Andre Panossian

ABSTRACT Vascular malformations require a multidisciplinary approach in diagnosis, management, and treatment. Capillary malformations are the most common vascular malformation and typically are associated with limb hypertrophy in the affected extremity. Venous malformations can lead to hemarthrosis, muscle contractures, or osseous deformities and fractures and require extensive management and therapy. Arteriovenous malformations cause painful skeletal overgrowth and limb hypertrophy and may lead to ischemia of the tips of digits. Vascular anomalies are among the most common congenital abnormalities in infants and children. The management of vascular anomalies requires interdisciplinary care and collaboration between orthopaedic surgeons, general surgeons, plastic surgeons, interventional radiologists, dermatologists, oncologists, and child life specialists, leading to the establishment of vascular anomaly teams at tertiary centers. Vascular anomalies are a heterogeneous group of conditions, with similar clinical appearance but substantially different manifestations. The development of an appropriate classification system has helped to reduce confusion regarding terminology, thus improving diagnosis and management. There are two groups of vascular anomalies: (1) vascular tumors and (2) vascular malformations. Differentiating between these two types, and their subtypes, is essential because of their distinct natural history and treatment. The goal of this review is to highlight the role of the orthopaedic surgeon in the approach and treatment of extremity vascular malformations and to discuss diagnostic strategies and management at a multidisciplinary level. We also review the most common associated syndromes seen in the orthopaedic practice, including Klippel-Trenaunay syndrome, Proteus syndrome, Maffucci syndrome, Gorham-Stout disease or syndrome, Parkes Weber syndrome, and Bannayan-Riley-Ruvalcaba syndrome. Classification In 1863, Virchow was the first to classify vascular anomalies into groups on the basis of their histopathologic appearance.

The first practical uses of polytetrafluoroethylene in medicine date back to 1972, when Soyer et al.1 introduced a new biocompatible polymer designed to serve as the next generation of vascular graft technology. Since then, the long-term safety and efficacy of the substance have been well documented, and the use of polytetrafluoroethylene grafts has extended to the realm of facial plastic surgery, with promising results. Currently, two formulations of expanded polytetrafluoroethylene are typically used in facial reconstruction today. Pure e-polytetrafluoroethylene is an expanded, strongly hydrophobic, fibrillated polymer that can be produced in sheets, three-dimensional strands, and suture material. It is composed of nodules of polytetrafluoroethylene interconnected through a fibrous mesh network, creating a three-dimensionally amorphous structure.2 Fluorinated e-polytetrafluoroethylene is based on the standard architecture of e-polytetrafluoroethylene with the addition of reinforci...

BACKGROUND Diffuse venous malformations that involve all tissues in the upper limb and ipsilateral chest wall are known as "phlebectasia of Bockenheimer." The authors describe their experience with management of this uncommon vascular anomaly. METHODS The authors' Vascular Anomalies Center registry comprised 18,766 patients over a 40-year period. This review identified 2036 patients with venous malformations of the extremities (10.8 percent), of whom only 80 (0.43 percent) had Bockenheimer disease. The authors retrospectively analyzed patient characteristics, diagnostics, treatments, and complications. RESULTS The venous malformation was first noted at birth or within the first few years of life with slow and gradual progression. Pain was related to engorgement of the limb. Thromboses and phleboliths were common, but diffuse intravascular coagulopathy occurred in only 12 patients (15 percent). Skeletal involvement was demonstrated as lytic lesions, cortical scalloping, osteopenia, and pathologic fractures. Management included compression garments (100 percent), sclerotherapy (27.5 percent), and resection of symptomatic areas in 35 percent of patients. Adjunctive pharmacologic medication was given in 7.5 percent. Following resection, 17 patients (60 percent) had one or more complications: hematoma, wound dehiscence, flap loss, contracture, and psychosis. There were no deaths. Symptoms improved in all patients with useful functional outcomes. CONCLUSIONS The decision to pursue compression, sclerotherapy, pharmacologic treatment, or resection alone or in combination was made by an interdisciplinary team. Although extensive venous malformations cannot be completely ablated, debulking of symptomatic regions, resection of neuromas, and noninvasive treatments improve the quality of life. Despite the bulk and weight of the arm, forearm, and hand, and the ominous appearance on magnetic resonance imaging, these patients remain functional. CLINICAL QUESTION/LEVEL OF EVIDENCE Therapeutic, V.

Copyright © 2013 Daniel E. Levin et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Objective: Vascular anomalies are a diagnostic and therapeutic challenge. Errors in diagnosis lead to treatment delay, inappropriate interventions and prolonged suffering. The aim of this study was to analyze patterns of misdiagnosis among patients re-ferred to a vascular anomalies center (VAC). This will better define the problem and may be used to refine and improve referral guidelines for patients with vascular anomalies. Patients and Methods: After IRB approval, we performed a single-center retro-spective review of all patients referred to a vascular anomaly between January 1, 2008 and December 15,

Facial paralysis remains a vexing problem in the treatment of posterior cranial fossa tumors in children. Fortunately, current techniques are available to reconstruct the paralyzed face in restoring balance, symmetry, and amelioration of functional sequelae. The restoration of structure and function of the paralyzed face is tantamount to proper social integration and psychosocial rehabilitation. In addition, the facial nerve is important in preventing drying of the eyes, drooling, and speech abnormalities, among other functions. The most visible evidence of facial paralysis is stark asymmetry, especially with animation. This is perhaps the most troubling aspect of facial paralysis and the one that leads to the greatest amount of psychosocial stress for the child and family members. Management strategies include early and late intervention. Early reconstructive goals focus on preservation and strengthening of intact motor end plates through native stimulatory pathways. Late reconstru...

1. J Leg Med. 1998 Jun;19(2):223-55. Criminalization of perinatal HIV transmission. Panossian AA, Panossian V, Doumanian NP. Tufts University School of Medicine, Boston, Massachusetts, USA. PMID: 9658740 [PubMed - indexed for MEDLINE]. Publication Types: Review. ...

We report a 7-year-old male with a history of recurrent kaposiform hemangioendothelioma (KHE) in the sacral area and multiple thoracic vertebral lesions. Tumor karyotype revealed a balanced translocation t (13; 16) (q14; p13.3). He had mildly decreased platelet counts but never experienced any episodes of Kasabach-Merritt phenomenon. He was treated with vincristine for one year with minor improvement. Significant clinical improvement was seen with sirolimus therapy. To the best of our knowledge, this is the first report to demonstrate that KHE is associated with a clonal karyotypic abnormality.

Lymphatic malformations are congenital vascular anomalies that occur from abnormal development of the lymphatic channels. Studies have shown that sclerotherapy can be a reliable alternative to surgery. The purpose of this retrospective study is to evaluate the safety and efficacy of percutaneous sclerotherapy with doxycycline and 3% Sotradecol as primary treatment for pediatric head and neck LMs, and to assess outcomes based on lesion classification, location and sclerosant used. This study was a single center, retrospective, case series study. The medical records of 38 children who underwent percutaneous sclerotherapy of LMs in the head and neck region at our tertiary care center between 1/1/2006 and 1/31/2011 were reviewed. A mean average of 2.9 (range 1-10) sclerotherapy sessions per child were performed. LMs involved primarily the face (61.3%), posterior neck (48.4%), submental area (45.2%), and anterior neck (35.5%). Twenty-nine subjects had adequate follow-up data, with 51.7% demonstrating complete resolution, 27.6% moderate improvement, and 20.7% no response. There was no significant difference in the outcome based on the sclerosant agent used or location of the lesion. Lesion type did affect outcome and macrocystic lesions were found to have a significantly higher resolution rate (95.2%) than microcystic or mixed lesions (p<0.05). The total number of complications was similar between agents and all were minor. Our results indicate that percutaneous therapy with doxycycline and Sotradecol is safe and effective for children with LMs of the head and neck. Better outcomes were observed with macrocystic LMs. 4.

BACKGROUND: Venous malformations (VM) of the upper limb are low-flow vascular lesions that often present complex management challenges. Present from birth, these masses can be discrete or rhizomal and diffuse. They are comprised of a dilated venous network that commonly plague patients with pain, thrombophlebitis, pathologic fractures, and disfigurement. Venous malformations may involve skin, muscle, bone, or joints and can produce trophic changes in the upper extremity. Unfortunately, there is no algorithm for identifying, diagnosing, and managing such complex lesions. We review our experience with diffuse upper extremity VM and present our anatomically-based, symptomatically-focused management algorithm. METHODS: The authors executed a 20-year retrospective study of 37 patients who presented with diffuse upper limb VM. Patients with only upper limb VM were included. Other parameters gathered were age of onset, age of presentation, gender, anatomical location, signs and symptoms, r...

Free muscle transfer for dynamic smile reanimation in facial paralysis is not always predictable with regard to cosmesis. Hospital stays range from 5 to 7 days. Prolonged operative times, longer hospital stays, and excessive cheek bulk are associated with free flap options. Lengthening temporalis myoplasty offers single-stage smile reanimation with theoretical advantages over free tissue transfer. From 2012 to 2014, 18 lengthening temporalis myoplasties were performed in 14 children for smile reconstruction. A retrospective chart review was completed for demographics, operative times, length of hospital stay, and perioperative complications. Fourteen consecutive patients with complete facial paralysis were included. Four patients underwent single-stage bilateral reconstruction, and 10 underwent unilateral procedures. Diagnoses included Möbius syndrome (n = 5), posterior cranial fossa tumors (n = 4), posttraumatic (n = 2), hemifacial microsomia (n = 1), and idiopathic (n = 2). Average patient age was 10.1 years. Average operative time was 410 minutes (499 minutes for bilateral lengthening temporalis myoplasty and 373 for unilateral lengthening temporalis myoplasty). Average length of stay was 3.3 days (4.75 days for bilateral lengthening temporalis myoplasty and 2.8 for unilateral lengthening temporalis myoplasty). Nine patients required minor revisions. Lengthening temporalis myoplasty is a safe alternative to free tissue transfer for dynamic smile reconstruction in children with facial paralysis. Limited donor-site morbidity, shorter operative times, and shorter hospital stays are some benefits over free flap options. However, revisions are required frequently secondary to tendon avulsions and adhesions. Therapeutic, IV.

ABSTRACT Vascular anomalies of the extremities are not uncommon and can significantly impede limb function. Currently, misdiagnosis is a frequent cause of delay in treatment or initiation of incorrect treatment that may detrimentally impact patients. With accurate diagnosis, the appropriate treatment can be implemented with good functional outcomes in most types of vascular anomalies. The goal of this paper is to review the classification, diagnosis, and treatment of vascular anomalies with particular focus on recent innovations and multimodal management. By clarifying the classification system and developing an algorithmic approach, successful management of vascular anomalies can be achieved. The complexity of these disorders requires communication across multiple disciplines for coordination of care and timely interventions. A typical multidisciplinary team will include an orthopedist, plastic surgeon, general surgeon, interventional radiologist, dermatologist, oncologist, and geneticist, among others.

ABSTRACT Background: Amputation often is a treatment of last resort for vascular anomalies of the extremities and, as such, infrequently is indicated. Our purpose was to review our institutional experience with amputations for vascular anomalies in children, specifically identifying indications and reviewing outcomes. Methods: A retrospective chart review was conducted of all amputations for vascular anomalies at a tertiary pediatric surgery referral center over a 10-year period. Demographics, clinical characteristics, diagnostic imaging, operative details, and outcomes data were collected and analyzed. Results: Eight extremity amputations were performed in six patients. The male-to-female ratio was 1:1. Median age at amputation was 10.7 (2.3-16.1) years. The indications for surgery included refractory pain, nonfunctional limb, inability to ambulate, ischemia, and bleeding. The most common preoperative radiographic study obtained was MRI. Nonoperative management options such as steroid injections, sclerotherapy, or embolization failed in several patients. The median length of follow-up was 3.7 (0.6-7.9) years. One mortality occurred 1 year after amputation secondary to multiorgan system failure in a child with Bannayan-Riley-Ruvalcaba syndrome. Conclusions: Extremity amputation is a radical form of resection for complications of vascular anomalies. However, most patients experience resolution with an acceptable outcome and an improved quality of life.

This study determined the effects of chondrocyte source, cell concentration, and growth period on cartilage production when isolated porcine cells are injected subcutaneously in a nude mouse model. Chondrocytes were isolated from both ear and articular cartilage and were suspended in Ham's F-12 medium at concentrations of 10, 20, 40, and 80 million cells per cubic centimeter. Using the nude mouse model, each concentration group was injected subcutaneously in 100-microl aliquots and was allowed to incubate for 6 weeks in vivo. In addition, cells suspended at a fixed concentration of 40 million cells per cubic centimeter were injected in 100-microl aliquots and were incubated for 1, 2, 3, 4, 5, 6, 9, and 12 weeks. Each concentration or time period studied contained a total of eight mice, with four samples harvested per mouse for a final sample size of 32 constructs. All neocartilage samples were analyzed by histologic characteristics, mass, glycosaminoglycan level, and DNA content. Control groups consisted of native porcine ear and articular cartilage. Specimen mass increased with increasing concentration and incubation time. Ear neocartilage was larger than articular neocartilage at each concentration and time period. At 40 million cells per cubic centimeter, both ear and articular chondrocytes produced optimal neocartilage, without limitations in growth. Specimen mass increased with incubation time up to 6 weeks in both ear and articular samples. No significant variations in glycosaminoglycan content were found in either articular or ear neocartilage, with respect to variable chondrocyte concentration or growth period. Although articular samples demonstrated no significant trends in DNA content over time, ear specimens showed decreasing values through 6 weeks, inversely proportional to increase in specimen mass. Although both articular and ear sources of chondrocytes have been used in past tissue-engineering studies with success, this study indicates that a suspension of ear chondrocytes injected into a subcutaneous location will produce biochemical and histologic data with greater similarity to those of native cartilage. The authors believe that this phenomenon is attributable to the local environment in which isolated chondrocytes from different sources are introduced. The subcutaneous environment of native ear cartilage accommodates subcutaneously injected ear chondrocyte transplants better than articular transplants. Native structural and biochemical cues within the local environment are believed to guide the proliferation of the differentiated chondrocytes.

We present a case of an adolescent with lower gastrointestinal bleeding caused by a colorectal venous malformation (VM) with concomitant portal hypertension. After an episode of massive gastrointestinal bleeding, we performed an extended right hemicolectomy and resection of the VM and selective portosystemic shunt. Here, we present the case and review the literature regarding portal hypertension and gastrointestinal vascular malformations. Additionally, we discuss the physiologic and hemodynamic effects of gastrointestinal vascular malformations on the portal system.

Single-stage lengthening of the forearm using callus distraction is well described; however, forearm lengthening using a 2-stage technique of distraction followed by bone grafting has received less attention. A 2-staged technique can be a better alternative in cases where the surgeon desires extensive lengthening. A retrospective review was undertaken of eleven 2-stage forearm lengthening procedures performed by 1 surgeon over a 15-year period. Indications were radial longitudinal deficiency (8 patients), neonatal ischemic contractures (2 patients), and septic growth arrest (1 patient). Average follow-up was 2.8 years. Distraction was performed on patients an average of 82 mm over an average duration of 24 weeks. Average time to union from the time of distractor removal and grafting was 87 days. Average healing index was 32.1 d/cm. Distraction problems were common and related to the length of time that the distractor was in place; they included pain, pin-related infections, and multiple mechanical device difficulties. Three patients had nonunion, and another had delayed union; however, additional procedures resulted in ultimate bony union in all patients. Demineralized bone matrix and autologous corticocancellous bone grafts yielded predictable healing and good functional results in short-distance distractions. For longer distractions, free vascularized fibula transfer produced the best outcomes. Intercalary cortical allografts did not heal well. Patients with neonatal Volkmann contractures had the most difficulty with distraction and healing, ultimately obtaining little to no lengthening and poor functional outcomes.