A 33-year-old male had recently presented to another hospital in the area just prior to admission at our hospital for weakness, fatigue and bilateral hip and leg abscesses. At that time he elected to be treated for cellulitis as an outpatient and was prescribed cephalexin 250 mg by mouth four times daily and sulfamethoxazole-trimethoprim 400-80 mg by mouth twice daily for ten days. He then presented to our hospital two days later with a chief complaint of difficulty swallowing and progressive weakness which he states he had for one week. His past medical history included daily heroin use, administered by skin popping or injecting the drug under the skin either intradermally or subcutaneously, and untreated hepatitis C.On presentation to our hospital, the patient was only able to open his eyelids 2-3 mm wide and had difficulty keeping them open, diplopia, difficulty swallowing and pooling of secretions after 1-2 words, voice changes, difficulty holding his head up and proximal upper extremity weakness. Strength in his upper extremities were 4/5. Initial vital signs included: blood pressure 136/80 mmHg; pulse rate 106 beats/min; respiratory rate 19 breaths/min; oral temperature 36.5°C (97.7°F); and room air pulse oximetry 98%. Five deep cavity abscesses were incised and drained from the patient’s hips and thighs, and standard and anaerobic wound cultures were collected. After a few hours, the weakness became more pronounced in the patient’s neck and extremities and breathing became very shallow. The decision was made to intubate the patient in the emergency department for airway protection and impending respiratory failure before transferring him to the [medical intensive care unit (ICU)]. A urine drug screen was positive for amphetamines, opiates, and acetone.
The constellation of symptoms seen in the emergency department was suggestive of botulism, with other differential diagnoses including Guillain-Barré Syndrome and myasthenia gravis. He reported not having eaten canned food that had been bulging and had not traveled outside of the country recently. Therefore, the suspicion was for possible wound botulism. The Maricopa County Department of Public Health was contacted, and receipt of heptavalent botulinum antitoxin (A, B, C, D, E, F, G) –BAT® Equine was coordinated directly with the [Centers for Disease Control and Prevention (CDC)]. The antitoxin arrived by freight air transport the next morning on hospital day two and was administered via infusion approximately nineteen hours after presentation to the emergency department. Serum, stool and wound cultures were sent to the CDC for analysis. Wound cultures were also sent to the hospital’s lab for analysis. In addition, vancomycin and piperacillin-tazobactam, were started for broad antibiotic coverage including possible [methicillin-resistant Staphylococcus aureus (MRSA)].
In the medical ICU, the patient’s weakness plateaued and antibiotics were deescalated to vancomycin and ampicillinsulbactam for continued coverage of his complicated skin-soft tissue infection. After three days in the ICU (hospital day four), a tracheostomy was performed for the expected long recovery time and a PEG tube was inserted. The patient was then transitioned to oral antibiotics, amoxicillinclavulanate 875 mg by mouth twice daily and sulfamethoxazole-trimethoprim 800-160 mg, two tablets by mouth twice daily for two weeks. On hospital day seven, the anaerobic wound cultures analyzed by the hospital’s lab resulted with growth of Clostridium subterminale . On hospital day ten, results from the CDC serum analysis reported toxin A which was identified using MALDI-TOF mass spectrometry, the wound culture analyzed by the CDC did not report botulinum toxin, and the stool was not analyzed.
After a twelve-day hospital stay, the patient was discharged to a skilled nursing facility. He continued to have bilateral ptosis, difficulty swallowing and generalized weakness. He continued to have 4/5 bilateral deltoid muscle strength but 5/5 strength in all other major muscle groups. He had a tracheostomy placed on hospital day 5 and was being weaned off the ventilator at time of discharge.


A 67-year-old woman discovered that there had been a burglary in her daughter’s house.
Immediately she developed severe chest pain combined with shortness of breath and her husband called the emergency services. Her medical history consisted of type 2 diabetes mellitus controlled by oral drugs and chronic obstructive pulmonary disease (COPD). During transport to the hospital, an electrocardiogram (ECG) was taken, and ST-elevation myocardial infarction was suspected.


A 34-year-old female with a history of BTI, persistent dry cough for 6 weeks duration, and breathlessness for the past 12 months. She was referred for investigation to Hematology Section, National Center for Cancer Care and Research She denied fever, wheeze, haemoptysis, back pain or any other relevant complaints.
Physical examination revealed mild pallor, a brownish skin, a blood pressure of 130/80 mmHg, pulse rate of 76 beats per min, respiratory rate of 16 breaths per min and scars of previous splenectomy and cholecystectomy. She was occasionally transfused in the past years. Chest auscultation was negative.
On admission, her laboratory investigations showed haemoglobin=8 g/dL, white blood cell count: 5.7×109/L, platelet count: 676×109/L, mean corpuscular volume; 76 fl. The liver function test showed ALT: 32 U/L (normal 0-40), total bilirubin: 16 mmol/L (normal: 4-18), direct bilirubin: 1 mmol/L (normal: 0-4). Serum ferritin level was 6350 ng/mL (normal: 20-200).
The initial chest radiography showed a right hilar mass and an increased trabecular pattern (). A magnetic resonance imaging (MRI) of the chest demonstrated a well circumscribed paravertebral thoracic mass, measuring 4.5x3.5x4.3, without adenopathy, or erosion of the vertebral bodies and the ribs ().
A CT-guided needle biopsy of the right mass was performed. Histological findings ( D) revealed three lineage hematopoietic cells with markedly increased erythroid precursors and scattered hemosiderin laden macrophages, consistent with extramedullary hematopoiesis (). Flow cytometry analysis performed on the core biopsy confirmed involvement by EMH ().
Because of severe iron overload and alloimmunization we started treatment with hydroxyurea (HU). However, the treatment with HU remained inefficient. A surgical excision of the largest mass was performed. Cough and breathlessness improved markedly after the operation.


A 59-year-old man presenting with asymptomatic gross hematuria was referred to our hospital for further examination. Cystoscopy revealed a nodular tumor 8 cm in diameter filling the bladder (Fig. ). CT and MRI revealed that the tumor occupied the bladder (Fig. , ). He had a smoking history, but his family had no remarkable medical history. Laboratory examinations showed almost normal values. Transurethral resection of the bladder tumor was performed. Histologically, the carcinomatous element consisted of urothelial carcinoma with squamous differentiation, and the sarcomatous element consisted of osteosarcoma and high-grade spindle cells (Fig. ). He rejected radical cystectomy initially, instead undergoing a second transurethral resection of the bladder tumor. The resected specimen showed the same elements with muscle invasion. Therefore, he was scheduled to undergo 2 courses of neoadjuvant chemotherapy (gemcitabine 1,000 mg/m2 on days 1 and 8 and cisplatin 70 mg/m2 on day 2) and then radical cystectomy. After neoadjuvant chemotherapy, CT showed bilateral enlarged pelvic lymph nodes and left hydronephrosis without distant metastasis. He was unable to undergo radical cystectomy and ultimately died of cancer 6 months after the initial visit.


A 3-year-old female child presented to the emergency department with irritability, inability to walk by herself, weakness of the left hand and leg and left-sided deviation of mouth as noticed by the parents. She had fever, cold, and cough for previous 3 days. On examination, the child was irritable, afebrile, and had stable vitals. Neurological examination revealed hypotonia and sluggish reflexes on the left side with the child unable to lift the left upper and lower limbs above the bed. The child was treated as postictal Todd's paresis secondary to partial febrile seizures. When the symptoms did not improve after 6 h, an urgent magnetic resonance imaging (MRI) of the brain was done [Figures and ]. The MRI revealed occlusion of the right middle cerebral artery at its origin with a large penumbra. The child was urgently shifted to the neurointervention suite for endovascular clot retrieval under general anesthesia. The child was induced with intravenous propofol (2 mg/kg). Muscle relaxation was achieved with intravenous atracurium (0.7 mg/kg). The airway was secured with appropriate-sized endotracheal tube. Anesthesia was maintained with a mixture of air and oxygen (50:50) and sevoflurane (0.5–1 minimum alveolar concentration). Cerebral angiography confirmed the MRI findings. The clot was retrieved using suction aspiration device. Postprocedure angiogram revealed complete revascularisation of the right middle cerebral artery and normal blood supply in other intracranial blood vessels. The child was extubated after adequate reversal of neuromuscular blockade. Postprocedure, prothrombotic workup and an echocardiography were done. The echocardiography revealed severe mitral regurgitation with vegetation on the anterior mitral leaflet. The child was treated with antibiotics for infective endocarditis and physiotherapy. The child was discharged with significant improvement in power of the left upper and lower limbs and was able to walk on her own without support.


A 67-year-old woman was referred to our department with ocular pain, hyperemia, and blurred vision in the right eye. Cataract surgery had been performed for both eyes in a clinic at 3 months ago. She had no history of systemic disease and was in an immunocompetent condition. At first presentation, best-corrected visual acuity (BCVA, measured with a standard Japanese visual acuity chart and converted to Snellen visual acuity) and intraocular pressure (IOP) in the right eye were 20/20 and 17 mmHg, but slit-lamp biomicroscopy revealed a white clump of 3 mm diameter, anterior inflammatory cells, and an iris posterior synechia in the anterior segment (). There was no inflammation in the posterior segment (). Serum examination showed a slight increase in white blood cells (8,600/mm3) but not in β-d-glucan (9 pg/dL) or inflammatory proteins. Bacterial culture of the anterior humor and histological analysis of the white clump did not detect the cause of the endophthalmitis. As well, increase of 18S/28S rDNA (18S rDNA, not detected; 28S rDNA 2.4×102 copies/mL) was not presented by broad-range PCR using the aqueous humor. Because the white clumps in the anterior chamber strongly suggested fungal endophthalmitis, treatment with systemic and topical antifungal agents (systemic fosfluconazole [504 mg/d, 4 days] and topical pimaricin ophthalmic ointment [4 times/day]) was initiated in addition to antibacterial treatment (systemic cefepime [1,000 mg/d, 4 days] and topical moxifloxacin hydrochloride [6 times/day], vancomycin eyedrops [6 times/day], and ceftazidime eyedrops [6 times/day]). These medications were tapered according to the remission of the clump and inflammation in the anterior segment. At 14 days, the endophthalmitis was resolved () and the subjective symptoms had disappeared.
On day 47 after the initiation of treatment, she visited our hospital for headache, recurrence of ocular pain, hyperemia, and blurred vision in the right eye. BCVA was decreased to hand motion, and IOP was elevated to 40 mmHg in the right eye. Slit-lamp biomicroscopy showed reappearance of a white clump in the anterior segment, anterior inflammatory cells, large iris nodules, fibrin formation, and a posterior synechia (). The posterior segment was not visible due to inflammation in the anterior segment. Serum examination did not present an increase in β-d-glucan. Vitrectomy was emergently performed, and the aqueous humor and vitreous humor samples were obtained. Examination of microbial culture using samples from both the anterior chamber and the vitreous identified Penicillium species. We diagnosed fungal endophthalmitis caused by Penicillium species, and treatment with systemic micafungin (100 mg/d, 9 days), oral voriconazole (200 mg/d, 9 days) and flucytosine (6,000 mg/d, 9 days), and voriconazole eye drop (4 times/day) was initiated. The ocular inflammation by fungal endophthalmitis was gradually resolved with contraction of the clump after 14 days (), although the iris nodules remained. BCVA was improved to 20/16, and IOP was decreased to 16 mmHg. No recurrence was found 16 months after the second treatment.


We present the case of an eight-month-old Hispanic female with feeding refusal, vomiting, and poor growth. Upon presentation, her weight was at the 12th percentile and length was at the 11th percentile for age. Upper gastrointestinal contrast series revealed a congenital antral web. She was evaluated by the pediatric surgeon in Guatemala; however, the family preferred a less invasive approach. Therefore, she was referred to our institution for therapeutic endoscopy.
Upper gastrointestinal endoscopy at our institution revealed a small membranous web in the antrum of the stomach, extending inward from the pylorus. Upon initial endoscopy, an Olympus GIF 160 (Olympus Co., Tokyo, Japan) endoscope with an external diameter of 8.6 mm was inserted atraumatically through a bite block and advanced through the esophagus and into the gastric antrum. Here, a congenital antral web measuring approximately 5 to 6 mm in diameter was identified (). Using a gentle torqueing maneuver, the scope was advanced beyond the web to reveal normal mucosa distally. Withdrawing the scope revealed a significant degree of antral web ablation secondary to the initial scope advancement. Controlled radial expansion balloons (CRE; Boston Scientific, Marlborough, MA, USA) were then used to sequentially dilate the antral web. The balloons were advanced in to the pylorus and gradually expanded first to 9 and 10 mm, then to 11 and 12 mm, then finally to 13.5 and 15 mm for 1 to 2 minutes per session. Upon reaching a diameter of 12 mm, the balloon met resistance and endoscopic visualization through the balloon showed disruption of the mucosa to reveal muscular fibers of the normal pylorus below (). The endoscope was then slowly withdrawn, revealing ablation of the web with no unintended injury (). The patient tolerated the procedure well, and after a period of observation, she was discharged with no significant complications. Upon returning to Guatemala, she underwent a second upper endoscopy with balloon dilation of the pylorus at 13 months of age, from 12 mm up to 15 mm in diameter, without any known complications.
Two years post operatively, follow up revealed continued improved feeding tolerance without recurrence of her symptoms. The patient's weight and height plots also improved from the 11th and 12th percentile respectively at the time of presentation to above the 75th percentile.
Informed patient consent was obtained for publication of the case details.


We presented a 66-year-old woman with a past medical history significant for colon cancer (status post-hemicolectomy in 2006, followed by two rounds of adjuvant chemotherapy) and bladder cancer (status post-neoadjuvant chemotherapy with gemzar and cisplatin). She underwent cystectomy with urostomy. She has metastatic disease to multiple bones, pelvic and para-aortic lymph nodes as revealed on PET scan in November 2016. The patient was treated with atezolizumab for 6 months (beginning on December 21, 2016 to the end of June 2017; 2,000 mg/cycle for a total of eight cycles). Atezolizumab was stopped because of tumor progression and the patient was switched to pembrolizumab (200 mg/cycle) on June 28, 2017 with a total of five cycles prior to the current admission. The patient presented to our hospital with dysphagia, abdominal pain, nausea and intractable vomiting of a few days’ duration. Systemic review revealed a 20-pound weight loss. She had no rash, fever, chills, malaise/fatigue, cough, shortness of breath, diarrhea, dysuria, joint pain, myalgia or jaundice. The patient has been on acetaminophen (325 mg every 6 h as needed for pain), amoxicillin (2 g/day), bisacodyl (10 mg/day), dronabinol (5 mg/day), lisinopril (10 mg/day) and pantoprazole (40 mg/day). Physical examination revealed normal body temperature (36.9 °C), pulse rate (85 beats/min), blood pressure (112/72 mm Hg), respiration rate (16 breaths/min) and body mass index (23.88 kg/m2). The patient was in no distress. There was tenderness in the epigastric area but without rigidity or guarding. She exhibited edema with trace pitting in the lower legs to the mid tibia. Her urostomy presented with clear yellow urine. Otherwise, her physical examination was unremarkable. There was no evidence of lymphadenopathy, hepatomegaly or splenomegaly. Laboratory tests revealed normal electrolytes, normal liver enzymes (alanine aminotransferase (ALT) 18 IU/L, aspartate aminotransferase (AST) 21 IU/L and alkaline phosphatase 64 IU/L), slightly elevated total bilirubin at 1.3 mg/dL, slightly low albumin at 3.2 g/dL, low total protein at 5.6 g/dL, normal blood urine nitrogen (BUN) (8 mg/dL), creatinine (0.98 mg/dL), normal hemoglobin (12.2 mg/dL), white cell counts of 3.7 × 103/µL and platelet of 111 × 103/µL. Abdominal computed tomography revealed gastritis.
Esophagogastroduodenoscopy (EGD) revealed diffuse severely erythematous mucosa with bleeding on contact involving the entire stomach. In addition, diffuse nodular, inflamed and ulcerated mucosa was found in the gastric antrum () with narrowing of the pyloric channel (). This finding led to a clinical suspicion of malignancy. EGD revealed normal duodenum and esophagus. Multiple biopsies were taken from the stomach for histology.
The biopsy from the gastric antrum and body showed similar features which are characterized by marked mononuclear inflammatory cell infiltration in the lamina propria, crypt apoptosis, apoptotic abscesses, crypt dropout, crypt epithelial lymphocytosis and neutrophilic infiltration in crypt epithelium (). Focal erosion and ulceration are noted (). In addition, a few prominent lymphoid aggregates are noted in the lamina propria. There was no atypia for intraepithelial and lamina propria lymphocytes. A few cytomegalovirus-infected cells were identified on routine stain and further confirmed by immunohistochemistry (). Oxyntic glands in the body mucosa were significantly lost, but no intestinal metaplasia was noted (pictures not shown). The residual glands in the antrum and body showed regenerative and hyperplastic changes without evidence of dysplasia or carcinoma. No Helicobacter pylori organisms were identified on immunohistochemistry. Immunohistochemistry for CD3 and CD20 revealed a mixed population of T cells and B cells with T cells primarily on the crypt epithelium and B cells in the lamina propria () and lymphoid follicles (pictures not shown). There was no evidence of lymphoma.
Blood cytomegalovirus (CMV) DNA polymerase chain reaction (PCR) testing ordered after the gastric biopsy revealed CMV DNA was detected, but the load was less than 100 copies/mL (reference range: not detected). The patient was treated with intravenous ganciclovir 2.5 mg/kg twice a day for 2 weeks with a plan to switch to valganciclovir for an additional 4 weeks’ treatment. Pembrolizumab was held. Five days after intravenous ganciclovir treatment, the patient’s symptoms (abdominal pain and vomiting) were significantly improved and she was discharged from the hospital in stable conditions. No corticosteroids were administered to treat her gastritis.


A 35-year-old mentally disabled woman with a psychiatric history of obsessive behavior in childhood and anxiety disorder consulted the dermatology department for generalized pruritus. She was diagnosed with atopic dermatitis which resolved after corticoid-based therapy. In 2005, she was referred to our department because of laterocervical lymphadenopathies and night fever with intense sweating. Body computed tomography (CT) showed supra- and infra-diaphragmatic lymphadenopathies with various abdominal masses, the most relevant being 8×6×8 cm3. A biopsy of a lymphadenopathy revealed NS classic HL. With a final diagnosis of NS HL stage IIIB with an International Prognostic Score 2, she received six cycles of ABVD chemotherapy regimen with partial response and an early relapse in March 2006 (primary refractory to ABVD). This was followed by a salvage chemotherapy regimen (two cycles of ESHAP) with no response. In September 2006, a third chemotherapy regimen (four cycles of GemOx) was initiated with neutropenia and thrombocytopenia grade 4, which led to dose-intensity failures followed by chemotherapy discontinuation and radiotherapy consolidation. One month after the last radiotherapy session, the HL progressed; so, two cycles of IFE regimen were given with stable disease. Considering the basal comorbidities of the patient, we decided to pursue a watch-and-wait policy from 2008 to 2010, treating localized areas of progression with radiotherapy and obtaining transient partial responses (). In October 2010, a new mass appeared in D8–D9, presenting with a medullar compression syndrome. After a biopsy that confirmed HL relapse, it was treated with radiotherapy and six cycles of GemOx, which led to a partial response. However, a new progression occurred in the left cervical and axillary nodes within 1 month, which was observed with a watch-and-wait policy with the consent of the family.
In May 2012, our patient presented a new clinically symptomatic disease progression confirmed by positron emission tomography (PET)/CT, requiring bendamustine 90 mg/m2 and finally reaching disease stability. In August 2014, the HL progressed again with significant B symptoms and worsening clinical status (). At this point, based on our experience of the potential role of COX-2 expression in HL, and that the patient was not a candidate for intensive chemotherapy and brentuximab was still not available in our center, we decided to start an experimental treatment with celecoxib 200 mg every 12 hours and lenalidomide 20 mg (for 3 of every 4 weeks). This regimen was obtained through a compassionate use request. Our patient received this treatment from August 2014 to January 2015 (six cycles) with unremarkable toxicity and excellent tolerance, finally showing a CR for the first time in both the interim after three cycles and the final CT/PET after six cycles (). This response was maintained in a new CT scan in August 2015. After this induction, we started 1 year of celecoxib maintenance (200 mg/12 hours) that had to be stopped because of anemia due to gastrointestinal bleeding in March 2016.
In July 2016, with the most durable response until that moment being 22 months, our patient presented a new disease relapse. CT/PET showed supra- and infra-abdominal adenopathies with associated hepatic and splenic lesions. As two different biopsies of hypermetabolic locations resulted negative and keeping in mind the asymptomatic situation of our patient, we decided to restart celecoxib 200 mg every 12 hours. In this context, the disease progressed to ascites requiring paracentesis. Biopsies were constantly negative until February 2017, when a final biopsy confirmed the persistence of HL. At that point, we decided to start brentuximab vedotin (two cycles). During the second cycle of brentuximab, the patient required intensive care admission due to a sepsis with a fatal outcome.


A 33-year-old male, visited the outpatient department because of a right testicular mass, detected by ultrasound during a routine health examination. Subsequently, the computed tomography scan was preformed, which showed a 3 cm×3 cm solid-cystic mass in the right testicle (). The patient then underwent unilateral orchidectomy, with the specimen analyzed by histological examination. No further adjuvant treatment was performed after diagnosis. The patient is currently disease-free 12 months after diagnosis. The patient was diagnosed as a rare case of MCST of the testicles, based on the histopathology, immunohistochemistry, and genetic analysis.
The surgical samples were fixed in 4% buffered formalin and embedded in paraffin. Sections were cut and stained with hematoxylin and eosin and reviewed by the authors. For immunohistochemical staining, 3-4-μm-thick sections were cut, and deparaffinized. Heat-induced antigen retrieval was performed with slides placing in 0.01 M of citrate buffer and 3% hydrogen peroxide methanol solution treatment was conducted for 30 minutes to eliminate nonspecific protein reactions. The antibodies are given as follows: vimentin (clone V9, 1:50), CD10 (clone 56C6, prediluted), β-catenin (clone 14, prediluted), cyclin D1 (clone SP4, 1:100), inhibin (clone R1, 1:20), calretinin (clone 5A5, 1:100), pan-cytokeratin (clone AE1/AE3, 1:100), cytokeratin (CK; clone Cam5.2, 1:200), hector battifora mesothelial epitope-1 (HBME-1; clone HBME-1, 1:100), WT-1 (clone 6F-H2, prediluted), sal-like protein 4 (SALL4; clone 6E3, 1:100), placental-like alkaline phosphatase (PLAP; clone 8B6, prediluted), octamer-binding protein 3/4 (OCT3/4; clone N1NK, 1:100), α-fetoprotetin (AFP; clone C3, 1:750), estrogen receptor (ER; clone 1D5, 1:50), progesterone receptor (PR; clone PgR636, 1:100), synaptophysin (Syn; clone SY38, 1:50), chromogranin A (CgA; clone LK2H10, 1:100), CD56 (clone 123C3.D5, 1:100), CD31 (clone JC70A, prediluted), CD34 (clone QBEnd/10, 1:250), erythroblast transformation specific related gene (ERG; clone EPR3864, 1:100), desmin (clone D33, 1:200), smooth muscle actin (SMA; clone 1A4, 1:400), epithelial membrane antigen (EMA; clone E29, 1:200), CD99 (clone 12E7, 1:20), and Ki-67 (clone MIB-1, 1:100). Binding of primary antibodies was visualized with an Envision two-step method. Positive and negative controls were included in this study. For genomic analysis, DNA was first extracted from the formalin-fixed, paraffin-embedded tumor blocks. Then polymerase chain reactions (PCRs) were performed to amplify exon 3 of catenin (cadherin-associated protein), beta 1 (CTNNB1) and exon 1 of forkhead box protein L2 (FOXL2) using following primer pairs: 5′-GATTTGATGGAGTTGGACATGG-3′ (CTNNB1 forward) and 5′-GCTACTTGTTCTTGAGTGAA-GG-3′ (CTNNB1 reverse); 5′-CCGCCACAACCTCAGCCTC-3′ (FOXL2 forward) and 5′-CGCCGGTAGTTGCCCTTCTC-3′ (FOXL2 reverse). Bidirectional Sanger sequencing of all PCR products was performed subsequently on a 3500 Genetic Analyzer (Life Technologies, Carlsbad, CA) using the BigDye Terminator v1.1 Cycle Sequencing Kit (Life Technologies), according to standard protocols.
In terms of gross anatomy, the mass was a well-circumscribed nodule measuring 3 cm in the largest diameter, with the cut surface being grey-white and solid. No typical macrocyst was observed. histopathologically, the tumor is composed of solid sheets of tumor cells and intervening bundles of hyalinized fibrous bands separating tumor clusters at low power view ( and ). In some regions, variable microcysts could be observed. At the high-power view, the uniform tumor cells contained granular eosinophilic cytoplasm with intracytoplasmic vacuoles, and round to oval nuclei with open chromatin and small nucleoli. There were no nuclear grooves and the nuclear membrane was irregular. There were no nuclear atypia or necrosis, with a low mitotic index. Immunohistochemically, the tumor cells had diffuse and strong cytoplasmic immunoreactivity for vimentin () and CD10 (), as well as nuclear stains for β-catenin () and cyclin D1 (), but no positive expression with sex cord markers (α-inhibin and calretinin), germ cell tumor markers (AFP, PLAP, Oct 3/4; and SALL4), and mesothelial markers (AE1/AE3, Cam 5.2, vimentin, WT-1, HBME-1, and calretinin). Other negative markers included hormone receptors (ER and PR), neuroendocrine markers (Syn, CgA, and CD56), vascular markers (CD31, CD34, and ERG), and other markers including desmin, SMA, EMA, CK7, and CD99. The Ki-67‒labeling index was about 10%. The result of sequencing showed that a heterozygous missense mutation (c.110C>G) in exon 3 of CTNNB1 was identified in this case (), resulting in amino acid changes. No mutation of FOXL2 was detected in the presented case (data not shown).


We present a case of a 13-year-old boy, admitted to hospital for planned neurosurgical procedure of clipping aneurysm of left middle cerebral artery (arteria cerebri media [ACM]). He was previously hospitalized for urgent embolization and reembolization of ruptured right ACM (5/2017 and 6/2017). During reembolization, he developed allergic reaction with severe rash after induction of anesthesia. For that reason, clinical allergological testing on different anesthetic drugs was done. After clinical testing, the patient was proved allergic to midazolam, propofol, fentanyl, sufentanil, sevofluran, thiopental, rocuronium, and vecuronium.
Before new planned aneurysmal clipping, he was prepared with metilprednisolon, antihistaminic, and ranitidine. Preoperative morning cortisol levels were slight higher (742 nmol/L) and adrenocorticotropic hormone (ACTH) was lower than range (<0.2 pmol/L).
Because of patient's allergy to different drugs used for induction and maintenance of general anesthesia, and due to high demands for hemodynamic stability for aneurysmal clipping, anesthesia was performed with continuous infusion of etomidate and remifentanil, with scalp block and without use of neuromuscular blocking agents. We had previous good experience with continuous etomidate infusion for total intravenous infusion (total intravenous anesthesia [TIVA]) in neurosurgical patients; so, we decided to use etomidate in this case also.
Our protocol for anesthesia maintenance with etomidate infusion is dose of 100 mcg/kg/min for the first 5 min after anesthesia induction, followed by 60 mcg/kg/min for the next 25 min and then 10 mcg/kg/min until the end of the surgery. The protocol is based on recommendations from other research,[] and our clinical experience.
Enhanced anesthetic monitoring was used, with invasive blood pressure and entropy of encephalogram (Datex-Ohmeda S/5 Entropy Module), and the doses of etomidate used in this case enable isoelectric EEG for the time of clipping the aneurysm or burst suppression ratio greater than 70%. The surgery lasted for 4 h. There were no complications, hemodynamic, or any other sequellae during surgical procedure, and afterward, he was placed in neurosurgical intensive care unit (ICU) analgosedated and mechanically ventilated. The patient was awake and extubated 1 h after admittance to ICU and Glasgow Coma Score (GCS) was 15.
Levels of cortisol and ACTH were measured during next three consecutive days. Only one measured cortisol value, in the morning the day after the surgery, was below reference range, and the values were back to normal until that evening [].
The explanation is more difficult due to the fact that the patient was prepared for the surgery with metilprednisolon because of his allergies which may diminish influence of perioperative stress on cortisol synthesis. ACTH returned to normal after the second day. He was dismissed from ICU on the second day with GCS 15.


A 41-year-old woman was hospitalized when she visited the Otorhinolaryngology department complaining of a headache and a drumming sound in her right ear. Otolaryngologic examination did not reveal any pathological findings. The patient was referred to our department after brain imaging including magnetic resonance angiography (MRA) revealed a petrous carotid aneurysm (). A digital subtraction angiogram (DSA) was performed for further evaluation; it confirmed an 8-mm- saccular aneurysm with a 6-mm-wide neck (). The patient was prescribed aspirin (200 mg/day) and clopidogrel (75 mg/day) for 7 days as premedication. Femoral artery puncture was performed and 5,000 units of intravenous heparin sodium were administered. A microcatheter was placed within the aneurysm using the jailing technique. To ensure that the coils stay in the aneurysm, a stent was deployed in the right petrous internal carotid artery (ICA) in front of the aneurysm neck. Next, coil embolization was performed using the jailed microcatheter. The final DSA revealed a slight remnant of the neck and almost complete obliteration of the aneurysm with right ICA patency (). After the procedure, both the tinnitus and the headache completely subsided without any neurological deficits. The symptoms heave not recurred during a follow-up period of 4 years.


A 28-year-old man with Fraser syndrome was referred to the Thomas Jefferson University Hospital otolaryngology clinic and the Wills Eye Hospital oculoplastic and orbital surgery clinic for the evaluation of bilateral medial canthal swelling. His medical history on presentation was remarkable for cryptophthalmos, lacrimal system dysgenesis, chronic sinusitis, and asthma. There was no significant family history. He had no lacrimal complaints such as epiphora or punctal discharge. Warm compresses, massage, and a course of oral steroids had been attempted to treat the bilateral medial canthal swelling with no improvement. The patient denied a history of chronic sinusitis but did report frequently noticing a foul smell in his right nostril. He denied nasal obstruction, rhinorrhea, post nasal drip, or anosmia. At the time of presentation, he was taking Flonase and Flovent daily. His Sino-Nasal Outcome Test 22 score at initial visit was 47.
On examination, he was well appearing and had no mental status or cranial nerve dysfunction. Ocular examination revealed bilateral complete cryptophthalmos. The patient had poorly developed ocular adnexa with fusion of skin from eyebrow to his cheek. There was no separation of the eyelids present from birth. He had a history of an attempt at separation of his right eyelids with socket reconstruction with an intent to wear an ocular prosthesis which was unsuccessful. There were no puncta or evidence of proximal lacrimal systems on either side. Large, firm, cystic-feeling masses were palpable in the medial canthus bilaterally. The patient had brisk light perception vision in his right eye through his fused adnexal skin and was no light perception in his left eye. B-scan ultrasounds of his orbits revealed microphthalmia of his right eye and phthisis on the left. The patient had bilateral cryptotia, and his ear canals were narrow but clear. He also had evidence of fusion of the anterior third of his true vocal folds and a breathy voice. His craniofacial abnormalities are shown in . Written consent was given by the patient to publish identifiable images.
Flexible nasal endoscopy revealed mucopus streaming from the right osteomeatal complex and a left septal deviation with crowding at the middle meatus. Laryngoscopy revealed fusion of the anterior third of the true vocal folds with a 3-mm posterior glottis gap and a breathy voice.
Computed tomography of the sinuses () and magnetic resonance imaging of the orbits () demonstrated right greater than left bilateral dacryocystoceles containing proteinaceous material extending into the lacrimal ducts. There was also complete opacification of the right maxillary sinus with expansion at the middle meatus.
Based on these findings, he was then taken to the operating room for a bilateral endonasal decompression of dacryocystoceles and a right maxillary antrostomy. An endoscopic approach was favored due to the patient’s monocular status. Due to the agenesis of the patient’s proximal lacrimal drainage system, further interventions prior to lacrimal sac marsupialization were impossible. A conjunctivodacryocystorhinostomy (CDCR) was not indicated, as it would not have added to the described surgery and would have put his barely seeing eye in undue risk as well as implanted a foreign body that carries further risks and complications. These were discussed, and the reported surgery decided upon.
The lacrimal bone and mucosa was taken down endonasally using a Stryker Sonopet™. The congenitally enlarged lacrimal sacs were then punctured using an 11-blade knife and marsupialized to ensure appropriate drainage. On the right, mucoid material was expressed, and on the left, serous fluid was encountered. At the time of surgery, polypoid inflammation was noted in the right maxillary sinus. Postoperatively, he had resolution of the foul odor and otherwise felt normal. At 2- and 6-month follow-up, he was noted to be doing well with no evidence of recurrence. His lacrimal sacs remained well marsupialized into the nasal cavities. Endonasal imaging from 6-month follow-up is shown in .


A 24-year-old male presented to the emergency department with a 3-day history of fever, fatigue, and severe headache. He had been living in a rural area and had been working in a forest for 1 month where he got wet in the rain 3 days before his presentation. History of tick and rodent contact was unclear. His medical history was positive for six pack-years of cigarette smoking. His family history was unremarkable. On physical examination, his temperature was 39.1°C, heart rate 92/min, blood pressure 120/70 mmHg, and respiratory rate was 16/min. His throat and conjunctiva were hyperemic and tongue and mucosa were dry. Pulmonary sounds were normal on auscultation. Spleen was palpable below the left costal margin. Other physical examination findings were normal. Laboratory values were white blood cell: 7.3 K/uL (n = 4–10.5), hemoglobin: 14.6 g/dL (n = 12-17.5), platelet: 34,000/mm3 (n = 140,000–450,000), urea: 18.7 mg/dL (n = 16.6–48.5), creatinine: 0.81 mg/dL (n = 0.7–1.2), alanine transaminase: 21 U/L (n = 0–41), aspartate aminotransferase: 39 U/L (N: 0-40), creatine kinase: 375 U/L (n = 39–308), and C-reactive protein: 59.5 mg/L (n = 0–5). HIV was negative. Urinalysis showed mild proteinuria and microscopic hematuria. Chest X-ray was within normal limits. Abdominal ultrasound showed a mild increase in the long axis of both kidneys and splenomegaly (long axis of spleen: 143 mm). He was admitted to inpatient clinic with a presumptive diagnosis of acute respiratory system infection. After collection of blood and urine cultures, ceftriaxone intravenous was started empirically. Serum samples were sent to the National Arboviruses and Viral Zoonoses Reference Laboratory to rule out Crimean-Congo hemorrhagic fever and hantavirus infection. Platelet transfusions were given to treat the thrombocytopenia. On the 3rd day of hospitalization, he suddenly developed severe dyspnea, massive dry cough and a dramatic decrease in oxygen saturation which was 79% on a finger pulse oximeter. During the deterioration, his blood pressure was normal (110/70 mmHg), platelet count was 67,000/mm3, renal function tests were urea: 28 mg/dL, creatinine: 1.51 mg/dL. Liver function tests and coagulation profile were in normal limits. On oxygen supplementation, arterial blood gas analysis was: pH: 7.43 (n = 7.35–7.45), PaCO2:29.9 (n = 35–45), PaO2:53.2 (n = 60–100). There was no hemoptysis. To rule out pulmonary embolism, computed tomography angiography of the thorax was done. There was no evidence of pulmonary embolism, however, bilateral extensive infiltrations and bilateral pleural effusion were detected []. As the patient had no finding of hemodynamic instability such as hypotension or bleeding, clinical exacerbation was thought to be related to impaired pulmonary ventilation due to infective process rather than pulmonary hemorrhage. Mild clinical and laboratory findings despite severe radiological findings suggested atypical pneumonia as the diagnosis. Intravenous moxifloxacin was added to the treatment. Then, the patient was referred to a tertiary care hospital due to possible risk of acute respiratory distress syndrome and admitted to its intensive care unit. Treatment was continued with ampicillin/sulbactam, clarithromycin, and oseltamivir. The next day, he was transferred to the ward as there was improvement in his clinical status. His renal function tests and arterial blood gas analysis returned to normal. There was no growth in blood and urine cultures. Legionella urinary antigen was negative. Other relevant microbiological agents were all negative []. Hantavirus IgM and IgG were positive on indirect immunofluorescence assay (Anti-Hantavirus IIFT, Euroimmun, Germany) immunoblot analysis (Antibodies against antigens of Hantavirus, Hanta Profile 1 Euroline, Euroimmun, Germany) and evaluation of the test strips using EUROLineScan program. The sensitivity of the Anti-Hantavirus IIFT test is reported to be 96% for IgM and 99% for IgG and the specificity 100% for IgM and 98% for IgG. The sensitivity of the Hanta Profile 1 Euroline IgM test is reported to be 91.4% for IgM and 84.5% for IgG, and the specificity 86% for IgM and 95.1% for IgG by the manufacturer. The serotype of the virus was detected as Puumala. Immunoblot strips and evaluation form images are shown in . After a 10-day hospital stay, the patient was discharged on oral cefdinir and clarithromycin. At 1 month follow-up, he was asymptomatic and his blood tests and computed tomography of the thorax were normal. At 2 months follow-up, antibody levels against hantavirus were increased by four-fold on immunofluorescence assay. Thus, the final diagnosis in our patient was HPS due to PUUV.


A 53-year-old African American female with a body mass index of 27.2 kg/m2 and a history of coronary artery disease, end-stage renal disease, cervical carcinoma and a right atrial thrombus, presented to our emergency department from an outside hospital bleeding profusely from her rectum. At an outside facility, two self-expanding nasal tampons (Rhino Rockets, Shippert Medical Technologies, Centennial, Colorado, USA) were placed in the rectum to control bleeding and two units of PRBCs were transfused. While still in the emergency department, MTP was started, and 2 g of intravenous tranexamic acid (TXA) were administered.
The patient was emergently taken to the operating room, but surgical efforts failed to control the bleeding as the pelvic radiation therapy she had previously received to treat a cervical carcinoma had distorted her anatomy such that her internal iliac artery could not be visualized. Proctoscopy performed after total colectomy revealed a still massive amount of blood coming from the lower rectum or anal canal. A balloon was fashioned with a Penrose drain and Foley catheter to create a tamponade effect in the rectum; however, this was effective for only a short while. Thromboelastography (TEG) results – a normal reaction time (R) time of 7 min, a decreased alpha angle of 40° and decreased maximum amplitude of 36.7 mm – pointed toward a coagulopathy secondary to fibrinogen and platelet deficiency. The patient continued to receive fresh frozen plasma (FFP), cryoprecipitate and platelets. While there was a concern of a coagulopathy related to the apixaban, the patient was prescribed for atrial thrombus, the etiology of bleeding, in this case, was surgical. During the consultation with the interventional radiology service for angioembolization, she was brought to the Surgical Intensive Care Unit (SICU) for interval management with the rectal balloon in place.
In the SICU, two rapid transfuser devices (Level 1, H-1200 Fast Flow Fluid warmer, Dublin, OH, USA) were assembled, and a wide-bore central venous catheter was inserted. MT was continued with a goal mean arterial pressure (MAP) of 50–60 mmHg through the administration of blood products, norepinephrine, and vasopressin infusion and intermittent boluses of epinephrine. Resuscitation was guided by vital signs, bedside echocardiography, serial TEG and perfusion biomarkers such as serum lactate and base deficit. A >14 L blood loss was recorded in the SICU in 90 min.
In the interventional radiology suite, a fistula between the right common iliac and the rectum was identified as the source of bleeding. A 10 mm × 40 mm covered self-expanding intravascular stent (Fluency, Bard Peripheral Vascular, Tempe, AZ, USA) successfully stopped the bleeding. In total, the patient received 60 units PRBC, 23 units FFP, 20 packs of platelets, 6 units cryoprecipitate, 2 g TXA, 30 L of crystalloid, and 2 L of albumin in <7 h.
Serial focused cardiac ultrasound (FoCUS) guided the resuscitation. Hypothermia, serum lactate, and base deficit improved from 35.4, 7 and − 10–36.8, 1.9 and − 4 respectively, in the next 4 h. By that point, the patient was able to follow commands and required minimal ventilatory support. In the ensuing days, there were no signs of lung injury, volume overload, coagulopathy, or cardiomyopathy. Her abdomen was closed 2 days later, successfully extubated shortly thereafter, and transferred to the floor.


A 40-year-old female with a past medical history of type II diabetes mellitus, hypertriglyceridemia, and pancreatitis presented with worsening blurry vision in her left eye. Previously, she had been treated for diabetic macular edema with a single intravitreal injection of bevacizumab. She reported that she was not currently on any medical treatment for her chronic conditions due to extenuating social circumstances. Best corrected visual acuity was 20/25 in the right eye and 20/400 in the left eye. Fundus examination of the right eye revealed mildly tortuous vessels with intraluminal whitening and white discoloration of the retina. Posterior segment examination of the left eye demonstrated similar vascular changes along with peripapillary and macular edema, intraretinal hemorrhages, and cotton wool spots (Fig. ). Though normal in the right eye, fluorescein angiography of the left eye demonstrated dilated vessels and late peripapillary leakage (Fig. ). Ocular coherence tomography (OCT) was significant for increased retinal thickening with intraretinal and subretinal lipid exudation (Fig. ). Lab workup at this visit was notable for a complete blood count that was within the normal limits, a hemoglobin A1c of 8.9%, and elevated levels of total cholesterol (> 650 mg/dL) and triglycerides (> 1,575 mg/dL). Subsequent lipoprotein electrophoresis confirmed a pattern of dyslipidemia consistent with type IV hypertriglyceridemia []. Upon confirmation that the intraluminal whitening represented lipemia retinalis, the patient was treated with an intravitreal injection of bevacizumab.
After three consecutive monthly intravitreal bevacizumab injections, the patient's vision in her left eye improved to 20/20, though peripapillary hemorrhages, macular edema, exudate, and intravascular whitening persisted (Fig. ). An OCT scan demonstrated improvement of intraretinal and subretinal fluid with persistent hyperreflective outer retinal exudates (Fig. ). In contrast to her visual improvement, repeat serum lipid examination showed persistently elevated total cholesterol (872 mg/dL) and triglycerides (> 4,425 mg/dL), and the patient was counseled again about the importance of starting systemic treatment. The patient's vision and retinal examination were stable 2 months after the last injection. She reported starting cholesterol lowering therapy with gemfibrozil 600 mg twice daily and atorvastatin 40 mg daily under the guidance of her primary care physician.


An 11-year-old girl, with history of total colectomy in infancy due to Hirschsprung disease, was admitted to our hospital due to acute onset of ataxia and chronic abdominal pain. Her pain started 2 months prior to admission, thought initially to be related to cholelithiasis evident on ultrasonography, and her gallbladder was subsequently removed. Despite that, she continued to complain of abdominal pain associated with intermittent nausea and decreased appetite, but no fever or vomiting. One week after her cholecystectomy, she developed unsteady gait and weakness, progressed to inability to walk without assistance. The patient had an ileo- anal pull-through as a young child, and currently has fecal incontinence controlled with loperamide. She also takes metronidazole, 1 week a month, for suspected small intestinal bacterial overgrowth (SIBO), although no hydrogen breath test or other confirmatory tests were performed. She lost 2.6 kg (~ 5% weight loss) since her abdominal pain started over the last 2 months. She was developmentally appropriate, and her immunizations were up to date. No sick contacts were reported. On physical examination; weight 49.6 kg (86th percentile), temperature 36.3°C, heart rate 95 beats/min, blood pressure 110/66 mmHg, respiratory rate 20 breaths/min, and oxygen saturation 98% on room air. She was alert and oriented, in no acute distress. She had normal cranial nerves examination with a muscle strength of 4/5 in the lower extremities. Deep tendon reflexes were present +2. She was unable to stand or walk without assistance and when she did, she could not maintain her balance. Romberg’s test was positive.
She had tingling of her toes with rare stabbing pain in the feet while walking. Her abdomen was diffusely tender to palpation, but no rebound or guarding. Bowel sounds were normal. The rest of her examination findings were unremarkable. Laboratory showed normal complete blood count, electrolytes, renal, liver and thyroid function tests. She had normal C-reactive protein, erythrocyte sedimentation rate, lipase, creatine kinase and D-lactate levels. Magnetic resonance imaging (MRI) of the brain was normal. Nerve conduction study showed mild demyelinating sensory neuropathy. A micronutrient-deficit neuropathy was suspect, so addition laboratories were obtained (Table ). She was treated empirically with 100 mg of vitamin B1 intravenously (IV), 0.4 mg folic acid IV and 500 µg of vitamin B12 intramuscularly before vitamins levels results were available. For her abdominal pain; the patient had an extensive gastrointestinal work-up including upper gastrointestinal series, abdomen MRI, upper and lower endoscopies; all were normal for her current anatomy. She was started on amitriptyline for her abdominal pain before she was discharged home with physical therapy follow up. Vitamin B1 serum level was reported low at 2 nmol/L after discharge (reference range: 4–15 nmol/L) and patient was diagnosed with dry beriberi. She was started on 50 mg of vitamin B1 orally, in addition to her multivitamin supplements. Her repeated B1 level 1 month later was normal at 11 nmol/L. At 3 months follow up; patient showed significant improvement; her ataxia almost resolved, and she was able to walk unaided with minimal imbalance. The tingling and stabbing pain in her feet had resolved as well, and her abdominal pain also improved. She returned to her normal diet and normal daily activities.


A 27-year-old male presented to our hospital with complaints of headache, vomiting, and blurring of vision for the past 3 months and imbalance and swaying toward the right side for the past 1 month. Examination showed dilated pupil with ptosis on the right side, decreased visual acuity of 6/24 on Snellen chart, and relative afferent pupillary defect. Magnetic resonance imaging head revealed a well-defined suprasellar mass lesion approximately 3 cm × 2.5 cm × 3.5 cm, T1 isointense, T2 heterointense, homogeneous, contrast-enhancing mass lesion with lobulated appearance seen compressing the midbrain and pushing and lifting up the third ventricle upward to the opposite side with proximal ventriculomegaly and periventricular lucencies [Figure –]. The mass lesion is predominantly seen in the interpeduncular cistern with extension across midline and compressing the cerebral peduncle, which seems to efface and displace the aqueduct [Figure and ]. It extends anteriorly along the cavernous sinus toward superior orbital fissure with compression of adjacent optic nerve. Differential diagnosis at that time included meningioma, schwannoma of cranial nerve, vascular pathology (thrombosed giant aneurysm), and lymphoma.
The patient was planned for right frontotemporal craniotomy with transslyvian and subtemporal approach for excision of the tumor. Right frontotemporal craniotomy with zygotomy was done, craniotomy was done flush with the temporal base, and dura was opened based on sphenoid ridge. Brain was seen bulging on opening the dura, and ventricular tap was done to release cerebrospinal fluid. Sylvian fissure was opened from lateral to medial side, and right temporal lobe was retracted medially to expose the tentorium and the tumor. Tentorial notch divided posteriorly to widen the exposure. The tumor was whitish soft to firm, partly suckable, and moderately vascular seen attached intimately to tentorium, extending posteriorly to basilar artery and posterior cerebral artery (PCA). Total excision of the tumor was done [Figure and ]. Intraoperatively, 4th cranial nerve was identified along the margin of tent, but the 3rd cranial nerve could not be identified separately from the tumor as it was involving the whole cisternal part of the 3rd nerve.
Histopathological examination of the tumor tissue showed tumor composed of spindle cells having hypocellular and hypercellular areas alternating with each other. The hypercellular areas were composed of cells displaying elongated nuclei with bland nuclear morphology, formation of verrucae bodies, focal areas of hemorrhage, and hemosiderin-laden macrophages [].
Postoperative contrast computed tomography head revealed total excision of the tumor. Postoperatively, the patient had right 3rd nerve deficit. At 2-month follow–up, the patient had improvement in headache with partial recovery of the right 3rd nerve palsy.


A 23-year-old male with no known comorbidities or addiction presented with low back ache associated with progressive paraplegia and loss of bladder and bowel function of 2-month duration. Magnetic resonance imaging (MRI) of the spine showed a 1.2 cm × 1.4 cm × 6.4 cm intramedullary lesion spanning D8–D10 with evidence of lytic areas within []. MRI brain was normal. He underwent laminectomy with excision of the intramedullary lesion. Histopathology report (HPR) showed features of necrosis, endothelial proliferation, brisk mitosis, and nuclear atypia suggestive of GBM []. Immunohistochemistry (IHC) was positive for glial fibrillary acidic protein (GFAP) [] with a Ki-67 of 60%, which confirmed the diagnosis. He received adjuvant RT to the spine to a dose of 5040 cGy in 28 fractions along with concurrent temozolomide (TMZ) which he tolerated well.
He was then started on adjuvant TMZ; however, during his fourth cycle, he developed headache and recurrent episodes of vomiting. MRI of the brain and spine revealed multiple, well-defined round-to-ovoid lesions attached to frontal, occipital horn and septum pellucidum of lateral ventricles, largest measuring 2.3 cm × 2.1 cm × 1.9 cm with resultant lateral ventricle, third ventricle dilatation with fourth ventricle obstruction with hydrocephalus []. Extensive areas of altered signal intensity with associated cord expansion were seen extending from cervicomedullary junction till cauda equina up to L1 vertebrae []. Whole-body and brain positron emission tomography scan showed multiple fluorodeoxyglucose (FDG) avid brain and spinal lesions without any FDG uptake in other parts of body. Craniospinal fluid (CSF) analysis was positive for tumor cells. The patient underwent craniotomy and ventriculoperitoneal shunt placement. Postoperative HPR [] revealed large areas of necrosis with peripheral pseudopalisades, microvascular proliferation, mitosis, and high nuclear-cytoplasmic ratio which suggested a metastatic deposit from spinal GBM. IHC showed positivity for GFAP, synaptophysin, and Ki-67 of 70%, thus confirming the diagnosis. In view of his Karnofsky Performance Score of 50% and prior spinal RT, only palliative whole brain RT (WBRT) to a dose of 34 Gy in 10 fractions was given. He was then put on maintenance metronomic TMZ 50 mg Once a day (OD), but his general condition kept on deteriorating and he finally succumbed to his illness.


A 79-year-old female presented as an outpatient with a 10-year history of severe neurogenic claudication and severe back pain. She also complained of difficulty with walking up stairs. Motor strength in the lower extremities was 4+/5 proximally and distally with no sensory changes and preserved reflexes. Physical therapy, epidural steroids, and oral medications were attempted initially. Computed tomography (CT) workup of the patient revealed L3–4 Grade 1 and L4–5 Grade 1 spondylolisthesis, unilateral complete pedicle fractures at L2 and L3 on the left, and incomplete pedicle fracture of L2 on the right and slight rotatory scoliosis [Figures and ]. At the L3 level, there was visible sclerosis on the right pedicle []. Flexion extension views of the lumbar spine revealed no dynamic instability. Magnetic resonance imaging (MRI) findings revealed spinal stenosis at L4–5 with severe bilateral neuroforaminal stenosis.
The patient underwent L4–5 minimally invasive decompression and fusion. The indication for surgery was bilateral lower extremity radiculopathy with foraminal and central canal stenosis as well as low back pain.
After the surgery, the patient continued to have a significant back pain. It was felt that the unilateral pedicle fractures needed to be braced due to the L4–5 fusion causing additional stress at the adjacent levels with pedicle fractures.
L2 and L3 unilateral pedicle screws were placed using biplanar fluoroscopy. The L2 and L3 left pedicles were cannulated with Jamshidi needles and K-wires. The K-wire tracks were not tapped or drilled, and a Medtronic 5.5 mm × 50 mm screw was inserted at L2 and L3.
The patient had a subjective relief of back pain and claudication following the two surgeries at her 3 months follow-up visit.
At 3 months postoperatively, CT showed bony trabeculation across the bilateral L2 fractures and left L3 fractures []. Nine-month postoperative CT revealed complete fusion of all pedicle fractures with clear cortical borders visible with resolution of sclerosis in the right L3 pedicle [Figures and ]. Four years after initial screw placement, MRI shows no evidence of marrow changes or edema within the pedicles.


A 3-month-old-male infant weighing 7 Kg presented with enlargement of head since birth. The patient was born full term by elective cesarean section to a 26-year-old mother (Gravida 1, Para 1). There was no history of consanguinity. Mother was hypothyroid, not receiving thyroxin with markedly raised levels (TSH 34.51 mIU/ml), and anemic (Hb 8 g %). Serological tests such as TORCH, Venereal Disease Research Laboratory, HIV, and HBsAg were negative. She had not received iron-folic acid tablets periconceptually. There was no history of infection, teratogen ingestion, and drug abuse or radiation exposure. There was no family history of congenital anomalies. The antenatal diagnosis of hydrocephalus was made at 26 weeks of gestation. The repeat antenatal ultrasonography (USG) performed at 34 weeks showed hydrocephalus with dilated lateral and third ventricles with thinned cerebral cortex and nonvisualization of fourth ventricle. His birth weight was 2745 g and head circumference was 41 cm. Postnatally, he developed physiological jaundice which was managed expectantly and was discharged with an advice for pursuing operative intervention for hydrocephalus. Milestones of the baby were delayed.
Clinical examination revealed enlarged head with nonpulsatile and bulging anterior fontanel, engorged scalp veins, and presence of sunset sign. The head size of the patient was 50 cm and skull vault appeared deficient superiorly and in the occipital region []. An accessory opening arising from the left nostril was present, with an internal diameter measuring 3 mm and external of 7 mm []. No active bleeding or discharge was observed. The ipsilateral normal left nostril (opening) was placed medially, appeared small (slit like) as compared to right nostril []. It was completely separate from the accessory cavity by thick bridge of skin encompassing cartilage. On probing with 6 French fine tubing, the accessory nasal cavity was blind ending (1.5 cm in depth), without any communication to the ipsilateral nasal cavity []. The right nostril was normal. Rest of the nasal cavity structure was normal. There was no columellar deviation.


A 61-year-old female suffered from sudden-onset headache and transient loss of consciousness. A plain head CT on admission showed massive SAH (H and K Grade II) []. 3DCTA on admission showed the cause of hemorrhage due to ruptured left carotid cave aneurysm []. On the same day, emergent direct clipping was performed through an extradural tempolopolar approach [Figure –]. SAH around the aneurysm was so thick, and rupture point was confirmed. Complete clipping was done []. Postoperative course was uneventful.


A 39-year-old male suffered SAH (H and K grade II) []. 3DCTA on admission showed the cause of hemorrhage due to ruptured dissecting aneurysm at the periphery of right PICA [, white arrow indicated]. On the same day, emergent trapping of the PICA lesions with OA-PICA bypass was performed through a transcondylar fossa approach [Figure –]. Complete clipping was done. The patency of the bypass and/or parent artery flow were confirmed. There was no appearance of the new cerebral infarction []. There was no conscious disorder and he was discharged for rehabilitation purpose.


A 58-year-old male suffered SAH (H and K Grade V) []. 3DCTA on admission showed the cause of hemorrhage due to ruptured dissecting aneurysm at the left side []. The lesion is confined to the VA on the peripheral side of the PICA bifurcation, which showed nondominant flow side []. On the same day, emergent trapping of the VA lesions was performed through a transcondylar fossa approach []. The patency of the bypass and/or parent artery flow were confirmed. There was no appearance of the new cerebral infarction []. There was no conscious disorder and he was discharged for rehabilitation purpose.


A 26-year-old male was injured during a fire show performance. After suffering severe burns, he fell and hit his head. Symptoms of injury manifested 6 months later when the patient performed a sudden head movement and began to display severe tetraparesis. Magnetic resonance imaging (MRI) at this time revealed dislocation of the anterior transligamentous atlas dislocation [].
The patient's burns were treated before admission for surgical treatment. Fibrous scarring over the burn areas resulted in rigid fixation of the head and cervical vertebral column into an abnormal position [].
Functional radiography of the cervical spine indicated the feasibility of a partially closed reduction of the atlas dislocation. Considering the location of the invaginated odontoid process above the hard palatine line [], a transnasal endoscopic odontoid process resection was selected for surgical treatment.
The patient underwent a two-stage surgical treatment. During the first stage of treatment, posterior decompression and fixation were performed under halo immobilization. In the second stage of treatment, an endoscopic approach to the odontoid process was performed. The anterior half ring of the C1 vertebra was exposed and the upper part of the half ring was resected. The odontoid process was located at 2 cm from the posterior edge of the C1 arc. An approach to the odontoid process of the C2 vertebra was performed under radiographic guidance. The odontoid process and upper part of the C2 vertebral body were identified and cleaned. The odontoid process and apical ligament were resected using a surgical drill and Kerrison cutters [Figure and ]. Low-intensity cerebrospinal fluid flow from a dural tear was observed after resection of the odontoid process apex, and apparent pulsation of dura was observed after the decompression was completed []. Dural repair was performed using a hemostatic sponge and biological glue. The glue was also used for fixation of the mucous membrane flap []. Postoperative MRI of the craniovertebral joint is shown in . There were no postoperative complications, and the patient was discharged in good condition 9 days after the surgery.


A 44-year-old woman with no history of smoking was referred to our hospital in July 2017 with a chief complaint of abdominal discomfort, which had lasted for a month. Transvaginal sonography suggested cervical carcinoma. Cervical smear cytology showed poorly differentiated adenocarcinoma of the cervix uteri (Fig a). Moreover, immunohistochemistry (IHC) revealed expression of CK7, TTF-1, and Ki-67 (25%) without GATA-3, P16, or P63 (Fig b–g). Mammography revealed a mass in the upper outer quadrant of both breasts, highly suggestive of category 4 malignancy, according to the Breast Imaging Reporting and Data System (BI-RADS). Ultrasound-guided breast puncture biopsy revealed poorly differentiated adenocarcinoma, which also suggested lung metastatic adenocarcinoma (Fig a). IHC staining showed positivity for TTF-1 and Ki-67 (25%) and negativity for GATA-3, ER, PR, and Her-2 (Fig b–g). Meanwhile, chest computed tomography (CT) showed a lesion in the upper lobe of the left lung and mediastinum, plus left hilar lymphadenopathy (Fig a). Computed tomography (CT)-guided percutaneous lung puncture biopsy revealed pulmonary adenocarcinoma (Fig a), and IHC staining showed positivity for CK7, TTF-1, and Ki-67 (≤ 10%) and negativity for ER, PR, Her-2, and GATA-3 (Fig b–e). We chose these as potential markers of malignant disease: TTF-1 is the most valuable specific antibody for identifying primary or metastatic lung adenocarcinoma; ER, PR, Her-2, and GATA-3 expression has great significance in diagnosing metastases of primary breast cancer; and Ki-67 is used to determine the proliferative activity of tumor cells. Thus, the patient was eventually diagnosed with lung adenocarcinoma with breast and cervical metastases. Subsequently, next-generation sequencing of DNA from the tumor biopsy specimens revealed consistent ALK rearrangements (EML4-ALK) between the primary lung lesion and metastases. As shown in Figure , there was mutant abundance among the different tissues. Mutant abundance is an important indicator of tumor heterogeneity. There are various tumor cells in the tumor tissue, and different tumor cells carry different mutant genes, thus mutant abundance between the primary lesion and metastases will vary by heterogeneity.
The patient was prescribed crizotinib (250 mg orally, twice daily) in August 2017, and experienced no obvious drug-related adverse effects during or following treatment. Follow-up CT scans taken at one week and at two months after treatment commenced demonstrated that the lung lesion was markedly diminished and was assessed as stable disease (Fig b,c). In addition, an ultrasound revealed that the metastases in the cervix and breast were obviously reduced after crizotinib treatment. Unfortunately, encephalic magnetic resonance imaging revealed multiple bilateral intracerebral metastases; therefore, the patient has received radiotherapy for the brain metastases while continuing oral crizotinib therapy to date.


A 90-year-old female patient was admitted to our medical center due to a mental change. The patient was in a stuporous state, and Glasgow coma scale (GCS) was checked by E2M5V1. She had experienced a head injury two months before the admission, and a post-traumatic computed tomography (CT) scan performed immediately after trauma indicated chronic SDH in the left hemisphere (). Although she had no neurologic symptoms at first, symptoms such as dysphagia and right-sided weakness gradually appeared. The follow-up brain CT scan showed increase in volume of the hematoma at the left hemisphere (). She got the burr hole trephination with closed drainage in the left parietal area under the general anesthesia. Follow-up CT scan performed the day after the surgery revealed well-drained chronic SDH (). Her right side motor weakness improved after surgery and GCS was checked by E3M6V3. On day 3 after surgery, the patient abruptly showed stuporous mentality and CT scan revealed acute hematoma inside a cavity of chronic SDH with the midline shift toward the right (). The CT image indicated newly development acute SDH over the pre-existing chronic SDH cavity. Her family did not want more surgical treatment because of her age, and they wanted conservative therapy. We decided to liquefy and drain the solid hematoma by urokinase irrigation using the existing closed drainage system. 17,000 IU of urokinase was injected into the hematoma cavity once daily for 2 days and clamped for 30 minutes. The total drainage volume was 183 mL for two days, and only a small amount of residual hematoma was observed in the CT scan performed two days after irrigation (). The patient made a remarkable recovery and had little neurological deficit. GCS was checked by E4M6V4 when discharged. A two-month follow-up CT scan indicated excellent brain re-expansion ().


A 58-year-old man with an incomplete SCI (ASIA C and neurological level at Th12) secondary to L1 vertebral fracture (Fig. ), presented a 32-year history of gait disorder with neurogenic bowel and bladder dysfunction.
The patient had undergone several years of rehabilitation without improvement in his neurological symptoms.
Intermittent bladder catheterization was used for urinary dysfunction and presented a severe constipation with a defecation frequency every week with the need of laxatives and digital evacuation.
Initial physical examination revealed an atonic anal sphincter and a pelvic floor descent.
An endorectal ultrasound was done, the external anal sphincter showed no lesions and a thin internal sphincter without mobility. Anorectal manometry showed basal resting pressure and anal squeeze pressure of 35–40 mmHg. Also, a hypotonic internal anal sphincter, lack of external anal sphincter contraction and a complete absence of rectal sensibility due to distension despite high volumes. Rectoanal inhibitory reflex was present.
Dynamic MRI: Important rectal prolapse and pelvic floor descent. The evacuation after the introduction of the endorectal contrast was incomplete. (Fig. )
In the Krogh´s Neurogenic Bowel Dysfunction scale [] presented a Severe condition with 19 points.
Previous authorization of the ethic committee and a signed informed consent, the patient was included in a clinical trial to study the effects of cell therapy in patients suffering SCI (EudraCT 2014-005613-24).
We used a cell therapy medicament (NC1) currently approved as a medicament under clinical investigation by the AEMPS (PEI No. 12–141). It consists of autologous MSCs and autologous plasma as its excipient.
For culture of MSCSs, 50 mL of bone marrow was aspirated under aseptic conditions from the iliac bones and sent to a cleanroom for culture and expansion under good manufacturing practice (GMP). The cultures were maintained at 37°C in a humidified 5% CO2 atmosphere for 3 days, after which non-adherent cells were removed by replacing the medium. When the cultures approached confluence (90–100%), adherent cells were detached by treatment with trypsin/ethylenediamine tetraacetic acid (EDTA) solution (BioWhittaker-Lonza).
Criteria for the administration of MSCs included a viability >95%, absence of microbial contamination (bacteria, fungus, virus or mycoplasma), expression of CD105, CD90, HLA I, CD73 and CD166 for more than 90% of cells, and absence of CD34, CD80, HLA II, CD45 and CD31 (expression of each <5%), as assessed by flow cytometry.
He received 300 million autologous MSCs in the subarachnoid space by lumbar puncture, at a dose of 100 millions every 3 months in the Cell Therapy Unit.
After the third administration, the patient had an important improvement in almost every functional scale of spinal cord injury, especially in the Krogh´s Neurogenic Bowel Dysfunction scale, at this point with a Minor Bowel Dysfunction grade with 6 points.
Six months after first treatment, an anorectal manometry was done, showing an increased basal resting pressure of 82 mmHg and anal squeeze pressure of 105.4 mmHg. Slight improvement in rectal sensibility. Rectoanal inhibitory reflex was present.
Dynamic MRI: Important rectal prolapse and pelvic descent without changes. The evacuation after the introduction of the endorectal contrast was complete (Fig. ).
The patient referred less soiling, but defecatory diary is not evaluable due to the regular use of a rectal irrigator device.


A previously healthy 16-year-old boy presented with generalized rash and severe abdominal pain, followed by fever and abnormal liver function within 4 d. Chickenpox was suspected, and intravenous acyclovir was started at a dose of 10 mg/kg/d. On admission, the patient was stable, alert, and oriented to person and place. The temperature was 38.5°C, the blood pressure 125/65 mmHg, the pulse 105 beats per minute, the respiratory rate 22 breaths per minute, and the oxygen saturation 99% while he was breathing ambient air. There were papulovesicular rashes on the face and trunk, with various stages of development including maculopapules, vesicles, pustules, and crusts (Figure and B). Extensive ecchymosis was noted on the lower abdomen and thighs (Figure ). The patient’s liver function deteriorated overnight with coagulopathy and grade 2 encephalopathy. The white blood cell count was 14.3×109/L; hemoglobin 118 g/L; platelet count 44×109/L; alanine transaminase 6499 IU/L, aspartate transaminase 8496 IU/L; total bilirubin 16.8 μmol/L; albumin 31.6 g/L; lactate dehydrogenase 12290 IU/L; international normalized ratio 1.65; and prothrombin activity 45%. An ultrasound of the abdomen showed splenomegaly, but neither hepatomegaly nor ascites.
The patient was diagnosed with ALF. A thorough investigation for an etiology was performed. Markers for hepatitis A, B, C, and E virus infection and for human immunodeficiency virus infection were negative. Antinuclear, anti-smooth muscle and anti-mitochondrial antibodies were negative. There had been no recent travel, illicit drug or alcohol use, or herbal medicine intake. In view of the recent onset rash, we tested the blood and blister liquid for herpes group viruses. The polymerase chain reaction (PCR) amplifications of Epstein-Barr virus (EBV), herpes simplex virus (HSV1, HSV2), cytomegalovirus (CMV), and human herpes virus 6 were negative, but varicella-zoster virus (VZV) PCR amplifications were positive, both in the blood and blister liquid. The patient recalled that one month before admission, a number of his schoolmates had developed chickenpox.
The patient was thus deemed as having varicella-induced ALF, and underwent a combination therapy of acyclovir (10 mg/kg every 8 h, for 10 d), intravenous immunoglobulin, and multiple transfusions of platelets and fresh-frozen plasma. On the 4th day of admission, his liver enzymes started to decline (Figure ) but pancytopenia developed (Figure and B). This prompted us to consider HLH. Further investigation revealed markedly high serum ferritin (69670 ng/mL) and hypofibrinogenemia (148 mg/dL), though bone marrow biopsy showed no evidence of hemophagocytosis. He met the diagnostic criteria for HLH according to the HLH-2004 guideline (Table )[], and was treated with dexamethasone and etoposide on the 5th day. Imipenem was added in this high-risk neutropenic patient, and de-escalation strategy was applied in the following days. His abdominal pain abated dramatically by the 8th day, the ecchymosis gradually resolved (Figure ), and the skin lesions regressed (Figure and E). Meanwhile, the laboratory values continued to improve (Figure ). The recovery was uneventful, and he was discharged from the hospital. The patient remained healthy without a recurrence of HLH during a 3-year follow-up.


A 23-year-old female, 10 years previously, developed a spontaneous left sided hemothorax. She was also found to have left rib lytic lessions.
Pleural and rib biopsies led to the diagnosis of GSS.
She received radiotherapy treatment focused on the left hemithorax. At age 18, she had a car accident and an upper thoracic kyphosis of 50 degrees without dislocation was diagnosed. Her neurological examen revealed a Frankel D score, with mild weakness and numbness on her left leg. She refused surgery at that time.
Eighteen months later, she presented with a paraparesis (Frankel B), due to a spontaneous (no trauma) T3-T4 fracture dislocation resulting in severe spinal cord compression [].
With halo traction, she underwent reduction, decompression, and C5 to T8 instrumented fusion [Figures and ].
Postoperatively, the patient improved to Frankel D, ambulating 2 years after surgery with only mild dysfunction.


A 53-year-old woman was referred to our department for an anterior neck mass. Prior
to presentation, she underwent fine-needle aspiration (FNA) biopsy, ultrasound, and
computed tomography scanning after the mass did not resolve with antibiotics.
Ultrasonography and computed tomography scan were notable for a hypervascular solid
mass measuring 3 × 2.5 × 1 cm anterior to the sternocleidomastoid muscle with a
large inferior feeding vessel (). The outside biopsy demonstrated spindled epithelioid cells in a background
of blood and was consistent with a benign vascular lesion. The patient was then seen
at our institution.
At our initial evaluation, the patient noted mild enlargement of the mass and
discomfort following biopsy. She denied prior neck trauma and surgery, as well as
voice, breathing, and swallowing changes. Her examination was significant for a
mobile right neck mass without adherence to underlying tissues. There was no
palpable lymphadenopathy or changes in the overlying skin. Flexible endoscopy
demonstrated bilaterally mobile true vocal cords. The remainder of her examination
yielded negative findings for other head and neck lesions.
Imaging and pathology were reviewed with the patient, and excision for definitive
diagnosis and treatment was recommended. Intraoperatively, the mass was noted to be
superficial to the platysma, bluish in color, well encapsulated, and hypervascular,
with multiple feeding blood vessels and a large amount of clot within the capsule.
All feeding vessels were ligated; the mass was completely excised and sent for
routine pathologic evaluation.
At 2 weeks postoperatively, the patient was noted to have a small seroma, which was
drained in office. She otherwise did well without recurrence of the lesion. Final
pathology showed a 3-cm mass containing intravascular papillary endothelial
hyperplasia consistent with IPEH ().


An 8-year-old Emirati boy was admitted to Severance Hospital as a marrow HPC donor for his sister in June 2017. He had no medical history. On admission, the results of physical examinations and laboratory tests including infectious disease testing showed that he was suitable for donation. His blood types were A, DCce, Le(a+b−), K−, Jk(a+b+), M+N−S−s+, and Fy(a−b−). The donor had no transfusion history. However, antibody screening test with ID-Diacell I, II (Bio-Rad, Cressier, Switzerland) and identification test (IDT) with ID-DiaPanel (Bio-Rad) using LISS/Coombs ID-card (Bio-Rad) based on the column agglutination test method showed moderate positivity (2+) in all three screening cells and 11 identification panel cells with negative reactions to autologous red blood cells (RBCs) in anti-human globulin phase. An IDT using enzyme (papain)-treated panel cells (ID-DiaPanel P, Bio-Rad) showed negative reactivities with all panel cells. The donor was suspected of harboring antibodies to high-prevalence antigens, and he showed Fy(a−b−) phenotype, which is extremely rare in Korean donors. Therefore, the donor donated two autologous RBC components before marrow harvest, because it could not be ruled out that the donor's antibody was anti-Fy3, which was expected to show incompatibility with almost all RBC components from Korean donors. The specimens from the donor with informed consent were sent to the Korean Rare Blood Program (KRBP) for genotyping of extended blood groups. The genotyping was performed using a Lifecodes RBC/RBC-R typing kit (Immucor, Norcross, GA, USA). The expected RBC phenotype based on genotyping is summarized in . These results were consistent with the phenotype tested using serologic methods, except the Duffy phenotype. Because the result of Duffy genotyping was FY*02/FY*02N.01 based on polymerase chain reaction–restriction fragment length polymorphism analysis, the expected Duffy phenotype of the donor was Fy(a−b+). In addition, the specimen of the donor was sent to the central laboratory of the Swiss Red Cross in Bern, Switzerland for further tests. In that laboratory, the anti-Ge antibody was identified. A negative result for Ge2 antigen was also shown using sera including anti-Ge2. The donor received two autologous RBC transfusions after successful marrow HPC collection, and the donor was discharged without requirement of allogeneic RBC transfusion during his hospitalization. This study was approved by the Institutional Review Board of Severance Hospital (approval number: 4-2018-0437).


A 35-year-old man, who was a farmer, presented with an extra nail plate over the left middle finger following a sharp injury at the same site 6 years back. Three months following the injury, he had noticed a nail-like outgrowth projecting from the proximal nail fold (PNF), which slowly grew just like the underlying nail plate, but was much thicker and deformed. This ectopic nail produced discomfort while working, although there was no pain or swelling.
On physical examination, a well-defined nail plate distinct from the classical nail plate was found to arise from the PNF. It was rough and pigmented. The underlying normal nail plate showed a linear depression and an altered curvature, corresponding to the overlying ectopic nail plate []. No bony deformity was apparent.
Dermatoscopic examination of the nail (onychoscopy) showed an ectopic nail structure, distinct from the normal underlying nail plate, with an evidence of hematoma within its structure. It was arising from its very own, well-formed PNF with normal nail fold capillaries; however, the cuticle was absent. This indicated that the ectopic nail matrix was probably situated in an invaginated pocket under the dorsal layer of the PNF.
A clinical diagnosis of acquired onychoheterotopia was made. As the ectopic matrix was seemingly placed underneath the dorsal layer of the PNF, we planned to avulse the ectopic nail plate with total matricectomy of the ectopic matrix. The procedure was explained to the patient and a written informed consent was obtained.
Under aseptic conditions, a proximal digital block was administered using 2% lignocaine without adrenaline. The digit was then exsanguinated and tourniquet was tied at the base of the digit to achieve an avascular operating field. The ectopic nail was avulsed [], exposing the ectopic matrix []. This was followed by total matricectomy of this ectopic germinal matrix with 88% phenol using cotton-tipped applicators (1min of application time) to ensure complete destruction of the displaced nail matrix []. The wound was then covered with nonadherent dressing. Postoperatively, oral antibiotics and analgesics were prescribed. The wound healed with a cosmetically acceptable outcome []. Histopathological examination revealed a well-formed nail plate with germinal matrix without a granular layer. No recurrence or spicule formation was reported over a further 6-month follow-up period.


The patient is an otherwise healthy, 18-year-old male with a body mass index of 37 kg/m2, who presented with chronic bilateral axillary, bilateral thigh, and posterior neck HS lesions. He had failed medical treatment including injectable tumor necrosis factor antibody therapy and presented for wide radical excision of his axillary lesion with a plan for later skin grafting by plastic surgery.
He was previously treated with adalimumab (Humira), a tumor necrosis factor antagonist, without resolution. He was brought to the operating room for excision of his right axillary lesions. General anesthesia was induced with 4 mg midazolam, 150 μg fentanyl, 80 mg lidocaine, 200 mg propofol, and 50 mg rocuronium. An 8.0 mm endotracheal tube was inserted through the vocal cords. Anesthesia was maintained using oxygen, air, and sevoflurane. Additional 400 mg propofol, 20 μg dexmedetomidine, and 100 μg fentanyl were administered in total during the surgery. Patient received 1 g intravenous acetaminophen, 30 mg ketorolac, and 1 mg hydromorphone for pain control. Four milligrams of ondansetron and 4 mg of dexamethasone were administered, and the patient was extubated successfully after reversal of paralytic with 4 mg of neostigmine and 0.8 mg of glycopyrrolate. Surgery involved excision of skin and subcutaneous tissue of the axilla and placement of a negative pressure wound vacuum.
The surgery team requested the regional anesthesia team to perform a block to control postoperative pain, as axillary resection of the soft tissue was extensive. Prior to placement of ESB, patient was awakened from general anesthesia and he recovered in the post-anesthesia care unit (PACU). He was evaluated by the regional anesthesia team 37 minutes after his arrival to the PACU, at which time he was fully awake and oriented to person, place, and time, and his self-reported pain score was 8 on the numeric rating scale.
We performed an ESB in the PACU, with the patient lying in left lateral decubitus position. The patient’s back was palpated for landmarks; the seventh cervical spinous process (C7) was identified and marked, and used to count caudally to thoracic vertebra 2 (T2). The T2 spinous process was identified and marked. A high-frequency linear transducer (HFL50; 15–6 MHz) was placed on the back, slightly to the right of midline in a coronal plane. The transducer was translated medially until the transverse processes (TPs) were identified, and then cephalad to identify the T2 TP, lateral to the marked T2 spinous process. Skin was then sterilized with ChloraPrep™ skin prep solution (2% w/v chlorhexidine gluconate in 70% v/v isopropyl alcohol; BD, Franklin Lakes, NJ, USA), and a 20-gauge, blunt-tipped, 100 mm echogenic block needle (B Braun, Bethlehem, PA, USA) was advanced in-plane from caudal to cranial to make osseous contact with the T2 TP. At this point, a small volume of saline was injected to assess for satisfactory spread of fluid underneath the erector spinae muscle (ESM) and above the TP. A volume of 30 mL of 0.25% bupivacaine hydrochloride (75 mg) with 3 mg of preservative-free dexamethasone was then injected under direct ultrasound visualization (Sonosite X-Porte; SonoSite, Bothell, WA, USA). Craniocaudal spread was noted below the ESM ().
Following block placement, he had a marked decrease in axillary pain, from the aforementioned pain score of 8 to 0 on the numeric rating scale, 25 minutes after completion of the ESB. He did not require any narcotics or adjuncts for pain in the recovery room. He was maintained on scheduled acetaminophen 1g every 6 hours, celecoxib 200mg every 12 hours, and gabapentin 300mg every 8 hours. Tramadol 100mg was given every 6 hours as needed for breakthrough pain. Neurological assessment to pin prick was performed, and diminished sensation was noted on the right side over the shoulder, scapula, axilla, and anterolateral chest wall ( and ). Handgrip, flexion and extension at wrist and elbow, and abduction and adduction at the shoulder were intact.
The patient underwent two more debridements of the axilla and placement of negative pressure wound vacuum at days 3 and 6 after his initial surgery under general anesthesia.
He requested the regional team perform the same block, as he was satisfied with the analgesia. ESB was performed in a similar fashion as described above with similar analgesic efficacy. Average time to first narcotic consumption after each T2 ESB was 39.3 hours.
This patient was receiving chemical venous thromboembolic (VTE) prophylaxis, beginning after his first surgery. He was receiving scheduled enoxaparin 40 mg once daily at the time of the second and third ESB.


An 8 days old exclusively breastfed female infant was referred to our hospital because of an infection of the umbilicus without fever. She was the second child of nonconsanguineous parents, both of Arab-Berber descent, born after 39 weeks of pregnancy, complicated by intrauterine growth restriction. Birth weight 2.570 kg (SD –1.6), length 48 cm (SD –0.8), and head circumference 32 cm (SD –1.7). The mother was known to have Crohn’s disease, treated with oral methylprednisolone, in a gradually reducing dose with a maximum of 32 mg daily, and adalimumab, 40 mg subcutaneously every 2 weeks for up to 3 months before delivery. Additional investigations revealed a latent tuberculosis (positive interferon gamma release assay), for which she used INH 300 mg once a day, in combination with pyridoxine 125 mg, which both were started immediately after delivery. Family history is negative for hematologic diseases, syndromes, or early unexplained death.
Physical examination of the neonate was, besides a local infection of the umbilicus, normal for her age. No (skeletal) malformations, cutaneous, or nail abnormalities; hepato-splenomegaly; or hypotonia were noted. However, complete blood count showed a picture of severe isolated neutropenia (hemoglobin 18.2 g/dL, mean corpuscular volume 97, plate-lets 254×109/L, leukocytes 7.56×109/L, and absolute neutrophil count [ANC] 0.04×109/L; C-reactive protein 73.1 mg/L). The child was admitted and broad-spectrum intravenous antibiotic treatment (ampicillin and cefotaxime) was started. Cultures of the umbilicus revealed the growth of Staphylococcus aureus. Urine and blood cultures remained negative.
The infection improved; however, the isolated neutropenia persisted. No viral etiology (TORCH, which includes Toxoplasmosis, Other [syphilis, varicella-zoster, parvovirus B19], Rubella, cytomegalovirus, and herpes infections, [para] influenza, respiratory syncytial virus, adenovirus) could be demonstrated. Testing for antineutrophil antibodies was not done, as the tests often show false-positive and false-negative results. Bone marrow aspiration revealed a severe dysgranulopoiesis, characterized by a maturation stop after meta-/myelocyte stage (), indicating severe congenital neutropenia. Maternal usage of adalimumab during pregnancy can cause neutropenia because it can cross the placenta from the maternal circulation into the fetal circula tion. Also, agranulocytosis due to INH, used by the mother while breast-feeding, could not be excluded. Tuberculosis was excluded in the child, and the complete blood count of the mother did not show neutropenia. As a probability scale, we use the Naranjo algorithm, and this case was scored for both medications separately. INH was scored “3,” while adalimumab was scored “4,” both as a “possible” likelihood to be responsible for the neutropenia (Naranjo scores: 9 or 10 indicate “definitely”; 5–8 rate the likelihood as “probable”; 1–4 “possible”; <1 “doubtful”).
Breast-feeding was terminated, and filgrastim (Neupogen®, Amgen Inc., Thousand Oaks, CA, USA) 5 µg/kg subcutaneously was started. A very slow increase of ANC was seen, and so filgrastim dose was increased to 10 µg/kg subcutaneously, with good improvement. ANC increased to a maximum of 35.85×109/L. Two months after birth, filgrastim was terminated which, initially, led to a decrease of ANC, before it stabilized in the normal range (). Two weeks after terminating, the filgrastim a new bone marrow aspiration () and a complete blood count was repeated. Both were normal, which excluded severe congenital neutropenia.


A 39-year-old male patient presented to the Gastroenterology Department complaining
of intermittent rectal bleeding for almost a year, without changes in bowel habits,
abdominal pain nor any weight loss. His only comorbidity was allergic asthma
diagnosed since he was 20 years old, for which he had been taking short-acting
bronchodilators. He was a non-smoker and did not acknowledge alcohol consumption.
Clinical examination revealed a normal weight patient, in good physical condition,
without skin pallor, with blood pressure 120/80 mmHg, pulse rate 68 bpm, and no
abdominal pain on palpation. Laboratory investigations showed values between normal
ranges, including haemoglobin levels, liver biochemistry, and kidney function tests.
Colonoscopy was performed which revealed a rectal tumour located between 3 and 6 cm
from the anal verge (Figure 1a). Histopathological examination of the endoscopic
biopsies demonstrated a moderately differentiated (G2) invasive adenocarcinoma.
Further diagnostic workup included a transrectal endoscopic ultrasonography (EUS) by
using a radial frontal view echoendoscope for local and regional staging, which
showed a hypoechoic mass located on the posterior rectal wall with mural invasion
limited to the muscularis propria (T2) (). No
lymph nodes were visualized in the tumour vicinity (N0), nor around the iliac
vessels. An increased intratumoural vascular signal was detected during Doppler
studies, and contrast examination was added to the EUS revealing homogenous arterial
tumour enhancement and wash-out in the venous phase (,).
Magnetic resonance imaging (MRI) of the abdomen and pelvis was also performed which
showed a fatty liver with only a 4 mm cystic lesion in the fifth segment and
thickening of the posterior rectal wall mucosa, without any suspicious lymph nodes
or peritoneal fluid. Carcinoembryonic antigen levels were between normal ranges.
After consultation with an oncologist the patient was referred for chemoradiotherapy
(CRT) with intent to avoid extensive surgery. External radiotherapy was applied in a
total dose of 50.4 Gy, 1.8 Gy/fraction by using the volumetric modulated arc therapy
(VMAT) technique. Concomitant radiosensitizing chemotherapy with oral capecitabine
was administered.
Four weeks after completing CRT we performed EUS for restaging purposes which showed
only slight thickening of the mucosa on the posterior rectal wall, just above the
anal canal but with all ultrasonographic layers preserved. The anal sphincter
appeared normal and also there were no visible perirectal lymph nodes. On endoscopic
examination a scar was visible above the dentate line with no residual macroscopic
tumour tissue (,). Multiple biopsies were taken which showed chronic inflammation,
some atypia which were classified as reactive, and dilated cystic glands. At MRI
examination slight thickening of the posterior rectal wall was also noted post-CRT,
but with no suspicious enhancement.
After consultation with the oncologist and surgeon, transanal endoscopic
microsurgery was performed 10 weeks after completing CRT, with excision of a
fragment of the rectal wall and mesorectum. Histopathological examination of the
surgical sample revealed areas of well differentiated adenocarcinoma infiltrating
the muscular layer and the perirectal fatty tissue. There were no signs of
perineural, lymphatic or vascular invasion and the resection margins were tumour
free. Consequently the case was classified as ypT3NxMx, and further extensive
surgery was recommended.


A 55-year-old woman presented with headache, dizziness and gait imbalance for 2 months. Patient’s history revealed a posterior fossa tumor that had been diagnosed 4 years ago prior to admission to our clinic. At that time, contrast enhanced of magnetic resonance imaging (MRI)showed the ill defined lesion, 5.5×3.5×4 cm in size without perilesional edema (Fig. A,B). During this 4 years period, her follow-up had been performed with serial MRI (Fig. A and B) and CT assessments (Fig. ). Until 2 months ago, she had only intermittent headache. On neurological examination, she had cerebellar signs including ataxia and also bilateral papilledema through ocular investigations. CT assessment showed punctate calcified lesion demonstrated with undifferentiated margins of the lesion from the cerebellar parenchyma. The last MRI findings showed heterogeneous ill defined lesion without perilesional edema in vermis and extending to the adjoining cerebellar hemisphere at the supracerebellar infratentorial space with a dimension of 6.7×4×4 cm size causing compression of the fourth ventricle and marked hydrocephalous. The patient underwent surgery through a midline supracerebellar infratentorial approach in the prone position. It was a soft whitish intra-axial tumor, moderately vascular with a good plane between the vermis and cerebellum. A complete resection was achieved. The cerebrospinal fluid shunt was not decided to be placed (Fig. ). Pathological diagnosis was reported as CLN. Immunohistochemical study, synaptophysin and neuron-specific enolase (NSE) immunopositivity were found (Fig. ). In the mean time, glial fibrillary acidic protein (GFAP) was immunonegative and Ki-67 index was 2%. After operation, she had uneventful postoperative period and was discharged on seventh day.