Endoscopic injection of lauromacrogol foam sclerotherapy for rectal cavernous hemangioma: A case report.

RATIONALE: Rectal cavernous hemangioma is a rare, benign vascular disease that seldom causes lower gastrointestinal bleeding, characterized by a high rate of misdiagnosis and missed diagnoses. Surgical treatment is considered to be relatively effective; however, it is accompanied by certain employed in the treatment of superficial hemangioma, boasting the advantages of minimally invasive surgery, including safety, effectiveness, reduced trauma, and rapid recovery. However, there is a lack of literature regarding the application of foam sclerosing agents for gastrointestinal hemangiomas. CASE CONCERNS: We present a case of a 60-year-old male who was admitted to our hospital with a history of recurrent hematochezia for >1 year and worsening symptoms for 1 week. The patient's medical history was unremarkable. DIAGNOSES: Following colonoscopy, nuclear magnetic resonance imaging, computed tomography, and other examinations, the final diagnosis was rectal cavernous hemangioma. INTERVENTIONS: Due to the patient's refusal of surgery, endoscopic foam sclerotherapy using a lauromacrogol injection was performed after obtaining informed consent from the patient and their relatives. OUTCOMES: Post-sclerotherapy, hematochezia symptoms ceased, and no adverse reactions were observed. Two months later, colonoscopy and nuclear magnetic resonance imaging showed that the hemangioma had almost completely disappeared, with only a small amount of tumor remnants, yielding a satisfactory curative effect. CONCLUSION: Our findings indicate that endoscopic injection of a lauromacrogol foam sclerosing agent is a safe, effective, and minimally invasive treatment option for gastrointestinal cavernous hemangiomas.

Hepatic Hemangioma: Review of Imaging and Therapeutic Strategies.

Hepatic hemangiomas are the most common benign liver tumors. Typically, small- to medium-sized hemangiomas are asymptomatic and discovered incidentally through the widespread use of imaging techniques. Giant hemangiomas (>5 cm) have a higher risk of complications. A variety of imaging methods are used for diagnosis. Cavernous hemangioma is the most frequent type, but radiologists must be aware of other varieties. Conservative management is often adequate, but some cases necessitate targeted interventions. Although surgery was traditionally the main treatment, the evolution of minimally invasive procedures now often recommends transarterial chemoembolization as the treatment of choice.

A Case of a Refractory Bleeding Giant Vaginal Wall Cavernous Hemangioma Successfully Managed with Sclerotherapy.

BACKGROUND Vaginal wall hemangiomas are extremely rare, benign, vascular tumors of the female genitalia. Most cases occur in childhood, but a few cases can be acquired; however, the mechanism of hemangioma formation remains unknown. Most hemangiomas involving female genital organs are small and asymptomatic. However, huge hemangiomas can cause irregular genital bleeding, infertility, and miscarriage. Surgical excision and embolization are the most common treatment options. We reveal that sclerotherapy achieved good outcomes in a patient with an intractable huge vaginal wall hemangioma. CASE REPORT A 71-year-old woman visited a local doctor with concerns of frequent urination. A ring pessary was inserted after a diagnosis of pelvic organ prolapse. However, symptoms did not improve, and the patient consulted another hospital. The previous physician diagnosed vaginal wall tumors and prolapse and performed a colporrhaphy. However, she was referred to our hospital with heavy intraoperative bleeding. Imaging examination revealed a huge hemangioma on the vaginal wall, which was histologically diagnosed as a cavernous hemangioma. Angiography revealed hemorrhage in the right peripheral vaginal artery. Owing to concerns regarding extensive vaginal wall necrosis caused by arterial embolization, sclerotherapy using monoethanolamine oleate was selected. Hemostasis was achieved 1 month after sclerotherapy, and postoperative imaging showed the lesion had shrunk in size. No recurrence of hemangioma was observed 19 months after surgery. CONCLUSIONS We report a case of a large vaginal wall intractable bleeding hemangioma. Sclerotherapy can be a suitable treatment option for large vaginal hemangiomas that are too extensive to be treated using surgery or arterial embolization.

Resection of a Large Cavernous Hemangioma Following Preoperative Embolization in a Child's Temporal Bone.

Primary intraosseous cavernous hemangiomas (PICHs) in the temporal bone are very rare. Most treated cases are in adult patients, and there are very few reports involving children. To our knowledge, no cases reported have ever actually adopted embolization in children, although several reports recommend preoperative embolization of the feeding arteries. Here, we present a case of a child with a large cavernous hemangioma developing in the temporal bone. The patient was an 11-year-old girl whose chief complaints were hearing loss, aural fullness, and otalgia. Based on imaging, a lymphoma was initially suspected, but extensive arterial bleeding occurred during biopsy under local anesthesia. Angiography was performed to evaluate the blood flow in the tumor, and revealed the middle meningeal artery as a main feeding artery to the tumor. A huge PICH at the temporal bone was successfully resected following preoperative embolization of the feeding artery.

Endoscopic Ultrasound-Guided Ethanol Injection Associated with Trans-arterial Embolization of a Giant Intra-abdominal Cavernous Hemangioma: Case Report and New Therapeutic Option.

BACKGROUND: Cavernous hemangiomas are congenital hamartomatous lesions that originate from mesodermal tissue composed of dilated blood vessels. Abdominal pain and palpable mass are the most common presenting symptoms. The different types of treatment for symptomatic patients remain controversial. However, surgical resection is always the most preferred method when possible. To date, there are no reports of endoscopic ultrasound-guided (EUS-guided) absolute ethanol injection as a treatment for such disease when surgery is not an option. CASE PRESENTATION: A 19-year-old girl with giant cavernous intra-abdominal hemangioma extending to the hepatic hilum, also affecting the gastric wall and occupying the entire supra-mesocolic cavity, initially presents with upper gastrointestinal bleeding and loss of 20 kg in 1 year (BMI = 18 kg/m2). Percutaneous angiography identified a mass with arterial blood supply by the left gastric artery that was embolized. After re-bleeding, an alternative treatment with EUS-guided injection of alcohol was proposed once resection was not feasible without major risks to a young patient. This procedure was repeated 15 and 45 days after the initial treatment, with the ethanol injection of 25 cc and 15 cc, respectively. On the second and third procedure dates, there was evident regression of the hemangioma. On the third procedure, it was possible to identify all anatomic structures that were not clear on the first EUS. After 45 days of last injection, abdominal CT and EUS showed notorious regression of the lesion. Eight months later, abdominal CT showed only a remnant lesion in the hepatogastric ligament with 129 cm3 on volumetry (87% lower in comparison to the initial image), and the patient remains asymptomatic with BMI of 26. In the most recent follow-up CT, 4 years and 2 months after first treatment, the patient presents with a slight increase in the hemangioma-now with 183 cm3 on volumetry. CONCLUSION: Transgastric EUS-guided ethanol injection in the treatment of giant intra-abdominal cavernous hemangioma can provide good outcomes without major complications and can be repeated if necessary.

A unique case of multiple calvarial hemangiomas with one large symplastic hemangioma.

BACKGROUND: Symplastic hemangioma is a benign superficial abnormal buildup of blood vessels, with morphological features which can mimic a pseudo malignancy. A few cases have been reported in the literature. We report here, a unique case of calvarial symplastic hemangioma, which is the first case in the calvarial region. CASE PRESENTATION: A 29-year-old male patient, with a left occipital calvarial mass since childhood, that gradually increased in size with age, was associated with recurrent epileptic fits controlled by Levetiracetam (Keppra), with no history of trauma. He presented to the emergency room with a recent headache, vomiting, frequent epileptic fits and a decrease in the level of consciousness 1 day prior to admission. A CT scan showed three diploic, expansile, variable sized lytic lesions with a sunburst appearance; two that were biparietal, and one that was left occipital, which were all suggestive of calvarial hemangiomas. However, the large intracranial soft tissue content, within the hemorrhage of the occipital lesion was concerning. The patient had refused surgery over the years; however, after the last severe presentation, he finally agreed to treatment. The two adjacent, left parietal and occipital lesions were treated satisfactorily using preoperative embolization, surgical resection, and cranioplasty. Histopathology revealed cavernous hemangiomas, in addition to symplastic hemangioma (pseudo malignancy features) on top at the occipital lesion. The right parietal lesion was not within the surgical field; therefore, it was left untouched for follow-up. CONCLUSIONS: Histopathology and radiology examinations confirmed the diagnosis as symplastic hemangioma, on top of a pre-existing cavernous hemangioma. To the best of our knowledge, this is the first case of a calvarial symplastic hemangioma, which we report here.

Urethral cavernous hemangioma: a highly misdiagnosed disease (a case report of two patients and literature review).

BACKGROUND: Diagnosis of urethral cavernous hemangioma (UCH) is very rare. It can be easy to misdiagnose and mistreat due to its atypical clinical manifestations and a lack of relevant knowledge. The study is to explore the diagnosis, differential diagnosis, and treatment of UCH. CASE PRESENTATION: The first patient was a 15-year-old male, who was admitted to the hospital for more than 1 year with repeated hematuria. UCH was diagnosed by cystoscope biopsy, and cured with local injection of pingyangmycin. The second patient was a 49-year-old male, who was admitted for repeated painless gross hematuria and intermittent urethral bleeding after penile erection for more than 20 years. The case had been misdiagnosed as seminal vesiculitis, urethritis, or prostatitis, for over 20 years, until it was diagnosed as UCH by MR examination of the penis. It was treated by injection of pingyangmycin into the hemangioma's lumen and base. A small incision in the ventral penile area was separated from the location of the hemangioma, which was injected with pingyangmycin again. A biopsy of resected tissue further confirmed the diagnosis of UCH. CONCLUSIONS: UCH is an easily misdiagnosed disease. Intermittent painless hematuria is important characteristic of UCH. Local injection of pingyangmycin is a good option for treatment of UCH.

Multidisciplinary management of an intra-sellar cavernous hemangioma: Case report and review of the literature.

Extra-axial cavernous hemangiomas (ECH) are rare vascular lesions with a tendency to grow within the medial structures of the middle cranial fossa. This pathological entity lacks specific symptoms, and falls into the category of differential diagnosis of space occupying lesions in the cavernous sinus (CS) with or without sellar involvement, including those of tumoral, vascular and inflammatory nature. Of note, ECH can also be indolent, and is at times discovered incidentally during autopsy investigations. On radiological studies, ECH with sellar extension are frequently mistaken at first for pituitary adenomas. Total removal of intrasellar-CS ECH is technically demanding and burdened by remarkable morbidity and mortality rates, mostly related to the complex neuroanatomy of the CS-sellar region (i.e., peri and postoperative bleeding, and transitory or permanent nerve palsies, hormonal deficits). Consequently, only a few cases of successful total removal have been reported so far in the literature. Surgical debulking with cranial nerve decompression followed by stereotactic radiosurgery is currently considered the best alternative to total removal when the latter carries excessive perioperative risks. We present a rare case of a mainly located intrasellar ECH extending to the left CS discussing its clinical features and focusing on the most relevant aspects of the surgical management along with a review of the pertinent literature.

The current management of spinal cord cavernoma.

BACKGROUND: Spinal cavernous malformations (SCM) were once thought to be rare lesions of the spinal cord. However, with the broad use of modern imaging techniques the incidence of SCM has significantly increased over the last decades. Management of both symptomatic and incidental findings is therefore of growing importance. However, experience with treatment and follow-up is very limited. METHODS: We performed a single institution retrospective review of consecutive patients with SCM treated at our Department between 2006-2016 and discuss the clinical features as well as surgical versus conservative outcomes. We further provide a systematic literature search and discuss the best management of SCM, analyzing recent publications on SCM imaging techniques, surgical approaches and natural history. RESULTS: From a total number of 406 consecutive patients with cavernous malformations (CM) treated at our Department between 2006-2016, 29 (7.1%) were found to be affected by SCM. The localization was cervical in 10 (34.5%), cervicothoracic in 3 (10.4%) and thoracic in 16 (55.2%) patients. In 90% of patients (N.=26) the diagnosis was made after onset of clinical symptoms. Conservative management was performed for 8 patients, whereas 21 patients underwent surgical removal of the lesion via a posterior approach using (hemi-) laminectomy or laminoplasty. Functional status improved in 15 patients (62.5%) and remained unchanged in 6 patients (28.5%) in the operative group, whereas 2 patients (25%) improved and 6 patients (75%) remained unchanged in the conservative group during long-term follow-up. CONCLUSIONS: Gross-total resection is the only definitive treatment option for symptomatic SCM. Surgical extirpation of the symptomatic SCM lesion through an unilateral laminectomy (=hemilaminectomy) approach within 3 months of presentation seems to be good treatment option with an acceptable risk of complications and good long-term outcomes. Conservative treatment should be performed in asymptomatic patients and seems to be an option as well in elderly patients and if patients' symptoms at diagnosis are mild and do not show progression over time.

Ultrasonography-guided percutaneous microwave ablation for large hepatic cavernous haemangiomas.

PURPOSE: To evaluate the efficacy and safety of ultrasound-guided percutaneous microwave ablation (MWA) for large hepatic cavernous haemangiomas (HCHs) with ablation of the feeding artery and aspiration of blood from haemangioma simultaneously. MATERIALS AND METHODS: From January 2011 to December 2016, 40 patients (29 females, 11male; average age 43.25 ± 7.65 years) with 42 giant hepatic haemangiomas (mean diameter 7.02 ± 1.55 cm, range 4.1-10.8 cm) were treated with image-guided percutaneous MWA with ablation of the feeding artery and aspiration of blood from haemangioma simultaneously. After MWA, therapeutic efficacy was assessed by contrast-enhanced imaging during follow-up. Median follow-up time was 24 months (range 6-60 months). RESULTS: Technical effectiveness rate was 100% with a mean ablation time of 1175 ± 516 (range 480-2340) seconds. The mean number of antenna insertions was 3.82 ± 1.23 for each lesion. Clinical effectiveness rate was 95% (38/40). There was a significant decrease of the tumour volume within three days after ablation compared with before ablation (p < 0.001). The mean tumour volume shrinkage rate was 59.67 ± 16.73% (range 28.20-89.72%) within three days after ablation. Minor complications mainly included fever, transient elevation of transaminase, pleura effusion, and haemoglobinuria. One patient developed a major complication with acute kidney injury (AKI) shortly after MWA, whose renal function gradually recovered after haemodialysis. No other severe complications occurred in perioperative and follow-up periods. CONCLUSIONS: Ultrasound-guided percutaneous MWA for large HCHs with ablation of the feeding artery and aspiration of blood from haemangioma simultaneously is safe and effective.

Features of sinonasal hemangioma: A retrospective study of 31 cases.

OBJECTIVE: Although hemangiomas are common lesions of the head and neck, sinonasal hemangiomas are rare. The purpose of this study was to analyze the clinical features (sex, age, symptoms, and size and anatomical location of the lesion) and the histological findings of sinonasal hemangioma cases, to assess preoperative transarterial embolization, and to evaluate the outcome (recurrence or no recurrence) of endoscopic sinus surgery. METHODS: Clinical records of 31 patients who underwent endoscopic sinus surgery for resection of sinonasal hemangioma between January 2010 and June 2015 were retrospectively reviewed. RESULTS: The study group consisted of 19 men and 12 women. Mean age was 53.3±15.9years. The principal symptom was epistaxis (81%). Mean tumor size was 12.6±8.2mm. The most common origin of hemangioma was the inferior turbinate (45%), followed by the nasal septum (39%), and, in both locations, the origin had a tendency to be located in the anterior portion. Thirty-one specimens were histologically categorized as 9 cavernous hemangiomas and 22 capillary hemangiomas. Preoperative transarterial embolization was performed in 2 cases. Only one recurrence was observed among 31 cases. In the recurrent case, the hemangioma of the nasal septum was resected during pregnancy. CONCLUSION: According to our results, the transnasal endoscopic approach can be useful for the resection of sinonasal hemangioma. However, sinonasal hemangioma in connection with pregnancy must be addressed with care to decide the appropriate time for treatment.

Synopsis of Guidelines for the Clinical Management of Cerebral Cavernous Malformations: Consensus Recommendations Based on Systematic Literature Review by the Angioma Alliance Scientific Advisory Board Clinical Experts Panel.

BACKGROUND: Despite many publications about cerebral cavernous malformations (CCMs), controversy remains regarding diagnostic and management strategies. OBJECTIVE: To develop guidelines for CCM management. METHODS: The Angioma Alliance ( www.angioma.org ), the patient support group in the United States advocating on behalf of patients and research in CCM, convened a multidisciplinary writing group comprising expert CCM clinicians to help summarize the existing literature related to the clinical care of CCM, focusing on 5 topics: (1) epidemiology and natural history, (2) genetic testing and counseling, (3) diagnostic criteria and radiology standards, (4) neurosurgical considerations, and (5) neurological considerations. The group reviewed literature, rated evidence, developed recommendations, and established consensus, controversies, and knowledge gaps according to a prespecified protocol. RESULTS: Of 1270 publications published between January 1, 1983 and September 31, 2014, we selected 98 based on methodological criteria, and identified 38 additional recent or relevant publications. Topic authors used these publications to summarize current knowledge and arrive at 23 consensus management recommendations, which we rated by class (size of effect) and level (estimate of certainty) according to the American Heart Association/American Stroke Association criteria. No recommendation was level A (because of the absence of randomized controlled trials), 11 (48%) were level B, and 12 (52%) were level C. Recommendations were class I in 8 (35%), class II in 10 (43%), and class III in 5 (22%). CONCLUSION: Current evidence supports recommendations for the management of CCM, but their generally low levels and classes mandate further research to better inform clinical practice and update these recommendations. The complete recommendations document, including the criteria for selecting reference citations, a more detailed justification of the respective recommendations, and a summary of controversies and knowledge gaps, was similarly peer reviewed and is available on line www.angioma.org/CCMGuidelines .

CAVERNOUS HEMANGIOMA OF THE RETINA: A Comprehensive Review of the Literature (1934-2015).

PURPOSE: Cavernous hemangioma of the retina (CHR) is a rare retinal vascular hamartoma. The objective of this study was to summarize the current knowledge of this rare condition based on a detailed review of 96 documented cases. METHODS: The incidence, clinical and histological appearance, differential diagnosis, treatment, and prognosis of CHR were analyzed by evaluating clinical data obtained from 96 cases in the literature since the first case report in 1934. RESULTS: More than 90% of cases of CHR have been reported in white individuals with even gender distribution. A majority of patients presented a unilateral unifocal lesion without positive family history. Our analysis showed bilateral cases were strongly correlated with family history, which suggests the importance of screenings for family members in bilateral CHR cases. Intracranial involvement was reported in 14% of CHR cases. Most patients were asymptomatic, but in patients with history of headaches, transient visual disturbance, or seizures 80% had intracranial involvement. CONCLUSION: It is important to screen family members in bilateral CHR cases, and it is critical to exclude cerebral vascular abnormalities in view of relative high incidence of intracranial involvement and high morbidity.

Possible toxicity following embolization of congenital giant vertex hemangioma: case report.

The authors describe the case of a 3-year-old boy with a giant congenital vertex hemangioma who underwent presurgical embolization with Onyx (ethylene-vinyl alcohol copolymer dissolved in dimethyl sulfoxide) and Glubran ( N-butyl-2-cyanoacrylate). This vascular tumor had no intracranial vascular communication as assessed by pre-embolization MRI and catheter angiography. All embolizations were performed by direct percutaneous injection. One week following the last embolization procedure the child presented with a 24-hour history of ataxia and extrapyramidal tremor. He was diagnosed with a possible immune-mediated reaction to Onyx or Glubran, which was treated with an urgent surgical excision of the hemangioma followed by intravenous administration of immunoglobulin and steroids. To the authors' knowledge, this is the first case of possible immune-mediated toxicity secondary to either Onyx or Glubran administration. This case highlights the need for awareness of potential toxic reactions to these embolic agents in the treatment of hemangiomas in the pediatric patient.

MR imaging features that distinguish spinal cavernous angioma from hemorrhagic ependymoma and serial MRI changes in cavernous angioma.

Cavernous angiomas of the spinal cord exhibit imaging characteristics that may overlap with those of hemorrhagic ependymoma. In the present study, we aimed to identify specific magnetic resonance imaging (MRI) findings that could be used to differentiate cavernous angioma from hemorrhagic ependymoma, and to evaluate serial MRI changes in cases of cavernous angioma. We retrospectively evaluated MR images of spinal cord tumors collected at our hospital from 2007 to 2015. From this cohort of images, 11 pathologically confirmed cavernous angiomas and 14 pathologically confirmed hemorrhagic ependymomas were compared with respect to the size of the tumor, longitudinal location, axial location, enhancement pattern, syrinx, edema, tumor margin, signal intensity of T2WI, signal intensity of T1WI, and longitudinal spreading of the hemorrhage. Serial MR images of seven spinal cavernous angiomas were reviewed. Small size, eccentric axial location, minimal enhancement, and absence of edema were more frequently observed on images of cavernous angioma compared to those of hemorrhagic ependymoma (p < 0.01). Serial MRI changes in cases of cavernous angioma included increased longitudinal spreading of the hemorrhage (6/7, 86 %) and emergence of high signal intensity on T1WI (1/7, 14 %). Small size, eccentric axial location, minimal enhancement, and absence of edema are significant MRI findings that may be used to distinguish Type I and Type II spinal cavernous angiomas from hemorrhagic ependymomas. Furthermore, longitudinal spreading of the hemorrhage may be observed on follow-up MRIs of cavernous angiomas.

Diagnosis and treatment of cavernous hemangioma of the internal auditory canal.

OBJECTIVE: Cavernous hemangioma of the internal auditory canal (IAC) is an extremely rare type of tumor, and only 50 cases have been reported in the literature prior to this study. The aim in this study was to describe the symptomatology, radiological features, and surgical outcomes for patients with cavernous hemangioma of the IAC and to discuss the diagnostic criteria and treatment strategy for the disease. METHODS: The study included 6 patients with cavernous hemangioma of the IAC. All patients presented with sensorineural hearing loss and tinnitus, and 2 also suffered from vertigo. Five patients reported a history of facial symptoms with hemispasm or palsy: 3 had progressive facial weakness, 1 had a hemispasm, and 1 had a history of recovery from sudden facial paresis. All patients underwent CT and MRI to rule out intracanalicular vestibular schwannomas and facial nerve neuromas. Five patients had their tumors surgically removed, while 1 patient, who did not have facial problems, was followed up with a wait-and-scan approach. RESULTS: All patients had a presurgical diagnosis of cavernous hemangioma of the IAC, which was confirmed pathologically in the 5 patients who underwent surgical removal of the tumor. The translabyrinthine approach was used to remove the tumor in 4 patients, while the middle cranial fossa approach was used in the 1 patient who still had functional hearing. Tumors adhered to cranial nerves VII and/or VIII and were difficult to dissect from nerve sheaths during surgeries. Complete hearing loss occurred in all 5 patients. In 3 patients, the facial nerve could not be separated from the tumor, and primary end-to-end anastomosis was performed. Intact facial nerve preservation was achieved in 2 patients. Patients were followed up for at least 1 year after treatment, and MRI showed no evidence of tumor regrowth. All patients experienced some level of recovery in facial nerve function. CONCLUSIONS: Cavernous hemangioma of the IAC can be diagnosed preoperatively through analysis of clinical features and neuroimaging. Early surgical intervention may preserve the functional integrity of the facial nerve and provide a better outcome after nerve reconstruction. However, preservation of functional hearing may not be achieved, even with the retrosigmoid or middle cranial fossa approaches. The translabyrinthine approach seems to be the most appropriate approach overall, as the facial nerve can be easily located and reconstructed.

En face mode of swept-source optical coherence tomography in circumscribed choroidal haemangioma.

PURPOSE: To describe the findings in circumscribed choroidal haemangioma (CCH) using en face swept-source optical coherence tomography (SS-OCT). METHODS: En face images were obtained employing DRI-1 Atlantis OCT (Topcon, Tokyo, Japan), using a three-dimensional volumetric scan of 12×9 mm. Images were obtained from the retinal pigment epithelium to 1000 µm in depth of the tumour. RESULTS: Twenty-two eyes from 22 patients with the clinical diagnosis of CCH were included. In 20 eyes (90.9%), a characteristic pattern was visualised in the en face image across the vascular tumour. A multilobular pattern, similar to a honeycomb, with hyporeflective, confluent, oval or round areas corresponding with the lumen of the tumour vascular spaces, and hyper-reflective zones, which may represent the vessels walls and connective tissue of the tumour. Ten eyes (45.4%) showed a hyper-reflective halo surrounding the tumour. Seventeen tumours (77.2%) showed small diameter vessels at the inner zone and larger vessels in the outer area. Twelve patients (54.5%) had previously received treatment (photodynamic therapy, transpupillary thermotherapy, dexamethasone intravitreal implant or brachytherapy with ruthenium-106). No differences were found between treated and untreated patients in any of the measured parameters. CONCLUSIONS: En face SS-OCT is a rapid, non-invasive, high-resolution, technology, which allows a complementary study to cross-sectional scans in CCH. A characteristic multilobular pattern, with a hyper-reflective halo surrounding the tumour, was found in en face SS-OCT images. No morphological differences were found between naïve patients and patients who received previous treatment.

[Portal cavernoma in children revealed by gastrointestinal haemorrhage: about a case]. / Cavernome portal chez l'enfant révélé par une hémorragie digestive: à propos d'un cas.

Portal cavernoma is a venous vascular anomaly characterized by the formation of a network of veins whose caliber is increased and carrying portal blood. It is due to a thrombotic and always chronic occlusion of the extra-hepatic portal venous system. This is one of the most common causes of portal hypertension in children. Its severity is mainly associated with an high risk of gastrointestinal haemorrhage. Very few cases have been described mainly in African literature. We report the case of a 4-year old boy admitted with very abundant haematemesis, melena and dizziness associated with anemic syndrome on examination. Laboratory tests showed severe microcytic hypochromic anemia with normal renal and hepatic function. Gastrointestinal endoscopy showed esophageal varices (grade III) with red signs. Abdominal ultrasound showed portal vein formation resulting in the classic "spiderweb", in favor of a cavernoma. Abdominal CT scan confirmed portal cavernoma associated with portal hypertensive syndrome and vascular anomaly like an ectopic splenic vein anastomosis with the trunk formed by the gonadal vein and the inferior mesenteric vein. Therapeutic approach was based on blood transfusion and beta-blocker treatment. Portal cavernoma can be a major complication of vascular malformations often unknown. In case of gastrointestinal haemorrhage in children, diagnosis should be suspected. Its management requires early treatment and should be adapted to the patient's condition in order to prevent a fatal evolution.