A 63-year-old female with no specific history visited the emergency room with complaints of dyspepsia and right lower quadrant pain that had begun 5 days prior. Her vital signs were a blood pressure of 111/75 mmHg, a pulse rate of 100 beats/min, a breathing rate of 24 breaths/min, and a body temperature of 36.3℃. The patient showed acute illness, and physical findings indicated right lower abdominal tenderness, but there was no rebound tenderness. The peripheral blood examination showed leucocytes had increased to 10,800/mm3 and C-reactive protein (CRP) to 10.7 mg/dL; liver function tests and electrolytes showed no abnormalities. No specific findings were observed on the plain abdominal X-ray. The abdominal CT findings showed a diverticulum of ~2 cm on the outside above the colonic wall of the ileocecal valve and ascending colon, which was accompanied by focal wall thickening, fat infiltration and inflammation of >5 cm (). Based on these findings, the patient was diagnosed with acute diverticulitis and was treated with intravenous antibiotics (ceftriaxone, metronidazole) for 10 days. She was discharged with improved symptoms. One month after discharge, the follow-up CT showed a cystic lesion of ~2.5 cm remaining in the area of diverticulitis; therefore, an abdominal CT was performed at the follow-up 3 months later to detect abscess formation. The size of the cystic lesion had increased to ~4 cm, and the focal wall thickening remained (). A biopsy was performed during colonoscopy for differentiation of a chronic abscess, a mucinous cyst and colon cancer. The ulcer lesion was covered with dirty exudates, and bleeding upon easy touch was evident in the proximal ascending colon and ileocecal valve (). The biopsy results confirmed an adenocarcinoma, moderately differentiated, with a carcinoembryonic antigen (CEA) level of 1.4 ng/mL. A right hemicolectomy was performed, and, based on the surgical specimen, the patient was finally diagnosed with a mucinous adenocarcinoma, moderately differentiated, at the T3N0M0 stage. The patient has been followed up without chemotherapy for 90 months; the cancer has not recurred to date.


A 46-year-old male visited the hospital with the complaint of right lower quadrant pain that had started approximately 1 week prior. At the time of the visit, the patient still had right lower abdominal tenderness and rebound tenderness. The peripheral blood examination showed that the leucocyte level had increased to 12,300/mm3 and the CRP to 2.42 mg/dL, and a plain abdominal X-ray showed no abnormalities. The abdominal CT revealed a diverticulum in the proximal ascending colon and a focal wall thickening of ~6 cm in the surrounding area, accompanied by peripheral fat infiltration and inflammation, although the appendix was normal. However, lymph nodes around the ileum were enlarged, so colon cancer could not be excluded (). Clinically, the patient was diagnosed with acute diverticulitis, and the symptoms improved after 5 days of antibiotics (ciprofloxacin) and conservative treatment. One month after discharge, a follow-up colonoscopy was performed for colon cancer differentiation. The colonoscopy showed irregular boundaries surrounding most of the lumen in the proximal ascending colon and an ulceroinfiltrative mass accompanied by changes in the mucosal layer (). The biopsy results confirmed an adenocarcinoma, poorly differentiated, with signet ring cells and a CEA level of 14.4 ng/mL. The patient underwent a laparotomy, but peritoneal seeding was found in all intraperitoneal areas and was strongly adhered in the ileum. Furthermore, because there was an obstruction in the lesion, only simple bypass surgery (ileum-transverse colon) was performed. Subsequently, the patient received palliative chemotherapy, but died 253 days from the date of surgery.


A 47-year-old male visited the hospital with the complaint of right lower quadrant pain that had started 3 days prior. He had a family history of his mother dying of colon cancer, but no other medical history. At the time of the visit, the physical examination results showed right lower abdominal tenderness, but no rebound tenderness. The peripheral blood examination showed that leucocytes had increased to 11,900/mm3 and the CRP to 12.31 mg/dL; no specific findings were observed on plain abdominal X-ray. The abdominal CT findings suggested 4 cm × 1 cm fluid retention in the proximal ascending colon and abscess formation due to diverticulitis perforation towards the outer wall of the ascending colon. In addition, the centripetal focal wall thickening was concurrent; therefore, colon cancer could not be excluded (). Ultrasound-guided percutaneous abscess drainage was performed, and conservative treatment with intravenous antibiotics (ciprofloxacin, metronidazole) was initiated. Three days later, tubography was performed, and because no residual abscess was found, the patient was discharged seven days after admission. After removal of the drainage catheter in the outpatient clinic, a colonoscopy was recommended due to the possibility of colon cancer. However, the patient did not revisit. Approximately two years later, the patient was readmitted with the main complaint of right lower quadrant pain that had started 1 month prior. The abdominal CT suggested diverticulitis-induced bowel perforation in the middle of the ascending colon, accompanied by a thickening of the wall of the ascending colon of ~7 to 8 cm in length (). A colonoscopy was performed to verify the lesion; the results showed an inflammatory mass surrounding the lumen in the proximal ascending colon accompanied by edema ().
The CEA level was 2.8 ng/mL, and the biopsy result was high-grade dysplasia; however, the abdominal CT and colonoscopy findings suggested colon cancer. Therefore, a right hemicolectomy was performed. Based on the surgical specimen, the patient was finally diagnosed with an adenocarcinoma, moderately differentiated, at the T3N2M0 stage. The patient is receiving palliative chemotherapy and has been followed up for 19 months to date.


An 80-year-old man was presented with acute onset of dyspnea associated with cough and sputum. He had suffered from a thyroid goiter for 50 years, although it was non-toxic and growing very slowly. Intermittent dyspnea had begun 10 years earlier and had worsened during the previous week. The patient complained of dyspnea while resting when he was seen in our Emergency Department. He had not undergone previous treatments for his thyroid goiter (). CT scans () obtained on his first admission showed remarkable narrowing of the tracheal lumen caused by enlarged bilateral thyroid glands. The narrowest diameter of the stenosis was 5.8 mm.
To relieve his severe dyspnea, a self-expandable stent was placed in the stenotic trachea (). The normal diameter of the adjacent trachea was 18 mm and the stricture length was 6 cm; therefore, we chose a stent with diameter of 18 mm and length of 8 cm. According to our specifications, the stent was designed to be retrievable, self-expandable, and silicone-covered with barbed metallic (S&G Biotech, Seongnam, Korea). The metal used for the stent was nitinol. The stent had a retrievable design for elective removals when it was no longer necessary or if there were stent-related complications.
Two RF ablation sessions were then performed one and six weeks following stent placement and for which we used an 18-gauge, 1-cm, active-tip, internally cooled electrode (VIVA, STARmed, Goyang, Korea). On the basis of our previous clinical experiences (, ), an ultrasound-guided, trans isthmic approach was used to insert an electrode along the short axis of the nodule, and all nodules were then managed by using the moving shot technique. With the moving shot technique, multiple, small, conceptual ablation units are ablated unit-by-unit by moving the electrode (-). We began ablations with 30 W of RF power. If a transient hyperechoic zone did not form at the electrode tip within 5-10 seconds, the RF power was increased in 10-W increments up to 80 W. The procedure was accepted by our patient, and ablation was terminated when the planned imaginary units were changed to transient hyperechoic zones.
We decided to remove the stent four weeks after the second RF ablation session because the thyroid volume had progressively reduced externally according to follow-up on CT scans (); there was also a concern regarding possible complications, such as stent migration or granulation tissue formation around the stent, resulting from a long-dwelling stent. The last follow-up CT scans () obtained five weeks following stent removal showed stable maintenance of the reduced thyroid volume and an apparent narrow tracheal lumen which was, however, relatively wider than that seen on the initial CT scans. The narrowest diameter measured 6.4 mm on the last follow-up CT scans. This report is recorded four months after the stent removel and the patient was able to breathe normally.


A 22-year-old woman who lost her maxillary right central and lateral incisor prosthesis presented to the Gangneung-Wonju National University Dental Hospital for treatment (). The prosthesis was lost with severe secondary caries around the metal post. Each root canal was incompletely filled with a radiopaque resinous hard material, and the right lateral incisor root had apical rarefying osteitis (). Extraction was necessary due to the large extent of secondary caries and the nonremovable canal filling material. A white spot was found in the maxillary left central incisor with poor oral hygienic control.
Because the patient refused tooth preparation, restoring the teeth with an implant prosthesis was planned. A diagnostic waxing of the teeth determined the installing site of implants to meet the esthetic needs and to fabricate a radiographic stent and a surgical stent. Six weeks after extraction, severe bone loss of the buccal plate was confirmed (). In view of the insufficient implant primary stability, guided bone regeneration was performed as defects were filled with a xenograft material (Bioss; Geistlich Pharma AG, Wolhusen, Switzerland) and covered with a resorbable bilayer collagen membrane (Bio-gide; Geistlich Pharma AG) (). Four months after guided bone regeneration, sufficient hard and soft tissue volume was acquired. A tapered internal hex titanium implants (Implantium; Dentium, Seoul, Korea) were installed, oriented by a surgical guide. An acrylic resin RPD was fabricated to replace the missing tooth during the healing period.
Six month after implant placement, a tissue-punch technique was used to place a screw-retained provisional restoration with a provisional plastic abutment (Plastic temporary abutment, Dentium). The treatment for the white spot of the left maxillary central incisor was also needed. Diagnosis for the white spot was performed with visual-tactile inspection evaluating change of the surface gloss and color after dessication. The lesion surface was hard and shiny, it was diagnosed as an inactive ICDAS (International Caries Detection and Assessment System) code 2 which requires esthetic improvement only. Regenerating the white spot on the prosthesis was not esthetic and removal of the white spot lesion was thus required. Gingival recession after a surgical procedure highlights the white spot in the cervical area (). Furthermore, bandlike whitish discoloration along the perikymata in the middle third coronal area also needed esthetic enhancement. The proposed minimally invasive technique is based on a combined approach of bleaching, resin infiltration and composite resin bonding. In-office bleaching treatment was applied according to the manufacturer's (Lumawhite; Luma Lite, Spring Valley, CA, USA) recommendations. After gingival tissues were isolated using a light-polymerized resin dam (Opal Dam; Ultradent Products Inc., South Jardan, UT, USA), 35% hydrogen peroxide (Lumawhite) was applied onto the left maxillary incisor with consistent coverage of 2 to 3 mm thickness for 8 minutes, for a total of 3 times (). Two weeks after treatment, the white spot lesion was still apparent though bleaching became effective on the normal tooth structure, requiring more effective treatment ().
Consequently, the decision was made to conduct the resin infiltration technique as it was more preservative than microabrasion or macroabrasion. On the enamel surface, careful application of 15% hydrochloric acid gel (ICON-Etch; DMG, Hamburg, Germany) was performed for 120 seconds to remove the surface layer less than 30 to 40 µm (). The acid gel was suctioned and washed away thoroughly. The lesions were desiccated using ethanol (ICON-Dry; DMG) for 30 seconds. At the moment of applying ethanol, the color change in the white spot lesion was confirmed with ethanol penetration. After thorough dessication with ethanol, an infiltrant resin (ICON-Infiltrant; DMG) was placed on the tooth surface for 3 minutes for inside penetration. Excessive resin was wiped away from the surface and the proximal spaces. Light polymerizing was performed for 40 seconds. Applying the infiltrant resin was repeated to compensate for the shrinkage after polymerization. The tooth surface was polished with polishing discs (Sof-lex disk; 3M ESPE, Saint Paul, MN, USA). After resin infiltration, some of the cervical white spot and bandlike whitish discoloration along the perikymata in the middle third coronal area disappeared (). However, resin infiltration in some parts of the cervical white spot lesion was ineffective, requiring an advanced approach.
The limited, unchanged white spot lesion was removed with a carbide bur (Carbide Burs Round; Mani Inc., Tochigi, Japan). The tooth structure was etched with 38% phosphoric acid (Scotchbond Etchant; 3M ESPE) for 15 seconds. The etching gel was washed away and dried. A bonding agent (Adper Single Bond Plus adhesive, 3M ESPE) was applied to the preparation area and light polymerized for 10 seconds. Composite resin (Filtek Supreme XT; 3M ESPE) was placed and polymerized for 40 seconds. Finally, polishing the restoration surface was performed with polishing discs (Sof-lex disk; 3M ESPE) ().
A complete maxillary arch impression was made with a silicone impression material (Exafine, GC, Tokyo, Japan). A customized abutment (Direct Casting Abutment; Dentium) was fabricated. Shade information was transferred to the dental laboratory after taking clinical photos with a shade guide (VitaLumin shade guide; VidentInc, Brea, CA, USA) and a detailed shade map (). A metal ceramic fixed dental prosthesis was cemented with implant cement (Premier Implant Cement; Premier Products Co., Plymouth Meeting, PA, USA) ().
The patient was followed for oral hygiene instruction and no complications were found after 6 months (). However, due to insufficient long term clinical research on color stability for resin infiltration treatment, continuous post-treatment monitoring should be followed. It is also important to monitor the maintenance of proper surface gloss, oral hygiene and health of peri-implant soft and hard tissue.


A 36-year-old female who was the unrestrained passenger in a vehicle, which was involved in a collision and roll over and she lost consciousness briefly and on regaining consciousness, she complained of neck pain. She was transferred to a nearby medical facility where radiographs revealed Hangman's fracture and the patient was subsequently transferred to our department for surgical management. On admission, she was noted to have no neurological deficits, but had tenderness over the cervical spine. Her cranial computed tomography (CT) examination was normal. Her radiographs of the cervical spine revealed Levine and Edwards type IIa Hangman's fracture []. Magnetic resonance imaging (MRI) of the spine revealed evidence of hematoma extending from clivus to C4 anteriorly and from occiput to C7 posteriorly []. There was also evidence of anterior translation and mild angulation at C2-C3 suggestive of type IIa Levine and Edwards Hangman's fracture. In view of the unstable nature of the fracture and the presence of angulation, it was believed immobilization with external orthosis will not be effective and hence, surgical fixation was opted for. Pre-operative traction was not attempted as the patient was not willing for the same. CT studies of the spine showed that the fracture fragments were displaced [] and hence, direct fixation of the pars fracture with pars screws as suggested by Bristol et al. and Dalbayrak et al. was not considered safe or feasible.[] Hence, C1-C3 lateral mass fusion was planned. With the patient kept under skeletal traction in the prone position, proper alignment was achieved under fluoroscopic control and then the region from C1 to C3 was exposed. The C2 root and ganglion were sectioned on either side and the C1 lateral mass was exposed. The C3 lateral masses were exposed on either side or 3.5 mm × 28 mm polyaxial titanium screws were inserted into the C1 lateral masses on either side followed by insertion of 3.5 mm × 14 mm polyaxial screws into the lateral masses of C3. For insertion of C1 lateral mass screw, the entry point was the middle of the lateral mass with 10* medial angulation aiming for the anterior tubercle of atlas and for C 3 lateral mass screw, the entry point was 1mm medial to the center of the lateral mass with 30* lateral and 15* rostral angulation. The exposed posterior surfaces of C1, C2, and C3 were decorticated and autologous grafts obtained from posterior iliac crest was applied as onlay graft and the wound was closed in layers. Post-operative radiographs showed good alignment with proper placement of screws []. Post-operatively, when examined 3 months later, there was no change in the patient's clinical condition except for numbness in the occipital region due to sectioning of C2 root.


A 22-year-old male was the unrestrained passenger in a vehicle involved in road traffic accident. Shortly, after the accident, he complained of severe neck pain. When examined in the hospital, he had no neurological deficits, but had severe pain and tenderness in the neck. His radiographs showed a type III Levine and Edwards Hangman's fracture []. His MRI of the spine showed hematoma extending from the clivus to C4 along with anterior translation and angulation of C2 over C3 along with disruption of the C2-C3 disc []. CT of the spine showed bilateral pars fracture of C2 with significant displacement []. In view of these findings, anterior approach with C2-C3 discectomy and plating was planned. With the patient under traction, the C2-C3 region was exposed through the anterolateral approach and discectomy and grafting of C2-C3 was carried out. However, attempt to plate C2 and C3 was not successful as the diameter of the available plate was greater than the diameter of the base of C2 body as was cautioned by Traynelis and Fontes.[] Hence, grafting alone was carried out. However, post-operative radiographs on day 2 revealed dislodgment of the graft with recurrence of subluxation. Hence, patient was taken up for posterior C1-C3 lateral mass fusion. The same approach as mentioned for case 1 was carried out uneventfully []. Post-operative radiographs showed good alignment with appropriate position of the screws []. Three months later, patient had no neurological deficits, but had a small trophic ulcer in the occipital region owing to numbness in the area and wearing of Philadelphia collar.


A 36 year old male patient presented to us with almost asymptomatic ichthyosiform lesions of three years’ duration which appeared gradually involving mostly the trunk and extremities. [Figures -] At the time of reporting lesions also started appearing over the sides of the face. The lesions were ichthyosiform patches of varying sizes measuring five to ten centimeters in diameter, the borders of which merged with intervening normal skin. The dry adherent pigmented scales were of varying shapes and sizes with mild telangiectasia noted over few lesions. There was no anesthesia either over the lesions or peripherally in glove and stocking distribution. There was history of application of emollient and topical corticosteroids with neither any change in the morphology of the lesions nor their size. There was no history of systemic medication prior to or during the course of the disease. No abnormalities were detected in general survey and systemic examination including absence of lymphadenopathy. A provisional diagnosis of ichthyosiform MF was made. Routine investigations including complete hemogram, chest X-ray, Mantoux test were within normal limits as was his thyroid profile. Histopathological examination of biopsy specimen revealed mild hyperkeratosis with localized parakeratosis, irregular acanthosis and mild spongiosis. [] Upper dermis contained dense lymphocytic infiltrate without any evidence of nuclear atypia or epidermotropism []. There was no granuloma formation. Immunohistochemical study of infiltrate expressed CD3, CD2, CD5 and CD4 but was negative for CD20, CD8, CD7 and CD30.
The above findings prompted us to reconsider our diagnosis to ichthyosiform LPP. The patient was put on systemic phototherapy, but without any improvement even after three months of treatment.


A 28-year-old male presented with asymptomatic cystic growth over vertex area of scalp of 6 years duration. The lesion started as small pea- sized cystic growth and gradually increased to the present size. There was no history of trauma, previous surgery, and similar growth over scalp. The growth was painless and there was no history of any surface changes over the cystic growth. Family history of similar complaints was negative. Examination revealed a 3 × 4 cm sized, mobile, soft to firm cystic growth over right vertex region with normal hairs over the surface []. Palpation revealed no tenderness and the cyst was not attached to the underlying fascia or bone. The growth was excised under local anesthesia. During excision, when skin was incised, a bilobed tumor with capsule was found and the tumor was sent for histopathology examination []. Histopathology showed, capsulated tumor with mixed Antoni A and Antoni B patterns in dermis. The Antoni A component is cellular, and Verocay bodies are a prominent feature. The loosely cellular, pale zones are Antoni B patterns [Figures and ].


A 44-year-old man presented to our department with a 1-month history of dyspnea and cough. There was no history of sputum, fever, loss of weight, and chest pain. He had smoked one pack cigarettes a day for 35 years. He had had mitral valve replacement 10-years ago and pericardiectomy 5-years ago. His current medications included diltiazem, warfarin, and digoxin.
On physical examination, he appeared cyanotic. Blood pressure was 100/70 mmHg, heart rate was 116 beats/min, body temperature was 36.7°C. His arterial oxygen saturation on room air was 84%. Auscultation of the chest revealed inspiratory crackles bilaterally. His abdominal examination was unremarkable. There was obvious (+2) pitting edema on pretibial region. Laboratory findings revealed were normal except Na: 121 mmol/L, K: 4.8 mmol/L, aspartate aminotransferase (AST): 204 U/L, alanine aminotransferase (ALT): 203 U/L, lactate dehydrogenase (LDH): 808 U/L.
The plain chest radiograph demonstrated diffuse opacities affecting both lungs. Thorax computed tomography (CT) scans revealed multiple mediastinal lymphadenopathies up to 22 mm []. In both upper lobes; there were diffuse, multifocal, patchy, ground-glass opacities resulting in a crazy-paving appearance [].
There was no pathological finding of 18-fluorodeoxyglucose (18-FDG) uptake on positron emission tomography (PET) scan.
There were biatrial dilatation, prosthetic mitral valve, and second-degree tricuspid insufficiency on his echocardiography. Pulmonary artery pressure was 45-50 mmHg, ejection fraction of the left ventricle was 60%, and so confirmed diastolic dysfunction.
The patient underwent endobronchial ultrasound (EBUS) with bronchoalveolar lavage and lymph node transbronchial fine needle aspiration biopsies (TBNA) were performed to mediastinal lymph nodes bigger than 2 cm []. The lavage fluid was evaluated for routine cytology, gram stain and bacterial culture, acid fast stain and mycobacterial culture; all tests were negative. The cytology of bronchoalveolar lavage showed inflammatory features. Periodic acid Schiff (PAS) stain was performed for differential diagnosis of pulmonary alveolar proteinosis, but did not stain. The lymphadenopathy biopsies demonstrated no pathological changes. There were blood cells, bronchus epithelium cells, and lymphocytes.
The patient was treated with intravenous furosemide and he improved rapidly. The largest lymphadenopathy size decreased to 9 mm and the ground-glass opacities disappeared on CT scan obtained 1 month after the diagnosis [].


A 51-year-old man visited the Sleep Center with a 2-year history of snoring and voice change. He had complaints about loud snoring and had been witnessed to have obstructive sleep apnea with excessive daytime somnolence. His Epworth sleepiness scale (ESS) score was 13. He denied any history of dyspnea. His medical history included mild hypertension and diabetes. His height was 160 cm, his weight was 62 kg and his body mass index was 24.2 kg/m2.
On physical examination, his Friedman tongue position [] was grade III (only the soft palate is visible) and the tonsils were hidden in the tonsillar fossa. On flexible laryngoscopy, about a 20×20 mm sized round cystic mass at the base of tongue was identified. The cyst bended the epiglottis posteriorly, which made the airway narrow. The vocal cords could not be observed because of the lesion ().
He underwent a full-night laboratory nocturnal polysomnography (PSG). PSG revealed that the respiratory disturbance index (RDI) was 32.2 events per hour of sleep with the supine RDI of 105.8 events/h. The longest apnea duration was 39.7 seconds. The lowest SaO2 was 89%, and the average SaO2 saturation was 97.2%.
The computed tomograms showed a 33×31×27 mm sized well-defined cystic lesion located at the midline of the tongue base, compressing the oropharyngeal airway (). The epiglottis was displaced posteriorly by the cystic lesion.
To evaluate the upper airway obstruction and to find any other obstructive lesion, sleep videofluoroscopy (SVF) was performed with administration of 0.05 mg/kg of midazolam as described previously []. SVF showed that the pharyngeal airway was completely obstructed during both inspiratory and expiratory phase when the mass pulled the epiglottis down and backward. The tip of epiglottis was in contact with the posterior pharyngeal wall irrespective of respiratory cycle ().
Sistrunk operation was performed via external approach instead of oral approach, because the mass was somewhat large. Round cystic mass was removed uneventfully. Soon after the operation, the signs and symptoms of OSA such as snoring and daytime sleepiness ceased and his voice was normalized. His ESS score reached 4.
Postoperative flexible laryngoscopy showed that the mass was disappeared completely (). Postoperative PSG (3 months after operation) showed that all the parameters were nearly normalized or improved. The RDI decreased to 4.0 events per hour and the supine RDI decreased to 13.4 events per hour. The longest apnea duration was 26.5 sec. The lowest SaO2 was 92%, and the average SaO2 saturation was 97.8%.
Postoperative SVF showed that the displaced epiglottis returned to normal position and the pharyngeal airway was not collapsed, however, during inspiration the hypopharyngeal airway was partially obstructed at the level of the epiglottis, which could explain the remnant RDI of 4.0 ().
The histopathology showed that the cyst was lined by stratified squamous epithelium, and that the deeper tissue showed fibrosis, skeletal muscle fragments and laryngeal gland cysts, which was consistent with a TGDC (). There has been no evidence of recurrence for 1 year.


A 6-month-old baby boy was referred for a pelvic X-ray because of restricted abduction of the right hip. On the presented image (Fig. ), there were no signs of dysplasia and the hips appeared normal. Because the image had clearly been electronically collimated, the radiologist asked for the original image. On the original non-collimated radiograph, a left distal femur fracture was suspected (Fig. ). An additional lateral view of the femur was made, confirming the fracture (Fig. ). This was followed by the non-accidental injury protocol.


A 3-year-old girl was referred for an X-ray of the cervical spine, because of mild torticollis after a fall. On the electronically collimated images (Fig. ) the fracture of the right clavicle, responsible for the torticollis would be missed (Fig. ).


An 11-month-old baby girl was referred for abdominal X-ray, in addition to abdominal ultrasound, because of continuous crying and a painful abdomen (Fig. ). In the initially presented radiographs no abnormalities were found and the child was admitted to the hospital for observation. The following day a swollen left upper leg was noted by a nurse. An X-ray of the left femur was made, demonstrating a distal femoral fracture (Fig. ). When asked for the original non-collimated X-ray of the abdomen, the distal femoral fracture could be seen retrospectively, thereby confirming that the fracture was already present at first presentation and probably the cause of the initial crying (Fig. ). This was followed by the non-accidental injury protocol.


A 32-year-old Korean woman found that she had visual loss in her right eye when she woke up in the morning and was referred to our clinic for acute CRAO 16 hours after symptom occurrence. Eight years prior, she had presented with itching and tender violaceous erythematous non-elevated patches with central necrotic vesicle on the dorsum of both the lower legs and feet () The lesions were improved by colchicines 0.6 mg/day, hydroxyzine, and methylprednisolone 4 to 8 mg/day. Biopsy showed obliterative vasculopathy consistent with LV (). A segmental limb pressure test with a bidirectional Doppler test showed normal blood pressures in all four extremities. She was intermittently treated with pentoxifylline and mupirocin ointment for skin lesions for three years. She was in good medical condition except for the skin lesions and had no experience of smoking. She was not taking oral contraceptives or suffering from migraine. She had one son and no history of spontaneous abortion.
At presentation, her visual acuity was hand motion in the right eye and 20 / 20 in the left eye. Fundus examination of the right eye showed whitish edematous retina, a cherry-red spot, and narrowing and segmentation of retinal arteries suggesting acute CRAO () Laboratory test results, including rheumatological tests such as anti-nuclear antibody; anti-Ro and anti-La antibodies; anti-neutrophil cytoplasmic antibody; anti-DNA antibodies; rheumatoid factor; cryoglobulin; antithrombin activity; protein C and S activity; D-dimer; anti-beta2-glycoprotein; anticardiolipin antibody; lupus anticoagulant; prothrombin gene mutation; factor V Leiden gene mutation; and homocysteine, were all within normal limits except for slightly elevated C-reactive protein (0.62 mg/dL) and fibrinogen (425 mg/dL). The serology tests were positive for hepatitis B antigen while other serology tests were normal. Echocardiography and Holter monitoring showed normal findings.
She consented to receive intra-arterial thrombolysis after being informed of the possible risks and benefits of the intervention. Cerebral angiography showed severe focal vasospasm of the right internal carotid artery as soon as the catheter was placed (), which was subsequently relieved by continuous intra-arterial infusion of nimodipine 5 mg. Selective angiography of the origin of the right ophthalmic artery showed no definite thrombus or steno-occlusive lesion within the ophthalmic artery (). The ophthalmic artery was infused with a fibrinolytic agent (500,000 units of urokinase) until the maximal dose of fibrinolytics was reached as based on our protocol, but there was no visual improvement during the procedure. Seven hours after intra-arterial thrombolysis, retinal arterial perfusion on fundus fluorescein angiography (FFA) did not improve (arm to retina time, 29 seconds; arteriovenous transit time: about 4 minutes) (). FFA three days after intra-arterial thrombolysis showed nearly complete restoration of retinal arterial perfusion (arm to retina time, 17 seconds; arteriovenous transit time, about 30 seconds) with the exception of the macular area (no reflow phenomenon). Six weeks after thrombolysis, her visual acuity remained hand motion and fundus photography showed severe retinal atrophy in the macula and disc pallor (). Retinal perfusion was restored sparing macula on FFA () and macular photoreceptor disruption was observed on optical coherence tomography.


A 39-year-old man underwent Moh's micrographic surgery to treat an infiltrative basal cell carcinoma (BCC) arising from the right nasal sidewall. The resulting full thickness defect measured 2 cm×2 cm and involved the dorsum of the nose, the right side wall, and the nasal tip. A three-stage forehead flap was planned for reconstruction.
During the first stage of surgery, in order to improve the final aesthetic outcome, the remainder of the involved subunits and the left nasal side wall were completely excised. The resulting final defect measured 4 cm×5 cm. Reconstruction of the lining and structural support of the nose were achieved using a mucosal advancement flap and septal cartilage grafts, respectively. A template using the excised nasal skin was used to accurately mark out a paramedian forehead flap. The flap was raised from its superior aspect to incorporate the skin, subcutaneous fat and part of the frontalis muscle. It was rotated 180 degrees and inset without tension. Once the primary nasal reconstruction was complete, the inferior aspect of the donor site defect was closed directly.
The superior aspect of the donor site measured 6 cm×5 cm () and was treated using the ReCell system in the manner described above.
Photographic assessment of the donor site was undertaken at 1 year () []. The Patient and Observer Scar Assessment Scale (POSAS) [] yielded a score of 32 (11=normal skin, 110 worst scar imaginable).


A 72-year-old man with a medical history of type 2 diabetes underwent Moh's micrographic surgery to treat a morphoeic BCC arising from the nasal tip. The resulting full-thickness defect measured 3 cm×2 cm and involved the nasal tip, the dorsum of the nose and both side walls. A three-stage forehead flap was planned for reconstruction and was performed in a manner similar to that described for case 1.
Following the first stage of surgery, the donor site was initially allowed to heal by secondary intention. One month later, it was noted that healing was delayed and epithelialization was minimal (). Consequently, during the second stage of surgery, undertaken 6 weeks after the primary reconstructive procedure, the donor site was treated using the ReCell system in the manner described above.
Photographic assessment of the donor site was performed 1 year after the application of ReCell (). The POSAS score was 22.


A 42-year-old female patient underwent a 2-cm wide local excision (WLE) of a malignant melanoma scar on her scalp (). The resulting wound measured 5 cm×7 cm with periosteum forming the base (). The patient was concerned about the aesthetic problem of alopecia following a skin graft as well as donor site morbidity. As a result, the ReCell system was used in the manner described above. At 1 year, the wound had contracted sufficiently to be covered by the patient's hair, making it imperceptible during day-to-day activities (). The POSAS score was 45.


A 6-year-old boy was diagnosed with cystic fibrosis at the age of 15 months due to
recurrent respiratory infections, diarrhea and therapeutic response to pancreatic
enzymes. Three sweat tests were negative.
At the age of 5 years, the patient began to experience pain in the lower limbs, laxity
of joints, lameness and frequent falls. There was no history of fractures, liver or
kidney disease, use of medications associated with osteoporosis or rickets, lack of
sunlight exposure, low calcium or vitamin D diet, or reports of metabolic bone disease
or bone dysplasia in the family. There is no evidence for consanguinity. On physical
examination his weight was 16 kg (10th centile), height 103 cm (5-10th centile), with
P1/G1 Tanner pubertal stage. The liver was palpable 3 cm below the right costal margin.
He also presented discrete genu varum, hyperextensible elbow joints and increased
anteroposterior diameter of thorax. Review of medical records showed chronic leukopenia
(leukocytes: 3.1-3.5 x 103/µL), neutropenia (segmented neutrophils: 15-22%),
but normal hemoglobin, hematocrit, and platelet count. A radiological study showed
metaphyseal chondrodysplasia, sclerosis and pseudocysts in radial, humeral, ulnar,
tibial and femoral metaphysis (). Bone
densitometry showed a z-score of -1.6.
Evaluation of serum calcium, phosphorus, alkaline phosphatase, 25(OH)Vitamin D,
1,25(OH)Vitamin D, parathyroid hormone, liver and kidney function, thyroid function,
electrolytes, lipase, amylase, blood sugar, immunoglobulin (IgG, IgA and IgM) and CD4
and CD8 lymphocyte counts were all normal. Hemoglobin electrophoresis showed: Hemoglobin
(Hb) A1: 91.7%; Hb A2: 2.6%; Hb F: 5.7%. Blood sugar, insulin and an oral glucose
tolerance test were normal, in spite of a raised glycated hemoglobin level of 6.5%
(normal: < 5.6%). Fecal fat excretion tested positive and an abdominal ultrasound
revealed increased pancreatic echogenicity. Sanger sequencing( demonstrated
known pathogenic mutations in exon 2 of the SBDS gene: 183-184TA - CT K62X (Stop codon
no exon 2) and 258+2T-C transition. In addition, a non-pathologic silent nucleotide A to
G transition at position 201 in the SBDS gene was also detected in heterozygosis.


A 47-year-old male presented to the ED with an acute flare of his chronic severe left hip due to osteoarthritis. He was being followed as an outpatient by the orthopedic service and was scheduled for an upcoming fluoroscopy-guided therapeutic hip injection. He complained of 8/10 pain in the left hip that was significantly limiting his daily functioning. In conjunction with orthopedics consultation and after informed consent, the left hip was injected according to the previously stated technique. At follow up 1 week later, the patient reported significant improvement in his pain and increased daily activity without evidence of infection.


A 67-year-old woman, 153 cm tall, and weighing 54 kg visited our hospital. She had fallen and experienced bone fractures in her wrist before she visited our hospital. At her first visit, she complained of right hip joint pain (P4). Plain radiographs showed OA at KL grading III (Fig. ). Since then, joint pain has shown repeated remission and deterioration. However, her pain has recently much decreased (P0). Recent plain radiographs also showed OA at KL grading III. Recent hip MRI showed no signal changes in right hip joints (Fig. , ).


A 74-year-old woman, 152.0 cm tall and weighing 57.5 kg with no history of OA visited our hospital. At her first visit, she complained of right hip joint pain (P4). Plain radiographs showed OA at KL grading IV (Fig. ). Since then, joint pain has shown repeated remission and deterioration. Recent plain radiographs also showed OA at KL grading IV. MRI was performed at the time of right hip joint pain (P4). MRI showed high signal change in the entire femoral head and acetabulum in T2-STIR (Fig. ).


A 72-year-old woman, 155.0 cm tall and weighing 57.0 kg with no history of OA visited our hospital. Bilateral hip joint pain started about 5 years ago; since then, bilateral joint pain has improved and worsened repeatedly. She first visited our hospital about 6 months ago and complained of continuous right hip joint pain (P4). Her left hip joint pain has improved (P1). Plain radiographs showed bilateral hip OA of KL grading III (Fig. ). MRI analysis showed no signal changes in the left hip joint, and bone cysts and local signal changes in her right femoral head (Fig. , ).


A 4-year-old girl presented with progressively decreasing mouth opening since birth and facial deformity. According to the parents, at birth there was a large soft-tissue mass involving the entire right cheek with restricted mouth opening, which was excised at 6 months of age when biopsy confirmed it to be a mature teratoma. Decrease in mouth opening continued even after surgery, with hypertrophy of the right infraorbital region.
On examination, a large cheek scar with loss of subcutaneous tissue severely restricted mouth opening (inter incisor distance < 3 mm) and maxillary hypertrophy in the infra orbital region were observed []. Oral hygiene was poor with absence of anterior teeth in both upper and lower jaw.
Computed tomography face revealed loss of joint space, bony ankylosis between the coronoid process and zygomatic process of the maxilla with hypertrophy of infraorbital rim and the adjoining zygomatic arch [].
Child underwent exploration of the facial skeleton using the previous preauricular scar. All soft-tissue adhesions between the cheek skin and the mandible were excised. Articular ankylosis had a fibrous component. Coronoidectomy was done to release the bony block between the infra orbital rim, zygomatic arch and the mandible with dramatic improvement in mouth opening []. After ensuring adequate mouth opening the hypertrophied malar prominence was shaved to ensure facial symmetry. As there was extensive scar tissue around the malar region most probably due to previous surgery, ultra-thin silicone sheet was used as an inter-positional material instead of standard temporalis muscle flap.
After complete transaction of the ankylotic bone, the gap was kept bare enough so as to pass 0.2 mm ultra-thin silicon sheet of size 5 × 5 cm []. Medially, it was anchored with 3-0 nylon to medial pterygoid muscle and laterally to periosteum of zygomatic arch so as to fix the sheet securely and also to cover the two transacted bone ends completely.
Post-operatively child was put on extensive physiotherapy using an acrylic dynamic jaw exerciser [ and ]. After 6 months of follow-up child has painless jaw movement with 2.5 cm inter incisor distance. Child is now planned for further refinement of facial contour.


A 65-year-old man visited the Department of Periodontics, Hasanambha Dental College and Hospital with a history of a swelling that had gradually increased in size during previous 2 years. He had no history of previous swelling in the oral cavity. Past medical and family history was non-contributory.
Intra oral examination revealed a solitary, pedunculated mass involving buccal interdental papilla and attached gingiva in relation to 26 and 27 []. Mass was pink in color with a smooth surface, measuring approximately 1.5 cm × 2 cm. No surface ulceration was noted. On palpation, it was non-tender and firm in consistency.
Intra oral periapical radiograph showed no significant bony changes. Provisional diagnosis of POF was considered.
Thorough scaling and root planning was performed to eliminate the irritating factors and after a week, complete surgical excision of the lesion was performed under local anesthesia. To prevent recurrence, complete removal of the lesion and gingival curettage is ensured followed by oral hygiene maintenance instructions to the patient.
Microscopic examination showed fibro-cellular connective tissue interspersed with plump fibroblasts in between the collagen bundles, surfaced by parakeratinzed stratified squamous epithelium []. Stroma showed large trabeculae of lamellar bone [] and scattered basophilic, cementum like substances []. The histopathological features were diagnostic of peripheral ossifying fibroma.
Healing was uneventful [] and patient is followed-up for 12 months without any recurrence.


A 59 year-old female presented with seizures. Computed tomography angiography (CTA) head demonstrated a giant right middle cerebral artery (MCA) aneurysm with mass effect on the adjacent frontal and temporal lobes (). The decision was made to treat the aneurysm with a flow-diverting stent. The patient underwent digital subtraction angiography (DSA), revealing a partially thrombosed, giant right MCA bifurcation aneurysm with a recanalized portion measuring 11.1×7×7 mm. A ReFlex 058 catheter (Reverse Medical, Irvine, CA, USA) was advanced through a 6 French shuttle sheath placed in the right common carotid artery (CCA) into the distal right internal carotid artery (ICA). Under continuous roadmap guidance, a Marksman microcatheter (ev3, Irvine, CA, USA) was advanced over a Transcend microguidewire (Boston Scientific, Fremont, CA, USA) through the ReFlex catheter into an M3 MCA branch distal to the aneurysm. A 3.25 × 25 mm PED was deployed through the Marksman catheter across the neck of the aneurysm.
However, during deployment of the PED, its proximal edge became pinched (). After removal of the ReFlex catheter, attempts to dilate the proximal portion of the stent with a balloon were unsuccessful. After control angiograms through the guide catheter in the CCA demonstrated sluggish flow through the distal right MCA branches, the decision was made to remove the PED. However, since the deployment wire had been removed from the stent, it was no longer possible to retract the proximally pinched stent. The Marksman catheter was removed and a final control angiogram showed less delay in distal MCA flow, no thrombus formation on the stent, and no evidence of distal emboli. The patient was taken to the intensive care unit on therapeutic anticoagulation with a heparin infusion in addition to standard dual antiplatelet therapy (aspirin 325 mg and clopidogrel 75 mg daily). She awoke with a left pronator drift but was otherwise neurologically intact. However, post-procedural CT perfusion continued to show delayed flow through the right MCA.
Therefore the patient was taken back to the neurointerventional suite the next day. Repeat DSA found thrombus formation along the proximal PED with sluggish filling of the distal MCA. An attempted thrombolysis was performed with an infusion of 10 mg of abciximab but was unsuccessful. Next, multiple attempts were made to retrieve the PED utilizing a variety of devices including Goose Neck snares (Microvena, White Bear Lake, MN, USA), an Alligator retrieval device (Chestnut Medical Technologies, Menlo Park, CA, USA), and a Merci Retriever (Concentric Medical, Mountain View, CA, USA) but they all failed. Ultimately the procedure was aborted. The final control angiogram at this point demonstrated a large, flow-limiting thrombus in the M1 trunk and very sluggish flow distally. The patient began to develop a dense left-sided hemiparesis after the failed retrieval attempt and post-procedure magnetic resonance imaging (MRI) demonstrated multiple acute right MCA distribution thromboembolic infarcts ().
Due to the patient's deteriorating neurological exam and the inability to retrieve the partially thrombosed PED by endovascular approaches, neurosurgery was consulted and we emergently decided to take the patient for microsurgical extraction of the PED to restore flow to the MCA. Upon performing a wide right-sided craniotomy, the giant, partially thrombosed MCA aneurysm was immediately evident upon dural opening. The calcified dome was opened and intra-aneurysmal thrombus was debulked. Upon removal of the thrombus, the lumen of the MCA was exposed and the PED was easily removed (). The proximal MCA M1 segment was identified just distal to the ICA bifurcation and proximal vascular control was achieved with a temporary clip. The M2 branches were seen incorporated within the aneurysm neck. The excess aneurysm dome was resected but due to the anatomy of the neck, the opened aneurysm dome could not be reapproximated with a clip. Therefore the remainder of the aneurysm was resected, resulting in detachment of the posterior MCA M2 division from M1 trunk. The senior author (KCL) attempted to reanastamose the posterior M2 division to the M1 but due to the friable nature of the vessel walls, the 8-0 nylon suture would not hold. Given the length of time that the vessel had been occluded and the likelihood that the infarct had completed, the decision was made to abort the surgery at this time to prevent hyperperfusion injury. The clips were left in place and the bone flap was left off to allow for infarct-related edema. As expected, the patient awoke from surgery with a dense left-sided hemiplegia. The patient underwent percutaneous gastrostomy and was discharged to a rehab facility 1 week post-operatively. The patient subsequently underwent cranioplasty 2 months later with an unchanged neurological exam.


A 29-year-old Caucasian male presented to our emergency department with complaints of decreased central vision in his left eye (LE), detected a few hours before. Ten years before, he had undergone ophthalmic surgery with phakic lens implantation due to high myopia in both eyes. The patient was taking benzodiazepines and beta-blockers due to panic disorder. He denied systemic disease known to be associated with choroiditis, systemic symptoms or recent illness. There was no relevant family history.
Best-corrected visual acuity (BCVA) in the LE was counting fingers at 50 cm. He described having blurred central vision in the LE tested on the Amsler grid. Fundoscopy of the LE evidenced multiple, round and yellowish lesions in the macula and nasal to the optic nerve, without signs of intraocular inflammation (fig. , left). BCVA in the right eye was 20/20, and ophthalmological examination showed slight changes of the RPE.
OCT showed increased retinal thickness with detachment of the neuroepithelium and a slight RPE detachment in the LE (fig. , middle). FA revealed hyperfluorescent lesions with blurred borders in the macula (fig. , right).
We performed a chest X-ray and an analytic study to exclude autoimmune and infectious diseases. Afterwards an intravitreal injection of bevacizumab (1.25 mg/0.05 ml) was administered in the LE, showing a significant reduction of the neuroepithelial detachment (fig. ). Four months later, BCVA in the LE was 20/200 and no increased retinal thickness was seen on OCT.
One year later, the patient was reexamined and showed a BCVA of 20/40 in the LE. FA and OCT appearances confirmed no active lesions, and fundoscopy revealed a chorioretinal scar on papillomacular beam (fig. ). The same findings were also observed 2 years later.


A 43-year-old, right-handed woman presented with complaints of pain and discomfort in her right shoulder. She had no history of previous injury or minor problems with her shoulder. The symptoms had begun after labor related to institutional food service for 1½ years. She had occasionally experienced a catching sensation and aggravated pain in the shoulder with labor activity. Symptoms were not alleviated by rest or non-steroidal anti-inflammatory drugs. Physical examination revealed positive findings for Neer's sign and Hawkins impingement test, Speed test and O’Brien's test. Patient's rotator cuff muscle strength was normal and no musculature atrophy was observed. The active range of motion (ROM) of the shoulder joint was mildly restricted during flexion, abduction and internal rotation, but passive ROM showed full range. The laboratory investigations including erythrocyte sedimentation rate and serology showed no significant results. Plain radiograph of shoulder demonstrated multiple rounds and ring like calcifications at the right glenohumeral joint with entheophyte at greater tuberosity of right humeral head []. Magnetic resonance imaging (MRI) of the shoulder showed a fluid-filled paralabral ganglion cyst that originated at the posterior superior labrum, forming a large cystic mass located at suprascapular and spinolgenoid notch around the spine of scapula [Figure and ]. The cyst originated at a tear of the superior labrum and extended to the posterior aspect of biceps long head origin [Figure and ]. The axial MRI also revealed fatty mass, which showed iso-signal intensity to the subcutaneous fat on all sequences, under intermuscular space located between anterolateral cortex of the humeral head and anterior belly of the deltoid muscle []. Another encapsulated mass was found in intermuscular space between infraspinatus and the deltoid muscle []. Abundant villous projection on the medial portion of the mass was located with multiple rings like or round low intensities on all pulse sequences. It showed background low signal intensity on T1-weighted image and high signal intensity on T2-weighted image, representing calcification arising from chondroid matrix [Figure and ]. Arthroscopy revealed a type II SLAP lesion with mild synovitis, but the articular cartilage of the humeral head and glenoid was intact. The detached posterior-superior labrum was fixed with sutures by insertion of two metal anchors. Patient subsequently underwent a separate incision laterally around the acromion and medially along the lateral one-third of the spine of the scapula. The origin of the deltoid was detached from the acromion and from the exposed part of the spine of the scapula. We retrieved LA and lipoma respectively. Lipoma size was 1.8 cm × 2.2 cm × 1.7 cm. LA size was 7.1 cm × 2.8 cm × 2.6 cm in the form of encapsulated mass. Opening the capsule revealed villous projection of fatty synovial tissue associated with central large whitish cartilaginous nodules attached to villous synovium []. Histologic examination demonstrated that diffuse papillary proliferation of the synovium with near total replacement of subsynovial tissue into mature fat tissue. Extensive area of osteochondral metaplasia in villous fat tissue was also seen [Figures and ].
Post-operatively, the arm was placed at the side in a sling for 3 weeks. Passive motion including external rotation of the shoulder and ROM of the elbow were started immediately. This was followed by a home-based rehabilitation program of progressive ROM. Strengthening exercises for the rotator cuff, scapular stabilizers and deltoid were started 6 weeks post-operatively. She achieved full recovery in her affected shoulder within 3 months of surgery. At 2-month follow-up, the ultrasonogram demonstrated complete removal of LA, lipoma and cyst. She was asymptomatic and showed no recurrence of calcification on plain radiographs at a final follow-up 3 years after surgery.


A 13-year-old male patient attended the Department of Periodontics, Mahatma Gandhi Post Graduate Institute of Dental Sciences, Pondicherry, in May 2011 for gingival enlargement and difficulty in mastication. The patient was otherwise healthy. He first noticed enlargement 6 months earlier along the buccal aspect of tooth 26, which increased in size slowly to the present clinical picture. The clinical examination was significant for the presence of pigmented diffuse gingival enlargement along buccal and palatal aspects of 24, 25, 26, 27 and lingual aspects of teeth 34, 35, 36, 37 []. Gingival enlargement was extending up to middle third of the crown of tooth 24, partially covering the occlusal surface of teeth 25, 26 and completely covering the tooth 27 [] and extending up to occlusal surface of teeth 34, 35, 36, 37. The gingival enlargement was firm in consistency, painless, brownish black in color with well-defined borders and there was no associated erythematous background.
Other significant dental findings included root canal treated fractured tooth 21 and congenitally missing tooth 23. Patient's oral hygiene was deemed satisfactory. There was no significant medical history. Patient was not having any associated cutaneous pigmentary changes. Based on the clinical findings, provisional diagnosis of idiopathic gingival enlargement was made and gingivectomy was planned for the treatment.
Patients’ parents provided oral consent for treatment prior to the initial and additional tests and the dental treatment that followed. Routine blood investigations were found to be within normal limits. Gingivectomy was performed under local anesthesia on the buccal aspect of II quadrant [] and the excised tissue was sent for biopsy []. One week later, gingivectomy was performed on the palatal aspect of II quadrant followed by lingual aspect of III quadrant after 1 week. Kirkland knives were used for incisions on the facial and lingual surfaces. Orban periodontal knives were used for interdental incisions. Patient experienced an uneventful post-operative recovery [] at 1 year recall examination there was no recurrence of gingival enlargement at the surgical sites []. Patient will continue to be monitored on a 6 month basis and any new gingival enlargement/lesion will undergo histopathologic investigation.
Histopathological examination revealed numerous dendritic melanocytes distributed in basal and suprabasal layers of acanthotic epithelium. Melanocytes did not display any cytologic atypia []. The connective tissue was normal except for the presence of occasional eosinophils. There was no spillage of melanin pigment/melanocytes in subepithelial zone []. Histopathologic diagnosis of oral melanoacanthoma was rendered.


A 66-year-old gentleman had generalized tonic-clonic seizures (GTCS) since the age of 14 years, which was well controlled for nearly two to three decades with antiepileptic drug (AED) viz., phenytoin – 300 mg/day and phenobarbitone – 120 mg/day. Cranial CT scan was reported to be normal. For the last 10 months, there was recurrence of seizures. The seizure frequency was 1-2 episodes per month in spite of polypharmacy with valproate (900 mg/day), carbamazepine (800 mg/day) and phenobarbitone (120 mg/day). He did not have any comorbid illnesses such as ischemic heart disease, diabetes mellitus or hypertension.
He was presented to us 3 days following an episode of GTCS with a confusional state. Initial examination revealed a disoriented patient with gait ataxia, fine tremor of outstretched hands, pes cavus and hammer toes and diminished ankle jerks. There were no other neurological or systemic deficits. Routine investigations including hemogram were within normal limits. Routine ECG on admission was normal. Scalp EEG at admission showed generalized slowing in theta range and there was no epileptiform activity. Routine serum biochemical parameters including liver and renal function tests and electrolytes were normal. Plasma ammonia was slightly raised (76.8 μmol/L; range: 11-35 μmol/L). MR imaging of brain carried out showed mild generalized atrophy with a cystic lesion secondary to gliosis of the left anterior temporal region []. The serum levels of phenobarbitone was elevated [53.35 μg/ml; reference range: 15-40 μg/ml], while that of carbamazepine was 7.11 μg/ml [range: 4-12 μg/ml] and valproate was 33.58 μg/ml [range: 50-100 μg/ml] were within therapeutic limits. A possibility of chronic uncontrolled symptomatic localization related secondary generalized epilepsy due to gliosis in the left temporal region with phenobarbitone toxicity was considered after evaluation. Tablet phenobarbitone was tapered and levetiracetam was initiated (1000 mg/day).
On the third day of hospitalization, the patient had a generalized seizure. During the immediate postictal period, he was drowsy and diaphoretic, with shallow respiration, bradycardia [pulse rate: 52/min, feeble radial pulse] and hypotension [systolic blood pressure: 60 mmHg]. There was no cyanosis. An electrocardiogram obtained at this time showed a heart rate of 43/min, with p-wave coming after QRS complex suggestive of junctional AV rhythm []. He was given supportive treatment with nasal oxygen (3 L/min), intravenous fluids and dopamine (10 μg/kg/min). About 40 minutes later, the heart rate normalized to 72 per minute and blood pressure rose to 100/80 mmHg. The 2-D echocardiogram showed only a mild left ventricular hypertrophy. The serum creatine kinase-MB [21 U/l; normal 9-25 U/l) and Troponin-I done after 4 hours was 0.6 ng/ml (ref: Up to 0.1). Repeat Troponin-I was 0.6 ng/ml. A repeat ECG carried out 2 hour later was normal []. He recovered completely and at the time of discharge 11 days later, he did not have any neurological deficits.
This event led to a possibility of self-remitting in-hospital symptomatic ictal bradyarrythmia (junctional AV rhythm) and hypotension during a generalized seizure. After 6 months of follow-up, he was asymptomatic and seizure free on levetiracetam and valproate.


An 11-year-old girl with no significant past medical history presented to the ED complaining of 2 days of worsening headache, vomiting, and double vision. Approximately 2 weeks earlier, the patient was seen by her primary care physician (PCP) for evaluation of flu-like symptoms, headache, and left-sided neck pain. Examination at that time revealed an erythematous right tympanic membrane. The patient was diagnosed with AOM and started on oral amoxicillin/clavulanate. The patient continued to complain of worsening headache for 7 days following PCP evaluation, at which point she developed diplopia, nausea, and vomiting. She was subsequently taken to the ED for evaluation.
Non-contrast CT revealed an asymmetrically hyperdense right transverse sinus []. Brain MRI demonstrated a thrombus in the right transverse sinus as well as fluid within the right mastoid air cells consistent with mastoiditis [Figures -]. A filling void in the right transverse sinus, consistent with a right transverse sinus thrombosis, was found on MRV [].
Neurology evaluation did not reveal any focal abnormalities but ophthalmic examination was incomplete; LP was not recommended. The patient was admitted to the pediatric intensive care unit where heparin drip and intravenous antibiotics were started. Hypercoagulablity studies were negative. Ophthalmology consultation found a small right esotropia in primary gaze; optic disc edema bilaterally; and nerve fiber layer hemorrhages more pronounced in the right fundus than left. The patient was diagnosed with papilledema and palsy of the right cranial nerve VI, secondary to increased ICP.
The patient was discharged on oral cefdinir without performing an LP or ICP-lowering therapy. Outpatient ophthalmology follow-up 2 weeks later found worsening headache, persistent papilledema, and esotropia in primary and right-gaze. Formal visual field testing revealed enlarged blind spots bilaterally. The patient was referred to the ED for LP which revealed an opening pressure of 310 mm H2O (upper limit of normal 210 mm); the patient was started on oral acetazolamide. CSF studies were otherwise unremarkable. Follow-up examination 2 weeks later revealed improvement in headache and complete resolution of diplopia and esotropia.


A 47-year-old Hispanic man with a history of end-stage kidney disease secondary to diabetic nephropathy underwent deceased donor renal allograft transplantation. His immediate posttransplant course was unremarkable, with prompt functioning of his allograft and decreasing creatinine levels. His immunosuppressant regimen included tacrolimus, mycophenolate sodium and corticosteroids. Ten days after the transplant, he developed suprapubic abdominal pain that was thought to be due to the indwelling ureteral stent, necessitating its removal with complete resolution of his discomfort.
Approximately 5 weeks following his transplant, the patient presented with fatigue, diarrhea and oligoanuria, without any associated fever or respiratory symptoms. He had significant allograft dysfunction with a creatinine level of 8.1 mg/dl up from a nadir of 2.7 mg/dl. Doppler ultrasound of the renal allograft showed an area of hypoperfusion affecting the upper pole of the renal allograft, with a moderate degree of hydronephrosis. A percutaneous nephrostomy tube was placed without any improvement in urine output or renal function. A renal allograft biopsy was then done to further elucidate the etiology of his graft dysfunction. This revealed diffuse C4d-positive staining in the peritubular capillaries suggestive of acute humoral rejection; however, the biopsy was also remarkable for numerous, broad, ribbon-like, nonseptate, fungal hyphae with wide-angle branching suggestive of Mucor infection. There was a focal cortical infarct accounting for approximately 10% of the overall biopsy, with focal small vessels containing fungal hyphae (fig. ). The patient was prepared for transplant nephrectomy and arteriovenous fistula thrombectomy. Postoperatively, his anti-rejection medications were discontinued, amphotericin B and micafungin were started and hemodialysis was initiated.
A pathology exam performed after the nephrectomy (fig. ) showed significant cortical infarction involving more than half of the cortex, with patchy, widespread involvement of fungal hyphae that involved the ureteral lumen, ureteral wall, renal parenchyma and numerous large and small vessels. Chest CT showed a large right upper lobe cavitating mass measuring 4.9 × 2.8 cm, with scattered areas of smaller nodules present throughout both lungs (fig. ). Abdominal CT revealed a wedge-shaped area of hypodensity within the anterior spleen compatible with infarction, along with additional areas of hypodensity within the spleen. There was also a nonspecific hypodensity in the right hepatic lobe. Head and maxillofacial CT were normal. The patient underwent a CT-guided biopsy of the right upper lobe of the lung, and pathology confirmed the clinical suspicion of disseminated mucormycosis. Within 1 week of presentation, the patient became hemodynamically unstable, requiring a transfer to the intensive care unit for mechanical ventilation and vasopressor support. As his mental status showed significant decline, a repeat head CT was done. This revealed interval development of areas of subcortical hypoattenuation involving the bilateral frontal lobes, bilateral occipital lobes and bilateral cerebral hemispheres, suggesting multifocal cerebritis with no abscesses (fig. ). The patient's condition continued to deteriorate, and he developed supraventricular tachycardia and the inability to tolerate hemodialysis. His family elected for comfort care and the patient subsequently died.
The patient's records did not reveal any previous history of significant upper or lower respiratory symptoms, and his previous chest X-rays, including the one that was done just before his transplant surgery, were unremarkable. A renal allograft biopsy was done on the recipient of the donor-matched organ (the second donor kidney) and showed no evidence of fungal infection, which made it unlikely to be a donor-transmitted infection.


A 66-year-old man was transferred to our hospital for the treatment of two asymptomatic saccular aneurysms caused by a penetrating atherosclerotic ulcer in the descending thoracic aorta and the infrarenal abdominal aorta. All of the laboratory data were within normal range except serum creatinine. The level of serum creatinine was slightly high at 1.5 mg/dL. A computerized tomography (CT) scan demonstrated that a saccular aneurysm measuring 60 mm in the longest diameter in the descending thoracic aorta was at the level of the 9th thoracic vertebra, and the other saccular aneurysm measuring 55 mm in the longest diameter in the infrarenal abdominal aorta was at the level of the 3rd lumbar vertebra (). We decided to perform staged endovascular repair on this patient in order to prevent contrast-induced nephropathy by reducing the total amount of contrast material administered, and to prevent paraplegia that could occur if the long segment of the aorta were covered with a stent graft in one session. In addition, we tried to avoid the risk of rupture while waiting for the second endovascular procedure by decreasing the interval time between the staged procedures.
During the first stage, a 34×100 mm stent-graft, a self-expandable custom-made type (S&G Biotech Inc., Seongnam, Korea), was deployed along the thoracic saccular aneurysm and spinal fluid drainage was performed to prevent paraplegia. Additional 36×80 mm stent-graft was used because of a wind-sock effect (). During this procedure, 200 mL of nonionic and iso-osmolar contrast medium iodixanol (Visipaque; Nycomed Imaging AS, Oslo, Norway) was used. In the second stage, one month later, Zenith stent-grafts (Cook Inc., Minneapolis, MN, USA) were placed from the saccular abdominal aneurysm to both common iliac arteries, thereby excluding the aneurysm from circulation. During this second stage, 240 mL of iodixanol (Visipaque, Nycomed Imaging AS) was used. Perioperative cerebrospinal fluid drainage was performed to prevent spinal cord ischemia (SCI). The patient presented no perioperative deterioration of renal function or paraplegia. A ten-month follow-up CT angiogram showed both stent-grafts were patent with no evidence of thrombus, endoleak, aneurysm growth, or complications derived from the procedure (). Two-stage endovascular repair may therefore be used to successfully treat separate saccular thoracic and abdominal aortic aneurysms without any deterioration of renal function or paraplegia.


A 31-year-old male, airline pilot, with no clinical history or cardiovascular risk factors, was admitted to the emergency room with 12 h history of retrosternal chest pain radiating to the jaw and upper extremities, during a trans-Atlantic flight, which did not improve with analgesia. Because of persistent pain, he came to our hospital. Upon arrival to the emergency room, his vital signs were stable and he had a normal physical examination but complained of precordial pain. The cardiac enzymes were elevated and the EKG detected S-T changes consistent with MI (Fig. ). A multidetector CT coronary angiography was performed showing left main trunk dissection with 50% stenosis (Fig. ).
The patient underwent emergent coronary revascularization with saphenous vein grafts to the first obtuse marginal and the left anterior descending artery (LAD). His postoperative course was uneventful and the patient was discharged home 7 days later. Control CT coronary angiography was performed 2 months later revealing patent aorto-coronary grafts (Fig. ).


A 74-year-old male patient suffered from progressive dyspnea, hoarseness, and stridor over a period of a few months prior to preoperative assessment. He had a total thyroidectomy and was treated with 3,700 MBq (100 mCi) of radioactive iodine following thyroidectomy due to thyroid papillary carcinoma. Computed tomography (CT) scan and fiberoptic bronchoscopy were performed with suspicion of recurrent papillary carcinoma in cricoid cartilage and trachea. There was a mass in the left side of the subglotic area with intact mucosa []. After exploration of the neck via a collar incision, a mass on the left side proximal to the trachea and cricoid cartilage with involvement of left recurrent nerve was discovered. Proximal trachea, in addition to the left half of cricoid cartilage, was resected.
In order to reconstruct the airway, the trachea was cut in the shape of the letter L. So, the left and longer side of the trachea was sutured to the thyroid cartilage and the short right side to right half of the cricoid cartilage. Fiberoptic bronchoscopy was used for airway assessment and the patient was put on orotracheal intubation for 48 h. After extubation 48 h later, good phonation was observed in the patient without any respiratory problems. The patient was discharged from the hospital without any complications. In gross pathologic examination, the specimen consisted of a fragment of trachea measuring 4 × 3 × 2 cm that in cut section showed a polypoid mass in the inner wall with chondroid consistency and gray color. Microscopically, the sections revealed neoplastic proliferation of chondrocytes in a pale, basophilic, hyaline chondroid matrix. The chondrocytes showed increased nuclear-to-cytoplasmic ratios, irregular nuclear membranes, and nucleoli. Mitotic activity was scant, but cellularity was markedly increased and several chondrocytes were seen on a single lacuna, indicating low-grade chondrosarcoma [].


Case 4 was a 20-year-old female patient, a diagnosed case of TOF who presented with a frontal lobe abscess with a 12 mm midline shift and features of septic shock. She was posted for emergency craniotomy and drainage of the abscess. There was a history of occasional episodes of cyanotic spells since the last 1 year. However, she had not undergone any corrective or palliative surgery and was not on any medication. Preoperative evaluation revealed SpO286% on room air, arterial blood gas (ABG) analysis showed PaO247.8 mmHg, pH 7.465, PaCO250.3 mmHg, and HCO330 mmol/l. She was being managed with vasopressors (noradrenaline: 0.05-0.07 g/kg/min) to maintain the mean arterial pressure (MAP) above 65 mmHg. After preoxygenation, the patient was induced with dexmedetomidine 1 g/kg and ketamine 2 mg/kg over 10 min. Anesthesia was maintained on controlled ventilation with vecuronium and an infusion of dexmedetomidine (0.7-1.0 g/kg/h) and ketamine (2 mg/kg/h). Paracetamol 1 g IV was added for analgesia though no inhalational anesthetic was used for maintenance. Cardiac output and systemic vascular resistance (SVR) were monitored by FlotracTM. SVR was maintained <1,200 dyne.s/cm5 by an infusion of noradrenaline (0.05-0.1 g/kg/min). There was a large parietal abscess and about 100 ml of pus was drained. In view of the raised intracranial pressure (ICP) and septic shock, she was electively ventilated in the ICU.


Case 5 was a 2-year-old boy who was diagnosed as TOF with a large subaortic VSD, bidirectional shunt, severe pulmonic stenosis, and overriding of aorta with a small sized pulmonary artery on echocardiography.[] The child had developed right hemiparesis at 1 year of age and his heart disease was detected during the work up. He recovered well over the next few months, but could not undergo any palliative surgery for TOF. He developed a malignant right parietal lobe infarct with features of raised ICP at 2 years of age. A decompressive right hemicraniectomy was done under general anesthesia. The child developed cerebrospinal fluid (CSF) leak from the surgical site and an external lumbar drain was placed 3 days later for controlled CSF drainage. During both the above procedures, dexmedetomidine (1 g/kg and 1-1.5 g/kg/h) and ketamine (2 mg/kg and 2-2.5 mg/kg/h), respectively were used for induction and maintenance of anesthesia. The former surgery was done with controlled ventilation using muscle relaxation and the latter procedure was carried out with the two drugs along with oxygen via face mask with spontaneous respiration. The child maintained hemodynamic stability perioperatively and recovered uneventfully.


A 42-year-old female patient was referred to the dental department complaining of difficulty in swallowing. She reported pain with every swallow (odynophagia), causing constant discomfort, but no other obvious symptoms or systemic problems. She had been a practicing dentist for the last 12 years, and reported mild pain on swallowing which had worsened within 2-3 days of onset. She was prescribed medication (azithromycin 500, cephalexin 500, and ibuprofen) for throat infection without any relief. Subsequently, she developed intermittent but acute throbbing pain and tenderness in her maxillary molars on the right side, and was referred to the dental department.
On examination, pain on percussion was evident with regard to teeth #16 and #17. Intraoral radiography showed proximal caries in tooth #17, while tooth #16 had evidently been satisfactorily root canal treated. As the pain was not relieved by medication (cephalexin 500 and serratiopeptidase-diclofenac potassium), endodontic treatment was administered to tooth #17. Four days after the treatment, the patient resumed with persistent dysphagia, without tooth pain. On examination, previously root canal treated tooth #16 was tender, and was therefore considered for re-endodontic treatment. A complaint of severe piercing pain on instrumentation to the mesio-buccal canal of tooth #16 perplexed endodontists, ultimately they decided to go for mesial root resection expecting that it would resolve the problem, but the dysphagia persisted [].
A fortnight later the patient returned with worsened dysphagia without dental pain. At the eye/ear-nose-throat (ENT) department, the otorhinolaryngologist diagnosed the case as tonsillitis, and a week later, as sinusitis. The treatment plan was changed with each different diagnosis, without any relief. The patient was then advised to undergo endoscopy, which ultimately proved to be normal. Ultrasonography (USG) of the neck and thyroid function tests also turned normal.
The patient was referred to the orthopedic department where they revealed a “stiff neck,” which proved to be the main cause of dysphagia. On palpation and pressure at C3 and C4 levels, the patient experienced pain with restricted spinal movements. Cervical radiographs revealed osteophytic projections impinging upon the posterior pharyngeal wall at the levels of C3, C4, and C5, and cervical hypolordosis []. The osteophyte at the pharyngo-esophageal junction was positioned in such a way that it interfered with the swallowing movement. The patient was ultimately diagnosed with osteophytosis involving C3, C4, and C5.
The initial treatment planned was nonsurgical. The patient was referred to the chiropractor/physiotherapist for management of stiff neck. In conjunction with nonsteroidal anti-inflammatory drugs, the nutritional supplements were prescribed for a month.
The treatment also included muscle relaxant massages, spinal adjustment with neck exercise, and traction sessions.


A 62-year-old male patient visited our hospital with dyspnea and mild dysphagia that began approximately 4 months prior to the visit as the main symptoms. Gentle exercise, such as steps, caused difficulty of inspiration 1 month ago, but it had become worse around the time of his visit, he appeared at rest and accompanied by dysphonia. Dysphagia emerged as foreign body sensation and intermittent aspiration during a solid meal, but he did not have history of weight loss or aspiration pneumonia. Various physicians, from a pulmonologist to an otorhinolaryngologist, failed to make an accurate diagnosis and the symptoms of the patient continued to progressively deteriorate.
The patient did not have any particular past internal or surgical medical history other than being a smoker for the last 20 years. The patient had clear consciousness without cognitive dysfunction at the time of his visit to our hospital. In physical examination, stridor was auscultated during inhalation. There was no abnormality of the tongue and no atrophy of the body muscles. The sensory tests displayed normal findings, and normal muscular strength was observed in all muscles of the upper and lower limbs on both sides in the manual muscular strength tests along with normal findings in the deep tendon reflex tests. Fibrillation was not observed in neurological examinations, and masticatory function and gag reflex were normal without observation of particular findings, such as dysarthria in linguistic assessments. Brain magnetic resonance imaging (MRI) for intracranial pathology did not show any abnormalities. There were no abnormal findings in the electrodiagnostic studies that were screened for neuromuscular diseases such as progressive bulbar paralysis. In addition, all the enzymes related to muscular diseases, such as creatine kinase, lactate dehydrogenase, and so on, were in the normal range of the laboratory test. Pulmonary function test was performed to find the cause of dyspnea, but normal pulmonary function was observed without abnormal findings, such as obstructive or restrictive pulmonary patterns. A VFSS showed that osteophytes were severely protruding at the anterior portion of the 5th to 7th cervical spine and translocated the lower pharynx and the upper esophagus anterosuperiorly. In the oral phase, there was no abnormality except for premature bolus loss. In the pharyngeal phase, although laryngeal elevation and epiglottic closures were normal and aspiration was not observed, a lowering of food took a circuitous route around the osteophytes and the pharyngeal transit time of the solid food was delayed by 1.4 seconds. X-ray and computerized tomography (CT) scan of the cervical vertebrae were performed to verify these anterior cervical osteophytes and revealed that the formation of large osteophytes was caused by overgrowth of cortical bones of the 5th to 7th cervical vertebrae. Osteophyte in the 5th cervical spine was the most prominent and protruded 8 mm outside of the vertebral body. C-spine MRI showed anterior herniation of the intervertebral disc accompanied with a ruptured anterior longitudinal ligament, with fluid collection in the retropharyngeal space. A T2-weighted image showed high signal intensity with irregular enhancement caused by retropharyngeal irritation and inflammatory changes due to the large cervical osteophytes (). Anatomical deformities or mass-like lesions on the oropharynx and laryngopharynx were not observed in the larynx endoscopic examination. However, the paradoxical vocal cord motion, adducted vocal cords during the time of inhalation, was observed.
Because we did not find any possible etiology of these symptoms other than the anterior cervical osteophytes and the patient displayed a tendency of gradually aggravating dyspnea with severe anxiety and sleep disturbances, we decided to perform surgical treatments. Surgery was conducted through the anterior approach method. Removal of the anterior osteophytes and 5th to 6th cervical herniated disc were performed and interbody fixation with a bone graft was executed.
Dysphagia disappeared immediately due to relief of mechanical pressure on the larynx, but dyspnea and paradoxical vocal cord motion still remained. Accordingly, for correcting the abnormal vocal cords motion, the patient performed the breathing exercises, such as relaxation technique and diaphragmatic breathing method. Finally, the respiratory difficulties and dysphonia were gradually improved after 3 months of continuous self-treatments.


A 44-year-old lady presented with hemoptysis, cough and shortness of breath. She had a 3-month history of progressive darkening of skin along with weakness, anorexia, nausea and weight loss of 3 kg over 1 month. She had a past history of a first trimester abortion. History of deep vein thrombosis, pulmonary embolism, connective tissue disorder was ruled out. Her body mass index was 18, goiter was absent, she had tachycardia (pulse rate 110/min) and blood pressure was 108/76 mm Hg with postural drop of 26. Pallor and skin and mucosal pigmentation were present.
On investigations: Hb 11.6 g%, total cholesterol 10,100 (N 72%, L 23%, E 5%), erythrocyte sedimentation rate:62 (1st h), platelets 86,000, urine R/E normal, Cr 1.1 mg/dl, liver function tests normal, echo normal.
Chest X-ray showed right middle zone opacity. Computed tomography (CT) scan of lungs revealed subpleural consolidation in mid and lower zones of the right lung with ground glass opacity and areas of hemorrhage and filling defect in right descending pulmonary artery. CT pulmonary angiography confirmed right descending pulmonary artery embolism. Duplex scan revealed right superficial femoral vein thrombosis. Renal and adrenal veins were normal in Duplex scan and magnetic resonance angiography (MRA).
Prothrombin time was 13.8 (normal [N] 11.2-14.2), activated partial thromboplastin time 142 s (N 31-42), fibrin/fibrinogen degradation products was raised, lupus anticoagulant and anticardiolipin antibody were markedly positive. Anti-nuclear antibodies, double-stranded deoxyribonucleic acid, anti-neutrophil cytoplasmic antibody and rheumatoid factor were negative. Human immunodeficiency virus and hepatitis serology was negative. Sputum and blood for Bactec culture for acid-fast bacteria and fungus was negative.
Baseline 8 A.M. cortisol was 2.3 mcg/dL with an adrenocorticotropic hormone (ACTH) of 306 pg/ml. 250 mcg ACTH stimulation test showed a 1-h cortisol level of 2.5 mcg/dL. Thyroid function test was normal, but thyroid peroxidase antibody was 590 mU/ml (normal <35). Follicle-stimulating hormone was 7 mIU/ml CT of adrenals was normal.
Based on clinical features and investigations our diagnosis was PAI probably of autoimmune nature in a case of pulmonary thromboembolism due to APS along with autoimmune thyroid disease. Our limitations: Antiadrenal antibody not estimated.
She was started on hydrocortisone 100 mg three times daily and heparin. She was subsequently discharged on replacement dose of hydrocortisone and warfarin. After 6 months follow-up, the patient did not have further thrombotic episodes and requires replacement hydrocortisone.


A 14-year-old girl was admitted with generalized tonic-clonic convulsions for 2 min along with tongue biting. Her family reported previous recurrent attacks of muscle cramps and carpo-pedal spasms of four months' duration prior to admission but they had never previously sought medical help.
The patient had a good appetite and no diarrhea, with no prior history of cardiovascular disease or symptoms. She was not on any regular medications. She lived mainly indoors, avoided sunlight, and wore a veil outdoors mainly due to traditional beliefs. Her family noticed an abnormal gait eight months prior to presenting at our clinic and she usually asked for help to stand from a squatting position.


A 39-year-old male presented with cough and blurred vision for 1 month. Six years ago he was diagnosed with HIV infection in another hospital and he was followed regularly at public health care facility. He had no previous history of pulmonary tuberculosis or other pulmonary diseases. He was a nonsmoker and nondrinker. Forty days ago before admission, he visited the other hospital due to blurred vision and cough for 1 month. CD4 cell counts were 3/mm3 and highly active antiretroviral therapy with combivir and kaletra was started with the concurrent treatment for cytomegalovirus retinitis. He was transferred to our hospital for further evaluation of chronic cough with an abnormal chest X-ray.
The patient had a chronically ill appearing and vital signs were stable. There were no specific findings in head and neck area. Breathing sounds were clear and there were no signs of abdominal tenderness. A complete blood count showed white blood cell count of 9,390/mm3 (neutrophil 61.0%, lymphocyte 21.0%, eosinophil 7.0%), hemoglobin 11.3 g/dL, and platelet 322×109/L with C-reactive protein of 3.62 mg/dL (normal, <0.5 mg/dL). A blood chemistry analysis was unremarkable. CD4 count was 247/mm3, and HIV viral load was 2,499 copies/mL. Tuberculin skin test revealed a negative result with a positive interferon-gamma release assay result. Initial chest X-ray showed bilateral hilar prominence, raising the possibility of bilateral hilar lymphadenopathy (). Chest computed tomography revealed multifocal nodular consolidations with some surrounding ground glass opacities in both lungs. There were several enlarged lymph nodes with some internal low-attenuated lesions in the paratracheal, subcarinal, and both hilar regions (). Bronchoscopy showed an elevated nodular lesion with central whitish necrosis at apical segment of right upper lobe and luminal narrowing at apical part of apicoposterior segment of left upper lobe (). Bronchial mucosal biopsy was performed at the both above segments. As bronchial washing fluids smear was positive acid fast bacilli, we started with standard antituberculous medication empirically. However, NTM was detected by polymerase chain reaction from the bronchial specimens and histologic findings at bronchial mucosa revealed chronic granulomatous inflammations with positive Ziehl-Neelsen stain (). One month later, M. kansasii was isolated in bronchial washing fluids and bronchial mucosa specimens. Finally, we diagnosed M. kansasii pulmonary disease with bronchial involvement and he was treated with isoniazid at 300 mg, rifabutin at 150 mg, and ethambutol at 1,200 mg. After the medication, his cough improved gradually and a chest X-ray after 18 months of treatment showed complete regression of the bilateral hilar lymphadenopathy ().


A 54-yr-old female was referred for neurologic consultation due to progressive dysarthria, dysphagia and left facial palsy for past 5 month in January 2011. She had no underlying medical illness such as diabetes mellitus, and had underwent first functional endoscopic sinus surgery (FESS) for bilateral maxillary sinusitis 4 yr ago. She had remained tolerable after the surgery for about 3. 5 yr then the symptoms of headache, hearing difficulty, dysarthria and dysphagia slowly emerged and progressed for about 6 months. Under the diagnosis of recurrent sinusitis involving frontal, ethmoid, sphenoid and maxillary sinuses, second FESS and broad-spectrum antibiotics were treated. Although these surgical and medical treatments were adequately performed, her symptoms worsened as aggravated hearing problem, severe bulbar symptoms and newly developed left facial palsy. The first brain MRI with using contrast enhancement was performed, only to reveal still existing bilateral otomastoiditis and extensive sinusitis (). Cerebrospinal fluid (CSF) had no cell counts and normal protein level. The empiric steroid therapy (methysol 2 mg/kg/day) without definite diagnosis has somewhat stabilized these series of aggravating symptoms. At then, she was referred to our clinic for more detailed evaluation.
In January 2011 when she was admitted in our clinic, all the vital signs including body temperature were stable. Accompanied by severe bulbar symptoms of dysarthria and dysphagia, she complained of constitutional symptoms such as general weakness, poor oral intake and weight loss of up to 12 kilograms during past 6 months. Neurologic examination revealed various lower cranial nerve palsies of both sides, which were overall more prominent on left side than right side. Demonstrated clinical manifestations and positive neurologic findings are; facial diplegia, progressive hearing difficulty resulting in near-deafness, bilateral tinnitus and hyperacusis, right deviation of uvula, bilateral decreased gag reflexes, flaccid dysarthria with breathy voice and hypernasality, dysphagia especially in liquid food, left vocal cord palsy, left sternocleidomastoid muscle weakness, impaired tongue protrusion and giggling, and fasciculation with slight atrophy on the left side tongue. In contrast to these various lower cranial nerve involvements, there was no afferent pupillary defect, and no limitation in extraocular eye movements. Otolaryngology report was given as extensive sinusitis involving bilateral frontal, sphenoid, and maxillary sinuses with nasal cartilage erosion and bilateral chronic otitis media.
Laboratory studies revealed increased C-reactive protein (CRP) of 29.90 mg/dL (normal range 0-0.3 mg/dL), increased erythrocyte sedimentation rate (ESR) of 67 mm/hr, mild anemia (hemoglobin 10.8 g/dL), but no eosinophila and mild leukocytosis (12,800/µL). Renal function (BUN 15 mg/dL, creatinine 0.6 mg/dL, glomerular filtration rate 111 mL/min) and microscopic urine analysis were normal. Serology test for anti-Ro/SSA, anti-La/SSB, antinuclear, anticardioplin antibody, and ANCA were negative. Neither immunofluorescence method nor direct enzyme-linked immunosorbent assay method for proteinase 3 and myeloperoxidase antigens could detect ANCA. FANA screening and VDRL were also negative, and angiotensin converting enzyme was within normal as 28 U/L (normal range 18-55 U/L). Rheumatoid factor was minimally elevated up to 20.4 IU/mL (normal range 10-18 IU/mL). Serum folate, vitamin B1 and vitamin B12 were measured as normal level. Tumor markers including AFP, CEA, CA19-9, and CA125 were normal.
Chest CT revealed no hilar enlargement or parenchymal lung lesion. Neck CT showed high density lesions in the nasopharynx with enhancement, penetrating the skull base and extensive sinusitis expanding into orbit and retromaxillary fat-pad (). Whole body positron emission tomography (PET) showed hypermetabolic lesion in bilateral tonsil area with multiple lymph nodes in level II/III of neck and also in bilateral frontal, ethmoid, sphenoid, and maxillary sinuses ().
In the facial nerve conduction study, the compound muscle action potential (CMAP) amplitude of left facial nerve was decreased to 60% that of right side. Blink reflex test showed delayed ipsilateral R1 and R2, but normal contralateral R2 responses on both supraorbital nerve stimulations. These electrophysilogical findings were indicative of bilateral facial neuropathies, more severe in the left side.
Endoscopic biopsy was performed twice due to failure to obtain pathologic confirmation at first biopsy. The first biopsy specimen was obtained from nasopharyeal wall layer through a punch biopsy under local anesthesia, but the second endoscopic biopsy was performed far deeper layer of retropharyngeal area under general anesthesia. Tissues obtained from the second biopsy showed granulomatous inflammation and vasculitis involving small sized blood vessels with ischemic necrosis and destruction of cartilage (). Period acid-Schiff, Gomori methenamine silver and acid-fast bacilli stains showed no histological evidence of fungal organism or mycobacterium. Immunostains for CD3 and CD56 had no abnormal infiltration of natural killer T cells. Ebstein-Barr virus-encoded RNA in situ hybridization resulted in negative findings.
All the clinical manifestations, laboratory and imaging studies indicated ANCA negative WG involving otolaryngeal space, upper respiratory tract and multiple lower cranial nerves. She was transferred to rhematology department and began therapy with steroid (methysol 1 mg/kg/day) and cyclophosphamide (2 mg/kg/day). Within the ten days of these treatments, the patient stated that her sufferings of headache and constitutional symptoms were prominently diminished. The hearing difficulty, which compelled her to wear a hearing aid, improved dramatically leaving no more need of the device. After three months of combination therapy of tapering steroid (prednisolone 0.6 mg/kg/day) and steady cyclophosphamide (2 mg/kg/day), the patient restored to almost her normal bulbar function and removed nasogastric tube for oral intake. Further repeated ANCA study was negative. Brain MRI was re-evaluated with a time interval of five months from the first MRI study, and showed persistent sinusitis of both frontal, ethmoid, sphenoid, and maxillary sinuses, in despite of marked improvement of clinical symptoms. Moreover, focal thickening with enhancement of the meninges around the area of tentorium cerebelli, inferior aspect of frontotemporal lobe adjacent to sinuses and nasopharynx were newly observed (). Clinical significance of these imaging findings was somewhat debatable because previous neurologic defects such as dysarthria, dysphagia, and hearing impairment had improved remarkably. However, re-evaluated CRP was still high as 5.75 mg/dL, which implied for further immunotherapy. Ultimately, potent immunotherapy consisting of high dose steroid (methysol 2 mg/kg/day) and cyclophosphamide (3 mg/kg/day) was performed. Three weeks later, the follow up CRP fall to 0.39 mg/dl and ESR normalized as 3 mm/hr. Taking only cyclophosphamide (0.25 mg/kg/day) after tapering steroid, she is still in a remission state for more than a year.


A 68-year-old woman presented to us with sudden jerky right sided movements of 10 days duration that were also causing her to fall. These movements had started acutely and predominantly involved the right sided arm and leg. They occurred multiple times a day and initially were spontaneous and brief lasting a few seconds. They interfered with walking and caused frequent falls. Observation of a few of these episodes revealed facial grimacing and brief right upper limb dystonic posturing lasting 1-2 s. While standing or walking, the movements occasionally involved the right leg causing buckling at the knee and falls. There was no significant past medical history and routine investigations including prolonged electroencephalography (EEG) monitoring and magnetic resonance imaging (MRI) brain were normal. She was diagnosed with late onset paroxysmal non-kinesogenic dyskinesia (PNKD), likely symptomatic and was put on clonazepam, phenytoin without significant improvement. She was discharged after 5 days. After 1 month, she was readmitted from the emergency room with drowsiness and hyponatremia (serum sodium 116 meq/l). After correction of Na and improvement in sensorium, she was noticed to have frequent bilateral alternating attacks up to 30-50/day involving the face, arm and legs. Carbamazepine, lamotrigine and sodium valproate were added to her regimen. She had some symptomatic relief, but the episodes persisted. Her falls abated, but she continued to have mild faciobrachial dystonic (FBD) attacks. Serum was stored for future analysis. She declined to be video graphed and was requested discharge from our facility. At telephonic follow-up 6 months later, she was asymptomatic and off on all medications.