Lymphatic Malformation

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Jonathan A Perkins - One of the best experts on this subject based on the ideXlab platform.

  • head and neck Lymphatic Malformation diagnosis and treatment
    2018
    Co-Authors: Jonathan A Perkins, Eric J Monroe, Randall A Bly, Gridhar Shivaram
    Abstract:

    Diagnosis of head and neck Lymphatic Malformation (HNLM) has changed over time from pure description to detection of their molecular cause (Luks et al, J Pediatr 166(4):1048–54. e1–5, 2015; Perkins et al, Otolaryngol Head Neck Surg 142(6):789–794, 2010). Nomenclature used to describe HNLM has broadened from “cystic hygroma” and “lymphangioma” to Malformation, as evidence for continued use of these terms is not apparent (Perkins et al, Otolaryngol Head Neck Surg 142(6):789–794, 2010). This is most apparent in HNLM prenatal diagnosis where in utero ultrasound imaging detects HNLM (Longstreet et al, Otolaryngol Head Neck Surg 52(2):342–347, 2015) (Fig. 20.1). In the perinatal literature, soft-tissue lucency and thickening in the posterior/dorsal neck are still called “cystic hygroma” and are associated with increased risk for abnormal fetal karyotype (Malone et al, Obstet Gynecol 106(2):288–294, 2005). Now the widely available highly sensitive and specific noninvasive prenatal testing (NIPT) can detect abnormal karyotypes and specific genetically determined syndromes (i.e., Noonan) from fetal DNA in maternal blood, without invasive testing (Grande et al, Ultrasound Obstet Gynecol 46(6):650–658, 2015). This shift in our understanding on maternal-fetal physiology and our ability to detect differences between circulating fetal and maternal DNA has also changed our understanding of in utero ultrasound detected large fluid-filled spaces in the head and neck, so we know that they are a result of molecular changes in DNA and not just “watery tumors” or “lymph tumors.” As investigation into the cause and nature of HNLM has occurred, it is felt that “Malformation” is a more accurate way of categorizing these lesions.

  • the Lymphatic Malformation function lmf instrument
    Otolaryngology-Head and Neck Surgery, 2015
    Co-Authors: Erin M Kirkham, Todd C Edwards, Edward M Weaver, Karthik Balakrishnan, Jonathan A Perkins
    Abstract:

    ObjectiveThe Lymphatic Malformation Function (LMF) instrument is a preliminary parent-report assessment designed to measure outcomes in children with cervicofacial Lymphatic Malformation (LM). This study aimed to assess the measurement properties of the LMF, refine it, test criterion validity, and evaluate the test-retest reliability.Study DesignCross-sectional.SettingTwo pediatric tertiary referral centers.SubjectsParents of 60 children from 6 months to 15 years old with cervicofacial LM.MethodsParents were recruited via mail and online. The LMF was administered on paper or online initially and again within 21 days. Response distributions and interitem correlations were examined for item reduction. Exploratory factor analysis was conducted on retained items. Cronbach’s α, Spearman correlation, and intraclass correlation (ICC) coefficients were calculated to test internal consistency, criterion validity (compared to stage), and test-retest reliability, respectively.ResultsOne item was removed due to a flo...

  • head and neck Lymphatic Malformation treatment a systematic review
    Otolaryngology-Head and Neck Surgery, 2012
    Co-Authors: Mary Theresa Adams, Jonathan A Perkins, Babette S Saltzman
    Abstract:

    ObjectiveTo systematically review literature pertaining to head and neck Lymphatic Malformation treatment and to use individual-level data presented in qualifying case series to describe the report...

  • facial nerve mapping and monitoring in Lymphatic Malformation surgery
    International Journal of Pediatric Otorhinolaryngology, 2009
    Co-Authors: Jospeh Chiara, Jonathan A Perkins, Gi Soo Lee, Sepehr Oliaei, Greg Kinney, Jefferson C Slimp
    Abstract:

    Abstract Objective Establish the efficacy of preoperative facial nerve mapping and continuous intraoperative EMG monitoring in protecting the facial nerve during resection of cervicofacial Lymphatic Malformations. Methods Retrospective study in which patients were clinically followed for at least 6 months postoperatively, and long-term outcome was evaluated. Patient demographics, lesion characteristics (i.e., size, stage, location) were recorded. Operative notes revealed surgical techniques, findings, and complications. Preoperative, short-/long-term postoperative facial nerve function was standardized using the House-Brackmann Classification. Mapping was done prior to incision by percutaneously stimulating the facial nerve and its branches and recording the motor responses. Intraoperative monitoring and mapping were accomplished using a four-channel, free-running EMG. Neurophysiologists continuously monitored EMG responses and blindly analyzed intraoperative findings and final EMG interpretations for abnormalities. Results Seven patients collectively underwent 8 Lymphatic Malformation surgeries. Median age was 30 months (2–105 months). Lymphatic Malformation diagnosis was recorded in 6/8 surgeries. Facial nerve function was House-Brackmann grade I in 8/8 cases preoperatively. Facial nerve was abnormally elongated in 1/8 cases. EMG monitoring recorded abnormal activity in 4/8 cases—two suggesting facial nerve irritation, and two with possible facial nerve damage. Transient or long-term facial nerve paresis occurred in 1/8 cases (House-Brackmann grade II). Conclusions Preoperative facial nerve mapping combined with continuous intraoperative EMG and mapping is a successful method of identifying the facial nerve course and protecting it from injury during resection of cervicofacial Lymphatic Malformations involving the facial nerve.

  • facial nerve anatomy dissection and preservation in Lymphatic Malformation management
    International Journal of Pediatric Otorhinolaryngology, 2008
    Co-Authors: Gi Soo Lee, Scott C Manning, Jonathan A Perkins, Sepehr Oliaei
    Abstract:

    Summary Objective To describe facial nerve anatomy and surgical techniques used for safe Lymphatic Malformation resection of Malformation involving the facial nerve. Methods Design: retrospective case series. Setting: tertiary pediatric hospital. Subjects: record review of Lymphatic Malformation patients after facial nerve dissection, from 1996 to 2005. Data collected included: facial nerve function, relationship of Lymphatic Malformation to facial nerve, facial nerve anatomy, dissection extent and clinical outcome. Results Sixteen patients who met inclusion criteria underwent a total of 21 facial nerve dissections. Mean age at dissection was 48 months (range 1–72 months). Mean follow-up was 38 months (range 8–144 months). Pre-operative Lymphatic Malformation stage by patient: II = 7/16, III = 4/16, IV = 2/16 and V = 3/16. Higher stage Lymphatic Malformations required more extensive dissections (p = 0.026). Pre-operative facial nerve function was House-Brackmann grade (HBG)-1 in 20, and HBG-6 in 1. Eight months postoperatively, facial nerve function was HBG-1 in 18, HBG-2 in 1, and HBG-6 in 2. The facial nerve was surrounded by Lymphatic Malformation in 10/21, deep to the Lymphatic Malformation in 5/21, superficial to the Lymphatic Malformation in 4/21, and not identified in 2/21. Imaging studies predicted facial nerve position in 15/21 procedures. Antegrade nerve dissection was performed in 10/21, retrograde in 7/21 and not done in 2/21. Abnormally elongated facial nerve was identified in 11/21 cases and required more extensive dissection (p = 0.040). Facial nerve monitoring was used in 15/21 dissections. Clinical outcomes were felt to be good in 19/21 dissections. Conclusions In Lymphatic Malformation surgery, the facial nerve is often abnormally elongated and encompassed by Malformation. Pre-operative imaging, facial nerve identification and dissection allow excellent postoperative facial nerve function.

Pahwa Manish - One of the best experts on this subject based on the ideXlab platform.

  • microcystic Lymphatic Malformation lymphangioma circumscriptum treated using a minimally invasive technique of radiofrequency ablation and sclerotherapy
    Dermatologic Surgery, 2010
    Co-Authors: Khunger Niti, Pahwa Manish
    Abstract:

    BACKGROUNDTherapeutic interventions for microcystic Lymphatic Malformation (MLM), including surgical resection, have historically been difficult and frustrating because of a high recurrence rate due to multifocal lesions and failure to address the deeper component and excessive morbidity associated

  • microcystic Lymphatic Malformation lymphangioma circumscriptum treated using a minimally invasive technique of radiofrequency ablation and sclerotherapy
    Dermatologic Surgery, 2010
    Co-Authors: Khunger Niti, Pahwa Manish
    Abstract:

    BACKGROUND Therapeutic interventions for microcystic Lymphatic Malformation (MLM), including surgical resection, have historically been difficult and frustrating because of a high recurrence rate due to multifocal lesions and failure to address the deeper component and excessive morbidity associated with excision of extensive lesions. MATERIALS AND METHODS This study represents a retrospective analysis of response to treatment of 14 consecutive patients with MLM. The first four patients were treated with sclerotherapy alone using 3% polidocanol. The next 10 patients were treated with the combination technique of radiofrequency ablation (RFA) and sclerotherapy. The sessions were repeated at monthly intervals until complete clearance. RESULTS Nine of 10 patients who were treated with the combination technique achieved near-complete clearance. There were minimal intra- and postoperative sequelae. The patients treated with the combination technique also achieved faster results. CONCLUSION RFA ablates the lesions and achieves hemostasis while the sclerosant injected in and around the lesion reaches the deeper vascular lesions, preventing recurrence. The minimally invasive combination technique of RFA with sclerotherapy is a modality that can be used in the treatment of extensive microcystic Malformations where surgical excision would lead to extensive scarring.

Reza Rahbar - One of the best experts on this subject based on the ideXlab platform.

  • Lymphatic Malformation of the Lingual Base and Oral Floor
    2010
    Co-Authors: Paul D. Edwards, Patricia E Burrows, Reza Rahbar, Nalton F. Ferraro, John B Mulliken
    Abstract:

    the tongue and floor of the mouth is associated with chronic airway problems, recurrent infection, and functional issues related to speech, oral hygiene, and malocclusion. There are no accepted anatomic guidelines or treatment protocols. Methods: This retrospective review focused on anatomic extent, treatment, complications, and airway management in 31 patients with Lymphatic Malformation of the lingual base and oral floor. Results: Involved adjacent structures included the neck (77 percent), mandible (41 percent), face (42 percent), lips (10 percent), pharynx (45 percent), and laryn

  • infantile myofibroma or Lymphatic Malformation differential diagnosis of neonatal cystic cervicofacial lesions
    Journal of Craniofacial Surgery, 2010
    Co-Authors: Stephen C Merrell, John B Mulliken, Ahmad I Alomari, Reza Rahbar, Horacio M Padua, Sara O Vargas, Ellis J Neufeld, Jennifer L Dearden, Arin K Greene
    Abstract:

    A 5-day-old male neonate was referred to our vascular anomalies center with a large cystic submandibular mass. History and physical examination and ultrasonographic results indicated the diagnosis to be macrocystic Lymphatic Malformation. Consequently, the child was treated with sclerotherapy and subtotal excision. The histopathological examination result showed that the lesion was infantile myofibroma. This diagnostic error was attributed to atypical features of infantile myofibroma in this child: unusually large cysts, rapid enlargement, and coagulopathy. This report expands the clinical spectrum of infantile myofibromatosis and suggests its consideration in the differential diagnosis of neonatal cystic cervicofacial lesions.

  • radiofrequency ablation of microcystic Lymphatic Malformation in the oral cavity
    Archives of Otolaryngology-head & Neck Surgery, 2006
    Co-Authors: Fredrik J Grimmer, John B Mulliken, Patricia E Burrows, Reza Rahbar
    Abstract:

    Objective To determine the efficacy and safety of radiofrequency (RF) ablation of vesicles and the resulting symptomatic control of microcystic Lymphatic Malformation (LM) in the oral cavity. Design An institutional review board–approved retrospective study with follow-up telephone interview. Setting Tertiary pediatric medical center. Patients Eleven children (6 girls and 5 boys), aged 4 to 16 years, presenting between August 1, 2002, and December 1, 2004. Intervention Radiofrequency ablation of LM in the oral cavity. Main Outcome Measures Symptoms related to LM, postoperative oral intake, and postoperative antibiotic requirements. Results Eleven patients presented with microcystic LM involving the lips, tongue, floor of the mouth, or buccal mucosa. Complaints included bleeding, infection, swelling, vesicle formation, and malocclusion. Patients underwent RF ablation (coblation) of oral cavity lesions. Seven (64%) of the 11 patients were able to tolerate oral intake in the recovery room. The need for antibiotics was reduced after RF ablation. All patients related diminished bleeding, pain, infection, or vesicle formation, with more than half reporting a significant improvement (6 patients) or complete resolution (1 patient). Five (62%) of 8 parents stated that the improvement after RF ablation was superior to that following previous procedures. Conclusions Subtotal RF ablation of LM appears to be safe, with early postoperative oral intake and minimal postoperative pain. Further studies are needed to determine long-term control of LM.

  • Lymphatic Malformation of the lingual base and oral floor
    Plastic and Reconstructive Surgery, 2005
    Co-Authors: Paul D. Edwards, Patricia E Burrows, Reza Rahbar, Nalton Ferraro, John B Mulliken
    Abstract:

    Background Lymphatic Malformation of the tongue and floor of the mouth is associated with chronic airway problems, recurrent infection, and functional issues related to speech, oral hygiene, and malocclusion. There are no accepted anatomic guidelines or treatment protocols. Methods This retrospective review focused on anatomic extent, treatment, complications, and airway management in 31 patients with Lymphatic Malformation of the lingual base and oral floor. Results Involved adjacent structures included the neck (77 percent), mandible (41 percent), face (42 percent), lips (10 percent), pharynx (45 percent), and larynx (26 percent). Fifty-eight percent of patients required tracheostomy during infancy; decannulation was possible in two-thirds of these patients. Management included resection alone (42 percent), resection and sclerotherapy (26 percent), resection and laser coagulation (16 percent), sclerotherapy and laser coagulation (16 percent), and resection and radiofrequency ablation (3 percent). Resection involved the neck (58 percent), floor of the mouth (52 percent), and tongue (42 percent); there were often multiple procedures. Aspiration was tried with little success in 10 percent of patients. Virtually all patients had residual abnormal Lymphatic tissue. Complications and posttherapeutic problems included infection (81 percent), neural damage (27 percent), difficulty in speech (23 percent), feeding problems (10 percent), and seroma or hematoma (6 percent). Associated dental/orthognathic conditions, particularly prognathism and anterior open bite, were documented in one-third of patients. Conclusions The initial step in the protocol is control of the neonatal airway. Staged cervical resection is undertaken in late infancy to early childhood; resection should also include abnormal tissue in the oral floor. Sclerotherapy is primarily for macrocystic disease or secondarily for recurrent cysts following partial extirpation. Vesicles of the mucous membranes and dorsal tongue are treated either by sclerotherapy, laser (carbon dioxide, yttrium-aluminum-garnet, or potassium-titanyl-phosphate), or radiofrequency ablation. Reduction for macroglossia is indicated for persistent protrusion or to allow correction of malocclusion. Embolization controls lingual bleeding. Orthognathic procedures are undertaken at the appropriate age, only after lingual size and position are acceptable.

  • management of a giant Lymphatic Malformation of the tongue
    Archives of Otolaryngology-head & Neck Surgery, 2002
    Co-Authors: Helena Rowley, Antonio R Perezatayde, Patricia E Burrows, Reza Rahbar
    Abstract:

    Lymphatic Malformations can occur anywhere in the head and neck, and when they do so in the oral cavity, they can present a potential hazard to the airway. We describe a 4-year-old girl with a giant Lymphatic Malformation of the tongue and neck. This report illustrates the particular difficulties that may be encountered in cases involving giant Lymphatic Malformations of the cervicofacial region in the pediatric population. An overview of the etiology, diagnosis, histology, and options for the management of such lesions is outlined.

Khunger Niti - One of the best experts on this subject based on the ideXlab platform.

  • microcystic Lymphatic Malformation lymphangioma circumscriptum treated using a minimally invasive technique of radiofrequency ablation and sclerotherapy
    Dermatologic Surgery, 2010
    Co-Authors: Khunger Niti, Pahwa Manish
    Abstract:

    BACKGROUNDTherapeutic interventions for microcystic Lymphatic Malformation (MLM), including surgical resection, have historically been difficult and frustrating because of a high recurrence rate due to multifocal lesions and failure to address the deeper component and excessive morbidity associated

  • microcystic Lymphatic Malformation lymphangioma circumscriptum treated using a minimally invasive technique of radiofrequency ablation and sclerotherapy
    Dermatologic Surgery, 2010
    Co-Authors: Khunger Niti, Pahwa Manish
    Abstract:

    BACKGROUND Therapeutic interventions for microcystic Lymphatic Malformation (MLM), including surgical resection, have historically been difficult and frustrating because of a high recurrence rate due to multifocal lesions and failure to address the deeper component and excessive morbidity associated with excision of extensive lesions. MATERIALS AND METHODS This study represents a retrospective analysis of response to treatment of 14 consecutive patients with MLM. The first four patients were treated with sclerotherapy alone using 3% polidocanol. The next 10 patients were treated with the combination technique of radiofrequency ablation (RFA) and sclerotherapy. The sessions were repeated at monthly intervals until complete clearance. RESULTS Nine of 10 patients who were treated with the combination technique achieved near-complete clearance. There were minimal intra- and postoperative sequelae. The patients treated with the combination technique also achieved faster results. CONCLUSION RFA ablates the lesions and achieves hemostasis while the sclerosant injected in and around the lesion reaches the deeper vascular lesions, preventing recurrence. The minimally invasive combination technique of RFA with sclerotherapy is a modality that can be used in the treatment of extensive microcystic Malformations where surgical excision would lead to extensive scarring.

Tommaso Lombardi - One of the best experts on this subject based on the ideXlab platform.

  • solitary superficial microcystic Lymphatic Malformation lymphangioma circumscriptum of the gingiva
    Journal of Periodontology, 2006
    Co-Authors: Christos D R Kalpidis, Stella Lysitsa, A Kolokotronis, Jacky Samson, Tommaso Lombardi
    Abstract:

    Background: Lymphatic Malformations are uncommon, hamartomatous, developmental aberrations of the Lymphatic system. The case presented in this report is a rare solitary gingival involvement of a microcystic-type Lymphatic Malformation. Methods: The lesion presented clinically as a small vesicular swelling of a buccal interdental papilla in a 16-year-old girl. Involved tissues were excised and submitted for routine histologic examination. The expression of the endothelial marker CD34 was investigated using immunohistochemical staining. Results: A physical examination failed to reveal similar or other abnormalities elsewhere in the body of the patient, including the oral cavity. Histopathologic analysis of the specimen demonstrated the presence of subepithelial, thin-walled, distended vascular cavities forming confluent vesicles containing lymph. The dilated Lymphatic formations were lined by flattened CD34-negative endothelial cells. These features were consistent with a microcystic gingival Lymphatic Malformation. To the best of our knowledge, only two additional reports of this Malformation have been published to date, but both presented with bilateral gingival involvement. Conclusion: Even though Lymphatic Malformations are encountered very infrequently on gingiva, they should be considered in the differential diagnosis of related conditions with a vesicular clinical appearance.