An 84-year-old male patient with past medical history consisting of hypertension, hypercholesterolemia, type 2 diabetes mellitus, and open angle glaucoma was admitted to ICU via A & E with an unwitnessed collapse and decreased level of consciousness. In emergency department (ED) his GCS was recorded as 5/15, blood sugar was found to be only 34 mg/dL, his heart rate was 34 beats per minutes (bpm), and BP was 58/43 mm Hg. His ECG showed sinus bradycardia with variable blocks including sinus node dysfunction and type 1 Mobitz heart block pattern (Figures and ). He was intubated and ventilated in ED and given dextrose 50% 100 mL and 0.5 mg Atropine. His heart rate rose to 88 bpm and with improvement of heart rate and correction of blood sugar his BP became 135/88 and his GCS improved to 13/15. Unfortunately, heart rate started to drop again to 40 s and blood sugar showed a downward trend for which he was given 1 mg glucagon and was transferred to ICU. By the time he reached ICU his heart rate had improved to 90 bpm. In ICU after 30 minutes his heart rate dropped again so he was given another dose of Atropine and a temporary transvenous pace maker (TPM) was inserted. The patient's blood sugar also improved temporarily but eventually required intravenous infusion of 10% dextrose for the next 12 hours before it was stabilized. His GCS improved to 14/15 and he was extubated the next day 16 hours after admission but repeated attempts to switch off his pace maker revealed underlying brady-arrythmias with heart rate dropping to 34–40 bpm associated with presyncopal symptoms comprising of deterioration in attention and episodes of drowsiness. The pace maker was eventually switched off after 26 hours of observation when no more episodes of bradycardia were observed.
A detailed neurological assessment was carried out after extubation which showed the patient to be confused with no motor deficit. His CT brain was done which showed no abnormality and a detailed history was sought from the patient's wife who pointed out that the patient has been behaving in a weird and confused way for the last 5 days but his wife attributed that to demise of one of his close relative who died a week ago. She further added that he stopped taking his oral medications 2 days before he was admitted but he was given his eye drops regularly by his daughter who feared blindness in case the medication was not given. His drug history consisted of Enalapril 10 mg OD, Atorvastatin 10 mg OD, Metformin 500 mg BID, and Timolol Maleate 0.5% one drop each eye twice a day which was started 35 days before admission to ICU when he was diagnosed as a case of glaucoma.
Tilt table test was not carried out and keeping in view his improvement in blood sugar and heart rate after administration of glucagon his clinical picture of hypoglycemia, confusion, and bradyarrhythmia was recognized to be because of ophthalmic Timolol Maleate which was changed to Travoprost (a prostaglandin F2 analogue) after consulting the ophthalmologist.
The patient was kept under observation in ICU for another 24 hours. His intrinsic heart rate improved to 82 bpm and his TPM wire was removed before transferring to ward. Patient's confusion also improved gradually over the next 3 days and he was discharged on Enalapril, Travoprost, and Metformin. His followup for the next two months was unremarkable.


A 42-year-old male patient presented with complaints of distension of abdomen, decreased urine output and edema of feet. He had a history of melena, paracentesis and upper GI endoscopy with banding of grade 3 varices. He had also history of type 2 diabetes mellitus. He was not alcoholic and his autoimmune antibody profile was negative. Twenty-four-hour urinary copper and serum ceruloplasmin were normal. Kayser Fleischer’s ring was not found on slit lamp examination. His lab findings on presentation included a total billirubin of 1.6 mg/dL (direct 1.0 mg/dL, indirect 0.6 mg/dL), alanine aminotransferase of 55 U/L, aspartate aminotransferase of 35 U/L, alkaline phosphatase of 120 U/L, international normalized ratio (INR) of 1.49, serum albumin of 2.8 g/dL, and a serum creatinine of 1.62 mg/dL. His Child-Turcotte-Pugh score was 8 and Modified End-stage Liver Disease (MELD) score was 11. Once diagnosed having decompensated cryptogenic cirrhosis of liver, he was placed on liver transplant waiting list. He was on diuretic on presentation. He was initially managed with plasma expander and omission of diuretics. Creatinine rose above 2 mg/dL. His urinalysis showed no evidence of microscopic proteinuria or microalbuminuria. All other possible causes of renal failure were ruled out. He was thus diagnosed with HRS type 1. The patient did not respond to combination therapy with albumin and terlipressin and his serum creatinine increased to 5 mg/dL. He was placed on hemodialysis. The patient was kept on hemodialysis for 10 weeks when he received a liver from a cadaver donor. Donor was a 50-year-old brain-dead woman who died of MVA. At that time, considering patient’s renal dysfunction, CLKT was carried out. The patient was maintained on continuous renal replacement therapy during perioperative period. The inferior epigastric artery was preserved to avoid wound-related complications. Postoperatively, the patient did not require any kinds of renal support. After two years of follow-up, the patient was maintained well on tacrolimus, mycophenolate mofetil and steroid.


A 43-year-old male with type 1 neurofibromatosis (NF 1) was referred to our hospital because of an increasing subcutaneous tumor of the right postauricular region, which was partially resected with positive margin and histologically proven as MPNST at the previous hospital (Figures –). The patient did not complain of right facial nerve paralysis or hearing impairment. Magnetic resonance imaging (MRI) demonstrated residual periauricular subcutaneous ill-defined lesions extending radially from the stylomastoid foramen and subsequent abnormal enhanced lesions in the intratemporal facial nerve from the stylomastoid foramen to the internal auditory canal (Figures –). Computed tomography (CT) of the temporal bone showed the subcutaneous lesions from the enlarged stylomastoid foramen and the enlarged facial canal (Figures –). On the basis of these findings, the patient was strongly suspected of having a MPNST that had spread proximally along the facial nerve to the internal auditory canal. In order to obtain clear surgical margins, CyberKnife stereotactic radiosurgery was undertaken with a single dose of 23 Gy to the right internal auditory canal. Thereafter, the patient was treated with subtotal temporal bone resection, right neck dissection, and reconstruction with the rectus abdominal muscle free flap. Postoperative histopathological examination demonstrated no residual MPNST without lymph node metastasis but neurofibroma involving the subcutaneous tissue and the entire portion of the facial nerve canal (Figures and ). The patient developed dysphagia because of incomplete lower cranial nerve palsy, in addition to 7th and 8th cranial nerves loss. After rehabilitation and surgical treatment with laryngeal elevation and cricopharyngeal myotomy, he gradually resumed eating. Although postoperative adjuvant therapy was not delivered, there was no recurrence at follow-up 24 months after surgery.


An eight years old girl has presented to pediatric emergency with a recent history of febrile odynophagie subsequently complicated by a torticollis that has settled 5days after. Clinical examination has found a febrile child at 39°C and a bulging anterior pillar of the right tonsil without rash or conjunctival hyperemia. CT scan was performed and has revealed right peritonsillar and parapharyngeal abscess (). At laboratory tests we found a white blood cell count (WBC) of 14000/mm3 and C - reactive protein (CRP) of 102mg / l. The child underwent a trans-oral drainage of the abscess under general anesthesia and airway protection. The bacteriological sampling objectified mixed unspecific flora sensitive to amoxicillin-clavulanic acid with no evidence of mycobacterium tuberculosis. Antibiotherapy was prescribed based on amoxicilline- clavulanic acid (80mg/kg a day) and aminoglycoside (3mg/kg a day for 5 days). The child was apyretic 48 hours after, and C- reactive protein 72 hours later was at 45 mg/l. Cervical spine traction was not necessary since the torticollis has been resolved after 5 days.


A 2 years old male infant has presented to pediatric emergency with an acute febrile torticollis that has occurred 3 days after a rhinopharyngitis onset. Physical examination has found febrile and eupneic child who has a stable hemodynamic status. Physical examination has shown a bulging posterior wall of the oropharynx with several lenticular cervical lymph nodes. CT scan () has revealed a retropharyngeal abscess with straightness of the cervical spine. The child underwent a trans-oral drainage of the abscess under general anesthesia. According to bacteriological findings the patient has received intravenous antibiotherapy based on cephalosporin of 3rd generation (Ceftriaxone 50mg/kg a day) and aminoglycoside (3mg/Kg a day) with good outcome on both clinical and biological levels. The child was discharged from the hospital after a week. Antibiotherapy has been taken for three weeks. The straightness of the spine has been resolved after surgical drainage of the abscess.


A 5 years old boy has presented to pediatric emergency with febrile odynophagia and torticollis that has occurred 2 days after he has eaten a prickly pear, no diarrhea or vomiting has been reported. The physical examination has found a febrile child with a right torticollis and no evidence of upper airways infection. Cervical and parapharyngeal CT scan () has revealed a foreign body that was a thorn with cervical abscess. At laboratory tests we found a WBC of 20000/mm3 and a CRP of 143mg/l. The child underwent surgical drainage of the abscess and removal of the thorn. Post operative course was uneventful. No miro-organism has been found in bacteriological samples, so the child received a wide spectrum antibiotic therapy based on cephalosporin of 3rd generation (Ceftriaxone 50mg/Kg a day) aminoglycoside (3mg/ Kg a day for 5 days) and metronidazole (40mg/Kg a day). Clinical, biological and radiological outcome was comforting (). The patient was discharged from the hospital four days after. The antibiotics have been taken for 3 weeks.


27-year-old male patient referred to our clinic complaining about his right upper third molar. According to clinical and radiographical examination, moderate infection was inspected, caused by wisdom molar. In order to cure infection and its symptoms the tooth was decided to be extracted. Suture needle was found to be broken while passing through the palatinal mucosa. Although it was tried to localise the broken needle fragment, it could not be managed to reach it in deep mucosal area. The broken fragment could not be prevented from migrating to deeper tissues while attempting over reaching. The flap was closed and the patient was gone under further diagnostic examination.
3D cone beam computed tomography (CBCT) (ProMax 3D Mid Machine, Planmeca Oy, Helsinki, Finland) and conventional OPTG were performed to localize the needle. According to tomographic images, location of needle was about under the palatinal mucosa, the region of right maxillary tuberosity, nearly just below the medial pterygoid plate. The broken fragment was detected distant from the localization it had been broken at the first place (Figures , , and ).
After detecting the exact location of the needle, the patient has gone under a second surgery immediately and the needle was reached with a more extensive incision of 35 mm from former horizontal incision of third molar surgery through buccal mucosae inferiorly. Blunt dissection was made through oropharyngeal region and the broken fragment was removed successfully ().


A 4-year-old, male Hannoverscher Schweisshund dog was referred to the same Hospital for severe respiratory distress and acute onset of dyspnoea. Hematological exam revealed a mild normocytic, normochromic, non-regenerative anemia in association with marked leucocytosis, marked absolute monocytosis, neutrophilia and left shift. Platelet and eosinophil concentrations were in the reference limits. Given the severe clinical picture and the hematologic abnormalities of the dog, an infective process, as a bacterial pneumonia, or a lung injury were first suspected. Radiographic evaluation of the thorax showed a marked generalized unstructured interstitial pattern. No sign of pulmonary hypertension could be detected, while lung sonography showed the presence of small scattered focal lesions in the pulmonary parenchyma and signs of hepatization of left caudal lung lobe (Fig. ). Diagnostic imaging lesions were suspect for a lungworm infection and specific tests for A. vasorum were performed. Evaluation of a direct fresh fecal smear revealed numerous nematode larvae morphologically compatible with A. vasorum. The infection was further confirmed by a positive antigenic test (IDEXX Angio DetectTM). After diagnosis the dog was immediately treated with a spot-on solution containing imidacloprid 10%/moxidectin 2.5% (Advocate®, Bayer Animal Health) in association with glucocorticoids and specific support therapy. The dog died six hours after therapy administration in a severe dyspneic status with profuse hemoptysis. Baermann’s test confirmed A. vasorum infection the day after.


A 6 month-old female Akita Inu was referred to a Veterinary Hospital in Novara municipality (northern Italy, Piedmont region) with a history of inappetence and chronic hemorrhages. Clinical examination revealed several foci of conjunctival hemorrhages, skin ecchymoses and petechiae, with no other clinical abnormality. Therefore, the first clinical suspect was an anticoaguant rodenticide poisoning. Nonetheless this suspicion was immediately discarded for the normal values of prothrombin time (PT) and activated partial thromboplastin time (aPTT). A complete blood count revealed a mild normocytic, hypochromic, non-regenerative anemia in association with moderate absolute monocytosis, moderate absolute eosinophilia and a severe thrombocytopenia, which was individuated as the cause of the primary hemostatic disorder showed by the dog. Radiographic examination of the thorax revealed a generalized unstructured interstitial to alveolar pattern compatible with lung hemorrhage or acute respiratory distress syndrome, which was considered the most probable differential due to the hemostatic disturbance of the dog (Fig. ). No evidence of cardiomegaly was radiographically detected. Therefore, an inherited, infective or immune-mediated cause of thrombocytopenia was suspected. Nonetheless, no predisposition for low platelet concentration is recognized in Akita Inu breed and specific tests for common infective or parasitic causes of thrombocytopenia (i.e. Ehrlichia canis, Anaplasma platys, Anaplasma phagocytophilum, Babesia spp.) were negative. The dog was then examined for A. vasorum, despite no records of this parasite existing in the Piedmont region of Italy. The dog tested positive at the IDEXX Angio Detect™ Test and this finding was confirmed by a Baermann test positive for A. vasorum. The dog was also positive at an antiplatelet antibody test. The dog was treated with fenbendazole 50 mg/kg sid for two weeks and tested negative for Baermann’s test two weeks after the end of the therapy. Suggested immunosuppressive approach was declined by the owner.


A 7-month-old female mix breed dog was referred to the same Veterinary Hospital of Case 4, with a history of anxiety, vocalizations, labored breathing in the previous day and an acute onset of paraplegia. Clinical examination revealed several neurologic deficits at the posterior limbs. Given the young age of the dog, the differential diagnoses included discospondylitis, myelitis or a compressive cause, with the former as the most likely diagnosis. Blood biochemistry and PT were in normal ranges and aPTT was slightly higher than the upper reference limits. Computed tomography (CT) of the vertebral column revealed an endocanalar hyperattenuating enhancing material with multifocal extra-dural distribution in the thoraco-lumbar tract compressing the spinal cord (Fig. ). Moreover a diffuse interstitial lung pattern with enlarged and tortuous pulmonary arteries was seen. Despite first clinical suspects, diagnostic imaging findings make the clinician suspicious of A.vasorum infection as the cause of the lung lesion and pulmonary hypertension, therefore a rapid antigenic test (IDEXX Angio Detect™) for A.vasorum was performed giving a positive result. The endocanalar multifocal material seen in CT images was interpreted as hemorrhages or granulomatous lesions caused by larval dissemination of the lungworm. The infection was further confirmed positive by Baermann’s test. The dog was treated with fenbendazole 50 mg/kg sid for two weeks and tested negative for Baermann’s test two weeks after the end of the therapy. After the specific antiparasitic and support therapy, the clinical picture of the dog improved dramatically.


Patient is a 41-year-old female originally from Nigeria, immigrated to the United States 9 years ago with no past medical history, who presented to the emergency department with worsening lower extremity weakness and swelling for 3 months. She also complained of 2 days of right eye swelling with diplopia and blurry vision and 1-day history of an inability to walk. Patient reported a flu-like illness with diarrhea prior to symptoms 5 months ago. She works as a house remodeler and denied any exposure to mold, asbestos, heavy metals, and silica. She gave no history of skin rashes, mouth ulcers, hair loss, photosensitivity, or arthralgia. On initial physical examination vital signs were stable; oxygen saturation was 98% on room air. There was no evidence of skin rash, she denied joint tenderness, and there was no palpable swelling. Heart lungs and abdominal exam revealed no abnormalities. There was bilateral lower extremity pitting edema. The neurological exam revealed that she was alert and oriented x3, followed commands, and had no aphasia with intact comprehension and fluent speech. Cranial nerve examination revealed anisocoria right > left, extra ocular muscles intact, and no nystagmus and the rest of cranial nerves were normal. Motor exam revealed 2/5 strength in the proximal upper extremities (abduction and flexion) and 5/5 in wrist flexion and extension. Hip flexion was 2/5 on the right and 3/5 on the left. Knee extension and flexion, dorsiflexion, and plantar flexion were 5/5 bilaterally. Reflexes were equal and symmetric in upper extremities but diminished in lower extremities.
Laboratory data revealed leukopenia 4.3 K/mcl and anemia with Hg/Hct at 6.6/19.5 g/dL, respectively; platelets were within normal limit. Liver function panel was also within normal limits except albumin at 2.3 g/dL. Prealbumin was low at 8.1 mg/dL and BUN/creatinine was at 29/1.29 mg/dL, respectively. Urinalysis revealed protein of 300 and RBC of 11–25/hpf. Head CT, MRI brain, and C-spine and T-spine CT scans revealed no abnormalities. CT thorax was obtained which revealed moderate bibasilar pleural effusion and moderate pericardial effusion. Echocardiography revealed ejection fraction of 60%.
Four days after admission, the patient's neurological status worsened. She became progressively weaker and lethargic and was only able to state her name. She had difficulty swallowing, speaking, taking deep breaths, and coughing. She was intubated for airway protection and placed on nasogastric tube (NGT) feeding. Neurologist evaluated the patient; exam was notable for absent reflexes in addition to progressive lower extremity weakness; GBS was suspected. She also developed ophthalmoparesis with inability of the eyes to cross the midline bilaterally. Stool studies were negative for Campylobacter jejuni and were guaiac negative. Lumbar puncture and plasma exchange were recommended. Cerebral spinal fluid (CSF) analysis revealed protein of 35 mg/dL; WBC was 8/cumm. Oligoclonal bands and myelin basic protein were absent, with Anti-GQ-1 antibody titers less than 1 : 100. West Nile viral titers were negative. Nerve conduction studies revealed absence of F wave response in right peroneal nerve, prolonged distal onset latency, and severe conduction block (); although CSF results were inconsistent with GBS as the protein was not elevated, a diagnosis was made clinically. Treatment with plasma exchange was begun 5 days after admission. She received 5 plasma exchanges with no improvement.
Due to the presence of leukopenia, pericardial and pleural effusions, proteinuria, and hematuria SLE work-up was sent with results showing the following: +ANA titers of 1 : 2560 4+ speckled, Anti-ds-DNA positive titer 1 : 80, and AntiSmith ab positive >8, low C3 and C4 levels at <40 mg/dL and <10 mg/dL, respectively. Serum antiribosomal P antibodies positive >8, serum antineuronal antibodies were negative, and lupus anticoagulant and anticardiolipin antibodies were negative. Patient fulfilled ACR SLE criteria and Pulse SoluMedrol therapy 1 gm daily for 3 consecutive days was started 7 days after admission. Renal function was rapidly deteriorating with anuria at 67 mL in 24 hours and worsening of BUN/creatinine to 50/3.29 mg/dL, respectively; hemodialysis (HD) was started 8 days after admission. Renal biopsy was performed which showed evidence of membranous and focal lupus nephritis [ISN/RPS 2004 classification lupus nephritis, classes III (A) and V], acute tubular necrosis ().
SoluMedrol was given on alternate days with plasma exchange and hemodialysis to avoid removal and maintain optimal serum levels of steroids. SoluMedrol was continued as maintenance therapy on 120 mg IV daily.
Cyclophosphamide 500 mg every 2 weeks as per EURO Lupus protocol was started 10 days after admission due to lupus nephritis. Patient failed multiple weaning trials and subsequently needed PEG tube and tracheostomy tube. Plasma exchange was stopped after 5 sessions on day 14 of admission due to the absence of improvement in neurological status. Patient was started on IVIG on day 17 of admission. Patient received 1st course of IVIG 0.4 g/kg/day for 5 days concomitantly with SoluMedrol maintenance therapy on 120 mg IV daily and cyclophosphamide. Renal function began to improve with resolution of anuria; hemodialysis was discontinued. Due to significant SLE serology improvement with normalized C3 and C4 and negative ds DNA and improved renal function off hemodialysis, SoluMedrol was tapered to 40 mg IV daily.
Cyclophosphamide was terminated after the 3rd dose, 39 days after admission as the patient developed pancytopenia and fever with pneumonia and worsening of the sacral decubitus ulcer. In view of SLE with positive antiribosomal P protein and lack of improvement in the neurological/GBS symptoms (no improvement in muscle strength, ophthalmoparesis, or reflexes) and with patient still being dependent on mechanical ventilation and PEG tube feeding, a 2nd course of IVIG was started 2 g/kg divided over 5 days. Her weight was 60 kg and she was started on a 2nd course of IVIG 24 g/day, 68 days after admission. The choice of therapy and the onset of treatment are summarized in .
Significant daily improvement in motor function and reflexes started to occur. Patient was able to start bedside physical therapy. Tracheostomy tube was subsequently removed as well as PEG tube. Patient was transferred to the inpatient acute rehabilitation unit for 2 weeks and was discharged home 131 days after initial presentation on prednisone 20 mg daily. Patient was seen in the rheumatology and neurology clinics within 1 month after discharge, with no significant residual motor or sensory deficits, walking without support, and asymptomatic for weakness. C3 and C4 levels were normalized at 100 mg/dL and 22.6 mg/dL, respectively. The patient was noted to have hematuria and low complement levels on subsequent clinic visits 2 months after and was started on mycophenolate mofetil in addition to prednisone.


A 7-year-old girl presented with a 1-week history of swelling in the right anterior neck and submandibular area. Her past history and family history were noncontributary. Her growth pattern and development were also normal. Inspection of the neck revealed swellings in the right upper and left submandibular areas with no distortion of the overlying skin (). Physical examination revealed a fixed, hard, mildly tender pretracheal mass, 1.5x2.0 cm in size and another fixed, hard submandibular mass, 0.5x0.5 cm in size. There was no enlargement of the cervical lymph nodes. The remaining findings of the physical examination were unremarkable.
The masses were evaluated using ultrasonography (USG) and computed tomography (CT). USG examination revealed well-defined masses with heterogeneous echogenicity and the thyroid gland was not in the expected cervical location. Neck CT revealed aberrant thyroid tissue as a 1.3x2.3 cm, round-shaped, high-density mass in the right pretracheal area and another 1.4x1.6 cm mass with the same characteristics in the region of the tongue base (). A 99m-technetium pertechnetate thyroid scintigraphy was performed, which demonstrated two ectopic areas of uptake (upper anterior neck and lingual areas), confirming dual thyroid ectopia (). There was no uptake in the normal anatomical location of the thyroid gland. Based on these findings, a diagnosis of dual ectopic thyroid was made. Biochemical determinations were consistent with a state of subclinical hypothyroidism. Serum triiodothyronine level was 1.7 ng/mL (normal, 0.9-2.4 ng/mL); free thyroxine (fT4), 17.2 pmol/L (normal, 10-28 pmol/L) and thyroid-stimulating hormone (TSH) level was 9.49 mIU/L (normal, 0.7-6.4 mIU/L).
Following the diagnosis, the patient was treated conservatively with oral levothyroxine, 0.05 mg per day. After treatment, she remained asymptomatic, the masses slightly decreased in size and repeated thyroid function tests were found to be within the normal limits.


A 27 year-old Hispanic female presented at 35 weeks gestation with a recent onset of lower quadrant abdominal pain and a rapidly increasing abdominal girth over a one-week period. At 35 weeks gestation her fundal height had increased to 37cm and her flanks were distended with a positive ascitic fluid wave. A transabdominal ultrasound revealed two large adnexal masses with both solid and cystic components and moderate ascites. The right adnexal mass measured 13.0×18.7×11.0cm, while the left adnexal mass measured 5.2×6.0×4.5cm (). There was increased blood flow to both ovaries, with a reduced resistant index of 0.31 (). An abdominal computed tomography scan confirmed bilateral ovarian masses () and revealed no evidence of intraperitoneal metastasis or omental caking. Tumor markers were drawn to rule out hormonally active ovarian tumors. CA-125 was elevated at 698U/mL (mean level during pregnancy is 204U/ml at term). Her serum testosterone was <16ng/ml (normal 0.1–0.9ng/ml), percent free testosterone was slightly elevated at 49% (normal 25%-35%), and free testosterone <8.0ng/ml (normal 0.002–0.2ng/ml). The remainder of the hormone profile included normal CA19-9, CEA, and Inhibin.
An ultrasound-guided paracentesis drained 250μL of serous fluid, which was negative for malignant cells. A preliminary diagnosis of ovarian neoplasm of unknown etiology and the decision to perform exploratory laparotomy to biopsy, debulk, and stage the ovarian tumor in the postpartum period was made. Within a couple of days of admission, the patient delivered a healthy female baby vaginally with no evidence of neonatal virilization. In the immediate postpartum period, the patient’s abdomen grew increasingly tense and distended and she developed intermittent ovarian torsion. An emergency exploratory laparotomy revealed three liters of ascitic fluid. A right oophorectomy yielded a 20 cm ovarian mass, which traversed the entire ovary. The right ovary weighed 2400g and had a soft, yellow consistency when cut (). The mass was homogenous with no cystic lesions, but some areas of infarction were present. The intraoperative frozen section showed steroid producing cells, most likely a steroid cell tumor, but possibly bilateral luteomas. Since the vast majority of steroid cell tumors have a low-grade malignant potential, the left ovarian mass was resected, but any remaining viable ovarian tissue was preserved, in order to preserve fertility as desired by the patient. There were no enlarged lymph nodes. An infracolic omentectomy was performed and no gross evidence of metastatic disease was present.
Permanent sections of pathology tissue, however, demonstrated signet-ring cells of epithelial origin, staining positive for mucin and cytokeratin () consistent with Krukenberg’s tumor. The ascitic fluid and the omentum were negative for malignancy. An interval upper gastroendoscopy revealed poorly differentiated signet-ring cells located in the cardiac region of the stomach, confirming the gastric origin of the Krukenberg tumor (). The patient was started on Oxaliplatin, 5-Florouracil, and Calcium Leucovorin chemotherapy. She was discharged home in stable condition on the 6th postoperative day. Nine months after surgery after surgery the patient remains in partial remission, but subsequently developed cancer progression, necessitating further gastric surgery and chemotherapy. Her daughter remained well and healthy.


The patient was an 18-year-old African American female with modestly controlled hemoglobin SC disease. She presented with severe lower back and bilateral leg pain. Her medical history was unremarkable except for a limited number of admissions for pain crisis, a positive sexual history, and a recent tattoo obtained two weeks prior to admission. Four days prior to presentation, she had undergone an elective erythrocytapheresis procedure in preparation for wisdom teeth extraction under general anesthesia. Her home medications included penicillin VK and regular intramuscular medroxyprogesterone acetate injections. Review of other systems was negative.
Initial studies (complete blood count, urine pregnancy test, urinalysis, and lumbar spine radiographs) were within normal limits (including hemoglobin and hematocrit of 11.3 g/dL and 32.5%, resp.). The patient was admitted for intravenous fluids and opioid analgesics; yet her pain remained poorly controlled. The patient was given azithromycin and ceftriaxone for uncomplicated cervicitis suggested by pelvic exam. She subsequently became febrile to 39.2°C and developed increasing drowsiness. Repeat laboratory studies revealed hemoglobin of 9.8 g/dL, hematocrit of 28%, and platelets of 91 × 109/L and sodium of 127 mEq/L.
On hospital day 2, the patient developed dizziness, tachypnea, and mild hypoxia. Significant new laboratory findings included elevated aspartate amino transferase (248 U/L) and C-reactive protein of 215.2 mg/L (normal < 5 mg/L). Antibiotics were adjusted to cefepime and vancomycin, and the patient was transferred to the pediatric intensive care unit.
Head CT was normal and CT of the abdomen and pelvis was unremarkable except for small bilateral pleural effusions. At that time, meropenem, acyclovir, and doxycycline were added to the patient's antibiotic regimen. Due to progressive neurological and cardiovascular decline, the patient was endotracheally intubated and started on cardiovascular support. An echocardiogram revealed right-sided enlargement and elevated pressures (two-thirds of systemic circulation).
Thrombocytopenia as well as abnormal coagulation labs, including a D-dimer, suggested disseminated intravascular coagulopathy. Despite worsening anemia, hemoglobin electrophoresis obtained on the second day of hospitalization revealed a hemoglobin S of only 9.1% reflective of recent erythrocytapheresis procedure.
The patient's cardiovascular and neurological status further deteriorated with eventual loss of brainstem reflexes and EEG activity. On hospital day 7, she was declared brain dead. Results of serology testing returned postmortem showed parvovirus B19 PCR 300 copies/mL (normal < 100 copies/mL).


61-year-old lady, right hand dominant homemaker, presented with a four-year history of right anterior elbow pains localised in the antecubital fossa associated with swelling and intermittent paraesthesia in the radial border of forearm extending towards the thumb limiting her thumb flexion at the distal interphalangeal joint. She denied any forms of trauma and symptoms of osteoarthritis and rheumatoid arthritis and was otherwise systemically healthy. She complained of pain on active elbow flexion and both pronation and supination. Prior to presentation to the orthopaedic outpatient clinic, she described a lengthy period of conservative management through simple analgesia including oral paracetamol, oral nonsteroidal anti-inflammatories, and physiotherapy.
On examination, a palpable mass was felt in the right antecubital fossa measuring roughly 5 cm in diameter fixed to the underlying soft tissue structures. There was pain on active elbow flexion and pronation, with reduction of range of motion in both ranges. Elbow extension and supination were neither painful nor limited.
Strength was equal to the contralateral side; however pain was elicited on all resisted movements. There was no vascular compromise, but reduced cutaneous sensation on the radial side of the thumb, corresponding to the distribution of the superficial radial nerve.
A plain radiograph was performed () which showed an irregular circumscribed radioopaque lesion overlying the proximal radius, in close proximity to the radial tuberosity and distal to the radial head. It was reported as a possible osteochondroma. In view of this inconclusive diagnosis a computer tomography (CT) scan was then performed. It also reported an indeterminate sessile osteochondroma. However, despite the overall morphology, given that the corresponding medullary cavity appears to be arising off the cortex, a differential diagnosis of a bony enthesopathic reaction was considered; thus magnetic resonance imaging (MRI) was therefore recommended.
MRI before and after gadolinium confirmed the appearance of an 8 mm by 9 mm fluid collection compatible with bicipitoradial bursitis and ongoing synovitis between the biceps brachii tendon and the radial tuberosity (Figures , , and ). Furthermore, there was evidence of distal biceps enthesopathy with associated adjacent bony changes. The biceps tendon was reported to be completely intact.
An ultrasound guided aspiration was performed () with aspiration of 3 mL of clear synovial fluid. There was no evidence of microbial infection nor malignancy from the fluid sample. She was reviewed in clinics after aspiration but her symptoms remained. It was thus decided to proceed with surgical exploration of the antecubital fossa.
Under tourniquet, a linear incision over the right antecubital fossa was performed distal and radial to the biceps brachii tendon. A combination of gentle blunt and sharp dissection through tissue layers was employed towards the direction of the biceps tendon insertion until exposure of the synovial bursa and biceps tendon (Figures and ). The superficial and deep radial nerve branches were identified and spared throughout the bursa dissection (). The surgical margins of the lesion did not extend beyond the radial tuberosity and was engulfing the biceps brachii tendon insertion. Laterally were the supinator and extensor carpi radialis longus muscles; medially was the pronator teres. The synovial cyst and bone mass with a chondral surface were dissected piecemeal and sent for histology. The radius bone was reached after complete removal of the bursa (). Soft tissue layers were closed with absorbable sutures (vicryl); skin was closed with nonabsorbable sutures (prolene). Postoperative care involved immobilisation of the elbow to 90 degrees and a shoulder immobilizer. She was discharged the following day with follow-up plan. She was reviewed at outpatient clinics one week after operation and reported improved symptoms. Subsequent histology reported a layer of synovial tissue with a thin overlying cartilage cap confirming an osteochondroma with bicipitoradial bursitis.
She was followed up after one month and then six months with near complete pain cessation along with improvements in ranges of elbow flexion and pronation. Follow-up plain radiographs show disappearance of the lesion ().


A 40 year old lady from Kosovo, was referred to our hospital for severe pulmonary arterial hypertension and a mass in right atrium suspected for vegetation. About one month before, she was admitted in another hospital and received iv medication. The patient was febrile and the C-reactive protein, white cell count and erythrocyte sedimentation rate were elevated. Blood cultures demonstrated a methacilin sensitive Staphylococcus aureus growth.
Transthoracic echocardiograhy demonstrated a mobile, irregularly shaped, oscillating and highly mobile mass, located above the tricuspid valve septal leaflet (Fig. ). A clear jet across a small defect between left ventricle and right atrium consistent with Gerbode type defect was identified. The direction of the Doppler signal also leads to the true diagnosis (Fig. ). Cardiac magnetic resonance demonstrated a supravalvular flow associated with infravalvular jet according to the type C acquired Gerbode defect (Fig. and ). A normal lung scan excluded pulmonary embolism. The tricuspid regurgitation was considered mild- to- moderate with estimated pulmonary arterial systolic pressure about 60-80 mmHg.
The patients underwent surgery after 2 weeks of antiobiotic therapy. Through a right atriotomy, large vegetation was attached to the septal leaflet and anterior leaflet of tricuspid valve was identified. On removal of the vegetation, a defect was found communicating between the left ventricle and right atrium (Fig. and ). This defect represented an acquired Gerbode defect and was closed by two 5/0 pledgeted prolene sutures (Fig. ). Then the septal leaflet of tricuspid valve was resected and was replaced with a trimmed autologous pericardial patch. Anteriorly the newly created septal leaflet was attached to the anterior leaflet. Then, two synthetic chorda were employed (Fig. ). The hydraulic maneuver demonstrated trivial tricuspid valve regurgitation (Fig. ). Then the right atrium was closed. After an uneventfully post-operative period, the patient was discharged home in good clinical condition. Echocardiogram demonstrated trivial tricuspid valve regurgitation and no residual shunt. One year later the patient was doing well. The transthoracic echocardiography at follow-up demonstrated a moderate tricuspid valve regurgitation and no residual shunt.


A 44-year-old, right-handed female presented with sudden hand motor deficits in her left hand. There was no trauma to the arm, she used no medications and she had no vascular risk factors.
Examination revealed severe motor deficits of the left hand with extension of the three ulnar fingers and wrist, muscle atrophy in the first dorsal interosseous muscle, and claw hand deformity without sensory deficits (Fig. ). According to the Medical Research Council scale examination revealed grade 1 in the left wrist flexor, grade 4 in the left wrist extensor, grade 3 in the left fingers flexor and grade 4 in the left fingers extensor. Adduction and abduction of the left fingers were also severely impaired (grade 3). Deep tendon reflexes were mildly brisker ipsilateral to the affected hand. No Babinski sign was observed.
Cervical computed tomography (CT) was unremarkable. Peripheral motor nerve conduction velocities were normal. Small, discrete ischemic lesions appeared hyperintense on diffusion-weighted magnetic resonance imaging (MRI) sequence (upper arrows on Fig. ). The infarct was located in the right posterior part of the precentral hand knob area (upper arrow on 1d), near the central sulcus (lower arrow on Fig. ). CT angiography showed significant stenosis in the right proximal middle cerebral artery (MCA) trunk.
Transesophageal echocardiogram and carotid ultrasound did not suggest an embolic source. Hypercoagulable screening and levels of markers specific to systemic vasculitis were all normal. An embolism arising from the ipsilateral MCA stenosis was subsequently considered as the etiology of the patient’s symptoms, and she received acetylsalicylic acid and clopidogrel for secondary stroke prevention.
At the 2-month follow-up, the patient complained of fatigue and progressive headaches. Cerebrospinal fluid analysis was normal. Follow-up 3-Tesla MRI with gadolinium contrast revealed focal wall enhancement in the right proximal MCA (Fig. ), indicating cerebral angiitis. Multiple stenoses at the same location were seen on digital subtraction angiography (DSA) (Fig. ). Brain biopsy was not performed.
Cyclophosphamide infusions were administered with glucocorticoids over the subsequent 15 weeks. Left-hand motor function improved, aside from her left fifth finger.


An otherwise healthy 23-year-old Caucasian man presented to our Emergency Department reporting a sensation of “bubbles” under the skin of his neck and mild chest pain. He denied any dyspnea, neck pain, dysphagia, odynophagia, or dysphonia. He reported that he had “snorted” a small amount of cocaine two nights prior to his Emergency Department presentation. He did not have a history of chronic or regular drug use. There was no history of any trauma or vigorous physical exercise. He had mild rhinorrhea but no coughing or vomiting. He did not take any regular medication, but reported the occasional use of illicit drugs such as cocaine and ecstasy.
On examination, he was alert and responsive without any airway compromise or respiratory distress. His pulse rate was 96 beats per minute with a blood pressure of 120/73mmHg. He had a respiratory rate of 18 breaths per minute with oxygen saturation of 97% on room air and he was apyrexial. Subcutaneous crepitations were palpable across his neck and the superior part of his chest. The rest of his physical examination was unremarkable.
Results from baseline blood tests, including arterial blood gases, were unremarkable and an electrocardiogram showed a normal sinus rhythm with no arrhythmias or segmental changes. A plain posterioranterior chest radiograph revealed subtle pneumomediastinum and soft tissue emphysema at the base of his neck with no associated pneumothorax (Fig. ). A plain radiograph of his neck demonstrated extensive subcutaneous emphysema (Fig. ). Computed tomography of his neck and chest was performed, and confirmed the above radiographic findings (Figs. and ). No additional sequelae of cocaine use, such as granulomas, emphysema, or bullae, were seen on pulmonary imaging. In order to rule out any esophageal involvement, our patient was transferred to a tertiary center for a barium swallow fluoroscopy study, which was normal (Fig. ).
He was admitted under the care of the cardiothoracic surgery team at the tertiary center. He was managed conservatively and observed for a further 24 hours, during which the subcutaneous emphysema gradually improved although did not resolve completely. He was discharged home after a total of 48 hours in hospital. He was followed-up in the community by his general practitioner and was found to be well 3 months later.


A 72-year-old female, exsmoker, presented to accident and emergency department with a 12-hour history of acute onset, sharp right sided abdominal pain associated with bilious vomiting. This occurred on a background of a recent aortic valve replacement (porcine valve) for critical aortic stenosis, currently warfarinised. Her past medical history included an open appendicectomy and open cholecystectomy. On examination, the patient was markedly cachexic, pulse was irregularly irregular at 140 beats per minute, and blood pressure and temperature were normal. Abdominal examination revealed localized peritonitis in the right iliac fossa. Blood results showed a markedly raised white cell count at 33.42 with an INR of 2.1. Hemoglobin, urea and electrolytes, amylase, and liver function tests and arterial blood gas were all normal. Abdominal CT scan with contrast demonstrated inflammatory changes of the pericolonic fat around the caecum (). Marked narrowing of the celiac artery and complete occlusion of the SMA were noted. The inferior mesenteric artery was markedly hypertrophied with extensive collateral vessels to the bowel. No arterial thrombi or emboli were seen. The patient was consented for an urgent laparotomy. Intraoperatively a well-demarcated ischaemic caecum was noted with early signs of necrosis. Left colon, splenic flexure, and small intestine were normal. Right hemicolectomy was performed with primary anastomosis. This was combined with a defunctioning loop ileostomy, created in the right iliac fossa. Postoperatively the patient was commenced on a heparin infusion. Bowel function gradually returned, the stoma functioned normally, and normal diet was introduced. However, the postprandial abdominal pain, nausea, and vomiting persisted. Upon further questioning, she recounted an 18-month history of chronic prandial abdominal pain, vomiting, and weight loss. An aortogram () was performed which confirmed occluded SMA and small calibre celiac axis with high grade stenosis. A markedly hypertrophied inferior mesenteric artery was also found with over 90% stenosis at its origin. Extensive collaterals were seen via marginal branch of Drummond and the Arc of Riolan which supplied the small intestine. Owing to the two recent major operations and the fact that the patient was severely malnourished, she was deemed high risk for complete visceral revascularisation which would have involved prolonged aortic cross clamping. It was therefore decided for single vessel revascularisation of the IMA via a common iliac to IMA bypass. The patient was returned to theatre for an iliomesenteric bypass. A laparotomy was performed and the right common iliac artery and inferior mesenteric artery were identified and isolated. The long saphenous vein was harvested from the right leg and was reversed and anastomosed end to side from the proximal right common iliac artery to the proximal inferior mesenteric artery (). Clamp time during this procedure was 20 minutes with the distal IMA continuously infused with saline/heparin. The patient was transferred to ICU and commenced on IV heparin. Postoperatively, patient made an uneventful recovery and was successfully introduced to a normal diet. After two weeks, she was discharged home on aspirin. At two-year follow-up the patient remains asymptomatic and had regained her lost weight. A duplex ultrasound revealed excellent flow in graft and a reversal of ileostomy is planned.


A 66-year-old man with hemoptysis was referred to our hospital because of a pulmonary malignant tumor. A bronchoscopy revealed a tumor arising from the right middle lobe bronchus (Fig. ), and a histological diagnosis based on a bronchial biopsy revealed a malignant melanoma. A positron emission tomography-computed tomography (PET-CT) scan revealed no other lesions that could have represented a primary site (Fig. ). A right middle lobectomy with mediastinal and hilar lymphadenectomy was performed. Some tumor cells had melanin in the cytoplasm (Fig. ). Immunohistochemical analysis showed positive cytoplasmic staining of the tumor cells for HMB-45, Tyrosinase, and MART1, although the expression of TTF-1 in tumor cells was negative. The postoperative course was uneventful. Combination chemotherapy of dacarbazine, nimustine, and vincristine was performed. At the time of the 22 months after surgery, he was alive without disease recurrence.


A 46-year-old woman was found to have an abnormal shadow on a chest X-ray during an annual check-up. A CT and PET-CT scan showed a tumor in the left lower lobe of the lung (Fig. , ). Pathological examination of a specimen obtained during a transbronchial lung biopsy revealed that the tumor was a malignant melanoma (Fig. ). Because further examinations did not reveal any other tumors that could have been the primary lesion, we diagnosed the lung tumor as a primary pulmonary malignant melanoma. A left lower lobectomy with mediastinal and hilar lymph node dissection was performed. Immunohistochemical staining was negative for S-100 protein, chromogranin, synaptophysin, keratin, and TTF-1 but positive for HMB-45 and vimentin. The postoperative course was uneventful. She refused to receive adjuvant chemotherapy. Eight months after the pulmonary resection, a PET-CT scan showed the uptake of fluorodeoxyglucose in the cecum (Fig. ). An ileocecal resection was performed, and the pathological examination indicated that the tumor was considered to be the recurrence of a melanoma.


A 3-year-old boy was referred to our hematology clinic because of ecchymosis on the legs and arms for 2 weeks in 2012, July. Medical history was unremarkable. Physical examination revealed ecchymosis on the legs, arms, and back. Muscle atrophy was evident on the upper legs and gluteal region, but there were no vascular lesions on the skin. It was also noted that the child had waddling gait. On the blood count, hemoglobin was 9 gr/dL, leukocyte count was 6100/μL, and platelet count was 7000/μL. On bone marrow examination, megakaryocytes were seen and exclusion of malignant diseases was made. On coagulation study, PT was 38.2 sec, aPTT was 52.4 sec, INR was 4, D-dimer was 2001 mg/dL, and fibrinogen level was 37.7 mg/dL. Abdominal ultrasonography and chest X-ray were normal. Because of the muscle atrophy in the legs and gait disturbance, spinal magnetic resonance imaging (MRI) was taken to exclude neurological problems. Diffusely infiltrating lesion in the paravertebral and retroperitoneal area from T11 to L5 vertebrae with extension into the left side of spinal canal was shown on the lumbar MRI examination. It was variable hypointermediate signal intensity on T1W images and predominantly hyperintense on the T2W images with heterogeneous areas. Postcontrast MRI showed diffuse contrast enhancement of all areas of lesions. The bony structure showed compression on especially central area of L1, L2, and L3 vertebrae corpus, and there was no signal change of the bone marrow of these vertebrae (). There was no evidence of any other lesion in the brain or abdomen. With the thrombocytopenia, consumptive coagulopathy, ecchymosis in physical examination, and locally aggressive lesions in the radiologic evaluation, lesion was considered to be vascular lesions, KHE mostly. Prednisolone was started at a dose of 2 mg/kg/day. But, with this treatment, severe thrombocytopenia and abnormal coagulation profile persisted. On the third week, propranolol was added to the treatment at a dose of 2 mg/kg/day. After 2 months of these treatments, clinical, radiological, and laboratory improvements were not observed. To rule out hemangioblastoma, biopsy was taken after the replacement therapies with platelet and fibrinogen infusions. But, just after the surgery, bleeding from the surgical site could not be controlled, and massive amounts of thrombocyte, erythrocyte, fibrinogen, and fresh frozen plasma (FFP) transfusions were needed. Ten hours after the surgery, respiratory distress developed and the patient was transferred to intensive care unit. He was extubated on the sixth day and was taken back to the hematology clinic. Immunohistochemistry staining showed Glut-1 positive erythrocytes in pathological examination and any malign transformation was not detected (). Propranolol and corticosteroid treatment did not improve thrombocytopenia and consumptive coagulopathy that was lasting for more than two months; interferon-α (IFN-α) treatment was started at a dose of 3 million U/m2/day subcutaneously. Steroid and propranolol treatments were stopped by tapering. On the third month of IFN-α treatment, the follow-up MRI showed moderate regression of lesion size, but there was no significant signal change before and after contrast images () and chronic disseminated intravascular coagulopathy symptoms persisted both clinically and in the laboratory tests. On the 4th month of IFN-α treatment, the patient had a minor knee trauma and the next day huge hematoma developed around the knee. Despite thrombocyte, fibrinogen, and FFP transfusions, bleeding in the knee and consumptive coagulopathy could not be kept under control. Two days after this incident, intracranial hemorrhage developed that caused the death of the patient, and while thrombocyte count was 5000/μL, fibrinogen level was 70 mg/dL and PT was 40 sec.


A 34-year-old systemically healthy Malay woman was referred to the Periodontics Specialist Clinic of the Kulliyyah of Dentistry, International Islamic University Malaysia, with a chief complaint of bleeding gums and mobility of her upper anterior teeth. Complete medical and dental histories were taken, and a full periodontal chart was recorded, including all the clinical periodontal parameters, plaque scores and bleeding on probing (BOP), probing pocket depth (PPD), clinical attachment loss (CAL), recession, mobility, and furcation involvement. The chart revealed grade II mobility of the UCI and pus discharge with deep probing depths and severe CAL >6mm. The three-dimensional picture in Fig. shows the circumferential bone resorption surrounding the UCI. The rapid rate of bone destruction affected the sites of the first and second molars, resulting in furcation involvement and mobility ranging from grade I to grade II with recorded PD and CAL greater than 6mm as well. The radiographic interpretation showed an angular bone defect mesial and/or distal to the first molars (Fig. ). Because the patient was systemically healthy except for the presence of periodontitis, and based on the patient’s history, clinical periodontal records, and radiographic findings, she was diagnosed with localized aggressive periodontitis []. A combination of oral metronidazole 400mg and amoxicillin 500mg were prescribed for 7 days as an adjunct to scaling and root planing (SRP). Six weeks after the NPT, the affected sites were reevaluated. The results revealed slight reductions in the patient’s BOP, PD, and CAL. However, the range of residual bleeding pocket depths was from 5mm to 6mm with no obvious clinical attachment gain. Therefore, the decision was made to provide regenerative therapy, and a surgical treatment was scheduled for the patient in spite of the questionable prognosis that had been assigned at baseline. The patient was informed verbally regarding the details of the surgical treatment plan and signed a written informed consent form before each periodontal surgical procedure as part of the clinical protocol of our institute.
The surgical procedure on the UCI included the use of a papilla preservation technique, which was applied to preserve the papilla and obtain primary closure of the inter-dental space (Fig. ). The technique of papilla preservation [] that we applied, compared with the modified technique [], provides better access to the palatal defect because it is applicable on the buccal and palatal aspects. Therefore, the incorporation of papillae with the facial part of the flap (Fig. ) was achieved through crevicular incisions made around each tooth without splitting the inter-dental papilla, and a semi-lunar incision was made across the palatal aspect of at least 5mm from the papillary crest, taking advantage of the keratinized palatal mucosa in protecting the graft during healing. Additionally, this palatal approach overcomes the post-operative labial scar formation in the aesthetic zone.
As a result of the intra-bony defect location, which is associated with two adjacent central incisors, significant supra-crestal components of the defect presented at the palatal area (Fig. ). The circumferential intra-bony defects were thoroughly debrided (Fig. ), and cerabone bovine xenograft granules (botiss biomaterials, Zossen, Germany) were used to fill the defects. An OsseoGuard resorbable bovine collagen membrane (Collagen Matrix/BIOMET 3i, Palm Beach Gardens, FL, USA) covered the bone substitute, with delicate trimming of the membrane done to extend inter-proximally (Fig. ) using sling sutures of absorbable material to adapt the membrane well and prevent its collapse over the bone graft. The flap was sutured using an internal horizontal mattress suture, which has the advantage of providing a tension-free flap (Fig. ) and a stable support for the grafting area. Simple interrupted sutures were applied at the rest of the flap sites. The sutures were removed 10 days after the surgical procedure. A fixed retainer using twisted wire (177.8mm; 3M Unitek, Loughborough, UK) was constructed palatally to splint the anterior teeth and stabilize the wound healing (Fig. ). This type of wire allows long-term stability with physiologic tooth movement.
Strict oral hygiene instructions were given; however, the patient was asked to avoid normal brushing and flossing in the treated area for 4–6 weeks and replace it with the use of 0.12% chlorhexidine mouthwash. The modified Stillman brushing method was recommended to prevent the interference beneath the gingival margin until complete resorption of the membrane was achieved. During the healing process, professional plaque control was scheduled every 2 weeks to prevent bacterial contamination of the surgical area. The patient was also instructed not to chew on the treated area for the first 4 weeks.
The application of the graft and barrier membrane resulted in remarkable bone gain and support for more than two-thirds of the root length of the UCI, as shown in the radiograph taken at 2 years (Fig. ), which confirmed the resolution of the defect present at baseline (Fig. ). There was absence of BOP, significant PD reduction up to 2mm, and greater than 5mm attachment gain. Additionally, there was almost an equivalent amount of attachment gain and bone fill. The intra-oral photographs in Fig. show the clinical improvement in the gingival tissue from edematous bleeding gingiva (Fig. ) to the closely adapted, firm, non-bleeding sites (Fig. ). Furthermore, Fig. depicts a good post-operative result without any inter-proximally soft tissue crater formation, which explains the pre-requisite of the papillary preservation technique.
Three more regenerative periodontal surgeries were performed for regions of teeth 46, 26, 27, and 36. In these procedures, we used different xenograft bone granules of bovine origin (Endobone from Collagen Matrix/BIOMET 3i and Bio-Oss from Geistlich Biomaterials, Wolhusen, Switzerland) and achieved successful results in respect to bone fill (Fig. ) compared with baseline (Fig. ). The patient was covered with systemic antibiotics for all periodontal regenerative surgical procedures. A decision was made to extract the partially erupted and angulated third molars because these teeth were either without antagonists or compromising the adjacent second molars. They also presented difficulties in self-performed plaque control and acted as a local factor for plaque accumulation in deep periodontal pockets. The surgical extractions were performed during the surgical periodontal therapy to minimize the required treatment time and healing process.
During the phase of supportive periodontal therapy, the patient was treated with a schedule of professional plaque control with adjunct use of photodynamic therapy (PDT). The recall system of the maintenance phase was arranged every 3 months. At 2 years after baseline, the full periodontal chart revealed a marked improvement in the clinical periodontal records, as the percentage of sites with BOP had decreased from 40% to 16% and there was a substantial decrease in the number of deep pockets, characterized by the absence of PPD greater than 4mm compared with 22.22% before the commencement of treatment. This PPD reduction was accompanied by an average CAL gain of 3.07mm.


A 25-year-old woman had multifocal, epithelioid gastric GIST (CARE checklist is available as Additional file ). KIT (exons 9, 11, 13, and 17) and PDGFRA (exons 12, 14, and 18) molecular analysis did not show evidence of mutations. The clinical/pathological features matched well with those describing dSDH GIST (female sex, young age, and multifocal and epithelioid gastric GIST). Moreover, in a gene expression analysis previously reported (GSE20710), we showed that the expression profile of this tumor (GIST_21) was similar to the profile of 3 patients (GIST_07, GIST_10, and GIST_24) who we subsequently identified as having mutated SDHA [, ]. To better characterize GIST_21, all SDHx subunits were sequenced by the Sanger method, but the analysis did not show any mutations.
To search for alterations that might not have been detected by Sanger sequencing, we performed paired-end whole exome sequencing of GIST_21 (see Additional file for materials and methods), without finding any mutations in any SDHx coding sequences or flanking intronic regions. Exhaustive exome sequencing analysis revealed no pathogenic mutations in other GIST-related genes, such PDGFRA, BRAF, and neurofibromin 1, and only a nonsynonymous missense variant in exon 18 of KIT. This variant is described in the COSMIC database (COSM133780) in 1 case of malignant melanoma. Because the matched normal sample for this patient was not available, we were not able to determine if this KIT variant was somatic or germinal.
By gene expression analysis, we examined the expression levels of the 4 subunits of the SDH complex; as shown in Fig. , we detected a remarkable down-modulation of SDHC mRNA in GIST_21 with respect to all other GIST samples, both SDHA-mutant and KIT/PDGFRA-mutant GIST. Moreover, Western blot immunoassay showed that GIST_21 had normal SDHA but markedly decreased SDHB protein expression (Fig. ) and would be predicted to have a loss of SDH complex activity. Additionally, SNP6.0 genotyping on GIST_21 showed a normal karyotype, with no detectable loss of heterozygosity or chromosome 1q deletion [].
Hypermethylation of the SDHC promoter region was recently associated with Carney triad–related GIST and dSDH GIST without SDHx mutation [, ]. Thus, we designed a bisulfite methylation assay to look for potential epimutations in the 2 known CpG islands of the SDHC promoter regions, CpG17 and CpG27. This analysis was performed in 4 dSDH (including GIST_21 and 3 GIST without SDHx mutations) and in 8 KIT/PDGFRA-mutated GIST. Hypermethylation of the SDHC promoter was found only in GIST_21, and this alteration was detected in heterozygosis at all CpG sites (Fig. ). The methylated case showed a significant 0.63-fold decrease in SDHC mRNA expression level (P = 0.0001) in comparison with the 11 methylation-negative cases (Fig. ).


A 43-year-old woman presented to the emergency department with acute agitation after she became violent and struck her mother, breaking objects throughout the house. The patient had a previous positive genetic test result for HD and a history of multiple inpatient psychiatric admissions and had been discharged from a regional psychiatric unit four days prior for impulsive outbursts of anger and aggression, along with numerous suicide attempts. The patient's home medications were chlorpromazine 50 mg daily, clonazepam 1 mg every 8 hours, divalproex 500 mg three times a day, and gabapentin 300 mg every 8 hours. Haloperidol 5 mg twice a day in the emergency department did not adequately control her impulsivity and agitation. She was involuntarily admitted to the inpatient psychiatric unit for close observation and medical management. The patient was discharged after ten days to her private home and returned two days later escorted by police, after attempting suicide by ingesting shampoo, hairspray, and toothpaste and expressing suicidal ideations. On admission, she appeared unkempt and withdrawn, resistant and uncooperative, whispering to herself, exhibiting bizarre behavior such as ritualistic muttering of statements under her breath, alogia, avolition, loss of social interest towards her surroundings, and an unwillingness to engage in eye contact or conversation.
According to her mother and maternal half-sister, the patient was perfectly normal five years ago, formerly trained and employed as a paralegal with four children. However, after the birth of her youngest child, a now five-year-old girl, the patient began to manifest symptoms of severe depression which progressively worsened to where she would cry inconsolably. The patient sometimes became verbally and physically abusive towards her family. The first motor symptoms appeared a year later with hand tremors and grasping difficulties, followed by gait imbalance. Eventually, the patient required assistance to manage her activities of daily living and had to move in with her mother, thereafter demonstrating progressively worsening motor symptoms, depression, mood lability, irritability, agitation, anxiety, and episodically violent behavior. The patient's mother had difficulty trying to control her behavior and had to call the police on several occasions so her daughter could get medically evaluated.
The patient's mother reported no family history on her side of abnormal movements or impulsive behavior. The patient's biological father emigrated to the United States (US) from Cuba in his early 50s. The mother recalled that the father at that time, about twenty years prior, had similar episodes of depression, anger outbursts, and abnormal movements. She observed him to be very nervous, anxious, and restless, constantly getting up from a seated position and had to be hospitalized several times at an inpatient psychiatric unit to stabilize his mood and behavior. The patient has two paternal half-siblings in Cuba who could not be contacted for their medical history and two maternal half-siblings in the US who are symptom-free. Although not confirmed, it appears this patient inherited the disorder from her father, who may have been the index case of HD in their family pedigree, based on the lack of symptomatic family history prior to her biological father.
While admitted in the inpatient psychiatric unit, the patient characteristically paced with a subtle choreiform gait, exhibiting unpredictable mood lability, confrontational behavior, and sporadic tearfulness. She sometimes became verbally abusive towards staff, yelling profanity. The patient communicated, on several occasions, the delusion that her husband had just visited her at the hospital, when he had not. She also fixated on the false belief that her husband and family were taking her home, despite no visits or calls from her husband or family. In addition, although not formally assessed, the patient had memory lapses. She would forget eating soon after she ate. She had obsessive thinking about “cleanliness” and a compulsive need to repeatedly shower, observed wandering the psychiatric ward with almost persistently damp hair, a behavior that resulted in a fall and head injury in the shower requiring neurological workup. The head computerized tomography from this workup revealed no fractures, only orbital soft tissue swelling, as well as imaging features compatible with HD neurodegeneration, including diffuse cortical atrophy, prominent sulci, and ex vacuo dilation of the ventricles (). The diagnosis of HD was verified by a genetic assay that subjected whole blood DNA to polymerase chain reaction (PCR) amplification and electrophoresis size analysis, quantifying the number of CAG repeats in the HD gene with sensitivity and specificity of 99% (ARUP Laboratories Inc., Salt Lake City, UT). The patient had one HD allele in the normal range at 17 CAG repeats and the other allele in the affected range at 45 CAG repeats, verifying the diagnosis of HD.
The patient was admitted and discharged a total of eleven times in five months, treated for numerous exacerbations of depression, anxiety, irritability, aggression, and suicidal and psychotic behavior, requiring acute inpatient stabilization. She was treated with atypical antipsychotics (quetiapine and olanzapine), typical antipsychotic (haloperidol), antidepressants (venlafaxine and escitalopram), mood stabilizers (lithium and divalproex), and benzodiazepines (clonazepam and lorazepam). The patient was encouraged to participate in group therapy and psychiatric treatment aimed at mood stabilization and building coping skills to better cope with chronic terminal illness. The patient responded well to a regimen of olanzapine 15 mg at night, escitalopram 20 mg daily, lithium carbonate 300 mg in the morning and 600 mg at night, divalproex sodium 500 mg twice a day, and clonazepam 1 mg twice a day. She reached the maximum benefit of treatment in the inpatient psychiatric unit while being compliant with medications and psychiatric therapy, as evidenced by (1) diminished impulsivity and aggressive behavior; (2) improved mood and affect; (3) improved interpersonal interactions with peers and nursing staff; and (4) reassuring reports that she was eating and sleeping well without any acute physical or psychiatric complaints. The patient was monitored closely for adverse effects of the medications with no reported emergent adverse effects and was eventually transferred to a state mental health long-term care facility.


An 81-year-old Japanese woman with a medical history of type II diabetes mellitus and arteriosclerosis obliterans was referred to our hospital for investigation of the frequent elevation of hepatic and biliary enzymes and dilatation of the intrahepatic bile ducts, which were identified by her family doctor during abdominal ultrasonography. The patient did not complain of abdominal pain or decreased appetite. Laboratory examinations revealed slightly elevated biliary enzymes, including 413 IU/L alkaline phosphatase (normal, 104–338 IU/L) and 184 IU/L -glutamyl transpeptidase (normal, 9–28 IU/L); however, other serum chemistry data were within normal limits. Levels of the serum tumor markers carcinoembryonic antigen and carbohydrate antigen 19-9 were also within normal limits. Contrast-enhanced abdominal computed tomography and magnetic resonance cholangiopancreatography showed dilatation of the intra- and extrahepatic bile ducts and the main pancreatic duct without any strictures (Fig. ), suggesting the presence of an ampullary tumor. Neither lymph node enlargement nor metastasis was observed. Endoscopic ultrasonography showed a hypoechoic mass of 9 × 9 mm in the ampulla of Vater (Fig. ). The tumor was located on the inside of the duodenal wall and had not invaded the duodenal muscularis propria, pancreas, common bile duct terminal, or main pancreatic duct. Duodenoscopy revealed a bulging oral protrusion because of the dilated distal bile duct and an exposed reddish tumor at the ampulla of Vater (Fig. ), which was diagnosed on biopsy to be adenocarcinoma with the SCC component. Endoscopic retrograde cholangiopancreatography (ERCP) showed dilatation of the upstream bile duct and main pancreatic duct, and ERCP followed by transpapillary biliary intraductal ultrasonography revealed a hypoechoic tumor confined within the ampullary portion of the duodenum, the common channel of the ampulla, and the ampullary portion of the bile duct area. However, no extension into the ampullary portion of the pancreatic duct or the distal bile duct was observed. Because the patient showed the onset of acute cholangitis secondary to ERCP, endoscopic retrograde biliary drainage was performed using a plastic stent on the day after ERCP. Based on these findings, the lesion was diagnosed as early-stage carcinoma of the ampulla of Vater with the SCC component without extension along the distal bile duct or main pancreatic duct. Subsequently, pylorus-preserving pancreaticoduodenectomy (PPPD) with regional lymph node dissection was performed. Macroscopic examinations revealed a whitish and solid exposed-type tumor, 11 × 8 mm in size in the ampulla of Vater (Fig. ). Pathological examinations showed the presence of two malignant components, including poorly differentiated tubular adenocarcinoma and SCC without invasion beyond the sphincter of Oddi or into the duodenal submucosa (Fig. ). SCC and adenocarcinoma components in the tumor comprised approximately 30 and 70 % of the tumor mass, respectively. No regional lymph node metastases or lymphovascular or perineural infiltrations were observed. Immunohistochemistry (IHC) analyses of the squamous marker cytokeratin (CK)-5/6 showed strong positive expression in the SCC component and slight expression in the adenocarcinoma component (Fig. ). In contrast, the adenocarcinoma marker CK-7 was strongly detected in the adenocarcinoma component and weakly detected in the SCC component (Fig. ). The postoperative course was uneventful and the patient experienced no tumor recurrence or metastasis until 20 months following surgery.


A 42-year-old white male presented to the emergency department with a 3-day history of fevers, shortness of breath, and a mucus-producing cough. He reported a history of hypertension, smoking half a pack to one pack of cigarettes per day, and drinking four to six beers per day. (Although not reported, the specific size of each beer is 22 ounces. Thus, the patient consumed 88 to 132 ounces of beer per day, an equivalent of approximately 7 to 11 standard drinks per day). He reported no history of lung disease, such as chronic obstructive pulmonary disease or asthma.
The patient received immediate attention as a result of having reduced oxygen supply. Oxyhemoglobin saturation, a measure of oxygen in the blood, was low (89 percent). His blood pressure was 102/72 mmHg and heart rate 118 beats per minute. This blood pressure was well below the patient’s baseline pressure. The patient appeared ill and had shortened speech pattern. No jugular venous distention was present. His cardiac examination was normal and the lung examination revealed dullness to percussion at the right base, along with bronchial breath sounds and increased resonance of vocal sounds (i.e., egophony) over the right lung base. The patient’s abdomen was non-distended and non-tender, he had normal bowel sounds, and his liver edge was 5 centimeters below the right costal margin. His extremities revealed no clubbing, skin discoloration as a result of low oxygen (i.e., cyanosis), or edema, and his neurological exam was remarkable for a patient who was alert and oriented to time, place, and location. The patient moved all his extremities spontaneously.
Laboratory data revealed elevated white blood cell count (i.e., leukocytosis) to 15,600 cells per microliter with 13 percent bands, toxic granulations, and mild low blood platelet count (i.e., thrombocytopenia) to 123,000 cells per microliter. Electrolytes were remarkable for low potassium levels (i.e., hypokalemia) to 3.2 millimoles per liter, which were repleted. Arterial blood gas was consistent with the buildup of carbon dioxide in the lungs (i.e., acute respiratory acidosis) and a low level of oxygen in the blood (i.e., hypoxemia). A chest X-ray revealed dense alveolar consolidation of the right lung base. The patient was found to have severely low blood pressure because of his ongoing infection and inflammatory response (i.e., septic shock) and overly active proteins that control blood clotting (i.e., disseminated intravascular coagulation) attributed to pneumonia.
The patient received supplemental oxygen and oxyhemoglobin saturation increased from 89 percent to 95 percent. Broad-spectrum antibiotics were initiated within 2 hours after the patient arrived at the emergency department. Despite administration of 2.5 liters of normal saline, the patient’s blood pressure further decreased to 92/54 mmHg. As a result, a central venous catheter was placed, and norepinephrine was given to raise the patient’s blood pressure. The patient was transferred from the emergency department to the medical intensive care unit. However, shortly after arrival to the medical intensive care unit, oxyhemoglobin saturation decreased to 86 percent, and the patient continued to have a difficult time breathing. He was emergently intubated with an endotracheal tube because of acute respiratory failure, and mechanical ventilation was initiated. A repeated chest X-ray revealed progression of air-space disease: It involved both lungs and all four quadrants. The patient was diagnosed with acute respiratory distress syndrome, and ventilator settings were adjusted to decrease tidal volume to 6 milliliters per kilogram ideal body weight. The patient also required increasing amounts of positive end expiratory pressure to 14 centimeters of water and fraction of inspired oxygen 70 percent. Over the ensuing 2 to 3 days, the patient’s blood flow improved slowly, his norepinephrine dose was decreased, and leukocytosis resolved. Blood cultures grew cephalosporine-sensitive Klebsiella pneumoniae.
Although the patient’s clinical condition improved, his neurocognitive function did not. He was given sedatives to facilitate mechanical ventilation. However, he required high doses of the depressant midazolam, and his mental status fluctuated from being unresponsive to sitting upright in bed attempting to remove the endotracheal tube. On hospital day 2, the patient developed delirium. The presence of the endotracheal tube prevented the patient from expressing himself verbally, and the medical team had very limited ability to communicate with him. The team was unable to determine whether the patient had a headache, nausea, or experienced hallucinations or the feeling of insects crawling on one’s skin (i.e., formication), signs of alcohol withdrawal. Rather, the medical team was limited to evaluating gross motor movements, behavior, and facial expressions. The patient’s fevers and rapid heart rate (i.e., tachycardia) persisted for days, and the physicians were unable to determine whether these were attributed to ongoing infection and inflammatory response (i.e., sepsis) or if the patient was developing superimposed alcohol withdrawal. Administration of midazolam was based on motor behaviors and a modified Clinical Institute Withdrawal Assessment for Alcohol (CIWA-Ar). Realizing its limitations in the non-verbal critically ill patient, physicians were left attempting different strategies for management of sedation. They increased the dose of midazolam, only to find that the patient became overly sedated and unresponsive. Empiric trials of opioids were attempted in order to determine if the patient was experiencing pain, but again this strategy did not seem to improve the patient’s neurocognitive function. Next, haloperidol, an antipsychotic, was administered to control agitation and perhaps aided in management of delirium. The team continued to attempt different strategies to manage his fluctuating agitation and sedation, including attempts to limit midazolam administration in favor of administration of haloperidol. However, after approximately 7 days, the patient was more alert and cooperative, but it was not clear what led to the improvement. He remained delirious but was able to follow commands. His septic shock resolved, and mechanical ventilation was removed.


A 32-year-old white woman presented to our county hospital with a history of nausea, vomiting, heart palpitations, trembling and extremity spasms. She had started a strict LCHF diet, with an estimated carbohydrate intake of less than 20g per day, 10 days before admittance, lost 4 kilograms and had felt growing malaise. She was breastfeeding her son of 10 months of age. She continuously denied any alcohol or drug intake. She had a past medical history of hypothyreosis and had a family history of high blood pressure but not for diabetes. She took acetaminophen occasionally but no other medications.
The initial examination in the emergency department revealed an unaffected woman with respiratory rate of 12 breaths per minute, oxygen saturation 96% on room air, body temperature 36.3°C, pulse 102 beats per minute and blood pressure of 110/80mmHg. Nothing abnormal was revealed on examination of her heart, lungs, abdomen and thyroid gland.
An arterial blood gas was taken. It revealed pH 7.20, base excess (BE) −19, partial pressure of carbon dioxide (pCO2) 2.8 kPa, glucose 3.8mmol/l and lactate 1.0mmol/l. Her blood ketones were 7.1mmol/l (reference 0 to 0.5mmol/l). No genetic testing of any kind was performed.
The primary diagnosis was thought to be ketoacidosis due to starvation induced by the LCHF diet but blood samples for s-paracetamol, s-salicylate, s-ethanol, s-methanol, s-ethylene glycol, kidney function, diabetic autoantibodies, plasma cortisol (p-cortisol) and tests for thyroid function were added. She was admitted to our medical ward, given an intramuscular vitamin B injection and started on a 10% glucose infusion. In total 3L of glucose were infused, with an infusion rate of 125mL/hour, during 48 hours.
The following day, after glucose infusion and small doses of human insulin administered intravenously and insulin aspart subcutaneously, her acidosis was reversed. In total 4 units of insulin were administered during 24 hours. She was discharged, fully recovered, after 3 days and follow-up was performed after 1 month.
Diabetes mellitus was excluded by normal blood glucose and C-peptide, glycated hemoglobin (HbA1c) 37mmol/mol and negative autoantibodies: islet antigen number 2 (IA2) <15 kE/L, reference <15 and glutamic acid decarboxylase (GAD) <5 kE/L, reference <5. No drugs could be detected in her blood and her kidney function was normal (S-creatinine 67μmol/L, reference 45 to 90). The day after admittance her thyroid-stimulating hormone (TSH) was normal (2.5 mIE/L, reference 0.4 to 3.7) but her triiodothyronine (T3) and thyroxine (T4) were low: T3 2.6pmol/L, reference 3.6 to 6.3 and T4 11pmol/L, reference 12 to 22pmol/L. However, on the follow up 1 month later, her thyroid tests had normalized and thyroid peroxidase (TPO) antibodies were negative. Her p-cortisol was normal, 674nmol/L, in the morning (reference 200 to 800) as well as adrenocorticotropic hormone (ACTH). The lack of other possible explanations supported the primary diagnosis: ketoacidosis due to starvation.


A 72-year-old white man presented with a 2-year history of gait and balance disturbance, associated with paresthesia to his upper and lower limbs and frequent episodes of falling to the ground. He recently referred episodes of morning vomiting. At a physical examination he presented with a short neck and a low hairline at the back of his head. A neurological examination revealed ataxic gait with retropulsion at the Romberg test, slight weakness in his right limbs, tetra hyperreflexia with bilateral Hoffmann and Babinski signs, and dysmetria in the finger-to-nose test. A magnetic resonance imaging (MRI) study revealed a fourth ventricle neoplastic lesion, with infiltrative features and non homogeneous signal. The lesion presented a uniform contrast enhancement in its posterior part while a peripheral enhancement was observed in its anterior portion.
A craniocervical computed tomography (CT) scanning revealed a scoliotic deformation of the vertebral column with C3 to C4 synostosis. These features allowed a diagnosis of Klippel–Feil syndrome (Fig. ).
The patient was operated through a suboccipital craniectomy with a C1 laminotomy and bilateral vertebral artery transposition. We performed a subtotal tumor resection using a telovelar subtonsillar route, because of tight neoplastic adhesions to the cranial portion of the floor of the fourth ventricle.
A histopathological examination revealed a periodic acid–Schiff (PAS)-positive dysmorphic material with pluristratified epithelial islands. A teratoma, partly cystic, with pseudo-cartilage areas and hair and tooth-like formations was identified. There were also features of a squamous cell carcinoma with atypical polymorphic epithelial cells, necrosis and focal cornification. The lesion was related to a teratoma with malignant transformation (Fig. ).
The postoperative course was uneventful with neurological improvement. The patient was able to walk without ataxic feature and the Romberg test was improved. A postsurgical CT scan demonstrated a partial removal (Fig. ).
At 6-months follow-up, a MRI showed an early regrowth of the fourth ventricle tumor, with the same radiological features (Fig. ). According to the wishes of the patient and his relatives, no further surgical treatment or adjuvant therapy was performed.


An otherwise healthy, 15-year-old female was referred for evaluation of a right breast mass, identified a few months before by self-examination. She reported no associated pain, nipple discharge, or skin changes and had no family history of breast or ovarian cancer. Physical examination identified a firm, mobile mass in the right upper outer quadrant with no associated lymphadenopathy. On ultrasound examination, a solitary, solid 3 × 2.1 × 2.2-centimeter mass was noted at the 10 o'clock position, with no vascularity on Doppler exam, () and interpreted as most consistent with a fibroadenoma.
The patient underwent excisional biopsy of the mass through a circumareolar incision adjacent to the mass. She was discharged home on the same day and had an uncomplicated recovery. Gross pathologic exam of the 3.5 cm specimen revealed a round 2.1-centimeter, demarcated, distinct, homogenously yellow mass (). Microscopically the lesional cells were large, round to polygonal, with large amounts of granular eosinophilic cytoplasm, small nuclei, and indistinct nucleoli, and were arranged in a thick cord-like pattern with delicate vascularity. The tumor was homogenous with no cytologic atypia, notable mitoses, or necrosis. Immunohistochemical staining for CD68 and S100 was diffusely positive () but negative for cytokeratin, consistent with a GCT. The tumor borders were pushing and compressing the stroma at low power, with short peripheral extensions and limited peripheral encasement of occasional normal ducts and lobules at high power ().


A 55-year-old woman was referred to our clinic with malaise and an abdominal tumour. In 2009 she had a LASH procedure with use of a morcellation device for bleeding problems caused by a myomatous uterus. At the primary surgery the BMI of the patient was 27.7. As the patient was premenopausal the adnexa remained in situ. Postoperative histological analysis on the morcellated tissue (total 242 grams) showed benign myomas, with some mitotic activity and infarct type necrosis. Although the patient received adequate thrombotic prophylaxis by dalteparin, the patient developed postoperatively a deep-venous-thrombosis but recovered quickly after adequate treatment. As the tissue showed benign myomas no follow-up was performed. The patient was referred now with malaise, weight loss, and an abdominal mass. A vaginal ultrasound showed a large tumour (13 cm × 13 cm × 10 cm) most likely attached to the adnexa. There was no free fluid seen intra-abdominally. CA-125 and CEA were analysed to determine the malignancy risk (CA-125 14, CEA < 0.3). As the tumour markers were normal, the patient was scheduled for surgical removal of the tumour. During an abdominal surgery procedure 2 normal adnexa were seen, and the tumour appeared malignant and seemed connected to the remaining cervical tissue, the greater omentum, and sigmoid. No other abnormalities were observed intra-abdominally. As the tumour was connected to the omentum and sigmoid, and since the tumour bled easily, a malignancy was suspected and biopsies were taken for further diagnosis and the procedure was ended. A CT scan was performed and showed a large tumour without any evidence for intra-abdominal or thoracal metastasis. Histological analysis of the biopsies showed a smooth muscle tumour with high mitotic rates, tumour necrosis, and nuclear atypia, diagnostic for leiomyosarcoma. The patient was referred to a third care centre for surgical treatment and follow-up. She had abdominal removal of the tumour and showed a quick recovery. Because of the unexpected findings the original uterine tissue was reanalysed. Reanalysis of the morcellated uterine tissue showed features which confirmed those described in the original report. The patient received standard follow-up care, and after 7 months a CT scan was made because of abdominal complaints. The CT scan showed a large intra-abdominal tumour with possible lung metastasis. The patient received palliative chemotherapy and died after 10 months.


A 57-year-old, asymptomatic woman with no history of smoking was referred to our hospital after a chest radiograph revealed a nodular shadow in the right middle lung field (Figure A). Chest computed tomography (CT) showed a well-defined, round, solid nodule, which was 10 mm in diameter, in segment 6 of the right lung (Figure B). Positron emission tomography/CT did not demonstrate any other lesions in other parts, including the bones or the soft tissue. Physical and laboratory examinations revealed no significant abnormalities. Furthermore, the levels of serum tumor markers were not elevated. Imaging analysis revealed that the pulmonary nodule was adjacent to the visceral pleura; however, it was difficult to use the endobronchial approach to obtain adequate samples for histological diagnosis. Therefore, we performed thoracoscopic wedge resection of the right lower lobe for diagnostic purposes; no other pulmonary nodules were found intraoperatively.
Macroscopically, the resected sample contained a homogeneous, jelly-like transparent tumor, 10 mm in maximum diameter, in the lung parenchyma (Figure A). Histologically, the tumor was well demarcated and paucicellular and it consisted of large tumor cells with a low nuclear/cytoplasmic ratio and prominent intracellular vacuolation (Figure B). The tumor, which lacked a fibrous stroma, was also devoid of blood and lymphatic vessels. Instead, the tumor cells had proliferated in a prominent myxoid background. Cuffs of lymphocytes and minimal destruction of the alveolar structure were observed at the periphery of the tumor (Figure C and D). Adipocyte-like, univacuolated, and multivacuolated cells were intermingled within the tumor; the latter resembled physaliphorous cells that are typically observed in chordomas. Moreover, most of the tumor cell nuclei were small and round, with fine chromatin and less conspicuous nucleoli. However, some of the tumor cells were irregular in shape and had larger nuclei (Figure E); neither mitotic figures nor vascular invasion was observed. A myxoid substance was observed in the cytoplasm of vacuolated tumor cells and microcystic space on alcian-blue staining, which also stained the adjacent alveolar spaces (Figure F).
On immunohistochemical staining, brachyury, a highly specific diagnostic marker of notochordal differentiation, was focally but undoubtedly positive in the nuclei of the tumor cells (Figure A). We compared the ratio of the brachyury-positive cells from the present case with 3 other conventional chordomas of the axial bone by counting 100 nuclei. This analysis was approved by the Institutional Ethical Committee (KH-1460) and the written informed consent was obtained from the patients. The results showed that the ratio of brachyury-positive cells in the present case (14%) was much lower than that of the control chordoma cases (90%–98%, mean 93.3%) (Figure B). The tumor was also positive for immunohistochemical markers for chordomas and BNCTs arising from the axial skeleton, including cytokeratins (CKs) AE1/AE3 (pan CK), CAM5.2 (low-molecular-weight CK), vimentin, S100 protein, CK7, CK18, and CK19 (Figure C and D). Conversely, the tumor cells were negative for CK5/6, CK20, thyroid transcription factor-1, napsin A, cluster of differentiation (CD) 68, and CD163 (Figure E and F). The ratio of MIB1 (Ki-67)-positive cells within the tumor was extremely low (2.2%).
In order to exclude the possibility of the lung tumor having metastasized from an unknown primary site, an extensive radiographic examination that included whole body CT, magnetic resonance imaging of the whole spine, and bone scintigraphy was performed. The results showed an absence of tumors throughout the body. Five years after surgery, the patient is alive without tumor recurrence or metastasis.


The 24-year-old male was referred from an external hospital after a job-related chemical injury to the dorsum of his left foot. He accidentally poured an unknown amount of caustic soda solution over his foot. The emergency treatment was performed in a general hospital near to the patient’s place of residence. He was admitted to our Burns Unit the following day. Patient examination showed extensive necroses from the ankle region until the distal third of the dorsum of his left foot and the metatarsals. Planta pedis, heel and toe region remained intact (Figure 1 ). Due to the extent of the clinical evidence we decided to perform a two-stage surgical approach with extensive debridement including intercurrent vacuum assisted therapy and reconstruction with a free flap. During surgical debridement we discovered a full-thickness liquefactive necrosis involving the tendons of the Musculus extensor digitorum longus (Figure 2 ). Covering the defect with a skin graft was not appropriate in this situation. After preoperative arrangements and preconditioning of the defect surface with the use of continuous vacuum assisted therapy we performed microvascular free temporoparietal fascial flap transfer on our patient.
No intra- and immediate postoperative complications occurred. The flap and the partial-thickness skin graft, which was used for flap coverage, showed no signs of inflammation. The donor sites at the right temporoparietal region and on the left thigh showed no signs of inflammation or neurologic disorders of the fascial or temporal nerve. The patient underwent early physiotherapy for remobilization. He was also assorted of compressive clothes to remodel the flap on his left foot. He was discharged to outpatient care on the 28th day after admittance in a healthy condition with good scarring. The initial care was performed by our outpatient unit with weekly follow-up visits and short-term visits after 9 weeks (Figure 3 ) and 5 months post surgery (Figure 4 ). Given full weight bearing the patient did not complain of any disturbance. There were no signs of hypertrophic scarring at the donor site with minimal hyperaemia. We did not discover any signs of paraesthesia or functional disorder in the facial region, also the scar in the temporal region remained unremarkable.
Surgery was performed as a two-team-approach. We decided to perform an end-to-side anastomosis to the dorsalis pedis artery after preoperative identification of three sufficient vessels in the patient’s left lower extremity with Doppler ultrasound. Pre- and intraoperative Doppler ultrasound measurement of the temporal region identified the superficial temporal artery with its bifurcation to the anterior and posterior part. We chose a preauricular incision and dissected the temporal fascia under constant surveillance of the superficial temporal artery and without crossing Pitanguy’s line (Figure 5 and Figure 6 ). Simultanously the preparation of the recipient vessels was performed, including the dorsalis pedis artery with two concomitant veins (Figure 7 ). The flap measured 10 by 6 centimetres, after the flap harvest was successfully performed we used a drainage and a resorbable intradermal running suture to close the defect. Under microscope the end-to-side anastomosis was performed before suture of the temporoparietal fascial flap. Eventually we took a split-thickness skin graft from the patient’s ipsilateral thigh and covered the flap (Figure 8 ).