Joyce T. Yuan

Fibrous dysplasia of the zygomaticomaxillary region: outcomes of surgical intervention.

Background: Fibrous dysplasia is the most common craniofacial tumor, presenting in both monostotic and polyostotic forms with varying degrees of severity. No consensus exists regarding the surgical management of craniofacial fibrous dysplasia, particularly in the zygomaticomaxillary region. The present study compared long-term outcomes of limited reduction burring versus radical resection of zygomaticomaxillary fibrous dysplasia. Methods: Patients with craniofacial fibrous dysplasia at the University of California, Los Angeles, Craniofacial Center from 1982 to 2008 were studied based on demographics, treatment, and follow-up data, including examinations, computed tomographic scans, photographs, physician Whitaker scoring, and patient surveys (n = 97). Outcomes were compared for zygomaticomaxillary disease treated with radical resection with cranial bone graft reconstruction or limited reduction burring (n = 58). Results: Thirty-four percent of patients had monostotic disease, 66 percent had polyostotic disease, 3 percent had McCune-Albright syndrome, and 2.1 percent had malignant degeneration into osteosarcoma. Most patients had surgical treatment (84.5 percent). Of the patients that required optic nerve decompression for vision changes (11.4 percent), most (75 percent) had vision stabilization postoperatively. Differences were recorded in zygomaticomaxillary disease treated with radical resection (63.8 percent) versus reduction burring (36.2 percent) according to age (19.6 versus 14.2 years), complications (13.5 percent versus 4.8 percent), recurrence (66.7 percent versus 24.3 percent), and number of subsequent procedures (2.8 versus 4.0). There were similarities in Whitaker outcome score (1.3 ± 0.3 versus 1.5 ± 0.6) and patient satisfaction (2.7 ± 0.4 versus 2.8 ± 0.3). Conclusions: Although different approaches have been advocated to treat fibrous dysplasia, the authors’ data support a more aggressive management for zygomaticomaxillary disease with radical resection and cranial bone graft reconstruction, especially for more involved disease. CLINICAL QUESTION OF EVIDENCE: Therapeutic, III.

Giant cranial osteoma: successful staged excision of the largest reported.

Craniofacial osteomas are benign, slow-growing neoplasms of the craniofacial region that are usually asymptomatic. Uncommonly, giant craniofacial osteomas may be symptomatic and cause serious morbidity including ophthalmologic problems, cerebral compression, pneumocephalus, and seizures. We present a case of a 15-year-old Asian adolescent girl with a giant cranial osteoma (17.5 × 13.2 × 5 cm: significantly larger than previously reported). She also had multiple other synchronous giant osteomas of the face causing facial asymmetry. These osteomas were resected in a multiple-staged approach with a good aesthetic and functional outcome.

Asymmetric class III malocclusion: association with cranial base deformation and occult torticollis.

Abstract The etiology of Angle class III malocclusion with facial asymmetry has not been fully elucidated. To investigate the etiology, patients with asymmetric prognathism (n = 30) from a single institution were assessed for previously undiagnosed torticollis and cranial base asymmetry. Presence of torticollis was determined by measuring restricted head movement when turning the head against a wall and cranial base tilt with upward gaze. Cranial base asymmetry was evaluated by preoperative three-dimensional computed tomography scans. Thirty-one percent of patients with prognathism presented with concurrent facial asymmetry. In patients with asymmetric prognathism, cranial base tilt was present on upward gaze in all patients; mean angle between head and wall was 31 degrees greater than that in control patients, and a 22% to 36% difference in the angle was present when comparing one side with the other. Based on these findings, all patients with asymmetric prognathism were found to be affected by torticollis. By computed tomography scan, 85% of these torticollis patients showed slight anteromedial displacement of the glenoid fossa ipsilateral to torticollis, and 73% demonstrated temporal fossa shift of 4 mm or greater. The current study demonstrates a strong association between asymmetric class III malocclusion, torticollis, and cranial base asymmetry. We conclude that undiagnosed torticollis is a likely etiology for otherwise idiopathic cranial base asymmetry and that cranial base asymmetry in turn causes facial asymmetry and malocclusion. This study highlights the importance of evaluating cranial base asymmetry and torticollis in patients with class III malocclusion to allow for earlier treatment and improved outcomes.

Complications of hidradenitis suppurativa

Hidradenitis suppurativa (HS) is a chronic inflammatory skin disease characterized by recurrent painful nodules and abscesses involving intertriginous areas. Repeated episodes of profound inflammation in HS can lead to a number of complications, causing significant morbidity and decreasing quality of life. Complications of HS may affect the skin alone or may have systemic impact. Cutaneous complications of HS include sinus tracts, fistulae, scarring and contractures, squamous cell carcinoma, and lymphedema. Systemic complications of HS include chronic pain, systemic amyloidosis, and possibly anemia. Preventing disease complications by controlling primary disease is a key component of HS management. Clinicians should be prepared to recognize complications early, as prompt management is necessary to minimize negative impacts.

Customized bilaminar resorbable mesh with BMP-2 promotes cranial bone defect healing.

BackgroundFor repair of cranial vault (skull) defects, alloplastic (methylmethacrylate or titanium mesh) techniques may result in nonhealing or infectious complications and autogenous (split rib or calvarial) techniques may result in excessive blood loss or donor-site morbidity. Osteogenic factors such as bone morphogenetic protein-2 (BMP-2) provide promising alternatives for repairing bone defects. As a new option for this reconstructive challenge, we investigated by using a bilaminar resorbable construct made from computer-aided design and computer-aided manufacturing computed tomographic scan technology with BMP-2 in hopes of providing initial structural support for the skull, followed by bone healing without permanent foreign body problems. MethodsOutcomes of consecutive adult patients with long-standing critical-sized cranial vault defects who underwent cranial vault reconstruction between 1999 and 2010 were studied (n = 69). Reconstructive options were titanium mesh, cryopreserved bone, patient-specific implants, autogenous (split calvarial), and a new bilaminar resorbable mesh construct sandwiched with BMP-2. These reconstructive options were compared for operating room time, blood loss, complications, reoperations, and bone healing (3-dimensional computed tomographic scans). ResultsDefects in the group were similar, 92 cm2 (60–176 cm2). The autogenous bone group had the longest operating room time (1.1 times longer than the BMP-2 construct group) and the greatest blood loss (1.5 times more than the BMP-2 construct group). Perioperative complications and reoperation rates were lower in autogenous bone (5%) and BMP-2 construct groups (14%) when compared with alloplastic groups (22%–36%). Bone healing was superior with BMP-2 construct and autogenous bone (85%; 90%) versus cryopreserved bone (18%) and minimal healing for alloplastic. ConclusionsIn select cases, a customized bilaminar resorbable construct with BMP-2 resulted in improved bone healing with fewer complications than alloplastic options and without the morbidity and blood loss associated with autogenous bone grafting. This allowed critically sized calvarial defects in adults to be successfully corrected with no residual foreign body.

Validity of skin cancer malignancy reporting to the Organ Procurement Transplant Network: A cohort study

Background The Organ Procurement Transplant Network (OPTN) registry collects data on posttransplant malignancies in solid organ transplant recipients. Complete and accurate registry data on skin cancer is critical for research on epidemiology and interventions. Objective The study goal was to determine the validity of Organ Procurement Transplant Network skin cancer data. Methods This cohort study compared reporting of posttransplant squamous cell carcinoma (SCC) and malignant melanoma (MM) in OPTN to medical‐record review‐derived data from the Transplant Skin Cancer Network (TSCN) database. In total, 4934 organ transplant recipients from the TSCN database were linked to patient‐level OPTN malignancy data. We calculated sensitivity, specificity, correct classification (CC), positive predictive value (PPV), and negative predictive value (NPV) for SCC and MM reporting in the OPTN database. Results OPTN reporting for SCC (population prevalence 11%) had sensitivity 41%, specificity 99%, PPV 88%, NPV 93%, and CC 93%. OPTN reporting for MM (population prevalence 1%) had sensitivity 22%, specificity 100%, PPV 73%, NPV 99%, and CC 99%. Limitations Only a subset of patients in the TSCN cohort had matched United Network for Organ Sharing cancer registry data for comparison. Conclusion OPTN reporting had poor sensitivity but excellent specificity for SCC and MM. Dermatologists and transplant physicians are encouraged to improve the validity of OPTN skin cancer data through improved communication and reporting.

Medical marijuana for the treatment of vismodegib-related muscle spasm

Author(s): Yuan, Joyce T; Tello, Tiffany L; Hultman, Carter; Barker, Christopher A; Arron, Sarah T; Yom, Sue S

Pathology Quiz Case 1

A 70-YEAR-OLD MAN PRESENTED WITH A REcurrent right parotid mass. Five months earlier, he had presented at an outside hospital with a 4.3 3.2 2.6-cm level 2 mass in the right side of his neck. Two months after the excision, the mass recurred. Magnetic resonance imaging of the neck showed a 6 5-cm ill-defined mass of low signal intensity in superficial and deep parotid lobes, with infiltration of the surrounding soft tissues (Figure1). A chest radiograph revealed multifocal opacities. Positron emission tomography–computed tomography showed diffusehypermetabolic lymphadenopathy throughout thebody and hypermetabolic activity in the right parotid gland. An excisional biopsy specimen of a level 2 lymph node on the right side demonstrated follicular hyperplasia, increased immunoblasts, germinal centers, and mantle zones, with no evidence of lymphoma. The patient underwent a right total parotidectomy with facial nerve preservation. Pathologic examination demonstrated marked periductal and interlobular sclerosis with prominent collagen bands, lymphoid hyperplasia with germinal centers, numerous small lymphocytes, and plasma cells (Figure 2 and Figure 3). Immunohistochemical analysis revealed markedly increased IgG4 cells (Figure 4), with more than 80 IgG4-positive plasma cells per high-power field and an IgG4-IgG ratio of 10:1.Pathologic examination showed no evidence of lymphoma. Serologic tests demonstrated elevated levels of IgG (2940 mg/dL [reference range, 690-1660 mg/dL]) and IgG4 (1030 mg/dL [reference range, 4-86]) (to convert IgG and IgG4 levels to grams per liter, multiply by 0.01). Prednisone and azathioprine therapy was initiated, and the patient showed rapid improvement of symptoms and resolution of pulmonary opacities on computed tomograms of the chest. The level of IgG4 decreased to 19.4 mg/dL after 1 month and was then successfully maintained within normal limits, with no disease recurrence at the 9-month follow-up visit. What is your diagnosis?

Abstract 100: Nager Syndrome Dental Pulp Stem Cells Have Osteogenic Potential

Purpose: Our laboratory is developing a regenerative peripheral nerve interface (RPNI) device. RPNI construction includes a light wrap of decellularized small-intestine submucosa (SIS) as a scaffold upon which an ionic/electrical conductive material is polymerized. However, the porosity of SIS and SIS with polymer is unknown. The purpose of this study is to determine RPNI revascularization, vitality, and reinnervation when SIS contains poly 3,4 ethylene dioxytheophene (PEDOT).