The Spectrum of Cavernous Sinus and Orbital Venous Thrombosis: A Case and a Review

Orbital venous pathologies encompass a broad range of entities including tumors, shunts, congenital anomalies, aneurysms, and obstructive lesions. Patients may present with a variety of clinical findings which may include a combination of tumefaction, vascular engorgement, orbital pulsation, and exophthalmos, depending on the relationship between the lesion and the vascular system. Clinical findings may be unreliable in excluding serious underlying disorders, and so an extensive clinical and radiologic evaluation is necessary. This article presents a rare case of spontaneous aseptic cavernous sinus-superior ophthalmic vein thrombosis in a woman on hormone replacement therapy, and illustrates the multidisciplinary approach in diagnosis and management. The literature on issues surrounding this case is reviewed.     

Intradural extramedullary cavernoma of a lumbar nerve root mimicking neurofibroma. A report of a rare case and the differential diagnosis

Background contextIntradural extramedullary (IDEM) cavernomas are rare vascular malformations. They are well-circumscribed dark berry-like lesions with a histologic appearance of sinusoidal vascular channels. Neurofibromas are the most common IDEM tumors, originating from all nerve elements and leading to firm enlargement of the affected nerve root. These lesions are completely different; however, they may involve the spinal nerve roots or the major nerve trunks. Any similarities in clinical findings are based on different pathophysiology.PurposeTo present a rare resemblance of an IDEM cavernoma to a neurofibroma.Study designThis is a case report with review of the literature focused on the differential diagnosis.MethodsA 79-year-old patient presented with acute sensorimotor disturbance from L2–S1 levels. The investigations showed an L2–L3 lesion occupying the canal. Findings resembled a neurofibroma and a surgical resection was decided.ResultsThe complete surgical resection revealed a vascular lesion originating from a nerve root. The histology confirmed an IDEM cavernoma. This is a unique case as such a clinical resemblance and a macroscopical appearance has not been reported for an IDEM cavernoma as yet. The patient showed full postoperative recovery from his initial symptoms.ConclusionsIntradural extramedullary cavernoma is a rare cause of compression to spinal cord or nerve roots. Its manifestation characteristics are well defined and should always be part of the differential diagnosis. Intraoperative findings aid the diagnosis in nontypical cases before the final histology. The nontraumatic and nerve tissue sparing surgical resection warrants optimal postoperative results and excellent prognosis.     

Angiofibroma of the larynx: report of a case with clinical and pathologic literature review

BACKGROUND: Angiofibromas are uncommon vascular tumors with a strong predilection for the nasopharynx of adolescent males. Although they are slow growing and histologically benign, they have the potential to cause significant morbidity with laryngeal involvement. METHODS: We describe the clinical characteristics, histopathologic findings, differential diagnosis, preoperative evaluation, and management of a case of laryngeal angiofibroma. RESULTS: The patient was initially seen with a 2 1/2-year history of progressive dyspnea and dysphagia. Preoperative evaluation suggested a vascular mass involving the left supraglottic larynx. A partial laryngopharyngectomy was performed without complication. The patient is alive and disease free 3 years postoperatively. Final histopathologic diagnosis is consistent with angiofibroma. CONCLUSIONS: Laryngeal angiofibroma is an extremely rare entity. Adequate preoperative imaging is necessary to confirm the vascularity of this lesion, because ill-planned biopsy may lead to significant blood loss. The role of preoperative embolization of other laryngeal vascular lesions has been well documented and may be useful in the management of laryngeal angiofibroma.     

Use of bisphosphonate to manage avulsion fracture of tibial tuberosity with underlying angiomatosis: a case report

An unusual case of avulsion fracture of the tibial tuberosity with underlying angiomatosis in a middle-aged man is described, with particular emphasis on the successful use of bisphosphonate in its treatment and the value of serial dual energy X-ray absorptiometry in its subsequent disease monitoring. This case illustrates the importance of careful correlation of the clinical, radiological, and histological findings in the management of skeletal tumour and tumour-like lesions. The differential diagnosis of osteolysis with vascular ectasia is discussed.     

Severe necrotizing mastopathy caused by calciphylaxis alleviated by total parathyroidectomy

Calciphylaxis is a complication caused by secondary hyperparathyroidism in patients with chronic renal failure. These patients may present with clinical findings of ischemic necrosis involving the skin and muscle resulting in subsequent gangrene and vascular calcifications. We report a rare case of necrotizing mastopathy caused by calciphylaxis in a 70-year-old female with end-stage renal disease whose symptoms resolved with a total parathyroidectomy.     

Delayed Presentation of Superficial Femoral Artery Injury: Report of a Case

We describe herein a patient who developed serious complications following a penetrating injury to the lower limb. There was minimal evidence of vascular injury on the initial presentation at the hospital; in particular the ankle systolic pressure was normal. Fourteen days following the initial injury, he was found to have a pseudoaneurysm of the superficial femoral artery associated with the arteriovenous fistula in his left thigh. The findings of this case suggest that a high index of suspicion and a careful clinical review is essential if vascular injuries and their complications are not to be missed. Received: May 8, 2000 / Accepted: September 26, 2000     

Brachial thrombosis in a premature neonate. A case report

Arterial injuries in infants are rare and in most cases are of iatrogenic origin as a consequence of catheterization, venipuncture or arterial blood sampling. These lesions require an accurate, noninvasive clinical diagnosis and prompt exploration and reconstruction using microvascular techniques to restore perfusion and to avoid morbidity and even mortality. We present a 3-day-old infant with a brachial artery thrombosis by a white thrombus as a consequence of an unsuccessful attempt to introduce an intravascular catheter. A microvascular reconstruction was performed, with complete restoration of the blood flow. Any suspected vascular injury needs immediate clinical and diagnostic assessment in order to avoid potential life-threatening complications. Surgery is mandatory in case of extensive arterial injuries, in case of inadequate distal blood supply or in case of progressive worsening of ischemic clinical findings.     

An unusual case of a large cavernous hemangioma invading the left ventricular apex

Cardiac hemangiomas are extremely rare benign vascular tumors of the heart. We present the unusual case of a 60-year-old man with a large cavernous hemangioma involving the left ventricular apex. The tumor was completely excised under cardiopulmonary bypass. The pertinent literature is reviewed and the technical steps, clinical presentation, and histopathologic findings are discussed.     

Fracture consolidation in a tibial nonunion after revascularization: a case report

Poor vascularity can be a potential contributor in the development of fracture nonunion. There is little evidence in orthopaedic literature suggesting that poor vascularity alone may lead to the development of nonunion. Experimental models addressing the effects of ischemia on fracture healing have yielded conflicting views. The case presented here describes a patient who sustained a Type IIIC tibial shaft fracture requiring vascular repair and soft tissue coverage. The patient developed an aseptic, atrophic nonunion. In preparation for operative management of her nonunion, the patient underwent angioplasty in the lower extremity for stenotic vessels. Shortly thereafter, the patient's nonunion went on to unite with no additional intervention, suggesting that revascularization alone resulted in this dramatic progress to union. Further prospective clinical studies may reveal a role for the use of vascular evaluation and intervention in the diagnosis and treatment of nonunion.     

Ankle swelling in a 6-year-old boy with unusual presentation: Report of a rare case

A 6-year-old boy with a history of pain and swelling in the right ankle was referred to our medical center. A cascading algorithm helped us to better evaluate this case of an osteochondroma of talus. The diagnosis was based on clinical and radiologic findings and on histopathologic analysis of the excised tissue. Although osteochondroma, or osteocartilaginous exostosis, is a common skeletal neoplasm, it is rarely seen in the foot. The rare location, a young age group, and an inexperienced surgeon may make the diagnosis confusing. We presented a case that shows the importance of an orderly evaluation of a musculoskeletal neoplasm. This may be helpful for the physician to better understand the clinical implications of any case before attempting a surgical intervention.     

Ultrasound Findings of an Intratesticular Varicocele. Report of a New Case and Review of the Literature

The authors present a new case of intratesticular varicocele(ITV). The ultrasound findings on gray-scale and color Doppler imaging as well as the pertinent clinical manifestations are discussed. A review of the literature is also presented. According to our knowledge only 34 cases of ITV have been reported in the literature and the clinical significance of this lesion is not yet well established. This is the first reported case of infertility caused by a solely ITV, showing that the clinical implications of this entity may be more significant than previously thought.     

Focal and segmental glomerular sclerosis (FSGS) in a man and a woman with Fabry's disease

We describe a man and a woman with Fabry's disease. Renal biopsies showed late and early stages respectively of focal and segmental glomerulosclerosis (FSGS) and vascular changes. Clinically the hemizygous patient had advanced renal disease with nephrotic range proteinuria and serum creatinine 122 micromol/l. The female carrier had minimal albuminuria, borderline GFR with a normal serum creatinine, acroparesthesias, moderate fatigue, tinnitus and headache accompanied by ischemic cerebral lesions. Enzyme replacement therapy (ERT) was initiated according to our Fabry protocol, partly due to the renal morphologic findings. We conclude that FSGS and vascular changes may be an early morphologic finding in Fabry's disease, even in patients with subtle albuminuria. The potential role of FSGS as a marker of progressive renal disease in some Fabry patients is discussed. As FSGS and vascular changes obviously may exist across a wide range of clinical presentations and have potential prognostic implications, we suggest that a renal biopsy should be performed prior to enzyme replacement therapy in all adult Fabry patients with proteinuria of various levels. Efforts should be made to develop a scoring system to evaluate potential histologic markers. Protocol biopsies may have therapeutic implications and may provide valuable information in the evaluation of start and dosing of ERT.     

Left partial anomalous pulmonary venous connection found during a lobectomy for lung cancer: Report of a case

We report herein the case of a 68-year-old man in whom a partial anomalous pulmonary venous connection (PAPVC) was found during an operation for primary lung cancer. The preoperative clinical findings did not suggest a vascular shunt, and intraoperatively the anomalous vein was seen to drain only from the left upper lobe into the left innominate vein. The lower pulmonary vein connected normally, and there was no atrial septal defect nor any other anomalous condition. A left upper lobectomy with ligation of the anomalous connected vein was performed uneventfully. This type of PAPVC is extremely rare, and is especially noteworthy because there were no clinical signs.     

Systemic cystic angiomatosis in a woman with hematuria and splenomegaly

Cutaneous hemangiomas are frequently found on routine clinical examination and generally are not important. But when internal organs are involved, these vascular tumours assume greater importance because of associated morbidity. A case is presented of multiple organ involvement by angiomas in a woman with a history of flank hemangioma who was investigated for hematuria and splenomegaly. She subsequently underwent splenectomy. The uncommon systemic syndromes characterized by vascular tumours are discussed, and the case reported is appropriately classified. The clinical picture, pathologic features and investigations appropriate for systemic cystic angiomatosis are briefly reviewed. The authors recommend conservative management when there is splenic involvement unless the size of the spleen makes its rupture likely or when there are hematologic complications.     

Congenital mesenteric defect: Description of a rare cause of distal intestinal obstruction in a neonate

INTRODUCTIONInternal hernias are a rare cause of bowel obstruction in the neonate and present with bilious vomiting. Newborns may be at risk of loss of significant length of bowel if this rare condition is not considered in the differential diagnosis of bilious emesis.PRESENTATION OF CASEWe report a case of a twin with an internal hernia through a defect in the ileal mesentery who presented with neonatal bowel obstruction. The patient had a microcolon on the contrast enema suggesting that the likely etiology was an intra-uterine event most likely a vascular accident that prevented satisfactory meconium passage into the colon.discussionAn internal hernia is rarely considered in the differential diagnosis of distal bowel obstruction in a neonate with a microcolon. Congenital trans-mesenteric hernias constitute only 5–10% of internal hernias. True diagnosis of trans-mesenteric hernias is difficult due to lack of specific radiology or laboratory findings to confirm the suspicion.conclusionWhen clinical and radiological findings are not classical, rare possibilities such as an internal hernia must be considered in the differential diagnosis, to avoid catastrophic bowel loss.     

Intramuscular vascular malformation of the infrazygomatic and temporal fossa

Intramuscular vascular anomalies are uncommon tumors in the head and neck region. The majority are in the masseter and trapezius, but they are rare in the temporal muscle. A rare case of an intramuscular vascular malformation of the temporalis muscle is reported. The clinical presentation, radiographic and histologic findings, treatment and outcome of this patient are presented. Received: 25 March 1997 / Accepted: 2 June 1997     

Diagnosis of vascular prosthesis infection with FDG-PET/CT

Fluorodeoxyglucose positron emission tomography (FDG-PET) is on the verge of becoming an established imaging tool in the fields of clinical oncology, cardiology, and neurology. Because of the high glucose uptake of inflammatory cells, FDG scanning is an appropriate tool for use in tracing suspected inflammation or to evaluate infection. PET, although highly sensitive, often lacks the ability to define the precise anatomic location of abnormal FDG accumulation. The new PET/computed tomography (CT) technology provides precise registration of metabolic and structural imaging data in a single session. We report positive FDG-PET/CT findings in an infected vascular prosthesis 6 months after grafting. Our experience and a few available case reports support the hypothesis that FDG-PET/CT may have a promising role in future noninvasive diagnosis of infected vascular grafts.     

Angiomyxolipoma (Vascular Myxolipoma) of the Oral Cavity. Report of a Case and Review of the Literature

Angiomyxolipoma is a rare tumor characterized by a proliferation of adipose tissue associated with a myxoid stroma and multiple vascular channels. To date, ten cases of angiomyxolipoma located in subcutaneous tissue, spermatic cord and subungual area have been reported in the literature. We describe the clinical and histopathologic findings of the first case of intraoral angiomyxolipoma in a 12 year-old male, as well as the utility of immunohistochemistry as a useful tool in differential diagnosis of this particular lesion with respect to other benign and malignant lesions derived from adipose tissue.     

Cutaneous ectopic breast calcification in a haemodialysis patient

This case report details the unique breast calcification and swellings of a 61-year-old lady. The ectopic breast calcification was a result of chronic renal failure and hyperparathyroidism secondary to vesiculo-ureteric reflux nephropathy. Subcutaneous nodules were also noted around her ileostomy (from surgery due to Crohn's disease) and abdominal wall. Mammogram revealed calcified breast discs and lobules with extensive vascular calcification. Histological biopsies revealed extensive calcium deposition (benign). Cutaneous deposition of calcium oxalate has been described in 13 patients world wide, usually involving fingers, ears, or nose. Two of these report breast involvement, but this is described as less extensive and more focal calcification on clinical findings and mammography. This case report describes a rare condition with extraordinary mammographic images.     

Carotid artery thrombosis and delayed stroke associated with the use of a shoulder belt in a teenager

Blunt carotid artery injury caused by shoulder belts is rare and requires a high index of suspicion to diagnose before the onset of neurologic sequelae. Neurologic abnormalities may be erroneously attributed to concomitant head trauma, and the onset of neurologic abnormalities may be delayed for hours or days. The case of a young, teenaged driver with a “seatbelt sign” and presentation of signs and symptoms of carotid artery thrombosis 13 days after injury is described. Occult vascular injury should be suspected if neurologic deterioration occurs after a lucid interval and/or computed tomography of the head is inconsistent with neurologic findings. Screening criteria and possible screening modalities are discussed.