PS01.018: PERSISTENT DYSPHAGIA AFTER FUNDOPLICATION WITH MESH PLASTY –MISSED OR MISTAKEN –A CASE REPORT

2018 ◽  
Vol 31 (Supplement_1) ◽  
pp. 55-55
Author(s):  
Servarayan Chandramohan ◽  
Visvarath Varadharajan ◽  
Madeshwaran Chinnathambi ◽  
Kanagavel Manickavasagam ◽  
Abishai Jebaraj ◽  
...  

Abstract Background Scleroderma esophagus is a rare entity. Only few case reports of esophagectomy were done and reported for this condition. We are presenting this rare case of failed fundoplication and mesh repair with a diagnosis of GERD and hiatus hernia, which was found later on due to Scleroderma with Esophageal involvement. Methods 58 year old female admitted with dysphagia following laproscopic fundoplication with mesh repair of crura with a diagnosis of GERD and hiatus hernia.She presented with persistent vomiting and loss of weight.On evaluation, her Upper GI scopy revealed dilated esophagus with sluggish peristalisis. Since the patient had tightness of skin over the distal extremities, face and fish mouth appearance with thinning of nail, Skin biopsy was taken. The skin biopsy was reported to be scleroderma.The esophageal manometry demonstrated failed esophageal peristalisis with high normal LES pressure due to tight fundal wrap.The patient was treated with mesh remova, Transhiatal esophagectomy with gastric pull-up and cervical Anastomosis.Post operatively the patient was treated with hydrocholoroquine and predinisolone. Results The patient is free of dysphagia and is on regular follow up. Conclusion In case of failure, detailed evaluation including High resolution manometry (MII HRM) has to be done before doing laparoscopic fundoplication for GERD has to rule out uncommon and rare disorders of esophagus. Detailed clinical examination in GERD patients has to be done to rule out systemic disease like scleroderma.In case of failed fundoplication for GERD, patients have to investigated for the failure.So patients with incapacitating esophageal neuromotor disease, a more radical approach in the form of esophagectomy may be safer and more reliable than attempting another procedure and risk another failure. Disclosure All authors have declared no conflicts of interest.

2018 ◽  
Vol 31 (Supplement_1) ◽  
pp. 180-180
Author(s):  
Damiano Gentile ◽  
Pietro Riva ◽  
Anna Da Roit ◽  
Silvia Basato ◽  
Salvatore Marano ◽  
...  

Abstract Background Gastric conduit used for reconstruction after esophagectomy for esophageal cancer (EC) has the potential to develop a metachronous cancer known as gastric tube cancer (GTC). The aim of our study was to review literature and evaluate outcomes and possible treatment strategies for GTC. Methods A comprehensive systematic literature search was conducted using PubMed. No restriction was set for type of publication, number, age and sex of patients. Study language was limited to English. Characteristics of EC and its treatment and GTC and its treatment were analyzed. Results A total of 26 studies were analyzed, 10 retrospective analysis and 16 case reports, involving 170 patients, 17 patients (10%) were affected by multifocal GTC. 143 ECs (84,1%) were squamous cell carcinomas. In 95 patients (55,9%) a posterior-mediastinal reconstructive route was used at the time of esophagectomy for EC. Mean interval between esophagectomy and diagnosis of GTC was 67,18 months (4–236 months). 184 GTCs were metachronous lesions (98,4%). 164 GTCs were adenocarcinomas (98,2%). 84 GTCs were located in the lower part of the gastric tube. 88 patients were endoscopically treated. 63 patients underwent surgery. 30 total gastrectomies + limphoadenectomy with colon or jejunal interposition were performed. 27 subtotal gastrectomies and 6 wedge resections were performed. Main reported post-operative complications were: anastomotic leak, vocal cord palsy and respiratory failure. 19 patients were treated with chemoradiotherapy and palliative care. 68,2% of endoscopically treated patients, 63,5% of surgically resected patients and 5,2% of patients who underwent chemoradiotherapy were alive at a mean follow-up of 25,5 months. Feasibility of endoscopic resections in patients diagnosed with superficial GTC has been established. Surgical treatment represents the preferred treatment modality in operable patients with locally invasive tumor. Patients treated with conservative therapy have a scarce prognosis. Conclusion Yearly endoscopic follow-up is of paramount importance in patients who underwent esophagectomy for EC with gastric tube reconstruction. At least, a 10-year endoscopic surveillance is recommended. Disclosure All authors have declared no conflicts of interest.


2018 ◽  
Vol 31 (Supplement_1) ◽  
pp. 56-56
Author(s):  
Lavinia Barbieri ◽  
Andrei Ilczysyn ◽  
Jafar Jafari ◽  
Abraham Botha ◽  
Edel Smyth

Abstract Background Peroral Endoscopic Myotomy (POEM) has gained ground for achalasia tratment. Although laparoscopic Heller's-Dor (LHD) is a proven intervention, the cut of the inner muscular layer during POEM offers a goal-directed procedure. However, concerns regarding post-procedure reflux had been raised. As during POEM long myotomy is generally adviced, we decreased the length to reduce reflux after treatment. Methods 19 patients with type 1 or 2 achalasia underwent POEM guided intraoperatively by Endoflip® (Crospon Ltd., Galway, Ireland) to target the myotomy where the major point of tightness was. We aimed for an increase of compliance and distensibility of three times of the lower esophageal sphincter (LOS) via a short myotomy. We compared these patients with a historic control LHD group of 15 patients in terms of Eckardt's score, GERD-HRQL questionnaire, high-resolution manometry to check pressure of the LOS 24h pH-impedence to measure acid exposure with minimum follow-up of 9 months. Results Length of myotomy was 7 cm (6–10) for POEM and 9 cm for LHD. Median Eckard's score after POEM was 0.5 versus 2 for LHD group. Median Eckardt's score and GERD-HRQL were 0.5 vs 2 and 13 vs 15, respectively after POEM or LHD. 24h pH-monitoring revealed pathologic reflux in one patient after POEM and in 3 after LHD, with median acid % time of 0,1 vs 1,2. Data are summarized in table. Conclusion POEM is effective in achieving symptom relief in patient with achalasia. Although randomized data are not available, reflux rate after POEM with short myotomy guided by Endoflip is comparable with LHD and lower than the data of literature. Disclosure All authors have declared no conflicts of interest.


Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 3675-3675 ◽  
Author(s):  
Daniele Focosi ◽  
Marco Tuccori ◽  
Corrado Blandizzi ◽  
Mario Del Tacca ◽  
Mario Petrini

Abstract Abstract 3675 Poster Board III-611 Progressive multifocal leukoencephalopathy (PML) is a rare complication of immunesuppression due to productive reactivation of JC polyomavirus (JCV) in glial cells. In the last decades many case reports of rituximab-associated PML have raised concerns, but no systematic incidence rate analysis has been ever performed. We retrospectively reviewed our clinical records with the aim of calculating the incidence rate of PML in patients with NHL who had been treated with regimens including rituximab. Data on HIV-negative patients who received the first dose of rituximab from January 1, 2000 to June 30, 2008, were analyzed. The follow-up period was from the first rituximab dose to the last recorded visit, up to September 30, 2008. PML cases were included if symptoms occurred at least 1 month after the first dose of rituximab and the diagnosis was supported by magnetic resonance imaging and detection of JCV DNA in stereotactic brain biopsies and/or cerebrospinal fluid. We collected data from 821 consecutive patients throughout the follow-up period. All patients received chemotherapy other than rituximab, and all completed their entire treatment course at the Hematology Unit in Pisa. No radiation therapy was administered. The median time of follow-up was 20 months (range = 1-106 month), resulting in 1725 patient-years at risk. Five cases of PML (two receiving maintenance rituximab) were identified, with an incidence rate of 2.89 cases per 1000 patient-years. We found that the incidence rate of PML in our population exceeded that observed in patients who are traditionally regarded as being at high risk of PML, namely patients with B-cell chronic lymphocytic leukaemia and AIDS. Rituximab might be a potential contributing factor to the development of PML in these patients. Nevertheless, rituximab currently represents an essential therapeutic tool that can positively affect the natural history of NHL. However, because of the expanding therapeutic indications, it seems reasonable to investigate the potential contribution of rituximab to PML occurrence. Disclosures: No relevant conflicts of interest to declare.


Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 5560-5560 ◽  
Author(s):  
Alejandro Garcia-Horton ◽  
Rosanne St. Bernard ◽  
Alejandro Lazo-Langner ◽  
Anargyros Xenocostas ◽  
Joy Mangel ◽  
...  

Abstract It is estimated that 4-10% of patients with chronic lymphocytic leukemia (CLL) will develop autoimmune hemolytic anemia (AIHA) over the course of their disease. Ibrutinib has proven to be effective in treatment of relapsed, refractory, 17p deleted, and treatment naïve CLL. The effect of ibrutinib on AIHA in the context of CLL has not been established since patients with active hemolysis were excluded from major trials. In this abstract, we present a case series of patients that were actively hemolyzing at the start of ibrutinib therapy and in which their AIHA achieved prolonged response. Patient characteristics and laboratory data are shown in Table. Five patients (3 women, 2 men), median age 61 years (range 57 to 78), with CLL and active, uncontrolled AIHA at the time of ibrutinib initiation were identified. Uncontrolled AIHA was defined as anemia with evidence of hemolysis (at least two of the following: increased reticulocyte count, elevated lactate dehydrogenase, elevated indirect bilirubin, and reduced haptoglobin and a positive direct antiglobulin test (DAT)). Patients had a median hemoglobin of 70 g/L (range 69-96) prior to start of ibrutinib and 3 of them required transfusion support for symptomatic anemia. All patients were receiving prednisone for management of AIHA at the time of ibrutinib initiation and had been on it for a median of 10 days (range 9 - 25) without AIHA resolution. 1 patient received intravenous immunoglobulin concurrently. All patients had received at least one line of therapy for CLL in the past and 3 had experienced previous AIHA responsive to steroids. AIHA in 2 patients was related to previous fludarabine exposure but had responded to a prednisone tapering schedule and were off steroids by the time of the new AIHA flare. Median hemoglobin of 130 g/L (range 113-149) was reached at time of AIHA response. All 5 patients tolerated 420mg oral daily of ibrutinib therapy and AIHA was controlled in a median of 6.5 weeks (range 6-10). Discontinuation of steroids was achieved in all patients at a median of 10 weeks (range 6-17) without evidence of further hemolysis. All patients except one are receiving ongoing follow up and have been followed up for a median of 130 weeks (range 15-150) since ibrutinib start. Patients have not shown evidence of AIHA relapse and continue off AIHA treatment (prednisone). One patient required discontinuation of ibrutinib 6 months after starting due to neutropenia but there was no evidence of AIHA relapse in follow up. The patient has passed away from unrelated GI bleed 2 years after the initial AIHA event. This is the largest case series to our knowledge on the safe start of ibrutinib in CLL complicated by active AIHA. Hemolysis in all patients responded to a short prednisone taper with ibrutinib concurrently and obtained a sustained response at follow up without any flare ups or further AIHA treatment use. These cases suggest that it is safe to start ibrutinib during uncontrolled, active hemolysis in contrast to 2 previous case reports that suggested causal relationship between ibrutinib and onset of severe CLL-associated AIHA (Rider et al, 2015; Hodskins et al, 2014). As previously reported, AIHA occurrence or relapse once ibrutinib has been started is rare (Rogers et al, 2016). Disclosures No relevant conflicts of interest to declare.


Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 4160-4160
Author(s):  
Paola Finsinger ◽  
Massimo Breccia ◽  
Clara Minotti ◽  
Ida Carmosino ◽  
Laura Cannella ◽  
...  

Abstract Abstract 4160 Acute Promyelocytic Leukemia (APL) is a rare subtype of Acute Myeloid Leukemia (AML) more common in younger adults, with a median age of 45 – 50 years at onset. The use of All-trans Retinoic Acid (ATRA) as tailored treatment has made APL a very curable disease also in patients aged > 60 years; however, there are only few case reports in very elderly APL patients. To address this issue, we revised clinical data and treatment results in 12 patients aged > 70 years with newly diagnosed APL followed at our Institution from 1/91 to 12/2008. Clinical characteristics at onset were as follows: M/F 7/5, median age 74.7 years (range 70.0 – 80.8), M3/M3v 11/1, median WBC 1.3 × 109/l (range 1.0 – 7.4), median PLTS 53 × 109/l (range 12 – 302), BCR1/BCR3 6/6. According to Sanz risk score, 7 patients were at low-risk and 5 at intermediate-risk; 6/12 patients had arterial hypertension, 4/12 a concomitant cardiologic disease, 3/12 a cerebro-vascular disease and 2/12 a previous neoplasia. Induction therapy consisted of ATRA + Idarubicin in 8 patients (2/8 with reduced Idarubicin dosage) and ATRA alone in 4 patients; in this latter group, however, 2/4 needed to add chemotherapy (CHT) based on Mitoxantrone + AraC due to hyperleukocytosis during ATRA treatment. All patients achieved both morphological and molecular Complete Remission (CR) after a median time of 50 (range 29 – 65) and 105 (range 51 – 239) days, respectively. Infective complications were observed in 10/12 patients (4 episodes of FUO, 6 sepsis, 2 cystitis and 1 oral abscess) while ATRA syndrome occurred in 2/12 patients; in addition, there were 3 episodes of cardiac ischemia and 3 episodes of paroxystic atrial fibrillation. All but one patient received consolidation therapy (based on CHT alone in 7 patients, CHT + ATRA in 3 patients and ATRA alone in 1 patient), followed by maintenance treatment in 8 patients. Four patients had a relapse (hematological in 3 cases and molecular in 1 case) after 8, 11, 35 and 56 months respectively. At present, 6 patients are still alive, 4 died due to disease progression (3) or senectus while in CR (1) and 2 were lost to follow-up while in CR: mean event-free survival and overall survival were 89.2 months (95%CI 52.6 – 125.8) and 99.9 months (95%CI 65.0 – 134.7), respectively. In conclusion, ATRA-based treatment of APL is safe and effective also in very elderly patients, with long-lasting disease-free and overall survival. Disclosures: No relevant conflicts of interest to declare.


2020 ◽  
Vol 1 (1) ◽  
Author(s):  
Daniel Ensley ◽  
David Myers ◽  
George Kallingal

This is an evaluation of a patient presenting with bladder amyloidosis and a seven year asymptomatic history between gross hematuria episodes. Cystoscopic evaluation showed a diffuse progression of amyloid deposits throughout the bladder. Biopsy confirmed the diagnosis and was negative for malignancy. Further evaluation ruled out systemic disease. To date, he has declined treatment. There is little available literature on long term urologic follow up for these patients. Bladder amyloidosis is rare, but most urologists will encounter it in their clinical practice. There are no agreed upon guidelines or recommendations for long term management of these patients, likely owing to its rarity. This patient had local progression confirmed on biopsy without evidence of malignancy. Evaluation for systemic amyloidosis was also negative. While a single, isolated case, it is unique for the length of follow up, the local progression and lack of symptoms. It is consistent with the low likelihood of systemic progression but does raise the question of how to treat these patients long-term. The authors recommend initial biopsy both to confirm diagnosis and rule out malignancy, evaluation for systemic disease and regular cystoscopic examinations to help direct therapy.


F1000Research ◽  
2020 ◽  
Vol 9 ◽  
pp. 661
Author(s):  
Abdallah Qasim ◽  
Omar Kousa ◽  
Mohamed Mansour ◽  
Ahmad K. Aly ◽  
Dana Awad ◽  
...  

Sarcoidosis is a systemic disease with heterogeneous clinical manifestations that is characterized histologically by the presence of noncaseating granulomas in the affected organs. It can be a diagnostic challenge, especially when mimicking malignancy or fungal infections. Previous case reports of sarcoidosis presenting with multiple masses are highly suggestive of infectious or malignant etiology.  In this case, our patient presented with enlarged lymph node and was found to have innumerable nodules in the mediastinum, lungs, and liver. Azygos vein enlargement was also seen on radiological imaging, and malignancy was highly suspected; hence, an extensive workup was conducted, including laboratory, radiology and biopsy evaluation, which were diagnostic of sarcoidosis. Our case showed the importance of correlation of the history, physical examination, radiological and histopathologic studies in confirming the diagnosis and the need to rule out other serious infections and malignancies, especially with azygous vein enlargement, which can sometimes be missed in chest radiograph.


2021 ◽  
Vol 23 (Supplement_G) ◽  
Author(s):  
Lorenzo Torselletti ◽  
Stronati Giulia ◽  
Francesca Corraducci ◽  
Sara Belleggia ◽  
Francesco Maiorino ◽  
...  

Abstract Methods and results A 79-year-old woman, with a history of subclinical hypothyroidism, obesity and smoke presented to the Emergency Room with dyspnoea and cold sweating. She had undergone her first dose of COVID-19 Moderna mRna vaccination just four days prior to her admission. She showed elevated HS troponin and elevated BNP at her laboratory exams. Her 12-lead ECG showed T wave inversion in the antero-lateral leads and prolongued QTc. Her transthoracic echocardiogram showed severe ejection fraction reduction due to hypokinesia of the mid-apical segments of the anterior and lateral walls of the heart. The patient was then admitted to the Cardiology ward in order to perform a coronary angiography which sowed no significant coronary stenosis. She was started on appropriate medication and discharged after a six day in-hospital stay. At discharge she showed partial recovery of her ejection fraction (EF 44%). A cardiac magnetic resonance was performed after discharge which showed no late gadolinium enhancement. Such finding allowed us to rule out the diagnosis of myocarditis. Moreover at her 3-month follow-up her ejection fraction had recovered completely. We concluded for the diagnosis of Takotsubo Syndrome secondary to vaccination. Conclusions We presented a case of Takotsubo syndrome after vaccination with the Moderna vaccine for COVID-19. Two other similar case reports can be found in current medical literature. Female sex, post-menopausal age and the inevitable psychological stress derived by the pandemic and the vaccination may have triggered the condition.


Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 1533-1533
Author(s):  
Saloni Tanna ◽  
Betsy Clair ◽  
Sejal Kuthiala ◽  
Teresa A. Coleman ◽  
Abdullah Kutlar

Abstract Abstract 1533 Poster Board I-556 Hydroxyurea (HU) is an S-phase specific cytotoxic agent (pregnancy category D), approved for use in sickle cell disease (SCD) primarily due to its ability to augment production of fetal hemoglobin (HbF), which has been shown to reduce the frequency of pain crises and decrease the need for blood transfusions. In all animal species tested, HU produced an increase in congenital anomalies. Defects of the central nervous system, palate, and skeleton occurred in rats treated with HU in the ninth to twelfth gestational days. Embyonic cytotoxicity may be secondary to formation of reactive free radicals. Because of its teratogenic potential, patients with SCD are instructed to practice contraception while on HU therapy. However, risks associated with maternal or paternal HU exposure at the time of conception is unclear. Our review of the literature has only shown six case reports of HU exposure in pregnancy for the treatment of SCD. Two of these pregnancies were terminated with medical abortion, the remaining four cases described liveborn infants with normal phenotypes at 15 to 21 months of follow up. 236 patients followed at the Medical College of Georgia Sickle Cell Center have been on HU for a period of 6 months to 14 years. Despite precautionary counseling, we have identified seven women and three men with SCD who have conceived while on HU therapy. We analyzed the birth records of ten children conceived during HU therapy. The duration of HU therapy varied from 2 weeks to 3 years at the time pregnancy was diagnosed; gestational age when HU was discontinued ranged from 3 to 10.5 weeks. Some children were born prematurely or preterm, with birth weights ranging from 922 to 3178 grams. APGAR scores ranged from 2 to 9 for the first minute and 8 to 10 for the five minute score. One child who had been born severely premature at 26 weeks with a birth weight of 922 grams and APGAR scores of 2 and 8 at one and five minutes to a father who had been on HU at the time of conception was ultimately withdrawn from life support measures. The remaining nine children were assessed for appropriate development by questionnaires provided to parents as well as pediatric records when available. One child with APGAR scores of 5 and 9 has been diagnosed with mental retardation and attention deficit disorder. Developmentally, the other eight children have achieved appropriate milestones. Women with sickle cell disease are more likely to develop complications such as intrauterine growth retardation and preterm delivery than those without the disease. These findings suggest that children conceived on HU therapy are not significantly different from other infants born to parents with SCD who are not on HU. Our sample size is not large enough to observe the potential effects of in utero HU exposure. The small number of subjects is likely secondary to potential teratogenicity discouraging women to become pregnant as well as paucity of exposed pregnancies due to menstrual disturbances induced by HU. It is therefore important to develop and maintain a national registry to allow longer follow up of exposed children and more careful assessment of fetotoxic effects for those conceived during HU therapy for SCD. Disclosures No relevant conflicts of interest to declare.


2016 ◽  
Vol 60 (1) ◽  
pp. 65-73 ◽  
Author(s):  
Camille Boulagnon ◽  
Alain Ducasse ◽  
Martine Patey ◽  
Marie-Danièle Diebold ◽  
Carl Arndt

Objective: To describe findings in vitreous fluid samples in routine cytology practice. Study Design: The pathology archives from 1988 to 2008 at our institution were searched for vitreous samples. The slides were reviewed and clinical and follow-up information was obtained. Results: One hundred and eighty-two vitreous fluid samples from 166 patients were analyzed. Most of the samples had been collected for vitreous hemorrhage (75 cases). The second reason for vitreous sample cytological evaluation was an intraocular inflammatory process (55 cases). A specific cause of inflammation was found by combining clinical, microbiological and cytopathological findings in 19 cases, i.e. infection in 7, sarcoidosis in 3, retinal necrosis in 3, lens-induced endophthalmitis in 2, uveitis associated with systemic disease in 2, retrobulbar neuritis in 1 and sympathetic ophthalmia in 1. Among the 19 samples from 16 patients collected to rule out malignancy, 8 had a confirmed intraocular malignancy. Malignant cells were observed in 5 cases (3 lymphomas, 1 melanoma and 1 carcinoma). Undiagnosed malignancies included 2 lymphomas and 1 choroidal melanoma. Other samples were collected during surgery for retinal detachment and cataracts. Conclusions: In routine practice, cytology of the vitreous fluid is performed in many and varied situations that are most often nonneoplastic. In nonneoplastic cases, cytology is a useful adjunct to vitrectomy, in spite of its limitations.


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