British Journal of Ophthalmology, 1986, 70, 732-736

Management of ipsilateral ptosis with hypotropiaL A FICKER, J R 0 COLLIN, AND J P LEE

From Moorfields Eye Hospital, London

SUMMARY Thirty-one patients presented for surgical correction of unilateral hypotropia of theglobe and blepharoptosis. The hypotropia and pseudoptosis were corrected by Knapp procedures.The Bell's phenomenon was thereby improved, allowing safe correction of the true ptosis,generally by an anterior levator resection whose magnitude depended on measured levatorfunction.

The association of unilateral ptosis and hypotropia ofthe globe arises in a number of clinical situations,most commonly in congenital double elevator palsy.Their surgical correction was approached by Beard,'who found double elevator palsy in 6% of his cases ofunilateral congenital ptoses. He noted levator resec-tion was less effective where superior rectus weak-ness coexisted, and recommended an additional4 mm approximately of levator resection. Heapproached the correction of hypotropia by superiorrectus resection and inferior rectus recession withrelease of the lower lid retractors. Knapp' subse-quently described transposition of the horizontalrectus tendons to that of the superior rectus. Thisimproved the position of the globe and its elevation.Scott and Jackson, recognised, by forced ductiontests, that inferior rectus restriction plays a part insome cases and advocated recession of the musclewith release of the lower lid retractors. This was notcombined in their work with the Knapp procedure asthey feared anterior segment ischaemia. Callahan'modified the approach (to reduce the risk ofischaemia) by routinely recessing the inferior rectuscombining this with a Jensen' style procedure, inwhich the active horizontal rectus muscles were splitand half of each muscle was sutured to the weaksuperior rectus.

Lee, Collin, and Timms" recommend that a forcedduction test is done initially. If the eye moves freely, aKnapp procedure alone is done. If there is limitation,the inferior rectus muscle is recessed, and this iscombined with a Knapp procedure except in thosecases considered to be at risk of ischaemia, that is,those patients over 25 years of age or with a largeglobe, in whom they do a Callahan procedure.Correspondence to L Ficker, FRCS, Moorfields Eye Hospital, CityRoad, London ECIV 2PD.

Contralateral superior rectus recession with a Fadenprocedure is used in selected cases to increase thedrive to the paretic superior rectus where thesemeasures prove inadequate. Elevation of the hypo-tropic globe is considered to be the primary step,followed by correction of the true blepharoptosis,which is the subject of this paper.

Patients and methods

Thirty-one patients presented for surgical correctionof unilateral hypotropia of the globe and associatedblepharoptosis. The aetiology of the combinedabnormalities included congenital double elevatorpalsy (15 patients), Marcus-Gunn jaw-winking syn-drome (eight patients), third nerve palsy (threepatients), neurofibromatosis with lid involvement(three patients), and superior rectus sling-induced(two patients).The surgical approach was to improve active

elevation of the globe, thereby enhancing the Bell'sphenomenon and correcting the element of pseudo-ptosis. A Knapp procedure was adopted, elevatingthe insertions of the horizontal rectus muscles to thesuperior rectus insertion. In addition any horizontaldeviation of the globe was corrected at the same timeby appropriate recession or resection of the hori-zontal rectus muscles, as described by Alvaro.7 Aforced duction test was performed in each case.Inferior rectus restriction, where found, was relievedby inferior rectus recession. Careful recession of thelower lid retractors was performed to preservenormal lower lid position.The true blepharoptosis was corrected by a levator

resection, which was denoted large (greater than 20mm), medium (15-20 mm), or small. The extent ofthe resection required depended on the levator

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Management ofipsilateralptosis with hypotropia

function (good greater than 10 mm, moderate 4-10mm, or poor). The minimum delay between theseprocedures was four months but ranged from fourmonths to two years. An anterior approach waspreferred for the large resections, affording goodexposure.

CONGENITAL DOUBLE ELEVATOR PALSYGROUP (15 patients)Five patients had previously undergone both verticalmuscle surgery (inferior rectus recession, superiorrectus resection, or both) and ptosis surgery. Threepatients had undergone vertical muscle surgery aloneand three ptosis surgery alone. Hence, four of the 15were cases not previously operated upon. Of thepatients previously operated upon one required amucous membrane graft to his scarred upper fornixand two required scleral grafting to raise adequatelythe lower lid following inferior rectus surgery. AKnapp procedure was indicated in all patients in thisgroup with additional inferior rectus recession in onlytwo. In one case inferior rectus recession was supple-mented by a contralateral superior rectus recessionwith a Faden procedure. Horizontal rectus recessionor resection was performed simultaneously with aKnapp procedure in seven cases. Two patients weremainly concerned by their ptoses and elected not toundergo strabismus surgery.The ptoses were corrected as follows. One patient

with no levator function underwent bilateral browsuspension. Eight had a large anterior levator resec-tion, one a moderate anterior levator resection, andfive small resections by either an anterior or posteriorapproach.

JAW-WINKING GROUP (8 patients)Six patients were found to require a Knapp pro-cedure and one a Callahan operation with inferiorrectus recession. A severely retarded child, in whomptosis correction was the only cosmetic goal, had alarge posterior levator resection without extraocularmuscle surgery.Where ptosis was the major problem, an anterior

levator resection was performed. Two patients had alarge anterior levator resection and one a cautiouslevator resection rather than a brow suspension, asthe Bell's phenomenon was not adequately enhancedby the Knapp procedure. Significant jaw-winking wascorrected by levator excision and bilateral browsuspension. Three patienits required bilateral browsuspension and another is yet to undergo ptosissurgery. The last patient declined ptosis surgery,being satisfied after the Knapp procedure.

THIRD NERVE PALSY GROUP (3 patients)The first patient had a post-traumatic third nerve

palsy with misdirection regeneration. The conditionhad been stable for five years. A Knapp procedurewith lateral rectus recession and medial rectus resec-tion was supplemented by inferior rectus recession.Later contralateral lateral rectus recession with aFaden procedure and medial rectus resection wereperformed to encourage medial rectus drive of theaffected eye. Levator function was good, and a smallposterior levator resection was finally done on theaffected side with a contralateral upper lid recession.The remaining two patients had congenital third

nerve palsies. One had previousLy undergone bothvertical muscle surgery and ptosis correction. Bothhad moderate levator function. They underwentKnapp procedures, achieving only reduced Bell'sphenomena. Large anterior levator resections weresubsequently carried out.

.NEUROFIBROMATOSIS (3 patients)Debulking of the plexiform neuromata precededcorrection of hypotropia and ptosis in two cases. Onepatient had a good Bell's phenomenon with reason-able elevation of the globe and underwent anteriorlevator resection alone. Of the other two, one had aCallahan procedure with inferior rectus recessionprior to a large anterior levator resection and furtherdebulking. The third had a Knapp procedure withinferior rectus recession. This was followed by upperlid debulking and canthal raising plus a dacryocysto-rhinostomy. Prior to a large anterior levator resec-tion, lower lid scleral grafting was required tolengthen the lower lid retractors.

SUPERIOR RECTUS SLING INDUCED (2 patients)Both patients presented with ptosis and hypotropiafollowing surgery elsewhere at which the superiorrectus muscle had been attached to the upper eyelidto elevate it. One case in which the original ptosis wasacquired following trauma required division of thesuperior rectus sling first, to restore proper superiorrectus function. The levator disinsertion was thencorrected via an anterior approach. The other casewas of congenital ptosis. A Knapp procedure wasperformed followed by a large anterior levatorresection, with freeing of all the adhesions of theprevious superior rectus sling operation.

Results

CONGENITAL DOUBLE ELEVATOR PALSY GROUPThe correction of blepharoptosis may be consideredin terms of both pseudoptosis and real ptosis. Follow-ing the Knapp operation as described by Lee et al.'successful active elevation of. the globe is accom-panied by correction of the pseudoptosis andimprovement of the Bell's phenomenon. This was

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Fig. I Primary gaze showing hypotropia ofthe right globeand bhlepharoptosis.

achieved in three of the four cases not previouslyoperated on; these patients underwent Knapp pro-cedures and had good levator function. The fourthpatient had poor levator function and gainedimprovement rather than correction of the hypo-tropia and pseudoptosis. Eight patients had pre-viously undergone surgery to the vertical rectusmuscles with no improvement of the hypotropia,pseudoptosis, or Bell's phenomenon. These weresatisfactorily corrected by a Knapp procedure in sixof the eight cases. The remaining two had consider-able scarring round the common sheath, but wereimproved. Three patients had undergone ptosissurgery with residual ptosis, pseudoptosis, and hypo-tropil. Correction of the pseudoptosis and elevationof the globe were achieved by Knapp surgery.The element of real ptosis was corrected to within

1 mm, with acceptable cosmesis, in 13 cases withsurgery based on the guidelines relating levatorfunction to the amount of levator resection required.'Where a large resection was indicated, it was facili-tated by the good surgical exposure gained via anaInterior approach.

Fig. 3 Pos.t-Knapp procedlure showing correction ofthehypotropia (i1(t revealing the true blepharoptosis.

Fig. 2 Cover test showing elevation ofthe right globe wcorrection ofthe pseudoptosis.

Two patients had a measured overcorrection, oneof whom needed an upper lid recession. Two of the 13patients, who had cosmetically good results with lidlevels within 1 mm of each other, had a functionalovercorrection reflected by punctate corneal stain-ing. These were asymptomatic and treated conserva-tively with lubricants.

JAW-WINKING GROUP

Of the six patients who underwent primary Knapp orCallahan procedures one was cosmetically satisfiedand had good levator function. Of the three requiringlevator resection, one was corrected despite thecomplication of orbital asymmetry due to a baso-cranial teratoma. The second had been unsuitable forbrow suspension in the absence of an adequate Bell'sphenomenon. The third is yet to undergo ptosissurgery. Two patients with signficant jaw-winkingwere corrected by brow suspension and levatorexcision procedures. Two patients underwent levatorresection alone (including the retarded child) andwere found to have residual pseudoptosis requiring aKnapp operation.

Fig. 4 After anterior levator resection, demonstratingsymmetry o'fglobe.s and lids.

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THIRD NERVE PALSY GROUPThe Bell's phenomenon was not markedly improvedin this group. After the Knapp operation, supple-mented by contralateral superior rectus surgery, as

described above in the case with misdirectionregeneration, levator resection achieved a satisfac-tory eyelid elevation correcting the lid levels to within1 mm of each other. The incidence of punctatecorneal staining was high in this group, as the Bell'sphenomenon was characteristically poor. This was

particularly marked in the case with misdirectionregeneration, whose poor ocular movements hadnecessitated bilateral strabismus surgery. Howeverall the patients were young, with good tear produc-tion, and have tolerated the punctate staining withthe aid of lubricants over a three-year follow-upperiod.

NEUROFIBROMATOSISThe ptosis was completely corrected in one case. A50% improvement of the ptosis was achieved in theother two cases. Residual bulkiness of the lid andsome hypotropia of the globe remained in all cases.

SUPERIOR RECTUS SLING-INDUCED GROUPThe ptosis was corrected to within 1 mm and thehypotropia was almost completely corrected in bothcases. There was a residual minimal vertical diplopiain the post-traumatic case.

Discussion

The group with congenital double elevator palsyillustrates the principles involved in the managementof combined hypotropia and blepharoptosis. In thepresence of good levator function, pseudoptosistends to account for much of the blepharoptosis andmay be corrected by a Knapp procedure alone. Twosuch patients did not require further surgery, and tworequired a small posterior levator resection. Beard'reported a lack of impressive improvement using a

Knapp procedure alone. Our experience suggeststhat this depends on the levator function.

Pseudoptosis may be difficult to assess in youngchildren, particularly where fixation by the affectedeye is poor, secondary to amblyopia. Where themagnitude of the pseudoptosis is in doubt, it shouldbe assumed to exist in the presence of a hypotropicglobe. The globe should be elevated first with a

Knapp procedure before ptosis surgery is performed.Satisfactory elevation of the globe and improve-

ment of the Bell's phenomenon was achieved in allfour cases not previously operated on, including onewith a large deviation (55 dioptres). Patients who hadpreviously undergone surgery to the vertical rectusmuscles were not corrected until a Knapp operation

was performed. Full correction of blepharoptosis cansafely be planned only in the presence of an adequateBell's response, and in our experience the Bell'sphenomenon was usually improved after a Knappprocedure. The magnitude of levator resection canbe reduced where exposure problems are antici-pated, as in the absence of a good Bell'sphenomenon.

Inferior rectus recession may cause lower lidretraction, which can aggravate corneal exposure. Itis significant, therefore, that in this series it was notfound necessary to recess the inferior rectus in mostcases (two out of 15 in the congenital double elevatorpalsy group). This emphasises the importance ofpreoperative assessment by means of the forcedduction test in deciding upon inferior rectus reces-sion. The age factor, with respect to anterior segmentischaemia, must be borne in mind when consideringdisinsertion of more than two extraocular muscles.Most patients in this series ranged from 3 to 18 yearsof age, but one aged 23 underwent an uncomplicatedKnapp procedure with simultaneous inferior rectusrecession without ischaemic problems.

Inferior rectus recession was routinely performedby Callahan4 and inferior rectus retraction requiringrecession was reported in 73% of cases by Scott andJackson. The need for this high rate of inferior rectusrecession was not confirmed in this series.The management of the ptosis in patients with jaw-

winking has received much attention."' This seriespoints to the relatively high incidence of jaw-winkingin patients presenting with a ptosis and hypotropia(25%) and should alert the ophthalmologist to lookfor the Marcus-Gunn phenomenon in all patientspresenting in this way. The importance of extraocularmuscle surgery in achieving symmetry of the eyes andan improved Bell's phenomenon cannot be over-emphasised, as this governs the type of ptosis surgerywhich is subsequently indicated.The cases with third nerve palsy illustrate the

importance of normal horizontal rectus muscles inthe achievement of good results following Knappprocedures. The patient with misdirection regenera-tion required supplementary contralateral surgery tothe horizontal rectus muscles with a Faden procedureon the lateral rectus to increase adduction of theaffected eye. This in turn aided active elevation of thehypotropic globe. Poor elevation of the globe limitedthe amount of levator resection it was safe toperform. Symmetry was therefore achieved by aplanned, small, ipsilateral levator resection com-bined with contralateral upper lid recession. Bothpatients with congenital third nerve palsy were foundto have persistently poor elevation following theirKnapp procedures. They did not suffer fromexposure problems, despite full correction of their

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true ptosis, but it should be remembered that tearproduction is generally adequate to compensate inyoung patients.The patients with neurofibromatosis have a con-

tinuing problem with recurrence of abnormal lidtissue during the growing period. Thus debulkingmust often be repeated. The levator is often grosslyinfiltrated, such that measurement of a resection isinaccurate. The globe may be buphthalmic as well ashypotropic. A Callahan procedure was preferred inone case because of the large abnormal globe, whichwas at risk of ischaemia. Levator resection followingeither a Callahan procedure or a Knapp operationachieved good results in two patients. The thirdpatient was educationally subnormal, and debulkingwith levator resection provided a satisfactory result.The last two cases show that the superior rectus may

be weakened mechanically by a superior rectus slingwhich incompletely corrects the original ptosis. Themanagement follows the same general principles ofactive elevation of the globe and levator surgery.Thus removal of the sling with division of adhesionsto the superior rectus was the first step, and it couldbe supplemented by a Knapp operation. This wasfollowed by appropriate levator surgery-reinser-tion in the traumatic case and resection in thecongenital ptosis case.

In summary, hypotropia, ptosis, and levator func-tion must be assessed carefully. Surgical correction isbest achieved by the logical sequence of a Knappprocedure followed by levator resection. This elimi-nates the confusion introduced by pseudoptosis andprovides a bonus safety factor of an improved Bell'sphenomenon at the time of lid raising. Horizontaldeviations are conveniently corrected by modifica-tions of the Knapp procedure. Inferior rectus reces-

sion is seldom required, and it is known to increasecomplications related to the lower lid position andnormal lid closure. Superior rectus resection whenthe muscle is known to be weak is illogical andunhelpful. It can be associated with disturbance ofthe upper fornix, which may require mucous mem-brane grafting. Superior rectus surgery is said toimpair levator function, since the attachment of thecommon sheath is interfered with. This may make theptosis worse, often without any significant effect onthe position of the globe. After a Knapp procedure alevator resection may be quantified and reliableptosis correction thereby achieved. A brow suspen-sion may be substituted where levator function is verypoor or in jaw-winkers in whom the levator muscle isexcised.

References

I Beard C. The surgical treatment of blepharoptosis: a quantita-tive approach. Trans Am Ophthalmol Soc 1966; 64: 401-87.

2 Knapp P. The surgical treatment of double elevator paralysis.Tratis Am Ophthalinol Soc, 1969; 67: 304-23.

3 Scott WE, Jackson OB. Double elevator palsy: the significanceof interior rectus restriction. Am OrthoptJ 1977; 27: 5-10.

4 Callahan M. Surgically mismanaged ptosis associated withdouble elevator palsy. Arch Ophthalmnol 1981; 99: 108-12.

5 Jensen EDF. Rectus muscle union: a new operation for paralysisof the rectus muscies. Tranis Pac Coast Oto-Ophthalmnol Soc1964; 45: 359-87.

6 Lee JP, Collin JRO, Timms C. Elevating the hypotropic globe.BrJ Ophthaltnol 1986; 70: 26-32.

7 Alvaro M. Simultaneous surgical correction of vertical andhorizontal deviations. Ophthaltnologica 1950; 120: 191-7.

8 Collin JRO. A anatiual of .sYstemnaic eYelid surgerY. Ist cd.Edinburgh: Churchill Livingstone, 1983: 44.

9 Beard C. Ptosis. 3rd ed. St Louis: Mosby, 1981: 142.10 Beard C. Ptosis. 3rd ed. St Louis: Mosby, 1981: 208.

A c c eptedfor public ationi 20 January 1986.

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