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[Orbital and endocranial complications in acute sinusitis in childhood].

Acute infection the paranasal sinus is a rather frequent pathology in children. On the contrary, local orbital complications are rare. The employment of antibiotics reduces the incidence of complications but can sometimes conceal their appearance so that diagnosis is delayed. Orbital as well as intracranial complications of ethmoidal and maxillary sinusitis are most often encountered in childhood. Within a brief time span, 8 patients with complicated frontal and ethmoid-maxillary sinusitis were diagnosed and treated: 4 cases of periorbital cellulitis, 2 of subperiorbital abscess and 2 of orbital abscess (one of which with cavernous sinus thrombosis). Particularly relevant from the diagnostic point of view was the employment of C.T. scan which furnished an accurate definition of the orbit and its surrounding structures which influenced treatment choice. Under antibiotic cover all patients but one (who had been treated very early and had responded immediately to intensive antibiotic therapy) were treated surgically and the clinical course of the disease showed significant improvement very shortly after surgical drainage.

Abscess↗

Cerebral venous thrombosis in children.

From May 1984 to April 1995, a total of 16 patients (12 females, 4 males) with cerebral venous thrombosis, diagnosed by computed tomography (CT), conventional cerebral angiography, magnetic resonance imaging (MRI) or magnetic resonance angiography (MRA), were reviewed retrospectively. The age ranged from 1 month to 16 years of age (average: 2.5 years) with 8 below 1 year of age. The presenting symptoms for infants were mental change (75%) and seizure (100%), mainly generalized (63%) in character. Associated illness was mainly closed head injury, diarrhea or dehydration. All infants had mild to severe motor handicap in a 1 to 10 year follow-up. In contrast, older children frequently presented with headache (37%) or consciousness change (50%), and were more frequently associated with sepsis or local infections. Four (50%) of them recovered completely, but two died and two were finally in a vegetative state. For the four patients with poor prognosis, all had severe initial insults and widespread sinus thrombosis. MRI and MRA are better than CT for the diagnosis of cerebral venous thrombosis. It was concluded that prognosis for venous thrombosis cases in infants is worse than in older children, but this also depends on the severity of initial insults. For infants who present with intractable seizures, cerebral venous thrombosis should be taken into account when the seizures are difficult to control.

Adolescent↗

Long-term prognosis in cerebral venous thrombosis. Follow-up of 77 patients.

BACKGROUND AND PURPOSE: Very little is known about the long-term outcome of patients with cerebral venous thrombosis (CVT), particularly regarding the risk of residual epilepsy and further thrombotic events. We retrospectively studied 77 patients with CVT diagnosed by angiography and/or MRI. METHODS: A cohort of 77 patients aged 18 to 77 (mean, 38.5) years with CVT, evaluated from 1975 through 1990, was followed up for a mean of 77.8 months (range, 14 to 204 months; median, 63 months). Information on death, neurological status, seizures, recurrent CVT, other thrombotic events, and subsequent pregnancies was obtained from direct observation, mail questionnaire, or telephone interviews. RESULTS: Sixty-six of 77 patients (85.7%) had no neurological sequelae during follow-up. Eleven patients (14.3%) remained neurologically impaired. Two who initially presented with isolated intracranial hypertension had blindness due to optic atrophy. The other 9 had focal signs at the time of CVT and were left with various cognitive or focal deficits. Four of 28 (14.3%) patients who had seizures at the acute stage had recurrent seizures. One of the 51 patients with lateral sinus thrombosis developed a dural arteriovenous fistula. Nine of the 77 patients (11.7%) suffered a second CVT, all but one in the first year. Noncerebral thrombotic events occurred in 11 patients (14.3%). No recurrence of CVT occurred during later pregnancies, but 1 patient had a postpartum deep vein thrombosis. CONCLUSIONS: In the present series, CVT has an essentially good long-term prognosis. The frequency of long-standing epilepsy was low, suggesting that long-term anticonvulsant treatment is not necessary in the majority of cases. A second CVT or another thrombotic episode occurred in 20% of patients, stressing the need in a minority of cases for long-term anticoagulation.

Adolescent↗

Alcohol embolization of carotid-cavernous indirect fistulae.

OBJECTIVE: Carotid-cavernous fistulae (CCFs) are abnormal communications between the carotid artery and cavernous sinus that may present with rapid visual deterioration and extraocular paresis as a result of increasing intraocular pressure requiring emergent treatment to preserve vision. We present a technique of balloon-assisted ethanol embolization of the cavernous carotid artery supply to indirect CCFs providing immediate reduction in intraocular pressure with symptomatic improvement. METHODS: We reviewed clinical and angiographic data and present a retrospective case series illustrating six patients who underwent endovascular embolization because of worsening visual acuity and extraocular motility disorder caused by CCFs. Cerebral angiography revealed significant blood supply from the cavernous carotid artery to these CCFs. We performed ethanol embolization of these branches with distal balloon protection. RESULTS: Five of the six patients experienced immediate and sustained (mean follow-up, 21 mo) decreases in intraocular pressure, with significant symptom improvement. One patient experienced cavernous sinus thrombosis after conclusion of embolization, which caused a temporary worsening of symptoms that improved gradually over time. CONCLUSION: Many surgical and endovascular options are available to treat indirect CCFs. Absolute ethanol is a liquid agent that causes immediate vessel sclerosis and occlusion, which makes it a dangerous but potent liquid embolic agent. With distal temporary balloon protection to prevent migration of ethanol, we achieved excellent clinical and angiographic results using absolute ethanol to embolize the cavernous carotid supply to indirect CCFs. This represents a safe and effective method of endovascular management of this complex vascular anomaly.

Aged↗

Rhinocerebral mucormycosis presenting as periorbital cellulitis with blindness: report of 2 cases.

Two cases of rhinocerebral mucormycosis in elderly, non-ketotic diabetics who were initially diagnosed and treated for bacterial periorbital cellulitis are reported. Both presented with a short history of periorbital pain and swelling followed rapidly by complete ophthalmoplegia and blindness. By the time of correct diagnosis, both cases were advanced with lower cranial nerve involvement, CT evidence of ophthalmic artery and cavernous sinus thrombosis and, in one, internal carotid artery invasion (demonstrated on MR angiography) with resultant cerebral infarction. One patient was treated with intravenous amphotericin B but died within a few days. The second patient had aggressive surgical resection and survived with significant residual morbidity. These cases illustrate that mucormycosis should be excluded in any diabetic patient presenting with orbital cellulitis, especially when there is early visual loss. Early aggressive treatment with surgery and antifungal agents is often successful whereas the outcome is almost universally fatal when the diagnosis is delayed.

Aged↗

Pneumococcal meningitis in adults: spectrum of complications and prognostic factors in a series of 87 cases.

Studies on the incidence and spectrum of complications and prognostic factors in adults with pneumococcal meningitis are scarce. Therefore, we analysed 87 consecutive cases who were treated in our department between 1984 and 2002. Meningitis-associated intracranial complications developed in 74.7% and systemic complications in 37.9% of cases. Diffuse brain oedema (28.7%) and hydrocephalus (16.1%) developed more frequently than previously reported. The incidences of arterial (21.8%) and venous (9.2%) cerebrovascular complications were also very high. Furthermore, 9.2% of cases developed spontaneous intracranial haemorrhages (two patients with subarachnoid and two with subarachnoid and intracerebral bleedings, all in association with vasculitis; one subject with intracerebral haemorrhage due to sinus thrombosis; and three cases with intracerebral bleedings of unknown aetiology). Other new findings were the incidence of acute spinal cord dysfunction due to myelitis (2.3%) and that of hearing loss (19.5% of all patients and 25.8% of survivors). The in-hospital mortality was 24.1%. Only 48.3% of the patients had a good outcome at discharge [Glasgow Outcome Scale Score (GOS) = 5]. Outcome did not change during the study period, as mortality and GOS were similar for patients treated between 1984 and 1992 and for those treated between 1993 and 2002. Factors associated with a bad outcome (GOS </= 4) were chronic debilitating diseases, low Glasgow Coma Scale Score and focal neurological deficits on admission, low CSF leucocyte counts, pneumonia, bacteraemia and meningitis-associated intracranial and systemic complications. Low CSF leucocyte counts were also associated with the development of meningitis-associated intracranial complications. Age > or =60 years was associated with a higher mortality (36.7 versus 17.5%), but the GOS of the survivors was comparable to that of the surviving younger patients. The causes of death were mostly systemic complications in the elderly and cerebral complications in the younger patients. A haematogenous pathogenesis seemed likely in asplenic patients, while contiguous spread from sinusitis or otitis was the major cause of meningitis in non-asplenic individuals. Furthermore, asplenic patients had a raised incidence of meningitis-associated intracranial complications, but their outcome was similar to that of non-asplenic subjects. The morbidity and mortality of pneumococcal meningitis in adults are still devastating. We report higher incidences (diffuse brain swelling, hydrocephalus, cerebrovascular complications) or new incidences (myelitis, hearing loss, subarachnoid bleeding) of intracranial complications. Our detailed analysis of prognostic factors may help clinicians to identify patients at risk and may also be helpful in the design of clinical trials.

Acute Kidney Injury↗

Central nervous system complications secondary to oto-rhinologic infections. An analysis of 39 pediatric cases.

Fifty-eight central nervous system complications were noted in 39 pediatric patients with a primary oto-rhinological infection. The ages ranged from 1 to 15 years. Eleven patients (25%) had more than one complication. 12.8% of the cases died. Leptomeningitis was the most common intracranial complication (54%) Lateral sinus thrombosis (LST) occurring in 10 patients (26%) was accompanied with other intracranial abnormalities in 80% of cases. Brain abscess as an initial or concomitant complication was associated with the highest mortality rates (40%). In two of these fatal cases multiple brain abscesses were detected. The low incidence of intracranial infections secondary to oto-rhinologic infections and the masking effect of antibiotics present difficulties in the early recognition of the CNS complication. Despite the value of the modern imaging techniques in the investigation of the CNS complication, the clinical oto-rhinologic examination is of paramount importance in detecting the original infection in the pneumatic spaces of the upper respiratory tract. Additionally, bone scans were found of value in demonstrating the osteitic process of these cases.

Adolescent↗

Carotid-cavernous sinus fistula after trauma to the head.

The similarities between the various conditions described are remarkable. The orbital or frontal bruit and pulsating exophthalmos are common only to the carotid-cavernous fistula. A recent history of dental treatment, of facial infection with an elevated white blood cell count, and leukocytosis suggests cavernous sinus thrombosis. The superior orbital fissure syndrome and orbital apex syndrome occur most frequently in conjuction with Le Fort II and III fractures, which can involve the optic foramen and superior orbital fissure, therefore disrupting the sensory and motor innervation to the orbit and adjacent structures (Fig 4). Direct orbital trauma without evidence of fracture and with all symptoms mentioned, except infection, bruit, and pulsating exophthalmos, suggests orbital hematoma. A thorough differential diagnosis with complete data is essential for a correct diagnosis, thereby reducing morbidity by institution of the appropriate treatment. Treatments for carotid cavernous fistula have varied over the years--all aiming toward abolition of the fistula. Currently, the preferred treatment is the supraclinoid clamping of the internal carotid artery, followed by muscle embolization of the internal carotid artery distal to the bifurcation, and clamping of the internal carotid artery in the neck.

Arteriovenous Fistula↗

Allergic rhinitis as a risk factor for orbital complication of acute rhinosinusitis in children.

The purpose of this article is to determine allergic rhinitis and age as potential risk factors for the development of orbital complications of acute rhinosinusitis in children. One hundred two children presenting with orbital swelling were investigated by computed tomography (CT) of the paranasal sinuses and the orbit as well as for underlying allergic rhinitis. Sixty (58.8%) patients had orbital complications of clinical and radiological acute rhinosinusitis. They were grouped accordingly: preseptal cellulitis (n = 24), periostitis (n = 10), and subperiosteal abscess (n = 26). No abscess within the orbit or cavernous sinus thrombosis was found. Thirty-four (56.7%) of the 60 patients underwent allergy investigation. Allergic rhinitis was found in 9 (64.3%) of 14 children with preseptal cellulitis, in 1 (25%) out of 4 children with periostitis, and in 13 (76.5%) out of 17 children with subperiosteal abscess. The prevalence of allergic rhinitis was significantly higher in patients presenting in pollen season from February to August (17:4) than in patients presenting in the period between September and January (6:7). Thus, allergic rhinitis may be a cofactor in the pathogenesis of orbital complications of acute rhinosinusitis. According to our study population, age only influences the type of orbital complication of acute rhinosinusitis in the sense that older children are more likely to develop subperiosteal abscess, whereas younger children develop preseptal cellulitis.

Acute Disease↗

Spontaneous thrombosis of a traumatic cavernous sinus fistula.

Post-traumatic thrombosis of cavernous sinus and orbital veins has been reported in conjunction with carotid-cavernous sinus fistula (CCSF). CCSF are abnormal communications between the carotid artery and the cavernous sinus. Spontaneous thrombosis of a CCSF is a rare event mainly seen in the indirect (dural) type of fistula. We report on a patient with a possible post-traumatic CSSF which underwent partial spontaneous thrombosis and presented with an extreme degree of proptosis.

Adult↗

Anticardiolipin antibodies are frequently present in patients with idiopathic intracranial hypertension.

BACKGROUND: Anticardiolipin antibodies (ACL-Ab) are associated with various neurologic syndromes, but idiopathic intracranial hypertension (IIH) has only rarely been reported in this context. OBJECTIVES: To delineate the frequency and clinical and radiological features of, as well as the cause-and-effect relationship between, ACL-Ab and IIH. METHODS: We analyzed the medical records of patients with IIH hospitalized between January 1989 and September 1995. All patients underwent magnetic resonance imaging or magnetic resonance venography or angiography. Excluded were patients with intracranial hypertension due to dural sinus thrombosis or traumatic, structural, neoplastic, or infectious disorders. Patients who were found on at least 2 separate occasions to have increased IgG titers of ACL-Ab were identified and compared with patients without ACL-Ab. RESULTS: Six (43%) of 14 patients with IIH had ACL-Ab. No differences in clinical, laboratory, or radiological variables could be found between patients with and without ACL-Ab. Only 3 of the 11 ACL-Ab-positive patients had previous systemic or neurologic abnormalities associated with ACL-Ab. CONCLUSIONS: Anticardiolipin antibodies may cause IIH through mechanisms unrelated to major venous thrombosis. Idiopathic intracranial hypertension is frequently associated with ACL-Ab and can be the presenting symptom of the antiphospholipid syndrome. There are no major clinical, laboratory, or radiological features that distinguish between patients with IIH with and without ACL-Ab.

Adolescent↗

Visual loss in pseudotumor cerebri of childhood. A follow-up study.

The occurrence of visual loss in substantial numbers of adult patients with pseudotumor cerebri is well recognized. In children, the disease has been said to spare the visual system. We evaluated the ophthalmologic features of 36 children with pseudotumor cerebri followed up for one to 12 years. Four patients had rapid, severe loss of visual acuity and visual field changes while receiving medical therapy. Six others had less-severe but prominent abnormalities of visual acuity and/or visual fields at some point during the disease process. Only one patient had a permanent, severe visual impairment, but five had moderate permanent visual abnormalities. A severe degree of papilledema was seen only in the group with visual loss, but some patients in this group had only moderate papilledema. More patients with dural sinus thrombosis had serious visual loss than did those with other associated diseases or idiopathic pseudotumor cerebri. Quantitative perimetry of some sort can be performed in most children and is the preferred sequential test for planning treatment.

Adolescent↗

Blindness and total ophthalmoplegia. A complication of transantral ligation of the internal maxillary artery for epistaxis.

Unilateral blindness and total ophthalmoplegia developed on the same side on which transantral ligation of the internal maxillary artery was performed for persistent posterior epistaxis. Evaluation with arteriograms and phlebograms showed no compromise of retinal vessels or cavernous sinus thrombosis. At reexploration of the maxillary antrum, marked swelling of previously placed oxidized cellulose gauze was found. An experimental model, using the rabbit, was designed to determine if implantation of oxidized cellulose gauze adjacent to the optic nerve would produce blindness and ophthalmoplegia.

Adult↗

Unusual complications of temporal bone fractures.

Eighty-two temporal bone fractures were diagnosed in 75 patients with high-resolution computed tomographic scanning. Excluding six gunshot injuries, 55 (72%) of the fractures were oblique, 11 (15%) were longitudinal, and ten (13%) were transverse. Facial paresis or paralysis occurred in 45 patients (60%), hemotympanum occurred in 67 (89%), and cerebrospinal fluid otorrhea occurred in 19 (25%). Among 66 patients in whom audiometry was performed, 20 (30%) had conductive hearing loss, nine (14%) had sensorineural loss, and 36 (55%) had mixed hearing loss. Vestibular symptoms were present in 23 patients (30%). Other unusual complications of temporal bone fractures were observed: bilateral abducens paralysis, three patients (4%); unilateral abducens paralysis, two (2.67%); trigeminal paralysis, one (1.33%); and aseptic sigmoid sinus thrombosis, one (1.33%).

Abducens Nerve Injury↗

Paraneoplastic cholestasis and hypercoagulability associated with medullary thyroid carcinoma. Resolution with tumor debulking.

The authors report a 69-year-old woman with a hypercoagulable state manifesting as superior sagittal sinus thrombosis, thrombocytosis, right lower extremity deep venous thrombosis, and subsequent pulmonary embolus. The liver enzyme values were elevated in a cholestatic pattern. Carcinoembryonic antigen level was markedly elevated. Evaluation revealed that her longstanding "goiter" had slowly enlarged during the past 6 years. The serum calcitonin level was markedly elevated. Subsequent biopsy revealed medullary thyroid carcinoma. Surgical debulking of the tumor and lymph nodes resulted in substantial reduction of the calcitonin and carcinoembryonic antigen levels in a matter of days. Long-term follow-up revealed normalization of cholestasis and resolution of the hypercoagulable state. Review of the literature revealed no previously reported cholestasis or hypercoagulable state associated with medullary thyroid carcinoma. The literature on paraneoplastic cholestasis, carcinoembryonic antigen production, and hypercoagulable states is reviewed.

Aged↗

Thrombocytosis as a presenting feature of acute lymphoblastic leukemia in childhood.

To determine the incidence of thrombocytosis at presentation in acute lymphoblastic leukemia (ALL), medical records of all children diagnosed at the Children's Hospital of Pittsburgh from 1980 to 1987 were reviewed. Out of 217 such patients, 7 (3.2%) had platelet counts greater than 400,000/mm3. All of the seven were boys compared with a male:female ration of 1.4:1 in the entire ALL population. Other than sex, no characteristics were clearly associated with thrombocytosis, including white blood cell count, hemoglobin, lymphoblast morphology, and immunologic or chromosomal markers. Apart from ALL, no inflammatory or infectious process which might have caused a thrombocytosis, was detected in any of these patients. The period of induction therapy was notable for the preservation of platelet counts greater than 20,000/mm3 in all patients. However, of the seven children with thrombocytosis, two had major induction complications: one, a cavernous sinus thrombosis; and the other, gastrointestinal bleeding with duodenal perforation. We conclude that thrombocytosis at diagnosis can be seen in children, particularly boys, with ALL. Based on small numbers, this group of patients may be at risk for major events during induction therapy. Large numbers, longer follow-up, and platelet function studies on similar patients will be of interest.

Biopsy↗

New onset focal weakness in children with Down syndrome.

New onset focal weakness is relatively common in patients with Down syndrome (DS), and has broad differential diagnosis. Ten cases of new onset focal weakness in patients with DS were encountered or are currently being followed in two DS clinics, with a combined population of patients of approximately 850, for a clinic population prevalence of 1.2%. The median age at presentation was 4 years old (range 1 month-44 years). The causes of new onset focal weakness were: stroke from Moyamoya disease (two patients); stroke from vaso occlusive disease (one patient); stroke from venus sinus thrombosis (one patient); traumatic subdural hematoma (one patient); brain abscess (one patient); spinal cord injury (SCI) from cervical spinal stenosis (two patients); SCI from atlantoaxial instability (AAI) (one patient); and brachial plexus injury (one patient). Of the 10 patients with focal weakness, 8 had potentially treatable conditions, and 5 had surgery. The differential diagnosis of new onset focal weakness in DS is broad, with diseases reported involving all levels of the nervous system from brain to muscle. For some diagnoses, expeditious diagnosis may improve outcome.

Adolescent↗