Cases from the University of California, San Francisco, and Hôpital St. Thérèse, Haiti


Case 1: Dyspnea in a young woman in Haiti

Varun Verma, MD; Robin Tittle, MD, ACP Resident/Fellow Member; and Jacqueline Auguste, MD

The patient

A 28-year-old woman with no known medical history presented to an outpatient clinic in rural Haiti with severe difficulty breathing. The patient reported that 9 days before presentation she began experiencing a sensation of “suffocation” at rest, particularly when she was lying down. She also noticed swelling in her lower extremities and experienced pain in her abdomen. Upon presentation to the clinic, she was afebrile, her blood pressure was 100/90 mm Hg, her heart rate was 110 beats per minute, and her respiratory rate was 28 breaths per minute. Pulse oximetry was not available, and chest radiography was available only for a fee that her family could not afford.

The patient was sent to the internal medicine ward at the local hospital for admission. Upon further questioning, the patient said that she had undergone an uncomplicated vaginal delivery at the same hospital 3 weeks before the current presentation. She had no complications during her previous 4 deliveries and had not experienced any similar symptoms in the past. The hospital did not have a functional EKG at the time of admission; pulse oximetry on the wards revealed a 94% oxygen saturation on room air. The patient had a notably swollen face, jugular venous distention with hepatojugular reflux, no evident murmurs, crackles in both lung bases, 3+ pitting edema of her lower extremities, and skin that was cool to the touch. Bedside ultrasonography performed by the hospitalist revealed no pericardial effusion and normal valvular function but a dilated left ventricle, severely depressed ejection fraction, and global hypokinesis.

Diuresis was initiated with IV furosemide, oxygen supplementation was provided via nasal cannula, and a urine catheter was placed to monitor output. The patient improved during her stay. By the fifth hospital day she was no longer tachycardic or tachypneic, her lower-extremity edema had resolved, and her oxygen saturation improved to 99% on room air. She was discharged home on oral furosemide and enalapril, and a follow-up appointment was arranged for 1 month with a plan for repeat echocardiography.

The diagnosis

Frequent challenges to effective global health care delivery are limited diagnostics, an incomplete formulary of available medications, and lack of trained specialty personnel to call for assistance. Given the patient's history of presentation shortly after delivery, her echocardiography findings, and her excellent response to diuretic treatment, she was diagnosed with peripartum cardiomyopathy. Peripartum cardiomyopathy remains a poorly understood condition and a diagnosis of exclusion. The widely accepted definition stipulates that symptoms occur during the final month of pregnancy or within 5 months after delivery. In the United States, the incidence is between 1 in 2,000 to 1 in 4,000; however, in Haiti, it is 1 in 300.

The diagnostics available in rural Haiti were suboptimal during the management of this patient (no available EKG or chest X-ray), and labs routinely ordered in the United States (such as brain natriuretic peptide and troponin) were unavailable. However, due to their proficiency with ultrasound and knowledge of the prevalence of peripartum cardiomyopathy in Haiti, the hospitalists were nevertheless able to make a correct diagnosis and effectively manage the patient's heart failure.

Pearls

  • For general practitioners to ensure the best possible care for patients in settings with limited resources, they must become proficient in performing diagnostics such as bedside ultrasound.
  • Peripartum cardiomyopathy is much more common in Haiti than in the United States, with a frequency of approximately 1 in 300.

Case 2: Complicated heart failure in a resource-poor setting

Robin Tittle, MD, ACP Resident/Fellow Member; Varun Verma, MD; and Pierre Auguste MD

The patient

A 70-year-old woman with no known medical history presented to a district hospital in Hinche, in Haiti's Central Plateau, with symptoms of dyspnea and lower-extremity edema. Her family reported that the symptoms had been present for 8 days. On admission, she had a blood pressure of 80/40 mm Hg, a heart rate of 110 beats per minute, and oxygen saturation of 93% on 4 L of supplemental oxygen. Her physical examination revealed bibasilar crackles and 3+ pitting edema of her lower extremities extending to the thighs. IV furosemide and dopamine were started for presumed cardiogenic shock secondary to previously undiagnosed heart failure.

On the third hospital day, a bedside ultrasound was performed and revealed severely reduced global systolic function and a large mobile mass within the left ventricle (see Figure 1). The images were reviewed remotely by a cardiologist, and suspicion of intra-ventricular thrombus was confirmed. Therapeutic doses of subcutaneous heparin were begun, and the patient was transitioned to warfarin for ongoing anticoagulation. Repeat ultrasound 6 weeks after presentation demonstrated no residual clot. http://www.acphospitalist.org/archives/2014/05/the-brief-case_f1.pdf

The diagnosis

Decompensated heart failure is a common inpatient diagnosis in all parts of the world, including Haiti. Complications such as intra-ventricular thrombus probably occur more frequently but are rarely diagnosed due to limited diagnostic capabilities. Left ventricular thrombus can occur as a complication of systolic heart failure or following myocardial infarction. Echocardiography is the diagnostic tool of choice, with an estimated sensitivity of 85% to 90% and a specificity of 95%. Patients with evidence of a mobile thrombus, as in this case, have a higher risk of embolization. For thrombi with an elevated risk of embolization, treatment with heparin followed by at least 3 months of warfarin is recommended. Death or severe morbidity is often the consequence of embolization from the left ventricle, and appropriate treatment relies on prompt diagnosis.

Pearls

  • Bedside ultrasound can be effectively applied to evaluate cardiac, abdominal, pulmonary, and vascular conditions in real time.
  • Transthoracic echocardiography is the diagnostic treatment of choice for left ventricular thrombosis, with mortality and morbidity best mitigated by rapid diagnosis and treatment.

Case 3: Neurologic and dermatologic symptoms in a traveler returning from Southeast Asia

Phuoc Le, MD

The patient

A 65-year-old man with a history of idiopathic hydrocephalus who had recently received a ventriculoperitoneal (VP) shunt presented with subacute confusion, headache, and ataxia. He was originally from California and had recently returned after 30 years living in India and Southeast Asia working as an adventure tour guide. He was diagnosed with hydrocephalus 2 years prior when he presented to a hospital in Goa, India, with similar symptoms. After placement of the VP shunt, his symptoms resolved, only to recur in the past several months. He did not report fevers, chills, cough, rash, joint pain, or seizures. He noted multiple small “bumps” in random distribution on his arms and legs. He also described a 20-pound weight loss over several months, along with poor appetite.

On examination, the patient was oriented only to person. Other pertinent findings included a lack of lymphadenopathy, unremarkable cardiopulmonary examination, and numerous palpable small subcutaneous nodules on the upper and lower extremities that were nonerythematous and nontender. There was no associated rash. Cranial nerve evaluation revealed no focal deficit, and the remainder of his neurological exam was normal except for a slow gait. There was a palpable VP shunt along the right neck.

Laboratory examinations were normal, including complete blood count and basic chemistries, thyroid-stimulating hormone, rapid HIV test, and rapid plasma reagin. VP shunt series revealed no evidence of shunt malpositioning but did show multiple small (≤1 cm) calcified subcutaneous lesions in the chest, neck, and abdomen (see Figure 2). http://www.acphospitalist.org/archives/2014/05/the-brief-case_f2.pdf CT and MRI of the brain revealed multiple extra-axial and subarachnoid cystic lesions consistent with neurocysticercosis (see Figure 3). The hydrocephalus was stable from prior imaging. Serology for cysticercosis ultimately returned positive. http://www.acphospitalist.org/archives/2014/05/the-brief-case_f3.pdf

The diagnosis

This patient had cysticercosis, which is caused by ingestion of eggs of the tapeworm Taenia solium. Cysticercosis is endemic in many parts of the developing world, including South America, sub-Saharan Africa, and Southeast Asia, and is one of the leading causes of seizure disorders. After the eggs hatch in the stomach, they can travel to any organ in the body, where cysticerci then form.

Ingestion of undercooked and contaminated pork can result in acquisition of the adult tapeworm in the gastrointestinal tract, but not cysticercosis. Diagnosis of neurocysticercosis can be made on imaging by identifying a scolex (the head of the larvae within the cyst) on MRI. If a scolex is seen, that is essentially pathognomonic for neurocysticercosis. Treatment of neurocysticercosis requires the use of albendazole for several weeks, plus the initiation of systemic steroids if the burden of noncalcified cysts is high; neurosurgical consultation for VP shunt placement may also be necessary if symptomatic hydrocephalus is present.

Pearls

  • Cysticercosis is acquired by ingestion of Taenia solium eggs, not the ingestion of contaminated pork. Vegetarians can acquire cysticercosis if they eat vegetables contaminated with eggs of the tapeworm.
  • Cysticercosis can be found in any part of the body, including subcutaneous tissue, muscle, eyes, bones, and brain; it is important to start systemic steroids simultaneously with antiparasitics if the burden of live cysts is high enough to present an inflammatory response.

Case 4: Painless cutaneous lesions in a woman in Nicaragua

Jack Chase, MD

The patient

A 34-year-old woman with an unremarkable medical history presented to a National Health Ministry clinic in Nicaragua for a painless ulcer on her right leg. She lived on a farm in a temperate, rural area in the North Atlantic Autonomous Region of Nicaragua with her husband and 3 children. The patient explained that 3 weeks earlier she developed a small pink bump on her right shin. The lesion grew in diameter, and the center ulcerated. She reported frequent bug bites and thought this lesion was a “spider bite.” She did not report fever, chills, cough, abdominal pain, nausea, vomiting, diarrhea, or use of bed nets or insect repellant.

Upon arrival at the clinic, the patient's temperature was 36.9°C, her blood pressure was 115/72 mm Hg, and her pulse was 84 beats per minute. On examination, the patient had a 4- 5-cm ulcer on her anterior shin (see Figure 4). The borders were raised, indurated, and nontender with intact sensation. The ulcer was filled with nonpurulent slough. Two similar ulcers were noted overlying the posterior lower leg. The remainder of her physical examination was unremarkable. http://www.acphospitalist.org/archives/2014/05/the-brief-case_f4.pdf

The diagnosis

This patient's diagnosis is cutaneous leishmaniasis, a vector-borne protozoan dermatosis transmitted by sandflies. Over 2 dozen species of the genus Leishmania are endemic on 4 continents and cause a spectrum of disease ranging from isolated, self-limited, cutaneous ulcers (cutaneous leishmaniasis) to organ infiltration, muscle wasting, and hematologic complications with high rate of mortality if left untreated (visceral leishmaniasis). The severity and chronicity of leishmaniasis depend on species virulence factors and host immune factors. Greater parasite burden and either inadequate or overexuberant immune response result in more severe disease.

Leishmaniasis can be diagnosed clinically, and disease manifestations depend on geography. The diagnosis can be confirmed by direct visualization of parasites on tissue biopsy (gold standard) or by antibody enzyme-linked immunosorbent assay (ELISA) testing, DNA polymerase chain reaction (PCR) of tissue samples, or urine antigen testing.

Leishmaniasis treatment is based on species and type of disease. Pentavalent antimonial compounds, such as sodium stibogluconate, are the historical mainstay of treatment. They require multiple parenteral administrations, are frequently difficult to procure, and cause significant side effects, including cardiac arrhythmias and liver toxicity. More recently, a single-dose infusion of amphotericin B has been used with success in multiple settings. A topical cream containing paromomycin, an aminoglycoside antibiotic, was effective against cutaneous leishmaniasis in a recent phase 3 trial. Pyrethroid-impregnated bed nets are the mainstay of prevention efforts in endemic areas. Vaccine trials have not yet shown a decrease in disease incidence in study populations.

Pearls

  • Leishmaniasis is a vector-borne disease with protean manifestations varying based on the parasite species endemic to the geographic location.
  • Cutaneous leishmaniasis is endemic on multiple continents, including Central America, and causes nontender ulcers that resolve over the course of weeks to months.

Case 5: Aseptic meningitis without rash

Brian Secemsky, MD, and Marcia Glass, MD

The patient

A 38-year-old man with minimal medical history presented to an outside hospital for 3 weeks of worsening headaches, fevers, nocturnal diaphoresis, and altered mental status. After 4 days of treatment, the patient came to the UCSF Medical Center for further care. He was fully oriented with photosensitivity, oropharyngeal exudates, and tender bilateral cervical lymphadenopathy. His neck was stiff and painful to palpation. He had no rash.

Lumbar puncture revealed cloudy cerebrospinal fluid (CSF) with an opening pressure of 13 cm H2O, a white blood cell count of 428 cells/μL with 54% lymphocytic predominance, a protein level of 88 mg/dL, and a glucose level of 37 mg/dL (serum glucose level, 102 mg/dL). The cellular specimen predominantly consisted of small, mature-looking lymphoid cells with admixed larger lymphocytes. The CSF sample was positive for varicella zoster virus (VZV) using PCR.

The patient was unsure if he had had chicken pox as a child but said he was exposed to varicella from an infant with chicken pox 1 week prior to the onset of his symptoms. He was treated for 2 days as an inpatient with marked clinical improvement. The patient preferred not to take IV antibiotics and went home with a regimen of oral valacyclovir.

The diagnosis

This patient was diagnosed with subacute aseptic meningitis due to VZV. VZV is a herpesvirus that causes 2 distinct diseases: varicella (chicken pox) and herpes zoster (shingles). In unvaccinated populations, these clinical forms of VZV have incidences of up to 95% and 35%, respectively. While these discrete illnesses classically involve a rash, uncommon manifestations of VZV can make an early diagnosis challenging to internists. As in our patient, this is especially true in lieu of skin findings. For this reason, it is imperative for practitioners to be aware of the extra-integumentary signs of VZV infection.

Aseptic meningitis is a rare complication of VZV infection that can occur in either stage of infection. Even so, in a cohort of patients with central nervous system (CNS) symptoms of suspected viral origin, VZV infection was found to be the most prevalent etiology (more than enterovirus and HSV combined). Consistent with our patient's CSF analysis, lumbar puncture often reveals a lymphocytic pleocytosis with an elevated protein concentration. A concomitant positive varicella PCR is a useful laboratory finding in diagnosing VZV meningitis.

Up to 60% of patients with VZV meningitis have no skin manifestations, which is rare in immunocompetent patients and suggests relatively frequent reactivation of VZV independently of vesicular eruptions. An additional challenge in diagnosing VZV meningitis is that patients who do have skin manifestations may experience a typical rash several months before or after CNS infection. Oral antivirals have minimal CSF penetration, thus making IV acyclovir the treatment of choice. Since our patient preferred not to take IV therapy at home, he was discharged on oral valacyclovir.

Pearls

  • It is important to recognize atypical clinical patterns of varicella zoster virus infection, especially those without characteristic rash.
  • Clinicians should obtain a thorough history, including exposures and vaccinations of varicella zoster virus, when considering subacute meningitis, especially in recent travelers or immigrants.

Case 6: The challenges of palliative care in rural Haiti

Aisling O’Riordan, MB BCh BAO, and Marcia Glass, MD

The patient

A 30-month-old girl presented to a district hospital in rural Haiti with altered mental status, seizures, and fever with onset over 1 week. Previously, the child was well and developmentally normal. On examination she had a decerebrate posture and an open anterior fontanelle. Neurological examination was notable for a spontaneous eye opening, extension to pain, no verbal response, and hypertonia and hyporeflexia in all limbs. Laboratory testing revealed a mild anemia; rapid malaria and tuberculin skin tests were negative. White cell count, bacterial cultures, lumber puncture, CT scan, and ultrasound were not available at this hospital.

The patient was treated empirically for meningoencephalitis with dexamethasone and ceftriaxone. Her condition remained unchanged, and after 10 days she was started on empiric treatment for tuberculous meningitis. Two weeks after admission, the patient was transported to Port-au-Prince, where she received a head CT that revealed hydrocephalus and a space-occupying lesion at the base of the brain, consistent with cerebral malignancy, likely a medulloblastoma. The patient was not a surgical candidate and was discharged home on paracetamol, ibuprofen, and steroids. She was scheduled for follow-up but was unfit for travel. Follow-up from the family described increasing rigidity and uncontrolled pain.

The diagnosis

Young children, like this girl presenting with convulsions and altered mental status, are a common challenge for physicians working in global health. Resources are often limited, and as in this case, diagnostic work-up may not include many key examinations. In these cases, the physician is required to treat empirically according to the most likely and life-threatening causes. Causes to be considered include meningitis, febrile convulsions, head injury, sepsis, and poisoning but should also include diagnosis specific to the region. In this case, it was necessary to rule out both cerebral malaria and tuberculous meningitis. For clinicians in this case, as for many physicians in resource-limited settings, intracranial imaging is difficult to access and costs remain prohibitive. In these cases, a course of empiric treatment is justified, but when a patient has not recovered following initial therapy, it is necessary to pursue more advanced diagnostics.

When this patient left the hospital, she received nonopioid analgesia. As her intracranial pressure increased, so did her analgesic requirements. This type of pain requires the use of weak and subsequently strong opioids as per the World Health Organization pain ladder, which relieves cancer pain in approximately 90% of patients. It is not uncommon for patients to travel great distances to reach hospital care; when sick patients are discharged home to die it is often not practically or financially possible for them to return, as was the case for this child. Despite proven effectiveness, opioid medication is often used sparingly in developing countries and only under the direct supervision of physicians. Poor availability and stigma surrounding addiction has lead to poorly controlled pain for many patients. A community-based approach to palliative care needs to be developed to meet the needs of terminally ill patients.

Pearls

  • Empiric treatment for life-threatening and geographically specific diagnoses is often necessary in resource-limited settings, and failure to respond to empiric treatment requires further evaluation.
  • Analgesia for patients in the developing world is often provided at the hospital level and may be inaccessible to terminally ill patients, leading to poorly controlled pain.

Case 7: Anasarca in an alcoholic patient without cirrhosis

Theresa Poulos; Marisa Morgan, MD; and Marcia Glass, MD

The patient

A 48-year-old woman presented at UCSF Medical Center with 5 days of abdominal pain and difficulty breathing, as well as a week of hemoptysis and painful swelling of her left lower extremity. Her history was notable for alcohol dependency with delirium tremens, chronic obstructive pulmonary disease, and hepatitis C. Upon admission, she was afebrile and her abdominal examination revealed epigastric tenderness to palpation, with erythema and induration of the left abdomen. She had 2+ pitting edema of the lower extremities to the mid-thigh bilaterally; both lower extremities showed marked erythema and were warm and tender to touch. She had a tremor of the upper extremities bilaterally. Her toxicology screen was positive for alcohol, and lactate level was elevated at 2.6 mmol/L. Lower-extremity Doppler ultrasound was negative for deep venous thrombosis. CT of the abdomen was remarkable only for diffuse subcutaneous edema. Transthoracic echocardiogram findings were consistent with a high cardiac output.

The diagnosis

The underlying cause of this patient's anasarca is wet beriberi, high-output heart failure due to severe thiamine deficiency. Fluid accumulation resulting in edema is a common presentation. Distinguishing between dependent and diffuse soft tissue edema can help to narrow the differential diagnosis and result in more timely diagnosis and treatment. Thiamine deficiency can manifest as a peripheral neuropathy (dry beriberi) or with predominantly cardiac symptoms (wet beriberi).

Initially, in wet beriberi, peripheral vasodilation results in a decreased systemic vascular resistance and increased venous return. Continued deficiency leads to impaired myocardial function due to the function of thiamine as a coenzyme in carbohydrate metabolism. Specifically, thiamine deficiency results in impaired utilization of lactate, which then accumulates in the blood causing elevated serum lactate, as was seen in this patient. While elevated blood lactate is nonspecific, it has been used in the diagnosis of beriberi. With continued vasodilation, the kidneys detect a relative volume loss and respond by conserving sodium. Fluid is reabsorbed, leading to diffuse edema, consistent with this patient's otherwise odd pattern of fluid retention.

The body cannot produce thiamine and is only able to store up to 30 mg in tissue. The half-life is 9 to 18 days, so individuals who lack thiamine in their diet will become deficient within a month. Thiamine deficiency is rare in developed countries because most foods are fortified; however, the World Health Organization has documented outbreaks of beriberi among refugees and in developing countries, particularly Thailand and in sub-Saharan Africa, where diets are based on thiamine-poor staples such as polished rice and cassava. This patient was deficient as a consequence of poor nutrition associated with her chronic alcoholism. Gastric bypass patients and individuals on “fad” diets are also at risk. IV thiamine should be administered whenever deficiency is suspected.

Pearls

  • Physicians need to consider thiamine deficiency when evaluating a patient with diffuse anasarca.
  • The diagnosis is largely clinical, as blood thiamine levels may not accurately represent tissue levels.