Cases from Baystate Medical Center


By Jin Kim, MD; Jorge A. Velez Garza, MD, ACP Resident/Fellow Member; Anita Mulye, MD; Anna Stepczynski, MD, ACP Resident/Fellow Member; Tara Lagu, MD; Thomas Chin, MBBS; Jaime Hernandez, MD, MPH, FACP; Abid Shah, MD; Alexandra Marchetta, MD; Christine Bryson, DO; and Gina Luciano, MD, FACP

Physician editor: Christopher Sankey, MD, FACP

Deputy physician editor: Benjamin Galen, MD, ACP Member

Case 1: Stones and the missing gallbladder

By Jin Kim, MD, and Jorge A. Velez Garza, MD, ACP Resident/Fellow Member

The patient

An 82-year-old woman with no history of abdominal surgery presented with acute right upper quadrant pain typical of biliary colic. Associated symptoms included anorexia and nausea without diarrhea, fever, or chills. Vital signs were normal with an exam significant for right-sided abdominal tenderness without peritoneal signs or appreciable masses. Pertinent laboratory results included a lipase level of 2,492 U/L, an aspartate aminotransferase (AST) level of 177 U/L, an alanine aminotransferase (ALT) level of 180 U/L, and a white blood cell count of 25,500 cells/mm3. Abdominal ultrasound demonstrated mild dilatation of the common bile duct, possibly due to postcholecystectomy state. CT of the abdomen/pelvis was significant for common bile duct dilatation with multiple gallstones and findings consistent with acute pancreatitis. Also noted was a congenitally absent gallbladder.

The patient's symptoms markedly improved with IV fluids and pain management; she was able to tolerate an oral diet and was discharged. Subsequent outpatient endoscopic retrograde cholangiopancreatography (ERCP) was performed, and multiple stones were removed; some stones appeared to be consistent with primary bile duct stones, while others appeared more obsidian-like,” resembling stones that would be typically found in the gallbladder.

The diagnosis

This patient developed gallstone pancreatitis in the setting of gallbladder agenesis (GA), which is a rare congenital abnormality with a reported incidence of 0.01% to 0.09%. Most patients with GA are asymptomatic, and the diagnosis is made incidentally. Symptomatic patients can present with biliary colic (54%), dyspepsia (34%), and/or jaundice (27%). In those with biliary colic, sphincter of Oddi dysfunction may be the underlying mechanism for these symptoms; in other cases, common bile duct stones may be the cause. Diagnostic imaging, including ultrasonography, cholangiography, hepatobiliary scintigraphy, and ERCP, can be misleading in GA, which can be mistaken for cystic duct obstruction or a small/contracted gallbladder. Magnetic resonance cholangiopancreatography (MRCP) can most accurately exclude an ectopic or absent gallbladder, as it is not compromised by biliary stasis.

Pearls

  • Gallbladder agenesis is typically an incidental finding but can present with symptoms typical of biliary colic or even gallstone pancreatitis.
  • Gallbladder agenesis should be suspected when the gallbladder is not well visualized on routine imaging, and MRCP is the imaging modality of choice when gallbladder agenesis is suspected.

Case 2: Chlorpromazine-induced hepatitis

By Anita Mulye, MD; Anna Stepczynski, MD, ACP Resident/Fellow Member; and Tara Lagu, MD

The patient

A 34-year-old woman with bipolar disorder and attention deficit hyperactivity disorder presented with jaundice and acute severe right upper quadrant pain in the setting of subacute intractable nausea and vomiting. She reported no fever, diarrhea, alcohol use, sick contacts, or travel. Reported medications included lithium, methylphenidate, and ondansetron. On physical examination, she was afebrile with normal vital signs. She had significant jaundice with scleral icterus and tenderness of the right upper quadrant without rebound or guarding. Laboratory results revealed an ALT level of 1,000 U/L (normal range, 5 to 35 U/L), an AST level of 474 U/L (normal range, 0 to 40 U/L), direct hyperbilirubinemia of 8.2 mg/dL (normal range, 0.0 to 0.3 mg/dL), and a mildly elevated alkaline phosphatase level. Albumin level, international normalized ratio, lipase level, and complete blood count were normal. Abdominal imaging was unrevealing, and ERCP revealed no obstruction. Viral and autoimmune hepatitis, primary biliary cirrhosis, and Wilson's disease were excluded.

On the third day of hospitalization, the patient developed a diffuse, pruritic, non-blanching, erythematous rash in conjunction with peripheral eosinophilia. The patient's hyperbilirubinemia and transaminitis persisted. Liver biopsy revealed cholestasis with marked eosinophilia, inflammation, and bile duct edema, all of which were consistent with drug-induced hepatitis. A review of the patient's medications revealed recent use of chlorpromazine that had not been documented on admission. The cutaneous symptoms improved with triamcinolone cream within 24 hours of treatment. This patient's abdominal pain dissipated, and hepatic function improved to near-normal levels approximately 42 days after presentation with withdrawal of chlorpromazine.

The diagnosis

This patient was diagnosed with drug-induced hepatitis secondary to chlorpromazine. Drug-induced hepatitis can occur as frequently as 1 in every 1,000 patients and is more common in women. The clinical manifestations of most drug-induced hepatitis resemble viral hepatitis with malaise, jaundice, and transaminitis. However, different pharmacological entities have distinct patterns of injury (hepatocellular, cholestatic, autoimmune, fibrosis) and may or may not be associated with classic allergic symptoms, including fever and rash.

Symptoms typically develop within 1 to 6 weeks of initial ingestion of the medication and may continue evolving even after the agent is withdrawn. Chlorpromazine-induced hepatitis occurs in 0.2% to 2% of patients, with 80% to 90% of cases developing within the first 4 weeks. The mechanism by which chlorpromazine decreases canalicular function and bile flow is thought to be poor genetic sulfoxidation of free radicals and hydrocarbons, which makes affected patients more susceptible to cholestasis. Histology shows cholestasis and a predominance of mononuclear and eosinophilic cells, although hepatocellular injury and granuloma formation are possible as well.

Pearls

  • Symptoms of drug-induced hepatitis can develop within 1 to 6 weeks of initial ingestion of the agent and may continue to evolve after it is withdrawn.
  • Chlorpromazine-induced hepatitis is typically associated with cholestasis but can also induce a mild allergic reaction presenting as a diffuse pruritic rash and eosinophilia.

Case 3: STEMI mimic on EKG: The Brugada pattern

By Thomas Chin, MBBS; Anna Stepczynski, MD, ACP Resident/Fellow Member; and Jaime Hernandez, MD, FACP

The patient

A 33-year-old man with a history of IV heroin abuse was found unresponsive by his relatives with a syringe in his arm. First responders noted pinpoint pupils and depressed respiratory rate; naloxone was administered. In the ED, he was tachycardic with otherwise normal vital signs. The patient was somnolent and responsive only to painful stimuli, with an unremarkable cardiovascular exam. Serum troponin, creatinine, and creatine kinase levels were elevated, and toxicology was positive for cannabinoids and opiates. Electrocardiogram (EKG) revealed sinus tachycardia with J-point elevation, down-sloping ST elevation, and T-wave inversion in leads V1 and V2 (see Image 1).

With improvement in mental status, the patient indicated no cardiorespiratory symptoms. He reported no previous syncopal episodes or family history of sudden cardiac death. He reported no ingestion of other medications, including tricyclic antidepressants or antipsychotics. Cardiac catheterization was deferred, and the EKG findings subsequently resolved to an incomplete right bundle-branch pattern without ST-segment elevation. Echocardiogram showed no structural abnormalities, and the patient had no significant arrhythmias noted during 3 days of cardiac monitoring.

The diagnosis

While there was initial concern for the possibility of acute ST-elevation myocardial infarction (STEMI), this patient's EKG abnormalities were consistent with a Brugada pattern. The elevated troponin level was attributed to the patient's rhabdomyolysis and not coronary ischemia. Recognition of the Brugada pattern on EKG is important because a proportion of patients with this EKG have Brugada syndrome, which is associated with ventricular arrhythmias and sudden cardiac death. EKG findings typical of the Brugada pattern consist of a pseudo-right bundle-branch block and persistent ST elevation in leads V1 to V3.

The Brugada EKG pattern can be transiently induced in patients with an apparently normal baseline EKG. Examples of triggers include fever, exposure to certain drugs (including cocaine and alcohol), or electrolyte imbalances. Case reports suggest cannabis as a possible cause of Brugada pattern on EKG as well. Heroin (along with other opiates) is not listed as a known cause of the Brugada EKG pattern, but it is possible that contaminants in the injected material could play a role. Approximately 10% of patients with the Brugada pattern have Brugada syndrome. Guidelines recommend that Brugada syndrome should be suspected in patients with the Brugada pattern EKG and at least 1 of the following: documented ventricular arrhythmia; family history of the Brugada pattern or sudden cardiac death; inducible ventricular tachycardia during electrophysiology study; unexplained syncope suggestive of a tachyarrhythmia; or nocturnal agonal respiration. These patients should be considered for placement of an implantable cardioverter defibrillator.

Pearls

  • The Brugada pattern consists of a pseudo-right bundle-branch block and persistent ST elevation in leads V1 to V3 and can mimic coronary ischemia.
  • It is important to recognize this pattern, as the Brugada syndrome can be associated with life-threatening arrhythmias and sudden death.

Case 4: Persistent breast erythema

By Abid Shah, MD, and Jorge A. Velez Garza, MD, ACP Resident/Fellow Member

The patient

A 62-year-old woman presented to her primary care physician with 2 weeks of increasing pain and erythema of her left breast. She reported no palpable masses, fever, nipple discharge, nipple inversion, or weight loss. Screening mammography 1 week prior to symptom development was normal. Her breast erythema progressed despite antibiotic therapy, and she was referred to the ED for further assessment. Vital signs were stable, and examination revealed a warm, erythematous left breast with thickening of the skin (see Image 2). There were no palpable masses, lymphadenopathy, or nipple changes. Complete blood count was unremarkable, and blood cultures were negative. An ultrasound of the left breast revealed diffuse thickening of the skin in the inferomedial compartment suggestive of cellulitis. However, despite IV antibiotics, there was no clinical improvement. A left breast punch biopsy revealed intra-lymphatic tumor emboli consistent with inflammatory breast cancer.

The diagnosis

Inflammatory breast cancer (IBC) is an uncommon type of invasive breast cancer and is associated with a poor prognosis and a high risk of early recurrence. Typically, IBC presents with rapidly progressive erythema, tenderness, induration, and enlargement of the affected breast, as well as skin color changes and thickening of the skin. Other suspicious physical findings include axillary lymphadenopathy and nipple involvement. The International Expert Panel on Inflammatory Breast Cancer has published the following diagnostic criteria:

  1. 1. rapid onset of breast erythema, edema, and/or peau d’orange, with or without an underlying palpable mass;
  2. 2. duration of no more than 6 months;
  3. 3. erythema occupying at least one-third of the breast; and
  4. 4. pathologic confirmation of invasive carcinoma.

Accurate diagnostic tests for IBC are limited, and a high index of suspicion is important to prevent delay in diagnosis and treatment. Mammography is the recommended initial diagnostic test and can reveal any of the following: tumor mass, calcification, parenchymal distortion, or breast thickening without a dominant mass. A full-thickness skin biopsy should be performed in patients in whom IBC is suspected despite negative imaging.

Pearls

  • Inflammatory breast cancer may mimic cellulitis and/or present subtly without masses or lymphadenopathy.
  • In the absence of positive findings on breast imaging, a full-thickness biopsy can be diagnostic.

Case 5: Code status in the perioperative period

By Alexandra Marchetta, MD; Christine Bryson, DO; and Gina Luciano, MD, FACP

The patient

A 78-year-old male with end-stage chronic obstructive pulmonary disease on home oxygen was admitted to the ICU after developing respiratory failure as a complication of a lung nodule biopsy. His code status was do not resuscitate/do not intubate (DNR/DNI) prior to the biopsy, but this had been reversed as per standard protocol for the procedure. A formal discussion with the patient concerning periprocedural code status was not documented. After the biopsy, the patient developed intrapulmonary hemorrhage and pneumothorax requiring intubation and chest tube placement, with his family's consent. He improved and was extubated on his second day of hospitalization. Later that day, the patient again developed respiratory failure and was re-intubated. The DNR patient consent form states that if the DNR order has been suspended during the perioperative period, discussions with the patient for reinstituting the DNR order must take place within the first 48 hours postoperatively. Only a few hours occurred between intubations, and the patient was not clinically well enough to participate in a meaningful code discussion, thus leading the team to continue with the full code order.

The next day, a family meeting was held during which many of the patient's family members expressed concern that the patient was re-intubated despite his previously documented DNR/DNI order. The family and patient ultimately decided to attempt extubation, with the understanding that he not be re-intubated. On day 4, the patient passed the weaning parameters and was extubated; he died soon after.

The diagnosis

Patient autonomy can be compromised when a previously documented DNR/DNI code status is routinely reversed for a procedure. A preoperative or preprocedural discussion regarding code status and patient wishes in the setting of a procedural complication can mitigate this to a certain extent. This conversation ideally includes information about how anesthesia may be similar to resuscitation and how resuscitation is more likely to be successful in the OR. If the patient decides to suspend a DNR/DNI order for a procedure, a reinstatement plan can be discussed and agreed upon pre-procedurally to maximize patient autonomy in the event of a complication.

In 1993, the American Society of Anesthesiologists recommended that the automatic reversal of DNR status for perioperative patients conflicted with a patient's right to self-determination. Despite these guidelines and the emphasis currently placed on respecting patient autonomy, many hospitals and institutions do not have a policy regarding DNR/DNI status in the perioperative period. Many institutions that do have a policy require automatic perioperative suspension of DNR/DNI orders. For all hospitalists and care team members of patients with clearly stated DNR/DNI orders, pre-procedure discussions should be held and documented to ascertain patient preferences in the event of a complication in order to maximize patient autonomy.

Pearls

  • Many hospitals and institutions either have no policy requiring the clarification of periprocedural DNR/DNI orders or have a policy that requires periprocedural suspension of DNR/DNI orders.
  • Discussing goals of care and contingency plans for potential procedural complications, and development of a specific DNR/DNI re-implementation plan periprocedurally can maximize patient autonomy and ensure that patients' wishes are respected.
  • Documentation of periprocedural code discussions is important to include in the medical record.