Introduction Chronic rhinosinusitis (CRS) and irritable bowel syndrome (IBS) can be comorbidities that are difficult to treat. In this patient, an evidence-informed treatment pathway guided by laboratory biomarkers was used to address both conditions. Case Presentation A 69-y-old female patient presented with a 50-y history of sinusitis that was worse in the winter, postnasal drip, frequent sore throats, gastrointestinal complaints, headaches, and yeast infections. Two sinus surgeries (in years 2000 and 2002) and multiple courses of antibiotics had not resolved her sinus symptoms. In addition to CRS and IBS, this patient was noted to have intestinal overgrowth of Candida albicans, multiple food sensitivities, and leaky gut syndrome. Conclusion Antifungal medication and dietary changes in the course of 8 mo resulted in the resolution of her CRS and IBS. PMID:27547167
Anatomy of Cavernous Sinus and Cavernous Sinus Syndrome dr najeeb torrent
We report the uncommon case of an acute cavernous sinus syndrome in a patient who was consequently discovered to have both a cavernous internal carotid artery aneurysm and bacterial meningitis. Which came first, the chicken or the egg? Which of the two, the aneurysm or the meningitis, gave rise to the patient's symptoms? We briefly reviewed the literature of similar cases and tried to analyze the possible pathophysiological relationship between these findings. Moreover, this case highlights the importance of a multidisciplinary management of these patients to better decide between a medical and a surgical and/or endovascular treatment.
A Border collie was presented at the age of 9 weeks with several lesions of the right forelimb, including a reddish-blue haemangiomatous macula in the medio-dorsal part of the elbow, multiple, scattered small cavernous haemangioma-like lesions at the plantar part of the foot and a general hypertrophy of the limb. X-rays of the limb showed osteolysis. On skin biopsy, telangiectatic veins were observed. The rest of the body did not show any skin lesions or hypertrophy. The dog was otherwise healthy. Due to the extension of the lesions and worsening of the limb swelling, it was decided to amputate the affected limb. The dog remained healthy for 2 weeks, but then passed through episodes of anaemia, and finally died suddenly with signs of shock. Dissection of the limb after amputation revealed hypoplasia and aplasia of the deep venous system in the lower part of the leg. No arterio-venous shunts were noticed. ln man, this syndrome, characterised by an insufficiently developed deep venous system associated with local overgrowth of the limb and cutaneous telangiectasia, is known as Klippel-Trenaunay syndrome.
Good syndrome (GS) is defined as the association between thymoma and immune deficiency. It is often complicated by broncho-pulmonary bacterial infections and rhinosinusitis. This disease accounts for only 5% of all parathymic syndromes. These recurrent respiratory infections can cause bronchiectasis associated with Good syndrome. We report the case of a 52-year old woman hospitalized for non resolutive infectious pneumonitis. Chest CT scan showed bronchiectasis associated with thymoma confirmed by biopsy. The discovery of hypogammaglobulinemia allowed the diagnosis of Good syndrome.
X-linked acro-gigantism (X-LAG) syndrome is a newly described disease caused by microduplications on chromosome Xq26.3 leading to copy number gain of GPR101. We describe the clinical progress of a sporadic male X-LAG syndrome patient with an Xq26.3 microduplication, highlighting the aggressive natural history of pituitary tumor growth in the absence of treatment. The patient first presented elsewhere aged 5 years 8 months with a history of excessive growth for >2 years. His height was 163 cm, his weight was 36 kg, and he had markedly elevated GH and IGF-1. MRI showed a non-invasive sellar mass measuring 32.5 23.9 29.1 mm. Treatment was declined and the family was lost to follow-up. At the age of 10 years and 7 months, he presented again with headaches, seizures, and visual disturbance. His height had increased to 197 cm. MRI showed an invasive mass measuring 56.2 58.1 45.0 mm, with compression of optic chiasma, bilateral cavernous sinus invasion, and hydrocephalus. His thyrotrope, corticotrope, and gonadotrope axes were deficient. Surgery, somatostatin analogs, and cabergoline did not control vertical growth and pegvisomant was added, although vertical growth continues (currently 207 cm at 11 years 7 months of age). X-LAG syndrome is a new genomic disorder in which early-onset pituitary tumorigenesis can lead to marked overgrowth and gigantism. This case illustrates the aggressive nature of tumor evolution and the challenging clinical management in X-LAG syndrome.
X-linked acro-gigantism (X-LAG) syndrome is a newly described disease caused by microduplications on chromosome Xq26.3 leading to copy number gain of GPR101. We describe the clinical progress of a sporadic male X-LAG syndrome patient with an Xq26.3 microduplication, highlighting the aggressive natural history of pituitary tumor growth in the absence of treatment. The patient first presented elsewhere aged 5 years 8 months with a history of excessive growth for >2 years. His height was 163 cm, his weight was 36 kg, and he had markedly elevated GH and IGF-1. MRI showed a non-invasive sellar mass measuring 32.5 23.9 29.1 mm. Treatment was declined and the family was lost to follow-up. At the age of 10 years and 7 months, he presented again with headaches, seizures, and visual disturbance. His height had increased to 197 cm. MRI showed an invasive mass measuring 56.2 58.1 45.0 mm, with compression of optic chiasma, bilateral cavernous sinus invasion, and hydrocephalus. His thyrotrope, corticotrope, and gonadotrope axes were deficient. Surgery, somatostatin analogs, and cabergoline did not control vertical growth and pegvisomant was added, although vertical growth continues (currently 207 cm at 11 years 7 months of age). X-LAG syndrome is a new genomic disorder in which early-onset pituitary tumorigenesis can lead to marked overgrowth and gigantism. This case illustrates the aggressive nature of tumor evolution and the challenging clinical management in X-LAG syndrome. PMID:26607152
The major mucous membrane and cutaneous manifestation of Sjögren's syndrome (SS) is xerosis. Severe dryness of the eyes, mouth, nasal passage, and vagina produce many annoying symptoms, including photophobia, burning and itching of the eyes, alterations in taste and smell, recurring nonallergic bacterial sinusitis rhinitis, and dysparunia. SS patients frequently complain of dryness of lips and soreness and a burning sensation of the tongue and oral mucosa. SS patients also complain of dryness of their hair and note a decrease in luster, and severe dryness of the skin is frequently accompanied by pruritus.
The blood supply to the choroid comes ultimately from the ophthalmic artery (#1 in figure). There are variations but quite posterior branches will become the central retinal artery (#2 in Figure), and ciliary arteries (#3 in Figure) on each side of the optic nerve. These vessels divide into 2 long posterior ciliary arteries(#4 in Figure) and 20 short posterior ciliary arteries (only one on each side is shown in the diagram #5 in Figure) that enter the eye immediately adjacent and around the optic nerve. The short posterior ciliary arteries directly supply the choroid and the long posterior ciliary arteries travel in the suprachoroidal space anteriorly (#6 in Figure) then supply the choroid anteriorly via recurrent branches. The ophthalmic artery (#1 in Figure) continues to provide branches for the posterior (#7 in Figure) and anterior (#8 in Figure) ethmoidal vessels. The superior oblique muscle is shown for orientation ( #9 in Figure).Blood in the choroid circulates through the choriocapillaries and larger vessels of the choroid to drain into 4-6 vortex veins. In the photograph of a transilluminated portion of the eye in which the retina has been cut away the whorled vortices of the choroidal veins are evident as they coalesce and drain into a single vortex vein. (Also see #1 in Figure drawing). The vortex veins emerge just posterior to the equator (#2 in Figure) in quadrants. The superotemporal and superonasal (#1 and 2 in Figure) vortex veins will drain into the superior ophthalmic vein. The inferonasal and inferotemporal vortex veins will drain into the inferior ophthalmic vein. These vessels will eventually exit via the cavernous sinus (#5 in Figure). The vortex veins anastomose with the anterior ciliary veins (not shown in the figures). The anterior ciliary veins normally carry blood only from the anterior ciliary muscle, but if the vortex veins are occluded (e.g. from a poorly placed scleral buckle) the anterior ciliary veins may become quite dilated.The superotemporal ophthalmic vein usually exits the eye directly adjacent to or underneath the superior oblique tendon. This has clinical implications for approaches to surgery in which the superior oblique tendon is recessed. Note that the superior ophthalmic vein moves inferiorly and temporally to exit the eye in the superior orbital fissure. This anatomic feature is important when evaluating radiologic studies for carotid cavernous fistula in which the superior ophthalmic vein is enlarged. The inferior ophthalmic vein is said to pass thru the inferior orbital fissure in section 2 of the BCSC. This may not be correct. Most texts show that it either communicates with the pterygoid plexus in the inferior orbital fissure but then either joins the superior ophthalmic vein or exits separately through the superior orbital fissue.The circulation of the anterior portion of the eye features an intricately anastomotic system that essentially join the anterior ciliary circulation and the long posterior ciliary circulation in 3 interconnected arterial circles, an episcleral circle where episcleral vessels join, an intramuscular ciliary circle and the major arterial iris circle (circumferential vessels in the ciliary body). The anterior ciliary venous system joins conjunctival and episcleral vessels at the limbus.This ends the required information for UCLA students taking the one week required clerkship in Ophthalmology. All others please continue. UCLA OP-264.01 students and Jules Stein residents please continue with the ocular pathology tutorial. 2ff7e9595c
Comentários