A 13-month-old, first born female child, born of nonconsanguineous mating presented with jaundice, high-grade, fever and passage of black-colored stools for three days. There was no history of hematemesis, decreased urinary output, seizures, pruritus, or indigenous drug intake. Antenatal, developmental, past, and family histories were unremarkable. The child was irritable, and had tachycardia (148/min) and pedal edema. Anthropometric indices of the child were normal for age [Weight, 9.4 kg (expected, 9.2 kg); height, 77 cm (expected, 75 cm; head circumference, 45 cm (expected, 45cm)], as tabulated in the relevant World Health Organization-Indian Academy of Pediatricians (WHO-IAP) combined growth chart for girls aged 0–18 years. The abdomen was distended, liver enlarged, firm-to-hard (7 cm below the costal margin), with a nodular, irregular surface, and the spleen was enlarged (3 cm below the costal margin) and firm. Signs of liver cell failure were absent. Other systems were unremarkable on examination.
Hematological workup revealed anemia (hemoglobin, 78 g/L; reference range, 115–135 g/L); elevated white blood cell count (WBC) 39 × 109/L (reference range, 5–17 × 109/L); neutrophilia and deranged prothrombin time (31 s; control, 11 s). Other findings were a normocytic normochromic peripheral blood with few schistocytes, a positive direct coombs test (DCT) (1+), and elevated reticulocyte count (7%, corrected, 3.5%; reference range, 0.8–2.2%). Serum haptoglobin, osmotic fragility/sickling/G6PD workup were unremarkable. Serum ferritin was elevated (194.9 μg/L; reference range, 5–25 μg/L). summarizes the results of major hematological and biochemical investigations displaying deranged parameters.
Viral (Hepatitis A, B, C, human Immunodeficiency virus, cytomegalovirus), autoimmune (antinuclear antibody, anti smooth muscle, anti liver kidney microsomal, anti liver cytosol type 1, anti soluble liver antigen antibodies), metabolic disease workup [urine gas chromatography-mass spectrometry (GC-MS), tandem mass spectrometry (TMS) for acyl carnitine and plasma amino acid profile, blood gas analysis] and thyroid profile (T3, T4, TSH) were unremarkable. Wilson's workup (Serum ceruloplasmin, urinary copper excretion, and slit lamp examination) were all within normal limits. Hepatic copper estimation could not be performed due to financial constraints.
Ultrasonography showed coarse liver echotexture, hepatosplenomegaly, increased periportal echogenicity, and moderate free fluid suggesting chronic liver disease (CLD). The gall bladder was visualized and the biliary system appeared patent. No cystic/mass lesion was apparent. Upper gastrointestinal endoscopy showed grade 2 esophageal varices.
An ascitic tap was performed and peritoneal fluid WBC count was 0.25 × 109/L (<0.1 × 109/L) with 90% polymorphs and albumin level of 10 g/L. Fluid culture showed pure growth of Staphylococcus aureus.
A working diagnosis of CLD with portal hypertension, spontaneous bacterial peritonitis, and possible autoimmune hemolytic anemia was made.
On admission, the child was put on a regimen of intravenous (IV) cefotaxime (450 mg) TDS for 5 days, IV furosemide (10 mg) BD, and oral aldactone (50 mg) BD (both 2 mg/kg/day). In addition, 225 ml N/4 saline in 5% dextrose with potassium chloride (1:100) was administered every 6 hours. Vitamin (A, B, C, D, E, K) and mineral (zinc, calcium) supplements were also given. Antibiotics were upscaled after 5 days to include IV amikacin (65 mg) BD and IV tazobactam (900 mg) every 8 hours as the patient showed little improvement. IV albumin and fluid restriction was initiated to manage increasing anasarca. The patient rapidly deteriorated in the next two days failing to respond to upscaled antibiotics and fluid restriction. A trial of empirical steroid (oral prednisolone) 10 mg BD produced transient improvement in ascites at which time a liver biopsy was done ensuring that the coagulation profile was normal at the time. However, after three days, the child succumbed to the disease. Consent for postmortem evaluation was declined.
Biopsied liver core tissue revealed loss of lobular architecture, hepatocyte ballooning, rosetting, lobular inflammation (6–8 foci/10 hpf), intrahepatocytic and canalicular cholestasis, and abundant Mallory hyaline. The portal tracts showed mild lymphocytic inflammation. Reticulin condensation with marked pericellular fibrosis, bridging fibrosis, and occasional nodule formation was noted [Figure and ]. Diffuse panlobular orcein positivity for copper-associated protein was noted. Marked copper deposition was confirmed on rhodanine stain [Figure and ]. Steatotic change, intranuclear inclusions, and giant cell transformation of hepatocytes were absent. Bile duct injury, inflammation, or periductal fibrosis was not seen. Perl's stain was negative for iron and Periodic acid Schiff (PAS) with diastase failed to reveal globular inclusions.
A young child with a negative viral, autoimmune, vascular, and metabolic workup as described here presented a major diagnostic dilemma. Female gender, transaminases in the range of 200–400 U/L, Coomb's positive hemolysis, and transient amelioration of symptoms after initiating steroids warranted the initial consideration of autoimmune hepatitis (AIH) despite the patient's age. Autoimmune profile, however, was negative.
Wilson's disease (WD) was also considered due to persistent hyperbilirubinemia. However, normal copper and serum ceruloplasmin did not support this diagnosis. No anatomical defects were noted on imaging. After three weeks, the diagnosis remained elusive. The liver biopsy had been withheld on account of the patient's acute symptomatology which was performed after a trial of steroids and antibiotic cover. Histologically, a cholestatic picture was observed. Marked orcein and rhodanine positive deposits with hepatocyte ballooning, marked pericellular fibrosis, Mallory hyaline, and nodule formation narrowed down the possibilities to WD and ICC.
ICC commonly occurs in the age group of 4 months to 5 years, as seen in this case.[] Cases of WD below 3 years are very rare and are generally asymptomatic.[] Features favoring ICC were normal serum ceruloplasmin levels, absence of neurological symptoms/KF rings, sharp liver margin on palpation, and rapidly progressive clinical course.[] Progressive liver failure though seen in both conditions is more characteristic of ICC. Aminoacidemia reflecting renal tubular dysfunction and hemolytic anemia may also be found in both. The histological picture of panlobular copper deposits, pericellular fibrosis, sparse regenerative activity, and diffuse Mallory hyaline favored ICC.[] Steatosis, glycogenated nuclei, irregular copper distribution, macronodules, and associated hemosiderin deposition, i.e., features more characteristic of WD,[] were absent in this case. Cholestasis is variable in both conditions and though supportive is not a sine qua non for histological diagnosis. The diffuse copper distribution pattern in ICC, however, is very characteristic and can be a useful pointer.[]
The needle biopsy with its limited sampling may be inappropriate to judge copper deposition, especially in cases of WD. However, the panlobular distribution seen here coupled with other diagnostic and clinical clues overwhelmingly shifted the diagnosis in favor of ICC. In view of the diffuse histological copper deposition seen in this case, a history of feeding via copper utensils was retrospectively enquired from the parents. No such history could be elicited. The child was also not exposed to copper through drinking water as the family dwelled in an urban colony where galvanization of water pipes was the norm. Genetic testing for ATP7B mutation was not performed in this case due to financial constraints.
In the present case, a panel of serum-based diagnostic tests and imaging modalities failed to reveal the etiology of CLD. The liver biopsy eventually proved to be the most informative diagnostic test. This reiterates the fact that, in an era where clinicians are inclined to employ noninvasive testing modalities, liver biopsy is still an essential important component of the CLD workup. However, practical problems affecting the timing and technique of the biopsy may adversely affect the final outcome.
In this case, ascites, deranged prothrombin time, and peritonitis prevented a timely percutaneous liver biopsy. The biopsy was eventually performed when the patient showed transient improvement on steroid therapy. This may have been prevented by performing a transjugular liver biopsy (TJLB). However, pediatric TJLB requires a high level of expertise, niche specialization, and a well-equipped angiography suite infrastructure that is not available at many centres in the country. Histologically, despite the lack of any history suggesting copper ingestion, classical features suggesting ICC, i.e., creeping pericellular fibrosis, abundant Mallory hyaline, extensive copper deposition, and parenchymal nodules were present. Because the duration of steroid therapy given to the patient before the liver biopsy was less than a week (three days), it is unlikely that a masking of histological features on the liver biopsy may have occurred.
In a complex scenario, suspecting ICC as a possible etiology of CLD by the clinician is difficult. More so, with the prevalent impression that ICC is an “extinct” entity. A PUBMED search performed using the term “Indian childhood cirrhosis” in March 2017 yielded 329 results, of which 25 were recorded after 2000. This suggests that, though on the decline, it is certainly not extinct. Its exact pathogenesis remains elusive; the role of copper ingestion being the most debated. The 2006 Multicentre National Collaborative study (MCNS) study[] found no credible evidence for the same, as seen in our case. Some authors, however, have maintained that copper plays a definite role in disease causation.[] Muller et al.[] investigated 138 cases of Tyrolean infantile cirrhosis (TIC), another non-Wilsonian “copper” toxicoses similar to ICC, and concluded that the disease was due to copper toxicosis and an inherited predisposition to cirrhosis. Interestingly, TIC has not been observed after 1974.[] Nayak and Chitale[] recently emphasized that ebbing of ICC was more due to time-related sociocultural and environmental change and the inability to diagnose the entity. This viewpoint was corroborated by Patra et al.[]
Pathologists must be aware that, apart from the classical picture of established ICC, pre-cirrhotic disease may exist. This is characterized by extensive hepatocyte swelling and focal reticulin condensation in a hepatitic setting. Occasional pseudolobule formation and fibrous bands joining the portal tracts are other indicators of early disease.[] Ramakrishna et al.[] described an “atypical” copper cirrhosis presenting in older children and having a slower progression. Hence, classical ICC may be evolving to a more insidious variant which may escape diagnosis. This makes the timely study of biopsied liver tissue essential for instituting correct management. After studying the clinicopathological aspects of the present case, we suggest a relevant diagnostic algorithm to avoid underdiagnosing potential cases of ICC []. We acknowledge that though the nonperformance of postmortem analysis and molecular tests is a limitation of our work, such a scenario may be encountered frequently in routine hospital practice. The key point the present case highlights is that, while specialized tests for autoantibodies, WD, and metabolic disease are being done, a simultaneous liver biopsy must be performed at the earliest possible clinical opportunity.


A nineteen-year-old male presented with a five-year history of a slowly growing, painless mass on the right ischioanal fossa. Haematochezia and change in bowel habit were not observed. Before referral to our institution, perianal percutaneous colour Doppler ultrasound revealed a solid lesion with abundant blood flow, and then, a diagnosis of haemangioma was made. After admission, physical examination revealed a multinodular cystic-solid mass of 5 cm × 3 cm on the perianal region, which was ductile, elastic, non-tender, movable and smooth (Figure ). No fistula tract could be palpated around the mass. Rectal digital examination could not detect any abnormity. No cafe-au-lait spots, axillary freckling or enlarged inguinal lymph nodes were observed. The results of routine laboratory tests and computed tomography of the chest and abdomen were normal. Pelvic MRI showed a well-circumscribed lobulated mass by a thin complete capsule, which demonstrated a T1-isointense signal and heterogeneously T2-hyperintense signal compared to striated muscle, and homogeneously intense enhancement on contrast-enhanced images (Figure -C). A neurogenic tumour was considered by the radiologist.
Local excision without preoperative biopsy was the treatment protocol because the anal sphincters and gluteus maximus were uninvaded. The operative procedure referenced the Bascom cleft lift technique[]. After intraspinal anaesthesia, the patient was placed in the prone jack-knife position. A longitudinal spindle incision was made with a 1 cm surgical margin. After the lesion completely resected, a skin flap 1 cm thick was freed from the right buttock until the wound could be sutured without tension. The defect was restored by suturing the fat cushion with vicryl suture. A negative-pressure drainage tube was put upon the fat cushion. The wound was closed by interrupted suture and then dressed with pressure by using an elastic abdominal bandage.
Histologically, the mass was composed of 10 variably sized nodules that appeared white-grey and were individually encapsulated. Observation in high-power fields confirmed a predominant Antoni type A structure with hypercellularity, Verocay bodies and a rare Antoni type B structure with hypocellularity. Spindle cells of the tumour were shown with elongated nuclei and palisading parallel rows (Figure ). Increased vascularity and sporadic haemorrhage were evident. Nuclear atypia and mitotic figures were not evident. Immunostains showed that the tumour cells were diffusely positive for vimentin, S100 protein and CD56 and negative for desmin, smooth muscle actin, CD34 and CD68 (Figure ). The Ki-67 labelling index was 3%. A definitive diagnosis of PS was made.


A 34-year-old, nulliparous woman became pregnant with dichorionic-diamniotic twins by in vitro fertilization-embryo transfer. At 21.4 weeks of gestation, the patient visited a tertiary hospital for watery vaginal discharge with no evident symptoms. She was diagnosed of spontaneous PPROM of twin A with shoulder presentation. At 22.0 weeks, the patient was transferred and admitted to our institution for further management. The right arm of twin A was protruding from the cervix into the vagina on initial speculum examination. Patient counseling was done considering the risk of both termination and expectant management. The patient expressed strong will to keep the pregnancy until twin B reaches the threshold of viability even at the cost of twin A. The next day, the wrist of twin A could be seen outside the vagina as depicted in . The medical team decided expectant management utmost to prolong gestational age at delivery of both twins so far as prolapsed hand of twin A was complicated with evident circulatory disturbance. Upon admission, intramuscular (IM) injection of 12 mg betamethasone every 24 hours for 2 days was given to the patient for fetal lung maturation. Initial C-reactive protein (CRP) level was slightly increased (1.31 mg/dL) and 7 days of prophylactic antibiotics with intravenous (IV) ceftriaxone 2 g every 24 hours, IV metronidazole 500 mg every 8 hours, oral clarithromycin 500 mg every 12 hours were given upon admission. The level of CRP decreased to 0.11 after one week of antibiotics and IV antibiotics were stopped, followed by oral clarithromycin 500 mg every 12 hours until delivery at 25.2 weeks.
The next day after hospitalization, the color changes in fingers were noted with desquamation of the skin. The change aggravated as time passed and daily dressing was done until delivery. Cleansing the hand with normal saline and applying collagen (Healoderm; Genewel, Seongnam, Korea) to dry skin and keeping moisture with silicone contact layer (Mepitel; Mölnlycke, Gothenburg, Sweden) was done on a daily basis. At 25.2 weeks, the patient received an emergent cesarean section due to uncontrolled preterm labor despite tocolysis. Since given 3 weeks prior, a rescue betamethasone (12 mg IM) was covered before delivery. Twin A was a male and had a birthweight of 810 g with an Apgar score of 1 and 5 at 1 and 5 minutes. Twin B was a male and had a birthweight of 750 g with an Apgar score of 5 and 8 at 1 and 5 minutes. Upon birth, both were admitted to the neonatal intensive care unit (NICU). The patient showed no signs or symptoms of postpartum infection and was discharged 3 days after delivery.
Twin B was under mechanical ventilation for 5 days, continuous positive airway pressure (CPAP) for 33 days. Initial brain ultrasonography showed grade 2 intraventricular hemorrhage (IVH) which subsided on the last ultrasonography. Two doses of surfactant were given due to respiratory distress syndrome (RDS). He showed stage 1 neonatal enterocolitis (NEC) and was treated with antibiotics. He reached full enteral feeding in 14 days and was discharged 111 days after birth with a body weight of 3,299 g. On the last outpatient clinic follow up, twin B's corrected age was 8 months with normal neurodevelopment. Twin A was in the PPROM state for 23 days before birth with the right-hand protruding outside the vagina. At birth, the right upper extremity was externally rotated with edema, redness, and desquamation up to the shoulder level. No other medical treatment was instituted other than normal saline irrigation with gentle handling and careful drying of the skin. The skin lesions improved in 3 days with no limitation in the range of motion of the right arm. Twin A was under mechanical ventilation for 52 days, CPAP for 144 days, and nasal cannula for 30 days. The initial brain ultrasonography showed grade 2 IVH and the last ultrasonography showed no specific findings. Three doses of surfactant were given due to RDS. He had no NEC but the blood culture revealed growth of Serratia marcescens and total 15 days of antibiotics were used. He reached full enteral feeding in 194 days and was discharged 218 days after birth with a body weight of 5,130 g. On the last outpatient clinic follow up, twin A's corrected ages was 17 months with questionable developmental delay according to the Denver Developmental Screening Test [] and is scheduled to follow up at the corrected age of 2 years for Bayley Scale of Infant Development.


A 69-year-old female patient was admitted to our clinic due to morning stiffness more than an hour and bilateral chronic arthritis. Swan-neck, Boutonnière deformities, and cutaneous vasculitis of lower extremities were observed on physical examination. The blood pressure was 125/65 mmHg, pulse 82 beats/min regularly, and temperature 36.5°C. She had no medical history of taking any drugs. The level of C-reactive protein (CRP) was 18.66 mg/dl and erythrocyte sedimentation rate was 91 mm/h. The patient clinical and laboratory features are shown in . The patient showed radiological erosion and deformities in the joints [ and ]. Thoracic computed tomography revealed a mass with a diameter of 51 mm × 28 mm × 32 mm on the right paracardiac area. Chiari's network in the right atrium and interatrial septum patent foramen ovale with normal cardiac motility was achieved on transthoracic and transesophageal echocardiography []. She had 8 points for American College of Rheumatology/European League Against Rheumatism classification criteria for RA and 5 for SLE. She was diagnosed with the rhupus syndrome according to clinical features/laboratory parameters and treated with hydroxychloroquine and prednisolone. Arthritis, vasculitis improved, and the inflammatory markers were decreased to normal values. The patient is still on our follow-up.


A 24-year-old man was referred by a primary-care physician to our orthopedic department because of a 4-year history of non-traumatic ulnar-sided pain in the right wrist. The patient was a smoker and worked as a carpenter. He had no other medical conditions and did not use medications. He had no family history of any neurological disease. Physical examination revealed normal findings with no signs of carpal instability or distal radioulnar joint disorders. Tests of sensation and muscle strength were normal. A plain X-ray (antero-posterior and lateral views) of the wrist was normal. Magnetic resonance imaging (MRI) of the right wrist showed edema in the triangular fibrocartilage complex (TFCC). A wrist arthroscopy was done during which a TFCC tear was repaired. An above-elbow cast was applied. One week after the surgery the patient felt pain, described as a burning sensation, in the proximal forearm and noticed that he could not actively flex his right thumb’s IP joint. The pain disappeared after a day. Physical examination of the hand showed total loss of the thumb’s active IP flexion but no other abnormalities.
MRI did not show any abnormalities involving the FPL. EMG done 2 weeks after the onset of paralysis showed delayed insertion activity in the FPL suggesting possible AIN lesion. A repeat EMG, 2 weeks later, could not confirm AIN lesion, but the examiners reported difficulties in locating the FPL due to the arm cast. At 5 weeks after onset, no improvement had occurred and the treating surgeon decided to proceed to surgery on suspicion of tendon rupture. During surgical exploration, the FPL tendon was found to be intact; exploration and decompression of the AIN was then performed with no abnormalities found.
A repeat EMG, 6 months after onset, showed denervation activity in the FPL indicating severe axonal loss to the FPL with no reinnervation, grade-2 fibrillation potentials (graded 0–4), and absent motor unit potentials. At 6 months after AIN decompression, no improvement in the thumb’s IP flexion had occurred. A tendon transfer using the flexor digitorum superficialis (FDS) of the ring finger was performed. Physical examination 14 months after onset showed strong active flexion in the right thumb’s IP joint and no other AIN-related symptoms. However, the patient complained of recurrent swelling and pain in the palm and ring finger and subsequently underwent surgery with excision of the remaining part of the FDS, synovectomy and FDP tenolysis.
Six years after the right-sided episode, the patient presented with symptoms in his left arm. He reported that he had felt pain in the left arm followed by loss of active flexion in the left thumb’s IP joint. Physical examination of the patient’s left hand showed total paralysis of the FPL but normal strength of the index finger and middle finger FDP and thenar muscles. EMG showed denervation activity in the FPL and PQ with no voluntary activity, findings that were interpreted as AIN lesion; all other muscles were normal and NCS of the median and ulnar nerves were normal. The pain gradually subsided. At 9 months after onset, the patient was examined by a hand surgeon at another hospital. The surgeon recorded presence of total FPL paralysis and scheduled the patient for AIN decompression. However, 15 months after onset of paralysis, the patient regained active IP flexion. On examination immediately before the planned surgery, the surgeon found good FPL strength and canceled the surgery. The patient has full strong active flexion and extension in the IP joints of both thumbs (). He was able to return to work as a carpenter but works currently as a truck driver for employment-related non-medical reasons.


A 53-year-old, asymptomatic female with no significant past medical history was diagnosed to have a mass in the left atrium (LA) on routine cardiac evaluation. Transthoracic echocardiography revealed a mass of size 1.5 cm × 2.6 cm in LA attached to the interatrial septum (IAS). Considering patient's age, coronary angiography was done which showed normal coronary arteries and a tumor blush due to feeding arteries supplying the LA mass. Coronary angiography also showed feeding arteries arising from the left circumflex (LCX) and left anterior descending vessels (LAD) []. The patient was posted for excision of LA mass.
The patient was induced as per the standard anesthesia protocol and a TEE probe was introduced without any difficulty. Precardiopulmonary bypass (CPB) TEE examination demonstrated a homogenous and irregularly-shaped mass in the LA of size 2.4 cm × 2.2 cm arising not only from IAS [] but also seen attached to the roof of LA, without prolapsing through the mitral valve. The aforementioned findings were confirmed by the surgeon intraoperatively.
Under standard hypothermic CPB with cold blood hyperkalemic cardioplegia, the LA mass was excised through the right atrial approach, and IAS was reconstructed using pericardial patch. The patient was weaned from CPB without any pharmacological support. Postprocedure, TEE showed complete excision of LA mass and absence of flow across IAS. However, an unusual single continuous jet with a peak velocity of 0.9 m/s was seen from LA roof into the LA chamber near the opening of right pulmonary vein [ and ]. With a suspicion of CCF, the coronary arteries were looked at, for any dilated course by direct visualization on the surface of the heart as well as on TEE and found that the coronary arteries were not dilated. The possibility of tumor-feeding vessel draining into LA was considered and protamine was administered with simultaneous monitoring of the continuous jet using TEE. Thirty-five minutes after administration of protamine, there was a gradual decrease in magnitude of flow as well as velocity of the jet which finally disappeared []. The postoperative course was uneventful, and the patient was extubated after 6 h of mechanical ventilation. Postoperative TTE did not reveal any unusual flow in LA. Histopathological examination confirmed the diagnosis of myxoma. One-year follow-up of the patient showed no recurrence of myxoma and ventricular dysfunction.


Case 1: A 30-year-old, Fitzpatrick phototype (FP) III woman was referred for treatment of extensive burn scars on the upper and lower extremities. Eight months prior she had suffered second-degree burn scars from a propane tank explosion at a picnic and her burns were treated non-surgically. She was managing the scars with silicone gel sheeting, scar massage and compression garments, as well as taking oral gabapentin for burning pain and itching symptoms in the scarred areas. The patient followed closely with a psychiatrist for significant psychological distress that she experienced following the traumatic accident. Physical exam showed hypertrophic, erythematous and dyspigmented scars of the bilateral lower and upper extremities, including the dorsal hands and digits, affecting approximately 30% body surface area (BSA). The dark hyperpigmentation was especially severe on the lower extremities ( and ).
The patient was treated with both the 595-nm pulsed dye laser (PDL) (Vbeam Perfecta, Syneron Candela, Wayland, MA, USA) and a NAFL dual wavelength device (Fraxel DUAL, Solta Medical, Hayward, CA, USA). Topical EMLA cream was applied to the treatment areas under occlusion 1 h before each procedure. She received three treatments with the PDL (7 mm spot size, energy 8 J, pulse duration 6 ms, 30:20 dynamic cooling), six treatments with the 1550 nm erbium:glass laser (energy 30 mJ,14% density, 4–8 passes) and five treatments with the 1927 nm thulium laser (energy 10 mJ, 30% density, 4–8 passes) to the bilateral upper and lower extremities. Treatments were spaced approximately one month apart. Immediate post-treatment care included application of triamcinolone 0.1% cream twice daily for three to four days and broad-spectrum sunscreen. Adjuvant therapy included a one-time 1 mL of triamcinolone acetonide (20 mg/mL) injected intralesionally to a symptomatic hypertrophic scar on the left hand. Tacrolimus 0.1% ointment was applied twice daily to hyperpigmented scars on the legs throughout the course of treatment. Treated burn scars became softer, thinner and more normal in texture and pigmentation with significant overall cosmetic improvement ( and ). The laser treatments were very well-tolerated with no downtime, with minor pain during the procedure being the only adverse effect. The patient also reported improved mood and decreased pain and pruritus associated with her scars and was able to discontinue gabapentin therapy.


Case 2: A 33-year-old, primarily Spanish-speaking, FP III man presented with burn scars on the left neck, trunk and upper extremity. The patient, a sanitation worker, sustained an occupational second-degree chemical burn injury four months prior after being accidentally splashed on the job with potassium phosphate. He reported intermittent pain and itching of the scarred areas as well as significant emotional distress due to the appearance of his scars. Additionally, the patient noted new areas of hair loss on the scalp, which he believed resulted from the chemical splash. He had no personal or family history of autoimmune disease. Physical exam revealed hypertrophic and erythematous scars of the left neck, torso and upper extremity affecting 7% BSA, and well-defined, circular, non-inflammatory, non-scarring alopecic patches on the left postauricular and right vertex of the scalp consistent with alopecia areata ( and ).
The patient was treated with a 595 nm PDL and the NAFL dual wavelength device. He received two treatments with the PDL (5 mm spot size, energy 7.5 J, pulse duration 6 ms, 30:20 dynamic cooling), four treatments with the 1550 nm erbium:glass laser (energy 30 mJ, 14% density, 4–8 passes) and two treatments with the 1927 nm thulium laser (energy 10 mJ, 30% density, 4–8 passes). All treatments targeted the left upper extremities and neck. Topical EMLA cream was applied under occlusion to the treatment area 1 h before the procedure. At two of his sessions, both non-ablative lasers (1550 and 1927 wavelengths) were used sequentially over the affected areas. Treatments were spaced approximately one month apart and were well-tolerated. Post-treatment care included application of triamcinolone 0.1% cream twice daily for three days and broad-spectrum sun protection. Adjuvant therapy included continued scar massage. There was vast improvement in the appearance of the scars with regard to both texture and skin colour (). His alopecia areata resolved with four monthly sessions of intralesional triamcinolone acetonide injections (concentration 10 mg/mL, volume 0.5–2.5 mL) ().


Our patient was a 56-year-old female, weighing 45 kg suffering from haemolytic anaemia, paroxysmal nocturnal haemoglobinuria (PNH) and jaundice for the past 30 years. She was admitted with a diagnosis of carcinoma endometrium with profuse bleeding per vaginum. She had hepatosplenomegaly (16 cm × 18 cm). Her transaminase levels were normal while serum lactate dehydrogenase was elevated (247 IU/L). Her viral markers were negative. She tested positive for indirect anti human globulin test. Haematological workup revealed anti-JKa (Kiddgroup) antibodies in her blood. Investigations for beta thalassaemia, other haemoglobinopathies and G6PD deficiency were negative. Pre-operative echocardiography revealed left ventricular (LV) ejection fraction 51% with mild-LV dilatation and mild-LV hypertrophy).
She was scheduled for urgent robot-assisted radical hysterectomy. Blood haemoglobin was 6.2 g% despite one unit blood transfusion a week back, and total serum bilirubin was 3.7 mg%, Pre-operative packed red blood cell (PRBC) transfusion attempted in the ward the night before surgery was abandoned following the development of hyperpyrexia, severe chills and rigors. The PRBCs given were best-matched. PRBC transfusion was immediately stopped. Intravenous (IV) pheniraminemaleate 23 mg, hydrocortisone 100 mg, dexamethasone 8 mg and paracetamol 1g, respectively, were administered. Transfusion reaction workup was done in the blood bank and included clerical check, blood group, cross-match, direct Coombs test and indirect Coombs test (ICT) on patient's pre and post-reaction blood samples. Blood for culture was also sent from patient's blood sample and blood from the bag. After screening 28 units of ABO-Rh compatible blood, the blood bank could provide four units of fully cross-matched JKa-negative PRBCs. All 28 units were tested against anti-Jka anti sera before cross-match. Column agglutination technique with polyspecific (IgG and anti-C3d) anti-human globulin cards were used for cross-matching. The blood group of the patient was B Rh Positive. Patient's Rh and Kell antigen phenotype was DCce. All the PRBC units were extended phenotype for Rh and Kell antigens and unit's phenotype issue for transfusion were DCe.
Preoperatively, she had tachycardia (157 beats/min), blood pressure 123/58 mmHg and LV dilatation on echocardiography, suggestive of impending high-output cardiac failure (HOCF). Total leucocyte count was not raised. There was no evidence of pontine haemorrhage, neuroleptic malignant syndrome or heat stroke.
The patient was euthyroid, and pheochromocytoma was ruled out by the normal preoperative plasma metanephrines; urinary vanillylmandellic acid and by abdominal ultrasound. ECG, non-invasive blood pressure, end-tidal carbon dioxide and non-invasive continuous haemoglobin (SpHb) pulse co-oximeter (Masimo®, Irvine, CA).[] were applied. A peripherally inserted central venous catheter was placed in the right basilic vein. Invasive blood pressure could not be measured due to difficulty in arterial cannulation. However, a non-invasive cardiac output (CO) monitor (ICON™; Osypka Medical GmbH, Berlin, Germany)[] was applied.
Anaesthesia was induced with IV fentanyl 100 μg, propofol 50 mg, esmolol 30 mg and atracurium 50 mg. Her direct laryngoscopic view was Cormack-Lehane Grade-III. The heart rate peaked to 177 beats/min during intubation and was 160–165 beats/min 10 min after intubation despite giving 100 mg esmolol, 4 mg metoprolol and 400 ml Plasmalyte™ (Baxter Healthcare, Deerfield, IL, USA). One unit of JKa-negative blood was already on flow when the patient arrived, and two more units were transfused intraoperatively. The SpHb values were 6.9, 7.4 and 8.6 g% and the heart rates were 156, 142 and 111 beats per min after completion of the first, second and third units of PRBCs, respectively.
After induction of anaesthesia, on urinary bladder catheterisation the urine was very dark coloured. The nasopharyngeal temperature probe showed an initial reading of 40.9°C. Active cooling measures were undertaken with cold IV fluids including three units of cold PRBCs, ice-cold saline through nasogastric tube and; exposure to cold ambient temperature (23°C). IV paracetamol 1000 mg was administered. The ICON™ non-invasive cardiac output monitor provided cardiac parameters by electrical cardiometry and velocimetry: (CO: 4.41–7.78 l/min), systemic vascular resistance (784–1033 dynes/cm5; Normal range: 1000–1500 dynes/cm5), (stroke volume variation: 5%–20%; Normal range: 4%–14%), index of contractility (ICON: 22.5–74.9;Normalrange: 40–60), (systolic time ratio: 0.25–0.83; Normal range: 0.3–0.5) and (thoracic fluid content: 23–33 l/kOhms; Normalrange: 25–35l/kOhms).
Anaesthesia was maintained with bispectral index-guided propofol infusion, peripheral nerve stimulator-guided atracurium infusion, sevoflurane in medical air and metoprolol infusion titrated to heart rate. IV 20% mannitol infusion (200 ml) and IV furosemide 20mg were administered for renal protection. Urine output was normal, but urine remained dark throughout surgery (80 min). On neuromuscular blockade reversal with neostigmine 2.5 mg and glycopyrrolate 0.4 mg, the core body temperature was 37.8°C. She was conscious, oriented and pain-free at extubation.
Postoperative liver function tests revealed elevated serum bilirubin levels of total/indirect: 44.9/24.9 mg% an hour after surgery, 68/35 mg%18 h post-surgery, 24.7/13 mg% 24 h post-operatively and 11.8/7.4 mg% 36 h post-surgery []. Patient's post-transfusion serum and plasma samples were inspected for evidence of haemolysis on obtaining a bilirubin value of 68 mg%, and no on-going haemolysis was detected. Thereafter, the urine had begun to clear and the bilirubin levels began reducing. Haemoglobin/packed cell volume levels were 8.6 g%/27.7, 7.8 g%/25.1 and 6.9 g%/22.5% immediately post-operative and on the 1st and 2nd post-operative days, respectively. Postoperatively, she was administered mannitol 50 ml intravenously 6 hourly, IV furosemide 10 mg every 8 h, ursodeoxycholic acid 300 mg through nasogastric tube 8 hourly. She was discharged on the 6th post-operative day and was recalled 10 days later for follow-up and radiotherapy.
The transfusions given preoperatively were best-matched units and were selected based just on cross-match compatibility. The transfusions given 1 week before surgery were JKa negative. The intra- and post-operative transfusions were also JKa negative.


A 22-year-old right-hand dominant woman presented to the Department of Orthopaedics, The First Affiliated Hospital of Sun Yat-sen University, Huangpu District, Guangzhou, Guangdong Province, China in February 2016 with an enlarged painless right wrist mass with focal swelling that had persisted for 5 years (). During the previous 6 months, the patient had felt something pop and experienced pain with limited motion in her right wrist. Initially, she took no action; and the pain and discomfort settled after a period of 3 days. After 1 month, the wrist still ached and felt weak, and the range of motion was limited. The patient reported that a GCTTS in her right elbow had been removed 10 years previously. No recent injury, antecedent trauma, local neurological symptoms, fevers, or changes in weight were found in the patient.
Physical examination revealed a non-tender, 3 × 3×3 cm firm, mobile mass mostly overlying the dorsal aspect of the scaphoid and trapezium. The swelling was non-bony. No rash or other identifiable overlying skin changes were noted, and the lesion did not transilluminate. The patient had normal strength. The range of motion of the wrist was limited compared with the left wrist, with a 20° loss of flexion, 25° loss of extension and 8° loss of radial and ulnar deviation. Full pronation and supination were noted. Sensation to light touch remained intact in all three nerve distributions. A negative Tinel’s sign was noted, and the mass was non-pulsatile with a normal vascular examination. Laboratory tests, including erythrocyte sedimentation rate, C-reactive protein and white blood cell, were normal.
The simple radiographs revealed a 3-cm solitary soft tissue shadow dorsal to the scaphoid and trapezium. The mass contained fluffy bone thickening and an almost complete destruction of the distal radius over a distance >0.3 cm without associated periosteal reaction, mineralization, extrinsic osseous erosion, or cystic change in the adjacent bone (). An MRI indicated that the mass was mainly located dorsal to the scaphoid and trapezium without communication with the radioulnar joint. The mass appeared hypointense in T1-weighted sequences and hyperintense in T2-weighted sequences ().
Under general anaesthesia, complete surgical resection of the mass was undertaken. The mass on the wrist joint level appeared firm and was multi-nodular with a yellowish-tan to mottled brown colour (). Intraoperatively, the mass was attached firmly to the surrounding tissue (). Less invasive leverage reduction with external fixator support and iliac crest bone autologous graft for treatment of carpal instability were performed (). The overall results of immediate postoperative radiographs and ultrasonic examination were satisfactory.
Microscopic examination revealed that the tumour was primarily composed of broad dense collagen bundles with inflammatory cells and round cells scattered throughout. A few areas of acellular, dense eosinophilic material were noted. The tumour contained foci of multinucleated giant cells, round synovial-like cells, inflammatory cells, xanthoma cells, and siderophages with haemosiderin pigment (). An immunohistochemistry analysis revealed that the internalized cells as well as the cannibal cells expressed cluster of differentiation (CD) 68, CD45, and Ki-67 (granular cytoplasmic expression), which are presented in , , and . Histopathological examination revealed that the lesion was a GCTTS.
The study was approved the Investigational Ethical Review Board of the First Affiliated Hospital, Sun Yat-sen University and written informed consent was obtained from the patient.


A 21-year-old woman was referred to the Internal Medicine Clinic by her family physician with a 4-week history of gradually progressive myalgia, lethargy, and declining renal function. She reported no history of rash, fever, dyspnea, or hematuria. She resided in a metropolitan area of Australia and denied any recent travel or exposure to animals or infectious contacts.
Her medical history was significant for mild childhood asthma and pregnancy 6 months prior to presentation that was complicated by pre-eclampsia for which she underwent a cesarean section at 33 weeks’ gestation. The only medication she used was a progesterone contraceptive implant. Her mother had a known history of idiopathic focal segmental glomerulosclerosis, which was being treated with daily administration of mycophenolate and prednisone. Her mother also had history of pulmonary sarcoidosis and was being investigated for newly diagnosed anti-neutrophil cytoplasmic antibody-positive vasculitis.
Upon examination, the patient showed periorbital and bilateral lower limb edema, as well as central obesity. No tonsillitis, painful goiter, muscle tenderness, and/or rash were observed. Her abdomen was nontender, and no palpable organomegaly was observed.
Blood tests ordered by her family physician included a complete blood count and assessment of inflammatory biomarkers, which were observed to be within reference range. Her creatine kinase (CK) was markedly elevated at 1,460 U/L (reference range, 30–150 U/L), serum creatinine was 136 mmol/L (reference range, 45–85 mmol/L), serum urea 4.9 mmol/L (reference range, 2.5–6.5 mmol/L), and her glomerular filtration rate (GFR) was 43 mL/min/1.73 m2 (reference range, >59 mL/min/1.73 m2). Baseline blood testing performed during antenatal care 6 months prior had revealed a normal GFR of 90 mL/min/1.73 m2, creatinine 77 mmol/L, and urea 3.0 mmol/L. Local laboratories measure creatinine using the alkaline picrate method, and calculate GFR using the Modification of Diet in Renal Disease Study equation []. Autoimmune markers and viral serology were negative.
Results of urine microscopy and culture were normal. No microalbuminuria or proteinuria were identified; however, urinary myoglobin was elevated (99 µg/L; reference range, <70 µg/L). Ultrasonography of the urinary tract did not reveal any remarkable findings.
Thyroid function tests (TFTs) were performed at the Internal Medicine Clinic to investigate the elevated CK—these confirmed a diagnosis of primary hypothyroidism. Her free thyroxine (T4) level was 3.2 pmol/L (reference range, 7–17 pmol/L), and her thyroid stimulating hormone (TSH) level was 160 mU/L (reference range, 0.3–4.5 mU/L). Thyroid autoantibodies were positive. Thyroxine at a dose of 100 mcg daily was initiated to treat hypothyroidism, and she was referred to the Department of Nephrology for further evaluation of her renal impairment. Renal biopsy was performed that same week and demonstrated no histopathological abnormalities (, ).
Following initiation of thyroxine, the patient’s myalgia and lethargy showed complete resolution at her 2-week follow-up with her family physician. CK had reduced to 462 U/L and GFR had normalized to 80 mL/min/1.73 m2. Her TFTs had improved to a T4 level of 7.8 pmol/L, and TSH was trending downwards at 71 mU/L. At the 3-month outpatient follow-up, the patient remained stable with the use of thyroxine and showed normal renal function.


A 17-year-old man presented with an increase in the size and number of cutaneous lesions (Fig. A) and subcutaneous soft lesions. 18F-FDG PET/CT has been underwent which showed diffuse hypermetabolic foci of the neck, clavicular region, near iliac vessels of left, near piriformis, and gastrocnemius of left leg. The hypermetabolic tissue in pharynx was physiological (Fig. C). And hypermetabolic tissues in clavicular region was brown adipose tissue (BAT) (Fig. D), because of this young man was too shin and underwent 18F-FDG PET/CT in winter.[ The lesions under sternocleidomastoid (Fig. C), gastrocnemius (Fig. E) of left were neurogenic tumors, which were benign neurofibroma (Fig. B) by pathology.
The standardized clinical diagnostic criteria of NF1, which have existed since 1987.[ Many numerous lentigines and multiple café-au-lait macules on his father were founded. So, NF1 of this young man was eventually diagnosed.
Ethical approval was given by the medical ethics committee of Shandong Cancer Hospital affiliated to Shandong University. The patient has consented for the publication of the present case report.


Louis is a 67-year-old man, diagnosed with vascular dementia about 6 months ago, shortly after his wife’s death. He has a long history of diabetes mellitus and hypertension. At present, both are well controlled: sitting BP 132/78, no orthostatic drop, and HbA1C: 6.7%.
Paul, Louis’s son. They have been living together since Paul’s wife died about 5 months ago.
Peter, Louis’s grandson, junior at college, visiting his dad and granddad.
It is Sunday afternoon. Louis, Paul, and Peter are watching a football game on TV. The game is slow, almost predictable. Louis is nodding off.
Peter says he is going for a drive. His father asks him where he is going. Peter replies that he is just going for a drive with his girlfriend. Paul starts cautioning his son about safe driving. Louis wakes up and says he wants to go for a drive, too.
Peter and Paul look at one another. They are worried. Paul says, “Dad, you can’t drive.” Louis responds, “Can’t drive? Why not? There’s nothing wrong with me and I don’t need any one’s permission to drive.” Paul counters, “Dad, your doctor said you couldn’t drive.” Louis cuts him off: “I don’t remember seeing any doctor and what do doctors know about driving anyway. Just let them take care of patients and stop meddling.”
Paul replies that Louis saw his family doctor who was concerned about his driving. He also patiently explains that recently Louis had been involved in several minor accidents: the garage door was clipped, a rose bush was run over, and about a week ago Louis received a warning ticket from a policeman because he ran a red light. Louis states categorically that all these accidents are other people’s fault. He is a safe driver; in fact, he taught Paul how to drive.
The argument escalates. Paul states firmly, “Dad. You cannot drive. It is not safe. We will not let you drive the car.” Angrily Louis says, “Son! This is my car. No one tells me when I can or cannot drive! I’ll drive whenever and wherever I want to!”
Very upset Louis goes to the side of the door where the car keys are hanging and takes them. Paul grabs Louis’ wrist. They both struggle for the keys. Louis defiantly slaps Paul on the face. Without thinking Paul retaliates by punching him. Louis falls backward, unconscious, blood oozing from his nose and mouth. A catastrophic ending.


A 56-year-old man with episodic transient visual loss in right eye (OD), of 2 days duration. He was not symptomatic at presentation and was using anti-coagulants for cardiovascular disease. Visual acuity was 6/6 both eyes (OU). Intra-ocular pressure and anterior segment were normal OU. OD had a finger like area of macular whitening sparing the fovea. This area corresponded to the vascular bed of a clinically appearing cilio-retinal artery (cilio-RA) []. However, on fluorescein angiography, the artery filled along with branches of central RA (CRA) [Fig. and ]. Swept source optical coherence tomography (SS-OCT) showed inner and middle retinal edema with shadowing of underlying layers []. The superficial capillary plexus was less affected on SS-OCT angiography (SS-OCTA). The deeper plexus revealed reduced capillary density nasal to the fovea, with an adjacent hypo-dense capillary nonperfusion area [Figure and ]. A diagnosis of occlusion of intra-neural branch of CRA was made.


In April 2013, a 63-year-old male was admitted to our cardiomyopathy clinic for the diagnosis of HCM. The patient had a negative family history for cardiomyopathies and sudden death. His previous clinical history was unremarkable. The ECG showed LVH with repolarization abnormalities in lateral leads. Routine laboratory tests (including proteinuria) were normal. Echocardiography showed severe LVH with maximal wall thickness of 27 mm, Type I diastolic dysfunction, and left ventricular outflow tract obstruction of 40 mmHg at rest. Cardiac magnetic resonance showed severe symmetric LVH with evidence of diffuse late gadolinium enhancement [] The patient underwent a systematic biochemical evaluation, according to the protocol of adult HCM in our cardiomyopathy clinic (including blood glucose, proteinuria, blood urea nitrogen, creatinine, electrolytes, lactate, ammonia, creatine phosphokinase, lactate dehydrogenase, and dry blood spot for lysosomial storage diseases and a NGS panel for sarcomeric gene disease). The enzymatic blood assay revealed no α-GAL activity in the patient's blood. Genetic analysis showed a gene mutation consistent with the diasgnosis of Anderson-Fabry disease (AFD). No sarcomeric gene mutation was found. The patients started enzyme replacement therapy (ERT).


A 54-year-old male was referred to our clinic for a severe HCM, with preserved systolic function, and with pericardial and pleural effusion. His family history was unremarkable. He started to suffer from hypertension since he was 45 years, proteinuria at 52 years, and bilateral carpal tunnel at 53 years. He presented with HFPEF (heart failure with preserved ejection fraction): dyspnoea, elevated brain natriuretic peptide measurement (2450 pg/ml), a chest X-ray showing bilateral pleural effusion. ECG showed low QRS voltages. Echocardiography showed biventricular hypertrophy, biatrial dilation, restrictive mitral inflow pattern, systolic pulmonary pressure of 50 mmHg, and pericardial effusion; a speckle tracking evaluation showed the “apical sparing” pattern []. A suspect of AL was based on clinical, imaging and lab findings. Cardiac biopsy showed interstitial, subendocardial, and small artery media deposits of amyloid which were confirmed on thioflavin T staining and with immunohistochemistry and immunofluorescence characteristic of λ light chain. The diagnosis was AL, and the patients started the specific chemotherapy protocol.


In April 2015, a 71-year-old female patient was referred to our group for osteopathic care after complaining of right hip pain. This patient was diagnosed with HIV 29 months prior (November 2012; 68-years old) and began a continuous ARV treatment regimen (nucleoside reverse transcriptase [lamivudine] and protease inhibitor cocktail [ritonavir+darunavir]) at this time. A plain spine radiograph obtained near the time of referral revealed potential osteonecrosis of the right femoral head. As part of the patient's care for HIV, a medical examination (including a computed tomography scan) was performed 8 months before her referral to our group. We reviewed this assessment and saw no indication of osteonecrosis (). Additionally, the patient had not received steroids since the time of diagnosis with HIV. Following our initial assessment, we recommended a magnetic resonance imaging (MRI) but it was not obtained because of the cost. Clinical (laboratory tests and physical examination) and radiological (pelvic computed tomography with contrast) assessments revealed no signs of infection. Total hip arthroplasty (THA) surgery of the right hip was then performed through a posterolateral approach under general anesthesia and taking all precautions published by AAOS (American Academy of Orthopedic surgeons) for surgery on HIV-positive patients to protect staff against HIV transmission. Specifically, the operating surgeon, two assistants and one scrub nurse wore disposable impervious gowns, helmets with facial shields and a body exhaust system, double hand gloves and impermeable boots. Disposable impervious drapes were used for draping. Instruments contaminated with blood were treated with well-established sterilization methods (e.g., autoclaving) and a 0.5% sodium hypochlorite solution was used to treat any blood spills. Intraoperative finding revealed femoral head collapse consistent with common osteonecrosis (). The anteroposterior view of an X-ray image confirmed repair of the right hip joint with implant and an intact left hip joint is intact. Bencox ID stem and cup (Corentec, Seoul, Korea) were used (). The test results for intraoperative bacterial identification was negative, and no signs of infection were observed during outpatient follow-up. No complications were apparent through the 12-month follow up; however, the patient began experiencing left hip pain 17 months after the right-hip THA. Three months later (20 months post right-hip THA), an X-ray revealed loss of the left femoral head (). A THA of the left hip was then performed using the same technique described above. Intraoperative results revealed more severe femoral head loss and osteolysis of the left hip compared with the right hip, but no signs of infection were observed (). Furthermore, intraoperative tests for bacteria were negative. Non-specific findings revealing chronic inflammation and fibrotic changes were identified upon histological exam (). No clinical or radiologic manifestations were present 3 months following the second THA ().


A 6-year-old boy reported to the Department of Pediatric Dentistry with a history of fall while playing 2 days ago. After the fall the child did not lose consciousness, had no history of vomiting or convulsions. There was a history of bleeding from gums, difficulty in the closing mouth and chewing food.
Clinical examination revealed a diffuse extraoral swelling in the right lower one-third of the face causing a asymmetry of the face, difficulty in opening and closing the mouth, and a cut on the chin and the lower lip. Intraoral examination revealed a vertical fracture line between right mandibular primary lateral incisor and primary canine associated with medially displaced left mandibular dentoalveolar segment with step deformity and altered occlusion leading to an open mouth appearance []. Preoperative orthopantamogram (OPG) was taken [], which confirmed right parasymphisis fracture. There was an associated fracture present on the left mandibular angle region.
Upper and lower arch alginate impressions were taken, and stone casts were poured. An acrylic splint was fabricated, and mandibular fracture was immobilized, fixed with the acrylic splint that was retained by circum mandibular wiring. Circummandibular wiring was done under general anesthesia by placing a small-stab incision on the inferior border of mandible on the right and left side 3–4 cm from the midline. The mandibular bone awl was used to enter lingually along the body of the mandible and piercing lingual mucosa the wire was fed and passed onto buccal sulcus along the body of the mandible. Wire held together and stent stabilized by winding wire in a clockwise direction at 83.84 region. The same procedure was repeated on the left side []. Another circum mandibular wire was placed at the left body region to stabilize the fractured fragment at the lower border of mandible. Postoperative OPG was taken with circum mandibular wires in place []. The patient was reviewed every week and on the fourth postoperative week, the circum mandibular wiring and splint was removed under ketamine sedation, and OPG is taken [Figures and ]. No mobility was present at the fracture site. Postoperative recovery was uneventful and occlusion achieved was satisfactory.


A 12-year-old female patient presented to the Department of Orthodontics in with a chief complaint of impaired esthetics. She presented with a dental history of feeding problem due to oronasal communication. Cheiloplasty and palatoplasty were performed at 5 months and 12 months of age, respectively. Her prognosis was poor according to Goslon Yardstick.[] Extraoral examination revealed a concave profile with a Class III skeletal pattern having a maxillary hypoplasia. Intraoral examination revealed that there is a prominent oronasal fistula perforation seen on the left side within palatal scar tissue. She had a Class I molar relationship on the right and left sides, anterior crossbite with reverse overjet of 4 mm, Unilateral open bite on the left side, palatally positioned 12, partially erupted 22 and incisors are in Class III relationship []. The mandibular arch is relatively undisturbed.
Cephalometric analysis showed Class III skeletal pattern with relatively retrognathic maxilla, vertical growth pattern, clockwise rotation of maxilla and mandible, retroclined and retruded upper incisors, normally inclined lower incisors and Class III soft-tissue profile [ and ].
The main treatment objective was to improve the facial esthetics and profile by correcting the Class III skeletal pattern and alignment of maxillary and mandibular dentition. Anterior maxillary DO was planned using an intraoral device to obtain correct skeletal relationship and adequate arch length to align the maxillary teeth. The main advantage of this procedure is decrease in the amount of relapse, because of soft-tissue histogenesis along with incremental traction of anterior maxilla.
Le Fort I osteotomy and anterior maxillary osteotomy are discussed as treatment alternatives but ruled out considering the amount of bone shift required to correct the crossbite.
The treatment was started when the patient is at 12 years 7 months of age. Surgery was aimed to provide alveolar bone support in the region of 22, 23 through autogenous bone graft raised from anterior iliac crest. The cleft region was untouched for 6 months for the graft take up []. After this secondary graft placement orthodontic correction was carried out to align upper and lower the teeth. The period of presurgical alignment and leveling was about 6 months. The surgical site for DO was decided in the interdental region between the maxillary first and second premolars on both sides. This surgical site provides the advantage of avoiding maxillary sinus exposure and taking more anchorage support from second premolars and molars on either side. Treatment simulation was made using dolphin digital imaging software (Paterson and Kelly, version 11.2) showed 7 mm advancement is required to achieve the adequate amount of overjet. To avoid the relapse after the surgery, it is planned to advance up to 9 mm [].
After the treatment simulation was finalized, a tooth-borne distraction device was custom made using a hyrax screw positioned in anteroposterior direction. The posterior anchorage unit was modified into an occlusal bite plane with cold cure acrylic. Anterior segment was consolidated by making a cap splint to prevent the cleft segment from parting away [].
Osteotomies for the anterior maxillary distraction were carried out under general anesthesia. Horizontal corticotomy cut was sited from premolar to premolar region 5 mm above the root apices. Vertical corticotomy cut was made interdentally between premolars on either side. Vertical cuts are extended to the palatal region on either side, and a greenstick fracture of the anterior maxillary segment had been done. Mucoperiosteal flap was closed primarily, custom-made distraction device was cemented to the teeth using glass ionomer cement and checked for its activation [].
After a latency period of 5 days, distraction was started from the 6th day after the surgery and was carried out at a rate of 1 mm per day with a rhythm of 0.5 mm twice a day. Distraction is done for 9 mm and the consolidation period took 2 months. Bone formation in the distraction site was confirmed on radiographs. At the end of this consolidation period, the distractor is removed [].
Postsurgical orthodontics was performed by placing the brackets on the maxillary teeth. Lateral open bite was developed in the region of 22, 23, and 24. This lateral open bite was corrected using the box elastics. The first premolar on either side is completely distalized and is included in the anchor unit. This is followed by distalization of canines on both sides. Palatally blocked out 12 is slowly aligned into this space. Left lateral incisor was in the cleft region; hence, very mild forces are used to derotate it [].
Retention is provided using upper fixed retainer from premolar to premolar and a Begg wrap on retainer, lower retention is attained by fixed retainer from canine to canine. Superimposition of the pre- and post-treatment lateral cephalograms showed a linear movement of maxillary anterior segment by 7 mm [ and ].
The results showed an improvement in skeletal, dental, and soft-tissue parameters. Anterior crossbite has been corrected. Overjet is improved from −4 mm to +2 mm. ANB angle was increased from −4° to 1°. Angle of inclination increased from 74° to 87°. Superimposition of the pre- and post-treatment lateral cephalograms showed a linear movement of maxillary anterior segment by 7 mm. Upper incisor was proclined little more than the ideal inclination to achieve acceptable interincisal relationship. The follow-up retention after 24 months showed a stabilized skeletal pattern with no signs of relapse [Figures and ].


In September 2014, a 28-year-old nonsmoking female was admitted to the hospital complaining of dyspnea and left chest pain. On chest CT scan, left massive pulmonary effusion with omolateral pulmonary atelectasis was observed (Fig. ). A pleural drainage was placed with immediate symptomatic relief, and cytological pleural fluid revealed metastatic adenocarcinoma.
The follow-up CT scan showed a left hilar tumor mass narrowing the left main bronchus associated with bilateral mediastinal and multiple systemic lymph nodes. A positron emission tomography-computed tomography confirmed the diagnosis of clinical stage IVB (T2b N3 M1c, TNM, version 8.0) (Fig. ). Definitive diagnosis of ALK-rearranged lung adenocarcinoma was obtained by bronchial biopsy of the tumor narrowing the left main bronchus (Fig. ).
Pemetrexed plus cisplatin (500 mg per square meter of body-surface area and 75 mg per square meter of body-surface area, respectively) was used every 3 weeks for up to 4 cycles, and successively oral crizotinib at a dose of 250 mg twice daily was given for a year. Although noticeable response was obtained, the delayed side effects including diarrhea, nausea, and constipation resulted in discontinuation of treatment within 10–12 months. Crizotinib was shifted to brigatinib (at a dose 180 mg daily), and improvement of disease was observed on CT scan after 1 year achieving a durable complete response after 2 years until today (Fig. ).


In March 2019, a 25-year-old nonsmoking female was admitted to the hospital complaining of cough and severe dyspnea. A chest CT scan showed right pleural effusion with bottom pleural thickening and subtotal atelectasis of the right lung associated with multiple mediastinal nodal involvement (Fig. ). Transthoracic ultrasound and medical pleuroscopy findings were mirror-like to chest CT scan imaging (Fig. ).
Exudative citrine yellow fluid was drained by ultrasound-guided thoracentesis, and diagnosis of ALK-rearranged lung adenocarcinoma was obtained on pleural fluid and confirmed on pleuroscopic pleural biopsies (Fig. ). Total body positron emission tomography with 18FDG confirmed a clinical stage IVA (cT3N2M1a, TNM version 8.0).
The chemotherapy with alectinib at 300 mg twice daily was performed, and the respiratory symptoms disappeared within 3 weeks. In 3 months, chest CT scan showed dramatic improvement of primary tumor with complete remission 15 months later (Fig. ).


A 14-year-old boy presented to the pediatric emergency department with a 3-day history of profuse vomiting and 1-day history of severe abdominal pain. He showed signs of dehydration with concerning vital signs: SpO2 − 98%, pulse rate 108/min, and BP 86/49 mm Hg. His abdomen was distended and tender on palpation. Initial blood analysis showed elevated leukocytes (31.8 × 10 9/L, 84.6 rel% granulocytes), urea (7.8 mmol/L), creatinine (109 μmol/L), bilirubin (109 μmol/L), alpha-amylase (132 U/L), lipase (496 U/L), and CRP (35.7 mg/L). His medical history included infantile left hemiparesis and operation of patent ductus arteriosus. Abdominal ultrasound was inadequate due to meteorism. A plain radiography of the abdomen showed an extremely dilated stomach, extending into the pelvis. Emergency CT scan confirmed the finding and reported gastric pneumatosis (shown in Fig. ). Vascular structures and the entire bowel were compromised by the distended stomach. The liver structure was heterogeneous, and air was detected in portal veins as well. Free abdominal fluid was noted. The patient was admitted to the pediatric surgery ward. A nasogastric tube was placed, and 2.5 L of dark, frothy liquid was drained. His vital signs were unstable, and he was transferred to the intensive care unit. Emergency surgery was scheduled. Initially, laparoscopy was performed, which showed a dark-tinted, distended stomach. The operation was converted to laparotomy. Before conversion, gastroscopy showed normal mucosa of the esophagus and dark, necrotic wall of the stomach. Over the whole operating field dominated a distinctly dilated stomach with necrotic wall and palpable crepitations as part of gastric pneumatosis (shown in Fig. ). Inspection of the distal bowel did not show a clear cause of obstruction, so the stomach dilatation was interpreted as idiopathic. It is important to point out that the finding suggested chronic dilatation with a peracute stop of bowel content which led to compression and gangrene, rather than an acutely occurred dilatation. A total gastrectomy was performed with esophagojejunal anastomosis. The stomach was sent for histopathological examination.
Postoperatively, still analgosedated and intubated, he was transferred to the pediatric IC unit. His hemodynamic parameters were stable, with oliguria. Furosemide was administered. Ultrasound of the thorax showed a left pleural effusion that did not need invasive treatment. Control laboratory results showed a decrease in leukocytes 15.7 × 10 9/L but a significant increase in CRP (320.8 mg/L). His coagulation tests were abnormal, so pooled human plasma was ordered. On the third postoperative day with his vital signs stable, he slowly regained consciousness and after a few hours was breathing spontaneously. After prior administration of dexamethasone, he was extubated and his response was stable. Control blood gas analysis was normal. Suddenly, he became restless, dyspnoic, and his oxygen saturation was decreasing. He abruptly fell into cardiopulmonary arrest. An immediate resuscitation protocol was carried out, with no ROSC outcome. The body was referred for an autopsy.
Due to the time required for analysis, the histopathological report of the stomach was complete after death. Histology showed full thickness necrosis with moderate neutrophil infiltration throughout all layers and hemorrhagic areas (shown in Fig. ). Air sacs were present in the mucosa and submucosa, suggestive of emphysematous gastritis (shown in Fig. ). There was abundant bacterial overgrowth, cuboid-shaped organisms arranged in distinctive basophile tetrad packets (shown in Fig. ). The bacteria were highlighted by strong positivity in Gram special stain. The final diagnosis of infection with S. ventriculi was established based on the characteristic morphology of these microorganisms and strong Gram positivity. The autopsy revealed multiple thrombi in the small blood vessels of the lungs and brain, with intra-alveolar hemorrhage and hemorrhagic necrosis of the liver and acute tubular necrosis. All this points to multi-organ failure and the development of disseminated intravascular coagulation. Areas of disturbed architecture were seen in the periventricular parts of the brain, with the demyelination of remaining neurons. Dystrophic calcifications were found in the surrounding areas. It was subsequently found heteroanamnestically that these changes were due to transient hypoxia in infancy. The finding indicated periventricular leucomalacia.


A 5-yr-old boy was admitted with a complaint of severe abdominal pain of three days' duration that had spontaneously occurred without a noticeable cause. At the time of admission, he had no other symptoms than abdominal pain and vomiting. His initial body temperature was checked at 38℃ with a slight fever that could be noticed until admission, with a pulse rate of 124 beats per minute and respiration at the rate of 30 per minute.
On physical examination, he appeared acutely ill, and skin rash, conjunctival injection and cervical lymphadenopathy were not seen. There was no specific lesion in the oral cavity or on the tongue. Neither erythema nor swelling on both extremities was found. His abdomen was soft and moderately distended with decreased bowel sound. Direct tenderness was so diffuse and severe, especially on the lower abdomen, that it was difficult to differentiate from the surgical abdomen. Rebound tenderness was obvious without muscle guarding. Hepatosplenomegaly or palpable abdominal mass was not detectable.
Initial laboratory examinations revealed white blood cell count of 13,790/µL with predominance of neutrophil 86.5%, red blood cell count of 4,390,000/µL, hemoglobin level of 12.4 g/dL, hematocrit of 35.6%, and platelet count of 229,000/µL. Aspartate aminotransferase (AST) and alanine aminotransferase (ALT) levels, urea, creatinine level and electrolytes were all within normal limits. Hematuria, pyuria and proteinura did not occur on a urine analysis. Erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) were elevated at 40 mm/hr (normal range, 0-10 mm/hr) and 15.85 mg/dL (normal range, 0-0.8 mg/dL), respectively. The result of blood culture was sterile. An abdominal radiograph showed distended bowel loop with fecal materials ().
He presented with atypically erythematous and maculopapular skin rash of irregular shapes and sizes just after admission. The rash predominantly involved the trunk, which was not improved with intravenous antihistamine. He continued to complain of abdominal pain with vomiting, without any improvement over two days.
Abdominal ultrasonography was performed to distinguish from acute appendicitis on day 2. It showed multiple variable lymph nodes in the right lower quadrant of the abdomen. On day 3, he revealed erythema and swelling of the hands and feet, and strawberry tongue. Secondary follow-up laboratory findings on day 4 showed slightly more elevated ESR and CRP, 50 mm/hr and 19.46 mg/dL, respectively.
High spiking fever was prolonged remittently for five consecutive days and unresponsive to antibiotics. At this time, we came to make a presumed diagnosis of KD. Meanwhile, his abdominal pain was not improved at all. The abdomen was soft, but tenderness was still observed without rebound tenderness. We performed abdominal ultrasonography again, which showed bowel wall thickening (). Subsequent abdominal computed tomographic scan showed circumferential enhanced wall thickening and edema in the distal descending and sigmoid colon and normal rectum (). We noted that his lips changed red on day 6, and that bilateral conjunctivas were injected without exudate. A right cervical lymph node was enlarged with a size of greater than 1.5 cm in diameter on day 7. On the basis of these characteristic features that corresponded to the diagnostic clinical criteria, we confirmably made the diagnosis of KD. Therefore, we perceived that gastrointestinal complications might be closely related to a vasculitis by KD. We intended to perform colonoscopy, but it could not be done because of his parents' refusal. Echocardiography performed on day 8 revealed minimal pericardial effusion and mild tricuspid regurgitation, and no dilatation of coronary arteries.
Abdominal pain and remittent fever persisted. He was treated with high-dose intravenous immunoglobulin, 2 g/kg in a single infusion, together with 50 mg/kg per day of oral aspirin on day 8 after admission. Not only did his fever begin to subside, but abdominal pain also diminished dramatically the day after IVIG administration. On the third day after the treatment, laboratory findings showed a slightly more elevated platelet count, 507,000/µL and a remarkable fall of the CRP levels at 1.29 mg/dL. No infectious foci were found. A rapid resolution of all the clinical features was noted. On follow-up abdominal ultrasonogrphy performed on day 11, we found that bowel wall thickening was almost all decreased, and the hepatobiliary system was unremarkable. On day 12, he was discharged with reduced dosage of aspirin, 5 mg/kg per day as a single dose.
About four weeks after discharge, desquamation was noticed on the fingers and the perianal area, but the patient was clinically free of abdominal symptoms.


A 31-yr-old female who had dispensed digestive drugs to admitted patients during 6 yr of employment as a university hospital nurse presented to allergy clinic 2 yr ago and complained of rhinorrhea and sneezing, but no asthmatic symptoms, when handling PPE powders. The patient was non-atopic and had no history of medical illness or family history of allergic disease. To evaluate her IgE responses, a skin prick test (SPT) was performed, and strongly positive responses were generated to PPE and α-amylase (5+ and 3+ wheal ratios of allergen to histamine, respectively). PPE and other medicinal extracts collected from her workplace were prepared according to the previously described method () and used to detect serum-specific IgE by enzyme-linked immunosorbent assay (ELISA). The positive cutoff value for a high level of serum-specific IgE antibody was defined as the mean±3 SD (standard deviations) of the absorbance value from unexposed healthy controls. The patient showed high levels of specific IgE to PPE and α-amylase by ELISA (1041 and 1003 absorbance value, respectively). To confirm bronchial sensitization, a methacholine bronchial challenge test (MBT) and a specific bronchial provocation test (BPT) were performed with PPE, and both generated negative responses (). The patient was thus diagnosed with occupational rhinitis to PPE. Antihistamine and a topical steroid were recommended to control her rhinitis symptoms, and she continued working in the same environment. Two years later, she revisited our allergy clinic complaining of rhinitis and additional asthmatic symptoms. A second SPT showed increased responses to PPE and α-amylase (6+ and 6+ wheal ratios of allergen to histamine, respectively), and her serum-specific IgE level was found to be highly elevated by ELISA (720 and 718 absorbance values and respectively). A second MBT demonstrated positive responses at 1.36 mg/mL, and the BPT showed an early asthmatic response after inhalation of PPE (); both tests were performed as previously described (). Negative responses were recorded for the other drug powders tested.


A 44-yr-old man with insomnia and depression was referred to the psychiatric department of our institution. He complained that he rarely fell asleep because of dysesthetic sensations and pain in the lower legs and feet and that he was depressed and feeling hopeless. Although he was aware of having had a high blood sugar level for 10 yr, he never sought treatment. About 3 yr earlier, in summer 2003, he began to experience cold, numbing, and pins-and-needles sensations on his toes that eventually spread throughout his feet. About a year later, he started taking hypoglycemic agents. However, the pain in his feet became severe, and restlessness as a result of creeping sensations in his lower legs gradually started to occur when he was lying down or sitting awake. He tried massaging and beating his legs and walking around. The unpleasant sensations and restlessness were temporarily relieved by massage and walking, but the pain in his feet remained little changed. These dysesthetic sensations were most severe when he lay down to sleep, increasing his sleep latency to longer than one hour. He woke repeatedly and had a difficulty falling back asleep because of further dysesthetic sensations. In July 2005, the patient received hemodialysis for end-stage renal disease. In those days his serum hemoglobin, iron, and ferritin levels were 7.4 g/dL, 29 µg/dL, and 97 ng/mL, respectively, so oral iron supplementation was administered. He gradually became depressed, experienced feelings of worthlessness, lost his appetite, and eventually stopped eating. His speech became slow, and his arms and legs trembled and twitched involuntarily. Subsequently, he showed irregular, spasmodic, involuntary movements in his limbs and on his face. He was taken to the emergency center of St. Vincent's Hospital on 26 June 2006 by his son. His blood sugar level was very low (49 mg/dL). T2- and fluid-attenuated inversion recovery (FLAIR)-weighted magnetic resonance imaging (MRI) scans showed high signal changes over the bilateral basal ganglia and right thalamus, although abnormal enhancing lesions were not found after contrast infusion (). The patient was diagnosed with hypoglycemic injury of both basal ganglia and the right thalamus and received conservative medical treatment. One week later, he presented to the psychiatric department complaining of depression and insomnia. He was depressed and feeling hopeless and had severe sleep disturbance and thoughts of suicide. He was transferred to the psychiatric ward. He complained that the most distressing problem was the difficulty in falling asleep and staying asleep because of the dysesthetic restlessness. He satisfied the diagnostic criteria for RLS in the International Classification of Sleep Disorders (). He was prescribed citalopram 20 mg for depression, gabapentin 900 mg, and clonazepam 0.5 mg for peripheral neuropathy and RLS, oral iron supplements for iron deficiency anemia and RLS, and haloperidol 1.5 mg for choreic movements, and received daily supportive psychotherapy. Also, he received hemodialysis three times a week.
The dysarthria and involuntary hyperkinetic movements gradually improved over his whole body, except for his left arm. The pain in his feet remained severe, and the dysesthetic restlessness continued in his lower legs. He still suffered from insomnia and depression. Four weeks later, follow-up T2-weighted MRI scans showed resolution of previously noted basal ganglia lesions, residua of the hyperintensity change in the left putamen, and an infarction as an old lesion in the right thalamus (). The patient showed increased deep tendon reflexes on neurological examination as well as delayed latencies and small amplitudes for complex muscle action potential and sensory nerve action potential on a nerve conduction test, suggesting peripheral polyneuropathy. He then underwent a nocturnal polysomnography to examine the causes of his insomnia. When he lay down for polysomnographic evaluation, he experienced dysesthetic sensations and an irresistible inner urge to move his legs, as usual. The patient complained that he could not endure the dysesthetic sensations and was given his usual medications before bed: gabapentin 300 mg, clonazepam 0.5 mg, citalopram 10 mg, and haloperidol 1.5 mg. The patient fell asleep 18.3 min after taking the medications. His total sleep time was 391.0 min, and sleep efficiency was 80.4%. There were 55.2 periodic limb movements per hour in sleep, and about 36% of them led to arousal. The complete diagnoses of the patient included chorea, peripheral polyneuropathy, RLS, periodic limb movement disorder, insomnia, and depression. During those days, his serum hemoglobin, iron, and ferritin levels were nearly normal: 11.4 g/dL, 62.0 µg/dL, and 210.0 ng/mL, respectively.
Although gabapentin, clonazepam, oral iron supplements, citalopram, and haloperidol were prescribed for 4 weeks, the pain, dysesthetic sensations, insomnia, and depression continued. To make matters worse, the choreic movements were aggravated. An increase in the dosage of haloperidol from 1.5 to 3 mg was prescribed. However, the choreic movements did not improve, and the dysesthetic restlessness, insomnia, and depression worsened. The haloperidol was reduced to 1.5 mg, and the gabapentine was replaced with carbamazepine 400 mg and topiramate 25 mg. However, there was no improvement after 2 weeks. Consequently, carbamazepine and topiramate were replaced with tramadol 150 mg, while maintaining haloperidol 1.5 mg, citalopram 20 mg, and clonazepam 0.5 mg. After the first administration of tramadol, the pain, dysesthetic restlessness, difficulty in initiating sleep, frequent awakenings, and difficulty in falling back to sleep after an awakening slightly improved, and then gradually improved further, followed by improvements in the depressed mood, lethargy, markedly decreased volition and appetite, and feelings of hopelessness.


A 25-year-old female patient was admitted in February of 2004, complaining of generalized easy bruising and weakness. Initial CBC showed bicytopenia: Hb 4.8g/dL, platelets 21,000/µL and WBC 14,280/µL (75% blasts). An immunophenotype was characterized by the expression of CD19 (89%) and CD22 (83.3%), in which HLA-DR (94%) and CD34 (99%) were strong. A bone marrow examination showed approximately 90% small-to-medium sized blasts with a small amount of blue cytoplasm.
In a chromosomal analysis of the bone marrow a 46, XX karyotype was found in 100% (20/20). RT-PCR analysis was negative for Bcr/Abl rearrangement. Based on these findings, we made the diagnosis of precursor B-cell ALL in our patient. Induction chemotherapy of the VPDL regimen consisted of 2mg intravenous vincristine on days 1, 8, 15 and 22; 60mg/m2 prednisolone on days 1 through 14; 45mg/m2 intravenous daunorubicin on days 1 to 3; and 4,000units/m2 intramuscular L-asparaginase on days 17 to 28. On day 42, bone marrow examination showed complete hematologic remission was achieved.
On day 43, our patient received prophylactic intrathecal chemotherapy via lumbar puncture. This consisted of preservative-free 12mg MTX, 40 mg cytarabine and 50mg hydrocortisone. Four days later, she was noted to have leg weakness, and symptoms progressed to the point that she could not walk. In addition, the patient could not move her legs without gravity. She had no deep tendon reflexes over her patellae or ankles, and exhibited a mild sensory deficit. We examined the first CSF cytology concurrently with the prophylactic intrathecal chemotherapy, and identified atypical lymphocytes which were suspected to be blasts. On the second, third and fourth CSF cytologies however; no leukemic involvement was evidenced. No further intrathecal chemotherapy was given to the patient. Electromyography (EMG) and nerve conduction velocity (NCV) studies were consistent with the presence of myelopathy and neuropathy in the motor and left median axons. On magnetic resonance imaging (MRI) of the lumbar spine, diffuse gadolinium enhancement of the anterior roots (ventral roots) was detected (). Methylprednisolone was administered at a daily dose of 30mg/kg for three days, however lower extremity weakness and urinary retention persisted. Eight months after the first MRI scan, following nine cycles of consolidation chemotherapy, a follow-up T-2 weighted MRI image was taken. This revealed heterogeneous high intensity lesions in the thoracic spinal cord, in addition to severe atrophy (). The patient was in complete hematologic remission and was given oral MTX, 6-mercaptopurine and prednisolone for maintenance chemotherapy. However she demonstrated no improvement in her paraplegia and areflexia 18 months following the first signs of irreversible paraplegia.


A 59-year-old man was admitted to Kyung Hee University Medical with fever, shaking chills, and bilateral leg pain. He felt general malaise and high fever about two days before admission, and he had eaten a bowl of rice capped with raw fish one week before admission. Except for hepatitis B, his medical and family history were unremarkable. He had never been evaluated for Hepatitis B. He did not consume alcohol nor did he smoke.
On arrival, his temperature was 36.9℃, heart rate was 108 beats/min, respiratory rate was 20 breaths/min, and blood pressure was 60/30 mmHg. Examination of the patient's legs revealed erythema on both posterior calves. Severe pain was present on the erythematous lesions, but no obvious external wound was present. The rest of the physical examination was unremarkable except for bilateral crackles with inspiration over the lower chest. The laboratory data on admission were as follows: peripheral white blood cell count 1,240/mm3, with 82% segmented neutrophils, hemoglobin 12.5 mg/dL, platelet cell count 38,000/mm3, C-reactive protein 2.2 mg/dL, prothrombin time 2.33 INR, serum albumin 2.0 g/dL, total bilirubin 2.81 mg/dL, aspartate aminotransferase 82 IU/L, alanine aminotransferase 28 IU/L, blood urea nitrogen 5.6 mg/dL, creatinine 2.0 mg/dL, creatinine kinase 992 U/L, myoglobin 2,678 ng/mL, lactic dehydrogenase 599 IU/L, arterial pH 7.30, PCO2 29.8 mmHg, PO2 98.3 mmHg, and bicarbonate 14.4 mmol/L on O2 2 L/min via nasal prong. The chest radiography taken on admission revealed pulmonary congestion on both lung fields, and coarse liver parenchyma was observed on abdominal ultrasonography.
V. vulnificus had been suspected in this cirrhotic patient who developed a septicemic illness associated with necrotizing cutaneous lesions after ingesting seafood. Empirical antibiotic therapy with vancomycin, ciprofloxacin, and doxycycline was started to treat V. vulnificus and community acquired soft tissue infections. Massive fluid and intravenous bicarbonate was administered to treat septic shock. The erythema had gradually enlarged in the first 20 hours after admission, and tense hemorrhagic bullae were also present on the erythema (). As time progressed, he complained of severe pain in both legs, and his leg showed marked edema with shallow pulses and physical findings of tender flesh. Both legs showed signs consistent with necrotizing fasciitis. Surgical exploration was not done because of the uncorrected septic shock and refusal by the patient and his family members. Twenty-four hours after hospitalization, the patient died from uncontrolled septic shock.
V. alginolyticus was isolated from the fluid culture of the bullae and three sets of blood cultures obtained on admission. After 48 hours of incubating the culture-positive samples, straight or curved, Gram-negative, oxidase-positive colonies were seen on blood agar, MacConkey agar, and thiglycollate medium. The colonies were smooth, convex, creamy in consistency, and gray-white with full margins. The bacteria were initially identified as V. alginolyticus by the API 20NE system (version 7.0 La Balme-Les Grotte, France) code number 6466644 at a 92.3% confidence level, but were unidentified by the Vitek system (Biomeriux, Hazelwood, MO, USA). After 24 hours of incubation on thiosulfate-citrate-bile salts-sucrose (TCBS) agar, yellow colonies were seen. Further identification was confirmed with the following biochemical tests. The colonies showed a positive reaction to indol production, methyl red, Voges-Proskauer, lysine decarboxylase, and ornithine decarboxylase. Negative findings included: growth on Simmons' citrate, H2S on TSI, gas production, urea hydrolysis, and arginine dihydrolysis. Sensitivity of the bacteria was determined by a Kirby-Bauer disk diffusion method, and the results were interpreted by the CLSI recommendations. The organism was susceptible to a variety of antibiotics including ampicillin, amoxillin-clavulanate, cephalothin, cefuroxime, gentamicin, ciprofloxacin, and trimethoprim-sulfamethoxazole.


A 58-year-old man presented with ventricular fibrillation. There was no family history of cardiomyopathy or sudden death. After successful defibrillation, his blood pressure was 106/60mmHg, and his pulse rate was 80beats/min. A 12-lead ECG showed ST-segment elevations in leads V3-V6 but no Q waves. Chest X-ray showed no cardiomegaly or pulmonary congestion. Laboratory examination revealed a mild increase in creatine kinase-MB (38mIU/mL) and troponin T (0.3ng/mL) levels. With provisional diagnosis of acute myocardial infarction, coronary angiography and left ventriculography were performed. Coronary angiography yielded normal results (), but the left ventriculogram showed severe left ventricular obstruction during systole with no aneurysmal bulging in the apical segment (). There was a significant pressure gradient (45mmHg) between the basal and apical sites of the left ventricle at rest (). Using continuous wave Doppler echocardiography, the pressure gradient was measured as 60mmHg. The patient had been diagnosed as having MVOHCM, and medical treatment with a beta blocker and amiodarone was started. ECG-gated, magnetic resonance imaging (MRI) was performed on the 10th hospital day, using an Intera Achieva (1.5 T, Philips Medical Systems, Best, Netherlands). Cine MRI showed mid-ventricular obstruction and an apical aneurysm with a large thrombus on long-axis () and 4-chamber () images. Initial gadolinium-diethylenetriomine pentaacetic acid-enhanced imaging showed reduced subendomyocardial perfusion in the mid-ventricular portion of the left ventricle (). Fifteen minutes later, delayed enhancement of the subendomyocardium was noted (). Because medical treatment with a beta blocker (carvedilol) did not reduce the intraventricular pressure gradient determined by continuous wave Doppler echocardiography, the patient underwent surgery in which left ventricular aneurysmectomy and removal of the thrombus were performed. The patient received a beta-blocker postoperatively, and the 8-week follow-up period was uneventful.


An 81-year old woman visited our emergency room after 2 days of severe ocular pain and loss of vision in her left eye. Her medical history included a cerebrovascular accident and hypertension and her medications included astrix, plavix and a beta-blocker. For the previous year, she had visited our clinic for visual disturbances due to a cataract but she had refused further evaluation. At that time, her visual acuity was 0.6 in the right eye and 0.4 in the left eye and a fundus examination had not been performed because of the patient's refusal.
Upon visiting our emergency room, her visual acuity was 0.4 in the right eye, but she had no light perception in the left eye. Her intraocular pressures (IOPs) were 14 mmHg in the right eye and 58 mmHg in the left eye. There was moderate conjunctival injection and a fixed mid-dilated pupil in the left eye. The right anterior chamber depth was normal, but the left anterior chamber depth was shallow. Gonioscopy showed a wide-open angle in the right eye, but revealed a closed anterior chamber angle in the left eye. The left cornea was edematous with diffuse microcysts. Posterior segment examination of the right eye was not done because of patient refusal. In the left eye, a large subretinal mass abutting the posterior lens surface was suspected. B-scan ocular ultrasonography and computed tomography () of the left eye showed a large, irregularly reflective mass that was thought to represent a large subretinal mass.
The patient was treated with intravenous mannitol, oral acetazolamide, and topical betoptic, cosopt®, and pilocarpine. However, these regimens failed to relieve the severe ocular pain and the high IOP persisted. did not decrease. The next day, her blind and painful left eye was enucleated under general anesthesia. An intraocular implant was not inserted and her pain was relieved.
The enucleated left eyeball measured 2.5 cm in the antero-posterior dimension and 2.5 cm in vertical length and weighed 5.4 gm. On horizontal section, the shallow anterior chamber and anteriorly displaced swollen lens could be seen (). The cut surface contained a brownish tan colored homogenous gelatinous material. The totally detached retina was adherent to the posterior aspect of the swollen, cataractous lens and a large amount of degenerated blood filled the subretinal and subchoroidal space. Microscopic examination revealed that the anterior chamber was filled with serosanguinous fluid (). The retina was massively detached by degenerated and clotted blood. Blood was also present in the subretinal pigment epithelium.


Case 1. A 31-year-old man presented with left periorbital swelling and diplopia after a blunt orbital trauma (). The man reported being hit by a lump of iron at work the previous day. The patient complained of diplopia in the primary position, left gaze, and downgaze. Hertel exophthalmometry measured 17 mm OU. Initial orbital computed tomography (CT) showed a large left inferior and medial orbital wall fracture extending posteriorly (), and soft tissue was found to be entrapped at the orbital floor fracture site. The patient underwent surgical repair of the medial and inferior orbital fractures. Incarcerated soft tissue was released from the fracture site and a 26×16×1 mm sized Medpor barrier sheet implant (Porex Surgical Products Group, Newnan, GA, U.S.A.) was placed over the defect. One month after surgery, the diplopia disappeared in the primary position but remained in downgaze, especially during adduction. In addition, we detected newly developed left upper eyelid retraction and lid lag on downgaze (). Hertel exophthalmometry measurement was the same as before surgery. An orbital CT scan revealed adhesion between the superior rectus and superior oblique muscle (). The patient was followed for 10 months and the left upper eyelid retraction persisted without improvement.


Case 3. A 44-year-old woman presented with left upper eyelid retraction following periorbital contusion one week prior to initial presentation. Upon examination we noted upper eyelid retraction with a 1 mm scleral show and a lid lag of her left eye (). Hertel exophthalmometry showed no difference between the right and left eyes. Thyroid function testing was performed to exclude thyroid ophthalmopathy, and an orbital CT scan was performed. The thyroid function test included T3, T4, free T4, TSH, TSH receptor antibodies, and antithyroglobulin antibodies. The laboratory results were normal and CT showed mild and diffuse thickening between the left superior rectus muscle and the levator complex (). She was followed for 6 months without improvement of the left upper eyelid retraction.


Case 4. A 56-year-old man visited our clinic for a left orbital contusion and lower canalicular laceration sustained in a traffic accident. His initial orbital CT scan revealed no specific findings, so the following day we repaired the canalicular laceration and inserted a Crawford tube. However, one month after surgery, even though the sutured wound was in good condition, he presented with upper eyelid retraction and a slight lid lag on the left eye ().
Routine laboratory tests, including a thyroid function test, were normal. A second orbital CT scan with enhancement was performed, but no noticeable findings resulted, unlike in cases 1 and 3 (). The patient was not overly concerned about the asymmetry of his upper eyelid. He was followed for 3 months and did not return for subsequent follow-up.


A 66-year-old male visited our ophthalmology department because of decreased visual acuity in his left eye of 10 days duration. The patient had a past history of ocular trauma to the left eye while mowing his lawn 6 months before his visit. He had not been treated because he did not feel any discomfort. On presentation, his best-corrected visual acuities (BCVAs) were 1.0 and 0.4 in the right and left eye, respectively. The intraocular pressure was 11 mmHg in the right eye and 14 mmHg in the left eye. In the right eye, the anterior segment did not show any obvious abnormalities. In the left eye, corneal opacity, which did not involve the visual axis, an incidental intralenticular metallic foreign body, and lens opacity were found. The posterior lens capsule was intact (). There was no inflammation in the cornea or anterior chamber. Funduscopy showed no definite abnormalities in either eye. Emergent surgical removal was performed. Under local anesthesia, capsulorrhexis of the anterior lens capsule and hydrodissection and hydrodelineation of the lens were performed via a corneal incision site. With viscoelastic aid, the intralenticular foreign body was elevated into the anterior chamber. The foreign body was then removed with foreign body forceps. Phacoemulsification and posterior chamber intraocular lens implantation was then performed. The implanted IOL was an AcrySof™ (SA60AT, Optic 6.0 mm, Length 13.0 mm, Alcon, USA). The diameter of the removed foreign body was about 1 mm and it was identified to be metallic by a magnet. It was thought to be a part of the lawn mower blade (). At 3 days postoperatively, the BCVA of the left eye was 1.0. There were no specific findings except trace cell reactions in the anterior chamber. At 17 days postoperatively, the BCVA of the left eye was 1.0. There was no inflammation in the anterior chamber and the IOL was well-seated ().