Congenital ptosis, longterm results using stored fascia lata

3
346 Congenital ptosis: longterm results using stored fascia lata James O’Reilly, Bernadette Lanigan, Roger Bowell and Michael O’Keefe Department of Paediatric Ophthalmology, The Children’s Hospital, Dublin, Ireland ABSTRACT. Purpose: Frontalis brow suspension, using stored donor fascia lata, allows early correction of severe congenital ptosis. We report longterm results of 18 patients, (29 ptotic eyelids). Methods: Patient records were retrospectively reviewed. Lid postion at the first and subsequent post-operative visits was recorded, as was the time when ptosis recurred. Results: The mean follow-up period was 42.9 months (range 7 to 84 months). All patients achieved a Good initial result. Partial ptosis recurrence was noted in 7 patients (10 eyelids), within 18 months of surgery. One of these patients required reoperation. Lid position did not change after the first 18 months. No late failures were encountered. Conclusion: Longterm lid position is remarkably stable after early surgical cor- rection of severe congenital ptosis using stored fascia lata. Key words: congenital ptosis – brow suspension – stored fascia lata – ptosis recurrence. Acta Ophthalmol. Scand. 1998: 76: 346–348 Copyright c Acta Ophthalmol Scand 1998. ISSN 1395-3907 T here is no single definitive surgical procedure for the early correction of severe congenital ptosis. After the age of three years, when the iliotibial tract is sufficiently well de- veloped, bilateral frontalis suspension with autogenous fascia lata, as originally described by Wright in 1922, has been shown to give excellent longterm results. Levator resection can be performed at an earlier age. However, it is impossible to adequately assess levator function in young children and resection of too much muscle may subsequently lead to a ‘‘frozen’’ and irreparable eyelid. Alterna- tively, frontalis brow suspension using materials other than autogenous fascia late can be easily performed in children under the age of one year. Stored fascia lata has, for many years, been widely used as a suspensory material. The technique was pioneered by Crawford (1956) and its usefulness has been con- firmed by many other authors, including Broughton et al. (1982) and Wagner et al. (1984). In our unit, all patients presenting with severe unilateral or bilateral congeni- tal ptosis during the period 1986 and 1994 underwent bilateral frontalis sling pro- cedure with stored fascia lata. In this report we present detailed, longterm follow-up results of our patient group. Materials and Methods A retrospective review of all children who underwent surgery for severe congenital ptosis between 1986 and 1994 was per- formed. A total of eighteen patients were identified. Seven of these patients had unilateral ptosis and eleven had bilateral ptosis on presentation, representing a total of 29 ptotic eyelids. Occlusion of the visual axis or a marked chin-up head posture were indi- cations for surgery. A Bilateral Frontalis Sling procedure using stored fascia late was performed in seventeen of the eighteen patients that underwent surgery. The remaining pa- tient, at the behest of the parents, under- went surgery on the side of the ptosis only. The mean age of the patients at the time of surgery was 11 months. Twelve of the patients were under one year of age (ranging from 2 to 9 months). The re- maining six patients presented at a later age and were operated on at between 14 and 32 months. All patients underwent a complete ocu- lar and systemic examination preopera- tively. Twelve of the eighteen patients had isolated congenital ptosis with no other systemic or ocular abnormalities. Three of the patients had systemic anomalies (including mental handicap, developmen- tal delay and various chromosomal dis- orders), but no ocular anomalies. The re- maining three patients all had ocular anomalies, (blepharophimosis, nanoph- thalmos and congenital fibrosis of the extraocular muscles, respectively). The surgical technique used was that described by Crawford (1956). Under general anaesthesia, three evenly spaced stab incisions were made 2 to 3 mm above the margin of the upper eyelid. Two further stab incisions above the eyebrow and one centrally in the forehead were made. A Wrights fascial needle was passed deep to the orbicularis and super- ficial to the tarsal plate and orbital sep- tum. Strips of stored fascia lata were passed through the eye of the needle and pulled through the track of the needle as it was withdrawn. The material was pull- ed up until the lid level was just below the superior limbus. It was then knotted and sutured and the brow incisions closed with 5/0 vicryl sutures. A Frost suture was inserted and the eye padded until the following morning. The initial post-operative lid position and that at subsequent follow-up visits

Transcript of Congenital ptosis, longterm results using stored fascia lata

346

Congenital ptosis: longtermresults using stored fascia lataJames O’Reilly, Bernadette Lanigan, Roger Bowell andMichael O’Keefe

Department of Paediatric Ophthalmology, The Children’s Hospital, Dublin, Ireland

ABSTRACT.Purpose: Frontalis brow suspension, using stored donor fascia lata, allows earlycorrection of severe congenital ptosis. We report longterm results of 18 patients,(29 ptotic eyelids).Methods: Patient records were retrospectively reviewed. Lid postion at the firstand subsequent post-operative visits was recorded, as was the time when ptosisrecurred.Results: The mean follow-up period was 42.9 months (range 7 to 84 months).All patients achieved a Good initial result. Partial ptosis recurrence was notedin 7 patients (10 eyelids), within 18 months of surgery. One of these patientsrequired reoperation. Lid position did not change after the first 18 months. Nolate failures were encountered.Conclusion: Longterm lid position is remarkably stable after early surgical cor-rection of severe congenital ptosis using stored fascia lata.

Key words: congenital ptosis – brow suspension – stored fascia lata – ptosis recurrence.

Acta Ophthalmol. Scand. 1998: 76: 346–348Copyright c Acta Ophthalmol Scand 1998. ISSN 1395-3907

There is no single definitive surgicalprocedure for the early correction of

severe congenital ptosis.After the age of three years, when the

iliotibial tract is sufficiently well de-veloped, bilateral frontalis suspensionwith autogenous fascia lata, as originallydescribed by Wright in 1922, has beenshown to give excellent longterm results.

Levator resection can be performed atan earlier age. However, it is impossibleto adequately assess levator function inyoung children and resection of too muchmuscle may subsequently lead to a‘‘frozen’’ and irreparable eyelid. Alterna-tively, frontalis brow suspension usingmaterials other than autogenous fascialate can be easily performed in childrenunder the age of one year.

Stored fascia lata has, for many years,been widely used as a suspensory material.The technique was pioneered by Crawford(1956) and its usefulness has been con-firmed by many other authors, includingBroughton et al. (1982) and Wagner et al.

(1984). In our unit, all patients presentingwith severe unilateral or bilateral congeni-tal ptosis during the period 1986 and 1994underwent bilateral frontalis sling pro-cedure with stored fascia lata.

In this report we present detailed,longterm follow-up results of our patientgroup.

Materials and MethodsA retrospective review of all children whounderwent surgery for severe congenitalptosis between 1986 and 1994 was per-formed. A total of eighteen patients wereidentified. Seven of these patients hadunilateral ptosis and eleven had bilateralptosis on presentation, representing atotal of 29 ptotic eyelids.

Occlusion of the visual axis or amarked chin-up head posture were indi-cations for surgery.

A Bilateral Frontalis Sling procedureusing stored fascia late was performed in

seventeen of the eighteen patients thatunderwent surgery. The remaining pa-tient, at the behest of the parents, under-went surgery on the side of the ptosis only.

The mean age of the patients at thetime of surgery was 11 months. Twelve ofthe patients were under one year of age(ranging from 2 to 9 months). The re-maining six patients presented at a laterage and were operated on at between 14and 32 months.

All patients underwent a complete ocu-lar and systemic examination preopera-tively. Twelve of the eighteen patients hadisolated congenital ptosis with no othersystemic or ocular abnormalities. Threeof the patients had systemic anomalies(including mental handicap, developmen-tal delay and various chromosomal dis-orders), but no ocular anomalies. The re-maining three patients all had ocularanomalies, (blepharophimosis, nanoph-thalmos and congenital fibrosis of theextraocular muscles, respectively).

The surgical technique used was thatdescribed by Crawford (1956). Undergeneral anaesthesia, three evenly spacedstab incisions were made 2 to 3 mm abovethe margin of the upper eyelid. Twofurther stab incisions above the eyebrowand one centrally in the forehead weremade. A Wrights fascial needle waspassed deep to the orbicularis and super-ficial to the tarsal plate and orbital sep-tum. Strips of stored fascia lata werepassed through the eye of the needle andpulled through the track of the needle asit was withdrawn. The material was pull-ed up until the lid level was just below thesuperior limbus. It was then knotted andsutured and the brow incisions closedwith 5/0 vicryl sutures. A Frost suturewas inserted and the eye padded until thefollowing morning.

The initial post-operative lid positionand that at subsequent follow-up visits

347

were recorded as Good, Moderate orPoor according to the following criteria:

Good: less than 2 mm of residual pto-sis without frontalis effort.

Moderate: 2 to 3 mm of residual ptosiswithout obstruction of the visual axis.

Poor: 3 mm or more of residual ptosisor a complete return to the pre-operativelid position.

The criteria defining a Moderate and aPoor result are the same criteria that wereused by Wilson and Johnson in 1991 todefine minimal and marked ptosis recur-rence, respectively.

ResultsThe mean length of the follow-up periodwas 42.9 months, ranging from 7 to 84months. The results are displayed inTable 1.

All eighteen patients underwent an un-complicated surgical procedure and hadgood lid position at the first post-operat-ive visit. An inflammatory brow reactionto the donor fascia lata (as has been welldescribed by Crawford) was noted in allpatients in the initial post-operativeperiod. This reaction was severe in threeof the eighteen patients, manifesting asencrusted lesions at the suture sites, simu-lating infection. However, all three settledspontaneously with the administration oftopical antibiotics, confirming the in-flammatory nature of this reaction.

Eleven of the patients retained good po-sition of both eyelids at the final follow-up

Table 1.

Age at Length ofPt. Surgery Uni./Bil. Uni./Bil. Initial Result Final Result follow-upNo. (months) Ptosis. Surgery OD OS OD OS (months)

1 3 Right Bilat. Good Good Mod. Good 802 5 Bilat. Bilat. Good Good Good Good 843 2 Bilat. Bilat. Good Good Mod. Mod. 794 3 Left Bilat. Good Good Good Mod. 685 30 Right Unilat. Good Good Good 656 14 Bilat. Bilat. Good Good Mod. Mod. 577 29 Bilat. Bilat. Good Good Good Good 458 3 Bilat. Bilat. Good Good Good Good 389 2 Right Bilat. Good Good Good Good 26

10 17 Bilat. Bilat. Good Good Good Good 2111 2 Bilat. Bilat. Good Good Good Good 812 2 Bilat. Bilat. Good Good Mod. Mod. 1613 4 Left Bilat. Good Good Good Mod. 714 9 Left Bilat. Good Good Good Mod. 715 27 Bilat. Bilat. Good Good Good Good 4416 32 Bilat. Bilat. Good Good Good Good 3717 5 Left Bilat. Good Good Good Good 3818 9 Bilat. Bilat. Good Good Good Good 44

visit. A further four patients were recordedas having a moderate position of one eye-lid and a good position of the other eyelid.In all four cases the ptosis had been unilat-eral initially and the partial recurrence oc-curred in the originally ptotic eyelid. Theremaining three patients had bilateral pto-sis initially and moderate ptosis recur-rence in both eyelids. Overall, of 29 ptoticeyelids in eighteen patients, 19 weregraded as Good at final follow-up and 10were graded as Moderate.

The time at which ptosis recurrencewas first noted in each of the seven pa-tients is displayed in Table 2. No changein eyelid position was recorded in any ofour patient cohort more than 18 monthsafter the initial surgery. Patient No. 3underwent two repeat procedures in orderto achieve the final lid position. However,this patients surgery was complicated bycongenital fibrosis of the extraocularmuscles.

DiscussionIndications for early intervention in casesof congenital ptosis include a severely ab-normal, chin-up head posture and/or oc-clusion of one or both visual axes. Achin-up head posture is a severe impedi-ment for a child faced with crucial devel-opmental tasks such as learning to crawland walk. Isolated severe congenital pto-sis is associated with a 3 to 10% incidenceof amblyopia (Katowitz 1979; Ander-son & Baumgartner 1980).

Table 2.

Patient Time elapsed after surgery whenNo. moderate ptosis was first noted.

1 15 months3 18 months Two repeat procedures

were performed after theoriginal surgery in orderto achieve the final result(complicated by congeni-tal fibrosis of theextraocular muscles).

4 11 months6 16 months

12 9 months13 7 months14 7 months

No change in eyelid position was recorded after18 months.

Frontalis brow suspension, using ma-terials other than autogenous fascia lata,is easily performed in young infants andis the only procedure that allows immedi-ate intervention in cases of severe con-genital ptosis. Crawford pioneered theuse of stored fascia lata as a suspensorymaterial (1956) and has recommendedimmediate bilateral frontalis suspensionusing stored fascia lata in cases of severeunilateral congenital ptosis (1986).

Stored fascia lata is well establishedas a suspensory material. Crawford hasreported an initial success rate in excessof 90% in a number of publications(1975, 1977, 1982). Other studies haveconfirmed this high rate of success.Wagner et al. (1984) documented only 2failures (at 4 and 14 months, respec-tively) in a study with a mean follow-upof 20.8 months (range 6 to 65 months).Beyer & Albert (1981) reported earlyfailure in 2 of 38 patients. Nine of thepatients were followed up for longerthan 3 years. Late recurrence of ptosiswas not documented in any of the 38patients. Furthermore, morphologicallyintact banked fascia lata has been docu-mented histopathologically by Beyerand Albert (1981), 5, 9, 16 and 36months after implantation and supportsthe opinion that ptosis repair withstored fascia lata may well be perma-nent.

Wilson and Johnson (1991) were thefirst to report a high incidence of latefailure of ptosis repairs performed withstored fascia lata. They reported long-term follow-up results on a series of 56patients who had undergone frontalissuspension using lyophilised fascia lata.

348

The mean follow-up period was 7.2years (range 6 to 123 months, median,100 months). Marked ptosis recurrencewas defined as a complete ptosis recur-rence to the preoperative level or 3 mmof droop in one or both eyelids com-pared with the initial postoperativelevel. Incomplete return of ptosis lessthan 3 mm was defined as minimal re-currence. Using this criteria, they dem-onstrated an increasing number of fail-ures with increasing length of follow-up;the success rate of surgery falling from90% at two to three years to 70% at 5years and 50% at 8 to 9 years.

In our study we used the same criteriaas Wilson et al. for assessing the successand failure of surgery. While the numberof patients and the follow-up period arenot as extensive as those of Wilson et al.,the total absence of any late recurrencesin our patient cohort is not compatiblewith the gradual and progressive failurerate reported by Wilson et al.

The divergence of our results fromthose of Wilson et al., may be explained,in part, by the fact that our results arethe results of two surgeons only (M.O’K.and R.B.) working closely together in thesame unit, whilst those of Wilson et al.represent the amalgamated results oftwelve collaborating surgeons working indifferent institutions.

Our results confirm the findings ofearlier, smaller studies which reported ahigh initial success rate with a small inci-dence of early recurrence. The more ex-tensive follow-up period of our study (12patients followed up for more than 3years, 5 of whom were followed for over5 years, including 3 who were followed upfor between 6 and 7 years), further con-firms our clinical impression of the per-manence of repairs performed withstored fascia lata.

The reasons underlying the partial re-currence of ptosis in 10 of the 29 eyelidsare unclear. The early and abrupt occur-rence of this phenomenon and the sub-sequent stability of the lid position sug-gests that it was due to slippage of thefascial sling and not absorption of thefascia. The fact that, in the techniqueused, the fascia was not sutured to thetarsal plate may have been a contributoryfactor.

Availability and expense are factorswhich limit the usefulness of stored fascialata. Fascia lata is obtained by collectingexcess unused fascia lata from caseswhere autogenous fascia is being har-vested. This fascia is then banked and ir-

radiated. The entire process is time con-suming and expensive and the availabilityof stored fascia is often erratic.

Furthermore, any donor material carr-ies with it the possibility of disease trans-mission. The prevalence of diseases suchas H.I.V. and Hepatitis B and C and re-cent publicity surrounding Prion diseaseshave lead to a reluctance amongst parentsto accept any form of donor materialthat is not absolutely necessary.

A number of synthetic materials, in-cluding Mersilene mesh (Downes andCollin 1990) and Polyfilament nylon su-ture (Supramid Extra) have been pro-posed as substitute materials (Katowitz1979; Saunders & Grice 1991). However,none of these materials have been par-ticularly successful. A high incidence ofptosis recurrence has been reported in as-sociation with the use of Polyfilament ny-lon (Wagner et al. 1984), while repeatsurgery is often difficult when Mersilenemesh has been used.

The lack of a synthetic material withproperties which equal those of autogen-ous fascia lata has prompted Manners etal. 1994) to suggest planned temporaryrepair of congenital ptosis using 4/0 Pro-lene until the child is old enough to un-dergo definitive repair using autogenousfascia lata. Good temporary results areachieved with Prolene slings and at re-surgery far less scarring is encounteredthan is encountered after the use of fas-cial slings. However, ‘‘cheesewiring’’ ofProlene sutures (Manners et al. 1994, andpersonal experience), can lead to earlyand progressive partial recurrence of pto-sis, necessitating early definite repair.Harvesting of autogenous fascia latafrom the limbs of small children can re-sult in disfiguring scars. The longtermstability of repairs performed usingstored fascia lata means that definite re-pair, if necessary, can be postponed untilthe child is older.

In conclusion, there is no single pro-cedure that can guarantee early andpermanent repair of congenital ptosis.While temporary repair with 4\0 Pro-lene, as suggested by Manners et al. isan effective strategy, stored fascia lata isthe only suspensory material currentlyavailable that offers a real prospect ofearly and longlasting repair of congeni-tal ptosis in a single procedure. It is arelatively uncomplicated procedure,early failures are amenable to repeatsurgery and there is considerable evi-dence that longterm lid position is re-markably stable.

ReferencesAnderson RL & Baumgartner SA (1980): Am-

blyopia in ptosis. Arch Ophthalmol 98:1068–1070.

Beyer CK & Albert DM (1981): The use andfate of fascia lata and sclera in ophthalmicplastic and reconstructive surgery. The 1980Wendell Hughes Lecture. Ophthalmology19: 869–886.

Broughton WL, Matthews JG & Harris DJ Jr.(1982): Congenital ptosis: results of treat-ment using lyophilized fascia lata forfrontalis suspensions. Ophthalmology 89:1261–1266.

Cole MD, O’ Connor GM, Raafai F. & Will-shaw HE (1989): A new synthetic materialfor the brow suspension procedure. Br JOphthalmol 73: 35–38.

Crawford JS (1956): Repair of ptosis usingfrontalis muscle and fascia lata. Trans AmAcad Ophthalmol Otolaryngol 60: 672–678.

Crawford JS (1975): Recent trends in ptosissurgery. Ann Ophthalmol 7: 1263–1267.

Crawford JS (1977): Repair of ptosis usingfrontalis muscle and fascia lata: a 20 yearreview. Ophthalmic Surgery 8(4): 31–40.

Crawford JS (1982): Frontalis sling operation. JPediatr Ophthalmol Strabismus 19: 253–255.

Crawford JS (1986): Congenital Ptosis: exam-ination and treatment. Transactions of theNew Orleans Academy of Pediatric Oph-thalmology and Strabismus. 34: 173–191.

Downes RN & Collin JRO (1990): The Mersi-lene mesh ptosis sling. Eye 4: 456–463.

Katowitz JA (1979): Frontalis suspension incongenital ptosis using a polyfilament, cabletype suture. Arch Ophthalmol. 97: 1659–1663.

Manners RM, Tyers AG & Morris RJ (1994):The use of Prolene as a temporary suspen-sory material for brow suspension in youngchildren. Eye 8: 346–348.

Saunders RA & Grice CM (1991): Early cor-rection of severe congenital ptosis. J PedOphthalmol Strabismus 28: 271–273.

Wagner RS, Mauriello JA Jr, Nelson LB, et al.(1984): Treatment of congenital ptosis withfrontalis suspension: a comparison of sus-pensory materials. Ophthalmology 91: 245–248.

Wilson ME & Johnson RW (1991): Congenitalptosis: long term results of treatment usingfascia lata for frontalis suspension. Ophthal-mology 98: 1234–1237.

Wright WW (1922): The use of living suturesin the treatment of ptosis. Arch Ophthalmol51: 99–102.

Received on February 24th, 1997.Accepted on September 29th, 1997.Corresponding author:

Michael O’KeefeDepartment of Paediatric OphthalmologyThe Children’s HospitalTemple StreetDublinIreland.