Noncirrhotic portal hypertension in primary biliary cholangitis with coexisting CREST syndrome.

Primary Biliary Cholangitis (PBC) is an autoimmune liver disease that is sometimes associated with CREST (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia) syndrome. If left untreated, PBC eventually progresses to liver cirrhosis. We describe an adult patient with CREST-PBC who presented with recurrent variceal bleeding and ultimately required transjugular intrahepatic portosystemic shunt (TIPS) insertion. Liver biopsy excluded cirrhosis, resulting in a diagnosis of noncirrhotic portal hypertension. This case report describes the pathophysiology of presinusoidal portal hypertension as a rare complication of PBC and its association with coexisiting CREST.

Cochlear implantation after radiosurgery for vestibular schwannoma.

OBJECTIVE: The object of this study was to ascertain outcomes of cochlear implantation (CI) following stereotactic radiosurgery (SRS) for vestibular schwannoma (VS). METHODS: The authors conducted a retrospective chart review of adult patients with VS treated with SRS who underwent CI between 1990 and 2019 at a single tertiary care referral center. Patient demographics, tumor features, treatment parameters, and pre- and postimplantation audiometric and clinical outcomes are presented. RESULTS: Seventeen patients (18 ears) underwent SRS and ipsilateral CI during the study period. Thirteen patients (76%) had neurofibromatosis type 2 (NF2). Median age at SRS and CI were 44 and 48 years, respectively. Median time from SRS to CI was 60 days, but notably, 4 patients underwent SRS and CI within 1 day and 5 patients underwent CI more than 7 years after SRS. Median marginal dose was 13 Gy. Median treatment volume at the time of SRS was 1400 mm3 (range 84-6080 mm3, n = 15 patients). Median post-CI PTA was 28 dB HL, improved from 101 dB HL preoperatively (p < 0.001). Overall, 11 patients (12 ears) exhibited open-set speech understanding. Sentence testing was performed at a median of 10 months (range 1-143 months) post-CI. The median AzBio sentence score for patients with open-set speech understanding was 76% (range 19%-95%, n = 10 ears). Two ears exhibited Hearing in Noise Test (HINT) sentence scores of 49% and 95%, respectively. Four patients achieved environmental sound awareness without open-set speech recognition. Two had no detectable auditory percepts. CONCLUSIONS: Most patients who underwent CI following SRS for VS enjoyed access to sound at near-normal levels, with the majority achieving good open-set speech understanding. Implantation can be performed immediately following SRS or in a delayed fashion, depending on hearing status as well as other factors. This strategy may be applied to cases of sporadic or NF2-associated VS. ABBREVIATIONS: AAO-HNS = American Academy of Otolaryngology-Head and Neck Surgery; ABI = auditory brainstem implant; CI = cochlear implantation; CN = cranial nerve; CNC = consonant-nucleus-consonant; CPA = cerebellopontine angle; EPS = electrical promontory stimulation; ESA = environmental sound awareness; HINT = Hearing in Noise Test; IAC = internal auditory canal; NF2 = neurofibromatosis type 2; OSP = open-set speech perception; PTA = pure tone average; SRS = stereotactic radiosurgery; VS = vestibular schwannoma; WRS = word recognition score.

Uveítis anterior crónica en paciente con síndrome de CREST / Chronic anterior uveitis in a patient with CREST syndrome

La esclerosis sistémica es una conectivopatía con unas manifestaciones clínicas muy heterogéneas, relacionándose en un pequeño porcentaje con enfermedades oculares inflamatorias. En el caso concreto de las uveítis, únicamente se han descrito casos aislados en la literatura, sobre todo con relación al síndrome de CREST. Presentamos el caso de una mujer de 53 años, con síndrome de CREST y uveítis anterior crónica, que consideramos de relevancia clínica dada su baja prevalencia

Systemic sclerosis is a connective tissue pathology with very heterogeneous clinical manifestations, associated in a small percentage with inflammatory eye diseases. In the specific case of uveitis, only isolated cases have been reported in the literature, especially in relation to the CREST syndrome. We present the case of a 53-year-old woman with CREST syndrome and chronic anterior uveitis, which we consider of clinical relevance given its low prevalence

Síndrome de CREST asociado a shock séptico por osteomielitis hematógena aguda / CREST syndrome associated with septic shock due to acute osteomyelitis hematologic

El síndrome de CREST (calcinosis, fenómeno de Raynaud, dismotilidad esofágica, esclerodactilia, telangiectasias) forma parte del espectro clínico de la esclerosis sistémica, enfermedad del colágeno, denominada en la clasificación clínica como esclerodermia cutánea limitada. Se presenta un paciente masculino de 53 años, raza blanca y procedencia rural, que desde hace 10 años presenta poliartritis, cambios en zonas distales de la piel que subyace adherida al hueso, falanges de manos y pies, fenómeno de Raynaud, aparición de nódulos subcutáneos de pequeño tamaño en varias localizaciones, que luego se tornan calcificaciones. Llega con una úlcera en el quinto metatarsiano del pie izquierdo, de 14 meses de evolución con varios ingresos para tratamiento de la lesión con resolución aparente y luego recidiva. Desarrolla un cuadro de osteomielitis hematógena aguda con shock séptico. El tratamiento multidisciplinario y oportuno permitió la sobrevida del paciente(AU)

CREST's Syndrome (calcinosis, Raynaud's phenomenon, dysmotilitic esofhagical, sclerodactilitys, telanghiectasis), the form departs from the clinical spectrum of the Systemic Sclerosis, disease of collagen, named in the clinical classification like cutaneous limited scleroderma. Patient, masculine of 53 years, white race, peasant procedence. 10 years ago with changes at zones level distally of the skin that underlies once the bone was adhered, phalanges of hands and feet, Raynaud's phenomenon so big a child's appearing of subcutaneous nodules at several locations, that next calcifications appear. Ulcer in metatarsal foot left-hand fifth, of 14 months of evolution with multiple entrances for treatment of the lesion with apparent resolution and next relapse. Develop acute osteomyelitis hematologic with septic shock. The multi-disciplinary and opportune treatment enabled the patient's over-life(AU)

Chronic Anterior Uveitis in a Patient with CREST Syndrome. / Uveítis anterior crónica en paciente con síndrome de CREST.

Systemic sclerosis is a connective tissue pathology with very heterogeneous clinical manifestations, associated in a small percentage with inflammatory eye diseases. In the specific case of uveitis, only isolated cases have been reported in the literature, especially in relation to the CREST syndrome. We present the case of a 53-year-old woman with CREST syndrome and chronic anterior uveitis, which we consider of clinical relevance given its low prevalence.

Acute Hand Ischemia and Digital Amputation After Transradial Coronary Intervention in a Patient With CREST Syndrome.

The radial artery approach for coronary angiography and intervention is rapidly replacing the femoral artery approach, largely because it reduces bleeding and vascular access site complications. However, complications associated with transradial access warrant attention, notably radial artery occlusion. This report focuses on a case of radial artery occlusion after percutaneous coronary intervention in a 46-year-old woman with CREST (calcinosis, Raynaud phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasia) syndrome, which ultimately led to acute hand ischemia necessitating amputation of her middle and index fingers.

A Rare Cause of Leg Ulcer: Calcinosis Cutis as a Part of CREST Syndrome.

Leg ulcers are not a disease themselves, they are a symptom of a disease. To manage them properly, finding the correct diagnosis of the disease is mandatory. Our case is a model to underline that leg ulcers are a significant burden for the patient, because leg ulcer got ahead of the patient's other serious complaints such as Raynaud's phenomenon or sclerodactyly. Furthermore, our patient is also a model, and an attentive clinical evaluation is inevitable to find the correct diagnosis. We present our case aiming to underline the significance of full dermatologic examination in each ulceration to discover underlying disease.

Orbital Inflammation as a Presenting Sign for CREST Syndrome.

A 61-year-old male was referred with a week's history of a painful and swollen left eye. Examination revealed normal visual acuities, left proptosis and global restriction of ocular ductions, and subretinal fluid at the macula. CT imaging confirmed thickening of the posterior scleral coat, with an associated choroidal effusion. Serology revealed positive antinuclear antibodies with a centromere staining pattern; subsequent rheumatology review revealed extensive telangiectasia with digital ulceration in both hands, and a diagnosis of limited cutaneous systemic sclerosis was made. Orbital inflammatory disease is often the initial presentation of systemic diseases such as sarcoidosis, granulomatosis with polyangiitis, and IgG4 disease. Limited cutaneous systemic sclerosis is rarely encountered in the context of orbital inflammation, but is a further systemic association, reminding the clinician of the diagnostic importance of peripheral symptoms and serological markers in patients presenting with orbital inflammation and scleritis.

Hemorragia digestiva alta (estómago de sandía) en paciente con esclerodermia limitada (síndrome de CREST) / Upper gastrointestinal bleeding (watermelon stomach) in a patient with limited scleroderma (CREST syndrome)

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Complications in Placement of a Glaucoma Drainage Device in a Patient With CREST Syndrome.

This case describes difficulty with conjunctival closure in an 80-year-old woman with CREST syndrome, a subset of systemic scleroderma. The patient underwent uneventful glaucoma drainage device implantation, but friability of the conjunctiva was noted during closure. Amniotic membrane was used to ensure secure closure, and the patient had a successful outcome. There is a paucity of existing studies on patients with CREST syndrome and their ocular findings. This report sheds light on ocular findings in this autoimmune disease and the necessity for alternative closure methods in special cases.

Cutaneous metastasis to the scalp as the primary presentation of colorectal adenocarcinoma.

Eruptaneous metastasis is an uncommon presentation of colorectal adenocarcinoma that can occur years after diagnosis of the primary cancer or manifest as the first sign of malignancy. It is essential to diagnose these metastases immediately, as this late-stage development carries a poor prognosis. The scalp is one of the less common sites for skin metastases and nodules may be mistaken for benign entities. In this case report, we report on the case of a 61-year-old woman with CREST syndrome who presented with a cutaneous metastasis to the scalp as the first sign ofcolorectal adenocarcinoma.

Manejo prostodóntico convencional de una paciente con síndrome de CREST: reporte de caso / Conventional prosthodontics management of a patient with Crest syndrome: case report

El síndrome de CREST se caracteriza por calcinosis, síndrome de Raynaud, dismotilidad esofágica, esclerodactilia y telangiectasia. Presenta signos y síntomas en el sistema estomatognático que merecen consideración en un tratamiento. El objetivo del presente artículo fue reportar las manifestaciones orales de un caso de síndrome de CREST y describir su tratamiento protésico realizado en el Departamento de Rehabilitación Oral de la Universidad Nacional de Colombia. Se reporta una paciente mujer de 42 años, con diagnóstico de síndrome de CREST por su médico tratante, que acude a la facultad de odontología para un tratamiento restaurador. El manejo prostodóntico incluyó coronas, prótesis parcial fija metal-cerámicas y prótesis parcial removible. Los resultados obtenidos nos permiten recomendar las alternativas restaurativas convencionales similares a las usadas en un paciente sin esta condición sistémica, pero manteniendo un control estricto.

The CREST syndrome is characterized by calcinosis, Raynaud's syndrome, esophageal dysmotility, sclerodactyly, and telangiectasia. It has signs and symptoms in the stomatognathic system that deserve consideration in its treatment. The objective of this paper is to report the oral manifestations of a case of CREST syndrome and describe the prosthetic treatment performed in the Department of Oral Rehabilitation in the National University of Colombia. The case is presented of a patient female aged 42 years, diagnosed with CREST syndrome by the treating doctor. She came to the Faculty of Dentistry for restorative treatment. The prosthodontic management included metal-ceramic crowns, fixed partial denture, and removable partial denture. The results demonstrate that conventional restorative alternatives, similar to that used in a patient without this systemic condition, while maintaining strict control of it, can be recommended.

Worsening of calcinosis cutis with teriparatide treatment in two osteoporotic patients.

We present two cases of patients with systemic autoimmune diseases (one with dermatomyositis and one with CREST syndrome) who presented with a worsening of calcinosis cutis after treatment of osteoporosis with teriparatide. To our knowledge, this association is not described in the literature and might be considered in the spectrum of adverse reactions to teriparatide.

Amyloidosis of the Breast: Three Different and Unusual Presentations of a Rare Entity.

BACKGROUND: Amyloidosis involving the breast is a rare finding and it may present as a solitary mass called 'amyloid tumor'. According to the largest case series, the amyloid deposits are usually of the AL type (commonly x03BA; light chain). METHODS: We report 3 cases diagnosed at our institution in the period from 2000 to 2015. Radiological, histological and immunohistochemical studies were performed. RESULTS AND CONCLUSIONS: Together with a case presenting in a patient with multiple myeloma, we describe 2 unique presentations including 1 associated with CREST syndrome in a patient with a previous history of breast carcinoma and another, also associated with cancer, with transthyretin deposits in a woman with a TTR gene mutation and a family history of familial amyloidotic polyneuropathy. These cases are an example of the vast heterogeneity of this disorder regarding its clinical presentation, the type of amyloid deposits and other diseases associated with breast amyloidosis.