OK-432 Sclerotherapy of Giant Cervicomediastinal Cystic Hygroma.

Cystic hygroma is one type of the benign malformations and typically located in the neck, clavicle, and others, in children under the age of 5 years. However, the incidence of giant cervicomediastinal giant cystic hygroma is very rare, especially in adulthood. Such a location and age make its diagnosis difficult because they are usually asymptomatic. Complete surgical resection seems impossible while multiple sites are involved. Herein, we present a case of giant cervicomediastinal cystic hygroma, describing the clinical presentation, radiographic features, and OK-432 sclerotherapy. In conclusion, repeated OK-432 sclerotherapy may be an effective treatment option in giant cervicomediastinal cystic hygroma. Pay close attention to patient's symptoms and vital signs, adjusting the OK-432 dose throughout the process.

Management of Concealed Type IV Endoleak and Aortic Sac Hygroma by Prone ContrASt EnHancement Computed Tomography Angiography.

BACKGROUND: Aortic sac hygroma and concealed endoleaks (EL) after endovascular aneurysm repair (EVAR) of abdominal aortic aneurysm needs particular attention with aggressive management as they are associated with rapid sac expansion and rupture risk. However, they can be erroneously reported as type IV or V EL with supine computed tomography (CT) scans, leading to delay in management. Therefore, we describe a novel diagnostic technique, 'Prone contrASt enHanced computed tomography Angiography' (PASHA), to document concealed EL METHODS: We present eight case descriptions with continuous sac expansion after primary EVAR. Management began with diagnosis using the PASHA imaging technique. PASHA is a multiphase CTA positional technique for increasing the accuracy of detecting EL after EVAR. Furthermore, the PASHA imaging technique also guides whether the open or endovascular intervention could be used effectively to manage the sac expansion. In synchrony with the PASHA technique, "EVAR GORE SalvAge FAbric Technique" (ARAFAT) was to salvage previous EVAR. RESULTS: The PASHA technique diagnosed all cases of type IIIb EL, as it enhanced the degree of contrast infiltration into the aortic sac when microleaks were present. ARAFAT was effectively used in five elderly patients. Another three had an open conversion; two with double breasting of the aortic sac and one EVAR explantation. CONCLUSIONS: The PASHA protocol helped classify and localize the concealed EL (type IV, V), which were not appropriately diagnosed by supine CT protocols. PASHA and ARAFAT were used as a fully functioning protocol to overcome apparent challenges in accurate diagnosis and subsequent concealed EL management in high-risk patients.

Outcome of antenatally diagnosed cystic hygroma - Lessons learnt.

PURPOSE: This study is carried out to characterize the prognosis and outcome of antenatally diagnosed cystic hygroma in a series of registered pregnancies. METHODS: This is a prospective cohort study, carried out over a period of 4 years (Jan 2016-Sept 2019). All the pregnancies referred from the department of obstetrics with antenatally detected cystic hygroma or increased nuchal thickness on level II ultrasonography suggestive of lymphangiomas were registered in the Pediatric Surgery outpatient department. Amniocentesis, fetal ECHO and fetal MRI (if indicated)were done. Prognosis was explained to the family and mode of delivery was planned as per the obstetric indications. Postnatal evaluation included general physical examination along with ultra sound (USG)doppler of the lesion. The neonates were admitted in neonatal surgical ICU for the administration of intralesional bleomycin in a dose of 0.3 IU/kg under strict observation. All the babies were followed up at 3, 6 and 12 months. Results were segregated as excellent, good and still in follow up cohort as per the final outcome. The fetal neck masses detected antenatally and the age at first dose of bleomycin were compared with the number of sclerotherapy doses required to achieve good response. RESULTS: Only nine patients out of 626 registered pregnancies (1.4%) were diagnosed with cystic hygroma. One antenatal mother opted for termination of pregnancy at 19 weeks of gestation and another patient was lost to follow up after receiving two doses of bleomycin in postnatal period. Maternal hypothyroidism was noted in two pregnancies. Antenatal USG shows increased nuchal thickness in 2 cases (first trimester) and 6 cases in level II scan. Amniocentesis shows normal fetal karyotyping in all these pregnancies. Fetal ECHO suggests normal biventricular function in 100% cases while fetal MRI done in one case completely ruled out any aerodigestive compromise. Mode of delivery is found to be planned elective LSCS in 57% of cases while normal vaginal delivery attained in 43% of cases as per the obstetric guidelines. Six cases achieved good response with >50% reduction in size out of which 4 cases received the dose at the first 10 days of life. Complete disappearance of lesion at the end of 6 months of follow up were seen in two babies. CONCLUSIONS: Antenatal screening for early detection of fetal cystic hygroma with possible associated congenital aneuploidies helps in prognostication and planning the mode of delivery. A multimodality approach during intra and postpartum increases safety margin. Even huge neck masses without associated anomalies carry fair prognosis. Intralesional bleomycin is safe and prevents surgical morbidity.

Spontaneous partial regression of a microcystic jejunal mesenteric lymphangioma and a proposed management algorithm.

Mesenteric lymphangiomas are relatively rare, with clinical symptoms ranging from an asymptomatic presentation to an acute abdomen. The natural history and biological behaviour of this entity can range from slow indolent lesions to aggressive tumours with a risk of malignant transformation. Spontaneous regression of a mesenteric lymphangioma is rare. We herein report a case of a jejunal mesenteric lymphangioma that was initially detected incidentally in an asymptomatic patient with a subsequent sudden increase in size with resulting surrounding mass effect after 9 months and a spontaneous partial regression at surgical resection. Our case is the first reported case which outlines a period in the natural history and evolution of microcystic mesenteric lymphangioma, illustrating the sudden enlargement likely attributable to spontaneous and self-limiting haemorrhage and subsequent partial regression at surgical resection. We thenceforth propose a possible management algorithm for adult patients with mesenteric lymphangiomas.

Dilemma in management of cervico-facial cystic hygroma.

Cervico facial cystic hygroma and tongue lymphagioma is rare representative of spectrum of lymphatic malformations. Conservative management with sclerosants alone has proven to be successful. However, sudden enlargement of these cervico facial lymphangiomas leads to catastrophic airway obstruction leading to debility in feeding and speech. Therefore, surgery is indicated in such case to prevent such a catastrophic problem. We report here the case of a 3-yearold boy with cervico facial hygroma involving the tongue. We successfully treated him with a combination of surgery and OK432 injection.

The Management of Giant Cerviofacial Lymphatic Malformations in Children.

OBJECTIVE: Lymphatic malformations (LMs) are rare and benign diseases. This article aimed to present a series of surgically treated giant cervicofacial LMs in children. METHODS: A retrospective analysis of giant cervicofacial LMs (tumor diameter greater than 10 cm) in children over the past 8 years was performed in our department. RESULTS: The 10 patients included 4 males and 6 females. Macrocystic lesion presented in 4 patients, mixed cystic lesion presented in 4 patients, and microcystic lesion presented in 2 patients. All the patients underwent surgical resection combined with bleomycin sclerotherapy. Despite the wide range of lesions, most patients (7/10, 70%) received only a single treatment, 3 patients had a recurrent lesion. Seven patients experienced complete or almost complete resolution of the disease with an average follow-up time of 26.4 months. CONCLUSIONS: Surgical resection is the preferred method for the treatment of giant LMs, combination of bleomycin sclerotherapy can reduce the recurrence rate. The curative effect of macrocystic disease is relatively enhanced compared with microcystic and mixed cystic diseases.

A Rare Cause of Dyspnea: Cervicothoracic Cystic Lymphangioma.

I read with great interest the article reported by Efe et al in Journal of Cranofacial Surgery (2016;27:1802-1803), presenting the excellent result obtained by the use of sclerotherapy with single-dose OK-432 in a growing cervical lymphangioma. Cervicothoracic lymphangioma is a rare congenital anomaly that is mostly asymptomatic. We would like to share our experience of a rare cause of dyspnea by lymphangioma. In our case, the patient presented with dyspnea and paroxysmal cough caused by cervicothoracic lymphangioma and sclerotherapy alleviated tracheal compression and relieved the dyspneic symptoms.

Management and outcomes of cystic hygromas: experience of a tertiary center.

PURPOSE: Cystic hygroma (CH) is a fetal sonographic finding with an incidence of 1%. Chromosomal abnormalities and structural malformations are commonly related with CH. We aimed to describe our experience and determine the association between diagnosis of CH and adverse pregnancy outcome. METHODS: We retrospectively reviewed data of prenatal CH diagnoses over a ten-year period. Cases were evaluated for maternal age, gestational week at CH diagnosis, invasive procedure, karyotype result, associated abnormality and perinatal outcome. We categorized cases into two groups to understand the impact of maternal age on perinatal outcomes, and the cut-off was 35 years old. RESULTS: Totally 28 individuals were enrolled in the study. The median maternal age at birth was 27.5 years old, and 7 patients were over 35 years old. Karyotype results were available for all individuals, and 15 had an abnormal chromosome (53.5%). Five fetuses had structural abnormalities, and they were all observed within patients under 35 years old. Healthy fetuses (without any problem at antenatal screening and birth) were found to be only 14.3% in advanced maternal age (AMA) group and 33.3% in young mothers. CONCLUSIONS: Women with AMA and CH had a higher risk of having a baby with a chromosomal abnormality, as compared with younger women.

Non-surgical treatment of a relapsed cystic hygroma in an adult.

Lymphatic malformations, also known as lymphangiomas or cystic hygromas, are benign masses that typically affect newborns and infants and involve the head and neck regions. They are, however, rare in adults and even rarer in the axillary region. Although surgery is considered to be the treatment of choice, we present a rare case of a recurrent cystic hygroma 32 years after the first surgical operation. Due to the cosmetic concerns and the risks of a surgical approach, non-surgical therapy with percutaneous sclerosants was performed, with a good outcome after a 2-year follow-up period.

Abdominal cystic lymphangioma in a term newborn: A case report and update of new treatments.

INTRODUCTION: Lymphatic malformations are benign anomalies derived from the abnormal development of lymphatic channels. Usually asymptomatic, they can cause compression on adjacent structures or present acute complications (bleeding or infection). Small asymptomatic lesions can be conservatively managed since the possibility of spontaneous regressions is described, while symptomatic lesions require active management. Less invasive therapeutic options are now preferred instead of surgery (sclerotherapy, laser therapy). However, there are not uniform therapeutic protocols. CASE REPORT: We present the case of a term newborn with an abdominal cystic lymphangioma extending from the umbilical to the right inguinal area, reaching the medial surface of the right tight. Despite its large dimensions, which classically request surgical management, the patient was by chance asymptomatic, and the mass did not determine compression on the surrounding organs. Therefore, conservative management was tried, and a close clinical and radiological follow-up was started. This approach permitted a spontaneous regression of the mass and to avoid major surgical intervention. CONCLUSION: Our purpose is to underline the possibility of conservative management of the major multicystic masses and to focus on less invasive therapeutic options, like sclerotherapy, oral therapy, and laser therapy.

[Unusual macrocystic lymphatic malformation in an adult patient]. / Malformation lymphatique macrokystique atypique chez un adulte.

Macrocystic lymphatic malformations (MLMs) constitute a circumscribed variation in deep lymphangiomas. They are characterized by a rare occurrence in adults, by their rapid expansion and by their frequent occurrence at the level of the cervicofacial region (75%) and axillary region (20%). They rarely occur in lower limbs. The diagnosis of MLMs is primarily based on clinical examination. Ultrasound has a valuable role in positive diagnosis and sometimes in differential diagnosis. MRI has a role in the diagnosis and the assessment of tumor extent, thus making a valuable contribution to surgery. Therapeutically, MLMs are mainly treated by sclerotherapy; surgery is rarely indicated. We report the case of 26-year old man with a one-year history of huge painless and rapidly evolving subcutaneous swelling in the right thigh. Clinical examination showed circumferential compressible non-beating infiltrated mass, surmounted by vegetative and ulcerated lesions. The remainder of the clinical examination showed the presence of inguinal ipsilateral lymphadenopathy, with mobility limitation of the right knee. Angio-MRI objectified diffuse heterogeneous gadolinium-enhancing cutaneous, subcutaneous thickening of the right thigh with perimuscolar aponeurotic involvement. Lymphoscintigraphy showed asymmetry of the accumultion of radiopharmaceutical kinetics that was reduced in the right lower limb in favor of an overload of the lymphatic system at rest. Given the clinico-radiological data, the diagnosis of MLM was retained. Given the seat of the lymphangioma and its extent, after consultation with the surgeons there was no indication for surgery. Immobilization with a removable bandage was indicated for our patient.

Case report of a chest wall cystic hygroma in a teenager.

Cystic hygroma or cystic lymphangioma is a congenital malformation of lymphatic origin. Their occurrence on the chest wall is very rare, and they progressively grow with age infiltrating into the local tissues, around muscle fibers and nerves, making them difficult and hazardous to remove. There are various treatment modalities of such lesion. Based on the literature surgical excision is the preferred treatment of choice in cystic hygroma because it gives a better cure rate compared to other modalities. We report a case successful excision of anterolateral chest wall cystic hygroma in a teenager in Hospital Serdang.

Sclerotherapy treatment of orbital lymphatic malformations: a large single-centre experience.

BACKGROUND: Percutaneous sclerotherapy is an alternative to surgery for the treatment of orbital lymphatic malformations (LMs). We present a large series of patients undergoing sclerotherapy for macrocystic LMs with detailed visual acuity (VA) outcome data. METHODS: Data were collected prospectively in all patients with macrocystic orbital LMs undergoing sclerotherapy. Sclerotherapy was performed under general anaesthesia, instilling sodium tetradecyl sulfate under imaging control. Procedures were repeated at 2-week to 6-week intervals, depending on clinical response. Patients underwent ophthalmological assessment, ultrasound and/or MRI before and after treatment. PRIMARY OUTCOME MEASURE: change in maximal radiological diameter of the LM. SECONDARY OUTCOME MEASURE: change in VA after treatment. RESULTS: 29 patients underwent 71 procedures (1-8 procedures per patient) over 6.7 years. Mean age=7.31 years. 11 patients (37.9%) had undergone previous treatment, including excision biopsy, drainage and decompression. All patients presented with proptosis and/or pseudoptosis. 23 patients (79.3%) had decreased VA at presentation. Average follow-up was 21.8 months (range 3-75 months). All patients achieved a reduction in maximal lesion diameter of ≥50%, with complete radiological resolution in 51.7% (n=15). VA improved in 18/23 patients (78.2%). Average logMAR before treatment=0.43 (SD ±0.47); average after treatment=0.25 (SD ±0.32); p<0.01. There was one complication (1.4%): one patient required a lateral canthotomy for an intralesional haematoma 2 h after sclerotherapy. CONCLUSIONS: Sclerotherapy is a safe and highly effective treatment for orbital LMs with excellent VA outcomes. It should be considered as the first-line treatment for this condition.

Cross-sectional study comparing different therapeutic modalities for cystic lymphangiomas in children.

OBJECTIVE: Here, we describe our experience with different therapeutic modalities used to treat cystic lymphangiomas in children in our hospital, including single therapy with OK-432, bleomycin and surgery, and a combination of the three modalities. METHODS: We performed a retrospective, cross-sectional study including patients treated from 1998 to 2011. The effects on macrocystic lymphangiomas and adverse reactions were evaluated. Twenty-nine children with cystic lymphangiomas without any previous treatment were included. Under general anesthesia, patients given sclerosing agents underwent puncture of the lesion (guided by ultrasound when necessary) and complete aspiration of the intralesional liquid. The patients were evaluated with ultrasound and clinical examinations for a maximum follow-up time of 4 years. RESULTS: The proportions of patients considered cured after the first therapeutic approach were 44% in the surgery group, 29% in the bleomycin group and 31% in the OK-432 group. These proportions were not significantly different. Sequential treatment increased the rates of curative results to 71%, 74% and 44%, respectively, after the final treatment, which in our case was approximately 1.5 applications per patient. CONCLUSION: The results of this study indicate that most patients with cystic lymphangiomas do not show complete resolution after the initial therapy, regardless of whether the therapy is surgical or involves the use of sclerosing agents. To achieve complete resolution of the lesions, either multiple operations or a combination of surgery and sclerotherapy must be used and should be tailored to the characteristics of each patient.

A review of the management of lymphangiomas.

INTRODUCTION: Lymphangioma is a rare benign cyst caused by congenital malformation of the lymphatic systems that often occurs in the cervicofacial region. There is no consensus on its management: Observation, aspiration, injection, cryotherapy, electrocautery, radiation, laser, ligation and excision. METHODS: We performed a literature search with the keywords "cystic hygroma", "lymphangioma", "management", "OK 432" and "picibanil" from Medline, Embase and PubMed databases. RESULTS: We present a review of the history, signs and symptoms, diagnosis, histology, classification and management options of cystic hygroma. CONCLUSION: There is no consensus on the treatment options. It should be individualised depending on the size of the lesion, anatomic localisation and complications.

Cross-sectional study comparing different therapeutic modalities for cystic lymphangiomas in children

OBJECTIVE: Here, we describe our experience with different therapeutic modalities used to treat cystic lymphangiomas in children in our hospital, including single therapy with OK-432, bleomycin and surgery, and a combination of the three modalities. METHODS: We performed a retrospective, cross-sectional study including patients treated from 1998 to 2011. The effects on macrocystic lymphangiomas and adverse reactions were evaluated. Twenty-nine children with cystic lymphangiomas without any previous treatment were included. Under general anesthesia, patients given sclerosing agents underwent puncture of the lesion (guided by ultrasound when necessary) and complete aspiration of the intralesional liquid. The patients were evaluated with ultrasound and clinical examinations for a maximum follow-up time of 4 years. RESULTS: The proportions of patients considered cured after the first therapeutic approach were 44% in the surgery group, 29% in the bleomycin group and 31% in the OK-432 group. These proportions were not significantly different. Sequential treatment increased the rates of curative results to 71%, 74% and 44%, respectively, after the final treatment, which in our case was approximately 1.5 applications per patient. CONCLUSION: The results of this study indicate that most patients with cystic lymphangiomas do not show complete resolution after the initial therapy, regardless of whether the therapy is surgical or involves the use of sclerosing agents. To achieve complete resolution of the lesions, either multiple operations or a combination of surgery and sclerotherapy must be used and should be tailored to the characteristics of each patient. .