anomalous chamber angle. 4 Removal of a subluxated or dislocated lens is typically difficult and attended by serious operative and postoperative complications. Because anterior capsulotomy with a Nd:YAG laser has been a safe, dependable aid for routine extracapsular cataract surgery in my experience, I used it in this case of Marchesani syndrome to avoid stressing the lens and vitreous while performing a precise capsulotomy. It was followed by easy, complicationfree aspiration of the soft nucleus and cortex (Figure 6).
Fig. 6.
(Woodward) Left eye one day after extracapsular removal oflens.
REFERENCES 1. Marchesani 0: Brachydaktylie und angeborene Kugellinse als Systemerkrankung. Klin Montsbl Augenheilkd 103:392-406, 1939 2. Weill C: Ectopie des cristallins et malformations generales. Ann Ocul 169:21-44, 1932 3. Jensen AD, Cross HE, Paton D: Ocular complications in the Weill-Marchesani syndrome. Am ] Ophthalmol 77:261-269, 1974 4. Feiler-Ofry V, Stein R, Codel V: Marchesani's syndrome and chamber angle anomalies. Am] Ophthalmol 65:862-866, 1968
AM INTRA-OCULAR IMPLANT SOC
J-
VOL 10, SPRING 1984
217
Fig. 1.
(Woodward) Right eye in patient with Marchesani syndrome, showing microspherophakia with nasal ectopia. Zonules on temporal side are practically nonexistent.
Fig. 2.
(Woodward) Left eye of same patient.
Fig. 3.
(Woodward) Serpiginous hyperpigmented lesions near macula of left eye.
Key Words: anterior capsulotomy, bilateral lens removal, Marchesani syndrome, Nd:YAG laser
CASE REPORT A 28-year-old white male who had been followed for myopia and dislocated lenses since age five years was seen because of blurred vision in his left eye of two months duration. Besides the blurred vision, he had been experiencing blurry spells with each eye for the previous year or longer. From age five years until the present, his refractive error changed from -9.00 -3.00 x 10 to -21.00 -2.00 x 10 in his right eye and from -9.00 -3.00 x 170 to -21.00 -2.00 x 170 in his left eye. With these corrections, his visual acuity was 20/20 in the right eye and 20/25 in the left eye. Ocular examination revealed a pupil diameter of 2.5 mm, blue irides, iridodonesis, shallow anterior chambers, and nasal dislocation of small, spherical lenses (Figures 1 and 2). Intraocular pressures (lOPs) were 18 mm Hg by Goldmann applanation. In the left eye, there was a serpiginous, hyperpigmented fundus lesion superonasal and inferior to the fovea and a depigmented area superotemporal to the fovea (Figure 3). The right fundus was normal. Gonioscopy showed a critically narrow chamber angle that deepened when mydriatic (2~% phenylephrine hydrochloride) drops were used to dilate the pupils to 7~ mm. The width of the angle appeared normal after dilating the pupils.
Reprint requests to Paul M. Woodward, M.D., 500 South Breiel Boulevard, Middletown, Ohio 45042.
AM INTRA-OCULAR IMPLANT SOC J-VOL 10, SPRING 1984
215
After dilation the lenses were noted to be clear, spherical, and dislocated nasally. The vitreous face was intact and only vestiges of the zonules were visible. Axial length measurement (A-Scan) was 24.45 mm in the right eye and 24.47 mm in the left eye. Keratometry measured 44.75 by 49.50 diopters (D), steep meridian at axis 100 degrees for the right eye; 44.00 by 48.75 D, steep meridian at axis 80 degrees for the left eye. General physical examination revealed the patient's height to be 63 inches. He was noted to be quite muscular although his work rarely required lifting and he did not exercise (Figure 4). His hands were spadelike with short stubby fingers and enlarged knuckles. His elbows could not be extended beyond 20 degrees. He was noted to walk on his toes because of restriction of flexion of the ankle joints. He had a loud systolic murmur.
Marchesani syndrome made lens removal desirable ifit could be safely performed. The axial length measurements and dioptric power of his corneas indicated that the ultimate refractive error in each eye would be +6.75 D if his lenses were simply removed. This seemed preferable to the highly myopic correction he was wearing. Vitreous loss followed by postoperative complications are frequent after an anterior approach to dislocated lens removal. Anterior capsulotomy using a mode-locked (dye) Nd:YAG laser followed in a few hours by irrigation-aspiration of the lens was chosen as the least traumatic, safest procedure. I used a Meditec N d: YAG laser; at the time it was used on this patient, it had an emission at a fixed quantity of 3.7 mJ. I operated on the left eye first. Ninety minutes prior to laser anterior capsulotomy the patient was given 25 mg of indomethacin (Indocin@), 125 mg acetazolamide (Diamox®), and 125 mg of methylprednisolone ~Solu-Medrol®). A drop of %% timolol (Timoptic ) was also instilled in the left eye. The pupil was widely dilated with 10% phenylephrine (Neosynephrine®) and 1% cyclopentolate (Cyclogyl®), four doses of each at 15 minute intervals. The laser was focused to produce optical breakdown at or just under the lens capsule for a circular anterior capsulotomy (Figure 5). I was careful to leave no visible
Fig. 5. (Woodward) Left eye immediately after anterior capsulotomy performed with a mode-locked Nd:YAG laser. Fig. 4.
(Woodward) Patient with Marchesani syndrome, illustrating brachymorphia characteristics.
The patient's condition was diagnosed as Marchesani syndrome with nasal dislocation of spherical lenses, refractive myopia, impending pupillary block glaucoma, and serpiginous retinopathy. The history of blurred vision, the critically narrow chamber angles as well as the notoriously high incidence of pupillary block glaucoma in patients with 216
intact bridges of capsule and to make laser sites no closer than 2% mm to the iris. One hundred fifty-two laser emissions were used to perform the capsulotomy. One hour after the laser procedure was completed, the lOP was 16 mm Hg by Goldmann applanation. Three hours after the anterior caps ulotomy the irrigation-aspiration procedure was performed under local anesthesia.
AM INTRA-OCULAR IMPLANT SOC J-VOL 10, SPRING 1984
A small peritomy was performed. A 2%-mm diamond knife incision was made. The anterior chamber was entered with a bent 25-gauge needle to check the completeness of the capsulotomy. The freeQ piece of anterior capsule was removed with KelmanMcPherson forceps. There were still some adhesions between the capsule and the underlying cortex so we had to be careful to remove this piece of capsule without tearing it and without injuring the remaining zonules or hyaloid face. The McIntyre irrigation-aspiration system was used to remove the lens material, which was soft and easy to aspirate. Low flow was used. A peripheral iridectomy was performed. There were no operative complications. One month later I performed a similar procedure on the right eye. Again there was no vitreous loss; however, there was some difficulty with wound closure. Despite low lOP, the iris tended to £rolapse into the wound. Sodium hyaluronate (Healon ) had to be used to keep the iris back even after a peripheral iridectomy was performed. I used the Cavitron 8000 unit on the right eye. The soft lens nucleus was removed with the Kelman phacoemulsifier-aspirator using irrigation-aspiration only (no ultrasound). The 0.3-mm irrigation-aspiration tip was used for cortex removal. Irrigationaspiration "minimum" (25 inches of water) was the only aspiration force used and the irrigation bottle was kept low. Both methods worked well for gentle removal of the lens material. The patient has been followed for four months for the left eye and three months for the right eye. In each eye the visual acuity is 20/20 and the lOP is 17 mm Hg by Goldmann applanation tonometry. The anterior chambers are deep and the angles are wide open. The refractive error is +7.50 -l.50 x 10 in the right eye and +7.50 -l.25 x 170 in the left eye. DISCUSSION Marchesani1 classified a syndrome first described by Weil12 as a congenital, systemic affection of mesodermal tissue. He related it to its counterpart, the Marfan-Achard syndrome. The Marchesani syndrome is a hyperplastic (brachymorphic) form of a systemic disorder, whereas the Marfan syndrome is a hypoplastic (dolichomorphic) form. Both syndromes display dislocation of the crystalline lens .1,3 Individuals affected by the Marchesani syndrome (spherophakia-brachymorphia) are short and have stubby hands with thick fingers, knobby knuckles, and enlarged interphalangeal joints. Range of motion in their fingers and joints is limited in extension and flexion. The lens in the Marchesani syndrome is dislocated,
spherical, and small. It is most commonly dislocated inferiorly or nasally but may be dislocated in any direction. The spherical shape of the lens usually leads to lenticular myopia that tends to be progressive and may be severe. Removal of the lens usually results in a typical aphakic refractive error. Glaucoma is common in this syndrome and may be secondary to pupillary block or peripheral anterior synechia caused by the dislocated lens, or may be due to an anomalous chamber angle. 4 Removal of a subluxated or dislocated lens is typically difficult and attended by serious operative and postoperative complications. Because anterior capsulotomy with a Nd:YAG laser has been a safe, dependable aid for routine extracapsular cataract surgery in my experience, I used it in this case of Marchesani syndrome to avoid stressing the lens and vitreous while performing a precise capsulotomy. It was followed by easy, complicationfree aspiration of the soft nucleus and cortex (Figure 6).
Fig. 6.
(Woodward) Left eye one day after extracapsular removal oflens.
REFERENCES 1. Marchesani 0: Brachydaktylie und angeborene Kugellinse als Systemerkrankung. Klin Montsbl Augenheilkd 103:392-406, 1939 2. Weill C: Ectopie des cristallins et malformations generales. Ann Ocul 169:21-44, 1932 3. Jensen AD, Cross HE, Paton D: Ocular complications in the Weill-Marchesani syndrome. Am ] Ophthalmol 77:261-269, 1974 4. Feiler-Ofry V, Stein R, Codel V: Marchesani's syndrome and chamber angle anomalies. Am] Ophthalmol 65:862-866, 1968
AM INTRA-OCULAR IMPLANT SOC
J-
VOL 10, SPRING 1984
217