**17. Retinal detachment in children**

Retinal detachment is not common in infants and children. While the incidence of retinal detachment is 12 in 100000 in all age groups, only between 1.7% and 5.7% are diagnosed during childhood. The association of retinal detachment with complex intraocular pathologies in young children often presents a challenge to treatment.

There are 3 types of retinal detachment: Rhegmatogenous retinal detachment develops when there is a hole or tear in the retina, which transmits intraocular fluid underneath the retina and subsequent separation of the retina from the underlying pigment epithelium occurs. The second most common form is tractional, where the retina is pulled away from the underlying tissues. Exudative detachment develops when subretinal fluid accumulates under the retina such as in Coat's disease or vasoproliferative disorders (Yokoyama et al., 2004).

#### **17.1 Clinic and diagnosis**

Symptoms of retinal detachment are sudden onset of floaters, light flashes, appearance of black veil and loss of visual acuity. It is important to recognize that most of the small children cannot express loss of visual acuity and often vision may already be low due to concomitant ocular pathologies. Therefore the delay in diagnosis is more common in the pediatric retinal detachments compared to that in the adult retinal detachments. In a significant proportion of the patients, the diagnosis is made by chance during a routine eye examination or by noticing leukocoria. It is important to realize that retinal detachment in children may occur in both eyes frequently.

#### **17.2 Etiology**

Trauma has been reported to be the cause in 27–51% of cases of childhood retinal detachment. Ocular trauma is an important cause of retinal abnormalities in children and it is more common in boys. Retinal dialyses may be found in the superonasal or inferotemporal quadrant. Traumatic retinal detachment is seen most commonly in older children and is usually caused by blunt trauma (Sarrazin et al., 2004). The second frequent etiological factor is high myopia in children and if it is congenital may indicate an underlying abnormality. Retinal detachment may also result from intraocular infection or inflammation. The existence of systemic or hereditary diseases such as Trisomy 13 (Patau syndrome), Walker Warburg syndrome, Meckel syndrome, Norrie disease and incontinentia pigmenti should be investigated in retinal detachments of early childhood (0-1 year). In such cases, central nervous system imaging and detailed neurological examination should be requested. Also it is important to rule out retinoblastoma. Some of these children do not live very long due to these systemic or hereditary diseases. Retinal detachment in infants and children should lead the physician to suspect a systemic disease, a syndrome, trauma, or a tumor in the eye. That should be ruled out with ultrasound or CT scan. Other causes of congenital or infantile retinal detachment should also be considered, including Stickler's syndrome, retinopathy of prematurity, persistent hyperplastic primary vitreous (PHPV), also called persistent fetal vasculature (PFV), may also present with a retinal detachment at birth; however, the associated microphthalmos and cataract often makes the diagnosis easier. Stickler's syndrome combines ocular, orthopedic and midfacial anomalies in an autosomal dominant inheritance pattern. Ocular abnormalities include high myopia, empty vitreous with membranes and bands.

#### **17.3 Management**

22 Complementary Pediatrics

necessary. Topical steroids are the mainstay treatment for anterior uveitis, while systemic steroids are indicated in most cases of intermediate and posterior uveitis. If the disease is unresponsive to systemic steroids and/or the patient cannot tolerate systemic steroids, other immunosuppressive agents including azathioprine, methotrexate and ciclosporin may be

Retinal detachment is not common in infants and children. While the incidence of retinal detachment is 12 in 100000 in all age groups, only between 1.7% and 5.7% are diagnosed during childhood. The association of retinal detachment with complex intraocular

There are 3 types of retinal detachment: Rhegmatogenous retinal detachment develops when there is a hole or tear in the retina, which transmits intraocular fluid underneath the retina and subsequent separation of the retina from the underlying pigment epithelium occurs. The second most common form is tractional, where the retina is pulled away from the underlying tissues. Exudative detachment develops when subretinal fluid accumulates under the retina such as in Coat's disease or vasoproliferative disorders (Yokoyama et al.,

Symptoms of retinal detachment are sudden onset of floaters, light flashes, appearance of black veil and loss of visual acuity. It is important to recognize that most of the small children cannot express loss of visual acuity and often vision may already be low due to concomitant ocular pathologies. Therefore the delay in diagnosis is more common in the pediatric retinal detachments compared to that in the adult retinal detachments. In a significant proportion of the patients, the diagnosis is made by chance during a routine eye examination or by noticing leukocoria. It is important to realize that retinal detachment in

Trauma has been reported to be the cause in 27–51% of cases of childhood retinal detachment. Ocular trauma is an important cause of retinal abnormalities in children and it is more common in boys. Retinal dialyses may be found in the superonasal or inferotemporal quadrant. Traumatic retinal detachment is seen most commonly in older children and is usually caused by blunt trauma (Sarrazin et al., 2004). The second frequent etiological factor is high myopia in children and if it is congenital may indicate an underlying abnormality. Retinal detachment may also result from intraocular infection or inflammation. The existence of systemic or hereditary diseases such as Trisomy 13 (Patau syndrome), Walker Warburg syndrome, Meckel syndrome, Norrie disease and incontinentia pigmenti should be investigated in retinal detachments of early childhood (0-1 year). In such cases, central nervous system imaging and detailed neurological examination should be requested. Also it is important to rule out retinoblastoma. Some of these children do not live very long due to these systemic or hereditary diseases. Retinal detachment in infants and

pathologies in young children often presents a challenge to treatment.

used (Jancevski ,2010).

2004).

**17.2 Etiology** 

**17.1 Clinic and diagnosis** 

children may occur in both eyes frequently.

**17. Retinal detachment in children** 

Prediction, prophylaxis and timely surgical treatment of retinal detachment may prevent visual loss. Retinal detachment in childhood and adolescence is different from adult cases due to higher rate of complicating predisposing factors such as trauma and high myopia and also due to the delay in the diagnosis that is mostly made after macular involvement. By appropriate surgical treatment the anatomic success rate may be as high as adult cases, however lower functional results are usually achieved because of higher rate of macular involvement (Butler et al., 2001; Topbas et al., 2003). It is important for the pediatrician to refer the children with signs and symptoms of retinal detachment, children with systemic disorders associated with retinal detachment and also children with ocular trauma history to an ophthalmologist.
