TENT-SHAPED RETINAL DETACHMENTS IN RETINOPATHY OF PREMATURITY MICHAEL J. SHAPIRO, MD, JOSHUA ALPERT, MD, RAHUL T. PANDIT, MD Background: Most retinal detachments associated with retinopathy of prematurity (ROP) are radial, segmental, or circumferential with cicatricial extraretinal fibrovascular proliferation (EFP) at the apex of the detachment. This report describes peculiar tent- shaped retinal detachments that developed among eyes with ROP. Methods: An observational case series consisting of 9 patients and 13 eyes with tent-shaped retinal detachments. Their morphology, clinical baseline, surgical course, and final retinal status were extracted from medical records. Results: Eight had simple tent-shaped retinal detachments, three had a double tent- shaped retinal detachment, one had a chevron-based retinal detachment, and one had a star-shaped retinal detachment. Each case had a disk based stalk that extended to the apex of the traction retinal detachment and continued anteriorly; 12 stalks inserted in the retrolental space and 1 terminated in the mid vitreous. Six eyes were stage 4A, two eyes were stage 4B, and five eyes were stage 5. Vitrectomy surgery was performed on 11 eyes. Surgery resulted in retinal attachment in nine eyes, and two retinas remained detached. Conclusion: Tent-shaped retinal detachments are seen in patients with ROP. A stalk should be sought in evaluation of these eyes. Vitreous surgery focused on relieving this traction is often successful. RETINA 26:S32–S37, 2006 n his description of the pathogenesis of late stages of been frequently noted.3–6 The dissection of the stalk Iretinopathy of prematurity (ROP), Machemer pre- was a well-documented step in membranectomy for sented observations about the development of retinal ROP. Eller et al. studied the association of the persis- detachments in ROP.1 He proposed a mechanical tent hyaloid artery with ROP and suggested that it was schema that showed that contraction of stage 3 ROP a likely part of the disturbance of vasculogenesis and could account for the progression to segmental, cir- angiogenesis that describe the advance stages of cumferential, and radial retinal detachments. He con- ROP.7 They also speculated that the hyaloid system cluded with a general statement that “All retinal could provide a scaffold for fibrovascular prolifera- changes seen in ROP can be explained by proliferation tion. The stalk also had practical implications since of tissue originating in the shunt area.” Extending when using the open sky technique this stalk may these observations, the international classification of increase the intraoperative hemorrhage,4 and using ROP further described the retinal detachment config- closed vitrectomy, aggressive surgery on the stalk urations.2 may lead to posterior retinal breaks.6 Recently, the The presence of a stalk originating on the optic disk stalk was identified as one of the many vectors of nerve in retinal detachments associated with ROP has traction involved in development of retinal detach- From the University of Illinois at Chicago, UIC Eye Center. ment in ROP.8 Although frequently observed, the The authors have no proprietary interests in the material de- identification of a disk stalk as a sufficient cause of scribed. Reprint requests: Michael J. Shapiro, MD, UIC Eye Center, retinal detachment separate from the extraretinal fi- 1905 W. Taylor, Chicago, IL 60612; e-mail: [email protected] brovascular proliferation (EFP) was often impossible S32 TENT-SHAPED RETINAL DETACHMENTS IN ROP ● SHAPIRO ET AL S33 Table 1. Case Summary Estimated Birthweight Gestational Detachment Structural Eye Patient (g) Age (wk) Location Eye Zone Treatment Morphology Stage Vitrectomy Outcome 1 1 692 25 Inborn L 1 Laser Tent 4B LSV Attachment 2 2 550 24 Inborn R 1 Laser Tent 4A LSV Attachment 3 Inborn L 1 Laser Tent 4A LSV Attachment 4 3 560 24 Inborn L 2 Laser Tent 4B LV Attachment 5 4 646 26 Outborn R 2 Laser Tent 4A None Attachment 6 L 2 Laser Tent 5 LV Attachment 7 5 620 23 Outborn L 2 None Star shaped 5 LV Attachment 8 6 1220 25 Outborn R 1 Laser Tent 5 LV Detachment 9 L 1 Laser Double tent 4A LSV Attachment 10 7 553 23 Outborn R 1 Laser Double tent 5 LSV Attachment 11 L 1 Laser Double tent 5 LSV Phthisis 12 8 655 26 Outborn R 1 Laser Tent 4A LSV Attachment 13 9 1800 NA Outborn L NA None Chevron 4A None Unchanged LSV ϭ lens sparing vitrectomy; LV ϭ lensectomy vitrectomy. because the vitreoretinal relationships were dominated the standard. Preoperative evaluation focused special by the EFP. attention on the retinal examination. The form of the In this article, we describe infants with ROP that retinal detachment was studied. The EFP was consid- presented with tent-shaped retinal detachments rather ered for extent, location, vascularity, and its position than radial, segmental, or circumferential folds. This relative to both the retina and the lens. Postoperative tent-shaped morphology is associated with persistent follow-up ranged from 6 months to 6 years and in- fetal vasculature (PFV),9 and very different from the cluded patient history, examination with indirect oph- retinal detachments generally seen in ROP. Careful thalmoscope, and slit lamp examination (when age inspection revealed that these retinal detachments appropriate). were not a result of the contraction of the extraretinal fibrovascular proliferation, but rather the contraction Results of a hyperplastic disk stalk probably related to the Tent-shaped retinal detachments were found in 13 hyaloid artery. Identification of these unexpected ret- eyes of 9 patients with ROP (Table 1). All patients inal detachment configurations is helpful for diagnosis were born prematurely and also had severe ROP in the by ophthalmologists who examine or treat ROP. contralateral eye. One was first examined as an adult; of the remainder, five infants were seen during the Materials and Methods active phase and three infants were referred during the Tent-shaped detachments were defined as a traction cicatricial phase of ROP. The birthweights ranged retinal detachment with five criteria: one, a well de- from 550 g to 1800 g. Among the pediatric cases fined small apex and relatively broad base; two, retinal (excluding Patient 9) the average birthweight was detachment apex well within the vascularized retina; 611 g. The infants were born at estimated gestational three, tubular fibrous stalk connected to the retinal ages that ranged from 23 to 26 weeks with a mean of apex; four, stalk emanating from the optic nerve disk; 24.5 weeks. Three of the infants were inborn. Five and five, traction from the stalk directed into central were referred for treatment. The one adult, ex-prema- vitreous. Records of patients with traction retinal de- ture neonate, was referred for long-term observation. tachments from ROP seen in a pediatric retina practice The retinal detachments evolved under supervision were reviewed. Cases with a documented tent-shaped among the inborn patients. All but two eyes among the retinal detachment were selected. Eyes with a radial or infants reached threshold before development of reti- circumferential fold or detachment as the predominant nal detachment. Eight were in zone 1 and four were in morphology were excluded. All eyes that had an optic zone 2. All threshold eyes were treated with laser nerve disk stalk discovered during surgical dissection photocoagulation. were also excluded. There were four configurations of retinal detach- The baseline characteristics were recorded, includ- ments. Eight eyes showed simple tent-shaped retinal ing the estimated gestational age and birthweight. The detachment (Figure 1). In these eight eyes, fibrous history was reviewed using the postmenstrual age as elements originated on the optic disk and connected S34 RETINA, THE JOURNAL OF RETINAL AND VITREOUS DISEASES ● VOL 26 ● NO 7 ● SUPPLEMENT 2006 Fig. 3. Left, the ultrasound of a tent-shaped detachment with a stalk seen extending from nasal to the disk to the retrolental space. Right, an ultrasound corresponding to Figure 2 shows a hyperplastic hyaloid artery extending into the central vitreous. Fig. 1. A simple tent-shaped retinal detachment accompanied by a mild circumferential fold from cicatricial stage 3 retinopathy of pre- maturity. The stalk connects from the disk to the apex of the retinal detachment within the vascularized retina and then extends into the and 9 the EFP involuted and exerted no traction on the vitreous and retrolental space. retina. Since the media were clear, ultrasounds were only occasionally performed. In one eye the ultra- along the retina surface to the apex of the retinal sound showed the stalk extending from adjacent to the detachment within the vascularized retina, and in- nerve to the retrolental space and in the second eye it serted anteriorly in the central vitreous, anterior vitre- extended from the disk to the central vitreous (Figure ous, or posterior lens (Figure 2). In eight eyes there 3). Connection to the condensed anterior vitreous was were accompanying peripheral segmental folds due to noted in five eyes, and in one a connection to vitreous contraction of persistent stage 3 ROP. In Cases 1, 7, 8, that bridged across a circumferential fold was re- corded. Among all the eyes with tent-shaped retinal detachments, the area of retinal detachment was lim- ited by the disk and the laser induced chorioretinal atrophy. Some vascular and retinal dragging was al- ways present among cases with tent-shaped retinal detachment. In five eyes more complicated retinal detachment configurations developed. One eye presented with a tent-shaped detachment with a chevron-shaped base (Figures 4 and 5). This shape reflected the straighten- ing of the arcade blood vessels proximal and distal to the apex forming a chevron as a result of the anterior traction at the apex. Three eyes presented with a double tent with the stalk connected to two retinal detachment apices, one nasal and one temporal.