Gurvits Syndrome - Symptoms, Diagnosis, and Treatment

What Is Gurvits Syndrome?

Gurvits syndrome is a rare acute condition that leads to esophageal necrosis, also known as “black esophagus” or “necrotizing esophagitis.” This condition is characterized by a circumferential black appearance of the esophageal mucosa or a striking diffuse patchy appearance that most commonly occurs in the distal esophagus and terminates the gastroesophageal junction. It commonly occurs in males at a ratio of 4:1 and has a high mortality rate. This condition also occurs in a setting of diabetic ketoacidosis.

What Is the History of Gurvits Syndrome?

Gurvits syndrome was initially described by Goldenberg and colleagues in 1990. Although cases have been identified since the 1960s, they were not labeled with the common names for this syndrome. Due to its rarity, only 88 cases have been formally recognized as Gurvits syndrome, though the actual number is likely higher. Gurvits and colleagues noted that this syndrome is "poorly described in the medical literature." Abdullah and colleagues conducted the first comprehensive review of acute esophageal necrosis, analyzing approximately 154 patients reported in the literature at the time.

What Are the Causes of Gurvits Syndrome?

Gurvits syndrome is potentially rare but is the most underdiagnosed condition. This condition arises due to an ischemic insult to the esophagus, an impaired mucosal barrier system, and backflow injury due to the chemical contents of gastric fluid and secretions. Cases are reported more in males than females. The most common symptom present is upper gastrointestinal bleeding with hematemesis and hemodynamic instability, which typically occurs in aged people. Those conditions occur due to broad-spectrum antibiotic usage, para esophageal hernia, hyperglycemia, diabetic ketoacidosis, gastric volvulus, any underlying malignancy, and Steven Johnson syndrome.

What Are the Signs and Symptoms of Gurvits Syndrome?

Gurvits syndrome presents with multiple symptoms. The symptoms vary according to the severity of the disorder. The most classic sign is the dark pigmentation of the esophageal mucosa that is detected by upper endoscopy. It is viewed as an ulcer or an infectious disease. Necrosis is found between the three distal parts of the esophagus but stops at the gastroesophageal junction. Other common symptoms include:

Blood in vomit.

Blood in stools.

Upper gastrointestinal bleeding.

Tarry black or bloody stools.

Abdominal pain.

Nausea.

Unstable vital signs.

Low-grade pyrexia.

Hypotension.

Tachycardia.

Hypoxia.

Hemodynamic instability.

Cardiogenic shock.

Even a cardiovascular event is reported in 10 percent of cases.

What Are the Risk Factors for Gurvits Syndrome?

The risk factors of Gurvits syndrome include:

Ischemic heart disease.

Hypertension.

Type 2 diabetes.

Alcoholism.

Solid-organ malignancy.

Chronic liver disease.

Chronic kidney disease.

Chronic obstructive pulmonary disease.

Peripheral vascular disease.

Poor nutritional state.

How Is the Diagnosis Made for Gurvits Syndrome?

Diagnosis of Gurvits syndrome is made by:

Blood Test: Tests are done to evaluate a complete blood count, likely to reveal raised white blood cells, lactic acidosis, and anemia.

Gastroscopy: This is performed in cases of gastrointestinal bleeding. In most cases, the necrosis results in the distal third of the esophagus. It can extend to the whole esophagus by 36 percent. Other pathologies include gastric outlet, blood clots, gastric ulceration, active bleeding, and hiatus hernia.

CT Scan: A CT scan of the chest shows esophageal perforation performed in cases of diagnostic concerns.

Tissue Biopsy: This procedure is not often recommended for Gurvita syndrome, but this test can help exclude other causes of the black esophagus.

What Is the Treatment for Gurvits Syndrome?

The treatment for esophagus necrosis depends on treating the underlying cause. Treatment for esophagus necrosis involves fluid resuscitation along with blood transfusion if there is significant anemia.

Patients are recommended not to take anything by mouth, and intravenous proton pump inhibitors are given to reduce local trauma to the vulnerable esophagus.

Sucralfate is used to reduce insult to the esophagus. A nasogastric tube should not be inserted as there is an underlying risk of esophagus perforation.

Antibiotics are given only if there is evidence of perforation or sepsis, or else they are not recommended.

Adrenaline injection during a gastroscopy or by metallic stent insertion is done to control localized bleeding.

In cases of perforation of the esophagus, surgery is the primary treatment. This involves emergency esophagectomy followed by elective esophageal reconstruction.

Stent insertion is effective but requires subsequent intervention to correct stent migration. A combination of VATS (video-assisted thoracoscopic surgery) and drain placement has been reported as successful.

What Is the Differential Diagnosis of Gurvits Syndrome?

The differential diagnosis of Gurvits Syndrome are:

Black Dye Ingestion: It is a condition caused by the ingestion of black dye.

Direct Caustic Injury: Condition caused by the ingestion of causative or corrosive agents that cause direct tissue injury.

Pseudomelanosis: A rare incidental finding during the endoscopic procedure by the darkly pigmented esophagus.

Malignant Melanoma: A serious skin cancer, melanoma occurs when pigment-producing cells cause the color of the skin and become cancerous.

Acanthosis Nigricans: A skin condition characterized by velvety, dark patches in body creases and folds.

Coal Dust Deposition: A condition that occurs due to coal dust deposition and injury to the tissue.

What Is the Prognosis of Gurvits Syndrome?

The prognosis for Gurvits syndrome is generally poor, with a high mortality risk of up to 32 percent. Most deaths are due to underlying conditions, while the mortality rate directly attributable to this syndrome is approximately 6 percent.

What Are the Complications Associated With Gurvits Syndrome?

The complications associated with Gurvits syndrome include:

• Esophageal perforation (a hole in the esophageal tube that carries food from the mouth to the stomach that can be caused by any trauma).

• Localized bleeding.

• Upper gastrointestinal bleeding (upper gastrointestinal bleeding is the most serious sign and complication of many underlying diseased conditions).

• Esophageal strictures (abnormal tightening of the esophagus; in rare cases, this condition may rip the lining of the esophagus, which can be a life-threatening condition).

• Trachea-esophageal fistulas (abnormal development of the tube that carries food from the mouth to the stomach before birth).

Complications do not develop until at least two weeks after the initial onset of Gurvits syndrome. Management of stricture is done by iterative balloon dilation; it is an effective treatment. This increases the recovery rate in individuals without complications. However, follow-up endoscopy is recommended for approximately one month to rule out other sequelae.

Conclusion:

Gurvits syndrome, or black esophagus, is a typical endoscopic manifestation of acute esophageal necrosis. Males are more affected than females, usually aged 60 years or above. Currently, there is no specific treatment for esophageal necrosis. The goal of treatment and therapy should be directed toward treating the underlying cause and medical condition. Though the prognosis is poor, correcting anemia, intravenous hydration, and red blood cell transfusion are considered in its initial management.