Hepatic venoocclusive disease (sinusoidal obstruction syndrome) is characterized by which of the following?
Q1352
Which of the following statements about Boerhaave's syndrome is true?
Q1353
Motility in the upper third of the esophagus is decreased in all of the following conditions except which one?
Q1354
A 50-year-old man presents with chronic abdominal pain and weight loss. A barium study shows a "string sign" in the terminal ileum. What is the most likely diagnosis?
Gastroenterology Indian Medical PG Practice Questions and MCQs
Question 1351: Hepatic venoocclusive disease (sinusoidal obstruction syndrome) is characterized by which of the following?
A. Central venous congestion
B. Hepatomegaly (Correct Answer)
C. Budd chiari syndrome
D. Portal vein obstruction
Explanation: ***Hepatomegaly***
- **Hepatic veno-occlusive disease (HVOD)**, also known as **sinusoidal obstruction syndrome (SOS)**, involves obstruction of the **hepatic sinusoids** and small hepatic venules [2].
- This obstruction leads to **congestion** within the liver, causing the liver to swell and become enlarged, which is termed **hepatomegaly**.
*Central venous congestion*
- While there is **congestion within the liver**, the primary pathology in HVOD is at the level of the **sinusoids and small hepatic venules**, not necessarily directly involving **central venous congestion** originating from the heart [2].
- Central venous congestion is more characteristic of conditions like **right-sided heart failure** [2].
*Portal vein obstruction*
- **Portal vein obstruction** involves blockage of the main **portal vein**, which supplies blood to the liver, leading to **portal hypertension** and symptoms like varices and ascites [1], [3].
- In contrast, HVOD/SOS is characterized by obstruction of the **intrahepatic sinusoids and venules**, primarily impeding blood outflow from the liver [2].
*Budd-Chiari syndrome*
- **Budd-Chiari syndrome** involves obstruction of the **hepatic veins** (larger veins draining the liver) or the **inferior vena cava**.
- Although it also causes liver congestion and often hepatomegaly, the **level of obstruction is different** from HVOD/SOS, which affects the smaller **intrasinusoidal venules** [2].
Question 1352: Which of the following statements about Boerhaave's syndrome is true?
A. Not typically iatrogenic
B. Presented with acute chest pain (Correct Answer)
C. Usually asymptomatic
D. Requires surgical intervention for large perforations
Explanation: ***Presented with acute chest pain***
- **Boerhaave's syndrome**, a spontaneous esophageal rupture, classically presents with sudden, severe **retrosternal chest pain** that can radiate to the back or left shoulder, often accompanied by **vomiting** or retching.
- The pain is typically excruciating and can be mistaken for a myocardial infarction or aortic dissection.
*Not typically iatrogenic*
- Boerhaave's syndrome is defined as **spontaneous esophageal rupture**, distinguishing it from iatrogenic perforations (e.g., during endoscopy).
- While it occurs spontaneously, it is usually precipitated by events causing a sudden increase in intra-abdominal pressure, such as forceful vomiting or straining.
*Usually asymptomatic*
- Boerhaave's syndrome is a **medical emergency** characterized by severe symptoms, making it highly unlikely to be asymptomatic.
- The extravasation of gastric contents into the mediastinum and pleural spaces causes immense pain and rapid deterioration, leading to sepsis if not treated promptly.
*Requires surgical intervention for large perforations*
- While large perforations often necessitate **surgical repair**, the statement is not definitively "true" as the primary characteristic of the syndrome.
- The management of Boerhaave's syndrome depends on the size of the perforation, time since rupture, and patient stability, ranging from conservative management for small, contained ruptures to surgical repair for larger tears.
Question 1353: Motility in the upper third of the esophagus is decreased in all of the following conditions except which one?
A. Pseudo-bulbar palsy
B. Chagas disease
C. Myasthenia gravis
D. Scleroderma (Correct Answer)
Explanation: ***Scleroderma***
- **Scleroderma** primarily affects the **smooth muscle** of the distal two-thirds of the esophagus, leading to decreased motility and reflux [1].
- The **upper third of the esophagus** consists of **striated muscle** and is typically spared in scleroderma [1].
*Pseudo-bulbar palsy*
- **Pseudo-bulbar palsy** involves motor neuron damage affecting the **corticobulbar tracts**, leading to impaired function of the **striated muscles** of the pharynx and upper esophagus.
- This results in **dysphagia** and reduced motility in the voluntary phase of swallowing, which includes the upper third of the esophagus.
*Chagas disease*
- **Chagas disease** causes destruction of **ganglion cells** in the myenteric plexus, leading to denervation of the smooth muscle.
- While it primarily affects the **distal esophagus** (leading to megaesophagus), it can also impact the coordinated activity of the **gastrointestinal tract**, including the more complex coordination required for the upper esophagus, especially if there's extensive involvement.
*Myasthenia gravis*
- **Myasthenia gravis** is an **autoimmune disorder** characterized by antibodies against **acetylcholine receptors** at the neuromuscular junction, affecting **striated muscles** [1], [2].
- Since the **upper third of the esophagus** is composed of striated muscle, its function is often impaired in myasthenia gravis, leading to **dysphagia** [1].
Question 1354: A 50-year-old man presents with chronic abdominal pain and weight loss. A barium study shows a "string sign" in the terminal ileum. What is the most likely diagnosis?
A. Crohn's disease (Correct Answer)
B. Diverticulitis
C. Irritable bowel syndrome
D. Ulcerative colitis
Explanation: ***Crohn's disease***
- The **"string sign"** observed in the barium study indicates narrowed, inflamed terminal ileum typical of Crohn's disease [1].
- Presents with **chronic abdominal pain** and weight loss, aligning with the patient's symptoms [1].
*Diverticulitis*
- Characterized by **inflammation of diverticula**, usually presenting with **left lower quadrant pain** and fever, not a "string sign".
- Barium studies typically reveal **diverticula**, not the strictures seen in Crohn's.
*Irritable bowel syndrome*
- IBS presents with **crampy abdominal pain** and changes in bowel habits, but not with severe **weight loss** or strictures.
- There are no specific findings like the **"string sign"** in imaging studies for IBS.
*Ulcerative colitis*
- Primarily affects the **colon** with continuous lesions and presents with bloody diarrhea, not with a "string sign".
- Does not typically cause **narrowing** of the intestines like seen in Crohn's disease [2].
