One-Stage Supramaximal Full-Thickness Wedge Resection of Vascular Lip Anomalies.

PURPOSE: Vascular lip anomalies include infantile hemangiomas, venous malformations, and arteriovenous malformations. Surgical management can be complicated by alterations in horizontal length, vertical height, and lip thickness from the underlying pathology. Additional reconstructive challenges include preservation of oral continence, vermillion definition, and the sublabial sulcus. This report describes a technique of supramaximal single-stage full-thickness wedge resection of these lesions. MATERIALS AND METHODS: A retrospective study approved by the institutional review board of patients who underwent full-thickness resection of vascular lip anomalies from December 2007 through February 2013 was performed. Patient demographics, final diagnosis, preoperative treatment, examination findings (pre- and postoperative), intraoperative management, and follow-up findings were reviewed. RESULTS: Eighteen patients (9 female) with a mean age of 25 ± 19.8 years (range, 3 to 70 yr) were identified. Underlying pathologies were arteriovenous malformation (6), port wine stain with secondary soft tissue hypertrophy (6), capillary malformation (1), venous malformation (3), and infantile hemangioma (2). Prior treatments included embolization, lasers, surgical excision, steroids, and propranolol; 8 patients were treatment naive. All patients underwent a single or double pentagonal-shaped wedge resection of the involved upper or lower lip. An average of 3.75 cm (41.7%; range, 20 to 70%) of horizontal lip length was excised. Four patients required additional concomitant debulking of the vermillion. No intraoperative complications were noted. Postoperatively, 1 patient developed wound dehiscence at 12 days and another had a midline depression with mild oral incontinence. Follow-up averaged 1,074 days (range, 371 to 1,777 days) and patients and their parents reported a high degree of satisfaction with the improvement in lip appearance and symmetry except for 1 patient who required further debulking. The sublabial sulcus and vermillion and cutaneous definition were preserved in all cases. CONCLUSION: Despite the traditional recommendation that no more than 30% of lip length be surgically removed, vascular lip anomalies result in tissue hypertrophy, horizontal elongation, ectropion labii, and tissue expansion that allow up to 70% of the lip to be excised.

The role of conjunctival biopsy in the diagnosis of granulomatosis with polyangiitis.

BACKGROUND: The purpose of this study is to describe a patient who was diagnosed with granulomatosis with polyangiitis based on conjunctival biopsy. This study is a case report and review of the literature. FINDINGS: A 48-year-old Caucasian woman presented with a 2-week history of a left eye peripheral corneal ulcer with adjacent conjunctivitis and a 4-month history of a non-resolving productive cough. Given her elevated serum perinuclear antineutrophil cytoplasmic antibody (P-ANCA) and erythrocyte sedimentation rate (ESR) levels as well as a chest computed topography (CT) that showed bilateral patchy infiltrates, suspicion of limited granulomatosis with polyangiitis with lung and ocular involvement was high. Because bronchoalveolar lavage was nondiagnostic for granulomatous disease, conjunctival biopsy was initially attempted in order to avoid a more invasive lung biopsy. The conjunctival biopsy revealed mixed subacute inflammatory mediators and vasculitis consistent with granulomatosis with polyangiitis. CONCLUSIONS: Conjunctival biopsy may be a valuable, minimally invasive method for diagnosing systemic granulomatosis with polyangiitis.

Extruded, partially disintegrated, poly-HEMA orbital implant (AlphaSphere).

A 54-year-old diabetic man underwent enucleation for endophthalmitis. Secondary implantation of a 2-hydroxyethyl methacrylate (HEMA) sphere (AlphaSphere, Addition Technology) was performed 2 weeks later. Six weeks after insertion, noninfectious disintegration of sutured tissue planes represented by Tenon's capsule, rectus muscle, and conjunctiva occurred, requiring removal of the fragmenting implant before uncontrolled extrusion occurred. Histopathologic analysis revealed an absence of infectious pathogens and no tissue necrosis, but rather breakup of the implant material that elicited a granulomatous response with sparse T-lymphocytes and almost no polymorphonuclear leukocytes. This distinctively designed poly-HEMA orbital implant incited a dramatic and irreversible host tissue response. Investigation of other cases will be necessary to determine the frequency of such a complication and should include rigorous histopathologic techniques.

Comparison of methodologies in volumetric orbitometry.

PURPOSE: The rate at which the orbit matures is not well-documented. Limiting this pursuit are the difficulties inherent in measuring orbital volumes accurately. This study compared 3 common methods of determining orbital volume and sought to identify an accurate, practical manner for doing so. METHODS: The volume of 1 orbit of 8 human cadaver heads was independently measured using 3 different methods: 1) CT was performed, and images were analyzed with 3-dimensional (3D) volumetric software; 2) The same orbits were then exenterated and a silicone cast was taken. The cast volumes were measured by water displacement; 3) The orbits were then filled with 1-mm glass beads that were transferred to a graduated cylinder where their volume was determined. The data were analyzed statistically. RESULTS: Intraobserver agreements were good for both beads and casts. Interobserver agreements were good for both beads and CT (p > 0.05). Values obtained using the bead method were equal to values obtained using the cast method (p > 0.05). However, agreement between direct (orbital fillers and casts) and indirect measurements (radiographic techniques) was not satisfactory (p < 0.05). CONCLUSIONS: Independent of method, determining orbital volume is inherently difficult owing to the hyperbolic parabola that is the orbit entrance; all methods require estimation. Glass beads and casts yielded more reproducible values but can only be used in cadavers. CT measurement is prone to error due to the variability of methodologies used but allows access to enormous testing populations. Interstudy comparison is currently not possible. CT volumetric software with strict universal standards for estimating the anterior limit of the orbit appears to be the best method of studying human orbital volumes.

Vascular anomalies of the head and neck: a review of genetics.

PURPOSE: Vascular anomalies comprise malformations, hemangiomas, and rare tumors. The commonality among these lesions is their origin in vascular endothelia. Most occur sporadically, but occasional inheritance is observed and thus allows genetic research and insight into etiology. This review highlights those vascular anomalies in which genetic inheritance has been demonstrated. METHODS: A comprehensive literature search was performed on PubMed. Fifty-five full-length articles were reviewed. RESULTS: Five categories of vascular anomalies with patterned inheritance were identified: arteriovenous malformation (AVM), capillary malformation (CM), lymphatic malformation (LM), venous malformation (VM), and infantile hemangioma (IH). Capillary and arteriovenous malformation subtypes are associated with a RASA-1 gene mutation and show autosomal dominant inheritance. VEGFR3 mutations have been associated with generalized forms of LM and lymphedema. Mutations in TIE2/TEK genes cause inherited forms of venous malformations also with autosomal dominant inheritance. Familial clustering and atopic disease are associated with infantile hemangioma, and gene expression varies with the developmental stage of these lesions. CONCLUSION: Most vascular anomalies occur sporadically, but several genes and genetic disorders have been associated with them. Specific forms of capillary malformation appear to be most convincingly associated with genomic errors. Further research promises new insights into the development of this diverse group of disorders.

A modified lacrimal sac implant for high-risk dacryocystorhinostomy.

PURPOSE: The reported 5% of patients with nasolacrimal duct obstruction who fail dacryocystorhinostomy likely include patients with severe mucosal disease or anatomical anomalies. The technique described herein avoids mucosal anastomosis and minimizes mucosal manipulation by inserting a permanent silicone conduit from the lacrimal sac into the nasal cavity. METHODS: This retrospective review of 9 surgical cases was performed with institutional review board approval. Six patients underwent 9 surgeries (3 sequentially bilateral) for dacryocystitis. Two patients had Wegener granulomatosis, 1 had pemphigoid, 1 sarcoidosis, 1 Rosai-Dorfman disease, and 1 congenital choanal atresia with chronic neonatal dacryocystitis. In each case, a modified Rains sinus stent was inserted through an external lacrimal sac incision with the draining end positioned in the nasal cavity. Two patients underwent concurrent canalicular intubation with Guibor silicone stents to prevent internal punctum obstruction by the lacrimal sac implant. Recurrence of symptoms, patient comfort, and modified Rains stent stability and patency were evaluated. RESULTS: Mean follow up was 30 months (range 7-59 months). The modified Rains stent remained stable and patent in 7 of 9 cases, and symptoms resolved in 8 of 9 cases. In 1 patient with sarcoidosis, the modified Rains stent became repeatedly obstructed with nasal secretions and ultimately dislodged after intranasal manipulation by a physician unfamiliar with the surgery. In no other case did the patient experience recurrent infection, and in those cases, epiphora resolved entirely. In the patient with pemphigoid, one of the modified Rains stents extruded 6 months postoperatively, but his symptoms remained controlled. No adverse reaction to the implant material was seen. CONCLUSIONS: A Rains silicone frontal sinus stent can be modified for implantation into the lacrimal sac and can safely and effectively drain the lacrimal sac into the nose in patients with severe mucosal disease or anatomical anomalies. Additional study and a stent specifically designed for this application will likely improve outcomes.

Orbital development as a function of age in indigenous North American skeletons.

PURPOSE: Infants with orbital hemangiomas and vascular malformations often develop expanded orbits or regional hyperostosis. Treatment in these cases depends, in part, on the stage of orbital development at the time of intervention; yet, orbital development with respect to age is not well-known. The authors sought to determine the rate of orbital development and the age of orbital maturation in a single ethnic population. METHODS: Skeletons recovered in North America and housed at the Peabody Museum of Archaeology and Ethnology, Harvard University, were inspected. The age of specimen was determined by dentition. Orbital volume was measured using 1-mm glass beads and a graduated cylinder. Linear measurements were taken with calipers and paper rulers. The measurements were plotted against age, and statistical analysis was performed. Relevant literature was reviewed. RESULTS: Of the hundreds of skeletons examined, 42 were sufficiently intact for orbital measurement. The specimens represented a period of up to 1000 years. Thirty-two were pediatric (defined prenatal to 18 years) and 10 were adults. Mean adult orbital volume was 26.2 ml. Based on the regression analysis, 60% of adult orbital volume was achieved at 4.35 years, 75% at 9.36 years, and 90% at 17.13 years of age. Linear dimensions progressively increased with age. CONCLUSIONS: This largest direct-measure study of pediatric orbital volume suggests that orbital growth continually decelerates from birth until maturity at 22 years. With 50% of orbital growth occurring by 16 months of age, surgeons removing periocular vascular anomalies after that age should consider concurrent skeletal management.

Self-irrigating piezoelectric device in orbital surgery.

PURPOSE: Orbital osteotomy risks injury to the eyeball and orbit soft tissues. Used extensively in oral and maxillofacial surgery, piezoelectric technology offers a greater margin of safety than traditional bone cutting instruments. The authors describe the novel use of this system in a variety of orbital surgeries. METHODS: This interventional case series was performed in accordance with institutional review board regulations. The medical records of all patients who had undergone orbital surgery using the piezoelectric blade at 3 institutions were reviewed. Indication for surgery, gender, age, duration of follow up, intraoperative complications, surgical result, and postoperative course was recorded. RESULTS: Sixteen patients underwent surgery on 18 orbits using the piezoelectric system between August 2011 and June 2012. Surgeries performed included orbital decompression (8), lateral orbitotomy (5), cranio-orbitotomy (4), and external dacryocystorhinostomy (1). Eight were female and 8 were male patients. Mean age was 55 years old (standard deviation 15 years). Mean follow up was 82 days. The osteotomy created by the blade was narrow and smooth in every case. The surgeons uniformly appreciated the precision and safety of the instrument compared with traditional electric saw blades. There were no soft tissue lacerations or intraoperative complications and reconstructions were uniformly uneventful. Postoperative healing was rapid with no unexpected inflammation, and no palpable bony defects were appreciated in the reconstructed cases. CONCLUSIONS: Because it does not cut soft tissue and cuts a narrow trough, the self-irrigating piezoelectric saw blade appears safer and more precise than traditional electric saw blades in and around the orbit.

Dynamic imaging of paralytic eyelid disorders.

PURPOSE: Eyelid dysmotility may result from trauma, tumors, inflammation, infection, and a variety of other conditions. In these cases, a mechanical effect is disrupting a normal neuromuscular apparatus. Dysmotility can also be caused by paralytic eyelid disorders; included in this broad category are neurologic and myogenic disorders of eyelid opening and/or closure. Secondary effects include spastic eyelid closure and synkinesis syndromes. These conditions, by definition, are disorders of movement, and can only be studied adequately using dynamic imaging techniques. METHODS: A comprehensive literature search was performed on PubMed. Ninety abstracts were reviewed. RESULTS: Dynamic eyelid imaging has evolved dramatically over the past two decades, at least partially due to the rise of inexpensive digital technology. Magnetic search coil imaging, high- and low-speed videography, electromyography, and high-resolution microscopy coil magnetic resonance imaging each has its advantages and disadvantages, an understanding of which will guide appropriate selection of technology in any given clinical situation. CONCLUSIONS: Dynamic eyelid imaging is useful to study dysmotility. The optimal technique depends upon the clinical setting and the physiologic or pathologic topic of interest. To our knowledge, a report of this type has not been previously summarized.