MRI findings in a child with sigmoid sinus thrombosis following mastoiditis

We describe the MRI features of sigmoid sinus thrombosis following mastoiditis in a 3-year-old girl. The features consisted of increased signal from the sinus on T2-weighted images and absence of flow on MR venography. It is concluded that MRI enabled a timely diagnosis of this life-threatening disease. MRI, as a non-invasive technique that does not use ionizing radiation, should be considered the investigation of first choice, especially in young patients.     

Frecuencia inusualmente elevada de complicaciones secundarias a otitis media aguda

IntroductionIntracranial complications (ICC) secondary to otitis media are unusual but potentially life-threatening. We report an unusually high frequency of these events, and describe their clinical and epidemiological features.Material and methodA retrospective study of all pediatric patients with ICC admitted to our tertiary hospital from April 2004 to November 2007.ResultsEight patients had ICC: Sigmoid sinus thrombosis (4), lateral sinus thrombosis (1), meningitis (2), epidural abscess (1), otitic hydrocephalus (1). Four of the patients had acute mastoiditis. Pre-admission oral antibiotics were administered in 87.5% of the patients. Fever, otalgia, headache and VI and VII cranial nerves paralysis were the most frequent symptoms associated. The microorganism could be isolated in 3 patients: S pyogenes (2) and P mirabilis (1); the remaining patients had negative cultures. All of the patients received broad-spectrum parenteral antibiotics and 2 of them underwent a radical mastoidectomy.ConclusionsWe report an unusually high incidence of cranial complications secondary to acute otitis media. Multicenter studies are needed in order to assess whether there is an increase in the incidence of these events.     

Gradenigo's syndrome with lateral venous sinus thrombosis: successful conservative treatment

Gradenigo's syndrome (GS) is a rare disease characterised by the triad otitis media, pain in the region innervated by the first and the second division of trigeminal nerve and abducens nerve palsy. Septic sinus thrombosis is one of the most frequent and relevant complication of GS; it is often due to persistent damage and late diagnosis. Computed tomography (CT) scan and magnetic resonance imaging (MRI) allow the correct diagnosis in most cases. Surgical therapy may be necessary for a better and more rapid resolution of the disease. We report the case of a 4-year-old child that was admitted for facial nerve palsy and abducens nerve palsy subsequent to a 2-week persistent pain in the right ear. MRI showed infective acute process of the right mastoid and partial ipsilateral sinus thrombophlebitis. The child was treated with high-dose intravenous antibiotics and with oral anticoagulants. A complete resolution of symptoms and radiological alterations were observed within 7 weeks. In conclusion, lateral sinus thrombosis and Gradenigo's syndrome are rare but potential fatal complications of otitis media and mastoiditis. High-dose intravenous antibiotics and a low dose of anticoagulant can achieve a complete recovery without surgery.     

Cerebral sinus occlusion in a boy presenting with asparaginase-induced hypertriglyceridemia

Cerebral sinus thrombosis is a rare but severe complication during treatment for acute lymphoblastic leukaemia (ALL). It mostly has been reported during treatment with asparaginase and dexamethasone. Hypertriglyceridemia has - albeit very rarely - also been associated with asparaginase therapy. The combination of cerebral sinus thrombosis and hypertriglyceridemia however, has not yet been reported. Here we describe a 15-year-old boy who presented with clinical symptoms and radiologic findings of a cerebral sinus thrombosis. In addition, a life-threatening hypertriglyceridemia was present. The complication was successfully treated by anticoagulation with low molecular weight heparin and the lipid regulator bezafibrate.     

Spontaneous dural sinus thrombosis in children

The clinical course of spontaneous dural sinus thrombosis in children varies from indolent to fulminant. Although many different etiologies for the development of dural sinus thrombosis have been described, a full recovery can be anticipated in most children following rehydration and the administration of systemic antibiotics. Steroids, systemic anticoagulation and intrasinus thrombolysis may be beneficial in selected patients, although the efficacy of these therapies has not been established prospectively in children. We reviewed 12 pediatric patients with spontaneous dural sinus thrombosis (1978-1998) to determine the etiology, clinical course and best treatment options. In the absence of a hypercoagulable state, pediatric patients generally recover well with rehydration and antibiotics and do not require anticoagulation.     

Sigmoid sinus thrombosis as a complication of otitis media

Complications of otitis media are relatively infrequent, especially since the introduction of effective antimicrobial agents and specific surgical techniques. We present a case of cerebral pseudotumor due to left sigmoid sinus thrombosis as an intracranial complication of otitis media. We review the various clinical manifestations of sigmoid sinus thrombosis and its therapeutic options.     

儿童原发性肾病综合征合并颅内静脉血栓4例病例报告

目的 通过对原发性肾病综合征合并颅内静脉血栓患儿行临床分析,探讨儿童NS合并颅内静脉血栓早期诊断和治疗的可行方案。方法 纳入2012年1月至2015年9月在上海市儿童医院住院的原发性肾病综合征且经头颅CT和（或）MRI确诊的颅内静脉血栓患儿,对其临床症状、实验室指标、影像学检查结果、疗效及预后进行分析。结果 4例原发性肾病综合征合并颅内静脉血栓患儿进入分析,均为男性,年龄5岁4个月至11岁4个月,出现颅内静脉血栓时间距原发性肾病综合征起病时间为1个月至7年余。4例颅内静脉血栓发病时均有神经精神系统症状,查体均未见神经系统阳性体征。3例在颅内静脉血栓发病期间D-二聚体、纤维蛋白原降解产物（FDP）均升高,抗凝血酶Ⅲ（AT-Ⅲ）下降;确诊后D-二聚体、FDP较前继续升高; 4例血清白蛋白明显降低,总胆固醇明显升高。4例出现颅内静脉血栓临床症状当日或次日均行头颅MRI增强+MRV+MRA检查,3例为左侧乙状静脉窦血栓,1例为脑栓塞。明确颅内静脉血栓诊断后,3例予尿激酶溶栓,低分子肝素钙和双嘧达莫抗凝治疗;1例脑栓塞患儿予对症和抗凝治疗;4例症状均明显改善。3例出院后6~12个月随访头颅MRI增强+MRV显示颅内异常信号均有不同程度吸收。结论 儿童原发性肾病综合征合并 颅内静脉血栓易发生于左侧乙状静脉窦。在原发性肾病综合征病程中如出现神经精神系统症状时,应及时行头颅MRI相关序列检查,有助于颅内静脉血栓早期诊断;早期积极溶栓治疗预后良好。     

Lemierre’s syndrome with double heterozygote status in the methylenetetrahydrofolate reductase gene

Background

There are some risk factors being more vulnerable to Lemierre’s syndrome such as a hypercoagulable state.

Methods

We report a rare case of Lemierre’s syndrome with ethmoid and maxillary sinusitis, bilateral mastoiditis, and sigmoid sinus thrombosis.

Results

Genetic study revealed a double heterozygote status in the methylenetetrahydrofolate reductase gene including C677T and A1298C.

Conclusion

It is suggested to screen patients with Lemierre’s syndrome for a hypercoagulable state to consider anticoagulant therapy.     

Mastoiditis,meningitis and venous sinus thrombosis caused byFusobacterium necrophorum

The authors report a case of septic venous sinus thrombosis (VST) and meningitis occurring as an early complication of mastoiditis caused byFusobacterium necrophorum. CT scan was normal, and cerebral angiography was required to diagnose the VST. The evolution was favourable with appropriate antimicrobial therapy and steroids.     

Pott's puffy tumor: a rare complication of mastoiditis

Pott's puffy tumor is rarely seen in the modern era of antibiotics. It is usually seen as intracranial complication following inappropriately treated frontal sinusitis. Up until 2001 there have been only 21 pediatric cases reported in the literature and most have been secondary to frontal sinusitis. Only 1 case has been reported following a latent mastoiditis and that has been in an adult [1]. We report a case in a child who developed Pott's puffy tumor following mastoiditis.     

Rare but threatening complication of otitis media: Lateral sinus thrombosis

Although appropriate use of antibiotics has decreased mortality, lateral sinus thrombosis is a rare, important intracranial complication of acute otitis media. Herein is described the case of a 5‐year‐old patient with lateral sinus thrombosis after acute otitis media. We emphasize the need to be alert for lateral sinus thrombosis when treating acute otitis media.     

Posttraumatic internal jugular vein thrombosis presenting as a painful neck mass in a child

An uncommon case of a pediatric traumatic internal jugular vein thrombosis is presented. A 7-year-old boy developed severe neck pain after falling from a bunk bed. Initially, the child was diagnosed and treated for a lymphadenitis with a possible abscess formation. Contrast-enhanced computed tomography and Doppler ultrasound imaging of the neck revealed the presence of an occlusive thrombosis of the left internal jugular vein. The patient was treated with intravenous antibiotics and followed closely over the next year. He had full resolution of his symptoms without the development of complications associated with this injury process. Internal jugular vein thrombosis is an uncommon and potentially life-threatening disorder caused by various conditions. This case illustrates the need for a systematic approach when evaluating neck masses, and internal jugular venous thrombosis should be included in the differential of anterior neck swelling.     

Severe <Emphasis Type="Italic">Fusobacteria</Emphasis> infections (Lemierre syndrome) in two boys

Abscess formation is a rare cause of febrile illness in childhood but always has to be considered in such clinical presentations. Belonging to the resident flora of the oropharyngeal region, Fusobacteria are known to cause local infections; from here they may extend to other sites via the bloodstream or are aspirated into the lung (Lemierre disease). We report on two boys with Lemierre disease due to infection by Fusobacteria in monoculture causing two different clinical phenotypes. Case 1 presented with a large subphrenic abscess and pneumonic infiltration of the right middle lobe. Primary focus of infection was periodontal disease. Case 2 presented with a life-threatening septicaemia due to a retropharyngeal abscess and perforated otitis media followed by osteomyelitis of the atlas and thrombosis of the left sigmoid sinus and internal jugular vein. CONCLUSION: Fusobacteria should be considered in any abscess formation in children. A thorough examination of the oropharyngeal region as a possible site of primary manifestation is mandatory.     

Hypopharyngeal or esophageal perforation in neonates

In spite of the many manipulations that are done in the upper airways of neonates, esophageal perforations are rare. But they may be life-threatening if not recognized and treated immediately. Along with a case history we present clinical and radiologic symptoms of a right sided pneumothorax and the feeding tube in the posterior phrenic recess. The principles of treatment are conservative with antibiotics and intravenous gavage feeding. Only with complications intensive radiography with contrast agents and surgery become necessary.     

Combined venous sinus angioplasty and low-dose thrombolytic therapy for treatment of hemorrhagic transverse sinus thrombosis in a pediatric patient

We describe the successful treatment of a 5-year-old girl with rapidly evolving left hemispheric hemorrhagic infarcts resulting from left transverse and sigmoid sinus thrombosis using combined endovascular dural sinus angioplasty and local low-dose thrombolytic therapy.     

Intracranial complications following mastoidectomy

Mastoidectomy is a common surgical procedure in otology. However, postoperative complications of various degrees of severity may occur. We present 4 children who underwent mastoidectomy for middle ear and mastoid disease and developed postoperative intracranial complications. One child was operated on for brain abscess 1 week after the initial mastoidectomy. Another child appeared with seizures 5 days after the initial mastoidectomy and a subdural empyema was drained during revision surgery. Large bone defects with exposed middle cranial fossa dura were found at revision surgery in both cases and Proteus vulgaris and methicillin-resistant Staphylococcus aureus were isolated from the mastoid and abscess cavities in these children. A small epidural collection was diagnosed in the third patient 2 days after initial mastoid surgery and was managed with intravenous antibiotics only. The other child was found to have sigmoid sinus thrombosis the day after mastoidectomy that was performed for nonresponsive acute mastoiditis. This child received both intravenous antibiotics and anticoagulants. Timely revision surgery, combinations of third- or fourth-generation cephalosporins with vancomycin or metronidazole and the addition of anticoagulants in cases of sinus thrombosis can lead to full recovery.     

Otitis media und ihre Komplikationen

Acute suppurative otitis media is one of the most common infections in childhood, caused by Streptococcus pneumonia, Haemophilus influencae, Staphylococcus or Pneumococcus infection via the Eustachian tube. Occasionally it can be blood-borne during viral or bacterial infections. With the improvement of tubal function as the child grows, the incidence of acute AOM decreases. Acute suppurative otitis media is a severe inflammation of the middle ear mucosa accompanied by severe pain, fever, reddening and bulging of the tympanic membrane which may perforate spontaneously. The course can be variable, but usually resolves without consequences. Diagnosis is made on the basis of otoscopic findings. It is important to promptly recognize possible complications such as mastoiditis, sinus thrombosis and brain abscess, which are rare but threatening situations. Treatment of acute suppurative otitis media consists of the application of topical decongestants, analgesics, antipyretics and antibiotics. In some cases paracentesis and adenoidectomy might be helpful. In the case of complications a surgical procedure is mandatory. As a residuum of acute otitis media a permanent perforation of the eardrum may result in chronic otitis media. Rarely, squamous epithelium may grow through the perforation into the middle ear and thus give rise to a cholesteatoma.     

Recombinant plasminogen activator therapy for cerebral vein thrombosis in a child carrier of prothrombin gene mutation

Venous thrombosis of transverse and sigmoid sinuses was diagnosed in a 3-year-old child who is a carrier of the G20210A prothrombin gene mutation. Recombinant tissue plasminogen activator (rt-PA) treatment was started 9 days following the onset of neurologic signs. Nine days of rt-PA therapy completely dissolved the thrombus. This case provides further evidence that rt-PA is useful and safe in children with thrombosis.     

Cerebral venous sinus thrombosis in children with acute lymphoblastic leukemia carrying the MTHFR TT677 genotype and further prothrombotic risk factors.

BACKGROUND: The present study was designed to prospectively evaluate the role of prothrombotic risk factors in leukemic children treated according to the ALL-BFM 90/95 study protocols with respect to the onset of cerebral venous sinus thrombosis. PATIENTS: 317 consecutive leukemic children aged 6 months to 18 years were enrolled in this study. 288 of the 317 patients were available for thrombosis-free survival analysis. RESULTS: In 17 (5.9%) of these 288 patients cerebral venous sinus thrombosis occurred. The overall event-free survival of thrombosis in the central nervous system in patients with at least one prothrombotic defect (n = 12) was significantly reduced compared with patients without a prothrombotic defect (p < 0.0001). 15 patients showed acute clinical symptoms at onset of cerebral venous sinus thrombosis, two were asymptomatic. Three of the 17 patients affected (17.6%) died directly associated with the thrombotic event during induction therapy, the remaining 14 patients did not show prolonged clinical symptoms. CONCLUSIONS: Prothrombotic risk factors should be included in a screening program in ALL children treated according to the BFM study protocols. Further prospective studies are recommended to establish adequate prophylactic anticoagulant treatment during ALL (BFM) polychemotherapy.