A 75-year-old female presented with dyspnea. She was an ex-smoker (60 pack-years). Her Eastern Cooperative Oncology Group performance status was 1. Chest computed tomography (CT) revealed a pulmonary mass in the right upper lobe with right-sided pleural effusion. She was diagnosed with advanced lung adenocarcinoma (cT2aN3M1a, stage IV) by transbronchial biopsy and imaging findings. Because peptide nucleic acid-locked nucleic acid (PNA-LNA) polymerase chain reaction (PCR) clamp analysis showed that the primary lung tumor had an EGFR mutation (15-bp in-frame deletion in exon 19, del E746-A750), she was treated with gefitinib as first-line therapy for 14 months. However, her disease progressed, and thereafter she received six cycles of carboplatin and pemetrexed chemotherapy followed by pemetrexed for maintenance. During the maintenance therapy, she developed nausea and anorexia and had a performance status of 3. Imaging studies showed that her right-sided malignant pleural effusion had increased in severity and that multiple brain metastases and meningitis carcinomatosa had developed. T790M EGFR-tyrosine kinase inhibitor-resistant mutation in exon 20, in addition to the EGFR exon 19 deletion mutation, was identified from the pleural effusion. Adenocarcinoma cells were also observed in her spinal fluid. PNA-LNA PCR clamp analysis of the spinal fluid revealed the same EGFR deletion mutation; however, the T790M mutation could not be detected. Thus, we administered erlotinib to control the central nervous system metastasis. After 4 weeks, the brain metastases responded completely and her digestive symptoms related to the central nervous system metastasis disappeared.
After receiving erlotinib for 3 months, the patient visited the emergency department with jaundice, epigastric pain, and a low-grade fever. Hematological investigation showed a hemoglobin level of 9.4 g/dL; her white blood cell count and differential leukocyte and platelet counts were within normal limits. Her serum C-reactive protein level was 1.76 mg/dL. Her other laboratory findings were as follows: total bilirubin, 5.8 mg/dL; direct bilirubin, 3.8 mg/dL; aspartate aminotransferase, 165 U/L; alanine aminotransferase, 111 U/L; γ-glutamyl transpeptidase, 148 U/L; lactate dehydrogenase, 223 U/L; amylase, 44 U/L; blood urea nitrogen, 23 mg/dL; creatinine, 0.58 mg/dL; prothrombin time, 11.3 seconds; activated partial thromboplastin time, 26.1 seconds; and D-dimers, 2.7 μg/mL. A urinalysis revealed bilirubinuria (1+)
Chest X-ray and CT revealed bilateral massive pleural effusion. Abdominal CT revealed dilatation of the intra-and extrahepatic bile duct, including the common bile duct, which showed abrupt termination on the distal side (). No metastases to the porta hepatis, including any originating from the lymph nodes, liver, or pancreas, were observed. Computer-reconstructed coronal section images also confirmed the obstruction of the distal common bile duct, and an intraductal tumor in the dilated common bile duct with slightly low attenuation was present on CT (). Endoscopic retrograde cholangiopancreatography and endoscopic ultrasonography also showed obstruction of the common bile duct due to the intraductal tumor (). The appearance of the intraductal tumor was observed by peroral cholangioscopy. The surface of the common bile duct showed irregular and inadequate angiogenesis. Detailed observation was difficult due to the presence of stenosis. Based on these findings, we suspected primary cholangiocarcinoma. An endoscopic retrograde bile duct drainage tube was placed, and the patient’s jaundice promptly improved. Adenocarcinoma cells in a specimen obtained by transbronchial biopsy at the time of diagnosis are shown in . The biopsy specimen obtained using digestive endoscopy revealed poorly differentiated adenocarcinoma that was positive for thyroid transcription factor-1 by immunohistochemistry (). PNA-LNA PCR clamp analysis revealed that the specimen had an EGFR exon 19 deletion mutation and T790M mutation in exon 20. Two weeks later, the patient received a replacement endoscopic retrograde bile duct drainage tube with a self-expanding metal stent.


A 39-year-old woman was admitted for a sudden-onset headache and generalized tonic-clonic seizure. On admission, no focal neurologic deficits were observed, only a drowsy state. The initial brain computed tomography (CT) showed a subarachnoid hemorrhage (SAH) in the basal cistern, suprasellar cistern, and right sylvian fissure. Subsequent CT angiography showed a dorsal wall aneurysm on the right ICA and two dissecting aneurysms on the bilateral distal vertebral arteries. Transfemoral cerebral angiography (TFCA) performed the next day confirmed a broad-based, saccular aneurysm measuring 2.5 mm in size on the dorsal wall of the right ICA and dissecting aneurysms on the bilateral vertebral arteries (). On day 2 after rupture, we performed a right pterional craniotomy for direct clipping. The aneurysm was completely dissected; however, the clip was not applied because of the very thin wall and neck (). Finally, she was treated with clipping after wrapping with the temporal fascia. She made a full postoperative recovery. Two weeks later, she underwent stent-assisted coil embolization for the left VA aneurysm. Follow-up TFCA performed one month after onset showed that the right ICA aneurysm had increased in size (). We performed coil embolization without a stent (), and she was discharged without any neurological focal signs.


A 58-year-old woman was transferred to our emergency department with sudden onset headache and vomiting. On initial neurological examination, her mental status was drowsy, but no focal neurological deficits were observed. The initial brain CT showed diffuse SAH and intracerebral hemorrhage (ICH) in the right temporal area. Subsequent CT angiography showed subtle bulging at the right ICA. TFCA performed the following day showed the same finding as the previous CT angiography (). Follow-up angiography for both carotid ICAs, performed two weeks after onset, showed a definite aneurysm measuring 3.9 mm in size at the location of the previous bulging lesion of the right ICA (). Craniotomy was performed for direct clipping. During the operation, the aneurysm was completely dissected, and its neck was relatively firm. We attempted to perform careful clipping of the aneurysmal neck directly, but a slight tear occurred; therefore, we performed the clipping on the wrapping with Lyodura® (B.Braun, Melsungen, Germany) after the bleeding had ceased (). She made a full recovery postoperatively. One month later, an increase in aneurysm size was observed on the follow-up angiography (). She underwent simultaneous coil embolization without a stent (), and no evidence of aneurysmal regrowth was observed by TFCA during the 2-year follow-up period ().


A 48 years old female visited with repeated vertigo attack which had emerged when she rotated her head to right side. She also complained of tinnitus on left side simultaneously. She had otitis media at left ear in the past but treated well and vestibular test showed normal result. On physical examination there was no neurologic deficit in neutral position, but left side nystagmus was found when her head was rotated. Computed tomographic angiography (CTA) showed that left vertebral artery was dominant and right vertebral artery was hypoplastic (). Angiography was performed in supine position. Arterial filling and patency was intact in neutral position. But in right side head rotation position, the patient experienced vertigo attack and angiography showed occlusion of left vertebral artery (). Surgical decompression was performed. After induction of general anesthesia, the patient was positioned in prone position and her head was mildly flexed. Midline vertical was incised and C1 posterior arch & C2 spinous process was exposed. A part of left posterior arch and transverse foramen of C1 were removed by kerrison rounguer and high speed drill (). Posterior side of vertebral artery was exposed and its pulsatile movement was observed after decompression. Gelfoam was applied on the decompressed site and wound was closed layer by layer. Immediately after surgery, the patient's symptoms were decreased. Vertebral artery angiography was performed 1 month later and occlusion of left vertebral artery during right side head rotation was not observed anymore (). The patient did not have vertigo attack again.


Our patient was a 76-year-old man with a coronary artery bypass graft due to unstable angina. During the postoperative period, the patient developed the adult respiratory distress syndrome due to pneumonia. A blood gas analysis showed a PaCO2 concentration of 45 mmHg, PaO2 concentration of 63 mmHg, and a pH of 7.40 during intermittent mandatory ventilation at a rate of 12 breaths per minute, a tidal volume of 500 mL, a positive end expiratory pressure of 10-cm H2O, and FIO2 of 100%. After two weeks of mechanical ventilation, tracheostomy was required. The neck was prepared with povidone iodine, surgical drapes were placed, and tracheostomy was started. After neck incision, subcutaneous tissue and pretracheal fascia dissections were performed using a monopolar electrocautery device to secure hemostasis. After the identification of tracheal rings, the second and the third rings were incised vertically along the midline by using a scalpel, and to control minor bleeding in the paratracheal area, an electrocautery device was used. However, during its use, a loud pop was heard and a flame erupted from the surgical site. Initially, we attempted to control the fire by applying manual pressure with a surgical towel but were unsuccessful because of the pure-oxygen spillage through the cuff and the use of positive end-expiratory pressure. We quickly disconnected the ventilator, removed the endotracheal tube, and extinguished the flame in the surgical field by using a saline spray. A tracheostomy tube was then inserted uneventfully. The skin at the peri-tracheostomy site was deeply burnt (), but flexible bronchoscopy revealed no definite thermal injury of the trachea. Unfortunately, the follow-up chest X-ray showed progressive pulmonary infiltration, and the patient subsequently succumbed to a multiorgan failure after unsuccessful recovery efforts.


A 76-year-old male underwent a left upper lobectomy with en bloc wedge resection of the superior segment of the left lower lobe using video-assisted thoracoscopic surgery (VATS) for non-small-cell lung cancer of the left upper lobe. Systematic dissection of the pulmonary and mediastinal lymph nodes was performed. The operation and immediate postoperative course were uneventful. The patient presented poor bronchial toileting and required supplementary oxygen. Chest radiography on the second postoperative day revealed atelectasis at the remaining left lower lobe, which was not resolved despite vigorous chest physiotherapy (). The patient had a mild fever and leukocytosis. In addition, a high-sensitivity C-reactive protein (CRP) test revealed an elevated CRP level. Fiberoptic bronchoscopy on the fourth postoperative day revealed narrowing of the left lower bronchus with purulent secretion (). Computed tomography showed an obstruction of the left lower lobar bronchus associated with acute angulation at the distal main bronchus. The left lower lobar artery showed right-angle angulation, and the left inferior pulmonary vein was stretched upward (). Perfusion to the pulmonary parenchyma was intact. The patient underwent exploratory VATS under the impression of bronchial kinking. The intraoperative findings showed an inferiorly kinked left lower lobar bronchus accompanied by an excessively upwardly displaced left lower lobe (). The left lower lobe was consolidated without congestion. After adhesiolysis, the kinked bronchus was straightened. There was no malacia at the kinked portion of the bronchus. In addition, there was no supportive structure around the main bronchus and lower lobar bronchus due to extensive lymph node dissection. Thus, bronchopexy was performed to the pericardium with two interrupted monofilament sutures to prevent recurrence of bronchial kinking (). The inferior pulmonary ligament was reattached to prevent excessive upward displacement. Postoperative follow-up bronchoscopy revealed an elliptical bronchial lumen without obstruction, and chest radiography showed no atelectasis of the left lung thereafter. However, pneumonia developed in the contralateral lung. The patient underwent a mechanical ventilation treatment for 1 month and was discharged on the 80th postoperative day with a tracheostomy. The tracheostomy tube was removed at an outpatient clinic 2 months after discharge. Chest radiography revealed no specific findings ().


A 65-year-old female who had diabetes mellitus visited a private clinic and presented with injection and visual disturbance in her right eye. Anterior uveitis was diagnosed, and she was referred to our hospital. Slit-lamp biomicroscopy revealed scleral injection, inflammatory cells in the anterior chamber as well as iris synechiae. The IOP in the right eye was 4 mm Hg and B-scan ultrasonography showed choroidal detachment. The patient did not have a history of herpetic eye disease. We diagnosed noninfectious anterior uveitis and began steroid treatment with a 0.1% dexamethasone ophthalmic solution every hour, 1% atropine eye drops every night, and oral prednisolone 25 mg/day. One month after treatment, the inflammation in the anterior chamber and choroidal detachment subsided while the IOP increased to 15 mm Hg. In addition, her blood sugar increased due to the adverse effects of oral prednisolone. Therefore, the systemic steroid was tapered and we continued topical steroids. The scleral injection, hypotony, and choroidal detachment reappeared after tapering the systemic steroids. We performed STI to reduce the anterior segment inflammation. She complained of redness, foreign body sensation, and ocular discharge 1 week after STI. Slit-lamp biomicroscopy showed conjunctival injection, discharge, blepharitis with vesicles of the lid margin, and geographic corneal and conjunctival epithelial defects (fig. , fig. ). The patient was suspected of having herpetic keratitis. The topical steroid was stopped and she was treated with topical acyclovir (3% eye ointment 5 times/day) and oral valacyclovir (3,000 mg/day). Real time polymerase chain reaction (PCR) of a corneal scraping was performed in order to detect a potential human herpes virus, including herpes simplex virus (HSV) types 1 and 2, varicella-zoster virus, Epstein–Barr virus, cytomegalovirus, and human herpes viruses 6, 7, and 8. The real-time PCR results were positive only for HSV DNA (3×109 copies/sample). Since the conjunctival injection and epithelial defect improved with the therapy, the valacyclovir was tapered within 1 month. The scleritis and the inflammation in the anterior chamber as well as her herpetic keratitis were controlled with topical acyclovir. However, the hypotony persisted and the cataract progressed markedly. Therefore, a lensectomy and vitrectomy with silicone oil tamponade was performed and the IOP increased to 10 mm Hg. Thereafter, the patient was administered topical acyclovir and steroids, without any uveitis recurrence.


A 51-year-old Caucasian female presented to her local Urologist with a history of recurrent urinary tract infections and new onset of left abdominal pain. Her past medical history is significant for intake of birth control pills for 15 years. There is no history of hematuria. She uses to smoke 2 packs of cigarettes every day for the past 30 years. Physical examination was unremarkable. Urine analysis showed trace of leucocytes but otherwise clear. A CT scan was performed and this revealed 11.5 cm left multiloculated cystic renal mass. Of note that the mass on the CT scan looked as if it is compressing or extending into the collecting system giving the impression of possible urothelial carcinoma. Given the symptomatic nature of the cystic disease and the risk that it could be malignant in nature, the patient was advised to proceed with open partial versus radical left nephrectomy ± ureterectomy with bladder cuff excision.


A 53-year-old Chinese female presented with a persistent productive cough with foul smelling phlegm and blood streaked sputum of several months.' This was not associated with significant weight loss or fever. She was in good general health, and physical examination showed no unremarkable results. The patient's temperature was normal, her respiratory rate was 16 breaths per minute, her blood pressure was 134/74 mm Hg, and her pulse was 64 beats per minute. Right upper lung crackles were noted. Her total white blood cell count was 4,700/mm3, with 2.9% eosinophilia, and the high-sensitivity C-reactive protein level was 0.03 mg/dL. A tuberculin skin test (TST) was 16 mm and QuantiFERON-TB (QFT-G; Cellestis Ltd., Carnegie, Australia) test was also positive.
The chest radiograph carried out on presentation showed small nodular opacity in the suprahilar region of right upper lobe (). Chest CT scans revealed focal parenchymal mass-like consolidation with cavitation and ground glass opacity in posterior segment of right upper lobe (), and pleural thickening in right lower lobe. Bronchoscopy revealed bronchial stenosis with hemorrhage that was associated with mucosal edema of the posterior segmental bronchus of the right upper lobe. Two samples of acid-fast bacilli (AFB) stain and one of polymerase chain reaction for Mycobacterium tuberculosis were negative. Cytological examination of the bronchial washing fluid showed no significant result. Although the patient had taken several courses of antibiotics, there were no clinical and radiological improvement observed. Though the microbiologic results were negative, the case showed radiological signs consistent with TB, and no response with antibiotics trial, and therefore smear-negative TB had been diagnosed based on algorithms. The patient was started on anti-TB therapy. After receiving 6 months of anti-TB therapy, her chest radiograph with CT scans proved improvement with remnant mass-like consolidation on posterior segment of right upper lobe (). Purulent phlegm and hemoptysis, however, lasted intermittently during and after treatment.
Follow-up chest radiograph obtained at 4-months after completion of anti-TB therapy, showed newly developed mass-like consolidation on right upper lobe (). Bronchoscopy with bronchoalveolar lavage (BAL) was performed and sent for examinations. Results of cytology, AFB stain, and polymerase chain reaction for Mycobacterium tuberculosis were negative, as were cultures for bacteria, fungi, and mycobacteria. We could not observe eggs in sputum, or BAL fluid. Bronchoscopic biopsy showed chronic inflammation with a giant cell. According to complete blood cell count finding of the patient follow-up, there was no signal of peripheral blood eosinophilia. Therefore, we recommended pathologic confirmation with thoracoscopic examination, however the patient refused it. She was re-treated with anti-TB medication, and made transient aggravation at 2 months, and subsequent improvement at 3 months after re-treatment (). Although we considered the possibility of the paradoxical response with anti-TB treatment, the patient suffered recurrent hemoptysis and her chest CT scans revealed newly developed cavitary nodules within right upper lobe at 5 months after re-treatment (). She underwent thoracoscopic pulmonary wedge resection because the lesion was not appropriate for conducting percutaneous needle aspiration or biopsy. Thoracoscopic pulmonary wedge resection was applied for the mass measuring approximately 2×2-cm size in the posterior segment of right upper lung, and the gross findings of transected specimen on operative field showed a movable parasitic worm measured about 1×0.5 cm in cavitary lesion to be surrounded with organized and yellowish pulmonary parenchymal tissue. The pathological examination reported no malignant findings in the lung, and granuloma, acute and chronic inflammatory cell infiltration with eosinophils caused by Paragonimus westermani was diagnosed (). Enzyme-linked immunosorbent assay (ELISA) antibody was positive for anibodies against P. westermani. A subsequent discussion with the patient revealed that she had eaten raw fresh water crabs several times before immigration to Korea from China several years ago. Anti-tuberculous medication was discontinued and praziquantel 75 mg/kg/day in three divided doses for 2 days were given. She was discharged with improvement of symptoms and signs. After that event, her symptoms and radiologic findings have maintained within normal range during 1-year follow-up period.


A 12-year-old girl presented with a history of transient ischemic attacks (TIAs) on the right side for 2 years. MRI and MRA showed a signal void structure in the right parietal lobe and steno-occlusive vascular lesions mainly involving the left ICA and the left posterior cerebral artery. Neither infarction nor hemorrhage was detected in both cerebral hemispheres. A neurological examination showed no deficits on admission. The patient underwent cerebral angiography and was diagnosed with MMD by bilateral steno-occlusive lesions and the typical occurrence of abnormal vascular networks (). Additionally, a small AVM was noted in the right parietal lobe supplied by the posterior parietal and angular arteries. Single photon emission computed tomography (SPECT) revealed global hypoperfusion in the left hemisphere and mild hypoperfusion in the right anterior temporal lobe. Encephalo-duro-arterio-synangiosis (EDAS) was performed on the left hemisphere using branches of the external carotid artery (ECA), followed by subsequent EDAS on the right hemisphere.
The incidental AVM was managed expectantly as obliteration of the main feeders was anticipated with the future progression of MMD. Follow-up angiography, performed 4.5 years after EDAS, showed progressive stenosis of the right ICA with decreased flow to the AVM (). The AVM appeared to regress on MRI taken 6.5 years after EDAS but subsequent MRI taken 3 years later showed the increased AVM. Subsequent angiography, performed 9.5 years after EDAS, demonstrated newly-developed AVM feeders from the EDAS site causing no significant change in the size of the AVM (). Gamma knife radiosurgery (GKS) was performed with a marginal dose of 28 Gy (50% of the maximal dose) considering an increasing steal effect by the persistent AVM. The patient is currently being closely followed with no significant neurological change, and obliteration of the AVM was strongly indicated by MRI taken 2 years after GKS ().


A 7-year-old girl visited our outpatient clinic complaining of TIAs in the left arm and leg for 3 years. Brain MRI did not reveal any parenchymal lesion, but severe ICA steno-occlusion was suspected on MRA. A neurological examination on admission revealed no focal neurological deficit. The patient underwent cerebral angiography, which revealed severe steno-occlusion of the right ICA, ACA, and MCA with abnormal vascular networks (). The left ICA was relatively unaffected but diffuse stenosis of the left MCA and ACA was observed. There was no arteriovenous shunting at that time. Diamox-enhanced SPECT revealed mild hypoperfusion in the right posterior parietal lobe and decreased vascular reserve in the right frontal, temporal, and parietal lobes. EDAS was performed on the right hemisphere, followed by subsequent EDAS on the left hemisphere. Follow-up MRI and MRA, taken 1 year after EDAS, confirmed the absence of abnormal signal void structures and the presence of transdural collateral vessels from the bilateral EDAS sites. The patient reported no TIAs thereafter but has been lost to follow-up.
The patient revisited our outpatient clinic 6 years after the last follow-up for evaluation of incidental lesions on MRI. Although no new infarctions or hemorrhages were identified, abnormal signal void structures were noted at the anterior part of the right cingulate gyrus (). Follow-up angiography was performed 8 years after EDAS and confirmed a newly developed AVM with MMD progression. The AVM was slowly fed by the reconstituted anterior cerebral artery and drained into the superior sagittal sinus through a single cortical vein (). The new but asymptomatic AVM is currently being closely followed for more than 2 years without further surgical intervention. Subsequent MRI performed 9.5 years after EDAS did not reveal any significant change.


A young girl (two years, four months old), who was born at term after a normal pregnancy, experienced poor weight gain despite eating normally and having no signs of diarrhea. She was admitted to our hospital at the age of two years eleven months for treatment of MDS. Before hospitalization at the age of two years four months, she had been admitted for acute bronchitis and suspected MDS with pancytopenia and an abnormal bone marrow examination. She was a well-developed child with no physical abnormalities, but she was only 86.6 cm tall (-1.2 standard deviation) and weighed 9.6 kg (-2.3 standard deviation). Hemoglobin level was 10.6 g/dL, white blood cell count was 1.89×109/L with 51% neutrophils and 32% lymphocytes, platelet count was 168×109/L, AST was 93 IU/L, ALT was 106 IU/L, IgG was 2390 mg/dL, IgA was 0 mg/dL, IgM was 148 mg/dL. Other test results are shown in . The diagnosis made by the central review system in Japan was selective IgA deficiency and refractory cytopenia with multilineage dysplasia. Her general condition remained good, but the white blood cell count and hemoglobin level slowly decreased. After undergoing multiagent immunosuppressive therapy (horse antithymocyte globulin 15 mg/kg for five days, cyclosporine 6 mg/kg for three months, methylprednisolone 2 mg/kg the first seven days and methylprednisolone taper over 28 days), the white blood cell count and hemoglobin level increased, and she was discharged because of transfusion independence at the age of three years. Three months later she began to have watery diarrhea several times a day and started to take oral antiflatulent. Sudan stain test found no fecal fat excretion. Pancreatic exocrine function remained within normal limits and computed tomography of the abdomen showed a normal image of the pancreas. Bone marrow aspiration showed no increase in hematopoietic cellularity at the age of three years six months. At the age of three years seven months, she was readmitted to our hospital and underwent a second course of multiagent immunosuppressive therapy with antithymocyte globulin, methylprednisolone and cyclosporine. Hematopoietic recovery was observed and she became transfusion independent again at the age of four years. However, she was repeatedly hospitalized because of infections, such as acute bronchitis and acute enterocolitis. At the age of four years four months, she presented subacute hypocalcemic and hypomagnesemic tetany and was hospitalized again. Pancreatic exocrine function was within normal limits. Her condition improved after the initiation of therapy that included elemental diet, calcium chloride and magnesium hydroride preparation through a nasogastric tube. The cause of the electrolyte abnormalities was unknown. At the age of five years three months, she underwent an upper endoscopy and colonoscopy to investigate the cause of persistent watery diarrhea and electrolyte abnormalities. Diffusely scattered granular mucosa was found in the duodenum and terminal ileum (). Pathological examination revealed severe villous atrophy and planarized mucosa. Moderate inflammatory cell infiltration in the mucosal stroma and mild lymphoid infiltration in the mucosal epithelium was detected (). IgA, IgM, IgG positive plasma cell infiltration was found in the mucosal stroma by immunostaining. These findings were consistent with celiac disease and a gluten-free diet was started. In spite of the diet, watery diarrhea continued, and electrolyte abnormalities sometimes appeared. The antiendomysial IgA antibody, antireticulin IgA antibody, and antigliadin IgA and IgG tests were all negative, and testing for anti-tissue transglutaminase (anti-tTG) antibodies was not performed. Serological testing to HLA-DQ typing showed DQ5/DQ8. We excluded the diagnosis of celiac disease because of lack of anti-tTG antibodies. At the age of five years seven months, she again had an upper endoscopy. The gluten-free diet was discontinued because there were no findings of macroscopic or microscopic improvement in the duodenum. Severe villous atrophy and planarized mucosa were found in the duodenum as had been seen by the previous endoscopy. At the age of five years nine months, no skeletal abnormalities including metaphyseal dysostosis, thoracic dystrophies, and low bone turnover osteopenia were observed by the pediatric radiologist. At the age of six years, she was administered parenteral nutrition through a central venous catheter because of persistent electrolyte abnormalities and poor nutritional status. After high-calorie infusion, electrolyte abnormalities improved and she gradually gained weight. At the age of six years four months, she was diagnosed with growth hormone deficiency by the growth hormone stimulation test and we initiated hGH treatment. At the age of seven years one month (five years after onset), genetic analyses of SDS were performed because she had MDS, chronic dyspepsia and lower limit of normal serum level of pancreatic exocrine enzymes. Mutations (183-184TA>CT and 258+2T>C mutations) of the SBDS (Shwachman-Bodian-Diamond syndrome) gene were detected and she was diagnosed with SDS. After diagnosis, she was treated with pancreatin, and after two months a slight improvement in diarrhea and weight gain was observed. She is soon to receive a bone marrow transplant because of ongoing pancytopenia.


A 60-year-old Indian male relative of a patient visiting the dental outpatient department was noticed with a massive swelling on the right side of his face. Verbal consent was obtained from him for examination. History revealed a slowly growing painless swelling of the right mandible of 30 years duration that gradually extended to involve the upper jaw, right side of face and temporal region. The swelling was well-circumscribed, roughly ovoid, lobulated, nontender and firm and measured 18 cm × 12 cm × 11 cm, extending 3 cm above supraorbital margin superiorly and behind pinna of the ear laterally on the right side. Intra oral examination revealed a huge nontender fleshy mass of the right mandible of differing consistency displacing the tongue, obliterating buccal sulci and extending from lower right canine to retromolar area. There was partial and spontaneous loss of dentition. Posterior-anterior view of the skull revealed a huge soft tissue mass shadow on the right side of face involving posterior part of the maxilla and with mandibular destruction []. Coronal section of computed tomography (CT) showed lesion extended from tempero-parietal region till the hyoid bone with displacement of greater wing of sphenoid, temporal bone and maxillary antrum. Axial section of CT scan at a higher level showed erosion of the temporal bone with dural extension. Three-dimensional reconstructed views showed the entire tumor extent that had resorbed the mandible, zygoma and temporal bone on the right side []. Baseline laboratory investigations revealed low hematocrit, raised platelet count and erythrocyte sedimentation rate, but normal protein levels. The patient was provisionally diagnosed with multicystic ameloblastoma. Fine-needle aspiration cytology from the swelling yielded a hemorrhagic, cystic fluid consisting of inflammatory cells in a fibrinous background. Histopathological examination of the tissue specimen obtained by incisional biopsy from the swelling revealed typical ameloblastomatous follicles with varying amounts of granular cell changes, cystic degeneration, and squamous metaplasia in the follicles []. It was thus diagnosed as a GCA. After improving patient's hematocrit, hemimandibulectomy with disarticulation was performed. The tumor mass was resected along with the parotid gland, facial nerve, zygoma and zygomatic arch of the ipsilateral side. Intra operatively, a 2 cm squamous part of the temporal bone was resorbed with a dural tear, but there was no cerebrospinal fluid leakage. Reconstruction was done with pectoralis major myocutaneous flap. The excised specimen measured 17 cm × 11 cm × 9 cm and weighed 500 g. The interior showed multiple locules, few with whitish nodular projections. Histopathological findings from the specimen samples were in agreement with the initial diagnosis. However, our attention was drawn to the unique epithelial changes seen in this case. There were bland looking epithelial cells with flattened epithelium and whorling resembling squamous odontogenic tumor and conventional ameloblastomatous islands budding from the oral epithelium with focal to extensive proliferations of GCA islands form oral epithelium. Granular cell changes were noted extending right across the entire oral epithelium, from the basal and spinous layers up to the superficial layers []. To ascertain the nature of granular cells, immunohistochemistry was performed with CD68, bcl-2, and Ki-67. Intense positivity for CD68 was shown by granular cells in the follicles, but not by the peripheral ameloblastic cells. The latter however showed moderate staining to bcl-2 and weak staining to Ki-67. The granular cells showed occasional faint to no reactivity toward Ki-67 []. The patient was disease free in a 4-year follow-up.


A female patient, 37 years old, came to the dermatology outpatient clinic one hour after
a meal, with increased volume of the left submandibular region associated with the onset
of a lesion described as a painless aphtha on the base of the tongue one day before. She
mentioned a sensation of having parotiditis, although she had had mumps in childhood.
Upon examination, tumefaction was observed in the proximal region of the lower lingual
frenulum with presence of a yellowish spot, hard on palpation, at the center of the
lesion (). Due to the topography and
hardened consistency, it was hypothesized that a sialolith might be obstructing the left
submandibular salivary gland ostium. A massage of this salivary gland ("milking"),
clamping and calculus extraction were carried (). After the duct of the salivary gland was unclogged, a great quantity of
saliva was drained and symptoms relieved ().


A 17-year-old young girl reported to hospital with complaints of pain in right iliac fossa since 2 days associated with mild grade fever and nausea. On examination, her pulse was 110/min, blood pressure of 110/70 mmHg, and temperature of 99°F. Abdominal examination revealed the presence of mild diffuse tenderness, rest of the clinical examination was within normal limit. Hematological investigations revealed hemoglobin of 10 g/dL, total leukocyte count of 14,000/uL with differential count of 60% granulocyte, 24% lymphocyte, and 16% monocyte. Her platelet count was 334,000/uL. Erythrocyte sedimentation rate was 40 mm/1st h. Biochemical investigations showed serum creatinine of 0.6 mg/dL with urea of 24 mg/dL. Urine examination was within normal limit. Ultrasound examination of the abdomen and chest radiograph did not reveal any positive finding. A probable diagnosis of acute colitis was kept. She was started on injection ceftriaxone 1 g 12 hourly along with intravenous fluid supplementation.
Five days after starting on intravenous ceftriaxone, she developed facial puffiness, anasarca, decrease urine output and reddish palpable purpuric nonblanching rash over both the legs [] which spread to the both upper limbs in next 24 h []. There was no evidence of gastrointestinal bleed or hematuria. Her urine examination showed the presence of albumin in the range of 30 mg/dL, urine microscopic examination showed the presence of eosinophils and granular cast. Serum creatinine jumped to 2.1 mg/dL with urea of 55 mg/dL. Urine output over next 24 h was 400 ml with total intake of 2 L. Patient was evaluated for the probable cause of acute renal failure and palpable rash. Laboratory tests for hepatitis B, hepatitis C antigens and antibodies, retrovirus test, antinuclear antibodies, antineutrophil cytoplasmic antibodies, C3, rheumatoid factor, and cryoglobulins were negative. Repeat chest radiograph did not show any evidence of tuberculosis. Echocardiography did not reveal any evidence of infective endocarditis. Repeat complete blood count showed the presence of eosinophilia (4%) with an absolute eosinophil count of 620/mm3. Skin biopsy was performed from the palpable purpura over lower limbs [] which revealed the presence of LCV. Immunofluorescence microscopy of skin biopsy did not show any IgA deposits. Suspecting a possibility of drug induced LCV, ceftriaxone was stopped immediately. In view of renal involvement, she was started on oral prednisolone 40 mg once a day. Investigations to search for the alternative cause for the LCV did not reveal any positive finding. Over next 48 h, patient improved clinically with a significant decrease in skin rash. Renal parameters came down to normal (serum creatinine 1.2 mg/dL, urea 20 mg/dL) 5 days after stopping ceftriaxone with adequate urine output. Patient was discharged after 1-week with a significant reduction in skin rash and normal renal parameters.


A 64 year old woman reported experiencing a sudden onset of neck pain, hand and foot paresthesia when she woke early in the morning. She was sent to a local hospital, where she was treated conservatively with external stabilization of the neck and bed rest. At 2 weeks later, the patient was transferred to our hospital because her symptoms had not relieved. There were no clinical or laboratory findings of rheumatoid arthritis. There was no history of head and neck trauma. X-ray showed that the atlantoaxial instability [Figure -]. Computed tomography revealed an abnormality of the odontoid process [Figure and ]. Magnetic resonance imaging detected an oval retro-odontoid cystic mass, which compressed the spinal cord. The mass showed uniform low intensity on T1-weighted image [] and uniform high signal intensity on T2-weighted image [].
The surgery was carried out with the patient positioned prone with the head slightly flexed and a midline incision was made. Subsequently, paraspinal muscles were peeled to expose the bilateral laminae of C1 and C2. Magerl's method was used to place pedicle screws on C1 and C2. The laminectomy of C1 and partial C2 were performed, and then the neck was slightly extended to reduce the atlanto-axial joint. A pedicle screw system was used for fixation. Finally, an autogenous iliac crest graft was implanted in to C1 and C2 bilateral joints to facilitate fusion. Spinal cord monitoring was available during the procedures.
Postoperatively, neck pain immediately disappeared and neurologic symptoms disappeared by 1 week. The patient was ordered to wear a cervical collar for 3 months for protection. X-ray [Figure and ], computed tomography [] and magnetic resonance imaging [] were taken on the day of surgery. At 24 months after surgery, the retro-odontoid cystic mass was no longer present [], and the patient remained symptom free and returned to independent daily living. Magnetic resonance imaging scans at 24 months after surgery showed that atlantodental interval was wider, compared to the day of surgery. There was complete bony union of C1-C2.


A 79 year old male weighing 55 Kg with a height of 168 cm, was scheduled for right middle lobectomy for suspected lung carcinoma. His concurrent co-morbidities included hypertension, hyperlipidemia, and non-insulin dependent diabetes mellitus. Prior to this current procedure, he had successful diagnostic mediastinoscopy for his hilar lymphadenopathy. Previous intubation was noted to be challenging with Cormack and Lehane grade III larynx and with the aid of LMA CTrach™ (The Laryngeal Mask Company Limited, Germany), he was then uneventfully ventilated and intubated. Decision was made to utilize Bonfils retromolar intubating fiberscope (Storz, Germany) as first-line airway device to intubate the patient with a left sided DLT using a 37Fr left Broncho-Cath (Mallinckrodt Medical, Ireland). The DLT was modified to accommodate the Bonfils retromolar intubation fiberscope into the bronchial lumen of DLT. The connecting tubes of both tracheal and bronchial parts of DLT were shortened by 1.5 cm to accommodate the length of the Bonfils, and the endotracheal tube (ETT) holder of the Bonfils had to be removed [].
The thoracic epidural at the level of T6-7 was placed prior to induction for intraoperative and postoperative analgesia. With adequate preoxygenation, anesthesia was induced with propofol and fentanyl, and muscle relaxation was achieved with rocuronium as authors were able to perform satisfactory bag and mask ventilation, and patient was intubated with the pre-prepared left sided 37Fr DLT using Bonfils retromolar intubating fiberscope. The Bonfils allowed a right retromolar approach for intubation and the vocal cords were easily visualized. Once the DLT just passed the vocal cords the Bonfils fiberscope was withdrawn. The DLT was then rotated 90° and advanced until slight resistance was felt. The double lumen position was confirmed by both auscultation and fiberoptic bronchoscope (Storz, Germany). He had stable hemodynamics throughout the procedure and was extubated uneventfully at the end of the procedure.


Our patient was a 42-year-old man. He has received insulin treatment for diabetes since the age of 34. Before his diagnosis of diabetes, he had drunk in large quantities, and he has had a smoking history of 20 cigarettes per day for 15 years. His family history was unremarkable. Regarding the present illness, he visited a neighborhood clinic with a chief complaint of severe back pain. Abdominal CT revealed dilatation of the main pancreatic duct and pancreatic stones (fig. ). Chronic pancreatitis was diagnosed, and the patient was referred to our hospital. Physical examination revealed no abnormal abdominal findings. Blood tests revealed no increase in amylase and hepatobiliary enzymes, the absence of jaundice, and no increase in inflammatory findings; however, his HbA1c level was high (7.8%).
First, we performed extracorporeal shock wave lithotripsy (ESWL). After several daily 1-hour ESWL sessions, ERCP was performed via the main pancreatic duct. However, deep cannulation attempts into the pancreatic duct failed because of pancreatic stenosis (fig. ). Next, we tried to achieve deep cannulation via the accessory pancreatic duct, but this was prevented by a stenotic lesion in the accessory pancreatic duct. We switched the procedure to the accessory pancreatic duct using a guide wire (fig. ). Contrast medium was then injected through a catheter and was seen to flow quickly in the direction of the liver (fig. ). We speculated that a fistula had developed between the posterior superior pancreatoduodenal vein and the accessory pancreatic duct, and so the procedure was terminated, and the catheter was withdrawn immediately. Duodenoscopy showed small amounts of blood from the minor papilla (fig. ). The patient's subsequent progress was uneventful, and no clinical symptoms of hemorrhage developed. There was no clot in the portal vein. Up to now he has been followed up using analgesics without surgical intervention.


A 17-year-old Caucasian female came under our observation with a history of full-term precipitous delivery. At birth, the Apgar score was 7 at minute 1 and 9 at minute 5, and she showed postnatal hepatosplenomegaly, jaundice, and a petechial rash on her face, limbs, and trunk. Motor development was delayed: she gained head control at 4 months, the ability to sit at 12 months, the ability to stand unassisted at 13 months, and learned to walk, albeit precariously, at 20 months. By this time, no verbal language had developed nor had she gained adequate voluntary control over chewing and tongue movements. When she was one year old, clinical examination showed bilateral sensorineural hearing loss and mild mental retardation. An auditory prosthesis was implanted, and logopedistic rehabilitation was suggested. Transfontanellar ultrasonography, made possible by persistent opening of the fontanelle over 12 months, showed mild dilatation of the ventricular system, more prominent in the left lateral ventricle than in the right, in association with bilateral parenchymal calcifications. A diagnosis of congenital cytomegalovirus (CMV) infection with intracranial calcifications was made. Clinical examination at admission to our institution when she was 17 years old showed myopathic facies with amimia, bilateral hyposthenia of the facial muscles, an elongated and partially protruded tongue, an ogival palate, and bilateral claw hands.
Neurologic examination showed a lack of speech with emission of inarticulate sounds and severe deafness. She was able to understand spoken language and communicate using international sign language. There was salivary incontinence due to facial muscle hyposthenia, tongue protrusion and lateral movements were impossible, chewing was inadequate, a bilateral eyelash sign was present, and automatic-voluntary dissociation was also recognized. Reflexes were normal, except for an indifferent plantar response bilaterally. The patient did not require assistance for activities of daily living, such as eating, bathing, and dressing. She was described by one of her parents as stubborn, immature for her age, and prone to aggressive outbursts when frustrated; she showed a narrow range of interests and preferred to spend her time alone. During neuropsychologic evaluation, the patient appeared wakeful and sufficiently compliant, although easily distracted and intolerant of errors. General cognitive abilities were found to be borderline impaired (intelligence quotient 77) when measured with Raven’s Progressive Matrices. There was also evidence of impaired visuomotor coordination and severe deficits in attention and executive function.
A computed tomography brain study revealed a few bilateral cortical convolutions and microscopic subcortical calcifications (), especially in the frontal regions. Magnetic resonance imaging (MRI) of the brain revealed smooth opercula and frontal (both superior and middle frontal circumvolutions) cortex () and a “cobblestone” appearance in both frontal cortices (). The Sylvian cisterns appeared vertical and shallow bilaterally. An electroencephalogram performed when she was 21 years old showed diffusely deregulated cerebral electrical activity together with ubiquitous fast paroxysms, prevalent in the left temporal area ().
The patient has been undergoing logopedistic rehabilitation since one year of age; she also underwent psychomotor rehabilitation from 3 years up to 10 years of age, with an improvement in communicative ability and emotional control. Her salivary incontinence was ameliorated by administration of an anticholinergic drug (trihexyphenidyl 2 mg twice daily).


A 30-year-old lady with a diagnosis of SLE, on oral prednisolone (10 mg OD) and hydroxychloroquine (400 mg OD) since 2 years, presented with a progressive neurological illness of 8 months duration. The illness heralded with holocranial-throbbing headache associated with photophobia and phonophobia. She also had history of intermittent fever (up to 103°F) with occasional chills and rigors for last 6 months. There was a history of slowly progressive painless diminution of vision in both eyes 4 months prior to admission. There was worsening in form of persistent vomiting for last 2 months along with reduced appetite and loss of weight. Examination was remarkable for pallor, neck stiffness and bilateral papilledema. Her visual acuity was 20/30 bilaterally using the Rosenbaum vision-screening chart. Confrontation testing revealed peripheral constriction of the visual fields. Rest of the general physical, neurological and systemic examination was normal. On investigations, she had bicytopenia {hemoglobin 80 g/L (8 g/dL), platelet count 76 × 109/L, WBC count 6.1 × 109/L}, low C3 levels {0.4 g/L (normal: 0.9-1.80 g/L)} and positive anti-double strand DNA antibodies. Initial CSF analysis showed no cells, normal protein and low glucose {1.9 mmol/L (34 mg/dl), CSF/serum glucose ratio = 0.34}. CSF bacterial and fungal cultures, PCR for tuberculosis, VDRL, India ink staining and cryptococcal antigen test were negative and CSF adenosine deaminase (ADA) levels were 3 IU/L (normal <10 IU/L). The cranial MRI and MR venography were unremarkable. Contrast enhanced CT scan of chest and abdomen showed abdominal lymph adenopathy. Fine needle aspiration from these nodes revealed reactive lymphadenitis. Considering her diagnosis to be immune mediated aseptic meningitis, the dose of oral prednisolone was increased to 60 mg OD. This led to symptomatic improvement for 5 days but later there was worsening with increasing headache and vomiting. She was empirically started on anti-tubercular treatment (ATT) (isoniazid 300 mg, rifampicin 450 mg, pyrazinamide 1250 mg, ethambutol 800 mg, pyridoxine 20 mg daily) and dexamethasone 8 mg IV, 8 hourly, which led to transient symptomatic improvement for 4 days, after which her meningitic symptoms reappeared. A repeat CSF examination was conducted 10 days after the first CSF, which revealed 10 cells/μl, all lymphocytes, raised protein (2.39 g/L), low glucose {1.8 mmol/L (33 mg/dl), CSF/serum glucose ratio = 0.14} and adenosine deaminase of 7 IU/L. CSF was negative for cytomegalovirus (CMV) and Epstein-Barr virus (by PCR). Qualitative real time PCR was positive for HSV-2 DNA. ATT was stopped (received for a total of 10 days) and she was administered acyclovir 500 mg (10 mg/kg) IV 8 hourly for 14 days. Prednisolone was tapered off to 10 mg daily dosage. Her headache and fever subsided and vision improved (to 20/20 bilaterally) over the next 2 weeks. At 3-month follow-up visit patient was free of all symptoms and signs of chronic meningitis.


A 5-year-old Korean boy with blisters and erosions on the palms and soles visited our clinic. The symptoms had been recurring since infancy from minimal mechanical trauma. Upon physical examination, several bullae vesicles with crusts and erosions on both soles, and a vesicle on the fingertip were observed on the patient. The oral mucosa appeared to be asymptomatic (). All affected lesions were limited to trauma-prone sites. A skin biopsy revealed a large intraepidermal vesicle in the suprabasal layer with scanty inflammation of the dermal portion (). These phenomena were recorded in the family history and presented in a pedigree with his younger sister and his father (). Mutational analysis of the patient and parental DNA showed a thymine-to-cytosine transition mutation in codon 608 of exon 2 in the KRT5 gene in the patient and his father only (). Identical digestion patterns were observed for the patient and his father in an RFLP analysis with Pst I (), but the patterns differed for his mother and the control group. These results indicate that the mutation was inherited from his father. The patient was treated with systemic and topical antibiotics as supportive treatment during the diagnosis as well as during regular follow-up appointments ().


A 13-year-old girl presented with reddish raised asymptomatic lesions over both hands and feet for the past five months. The lesions appeared spontaneously as lentil-sized raised lesions over the right index finger and gradually increased in size and number and coalesced to form larger plaques with appearance of similar lesions over other fingers as well as soles. No personal or family history of oral ulcers, joint pains, photosensitivity, frothing of urine or any systemic complaints in the form of chest pain, abdominal pain, or breathlessness was found. Examination of hands and feet revealed multiple papular lesions, which were erythematous and edematous. They were discoid erythematous scaly atrophic papules to plaques over the ventral and lateral aspect of the fingers of hand and feet as well []. The lesions over the palms and soles were tender to touch. Dorsal aspect of hand had multiple hyperpigmented macules suggestive of postinflammatory hyperpigmentation. Bilateral concha showed violaceous discoid plaques of size 1 × 1 cm []. Nails of both the hands showed ragged cuticle. There was no significant lymphadenopathy and all mucosae were normal.
Hematological investigations were within normal limits except anemia (Hb-10.9g/dL) and raised erythrocyte sedimentation rate (30 mm in first hour). Biochemical tests for hepatic and renal functions, serum proteins, urine examination and chest radiograph were normal. VDRL was non-reactive. Rheumatoid factor was negative. Anti-nuclear antibody test was positive with a speckled pattern of immunofluorescence, and the titer being 1:160. These antibodies were identified as anti-dsDNA, anti-Sm, anti-Ro52, anti-histone and anti-nucleosomal antibodies. Histopathology from the lesion over the finger showed focal basal cell vacuolization of the epidermis and moderate perivascular and periadnexal lymphocytic infiltrate in the dermis []. Direct immunofluorescence (DIF) of lesional skin showed IgM and C3 deposition along the basement membrane zone. Based on the clinical and laboratory findings a diagnosis of CHLE was made and treatment was initiated with potent topical steroids twice daily until complete remission.


A 50-year-old female diagnosed with osteochondritis dissecans of the right talus underwent osteochondral allograft surgery for the second time. The patient was advised to wait 10 days after removal of the stitches before applying any topical medication. After 10 days, the patient initiated application of the topical anhydrous silicone base containing pracaxi oil, with no additional active substances, twice daily (Fig. a). Following 2 weeks of application, the thickness of the surgical scar was visibly reduced (Fig. b), and the wound was graded as “much improved”. The patient reported drastic improvement in the feel and size of the scar, and noted less irritation in the area.


A 57-year-old female with type I diabetes underwent 3 reconstructive surgeries following a fractured ankle. These surgeries resulted in 3 ankle scars, including one hypertrophic scar. Prior to this study, the patient had applied several different products to the scars, but still experienced itching associated with the hypertrophic scar and was generally not satisfied with the aesthetics of the scar (Fig. a). One year after her last surgery, the patient began application of compounded topical anhydrous silicone base containing pracaxi oil, without additional active substances, twice daily to the hypertrophic scar. Following 1 week of application, the patient reported that the overall severity as well as the thickness and coloration of the scar were considerably improved (Fig. b), and the scar was graded as “much improved”.


A 72-year-old male underwent a surgical removal procedure and 33 sessions of radiation therapy on the right side of the head and neck to treat acinic cell carcinoma. The patient experienced radiation burns at the site of treatment with extremely sensitive, dry, and scaly skin resulting in extreme pain and discomfort (Fig. a). Topical compounded anhydrous silicone base containing pracaxi oil without additional active ingredients was applied to the radiation burn 4 times daily, or as needed to relieve discomfort. Following 4 days of application, the patient acknowledged significant healing and tissue regrowth. By day 10, the radiation burn was almost completely healed and pain free, and on day 14, the skin was smooth, soft, and pink and the patient reported that he was pain free (Fig. b). The burn was considered “much improved” by an independent dermatology reviewer.


The guardians of a 15-month-old female suffering from diaper rash, a moisture-associated skin condition, had previously treated the patient with several over-the-counter topical products without success. The condition worsened with time, resulting in increased pain and discomfort for the child (Fig. a). Application of compounded topical anhydrous silicone base containing pracaxi oil (without additional active ingredients) was recommended two to four times daily at diaper change. After 48 h of application, the diaper rash had improved (Fig. b) and the toddler no longer appeared to experience pain or discomfort. The rash was considered “improved” from baseline by an independent dermatology reviewer.


A 5-month-old female was severely scalded with liquid on the face, resulting in a major second degree burn (Fig. a). Following the incident, compounded topical anhydrous silicone base containing pracaxi oil without additional active ingredients was applied twice daily to the burn, with sterile bacitracin ointment to prevent infection. After 8 days of application, the infant’s facial wound healed completely with no evidence of scarring (Fig. b). The burn was considered “much improved” from baseline after the application of compounded anhydrous silicone base containing pracaxi oil.


A 22-year-old female was left severely scarred on the face following a car accident. The patient had undergone six plastic surgery procedures and had previously used several treatments to lessen scar formation. However, she was still unsatisfied with her facial scars and overall complexion (Fig. a). Four years after the accident occurred, the patient began application of a medicine containing 1% pentoxifylline (a collagen and wound contraction inhibitor), 1% caffeine (to increase blood flow to the area), and 1% tranilast (a collagen synthesis inhibitor) in topical compounded anhydrous silicone base containing pracaxi oil.
The compounded medicine was applied twice daily to the facial scars. After 3 weeks of application, there was an overall improvement in the severity of the scars and the patient reported that the skin around the scar was softer and that the redness of the scar had decreased (Fig. b). The scars were considered “much improved” from baseline following application of the compounded medicine.


A 40-year-old female fell on both elbows and was painfully injured as a result of repeated impact with the ground during a fitness competition. Multiple layers of skin were scraped off and the patient presented with open wounds on the right and left elbows (Fig. a, b).
The patient was instructed to apply a compounded medication containing 2% mupirocin in compounded topical anhydrous silicone base containing pracaxi oil three times a day. Mupirocin 2% was added to the compounded base to prevent bacterial contamination of the wounds. After 11 days of application, the wounds on both the right and left elbows were completely closed and the patient reported fast improvement in the use of her elbows (Fig. c, d). The wounds were considered “much improved” from baseline following application of the compounded medicine.


A 30-year-old housewife presented with 7 days history of fever with chills, headache and with four episodes of generalized tonic clonic seizures for 2 days prior to hospital admission. On examination, there was eschar on the right neck region [] and the vitals were stable. The central nervous system (CNS) examination showed no focal neurological deficits and there was terminal neck rigidity. The other system examination was within normal limits. Laboratory examination revealed normal renal and liver function tests. The peripheral smear and total count was normal. Cerebrospinal fluid (CSF) was clear with elevated protein (100 mg/dl) and there were few lymphocytes. The enzyme-linked immunosorbent assay for O. tsutsugamushi was positive. The CSF, blood and urine cultures were sterile.
Leptospira serology and WIDAL were negative. Magnetic resonance imaging brain was normal. She received acyclovir for 1 day and on day 2 she was started on empirical doxycycline (100 mg BID). Her symptoms completely resolved in 48 h and were discharged from the hospital on day 8.


A 23-year-old female presented with a history of fever, chills, and headache for 5 days and altered sensorium for 1 day. Her pulse rate was 98/min, blood pressure 100/70 mmHg, temperature 39°C, and no lymph node enlargement. The CNS examination showed neck rigidity. Her other system examination was normal. The peripheral smear showed platelet count of 200,000/dl with normal hemoglobin and normal total count. The WFT was positive for scrub typhus. The CSF study showed elevated protein (120 mg/dl) and 100 lymphocytes. The leptospira serology and WIDAL were negative. The renal and liver function tests were normal. The patient was treated empirical with doxycycline (100 mg BID orally for 7 days). Her fever subsided on day two and discharged from the hospital on day 7.


A 55-year-old man (subject 1) presented with bilateral metamorphopsia, with decimal visual acuity being 0.8 in the right eye and 1.2 in the left. The clinical diagnosis of VKH was made based on bilateral panuveitis and multifocal exudative retinal detachments at the posterior poles (). High-dose corticosteroid therapy with gradual tapering was initiated 3 days after presentation. The serous detachments entirely resolved within 9 weeks and visual acuity in the right eye improved to 1.2.
Serial spectral-domain OCT (SD-OCT) images obtained at the preuveitic, uveitic, and posttreatment phase are shown in . SD-OCT obtained 1 year before the onset of VKH due to symptoms of a floater and visual disturbance demonstrated a dome-shaped posterior protrusion of the RPE and outer retinal layers into the choroidal cavity (ie, conforming FCE) at the fovea in the right eye. SD-OCT at the uveitic phase identified multiple bilateral sensory retinal detachments, with the FCE now involving only the RPE (ie, nonconforming FCE). Nine weeks after the treatment, the nonconforming FCE returned into a conforming pattern.
The FCE pattern and its alteration during treatment of the case with VKH were compared to those of four subjects with FCE associated with other chorioretinal conditions (). This comparison group consisted of two patients with age-related macular degeneration (AMD; subjects 2 and 3), one subject with a macular hole (MH; subject 4), and one asymptomatic individual (subject 5). Treatment with intravitreal ranibizumab injections and vitrectomy had been performed in the patients with AMD and MH, respectively. A conforming FCE was observed in three patients (subjects 2, 3, and 5) and pattern alteration from nonconforming to conforming FCE was found after treatment in one subject (subject 4). In the two subjects with AMD, a hyperreflective material around the FCE was absorbed, resulting in a well-demarcated conforming FCE after treatment.


A 22-year-old female patient was referred with a complaint of gummy smile and hyperpigmented gingiva []. On examination, pseudopockets (approximately 2–3 mm) were detected in the maxillary and mandibular anteriors along with generalized excessive amount of melanin pigmentation on the gingiva []. The patient was explained about the treatment options and informed that, after depigmentation, melanin pigmentation tends to recur and, as gingivectomy is also performed simultaneously, the overall appearance would be worth doing it.
Gingivectomy and depigmentation were planned in the 15–25 region and the 34–44 region using a 810 nm diode laser. Although procedures like this can be performed under topical anesthesia, infiltration was planned as the patient was anxious and sensitive.
Under infiltration anesthesia with 2% lignocaine (with 1:100,000 adrenaline), an 810 nm GaAlAs diode laser (Picasso™)*(Picasso, by AMD lasers) was used in contact mode with the gingival tissues. The procedure was performed at 1.5 W in pulsed mode. Initially, gingivectomy was performed followed by gingivoplasty and depigmentation []. The patient was extremely cooperative and comfortable during the procedure. No dressing was applied after the surgery. Postoperatively, a vit. E gel was prescribed for local application -three to four times a day for 2 days. Because a large area of the raw wound was kept exposed, antibiotic Cap. Amoxicillin 250 mg three times a day for 3 days and an analgesic ibuprofen–paracetamol was prescribed twice a day for 1 day and later SOS.
The patient reported no adverse effects or discomfort during the postoperative healing phase. She was happy and satisfied with 6-month postoperative results [Figures and ].


Ms. B, a 16-year-old unmarried girl, a student of 1st psychiatric urgent care, belonging to a Hindu lower socioeconomic strata family, with no past or family history of any psychiatric illness or substance abuse, was brought with a history of eating paper and inhaling substances like kerosene for 3 years. Her family members would chew betel nut, and she also started chewing betel nut regularly without her parents’ knowledge. When her family came to know about this, she was restricted and was advised against it.
At the same time when alone at school 1-day, she felt like eating notebook paper and got immense pleasure after it. An hour later she had another piece of paper and passed the whole day without having any adverse impact. Next day also without the knowledge of her classmates she ate three pieces of paper. Within a month, she developed a habit of eating 4-5 A4 size papers daily during school time. She had never tried to cut down on eating paper until she was brought to the hospital. Meanwhile, while transferring kerosene to another container she accidentally experienced the smell of kerosene and was inclined towards that smell. She felt good, and that led her to smell kerosene daily without her family's knowledge. She used to feel relaxed and detached from the surroundings after inhaling the kerosene for about 3-4 h. After 6 months of this practice she realized that if she misses inhaling the kerosene for even 1 or 2 days she will feel restless, having low mood at school, not interested in doing any household work and craving to inhale kerosene. In that frustration, she used to eat around 10 papers daily and run behind autos and other vehicles to inhale that smell. Her family noticed that she had academic problems, had poor concentration and was not helping in household works. She was frequently angry, argumentative, throwing things at parents and trying to harm herself.
After about 3 years family noticed her addiction practices and brought her for evaluation.
On examination she was well kempt, cooperative and rapport could be established. There was no mood, thought or perception abnormality. She had partial insight and was willing for the treatment. Cognitive functions, physical examination, investigations such as complete blood picture, urine routine and X-ray abdomen, liver function and serum electrolytes were normal. Possibility of lead poisoning was also excluded after peripheral blood smear examination.
She was diagnosed to have pica (xylophagia) with mental and behavior disorder due to use of volatile solvents. She was admitted and started with paroxetine 25 mg in 2 divided doses. To control her withdrawal symptoms clonazepam 0.5 mg was added. After 2 days her withdrawal symptoms were under control, baclofen 20 mg was added for control of craving and supplemental multi-vitamin injectable preparations.
Patient and family members were psycho-educated about the nature of the illness and harmful consequences of addiction. Cognitive behavior therapy (CBT) was employed for stress reduction, coping skills, challenge distressing thoughts and preventing damaging behaviors. After 6 sessions of CBT she started showing improvement in her motivation levels, compulsive behavior and role of psycho-therapy. During the 12 days of hospital stay she remained abstinent from eating paper and inhaling the kerosene, and was considered fit for discharge with an advice for follow-up and continued medications with CBT.


A seven-year-old girl, who had bruises and scars predominantly all over her face [], lower extremities and on her body was referred by a forensic expert. She lived with her mother, and her father had passed away. The child was evaluated in the pediatric clinic and child abuse was suspected as the cause of the bruises and scars, and hence the child was admitted to the pediatric ward for evaluation.
The mother was significantly upset by the suggestion that child abuse may have occurred and refused to allow her daughter to remain in the clinic for observation. The child was born by normal vaginal delivery and there were no complications associated with her birth. On examination, the child appeared normal.
Dermatologic examinations revealed that the child had very smooth, lax skin, but she had wide, atrophic, cigarette paper-like scars on both shins. Other clinical findings included hyperextensible skin [], increased range of motion of the joints, long fingers, toes that bruised easily and fragile skin that had a brownish discoloration []. There were hyper mobile joints with the score of 6/9 on the Beighton scale.
There were no joint dislocations. She had no major health concerns. Other investigations, such as complete blood count and routine examination of the stool proved normal, and the urine and serum levels of sodium, potassium, urea and creatinine were within the normal limits. Reviewing her family history indicated hyperextensible joints and skin in her father's family. Based on the characteristics of the clinical findings and the positive family history, she was thought to have classic EDS, type I or II. Thus, the EDS were included in the differential diagnosis to avoid possible harm to the family that could result from an allegation of abuse. The child was returned to her mother after the correct diagnosis.


In January 2007, we admitted a 64-year-old woman with acute abdominal pain, nausea and vomiting in Ghaem Hospital, Mashhad University of Medical Sciences, Iran. She had low appetite and inability in passing stool and gas in the recent week. On physical examination, she had a distended abdomen and on abdominal radiography, there were multiple air-fluid levels, suspected of bowel obstruction. At laparotomy with a large midline incision, there was adhesion of omentum and bowels on a large heterogeneous pelvic mass. After releasing the adhesions, right ovarian tumor was revealed. Cytoreductive surgery by transabdominal hysterectomy and bilateral salpingoopherectomy (TAH + BSO) and complete surgical staging was done. Histopathologic examination of Hematoxylin-eosin stained tissue samples revealed MMMT in right ovary ( and ) with metastasis to the left adnexa and omentum.
According to the International Federation of Gynecology and Obstetrics (FIGO) staging system (), the patient was in stage IIIA. The serum CA-125 was elevated (386 IU/mL) but the level of CEA and CA19-9 were within normal limits (3.5 IU/mL and 9 IU/mL, respectively).
The patient received six courses of chemotherapy consisting of carboplatin 6AUC and paclitaxel (270 mg/m2) regimen every three weeks. After six courses of chemotherapy, abdominopelvic computed tomography (CT) findings were normal and there was no evidence of residual tumor in the pelvic cavity. CA-125 reverted to normal levels after surgery and six courses of chemotherapy. The patient was followed up every three months by pelvic examination, abdominal and pelvic sonography, and CA-125 tumor marker. After 31-month disease-free survival, a nodule of about 1.5-cm diameter was palpated in presacral region on rectal and vaginal examination. In July 2010, a round, hypoechoic solid mass of 16 × 18 mm at the posterior right part of the rectum was found in transvaginal ultrasonography. Magnetic resonance imaging confirmed the existence of a mass of 17-mm diameter, compatible with a lymph node. CA125 level was increased (65 IU/mL). We recommended transrectal ultrasound-guided biopsy but the patient did not accept. In May 2011, a CT scan showed persist lymphadenopathy of 21 × 11 mm in the presacral region. In June 2011 and after 42 months from the primary surgery, a CT-guided biopsy of the presacral mass was performed and histopathologic examination revealed a metastatic carcinoma. The patient underwent chemotherapy with primary regimen again (carboplatin 6 AUC and 270-mg/m2 paclitaxel) every three weeks and 50.4 Gy radiotherapy in 28 fractions to a standard pelvic box field with photon of 15 mV. After 26 months follow-up, she became progression free with normal abdominopelvic CT findings and normal levels of tumor marker (CA-125) ().


The health of a 7-year-old male child with type I diabetes was maintained on a daily regimen of insulin. One day, however, the child developed sudden onset of cold-like symptoms such as runny and stuffy nose with a mild fever. He was diagnosed with acute sinusitis by a local general practitioner and prescribed three different medications namely paracetamol, chlorpheniramine, and a combination of amoxicillin and clavulanate at their regular doses in syrup form. On the third day of his treatment, the child was brought to a local hospital's emergency department with symptoms of nausea, vomiting, weak and rapid pulse, deep sighing breaths, drowsiness, and unsteady gait. On examination, his blood sugar was extremely high and showed an arterial blood gas pH of 7.12 with base excess of −7 mmol/L, and a bicarbonate of 12.6 mEq/L. The WBC count was 15.2 × 103/μL, hemoglobin of 12.6 mg/dL, and hematocrit of 40.1%. His chemistry panel demonstrated a serum sodium of 134 mEq/L, potassium 4.5 mEq/L, chloride 91 mEq/L, BUN 11.6 mg/dL, creatinine 1.2 mg/dL, and a blood sugar of 440 mg/dL. The serum ketones were positive on urine dipstick. He was admitted as a medical emergency and treated for diabetic ketoacidosis with fluid replacement and insulin therapy to prevent further complications and achieve a normal health state.


Mrs MJ (83 years old) was referred for a routine Home Medicines Review in view of her multiple medications (). Home Medicines Review is a government-funded program in Australia that allows a pharmacist to review patients’ medications at their homes and make recommendations to their general practitioners.,
Mrs MJ’s medical history included: atrial fibrillation, analgesic nephropathy (AN), chronic kidney disease (stage 3, estimated glomerular filtration rate 60 mL/min/1.73 m2), gastroesophageal reflux disease, vitamin B12 deficiency, hypertension, hypercholesterolemia, hyperhomocysteinemia, hypothyroidism, myocardial infarction (two in the 1970s), degenerative spondylolisthesis, and transient ischemic attack.
At review, Mrs MJ described persistent back pain, but was reluctant to take paracetamol, due to her renal disease and previously documented analgesic-induced nephropathy in 1999. The patient had been told by health professionals to be careful with “some painkillers” to avoid further kidney damage, and subsequently had chosen to avoid paracetamol. The patient was unsure which health professional had told her to avoid paracetamol, but had decided it would be prudent to avoid “all painkillers”, and she could not recall a health professional telling her paracetamol was safe. Further, it was documented that the patient had previously experienced acute renal failure from the use of an NSAID. The patient was unsure which NSAID had caused her acute kidney failure, and it was not documented in her medical history.


A 58-year-old male complained of difficulty in swallowing. He had a history of Helicobacter pylori infection and was first diagnosed with p16 protein (p16) positive SCC of right tonsil in 2009. He had a 30-year history of smoking. He was treated with cisplatin, cetuximab, and radiation therapy for the T2N2M0 SCC. A new primary SCC of left tonsil was diagnosed in 2011. After this, the patient underwent another round of chemotherapy with cisplatin, cetuximab, and fluorouracil. In May 2013, an (18) F-fluorodeoxyglucose-positron emission tomography/computed tomography (FDG-PET/CT) was done to assess the response to chemotherapy. A 3.4 cm mass was found in the right thyroid gland, which exhibited irregularity, causing deviation of the underlying trachea []. Fine needle aspiration (FNA) of both lobes of the thyroid gland revealed tonsillar SCC.


A 66-year-old male presented with a complaint of a mass in the neck. He was diagnosed with P16 positive T2N2M0, stage 4B supraglottic SCC in December 2012. He has a history of heavy drinking and is a current smoker with a 40-year history of smoking. He was treated with cisplatin and radiation therapy. He was unable to complete the last 3 days of radiation therapy due to persistent emesis. On a restaging FDG-PET/CT in January 2014, a new intensely hypermetabolic, multifocal, infiltrative mass was found in multiple areas of the thyroid gland []. FNA of both lobes of thyroid gland revealed supraglottic SCC. The patient was referred to hospice care.


A 44-year-old man with a medical history of hypertension and diabetes presented with a 3-month duration of progressive upper back pain and myelopathic symptoms. His chief complaint was unsteadiness with ambulation. In addition, he also noticed increasing hand numbness and weakness in his grip strength.
Clinical examination revealed tenderness at the midthoracic region. He was unable to walk without aid, due to poor balance. The power of his upper limbs was weak, with severe wasting of his triceps and intrinsic muscles of the hand (Medical Research Council, grade 4 power for C5 and C6; grade 3 for C7, C8, and T1 bilaterally). The power in his lower limbs was full. Sensation was preserved; and there was no hyper-reflexia, clonus or spasticity. The Hoffman's test was negative.
A magnetic resonance imaging (MRI) revealed that this sclerotic lesion had infiltrated the spinal canal, and was causing significant spinal cord compression. In addition, there were other lesions in the cervical spine at C5 and C6, which showed cord compression with spinal cord signal changes (). Radiographs of the cervical spine were unremarkable, but the thoracic spine showed a sclerotic lesion at T5 ().
Blood investigation showed normal infective and inflammatory markers (white blood cell, 9.02; erythrocyte sedimentation rate, 6; C-reactive protein, 1.9), with an abnormally high prostate-specific antigen (PSA>100). A Tc99 bone scan showed vertebral hotspots similar to those identified on the MRI. There were no other lesions apart from the multiple ribs involvement (>3). CT scan revealed an isolated lung lesion and an enlarged prostate. No liver metastasis was found. A TRUS (transurethral biopsy) performed by the urologist returned with a histology that was consistent with prostatic adenocarcinoma. The patient was diagnosed with stage 4 prostate cancer, with metastasis to the spine, presenting as cord compression. He was started on dexamethasone and scheduled for surgical decompression and stabilization. His preoperative hemoglobin was 16.7 g/L.
A posterior decompression (laminectomy) of C5, C6, and T5 with an instrumented fusion from C3-T3 was performed. The procedure was uncomplicated. His intraoperative blood pressure was stable around 90/60 mm Hg, with no hypotensive episodes. His heart rate never exceeded 110 beats per minute. Blood pressure was closely monitored using a combination of arterial and noninvasive blood pressure measurements. The surgery took a total of 4 hours and 10 minutes. Throughout the surgery, there was no drop in the neuro-monitoring signals (sensory/motor-evoked potentials). Rather, the sensory signals (amplitude) for the right lower limbs improved following the decompression.
There was an estimated intra-operative blood loss of 3.0 L, with the majority arising from the decompression stage of the surgery. An intraoperative transfusion of 4 units pack cells (approximately 1 L), 1.5 L of Ringer's lactate and 1.0 L of colloid were transfused. Once the patient was extubated, he was reviewed in the high dependency ward. He was alert, moving his legs with lower limb power of grade 5. There was no change in the power of his upper limbs.
Three hours postoperatively, the patient complained that he was unable to move both his legs. Clinical examination revealed motor power of grade 0 bilaterally from L2 to S1. Sensation of all dermatomes was preserved, and his anal tone was intact. Vital signs at this time showed tachycardia of up to 130 beats per minute, and persistent hypotension with a systolic blood pressure of <80 mm Hg. He was still alert and not confused. His drain output at this time was 1,000 mL. His hemoglobin was 9.4 g/L, indicating a drop of 7.2 g/L from his preoperative hemoglobin of 16.7 g/L. An urgent MRI scan was ordered to exclude the possibility of hematoma causing extrinsic cord compression. However, there was no sign of external compression. Instead, an improved canal diameter following the decompression was seen (). The implants were well-positioned ().
A diagnosis of hypotensive shock causing spinal cord ischemia was made. As there was no extrinsic compression observed, a medical treatment to improve his cord perfusion was initiated. This included a blood transfusion to keep his hemoglobin higher than >10 g/L, nasal oxygen and maintaining a normotensive blood pressure. Dexamethasone 8 mg, three times a day, started preoperatively was continued. Over the next few days, the patient made good progress. The power in his lower limbs gradually improved from grade 0 to grade 4 over one week, with an improvement of one grade per day. By the end of two weeks, he was attempting to stand with assistance.


A 58-year-old male patient had developed a chicken egg-sized painful lump on the left popliteal fossa two months before he visited our center. He had no obvious trauma history in the affected site. He was diagnosed with osteoarthritis in his knee joints about 5 years ago. At first, he went to an internal medicine clinic, and his doctor prescribed some oral medication. However, there was no change after he took the oral medicines. After trekking a mountain, the pain and swelling extended to his lower leg. He was referred to our outpatient clinic. His visual analogue scale was 5 and tenderness was present at the popliteal fossa and the calf. Pain was aggravated when the foot was dorsiflexed. On examination, unilateral grade 1 pitting edema accompanied by slight redness and warmth was noted in his left leg below the knee and the circumference of the left leg was 1 cm greater than right when measured at the calf (). He said the pain and swelling of the leg had been improved since the trekking. There was no motor weakness or sensory change. Weight-bearing posteroanterior plain radiography showed Kellgren-Lawrence grade 1 radiographic alterations bilaterally.
Ultrasonography revealed large hypoechoic space that was divided into two compartments behind MHG. Ultrasound of the popliteal fossa showed the presence of a well-defined cyst connected knee joint. It had different component of a typical popliteal cyst: the base, the neck, and the body located between the tendon of the semimembranosus and the tendon of the MHG (). The body of the cyst was communicated with fluid collection of the calf. In addition, some joint effusion and synovial hypertrophy were seen. Initially, ultrasoundguided puncture aspiration was performed to obtain 10 mL of dark red-colored translucent liquid (). We performed the intralesional injection of corticosteroid (triamcinolone acetonide 20 mg) and used some nonsteroidal anti-inflammatory drug (aceclofenac). A few weeks later, he still complained of calf pain. A follow-up ultrasound revealed a fluid collection in his proximal calf. After re-aspiration of fluid under ultrasound guidance, the pain was relieved.


A 67-year-old Thai woman was diagnosed with endometrium carcinoma stage IC. She underwent total abdominal hysterectomy with bilateral salpingo-oophorectomy followed by pelvic radiation in 2004. The total radiation dose was 50 Gy.
In 2014, she presented with a gradually enlarging mass on the pubic area extending to the mons pubis for the past 2 months. It also was painful on palpation and bled easily. The affected site was within the field of prior radiation. She had a history of loss of appetite and weight loss of 3 kg within 2 months. She denied any history of trauma and was not taking any medication.
Clinical examination revealed a large well-defined firm violaceous plaque, 12 × 25 cm in size, and several bluish nodules on the mons pubis and labia majora, in the shape of a pair of panties (fig. ). Pelvic examination was not performed due to the obstructing mass. There was no palpable lymphadenopathy.
Histopathology showed poorly circumscribed dermal tumours extending to the subcutaneous tissue (fig. ), composed of multiple cords and strands of atypical cells with little mitosis, some forming irregular dissecting anastomosing lumen and papillary projection into the lumen (fig. ). These atypical cells were positive for CD31, but negative for AE1/AE3 and HHV-8.
The diagnosis was radiation-associated angiosarcoma (RAAS). In our patient, the large tumour could not be resected; chemotherapy was started with paclitaxel 70 mg/m2 every 2 weeks. After completing the fifth cycle of paclitaxel, the lesion was markedly decreased in size and the symptoms previously described were also completely resolved.


A 67-year-old male presented to the eye casualty department in December 2008 with a 1-week history of distorted vision in his left eye. His past ocular history was significant in that he had a disciform scar in his right eye leaving him with hand movement vision. He has also been under the care of the medical retinal consultant since January 2008 with a left occult choroidal neovascular membrane for which he had received 5 intravitreal injections of bevacizumab.
His past medical history included peripheral vascular disease, essential hypertension, impaired glucose tolerance, early dementia and depression.
On examination at the casualty department, his visual acuity was 6/38 + 2 in the left eye with microaneurysms at the fovea with minimal oedema. A fundus fluorescein angiogram (FFA) and optical coherence tomography (OCT) were requested, which revealed the following (fig. , fig. ).
The patient was seen in at clinic as a matter of urgency. A decision was made with the patient to start intravitreal ranibizumab due to the presence of only one functioning eye. Bevacizumab was stopped, and treatment was initiated a week later. The patient showed significant improvement after the first 3 injections, and his vision remained stable. Figure shows his OCT image.
The patient received a total of 10 intravitreal ranibizumab injections within the first year, with stable vision at 6/24 (40 letters). However, in May 2010, during routine follow-up, the patient presented to the Age-Related Macular Degeneration (ARMD) clinic complaining of reduced vision. Visual acuity was 6/120 + 4 (8 letters), and his OCT scans are shown in fig. . A repeat FFA was organised immediately (fig. ).
The decision was made to treat the patient with IVTA, and the patient was given a single IVTA injection. The patient was given the IVTA injection in the first week of June 2010. During the subsequent visits, an immediate improvement in vision was noted as well as improvements in his OCT imaging. His visual acuity improved to 6/48 (25 letters) at the first visit and remained stable for the following 2 months. His OCT scan 1 month after IVTA is shown in fig. .
He required no further intravitreal triamcinolone injections, and ranibizumab injections were continued based on OCT appearances on a monthly basis. Since the intravitreal triamcinolone injection, he has received a total of 7 intravitreal ranibizumab injections to the left eye over a 4-year period based on his OCT appearances, with the last injection in October 2013. His vision fluctuates on a monthly basis but it has remained between 6/48 + 3 (28 letters) and 6/30 – 1 (34 letters). OCT images at the 1-, 2-, 3- and 4-year follow-up are shown in fig. .
Currently, the patient is still under follow-up at the ARMD clinics. His right eye shows no signs of RAP and is being monitored with OCT imaging. As the patient had an almost immediate improvement with IVTA injection based on vision and intra-retinal fluid, we are able to confirm the inflammatory nature of RAP and the effects of IVTA.


A 45-year-old male patient visited Chungbuk National University Hospital for extraction of #48 tooth. Radiological and clinical examinations revealed an impacted third molar, a periapical and a periodontal lesion of #47, and an edentulous alveolar ridge on #44 with narrow ridge (). Therefore, surgical extraction of #47 and 48 and implant placement on #44 and 47 with ADD were planned. #47 and 48 teeth were extracted before surgery, and block-type autogenous tooth graft materials were produced in a clinic. For personal reasons, surgery was delayed for six months, and processed materials were stored at −70°C in a freezer. Preoperative computer tomography was performed (). The patient was prepared and sterilely draped for intraoral surgery. Significant narrow ridges on #44 (2 mm) and #47 (4 mm) edentulous sites were noted. Implants were placed on the #44 (diameter 4.0 mm, length of 10 mm; TSIII; Osstem, Seoul, Korea) and #47 area diameter 4.5 mm, length of 10 mm; TSIII). Exposed implant threads were noted due to the narrow ridges (). An autogenous tooth chip was prepared with the bone mill and placed on the buccal side of alveolar ridge. To increase volume, a block type of ADD was transplanted upwards autogenous tooth chip with resorbable collagen membrane (CyADDlast; Osteogenic Biomedical Inc., Lubbock, TX, USA) (). After suturing without tension, antibiotics and anti-inflammatory analgesic were administered and the patient gargled with saline.


A 48-year-old male patient was referred from a private dental clinic for evaluation and removal of a micro-screw, lost during an onlay bone graft procedure with titanium mesh and screws in the lower alveolar ridge. Routine radiographs and CT images revealed a 4-mm-long micro-screw on the right side of sublingual space, in the medial space of the mandibular body (). Under local anesthesia, we attempted removal of the lost material after elevation of the lingual flap from the mandibular medial surface. However, the screw could not be identified and so the removal attempt failed. We assumed the lost screw became repositioned in the intrinsic tongue muscle after penetration through the periosteum. Under general anesthesia, we attempted removal of the foreign body in the sublingual space using C-arm fluoroscopy (Ziehm 8000™; Ziehm Imaging, Nuremberg, Germany). The exact three-dimensional position of the foreign body was defined with guidance of intraoperative real-time image of C-arm. The foreign body was located in the intrinsic tongue muscle, about 5 mm from the inner layer of periosteum (). We successfully removed the foreign body after careful dissection of intrinsic tongue muscle. To prevent postoperative infection, all bone graft materials, including allogenic bone particles, titanium mesh, and screws, were also removed from the alveolar ridge (). The patient experienced uneventful healing without specific complications, including lingual nerve damage or tongue motion limitation.


A 13-year-old girl was seen for complaining of decayed teeth. This was her first dental visit.
On examination, the mandibular right lateral incisors and bilateral central incisors were missing and the left mandibular lateral incisor was decayed. She also had generalized enamel hypoplasia and angle’s class I malocclusion with bimaxillary protrusion. Her tongue was depapilated and ankylosis of the tip of the tongue with the labial frenum of the lower lip was noticed ().
Movements of the tongue were restricted and pronunciations of consonants were affected. Family history was unremarkable. A pediatrician evaluated her general health status and she was found to be suffering from iron deficiency anemia.
After taking detailed history and complete examination, a provisional diagnosis of labial ankyloglossia associated with oligodontia and iron deficiency anemia was considered.
Panoramic radiography confirmed missing of the permanent mandibular central incisors and the right lateral incisor ().
After routine blood investigations, surgical release of the ankyloglossia with “Z” plasty using a 15 no. B.P blade was carried out under local anesthesia and was sutured using vicryl 3-0 suture material. Post treatment healing was uneventful and tongue movement was restored to normalcy (). Iron deficiency anemia was managed by the pediatrician uneventfully.


A 41-year-old female was admitted to our department of gynecology with metrorrhagia. Pelvic ultrasonography revealed bilateral pelvic tumors and a myoma of the uterus. To make a definitive diagnosis of the bilateral pelvic tumors, total hysterectomy with bilateral salpingo-oophorectomy was planned.
At the same time, she was referred to the breast cancer outpatient clinic for bilateral breast pain and swelling. She had no particularly relevant past history or family history. Physical examination revealed diffuse induration with uneven surface and tenderness in the bilateral breast. Although the density of the bilateral breast was high, mass or calcification was not detected by mammography. Ultrasonography showed diffuse mottled hypoechoic lesions in the bilateral mammary glands. Magnetic resonance imaging (MRI) of the right breast showed an extensive enhanced area in the mammary gland () with edema of retromammary adipose tissue and skin in the delayed phase (). Axillary lymph node swelling was also detected by MRI. Core needle biopsy of the right breast revealed diffuse infiltration of signet-ring cells in the mammary gland (), and intracytoplasmic lumens also were seen ().
Because the pathologic examinations of the resected pelvic tumors also revealed signet-ring cells in the bilateral ovaries, suggesting that the tumor metastasized from another site, upper gastrointestinal endoscopy was performed to identify the primary site. Upper gastrointestinal endoscopy showed an ulcerative lesion similar to a Borrmann type 4 tumor in the lower part of the stomach. Pathologic examination of a biopsy specimen from the stomach revealed infiltration of signet-ring cells similar to the infiltration observed in the breasts and ovaries. Immunohistochemical analyses showed positive staining for p53 in the stomach and breast lesions. In addition, staining of MUC5AC and HIK1083, which are specific markers of gastric gland mucin, was also positive in the stomach and breast lesions. Based on these findings, we diagnosed the breast lesions as metastases from the gastric carcinoma.
Combination therapy of S-1 and cisplatin was administered after the diagnosis, and the metastatic tumors in the breasts became impalpable after two cycles of therapy. Further, MRI of the right breast showed neither enhancement nor edematous changes. Although there was no recurrence of breast lesions, the patient died from meningeal dissemination of gastric cancer approximately 10 months later.


A cervical polyp was detected in a 34-year-old female during health screening. Biopsy of the cervical polyp revealed poorly differentiated adenocarcinoma with signet-ring cells. To explore the primary site, a computed tomography scan was performed, which revealed a small nodule enhanced by contrast material in the left mammary gland. She was referred to our department for further examination of the breast.
Physical examination revealed a 1 cm diameter nodule in the upper outer quadrant of the left breast. Mammography showed no abnormal findings. Ultrasonography showed a hypoechoic 1 cm diameter nodule with irregular shape in the upper outer quadrant of the left breast. MRI of the left breast showed a 1 cm diameter nodule that was enhanced by contrast material in the early phase, but the enhancement was washed out in the delayed phase (). The axillary lymph node was not swollen. Core needle biopsy of the left breast tumor revealed diffuse infiltration of signet-ring cells in the mammary gland (), which were estrogen receptor-negative, progesterone receptor-negative, and human epidermal growth factor receptor type 2-negative. The pathologic findings of the breast and cervical lesions suggested that there might be a primary lesion in the stomach. Subsequent upper gastrointestinal endoscopy showed an ulcerative lesion with a Borrmann type 3 appearance on the wall of the middle gastric body. Biopsy of the gastric lesion revealed infiltration of signet-ring cells (). Positive staining of specific markers of gastric gland mucin (HIK1083, GCDFP15, and MUC6) was observed in the breast () and gastric lesions (). Based on these findings, we diagnosed the breast lesions as metastases from the gastric carcinoma.
The patient received combination chemotherapy of S-1 and cisplatin. Her condition has remained stable without progression of the primary tumor in the stomach or of the metastatic tumor in the breast for 7 months.