Extensive Hemangioma Research Articles

THE USE OF CRYOSURGERY IN THE TREATMENT OF EXTENSIVE LOWER LIP HEMANGIOMA

Hemangioma is a rapidly growing benign neoplasm that can cause both esthetic and functional impairment. It is relatively rare in the oral cavity and its most frequent site is the lower lip. Treatment mainly depends on the correct diagnosis and anatomical location. This report describes a clinical case of extensive lip hemangioma treated with cryosurgery. A 42-year-old Caucasian male presented a congenital hemangioma of the lower lip, which had been growing over the years. The patient was treated with 12 sessions of cryosurgery (5 cycles/session) with a significant reduction in the lesion. This minimally invasive approach is more comfortable and involves a lower risk of bleeding when compared to conventional surgery. In conclusion, cryosurgery is a viable option for the treatment of extensive hemangioma of the lip. Hemangioma is a rapidly growing benign neoplasm that can cause both esthetic and functional impairment. It is relatively rare in the oral cavity and its most frequent site is the lower lip. Treatment mainly depends on the correct diagnosis and anatomical location. This report describes a clinical case of extensive lip hemangioma treated with cryosurgery. A 42-year-old Caucasian male presented a congenital hemangioma of the lower lip, which had been growing over the years. The patient was treated with 12 sessions of cryosurgery (5 cycles/session) with a significant reduction in the lesion. This minimally invasive approach is more comfortable and involves a lower risk of bleeding when compared to conventional surgery. In conclusion, cryosurgery is a viable option for the treatment of extensive hemangioma of the lip.

МЕЖДИСЦИПЛИНАРНЫЙ ПОДХОД К ЛЕЧЕНИЮ МЛАДЕНЧЕСКИХ ГЕМАНГИОМ

The aim of the study is to find the best approaches to the treatment of infantile hemangiomas (IH). Materials and methods of research: the experience of treatment of pediatric patients with IH of various localization is presented, which was carried out according to the protocol elaborated by the authors. Systemic therapy with propranolol, local therapy, laser therapy were used in the complex of treatment. The age of patients at the beginning of therapy ranged from 1 month up to 4,5 years. Results: in all cases, patients with propranolol treatment showed a quick and stable effect. Even at the time of the selection of the dose of the drug, IH patients began to involute, became paler, less tense. The thickness of the soft tissues in the IH area decreased by 65% compared to the baseline, the number of functioning vessels decreased by 86%. The maximum effect was achieved within the first 6 months from the start of therapy. Clinical case report – a 2,5-year-old child with extensive hemangioma of the right buttock. There was a complex treatment with propranolol, local therapy, laser therapy. The general course of treatment with propranolol was 2 years 8 months. The total number of laser interventions was 6 with the overall effect in the form of maximum regression of residual clinical manifestations of IH. A positive clinical and ultrasound picture was noted, indicating a pronounced involution of IH, and a gradual withdrawal of propranolol was started. Conclusion: the effectiveness of a comprehensive interdisciplinary approach to the treatment of extensive complicated IH was demonstrated with the participation of specialists of various disciplines: pediatricians, cardiologists, surgeons, specialists in functional and ultrasound diagnostics, specialists in laser therapy, endocrinologists in a severe premature baby with a large number of somatic problems. The clinical treatment algorithm developed and implemented for patients with IH makes it possible to avoid more aggressive methods of treatment of this pathology and to improve the quality of life of this category of patients. On the basis on the results obtained, indications for laser therapy with a selective pulsed laser were formulated.

Nd:YAG laser in association with pulsed dye laser for the treatment of PHACES syndrome.

The acronym PHACES stands for posterior fossa malformations, hemangiomas, arterial anomalies (cardiovascular or cerebrovascular), coarctation of the aorta/cardiac defects, eye abnormalities, and sternal defects. The characteristic dermatological clinical manifestation of PHACES syndrome is a segmental and extensive hemangioma, usually on the face. A combined therapy with 1,064 nm Nd-YAG/595-nm pulsed dye laser was performed in a young 15-year-old patient with PHACES syndrome, who presented a hemangioma on the left side of the face, located in the periorbital region. A first session with Nd-YAG laser (2,5 mm spot size, fluence 100 J/cm2, pulse duration 7 ms) for the treatment of teleangectasias and subsequently, three treatment sessions with pulsed dye laser (12 mm spot size, fluence 7 J/cm2, pulse duration 0,5 ms, repetition rate 0,6 Hz), once every 2 months, were performed. No postoperative complications were recorded, except for transient purpura after the pulsed dye laser sessions. The vascular lesion had a decrease in size bigger than 75%, and these results was maintained 6 months after the last treatment. Combined therapy Nd- YAG/pulsed dye laser is an effective and noninvasive procedure for hemangiomas in patients with PHACES syndrome.

Individualized approach for Coarctation of the Aorta in a Patient with PHACE syndrome and Extensive Hemangiomas

PHACE syndrome is a neurocutaneous disorder with large facial segmental hemangiomas associated with anomalies of the brain, eye and heart. We report a case of a 3 years old girl who presented at the age of 6 weeks with severe coarctation of the aorta and aortic arch aneurysm. In addition, she had extensive strawberry hemangiomas over the scalp, neck and chest which, after extensive investigation, led to the diagnosis of PHACE syndrome. After complete evaluation we successfully initiated treatment with propranolol that was pursued without complications over three years with striking success and remarkable regression of her hemangiomas. Meanwhile the coarctation was managed conservatively. This case highlights that propranolol treatment, while effective against hemangiomas, may contribute to slow the progression of aortic arch aneurysm and delay the need for high risk surgical intervention.

Application of internal and external lysis combined with Ilizarov external fixation technology for severe knee pathological flexion contracture deformity in children

To investigate the effectiveness of internal and external lysis combined with Ilizarov external fixation technology for severe knee pathological flexion contracture deformity in children. A retrospective analysis was made on 12 children (12 knees) with severe knee pathological flexion contracture deformity who were treated with internal and external lysis and Ilizarov external fixation between August 2012 and January 2017. There were 9 boys and 3 girls with an age of 3-12 years (mean, 8.4 years). There were 8 cases of tuberculosis, 3 cases of haemophilia A, and 1 case of residual deformity after extensive hemangioma drug injection. The disease duration ranged from 5 months to 4 years, with an average of 20.3 months. The degree of knee contracture was (67.42±23.30)°, and the range of motion of knee was (38.33±14.98)°. The preoperative Western Ontario and McMaster University Osteoarthritis Index (WOMAC) score was 78.42±15.57. The complication was observed after operation, and the degree of knee contracture, range of motion, and WOMAC score at 3 months and 1 year after operation were recorded and compared with those before operation. The operations completed successfully in all children. All the 12 cases were followed up 9-24 months (mean, 14.5 months). All incisions healed by first intention after operation. The knee function of all children improved significantly and the weight-bearing walking function of the lower limbs restored. The degree of knee contracture, range of motion, and WOMAC score were significantly improved at 3 months and 1 year after operation (P<0.05), but there was no significant difference between 3 months and 1 year after operation (P>0.05). For severe knee pathological flexion contracture deformity in children, application of internal and external lysis combined with Ilizarov external fixation has advantages, such as small trauma, rapid recovery, and early postoperative knee function training, and good effectiveness.

LIP HEMANGIOMA IN A YOUNG PATIENT: AN AESTHETIC CHALLENGE MANAGED BY AN ASSOCIATION OF TREATMENT TECHNIQUES

Treatment of benign lip vascular anomalies can be an aesthetic challenge even though several techniques are available to manage these lesions. A difficult case treated with an association of techniques is presented. A 12-year-old boy with an extensive congenital hemangioma in the upper lip was referred with an aesthetic complaint and past history of treatment failures. Sclerotherapy with 5% ethanolamine oleate was proposed. Intriguingly, after 6 sessions of sclerotherapy, the size and the consistency of the lesion remained almost the same, although some color improvement was observed. Therefore, an aesthetic surgery was considered and the patient was referred to a vascular surgeon. After arteriography, 3 sessions of percutaneous embolization with 3% polidocanol foam was performed. Afterward, the patient underwent 2 surgical interventions using diode high-potential laser achieving a satisfactory result. Although uncommon, a refractory vascular lesion may occur and an association of different techniques may be necessary.

Endoscopic management of intranasal meningoceles and meningoencephaloceles

Objective: Determine the efficacy of the anterior skull base reconstruction in adult patients with intranasal meningoceles and meningoencephaloceles treated by endoscopic endonasal approach. Study design: Retrospective observational study of adult patients admitted to a tertiary hospital. Materials and methods: Intranasal meningocele was defined as a protrusion of meninges through a defect in the skull base forming a cyst filled with cerebrospinal fluid in the nasal cavity or paranasal sinuses. If the cyst had brain tissue it was called meningoencephalocele. The electronic clinical records of patients admitted with suspected of intranasal MC or MEC between January 2010 and December 2018 were reviewed. All patients treated by endoscopic endonasal approach were included. We excluded those cases with anterior skull base previous surgeries (iatrogenic or following tumoral resections), reconstruction with external surgical approaches and those who were less than 18 years old. Results: Intranasal MC was the most frequent lesion (6/5) and there was one patient with MEC (1/6). 5 cases were idiopathic and one patient had history of transnasal surgery. A total of 83,3% of the cases were primary surgeries and one patient had 3 prior MC surgeries. Only in four of them (66,6%) the lesion location was detected by at least one of the studies (CT, MRI and/or endoscopy). We performed a multilayer closure technique in all patients. During the immediate postoperative period, one patient had acute meningitis. One patient had a CSF leak recurrence 4 months after surgery. The success rate of the reconstructions performed by endonasal approach was 83.33% (5/6). The average follow-up of the patients was 15 months. Conclusion: The effectiveness of the anterior skull base reconstruction in adult patients with meningoceles and meningoencephaloceles performed by endoscopic endonasal approach in our series was 83.33% (5/6). This procedure is currently the gold standard due to its high efficacy and low morbidity. According to current studies, it is recommended to perform the reconstruction with a multilayer technique.

Experience in the Treatment of Hemangioma in Children

The article reflects modern views on epidemiology, clinical manifestations, classification, principles of diagnostics and treatment of hemangiomas. The purpose of our study was the analysis of the results of different methods of treatment inof children with hemangioma over a ten-year period (from 2007 to 2017) at Irkutsk Regional Children Hospital. We have found a significant reduction in cases of invasive treatment in patients with this pathology. In 2017, inhemangioma was excised in 179 children, and in 2017, this method was used only in 28 patients. Since 2009 cryolysis of vascular neoplasms has not been performedcryolysis. Conservative treatment of hemangiomas include hormone therapy with prednisone as well as systemic and local treatment with beta-adrenergic blocking agents. At the same time, we showed that conservative treatment of hemangiomas is effective before surgery. That is confirmed by the clinical observation of the child with an extensive perineal hemangioma, who underwent the combined treatment using surgical cryodestruction and hormone therapy. This example shows that a good aesthetic result has been obtained only when using hormone therapy, while partial excision of hemangiomas resulted in a violation of the anatomy of the external genitalia, and cryodestruction resulted in an extensive hypertrophic scar on both buttocks. It is concluded that the reasons for the change in treatment tactics in children with hemangiomas are determined by the possibility of using non-invasive methods with obtaining the optimal result of treatment in both oncological and aesthetic terms.

Endoscopic surgery in hemangiomas of paranasal sinuses and nasal cavity

Objectives: To determine the possibility of performing complete resections of rhinosinusal hemangiomas greater than 2cm treated by an endonasal approach assisted with endoscopes. Study design: Descriptive, retrospective Methods: We analyzed the clinical histories of all adult patients who underwent an endonasal approach with endoscopes, for having hemangiomas of the nasal cavity and paranasal sinuses in the Italian Hospital of Buenos Aires, between June 2006 and December 2016. We excluded patients with hemangiomas who were less than 18 years old, with small tumors (less than 2 cm), vascular malformations, and those who underwent surgical procedures performed externally. The surgeries were performed under general anesthesia, with endoscopes of 0° and 30°, conventional surgical instruments were used for rhinosinusal endoscopic surgery. The follow-up was performed by nasal endoscopy, computed tomography and/or magnetic resonance with contrast. Results: Four patients were treated for nasal cavity (1/4) and paranasal sinuses (3/4) hemangiomas through an endonasal approach with endoscopes. There were no complications. The follow-up was greater than 5 years in two, 2 years in one and 1 year in another patient. No recurrences were detected. Conclusión: Trans-nasal surgery assisted with endoscopes of paranasal sinus and nasal cavity hemangiomas greater than 2 cm in adults, allowed perform complete resections in 100% of treated patients. In extensive hemangiomas and when it was not possible to detect the site of vascular tumor implantation, it was important to previously devascularize the lesion by preoperative embolization and cauterization of the vascular pedicles before resecting the tumor.

Evolution of surgical and therapeutic approaches in cases of extensive hemangioma in children

Evolution of surgical and therapeutic approaches in cases of extensive hemangioma in children

Можливості компресійної терапії при консервативному лікуванні поширених складних гемангіом у дітей

Можливості компресійної терапії при консервативному лікуванні поширених складних гемангіом у дітей

GE-01 * IDH1-MUTATED PITUITARY ADENOMA CHALLENGES TRADITIONAL CHARACTERIZATION OF MAFFUCCI SYNDROME

Maffucci syndrome is a rare, non-inheritable condition of mesodermal dysplasia, presenting in childhood with formation of multiple enchondromas and soft-tissue hemangiomas. Approximately, 40% of enchondromas undergo malignant transformation into chondrosarcomas. The underlying genetic etiology lies in somatic mosaicism of mutations in isocitrate dehydrogenase 1 (IDH1) or IDH2, which have been detected simultaneously in both tumors and blood leukocytes of affected patients. We describe a 7-year old male who presented with extensive enchondromatosis and subcutaneous hemangiomas, clinically diagnosed as Maffucci syndrome. Years later, he developed sub-acute dysphasia, dysarthria, and loss of left visual acuity. A brain MRI demonstrated two heterogeneously enhancing lesions, one centered in the left jugular foramen and the other located in the supra-sellar region. Surgical resections of both intracranial tumors were performed over a period of ten months. Histopathological review revealed diagnoses of a grade II chondrosarcoma as well as a non-functional pituitary adenoma after subsequent neuroendocrine studies were unremarkable. Genotypic studies of the tumors were performed to identify common IDH1/2 mutations. DNA sequencing utilized standard Sanger sequencing in conjunction with a target-specific peptide nucleic acid (PNA) to detect IDH1 mutations in tumor tissues. The results demonstrated identical IDH1 mutations (c.394C > T; R132C) in both tumors. This result represents the first genetic evidence to establish causality between pituitary adenoma formation and tumor predisposition in Maffucci syndrome. As neuro-ectodermal tissue, this IDH1-mutated pituitary adenoma challenges the notion that Maffucci syndrome only involves mesodermal tissues. As such, in pediatric patients newly diagnosed with Maffucci syndrome, it may be appropriate to monitor for development of pituitary pathology and neuroendocrine dysfunction. Conversely, in the rare sub-population of pediatric neuro-oncology patients with concomitant non-nervous system tumors, further investigation is warranted to rule out Maffucci syndrome, as there may be considerable risk for malignant transformation as well as additional tumor formation.

Klippel-Trenaunay syndrome presenting with acanthocytosis and splenic and retroperitoneal lymphangioma: a case report

IntroductionKlippel-Trenaunay syndrome is a rare congenital mesodermal abnormality characterized by bone and soft tissue hypertrophy, extensive hemangioma and venous abnormalities. We report the case of a patient with two additional rare clinical manifestations in the background of Klippel-Trenaunay syndrome, namely, acanthocytosis and splenic and retroperitoneal lymphangioma.Case presentationA 24-year-old Sri Lankan man from North Central Province in Sri Lanka presented to our general medical unit with symptomatic anaemia. He had been diagnosed with Klippel-Trenaunay syndrome at the age of six years, with hemihypertrophy of his right lower limb and strawberry naevi over both lower limbs. His blood film results were positive for acanthocytes, which accounted for more than 20% of the red blood cell population. He was also found to have extensive splenic lymphangiomas and a large retroperitoneal lymphangioma encasing the mesentric vessels in the right para-aortic region. An extensive battery of tests to identify a secondary cause for the acanthocytosis failed to show any positive results.ConclusionsRetroperitoneal lymphangioma has been reported in association with Klippel-Trenaunay syndrome once before, but an association with acanthocytosis has never been reported. Given the rarity of all three conditions this is not surprising. The cause of acanthocytosis in this setting is currently unresolved. It is plausible that this may be a primary association with Klippel-Trenaunay syndrome, as an alternative aetiology was not found.

A Creative Therapy in Treating Cavernous Hemangioma of Penis with Copper Wire

Cavernous hemangiomas of penis are rare benign lesions infrequently described in the literature. No completely satisfactory treatment has been found to correct the cosmetic deformities especially the extensive hemangiomas of corpus penis. In light of the promising application of copper wire/needle in vascular malformations, we began a clinical study to investigate the safety, feasibility, and cosmetic effect of copper wire therapy in treating cavernous hemangioma of penis. Seven patients ranging in age from 12 to 32 years with penile cavernous hemangiomas entered our study from 2005 to 2011. All patients received treatments with percutaneous copper wires. Perioperative data including mean operation time, estimated blood loss, length of copper wire retention, and length of hospital stay were analyzed. All possible complications were noted, and cosmetic result was evaluated. Patients were followed up after discharge from the hospital. All operations were successful, and no obvious complications were observed. The patients were satisfied with the aesthetic results. Follow-up time ranged from 1 to 5 years. Recurrence was discovered in a patient with the largest lesion of corpus penis 2 months after the treatment. Secondary procedure was carried out with the same technique, and no lesions were found later. The shortage of studies on this topic prevented us from defining a therapeutic reference standard. The results of our study confirmed that copper wire therapy was a simple, safe, and useful option for penile cavernous hemangioma.

A Rare Case of Extensive Cavernous Hemangioma of the Brainstem Combined with Multiple Pathological and Anatomical Abnormalities

We present the case of a 14-year-old Caucasian female, with intracranial hemorrhage due to an extensive brainstem cavernous hemangioma, asymptomatic until the date of her admission. Further investigation revealed multiple brain cavernous hemangiomas with two supratentorial hemangiomas demonstrating signs of past bleeding, a cervical vertebral dysplasia (Klippel-Feil type II) and nephrolithiasis, in addition to her history of polycystic ovaries and impaired glucose tolerance, all together presenting a novel clinical combination. We are describing the management of such a patient, according to our current knowledge, inquiring into the possible connections between the conditions detected, as well as, discussing the questions that arise from this rare case.

An unusual presentation of hemangiomatosis of the liver

Diffuse hemangiomatosis of the liver is a rare condition of infancy characterized by extensive hemangiomas in the liver. It is extremely rare in adults. The imaging findings are similar to hemangioma showing centripetal enhancement with stasis of contrast. We present a case of hemangiomatosis of the liver and spleen in a 52 year old male patient. Following splenectomy there was enhanced growth of the hepatic hemangiomatosis within a period of four months and died soon after due to liver failure. We could not find a similar rapid progression of hepatic hemangiomatosis in our literature search.

Klippel-Trenaunay syndrome and type 1 neurofibromatosis: A chance association?

Klippel-Trenaunay syndrome and neurofibroma type I (NF1) are rare disorders with cutaneous and neurological features and not reported to be associated together. We report an 11-year-old female child who presented with extensive skin lesions, giant hemangioma and left lower limb hypertrophy. Her father had features of NF1. We report this case because of its rarity and need for long-term follow-up.

Extensive Facial and Orbital Infantile Hemangiomas Associated with High Intraocular Pressure

High intraocular pressure is a rare ophthalmic condition associated with infantile hemangiomas that involves the orbit, eyelid, or both. Here, we describe a patient with extensive facial and orbital infantile hemangiomas associated with high intraocular pressure in the affected eye. The prompt management of this challenging condition is essential.

Aortic Coarctation in the Presence of Capillary Hemangioma

We report posterior mediastinal hemangioma in a 14-year-old girl patient with an extensive hemangioma encompassing the thoracic aorta, associated with complex coarctation. The masses were identified by contrast-enhanced computed tomography. An extra-anatomical bypass was made by grafting from the ascending to the distal descending aorta. Histological evaluation of the surgical specimens established the final diagnosis. Presence of mediastinal hemangiomas should be regarded as an important differential diagnosis of coarctation of aorta, although these are very rare tumors.

Propranolol for extensive hemangiomas of infancy: two case reports

Hemangiomas are the most common benign tumors of childhood. They show rapid growth, followed by a regression phase that culminates in the partial or total disappearance of the lesion. Therapeutic options should be evaluated for extensive cases. Systemic glucocorticoids are the therapy of choice; however, there are reports that propranolol offers better and faster results. We report two cases of large volume infantile hemangioma associated with functional limitation and aesthetic disfigurement, treated successfully with propranolol, a drug that comes as a therapeutic option providing satisfactory and maintained results, with few side effects.