Título / Title

SPONTANEOUS RETINAL REATTACHMENT AFTER A 300-DEGREE GIANT RETINAL TEAR IN A CHILD WITH STICKLER SYNDROME.

Introdução / Purpose

Stickler Syndrome (SS) is a multisystem connective tissue disorder. Autosomal dominant is the most common form. Craniofacial, skeletal/joint, and inner ear structures are often involved.
SS confers a risk for severe myopia, retinal detachment and blindness.Depending on the genetic group 50% to 70% of patients diagnosed with SS had already suffered a retinal detachment and almost half of them had bilateral involvement.

Material e Método / Methods

This is a case report of a bilateral retinal detachment in a SS.

Resultados / Results

A 7-month-old boy came referred to our service with the suspicion of retinal detachment. The parents' only complaint was an eye deviation noticed approximately one month ago.
Examination under sedation was performed and found upper and lower retinal detachment in the right eye and total detachment in the left eye. A photocoagulation was performed in the right eye on the same day.
After 10 days, vitreous hemorrhage was seen in the right eye, without retinal tear. Posterior vitrectomy, cryotherapy in the four quadrants, endolaser and scleral buckling with a 240 encircling band was performed.
After 50 days, the right eye had clear vitreous, attached retina and 360° laser marks. The left eye had an attached posterior pole, and only peripheral detachment. It was opted to perform photocoagulation surrounding the attached area and to schedule surgery in a week.

Discussão e Conclusões / Conclusion

A high index of suspicion of SS allows for early diagnosis and better clinical outcomes. Once retinal alterations are diagnosed, prophylactic intervention should be done. In this case, if photocoagulation in the right eye had not been performed, it would have rapidly progressed to a total retinal detachment since the vitreous hemorrhage occurred a few days later.
This case report is surprising because the retinal detachment in the left eye resolved spontaneously. As far as we know, this has never been published before, and we believe that the total detachment of the vitreous may have contributed to the retina coming back into place.

Palavras Chave

Stickler Syndrome, retinal detachment, retinal detachment in children, spontaneous resolution of retinal detachment

Area

CLINICAL RETINA