A 72-year-old woman comes to the physician for follow-up care. One year ago, she was diagnosed with a 3.8-cm infrarenal aortic aneurysm found incidentally on abdominal ultrasound. She has no complaints. She has hypertension, type 2 diabetes mellitus, and COPD. Current medications include hydrochlorothiazide, lisinopril, glyburide, and an albuterol inhaler. She has smoked a pack of cigarettes daily for 45 years. Her temperature is 37°C (98.6°F), pulse is 90/min, respirations are 12/min, and blood pressure is 145/85 mm Hg. Examination shows a faint abdominal bruit on auscultation. Ultrasonography of the abdomen shows a 4.9-cm saccular dilation of the infrarenal aorta. Which of the following is the most appropriate next step in management?
Q2
A 58-year-old man is brought to the emergency department by his family because of severe upper back pain, which he describes as ripping. The pain started suddenly 1 hour ago while he was watching television. He has hypertension for 13 years, but he is not compliant with his medications. He denies the use of nicotine, alcohol or illicit drugs. His temperature is 36.5°C (97.7°F), the heart rate is 110/min and the blood pressure is 182/81 mm Hg in the right arm and 155/71 mm Hg in the left arm. CT scan of the chest shows an intimal flap limited to the descending aorta. Intravenous opioid analgesia is started. Which of the following is the best next step in the management of this patient condition?
Q3
A 68-year-old man presents for a screening ultrasound scan. He has been feeling well and is in his usual state of good health. His medical history is notable for mild hypertension and a 100-pack-year tobacco history. He has a blood pressure of 128/86 and heart rate of 62/min. Physical examination is clear lung sounds and regular heart sounds. On ultrasound, an infrarenal aortic aneurysm of 4 cm in diameter is identified. Which of the following is the best initial step for this patient?
Q4
A 55-year-old man is brought to the emergency department 30 minutes after the sudden onset of severe, migrating anterior chest pain, shortness of breath, and sweating at rest. He has hypertension, hypercholesterolemia, and type 2 diabetes mellitus. Medications include atorvastatin, hydrochlorothiazide, lisinopril, and metformin. He has smoked one pack of cigarettes daily for 25 years. He is in severe distress. His pulse is 110/min, respirations are 20/min, and blood pressure is 150/85 mm Hg. Pulse oximetry on room air shows an oxygen saturation of 98%. Cardiac examination shows a grade 3/6, high-pitched, blowing, diastolic murmur heard best over the right sternal border. The lungs are clear to auscultation. Femoral pulses are decreased bilaterally. An ECG shows sinus tachycardia and left ventricular hypertrophy. Which of the following is the most likely diagnosis?
Q5
A 10-month-old boy is brought to the clinic with a history of recurrent episodes of stridor and wheezing. His mother reports that his wheezing is exacerbated by crying, feeding, and flexion of the neck, and is relieved by extension of the neck. Occasionally he vomits after feeding. What is the most likely diagnosis?
Q6
A 43-year-old man comes to the physician because of increasing shortness of breath for 1 month. He has been using two pillows at night but frequently wakes up feeling as if he is choking. Five months ago, he underwent surgery for creation of an arteriovenous fistula in his left upper arm. He has hypertension and chronic kidney disease due to reflux nephropathy. He receives hemodialysis three times a week. His current medications are enalapril, vitamin D3, erythropoietin, sevelamer, and atorvastatin. His temperature is 37.1°C (98.8°F), respirations are 22/min, pulse is 103/min and bounding, and blood pressure is 106/58 mm Hg. Examination of the lower extremities shows bilateral pitting pedal edema. There is jugular venous distention. A prominent thrill is heard over the brachiocephalic arteriovenous fistula. There are crackles heard at both lung bases. Cardiac examination shows an S3 gallop. The abdomen is soft and nontender. Which of the following is the most likely cause of this patient's symptoms?
Q7
A 75-year-old Caucasian man presents to the emergency department with abdominal pain. The patient states he was at home eating dinner when he began to experience severe abdominal pain. The patient has a past medical history of diabetes, hypertension, and atherosclerosis. He lives at home alone, smokes cigarettes, and drinks 1 to 2 alcoholic drinks per day. The patient is given IV morphine and an ultrasound is obtained demonstrating a dilated abdominal aorta. The patient states that his father died of a similar finding and is concerned about his prognosis. Which of the following is the greatest risk factor for this patient's presentation?
Q8
In a routine medical examination, a young man is noted to be tall with slight scoliosis and pectus excavatum. He had been told that he was over the 95% percentile for height as a child. Auscultation reveals a heart murmur, and transthoracic echocardiography shows an enlarged aortic root and mitral valve prolapse. Blood screening for fibrillin-1 (FBN1) gene mutation is positive and plasma homocysteine is normal. This patient is at high risk for which of the following complications?
Q9
A 67-year-old man presents to the emergency department with abdominal pain that started 1 hour ago. The patient has a past medical history of diabetes and hypertension as well as a 40 pack-year smoking history. His blood pressure is 107/58 mmHg, pulse is 130/min, respirations are 23/min, and oxygen saturation is 98% on room air. An abdominal ultrasound demonstrates focal dilation of the aorta with peri-aortic fluid. Which of the following is the best next step in management?
Q10
A 31 year-old-man presents to an urgent care clinic with symptoms of lower abdominal pain, bloating, bloody diarrhea, and fullness, all of which have become more frequent over the last 3 months. Rectal examination reveals a small amount of bright red blood. His vital signs include: temperature 36.7°C (98.0°F), blood pressure 126/74 mm Hg, heart rate 74/min, and respiratory rate 14/min. Colonoscopy is performed, showing extensive mucosal erythema, induration, and pseudopolyps extending from the rectum to the splenic flexure. Given the following options, what is the most appropriate treatment to induce remission in this patient?
Thoracic aortic aneurysm management US Medical PG Practice Questions and MCQs
Question 1: A 72-year-old woman comes to the physician for follow-up care. One year ago, she was diagnosed with a 3.8-cm infrarenal aortic aneurysm found incidentally on abdominal ultrasound. She has no complaints. She has hypertension, type 2 diabetes mellitus, and COPD. Current medications include hydrochlorothiazide, lisinopril, glyburide, and an albuterol inhaler. She has smoked a pack of cigarettes daily for 45 years. Her temperature is 37°C (98.6°F), pulse is 90/min, respirations are 12/min, and blood pressure is 145/85 mm Hg. Examination shows a faint abdominal bruit on auscultation. Ultrasonography of the abdomen shows a 4.9-cm saccular dilation of the infrarenal aorta. Which of the following is the most appropriate next step in management?
A. Elective endovascular aneurysm repair (Correct Answer)
B. Adjustment of cardiovascular risk factors and follow-up ultrasound in 12 months
C. Adjustment of cardiovascular risk factors and follow-up ultrasound in 6 months
D. Elective open aneurysm repair
E. Adjustment of cardiovascular risk factors and follow-up CT in 6 months
Explanation: ***Elective endovascular aneurysm repair***
- The patient's **infrarenal aortic aneurysm** has grown from 3.8 cm to 4.9 cm in one year, approaching the **5.0 cm threshold for intervention in women** (compared to 5.5 cm for men). The **rapid growth rate of 1.1 cm/year** (normal is <0.5 cm/year) significantly increases rupture risk and is an indication for intervention even before reaching the absolute size threshold.
- Given her multiple comorbidities (hypertension, diabetes, COPD, 45 pack-year smoking history), **endovascular aneurysm repair (EVAR)** is preferred over open repair due to lower perioperative morbidity and mortality in high-risk surgical candidates.
- The combination of near-threshold size and rapid growth makes elective repair appropriate now rather than continued surveillance.
*Adjustment of cardiovascular risk factors and follow-up ultrasound in 12 months*
- While **risk factor modification** (smoking cessation, blood pressure control) is essential, it is insufficient as the primary management given the aneurysm's significant growth and imminent rupture risk.
- A 12-month follow-up interval is too long for a rapidly growing aneurysm (grew 1.1 cm in the past year), as this increases the risk of rupture without intervention.
*Adjustment of cardiovascular risk factors and follow-up ultrasound in 6 months*
- **Risk factor management** is important but does not address the immediate need for intervention due to the aneurysm's size approaching the threshold and concerning growth rate.
- While 6-month surveillance might be considered for smaller aneurysms with slower growth, this aneurysm's rapid expansion rate suggests it will exceed 5.0 cm well before the next surveillance interval, increasing rupture risk unnecessarily.
*Elective open aneurysm repair*
- **Open aneurysm repair** is an effective treatment but carries significantly higher perioperative risks (30-day mortality 3-5% vs 1-2% for EVAR) compared to endovascular repair, especially in patients with multiple comorbidities.
- Given this patient's COPD, smoking history, and cardiovascular risk factors, EVAR is the preferred approach to minimize surgical stress and improve perioperative outcomes.
*Adjustment of cardiovascular risk factors and follow-up CT in 6 months*
- **Risk factor modification** alone is insufficient given the aneurysm's proximity to intervention threshold and rapid growth rate.
- While CT provides more detailed anatomic imaging for surgical planning, continued surveillance is inappropriate when the patient already meets criteria for intervention. Additionally, CT involves radiation exposure and is typically reserved for pre-operative planning rather than routine surveillance.
Question 2: A 58-year-old man is brought to the emergency department by his family because of severe upper back pain, which he describes as ripping. The pain started suddenly 1 hour ago while he was watching television. He has hypertension for 13 years, but he is not compliant with his medications. He denies the use of nicotine, alcohol or illicit drugs. His temperature is 36.5°C (97.7°F), the heart rate is 110/min and the blood pressure is 182/81 mm Hg in the right arm and 155/71 mm Hg in the left arm. CT scan of the chest shows an intimal flap limited to the descending aorta. Intravenous opioid analgesia is started. Which of the following is the best next step in the management of this patient condition?
A. Intravascular ultrasound
B. Emergency surgical intervention
C. Sublingual nitroglycerin
D. Intravenous esmolol (Correct Answer)
E. Oral metoprolol and/or enalapril
Explanation: ***Intravenous esmolol***
- This patient presents with an **acute aortic dissection** (descending aorta, Type B), characterized by sudden severe ripping back pain and a significant blood pressure difference between the arms [1]. The immediate priority is to reduce **heart rate** and **blood pressure** to decrease shear stress on the aortic wall and prevent progression of the dissection.
- **Intravenous beta-blockers** like esmolol are the first-line medical treatment for uncomplicated Type B aortic dissections, as they rapidly decrease heart rate and blood pressure, which helps to mitigate further aortic injury.
*Intravascular ultrasound*
- While intravascular ultrasound can provide detailed imaging of the aorta, it is an **invasive procedure** and not the immediate next step in managing an acute, hemodynamically unstable condition like aortic dissection where rapid blood pressure control is paramount.
- The patient already has a diagnostic CT scan confirming the intimal flap; thus, additional imaging during the acute stabilization phase is not typically the first priority over medical management [2].
*Emergency surgical intervention*
- **Emergency surgical intervention** is primarily indicated for **Type A aortic dissections** (involving the ascending aorta) or for complicated Type B dissections (e.g., malperfusion, rupture, rapid expansion) [1].
- This patient has an **uncomplicated Type B dissection** (limited to the descending aorta) that is initially managed medically with aggressive heart rate and blood pressure control.
*Sublingual nitroglycerin*
- **Nitroglycerin** primarily causes **vasodilation**, which can lower blood pressure but also induces reflex tachycardia, potentially increasing shear stress on the dissected aorta.
- It is **contraindicated** in acute aortic dissection as the increase in heart rate can worsen the dissection.
*Oral metoprolol and/or enalapril*
- **Oral medications** like metoprolol and enalapril are not suitable for the **initial acute management** of aortic dissection because their onset of action is too slow to achieve rapid and precise control of heart rate and blood pressure.
- **Intravenous agents** are required for immediate and titratable blood pressure and heart rate reduction in this emergency setting.
Question 3: A 68-year-old man presents for a screening ultrasound scan. He has been feeling well and is in his usual state of good health. His medical history is notable for mild hypertension and a 100-pack-year tobacco history. He has a blood pressure of 128/86 and heart rate of 62/min. Physical examination is clear lung sounds and regular heart sounds. On ultrasound, an infrarenal aortic aneurysm of 4 cm in diameter is identified. Which of the following is the best initial step for this patient?
A. Reassurance
B. Beta-blockers
C. Urgent repair
D. Surveillance (Correct Answer)
E. Elective repair
Explanation: **Surveillance**
- An **infrarenal aortic aneurysm** of 4 cm in diameter in an asymptomatic patient is typically managed with **regular surveillance** to monitor for growth.
- Surgical intervention is generally reserved for aneurysms larger than 5.5 cm or those that are rapidly expanding or symptomatic.
*Reassurance*
- While it's important to provide reassurance, simply doing so without a concrete plan for follow-up would be inappropriate given the potential for **aneurysm expansion** and rupture.
- The patient's **tobacco history** is a significant risk factor for aneurysm progression and warrants monitoring.
*Beta-blockers*
- Beta-blockers may be part of the medical management for **hypertension** and could theoretically slow aneurysm growth by reducing pulsatile stress.
- However, they are not the primary **initial step** for an asymptomatic aneurysm of this size and do not replace the need for surveillance.
*Urgent repair*
- **Urgent repair** is indicated for symptomatic aneurysms, those that are rapidly expanding, or those showing signs of rupture or impending rupture, none of which are present here.
- A 4 cm aneurysm in an asymptomatic patient does not meet the criteria for **urgent intervention**.
*Elective repair*
- **Elective repair** is typically considered for aneurysms exceeding 5.5 cm in diameter or those that are symptomatic or rapidly growing.
- A 4 cm aneurysm is below the threshold for **elective repair** in an asymptomatic patient without other high-risk features.
Question 4: A 55-year-old man is brought to the emergency department 30 minutes after the sudden onset of severe, migrating anterior chest pain, shortness of breath, and sweating at rest. He has hypertension, hypercholesterolemia, and type 2 diabetes mellitus. Medications include atorvastatin, hydrochlorothiazide, lisinopril, and metformin. He has smoked one pack of cigarettes daily for 25 years. He is in severe distress. His pulse is 110/min, respirations are 20/min, and blood pressure is 150/85 mm Hg. Pulse oximetry on room air shows an oxygen saturation of 98%. Cardiac examination shows a grade 3/6, high-pitched, blowing, diastolic murmur heard best over the right sternal border. The lungs are clear to auscultation. Femoral pulses are decreased bilaterally. An ECG shows sinus tachycardia and left ventricular hypertrophy. Which of the following is the most likely diagnosis?
A. Esophageal rupture
B. Pulmonary embolism
C. Aortic dissection (Correct Answer)
D. Papillary muscle rupture
E. Spontaneous pneumothorax
Explanation: ***Aortic dissection***
- The sudden onset of **severe, migrating anterior chest pain**, autonomic symptoms (sweating at rest), and **decreased bilateral femoral pulses** are highly characteristic of aortic dissection.
- The presence of a new **diastolic murmur** (indicating aortic insufficiency due to dissection extending to the aortic valve), along with a history of **hypertension** and **smoking**, further supports this diagnosis.
- **Decreased bilateral femoral pulses** suggest involvement of the descending aorta compromising flow to both lower extremities.
*Esophageal rupture*
- While esophageal rupture can cause **sudden, severe chest pain**, it typically presents with **odynophagia**, **vomiting**, and often **subcutaneous emphysema** or **Hamman's sign**, which are absent here.
- It would not explain the **diastolic murmur** or the **bilateral decreased femoral pulses**.
*Pulmonary embolism*
- **Shortness of breath** and **chest pain** can occur with pulmonary embolism, but the pain is typically **pleuritic** rather than migrating.
- It would not cause a **diastolic murmur** or **decreased peripheral pulses**.
*Papillary muscle rupture*
- Papillary muscle rupture is a complication of **myocardial infarction**, usually causing **acute severe mitral regurgitation** with a new **systolic murmur** and signs of **heart failure**.
- The patient's presentation with migrating pain and a **diastolic murmur** is inconsistent with this diagnosis.
*Spontaneous pneumothorax*
- A spontaneous pneumothorax causes **sudden, sharp, pleuritic chest pain** and **dyspnea**, often accompanied by **decreased breath sounds** on the affected side.
- It would not cause a **diastolic murmur** or **decreased femoral pulses**.
Question 5: A 10-month-old boy is brought to the clinic with a history of recurrent episodes of stridor and wheezing. His mother reports that his wheezing is exacerbated by crying, feeding, and flexion of the neck, and is relieved by extension of the neck. Occasionally he vomits after feeding. What is the most likely diagnosis?
A. Laryngomalacia
B. Congenital subglottic stenosis
C. Gastroesophageal reflux disease
D. Double aortic arch (Correct Answer)
E. Recurrent viral wheeze
Explanation: ***Double aortic arch***
- A **double aortic arch** forms a complete vascular ring around the trachea and esophagus, causing symptoms that worsen with feeding and neck flexion due to increased pressure on these structures.
- The characteristic relief with neck extension and symptoms like stridor, wheezing, and vomiting after feeding are classic signs of airway and esophageal compression by a **vascular ring**.
*Laryngomalacia*
- This condition involves the **collapse of supraglottic structures** during inspiration, typically causing inspiratory stridor, which is often louder when supine or agitated.
- While symptoms can worsen with feeding and crying, the hallmark relief with neck extension and vomiting after feeding due to esophageal compression are not typical features.
*Congenital subglottic stenosis*
- This involves a **narrowing of the airway** below the vocal cords, leading to inspiratory and expiratory stridor, and often biphasic stridor.
- The symptoms are usually persistent and are not characteristically relieved by neck extension or exacerbated by feeding and crying in such a distinct manner.
*Gastroesophageal reflux disease*
- While GERD can cause recurrent vomiting, cough, and even wheezing due to aspiration, it typically does not present with stridor.
- The clear correlation of worsening with neck flexion and relief with neck extension strongly points away from isolated GERD as the primary cause.
*Recurrent viral wheeze*
- This common condition in infants involves episodes of wheezing that are often triggered by **viral respiratory infections**.
- It usually lacks the specific exacerbating and relieving factors related to neck position and feeding, such as relief with neck extension and concurrent vomiting after feeding.
Question 6: A 43-year-old man comes to the physician because of increasing shortness of breath for 1 month. He has been using two pillows at night but frequently wakes up feeling as if he is choking. Five months ago, he underwent surgery for creation of an arteriovenous fistula in his left upper arm. He has hypertension and chronic kidney disease due to reflux nephropathy. He receives hemodialysis three times a week. His current medications are enalapril, vitamin D3, erythropoietin, sevelamer, and atorvastatin. His temperature is 37.1°C (98.8°F), respirations are 22/min, pulse is 103/min and bounding, and blood pressure is 106/58 mm Hg. Examination of the lower extremities shows bilateral pitting pedal edema. There is jugular venous distention. A prominent thrill is heard over the brachiocephalic arteriovenous fistula. There are crackles heard at both lung bases. Cardiac examination shows an S3 gallop. The abdomen is soft and nontender. Which of the following is the most likely cause of this patient's symptoms?
A. AV fistula aneurysm
B. Pulmonary embolism
C. Constrictive pericarditis
D. Dialysis disequilibrium syndrome
E. High-output heart failure (Correct Answer)
Explanation: ***High-output heart failure***
- The patient's symptoms of **dyspnea, orthopnea, pitting edema, jugular venous distention, crackles**, and **S3 gallop** strongly indicate **heart failure**. The **bounding pulse** and **wide pulse pressure** (systolic 106, diastolic 58) in the presence of an **arteriovenous fistula** suggest a **high-output state**.
- An **arteriovenous fistula** used for hemodialysis can significantly increase **cardiac preload** and reduce **afterload**, leading to a persistent increase in **cardiac output**. Over time, this chronic increase in demand can overwhelm the heart, resulting in **high-output heart failure**.
*AV fistula aneurysm*
- An **AV fistula aneurysm** is a localized dilatation of the fistula and would typically present as a painful or compressible mass.
- While it's a complication of AV fistulas, it does not directly explain the systemic signs of **heart failure** observed in this patient.
*Pulmonary embolism*
- **Pulmonary embolism** typically presents with sudden onset **dyspnea, pleuritic chest pain**, and sometimes **tachycardia** and **hypoxia**.
- This patient's symptoms are of gradual onset, accompanied by clear signs of **fluid overload** and **cardiac dysfunction** like an S3 gallop, which are not typical for a PE.
*Constrictive pericarditis*
- **Constrictive pericarditis** causes symptoms of **right-sided heart failure** due to impaired diastolic filling, often with a **pericardial knock** and **Kussmaul's sign**.
- While it can manifest with pedal edema and JVD, the **S3 gallop** and especially the **bounding pulse** and **wide pulse pressure** are inconsistent with constrictive pericarditis, which would typically cause a low-output state.
*Dialysis disequilibrium syndrome*
- **Dialysis disequilibrium syndrome** occurs shortly after hemodialysis, usually during or immediately after the first few sessions. It is characterized by neurological symptoms such as **headache, nausea, vomiting, confusion**, and **seizures**.
- The patient's symptoms have been evolving over a month and describe a state of **fluid overload** and **cardiac dysfunction**, not acute neurological symptoms related to dialysis.
Question 7: A 75-year-old Caucasian man presents to the emergency department with abdominal pain. The patient states he was at home eating dinner when he began to experience severe abdominal pain. The patient has a past medical history of diabetes, hypertension, and atherosclerosis. He lives at home alone, smokes cigarettes, and drinks 1 to 2 alcoholic drinks per day. The patient is given IV morphine and an ultrasound is obtained demonstrating a dilated abdominal aorta. The patient states that his father died of a similar finding and is concerned about his prognosis. Which of the following is the greatest risk factor for this patient's presentation?
A. Male gender and age
B. Caucasian race
C. Cigarette smoking (Correct Answer)
D. Family history
E. Atherosclerosis
Explanation: ***Cigarette smoking***
- **Cigarette smoking** is the most significant modifiable risk factor for the development and expansion of **abdominal aortic aneurysms (AAAs)**, directly contributing to vascular inflammation and degradation.
- The patient's history of smoking suggests a strong causal link to his current presentation of a dilated aorta, which is highly indicative of an AAA.
*Male gender and age*
- While **male gender** and **advanced age (over 65)** are significant demographic risk factors for AAA, they are considered non-modifiable and less impactful than smoking in terms of risk magnitude.
- These factors increase predisposition but do not exert the same direct, damaging effect on the arterial wall as chronic smoking.
*Caucasian race*
- **Caucasian race** is a known demographic risk factor for AAA, with higher prevalence rates compared to other ethnic groups.
- However, this is a non-modifiable genetic predisposition and contributes less to the overall risk than modifiable lifestyle factors like smoking.
*Family history*
- A **family history** of AAA, as suggested by the patient's father having a similar condition, increases an individual's susceptibility.
- This is a significant non-modifiable risk factor, indicating genetic predisposition, but its overall impact on aneurysm formation and progression is typically less than that of active smoking.
*Atherosclerosis*
- **Atherosclerosis** is a strong associated condition with AAA, as both share common risk factors and pathology related to arterial wall degeneration.
- While atherosclerosis contributes to the overall vascular compromise, smoking specifically has a more direct and potent effect on promoting aneurysm formation and rupture independently.
Question 8: In a routine medical examination, a young man is noted to be tall with slight scoliosis and pectus excavatum. He had been told that he was over the 95% percentile for height as a child. Auscultation reveals a heart murmur, and transthoracic echocardiography shows an enlarged aortic root and mitral valve prolapse. Blood screening for fibrillin-1 (FBN1) gene mutation is positive and plasma homocysteine is normal. This patient is at high risk for which of the following complications?
A. Intravascular thrombosis
B. Infertility
C. Aortic dissection (Correct Answer)
D. Spontaneous pneumothorax
E. Mucosal neuromas
Explanation: ***Aortic dissection***
- The constellation of **tall stature**, **scoliosis**, **pectus excavatum**, **enlarged aortic root**, **mitral valve prolapse**, and a **positive FBN1 gene mutation** is highly indicative of **Marfan syndrome**.
- **Aortic dissection** is the most life-threatening complication of Marfan syndrome due to the weakened connective tissue in the aortic wall, a direct consequence of the FBN1 mutation affecting fibrillin-1 protein.
- Patients with Marfan syndrome require regular cardiovascular surveillance and prophylactic beta-blocker therapy to reduce aortic wall stress.
*Intravascular thrombosis*
- This complication is more characteristic of conditions like **homocystinuria**, which is ruled out by the **normal plasma homocysteine** level.
- Marfan syndrome primarily affects connective tissue strength, not coagulation pathways or endothelial function.
*Infertility*
- Marfan syndrome does not inherently cause **infertility** in males or females.
- The primary health risks for Marfan patients are cardiovascular, musculoskeletal, and ophthalmologic (lens dislocation).
*Spontaneous pneumothorax*
- While **spontaneous pneumothorax** can occur in Marfan syndrome due to apical blebs and weakened pleural tissue, it is far less immediately life-threatening than aortic dissection.
- The question asks for the "high risk" complication, and cardiovascular complications (particularly aortic dissection and rupture) are the leading cause of mortality in Marfan syndrome.
*Mucosal neuromas*
- **Mucosal neuromas** are characteristic of **Multiple Endocrine Neoplasia type 2B (MEN2B)**, not Marfan syndrome.
- MEN2B involves RET proto-oncogene mutations and is associated with medullary thyroid cancer, pheochromocytoma, and a marfanoid habitus, which can be mistaken for Marfan syndrome.
Question 9: A 67-year-old man presents to the emergency department with abdominal pain that started 1 hour ago. The patient has a past medical history of diabetes and hypertension as well as a 40 pack-year smoking history. His blood pressure is 107/58 mmHg, pulse is 130/min, respirations are 23/min, and oxygen saturation is 98% on room air. An abdominal ultrasound demonstrates focal dilation of the aorta with peri-aortic fluid. Which of the following is the best next step in management?
A. Serial annual abdominal ultrasounds
B. Emergent surgical intervention (Correct Answer)
C. Administer labetalol
D. Counsel the patient in smoking cessation
E. Urgent surgery within the next day
Explanation: ***Emergent surgical intervention***
- The patient's presentation with acute **abdominal pain**, **hypotension**, and **tachycardia** combined with ultrasound findings of focal aortic dilation and peri-aortic fluid strongly suggests a **ruptured abdominal aortic aneurysm (AAA)**.
- A ruptured AAA is a life-threatening emergency requiring immediate surgical repair to prevent further hemorrhage and death.
*Serial annual abdominal ultrasounds*
- This approach is appropriate for asymptomatic patients with smaller, stable AAAs (typically <5.5 cm) to monitor for growth.
- In this case, the patient is symptomatic with signs of rupture, making surveillance an inappropriate and dangerous management strategy.
*Administer labetalol*
- Medications like labetalol are used to control blood pressure in conditions like aortic dissection or to slow the progression of AAAs, but they are contraindicated in hypotensive patients with a ruptured AAA.
- In this patient, labetalol would worsen the existing hypotension and could lead to cardiovascular collapse.
*Counsel the patient in smoking cessation*
- Smoking cessation is a crucial long-term intervention to reduce the risk of AAA expansion and rupture.
- While important, it does not address the immediate, life-threatening emergency of a ruptured AAA.
*Urgent surgery within the next day*
- Waiting until the next day for surgery in a patient with a suspected ruptured AAA is unacceptable.
- The patient's hemodynamic instability (hypotension, tachycardia) indicates active bleeding, and any delay significantly increases morbidity and mortality.
Question 10: A 31 year-old-man presents to an urgent care clinic with symptoms of lower abdominal pain, bloating, bloody diarrhea, and fullness, all of which have become more frequent over the last 3 months. Rectal examination reveals a small amount of bright red blood. His vital signs include: temperature 36.7°C (98.0°F), blood pressure 126/74 mm Hg, heart rate 74/min, and respiratory rate 14/min. Colonoscopy is performed, showing extensive mucosal erythema, induration, and pseudopolyps extending from the rectum to the splenic flexure. Given the following options, what is the most appropriate treatment to induce remission in this patient?
A. Azathioprine
B. Mesalamine
C. Total proctocolectomy
D. Sulfasalazine
E. Systemic corticosteroids (Correct Answer)
Explanation: ***Systemic corticosteroids***
- The patient presents with classic symptoms of an acute **ulcerative colitis flare**, including bloody diarrhea, abdominal pain, and colonoscopy findings of extensive inflammation from the rectum to the splenic flexure (consistent with **left-sided colitis**).
- **Systemic corticosteroids** such as prednisone or methylprednisolone are the **most appropriate treatment to induce remission** during active flares of moderate to severe ulcerative colitis due to their potent **anti-inflammatory effects** and rapid onset of action.
- This patient has moderate to severe disease based on extent and symptom severity, warranting systemic corticosteroids rather than topical or aminosalicylate therapy alone.
*Azathioprine*
- **Azathioprine** is an **immunomodulator** used for maintaining remission in inflammatory bowel disease, not for acute flare treatment.
- Its onset of action is slow (weeks to months), making it unsuitable for immediate symptom control in an acute flare.
*Mesalamine*
- **Mesalamine** (an aminosalicylate) is a **first-line therapy** for inducing and maintaining remission in **mild to moderate** ulcerative colitis, particularly for proctitis or left-sided colitis.
- However, for extensive disease with significant symptoms as seen in this patient, **systemic corticosteroids** are preferred due to greater potency and more rapid induction of remission in moderate to severe flares.
*Total proctocolectomy*
- **Total proctocolectomy** is a surgical procedure that provides a **definitive cure** for ulcerative colitis by removing the entire colon and rectum.
- However, surgery is reserved for cases of **refractory disease** (failure of medical therapy), severe complications (e.g., toxic megacolon, perforation, severe hemorrhage), or high risk of dysplasia/cancer.
- This patient is presenting with an acute flare and should be managed medically first; surgery is not the initial treatment approach.
*Sulfasalazine*
- **Sulfasalazine** is an aminosalicylate similar to mesalamine, used for inducing and maintaining remission in mild to moderate ulcerative colitis.
- While effective for mild disease, systemic corticosteroids are preferred for moderate to severe acute flares due to their stronger and more rapid anti-inflammatory action when the disease is extensive and symptomatic.
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