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.

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.

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.

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.

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.

Intrathecal saline infusion: an emergency procedure in a patient with spontaneous intracranial hypotension.

BACKGROUND: Spontaneous intracranial hypotension (SIH) is a neurologic condition with the prototypical symptom of orthostatic headache. We report a dramatic case of SIH with life-threatening bilateral hygroma and uncal herniation. METHODS: Case report. RESULTS: A 44-year-old male patient presenting with orthostatic headache and double vision was diagnosed with SIH. Diagnostic imaging showed meningeal enhancement and bilateral hygroma. A conservative treatment regime was initiated. The patient's condition rapidly deteriorated with progressive loss of consciousness. Cranial MRI showed beginning uncal herniation. As an emergency treatment measure, an intracranial pressure (ICP) probe was inserted and intrathecal lumbal saline infusion was initiated. This led to a stabilization of ICP and allowed further diagnostics and treatment. CONCLUSION: Intrathecal lumbal saline infusion in combination with ICP monitoring can be a life-saving treatment option in unstable SIH patients.

Percutaneous drainage and ablation as first line therapy for macrocystic and microcystic orbital lymphatic malformations.

PURPOSE: To review the management of orbital lymphangiomas and to propose a new treatment for both macrocystic and microcystic lymphatic malformations of the orbit. METHODS: A retrospective case series of all patients from the authors' practice from 2001 to the present who met the histopathologic and/or diagnostic imaging criteria for orbital lymphatic malformation was reviewed. Lymphatic malformation was diagnosed if there was a multilobulated pattern on CT or a cystic internal structure on ultrasonography. In patients that were treated, macrocysts (>1 cm) were treated with dual-drug chemoablation (sequential intracystic sodium tetradecyl sulfate and ethanol); doxycycline injections were used for microcysts. The goal of treatment was complete cyst ablation documented by ultrasonography or MRI. RESULTS: Twenty patients met the inclusion criteria. They were separated in 3 groups based on the anatomical location of the lymphatic malformation: deep, superficial, or combined. Deep orbital lymphatic malformation presented in 14 patients (70%), superficial presented in 4 patients (20%), and both deep and superficial presented in 2 patients (10%). Thirteen of the 20 patients underwent percutaneous sclerotherapy. Of those treated, 7 patients (53.8%) had lymphatic malformations (LM), while 6 patients (46.2%) had venous-lymphatic malformations (VLMs). The average number of treatments required to achieve complete cyst ablation in patients with LM was 1.7. The average number of treatments required for patients with VLM was 3.0; however, some of these patients continue to have the venous component of their lesions treated. Clinically, all treated patients maintained or improved an average of one Snellen line (-0.16 decimal Snellen equivalent) from their preoperative visual acuity to their last recorded follow-up visit. There was a mean reduction in proptosis of 2.4 mm (p - 0.003, confidence interval [CI] 0.838 to 3.962), which was statistically significant. There were no recurrences (0%) in patients who completed treatment with cyst ablation (n - 8) at an average follow-up period of 43 months (range 6-96, standard deviation 30). There were no data available as to the recurrence status of one patient. Four patients were still undergoing treatment for a venous component at the time of this review. CONCLUSIONS: Percutaneous sclerotherapy provides a safe and effective treatment for both macrocystic and microcystic orbital lymphatic malformations as a primary treatment or for recurrence after surgical intervention.

New treatment for cystic lymphangiomas of the face and neck: cyst wall rupture and cyst aspiration combined with sclerotherapy.

Cystic lymphangioma is a congenital malformation occurring most frequently in the necks of infants and young children. It is histologically benign, but the lesion can extend deep into the tissue. Thus, surgical treatment is often difficult. In recent years, OK-432 sclerotherapy has become the first-line therapy for cystic lymphangioma, and many reports have discussed its effectiveness. However, it is difficult to achieve a complete response to OK-432 in polycystic cases and cases with small cystic areas, and it is sometimes ineffective. In the current study, we performed a new combination therapy on 5 cases of cystic lymphangioma of the face and neck. In this combination therapy, we ruptured the cyst wall of lymphangioma using a liposuction device and subsequently used OK-432 to induce inflammation and to cause adhesion of cyst walls. The combination therapy produced very good results. None of the patients had any major complication or recurrence. All patients had lymphangiomas that markedly reduced in size. This new method can be performed regardless of the cyst type of lymphangioma and is thought to be a useful therapy.

[Cystic hygroma : report of 25 cases]. / Les lymphangiomes kystiques : A propos de 25 cas.

BACKGROUND: The cystic hygroma is a benign lymphatic malformation, a rare but potentially serious in its character and its evolutionary trend dissecans. The head and neck region constitutes the favorite seat (75%). If the diagnosis is usually easy, the therapeutic management remains controversial. AIM: To analyze clinical and paraclinical characteristics of cystic hygroma and to discuss the various therapeutic methods. METHODS: We report a retrospective study about 25 cases of head and neck cystic hygroma collected during a period of 11 years (1998- 2008) in the ENT department of the hospital The Rabta Tunis. RESULTS: The average age of our patients was 18 years and 5 months. All patients consulted for a neck mass. It was localized in the submandibular region in 7 cases, 3 cases in parotid region, jugulocarotid artery in 3 cases and affecting the posterior triangle in 12 cases. Cervical ultrasound was performed in 22 cases (88%) evoking the diagnosis of cystic hygroma in 16 cases (72%). CT was performed in 10 cases finding hypodense aspect in 8 cases. MRI was carried out in 9 cases. It showed the aspect hyperintense T2, hypointense T1 in 7 cases. Surgical excision was performed in 22 cases and sclerotherapy in 3 patients. During the evolution, a recurrence was observed for each treatment modality. CONCLUSION: Surgery is treatment of choice of cystic hygroma. Sclerotherapy may be indicated as an alternative to surgery in localized and diffuse macrocystic forms.

Anaphylaxis to ethylene oxide - a rare and overlooked phenomenon?

Spina bifida patients have been reported to be at increased risk of anaphylactic reactions during general anaesthesia. Following a reaction, latex is often incriminated as spina bifida patients are known to have an increased incidence of latex allergy. Ethylene oxide (EO) has recently been suggested to be an alternative cause, but in many cases reported in the literature, it seems that EO has not been considered as a cause. EO is a highly reactive gas widely used to sterilise heat-sensitive medical devices, and traces of EO can be found in many of the same products as latex. We present the case of a spina bifida patient with a known latex allergy, where EO was found to be the cause of an anaphylactic reaction during general anaesthesia. In addition, we describe measures taken during preparation of a subsequent general anaesthesia to minimise exposure to EO. Spina bifida patients seem to be at increased risk of sensitisation against EO due to repeated exposure, but only limited literature is available. To ensure that EO is considered as a cause in these cases, we recommend that testing for latex and EO go hand in hand following an anaphylactic reaction in this high-risk population.

The management of primary nonparasitic splenic cysts.

Primary nonparasitic splenic cysts (PNSC) are rare and their management in children has been controversial. We conducted this study to discuss various treatment modalities. The medical records of patients with PNSC (1991-2008) were evaluated retrospectively, including age, sex, history of trauma, presenting symptoms, physical examination and radiological findings, therapeutic approaches, and outcomes. Six patients, between 3 to 12 years of age with a male/female ratio of 2, were included. The presenting symptom was abdominal pain in all but one asymptomatic patient. Physical examination findings were unremarkable in all except for palpable spleen in two patients. Cyst sizes ranged from 3 x 4 cm to 10 x 12 cm. The patients were treated with aspiration-sclerotherapy (n=2), total splenectomy (n=2), partial splenectomy (n=1), and cyst excision (n=1). The pathological diagnoses were epidermoid cyst (n=5) and lymphangioma (n=1). The postoperative course was uneventful except for postsplenectomy fever (n=1), recurrence (n=1) and residual cyst (n=1). PNSC larger than 5 cm in diameter or those that are symptomatic should be treated surgically. Total splenectomy should not be done in children to avoid infectious postsplenectomy problems unless there is a mandatory condition like intraoperative bleeding. Aspiration-sclerosis is not recommended because of recurrence.

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.

Multidisciplinary approach in cystic hygroma: prenatal diagnosis, outcome, and postnatal follow up.

BACKGROUND: The aim of the present study was to determine prenatal follow up and clinical outcome in fetuses born with cystic hygroma. METHODS: A series of 64 cystic hygroma patients, who were diagnosed in the first and the second trimester of pregnancy, was enrolled. Associated structural abnormalities, karyotype analysis and pregnancy outcome were studied. Survivors were followed for their fetal outcome and prognosis. RESULTS: There were 64 new cases of cystic hygroma in 8524 subjects screened (0.75%). Thirty-nine (60.9%) were of non-septated and 25 (39.1%) were of septated cystic hygroma. Chromosomal abnormalities were present in 25 (39.1%). The most common abnormality in non-septated cystic hygroma was trisomy 21 (10, 27.8%), and that in septated cystic hygroma was Turner syndrome (5, 23.8%). Associated structural malformations are common in cystic hygroma and overall survival was poor. Nine of the present infants were live-born and were subsequently followed up. Two had cardiac pathology and died after cardiac operation, two others were diagnosed with axillary cystic hygroma, had an excellent prognosis and responded well to treatment, and another two had cranial findings with mild neurological sequel. Only three cases had, at birth and in the follow-up period, no complications. CONCLUSION: Cystic hygroma is highly correlated with adverse perinatal outcome. Prenatal diagnosis and invasive procedures are vital for counselling with close follow-up after delivery for appropriate medical support. A multidisciplinary approach is strictly recommended in live-born children.

Giant lymphangioma of the tongue.

This report presents the treatment of an extensive lymphatic malformation of the tongue. Sclerosing agents are now widely used as the first-line treatment of lymphatic malformation. However, treatment of lymphatic malformation involving the face and the vital structures such as the airway remains to be challenging. A 4-year-old boy underwent a total of 15 OK-432 injection sclerotherapy treatments over a 2-year period, having slow progress until sudden enlargement of the tongue was noted shortly after the last injection. Partial excision of the lesion was performed. This case demonstrates the risk in treating large microcystic lymphatic malformation of the tongue with sclerotherapy and provides an insight in the management protocol.

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.

Percutaneous sclerotherapy of a giant mediastinal lymphangioma.

The present study reports a case of percutaneous sclerotherapy of a giant mediastinal cystic lymphangioma using Ethibloc (Ethicon, Norderstedt, Germany) and absolute ethanol in a 59-yr-old female. The tumour, situated predominantly in a retrocardiac location, caused dyspnoea and dysphagia by compression and was considered unresectable. Follow-up computed tomography 3 yrs after treatment showed a 90% volume reduction of the tumour. The patient is currently asymptomatic. To the best of the present authors' knowledge, percutaneous transthoracic sclerotherapy of a mediastinal lymphangioma has not previously been reported in the literature available in English.

[Cystic lymphangioma: an unusual cause of lower limb lymphedema]. / Malformation lymphatique kystique : cause rare d'un lymphoedème unilatéral des membres inférieurs.

We report an unusual cause of unilateral lymphedema of the right limb rapidly increasing in a young woman. Ultrasonography ruled out the diagnosis of iliac deep venous thrombosis or extrinsic compression: the B mode scan revealed a mass located below the aortic bifurcation and along the iliac vessels, without any compressive effect. The lesion was heterogeneous associating both tissular and cystic aspects. The lower limb lymphoscintigraphy showed an interruption of the colloid circulation at the right iliac level. Computed tomography did not give any additional information. Magnetic resonance imaging before then after gadolinium showed typical aspects of cystic lymphangioma and confirmed the ultrasonography hypothesis. Considering that this tumor is benign, that surgery would be difficult because of the anatomic situation of the mass, and that post-operative involution of the edema is uncertain, the treatment was based on compressive stockings and regular follow up.