Download the Free Unbound MEDLINE PubMed App to your smartphone or tablet.
Available for iPhone, iPad, iPod touch, and Android.
- Progressive Postnatal Pansynostosis. [JOURNAL ARTICLE]
- Cleft Palate Craniofac J 2014 Oct 28.
Objective : To describe the subtle clinical features, genetic considerations, and management of progressive postnatal pansynostosis, a rare form of multisutural craniosynostosis that insidiously occurs after birth and causes inconspicuous cranial changes. Design, Participants, Setting : The study is a retrospective chart review of all patients diagnosed with progressive postnatal pansynostosis at a major craniofacial center between 2000 and 2009. Patients with kleebattschädel were excluded. Results : Nineteen patients fit our inclusion criteria. Fifteen patients had a syndromic diagnosis: Crouzon syndrome (n = 8), Saethre-Chotzen syndrome (n = 5), and Pfeiffer syndrome (n = 2). With the exception of one patient with moderate turricephaly, all patients had a relatively normal head shape with cranial indices ranging from 0.72 to 0.93 (mean, 0.81). Patients were diagnosed at an average of 32.4 months; craniosynostosis was suspected based on declining percentile head circumference (n = 14), detection of an apical prominence (n = 12), papilledema (n = 7), and worsening exorbitism (n = 3). Nearly all patients had evidence of increased intracranial pressure. Conclusion : Progressive postnatal pansynostosis is insidious; diagnosis is typically delayed because the clinical signs are subtle and appear gradually. All infants or children with known or suspected craniosynostotic disorder and a normal head shape should be carefully monitored; computed tomography is indicated if there is any decrease in percentile head circumference or symptoms of intracranial pressure.
- POEMS syndrome as an uncommon cause of papilledema. [Journal Article]
- Nepal J Ophthalmol 2014 Jan; 6(11):105-8.
Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome is an uncommon condition related to a paraneoplastic syndrome secondary to an underlying plasma cell disorder. Among the myriad of manifestations of the disease, ocular signs and symptoms are relatively prevalent, affecting about half of all patients with the disease.To report the ocular manifestations of POEMS syndrome.A 47-year-old lady diagnosed to have POEMS syndrome presented with painless progressive visual diminution. Her color vision was impaired. There was bilateral papilloedema.POEMS syndrome should be considered among the differential diagnoses of all patients with a bilateral papilledema in which no other cause can be readily elucidated.
- Papilledema Associated with Puberty. [JOURNAL ARTICLE]
- Clin Pediatr (Phila) 2014 Oct 15.
- The usefulness of multimodal imaging for differentiating pseudopapilloedema and true swelling of the optic nerve head: a review and case series. [JOURNAL ARTICLE]
- Clin Exp Optom 2014 Oct 14.
Ophthalmic practitioners have to make a critical differential diagnosis in cases of an elevated optic nerve head. They have to discriminate between pseudopapilloedema (benign elevation of the optic nerve head) and true swelling of the optic nerve head. This decision has significant implications for appropriate patient management. Assessment of the optic disc prior to the advanced imaging techniques that are available today (particularly spectral domain optical coherence tomography and fundus autofluorescence), has mainly used diagnostic tools, such as funduscopy and retinal photography. As these traditional methods rely on the subjective assessment by the clinician, evaluation of the elevated optic nerve head to differentiate pseudopapilloedema from true swelling of the optic nerve head can be a challenge in clinical practice with patients typically referred for further neuroimaging investigation when the diagnosis is uncertain. The use of multimodal ocular imaging tools such as spectral domain optical coherence tomography, short wavelength fundus autofluorescence and ultrasonography, can potentially aid in the differentiation of pseudopapilloedema from true swelling of the optic nerve head, in conjunction with other clinical findings. By doing so, unnecessary patient costs and anxiety in the case of pseudopapilloedema can be reduced, and appropriate urgent referral and management in the case of true swelling of the optic nerve head can be initiated.
- Perimetry, Retinal Nerve Fiber Layer Thickness and Papilledema Grade After Cerebrospinal Fluid Shunting in Patients With Idiopathic Intracranial Hypertension. [JOURNAL ARTICLE]
- J Neuroophthalmol 2014 Oct 7.
To investigate the effect of cerebrospinal fluid (CSF) shunting on quantitative perimetry and papilledema in patients with uncontrolled idiopathic intracranial hypertension (IIH).We retrospectively reviewed all cases of IIH with CSF shunting at our institution between 2004 and 2011. Perimetry was performed before and after surgery in 15 patients, and the mean deviation (MD) was compared before and after surgery to assess the effect of the intervention.Fourteen of the IIH patients were female and 1 was male. The average age was 34 years. CSF shunting resulted in significant improvement in the perimetric results with an increase in the MD of 5.63 ± 1.19 dB (P < 0.0001). Additionally, average retinal nerve fiber layer (RNFL) thickness measurement by optical coherence tomography decreased by 87.27 ± 16.65 μm (P < 0.0001), and Frisen papilledema grade decreased by 2.19 ± 0.71 (P < 0.0001).Our results suggest that CSF shunting results in improvement in perimetry, RNFL swelling, and papilledema grade in patients with IIH.
- [Diagnosis and treatment of cerebral venous sinus thrombosis.] [JOURNAL ARTICLE]
- Ugeskr Laeger 2014 Aug 11; 176(33)
Thrombosis of the cerebral sinuses most often affects younger adults. Headache is a common complaint and can be accom-panied by vomiting and papilloedema. The diagnosis rests on magnetic resonance imaging and the treatment consists of heparin or low-molecular weight heparin followed by vitamin K antagonists for three months or more. In fulminant cases local thrombolysis is administered, while symptomatic treatment for increased intracranial pressure is given. In retrospective analyses this approach has been associated with a good outcome - even in cases treated by decompressive craniectomy.
- Neuro-ophthalmic presentations and treatment of Cryptococcal meningitis-related increased intracranial pressure. [Journal Article]
- Can J Ophthalmol 2014 Oct; 49(5):473-7.
To illustrate three different ophthalmic presentations of cryptococcal meningitis (CM).CM is the most common manifestation of extra-pulmonary cryptococcosis. Intracranial hypertension occurs in up to 75% of patients with CM and is associated with increased mortality. CM can present to the ophthalmologist as vision loss, papilledema, abducens palsy, and/or other cranial neuropathies.We report three cases, two C. neoformans and one C. gattii, highlighting the various CM presentations. The first was a woman immunosuppressed following kidney transplantation in whom idiopathic intracranial hypertension (IIH) was initially suspected. The second was a man immunocompromised by previously undiagnosed HIV/AIDS who presented with signs and symptoms of increased intracranial pressure. The third case is an immunocompetent man with bilateral disc edema and an incomplete macular star diagnosed with presumed neuroretinitis. Further evaluation revealed positive CSF cryptococcal antigen with culture positive for C. gattii.Ophthalmologists should be aware that cryptococcosis can mimic more benign etiologies including IIH and neuroretinitis. Additionally, C. gattii, an emerging organism, can infect immunocompetent patients. In contrast to the typical treatment of increased ICP, serial lumbar punctures are recommended while acetazolamide and surgical CSF shunting may be harmful.
- Acute central retinal vein occlusion secondary to reactive thrombocytosis after splenectomy. [Journal Article]
- Case Rep Ophthalmol Med 2014.:930843.
The diagnosis and treatment of central retinal vein occlusion was reported in a young patient. Central retinal vein occlusion was probably related to secondary to reactive thrombocytosis after splenectomy. The patient was treated with steroids for papilledema and administered coumadin and aspirin. The symptoms resolved, and the findings returned to normal within three weeks. Current paper emphasizes that, besides other well-known thrombotic events, reactive thrombocytosis after splenectomy may cause central retinal vein occlusion, which may be the principal symptom of this risky complication. Thus, it can be concluded that followup for thrombocytosis and antithrombotic treatment, when necessary, are essential for these cases.
- Endoscopic endonasal optic nerve and orbital apex decompression for nontraumatic optic neuropathy: surgical nuances and review of the literature. [Journal Article]
- Neurosurg Focus 2014 Oct; 37(4):E19.
Object While several approaches have been described for optic nerve decompression, the endoscopic endonasal route is gaining favor because it provides excellent exposure of the optic canal and the orbital apex in a minimally invasive manner. Very few studies have detailed the experience with nontraumatic optic nerve decompressions, whereas traumatic cases have been widely documented. Herein, the authors describe their preliminary experience with endoscopic endonasal decompression for nontraumatic optic neuropathies (NONs) to determine the procedure's efficacy and delineate its potential indications and limits. Methods The medical reports of patients who had undergone endoscopic endonasal optic nerve and orbital apex decompression for NONs at the Lyon University Neurosurgical Hospital in the period from January 2012 to March 2014 were reviewed. For all cases, clinical and imaging data on the underlying pathology and the patient, including demographics, preoperative and 6-month postoperative ophthalmological assessment results, symptom duration, operative details with video debriefing, as well as the immediate and delayed postoperative course, were collected from the medical records. Results Eleven patients underwent endoscopic endonasal decompression for NON in the multidisciplinary skull base surgery unit of the Lyon University Neurosurgical Hospital during the 27-month study period. The mean patient age was 53.4 years, and there was a clear female predominance (8 females and 3 males). Among the underlying pathologies were 4 sphenoorbital meningiomas (36%), 3 optic nerve meningiomas (27%), and 1 each of trigeminal neuroma (9%), orbital apex meningioma (9%), ossifying fibroma (9%), and inflammatory pseudotumor of the orbit (9%). Fifty-four percent of the patients had improved visual acuity at the 6-month follow-up. Only 1 patient whose sphenoorbital meningioma had been treated at the optic nerve atrophy stage continued to worsen despite surgical decompression. The 2 patients presenting with preoperative papilledema totally recovered. One case of postoperative epistaxis was successfully treated using balloon inflation, and 1 case of air swelling of the orbit spontaneously resolved. Conclusions Endoscopic endonasal optic nerve decompression is a safe, effective, and minimally invasive technique affording the restoration of visual function in patients with nontraumatic compressive processes of the orbital apex and optic nerve. The timing of decompression remains crucial, and patients should undergo such a procedure early in the disease course before optic atrophy.
- The cost-effectiveness of different strategies to evaluate optic disk drusen in children. [JOURNAL ARTICLE]
- J AAPOS 2014 Sep 27.
To compare the costs of diagnostic work-up for optic disk drusen where ophthalmic ultrasound was performed prior to imaging and invasive studies with those where ophthalmic ultrasound was performed after such studies.The medical records of patients <18 years of age evaluated at a tertiary referral center between 2007 and 2012 for "swollen" optic nerves were retrospectively reviewed. The main outcome measure was cost of diagnostic work-up according to Georgia Medicaid global reimbursement rates.A total of 46 children with a B-scan ultrasound-confirmed diagnosis of calcified optic disk drusen were included. Neuroimaging was performed in 23 patients, of whom 20 had the study prior to ophthalmic ultrasound. The mean cost of evaluations for patients undergoing ancillary testing prior to ophthalmic ultrasound was $1,173; for those undergoing ancillary testing after, $305.Because optic disk drusen can mimic the appearance of papilledema, it is more cost-effective to perform ophthalmic ultrasonography prior to neuroimaging, especially when the patient is asymptomatic. If ophthalmic ultrasonography confirms the presence of drusen, it is more cost-effective to reassess the clinical picture before proceeding with further tests.