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A 57-year-old man presented with dyspnea on exertion and leg swelling for four months. He had undergone surgical repair of ruptured right SVA by direct closure at age of 19, and remained asymptomatic after the operation. On physical examination, a grade 3/6 continuous murmur was heard at his right lower sternal border, with increasing intensity while he was leaning forward. Electrocardiogram (ECG) showed atrial flutter with 2:1 atrioventricular conduction (ventricular rate around 100 beats per minute) and complete right bundle branch block. Chest X ray revealed enlargement of right atrium and ventricle, but no pulmonary congestion. Transthoracic echocardiography revealed bicuspid aortic valve and a large cystic structure protruding from right sinus of Valsalva to the right atrium. The wall of the cystic structure was relatively thick and hyperechoic, which was distinct from the congenital form of SVA (Figure a, b). Color duplex showed a continuous shunt from the aorta to the right atrium through a small defect at the posterior aspect of the aneurysm (Figure c, d, e and Additional file : Movie 1). Gradual bulging of the previous patch sutured at the SVA 38 years ago with recurrent rupture was impressed. Multidetector computed tomography also demonstrated a large SVA with a defect connecting to a dilated right atrium (Figure ).Surgical exploration from both right atrial side and aortic side confirmed the communication between SVA and the right atrium (Figures and ). The defect was located at a weak point of suture between the previous patch and the sinus of Valsalva. Double patch method was performed with bovine pericardium patches. From the aortic side a patch was sutured from the aortic annulus to the inferior rim of the right coronary artery; and from the right atrial side the redundant sac of the SVA was resected and the defect was repaired with another patch. The patient was discharged uneventfully two weeks after surgery. |
On March 2012 we visited a 45-year-old Caucasian woman with a very extensive, untreated G1 cutaneous SCC infiltrating widely the lumbar region until bone. A physical examination showed a locally advanced lesions with necrotic and ulcerated areas involving the entire lumbar area until sacrum and buttocks. There was also a superinfection due to Pseudomonas Aeruginosa resistant to antibiotics. The patient referred functional limitation in movement, difficulty in walking, pain, and consequent serious relationship problems with a worse status of quality of life. The patient had G3 anemia due to the bleeding lesion, and performed red blood cells transfusion before to start the treatment. ECOG Performance Status was 2. A contrast-enhanced magnetic resonance (RM) of pelvis showed extensive skin thickening from the front region of the iliac spine up to posterior sacral region, involving the contralateral lumbar region. The lesion extended from the right of right iliac crest (where it is in contact with the lateral abdominal muscles and with the cortex of the iliac crest) until ipsilateral gluteal region, involving deep muscular tissue.\nThe patient’s case was discussed by a multidisciplinary committee involving surgeons, radiotherapists and oncologists. The patient was treated with Cetuximab at the loading dose of 400 mg/m2, followed by weekly doses of 250 mg/m2,from 28 March 2012 to 13 March 2013. HT has been performed concomitant with the Cetuximab from 28 march 2012 to 19 May 2012, at the total dose of 60 Gy (2 Gy/fx).\nPatient was immobilized in the prone position without application of bolus. Planning computed tomography (CT) images were acquired through the region of interest using a 3 mm slice thickness and transferred to the contouring workstation for TomoTherapy Hi · Art System®. Set up was based on fiducial markers and tattoo aligned with a room laser system before treatment.\nThe gross target volume (GTV) was considered as right gluteal region outlined based on the depth of involvement and the extent of disease. Clinical target volume (CTV) was created adding a margin of 3 mm at GTV. Doses to organs at risk (rectum, bladder, right and left femur, right and left kidney, pubis, bowel) were defined in accordance with dose constraints. Each therapy session was preceded by a MVCT in order to ensure the correct repositioning of the patient and to adjust the distribution of the irradiation dose according with the evolution of the tumor during therapy. The accepted tolerance at our institution was 5 mm. After a new CT scan for contouring, a new treatment plan was elaborated at 30 Gy according with volumetric reduction of lesion. The time of delivery (around 10 minutes for fractions) were well tolerated by the patients. At the end of the combined radio target therapy, Cetuximab treatment continued for the following 10 months.The patient achieved complete response, confirmed at imaging evaluation, and the lesion reduced until disappear even after the suspension of the treatment (Figures and ). Toxicity resulted in G1 cutaneous rash and G2 toxicity to the nails according to typical toxicity profile of the anti-EGFR therapies, appeared 5 months after treatment. One month after therapy the Pseudomonas Aeruginosa superinfection totally disappeared as the G3 anemia.\nQuality of life resulted significantly, dramatically improved with progressive reduction until discontinuation of anti-pain drugs. At follow up of 15 months there was no evidence of active disease, moreover she was able to get back a normal social life. Actually the ECOG Performance Status is 0 (zero). |
A 46-year-old male came to the emergency room (ER) complaining of double vision and difficulty walking. The double vision started five days prior, followed by weakness in both the legs leading to a wobbly gait. The patient was evaluated by the neurology team in the ER. The patient had a mild upper respiratory infection about ten days before the symptoms started. He denied any diarrhea or tick bite. He had no urinary/bowel incontinence. His past medical history was significant for asthma that was well controlled by albuterol inhaler as needed. He had no surgeries in the past. The family history was non-contributory. He denied smoking, alcohol use, or drug abuse. He took no medication except the albuterol inhaler as needed. He had no occupational exposure to neurotoxins.\nOn physical examination, his vital signs were within the normal range. He was fully alert and oriented to time, place, and person with an intact memory. There was paralysis of left eye abduction on cranial nerve examination. Both pupils were round, equal, and slowly reactive to light. No facial muscle asymmetry was noticed, with no evidence of seventh cranial nerve deficit. The muscle stretch reflexes were 1+ in all four extremities, and the plantar response was flexor. The Romberg test was negative. There was no focal sensory or motor deficit in the coordination of upper limbs. The heel-knee-shin test was mildly impaired with gait unsteadiness. The unsteady gait was demonstrated regardless of diplopia. An ophthalmology evaluation did not reveal the visual and retinal change. Next day, the patient complained of worsening of all symptoms and had total bilateral external ophthalmoplegia and sluggish pupillary light reflex. The muscle stretch reflexes became 0+ in the bilateral lower extremities and remained so until discharge.\nThe laboratory testing revealed normal complete blood count, comprehensive metabolic panel, thyroid stimulating hormone, and cardiac markers. The urine toxicology screens were negative for common substances of abuse. The serum alcohol level was normal. The electrocardiogram was normal. The erythrocyte sedimentation rate was 4 mm/hour. The serum anti-cholinesterase antibody test results were negative, which eventually refuted myasthenia gravis as a diagnosis. The chest radiograph showed no evidence of acute or chronic disease processes. The carotid Doppler did not show any sign of carotid stenosis. Computed tomography (CT) and magnetic resonance imaging (MRI) of the brain demonstrated no mass effect or evidence of an acute infarct. The serum glucose was 102 mg/dl, and the hemoglobin A1c was normal. The rapid plasma reagin for syphilis was negative. The serum antineutrophil cytoplasmic (ANCA) IgG titer was within normal reference range. The serum Lyme titer and Epstein-Barr virus (EBV) antibody titers were within the normal reference range. Aldolase was 8.2 units/liter (within normal range). A lumbar puncture was done, and the cerebrospinal fluid (CSF) analysis showed protein 43 mg/dl, glucose 66 mg/dl, white blood Cells 0/ul, red blood cells 0/ul, the presence of oligoclonal bands and negative gram stain and cultures. The electromyography (EMG) was normal. The test for anti-GQ1b antibodies was not done as the clinical presentation was classical for the diagnosis.\nThe patient was given steroids in the ER and subsequently treated with intravenous immune globulin (IVIG), and significant clinical improvement was noticed. Physical and speech therapy specialists worked with the patient on a daily basis. He was able to ambulate with some assistance. He was discharged on the 10th day of hospitalization to a subacute rehabilitation facility. |
The patient was 50 years old (1987) when he suffered from a 4 cm left renal mass with no evidence of metastasis for which left radical nephrectomy was performed. Histopathology revealed clear-cell renal cell carcinoma (cRCC), grade II, stage pT1a, with negative surgical margins. The patient was then followed up regularly.\nIn 1995, the patient had a malignant melanoma excised from the left arm. He also underwent Whipple operation in 1996 for a neuroendocrine tumor of the pancreatic head that recurred in February 2000 and partial resection of the pancreas with intraoperative irradiation of surgical bed was done.\nIn October 2000, lower cuts of the contrast-enhanced chest CT revealed a small 8 mm basolateral left pulmonary nodule and metastasectomy was performed. Histopathology revealed the first reported recurrence of the cRCC 13 years after the initial diagnosis with negative surgical margins.\nIn November 2001, the patient underwent right lobar thyroidectomy for asymmetrical thyroid enlargement revealing metastasis of the cRCC with negative surgical margins. Afterwards the patient started interferon-alpha (INF-α), interleukin-2 (IL-2), and 5-fluoruracil for 8 weeks as first- line adjuvant systemic therapy for mRCC.\nIn December 2003, brain CT for severe headache was performed, revealing a 3 × 2 cm focal metastasis in the brain, likely of cRCC type, with temporal lobe hemorrhage. The patient survived the incident and received palliative radiotherapy on this lesion in 2003, 2004, and 2005 (see ).\nIn November 2007, 20 years after the first renal tumor therapy, a 5 cm local tumor recurrence appeared at the left retroperitoneum in the contrast-enhanced CT scan. The cancer recurrence was surgically excised. Recurrence was also observed in the remaining part of the thyroid gland (4 cm), which was again excised and revealed cRCC. Therefore, a second line of systemic therapy with sunitinib (Sutent 50 mg once daily, 4 weeks on and 2 weeks off) was started. The patient received 30 cycles of sunitinib until September 2011.\nIn September 2011, a suspicious 3 cm hepatic focal lesion appeared in the regular follow-up which was operated on through laparotomy and excision of segment IVa, revealing cRCC, GII. Therefore, the regimen was shifted in November 2011 to a third line of systemic therapy, 10 cycles of temsirolimus (Torisel 25 mg, Pfizer; i.v. injection over 30–60 minutes) till follow-up CT in January 2012 revealed small subpleural focal lesion (2 mm) and a posterobasal lesion in the lower left lung lobe (1 cm). The regimen was shifted to a fourth line of systemic therapy with oral sorafenib twice daily (Nexavar 400 mg, Bayer).\nIn October 2012, the follow-up CT showed progressive enlargement of the pulmonary metastasis up to 4 cm and appearance of a new focus at the apical part of the lower right lung lobe and five metastatic foci in the liver. The institutional tumor board decided to start with the fifth line of therapy, bevacizumab (Avastin, Genentech Roche) 10 mg/kg i.v. injection every 2 weeks and INF-α 3 μIU subcutaneous injection three times/week since January 2013. The patient received 18 consecutive cycles of this therapy.\nIn November 2013, two osteolytic bone lesions appeared in two right ribs and the lung metastases progressed. The local tumor board decided radiotherapy of the osteolytic lesions and to start the sixth line therapy with axitinib (Inlyta, P fizer; 5 mg twice a day). Due to intolerable side effects (fatigue and diarrhea), the patient soon stopped the medication on his own.\nIn December 2013, a seventh line therapy was initialized with everolimus (Afinitor, Novartis; 10 mg once daily). Because of progressive enlargement of the lung, liver, and right hilar LN metastases, treatment shifted in June 2014 to eighth line of systemic therapy for mRCC, pazopanib (Vortrient, GSK, 400 mg oral tablets twice daily).\nCurrently, the patient is still coming for regular follow-up checks to our clinic, getting the pazopanib (his 8th line of systemic treatment) as scheduled up to the day. However, the patient is suffering from multiple metastatic symptoms in liver, ribs, and lung as previously described (see ). |
A 23-year-old unmarried Omani woman was brought to the psychiatric clinic by her parents, with a 3 year history of behavioural and mood changes. Her mother had observed that these cyclical behavioural changes occurred during certain times of the patient’s menstrual cycles. Three days prior to menses, the patient became depressed, isolated herself and cried for no obvious reason. Shortly after her menstrual bleeding, she became excited and moved in back and forth pattern. She appeared awake and aware but emotionally detached from surrounding with incomprehensive speech. She became suspicious of her mother and had attacked her on several occasions. She would spend the night looking out of the window, believing that her persecutors will appear at any moment. She was observed to mutter to herself excessively and would make gestures as if protecting herself against invaders. Her condition progressed over time and on day 3 of her menstrual cycle, she would become mute, look perplexed and fearful and would kneel (in a Muslim prayer manner) for a long time.\nHer symptoms would begin to improve on day 5 of the menstrual cycle and would almost always be completely resolved by day 8.\nDuring interview, the patient was in the fifteenth day of her menstrual cycle, and could not recall most of her symptoms, as described by her parents. However, she reported having ‘strange feelings’ prior to her menstrual bleeding and ‘weird experiences’ following. She remembered hearing an old woman talking to her and seeing lines of blood on the walls of her room with intact orientation and awareness throughout her presentation as mentioned by the patient and her family.\nHer symptoms would almost always start 3 days prior to menstrual bleeding and resolve completely by day 8 of the menstrual cycle. During the interval between these episodes, the patient was reported to be asymptomatic, interactive and socially active. Her first menstrual cycle had occurred at the age of 14 and experienced regular cycles.\nShe had no relevant medical history and had no family history of mental health disorders. She had never smoked tobacco, drank alcohol or used drugs.\nA physical examination was unremarkable and her medical workup, including a hormonal study, brain computed tomography (CT) scan and electroencephalogram were unremarkable for any underlying medical disorders. She was prescribed olanzapine 5 mg once daily and was offered an appointment in the clinic during her next cycle. Her symptoms had slightly improved and had disappeared on day 5 of the menstrual cycle.\nHowever, she was still reported to have episodes of screaming and mood lability on the first day of menstrual bleeding. The olanzapine prescription was increased to 10 mg once daily and the patient is still undergoing psychiatric follow-up. |
Patient 3: A 24-year-old gravida 6 para 4 female at 31 weeks of gestation presented to the emergency department as a belted passenger in a 45-mph motor vehicle collision. Her pregnancy had been complicated by anemia and obesity. Plain film radiography demonstrated a mildly comminuted and displaced right femoral shaft fracture with foreshortening (). Her obstetric history included four previous full-term spontaneous vaginal deliveries and one elective abortion. She had no other significant past medical history. Laboratory evaluation was remarkable for a white blood cell count of 20.7×103/μL and hemoglobin of 9.1 g/dL, with a normal calcium level of 8.6 mg/dL. She was transferred to labor and delivery where fetal monitoring was reassuring. Uterine tocometry showed contractions every 2–10 mins and the decision was made to give a 12 mg dose of intramuscular betamethasone for fetal lung maturity prior to surgical intervention.\nOn hospital day 1, the patient underwent an uncomplicated anterograde rod insertion of her right femur under general endotracheal anesthesia. Fetal monitoring before and during surgery was reassuring. Total preoperative and intraoperative radiation dosage was 135.2 mGy and a lead apron was used to shield the maternal abdomen during all images. The patient was started on 30 mg twice daily enoxaparin sodium for VTE prophylaxis, physical therapy as well as oral cholecalciferol and calcium acetate for daily supplementation. Throughout her postoperative course, the patient was not meeting ambulation goals necessary for discharge and on postoperative day 5, she was diagnosed with preterm premature rupture of membranes. Azithromycin and amoxicillin were started as latency antibiotics. On postoperative day 7, the patient complained of increasing pain and pressure, the cervix was found to be fully dilated, and she vaginally delivered a live born male fetus at 32 weeks and 1-day gestation from the breech presentation. The patient had an unremarkable postpartum course and after ambulating with a walker was discharged home on postpartum day 2. |
A 41-year-old female patient presented with a sudden onset of paralysis and numbness of the left limbs 10 days ago. The muscle strength of the left upper and lower limbs was grade I and grade III, respectively. Hypotonia, ataxia, and decreasing of superficial sensibility of the left limbs were also detected. The Babinski sign was positive on the left side. Preoperative CT/MRI showed hematoma with mixed density located at the right frontal lobe on the corona radiata level (). The preliminary diagnosis was CCM bleeding. Preoperative DTI and 3DT1 showed that the lesion was just located under the central sulcus and involving the CST (). The mean FA value of the ipsilateral CST was 0.45, and the fiber number was 131. Considering the mass effect of hematoma, the motor and sensory function deficits, the risk of rebleeding from CCM, and the timing of resection, surgical treatment was recommended. The key points of surgery were as follows: (1) there is a need to protect the cortical and subcortical tissues to reduce surgical complications, (2) small lesion needs accurate positioning, and (3) radical resection is needed to avoid rebleeding. After multidisciplinary discussion, endoscopic surgery with intraoperative neuronavigation, and electrophysiological monitoring was scheduled. A small area shaving and a 5-cm linear incision were performed (). The diameter of the bone flap was 4 cm (). Intraoperatively, after the lesion was located by navigation, we identified the central sulcus and dissected the arachnoid about 2 cm (). Then, the introducer and elliptic endoport were inserted under the guidance of the navigation pointer (). About 3.5 cm beneath the cortex, we saw the hemosiderin-stained surrounding parenchyma and hematoma (). The hematoma was evacuated and the lesion was dissected at the interface of the CCM and yellow-stained white matter. The CM resections were completed with special care to preserve the white matter tracts with the help of DTI navigation and intraoperative monitoring of evoked potentials (). After the endoport was removed, the cortex was intact without crush injury (). One week later, conventional MRI was performed to confirm radical obliteration. The postoperative course was uneventful. On discharge, the muscle strength of the left upper limb and left lower limb was grade II and grade IV, respectively. Hypotonia, ataxia, and numbness of the left limbs were relieved. The Babinski sign was still positive on the left side. The pathological examination confirmed the diagnosis of CCM and hematoma (). Three months later, DTI and 3DT1 examination were performed again to compare with preoperative ones. The mean FA value of the ipsilateral CST was 0.45, and the fiber number was 139. The muscle strength was recovered to grade IV of the left limbs at 6 months after surgery (). |
A 52-year-old woman was referred to our hospital because of severe dyspnea and the presence of a neck tumor. She had experienced dyspnea for the past 6 months and had recently noticed bloody sputum. A computed tomography examination of the neck revealed a thyroid tumor about 8 cm in size. The tumor had invaded the trachea, obstructing 90% of the tracheal lumen (fig. ). An emergency tracheotomy was performed to maintain an airway through the tumor tissue. On biopsy, the tumor was diagnosed as CASTLE, suggesting a favorable prognosis if a complete resection could be performed. Magnetic resonance imaging and an esophageal fibroscopy examination revealed a low possibility of esophageal invasion (fig. ). Under general anesthesia, a thyroidectomy was initially performed via a neck incision. The invaded portion of the trachea was sharply dissected. A partial sternotomy was performed at the level of the 2nd intercostal space to identify the distal portion of the intact trachea and to mobilize the inferior tracheal segment. The trachea was transversely opened to find the intact portion. The length of the resected trachea was 6 cm (fig. ). Bilateral recurrent nerves were macroscopically preserved. We performed a suprahyoid release to mobilize the superior tracheal segment, but these mobilizations were insufficient to perform an anastomosis. Thus, to allow additional mobilization, we performed a clamshell thoracotomy and incised the pericardium 360° around the hilum, dissecting the pulmonary ligament bilaterally. These procedures allowed an anastomosis. An end-to-end bronchial anastomosis was carried out using running 4-0 PDS II sutures (Ethicon, Somerville, N.J., USA) for the membranous portion and interrupted 4-0 PDS II sutures for the cartilaginous portion. The bronchial anastomosis was wrapped with an omental pedicle flap that was lifted up through a retrosternal route via a laparotomy. A gastrostomy was also performed for postoperative enteral nutrition. For management after surgery, a chin suture was placed. Neck mobilization was restricted for the placement of the chin suture and airway fixation. After confirming an improvement in the laryngeal edema using a laryngoscopy, extubation was performed on postoperative day (POD) 17. The patient exhibited swallowing disturbances and underwent swallowing rehabilitation. Enteral nutrition was provided through the gastrostomy. The patient was discharged on POD 31, and per os feeding was started on POD 51. Radiotherapy (60 Gy in 40 daily fractions of 1.5 Gy) was performed as an adjuvant therapy. Throughout the clinical course, no major complications, including swallowing disturbances, hoarseness or anastomotic problems, were observed. A histopathological examination of the resected specimen revealed the characteristic morphology of CASTLE (fig. ); immunohistochemistry was positive for CD5 (fig. ) but negative for thyroid transcription factor 1 (TTF-1; fig. ).\nEleven months after surgery, lower back pain and right leg numbness developed and led to gait inability. Multiple lung and bone recurrences were observed, but no local recurrence. In order to ease the back pain, palliative radiotherapy (35 Gy in 14 fractions) to the bone metastasis was administered. Twelve months after surgery, drainage of pleural effusion was performed. She died of pleural metastasis 14 months after the initial diagnosis of CASTLE. |
A 26-yr-old man, with no other medical history, complained of progressive headache and vomiting for 3 months. A neurological and physical examination revealed no abnormal findings. Laboratory findings including karyotyping were normal.\nComputed tomographic (CT) scans demonstrated only asymmetry of the lateral ventricles, with enlargement of the right lateral ventricle (). Subsequent magnetic resonance imaging revealed a large, cystic, nonenhancing lesion with a thin wall in the right lateral ventricle. The cyst contents had the same signal intensity as cerebrospinal fluid (CSF) on MRI ().\nThe patient underwent endoscopic transventricular cyst resection. The patient was positioned supine with head in neutral position and the thorax elevated 15 degree. A linear incision on the right frontal scalp, parallel to the sagittal suture, was applied to the pericranium. A 15-mm burr hole was placed 3 cm from the midline and just behind the hair line. The dura was incised in cruciate fashion. A 12-French introducer sheath was inserted into the frontal horn of the right lateral ventricle under freehand-guidance. A 2.7 mm rigid telescope was introduced, and the cyst wall obstructing the right foramen of Monro was identified (). An avascular area in the midportion of the cyst wall above the foramen of Monro was chosen for fenestration. The cyst wall was punctured by gentle pushing the tip of the probe. With microscissors and forceps, the cyst wall was opened as widely as possible (). CSF in the cyst was expelled and the foramen of Monro was opened. The shrunken cyst was separated from the ventricle around the foramen of Monro. We drew the margin of opening with forcep carefully. The cyst was removed totally with no resistance (). At that time, subsequent intraventricular hemorrhage was identified, so continuous irrigation of lactated Ringer's solution was done because the bleeding focus could not be found. After identifying no active bleeding, the neuroendoscope was then withdrawn remaining the external ventricular drainage catheter. A small piece of Gelfoam was placed in the cortical chimney, and the wound was closed in a routine fashion.\nHistological examination revealed a fibrous vascular stroma covered by cuboidal epithelium. The appearance was that of choroid plexus neuroepithelial cells ().\nImmediately after surgery, he complained headache with no neurologic deficit. Intraventricular hemorrhage was detected on CT scan obtained at that time. External ventricular drainage catheter was changed due to catheter infection at 8 days after surgery and it was removed at 14 days after surgery. A CT scan performed 14 days after endoscopic removal of the cyst showing no intraventricular hemorrhage (). Infection was controlled soon. He was discharged home at 37 days after the first surgery with no neurologic deficit. His headache significantly improved on follow-up visits. |
A 58-year-old female with a history of lupus and plaque psoriasis presented to the emergency department with new onset hallucinations and suicidal ideation, as well as one witnessed attempt to throw herself into traffic. The patient was brought to the hospital after what her husband said were three days of the patient talking to people who were not there, crying frequently, not sleeping, openly expressing a desire to die, and one attempt to run from their home to a nearby highway in order to jump in front of a car. The patient's husband had chased after and restrained her, after which he brought her to the hospital.\nThe husband relayed that the patient had never before in the course of their 35-year marriage behaved in this manner, and he further insisted that the patient was previously generally “happy and smiling.” In-house hospital records showed a long history of lupus and psoriasis management as well as a recent admission two weeks prior for mastoiditis; she had received IV clindamycin and ceftriaxone and then been discharged on oral clindamycin. There was no record of any mental health history.\nThe medical records showed that, during the recent hospital admission, the patient was discovered to have a depressed thyroid stimulating hormone (TSH) level; she was diagnosed with hyperthyroidism and treated with methimazole (MMU) and propranolol for presumed Grave's disease. At the time of discharge, she was continued on a half dose of methimazole. Propranolol was discontinued before discharge based on clinical response to treatment, with endocrinology clinic follow-up scheduled for three weeks from the time of discharge.\nThe patient's husband further relayed that the patient had been searching their home for a knife for the past two days while telling him that she “needed to die,” compelling him to remove all kitchen knives from their home. On interview, the patient was asked why she was looking for a knife and she responded “because I want to die.”\nThe patient and her husband both denied any recent head trauma. They both further denied any alcohol use or illicit drug use. The patient denied any abdominal pain, diarrhea, dysuria, vomiting, or shortness of breath. She endorsed restlessness, palpitations, and a sensation of “feeling too hot.”\nOn exam, blood pressure was 145/88, temperature was 37.5, respirations were 17, oxygen saturation was 99 percent on room air, and heart rate was 129 beats per minute. On the cardiac monitor, she was in gross atrial fibrillation, which had previously not been documented in her medical record. During the exam, the patient was noted to have rapid, pressured speech. Voice volume was normal, and her mood was depressed. She became tearful during the interview, and she intermittently appeared to be responding to internal stimuli. Of note, she seemed to be quite open about all her symptoms and was an excellent historian. She freely admitted to suicidal ideation (SI) and audiovisual hallucinations (AVH). She admitted to attempting to run into traffic in order to kill herself. She described hearing what she described as supernatural voices speaking to her; she said that she could see “spirits and lions” that were likewise speaking to her. She did not believe that the voices were telling her to kill herself but rather said that she wanted to die “because I am so sad.” She was oriented to her name only. She did not know the year and believed she was currently in a prison. She asked the doctor for a knife several times. She also said that she felt as though she wanted to start running “because I can't keep my legs from moving.” She had pulled all the blankets off the bed, had removed much of her hospital gown, and asked if she could have some ice water “because it is so hot in here,” despite the fact that the emergency department was air-conditioned. The remainder of the exam was notable for an irregularly irregular heartbeat, clear lungs, tongue fasciculations, tremulousness, diffuse hyperreflexia, and a large, nontender thyroid gland.\nThe patient was placed on a medical detainment and assigned a one-to-one sitter to monitor her. Psychiatry was consulted. An electrocardiogram was ordered, which showed sinus tachycardia with resolution of the previously noted atrial fibrillation. Urine toxicology was negative. Labs showed a slightly low potassium level, in keeping with a hyperadrenergic state. TSH was undetectable.\nIt was well past midnight, so the decision was made to treat empirically for thyroid storm. Propranolol 80 milligrams PO and methimazole (MMU) 20 milligrams PO were ordered. The patient took the medication without any resistance. She was given a large cup of ice water, as well as an IV bolus of a liter of normal saline. Endocrinology was consulted, and the head of endocrinology began driving to the hospital from his home to see the patient. Dexamethasone 10 milligrams IV was then given. Meanwhile, the psychiatry service interviewed the patient. They agreed with the plan to treat for hyperthyroidism and placed the patient on a 72-hour psychiatric hold for danger to self and grave disability.\nTwo hours after treatment was initiated, the patient's heart rate had slowed to 89 beats per minute. The cardiac monitor showed normal sinus rhythm. Tongue fasciculations had stopped, though hyperreflexia remained. The patient said that she felt “so much better” and was at this point denying SI and AVH. She showed insight into her condition, saying “I was feeling very upset and scared. I was seeing lions, and they were talking to me. I was so scared. I could hear voices that I knew weren't there, but I felt so sad about how I felt that I wanted to die.” She wanted to know what medicine she had been given that had made her feel better, and she asked if she could have a prescription for it.\nEndocrinology consult arrived and assessed the patient, likewise finding hyperreflexia and an enlarged thyroid gland. Endocrinology made a diagnosis of thyroid storm in the setting of Grave's disease. Both consult services recommended a brain MRI to rule out an intracranial process. This showed no evidence of bleeding or stroke, nor any evidence of lupus cerebritis (which had been included on the differential due to the patient's lupus history).\nThe patient was admitted to medicine and was followed by psychiatry. The day after admission, she again began endorsing SI and AVH. Free T4 and T3 levels at this time were 2.10 ng/dL and 2.09 ng/dL, respectively. Psychiatry recommended olanzapine (Zyprexa) 5 milligrams daily, which was initiated to good effect. Propranolol, MMU, and steroids were continued and adjusted based on endocrine consult recommendations. Thyroid stimulating immunoglobulin (TSI), ordered as a send-out lab during the patient's previous admission for mastoiditis, returned positive, and the diagnosis of Grave's disease was made.\nAs the patient's thyroid labs normalized, her psychiatric symptoms resolved. Olanzapine was discontinued at the recommendation of psychiatry. Endocrinology recommended radioactive iodine ablation of her thyroid gland, which was scheduled and then aborted when she was found to have sudden onset pancytopenia, a well-documented side effect of methimazole []. Methimazole was discontinued, and her cell lines began to recover. After thorough reassessment, psychiatry lifted the hold, and the patient was discharged from the hospital on a steroid taper and propranolol. |
A 3.5 years old female child was brought to department of oral medicine and radiology with a complaint of mobility of right lower back tooth of 1 week duration. There was no history of toothache, trauma or associated symptoms. Extraoral examination revealed an irregular lobulated, nontender bony hard swelling of size 3 × 3.5 cm with uniformly blending borders on buccal aspect of right side body and angle of mandible (). Lower border of the mandible showed a discontinuity without considerable expansion. It extends to midline through submandibular region with a size of 4 × 5 cm. Local rise in temperature noted on overlying skin and is not fixed to swelling.\nIntraorally, right lower gingivobuccal sulcus and floor of mouth obliterated due to swelling from 84 region to retromolar region (). An irregular lobulated swelling of variable consistency with bilateral cortical plate expansion was seen on premolar-molar region. Hard tissue examination showed full complement of healthy teeth except grade III mobile nontender, 85.\nOcclusal and panoramic radiographs revealed ill-defined lytic lesions involving premolar-molar region of right side body of mandible, extending from 83 to angle of mandible. Cortex and periosteum was characterized by erosion, thinning and discontinuity on buccal cortex; radiating spicules on lingual cortex; irregular thinning of lower cortical plate seen without considerable expansion. Multiple irregular patchy radiolucent areas in internal structure of permeative and moth eaten pattern with size varying from 0.5 to 2 cm was noted in internal structure (). Considerable root resorption was seen on 84, 85. Other effects on surrounding structures include posterior superior displacement and mesial tipping of developed crown of dental follicles of 46, 47 and missing dental follicle of 45. Loss of alveolar canal outlines medial to angle of mandible (). Computed tomographic (CT) axial section showed multilocular expansile lytic lesion in body of mandible right side with significant enhancing soft tissue matrix ().\nTrucut needle biopsy showed spicules of bone with a cellular cytoplasm composed of round cells with scanty cytoplasm and pleomorphic round or oval nuclei in sheets and sinusoidal pattern (). Immunohistochemistry showed diffuse strong membrane positivity for MIC2 (), focal positivity for synaptophysin and negativity for desmin and diagnosis came compatible with ES or primitive neuroectodermal tumor (PNET).\nPatient underwent radiotherapy and adjuvant chemotherapy treatment with vincristine, cyclophosphamide, etoposide and mesna in Regional Cancer Centre, Thiruvananthapuram, Kerala. One year follow-up clinical review showed bony hard nontender diffuse expansion of right side mandible without apparent soft tissue swelling extraorally (). Intraorally missing 85 was noted due to exfoliation, 6 months after onset of treatment ().\nRadiological follow-up using occlusal and panoramic radiography after 1 year of treatment showed mixed radiodensity on premolar-molar region and anterior border of ramus of right side of mandible involving 82 to 47. Intact and visible uniform expansion with cortical regeneration was seen throughout the lower border of the mandible near the lesion. Cortical break in buccal aspect remodeled with overzealous cancellous bone apposition resulted in an increased buccolingual width. Periosteal reactions including vertical spiculations completely resolved and cortical out line re-established in lingual aspect (). Considerable reduction in permeative and moth eaten pattern and replacement with accentuated multiple linear and granular trabeculae with a multiseptated pattern was noted in internal structure. Enamel formation completed and dentine formation started 44 and 47. Relative developmental delay was observed on 46 (). Radiological features suggestive of cessation of malignant tissue growth and active destruction, followed by vigorous regeneration and reparative reactions of healthy osseous and dental tissues and arrested growth of dental follicle near epicenter of the lesion. |
A 78-year-old male patient presented to our outpatient clinic with a complaint of numbness and pain on the left site of the mandible. The patient stated that he had been suffering from this numbness for 1to 2 months. In the medical anamnesis, it was discovered that patient suffered from prostate CA 5 years previous, and since then he had visited his doctor periodically for an annual examination. In the examinations and on the basis of tests carried out at the hospital 1 year previous, it was stated that no CA relapse traces were detected. The patient had visited his dentist 2 months previous for pain and numbness of the left molar region. He stated that after root canal treatment, his dentist extracted the second left molar due to calcification of the canal and also due to pain and numbness. After extraction, the numbness and pain of the patient worsened; two weeks later, a panoramic radiograph was taken by his dentist, who did not perform any treatment other than to advise follow-up (Figure ).The patient presented to our clinic one and a half months later for further evaluation of his condition. Initially, a panoramic radiograph was taken. A moth-eaten shaped radiolucence was observed in the posterior mandible extending along the temporomandibular joint area. No pathologic findings were detected in other areas (Figure ). After this initial appearance, in order to examine the lesion in detail, a decision was made to perform cone beam computed tomography (Newtom 3G, CBCT, QR Verona, Italy) with three-dimensional (3D) reconstruction to obtain a more precise location and definition of the pathologic features. In the 0.4 mm CBCT sections, a moth-eaten shaped radiolucent lesion extending from the molar region was observed. The lesion was also involved in the mandibular canal at the level of the lingula mandibula (Figures and ). Panoramic reconstructions and 3D CBCT images showed severe moth-eaten shaped erosion in relation to the mandibular nerve (Figure ).\nOn consideration of the patient anamnesis and radiographs, a biopsy was planned for definitive diagnosis. The specimen showed that the bone marrow was filled with prostate cancer cells. The expression of prostate-specific antigen (PSA), cytokeratins (CKs) 7 and 20 was assessed by immunohistochemistry. The specimen showed a heterogeneous expression of PSA. CK stains of the specimen demonstrated a marker status of CK7 (-), and CK20 (-). Moreover, P504S/α-methylacyl coenzyme A racemase (AMACR) was used to as positive tissue marker to confirm the final diagnosis. A prostate carcinoma with metastasis was detected. The patient was sent for medical consultation. In the blood tests that were performed, gamma glutamine transferase was found to be over 295 iu/l, cea 817 ng/ml, and ca-15 600 U/ml. As a result, the patient was diagnosed as having recurrent prostate carcinoma, along with metastatic focuses that were detected at the femoral head, vertebra and shoulder head. No operation was planned because of severe metastasis of the CA throughout the body. No further follow-up was achieved. The patient died after 6 months due to the severe metastasis throughout his body. |
A 20-year-old Japanese woman visited our institute for treating her scoliosis. She was a second-born child among dichorionic diamniotic twins. She had visited a pediatrics division at another hospital because she experienced restriction in the range of the motion in both elbow joints, and clubfoot. When the patient was 3 years old, she was diagnosed with focal segmental glomerulosclerosis due to proteinuria. At the age of 7 years, she was diagnosed with MCTO based on multiple osteolysis and a typical facial appearance associated with a small forehead and hypotelorism. At the age of 18 years, she underwent genetic testing; a MafB missense polymorphic mutation was identified, and a definitive diagnosis was established. Scoliosis was apparent since she was 12 years old, but she had not sought treatment until the age of 19. The patient weighed 38.6 kg and had a height of 155 cm. Medical examination showed a typical facial appearance associated with a small forehead and hypotelorism; shortening of the fingers of both hands fingers and both upper limbs were observed in addition to clubfoot. No café au lait spots or mental retardation were seen. On the other hand, the trunk showed evidence of an irregular waistline and a rib hump that obviously suggested scoliosis (Fig. ).\nDue to the remarkable deformation of upper limbs beyond the elbow joint, upper limb muscular strength could not be evaluated accurately. Further, due to the remarkable deformation of lower limbs beyond the ankle joint, lower limb muscular strength could not be evaluated accurately. Other muscle weakness was not present. There were no walking problems. No hypoesthesia, numbness, or dysuria was found. Hyperreflexia was observed in the upper and lower limbs, but no neurological deficit was observed. No laterality was found in the limbs and Babinski reflex was positive. Spirometry showed a decrease in forced vital capacity (FVC) by 67.4%. Although proteinuria was observed, no renal dysfunction or hypertension was present.\nX-ray images of the limbs showed remarkable osteolysis in joints of the hand, elbow, and ankle (Fig. ).\nThe major curve of scoliosis was 82° (MC, Th7-L2; Th11 apical vertebra), and the upper curve was 77° ay (UC, Th1-6; Th3 apical vertebra). In a recumbent-traction position, major curve was 54° and upper curve was 56°. The results of sagittal plane measurement were as follows. Sagittal vertical axis: 30 mm; pelvic tilt (PT), 17°; sacral slope (SS), 60°; lumbar lordosis (LL), 71°; and pelvic incidence (PI), 77°. In terms of sagittal plane alignment, the PI–LL angle was <10° and no mismatch was observed; thoracic kyphosis was decreased to 16° (Fig. ).\nComputerized tomography revealed a partially narrowed vertebral arch, with no findings suggesting osteolysis. Magnetic resonance images showed deviation of the spinal cord, while neither Chiari malformation nor spina bifida was observed (Fig. ). |
A 59-year old woman came to our hospital in May 2013 because of low grade, continuous pain in the left axilla, without fever, chest pain or dyspnea.\nNine years before presentation, dilated cardiomyopathy with normal epicardial coronary arteries was diagnosed. Due to worsening of this condition, a dual-chamber AICD was placed in 2007. In October 2009, due to malfunction of the ventricular lead, a second ventricular catheter was added, without removal of the previous one (). Eight months later, the patient developed swelling, pain and redness at AICD generator site and pocket infection was diagnosed. After an ineffective course of antibiotics and anti-inflammatory drugs, pocket revision was performed, with removal of infected subcutaneous tissue and generator exchange. No relapse of infection was observed during the next 18 months. In February 2012, because of progression to end-stage heart failure, the patient underwent orthotopic heart transplantation (OHT) with AICD removal. However, the proximal end of one ventricular lead was not completely removed, and was abandoned within the innominate vein and the AICD pocket (). The patient was then started on a standard immune suppressive therapy with cyclosporine A, everolimus and prednisone. Fifteen months later, the patient presented to us with left axillary pain. On clinical examination, she showed a tender, fluid collection in the left axilla that was drained by US-guided percutaneous tap. Fluid culture grew methicillin-resistant Staphyloccoccus epidermidis and cytology and chemistry were consistent with a purulent material. Antibiotic therapy with cotrimoxazole was started and a transesophageal echocardiography performed. No vegetations were seen on the intravascular portion of the retained lead fragment within the superior vena cava as well as the right heart chambers. Plasma d-dimer levels were normal, as were C-reactive protein levels and white blood cell count. One week later, transvenous lead extraction was performed for adequate infection source control. After having carefully freed the catheter, telescopic sheats were used to dissect the initial intravascular portion of the retained lead. Subsequently, lead dissection was completed using an Evolution mechanical dilator sheat. No locking stylet was used. There were no complications. Lead culture grew the same Staphylococcus species previously isolated from fluid culture. No recurrence of pocket swelling was observed during the following two years and the patient remains well at present. |
A 34-year-old woman with no significant past medical history presented with the chief complaint of left facial numbness, left ear pain and decreased hearing in the left ear of three years duration. The patient had also been experiencing sharp and shooting pain in different areas of her left thigh. The pain was not associated with any weakness, tingling or numbness. Besides mild fatigue she denied having any fevers, night sweats or weight loss. Neurological physical examination was grossly intact except for sensory loss in the V2 (maxillary) and V3 (mandibular) distribution of trigeminal nerve (cranial nerve V). Abdominal exam was without evidence of lymphadenopathy and hepatosplenomegaly. Given deficits in the sensory distribution of trigeminal nerve, she was initially evaluated by ear, nose and throat (ENT) and underwent two sequential minimally invasive surgeries for nasal polyps without significant resolution of her symptoms. She was subsequently referred to a neurologist and had magnetic resonance imaging (MRI) of the brain performed. Brain MRI revealed a soft tissue mass with expansion in the left Meckel’s cave, measuring 22 x 16 x 12 mm (Figure ), raising concerns for a trigeminal schwannoma. She was evaluated by the neurosurgery and underwent an orbital zygomatic craniotomy and left trigeminal schwannoma resection. Pathology revealed deposition of abundant hypocellular eosinophilic material on light microscopy examination (Figure ). Congo red staining demonstrated characteristic 'apple-green birefringence' upon polarization (Figure ), consistent with diagnosis of trigeminal nerve amyloidoma. Unfortunately, no immune fluorescence or electron microscopy was done on the pathology specimen to determine the amyloid subtype. Postoperatively, the patient was referred to oncology to rule out systemic amyloid deposition. Basic workup including complete blood count was unremarkable except for hemoglobin of 12 g/dL with mean corpuscular volume (MCV) of 76 fL per cell. White blood cell and platelet counts were within normal limits. Comprehensive metabolic panel did not reveal any liver or renal abnormalities. Coagulation profile including prothrombin time (PT), activated partial thromboplastin time (APTT), international normalised ratio (INR) was normal. The patient had both serum and urine protein electrophoresis with immunofixation done which did not reveal any monoclonal protein. Serum free light chains were normal with kappa free light chain 16 mg/dL, lambda free light chain 12 mg/dL and kappa lambda free light chain ratio of 1.36. Urinalysis was without evidence of hematuria or proteinuria. Electrocardiogram (EKG) revealed normal sinus rhythm. Two-dimensional (2D) echocardiogram revealed ejection fraction 60% and normal ventricular wall thickness. Abdominal fat pad biopsy as well as a bone marrow biopsy was performed and both were without evidence of amyloidosis or other plasma cell dyscrasia or lymphoproliferative disorder. The patient was not offered any more localized and systemic therapy with follow-up brain MRI three months after surgical resection without evidence of recurrent amyloidoma. |
A 69-year-old Saudi man with diabetes mellitus was admitted to the Neurology Department with an unremitting headache lasting 5 days, episodic confusion, and visual disturbances. According to his family, the headache started gradually over the left side of his head and then became holocephalic and moderate to severe in intensity. The patient reported feelings of nausea and 2 episodes of vomiting. Moreover, his family stated the patient was often seen “bumping” into surrounding objects while ambulating. The patient reported experiencing some visual disturbance during this period. The patient's family felt he appeared confused at times and was not responding to his surroundings. He had no clear history of seizure, according to the family, and his past medical history was unremarkable apart from diabetes. He had sustained a minor head trauma 3 years prior with no concussion; however, his scan was reported to have shown “scattered areas of bleeding” in his brain. He otherwise maintained a healthy life and never required a hospital visit for any medical issues. His family reported the patient had normal cognitive function, especially as someone who ran his own business.\nThe patient was lethargic upon arrival to the accident and emergency department. Given the apparent risk of airway obstruction, the emergency physician intubated the patient. On general examination, we found no facial phakomas. While the patient was sedated, we found tonic eye deviation with nystagmoid-like eye movement and subtle myoclonic jerks of the distal limb suggestive of subclinical seizures. He was given an intravenous (IV) loading dose of phenytoin in addition to a midazolam infusion. Despite this, he sustained several clinical seizures in the subsequent days. Therefore, he required further titration of midazolam infusion (up to 14 mg/hr) and IV levetiracetam was added to optimize the antiepileptic coverage. His electroencephalogram was obtained postictal. His brain MRI was obtained 1 day following suspected subclinical seizures. Laboratory investigations showed peripehral blood cell count, haemoglobin, renal and liver function within reference range, and his blood glucose was elevated (14.7 mmol/L/264mg/dl).\nMoreover, the results of his thyroid function test and his parathyroid hormone and serum vitamin B12 levels were normal. His serum folate level was not available. The screening tests for Hepatitis B antigen, Hepatitis C virus, and human immunodeficiency virus antigens and antibodies were all negative. His anti-TTG immunoglobulin A (IgA) titre was high at 35 U (reference range is up to 20 U), and the screen for antiendomysial antibody was negative, and his anti-Gliadin IgA antibody results were within reference range. Cerebrospinal fluid (CSF) study showed a WBC of 1, red blood cell count of 1, protein level of 36mg/dL, and a glucose level of 7.6mmol/L. The results of the CSF tuberculosis and herpes simplex virus 1 and 2 polymerase chain reaction were negative. We performed a duodenal endoscopic biopsy, but the specimen was not prepared properly; therefore, histopathological examination was suboptimal. We did note, however, increased intraepithelial lymphocytes with normal villous architecture.\nThe computed tomography (CT) of the patient's brain showed bilateral scattered corticosubcortical parietooccipitotemporal calcification with no oedema or mass effect (). The brain MRI with and without contrast showed diffusion-weighted imaging (DWI) restriction over bilateral occipital cortex (more so over the left side) in a gyriform pattern () with concordant area of apparent diffusion coefficient (ADC) hypointensity (). Susceptibility weighted magnetic resonance sequences (susceptibility weighted imaging [SWI], susceptibility weighted angiography [SWAN]) demonstrated hyperintensity corresponding to the area of DWI restriction (). T1-weighted imaging with contrast showed no contrast uptake (), and we saw no oedema or mass effect. We found no cortical atrophy or any deep cerebral vein enlargement. A second brain CT after 1 month () showed no interval change compared to the initial CT and no evidence of residual changes observed in MRI (DWI, ADC, and SWI).\nBilateral cortical calcification has a distinct set of mimickers, and Sturge–Weber syndrome (SWS) is first among the possible differentials. Our patient's brain CT may indeed look identical to SWS. However, it is the constellation of clinical and radiological features that differentiate the two conditions. The important clinically distinguishing feature of SWS is our patient's normal cognition. Psychomotor retardation is seen in 50% of SWS cases [], and our patient lacked facial nevus which is prevalent in most SWS cases (except SWS Roach classification type 2) []. Our patient also had no ocular disease, a presentation found in 77% of SWS cases. Radiologically, SWS has tram line or gyriform-only cortical calcification (and not subcortical, as in our patient). Bilateral calcification (as seen in our patient) occurs in only 25% of SWS patients []. Other radiological findings of SWS not seen in our patient are ipsilateral choroid plexus hypertrophy [], enlarged transcortical (medullary) veins [], ipsilateral cortical atrophy [], enlargement of the ipsilateral ventricle, loss of volume of the ipsilateral cranial cavity [], and pial enhancing angiomatous malformation (usually ipsilateral to the facial angioma). Therefore, many classic SWS features were not identified in our patient, distinguishing his condition from SWS.\nOther conditions known to cause cortical calcification in a similar pattern are, namely, congenital folate malabsorption or the adverse effects associated with methotrexate and antifolate agents. There was no mention of the use of either of these agents. Congenital folate malabsorption would have an associated life-long history of symptoms of malabsorption [] which is not apparent in our patient. Cortical laminar necrosis could account for such radiological presentation. However, this entity is conventionally thought to be a sequela of a remote traumatic brain injury, anoxic-ischaemic injury, or a metabolic insult that is not present in this patient's past history. Moreover, characteristically, cortical laminar necrosis has T1-weighted gyriform hyperintensity in MRI which was not noted in this patient. Although each of these conditions gives rise to calcified shadows in brain imaging, none are anti-TTG IgA-positive which is specific for the diagnosis of CD.\nAntiepileptic treatment with phenytoin and levetiracetam was commenced immediately upon the realisation that the patient has seizures. With the diagnosis of probable CEC, he was started on a strict gluten-free diet.\nThe patient recovered remarkably and regularly submits to follow-up examinations at our neurology clinic. At his last visit (9 months from his presentation), we found no residual neurological deficit other than mild homonymous hemianopia. Currently, he is fully active, self-sufficient, and has not sustained any seizure for the past 9 months. |
We present a case report of a 45-year-old female operated for parotid tumor presenting with features suspected of malignant hyperthermia. On preanesthetic evaluation, patient had no comorbid condition was not on any drugs had no previous anesthetic exposure. She was suspected to have difficult airway in view of swelling due to tumor and mallampati Grade 3. On the day of surgery, venous access was established minimum basic monitoring was attached to the patient. Her blood pressure was 130/80 mmHg, heart rate 78 beats/min and oxygen saturation were 98% on the operation table on the day of surgery.\nAnesthesia was induced on propofol and suxamethonium and ventilated with oxygen and halothane. Laryngoscopy was difficult due to masseter rigidity attributed to suxamethonium; patient was intubated with size 7.5 ID cuffed polyvinyl endotrachial tube and connected to ventillator. The patient was given morphine for analgesia; anesthesia was maintained with 50% oxygen: 50% nitrous oxide and isoflurane with boluses of atracurium.\nTen minutes into surgery a rising trend in endtidal CO2 was noticed, initially, adjustment in minute ventilation, circuit check, sodalime replacement was done, but endtidal CO2 continued to rise with maximum rise of endtidal to 80 mmHg. There was associated rise in temperature with maximum reading of 104 F recorded by nasopharyngeal probe. The patient also had risen in heart rate and blood pressure with maximum reading of 150 beats/min and 180 mmHg, respectively. Suspecting malignant hyperthermia isoflurane was stopped propofol infusion was started, and patient was ventilated with 100% oxygen through a fresh banes circuit using higher gas flows and higher minute ventilation.\nSurgeon was informed and asked to expedite surgery. Active cooling was started with ice cold saline intravenously and irrigation through Ryles tube and bladder catheter. Ice packs and cold towels were used for surface cooling to control the rising temperature. Blood gas sample at this time showed the following result: pH-7.12, pCO2-96 mmHg, pO2-224 mmHg, base excess-6, HCO3-20 mEq/L, Na-142 mEq/L, and K-5.5 mEq/L suggestive mixed respiratory and metabolic acidosis. Patient's other tests done intraoperatively were creatinine kinase −1300 IU LDH-120 IU, blood urea-20 mg%, s. creatinine-1.2 mg%, blood sugar 138.9 mg%. Patient also showed features of rigidity in limbs along with mottling. Dantrolene drug of choice for malignant hyperthermia could not be used due nonavailability of the drug in our hospital.\nWith active cooling patients temperature stabilized and then started to drop toward normal. With high minute ventilation and higher flows end tidal and PaCO2 were also controlled and then started to drop. Surgery was completed within 2 h and patient was shifted to Intensive Care Unit for postoperatively management. Blood samples including thyroid function test and urine for myoglobin sent from Intensive Care Unit were within normal limits. The patient was extubated after 2 h of ventilation once endtidal CO2 temperature and acid-base status returned to normal and patient achieved criteria for extubation.\nPatient and her attendants were made aware of suspected diagnosis of malignant hyperthermia in her and risks of recurrence in the patient and other family members on future exposure to anesthesia. The episode was also mentioned in anesthesia record of the patient for future reference. |
The patient was a 61-year-old Caucasian male with a history of coronary artery disease and hyperlipidemia, who initially presented with right shoulder and arm pain, as well as numbness and tingling. He was very active, working as a tennis instructor without any neurological deficits. On exam, motor/sensation was fully intact throughout both upper and lower extremities, with no upper motor neuron signs. He was classified as American Spinal Injury Association class E, Nurik Grade 0, with a full mJOA score. Despite this, the patient had failed conservative measures such as over-the-counter anti-inflammatory medications, but had not received prior spinal injection nor surgical intervention. Advanced imaging was obtained. Magnetic resonance imaging (MRI) was notable for a cystic mass within the spinal canal on the right side posterior to the C6 vertebral body, causing spinal cord compression and right neural foraminal stenosis (, ). This was attributed to synovial facet cyst formation. Given failed conservative measures, patient preference, and clear evidence on imaging, surgical intervention was recommended. The surgical plan was for C6 laminectomy decompression, right-sided C6-7 foraminotomy, and surgical excision and biopsy of mass under general anesthesia with intraoperative neuromonitoring.\nThe C6-7 laminectomy and foraminotomy were performed and the cystic mass was visualized on the right side, compressing the dura. The mass was isolated and excised. Pathology revealed a synovial cyst. Motor and sensory evoked potentials remained at baseline with no changes throughout the entire procedure. Estimated blood loss was 100 cc. A subfascial drain was also placed which was removed on POD2. Output was 275 cc in the first 24 h, and then slowed to 40 cc over the second 24 h. Approximately 45 min after the procedure, he had full strength and sensation in the bilateral upper and lower extremities.\nTwo hours postoperatively; the patient complained that he was unable to move his arms or his legs after being sat up by nursing staff. He was found to have 0/5 strength throughout all motor groups in the bilateral lower extremities, dullness to sensation throughout the bilateral lower extremities, 5/5 deltoid strength bilaterally, 4/5 biceps strength bilaterally, and 3/5 triceps, wrist flexor/extensor, and interossei strength bilaterally. Patellar and Achilles reflexes were absent. The mean arterial pressure (MAP) before this episode was 91. Leading diagnosis at this time was ACS due to transiently decreased perfusion related to hypotension.\nTen minutes following onset of his weakness, he was found to have near full strength and sensation, with the exception of 4/5 triceps, wrist extensors, wrist flexors, and interossei. The MAP, at this time, was 101. After another 10 min, he again had recurrence of the neurological deficits. The MAP was measured at 93. This second episode resolved in approximately 10 additional min. BP readings were obtained per Recovery Unit protocol. In the Recovery Unit, these measures are obtained every 5–10 min. An urgent MRI was performed, which demonstrated expected post-surgical changes without evidence of cord signal change or compression and no compressive hematoma. In addition, there was a low likelihood of post-operative hematoma given the transient/intermittent symptoms described above ( , ).\nThe patient was admitted to the intensive care unit (ICU) for close monitoring of both his BP (2 min cycles) and neurologic exam (whenever MAP <95, otherwise every 1–2 h per ICU protocol). He was given one dose of steroids postoperatively (dexamethasone 10 mg IV) in response to his symptoms as reperfusion injury was suspected. MAPs were maintained in the high 90s without the need for pressure support (norepinephrine or alternative therapies). High dose steroid treatment was not utilized. Over the next 24 h, his neurologic exam stabilized at full strength and sensation, with no recurrence of neurologic deficits. He had complete resolution of his pre-operative symptoms with normal neurological function and returned to teaching tennis over 1 year after surgery. |
A 77-year-old Middle-Eastern female with a medical history of hypertension and hyperlipidemia presented to the emergency department (ED) from a day care facility apartment where 2 people at the facility have tested positive for COVID-19 but she did not have any direct contact with these individuals. About 5 days before admission the patient developed a fever with a temperature of 102°F at home, and went to her primary medical doctor who sent her to the ED. In the ED she was found to have bilateral opacities on chest X-ray and had continued intermittent fevers with generalized weakness, cough, lethargy, and dyspnea and was sent for testing for COVID-19 then transferred to our facility for further management. In our facility, her temperature was 101.7°F, blood pressure 148/76 mm Hg, heart rate of 99 beats per minute, respiratory rate of 18 per minute, and oxygen saturation of 93% on room air. Physical exam was significant for a dry cough and bilateral rales on auscultation of the lung fields bilaterally but was unremarkable otherwise. A chest X-ray (Fig. ) was performed showing bilateral opacities throughout the lung fields with predominance of the lower lung lobes she was admitted for possible pneumonia with isolation precautions for suspected COVID-19 and was started on oxygen via nasal cannula and on 1-gram ceftazidime intravenously every 8 hours and 500 mg azithromycin orally daily. CT scan of the chest (Fig. ) was performed showing bilateral ground glass appearance throughout the lung with predominance in the peripheral lower lobes. Respiratory viral panel was sent including a repeat COVID-19 test (Table ). All results came back negative however the patient's condition deteriorated 2 days after admission to our facility, and she became hypoxic to 85% oxygen saturation while on nasal cannula and remained spiking fevers up to 103.4°F. She was intubated and transferred to the intensive care unit (ICU) for further management and was switched to ceftriaxone 1 g intravenously daily and azithromycin 500 mg via orogastric tube daily and was started on hydroxychloroquine 400 mg loading dose followed by 200 mg twice daily for a 7-day course. She required 100% fraction of inspired oxygen (FiO2) and a positive end-expiratory pressure (PEEP) of 12 to maintain an oxygen saturation of >90%. 12 hours later, the COVID-19 test from the initial facility returned positive results. On day 3 of hospitalization she was started on 6 g of IV ascorbic acid twice daily and given one dose of 8 mg per kg (567 mg) of tocilizumab, an anti-interleukin-6 monoclonal antibody. Due to a shortage of vitamin C in the hospital, her dose was decreased to 1 g IV daily on the 6th day of hospitalization and she was given another dose of tocilizumab. On day 7, her PEEP increased from 12 to 16 due to worsening oxygen saturation and increased requirement despite 100% FiO2. Due to severe ARDS, the decision was made to prone the patient for 18 hours a day. She completed her course of antibiotics and hydroxychloroquine but remained on vitamin C and zinc. Approval for remdesivir was obtained from Gilead Sciences Inc and she was given a loading dose of 200 mg on day 10 and due to worsening oxygen saturation her PEEP was again increased to 18. On day 11, the patient was unable to tolerate being prone due to significant desaturation to 65% on pulse oximetry and remained supine. She eventually required levophed for maintenance of hemodynamic stability and her creatinine increased from her baseline of 0.5-0.6 since admission until day 10 to 2.65 on day 12. For this reason, remdesivir was discontinued and nephrology was consulted and recommended CVVHD on day 13. On day 14 her PEEP requirement again increased to 20 while on 100% FiO2 to maintain an oxygen saturation >90%. Her condition remained critical while being aggressively managed in the ICU and ultimately the patient's family decision was to pursue comfort measures and the patient passed away. |
A 34-year-old woman with no significant past medical history presented with the chief complaint of left facial numbness, left ear pain and decreased hearing in the left ear of three years duration. The patient had also been experiencing sharp and shooting pain in different areas of her left thigh. The pain was not associated with any weakness, tingling or numbness. Besides mild fatigue she denied having any fevers, night sweats or weight loss. Neurological physical examination was grossly intact except for sensory loss in the V2 (maxillary) and V3 (mandibular) distribution of trigeminal nerve (cranial nerve V). Abdominal exam was without evidence of lymphadenopathy and hepatosplenomegaly. Given deficits in the sensory distribution of trigeminal nerve, she was initially evaluated by ear, nose and throat (ENT) and underwent two sequential minimally invasive surgeries for nasal polyps without significant resolution of her symptoms. She was subsequently referred to a neurologist and had magnetic resonance imaging (MRI) of the brain performed. Brain MRI revealed a soft tissue mass with expansion in the left Meckel’s cave, measuring 22 x 16 x 12 mm (Figure ), raising concerns for a trigeminal schwannoma. She was evaluated by the neurosurgery and underwent an orbital zygomatic craniotomy and left trigeminal schwannoma resection. Pathology revealed deposition of abundant hypocellular eosinophilic material on light microscopy examination (Figure ). Congo red staining demonstrated characteristic 'apple-green birefringence' upon polarization (Figure ), consistent with diagnosis of trigeminal nerve amyloidoma. Unfortunately, no immune fluorescence or electron microscopy was done on the pathology specimen to determine the amyloid subtype. Postoperatively, the patient was referred to oncology to rule out systemic amyloid deposition. Basic workup including complete blood count was unremarkable except for hemoglobin of 12 g/dL with mean corpuscular volume (MCV) of 76 fL per cell. White blood cell and platelet counts were within normal limits. Comprehensive metabolic panel did not reveal any liver or renal abnormalities. Coagulation profile including prothrombin time (PT), activated partial thromboplastin time (APTT), international normalised ratio (INR) was normal. The patient had both serum and urine protein electrophoresis with immunofixation done which did not reveal any monoclonal protein. Serum free light chains were normal with kappa free light chain 16 mg/dL, lambda free light chain 12 mg/dL and kappa lambda free light chain ratio of 1.36. Urinalysis was without evidence of hematuria or proteinuria. Electrocardiogram (EKG) revealed normal sinus rhythm. Two-dimensional (2D) echocardiogram revealed ejection fraction 60% and normal ventricular wall thickness. Abdominal fat pad biopsy as well as a bone marrow biopsy was performed and both were without evidence of amyloidosis or other plasma cell dyscrasia or lymphoproliferative disorder. The patient was not offered any more localized and systemic therapy with follow-up brain MRI three months after surgical resection without evidence of recurrent amyloidoma. |
A 77- year old East Indian female presented with a one week history of fatigue, high-grade fever and right knee pain. On examination, she was diagnosed with pneumonia and placed on Zithromycin 250mg for five days. Three days later, the patient was rushed to ER and admitted to the hospital for worsening in fever, headaches and confusion. She had a previous history of pneumonia, gastroesophageal reflux disease, hypertension, hyperlipidemia, prolapsed uterus, urethral dilation, vaginal hysterectomy, right total knee replacement and bilateral cataract surgeries. Cerebrospinal fluid was obtained and was noted to have 30-70 white blood cells of which 92% were polymorphonuclear neutrophils (PMNs) with less than 1 glucose and 440 mg/dl of protein, and the gram stain revealed gram-positive cocci. CT scan of the brain was unremarkable. Patient had pneumococcal bacteremia and meningitis and was treated with Levaquin 750mg initial dose, 500mg IV q. 24 hours and Ceftriaxone 2g IV q. 12 hours.\nOn the second day of treatment, the patient decreased responsiveness and developed stroke with a systolic blood pressure of 179 mm Hg and a diastolic blood pressure of 69 mm Hg, pulse rate of 85 beats per minute, and core temperature of 99.8 °F. She was transferred to the intensive care unit. On the third day of admission, the patient complained of swelling and redness in the right eye. Ophthalmologic examination showed conjunctival injection, three corneal ulcers and periorbital cellulitis in the right eye. However, the right anterior chamber was well performed and no hypopyon was noted. Her right fundus was not visible ophthalmoscopic ally due to complete opacity. Visual acuity was light perception in the right eye and 25/20 in the left eye. Based upon the clinical examination, endogenous endophthalmitis was diagnosed, although MRI and CT scan of the brain did not indicate any endophthalmitis type of picture. The left eye was normal.\nIntravenous vancomycin treatment was initiated the following day. The consultation was performed because it was believed that the consultation could be used as a means to give the patient broad-spectrum antibiotic coverage in order to treat the patient's pneumococcal bacteremia and meningitis. Additionally, the patient was believed to have had bilateral cataract surgery and their last examination was 3-4 years ago. On day 18, her periorbital edema had nearly resolved. The patient’s condition continued improving with antibiotic drops and ointment. Due to a lack of ophthalmic equipment at the hospital, the patient was brought to the office for a further examination. B-scan ultrasonography of the posterior segment indicated vitreous opacity with no abscess or retinal detachment. Despite intraocular injection and antibiotics drops, the patient had permanent vision loss in the right eye. |
A 28-year-old Asian male patient was admitted to our hospital for a refractory migraine of 3 years. He complained hours of pulsatile pain with photophobia, haphalgesia of the scalp, and fatigue during each attack. His medical history was unremarkable, and his magnetic resonance imaging and computed tomography angiography revealed no imaging anomaly. Accordingly, he was treated with naproxen (0.5 g qd) and flunarizine hydrochloride (10 mg qd) with poor improvement. Thus, treatment with an ultrasound-guided stellate ganglion block on each side a time was planned as a complementary therapy.\nWith the patient's informed consent, the first block was successfully performed on the right side with temporary Horner syndrome, decreasing attacks on the first day. Thus, the next block for the other side was administrated the day after. During the practice, the patient laid in the supine position with a thin pillow under his shoulder to ensure the extension of the anterior neck. Before the procedure, the patient was instructed to raise his hand if he felt any discomfort but was not allowed to speak or swallow. He was monitored by pulse oximetry and noninvasive blood pressure. We adopted the in-plane approach because of the clear ultrasonography at the level of the 6th cervical vertebra (C6) transverse process and prepared 4 ml of 1% lidocaine with a 22-gauge needle and 12-MHz linear probe. During the procedure, the needle was supposed to be inserted between the carotid artery and thyroid toward the prevertebral fascia anterior to the longus colli muscle (Fig. ). However, we lost track of the tip when the inserting the needle, which passed the fascia of the artery and thyroid. Hence, the operator cautiously jiggled the needle to detect the tip. After the tip was confirmed by the concomitant jitter of the target tissue (the surface of the longus colli muscle), 0.5 ml of 1% lidocaine was injected; however, the injectate could not be clearly visualized, prompting the operator to slowly withdraw the needle and decide using the in-plane approach instead. The patient suddenly raised his hand and lost consciousness followed by approximately 2-minute tonic–clonic seizure. The patient was immediately ventilated manually with 100% oxygen by a facemask, and an intravenous line was set up followed by an injection of 2-mg midazolam. The patient quickly returned to calm and natural breathing state, and, after 10 minutes of somnolence, regained complete consciousness but could not recall the previous experience.\nLater, we used ultrasound to re-examine the patient, but found no hematoma. However, further examination revealed that a 6-mm-diameter artery at the C6 level was in the posteromedial of the carotid artery, which went completely unrecognized at the initial scanning, and could easily slide to the lateral side when the operator pressurizes the probe (Fig. ). Then, we traced cranially to determine the entry of the artery into the transverse foramen of the 5th cervical vertebra (C5) (Fig. ), which was preliminarily determined as a variant vertebral artery. The patient was discharged after 2-hour of observation and was followed up for 2 months. No complaints of dizziness, tinnitus, and dysphagia were reported. |
A 52-year-old woman (gravida 1, para 1, and abortion 1) was referred due to the pelvic mass which was found by the transvaginal ultrasound in private clinic. Transvaginal ultrasound demonstrated a large irregularly marginated solid mass in the pelvic cavity. She denied the abdominal bloating and the pain except for the vaginal discharge. Her appetite, bowel and urinary function were normal with no recent weight loss. The medical history was unremarkable; there was no history of the abdominal surgery, gynecologic disorders, and family history was not notable. On the physical examination, there was no palpable mass in the abdomen and the cervix appeared normal. Laboratory tests, including tumor markers (CA-125, CEA, and CA-19-9) were within the normal range. Pelvic computed tomography (CT) revealed a 12×9 cm2, lobulated and well-enhanced mass (). Expected origin was the left ovary and the patient was underwent laparoscopic surgery. On the laparoscopy, the uterus and both sides of adnexa were appeared normal. The tumor was not originated from ovary and located in the presacral retroperitoneal space. The tumor was bluish-colored, and adhered densely to the adjacent tissues. The surface of tumor was fragile to bleed easily during dissection (). Since the mass was large and fixed with adjacent tissue, the mass was removed with several pieces. The frozen section biopsy (FSB) was conducted with those pieces. The FSB test result revealed the stromal tumor which cannot be excluded the chance of malignancy. We decided to the laparotomy considering result of FSB. After midline incision, the whole abdomen, including appendix, liver, and diaphragm, were explored carefully and revealed no other abnormalities. Tumor was adhered to the sigmoid colon on the right side of the pelvis and to the iliac vessels and the ovary on the left side of the pelvis. During the sharp dissection around tumor, the left internal iliac vein was injured. It caused fatally massive hemorrhage. It was hard to identify exact site of the torn vessel which was caused by compressing the left common iliac vein and distal part of left internal iliac vein with two suction tips. Immediately, we called a vascular surgeon for help. The vascular surgeon repaired torn vessel. After the tumor resection, the patient was transferred to intensive care unit. Several hours later, rebleeding occurred. The patient was transferred to other tertiary hospital and underwent second surgery which was successful to repair the vessel injuries of external and internal iliac vein. Grossly, the maximum diameter of the tumor was 12 cm, and the appearance of the tumor was white and lobulated mass encapsulated with fibrous coating. Multifocal degenerative areas were found. Histologically, the tumor showed a richly vascular pattern consisting of large and small vessels lined by a single layer of flattened endothelial cells (). The tumor was composed of spindle cells arranged patternlessly next to dense collagen. The tumor cells had high cellularity, and it was mild to moderate cytologic atypia. Mitosis was 1/10 on the average in high power fields (HPF) as shown in . However, focal area showed increased mitotic rate (7/10 HPF). Immunohistochemically, these cells were positive for CD34 () but negative for pancytokeratin, smooth muscle actin, desmin, S-100 protein, CD31, factor VIII, c-kit, and HMB45. Finally, the tumor was diagnosed as a SFT with focally malignant feature. The postoperative course was uneventful. The patient had refused any adjuvant treatment and has been followed up for 3 years. There was no clinical evidence of disease recurrence. |
A 25-year-old male initially presented with a melanoma on the left scalp with cervical and inguinal adenopathy. After biopsy confirmed metastatic disease with BRAF V600 mutation, the treatment option was debated between targeted agents and immunotherapy. In the hope of a long-term remission, it was decided to treat with immunotherapy. As ipilimumab was the only approved agent at the time, this treatment was initiated and four cycles were completed. Two years later, the patient developed a new subcutaneous nodule on his back. The biopsy confirmed a subcutaneous metastasis and the positron emission tomography (PET) scan showed an 8 mm lesion at the surface of the spleen. Given the progression of his disease, he received a treatment of pembrolizumab at a dose of 2 mg/kg every 3 weeks, for a total of 15 cycles. The best response achieved on treatment was a partial response. At his 10th cycle of pembrolizumab, the patient presented subjective morning stiffness and edema in the legs and hands, as well as pain in both knees. The patient later experienced range of motion restriction, mainly preventing him from closing his hands. Cutaneous examination revealed erythema, edema as well as cutaneous sclerosis involving metacarpal joints and upward toward the wrist as well as distal legs up to the calves. Pitting edema was prominent on the lower legs; however, Stemmer’s sign was negative which entails pinching and tenting the dorsal tissues of the foot. There was a positive prayer sign. No groove sign was noted. No calcinosis nor telangiectasias.\nLaboratory tests revealed a progressive eosinophilia over 6 months, increasing from 700 cells/mm3 to 3400 cells/mm3. Skin biopsy of the second finger of the left hand and lateral malleolus of the left ankle revealed chronic subcutaneous inflammation, fibrosis of the dermis and infiltration of the fascia with mononucleated cells, confirming the diagnosis of eosinophilic fasciitis (shown in ). The use of pembrolizumab was discontinued at that time after a total of 15 cycles. Systemic steroids were offered but declined as spontaneous resolution was already underway with drug cessation. A complete resolution of symptoms and eosinophilia was reached within 4 months without corticosteroid treatment. Despite 45 weeks of exposure to pembrolizumab, the patient still managed to reach a complete remission of the immune-related adverse effect. A radiological follow-up with PET scans was undertaken after treatment discontinuation. The disease remained stable for 18 months. Then, the patient became symptomatic due to a rapidly evolving intraabdominal relapse and was started on targeted therapy with a combination of BRAF and MEK inhibitors. |
A 61-year-old female with poorly controlled diabetes mellitus with severe bilateral PDR presented to the emergency department at Upstate University Medical Center. Initially, the patient presented to an outside emergency room with conjunctival injection, copious purulent discharge and associated painful vision loss to no light perception (NLP) in the left eye. Patient was then transferred to Upstate University Medical Center for higher level care and was evaluated by the ophthalmology service 18 hours after symptom onset. She underwent lateral canthotomy and cantholysis at the outside hospital 6 hours prior to arrival, due to severe pain and concern for orbital compartment syndrome; however patient endorsed complete loss of vision 12 hours prior in her left eye. She had no ocular trauma or history of intravenous drug abuse. However, she noted to have a two-month history of intermittent left abdominal pain and diarrhea, along with an abdominal computed tomography (CT) reporting diverticulitis. On presentation, examination revealed an edematous, erythematous, and proptotic left eye with severely restricted extraocular movements (). The anterior segment exam revealed diffuse conjunctival injection, mucopurulent discharge from superotemporal globe (), diffuse corneal edema and haze, and extensive fibrin in the anterior chamber. The dilated exam was limited due to corneal edema, diffuse anterior chamber reaction, and dense vitritis. Ophthalmic ultrasonography revealed a subluxed lens with diffuse vitritis (). Orbital CT confirmed no retained intraocular foreign body () or occult penetrating injury.\nUpon admission, patient's blood and specimens from ocular mucopurulent discharge were sent for gram stain and culture. The gram stain revealed gram-positive bacillus. The patient was admitted and started on intravenous (IV) broad spectrum antimicrobial therapy (vancomycin, meropenem, and amphotericin B). Due to progressive clinical decline with associated leukocytosis and encephalopathy 48 hours after initiating systemic antimicrobial treatment and gram stain results, she underwent enucleation of the left eye. Postoperatively, the patient completed a full course of IV vancomycin and meropenem.\nReflex anaerobic culture from the mucopurulent collection grew Clostridium perfringens and gross specimen from the enucleation revealed numerous gram-positive bacillus microbes. Anaerobic microbes seen on staining and samples were inoculated onto prereduced anaerobically sterilized Brucella blood agar, phenylethyl alcohol blood agar, kanamycin-vancomycin laked blood agar, and Bacteroides bile esculin agar (Oxyrase, Inc., Mansfield, OH). The inoculated plates were incubated at 35 degrees C anaerobically using the AnaeroPack-Anaero Anaerobic Gas Generator (Mitsubishi Gas Chemical America, New York, NY). After 48 hours of incubation, 2+ growth of a gram-positive bacillus was noted on the Brucella blood agar. These colonies were identified as Clostridium perfringens by Matrix-Assisted Laser Desorption Ionization-Time of Flight Mass Spectrometry (Vitek MS, bioMerieux, Inc., Durham, NC). No other growth was observed.\nAmphotericin B was discontinued once fungal etiology was excluded. Subsequently, both the periorbital edema and erythema resolved. Additionally, the patient regained consciousness, as her encephalopathy and infection improved. She was subsequently transferred to local rehabilitation facility. A repeat CT abdomen demonstrated the previous inflammation had resolved, likely due to the aggressive inpatient broad spectrum antibiotics. No comorbid occult distal gastrointestinal malignancies were found. |
X. is a 9-year-old girl who suffers from MPS IIIA. She was diagnosed when she was 3 years old, at the same age when the symptoms started due to a gait anomaly. There are seven people in the household. She is the middle child, and her two siblings also suffer from MPS III-A. She has special educational arrangements at school and attends speech therapy sessions. According to the Barthel Index, she has a severe level of dependency. The most notable symptoms are motor clumsiness and reduced mobility. She had convulsions once and has poor circulation and intestinal disorders that result in poor sleep quality. She also has three crushed vertebrae and scoliosis. She was hospitalised when she was 5 years old to have her adenoids removed due to apneas at night and otitis. She takes natural enzymatic and vitamin supplements. She usually has intestinal parasites and receives treatment for deworming, and the mother mentions that once a month she goes to energy-based therapies like energetic kinesiology which focuses on the energetic processes in the body.\nThis section analyses the calculation of the variables observed in the eight sessions of X.’s case through the registration template. This was later converted into means and deviations, resulting in the findings shown in Fig. . As can be seen in the figure, a slight clinical improvement is obtained in all the post-evaluation carried out, including the physical, communication, social, emotional and cognitive variables.\nThe researcher’s qualitative observations relating to the physical, communicative, emotional and cognitive dimensions are described below. Before beginning the sessions, there was restlessness in X.’s legs and strong emotions, notably anguish and general restlessness. Restlessness in her legs diminished with music, and her level of relaxation increased. She communicated by repeating the sounds made by the MT, or creating her own singing, and playing the instrument played by the MT. At the gestural and body level, she frequently held the MT’s hand and maintained visual contact with him. The social and emotional aspects were also intensified, with positive or neutral emotions predominating. X. smiled at the MT and approached the people who were present, showing group integration. At the cognitive level, the most remarkable thing was her ability to pay attention when the MT played an instrument, and her creativity, as she created new harmonies by singing.\nX.’s mother considered the role of music in people's lives to be fundamental. She mentioned the difficulty in terms of being able to observe changes, and of assessing what remained after leaving the sessions, given the neurodegenerative disease suffered by her daughter. X. really likes music, and she sings and dances daily. This is a way of communicating with her that her mother has used since childhood. X.’s mother reported that, in general, X. was relaxed when she went home after the sessions. For example, there were days that she cried when she went to the session due to back pain. However, when she came out of the session, she felt more relaxed. Her mother stated that X. clearly enjoyed the sessions.\nThese variables were analysed by finding the means and standard deviations of X.'s diastolic blood pressure, systolic blood pressure and heart rate that were collected before and after the sessions. The results are shown in Fig. . |
A 69-year-old Caucasian male initially presented with melanoma in the left posterior auricular area in August 2010. Breslow’s thickness was 5.5 mm with ulceration and one of five sentinel lymph nodes in the left cervical basin were involved. A positron emission tomography–computed tomography (PET/CT) scan revealed no evidence of distant metastatic disease. Left neck dissection performed in October 2010 showed three additional lymph nodes were involved – the final pathologic stage was stage IIIC (T4bN3M0). He had significant comorbidities, including ESRD secondary to glomerulonephritis, for which he had been on hemodialysis since 2009. In the adjuvant setting, he received radiation to the left neck and was not considered fit for systemic intervention due to his comorbidities.\nHe remained without evidence of disease recurrence until September 2011, when a PET/CT scan showed several new metabolically active hepatic lesions, as well as small sub-centimeter lung nodules consistent with metastatic disease. An ultrasound-guided biopsy of one of the liver lesions confirmed metastatic melanoma. He was treated with temozolomide at a dose of 200 mg/m2 orally for 5 days on a 28-day cycle that he tolerated well. He was treated from September 2011 until repeat imaging in April 2012 showed clear progression of disease. His tumor was found to harbor the BRAF V600E mutation, and he was started on vemurafenib 720 mg orally twice daily, with weekly monitoring due to his ongoing hemodialysis. Restaging PET/CT scan in July 2012 showed a partial response to therapy with a decrease in size of some of the lesions, as well as a reduced standardized uptake value. The response was short-lived, with clear progression in October 2012, followed by discontinuation of vemurafenib ().\nHe was started on immunotherapy with ipilimumab and received four doses of ipilimumab at 3 mg/kg over 12 weeks. He was clinically noted to have a response in the dermal deposits as early as the second dose of treatment. He developed grade 1 pruritus after his second dose of ipilimumab that was managed with topical over-the-counter hydrocortisone ointment. Overall, his tolerance remained excellent, and he did not require any other intervention. Imaging obtained in January 2013 showed a significant response, which was confirmed at week 16. His last imaging studies obtained on May 16, 2013 showed ongoing response (). The patient continues to do well on hemodialysis with his Eastern Cooperative Oncology Group (ECOG) performance status preserved at 0. |
A 57-year-old female was brought to the emergency room by her daughter because the patient was exhibiting signs and symptoms of paranoia and frank persecutory delusions for the past year. She believed that her next door neighbor was watching her and listening to all of her conversations. She also believed that her neighbor had wired the house and that she was being monitored. She had a past history of paranoid personality but has been functional and socially active and had worked for an insurance company as a senior adjuster for about 30 years until 2 years earlier when she was laid off. She is divorced and has two grown children. She had no previous psychiatric admission. Her past medical history was significant for hypertension which was well controlled. Physical examination finding showed blood pressure 135/82 mm Hg, respiration rate 20/min, pulse 85/min, oxygen saturation on room air 100%, and temperature 99.4 °F. She was neatly dressed but was maloderous. On mental status examination, she scored 14 on the Montreal cognitive assessment test. Memory and mini-mental status exam was unremarkable. Other than speech fluency that was reduced, the rest of her physical examination was unremarkable. A complete blood count and complete metabolic profile were within normal limits. Vitamin B12 and thyroid stimulating hormone levels were within normal limits. A computerized tomography (CT) scan of the brain was unremarkable. A diagnosis of paranoid and delusional disorder non-otherwise specified was made, and she was started on olanzapine 2.5 mg daily and was considered for discharge home. The daughter at this time further expressed concerns for safety as she said the patient sometimes forget to pay her utility bills and that her house might be without electricity. At this point, with the new information of the patient’s inability to care for herself, a diagnosis of FTD was considered, taking into account her age and the time frame of her symptoms. The patient was admitted to the hospital, and a subsequent magnetic resonance imaging (MRI) of the brain was obtained which showed atrophy of frontal and temporal lobes bilaterally, left more prominent than right, which confirmed the diagnosis of FTD (, ). The patient was appropriately treated thereafter with olanzapine 10 mg daily and 5 mg as needed for agitation, and donepezil 5 mg at night as well. She improved symptomatically and was later discharged home to her family in stable condition with appropriate follow-up. |
A 53-year-old African-American woman with ESRD was transferred from dialysis clinic to the emergency room (ER) for evaluation of non-radiating and dull epigastric pain for two weeks associated with fever and chills during hemodialysis (HD). Three months ago, she was hospitalized and treated for Streptococcus pneumoniae and Enterobacter cloacae bacteremia. A year ago she was treated for S. maltophilia bacteremia secondary to an infected dialysis catheter. Past medical history was also significant for hypertension, atherosclerotic vascular disease pending elective coronary artery bypass graft (CABG) surgery, and sudden cardiac arrest followed by ICD placement. With the current presentation, both blood and catheter cultures obtained at the dialysis clinic were positive for S. maltophilia, prompting her subsequent arrival to the emergency room. She presented with a continuation of fever and chills, as well as tachycardia and episodic hypotension. She was noted to have mild epigastric tenderness. There was no surrounding erythema, discharge, or tenderness noted around the tunneled dialysis catheter on the right anterior chest. Initial workup showed elevated troponin and procalcitonin. Chest X-ray findings were suggestive for left lower lobe pneumonia. Within the ER, the patient's hypotension resolved with fluid resuscitation and intravenous levofloxacin therapy was started with blood cultures drawn.\nCardiology was consulted for persistent elevation of troponins and it was presumed secondary to impaired clearance in ESRD. Transthoracic echocardiography (TTE) was done to evaluate for endocarditis given the presentation of bacteremia and fevers. TTE revealed artifact noted on an abandoned ICD lead in the right heart concerning for possible vegetation. Repeat blood cultures were positive for S. maltophilia and the patient was continued on levofloxacin. Infectious Diseases was consulted and as per their recommendation the infected tunneled dialysis catheter was removed on the 2nd day of the hospitalization (DOH). The patient continued to be febrile despite levofloxacin therapy and a transesophageal echocardiogram (TEE) was performed to look for a cardiac source. Subsequent blood cultures were negative at this time but the patient continued to be symptomatic. The TEE was done on the 4th DOH and showed a 1 x 0.5 cm echodensity attached to an abandoned right ventricular (RV) ICD lead in the superior vena cava (SVC) as it entered into the right atrium (RA). The echodensity was concerning for a vegetation due to IE. ID consultation recommended removal of the abandoned lead with culture of the probable vegetation that may have served as a nidus for recurrent bacteremia. Levofloxacin therapy was continued and a new dialysis catheter was placed on the 5th DOH with HD restarted. At this time, the patient became afebrile and reported symptomatic improvement. Despite a strong suspicion for IE by Duke criteria, fluorodeoxyglucose positron emission tomography (FDG PET) on the 6th DOH was non-diagnostic for differentiating infective etiology from thrombotic. Cardiothoracic surgery was consulted for removal of the abandoned lead for culture and planned to do so in coordination with her pending elective CABG procedure. In the interim, repeat blood cultures remained negative on the 7th DOH and the patient was stable and discharged on the 12th DOH with instructions to transition to oral levofloxacin until her CABG procedure, scheduled 18 days from discharge.\nUnfortunately, the patient expired due to complications from cardiac arrest secondary to severe hypokalemia in the postoperative period after removal of the infected ICD lead and successful CABG. No vegetation could be appreciated on gross inspection of the removed ICD-lead and subsequent culture was negative for any growth, indicating a resolution of the IE over the one-month course of levofloxacin treatment since presentation. |
A 42-yr-old man was referred to us by his local physician after a 1.5 hr episode of oral bleeding. His history was positive for a sore throat for 3 months that had been treated intermittently with antibiotics. There was no prior history of oral bleeding. An examination revealed bilateral tonsillar hypertrophy with hyperemia, and a blood clot in the lower pole of the right tonsil. However, no active bleeding was seen. The white blood cell count, hemoglobin, prothrombin/partial thromboplastin levels and bleeding time were normal. The blood clot was removed and the lower pole of the right tonsil was cauterized with silver nitrate. Oral antibiotics were prescribed and the patient was scheduled for a tonsillectomy.\nSeven days later the patient presented to the emergency room because of spitting fresh blood from the mouth for 1 hr. His tonsils were congested and fresh blood was seen oozing from the lower pole of the right tonsil (). His hemoglobin level fell from 14.9 g/dL to 11.2 g/dL. The patient was admitted to the hospital and an emergency tonsillectomy was performed.\nOn postoperative day 4, the patient presented to the emergency room with a history of bright red bleeding from the mouth for a few hours. His hemoglobin level was 6.9 g/dL and he received 2 units of packed red blood cells. In the operating room, diffuse oozing from the tonsillar beds was cauterized with bipolar diathermy hemostasis under general anesthesia. The hemoglobin level was 9.5 g/dL (post-transfusion). On postoperative day 9, the patient complained of fresh bleeding from the mouth. The bleeding was controlled by bipolar diathermy hemostasis under local anesthesia. The hemoglobin level was 6.7 g/dL and the patient received 2 units of packed red blood cells.\nDuring hospitalization, further evaluations for a bleeding disorder were carried out. However, there was no clinical or laboratory evidence of a hematological or clotting disorder. On postoperative day 14, the patient was discharged from the hospital. |
A 27-year-old G3P22002 female presented at 19 weeks and 1 day of gestation by last menstrual period for scheduled prenatal visit. Ultrasound study was performed and revealed a single fetus and adequate amniotic fluid with limited fetal measurements consistent with estimated gestational age of 17 weeks, that is, an age 2 weeks less than the estimated gestational age. Diffuse subcutaneous edema was seen, with possible multiloculated nuchal cystic hygroma. Fetal heart tones could not be detected and a three-vessel umbilical cord could not be documented.\nThe maternal history included 2 previous uncomplicated term vaginal births of children with no anomalies. Age at menarche was 13 years. The mother is a nonsmoker and she was taking supplemental vitamins. She reported no alcohol, caffeine, or illicit drug intake. She is sexually active with a single partner. Most recent cervical pap smear was approximately 4 years ago, with normal results. Prior to this pregnancy, her periods were regular and heavy. Her IgG for rubella was positive approximately at 8 weeks' gestation. The patient denied any significant family history for congenital conditions.\nBecause of fetal demise, labor was induced with misoprostol and resulted in a spontaneous vaginal delivery of a stillborn female fetus followed by spontaneous delivery of the placenta. The fetus and placenta were transferred to our institution for autopsy and placental examination.\nAt autopsy, a slightly growth-restricted previable, immature female fetus with grade 1-2 maceration and moderate-to-marked hydrops was examined. The posterior nuchal fluid accumulation was prominent. The ears were low-set and posteriorly rotated. The fingers were short bilaterally (), and the right foot showed absence of the second and third digits (). The pulmonary artery was slightly narrow, with no other cardiovascular findings. Microscopic examination showed desquamated epidermis and nuchal edema. Evaluation of the organs showed predominantly marked autolysis consistent with retained stillbirth.\nPlacental examination revealed membrane laminar necrosis, consistent with acute hypoxic injury. The undersurface of the chorionic plate showed focal pseudovillous papilliform cytotrophoblastic proliferation (). This was highlighted by double immunohistochemical stain for E-cadherin/CD34 (). The chorionic villi were large and cellular, with convoluted outlines and presence of both trophoblastic pseudoinclusions (), secondary to villous scalloping, and focal robust ferrugination of the basal lamina (), highlighted by iron stain (). Rare clusters of villous cytotrophoblasts were seen, with normal villous vascularity (), highlighted by E-cadherin/CD34 immunostain (). The number of cell islands was increased. The chorion laeve showed an uneven distribution of perpendicularly oriented and elongated clusters of sclerotic chorionic villi ().\nG-banded karyotyping of placental tissue revealed a 48,XXX,+18 karyotype (). Due to maceration, karyotype of fetal tissue was not obtained. Fluorescence in situ hybridization testing for chromosomes X and 18 was performed on relatively well-preserved thymic tissue and highlighted double trisomy of these chromosomes (), which rules out an isolated placental mosaicism. |
A 26-year-old male presented at the Department of Orthopedic Surgery, Southwest Hospital, complaining of a dull intermittent pain, for three months, along the anterolateral aspect of the right knee. The pain was relieved by taking Celebrex®, however, it was exacerbated by activity. The patient had identified a slow-growing mass on the outside of the right lower thigh and, a month prior to admission, the patient had noted a moderate sensation of warmth in this region. There was no history of accident, injury, fever, weakness or weight-loss. The patient visited another hospital in April 2012 and radiographs demonstrated a tumorous lesion in the right distal femur. The patient was referred to the Southwest Hospital in May 2012 with an unremarkable medical history. However, the physical examination was notable due to swelling, a decreased range of motion of the right knee and a palpable mass (size, ~5×4×3 cm). The laboratory assessments of the patient’s alkaline phosphatase levels were unremarkable. The radiographs revealed an expansile and osteolytic lesion of the right distal femur with a periosteal reaction (). Magnetic resonance imaging demonstrated an expansile, intramedullary, poorly defined neoplasm with a moderately high signal intensity area on T1- and T2-weighted images in the distal part of the femur, with diffuse erosion of the cortex and involvement of the surrounding soft tissue (). The intraosseous central location and uniform cortical destruction indicated that the lesion was not a soft-tissue sarcoma, which are associated with bone invasion. An emission computed tomography (CT) bone scan demonstrated an abnormal isolated concentration of radioactive agent (used for bone imaging) at the femoral site, which revealed that no distant metastases had occurred (). Chest CT did not demonstrate any thoracic abnormality and the CT-guided biopsy results showed blood clots as well as a small quantity of heterogeneous cells (). Therefore, the clinical diagnosis was a malignant bone tumor, with the most likely diagnosis considered to be an osteosarcoma, as a primary bone tumor.\nA limb-salvage procedure, involving a wide resection and a total knee endoprosthesis replacement, was performed in May 2012. Examination of the resection specimen showed a soft, light-yellow (or gray), gelatinous tumor measuring 13 cm along the long axis of the femur and 6 cm transversely. The tumor was centered in the distal part of the femur, with involvement of the surrounding soft tissues. A central hemorrhagic and necrotic area contained serosanguineous fluid, with an abundant local blood supply (). No intra-articular extension was observed and there was no indication of regional metastasis on dissection of the popliteal fossa lymph nodes. The patient’s postoperative course was uneventful.\nMicroscopically, the tumor was identified as a sarcoma, which demonstrated ovoid and polygonal tumor cells that were of diffuse distribution with obvious atypia and mitotic figures. In addition, a combination of numerous giant tumor cells and fatty tissue with different degrees of differentiation was observed (). Immunohistochemistry showed positive staining for S-100 protein and cluster of differentiation (CD)68, and negative staining for smooth muscle actin, CD34 and vimentin. The final pathological diagnosis was a primary dedifferentiated liposarcoma of the femur, and the dedifferentiated tissue was identified as malignant fibrous histiocytoma.\nNo other types of therapy, including chemotherapy and radiation, were selected prior to and following surgery. The patient was followed up for 12 months after surgery, and no recurrence and metastasis was detected. In addition, radiography indicated that the artificial joint was in good condition. |
A 29-year-old male with no pertinent past medical history presented to our ED with two weeks of intermittent headaches. His headache was described as throbbing in character, localized to the bitemporal region, non-radiating, and non-positional. The headache occurred daily, lasting a few minutes to hours, with no particular exacerbating factors. His symptoms improved with acetaminophen, which he used sparingly. He reported no associated vomiting, gait abnormalities, vision changes, confusion, urinary changes, or other neurologic abnormalities. He had been treated at four different EDs in the two weeks prior to presentation for the headaches, but no imaging studies had been performed. The patient had no psychiatric history. His highest level of education was a high school diploma, and he was unemployed.\nOn arrival, the patient was afebrile with pulse, blood pressure, and respiratory rate all within the normal range. Physical examination revealed an anxious male who was alert, oriented, and in no acute distress. His head was normocephalic with no evidence of trauma. His pupils were equal, round, and reactive to light. His neurological examination did not reveal any cranial nerve deficits, speech abnormalities, muscle weakness, or loss of sensation. His reflexes were intact and symmetrical. His coordination was normal. His gait was stable with balanced cadence, and he exhibited a negative Romberg test. His visual acuity was 20/20 in both eyes. The remainder of the examination was unremarkable.\nLaboratory values of complete blood count and complete metabolic panel were unremarkable. The serum carboxyhemoglobin level was within the normal range. Due to the patient’s headache not being fully consistent with a primary headache and his multiple visits to the ED without a history of imaging, computed tomography (CT) of the head was ordered to assess for a possible anatomic cause of his symptoms. The CT was notable for marked enlargement of the occipital horns of the lateral ventricles with agenesis of the corpus callosum, consistent with colpocephaly ( and ).\nThe patient was evaluated by the neurology service in the ED. It was their opinion that his headaches were primary in nature and not associated with the incidental finding of colpocephaly. His headache resolved after receiving 10 milligrams (mg) of intravenous metoclopramide and 50 mg of oral diphenhydramine, and he was discharged home with neurology follow-up. The patient returned to the ED one month later for an unrelated complaint and did not report a headache at that time. |
A 42-year-old smoker female patient came to the Dental School complaining of facial pain and a poor appearance of her teeth. Based in a visual analogue scale (VAS), the patient classified herself as having level 7 of orofacial pain. In addition, patient reported to suck lemon and eat spicy food daily. During clinical examination, good oral hygiene could be noted. The patient showed extreme corrosion of occlusal surfaces of the maxillary posterior teeth and the palatal surfaces of the maxillary anterior teeth (). Anterior teeth presented extensive composite resin restorations, worn incisal edges with loss of restoration/enamel gloss at labial surfaces (), which is a characteristic appearance of exogenous corrosion. On the palatal surfaces, it was noted loss of enamel with smooth and shiny appearance, and worn incisal surfaces, suggesting bruxism activity. Posterior amalgam restorations were salient and fractured, being almost out of the teeth they were inserted in the past, due to enamel corrosion ( and ). Mandibular teeth did not have important alterations at that moment, but the patient had already installed 3-element posterior fixed metal-ceramic prosthesis, full crows, one indirect partial metallic restoration, satisfactory amalgam restorations, and had no worn teeth ().\nBased on clinical interview and clinical examination, worn teeth were related to the association among bruxism, perimolysis and exogenous corrosion by food habits. The patient's occlusion was clinically evaluated and it was found a decreased vertical dimension, secondary to bruxism and enamel erosion. The decreased vertical dimension and the absence of anterior and lateral guides seemed to be associated with muscular pain and difficult mandibular movements.\nAfter determining the etiologic factors, a multiphase treatment was proposed. Firstly, the patient was asked to settle an appointment with a gastroenterologist. Concomitantly, she was warned about her general and dental health, receiving nutritional instructions and counseling and information on the importance of reducing the smoking habit. In the second phase, the use of an occlusal splint to reestablish vertical dimension and control the muscular pain was indicated. The third phase was the esthetic functional rehabilitation with direct resin-based composite restorations, with definitive reestablishment of previously determined vertical dimension and anterior guidance. Occlusal adjustment and a second occlusal splint, comprising the last phase, are essential for good clinical longevity.\nThe patient was advised about smoking consequences after and during dental treatment and also on general health. In addition, she was warned about her destructive bruxism habit and counseled to control it consciously during waking hours, as much as possible. After these simple initial counseling, the patient was referred to a gastroenterologist for medical consultation that confirmed GERD diagnosis. Endoscopy examination showed a hiatus hernia. The gastroenterologist prescribed Omeprazole 40 mg/day to the patient and suggested her to elevate the head of her bed during sleeping and avoid acid, fatty and spicy food, especially right before bedtime. This was intended to provide a symptomatic relief and to discourage further progression of erosion. In these cases, after successful medical intervention, dental therapy is necessary to restore dental esthetics, form and function.\nAccording to Kirveskari, et al. (1989), there is an association between occlusal interferences and signs of craniomandibular dysfunction. Therefore, in the present case, patient TMD signs and symptoms seemed to be related to tooth wear. As prosthetic rehabilitation aims at fulfilling optimal occlusal parameters, this situation cannot be postponed. Additionally, the lack of space in patients with reduced vertical dimension of occlusion makes any rehabilitation therapy impossible to be performed. In these situations, some authors have recommended occlusal splints for pain elimination because it is a reversible and efficient treatment approach·. An occlusal splint was fabricated enabling jaw position in centric relation of occlusion (CRO), occlusal stability and anterior guidance (), controlling pain episodes and reestablishing vertical dimension. The occlusal splint was adjusted according to the parameters of an organic occlusion: the occlusal forces should be directed to the long axis of the posterior teeth () and when posterior teeth occlude there should be no contact among upper and lower anterior teeth (and vice-versa) (, and ); After using the splint for one week, the patient reported absence of pain episodes (VAS = 0), which confirmed previous muscle pain diagnosis and occlusal splint efficiency. The occlusal splint was used for 90 days, with monthly clinical appointments (VAS = 0).\nAfter 90 days, the patient returned to continue the treatment. Again, she reported absence of muscle pain and TMJ comfort, which mean that a perfect adaptation to the correct vertical dimension of occlusion was obtained. The technique of choice for restoration of the worn teeth was the use of direct hybrid composite resin restorations.\nAlginate impressions were taken and the cast models were mounted in CRO on a semi-adjustable articulator by means of a jaw interference guide (JIG) device, intended either to act as an occlusion program changer or to facilitate the manipulation into CRO as it relaxes the musculature. The areas to be restored () were waxed up on the models for diagnostic analysis of the desired occlusal relation (, and ). The possibility of establishing uniform contacts during mandibular movements, correct dental contour and maintenance of a desirable stomatognathic system balance was verified during waxing of stone models (). A lingual matrix was created with condensation silicone (Silon 2 APS, Dentsply, MD, USA) () because this has been reported to be the ideal method to transfer the occlusal scheme obtained on the articulator to the clinical situation. Teeth were restored according to the previous occlusal adjustment that was performed on the occlusal splint (). After restoration of a tooth quadrant, the splint was cut and occlusal adjustment was performed (, and ).\nThe first step on an adhesive restoration is color selection. The teeth should be thorough cleaned with water/pumice slurry prior to shade selection. Colors A3.5 and A3 for dentin and A2 for enamel were selected. The cavities prepared for corrosion/abrasion lesions are limited to the removal of preexistent restorations and carious tissue. Nevertheless, caries are generally an uncommon occurrence in patients with erosion, and teeth do not tend to retain plaque due to presence of smooth and polished surfaces.\nAfter pumice prophylaxis, enamel and dentin surfaces were etched with 35% phosphoric acid (Scotchbond Etch gel, 3M-ESPE, St. Paul, MN, USA) for 15 seconds (). After rinsing with air/water spray for 20 seconds, an one-bottle adhesive (Adper Single Bond 2; 3M-ESPE, St. Paul, MN, USA) was applied, left untouched for 20 seconds, re-applied and light cured for 20 seconds ().\nHybrid composites better enable the reproduction of areas that receive normal occlusal load because they have a reinforcement of the organic matrix resulting on better physical properties. Increments of the universal hybrid composite Filtek Z250 (3M-ESPE, St. Paul, MN, USA) were applied to the silicone matrix (shade A2) (), correctly positioned at the respective surfaces and light cured for 20 seconds in order to restore the palatal surfaces, simulating the lost enamel layer. On anterior teeth, dentin shade composite resin should be applied over the transition line between composite and tooth structure in order to mask this region. This composite resin is also applied to create the basic buccal tooth anatomy represented by dentin development lobes. A3.5 shade was used to create dentin lobes close to cervical tooth regions and A3 at mid third to incisal or occlusal surfaces ( and ). Enamel was always restored with shade A2. Hybrid composite offers the resistance and opacity of dentin, contributing to the maintenance of the treatment and also enabling good esthetics.\nTeeth were adjusted according to the same parameters used for occlusal splint installation (). These parameters tend to maintain a stable occlusion during many years, being associated to a reduced occurrence of TMD's. Some authors have recommended the employment of cephalometric tracing or computer generated axiography on reestablishing anterior guidance. However, in this case, phonetic tests and an optimal occlusal adjustment produced good results with desirable function and elimination of disorder symptoms (muscle tenderness and TMJ sensivity). During occlusal adjustment, modifications are done on the surfaces of tooth, restorations or prostheses by means of selective wear or addition of restorative materials, seeking to harmonize maxillomandibular functional aspects in CRO and eccentric movements. On this full-mouth rehabilitation case, selective wear of tooth surfaces was performed. It is of paramout importance to correctly adjust occlusion for greater treatment longevity. For this, it is important to control the direction, magnitude, distribution and intensity of functional and parafunctional occlusal forces.\nTo develop the functional stability necessary for the success of the treatment, the dentist must evaluate the determinants of occlusion. The muscles of mastication must be examined and palpated for tenderness. The condition of the TMJs should be evaluated. A determination of the relationship of the condylar guidance to the anterior guidance has to be evaluated in cases associated with severe anterior wear. To meet all these requirements, the following criteria must be followed: the patient can function from centric relation to occlusion without occlusal interferences (); the TMJ is loaded without tenderness; there are posterior centric stops between the maxillary and mandibular arches before contact of anterior teeth; anterior coupling allows for disocclusion of posterior teeth (, and ); teeth are positioned in balance with the tongue and facial muscles.\nThe precise individual anatomy is correctly determined during finishing and polishing procedures. Ultra-fine diamond burs or multi-bladed burs are indicated to remove little excesses. After that, fine aluminum oxide discs (Sof-Lex, 3M-ESPE, St. Paul, MN, USA) at low speed were used in order to achieve surface gloss and smoothness. To obtain restorations that reproduced the enamel surface texture a diamond bur was used, at low speed, and then only a superfine grit disc, resulting in an excellent final esthetic result. |
MO was a 44-year old single woman born in Artvin in Turkey. She was unemployed and had dropped out of university in her sophomore year. She was brought into our hospital by her family against her will. She was complaining of paranoia and introversion. In addition, she refused to speak, eat or leave her house. She also rejected other people’s company and preferred to stay on her own. She could not sleep.\nAccording to her family, her first complaint had started at the age of 22 before she went to university in Ankara. Based on information from her family, we learnt that she had begun to skip classes and this had had a negative impact on her academic achievement. Also her family said that she was hearing strange noises. Since then she had been treated in different hospitals and cities, being given various medications based on the diagnosis of paranoid schizophrenia. It was reported that in the first 15 years of her treatment she was prescribed with haloperidol (20 mg) irregularly and then she was prescribed with anti-psychotics such as fluphenazine decanoate (25 mg), trifluoperazine (5 mg), thioridazine (100 mg), zuclopentixol (200 mg). It was also said that in the last five years of her treatment she was prescribed with olanzapine 20 mg/day and risperidone 6 mg/day irregularly. Some side effects, such as a frequent sensation of slowing down, limited movements, tremor in her hands and dryness in her mouth, were observed during the period of medication. The family pointed out that there had been some improvement in her condition while she was taking her medications, but their side effects compromised continuity of treatment. Her symptoms worsened and her family brought her to the hospital. On presentation she had not taken prescribed medication for seven months.\nMO was the youngest of 3 children and had an older brother and sister. Her mother was a 68-year old housewife who had finished elementary school. Her father had died in an accident 20 years earlier. There was no history of any neurological or psychiatric disease in the family. She had been successful at school and had gone to Ankara to study at the university after passing the entrance exam. In the first year, she had stayed in a dormitory with her friends but in the second year she began to live alone in a flat. She had to drop out of university in the second year because of her illness. She had not had any medical problems before her illness. She had been smoking one packet of cigarettes every three days. However, this amount had increased over the previous few months. Six months before presentation she had begun to suffer from eating disorders and insonnia, although she had had no previous history of either. She refused meals prepared by her mother, believing them to be poisoned. According to her family, the patient had been extrovert, more talkative, and generally happier and livelier before her illness. She had subsequently become introverted and never wanted to leave the house. She was still living with her mother and her unmarried sister, six years older than herself.\nVital findings, neurological and other system examinations were all normal.\nLaboratory findings, hemogram, EFG, and brain magnetic resonance image (MRI) were also all normal.\nAt her first psychiatric appointment, the female patient, who looked older than her years, looked well groomed and behaved with respect. She kept looking around, didn’t speak spontaneously, and replied hesitantly only when asked questions. During the interview she was agitated, her attention and concentration were poor, and she did not always seem to understand what was being asked of her. She showed no emotional response to the conversation and admitted to hearing voices that discussed her actions, ridiculed her, and at times warned her not to interact with family members. She had a persecution complex and expressed illusions of grandeur.\nAt the first psychiatric evaluation of the event it was confirmed that the patient had used classic and atypical antipsychotic, anticholinergic drugs for various periods over 22 years. During these periods she had experienced serious side effects such as akathisia, tremors, and deceleration in her activities, and these had not been completely resolved by the medication received. The patient was diagnosed with treatment-resistant schizophrenia and was treated with clozapine. Drug dose was gradually increased over six weeks (started at 12.5 mg/day and titrated up to a target dose of 450 mg/day). About 20 months after the initiation of clozapine, the patient developed involuntary movements of her mouth. Signs of typical RS were evident. The movements were fine, rhythmic, and rapid, along the vertical axis, and without lingual involvement. The patient and her family reported that dyskinetic movements of the patient’s lips occurred during the examination, performed in the 16th month of her treatment, and that her social activities and communications with others had improved. The patient and her family were informed about the progress of the illness. Clozapine was stopped and treatment was changed to quetiapine. After four weeks of quetiapine treatment (starting dose of 100 mg/day and titration up to 700 mg/day) her RS symptoms diminished significantly with further improvements during the follow-up period. Her Abnormal Involuntary Movement Scale (AIMS) score dropped from 14 to 8. The most prominent, albeit controversial, treatment agents for RS are benzhexol and biperiden. We were anxious to avoid aggravation or TD by the addition of anticholinergic agents. |
A 23-year-old athletic female in the United States military presented with a five-year history of chronic left-sided anteromedial hip pain and urinary incontinence. The initial onset of pain was attributed to a grade II stress reaction of the proximal left femoral diaphysis at the adductor insertion from frequent running. While she did recover from this injury with rest and use of crutches, the patient had intermittent hip pain with running, hiking, and lifting. Over-the-counter non-steroidal anti-inflammatory medications, such as ibuprofen, did not improve the pain. The patient’s hip pain worsened one year prior to the presentation during her second pregnancy. Her symptoms worsened to include bilateral hip pain, low back pain, pelvic pain, and pelvic floor dysfunction. She completed a six-month course of pelvic floor physical therapy resulting in improvements in urinary continence, but not pain and function. A repeat left hip radiograph did not demonstrate any new fracture or stress injury. She had been seeing her primary care provider prior to her current presentation, who was able to determine that her current pain was unrelated to an infectious or rheumatological disorder. She was subsequently referred to an Interdisciplinary Pain Management Clinic (IPMC) for evaluation and management. The patient was evaluated by a pain management physician who recommended a comprehensive, multi-modal rehabilitation plan to include myofascial mobilization, consideration of platelet-rich plasma injections, yoga, and referral to the pain management physical therapist.\nUpon presentation to the clinic’s physical therapist, the patient’s goal was to run and exercise without pain. She wanted to explore all treatment options including another trial of physical therapy. The standard physical therapy evaluation and treatment practice incorporate structured history-taking to develop a working diagnosis and differential, a physical examination designed to rule in or out a specific diagnosis, and a rehabilitation program to address the impairments identified during the exam. Unfortunately, the patient’s pain level was elevated on initial presentation and the threshold for tissue irritability was low; therefore, the likelihood of the examination causing too much pain in which the patient would not want to engage in an exercise program was high. Instead, the examination was adjusted to maneuvers that were the least pain-provoking.\nPhysical Examination\nPhysical evaluation at the IPMC revealed that the patient’s pain was localized to the left anteromedial hip at the inferior pubic ramus and the iliopsoas tendon as it passes under the ilioinguinal ligament to the insertion at the lesser trochanter. The patient’s average pain level was rated as a 4/10 on a visual analog pain scale. With this scale, the pain was measured with 0 indicating no pain and 10 indicating the worst pain the patient has ever experienced. She did not report any pain along the lumbar spine or SIJs or radiation of pain down her legs. A neurological clearing examination of L1-S1 revealed no dermatomal sensation changes or myotomal weakness in her bilateral lower extremities. A straight leg raise test was negative bilaterally. While standing, palpation revealed asymmetry in the patient’s pelvic landmarks with her posterior superior iliac spine (PSIS) and iliac crest elevated on the right side as compared to the left. The patient’s lumbar range of motion (ROM) was full and pain-free. Hip ROM was assessed passively with a goniometer and found to be within normal limits for all motions except extension. The hip extension was limited to five degrees on the left side. With moving of her leg passively into resistance, pain was reproduced. A positive flexion abduction external rotation (FABER) test was appreciated with decreased hip motion and pain. While standing, she was instructed to actively flex her hip towards her chest. As she lowered her leg, pain and a clicking sensation were appreciated with palpation over the iliopsoas tendon. During ambulation, she exhibited a Trendelenburg sign.\nMotion Palpation Tests\nThe motion palpation tests utilized included the forward flexion test and Gillet’s test. In the forward flexion test, which was assessing for hypomobility of the SIJ, the test was positive when the PSIS on the involved side moved first when the patient bent forward. Gillet’s test, which was assessing for underlying sacroiliac dysfunction, was positive since the PSIS on the involved side did not move inferiorly as the patient flexed her hip actively.\nPain Provocation Tests\nIn the prone position, pressure was applied from a posterior to the anterior direction to the spinous processes from L1 to L5. No pain or segmental hypomobility was noted during the maneuver, indicating no pain referral from the lumbar spine. While supine, a thigh thrust test was performed to assess for SIJ dysfunction. With the patient’s knee and hip flexed, force was applied axially through the knee producing a shear force to the SIJ. The test was positive for pain and joint hypomobility. Finally, a piriformis test was performed to detect muscle tightness, which was found to be positive.\nStrength Testing\nStrength testing was performed while supine. Pain was partially reproduced with resisted hip adduction and flexion. In standing, the patient was instructed to push a 10-pound weight with her foot towards the right to simulate contraction of the hip adductors. This maneuver was selected as she reported an acute reproduction in her pain produced when she tried to move a 10-pound weighted box with her foot at work the previous week. She was unable to slide the dumbbell and her pain level increased to an 8/10 level, but quickly returned to her baseline of 4/10. This activity would serve later as a litmus test to determine the efficacy of selected treatment interventions directed at each impairment identified during the physical examination.\nDiagnosis/Prognosis\nThe differential diagnosis specific to this patient’s symptoms upon initial evaluation to the IPMC included a hip adductor or flexor muscle strain or tendinopathy, intra-articular etiology (i.e., femoral acetabular impingement, osteoarthritis, or labral pathology), pelvic pain syndrome, snapping hip syndrome, or SIJ dysfunction. Given the lack of trauma at the time of her pain recurrence, a labral tear or other intra-articular pathology was excluded. Her pain reoccurred during her second pregnancy; therefore, SIJ dysfunction was considered high on the differential. However, the high level of pain localized to the anterior hip with pain reproduced with contraction of the hip flexor and adductors suggested a muscular pain generator, such as pregnancy-related PGP.\nThe prognosis for full resolution of symptoms was poor given the chronicity of symptoms, the poor response to previous conservative measures, and the short period of time that the patient would be available for care. The patient was planning to relocate in four weeks' time.\nIntervention\nTo validate the working diagnosis and identify contributing pain generators, an intervention-based diagnostic approach was utilized. First, biomechanical faults remote to the area of pain were addressed. Surrounding tissues to include the iliopsoas were treated to determine if this was the primary pain generator or contributing to her inguinal pain. After each intervention, the patient tried to move the dumbbell with her foot to validate if the tissue being assessed was a pain generator.\nUpon initial treatment, the patient underwent a common osteopathic manipulation technique (OMT) directed at the left ilium to address the SIJ dysfunction (Figure ). This technique allows the provider to apply a force at the ileum to posteriorly rotate it as the lumbar spine is locked down. The goal is to improve sacroiliac motion and improve a potential leg length discrepancy. After the maneuver, the patient reported less pain with contraction of the hip adductors against manual resistance, but was unable to move the dumbbell by contracting the left hip adductors in standing.\nTo identify the impact of iliopsoas tightness upon the patient’s pain presentation and the effect of stretching upon achieving treatment success, an active release technique was applied to the iliacus muscle (Figure and 2b). The active release technique artificially lengthens the muscle by decreasing tissue tension. By lengthening the tissue, the technique validates the importance of stretching the muscle in reducing pain. This procedure was repeated five times. The patient was then placed onto her right side, and the provider manually stretched her iliopsoas for five repetitions of 30 seconds. After this technique, the patient was able to move the dumbbell with a 1/10 pain level.\nThe patient was started on a home exercise program consisting of piriformis stretching, iliopsoas stretching, and lumbopelvic stabilization exercises directed at improving muscle endurance of the abdominals, hamstrings, and hip adductors. The patient was asked to follow up one week later.\nDue to the patient's work and scheduling conflicts, the patient was not able to follow up with physical therapy until two weeks later. Upon this visit, the patient reported the most pain relief that she had gotten over the last year. Her average pain level decreased by 50% to a 2/10. Pain with activity decreased from 8/10 to a 3/10 level. The iliopsoas stretching was reported to be the most effective exercise prescribed.\nPhysical examination revealed symmetrical pelvic landmarks and no joint hypomobility or pain derived during the thigh thrust test. Gillet’s and forward flexion tests were negative. Mild piriformis tightness was appreciated on the left side. The patient’s pain was mildly reproduced with passive hip extension with over pressure. The left iliopsoas was tighter than the right side. Active hip flexion in standing revealed a “click” consistent with snapping hip syndrome, but there was no pain. Contraction of the hip flexors and adductors in standing by moving the 10-pound dumbbell forward and medially with the foot did not reproduce her pain. The pain was reproduced with palpation to the iliopsoas tendon between the ilioinguinal ligament and lesser trochanter but not at the inferior pubic ramus.\nTreatment consisted of active release and manual stretching directed at the iliopsoas muscles for ten minutes. Right-sided piriformis tightness was addressed with instrument-assisted deep tissue mobilization using the VibraCussor® (IMPAC Inc, Salem, OR) directed at the piriformis for five minutes (Figure ) followed by manual stretching for another five-minute period. The patient was instructed to continue her home exercise program and to go jogging prior to her follow-up in two weeks to determine her progress towards her functional goal.\nUpon the third and final visit, the patient reported no pain at rest or with jogging. She ran three miles twice to validate that her goal had been met. The physical examination revealed iliopsoas tightness, but no pain was reproduced with resistance applied to the hip flexors or adductors. Pain was not reproduced with digital pressure over the iliopsoas tendon. She was encouraged to continue her stretching program at a minimum to optimize tissue length and reduce the snapping sensation. She was subsequently discharged from the IPMC. |
A 27-year-old woman, who was diagnosed with Wyburn-Mason syndrome at age 11 years, visited our clinic for a regular examination. At age 11 years, her best-corrected visual acuity (BCVA) was 20/500 in the left eye, and cerebral angiography demonstrated AVMs along the course of the left ophthalmic artery. There was no history of systemic diseases. On a regular examination at age 27 years, she was in the 8th month of pregnancy, and her obstetrician consulted us on the risk of hemorrhaging from the retinal AVMs at the time of parturition. Her BCVA was 20/12.5 in the right eye and light perception in the left eye. Exophthalmos was not found, and the ocular motility was normal. The intraocular pressures were 16 mmHg OD and 17 mmHg OS. The anterior segments and ocular media of both eyes and fundus of the right eye were within normal limits. However, severely dilated tortuous vascular loops consistent with retinal AVMs were distributed from the optic disc over all four quadrants of the left eye (Figure A). The arteries were directly connected to the veins without passing through any capillaries. The optic disc was obscured by the large vascular loops. Numerous anastomosing vessels made it difficult to separate the arterial and venous components. The vascular loops in some areas were more dilated and tortuous than 16 years earlier (Figure C). Magnetic resonance imaging revealed orbital and retro-orbital AVMs including canalicular segments of the optic nerve on the left side. Prominent serpiginous vessels could be detected in the left orbit, including the apex which encased the optic nerve. Neurologic examinations were unremarkable, and no changes were found in the facial skin.\nAfter delivering by Cesarean section, fluorescein angiography (FA) and indocyanine green angiography (IA) were performed. FA in the early phase showed a rapid transit of dye through the vascular loops without an intervening capillary bed and leakage from the loops, which indicated direct arteriovenous communications (Figure B). IA in the early phase (approximately 16 s after the injection of dye) also showed a transit of dye through the vascular loops. IA did not show any findings suggestive of choroidal AVMs, because details of the choroidal vessels in the posterior pole were obscured by the large retinal vascular loops.\nSD-OCT was performed with the Heidelberg Spectralis (Heidelberg Engineering, Heidelberg, Germany). SD-OCT demonstrated retinal edema with cystic changes and oval-shaped structures that represented cross sections of abnormal retinal vessels in the left eye (Figures E and F). Binarization of a choroidal area in the EDI-OCT image was performed by a modified Niblack’s method as reported in detail (Figure ) []. Briefly, an EDI-OCT image was analyzed by ImageJ software (ImageJ version 1.47, NIH, Bethesda, MD, USA). The examined area was 1,500 μm wide in the subfoveal choroid, and extended vertically from the retinal pigment epithelium to the chorioscleral border. This choroidal area was selected with the ImageJ ROI Manager. Three choroidal vessels with lumens larger than 100 μm were randomly selected by the Oval Selection Tool on the ImageJ tool bar, and the average reflectivity of these areas was determined. The average brightness was set as the minimum value to minimize noise in the OCT image. Then, the image was converted to 8 bits and adjusted by the Niblack Auto Local Threshold. The binarized image was converted to the RGB image again, and the luminal area was determined using the Threshold Tool. The light pixels were defined as the interstitial areas, and the dark pixels were defined as the luminal areas. After adding the data of the distance of each pixel, the luminal and interstitial areas were automatically calculated.\nIn the EDI-OCT images, the subfoveal choroidal thickness was computed to be 250 μm OD and 462 μm OS using the caliper function (Figures D and F). The lumens of choroidal vessels appeared larger in the left eye (Figures A and B). In the binarized images, the luminal areas of the 1,500 μm wide choroid were 307,165.6 μm2 OD and 545,780.7 μm2 OS, while the interstitial areas were 175,593.5 μm2 OD and 273,833.0 μm2 OS. The percentages of the luminal areas relative to the total choroidal areas were 63.6% OD and 66.6% OS. Enlargement of the luminal area in the left eye was evident with accompanying interstitial enlargement. |
A 55-year-old male presented with worsening shortness of breath and palpitations. He had been well until 1 week prior to presentation, when he started feeling short of breath initially on exertion, but later at rest. He also reported orthopnea. He had intermittent cough productive of white sputum and wheezing. He was anxious and tremulous. Since the onset of symptoms his exercise tolerance had declined from a baseline of more than ten blocks to two blocks. He reported no fever, chest pain, dizziness, syncope, rash or weight loss.\nHis medical history included hypertension, diabetes mellitus and hyperlipidemia. Medications at the time of presentation included aspirin 81 mg daily, hydrochlorothiazide 12.5 mg daily, diltiazem 30 mg three times a day, losartan 25 mg daily as well as fenofibrate and subcutaneous insulin (70/30) 40 units twice a day. He had undergone appendectomy 10 years before for acute appendicitis. He was allergic to seafood and ACE inhibitors. He reported active tobacco use with a five pack year history of smoking, drank three beers daily and had occasionally snorted cocaine, but stopped 2 years earlier. He had been born in US, had studied until 11th grade and had worked in a food store, but was currently unemployed. He was living in an apartment with his wife and was sexually active in a monogamous relationship. His mother and sister have diabetes mellitus and his father had died of an unknown cancer.\nOn examination, he was afebrile. His pulse rate was 100 beats per minute, blood pressure 142/84 mm Hg, respiratory rate 18 breaths per minute with oxygen saturation 94% on room air that improved to 100% on 2 liters of oxygen through a nasal cannula, and his body mass index was 28.8. He appeared comfortable. Cardiac examination revealed normal heart sounds and no jugular venous distention. There were bibasilar rales on auscultation of the lungs and no wheezing. The abdomen was non-distended, soft and non-tender; the liver was palpable 5 cm below the right costal margin and bowel sounds were normal. He was alert and fully oriented, with motor strength and sensation normal in all extremities. Extremity examination was unremarkable, without any edema.\nHematocrit was 41.2%, white blood cell count 6.4 × 109/l and platelet count 245 × 109/l. The basic metabolic panel including serum sodium, serum potassium, blood urea nitrogen and serum creatinine was normal. The results of liver function tests, iron studies and tests to evaluate other etiologies of elevated transaminases are given in table . The blood level of proBNP was elevated to 1,002 pg/ml and hemoglobin A1C was 7.8%. Serologic human immunodeficiency virus testing and urine toxicology screen were negative. Electrocardiogram revealed sinus tachycardia, left atrial enlargement and QT interval prolongation. Chest X-ray showed pulmonary vascular congestion but no infiltrates. Echocardiogram showed severely reduced ejection fraction of 32% with global hypokinesis and eccentric left ventricular hypertrophy. Ultrasound of the abdomen revealed a large heterogeneous liver measuring 18 cm in length.\nThe patient was admitted to hospital with an initial impression of systolic congestive heart failure. He was treated with intravenous furosemide for diuresis with subsequent rapid symptomatic improvement. High levels of serum ferritin in conjunction with the clinical findings of heart failure, hepatomegaly and diabetes mellitus suggested an iron overload state. We expected the transferrin saturation to be >45%, as seen in hereditary hemochromatosis, the most commonly identified genetic iron overload disorder. However, the calculated transferrin saturation was only 28%. DNA mutation analysis showed that the patient was heterozygous for C282Y mutation and negative for H63D mutation. |
A 40-year-old female was found to have an asymptomatic distal SAA by abdominal ultrasound during an annual health-screening medical examination 1 year ago. She had no history of aneurysm, pancreatitis, trauma or connective tissue diseases, and no positive signs on physical examination. She was then referred to our hospital for further diagnosis and treatment. After admission, we used 2-dimensional color Doppler ultrasound to find that the aneurysm was located in the splenic hilum, with a maximum diameter of 2.7 cm. This was confirmed by CT scan (Fig. A). A multidisciplinary treatment (MDT) meeting was held to discuss the therapeutic strategy for this patient. Since the proximal splenic artery was tortuous, interventional radiology suggested coil embolism of the splenic aneurysm inflow and outflow. Considering the high risks of postoperative splenic infarction, postsplenectomy thrombocytosis and potential immunodeficiency, the patient preferred laparoscopic aneurysmectomy with reconstruction of the splenic artery in order to preserve the spleen.\nThe laparoscopic technique was performed with the patient in the supine position. The patient was put under general anesthesia and received endotracheal intubation, a nasogastric tube, and urinary catheterization. The surgeon stood to the patient's left, and the first assistant and camera assistant stood to the patient's right. After the pneumoperitoneum was established, another 3 trocars were inserted and the port sites positioned, as depicted in Fig. A. Part of the gastrocolic ligament was opened and the stomach was retracted upward to expose the splenic artery and SAA. The portion of the splenic artery proximal to the SAA was dissected and looped to prevent injury (Fig. B). The inflow tract of the SAA was carefully dissected, and temporarily clipped to evaluate the residual blood supply to the spleen (Fig. C). Most of the spleen appeared ischemic after clamping SAA inflow. Next, the SAA was resected and the splenic artery reconstructed by end-to-end anastomosis, using a running 5-0 prolene suture (Fig. D and E). Blood supply to the spleen was recovered after reconstructing the splenic artery (Fig. F).\nThe laparoscopic procedure was completed in 170 minutes without blood transfusion. Total blood loss was 100 mL. The patient was ambulated 12 hours postoperation and discharged 5 days after surgery. On the third day after operation, CT showed no thrombosis or stenosis of the splenic artery and a well-perfused, homogeneous splenic parenchyma (Fig. B). Splenic function was normal per the postoperative blood count. Postoperative recovery was smooth without hemorrhage, infarction, splenic abscess, or splenic artery thrombosis. Postoperative pathological results show that this was a true splenic aneurysm (Fig. ). No hemorrhage, aneurysm recurrence, splenic infarction, splenic artery stenosis, or thrombosis occurred in the 10 months following. |
We present the case of a 54-year-old patient with bacterial endocarditis who has been regularly visiting his cardiologist for follow-up on a mitral valve prolapse and mild to moderate mitral regurgitation (MR) for the last 11 years. Although the patient was not at high risk of developing IE and the 2009 ESC Guidelines on the prevention, diagnosis, and treatment of infective endocarditis did not recommend antibiotic prophylaxis in the patient, he was advised by his cardiologist that he underwent antibiotic prophylaxis for invasive manipulations including dental procedures. The patient generally followed this advice, and at the time of his current admission, his past medical history was insignificant except for a posterior ST-elevation myocardial infarction treated with aspiration thrombectomy in 2011. In November 2019, the patient visited his dentist for a dental procedure. Antibiotic prophylaxis was not used. In January 2020, the patient visited his primary care physician due to complaints of fever up to 37.5°C and cough. Symptomatic treatment was initiated with a reduction in temperature to around 36.9–37.0°C. In February, the patient made another visit to his primary care physician due to low-grade fever, persistent cough, and malaise. He was referred to a pulmonologist and antibiotic therapy with cefuroxime 500 mg b.i.d. was commenced with an initial positive effect. Due to relapse of symptoms in March and April, another two oral antibiotic courses were prescribed with little effect. In May 2020, after testing negative for COVID-19 in both Reverse transcription polymerase chain reaction (RT-PCR) and rapid IgM/IgG tests, he was prescribed his fourth course of antibiotic therapy with clarithromycin SR 500 mg/day. In the 2 weeks leading to the date of admission, his fever rose to 38.5°C and his cough became especially pronounced in the supine position which led his pulmonologist to refer him for a cardiology consult.\nThe patient was admitted to the intensive cardiac care unit after his transthoracic echocardiography (TTE) showed a previously non-existent structure on the posterior mitral valve leaflet (PMVL) with an increase in the severity of mitral regurgitation (see ). The patient did not report taking any medical therapy at the time of admission. His overall condition was satisfactory (see ); his blood pressure was 125/81 mmHg and his heart rate 74 b.p.m. A pansystolic murmur could be heard at the apex with radiation to the left axilla. Peripheral oedema was not present. He was afebrile at first, and later during the day of admission, he developed a temperature of 37.7°C.\nThree sets of blood cultures were taken and empiric antibiotic therapy was started with vancomycin and gentamicin in addition to intravenous furosemide. Five days later, the blood cultures came back positive for Streptococcus viridans which was sensitive to vancomycin and ceftriaxone but resistant to gentamicin. The patient had two major and two minor criteria (definite IE) according to the 2015 ESC Guidelines for the management of infective endocarditis. On account of the large size of the vegetation, it was decided to continue the antibiotic therapy with both vancomycin and ceftriaxone. By the end of the first week, his fever and cough had subsided completely. Due to the size of the vegetation (approximately 15 mm × 10 mm), severe mitral regurgitation and the high risk of embolization assessed through TTE, it was recommended the patient underwent surgical mitral valve replacement (MVR) (Class IIa recommendation). Nevertheless, the timing of surgery was decided to be elective (after completing a full antibiotic course), a decision based mainly on the positive therapeutic effect and the low risk of embolism (∼7%) at 4 weeks after admission as assessed by using a symptomatic embolism risk calculator.\nAfter completing the 4-week antibiotic course, the patient’s overall condition had improved which was also noticeable in the laboratory tests (see ). The follow-up blood cultures, taken after normalization of PCT, did not show bacterial growth.\nHowever, the surgical intervention was postponed for another 2 weeks due to a COVID-19 case in the department of cardiac surgery. While waiting for surgery the patient suffered a transient ischaemic attack (TIA) with a short-lasting episode of dizziness, blurred vision, and vomiting. Diffusion-weighted MRI did not detect cerebral ischaemia and concerns about a septic embolization were cast aside after TTE did not show any change in the morphology of the vegetation (see and and ).\nSubsequently, in July 2020, the patient underwent a successful MVR surgery with St. Jude Medical (SJM) No. 35 mechanical valve (Abbott Laboratories, Chicago, IL, USA) via a standard approach. His pre-operative markers of inflammation including PCT were within reference ranges (see ).\nAt a 5-month follow-up in December 2020, the patient was feeling well with no signs of infection, had a normal functioning mechanical valve assessed by transthoracic echocardiography and a therapeutic international normalized ratio (INR). |
The 83-year-old patient presented herself at a neurological emergency department with bilateral blindness and a history of severe headache, which she described the day before. An urgent computed tomography (CT) scan of the brain had been arranged, and a massive pituitary adenoma with hemorrhage was observed, and admission to the neurosurgical department immediately followed. When arriving at the hospital the hemodynamic parameters were stable (BP 130/90, 65 BPM); Glasgow coma score was 14/15. Her medical history included chronic renal failure (III°) as well as cardiovascular disease with hypertension, chronic atrial fibrillation, coronary heart disease with percutaneous transluminal coronary angioplasty, and stent. Therefore, dual oral anticoagulation with clopidogrel and acetylsalicylic acid was administered. Multiple electrode aggregometry did not detect effects of both anticoagulants. The neuro-ophthalmic examination revealed no perception of light in both eyes. Fundoscopy was normal without pallor of the optic nerves, no meningeal signs, and no overt clinical signs of hormone imbalance, but the measured pituitary hormone profile had a slight elevated prolactin level and suppression of sex hormones and thyroid profile (Table ). The blood tests revealed normal electrolytes and infection parameters. A magnetic resonance imaging (MRI) demonstrated a large 22 mm x 18 mm x 15 mm enhancing hemorrhagic pituitary macroadenoma with suprasellar extension and compression of the optic chiasm (Figure ). Emergency surgery was indicated. A transsphenoidal approach was carried out under general anesthesia. The pituitary tumor was identified, though it was not characteristic of a hemorrhagic pituitary macroadenoma. The tumor was tough and yellow colored, and debulking with a sharp curettage was difficult. Acute hemorrhage ceased after resection of tumor. Dopamine agonist was not used due to the patient experiencing complete blindness. After decompression, the surgery was concluded. After a period of cardio-pulmonary stability in the ICU, the patient was extubated. In this case, the transsphenoidal decompression of the optic chiasm provided an improvement in both eyes. Her visual disturbance improved with visual acuity from 0 to 5/100 for both eyes postoperatively. Counting fingers, identifying persons, and color vision were possible. On the first postoperative day, she was anxious and complained of gradual increase in shortness of breath. A non-ST elevation myocardial infarction was diagnosed. Additionally, to that, she developed an acute renal failure requiring temporary hemodialysis. After a short period, kidney function recovered, and cardiovascular stability was observed. The pathological examination confirmed a pituitary adenoma, most of it undergoing infarction. |
A 30-year-old Chinese female with no obvious cause appeared with numbness and inductance of lower extremities 2 months previously. It initially manifested as double plantar numbness, which gradually progressed to both legs, and was accompanied by double leg weakness, walking instability and a foot cotton feeling. There was no muscle soreness or beating, no dizziness and headache, no nausea and vomiting, no blurred vision, no difficulty in swallowing and drinking water, and no incontinence. The symptoms of numbness and weakness gradually progressed to the hands, the hands could not bear weight, and the symptoms gradually progressed. For nearly 2 days, the lower extremity weakness symptoms were significantly worse than before, and she was unable to stand firmly alone or squat. She was then admitted to our hospital. Her diet, sleep quality, urination and defecation were normal. Weight loss was 4 kg in the past 2 months. There were no bladder or bowel complaints, and no involuntary movements were noticed. There was no history of dog bite or vaccination. There was no past history of any illness, and she had not had any previous episodes of weakness. The patient did not have any addictions and gave no history of high-risk sexual behavior. Her menstrual history was noncontributory. She was married and had 2 children. There was no history of abortions or stillbirths.\nPhysical examination at admission revealed that she had a temperature of 36.5°C, heart rate of 105 bpm, respiratory rate of 20 breaths/min, blood pressure of 135/90 mm Hg and oxygen saturation of 98% in room air. She had no malar rash, oral ulcer, alopecia, arthritis or muscle inflammation. Neurologic examination indicated that the muscle strength in the legs was of grade 4/5 proximally and distally bilaterally, and there was an absence of deep tendon reflex in both knees and ankles. Paresthesia was observed in the distal limbs, with glove and stocking distributions. Her sense of acupuncture analgesia and vibration at the distal extremities was decreased. The deep tendon reflexes were absent. The bilateral Babinski test was unremarkable. The Romberg sign and heel-knee-tibia test were unstable.\nLaboratory data revealed leukopenia 3.16×109/L and anemia with hemoglobin at 112 g/L, respectively. Platelets were within the normal limits. The liver function panel results were also within normal limits, except for albumin at 26.4 g/L and hyperlipidemia. Prealbumin was low at 30 g/L and BUN was at 5.32 mmol/. Creatinine was at 43 μmol/L. Urinalysis revealed protein of 3+, and blood tests revealed red blood cells (RBCs) of 13.1/HPF. The erythrocyte sedimentation rate (ESR) and C reactive protein (CRP) level were in the normal range. There was a positive ANA +1:1000, anti-dsDNA, antinucleosome, anti-SSA antibody and low levels of serum complement components (C3, C4). Anticardiolipin antibodies and antineutrophil cytoplasmic antibodies (ANCA) were negative. Serologic tests for HIV, hepatitis B/C, and syphilis were all negative. Cerebrospinal fluid examination revealed albumino-cytological dissociation (total protein: 1370 mg/L and white blood cell: 10×106/L). Head computed tomography (CT) revealed no abnormalities. Chest CT showed reduced cardiac cavity density and anemia. Abdominal CT showed that the liver and spleen were larger. A cardiac ultrasound showed mitral valve, mild tricuspid regurgitation. Electrocardiogram (ECG) revealed no obvious abnormalities. Electroneuromyography (ENMG) was highly suggestive of demyelinating polyradiculoneuropathy. The details are described below. Bilateral sural nerve sensory conduction was normal, and bilateral median nerve, ulnar nerve sensory conduction did not lead to a certain waveform, that is, a feeling of separation. Bilateral median nerve, ulnar nerve, and peroneal nerve terminal latency were significantly prolonged, with a slight slowdown in motor conduction velocity. Further, the bilateral ulnar nerve, peroneal nerve slower transmission through the head of the fibulain and the elbow, and the right peroneal nerve conduction block were suggestive of comprehensive demyelination damage. In patients with low motor transmission, a compound muscle action potential (CMAP) amplitude in the normal range compared with their peers and a low volatility do not rule out the possibility of secondary axonal damage. The latency of the detected F wave was significantly prolonged, and the incidence of the F wave in the lower extremities was significantly reduced, suggesting demyelination of the proximal nerve or nerve root. Needle electromechanical tips in some of the distal muscles showed a nerve predominant potential, which suggested that there was movement of fiber axon damage (Table ). A percutaneous renal biopsy was performed on the patient after hospitalization. The kidney biopsy specimen showed glomerular basement membrane mild diffuse thickening (WHO classification lupus nephritis, class V. Activity index: 1 point, chronic index: 0 points). Immunofluorescence examination showed a glomerular distribution along the mesangial area: IgA ++, IgG+ along the capillary, C3 + along the capillary wall, F +, IgM +, C1q +, Kappa +, Lamda +. HE and special staining showed a total of 14 glomeruli, glomerular capillary wall mild diffuse thickening, mesangial area without proliferation, mesangial cells 1–3/mesangial area, mesangial matrix without expansion, and endothelial cells without proliferation. Masson staining showed a small amount of hyperhidrosis protein deposition in the capillary wall. Tubular atrophy, part of the renal tubular epithelial cell swelling, granular degeneration, and no clear tube were observed. The renal interstitial area was without edema, fibrosis and lymphocyte and mononuclear cell infiltration. The interstitial small tube wall showed no thickening. Congo red stain was negative (−) (Fig. ). A final diagnosis of SLE with GBS was made. The patient was initially treated with intravenous pulses of methyl prednisolone 0.5 g daily for 5 days. Maintenance therapy with 48 mg methyl prednisolone once a day, combined with cyclophosphamide treatment 1 g which was provided once a month. |
A 20-year-old male patient was admitted to our neurology department in February 2015 with intermittent numbness and weakness of the limbs that had lasted for more than 10 years, and right leg weakness over 6 months.\nTen years ago, he developed right lower extremity weakness and foot drop while walking. The patient was given treatment of traditional Chinese medicine at a local hospital which had no obvious effect, and then the symptoms were slowly alleviated without further treatment. And later, he noticed many instances of numbness in his both upper extremities while holding his arms long time on the table or head on his arms, which could last for several days. Numbness could also occur in the both lower extremities while lying on the side. In August 2014, right leg weakness and numbness occurred again without apparent cause. The right leg weakness aggravated in the next months, which caused him to fall down the stairs once in December 2014. He was diagnosed with peripheral neuropathy in his local hospital and was given therapy of Vitamin B1, cobamamide, and traditional Chinese medicine. His symptoms were slightly improved but not completely alleviated, and then he was referred to our department.\nIn neurological examination, there was weakness (2/5) in his foot back muscle groups of right lower extremity. The other muscle groups were normal (5/5). Tendon reflexes were decreased in all the extremities. Hypalgesia in his right lateral leg and 1.5 left ulnaral fingers were noted. Bilateral finger-to-nose test and heel–knee–shin test were normal. The Babinski sign and meningeal irritation sign were negative.\nFamily history investigation revealed that the patient's 47-year-old father had a medical history of recurrent pressure induced numbness in the extremities. Decreased tendon reflexes were noted in the father's extremities.\nIn laboratory investigations, the complete blood count, stool routine examination, blood biochemical test, liver function test, blood lipid test, erythrocyte sedimentation rate, and coagulation function were normal. Immunologic laboratory tests found antinuclear antibodies and antiganglioside antibodies were negative, as well as serum immunofixation test and Bence–Jones protein urine test.\nMedical history investigation found that the polyuria polydipsia symptoms had occurred since 2 years ago, and the patient had lost 15-kg weight. Further investigation showed that his fasting blood glucose level was 14.59 mmol/L. Postprandial blood glucose level was 38.74 mmol/L. Glycosylated hemoglobin level was 14.0% (normal range: 4.5%–6.0%). Glucose urine test was ++++. Diabetes-related autoantibodies test found that insulinoma-associated antigen-2, glutamic acid decarboxylase autoantibodies, islet cell cytoplasmic autoantibodies, and insulin autoantibodies were all negative.\nPhysical examination revealed red papules covered with scales on his scalp, bilateral anterior legs, and right foot back. Auspitz sign was positive.\nThe patient and his father were examined with electrophysiological examinations, and the findings are shown in Table . Motor and sensory conduction studies of nerves of the upper and lower extremities disclosed slowing of motor and sensory conduction velocities (CVs) and prolonged distal motor latencies in all nerves, markedly reduced compound muscle action potential (CMAP) amplitudes, and reduced F-wave CV in the young patient, which were much more serious than in his father. Electromyography revealed profound demyelinating injuries and secondary axonal injuries in distal peripheral nerves and parts that were easy to be compressed, involving the facial and extremity nerves, especially in the right lower extremity.\nHistopathological examination of a biopsy from the patient's sural nerve showed sausage-like enlargements of the myelin sheath (tomaculae) and thinly remyelinated internodes (Fig. A). Transverse Epon sections demonstrated marked variation in myelin thickness and several large hypermyelinated axons (Fig. B and C). Electron microscopy confirmed that fiber enlargement was due to a markedly thickened myelin sheath (Fig. D).\nGenetic testing by multiplex ligation-dependent probe amplification demonstrated PMP22 heterozygous deletions in the patient and his father.\nThe patient was diagnosed as HNPP, with T2DM and psoriasis.\nThe young patient was treated with continuous subcutaneous insulin infusion and blood glucose monitoring and then transferred to oral acarbose therapy. The psoriatic lesions were treated with calcipotriol ointment. He was discharged on oral therapy of 200 mg/d acarbose, 30 mg/d dibazol, 30 mg/d coenzyme Q10, 30 mg/d vitamin B6, 30 mg/d vitamin B1, and 1500 μg/d mecobalamine. Blood glucose monitoring was recommended. In the follow-up, the right leg weakness was alleviated, and his gait was improved. |
A 65-year-old male, a farmer by occupation, with normal birth and developmental history presented with 30-year duration of nonprogressive postural and action tremors of the fingers. There was no ataxia of limb or stance. These tremors did not interfere with his tasks of daily living. Since past 5 years he started having myoclonic jerks, which were present both in limbs and axial musculature. The myoclonic jerks were more severe in early morning and were absent in sleep. Since past 1 year the myoclonic jerks increased in form that it occurred throughout the day and even persisted in sleep. The myoclonic jerks were stimulus sensitive, being provoked by bright light and loud sound. He had significantly increased intensity of myoclonic jerks on exposure to sunlight and television light. He also had recurrent episodes of tonic-clonic seizures, which were present in early morning on awakening. He was put on sodium valproate (1 g/day in divided doses) for this, and his seizures were well controlled. Over past 1 year he had cognitive decline in form of executive dysfunction and recent memory loss. General physical examination was normal. There was no organomegaly. Nervous system examination revealed a mini-mental state examination (MMSE) score of 10/30 with impairment in registration and recall. Detailed tests for lobar function revealed involvement of frontal and temporal lobes in form of executive dysfunction and impairment in memory. The cranial nerve examination and optic fundus was normal. Power was 4/5 at all joints. Sensory examination was normal. Action and postural tremors were present in both upper limbs more distally, but there were no intention tremors. Myoclonus of the upper limbs and axial musculature was present that was provoked by exposure to torch light and loud clap. Hence, it was a case of autosomal dominant postural and action tremors, stimulus-sensitive myoclonus, epilepsy in form of tonic-clonic seizures, and cognitive decline. On the basis of the above clinical picture and investigations, the following possibilities were considered:\nAutosomal dominant cortical tremor, myoclonus, and epilepsy (ADCME) Progressive myoclonic epilepsy\nElectroencephalogram (EEG) [] of Case 1 showed paroxysm of generalized sharp and slow wave complexes. Intermittent photic stimulation (IPS) at frequencies between 10-25 Hz triggered generalized spike and wave complexes. Magnetic resonance imaging (MRI) brain [] was normal. The thyroid profile, vitamin B12 levels, and renal and liver function tests were normal. Computed tomography (CT) chest and abdomen was normal. |
A 69-year-old man with a history of hypertension, bilateral thalamic hemorrhage, and decreased kidney function was admitted to our hospital because of congestive heart failure with extracellular volume overload. Volume control with drugs, such as diuretics, was unsuccessful and he was started on hemodialysis. Thereafter, he received various antibiotics for bacterial infections, including pneumoniae and urinary-tract and catheter-related infections. Simultaneously, he had continuous watery and sometimes bloody diarrhea, the etiology of which was considered to be CDAD due to a positive stool test for CD toxins. Despite treatment with metronidazole and vancomycin, the severity of the diarrhea was not ameliorated. Abdominal X-ray and computed tomography did not reveal any causal factors, and laboratory tests showed only slight elevations in the white blood cell count and C-reactive protein level. We suspected involvement of uncontrollable CDAD or other type of infection (such as mycosis, tuberculosis, or cytomegalovirus infection) and inflammatory bowel disease (IBD).\nColonoscopy showed multiple segmental ulcers in the ascending, transverse, and sigmoid colon, but not the rectum, which suggested infectious colitis or ischemic colitis (Fig. ). A biopsy specimen showed loss of glands, fibrosis, congestion, and edema, suggesting ischemia, with no findings of infection (Fig. ). Given these results and the fact that the patient was on dialysis and had severe arteriosclerosis, we provisionally diagnosed ischemic colitis caused by arteriosclerosis and hemodialysis. We stopped or adjusted enteral nutrition, avoided low blood pressure, and withdrew antibiotics; however, his diarrhea persisted. It was impractical to perform a second colonoscopy, as his performance status was severely worsened. Finally, he died of cardiac arrest 193 days after admission. The autopsy showed longitudinal and annular ulcers in the sigmoid colon. An autopsy specimen of the ulcers showed inflammatory polyposis, cryptitis with crypt abscess formation, and focally severe lymphocyte infiltration near the muscularis mucosa; these findings are fully compatible with UC (Fig. ). We retrospectively suspected that the cause of the intractable diarrhea was UC, which had been masked by the clinical course and findings of CDAD and ischemic colitis. |
A 54-year-old female patient was admitted to our outpatient clinic with complaints of left hip pain markedly increasing for 10 days and difficulty in walking. She stated that she had hip pain for about 2 months, and she was treated by many physicians. Patient had difficulty in load transfer during walking and therefore had ambulation difficulty. She defined loss of appetite and sometimes fever. She did not define low back pain, radicular symptoms, neuropathic complaints, trauma, rash, aphtha, diarrhea, arthritis, abdominal pain, a recent infection, a history of intramuscular injection, weight loss, history of eating fresh cheese, and history of tuberculosis. Patient received chemotherapy and radiotherapy 3 years ago due to cervical cancer, and she was a diabetes patient using insulin.\nIn patient's evaluation, her arterial blood pressure measurement was 110/70 mmHg, her fever was 37.1°C, and her pulse rate was 95/minute. Patient appeared to be pale and tired, and she was mobilized on wheelchair. Due to pain, she could not bear load on left lower extremity, she needed assistance in walking, and she had difficulty in transfer activities. Left hip joint was flexed to 50 degrees. Passive joint space could not be evaluated. She defined tenderness at trochanter major by palpation. Right hip and vertebral movements were extended and painless. Spasm was detected in left paravertebral muscles at lumbar region. No neurological deficit was detected, and no pathological reflex was present. She had no edema in lower extremities, and all pulses could be measured.\nAfter initial evaluation, lumbar, pelvis, and left hip roentgenogram and ultrasonography (US) for hip joint and gluteal region were planned. Roentgenograms were normal except mild osteodegenerative findings at lumbar vertebra. Slight amount of fluid was observed at left trochanteric bursa in US. Hip magnetic resonance examination (MR) was requested for clear evaluation of hip joint and adjacent structures. However, it could not be performed since the patient could not be positioned due to pain. In her laboratory examination, the following was detected: leucocytes: 17.86 (4–10) k/uL, hemoglobin: 7.3 (11–16) g/dL, sedimentation (ESR): 110 mm/hour, creatinine: 1.56 (0.6–1.1) mg/dL, C-reactive protein (CRP): 31.18 (0.01–0.82) mg/dL, and brucella agglutination: negative. In her urinalysis, protein (+++) and leucocytes 1500 p/HPF were found. Patient had history of radiotherapy and chemotherapy due to cervical cancer, and urgent nonopaque abdominal computerized tomography (CT) was performed for the patient with current findings and prediagnosis of metastasis and abscess. Soft tissue lesion was observed consistent with abscess at left iliac fossa in iliopsoas muscle, filling paravertebral region with lucent gas inside (Figures and ). PA was detected and she was hospitalized in urology clinic for treatment. Blood culture was negative and, following preferred metronidazole and piperacillin/tazobactam treatment, patient had an operation. PA, ureter, and sigmoid colon perforation were detected. In pathological examination of nephroureterectomy and sigmoid colon resection material, ureter and sigmoid colon metastasis of squamous cell carcinoma were detected. |
An 81-year-old Japanese man was diagnosed with BD in 1981 on the basis of recurrent oral and genital ulcers, erythema nodosum, and uveitis, meeting the classification criteria for BD established by the Japanese Ministry of Health and Welfare. He lost eyesight in his left eye in 1992, due to recurrent uveitis. He was referred to our hospital in 2007 with recurrent attacks of inflammation affecting the right eye. He had been treated with betamethasone eyedrops, but had never been treated with colchicine or cyclosporine A before the first visit to our hospital. As for initial ophthalmic presentation at our hospital, visual acuity in the right eye was at the level of recognizing hand motion, and keratic precipitate, anterior chamber cells (++), flare (++), and grade 3 vitreous haze were identified. The left eye showed no inflammation, and light perception on electroretinography was unrecordable.\nThe patient was initially treated with colchicine and betamethasone eyedrops, but attacks of ocular inflammation persisted, occurring six times in 8 months (equivalent to nine times/year). Immunosuppressive agents, such as cyclosporine A, were difficult to use because he had undergone removal of the left kidney due to cancer in 1998. In May 2008, administration of infliximab was started at a dose of 5 mg/kg, followed by a regular treatment schedule (initially in weeks 2 and 6, then every 8 weeks). This treatment reduced the frequency of ocular inflammatory attacks to seven times in 60 months (1.4 times/year). Right visual acuity improved to 1.0. However, the patient experienced exanthema after the ninth infusion of infliximab in May 2009 (54 weeks after starting infliximab treatment). That episode was considered to represent an infusion reaction to infliximab, so 5 mg of D-chlorpheniramine maleate and 20 mg of epinastine hydrochloride were administered before each infusion of infliximab thereafter.\nThe frequency of ocular attacks increased from December 2011 (3 years and 7 months after starting infliximab treatment), and oral ulcers and abdominal pain occurred in the same period. After 4 years and 8 months, intestinal lesions with bleeding were identified, and infliximab was judged inadequate to control intestinal lesions. Adalimumab was thus considered as an alternative treatment, and started as a substitute for infliximab. Adalimumab was administered at a dose of 80 mg the first time, with 40 mg administered every 2 weeks thereafter. Adalimumab was self-administered subcutaneously by the patient. Visual acuity prior to the initiation of adalimumab was 1.0. After switching to adalimumab, uveitis was well controlled, right visual acuity remained at 1.0, and ocular inflammatory attacks did not occur during 2 years of follow-up ().\nVitreous haze in the right eye had disappeared with the use of infliximab, and did not reappear after switching to adalimumab. Fluorescein angiography examination showed leakage from retinal vessels at the first examination, but this leakage was dramatically improved by infliximab, and the improvement was maintained on adalimumab (). Intestinal lesions were also well controlled. |
A 71-year-old man with osteoarthritis of the medial compartment of the left knee (varus knee) received a primary cemented UKA without complications in 2007. His postoperative course was uneventful until March 2017, when he felt that his knee was becoming increasingly unstable, making frequent clicking noises with pain and increasing disability. Since his symptoms were getting worse, the patient came for a clinical assessment in February 2018. At that time, he had severe pain, swelling and an irritating feeling of ‘metal on metal’ when the knee was moved. At the clinical evaluation, there was an effusion, but there weren’t any other signs of infection (there wasn’t any redness and the temperature was similar to the other knee), the range of motion (ROM) was limited (110° of flexion and – 10° of extension) by the swelling and the pain and there was a slight instability at the varus/valgus stress tests. Regarding the general examination, there wasn’t anything to report besides a BMI of 35. The patient never had any fever. Blood tests were performed: complete blood count and differential leucocyte count, VES and PCR; the results of these were normal.\nSince there were no local or systemic signs of infection, an aspiration was not performed.\nIn a CT scan performed in June 2017 () no signs of alteration were evident, but an X-Ray performed in January 2018 () showed a rupture in the UKA femoral component.\nRevision surgery was performed at the end of February 2018. At the time of revision surgery, we noticed a very clear synovitis associated with a massive metallosis. The tibial component was well fixed while the femoral component was broken (-).\nThere was a severe wear of the polyethylene tibial insert () that caused a minor friction between the femoral component and tibial metal surface with a huge abrasion of the metal femoral component. The periprosthetic tissue affected by the metallic debris was cleaned up and several swab samples were taken from the articular structures. Then a cemented medial pivot total knee arthroplasty was performed (revision Advance, MicroPort®) ().\nBacteriological samples of the fluid and surfaces, taken during the surgery from the joint, did not show any growth. Histological examination confirmed the presence of a massive metallosis with a large amount of opaque pigment in histiocytic cells.\nThe patient had a satisfactory rehabilitation from the revision surgery. After two months, the range of motion was optimal (flexion 130° and extension 0°), the knee was stable and the patient was able to walk without any support. |
A 45-year-old Caucasian woman, mentally disabled, living in an institution of special care, presented with an acute onset chocking with respiratory distress during her meal. The care-taker nurse had noticed that she had eaten a large piece of chicken meat. Instantaneously, the nurse performed Heimlich's maneuver on three separate occasions. Immediately after the maneuver, the acute respiratory distress partially resolved, though the patient developed subcutaneous emphysema extending from the thorax to the face closing the eyelids completely. The blood oxygen saturation was above adequate. A transnasal fibreoptic laryngoscopy showed salivary stasis in both piriform sinuses. There was no laryngeal edema and vocal cord mobility was conserved. An urgent cervicothoracic CT scan () done in the following hour revealed a three cm long foreign body of bone density located at the esophageal opening. In addition, massive subcutaneous emphysema was seen, cranially from the fat pad of Bichat extending posteriorly to the retropharyngeal space, the occipital region descending caudally to the axilla, and the mediastinum (Figures , , and ). There the lung parenchyma was normal and there was no pleural effusion. A rigid pharyngoesophagoscopy was done 6 hours later extracting a large piece of bony chicken meat which was impacted in the right piriform sinus. On repeated endoscopy a 3 mm tear was seen at the apex of the right piriform sinus extending until the cricopharynx. Because the size of the tear was small we decided against an endoscopic repair of the tear only inserting a nasogastric feeding tube under endoscopic control. The patient was covered with amoxyclavulanic acid 1.2 g three times a day.\nTwenty-four hours later, the patient redeveloped a progressive respiratory distress with increasing inflammatory parameters. The patient was febrile and had tachycardia (HR > 110/min). A new cervicothoracic CT scan was performed. It revealed a regression of the subcutaneous emphysema and the pneumomediastinum but showed evidence of bilateral pleural effusion and atelectasis. Bilateral intercostal drains were inserted in emergency. A new pharyngoesophagoscopy showed pus in the right piriform sinus. A right exploratory cervicotomy was performed to evacuate the abscess and showed no evidence of residual foreign body. The site was rinsed with dilute hydrogen peroxide and betadeneR and closed over 2 easy-flow drains. The wound was rinsed with dilute betadine solution 2 times a day for the next 3 days. Antibiotherapy (amoxicillin-clavulanate) was continued for 10 days. The thoracic tubes were pulled out at day 5. Over the next few days the inflammatory parameters settled and the general condition improved. The cervical drains were removed on the sixth day. A cervicothoracic CT scan performed on day 9 showed a complete resolution of the cervical pneumomediastinum, the pleural effusion, and the subcutaneous emphysema. A barium study done at 2 weeks was normal and the patient was restarted on feeds. |
A 33-year-old male was brought to emergency room of a large multispecialty hospital in Eastern India with chief complaints of fever for 7 days associated with nausea, vomiting, and abdominal pain. The initial blood parameters on admission showed platelets to be 35,000/mm3 and nonstructural protein 1 (NS1) IgG antigen to be positive suggesting a serological diagnosis of dengue fever. He was started on supportive therapy which included intravenous colloids, broad spectrum intravenous antibiotics, and platelet transfusions (4 units of 50 ml).\nOn the 3rd day of hospitalization, the patient developed pain, redness, and proptosis in the right eye for which ophthalmic opinion was sought from a tertiary eye care center in Eastern India. On bedside ophthalmic examination, the patient was partially conscious, not oriented to time, place, or person; hence, visual acuity and ocular movements could not be elicited. Right eye showed lid edema with conjunctival chemosis and congestion, corneal edema, exposure keratopathy inferiorly, and raised finger tension []. Anterior and posterior segments had no view due to corneal edema. The left eye was within normal limits. The patient was started on intensive topical antibiotics along with broad-spectrum intravenous antibiotics (vancomycin, metronidazole, and meropenem) according to the internist discretion. Ophthalmic ultrasound facility was not available in that center. Magnetic resonance imaging brain with orbit showed multiple basal ganglia infarcts with hyperintensity and thickening of right ocular coats with hazy vitreous and periocular extension of the inflammation, which pointed toward the diagnosis of acute dengue encephalitis with panophthalmitis [Fig. and ].\nAs there was no clinical improvement within next 24 h, the patient was advised and underwent evisceration of the right eye. Eviscerated sample was sent for Gram staining, KOH staining, bacterial culture, and fungal culture and was tested for dengue virus genome. The result of eviscerated sample came out to be positive for bacilli in clusters and culture showed growth of Bacillus cereus. The IgG blood serum was positive for dengue, but polymerase chain reaction (PCR) of the eviscerated sample was negative for dengue genome.\nPostoperative period was uneventful with a healthy socket []. No complications of evisceration were noted. The systemic condition rapidly improved 1 week postoperative, with rise in platelet counts to 1.74 lakh/mm3. |
Our patient was a 47-year-old male with past medical history including aorto-occlusive disease status post femoral-popliteal bypass, with peripheral artery disease, coronary artery disease, and tobacco dependence. He initially presented with right groin and lower-extremity numbness with an otherwise unremarkable review of systems. The patient was diagnosed with right limb occlusion with critical limb ischemia of the right lower extremity due to an aorto-femoral bypass graft occlusion. Initial workup included a computed tomography (CT) angiogram of the chest, prior to treatment of the occlusion with a femoral-femoral bypass.\nComputed tomography angiography (CTA) of the chest revealed a 1.4 cm nodule at the left lung apex, slightly cavitary in nature together with a left paratracheal soft tissue density that was suspected to be adenopathy related to pneumonia that was being treated. The lesion was considered to be incidental with the recommendation of short-term follow-up with another chest CT in three months. There was no prior imaging for comparison.\nTwo months later, the patient presented to the emergency room with bilateral chest pain and associated shortness of breath and dyspnea. He was admitted to the intensive care unit (ICU) for respiratory instability and treated for multiple bilateral pulmonary embolisms. The diagnosing CTA of the chest showed an increase in the left upper lobe mass density with 2.5 cm x 2.4 cm dimensions including marked interval increase in diffuse mediastinal and bilateral hilar adenopathy involving levels T5, T10, and T11, suggesting a primary neoplasm with metastatic disease. The primary lesion was pleural based and thought to be invading the pleura. Once the patient stabilized, a CT-guided left upper lobe biopsy was obtained.\nBiopsy revealed a poorly differentiated non-small cell carcinoma consistent with squamous cell carcinoma. Sections showed nests and individual large cells with brisk mitotic activity with medium to large nuclei. There was considerable tumor necrosis. Immunohistochemical stains showed positive staining for p63 and negative for TTF1. Morphology and stains were consistent with squamous cell carcinoma of the lung. It was suggested that the pulmonary embolisms the patient experienced were attributed to a hypercoagulable state related to malignancy.\nOncologic positron emission tomography (PET)/CT scan suggested invasion of the pleura with perivascular and lymphatic metastatic involvement, confirming a hypermetabolic left upper lobe mass of 2.5 cm x 2.8 cm with SUV of 10.7 (Figures -) and hypermetabolic left hilar adenopathy (Figures -). Subsequently, a magnetic resonance imaging (MRI) of the brain was completed for evaluation of metastasis. A 6 mm ring-enhancing metastatic lesion was noted in the left frontal lobe with surrounding edema (Figures -). The imaging was otherwise unremarkable.\nThe initial treatment plan included a radiosurgery approach to the solitary brain lesion. The lesion was treated with five volumetric arc therapy (VMAT) beams with the isocenter located in the center of the lesion, which was contoured on the MRI images. A total peripheral dose of 2500 cGy delivered in five fractions was prescribed to the planning target volume (PTV). Dose-volume histogram (DVH) analysis of the target lesions showed dose statistics (minimum, maximum, and mean) of 2396.0 cGy, 2941.2 cGy, and 2759.3 cGy to the PTV, respectively. The total volume of the gross lesion and margins was 0.4 cc. The total volume of tissue receiving 100% of the total prescribed dose (V100%) was 0.5 cc. The patient did not receive any form of systemic therapy, such as chemotherapy or immunotherapy.\nPost-treatment MRI of the brain at one-month follow-up after the initial brain lesion diagnosis showed a reduction in size from the original 6 mm nodule (Figures -) on the stealth protocol study. Remarkably, repeat PET/CT four weeks post-treatment stereotactic radiosurgery treatment (SRS) revealed no appreciable mass in the left upper lobe (Figures -) with resolution of hypermetabolism. Fluorodeoxyglucose (FDG) activity in the left hilum gave SUV level 2.2-2.7, an improvement from the previous SUV of 9.3 (Figures -). Chest CT confirmed that there was no longer an appreciable left upper lobe mass. An additional follow-up chest CT was completed two months later (three months post-treatment) confirming complete resolution of the original left upper lobe pleural-based mass.\nThe most recent imaging at seven months post-treatment continued to remain free of residual left upper lobe and mediastinal masses on chest CT. Brain MRI showed complete resolution of the solitary metastatic focus in the left frontal region. There was no evidence of new metastatic disease. |
An 82-year-old female patient with a symptomatic gallstones disease and a recent weight loss was admitted to our hospital. The patient’s past medical history was free of other diseases and on physical examination, a Murphy sign was present. The abdominal ultrasound mentioned a large gallstone in the gallbladder and a hypoechoic liver mass. Liver blood tests, including tumor markers CEA and CA 19-9 were normal. Magnetic resonance imaging-magnetic resonance cholangiopancreatography (MRI-MRCP) revealed a liver tumor mass (4.5x3.5 cm) located mainly in segments IVa and VIII of the liver with an extent to segment I (Figure ).\nThe tumor displaced the adjacent hepatic veins and the inferior vena cava (IVC) without any signs of vessel invasion. There were no signs of liver cirrhosis and no dilated bile ducts or capsular retraction were noted. There was no associated lymphadenopathy. At this point, imaging characteristics were controversial regarding diagnosis. The differential diagnosis tilted in favor of ICC, mainly due to the enhancement characteristics and the absence of liver cirrhosis, as seen in Figure .\nThe patient was scheduled for exploratory laparotomy with a provisional diagnosis of an ICC. Intraoperatively, a cholecystectomy and lymph node sampling from the hepatoduodenal ligament were performed and both specimens were negative for malignancy on frozen section. Next, the liver was mobilized and the tumor was carefully dissected free of the hepatic veins, the IVC, and the rest of the liver parenchyma. The gross morphology of the liver specimen revealed a solid, grey-yellow liver lesion with a soft consistency. In the center, a light yellow region was noted, as can be seen in Figure .\nThe frozen section was negative for malignancy. Histologically, the tumor was characterized by a heavy inflammatory infiltrate in myxoid collagen stroma, consisting primarily of plasma cells, lymphocytes, and eosinophils. Fibroblast cells without significant fibrosis composed the stroma. There was no evidence of malignancy in the tissue examined. The final pathology report revealed an IPT of the liver. |
A 23-year-old female patient arrived at the Emergency Department trauma bay following an MVC. She was the restrained driver when she was rear-ended by a truck and slammed forward into a guardrail. Patient was unresponsive at the scene for paramedics and required extrication from the vehicle. Upon arrival in the trauma bay, patient was awake and responsive, repeatedly crying out and answering, “I do not know” and “ok” to all questions. GCS was 14 and no injuries were immediately noted. She was mildly tachycardic with a pulse of 102; her other vital signs were normal. The patient was unable to provide a medical history, but her phone had a message on it stating that she had holocarboxylase synthetase deficiency. When parents arrived, they confirmed this and stated that she had a history of Asperger's syndrome.\nCT scan of the patient's cervical spine was performed with contrast, and she was found to have bilateral internal carotid and vertebral artery dissections without signs of bony injury. Subsequent MRI of the brain showed acute infarcts of bilateral cerebral hemispheres, greater on the left than on the right, and a right-sided cerebellar infarct, without signs of cervical spine ligamentous injury. CT angiography showed dissection of the left internal carotid starting at the level of C2, dissection of the right internal carotid originating at the bifurcation, and bilateral vertebral dissection at the level of C2-C3, with significant thrombosis present in the vertebral arteries.\nThe patient developed worsening mental status and became unresponsive to stimuli, though she was able to maintain her airway. She would still intermittently spontaneously move her left side but had little to no movement of the right upper and lower extremities and had a leftward deviated gaze. Heparin drip and aspirin were started. Patient was not considered a candidate for endovascular intervention since she had no focal areas that could be addressed operatively. By the subsequent day, patient had developed a significant lactic acidosis, with a bicarbonate of 8 mmol/L, an anion gap of 26 mmol/L, and a lactate of 6.2 mmol/L. This was likely secondary to the patient's underlying genetic metabolic disorder. Patient was started on a bicarbonate drip. Neurology was consulted and a continuous EEG was obtained, which showed areas of decreased activity without signs of seizure. A nasointestinal tube was placed and the patient's home regimen of biotin and carnitine was able to be resumed. The lactic acidosis was corrected, and the bicarb drip was stopped.\nOn day 3, patient was able to transition out of the ICU, though she was still not responding to commands or interacting in any way. Two days later she was transitioned from the heparin drip to an enoxaparin bridge to warfarin. Serial CT scans of the head revealed evolving infarct without signs of hemorrhagic conversion. By day 6, the patient's neurologic status was slightly improving. She was able to answer yes/no questions and state her name. On the day of discharge two days later, repeat CT angio of the head and neck showed improvement of the vascular function and the patient was able to follow simple commands and walk during physical therapy. She was discharged to acute rehab on warfarin, biotin, and carnitine. |
Our patient is a 16-year-old male who presented to the neurosurgery clinic with a 1-year history of progressive left scalp swelling and tinnitus. He had a history of trauma 7 years prior resulting in a left 2.5 cm temporal epidural hematoma [] which required evacuation [] and a left posterior petrous temporal bone fracture. There was no scalp swelling in the immediate postoperative period. Seven days after the trauma, he presented to the emergency department with drowsiness, left cranial nerve VI palsy, and papilledema. He was found to have a left sigmoid, jugular bulb, and internal jugular venous thrombosis likely secondary to the overlying skull fracture. Thus, the patient was treated with 4 months of Lovenox® (enoxaparin) and 1 month of Diamox (acetazolamide).\nOn presentation, our patient had an intact neurological examination including cranial nerve and motor and sensory systems. There was no papilledema. Fullness was noted over the left forehead and temporal area. A tortuous vein was seen over the lateral aspect of the left eyebrow [ and ]. A large dilated compressible area over the left temporalis incisional flap measuring 7–8 mm was noted. A loud bruit was present over this area on auscultation and a thrill on palpation. No chemosis or proptosis was noted.\nA computed tomography angiography (CTA) of the head was obtained that demonstrated numerous dilated scalp veins overlaying the temporalis muscle and extending into the face [ and ]. A four-vessel angiogram was performed and showed a scalp AV fistula supplied by the deep temporal arteries with collateral flow to the fistula from the superficial temporal artery and the ophthalmic artery. There was no evidence of intracranial venous drainage but there was significant hypertrophy of the deep temporal arteries supplying the fistula to a large venous pouch on the patient’s scalp [].\nThe AV fistula was embolized using a total of eight Penumbra smart coils® (Penumbra, Inc.; Alameda, CA). Four coils were deployed inside of venous pouch. One traveled distally into the venous pouch and three landed proximally. After deployment of these coils, there was good distal occlusion in the venous pouch. The microcatheter was then pulled back slightly until it landed at the fistula site. An additional four coils were deployed at this site. Follow-up angiogram showed no residual filling of the fistula [].\nThe postoperative course was unremarkable without complications from the coil embolization procedure. The engorged facial veins were no longer visible. At 1-month follow-up, the patient denied any tinnitus, headaches, blurry, or double vision. No engorged facial veins were visible at the time. A 6-month postoperative angiogram was performed that demonstrated successful obliteration of the scalp AV fistula []. The deep temporal and superficial temporal arteries were no longer feeding the fistula. |
A 75-year-old married female presented to the inpatient unit with a history suggestive of severe depression with psychotic symptoms for the last 3 months. Evaluation of the history revealed that the patient had been suffering from acid peptic disease for 20 years. About 4 months before presentation, the patient developed myocardial infarction and underwent percutaneous coronary angioplasty. Within a month of myocardial infarction, the patient's gastrointestinal symptoms increased and she started to remain sad, developed anhedonia, poor interaction, poor attention and concentration, low self-esteem, decreased sleep, and appetite, and had weight loss. She would be preoccupied with the abdominal complaints and report of something moving in her abdomen. She was taken to physicians, and whenever she would be examined, she would not allow the family members to accompany her, which family members found to be odd. When asked, she would say that she was too embarrassed to tell them the truth. On insistence of the family members, later, she disclosed that she was pregnant and was able to feel the movements of the baby in her abdomen. When asked further, she disclosed that she has been pregnant for 19 years. When family members tried to reassure her or reason with her, the patient would not be convinced and would express ideas of guilt. The patient was taken to a gynecologist, and even after examination, she was not convinced about lack of pregnancy. Following this, she was referred for psychiatric evaluation. There was no history suggestive of other depressive cognitions, first rank symptoms, free-floating anxiety, phobias, seizure, head injury, hypothyroidism, and substance use.\nHer past history revealed evidence of a moderate depressive episode without somatic symptoms (as per the ICD-10) 10 years back, lasting for 6 months. Family history was not contributory.\nPhysical examination of the patient did not reveal any abnormality. Her body mass index was 22 kg/m2, and there was no evidence of any objective signs compatible with pregnancy. On mental status examination, she was found to have low mood, psychomotor retardation, ideas of guilt, and DOP. She disclosed that the fetus is present for the last 19 years and she is able to perceive the movements since then. Despite providing her scientific reasoning, she could not be convinced against the presence of pregnancy and it lasting for 19 years. Her mini mental status examination score was 27.\nRoutine investigations in the form of hemogram, renal function test, liver function test, serum electrolytes, and thyroid function test did not reveal any abnormality. Magnetic resonance imaging of the brain revealed mild cerebral atrophy with small vessel ischemic change, small (0.5 cm × 0.5 cm) right temporal convexity meningioma. Her Beck depression inventory rating at the initial evaluation was 30.\nBased on the available information, a diagnosis of recurrent depressive disorder, current episode severe with psychotic symptoms (F33.3), was considered. A possibility of independent persistent delusional disorder was also considered. Initially, she was managed with tablet sertraline 50–100 mg/day and tablet olanzapine 5–10 mg/day and was considered for electroconvulsive therapy (ECT) in view of marked distress in the patient. After detailed cardiology evaluation (electrocardiography, echocardiography, and stress scintigraphy – all of which were found to be within normal limit), she was started on ECT. She received six effective bitemporal modified ECTs, with which she showed significant improvement in her depressive symptoms. However, her DOP continued. In view of the same, independent diagnosis of persistent delusional disorder was confirmed. The delusional belief continues to remain as such, despite being on antipsychotics for 2 years, making the total duration of DOP to be 21 years. |
An 11-year-old boy visited genetic clinic with a chief complaint of missing teeth since childhood and facial dysmorphism. He was first in birth order of nonconsanguineous couple, delivered at full term with uneventful prenatal and perinatal period. Mother noted inability to open mouth at birth. He was found to have ankylosis (fusion) of bilateral TMJ with posterior cleft palate on oral examination. He was operated by fusion release of the band of right and left TMJ ankylosis with tracheostomy at 7 months of age. Cleft palate was partially repaired and is persistent yet. Postoperative spiral computed tomography scan of the face and neck at 7 month of age showed posterior cleft palate [Figure and ]. History of present illness revealed that teeth were congenitally missing. There was a history of several episodes of high fever during infancy. Patient's mother noted that he remained warm, especially during the summer and was not able to sweat adequately. He had motor developmental delay as started walking at 2.5 years. The mental development was normal for his age. On examination, at 11 years of age, his weight and height were plotted <3 standard deviation for his age. His skin was dry and scaly. Hair examination revealed fine lusterless appearance with scanty body hair. Facial features revealed sparse scalp hair, frontal bossing, scanty eyebrows and eyelashes, hyperpigmentation around the eyes, depressed nasal bridge, prominent lips, bilateral low set ears []. Eye examination showed dryness of the cornea and decreased tearing. Intraoral examination revealed two peg-shaped lower canine and two first upper molar teeth, the other were missing, dry mucous membrane, bifid tongue and posterior cleft palate []. The color of alveolar mucosa and gingiva was normal. Vertical dimension of the lower face is reduced, and vermilion border was not present, giving senile look to the child as compared with children of his age. Hands and feet examination were normal. The nails were thin and brittle. None of the family members has a similar history in previous generations. The routine biochemical tests were within normal limits. Skeletal survey was normal for the age. Echocardiography and ultrasound abdomen were normal. Orthopantomogram revealed malformed incisors, impacted maxillary and mandibular teeth, and true partial anodontia []. Based on the history, oral and cutaneous examination, diagnosis of HED was made. Mutation analysis was not possible due to financial constringent. Patient was evaluated by dentist and planned for removable partial denture after reshaping the existing teeth with composite. As the patient was growing, parents were instructed to defer the placement of the implant until after the completion of jaw growth and eruption of other permanent teeth. Parents were also educated regarding periodic follow-up for modification and replacement. They did not follow for the treatment. |
In August 2010, a 49-year-old male working as a dental radiographer, lost his distal phalanges of both thumbs as a result of development of squamous cell carcinoma, due to neglect of the guidelines of protection related to X-ray imaging.\nHe was appointed in 1994 by the Radiology Division, primarily as an in-charge clerk within the Oral Medicine Department (Faculty of Dentistry, Damascus University). As a school graduate with an intermediate certificate, his main task was merely to monitor the radiographic equipment.\nGiven that there was only one radiographic technician at that time, it was decided in 1995 to train him on how to use the dental X-ray machines and how to radiograph patients. He was warned about the radiation risks and thoroughly educated on the appropriate protection standards. He was somewhat committed to wearing the lead apron and the dosimeter. On the other hand, he was accustomed to handling the periapical films with his thumb fingers.\nIn 2003, he noticed a very small ulcer at the tip of his right thumb. Although it was asymptomatic, he considered it as a traumatic ulcer and hence willingly neglected it for a period, which under normal circumstances would be considerably enough for such an ulcer to heal. Subsequently he then experimented with various courses of local and systemic antibiotic, but the ulcer did not show any signs of healing. Based on his judgment, it was asymptomatic and unprogressive, and accordingly he perceived to be a normal exfoliation, the result of his long-term handling of chemical processing solutions.\nIn August 2010 (after 15 years of handling dental X-ray), he noticed a discharge oozing from his right thumb. By that time, he also took notice of a similar ulcer in his left thumb. He consulted the professors in the department who in turn referred him to a general surgeon. On plane hand radiographs, an erosion of the distal phalanges of both thumbs was clearly evident [Figures and ]. Consequently, the surgeon planned to excise the discharging ulcerative lesion of the right thumb and to submit it for histopathological diagnosis, which clearly revealed squamous cell carcinoma Grade II []. Based on such a diagnosis, the surgeon decided to excise the distal phalanges of both thumbs with tumor free-margins []. Out of ethical consideration, the radiographer was well-informed about the intent to publish his case and hereby he signed an informed consent. |
A 27-year-old nullipara presented to her local hospital at 33 weeks' gestation with decreased fetal movement, uterine contractions, and possible leakage of fluid from the vagina. Testing confirmed rupture of membranes; therefore, the patient was started on antibiotics to increase latency and was given betamethasone to hasten fetal lung maturity. An ultrasound at the community hospital showed polyhydramnios and a fetal double bubble sign consistent with duodenal atresia. The mother was transferred to Penn State Milton S Hershey Medical Center for anticipation of preterm delivery in a fetus that would require postnatal surgery.\nAfter transfer, fetal monitoring showed normal fetal heart rate variability with accelerations and occasional decelerations related to contractions. An ultrasound confirmed the double bubble sign and polyhydramnios. Blood was observed in real-time swirling into the amniotic fluid from the umbilical cord (). The patient was taken to the operating room and an emergent cesarean section was performed. The amniotic fluid was grossly bloody. A viable male infant weighing 2295 grams was delivered with Apgar scores of 7 and 8 at one and five minutes, respectively.\nThe umbilical cord overall length was 29.5 cm and there were 13, 0.5- 1 cm exposed segments of the umbilical arteries spiraling along the length of the cord (). A discrete area from which the hemorrhage emanated was not identified. The placental disk was of normal weight and appeared grossly normal. Histopathologic examination of the cord showed absence of Wharton's jelly covering the umbilical artery, extreme attenuation of the media in the portion of the vessel exposed to the amniotic fluid, and degeneration of the overlying amnion (Figures and ).\nThe infant had no stigmata of Down syndrome. He appeared pale at birth and an initial hematocrit was 29.4% that was treated by a blood transfusion upon admission to the neonatal intensive care unit. Postnatally the abdominal X-ray revealed air in the stomach and first portion of the duodenum with the remainder of the abdomen appearing gasless, consistent with duodenal atresia. A nasogastric tube was placed and 37 ml of bloody secretions were suctioned. The infant underwent laparotomy on the second day of life where an atresia was noted in the third portion of the duodenum and duodenojejunostomy was performed. He did well postoperatively and was discharged on day 30 of life. |
We report on a 30-year-old female patient who presented to the Ear, Nose and Throat (ENT) clinic complaining of neck swelling, progressive dysphagia of 7 months duration, headaches, dyspnea primarily during the evening and stridor. The patient had a history of allergic rhinitis. A clinical exam revealed a swelling process in the oropharyngeal region behind the left anterior pillar distorting the palatal area and producing a significant elongation of the isthmus. A CT was performed revealing a large expansive cervical process on the left retropharyngeal region. It was ovoid in shape, heterogeneous and displaying moderate enhancement following intravenous contrast injection (fig. , fig. ). The medial border of the mass crossed the middle line into the right side involving the left carotid space and pterigoid muscles (fig. ). It reached the base of the skull and the left arch of C1. Due to its size, existent ipsilateral lymphadenopathies with hypodense centers in the II, III and IV ganglionar levels as large as 20 mm were present.\nIn addition, MRI was performed, showing an expansive process partially delimited and possibly encapsulated (fig. , fig. ) with heterogeneous enhancement following intravenous contrast administration (fig. ). The presence of some weak signals was suggestive of vascular structures within the mass (fig. ). The mass measured approximately 4.8 × 2.6 × 4.1 cm in size with a weak left paravertebral enhancement around the ipsilateral vertebral artery at the C1 level. A biopsy revealed an undifferentiated sarcoma (fig. , fig. ). Due to respiratory difficulty, a tracheostomy was performed.\nThe case was presented to the Head and Neck Tumor Board, where it was staged as an T3N2BM0 oropharyngeal tumor. Since the tumor was inoperable, chemotherapy followed by radiation therapy was recommended as the treatment of choice (fig. ). After 6 months of treatment, the tumor was stable but persisted without evidence of progression. Consequently, the patient was enrolled in a pain management and palliative care program. The patient is currently being followed. |
A 47-year-old medically complex male with myasthenia gravis on long-term systemic corticosteroids, diabetes mellitus, hypertension, dyslipidemia, chronic kidney disease, gout, and obstructive sleep apnea presented to the dermatology clinic with painful undermined ulcers on the right lower leg. About 1 year prior to these ulcers developing, he had traumatized this area on his leg after bumping into a metal rod. Although this wound healed, within about 4 months, he subsequently developed several painful, rapidly expanding violaceous ulcers with undermined borders to the lower leg. PG was suspected based on morphology and history and was supported by results of a 4 mm punch biopsy, which revealed mixed dermal infiltrate of predominantly neutrophils, with overlying dermal fibrosis at the deep edge of the dermal biopsy. Workup revealed no evidence of inflammatory bowel disease, no features or serology consistent with autoimmune arthritis, and no other known associations with PG. However, he was found to have a monoclonal gammopathy of unknown significance (with a normal bone marrow biopsy), hepatic steatosis, and was anti-HBC positive, hepatitis B core antibody reactive, though HBsAg was negative and hepatitis B surface antibody was protective. It should be noted that the PG developed while he was on high-dose prednisone for his myasthenia gravis. His PG was initially treated with topical clobetasol 0.05% ointment and intralesional corticosteroid injections with limited success. For his medical comorbidities, intravenous immunoglobulin (IVIg; 100 g/day for two consecutive days per month) was initiated in July 2017 and mycophenolate mofetil (1 g twice daily) was started in October 2017, and both treatments were maintained to the end of the case report period. Ustekinumab was initiated in December 2017 according to the IBD dosing protocol with a 520-mg IV infusion at week 0, and 90-mg subcutaneous injections at week 8 and then every 8 weeks thereafter. On treatment with ustekinumab over a 6-month period, the patient experienced a dramatic improvement in the size of the ulcer, noting a decrease in size by approximately half. Before and after treatment with ustekinumab response is depicted in . |
A 27-year-old female patient with a known case of triple-negative breast cancer admitted to the emergency room complaining of documented fever 40°C at home which was relieved with an antipyretic. She was status post 4 cycles of neoadjuvant dose-dense AC regimen which consists of doxorubicin 60 mg/m2 and cyclophosphamide 600 mg/m2 administered every 14 days. She was prescribed primary prophylaxis filgrastim after all cycles. Physical examination was unremarkable apart from her fever. The patient was admitted to the hospital having febrile neutropenia with no focus of infection and started on empiric antibiotics and filgrastim. She had a low white blood cell (WBC) count with an absolute neutrophil count (ANC) of 1100 cells/microlitre on day 11 after cycle 4 despite being on appropriate filgrastim dose at 300 μg per day for 9 days. On the second day of her admission, she recovered from neutropenia but continued to have persistent high-grade fever for almost two weeks despite escalation of the antibiotics and addition of an antifungal agent. She underwent series of investigations to identify the cause of her unexplained fever. She had extensive blood and urine cultures after each spike of fever, which all came back negative. Computed tomography (CT) scan ruled out infectious focus and showed hepatosplenomegaly with multiple splenic hypodensities and minimal perisplenic fluid which did not appear in the baseline scan (Figures and ). Infectious disease team advised for a splenic biopsy which showed splenic infarction only with no evidence of bacterial, fungal, viral, or malignant involvement (). She also underwent an echocardiogram study, sinoscopy, and series of rheumatologic investigations that were normal. General surgery team was consulted and did not recommend any surgical intervention since the follow-up CT scan turned out negative for splenic abscesses with interval improvement in the previous splenic wedge-shaped hypodensities (). Eventually, the patient was discharged on oral antibiotics with infectious disease and oncology clinic follow-up after being afebrile and asymptomatic for more than 72 hours. |
A 79-year-old woman was admitted to the internal medicine ward with painful lesions on her lower left leg of recent onset. Her medical history included prosthetic aortic and mitral valves, chronic ischaemic stage 4 kidney disease, multifactorial anaemia (due to valvar leak, gastrointestinal loss and chronic kidney disease) and a recent diagnosis of oligoarthritis of unknown aetiology. She was under the care of cardiology, internal medicine and rheumatology clinicians.\nThe patient had been on warfarin for over a decade. She was also taking calcium carbonate supplements, receiving weekly intravenous iron supplements, and had been recently started on prednisolone 20 mg daily for the oligoarthritis.\nThe lesions were described as initially small violaceous nodules with diameter of 3 cm or less on the posterior side of the patient’s left leg. By the time of admission, they had progressed to typical stellate ulcers (). The initial differential diagnosis by a dermatologist was warfarin-related ulcers, vascular ulcers or calciphylaxis. Warfarin was suspended and replaced with low molecular weight heparin adjusted for renal function. A first skin biopsy was inconclusive.\nWithin 5 days the small nodules had developed into necrotic ulcers with eschars that covered the entire left calf. General surgery, palliative care and nutrition specialists were consulted. Pain control and wound management were essential as the necrotic bed was spreading and there was a risk of superimposed infection ( and ). Pain management was also challenging as the patient reacted with confusion and agitation to an opioid combination. Administration of a morphine bolus, transdermal fentanyl and oral gabapentin was effective.\nLaboratory findings were non-specific and did not suggest calciphylaxis. The patient’s anaemia worsened and gradual acute-on-chronic renal failure was observed. Parathyroid hormone (PTH) levels were normal, as were calcium and phosphorous levels throughout the patient’s admission. However, clinical suspicion of calciphylaxis was very high and a second biopsy was obtained. Histological findings suggested calciphylaxis almost 30 days after the patient was first admitted ( and ).\nDuring this time, general surgery clinicians were responsible for wound care and the patient underwent surgical and chemical debridement. Different types of dressings were applied but negative pressure wound therapy was unavailable. Nevertheless, superimposed infection developed despite this satisfactory care. For the first 10 days of her admission, the patient received empirical antibiotic therapy with ceftazidime, with treatment later escalated to ceftriaxone for 12 more days after the identification of Morganella morganii and Citrobacter freundii.\nAlthough a clean wound bed was achieved after 2 weeks, new lesions developed and the wound again became infected. The patient was started on imipenem and vancomycin. Newly obtained blood cultures revealed methicillin-resistant Staphylococcus aureus, Klebsiella pneumoniae and Pseudomonas aeruginosa. A few days later the patient died from refractory septic shock and multi-organ failure. |
CB is a 16-month-old, previously healthy, female that initially presented to her primary care physician two weeks prior to presenting to our orthopaedic clinic with limping and intermittent refusal to bear weight through the left leg. The mother of the patient denied any previous trauma or constitutional symptoms but did endorse foreign travel; they were living in Japan at the time of presentation to our department. The patient was current on all vaccinations.\nThe initial orthopaedic evaluation revealed a well-appearing, healthy child in no acute distress. The gait exam revealed that she refused to weight bear on the left lower extremity. The patient had very mild generalized tenderness in the left midfoot region; otherwise, no other area of tenderness was appreciated upon further examination of the lower extremities. She had full, painless range of motion of her hip, knee, and ankle joints. There was no erythema or swelling of the left foot; however, there was a mild effusion of the ankle. She was neurovascularly intact with normal reflexes.\nShe was afebrile, and vital signs were within normal parameters. Radiographs of the left lower extremity revealed no osseous abnormality (). Laboratory findings revealed a slightly elevated erythrocyte sedimentation rate of 34 mm/hr; otherwise, the white blood cell count (10,200 cells/μL), differential (45% segmented neutrophils, no bands), and C-reactive protein (<0.05 mg/dL) were normal []. An MRI of her left ankle showed an ankle joint effusion, a 16 mm fluid collection with a high T2 signal with surrounding bone marrow edema, and a low signal on T1 (Figures and ). The findings were consistent with a Brodie abscess with surrounding osteomyelitis and a possible septic ankle. Furthermore, there was rim enhancement with gadolinium contrast, making an abscess more likely than a tumor () [].\nThe diagnosis and treatment were discussed with the parents, and she was consented for surgery. An anteromedial incision was used to approach the talus. Normal-appearing synovial fluid was encountered and cultured. The talus was then drilled through a nonarticular region with a 2.0 mm drill under fluoroscopic guidance, aiming in a posterolateral trajectory, resulting in an egress of purulent fluid which was sent for Gram stain and culture (). The cavity was then thoroughly irrigated with saline, a Penrose drain was placed into the wound, and the incision was loosely closed around the drain. A long leg splint was applied to protect the soft tissues and prevent weight bearing. Infectious diseases was consulted, and the patient was admitted to the hospital for empiric intravenous antibiotics (Clindamycin) while awaiting aerobic/anaerobic, fungal, and acid fast bacilli cultures.\nGram stain of the purulent fluid revealed Gram-negative rods. Synovial fluid Gram stain and final cultures were negative, as were the fungal and acid fast bacilli cultures. On postoperative day (POD) #1, ceftriaxone was added to Clindamycin for Gram-negative coverage. On POD #2, aerobic and anaerobic cultures grew Morganella morganii; Clindamycin was discontinued, and the patient remained on ceftriaxone. Antibiotic sensitivities revealed resistance to ampicillin/sulbactam but sensitivity to cefepime, ciprofloxacin, gentamycin, and trimethoprim/sulfamethoxazole. On POD #4, she was transitioned to a three-week course of oral cefixime and discharged with close follow-up. She recovered well without any recurrence of symptoms at her scheduled postoperative visits (). |
A 39-year-old man presented with a 19-year history of bilateral, slowly progressive, painful swelling of the Achilles tendon and a 1-year history of hypesthesia over the right sole without any trauma. The patient had no history of cataract or epilepsy, although he did have chronic diarrhea and had attended a special school due to learning disabilities.\nPhysical examination found a huge ovoid mass in bilateral Achilles tendons (). The patient experienced no pain during rest or ankle motion. Muscle strength was normal, except for mild weakness in both ankles. A sensory test revealed that the patient had decreased sensation in both feet, especially to light touch. The deep tendon reflex was increased in both lower extremities. Babinski sign and ankle clonus were positive on both sides. Magnetic resonance imaging (MRI) of the ankle showed fusiform thickening of the Achilles tendon and an enlarged tibial nerve (). The patient was mentally alert and he scored 25 points in the Mini-Mental State Examination with a very low IQ of 52 points.\nUltrasonography showed diffusely enlarged peripheral nerves in the lower limbs, upper limbs, and C5–C7 nerve roots. We also performed ultrasonographic evaluation of the peripheral nerves in a normal male subject of similar age and anthropometry. Cross-sectional areas (CSA) of the peripheral nerves were measured as shown in . Significantly larger CSA values were found in the patient with CTX than in the normal subject, for the posterior tibial nerve behind the medial malleolus and for the median nerve at both the wrist and the elbow, like the other peripheral nerves ().\nNerve conduction studies showed decreased sensory conduction velocity of the ulnar nerve on both sides (right, 43.7 m/s; left, 46.0 m/s) and decreased motor conduction velocity of the peroneal nerve (right, 37 m/s; left, 38.2 m/s). The posterior tibial nerve somatosensory evoked potential test (SEP) showed no response, while the median nerve SEP was normal. Brain MRI revealed mild cerebellar atrophy. T2-weighted image and fluid-attenuated inversion recovery (FLAIR) images of the brain showed areas of slightly high signal intensity in the bilateral fronto-parieto-occipital periventricular white matter ().\nThe results of laboratory tests, including serum total cholesterol, low density lipoprotein cholesterol, triglyceride, human leukocyte antigen B27, C-reactive protein, rheumatoid factor, antinuclear antibodies, and thyroid function, were normal, with the exception of cholestanol, which was high at 21.85 µg/mL (normal, <3.71 µg/mL). Genetic analysis revealed two mutations in exon2 of the CYP27A1 gene: c.435G>T in the splice site and a missense mutation of c.379C>T.\nThe above findings allowed us to make the diagnosis of CTX in the patient. The patient was treated with comprehensive physical therapy and 750 mg/day of chenodeoxycholic acid. |
A medically fit and well 13-year 11-month-old male was referred to the oral and maxillofacial surgery department at Alder Hey Children’s Hospital in Liverpool in regard to an intra-oral swelling of the right palatal region (Fig. ). An incisional biopsy was initially reported as a pleomorphic adenoma of the premolar region. Subsequently, a CT scan showed no significant bony abnormality, and a wide local excision was carried out with the application of a surgical palatal dressing plate. Histopathology of this resected tissue appeared to show tumour of intermediate malignant grade at the base of the specimen.\nFurther investigations undertaken to stage the tumour included a repeat CT scan which presented no evidence of significant bony involvement or erosion. An MRI scan showed no significant asymmetry or signal abnormality in the region of the hard palate, and there was no evidence of loco-regional metastasis of this tumour.\nFollowing a discussion of the craniofacial multidisciplinary team and numerous paediatric pathologists, a diagnosis of intermediate-grade sarcoma of the oral mucosa and hard palate was re-affirmed. A partial right-sided maxillectomy was planned to gain adequate tumour clearance, and prior to surgery, the patient attended for dental impressions and counselling regarding the procedures involved, together with instructions regarding the obturator prosthesis.\nA low-level right-sided standard hemi-maxillectomy was carried out via an intra-oral approach with preservation of the pterygoid plates (December 2013). The anterior alveolar cut was undertaken through the right lateral incisor socket following the extraction of this tooth in order to maximise the bone support on the maxillary central incisor abutment tooth. The residual zygomatic body on the right side was exposed, and two 37-mm zygomatic oncology implants (Southern Implants Ltd, South Africa) (Fig. ) were placed with excellent stability, ensuring that the prosthetic heads were positioned beneath the body of the obturator prosthesis and in a useful position for retention of the obturator. The posterior aspect of the cavity was dressed using the buccal pad of fat and the right inferior turbinate removed to facilitate access to the defect for the obturator. An interim prosthetic obturator was fitted and relined with silicone putty material and retained by dental clasps and a single bone screw into the midline of the remaining palatal bone. Recovery from the procedure was uneventful, and the patient was discharged home the following day. Histopathology confirmed the diagnosis of myxoid spindle cell carcinoma of the right maxilla excised with good margins with no need for adjuvant treatment.\nFour weeks later, the patient was returned to the operating room for removal and modification of the obturator. The cavity was healing well, and both implants were firm with no evidence of infection. The initial obturator was modified with the application of a soft lining material and the patient subsequently discharged with instructions on the insertion and removal of the obturator.\nAt the 12-week review (Fig. ), it was noted the patient had a degree of mucosal polypoidosis in the antral cavity, most probably plaque induced, where the patient found it difficult to clean around the implants. Oral hygiene instruction was reiterated, and construction of the definitive implant bar-retained obturator was commenced.\nFour months following surgery (April 2014), a definitive implant bar-retained maxillary obturator was fitted utilising precision attachments (Rhein attachments, Rhein83, NY, USA.) (Figs. and ). The retention and support given by the obturator was excellent, and the patient and parents were very pleased with the aesthetic and functional outcome (Figs. , , and ) provided by this prosthetic rehabilitation. The patient was put on a regular maintenance programme of review at 6-month intervals and continued to display an excellent standard of oral hygiene around the implants and to report a high degree of oral functioning using it. All mucosal polyposis resolved very quickly following the patient’s improved hygiene measures. He continued under review with no evidence of recurrence or problems with the implants or prosthesis in the 22 months since the surgery. The plastic Rhein female attachments were replaced at 18 months, but no other modifications have been required to this obturator since it was fitted. A recent radiographic review (Fig. ) demonstrated no significant peri-implant bone resorption, and clinically, there had been no alteration in facial growth or appearance (Fig. ) of this young patient who was 16 years of age at the time of his final review (February 2017). He continues under regular review. |
A 42-year-old male was referred to our neurosurgery clinic for management of newly discovered brain metastases secondary to malignant melanoma. He was initially diagnosed with melanoma in early 2012 after he noted a lesion on his scalp and developed local lymphadenopathy. He underwent biopsy followed by wide excision and lymph node dissection which confirmed a diagnosis of metastatic melanoma. His tumor was found to carry the BRAF V600E mutation. He progressed quickly, with diffuse metastatic disease throughout the body by August 2012.\nShortly after his diagnosis with metastatic disease, he was placed on a trial and treated with PD-1 ligand antibody and vemurafenib. Despite this therapy, in February of 2013, a surveillance magnetic resonance imaging (MRI) of the brain demonstrated approximately 20 new brain metastases (see Figure ). His systemic therapy was switched to ipilimumab at that time and he received four doses from early March to May of 2013. Due to the fact that the patient remained quite well from a clinical standpoint (Karnofsky = 100) and was completely asymptomatic from his brain metastases, we recommended that the patient undergo stereotactic radiosurgery (SRS) with the Gamma Knife. We elected to treat him in stages and his first treatment was in late March of 2013. At the time of every follow-up Gamma Knife scan, several new metastases were noted. In all, the patient underwent seven Gamma Knife procedures to treat a total of 98 brain metastases. His last treatment for intracranial disease was in May of 2015 at which time a lesion that was previously treated with SRS twice was surgically resected due to continued radiographic progression. Since that time, he has had no progression of his intracranial disease.\nThroughout all of the intracranial treatments, the patient has enjoyed a good clinical status with a Karnofsky score of 100 and has been able to continue his full-time position as a software engineer. |
A 74-year-old man presented to the primary hospital complaining of frequent urination. A tumor in the left kidney was revealed via an abdominal computed tomography (CT) scan (Fig. ). The patient then visited the Department of Urology at our hospital. Whole-body bone scintigraphy using technetium-99m methylene diphosphonate (Tc-99m MDP WBBS) demonstrated an abnormally increased uptake in the left mandibular angle (Fig. ). The patient was referred to the Department of Dentistry and Oral Surgery at our hospital for further evaluation. A panoramic radiograph and CT scan of the maxillofacial region revealed an osteolytic lesion accompanied by a slight expansion of the cortical bone in the mandibular angle and a cystic lesion accompanied by expansion of the cortical bone in the maxillary anterior region (Fig. ). He had no subjective symptoms in the maxillofacial region, and a physical examination revealed no symptoms. An intraoral examination demonstrated swelling of the maxillary anterior region. MRI of the mandibular lesion showed low signal intensity on a T1 weighted image and high signal intensity on a T2 weighted image in the central area (Fig. ). The maxillary lesion showed high signal intensity on the T2 weighted image. Whole-body 18F-fluorodeoxyglucose positron emission tomography/computed tomography (FDG-PET/CT) showed abnormally increased FDG uptake in the left kidney, but there was no abnormal uptake in the maxillofacial region. The results of the imaging examinations suggested a diagnosis of left kidney cancer (cT2aN0M1, Stage IV). They also revealed an osteolytic lesion of the mandible and a cystic lesion of the maxilla. The possibility of kidney cancer with mandibular metastasis could not be denied by the results. Left nephrectomy was performed by urologists under general anesthesia, and the patient was diagnosed with clear cell renal cell carcinoma (RCC) of the left kidney.\nApproximately 1 month later, resection of the mandibular lesion with a safety margin and removal of the maxillary lesion were performed under general anesthesia by oral and maxillofacial surgeons. The findings of an intraoperative rapid-frozen pathological assessment of the mandibular lesion suggested that it was not a metastatic lesion from RCC. Concomitantly, mandibular reconstruction using a titanium plate and screws was performed to prevent mandibular fracture. The patient was diagnosed with ossifying fibroma of the mandible and an odontogenic keratocyst (OKC) of the maxilla via a postoperative histopathological examination (Figs. and ). His postoperative course has been uneventful (Fig. ).\nThere was no evidence of recurrence of RCC and jaw lesions 18 months after the second surgery. |
We present a case of a 50-year-old Caucasian male who presented to the emergency department with complaints of lower abdominal pain, fever and sweating. On examination, the patient had tenderness to palpation in the right iliac fossa, with significant rebound tenderness and guarding. Body temperature was recorded at 38.5°C.\nThe patient's past medical history was significant for an incident of similar pain six months prior to presentation. He was diagnosed with sigmoid diverticular disease confirmed by computed tomography (CT) scan and managed conservatively. A subsequent colonoscopy confirmed the diagnosis, and did not reveal any other colonic pathology.\nFurther investigations revealed a raised white blood cell count of 16,000 per microliter. CT scan of the abdomen and pelvis disclosed evidence of extensive free gas under the right dome of the diaphragm confirming suspicion of a perforation. Fluid-filled prominent loops of small bowel were noted. However, none of them were dilated to suggest obstruction. Mild bowel thickening was also noted around the cecum. Pneumatosis coli suggestive of ischemic bowel, extending from the cecum to the proximal ascending colon was seen. A blind ending structure with calcifications was also seen (Figure ). Other significant findings on the scan included a liver cyst, consistent with the patient’s previous CT scan, and consolidation at the base of the right lung.\nSubsequently, the patient underwent an emergency laparotomy and a diffuse four-quadrant peritonitis was seen. A giant perforated MD, 80 cm proximal of the ileocecal valve was identified as the cause of the peritonitis. The giant MD measured approximately 10 cm in length and 2.5 cm in width (Figure ). A small perforation at the tip of the MD was observed. No other intraabdominal pathology was identified, in particular, there was no evidence of colonic ishchaemia. Resection of the segment of small bowel bearing the MD was performed with a side-to-side stapled anastomosis.\nPathologic examination showed a T-shaped length of bowel, 6 x 5 x 3 cm in maximal dimension, with a 3 x 5 x 6 cm portion of mesentery attached. The presumed diverticulum was 5 cm long with a maximal inner circumference of 5 cm. The perforation measured 0.2 cm and the lumen of the specimen was stained green. No heterotropic tissue was identified.\nPost-operative recovery was uneventful and the patient was discharged on oral antibiotics. |
The patient was a 1.5-year-old boy who was admitted to the hospital with a primary complaint of fever, diarrhea, and vomiting. At the beginning of the hospitalization, the patient had a generalized clonic-tonic seizure. The patient was dispatched to our intensive care unit on the fourth day of admission due to reduced consciousness.\nPatient is the first child of a family and is resident of the village. He had a history of pneumonia at 6 months of age. There was a history of seizure of fever in his family. From the outset, he was treated with ceftriaxone, vancomycin, phenytoin, and acyclovir. The growth and development were normal.\nThe patient had tachypnea and tachycardia, and fever was 39°C. Percent oxygen saturation without getting oxygen was 98%. It was toxic and had GCS = 8. The patient had a mild tenderness on the right side of the abdomen with a predominance in the RLQ (right lower quadrant). The rectal examination was normal. The amount of urine output was normal. Patients were treated with liquid therapy, and intravenous antibiotics were changed to moropenem and vancomycin. Patient tests: serum electrolytes (sodium and potassium), blood gas analysis, coagulation tests, liver and kidney function tests, vidal test and Albumin were normal.\nSome of the patient's laboratory results were as follows:\nIn addition, he had hypocalcemia and Hypomagnesemia and were treated with sepsis as a common symptom. Due to biliary secretion from the stomach tube, bloody diarrhea, tenderness and abdominal distension, abdominal ultrasonography was performed with suspicion of obstructive problems such as enuresis and acute appendicitis.\nUltrasound findings favored the diagnosis of acute appendicitis: insignificant free fluid in the space between the interleuk and a non-compressible appendix of 65 mm in the lower right quadrant of the abdomen was evident. The patient underwent appendectomy (Figure ). One day after the surgery, the child was alert (Glasgow Coma Scale /Score GCS = 12), but the fever continued. Metronidazole and intraperitoneal ciprofloxacin were administered. Two days after the commencement of these antibiotics and appendectomy, the patient's alertness became normal, and the general condition was satisfactory with erythrocyte sedimentation reaction (ESR) = 32, and platelet count and white blood cell count increased. A pathological report of acute puffiness appendicitis with peri appendicitis confirmed. After two weeks, the patient was discharged with a good general condition. |
A 48-year-old male reported to the pain clinic at our institution with a history of chronic ISP. The pain was 8/10 on the visual analog scale (VAS). The patient had consulted a urologist, a neurologist, and rheumatologists with no relief of symptoms.\nThe patient was suffering from ISP for the last 3-4 years, the pain was nagging in character and was associated with a perception of swelling and heaviness in the inguinoscrotal region. There was restriction of forward bending along with mild pain. The pain in the inguinal region worsened when the patient tried to stand from a sitting position. He complained of morning stiffness in the back with gluteal pain that was suggestive of sacroilitis. He had a restricted and painful forward flexion. The only significant finding was paramedian tenderness in the upper lumbar region. There was no history of fever or any previous surgery. There was no tenderness in the inguinal or scrotal region. Ultrasonography of the testis did not reveal any abnormality. Abdominal hernial sites were normal. Erythrocyte sedimentation rate was persistently raised. Rheumatoid arthritis screening and HLA B27 were negative.\nThe clinical workup of the patient led to the provisional diagnosis of myofascial pain involving the psoas muscle. A trigger point injection of 8 mL of 0.25% lignocaine in 5% dextrose was performed under ultrasound guidance in the psoas muscle. The back pain was partially relieved along with complete relief in the inguinoscrotal pain. Pain relief lasted only for a few hours, correlating with the duration of action of the local anesthetic. After the trigger point injection, the patient reported gradual progressive worsening of the back pain so much that he had to take time off work.\nOn re-examining the patient after 15 days, tenderness was elicited in the upper lumbar region. A repeat MRI of the lumbosacral spine was advised which revealed Pott's spine at the L2-3 level with associated edema in the psoas muscle of the affected side [Figures and ]. The patient was started on antitubercular therapy (ATT) and was relieved of his symptoms in 4 weeks. The patient is still on ATT and is under our follow-up. |
A 46-year-old male with a past medical history only of treated tuberculosis presented to our emergency department (ED) via ambulance after being found lying in the hallway of his apartment building. The paramedics did not find signs of trauma at the scene. The patient was fully alert and oriented and complaining of right-sided upper abdominal and chest pain. He was afebrile with a blood pressure of 92/65mm Hg, heart rate 89 beats/min, respiratory rate 24 breaths/min, and an O2 saturation of 96% on room air. On repeat measurement, blood pressure and respiratory rate improved spontaneously and remained stable throughout the remainder of his ED course. On questioning, the patient was uncertain of the duration of his pain but stated that he believed it began upon awakening earlier in the morning. He denied associated nausea, vomiting, dyspnea, fevers, chills, cough, recent illness, or similar past episodes and was able to tolerate oral food and fluids without difficulty prior to arrival. He denied taking any medications and had no known drug allergies. His social history was positive for alcohol and crack cocaine use, most recently on the night prior to arrival.\nPhysical examination revealed a thin, middle-aged male with bi-temporal wasting who was speaking easily and did not appear to be in any distress. He had no obvious signs of recent trauma. Of note, there was no blood visible on examination of his skin, clothing, or stretcher. He had no jugular venous distension, his trachea was midline, and his cardiovascular examination was normal. However, the lung examination revealed decreased breath sounds on the right and diffuse abdominal tenderness with voluntary guarding that was increased in the mid-epigastrium and right upper quadrant. Peritoneal signs were absent, and the patient tested heme-occult negative on rectal examination.\nAn initial ECG obtained at triage revealed a normal sinus rhythm at 91 beats/min with a QRS axis of +75°and a QT interval (QTc) of 450ms. A large QRS voltage, prominent peaked T waves in the lateral pre-cordial leads, and prominent P waves inferiorly were also noted (). Treatment at triage was initiated with oxygen, aspirin, and nitrates. Following physician evaluation, intravenous calcium was administered despite no known history of renal insufficiency or hyperkalemia and the patient was given morphine 2mg IV with complete resolution of his pain. Following these early interventions, a repeated ECG showed no change. A portable chest x-ray was then obtained, which revealed a right-sided tension hemopneumothorax ().\nAdditional physical examination at that time revealed a healing 1cm linear laceration in the right posterior axillary skin fold. No crepitus, erythema, bleeding, or dried blood was present at or surrounding the site. When confronted, the patient admitted to a stab wound with an unknown object three days earlier. Thoracostomy was performed with immediate return of 1200cc of dark, non-clotted blood, and he was admitted to the hospital by the trauma service. Repeat ECG following thoracostomy revealed resolution of the peaked T waves, changes in the voltage of the P and QRS waves, a shortening of the QTc, and a shift in the QRS axis (). The patient’s initial serum potassium was 3.9 mmol/L (normal 3.5–5.0 mmol/L), and he did not develop any abnormalities in serum cardiac markers during his hospitalization. Cardiac ultrasonography was not performed. The chest tube was removed on the second day without re-accumulation of the hemopneumothorax and the patient was discharged on hospital day three. |
A 46-year-old Caucasian male was referred to our neurology clinic due to sensory symptoms of lower extremities. Presence of dark urine at 8 years of age revealed that he was diagnosed with AKU (urinalysis and biochemical testing confirmed the presence of HGA at the time of diagnosis). He developed progressive lower back and shoulder pain before 25 years of age and first signs of ochronosis were evident as early as 12 years of age on pinna of ears. Family history was unremarkable (no report of osteoarthritis, AKU or consanguinity). The patient had extensive arthritic and cartilage damage on all weight bearing joints of knees, hips and vertebral column. Shoulder joints were the most severely affected showing extensive swelling, limited mobility and arthritic degeneration [Figures and ]. Clinical examination of the spine showed impaired cervical, thoracic and lumbar mobility. An MRI of spine displayed multi-level disc space narrowing, spinal cord compression and disc herniations at cervical and lumbar spine with effacement of cerebrospinal fluid space [Figures and ]. The patient experienced persistent tingling, diffuse constant pain and numbness in both of his feet and toes. Motor testing of the lower extremities showed normal muscle tone and strength, however, motor movements of the upper extremities were sluggish and painful, and muscle strength was slightly weak. Sensory testing of the extremities to pin prick, light, touch, vibration and temperature was normal. Examination of the patient's gait was also normal. Patient reported no trouble with vision, speech or swallowing. Due to extensive shoulder arthropathy and cartilage damage, the patient required bilateral shoulder replacement and underwent total right shoulder replacement surgery. The patient showed symptoms of dysthymia, although he did not meet formal criteria for depression. Limited mobility, decreased range of motion (ROM) and lost function of day-to-day basic activities initiated irritability, social isolation, feeling of worthlessness and diminished interest in pleasurable activities as major psychological issues. The surgery improved some ROM. The patient adhered to a low protein diet. The patient was administered multiple steroid injections and analgesics in both shoulders over the past 3 years to manage pain. Despite some reduction in pain, the injections were not very effective to change the quality of life. Our study is in contrast to other findings that report no correlation between disability of AKU patients with severe impairments and self-reports of mental competencies.[] |
A 41-year-old, otherwise healthy male patient, pig farmer by profession from Ciego de Avila was admitted to a local hospital on 21 February 2010 because of burning ocular pain, blurred vision and redness of the left eye. He could not recall any ocular trauma. Empirical topical antibacterial treatment with ceftazidim (3 %) and vancomycin (3 %) was instituted (one eye drop every hour). The reaction to therapy was poor with increasing infiltration and ulcer extension. Corneal scraping grew methicillin-resistant Staphylococcus aureus (susceptible to amikacin, ciprofloxacin and gentamicin) and treatment was changed to amikacin (3 %) and ceftriaxone (3 %) eye drops every 30 min. The first few days the patient experienced a slight improvement but after a week clinical manifestations worsened, so he was referred to the Ophthalmological Institute 'Ramón Pando Ferrer' in Havana. At arrival the patient had slight oedema of the upper and lower lids of his left eye, scanty yellow secretions, intense cilium-conjunctival injection and a dense corneal yellowish infiltrate leaving only 1 mm of the peripheral limbo clear. Slit lamp examination showed central extensive epithelial defects with irregular and thick borders involving all corneal layers. There were no satellite lesions or evidence. No defects were observed in the anterior segment and his right eye was normal. Ocular ultrasound examination on 10 March 2010 showed a vitreous cavity without opacities and normal retina. Ten days later a filamentous fungus from corneal scrapings was reported and the patient started treatment with miconazole (1 %) one eye drop every hour, ketoconazole (1 %) one eye drop three times per day, and oral ketoconazole (200 mg daily for 7 days). On 22 March, he underwent penetrating keratoplasty and miconazole was replaced by natamycin (5 %) eye drops every 30 min. Eight days later topical ketoconazole was stopped and empirical oral moxifloxacin (400 mg daily for 7 days) was added. Natamycine was continued until discharge on 7 April 2010. The outcome was favourable with a five-year follow up. |
A 58-year-old male patient had developed a chicken egg-sized painful lump on the left popliteal fossa two months before he visited our center. He had no obvious trauma history in the affected site. He was diagnosed with osteoarthritis in his knee joints about 5 years ago. At first, he went to an internal medicine clinic, and his doctor prescribed some oral medication. However, there was no change after he took the oral medicines. After trekking a mountain, the pain and swelling extended to his lower leg. He was referred to our outpatient clinic. His visual analogue scale was 5 and tenderness was present at the popliteal fossa and the calf. Pain was aggravated when the foot was dorsiflexed. On examination, unilateral grade 1 pitting edema accompanied by slight redness and warmth was noted in his left leg below the knee and the circumference of the left leg was 1 cm greater than right when measured at the calf (). He said the pain and swelling of the leg had been improved since the trekking. There was no motor weakness or sensory change. Weight-bearing posteroanterior plain radiography showed Kellgren-Lawrence grade 1 radiographic alterations bilaterally.\nUltrasonography revealed large hypoechoic space that was divided into two compartments behind MHG. Ultrasound of the popliteal fossa showed the presence of a well-defined cyst connected knee joint. It had different component of a typical popliteal cyst: the base, the neck, and the body located between the tendon of the semimembranosus and the tendon of the MHG (). The body of the cyst was communicated with fluid collection of the calf. In addition, some joint effusion and synovial hypertrophy were seen. Initially, ultrasoundguided puncture aspiration was performed to obtain 10 mL of dark red-colored translucent liquid (). We performed the intralesional injection of corticosteroid (triamcinolone acetonide 20 mg) and used some nonsteroidal anti-inflammatory drug (aceclofenac). A few weeks later, he still complained of calf pain. A follow-up ultrasound revealed a fluid collection in his proximal calf. After re-aspiration of fluid under ultrasound guidance, the pain was relieved. |
A 30-year-old woman underwent radical nephrectomy for clinically localized left RCC in 1998, and was pathologically diagnosed with clear cell RCC, pT2b and Fuhrman grade 2. Bilateral lung metastases were found eight years after nephrectomy, and both lesions were surgically removed, followed by postoperative interferon treatment. However, by 2010 she underwent five lung resections and also one radiofrequency ablation, and the patient achieved long-term response with adjuvant sunitinib. In May 2013, a right ovarian metastasis was detected, which was surgically excised. In July 2013, metastases to the bones (pelvic and lumber vertebrae) and hilar lymph nodes were detected, and she underwent treatment with axitinib followed by sunitinib. In 2016, her mediastinal lymph nodes were treated with EBRT of 60 Gy in 30 fractions at an external institution. In May 2018, liver metastasis was detected, and this lesion was treated with radiofrequency ablation. In September 2018, metastasis in the right ovary led to its surgical excision, followed by nivolumab at the standard dosage (infusions of 240 mg every alternate week) as third-line therapy. Four months after the initiation of nivolumab, computed tomography (CT) imaging revealed the progression of lung, liver and bone metastases and nivolumab monotherapy was further continued for two months. In January and February 2019, she underwent radiofrequency ablation for newly discovered liver metastases. In March 2019, CT imaging demonstrated gradual growth of the left iliac bone metastasis. The Tumor Board at our hospital decided that there was no surgical indication for this lesion.\nThe CT scan acquired prior to her visit to our hospital revealed an iliac crest tumor measuring 9.4 × 8.8 × 6.0 cm () and tumor volume measuring 347.5 cm3. She was referred to our department for treatment and considering the large tumor burden other than metastases, we proposed HDR-ISBT for tumor control and volume reduction. Informed consent was obtained from the patient and her family. Nivolumab was continued until 10 days before the initiation of brachytherapy. After administration of spinal anesthesia, 13 applicator catheters were percutaneously inserted into the tumor under real-time CT guidance. These applicators were placed so that the whole tumor area was irradiated with simultaneous controlling the position of the needle against the adjacent structures.\nAfter completion of this procedure, CT scan was taken for planning radiation therapy. In the treatment planning system (Oncentra® Brachy; Elekta AB, Stockholm, Sweden), clinical target volume (CTV) was set up based on the acquired CT images and 7 Gy per fractionated dose was prescribed in 100% of the CTV. The doses encompassing 90%, 95%, and 98% of the CTV (D90, D95, D98) were 9.2 Gy, 8.5 Gy, and 7.9 Gy per fraction, respectively. The minimum dose to the most exposed 0.1 cc, 1.0 cc, and 2.0 cc (D0.1cc, D1cc, D2cc) of the small bowel were 7 Gy, 6.5 Gy, and 6.3 Gy per fraction, respectively. The HDR-ISBT of 35 Gy in five fractions for three days (two times a day with six hours’ interval) was administered (). Brachytherapy was carried out using a 192Ir remote after-loading system (MicroSelectron v3® HDR, Nucletron, ELEKTA AB, Stockholm, Sweden).\nNivolumab was restarted nine days after HDR-ISBT. Three months after completion of HDR-ISBT, shrinkage of the irradiated left iliac lesion () and left internal iliac lymph node () was evident by CT. Simultaneously, the metastatic lesion in the lumbar vertebrae (L4), which was the non-irradiated site, showed evidence of disappearance on the same CT (). There was no apparent increase in size of the remaining non-irradiated metastatic lesions. presents the summarized imaging studies and clinical course after the initiation of nivolumab.\nDuring the HDR-ISBT period, pain associated with applicator placement was addressed using continuous venous analgesia. The day after the applicator was placed, a significant elevated serum lactate dehydrogenase (LDH) level was observed in the blood test () that lasted for seven months. As we suspected tumor lysis syndrome, we performed intravenous rehydration, which led to a decrease in serum LDH levels within a few days, and the treatment was completed without any problems, and reached the normal range three months after brachytherapy (, reference range 124-222 U/l). After the treatment, there were no complications except for mild dermatitis. |
A 16-day-old full-term boy was admitted to hospital with dehydration after he had refused breastfeeding. Clinical and laboratory examination did not reveal any underlying specific disease, and he was discharged after 2 days. No information on the content of the scrotum was recorded in the EPJ notes from this admission. 4 days later, he was readmitted with pain and the appearance of a bulge in the right, lower part of the abdominal wall (Fig. ) and no testis in the right scrotum. The abdominal wall swelling was not reducible after sedation. An ultrasound revealed a hernia sac located between the m. obliquus externus and m. obliquus internus, penetrating through a 7 mm wide hernia opening. The sac contained a normal-sized right testis and a loop of small intestine with weak peristaltic movements. A normal X-ray did not show any sign of ileus.\nThe hernia was repaired the same day under general anesthesia. Following a horizontal, small skin incision, an elongated hernia sac directed in a craniolateral direction was dissected from the position under the obliquus externus muscle with its port close to the lateral edge of the rectus muscle (Fig. a). The sac was opened and contained a normal-sized right testis, with no gubernacular cord attached to the epididymis in the lower pole of the testis, and the mesorchium of the testis was connected directly to the wall of the hernia sac.\nIn addition, a loop of the small intestine with compromised circulation (Fig. b) was incarcerated inside the hernia sac. The small intestine was recovered and returned to the abdomen, aided by a small incision in the hernia port. The testis, with a sufficient long spermatic cord, was dissected from the hernia sac and tunneled subcutaneously into a subdartos pouch. The hernia sac was then resected, and the opening was closed around the cord. No exploration for an inguinal canal was performed, and no complications occurred. |
A 43-year-old woman with a prior history of an enterocutaneous fistula, who had required multiple abdominal surgeries, including bowel resections and prior pulmonary embolism, presented to the emergency department with recurrent enterocutaneous fistula and synchronous acute pulmonary embolism. CT scan of the abdomen and pelvis on admission demonstrated rotation and mild inferior displacement of the right kidney, with the long axis of the kidney lying in the horizontal plane [Figure and ]. This was a new finding compared with multiple prior CT scans available within the last 2 years in which the kidneys were normally oriented parallel to the psoas muscles. Increased size of both the liver and right kidney was also identified. CT pulmonary angiography performed synchronously for syncope and pulmonary decompensation demonstrated a segmental pulmonary embolism and right heart strain,[] including reflux of contrast material into the inferior vena cava, interventricular septal bowing, enlarged main pulmonary artery (34 mm), and right ventricular dilatation (RV/LV ratio of 1.8) [Figure –]. The most recent CT scan done 45 days prior to this admission is shown in Figure and .\nFollow-up imaging done after 10 days demonstrated normalization of the right kidney axis, position, and size, as well as decrease in hepatomegaly [Figure and ]. Calculation of liver and kidney volumes from CT scans done 45 days before presentation, on the day of admission, and 10 days following admission, using the method described by Dello et al.,[] is presented in . Repeat CT pulmonary angiogram [Figure and ] performed 10 days after presentation showed normalization of the thoracic changes including normal size of main pulmonary artery (25 mm) and normal right ventricular size (RV/LV ratio of 0.86). Nephroptosis,[] which is a condition in which the kidney descends more than two vertebral bodies (or >5 cm) during a position change from supine to upright, was excluded by ultrasound examination of the right kidney which failed to deviate significantly when the patient changed from supine to upright position. |
The patient was a 51-year-old woman who consulted our department in February 2013 about discomfort in the mandible. She had no medical history. Ameloblastoma was diagnosed during the first oral surgery in 2006, and she underwent fenestration procedures. The tumor recurred frequently, and she underwent repeated fenestration. She was dissatisfied with the outcomes of previous hospital's treatment policy and came to our department hoping for the complete resection of the tumor.\nAt the first visit, she described discomfort in the right side of the mandible and the perceptual abnormality of the mentum. Her face was symmetrical, but the mandibular right molar aspect bulged slightly; the swelling seemed like a parchment. On orthopanoramic radiographs (Figure ), a multilocular radiolucent lesion was observed at the teeth position of 43 to 46 and extending to the lower edge of the mandible. Computed tomography (CT) images (Figure A, B) revealed that the right mandible was filled with tumor; multifocal resorption was evident, and the buccolingual aspect of the mandible was bulging. The inferior alveolar tube could not be distinguished from the lesions.\nWe diagnosed recurrence of ameloblastoma and proposed a treatment protocol of radical resection and immediate bony reconstruction. The graft donor site selected was a nonvascularized iliac bone. She consented to our surgical plan.\nIn March 2013, the patient, under general anesthesia, underwent right mandibular resection (teeth 43 to 46; length 35 mm) and reconstruction with nonvascularized free iliac bone. The mandibular defect classification was “L”: lateral defect without the condyle and not crossing the midline. Before resection of the mandible, a memory plate was installed to maintain the shape of the mandible. The memory plate was prepared prior to surgery by modifying the surgical model to mirror the healthy side of the mandible. A 4 × 4 cm2 section of the iliac bone was harvested at the inner plate (Figure ). The iliac crest was placed at the lower rim of the mandible and affixed to a mini-plate (Figure A, B).\nHistopathological study (Figure ) revealed that the tumor nests comprised the peripheral cylinder layer and stellate reticulum, but had no mitotic figures and atypia. The final pathological diagnosis was ameloblastoma.\nAt follow-up 1 year and 8 months after surgery, the tumor had not recurred. However, the patient was suffering from right-sided difficulty in mastication; removal partial denture was not aesthetically preferable, and therefore, she wanted a fixed prosthesis. We decided to start occlusal construction of a fixed prosthesis with dental implants. The reconstructed jaw bone was 30 mm high and 10 mm wide, which was large enough to receive the dental implants (Figure A, B). Osteosclerotic findings like the cortical bone were observed around the grafted bone on CT images (Figure C). The occlusal relationship was examined on the setup model, and the positional direction of the dental implant was determined.\nIn November 2014, with the patient under general anesthesia, the mini-plate was removed from the grafted bone, and the dental implants were placed. We placed four dental implants using a surgical stent (for teeth 43, 44, and 45, 3.5 mm in diameter and 11 mm in length and for tooth 46, 3.5 mm in diameter and 9 mm in length). (Astra Tech Implant System, Dentsply Sirona Inc). All the implants had the appropriate insertion torque value. Five months after primary surgery, vestibuloplasty (vestibular extension with periosteal separation procedures) was performed under local anesthesia because oral hygiene management was difficult due to narrow oral vestibule and mobile mucosa. During the second procedure conducted in June 2015, we confirmed osseointegration in all four implants. The patient wore the final fixed prosthesis in October 2015. We regularly performed maintenance therapy to manage tumor recurrence (CT examination every 6-12 months) and to check or provide care for oral hygiene every 3-4 months (Figures and ). At the time of writing, the tumor had not recurred, and there was no sign of abnormal bone absorption or disintegration of the dental implants. The patient's oral hygiene is good, with no implant movement or irritation of the mucosa around the implant. She is satisfied with the results both aesthetically and functionally. |
A 71-year-old female presented to the emergency department with constipation and obstipation for the previous 3 days. Associated with that she had nausea and vomiting.\nHer past medical history included type 2 diabetes mellitus. Her surgical history was significant for a primary cutaneous melanoma excision on her right arm 9 years prior to her presentation. She had attended routine follow-up for 5 years following her melanoma surgery.\nOn initial examination she had clinical signs of dehydration—dry mucous membranes, oliguria, decreased skin turgor—a grossly distended abdomen, no abdominal scars or herniae and high-pitched bowel sounds.\nA plain-film of the abdomen and erect chest x-ray showed multiple dilated loops of small bowel. A subsequent CT scan showed a high-grade SBO involving the proximal mid small bowel as well as a small bowel to small bowel intussusception (Fig. ). There were also multiple mucosal-enhancing lesions in the stomach, duodenum and small bowel most consistent with metastases (Fig. ), as well as a suspicious mass in the lung base.\nThe patient was initially managed conservatively with wide-bore sump NG as well as intravenous hydration. She was then brought to the operating theatre and underwent a laparoscopy where multiple dilated loops of small bowel were seen and then converted to laparotomy to avoid enterotomy. The small bowel was examined where six discrete lesions of intussusception were identified (Fig. ), distributed all along the small bowel from the DJ flexure to terminal ileum, and manually reduced (Fig. ). This provided satisfactory relief of the obstruction. All small bowel was re-examined and was viable. The lead point for each intussusception was an intraluminal lesion in each site. Due to the extent of the intestinal disease, coupled with suspicion of further distal disease in the chest on the pre-op CT, it was decided that it would be inappropriate to perform resections. Further, resecting all lesions would have carried a considerable risk of short-bowel syndrome. One lesion was biopsied to obtain a tissue diagnosis, revealing epithelioid cells with central nuclei and prominent nucleoli with a nested growth pattern, positive for S100 (Fig. a) and melan A (Fig. b) and a diagnosis of metastatic melanoma was made. The patient recovered well following her surgery. She was discharged on post-operative Day 7 with medical oncology follow-up arranged.\nSubsequent staging CT demonstrated brain and lung metastasis. The patient was commenced on systemic chemotherapy, in addition to a low-residue diet and did not require any further intervention for bowel obstruction. She died 9 months later from metastatic disease. |
A 56-year-old female was noted to have a solitary left thyroid nodule, discovered incidentally on CT scan following neck trauma. The patient's past medical history was significant for hypertension, hyperlipidemia, gastroesophageal reflux and depression. The patient reported no symptoms of dysphagia, history of neck irradiation or symptoms of thyroid dysfunction and denied family history of thyroid disease, including thyroid cancer. Neck ultrasound demonstrated a dominant, heterogeneous, 2.9-cm solid nodule with microcalcifications. Ultrasound-guided, fine-needle aspiration cytology revealed a follicular lesion of undetermined significance. The patient was referred to a local general surgeon and underwent left thyroid lobectomy. According to the operative report, the recurrent laryngeal nerve was identified and preserved throughout its course. The patient tolerated the procedure well and was noted to have normal speech following the operation.\nThe original thyroid lobectomy operation was performed by a general surgeon who typically performs half-dozen thyroidectomies a year at a rural hospital. The surgeon described the original operation as ‘uneventful’ in a letter accompanying the patient, and a detailed review of the operative report by the authors reveals no mention of any inflammatory reaction or fibrosis surrounding the tumor. The original pathology report describes a well-differentiated papillary thyroid carcinoma 3.0 × 2.5 × 1.5 cm (Staging classification T2 N0 M0). There was no lymphatic or vascular invasion (V0). There was no extra capsular extension and margins were negative (R0). A normal parathyroid gland was identified, but no lymph nodes were seen.\nOn the third postoperative day, however, the patient returned to the surgeon's office with complaints of neck pain and swelling. A CT scan of the neck performed at that time revealed a 7.7 × 3.7 cm fluid collection in the left neck (Fig. ). The patient was taken emergently to the operating room, where upon opening the incision a large amount of purulent fluid was evacuated. There was a thick, yellowish exudate adherent to the involved surfaces, and the strap muscles were noted to be thickened, stiff and friable. Inspection of the esophagus revealed no obvious injury, but surrounding tissues were noted to be discolored and inflamed, making visualization of the esophagus difficult. The recurrent laryngeal nerve, which had been identified during the initial operation, was not seen.\nTo better characterize the nature of the injury, the wound was filled with sterile water, and air was instilled into the esophagus via placement of an orogastric tube. Air was then seen bubbling into the wound. Indigo carmine diluted in water was instilled into the esophagus, whereupon blue-tinged fluid was seen leaking into the wound from an upper esophageal defect. A 3.0 silk suture was placed in the area of the defect, and the repair buttressed with omohyoid muscle. The strap muscles were closed in the midline, and two Jackson–Pratt drains were placed for wide drainage.\nThe patient soon demonstrated a recurrent esophageal leak (Fig. ), which was managed by maintaining the patient nil per os, providing nutrition via a feeding tube, and applying negative pressure to the wound. The esophageal leak gradually resolved, as evidenced by multiple contrast esophagrams, permitting removal of the patient's drains. Prior to discharge from the hospital, barium esophagram showed no evidence of contrast extravasation.\nPathology demonstrated a 3-cm, well-differentiated papillary thyroid carcinoma with negative margins and no lymphatic or vascular invasion (T2, N0, M0, R0 and V0). The patient was referred to an experienced endocrine surgeon at a tertiary care institution for completion thyroidectomy; that surgeon performs ∼250 thyroidectomies per year. Upon evaluation, the patient complained of hoarseness and dysphagia, most pronounced with liquids, and flexible laryngoscopy revealed a fixed and paramedian left true vocal cord. Thyroid ultrasound showed multiple sub-centimeter nodules of the remaining thyroid, diffuse heterogeneity and no evidence of suspicious cervical lymphadenopathy.\nUncomplicated completion thyroidectomy was performed ∼11 months after the patient's initial operation. Prior to completion thyroidectomy, the patient's voice quality had improved considerably, but nasolaryngoscopy again showed a fixed paramedian left vocal cord. Final pathology revealed no evidence of malignancy, and the patient was subsequently referred for adjuvant radioactive iodine therapy. |
A 27-year-old nullipara presented to her local hospital at 33 weeks' gestation with decreased fetal movement, uterine contractions, and possible leakage of fluid from the vagina. Testing confirmed rupture of membranes; therefore, the patient was started on antibiotics to increase latency and was given betamethasone to hasten fetal lung maturity. An ultrasound at the community hospital showed polyhydramnios and a fetal double bubble sign consistent with duodenal atresia. The mother was transferred to Penn State Milton S Hershey Medical Center for anticipation of preterm delivery in a fetus that would require postnatal surgery.\nAfter transfer, fetal monitoring showed normal fetal heart rate variability with accelerations and occasional decelerations related to contractions. An ultrasound confirmed the double bubble sign and polyhydramnios. Blood was observed in real-time swirling into the amniotic fluid from the umbilical cord (). The patient was taken to the operating room and an emergent cesarean section was performed. The amniotic fluid was grossly bloody. A viable male infant weighing 2295 grams was delivered with Apgar scores of 7 and 8 at one and five minutes, respectively.\nThe umbilical cord overall length was 29.5 cm and there were 13, 0.5- 1 cm exposed segments of the umbilical arteries spiraling along the length of the cord (). A discrete area from which the hemorrhage emanated was not identified. The placental disk was of normal weight and appeared grossly normal. Histopathologic examination of the cord showed absence of Wharton's jelly covering the umbilical artery, extreme attenuation of the media in the portion of the vessel exposed to the amniotic fluid, and degeneration of the overlying amnion (Figures and ).\nThe infant had no stigmata of Down syndrome. He appeared pale at birth and an initial hematocrit was 29.4% that was treated by a blood transfusion upon admission to the neonatal intensive care unit. Postnatally the abdominal X-ray revealed air in the stomach and first portion of the duodenum with the remainder of the abdomen appearing gasless, consistent with duodenal atresia. A nasogastric tube was placed and 37 ml of bloody secretions were suctioned. The infant underwent laparotomy on the second day of life where an atresia was noted in the third portion of the duodenum and duodenojejunostomy was performed. He did well postoperatively and was discharged on day 30 of life. |
An 89-year-old man underwent distal gastrectomy with lymph node dissection and concurrent resection of metastasis in the right axilla 6 years ago. The pathological diagnosis was T3N0M1 (skin), pStage IV. Immunohistochemistry of both the gastric cancer and right axillary metastasis tissues showed CK7 positivity, CK20 negativity, CDX-2 negativity, and a final diagnosis of solitary synchronous cutaneous metastasis originating from gastric cancer was made. The primary and metastatic tumors were resected with negative surgical margins. As adjuvant chemotherapy, seven courses of S-1 were administered. The patient was regularly followed up with laboratory tests and imaging studies with no signs of recurrence. After 6 years, the patient became aware of small, slowly growing reddish nodules on the scar of the primary surgery in his right axilla (Fig. ). On physical examination at the first visit, the vital signs were within normal limits, and the abdomen was soft and flat without tenderness, or rebound tenderness. The serum level of carcinoembryonic antigen was normal (4.8 ng/mL), and carbohydrate antigen 19-9 was slightly elevated (44 U/mL). Other laboratory data showed no abnormalities. Ultrasonography showed merged low echoic masses with a maximum size of 7 mm with high pulsation. Computed tomography revealed irregular thickening of the right axillary skin (Fig. ), and no other signs of recurrence including lungs and the abdomen were observed. Based on these findings, a diagnosis of late cutaneous metastasis from gastric cancer was made. For diagnosis confirmation and treatment, resection as a total biopsy was performed. The operation was performed with the local anesthesia without any complication. Histopathological examination revealed moderately to poorly differentiated adenocarcinoma with proliferation of atypical epithelium forming ductal and nest-like structures, and immunohistochemistry showed CK7 positivity, CK20 negativity, CDX-2 negativity (Fig. ). The histological appearance and the immunohistochemistry were identical to the tissues of the gastric carcinoma and the synchronous cutaneous metastasis resected 6 years ago (Fig. ); therefore, a diagnosis of solitary late cutaneous metastasis from gastric cancer was made. Considering his age and the absence of recurrences elsewhere, the patient received no additional treatment postoperatively and was only observed. |
A 38-year-old woman known for anxiety disorder developed constant epigastric pain without radiation toward the back. She mentioned that the pain was not related to the food intake and scored it between four and six (out of ten) on a visual analog scale. She had multiple episodes of vomiting, no bowel movement problems, and no fever. Her past medical and surgical history was otherwise uneventful. She went to her general practitioner who first performed an ultrasonography (US) and a CT scan. These exams showed a cystic-like retroperitoneal mass on the left side measuring 13.4 × 7.2 × 5.2 cm. Laboratory tests were normal. No further exams were undertaken at this point. The pain slightly diminished with paracetamol and non-steroidal anti-inflammatory drugs. However, after a couple of months, the pain reappeared, and the patient was scheduled for a CT-guided puncture of this cyst.\nAt first, the radiologist was not able to puncture the cyst due to a thick capsule. The cyst was finally punctured under US control but could not be completely evacuated due to technical problems (dysfunctional guide wire). Cytology came back negative for malignant cells and was compatible with a cystic lymphangioma. Unfortunately, the puncture of the cyst did not help to relieve the patient’s symptoms. The patient then underwent a magnetic resonance imaging (MRI) to assess more precisely the location of this retroperitoneal cyst and its anatomic relations. T2-weighted turbo spin-echo MR-sequences showed a lesion compatible with a cystic lymphangioma originating from the left adrenal gland (Figure ). The cystic lesion extended inferiorly to the renal vein and superiorly to the diaphragmatic pillar. Due to persisting invalidating pain, a surgical resection of this cystic lesion was proposed. The patient accepted the operation and signed the informed consent form.\nDue to the size and position of the cyst, and to avoid a rupture of the cyst during laparoscopy, a left subcostal laparotomy was performed. During surgery, the adrenal origin of the cyst was confirmed, and an ‘en bloc’ left adrenalectomy was performed without rupture of the cyst. No drain was left in place. The postoperative course was uneventful, and the patient was discharged on postoperative day 5. The patient was seen at the outpatient clinic one month after surgery and described no more symptoms.\nThe specimen consisted of an ovoid, cystic mass measuring 8.5 × 4.3 × 2.8 cm (Figure ). An unremarkable adrenal gland, measuring 3 × 1.2 × 0.7 cm, partially surrounded the cyst. The inner and outer surfaces of the cyst were smooth with no evidence of rupture. The wall of the lesion was thin with no tumor excrescences. The cyst was filled with clear fluid. Hematoxylin and eosin stain showed a cystic space lined by a single layer of flattened cells, with occasional pseudopapillae formation and bands of smooth muscle in the wall (Figure ). The lining cells had oval, regular nuclei and showed no atypia. The lining cells showed strong immunoreactivity for D2-40, PROX1, and CD31 and absence of staining for CD34 and CKAE1/AE3 (Figure ). The diagnosis of cystic lymphangioma originating from the left adrenal gland was then confirmed.\nThis article reports a rare case of symptomatic cystic lymphangioma originating from the left adrenal gland in a female patient successfully treated by complete surgical resection.\nThe majority of intra-abdominal cystic lymphangiomas is located in the mesentery, in contrast to adrenal location that is very rare []. No more than 30 cases of adrenal cystic lymphangioma have been described in the literature [,,-]. Table summarizes the English-written cases reported in the literature since 2000. As confirmed in this case report, age at symptom onset usually ranges from 30 to 50 years with a peak incidence during the fourth decade [,-,]. Cystic lymphangioma can occur in both adrenals [], but the right side is more often affected []. It also occurs more frequently in women [].\nOf note, lymphangioma is the generic term for a tumor arising from the lymphatic vessels and is often found in children. Lymphangiomas have an endothelial origin. The exact pathogenesis is currently not completely elucidated, and whether lymphangioma of the adrenals is a real neoplasm remains unclear []. The most likely etiology is a developmental abnormality or ectasia of the lymphatic vessels [,]. Cystic lymphangioma develops when a blockage of the lymphatic vessels occurs due to a benign proliferation. It should not be confused with lymphangioma-like adenomatoid tumors which have a different embryonic origin [].\nDifferential diagnosis of a retroperitoneal cystic-like lesion includes primary adrenal tumors, metastatic adenocarcinomas, angiosarcomas, multicystic mesotheliomas, or adrenal cysts []. Adrenal cysts can be further subdivided into pseudocysts, endothelial cysts (lymphangiomatous or angiomatous), and epithelial cysts []. Most of the time, lymphangiomas are non-secreting and are discovered incidentally during a radiological exam or a surgery. Symptomatic tumors can induce pain, fever, gastrointestinal disturbances, or hypertension [,]. Complications of this kind of tumors mainly are enlargement-causing pain or hemorrhage into the cyst. Diagnostic suspicion is based on clinical presentation, radiological images, and cytological exams.\nCystic lymphangioma of the adrenals does not have a pathognomonic radiological presentation, but new imaging modalities bring useful information helping the diagnosis []. As the lesion is rare in this organ, the radiological images lack specificity. On US, adrenal lymphangioma appears as an anechoic lesion in the suprarenal location [,]. US can be a good first exam modality []. Usually adrenal lymphangioma appears hypodense with smooth borders on CT scanner []. On MRI, cyst borders are delineated by injection of contrast. T1- and T2-weighted MR images are not pathognomonic, but adrenal lymphangioma usually appears as hypointense on T1-weighted sequences and homogeneously hyperintense on T2-weighted sequences. MRI being far more specific than CT, it usually allows distinguishing malignant adrenal lesions from benign ones []. Differential diagnosis includes metastatic tumors, carcinomas, or pheochromocytomas. Moreover, MRI is more sensitive than CT scan to detect degeneration of the cyst or intracystic hemorrhage [,].\nImmunohistochemistry is an important tool to differentiate this pathological entity from other diagnoses. Lymphangiomas usually display D2-40, PROX1, and CD31 positivity and absence of CD34 and CKAE1/AE3 stains []. Final diagnosis is made by histopathology combined with immunohistochemistry [].\nAsymptomatic cystic lymphangioma discovered incidentally can just be followed clinically or with control imaging, as there is no risk of malignant degeneration []. Puncture of the cyst can help the diagnosis but is not a therapeutic measure, as a punctured cystic lymphangioma will recur rapidly as demonstrated in this case. Puncture with injection of sclerosing agents like bleomycin have been tried but showed the same recurrence risk []. Surgical resection represents the definitive treatment if the cystic lymphangioma is symptomatic. Complete resection of the cyst is recommended, and associated parenchymal resection (adrenalectomy) depends on the location of the cyst and on the intraoperative dissection []. Decision to undertake a laparotomy or a laparoscopy depends on the position, the size, and the risk of rupture of the cyst. No data on the recurrence risk if intraoperative cyst perforation occurs are currently available in the literature. If the cystic lymphangioma is bleeding, preoperative embolization can also be considered []. |
A 39-year-old female came to our clinic complaining of gradual loss of visual in the left eye for 5 months. She had been diagnosed with acute retinal necrosis in the right eye, and wide-field photograph of the right eye showed patchy and diffusive retinal necrotic lesions (Fig. ). Pars plana vitrectomy was performed due to retinal detachment of the right eye 5 months earlier. The visual acuity in the left eye at the time of initial presentation was 20/20. In the meantime, she noticed floaters in her left eye. Superior retinal necrosis had been detected and it was recorded in her previous medical chart. The aqueous fluid from the left eye was positive for herpes zoster virus (VZV). She received repeated (nine rounds) intravitreal ganciclovir injection (3 mg/0.1 ml) into her left eye, one injection every 2 weeks, and systemic antiviral therapy for 5 months. The patient noticed a decrease in the visual acuity of the left eye and was referred to our clinic.\nWhen the patient came to our clinic, her best corrected visual acuity was 20/400 OS, 20/33 OS. The intraocular pressure was within the normal range in both eyes. There was no inflammation in the anterior chamber, and the lens was normal in the left eye. There was no posterior necrotizing involvement and no macular edema or exudation in the left eye. Indirect ophthalmoscope examination revealed pigmentary change in the superior peripheral retina of the left eye. Slight abnormality of the interdigitation zone in the fovea area of the left eye was detected on OCT. Angio-OCT revealed normal capillary density of superficial retinal capillary, deep retinal capillary, outer retina, and choriocapillaris layers in the left eye (Fig. ). The Humphrey visual field (central 24–2 threshold test) showed massive visual field damage in the left eye. The amplitude and implicit times of pattern visually evoked potential (VEP) and flash VEP were within the normal range in the left eye. The amplitudes of dark-adapted flash electroretinography (ERG) were slightly low in the left eye. Photopic single-flash response showed decreased amplitude of a-wave and b-wave in the left eye. The amplitudes of photopic 30-Hz flicker were decreased in the left eye. Trace arrays showed subnormal multifocal electroretinography (mERG) with decreased amplitudes for the left eye. The three-dimensional topography map showed a blunted foveal peak (Fig. ). |
A 35-year-old African American man was initially sent to our emergency room to evaluate a possible head trauma after a witnessed fall. He complained of frequent falls and leg weakness for 3–4 months. The weakness appeared to be progressive and persistent without episodic worsening. He had to use a cane to compensate but was still able to ambulate. His family members also endorsed cognitive defects over the past few months before admission. These impairments consisted mainly of apathy and withdrawal from social interactions; he used to be fully independent but now stayed at home most of the time. His medical history was only significant for alcohol abuse (three to four cans of beer and red wine daily for 2 years), and his family history was unremarkable.\nPhysical examinations showed an age-appropriate, alert, and oriented man. He was able to engage in conversations with a paucity of speech and flat affect; he also made eye contact and followed all commands during our evaluations.\nThere was bilateral weakness with spasticity in all lower extremity muscle groups. The deep tendon reflex was hypoactive with an upward response of the plantar reflex on the left side. He also had a wide-based unsteady gait and poor limb coordination. The remainder of the neurological examination and a general examination were unremarkable.\nInitial brain computed tomography (CT) in our emergency room demonstrated a mass lesion occupying the left caudate and extending into his frontal lobe (Fig. ). A brain MRI showed bilateral and symmetric hyperintense signals in the corpus callosum, periventricular white matter, and internal capsule (Fig. ) with an axial fluid-attenuated inversion recovery (FLAIR) sequence with gadolinium enhancement (Fig. ). A cervical spine MRI was negative for cord compression and abnormal signals.\nRoutine blood tests, as well as human immunodeficiency virus (HIV), syphilis, and thyroid function tests, were unremarkable, and his serum vitamin B12 level was borderline (289 picogram per milliliter). A cerebral spinal fluid sample revealed a mildly elevated protein level (120 mg per deciliter) with a normal cell count (two cells per milliliter) and glucose level (79 mg per deciliter). The sample was negative for cytology, oligoclonal bands, culture, and Epstein–Barr virus DNA polymerase chain reaction (PCR). His urine toxicology panel was negative for common recreational drugs.\nThe initial impression was an acquired demyelinating white matter disease due to nutritional deficiency. A trial vitamin supplement (500 mg administered intravenously three times a day and 1000 μg of cyanocobalamin administered intramuscularly daily) was given based on his alcohol abuse history, but no symptom improvements were seen during the following week. An inherited condition was thus suspected given the negative workup, the non-response to treatment, and the symmetric involvement in the brain MRI. This suspicion was validated by his peroxisomal fatty acid profile (Table ), and a diagnosis of X-ALD was confirmed according to the identification of a pathogenic mutation in the ABCD1 gene: c.1489 2A>G (g.153005544). Adrenocortical insufficiency was not identified during the evaluation. No seizure activity was witnessed or shown on video-electroencephalography.\nHis neurological condition deteriorated rapidly. He progressed into a non-verbal, quadriplegic patient dependent on a ventilator within 5 months of the initial encounter, and after 1 year of follow up he had degenerated into a vegetative state. Genetic counseling was offered to his family, and all of his siblings were negative for the ABCD1 gene mutation. |
A 27-year-old female patient with a known case of triple-negative breast cancer admitted to the emergency room complaining of documented fever 40°C at home which was relieved with an antipyretic. She was status post 4 cycles of neoadjuvant dose-dense AC regimen which consists of doxorubicin 60 mg/m2 and cyclophosphamide 600 mg/m2 administered every 14 days. She was prescribed primary prophylaxis filgrastim after all cycles. Physical examination was unremarkable apart from her fever. The patient was admitted to the hospital having febrile neutropenia with no focus of infection and started on empiric antibiotics and filgrastim. She had a low white blood cell (WBC) count with an absolute neutrophil count (ANC) of 1100 cells/microlitre on day 11 after cycle 4 despite being on appropriate filgrastim dose at 300 μg per day for 9 days. On the second day of her admission, she recovered from neutropenia but continued to have persistent high-grade fever for almost two weeks despite escalation of the antibiotics and addition of an antifungal agent. She underwent series of investigations to identify the cause of her unexplained fever. She had extensive blood and urine cultures after each spike of fever, which all came back negative. Computed tomography (CT) scan ruled out infectious focus and showed hepatosplenomegaly with multiple splenic hypodensities and minimal perisplenic fluid which did not appear in the baseline scan (Figures and ). Infectious disease team advised for a splenic biopsy which showed splenic infarction only with no evidence of bacterial, fungal, viral, or malignant involvement (). She also underwent an echocardiogram study, sinoscopy, and series of rheumatologic investigations that were normal. General surgery team was consulted and did not recommend any surgical intervention since the follow-up CT scan turned out negative for splenic abscesses with interval improvement in the previous splenic wedge-shaped hypodensities (). Eventually, the patient was discharged on oral antibiotics with infectious disease and oncology clinic follow-up after being afebrile and asymptomatic for more than 72 hours. |
A 63-year-old man with progressive vertical deviation of the right eye presented to our strabismus department (), noting that the ocular motility problem had been present for the past 6 months and was not associated with pain. The patient had no past medical history or systemic disease.\nPhysical examination indicated that corrected visual acuity and intraocular pressure were 20/20 and 18 mmHg, respectively, in both eyes. There was a 3mm-proptosis of the right eye compared with the left eye, and the upper and lower eyelids were slightly edematous, but with no chemosis or hyperemia; pulsation or bruit was not noted. He had 60PD of right hypertropia at both distance and near in primary gaze. He could fix objects with his right eye only when his left eye was occluded. () Significant limitation of his downgaze was noted. ()\nOrbital magnetic resonance imaging (MRI) studies revealed fusiform enlargement of the right superior rectus muscle, with prominent but irregular enhancement following gadolinium administration. () All other structures in the right orbit were normal except an incidental middle cranial artery (MCA) aneurysm in the left hemisphere, which was clipped by a neurosurgeon; the neurosurgeon indicated that the MCA aneurysm was not associated with the tumor.\nA biopsy through the infrabrow was performed under general anesthesia with a subperiosteal approach to the midsuperior orbit. Upon incision of the superior periorbita and levator muscles, the right SR appeared considerably thickened, and was dark purple and tense; the tendinous region was normal. An incisional biopsy was taken 15 mm behind the insertion of the muscle. The patient underwent a right superior rectus recession of approximately 15 mm by a hang-back technique and a 4-mm right inferior rectus resection with a conjuctival approach. The elasticity of the right SR was markedly reduced, and posterior portions were two or three times thicker than their normal size. Light microscopy of these sections revealed several blood-filled vessels of variable size surrounding muscle fibers and islands of fibrofatty deposits. () Capillary proliferation was not present, and there was no evidence of fibrotic intimal thickening or mitotic figures. An immunohistochemical study with antismooth muscle actin antigen (clone 1A4 at 1/25 titration, DAKO monoclonal, Denmark) demonstrated the presence of smooth muscle cells in the walls of blood vessels. () Biopsy results revealed an intramuscular hemangioma in the superior rectus muscle with cavernous-type vessels.\nSystemic corticosteroids were utilized for four weeks postoperatively to ameliorate swelling, not to shrink the tumor. Three months after surgery there were no signs of tumor progression. Mild ptosis and 15 PD of right hypertropia has since been noted in the patient's primary gaze. |
In October 2010, a 16-year-old girl was referred to our maxillofacial radiology clinic for radiographic examination for orthodontic treatment. Accidentally, in the panoramic radiograph, a unilocular mixed lesion was detected in the left mandibular angle.\nThe posterior borders of the lesion were poorly-defined; whereas, the anterior borders were ill-defined. In the panoramic view, intra-lesion calcifications were evident (). In the panoramic radiography and lateral cephalography, a mild expansion was observed in the posterior and inferior borders of the mandibular angle (). The mandibular cortical borders appeared completely intact. Root resorption was not present on any of the teeth adjacent to the lesion. The approximate dimensions of the lesion were 3 × 5 cm. The patient had no noteworthy medical history.\nShe did not experience pain in the region, and the lesion was non-tender on palpation. On clinical examination, a slight swelling was observed in the left mandibular angle. No enlarged lymph nodes were detected on palpation.\nOn intra-oral examination, the overlying mucosa of the region was smooth and of normal color.\nTo localize the intraosseous lesion and perform further investigations on the effects of the lesion on the surrounding structures, we performed cone beam computed tomography.\nOn CBCT examination, a multilocular lesion with coarse septa, without sclerotic borders was observed.\nThe cortical borders of the mandible and follicular space of the unerupted left third molar were normal. No displacement and root resorption was found on the teeth adjacent to the lesion ().\nMoreover, no displacement in the inferior alveolar nerve canal position was noted, but the cortical borders of the canal were thinned ().\nBased on age, clinical behavior, and radiographic findings, the differential diagnoses of fibrous dysplasia and desmoplastic ameloblastoma were made. Incisional biopsy of the lesion was performed.\nHistologic examination of the lesion revealed sheets and islands of large eosinophilic cells with abundant granular cytoplasm with small islands of odontogenic epithelium (). Foci of dystrophic and cementum-like calcifications were detectable in the lesion (). The fibrous stroma contained fibroblasts, and in the decalcified sections, bone trabecules were evident (). Histologic examination confirmed the diagnosis of central granular cell odontogenic tumor (CGCOT). Resection of the lesion was carried out to treat the patient. For mandibular reconstruction, allogen graft from the fibula was applied (). Histopathological examination of the excised specimen confirmed the diagnosis of CGCOT.\nThe patient is on follow-up periodic check and there has not been any radiographic evidence of recurrence on the follow-up radiograph after 2 years of operation (). |
A 44-year-old female patient, whose medical history included Crohn's disease since 2003, was admitted to our hospital for squamous cell carcinoma of the anal canal which was accidentally discovered during a consultation to control her Crohn's disease. The patient's management was delayed due to the confinement of the COVID-19 pandemic. A physical examination showed a painful mass in the external anal sphincter and extended to the gluteal muscle. The performance status was at 2–3.\nPelvic magnetic resonance imaging (MRI) showed an ulcerated anoperineal tumor process in hypo signal on T1-weighted sequences, a hyper signal on T2-weighted sequences, and intensely enhanced after injection of gadolinium. This process is locally extended in gluteal soft tissue with mesorectal nodules, cutaneous nodules of the sacral region, and bone lesions of the left greater trochanter. Total body computerized tomography was performed and no systemic involvement was noted. Colonoscopy revealed no synchronous tumor.\nThe case was discussed in a multidisciplinary consultation meeting. A concomitant radiochemotherapy treatment has been retained. Treatment was then initiated with combined chemoradiation therapy with Capecitabine 825 mg/m2 twice daily, 5 days per week, 2 piles of 500 mg in the morning and 2 piles in the evening for a body surface of 1.28, cisplatin 80 mg/m2 with a dose reduction of 20% given the performance status at 2, and a total dose of 80 mg every 28 days. And external radiotherapy total dose of 60 Gy, 2 Gy/fraction, and 5 fractions/week.\nDuring the treatment, the patient presented with a 3 days history of headaches. Cerebral MRI was performed and revealed an important heterogeneous metastatic lesion in the left parietal and frontal bone extended to the adjacent soft tissue of the scalp (shown in Fig. ). Histopathology confirmed the cerebral lesion to be a poorly differentiated squamous cell carcinoma, consistent with the known primary tumor of the anal canal. The patient underwent total brain external radiotherapy, a total dose of 20 Gy, 4 Gy per fraction, and 5 fractions per week. The patient succumbed to systemic complications of the disease during her treatment. |
A 45-year-old multiparous Taiwanese woman had undergone abdominal total hysterectomy for adenomyosis and endometrioma at a local hospital 53 days prior to admission. Details of the surgery were not known. She had an uncomplicated postoperative course. One day prior to admission at Chang Gung Memorial Hospital, Linkou, Taiwan, she experienced lower abdominal pain and vaginal discharge after sexual intercourse. There was no history of fever, chills, dysuria, and fecaloid discharge from her vagina. She was evaluated at our emergency department where a pelvic examination showed bowel content protruding into her vagina with some purulent discharge. Her general condition was stable with normal results of hematology and biochemistry tests. With the clinical impression of complete VCD with cuff wound infection, she was planned for laparoscopic surgery.\nA standard four-port laparoscopy was performed with the umbilicus as the primary entry site using an 11 mm primary port and three 5 mm lateral accessory ports. On intraoperative examination, there were moderate adhesions between the left pelvic side wall and colon. There was a short loop of ileum protruding through the cuff which looked viable with no signs of ischemia or perforation (Fig. ).\nAfter a thorough survey of her peritoneal cavity, adhesiolysis was done and the loop was carefully extracted from the cuff using atraumatic graspers. There were pockets of pus collection in the cul-de-sac that were cleared after adhesiolysis of the bowel loops. Complete dehiscence of the vaginal cuff was noted measuring approximately 5 cm. The cuff margins appeared inflamed. Careful downward dissection of her bladder and posterior peritoneum was carried out to achieve an adequate margin of vaginal cuff. Approximately 0.5 cm of cuff margin was excised circumferentially leaving viable tissue for proper suturing (Fig. ).\nThe vaginal cuff was closed with a running Monocryl suture number 1–0 (poliglecaprone 25) with a depth of 1 cm vaginal cuff margin. Copious irrigation of her abdominopelvic cavity with saline solution was performed. A Jackson-Pratt drain was placed. Her postoperative course was uneventful. Intravenously administered antibiotics were completed and the drain was removed on the fifth postoperative day. She was followed up 2 weeks after discharge from our hospital: her pelvic examination revealed that the vaginal cuff was healing well. At a 1-month follow-up, her vaginal cuff was completely healed. |
A 41-year-old woman with asthma (well-controlled on inhalers), hypothyroidism (controlled on thyroxin), and gastroesophageal reflux disease presented to the rheumatology clinic at the Royal Hospital with a one-month history of bilateral swelling of the forearms with skin tightness and fingers contraction. She had no constitutional symptoms or history of Raynaud’s phenomena, weight loss, or change in her bowel habits. Physical examination revealed edema and hardening of the subcutaneous tissue of the forearm. The skin of both forearms showed a linear depression along the course of the superficial veins consistent with groove sign. She was unable to flex or extend her fingers and to make a fist or hold objects well. The skin over her fingers and palms was normal. Her face was unaffected. There were no clinical features suggestive of malignancy or infection. Laboratory tests revealed raised eosinophil count of 1.8 × 109/L (normal range 0–0.5 × 109 g/L). Her level of C-reactive protein was mildly raised; creatine kinase and erythrocyte sedimentation rate (ESR) were normal. Rheumatoid factor, anti-nuclear antibody, and extractable nuclear antigen and lactate dehydrogenase were negative. Full-thickness biopsy of the skin and muscles of the forearms showed inflammatory process involving the interstitial tissue in and around the skeletal muscle along with occasional muscle necrosis and some regenerative fibers with an increased number of eosinophils in the fascia fibroconnective tissue [], which confirmed EF. Contrast MRI revealed extensive bilateral enhancing thickened fascia between the muscles of the forearm []. She was started on oral prednisolone 0.75 mg/kg for four weeks, which was subsequently slowly tapered. She made a remarkable response with reduced limb swelling and normal mobility. Unfortunately, the disease relapsed on tapering and high dose prednisolone was restarted along with adding oral methotrexate 20 mg per week. Her disease responded well to treatment; however, she was lost to follow-up and stopped the medication resulting in recurrence of her disease. |
The patient was a 37-year-old married Japanese woman. She was referred to the Department of Psychiatry at the Toho University Omori Medical Center, Tokyo, to receive care for a severe postpartum depressed mood and intense suicidal ideations. The participants provided written informed consent prior to enrollment in this case report.\nShe had no previously documented psychiatric history and no documented family history of psychiatric or perinatal illness. She had been brought up in an urban environment since childhood. She had a good relationship with her parents and did not experience any abuse or maltreatment. Her character was honest, diligent, and orderly, and she had an especially strong sense of responsibility. After graduating from university, she worked as an assistant curator in a museum. At the age of 34 years, she met and married her husband, who was an engineer. They were not eager for her to become pregnant. Although she and her husband moved to the countryside because of his work, she decided to continue her job because, despite a 3-hour commute, she found her work to be very fulfilling.\nAt the age of 36 years, she became pregnant. She quit her job to become a housewife and became bored with her daily life; she also felt that living in the countryside was inconvenient. Her husband was busy with work and left all the pregnancy preparations to her, which caused her to feel frustrated. She started feeling very anxious about her primiparity and child-care, and she moved to her parents' house to receive their support. During her 39th week of pregnancy, she had a forceps delivery because of a birth canal infection. The delivered boy was 3150 grams and had no deformities of any kind. However, she could not hold her child immediately after childbirth because she was receiving treatment for her infection. She appeared to lack affection towards her baby because she could not remember how she felt when she eventually held her baby for the first time.\nIt was difficult for her to control her baby when he cried, and she felt fatigued and anxious. He was very demanding for his mother's breast milk, and she continued to feed him because of a feeling of responsibility. A month after the delivery, depressive symptoms (depressed mood, abnormal fear, and insomnia) appeared and gradually worsened. She felt a strong sense of distress while she was with her baby and was confused as to how to care for him. She had difficulty asking for help because she felt that she should do everything on her own. She began to regret having given birth because it had led to her present circumstances. Her husband was still unable to provide her with either emotional or physical support, but her mother continued to help her care for her baby. The patient's lack of affection worsened and was accompanied by other depressive symptoms, such as poor concentration, indecisiveness, and reduced energy. The patient began to worsen daily and began to have difficulty taking care of both herself and her baby.\nThree months after the birth, she visited a psychiatric outpatient clinic and was diagnosed as having postpartum depression and bonding disorder. Her Temperament and Character Inventory (TCI) scores were as follows: Novelty Seeking (NS): −1.78; Harm Avoidance (HA): 2.52; Reward Dependence (RD): −2.09; Persistence (PS): 1.78; Self-directedness (SD): −2.1; Cooperativeness (CO): −1.35; and Self-transcendence (ST): −0.54. Her temperament was judged as logical and obsessive-compulsive, and her character was judged as melancholic and schizoid.\nTreatment with an antidepressant (sertraline, 25 mg/day) and psychotherapy was started, but her depressive symptoms worsened. Because of strong nausea, the treatment with sertraline was discontinued and treatment with mirtazapine (15 mg/day) and olanzapine (initial dose, 2.5 mg/day) was started. Finally, she was admitted to the Department of Psychiatry at the Toho University Omori Medical Center.\nOn admission, her Hamilton Rating Scale for Depression (HRSD) score was 35. She was characterized as having a depressive mood most of the day, markedly diminished interest and pleasure, insomnia, psychomotor agitation, loss of energy, feelings of worthlessness, indecisiveness, suicidal ideation, and a suspicious attitude. We diagnosed her as having peripartum-onset major depression, and we gradually increased the dosage of mirtazapine up to 45 mg daily and that of olanzapine up to 10 mg daily, with poor results. Therefore, nortriptyline was prescribed along with these drugs. After the dosage of nortriptyline was increased to 100 mg, her depressed mood and indecisiveness began to improve at around day 45 of her hospitalization. She was allowed to stay overnight at her house, with her child, on day 53. However, her depressed mood and anxiety were suddenly exacerbated when she returned to the hospital. Hence, we started a series of electroconvulsive therapy (ECT) treatments beginning on day 72 and her depressive state improved somewhat.\nShe made comments such as “Even though I should have been happy to have been with my child, it did not go so well emotionally” and “I'm convinced that I'm incurable.” Her score on the Mother to Infant Bonding Scale (MIBS), which is a 10-item self-reported instrument, was 12, indicating a severe bonding disorder []. We focused on the bonding disorder and provided Kangaroo Care during family sessions. We started providing Kangaroo Care with her baby for two hours in a private room of the ward while her husband was present. During the Kangaroo Care session, we facilitated skin-to-skin contact and educated her regarding parenting behavior in cooperation with the ward nurses. Two sessions were provided, and similar follow-up care was subsequently provided by midwives. She learned to recognize her baby's gestures, facial expressions, and emotions. She was transfixed and embarrassed when the baby was crying, and she barely smiled at the baby during the first session. To allow the patient to gain self-confidence, we first had the patient hold her baby while he was in a good mood. We repeatedly showed her how to cope with the baby's discomfort in a concrete manner and told her that her baby was very cute to improve her capacity to verbalize her emotions. Her husband was encouraged to send her photos and movies of her child taken at home every day based on the policy of imaginary exposure. After this intervention, her awareness of her child's feelings and her ability to provide flexible care for her baby improved.\nThe patient began to experience tender feelings towards her child and her anxiety decreased (HRSD = 7). She tried staying at home overnight, and her depression and bonding problem did not worsen. On day 114, she was discharged from the hospital. She was taking maintenance doses of 100 mg of nortriptyline, 30 mg of mirtazapine, and 5 mg of olanzapine per day at the time of her discharge. We continued outpatient treatment and supported her in caring for her child. In addition, we liaised with midwives, public health nurses, and home visit nurses regarding her care and ideal environment after discharge. At 9 months after her discharge, her MIBS score was 5 (). A tendency towards an improvement in her bonding disorder was seen, and her depression remained in remission (HRSD = 6). |
An otherwise healthy 33-year-old woman, gravida 3, para 2, from a Sephardic Jewish origin, was initially referred to our institution at 30.6 weeks of gestation due to a large neck mass found on prenatal ultrasonography (US). Her previous two pregnancies were uncomplicated. The fetal sonogram showed a 10 by 8 cm mass on the right side of the neck, which was not present in detailed scans taken at 14 and 22 weeks. The mass was composed of a cystic portion and a solid portion containing blood vessels and was growing rapidly in subsequent ultrasound studies. A significant polyhydramnios with amniotic fluid index (AFI) of 50 suggested an upper gastrointestinal obstruction and a highly possible airway obstruction as well. Findings were confirmed by fetal magnetic resonance imaging (MRI). In anticipation of the difficulty in establishing a secured airway at birth and the potential complicated resection of the giant tumor after birth, the mother was referred to our hospital for consultation.\nThe parents were in consultation with the maternal fetal team, neonatologist, anesthesiologist, pediatric surgeon, and otolaryngologist. The parents were presented with a guarded prognosis but insisted that the pregnancy continue with maximal efforts during delivery and during the neonatal period.\nTherefore, a planned EXIT procedure, which provides the best chance to establish a patent airway, was offered to our patient, presenting the risks []. Specifically, we informed the parents about the risks for the mother, including significant hemorrhage from the uterus due to the uterine relaxation necessary to avoid placental separation, with a possible uterine resection in the case of a life-threatening hemorrhage.\nKnowing the risk of an unplanned preterm delivery due to polyhydramnios and uterine contractions, we scheduled our patient for a planned cesarean delivery at 34 weeks organizing and preparing a multidisciplinary team ready to perform the EXIT procedure.\nA multidisciplinary team including obstetricians, anesthesiologists, neonatologists, otolaryngologists, pediatric surgeons, pulmonologists, cardiologists, and nursing staff participated. A combined epidural and general anesthesia was planned. Our patient’s blood pressure was monitored continuously to detect and treat a possible event of maternal hypotension in order to maintain good fetal perfusion. After epidural catheter placement while lying on her left side, the parturient returned to lie on her back. Immediately, a severe hypotension (65/30 mmHg) with tachycardia (150 beats/minute) appeared. We related this complication to the polyhydramnios causing a significant decrease in the vena cava flow. After left uterine displacement and bolus of phenylephrine, her blood pressure and heart rate returned to normal. General anesthesia with rapid sequence induction was induced with succinylcholine (100 mg) and propofol (150 mg). During the EXIT procedure an appropriate uterine relaxation was maintained to prevent placenta expulsion. We used a high dose of inhaled anesthetics and minimal nitroglycerine intravenous drip. No bleeding occurred during the procedure.\nA classical uterine incision was made and only the fetal head and upper chest with the cervical mass were delivered through the uterine incision. The rest of the body and the cord were left in situ to avoid placental separation. The amniotic fluid was slowly drained to avoid an abrupt drainage of the fluid and an early separation of the placenta.\nThe multidisciplinary team had planned and rehearsed the following escalating step-by-step scenarios for establishing a secured airway: (1) direct laryngoscopy and intubation attempt by a pediatric anesthesiologist with the aid of a neonatologist, (2) rigid bronchoscopy by an otolaryngologist and possible aid of flexible bronchoscopy by a pediatric pulmonologist, (3) if laryngoscopy and bronchoscopy failed, a tracheostomy was planned by an otolaryngology team. As the tumor was highly vascularized, any debulking procedure was impractical and would have imminently put both the mother and newborn at the risk of death. Analgesia for the newborn using intramuscular fentanyl was prepared in advance.\nAfter the head of the newborn emerged, a direct laryngoscopy was attempted; however, the larynx was not visible as the tumor obstructed the pharynx. Attempts at direct intubation were abolished after 1 minute. Next, a rigid bronchoscopy was performed and only the tip of the epiglottis was visualized. At 11 minutes from delivery, endotracheal intubation was successfully performed. From the time of delivery to the time of intubation, the presence of a good heart rate of the fetus was monitored by echocardiography. After the airway was secured, the female newborn was delivered, the cord was clamped, and the placenta extracted. The arterial cord pH was 7.01 with CO2 of 71 mmHg and lactate of 9.5 mmol/L. Immediately after delivery, a computed tomography (CT) scan was performed under general anesthesia that demonstrated that a tracheostomy could be performed without interfering with the tumor. During the tracheostomy, a biopsy was taken from the tumor that demonstrated both mature and immature teratoma (Fig. ).\nThe birth weight of the newborn girl was 3 kg including the tumor. A giant submental mass that protruded from the right side of her neck was noted. The tumor was covered with a thin skin layer with a large cystic and solid appearance. There were occasional bleedings from several lacerated areas on the tumor surface (Fig. a). A CT study demonstrated the abundant blood supply of the tumor including the fact that the right carotid artery was supplying this tumor (Fig. b). During the first 2 days of life, before the next procedure, the tumor continued to grow significantly, probably partially due to internal bleeding. Her heart function was normal but prerenal azotemia evolved due to loss of large amounts of serotic fluid as well as blood from the lacerated mass.\nBecause resection of the giant, highly vascularized, cervical teratoma could be a fatal procedure due to massive bleeding, we used endovascular embolization a day prior to the tumor resection. The embolization was done by a pediatric cardiologist and an interventional neuroradiologist. Cervical angiography via a femoral line catheter demonstrated the highly vascularized tumor supplied by the external right carotid artery (Fig. a). A carotid occlusion test was performed and showed good blood supply from the contralateral internal carotid artery (ICA). Next, the origin of the external carotid was occluded using detachable platinum coils. Since resection of the tumor implied the sacrifice of all cervical carotid branches it was mandatory to also occlude the cervical ICA and common carotid artery to achieve minimal blood loss during surgery (Fig. b). The procedure was done under general anesthesia.\nUpon withdrawal of the angiography catheter a sudden deterioration of our patient was noted. Fluoroscopy and echocardiography demonstrated cardiac tamponade, probably from a small laceration in the aorta. An urgent pericardiocentesis retrieving 3 ml of blood from the pericardial space allowed fast and full hemodynamic recovery (Fig. c, d). There was no re-accumulation of pericardial blood.\nLess than 24 hours after the embolization, the surgical team, which included a head and neck surgeon, pediatric otolaryngologist, and a plastic surgeon, performed the surgery with a pediatric anesthesiologist. Using LigaSure™ Sealer/Divider (Medtronic), the tumor was dissected including a section of the lacerated skin. Because the main blood supply of the tumor was embolized, the surgery was performed from its distal part along our patient’s mandible towards the proximal part at the junction of her neck and thorax. The tumor impinged into her pharynx, and her larynx and epiglottis were identified and preserved. Her mandible and neck muscles were preserved; however, her vagal nerve was surrounded by the tumor and was sacrificed with the carotid artery. The tumor weighted 800 grams and the overall blood loss was 300 ml. After tumor resection and closure of the skin (Fig. ), the tracheostomy tube was changed. This was followed by a pneumothorax that required a chest tube insertion.\nGradually, after the tumor resection, our patient was weaned from mechanical ventilation. She was fed initially using an orogastric tube and gradually learned to feed orally. After the resection, a right vocal cord paralysis was observed using a flexible fiberoptic laryngoscopy; therefore, we decided to leave the tracheal tube in place. We attributed this finding to an injury of the recurrent laryngeal nerve during the resection. Another neurological sequela was an abduction weakness of her right shoulder which slowly recovered. Neck teratomas can arise from and completely replace the thyroid tissue []. Thyroid function tests demonstrated hypothyroidism and she started receiving thyroid replacement therapy. A follow-up ultrasound of her head was normal. A brain MRI done a month after the final surgery demonstrated normal brain appearance. She was discharged to her home at 3 months of age (Fig. ). |
The second patient was an 84-year-old male who developed an erythematous nodule on the proximal phalanx of the left ring finger, which had suddenly increased in size. A biopsy confirmed MCC, which was positive for MCPyV (). Given that the surgical margins were not clear after local excision, the decision was made to amputate the digit. In light of the patient’s overall condition and co-morbidities, a sentinel lymph node biopsy was not performed. The patient did not undergo adjuvant radiotherapy.\nThe patient had been diagnosed with seropositive RA by a rheumatologist some 5 years previously. The RA had initially been treated with intermittent courses of systemic CSs. His symptoms included pain and swelling in the fingers, particularly affecting the MCP joints. MTX (10 mg s.c., weekly) had been initiated 5 months before the diagnosis of MCC by his rheumatologist owing to persistent and significant disease activity, reflected in elevated serum CRP concentration.\nSeven months after the diagnosis of MCC was made, the patient developed a s.c. swelling on the dorsal aspect of his left wrist, which was surgically excised. Histology confirmed an MCC metastasis, which extended to the surgical margins. Whilst re-excision was being planned, the patient developed a further 3 cm × 4 cm nodule in the left antecubital fossa, radiologically consistent with a metastasis. Further radiological staging showed no evidence of visceral or bony metastases.\nThe patient was referred to our department to assess suitability for systemic anti-tumour therapy, given both the rapid disease progression and the persistence of tumour in the surgical margins. After discussion in the interdisciplinary tumour board, the patient’s immunosuppressive therapy was discontinued and therapy with avelumab initiated (10 mg/kg, fortnightly).\nInitial staging after 3 months of treatment revealed complete remission of the MCC metastasis over the dorsal aspect of the left wrist and a significant reduction in the size of the metastasis in the antecubital fossa. The decision was made to continue the treatment. One month later, the patient experienced a significant relapse of his RA, with erythema, swelling and pain affecting the MCP joints, in the context of an anti-PD-L1 immune-related adverse event. Prednisolone (10 mg) daily was commenced. Routine staging investigations 3 months later revealed a recurrent metastasis over the left humeral epicondyle, compressing the basilic vein. In the absence of further metastases, radiotherapy was initiated (50 Gy). Treatment with avelumab was recommenced because the patient’s RA was no longer symptomatic despite tapering and withdrawal of prednisolone therapy.\nHowever, shortly after recommencing avelumab the patient again experienced a flare of his RA, with the same rheumatological symptoms. After consulting his rheumatologist, the patient began treatment with MTX (10 mg. s.c, once per week) and avelumab treatment was temporarily interrupted. Unfortunately, the metastasis over the left humeral epicondyle increased in size, prompting surgical removal. Despite having experienced two flares of his RA during avelumab therapy, the patient was keen for avelumab to be re-introduced under ongoing MTX therapy. Routine staging investigations 3 months later revealed multiple pulmonary () and lymph node (mediastinal and left axillary) metastases. MTX treatment as withdrawn in view of the development of widespread metastases. Continued administration of avelumab resulted in a complete remission of the MCC, with no evidence of distant () or local disease recurrence for >8 months. To date, there have been no other immune-related adverse events and no further exacerbations of the patient’s RA. |
A 38-year-old male was initially seen by the ophthalmology service due to blunt trauma to the right eye secondary to assault with a metal pipe. On initial consultation in the hospital, he was diagnosed with a right-sided closed orbital wall fracture, traumatic iritis with microhyphema, and a choroidal rupture in the right eye. Vision at that time was hand motion, and intraocular pressure (IOP) was 44 mmHg through rebound tonometry. After initial treatment with topical steroids, his visual acuity improved from hand motion to 20/60 over the course of 2 months. During his recovery, the patient was noted to have persistent hypotony and macular folds []. No cyclodialysis cleft was initially seen, although he was persistently dilated on cycloplegia for the iritis during initial gonioscopy. In addition, his IOP was noted to be consistently lower than the left eye, with at least a 5 mmHg difference each visit, and ranged between 6 and 11 mmHg. A month after his initial injury, choroidal folds were noted on examination and on optical coherence tomography. Although his IOP at this time was 9 mmHg, the IOP in his left eye was 20 mmHg.\nThree months after his initial injury, he was referred to the retina service for the evaluation. Best-corrected visual acuity (BCVA) was 20/60 in the right eye, IOP was 11 mmHg in the right eye, and 20 mmHg in the left eye. Examination of the anterior segment revealed a shallow anterior chamber, and undilated gonioscopy revealed a 5-clock-hours cyclodialysis cleft located temporally. The patient was then referred to the glaucoma service for cyclodialysis cleft closure. Initial surgical repair was performed by direct cyclopexy through scleral flaps with 10–0 prolene suture. Afterward, a small area (<1 clock h) of open cleft was still noted superotemporally, which did not close after application of argon laser. A repeat direct cyclopexy was performed a month after initial surgery with 9–0 prolene suture, but was unsuccessful in relieving his hypotony.\nGiven his refractory course, he was referred to another glaucoma specialist for evaluation and treatment. Ultrasound biomicroscopy (UBM) examination revealed shallow choroidal serous detachments with an atrophic, detached ciliary body processes corresponding to the location of the cyclodialysis cleft. Gonioscopic examination showed an area concerning for residual cleft opening between 7 and 8 o'clock, but closure of the cleft was confirmed with pressure on the globe during UBM []. His hypotony was thought to be secondary to infarcted ciliary body processes secondary to detachment. Due to the cause of hypotony, the glaucoma specialist suggested phacoemulsification with capsular tension ring (CTR) insertion with the IOL, and pars plana vitrectomy with gas tamponade to bolster and reattach the ciliary body as a more heroic measure.\nA month afterward (over 3 months since the initial injury), a combined phacovitrectomy with IOL, CTR implantation, and gas tamponade with 22% sulfur hexafluoride (SF6) was then performed. During phacoemulsification, zonular instability was noted in areas associated with the cyclodialysis cleft. A MORCHER® Type 14C CTR (FCI Ophthalmics, Pembroke, MA, USA) was inserted into the capsular bag to provide capsular support, and an +22.50D SN60WF acrylic monofocal IOL (Alcon, Fort Worth, TX, USA) was placed in the capsular bag without complication. The diopter was chosen based on the calculations and axial length of the normal contralateral eye. A complete vitrectomy was performed, with induction of a posterior vitreous detachment and vitreous skirt shaving. After air-fluid exchange, the air was exchanged with 22% SF6 in the vitreous cavity. He was then instructed to remain supine for a week. Postoperatively, the patient had short-term ocular hypertension above 30 mmHg that was well controlled with topical therapy and oral acetazolamide. A little over 1 month after the surgery, the hypotony resolved in the right eye, with normalization of the anterior chamber and ocular hypertension at 28 mmHg on topical steroids. His topical steroids were tapered off over the next 2 weeks, and his pressure decreased to 14 mmHg without any topical aqueous suppressants. Three months after the surgery, his BCVA improved to 20/25 (requiring +0.50 D correction) and his IOP was 14 mmHg without any medications, with some resolution of his choroidal folds. His cyclodialysis cleft was noted to be closed on gonioscopy at this visit. A year after his last surgery, BCVA remained 20/25 without any complications, with a measured IOP of 18 mmHg off all drops. No lens decentration or dislocation was noted at present. His choroidal folds were noted to have improved significantly, though not completely resolved []. |
A 52 years old male presented to the accident and emergency department of our institution with complaints a painful swelling over the right lateral malleolus for two days. () This swelling was localized to the distal end of a surgical scar from an open reduction and internal fixation of a Weber B ankle fracture 20 years ago.\nClinical examination revealed a 3x3 cm fluctuant swelling surrounded by soft tissue oedema and erythema over the lateral malleolus. The swelling was inflamed and tender. The erythema was limited to the lateral malleolus and did not extend proximally. The skin was intact. There were no palpable lymph nodes proximally. The ankle movements were significantly restricted due to the pain over the fibular end but the patient was able to fully weight-bear with some discomfort.\nThe patient was apyrexial and systemically well. His white cell count was normal. His CRP and urate were increased at 50 mg/L (normal <5) and 493 µmol/L (normal 200-430 µmol/L) respectively. Radiographs revealed no recent bony injury with intact screw and plate fixation over the distal fibula. Although there was significant soft tissue swelling over the lateral malleolus, there were no signs of osteomyelitis.\nThe patient was suffering from osteoarthritis of the ankle requiring non-steroidal anti-inflammatories as necessary and allopurinol 300mg once daily for hyperuricaemia. The patient had sustained a Weber B right ankle fracture 20 years ago, which was treated with a lag screw and a neutralization one-third tubular plate. Since the fixation the patient had experienced occasional mild discomfort but not requiring medical attention.\nBased on the clinical findings, an infection involving the implants was suspected and the patient was scheduled for incision and drainage of the removal of the implants.\nIntra-operatively, no pus collection was found, no signs of loosening of the implants and no infected bone. The deposit was evacuated, the soft tissues debrided and the implants removed. Samples of the material submitted for microbiology and histology were clear of microorganism but were positive for polymorphs and monosodium urate crystals.\nFollowing surgery, the wound healed well within two weeks. The patient was discharged and no complications were noted at 3 months post-operative follow-up. |
A 37-year-old male patient with a background of hypertension was admitted to our emergency department with sudden dizziness and left limb weakness. Non-jet vomiting occurred before transfer to our hospital for further management, and the vomitus was gastric content. He reported no trauma, fever, or coma, he was a non-smoker, and he had no history of drug use or cardiac events. The patient was immediately subjected to a non-contrast computed tomography (CT) scan of the brain, which revealed hemorrhage in the occipital and parietal lobes. The diagnosis of intracerebral hemorrhage (ICH) was made. He developed sudden onset chest tightness, shortness of breath, and occasional chest pain behind the sternum around 48 h after emergency hematoma evacuation. The pain was stuffy in nature and did not radiate to other places. His symptoms continued to worsen gradually, until breath sounds were observed when the patient was sitting up. There were no severe symptoms of dyspnea, cough, hemoptysis or sputum (a characteristic pink frothy sputum is often produced).\nThe patient’s medical history was only hypertension; he had not been treated regularly, and the blood pressure control was unknown. There was no other history of illness or medication.\nThe patient had warm extremities with hemodynamically stable blood pressure maintained at 128/73 mmHg and a heart rate of 77 beats per minute (bpm) on initial assessment. He was breathing smoothly on room air with clear lungs. His cardiac examination was normal and did not show any murmurs or gallops.\nThe physical examination was unremarkable, except for the low left limb muscle tension and the third limb muscle strength. The physical examination was carried out when the patient suffered from chest tightness. On further examination, his heart rate was faster than 100 bpm and his blood pressure had risen to 150/90 mmHg; other general conditions were stable. However, when the patient was breathing in a sitting position, auscultation revealed abnormal breath sounds, which manifested as obvious bilateral bubbling rales in the lower part of the lungs.\nThe serum level of creatine kinase (CK) was 837 U/L (reference range, 38-174 U/L), that of CK isoenzyme was 26 U/L (reference range, 2-25 U/L), lactate dehydrogenase (LDH) was 269 U/L (reference range, 109-245 U/L) and hydroxybutyrate dehydrogenase (HBDH) was 249 U/L (reference range, 72-182 U/L) at admission; other blood values were within the normal range. When the symptoms of chest tightness worsened, the patient’s blood was immediately checked for any sign of heart failure (HF). The laboratory examination showed the concentration of N-terminal pro b-type natriuretic peptide (NT-proBNP) was 493 pg/mL (reference range, < 85 pg/mL). Myocardial enzyme levels, including cTnI, did not exhibit normal values after serial determination: the cTnI level was increased to 0.175 ng/mL in the resting state (reference range, < 0.06 ng/mL), the CK level was 660 U/L, the CK-MB level was 26 U/L, LDH was 298 U/L, and HBDH was 271 U/L. Repeated measurements 5 d later showed the values had decreased: CPK, 49 U/L; CK-MB, 12 U/L; LDH, 231 U/L; and HBDH, 187 U/L (Figure ).\nOn admission, the emergency cerebral CT image revealed cerebral hemorrhage in the occipital and parietal lobes (Figure ), while the initial 12-lead ECG (Figure ) revealed a normal sinus rhythm and a regular rate within the normal range; however, T wave inversions and ST segment in leads V5 and V6 were already observed.\nWe made another ECG at the onset of chest tightness. It is notable that the ECG demonstrated extensive depression of the ST segment and T wave inversion, suggestive of myocardial injury. At this time, there was a prolongation of the corrected QT interval (QTc, 487 ms) (Figure ). But at this time, the chest radiograph showed no bilateral patchy opacities that are typical of pulmonary edema and consistent with the breath sounds when the patient was in a sitting position (Figure ).\nIn sum, the working hypothesis of a non-ST elevation myocardial infarction (NSTEMI) could be established. Further cardiological investigations, including coronary arteries with CT angiogram (CTA) of the coronary arteries, were needed to make a definite diagnosis. Considering the patient was in acute stage of ICH, coronary angiography was not timely completed.\nA coronary CTA and echocardiogram (ECHO) were made at 72 h after the initial symptoms when the patient’s chest discomfort disappeared. The ECHO revealed normal sized chambers with an ejection fraction of 60% and showed no significant valvular abnormality or any wall motion abnormality (Figure ). Coronary arteries did not reveal any sign of obstruction or significant narrowing (Figure ). Another 12-lead ECG was made 11 d after the patient’s symptoms improved, by this time, the patient had returned to the same state as at admission with the recovery of ICH, but the QTc interval was now prolonged (QTc, 424 ms). |
A 46-year-old man was transferred to our hospital for further evaluation and management of a gastric lesion that had been detected by gastroscopy during a medical checkup at a local clinic. The gastroscopy showed an approximately 2.5-cm sized submucosal mass with two active ulcers on the mucosal surface (). The diagnostic impression of the referring physician in the local clinic was a GIST or lymphoma. After a gastroscopic biopsy of the mucosal ulcer, the gastric lesion was diagnosed pathologically as an adenocarcinoma at this clinic.\nA contrast-enhanced CT of the abdomen using a 64-MDCT scanner (LightSpeed VCT, GE Healthcare, Milwaukee, WI) was performed at our hospital. The CT images revealed a 1.5 × 2.5-cm ovoid mass with contrast enhancement at the lesser curvature wall of the gastric high body near the cardia (). Because intact overlying mucosa was identified, the mass was initially thought to be a submucosal tumor such as a GIST. There was no evidence of perigastric infiltration, enlarged lymph nodes, or distant metastasis on CT. After reviewing the clinical information of the biopsy-proven adenocarcinoma, a gastric carcinoma was included in the differential diagnosis on the radiological report. However, because the enhanced mass in the submucosal layer was not the usual CT finding for a gastric adenocarcinoma, comparison with the clinical findings of gastroscopy and pathology was recommended.\nA total gastrectomy was carried out to remove the gastric mass. The resected specimen appeared as a 2.0 × 2.5-cm submucosal mass with small elevated lesions and central ulcerations on the mucosa. On light microscopy, poorly differentiated adenocarcinoma with extensive lymphocytic reaction in the mucosa that invaded the submucosa and subsequently formed a submucosal mass was confirmed (). The tumor invaded the subserosal adipose tissue, and was of the diffuse histological type (Lauren's classification). No lymph node metastasis was present. The presence of EBV in gastric tumor cells was confirmed by performing EBV-encoded RNA-1 (EBER-1) in situ hybridization. |
Our patient was a 47-year-old male with past medical history including aorto-occlusive disease status post femoral-popliteal bypass, with peripheral artery disease, coronary artery disease, and tobacco dependence. He initially presented with right groin and lower-extremity numbness with an otherwise unremarkable review of systems. The patient was diagnosed with right limb occlusion with critical limb ischemia of the right lower extremity due to an aorto-femoral bypass graft occlusion. Initial workup included a computed tomography (CT) angiogram of the chest, prior to treatment of the occlusion with a femoral-femoral bypass.\nComputed tomography angiography (CTA) of the chest revealed a 1.4 cm nodule at the left lung apex, slightly cavitary in nature together with a left paratracheal soft tissue density that was suspected to be adenopathy related to pneumonia that was being treated. The lesion was considered to be incidental with the recommendation of short-term follow-up with another chest CT in three months. There was no prior imaging for comparison.\nTwo months later, the patient presented to the emergency room with bilateral chest pain and associated shortness of breath and dyspnea. He was admitted to the intensive care unit (ICU) for respiratory instability and treated for multiple bilateral pulmonary embolisms. The diagnosing CTA of the chest showed an increase in the left upper lobe mass density with 2.5 cm x 2.4 cm dimensions including marked interval increase in diffuse mediastinal and bilateral hilar adenopathy involving levels T5, T10, and T11, suggesting a primary neoplasm with metastatic disease. The primary lesion was pleural based and thought to be invading the pleura. Once the patient stabilized, a CT-guided left upper lobe biopsy was obtained.\nBiopsy revealed a poorly differentiated non-small cell carcinoma consistent with squamous cell carcinoma. Sections showed nests and individual large cells with brisk mitotic activity with medium to large nuclei. There was considerable tumor necrosis. Immunohistochemical stains showed positive staining for p63 and negative for TTF1. Morphology and stains were consistent with squamous cell carcinoma of the lung. It was suggested that the pulmonary embolisms the patient experienced were attributed to a hypercoagulable state related to malignancy.\nOncologic positron emission tomography (PET)/CT scan suggested invasion of the pleura with perivascular and lymphatic metastatic involvement, confirming a hypermetabolic left upper lobe mass of 2.5 cm x 2.8 cm with SUV of 10.7 (Figures -) and hypermetabolic left hilar adenopathy (Figures -). Subsequently, a magnetic resonance imaging (MRI) of the brain was completed for evaluation of metastasis. A 6 mm ring-enhancing metastatic lesion was noted in the left frontal lobe with surrounding edema (Figures -). The imaging was otherwise unremarkable.\nThe initial treatment plan included a radiosurgery approach to the solitary brain lesion. The lesion was treated with five volumetric arc therapy (VMAT) beams with the isocenter located in the center of the lesion, which was contoured on the MRI images. A total peripheral dose of 2500 cGy delivered in five fractions was prescribed to the planning target volume (PTV). Dose-volume histogram (DVH) analysis of the target lesions showed dose statistics (minimum, maximum, and mean) of 2396.0 cGy, 2941.2 cGy, and 2759.3 cGy to the PTV, respectively. The total volume of the gross lesion and margins was 0.4 cc. The total volume of tissue receiving 100% of the total prescribed dose (V100%) was 0.5 cc. The patient did not receive any form of systemic therapy, such as chemotherapy or immunotherapy.\nPost-treatment MRI of the brain at one-month follow-up after the initial brain lesion diagnosis showed a reduction in size from the original 6 mm nodule (Figures -) on the stealth protocol study. Remarkably, repeat PET/CT four weeks post-treatment stereotactic radiosurgery treatment (SRS) revealed no appreciable mass in the left upper lobe (Figures -) with resolution of hypermetabolism. Fluorodeoxyglucose (FDG) activity in the left hilum gave SUV level 2.2-2.7, an improvement from the previous SUV of 9.3 (Figures -). Chest CT confirmed that there was no longer an appreciable left upper lobe mass. An additional follow-up chest CT was completed two months later (three months post-treatment) confirming complete resolution of the original left upper lobe pleural-based mass.\nThe most recent imaging at seven months post-treatment continued to remain free of residual left upper lobe and mediastinal masses on chest CT. Brain MRI showed complete resolution of the solitary metastatic focus in the left frontal region. There was no evidence of new metastatic disease. |
A 62-year old gentleman presented with sudden onset, severe generalised abdominal pain. He had normal vitals signs but on examination demonstrated generalised peritonism. Biochemistry showed lactataemia of 4.2 mmol/L but was otherwise unremarkable. Computer tomography (CT) imaging of his abdomen was equivocal for an early superior mesenteric artery dissection. Spontaneous and complete resolution of the patient’s symptoms occurred within an hour of presentation and he was admitted for monitoring. Twenty-four hours later the patient experienced the same severe, sudden onset, abdominal pain accompanied by hypotension and syncope. A CT mesenteric angiogram demonstrated two pseudoaneurysms arising from the splenic artery with associated rupture of the proximal pseudoaneurysm and retroperitoneal haemorrhage (). The proximal pseudoaneurysm was embolised by interventional radiology. Four hours later, the patient became haemodynamically unstable with a significant haemoglobin drop (24 g/L) requiring massive transfusion. Repeat CT mesenteric angiogram demonstrated new left gastric, short gastric and distal splenic pseudoaneurysms with rupture of the distal splenic pseudoaneurysm. Coil embolisation was performed on the haemorrhaging distal splenic artery pseudoaneurysm and preventatively on the left gastric artery pseudoaneurysm (). The patient had no further bleeds and was monitored in intensive care unit. Subsequent CT angiogram demonstrated further aneurysmal dilatations along the gastroepiploic artery, middle colic branch of the superior mesenteric artery (SMA) and left common iliac artery (). A dissection flap at the mid SMA was also noted, however, this was present on previous CT imaging and unchanged. All vasculitis, thrombophilic and infective screens were negative. This patient’s clinical presentation combined with the radiological findings were consistent with SAM.\nHis recovery was complicated by multiple segmental and subsegmental pulmonary embolisms requiring insertion of an inferior vena cava filter and therapeutic anticoagulation. However, he had no further haemorrhagic events. He presented 3 months later after falling off a horse and suffered 6 rib fractures on his left side. Despite the significant force from the fall, abdominal imaging demonstrated stable aneurysmal dilatations and the patient demonstrated no further abdominal bleeding episodes. He was followed up 6 months after the event with no further bleeding. |
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