Intermittent Manual Carotid Compression as a Treatment Option for Indirect Low Flow Carotid Cavernous Fistula.

PURPOSE: To evaluate the efficacy of intermittent manual carotid compression (IMCC) in the management of low-flow carotid cavernous fistulae (CCF). MATERIALS AND METHODS: Patients diagnosed with low-flow CCF and treated with IMCC over a period of 13 years were retrospectively analyzed. Data analyzed included demographic details, clinical features, type of CCF, and response to therapy. Outcomes were categorized as complete cure, partial cure, and no cure or worsening. Patients with complete and partial cure were grouped as good outcome whereas those with no cure or worsening as poor outcome. RESULTS: A total of 44 patients were advised IMCC for low-flow CCF, of whom five were lost to follow-up. Results of the remaining 39 patients were analyzed, of whom 21 (53.8%) were males. The mean age at presentation was 54.38 ± 14.54 years. The median duration between the onset of symptoms and presentation was 5.0 ± 4.10 months. The common presenting features were episcleral congestion and proptosis (89.7%), extraocular motility restriction (66.7%), and diplopia (48.7%). Most common CCF type was type D (34, 87.2%). All patients were advised to undergo IMCC. Good outcome was seen in 35 patients (89.7%, 95% CI: 80.2% to 99.2%), whereas poor outcome was seen in four (10.3%) patients. The mean duration of follow-up was 15.91 ± 21.87 months. CONCLUSION: The present study found a good outcome with IMCC in 89.7% cases of indirect low-flow CCF with no major complications. It should be considered the management of choice in patients who are systemically unfit or cannot afford endovascular embolization.

Treatment of facial hypertrophic capillary malformations with tumescent-assisted sclerotherapy.

Facial capillary malformations (CMs) become hypertrophic and nodular overtime and pose great therapeutic challenge. Here, we describe safe and effective use of tumescent-assisted sclerotherapy (TAS) in conjunction with yellow vascular laser (577 nm) for the treatment of HFCMs. Three patients underwent TAS were included in the case series, and complete resolution in nodularity was achieved in all patients with TAS, with no major complications such as skin necrosis, distal embolisation, blindness and neurological adverse events such as stroke or TIA occurred in any patients.

[Ascending aorta and aortic arch hypoplasia in a patient with connective tissue dysplasia and multiple deficit of coagulation factors]. / Gipoplaziya voskhodyashchego otdela i dugi aorty u bol'nogo s displaziei soedinitel'noi tkani i kombinirovannym defitsitom plazmennykh faktorov svertyvaniya.

Connective tissue dysplasia (CTD) is an urgent problem, especially in young and employable people. Damage to cardiovascular system and, in particular, aorta is predominant factor determining the incidence of various complications and mortality in patients with CTD. The authors report surgical treatment of aortic hypoplasia in an adolescent patient with connective tissue dysplasia syndrome and combined deficiency of coagulation factors.

RETINAL VASCULAR ABNORMALITIES RELATED TO NEUROFIBROMATOSIS TYPE 1: Natural History and Classification by Optical Coherence Tomography Angiography in 473 Patients.

PURPOSE: To analyze and classify neurofibromatosis Type 1 (NF1)-related retinal vascular abnormalities (RVAs), their natural history and correlation with disease severity, in a large cohort of patients. METHODS: This was an observational longitudinal study with prospective enrollment. Four hundred and seventy-three patients affected by NF1 and 150 age-matched healthy subjects were consecutively enrolled. Retinal vascular abnormalities were detected by means of near-infrared reflectance and studied by optical coherence tomography angiography. The superficial vascular plexus and the deep vascular complex (DVC) were quantitatively and qualitatively analyzed. RESULTS: We identified RVAs in 82 of 473 (17%) NF1 patients, but in none of the 150 healthy subjects. A comparison revealed that NF1 patients with RVAs showed a higher number of NF1 diagnostic criteria (4.3 ± 1.5 vs. 3.9 ±1.5, respectively; P = 0.02) than patients without RVAs. Three different RVA types were identified on optical coherence tomography angiography: macrovascular angiomatosis of the sole superficial vascular plexus; macrovascular angiomatosis of the superficial vascular plexus combined with microvascular angiomatosis of the deep vascular complex; and combined macrovascular angiomatosis of both superficial vascular plexus and deep vascular complex. The prospective analysis of optical coherence tomography angiography images showed no significant longitudinal evolution of RVAs (mean follow-up: 3.7 ± 2.8 years). A single patient developed a de novo single RVA, and two RVAs showed detectable changes during follow-up. CONCLUSION: In NF1 patients, RVAs are a characteristic sign that correlates with a more severe systemic disease expression, usually remaining stable during time. Optical coherence tomography angiography allows for the identification of different RVAs subtypes.

Sclerotherapy treatment of a large venous malformation invading the bladder wall related to Klippel-Trenaunay syndrome.

We report the case of a 35-year-old woman who presented with recurrent macroscopic haematuria and known diagnosis of Klippel-Trenaunay syndrome. Imaging and cystoscopy identified an extensive venous malformation involving a large area of the bladder wall. Holmium laser therapy was ineffective at obtaining symptom control. Following a multidisciplinary team meeting, transvenous sclerotherapy with sodium tetradecyl sulphate was performed under image guidance. A reduction in venous density was observed on cystoscopy and the patient has had complete resolution of symptoms within 6 weeks and continued to be asymptomatic up to 24-month follow-up. We propose that transvenous sclerotherapy is considered first-line treatment in this clinical setting.

Symptomatic Intraosseous Vascular Malformation of Infraorbital Rim: A Case Report With Literature Survey.

BACKGROUND/AIM: Intraosseous orbital hemangiomas or vascular malformations (VM) are rare. This report is intended to complement the experience of diagnosing and treating a rare vascular lesion at this site. Special attention is paid to three-dimensional imaging and the morphological distinction between the two entities in this location. CASE REPORT: A 54-year-old female was examined and surgically treated for an exophytic firm mass of the infraorbital, which had become palpable as a hard mass due to growth in size. At first, a bone tumor, for example, an osteoma, was suspected. Intraoperatively, an osseous expansion with distinct fenestrations of the newly grown bone's surface, was detected. The lesion was firmly attaching to the orbital rim. The densely vascularized tumor was well defined to the soft tissues but had grown in continuity from the orbital floor and rim. Vascularized cavities caused the tumor to have a slightly reddish color. The histological examination confirmed the suspicion of the lesion's vascular origin. The lesion's immunohistochemical expression profile approved the final diagnosis of intraosseous VM. CONCLUSION: The symptoms of intraosseous vascular lesions of the orbit are determined by location and size. Modern imaging techniques facilitate the estimation of tumor-like expansion of lesions. However, the imaging characteristics of intraosseous vascular lesions are very variable. The symptoms of the patient presented herein show that growth phases of a vascular orbital malformation can occur in later stages of life and are initially indistinguishable from a neoplasm. In individual cases, patient care necessitates advanced diagnostic measures to establish the diagnosis and determine surgical therapy.

A combination of single-balloon enteroscopy-assisted laparoscopy and endoscopic mucosal resection for treating gastrointestinal venous malformations in blue rubber bleb nevus syndrome: a case report.

BACKGROUND: Blue rubber bleb nevus syndrome (BRBNS) is a rare congenital disease characterized by multifocal venous malformations. It remains a considerable challenge in treating the gastrointestinal (GI) venous malformations due to multiple lesions throughout the GI tract, and the likelihood of recurrence. We report a case study of BRBNS in the GI tract, in which GI venous malformations and related GI bleeding were successfully treated with a combination of multiple endoscopic procedures. CASE PRESENTATION: A 17-year-old man was admitted to our hospital for dizziness and hypodynamia. The symptoms persisted for nearly 1 year. The laboratory tests revealed iron-deficiency anemia with abnormally low hemoglobin (Hb), and a strong positive fecal occult blood test. A total of four hemangiomas were detected: one in the stomach, one in the descending colon, and two in the small intestines with a high risk of hemorrhage. Under gastroendoscopy, enteroscopy, and video capsule endoscopy (VCE) throughout the GI tract, the patient underwent surgical treatment. Endoscopic mucosal resection was initially performed in the stomach and colon, and the lesions in the small intestine were resected with laparoscopy auxiliaried by single-balloon enteroscopy (SBE), during which SBE assisted in identifying the lesions. The patient well-tolerated the procedures, and had a favorable prognosis. CONCLUSION: The combination of single-balloon enteroscopy-assisted laparoscopy and endoscopic mucosal resection was effective for the present case, which could be considered for patients with similar clinical conditions.

Liver transplantation in the setting of a spontaneous shunt between superior mesenteric vein and right renal vein.

Shunts between the superior mesenteric vein (SMV) and the right renal vein (RRV) are very rare. Here, we describe and depict the rare case of a liver transplant (LT) in the setting of shunt between SMV and RRV. A 67-year-old white man presenting with Child C cirrhosis secondary to hemochromatosis and persistent encephalopathy was listed for LT. Preoperative abdominal angiotomography revealed the presence of a large spontaneous shunt between the SMV and the RRV. The patient underwent LT by receiving a liver from a 17-year-old brain-dead deceased donor victim of trauma. A large shunt between the SMV and the RRV was confirmed intraoperatively. Although there was no portal vein (PV) thrombosis, the PV was atrophic and had a reduced flow. PV pressure was 22mmHg (an arterial line was inserted inside the PV stump, and this line was connected to a common pressure transducer, the pressure readings was expressed in the anesthesia monitor). After shunt ligation PV pressure increased to 32mmHg. There were no post-transplant vascular complications, and the patient was discharged home in good health. Preoperative study of all LT candidates with angio CT scan is mandatory. Whenever there is PV thrombosis, an attempt to remove the entire thrombus is warranted. After thrombectomy or whenever there is not PV thrombosis, all large shunts should be ligated. PV pressure and flow should be measured before and after shunt ligation. In the absence of PV thrombosis, ligation of the shunt should enable an increase in PV flow and pressure, as reported herein.

Vascular anomaly of the posterior circulation associated with intracranial lipoma-like lesion in the cerebral peduncle manifesting as oculomotor nerve palsy.

Intracranial lipomas are rare and often located in the midline of the brain. Intracranial lipomas are often associated with malformations of the brain such as dysgenesis of the corpus callosum, but rarely with vascular malformations. A man presented with left-sided facial pain at the age of 31. He developed left oculomotor nerve palsy at the age of 38 years and was referred to our hospital at the age of 48. Radiological findings revealed vascular anomalies of the left posterior cerebral and superior cerebellar arteries with intracranial lipoma-like lesion in the cerebral peduncle. Surgical treatment was complicated by the lesion location, so we administered conservative therapy. Despite treatment with corticosteroids, his symptoms have not improved. This unique case documents the presentation of vascular anomalies of the left posterior cerebral and superior cerebellar arteries associated with lipoma in the cerebral peduncle.

Widely Divergent Congenital Inferior Tibiofibular Diastasis with Separate Soft-Tissue Cover and Persistent Sciatic Artery: A Previously Unreported Combination: A Case Report.

CASE: We report a case of a child with widely divergent congenital inferior tibiofibular diastasis with persistent sciatic artery (PSA). The dysplastic tibia and fibula were widely divergent, and the fibula was displaced proximally and medially with the foot alongside the thigh between the 2 legs, with PSA diagnosed on computed tomography angiogram. The child was treated with fibula-foot complex excision and below-knee prosthesis and was ambulating independently at 1-year follow-up. CONCLUSIONS: The combination of a major structural anomaly (tibiofibular diastasis with a separate soft-tissue cover) and an unusual vascular malformation (PSA) has not been reported previously and made surgical reconstruction challenging.

Association between the posterior part of the circle of Willis and the vertebral artery hypoplasia.

BACKGROUND: It is not clear whether the configuration of the posterior part of the circle of Willis (CW) depends on the proximal part of the vertebrobasilar system. Our aim is to evaluate the posterior part of CW in association with different size of vertebral arteries (VA) in healthy volunteers. MATERIALS AND METHODS: The present study was based on a sample of 923 healthy volunteers who were examined from 2013 through 2018. The duplex ultrasonographic examination of the extracranial vertebral (VA) and carotid arteries was performed. VA was defined as hypoplastic (VAH) when VA diameter in the entire course was less than 2.5 mm. All the participants underwent magnetic resonance angiography (MRA) examination. All the component vessels of the circle of Willis were assessed in each individual. We classified the posterior communicating artery (PCoA) as presence PCoA, absence/hypoplastic PCoA and fetal-type posterior circle of Willis (FCW) in which the major stem of the posterior cerebral artery (PCA) arises from ipsilateral internal carotid artery (ICA). The comparison of the posterior part of CW was made in subjects with normal VA and VAH of a different degree (communicating with basilar artery (VAH-BA) and not communicating with the basilar artery (VAH-PICA)). RESULTS: FCW was found in 15.9% of subjects, bilaterally-in 2.3%. FCW was more frequent in individuals with VAH than in those with normal VA (accordingly, 28.8% vs. 13.5%, p<0.001. Moreover FCW was recorded in 50% of the subjects with VA-PICA in comparison with 13.5% of those with normal VA and 22.8% with VAH-BA, p<0.005. On the contrary, absence/hypoplasia of both PCoA was mostly found in the group with normal VA in comparison with VAH-BA and VAH-PICA (accordingly, 50.7%, 38.6% and 12.5%, p<0.01). CONCLUSION: Individuals with VAH have a different pattern of the posterior part of CW in comparison with those with normal VA. With the increasing degree of VAH, the proportion of FCW increases, while the proportion of absence/hypoplastic of both PCoA decreases.

Thrombophlebitis hiding under a KILT - case report on 40 years long-term follow-up of neonatal renal vein thrombosis.

BACKGROUND: Neonatal renal vein thrombosis is a recognised cause of renal and inferior caval vein atresia (IVCA). However, the long-term impact of the condition is underrecognized with a high burden of morbidity for the patient, especially in adulthood. IVCA has been shown to be an independent risk factor for deep venous thrombosis (DVT) with a high risk of recurrence. The acronym KILT for kidney and inferior vena cava anomaly with leg thrombosis summarizes the pathological situation. CASE PRESENTATION: We present the case of a 40-year-old patient with pain in the right lower limb resulting from acute thrombophlebitis. No risk factors could be identified. His history was remarkable with two episodes of deep venous thrombosis first of the left, then the right leg 22 years earlier; at that time also, no risk factor was identified. Because of the idiopathic character of that thrombosis, the patient remained on long-term anticoagulation with phenprocoumon. The present thrombophlebitis occurred while the INR was not therapeutic in the preceding weeks. A CT with contrast showed atresia of the inferior vena cava and of the right kidney, and presence of numerous collaterals. A thorough medical history revealed a renal vein thrombosis as a neonate. Anticoagulation was intensified, and stent placement became necessary after a further 2 years. DISCUSSION AND CONCLUSIONS: KILT syndrome is a rare but underrecognized condition. Complications may arise in young adulthood only, and it is of prime importance to instruct parents of the pediatric patient of the possible consequences of renal vein thrombosis and to assure guidance from the treating physicians throughout adulthood. Diagnosis of IVCA is by CT with contrast or by MRI, and lifelong anticoagulation may be necessary. Since the KILT syndrome is widely underdiagnosed, we challenge the clinicians to keep it in mind when confronted with thrombophlebitis or thrombosis of the young, male and with no other identifiable risk factors for deep vein thrombosis.