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Pierson syndrome is a rare pediatric disease characterized most commonly by congenital nephrotic syndrome and ocular and neurologic abnormalities. On the other hand, it is known that the severity of the clinical spectrum can be variable. Herein, 1.5-year old child with severe ocular findings and proteinuria who was genetically diagnosed as Pierson syndrome is presented. During the follow-up for a duration of 8 years, it is noteworthy that the patient had no renal involvement other than nephrotic range proteinuria. As our case demonstrates the mild renal variant of Pierson syndrome, the presence of this rare type of presentation should always be kept in mind.

OBJECTIVE: To investigate the e?ect of strabismus surgery on subfoveal choroidal thickness (CT). MATERIAL AND METHODS: Measurements of subfoveal CT in patients who underwent strabismus surgery were obtained by enhanced depth imaging optic coherence tomography (EDI-OCT). Patients were grouped as those who had had one rectus muscle surgery (superior; inferior; medial or lateral; or resection or recession) (Group 1) and those who had had inferior oblique muscle surgery (myectomy) (Group 2). The CT in patients was measured preoperatively and at one day and one week postoperatively. RESULTS: Included in the study were 41 eyes of 41 patients. It was found that the preoperative, first day postoperative, and first week postoperative mean subfoveal CTs in Group 1 were 390.57±89.54 µm, 415.23±91.77 µm, and 394.8±90.97 µm, respectively. The preoperative and first-day postoperative comparison revealed an increase in CT (p=0.002). However, when the first day postoperative and first-week postoperative measurements were compared, a statistically significant decrease was found (p=0.007). In Group 2, the preoperative, first day postoperative, and first week postoperative mean subfoveal CTs were 347.75±83.58 µm, 332.75±84.95 µm, and 332±79.15 µm, respectively. The preoperative and first-day postoperative comparison showed a decrease in CT that was not statistically significant (p=0.532). CONCLUSIONS: Rectus muscle surgery causes an increase in subfoveal CT, which is possibly caused by the altered choroidal microcirculation mechanical traction during surgery and by postoperative in?ammation. We did not see a similar increase in Group 2; this may be due to the relaxation of the inferior oblique muscle’s traction in the macular region and a relatively low level of postoperative in?ammation. AMAÇ: Şaşılık cerrahisinin subfoveal koroid kalınlığı üzerine etkilerini araştırmak. GEREÇ VE YÖNTEM: Şaşılık cerrahisi geçiren hastaların subfoveal koroid kalınlık (KK) ölçümleri optik koherens tomografi (EDI-OCT) ile elde edildi. Hastalar 1 rektus kas cerrahisi geçirenler (superior; inferior; medial ya da lateral; ve rezeksiyon veya geriletme) (Grup 1) ve inferior oblik kas cerrahisi geçirenler (miyektomi) (Grup 2) olarak sını?andı. Hastaların preoperatif, postoperatif ilk gün ve postoperatif 1. hafta KK ölçümleri kaydedildi. BULGULAR: Çalışmaya 41 hastanın 41 gözü dahil edildi. Grup 1’deki preoperatif, postoperatif birinci gün ve birinci hafta ortalama subfoveal KK’ların sırasıyla 390.57 ± 89.54 µm, 415.23 ± 91.77 µm ve 394.8 ± 90.97 µm olduğu tespit edildi. Grup 1’de preoperatif ve postoperatif ilk gün karşılaştırıldığında KK’da bir artış olduğu izlendi (p = 0.002). Ayrıca, grup 1’de postoperatif ilk gün ve 1.hafta karşılaştırıldığında, istatistiksel olarak anlamlı bir düşüş bulundu (p = 0.007). Grup 2’de preoperatif, postoperatif ilk gün ve postoperatif 1. hafta ortalama subfoveal KK’ları sırasıyla 347.75 ± 83.58 µm, 332.75 ± 84.95 µm ve 332 ± 79.15 µm idi. Preoperatif ve postoperatif ilk gün KK’ları karşılaştırıldığında istatistiksel olarak anlamlı olmayan bir düşüş gösterdi (p = 0.532). SONUÇ: Rektus kası cerrahisi subfoveal KK’da artışa neden olmaktadır, bunun cerrahi sırasındaki mekanik traksiyon, değişen koroid mikrosirkülasyon ve postoperatif in?amasyona bağlı olduğunu düşünmekteyiz. Grup 2’de benzer bir artış görmememizin nedeni; inferior oblik kasın maküler bölgedeki traksiyonunun gevşemesi ve nispeten düşük bir postoperatif in?amasyon nedeniyle olabilir.

Anterior segment optical coherence tomography (AS-OCT) is a valuable tool in corneal transplantation surgery. In deep anterior lamellar keratoplasty (DALK), AS-OCT gives valuable information on preoperative decision process. AS-OCT image of the recipient cornea helps the surgeon to decide whether to proceed with a big bubble DALK or a manual dissection. Preoperative AS-OCT imaging of recipient stroma can also predict Descemet membrane perforation during big bubble induction. Intraoperative AS-OCT assures reaching the proper depth in order, and postoperatively, a surgeon can objectively assess the Descemet membrane and donor association and define complications such as a double anterior chamber. Before deciding for a Descemet membrane endothelial keratoplasty (DMEK), preoperative stromal scars can be identified with an AS-OCT and might affect the decision of the surgical procedure (DMEK or penetrating keratoplasty). After DMEK, graft detachment can be visualized by AS-OCT and treated accordingly.