In Vivo Imaging of the Buccal Mucosa Shows Loss of the Endothelial Glycocalyx and Perivascular Hemorrhages in Pediatric Plasmodium falciparum Malaria.

Severe malaria is mostly caused by Plasmodium falciparum, resulting in considerable, systemic inflammation and pronounced endothelial activation. The endothelium forms an interface between blood and tissue, and vasculopathy has previously been linked with malaria severity. We studied the extent to which the endothelial glycocalyx that normally maintains endothelial function is involved in falciparum malaria pathogenesis by using incident dark-field imaging in the buccal mucosa. This enabled calculation of the perfused boundary region, which indicates to what extent erythrocytes can permeate the endothelial glycocalyx. The perfused boundary region was significantly increased in severe malaria patients and mirrored by an increase of soluble glycocalyx components in plasma. This is suggestive of a substantial endothelial glycocalyx loss. Patients with severe malaria had significantly higher plasma levels of sulfated glycosaminoglycans than patients with uncomplicated malaria, whereas other measured glycocalyx markers were raised to a comparable extent in both groups. In severe malaria, the plasma level of the glycosaminoglycan hyaluronic acid was positively correlated with the perfused boundary region in the buccal cavity. Plasma hyaluronic acid and heparan sulfate were particularly high in severe malaria patients with a low Blantyre coma score, suggesting involvement in its pathogenesis. In vivo imaging also detected perivascular hemorrhages and sequestering late-stage parasites. In line with this, plasma angiopoietin-1 was decreased while angiopoietin-2 was increased, suggesting vascular instability. The density of hemorrhages correlated negatively with plasma levels of angiopoietin-1. Our findings indicate that as with experimental malaria, the loss of endothelial glycocalyx is associated with vascular dysfunction in human malaria and is related to severity.

Gamma-heavy chain monoclonal gammopathy with undetermined significance (MGUS).

Gamma-heavy chain disease (γ-HCD) is a rare B-cell tumor producing truncated IgG lacking the light chain. The clinical features of γ-HCD are heterogeneous, similar to lymphoplasmacytic lymphoma, and most patients have generalized and progressive disease. In some γ-HCD patients, autoimmune diseases are associated. Thus, γ-HCD as a restricted or indolent disease is exceptional. A 66-year-old male was referred to our hospital because of subungual hemorrhage at the bilateral halluces. Physical and laboratory examination results were nonspecific, and the hemorrhage was revealed to be traumatic. However, serum electrophoresis demonstrated a small M-peak, which was monoclonal IgG-Fc without the corresponding light chain on immunofixation and immunoelectrophoresis. Bone marrow aspirate demonstrated a small number of lymphoplasmacytic cells that were positive for CD19, CD38, CD138, and cyIgG, but negative for cyκ- and -λ light chains on flow cytometry. A diagnosis of γ-HCD was made. Chest and abdominal CT demonstrated neither hepatosplenomegaly, lymphadenopathy, nor bone lytic lesions. The serum concentrations of IgG and M-peak configuration have remained relatively unchanged for nearly 3 years. Therefore, this γ-HCD may correspond to a rare form of monoclonal gammopathy with undetermined significance.

High-flow paediatric mandibular arteriovenous malformations: case reports and a review of current management.

High-flow vascular malformations in the paediatric population are potentially life-threatening and are challenging to treat. This paper describes the management of three cases of mandibular arteriovenous malformations and reviews the contemporary management options for these serious lesions.

A Case of Acute Upper Airway Obstruction in a Pediatric Hemophilia A Patient Because of Spontaneous Retropharyngeal Hemorrhage.

We report a rare case of acute upper airway obstruction caused by spontaneous retropharyngeal hemorrhage as a result of hemophilia A in a 16-year-old pediatric patient who routinely received factor VIII replacement. Initial diagnosis was delayed because the patient presented with symptoms, such as throat pain and odynophagia, similar to those of common benign upper airway infections. Within 2 days of the initial presentation of symptoms, the patient went into respiratory failure as a result of retropharyngeal hemorrhage. The possibility of spontaneous retropharyngeal or epiglottic hemorrhage or hematoma should be considered as a cause of rapidly progressing odynophagia and dyspnea by hemophilia patients.

Pseudoaneurysm of the facial artery occurred after mandibular sagittal split ramus osteotomy.

INTRODUCTION: Pseudoaneurysms are caused by rupture of arteries with extravasation of blood. The compressed perivascular tissue forms the wall of aneurysmal sac. Pseudoaneurysm directly related with surgical procedure of sagittal split ramus osteotomy (SSRO) was reported quite rarely especially related with facial artery during the vertical osteotomy. CASE REPORT: SSRO was carried out for a 19-year-old male; the patient visited the emergency room with notable swelling 3 weeks after the surgery. We experienced severe intra-oral bleeding with surgical exploration. Angiography revealed a pseudoaneurysm of the right facial artery that might be related with vertical osteotomy over lateral cortex of the mandibular body during orthognathic surgery. This implies that the minor vascular trauma from vertical osteotomy of the mandibular body during the conventional orthognathic surgery might cause later development of pseudoaneurysm.

Life-threatening arteriovenous malformation of the maxillomandibular region and treatment outcomes.

We report on cases of life-threatening maxillomandibular arteriovenous malformations (AVM) whereby patients had successful endovascular treatment with good outcomes. Out of a total 93 facial AVMs treated endovascularly between 1991 and 2009, five patients (5.4%) had maxillomandibular AVMs. All presented with uncontrolled dental bleeding. Endovascular procedure was the primary treatment of choice in all cases, either transfemoral approach with arterial feeder embolization or transosseous puncture, depending on the accessible route in each patient. NBCA (glue) was the only embolic agent used. Tooth extraction and dental care were performed after bleeding was controlled. All five patients (8-18 years) with a mean age of 12.4 years presented with massive dental bleeding following loosening of teeth, dental extraction and/or cheek trauma. The plain films and CT scans of four patients with AVMs of mandibles and one of maxilla, revealed expansile osteolytic lesions. The mean follow-up period was 6.6 years (ranging between one and 19 years). Three cases developed recurrent bleeding between two weeks to three months after first embolization, resulting from residual AVM and infection. Late complications occurred in two patients from chronic localized infection and osteonecrosis, which were successfully eradicated with antibiotic therapy and bony curettage. Complications occurring in two patients which included soft tissue infection, osteomyelitis and osteonecrosis were successfully treated with antibiotics, curettage and bone resection. No patient had a recurrence of bleeding after the disease had cured Initial glue embolization is recommended as the effective treatment of dental AVMs for emergent bleeding control, with the aim to complete eradicate the intraosseous venous pouches either by means of transarterial superselection or direct transosseous puncture. Patient care by a multidisciplinary team approach is important for sustained treatment results.

Radiation carotid blowout syndrome in nasopharyngeal carcinoma: angiographic features and endovascular management.

OBJECTIVE: To report clinical manifestations, angiographic features, and outcomes of endovascular management in 14 patients with 15 radiation carotid blowout syndromes of nasopharyngeal carcinomas. STUDY DESIGN AND SUBJECTS: Retrospective chart review of 14 patients with nasopharyngeal carcinomas (mean age 49 years) with 15 radiation carotid blowout syndromes who had undergone endovascular embolization to manage oronasal bleeding in the past 10 years. RESULTS: Average radiation dose to affected carotid artery was 73 gray units (latent period: 33 months). Radiation carotid blowout syndrome was detected in internal (n = 10), external (n = 4), or common carotid artery (n = 1). Detachable balloons were used in 11 affecting arteries for vascular occlusion; 4 were treated by liquid adhesives or coil. Endovascular treatment was successful in all 15 radiation carotid blowout syndromes with cessation of hemorrhage. One patient had hemiparesis after embolization. Mean clinical follow-up was 21 months. CONCLUSION: Radiation carotid blowout syndrome in nasopharyngeal carcinoma may occur in various periods or arteries. Endovascular embolization provides both safe and effective management.

CT angiography before embolization for hemorrhage in head and neck cancer.

We present a patient with advanced head and neck carcinoma and a bleeding pseudoaneurysm diagnosed by means of CT angiography; this was not apparent on conventional digital subtraction angiograms. The information provided by CT angiography facilitated rapid identification of the pseudoaneurysm and treatment with embolization. CT angiography may be helpful before embolization in cases of hemorrhage in head and neck cancer.

Massive bleeding from an ectopic lingual thyroid follicular adenoma during pregnancy.

Ectopic thyroid is a rare developmental anomaly. It can be found anywhere between the foramen cecum and the normal position of the thyroid gland. Massive bleeding from an ectopic lingual thyroid is unusual and occasionally fatal. We present a case of a 22-year-old woman who had a large mass at the base of the tongue for over 6 years. In the sixth month of pregnancy, the patient experienced massive bleeding from the tongue base mass. A thyroid scan revealed that this mass was the only functioning thyroid tissue. Because of massive bleeding and her shock status, the patient received an emergent embolization of the bilateral lingual arteries. Then the huge lingual thyroid was subsequently excised via a mandible swing approach to prevent further episodes of bleeding. Pathology analysis indicated ectopic thyroid tissue with follicular adenoma. She delivered without complications in the 36th week and had a normal baby. This case was a very rare one in our review. Although ectopic lingual thyroid usually is not managed surgically, excision of ectopic lingual thyroid can be life-saving when it is causing bleeding or airway obstruction.

[Severe hemorrhage from rupture of an intra-oral arteriovenous malformation]. / Hémorragie grave par rupture d'une malformation artérioveineuse intrabuccale.

We report the case of a 47-year-old woman with a sudden, spontaneous exsanguinating haemorrhage from an arteriovenous malformation of the mandible. The diagnosis was obtained with a contrast enhanced CT-scan and a digital subtraction angiography. As arterial embolisation was not available the lesion was treated surgically.

Arteriovenous malformations of the dental arcades. The place of endovascular therapy: results in 12 cases are presented.

Twelve arteriovenous malformations of the dental arcades (AVMDAs) (seven mandibular and five maxillary) were seen in our institution between 1977 and 1997. All these lesions consisted of true arteriovenous shunts (of either nidus or fistulous type) involving the bone, with or without soft tissue extension. Haemorrhage was present in eight patients (67%); either torrential, necessitating emergency embolization, or recurrent and progressive. Teeth instability was detected in all patients and was the origin of the bleeding. All lesions were embolized. Lesions in nine patients were embolized with Polyvinyl Alcohol Particles (PVA): this helped to stabilize the situation but could not avoid recurrences in all patients, necessitating complementary embolizations and or surgery. The use of acrylic glue (N-Butyl-Cyano-Acrylate [NBCA] Histoacryl) as the embolic agent has changed the results obtained tremendously. Eight patients have been treated with NBCA (five as complementary therapy to PVA during later sessions and three at the first attempt); injection either via the transarterial route or direct transcutaneous puncture (four patients) achieved a cure in four of these lesions (34%) with stability at long-term follow-up of all the other AVMs. Embolization with glue represents the therapy of choice in these sometimes life-threatening lesions, achieving a cure if directed towards the osseous venous lakes. Surgery, often leading to facial mutilation and necessitating massive reconstruction should be avoided nowadays, at least as the initial therapy.

Transvenous embolisation of an arteriovenous malformation of the mandible via a femoral approach.

Arteriovenous malformations (AVM) of the mandible are uncommon but can give rise to sudden massive haemorrhage. Transarterial or direct transosseous embolisation can be used to treat this condition but is not always effective. We describe a case of mandibular AVM with a single draining vein which was embolised successfully via a femoral transvenous approach.

Sentinel transoral hemorrhage from a pseudoaneurysm of the internal maxillary artery: a complication of CT-guided biopsy of the masticator space.

A patient presented with transoral hemorrhage 3 months after CT-guided percutaneous biopsy of the masticator space, prompting concern about sentinel hemorrhage from impending carotid artery rupture related to prior radical head and neck surgery and radiation therapy. Angiographic evaluation showed the internal carotid artery to be normal but demonstrated a pseudoaneurysm of the buccal branch of the internal maxillary artery exactly corresponding to the site of prior fine-needle biopsy.

The diagnostic and therapeutic role of angiography in lingual arterial bleeding.

Selective external carotid angiography proved effective in the diagnosis of lingual arterial bleeding in seven patients. The cause of the hemorrhage was carcinoma of the tongue in six patients and stab wounds in one. Therapeutic Gelfoam embolization controlled hemorrhaging in four patients.