Encephalocraniocutaneous Lipomatosis: A Case Report and Literature Review.

Encephalocraniocutaneous lipomatosis (ECCL) or Haberland syndrome is a neurocutaneous disorder of the skin, eye, and central nervous system. A three-month-old girl was referred to our center for further management of a large left eye corneal dermoid. At birth, a small lesion was noted. Magnetic resonance imaging (MRI) around the first week of life showed an extraocular dermoid cyst measuring 1 mm x 7 mm, dysplasia of the left greater wing of sphenoid, closed-lip schizencephaly of the left parietal lobe, and polymicrogyria. During examination under anesthesia at our center, we found that the corneal dermoid had grown in size to 17 mm x 16 mm, with posterior embryotoxon, a hazy cornea, and intraocular pressure of 26 mmHg. With the anterior segment dysgenesis and secondary glaucoma, we started Gutt Timo-Comod BD. Serial MRI imaging at four months of age revealed further enlargement of the dermoid, a new left retrobulbar mass, and multiple intracranial lipomas. A diagnosis of ECCL was made at this point based on the MRI and clinical findings. A multidisciplinary meeting was held among ophthalmology, neurosurgery, radiology, and otorhinolaryngology (ORL) teams, which concluded that surgical intervention such as tumor debulking might cause more harm than benefit. Hence, she was planned to undergo close monitoring with serial MRIs and only for surgical intervention, in the presence of airway compression or any neurological deficits. The ophthalmologist should be aware of the specific radiological and clinical findings in ECCL as management of the condition would be best through a multidisciplinary approach.

Recurrence of SRF Post Intravitreal Ranibizumab for Pediatric Choroidal Neovascularisation Secondary to Best Disease.

We present five cases of choroidal neovascularization secondary to pediatric Best disease which were treated with two different doses of intravitreal ranibizumab. Optical coherence tomography was used for monitoring of the cases. Three cases had subretinal fibrosis at presentation and two out of these 3 cases required repeat intravitreal ranibizumab at one year follow-up due to recurrence of subfoveal subretinal fluid.

Endogenous endophthalmitis: a 9-year retrospective study at a tertiary referral hospital in Malaysia.

BACKGROUND: The objective of this study was to determine the clinical presentation, systemic risk factors, source of infective microorganism, treatment outcomes, and prognostic indicators of endogenous endophthalmitis at a main tertiary referral hospital for uveitis in Malaysia. A retrospective review of medical records of 120 patients (143 eyes) with endogenous endophthalmitis over a period of 9 years between January 2007 and December 2015 was undertaken. RESULTS: Identifiable systemic risk factors were present in 79.2%, with the majority related to diabetes mellitus (60.0%). The most common source of bacteremia was urinary tract infection (17.5%). A positive culture from ocular fluid or other body fluids was obtained in 82 patients (68.9%), and the blood was the highest source among all culture-positive results (42.0%). Gram-negative organisms accounted 42 cases (50.6%) of which Klebsiella pneumonia was the most common organism isolated (32.5%). Sixty-nine eyes (48.6%) were managed medically, and 73 eyes (51.4%) underwent vitrectomy. Final visual acuity of counting fingers (CF) or better was achieved in 100 eyes (73.0%). Presenting visual acuity of CF or better was significantly associated with a better final acuity of CF or better (p = 0.001). CONCLUSIONS: The visual prognosis of endogenous endophthalmitis is often poor, leading to blindness. As expected, gram-negative organisms specifically Klebsiella pneumonia were the most common organisms isolated. Urinary tract infection was the main source of infection. Poor presenting visual acuity was significantly associated with grave visual outcomes. A high index of suspicion, early diagnosis, and treatment are crucial to salvage useful vision.