Andrew C. Berry

An uncommon obliterative lung disease: Swyer–James–MacLeod syndrome

A 42-year-old woman presented with substernal chest pain, productive cough with yellow sputum, and shortness of breath. Chest pain was present for 1 day, and worsened with deep inhalation and movement. The patient denied fever, chills, and diaphoresis. Physical examination revealed a well-appearing women in no apparent distress. Auscultation of the lungs demonstrated moderate crackles in the right lower lung, and chest examination was positive for mild tenderness to palpation along the right anterior chest wall. The remainder of the physical examination was unremarkable. A complete blood count with differential, complete metabolic panel, cardiac enzymes, and electrocardiogram were unremarkable. Anterior–posterior chest X-ray study demonstrated diminished right thoracic cage volume and diminished right lung volumes compared to the left (Fig. 1a). Computed tomography angiogram (CTA) revealed a hyperlucent right lung with diminished vascularity, and an overall reduction in right lung volume compared to the left (Fig. 1b). Ventilation–perfusion scan demonstrated matched defects. Specifically, there was decreased radioisotope uptake in the right lung on both the ventilation and perfusion images, compatible with a destructive process of the entire right lung (Fig. 1c, d). Clinical appearance and imaging supported a diagnosis of Swyer–James–MacLeod syndrome, a rare acquired obliterative lung syndrome. Swyer–James–MacLeod syndrome (SJMS) is an uncommon obliterative lung disease that is a rare complication of a lower respiratory tract infection during childhood [1]. This syndrome is most often seen in children, but can rarely present in adults with a past medical history of recurrent respiratory infections. SJMS can be caused by numerous microbes including adenovirus types 3,7, and 21, paramyxovirus morbillivirus, Bordetella pertussis, Mycoplasma pneumoniae, Mycobacterium tuberculosis, and influenza A [2, 3]. These infections can cause post-infectious bronchiolitis obliterans, and may also cause hypoplasia or agenesis of the pulmonary vasculature. In the latter scenario, hypoperfusion of the pulmonary parenchyma will result in unilateral hyperlucency of the lung on radiographic images [2, 3]. The symptoms of SJMS can vary from asymptomatic to chronic or recurring lung infections, dyspnea, or hemoptysis [1]. Pathogenesis involves inflammation of the respiratory bronchioles, leading to bronchiole wall fibrosis and concurrent narrowing of the bronchiole lumens. Fibrosis in the interalveolar septae is responsible for the destruction of the pulmonary capillary beds, which results in decreased perfusion to the pulmonary artery, leading to pulmonary artery hypoplasia [1]. Diagnosis is made following radiographic evidence (plain film and CT) of the classic SJMS triad: which includes a unilateral hyperlucent lung, diffusely decreased ventilation, and matching decreased perfusion in the affected lung [3]. Unilateral lung hypoplasia, destruction of the pulmonary parenchyma and air trapping (bronchiectasis) are also likely to be visualized on CT imaging [1, 2]. Abnormal pulmonary arterial vasculature characterized by decreased caliber may be visualized if CTA of the pulmonary vasculature is performed [2]. The treatment of SJMS is often conservative, and typically involves & Katherine M. Wojcicki Wojcickk@my.canisius.edu

Osmotic demyelination syndrome: plasmapheresis versus intravenous immunoglobulin?

A 63-year-old man with a past medical history of nonalcoholic steatohepatitis cirrhosis complicated by hepatic encephalopathy and non-bleeding esophageal varices presented for orthotopic liver transplantation. The patient had no acute complications in the immediate post-operative period, and was extubated on post-operative day (POD) 1. At that time, he was neurologically intact, alert and oriented and with no focal neurological deficits. On POD 3, he became lethargic and quadriplegic (Medical Research Council Scale Grade 0), and developed right-sided focal seizures with secondary generalization. His serum sodium was 128 mmol/L. He was re-intubated, and treated for his seizures with lorazepam 4 mg and levetiracetam 2 g, and then continued on levetiracetam 1 g two times a day. The following day, he was unresponsive and had no motor response to painful stimuli. His serum sodium had corrected without additional exogenous intervention to 135 mmol/L. On post symptom onset day (PSOD) 3, an MRI brain without contrast showed chronic small vessel ischemic changes but no other abnormality (Fig. 1a). The EEG did not show any seizure or epileptiform discharges. Serum chemistry and cerebrospinal fluid analysis did not show any significant abnormalities. On PSOD 13, his presentation remained the same. An MRI brain was repeated showing DWI restriction and high T2 signal in the central pons, suggestive of ODS (Fig. 1b). On PSOD 19, he was started on both IVIG and PP for a total of 5 days. Approximately 3 weeks after treatment with IVIG and Plasmapheresis, a repeat MRI showed similar prominence of T2 hyperintensity in the central pons with sparing of the periphery as compared to prior, findings consistent with central pontine myelinolysis/osmotic demyelination syndrome (Fig. 1c). Over the next 90 days the patient improved, becoming fully alert, regaining spontaneous muscle flicker in all four extremities (Medical Research Council Scale Grade 1), full eye movements and the ability to swallow. Osmotic demyelination syndrome (ODS) is a disorder characterized by the destruction of neuronal myelin sheaths in either the central area of the pons as in central pontine myelinolysis (CPM), or in other susceptible areas such as the basal ganglia, hippocampi or cerebellum known as external pontine myelinolysis (EPM). CPM can present with T2 hyperintensities on MRI in a classic trident-shape pattern. ODS usually presents as a complication of rapid correction of hyponatremia. Although no specific treatment has been described, plasmapheresis (PP) and intravenous immunoglobulin (IVIG) have been suggested as possible options for the management of ODS [1]. A clear association has been established between rapid correction of hyponatremia and the development of ODS. Although not completely understood, the pathophysiology of ODS classically described is the reduced extracellular osmolality causing brain cells to release osmotically-active substances in an attempt to achieve osmotic equilibrium. These osmotic substances cannot be reabsorbed rapidly, and when sodium levels are increased, they create an osmotic stress that leads to a disruption in the blood brain barrier, leading to myelinolysis. In addition, the death of oligodendrocytes via apoptosis from osmotic shifts has also been suggested & Andrew C. Berry aberry5555@gmail.com

Immunomodulator and Biologic Agent Effects on Sleep Quality in Patients With Inflammatory Bowel Disease

Background Inflammatory bowel disease (IBD) can disrupt normal sleep physiology and amplify a negative perception about quality of life. Evidence suggests increased circulation of inflammatory cytokines, such as tumor necrosis factor-alpha and interleukin-1, may play a role. Methods A total of 56 patients completed the Pittsburgh Sleep Quality Index (PSQI) to measure 7 sleep domains: sleep quality, sleep latency, sleep duration, sleep efficacy, sleep disturbance, sleep medications, and daytime dysfunction. Domain scores were summed to determine the presence or absence of sleep impairment. We compared patients taking immunomodulators or biologic agents to patients not on immunomodulator or biologic agent therapy. Demographics and IBD-related clinical information were collected to adjust for potential confounders that may secondarily affect sleep, such as body mass index, depression/anxiety, and sleep-affecting medications. Results The majority of patients with IBD (46 [82%]) reported poor sleep quality; 22 (79%) of the patients taking immunomodulators or biologic agents and 24 (86%) of the patients not on these therapies had a global PSQI score ≥5, suggestive of poor sleep quality. However, we found no significant difference between the 2 groups. When we analyzed the 7 PSQI sleep domains individually, we found improved sleep duration in the group taking immunomodulators or biologic agents compared to the group not on therapy, although the difference was not statistically significant. Conclusion The majority of patients with IBD experience some degree of sleep impairment, and treatment with immunomodulators and biologic agents does not appear to improve sleep quality. A multicenter study with a larger sample size is warranted to better assess the diverse population of patients with IBD and the factors that impact their sleep. Routine assessment of sleep quality during IBD clinical encounters is recommended.

Intracranial hypotension: diagnosis by trial of Trendelenburg positioning and imaging

An 80-year-old woman with well-controlled hypertension, hypothyroidism, and an atraumatic fall 3 weeks prior, presented with complaints of altered mental status and left eye drooping for 1 day, without headache. On initial examination, she was noted to be lethargic with a partial left third nerve palsy, with preserved but sluggish pupillary light reflex. A CT head scan was done, revealing bilateral acute on chronic subdural hematomas with an admixture of hypodense material in the subdural space and some hyperdense components (Fig. 1). Given her third nerve palsy, there was concern for pathology other than the subdural hematoma. An MRI brain scan with and without contrast was done, and revealed diffuse pachymeningeal enhancement, in addition to bilateral frontoparietal subdural collections. (Fig. 2d–f) The collections were heterogeneous and thought to represent a mixture of proteinaceous CSF material and acute blood (Fig. 2d–f). A lumbar puncture was performed, with an opening pressure of 9 cmH2O. CSF studies showed: glucose 64 mg/dL, protein 129 mg/dL, 4444 red blood cells, 167 white blood cells (24 % neutrophils, 76 % lymphocytes), and a negative Gram’s stain. The pachymeningeal enhancement, new cranial nerve palsy, and elevated CSF white blood cell count were thought to be consistent with chronic subdural hematoma versus lymphoma, inflammatory process, or other malignant process. While CSF cultures, cytology and flow cytometry were pending, the patient’s course improved, and she was able to maintain upright posture and feed herself. However, within a day of this improvement, she was again noted to have fluctuating mental status, ranging from baseline intact mental status to lethargic and obtunded. She also developed a new right third nerve palsy, which intermittently resolved and recurred spontaneously. EEG monitoring was done and revealed only diffuse slowing with no epileptiform activity. In addition, she developed episodes of hypertension with bradycardia and irregular breathing, which are the symptoms of Cushing’s triad, a syndrome associated with increased intracranial pressure. Given a fluctuating course and concern for increased intracranial pressure with the signs of Cushing’s triad, imaging was reviewed again. On second review, further abnormalities were noted, including engorged cortical veins and venous sinuses and signs of brain sagging. Brain sagging describes the downward displacement of the brain that was demonstrated in our patient with: ventricles smaller than expected for age, increased anterior–posterior and decreased bipeduncular diameter of the mesencephalon, an effaced interpeduncular cistern, enlarged pituitary gland, and a sagging corpus callosum with a down-pointing splenium (Figs. 1, 2) These imaging findings raised concern for spontaneous intracranial hypotension (SIH). The patient was subsequently placed in Trendelenburg position, and her mental status improved dramatically. She did, however, have a persistent partial left third nerve palsy. Although this was thought to support the diagnosis of SIH, there was some concern that the clinical improvement may have been coincidental, as her mental status had been fluctuating throughout her hospital course. A CT myelogram was done, revealing CSF extravasations at T11/T12, supporting the diagnosis of SIH (Fig. 3). As the patient’s examination continued to fluctuate with position, a lumbar epidural blood patch was performed. The patient showed significant & Andrew C. Berry Aberry5555@gmail.com

Relationship between Patient Knowledge and Medication Adherence in Inflammatory Bowel Disease

nal ileum (Fig. 1A). Esophagogastroduodenoscopy showed round ulcers in the middle esophagus (Fig. 1B). Biopsy specimens revealed no specific findings except for inflammatory cellular infiltrates (Fig. 1C). Stool culture and an examination by an ophthalmologist showed no specific findings, and HLA B51 and A26 were negative. Based on these CD/ Behçet’s disease–like findings, we treated the patient with oral prednisolone (40 mg/d). Two months later, no recurrent lesions were seen on colonoscopy, esophagogastroduodenoscopy, or a skin examination except for a longitudinal ulcer scar in the terminal ileum. The risk of paradoxical psoriasis has been reported to be as high as 1.6% to 10.1% in patients with CD treated with tumor necrosis factor-alpha inhibitors,3 which are also used for the treatment of psoriasis. Although the mechanism underlying paradoxical psoriasis has not yet been elucidated, an imbalance of cytokines is suspected to be involved.3 The blockade of IL-17 may also induce an imbalance in the cytokine levels, thereby resulting in the onset of CD or Behçet’s disease.

Giardiasis: a malignant mimicker?

SIMI 2015 A 27-year-old man, with no pertinent past medical history, presented with a 3-week history of severe nausea, vomiting, diarrhea, and abdominal pain. The pain was nonspecific and described as bloating. Associated symptoms included subjective fever and flatulence. The patient worked as a truck driver, traveling often, who had recently transported a shipment of river mulch. His mother was recently diagnosed with colon cancer and grandmother with lymphoma. The patient was afebrile, normotensive, and mildly tachycardiac on arrival. No cough, chest pain, alcohol use, weight change, sick contacts, or bleeding was reported. Physical examination revealed diffuse abdominal tenderness without peritoneal signs. Complete blood count showed a leukocytosis at 17.4 9 10 3 /lL. Folate, B12, and

Ring-enhancing brain lesions: Listeria monocytogenes abscesses.

An 82-year-old woman with a history of bilateral temporal arteritis and atrial fibrillation presented with short-term memory loss and feeling of being off-balance the previous 1 week. She could not remember her grandchildren’s names and birthdays, which her family deemed unusual. Neurological evaluation a year prior to admission did not show dementia or any other alarming neurological symptoms. The patient had a head computed tomography (CT scan) without contrast 2 days prior to admission with neurology that was concerning, and she was subsequently sent to our emergency department (ED) (Fig. 1a). She had biopsy-proven bilateral temporal arteritis 3 months prior to admission, and had been on a prednisone taper since. The vital signs on admission included: 35.9 C (96.7), 166/79 mmHg, 65 beats/min, 17 breaths/min, 96 % oxygen saturation on room air, and body mass index of 24. The physical examination was unremarkable except for a moon facies, facial erythema, irregular irregular heart rate and rhythm, and right eye blindness. The patient was oriented to self, not time, and could name and slowly follow commands. Cranial nerves 2–12 were grossly intact. Strength 5/5 in all limbs, but the patient was slow with movements. Sensation was grossly intact. Repeat head CT with contrast was performed and revealed three ring-enhancing lesions of the biparietal–occipital lobes surrounded by vasogenic edema and effacement of the occipital horn of the left lateral ventricle (Fig. 1b). There was no evidence of hemorrhage, midline shift, or acute infarct. The head CT scan without contrast prior to admission showed two ringenhancing lesions (Fig. 1a). RPR, toxoplasma antibody, and HIV were all negative. Blood cultures revealed 1 of 2 Gram-positive rods, later confirmed as Listeria monocytogenes (Fig. 1c). Culture of the brain lesion biopsy further confirmed L. monocytogenes brain abscesses (Fig. 1d). The patient was switched from empiric vancomycin, ceftriaxone, and metronidazole to ampicillin 2 g IV Q4H to be continued for 6–8 weeks. In patients presenting to the ED with acute confusion, neurological decline or deficits, or any other change in mentation, thorough clinical examination and neurological imaging are vital for proper medical workup. There are many possible culprits of ring-enhancing lesions such as radiation necrosis, metastasis, abscess, glioblastoma multiforme, subacute infarct, contusion, or lymphoma. It is imperative to delineate the cause of the ring-enhancing lesion, as the treatment varies extensively among the aforementioned causes. Groups at risk for listerial infection include pregnant women, immunocompromised, elderly, or debilitated adults with underlying diseases. Ingestion of listeria-contaminated food is likely the source of infection in all human listerial infections. In our case a particular brand of ice cream consumed by the patient with a recent history of United States listeria outbreak remains the likely culprit. In the immunocompetent human, L. monocytogenes exposure usually results in self-limited, febrile diarrheal gastroenteritis lasting 1–3 days. In the immunocompromised, gastrointestinal invasion can lead to bacteremia, with a predilection for the CNS, where it can cause meningitis, meningoencephalitis, or rhombencephalitis [1]. Although L. monocytogenes is a well-known cause of meningitis and encephalitis, brain abscesses caused by this organism are highly uncommon and reported to occur in & Andrew C. Berry aberry5555@gmail.com

Fibromatosis colli spuriously presenting as a retropharyngeal mass on cervical spine radiographs

A 6-month-old boy with a 6-month history of torticollispresented to the Emergency Department (ED) from clinicbecause of an outpatient cervical spine X-ray study thatshowed abnormal pre-vertebral soft tissue swelling in theneck, suspicious for retropharyngeal abscess (Fig. 1). Thepatient was referred to the ED to obtain a computedtomography (CT scan) of the neck to evaluate for abscessversus retropharyngeal mass. The patient’s mother reporteda 6-month history of the patient holding his head mostly tothe right side with considerable right-sided neck weakness.The mother also reported that the patient had not beenreaching some developmental milestones due to hisinability to lift his head or sit up without support. A neu-rologist had recently evaluated the patient, and an elec-troencephalogram (EEG) was normal. The patient had ahistory of GERD and asthma being treated with zantac andalbuterol, respectively. Birth history was unremarkable,and the patient was up to date on his immunizations. Whilethe history was positive for neck pain to the right, it wasnegative for fever, drooling, difficulty breathing, neckswelling, cough, and congestion. Physical examinationrevealed a playful, well-developed 6-month-old male infantin no apparent distress, with no masses or swelling of theneck. The neck had a full range of motion in all directions,yet patient favored the right. The patient would not lift hishead off the bed, roll over, or sit up on his own. He wasable to drink well without difficulty. The patient’s vitalsigns, complete blood count, and complete metabolic panelwere all unremarkable. Blood cultures were negative. NeckX-rays in neutral, flexion, and extension were obtained thatshowed persistent cervical pre-vertebral soft tissue thick-ening in all three positions. A CT scan of the neck withcontrast demonstrated no retropharyngeal fluid collectionor inflammatory process, and there was proper patency ofthe airway. Asymmetric thickening of the right stern-ocleidomastoid muscle, consistent with fibromatosis colli,was identified. These results, together with clinical exam-ination, suggest the diagnosis of torticollis secondary tofibromatosis colli. The apparent pre-vertebral soft tissuethickening seen on earlier lateral X-rays was concluded tobe from overlapping soft tissues secondary to torticollis.Fibromatosis colli (FC) is a rare benign tumor of thespindle cells of the sternocleidomastoid (SCM) muscle.This lesion is characterized by diffuse or focal enlargementof the SCM muscle by fibrous tissues. It is only seen in0.4 % of infants, with 75 % of these cases involving theright SCM muscle. There is a slight male predominance[1]. Although the exact etiology of FC is unknown, it isthought that FC is related to intrauterine or birth traumaresulting in ischemia and scar-like retraction of the SCMmuscle. Typical examples of birth trauma leading to FCinclude difficult or prolonged labor, forceps assisteddelivery, and breech presentation [2]. FC typically presents2–4 weeks after birth as a nontender, palpable neck massalong the course of the SCM muscle. Decreased range ofmotion of the neck is common, and congenital torticollis isseen in approximately 20 % of infants with FC [3].When an infant presents with suspected congenital tor-ticollis or a neck mass, ultrasound imaging is the preferredinitial diagnostic tool due to its low cost and lack of

Initial Presentation of a Pancreatic Mass

Question: A 55-year-old African American man with a medical history of chronic pancreatitis, pancreatic mass, alcohol abuse, hypertriglyceridemia, gastroesophageal reflux disease, and essential hypertension presented to the emergency department with severe abdominal pain radiating to his back that was associated with diaphoresis and weight loss. The initial workup demonstrated acute pancreatitis. Prior radiologic studies were reviewed to better characterize the pancreatic mass. More recent computed tomography and MRI scans demonstrated an enlarging, cystic and septated mass within the pancreatic head with marked dilatation of the patient’s pancreatic, and intrahepatic and extrahepatic biliary ducts (MRI; Figure A). Additionally, there was evidence of peripancreatic fat infiltration and multiple enlarged peripancreatic lymph nodes. Ultrasonography had also shown that the mass was displacing the patient’s duodenum.

Hepatobiliary and Pancreatic: Extrahepatic cavernous hemangioma presenting as a palpable abdominal mass

An 89-year-old Caucasian female with history of total abdominal hysterectomy with unilateral oophorectomy presented with abdominal pain, cramps, nausea, and diarrhea. Her vital signs were normal and physical examination was unremarkable except for a palpable abdominal wall mass of ~10 cm in the right upper quadrant. The mass was non-tender and non-pulsatile. Lab testing including complete blood count, basic metabolic panel, urine analysis, Cancer antigen (CA) 125, CA 19-9, and occult blood were all within normal limits. Computed tomography (CT) of the abdomen with contrast showed re-demonstration of benign cavernous hemangioma in the dome of the liver consistent with previous imaging five years prior (Fig. 1a). A new mass was present at the abdominopelvic junction on the right measuring 13 × 9 × 10 cm (Fig. 1b). The low attenuated mass with regions of nodular enhancement abuts the liver as well as loops of bowel including the duodenum. A fat plane is present between parts of the mass and the adjacent liver. An ultrasound-guided biopsy via core needle technique with subsequent histological workup revealed a hypervascular peritoneal mass consistent with a benign cavernous hemangioma and no evidence of malignancy (Fig. 2). Hepatic hemangiomas are the most common benign mesenchymal hepatic tumors, with prevalence ranging from 0.5–20%. The majority are discovered at autopsy or incidentally during imaging. Most patients with hemangiomas are asymptomatic, with lesions greater than 4 cm more likely symptomatic. Abdominal hemangiomas have been described in numerous locations, including the gastrointestinal tract, retroperitoneum, skeletal muscle, and mesentery. They traditionally are diagnosed on CT, but with unclear demarcation of its origin, subsequent imaging modalities should be considered. This patient’s clinical presentation was suspicious for a malignant mass in the abdomen. With histology confirming a benign cavernous hemangioma, the suspicion of a exophytic tumor remained. However, CT of the abdomen with contrast demonstrated no clear connection with the liver and made identification of hepatic origin challenging. Pedunculated exophytic hemangiomas show a thin pedicle that contains a feeding artery and draining vein, and this pedicle connects the hemangioma to the liver. In contrast, usual exophytic hemangiomas just show contrast enhancement at the contact surface with the caudate lobe. This case does not show evidence of either traditional finding on contrast CT, thus, the origin of the benign cavernous hemangioma remains unclear. Magnetic resonance imaging has been shown to be an excellent modality for both benign and exophytic liver tumors, and would be an useful imaging modality in this case. The differential diagnosis of intraabdominal masses in the elderly population is extensive and must include hemangioma. Contributed by AC Berry,* R Nakshabendi and BB Berry *University of South Alabama, Medicine, Mobile, Alabama, University of Florida College of Medicine-Jacksonville, Medicine, Jacksonville, Florida, and Wheaton Franciscan Healthcare, Medicine, Milwaukee, Wisconsin, USA Figure 2 Fibrovascular tissue with large irregularly shaped and dilated blood vessels, and lined by a single layer of endothelial cells. a b