Current Management of Late Posttraumatic Enophthalmos.

LEARNING OBJECTIVES: After studying this article, the participant should be able to: 1. Describe the orbital anatomy and completely understand the important components relevant to surgical correction of enophthalmos, including oculo-orbito relations. 2. Understand the pathophysiology and predictive factors for posttraumatic enophthalmos and identify the challenges associated with correction of enophthalmos in the late setting. 3. Develop a surgical plan for late enophthalmos repair and understand the value and utility of osteotomies, intraoperative navigation, and patient-specific implants. 4. Discuss the expected outcomes, possible complications, and adjunctive surgery as related to late enophthalmos repair. SUMMARY: This article addresses the current management of late posttraumatic enophthalmos. In this article, the authors describe surgically relevant orbital anatomy and oculo-orbital relations, the pathophysiology of enophthalmos, clinical and radiologic findings, decision-making in management, and surgical treatment. The authors attempt to cover some of the main challenges and recent advances in the management of late posttraumatic enophthalmos, including intraoperative navigation and patient-specific implants.

Functional Outcomes of Late Posttraumatic Enophthalmos Correction.

BACKGROUND: Posttraumatic enophthalmos has debilitating functional consequences, resulting in restriction of ocular motility and diplopia. Surgical correction aims to restore the globe position and ultimately improve function. This study evaluates the functional outcome of posttraumatic enophthalmos repair. METHODS: Patients included in this study had posttraumatic enophthalmos and diplopia requiring enophthalmos repair. Diplopia was graded from 0 (no diplopia) to 4 (constant diplopia) based on the Functional Diplopia Grading Scale. Limitations of eye movements were recorded in the vertical, horizontal, and torsional directions. Data were gathered prospectively at preoperative assessment, postenophthalmos repair, and final follow-up. RESULTS: Between 2002 and 2014, 41 patients fulfilled inclusion criteria. Substantial functional improvement, defined as a decrease of greater than or equal to 1 grade of diplopia, was achieved in 65.9 percent of patients (27 of 41) after all surgical interventions. Patients with residual diplopia (34 of 41) after enophthalmos surgery were managed with secondary strabismus surgery (10 of 34) and/or prism glasses (four of 34). After all interventions, vertical restrictions improved from -1.95 ± 1.13 to -1.06 ± 0.98. Horizontal restrictions improved from -0.88 ± 0.62 to 0.59 ± 0.6. Adequate clinical correction of enophthalmos to within 2 mm of the contralateral globe was achieved in 37 of 41. CONCLUSIONS: This is the largest case series evaluating functional outcomes of patients undergoing posttraumatic delayed enophthalmos repair. A multidisciplinary care approach resulted in improved globe position and eye movement, and improvement of diplopia. Further studies with larger sample sizes are needed to better understand and treat this important and challenging problem. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

The short adjustable suture.

OBJECTIVE: To describe a new, adjustable suture technique for strabismus surgery that is safe and effective and allows for adjustment during the postoperative week only when required. DESIGN: Retrospective review. PARTICIPANTS: A total of 304 patients, of which 149 were male and 155 female, with an age range from 4 to 89 years and a median age of 42 years. METHODS: All patients treated with the short adjustable suture technique between September 2007 and April 2009 were reviewed retrospectively. Details of cause, complexity and reoperation, operative success, requirement for adjustment, and success of adjustment were collected. Success was defined as horizontal deviation < or = 10 prism diopters (PD) and vertical deviation < or = 6 PD. RESULTS: Overall, 84% of horizontal deviations and 74% of vertical deviations were treated successfully with 1 operation. Twenty-one adjustments were performed. Complications included 1 slipped slip knot and 6 conjunctival or Tenon cysts. CONCLUSIONS: The short adjustable suture is a safe and effective variation of the standard slip-knot adjustable suture technique. It allows for adjustment up to 6 days postoperatively with minimal patient discomfort. When adjustment is not indicated, the suture can be left in place to absorb.

Retinoblastoma CSF metastasis cured by multimodality chemotherapy without radiation.

OBJECTIVE: Cerebrospinal fluid (CSF) metastasis is the most difficult type of retinoblastoma metastasis to cure, even with bone marrow transplant. Most metastatic retinoblastoma cells express P-glycoprotein causing multidrug resistance (MDR). P-glycoprotein-rich blood vessels form blood-brain and blood-eye barriers, inhibit drug entry into central nervous system (CNS) and eyes. High-dose craniospinal radiation is too morbid for treatment of young children. To cure CSF metastasis without radiation, we designed an intensive multimodality chemotherapy regimen. METHOD: After left eye enucleation, a 4-month-old boy with bilateral International Intraocular Retinoblastoma Classification Group E eyes and CSF metastasis was treated with 7-cycle high-dose carboplatin and etoposide, standard-dose vincristine, and high-dose/short-infusion cyclosporine to inhibit P-glycoprotein. Intraventricular drugs, non-substrate of P-glycoprotein (cytarabine), or less susceptible to MDR (topotecan), contributed to treatment of the metastasis. On achieving complete response, he was consolidated with supralethal-dosage carboplatin, etoposide, and cyclophosphamide, and his bone marrow rescued with autologous cord blood stem cells. RESULTS: Following 1-cycle systemic chemotherapy and 2-dose intraventricular chemotherapy, the CSF metastasis cleared. The right eye tumor regressed completely. The patient remains in remission 8.3 years after diagnosis and 7.8 years post-transplant. CONCLUSION: Intensive multimodality chemotherapy can cure CSF metastasis in retinoblastoma without incurring extreme morbidity from craniospinal radiation.

Toxocariasis mimicking liver, lung, and spinal cord metastases from retinoblastoma.

A boy with bilateral retinoblastoma underwent metastatic surveillance for increased risk of systemic and central nervous system metastasis because of the extensive choroid and optic nerve invasion in his enucleated eye. Two years after finishing chemotherapy, surveillance MRI showed multiple new liver, lung and spinal cord lesions. High Toxocara antibody titers, eosinophilia, and elevated IgE levels supported a diagnosis of toxocariasis, rather than retinoblastoma metastasis. This is the first report of early, asymptomatic spinal cord toxocariasis diagnosed incidentally through metastatic surveillance.

Trilateral retinoblastoma with pituitary-hypothalamic dysfunction.

Trilateral retinoblastoma is characterized by retinal tumors in one or both eyes, as well as tumors of the pineal gland or parasellar region of the brain. Here we describe a 4-month-old girl, presenting with pituitary dysfunction, hypothalamic overgrowth syndrome and central blindness, in addition to suprasellar and bilateral retinal tumors. Biopsy of the suprasellar tumor confirmed the diagnosis of trilateral retinoblastoma. After biopsy, cerebrospinal fluid (CSF) metastasis was discovered. Overgrowth persisted, but blindness and pituitary dysfunction resolved when the suprasellar tumor and CSF metastasis responded to cyclosporine-modulated systemic chemotherapy with intraventricular chemotherapy, consolidated with marrow-ablative chemotherapy and stem cell rescue of the bone marrow. Twenty months after diagnosis and 12 months after transplant, an unusual pattern of tumor recurrence was observed along the catheter of the Ommaya reservoir used for delivering intraventricular chemotherapy, which was also at the site of the previous suprasellar needle biopsy. Salvage therapy consisted of resection, stereotactic radiation, and further systemic and intraventricular chemotherapy. At 25 months after diagnosis, the patient was developing well and seeing better. However, she died 32 months after diagnosis despite the salvage therapy. This case highlights the possibility of tumor dissemination after needle biopsy of a suprasellar tumor. Biopsy may be avoided if a characteristic clinicoradiological picture of trilateral retinoblastoma is recognized. We recommend that if a pineal or suprasellar tumor is observed in a child, the eyes should be examined for retinoblastoma, thereby avoiding biopsies of the intracranial tumor, which may track difficult-to-treat tumor cells through the brain, and disseminate tumor cells into the CSF.

Assessment of change of optic nerve head cupping in pediatric glaucoma using the RetCam 120.

PURPOSE: To ascertain the interobserver agreement among various specialists when analyzing images of the optic nerve head taken with the RetCam 120 (Clarity Medical Systems, Inc., Pleasanton, CA) for assessing the progression of optic disk cupping in pediatric glaucoma. METHODS: Five observers compared pairs of RetCam 120 two-dimensional photographs from 64 eyes to identify whether or not changes in the optic disk had occurred. Observers included a pediatric glaucoma specialist, pediatric neurophthalmologist, strabismologist, pediatric ophthalmic imaging specialist, and pediatric ophthalmology fellow. Each was masked to the patient identity and clinical course. For each patient, the observers reviewed the photographs taken prior to therapy and at a minimum of 6 months following initiation of therapy. Interobserver variability was calculated and analysis conducted to identify influential variables. RESULTS: Poor agreement was noted between the observers. No significant association was obtained between agreement level and variables affecting photographic quality: variability of contrast and illumination, exposure and magnification, image clarity, presence of fluorescein on the cornea, and pixelation of the image with poor resolution. Raters noted problems with these image variables in 75.6% of the observations. The Kappa statistic obtained was kappa = 0.29 with overall proportion of agreement of p = 0.69, suggesting a fair agreement but not a moderate agreement. CONCLUSIONS: Comparison of RetCam 120 pediatric optic nerve head photographs, when performed subjectively by observers with different perspectives, and in isolation from clinical information, may not be a reliable indicator of cupping change. In addition, variations in image contrast, luminance, color, and pixelation pose significant challenges to agreement between observers. Comparison of RetCam 120 images should not be considered the sole criterion for monitoring pediatric glaucoma.

Ocular motility changes after subtenon carboplatin chemotherapy for retinoblastoma.

BACKGROUND: Focal subtenon carboplatin injections have recently been used as a presumably toxicity-free adjunct to systemic chemotherapy for intraocular retinoblastoma. OBJECTIVE: To report our clinical experience with abnormal ocular motility in patients treated with subtenon carboplatin chemotherapy. METHODS: We noted abnormal ocular motility in 10 consecutive patients with retinoblastoma who had received subtenon carboplatin. During ocular manipulation under general anesthesia, we assessed their eyes by forced duction testing, comparing ocular motility after tumor control with ocular motility at diagnosis. Eyes subsequently enucleated because of treatment failure (n = 4) were examined histologically. RESULTS: Limitation of ocular motility was detected in all 12 eyes of 10 patients treated for intraocular retinoblastoma with 1 to 6 injections of subtenon carboplatin as part of multimodality therapy. Histopathological examination revealed many lipophages in the periorbital fat surrounding the optic nerve in 1 eye, indicative of phagocytosis of previously existing fat cells and suggesting prior fat necrosis. The enucleations were technically difficult and hazardous for globe rupture because of extensive orbital soft tissue adhesions. CONCLUSIONS: Subtenon carboplatin chemotherapy is associated with significant fibrosis of orbital soft tissues, leading to mechanical restriction of eye movements and making subsequent enucleation difficult. Subtenon carboplatin is not free of toxicity, and its use is best restricted to specific indications.

Unique insertional translocation in a childhood Wilms' tumor survivor detected when his daughter developed bilateral retinoblastoma.

Retinoblastoma and Wilms' tumor are rare childhood embryonic tumors associated with loss or inactivation of tumor suppressor genes, RB1 located within 13q14, and WT1 located within 11p13. Interchromosomal insertional translocations occur rarely, and such rearrangements within RB1 or WT1, even rarer. We report a unique family in which an insertional translocation of a chromosomal segment that included band 13q14 inserted into 11p13 caused childhood Wilms' tumor in the father, and whose child developed bilateral retinoblastoma. This is the first case of an insertional translocation that caused both tumors. This insertional translocation had significant consequences for genetic counseling and in utero diagnosis. The estimated risk for an offspring of this father to develop Wilms' tumor is up to 50%, to develop retinoblastoma up to 25%, to have neither tumor 25%, and to have both tumors 0%.

The effects of strabismus on motor development.

INTRODUCTION: Blindness in young children has been shown to lead to significant motor delay. Few studies to date have evaluated the effects of strabismus and its treatment on motor development. METHODS: We evaluated nine children prior to and post eye alignment via strabismus surgery to determine the effect of this treatment on motor development. Developmental milestones using the Bayley and modified Denver developmental scale were recorded prior to and post surgery. RESULTS: It was found that in this study sample there was developmental advancement within days of surgery in every case. CONCLUSION: We feel that eye alignment at an early age in strabismic children is an important factor contributing to age appropriate motor development as measured by developmental milestones. A method for a larger scale objective study to better define the effect of strabismus surgery on motor development is currently being planned.