Cervical Lymphangioma Research Articles

Gorham-Stout disease and multiple cervical lymphangiomas: case report.

Gorham-Stout disease is a rare condition characterized by extensive bone loss due to the proliferation of new vascular and lymphatic structures. It can occur in any bone and cause pathologic fractures with poor bone healing. Complications such as effusions and lymphangiomas can also develop. Gorham-Stout disease pathogenesis is still being studied, and treatment options are limited, but sirolimus has shown promise in stabilizing or reducing symptoms. We present a case of a 19-year-old male with Gorham-Stout disease, multiple cervical lymphangiomas, and several thoracic complications successfully treated with sirolimus. Rare lymphatic diseases should be considered as a potential cause in adult patients with bone involvement and multiple cystic lesions in the neck, axillary, or abdominal regions after excluding more common causes. The complexity of diagnosing Gorham-Stout disease should be emphasized.

Rare Yet Relevant: Adult Onset Cervical Lymphangioma - Our Experience and Comprehensive Review.

Cervical lymphangiomas are anomalies that arise from lymphatic vessels, characterized by gradual and painless growth. While predominantly located in the neck, they may also manifest in the axilla, mediastinum, groin, and abdominal organs. Cervical lymphangioma usually occurs in children and is relatively rare in adults. The purpose of this study was to investigate the characteristics of cervical lymphangiomas in adults. We reviewed the medical records and analysed clinical data obtained from fifteen adult patients who suffered from cervical lymphangioma and were operated from January 2021 to January 2023. Out of 15 cases, 73.3% were females and 26.7% were males. All tumours were unilaterally located. Five patients had prior history of associated dyspnoea / dysphagia. All 15 cervical lymphangiomas (100%) were adjacent to the carotid sheath .Tumour size ranged from 6cm to 15cm, with the maximum diameter of ≥ 15cm in two cases, between 6cm and 10cm in eight cases and less than 6cm in five cases. Further, in this study all cervical lymphangiomas were surgically excised. Postoperative courses were uneventful, and histo-pathological examinations confirmed all cases to be cystic lymphangiomas. A 6 -month follow-up in all 15 cases showed no recurrences. Overall, this study underscores the importance of a multidisciplinary approach involving clinicians, radiologists, and surgeons in the management of adult cervical lymphangiomas, with an emphasis on accurate diagnosis, appropriate classification, and tailored treatment strategies to optimize patient outcomes and minimize complications.

Giant Cervical Lymphangioma Extending from Skull Base to Clavicle- A Rare Case.

Lymphangiomas are congenital benign lesions commonly seen in pediatric age group with a rare occurrence in the region of head and neck, commonly presenting as asymptomatic masses. Cervical lymphangioma is a rare entity among adults, and giant sized lymphangiomas in this region have sparse mention in literature. This case report describes the rare presentation of a giant cervical lymphangioma and the challenges involved to treat such masses in the region of head and neck. We encountered a rare case of a 52 year old female who presented with a 10 × 5cm swelling in the right posterior triangle of neck swelling since 6 months extending from skull base up till clavicle. Pathological and radiological entities confirmed the lesion as a "giant cervical lymphangioma". Head and neck lymphangiomas are congenital lesions of benign origin. Lymphangiomas have been classified as macrocystic if larger than 2cm, and microcystic if less than 2cm. Our description of a macrocystic 10 × 6 cm lesion is hence definitely one of the largest cervical lymphangiomas encountered till date. It's a problematic and arduous plan of treatment for such masses in head and neck with a high chance of recurrence if incompletely removed.

Cervical Thymic Cyst - An Overlooked Entity

Thymic cysts are one of the rare causes of neck masses in the paediatric age group. Patients may complain of neck swelling either central or lateral neck swelling. There is difficulty in diagnosing a cervical thymic cyst as it may mimic a branchial cleft cyst or a cervical lymphangioma. Definitive diagnosis of a thymic cyst requires histopathological examination of thymic tissue. We report a case of a 6-year-old girl who presented with painless lateral neck swelling for 2 months duration. Computed tomography (CT) scan showed a left branchial cyst with retropharyngeal extension. The patient underwent complete excision of the mass and histological examination was reported as a cervical thymic cyst. Postoperatively, she was able to be discharged after serial follow-ups with no signs and symptoms of recurrence.

Case Series of Cervical Lymphangioma with Different Presentation.

Cervical lymphangioma is a congenital malformation of the lymphatic system. They are most common in children between the ages of 3 and 5years and rarely present in adults. It is most often present in the posterior triangle of the neck. The treatment of choice is complete surgical resection, although other treatment alternatives are reported; the most significant being sclerotherapy. We have included six cases with large neck lymphangiomas in the period of 2020-2023. Two lympangioma were located on the left side of neck, one over the midline of neck, one at the right side of the neck, one in the right submandibular space and one at the right side parotid region. All were surgically resected sucessfully. No recurrence was seen in any cases.

Giant cervical lymphangioma encompassing the neck great vessels (carotid artery and jugular vein): a case report.

An 18-month-old child was brought to our teaching hospital's pediatric surgery department with a huge cervical mass on the right side since birth. Following the completion of work-up with the laboratory and imaging (computerized tomography scan) diagnostic modalities, the patient was prepared for definite treatment. Our team approached the mass through a right neck hockey stick incision, and with the preservation of the neurovascular bundle, the mass was completely excised. The patient was followed up for 12 months on two occasions, with excellent esthetic results and no relapse. Lymphangiomas confined to the posterior cervical triangle are a common problem in children. Lesions extending to the anterior of the neck, especially those involving the neck neurovascular bundle, are uncommon entities. The decision to proceed with sclerotherapy or surgical excision should be justified, provided that, during the surgical procedure, the neurovascular bundle is preserved and none of the vital organs (neurovascular components) are compensated with the goal of a complete mass excision.

A Rare Massive Congenital Cervical Lymphangioma: Case Report with Systematic Review of the Literature

A Rare Massive Congenital Cervical Lymphangioma: Case Report with Systematic Review of the Literature

154. Cervical lymphangioma in adulthood - a diagnostic dilemma

Lymphangioma is a vascular abnormality of the lymphatic system that normally arises at birth, 90% grow by 2 years of age. Only 150 cases of adult lymphangiomas are reported in English literature. Nearly 75% of all lymphangiomas arise in the head and neck region, typically in the posterior triangle of the neck.

Cervical Lymphangioma of the Cystic Hygroma Type in an Adult Patient: Case Report of a Rare Condition

Introduction: Cervical lymphangiomas are lymphatic malformations that occur rarely in adult patients, they are usually masses that have their diagnosis and resolution at early ages, their origin is produced by embryological defects during development, they are frequently located in the cervical region particularly in the posterior triangle. Computed tomography scan is the study of choice due to its great contribution to determine extension and relationship with neighboring structures. As for treatment, the indication is complete resection of the mass. The definitive diagnosis will be based on the histopathological study of the sample. Clinical case: We introduce the case of a 78-year-old patient, who comes to consultation due to a cervical mass of 1 year of evolution and progressive growth. A right cervical mass is evidenced on physical examination; complementary tests are performed. Therefore, a complete resection of the mass is decided, procedure that is carried out without complications. In the histopathology study, a mass compatible with lymphangioma of the cystic hygroma type was reported. Conclusions: Cervical lymphangiomas are masses frequently found in children and exceptionally rare in adult patients, however, they should be included in the differential diagnosis of cervical masses. The treatment of choice is complete resection of the mass to avoid recurrence. In our case, complete resection of the cervical mass was achieved, without any complication and in subsequent controls no signs suggesting recurrence were observed.

Cervical Lymphangiomas in Children: Non-Surgical Treatment with Focus on Sclerotherapy. Literature Review

Objectives: To discuss the non-surgical strategies for the management of cervical lymphangiomas in children, with particular attention to sclerotherapy. Methods: A literature review of the last fifteen years about sclerotherapy of cervical lymphangiomas in children was performed and the main sclerosing agents were reported, highlighting the nature, the mechanism of action and the rates of success, recurrences and complications related to each and every substance; furthermore, the several steps of the procedure are illustrated. Alternative approaches are also mentioned. Results: 47 articles were collected, mostly dealing with sclerotherapy with OK-432, bleomycin/pingyangmycin and doxycycline; other potential substances include, above all, sodium tetradecyl-sulphate and ethanol. Laser therapy and radio-frequency ablation are valid options in case of mucosal micro-cystic lesions; oral medications (e.g. Sirolimus) and the newest target-therapies are added to local treatments in some Series.

Cystic hygroma in a young adult: A case report and recent management

We are reporting a case of a 27-year-old young female who presented with right side neck swelling without any associated obstructive symptoms and any other grave signs and symptoms. She noticed a gradual increase in the size of the swelling within a period of 2 years. After investigation and surgical excision, the swelling was diagnosed as cystic hygroma. The root cause of the development of cervical lymphangioma is the congenital malformation of the developing lymphatic system. Cystic hygroma is benign in nature and the cause in adults is still unclear. The most common site of origin is in head and neck region, and cystic hygroma accounts for 75% of lymphatic malformations. The most common presentation of cystic hygroma is painless swelling with ill-defined lesion, most commonly located at the posterior triangle of the neck. The common age group is between birth and 2 years of age, with very rare presentation in adults. Hence, it is necessary to rule out all differential diagnosis of cervical lymphangioma, which is presented with cystic neck swelling. Complete surgical excision is the recommended standard treatment.

Cervical lymphangioma in adults: A report of seven cases and review of the literature.

BackgroundCervical lymphangioma usually occurs in children and is relatively rare in adults. The purpose of this study was to investigate the characteristics of cervical lymphangiomas in adults.MethodsWe retrospectively reviewed the medical records and analyzed clinical data obtained from seven adult patients who suffered from cervical lymphangioma from January 2008 to June 2021.ResultsFive (71.43%) of seven cases were asymptomatic and four (57.14%) lymphangiomas had a maximum diameter ≥ 10 cm. Each cervical lymphangioma was located adjacent to the carotid sheath. All the cervical lymphangiomas were completely surgically excised and were pathologically diagnosed as cystic lymphangioma. After a median 32 months follow‐up period, no recurrence was observed.ConclusionCervical lymphangiomas from adults are often large asymptomatic masses infiltrating or adjacent to surrounding vital structures which brings many difficulties to the surgery, requiring an experienced surgeon to remove the mass.

Cervical lymphangioma with myxoid degeneration: a rare case report and literature review

BackgroundLymphangioma is a multi-systemic disease mostly affecting the mesentery, omentum, mesocolon and retroperitoneum and rarely involving bone, with fewer than 30 cases reported so far. Lymphangioma usually has no specific clinical manifestation and could be easily misdiagnosed. Lymphangioma with myxoid degeneration was not reported. We report a histopathologically proved case of lymphangioma of 6th and 7th cervical vertebra (C6–7) with myxoid degeneration.Case presentationA 45-year-old male who suffered from pain in the right shoulder for 1 month, aggravated with numbness in the right little finger and ring finger and radiating pain for half a month. CT showed multiple cystic bone destruction areas in C6–7 vertebral right part and appendage with marginal osteosclerosis and surrounded by soft tissue density. The size of the lesion was about 36 mm × 41 mm . MRI: the margin of the lesion appears lobulated. Lesion showed slightly hypointense on T1WI, slightly hyperintense on T2WI and STIR. Multiple patchy T1WI hypointense and T2WI hyperintense shadows were seen within the lesion. No enhancement was observed in the lesion after injection of contrast, and there was no abnormal signal in surrounding soft tissue and bone. The histopathological examination confirmed the diagnosis of lymphangioma with myxoid degeneration after surgical resection. After over 2 years of follow-up, there were no signs of disease recurrence and progression.ConclusionsTo the best of our knowledge, this is the first case reported in English language literature of lymphangioma with myxoid degeneration.

The Diagnosis as a Guide for a Life Trajectory: An Autobiographical Case Report

In surgical procedures in the pediatric population, the doctor-patient relationship becomes more complex, extending to the doctor-patient-family relationship. This case report presents my perspective as a pediatric patient with the diagnosis and surgical excision of a cervical lymphangioma and later a hemangioma, demonstrating the impacts and repercussions on my life trajectory. The quality of medical services depends on the relationship between professionals and the user. Thus, medical care can no longer be organized considering only the effectiveness. It is the doctor's responsibility to understand and manage his attitudes so that all patients have individualized care worthy of a life trajectory with resilience. By doing this, who knows, maybe we are generating a great stream of future doctors who can better understand their patients?

A Long-term Follow-up of Mandibular Deviation Caused by Congenital Cervical Lymphangioma Treated with an Orthodontic Approach

To report a treatment case of mandibular deviation caused by congenital cervical lymphangioma with traditional orthodontic techniques, following-up by 10-year retention. Lymphangiomas, developmental anomalies, can induce various disturbances of swallowing, mastication, speech, breathing, and skeletal deformities as well as psychological stress and anxiety for the patient and their family. Lymphangiomas are benign with virtually no possibility of turning into a malignant lesion, so clinical management aims to treat the patient functionally. A girl, aged 6 years and 4 months, complained about facial asymmetry and anterior crossbite caused by congenital cervical lymphangioma. Her facial profile was the straight type with an adequate lip position. Anterior and right-side posterior crossbites were observed. On the frontal cephalogram, the menton shifted 3.0 mm to the right. A functional appliance with an expander was placed to correct her dental midline deviation and posterior crossbite. After 2-year treatment, the anterior and right-side posterior crossbites were improved. Multibracket treatment began after the growth spurt. After 44-month active treatment, a functional occlusion, including a Class I molar relationship with a proper interincisal relationship, was achieved. A functional occlusion was maintained during a 10-year retention period, while a mandibular downward growth was observed through the retention period. Conventional orthodontic techniques enable functional and stable occlusion even in patients with mandibular deviation caused by congenital cervical lymphangioma, although only using early orthodontic management by itself may have some limitations. The hybrid technique combining functional appliance and intermaxillary elastics proves to be an effective therapy for correcting occlusal cant and mandibular deviation caused by cervical lymphangioma.

Lymphangioma of the fetus: prenatal diagnosis and the possibility of intrauterine correction

A case of prenatal diagnosis of fetal cervical lymphangioma is presented. During dynamic observation, the tumor was growing. At 36 weeks the tumor has reached a large size. In order to reduce the risk of shoulder dystocia during childbirth, lymphangioma was punctured under ultrasound guidance. An example of successful intrauterine decompression of large cervical lymphangioma, shows the possibility to use it in practice. This can relieve anxiety about shoulder dystocia and avoid the need for a cesarean section, and further helps reduce the possibility of early neonatal complications. A literature review is presented.

A case of critical airway stenosis with difficult intubation following OK-432 treatments against giant cervical lymphangiomas in neonate

A case of critical airway stenosis with difficult intubation following OK-432 treatments against giant cervical lymphangiomas in neonate

Un’opprimente tumefazione laterocervicale... da far mancare il respiro

Lymphatic malformations are low-flow vascular anomalies of the lymphatic system that occur in one out of 2-4,000 live births. They may be classified as macrocystic (diameter > 1 cm), microcystic (diameter < 1 cm) or mixed. Lymphatic malformations that are not prenatally diagnosed are typically diagnosed at birth or in early childhood. The clinical presentation can be quite variable, ranging from a focal area with minimal swelling to large involvement with compromise of adjacent structures. Up to 75% of lymphatic malformations are found in the cervicofacial region and their presence in the aerodigestive tract can lead to life-threatening airway obstruction. Lesions can grow slowly or rapidly and suddenly due to infection, trauma, bleeding or hormone level changes. The paper describes the case of a 9-month-old male infant with cervical macrocystic lymphangioma appeared during a febrile airway infection and misdiagnosed with bacterial lymphadenitis.

Cervical Cystic Lymphangioma in an Adult Patient. A Case Report of a Rare Entity

Cystic lymphangioma (CL) is a rare benign tumour that arises from the lymphatic vessels. The most common site of presentation is the posterior triangle of the neck. 90% of the lesions are diagnosed before the age of two years old and only a small number is reported in adults. In this paper, we describe the diagnostic and treatment approach of a cervical CL in an adult male.

Ex-Utero Intrapartum Treatment (EXIT): indications and outcome in fetal cervical and oropharyngeal masses

BackgroundThe “Ex-Utero Intrapartum Treatment” (EXIT) procedure allows to ensure fetal airway before completion of delivery and umbilical cord clamping while keeping uteroplacental circulation. Airway obstruction in fetal oropharyngeal and cervical masses can be life-threatening at birth. In those situations, controlled access to fetal airway performed by a trained multidisciplinary team allows safe airway management, while feto-maternal circulation is preserved. We aim to review the indications and outcome of the EXIT procedure in a case series of fetal cervical and oropharyngeal masses.MethodsWe have carried out a retrospective review of all patients with fetal cervical and oropharyngeal masses who underwent an EXIT procedure between 2008 and 2019. Variables evaluated included indication for EXIT, ultrasound and MRI findings, the need of amnioreduction, gestational age at EXIT, birth weight, complications, operative time, survival rate, pathological findings, and postnatal evolution. Five patients are included in this series. One additional case has already been published.ResultsThe diagnosis were cervical teratoma (n = 1), epulis (n = 1) and lymphangioma (n = 3). Polyhydramnios was present in 2 patients, requiring amnioreduction in one of them. Mean gestational age at EXIT was 36–37 weeks (range, 34–38 weeks). Median EXIT time in placental support was 9 min (range, 3–22 min). Access to airway was successfully established in EXIT in all cases. All children born by EXIT are currently healthy and without complications.ConclusionThe localization and characteristics of the mass, its relationship to the airway, and the presence of polyhydramnios seem to be major factors determining indications for EXIT and clinical outcome.