A 75-year-old Japanese male was transferred from another hospital after presenting with hemorrhagic shock due to hematochezia. He had been transported by ambulance to the previous hospital after complaining of discomfort and bloody stool on the previous day. He had undergone distal pancreatectomy and right lower lobectomy for pancreatic cancer and lung cancer, respectively, 5 years prior and total pancreatectomy due to residual pancreatic cancer eight months prior. The reconstruction procedure consisted of hepaticojejunostomy, Braun’s anastomosis, and gastrojejunostomy. In addition, a stent had been inserted for stenosis of the hepaticojejunostomy 1 month prior. The patient’s medical history also included atrial fibrillation, and he was taking insulin and apixaban. On arrival, he appeared to be pale and his extremities were cold. His vital signs were as follows: respiratory rate, 20 breaths/min; pulse rate, 105 beats/min (bpm); blood pressure, 77/54 mmHg; temperature, 34.9 °C. Arterial blood gas analysis detected severe lactic acidosis (lactate concentration: 9.9 mmol/L) and anemia (hemoglobin level: 3.1 g/dL). The laboratory data showed a white blood cell count of 5850/μL, C-reactive protein level of 0.08 mg/dL, procalcitonin level of 0.097 ng/mL, and serum glucose level of 389 mg/dL. Contrast-enhanced computed tomography of the abdomen did not detect any active bleeding. His melena had already ceased. Fluid resuscitation and massive transfusions resolved his hemorrhagic shock. Crystalloids were administered 800 mL over an hour. Blood products were initiated 30 min after the patient’s arrival. The patient required 16 units of red blood cell concentrate, 14 units of fresh frozen plasma and 20 units of platelets within 12 h of his arrival for hemostatic resuscitation. A classification of hemorrhage of the patient was considered as class IV based on the American College of Surgeons. Urgent upper gastrointestinal endoscopy was a poor study because of food residue and failed to identify the source of the patient’s bleeding. Thus, he was admitted to the emergency intensive care unit for careful observation. The patient’s acute physiology and chronic health evaluation (APACHE II) score on the day of admission was 24, and the Charlson comorbidity index was three.
Since fresh bleeding was detected in the terminal ileum during capsule endoscopy the next day, colonoscopy was performed without bowel cleaning. But the exam produced poor findings due to blood clot. Therefore, transanal double balloon enteroscopy was scheduled for further investigation 4 days after the patient’s admission. The patient took 5 h to ingest 2 L of PEG plus an additional liter of PEG for bowel preparation. This was the first time he had consumed PEG. 1 h after consuming the 3 L of PEG, he complained of a feverish chill and his heart rate and temperature increased to over 130 bpm and 39 °C, respectively. The transanal double balloon enteroscopy exam was performed uneventfully and did not reveal the source of the patient’s bleeding.
After the examination, he appeared to be agitated and distressed and exhibited hypotension, high fever, and an elevated lactate level, which indicated septic shock. He did not have any other complaints. His abdomen was soft, flat, and non-tender. Empirical antibiotic therapy with meropenem was administered, and two sets of blood cultures were obtained at the same time. Fluid resuscitation and a noradrenaline infusion were initiated due to septic shock, followed by intubation and mechanical ventilation. Table shows the patient’s laboratory data at the time that he suffered septic shock. Repeated lab tests revealed a significantly elevated procalcitonin level. Liver function tests produced normal results during the course, so we excluded biliary stent infection. Figure shows the patient’s clinical course during the first 15 h after he ingested PEG, the hypotensive phase of his condition, and the resuscitation period. Ongoing intensive care led to a gradual improvement in the patient’s condition and he was successfully extubated on day 4 (the day when the PEG preparation was administered was defined as day 0, at which APACHE II score was 34). An examination of his blood cultures detected C. braakii and we replaced the meropenem with ceftazidime based on the results of sensitivity tests. Later, the identity of the pathogen was confirmed by biochemical analysis and partial sequencing of 16S rRNA. Urinalysis produced normal results. Cultures of the patient’s urine and sputum at the onset of septic shock were negative. The tip cultures of central venous catheter which had been placed in a femoral vein on admission were found to be negative. Stool culture was obtained 1 week after presenting septic shock, which was also negative. Antibiotics were administered for a total of 10 days. The patient was discharged from the emergency intensive care unit on day 8. Figure shows the patient’s clinical course over the 8 days after he first suffered septic shock.
Melena occurred intermittently. Upper gastrointestinal endoscopy subsequently identified marginal ulceration of the gastrojejunal anastomosis, which was successfully treated with coagulation hemostasis. The patient was discharged on day 33.


A 71-year-old man was referred to our clinic for treatment of an iatrogenic total iridodialysis. Just before the referral, his iris had been totally torn out and jammed into the hinge of a prechopper during the removal of an instrument during cataract surgery. Examination revealed a visual acuity (VA) of hand-motion in the left eye. A complete iris defect with remaining lens cortex, a ruptured posterior lens capsule with radial tear of the capsule, and an intraocular lens (IOL) implanted in the sulcus were noted (Fig. ). The totally dialyzed iris was sent to our clinic preserved in sterile cold balanced salt solution, packed in a sterile biopsy bottle surrounded by a towel to prevent direct contact with ice cubes, and was transported in an icebox.
We decided to perform surgery under general anesthesia considering the patient’s poor cooperation due to dementia. To minimize IOL decentration during scleral fixation, we used a toric axis marker and marked the fixation axis (Fig. ). After the scleral flaps were in two positions 180° apart, a 10–0 polypropylene suture was passed through the bed of half-thickness scleral flaps 2.0 mm posterior to the limbus (Fig. ). A sulcus positioned IOL (PC-60 AD, HOYA Corporation, Tokyo, Japan) was repositioned and fixed by ab externo scleral sutures (Fig. ). We conducted a pars plana vitrectomy to remove the remaining lens cortex material and vitreous fibre anterior to the equator to avoid trapping the vitreous during the iris-fixating suturing (Fig. ). The preserved iris was examined. It did not show any signs of necrosis but kept its own color and morphology soundly (Fig. ). We spread out the iris on the patient’s cornea to estimate the range of damage and locate a wider part of the iris inferiorly to minimize the glare after iridopexy (Fig. ). A 10–0 Prolene on a CIF4 needle (Ethicon, Somerville, New Jersey, USA) was consecutively passed through the iris (Fig. ) and sclera 1.0 mm posterior to the limbus at the 6’ O/C position (Fig. ). Properly using both an iris spatula and ocular viscoelastic devices (OVDs), we inserted the iris into the anterior chamber completely and unfolded it to its proper position (Fig. ). The estimated cool-to-anterior chamber insertion time of the preserved iris was 8 h. Four more points of ab interno scleral sutures (4’, 1:30, 10:30 and 8’ O/C positions in sequence) were made (Fig. ). Then, the remaining vitreous, OVDs, and dispersed iris pigments were removed using a vitreous cutter (Fig. ).
One week postoperatively, intraocular pressure (IOP) increased up to 30 mmHg because of hyphema from the torn root of the iris (Fig. ); however, 3 weeks postoperatively, hyphema decreased with improved VA (20/200) and lowered IOP (15 mmHg) (Fig. ). At 4 weeks postoperatively, a much improved VA (20/100) and lowered IOP (14 mmHg) were detected (Fig. ). At 7 weeks postoperatively, VA was 20/63, IOP was 14 mmHg and there were no signs of inflammation in the anterior chamber (Fig. ). Until 6 months postoperatively, the engrafted iris did not have any signs of atrophic change, depigmentation, or inflammation; the patient complained of minimal glare, and the uncorrected VA was 20/25 with the IOP of 13 mmHg (Fig. ).


In November 2014, a 65-year-old white woman with a history of progressive dyspnea presented to our hospital for evaluation. She complained about dyspnea on exertion and had had several infectious exacerbations of chronic obstructive pulmonary disease (COPD) during the last year, one requiring hospitalization. During those exacerbations she had been treated with tapering doses of systemic corticosteroids showing improvement in hypoxemia and obstructive symptoms. At the point of presentation her medication for inhalation included: budesonide/formoterol 400 μg/12 μg twice a day, tiotropium bromide 18 μg once a day, and salbutamol as needed, which she was using at least once a day. Her general patient history was negative for atopic diseases or allergies. At the age of 54 she was diagnosed as having COPD due to dyspnea and typical lung function tests. She stated that she had never smoked tobacco. A skin prick test revealed no hypersensitivity. Since the diagnosis of COPD had been established she was regularly followed-up by a pulmonologist. At hospital admission, her general condition was slightly disturbed. She was hypoxic at rest. The saturation level of oxygen in hemoglobin (SaO2) was 92%, without providing indication for oxygen therapy.
A body plethysmography showed forced vital capacity (FVC) 1.47 L (62%), forced expiratory volume in 1 second (FEV1) of 0.8 L (40%), airway resistance of 1.62 kPa × second/L (538.9%), residual volume of 3.30 L (188%), and a total lung capacity of 4.50 L (108%). Those results were evaluated as severe obstruction with massive air trapping compatible with the diagnosis of COPD. During the further evaluation process different methods of imaging were conducted. A chest X-ray showed well-ventilated lungs and discreet apical pleural callosity. Transthoracic echocardiography showed normal cardiac structures without evidence of pulmonary hypertension. In a computed tomography (CT) scan, disseminated small nodules between 2 and 4 mm in all lobes, ground glass characteristic, and partial mosaicism on both lungs were strikingly apparent. Focal bronchial thickening could be seen. Furthermore, mediastinal lymph nodes were heightened (Fig. ).
As a result of lung imaging, bronchoscopy was initiated. Forceps biopsies of her central airways were taken, showing normal respiratory mucosa without any changes suspicious for a specific inflammation. In particular, granulomas or giant cells could not be detected. Due to ongoing clinical suspicion for interstitial lung disease a further attempt of forcing a histological diagnosis was made.
By method of transbronchial cryobiopsy (ERBECRYO2, diameter 1.9 mm; Tübingen, Germany), lung tissue samples of the middle and inferior lobes of her right lung were gained. The material was formalin fixed and paraffin embedded, cut into 4 μm-thick sections and stained with hematoxylin and eosin (H&E). The fragmented biopsy measured an overall area of 30.9 mm2 compared with the initial biopsy of 7.5 mm2 (Mirax Viewer Image Software Version 1.12, Zeiss Microimaging, Oberkochen, Germany and 3D Tech, Budapest, Hungary). Besides small airways, a regular lung parenchyma with an alveolar basic structure could be seen. The bronchioles were lined by a regular respiratory epithelium. Furthermore, in her bronchiolic mucosa, linear and nodular proliferates of small uniform cells with round to slightly ovoid nuclei and disperse chromatin were located within the epithelial basement membrane and bulged into the lumina (Fig. ). Peribronchiolar and perivascular aggregates of cells showing this morphology were also found, measuring less than 5 mm in diameter at maximum. No signs of malignancy could be found; neither could we find any mitotic activity, desmoplastic stroma reaction, or any invasive aspect. In association with the described cell cluster, slight fibrosis could be seen. In the Elastica van Gieson stain, the walls of some bronchioles were broadened and contained an increased amount of elastic fibers. Immunostainings revealed strong positivity for synaptophysin (Fig. ), chromogranin A, and CK7 in the cell cluster described. The proliferative index determined by Ki67% (Mib-1) was beneath 1% (antibodies by Roche, Rotkreuz, Switzerland). Based on these findings, the diagnosis of a neuroendocrine cell hyperplasia could be made and a possible association with DIPNECH was noted.
Due to concomitant bronchitis our patient received antibiotic therapy. Obstructive symptoms were treated with inhaled steroids and beta-2-mimetics continuously. Comparing current CT scans of our patient with scans in 2014 no significant changes are described. Last ambulatory checks occurred in January and May of 2016. The course of disease and the extent of limitation of lung function have remained stable.


A 50-year-old Pakistani gentleman, who was previously healthy, was admitted to our surgical department with generalized abdominal pain, absolute constipation, and abdominal distention. He had multiple admissions during the previous eighteen months with similar episodes, which were diagnosed as small bowel obstruction, by the clinical picture, and multiple air-fluid levels on abdominal X-rays, and always resolved on conservative measures. He was afebrile and hemodynamically stable. His abdomen was distended with no scars. He had generalized abdominal tenderness and exaggerated bowel sounds. His rectum had remnant of stools on PR examination. He had neutrophilic leukocytosis, but the rest of his labs were within normal limits. His axial abdominal X-ray showed multiple air-fluid levels. A computed tomographic scan of his abdomen, which was performed on the same day of admission, showed evidence of multiple bowel loops dilated in the left side of the abdomen with distal collapse of bowel (transition zone) with swirling sign of the vessels. Additionally, an encasement of the small bowel within a membrane-like sac was observed, with a few calcifications (Figures and ). No mesenteric thickening, ascitic fluid, or lymphadenopathy was seen. The patient was kept nil per oral, on intravenous fluids and parenteral antibiotics, and nasogastric tube was inserted. The next day the patient became toxic with temperature of 39 degrees Celsius, and the decision was made to take the patient for diagnostic laparoscopy (DL). DL showed amalgamated bowel, encased in a thick fibrous tissue, and then was converted to laparotomy. The fibrous case () was opened, dissected, and separated from the bowel loops. A 30 cm portion of gangrenous ileum, approximately 30 cm away from the ileocecal valve, was identified (). The proximal portion of the obstruction bowel was massively dilated. The gangrenous bowel was resected and primary side-to-side anastomosis was performed using GI stapler. There was no lymph node involvement observed. No antiadhesion agent was administered, and the patient's abdomen was closed, and a drain was deployed. The postoperative course was uncomplicated. The drain was removed when it gradually became nil. The patient started passing gas on the eighth postoperative day and passed motion on the tenth postoperative day, and eventually the nasogastric tube was removed. The abdominal distention was gradually decreasing on the right side but remained to have a bulge on the left side. Histopathology of the bowel adherent nodule was consistent with reactive fibrosis with hyalinization and calcifications. The dissected small bowel showed transmural ischemic necrosis, consistent with gangrenous small bowel with free surgical margins. The patient was diagnosed as a case of idiopathic sclerosing encapsulating peritonitis, as his past medical history is unremarkable. The patient was ambulating, was started on clear fluids on postoperative day 10 and soft diet two days later, and was tolerating well. The patient was discharged on the sixteenth postoperative day, with scheduled outpatient clinic visit, but he was lost to follow-up.


A 73-year-old man, Asian, and ex-smoker presented to our department with complaints of fever, headache and appetite loss after 10 months of steroid and immunosuppressive therapy for cryptogenic organizing pneumonia. Physical examination revealed a heart rate of 88 beats/min, blood pressure of 142/74 mmHg, respiratory rate of 20 breaths/min, temperature of 38.5 °C, and oxygen saturation of 95% on room air. Chest examination revealed coarse crackles on right lower lung field. Chest radiograph and computed tomography showed infiltrates on the right middle lobe (Figs. and ). The white blood cell count, C-reactive protein, and procalcitonin levels were 15,100 /μl, 5.73 mg/dl, and 0.221 ng/ml, respectively. Test for human immunodeficiency virus (HIV) infection was negative. Expectorated sputum smears were negative for bacteria and acid-fast bacilli. Urinary antigen test (Binax NOW; Binax, Inc., Portland, ME) for Streptococcus pneumoniae and Legionella pneumophila were negative. Although it was difficult to determine the causative bacteria, a presumptive diagnosis of community-acquired pneumonia was made, and treatment with ampicillin/sulbactam (ABPC/SBT) 1.5 g intravenously every 6 h was initiated. On day 8, white blood cell count normalized and chest radiograph showed improvement; however, fever persisted. Due to a suspicion of drug fever, ABPC/SBT was discontinued. However, fever persisted even after 4 days of ABPC/SBT discontinuation. To search for the source of infection, cranial magnetic resonance imaging (MRI) was done and showed multiple masses (Fig. ); brain abscess was diagnosed. Serum Quantiferon test (QuantiFERON-TB Gold In-Tube; Cellestis, Chadstone, Australia), cryptococcus antigen and toxoplasma antibody were negative. Galactomannan (Platelia Aspergillus Ag; Bio-Rad, Marnes-la-Coquette, France) and β-D-glucan (Wako; Wako Pure Chemical Industries, Ltd., Tokyo, Japan) were elevated at 0.6 index, and 94.7 pg/ml, respectively. On day 11, voriconazole (VRCZ) 200 mg intravenously every 12 h was initiated for presumed aspergillus brain abscess. However, after 5 days of treatment, fever continued and consciousness level deteriorated. On day 16, drainage of brain abscess was performed; Gram stain revealed many neutrophils and gram-positive rods with branching, filamentous hyphae (Fig. ). Kinyoun acid-fast stain of the gram-positive rods was positive (Fig. ). On the basis of these findings, disseminated nocardiosis was diagnosed, and VRCZ was shifted to intravenous trimethoprim/sulfamethoxazole (TMP/SMX) 240 mg/1200 mg every 8 h. The cultured strain was sent to Department of Laboratory medicine of Nagasaki University Hospital, where the presence of Nocardia farcinica was confirmed by 16S rRNA gene sequence. Treatment course was continued. BDG level normalized after 1 month, therefore intravenous TMP/SMX was shifted to oral TMP/SMX 240 mg/1200 mg every 8 h. Cranial MRI showed almost complete improvement after 2 months. Unfortunately, the patient died from aspiration pneumonia after 6 months of treatment.


A 77-year-old Caucasian man of Mexican ethnicity presented to our clinic 4.5 years after a knee replacement surgery. A retired repairman and avid cyclist, our patient had generally been in good health prior to the procedure. He was responsible for all his own daily activities such as feeding and bathing, cycled 25 miles per week, was an automobile enthusiast who went on regular walks to admire cars, and socialized with his family.
The patient’s family reported that they noticed marked changes in the patient starting immediately post-surgery. The night of the surgery, he became fearful he had been kidnapped and, in an attempt to escape, fell. He was unable by the next day to remember how to write his name. The family was assured by hospital staff that no adverse events had occurred that would impact the patient’s health. Nonetheless, over the following 3 months the patient continued to show dramatic cognitive, physical, and emotional impairment compared with his presurgical state. He could not recall his date of birth, sign his name, remember his children’s names, or find his way home from previously familiar locations. When spoken to, the patient appeared unable to comprehend language. He could not bathe himself, use the restroom, or eat on his own. He was unable to ride his bike, seeming unsure of the necessary movements. He slept through much of the day. At night, the patient could not sleep and would attempt to leave his home, although he was largely unable to walk. For the first time in his life, the patient showed high levels of anxiety.
Three months post-surgery, the patient was taken to a neurologist who diagnosed him with Alzheimer’s disease. The patient was started on 10 mg of diazepam and 13.3 mg of Exelon (rivastigmine) once daily. The medication sedated the patient during the day, although his insomnia persisted at night and the medication did not improve his cognitive abilities or activities of daily living (ADL). The patient’s primary care physician also concluded he had dementia and advised that little could be done to restore him to his previous level of functioning.
The patient’s symptoms/signs continued during the following years and were still present when he arrived at our clinic 4.5 years post-surgery. His medical history was notable for type 2 diabetes, hypertension, prostate hypertrophy with history of urine retention, arthritis, and obesity. His family history was positive for type 2 diabetes. His medication list included diazepam, Exelon (rivastigmine), Rapaflo (silodosin), atorvastatin, quetiapine, pantoprazole sodium, cephalexin, Janumet XL (sitagliptin and metformin hydrochloride), meclizine hydrochloride, Namenda (memantine), omega-3, metoprolol, ibuprofen, ramipril, glimepiride, and Lyrica (pregabalin). In view of his clinical status, a neuropsychologic testing could not be performed. He was essentially mute and disengaged from the clinical interview.
We performed a baseline single-photon emission computer tomography (SPECT) functional imaging with 99 m-Tc HMPAO. Brain SPECT is a neuroimaging technique that reveals the functional status of gray mater areas via relative perfusion three-dimensional mapping [–]. Results from this initial assessment (see upper row of Fig. ) showed extensive left hemispheric underperfusion, and multiple localized underperfusions in the right hemisphere. On both sides there was also involvement of the dorsolateral prefrontal cortex (DLPFC), and extensive underperfusion in the temporal lobes (primarily on the left) and to a lesser extent in the orbitofrontal areas. Such a combination of abnormal areas can be seen in major cognitive dysfunctions.
Concerning the etiology of our patient’s symptoms, while a component of degeneration (Alzheimer’s disease) could be considered, this alone was not justified by the SPECT scan. A main reason is that the posterior cingulate/precuneus area would have been expected to be definitely abnormal, but there was only very limited underperfusion in that region. The possibility of superimposed vascular etiology therefore had to be considered. Based on our patient’s medical history and SPECT results, we hypothesized that he had experienced a cerebral insult during his knee surgery, namely that the injury resulted from multiple causes, including consequences of surgery, and anesthetic effects [, ] that created perfusion abnormalities simulating those observed after stroke. To address these cognitive, physical, and emotional impairments, a treatment plan was prepared including both HBOT and PSE administration. The rationale was that our patient’s clinical status was so abnormal that multiple ways to reduce inflammation and increase stem cell activity were needed as soon as possible. Additionally, based on previous experience related to the treatment of head injuries separately with HBOT and PSE injections, we believed that their effect would be additive. Based on our experience over the past 15 years a 40-session course of HBOT was initiated. Indeed it is known from the treatment of traumatic brain injury (TBI) that for significant improvements to occur, a minimum of 20 sessions is needed. In his case the damage was so extensive that 40 sessions were considered as a necessity. The treatments were administered daily in a multiplace chamber for 60 minutes at a depth of 1.75 atmosphere absolute (ATA). After the first ten HBOT treatments, we began administering a 25-mg PSE injection approximately once weekly for 5 months. Before starting PSE, the patient had a purified protein derivative (PPD) test, which was negative. The 20 injections were made subsequent to HBOT sessions during the lengthy duration of the HBOT treatment and beyond, at 1-week intervals for 5 months. The number of injections was higher than in most patients based on the same consideration as above and on our experience of 4 years with PSE.
Concerning both treatments, based on literature and on our experience, there are very rare side effects for either procedure and when they occur are easily treated and reversible.
Our patient began showing progressive improvements starting after the first PSE injection. By the 5-month follow-up, his cognitive functioning had been substantially restored. He remembered his own name and those of his children. Although he was still not able to speak as well as he could presurgery, he returned to socializing with his family and was able to clearly understand and engage in conversation, for example laughing at a joke or telling a family member to “calm down” when upset. Perhaps even more remarkably, our patient’s physical functioning improved. We view this as consistent with the hypothesis that his symptoms reflected an acute event that left dormant but preserved viable tissue surrounding the main affected areas, as opposed to a degenerative disease. Our patient improved to the point of being able to completely care for himself, including feeding and bathing. He could go for walks on his own without getting lost, during which he showed interest in cars as he had before, and his premorbid personality style returned. A healthy sleep pattern was largely restored, with our patient sleeping about 7 hours each night. This was in stark contrast with his complete inability, when he first presented, to care for himself or interact with others. Because no neuropsychologic evaluation could be done originally, we considered that there was no point doing so at this time. His relatives were elated that he was now able to return to his place in the family dynamics.
A follow-up brain SPECT scan was done 5 months after the baseline study. While the overall appearance looked similar to the first scan, we nonetheless found increased perfusion in multiple small, localized areas in cortical and subcortical structures (see the lower row and green arrows in Fig. ). Among them were parts of the anterior aspect of the prefrontal cortex (including the ventromesial aspect), right superior parietal, right lateral occipital, superior aspect of the left frontoparietal area, posterior cingulate/precuneus and apico-mesial aspect of the right temporal lobe. In addition, there was a significant perfusion increase in the striatum bilaterally.
At this point in time our patient is at 16 months since the end of the combined treatment and all the clinical improvements mentioned above are still maintained.


A 16-month-old girl from Ankole in Western Uganda was admitted to the surgical ward of Mbarara University Teaching Hospital in Western Uganda with a dark lesion on the left cheek. This lesion had been present for 5 days prior to admission. It had started as a small red lesion on the left side of the upper gum and had quickly spread to the inner cheek. The child had been subjected to a tooth bud extraction on that side of the gums by a traditional herbalist 1 week prior to admission. The procedure had been done to cure Ebiino, a diagnosis that was reached by the traditional herbalist after the parents reported that the child was experiencing cough and flu and that they had spotted erupting tooth buds.
Associated with this dark lesion was a high-grade intermittent fever and pain. The girl’s feeding was also described as difficult, owing to her pain, but manageable. The child is the first born of the family and was up-to-date with her growth milestones and immunizations. Her mother has never attended any formal education and is a small-scale subsistence farmer together with the father of the child. The family hails from a village in Bushenyi district, which is located in Western Uganda.
A physical examination revealed that the child was in fair general condition. She had features of malnutrition that included brown, sparse hair, and she weighed 7.3 kg, which is below the fifth percentile on the weight-for-age chart adopted from the National Centre for Health Statistics. This showed failure to thrive. The child also had moderate pallor of the mucous membranes and was afebrile with a temperature of 36.6 °C.
Locally, she had a dark necrotic patch on the left cheek that involved most of the upper lip and nose and extended into the left upper gingival area. The lesion was generally oval in shape and measured about 8 cm × 5 cm. It was tender to touch. The rest of the local examination was unremarkable.
The history and findings pointed to the diagnosis of noma. The child was admitted to our hospital and started on intravenous ceftriaxone 400 mg once daily as well as rectal paracetamol for pain. A blood sample was also taken for a complete blood count, which showed mild leukocytosis and an estimated hemoglobin level of 10 g/dl. Radiology services were not available at the hospital at the time, and the parents could not afford to have it done outside the hospital. Surgical debridement was also done (Fig. ). The necrotic tissue was removed to show the full extent of involvement (Fig. ). A nasogastric tube was inserted to aid feeding. The child was then referred to a specialist hospital for more rehabilitation and reconstructive surgery.


The patient is a 66-year-old male with a history of hypertension, hypertrophic obstructive cardiomyopathy, Meniere's disease with intermittent lightheadedness, and gait instability that is controlled with intermittent prednisone. His usual symptoms progressively worsened with new polyuria, polydipsia, decreased oral intake, and nausea. On the day of admission, he complained of lightheadedness as he got up from the toilet and lost consciousness. He had never had a prior syncopal episode.
Initial physical exam revealed irregularly irregular heart rate at 116 bpm, respiratory rate 26, and BP 108/74 with negative orthostatics. Initial labs revealed glucose of 526 mg/dL and blood gas pH of 7.12. Troponin was 13.8 ng/mL. HgbA1c of 11.8 later revealed a new diagnosis of diabetes mellitus. EKG showed atrial fibrillation with rapid ventricular response as well as ST elevations in leads II, III, aVF, and V3–V6 ().


A 43-year-old man came to our Institution to undergo a PCNL procedure. A ureteral Double-J stent had already been placed in another Institution 6 months before to relieve symptomatic hydronephrosis. His previous surgery included three left ureteroscopies and four left renal extracorporeal lithotripsies for urinary stones. No other relevant pathologies emerged from his medical history and no previous metabolic analysis was carried out.
The patient was 182 cm tall and his body mass index was 22.6 kg/m2. Vital signs were normal. Routine preoperative work-up and urine culture were normal.
Preoperative noncontrast CT scan revealed multiple and large stones, involving left renal pelvis and lower pole calix (). Pelvic stone was about 4 cm in length whereas caliceal stone was 1.5 cm in length. Mild hydronephrosis was present and density of stones was about 1100 Hounsfield units.
The patient was placed in the Galdakao-modified supine Valdivia position. After Double-J stent removal, retrograde pyelography was carried out after placement of an open-ended ureteral catheter 5F (Boston Scientific®) under fluoroscopic guidance. A percutaneous renal access was carried out by puncturing a medial-posterior kidney calix with a Chiba needle 18-gauge under biplanar fluoroscopic and ultrasound guidance. The tract was dilated to 24F by using a balloon (BARD X-Force®) and an Amplatz working sheath 24F was located. The stone was fragmented using combined ballistic and ultrasound (SWISS LITHOCLAST-MASTER®). The stone fragments were extracted by using a 10F extractor (Perc N-Circle-COOK®). A 1.5 cm lower pole stone could not be removed by the ante grade approach because it was located in a calix not aligned to the access accomplished. Although the Validivia–Galdakao position usually allows for combining retrograde and antegrade approaches and at the same time improving stone-free rate, retrograde management of the residual stone during the same session was not attempted because of poor ureteroscopic vision for little bleeding. At the end of the procedure, a Double-J ureteral stent 6F (Boston Scientific) and a nephrostomy tube 20F (Soft Drain BARD®) were placed for a second-look PCNL. The time of the operation was about 90 minutes and blood loss was negligible during the surgery. Laboratory studies showed stable levels of hemoglobin, hematocrit, and renal function.
The following postoperative 7 days were uneventful and the patient was scheduled for the residual stone treatment through the same path accomplished before. In our practice, quick PCNL second looks are usually performed under sedation without an Amplatz sheath and by using a 15 flexible nephroscope, Holmium laser to fracture stones, and no-tip baskets to retrieve fragments. The procedure is usually completely nontraumatic because the discrepancy between the 20F mature tract and the 15F flexible nephroscope allows, immediately after the tube removal, to get through the path to the kidney collecting system without any friction and patient discomfort. One centimeter length residual stone was still present in a lower calix because it was unreachable through the path established and the patient was discharged with the only Double-J stent the day after. The plan was to perform a retrograde intrarenal surgery (RIRS) in 3 to 4 weeks to complete the treatment.
On the 7th day after surgery, the tract was completely sealed but the patient started to complain about flank discomfort and fever. Hemoglobin, hematocrit, and renal function were stable. Ultrasonography and CT scan with intravenous contrast showed a 6 cm subcapsular intraparenchymal collection. A belated sequence of CT scan showed accumulation of contrast into the collection. After 3 days of conservative management with ciprofloxacin 1 g per day, the subcapsular collection did not resolve (). The patient was readmitted and a percutaneous 6F mono-J drainage was located in the collection under a combined ultrasonographic and radiologic guidance by an interventional radiologist (). Although the first output from the drainage was purulent, later on it turned into clear urine throughout the days after. No bloody output was observed neither when the drain was placed nor in the days after, and the hemoglobin and hematocrit remained stable. Culture analysis was positive for Escherichia coli infection and carbapenemic-targeted antibiotic was offered to the patient until drainage removal. The patient was discharged after 2 days when the fever and the pain ceased. From the collection, about 400 cc was drained in 7 days and the drainage was removed after 8 days when the liquid output was <10 cc per day (). The patient recovered and RIRS was scheduled in 1 month to manage the residual stone.


A 41-year-old Caucasian gentleman with a background of surgical closure of atrial septal defect aged 2 years old underwent routine echocardiography (surgical details unknown). The study was remarkable for the presence of a dilated right coronary ostium. No previous imaging was available for comparison, reflecting the remote nature of the patients' previous surgery. There was no history of collagen vascular diseases. There was no suggestion of an episode typical of Kawasaki disease in childhood. The patient was asymptomatic with preserved effort tolerance. Physical examination was normal. Specifically, there was no evidence of cardiac failure and no murmur was noted on examination. Transthoracic echocardiography demonstrated aortic root dilatation at the level of the sinus of Valsalva (4.9 cm) with an appearance suggestive of right coronary artery ostial dilatation; the proximal ascending aorta measured 4.1 cm. The remainder of the echocardiogram was within normal limits. There was no evidence of resting ischaemia or previous infarction. Given these findings, CT coronary angiography was performed which demonstrated dilatation involving the right sinus of Valsalva extending into the ostium of the right coronary artery (). The remainder of his right coronary artery and the left sided coronary arteries were of normal calibre and had minor irregularities only noted. Subsequent diagnostic cardiac catheterisation confirmed the appearance of severe dilatation of the right coronary ostium (). There was no obvious stenosis noted distal to the aneurysmal dilatation.
Due to concerns regarding possible subsequent coronary artery dissection, aneurysmal thrombus formation, and aneurysm rupture, the patient underwent surgical intervention.
Following redo sternotomy, a transverse aortotomy was performed. Inspection confirmed a right coronary sinus of Valsalva aneurysm involving the right coronary ostium. A bovine pericardial patch was placed between the aortic annulus and sinotubular junction excluding the aneurysm. The true ostium of the right coronary artery was then anastomosed to a saphenous vein graft; the internal mammary artery was of insufficient length to serve as a conduit. The postoperative course was uneventful.
Postoperative echocardiography was within normal limits. Repeat CT imaging confirmed bypass graft patency and no residual right coronary ostial dilatation ().


A 65-year-old woman was referred by her dentist to the emergency department of Spedali Civili Brescia, Italy, with a large swelling of the face and the neck that commenced at the upper left eyelid. Pain was not aggravated on palpation, but dysphagia, dyslalia, and subcutaneous crepitus were evident. An intraoral examination revealed no visible incision; slight bleeding was apparent around an implant in the region of 34. She reported that she had undergone an airflow procedure performed by a dental hygienist in the region of the implant earlier the same day and had felt pain in the left submandibular region during the procedure. She was diagnosed with subcutaneous emphysema (procedural complication).
Her vital signs were as follows: heart rate 65 beats per minute; blood pressure 145/90 mmHg; respiration 19 breaths per minute; and oxygen saturation 96%.
To evaluate the dysphagia and dyslalia, we obtained CT from the maxillofacial region to the thorax. These revealed significant soft-tissue emphysema extending from the left parietal region to the left soft tissue of the face, then bilaterally to the paraspinal muscles of the neck and the pterygoid regions, and posteriorly to the pharynx.
More distally, the emphysema splayed the vascular bundle and the thyroid lobes, widened the pectoral muscles posterior to the clavicles, descended to the mediastinum (where it was evident principally in the anterior part of the perivascular adiposity), and surrounded the trachea and oesophagus posteriorly.
The emphysema terminated in the region of the upper diaphragm (Figures –).
To prevent the expansion of the emphysema, the patient was immediately hospitalised in the Maxillofacial Surgery Unit and was prescribed intravenous antibiotics because of the high risk of infection associated with the access of large amounts of air and water to soft tissue during a dental procedure. Indeed, dental compressed air, and not sterile water, contains Legionella and Pseudomonas, rendering antibiotic therapy and microbiological monitoring even more critical [–].
The patient was discharged 4 days later.


This case corresponded to a 7-year-old Arab girl who was diagnosed with PCG at the age of 4 months. When first seen at HCSC (21-months-old) she had undergone two goniotomy and one trabeculectomy in both eyes (OU). The ophthalmological examination revealed an increased IOP (35 mmHg) and an enlargement of the corneal diameter OU despite being on topical treatment with two glaucoma drugs. A third goniotomy and a second trabeculectomy were then performed OU. At the age of 3, the IOP rose again so that a new trabeculectomy (the 3rd) OU was undertaken. During the following years, topical glaucoma treatment with three drugs was necessary to keep the IOP under control. At the age of 5, an Ahmed valve was implanted due to bilateral corneal edema which resulted in recovery of the corneal transparency and control of the IOP. Since then, the target IOP has been achieved without topical glaucoma medication in the right eye (OD) and with one glaucoma drug in the left eye (OS) for up to 2 and a half years of follow-up “”.
The tissue analyzed in this patient was obtained from the third trabeculectomy in the OD. In semithin sections, we observed that the area corresponding to the outflow pathway was replaced by a compact tissue in which neither the SC nor other components of the aqueous outflow pathway could be identified “”. At TEM, the region corresponding to the UTM and CTM comprises a compact tissue constituted by trabecular beams with no intertrabecular spaces “”. At higher magnification, it could be seen that in some areas, the trabecular beams fused together due to endothelial cells loss “”. The trabecular beams were constituted by collagen, elastic-like fibers and large endothelial cells “”, some of them being necrotic. The collagen, predominantly of fibrillary type, was the main component of the trabecular beam “”. The ciliary muscle was abnormally inserted, overlapping the undifferentiated trabecular meshwork “”.


This case corresponds to a 12-year-old Caucasian girl who was diagnosed at birth of bilateral PCG. At the age of 4, she presented the HCSC with a past medical history of two trabeculotomies (at 7 and 15 days old) and a pediatric Ahmed valve (at 8 months old) OU. At age 6, she underwent a trabeculectomy in the OS due to poor control of IOP despite being on topical glaucoma treatment with 3 drugs. During the following 3 years, the IOP in the OS was pharmacologically controlled. At age 9, she required a trabeculectomy OU. Five months later, the pediatric Ahmed valve was replaced by an adult implant in OU. Since then, the IOP has been controlled OU for up to 3 years of follow-up with one topical glaucoma medication “”.
The tissue analyzed in this instance was taken from the trabeculectomy of the OD. The histology of this surgical sample was influenced by the previous trabeculotomies suffered by the patient. For this reason, the SC and JCT could not be appropriately evaluated. However, some rest of trabecular beams, as observed in the semithin section “”, remained, allowing its analysis. No collector channels were detected “”. The analysis of the ultrastructure revealed that in the region corresponding to the TM remains there was a compact tissue composed of abundant coalescent fibrillary collagen, large amount of elastic fibers and scarce endothelial cells which showed signs of necrosis and apoptosis “”. It was difficult to identify the trabecular beams and no intertrabecular spaces were observed “”. By contrast, in some areas the trabecular beams and the intertrabecular spaces were present “”. Most endothelial cells lining these trabecular beams had disappeared, those remaining showing signs of necrosis and apoptosis “”. The trabecular beams were filled by coalescent fibrillary collagen and abundant elastic-like fibers “”. The ciliary muscle was inserted posterior to the TM “”.


This case corresponds to a 16-year-old Caucasian male, who was diagnosed with PGC at the age of 5 and was brother of case 2. He was on medical treatment (one drug) for elevated IOP from age 5. At presentation in HCSC, an enlarged corneal diameter was the only sign detected. At the age of 16 a goniotomy in the OS was performed because of poor IOP control despite being on three topical glaucoma medications. Five months later, a trabeculectomy was required in the OS, achieving IOP control since then without medication. The IOP in the OD has been controlled up to the present with three topical glaucoma medications, and no previous surgical procedures were required.
The trabeculectomy from the OS of this patient contained two pieces of tissue, one corresponding to a part of the sclera and another containing the rest of the sclera, the SC and the TM. In semithin sections, we observed a SC with an open lumen, a quite compact CTM, and an UTM with well-developed intertrabecular spaces “”. In either pieces of sclera, we found no collector channels. The TEM revealed that most of the SC endothelial cells had disappeared with only debris of necrotic cells being observed “”. The JCT was composed of alternating layers of abundant ground substance, elastic-like fibers, fibrillary collagen and necrotic stellate cells “”. The CTM was difficult to recognize because the tissue was quite disorganized “”. In some areas the trabecular beams were separate and the zones that would correspond with the inter-trabecular spaces were occupied by the remains of necrotic cells and by collagen that had been detached from the trabeculae “”. In other areas the trabecular beams were fused, the boundary between them being recognized by the presence of necrotic cells debris “”. The trabecular beam had basal membranes, many elastic-like fibers, fibrillary collagen and abundant ground substance “”; the latter gave to the trabecular beam an “empty” appearance “”. The endothelial cells that delineate the trabecular beams were mostly necrotic “”, and those remaining showed autophagic activity “”.
As occurs in the CTM, the UTM core had an “empty” appearance in some regions due to the presence of abundant ground substance “”. In addition, there were elastic-like fibers and abundant type VI collagen, which intermingled with the collagen of the basal membranes (type IV) “”. In some zones the whole trabecular beam was filled up by collagen VI “”. The type VI collagen was degraded in the periphery of the trabecula and broken away from it “”. All endothelial cells lining the trabecular beams had disappeared due to cell death by necrosis “”. In this surgical piece of trabeculectomy the ciliary muscle was not included.


Patient 4 was a 8-year-old Caucasian girl who was diagnosed with PGC at birth. She underwent a goniotomy at HCSC 6 days after birth. A second and a third goniotomy performed at age 6 months and 16 months, respectively, kept IOP under control without medication until the age of 3 years. At that time, a trabeculectomy in the OD was performed because an increase in the papillary excavation was detected. At the age of 4, she underwent the same surgical technique applied to the OS due to poor IOP control. Since then, her IOP was controlled with three topical glaucoma medications in the OD and without medical treatment in the OS for a follow-up period of 4 years “”.
In semithin sections obtained from the trabeculectomy of the OS, we observed a SC with a wide open lumen and a well-developed TM with abundant intertrabecular spaces “”. No collector channels were observed “”. However, at TEM, most of the structures of the aqueous outflow pathways showed pathological changes. Some of the endothelial cells lining the inner wall of the SC were necrotic “ inset” and in those remaining, few vacuoles were observed “ inset”. The JCT had two clearly differentiated areas “”. The first one consisted of a thick band of fibrillary collagen densely packed “” located next to the endothelial cells of the inner wall of the SC. In this band, no JCT stellate cells were observed and the elastic-like fibers were difficult to find “”. In some zones, “optically empty spaces” “” were observed whereas in others the collagen fibers were so coalescent that those spaces could not be discerned “”. Underneath this band of collagen, a second area formed by stellate cells (some of them necrotic), abundant elastic-like fibers, fibrillary collagen and “optically empty spaces” was observed “”. In the CTM some of the trabecular beams were fused due to the disappearance of endothelial cells “” while others, showed large intertrabecular spaces “”. Most of the CTM trabecular endothelial cells were necrotic “”. The CTM trabecular core possessed abundant coalescent fibrillary collagen that mostly filled the trabecular beam. Besides, scarce “optically empty spaces”, elastic-like fibers and basal membranes (thickened in some areas), were visible “”. The UTM had large intertrabecular spaces “”. The core of the UTM beams was filled up by coalescent fibrillary collagen, basal membrane collagen and elastic-like fibers “”. The collagen was arranged in onion-like layers “”. Apparently, all the UTM endothelial trabecular cells were necrotic “”. The ciliary muscle was abnormally inserted, overlapping the SC “”.


Patient 5 was a three-and-a-half-year old Caucasian female who was diagnosed with PGC at HCSC when she was one month old. This patient had an enlarged cornea, corneal edema and high IOP by digital palpation OU at presentation. A bilateral trabeculectomy was performed which resulted in corneal transparency and IOP control thereafter “”.
In this patient, the piece of trabeculectomy from the OD was considered for analysis. A developed SC “Figs , ” and collector channels “” with open lumens were observed. The endothelial cells covering de inner wall of the SC showed some giant vacuoles and abundant caveolae “Figs and ”. The JCT was thick, due to the presence of abundant and disorganized fibrillary collagen and abundant stellate cells (resembling fibroblast) and elastic-like fibers “Figs and ”. “Optically empty spaces” were observed between the fibrillary collagen “Figs , ”. All these findings result in a JCT with a compact appearance “”. The CTM was formed by several layers of trabecular beams. Although these trabeculae were quite compact, small intertrabecular spaces between them were visible “Figs and ”. The trabecular beams were lined by endothelial cells which were separated from the core of the trabecula by a basal membrane “”. These cells showed numerous pinocytosis vesicles and dense bodies indicating a considerable phagocytic activity “”. The core of the CTM trabecular beam possessed abundant disorganized fibrillary collagen that mostly filled the entire trabecula “”. In addition, a few “optically empty spaces” containing an electron-lucent ground substance were observed “”. The UTM were more separate between them than the corneal ones “Figs and ”. Many necrotic endothelial cells appeared “” together with a large amount of disorganized fibrillary collagen filling the trabecular core “”. Electron-lucent ground substance and elastic-like fibers were also found “”. The ciliary muscle was inserted posterior to the TM “”.


A 70-year-old male presented for a complete skin examination in July 2016. He had a history of actinic keratoses (AK) and three basal cell carcinomas. He had new skin lesions on his nose and back.
His past medical history is significant for benign paroxysmal positional vertigo (BPPV), hyperlipidemia (treated with atorvastatin), gout (treated with allopurinol), nephrolithiasis and recent stenting of his right coronary artery one month ago and currently receiving (clop nephrolithiasis dogrel).
The cutaneous examination was remarkable for 22 erythematous scaling plaques on his scalp, face, and arms, consistent clinically with actinic keratoses. He had yellow longitudinal streaks on his left great and fifth toe, consistent with onychomycosis. On his nasal tip, there was a 3 x 3 mm pearly-colored papule with a red dot of less than 1 mm in the center (Figures -). In addition, there was a 1 x 1 mm black macule with an adjacent 6 x 6 mm white plaque on his upper mid back (Figures -).
The actinic keratoses were treated with liquid nitrogen cryotherapy and the patient declined treatment for his toenail fungal infection. The back and nose lesions were each biopsied using a punch and shave technique, respectively. The nasal tip lesion red dot showed superficial buds and nodular aggregates of basaloid tumor cells extending from the epidermis into the dermis; there were also telangiectatic blood vessels in the papillary dermis (Figures -). The upper mid back lesion showed an atypical proliferation of basaloid cells both emanating from the epidermis as oblong clusters and as discrete nests in the dermis; focally, some of the basaloid cells harbored cytoplasmic melanin pigment.
Correlation of the clinical presentation and pathologic findings of the nasal tip lesion was a red dot basal cell carcinoma (with superficial and nodular tumor aggregates). The tumor was excised using the Mohs technique. Two stages were required for cancer removal; the size of the defect was 11 x 10 mm. A full-thickness skin graft was used to repair the surgical wound.
The pigmented superficial and nodular basal cell carcinoma on his upper mid back was excised and the surgical wounds were closed with complex layered closure. Follow-up examination five months later showed excellent healing of both surgical sites and no evidence of recurrence.


A 71-year-old male presented for a complete skin examination in November 2016. He had a history of actinic keratoses, a basal cell carcinoma, and a squamous cell carcinoma. He had new skin lesions on his nose.
His past medical history was significant for hyperlipidemia (treated with simvastatin) and coronary artery disease (and currently receiving clopidogrel).
The cutaneous examination was remarkable for 11 erythematous scaling plaques on his face and arms, consistent clinically with actinic keratoses. There is a 1 x 1 mm red dot surrounded by a 2 x 2 mm flesh-colored papule on his left nostril (Figures -). The lesion did not blanch when pressed with a glass microscope slide (Figure ).
The actinic keratoses were treated with liquid nitrogen cryotherapy. The lesion on his left nostril was biopsied using the shave technique. Microscopic examination of the lesion showed nodular aggregates of basaloid tumor cells extended from the adjacent epidermis into the dermis. There were also telangiectatic blood vessels in the papillary dermis (Figures -).
Correlation of the clinical presentation and pathologic findings of the nasal tip lesion was a red dot basal cell carcinoma (with nodular tumor aggregates). Excision of the tumor using the Mohs technique is planned.


A 74-year-old female presented for a complete skin examination in May 2016. She had a history of actinic keratoses and five basal cell carcinomas. There were several new skin lesions since her last visit six months earlier.
She also had a previous mantle cell lymphoma. In February 2015, she had achieved a clinical remission after receiving three cycles of Rituxan and Treanda that was followed by an autologous stem cell transplant in April 2015. She also has a past medical history of diabetes mellitus (controlled with diet), obstructive sleep apnea (treated with continuous positive airway pressure therapy) and a recent deep vein thrombosis (that is currently being treated with Coumadin).
The cutaneous examination was remarkable for four erythematous scaling plaques on her arms and back, consistent clinically with actinic keratoses. She had three red plaques, each approximately 6 x 4 mm, on her right upper central back, right upper mid back and right hip (Figure ). In addition, she had a 2 x 2 mm red dot within a 7 x 9 mm area of erythema on her left mid back (Figures -); the lesion blanched when pressed with a glass microscope slide (Figure ).
The actinic keratoses were treated with liquid nitrogen cryotherapy. The back lesions were each biopsied using a shave technique. The red dot on the left mid back showed superficial buds and nodular aggregates of basaloid tumor cells extending from the epidermis into the dermis; there were also telangiectatic blood vessels in the papillary dermis (Figures -). The other back lesions and right hip lesion also showed similar appearing tumor cells along the basal layer of the epidermis and in the superficial dermis.
Correlation of the clinical presentation and pathologic findings of the left mid back lesion was a red dot basal cell carcinoma (with superficial and nodular tumor aggregates). The tumor was excised using the Mohs technique. Only one stage was required for cancer removal; the size of the defect was 15 x 11 mm. A complex layered closure was used to repair the surgical wound.
The three superficial and nodular basal cell carcinomas on the right back and right hip were also subsequently excised using the Mohs technique; the surgical wounds were closed with complex layered closures. Follow-up examination six months later showed excellent healing of the four surgical sites and no evidence of recurrence.


Patient 1 (II-2, a) is a 15-year-old boy who tripped and fell frequently since infancy. He fractured his ankle at 10 years old and started to walk with an awkward gait after his plaster was removed. Subsequently, he developed slowly progressive weakness of his lower limbs.
Examination revealed muscle wasting of both distal leg and foot muscles, with inverted champagne bottle appearance of bilateral legs and foot drop. He walked with a high stepping gait. Examination of his upper limbs showed mild thenar and hypothenar muscle wasting with normal power. The peripheral reflexes and sensation were normal. He had no facial weakness, ptosis or respiratory problem. Nerve conduction studies (NCS) showed absent compound muscle action potential upon supra-maximal stimulation of both peroneal and tibial nerves. The nerve conduction velocities of the median and ulnar nerves and the sensory nerve action potential of both sural nerves were normal. The result was suggestive of axonal degenerative motor neuropathy. Previous genetic tests showed no duplication of PMP22 or mutation in GJB1 and MFN2.
The patient’s parents are cousins, and there is also a history of consanguineous marriage in the last three generations of the family. Upon inquiry, the 17-year-old sister of Patient 1 was noted to be clumsy since infancy and could only walk independently from 18 months onwards. She developed frequent falls since 14 years of age but she remained ambulatory. Examination revealed muscle wasting of distal lower limb muscles and bilateral foot drop. She also walked with a high stepping gait. Nerve conduction studies also revealed similar findings to that in Patient 1. No previous genetic test was performed in this sibling.


A 81-year-old lady who has known critical aortic stenosis (AS) with left ventricular dysfunction presented to the hospital with ischemic chest pain and worsening exertional dyspnea. She was an ex-smoker but had active history of COPD and depression. There was no history of stroke or peripheral vascular disease.
While awaiting an angiogram, she had brief periods (lasting <30 sec each) of altered speech, confusion, and seizure-like activity without any neurological or postictal sequelae. CT brain and carotid Doppler ultrasound ruled out a cerebrovascular accident. Echocardiogram confirmed critical AS (mean gradient of 74 mmHg) with moderate mitral regurgitation and pulmonary hypertension (PA systolic 51 mmHg). The LV function was moderately impaired. Her ongoing hypotension (70–85 mmHg systolic BP) necessitated her transfer to intensive care unit (ICU) for hemodynamic optimization prior to her scheduled cardiac surgery. In ICU, her BP improved to around 90/50 mmHg (MAP between 59 and 74 mmHg), with intravenous fluid resuscitation without the need for vasopressor support. She had arterial and central venous cannulation prior to cardiac surgery. Her neurological examination prior to surgery was essentially normal.
The operation was undertaken on cardiopulmonary bypass (CPB) with general anesthesia with no epidural analgesia. CPB cannulation was established uneventfully in the routine fashion, by cannulation of the ascending aorta for arterial return and 2-stage right atrial cannulation for venous drainage. The aorta was cross-clamped and heart arrested with antegrade followed by retrograde tepid blood cardioplegia. She had mitral annuloplasty and porcine bioprosthetic aortic valve replacement. Uncomplicated CABG was performed with a single right long saphenous vein graft to the mid-right coronary artery. Following cross-clamp release, heart required single DC (10J) shock prior to running stable sinus rhythm. CPB was then slowly discontinued. Her blood pressure was maintained with 0.04 mcg/kg/min of adrenaline and 0.1 mcg/kg/min. The total CPB time was 1:38:16 and cross-clamp time was 1:25:29. Patient required 4 mg of metaraminol while on CPB.
Upon return to ICU, there was profound hemodynamic instability and low cardiac output with high vasopressor and inotropic requirements [noradrenaline as high as 50 mcg/min (1 mcg/kg/min) and adrenaline 0.2 mcg/kg/min; cardiac index of 1.6 L/min/M2, SVR 1415 dynes/sec/cm5]. The cardiac output subsequently improved (CI 2.6 L/min/M2, SVR 986 dynes/sec/cm5) with increase in inotropic support (adrenaline to 0.4 mcg/kg/min). Urgent hemofiltration was initiated for worsening metabolic acidosis and anuria. She also had hyperlactatemia with serum lactate of 10 mmol/L, which was multifactorial, probably a combination of hypotension, poor perfusion, adrenaline use, and liver dysfunction. Immediate bedside transthoracic echocardiogram revealed no aortic or mitral regurgitation with improved LV function and ruled out pericardial effusion. She also had elevated transaminase levels, suggestive of ischemic hepatitis. Given the hemodynamic instability, the patient was deeply sedated and intermittently paralyzed making a thorough neurological examination impossible.
The next day, she started to improve with adrenaline weaned off and noradrenaline stabilized between 0.2 mcg/kg/min and 0.36 mcg/kg/min. She started obeying commands moving her upper limbs but not the legs. Neurological examination revealed no motor power in all lower limbs muscle groups, a sensory level at T6-T7, light touch, and proprioception preserved; however, pain and temperature sensations were absent. There were no bowel or bladder problems. Urgent CT brain was essentially normal; CT spine revealed chronic degenerative changes, but was unable to exclude ischemic spinal injury. Patient was extubated on day-2. Subsequent MRI spine showed abnormal signals beginning at T6-7, and extending down to the conus with the cord expanded centrally, the signal was abnormal with a rim of normal periphery all suggestive of anterior spinal artery (ASA) ischemic syndrome (Fig. ). There was no evidence of aortic dissection or hematoma. MRI brain interestingly revealed two small subcortical acute ischemic infarcts in bilateral cerebral hemispheres, most likely representing embolic phenomena (Fig. ).
The patient was provided supportive care including hemodynamic optimization and renal replacement therapy (RRT) to assist in her recovery. She required RRT for a further 2 weeks. Her acute kidney injury improved subsequently and was discharged to the ward. She was transferred for rehabilitation after 3 weeks. She underwent intense rehabilitation for 6 weeks and was subsequently discharged home. At 1 year follow-up, her kidney function had normalized. There has been no improvement in lower limb motor power.


A 61-year-old male with history of hypertension presenting with transient consciousness disturbance and suspected seizure attack was sent to emergency room (ER) by ambulance. At arrival, his was clear, afebrile, no tachypnea, no tachycardia, no hypotension (blood pressure [BP]: 115/59 mm Hg), and no hypoxia (SpO2: 100%). He reported a sensation of out of breath and then loss of consciousness. He denied headache, chest pain, back pain, and abdomen pain. Initial 12-lead electrocardiogram (EKG) demonstrated normal sinus rhythm. Loss of consciousness with convulsive movement was noted in the ER while we were waiting for laboratory tests results. Therefore, he was transferred to critical area and on EKG monitor, BP monitor, and oximeter. His hemogram indicated mild anemia (hemoglobin: 11.8 mg/dL and hematocrit: 35.1%). Liver function test and creatinine level were unremarkable, but there were increased C-reactive protein (122 mg/L) and d-dimer (1.56 mg/L) level. Blood gas analysis was also unremarkable. Computed tomography (CT) of head without contrast enhancement did not reveal ischemic changes, intracranial hemorrhages, or space-occupying lesions. However, his chest radiography indicated a widening of mediastinum, and contrast-enhanced chest CT demonstrated Stanford type A aortic dissection extending from the aortic root to the bifurcation of common iliac arteries (Fig. ). Consciousness loss accompanying with upward gaze and limb convulsion for about 10 seconds was noted again in ER, and EKG monitor recorded a transient cardiac asystole then spontaneous recovery of sinus rhythm without cardiopulmonary resuscitation (Fig. ). The patient underwent a successful operative replacement of the ascending aorta and semiarch replacement with a Dacron graft as well as aortic valve suspension.


A 16-year-old Chinese girl presented complaining of anemia and interval hematochezia. Her hemoglobin was 54 g/L on admission. Capsule endoscopy and double-balloon enteroscopy showed mucosal hyperemia, edema and mass protrusion on the ileal wall. Computed tomography (CT) scans and three-dimensional reconstruction revealed a 10.0 × 7.3 × 5.3 cm irregular mass that had developed from the ileal wall in the right lower quadrant (Fig. -). The lesion showed intense but inhomogeneous enhancement following contrast administration (Fig. -), particularly in the arterial phase. There was a small amount of effusion in the pelvic cavity. Pelvic magnetic resonance imaging (MRI) indicated a right ovarian cyst in addition to the above mass. Both CT and MRI prompted suspicion of malignant GIST of the small bowel. Her serum CA-125 was slightly increased (50.5 U/mL, standard 0–36 U/mL), but the other markers were within normal limits. The tumor and a loop of small intestine were resected through a right ventral midline incision. The patient recovered uneventfully. Postoperative bone scintigraphy proved that there was no lesion in the skeletal system (Fig. ). Her chest CT scan and cerebral MRI were also unremarkable. Thus, the patient was classified as T2aN0M0 according to the 8th edition of the AJCC Cancer Staging Manual.
On laparotomy, a large cystic and solid mass 10.5 cm in diameter was found arising from the ileal wall. The cut tumor surface showed large central hemorrhagic and necrotic changes and pseudocystic degeneration. The tumor tissue was mostly light gray and solid, with some softer and more friable reddish congested areas (Fig. ).
Cross sections revealed solid nests of small round tumor cells arising from the muscular layer and infiltrating all layers of the ileum wall. Cystic and hemorrhagic changes were seen on part of the sections, as were sharply demarcated borders that were frequently covered by intact serosa. No vascular tumor embolus or perineuronal invasion were observed. The serosal layer and the surgical margins of the specimen were free of disease. Under high-power view, tumor cells were round or elliptical, possessing scant eosinophilic cytoplasm and abortive pseudorosette formation. The tumor cell nuclei were round, with exquisite chromatin, ambiguous nucleoli, and 9/10 high-power-field pathological mitoses (Fig. -). Tumor cells showed positive immunoreactivity for Vimentin and CD99 (Fig. ) and moderate staining for Cam5.2, Syn and PR (Fig. ). Results were negative for CKpan, LCA, S-100, HMB45, Melan-A, CD31, CD34, NSE, P53, CD56, CgA, SMA, Desmin, CD117, Dog-1, ER, Bcl-2, and alpha-inhibin.
Dual color break-apart probe FISH examination showed that 90% of the cells (100 counted cells per slide) exhibited 1 yellow and 1 red signal (1F1R) and that 6% of the cells exhibited 1 yellow, 1 red and 1 green (break-apart) signal (1F1G1R). However, only 4% cell had two yellow signals, which proved a break of the EWSR1 locus (2 F) (Fig. -).
Transmission electron microscopy revealed dense clusters of tumor cells, interspersed with a few interstitial cells (Fig. ). The tumor cells were small and irregular, with scant cytoplasm and organelles, and significant nuclear atypia (Fig. ). Some cells had small nucleoli (Fig. ). Occasionally, gap junctions between the cells were observed (Fig. ), but neuroendocrine granules in the cytoplasm were rarely seen (Fig. ). Most cells had glycogen particles attached to the endoplasmic reticulum (Fig. ).
The patient underwent an exploratory laparotomy, and tumor resection was performed along with 60 cm of ileum. The patient refused chemotherapy and/or radiotherapy as adjuvant treatments. She is currently alive (10 months after the surgery) without any signs of recurrence.


An 86-year-old Japanese man underwent esophagogastroduodenoscopy for the investigation of anemia. An acute gastric mucosal lesion was identified in the stomach that was thought to be the cause of his anemia. In addition, a solitary submucosal tumor approximately 10 mm in diameter was found in the second portion of the duodenum (). The top of the tumor was slightly depressed, showing a reddish color. Histological analysis of the biopsied samples obtained from the reddish part of the tumor revealed adenocarcinoma. The patient was referred to our hospital for further investigation and treatment.
The patient had been consuming medication for atrial fibrillation, diabetes mellitus, and constipation but had no history of gastroduodenal disease. A physical examination revealed conjunctival pallor and arrhythmia, but there were no abnormalities in his abdomen. Laboratory findings revealed decreased levels of red blood cells (4.03 × 106/mm3), hemoglobin (10.6 g/dL), hematocrit (34.2%), serum iron (20 μg/dL), and ferritin (29.6 ng/mL). The levels of carcinoembryonic antigen and carbohydrate antigen 19-9 were within the normal range. The patient tested positive for Helicobacter pylori infection.
Hypotonic duodenography showed a submucosal tumor with bridging folds in the inferior duodenal angle (). Endoscopic ultrasonography revealed a hypoechoic mass mainly confined to the mucosal layer (). A small cystic area was also identified on ultrasonography (). Based on the imaging and pathological studies, we diagnosed the duodenal tumor as adenocarcinoma arising from Brunner's glands. Due to the advanced age and multiple underlying disorders, the risks of general anesthesia, artificial ventilation, and radical surgical resection were considered too high. Moreover, endoscopic ultrasonography indicated that the tumor appeared to be resectable using the endoscopic mucosal resection technique. Therefore, we performed endoscopic treatment rather than surgical resection (). Hyaluronic acid solution (MucoUp®, Johnson & Johnson K. K., Tokyo, Japan) was injected into the duodenal submucosa using a 23 G injection needle (TOP Co., Tokyo, Japan). Subsequently the tumor was resected with a bipolar snare (Zeon Medical Inc., Tokyo, Japan). The resected area was closed by metallic clips (Olympus Medical Systems Co., Tokyo, Japan). There were no procedure-related adverse events during or after the endoscopic resection.
Pathological analysis of the resected specimen revealed proliferation of Brunner's glands (Figures and ). Gastric foveolar metaplasia was also identified (, blue square, and ). Atypical cells with pleomorphic macronuclei containing dense chromatin forming an irregular glandular structure were identified towards the lumen in the superficial layer (, red square, and ). Immunohistochemical studies revealed that proliferated Brunner's glands were positive for MUC6 (, white arrow) and negative for MUC5AC (, white arrow). In contrast, most of the cells with gastric foveolar differentiation (Figures and , arrowhead) and atypical cells (Figures and , black arrow) were positive for MUC5AC but negative for MUC6. However, both gastric foveolar metaplasia and atypical cells partly showed dual positivity for MUC5AC and MUC6, particularly in the deeper layers (Figures and , arrowhead). Some atypical cells showed relatively higher positivity for Ki-67 staining (, arrows). Consequently, a diagnosis of adenocarcinoma in situ arising from Brunner's glands was made. Components of the adenocarcinoma, gastric foveolar metaplasia, and Brunner's gland hyperplasia were completely resected by endoscopic mucosal resection.


A 33-year-old lady presented to the emergency department with a chief complaint of exertional dyspnea while performing her activities of daily living. This symptom had progressively worsened over a period of two years, to the point of the development of dyspnea at rest. Her past medical and surgical history was unremarkable. She denied any history of smoking or illegal drug abuse; however, she did have exposure to secondhand smoking during her childhood.
On initial physical examination, the patient was comfortable at rest but had significant dyspnea on minimal exertion. The patient's blood pressure was 95/56 mm Hg; heart rate was 83/min; respiratory rate was 18/min (at rest); and oxygen saturation while breathing supplemental oxygen at 28% was 94%. She had decreased chest movement of the right hemithorax with a significant decrease of air entry in that same side; left lung auscultation revealed clear breath sounds.
A chest radiograph revealed an increased translucency of the whole right hemithorax and lateral displacement of mediastinal structures (see ). A computed tomography (CT) scan of the chest with contrast was then requested to address the abnormalities noted on chest radiography and depicted severe bullous changes occupying approximately 85% of the right lung and displacing the mediastinum and left lung laterally (see ). In addition, smaller bullous changes were noted in the remaining aerated portion of the right lung. The left lung was also significantly compressed but remained aerated.
Based on clinical and radiographical findings a diagnosis of right upper lobe congenital emphysema was made. The patient was tested for α1-antitrypsin deficiency, which was normal with the phenotype being M2 M2.
Pulmonary function tests revealed forced vital capacity (FVC) of 1.26 L (37% of predicted) and forced expiratory volume in 1 second (FEV1) of 0.89 L (33% of predicted), with a FEV1/FVC ratio of 70%. Patient total lung capacity (TLC) was 6.2 L (131% of predicted) and a residual volume of 4.93 L (366% of predicted) with corrected diffusion capacity of 75% of predicted. These findings were consistent with severe obstructive lung disease. A quantitative perfusion scan demonstrated 10% contribution from the right lung with 5% from the right upper lobe and 5% from the right lower lobe. The remaining 90% came from the left lung. A 2D echocardiogram revealed no evidence of shunt and an ejection fraction of 55%. The patient was taken to the operating room for an elective right upper lobectomy; however, intraoperatively, the right upper lobe was found to be hypoplastic, and the majority of bullae were coming from the right lower lobe. A right lower lobectomy was performed and postoperatively the patient was rapidly extubated. Postoperative X-ray chest is shown in . Pathology specimen showed large dilated alveolar spaces with some containing free-floating septa, which was consistent with emphysematous changes. There was no associated inflammation or malignancy (Figures and ). She had significant improvement in her dyspnea and symptoms. Patient was discharged home on the 5th postoperative day.


A 41-year-old, 75 kg, 150 cm (body mass index of 33), woman with thyroid cancer metastatic to the bilateral neck underwent thyroidectomy, including left lateral and bilateral central neck dissection. No chemotherapy or radiation therapy was employed as an adjunctive prior to surgery. Her medical history included well-controlled arterial hypertension and diabetes mellitus type 2. She was a nonsmoker and denied any history of cardiac or pulmonary disease. The physical exam prior to surgery was unremarkable. The initial CT scan of the neck showed no abnormalities of the trachea (). The preoperative vital signs were as follows: blood pressure 121/81 mmHg, heart rate 91/min, respiratory rate of 16/min, temperature 37.4 Celsius, and a SpO2 of 96% on room air.
Standard anesthesia monitoring (ECG, SpO2, ETCO2, and NIBP [IBP added after 4 hours of surgery]) was established and maintained throughout the procedure. After intravenous pretreatment with midazolam 2 mg and fentanyl 0.15 mg, general anesthesia was induced using propofol 200 mg, followed by 80 mg succinylcholine to facilitate intubation. We maintained anesthesia using desflurane inhalation and remifentanil infusion (0.2 μg/kg/min). To minimize the risk of recurrent laryngeal nerve damage, we used an electromyogram endotracheal tube (EMG-ETT, Medtronic), placed using a video laryngoscope and inflated the cuff to a pressure of 25 cm H2O. Then, guaranteed volume, pressure-controlled ventilation was established and we maintained the end-tidal CO2 (ETCO2) levels at 35 mmHg with tidal volumes (TV) of 350 ml and a respiratory rate of 15/min. The adjustable pressure limiting valve was set to 5 cm H2O and average peak inspiratory pressures (PIP) and mean airway pressures (AP mean) were 22 cm H2O and 12 cm H2O, respectively.
During positioning, the patient coughed (TV 139 ml, PIP 37 cm H2O, AP mean 16 cm H2O) and received 50 mg propofol (i.v.). During cervical lymph node dissection and thymus removal, the care team noted a gurgling and bubbling sound radiating from the thorax. We checked the cuff pressure and tube position using a video laryngoscope, but no abnormalities were found. Three hours into the surgery the thyroid was unroofed and the thyroid ligament was dissected. In addition, all the lymphatic tissue from the carotid and innominate artery towards the midline was cleared out. In addition, the esophagus nerve and the side of the trachea from the thyroid cartilage down to the innominate artery just below the sternum were skeletonized. Again, a cycle of gurgling and bubbling of air into the neck occurred and the patient became difficult to ventilate. The patient slightly desaturated (SpO2 91%) and low tidal volume alarms were noticed. The FiO2 was set to 1.0 and the patient was manually ventilated. During manual ventilation decreased resistance was noted and TVs were as low as 42 ml, PIPs were as low as 11 cm H2O, and AP mean was as low as 7 cm H2O. The ETCO2 was 30 mmHg due to increased manual ventilation rates (30/min). To check for an airway leak, the surgical team poured normal saline over the surgical field and observed bubbles in the saline but could not identify any lacerations of the trachea or pleural defects. As ventilation remained difficult with TVs constantly dropping and requiring consecutive recruitment maneuvers over the next 30 minutes, a bronchoscopy was performed. The cuff was deflated and the ET tube was withdrawn until the vocal cords became visible. Ventilation was paused and the bronchoscopy was performed which showed a normal looking tracheal and lobar bronchial mucosa. However, the distal trachea and left mainstem bronchus appeared dynamically collapsed. Subsequent intraoperative chest X-ray revealed a small left-sided pneumothorax and a mild pneumomediastinum (). The surgeon placed a chest tube to relieve the pneumothorax. Several hours later, the patient once again became difficult to ventilate and a second chest X-ray was performed to verify the correct position of the chest tube. While the left pneumothorax had decreased in size, a new right pneumothorax had developed () and a second chest tube was placed. After parathyroidectomy, near total thyroidectomy, bilateral central neck dissection, and left lateral neck dissection the surgery was aborted after 11.5 hours secondary to recurrent unstable clinical status. The patient was transferred, intubated, and hemodynamically stable, to the intensive care unit. On postoperative day 1, the patient met extubation criteria. Postextubation chest X-ray showed proper chest tube position, but the pneumothoraces had increased (). In the hours following extubation, the patient stated shortness of breath and experienced labored breathing and pressure in the chest. The heart rate [104/min] and the systolic blood pressure became elevated [180 mmHg], and the SpO2 dropped to 80% with a respirator rate of 41/min, indicating acute hypoxic respiratory failure and requiring reintubation (). An echocardiogram showed normal left ventricular function (estimated ejection fraction 55.9%) and a severely dilated right ventricle with mild right ventricular hypertrophy. With poor oxygenation (PaO2 60 mmHg, FiO2 0.8) in the absence of cardiac failure, the care team diagnosed a severe respiratory distress syndrome (ARDS) based on the Berlin definition (PaO2/FiO2 < 100 and PEEP 5+, ) []. Following intubation sequential bronchoscopies revealed no evidence for a tracheal injury. As hypercarbic respiratory failure worsened (PaCO2 71 mmHg, pH 7.1, RR 35/min, PEEP 20 cm H2O, plateau pressure 32 cm H2O, Vt 6.5 ml/kg, I : E ratio 1 : 1, and Adaptive Support Ventilation [Hamilton G5]) with SpO2s in the 80s (FiO2 1.0, O2 saturation mixed venous 55%), the patient was placed on venovenous extracorporeal membrane oxygenation (vvECMO). Over the following three days the patient's respiratory function improved. VvECMO therapy was discontinued and the patient was subsequently extubated. Follow-up echocardiography found normal heart function with an EF of 73%. We discharged the patient from the hospital on postoperative day 12 in good physical condition. Due to posttraumatic stress disorder, the missing right lateral neck dissection was postponed but successfully performed eight months later.


A 33-year-old woman, gravida 8 para 4 abortus 4, was admitted to our tertiary centre with complaints of vaginal spotting and mild lower abdominal cramping. She was in her third week of amenorrhea.
Her obstetric history included four caesarean sections. The patient’s medical history was unremarkable, and her vital signs were within normal limits. The abdominal and vaginal examination revealed mild lower-right quadrant tenderness, and no significant vaginal bleeding was noted. Hemoglobin, complete blood count and serum biochemistry including liver enzymes, blood urea nitrogen and creatinine levels were within normal limits.
At the time of admission, serum level of beta human chorionic gonadotropin (ß-hCG) was 1263 IU/L. Transvaginal ultrasound revealed an empty uterine cavity and a mass of 20×19 mm with a hypoechoic central area in the right horn (). No sign of foetal pole or yolk sac was noted. The Doppler showed the vascular ring sign, which proved an intense peripheral vascularization. Both ovaries appeared normal. No free fluid was noted in the Pouch of Douglas.
Considering the serum level of ß-hCG (<10000 IU/L), the patient’s haemodynamic stability, the size of the mass (diameter <4 cm), the absence of pelvic free fluid and the absence of haematologic, renal and hepatic impairment, systemic MTX therapy was recommended as a first option for treatment. The patient was given an intramuscular MTX (Methotrexate; Atafarm, Istanbul, Turkey) 50 mg/m2 (Day 1). She was hospitalized for seven days after MTX injection and remained asymptomatic during this period. There was a more than 15% decrease in ß-hCG levels between the 4th and 7th days after injection (654 IU/L and 464 IUI/L on Day 4 and Day 7, respectively). In addition, transvaginal ultrasound revealed reduction of peripheral vascularization, and the ectopic mass became more echogenic with the same size. No pelvic free fluid was noted, and the patient did not have any side effects after treatment.
The ß-hCG level became negative 45 days after MTX injection. Follow-up ultrasonography at two months after treatment showed a significant reduction (9×6 mm) in the size of the mass and no peripheral vascularization around it. The mass could not be visualized at the third month after treatment.


A 25-year-old gravida four para two presented at our emergency department with complaints of generalized abdominal pain of six hours duration of acute onset and dizziness. The pain was aggravated upon lying down and radiated to both shoulders. She was in her second week of amenorrhea.
Her obstetric history included one pregnancy termination at 18th weeks due to multiple foetal anomalies three years prior. In addition, she had undergone laparoscopic right salpingectomy due to right tubal pregnancy two years before.
On examination, a mild degree of pallor was noticed. Her pulse and blood pressure were 105 bpm and 90/60 mm Hg, respectively. Her abdominal examination revealed generalized tenderness. Her vaginal examination revealed cervical tenderness, and her fornices were full. Transvaginal US examination revealed an empty uterine cavity, free abdominal fluid and coagulum in the cul-de-sac. At the time of admission, serum level of ß-hCG was >10000 IU/L. A complete blood count on the day of admission revealed a hemoglobin level of 6 g/dL.
The clinical and ultrasonographic findings were suggestive of ruptured ectopic pregnancy. The patient’s consent was taken for an emergency exploratory laparotomy, which showed a massive hemoperitoneum and a ruptured right interstitial pregnancy. The site of the bleeding was clamped, and cornual resection with the conception products inside was done (). Uterine cornu was sutured separately with 0-Vicyrl. The ovaries, left fallopian tube one1 unit of fresh frozen plasma.
The postoperative period was uneventful. Her hematological and clinical biochemistry values were within normal range on her immediate postoperative follow-up and postoperative day 1. The patient was discharged on the third postoperative day and called for the control examination a week later. The control examination findings showed no significance, and the histopathology report of the specimen confirmed interstitial ectopic pregnancy. Serum β-hCG level decreased by 98 % at postoperative day 7.


A 39-year-old gravida 5 para 2 abortus 2 was admitted to our clinic at her eighth week of amenorrhea. Her obstetric history included two normal spontaneous deliveries, and her general condition was stable. Her complaints included vaginal bleeding, left-lower quadrant pain and 17 days of amenorrhea. Abdominal examination revealed left-lower quadrant tenderness. Bleeding from the cervical canal was noted.
At the time of admission, serum level of ß-hCG was 9277 IU/L. Transvaginal ultrasound showed a mass in the left uterine horn and an empty endometrial cavity (). The yolk sac and foetal pole were also noted within the gestational sac. Crown-rump length (CRL) was measured 5.9 mm. No foetal cardiac motion was noted.
The Doppler showed significant peripheral vascularization. The ovaries appeared normal bilaterally, and no free fluid was noted in the Douglas pouch. Serum chemistry values and complete blood counts were within normal limits. The patient was counselled on all available management options, including minimally invasive laparoscopic, hysteroscopic and medical management. She elected first for hysteroscopic exploration and transcervical evacuation under laparoscopic guidance if needed.
After obtaining consent for all of the procedures listed above, hysteroscopy was performed with a 5-mm operative hysteroscope with a 2.9 mm 30º lens (Karl Storz, Tuttlingen, Germany) and normal saline as the endometrial distending media. No pathologic lesion was visualized within the endometrial cavity. The right tubal ostium was identified, and a coagulum-like lesion was visualized in the left tubal ostium bulging into the endometrial cavity. At that point, the procedure was terminated. The cervical canal was dilated to 8 mm, and an 8 mm flexible cannula was placed into the endometrial cavity just near the gestational sac under direct abdominal ultrasound visualization. Vacuum aspiration was performed, and the conception product was removed with several passes. Histopathologic examination results reported necrotic chorionic villi. There was no need for laparoscopy because the abdominal and vaginal ultrasonography provided sufficient visualization.
No complications were noted during the procedure, and the patient was discharged on the same day. She was monitored for serum level of ß-hCG, which was identified to be 156.3 IU/L at the tenth day, 35.01 IU/L at fifteenth and 1.68 IU/L at twenty-fifth day after the procedure.


A 28-year-old gravida one para 0 was referred to our tertiary centre with a diagnosis of ectopic interstitial pregnancy. She complained of amenorrhea for seven weeks and lower abdominal pain. Her abdominal examination revealed lower abdominal tenderness bilaterally. Vaginal examination was normal, but transvaginal ultrasonography revealed left-sided interstitial pregnancy, with gestational sac and foetal pole within. CRL measured 31 mm, and foetal cardiac motion was detected (). The myometrial thickness measured 3 mm around the gestational sac. A subchorionic haematoma with dimensions of 44×11 mm was also visualized near the gestational sac. No free abdominal fluid was noted.
The patient’s general condition was stable. Her pulse was 100 bpm, and her blood pressure was 100/70 mm Hg. Serum chemistry values and complete blood counts were within normal limits. Laparoscopy was performed. A 5 mm incision was made in the umbilicus, and a Veress needle was inserted to obtain pneumoperitoneum. The Veress needle was replaced with 10 mm trocar after confirmation of direct intra-abdominal placement laparoscope was made. An auxiliary 5 mm port was opened thereafter. The exploration revealed bulging of the left cornual region lateral to the round ligament. Bilateral ovaries and fallopian tubes remained normal. No gross placental accreta sign, blood or coagulum was noticed. With the findings mentioned above, we decided to perform transcervical suction under laparoscopic visualization. The cervical canal was dilated to 8 mm, and an 8 mm flexible cannula was placed into the endometrial cavity. The products of conception evacuated with three passes. Frozen pathology revealed products of conception. The left cornual region remained unruptured during the procedure. The patient tolerated the operation well and was discharged the next day. The serum level of ß-hCG was detected as < 5 IU/L on the seventh day after the procedure.


Evelyn is a 47-year-old woman who lives with her 71-year-old mother, who has diabetes and is hypertensive. Complications from the diabetes resulted in the amputation of one leg at the knee and is gradually causing visual impairment and incontinence. Evelyn is the sole caregiver because she is an only child and her own children are too young to give her meaningful assistance. By nature gregarious and extroverted, Evelyn looks forward to her daily quiet time with as little external input as possible, especially after a long day. But caring for a visually impaired mother who relies heavily on audio input to orient and to feel connected with the outside world requires that the radio in her home is constantly blurring. Even though she sincerely dislikes the constant ‘noise’ from the radio, she leaves it on ‘for mother's sake’ and ‘I put up with it’. She came to therapy emotionally and physically exhausted and consumed by the role of providing care. She wanted to ‘find an outlet’ and ‘someone to talk to’.


Mercy is a 28-year-old mother of a 10-year-old boy who is mentally challenged with an expressive speech disorder. She is the sole caregiver of her son because ‘my husband blamed me for Kofi's problems and divorced me when he was barely two years old’. After the divorce she moved home to live with her parents. This placed her as an informal caregiver of her aged father (76 years old) who had an accident a few years prior and lost mobility in his right leg. Her presenting complaints included feelings of depression, guilt, anger and resentment at having to be the primary caregiver to her son and father. She was desperately seeking for a special school where she could place her child, hopefully remarry, find another caregiver for her father and ‘move on’ with her life.


A 40-year-old Liberian man, who had been living in Elwa, Monrovia, travelled by air through Accra (Ghana) and Lomé (Togo) to Lagos, Nigeria. Upon arrival at Lagos International Airport on 20 July 2014, he collapsed and was taken to a private hospital in the Obalende area of Lagos, Nigeria (), where he was admitted. He presented with high fever, lymphadenopathy and sore throat and was treated for malaria and typhoid fever until he later developed diarrhoea, vomiting and microscopic haematuria. The culmination of the clinical presentation, the patient’s non-response to treatment for malaria and typhoid, as well as his epidemiological link to Liberia, raised the suspicion for EVD. On 22 July 2014, blood samples in EDTA tubes and urine samples were collected and sent for viral investigations to the CMUL-LUTH Virology Unit Laboratory. Aliquots of the specimens were sent in parallel to the African Center of Excellence for Genomics of Infectious Diseases (ACEGID) at Redeemers University in Ogun State, Nigeria.
No ethics approval was required for the diagnosis of an infection of great public health importance and which was part of the normal course of management of any infected patient.


An 84-year-old woman presented with a history of multiply recurrent basal cell skin cancers in the face and scalp, initially treated in an outside institution. The first documented occurrences date back to March 2010, at which time she underwent punch biopsy of a right upper lip tumour and shave biopsy of the left forehead and anterior scalp. Pathology confirmed that all three lesions were basal cell carcinoma (BCC). In 2014, she underwent Moh’s surgery for squamous cell carcinoma (SCC) of the right forehead. In March 2014, she had Moh’s surgery for BCC of the mid-forehead. In November 2015, she was referred to our institution for the consideration of radiation therapy to an extensive recurrent BCC of the left forehead and both temple areas extending into the hairline (). The patient had previously been recommended treatment with chemo/immunotherapy for this lesion, but had declined any form of systemic therapy.
Simulation was performed with 3 mm cuts in a SOMATON™ Definition AS 64-slice CT scanner (Siemens Medical Systems®). The clinical target volume (CTV) included the skin surface to the depth of the cranium over the extent of the patient’s scalp (). The planning target volume (PTV) was delineated as the CTV plus 3 mm margin. A 1 cm superflab bolus helmet was created.
The prescribed dose was 50 Gy (2 Gy per session) to the PTV followed by a 10 Gy conedown to the macroscopic disease on the forehead. The 1 cm bolus was used in both phases.
The treatment was delivered on the Edge™ unit (Varian Medical System®) using 6 MV X-rays. Cone beam CT was used prior to every fraction for daily position verification. Patient specific QA was performed using the ArcCHECK™ with verification system. The volumetric arc therapy (VMAT) plan was generated using four half-field arcs and one vertex. Details of each arc are described in and shown in .
The total number of monitor units (MU) per cGy was 4.9 MU/cGy (3.38 MU/cGy for the cone down phase). The treatment required an average of 15 minutes of machine time: 8 minutes for bolus (1 cm) placement and patient set-up, two minutes to obtain and analyse the CBCT images and 5 minutes for treatment delivery including the time for the couch rotation.
The conformity index to the PTV was 1.06.
The dose volume histogram (DVH) (), demonstrated excellent coverage and a homogeneous dose within the target with acceptable dosage of the organs at risk. The homogeneity index: H index = [(D2%-D98%)/D50%] equals to 0.104. The mean dose to the brain in the composite plan is 1892 cGy.
During treatment, the patient only reported mild radiation dermatitis (Grade I). Mild pruritus was present during early follow-up, which resolved with topical medication.
The patient was seen at 6 and 12 months after completing treatment with sustained clinical response. The only acute toxicity was alopecia which is resolving, as seen in her most recent follow up. No late toxicities were reported ().