Indian Journal of Ophthalmology

REVIEW ARTICLE
Year
: 2019  |  Volume : 67  |  Issue : 6  |  Page : 732--739

Pediatric vitreous hemorrhage: A narrative review


Anmol U Naik, Ekta Rishi, Pukhraj Rishi 
 Shri Bhagwan Mahavir Vitreoretinal Services, Medical Research Foundation, Sankara Nethralaya, Chennai, Tamil Nadu, India

Correspondence Address:
Dr. Ekta Rishi
Senior Consultant, Shri Bhagwan Mahavir Vitreoretinal Services, Medical Research Foundation, Sankara Nethralaya, 18, College Road, Chennai - 600 006, Tamil Nadu
India

Abstract

Vitreous hemorrhage is one of the most common causes of sudden, painless loss of vision in adults. This is probably one of the reasons why it has been extensively studied and reported in literature. However, the same cannot be said when it comes to vitreous hemorrhage in the pediatric age group. The causes of vitreous hemorrhage in children tend to differ from those of adults. Not much data exist regarding their presentation and management. In addition to trauma, certain spontaneous causes such as pediatric tumors and congenital conditions assume importance while considering the differential diagnosis of vitreous hemorrhage in the pediatric age group. However, it is natural that the treating ophthalmologist is faced with challenges when a child presents with vitreous hemorrhage. In this narrative review, we have attempted to analyze the retrospective observational studies regarding pediatric vitreous hemorrhage reported in English literature till date. The article sheds some light on the prevailing epidemiology, management strategies employed and the visual outcome among different regions of the world.



How to cite this article:
Naik AU, Rishi E, Rishi P. Pediatric vitreous hemorrhage: A narrative review.Indian J Ophthalmol 2019;67:732-739


How to cite this URL:
Naik AU, Rishi E, Rishi P. Pediatric vitreous hemorrhage: A narrative review. Indian J Ophthalmol [serial online] 2019 [cited 2024 Mar 28 ];67:732-739
Available from: https://journals.lww.com/ijo/pages/default.aspx/text.asp?2019/67/6/732/259049


Full Text



Vitreous hemorrhage is defined as the presence of extra-vasated blood within the space outlined by the internal limiting membrane of the retina posteriorly and laterally, the non-pigmented epithelium of the ciliary body laterally, and the lens zonular fibers and posterior lens capsule anteriorly.[1] The etiologies, clinical features, and management strategies of vitreous hemorrhage have been well described in adults.[1],[2],[3],[4],[5] However, vitreous hemorrhage is rare in children and adolescents and is not well-described. Pediatric vitreous hemorrhage is a challenging entity in terms of diagnosis and management. The challenges in evaluation include difficulty in eliciting reliable history, poor patient cooperation and difficulty in assessing visual acuity.[6] Unlike adults, presence of co-morbidities, risks of anesthesia and post-operative management in terms of positioning (in case of tamponade) and refractive rehabilitation pose a challenge to comprehensively manage this entity.

There is paucity of literature regarding the epidemiology and management of vitreous hemorrhage in children. The causes of vitreous hemorrhage in the pediatric age group are different from the principal causes of vitreous hemorrhage such as posterior vitreous detachment and diabetic retinopathy in adults.[7] On reviewing the literature, different causes of pediatric vitreous hemorrhage have been published, most of these either being case reports or case series that have focused on different modalities of treatment.[8],[9],[10],[11],[12],[13],[14],[15],[16],[17],[18],[19],[20],[21],[22],[23],[24],[25],[26],[27],[28] There is lack of evidence comprehensively reviewing the etiologies or giving definite guidelines regarding the management of vitreous hemorrhage in the pediatric age group. We attempt to present a narrative review on the demographic profile, etiology, clinical manifestations, treatment modalities, and outcomes of vitreous hemorrhage in children from the major studies available in literature till date.

 Methods



PubMed database was searched using the keywords “pediatric,” “children,” “vitreous,” and “hemorrhage” in the title. The search retrieved 19 results of which six manuscripts in English [6],[29],[30],[31],[32],[33] dealt with epidemiology and outcomes of vitreous hemorrhage in children. Three of these studies [6],[29],[30] dealt with pediatric vitreous hemorrhage irrespective of laterality or cause. One each dealt with exclusively bilateral vitreous hemorrhage,[31] traumatic vitreous hemorrhage [32], and spontaneous causes of vitreous hemorrhage.[33] These six manuscripts were included for the purpose of this review. Non-English manuscripts and isolated case reports were excluded from this review.

The study design and main parameters of the articles reviewed are presented in [Table 1]. All six studies were hospital-based, had a retrospective design and were reported from three different geographical regions (United States,[29] India,[6],[31],[32],[33] and Saudi Arabia [30]). The studies on bilateral, traumatic, and spontaneous vitreous hemorrhage were reported from a single institute in India. Five studies [6],[29],[31],[32],[33] included patients less than 18 years of age in their sample, while Al Harkan et al. limited [30] their sample age to less than or equal to 16 years. The criteria of recruiting study subjects by various studies are comprehensively illustrated in [Table 2]. There was no uniformity in the criteria employed by different authors. While some used the International Classification of Diseases code,[6],[29] others stuck to their hospital database with diagnosis of vitreous hemorrhage with complete records and without any coding errors.[30],[31],[32],[33]{Table 1}{Table 2}

The demographic profile of study subjects of all the studies has been illustrated in [Table 3]. The age range for most of the studies was between 7–12 years. The mean age at diagnosis reported by Rishi et al.[6] was greater than that reported by Spirn et al.[29] and Al Harkan et al.[30] In studies conducted by Sudhalkar et al.,[31],[32],[33] the mean/median age was higher than the studies reported from outside India. It is evident that the mean age at presentation was greater in the studies reported from the Indian scenario than other regions. Rishi et al.[6] mention that this might be because of the greater proportion of subjects with Eales' disease in the Indian sub-continent. Eales' disease is endemic to South Asia and seen predominantly in the Indian subcontinent although rarely in the West.[34] Male children outnumbered female children in terms of proportion of cases presenting with vitreous hemorrhage in all the studies except in the study reporting spontaneous vitreous hemorrhage cases.[33] This gender difference was statistically significant as noted by Spirn et al.[29] in their study. Sudhalkar et al.[32] attribute this to the fact that male children are probably involved in aggressive indoor and outdoor activities more than their female counterparts. Most of the studies reported a higher proportion of unilateral cases except in case of spontaneous vitreous hemorrhage [33], where majority of the cases were bilateral. There was no predisposition to the eye that was involved, as reported in three studies.[6],[29],[30]{Table 3}

Decreased vision was the most common presenting symptom in most studies [Table 4]. An exception to this is the subset of children less than 3 years, where nystagmus and behavioral changes were the most common presenting complaints as reported by Spirn et al.[29] and Sudhalkar et al.,[32] respectively. This subset includes the pre-verbal children and it is natural that decreased visual acuity is not realized till the consequences of it such as strabismus/nystagmus (amblyopic sequelae) or behavioral changes set in.{Table 4}

Unilateral traumatic pediatric vitreous hemorrhage

A comparative description of the incidence of traumatic vitreous hemorrhage in the pediatric group along with mode of injury and etiologic agents is given in [Table 5]. An age-group wise distribution of the etiology within the pediatric patients was attempted by Rishi et al.[6] and Sudhalkar et al.[33] Rishi et al. divided the 0–18 years age groups into four sub-groups: <4 years (pre-school), >4 and <8 years (primary school), >8 and <12 years, and >12 and <18 years (adolescent). Sudhalkar et al.[33] divided the age range into three groups: <1 year, >1 to 10 years, and >10 years. Both studies found that the proportion of traumatic cases showed an upward trend with advancing age, i.e., as the age increased, the proportion of traumatic cases also increased [Table 6]. Trauma was the most common cause of pediatric vitreous hemorrhage in all studies. However, the proportion of cases because of the blunt versus penetrating trauma varied. All authors except Al Harkan et al.[30] reported blunt trauma to constitute a major proportion of the cases responsible for vitreous hemorrhage. This could possibly correlate with most common type of agents that were responsible for the trauma in the first place. For example, cricket ball or fist injuries are more likely to cause blunt trauma, while sticks, sharp objects such as needles are more likely to cause penetrating trauma.{Table 5}{Table 6}

Of the traumatic cases, a variety of concomitant posterior segment findings were noted in addition to vitreous hemorrhage. These include lens dislocation/subluxation, posterior vitreous detachment, retinal detachment, choroidal detachment, intra-ocular foreign body (IOFB), and optic nerve trauma/avulsion. In penetrating trauma cases, the presence of IOFB ranged from as low as 6.4% to as high as 21.7%.[6],[29],[30],[33] The local epidemiology seems to be responsible for variation in the type of trauma causing vitreous hemorrhage. Rishi et al.[6] observed that in the Indian sub-continent, children tend to be involved more in popular trauma-prone outdoor sports such as cricket, gilli-danda (a local sport in which a spindle-shaped smaller stick and a larger wooden stick are used)and bow-arrow. This could also be responsible for males occupying a major proportion of traumatic vitreous hemorrhage cases as mostly male children engage in most of these sports activities. Rishi et al.[6] stated that the differences in the nature of trauma accounting for vitreous hemorrhage could be attributed to regional preference to outdoor versus indoor sports and socio-economic factors including the practice of child labor in the developing world. Elsewhere, Rostomian et al.[35] reported that indoor items such knives, toys, etc. were common causes for open globe injuries in children, making it evident that the etiological agents, the local circumstances in which trauma occurs varies between populations and hence, the associated findings vary.

Unilateral spontaneous pediatric vitreous hemorrhage

Spontaneous vitreous hemorrhage can be purely due to underlying ocular causes or due to a sinister systemic pathology. The list of disorders/conditions described in literature that lead to spontaneous vitreous hemorrhage in children is long and exhaustive. Excluding isolated case reports, a comparison of five studies under the purview of this review is illustrated in [Table 7]. While Eales' disease and vasculitis-related vitreous hemorrhage were common in Indian studies,[6],[31],[33] regressed retinopathy of prematurity (ROP) and tumors dominated the share of spontaneous vitreous hemorrhage elsewhere.[29],[30] Sudhalkar et al.[33] attribute this to the fact that immunologic reactions to infectious agents such as tuberculosis were more likely to occur in a developing country such as India. In an age-wise analysis attempted by Sudhalkar et al.,[33] vasculitic and hematological disorders were more likely to cause vitreous hemorrhage after infancy. In infants, idiopathic causes were more compared to children over 1 year of age.[14] This was also noted by Al Harkan et al.[30] This also corroborates with a previous study[28] which stated that in many cases, the exact cause was unknown in infants. Sudhalkar et al.[33] state that this has more to do with the practical difficulty in eliciting appropriate history and clinical signs and symptoms in young children. Pars planitis is a well-known cause of vitreous hemorrhage in children.[18] It was noted in numerous studies [6],[29],[30],[33] irrespective of the geographical regions where they were conducted. Sudhalkar et al.[35] in their study dealing exclusively with spontaneous vitreous hemorrhage, divided the study sample into three age groups: <1 year, >1–10 years, and <10 years. In the first group, tumors, familial exudative vitreoretinopathy, and idiopathic causes occupied a major share. Vasculitis and hematological disorders accounted for the majority of cases (70%, 77/110 eyes) in the other two groups. Thus, the etiology is extremely varied, even within different regions of a particular country, and further variation is possible as the children grow older. It is important to note that spontaneous vitreous hemorrhage in children is a condition with a diverse etiology that mandates a thorough systemic examination and investigations to reach an appropriate diagnosis. As Sudhalkar et al.[33] have mentioned, vitreous hemorrhage in such cases can be a pointer to malignancy, both ocular and systemic. From their observations, they formulated an algorithm to approach a child presenting with vitreous hemorrhage,[33] which could be quite helpful.{Table 7}

Bilateral vitreous hemorrhage

The proportion of patients presenting with bilateral vitreous hemorrhage and the most common cause differs between different studies. In the Western literature,[29] non-accidental trauma or the battered baby syndrome appears to be the most common cause. However, no cases of battered baby syndrome were recorded by any of the Indian studies [6],[31],[32] or by Al Harkan et al.[30] Shaken baby syndrome apparently occurs with less frequency in the developing countries.[36],[37] Al Harkan et al.[30] attributed this to the fact that their study was conducted in a specialized eye hospital where only patients with medically stable condition were referred. Within the same country too, the common causes of bilateral vitreous hemorrhage seem to vary among different regions. While Rishi et al.[6] noted all cases of bilateral vitreous hemorrhage to be spontaneous in their study, trauma due to firecracker injuries was the most common cause noted by Sudhalkar et al. in two separate studies.[31],[32] IOFB was reported in 16.6% of the bilateral cases by Sudhalkar et al.[31] Firecracker injuries have particular spike in incidence during the time of festivals such as Diwali in India, as noted by Sudhalkar et al.[31] Among spontaneous causes for bilateral vitreous hemorrhage, data available from the Indian sub-continent points to Eales' disease and vasculitic pathologies to be the most common cause. Etiologies such as firecracker injuries are modifiable factors through increased awareness programs at the target population. This calls for not only awareness and counseling at the health-care center level but also at the administrative level in the form of efficient legislative measures.

Management and outcomes

Globally, there is no specific consensus on the approach to and management of a pediatric patient with vitreous hemorrhage. Majority of the studies have divided management strategies into three categories: observation/medical therapy, non-incisional therapy (laser/cryotherapy), and surgery. A review into the existing literature is shown in [Table 8]. Half of the cases were observed by Spirn et al.[29] However, Rishi et al.[6] and AlHarkan et al.[30] reported surgical intervention in a majority of their cases. As far as bilateral vitreous hemorrhage is considered, 40.5% cases were managed by observation or topical/systemic medical therapy in the traumatic group. Surgical intervention was required in more than two-thirds of the bilateral traumatic group versus approximately one-fifth of the bilateral spontaneous cases. The proportion of traumatic cases that required surgical intervention was in the range of 55–90% cases, while that for blunt trauma cases was in the range of 25–96%.[6],[29],[30],[32] In certain studies, the proportion of penetrating trauma cases requiring surgical attention was higher,[6],[29],[30] while in others blunt trauma cases required surgery in a major proportion compared to penetrating trauma.[32] Therefore, a thorough primary evaluation is necessary to determine whether to plan observation, medical management, or proceed for surgery depending on the etiology, mechanism of trauma, age of the patient and associated risks for surgery including those of general anesthesia.{Table 8}

Factors affecting visual outcome

No uniformity was observed in the factors that positively or negatively affected visual outcome among available studies. Spirn et al.[29] observed that etiology of the vitreous hemorrhage had a major effect on the final visual outcome. While cases such as ROP fared well, those due to penetrating trauma had poor visual outcome. However, the limitation in their study was that visual acuity was not recorded in a standardized manner. Approximately, 39% of the patients had severe visual loss, defined by the authors as one of the following: final visual acuity of less than 20/800 (1.6 logMAR), no fixation behavior and no blink to light. Penetrating trauma was the most common cause responsible for severe visual loss, amounting to 40% of the cases. They also reported that younger children had a higher proportion of severe visual loss than elder ones, although this difference was not statistically significant. Rishi et al.[6] defined severe visual loss as visual acuity of ≤ 3/60 (1.3 logMAR) and found that risk factors for severe visual loss were male sex, age greater than 8 years, unilateral presentation and surgical treatment. They also observed that eyes sustaining blunt trauma had a higher risk of severe visual loss among children with vitreous hemorrhage than eyes sustaining penetrating trauma. Of the 223 (of 261) eyes in which visual outcome could be determined, improvement was noted in 118 (52.9%), stabilization was achieved in 92 (41.3%), and the remaining 13 (5.8%) worsened despite treatment. Al Harkan et al.[30] analyzed the outcomes of traumatic and non-traumatic vitreous hemorrhage separately. They observed that in traumatic cases, the density of vitreous hemorrhage had a significant impact on the eventual visual outcome. However, performance of surgery had no significant correlation with the prediction of visual outcome. Visual acuity of ≥ 20/200 (1.0 logMAR) at presentation had a favorable visual outcome. However, poor initial visual acuity (≤ hand movements), age ≤ 3 years, and severe vitreous hemorrhage at presentation were significantly associated with a poor visual outcome. Contrary to traumatic cases, they observed that density of vitreous hemorrhage at presentation had no significant bearing on the eventual visual outcome in non-traumatic cases. In addition, retinal (detached versus attached) and lens (clear lens/pseudophakia versus cataract/aphakia) status at presentation had a correlation with the final visual outcome. Spontaneous causes, younger age, presence of retinal detachment, cataract, and aphakia at presentation had poor visual outcome. Al Harkan also observed that the rates of surgery (55.6% versus 28.6%) and enucleation/evisceration (11.9% versus 1.6%) were significantly higher in the non-traumatic group. One peculiar finding was that the incidence of retinal detachment at last follow-up (42.9% versus 2.1%) was significantly higher in the non-traumatic cases. They explained this by the fact that many of the non-traumatic causes such as hereditary diseases or retinoblastoma tend to occur in younger age group, which are usually associated with retinal detachment, while trauma tends to occur in a physically active child (sic). In studies where comparison of mean final best corrected visual acuity (BCVA) to the mean BCVA at presentation was available, all of them reported improvement in the mean final BCVA,[29],[31],[32],[33] some being statistically significant.[31],[32],[33] [Table 4]. The mean BCVA at final follow-up for closed globe injuries/non-penetrating trauma was significantly better in certain studies,[31],[33] while no such significant difference was noted in others.[30]

 Conclusion



Vitreous hemorrhage in the pediatric age group poses unique challenges for the ophthalmologist. These challenges range all along the management course right from lack of a reliable history, challenges in objective evaluation, to treatment approaches and post-operative rehabilitation. As evident from this review, the etiology for both traumatic and spontaneous cases can be highly varied. One should not hesitate in subjecting the child to examination under general anesthesia if it is essential. However, the risks of general anesthesia and systemic co-morbidities need to be duly considered during such an examination and also during surgical intervention. There is no straightforward consensus regarding the timing of intervention and the mode of management. One needs to decide the same from experience, available evidence, and tailor it according the individual case, considering the age of the patient, laterality of the hemorrhage, risk of amblyopia, and compliance of the patient with treatment protocol and follow-up. Certain cases may need a multi-pronged approach involving not only the ophthalmologist but also the pediatrician and the anesthetist. However, available literature does suggest that improvement in visual acuity is possible with the optimal management strategy. All studies available in the literature are from retrospective review of institutional records and suffer from limitations such as non-standardized inclusion and exclusion criteria and visual acuity evaluation. This calls for large scale prospective studies to be undertaken, as vitreous hemorrhage in children can be quite debilitating in not only visual but also in psychosocial development of the patient.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

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