#Heerfordts syndrome

[mcatmemoranda]

Reviewing questions:

Blindness is a potentially devastating complication of sarcoidosis. Ocular sarcoid can develop in up to half of all cases and most commonly presents as anterior uveitis or keratoconjunctivitis. The most serious complication is optic neuritis.

A complete ophthalmologic examination at the time of diagnosis is recommended, as asymptomatic disease can result in permanent visual impairment. All patients with sarcoidosis should have a careful history and physical examination to identify other organ involvement before having further testing, including ECG, pulmonary-function testing, and baseline laboratory studies (calcium level, alkaline-phosphatase level, renal-function, and hepatic-function tests) to evaluate the extent of disease.

McConnell's sign is defined as right ventricular free wall akinesis with sparing of the apex. Typically this looks as if the apex of the RV is a trampoline. Echocardiogram shows right ventricular strain and a positive McConnell sign, which is indicative of pulmonary embolism (PE).

All patients who have known or suspected bronchiectasis and who are acutely ill with suspected lower respiratory tract infection should be empirically treated with an antipseudomonal antibiotic, such as cefepime until culture results are available to guide treatment decisions.

Heerfordt’s syndrome (uveoparotid fever) is another collection of clinical findings specific enough to diagnose sarcoidosis without a biopsy. These signs and symptoms include uveitis, parotid gland enlargement, and facial nerve palsy.

Lofgren’s syndrome is a form of acute sarcoidosis. Sarcoidosis is a multisystem disease characterized by the presence of noncaseating granulomas and although classically described in African Americans, it has one of its highest prevalences in the northern European population. Clinical variants exist with certain forms having diagnostic and prognostic value. Lofgren’s syndrome is the combination of erythema nodosum, bilateral hilar adenopathy, migratory polyarthralgias, and fever. This pattern is so consistent that it has 95% diagnostic specificity and allows diagnosis of sarcoidosis without a biopsy. Among European patients, the presence of Lofgren’s syndrome portends to a good prognosis. For mild disease symptomatic treatment with non-steroidal anti-inflammatory agents is reasonable or low dose prednisone may be added.

The optimal management of laryngotracheitis (croup) is determined by the severity of disease. There are numerous scoring systems for croup severity, with the Westley croup score being the most validated and most often used. The Westley croup severity scale includes evaluation for the presence of stridor at rest, retractions, and cyanosis as well as assessment of mental status and air entrance. Regardless of any official scoring system, most physicians would consider the presence of retractions and stridor at rest to be indications of moderate to severe disease that requires evaluation in the emergency department. The presence of cyanosis, confusion, depressed mentation, agitation, severe retractions, or absent breath sounds would indicate severe disease and/or the possibility of impending respiratory failure.

Mild cases of croup are treated on an outpatient basis with cool mist therapy and fluid replacement. Moderate cases may require supplemental oxygen, oral or intramuscular corticosteroids, or racemic epinephrine. Severe cases are best treated with hospitalization and racemic epinephrine.

Bottom Line: The most important step in initial management of laryngotracheitis includes nebulized epinephrine (racemic or L-epinephrine) and dexamethasone along with humidified oxygen, fever reduction, and hydration. Epinephrine acts almost immediately, while steroids have a delayed onset of action.

Pneumothorax is defined as a collection of air in the pleural space. The result is pleural separation of the visceral and parietal pleura. Spontaneous pneumothorax lacks an antecedent event (e.g. trauma). There are two types: primary and secondary. Primary spontaneous pneumothorax (PSP) occurs in patients without recognizable underlying lung disease. Secondary spontaneous pneumothorax (SSP) occurs in patients with visible underlying lung disease (e.g. COPD). The most common etiology of PSP is rupture of a subpleural bleb (which are usually not seen on imaging and are undiagnosed). Many cases initially diagnosed as PSP may eventually be diagnosed as SSP after further workup.

Management depends on the size of the pneumothorax. The spectrum of management includes watchful waiting, aspiration, chest tube insertion, and thoracoscopy with pleurodesis or lung resection.

Rhinosinusitis can be defined as either viral/bacterial and if bacterial, as complicated/uncomplicated. Complicated rhinosinusitis implies the extension of disease outside the nasal cavity/sinuses into adjacent structures, such as soft tissues, ophthalmologic tissues, and nervous system.

Current criteria for the presumptive (many of these cases may still be viral) diagnosis of acute bacterial rhinosinusitis (ABRS) include persistent symptoms lasting more than 10 days without any evidence of clinical improvement or a biphasic illness pattern which is also called double worsening, meaning the patient was sick, improved, and then became sicker a second time within a short time frame, usually within a 10 day period. The IDSA guidelines from 2012 also recommend the use of a high fever greater than 39C associated with severe symptoms for 3-4 days as being more consistent with bacterial rhinosinusitis. According to the guidelines by the American Academy of Otolaryngology-Head and Neck Surgery published in 2015, fever itself early in the course of illness is not sensitive or specific enough to warrant treatment based on this vital sign alone, and they do not recommend using this as criteria for the diagnosis of ABRS. They agree with the other criteria used by the IDSA and acknowledge that a "severe" presentation may warrant the use of antibiotics as recommended by the IDSA and the American Academy of Pediatrics which considers more than 3 days of high fever and purulent nasal discharge as a severe presentation of ABRS in children.

Summary Criteria for the Diagnosis of ABRS

Signs or symptoms of acute rhinosinusitis present for 10 days or more after symptom onset with no improvement

Acute worsening of improving rhinosinusitis within 4-6 days of symptom onset or "double-sickening."

Severe rhinosinusitis: onset of high fever greater than 39C and severe symptoms within 3-4 days of onset

Tx of ABRS: amoxicillin, amoxicillin-clavulanate, doxycycline if PCN allergic, or respiratory fluoroquinolone in kids who can't take doxycycline. I just had a pt in clinic who had bacterial rhinosinusitis and I gave him amoxicillin-clavulanate.

Bottom Line: In patients with ABRS who require antibiotic therapy, the initial therapy of choice is with amoxicillin with or without clavulanate.

One of the feared complications of acute bacterial rhinosinusitis (ABRS) is orbital cellulitis. This infection commonly develops in the setting of ABRS due to the direct extension of bacteria from the sinus cavity into the orbit. Commonly it can project through the roof of the maxillary sinus. Additionally, there can be extension through the adjacent soft tissues. The diagnosis of this condition is clinical and is confirmed with radiographic imaging (CT scan of the sinuses). The presence of pain/difficulty with eye movement, double vision, eye swelling, and erythema should be concerning for orbital infection. The initial imaging test of choice would be with a contrasted CT study of the face and orbits. An MRI could also be performed and has similar accuracy, but is generally slower and more expensive to obtain. Additionally, patients with suspected orbital cellulitis should be evaluated by ophthalmology in the emergency department and started on IV antibiotics with coverage for MRSA (vancomycin generally).

If the sinuses are the suspected source, ampicillin-sulbactam or piperacillin-tazobactam can be added to vancomycin as long as there is no concern for CNS involvement. If the CNS is involved, it should be noted that both ampicillin-sulbactam and piperacillin-tazobactam have relatively poor CNS penetration, and a 3rd generation cephalosporin (ceftriaxone) should be used with the addition of metronidazole for coverage of anaerobic organisms. General guidelines for sepsis and infection should be followed with the physician obtaining the regular laboratories including a complete blood count, metabolic profile, blood cultures, and lactate.

Symptoms of Complicated ABRS:

Proptosis or impaired extraocular movements Painful eye movements Diplopia or impaired vision Periorbital edema or erythema Cranial nerve palsies Altered mental status Neck stiffness/meningeal signs Papilledema

Complications of ABRS:

Meningitis Orbital cellulitis Cavernous sinus thrombosis/thrombophlebitis Preseptal cellulitis

Ruptured eardrum:

Tympanic membrane perforations are commonly caused by trauma or acute otitis media and are usually noted on otoscopic examination. Perforations with marked hearing loss or other concerning neurologic signs such as nystagmus, ataxia, or vomiting should receive a prompt evaluation by an otolaryngologist. Supportive care only is a perfectly reasonable treatment plan for patients with simple perforations with minimal hearing loss and no neurologic signs. Many perforations will heal spontaneously within 4 weeks and require no intervention.

[sawtelghad]

‫ما متلازمة هيرفوردت؟ https://sawtelghad.net/54832

[burningladywitch]

Heerfordt syndrome Symptoms, Causes, Treatment, prognosis

Heerfordt syndrome Symptoms, Causes, Treatment, prognosis

Heerfordt syndrome (also perceived as uveoparotid fever and Heerfordt-Waldenström syndrome) is a critical syndromal presentation of sarcoidosis. Multiple nodular sores identify sarcoidosis in the skin, eyes, internal organs, salivary glands, and lymph node enlargement. In 1909, an ophthalmologist from Denmark, C. F. Heerfordt, first described the indications – uveitis, parotid gland enlargement,…

[doctorfoxtor]

I have been saying TIL WAY too often lately lmao

100 days of productivity

day 28 + 29

CVS/RS

contraindication to lung resection - ROMP: reduced FEV1 <1.5 l/min, Obstructed SVC, Malignant effusion, Paralysis of vocal cords

amlodipine is the only CCB approved for use in CHF

anticoagulation INR targets: 2-3 (2.5) for single DVT, afib, replaced aortic valve; 2.5-3.5 (3) for replaced mitral valve; 3-4 (3.5) for recurrent DVT

recommended initial BIPAP settings: IPAP 10 cmH2O, EPAP 4-6, back-up RR 2 below spontaneous rate (usu. 15/min)

Heerfordt syndrome = pulm sarcoidosis + sarcoid uveitis + fever

venous ulcer → compression *bandaging*, not stockings (which are for prophylaxis)

pulsus alternans in LVF

smoking seems to actually be ?protective against PIH/GH/preeclampsia

passive smoking accounts for about 15% of lung ca in non-smokers

CNS/Ophthal/Psych

in the presence of a known demyelinating lesion, visual evoked potentials actually contribute nothing to the diagnosis of MS

pls ready the bloody vignette. Just because they have tunnel vision it doesn't mean it's POAG: tunnel vision + night blindness + family hx = retinitis pigmentosa

dubious evidence: newer antipsychotics cause cardiac ischaemia in younger pts, but not in older pts; in older pts they are associated with stroke and VTEs; also, they supposedly reduce seizure threshold to a greater level than earlier/typical antipsychotics

Endocrine/GIT

↑leptin → ↑POMC secretion

↓leptin → ↑neuropeptide Y secretion

iron and calcium can interfere with L-thyroxine absorption!

mild elevations in ferritin (<1k) are common in NAFLD; differentiate from haemosiderosis/chromatosis, when ferritin is often >1k and transferrin saturation (= iron/TIBC) >40-50%

Rheum/Derm

patients on glucocorticoids: offer alendronate if T <-1.5; repeat DEXA in 1-3 yrs if T <0!

the most common vasculitis symptom of EGPA is mononeuritis multiplex

post significant complication of PUVA is SCC

the only disease-modifying therapy in ankspon is NSAIDs; anti-TNFs do NOT prevent structural damage

small papule progressing to a large painless indurated ulcer with central depression → leishmaniasis > M. ulcerans

Onc/Haem

cisplatin is assoc w/ hypomagnesaemia

acute leukaemias - immunophenotype diagnostic, cytogenetics prognostic

CLL - immunophenotype diagnostic, immunophenotype + cell counts prognostic

CML - cytogenetics (t(9;22)) diagnostic

when GFR >10 ml/min, the safer opioids to choose are oxycodone, hydromorphone, fentanyl and buprenorphine

PVC is assoc w/ angiosarcoma liver

ID/Misc

run quickly to the east for a sure meal: east African trypanosomiasis = T. b. rhodesiense = acute onset (stage I suramin; stage II melarsoprol)

gamble slowly to the west for pens with nibs: west African trypanosomiasis = T. b. gambiense = chronic onset (stage I pentamidine; stage II nifurtimox ± eflornithine)

ion channel-linked receptors - GANG: GABA-A, nicotinic, glutamate

tyrosine kinase-linked receptors - big, bloody, interesting, lactating diabetic: GH/IGF-1, EPO, interleukins, prolactin, insulin

guanylyl cyclase: ANP, BNP

Gq-PCRs - HAV 1 MIME: H1, alpha-1, V1, M1, M3

Gi-PCRs - Gaby is 2 MAD: GABA-B, M2, alpha-2, D2

Gs-PCRs - everything else lol

[juniperpublishersanesthesia]

Heerfordt's Syndrome: A New Atypical Clinical Presentation-Juniper Publishers

Abstract

Heerfordt’s syndrome is an unusual clinical manifestation (less than 5%) of sarcoidosis, characterized by parotitis, uveitis, and peripheral facial palsy, febrile syndrome is usually associated. Oral corticosteroid is the treatment of choice. We report a case of a patient in which the syndrome of Heerfordt was retained.

Keywords: Heerfordt’s syndrome, parotitis, uveitis, sarcoidosis, facial palsy.

Introduction

The sarcoidosis is a systemic granulomatosis of unknown cause, characterized by its clinical polymorphism and a wide variety of its modes of presentation. The combination of fever, uveitis, parotitis and peripheral facial paralysis defines Heerfordt’s syndrome which presents an unusual manifestation of this disease. We report a case of a patient in which the syndrome of Heerfordt was found.

Case Report

A 49 year-old female followed in ophthalmology for bilateral uveitis, was referred to the ENT consultation for nasal obstruction with crusting rhinitis. General examination was unremarkableexcept a low-grade febrile state, especially there was no facial palsy. The patient has undergone a rhinocavoscopy which revealed the presence of nodular lesions in the lower horn. A biopsy of the lesions was performed which results normal. The evolution of the symptomatology was marked by the appearance of erythema nodosum on the face and the upper limbs (Figure 1).

The diagnosis of sarcoidosis was confirmed by the biopsy of the cutaneous lesions which showed non-caseatingepithelioid granulomas.

Facial CT scan highlighted a multinodular bilateral chronic parotitis (Figure 2), therefore, the diagnosis of Heerfordt’s syndrome was retained in view of these highly evocative criteria even if the symptomatology was incomplete given the absence of facial palsy. The patient was initiated on long-term steroid therapy. After a month of treatment with oral steroids, clinical symptoms have shown a clear improvement.

Discussion

Sarcoidosis is a chronic inflammatory disorder of unknown etiology, characterized by noncaseating granulomas involving the lungs in more than 90% of patients. Ocular, lymph-node, and cutaneous manifestations are next in frequency, but any organ system can be affected by the disease.The diagnosis requires exclusion of other etiologies, such as Sjogren's syndrome, tuberculosis, fungal and parasitic infections, Wegener's granulomatosis [1].

Otolaryngologic manifestations are identified in 10-15 % of patients, the most common being cervical adenopathy [2]. Salivary glands are less frequently involved, unilateral or bilateral swelling of the parotid gland was reported in only 6-8% of patients with sarcoidosis [3]. It can be included in Heerfordt’s syndrome (uveoparotid fever).

Heerfordt’s syndrome is a sarcoidosis syndrome characterized by mild fever, painless parotid gland enlargement, cranial nerve involvement, and anterior uveitis [2]. It is considered as one of the first central nervous system involvements to be described as a neurological presentation of sarcoidosis. The complete form occurs in approximately 0.3% of all sarcoidosis cases [4]. The etiology of this condition is still unclear and, as a result, so is the pathogenesis [1].

The incidence of cranial nerve palsy in sarcoidosis is about 5 % [5], with the facial nerve followed by the optic and the trigeminal nerves being the most common nerves involved [6]. Facial palsy forms an important defining component of Heerfordt’s syndrome. Its approximate incidence in this syndrome is 25-50%. However, its lack doesn't t eliminate the diagnosis as we have noticed in our case, in which the diagnosis of Heerfordt’s syndrome was made based on the association of other highly suggestive criteria such as uveitis and parotitis.

The combination of both uveitis with blurred vision and facial nerve palsy could be performed as sarcoidosis [7]. As it is obviously noted in the literature that eye involvement was the most consistent finding in patients presenting with Heerfordt’s syndrome, along with unilateral or bilateral facial palsy or parotid gland swelling [1]. Glucocorticosteroids remain the first- line therapy of Heerfordt’s syndrome [1].

This case emphasizes the importance of recognizing the main signs and clinical symptoms which may be quite modest to indicate the diagnosis of this syndrome.

Conclusion

Head and neck manifestations of Heerfordt’s syndrome are non-specific and a high suspicion is required to diagnose the condition early. In our case, Heerfordt’s syndrome was retained before the association of parotitis and uveitis confirmed subsequently by the histological examination. The presence of peripheral facial paralysis is not necessary to make the diagnosis and must not delay its management.

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