Purpose
To determine the clinical profile, causes, and outcomes of “spontaneous” vitreous hemorrhage in children (<18 years).
Design
Retrospective computer-assisted chart review.
Methods
Charts of 124 eyes of 76 children who presented with nontraumatic, nonsurgical vitreous hemorrhage between 2002 and 2012 were reviewed. All children underwent an appropriate ocular and systemic examination. Data collected included demographics, visual acuity, cause of “spontaneous” vitreous hemorrhage, ocular and systemic findings at presentation and at last follow-up, investigations, management details, and visual outcomes.
Results
The median age was 153.45 ± 56.19 months. There were 39 female and 37 male patients. Forty-eight patients had bilateral vitreous hemorrhage. The most common presenting complaints were diminished vision (96.45%) and behavioral changes (87.24%). The mean baseline BCVA in logMAR was 2.25 ± 1.11. The most common causes included vasculitis (34.6%) and hematologic disorders (27.4%). Patients were given medical therapy (topical and/or systemic) or underwent laser photocoagulation (29%) and/or surgery (55.6%). Twenty-nine eyes (23.3%) did not require any intervention. The mean number of surgeries was 1.89 ± 1.45 (range 1-4 surgeries). The mean final visual acuity was 0.76 ± 0.58 logMAR and was significantly better than the baseline ( P < .001). The best anatomic and visual outcomes were seen in vasculitis, whereas congenital disorders such as retinoschisis had the worst.
Conclusions
Spontaneous pediatric vitreous hemorrhage has a diverse etiology, vasculitis being the most common cause in our series. A comprehensive evaluation (systemic and ocular) is required to ensure that vision- and life-threatening conditions are not missed.
Vitreous hemorrhage, by itself, is an insufficient diagnosis and is an indicator of the need for a systematic analysis of its etiology. The diverse etiology can range from purely ocular conditions, such as posterior vitreous detachment, to diseases with an underlying systemic etiology, such as hypertension, diabetes mellitus, or vasculitis. Concurrent pathology is often the main determinant of the anatomic, visual, and functional outcome in any patient with vitreous hemorrhage.
Vitreous hemorrhage, its underlying causes, and their management are well described in adults. Literature on vitreous hemorrhage in children is very limited, including mostly case reports and case series. A large series by Spirn and associates has added considerably to our understanding of the factors causing and the determination of the etiology-specific outcomes of vitreous hemorrhage in children. Trauma, expectedly, was found to be the dominant cause of vitreous hemorrhage in children. Certain other causes leading to so-called “spontaneous vitreous hemorrhage,” some of which can be life threatening, merit further discussion. These potentially life-threatening conditions include retinoblastoma or hematologic disorders. It is often very difficult to establish a correct diagnosis in children, as history and elucidation of signs and symptoms is often not forthcoming. Considerable regional variation can be expected when analyzing a condition with such varied etiology, given that each place is socioeconomically, ethnically, and culturally diverse. It follows, thus, that appropriate documentation of characteristics that define or are common to vitreous hemorrhage secondary to such conditions is essential, as an aid to establishing the correct diagnosis.
We thus aimed to determine the causes, clinical presentation, and anatomic and functional outcomes of “spontaneous” vitreous hemorrhage in the pediatric population as noted at a tertiary eye care center in India.
Methods
A retrospective computer-assisted database search and chart review of all patients aged less than 18 years with vitreous hemorrhage who presented to L.V. Prasad Eye Institute, Kallam Anji Reddy campus, Hyderabad between January 1, 2002 and May 31, 2012 was conducted. All procedures conformed to the Declaration of Helsinki for research involving human subjects. The Institutional Review Board of L.V. Prasad Eye Institute, Hyderabad, India approved this retrospective review of these patients’ records for the study. Written informed consent for all subjects was taken from their guardians/parents for the diagnostic and treatment procedures conducted. Cases of vitreous hemorrhage secondary to retinopathy of prematurity (ROP) or trauma (surgical or nonsurgical) and those with incomplete records or diagnosis and/or a follow-up of less than 1 month were excluded. The chart review adhered to previously established guidelines, described in earlier publications. Data collected included demographics; laterality; presenting symptoms and signs (if the child was old enough to cooperate for appropriate history taking; if not, information about the onset of symptoms, possible causes, and previously existing conditions, if any, was obtained from parents); the presenting corrected distance visual acuity; details of the examination procedure; additional investigations and systemic examination, if conducted; the cause, if evident; the treatment offered; the duration of follow-up; and the final anatomic and visual outcomes. Visual acuity was measured using the ETDRS (Early Treatment Diabetic Retinopathy Study) chart and the results subsequently converted to logarithm of minimal angle of resolution (logMAR) chart and noted in logMAR units. For children in the age range of 6 months to 4 years, Teller acuity charts were used and similarly converted to logMAR units. A comprehensive ocular examination was performed in all cases, including under short general anesthesia if the child was not cooperative. If media clarity was insufficient to permit adequate fundal examination, or when additional information, such as choroidal thickness or orbital status, was required, an ultrasound B-scan was performed (OPKO OTI Pvt Ltd., Miami Florida, USA). Further investigations were decided based on the clinical impression. Investigations included ocular coherence tomography (Carl Zeiss Meditec Pvt Ltd., Erlangen, Germany); fluorescein angiography (including under anesthesia, if indicated); visual evoked potential; radiologic imaging (such as computerized tomography scans); blood and urine examinations such as complete blood count, erythrocyte sedimentation rate, C reactive protein, Quantiferon tuberculosis (TB) test, serum calcium, serum angiotensin converting enzyme level, rheumatoid arthritis factor, antinuclear antibody, and urine gross and microscopic examination; and systemic and laboratory tests for specific vasculitis. Treatment, both ocular and systemic, was planned on the basis of diagnosis and can be broadly classified as surgical or nonsurgical. Surgical intervention ranged from vitreous biopsy as a diagnostic aid to combined buckling and vitreoretinal procedures for associated vitreoretinal pathology. Nonsurgical therapy consisted of observation, topical or systemic medical therapy, and/or laser photocoagulation. Frequent cycloplegic refractions and amblyopia therapy by part-time occlusion was part of the management protocol.
Statistical analysis was performed using SPSS (v. 16; SPSS, Inc, Chicago, Illinois, USA), with special emphasis on the aforementioned information. Statistical significance was set at P < .05.
Results
A total of 619 pediatric patients with an entered diagnosis of vitreous hemorrhage in our medical records department database were identified. Analysis after exclusion of acute ROP cases (n = 34 patients) and those with an incorrect diagnosis (n = 27 patients) or coding errors (n = 13) was performed on 545 patients.
A total of 656 eyes with any vitreous hemorrhage (as the diagnosis) were available for analysis. We identified and included 76 patients (124 eyes; 18.09% of the total number of pediatric patients (619) with vitreous hemorrhage who presented to our institute in the stated period) of nontraumatic and nonsurgical “spontaneous” vitreous hemorrhage and performed the analysis. Six patients had an identified cause of spontaneous vitreous hemorrhage; a follow-up of less than 1 month led to their exclusion. Their baseline characteristics did not differ significantly from those included in the study.
The median age of the patients was 12.78 ± 4.68 (standard deviation) years (range 4 months to 17 years). There was no significant sex bias (39 female vs 37 male patients). Forty-eight patients presented with bilateral vitreous hemorrhage; Table 1 shows the proportion of patients having a specific cause in different age groups. All patients with an identified cause for the vitreous hemorrhage had local and/or systemic comorbidities, which provided the underlying pathologic mechanism of hemorrhage.
| Causes (n = Eyes) | <1 Year Age (14 Eyes) | >1-10 Years Age (41 Eyes) | >10 Years Age (69 Eyes) |
|---|---|---|---|
| Vasculitis | – | 9 | 34 |
| Hematologic disorders | – | 13 | 21 |
| Ocular tumors/vascular malformations | 3 | 4 | 1 |
| Familial exudative vitreoretinopathy | 3 | 8 | 2 |
| Retinoschisis | – | 2 | 3 |
| Retinal breaks/detachment | – | 2 | 11 |
| Diabetic retinopathy | – | – | 4 |
| Pars planitis | 2 | – | – |
| Hepatorenal dysfunction | – | 1 | – |
| Sepsis | 1 | – | – |
| Unknown etiology | 5 | 2 | 1 |
The mean follow-up was 28 ± 18.38 months. The most common presenting complaint in children aged more than 3 years was diminished vision (96.45%). The most common presenting complaint in children aged more than 3 years was diminished vision (119 of 124 patients, 95.96%). The most common presenting complaint in children aged less than 3 years (as reported by the parents) was behavioral changes (110 of 124 patients, 88.7%), such as not being able to recognize the mother, or a recent-onset lack of interest in surroundings. The mean baseline corrected distance visual acuity in logMAR was 2.25 ± 1.11. In addition to vitreous hemorrhage, 76 eyes (61.3%) had sufficient view of the fundus and showed typical features of the underlying disease on fundus examination. A total of 51 eyes required an ultrasonography for evaluation of the posterior segment of the eye. Fifty patients required hematologic, immunologic, or radiologic investigations.
Patients were given medical therapy (topical and/or systemic) or underwent laser photocoagulation (36 eyes) and/or surgery (69 eyes). Overall, 69 eyes (55.64%) required some form of surgical intervention, such as scleral buckling, vitrectomy, lensectomy, plaque placement, enucleation, or diagnostic vitreous biopsy. Table 2 shows various strategies for management in different age groups. The mean number of surgeries was 1.89 ± 1.45, with a range of 1-4 surgeries. None of the operated cases developed endophthalmitis or secondary glaucoma. A total of 19 eyes underwent surgery for tractional or rhegmatogenous retinal detachment, with 3 eyes having a recurrent retinal detachment at final follow-up. Two of these eyes had familial exudative vitreoretinopathy (FEVR) and 1 eye had retinoschisis.
