e2 Volume 13 Number 1 / February 2009

Results: The mean age at presentation was 2.3 years. RB1 mutantalleles were detected in 92% of cases, with 13% carrying a germlinemutation. Thirteen patients (IIRC Stage A:1; B:4; C:7; D:1) were initiallymanaged without primary enucleation. Three of these patients werediagnosed early as the result of family history. Four patients retainedgood vision in the eye with median follow-up 3.9 years (range, 0.4-11.3 years). Three patients eventually underwent enucleation.One died of metastases after parental refusal to accept primaryenucleation.

Discussion: Enucleation remains the treatment of choice for ad-vanced unilateral retinoblastoma. Conservative treatment is an op-tion when there is good potential to salvage useful vision withoutprolonged, costly therapy with potential side effects. Most childrenare best treated by simple enucleation, reducing the risk of maskinghigh risk pathology which may be a sign of metastases.

Conclusions: Current chemotherapy/focal therapy protocols donot replace primary enucleation as the treatment of choice for ad-vanced unilateral retinoblastoma.

006 Prognostic factors in infants with decreased visualresponsiveness. Roua Azmeh, Gregg T. Lueder

Introduction: Children may present in infancy because of the lackof visual responsiveness. This study evaluated prognostic factors topredict which infants would spontaneously improve.

Methods: This was a retrospective study of infants who were eval-uated for lack of visual responsiveness. Children were included ifthey met the following criteria: the presence of some visual fixation,normal pupil responses, and the absence of nystagmus and struc-tural ocular abnormalities. Children with developmental delay andpremature birth were excluded. Follow-up information was obtainedfrom office visits and telephone contact.

Results: One hundred five children were evaluated at 2-3 monthsof age for decreased visual responsiveness. Seventy-three were ex-cluded because of premature birth, developmental delay, or ocularabnormalities. Thirty-two children met the criteria noted previously.Six were excluded because of a lack of follow-up. Four were normalby the time of their initial examination. Follow-up for the remainingpatients ranged from 5 months to 11 years (mean, 4.2 years). One pa-tient (4%) developed strabismus and required surgery. The remainingpatients all had normal vision and none had developmental problems.

Discussion: The presence of some fixation, normal pupil re-sponses, and absence of nystagmus and structural ocular abnormal-ities is predictive of a good visual outcome in infants with decreasedvisual responsiveness.

Conclusions: The prognosis for infants with decreased visual re-sponsiveness who have the findings noted previously is excellent.Additional testing is not necessary unless other abnormalities arepresent. This information will be useful in counseling the parentsof such infants.

007 Palmaris longus tendon as an autogenous expander forBrown syndrome: A novel technique. Ahmad Batal, Osama Batal

Introduction: Palmaris longus tendon (PLT) is the most commondonor tendon in orthopedic and reconstructive practice and recentlyused in cardiac valve surgery. Here we present a new surgical tech-nique using this autogenous tissue for lengthening the superior obli-que tendon in cases with moderate to severe Brown syndrome.

Methods: Four children with Brown syndrome were carefullyselected and consented. Preoperative motility assessment and intra-operative forced duction test was done. Harvesting PLT was per-formed by the orthopedic surgeon in three patients, with the fourth

case performed completely by the ophthalmic surgeon under generalanesthesia. Follow-up ranged from 9-21 months.

Results: Preoperatively, our patients demonstrated hypotropia of16D-30D with some degree of abnormal head posture (AHP). Postop-eratively, all patients showed an improvement in hypotropia to \ 3D

(90%-100%) with complete resolution of AHP. Improvement of eleva-tion in adduction was not found to match that of vertical deviation, as2 patients improved from �4 to �2 (50%) and 2 improved by 60% to75%. None of the patients developed any of the complications en-countered with Silicon Expander like extrusion, tendon related in-flammation, foreign body sensation or consecutive superior obliquepalsy, and none of them needed further surgery. Wrist functionwas completely normal in all patients postoperatively.

Conclusions: Palmaris longus tendon is an effective autogenoustissue that can be used as a donor expander for superior oblique ten-don with good outcomes and minimal complications compared to thesynthetic material. More cases and studies to compare these twomaterials seem warranted.

008 Timing of surgery for subluxed lenses: A 10-year review. AjayBhatnagar, Harry E. Willshaw, Claire Dewsbury, John R. Ainsworth,Manoj V. Parulekar

Introduction: Management of subluxated crystalline lens in chil-dren is challenging because of the potential complications of surgeryand risk of amblyopia.

Methods: We conducted a retrospective review of all patients withsubluxated lens referred to a tertiary care paediatric eye unit duringthe past 10 years.

Results: A total of 32 cases seen between 1997 and 2007 were in-cluded: 45 eyes of 25 children underwent lensectomy with meanpostoperative follow-up 4.8 years (range, 2-9 years); 7 were managedconservatively (4 presented late with dense amblyopia; and 3 weremanaged with aphakic correction). All received refractive and am-blyopia treatment. A total of 24 eyes (53.3%) had postoperativebest corrected visual acuity (BCVA) # 0.3 LogMAR, and 11 (24%)were $0.6 LogMAR. Mean age at surgery in the group with good vi-sual outcome was 4.2 years (mean duration from diagnosis to surgery1.8 years) compared with 6.3 years for eyes with poor visual outcome(mean duration from diagnosis to surgery 2.9 years) p\0.01. Postop-erative glaucoma, retinal detachment, or contact lens problems didnot occur in any case. In our series, factors associated with poor vi-sual outcome were older age and longer interval from diagnosis tosurgery. Sixty percent of patients (15/25) achieved postoperativeBCVA #0.3 LogMAR in at least one eye.

Conclusions: Timing of surgery is important in the management oflens subluxation. Prolonged attempts at optical correction may resultin irreversible amblyopia and a poor visual result. Earlier surgery fol-lowed by contact lens aphakic correction is safe with a good visualoutcome.

009 Effect of granulocyte colony stimulating factor on retinopathyof prematurity. Rahul Bhola, Todd Purkiss, Stephen Hunter, DanStewart, Paul Rychwalski

Purpose: To study the effect of granulocyte colony stimulating fac-tor (GCSF) on the incidence and progression of retinopathy of prema-turity (ROP).

Methods: A retrospective chart review of all Neonates who re-ceived GCSF at our Neonatal Intensive Care Unit during a periodranging from January 2003 to September 2007 was performed. Ofthe 213 patients identified, 50 patients (Group 1) with a birth weight\1,500 g and gestational age\32 weeks were included in this study.

Journal of AAPOS