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Venous malformation that involved the soft palate, nasopharynx, and oropharynx. Note the significant airway narrowing. The patient presented with intermittent sleep apnea and occasional dysphagia. Axial postcontrast T1-weighted image (A) and coronal pre-(B) and post-contrast (C) T1-weighted images demonstrate avid and homogeneous enhancement of the venous malformation in this case.
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Mulliken and Glowacki's seminal classification of vascular anomalies into vascular tumors (with infantile hemangiomas being paradigmatic) versus nontumorous vascular malformations has been as important in the head and neck region as elsewhere. These latter are congenital, have an equal gender incidence, virtually always grow in size with the patien...
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Liquid sclerotherapy, laser and surgery have been used in the treatment of head and neck vascular anomalies with variable success for many years. A multidisciplinary team consisting of plastic surgery, maxillofacial surgery and interventional radiology currently treats such lesions by converting liquid sclerosant into foam. Foam sclerotherapy is cu...
Citations
... All these histological subtypes may coexist within the same lesion. FLMs are also classified as macrocystic, microcystic or mixed, depending on their size, with radiologically discernible lesions termed as macrocystic [8]. ...
Fetal lymphatic malformations (FLM) are benign hamartomatous lesions of the lymphatic vasculature usually localized in the cervicofacial and axillary regions and less frequently in the abdomen or inguinal areas, with an overall incidence of 1:6000 to 1:16,000 live births. FLM has a potential association with chromosomal aneuploidies such as Trisomy 21,18,13, Turner syndrome and genetic conditions such as Noonan syndrome, and with an excess risk for co-existing structural defects. A progressive increase in the dimensions of FLM's may result in polyhydramnios, hydrops, intrauterine fetal demise, airway obstruction and neonatal respiratory distress. We report a case of fetal orofacial lymphatic malformation detected in the mid-trimester anomaly scan with details of two- and three-dimensional imaging, prenatal counseling, cytogenetic workup, serial ultrasound surveillance, postnatal magnetic resonance imaging, and management with an intra-lesional sclerosing agent. The case depicts the strategic importance of multidisciplinary inputs in managing cases of prenatally diagnosed oral-cervical masses for optimizing perinatal outcome.
... Cystic hygromas is also associated with signs of localized infection, inflammation, and bleeding [4]. It is uncommon for lymphatic abnormalities to be cured with therapy [5,6]. Because lymphatic Original Research Article malformations can become entangled inside muscles or organs and because inadequate excision of lymphatic malformations can induce recurrence, surgery is not always an option [7,8]. ...
... For treating microcystic and mixed lesions, sclerosants such ethanol, doxycycline, bleomycin, and OK-432 are less effective. Although large cystic lesions may at first respond nicely to sclerotherapy, patients frequently require recurrent sessions throughout their lifespan [5,10]. The best approach for treating lymphatic abnormalities may be oral medicines. ...
Introduction: Cystic hygroma is a type of cyst that occurs when lymphatic fluid collects in the lymph nodes which occurs in infants and can cause significant swelling in the neck or other parts of the body. Sildenafil is a possible medication that can be used to treat erectile dysfunction in infant. Aim of the study: The aim of this study was to assess the effectiveness of oral sildenafil in the treatment of cystic hygroma in infant. Methods: This retrospective study was conducted from January 2020 to June 2022 in The Division of Pediatric Surgery, Bangladesh Shishu Hospital & Institute and three other private clinics at Dhaka. Total 55 patients (aged between 2 months to 1 year) with cystic hygroma were included in the study. Result: In the present study, the median (Range) age of 55 patients was 6.2 (2-12) months. The median (Range) weight of 55 patients was 6.5 (4-12) kg. In this study, majority (60%) of the patients were male. More than half (52.73%) patients had the lesion on neck. The median (Range) pretreatment size of lesion was 13.30 (9.88-16.93) cm2. The median pretreatment size of lesion was 13.30 cm2 and median post treatment size of lesion was 0.00 cm2. There was highly significant difference (p <0.001) between pre-treatment and post-treatment size. In this study, excellent response was found in 69.09% patients, good response was found in 21.82% patients. In the current study, 3 patients (5.45%) had cough, 4 patients (7.27%) had fever, 2 patients (3.64%) had rhinorrhoea and 3 patients (5.45%) had vomiting. In the current study, 12 (21.82%) patients had adverse effects. Cost of sildenafil per tablet was 34 cents (100 cents =1 $). Conclusion: Oral sildenafil resulted excellent size regression and low adverse effects for the treatment of cystic hygroma. It was also very cost effective and convenient to administer.
... Ultrasound can also help to confirm diagnosis of a VM with characteristic findings of hypoechoic spaces with septations and no flow on color Doppler. Phleboliths may be reported on ultrasound as hyperechoic with acoustic shadowing [13,14]. Other imaging studies such as MRI may demonstrate infiltration of surrounding structures, phleboliths, and an anomalous venous drainage system [13]. ...
Venous malformations (VMs) may manifest clinically in a broad spectrum. Most VMs are sporadic with previous studies reporting less than 1.2% to be inherited. Conversely, multifocal lesions, such as glomuvenous malformations (GVMs), which have glomus cells in their vascular walls, have been reported to have a frequency of inheritance of 63.8%. Both VMs and GVMs may occur due to sporadic mutation and must be differentiated clinically because this will dictate their proper treatment. Sporadic GVMs involve skin and subcutis, with bluish-purple coloration, are painful to compression, and have no radiographic evidence of phleboliths. Previous studies have demonstrated that VMs are almost always associated with a single lesion that is nontender to compression and are often able to be diagnosed by the presence of phleboliths on radiographic imaging. We present a case of a 14-year-old right-hand-dominant male who presented with two distinct VMs on the dorsum of the right index finger at the proximal and middle phalanges. A previously biopsied lesion overlying the ipsilateral olecranon, which was reported as a possible glomus tumor versus vascular malformation, was present as well. Based on history, physical examination, multicentric presentation, and radiographic findings, the presumptive diagnosis was that the lesions were GVMs. However, after surgical excision and histopathologic examination, the lesions were determined to be VMs because of the absence of glomus cells. Due to the difference in treatment modalities for VMs and GVMs, the ability to accurately diagnose these lesions clinically is essential. This case represents an anomalous presentation of multiple venous malformations occurring in two distinct locations in a 14-year-old boy.
... It acts by precipitating the endothelial proteins thereby inducing rapid thrombosis. 13 Absolute ethanol injections are very painful and hence require general anaesthesia. 14 Another serious drawback with ethanol is the risk of various complications during the procedure including ischaemic necrosis of skin, haemoglobinuria, anaphylaxis 15 and other complications like blood loss, acute pulmonary hypertension with cardio-pulmonary collapse. ...
Background:
Vascular malformations of the head and neck are common. The management of these lesions is complex and challenging due to the high complication rate and recurrence following treatment. Palatal vascular malformations (PVMs) are infrequent and present as slow growing lesions in the palate with recurrent bleeding and pain. These lesions are best managed by sclerotherapy due to their posterior location and risk of bleeding if surgery is attempted. Many sclerosants have been used for treating PVMs but the use of intralesional bleomycin for these lesions has not been reported at length. This paper describes the use of intralesional bleomycin injections for the treatment of deep-seated palatal vascular malformations.
Methods:
Intralesional bleomycin injections were given directly into the lesion with the patients under short general anaesthesia. The total dose of bleomycin ranged between 8 and 15 IU, which depends upon the body weight and was repeated every four weeks till the resolution of lesion was observed.
Results:
All the lesions in 12 patients regressed significantly with serial bleomycin injections. Clinically, the involved palatal mucosa became normal and magnetic resonance imaging demonstrated the significant regression of the lesion in all the cases. No complications were encountered with the use of intralesional bleomycin.
Conclusions:
Intralesional bleomycin injections have proved to be an emerging modality in the management of remotely situated palatal vascular malformations. Their rapid regressive effect on the lesion coupled with a high safety margin makes bleomycin sclerotherapy the first choice of treatment for palatal vascular malformations.
... Later, clinicians noted that bleomycin also has a potent sclerosing effect and leads to less severe local adverse events such as swelling, necrosis, and nerve injury compared with other sclerosing agents. [5][6][7] At present, bleomycin (and its derivatives) is one of the most widely used sclerosing agents for slowflow vascular malformations (venous and lymphatic malformations). 8 However, its effectiveness has been limited, with approximately one half of patients reporting improvement in overall health status after bleomycin sclerotherapy for slow-flow vascular malformations, in particular, in the areas of pain and the severity of symptoms. ...
Objective
Bleomycin is one of the most commonly used agents in sclerotherapy of slow-flow vascular malformations worldwide, but its efficiency remains unknown. The objective of this study was to assess whether reversible electroporation in combination with Bleomycin increases the sclerotherapy effect in previously unsuccessfully treated patients with venous malformations (VM).
Methods
First-time retrospective observational case series from 01/2019 to 01/2020 in seventeen patients (20 lesions) with symptomatic VM, who had previously undergone at least two unsuccessful invasive treatments. Reversible electroporation was performed with variable electrodes and directly injected Bleomycin. All patient records, MR imaging data, documentations of previous treatments, data regarding the intervention, complications and clinical symptoms were analysed.
Results
Seventeen VM patients (mean age 20.8 years; 9 females) who previously had 4.2 invasive treatments on average were treated with 22 electrosclerotherapy sessions of 20 lesions. The median dose of Bleomycin was 3 mg. The median value of MRI derived lesion volumes before treatment of 24.9 cm³ decreased by 86% to 3.5 cm³ post treatment. After 3.7 months, eight patients were asymptomatic without residual symptoms and nine patients showed improvement.
Conclusions
Bleomycin electrosclerotherapy appears to be an effective therapy for patients with VMs resistive to previous invasive therapies. Due to the small sample size and short follow-up period, our results should be further examined using a larger patient population.
... The trunk region accounts for only 20%. [2] They can affect any tissue and may involve multiple tissue types or organs at the same time. They may not confine themselves to anatomical planes. ...
... Large or deep LMs are assessed by MRI to: (1) confirm the diagnosis; (2) define the extent of the malformation; (3) plan the treatment. LM appears as either a macrocystic, microcystic or combined lesion with septations of variable thickness (8,9) . Histological confirmation of LM is rarely necessary (10) . ...
... Sclerotherapy is first-line management for large or problematic macrocystic/combined LM and its preferred due to lower complications rate than attempted resection (11) . Several sclerosing agents are used to shrink LM likes doxycycline, sodiumtetradecyl sulfate (STS), ethanol, bleomycin, and OK-432 (9,12) . ...
Abstract:
Objectives: The purpose of this paper was to discuss and evaluate the treatment plan selection and the outcomes of 82 cases
of lymphatic malformation in oral & maxillofacial region.
Materials & methods: The analysis included of 82 cases of lymphatic malformation in oral & maxillofacial region during the
period between January 2004 to November 2013 at maxillofacial department in Al-Hilla General Teaching Hospital. The
treatment plans selection depend on details patient history, clinical examination & imaging investigations. Different
techniques were used depended on age, extension, site & types of lymphatic malformation. The treatment methods were
conservative treatment, surgery, use of sclerosing agents or combinations of them.
Results: Total number of the patients were 82 complains from different types lymphatic malformation, 30 were males
constituting 36.5% while 52 were females constituting 63.5% . The youngest patient was 5 days, while the oldest one was 45
years. Forty case treated by sclerotherapy and surgery (48.7%) . Surgery alone as primary treatment done for 27 patients
(32.9%) other 10 cases treated by sclerotherapy alone ( 12.1%) & 5 cases ( 6% ) only needs observation .
Conclusions: Careful treatment plan selection depends on age, extension, type of lesion & experience of surgeon associated
with good prognosis. Conservative resection was the most effective method in treatment of lymphatic malformation.
Keywords: Vascular malformation, lymphangioma, sclerosing agent.
... Bleomycin is a sclerosing agent used for vascular malformations [12,15], that was originally discovered for its cytotoxic and antibiotic properties [16][17][18]. As clinicians noticed that bleomycin also has a potent sclerosing effect, and may lead to less severe local adverse events (e.g., swelling, nerve injury) than other sclerosing agents [19][20][21], it is now one of the most frequently used sclerosing agents for vascular malformations [8]. Previously published observational studies show that bleomycin can reduce the size of both LMs and VMs [12,15]. ...
Purpose:
There is paucity of data on patient-perceived outcomes of bleomycin sclerotherapy for low-flow vascular malformations. In this study, the long-term outcomes of bleomycin sclerotherapy were investigated in terms of quality of life (QoL) and patient-perceived changes in health.
Materials and methods:
A cohort of Dutch patients with vascular malformations treated with bleomycin sclerotherapy (June 2010-November 2015) completed a questionnaire evaluating disease symptoms, QoL (Short Form 36), patient-perceived change in health status (Global Rating of Change scales) and treatment satisfaction. QoL was assessed for the patient's status before and after treatment and was analyzed relative to an age and sex-matched Dutch reference population. Predictive factors associated with QoL and patient-perceived improvement in overall health status were assessed using multivariable linear and logistic regression analyses, respectively.
Results:
Seventy-seven patients, with a median follow-up of 22 months, were enrolled. About half of the respondents (49.3%) indicated that they perceived (any form of) improvement in their overall health status. Most often improved were the specific health aspects 'pain' (54.5%) and 'overall severity of symptoms' (57.1%). No factors were significantly predictive for patient-perceived improvement in health with respect to the vascular malformation. Impairment in work- or study-related activities prior to sclerotherapy was found to negatively impact physical QoL at follow-up (p = 0.03).
Conclusion:
Approximately half of patients with low-flow vascular malformations indicate an improvement in overall health status following bleomycin sclerotherapy, particularly concerning pain and severity of symptoms. However, most patients only perceived little to moderate improvement to their health and desire further treatment.
... Growth is most pronounced during puberty and pregnancy (1,(7)(8)(9)2). Venous malformations have no gender predilection, and the age at presentation is typically during early adulthood, though this is largely dependent on the location, size, mass effect on adjacent structures, and associated symptoms (11). ...
... The slow-flow hemodynamics and abnormal venous architecture of venous malformations often triggers thrombosis. Recurrent induction of the coagulation cascade depletes coagulation factors and increases fibrinolysis (11,23,24). Continual thrombus turnover within venous malformations may lead to localized intravascular coagulopathy, characterized by decreased plasma fibrinogen, factor V, factor VIII, factor XIII, and increased D-dimer (25). ...
... CT is typically reserved for diagnosis, surgical planning and follow up of bony venous malformations (8,2,14). Other cross-sectional imaging modalities such as magnetic resonance imaging is preferred over CT for characterizing venous malformations in the soft-tissues (2,7,8,11,14). Venous malformations on non-contrast CT appear as homogenously isodense unless it involves adipose tissue where it may appear heterogeneous (2). ...
Venous malformations are the most common type of congenital vascular malformation. The diagnosis and management of venous malformations may be challenging, as venous malformations may be located anywhere in the body and range from small and superficial to large and extensive lesions. There are many treatment options for venous malformations including systemic targeted drugs, open surgery, sclerotherapy, cryoablation, and laser photocoagulation. This manuscript reviews the natural history, clinical evaluation, imaging diagnosis, and treatment modalities of venous malformations.
... Large or deep LMs are assessed by MRI in order to confirm the diagnosis, to define the extent of the malformation, and to plan the treatment. The LMs are hyperintense on T2-weighted sequences and do not show diffuse enhancement [8]. The lesions are further subdivided into macrocystic and microcystic based on the cystic spaces. ...
Lymphatic malformations are rare slow-flow vascular malformations, with high tendency to appear in the head and neck region. The treatment of these lesions ranges from follow-up to sclerosing agent injection to surgical excision. The authors present a case of a new born with large extensive lingual and submandibular lymphatic malformation, for which the patient underwent tracheostomy and gastrostomy insertion. He was then treated successfully with sclerosing agent injections followed by surgical excision, with 7 years follow-up. The second case presented is a two and a half baby with large lingual lymphatic malformation, treated successfully with doxycycline injections followed by intraoral excision of the lesion. Pathology of the excised lesion is then demonstrated, which shows for the first time the different layers affected by the sclerosing agent.
