Lens-sparing vitrectomy for shaken baby syndrome

Fiona Chew Lee Min, Joseph Alagaratnam, Sunder Ramasamy, Jamalia Rahmat, Irene Guat-Sim Cheah, Azmi Alias, Karunakar Tirmandas Venkata Narsimha

1Department of Ophthalmology, Hospital Kuala Lumpur, Kuala Lumpur 50586, Malaysia

2Department of Paediatrics, Hospital Kuala Lumpur, Kuala Lumpur 50586, Malaysia

3Department of Neurosurgery, Hospital Kuala Lumpur, Kuala Lumpur 50586, Malaysia

Correspondence to:Fiona Chew Lee Min. Department of Ophthalmology, Hospital Kuala Lumpur, Jalan Pahang, Kuala Lumpur 50586, Wilayah Persekutuan, Malaysia. sabrefmin@gmail.com

Received： 2014-11-14　　Accepted： 2015-08-20

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Lens-sparing vitrectomy for shaken baby syndrome

Fiona Chew Lee Min1, Joseph Alagaratnam1, Sunder Ramasamy1, Jamalia Rahmat1, Irene Guat-Sim Cheah2, Azmi Alias3, Karunakar Tirmandas Venkata Narsimha1

1Department of Ophthalmology, Hospital Kuala Lumpur, Kuala Lumpur 50586, Malaysia

2Department of Paediatrics, Hospital Kuala Lumpur, Kuala Lumpur 50586, Malaysia

3Department of Neurosurgery, Hospital Kuala Lumpur, Kuala Lumpur 50586, Malaysia

Correspondence to:Fiona Chew Lee Min. Department of Ophthalmology, Hospital Kuala Lumpur, Jalan Pahang, Kuala Lumpur 50586, Wilayah Persekutuan, Malaysia. sabrefmin@gmail.com

Received： 2014-11-14Accepted： 2015-08-20

保留晶狀體的玻璃體切除術(shù)治療嬰兒搖蕩綜合征

引用：Chew FLM, Alagaratnam J, Ramasamy S, Rahmat J, Cheah IGS, Alias A, Karunakar TVN. 保留晶狀體的玻璃體切除術(shù)治療嬰兒搖蕩綜合征.國際眼科雜志2016;16(2):213-217

Abstract

?AIM: To document the ophthalmological findings and visual outcomes for shaken baby syndrome (SBS) patients who had lens-sparing vitrectomy (LSV) for non-resolving premacular and vitreous hemorrhages obscuring the macula.

?METHODS: A retrospective review and statistical analysis of ophthalmological and demographic data of patients admitted with SBS from 2010 to 2012 was done. Patients with premacular and vitreous hemorrhage obscuring the macula who underwent LSV were included as subjects. Paired samplest-test was used to compare means, categorical data was analysed using Fisher’s exact test and Pearson Chi-squared test.Pvalue of less than 0.05 was considered as statistically significant.

?RESULTS: Thirty two subjects were recruited with a mean age of 5.09±1.96mo. Twenty one (65.6%) subjects had visual acuity of no light perception at presentation. Subjects with poorer initial visual acuity had larger pupil sizes (P=0.021) and most of them had neurosurgical intervention (P=0.027). The mean duration to perform LSV was 28.56±20.83d. Our vitrectomy complication rate was 4.26%. Post-operatively, 26 (80%) subjects had vision of light perception or better, the spherical equivalent was significantly more myopic (P=0.001).

?CONCLUSION: Prompt ophthalmological assessment is vital to ensure optimum visual rehabilitation in SBS patients. With low complication rates, early surgical intervention with LSV represents a promising option for non-resolving intraocular hemorrhages obscuring the macula in SBS.

KEYWORDS:?vitrectomy; shaken baby syndrome; macula hemorrhage; vitreous hemorrhage

Citation:Chew FLM, Alagaratnam J, Ramasamy S, Rahmat J, Cheah IGS, Alias A, Karunakar TVN. Lens-sparing vitrectomy for shaken baby syndrome.GuojiYankeZazhi(IntEyeSci) 2016;16(2):213-217

INTRODUCTION

Shaken baby syndrome (SBS) causes a significant amount of morbidity and mortality to the paediatric population. SBS is diagnosed by the triad of retinal hemorrhages, subdural hemorrhage and encephalopathy[1]. It is reported that 64-70% of SBS patients never fully recover from their injuries sustained and 15-38% of SBS patients die during their hospital admission[2-3]. Abnormal vision has been reported in 65% of SBS survivors[4]. Premacular and vitreous hemorrhages in SBS frequently obscure the macula and become an amblyogenic threat to the developing eye. Lens-sparing vitrectomy (LSV) has been suggested as a viable option to treat persistent visually-depriving hemorrhages in children as it reduces the rate of cataract formation and negates the need for lensectomy for surgical access to the pars pliacata region[5-7]. Limited data however exists regarding the eventual visual outcomes and safety profile of LSV in SBS. This study aims to document the ophthalmological findings and visual outcomes for SBS patients who had LSV for non-resolving premacular and vitreous hemorrhages obscuring the macula.

SUBJECTS AND METHODS

This retrospective study was conducted in Hospital Kuala Lumpur (HKL) from Nov.1st2012 to Mar.1st2013. The study and data collection conformed to all local laws and was compliant with the guidelines of the Declaration of Helsinki. Ethical approval was obtained from the Medical Research and Ethics Committee of the Malaysian Ministry of Health (NMRR ID NMRR-13-359-15951 S1 R0). All consecutive patients admitted to the pediatric and neurosurgical wards with a diagnosis of subdural haemorrhage secondary to suspected SBS from Jan.1st2010 to Dec.31st2012 were identified from ward registers. The patients were recruited as study subjects if they had documented evidence of premacular or vitreous hemorrhage confirmed by ophthalmological assessment and had LSV. The patients were excluded if they had trauma or disease which caused the intraocular or subdural hemorrhage. The patients were also excluded if they passed away during admission or did not have a minimum of 1mo ophthalmological follow-up postoperatively.

The criterion for subjects to undergo LSV was premacular or vitreous hemorrhage obscuring the macula which did not spontaneously resolve within a period of 4wk of observation or severe vitreous hemorrhage which completely obscured fundal view. All the LSV surgeries were performed under general anaesthesia by a single surgeon. The distance of the sclera incision from the corneal limbus ranged from 1.5 mm to 2.0 mm depending on the age of the child. A three-port, trans pars plana vitrectomy (23 gauge system) using Topcon Offis Wide angle viewing system was used according to the method already described in previous literature[6].

Epidemiological data consisted of the patients’ ages, gender, ethnic group, primary caregiver, birth history, medical problems, family members, presenting symptoms, medical and neurosurgical treatment and eventual outcomes.Ophthalmological data collected included presenting visual acuity, documented eye findings, ocular surgical intervention and final visual acuity after a minimum of 3mo follow-up. For all subjects, data from one eye was taken for data analysis. The eye chosen was the eye which underwent LSV. If both eyes had LSV, then the worst eye was chosen for analysis. Visual acuity at presentation was divided into visual acuity of perception of light or non-perception of light. Pupil measurements were taken using a ruler. Anterior segment examination was done using a portable slit lamp and binocular indirect ophthalmoscopy was utilised for fundus examination. Intraocular pressure was measured using tonopen. All data was entered into a standardized data collection sheet and analyzed by an ophthalmologist. No new investigations were ordered and all data was handled anonymously. There was no communication between the researcher and the patient, the patient’s family, the patient’s treating physicians or the investigating police officers or social welfare officers during the course of data collection.

The data collected was entered into an Excel spreadsheet. SPSS version 13 (SPSS Statistics: Windows Student Version 13, Chicago, Illinois, USA) was used for data analysis. Paired samplest-test was used to compare means and categorical data was analysed using Fisher’s exact test and Pearson Chi-squared test.Pvalue of less than 0.05 was considered as statistically significant.

RESULTS

The number of patients admitted with suspected SBS was 17, 23 and 36 patients in the year 2010, 2011 and 2012 respectively. A total of 32 subjects were recruited from the 76 patients admitted with suspected SBS and data from 32 eyes were used for statistical analysis. Nine patients were excluded as they had passed away during admission and 35 patients were excluded as they did not require LSV. The subjects recruited represented 42.11% of all patients admitted to HKL for suspected SBS within a 3-year period.

The subjects’ ages ranged from 2 to 11mo of age with a mean age of 5.09mo (SD: 1.96). More than half of the subjects (59.4%) were males. A majority of the subjects (90.6%) were Malay, followed by Chinese patients. Only 1 subject was born prematurely. The mean birth weight of the subjects was 2.97 kg (SD: 0.49). The medical history of all subjects was unremarkable except for 1 subject who had Down’s syndrome. Almost half of the subjects (47.2%) did not have siblings while the remaining subjects that had siblings were the youngest in their respective families. Most of the subjects (81.8%) were in nursery or in the care of a babysitter when the incident occurred.

Table 1Pre-operative visual acuity, pupillary size and subjects requiring neurosurgical intervention

Pre-operativedetailsVisualacuityBetterthanNPLNPLPupilsize(n=32) <4mm850.021 ≥4mm316Neurosurgicalintervention(n=32) Yes3150.027 No86

PL: Perception of light; NPL: Non-perception of light.

Fifty percent (16/32) of the subjects presented with seizures and 37.5% (12/32) of subjects were found unresponsive at the time of presentation to hospital. The majority of the subjects had a Glasgow Coma Scale (GCS) score of less than 8 at the time of hospital admission. Ninety point six percent (29/32) subjects had their first ophthalmological assessment within 10d of hospital admission. The pupillary size of the subjects ranged from 2 mm to 8 mm with a mean of 4.91 (SD: 1.86). No documentation was available with regards to environment illumination during pupillary size measurement. The subjects presenting spherical equivalent was plano to +5.50 D with a mean of 1.68 D (SD: 1.54).

All subjects had normal anterior segment findings and normal intraocular pressure. Twenty-nine subjects (90.6%) had bilateral intraocular haemorrhages. Out of the 61 eyes with intraocular hemorrhage, 22 eyes were noted to have traumatic macular retinoschisis from fundus examination. Ten eyes presented with vitreous hemorrhage. Upon first presentation, 65.6% (21/32) subjects had visual acuity of non-perception of light (NPL) while 34.4% (11/32) subjects had visual acuity of perception of light. Our study found that subjects with presenting visual acuity of NPL had larger pupils (P=0.021) and more of them had neurosurgical intervention (P=0.027) in comparison to subjects with visual acuity of better than NPL. (Table 1)

The duration to perform LSV ranged from 8 to 90d post ocular diagnosis with a mean of 28.56d (SD: 20.83). Seventy-eight point one percent (25/32) of subjects had LSV within 30d post ocular diagnosis. Four subjects had delayed surgery beyond 2mo as 2 subjects were intially unfit for surgery and 2 other subjects had been treated at other health facilities before being transferred to our center for non-resolving vitreous hemorrhage. Out of the 61 eyes presenting with intraocular hemorrhage, 47 eyes underwent LSV. Two of the 47 eyes that underwent vitrectomy had surgical complications. One eye developed retinal detachment post-operatively which was treated with circumferential scleral buckling and laser. The subject’s eye later developed cataract and plain lens aspiration was done in view of the poor visual prognosis. Another subject’s eye had an iatrogenic retinal break during peeling of the internal limiting membrane. The retinal break was treated successfully with barricade laser and the subject had good post-operative vision of 6/24 at 3y follow-up. Twenty-one point eight percent (7/32) subjects underwent simultaneous bilateral LSV. No cases of endophthalmitis or persistently raised intraocular pressure were reported.

Table 2Final visual outcomes of lens-sparing vitrectomy

FinalVisualoutcomesPreopPostopPVisualacuity NPL21(65.6%)6(18.75%) PLorbetter11(34.4%)26(81.25%)0.000SE(x±s)1.68±1.54-1.03±1.910.001

NPL: No perception of light; PL: Perception of light; SE: Spherical equivalent.

The follow-up of subjects post-operatively ranged from 1 to 48mo (Mean: 10.75, SD: 10.97). Eighty percent of the subjects (26/32) had vision of perception of light or better, and the post-operative spherical equivalent was significantly more myopic in comparison to the spherical equivalent at presentation (Table 2). Fifty-six point three percent (18/32) subjects required neurosurgical intervention for intracerebral hemorrhage and 78.1% (25/32) of subjects had signs of developmental delay.

DISCUSSION

An increasing number of patients with suspected SBS were admitted to our centre every year. This trend is worrying as SBS is a preventable tragedy. The increase may be an actual increase or secondary to heightened awareness regarding SBS. Urbanisation of Malaysia has resulted in more women entering the workforce. This has caused an increase of childcare by babysitters or nurseries, with many having untrained caregivers who are ill prepared to handle young infants[8]. Our study noted that babysitters and nurseries made up more than 80% of the primary caregivers of the affected children. Our SBS subjects’ ages ranged from 2 to 11mo and majority of the subjects were male patients. This corresponded to published reports on SBS[4,9]. Prematurity was not a risk factor for SBS as the majority of our study subjects were born at full term. This contrasted with studies which cited prematurity as a risk factor for SBS[10]. In addition, only 1 of the 32 patients was developmentally delayed prior to hospital admission.

Most of our subjects were admitted with seizures, or were found unresponsive at first hospital presentation. This was consistent with published clinical presentations of SBS[11]. Larger pupillary size was reported to be associated with poorer survival in trauma patients[12]. We noted that a pupillary size of 4 mm or larger was associated with poorer visual acuity at initial hospital presentation. This may reflect the increased severity of ocular and cerebral trauma inflicted, and explain why more of these subjects had neurosurgical intervention. The duration from the time of admission until an ophthalmological assessment was performed ranged from 0 to 15d. Reasons for delayed eye assessment included the unstable condition of patients, misdiagnosis or missed eye referrals during transfer between wards or hospitals. An increased awareness for an examination by an ophthalmologist is essential as missed retinal haemorrhages may lead to missed diagnosis of SBS. In previous literature, non-ophthalmologists were only able to identify 87% of retinal hemorrhages in SBS patients[13].

A majority of subjects were noted to have bilateral intraocular hemorrhages. This was consistent with Maguire and colleagues who noted a predominance of bilateral retinal hemorrhages in SBS[14]. Vitreous hemorrhage is an uncommon but significant finding in SBS as it has the most evidence linking it to SBS. It is believed to be secondary to progressive breakthrough bleeding of retinal hemorrhages into the vitreous over a number of days and is not thought to be an acute event[15]. We noted that SBS subjects presenting with vitreous hemorrhage frequently required LSV as the vitreous hemorrhage was non-resolving. The non-resolution of vitreous hemorrhage in SBS may be due to injury chronicity and poor blood absorption by the infantile vitreous.

Early visual stimulation is crucial for the development of neural infrastructure for visual learning[16]. A minimum vision of perception of light is required for circadian rhythms and maximizing the potential for cognitive skills in children[17]. Hence, any form of visual deprivation regardless of duration will have a lasting impact on a child’s visual development even after the deprivation has ended. This emphasizes the necessity for early removal of intraocular haemorrhages obscuring the macula to reduce the amblyogenic potential of these hemorrhages. In addition, early removal of such haemorrhages has a protective effect on the macula as late retinal scarring has been documented in SBS patients due to the migration of blood from the retina to the vitreous and excess iron causes oxidative damage to the retina[18-19]. With the advent of LSV, the treatment of amblyogenic, persistent intraocular haemorrhage is surgical. The ideal time for LSV for SBS is not known but it is suggested that earlier surgical intervention may be best between 11 to 28d post ocular diagnosis in a child with SBS[20]. The majority of our study subjects had LSV within 30d of ocular diagnosis.

We noted that early intervention for non-resolving hemorrhages obscuring the macula allowed for earlier visual rehabilitation. This was supported by the fact that even though 65.6% (21/32) subjects had poorer vision at presentation, 81.25% (26/32) subjects had a visual acuity of perception of light or better post LSV. Twenty out of the 32 cases who went for vitrectomy had normal fundi post-operatively. Although only 5 subjects managed to achieve vision better than 6/24 at 3y follow-up, the final visual acuity may be much better should the subjects be followed up longer. Our vitrectomy complication rate was 4.26% where 2 of the 47 vitrectomised eyes developed complications. This result is better in comparison to the study by Capone[6]who noted a complication rate of 2 out of 16 eyes (12.5%) which underwent LSV for infantile vitreous haemorrhage. Of the 10 subjects that completed a post-operative follow-up of 6mo or longer, there was no documented cataract or development of glaucoma noted.

Myopic progression was noted in our study subjects post LSV. This myopic progression could be similar to the myopic progression seen in adults post vitrectomy, where the myopic shift was secondary to corneal or sclera stretching, anterior chamber depth changes and silicone oil tamponade[20]. Also, the infantile macula has been noted to be more vulnerable to the presence of blood leading to progressive myopia[21]. In addition, the presence of dense vitreous hemorrhage could have caused stretching of the cornea and sclera leading to corneal enlargement and increase in axial length[20]. This could not be confirmed in our study as no A-scans were done to document the axial length pre- and post-vitrectomy.

A majority of the subjects were eventually noted to be developmentally delayed.This may be due to the chronicity and increased severity of the ocular and brain injuries sustained. The chronicity and increased severity of the brain injuries may also explain why some subjects had later eye assessments as the subjects may have been medically unstable for eye examination and may have been transferred to multiple wards leading to missed diagnosis.

The limitations of our study were that it was a retrospective study comprising of a small number of patients. The follow-up was short and data collection was dependant on the available documentation. Documentation regardig pupillary responses and afferent pupillary defect were sparse due to the difficulty of checking relative afferent pupillary defect in children. Also, investigations like visual evoked potential, electroretinogram and visual fields were not performed for quantitative visual assessment. Despite the limitations, our study is the largest to date and the first in reporting the results of LSV for premacular and vitreous hemorrhage obscuring the macula for SBS patients in Malaysia. HKL is also the sole tertiary referral centre for LSV for children in Malaysia and may accurately reflect the presentation of SBS patients requiring LSV in the urban Malaysian population.

With low complication rates, our study suggests that early surgical intervention with LSV is a viable option for non-clearing premacular and vitreous hemorrhages obscuring the macula in SBS. This study also highlights the need for early ophthalmological assessment to ensure optimum visual rehabilitation in SBS patients.

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·Original article·

Visual and refractive outcomes with ReLEx?SMILE in 600 eyes

Sri Ganesh1, Rishika Gupta2

1Chairman and Managing Director, Nethradhama Super Speciality Eye Hospital, Bangalore 560082, India

2Department of Ophthalmology, Nethradhama Super Speciality Eye Hospital, Bangalore 560082, India

Correspondence to:Rishika Gupta. Nethradhama Super Speciality Eye Hospital, 256/14, Kanakpura Main Road, 7thblock Jayanagar, Bangalore 560082, India. rishikagupta55@yahoo.com

Received： 2015-03-24Accepted： 2015-08-20

·Original article·

Fiona Chew Lee Min1, Joseph Alagaratnam1, Sunder Ramasamy1, Jamalia Rahmat1, Irene Guat-Sim Cheah2, Azmi Alias3, Karunakar Tirmandas Venkata Narsimha1

(作者單位：1馬來西亞，吉隆坡 50586，吉隆坡醫(yī)院眼科；2馬來西亞，吉隆坡 50586，吉隆坡醫(yī)院兒科；3馬來西亞，吉隆坡 50586，吉隆坡醫(yī)院神經(jīng)外科)

摘要

目的：分析嬰兒搖蕩綜合征(SBS)患者黃斑前及玻璃體出血遮擋黃斑的眼部特征及視力?；颊呔邮鼙Ａ艟铙w的玻璃體切除術(shù)(LSV)。

方法：回顧性研究。選取2010年至2012年確診為黃斑前及玻璃體出血遮擋黃斑的SBS并接受LSV的患者，對其眼部特征和人口統(tǒng)計學數(shù)據(jù)進行分析。均數(shù)的比較采用配對t檢驗，分析分類數(shù)據(jù)采用Fisher精確檢驗和Pearson卡方檢驗。P<0.05有統(tǒng)計學意義。

結(jié)果：共32例患者納入本研究，平均年齡為5.09±1.96mo。在本研究中，21例(65.6%)患者視力為無光感。患者瞳孔較大者初始視力較差(P=0.021)，且大部分接受過神經(jīng)外科手術(shù)(P=0.027)。行LSV的平均間隔為28.56±20.83d。我院玻璃體切除術(shù)的并發(fā)癥發(fā)生率為4.26%。術(shù)后26例(80%)患者視力為光感或更好，術(shù)后等效球鏡大多為近視(P=0.001)。

結(jié)論：SBS患者的眼科評估對于最佳視覺預(yù)后非常重要。對于未清除眼內(nèi)出血致黃斑模糊的SBS患者，LSV的并發(fā)癥發(fā)生率較低，不失為早期手術(shù)治療中的好選擇。

關(guān)鍵詞：玻璃體切除術(shù)；嬰兒搖蕩綜合征；黃斑出血；玻璃體出血

DOI:10.3980/j.issn.1672-5123.2016.2.04

通訊作者：Fiona Chew Lee Min. sabrefmin@gmail.com