Rare complication of a type IV paraoesophageal hiatal hernia in a Marfan syndrome patient

2021 ◽  
Vol 14 (4) ◽  
pp. e239641
Author(s):  
Lachlan Hou ◽  
Sunita Dhanda ◽  
Dan Xu
Keyword(s):  
Aortic Dissection ◽  
Hiatal Hernia ◽  
Marfan Syndrome ◽  
Rare Complication ◽  
Left Lung ◽  
Lower Lobe ◽  
Type Iv ◽  
History Of ◽  
Medical Histories ◽  
Long Term Prognosis

A 77-year-old female patient with Marfan syndrome presented with a 1-week history of worsening malaise and poor appetite. This was associated with a dramatic unintentional loss of weight as well as shortness of breath worsened by exertion. She has significant medical histories of a hiatal hernia and chronic type B aortic dissection. CT scan of the chest was able to confirm a type IV paraoesophageal hiatal hernia compressing on the lower lobe of her left lung without any progression of her aortic dissection. As surgical intervention was contraindicated in light of her advanced age and comorbidities, she was managed conservatively with a clinically satisfied outcome in the short term. Her long-term prognosis, however, is still poor with a high mortality of 1 and 5 years.

Fatal Aortic Dissection in a Patient With a Family History of Marfan Syndrome

2008 ◽  
Vol 112 (2, Part 2) ◽  
pp. 472-475 ◽  
Author(s):  
Meredith L. Birsner ◽  
John L. Farber ◽  
Vincenzo Berghella
Keyword(s):  
Family History ◽  
Aortic Dissection ◽  
Marfan Syndrome ◽  
History Of

scholarly journals Pulmonary Gangrene - Severe And Rare Complication Of Pneumonia

2014 ◽  
Vol 7 (2) ◽  
pp. 155-160 ◽  
Author(s):  
Guergana Petrova ◽  
Penka I. Perenovska ◽  
Dimitrinka Miteva ◽  
Radost T. Kabakchieva ◽  
Ognyan G. Brankov ◽  
...  
Keyword(s):  
Rare Complication ◽  
Left Lung ◽  
Lower Lobe ◽  
Good Health ◽  
Surgical Department ◽  
Intravenous Antibiotics ◽  
Histology Result ◽  
Severe Infections ◽  
The Right ◽  
Surgery Department

SummaryPneumonia is an inflammatory lung disorder characterized by consolidation due to presence of exudates in the alveolar spaces. Most pneumonias can be effectively treated with appropriate oral antibiotics, with intravenous antibiotics being reserved for those with severe infections. We present two cases of girls admitted in our clinic with pneumonia where our conventional therapy was not sufficient. Case 1: A 15-year-old girl with cystic fibrosis, with left lobular pneumonia, for which an aggressive conservative treatment was initiated. After significant improvement, sudden detorioration and pneumothorax of the left lung occurred. She was transferred to the surgical department for intervention. Due to failure to respond to initial drainage she underwent thoracotomy and resection of the left lower lobe of the lung. The histology result confirmed gangrene. Case 2: A four-year old girl was treated for pneumonia in the right lung with aggressive intravenous antibiotic. After temporary improvement sudden deterioration was observed. The patient was transferred to the surgery department, where pulmonary gangrene was confirmed. After the lower lobe of the right lung was resected, she was discharged in good health. The careful follow up, accurate diagnosis and correct medication choice are crucial for reducing the complications of “common” pneumonia.

scholarly journals Spontaneous Aortic Dissecting Hematoma in Two Dogs

2005 ◽  
Vol 17 (5) ◽  
pp. 492-497 ◽  
Author(s):  
Theresa Marie Boulineau ◽  
Lydia Andrews-Jones ◽  
William Van Alstine
Keyword(s):  
Aortic Dissection ◽  
Marfan Syndrome ◽  
Pericardial Tamponade ◽  
Ascending Aorta ◽  
Thoracic Cavity ◽  
Mediastinal Hematoma ◽  
Intimal Proliferation ◽  
First Case ◽  
History Of ◽  
Gross Lesions

This report describes 2 cases of spontaneous aortic dissecting hematoma in young Border Collie and Border Collie crossbred dogs. Histology was performed in one of the cases involving an unusual splitting of the elastin present within the wall of the aorta, consistent with elastin dysplasia as described in Marfan syndrome in humans. The first case involved a young purebred Border Collie that died suddenly and the second case involved a Border Collie crossbred dog that died after a 1-month history of seizures. Gross lesions included pericardial tamponade with dissection of the ascending aorta in the former case and thoracic cavity hemorrhage, mediastinal hematoma, and aortic dissection in the latter. Histologic lesions in the case of the Border Collie crossbred dog included a dissecting hematoma of the ascending aorta with elastin dysplasia and right axillary arterial intimal proliferation.

scholarly journals Endovascular Repair as a Bridge to Surgical Repair of an Aortobronchial Fistula Complicating Chronic Residual Aortic Dissection

2014 ◽  
Vol 41 (2) ◽  
pp. 198-202 ◽  
Author(s):  
Jesus M. Matos ◽  
Kim I. de la Cruz ◽  
Maral Ouzounian ◽  
Ourania Preventza ◽  
Scott A. LeMaire ◽  
...  
Keyword(s):  
Aortic Dissection ◽  
Surgical Repair ◽  
Left Lung ◽  
Lower Lobe ◽  
Presumptive Diagnosis ◽  
Stent Grafting ◽  
Thoracic Aortic Dissection ◽  
Computed Tomographic ◽  
Aortobronchial Fistula ◽  
Definitive Surgery

Endovascular and open surgical repair have been used in patients with descending thoracic aortic dissection; however, the appropriate treatment is debated. We describe the case of a 60-year-old woman who had a symptomatic, chronic, residual, descending thoracic aortic dissection that was complicated by an aortobronchial fistula. She underwent emergent thoracic endovascular stent-grafting but remained symptomatic. Computed tomographic angiograms showed a contained rupture into the lower lobe of the left lung. The patient underwent definitive surgery to remove the stents, reconstruct the aorta, and resect the nonviable lung tissue. The remainder of her postoperative course was uneventful, and she was discharged from the hospital 13 days after the 2nd operation. Results of genetic testing confirmed an earlier presumptive diagnosis of Marfan syndrome. In an emergency, the best initial option for patients with a complicated descending thoracic aortic dissection might be thoracic endovascular aortic repair, which could serve as a bridge to definitive open repair.

Ascending aortic aneurysm and diaphragmatic hernia in a case of Marfan syndrome

2017 ◽  
Vol 25 (5) ◽  
pp. 378-380 ◽  
Author(s):  
Jignesh Kothari ◽  
Manish Hinduja ◽  
Kinnaresh Baria ◽  
Himani Pandya
Keyword(s):  
Aortic Aneurysm ◽  
Hiatal Hernia ◽  
Marfan Syndrome ◽  
Diaphragmatic Hernia ◽  
Rare Case ◽  
Surgical Repair ◽  
Gastrointestinal Involvement ◽  
Ascending Aortic Aneurysm ◽  
Diagnosis And Management ◽  
Type Iv

Marfan syndrome commonly affects the skeletal, ocular, and cardiovascular systems. Involvement of the gastrointestinal system is known but uncommon. Intervention depends upon the system involved and the severity of symptoms. Special awareness is required for the diagnosis and management of gastrointestinal involvement in these patients. We report a rare case of simultaneous surgical repair of an ascending aortic aneurysm and a type IV hiatal hernia in a 35-year-old man with Marfan syndrome.

scholarly journals A Case of an Abdominal Aortic Dissection in a Hemodynamically Stable Marfan Syndrome Patient Presenting without Pain

2020 ◽  
Vol 2020 ◽  
pp. 1-4
Author(s):  
Hussein Al-Mohamad ◽  
Kara Stout ◽  
Taryn Bolling ◽  
Ronald Walsh
Keyword(s):  
Aortic Dissection ◽  
Ct Scan ◽  
Marfan Syndrome ◽  
Connective Tissue Disorder ◽  
Dissecting Aneurysm ◽  
Aortic Aneurysms ◽  
Abdominal Aortic ◽  
Fibrillin 1 ◽  
History Of ◽  
Abdominal Aortic Dissection

Introduction. Marfan syndrome (MFS) is a rare connective tissue disorder attributed to a defect in the fibrillin-1 gene. Aortic aneurysms and dissection are common causes of morbidity and mortality in Marfan syndrome. Case Report. A 43-year-old female with a history of MFS and a 4.0 cm dilated ascending aorta presented to her cardiologist reporting that since a C-section two years prior, the left side of her abdomen painlessly protruded when standing. An outpatient CT scan of the abdomen/pelvis noted a 5.5 cm abdominal aortic dissection, and she was directed to the hospital. Repeat CT scan of the abdomen/pelvis revealed a 5.6 cm dissecting aneurysm of the infrarenal abdominal aorta. The patient was admitted to the ICU and started on a nitroglycerin drip to maintain systolic blood pressure less than 110 mmHg. The patient underwent repair of her abdominal aortic dissection via a retroperitoneal approach, and she tolerated the procedure well. She was started on metoprolol tartrate 12.5 mg BID and aspirin 81 mg postoperatively. She was safely discharged with follow-up care. Conclusion. This case stresses the importance of having a low threshold to obtain imaging in a MFS patient with protruding abdomen, even though the patient may not have pain and be hemodynamically stable.

scholarly journals Mycoplasma pneumoniae infection mimicking tuberculous pleurisy in a young woman: a case report and literature review

2020 ◽  
Vol 48 (4) ◽  
pp. 030006052091870
Author(s):  
Peng Wen ◽  
Min Wei ◽  
Xue Guo ◽  
Yu-Rong Xu
Keyword(s):  
Pleural Effusion ◽  
Adenosine Deaminase ◽  
Mycoplasma Pneumoniae ◽  
Left Lung ◽  
Lower Lobe ◽  
Anterior Lobe ◽  
Immunoglobulin M ◽  
Recent Infection ◽  
History Of ◽  
Tuberculous Pleurisy

A 30-year-old woman was admitted to a different hospital with a 2-day history of fever, cough, and expectoration. She had a history of left pulmonary tuberculosis 8 years previously. Chest computed tomography showed an infiltrate in the inferior lobe of the left lung and spot-like calcifications in the anterior lobe of the upper left lobe and lower lobe of the left lung. After antibacterial treatment, the patient’s condition deteriorated and she developed significant pleural effusion on the left side. The pleural effusion assay showed a lymphocyte-predominant exudate with a significantly increased adenosine deaminase level. The patient was transferred to our hospital with a suspected diagnosis of tuberculous pleuritis. A serum test for Mycoplasma pneumoniae-specific immunoglobulin M was positive. Because of the limitations of this test in determining the occurrence of recent infection, a thoracoscopic pleural biopsy was performed, and M. pneumoniae DNA was detected in the biopsy tissue using M. pneumoniae-specific polymerase chain reaction. Thus, the patient was diagnosed with M. pneumoniae-related parapneumonic effusion. Clinicians must be aware of the usefulness and limitations of a high adenosine deaminase level and know that lymphocyte predominance in pleural effusion does not always indicate tuberculous pleurisy, especially in areas of high tuberculosis prevalence.

scholarly journals Rare Case of Upside-Down Stomach in Advanced Hiatal Hernia

2019 ◽  
Vol 7 (31) ◽  
pp. 52-55
Author(s):  
Nooraldin Merza ◽  
John Lung ◽  
Omar Bazzaz ◽  
Farah Aljuboory ◽  
Mazin Saadaldin
Keyword(s):  
Myelodysplastic Syndrome ◽  
Hiatal Hernia ◽  
Posterior Mediastinum ◽  
Surgical Interventions ◽  
X Ray ◽  
Type Iv ◽  
Acute Distress ◽  
History Of ◽  
Chest X Ray ◽  
High Degree

Upside-down stomach (UDS) is the rarest type of hiatal hernia, which is migration and volvulus of the entire stomach into the posterior mediastinum. We present a case of a patient with chronic gastroesophageal reflux disease (GERD) and myelodysplastic syndrome diagnosed 3 months ago, admitted for syncope and signs of aspiration pneumonia, with incidental findings of hiatal hernia type IV with UDS. A 74-year-old Caucasian male presented to the emergency department complaining of syncope. The patient had been experiencing generalized weakness and a history of multiple falls without head injury. Upon physical examination the patient was not in acute distress. The chest x-ray was suspicious for pneumonia. Upon admission, the patient had a hemoglobin level of 6.8 g/dl. He was transfused with multiple units of packed RBCs during his stay for treatment of his anemia secondary to myelodysplastic syndrome. A CT thorax revealed a large hiatal hernia type IV with UDS within the posterior mediastinum. Surgical intervention was recommended, but the patient elected to postpone surgery due to no symptoms and was discharged. The patient died one month later from complications related to myelodysplastic syndrome. The diagnosis of upside down stomach can be suspected on chest X-ray but requires a high degree of suspicion. All surgical interventions for UDS must consider the overall prognosis of the patient and estimated improved quality of life before repair, especially with the high recurrence of UDS even after surgical repair.  

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