This was a case report of a 56-year-old Caucasian woman who presented with the chest tightness and discomfort and inability to take a deep breath to the Adnan Menderes University Hospital, Department of Pulmonary Medicine in Aydin, Turkey. Her symptoms started 2 weeks ago with fever, vomiting, coughing, a small amount of purulent sputum and dyspnea in May 2012. She was given levofloxacin 750 mg oral tablet once a day considering the diagnosis of pulmonary infection. Her chest X-ray and CT of the thorax showed bilateral multiple small pulmonary nodules predominantly in the left lower zone. She referred to our department with findings in question. Her medical history was a 10 pack-year of smoking and had undergone cholecystectomy 20 years ago. She had a family history of breast cancer.
Her vital signs on the admission were all normal. The oxygen saturation was 98% on room air. Lung auscultation revealed crackles predominately at the left lung base. An incision scar was observed with an examination of the abdomen. Laboratory studies demonstrated a hemoglobin level of 11.3 g/dL (normal range for female: 11.7-15.5) with microcytic hypochromic anemia, hematocrit of 37.3%, white blood cell count of 14.01/mm3 (normal range: 4.1-11.2/mm3) with a normal differential cell count. The erythrocyte sedimentation rate was 102 mm/h and C reactive protein level was 81 (on admission)/57.5 (1 week later) mg/L (normal range: 0-5 mg/L).
The level of albumin was 3.3 g/dL (normal range: 3.5-5 g/dL), alkalen phosphatase was 212 U/L (on admission)/158 (1 week later) (normal range: 40-150 U/L), γ-glutamyl transpeptidase was 80 U/L (on admission)/56 (1 week later) (normal range: 9-36 U/L), lactate dehydrogenase was 250 U/L (normal range: 125-243 U/L). Other liver and renal function tests were within the normal ranges. The serum levels of tumor markers include CA 15-3, CA 19-9, alpha-fetoprotein, carcinoembryonic antigen were normal. The level of CA-125 was high as 228.7 U/mL (normal range: 0-35 U/mL).
Chest X-ray and thorax CT showed pleural nodularity associated with pleural effusion in the left side of the thorax. A polygonal subpleural nodule with 6 mm in diameter was identified in the posterior part of the right upper lobe []. CT of the abdomen showed several paraaortic lymphadenomegalies reaching maximally about 1.5 cm in diameter and the gallbladder surgically removed.
Because the pneumonia was diagnosed, it has been started sulbactam-ampicillin intravenously four times a day for 1 gram and peroral 500 mg clarithromycin per 2 times a day. Three days after treatment begins, the culture of sputum yields Klebsiella pneumonia that is sensitive to cefazoline and levofloxacine. Treatment was continued without change and completed to ten days.
Oesophagogastroduodenoscopy was performed due to gastric symptoms and it was revealed a mucosal hyperemia with edema of the gastric antrum and some erosive lesions []. The antral erythematous gastritis and erythematous pangastritis were diagnosed. The specimen was negative for Helicobacter pylori. Mucosal biopsies from the lesions of the gastric antrum showed non-caseating granulomas composed of epithelial and multinucleated giant cells []. Nor microorganism was detected with Periodic Acid-Schiff (PAS) ve Ziehl-Neelsen staining nor foreign or phagocytic particles.
Due to suspicion of malignancy for pulmonary nodules, we performed 18F-FDG PET/CT that it is revealed multiple nodules of different size bilaterally in the lower lobes. The nodules showed pathological uptake of 18F-fluoro-2-deoxy-d-glucose (18F-FDG) (The SUV of the larger nodule which is 1.5 cm diameter was 5.5). Multiple mesenteric lymphadenomegaly which are different sizes from the coeliac area, peripancreatic area to the iliac bifurcation were detected with pathological uptake of 18F-FDG (maximum SUVs were 6.6). showed 18F-FDG PET/CT sections which demonstrate pathological uptake of 18F-FDG of the stomach and mesenteric lymphadenopathies.
Thoracotomy was performed due to increase in the probability of malignancy of the lung nodules. Furthermore, bilateral pleural irregularities which had pathological uptake of 18FDG suggested empyema, though it is aimed to clarify. Partial pleural decortication for pleural thickening and wedge resection for lung nodules were performed. The pathological examination of the wedge resection material of the lung revealed non-caseating granulomas including giant cells, accompanying inflammatory cells, including monocytes, macrophages and lymphocytes. Moreover, chronic active inflammation from decortication material of the pleura was found. No specific microorganism was detected with PAS ve Ziehl-Neelsen staining. Tuberculin skin test was 8 mm in diameter and she had 2 scar of Bacille Calmette Guerin vaccine. Sarcoidosis considered in the differential diagnosis and the level of angiotensin-converting enzyme (ACE) was measured. The ACE level was within the normal limits as 48.60 (8-52) U/L.
The evaluation of patient by the gastroenterologist was concluded that the clinical signs, endoscopic and pathologic findings of the patients are not compatible with Crohn's disease. Clinical assessments and 18F-FDG PET/CT examination showed no other organ involvement. Spirometry was normal. It is considered the case is limited sarcoidosis with pulmonary and gastric involvement. Pantoprazole of 40 mg per day, metoclopramide hydrocloride of 10 mg tablet three times per day, sucralfate 2 gr two times per day were recommended for gastric symptoms.
The patient experienced no further symptoms and remained well on proton pump inhibitor (PPI) (pantoprazole) therapy. She experienced gastric symptoms occasionally within 1-year follow-up. Endoscopy was performed again in order to check the findings and no abnormality was found. The biopsy from the gastric antrum revealed non-caseating granulomatous inflammation consistent with sarcoidosis.


The 33-year-old Caucasian female was first admitted to our hospital in 2003. At that time, the patient had a history of two spontaneous abortions followed by amenorrhea and galactorrhea, and also a history of migratory arthralgia of the large joints. She suffered from nonspecific symptoms including lower back pain accompanied by mild fever. Despite a lack of neurological manifestations, the patient underwent several investigations including brain computerized tomography (CT) and MRI, which surprisingly showed a mass-like lesion in the right temporal lobe with cystic and infiltrative portions that partially enhanced following intravenous (IV) gadolinium administration. Additionally, a smaller lesion was detected in the hypothalamus ( and ). Glioma was suspected based on the imaging results, and stereotactic biopsy of the lesion was performed. Histological analyses revealed granular, foamy, periodic acid–Schiff (PAS)-positive macrophages with no signs of tumor. WD was suspected and then confirmed at a referential histopathological laboratory (Bonn, Germany) using PAS staining technique. For WD confirmation, the patient underwent a thorough gastroenterological evaluation including repeated duodenal biopsy; all biopsies were deep; histology was done with correct PAS staining and the specimens examined by experienced pathologists were found to be negative. The diagnosis of primary CNS WD was postulated and was supported by spinal tap, which showed polymerase chain reaction (PCR)-positive results for WD. Antibiotic treatment was initiated, first with IV ceftriaxone 2 g bid for 2 weeks followed by oral trimethoprim–sulfamethoxazole 160/800 mg bid. The fever subsided with treatment; however, the patient still suffered from lower back pain.
In 2005, the patient developed diplopia despite ongoing oral antibiotic treatment. Neurological investigation revealed vertical gaze paresis. Brain MRI was then performed, which showed partial regression of the temporal lesion, no significant change of the hypothalamic lesion, and two new lesions. One small lesion was situated in the right thalamus with discrete, central postgadolinium enhancement (). The second new lesion had an infiltrative appearance and surrounded the cerebral aqueduct in the mesencephalon; discrete dot-like enhancement was also apparent (). Antibiotic treatment remained unchanged and the patient’s symptoms improved after a few days.
In 2006, the patient suffered from recurrent diplopia and paresthesia of the right arm. Vertical gaze palsy again was observed, and additionally, paresis of the left abducens nerve was also detected. Brain MRI showed a new mass-like lesion in the brain stem with small ring-like, central, postgadolinium enhancement ( and ). The lesion previously observed in the right thalamus as well as hypothalamic infiltration had completely regressed. Remarkable regression of the initial mass-like lesion in the right temporal lobe was also noted. Spinal tap was again performed, and although the cerebrospinal fluid (CSF) was PCR-negative for WD, no other cause was found. Antibiotic treatment therefore remained unchanged (oral trimethoprim–sulfamethoxazole 160/800 mg bid).
A few months later, the patient stopped visiting our outpatient clinic and we had no contact with her for 8 years. Her next visit took place in October 2014. At that time, she reported that she had stopped taking antibiotics. She denied any worsening of her neurological status in the previous period and was able to attend work as an office clerk. She still suffered from vertical gaze palsy and sixth cranial nerve paresis on the left side. MRI from November 2014 found no new lesions; the previous lesions had partially regressed. Resumption of antibiotic treatment was suggested but was refused by the patient.
In December 2014, the patient was found unconscious by her family. Upon admission to the hospital, she was severely dehydrated with hyperosmolarity, metabolic acidosis, and hypotension. After rehydration and correction of the acid–base dysbalance, she regained consciousness. Right-sided abducens paresis in addition to her previous ocular palsies and severe cognitive impairment was found. She suffered from short- and long-term memory loss and had retrograde amnesia spanning several months prior to the new episode. MRI showed diffuse brain atrophy and severe atrophy of the right hippocampus with hippocampal sclerosis (). All enhancing lesions on MRI had regressed, only postinflammatory gliosis in the right temporal lobe and a small periaqueductal pseudocyst were found. Oral antibiotic treatment with trimethoprim–sulfamethoxazole 160/800 mg bid was reestablished. Unfortunately, her neurological status thus far (April 2015) remains the same. The patient signed the informed consent and agreed with publishing medical data in scientific literature in anonymous form. This case report was approved by the ethics committee of Na Homolce Hospital, Prague, Czech Republic.


A previously healthy 20-year old male soldier was admitted with fever and painful wounds over his sternum, right leg and left arm since three days. He also had swelling, redness and pain over his left eye. Four days prior he underwent a dental filling procedure after which flurbiprofen was prescribed. One day later he had fever along with reddish skin lesions and painful swelling around his left eye. Within 24 hours, the reddish lesions progressively developed a central black hue without suppuration. He had no history of immunosuppressive disease in the past or any drug intake other than flurbiprofen. On admission he was febrile and physical examination revealed oral aphthous ulcers, left periorbital swelling and redness, and multiple, tender, necrotic lesions surrounded by erythema over the chest, right leg, and right arm. The largest lesion was 5 × 4 cm in diameter. The skin lesions were accompanied by fever till the fourth day of admission [Figures and ]. Investigations revealed leukopenia (910/µL; normal range, 3700 to 9700/µL) with severe neutropenia (neutrophil count, 50/µL; normal range, 2000 to 6700/µL). The hemoglobin level was 13.8 g/dL and the platelet count was 228000/mm3. His inflammatory markers were elevated: C-reactive protein 135 mg/dL (normal range, 0 to 3.5 mg/L) and erythrocyte sedimentation rate 83 mm/hr (normal range, 0 to 10 mm/hr). All other biochemical parameters were in the normal range. Blood and urine cultures, wound aspiration culture and skin biopsy were performed. While the results of cultures were pending antibiotic therapy with piperacillin tazobactam (4.5 g every 8 hours) was initiated. Chest radiography was normal. Transthoracic echocardiographic examination was normal, hence the formation of septic emboli due to endocardial vegetations was ruled out. Orbital magnetic resonance imaging revealed increased diffuse contrast enhancement in the left periorbital soft tissues. Bone marrow biopsy revealed hypocellularity of the marrow without blast cells. Serology for HIV was negative by ELISA. Skin biopsy revealed inflammatory cell infiltration, vascular proliferation and extensive keratinocyte necrosis. Repeated blood and urine cultures remained sterile but wound aspiration cultures yielded P.aeruginosa, sensitive to all antibiotics. Based on the clinical and laboratory inputs, a diagnosis of EG associated with P.aeruginosa was made []. Fever resolved completely after 72 hours of initiation of therapy. Neutropenia resolved by the seventh day of therapy. Two weeks later, skin lesions and periorbital cellulitis healed completely.


An 18-day-old male newborn was admitted with vomiting and failure to thrive. He had a 13-year-old healthy sister and was born from non-consanguineous and healthy parents, after an uneventful pregnancy. Birth weight and length were adequate for gestational age. He showed severe signs of dehydration and malnutrition, generalized skin hyperpigmentation and normal genital development.
Biochemical measurements showed hyponatremia, hyperkalemia and metabolic acidosis. Hormonal evaluation revealed high levels of adrenocorticotropic hormone (ACTH) [2615 pg/mL; reference values (RV): 0-46 pg/mL], and mildly elevated plasma 17-OH progesterone (13.1 ng/mL; RV: 1-7 ng/mL) and androstenedione levels (4.6 ng/mL; RV: 0.6-3.1 ng/mL). Aldosterone was low (20.6 ng/dL; RV: 35-300 ng/dL), active renin high (1170 pg/mL; RV: 1.6-15 pg/mL) and serum cortisol was inadequately normal (11.1 µg/dL; RV: 4.3-23 µg/dL) given the stressful context. Primary adrenal insufficiency due to congenital adrenal hyperplasia (CAH) was suspected. Treatment with hydrocortisone, fludrocortisone and salt was initiated with good response. He was discharged home, maintaining follow-up care in our unit and treatment with good adherence. Throughout infancy he maintained adequate growth, normal development, with no major illnesses. However, his ACTH levels were persistently raised, and 17-OH progesterone and androstenedione markedly low (). Adrenal ultrasonography was normal. At the age of 6 years, these findings prompted further investigation to exclude an abnormal gland development, rather than a steroid biosynthesis defect as was first hypothesized. DNA sequence analysis revealed a hemizygous deletion mutation in NR0B1 gene (c.1292delG), originating a non-functional DAX1 protein. The final diagnosis of congenital adrenal hypoplasia was then made. His mother was identified as a carrier of this mutation and his sister was found to be unaffected.
Delayed onset of puberty and consistently low levels of gonadotropins and testosterone () were observed throughout his preadolescent and adolescent years. This led to the initiation of therapy with testosterone at the age of 14. He is currently 16 years old, has adequate growth and a A3P3G4 Tanner stage with prepubertal testes.


A 27-year-old woman who was 7 months into her pregnancy was referred to the Department of Urinary Surgery at The First Hospital of Jilin University on 3th, April. She had suddenly felt continuously swelling pain on the left side of the waist and abdomen accompanied by fever one day earlier. The pain was alleviated without intervention. She went to her local hospital when the symptoms presented again more aggravated than before. Since CT showed a retroperitoneum mass, she was referred to our hospital.
On admission, her blood pressure was 108/62 mmHg. Physical examination showed left upper-quadrant tenderness, left renal area tenderness and knocking pain. There were no palpable abdominal masses or superficial lymph nodes. No endocrinological disorders were presented. Laboratory findings were as follows: urinalysis showed urine ketone bodies (KET) was 3+; peripheral blood examination showed white blood cell count of 11.24×109/L with a neutrophilic granulocyte percentage of 0.76 and a lymphocytes percentage of 0.11. Blood biochemistry showed a glucose value of 6.93 mmol/L. Coagulation examination showed a PT of 14.4 seconds and a international normalized ration of 1.23. Tumor marker showed neuronspecific enolase at 289.46 ng/mL. The systemic blood concentration of adrenalin, noradrenaline, and dopamine was 10.5 pg/mL, 38.6 pg/mL, and 13.6 pg/mL, respectively. The concentration of adrenocorticotrophic hormone was 5.76. While the concentration of aldosterone (clinostatism) was 13.6 pg/mL, aldosterone (8:00 AM) was 745.03 mmol/L. Twenty-four-hour urinary sample for vanilmandelic acid (VMA) was not possible to collect. All the endocrinological results mentioned above were in the normal range, except for the aldosterone (8:00 AM). Electrocardiography showed sinus tachycardia and brain CT was normal. Renal CT () revealed a large (11.4 cm × 9.4 cm), oval and cyst-solid mixed tumor with slightly indistinct margins and septation in it. It was located in the left retroperitoneum close to the left kidney superiorly, the pancreatic body and tail posteriorly, and the spleen interiorly. In contrast-enhanced scan, solid portion of the tumor suggested enhancement while the cyst portion had no enhancement. There was no clear boundary between the spleen and the tumor, the left kidney and the left kidney vein were pressed and the fat gap around the tumor was turbid.
The possible diagnosis of malignancy and the pressure appearance of the surrounding organs compelled us to remove the tumor surgically. Given the fact that the present patient had no expression of hypertension or other cardiovascular disorders, (while only slight endocrinological abnormalities were present), there was a small chance that the tumor was pheochromocytoma. Therefore, in case it was atypical pheochromocytoma, the necessary preoperative preparation was undertaken. Starting from the three days before surgery, 500-mL voluven and 500-mL saline were given by intravenous injection once a day while 10-mg phenoxybenzamine hydrochloride was administered by mouth three times a day.
Due to the close connection between the tumor and surrounding organs, the tumor cannot be easily resected en bloc. Pancreatic body and tail, left kidney and spleen were removed along with the tumor in order to assure complete resection of the tumor.
Gross examination () of the resected specimens revealed an 11 × 9 × 7.8-cm nodular tumor, the cut surface of the spleen, the pancreas and the left kidney had no apparent changes. There was some adrenal gland tissue on the margin of the tumor. The tumor was solid, soft nature, grey yellow or tawny in color and had a complete capsule. Histopathologically, the tumor turned out to be adrenal ganglioneuroblastoma-intermixed. There was extensive hemorrhage and necrosis with focal calcification inside the tumor tissue. Vessels and nerves were not infiltrated with the tumor cells, likewise the excised pancreas, spleen and kidney tissues. Histologic examination showed neuroblasts and gangliocytes (). Immunohistochemistry showed that CgA, Syn, and NF were positive () while CD99 and Desinin were negative. Because of no effectiveness of follow-up treatment was reported, no adjuvant chemotherapy or radiation was undergone for our patient. Five months after operation, the patient remained healthy and there were no evidence of recurrence and metastasis.


A 39-year-old female presented with reduced muscle strength in the right upper limb was admitted to our hospital in April 2011. Cerebral MRI showed encephalic multiple foci, indicating the possibility of a metastatic tumor. According to the chest CT scan, the patient was diagnosed with right lung carcinoma accompanied by metastases to the mediastinum lymph nodes, both lungs, bone, and brain.
The patient underwent a needle biopsy of the inferior pulmonary focus under CT scanning, and pathological diagnosis confirmed that she had adenocarcinoma. Nevertheless, we could not perform an EGFR mutation test due to the limited size of samples. Considering all the examination data and images, the stage of lung adenocarcinoma in this case was diagnosed as T4N2M1 (stage IV).
The patient refused chemotherapy. Considering that the patient had favorable predictor factors for EGFR-TKI efficacy in NSCLC with unknown EGFR gene status, such as being Asian, having an adenocarcinoma, being female, and being a nonsmoker, the patient received first-line treatment with 250 mg/day gefitinib starting March 1, 2011. Partial response (PR) was detected, and progression-free survival (PFS) lasted for 14 months (). In addition, she received whole-brain radiation therapy with Dt40Gy/20f starting March 3, 2011. From June 22, 2012 to November 27, 2012, the patient received second-line chemotherapy with six cycles of a cisplatin and pemetrexed regimen. Next, she received two cycles of pemetrexed chemotherapy, and the best response was stable disease with PFS lasting for 8 months. As the disease was not improved significantly, she received docetaxel combined with carboplatin for four cycles with the best response of stable disease and PFS of only 3.5 months. Then, the patient was treated with carboplatin plus gemcitabine as fourth-line therapy for two cycles with the result of progressive disease.
At the end of September 2013, the patient’s condition had deteriorated significantly. She had difficulty of moving both lower limbs, especially the right lower limb, gradually leading to an incomplete paralysis. Cervical vertebral MRI showed a metastatic tumor in the cervical vertebral canal that compressed the spinal cord at the second cervical level. After multidisciplinary consultation, the patient refused treatment with surgery and local radiation therapy. Therefore, we chose erlotinib as the fifth-line therapy at the dose of 150 mg/day starting October 10, 2013. After the second administration of an EGFR-TKI, the paraspinal tumor disappeared (), and tumors in both lungs shrank significantly (). The objective response was PR. Additionally, the muscle strength in both upper limbs recovered to degree IV, and that in the lower limbs recovered to degree I after 1 week of erlotinib treatment. After 4 months of erlotinib treatment, the muscle strength had recovered to degree V in the upper limbs and to degrees II and III in the lower limbs.
Because the patient refused a second puncture biopsy, a plasma sample was taken for EGFR mutation detection using the amplification refractory mutation system (ARMS) method on February 28, 2014, and the result was negative. According to the latest follow-up on August 17, 2014, the patient continued taking erlotinib orally.
Written informed consent was obtained from the patient according to the Declaration of Helsinki, and the study protocol was approved by the research ethics committee of the Affiliated Hospital of Academy of Military Medical Sciences, Beijing, People’s Republic of China.


A 14-year-old boy presented with a gradually increasing left-sided painless scrotal swelling for the past year. On clinical examination, there was a left sided scrotal mass measuring 10×4.5 cm. The mass was firm in consistency, occupying the left hemiscrotum. The overlying skin was freely mobile over the mass. Testis could not be palpated separately. A nodule measuring 3×2 cm was palpable at the left inguinal region. No lymphadenopathy was noted in the right inguinal region. Clinically a diagnosis of primary malignant tumor of left testis with inguinal metastasis was made Laboratory investigations revealed high level of serum lactate dehydrogenase (3536 U/L), however; serum levels of -fetoprotein and β-human chorionic gonadotropin were within normal range. A contrast enhanced computed tomography (CECT) scan of abdomen showed a hypodense mass measuring 10×6×2.5 cm in the left paratesticular region with left inguinal lymphadenopathy. However no lombo-aortic lymphadenopathy was revealed. The thoraco-abdomino-pelvic CT scan did not reveal any metastases. Bone marrow aspiration was done, which was negative for infiltration. Left high inguinal orchiectomy was performed. The metastatic nodule in the left high inguinal region was also excised.
On gross examination, the tumor was globular, grey-white and measured 10×6×3 cm. On cut section, the tumor was solid and grey-white in color. Normal testis with intact tunica measuring 1.5×1×0.8 cm was entrapped within the tumor (). Microscopic examination demonstrated a highly cellular tumor composed of fascicles of spindle cells with ovoid hyperchromatic nuclei with blunted ends and containing eosinophilic cytoplasm (). Also seen interspersed were elongated cells with more cytoplasmic eosinophilia (rhabdomyoblasts) (). There were large areas of necrosis. The testis showed infarction and was compressed within the tumor, however was uninvolved. The resected end of the spermatic cord as well as the left inguinal node showed involvement by the tumor. On immunohistochemistry, the tumor cells were positive for desmin and MyoD1 () and negative for smooth muscle actin (SMA), CD34 and S-100. Based on morphological and immunohistochemical features, diagnosis of paratesticular spindle cell RMS with left inguinal nodal involvement was made. The tumor was staged as stage 3 according to the Intergroup Rhabdomyosarcoma Study Group (IRSG) staging system, and as group II according to the IRSG post-surgery pathologic groups. Four cycles of chemotherapy was administered using the VAC regimen (vincristine, actinomycin-D and cyclophosphamide). However the patient’s condition worsened and the patient expired 12 months following initial diagnosis.


A 79-year-old Japanese man suffered from plaque-type psoriasis vulgaris for 8 years and was treated with topical steroids, oral antihistamine, 5-10 mg oral prednisolone daily and 50 mg oral cyclosporine daily at a dermatologic clinic. After he stopped oral prednisolone 3 months earlier, his condition worsened and he finally developed erythroderma. The patient was febrile and had scaly erythema covering most of his body and multiple tense vesicles and bullae on his trunk and extremities []. The blisters measured 5-20 mm in diameter, but did not show an annular arrangement. No mucous membranes were involved.
Histopathological examination of the specimen obtained from a bullous lesion showed a subepidermal blister containing fibrin nets and eosinophils []. Another skin biopsy from an erythematous lesion revealed a subcorneal neutrophilic infiltration forming Munro's microabscess and club-shaped extension of the epidermis.
Indirect immunofluorescence, in which normal human skin was used as a substrate, demonstrated a high titer of circulating IgG autoantibodies against the basement membrane zone (BMZ) (a titer: >1: 160). Indirect immunofluorescence 1M NaCl-split skin revealed circulating IgA and IgG autoantibodies, (both titers; 1: 40) that bound to the epidermal side of the split skin [Figure and ]. Through an ELISA using a BP180 NC16a domain recombinant protein, the index value was found to be 195.95 (normal range: <15).
Immunoblot analysis using normal human epidermal extracts detected circulating IgG autoantibodies against the BP180 antigen []. Interestingly, both IgG and IgA antibodies reacted with the BP180 NC16a domain recombinant protein []. In addition, IgG antibodies reacted with the BP180 C-terminal domain recombinant protein, [] and both IgA and IgG antibodies showed reactivity with the 120-kDa LAD-1 by immunoblot analysis using concentrated HaCaT cell supernatant []. Furthermore, immunoblot analysis using normal human dermal extracts detected IgG antibodies against a 200-kDa antigen (laminin gamma-1) [].
After the patient was treated with oral prednisolone at a dose of 20 mg daily, the number of blisters decreased. No new blisters developed 1 week after initiation of the treatment. Since then, the dose of prednisolone tapered.


A 31-year-old man with no past history of known medical illnesses was admitted to our emergency department because of severe dyspnea and accompanying tachypnea (30 breaths per minute). The patient had never smoked and had no previous history of allergic diseases. On physical examination, there was dullness over the left lung field, and decreased breath sounds on the left lung heard on auscultation. There were no enlarged lymph nodes in the body. Arterial blood gas analysis revealed PO2 80.3 mmHg, PCO2 33.1 mmHg, pH 7.46, and SaO2 96.5%. Laboratory data showed that platelets, white blood cells, and differential cell counts were within the normal range. The patient's erythrocyte sedimentation rate was 70 mm/hour (reference range: 0–9 mm/hour) and C-reactive protein was 27.75 mg/L (reference range: 0–5 mg/L), which together indicated an underlying inflammatory process. Notably, urine glucose was 4+ and HbA1c was 19.4%, confirming underlying diabetes mellitus.
On imaging studies, chest radiographs showed total opacification of the left lung (Figure A). Subsequently, chest computed tomography (CT) revealed total atelectasis of the left lung and a mass-forming lesion at the distal trachea (Figure B, white arrow). However, simultaneous mediastinal or hilar lymphadenopathy was not detected. Examination of the lesion with bronchoscopy revealed an erythematous, swollen, and soft mass approximately 4 cm above the carina (Figure C). We subsequently performed transtracheal biopsy of the lesion; however, we were unable to obtain enough tissue for culture due to the severe clinical condition of the patient during bronchoscopic examination. Bronchial washing culture revealed no microorganisms; however, upon histopathologic examination using Grocott-Gomori's methenamine silver staining (Figure A, black arrows), invasion of the tracheal cartilage by septate hyphae was observed (Figure B, black arrows). In addition, numerous inflammatory cells were recruited to the adjacent tracheal epithelium (Figure B, inset box). The invading hyphae exhibited acute angle branching patterns, indicating possible infection with an Aspergillus species. Based on these findings, a presumptive diagnosis of ATB was established.
During the bronchoscopic examination, we attempted to perform debridement of the lesion via bronchoscopy to maintain airway patency. However, the patient's clinical condition and respiratory symptoms worsened after bronchoscopy necessitating endotracheal intubation and mechanical ventilation (oxygen saturation was 74.8% in spite of maximal degree of supplementary oxygen). Moreover, the likelihood of procedure-associated adverse events (eg, bronchoesophageal fistula) was substantially high considering the severity of the pathologic findings including deep invasion of the Aspergillus species into the cartilage wall and the presence of uncontrolled diabetes. Thus, we decided to treat the patient with voriconazole. On the first day of treatment, 6 mg/kg of voriconazole was administered intravenously for every 12 hours as a loading dose, which was then switched to 400 mg/day of oral voriconazole as a maintenance dose according to the guidelines for treating invasive aspergillosis.
After treatment with voriconazole, the respiratory symptoms of the patient gradually improved without additional invasive management. On the 19th day of medication, follow-up CT and bronchoscopy showed successful resolution of the lesion (Figure C, D). After a month of admission, the patient was discharged without any respiratory sequelae. The patient was treated with voriconazole for a total of 10 weeks, and there has been no evidence of recurrence of disease in this patient at the time of this publication.


A 69-year-old male with underlying hypertension was admitted with a complaint of general weakness started 10 days ago. The patient had been taking irbesartan 150 mg and thiazide 12.5 mg for 3 years. He had 20 pack-year history of smoking. The initial blood pressure was 128/82 mm Hg, the respiratory rate 20 breaths per minute, and the temperature was 36℃. Chest auscultation revealed decreased breathing sound on the left upper area of chest wall.
Laboratory findings were as follows: white blood cell count, 8,190×109/L; hemoglobin, 16.4 g/dL; platelet count, 170×109/L; glucose level, 235 mg/dL; sodium, 141 mEq/L; potassium, 1.7 mEq/L; chloride, 88 mEq/L; lactic dehydrogenase, 863 U/L; D-dimer, 0.08 µg/L; and C-reactive peptide, 2.30 mg/L. Arterial blood gas analysis was pH 7.574, pCO2 50.1 mm Hg, pO2 122 mmHg, HCO3 40 mmol/L, and oxygen saturation was 98.8% suggesting metabolic alkalosis.
The chest radiography taken on admission showed left upper lobe atelectasis and mass like lesion infiltrating the left upper bronchus (). Chest computed tomography (CT) scans revealed about 7×5-cm-sized mass in left upper lobe showing invasion to the main pulmonary arterial trunk (). Biopsy was done with bronchoscopy and the pathology confirmed small cell carcinoma. Positron emission tomography CT showed hepatic and both adrenal gland metastases.
Our primary impression was hyperaldosteronism or pseudohyperaldosteronism according to the lab finding of hypokalemia associated with hypertension. Aldosterone and renin level being normal, hypokalemia with metabolic alkalosis in patient with lung cancer infiltrating left upper lobe suggested Cushing syndrome due to ectopic ACTH secreting tumor. The ACTH level was elevated to 535.61 pg/mL and the cortisol level was elevated to 47.43 µg/dL. The level of cortisol was not suppressed with low dose and high dose dexamethasone suppression test and the 24-hour urinary free cortisol was still elevated to 1,796.93 µg/day. Brain magnetic resonance imaging showed normal pituitary gland and no cerebral metastases. The malignant tissue obtained with bronchoscopy was positive in ACTH marker stain. These finding were consistent with Cushing syndrome caused by ectopic ACTH production (). The patient had no cushingoid appearance such as moon face, skin change or central obesity, and hypokalemia was the only clinical feature to suspect diagnosis.
To reduce the high cortisol level before chemotherapy, ketoconazole (400 mg two times a day) and spironolactone were administered for EAS. As hypokalemia resolved, so did the metabolic alkalosis. It was not needed to maintain corticosteroid treatment after normalization of hypokalemia. Once ectopic ACTH production controlled, the patient was transferred to the oncology division to have systemic chemotherapy and survived for 15 months.


In December 2013, a 65-year-old Saudi woman presented to us with the complaint of painful lump over the occipital region of scalp. She had noticed this lump for 6 months and it was increasing in size over 2 months; she was taking nonsteroidal anti-inflammatory drugs but with minimal improvement. Her previous medical history was not significant, and she denied any history of trauma, smoking, or weight loss. Past surgical history revealed that she had undergone total thyroidectomy and lymph node dissection for follicular variant papillary thyroid carcinoma pT2N1bM0 in June 2006. Postoperative thyroglobulin (TG) was 2.4 ng/mL. Subsequently, the patient was given radioactive iodine ablation 150 mCi in August 2006.
On physical examination, she was found to be in good general condition, and her vitals were stable. Head and neck examination revealed a hard fixed and tender mass of size 4 cm × 4 cm in the occiput without any ulceration of overlying skin, and the rest of the systemic examination was unremarkable. Clinical differential diagnosis was meningioma, sarcoma, giant cell tumor, or metastasis.
Hematology, renal and liver function tests, serum electrolytes, thyroid-stimulating hormone, and thyroxin were found to be within normal limits. Serum TG levels were markedly elevated, ie, 3,064 ng/mL (normal: 5–25 ng/mL). Whole body iodine scintigraphy was nonavid. Head and neck computed tomography (CT) showed a large left occipital bone mass of size 5.7 cm × 1.9 cm with intracranial extension and was extending superiorly to involve the parietal bone. Another intracranial dural-based lesion of size 2.6 cm × 2.3 cm was also found at the top of the splenium of corpus callosum (). Magnetic resonance imaging () and CT–positron emission tomography confirmed the findings of CT imaging. Fine needle aspiration of calvarial lesion confirmed the malignant follicular cells of PTC.
The patient was operated in January 2014; however, surgical attempt for complete resection of both lesions was foiled by perioperative profuse bleeding. Histopathology of the dural lesion revealed follicular pattern with numerous colloid-filled follicles with characteristic nuclear features of papillary carcinoma (enlarged and elongated nuclei and nuclear grooves), and immunopositivity for thyroid transcription factor-1 and cytokeratin 19 confirmed the diagnosis of metastatic follicular variant PTC (). Genetic testing revealed a BRAF V600E mutation. After successful recovery, patient was treated with intensity-modulated radiation therapy. Total dose of 5,400 cGy in 27 fractions over 6 weeks was given to both lesions. The treatment course was completed without any significant acute toxicity. Due to radioactive iodine refractory disease, the patient was started on sorafenib (Nexavar; Onyx Pharmaceuticals, Emeryville, CA, USA, and Bayer Healthcare, Wayne, NJ, USA) 400 mg orally twice daily after the completion of radiation therapy. At 8 months of follow-up period, the patient was doing well with stable skull mass and decreasing pattern of TG levels (37 ng/mL).


A 19-year-old man complained right shoulder pain for three weeks. A week before visit to hospital, he experienced bilateral upper extremity weakness that eventually spread to his lower extremities and resulted in his admission to our hospital via the emergency room. At the time of his admission, his neurological exam revealed quadriparesis, with a motor grade of grade 2 out of 5 for his right upper extremity (UE), grade 3 for his left upper extremity, and grade 4 for his right lower extremity (LE). He exhibited hypesthesia at his UE/LE and an increased deep tendon response at his LE.
The magnetic resonance imaging (MRI) of his cervical spine showed a huge lobulating mass at the right C3-C7 level with an invasion of C5-6 neural foramen. The tumor caused bone erosion in the C4-5 vertebral body. Moreover, the tumor extended into the central canal of the C3-5 level, which caused spinal cord myelopathy. The lesion had relatively low signal intensity on the T2-weighted images and isointense on the T1-weighted images analogous to the muscle signal intensity. In the post-contrast image, the tumor was well-enhanced with heterogeneous signals ().
We performed transfemoral spinal angiography and tumor embolization before surgical treatment. The patient underwent resection of the tumor through the combined anterior-posterior approach. First, to decompress the myelopathy, we used a posterior approach. Epidural mass decompressive tumorectomy through the posterior approach. We performed a C3 to C6 posterior fusion. And second stage operation was performed by subtotal resection of the epidural tumor through the anterolateral approach. The tumor was whitish with a firm mass and was highly vascularized. The adhesion of tumor to the surrounding tissues was moderate. The tumor had enclosed vertebral artery and nerve root. The pathological diagnosis was epithelioid sarcoma. The immune-histochemical study results were positive for beta-catenin, CD99, epithelial membrane antigen (EMA), and vimentin. The negative S-100 staining excluded the diagnosis of a malignant peripheral nerve sheath tumor (). Postoperatively, the patient's symptoms such as weakness and hypesthesia improved gradually. Three weeks after surgical treatment, primary chemotherapy with ifosamide and adriamycin was performed. The patient showed no neurological change, but follow-up MRI after four months showed a newly pathologic compression fracture at C4 that involved large epidural mass formation. The patient underwent third operation that included C4-5 corpectomy and fusion from C3 to C6 (). After last surgical treatment, chemotherapy regimen was changed to gemcitabine/docetaxel, followed by adjuvant radiation therapy. The total dose administered was 4,000 cGy in 30 fractions over 4 weeks. The patient received five courses of chemotherapy in 12 weeks. He was initially stable, but he underwent acute respiratory distress. He died after having survived for 25 months after diagnosis.


A 54-year-old male experienced a sudden onset of headache which had developed 2 days ago. After the initial diagnosis of subarachnoid hemorrhage (SAH) using brain computed tomography (CT) in another hospital, he was transferred to the emergency room where his Glasgow Coma Scale score was E4V5M5. Despite neck stiffness, there was no focal neurological sign. His medical history was non-contributory and laboratory blood tests found no abnormalities.
A brain CT scan taken on admission showed focal SAH concentrated in the anterior portion of the basal cistern (). Digital subtraction angiography (DSA) confirmed the presence of the anterior communicating artery (ACoA) aneurysm and 3D rotational angiography (3DRA) showed that the aneurysm had a very narrow neck, which measured 5.9 × 2.7 × 1.7 (width × height × neck diameter) (). Based on the distribution of the SAH, we considered that the ACoA aneurysm had bled. As morphologic features of the aneurysm were adequate for coil embolization, we decided to perform an emergency endovascular coil embolization on the day of admission.
The embolization procedure was performed with the patients under general anesthesia. DSA was performed again to assure the aneurysm. After confirming that the result on DSA was identical to the previous one, a microcatheter was carefully guided over a microguidewire into the aneurysm. As the tip of the catheter was kept at the neck of the aneurysm, the first coil (Target® Detachable coil 3 mm × 8 cm; Stryker Neurovascular, Fremont, CA, USA) placement was attempted very slowly. At the last moment of deploying the first coil, the tip of the microcatheter was seen to move back from the aneurysm and the projection of the coil loop outside the aneurysm was observed (). Consequently, the first coil was gently withdrawn from the aneurysm. However, a subsequent angiogram showed near obliteration of the aneurysm without evidence of vasospasm. Only a tiny stump at the neck was noted (). Because acute thrombosis facilitated by an exposed coil within the aneurysm was assumed, and, sooner or later, recanalization could occur, we decided to insert a smaller coil into the remaining stump of the neck. With the coil (HyperSoft® 3D complex Coil 2 mm × 4 cm; MicroVention, Tustin, CA, USA), embolization was performed safely and post-embolization DSA showed complete occlusion of the aneurysm (). There was no occurrence of procedure-related complication during coil embolization and the patient recovered without neurologic deficit after the procedure.
Follow-up DSA the next day showed persistent occlusion of the aneurysm (). The patient was discharged without neurologic deficit of the modified Rankin scale score 0. Magnetic resonance (MR) angiography at 3-month follow-up showed no evidence of residual neck or recurrence of recanalization. On 1 year follow-up DSA, despite change in the configuration of the coil, the aneurysm remained to be completely obliterated ().


A 48-year-old male was referred to our hospital due to abnormal brain MRI findings. He had undergone brain MRI for a mild headache without other neurological deficits such as weakness, paresthesia, or aphasia. The patient had no family history of intracranial lesions such as stroke, vascular malformations, or tumors. The initial brain MRI and computed tomography (CT) scan showed a single cystic mass located in the left basal ganglia with a fluid-fluid level and without peri-lesional edema (). Because the initial brain MRI was performed without enhancement at another hospital, an enhancing nodular lesion or peripheral rim enhancement could not be verified, however no nodular lesion or capsule-like appearance was detected using T2-, T1-weighted MRI. There was no evidence of calcification of the lesion in CT and gradient recalled echo T2-weighted imaging. To rule out vascular diseases, cerebral angiography was performed, which did not demonstrate tumor staining or a vascular malformation (). The first repeat brain MRI with Gadolinium enhancement performed 5 months after the initial brain MRI showed no evidence of peripheral or nodular enhancement. A spherical mass with a diameter of 16 mm had enlarged to 25 mm. Suspicious findings consistent with cavernous malformation were observed on axial T2-weighted MRI, which showed a reticulated "salt and pepper" pattern in the core and a peripheral halo of low signal intensity suggesting a hemosiderin rim (). These findings supported a diagnosis of cavernous malformation. The patient's symptoms were not remarkable; therefore, we decided to observe the natural course of the lesion using serial MRI. After 2 months, the patient attended our emergency department with a significant right hemiparesis of 3-hour duration. He was able to move the limbs on his right side through a full range of motion against resistance, but their power was weak. Brain MRI performed immediately showed an increase in the size of the lesion to 35 mm in diameter with heterogeneous internal signal intensity, multi-stage hemorrhage, and aggravated peri-lesional edema (). Based on the imaging findings, the lesion was diagnosed as a CM with recurrent bleeding. A left pterional craniotomy was performed, followed by a trans-sylvian approach, trans-insular cortisectomy for removal of the mass. The margin of the mass was clearly defined and the bulk of the mass had the gross appearance of a hematoma with a yellowish cystic fluid and no encapsulation. A gliotic plane surrounding the mass was observed. There was no active bleeding or evidence of abnormal vessels in the operative field. Intraoperative neuromonitoring included somatosensory evoked potentials (SSEP) and motor evoked potentials (MEP). Median and tibial nerves SSEPs on both sides showed normal values in latency at baseline and no significant interval changes during surgery. MEP decreased by nearly 50% in amplitude at the right extensor digiti minimi during resection of the tumor. Final MEP waveforms after skin closure showed improvement and decreased by < 50% in amplitude and increased < 10% in latency. A neurologic examination performed immediately after surgery showed no significant motor changes compared with the pre-operative examination. After the operation, the patient recovered rapidly from hemiparesis within 2 weeks and was discharged without neurological deficits. The histopathological features were consistent with CM (). After an additional 5 months, follow-up MRI showed no residual lesion () and the patient's neurologic status was normal.


A 42-year-old female presented with a history of chronic iron deficiency anemia and menorrhagia for several years. An ultrasound of the pelvis was requested to determine the cause for menorrhagia. Ultrasound was technically difficult, but demonstrated multiple fibroids [] and a simple cyst within the left ovary. The right ovary was not visualized, but ultrasound demonstrated a complex cystic right “adnexal mass” measuring 9.7 × 5.2 × 6.3 cm that was presumed to originate from the right ovary [].
An MRI was advised for definitive characterization. This confirmed multiple uterine fibroids, T1 bright foci within the left ovary suggestive of an ovarian endometrioma, as well as a dilated left fallopian tube with T1 high signal consistent with a hematosalpinx. The right “adnexal lesion” was only partially visualized on the pelvic MRI, but was deemed to originate from the abdomen rather than the ovary, which was tethered to the uterus []. A gadolinium-enhanced MRI of the abdomen was requested to delineate the mass.
A gadolinium-enhanced MRI of the abdomen was performed 2 weeks later. This study demonstrated a cystic mesenteric lesion with high T2 signal and low T1 signal and measuring 17.6 × 6.8 × 8.7 cm. The lesion insinuated around adjacent vessels within the small bowel mesentery and demonstrated multiple thin internal septations []. No solid components, calcifications, or enhancement were demonstrated. Mass effect was notably absent, and the cisterna chyli and thoracic duct were not enlarged. The lesion was considered to represent a mesenteric lymphangioma.
A 13-month follow-up MRI examination showed that the lymphangioma had increased in size compared to the initial examination, measuring 17.6 × 8.4 × 11.4 cm and extending from the uncinate process of the pancreas to the uterine fundus []. The increase in size prompted a surgical referral. Furthermore, the cystic fluid of the lymphangioma demonstrated signal loss on the T1 out-of-phase sequence compared to the T1 in-phase sequence, consistent with microscopic lipid content. The signal intensity on T1 in-phase was 78.4 and on T1 out-of-phase was 56.2, thus denoting a 28.3% signal intensity loss between phases. This finding is compatible with the presence of chylous fluid and is a very suggestive feature of a lymphangioma [].


A previously healthy 9-year-old girl presented with a solitary non-tender skin-colored 1×1 cm-sized slightly raised papule on right forearm (). The presumptive differential diagnosis included dermatofibroma. Punch biopsy showed a dermal tumor composed of nests of large polygonal cells containing abundant eosinophilic cytoplasm with fine granules, consistent with GCT (). S-100 was positive in tumor cells (). The tumor was totally excised without recurrence for 4 years.


A 26-year-old female complained of a solitary bean-sized brownish nodule on right palm for 3 years (). There had been intermittent tenderness, but no difficulties in movement of palm and fingers. Initially, dermatofibroma and schwannoma were clinically suspected. Histopathology demonstrated a well-circumscribed dermal proliferation of cells with small oval nuclei and abundant pink granular cytoplasm arranged in fascicles (). The tumor cells were stained with S-100 (). The tumor was surgically removed with no evidence of recurrence for 5 years.


A 69-year-old female patient presented with a linear depression on nail plate with matchhead-sized hyperpigmented crust on right middle fingernail (). The lesion was intermittently painful. Initially, digital mucous cyst or glomus tumor was suspected. Histopathology of the nail matrix showed proliferation of round cell having abundant eosinophilic granular cytoplasm, suggesting GCT (). The tumor cells were positive for S-100 (). The tumor was removed by excision.


A 52-year-old female had a pea-sized slightly brownish palpable firm nodule on the right thigh for several months (). Clinically, dermatofibroma or calcinosis cutis was considered as differential diagnosis. Punch biopsy revealed sheets and clusters of large cells with abundant granular eosinophilic cytoplasm and small-to-medium sized central round-to-oval nuclei, consistent with GCT (). Immunohistochemical staining was positive with S-100 in tumor cells (). The remaining tumor was excised without involvement of resection margin.


Two non-tender nodules, each 3×1.5 cm and 1×1 cm sized, had been on the right labium majora of a 56-year-old female patient for 4 years (). She had a history of total laparoscopic hysterectomy due to uterine leiomyoma 6 years ago. Histopathologic evaluation showed a circumscribed tumor, composed of large polygonal cells with abundant granular eosinophilic cytoplasm and uniform-looking round to oval nuclei, forming sheets and nests irregularly infiltrating between collagen bundles, consistent with granular cell tumor (). Both tumors were removed by Mohs micrographic surgery (MMS) and free resection margin was confirmed by frozen section. The cells were positive for S-100 () and periodic acid-Schiff (PAS) stain.


Female 31-year-old patient visited our hospital with pain in buttock that lasted for 4 months. Magnetic resonance (MR) image showed a huge enhancing mass involving the sacrum (). The patient underwent STR, and the pathologic examination revealed to be GCT. On Computed tomography scan (CT) after the operation showed remnant tumor on the ventral side of S2 spinal canal (). Adjuvant treatment was not performed. Four months after the initial operation, MR image showed the progression of the tumor extending to the left side (). The patient underwent GTR. Ninety-four months after re-operation, the tumor did not recur ().


33-year-old male patient complained lower back pain and radiating pain into both legs for 4 months. MR image showed a huge enhancing mass involving the entire sacrum (). STR was performed (). Cyberknife® (Accuray, Inc., Sunnyvale, CA, USA) radiosurgery was done to the remnant tumor located in front of the sacrum (). The patient's pain was relieved and he has been stable for 68 months ().


A 21-year-old pregnant woman with 23 weeks of gestational age was referred to our clinic for advanced ultrasonography and Doppler examination with prediagnosis of fetal anomaly. Her medical history and family history were unproblematic and she did not have any systemic diseases. On obstetric ultrasonography, she had an intrauterine monochorionic monoamniotic twin pregnancy (). Cardiac activity was rhythmic in the first fetus and fetal heart rate was 149 beat per minute and morphometric measurements were consistent with 23 weeks and 4 days, and no anomalies were detected. Fetal cardiac activity was not detected in the rudimentary thorax of the second fetus. Biparietal diameter and head circumference measurements could not be done as the fetal cranium could not be observed. The fetal abdomen was surrounded with a large hydrops ring and the lengths of the extremities were consistent with 20 weeks and 2 days (). Umbilical circulation occurred through reversed perfusion in the recipient twin. On Doppler USG, umbilical artery systolic/diastolic (S/D) ratio increased in the pump twin and within normal ranges in the recipient twin (). Under the light of these data, twin-twin transfusion syndrome and TRAP sequence were considered in the radiologic differential diagnosis of this complicated monochorionic–monoamniotic twin pregnancy. Chorionicity, presence of hydropic, acardiac and acephalic twin and Doppler USG findings were consistent with TRAP sequence.


An 8-year-old female patient visited the university hospital due to delayed eruption of her right permanent maxillary central incisor. Her medical history revealed no systemic diseases, and the dental history proved no facial trauma or other tooth abnormalities have occurred. She avowed that she was operated on, at the age of seven years, for a supernumerary tooth delaying the eruption of the left permanent maxillary central incisor. A previous panoramic radiograph (one year ago) revealed the presence of a mesiodens associated to two supernumerary teeth [].
Clinical examination revealed a mixed dentition and the absence of the right maxillary permanent central and lateral incisors []. The left ones have already erupted.
The patient was referred to CBCT examination of the maxilla to assist the localization and orientation of the mesiodens. CBCT images were requested for diagnosing accurately the morphology and exact location of the mesiodens and the radicular formation of the permanent maxillary central incisors. The images were created and viewed interactively using a dental computed tomography (CT) software program.
The 3D CBCT panorex showed the impaction of the right permanent maxillary incisor and the presence of a supernumerary tooth; moreover, the axial slice image of CBCT revealed the palatal position of the impacted and inverted mesiodens and the sagittal slices showed that the supernumerary tooth was in palatal position over the impacted permanent maxillary central incisor [].
A comprehensive treatment plan was formulated, which included surgical extraction of the mesiodens and the supernumerary tooth []. The patient was administered local anesthesia. Using a mucoperiosteal elevator, a full thickness palatal flap was raised. The supernumerary tooth was exposed, luxated, and removed. Hemostasis was achieved and the flap was replaced back and sutured. For the mesiodens, a simple palatal anteroposterior incision was done and the extraction followed. Postchirurgical instructions were explained to the patient along a prescription of antibiotic and analgesic treatment. The recall visits were scheduled for the following week to remove suture and evaluate the healing process. The patient was kept under the observation until the successful eruption of permanent central and lateral incisors.


A 10-year-old boy presented with a history of diminution of vision left face turn, nystagmus and photophobia of both eyes for the last 7 years. There were no similar complaints in the family members and no history of any trauma or any systemic disease. He had a visual acuity of 20/100 in right eye (RE) and 20/80 in left eye (LE) (Snellen visual acuity chart) and was not improving with correction. His near vision was N6 in both eyes (Times new roman chart). Cycloplegic refraction showed a refractive error of + 1.0 Dioptre Sphere (DS) −1.5 Dioptre cylinder (DC) at 180° in the RE and + 0.5 DS − 1.0 DC at 160° in the LE.
The child adopted a left face turn for both distance and near. Cover test for distance and near, showing a left hypertropia in the forced primary position. A Prism Bar Cover test showed 20 prism diopters (PD) of left hypertropia for distance and 15 PD of left hypertropia for near. He was orthophoric in the preferred head posture. Ocular movements in RE showed a limited elevation in midline and on adduction. Elevation was normal in abduction []. Worth Four Dot Test revealed the fusion for near and distance with the preferred head posture. Stereoacuity with Randot showed a stereopsis of 600 s of arc. A horizontal jerk nystagmus with null point in the right gaze was seen. On slit lamp examination, iris hypopigmentation with transillumination defects was seen with normal pupillary reaction. Indirect ophthalmoscopic examination showed an albinotic fundus with foveal hypoplasia [Figures and , and ]. The foveal hypoplasia was confirmed on optical coherence tomography (Cirrus; Carl Zeiss Meditec, Dublin, CA) []. However a 3-lead visual evoked potentials was not performed.


A 40-year-old male reported to the outpatient department with a chief complaint of ulcerated lesions on the buccal mucosa, gums, and palate since 2–3 years, which progressively increased in size. The medical history revealed persistent dry cough, dyspnea, fatigue, and pain in multiple joints since 2–3 years.
Extraoral examination revealed no significant findings while intraorally multiple, asymptomatic ulcerated lesions on the right and left posterior buccal mucosa measuring 2 cm × 2.5 cm in diameter from third molar to retromolar pad area [Figures and ] and hard palate were observed []. A diffuse erythematous gingival lesion was present on the facial aspect of upper anterior teeth [] with multiple mobile teeth evident in the oral cavity. An orthopantomogram was advised, which showed severe alveolar bone loss []. Incisional biopsy was taken from lesions of the hard palate and sent for histopathological examination.
The patient was referred to a physician for his medical problems, where he was advised hematological, biochemical, and pulmonary function tests along with a chest radiograph.
Evaluation of forced expiratory volume and diffusing capacity appeared to show decreased pulmonary function. Analysis of blood calcium, 24 h urine calcium, and serum angiotensin-converting enzyme revealed higher than normal values [].
Posteroanterior radiographic view of the chest showed bilaterally prominent hilar shadows with soft parahilar opacities [].
Histopathological examination showed numerous non-caseating granulomas which contained epithelioid macrophages and some multinucleated Langhans type giant cells [].
The laboratory tests and histopathological reports facilitated the diagnosis of sarcoidosis. In the present report, the patient was treated with prednisolone 60 mg/day for the management of painful and progressive lesions in consultation with the patient's physician. A planned session of scaling and root planing was performed. Patient was instructed to maintain good oral hygiene with the use of soft toothbrush, tooth paste, and 0.2% chlorhexidine oral rinses. The mobile tooth were splinted and after 3 weeks there was marked clinical improvement and resolution of symptoms of ulcerated lesions on buccal mucosa, gums and palate, reduction in patient discomfort and improvement in masticatory function was seen after 3 weeks.


A 79-year-old male had a large anterior ST segment elevation myocardial infarction (STEMI) due to a single vessel disease (of the left anterior descending coronary artery) and was treated after 4 h with coronary angioplasty and bare metal stent. A 99mTc-tetrofosmin myocardial scintigraphy at rest and an I23I-iobenguane (MIBG) myocardial scintigraphy was performed within 15 days after angioplasty []. The late heart/mediastinum (H/M) ratio was calculated to be 1.32% and the washout rate was 1%. The images demonstrated a large perfusion defect in the anterior wall, apex, infero-apical and latero-apical segments; the Summed Rest Score was calculated as 26; the scintigraphic ejection fraction was 27% with a clear ventricular enlargement (end-diastolic volume 153 ml). The MIBG scintigraphic image showed a severe reduction of the tracer uptake in the anterior wall, apex, infero-apical and latero-apical segments demonstrating, as usual, a larger defect in the sympathetic innervations in comparison with the perfusion defect. The echocardiographic examination confirmed the severe systolic dysfunction (LVEF = 30%) underlying enlargement of LV as well (diastolic volume 143 ml); in the segmental kinesis analysis, there was evidence of an anterior and apical akinesia. At 40-day follow-up after the angioplasty, a transthoracic echocardiogram was repeated which gave a similar value of LVEF (32%) and an unchanged pattern of segmental kinesis, and the patient was diagnosed with NYHA class of II–III CHF, though he was already put on optimal pharmacologic therapy. An ICD was implanted after few days as prescribed by the current guidelines that underline the implantation of an ICD as effective in reducing mortality from ventricular arrhythmias in patients with CHF, classified as NYHA class II or III, and with an LVEF ≤35%.[] He was then re-evaluated some months later by a transthoracic echocardiogram, which showed a stable LVEF of 30%, NYHA class of II–III CHF; the device had not yet delivered any therapy because no repetitive ventricular arrhythmias were registered in those months. ICD in primary prevention usually delivered electric therapy in no more than 5–6% of implanted CHF patients and our case report confirms that a longer follow-up and larger experiences are needed to observe ICD functioning in primary prevention. The interpretation of MIBG scan in our patient helped us to predict the absence of LVEF recovery and, therefore, the need for ICD implantation after the “waiting period” of 40 days following an acute MI as specified in the current guidelines.


A 36-year-old female presented to her primary care physician with progressive right lower quadrant abdominal pain without any significant past medical and family history. Computed tomography (CT) scan of the abdomen and pelvis with contrast showed a 4.9 × 3.5 × 3.1 cm, lobulated, septated cystic mass arising from the cecum. The mass demonstrated wall enhancement with multiple coarse calcifications and ascites inferior to the mass (fig. ). No lymphadenopathy was seen on CT scan. She subsequently underwent surgical resection.
The specimen revealed organ-confined disease with final pathological staging IIA (pT3, pN0, pMx). High-grade mucinous carcinoma with signet-ring cells invaded the muscularis propria into the subserosal adipose tissue (fig. ). The pathological specimen had negative margins and showed no lymphovascular or perineural invasion. Fourteen resected lymph nodes revealed no nodal metastasis (0/14). The cells clustered into various sizes of clusters and gland-like structures at ×40 magnification. At ×400 magnification, the moderately differentiated malignant cells formed classical columnar strips (fig. ). At ×600 magnification, the cells floated within the abundant extracellular mucin pools as either clusters or isolated single cells (fig. ). Molecular study specific for MSI showed the preserved protein expression of MLH1, PMS2, MSH2 and MSH6. MSI by PCR was noted to be stable. Carcinoembryonic antigen was within the normal limit (<0.3 ng/ml).
Due to the patient's young age, early-stage disease, and aggressive high-grade mucinous carcinoma with microsatellite stability, an adjuvant 5-FU-based therapy with oral capecitabine was commenced to prevent recurrent disease. The patient has completed 4 of the planned 8 cycles and has tolerated and responded to the therapy without any significant hematological or nonhematological toxicities.


A 60-year-old female presented with complaints of progressively increasing left flank pain, high-grade fever, and associated burning micturition for the last 15 days. She also complained of weakness. Patient was a known case of diabetes mellitus and on hypoglycemic agents for the last 2 years. On examination, patient was febrile, pale looking, and had tachycardia of 100/min. Abdominal examination showed a large palpable lump in the left lumbar region with severe tenderness associated with inflammatory changes.
The routine investigations showed increased blood sugar level (250 mg/dl), serum creatinine (3 mg/dl), and increased leukocytes. Ultrasound abdomen showed mild dilation of the pelvicalyceal system of the left kidney. The noncontrast CT-kidneys, ureters, and bladder (KUB) suggested Class 3B EPN involving the left kidney with gross peri-renal collection []. Emergency pigtail insertion was done. The discharge was purulent for the next 3–4 days which slowly turned to urinous. Patient's clinical condition improved and the investigations including the serum creatinine and total leukocyte count returned to the base line. The culture report from the drain was negative. The pigtail showed clear urine around one liter. A repeat contrast-enhanced CT (CECT)-KUB was done after 1 week which showed destruction in the upper and lower pole with spared middle pole []. Patient was taken up for open drainage through the left flank incision. Intraoperatively, multiple perinephric pus pockets with completely destroyed upper and lower pole of the kidney were noted []. The middle pole of the kidney showed good parenchyma. Both the upper and lower pole were debrided and left open with a perinephric drain, as there was no intraoperative leakage of urine. Postoperatively, there was continuous urinous drain output around one liter, so a double J stent was placed. The drain output gradually decreased, and the patient was discharged with drain. In the follow-up period at 1-month, the drain output completely stopped and was removed. The stent was removed at 6 weeks postoperatively. The repeat CECT-KUB done at 2 months showed the spared kidney with good parenchyma and normal function [].


A 67-year-old female patient presented with neck pain and stiffness for the past 4 months. The neck pain radiated to both upper limbs and was associated with tingling and numbness. The symptoms had increased in intensity over the past month.
The patient had constitutional symptoms, including loss of appetite, loss of weight (5 kg over a month), evening rise of temperature, and night cries. She was disabled and bedridden due to her severe imbalance while walking. She did not have any history of trauma or coexisting medical illnesses.
On physical examination, there was severe tenderness over the lower cervical spine. Neck movements were painful and grossly restricted. There was no sensory or motor weakness. Reflexes were brisk. Bladder and bowel function was normal. There were no other respiratory or systemic complaints. X-rays of cervical spine showed a kyphotic deformity, destruction of C6 vertebral body with only a thin part of inferior end plate of C6 remaining, and the associated posterolisthesis of C5 over C6 ().
Blood reports showed lymphocytosis with raised erythrocyte sedimentation rate and C-reactive protein values. A magnetic resonance imaging (MRI) showed infective spondylitis of C5/C6 with a pre/paraspinal abscess from C2 to T2 which indented the pharyngeal wall (). A computed tomography scan showed posterolisthesis of C5 over C6 with bony spinal canal stenosis, anterior wedging of C6, erosion of the superior end plate of C5, and prevertebral soft tissue from C3 to C6 ().
A diagnosis of infective spondylodiscitis of C5 and C6 secondary to tuberculosis was made. Although the patient was intact neurologically, there was extensive bone destruction and high grade spondylolisthesis; thus, an operative line of management was preferred.
Since operative management was likely and the MRI was highly suggestive of tuberculous spondylodiscitis, a preoperative biopsy was not considered. The patient was scheduled for anterior decompression and stabilization. The surgery was performed under general anesthesia in the supine position through the right anterior approach. C5 and C6 corpectomy using an anterior cage and cervical plating from C4 to C7 was performed (). Tissues were taken for biopsy and sent for histopathology examination. Postoperative antitubercular treatment was started. The histopathological examination confirmed tuberculosis.
Postoperatively, there was no neurological deficit and patient was given a cervical collar. The anti-tuberculous treatment was continued for 18 months. After 2 years, an MRI was performed, which showed that the disease had been healed (). A radiograph showed solid fusion (). After 3 years, the patient was symptomatically better and did not have any recurrence of the disease.


A 17-year-old male from north-east part of India was presented to us with gradual painless diminution of vision and abnormal eye movements noticed by parents since 2 years of age. He was using power eight diopter myopic glasses since last 5 years for both the eyes. On examination, he had best corrected visual acuity (BCVA) of 20/200 in both the eyes with latent/manifest nystagmus. Slit lamp examination revealed congenital cataract (involving fetal and embryonic nucleus) in both the eyes. Ultrasound biometry showed axial length of 26 mm in both eyes. Diagnosis of high myopia with congenital cataract with manifest/latent nystagmus was made in both the eyes. Preoperative work-up for intraocular power calculation was done. Routine blood investigations like hemoglobin, total leukocyte count, and platelet count were within normal range. After informed consent was taken from the parents, he was posted for lens aspiration under peribulbar block in both the eyes. Right eye lens aspiration was uneventful. Day 1 post-op showed a BCVA of 20/60 and subconjunctival hemorrhage was noted. He was posted for left eye surgery by the same surgeon after 7 days. A peribulbar block using 26 G 12.5 mm needle was given in the left eye after the lower lid retracted manually, and the needle was placed half way between the lateral canthus and the lateral limbus. The needle was advanced in the sagittal plane, parallel to the orbital floor passing under the globe. When the needle tip was judged to be past the equator of the globe, the direction was changed to a point slightly medial (20°) and cephalad (10° upward) to avoid the bony orbital margin. The needle was advanced without any resistance till the hub of the needle was at the depth similar to that of the iris plane. Following negative aspiration, 5 mL of local anesthetic agent (a mixture consisting of equal volumes of 2% lignocaine and 0.75% bupivacaine with hyaluronidase 7.5 IU/mL) was injected. Immediately after giving a peribulbar block, a complete akinesia, subconjunctival hemorrhage, tight eyelids, and stony hard eyeball was noted. Surgery was cancelled, a provisional diagnosis of left eye retrobulbar hemorrhage was made, and he was immediately started on intravenous (IV) mannitol 250 mL (7 mL/kg). Patient was reviewed after an hour. An abaxial proptosis of approximately 7 mm, inferior dystopia with ecchymosis, and subconjunctival hemorrhage was noted ( and B); intraocular pressure (IOP) was 30 mmHg. Lateral canthotomy and inferior cantholysis were performed by our oculoplasty colleague on emergency basis under local infiltration of a mixture of lignocaine 2% and adrenaline (1 in 20,000) and he found it difficult to control the bleed ing. He managed to reduce proptosis to 5 mm and IOP was 22 mmHg. Indirect ophthalmoscopy showed no evidence of intraocular or subretinal bleeding, and central retinal artery pulsations were inducible after giving pressure over eyeball with cotton tip applicator. A pulse dexamethasone therapy for 3 consecutive days, 100 mg in 150 mL of 5% dextrose over 2–3 hours daily, was started. Topical eye drop moxifloxacin 0.5% qid (four times a day) and eye drop prednisolone acetate 1% qid were started. Oral acetazolamide 250 mg qid and topical eye drop timolol 0.5% bid was given to control IOP. Frequent lubrication was given to prevent exposure keratopathy. Due to uncontrolled bleeding, coagulation abnormality was suspected and bleeding time–clotting time was determined immediately, which came to be normal. Proptosis worsened to 8 mm next day. A contrast-enhanced computed tomo graphy (CECT) scan showed inferolateral subperiosteal hematoma in the left eye (). He was posted for drainage of subperiosteal hematoma, but due to strong clinical suspicion of coagulation disorder, prothrombin time (PT) and activated prothrombin time (aPTT) were determined, which were observed to be prolonged, that is, PT was 22 seconds and aPTT was 50 seconds. So drainage of hematoma was cancelled. Fresh frozen plasma was transfused for stabilization of coagulation factor deficiency. After 5 days, coagulation profile came to be within normal range and tarsorrhaphy was performed for exposure keratopathy. Repeat hematology evaluation after 2 weeks detected factor V deficiency. He was diagnosed to have Owren’s disease or parahemophilia.
Our first important differential diagnosis was retrobulbar hemorrhage which was ruled out after CECT scan, and the second differential diagnosis was any coagulopathy which was supported by PT and aPTT and confirmed by coagulation factor assay.
At discharge, he had BCVA of 20/80 in right eye and no perception of light (No PL) in left eye due to compressive optic neuropathy. On 7 days follow-up, there was a healing corneal ulcer of 3×2 mm size, and after 2 weeks of follow-up the ulcer healed completely with No PL in the left eye. Three weeks postoperatively, left eye was undergoing phthisis ().


A 74-year-old male was admitted in the emergency room for chest and epigastric pain radiating to the left arm and lasting for 3 h. He had a known diagnosis of CHS of the right arm with metastasis to the lungs. Following amputation of the right arm, the patient was treated with cytostatic drugs for palliative purposes. He suffered from chronic obstructive pulmonary disease.


A 27-year-old woman with Marfan's syndrome, who had a replacement of her ascending aorta and insertion of a mechanical aortic valve in 2006, presented to a district hospital with hypoxia and acute cardiogenic pulmonary oedema. She had a history of a small stroke, confirmed by CT scan, in 2009 from which she had fully recovered. There was a 2-week history of rhinorrhoea, increasing dyspnoea and central chest pain referred to the back. Over the last two nights, she started to have night sweats but neither fever nor rigors. Her only drug therapy was warfarin and according to the patients' clinic records, the international normalised ratios (INRs) had been within the recommended therapeutic range. Transthoracic echocardiography (TTE) demonstrated a mean transvalvar gradient of 88 mmHg. The patient had been diagnosed as with cardiogenic pulmonary oedema and Furosemide 40 mg was administered intravenously and she was transferred immediately to a tertiary cardiac intensive care unit.
On arrival, the patient was fully conscious but with cardiogenic pulmonary edema. Her respiratory rate was 50 cycles/min, with coarse crepitations heard over all lung zones; arterial saturation was 85–88%. A sinus tachycardia at a rate of 145 beats/min was present and the blood pressure was 109/65. Blood tests showed international normalized ratio (INR) 1.3, activated partial thromboplastin time (APTT) ratio 0.95, Troponin I was 4289 ng/l (n<50), creatinine 51 μmol/l, ALT 115 (n=0–40 U/l) and CRP 67. Full blood count showed Hb 146 g/l, WBC 28.2×109/l (neutrophils 25.4×109/l) and platelets 233×109/l.
A repeated transthoracic echocardiogram was performed with good imaging windows. There appeared to be severely limited excursion of the bileaflet discs with no obvious thrombus or vegetations. The peak gradient across the valve was 128 mmHg and the left ventricle was hyperdynamic and non-dilated but severely impaired. Arterial oxygen saturation was 96% and lactate was 1.4 mmol/l. The chest X-ray showed a picture of pulmonary oedema (). Owing to the degree of respiratory and haemodynamic compromise, transesophageal echocardiography (TEE) was deferred until the patient was anesthetised with a fully prepared theatre team.
The TTE (A, B and C; Videos and ) demonstrates a bileaflet mechanical St Jude prosthesis with severely limited excursion of the leaflets. The differential diagnosis was thrombosis, pannus formation or least likely endocarditis. The history, in particular the acute presentation, suggested prosthetic thrombosis. Furosemide 80 mg was administered intravenously and continuous positive airway pressure was applied via face mask, producing a reduction in both dyspnoea and respiratory rate. Discussion centered around i.v. thrombolysis, but was not performed due to lack of identifiable thrombus and the history of stroke. Blood cultures were taken, i.v. antibiotics and heparin infusion were commenced and emergency surgery was scheduled as soon as a theatre and team could be assembled.
The patient was anesthetised within 4 h. Haemodynamic stability was maintained at induction and a TEE probe was inserted. The images thus obtained did not show thrombus or vegetations, but confirmed severe restriction of the leaflets (A, B and C; Videos , , and ). The TEE confirmed the high transvalvular gradient obtained by TTE. Following this confirmation of the TTE findings, a secondary median sternotomy was performed and division of adhesions, cardiopulmonary bypass was established uneventfully. The St Jude valve was exposed and extensive pannus and granulation tissue were revealed overlying the valve and interfering with the movement of the leaflets. There was no evidence of infection, but a tissue sample was sent for a gram stain. All granulation tissue was removed along with the valve. A 25 mm ATS bileaflet mechanical valve was then inserted and the aorta was closed. The patient was then taken off bypass with a minimal inotropic support and transferred to the intensive care unit (ICU). There were no complications and the patient was discharged home on day 10. None of the samples sent for culture returned any positive microbiological growth.


A 20-year-old male complained of nocturnal pain in his left anterior chest which had persisted for 2 years. Conservative treatment was performed for a period of 22 months with administration of NSAIDs at a nearby hospital. His symptoms were refractory to medication. Therefore, he was referred to our hospital for further treatment. At the initial visit to our hospital, there was no local tenderness, swelling, or redness. A plain radiograph showed bulging and mild bone sclerosis of the left 8th rib (fig. ). CT examinations revealed a well-demarcated 5-mm nidus with peripheral bone sclerosis in this area (fig. ). The bone scintigraphy showed focal uptake of radioisotope only on the left 8th rib. Clinical and radiological findings were compatible with an OO of the left 8th rib. Because the patient refused to undergo en bloc rib resection, the operative plan was to perform resection of the nidus under CT guidance. The operative procedure was performed in the CT room under total anesthesia. After identifying the nidus with a marker, a guide pin was inserted by a power drill just adjacent to the edge of the nidus. A 5.0-mm cannulated drill was inserted over the guide pin to remove the nidus. After removing the lesion, the bone specimen which resided in the cannulated drill was sent for histological study. Subsequently, heat ablation by an electrosurgical knife using a standard electrosurgical generator was performed to completely destroy any residual tumors. Each step was confirmed by CT imaging (fig. ). Histopathological studies showed the characteristic appearance of an OO with differentiated osteoblasts lining the osteoid and interconnected trabeculae of woven bone (fig. ). Complete pain relief associated with the nidus resection was achieved from the first postoperative day. The patient was free of pain at the final follow-up after 4 years, and no local recurrence was observed.


A 7-year-old boy presented to Maxillofacial Surgery Department with the complaint of nontender swelling in right maxillary region since last 6 months. Swelling was beneath the right infraorbital region extending from lateral to ala of nose and going posteriorly till the arch of zygomatic bone. Intraorally, there was an expansion of buccal and palatal cortices from upper right canine to first molar on the same side. Overlying mucosa was of pink color, nonulcerated, and firm on palpation. The patient had squint in right eye since birth [].
Computerized tomography (CT) scan revealed a homogenous mass in the right maxillary antrum region compressing medially, superiorly it was seen abutting floor of orbit, and inferiorly expanding maxillary alveolus from canine to second molar [Figure and ]. Incisional biopsy under local anesthesia was planned, which revealed OM.
Immunohistochemical stains with neuron-specific enolase and vimentin were positive [Figures and ]. The lesion was completely excised with peripheral ostectomy through intraoral buccal vestibule approach considering the age of the patient, and Carnoy's solution was applied under general anesthesia. The patient is still under observation 4 years after surgery and there is no recurrence.


A 50-year-old female reported with complaint of a painless swelling in the anterior maxillary region for a period of 1-year. Initially, the swelling was small and showed a gradual increase to size of its present dimensions. The swelling extended from 13 to 22 causing displacement of teeth, exfoliation of 21, and obliteration of buccal vestibule [].
Extraoral examination revealed diffuse swelling in the upper lip region protruding beyond it. The mucosa over the swelling was of the same color and texture as the surrounding mucosa with no local rise of temperature. On palpation, the swelling had firm consistency. Buccal and palatal cortices were expanded. The radiographic examination revealed a well demarcated multilocular radiolucent lesion with “tennis racket” appearance involving anterior maxilla. CT was performed which showed cortical distension in a buccal and lingual direction that had breached in some region. Incisional biopsy was performed and it revealed myxoma.
Taking into consideration the extent of tumor, anterior partial maxillectomy through intraoral approach using vestibular incision was done extending from 14 to 23 under general anesthesia. A surgical obturator was given to protect the wound. The final obturator was given after 6 months. Histopathological examination of excised specimen showed loose myxomatous tissue alternating with fibrous area and islands of residual bony trabeculae. The patient was followed up for 6 years and has remained disease free.


A 25-year-old young female reported to our department with complaint of appearance of painless lump intraorally in the left posterior region of lower jaw after extraction of left second molar, a month back in a private clinic. On examination, no extraoral swelling was present. Intraorally, a small exuberant growth protruding from the extraction socket of lower left second molar was seen, surrounding area was covered by normal appearing mucosa. A panoramic radiograph revealed the presence of a well demarcated, multilocular radiolucent lesion extending from first molar to third molar on the left side. A biopsy of the lesion was performed which revealed OM []. Aggressive curettage with extraction of 36 and 38 followed by peripheral ostectomy was done intraorally under local anaesthesia (LA), keeping in view the young age of female and literature showing less recurrence in mandible []. Histopathological examination showed unencapsulated tumor mass showing spindle- and stellate-shaped cells in loose connective tissue stroma. Odontogenic epithelial rests were not observed and are not essential for diagnosis []. The healing was uneventful with good amount of bone formation seen radiographically. The patient has been followed up for 7 years with no recurrence.


An 82-year-old woman with a medical history of classic Whipple surgery due to pancreatic adenocarcinoma presented 3 months prior with smouldering acute pancreatitis secondary to an obstructing pancreatic ductal stone (). Attempted endoscopic retrograde pancreatography (ERP) with a paediatric colonoscope (Olympus PCF-PH190, Center Valley, Pennsylvania, USA) failed to reach the biliary and pancreatic anastomoses. Endoscopic ultrasound (EUS)-guided intervention was undertaken. A linear echoendoscope (Olympus GFUCT-180) was positioned in the stomach. Endosonographic signs of chronic pancreatitis were present, with 6 mm pancreatic duct dilation. The wall of the pancreatic duct was punctured transgastrically with a 19-gauge fine-needle aspiration needle (Expect, Boston Scientific, Marlborough, Massachusetts, USA) (). Contrast was instilled under fluoroscopic guidance and a pancreatogram was obtained (). An obstructive stone was identified near the pancreatic anastomosis. A 260 cm 0.035″ hydrophilic guidewire (GLIDEWIRE, Terumo Medical Corporation, Somerset, New Jersey, USA) was passed though the transgastric access into the pancreatic duct and across the surgical pancreaticojejunostomy into the jejunum. Using electrocautery, a needle knife (Clevercut 3V, Olympus) was advanced over the wire to allow passage through the stomach wall and pancreatic parenchyma into the pancreatic duct. After the needle knife was passed into the jejunum (), the wire was exchanged for a stiffer wire (Superstiff Jagwire, Boston Scientific). The surgical pancreaticojejunostomy and the EUS created pancreaticogastrostomy tracts were balloon dilated to 4 mm (Hurricane, Boston Scientific) (). A 7 Fr 10 cm double pigtail stent (Advantix, Boston Scientific) was placed transgastrically across the gastrojejunostomy and pancreaticojejunostomy with the proximal end in the stomach and distal end in the jejunum ( and online supplementary video). The procedure was completed as an outpatient; there were no adverse events and the patient is clinically well 4 months later. The patient was offered additional interventions for stone removal to allow the stent to be removed, but she refused.
ERP is often impossible to perform in patients with post-Whipple procedure anatomy due to inaccessibility to the pancreaticojejunostomy anastomosis. EUS-guided pancreatic drainage, first described in 1995, may be offered in these patients in whom the pancreatic duct cannot be accessed at ERP. It has been used as a platform for access to and drainage of the pancreatic duct either by rendezvous or transmural drainage. However, only one of four patients achieve successful completion of the rendezvous procedure. Even with transmural drainage, mean technical success may not be superior to 82%, possibly due to lack of a dedicated pancreatic stent designed for EUS-guided placement. Transmural drainage may be performed from the gastric
 or jejunum side.
 Even in cases where it is possible to reach the pancreaticojejunal anastomosis, retrograde pancreatic stent placement may be associated with difficulties in creating and dilating the fistula, when using a conventional echoendoscope. In our case, we were able to create a gastro-pancreaticojejunostomy anterogradely, with dilation of both the anastomoses in the same procedure, with subsequently decompression of the pancreatic duct. There are limited data regarding safety and long-term outcome of this procedure, as well as little information on the optimal time for leaving stents in place. We believe definitive endoscopic therapy should be attempted, whenever possible, after relief of obstruction. In the cases where pancreaticojejunal anastomosis can be reached with a colonoscope, a rendezvous stent placement could be considered, without need of creating a larger transgastric tract, which could potentiate a leak between the gastric wall and the pancreas. However, we could not reach the pancreaticojejunal anastomosis. Our option for future treatment would be electrohydraulic lithotripsy assisted by anterograde pancreatoscopy, followed by advancement of the stone fragments into the jejunum through the pancreaticojejunal anastomosis. However, the patient refused any additional interventions. Considering this, we expect that stent occlusion is inevitable, however, long-term drainage may still be possible due to drainage occurring between the stent and the stone.


A 16-year-old male achondroplastic (ASAI, 94 cm, 16 kg) diagnosed with AAD and basilar invasion (cervical collar in situ) was scheduled for transoral odontoidectomy and posterior fusion []. Patient had a history of gradually progressive spastic quadriparesis (3/5 power in all limbs) with restricted neck movement. Computed tomography scan of cervicovertebral junction showed hypoplastic dens and magnetic resonance imaging demonstrated compressive myelopathy of cord [Figures and ]. Patient was unable to perform pulmonary function tests. Chest X-ray showed ribs aligned horizontally with decreased intercostal spaces. Routine lab values, electrocardiography, and transthoracic echocardiography were normal. The patient was counseled for awake fiberoptic intubation and the possibility of postoperative mechanical ventilation. He was prescribed 0.25 mg alprazolam and ranitidine 150 mg at night before surgery and morning of surgery. In the operation theater, after connection of monitors, venous access was secured with two 18 gauge cannulas using AV300 Vein Viewing System. Preinduction arterial cannulation was performed under local anesthesia and ultrasound guidance for the beat to beat invasive blood pressure monitoring and for repeated sampling if needed. Awake fiberoptic intubation was achieved with a flexometallic endotracheal tube (7.5 mm ID) following local anesthetic gargling and airway blocks (superior laryngeal and transtracheal). Injections propofol (30 mg) and rocuronium (20 mg) were administered intravenously to induce sleep and achieve muscular relaxation. The patient was then catheterized with a Foley's catheter. Anesthesia was maintained with air and oxygen (50:50) and propofol infusion with intermittent boluses of rocuronium. Injection fentanyl (50 µg) was used for intraoperative analgesia. Throat packing was done to prevent blood aspiration. Transoral odontoidectomy commenced in the supine position and following completion of the same, throat pack was removed, and hard collar reapplied. The patient was then cautiously turned prone over bolsters and horseshoe headrest, taking utmost care not to disturb the alignment or cause any flexion or extension of the neck. Avoidance of direct pressure on the eyes, padding of dependent areas, and unrestricted abdominal excursions were ensured. Blood gas analysis was done twice intraoperatively to gauge the adequacy of ventilation in the prone position. After posterior fusion was completed, the patient was turned supine (after hard collar application) and a nasogastric tube was placed for enteral feeding. Total time needed for the surgery was 7 h during which 350 ml blood was lost occurred, and urine output was 250 ml. Patient remained hemodynamically stable throughout the intraoperative period. The patient was shifted to the Intensive Care Unit (ICU) for elective postoperative ventilation (pressure-controlled mode). Intravenous dexmedetomidine (0.5 µg/kg/h) was used for sedation during ventilation. Analgesia was maintained with paracetamol infusions (250 mg 6 hourly for 3 days). After overnight ventilation, the patient was safely weaned and extubated the following morning. Enteral nutrition commenced 6 h after extubation after ensuring normal blood gases reports on room air. Postoperative respiratory infections were prevented by broad-spectrum intravenous antibiotics and aggressive physiotherapy and spirometry. Following 2 days of ICU stay, the patient was shifted to ward and 6 days thereafter, was discharged.


An 18-year-old female presented to the Department of Plastic Surgery in surgical super specialty block. History revealed repeated administration of intramuscular injections at bilateral gluteal regions. Clinical examination showed hard mass in bilateral gluteal area. Ultrasound scan of the gluteal areas showed hard lesions with pockets of necrosis within. Biopsy revealed calcified tissue. Patient was further evaluated by dermatologists for connective tissue disorders and muscular dystrophies. Laboratory investigations for rheumatoid arthritis, systemic lupus erythematousus (SLE) were done. Patient's clinical examination, blood investigations for connective tissue disorders and muscular dystrophies were negative.
The lady was posted for bilateral gluteal mass excision and flap cover procedure under anesthesia. Patient's preanesthesia check-up was done a day prior to surgery. Lady gave a history of getting intramuscular injections for fever and body ache from adolescence. Lady weighed 40 kilos and height of 150 cm. General physical examination showed thickened skin and small discrete bony masses beneath the skin in areas like face, elbows, knees and surgical site. Airway examination-mouth opening vertically allowed three fingers. Angles of the mouth were inelastic and allowed three fingers with difficulty. Jaw and neck were normal with a normal range of neck movements. Systemic examination was normal. There was restriction in the movement of the hip joint due to hard mass over gluteals. Fungal infection was present on the back till lumbar region. Anesthesia technique planned was general anesthesia with the endotracheal tube as position needed for surgery was the prone position.
Nil per oral guidelines were advised, and a written informed consent was taken from the patient Tablet Ranitidine 150 mg and Tablet Alpralozam 0.5 mg was advised for the night. On the day of surgery intravenous line was secured in preoperative holdup area and intravenous premedication of injection midazolam 1 mg was administered. Patient was shifted to operation theater and preinduction monitors for pulse oximetry, electrocardiography and noninvasive blood pressure were placed. Premedicated with the injection fentanyl 2 mcg/kg; preoxygenated for 3 min and induced with injection propofol 2.5 mg/kg. After confirming the ease of mask ventilation, injection rocuronium bromide 0.9 mg/kg was administered. Ventilated with air-oxygen mixture and sevoflurane. Laryngoscopy was tried with Macintosh blade 3 at a neuromuscular score of 0 and MAC of 1. Cormack–Lehane view was grade 4. BURP maneuver did not improve the view. Second dose of injection propofol 1 mg/kg was administered, and laryngoscopy was reattempted with MacCoy blade 3. Bougie was used and no. 7.5 Endotracheal tube was placed and secured with tape. Positioning of the tube was confirmed with capnography tracing and auscultation. Patient was positioned in a prone position with adequate padding at pressure points. Intraoperatively patient was maintained on IPPV with isoflurane, air-oxygen and intermitted doses of rocuronium and injection fentanyl.
Surgical duration lasted 3 h. Patient was turned supine and extubated when fully awake. Patient was discharged on 7th post-operative day. Postprocedure follow-up was done and histopathology report showed calcification in the dermis layer. This confirmed the diagnosis of calcinosis cutis.