A 39-year-old female presented to the outpatient clinic with the complaints of recurrent painless swelling in the left thigh and had a history of surgery twice for its removal. She was apparently well until 2001 when she noticed a painless swelling over left mid-thigh. The swelling progressively increased in size and she underwent surgical excision in 2005. She remained asymptomatic for the next 2 years when she again noticed a new swelling over the left distal thigh which was excised surgically in 2009. She remained asymptomatic for the next 3 years when she had a recurrent swelling over the left distal thigh. The swelling was progressively increasing in size and was associated with some discomfort. At the time of presentation to us, she had a large, oval, around 8 cm × 4 cm size, painless swelling over the medial aspect of the left distal thigh. There was a healed scar over the left medial thigh with quadriceps wasting. The swelling was intramuscular on clinical examination. Systemic examination was unremarkable. She was further evaluated with radiographs of the thigh which was suggestive of fibrous dysplasia. This led us to go for a thorough skeletal survey, which showed the involvement of the left femur and tibia (). She was also evaluated with magnetic resonance imaging (MRI) of whole of the thigh which showed two additional soft tissue masses; one in left mid-thigh and the other in the left inguinal region (). As the previous biopsy report was suggestive of myxoma, clinical diagnosis of Mazabraud’s syndrome was made. The patient decided for operative management of the large distal thigh symptomatic swelling and conservative management for the remaining two small, asymptomatic swelling. After informed consent, she underwent wide excision of the distal thigh swelling through the previous skin incision. The mass was intramuscular, located within vastus medialis. A single, globular, pale white, encapsulated nodular mass measuring 8 cm × 4 cm × 3 cm was excised (). The mass weighed around 88 g. Histopathological examination was suggestive of intramuscular myxoma (). No necrotic areas or atypical mitotic figures were identified, ruling out any malignancy. The patient was managed with weekly oral bisphosphonates for fibrous dysplasia, which was stopped after 1.5 years. A regular MRI every 6 months was advised to assess the recurrence and interval enlargement of the remaining two lesions. On follow-up, MRI was done 6 months postoperatively, and these two masses seemed to have slightly increased in size; however, the patient remained asymptomatic (). At latest follow-up after 2 years, the patient was pain free with no recurrence and the MRI showed decrease in the size of the mid-thigh lesion and minimal increase in the size of the left inguinal lesion ().


A 55-year-old male with an L2 wedge compression fracture first underwent an L1–L3 pedicle screw fusion elsewhere. For persistent increasing back pain, progressive pseudoarthrosis, vertebral body collapse, and focal kyphotic angulation, he required an MI lateral corpectomy [: Case 1].
This included partial resection of the 12th rib, placement of an interbody expandable cage, and revision of the posterior fusion using T12 to L4 percutaneous pedicle screw fixation [: Case 1]. He remained asymptomatic 6 months later.


A 63-year-old female with multiple myeloma on chemotherapy presented with a pathological L3 vertebral body compression fracture presenting with worsening low back pain limiting her daily routine activities. The lumbar magnetic resonance imaging revealed 50% reduction in the L3 vertebral body height with dual-energy X-ray absorptiometry revealing osteopenia [: Case 2 and : Case 2].
She underwent an L3 MI lateral corpectomy using an interbody expandable cage, supplemented with polymethyl methacrylate followed by percutaneous posterior pedicle screw fixation from L2 to L4 [ and : Case 2]. She too was ambulated on post operative day 1. Follow-up X-rays confirmed adequate implant position and fusion across L2–L4 [: Case 2].


A 45-year-old female with type II diabetes presented with a 5-month history of severe back pain. She was a homemaker who kept pigeons as pets. Her laboratory investigations revealed an erythrocyte sedimentation rate (ESR) of 38 mm/1st h and a C-reactive protein (CRP) of 18 mg/l. The magnetic resonance imaging (MRI) demonstrated T4 osteomyelitis with relatively well-preserved intervertebral disc spaces []. Although fine-needle aspiration cytology was inconclusive, the patient was presumptively placed on antitubercular drugs. One month later, she became fully paraplegic. At that point, the MRI demonstrated osteolytic lesions of the T4 vertebral body with cord compression, accompanied by bilateral paraspinal soft tissue masses extending to the T5-T6 level; there was, however, no disc space involvement []. She underwent a biopsy of the T4 vertebral body, accompanied by transfacet T4 decompression, and posterior T2- T6 pedicle screw instrumentation [].
The culture and histopathological examinations revealed a cryptococcal fungal infection that was sensitive to amphotericin B; this was administered intravenously (150 mg/day) for the next 3 months []. However, the patient developed an abscess at the surgical site 2 weeks later, which required drainage. Three weeks postoperatively, she also developed the sudden loss of vision bilaterally; the ophthalmological examination revealed bilateral retinal fungal deposits and endophthalmitis. One eye’s vision recovered after intravitreal voriconazole, but she lost vision in the other eye despite vitrectomy. The amphotericin B antibiotic regimen was then supplemented with flucytosine (i.e., 500 mg thrice a day) until the 3rd postoperative month.
Three months following the spinal surgery, she regained 4/5 function in both lower extremities. Although the MRI still showed some prevertebral collection compared with the preoperative MRI, there was resolution of the T4 cord compression []. Eight months postoperatively, the patient could walk with a cane but had residual unilateral loss of vision (perception of light only). Furthermore, the ESR and CRP levels both normalized. In addition, the MRI now showed complete resolution of the prevertebral abscess and the previously abnormal signal within the T3-T5 vertebral bodies []. After 3 postoperative months passed, intravenous amphotericin B was stopped, but she was kept on oral fluconazole 400 mg once a day, and flucytosine 500 mg thrice a day for 5 months.


A 66-year-old woman was referred to a hospital near her residence for a chief complaint of left orbital swelling. There was a tumor originating from the left ethmoid sinus, and biopsy from the nasal cavity revealed a histopathological diagnosis of malignant melanoma. The patient was then referred to our hospital one month later, for radical therapy against the tumor. Computed tomography (CT) image revealed that the tumor was located in the left ethmoid sinus invading the left orbital cavity and skull base (Figure A), and lung metastasis was suspected (data not shown).
The patient underwent carbon-ion radiotherapy (CIRT) in another hospital from April to May, 2016. The total dose of carbon-ion beam was 57.6 Gy/16 fr, and 1 course of DAV (DTIC 120 mg/m2: day 1-5, ACNU 70 mg/m2: day 1, VCR 0.7 mg/m2: day 1) was carried out during CIRT. Although the ethmoid sinus tumor showed good regression (Figure B), the metastases of the lung and mediastinum still progressed (Figure A, left panel).
The patient was then referred to our hospital to treat the lung and mediastinal metastatic tumors. Nivolumab injection was administered in June 2016, and a 2 mg/kg injection (basically tri-weekly) following the manufacturer's instruction was continued since then. The injection was administered thrice in August, and the ethmoid sinus tumor further regressed (Figure C) along with complete disappearance of the lung and mediastinal tumor (Figure A, right panel).
Occlusion of the left retinal artery due to CIRT occurred in March 2017. The patient was referred to the ophthalmologist, aspirin was prescribed, and there have been few complaints of mild epistaxis since then.
After nivolumab was injected 16 times, mild hyperthyroidism occurred and recovered in June 2017.
After injecting nivolumab 24 times in April 2018, IP occurred suddenly (Figure B) and nivolumab injection was stopped. Figure shows the serum sialylated carbohydrate antigen (KL-6) levels throughout the therapy. Prednisolone therapy was started with the first dose of 80 mg/d and then tapered down step-by-step (Figure ).
Although the IP improved 8 weeks after the prednisolone therapy began, lung metastases reappeared in August 2018 when the prednisolone dose was 10 mg (Figure C, left panel and Figure ). The locations of newly developed metastatic tumors were different from the previous metastatic tumors 2 years ago, with no recurrent tumors in the previous metastatic sites. However, these metastases disappeared again in November when the prednisolone dose was 7.5 mg/d (Figure C, right panel, Figure ). Although laboratory data indicated that number of the white blood cells was within normal limits from the beginning of prednisolone administration, number of the neutrophils had been increased and number of the lymphocytes had been decreased until the dose of prednisolone was decreased. Serum levels of KL-6 also decreased (Figure ). The patient is currently on low dose prednisolone and is in good health without any tumor recurrence.


A 9-year-old, white female child presented with abdominal pain and diarrhea mixed with bright red blood lasting for 2 days followed by hematemesis. Parents denied fever, foreign bodies, or caustic ingestion. The patient's medical and familial history was negative. The physical examination was unremarkable except for pallor and tachycardia (110 beats/min). Routine laboratory tests were within the normal limits, except for microcytic anemia (hemoglobin concentration: 8.3 g/dL, with mean cell volume of 78 fl).


A 58-year-old male had developed exudative otitis media 2 months prior to his admission. He had had pain in the left maxillary third molar, which had been gradually progressing over 1 month. The symptoms had developed on the left side of the head 1 week prior to his admission. On admission, his temperature was 36.9°C. Physical examination showed normal findings. Neurological examination revealed paresthesia and pain in his left face and head. His motor function was intact. All deep tendon reflexes were normal. Bilateral Babinski reflexes were absent.
Laboratory tests showed a white blood cell count of 6,100/μL and a C-reactive protein level of 9.45 mg/dL, with an erythrocyte sedimentation rate of 134 mm. The serum procalcitonin and β-D-glucan levels were normal (procalcitonin: 0.04 ng/mL; β-D-glucan: 8.5 pg/mL). The serum PR3-ANCA level was elevated to 84.8 U/mL. Other autoimmune markers, such as antinuclear antibody, anti-DNA antibody, rheumatoid factor, myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA), and anti-cyclic citrullinated peptide, were unremarkable. Urine examination showed no proteinuria. Cerebrospinal fluid analysis showed normocytosis with an increased protein concentration of 82 mg/dL. A cerebrospinal fluid culture was negative.
Brain magnetic resonance imaging revealed bilateral paranasal sinusitis on the T2-weighted image, left frontal lobe edema on fluid-attenuated inversion recovery (FLAIR) imaging, and a thick dura mater with abnormal enhancement in the frontotemporal lobe (Fig. , arrows). Magnetic resonance venography detected stenosis of the superior sagittal sinus (Fig. , arrow).
The patient was diagnosed as having GPA associated with HP and cerebral venous thrombosis. He was treated with 3 courses of intravenous methylprednisolone (1,000 mg/day for 3 days) and 10 mg/day apixaban. He was followed by daily prednisolone (30 mg) and cyclophosphamide (50 mg). His C-reactive protein level had normalized by 25 days after admission. The bilateral paranasal sinusitis on T2-weighted imaging, left frontal lobe edema on fluid-attenuated inversion recovery imaging, diffuse enhancement of the dura matter in the frontotemporal lobe, and stenosis of the superior sagittal sinus had improved by 30 days after admission (Fig. , arrow). His symptoms had disappeared by 33 days after admission. Upon discharge, 34 days after admission, the dose of prednisolone was tapered.


A 13-year-old previously healthy boy presented to the emergency department (ED) in August 2018 with neck pain and left sided facial drooping which started on the day of presentation. He had history of nasal congestion, cough, and fever for five days. He was initially diagnosed with Bell’s palsy and started on prednisone. The patient returned to the ED two days later with new onset right shoulder and arm weakness. He was tachypneic to 32 breaths/minute, tachycardic to 108 beats/minute, and afebrile with normal blood pressure. He was alert and oriented with Glasgow coma scale 15/15. Neurological examination was significant for left facial drooping and inability to fully close his left eye. He had neck weakness as well and was unable to hold his head unsupported. Pupils were equal and reactive to light. Muscle strength in the right upper extremity was 1/5 proximally and 4/5 distally. He had normal strength of left upper extremity and both lower extremities. Reflexes were absent in right biceps and brachioradialis, but normal on the left side. There was no sensory disturbance or numbness. There were no gait abnormalities and no bladder or bowel incontinence. The rest of his physical examination was normal. Cerebral spinal fluid (CSF) analysis showed mild pleocytosis of 18/mm3 with 76% lymphocytes and 22% monocytes. CSF protein 52 mg/dL, and glucose 61 mg/dL. Contrast enhanced magnetic resonance imaging (MRI) of brain and cervical spine revealed cord swelling with T2 prolongation and without contrast enhancement extending from the brain stem at the level of middle cerebellar peduncles through the medulla and the cervical spinal cord to the C7 level. No other area of abnormal signal was present ( and 2). These findings are most consistent with longitudinally extensive transverse myelitis (LETM) due to involvement of ≥3 vertebral segments.
Extensive CSF studies were performed including antibodies for arboviral encephalitis (West Nile, St. Louis, California, Eastern Equine, and Western Equine), polymerase chain reaction (PCR) for herpes simplex virus and enterovirus, Lyme antibodies, and oligoclonal bands. All were negative. His nasopharyngeal swab was also negative by PCR for adenovirus, enterovirus/rhinovirus, human metapneumovirus, parainfluenza, influenza, respiratory syncytial virus, coronavirus, Chlamydia pneumoniae, and Mycoplasma pneumoniae. Serum aquaporin 4 receptor antibody, antinuclear antibody, and antidouble strand DNA were negative as well. Stool viral culture was negative as well as rectal swab for enterovirus by PCR. His CSF M. pneumoniae antibodies were elevated; IgG 2.31 U/L and IgM 2.08 U/L. Reference ranges are: less than 0.09 and 0.76, respectively. A three-day course of high dose intravenous methylprednisolone (30 mg/kg/day) was started, followed by plasmapheresis due to lack of improvement on steroids, in addition to doxycycline (to treat his Mycoplasma infection). The patient was monitored closely in the intensive care unit. His respiratory status remained stable and he did not require ventilatory support. He did not develop any signs of autonomic instability such as bradycardia or hypertension. He had no urinary retention or constipation. He was fed initially through nasogastric tube due to development of dysphagia. Video fluoroscopic swallowing function study was performed a week after admission to the hospital and showed no tracheal aspiration.
Subsequently, the patient could feed orally without complications. The patient was hospitalized for two weeks to recover. At the time of discharge, he had mild improvement in his right upper extremity motor functions, he was able to walk independently and he had some improvement in his facial palsy. He received extensive physical therapy as an outpatient. At 2 months follow up visit, he had minimal weakness in his right hand and left face with complete resolution of his neck weakness.


A 47-year-old Thai man presented with severe ocular pain and decreased vision in the left eye lasting for 4 days. He had been diagnosed with active PV 3 weeks earlier, for which he had been treated with oral prednisolone for 2 weeks. Because of the only slight improvement of the skin lesions, the patient then was treated by intravenous dexamethasone (5 mg every 6 h) with prophylaxis doses of intravenous cloxacillin (1 g every 6 h) for 1 week during hospitalization. Although there was a significant improvement of the skin lesions, the patient started to report ocular pain and blurred vision in the left eye. The pain, redness, and swelling around the upper and lower eyelids progressed so rapidly that the patient could not open his eye. The vision reduced to light perception in 4-day time. After stepping the antibiotics up to intravenous imipenem (500 mg every 6 h), his symptoms gradually worsened. Then, he was referred to our hospital.
Physical examination on admission revealed high body temperature (38.1°C) as well as skin lesions with multiple discrete flaccid blisters and oval-shaped erosions covered with hemorrhagic crust distributed along the trunk and extremities. Some of the lesions had healed as postinflammatory hyperpigmented patches without evidence of cellulitis or pustules (Fig. ). On ophthalmic examination, periorbital erythema and marked soft tissue swelling with hyperpigmented flaccid bullous were noted at the left upper eyelid (Fig. ). His visual acuity was reduced to light perception with poor light projection in the left eye and 20/30 in the right eye. Intraocular pressure in the left and right eyes were 46 and 16 mm Hg, respectively. Slit-lamp examination demonstrated severe chemosis with a yellowish discharge, totally haze cornea, a generalized shallow anterior chamber with marked anterior chamber reaction, 2 mm of hypopyon, and plasmoid formation. The pupil of the left eye was slightly reactive to light and positive for relative afferent pupillary defect. The patient's fundoscopic view was obscured. The extraocular muscles were limited in all directions of gaze. The right eye was unremarkable.
Ultrasonography of the left eye showed generalized opacity of the vitreous and chorioretinal thickening with no evidence of retinal abscess (Fig. ). A computed tomography scan demonstrated left proptosis with periorbital soft tissue swelling, diffused scleral thickening, and retrobulbar fat stranding compatible with orbital cellulitis (Fig. ). The paranasal sinuses were normal. No subperiosteal abscess was documented.
Laboratory examination of renal and liver function was normal. The urinalysis showed no infection. Anti-HIV and diabetic test were negative. Blood culture and skin biopsy results were negative for organisms. Dental examination, nasal endoscopy, and liver ultrasonography were conducted but failed to identify the primary source of infection.
The patient was diagnosed infective panophthalmitis. He was treated with intravenous ceftazidime and vancomycin. Despite the stepping up of antibiotics, the condition worsened. Risks, benefits, and alternatives to pars plana vitrectomy and intravitreal antibiotics injection were extensively discussed. Due to the severe painful eye with extensive orbital lesion, poor visual prognosis after surgery, and low socioeconomic status, the patient finally made the decision to undergo enucleation with glass ball implantation. Moderated growth of A. hydrophila was isolated from the vitreous culture and was susceptible to ceftazidime, gentamicin, and ciprofloxacin. The systemic administration of antibiotics was continued for 10 days then stepped down to oral administration.
The infection was controlled after the enucleation. High doses of oral prednisolone, dapsone and azathioprine were administered in order to treat the active PV. Fortunately, no evidence of fellow eye infection was detected after 1 year of follow-up.


A 62-year-old male with a past medical history remarkable only for hypertension first presented in May 2016 to his primary care physician with left-sided progressively worsening vision associated with discomfort, diplopia worse on right gaze, epiphora, and clear discharge that had developed over the past year. On examination, he had limited extraocular movement in the left eye and was subsequently referred to an optometrist and ophthalmologist. An MRI was performed with evidence of sinus disease, including a likely mucocele (Fig. ). He was referred to Otolaryngology for further evaluation and management.
Endoscopic sinus surgery was discussed and Oculoplastic Surgery was consulted given evidence of orbital invasion on imaging. When the patient was first seen in the Oculoplastic Clinic his vision had declined further to 20/400 (20/300 on pinhole) and there was demonstration of optic neuropathy and relative afferent pupillary defect (RAPD), likely from optic nerve compression by the sinus mucocele, with possible infection. On examination, he exhibited unilateral proptosis and lateral gaze palsy of the left eye (Fig. ). Dilated fundus examination was notable only for venous tortuosity in the left eye.
Given concern for worsening optic neuropathy in the setting of an enlarging ethmoid mucocele with possible super-infection, the patient was admitted for IV steroids and antibiotics, and scheduled for urgent endoscopic drainage. The patient underwent endoscopic sinus surgery with bilateral total ethmoidectomy, mucocele excision, and removal of the compressive effects on the optic nerve on the left without any noted complications.
Histological and immunohistochemical testing noted minimal eosinophils and no fungal organisms, malignancy, or papilloma in any parts of the samples sent. Both the mucocele wall and left sinus samples were significant for bone and fibrotic tissue with focal old haemorrhage, whilst the left sinus sample also showed reactive-appearing histiocyte aggregates.
Post-operatively, his vision was 20/25–2 with persisting RAPD. His vision and motility had significantly improved (Fig. ). There was significant proptosis reduction, with slight ptosis from the resulting enophthalmos. A month later, there was residual mucus discharge from the left nasal cavity. On nasal endoscopy there was expected post-surgical crusting in the ethmoid area. The patient was recommended to continue daily sinus rinses.


A 44-year-old man complained of a recurrent bleeding, erythematous, scaly, papillomatous left lower eyelid lesion for 2 years (Fig. ). Two prior biopsies of this lesion were diagnosed as skin tags. Past medical history included three fatty tumors removed as a child (left forehead, lower abdomen, and back). Histopathology of the most recent shave biopsy showed skin with an intradermal proliferation of basaloid cells surrounding foamy cells, which formed lobules (Fig. ). Many mitotic figures were seen. The epithelium contained hyperkeratosis, parakeratosis, and pagetoid spread of atypical foamy cells, consistent with a well-differentiated sebaceous carcinoma. The remainder of the tumor was removed with Mohs micrographic surgery, and negative margins were confirmed by Oil Red O staining. The defect was closed directly. Though his lesion was stage IA (T1N0M0) based on its size, superficial nature, and his clinical examination, due to the patient's young age and family history of colon cancer (2 maternal aunts), there was suspicion for an associated MTS, and immunohistochemistry (IHC) was ordered for the mismatch repair proteins: MLH1, MSH2, MSH6, and PMS2 (Ventana Medical Systems, Tucson, AZ, USA), along with MSI testing (Life Technologies; Carlsbad, CA, USA).
IHC showed a loss of the mismatch repair proteins MSH2 and MSH6 (Fig. ). Formalin-fixed paraffin-embedded tissue sections were reviewed by a surgical pathologist, who macrodissected tumor and nontumor tissues separately using an automated procedure (Promega, Madison, WI, USA). Multiplex PCR amplification of five mononucleotide microsatellite (BAT25, BAT26, NR21, NR24, and NR27) and two pentanucleotide microsatellite loci (PentaC and PentaD) was performed on the DNA extracted from tumor and nontumor samples. The mononucleotide markers were used for MSI assessment, while the pentanucleotide markers were used to detect potential laboratory errors and/or contamination. Fluorescently labeled amplification products were separated by denaturing electrophoresis and detected using a fluorescent capillary analyzer (Life Technologies). The size profiles of the mononucleotide products were compared directly to those of nontumor tissue. MSI, defined as a change in any length due to insertion or deletion of repeating units within a microsatellite of a tumor compared to nontumor (normal) tissue, was not detected (tumor was MSI-stable). Due to these discordant results, a more detailed patient history was obtained, revealing a family history meeting the Revised Amsterdam II criteria for the diagnosis of Lynch syndrome (Table ; Fig. ) []. The patient was referred to a genetic counselor to coordinate testing for a germline mutation. A heterozygous germline MSH2 mutation (c.1226_1227del) known to result in the premature truncation of the MSH2 protein at amino acid position 415 was identified, confirming Lynch syndrome (COLARIS®, Myriad Genetics, Inc., Salt Lake City, UT, USA).


A 23-year-old male patient who had previously been healthy presented at the university eye clinic complaining about slightly blurred vision and “some spots” in his right visual field. Best-corrected visual acuity (BCVA) was 0.7 in the right and 1.0 in the left eye. Ophthalmic examination showed a mild prominence of the disc in the left eye and a distinct prominence with cotton wool spots and tortuous retinal vessels in the right eye (Fig. ). Additionallycotton wool-like spots/perivascular sheathing (Fig. ), inflammatory cells in the anterior chamber as well as in the vitreous body and cell deposits at the corneal endothelium were found – unmistakably findings of intraocular inflammation (consistent with uveitis and vasculitis in the vitreous and at the disc). Case workup (cooperation of ophthalmology with neurology and internal medicine) concentrated on different pathologies that were associated with a prominent disc and on diagnostic ways to achieve a differential diagnosis (papillitis, papilledema, encephalitis, sinus vein thrombosis, infarcts, intracranial hemorrhages, tumors, pseudotumor cerebri, hypertensive retinopathy [stage IV], Vogt-Koyanagi-Harada syndrome, and multiple sclerosis). A contrast cMRT was performed and encephalitis, sinus vein thrombosis, recent infarcts, and intracranial hemorrhages were excluded.
Blood pressure was regular. The cerebrospinal fluid (CSF) opening pressure values were normal (21 cm H2O) as well as the liquor spaces in cCT and cMRI, thus a pseudotumor cerebri could be excluded. Initial blood findings were unspectacular except for strongly increased D-dimers of 895 ng/mL (norm <500) and calcium in blood 2.62 mmol/L (norm 2.09–2.54). The CSF analysis revealed a leukocytosis of 48/μL (norm 0–4), a slightly increased total protein of 571 mg/L (norm <450) and slightly increased immunoglobulins: immunoglobulin G 45.3 mg/L (norm 10–40) and immunoglobulin A 7.1 mg/L (norm 0.5–6). Lactate with 2.24 mmol/L (norm 1.1–2.4) and glucose with 43.3 mg/dL (norm 40–70) were normal. Inflammatory liquor changes of viral genesis were suspected, therefore, we decided to determine neurotropic pathogens (in serum and CSF) and initiated extended diagnostic procedures to find a reason for the vasculitis. While waiting for further blood serology results, a virostatic agent (acyclovir 500 mg i.v. three times a day) and an antibiotic drug (cefotaxime 2.0 g three times a day) were given. Additional systemic steroids were considered based on serology values.
Three days later the retinal examination with OCT detected a detachment of the neurosensory retina in the macula suspicious of being connected to an optic disc edema (Fig. ). In addition, the ensheathing of veins with perivascular infiltrates in the fundus appeared to be progressive. In the patient's left eye, the disc was still less prominent than in the right eye and the macula was dry. Fluorescein angiography could not be performed because of the patient's circulatory instability. As the cause of the disc edema was still unknown, the workup was extended: a chest X-ray (Fig. ) was performed and additional blood testing was initiated.
The X-rays (Fig. ) showed bihilar shading with donut sign, compatible with a lymphadenopathy, plus bilateral, basally pronounced streaky pulmonary lung patterns. Serology findings were negative for neurotropic pathogens, borreliosis, HSV, CMV, EBV, VZV, HIV, tuberculosis, rheumatic factor, ANA, and ANCA. Coagulation factors, antiphospholipids and liver enzymes were normal and there were no signs of thrombophilia.
Blood results that matched with a sarcoidosis included an increase of IL2R: 1,681 U/mL (norm 158–623), increased erythrocyte sedimentation rate of 20 mm/h (norm <15), and increased IL2R in the CSF with 99.2 U/mL (norm <8). With 42.0 U/L, the ACE level was in the high-end normal range (8–52). In an extracranial and transcranial Doppler sonography, there were no signs for a vasculitis or clinically relevant vascular stenosis. Spirometry showed a slightly restricted ventilatory disorder. This was a key situation: the additional blood tests and the X-ray findings were compatible with a sarcoidosis type II. Chest CT showed mediastinal and bihilar enlarged lymph nodes confirming the diagnosis of sarcoidosis (Fig. ). Thoracic endosonography (endobronchial) with needle aspiration excluded lymphoma, tuberculosis, and malignant processes and gave histopathological confirmation of the diagnosis of sarcoidosis. The bronchoalveolar lavage fluid (BALF) showed the characteristic elevation in the CD4/CD8 T-cell ratio. A therapy with prednisolone 250 mg i.v. was started for 3 days, then 100 mg prednisolone 1–0-0 was administered orally.
After 2 weeks of treatment, a decrease in the optic disc edemas on both sides and an almost reconstituted macula in the right eye was found. The retinal deposits in the periphery suspicious of vasculitis were scarce on both sides. The subjective symptoms also improved, and the BCVA was 0.8 in the right eye and continued to be stable with 1.0 in the left eye.
With the help of optic coherence tomography (OCT-retina) and fluorescein angiography, retinal granulomas could be detected (Fig. ).
Three weeks later, the low amount of residual disc swelling and perivascular infiltrations allowed reduction of the steroids every 10 days down to 10 mg per day. At the 3-month follow-up, there were no signs of a vasculitis anymore and the IL2R control parameter was down to 50% of the initial value. Since there was no recurrence, the steroids could be reduced to a maintenance dose.
After 2 months of steroid treatment on a low maintenance dose, the patient presented with no subjective symptoms and full BCVA, mild residual prominence of the disc (Fig. ), reduced sheathing of the vessels (Fig. ) and a fully attached macula (Fig. ). The IL2R profile (norm 158–623 U/mL) was evaluated over time: 1,681 U/mL (initial presentation, – 765 – 759 – 615 – 446 – 498 under steroid maintenance dose) and further monitored to decide about the need to continue steroid therapy.
One year after the first presentation, the patient had full vision (cc 1.25) with no visual disturbances. There was no relapse of the initial ophthalmic findings, but still increased tortuosity of the retinal vessels; f/u X-ray was performed and still showed enlarged lymph nodes. IL2R was slightly increased to 579 U/mL but remained in the normal range. Both parameters together with a constantly increased calcium blood level (ongoing production of 1.25-dihydroxy-cholecalciferol by macrophages in granulomas) and intermittent coughing implied that the steroid therapy should be continued. Due to the long intake of steroids, the patient's bone density was reduced, therefore the steroid dose was lowered to 2.5 mL/day and the monitoring interval tightened. Because of the improvement of symptoms, we applied a wait-and-see behavior regarding a steroid sparing immunosuppressive therapy at that time.
Lately, 17 months after initial presentation, the patient was stable without new ocular symptoms and signs under a steroid dose of 2.5 mL/day. X-rays showed no progression of disease and the IL2R level decreased again, so all disciplines involved into the treatment agreed to start an attempt to withdraw the steroids completely with an ongoing tight monitoring interval.
In summary, it is important to retain a constant treatment period of at least 6 months to avoid a relapse. During this time, the steroid dose should be adapted to the clinical course of disease and should not fall short of the maintenance dose. In case of aggravation of the disease or ongoing steroid-related adverse events, a long-term steroid sparing therapy with, for example, methotrexate should be initiated.


An 84-year-old woman with progressive dyspnoea was admitted to our department upon presentation with respiratory distress (oxygen saturation (SpO2): 80% on room air). A chest X-ray showed left atelectasis (Fig. A). Chest CT revealed an occluded left main bronchus (Fig. B). The patient was admitted to the intensive care unit, where bronchoscopy confirmed left main bronchus occlusion. Bronchial toileting was able to open the left upper bronchus, but not the left lower bronchus, which was completely occluded by a tumour and was bleeding. A later histopathological examination revealed the tumour to be an adenocarcinoma with a positive epidermal growth factor receptor (EGFR) gene mutation. After bronchial toileting, the patient's SpO2 improved to 95% under inhaled oxygen (2 L/min flow); an X-ray revealed that the left upper lobe could almost inflate. On day 3, she had progressive dyspnoea and decreased SpO2 caused by reocclusion of the left main bronchus. Bronchial toileting improved the path to the left upper bronchus, but the left bronchial occlusion required repeated treatment. We thus decided to place a stent to maintain airway patency before beginning chemotherapy.
We prepared a customized silicone stent using a 3D-printer and lexible filaments. We obtained 3D data of the lesioned area using Osirix MD (Pixmeo SARL, Switzerland) and chest CT data (Fig. A). We used the 3D-printer (DREAMER; FLASHFORGE JAPAN, Japan) to create a bronchial 3D mould with flexible filaments (FilaFlex®; Recreus, Spain), composed of a polyurethane copolymer. We then customized a ready-made stent (Dumon BD; Novatech SA, France) to fit the printed mould (Fig. B, C).
The patient underwent rigid bronchoscopy and customized stent placement. Tumour debulking extended the lesion's lumen, and the stent was placed from the left main bronchus to the upper bronchus, where it achieved a snug fit and allowed the left upper lobe to maintain ventilation (Fig. D). The patient followed an uneventful course post-operatively. After receiving gefitinib therapy (250 mg), she was discharged from our department. Although she continued receiving chemotherapy, she died 11 months after stent placement.


A 53-year-old man was admitted with a history of a left- sided weakness and facial drooping 3 h before the arrival. On neurologic examination, left hemiparesis, dysarthria, and facial palsy were noted at the admission (National Institutes of Health Stroke Scale [NIHSS]: 11).
A noncontrast computed tomography (CT) scan showed an acute thrombus extending from the right ICA bifurcation into the ipsilateral middle cerebral artery (MCA). Magnetic resonance (MR) imaging demonstrated an infarct involving the right basal ganglia and the insula (Alberta stroke programme early CT score: 7) along with an eccentric hyperintensity extended within the cervical segment of the right ICA wall, suggestive of an intramural hematoma. At the MR angiography, ICA terminus (ICA-T), MCA, and A1 segment of the anterior cerebral artery of the right side were absent. Fluid-attenuated inversion recovery and susceptibility-weighted imaging images showed good collaterals and a large thrombus (27 mm length) on the right ICA-MCA, respectively.
The patient underwent dynamic cerebral angiography for endovascular treatment. The right carotid angiogram showed a severe stenosis of ICA in the cervical segment and an ICA-T occlusion. After three attempts of direct thrombus aspiration (ADAPT technique) at the ICA-T and MCA, a successful revascularization of the vessels was obtained. After 12 h, his NIHSS improved to 3. Three days later, a CTA showed a bilateral ICA dissection with bilateral elongated SPs (35 mm) and a close proximity of ICA with both SPs anteriorly and C1 transverse process posteriorly []. The left ICA seemed to be compressed by both EPs and C1 transverse process.
A surgical treatment was planned to free the left ICA. The patient underwent surgical treatment by an extraoral approach. A vertical skin incision of 4 cm in length was performed from the posterior border of the mandibular angle along with the anterior edge of sternocleidomastoid muscle (SCM). Once the platysma was opened, the superficial layer of deep cervical fascia was then excided. The head of the submandibular gland was mobilized superiorly while the SCM and posterior digastric muscle belly were retracted posteriorly to expose the SP. The SP was medially directed and the left ICA compressed by both SPs anteriorly and C1 transverse process posteriorly, with a direct impingement by the latter. To decompress the left ICA and at the same time, preserve the main muscular and ligamentous connections of the styloid chain, a portion of the left C1 transverse process was removed using a piezosurgical device [].
A temporary paresis of the ipsilateral lower lip lasted 1 month, with a partial remission after 3 months. Overall, the patient reported significant improvement of symptoms with a good esthetics and functional outcome.


A 70-year-old Caucasian man with a past medical history of hypertension, significant smoking history, and atrial fibrillation presented to the emergency department with complaints of chest pain and dizziness. The chest pain began 5 h prior to presentation; it was described as dull, retrosternal, non-radiating, and associated with a sensation of dizziness. Of note, the patient had initially been prescribed apixaban, however, during an outpatient visit with his primary care physician, a decrease in haemoglobin was noted and due to concerns for gastrointestinal bleed, apixaban was held 4 months prior to presentation. An electrocardiogram showed atrial fibrillation with a rapid ventricular response. Vitals upon arrival showed a blood pressure of 136/84, heart rate of 128 p.m., respiratory rate of 16 breath/min, and oxygen saturation of 97% with nasal cannula flow of 5 L/min. Physical exam was remarkable for an irregular heart rate and rhythm, and decreased breath sounds at the right lower lung base. Troponin levels were normal. Chest X-ray demonstrated mild cardiomegaly and a right pleural effusion. Left venous lower extremity duplex was positive for deep vein thrombosis of the popliteal and gastrocnemius veins. A transthoracic echocardiogram (TTE) was performed which showed a normal sized right ventricle with normal function. Most notable was the presence of a large mobile echogenic mass swirling around in the right atrium, consistent with a thrombus in transit, elevated pulmonary artery systolic pressures, and severely reduced left ventricular function (). Computed tomography pulmonary angiography demonstrated vermiform filling defects in the right atrium extending to the inferior vena cava and right ventricle, dilation of the right ventricle and right atrium with increased right cardiac pressures, segmental acute pulmonary embolism in the left lower lobe, and chronic pulmonary embolism in the right main pulmonary artery extending to the proximal right middle lobe pulmonary artery (). Rate control management for atrial fibrillation and weight-based protocol for venous thromboembolism with intravenous unfractioned heparin infusion was initiated. Subsequently, the patient was admitted to the cardiac care unit, with repeat TTE after 8 h, showing resolution of thrombus.


A 10-year-old boy was diagnosed with BMD having presented to the neuromuscular clinic with a history of muscular pain during exercise and elevated serum creatine kinase levels (2495 U/L). Targeted neuromuscular examination revealed bilateral calf hypertrophy, decreased ankle joint range, and very mild weakness in hip abduction and adduction (Medical Research Council (MRC) scale power Grade 4), with preserved muscle strength in all other muscle group. He was asymptomatic from a cardiovascular point of view and his cardiovascular examination was unremarkable. Genetic testing confirmed an in-frame deletion of exon 45–48 of the dystrophin gene (DMD c.6439-72951_7098 + 1309del; p.Glu2147_Lys2366del hemizygosis). Following the diagnosis, cardiac screening was arranged as per the standards of care. A 12-lead electrocardiogram showed sinus rhythm with normal PR interval for his age, normal QRS axis, voltage criteria for biventricular hypertrophy with pathological Q waves inferolaterally (Figure ). The echocardiogram showed asymmetric septal hypertrophy affecting primarily the anteroseptal wall and extending more distally towards the apex. Maximal left ventricular wall thickness was 18 mm at the mid-septum. The left ventricular cavity was relatively small and globally hyperdynamic. Left ventricular diastolic performance was normal and left atrial dimensions were also normal. There was no systolic anterior motion of the mitral valve or left ventricular outflow tract obstruction at rest (Figure ). A cardiac magnetic resonance imaging was performed, confirming an asymmetric septal hypertrophy affecting primarily the basal and mid septum with a maximal left ventricular wall thickness of 18 mm and no left ventricular outflow tract obstruction (Figure ). There was patchy late gadolinium enhancement of the hypertrophied myocardium (Figure ).
Given the unusual finding of HCM in this patient with BMD, we explored the possibility of an additional aetiology for the left ventricular hypertrophy. Genetic testing for HCM-causing mutations was performed on a 104-gene panel, including the sarcomere protein gene, and identified likely pathogenic variants in heterozygosis in the beta-myosin heavy chain gene (MYH7 c.2701G>C; p.Ala901Pro). Family screening for this mutation has been recommended but has not been undertaken to date. The only family member with a history of cardiac disease was the maternal grandfather, who was reported to have undergone a heart transplant due to cardiac sarcoidosis and had died in his forties as a result of subsequent rejection. There was no history of premature cardiac disease or sudden cardiac death in any other family members.
The patient remains asymptomatic from a cardiac point of view without treatment, with no progression of his wall thickness on his follow-up echocardiograms at 1 year. As the concomitant diagnosis of BMD and sarcomeric HCM has not been previously reported in childhood, it is unclear how and whether this combination will affect his prognosis, but he will require regular cardiac follow-up.


A 78-year-old woman experienced sudden onset of stomach ache and vomiting and developed AAD. She was taken to another hospital by an ambulance. The diagnosis was confirmed using enhanced computed tomography (CT) at the hospital. Although CT revealed that the proximal CEA and SMA diverged from the FL of the aorta (), it was considered that there was no malperfusion because of enhanced CEA and SMA. Hence, central aortic repair via ascending aortic replacement was performed. On admission at this hospital, blood tests revealed aspartate transaminase (AST) levels of 115 U/I and alanine transaminase (ALT) levels of 80 U/I. Thereafter, there were no signs of ischemia, and the general condition of the patient improved. CEA and SMA were monitored as a preventive measure, but no additional treatment was administered. Tracheal extubation was performed on postoperative day 2. However, abdominal discomfort reappeared on postoperative day 3. AST and ALT levels were 7,089 and 4,060 U/I, respectively. Lactic acid levels also markedly increased, and enhanced CT showed obstruction of the proximal SMA and CEA and poor contrast enhancement of the liver (). Thus, the patient was transferred to our critical care center.
We suspected that hepatic malperfusion may have resulted from the obstruction of the proximal part of CEA and SMA. However, it was difficult to secure vascular access due to obesity. Therefore, the brachial artery was secured, and a 9-Fr vascular sheath was inserted. SMA arteriography was performed using a guiding catheter (Parent Plus60, Medikit, Tokyo, Japan). SMA arteriogram demonstrated proximal occlusion of SMA near the ostium of this artery (). To restore the true lumen (TL) of SMA, a 0.016-inch guide wire (Asahi Intecc, Nagoya, Japan) was inserted to the distal site and percutaneous transluminal angioplasty (PTA) was performed using a 5-mm balloon catheter (Boston Scientific, Marlborough, MA, USA) (). Subsequent SMA arteriography showed improved blood flow, and CEA branches were perfused via the inferior pancreaticoduodenal artery (IPDA). Then, a 5 mm×19 mm monorail premounted stent (Express, Boston Scientific) was placed in the stenosed SMA (). Balloon dilation and stent placement were successfully performed without any complications.
Because CEA could not be approached, it was not unblocked. The suspected diagnosis was mesenteric malperfusion; therefore, an emergency laparotomy was performed. Ascites (light yellow) was confirmed during laparotomy. The color of the small intestine was normal, although few dark red portions, suggesting ischemia, were confirmed on the other side of the mesentery. Moreover, the ischemia was irreversible. There was no ischemia in the colon. The color of the liver changed to dark red, which indicated a change in the ischemic condition.
Chronic hepatic disease was also suspected as the hepatic surface had blunt margins. The emergency laparotomy was terminated without additional treatment for the ischemic findings to reduce the operative time and because CEA was being imaged through angiography.
Although angiography was performed again the day after the surgery due to persistent, high lactic acid levels, no blood flow issues were observed in SMA. Optimum blood flow in the portal vein was also confirmed using ultrasound, and hence, no additional treatment was performed, except conservative management.
Transfusion with fresh frozen plasma was continued to compensate for the liver function. AST and ALT levels increased to 17,010 and 5,470 U/I but reduced to 3,032 and 1,497 U/I after 3 days, respectively, indicating improvement. In addition, 8 days later, both the levels decreased to less than 100 U/I. Prothrombin time and international normalized ratio increased to a maximum of 6.04.
Long-term respiratory management was necessary due to massive transfusion. The patient was extubated on day 17 and was transferred to the previous hospital on day 38.
This report has been approved by our Institutional Review Board (approval number: 1278).


A 10-year-old boy, run over by a trolley, presented after 5 days of trauma to our center in tachypneic state with a chest tube in situ and oxygen saturation of 80%, which improved to 98% with oxygenation. The child had blood pressure of 99/72 mmHg and tachycardia of 112 beats/min, with Glasgow Coma Score of 15/15. On examination, there were diminished breath sounds on the right hemithorax, and tenderness was present in the right upper abdomen with overlying abrasion. The chest X-ray showed right costophrenic angle blunting with a chest tube in situ and tenting of the right dome of the diaphragm. Contrast-enhanced computed tomography (CECT) of the chest and abdomen showed grade intravenous (IV) liver laceration, hemoperitoneum, and Grade III diaphragmatic injury with herniation of liver into right hemithorax.[] Atelectasis of the right lower lobe and right-sided moderate pleural effusion was also present. The patient was started on IV fluids and IV antibiotics. Avulsion injury of the right dome of the diaphragm was visualized on right posterolateral thoracotomy. The diaphragm was detached from parietes from midline anteriorly to posterior axillary line, and laceration of liver was also present [Figures and ]. Repair of the diaphragmatic injury was done by restoring attachment of diaphragm to the chest wall using polypropylene sutures. Liver laceration was not actively bleeding and hence did not require any repair. Right lung expanded fully on positive pressure ventilation. Postoperatively, the child had an uneventful recovery, and a follow-up chest X-ray after 3 months showed normal contour of the diaphragm and normal lung fields.


A 45-year-old female incurred multiple rib fractures with right-sided hemothorax following a road traffic accident, and a thoracostomy tube insertion was done at the primary treating hospital. The patient also had fractures of the right fibula and tibia which were managed with open reduction and internal fixation at the same center. A CECT thorax was done which showed second and third right rib fracture with collapse and consolidation of the right lung and laceration of the right lobe of liver with contusion. She was finally referred to our tertiary care center after 1½ months from the antecedent injury. On examination, she was conscious, oriented, pulse rate was 118/min, blood pressure was 108/82 mm Hg with severe dyspnea (respiratory rate of 25–30 breaths/min), and decreased air entries at the lung bases bilaterally. The patient was intubated and shifted to ICU for ventilatory support. A repeat Chest X Ray and CECT thorax showed bilateral multiple rib fractures, right-sided hemothorax with air foci in the collection, and an indistinct outline of the diaphragm [Figures and ]. The patient was taken up for exploration in view of a possible diaphragmatic injury, and right posterolateral thoracotomy was done. Intraoperatively, we found necrotic sloughed out the right dome of the diaphragm with collapsed right lung. An organized collection was present in the right hemithorax and the liver was lying in the lower thoracic cavity. All the necrotic tissues and organized hematomas were removed, and an intercostal tube was placed between liver and the collapsed lung. No repair of diaphragm could be done. Postoperatively, she had stable recovery but could not be weaned off mechanical ventilation. A tracheostomy was done in view of long-term intubation. Enteral feeds were started through nasogastric tube. The patient was transferred to the cardiothoracic surgery unit after 3 weeks for further management. However, in view of her poor general condition, no further surgery was performed and she died after 1 month.


A 7-day-old girl had been born by cesarean delivery at a gestational age of 39 weeks due to prenatal diagnosis of a cardiac tumor in fetal ultrasound. The mother had attended ten prenatal consultations and had negative serologies to HIV, syphilis, and hepatitis B. Relevant family history comprised the presence of dermatological lesions in the patient’s mother, aunt, and grandfather, and both mother and aunt reported having seizures since childhood.
The newborn’s Apgar scores were 7 and 8, and the birth weight was 2745g. Upon initial physical examination, she presented a systolic heart murmur on the left medium sternal border, intensity 3/6. The remaining physical examination was unremarkable. Initially, the patient was hemodynamically stable, without signs of low cardiac output such as hypotension, tachycardia, breathlessness and hypoxemia, alterations in the level of consciousness, cold peripheries, or delayed capillary refill time. Nonetheless, she was kept in the Neonatal Intensive Care Unit (NICU) under rigorous cardiac monitoring. Two echocardiograms were performed and confirmed the presence of multiple tumors in the ventricular walls and interventricular septum. There were two main lesions in the interventricular septum, the largest one located at its basal portion measuring 14X13mm in its longest axis, protruding (8.2mm) into the left ventricular outflow tract, markedly obstructing it. The pressure gradient was approximately 95 mmHg. Rhabdomyomas as the working diagnosis was considered, and surgical intervention was scheduled for the 7th day after birth. The physical examination, at this moment, showed normal intensity and regular peripheral pulses at a rate of 162 per minute. The girl’s blood pressure was 58/33 mmHg, and her room-air oxygen saturation was 97%. Peripheral perfusion was normal, and no respiratory distress (respiratory rate was 37 breaths per minute) was depicted. The cardiovascular examination demonstrated the persistence of the systolic murmur grade 3/6 at the left sternal border. Pre-op laboratory exams, including complete blood count, erythrocyte sedimentation rate, C-reactive protein, serum electrolytes, liver, and renal function tests were all unremarkable. However, during anesthetic induction, the patient developed hemodynamic instability, bradycardia, and cardiac arrest. Unfortunately, she died despite the attempts of resuscitation. An autopsy was performed with the informed consent of the family.


An 84-year-old woman was brought to hospital by ambulance with acute right lower chest pain, unexplained anaemia and newly deranged liver function. The patient denied any history of cough, shortness of breath or palpitations. She had no history of venous thromboembolism (VTE) and denied risk factors for VTE. She did not have abdominal pain, vomiting, urinary symptoms, active bleeding or melena. She had normal stool habits. However, she had a history of hypertension, osteoarthritis, ischaemic heart disease with previous coronary artery bypass graft surgery many years previously, and chronic kidney disease stage 3. She was taking aspirin, and anti-hypertensive and anti-angina medication as well as a glyceryl trinitrate (GTN) spray. She had a good performance status, lived alone independently and was helped by carers only twice a week.
On examination, the patient was alert and fully conscious. Her vital signs were stable with a respiratory rate of 16 and saturating 100% on air, blood pressure of 105/59 mmHg, heart rate of 75 bpm and temperature of 36.8°C. Auscultation revealed normal heart sounds with no murmurs, and bilateral good air entry with right basal crepitations. The abdomen was soft and non-tender, bowel sounds were normal, and digital rectum examination revealed normal stool colour with no signs of fresh blood or melena. The calves were soft and non-tender and no focal neurological deficits were demonstrated.
Blood results initially showed a raised white cell count of 14×109/l (normal 4.0–10.0×109/l) and a C-reactive protein (CRP) level of 28 mg/l (0–5). Haemoglobin was 89 g/l (120–150) compared to a previous haemoglobin of 127 g/l approximately 2 months previously. Mean corpuscular volume (MCV) was 86 fl (83–101) and liver enzymes were newly deranged as follows: bilirubin 15 μmol/l (0–21), alanine aminotransferase (ALT) 304 U/l (0–33) and alkaline phosphatase (ALP) 507 U/l (30–130). We also noted urea of 11.8 mmol/l (2.5–7.8) and creatinine of 92 μmol/l (44–80). Chest x-ray showed mild haziness in the right lower zone consistent with infective changes.
Initially the patient was treated for right basal pneumonia with associated pleurisy. Because of the new anaemia, raised urea level and newly deranged liver function, she underwent urgent oesophagogastroduodenoscopy (OGD) and ultrasound of the abdomen. At this stage she was stable. OGD showed hiatus hernia and severe oesophagitis but no source of active bleeding.
Two hours later, the patient started passing a large amount of melena and her systolic blood pressure dropped to 70 mmHg despite initial intravenous fluid boluses. On arrival of the emergency team, the patient was spontaneously ventilating, on 4 l/min of oxygen saturating 98%, with good air entry bilaterally, a blood pressure of 70/48 mmHg and receiving continuous intravenous fluid resuscitation, a heart rate of 117 bpm, and a capillary refill time of 3 seconds. Blood sugars were normal and the Glasgow Coma Scale (GCS) score was 15/15. The patient was complaining of abdominal pain with a distended abdomen, generalised abdominal tenderness, involuntary guarding and sluggish bowel sounds. There were also weak peripheral pulses and no sign of VTE.
Management at this stage was for massive upper GI bleeding and included fluid resuscitation, activation of the massive haemorrhage protocol (transfusing Group O negative blood until matched bloods became available and fresh frozen plasma), proton pump inhibitor infusion, tranexamic acid 1 g and terlipressin 2 mg given on the basis of deranged liver function. A CT angiogram of the abdomen and pelvis was arranged once the patient was haemodynamically stable, and surgical review was sought for a possible perforated bowel. A CT angiogram of the abdomen (portal, venous and arterial phases) suggested a suspected pseudoaneurysm in the right hepatic artery, with surrounding fluid indicating potential active bleeding and suspected haemorrhagic content in the distended biliary tree ().
The patient was stabilised with a blood pressure of 124/78 mmHg and heart rate of 95 bpm. There was no further melena. The patient was discussed with the vascular team at the nearest tertiary centre who accepted her for transfer overnight. TAE was performed successfully and the patient was monitored for 48 hours, during which time no further signs of GI bleeding were noted. The patient improved significantly and physiologically returned to her baseline. She was discharged to the community for rehabilitation and subsequently discharged home after 2 weeks. She was followed up over the phone after 4 weeks: she was doing very well, living a normal life and reported no further chest or abdominal pains and no further GI bleeding symptoms.


We present the case of a 67-year-old male patient, which was hospitalized in the Otorhinolaryngology (ENT) Clinic of the Clinical Emergency Hospital of Craiova for a one year history of nasal airway obstruction, mucopurulent rhinorrhea, repeated epistaxis and anosmia.
Symptomatology had an insidious debut and worsened in its evolution, ending with the exteriorization of a polypoid tumor from the left nasal cavity.
Clinical examination revealed an irregular, polypoid tumor, that showed areas of bleeding, covered with mucopurulent secretions, and which completely obstructed the left nasal cavity (Fig.). Rhinopharyngeal endoscopy revealed that the tumor occupied almost the entire nasopharynx.
We decided to perform a computed tomography (CT) of the nose and paranasal sinuses, examination that was performed using a Toshiba Astelion Advance, with intravenous administration of contrast medium (iohexol, Omnipaque 350mg). The CT showed an iodophilic and non-homogeneous tumor, with areas of necrosis, which included the entire left nasal cavity, with extension to the rhinopharynx and the left maxillary sinus. No osteolytic changes have been seen in the skull bones, that might have suggested a malignant tumor (Fig.).
We completely removed the tumor through an endoscopic medial maxillectomy, under general anesthesia with orotracheal intubation, collecting the fragments for histopathological examination (Fig.).
The macroscopic description of the tumor mass removed from the nasal cavity was of multiple fragments varying between 0.6cm and 5.2cm, the largest fragment exhibiting a relatively homogeneous, white-yellow appearance.
All the tissue was fixed in 4% neutral buffered formalin and routinely processed for paraffin embedding and sectioning as 4µm thick sections, in the Pathology Department from the Emergency County Hospital Craiova. Sections were processed for Hematoxylin and Eosin staining, dehydrated, cleared, and coversliped in a xylene-based mounting medium.
Microscopy evaluation revealed upper respiratory mucosa (Fig.) showing at the level of the lamina propria, a papillary epithelial proliferation with endophytic pattern, consisting of stratified squamous epithelium (Fig.) with intermixed regions of columnar epithelium (Fig.).
Also, we noticed small cysts containing polymorphonuclear neutrophils (PMNs) and/or necrotic detritus, disposed on the inner regions of the epithelial tumoral islands (Fig.).
Some regions exhibited also low grade dysplasia (Fig. , ).
Taking together these features, histopathological evaluation established a final diagnosis of inverted papilloma, with areas of low grade dysplasia, and also of areas with oncocytic Schneiderian papilloma.
Postoperative evolution was favorable, the patient was released from hospital two days after surgery and received oral antibiotherapy for 7 days, as well as local treatment with saline nasal spray and topical steroids. Six months postoperative control, which consisted in clinical, endoscopic and CT examination, did not reveal any tumor recurrence.
A written informed consent was obtained from the patient regarding his agreement to publish these data.


A 40-year-old male with a history of smoking and untreated HIV infection presented to the hospital with concerns for a 10 lb weight loss, night sweats, and a nonproductive cough for 2 months. His initial laboratory studies demonstrated a white blood cell count of 2.6 K/μl, lymphocytes of 0.24 K/μL, and CD4 count of 0. A chest X-ray demonstrated a thick-walled cavitary lesion in the right upper lung, which was confirmed on a CT scan of the chest []. The right upper-lobe cavitary lesion was accessed by ENB, and several (seven FNA and seven forceps biopsy) samples were collected. Rapid on-site evaluation (ROSE) was available for immediate microscopic assessment of the tissue, and a preliminary diagnosis was rendered as concerning for the presence of P. jirovecii. This diagnosis was confirmed with the use of Grocott–Gomori's methenamine silver stain []. A bronchoalveolar sample was also collected from the right upper lobe, and P. jirovecii organisms were detected on cytologic evaluation of the BAL fluid. There was no malignancy or other infectious organism detected from the tissue of BAL samples.


A 36-year-old man was admitted because of dyspnea and chest pain for 1 month prior to presentation. A grade 4/6 systolic murmur was auscultated at the right second intercostal space. Initial chest roentgenography showed mediastinal widening, and electrocardiography (ECG) showed a 2:1 atrioventricular block (). Transthoracic echocardiography (TTE) showed normal left ventricular systolic function (ejection fraction, 62%) with concentric left ventricular hypertrophy and typical findings of severe aortic stenosis, including mild aortic regurgitation, extensive calcification of the aortic valve, a mean transaortic valve pressure gradient of 49 mm Hg, and an aortic valve area of 0.8 cm2. However, there was no definite commissure or raphe between the aortic cups, implying a unicuspid aortic valve (UAV). Subsequent transesophageal echocardiography (TEE) revealed a UAV with a single posterior commissure (). Computed tomography showed aneurysms of the aortic root (5.0 cm in diameter) and ascending aorta (5.5 cm in diameter) ().
An incision made through the ascending aorta revealed an extensively calcified aortic valve (). The aortic valve was morphologically unicuspid with a single, unfused commissure that had a slit-shaped appearance, and it was distinguishable from a bicuspid aortic valve (BAV) (). The diseased aortic valve was carefully removed using a surgical blade and rongeur. However, extensive aortic annular calcification had deeply invaded the annulus, especially on the left coronary cusp, and after removal of the calcified tissues, the annular tissue defects were reconstructed with bovine pericardium (). After removal of the aneurysmal aortic root and ascending aorta, a Bentall operation was performed using a 27-mm mechanical valved conduit.
After surgery, ECG showed intermittent complete atrioventricular block, and we considered a temporary pacemaker with an epicardial pacing wire. The patient was weaned from mechanical ventilation on postoperative day 1 and transferred to the general ward on postoperative day 2. He underwent 24-hour continuous Holter monitoring. The minimum heart rate was 44 bpm and the maximum R-R interval was 1.48 seconds. He was discharged on postoperative day 12 without dizziness or fainting. However, he was readmitted because of syncope on postoperative day 21. Intermittent complete atrioventricular block was noted, with a minimum heart rate of 19 bpm and a maximum R-R interval of 14.16 seconds. Thus, a permanent pacemaker was placed.
After discharge from the hospital in a stable condition, the patient was placed under appropriate oral anticoagulation therapy and was followed up at an outpatient clinic. Follow-up echocardiography performed 2 years after surgery revealed a diminished left ventricular mass index (from 121.00 to 77.14 g/m2).
The Institutional Review Board of Sanggye Paik Hospital approved the present case report (IRB approval no., SGPAIK 2019-02-016), and informed consent was obtained from the patient.


A 74-year-old woman (height, 156 cm; weight, 63 kg), with American Society of Anesthesiologists physical status grade II, was diagnosed with malignant fibrous histiocytoma in the left buttock, for which she underwent surgery and left gluteal flap closure. She had a medical history of hypertension and chronic renal failure. The surgery was performed under general anesthesia for a prolonged time (24 h and 55 min), and the patient was intubated with a 7.0-mm-internaldiameter Mallinckrodt™ (Covidien, Dublin, Ireland) cuffed reinforced ETT. The patient was transferred to the intensive care unit (ICU) and remained intubated because of postoperative upper airway edema. An oral airway was inserted as a bite block to prevent collapse of the tube. She was kept in a prone position with the reinforced ETT because the gluteal flap turned blue in the lateral decubitus position. During the ICU stay, she was sedated with dexmedetomidine and remifentanil. The infusion rate of the drugs was controlled on the basis of the level of sedation, with a goal of achieving Richmond Agitation Sedation Scale (RASS) level 2. Although she was weaned from the mechanical ventilator in a pressure support ventilation mode, she could not be extubated because she tested positive on the cuff-leak test and underwent two additional emergency surgeries—one on the same day and another 48 h later. On postoperative day 3, she still tested positive on the cuff-leak test one and a half hours before the event. Also, the charge nurse had difficulties passing a suction catheter through the lumen of the ETT because of the narrow lumen, but the tidal volume and peak inspiratory pressure were not changed. Thirty minutes before the event, the tidal volume and peak inspiratory pressure were still unchanged. Peak airway pressure suddenly increased with a decrease in the tidal volume, and oxygen saturation was reduced to 85% despite the delivery of 100% oxygen. We attempted to pass a suction catheter through the lumen of the ETT, but the catheter was obstructed at the level of the incisor teeth. After placing the patient in a supine position, we found that the ETT had been completely severed and the oral airway had been spat out. Upon inspection of the oral cavity, we discovered that the distal portion of the ETT was missing. Therefore, we performed fiberoptic bronchoscopy after normalizing the oxygen saturation to 98-100% (flow rate, 10 L/min) by using a facial mask. Although the severed distal portion of the ETT was observed beneath the vocal cord, it could not be removed. Chest anteroposterior radiography showed that the severed portion was lodged in the right main bronchus (). Her self-respiration was maintained without upper airway irritation during the time required for exams and the surgical procedure. The patient was taken to the operating room for emergency bronchoscopy. The ETT was surgically removed by an otolaryngologist under general anesthesia. In the operating room, she was preoxygenated with 100% oxygen, induced with intravenous propofol 1.5 mg/kg without the use of a muscle relaxant and anesthesia was maintained with manual ventilation with 100% oxygen and sevoflurane. After achieving adequate depth, a micro-laryngoscope was introduced and the severed ETT was removed with a forceps successfully (). Micro-laryngoscopy revealed a mild subglottic swelling right after removal of the ETT. As the patient recovered spontaneous breathing, she was transferred to the ICU. Additional intubation was not needed and 5 mg dexamethasone was intravenously injected twice a day for 2 days for relieving upper airway edema. The remainder of the patient’s ICU stay was uneventful, and she was transferred to a ward without any difficulty with respiration. Further laryngeal evaluation showed no abnormal findings. However, the gluteal flap did not remain viable and underwent necrosis; hence, the patient had to undergo another surgery for flap removal. She was then transferred to the regional hospital for palliative care.


A 63-year-old female with a 4-month history of an enlarging perineal itching nodule was referred to our hospital. On gynecologic examination, a 4 cm-sized well circumscribed pink perineal-anal nodule with ulceration was detected (Fig. ). The perineal-anal nodule did not invade the urethra or vagina. Digital examination and inspection of the rectum revealed that the perineal nodule continued to the rectum via the anal canal. Biopsy specimens from the rectal mucosa and perineal nodule showed a poorly differentiated squamous cell carcinoma. Magnetic resonance imaging and 18F-fluorodeoxyglucose positron emission tomography showed a primary tumor located from the perineum to the rectum along with the anal canal (Fig. ). In addition, right inguinal and internal iliac lymph nodal metastases (Fig. ) and a 2 cm-sized isolated nodule in the right labia majora were observed (Fig. ). The isolated nodule in the right labia majora was clinically judged as a solitary cutaneous nodular metastasis from anal cancer via lymph channels. She was diagnosed as having anal squamous cell carcinoma that was clinical stage IV (T3N1M1) based on the Union for International Cancer Control TNM, 7th edition.
The patient had good performance status and effective organ function. The cutaneous metastasis was a solitary lesion. The entire gross tumor, including the solitary cutaneous metastasis in the labia majora, could be encompassed by the irradiation fields. It was recommended that she undergo definitive chemoradiotherapy with curative intent rather than palliative treatment. She consented to undergo definitive treatment. Chemoradiotherapy was started with 60 Gy in 30 fractions with concurrent administration of mitomycin (10 mg/m2 on day 1, 29) and 5-fluorouracil (1,000 mg/m2 on days 1–4 and 29–32). Volumetric-modulated arc radiotherapy using two-coplanar arcs with a 15 megavoltage X-ray was planned. Simultaneous integrated boost method was used to deliver irradiation doses []. The prescribed doses were 60 Gy in 30 fractions to the gross tumors including the primary tumor and pelvic lymph nodal metastases and the solitary cutaneous nodular metastasis, and 45 Gy to the subclinical lymph nodal region including perirectal, inguinal, internal iliac, and external iliac lymph nodal regions in 30 fractions (Fig. ). Intensity-modulated radiotherapy spared the external genitalia and intestines. She completed the chemoradiotherapy without unplanned treatment breaks of radiotherapy and dose reduction of chemotherapy. As an acute adverse event, Grade 3 leukocytopenia, thrombocytopenia, anal pain, and radiation dermatitis were observed. No adjuvant chemotherapy was performed. Colonoscopy, magnetic resonance imaging and 18F-fluorodeoxyglucose positron emission tomography showed a complete response; that is, there was no sign of residual tumor without any mucosal erosion or ulceration at seven months after the initiation of chemoradiotherapy (Fig. ). To date, i.e., 42 months after the initial day of chemoradiotherapy, she has not experienced any disease recurrences or ≥ grade 2 late toxicities, such as a rectal bleeding.


A 36-year-old otherwise healthy Moroccan man presented with 2 years of fulminant, folliculocentric pustules and nodules which began on the trunk and spread to the extremities, genitals, face, and scalp, sparing the palms and soles. He reported sweats and subjective fevers, concurrent with rash onset and intermittently recurring. Over the past year, he experienced fatigue and a 10-lb weight loss. He completed a 6-month course of doxycycline which helped with rash severity, yet lesions still recurred. He applied clobetasol cream intermittently and chlorhexidine gluconate solution (Hibiclens) without sustained improvement. His labs were negative for HIV, HCV, treponemal antibody, ANA, RF, and ANCA, while his CRP was 82 mg/L (reference range <3 mg/L) and ESR 69 mm/h (reference range <15 mm/h). An initial punch biopsy from his left arm showed acute folliculitis; Brown Hopps stain revealed Gram-positive cocci in the follicular infundibulum.
Three months later in follow-up, his rash had worsened, with many new, generalized and painful lesions (Fig. ). An intensive decolonization regimen was recommended, along with two systemic antibiotics (clindamycin and rifampin) pending bacterial culture results of a pustule. Bacterial culture and biopsy were repeated a month later at another hospital, yielding results again consistent with folliculitis. Oral clindamycin and rifampin were initiated. A week later, he presented to the emergency department with 2 days of severe right upper quadrant pain, with examination revealing right axillary lymphadenopathy. Abdominal CT showed multiple hepatic ill-defined hepatic lesions. Dermatology was consulted and performed two 3-mm punch biopsies of lesions identified by the patient and his wife as the newest-appearing lesions [].


Inherently healthy, an asymptomatic 19-year-old woman showed an abnormal chest shadow on physical examination in 2001. Thereafter, she was diagnosed with aPAP by video-assisted thoracic surgery and serological test at a local hospital. For several years she was followed up and remained asymptomatic until she became aware of shortness of breath at the end of 2014. Peripheral capillary oxygen saturation (SpO2), Krebs von den Lungen-6 (KL-6, normal range: <500 U/mL). Surfactant protein-D (SP-D; normal range <110 ng/mL) were 90%, 7704 U/mL, and 455 ng/mL, respectively. She was referred to Hachioji Medical Center where she underwent six times whole lung lavages (WLL) repeatedly during March 2015 to May 2017. Her aPAP condition recurred with remission and exacerbation during the period, and she became pregnant in July 2017. She continued her pregnancy in consultation with her treating doctor who decided to avoid WLL because of its adverse effects on the foetus. However, her hypoxaemia became progressively worse with partial pressure of oxygen (PaO2) under 60 mmHg at room air, and thus, she was forced to receive home oxygen therapy (HOT; Fig. ) to keep oxygen saturation more than 88%. At 36-week gestation, the patient delivered a girl by caesarean section.
Hypoxaemia of the mother with PaO2 at 56.3 mmHg (room air) just before the delivery did not improve but further exacerbated to the level of 39.3 mmHg within one week. Therefore, she underwent three times WLL that improved her hypoxaemia from SpO2 level at 88% (3 L O2 nasal cannula) to 97% (room air) within a month. As aPAP entered remission, HOT was discontinued. The remission has continued until now without elevation in the serum KL-6 and SP-D level , . On the other hand, the newborn did not show hypoxaemia after delivery with stable SpO2 ranging from 98% to 100% (Fig. , red bar). As both serum KL-6 and SP-D have kept within normal limit (68–125 U/mL and 49.5–70.8 ng/mL, respectively), we consider that the newborn has developed normally so far. These data indicated that the newborn did not develop aPAP.
The serum GMAb level in the mother had been as high as 150 μg/mL increasing slightly after the delivery according to the increase in the total IgG, peaked at 3 months, and then gradually decrease to the baseline level (Fig. ). In contrast, the serum GMAb level in the newborn rapidly decreased exponentially to the level under the cut-off level (1.65 μg/mL), whereas the serum IgG level also decreased by 3 months after the birth and then reached a steady state.


A 25-year-old woman visited our hospital with recurrent abdominal pain.
There was intermittent epigastric pain that started 1 day before her visit to our hospital.
The patient had no specific history of past illness such as trauma or medication. Due to abdominal pain 1 year prior, she underwent computed tomography (CT) of the abdomen and pelvis at another hospital and was transferred to our hospital. A contrast-enhanced CT scan revealed a poorly enhanced heterogenous mass adjacent to the anterior wall of the gastric lower body (Figure ). A perigastric abscess or hematoma or a gastric SMT was suspected at that time because the patient had mild fever, abdominal pain, and a poorly enhanced heterogenous mass, and was hospitalized. The patient underwent sonography for percutaneous drainage insertion after admission and a focal hypoechoic solid-like lesion with an internal echogenic component on the anterior wall of the gastric antrum was found, but there was no evidence of an internal cystic lesion, suggesting that the hematoma was suspicious rather than an abscess because a small amount of blood component had drained (Figure ). She was treated with antibiotics, tranexamic acid, a proton pump inhibitor, and conservative therapy. Because her symptoms improved, the patient was discharged without any further examination (such as endoscopic ultrasound) and underwent regular follow-up in the outpatient clinic.
The patient had no personal or family history.
The patient exhibited tenderness over the entire abdominal, with epigastric pain dominant, and a fever of 38.4°C.
Blood analysis showed a mild leukocytosis (white blood cell count, 11770/µL), with dominant neutrophils (78.7%) and a normal hemoglobin level and platelet count. The serum C-reactive protein level was elevated, at 31.2 mg/L (normal range, 0-5 mg/L). The other laboratory findings were unremarkable.
Abdominal CT showed a well-circumscribed, highly attenuated, and slightly homogenous round mass in the peri-gastric region (Figure ). A SMT, peri-gastric hematoma, and gastric endometriosis were identified by differential diagnosis on CT scan. Thus, upper gastrointestinal endoscopy was performed, and a 6.7 cm × 2.7 cm subcutaneous lesion on the endoscopic anterior wall of the great curvature of the gastric antrum was identified (Figure ). Endoscopic ultrasonography (EUS) was performed next, and the findings were a heterogeneous hypoechoic mass of approximately 6.35 cm × 3.00 cm that appeared similar to a gastric SMT of fourthlayer origin (Figure ). EUS-guided fine needle biopsy (EUS-FNAB) was performed due to suspicion of a gastric SMT based on the EUS findings, but because the tumor was not diagnosed histologically, surgical resection was planned.
The mass was mostly well circumscribed with a white-tan cut surface, showed focally infiltrative growth in fat tissue. The grossly confirmed mass was 6.5 cm × 4.0 cm × 1.0 cm, and a calcified fibrous tumor that originated from the gastrohepatic ligament and adhered to the lesser curvature of the gastric antrum was identified (Figure ).
The tumor consisted of a sparse population of fibrous cells, hyalinized collagen, and a haphazard collagenous matrix; a scattered dystrophic calcification was observed. Inflammatory cells, consisting of lymphocytes and plasma cells, were the dominant infiltrating cells, and bland spindle cells were embedded (Figure , ). No germinal center was observed. A fibrinoid degenerative lesion due to a previous histologic examination by EUS-FNAB was present. Immunohistochemical staining results indicated that the tumor was CD34 negative, SMA negative, CD117 negative, Bcatenin negative, and Factor XIIIa positive for spindle cells (Figure ).


A 12-year-old boy old presented to the emergency department complaining of severe persistent right iliac fossa pain associated with subjective fever, nausea, vomiting and anorexia. On examination, his vital signs were found to be within normal limits. An examination of the abdomen revealed right iliac fossa tenderness and rebound tenderness.
Laboratory investigations showed following results: white blood cell count 24.00 × 103/μl (reference range 4.5–11), hemoglobin 13.6 g/dl (reference range 13–16), C-reactive protein 76.5 (<5 nl). A computed tomography (CT) scan of the abdomen and pelvis using oral and intravenous contrast showed a distended appendix filled with fluids (). Also observed were pockets of air with a larger proximal and smaller distal fecolith with free fluid in Morison’s pouch, right paracolic gutter and rectovesical pouch consistent with acute complicated appendicitis.
The patient underwent a laparoscopic appendectomy. Following three trocars were inserted: a 5 mm supraumbilical trocar, a 5 mm suprapubic trocar with a balloon and a 10 mm in the right lumbar area. The procedure was uneventful with no intraoperative complications. A 15 F drain was inserted through the suprapubic incision, and the patient was admitted to the surgical ward for observation.
The patient’s postoperative course was remarkable for a low urine output, which was noted on the night of his surgery. His supraoptic dressing was soaked with a clear yellow fluid, and the patient was complaining of mild suprapubic pain. An examination revealed suprapubic tenderness, and the drain contained 80 ml of a clear yellow fluid. The patient’s dressing was changed and closed, and his urine output was observed. The drained fluid was sent for analysis.
On a postoperative day 1, the suprapubic dressing was soaked with urine and the drain contained 200 ml of a clear yellow fluid. The patient did not urinate at all postoperative.
An abdominal examination revealed generalized guarding. A Foley catheter was inserted, and it yielded 275 ml of clear urine. A potential diagnosis was made for iatrogenic bladder injury.
A CT cystography was performed, and it showed a vesicourachal diverticulum. The suprapubic drain that was placed intraoperatively was shown to be penetrating the diverticulum (). The urology department was consulted and the patient’s condition was discussed with his family. Management options were offered, including conservative management with Foley catheter placement and operative repair; the family opted for conservative management. The management included hourly monitoring of his urine output, antibiotic prophylaxis with intravenous cefuroxime 625 mg thrice a day and intravenous metronidazole 350 mg thrice a day, daily laboratory investigations (complete blood count, renal function tests and serum electrolytes), and a cystography performed after 7 days. The patient’s daily urine output is shown in .
A contrast cystogram was performed on postoperative day 9, and a pelvic scan showed no leak or extravasation of the contrast fluid (). The catheter was removed, and the patient was discharged after successful micturition trials. Follow-up examinations performed 2 weeks and 1 month post-discharge were unremarkable and the patient presented no complaints.


The patient, a 54-year-old man, was admitted to the hospital because of "a space-occupying lesion in the right kidney for 2 d upon ultrasound examination".
His past history was unremarkable.
His family history was unremarkable.
His physical examination on admission was unremarkable.
The results of a hemogram, blood biochemistry, and tumor markers were in the normal range.
Computed tomography (CT) showed the mass as a soft tissue density shadow with a diameter of approximately 6.8 cm in the right renal pelvic area. The density was not uniform, and the boundary was not clear. It extended into the renal sinus and showed uneven enhancement (Figure ), which manifested as partial deformation, a disappearance of the pelvis and calyces of the lower pole of the right kidney, and delayed enhancement of the left right renal parenchyma. No enlarged lymph nodes were observed behind the peritoneum. Ultrasound images showed a cystic solid mass of approximately 6.8 cm × 6.5 cm that was visible in the right kidney, which had an unclear boundary and irregular shape. Color Doppler flow imaging (CDFI) revealed dotted blood flow signals in the periphery and interior. CEUS revealed that, after the mass injection of the contrast agent, the right renal cortex began to enhance at 9 s, the renal mass began to enhance at 11 s, and the mass began to peak at 28 s. The mass subsided more rapidly than the renal cortex, and the right renal mass showed "slow in and fast out" hyperenhancement (Figure ). The cystic solid masses were in the renal medulla, pelvis, and calyces, and they had a size of 6.8 cm × 5.5 cm × 5.5 cm. The abundant tumor cells were of spindle cell type, and a small number of tubules were observed among spindle cells through pathological examination. No tumor cells were found at the ureteral resection margin. Immunohistochemistry showed that the lesion was positive for SYT genome rearrangement, B-cell lymphoma-2 (Bcl-2), Vimentin, Pax-8, and CK7, and negative for smooth muscle actin (SMA), soluble protein-100, epithelial membrane antigen, CD99, and Pax-2. PRSS was diagnosed according to its imaging manifestations and pathological results (Figure ).


An 11-year-old male came to our observation with skeletal class III malocclusion, class III molar relationship, and negative overjet (−1 mm). At the initial observation, he was in the CS1 stage of skeletal maturation, according to the CVM method ().
The patient was treated with the protocol described in the Materials and Methods section: we bonded a rapid palatal expander to the first upper molars using a vetroionomer cement; in this case, we did not use a two-hinged expander because first premolars were not present (). Each cycle of expansion and constriction needed 2 weeks to be done (1 week of expansion and 1 week of constriction), for a total amount of 6 weeks. We gave instructions to patients’ parents to activate and deactivate the device: they had to activate the screw twice a day (0.50 mm) for 1 week and then to deactivate the screw twice a day for 1 week, repeating this protocol three times. After this period, we decided to expand for another 10 days to correct posterior crossbite and to gain an adequate palatal transverse diameter (2 activations of the screw a day).
During all this period, we checked weekly the patient to evaluate the opening of the suture (that was confirmed by the immediate opening of interincisal diastema after few days of expansion) (), the stability of the device, and the status of symptoms.
The patient complained of little pain to the area of the nose and the palate and to the upper molars during the first phase of the Alt-RAMEC period; however, it was not necessary to take medicines to alleviate symptoms. Subsequently, he did not have any ache probably because the sutures were almost distracted.
Just after the Alt-RAMEC protocol, we adapted the Delaire mask on the patient's face to make it more comfortable on the chin and the lips; then, we gave the patient the mask and instructions about how to wear it. We recommended to use it all the night and as much as possible during the day ().
After 11 months of active treatment, the patient had a class II molar relationship and positive overjet ().
We removed the rapid maxillary expansion (RME) and took a cephalogram to compare with the T0 radiograph. Comparison between cephalometric analysis at T0 and T1 showed that the correction of the skeletal class III malocclusion was obtained (); the profile changed from flat to concave due to the advancement of the maxilla, becoming more harmonious in the medium and the lower third with respect to the E line.
Interestingly, the gingival recession on the lower central incisor that appeared because of the edge-to-edge contact and poor oral hygiene went to spontaneous resolution after overjet correction ().


A 9-day-old African–American girl presented to the pediatric surgery team for evaluation of a well-circumscribed, nontender, and firm soft tissue mass on the anterior abdominal wall. The mass was noted at birth but was undetected on prenatal ultrasound evaluations. Similar subcutaneous nodules were also noted on the upper back and left hip. The full-term child, born after an unremarkable prenatal course, was in otherwise good health. An abdominal ultrasound on day of life 1 showed the left upper quadrant soft tissue mass to measure 1.9 cm × 1.2 cm × 2.7 cm and confirmed its location in the subcutaneous tissue. A small focus of vascular flow was thought to be consistent with a hemangioma or congenital vascular anomaly. Following a period of observation, abdominal MRI at ∼2 mo of age revealed multiple, rim-enhancing lesions within the abdomen and pelvis (A). These included both osseous and muscular lesions and one within the pancreatic head. An additional 10-mm lesion was noted in the left ventricular free wall on echocardiogram. The radiographic differential diagnosis of these lesions included infection, neurofibroma, multifocal Langerhans cell histiocytosis, metastatic neuroblastoma, and IM.
The child underwent an incisional biopsy of the left upper quadrant subcutaneous lesion, which had remained unchanged in appearance since birth. Intraoperatively, the mass was noted to be cystic and fused to the anterior abdominal wall, which prevented a complete excision without excessive morbidity. Gross pathologic analysis revealed a tan-pink to tan-white, firm, rubbery mass. Light microscopy revealed bland, medium-sized, round to spindled cells with vacuolated nuclei arranged in a fascicular pattern and rare mitotic figures in a collagenous fibromyxoid stromal background. Immunohistochemical stains were performed, and the tumor stained positive for β-catenin (cytoplasmic only) and smooth muscle actin (B; and data not shown). These pathology studies and the clinical presentation were sufficient to make a diagnosis of IM. The child was treated with vinblastine and methotrexate chemotherapy and had a stable disease at 13 mo of age, after 9 mo of therapy. The child has since been lost to follow-up. Whether vinblastine/methotrexate contributed to the disease stabilization is not clear.


A 26-year-old Japanese male presented with headaches, anorexia, and diplopia. The enhanced computed tomography scan showed two small intracranial masses; one was a suprasellar lesion and the other appeared at the aperture of the aqueduct, resulting in obstructive hydrocephalus. No lesions were found in the spinal cord. An endoscopic biopsy was performed of the suprasellar mass, and the accompanying third ventriculostomy resolved the hydrocephalus. The pathology revealed a germinoma and he received three courses of chemotherapy (carboplatin, 450 mg for 1 day; etoposide, 1100 mg for 5 days). This was followed by whole-brain radiation (24 Gy). Ultimately, the intracranial lesions disappeared.
Three years later, however, the patient experienced vacillating paresthesia in his right hand and both legs, but without a focal neurological deficit. Human chorionic gonadotropin β-subunit (βhCG) and α-fetoprotein (AFP) were within normal limits in the serum (βhCG <0.1 ng/ml and AFP 2.2 ng/ml), CSF βhCG was 0.4 ng/ml, and AFP was 0.2 ng/ml. The cytological examination of CSF was negative. However, oligoclonal IgG bands were positive in CSF (IgG index, 0.66; myelin basic protein, 45.8 pg/ml).
The cervical MR revealed a heterogeneously enhancing, expansile intramedullary cord lesion at the C1-C2 level, accompanied by marked edema extending from the medulla oblongata to the C4 level []. There were no accompanying intramedullary or extramedullary lesions in the thoracic or lumbar spinal studies.
The main differential diagnoses included; astrocytoma, ependymoma, or germinoma along with other nonneoplastic diseases (e.g., MS, other demyelinating diseases, or inflammatory myelitis). Due to the potential diagnosis of MS, the patient received steroid pulse therapy with methylprednisolone (1 g/day) for 3 days. The more likely diagnosis of a tumor was later confirmed when the follow-up magnetic resonance imaging (MRI) showed reduced edema around the unchanged contrast-enhancing C1-C2 intramedullary mass [].
One month later, the patient underwent a C1 laminectomy/ C2 partial laminectomy with revised laminoplasty of the C2 spinous process for resection of the intramedullary cervical lesion. A myelotomy was performed along the posterior median sulcus; just under the cord surface, the tumor was grayish, soft, and nonhemorrhagic and appeared to grow into the central canal. As the intraoperative frozen section diagnosis was consistent with germinoma, a sufficient biopsy/decompression was performed without the need for total resection.
The tumor consisted of a mixed population of large cells (e.g., large round nuclei, hyperchromasias, with clear cytoplasm) and mature lymphocytes [two-cell pattern, ]. There were no syncytiotrophoblastic giant cells. Immunohistochemical studies positively stained for placental alkaline phosphatase and C-kit []. Staining for βhCG and AFP was negative, while the Ki-67 labeling index was approximately 30%. These findings confirmed the diagnosis of a germinoma that was unchanged compared to his prior intracranial surgery. Most likely, the cervical tumor resulted from dissemination of germinoma cells through the central canal. The patient recovered from surgery without any sequelae. The postoperative MRI revealed partial resection of the tumor, and he was treated with three courses of chemotherapy (e.g., carboplatin and etoposide) and begun on holospinal radiation (30 Gy).


A 28-year-old male presented with a chronic dry cough and weight loss of 1-year duration, and 2 months of worsening mid-thoracic back pain accompanied by bilateral lower extremity weakness. Notably, there were no known TB exposure, recent travel, smoking, or history of intravenous drug use. On the initial physical examination, he had bilateral rales and left lower extremity weakness (4/5).
Magnetic resonance imaging revealed an epidural collection causing compression of the spinal cord from T5 to T6; this was accompanied by a moderate-sized, left-sided pleural effusion, a left lingular/lower lobe consolidation, and multiple cavitary lesions in the right lung [-]. The patient was initially diagnosed with pulmonary TB/Pott’s disease and myelopathy, resulting in the standard TB regimen of rifampin, isoniazid, pyrazinamide, and ethambutol.
Surgery included a T5-T6 decompression (e.g., costotransversectomy); the largely ventral mass (e.g., anterior to the cord) was removed. This was followed by open reduction and a posterior T4-T8 fusion.
A 1.2 cm × 0.9 cm × 0.6 cm bone tissue fragment from the T6 lamina and 3.0 cm × 2.0 cm × 1.0 cm aggregate from the T6 epidural tissue demonstrated metastatic adenocarcinoma, likely primary from the lung.
The patient’s postoperative course was complicated by an inability to be weaned off the ventilator; the high oxygen requirements were attributed to the left lung disease. A chest radiograph performed the day of surgery demonstrated an enlarging left pleural effusion and a left pneumothorax. There were also patchy right perihilar airspace opacities/scattered nodules, considered infectious/inflammatory []. The patient subsequently experienced acute hypoxemic respiratory failure, acute respiratory distress syndrome, shock, and cardiac arrest. Of interest, the TB polymerase chain reaction came back negative.