Kristin E. Hirabayashi

Imaging of eyelid lymphatic drainage

PURPOSE Traditional descriptions of lymphatic drainage show eyelids emptying into the submandibular or preauricular basin. However recent studies based on in vivo lymphatic imaging show a possible predilection for the preauricular basin. We describe lymphoscintigraphy and report findings in patients with eyelid malignancies undergoing sentinel lymph node biopsy (SLNB). METHODS Retrospective chart review of 15 consecutive patients at a single institution with eyelid carcinoma undergoing SLNB. The primary outcome measure was primary facial lymphatic drainage site from the eyelid as determined by lymphoscintigraphy. RESULTS The preauricular basin was the site of focal radioactive uptake in all 15 patients. The location of the primary tumor was as follows: medial upper eyelid (1), medial canthus (3), medial lower eyelid (3), lateral upper eyelid (3), and lateral lower eyelid (5). The types of tumor were: invasive squamous cell carcinoma (7), malignant melanoma (3), and sebaceous cell carcinoma (2), Merkel cell carcinoma (2), and conjunctival spindle cell carcinoma (1). CONCLUSIONS Lymphoscintigraphy is increasingly used in the context of SLNB for periocular malignancy. The recent literature suggests that the preauricular lymph node basin may be the primary site of eyelid lymphatic drainage and this is corroborated by our series. Further data will elucidate the biology of eyelid lymphatic channels in humans but the preauricular basin may be the prime lymphatic metastastic site in eyelid malignancies.

Reply re: "the efficacy of a midfacial seal drape in reducing oculofacial surgical field fire risk".

Purpose: To evaluate the efficacy of a midface seal drape in eliminating fire risk oxygen concentrations from nasal cannulated oxygen delivery compared with a standard open oculofacial surgical field. Methods: Controlled experiment using the SimMan patient simulator and an oxygen detector. Oxygen concentrations were measured at 9 facial surgical locations with nasal cannula flow rates of 2, 4, and 6 l/min of 100% FiO 2 in both the draped and undraped conditions. Results: The mean oxygen concentration in the oculofacial surgical field with the seal drape was 21.4% and 26.3% without (p = 0.0002; paired t test, 2-tailed). The draped condition provided safe oxygen concentration levels at all anatomical landmarks at all 3 flow rates, whereas the undraped condition was associated with suprathreshold oxygen concentration levels at 13 of 27 measurements. There was a direct correlation between oxygen flow rate and surgical field oxygen concentration in the undraped condition. Conclusions: A midfacial seal drape reduced oxygen concentrations from nasal cannula oxygen in the oculofacial surgical field and may reduce fire risk.

Invasive fungal sinusitis: treatment of the orbit

Purpose of review To summarize diagnostic techniques for invasive fungal rhinosinusitis and provide a review of treatment options once disease has spread to the orbit. Recent findings Improved imaging criteria, polymerase chain reaction and other serologic tests show promise in advancing our ability to accurately diagnose invasive fungal disease. Currently, there exists three treatment options for infected orbital tissue: exenteration, conservative debridement and transcutaneous retrobulbar injection of amphotericin B. Exenteration, the most frequently reported intervention, has not been proven to enhance survival. Conservative debridement and transcutaneous retrobulbar injection of amphotericin B are increasingly considered reasonable first-line options. Summary Although investigative tools are improving, invasive fungal rhinosinusitis can still pose a diagnostic challenge. No one treatment option for the orbit has been proven superior to another. Therefore, it is justified to initiate therapy by prioritizing less morbid procedures. If deterioration is continually noted, more invasive interventions can then be employed. The treatment algorithm established at our institution is provided.

Oral Pharmacotherapy for Benign Essential Blepharospasm

Benign essential blepharospasm (BEB) is a focal dystonia characterized by bilateral involuntary synchronous spasms of the eyelid protractors, whichmay be brief or sustained. It has an estimated prevalence of 4 per 100,000, affects women approximately three times more commonly than men, and generally presents in the fifth to seventh decade of life.1–3 Clinical presentation varies from a mildly increased blink rate to forceful and sustained contraction of the periocular muscles resulting in functional blindness. The uncontrollable nature of the spasms can be anxiety provoking and socially disabling. It may also affect contiguous areas of the mid and lower face and/or neck, and in these instances, is termed a segmental cranial dystonia. The disease course is generally progressive, especially during the initial years, with few patients ever achieving remission.2,4,5 Mainstay treatment options can be divided into three areas including management of sensory input, motor output, and central control. Although established treatment modalities have been identified and developed for the former two, central control of the disease has been more elusive for clinicians. Because the neuronal pathways of blinking have yet to be fully elucidated, drug therapy has proven to be challenging. Without a clear pharmacologic target, a variety of medications from a wide range of drug classes have been tried with varying success and associated side effect profiles. Treatment response to oral medication is typically characterized as partial, if any, resulting in individuals being tried on a variety of drugs.

Midface-lift with lateral orbicularis oculi flap excision or imbrication.

BACKGROUND The orbicularis oculi advancement midface-lift treats rhytides and infraorbital grooves. The muscle flap smoothes the subciliary lid and cheek zone but may produce contour abnormalities laterally. OBJECTIVE To describe a technique of orbicularis flap midface-lift with excision or imbrication of muscle and to evaluate the results—to include characterizing the lateral contour abnormalities—in a large series. MATERIALS and METHODS A total of 108 patients received a primary aesthetic orbicularis advancement flap midface-lift. Two masked observers scored each patients result based on preoperative and postoperative images. Patients also scored their satisfaction of the results of their procedures. Patient age, sex, surgical technique specifics (i.e., excision or imbrication), and complications were recorded. RESULTS All patients had favorable aesthetic improvement scores with no significant complications. In patients who received orbicularis excision (N = 33), lateral hollowing occurred in 3 patients; none desired correction. With an imbrication technique (N = 75), lateral mounding occurred in 6 patients; 3 of these patients were treated with elliptical excision. CONCLUSION Orbicularis advancement midface-lifting was safe and effective in rejuvenating the midface in this series. Aesthetic complications were infrequent but include lateral hollowing when orbicularis is excised and lateral mounding with muscle imbrication.

Levator Aponeurosis Dehiscence: External Levator Advancement

Dehiscence of levator aponeurosis was historically viewed as the exclusive cause of acquired blepharoptosis with normal levator function. In reality, acquired blepharoptosis with normal levator function is likely triggered by many factors including levator aponeurosis dehiscence from the anterior face of tarsus, stretching of the aponeurosis, and/or fatty infiltration of levator muscle. Irrespective of the underlining pathophysiology, external advancement of levator aponeurosis can effectively treat acquired blepharoptosis with normal levator function. Even in experienced hands, especially when an eyelid has been previously operated or there is significant fatty infiltration of levator muscle, external levator advancement can be a challenging operation. The main steps of this surgery are as follows: identification of the anterior face of levator aponeurosis, complete disinsertion of levator aponeurosis from its insertion on tarsus, advancement of the free edge of aponeurosis, and reattachment of this edge onto tarsus.

Congenital sodium diarrhea and chorioretinal coloboma with optic disc coloboma in a patient with biallelic SPINT2 mutations, including p.(Tyr163Cys)

Congenital sodium diarrhea is a rare and life‐threatening disorder characterized by a severe, secretory diarrhea containing high concentrations of sodium, leading to hyponatremia and metabolic acidosis. It may occur in isolation or in association with systemic features such as facial dysmorphism, choanal atresia, imperforate anus, and corneal erosions. Mutations in the serine protease inhibitor, Kunitz‐Type 2 (SPINT2) gene have been associated with congenital sodium diarrhea and additional syndromic features. We present a child with congenital sodium diarrhea, cleft lip and palate, corneal erosions, optic nerve coloboma, and intermittent exotropia who was found to have biallelic mutations in SPINT2. One mutation, c.488A > G, predicting p.(Tyr163Cys), has been previously associated with a syndromic form of congenital sodium diarrhea. The other mutation, c.166_167dupTA, predicting p.(Asn57Thrfs*24) has not previously been reported and is likely a novel pathogenic variant for this disorder. We found only one other report of an optic nerve coloboma associated with SPINT2 mutations and this occurred in a patient with congenital tufting enteropathy. Our patient confirms an association of ocular coloboma with presumed loss of SPINT2 function.

Normalization of Congenital Venous Stasis Retinopathy Following Sclerotherapy of a Macrocystic Lymphatic Malformation

The authors describe, for the first time to their knowledge, a case of a congenital macrocystic lymphatic malformation of the orbit with associated venous stasis retinopathy that acutely normalized after drainage and sclerotherapy of the lesion. Prenatal ultrasound revealed prominence of the left orbital soft tissue, and at birth, the patient was noted to have unilateral proptosis, tortuous retinal vessels, and intraretinal hemorrhages in all 4 quadrants in the left eye. MRI demonstrated a primarily intraconal, multiloculated, T2-hyperintense mass consistent with a lymphatic malformation. Ultrasound-guided cyst aspiration and sclerotherapy was performed, with subsequent improvement of the proptosis and resolution of the vessel tortuosity and intraretinal hemorrhages. Although venous stasis retinopathy is usually related to central retinal vein occlusion or carotid artery occlusive disease, any entity that increases orbital venous resistance can generate retinal venous dilation and intraretinal hemorrhages, including an orbital lymphatic malformation.

Invasive Aspergillosis Masquerading as a Lacrimal Sac Abscess.

A 5-year-old male with B-cell acute lymphoblastic leukemia was referred for presumed left-sided acute bacterial dacryocystitis. He had left medial canthal edema and hyperemia and outside CT imaging was consistent with bacterial dacryocystitis (Fig. 1A). MRI on arrival revealed loss of contrast enhancement within the lacrimal sac and surrounding tissues, which was highly concerning for invasive fungal disease (Fig. 1B). Interestingly, the area of nonenhancement tracked from the lacrimal sac to the inferior meatus, implicating the entire left lacrimal system and adjacent soft tissue and bone (Fig. 1C). Intraoperative exploration confirmed the lack of an abscess. Instead, necrosis was found throughout and surrounding the left lacrimal system. Frozen sections of the left inferior turbinate, medial canthus, and inferior orbital margin confirmed invasive fungal disease. The medial orbit, lateral nasal cavity, and adjacent sinuses were debrided and retrobulbar amphotericin B was injected (Fig. 2A). MRI following debridement showed complete removal of nonenhancing tissue (Fig. 2B). Cultures grew Aspergillus flavus and the patient was treated with systemic voriconazole and caspofungin. Although invasive fungal disease may initially enhance on imaging, the involved tissues become nonenhancing as vascular invasion, occlusion, and subsequent necrosis ensue. Both a bacterial abscess and advanced invasive fungal disease can appear as an area of nonenhancement surrounded by reactive inflammation. However, a bacterial abscess usually occupies a well-delineated space, such as the lacrimal sac, whereas invasive fungal disease diffusely invades adjacent anatomy. Therefore, on CT imaging, this patient presented with findings suggestive of acute bacterial dacryocystitis. However, MRI later revealed signs of diffuse soft tissue and bony necrosis emanating from the entire left lacrimal system that, although highly atypical, was indicative of an invasive fungal process. DOI: 10.1097/IOP.0000000000000895 Accepted for publication January 24, 2017. Funded in part by an institutional grant from Research to Prevent Blindness. The authors have no financial or conflicts of interest to disclose. Address correspondence and reprint requests to Kristin E. Hirabayashi, M.D., 10 Koret Way, Box 0730, San Francisco, CA 94143. E-mail: khirabay@ gmail.com Invasive Aspergillosis Masquerading as a Lacrimal Sac Abscess

Fire Risk with Nasal Cannula Oxygen

Dear Editor: Huddleston et al raise awareness of fire hazards with supplemental oxygen during ophthalmic plastic surgery and succinctly outline the variables that contribute to devastating operating room fires. We question the conclusion that “insisting on nasal cannula use should be our next step toward eliminating surgical fires.” Several studies demonstrate the fire dangers of oxygen supplementation via nasal cannula. The number of locations in the facial field that breach thresholds for combustion riskmay correlate directly with flow rate. Huddleston et al do not provide flow rates that may be relevant. Nevertheless, even at low flow rates, nasal cannulated oxygenmay still cause serious fire hazards during oculofacial surgery with ignition sources such as cautery or laser in close proximity. Although there are limitations with each, we are aware of 3 strategies to reduce the risk of fire with nasal cannulated oxygen: (1) The Emergency Care Research Institute recommends stopping supplemental oxygen 1 minute before and during the use of electrocautery devices or lasers, (2) Engel et al describe a modified nasopharyngeal tube to direct oxygen into the posterior pharynx, and (3) we described the use of a midfacial seal drape to achieve a barrier between excess oxygen and the surgical field. Operating room fires are underreported and represent a preventable cause of morbidity and mortality. Further attention to the topic may clarify the safest methods for oxygen delivery during oculofacial surgery and forestall further devastating events.