We report a 43-year-old man who presented to his general practitioner (GP) after 12 hours of fevers, rigors, severe abdominal cramps, nausea, vomiting and diarrhoea. His main complaint was intense epigastric pain radiating through to his back. The GP noted he was centrally cyanosed, tachypnoeic, febrile (39.5°C) and intermittently drowsy, so urgent transfer to hospital by ambulance was arranged. He was promptly attended in the emergency department, where worsening tachypnoea, abdominal pain, and profound central and peripheral cyanosis were noted. His skin was generally mottled and dusky, and there was evolving purple discolouration of his fingers, toes, nose, ears and lips. His extremities were cold to touch. He was distressed and mildly confused. Focused neurological, respiratory and abdominal examinations were otherwise unremarkable. Face mask oxygen was administered, intravenous access was secured, and 3000 mL intravenous crystalloid was given within the first 2 hours. Blood cultures were taken by separate venipuncture, but only after he had received his first dose of intravenous antibiotics. Parenteral opioid analgesia was titrated to relieve pain and distress.
Significant medical history included hereditary spherocytosis with splenectomy in childhood. He had maintained prophylactic immunisation but did not take regular antibiotics. He suffered a right-sided precentral gyrus infarct (stroke) in early adulthood, after which an incidental patent foramen ovale was detected. Rehabilitation was complete, with no residual neurological deficits.
Initial blood results demonstrated marked neutrophilia with bands, acute kidney injury and DIC. Liver function tests were mildly elevated, and serum lipase was normal (). Chest X-ray and 12-lead ECG were both unremarkable. CT of his abdomen was reported as showing bilateral perinephric stranding and pancreatic inflammation. Microbiology samples of blood and urine were sent. Pneumococcal DNA was detected in blood via PCR, and antigen for S. pneumoniae was detected in urine.
Following the first 1000 mL intravenous crystalloid bolus, the patient’s blood pressure incremented slightly and his mental state improved. However, the prolonged central capillary refill time, mottled skin, and deep cyanosis of his ears, nose, lips, hands and feet visibly worsened, despite further volume resuscitation. A radial arterial line was inserted for haemodynamic monitoring and blood gas analysis, revealing a high anion gap metabolic acidosis, elevated lactate and respiratory alkalosis, all typical of early sepsis ().
Despite haemodynamic stability, ongoing clinical concern warranted admission to intensive care unit (ICU), where central venous access was inserted. A transthoracic echocardiogram revealed a well-filled heart with a normal systolic ejection fraction and no valvular lesions. ‘Normal’ function, however, was considered inadequate owing to the unimproved peripheral circulatory signs, persistent lactic acidosis and now established anuria, despite adequate blood pressure. Milrinone was commenced at 0.25 µg/kg/min, without bolus, to improve peripheral perfusion and augment cardiac output. Over the next 20 min, his ears, nose, lips and central capillary refill all visibly improved, and urine appeared. Despite easily palpable radial and dorsalis pedis pulses (also audible on Doppler ultrasound), both hands and feet remained cold and were now numb. His digits were symmetrically purple-black. Over the next few hours, this numbness was gradually replaced by pain and paraesthesiae, particularly in the fingers.
In the absence of active bleeding, the DIC was not treated with clotting factors or plasma products owing to concerns these could increase the risk of microvascular thrombi in his digits and potentially worsen the situation.
Early referrals were made to several specialty teams, including vascular surgeons, to consider the optimal management of the profound digital ischaemia. The treatments recommended, and those provided, are detailed in .
The patient was competent to provide consent for his own care throughout his illness, although in hindsight he recognised his ‘thoughts were clouded’ in the first 24 hours of his admission. He was most concerned about any intervention that might increase his risk of another stroke.
Catheter-directed, intra-arterial thrombolysis was discussed and dismissed. There is no consensus on agent or dosage, and the risk of inadvertent systemic thrombolysis is unknown in the setting of concurrent administration into multiple vessels in the presence of DIC. He was willing to accept systemic anticoagulation with unfractionated heparin, as this is commonly used in ICUs and can be rapidly reversed with protamine. The theoretical therapeutic intent was thrombus prevention. This was started 16 hours after ICU admission, using a standard protocol.
The patient reported feeling an instant benefit with intravenous heparin. He said that his fingers felt warmer and started to regain feelings, although more painful ones. Objectively, there was little improvement. The visible zones of colour demarcation were not appreciably different, and the digits remained cold to touch. The infusion was continued and monitored by serial activated partial thromboplastin time (aPTT).
Glyceryl trinitrate (GTN) paste was applied to his hands but was soon removed because of severe headache, which then resolved. Subsequent systemic administration of intravenous GTN provided no obvious benefit, or side effects, but it was continued, nevertheless.
A right-sided axillary plexus block with lignocaine, epinephrine and clonidine provided temporary and welcome analgesia to the right hand. The block was technically successful in that the arm lost motor function and his wrist and forearm were appreciably warmer to touch, but there was no visible indication of circulatory improvement in the hand. It was thus not repeated on the left side. Additional concern over possible cumulative local anaesthetic toxicity was another factor in that decision. The motor block lasted over 8 hours.
Over the next 72 hours, there was a steady and marked improvement in both the appearance and function of all four limbs, more so in his hands than his feet. This seems to be the usual pattern reported in other cases. Milrinone was ceased after 24 hours, GTN was continued for 68 hours, and heparin was continued for 72 hours. Gabapentin and oxycodone made his pain bearable. At the time of ICU discharge, the patient’s fingers were no longer purple, but they were still dusky and swollen (). Gangrenous demarcation was evident in both feet ().
Topical application of alcohol and betadine-soaked dressings began under the surgical team’s direction after transfer to the ward. He was provided hand therapy by an allied health team and daily review by an acute pain service.


A 75-year-old woman, presenting with recurrent nausea and vomiting for several months, was admitted to the Department of Gastroenterology in our hospital on 15 March 2018 with the preliminary diagnosis of chronic gastritis. A few months ago, the patient developed nausea and vomiting after catching a cold. The vomit was gastric contents, no black stool, no fever, dizziness and headache occurred. Gastroscopy in the local hospital showed chronic gastritis, then treated by acid suppression and gastric care medicine, but vomiting relief was not obvious, so she came to our hospital clinic and was admitted to the ward. Since the onset of the disease, the weight loss of the patient has more than 5 kg. Cholecystectomy was performed 20 years ago, and right breast mastectomy for breast cancer 9 years ago. The patient had complained of polydipsia and polyuria since birth. She drank 5 to 6 L of water per day, and her urine output was large as well. She denies recent history of using renal injury agents. Her family history showed her brothers and sisters have the same symptoms of polydipsia and polyuria, but her brothers’ symptoms are more serious than her sisters. Her younger brother died of unknown reason and one of her nephews died of infection. Because of her seniority and siblings sharing the same symptoms, she had not paid attention to the disease, and received no diagnosis or treatment.
On examination, she had a blood pressure of 130/100 mm Hg, regular pulse rate of 110 beats/min, and body temperature of 36.7°C. There were no other abnormal findings on physical examination. Laboratory data were as follows: white blood cells 8.85 × 109/L, red blood cells 3.93×1012/L, platelet count 284x109/L, hemoglobin 122 g/L, serum liver and renal functions were normal. Serum tumor markers including AFP, CEA, CA125, 153, 199, 724, SCC-Ag and total β-hCG were normal. Thyroid and adrenal function were normal. Serum sodium and chloride levels were increased above normal values (159.6 mmol/L and 117.4 mmol/L, respectively), with an effective osmolality of 307 mOSM/kg (normal range: 275–295 mOSM/kg). Urine pH was 5.0, urine-specific gravity was 1.003 with a decreased urine osmolality level of 83 mOSM/kg (normal range: 150–1150 mOSM/kg), whereas urine creatinine and electrolyte levels were normal. Fluid intake volume was 3840 ml/day and urinary volume was 6200 mL/day. Electrocardiogram (ECG) suggested sinus tachycardia and no abnormality in cardiac function was found by echocardiography. Computed tomography (CT)-scan of the head suggested mild lacunar cerebral infarction. And CT angiography in the head and neck showed mild arteriosclerosis. Magnetic resonance imaging (MRI) of the pituitary was normal. Positron emission tomography computed tomography (PET-CT) image did not show any sign of tumor recurrence and metastasis. Based on the clinical symptoms and laboratory tests, diabetes insipidus was suspected. Combined with the family analysis (Fig. ), the primary diagnosis of X-linked hereditary NDI was made. In order to make a molecular diagnosis, DNA sequencing analysis was carried out on samples from the patient, her 3 first-degree relatives (her brother, sister and son), 2 second-degree relatives (her niece and nephew) and one third-degree relative (her grandnephew, who is her niece's son). Her other relatives refused to have genetic testing, therefore they were not included.
Direct sequencing analysis of the antidiuretic hormone arginine vasopressin receptor 2 (AVPR2) identified the novel missense mutation (c.492G>G/C) in exon 2, leading to a p. Trp164Cys (T164C) amino acid substitution in the patient. There was a heterozygous mutation in the patient's sister and niece (Fig. A), while there was a mutation in her sons, brother and nephews (Fig. B), and her grandnephew is normal (Fig. C). The locus is located on the X chromosome Xq28, and its mutation can lead to X linked recessive NDI. The p. Trp164Cys mutation of AVPR2 gene has not been reported in literature before. The possible effect of the T164C mutation was predicted to be probably damaging by several prediction methods, including SIFT[ and PolyPhen-2.[ There was no significant abnormal variation in other detection regions of the gene. Also there was also no abnormal variation in AVP and AQP2 genes in this family.
Then we treated the patient with desmopressin (DDAVP), antiemetic drugs, and massive infusion glucose transfusion. The patient's urine volume decreased, about 3 to 4 L per day, then electrolyte disturbance was corrected, and the symptoms of nausea and vomiting gradually disappeared. The electrolyte results of the re-examination showed that the serum sodium and chloride levels were 141.9 mmol/L and 103.3 mmol/L respectively, which were in the range of normal values.
Six months after discharging from hospital, we followed up the patient by telephone. The patient was generally in good condition, the electrolytes were normal, no nausea and vomiting occurred, and her weight increased by 4 kg.


We saw this 34-year-old Indian lady in late February 2017. She had been in her usual state of health till 20 years of age when she had an episode of facial weakness (? side) which resolved over 1 ½ months. She was then asymptomatic, got married and she went to the USA at the age of 25. For the next 7 years, she did not have any symptoms. 3 years before presentation (in March 2014), she had a transient episode of slurring of speech, which resolved in less than 24 hours. In September 2015, she developed bilateral lower limb swelling and erythema with a maculopapular rash which resolved in a few days. In November 2015, when she visited India, she had tingling and numbness of both lower limbs, bilateral painful swollen legs, maculopapular rash below knees (treated by a local practitioner with oral corticosteroids). Rash and swelling resolved in 2 days but paresthesias persisted. She was continued on oral steroids for the next few weeks before she left back to USA and steroids were stopped.
In March 2016, she developed high grade fever of 104°F, accompanied with right lower limb weakness and worsening of lower limb paresthesias. She was admitted and evaluated in a teaching hospital overseas. The following data were extracted from the documents from the treating hospital. Clinically she was alert, oriented and her speech, language and cranial nerves were normal. She had right foot drop with bilateral ankle hyporeflexia with normal upper limb and knee reflexes. At this point of time she was investigated extensively. MRI of the brain was done which showed multifocal lesions in both supratentorial and infratentorial compartments; predominantly in deep and subcortical white matter. These lesions were predominantly hyperintense on T2 and FLAIR images; some of the lesions contained foci of hypointensities on susceptibility-weighted images suggesting hemorrhages and some had a peripheral rim of diffusion restriction. Minimal perilesional edema was seen []. CSF examination revealed pleocytosis with raised protein. She had pancytopenia and anti-nuclear antibody was positive with a speckled pattern. The investigations done at this point of time are enumerated in .
She also underwent bone marrow, lung, brain and lymph node biopsies. However, no definite conclusion could be drawn. As patient and relatives wanted definite diagnosis, no empirical treatment was started.
In the subsequent months, she worsened progressively. In October 2016, she fell down at home, had a fracture of the right femur, developed progressive cognitive decline and was confined to bed. She was placed in a nursing care facility. By December 2016, due to progressive cognitive decline she became dependent for activities of daily living and personal hygiene. By the end of January 2017, over one month, she became incontinent with irrelevant talking and reduced oral intake. She was shifted to India in an unconscious state. When she presented to this hospital, she was E1V1M2, had upper GI bleed in Ryle's tube aspirate and she succumbed in less than 24 hours of admission. After consent from caregivers, her autopsy was done.


Case 1. A primigravida woman aged 26 years at 26 weeks of gestation was admitted to the division of maternal–fetal medicine. FGR with disturbed uteroplacental and umbilical hemodynamics was diagnosed via ultrasound. No maternal comorbidities were noted. Diastolic flow in the umbilical artery (UA) was absent. A pulsatile index >95th percentile and an absent a-wave in the ductus venosus (DV) were noted. Pulsations of the umbilical vein (UV) were detected. The conventional CTG pattern was non-reactive. A course of betamethasone was initiated. NI-FECG tracing was obtained from the maternal abdominal wall using Cardiolab Babycard equipment (the KhAI Medica Scientific Research Centre, Kharkiv, Ukraine) []. The sampling rate was 500 Hz. The study protocol was approved by the Bioethics Committee of the Kharkiv Medical Academy of Postgraduate Education (registration No. 0105U002865). The NI-FECG data was rather surprising. The tracing of beat-to-beat variations (an analog of the ultrasound CTG curve) demonstrated a reactive pattern in the nonstress test and normal LTV (=34.4 ms) and STV (=6.0 ms) variables (). The levels of HRV markers of fetal distress were 943 (SI) and 66% (AMo). These descriptors were normal. The calculation of the fABAS gave a value of 0.03 standard deviations (SDs) of weeks of gestation below the mean of the normal population from the Jena Study Centre. This value indicated that the autonomic maturation was within the normal range. Observation and fetal monitoring were prolonged to 30 weeks of gestation. A male baby weighing 820 g, 35 cm in length, with a 26-cm head circumference and Apgar score of 5→6 was delivered via cesarean section. The newborn was passed to the neonatal resuscitation unit and was discharged from the hospital 36 days later.


Case 2. A pregnant woman aged 30 years was admitted to the division of maternal–fetal medicine at 26 weeks of gestation. She had attended irregular antenatal visits, which revealed no abnormalities. She had a blood pressure of 140/90, a pulse of 82 per minute, and unremarkable laboratory findings with a trace of proteinuria in urinalysis. She had no prodromes suggestive of arterial hypertension. She had mild pre-eclampsia and early-onset FGR. No maternal medical diseases were known. The reversed diastolic component in the UA, absent a-wave in the DV, and UV pulsations were found. Non-reactivity was detected on the CTG record, but the fetal heart rate was normal. An investigation of beat-to-beat variations using NI-FECG demonstrated fetal non-reactivity with a decreased value of STV (=1.2 ms) and a lack of accelerations. Two decelerations were found on this tracing. The HRV variables were also pathological: SI, 3102; AMo, 85%. The fABAS value of 3.89 SD of weeks of gestation below the mean of the normal population indicated a clear reduction in autonomic maturation/activity outside the normal range. Fetal antenatal death occurred the next day. Labor was induced. She delivered a stillborn male baby weighing 410 g, 27 cm in body length, and with a 19-cm head circumference.


Case 3. A 26-year-old pregnant woman was admitted to the department of maternal–fetal medicine at 26 weeks of gestation. Early FGR, reversed diastolic umbilical blood UA flow, reversed DV a-wave, and UV pulsations were found. Conventional CTG tracing was non-reactive. No further clinical or instrumental pathological findings were detected in this case. An assessment of fetal HRV by NI-FECG revealed non-reactivity with low STV (=1.8 ms) and lack of accelerations. The HRV descriptors were pathological: SI, 2104; AMo, 81%. The estimated value of the fABAS was 3.85 SD of weeks of gestation below the mean of the normal population, which indicated fetal autonomic malfunction. Furthermore, antenatal fetal death occurred in several days. She delivered a stillborn male baby weighing 440 g, 29 cm in body length, and with a 19-cm head circumference.


A 50-year-old female presented at the orthopedic out-patient department with progressively increasing painless swelling in the right arm for a year. Clinically, the swelling was bony hard and non-mobile. Radiograph of the right arm showed an eccentric, lytic and expansile lesion with thin shell in the mid-diaphyseal region of right humerus []. Subsequently the MRI showed cortical-based expansile lesion with heterogeneously hyperintense soft tissue component on fat-suppressed sequences with blood-fluid levels and extensive surrounding soft tissue and bone marrow oedema.


A 27-year-old female presented with gradually progressive painless swelling in the left forearm of 6 months’ duration. Radiograph of the forearm showed a cortical-based expansile lytic lesion with a thin shell located in the diaphysis of the ulna []. On MRI, the lesion showed heterogeneously hyperintense soft tissue component on T2WI and STIR sequences with a single large blood-fluid level. Extensive marrow and soft tissue oedema were present around the tumour.


A 30-year-old female presented with swelling in the right arm for 8 months. Though painless, the patient complained of an increase in size and associated cosmetic deformity. The radiograph showed an expansile, lytic lesion, eccentrically located in the mid-humeral diaphysis. The MRI showed heterogeneously hyperintense soft tissue on T2WI and STIR sequences with the predominant solid soft tissue component admixed with cystic areas having small blood-fluid levels. Akin to the above two cases, soft tissue and marrow oedema were characteristically present [ and histopathology in ].


A 16-year-old female presented with progressive insidious onset swelling in the right forearm for 1-year duration. Clinically the lesion was painless and bony hard. Radiograph of the right forearm showed an expansile lytic lesion with a thin cortical shell in the radial diaphysis. The MRI for further characterization revealed a large soft tissue component with few blood-fluid levels as well as extensive bone marrow. Soft tissue oedema was also present [].


A 24-year-old female patient visited the Department of Conservative Dentistry with the complaints of swelling, recurrent pus discharge, and discoloration of the upper left incisor. A local dental clinic referred her to our specialist clinic due to a root anomaly. Clinical examination of the maxillary left lateral incisor showed grayish discoloration, insensitivity to percussion, and no mobility. The palato-gingival groove was examined, and the periodontal probing depth was within the normal range. Six days before presentation, a coronal access cavity into the pulp chamber had been prepared and sealed with temporary material at the local clinic (). Periapical X-ray of tooth #22 showed a periapical lesion with a diameter of approximately 8 mm and Oehlers' type II DI (). The patient had no history of trauma, and her medical history was noncontributory. Tooth #22 was diagnosed with pulp necrosis and chronic apical abscess. In addition, a periapical x-ray revealed DI of the maxillary central and lateral incisors bilaterally (). The anatomic form of tooth #22 was similar to that of tooth #12. Whereas both maxillary lateral incisors had lingual pits, the maxillary central incisors had no anatomic variations (). Generalized pulp stones were identified in the bilateral maxillary canines, first and second premolars, and in the bilateral mandibular incisors, canines, and first premolars by panoramic and periapical X-rays (). Tooth #46, which was previously treated with a root canal and restored with a full-coverage gold crown, was diagnosed with chronic apical periodontitis. Except for teeth #22 and #46, all teeth responded to an electrical pulp test and showed normal periapical status. Root canal treatment of tooth #22 was planned, followed by non-vital tooth bleaching.
The temporary filling material in the access cavity was removed, revealing a foul odor from the pulp chamber. After cleaning the coronal access, a DG-16 endodontic explorer (Hu-Friedy Mfg Co., Chicago, IL, USA) was used to locate the orifices. An intracanal-invaginated mass was removed with ultrasonic tips (ProUltra Endo tip, Dentsply Tulsa Dental, Tulsa, OK, USA) () under an operating microscope. Thereafter, patency of the root canal was achieved, and working length was determined using a size 15 K-file (Mani, Tochigi, Japan) (). The root canal was enlarged to an apical size of #50 and copiously irrigated with 3.5% NaOCl via a side-vented 27G needle. Calcium hydroxide dressing was used during the 1-week inter-appointment period (). The root canal was obturated with gutta-percha and AH 26 sealer (Dentsply DeTrey GmbH, Konstanz, Germany) using the continuous wave of condensation technique (). After resin-modified glass ionomer was applied upon the root canal filling material, non-vital bleaching was performed using 3% hydrogen peroxide and sodium perborate (). The patient returned every week to change the bleaching agent. After 2 visits, there was a drastic change in the tooth color. The cavity was irrigated, and the bleaching agent was washed out. At 10 days later, the cavity was restored with composite resin. The patient was regularly followed to check for color stability and complications ( and ). After 13 months of follow up, a periapical radiograph showed that the periapical lesion in tooth #22 had decreased in size and showed no sign of external cervical resorption ().


A 35-year-old woman with a crusted erythematous lesion on her scalp referred to Razi hospital affiliated to Tehran University of Medical Sciences (Tehran, Iran) in September 2016. She had the lesion for 2 years and a previous biopsy indicated PV. In addition, the direct immunofluorescence (DIF) study had been negative. Regrettably, her past medical information was not available and the last biopsy sample could not be reviewed. She had been treated with an over-the-counter topical cream.
Her medical history and general physical examination were unremarkable. She also did not smoke nor consumed alcohol. There was no history of other dermatologic
or autoimmune disorders among her family members. Physical examination revealed a 4-cm diameter lesion that appeared as scaly, erythematous, crusted plaque,
and alopecia, which was confined to the parietal scalp region (). Since the lesion did not respond to the topical
treatment and enlarged, a second biopsy with differential diagnosis of pemphigus vulgaris, pemphigus foliaceus, erosive pustular dermatosis, and cicatricial
pemphigoid was conducted. The histological examination revealed atypical cell proliferation through the whole thickness of the epidermis and follicular epithelium,
with dermal microinvasion along with acantholysis and clear cell formation (with pagetoid features). The tumor cells showed high nuclear pleomorphism, hyperchromatic nuclei,
increased number of mitotic figures, and negative DIF (, ).
The neoplastic cells were positive for cytokeratin 7 (CK7) and negative for carcinoembryonic antigen (CEA), cytokeratin 20 (CK20), and S100 protein.
The patient underwent total lesion excision (1 cm margin) with the diagnosis of acantholytic and pagetoid subtypes of BD and dermal microinvasion.
A subsequent histological study confirmed the initial diagnosis and a negative resection margin. A follow-up in September 2018 showed no recurrence
of the lesion. Written informed consent was obtained from the patient for the publication of her case report.


A 35-year-old woman presented to the outpatient department with complaints of bilateral nasal obstruction for 1 month associated with intermittent blood stained nasal discharge. The patient also gave history of swelling over the dorsum of the nose which developed rapidly over 1 month. There was no history of headache, facial pain, or visual disturbance. There was history of similar swelling in the past over the same site for which she had undergone a minor surgery 5 years back at a different hospital and the records of the biopsy were untraceable.
On examination, there was a firm, fixed, and well-defined swelling of size 2 × 2 cm over the root of the nose in the midline. On nasal endoscopy, left nasal cavity showed a fleshy mass filling the entire cavity pushing the septum to right lateral nasal wall.
Contrast-enhanced computed tomography of the nose and paranasal sinuses showed a heterogeneously enhancing soft-tissue density over root of the nose with erosion of nasal bone and extension into left nasal cavity and filling left maxillary sinus, anterior, and posterior ethmoid sinuses and extending into right nasal cavity with erosion of bony septum (. There was no skull base or lamina papyracea erosion or any enlarged neck nodes. Fine-needle aspiration of the external nasal swelling and biopsy from the nasal mass showed scattered pleomorphic epithelial cells having hyperchromatic nuclei and scanty cytoplasm with 1 to 2 mitotic figures per high-power field, surrounded by abundant basophilic chondromyxoid stroma suggestive of malignant chondroid syringoma. Patient underwent whole-body positron emission tomography–computed tomography to rule out metastasis in lungs, liver, and skeletal system which failed to show any uptake in the site other than nose and paranasal sinus region.
Patient underwent excision of the mass by external approach (lateral rhinotomy incision) along with left medial maxillectomy and excision of the external subcutaneous component (). Postoperative biopsy confirmed the diagnosis of malignant chondroid syringoma. Patient received intensity-modulated radiotherapy 60 Gy/30 fractions, 4 weeks after the surgery. Patient was followed up every month in the first 6 months with diagnostic rigid nasal endoscopy, followed by 6 monthly interval for 2 years and found to have no further recurrence.


A 44-year-old male patient reported to the dental outpatient department with the chief complaint of ulceration and burning sensation in the oral mucosa for 5 days. Patient reported that he noticed multiple erythematous sites intraorally which are associated with pain which aggravates on mastication and gets relieved after sweet food. The patient reported that intra-oral erythematous sites exacerbate and gets healed by its own for the past 6 months. There was no other history of sensory loss, intraoral burns, pus discharge, or bleeding from the affected areas. There was no history of any prior medical illness before 2011. The patient is under HAART therapy since 2011. He was married and had one son who was unaffected, but his wife was also suffering from HIV infection since 2014. The patient gave a history of tobacco chewing 4–5 times/day for 20 years. On general examination, temperature afebrile, pulse 80/min, blood pressure 105/80 mmHg, and respiratory rate 15/min. The patient was well oriented to time, place, and person. Built was lean and gait was normal. On extra-oral examination, the face was bilaterally symmetrical, lips were competent. Deep fissures, cracks with inflammation, redness were present at the angle of the mouth and corners of the lips suggestive of angular cheilitis []. On temporomandibular joint palpation, there was no clicking or crepitus present. The right submandibular lymph node was palpable of size 1 cm × 1.5 cm approximately, shape was roughly oval, firm, mobile, and nontender on palpation. There were no other cervical lymph nodes palpable.
On intra-oral examination, there was complete depapilation of tongue suggestive of the bald tongue []. Multiple white papular areas were present on the posterior one-third and the left buccal mucosa. White radiating lines were seen on the upper right and left buccal mucosa with intense erythematous surrounding areas suggestive of erosive lichen planus [Figures and ]. Generalized gingival erythema was present.
Treatment was to stop the habit followed by oral prophylaxis. Medications advised were candid mouth paint four times daily for 7 days, liveril syrup two tablespoon twice daily for 7 days and patient was recalled after 1 week.
The patient came after 7 days and said that he had 80% relief from the previous signs and he could eat food after the treatment. He got complete relief from angular cheilitis []. There was also reduced erythema on the right and left buccal mucosa.


A 37-year-old healthy lady was referred for left eye (OS) upper lid ptosis correction with history of gradually progressive upper lid drooping for 2 years and, an episode of subconjunctival hemorrhage. There was no history of trauma, allergy, diurnal variation or generalized weakness. Best-corrected visual acuity was 20/20 in both eyes (OU). External ocular examination revealed significant blepharoptosis in OS []. Eversion of the upper lid revealed a yellowish-pink sub-conjunctival lesion throughout the length of the upper edge of the tarsus []. Anterior segment evaluation of right eye (OD) showed a yellowish-pink subconjunctival mass near the caruncle, extending into medial bulbar conjunctiva and inferior fornix [Fig. and ]. Fundus evaluation was unremarkable.


A 60-year-old lady presented with sudden onset diminution of vision in her right eye one month ago. She could appreciate hand movements close to face in the right eye and best corrected visual acuity (BCVA) was 20/80 in the left eye. Intraocular pressure was 20 mm of Hg in her right eye and 16mm of Hg in left eye. Slit-lamp examination of the right eye showed two oval shaped, elevated sub conjunctival lesions at 10 o' clock position 3mm posterior to limbus. The lesions were jet black in color []. Conjunctiva over the lesion was freely mobile with associated dilated episcleral vessels. Anterior chamber and iris were normal. Posterior sub capsular cataract (PSC) with fine pigments over the anterior capsule was noted. Gonioscopic examination of the right eye showed pigmented angles. There was no evidence of distortion of the angles or neovascularization. Posterior segment examination revealed dense pigment clumps in vitreous cavity. Fundus details were not seen. Ultrasound biomicroscopy (UBM) showed a heterogenous mass in the ciliary body in superotemporal region extending from 7 to 11 o'clock hour []. Ultrasonography of the right eye showed high reflective dot echoes in vitreous cavity []. On magnetic resonance imaging (MRI), the lesion was hyperintense on T1, hypointense on T2 and showed contrast enhancement []. Abdominal ultrasonography, liver function tests and chest X-ray were normal. Incisional biopsy of the sub conjunctival pigmented lesion was performed. Histopathological examination revealed melanin pigment. Melanin bleach failed to identify cellular elements. 3 weeks later, slight increase in size of the sub conjunctival pigmented lesion was noted []. On repeat incisional biopsy, sheets of densely pigmented cells were seen in an eosinophilic stromal background [Fig. and ]. On melanin bleach, cells were small, round to polygonal and bland appearing with scant cytoplasm, without prominent nucleoli. No malignant cells were seen. Clinical evaluation, imaging and histopathological examination were suggestive of a ciliary body melanocytoma with extra scleral extension and vitreous seeding. In view of PSC and dense vitreous seeding, phacoemulsification, intraocular lens implantation and 23 gauge pars plana vitrectomy was performed. On scleral indentation, a darkly pigmented ciliary body mass was noted in the superotemporal quadrant. Histopathological examination of the vitreous sample revealed pigmented cells arranged in clusters on a clean background. On melanin bleach features were consistent with melanocytoma and negative for atypical cells []. 3 months post-operatively, patient had a BCVA of 20/40 in right eye. Pigment clump was seen over the disc and fovea []. No change in the size of the lesion was noted.


A 36-year-old male presented with complaints of blurring of vision and distortion of images in the right eye since 4 weeks. His best-corrected visual acuity in both eyes was 6/6, N6. Anterior segment examination of both eyes was normal. Fundus examination of the right eye showed an irregular brownish, elevated mass lesion over the disc and the papillomacular bundle and a U-shaped extension below the disc, measuring 3–4 disc diameter vertically and 2–3 disc diameter horizontally. There was subretinal fluid around the lesion, extending to the fovea []. The extent of the lesion was better appreciated with infrared reflectance imaging []. OCT of the lesion showed a bi-lobed lesion with a upper elevated lesion over the disc []. Ultrasonography showed a dome-shaped lesion located over the disc with high surface reflectivity and moderate internal reflectivity measuring 3.04 mm horizontally, 3.9 mm vertically, and thickness of 2.24 mm. According to the American Joint Committee on Cancer staging manual, the tumor stage was T1aN0M0.[] OCTA was performed with swept-source OCTA (Topcon DRI OCT Triton; Topcon Corporation, Japan) 6 × 6 mm protocol over the tumor. Automated segmentation showed segmentation artifacts which could be overcome by manual segmentation [Fig. and ]. OCT angiograph over the lesion demonstrated large retinal vessel over the tumor and the tumor intrinsic vasculature. The intrinsic choroidal vasculature was seen as fine interlacing vessels [Fig. 2c]. Magnetic resonance imaging with contrast showed an elevated lesion over the disc which was hyperintense on T1 and hypointense on T2 with minimal contrast enhancement. The tumor was treated with a ruthenium-106 (Ru-106) COB notched plaque (Eckert and Ziegler BEBIG GmbH, Berlin, Germany), tumor free margin of 2 mm, delivering 8500 cGy radiation to tumor apex for 5 days. The notch diameter was 5 mm and was adequate enough to accommodate the optic nerve. Intraoperatively, the adequacy of coverage of the plaque was confirmed by transillumination. The treatment was in accordance with the American Brachytherapy Society consensus guidelines for plaque brachytherapy of uveal melanoma.[] On follow-up visits, at 3 and 6 months, OCTA was repeated over the tumor. The serial OCTA showed significant reduction in the density and caliber of interlacing choroidal vessels [Fig. –]. Fundus FA and indocyanine green angiography (ICGA) was done at 6 months of follow-up to assess the vascularity of the tumor [Fig. and ].


A 65-year-old man visited the emergency department with issues such as dizziness, melena, and hematemesis. Two years previously, he was diagnosed with gallbladder cancer, and he underwent extended cholecystectomy. Emergent esophagogastroduodenoscopy (EGD) showed a crater-like ulcer with massive spurting bleeding at the duodenal bulb. Endoscopic hemostasis could not be achieved. Trans-arterial angiography was urgently performed, and a significant contrast extravasation in the proximal portion of the gastroduodenal artery was observed (). The active bleeding was managed with TAE (). He became stable without any additional bleeding or immediate complications occurring. However, EGD performed three days after TAE indicated a duodenal ulcer and a metal object impacted at the duodenal surface (). Based on these findings, coil migration was highly suspected. He underwent aggressive proton pump inhibitor therapy and was discharged without adverse events 10 days after TAE. There was no change in coil position as observed on the abdominal X-ray at the time of discharge (). However, five days after discharge, he presented to the emergency department with massive hematemesis and cardiac arrest. Plain radiography revealed that the coil originally present in the gastroduodenal artery had migrated to the small bowel and colon (). Although intense resuscitative efforts were made and emergent angiography was planned, the patient eventually died.


This is a case of a 27-year-old female who presented with 3 months of loss of appetite associated with mild abdominal distension and a sense of fullness in the right upper quadrant. She had no significant past medical history, with a cigarette smoking habit of consuming one pack per year. On physical examination, no icterus was noted. Mild distention in the right superior quadrant of the abdomen was recognized, a tender and smooth-surfaced mass was identified by liver palpation. Routine clinical investigation revealed a carcinoembryonic antigen of 6.5. All other haematological and biochemical parameters were within the reference range. An abdominal contrast-enhanced computed tomography (CT) showed an ill-defined contrast-enhancing, multilobulated cystic lesion involving segments II, III, VI, VIII measuring 19 × 15 × 10 cm, containing adipose tissue with calcifications and multiple septations (). These findings were suggestive of teratoma. Beta human chorionic gonadotropin and Echinococcus serology were negative. Intraoperatively, a well-defined cyst was confirmed occupying segments IV, V, VIII and partially segments II and III; a mesohepatectomy and cholecystectomy were performed () with two 15-minute pedicular clampages (Pringle manoeuvre). The right and left hepatic veins were exposed. Due to compression and distortion of the structures, inadvertent opening of the anterior sectorial biliary branch occurred. As a small amount of parenchyma was still draining through this duct, a reconstruction via a Roux-en-Y Hepatico-Yeyunostomy was performed. The patient had an uneventful postoperative evolution, dispatched on day eight. Histopathology reported a surgical specimen with a weight of 1837 g with the dimensions 21 × 18 × 12 cm. The outer surface was smooth and yellowish-grey in colour. The cystic wall had a pattern of irregular thickness, with a maximum thickness of 2 cm with calcified multilobulated areas, containing yellowish sebum paste and hair, the diagnosis was mature cystic teratoma (MCT) (). In subsequent follow-up, patient reintegrated actively in her socio-laboral environment.


A 26-year-old male with a medical history of asthma and food allergy to nuts and legumes presented to the emergency department with pain and a non-pruritic erythematous skin eruption on the dorsum of both hands for three days prior to admission. On the day prior to admission, he developed large bullae over his middle and ring fingers. Prior to the onset of his symptoms, the patient was performing outdoor activities on a sunny day, mostly squeezing lime. The patient denied any fever, constitutional symptoms or similar experience in the past. His vital signs were stable. A physical examination revealed two continuous, tense bullae measuring ~3×3 cm along the dorsal aspect of the second and third digits of the right hand (). There were no other identifiable skin lesions elsewhere. The patient had a normal x-ray of his right hand, and laboratory studies revealed a normal erythrocyte sedimentation rate, C-reactive protein, complete blood count, comprehensive metabolic panel and creatinine kinase level. Rheumatologic work-up was non-diagnostic including a negative serology for anti-nuclear antibody. The patient was evaluated by a dermatologist who made a final diagnosis of phytophotodermatitis secondary to lime exposure based on the clinical appearance of the lesion as it had a linear gravity pattern of pigmentation—a characteristic of phytophotodermatitis. The patient’s condition was treated with a local application of cold water, and he was advised to follow up as an outpatient. On the outpatient follow-up, his hand blisters were drained, and he was prescribed a short course of tetracycline for primary prevention of infection. The lesion ultimately healed well without complication.


Patient 1, a 30-year-old female, had a BMI of 44. She underwent endoscopic intragastric balloon inflation in 2015, ESG in 2016, and then repeated in 2017. Patient 2, a 32-year-old female, had a BMI of 51 kg/m2. She, similarly, underwent repeated ESGs in 2016 and 2017. Repeat procedure was performed due to poor weight control and persistent metabolic derangement in the second patient.
The endoscopic treatments were performed by the same endoscopist, and it involved performing gastroscopy during which three rows of full-thickness sutures were placed along the greater curvature of the stomach using the Overstitch (Apollo Endosurgery) and additional four mattress sutures were placed to re-enforce the suture line. Further sutures were used to capstone the stitches within the fundus. The resultant gastric volume was described to be 25% of pre-ESG size. All procedures were reported to have no immediate complications. Patients were discharged on the same day. Both were instructed to remain fasted for 2 days during which they received IV fluid infusions in an outpatient clinic. Clear fluids were then permitted for 3 days, followed by free fluid diet for a month.


On January 29, 2018, a 57-year-old female patient presented with symptoms of an upper respiratory tract infection, which were manifesting as cough and fever (up to 38°C).
Since the onset of the disease, the patient showed the following symptoms: Gradually changing sputum color, back pain, limb weakness, difficulty swallowing, coughing blood, wheezing, chest pain, and stool abnormality. Nevertheless, she was in good spirits, was in good health, was sleeping well, and had no substantial alterations in weight or physical manifestations on the upper face.
The patient had no history of hypertension, diabetes, nerve disease, cerebrovascular disease, neuropsychiatric disease, hepatitis, tuberculosis, malaria, mental process, trauma, blood transfusion, or food or drug allergies, and the history of vaccination was unknown.
The patient had no epidemic exposure and no exposure to chemical, radioactive, and toxic substances. The patient had no history of drug abuse, smoking, or drinking. The patient’s parents are still alive, and the investigation found that there was no family genetic history of lung cancer.
The patient’s body temperature was 37°C, pulse was 60/min, respiratory rate was 20/min, and blood pressure was 120/80 mmHg. There was no yellow staining, rash, subcutaneous bleeding, subcutaneous nodules, or scar ulcers. In addition, there were no masses in the superficial lymph nodes or eyelid edema. The patient had a normal conjunctiva, no yellow staining in the sclera, normal pupil, no abnormal secretions in the external auditory canal, no tenderness in the mastoid handling, and a normal sense of olfactory perception. At the time of admission, the patient had a normal gingiva and oral mucosa. The tongue color was normal, and the patient did not have enlarged tonsils or thyroid gland. In addition, there were no deformities in the chest, no veins in the chest wall, and no tapping pain in the breastbone. Respiratory movement, intercostal space, and tremor were normal. The patient had normal breathing, obvious respiratory sounds in both lungs, no dry or wet sounds, and no pleural friction sounds.
There was no uplift in the precordial region, the apical pulsatile position was normal, the heart rate was 60 beats/min, the heart sound was powerful and steady, the murmur was not heard in each periodical auscultation area, and there was no pericardial friction. The abdominal area of the patient was soft; the patient had no abdominal wall varicose veins, no tenderness, and no rebound pain. The liver and spleen were not touched, Murphy’s sign was negative, there was no mobile voiced sound, and there was no immediate pain in the retinal area. Bowel sounds were normal at 4 times/min.
Hematoxylin and eosin staining was performed on the tumor tissue, and microscopic observation showed that the tumor presented with multiple micronodules, which were separated by abundant lymphocyte interstitium and formed lymphoid follicles. No necrotic structures were found in the tumor tissue, and tumor cells infiltrating the surrounding adipose tissue could be caught at the boundary of the neoplasm. Residual thymic tissue was observed around the adipose tissue. The neoplastic cells showed marked heteromorphic structures with nucleoli and mitotic figures in some areas (1 to 9/10 high power fields [HPF] at the high power field). The staining of different areas of the tumor is shown in Figure .
Immune status of T lymphocytes and B lymphocytes in the tumor was detected by the EnVision immunohistochemical method[], and the results showed that there was strongly positive expression of CD5, CD117, pancytokeratin (CK-pan) (AE1-AE3), cytokeratin 19, and B-cell lymphoma-2 (Bcl-2) in tumor tissues. The rate of Ki-67 positive cells reached up to 30%. Human epidermal growth factor receptor 2 (HER2) and P53 were negative.
The expression of CD20 and CD3 in stromal lymphocytes was positive, the positive rate of CD20 was high, and lymphoid follicle formation was also noted. Terminal deoxynucleotidyl transferase (TdT)-positive immature lymphocytes were not found in tumor tissues (Figure , Table ).
The patient’s computed tomography (CT) image is shown in Figure . The tumor was found to be a solid mass in the anterior mediastinum with pericardial invasion. However, there was adhesion between the tumor and pericardium, but at that stage, there was no definite pericardial invasion and the tumor should be classified as Masaoka stage IIb. The lump was completely resected under thoracoscopy, and the patient was not treated with radiotherapy or chemotherapy postoperatively. The nodular lump measured 5.5 cm × 5 cm × 1.8 cm in size. The tumor was encapsulated with a clear margin. The hybrid section was solid, grayish-white, grayish-brown, and somewhat textured. There was no clear border between the tumor and the surrounding adipose tissue (Figure ).


A Caucasoid female patient, aged 21 years [], presented to the periodontics clinic, with the main complaint of poor esthetic condition and dentin hypersensitivity due to multiple Miller Class I and II[] gingival recessions [Figure –]. The gingival recessions were associated with thin gingival biotype and history of orthodontic therapy as well as traumatic brushing.
Initially, the patient was submitted to scaling and root planing, oral hygiene instructions, and occlusal adjustments. Instructions to use brush with ultra-soft bristles and a low abrasive dentifrice were made. Initially, treatment with SCTG associated with coronally advanced flap was proposed. However, the patient refused a bilateral palatal approach to remove sufficient quantity of tissue. As a result, the proposed procedure was the use of a tissue substitute, the ADM (AlloDerm®-LifeCell-The Woodlands, TX) associated with coronally advanced flap involving maxillary incisors, canines, and premolars []. The surgical technique was performed by modified interproximal incisions in the papilla (surgical papilla design) and marginal intrasulcular incisions. The anatomical interproximal papilla was de-epithelialized for posterior accommodation of the flap when coronally displaced. The partial-thickness flap surpassed the mucogingival junction acquiring a passive coronal mobility of the flap when coronally positioned. Regularization of the root surfaces was performed using a conical diamond bur at high speed with constant irrigation []. Biomodification of the root was performed by root scaling with Gracey manual curettes [] followed by conditioning [] with citric acid and tetracycline (pH 1) applied for 3 min.
The ADM (Alloderm®-LifeCell-The Woodlands, TX, USA) was adapted according to the shape of width and length of the receptor site []. According to the manufacturer's instructions, before adapted at receptor site, the matrix should be hydrated by a duplicate cleaning process with sterile saline for at least 10 min. After that, matrix was stabilized at the CEJ [], and the flap was coronally positioned covering the entire matrix and stabilized by interrupted absorbable sutures [] (Vycril 4.0) above the CEJ.
The patient received postoperative instructions and was advised to use antibiotic (amoxicillin 500 mg every 8 h for 7 days) and anti-inflammatory (nimesulide 100 mg every 12 h for 3 days), as well as mouthwashes with 0.12% chlorhexidine gluconate (2× per day for 10 days), avoiding brushing and flossing in the region during the postoperative period.
The postoperative follow-up and maintenance control of the patient were performed weekly for 90 days; after that period, the patient did not return for personal reasons. Her control was eventually done only 2 and 12 years after the surgical intervention.
The surgical area presented slight exposure of the matrix associated with localized inflammatory areas after 15 days [Figure –]. After 2-year follow-up, the area showed an expressive gain of gingival width, complete root coverage in the anterior area and partial coverage for premolars. A substantial improvement in root coverage (“creeping attachment”)[] was demonstrated with satisfactory gingival tissue texture and color [Figure –]. At the 12-year follow-up, minor areas of relapse of gingival recession and some interproximal tissue loss were observed, but in general with stability of the outcomes achieved [Figure –]. The patient reported traumatic brushing and restorative procedures during this period [Figure –]. In addition, the patient did not attend the maintenance care controls after 90 days postoperatively, only 2-and 12-year follow-ups after the surgical procedure, not reporting the reasons for missing the recalls.
Tables and present the clinical results at baseline and at 2- and 12-year follow-up. A formula to access the percentage of root coverage (% RC) and gain or loss of keratinized mucosa (% KM) was adopted according to Zucchelli and De Sanctis.[]
This case report comprised the use of ADM associated with coronally advanced flap, and the results evidenced root coverage of the multiple gingival recessions with stability of the clinical outcomes achieved. At 12-year follow-up, the root coverage remained satisfactory, despite small areas of interproximal loss and small increase in gingival recession for posterior upper teeth on the right side [].


We report an unusual case of 33-year-old woman, without chronic diseases, 6 months after childbirth, who was admitted to our clinic because of the edema of the right orbit and upper eyelid with headaches of 1-month duration. On physical examination, the patient had a right superior and lateral swelling of the eyelid and the orbit and right enophthalmos, without blurred vision.
On admission, magnetic resonance imaging (MRI) showed well-defined, expansile, intensely homogeneously enhancing mass lesion in the right superolateral orbital rim with destruction of the upper wall of the orbit, growing into the frontal part of the cranium with the bold of the dura mater in this region [-].
Radical excision of the tumor was achieved through a right fronto-temporo-orbito-zygomatic craniotomy.
On histopathology, sheets of histiocytic cells with indented pale nuclei with nuclear grooves suggestive of LCH were seen. Positive immunohistochemical staining for S-100, CD1a, and CD68 confirmed the diagnosis. Ki67 was about 30%.
The whole-body scintigraphy showed the increased collection of the tag in the region of the right orbit, but the postoperative MRI showed no features of the recurrence. We do not decide to use the radio- or chemotherapy in this case. One year after treatment, there is still no recurrence, yet.


A 38-year-old male suffering from alcoholism visited our outpatient department complaining of repeated dizziness and a buzzing sound in the left ear. His first ear symptom was a hearing difficulty commencing 1 year prior. Dizziness and a buzzing sound developed when he turned his head to the right, commencing a few months prior. Sometimes, the dizziness was so severe that he could not open his eyes. Then, he failed to maintain balance and fell, losing consciousness for 3–4 seconds. On examination, we found that the symptoms appeared only when he turned the head to the right, whether he was standing, sitting, or lying. No symptoms were evident when the head was rotated to the left or in the neutral position. A down-beat nystagmus appeared when the head turned to the right.
We used VNG to determine whether the problem was peripheral or central. VNG revealed right-beating nystagmus with a down-beat component when the head was turned to the right (), indicating a central rather than a peripheral origin. We scheduled brain and neck CTA, brain MRI, and three-position DSA to evaluate central origin. CTA revealed a hypoplastic right VA and left VA stenosis at the lateral curvature of the VA at C2 transverse foramen. () Brain MRI revealed a tiny, acute ischemic infarction in the right cerebellum. () DSA was performed in three positions (with the head neutral, 90° to the left, and 90° to the right). In the neutral position, left VA stenosis was observed at the C2 transverse foramen, as on CTA. Notably, when the head was turned to the left, the VA stenotic lesion became wider than in the neutral position. When the head was turned to the right, the blood flow tapered at the C3–4 level and was totally occluded at the C2 level. () Few minutes after angiography with 90° to the right, the patient lost consciousness and DSA was halt. As the right VA was hypoplastic and flow almost absent, the posterior circulation was highly dependent on the left VA. We concluded that the left VA became kinked at the C2 transverse foramen when the head was turned to the right. We decided to perform anterior decompression of the left VA.
The patient was placed in the supine position and a transverse skin incision was created on the medial border of the sternocleidomastoid muscle at the C2-3 level. The anterolateral portion of the left transverse foramen that covered the VA was carefully drilled. The VA was fully decompressed, and VA pulsation immediately became more active. Postoperative CTA showed a wider transverse foramen than preoperatively (). The symptoms improved dramatically after surgery. Neck movement was not limited, and no instability was noticed on a dynamic X-ray of cervical spine.


A 32-year-old male without any significant medical history presented to the emergency department (ED) with complaints of lower back pain, weakness in the legs, and gait disturbance. The weakness was gradual, started 3 h before coming to the hospital, and ascended to involve bilateral upper extremities. He also had four episodes of nonbloody, nonbilious emesis. The patient denied any similar previous events. Upon further questioning, he reported a 15 pound unintentional weight loss since he did landscaping a month ago. Vitals on presentation showed a blood pressure of 148/70 and heart rate of 107 beats/min. The patient had a body mass index of 28.72 kg/m2. On physical examination, motor strength was 2/5 in both upper and lower extremities, and the patient was only able to move the digits of the hands and feet. Sensory examination was benign, upper extremities deep-tendon reflexes (DTR) were 1/4 bilaterally, knee DTRs were 1/4, and ankle DTRs were 0/4. On cardiovascular examination, the patient was tachycardic but regular without murmurs or gallops and cranial nerves were intact without facial muscle weakness. He denied any dyspnea, chest pain, palpitations, diaphoresis, and heat intolerance. He did not have a previous illness or gastrointestinal symptoms before the episodes. Past medical, family, and social history were unremarkable.
Initially, in the ED, orthopedics was consulted, and a magnetic resonance imaging of the lumbar spine was completed; the results were not significant. Due to the progression in the weakness, neurology was also consulted for a possible workup of Guillain–Barre syndrome.
Electrocardiography (ECG) [] showed a wide complex tachycardia consistent with VT. Advanced cardiac life support was initiated with an amiodarone infusion, and eventually QRS complex narrowed [] and wide complex tachycardia resolved. Laboratory values revealed a potassium level of 1.6 mEq/L (reference range: 3.5–5.0 mEq/L), phosphorus level of 1.7 mg/dL (reference range: 2.5–5.0 mg/dL), and magnesium level of 1.6 mg/dL (reference range: 1.7–2.5 mg/dL). Critical care team was consulted, and the patient was admitted to the intensive care unit. Intravenous potassium chloride (KCl) 40 mEq over 2–3 h and oral KCL 40 mEq were administered to treat the electrolyte imbalance. Patient paralysis was quickly reversed; motor function was regained with movement of the lower extremities. Electrocardiogram was repeated []. The patient was able to ambulate on his own and with assistance of physical therapy, his strength was 5/5 in all extremities, and he regained normal DTR.
Thyroid profile revealed that the patient had hyperthyroidism with a thyroid-stimulating hormone of 0.018 micro-international units/mL (reference range: 0.35–5.60 micro international units/mL); thyroxin (T4) level of 18.4 μg/dL (reference range: 4.5–10.9 μg/dL); and triiodothyronine (T3) of 2.52 ng/mL (reference range: 0.60–1.81 ng/mL). After the correction of the hypokalemia and paralysis and obtaining the results of the thyroid profile, the patient was started on methimazole 20 mg BID, propranolol 20 mg BID, and decadron 2 mg Q8 h; however, his potassium was overcorrected to 6.1. The patient was eventually stabilized and discharged home on medical therapy with planned outpatient follow-up.


‘J’ was a 35-month-old girl, the third of three children of married parents. She had congenital medical problems requiring multiple surgeries, and she continued to undergo regular follow-up procedures. J met the criteria for separation anxiety disorder with marked severity, mild social phobia and mild specific phobia. Although she was able to attend her familiar day care if handed directly to a teacher and attend a gymnastics class with a friend while her mother waited in the hall, J showed great distress if apart from her mother at home. If her mother left her sight (eg, to use the bathroom), J would sob, cry and try to open the door to get in. If her mother went out and left her with a family member, J would fuss, cry and try to come along, and would continually ask to video-call her, so her mother would cut her outings short. J also had fears of doctors’ visits, of riding in the car seat, and of walking independently up and down a staircase at home. She would approach new children only with assistance from her mother, and she was afraid to take part in gymnastics performances.
J also had some mood symptoms possibly related to her medical issues. She would intermittently have days when she was much more clingy, had uncharacteristically low energy, would want to be held, and would say ‘ow, ow’ if put down to stand. She also had difficulty staying asleep and would periodically wake up with respiratory difficulties.


‘K’ was a 26-month-old boy, the only child of married parents. He met the criteria for moderate separation anxiety disorder. Although able to go to a day care he had been attending since infancy, he showed distress at drop-off particularly at the start of each week, crying for 15 min. He feared being apart from his mother in the house: he could not tolerate his mother leaving the room even to change clothes and would cry if his mother left the playroom while K played with his father. He would get distressed if his father took him on outings without his mother. He could not be dropped off at a childcare centre at his parents’ gym, leading to their avoiding exercise. He slept in his own crib, rocked to sleep by a parent, but would wake in a panic (alert but distressed) two to three times per month, crying for over an hour until his parents took him into their bed. K also was very particular about where objects were placed in the playroom and would fuss if they were put in the wrong place. He got anxious about deviations in routine (eg, taking a different path on a walk) and had trouble throwing things away (eg, used Band-Aids).


A 60-year-old female presented to the thoracic surgery department with a history of an anterior mediastinal mass. She was a 14-pack-year smoker but denied history of any recent trauma or significant disease. A chest CT scan revealed a poorly enhanced anterior mediastinal mass, with signs of infiltration to the adjacent structures with sparse calcifications (Fig ). No lymphadenopathy or other mass lesions were identified. The preliminary diagnosis was thymic carcinoma. The patient subsequently underwent extended thymectomy via median sternotomy, complete excision of the tumor and lymph node dissection. The lesion was resected and frozen section biopsy revealed mediastinal VLM. The lesion showed a fragment of yellowish gray soft tissue, measuring 6.5 × 6.0 × 3.5 cm. Gross and microscopic features of the lesion are described in Figure . The patient had an uneventful clinical course and was discharged without further complication.


A 1-year-old girl with head trauma was admitted to our hospital in November 2016. At four weeks after surgery, the patient was febrile and disturbance of consciousness was observed.
The patient fell off the bed and mild head trauma was suspected. Incidentally, a computed tomography (CT) scan of the head revealed an intracranial space-occupying lesion (Figure ). One week after the admission, the child underwent a brain tumor resection. Immunohistochemical staining of the specimens indicated a choroid plexus papilloma. On the fourth postoperative week, she suddenly developed an altered state of consciousness with febrile illness. External ventricular drainage was performed. During the course of the disease, the patient had no diarrhea, abdominal pain, nausea, vomiting, cold, or any other discomfort.
The patient had no medical history of chronic diseases such as diabetes, coronary and other heart diseases, infectious diseases such as hepatitis and tuberculosis, surgery, blood transfusion, trauma, and drug allergy. The patient’s history of preventive inoculation was unknown.
She was operated for a choroid plexus papilloma in November 2016. The rest of the personal and family history was unexceptional.
At admission, the patient showed stable vital signs; however, the skin on the right side of the forehead was swollen, approximately 5.5 cm × 4 cm in size. On the fourth postoperative week, she suddenly developed an altered state of consciousness and hypertonia of limbs, along with the disappearance of the direct and indirect pupillary light reflexes.
Cerebrospinal fluid (CSF) examination showed a white blood cell count of 500/mm3. The patient was diagnosed with meningitis and intravenous administrations of meropenem (120 mg/kg/d divided q8h) and vancomycin (60 mg/kg/d divided q6h) were initiated; however, the patient did not show any improvement. After 5 d, CSF analysis showed a white blood cell count of 40000/mm3, protein concentration of 573 mg/dL, and glucose concentration of 0 mmol/L. CSF culture was positive for A. baumannii, which was sensitive only to tigecycline and colistin while it showed varying degrees of resistance to penicillin, amikacin, ceftriaxone, cefixime, cefotaxime, ciprofloxacin, levofloxacin, gentamicin, meropenem, imipenem, and tobramycin.


A 45-year-old mixed-race man was referred to the Infectious Diseases Department of our hospital to be investigated for chest pain, dyspnea, cough, chills, diarrhea, and vomiting, which was associated with anorexia and a weight loss of 10 kg. He smoked tobacco and crack and was an alcoholic.
On admission to the previous hospital, the man was pale and malnourished, with poor oral hygiene. Pulmonary auscultation revealed decreased breaths sounds in the right hemithorax and diffused rhonchi. Hepatomegaly and tenderness elicited by palpation were also present. Chest x-ray revealed pneumothorax and findings consistent with empyema in the right hemithorax. Thoracostomy released abundant purulent and fetid fluid from the right hemithorax. Direct examination of the pleural fluid with saline, in light microscopy, revealed numerous active trophozoites similar to Trichomonas (); Gram staining was not performed. The fluid culture was negative (probably due to improper sample handling). Antibiotic therapy with cefepime and metronidazole was initiated, and the patient was transferred to the Infectious Diseases Department of our hospital on day 10 for further management.
On admission to the Infectious Diseases Department, the patient was pale, disoriented, had tachycardia, decreased breath sounds at the base of the right hemithorax, which had a thoracic drainage tube. A new chest x-ray revealed right pneumothorax and an ovoid, homogeneous opacity without any air-fluid level in the right lower lung lobe, and the presence of thoracic drainage (). A non-contrast chest computed tomography was performed, revealing the pneumothorax with irregular cavitation in the right hemithorax.
The cavitation measured 10.0 × 7.5 × 5.0 cm, had thickened walls, and was located in the pleuropulmonary interface of the posterior margin of the right lower lobe. Its interior showed tissue bands indicating permeation and fluid collection consistent with an abscessed/necrotizing lung lesion in contact with the pleural space. Centrilobular ground-glass opacities, predominantly in the central regions of the left lung, were also visualized, probably representing a non-specific, inflammatory/infectious process ( and ).
A pleurostomy was performed. Post-operatively, the man remained clinically and hemodynamically stable in the intensive care unit; however, on the second postoperative day, he suffered sudden major hemorrhage emanating from the surgical wound. Vasoactive drugs were administered, and he was immediately transferred for emergency thoracotomy, but died on the way to the operating room.


An 83-year-old woman presented to the Emergency Department with a 6-month history of recurrent and progressively severe abdominal pain, along with constipation, vomiting and unintentional 5-kg weight loss. Her medical history was notable for deep venous thrombosis and pulmonary embolism, osteoporosis, polymyalgia rheumatic and autoimmune thyroiditis. The clinical examination revealed normal primary vital signs, abdominal distension and a palpable mass in the right iliac fossa without signs of peritonitis. Blood tests were normal. A computed tomography (CT) scan with intravenous contrast demonstrated a large ileocolic invagination associated with diffuse colon and small bowel wall thickening and initial evidence of vascular congestion without signs of bowel obstruction []. Hydration and antibiotic prophylaxis were started. Colonoscopy was performed, and an endoscopic reduction of the intussusception through carbon dioxide and water insufflations was obtained; ischaemic injury of the surrounding mucosa was noticed. After the reduction, a large villous lesion of the right colon was revealed with irregular pit pattern highly suggestive of malignancy. The patient underwent an explorative laparoscopy that showed a complete resolution of the ileocolic invagination and right colectomy was performed adhering to oncological criteria []. The postoperative course was uneventful, and the patient was discharged after 8 days. The final pathologic examination revealed a moderately differentiated adenocarcinoma pT3 N1a (1/27).


We present the case of a 70-year-old man who underwent dipyridamole Rb-82 positron emitting tomography (PET) 3 days following percutaneous coronary intervention with right coronary artery (RCA) stenting []. There was complete occlusion of the proximal RCA, as well as multiple borderline lesions in the left anterior descending (LAD) and circumflex territories (LCx). Postthrombectomy and stenting [], angiography showed complete resolution of the proximal RCA lesion and no residual disease in the remainder of the RCA territory. No intervention was attempted for the lesions in the LAD and LCx territories.
A follow-up dipyridamole Rb-82 PET MPI study was requested to assess the significance of the lesions in the LAD and LCx territories []. Perfusion images showed significantly decreased tracer uptake in the inferior wall, which normalized on the poststress images. No other significant perfusion defects were appreciated. Flow quantification revealed an impaired global myocardial flow reserve (MFR) of 1.37 with relatively homogeneous myocardial blood flow (MBF) in all territories at stress at a stress rate-pressure product (RPP) of 7850 mmHg × bpm. Hypokinesis of the inferior wall was present on both rest and poststress studies, in keeping with stunned myocardium. No misregistration errors, or other obvious source of artifact, were noted on either rest or poststress images. On the rest study, there was a clear defect in the inferior wall, which normalized on the stress acquisition following a dipyridamole infusion. On the same day, the patient underwent a repeat MPI study, using dobutamine as the stressing agent. This study again demonstrated a defect in the inferior wall which was present on both the rest and stress studies. Aminophylline was administered after the study dipyridamole study with no additional interventions performed in the time between the two studies.
Eight hours later, a repeat Rb-82 PET MPI study was performed using dobutamine as a stressing agent []. Stress RPP reached 14350 mmHg × bpm. The inferior wall defect was again seen on the rest study; however, the defect persisted on stress images. MFR to the inferior wall was again diminished on both sets of images (rest = 0.59, stress = 1.21), with a corresponding regional wall motion abnormality. The reason for the difference in findings between the two studies is unclear but is likely related to the differences in mechanism of action between the two stressing agents.


A 50-year-old man presented with progressive bilateral neck swelling of 3 months. Biopsy from right level V node revealed metastatic squamous cell carcinoma. Whole-body FDG positron emission tomography-contrast enhanced computed tomography (PET-CECT) was done but could not detect a primary site of malignancy. The patient was treated with bilateral modified neck dissection, followed by adjuvant concurrent chemo-radiotherapy. Whole-body FDG PET-CECT was done 12 weeks after the completion of radiotherapy for response evaluation. It showed hypermetabolic soft-tissue thickening involving the right pyriform fossa (PFS) with maximum standard uptake value of 9.01 [Arrows in ]. This appeared suspicious for a malignant etiology. Direct laryngoscopic examination revealed mucosal bulge in the right PFS. Subsequent histopathology revealed ulcerated squamous mucosa with necrosis and gram-positive actinomycotic colonies with abundant sulfur granules, with no evidence of malignancy [–]. The patient was put on anti-chondronecrosis treatment afterward.


A 41-year-old woman presented to the outpatient department seeking treatment for a painless mass on her left eyebrow (). She had no relevant medical history other than trauma that occurred 15 years prior: she had bumped her head. On physical examination, the mass measured 2.5×2 cm, and it was firm, round, and hardly movable. No cutaneous abnormalities were observed around the left eyebrow, and the patient reported no neurological symptoms such as sensory deficit.
A computed tomography (CT) scan revealed a 2.5×2×0.7-cm radio-opaque tumor just medial to the left supraorbital foramen ().
An elective operation under general anesthesia was planned. The patient’s skin was incised horizontally along the wrinkle line, just above the hair-bearing area of the left eyebrow (suprabrow incision). The subcutaneous fat, as well as the frontalis and corrugator muscles, was dissected along the direction of the muscle fibers. In the surgical plane deep to the corrugator and superficial to the periosteum, a branch of the supratrochlear nerve was encountered; this was gently turned over using a vessel loop to the lateral aspect. A vertical incision was made in the periosteum, and a 2.5×2-cm osteoma was observed attached to the cortex of the frontal bone. Ostectomy was then performed using a chisel and mallet. The osteoma was extracted in multiple fragments to minimize trauma to the nerve. Contour was compared to that of the contralateral eyebrow and bilateral eyebrows were symmetrical. A postoperative CT scan showed that bone symmetry was adequately achieved (). Postoperative pathological findings were consistent with those of an osteoma.
After surgery, no sensory deficit was observed. A clinical photograph was taken after 1 week and showed that the scar was well hidden ().


A 42-year-old woman visited the outpatient department to manage a slowly growing, painless mass on her left cheek. It had been over 9 years since she first detected the lump on her preauricular area; its size had gradually increased. She had no noteworthy medical history, and no history of trauma or surgery. Upon physical examination, the mass was measured to be 3×3 cm, hardly movable, and non-tender. No cutaneous abnormalities were observed above the tumor and regional lymphadenopathy was absent. Neurological symptoms, including sensory deficit, facial palsy, trismus, or impaired mastication, were not observed.
In the enhanced computed tomography (CT) scan, a 2.8×2.8×1.8 cm sized tumor was detected in the left masseteric muscle. It was hypodense compared to muscle and was heterogeneously enhanced. The clinical diagnosis made at this point was intramuscular hemangioma ().
It was decided that the tumor would be removed under general anesthesia. A facelift incision with pretragal and occipital incisions was chosen over a modified Blair incision to avoid a conspicuous scar. A skin and subcutaneous flap was raised through the facelift incision until the anterior border of the left parotid gland. The parotid duct and buccal and zygomatic branches of the facial nerve were identified. The gland was elevated from the masseter muscle and branches of the facial nerve were retrogradely dissected and preserved. The tumor was palpated above the masseter muscle and the muscle fibers were dissected. A 2.5×2.2 cm, yellowish, solid mass was found. It was well separated from the surrounding muscle fibers and was continuous with a small nerve branch. The mass was excised, along with a short segment of the nerve branch ().
The pathological examination showed characteristic Antoni A and Antoni B areas. Immunohistochemical staining was highly positive for S100 protein. Thus, the diagnosis was revised to schwannoma ().
After surgery, any sensory deficits or motor dysfunctions, including facial nerve palsy or impaired mastication, were not observed. Clinical photography after 6 months shows a wellhidden scar with no tragal distortion ().


A 47-year-old lady with short stature (145 cm) had type 2 diabetes mellitus for the last 20 years. She had a history of severe sensorineural hearing loss for 12 years and ischemic heart disease for 5 years. Her mother and maternal aunt had developed diabetes and deafness before the age of 40 years. Her siblings and son had no systemic complaints. The best-corrected visual acuity (BCVA) was 20/20p in both eyes (right eye -4.75 DS, left eye -5.00 DS). Anterior segment and intraocular pressure were unremarkable in both eyes. The fundus showed well-defined, round to oval areas of atrophy surrounding the fovea in a circumferential manner in both eyes [Fig. and ] which is very similar to the ocular features of MIDD. Autofluorescence accentuated the appearance and showed sharply defined discontinuous areas of perifoveal hypoautofluorescence surrounded by speckled hyper autofluorescence which was not clinically obvious [Fig. and ]. The OCT [Fig. -] showed depression of inner retinal surface, inner retinal hyporeflective spaces [pseudocysts, Fig. and , *], disorganization/thinning of outer retina, outer retinal tubulation (ORT) , <], loss of external limiting membrane, ellipsoid and interdigitation zone, and thinning of the RPE and choriocapillaris. OCT angiogram showed some reduced flow at the area temporal to the fovea in the superficial retinal slab [] and artifacts showing superficial vessels due to retinal thinning in the deep retinal slab []. Outer retinal slab appeared to have 2 unmasking artifacts in the area of atrophy [], and the choriocapillaris slab showed signal void areas at the uninvolved area along with unmasking of underlying choroidal vessels at the involved area []. The multifocal electroretinogram (mfERG) revealed reduced foveal and perifoveal amplitudes which were more depressed in the right eye [] compared to the left eye [].


Mother of the index case was a 68-year-old female. She had posterior subcapsular cataract in both eyes (right > left). BCVA was 20/30 in the right eye and 20/20p in the left eye. She had barely visible RPE changes around the fovea and the optic disc [Fig. and ] which became obvious with autofluorescence imaging [Fig. and ]. Case 1 was noted to have A3243G mutation in MTTL1 gene using Sanger sequencing. Due to financial constraints, the genetic analysis of family members could not be performed.


A 14-year-old boy, known to have sickle cell anemia, was admitted with a history of abdominal pain and four vomiting episodes, which were non-bilious and non-bloody. He had no recent illness and no history of fever or chills and trauma. His medical history indicated that he was diagnosed as having sickle cell anemia and was put on hemoglobin electrophoresis at the age of 6 months, and he received his last transfusion 2 years prior to the study. His family history indicated that his brother also had SCD.
On physical examination, constant and non-positional abdominal pain, not aggravated by food, was located in the left upper quadrant (LUQ) and epigastric area. The abdomen was not distended, and no mass could be felt. The other physical examination findings were unremarkable.
His vital signs were as follows: temperature 37.6°C, pulse rate 100 beats/minute, blood pressure 80/50 mm Hg, and respiratory rate 20 breaths/minute. At the time of admission, the patient’s complete blood count findings were as follows: white blood cell (WBC), 18 000/mm3 with 85% polymorphonuclear cells and 13% lymphocytes; hemoglobin (Hgb), 7.9 g/dL; platelets, 140 000/mm3; and red blood celld, 1 900 000/mm3.
He was then admitted to the hospital, and an abdominal ultrasound was performed in which cholelithiasis was revealed. During the next few days, his abdominal pain persisted, and the patient gradually became febrile and dyspneic; thus, imaging studies were conducted.
Chest X-ray showed elevation of the left side of the diaphragm ().
Based on these findings and the patient’s symptoms, emergency abdominal ultrasound revealed pancreatitis, cholelithiasis with several stones, and enlarged spleen in which a mass was observed on one-third of its lower part, which was suggestive of splenic infarction, inflammatory mass, and/or tumoral lesions. Free fluid was observed in the pelvic and peritoneal cavities. Doppler ultrasound revealed splenomegaly and non-visible vessels in the spleen parenchyma.
A computed tomography (CT) scan with oral and intravenous (IV) contrasts of the abdominopelvic region showed splenic abscess and splenomegaly associated with infarction. Further, free fluid was observed in the pleural cavity ().
The laboratory values were as follows: WBC, 21,200/mm3; neutrophils, 82%; lymphocytes, 16%; monocytes, 2%; Hgb, 7.2 mg/dL; C-reactive protein (CRP), 123 mg/dL; lipase, 395 U/L; gamma-glutamyl transferase (GGT), 48 U/L; albumin, 3.42 g/dL; erythrocyte sedimentation rate (ESR), 21 mm/h; total bilirubin, 3.1 mg/dL; indirect bilirubin, 0.5 mg/dL; sodium (Na), 125 meq/L; and BS, 147 mg/dL. The urine and blood culture findings were negative, and vein blood gas (VBG) test findings were as follows: pH, 7.34; PCO2, 53 mm Hg; PO2, 44 mmHg; and HCO3, 19 mmol/L. The other laboratory data, such as the Serum glutamic oxaloacetic transaminase (SGOT), serum glutamic pyruvic transaminase (SGPT), phosphorus (P), potassium (K), cholesterol, triglycerols (TG), amylase, blood urea nitrogen (BUN), and creatinine (Cr) levels, were within the normal limits.
The patient was initially managed conservatively with analgesic administration, IV fluid infusion, and oral nutrition, as tolerated. With the presentation of symptoms such as fever, abdominal tenderness, and guarding behavior, and laboratory and imaging assay findings, he was treated with IV broad spectrum antibiotherapy, blood transfusion, and total parenteral nutrition; splenectomy and cholecystectomy were considered (). Nine days after admission and the patient becoming stabilized, he was transferred to the surgical unit.
During the surgery, a midline skin incision from the subxiphoid to the subumbilical region was performed. In the peritoneal cavity, multiple severe adhesions were noted between the enlarged spleen and the omentum, transverse colon, and stomach. After adhesion excision, splenectomy was performed. The gallbladder serosal surface was also incised to retract it in the cephalic direction to explore Calot’s triangle. Cholecystectomy was completed. Broad spectrum antibiotic therapy was provided, and packed red cells and fresh frozen plasma (FFP) were transfused. Oral nourishment was established. Eight days after surgery, the patient was discharged without complications and followed up in the outpatient clinic. The pathology was reported as splenic necrosis and abscess.
Informed consent was obtained from the patient and his parents who participated in this study.


A 42-year-old female patient was initially diagnosed in August 2012 to have a stage-III melanoma of the right upper skin of the back. At that time, a local surgical excision was performed to remove her tumor. The pathology report indicated that the patient had positive lateral margins and tumor invasion up to 4.0 mm. The wide local excision or sentinel lymph node staging was not performed at that time due to limitations of patient’s health insurance, but the patient remained asymptomatic for over 5 years. However, in September 2017, the patient developed a large metastatic lesion in her upper right retropectoral region of the chest wall. Molecular analysis (Caris Life Sciences, Irving, TX, USA) of the initial biopsy of the chest lesions in January 2018 revealed the presence of a BRAF mutation (p.V600E) on exon 79 and RBI mutations on exon 24 (C.540-1 G>A) as well as on exon 60 (C.2490-1 G>A) (). Moreover, the initial immunohistochemistry assessment of biopsy chest lesions indicated an over expression of multiple tumor biomarkers such as excision repair cross-complementation group 1 (ERCC1), O6-methylguanine-DNA-methyltransferase (MGMT) and class III β -tubulin (TUBB3) ().
Since BRAF mutations are sensitive to molecularly targeted therapies, dabrafenib therapy with a MAPK kinase (MEK) inhibitor, trametinib was recommended for the patient. Unfortunately, prior to the start of the dabrafenib and trametinib therapy, the patient developed CNS symptoms including headache, dizziness and disequilibrium. Further staging with a computerized tomography (CT) scan of the abdomen and pelvis that was performed and it revealed a cystic right ovary of a size of 3.7 × 3.1 cm2 without any evidence of metastatic disease or regional metastasis. An MRI of the brain revealed at least eight ovoid enhancing lesions measuring up to 0.9 cm, which were scattered throughout the bilateral cerebral hemispheres and left thalamus compatible with metastatic melanoma. No intracranial hemorrhage or other fluid was noted.
An imaging of the CT scan of the chest () in December 2017 was revealed a large right infraclavicular and right chest wall soft tissue mass measuring 10.3 × 5.1 cm2, which was intimately, related to the neurovascular structures. A confluent mass measuring 5.4 × 3.6 cm2 and inferior 5.6 × 3.9 cm2 mass was also visualized in the axilla (). In addition, a 7.0-mm nodule was noted in the right upper outer quadrant of the breast. The chest wall otherwise appeared free of disease and the patient had a normal bone density. A bone scan showed no evidence of osseous metastasis. Since the patient had multiple brain metastases, stereotactic radiation therapy (SRS) was deemed not to be an appropriate strategy for the patient. Whole brain radiation therapy (WBRT) remains standard for patients whose brain metastasis is not amenable to SRS [,]. Therefore, the patient was administered with WBRT to a dose of 30 Gy delivered in 15 fractions to her multiple CNS lesions. Concurrently, the patient also received genetargeted therapy with systemic dabrafenib and trametinib. The basis for selecting the moderate fractionation of radiation for this patient was our previous experience that we had when we used a shorter course and higher fractionation schedule in patients who were treated with radiation and targeted therapies. The local effects of the treated tissue were exacerbated despite the use of targeted conformal approach. Therefore, we used a standard fractionation schedule to prevent the potential increased local reaction.
A repeat CT scan of the patient’s chest in April 2018 revealed a significant reduction (90%) in her chest mass with no pleural effusion (). The confluent tumor mass also reduced considerably in the axilla (). The residual patient’s disease in the chest wall and axilla was treated with a local field to a dose of 66 Gy delivered in 33 fractions with concurrent systemic dabrafenib and trametinib therapy. This portion of the treatment was administered between March 5, 2018 and May 22, 2018. The patient’s restaging bone scan after her treatment was negative for metastasis (). The patient had an excellent resolution of all residual disease in her chest. The patient was clinically disease free and her CNS lesions had regressed completely.
The abscopal effect was noted during the time the patient received cranial radiation therapy to her CNS and was receiving concurrent systemic gene-targeted therapy. She had a significant resolution of her large chest wall mass within 3 weeks of beginning her CNS treatment. The remainder of the chest wall lesion responded extremely well to local radiation therapy given with her gene-targeted therapy. Due to the patient’s insurance delay, her radiation therapy was ended while continuing with dabrafenib and trametinib therapy. During the absence of her radiation therapy, the patient developed new lesions in her chest area. Molecular profiling of a repeat biopsy of her new chest lesions in July 2018 revealed the presence of several gene mutations including BRAF mutation (). Repeat immunohistochemistry assessment of her biopsy indicated an over-expression of numerous tumor biomarkers including ERCC1, MGMT, and TUBB3 ().
While continuing on dabrafenib/trametinib therapy without radiation therapy, the patient experienced recurrent disease immediately inferior to her prior treated fields that quickly became metastatic to other chest and body sites. These findings indicate the dabrafenib/trametinib therapy was not effective possibly due to a BRAF inhibitor resistance. Moreover, the findings of this case also suggest that patient experienced an initial abscopal response during cranial radiation therapy to her brain lesions. Despite continued systemic gene-targeted therapy, the patient died due to wide spread disease recurrence outside of her treatment fields approximately 4 months later.


A 57-year-old male patient was referred to our outpatient department. He was diagnosed to have carcinoma colon in 2015 (adenocarcinoma of sigmoid colon) and had underwent hemicolectomy with lymphadenectomy with eight cycles of adjuvant chemotherapy (XELOX scheme). His follow-up colonoscopy was negative and he was on regular follow-ups. Positron emission tomography (PET) CT was done in April 18, 2018, which showed micronodules with a bigger nodule of 7 mm in the lower lobe of the right lung []. Subsequent PET-CT in October 23, 2018, showed increase in the size of this nodule to 11 mm in the superior segment of the right lower lobe. Contrast-enhanced CT (CECT) chest was also done [].
The case was discussed in multidisciplinary meet and it was decided to do EBUS fine-needle aspiration cytology of the nodule. Hemogram, serum electrolytes, prothrombin time, activated partial thromboplastin time, and international normalized ratio were unremarkable. As seen in the CT scan, the pulmonary nodule was 5 mm away from the airway with intervening normal lung tissue in between. It was not accessible through conventional technique of EBUS []. EBUS was performed by compressing the abutting normal lung tissue, thus causing compression collapse of the intervening normal lung tissue which helped in visualization of the pulmonary parenchymal nodule. After reaching into the bronchus intermedius, EBUS probe was turned to the left side (−90) and then we inflated the balloon and pushed down the lever to compress the lung tissue. The lung sliding sign which was visualized initially was replaced with isoechoic lesion [ and ]. We labeled this technique as the ZUTAM technique. TBNA was done from the lesion with an Olympus 22 gauge needle. There were no complications related to the procedure. EBUS-TBNA report confirmed the lesion to be metastasis from adenocarcinoma of the colon.
[MULTIMEDIA:1]


A 23-year-old man reported to Health Care Center, Mumbai, Maharashtra, India, with a chief complaint of excessive gum display as well as dark gums while smiling.
On clinical examination, he had gingival display of approximately 8 mm with a high smile line and a wide smile extending from maxillary right first molar (16) to maxillary left first molar (26) with dark brown pigmented patches on attached gingiva, whereas the marginal and interdental gingiva were pale pink []. His wheatish complexion did not coincide with the intensity of gingival pigmentation. All the anterior teeth were small []. The patient’s upper lip significantly drifted apically exposing excessive amount of gingiva while smiling. He had no adverse habits as well as a noncontributory medical history. Therefore, the following treatment plan was selected to correct the smile problems:
Crown lengthening from upper right first premolar (14) to upper left first premolar (24) to correct the teeth size.
Lip-repositioning surgery to restrict the hypermobility of upper lip while smiling.
Gingival depigmentation using electrocautery to eliminate the pigmented patches.
All the three surgical techniques were performed together.


A 70-year-old male resident of Wardha district of Maharashtra presented with swelling of the scrotum and penis with a recurrent history of fever and lymphangitis. For 30 years, he also developed edema of the right lower limb. The patient was a known case of filariasis, and with the course of time, he suffered recurrent episodes of inflammation due to which there was progressive increase in the size of the scrotum and penis. The patient neglected the symptoms until it started interfering with his normal routine work following which he presented to the surgery department.
On general examination, the patient was obese and hypertensive but nondiabetic; other findings were normal. On systemic examination, cardiovascular system and respiratory systems were normal. The abdomen was soft and nontender on palpation, and there was no organomegaly.
Local examinations revealed large thickened, firm filarial scrotum having nonpitting skin along with Ram's horn penis [] which had thickened glans, and the prepuce was nonretractile. The right lower limb had non-pitting lymphedema and minimal verrucae at the toes []. The presence of fungal infection was noted at the site. The inguinal lymph nodes were 2–3 in number, firm, and nontender on palpation.
Hematological and biochemical investigations were normal. Examination of scrotal aspirate revealed microfilariae [Figures and ]. Under anesthesia, scrotectomy was performed. Preputial skin was opened dorsally, and to our surprise, a cauliflower-like growth was seen over the glans involving the corona and part of the prepuce of 2.5 cm × 2.5 cm infiltrating on to the shaft of the penis []. Fine-needle aspiration cytology taken from both sides of the inguinal lymph nodes was negative for any malignancy. The frozen section confirmation of growth biopsy revealed it to be a case of SCC.
After proper explanation to the patient's relatives and with due consent, total amputation of the penis was performed [], and the perineal urethral opening was created. The wound was closed keeping drain and catheter in situ. The patient made an uneventful postoperative recovery, being discharged from the hospital after the surgery.
Two weeks postoperation, the patient was re-evaluated for any inguinal metastasis, which came out to be negative after which he was put on regular follow-up.


A 69-year-old male with a body mass index of 21.5 kg/m2 was referred to our hospital due to jaundice for a suspected cholangiocarcinoma.
His past medical and surgical history was unremarkable, except for diabetes type 2 and hypertension; both conditions were controlled with medications, including metformin 500 mg twice a day, glimepiride 1 mg twice a day, nifedipine CR 20 mg daily, valsartan 80 mg daily and enalapril 5 mg daily.
His social history was significant for smoking 20 cigarettes per day for 46 years and the frequent intake of alcohol at approximately 360-540 mL a day.
Family history was notable for a father diagnosed with esophageal cancer, a mother with uterine cancer and an elderly brother with duodenal cancer and leukemia.
On examination, the patient had icteric sclera and pale conjunctiva. His vital signs were stable, and physical examination did not reveal any extraordinary signs.
Laboratory tests on admission were within the normal range, except for a mild elevation in the levels of alkaline phosphatase (507 IU/L; normal range, < 359 IU/L), γ-glutamyl transpeptidase (199 IU/L; normal range, < 47 IU/L) and total serum bilirubin (1.4 mg/dL normal range, < 1 mg/dL). Carcinoembryonic antigen and CA 19-9 were within the normal range (2.7 ng/mL, 13.6 U/mL, respectively). Serology was negative for hepatitis B and C infections.
Endoscopic ultrasonography detected a solid lesion filling the intrapancreatic CBD to the main biliary. There was no evidence of extension outside of the bile duct wall, and invasion into the right hepatic artery was not observed. In addition, multilocular and unilateral cystic lesions of the pancreas, which had a maximum diameter of 13.1 mm, were found. Endoscopic retrograde cholangiography revealed bilateral intrahepatic biliary tree dilatation with a ﬁlling defect in the upper and middle bile duct, which extended partly to the left hepatic duct. Intraductal ultrasonography confirmed that there was no extended lesion in the right bile duct. Tumor and adjacent tissue biopsies were performed, and a 5 Fr endoscopic nasobiliary drainage ENBD was placed to facilitate biliary drainage. Pathological results endorsed IPNB with high grade and intestinal type neoplasia. In addition, images obtained from peroral cholangioscopy POCS delineated a tumor with a papillary pattern located at the CBD to the left hepatic duct (Figure ). Multidetector computed tomography detected an interruption for a long segment of CBD causing biliary occlusion, which resulted in bilateral intrahepatic biliary tree dilatation with no intrahepatic lesion. The tumor was predominantly extended into the left hepatic duct. An enlargement of the pancreatic duct suggested the extension of injury into the intrapancreatic CBD. Traces of metastasis, such as lymph node growth, ascites, lung mass or pleural effusion, have not been reported. Three-dimensional liver surgery simulation demonstrated that the right hepatic artery and right portal vein were not in contact with the tumor. Mmagnetic resonance cholangiopancreatography confirmed these findings (Figure ).
Therefore, an initial diagnosis of IPNB cT1N0M0 was established with good prognosis.