{"title":"Diagnostic Challenges in Sporadic Creutzfeldt-Jakob Disease: A Case Study of Typical Clinical Presentation with Negative Findings.","authors":"Xiaoyu Zhu, Ran Li, Yu Zhu, Yunlong Tan","doi":"10.12659/AJCR.945795","DOIUrl":"10.12659/AJCR.945795","url":null,"abstract":"<p><p>BACKGROUND Creutzfeldt-Jakob disease (CJD) is a rare and fatal neurodegenerative disorder caused by the misfolded isoform of the prion protein (PrPSc). The disease is characterized by rapid progression and the absence of effective treatment options, making it particularly devastating. Among its various subtypes, sporadic Creutzfeldt-Jakob disease (sCJD) is the most common in humans. Diagnosing CJD before death is challenging due to its nonspecific symptoms and the difficulty in distinguishing it from other neurodegenerative conditions. CASE REPORT We detail the initial presentation of a 58-year-old woman with suspected sCJD, highlighting the atypical symptoms and prolonged survival. The uniqueness of this case lies in the fact that, despite the patient exhibiting relatively typical clinical features, all laboratory and examination results for CJD consistently returned negative. This led to the patient being transferred between multiple departments, including psychiatry, during the early stages of the disease, making it difficult to receive a correct diagnosis and treatment. The patient's journey through various medical consultations underscores the complexity of diagnosing such a rare condition. It illustrates the importance of a holistic approach that considers both clinical presentation and supplementary examinations. CONCLUSIONS Excessive reliance on supplementary examinations, coupled with insufficient awareness of sCJD across multiple clinical departments and a lack of attentiveness to clinical symptoms, culminated in a misdiagnosis as a psychiatric disorder. This initiated a convoluted and protracted diagnostic process, significantly exacerbating the burden on both the patient and her family. This situation underscores the critical importance of recognizing clinical presentations in rare diseases like sCJD while employing diagnostic tools. Furthermore, it highlights the necessity for enhanced interdisciplinary communication and collaboration among clinical departments to facilitate timely and accurate identification of sCJD.</p>","PeriodicalId":39064,"journal":{"name":"American Journal of Case Reports","volume":"26 ","pages":"e945795"},"PeriodicalIF":1.0,"publicationDate":"2025-02-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11829749/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143382989","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"A 73-Year-Old Man Presenting with Shoulder Muscle Pain and a Diagnosis of Guillain-Barré Syndrome.","authors":"Yupei Cheng, Bangqi Wu, Yang Guo, Jingjie Huang","doi":"10.12659/AJCR.945539","DOIUrl":"10.12659/AJCR.945539","url":null,"abstract":"<p><p>BACKGROUND Guillain-Barre syndrome (GBS) commonly presents with motor weakness and neurological symptoms and signs that include loss of tendon reflexes. However, patients with GBS also experience nerve pain (radicular pain) and deep muscle pain. This report is of a 73-year-old man presenting with shoulder muscle pain and a diagnosis of Guillain-Barre syndrome. CASE REPORT A 73-year-old man initially sought medical attention for severe left shoulder muscle pain. One week prior, he had experienced a cold and diarrhea, which improved with over-the-counter medication. Physical examination revealed normal shoulder joint motion without swelling or stiffness. Cervical spine magnetic resonance imaging (MRI) revealed osteophytes and disc protrusions from C3/4 to C6/7, leading to an initial diagnosis of cervical spondylosis. Two days later, he developed progressive numbness and weakness in both upper limbs. Upon hospital admission, further evaluation revealed partial cranial nerve dysfunction, elevated cerebrospinal fluid (CSF) protein levels without pleocytosis, and peripheral nerve damage on electromyography (EMG). GM1 antibody was positive, confirming GBS. Treatment with intravenous immunoglobulin (IVIG), gabapentin for pain management, and acupuncture targeting pain and limb symptoms resulted in the complete recovery of pain and limb function within a short period. CONCLUSIONS This report shows that deep muscle pain can be a symptom of Guillain-Barre syndrome. Recognizing such atypical presentations is crucial for timely diagnosis and effective management. This case provides a clinical basis for the diagnosis of atypical GBS and offers insights into pain management in GBS.</p>","PeriodicalId":39064,"journal":{"name":"American Journal of Case Reports","volume":"26 ","pages":"e945539"},"PeriodicalIF":1.0,"publicationDate":"2025-02-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11818942/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143374572","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Ana Sofia Soto Torselli, Miriam Duci, Maurizia Grazzini, Costanza Tognon, Pedro Araujo Rossati Sanches, Piergiorgio Gamba, Francesco Fascetti-Leon
{"title":"Optimizing Surgical Techniques for Anal Canal Duplications in 2 Pediatric Patients.","authors":"Ana Sofia Soto Torselli, Miriam Duci, Maurizia Grazzini, Costanza Tognon, Pedro Araujo Rossati Sanches, Piergiorgio Gamba, Francesco Fascetti-Leon","doi":"10.12659/AJCR.945747","DOIUrl":"10.12659/AJCR.945747","url":null,"abstract":"<p><p>BACKGROUND Anal canal duplications (ACD) are rare in children older than 2 years old. While some patients are asymptomatic, others have a variety of nonspecific symptoms. The definitive treatment is surgical removal, but the best technique varies with each case based on the local situation and the location of the lesion. CASE REPORT We present 2 cases of anal canal duplications in pediatric patients. Despite the atypical presentations of a concomitant cystic, a high suspicion of ACD led to surgical management using different techniques based on its location and local situation. The first patient had a sterile perianal abscess in the ischiorectal fossa, unresponsive to antibiotics and recurrent after surgical drainage. MRI revealed a fistula connecting the cyst to the exterior at the pectineal line, prompting a posterior approach for complete resection of the mass from the rectum. The second patient had an incidentally discovered ischiorectal mass. Imaging suggested a homogeneous cyst adhered to the rectal wall and easily accessible, leading to a successful robotic-assisted surgical resection. Both patients achieved complete anal sphincter function and full recovery postoperatively. Histological analysis in both cases presented typical characteristics found in ACD. CONCLUSIONS Although ACD is rare in pediatric patients over 2 years of age, cystic or infected lesions in the ischiorectal fossa should be considered as differential diagnoses. Radiological imaging is crucial for understanding the cyst's location and determining the optimal surgical approach. As presented in our cases, an open posterior approach may be considered when the lesion is or has been complicated by previous infection, associated with a fistula, as opposed to non-complicated cases in which a mini-invasive approach may be ideal.</p>","PeriodicalId":39064,"journal":{"name":"American Journal of Case Reports","volume":"26 ","pages":"e945747"},"PeriodicalIF":1.0,"publicationDate":"2025-02-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11818941/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143374575","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Mustafa Nuaimi, Brian Shaw, Sara Ubosy, Murali Iyyani, Ryan Shaw, Mario Madruga, Kwabena Ayesu, Stephen J Carlan
{"title":"Constrictive Pericarditis as a Post-Cardiac Surgery Complication.","authors":"Mustafa Nuaimi, Brian Shaw, Sara Ubosy, Murali Iyyani, Ryan Shaw, Mario Madruga, Kwabena Ayesu, Stephen J Carlan","doi":"10.12659/AJCR.945294","DOIUrl":"10.12659/AJCR.945294","url":null,"abstract":"<p><p>BACKGROUND Constrictive pericarditis is a chronic inflammatory process characterized by fibrosis and impaired ventricular filling. The diagnosis is challenging because of overlapping clinical features with other pathologies such as cardiac tamponade, and restrictive cardiomyopathy. We report a case of effusive-constrictive pericarditis with cardiac tamponade in a patient with a history of multiple myeloma, bone marrow transplantation, and colchicine treatment. CASE REPORT A 62-year-old woman was admitted to a hospital in Germany due to cardiac tamponade secondary to bacterial pericardial effusion that necessitated emergency sternotomy and pericardial washout. After a prolonged and complicated hospital course, she flew back to Orlando and presented on her day of arrival because of progressive dyspnea. A computed tomographic angiogram of the chest ruled out pulmonary embolism but showed a moderate pericardial effusion with a mass effect on the right ventricle showing a recurrent cardiac tamponade. Echocardiography suggested the diagnosis of constrictive pericarditis. Cardiac catheterization showed a mean right atrial pressure of 25 mmHg and a simultaneous left and right ventricular pressure waveform of ventricular interdependence, diagnostic of effusive-constrictive pericarditis. Colchicine, along with heart failure core measures, were started and resulted in symptomatic improvement. Her first pericardial effusion was bacterial, while the second was possibly a consequence of the cardiac surgery. CONCLUSIONS Cardiac catheterization has been the criterion standard for diagnosis of constrictive pericarditis. Colchicine may be curative for individuals exhibiting subacute symptoms. This case highlights the presentation and diagnosis of effusive-constrictive pericarditis, which can be associated with cardiac tamponade.</p>","PeriodicalId":39064,"journal":{"name":"American Journal of Case Reports","volume":"26 ","pages":"e945294"},"PeriodicalIF":1.0,"publicationDate":"2025-02-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11809931/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143366122","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Button Battery Ingestion in a Neonate: Risk, Management, and Implications.","authors":"Sulafa Sindi, Samah Al-Harbi","doi":"10.12659/AJCR.944479","DOIUrl":"10.12659/AJCR.944479","url":null,"abstract":"<p><p>BACKGROUND Although the ingestion of button batteries (BBs) in neonates is exceedingly rare, it poses severe clinical challenges with potentially catastrophic outcomes. The increase in such cases, particularly among toddlers, is largely due to the widespread availability of portable electronic devices. Ingestion of button or disk batteries is notably more dangerous than other foreign bodies, often leading to acute complications such as burns and esophageal perforation. This report details the diagnosis and management of a 21-day-old neonate who presented with a button battery lodged in the esophagus. CASE REPORT We report the case of a 21-day-old neonate who initially presented with 3 days of persistent vomiting. Diagnostic imaging with a chest radiograph revealed a radiopaque foreign body in the esophagus, identified as a button battery. It was suspected that the battery was inadvertently placed in the neonate's mouth by a sibling with an intellectual disability. Initial attempts to remove the battery using 4-, 5-, and 6-mm endoscopes were unsuccessful. However, extraction was eventually accomplished with a 2.5-mm rigid esophagoscope. Following the removal, the neonate developed significant complications, including a tracheoesophageal fistula and esophageal stenosis. Extensive follow-up care led to a full recovery, demonstrating resilience despite the severe initial challenges. CONCLUSIONS This report emphasizes the critical need for swift identification and removal of ingested button batteries. It details the diagnostic and management strategies employed for a neonate, illustrating the urgency and precision required in such cases.</p>","PeriodicalId":39064,"journal":{"name":"American Journal of Case Reports","volume":"26 ","pages":"e944479"},"PeriodicalIF":1.0,"publicationDate":"2025-02-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11809932/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143257005","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Stephanie Matsuura, Reema Ghatnekar, Kelly Yamasato
{"title":"Extreme Pregnancy-Induced Hypertriglyceridemia Resulting in Pancreatitis: A Case Report.","authors":"Stephanie Matsuura, Reema Ghatnekar, Kelly Yamasato","doi":"10.12659/AJCR.946974","DOIUrl":"10.12659/AJCR.946974","url":null,"abstract":"<p><p>BACKGROUND Pancreatitis in pregnancy is rare but increasing in frequency, posing potentially serious maternal and fetal consequences. Early recognition of its variable presentations is essential for effective management. CASE REPORT We describe a case of recurrent pancreatitis across multiple pregnancies due to severe pregnancy-induced hypertriglyceridemia. This 33-year-old gravida 7 para 5 woman presented at 37 weeks of gestation with epigastric pain, nausea, and emesis after a high-fat meal. She had a history of hypertriglyceridemic pancreatitis in 4 previous pregnancies but only mildly elevated triglyceride levels outside of pregnancy. On presentation, she was promptly diagnosed with pregnancy-induced hypertriglyceridemic pancreatitis and treated with intravenous insulin, bowel rest, niacin, and gemfibrozil, resulting in clinical improvement by hospital day 5. She underwent labor induction, delivering a healthy female infant vaginally without complications. She was discharged on postpartum day 2 with niacin and gemfibrozil, although she later discontinued them. However, she was found to be doing well at her postpartum visits. CONCLUSIONS This case highlights the recurrent nature of pregnancy-induced hypertriglyceridemic pancreatitis, even in the absence of significant baseline hypertriglyceridemia. This report therefore increases awareness of the potential severity of pregnancy-induced hypertriglyceridemia. It also suggests that providers should consider hypertriglyceridemia in pregnant women with pancreatitis, even in the absence of a significant hypertriglyceridemia history. The timely and accurate diagnosis of pregnancy-induced hypertriglyceridemic pancreatitis should lead to effective, expedited treatment that can improve patient outcomes in this life-threatening condition.</p>","PeriodicalId":39064,"journal":{"name":"American Journal of Case Reports","volume":"26 ","pages":"e946974"},"PeriodicalIF":1.0,"publicationDate":"2025-02-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11809930/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143190583","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Septic Thrombophlebitis in the Portal Veins: A Case of Pylephlebitis Linked to Colo-Venous Fistula and Diverticulitis.","authors":"Larissa Befurt, Arash Ghadim Khani, Ernst-Joachim Malzfeldt, Alexander Tobisch, Asad Kutup","doi":"10.12659/AJCR.946107","DOIUrl":"10.12659/AJCR.946107","url":null,"abstract":"<p><p>BACKGROUND Pylephlebitis is a septic thrombosis in the portal or mesenteric venous system that occurs as a complication of an intra-abdominal inflammatory process. We present the case of a 43-year-old man with a septic thrombosis of the portal drainage area and a colo-venous fistula complicating a sigmoid diverticulitis. CASE REPORT The patient presented after collapsing at home with unspecific symptoms such as diffuse abdominal pain. On physical examination, he was tachycardic (140 beats/minute), with chills and fever to a temperature of 38.3°C. The remaining examination findings were unremarkable. Initial laboratory investigations were significant for an infection. Computed tomography (CT) with contrast of the abdomen and pelvis showed a sigmoid diverticulitis with covered perforation, forming a colo-venous fistula to the superior mesenteric vein, with gas trapped in the hepatic portal branches and a pylephlebitis of the superior mesenteric vein. An en bloc resection of the sigmoid and segmental resection of adherent ileum were performed, preserving intestinal continuity. Pylephlebitis was treated conservatively with antibiotics and anticoagulation. The patient recovered completely. CONCLUSIONS Pylephlebitis is the term used for septic thrombophlebitis of the portal veins. It is a rare but potentially fatal complication of an intra-abdominal infection, which can lead to septic shock, intestinal ischemia, or liver abscesses. The unspecific symptoms make early diagnosis difficult. Surgical removal of the inflammatory process, antibiotic therapy, and anticoagulation form the cornerstones of therapy. Although the unrestricted use of anticoagulation in pylephlebitis continues to be the subject of controversy due to conflicting results in the literature.</p>","PeriodicalId":39064,"journal":{"name":"American Journal of Case Reports","volume":"26 ","pages":"e946107"},"PeriodicalIF":1.0,"publicationDate":"2025-02-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11804829/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143123711","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Insulin Resistance in an Underweight Woman with Polycystic Ovary Syndrome: A Case Study.","authors":"Besa Gacaferri Lumezi, Violeta Lokaj-Berisha","doi":"10.12659/AJCR.945814","DOIUrl":"10.12659/AJCR.945814","url":null,"abstract":"<p><p>BACKGROUND Polycystic ovarian syndrome (PCOS) is one of the most prevalent endocrine disorders affecting women of reproductive age. PCOS has a typical presentation with obesity, hirsutism, and menstrual irregularity but can also be associated with insulin resistance and an increased risk of type 2 diabetes mellitus. This report presents the case of a 24-year-old underweight woman with a body mass index (BMI) of 15.9 kg/m2 with insulin resistance, hirsutism, and a diagnosis of PCOS. CASE REPORT A 24-year-old woman presented with hyperandrogenic signs, hirsutism, acne, and irregular menstrual cycles. During the clinical examination, acne vulgaris on the face was recorded, but not alopecia or acanthosis nigricans. Blood pressure was 110/70 mmHg, WHR (waist-to-hip-ratio)=87 and BMI (body mass index)=15.9. Ferriman-Gallwey's scoring system was used to assess for hirsutism. All hematological and biochemical results were normal. Hormonal tests revealed elevated testosterone, androstenedione, and 17-OH progesterone levels, confirming the diagnosis. Conditions such as Cushing's syndrome, hyperprolactinemia, and thyroid disorders were excluded based on normal levels of adrenocorticotropic hormone, cortisol, prolactin, triiodothyronine, thyroxine, and thyroid-stimulating hormone. Despite the patient's underweight status, insulin resistance was identified with OGTT (oral glucose tolerance test), making it the primary treatment target. Treatment addressed the underlying pathology with metformin and dexamethasone. Significant improvement in menstrual cycle, acne, and hirsutism was observed after 6 months. CONCLUSIONS This report has highlighted that when patients with PCOS also have insulin resistance, they may be of normal weight or underweight and require a multidisciplinary approach to diagnosis and management.</p>","PeriodicalId":39064,"journal":{"name":"American Journal of Case Reports","volume":"26 ","pages":"e945814"},"PeriodicalIF":1.0,"publicationDate":"2025-02-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11803316/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143081134","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"A Role for IgE in Post-Orgasmic Illness Syndrome: Successful Omalizumab Treatment in Absence of Positive Skin Test to Self Semen.","authors":"Orit Gourgy Hacohen, Nizar Abo-Helo, Shai Cohen","doi":"10.12659/AJCR.945529","DOIUrl":"10.12659/AJCR.945529","url":null,"abstract":"<p><p>BACKGROUND Post-orgasmic illness syndrome (POIS) is a rare disease with an unknown etiology and pathophysiology. Several publications have previously reported successful results with various treatments, including antihistamines, selective serotonin reuptake inhibitors, and non-steroidal anti-inflammatory drugs. Here, we report the successful treatment of POIS with omalizumab in a patient with negative skin tests for self-semen with complete resolution of symptoms. So far, there has been only 1 previous report of omalizumab treatment for this syndrome. CASE REPORT A 22-year-old man with constitutional, neurological, gastrointestinal, and allergic-like symptoms after ejaculation presented to our clinic. The patient had a negative intradermal test for self-semen, normal tryptase levels (pre- and post-ejaculation), and elevated total IgE. Several treatments had been prescribed. The most efficient combination treatment prior to omalizumab included high-dose antihistamines plus anti-inflammatory medications (steroidal or non-steroidal). This regimen alleviated some but not all his symptoms. Due to the nature of the symptoms, we hypothesized that IgE and mast cells could be involved in the pathophysiology of POIS in this patient. He was started on omalizumab, with complete resolution of symptoms. After 7 months, a treatment cessation attempt resulted in recurrence of the symptoms. Currently, while on omalizumab, the patient is symptom-free and feels comfortable engaging in sexual activity. CONCLUSIONS Omalizumab may be considered for the treatment of POIS in patients with allergy-like symptoms and symptoms that cannot be controlled with other medications, even in the absence of a positive skin test to semen. Lower doses may be ineffective.</p>","PeriodicalId":39064,"journal":{"name":"American Journal of Case Reports","volume":"26 ","pages":"e945529"},"PeriodicalIF":1.0,"publicationDate":"2025-02-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11803315/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143075714","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Joao Da Costa Rodrigues, Corinne Gazarian, Julien Maillard, Gergely Albu, Benjamin Assouline, Frédéric Lador, Eduardo Schiffer
{"title":"Emergency ECMO Deployment During Liver Transplantation in Portopulmonary Hypertension Patients.","authors":"Joao Da Costa Rodrigues, Corinne Gazarian, Julien Maillard, Gergely Albu, Benjamin Assouline, Frédéric Lador, Eduardo Schiffer","doi":"10.12659/AJCR.946268","DOIUrl":"10.12659/AJCR.946268","url":null,"abstract":"<p><p>BACKGROUND Portopulmonary hypertension (POPH) is part of Group 1 of the clinical classification of pulmonary hypertension and represents 5-15% of patients with pulmonary hypertension, with a 5-year mortality rate of 40%. The implementation of preoperative pulmonary antihypertensive treatment allows liver transplantation depending on clinical response, which constitutes potential curative treatment. Uncontrolled pulmonary hypertension is a major risk factor of perioperative morbimortality in the context of liver transplantation. In case of major hemodynamic instability, extracorporeal membrane oxygenation (ECMO) can be placed to manage circulatory failure. We describe a case of a patient with POPH in whom an emergency ECMO was implanted during liver transplantation complicated by an intraoperative worsening of pulmonary vascular resistances leading to cardiac arrest. CASE REPORT A 16-year-old patient with POPH had an orthotopic liver transplantation (OLT) after management of pulmonary hypertension with a triple antihypertensive therapy, which was complicated by hemorrhagic shock. Management of hemorrhagic shock led to greatly increased pulmonary vascular resistances, which led to a perioperative cardiac arrest, necessitating the implantation of a veno-arterial ECMO, allowing the completion of critical surgical steps before admission to the intensive care unit. CONCLUSIONS POPH is a challenge in the perioperative setting. OLT is a therapeutic option in that setting. ECMO may be necessary for patients with POPH in the perioperative hemodynamic management during OLT. In highly selected cases, VA-ECMO implantation and timing should be discussed by a multidisciplinary team before induction. The emergency perioperative implantation of ECMO is a realistic alternative.</p>","PeriodicalId":39064,"journal":{"name":"American Journal of Case Reports","volume":"26 ","pages":"e946268"},"PeriodicalIF":1.0,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11791688/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143075717","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}