A 34-year-old male patient presented to our center following a fall from a height. The patient had no history of medical illness, family history, or history of drug intake. The patient was resuscitated and managed according to the advanced trauma life support protocol, and he was found to be a transient responder (bleeding 20–40%).
Focused assessment sonography in trauma was positive for fluid in Morrison's pouch. Computed tomography (CT) showed multiple bilateral nondisplaced rib fractures, comminuted scapular fractures, and bilateral lung contusions. Furthermore, abdominal injuries were found, such as grade 4 liver laceration, left renal artery injury resulting in left kidney devascularization, and a right adrenal hematoma. Although initially responsive, following a CT scan, the patient became vitally unstable. Therefore, resuscitation was started, and a plan to take him to the operative room was made.
Intraoperatively, the liver was found to be the primary site of hemorrhage, which was controlled with packing. The lesser sac was opened, and no blood or bile was found, and postrior pastric wall was inspected with no injuries,
Additionally, a serosal tear was found in the transverse colon, which was primarily repaired with continuous PDS suture. Packing with temporary closure of the abdomen was performed (damage control laparotomy), and the patient was sent to the intensive care unit (ICU).
Forty-eight hours later, the patient was taken to the operating room (OR) for a second look. Exploration was uneventful, and the abdomen was successfully closed.
Despite an initial uneventful postoperative recovery, 2 weeks after surgery, the patient’s condition deteriorated. Abdominal examination suggested the presence of gas coming from the upper part of the laparotomy wound, and a diagnosis of septic shock was made. Following resuscitation, the patient underwent an emergency exploratory laparotomy, which was performed by an on-call trained trauma consultant.
Upon exploration, a 2-cm long, full-thickness posterior gastric injury was found at the level of the cardia just below the GE junction. The necrotic and ischemic margins were debrided resulting in a larger defect that extended up to the GE junction (). Despite the difficulty in gaining good exposure of the injury, primary two-layer closure of the gastric rupture was performed with continuous 2.0 PDS sutures (Z775D) and reinforced with Vicryl 2.0 (J589H) interrupted sutures. No other injuries were found. The patient was sent to the surgical ICU with an open abdomen.
After 48 hrs of observation, the patient was returned to the OR. A jejunostomy feeding tube was inserted, and the abdomen was closed using an abdominal wall closure system device (ABRA; CWK08, Southmedic Inc. 50 alliance Blvd, Barrie, Ontario, Canada).
During the third exploration, the gastric injury that was previously repaired showed a persistent leak. A trial of nonoperative treatment with a Niti-S esophageal BETA-2 covered stent (size 28 mm × 180 mm, made in Korea, LBN 00-01) and wide drainage were initiated through gastroscopy.
Five weeks later, a fluoroscopy study showed a para-stent leak at the proximal end (). Later on, upon endoscopy for the replacement of the stent, the previously noted perforation decreased in size. However, a gastric ulcer was found at the level of the pylorus (the distal end of the stent) (), so re-stenting was deferred. Conservative management was planned in the form of jejunostomy tube feeding and 40 mg proton pump inhibitor was given for 2 weeks. Furthermore, endoscopic stenting was performed. The patient tolerated the consequences of the above management option with complete satisfaction.


A right-handed 27-year-old female, 2nd year Internal Medicine resident had no relevant family history. In her past medical history, she had an event of right optic neuritis with amaurosis that resolved without sequelae 3 years before present medical history. At that time, she was treated with intravenous steroids and was followed up by a neurologist at her hometown. No further investigations or antimyelin oligodendrocyte glycoprotein (anti-MOG) antibodies testing were performed; the optic neuritis was a single self-limited event.
She began 2 years ago with a sudden feeling of “joy” that lasted for 40s, followed by paresthesia in both inferior extremities with loss of awareness and manual automatisms with the right hand that ended with a tonic posture of the four extremities. Phenytoin (PHT) was initiated, with a diminished frequency of seizures, about once per week. One month later, during a flight, the patient experienced transient symptoms of inability to recognize faces (prosopagnosia) and incapacity to interpret words (she describes an event where she saw the word “Mexico” but read “Comiex”). Her symptoms were brief (about 1 min each) and occurred many times during that day. A diagnosis of non-motor status epilepticus was made with an electroencephalogram (EEG), oxcarbazepine (OXC) was initiated, PHT was discontinued, and patient became seizure free.
Six months later, she developed hyponatremia due to OXC, and her antiepileptic treatment was again modified to levetiracetam (LEV). During her outpatient follow-up, she still presented with “flashing lights in colors predominantly red, blue and green in her lower right visual field,” prosopagnosia, and other visual disturbances including seeing her hand as if it were torn in half, finally followed by visual hallucinations characterized by hair invading the faces of people around her with an intense sense of fear. These always occurred in a stepwise manner, and the patient drew her seizures (). Lacosamide (LCM) and topiramate (TPM) were added to her antiseizure (AS) treatment.
In the course of the following year, she was admitted in second-level care centers on four occasions for treatment and observation, with recurrent EPC as described. Due to the lack of improvement in her symptoms, suspicion arose of a diagnosis of a non-neurological entity such as psychogenic non-epileptic seizures (PNES); this was assumed to be secondary to stress from workload in her residency. She was sent to a local psychiatrist, beginning treatment with fluoxetine and, due to poor response in her symptoms, was switched to escitalopram. The patient came to our third level of attention to the emergency room, as she had continued episodes as previously described for approximately 2 weeks. These episodes appeared intermittently during the day, lasting for brief seconds, but the patient constantly experienced them, which could also be present in her sleep appearing as dreams with the same manifestations (see ). Upon neurological evaluation, a cecocentral scotoma was detected (confirmed by the Neuro-ophtalmology team); the rest of her exam was normal. A conclusion of a misdiagnosis of PNES was established. IV PHT load was given (1,250 mg), and she was transferred to the Neurology ward for clinical workup.


A 36-year-old male from central region of Kenya presented to our institution with a 3-day history of back pain, followed by itching and numbness of the feet and ankles. On presentation, his symptoms had progressed to weakness of the lower limbs with inability to walk and he had developed urinary incontinence. He denied speech and swallowing difficulties and reported no weakness of the upper limbs. The patient reported no history of fevers, blurred vision, loss of consciousness, or seizure. He also denied a history of trauma, use of blood thinners, use of illicit drugs (including tobacco and alcohol use), a history of exposure to tuberculosis, or ingestion of raw milk. He did report swimming in a nearby pond for many years. His family history was unremarkable, and he was an only child.
On examination, he had normal vital signs and was conscious and alert. A neurological exam revealed normal cranial nerves. Visual field was also preserved. Examination of both his upper limbs was normal. He had tenderness of the lower back, power grade 4/5 on hip flexors and extensors bilaterally, grade 4/5 and 2/5 on the right and left knee extensors, respectively, and power grade 3/5 and 1/5 on the right and left knee flexors, respectively, and dorsiflexion was grade 1/5 bilaterally. A knee jerk reflex was present on his right lower extremity but reduced on the left. Ankle reflexes were absent bilaterally. Sensory level was present at L2.
His complete blood count and a comprehensive metabolic panel were within normal limits. A lumbar puncture revealed a protein count of 0.97 g/l (0.15–0.45 g/l), with a pleocytosis of 35 mm3(normal <5). A differential count showed 30% of segmented neutrophils and 70% of lymphocytes. Monocytes, eosinophils, and basophils were absent. The glucose level was normal. Cultures of the cerebrospinal fluids, including for tuberculosis, were negative. Serological test for HIV, herpes virus, and syphilis was also negative. An autoimmune screen including ANA, anti-ds DNA, and ENA was negative. A vasculitis screen and serum angiotensin-converting enzyme were also reported as normal.
His thyroid stimulating hormone, vitamin B12, and folate level were all within normal limits.
An MRI of the spine showed longitudinally extensive transverse myelitis (T2 hyper intense intramedullary signal spanning T6-L1 with enhancement) (Figures –).
Based on his MRI results and clinical symptoms, neuromyelitis optica (NMO) was high on the differential diagnosis. He was initiated on high-dose steroids, methylprednisone one gram intravenously for 5 days. However, his neurological status continued to decline. Five sessions of therapeutic plasma exchange were also attempted with no improvement in his symptoms. Serum and CSF neuromyelitis antibodies and oligoclonal bands as well as serum MOG antibodies (myelin oligodendrocyte glycoprotein) were later reported as negative.
A few days into his hospitalization, his Schistosoma antibody (IgG) titer was reported at at 1 : 80. A subsequent liver ultrasound was reported as normal. He was immediately started on praziquantel at 60 mg/kg/dose and high-dose steroids with minimal improvement in his overall symptoms. He was discharged home with recommendations for home care and aggressive physical therapy.


A 36-year-old male patient came to the Department of Maxillofacial Surgery, University Hospital Clinic Zagreb in December 2010 due to a swelling of the left body of the mandible that was noticed 4 months earlier. The patient did not report any other symptoms. After examination, the patient was referred to a maxillofacial surgeon regarding an atypical clinical and radiological finding in the form of a multicystic appearance (bubble-like) in the corpus of the left mandible and pathological teeth mobility in the region of the swelling. There were no enlarged lymph nodes in the neck and head area. Differential diagnosis included keratocystic odontogenic tumor, ameloblastoma, central giant cell granuloma, and odontogenic myxoma.
The patient denied any systemic disease or condition and reported no previous surgeries. A general physical examination was unremarkable. The patient did not have any deleterious habits such as alcohol consumption or smoking and without previous history of swelling or trauma of the mandible. On extraoral examination, the patient displayed some facial asymmetry with an obvious firm and diffuse swelling on the left side of the mandible. The overlying skin was normal in appearance. Intraorally, there was a diffuse swelling in the buccal vestibule. Teeth in the third quadrant were without any pain sensation but with pathological mobility. Lower incisors and canine had mobility grade I and premolars and first molar grade II according to the Miller classification.
The orthopantomogram (OPG) showed a multilocular radiolucency, with fine trabeculation, extending from the left canine, towards the first molar () with no root resorption. A CT scan was made afterwards and revealed the destruction of the buccal cortex of the left body of the mandible. A probatory biopsy was performed under local infiltration anesthesia (4% articaine with epinephrine 1 : 200 000; 1.8 mL), and the specimen was sent for histopathologic examination which reported odontogenic myxoma.
In accordance with the ethical protocol of the School of Dental Medicine, University of Zagreb, Croatia, written consent was obtained from the patient before surgery in general anesthesia in January 2011. Lower incisors, canine, premolars, and first molar were extracted just before the first incision. A mucoperiosteal flap with full exposition of the buccal aspect of the tumor was performed. The tumor was removed with a marginal resection of the mandible from the left first incisor to the left second molar. The surgical site was examined and precociously cleaned of all myxomatous tissue, and a reconstruction plate was used to reinforce the resected mandible. The surgical specimen was a soft, gelatinous mass measuring approximately 1 cm in diameter, with a mucinous appearance. Histologically, the tumor was composed of randomly oriented spindle-shaped cells with long fine cytoplasmic processes, within an abundant, myxoid ground substance, containing small capillaries and fragments of bone trabecula. Odontogenic epithelium was absent, and clear margins were confirmed (Figures and ). The patient recovered completely, with no postoperative paresthesia or facial asymmetry.
Two years after surgery, in January 2013, the patient was referred to the Department of Oral Surgery, School of Dental Medicine, University of Zagreb for implant-prosthodontic rehabilitation. A control OPG () and clinical examination (Figures and ) showed complete recovery with a residual defect in the alveolar extension and left concavity.
Due to the deep bite, orthodontic therapy was started in the upper jaw, and in the lower jaw, implant-prosthodontic rehabilitation was planed with minimally invasive procedures because of the lack of a support zone and defect of soft and bone tissues. Regional nerve block anesthesia (4% articaine with epinephrine 1 : 200 000; 3.6 mL) was administered. After carefully raising the mucoperiosteal flap and isolating the mental nerve, the bone crest was expanded at the planned implant sites, with a piezosurgical bone saw (Piezomed, W&H; Austria: power 90%, cooling 80%, “power” operating mode), and two narrow diameter implants (Straumann, Bone Level Roxolid 3.3 × 10 mm, Basel, Switzerland) were placed at positions 32 and 36 (Figures and ). After implant placement augmentation was performed with xenogenic bone material (Cerabone, Botiss, Germany) and a resorptive native pericardium membrane (Jason membrane 15 × 20 mm, Botiss, Germany), the sutures were removed on the 10th postoperative day at which time the surgical site was healing as expected.
Three months after surgery, a cover denture was made with locator connections to compensate for lost teeth and to correct the ratio of soft tissues and facial contours. No tumor recurrence was found on the follow-up OPG and CBCT for 7 years after implant placement and 9 years postresective surgery (Figures –). The patient was subjectively free from functional and aesthetic problems.


A 15-year-old lady presented with symptoms of allergic conjunctivitis such as epiphora, pain, and redness of the lid margin of the right eye. On her eye examination, she had visual acuity of counting finger from 1 m, diffuse vascularized corneal opacity, ectropion and injected conjunctiva with papillary reaction. Additionally, she had physical signs of allergy such as periorbital edema, erythema and scaling of adjacent skin (Fig. a, b). However, there were not any signs of cicatricial component such as lamellar shortening or lid retraction. She had a history of admission in our ward for the same eye fungal keratitis 9 months ago (Fig. c). She had been treated with topical Natamycin (Natacyn, Alcon, Fort Worth, TX). After partial improvement of the corneal ulcer, she had been discharged with topical 5% Natamycin four times a day. She hadn’t come for follow up visits and continued using Natamycin for a total of 9 months until her recent visit. She did not use any other topical or systemic medication during that time. We stopped Natamycin and administered lubrication and a topical steroid (0.1% Fluorometholone (FML, Allergan, Inc., Irvine, CA) every 6 h. One month later, the ectropion resolved completely (Fig. d, e).


A 79-year-old man with a 66 mm ruptured AAA was admitted to our hospital in shock. Computed tomography (CT) revealed a 51 mm serial left common iliac artery (CIA) aneurysm from the AAA, retroperitoneal hematoma, and evidence of active bleeding from the anterior wall of the AAA (). EVAR was conducted as an emergency surgery. Before EVAR, an intra-aortic balloon was inserted and inflated at the descending aorta for hemostasis. Initially, the left internal iliac artery (IIA) was occluded by using embolization coils (Tornado® Embolization Coil, Cook Inc., Bloomington, IN, USA). A bifurcated endovascular graft (Gore Excluder Endoprosthesis, W. L. Gore & Associates Inc., Flagstaff, AZ, USA) was deployed at the infrarenal lesion to land distally in the right CIA and external iliac artery (EIA) with limb extension. The patient’s blood pressure increased immediately after completion of EVAR, although more than 4 h had passed since the onset of the AAA rupture. The operation time was 115 min.
On the second postoperative day, the patient was found to have developed left lower limb incomplete paraplegia. Based on suspicion of SCI, the patient was treated with cerebrospinal fluid (CSF) drainage, high-dose steroid infusions, naloxone, and edaravone. Magnetic resonance imaging (MRI) identified spinal cord infarction at T11–L1. Retrospective analysis of preoperative CT scans revealed the origin of the Adamkiewicz artery at L1–L2 level (). This origin may have been unintentionally covered by the endograft. Based on patient records, the patient did not have any drop in his blood pressure below 80 mmHg in the postoperative period. Currently, the patient is receiving walking rehabilitation; his bladder incontinence has remained unchanged 43 months post-surgery.


A 76-year-old man with AAA presenting with severe abdominal pain was found to have a 60 mm infrarenal AAA on non-contrast-enhanced CT. A contrast-enhanced CT conducted 4 years ago showed an extremely shaggy aorta (). This patient was considered unsuitable for open surgery because of his poor general condition, severe renal dysfunction, and sarcopenia. Emergency EVAR was conducted to prevent AAA rupture. The right IIA was occluded by using embolization coils since the landing length of right CIA was short (15 mm) with a hostile neck and raised concerns regarding a type1b endoleak. This was followed by EVAR with Gore Excluder endoprosthesis. The distal landing sites were left CIA and right EIA with limb extension. The patient was diagnosed with complete paraplegia soon after EVAR. Although SCI was intensively managed, including CSF drainage, steroid infusions, and naloxone, the patient died 9 days following EVAR secondary to bowel necrosis.


A 77-year-old man with AAA underwent elective EVAR. Preoperative CT scan identified a 56 mm infrarenal AAA and a 26 mm right IIA aneurysm, with chronic total occlusion of left IIA, and collateral circulation from the left femoral artery (). At first, the right IIA was embolized by using Tornado coils. Subsequently, EVAR was conducted using an Endurant stent graft system (Medtronic, Inc., Santa Rosa, CA, USA). Distal legs of the endograft were placed in the right EIA and left CIA. Four days after EVAR, the patient complained of right lower limb paresis. Brain MRI and CT scans did not identify any abnormal findings. Initially, the patient appeared to recover well and was able to walk around the ward. However, on the 12th postoperative day, he developed right lower limb paralysis for the second time. Emergency spinal MRI confirmed subacute spinal cord infarction at T3–T6. He was treated medically without CSF drainage. Although his muscle power improved to the point that he could walk with a cane, the patient died of lung cancer 21 months later.


A 63-year-old nonverbal male with moderate ID who lives in a group home had a history of multiple significant comorbid diagnoses, including arthritis, anemia, gastroesophageal reflux disease (GERD), hyperlipidemia, and Parkinson's disease (). In addition, the patient was diagnosed with major depressive disorder and had been treated for several years with the atypical antipsychotic, quetiapine (300 mg once daily (QD)).
TD symptoms included constant tongue protrusion and intermittent chewing movements of the jaw that led to severe drooling and difficulty swallowing. The patient also had excessive, pronounced eye blinking, bilateral shoulder/hand/finger movements (predominant on the left side at rest), and constant left foot tapping. His abnormal body movements affected his balance and stability such that he could not stand stably without support and required a wheelchair for mobility (). In addition, his abnormal movements caused difficulty performing activities of daily living (ADLs), such as feeding himself. The duration of these symptoms is unknown. The patient was initiated on valbenazine (40 mg every night at bedtime (QHS) for 1 week, discontinued for 1 week due to lack of insurance, then resumed at 80 mg QHS) in April 2018. No other changes were made to the medication regimen at the time of valbenazine initiation. After 2 months of valbenazine treatment, the patient had no tongue protrusion or chewing movements and was able to close his mouth, which led to decreased drooling and improved swallowing. His eye blinking was less frequent and less pronounced, and he had minimal shoulder/hand/finger movements and no foot tapping. The decrease in abnormal movements led to improved stability and posture such that he could stand and ambulate with a walker (). Additionally, he was able to feed himself independently and was more interactive with caregivers. The patient remains stable on valbenazine (80 mg QHS) with no changes to his medication regimen and no reported adverse events ().


A 63-year-old female with mild ID who lives at home with caregivers had a history of multiple significant comorbid diagnoses, including type 2 diabetes, fibromyalgia, GERD, hyperlipidemia, hypertension, hyperthyroidism, overactive bladder, and neuropathy (). In addition, the patient was diagnosed with schizoaffective disorder, bipolar type, and had prior long-term exposure to the atypical antipsychotics, olanzapine (20 mg QD) and risperidone (3 mg QD).
TD symptoms were repetitive tongue protrusion, lip smacking, and chewing movements of the mouth/jaw that led to unclear speech and difficulty communicating. The patient also had constant bilateral arm/hand/finger movements which caused instability during ambulation and led to the need for wheelchair assistance (). Her abnormal movements, especially the tongue protrusions, had caused her to become very self-conscious and isolated. The exact duration of the TD symptoms is unknown, but the patient reported having them for at least 4 years. She was initiated on valbenazine (40 mg QHS for 1 week, then escalated to 80 mg QHS) in June 2017. At the same time that valbenazine was initiated, olanzapine and risperidone were discontinued due to parkinsonism and switched to asenapine (titrated over 2 weeks from 5 mg QD to 10 mg twice-daily (BID)).
Within 1 week of initiating valbenazine treatment, caregivers noted an improvement in TD symptoms and no adverse events. After 2 months of valbenazine treatment, the patient had minimal tongue protrusion and lip/jaw movement, resulting in clearer speech, and her arm/hand/finger movements were decreased, leading to improved stability during ambulation (). She no longer needed a wheelchair and needed less assistance for ADLs such as personal hygiene. She was less isolated and able to go to a restaurant for the first time in months and had started participating in family and church activities. The patient remains stable on valbenazine (80 mg QHS) with no changes to her medication regimen and no reported adverse events ().


A 28-year-old male with moderate ID who lives in a group home had a history of significant comorbid diagnoses, including autism spectrum disorder (ASD), hypertension, hypothyroidism, and GERD (). Psychiatric diagnoses included excoriation disorder and schizoaffective disorder, bipolar type. In addition, he was anxious, restless, irritable, easily provoked, and physically aggressive and had begun isolating himself from group activities. The patient's medication history included long-term exposure to quetiapine (200/400/200 mg once daily (QD)).
TD symptoms were intermittent chewing movement of the jaw, constant bilateral hand/arm movements (e.g., opening and closing of hands/fingers; finger tapping), repetitive bilateral foot tapping, and truncal rocking (). The duration of these symptoms is unknown. He was initiated on valbenazine (40 mg QHS for 1 week, then escalated to 80 mg QHS) in February 2018. Due to persistent psychosis, the patient was also tapered off quetiapine and started on clozapine (titrated over 2 weeks to 300 mg daily).
Four days after starting valbenazine treatment, improvement in abnormal movements was noted. After 2 months of valbenazine treatment, the patient had no abnormal jaw movements, minimal to no arm/hand/finger movements or foot tapping, and no truncal movements (). Caregivers reported that the improvements in movements seemed to result in less isolating, aggressive, and confrontative behavior. He was also more cooperative, was less resistant to caregiver assistance with ADLs, and had increased participation in group home activities. The patient remains stable on valbenazine (80 mg QHS) with no changes to his medication regimen and no reported adverse events.


A 61-year-old male with moderate ID who lives in a large group home had a history of multiple significant comorbid diagnoses, including type 2 diabetes, enuresis, hyperlipidemia, hypertension, hypothyroidism, GERD, glaucoma, and tachycardia (). Psychiatric diagnoses included major depressive disorder, impulse control disorder, obsessive-compulsive disorder, and schizophrenia (paranoid type), for which he had received long-term treatment with multiple typical antipsychotics (chlorpromazine (dose, duration unknown), haloperidol (dose, duration unknown), and thioridazine (dose, duration unknown)) and atypical antipsychotics (clozapine (dose, duration unknown), quetiapine (600 mg QD, duration unknown), and risperidone (25 mg BID, duration unknown)) and was currently receiving treatment with clozapine (50 mg BID and 100 mg QHS).
TD symptoms included intermittent tongue thrusting, chewing motion of the jaw, facial grimacing, and nodding/forward movement of head and neck. In addition, he had frequent eye blinking and constant bilateral hand movement (). Prior to the onset of his TD symptoms, the patient had been very social and active and was voted “citizen of the year” in his group home, but as his TD symptoms progressed, he had become very agitated, irritable, impatient, and isolated and had stopped participating in group activities. Valbenazine treatment (40 mg QHS for 1 week, then escalated to 80 mg QHS) was initiated in October 2017. No other changes were made to the medication regimen.
Two weeks after starting treatment with valbenazine, abnormal facial movements had improved, and 1 month after initiation, there was no tongue thrusting or chewing motion, minimal facial grimacing and head/neck movement, and normal eye blinking. By 2 months, there were no abnormal hand movements, and he began integrating back into group activities (). The patient remains stable on valbenazine (80 mg QHS) with no other medication changes and no reported adverse events.


A 45-year-old male with mild ID who lives at home with caregivers had a history of significant comorbid diagnoses of hepatitis C, seizures, chronic obstructive pulmonary disease, and paranoid schizophrenia (). His medication history included prior exposure to the typical antipsychotic, haloperidol (dose, duration unknown), and multiple atypical antipsychotics (aripiprazole, brexpiprazole, olanzapine, quetiapine, risperidone, and ziprasidone (all doses, durations unknown)) and current treatment with clozapine (350 mg daily).
TD symptoms were throughout all body areas, including intermittent lip puckering, jaw movements, facial grimacing, and truncal rocking, as well as constant bilateral shoulder, arm, and hand movement, including piano-playing movements of the fingers (). His abnormal movements had escalated over the past few months to the point that he had become unable to ambulate and needed wheelchair assistance as well as caregiver assistance with most of his ADLs. In addition, he had become increasingly anxious and hopeless, and had expressed suicidal thoughts. The patient was initiated on valbenazine (40 mg QD for 1 week, then escalated to 80 mg QD) in October 2018. No other changes were made to the medication regimen.
Four days after starting valbenazine treatment, there was a noticeable decrease in abnormal movements, and he was able to ambulate independently with a walker. Two weeks after starting treatment, there were almost no abnormal movements. After 3 weeks of valbenazine, the patient was able to walk independently with a walker, was more independent with his ADLs, and appeared more organized in speech (). (80 mg QHS) with no changes to other medications and no reported adverse events.


A seventy-two-year-old female, a known case of Type 2 Diabetes, hypertension, and subclinical hyperthyroidism, presented with jaundice associated with dark urine, pale stool, fatigue, and poor appetite for 2 weeks. She did not complain of fever, abdominal pain, or vomiting. She also denied itchiness, hematemesis, melena, or significant weight loss. Her surgical history was unremarkable. She was compliant with her diabetic medications, ie, metformin and Sitagliptin. Two weeks before the presentation, the patient was seen in primary healthcare with mild dyspepsia for 1 month. She tested negative for Helicobacter Pylori was prescribed omeprazole 20 mg daily for 4 weeks, to which she was compliant. She had no prior gastric complaints and had not taken proton pump inhibitors before.
The patient did not have a history of smoking or alcohol intake and had no known allergies. She was not taking any herbal or over the counter medicines. She denied a family history of liver or biliary disease and malignancies. None of her other family members had similar symptoms. She did not have any recent travel outside Qatar.
On physical exam, she was afebrile (36.7°C), with a blood pressure of 142/79 mm Hg, pulse rate 88 beats/minute, respiratory rate 16 breaths/minute, and oxygen saturation 97% on room air. She had yellow discoloration (jaundice) of her eyes. There was no lymphadenopathy or organomegaly, and the rest of the system examinations were unremarkable.
Basic labs revealed a raised total and direct bilirubin, raised ALP, ALT, and AST (Table ). Complete blood profile, renal function, serum electrolytes, including sodium, potassium, calcium, magnesium, and INR, were normal (Table ). Ultrasound of the abdomen showed a 13 cm liver with normal echotexture. No lesions or Intra Hepatic Biliary Radicle dilatation were noted. The common bile duct was 2.6 mm. Hepatitis A and E IgM antibodies, hepatitis B surface antigen, and anti-hepatitis C antibody, anti – HIV antibodies were negative. Additionally, CMV and EBV PCR were negative. The patient's biochemistry was favoring the possibility of AIH vs DILI. The patient's medications were carefully reviewed. As omeprazole was the only medicine recently started, and with some scarce evidence in the literature, it was suspected to be the culprit drug and was thus discontinued on day 3.
As the patient's liver enzymes showed a mixed picture of cholestasis and hepatitis without any obvious obstruction of the biliary tract, an MRCP was arranged. Pancreas and biliary tree were unremarkable on MRCP, with no evidence of any underlying neoplastic process. However, there was periportal edema and trace of perihepatic free fluid, suggestive of diffuse liver disease.
On day four, the AIH work up was back. ANA and ASMA were positive with titers of 1:160 (nucleolar pattern) and 1:80, respectively. IgG (22, NR 7-16 gm/L) and IgG subclass 1(1370, NR 405-1011 mg/dL) were raised. The rest of the AIH workup, including AMA, ALKM, ANCA, APLAR-2 AB, anti – MPO, and anti-PR – 3, was unremarkable.
Although the liver enzymes were slowly declining (Table ) but were still considerably high; hence on day 10, an ultrasound-guided liver biopsy was done. The histopathology (Figure ) revealed confluent zone 3 necrosis with moderate portal tract inflammation and focal lobular inflammation: features highly suggestive of DILI, and not entirely compatible with autoimmune hepatitis.
The patient's liver enzymes kept trending down during her hospital stay. Her condition did not require steroid treatment. Gradually her jaundice improved, and the patient was discharged with a follow-up in medicine and gastroenterology clinic.
The patient was seen in an acute medicine clinic for follow-up after 1 month. She had no residual jaundice, and liver enzymes had returned to normal (Table ). A repeated ANA, ASMA, and ALKM titer were negative, suggesting the diagnosis as AIH-DILI secondary to omeprazole.


The case report was approved by the ethics committee of Zhongshan Hospital affiliated to Fudan University.
A 67-year-old man presented to the hospital with severe anemia. He had been diagnosed with cardia telangiectasias by gastroendoscopy and treated with blood transfusions, proton pump inhibitors, iron supplement, hemocoagulase, octreotide, thalidomide during the past 5 years by several hospitals. However, the symptoms of dizziness and fatigue occurred repeatedly with blood tests showing severe iron-deficient anemia and hemoglobin levels less than 60 g/L. He had melena intermittently, insomnia, but no symptoms of epistaxis, hematemesis, dysphagia, arthralgia, and Raynaud’s phenomenon. He had no history of hypertension, diabetes, and coronary heart disease, and no history of any nonsteroidal anti-inflammatory drug (NSAID) use. He had a previous history of dysentery at teenager but no hepatitis and tuberculosis, and other infectious diseases. He denied smoking and drinking habits. He also denied a family history of genetic diseases and infectious diseases. He worked as a facility administrator and retired seven years before the presentation and had no history of chemical exposure.
At presentation, his vitals were within normal limits with a body temperature of 36.9°C, pulse rate at 75 beats per minute, and systolic/diastolic blood pressure 122/82 mm Hg. He was noticeably pale and appeared fatigued. He had no signs of jaundice, cutaneous telangiectasias, spider nevi, or Raynaud's phenomenon. Cardiovascular, respiratory, abdominal, and nervous system examinations were unremarkable. Laboratory tests, including blood routine and biochemistry, are shown in Table , indicating hypochromic microcytic anemia and mitochondria antibody specific for PBC positive.
Telangiectasias at the cardia of the stomach were found under gastroscope (Figure ). Esophageal mucosa was normal. Endoscopic therapy using argon plasma coagulation (APC) was first considered for treating the patients, but the operation was canceled by a suspicion that the patient may suffer from a potential chronic liver disease.
Previous liver function tests (LFTs) of this patient only recorded mild increases of γ-glutamyl transferase (GGT). A re-examination of his LFT showed a normal serum bilirubin level, mild hypoalbuminemia (35g/L), a pattern of cholestatic liver biochemistry noted with increased activities of alanine aminotransferase (ALT) 78U/L, aspartate aminotransferase (AST) 69U/L, alkaline phosphatase (ALP) 205U/L, and GGT 270 U/L. He also had a mild prolonged prothrombin time (13.2 seconds) and a lower platelet count (102 × 10^9/L). Hepatitis B surface antigen (HBsAg) and hepatitis C antibody (HCV-Ab) were negative. Positive results were found in testing antinuclear antibody (ANA) (cytoplasmic speckled pattern 1:1000), antimitochondrial antibody (AMA), and antimitochondrial M2 subtype antibody (AMA-M2). FibroScan showed a liver stiffness of 9.6kPa. Computed tomography angiogram (CTA) of hepatic artery, portal vein, hepatic vein, and inferior vena cava showed mild liver cirrhosis with splenomegaly, without significant abnormality in portal vein system, hepatic vein, and inferior vena cava.
Ursodeoxycholic acid (UDCA, 250 mg PO twice daily) was then prescribed to the patient after a definite diagnosis of primary biliary cirrhosis (PBC) was established. Liver function was improved in months after UDCA treatment, but the amelioration of anemia was only achieved by a further administration of carvedilol (Figure ). The dosage of carvedilol started with 5 mg once a day as the patient had severe anemia at the beginning. The drug was prescribed as half a tablet (=5 mg) per day first, with blood pressure and pulse rate monitored. The dosage increased to tolerable 5 mg twice daily in months to maintain systolic/diastolic arterial blood pressure around 100 ~ 110/60 ~ 70 mm Hg and resting heart rate 60 ~ 65 beats per minute. The patient had been following up for one year with good compliance. His hematocrit remained stable at 9 ~ 11g/L, and he required no additional blood transfusions.


A 85-year-old female patient was admitted to hospital due to respiratory failure. Her clinical history included AF, congestive heart failure (CHF), type 2 diabetes mellitus (T2DM), arterial hypertension, obesity, hypothyroidism, and venous insufficiency of the lower limbs. The therapy included the initiation of IV methylprednisolone and the replacement of home ACT (warfarin used to treat AF) by enoxaparin (with dose adjustment for the renal function): 80 mg, SC, qd, during 8 days, followed by a dose increase to 80 mg, bid, for 6 days, and a further dose reduction to 80 mg, SC, qd, until the day of discharge to another hospital. Twelve days after initiation of therapy with enoxaparin, a phlebotomy was performed to extract 200 cc of blood due to high hemoglobin (Hb) count (19.8 g/dL), and the subsequent arterial blood gas (ABG) analysis showed Hb = 16.3 g/dL. On the following day, the patient presented Hb = 12.5 g/dL, hypotension, acute renal failure, and hyperlactacidaemia; she also had large hematomas on both the abdominal wall and the right thigh. An abdominal CT scan showed an active hemorrhage, and RSH was diagnosed (Figure ). Hypotension was nonresponsive to fluid resuscitation (systolic blood pressure: 60 mm Hg). The patient progressed to hemorrhagic shock. Blood transfusions were performed, and she was further transferred to the department of surgery of another hospital for embolization of bleeding arteries. The patient died a few days later (PT-INFARMED-B201805-241).


A 80-year-old female patient was admitted to hospital due to infected bronchiectasis. Treatment with oxygen (O2), acetylcysteine, ipratropium bromide, antibiotics, and corticosteroids (oral prednisolone, 20 mg, qd) was initiated. Enoxaparin, 40 mg, SC, qd, was prescribed to prevent venous thromboembolism (VTE). Five days later, the patient presented abdominal pain and a large palpable mass in the right hypochondrium and flank. There was no hemodynamic rebound, but there was a drop of 1 g/L in the levels of Hb. An abdominal ultrasound showed a right-sided RSH (Figure )—diffuse wall thickening from the upper to the lower insertion of the rectus abdominis muscle, with several internal liquid loci, within which solid material was observed, and with “fresh” and organized areas. The approach was based on conservative measures that included discontinuation of enoxaparin, analgesics, and ice packs. The patient recovered within 1 day (PT-INFARMED-B201805-262).


A 81-year-old male patient was admitted to hospital due to Influenza B virus-associated bronchopneumonia. The clinical history of the patient included CHF (biological prosthesis for aortic valve), CKD (estimated glomerular filtration rate (eGFR) <15 mL/min), type I respiratory failure, AF, and thrombocytopenia. Treatment with cefuroxime, oseltamivir, and IV methylprednisolone was initiated. Enoxaparin, 60 mg, SC, qd, for 5 days and 20 mg, SC, qd, for 1 day, was given to replace home ACT (oral warfarin) to prevent thromboembolism in AF. Two days later, the patient presented a fixed, nonreducible abdominal wall swelling, without hemodynamic rebound. The ultrasound showed a left-sided RSH (95 × 48 × 92 mm) (Figure ). A conservative clinical approach was adopted, including discontinuation of enoxaparin, blood and plasma transfusion and vigilance. A CT scan showed that the hematoma was sustained by the aponeurosis in the lower extremity of the left rectus abdominis. The rate of decrease in the level of Hb was slow, and therefore, conservative treatment was maintained. The patient recovered and was discharged 9 days later (PT-INFARMED-B201805-940).


A 67-year-old male patient was admitted to hospital due to pneumonia. Treatment with O2, antibiotics, and salbutamol + ipratropium bromide was initiated. Enoxaparin, 80 mg, SC, bid, was given to replace home ACT (oral dabigatran etexilate, 110 mg, bid) due to AF. Four days later, the patient presented abdominal pain in the right hypochondrium, nausea without vomiting, and a large palpable mass in the right quadrants. There was a drop in the levels of Hb of 4 g/L. A CT scan showed a large hematoma (19 × 15 × 8 cm) with small foci of active bleeding in the upper portion, aneurysmal dilatation of the abdominal aorta with a maximum caliber of 58 mm over a length of 77 mm, and a parietal thrombus (maximum 25 mm) (Figure ). Despite treatment, the patient progressed to hemorrhagic shock. Embolization of bleeding arteries and transfusion of 2 units of packed red blood cells were performed, two suction drains were placed in the hematoma cavities, and the patient was transferred to the intensive care unit (ICU). The patient initially recovered, but later developed hospital-acquired pneumonia associated with invasive intubation/ ventilation and died 13 days later (PT-INFARMED-B201806-781).


A 72-year-old female patient was admitted to hospital due to DVT. Treatment with enoxaparin, 60 mg, SC, bid, was initiated. The patient was also on oral fluconazole (100 mg, qd) and 5 days later initiated warfarin (2.5 mg, qd). Eight days after having initiated enoxaparin, the patient had pain in the inguinal region, with further worsening, involving the iliac fossa and swelling. She was hemodynamically stable, but there was a decrease of 2 g/L in the levels of Hb and the international normalized ratio (INR) was estimated at 1.7. The abdominal ultrasound showed RSH (194 × 119 × 102 mm), later confirmed by CT scan (Figure ). After contrast injection, denser intraluminal foci were observed, indicating active bleeding, but it was not possible to identify the bleeding vessel. There was also a marked densification of the peripheral muscle planes, probably due to hematic infiltration and surrounding effusion, and a small intraperitoneal effusion in the pelvic and sub-hepatic cavity. Blood transfusion was performed. The patient was transferred to another hospital to perform embolization and recovered within 4 days (PT-INFARMED-B201905-992).


A 50-year-old Caucasian man, residing in an urban area, with right laterality and no history of any chronic illnesses, was admitted to our Neurology department with the complaint of weakness in the right limbs. The patient's symptoms had an acute onset 2 days prior to presentation. Family history revealed that the patient's mother suffered from an ischemic stroke at the age of 87. The patient was an artist and a painter, admitted to being a cigarette smoker (1 pack of cigarettes per day for over 30 years) and to consuming alcohol daily in the last 10 years (about 50 cmc of spirits per day equivalent to 2 units of alcohol per day). There was no history of head trauma or any known allergies.
The general examination revealed that the patient was conscious and aware and had normal body temperature, no signs of recent trauma, and a BMI (Body Mass Index) of 21 kg/m2. The blood pressure was 234/146 mm Hg, and the heart rate 104 beats per minute.
The neurological examination showed the following:
Pyramidal tract signs characterized by hemiparesis regarding the right limbs with a score of 4/5 (on the MRC—Modified Research Council scale). Extensor plantar reflex was objectified in the right leg. The patient also had central face palsy on the same side. Extrapyramidal signs characterized by slowness, left upper limb rigidity, hypomimia and a low-volume, monotonous speech. Mild cognitive impairment on MMSE testing (a score of 27/30) and on MOCA testing (25/30). The abilities affected in our patient were visuospatial/executive functions, short-term memory, and mathematical functions.
On admission a head CT without contrast dye was performed (Figure A-E). The CT revealed a small hypodense lesion (Figure B), with ill-defined borders, located in the posterior limb of the left inner capsule that was interpreted as an acute lacunar stroke.
Diffuse white matter hypodensity was observed, with symmetrical pattern, regarding the periventricular region, the centrum semiovale, and the inner capsule. The juxtacortical arcuate fibers were spared. The hypodense white matter lesions extended in the brainstem, cerebellar peduncles, and the cerebellum.
For a more precise evaluation of the white matter changes, a native MRI was performed (Figure A-F).
MRI revealed an acute ischemic lesion (10/19 mm), hyperintense on the DWI, FLAIR, and T2WI sequences (Figure A,B), located in the posterior limb of the inner capsule that extended to the lenticular nucleus on the left. Diffuse hyperintense areas on T2WI and FLAIR sequences were also observed in the white matter, with the same distribution (supratentorial and infratentorial) as the hypodense areas described on head CT (Figure A-D), without abnormal restricted diffusion (Figure B). On the SWI sequences, conglomerate hypointense lesions adjacent to the midline of the pons ware observed (Figure F) that were interpreted as old hemosiderin deposits. Similar lesions were observed in the centrum semiovale (Figure E).
Complete bloodwork was done. No notable laboratory changes regarding the hematological profile, liver and kidney functions, and coagulation parameters were observed. No biological inflammatory syndrome was present on admission and during hospitalization. Other notable bloodwork parameters will be mentioned below in the differential diagnosis subchapter. ECG was within normal range.
A carotid Doppler ultrasonography was performed which showed bilateral nonstenotic atheromatous plaques, with heterogenous echogenicity, irregular surface, and a thickness ranging from 2.7 mm in the left common carotid artery to 4.2 mm in the right common carotid artery were observed.
A positive diagnosis of Binswanger's disease, lacunar stroke in posterior limb of the left inner capsule associated with pure motor hemiparesis regarding the right side of the body, and stage III arterial hypertension at presentation was established.
Using the imaging aspects of the disease on MRI, we decided taking into account for the differential diagnosis disorders which are compatible with the pattern of the white matter lesions as presented in the article written by Schiffmann et al These disorders where afterward excluded using clinical and biological findings.
CADASIL (Cerebral Autosomal Dominant Arteriopathy With Subcortical Infarcts and Leukoencephalopathy) usually has an earlier onset, during the third decade. The lack of findings in the family history of our patient, the absence of lesions on imaging anterior to the temporal horns of the lateral ventricles and of the migraines makes this diagnosis improbable. Also, the presence of vascular risk factors (arterial hypertension and smoking) plead for an acquired microangiopathy., Multifocal progressive leukoencephalopathy may present itself with similar imaging features to Binswanger's Disease, but cerebral lacunes should be absent 9. Characteristic lesions on MRI are more often localized at the gray-white matter junction with damage to the juxtacortical association fibers. On clinical examination, motor coordination and gait disturbances are frequently found alongside visual field deficits and language abnormalities. A normal hematological and immunological profile and the absence of opportunistic infections and other infectious events in the past that would suggest an acquired or intrinsic immunodeficiency are also favorable for excluding this diagnosis. Cerebral vasculitis as part of a systemic disorder may have various features on MRI imaging, and the clinical and biological features of the disease include: signs of a systemic disease, headaches, elevated acute-phase reactants, inflammatory microcytic anemia, and the presence of autoantibodies. None of these changes were found in our patient. Antinuclear antibodies, antineutrophil cytoplasmic antibodies, rheumatoid factor, and circulating immune complexes were all in normal range. Primary angiitis of the central nervous system (PACNS) is rare disorder and should be suspected in younger patients with strokes, without any known vascular risk factors. The major symptoms of this disease include headaches (60% of cases), cognitive impairment (50% of cases), and neurological focal signs. Systemic and biological changes that were described in the cases of systemic vasculitis are often absent. On MRI, the lesions are typically multifocal, bilateral, hyperintense on T2WI and FLAIR sequences, and can be found in the superficial and deep white matter, and in the cortical gray matter and basal ganglia. Of cardinal importance are the bilateral stenosis and dilation of various sized blood vessels which were absent in our patient. The gold standard for diagnosing or excluding PACNS is represented by cerebral biopsy, which we preferred not to perform due to lack of sufficient arguments that would justify the risk of the investigation. Toxic leukoencephalopathy develops in the context of chronic exposure to leukotoxic substances such as some illicit drugs (opioids, cocaine, amphetamines). Leukoencephalopathy caused by use of heroin, also known as "chasing the dragon" syndrome, is one of the most studied of these rare occurrences, and it usually presents on MRI with white matter hyperintensities that are symmetrical, especially with changes in the corticospinal tracts of the pons, perirolandic white matter, cerebellar white matter with sparing of the juxtacortical white matter and the gray matter., These lesions are similar to those seen in our case. The patient denied any use of illicit substances, did not have any signs of chronic toxicity of the substances mentioned above, and did not develop any withdrawal events during the 2-week hospitalization period. Toxic leukoencephalopathy may also develop after chronic exposure to toluene. Our patient was a painter and toluene can be found in many paints and solvents. The main neurological feature of chronic toluene toxicity is the cognitive impairment. The patient refused to give any information about the ingredients of the paints used by him. Nevertheless, the presence of other lesions associated on MRI, other than the white matter hyperintensities, are not typical in chronic toluene toxicity. There were also no other systemic disturbances that would suggest chronic toluene toxicity (altered kidney function, respiratory, dermatological, gastrointestinal, or musculoskeletal abnormalities). HIV-associated dementia is associated with a symmetrical, periventricular pattern of changes to the white matter with sparing of the juxtacortical and infratentorial white matter, atrophy of the gray matter of the cerebral cortex, atrophy of the deep white matter and volumetric changes of the basal ganglia, and changes that were not present in our patient. Also, the lack of criteria that would suggest and acquired immunodeficiency as mentioned previously makes this diagnostic much less probable. Other illnesses that have a genetic etiology are usually detected during childhood or adolescence and are frequently associated with systemic abnormalities. Out of these pathologies, Fabry disease is still worth mentioning. There are some rare cases described in literature, in men, that had a late-onset at adult age and in which only one or two organ systems were affected. The kidneys (chronic kidney disease) and the cardiovascular system (hypertrophic cardiomyopathy, valvular anomalies, conduction disorders) were most frequently affected., Our patient had no clinical or paraclinical evidence of a kidney or heart abnormalities. When present, central nervous system affliction leads to frequent ischemic strokes and cognitive impairment. On MRI, white matter lesions are present in 80% of cases and can be heterogenous, ranging from small, scattered, and punctuate T2-weighted hyperintense foci to bilateral diffuse, patchy, and partly confluent white matter hyperintensities. An important characteristic is that the lesions in Fabry disease only present themselves in the supratentorial white matter, which was not the case in our patient.
The therapeutic management targeted lowering of blood pressure in accordance with the actual guidelines and secondary stroke prevention using antiplatelet monotherapy and statins. Blood pressure was especially difficult to control requiring multiple associations of anti-hypertensive drugs. Rosuvastatin 20 mg per day and clopidogrel 75 mg per day were started in the first 24 hours. Urapidil iv was used to control the blood pressure during the first 48 hours. We started with 3 doses of 12.5 mg each at 10-minute intervals followed by continuous infusion at a variable rate (10-30 mg per hour) in order to slowly reduce and maintain the blood pressure at 180/110 in the first 24 hours. Indapamide 1.5 mg per day, Perindopril 5 mg per day, and Amlodipine 5 mg per day were also added, and the dosages were adjusted in order to maintain the blood pressure at 130-140/80-90 mm Hg during the following weeks. The patient was also evaluated in the first 72 hours after admission by a kinetotherapist, and daily sessions of active and passive mobilization were performed. The patient was referred to a cardiologist after discharge.
Regarding the clinical and neurological evolution, the motor deficit, fine motor control, and prehension improved during admission and during the first month after discharge from hospital.


A 48-year-old woman presented with esthetic concerns regarding her smile, which were attributed to a high smile line, slight chipping of her porcelain crowns. The patient had noticed these problems for approximately 1 year. Tooth mobility was confirmed upon intraoral examination, and a probing depth of 9 mm was recorded at the mesiopalatal side of tooth 11. Additional investigations were subsequently performed. A small vertical bone defect was observed at the mesiopalatal side of tooth 11 on a cone-beam computed tomography scan and a periapical radiograph. Areas of cementum and dentin resorption were found, in addition to adjacent bone loss. The pulp chamber was also enlarged at the cervical region. Tooth 11 was nonresponsive to pulp vitality testing (Figure ).
After evaluation of the local factors and considering the fact that the clinical symptoms had been evident for more than 1 year, a treatment plan was proposed for the flapless extraction of tooth 11, followed by immediate implant placement with instant provisionalization.
Full-face photography was performed before and after implant placement. Digital scanning was conducted, and a single mock-up defining the new incisal edge position was made for tooth 11. All data were transferred to an online-based digital laboratory (Figure ).
We used an online-based smile design software (Rebel Simplicity; Visagismlile Ltd.) to create 2D digital designs of the restorations, which reflected the patient's esthetic preferences and expectations. The 2D designs were used to guide the complete rendering of the 3D digital model for subsequent printing. Once the positions of the incisal edges of the incisors were defined, we created new 3D digital designs for the teeth proportions and extended the length of the teeth in the coronal and apical directions (Figure ). The single mock-up for the incisors was used as a reference for the automatic creation of a complete 3D digital mock-up (on STL files) for the subsequent printing of a resin model.
A silicon index was made from the printed model, and a new mock-up for clinical try-in was fabricated with bis-acryl resin (Figure ). After the patient's approval of the mock-up, preoperative antibiotics were administered (2 hours prior to the surgery; Augmentin 1000 mg), and tooth 11 was extracted under local anesthesia. As the vestibular cortical bone was observed to be thin and fenestrated during the implant procedure, a full mucoperiosteal flap was made with one vertical-releasing incision between the right central and lateral incisors. A titanium dental implant (grade 4) with a diameter of 4.0 mm and a length of 10 mm was inserted with a torque of more than 45 Ncm to achieve a high primary stability (Naturall+; NICP 40 100; Euroteknika). A xenograft material (Gen-Os; Osteobiol®) was applied to fill the space between the implant surface and the facial wall, in order to prevent bone alteration during the healing process. A collagen membrane (Evolution, Osteobiol®) was applied to promote wound healing and regeneration (Figures , , , ), and the flap was adapted and sutured. Postoperative antibiotics (1000 mg Augmentin, GSK, twice daily for 7 days), as well as a mouth rinse (0.1% chlorhexidine, Eludril Classic, twice daily for 14 days), were prescribed.
Due to the sufficient primary stability of the implant, a provisional implant-retained restoration was placed without immediate loading. The transitional zone of the crown was designed with a narrow and concave profile on the facial aspect and a slightly convex profile on the proximal surfaces to allow a greater volume of gingival tissue in the interproximal areas for support. Contact points with the adjacent teeth were tight and positioned 5-6 mm above the bone level.
At the 1-year follow-up, no soft-tissue inflammation or marginal bone loss was observed (Figures and ). All anterior teeth (from canine to canine) were prepared for lithium disilicate crowns, and a final impression (that captured the transitional zone at the implant site) was taken (Figure ). Upper and lower models were mounted in a semi-adjustable articulator, and the distance between the zenith points of the anterior teeth was measured to determine the clinical gingival contour. The design of the tooth preparations and zirconium abutments was guided by the digital mock-up (Figure ). The individual restorations for the prepared teeth and implant abutment were subsequently designed, and a wax model was milled from the CAD data. Final restorations were fabricated and tried in. All crowns were glazed following chair-side adjustments and cemented with a resin luting agent (RelyX™ Lutin Cement; 3M). We utilized a lithium disilicate crown for the zirconium abutment, as opposed to a screw-retained monolithic zirconium crown, in order to more closely match the optical characteristics of the adjacent restorations.


A 75-year-old female patient presented to tertiary referral academic hospital, Harare Central Hospital, from a provincial hospital with a 2 week history of pain and darkening of all her fingers and toes. A month prior to visiting hospital she developed a constant rest pain in her hands and feet. The pain was exacerbated by manual work and was not relieved by analgesia. She highlighted intermittent episodes of severe pain in her hands and feet occurring over the last 1 year. Her fingers and toes became dusky in appearance, and this was associated with blistering of the fingers and toes. The patient highlighted sensory loss in her fingers and toes. There was no history of intermittent claudication. The patient did not drink alcohol or smoke, and she had no known chronic illnesses. There was no family history of diabetes mellitus, hypertension, heart disease, dyslipidemias, and connective tissue disorders. The patient did not admit to a history of eating mouldy grain or bread. In the review of systems, she did not have any history of chest pain or palpitations. The patient did not have any history of headaches, seizures, or hallucinations. The past medical history was insignificant. She was negative for human immunodeficiency virus (HIV) and was not on any medications.
Examination of the patient at the referral center revealed she was fully conscious, and not in any apparent distress. She had a normal pulse rate of 90 beats per minute, with a normal blood pressure (systolic reading of 128 mm Hg and diastolic of 59 mm Hg). She had a pyrexia of 37.8°C with no lymphadenopathy. There were no Janeway lesions or Xanthoma on examination. The cardiac, respiratory, and abdominal examinations were normal. On examination of the hands, the patient had dry gangrene of all the fingers of the hands which had demarcated. She had very good peripheral pulses in the upper limbs. In the lower limbs, she had dusky hyperpigmented toes. All the peripheral pulses in the lower limb were present and full volume. See Figure
The complete blood count on admission showed a raised white cell count of 16.1 × 109/L, with a low hemoglobin of 11.6 g/dL. Her renal function and lipid profile were normal. A random blood sugar done on admission was within normal limits. A chest x-ray and electrocardiogram (ECG) were done and were normal. An echocardiogram (Echo) was done as part of her workup, and she had a structurally normal heart with no thrombi and a good ejection fraction of 67%.
Fluid resuscitation was commenced with intravenous crystalloid solution and empiric antibiotics therapy with her pyrexia settling on the third day after admission. Her toes demarcated as dry gangrene by day 3 postadmission. The patient was consented for amputation of the gangrenous parts of her hands and feet. Amputation of the digits of her fingers was done with transmetartasal amputation of her feet done bilaterally. The surgery was uneventful. Two weeks after the initial surgery, the patient had split skin grafting to cover the bare amputation sites with excellent uptake of the grafts. See Figure . Pathological evaluation of amputated tissue did not report any vasculitis. There were microthrombi in the small vessels with sparing of the large caliber vessels.
She was discharged home 5 days after split skin grafting. The patient underwent physiotherapy and occupational therapy sessions at her local hospital to assist with rehabilitation and mobilization. On review 1 month later, all her wounds had healed and the patient was well with no complaints.


A 67-year-old man was referred to our hospital with a left dorsal lump, neglected for 2 years but having recently increased in size.
Clinical examination identified a huge dorsal mass of 25*22 cm. It was located opposite the left shoulder blade and it was moderately firm with ulcero-necrotic center fistulized to the skin, (Figure ). The patient had no further complaints.
MRI of the dorsal soft tissues showed a malignant tumor of 18.5*20 cm within the left para-vertebral muscles and invading the 5th and 6th ribs' posterior arch (Figure ). A chest CT scan revealed multiple pulmonary metastases. A biopsy was performed. Histology identified (Figures and ) epithelial malignant tumor proliferation, with high cell density and monotonic tumor cells arranged in compact trabeculae or acini, with polarized nuclei arranged radially around acinar lumens; and an abundant eosinophilic granular cytoplasm. Immunochemistry showed an intense and diffuse positivity of tumor cells for alpha-antitrypsin (acinic marker; Figure ), a moderate and heterogeneous staining with CD56 (neuroendocrine marker). Other markers such as synaptophysin, chromogranin A, CK19, CK7 were negative. Soft tissue metastasis from an ACC was confirmed.
An abdominal CT scan was performed and did not reveal any pancreatic lesion. MRI of face and neck to search for a salivary gland origin of the metastases was unremarkable.
Chemotherapy with Paclitaxel (175 mg/m2 every 3 weeks) and Carboplatin (AUC:5 according to Calvert formula, every 3 weeks) was indicated. After three cycles, the patient experienced disease progression. A second line of chemotherapy by FEC75 (5-fluorouracil at 500 mg/m2 [at day1], epirubicin 75 mg/m2 [at day1], Cyclophosphamide 500 mg/m2 [at day1], every 3 weeks) was administered. A locoregional and metastatic pulmonary progression were diagnosed after four cycles. The patient died 6 weeks later.


A 15-year-old girl was referred to a pediatrician due to repeated episodes of palpitations, dizziness, and presyncope (Table ). Seven months prior, she experienced chest discomfort and palpitations followed by syncope while walking. She had a history of post-traumatic stress syndrome but was free from pharmacological therapy. Family history with regard to cardiac disease was unremarkable. Twelve-lead ECG was normal except for frequent monomorphic PVCs (Figure ). Echocardiography ruled out structural heart disease. A 24-hour ambulatory ECG showed sinus rhythm with more than 20,000 monomorphic PVCs with left bundle branch block morphology in V1-V2 and electrical inferior frontal axis between 90° and 120°. During a bicycle exercise testing, the number of PVCs decreased when heart rate elevated. A Holter recording four months later revealed 86 episodes of monomorphic nonsustained VT (NSVT) with the same morphology as the PVCs in the initial Holter recording were detected. There were no late potentials in a signal-averaged ECG. The patient had no further syncope but continued to experience palpitations and metoprolol 100 mg once daily was started to alleviate symptoms. After this, she was lost to follow-up for four years due to social factors.
She was mainly asymptomatic during these years but at an age of 18 she experienced a single episode of palpitations during pregnancy. Her heart rate was irregular but no ECG was taken. A cesarean section was performed without complications. Two years later during her second pregnancy, she was referred to the cardiology clinic due to frequent palpitations. During a 24-hour ambulatory ECG monitoring, the longest NSVT was 12 beats at a rate of 200 beats per minute. After childbirth, she was assessed at a tertiary center with cardiac magnetic resonance that was normal except for an area that was 2 mm in diameter in the right ventricle with increased adipose tissue. This was deemed not diagnostic for arrhythmogenic right ventricular cardiomyopathy, and no arrhythmias were inducible in an electrophysiological study (EPS).
Palpitations continued despite beta-blocker therapy, and three years later, the patient received an insertable cardiac monitor. A few months later, she had recurrent syncope and several episodes of rapid NSVT up to 33 beats were revealed. EPS was repeated and a short run of VT arising from the RVOT was induced. This was not reproducible and ablation could not be performed. Instead, flecainide 100 mg twice daily and sertraline 50 mg once daily was initiated due to palpitations and anxiety, respectively. Arrhythmia symptoms were ameliorated by flecainide, but the medication had to be stopped due to visual adverse effects. Thereafter, the patient was referred to another tertiary center for a third EPS and ablation. However, 5 days later recurring NSVT returned. Intravenous amiodarone decreased the frequency and duration of the VTs, but the patients remained highly symptomatic, and she was transported with airborne ambulance back to the tertiary center for reablation. Despite several ablations in the RVOT, the arrhythmia did not stop and antiarrhythmic medication was reinitiated. Four months later, she presented to the emergency department with electrical storm due to repeated episodes of sustained monomorphic VT (Figure ), which terminated following intravenous amiodarone.
After washout of antiarrhythmic medications, a fifth EPS was performed with ablation in the RVOT but arrhythmia remained after the procedure. It was decided to refer the patient to a tertiary arrhythmia center in Finland. In the initial EPS in Tampere University Hospital, neither PVCs nor any VT was detected and no ablation was performed. However, during the following night the patients had frequent PVCs and multiple VTs. She was taken back for EPS in the next morning. Electroanatomical mapping using remote magnetic navigation revealed slightly earlier activation in the aortic root between the right and noncoronary cusp than in the RVOT area. Pace map in this site was excellent. Following ablation in this site, VT stopped but the PVCs did not disappear despite multiple ablations. Mapping of the distal coronary sinus and left ventricular outflow tract was not successful, whereas remapping of the RVOT revealed early activation and good pace map. Upon ablation in the RVOT (Figure ), PVCs disappeared and no VT was inducible at programmed ventricular stimulation. The patient was discharged two days later with no symptoms. During the seven-year follow-up period after the last ablation, she has had sporadic PVCs from another focus but no sustained or nonsustained VT has been detected by the insertable cardiac monitor or by ECG.


A 72-year-old man was admitted to a local hospital with complaints of general fatigue and body weight loss. A computed tomography (CT) scan of his chest was performed and showed lymphadenopathy in his whole body, including axillar, hilar, mediastinal, and retroperitoneal regions (). The patient was suspected of having malignant lymphoma and underwent excisional biopsy in his right groin. One week after the biopsy, he suddenly presented with a complaint of acute abdominal pain. An emergency contrast enhanced CT scan was performed and revealed a massive retroperitoneal hematoma. Consequently, he was transferred to our hospital for the suspicion of ruptured abdominal aortic aneurysm.
Upon arrival, he was drowsy, pale, and complained of progressively worsening abdominal pain. His blood pressure was 90/50 mmHg, the pulse rate was 110 beats/min, the hemoglobin was 10.0 g/dL, and the oxygen saturation was 99%. He had no past history of hypertension. A contrast enhanced CT scan was performed repeatedly and revealed a massive retroperitoneal hematoma with active extravasation arising from the infrarenal abdominal aorta coexisting with an extensive retroperitoneal mass surrounding the aorta (). The aorta appeared to be almost of normal caliber, with slight expansion. Radiologic interpretations by a specialist pointed out the density difference between the hematoma and the periaortic soft tissue in the retroperitoneal lesion. As a result, we suspected abdominal aortic rupture caused by invasion by the malignant lymphoma and decided to perform an emergency operation. Considering the risk of tight adhesion between the aorta and the lymphoid tissues, conventional open repair could have been technically difficult. Therefore, we chose to perform endovascular aneurysm repair (EVAR) as an alternative.
An emergency EVAR was performed under local anesthesia with mild sedation. Initial angiography confirmed an active extravasation arising from the infrarenal abdominal aorta (). An AFX® endoprosthesis (Endologix, Irvine, CA, USA) was placed below the origin of the bilateral renal arteries covering the ruptured aortic segment using standard techniques. In order to avoid type IA endoleak, another extension cuff was placed in the proximal region. Completion angiography showed the endoprosthesis was in a good position and no evidence of endoleak was observed ().
The patient’s postoperative course was uneventful and postoperative CT scan demonstrated complete sealing of the rupture point with no evidence of endoleak (). Five days after the treatment, histological examination of the previous biopsy confirmed the diagnosis of mantle cell lymphoma (MCL), which is one of several subtypes of B-cell non-Hodgkin lymphoma. Afterwards, the patient was transferred to a specialized hospital for the purpose of receiving chemotherapy for MCL.


The patient is a 61-year-old male with a history significant for NF-1, hypertension, esophagitis and intermittent gastrointestinal bleeding, who initially presented to an outside institution for acute onset of bleeding per rectum. The patient noted bloody bowel movements with diarrhea for several days, which had increased in frequency over the past 24 hours. The patient reported mild abdominal pain and denied nausea, emesis, chest pain, shortness of breath, syncope or other symptoms at the time. He was not on anticoagulation and denied excessive alcohol or nonsteroidal anti-inflammatory drug (NSAID) use. His family history was significant for NF-1 diagnosed in his mother and brother. He had no significant surgical history other than a total hip replacement.
On admission at the outside hospital, the patient’s hemoglobin was 6.6 g/dl, with normal platelets, partial thromboplastin time and prothrombin time. Diagnostic workup at the outside institution included a nuclear medicine gastrointestinal scan (mTc-labeled RBCs), which was negative for abnormal bleeding. On hospital Day 2, his hemoglobin failed to respond appropriately to transfusions and a computed tomography angiogram (CTA) was completed, which showed a 6.9 × 5.8 × 6.8 cm hyper-vascular mass in the left pelvis; abutting the left border of the bladder medially, iliac arteries and veins posteriorly, rectus sheath anteriorly and the small and large bowel (). Vascular supply of the mass was identified to originate from branches of the superior mesenteric artery (SMA) and appeared to be contiguous with the adjacent jejunum. The mass was noted to also abut, but not involve, the sigmoid colon. The CT scan further noted a 9 mm enhancing tumor in the second portion of the duodenum anteriorly (). After administration of five total units of packed red blood cells with limited improvement in hemoglobin, the patient was transferred to our institutions for further management.
The patient arrived hemodynamically stable and underwent esophagogastroduodenoscopy (EGD). The EGD was unremarkable with no visualization of the previously mentioned duodenal mass. Colonoscopy was performed, which was notable for bright red blood present throughout the entire colon as well as blood 5 cm proximal to the ileocecal valve (). As the patient continued to have bloody output per rectum, with insufficient response to transfusion, interventional radiology was consulted and proceeded with mesenteric angiography with intervention (). The hyper-vascular mass was found to be supplied by ileal branches of the SMA, which were successfully embolized (). After conversations with the interventional radiology team and the patient, the decision was made to proceed with an exploratory laparotomy and resection of the identified mass.
Exploratory laparotomy was notable for a 7 cm mass originating from the distal jejunum (). This mass was mostly exophytic and a single firing of a GIA load was used to excise the mass with preserved adjacent normal wall of the jejunum. Kocherization of the duodenum revealed a single sub-centimeter duodenal mass at D2. The mass was excised with subsequent primary closure of the defect. Taking down the Ligament of Treitz allowed us to identify two additional proximal jejunal lesions that were resected and resulting defects were primarily closed. The remainder of the jejunum was evaluated and a stretch of proximal jejunum, about 10 cm from Treitz, containing 10 small (0.5 to 2 cm) lesions was identified (). The decision was made to resect a 30 cm segment of jejunum with creation of a side-to-side functional end-to-end anastomosis using a linear stapler. This allowed complete resection of macroscopic disease. The mesenteric defect was closed using multiple interrupted silk sutures. The remainder of the bowel was examined and found to be normal. The patient returned to the intensive care unit and recovered over the course of the coming days without immediate complications and without requiring additional transfusions.
Five surgical specimens were sent to pathology, including the large intra-abdominal mass, a segment of proximal jejunum (30 cm), the duodenal lesion and two proximal jejunal lesions. Microscopic examination revealed a total of 13 foci of tumor. All tumor margins were negative and 12 regional lymph nodes were examined and revealed no evidence of nodal metastases. All tumors underwent H&E staining () and immunohistochemical staining for DOG-1 (), which was strongly positive, confirming a diagnosis of GIST for all tumors.
Genetic analysis of the tumors samples was performed on paraffin-embedded tissue using the Tempus XT next generation sequencing (NGS) platform, which includes 648 cancer-related genes. No KIT or PDGFRA mutations were detected (both were wild type). Instead, NF-1 was found to harbor a loss-of-function single nucleotide alteration (c.654 + 1G > T splice region variant).
The patient recovered appropriately from his surgery and was discharged one week after the operation. He continues to do well and has returned to full-time work.


A 16-year-old adolescent boy, trisomy 21 with multiple cardiac shunts (VSD, and PDA) and echocardiography-estimated RV systolic pressure 73 mm Hg plus right atrial pressure based on tricuspid regurgitation jet (). He was considered inoperable due to bidirectional shunting across VSD () by echocardiography and referred to our institution for evaluation. He underwent CMR-guided RHC and pulmonary vasodilator testing in the Phillips Ingenia 1.5 Tesla MR/catheter hybrid laboratory suit (). The hybrid Cath-CMR setting and iCMR procedural details at Children’s Medical Center, University of Texas Southwestern Medical Center, was described previously [].
Total pulmonary blood flow assessment was done by summing the flow in the right pulmonary artery (RPA) and the left pulmonary artery (LPA) distal to PDA entrance, the approach based on the assumption that total pulmonary flow can be more accurately quantified by measuring flow in each branch artery []. Cardiac output was calculated by measuring blood flow in the ascending aorta as previously described []. The total pulmonary blood flow was calculated using the measured RPA and LPA net antegrade blood flow volume []. A 6 French balloon wedge catheter was used to perform the CMR-guided RHC procedure. Using the Philips interactive scanning mode guidance (Philips Healthcare), the interventionalist advanced the gadolinium-filled balloon tip of the wedge catheter and MR-conditional guidewire (Emeryglide MRWire Nano4Imaging, Aachen, Germany) (). Magnetic resonance imaging provided excellent soft-tissue contrast for completing the right heart catheterization, including 3D reconstruction of the aortic arch, PDA, and branch pulmonary arteries ().
The hemodynamics and MRI results obtained in our patient are summarized in and . The pulmonary and systemic cardiac output was also measured by the traditional Fick’s principle method using presumed oxygen consumption, and catheter-measured oxygen saturations obtained at RHC. Based on the hemodynamic and pulmonary blood flow data obtained by traditional Fick’s principle-based calculations, indexed PVR (PVRi) was calculated to be 10.4 Wood unit × m2 at baseline and with vasodilator challenge (100% oxygen and 40 ppm nitric oxide) decreased to 9.4 Wood unit × m2 in the setting of multiple intracardiac shunts. However, the iCMR PVRi was calculated by dividing the catheter-measured transpulmonary pressure gradient by indexed pulmonary blood flow, calculated accurately by MRI blood flow data, taking into account the various pulmonary blood supply sources related to multilevel left-to-right shunting []. The iCMR PVRi was calculated to be 8.7 Wood unit × m2 at baseline and decreased to 6.8 Wood unit × m2 with vasodilator challenge. The RV size and systolic function were normal (EF 65%), and there was no evidence of scarring. The perimembranous VSD measured 22 × 17 mm with inlet extension to inlet muscular septum and left-to-right shunt. The PDA was large and measured 9 × 8 mm at its narrowest pulmonary end with a left-to-right shunt. Both the VSD and PDA shunt flow increased with vasodilator testing (). The trachea and main stem bronchi were normal while on positive pressure ventilation. There was mild posterior tracheal flattening. Lung parenchyma was normal. Because the PVRi was 6.8 Wood unit × m2 with vasodilator challenge by iCMR calculations, it was decided to proceed with surgery to close the VSD and PDA in our patient.


A 49-year-old woman presented to the hospital with a 20-day history of an enlarging painful mass on the left side of neck, fever, poor appetite, and weight loss of 5–6 kg since the start of her symptoms. She was a housewife from a semi-urban area and a known case of diabetes mellitus on medication, but with poorly controlled glycaemic status. She had no history of contact with tuberculosis. On examination, the neck mass was found to be located on the left upper third cervical region abutting left of the mandible, measuring approximately 5.5 × 4 cm, firm, and tender with local rise of temperature and overlying erythema. Vital signs were stable with a pulse rate of 98/min, regular respiratory rate of 22/min, blood pressure of 100/74 mm Hg, and temperature of 39.2 °C. She weighed 44 kg. Laboratory studies showed leucocytosis (total leucocyte count 13.9 × 109/L; 80% polymorphs, 16% lymphocytes, 2% eosinophils, 2% monocytes), mild thromobocytosis (platelet count 475 × 109/L), raised erythrocyte sedimentation rate (ESR) 60 mm/h, and low hemoglobin 101 g/L. Blood metabolic panel suggested poor diabetes control (random blood sugar 351 mg/dL, fasting blood glucose 202 mg/dL, postprandial blood sugar 382 mg/dL), with urea 28 mg/dL and creatinine 1mg/dL.
Contrast enhanced computed tomography (CECT) scan of the neck showed the presence of a large, heterogenous enhancing hypodense nodal mass in left cervical region (), likely to represent a pyogenic or tuberculous abscess. A fine needle aspirate of the pus sample from the mass showed sheets of neutrophils, degenerated cells, and lymphocytes in a dirty background with no granulomas, necrosis or atypical cells. Mycobacterial smear and culture were negative. Bacterial culture yielded an oxidase-positive gram-negative rod, with an unusual pattern of antibiotic resistance with resistance to colistin, polymyxin B and aminoglycosides. Blood culture was negative. The pus isolate was identified as Burkholderia pseudomallei by its characteristic growth on blood agar and MacConkey’s agar (small, smooth, nonlactose fermenting colonies after overnight incubation, which turned pink with a metallic sheen after 48 h and dry and wrinkled after 96 h of incubation), typical morphology on microscopy (motile, gram negative bacilli with bipolar staining giving a “safety-pin” appearance) and standard biochemical tests () [,,]. The isolate was sensitive to ceftazidime, co-trimoxazole, imipenem, meropenem, doxycycline and amoxicillin—clavulanate []. Minimum inhibitory concentration (MIC) of ceftazidime was 4 µg/mL by Etest (bioMérieux, Marcy l’Etoile, France).
Following the culture report, the patient underwent incision and drainage of the abscess mass and was commenced on intravenous (IV) ceftazidime administered as intensive therapy (2 gm 8-h) for a prolonged duration of three weeks followed by oral cotrimoxazole (Trimethoprim 240 mg and Sulfamethoxazole 1200 mg) twice daily as maintenance therapy. Glycemic control was optimized with insulin therapy. The fever subsided within a week of ceftazidime therapy followed by symptomatic healing of the neck lesion. The patient was discharged from the hospital on the 25th day of admission with advice to continue cotrimoxazole for six months. On follow-up two-and-a-half months later, complete healing of the lesion was noted.


A 27-year-old village-dwelling housewife presented with history of mild and continuous fever, cough without expectoration and left-sided neck swelling for one month. The lesion initially started as a small marble-sized tender nodule progressing to a full-fledged painful neck abscess within the next 25 days and started to rupture and discharge spontaneously with oozing of pus (a). Examination revealed the swelling to be about 3.0 × 2.0 cm, soft to firm, and tender, with a thick, white discharging pus. The patient was afebrile, had blood pressure 110/70 mmHg, heart rate 86/min, and respiratory rate 20/min. Laboratory investigations showed an extremely low hemoglobin level of 79 g/L and raised ESR of 80 mm/h. Other haematological and biochemical indices, including plasma glucose level, were within normal ranges. The discharging pus was negative for acid fast bacilli (AFB), but Gram stain showed eight to ten pus cells per oil immersion field (oif) with scanty gram-negative bacteria. Culture revealed growth of B. pseudomallei sensitive to ceftazidime, co-trimoxazole, meropenem, imipenem and doxycycline; MIC of ceftazidime was 4 µg/mL []. The patient was started on standard regimen of parenteral ceftazidime for three weeks followed by oral co-trimoxazole along with oral iron therapy for correction for anemia. At her latest follow-up four months after initial diagnosis of melioidosis, the lesion had healed without any residual clinical sign of infection (b).


A 30-year-old male, working as an office staff presented with history of swelling and pain in the right pre-auricular region of 20 days duration. He had difficulty in opening his mouth and also complained of fever since the last 10 days. Examination revealed a cystic, warm, fluctuant swelling approximately 30 × 20 mm in diameter, with induration and tenderness. Laboratory investigations showed hemoglobin level 151 g/L, total leucocyte count (TLC) 15.8 × 109/L; (55% polymorphs, 32% lymphocytes, 8% eosinophils, 5% monocytes), raised ESR 40 mm/h and random blood sugar level of 73 mg/dL. Non-contrast computed tomography (NCCT) revealed an irregular hypodense lesion of 43 × 22 × 23 mm diagnosed as a right parotid abscess. Approximately 5 mL of seropurulent aspirated fluid from the abscess received in the Microbiology Laboratory yielded a pure growth of B. pseudomallei. A direct Gram stain examination of the sample revealed more than 100 pus cells per oif with moderate number of gram-negative rods. The isolate was resistant to ceftazidime and amoxycillin-clavulanate, but susceptible to co-trimoxazole, imipenem, meropenem and doxycycline. In view of ceftazidime resistance, the patient was treated with parenteral meropenem for three weeks with advice of oral co-trimoxazole for six months along with incision and drainage of the abscess. Complete healing of the lesion was noted at a five-month follow-up period.


A 17-year-old female student was admitted in view of a progressively increasing right pre-auricular swelling since the last 7 days associated with pain and fever. She was found to be a resident of the same village as Case 3. Laboratory investigations showed hemoglobin level 115 g/L, TLC 31.1 × 109/L (85% polymorphs, 10% lymphocytes, 3% eosinophils, 2% monocytes), raised ESR 60 mm/h and random blood sugar level of 86 mg/dL. NCCT revealed a heterogenous hypodense lesion of 63 × 32 × 42 mm diagnosed as a right parotid abscess. Incision and drainage was performed and approximately 20 mL of frank pus drained which showed 40–50 pus cells per oif with plenty of gram-negative bacilli and yielded B. pseudomallei on culture. The patient was treated with the standard regimen of parenteral ceftazidime and upon discharge was advised to continue oral co-trimoxazole for a period of six months.


A 31-year-old female, presented with a warm, fluctuant left neck swelling for the last 15 days associated with pain and fever. Laboratory investigations revealed TLC of 20.6 × 109/L, raised ESR of 50 mm/h and random blood sugar level of 327 mg/dL. Gram stain examination of a pus aspirate from the abscess showed plenty (>100) of pus cells per oif and culture yielded pure growth of B. pseudomallei resistant to amoxycillin-clavulanate. Her lesion healed following incision-drainage and standard therapy for melioidosis with ceftazidime and co-trimoxazole.


A 35-year-old female had a swelling on left side of neck (approximately 60 mm × 50 mm in size, soft in consistency, erythematous and tender) for the last three months associated with evening rise of temperature. Following growth of B. pseudomallei in culture of the drained pus, the patient was started on standard therapy for meliodiosis and discharged.


An 18-year-old male presented with right neck abscess since the last 15 days associated with pain and fever. B. pseudomallei grown from the abscess pus showing 70–80 pus cells per oif with plenty of gram-negative bacilli was susceptible to ceftazidime, co-trimoxazole, imipenem, meropenem and doxycycline but resistant to amoxicillin-clavulanate. The lesion was completely healed after incision and drainage and therapy with ceftazidime.


A 53-year-old male, farmer by occupation, was admitted with history of fever and a spontaneously developing painful swelling on right scalp of one-month duration. The patient was a known diabetic and the random blood sugar level on presentation was 364 mg/dL. Other laboratory investigations revealed TLC of 16.9 × 109/L and raised ESR of 60 mm/h. A pus culture which on Gram stain showed plenty of pus cells and gram- negative rods grew B. pseudomallei in pure culture. Incision and drainage was done and the patient commenced on parenteral ceftazidime for three weeks. He was advised a 6-month course of oral co-trimoxazole upon discharge.


A 29-year-old gentleman with an illness-free past medical history presented with dyspnea. The patient was doing well throughout his life until a week before his presentation when he started to experience exertional dyspnea with moderate activities associated with atypical pricking chest pain. The patient was capable of lying flat, and his vitals were normal apart from an approximately blood pressure of 150/90 mmHg in all limbs. Physical examination revealed an elevated jugular venous pressure and a harsh systolic ejection murmur best heard at the left upper sternal border. Basic blood tests were normal; however, ECG exhibited a right bundle branch block (RBBB) and right ventricular hypertrophy (RVH) strain pattern (Figure ).
CXR was also obtained and revealed a normal appearance of the heart (Figure ).
An urgent echocardiogram for the patient showed a membranous ventricular septal defect (VSD) with a right to left shunt (Figure ), severe pulmonic valve stenosis with a presence of dynamic obstruction (Figure ), severe RVH with severe tricuspid valve regurgitation (Figure ), and overriding aorta (Figure ).
The cardiac magnetic resonance image (MRI) confirmed the echocardiogram findings. After establishing the diagnosis of ToF, the patient was referred to cardiac surgery for a repair. Total correction of ToF was executed, resulting in VSD closure with a pericardium patch, pulmonic valve replacement with bioprosthetic PERIMOUNT Magna valve in addition to annuloplasty with an MC3 ring, and an intentional patent foramen ovale was performed. The patient's symptoms improved after the corrective surgery, and he was discharged on perindopril, metoprolol, and aspirin. A five-month follow up echocardiogram demonstrated similar findings as to the previous study with a satisfying prosthetic pulmonary valve and non-leaking VSD closure (Figure ).
Two years afterward, the patient was doing well, not complaining of any symptoms, and maintaining regular daily activities without any inconvenience.


We report a case of a 20-year-old Hispanic female with a past medical history of rheumatoid arthritis (RA) and juvenile idiopathic arthritis (JIA) diagnosed at 18 years.
The patient presented to the emergency department with complaints of subjective fevers of four to six weeks duration, intermittent, and mostly occurring at night. Her fever was associated with a rash on bilateral hands with left wrist pain and swelling. The patient also complained of swollen lymph nodes bilaterally in her neck, axilla, and inguinal regions, along with unintentional weight loss of 20 pounds over two months associated with night sweats.
On physical examination, the patient was found to have a temperature of 102 degrees Celsius, heart rate (HR) 112 beats/min, blood pressure (BP) 110/70 mmHg, respiratory rate: 18/min. Her complete blood count revealed white blood cell count (WBC) of 2.3 x109/L (normal range: 4.5-11.0 x 109/L) with lymphocytes count of 1.2 x103/mm3, (normal range: 1,000-4,000/mm3) erythrocyte sedimentation rate (ESR): 110 mm/hr (normal range: 0-22 mm/hr), C-reactive protein (CRP): 80 mg/L (normal range: <10 mg/L). The patient’s HIV, hepatitis panel, EBV, and CMV results were all negative.
The computer tomography (CT) imaging of her chest, abdomen, and pelvis revealed cervical, mediastinal, and inguinal lymphadenopathy along with hepatomegaly, and splenomegaly (Figures -).
Based on history, physical examination, routine labs, and imaging studies, the initial working diagnosis was Hodgkin's disease. We decided to do an excisional cervical lymph node biopsy.
The excisional biopsy was consistent with KFD disease, demonstrating a necrotizing pattern, and the patient was subsequently started on a long course of steroids (Figures -). The patient initially responded well for a few months but had multiple relapses on steroids. Her repeat labs revealed persistent leukocytosis with high inflammatory markers like ESR and CRP. Her repeat CT scan showed an increase in the size of her cervical, mediastinal, and inguinal lymphadenopathy and an increase in the size of her spleen.
Based on these findings, the steroids were tapered and discontinued. The patient was subsequently started on the second line treatment drug, anakinra (interleukin-1 (IL-1)). She demonstrated good clinical response and has been in remission for three years now with the newer agent, anakinra.