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A 67-year-old Caucasian man presented to the emergency room because of a 4 days’ history of abdominal pain, with one episode of vomiting.\nThe patient’s past medical history was significant for colonic diverticulosis and an episode of gastrointestinal bleeding one year before. The event had been investigated by two different gastroscopies, a colonoscopy and a MDCT, which produced inconclusive results. Ten months later he was newly admitted because of abdominal pain and fever at 38.0 C°, with valid urination and defecation. A CT of the abdomen was performed, which confirmed the colonic diverticulosis and revealed the presence of multiple diverticula of the small intestine, fat stranding, signs of inflammation as well as a small amount of free liquid in the abdomen. The patient was hence diagnosed with jejunal diverticulitis and managed conservatively with intra-venous antibiotics, with an apparent complete recovery.\nHe re-presented to the emergency department two months later with acute abdominal pain. The pain was described as severe and constant, localized mainly in the lower abdomen with clinical signs of peritonitis. No change in bowel habits nor urinary symptoms were complained. His vital signs were stable, with a temperature of 37.2 C°; he appeared fully oriented and not in any acute distress. Laboratory examination reported a hemoglobin of 121 g/dL, a WBC count of 12.2 × 10E9/L and a CRP of 249 mg/L. Other laboratory data were within normal limit. An abdominal and pelvic contrast-enhanced computed tomography, with administration of oral contrast, was performed. Jejunum and ileum showed several diverticula as well as an inflammatory thick-walled mass involving different loops of the intestine. In addition, free fluid in the abdomen and a small amount of subdiaphragmatic air were reported (, ). On the basis of these findings, the diagnosis of perforated diverticulitis was hereby proposed.\nThe patient underwent a diagnostic laparoscopy which revealed plenty of purulent yellowish liquid collected in the right abdomen and a conglomerate of intestinal inflamed loops. We hence decided to convert immediately the procedure to laparotomy. Large multiple diverticula were found covering a section of small intestine approximately 2.5 m long, without signs of obvious macro perforation. Among the middle distal tract of the jejunum and the middle distal tract of ileum, strong adhesions were identified (). The involved segments of jejunum and ileum were connected by an intestinal loop free of signs of diverticulosis (). There were no signs of bowel ischemia. Adhesiolisis was partially carried out, however, because of difficulties associated with the procedure, we opt to perform a double enterectomy, removing only those segments involved in the intestinal conglomerate and deeply affected by the pathology. Roughly 25 cm of ileum and 80 cm of jejunum were resected. Bowel continuity was restored with an ileo-ileal and a jejuno-jejunal anastomosis. Almost 700 cl of pus were drained and the peritoneal cavity was washed with 10 L of saline solution. The postoperative recovery was uneventful and the patient was discharged 8 days later. There were no signs of malignancy in the resected intestine. |
A 9-year-old girl presented with a painless and deformed right elbow [] since 5 years and a painless, deformed right ankle [] since 3 months The deformities of joints preceded with history of repeated trauma. Parents brought her with complaints of swelling in her right ankle and limp since 3 months. Over the past years they complain of her being insensate to pain, lack of tears, dry skin, recurring bouts of fever, constipation, biting tongue and ulcers over her back.\nOn examination she has generalised absence of pain perception, swollen right proximal forearm with abnormal mobility suggesting nonunion. Her right ankle joint appeared to be medially displaced []. She had shortening of her right lower limb by 3 cms and had short limb gait. She had a bifid tongue [], absence of most of the teeth, lack of response to painful stimulus, diminished temperature perception, normal touch perception, diminished deep reflexes, fluctuating blood pressure, multiple scars over the body [] and ligamentous laxity []. Radiographs of her right elbow with forearm revealed atrophic nonunion of proximal ulna with displaced radial head [Figure and ] suggesting old Monteggia fracture dislocation for which she was operated at the age of 6 years at another private hospital. Her right ankle X-rays revealed bimalleolar ankle fracture dislocation [Figure and ].\nHer parents are asymptomatic and have history of consanguineous marriage. There is no history of similar complaints from either of the parent’s side. Their son who is seven years old (younger brother of our patient) also has history of tongue bites, lack of tears and insensate to pain but to a very lesser degree and does not present with Orthopaedic manifestations.\nIntradermal injection of 0.1 ml of 1:1000 solution of histamine produced a wheal but no pain and no axon flare around it. Nerve conduction velocity was normal, serum creatinine phosphokinase level was elevated and findings of the remaining investigations were within normal limits. The patient was operated at the age of nine years for bimalleolar fracture dislocation. Open reduction and internal fixation with malleolar screws and multiple K-wire was done [Figure and ]. Post-operatively, the limb was immobilised in a below knee slab for 6 weeks. Intra-operative and post-operative recovery was uneventful. However, there were no radiological signs of fracture healing after 6-8 weeks, and she was allowed to ambulate with the help of ankle braces. The fracture did not unite even at one year followup. She had right tibial shortening and managed to walk with limp. At 2 year follow-up she presented with pain and swelling in her right ankle and she was diagnosed as septic arthritis of right ankle joint. She had progressed from pain insensitivity to painful ankle joint, for which she underwent surgical debridement and implant removal. Intra-operatively, implants were buried inside the callus, but fracture resulted in pseudoarthrosis. The nonunion of the proximal right ulna was left alone, as she did not present with any complaints. |
A 4-year-old boy was presented to our unit on referral with a three-week history of high-grade fever and a five-day history of generalized abdominal pain and abdominal distension. There was an associated history of headaches and body weakness. He had no history of jaundice. He had several episodes of vomiting which was initially none bilious, but later became bilious. There was an associated history of passage of diarrhea stools two days before presentation, although the patient had not passed stools on the day of presentation. He had no history of passage of melena or of hematochezia. His parents complained that he had been passing scanty urine for about five days before presentation. He had been receiving medications from the referring hospital for about two weeks before presentation.\nAt presentation, he was chronically ill looking with a toxic facie, febrile (temperature 38.5°C), pale, dehydrated, anicteric, and had no pedal edema. His respiratory rate was 28/minute; he had reduced air entry on both lung bases posteriorly. His pulse rate was 128/minute, which was regular but of small volume. Only first and second heart sounds were heard. Abdominal examination showed a distended abdomen which did not move with respiration. He had generalized tenderness with guarding. The bowel sounds were absent. A digital rectal examination showed an empty rectum with a full and tender rectovesical pouch. An initial assessment of a generalized peritonitis was made. The patient was placed on NPO with nasogastric tube for gastric decompression. He was commenced on IV fluid resuscitation and broad spectrum IV antibiotics (ceftriaxone and metronidazole) and was worked up for surgery. Initial laboratory investigations showed a hematocrit of 24% and hypokalemia (2.5 mmol/l) and a serum urea of 10 mmol/l. After an initial fluid resuscitation and correction of serum potassium, he had 300 ml of whole blood transfused. He had an exploratory laparotomy on the second day of admission. At surgery, about 600 ml of a feculent peritoneal fluid was drained, and a single perforation on the antimesenteric border of the terminal ileum was closed in two layers. An intraoperative diagnosis of perforated typhoid enteritis was made. Blood and tissue cultures were not done. He was continued on IV fluids and the same empiric antibiotics after the surgery.\nOn the third day of postoperation, the abdominal wound had a purulent discharge from the distal end. The wound was opened and stitches were removed from the site of drainage, with the institution of daily wound dressing. By the fifth day of postsurgery, a dark patch was noticed to have developed in the skin over the right iliac fossa and measured about 5 cm in its widest diameter. At about the 7th day of postoperation, the dark dry patch had extended to the right hypochondrium, and a similar patch had appeared along the edges of the abdominal incision and the left iliac fossa. A diagnosis of anterior abdominal wall gangrene was made. On the 8th day of postsurgery, the patient was noticed to have developed a complete wound dehiscence () and a fecal fistula. He had fluid resuscitation and was taken back to the theatre on the 10th day after the first surgery, for wound exploration and debridement of the anterior abdominal wall gangrene. A new intestinal perforation was seen at about 2 cm from the initial perforation. The intestinal perforation was exteriorized as an ileostomy, and the abdominal wound closed with tension sutures. Postoperatively, the patient's condition remained unstable, with intractable shock. He died six days after the reexploration of septic shock. |
A 66-year-old woman, a nonsmoker with no comorbidities, presented with complaints of dyspnea and dry cough in October 2014. Her hematological and biochemical parameters were within normal limits. A chest X-ray showed right lower lobe infiltration and right pleural effusion (Fig. ). Computed tomography (CT) of the chest was suggestive of a primary mass lesion in the right lower lobe, 6.5 cm in diameter with hilar lymph node enlargement. A CT scan of the abdomen showed a metastatic lesion of the left adrenal gland. Pleural fluid cytology was positive for lung adenocarcinoma. The stage of the disease was cT3N1M1b, and her EGFR mutation status was reported as deletion in exon 19. The patient was treated with a first line of a TKI (gefitinib at 250 mg) for 30 months. A partial radiological response was achieved according to RECIST (Response Evaluation Criteria in Solid Tumors) v.1.1.\nIn March 2017, radiological progression of the disease was observed, without clinical deterioration. CT scans of the chest and abdomen showed an increased primary tumor in the right lung and metastatic lesions in the left adrenal gland. The patient underwent bronchoscopy for the first time. The endoscopic finding was normal, and histological examination of the transbronchial biopsy (TBB) from the right upper lobe confirmed lung adenocarcinoma. However, EGFR testing of the TBB material was negative. Since there was an insufficient amount of tissue available from the TBB, the multidisciplinary tumor board decided to repeat bronchoscopy for a wider testing panel including PD-L1 testing, which had become available meanwhile. Once again, an EGFR test was performed on the histology sample, and immunohistochemistry for ALK and PD-L1 was done for the first time. All three tests came out negative. Since the patient had an Eastern Cooperative Oncology Group (ECOG) performance status of 0, without any symptoms, the patient requested to have a liquid biopsy test performed. Next-generation sequencing tests were not available at that time, but liquid biopsy EGFR testing was available in house.\nA liquid biopsy was performed, and the result indicated the presence of T790M mutation. The patient started treatment with osimertinib in the second line within a compassionate use program. CT scans of the chest and abdomen showed a partial therapeutic response after 3 months of treatment (Fig. ), while brain CT results remained normal throughout treatment. The patient is still on osimertinib therapy (total duration: 59 months) without symptoms from the primary tumor and without side effects. |
A 50-year-old male patient with aortic dissection originating just above the aortic valve and extending down to the common iliac arteries (Stanford A, Figure ) underwent immediate surgery with repair of the ascending aorta in moderate hypothermia under cardiopulmonary bypass. The arterial line was inserted in the right axillary artery, the vent was placed in the right upper pulmonary vein, and two-stage venous line was inserted through the right auricula. The intact aortic valve was resuspended. Extracorporeal circulation was suspended after induction of moderate hypothermia (25°C) and the aortic cross-clamp from the ascending aorta was removed. The false lumen was then glued and an open distal anastomosis to a prosthetic graft was constructed. Then, extracorporeal circulation was resumed, systemic circulation was deaired and the patient was warmed.\nEarly on the first postoperative day, CT of the aorta was requested because of marked elevation of lactate-dehydrogenase (129 μkat/l) and signs of acute renal insufficiency (creatinine 292 μmol/l). CT showed good postoperative result in the ascending aorta, but large amount of air in the branches of the superior mesenteric artery up to the arcades was found (Figure ). There was absolutely no air in the portal-venous system including the liver. The bowel loops were not distended, and there were no signs of bowel paralysis.\nAt midnight on the first postoperative day, a biphasic CT scan of the abdomen was requested due to elevated intra-abdominal pressure (18 mmHg). The CT showed distribution of the intra-arterial gas more into the periphery and into the wall of the bowel loops that still did not display signs of ileus (Figure ). The next morning, surgical exploration was performed due to increasing intra-abdominal pressure as a sign of imminent abdominal compartment syndrome. An extensive bowel resection from oral ileum down to the splenic flexure had to be performed due to extensive necrosis of the bowel. Shortly after the operation, the patient became hypotensive with signs of overwhelming vasoparalysis and died on the third postoperative day of multiple organ dysfunction syndrome (MODS) with systemic inflammatory response syndrome (SIRS). |
The case involves a male who was 67 years old at the time of his death. He was a retired businessman, married with several children, and socioeconomically advantaged. He lived with his wife and children, and his mother. He spoke both English and Hindi fluently, and had no sensory or cognitive impairment. His only co-morbidities were longstanding well controlled hypertension and impaired glucose tolerance.\nHe was diagnosed with chronic kidney disease secondary to focal segmental glomerulosclerosis in 2003. He commenced peritoneal dialysis for end stage kidney failure in 2006, which continued without any infectious or technical complication for the entire period of his treatment with this modality. He developed peritoneal membrane failure after 4 years; we made a decision to transition him to home hemodialysis in early 2010.\nHe had an arteriovenous fistula placed on 21st July 2010, and commenced home hemodialysis training 14th September 2010. He completed training on 7th February 2011, and commenced home hemodialysis thereafter. There were no reported problems or technical difficulties throughout his training. His hemodialysis prescription was for 5 hours of dialysis three times a week, using a hollow fibre polyamide 2.1m2 high flux dialyser, bicarbonate-based dialysate with a [K+] of 1.25 mM, and a Gambro AK200S machine (Gambro AB, Lund, Sweden).\nOn the day of this death, 3rd March 2011, the patient had contacted his case manager in the morning to propose a decrease in his target weight and the removal of more fluid than prescribed. He was advised against this. Several hours later, his family called the unit to report that they had returned home to discover him dead while still attached to his dialysis machine.\nInvestigations showed that the patient had exsanguinated during the wash back procedure. During the wash back, a 1 L bag of saline had been misconnected to the venous end of the extracorporeal blood circuit, rather than the arterial end. As a result, the patient’s blood was pumped out of his circulation into the bag of saline, instead of saline being pumped out of the bag through the extracorporeal blood circuit (Figure ). The bag of saline is shown in Figure , and weighed 3.3 kg implying at least 2.3 L of the patient’s blood in addition to 1 L of saline. Of note, his last blood pressure reading was recorded one and half hours before the end of dialysis (patients are usually advised to take their observations hourly), and at that time his blood pressure and heart rate were satisfactory (146/84 and 59 bpm respectively).\nExhaustive testing of the patient’s hemodialysis machine showed that it was functioning correctly, and in particular venous alarms were triggering normally. To replicate the patient’s situation, we tested the distensibility of a bag of normal saline and determined that it can expand to several times its volume to accommodate at least three litres of extra fluid, all without substantial increase in venous pressure on the dialysis machine or bag rupture (Figure ).\nAt a blood pump speed of 200 mL/min, it would have taken more than 10 minutes to fill the saline bag with as much blood as we found. Consequently, we were concerned that the patient may have been acutely incapacitated by a primary cardiovascular or cerebrovascular event at the time of the wash-back. A post mortem examination was performed, with the forensic pathologist reporting no specific anatomic, toxicological or biochemical abnormality to account for his death. Minor arthrosclerosis was noted in the left anterior descending and right coronary arteries, left vertebral artery, and the infrarenal aorta. The myocardium and brain were normal on multiple sections. The Coroner’s conclusion was that death was due to hypovolaemic shock arising from blood loss into an incorrectly connected saline bag.\nThree providers of home hemodialysis around New Zealand have reported similar incidents. Two occurred in setting of home hemodialysis training rather than dialysis at home. In both of these cases, the trainer noticed the error immediately and was able to rectify the problem and use it as a teaching point. The third case occurred under ostensibly similar circumstances to ours, also appearing to lead to the patient’s death. That case is currently sub judice with the Coroner’s Office.\nThe death of our patient prompted an external formal review of our home hemodialysis training program, resulting in the following changes. We now make patients aware of this specific potential complication, and emphasize to them that they must ensure clearing of the extracorporeal blood circuit after a minute or two of the wash back procedure. We also emphasize the potential dangers of performing home hemodialysis alone, especially in those who are newly trained, and re-iterate our recommendation that there is someone else in the home when they dialyse if that is possible. Finally, we have developed a one-way valve that we are incorporating into the saline line for all of our home hemodialysis patients. This valve prevents reflux of blood back into the saline bag from the extracorporeal blood circuit in the event of misconnection, and triggers pressure alarms that alert patients to their error.\nAnother potential solution is the “closed circuit” wash back procedure. In this procedure, there is a Y-connection close to the arterial end of the extracorporeal blood circuit where it attaches to the arterial needle. At the discontinuation of dialysis, the patient attaches the saline line (or on-line ultrapure/sterile dialysate line) to the Y-connect, then clamps the line between the arterial needle and the Y-connect, before using the blood pump to draw saline through the extracorporeal blood circuit for wash back. The procedure is illustrated in Figure . The primary shortcoming of this procedure pertains to the residual blood between the arterial needle and the Y-connect, usually 2.5-5mL depending on manufacturer. This blood can either be discarded, or returned to the patient by stopping the blood pump and allowing gravity to motivate saline from the hanging bag towards the arterial needle in a retrograde fashion through the circuit. This effectiveness of the last part of this procedure is limited by the opposing pressure in arteriovenous access, and in our experience is not successful in many patients without pressure applied to the saline bag which is generally not a standard or recommended practice.\nThe manufacturer of our patient’s dialysis machine is aware of this incident. There have been discussions around the feasibility of changing the extracorporeal blood circuit to make such an error impossible in the future. For instance, the arterial and venous ends of the circuit could be produced with different coloured or incompatible connections to make it difficult for patients to misconnect them. Currently, this fail-safe facility is not available from any manufacturer of dialysis machines. |
A forty-three-year-old female patient was taken to the NCI with a clinical picture of an ulcerated lesion on the lingual left lateral edge with seven months of evolution that reaches the floor of the mouth. The chest CT did not have pathological findings. The neck CT confirmed the existence of a nodule on the floor of the mouth, whose etiology should be determined by its histopathologic study; also some submental and left jugular lymphs were observed. And incisional biopsy whose histopathological study showed an infiltrating, nonkeratinized moderately differentiated OSCC was carried out. The patient had personal smoking records (7 cigarettes per day) and did not have a history of long-term alcohol abuse.\nOnce at the NCI, there was a clinical exploration in which an endophytic tumor on the left lateral lingual edge spread to tongue, retromolar base, and trigone. The lesion was classified as T4a N1 M0.\nA total glossectomy was carried out through mandibular swing and bilateral modified radial lymphatic cervical dissection. A second biopsy was taken from the tumor and microscopical exam revealed a carcinoma with two neoplastic cellular components, on one hand a proliferation of atypical spindle cells and on the other hand nests of squamous epithelial cells with hyperchromatic nuclei, prominent nucleoli, and atypical mitosis. The studies with IHC revealed that vimentin was strongly positive in the component of spindle cell as in epithelial cells. Cytokeratin AE1/AE3 was strongly positive in epithelial cells and focally positive in spindle cells. EMA antigen was positive for epithelial neoplastic cells and a weak expression in the spindle component was observed. Both neoplasia components were negative for desmin, S-100, and smooth muscle actin alpha (). Based on the previous results, a SC was diagnosed. A treatment with postoperative radiotherapy and chemotherapy was indicated. After two months of the surgical procedure, the patient is in good general condition without evidence of metastasis. |
A 75-year-old female patient visited the Emergency Department due to hypotension. She was on warfarin after aortic valve replacement surgery with a mechanical prosthesis for 26 years and in the state of right hemiplegia due to subacute subdural hematoma for 2 years. One year ago, multiple bullous lesions occurred on whole body, and the diagnosis of bullous pemphigoid was made after skin biopsy. The bullous lesions had never been hemorrhagic, and they had been well controlled with prednisolone 20 mg once a day and methotrexate 10 mg a week. Upon examination, she had a large hematoma on right buttock area and laboratory test revealed low hemoglobin, 7.1 g/dL and prolonged prothrombin time, >120 seconds, <10%, >17.8 international normalized ratio (INR). One week before visiting our hospital, generalized tonic-clonic seizures occurred and valproic acid was started by a neurologist. Because there was possibility of drug interaction between warfarin and valproic acid as a cause of increased prothrombin time, valproic acid discontinued. In addition, fresh-frozen plasma and vitamin K were administered and prothrombin level was normalized.\nOn the 10th day of hospitalization, enoxaparin sodium (40 mg every 12 hours by subcutaneous injection) was started for anticoagulation. Three days after starting enoxaparin therapy the patient developed several tense hemorrhagic bullae on distant sites: the right forearm, the dorsum of the right hand and the left foot. The most severe lesion was on the right forearm (), 2 days later, bleeding occurred due to a rupture of hemorrhagic bulla (). The patient was referred to an allergist. Bullous hemorrhagic dermatosis related to enoxaparin use was suspected. Enoxaparin was stopped from that day and readministrated with caution 4 days later after improvement of the hemorrhagic bullae because of the high risk of thromboembolic event due to the artificial valve and previously too prolonged prothrombin level. However, as hemorrhagic bulla worsened again, enoxaparin was discontinued. Methylprednisolone 40 mg (1 mg/kg) every 12 hours by intravenous injection was administered after skin biopsy.\nLaboratory test revealed a low level of hemoglobin (9.6 g/dL), normal platelet count (307,000/µL) and normal prothrombin time (61%, 16.8 seconds, 1.38 INR), and an elevated activated partial thromboplastin time (59.2 seconds). Results of serum biochemistry test were unremarkable. A punch biopsy was performed from the lesion over the right forearm and pathologic findings revealed increased vascular channels with hematoma formation and superficial perivascular lymphocytic and a few eosinophilic infiltration (). There is no evidence of vasculitis or capillary thrombosis. Direct immunofluorescence showed that C3 was strongly positive at the dermoepidermal junction and this result may be due to the underlying bullous pemphigoid ().\nAfter the administration of high-dose steroid, the bullous hemorrhagic lesions were improved dramatically (). Warfarin was administered for anticoagulation without any further problem. The hemorrhagic bulla regressed without sequelae. Methylprednisolone was administered 25 mg once a day because of the underlying bullous pemphigoid. The patient was discharged from the ninth week of hospitalization without any recurrence of hemorrhagic bullous lesions or bullous pemphigoid. |
Here was a 55-year-old female patient with episodic stabbing pain on the right superior eyelid for 2 years. The VAS score was eight. The onset time was about 1 minute and the remission period was completely pain free. The pain could be triggered by daily activities like smiling, washing face, speaking and eating. Any contact with the upper eyelid could lead to pain outbreak. Apart from the allodynia on the skin of upper eyelid, no abnormality was found in neurological examinations. Also, the ophthalmologic exams including funduscopy, intraocular pressure, visual field testing was normal. The head CT and MRI scan were also normal. The patient had no history of surgical operation and trauma, no history of diabetes mellitus, high blood pressure, chronic heart diseases, hepatitis, and some other relevant chronic diseases. The patient was diagnosed as primary TN.\nThe treatment was initiated by oral carbamazepine. The pain could be relieved by carbamazepine 200 mg daily at first. Gradually increasing dose of carbamazepine was reported during the medication period. About 2 weeks prior to the treatment, the pain could not be controlled by carbamazepine 800 mg daily, and dizziness and vertigo developed simultaneously.\nPeripheral branch neurolysis was planned. Firstly, right supraorbital nerve block was implemented as a diagnostic test. After identifying the supraorbital notch through palpation, a 25-gauge needle was inserted at the level of supraorbital notch. 0.5 ml 1 % lidocaine was injected slowly. Ten minutes later, hypoaesthesia in the skin of the forehead instead of the upper eyelid was observed, and the pain continues. This result indicated that supraorbital nerve was not involved. Then, diagnostic blocks to lacrimal, infratrochlear and supratrochlear nerves were successively performed. The puncture point slightly above the lateral canthus along outer border of the orbit was selected for the lacrimal nerve block. As infratrochlear and supratrochlear exiting points were located closely together, we chose the point where the bridge of the nose abuts the supraorbital ridge as the single puncture point (Fig. ). The patient was put in supine position while eyes firmly closed. After disinfection, a 25-gauge needle was punctured at the lacrimal nerve point described above. The needle was slid over the edge of the orbit, and advanced about 4 mm along the lateral wall of the orbit. With no aspiration of blood, 0.3 ml 1 % lidocaine was injected slowly. Five minutes later, hypoesthesia was found on the lateral part of the upper eyelid, while the medial part remained allodynic. Then the infratrochlear and supratrochlear nerve blocks were performed. The needle was inserted in the previously mentioned point, just slid over the edge, with the same depth along the medial wall. The same amount of lidocaine was injected slowly. The whole upper eyelid and adjacent areas were found hypoesthetic. The patient was completely pain free for the whole day. Then lacrimal, infratrochlear and supratrochlear nerves radiofrequency thermocoagulation (RT) was planned the next day. With supine position, the skin of upper eyelid and adjacent areas was disinfected. From the two puncture points above-mentioned, a 22 G radiofrequency needle with a working tip of 4 mm was slowly inserted successively. Slide over the edge of the orbit and advance about 4 mm. Repositioning the needle slightly to the point where sensory test stimulation at 0.3 mA and 50 Hz could evoke obvious paresthesia in the periocular region. As for infratrochlear and supratrochlear nerves RT, with the aim to ablate both the two nerves, redirect the needle tip superiorly and inferiorly along the medial wall of the orbit to make additional ablation. After local anaesthesia, RT with tip temperature of 80 °C and duration of 80 s was performed.\nThe patient was discharged after 2 h observation. There were no complications, except for hypoesthesia in the upper eyelid and eyelid swelling for 2 days. The pain was completely relieved within the follow-up period of 6 months without any medication. |
A 25-year-old lady presented to the emergency department with a two-day history of left iliac fossa pain and vomiting. She was normally fit and well with a history of having had an appendectomy. She did not have any symptoms to suggest urinary tract infection, and she was on her menstrual period during presentation. She did not have any known personal or family history of gynecological problems. She was not on any regular medication.\nGeneral examination was unremarkable. Abdominal examination revealed a soft abdomen with tenderness in the suprapubic region and left iliac fossa.\nRoutine blood results including inflammatory markers were normal (Table ).\nAfter the initial review, the patient was sent home with analgesia and to have an outpatient ultrasound scan of the pelvis with transvaginal views. The ultrasound scan, which was performed a month later, revealed a mixed echogenic mass measuring 12.6 cm x 8.5 cm x 6.4 cm within the left adnexa. Within the right adnexa, a similar appearing mass was also demonstrated measuring 6.9 cm x 6.1 cm x 4.1 cm. No free fluid was seen in the pelvis. The appearance was consistent with bilateral ovarian dermoid cysts (Figure ).\nAt a three-month clinical review, a magnetic resonance imaging (MRI) scan was booked. However, she was admitted to the emergency department again with left iliac fossa pain and vomiting. Abdominal examination was unremarkable, and routine blood tests were normal. The plan was then made to send her home and wait for the MRI scan on an outpatient basis, and if the pain worsened in the interim, to have emergency surgery to remove the cysts.\nThe MRI scan revealed large bilateral adnexal masses, measuring up to 16.6 cm x 13.7 cm x 9.1 cm on the right side and 13.2 cm x 10.7 cm x 8.2 cm on the left side. The right-sided lesion occupied most of the right hemi-pelvis and descended between the uterus and rectum caudally. The left-sided lesion was located between the uterus and anterior abdominal wall extending cranially and occupying partially the left upper quadrant of the abdomen. Both lesions were complex with several components, some cystic and some containing large amount of fat in keeping with dermoid cysts or teratomas (Figure ).\nAfter discussing the risk of surgery and possible effects on future fertility, the patient underwent laparoscopic surgery two months later. This revealed two large bilateral ovarian cysts filling the pelvis. The right ovary had multiple dermoid cysts, which were removed piecemeal sparing a large remnant of the right ovary together with a normal right fallopian tube. The left ovary appeared inflamed and gangrenous, and it was surrounded by omental adhesions, most likely due to chronic torsion. Removal of the omental adhesions exposed the fact that the left ovary had undergone torsion twice, and it was felt that this ovary should be removed. There were no intra-operative complications. The majority of the operation was undertaken laparoscopically although the large cyst was taken out through a small Pfannenstiel incision with plenty of pelvic wash out.\nHistology confirmed bilateral large benign dermoid cysts. There was also evidence of focal infarction of the left ovary in keeping with ovarian torsion.\nShe was followed up in the gynecology clinic two months later. She was doing very well and had two normal menstrual periods since the operation. She had no more pain or discomfort. However, her level of anti-Mullerian hormone level becomes low at 1.8 pmol/L indicating very low ovarian reserve (very low fertility potential) in the remaining right ovary. A repeat transvaginal ultrasound scan revealed a right ovary with small cysts (Figure 3). |
A 33-yr-old man presented with decreased vision of the right eye referred to Amiralmomenin Hospital, Rasht, Iran in November 2018. His symptoms were not accompanied by any other ocular symptoms. The patient had no relevant family history or prior ophthalmological problems or head trauma/injury. He had no significant medical history, including diabetes mellitus and hypertension, and no history of smoking. He had been treated with clomiphene citrate 100 mg for 2 wk for infertility issues. The patient presented with a sudden decrease of visual acuity in the right eye on the 14 day of taking the treatment and subsequently developed complete loss of inferior visual field in a few days. He had no history of other medicine or herbal intake.\nAt initial presentation, his best-corrected visual acuity was 6/20 in the right and 20/20 in the left eyes, with a right relative afferent pupillary defect and decreased red color saturation. The intraocular pressure was 13 and 11 mmHg in the right and left eyes, respectively. Extraocular movements were normal in both eyes. The slit-lamp examination of the anterior segment and anterior vitreous were unremarkable in both eyes. The fundus copy of the right eye revealed a swelling of the optic nerve head with blurred margin, especially in the superior pole pallor, and venous congestion was noted. The macula seemed to be normal. Fundus copy of the left eye showed a small cup–disc ratio of 0.2 without any other pathological findings (Figure 1). Based on these findings, immediate neurologic consultation was performed and brain and orbital MRI and computer tomography were obtained, which showed no pathologic findings. Cardiologic examinations including carotid ultrasound and blood pressure monitoring were also unremarkable. Further serologic studies for infection and hypercoagulable states (complete blood count, thyroid hormone levels, erythrocyte sedimentation rate, C-reactive protein, angiotensin-converting enzyme, toxoplasmosis antibodies, antithrombin III, factor V Leiden, protein C, protein S, anti-phospholipid antibody, and lupus anticoagulant) were evaluated. All laboratory tests were within the normal range.\nPerimetry by a Humphrey Visual Field Analyzer (24-2 threshold program) revealed an absolute inferior altitudinal visual field deficit in the right eye and a normal visual field in the left eye (Figure 2). Optical coherence tomography of the optic nerve showed thickening of the nerve fiber layer of the right eye and the macular optical coherence tomography revealed no significant changes.\nPhysical examination and complementary evaluations were suggestive of non-arteritic anterior ischemic optic neuropathy (NAION) of the right eye. Considering the normal laboratory and cardiologic findings and that the patient had been taking clomiphene citrate, it was concluded that clomiphene citrate was accountable for the anterior ischemic optic neuropathy. The patient was asked to discontinue clomiphene citrate. Three months later despite the drug discontinuation, the visual acuity was 10/20 in the right eye with a relative afferent pupillary defect and superior disc pallor, with a persistent inferior altitudinal visual field defect.\nThe case report was fully explained to the patient and written consent was obtained to authorize the authors to write and publish a case report. |
A 62-year-old female was found to have bilateral pulmonary emboli on computed tomography pulmonary angiography (CTPA) following two episodes of syncope (). She underwent transthoracic echocardiography (TTE) and a large, mobile thrombus was reported in her right atrium (RA) and IVC (). She underwent emergency removal of the intracardiac mass and a large, white tumor mass protruded through the atriotomy when the right atrium was opened for venous cannulation for cardiopulmonary bypass (). The right atriotomy was extended and the mass was well visualized and divided at its apparent attachment to the IVC (). Histopathology identified a low-cellularity fibrous tumor with smooth muscle cells, with no areas of necrosis or atypia. Immunostaining was positive for smooth muscle actin and desmin, confirming the diagnosis of IVL (). Further abdominal imaging with CT identified a distended IVC, right common iliac vein, and right internal iliac vein, filled with low-density intraluminal material and a soft tissue density mass in the pelvis (). She underwent a combined procedure with gynecologic oncology and vascular, general, and cardiothoracic surgery, all present. Redo-sternotomy and laparotomy were performed, the IVC was opened after gaining control of the proximal and distal vessels, and a white, firm, rubbery tumor nearly 30 cm long was excised with gentle traction (). The internal iliac vein had tumor remnant in it that was not resectable and the vein was ligated. The pelvic mass was identified abutting but not invading the sigmoid colon and was excised (). Histopathology identified identical material to the original specimen with strong estrogen and progesterone receptor positivity on immunostaining (). Her postoperative recovery was complicated by deep venous thrombosis of her right lower limb, treated with warfarin. Her recovery was otherwise unremarkable and she was commenced on Anastrozole 1 mg daily as per oncologic recommendation. She continues to remain well six months following her hospital discharge with ongoing oncology follow-up. |
We present the case of a three-year-old Caucasian girl with ASD, focusing on her PEH (Table ). She was born after 41-weeks gestation via caesarean section and had an Apgar score of 9/10. A birth weight of 3.85 kg, head circumference of 37 cm and length at birth of 50 cm were recorded. No dyschromias or malformations were found. Our patient's growth and karyotype were otherwise normal. She was breastfed until the age of nine weeks. By five months, she was able to hold her head steady and erect, and was able to walk on her own at 15 months of age. Our patient currently walks on her tiptoes and began this when she first started walking. On examination, our patient had normal growth and anthropometric data. Her muscle tone was moderately decreased, widespread and symmetric, with normal osteotendinous nerve reflexes. Acquisition and delayed maturation of language was observed. Sensory perception and attention were thought to be normal until 12 months of age. Subsequently, her parents noticed that our patient did not respond to their calls and seemed isolated with introverted behavior. Our patient was not communicative and was consistently unresponsive to the calling of her name. In addition, she did not interact with other children and presented with restricted interest and repetitive stereotypical behavior. Our patient did not show imitative or projective play. In terms of education, starting at two years of age she was schooled with additional support from an early learning program. Brain magnetic resonance imaging (MRI) scans appeared normal. Our patient was diagnosed with pervasive developmental disorder of the autistic spectrum and moderate mental retardation.\nThe 32-year-old mother and 36-year-old father lived on the second floor of a 14-year-old building in Puente Tocinos, Murcia. The mother had given birth to a healthy boy six years earlier. In the current case, the pregnancy was not planned. During the peri-conceptional stage, the mother ingested approximately 20 to 40 g of ethanol per day, while the father ingested around 40 to 60 g of ethanol per day during the first nine days of embryonic development. The alcohol intake subsequently decreased to 10 g/day for both parents until the 16th to 18th days of embryonic development when the parents became aware of the pregnancy and ethanol consumption was completely eliminated. Neither parent smoked during the pregnancy or during our patient's first year of life, and our patient was not exposed to any other drugs. Her serology was normal, and blood test results were positive for rubella IgG antibodies. Both parents had post-high-school education.\nThe mother was employed in the food manufacturing industry and occupational exposures during pregnancy included: phosphoric acid, alkylbenzenesulfonic acid, hydroxide and sodium hypochlorite, nitric acid, sodium hydroxide and alkyl alcohol ethoxylate. The father was employed as an auto mechanic and reported a null or slight possibility that he had indirectly brought home traces of chemicals or solvents that had soiled his clothing or shoes.\nFrom a year prior to conception the mother began a weight loss diet and ingested approximately 1200 mg/day of 'horsetail' (Equisetum arvense) herbal remedies up to three years after birth. At conception maternal body mass index (BMI) was 31.6 kg/m2, which decreased to 30.1 kg/m2 at the end of gestation, a net loss of almost 4 kg. Throughout pregnancy, despite adequate caloric intake, the mother reported a daily food intake of folate (199 μg), vitamin B1 (1.18 mg), vitamin B6 (1.31 mg) and vitamin B12 (30.8 μg). Vitamin B12 and folate intake supplements were started on approximately days 42 to 48 of gestation. |
A 40-year-old male with primary sclerosing cholangitis and a previous history of colectomy with j-pouch for ulcerative colitis was listed for cadaveric liver transplant with a MELD of 33. During his preoperative work-up he was found to have complete PVT. He did not appear to have any enlarged venous collateral that could be used to reconstruct the portal system during transplant. Therefore, our team was prepared to perform a CPH if needed [, ].\nThe patient was taken to surgery after a 16-year-old cadaveric liver was procured for him. The hepatectomy was difficult secondary to adhesions from both his inflamed liver, percutaneous biliary tubes, as well as his prior surgeries. The dissection was carried out with standard piggyback technique dissecting the liver off the vena cava to the level of the hepatic veins []. Thus, the vena cava was easily accessible distally to the level of the renal veins. No portal vein was found, and attempts to find suitable venous collaterals were unsuccessful.\nAt this point there was no other option to reconstruct the portal system except to perform a CPH. Because we had prepared the vena cava of the recipient during the hepatectomy, it was easily ligated below the hepatic vein—supracaval anastomosis allowing for maximal caval length. The donor portal vein was then anastomosed to the suprarenal vena cava in an end-to-end fashion (). Reperfusion proceeded without complication. Arterial flow was then re-established, and the biliary system was reconstructed with a choledochoduodenostomy [].\nThe patient had an uncomplicated postoperative course. His liver function normalized within the first week, and he has never had rejection with a 12 month followup. He did not experience any ascites, peripheral extremity edema, or renal insufficiency. Furthermore, he had return of bowel function similar to his preoperative j-pouch function. Both a postoperative ultrasonography and CT-venogram documented excellent flow through the portal vein (Figures and ). |
A 52-year-old man who was employed at a titanium-machining factory and who had a history of cancer of the right lung (spindle cell carcinoma, clinical stage T3N0M0) underwent right upper lobectomy and extended combined resection of the chest wall (resection of the 1st to the 5th ribs, right thoracotomy) to remove lung cancer. Chest wall reconstruction was not performed during this procedure on account of the judgment of the thoracic surgeon. He was discharged from the hospital without complications. One week after discharge, sudden pain appeared in his right shoulder during actions such as rolling over in bed, getting up, washing his face, or driving a car, and he was referred to our department with suspected dislocation of the shoulder. Physical examination revealed no resting pain and no restricted range of motion of the shoulder, with only winging present at rest. After the patient performed a push-up movement, however, the pain appeared, and the shoulder girdle was locked at a horizontal abduction angle of 45° (). Computed tomography (CT) revealed that when the symptoms appeared, the inferior angle of the scapula was caught inside the top of the sixth rib. This locking was not evident on CT when the pain was absent ().\nOn the basis of these findings, recurrent intrathoracic locking of the scapula was diagnosed. Conservative management was implemented in combination with physiotherapy, using of a clavicle band to restrict the movement of the scapula. However, because there was no improvement in either the locking symptoms or pain, it was decided to adopt a surgical approach.\nSurgery was carried out via a high posterolateral incision (using the skin incision made during chest surgery) with the patient in the lateral decubitus position (). The teres major and rhomboid major muscles on the dorsal surface were detached from the periosteum to expose the bone surface of the scapula. The serratus anterior muscle, which had been conserved during the previous surgery, was also detached to enable the inferior angle of the scapula to be raised sufficiently, after which the inferior angle of the scapula was locked inside the thorax and marked with an electric scalpel. A bone saw was then used to remove the inferior angle of the scapula, leaving a margin of +1 cm (). A hole was made in the osteotomy section by using a 2.0 mm K-wire, and the teres major and rhomboid major muscles were secured to the chest wall.\nPostoperatively, the locking symptoms improved, but pain still occurred when the resection margin collided with the ribs (). There was no change in the range of motion from before. Although another operation was considered, the decision was made to implement palliative care because localized recurrence of the lung cancer was present and metastases had enlarged. The patient died ten months after surgery. |
A 17-year-old male with Marfan syndrome was admitted for the repair of a LVOT PsA which was detected on a routine postoperative follow-up imaging study. He underwent total 9 cardiac surgeries including Bentall operation, total arch and descending thoracic aorta replacement with tricuspid and mitral annuloplasty. Among these surgeries, two were performed for treating graft infection with abscess formation and the other surgeries were performed for repair of aortic dissection. The serial chest computed tomography (CT) scans showed that the diameter of PsA was increased from 30 mm to 33 mm in a year and that it was located just below the left main coronary arterial opening (). Transthoracic echocardiography (TTE) showed communicating flow across the PsA. Through the preoperative meeting among pediatric cardiologists, cardiac surgeons, and anesthesiologists, percutaneous intervention was chosen for the treatment of this patient because surgical repair was expected to carry a very high risk.\nOn arrival at the hybrid operating room, anesthesia was induced using thiopental rocuronium bromide. Anesthesia was maintained with desflurane in a mixture of oxygen and medical air. After anesthesia induction and orotracheal intubation was performed, a TEE (iE33 xMATRIX Echocardiography system, Philips, NV, USA) probe was placed and continuous TEE monitoring was performed during the whole procedure by well-experienced cardiac anesthesiologists. Pre-procedural intraoperative TEE examination showed good left ventricular contractility with mild aortic and mitral regurgitation and the location of PsA, adjacent to the aorto-mitral junction and it was communicating with the LVOT (). There was no significant pathology related to the previously replaced prosthetic aortic valve.\nBefore the procedure, 5,000 units of unfractionated heparin were injected intravenously and the activated clotting time (ACT) measured following the injection was 216 seconds. Using the approach via the right common femoral artery, an 8-Fr guiding sheath was advanced into the LVOT and placed across the neck of the PsA under biplane fluoroscopy and TEE guidance (). Fluoroscopy showed a pulsating pouch derived from the aorta. Simultaneously, TEE demonstrated a wide jet of swirling arterial blood flow within the PsA. The diameter and the length of aneurysm neck measured by intra-procedural TEE were 13.6 mm and 8.6 mm, respectively (). During the first several attempts to advance the guidewire into the lumen of the PsA, the guidewire passed through the mitral leaflet into the left ventricle, which caused mitral regurgitation (). After each attempt, we discussed and modified the direction of the guidewire. Finally, the guidewire was successfully advanced into the lumen of the PsA after several failed attempts ().\nA 16-mm-diameter type II Amplatzer vascular plug (AVP, AGA Medical Corporation, MN, USA) was chosen and positioned across the neck of the PsA. After confirming the acceptable position and the intact aortic and mitral valvular function using angiography and TEE, the AVP was deployed. Successful deployment without leakage flow around the AVP was confirmed through the following TEE examination (). Angiography also showed diminished flow communication in the PsA (). After the uneventful procedure, the patient was transferred to the post-anesthesia care unit. The post-procedural TTE also showed no residual flow in the communicating pouch on the aortic root PsA and adequate position of the device (). The post-procedural course was unremarkable and the patient was discharged on post-procedural day 3 without any complications. |
A 35-year-old African American male with a 20-year history of type 1 AIH, liver cirrhosis, primary sclerosing cholangitis, and esophageal varices presented with a two-day history of confusion and bizarre behavior. He had been recently diagnosed with HCC and was taking lactulose and lenvatinib 12 mg daily two weeks prior to presentation. He was not able to titrate his bowel movements with lactulose at home due to chemotherapy-induced diarrhea, which resulted in missing two doses of lactulose. The patient was subsequently diagnosed with hepatic encephalopathy secondary to lactulose non-adherence in the setting of chemotherapy-induced diarrhea. During his hospital course, the patient was treated with lactulose and rifaximin, which resolved his encephalopathic symptoms. He is currently being evaluated for a liver transplant and is being monitored closely by his oncology team at a tertiary care center.\nCase background\nThe patient was diagnosed with type 1 AIH at the age of 15 following a liver biopsy, which showed cirrhosis and inflammatory activity consistent with AIH. To manage recurrent AIH episodes, he was prescribed azathioprine, prednisone, and propranolol by a gastroenterologist at a tertiary care hospital. Unfortunately, due to complex social determinants of health including insurance coverage issues and unwanted medication side effects, he was mostly non-adherent to the management plan. Due to the patient’s multiple gastrointestinal comorbidities, a series of surveillance tests were prescribed in 2013, including an outpatient MRI, AFP monitoring, and surveillance abdominal ultrasounds every six months to screen for cholangiocarcinoma and primary liver cancer. However, insurance coverage challenges prevented the patient from maintaining consistent follow-up.\nApproximately one month prior to his hospital presentation with hepatic encephalopathy, he was diagnosed with HCC. This was determined after a workup for presentation with bilateral lower extremity and testicular swelling yielded the presence of a hepatic mass (4.91 by 3.93 mm) seen on MRI with a right portal vein thrombus (Figure ).\nLaboratory studies were notable for elevations in AFP (8418.2 ng/mL) and bilirubin (6.7 mg/dL). Subsequent percutaneous needle biopsy of the liver confirmed the diagnosis of well-differentiated HCC, with biopsy positive for arginase-1 and negative for cytokeratin-7. Subsequent CT of the chest, abdomen, and pelvis with contrast for staging was performed one month after the initial diagnosis. While it demonstrated no evidence of metastatic disease, it did reveal progression of the hepatic mass to 5.15 by 3.83 mm (previously 4.91 by 3.93 mm) along with central invasion of the tumor (Figure ).\nWith these findings, our patient was diagnosed with stage IIIB HCC. |
A 40-year-old man sought evaluation of a 4-year-history of perianal and perineal pain, including the testes and penis. In this patient, there was no notable history of trauma or disease. On MRI and CT scans, which included the abdomen and pelvis, there were normal findings. At the time of admission, the patient had a visual analog scale (VAS) of 8/10. The patient had the persistent presence of pain, which was characterized as bursting and explosive. Due to the presence of pain, the patient could not assume a sitting position for more than 5 minutes. The patient also stated that he could not perform work or household chores. Four years before the initial onset of pain, the patient had been working as aresearch staff in the US. At the time, the patient sought evaluation in a urology department under the assumption that the condition originated from the prostate gland. Following evaluation, the patient was considered normal without a discernible etiology for the pain. The patient was therefore transferred to a pain clinic, where the patient was given oral medications (gabapetin 1,800 mg/day and methadone) in an outpatient setting. The patient also received superior hypogastric, caudal, and T12-L1 epidural blocks. However, these procedures had no effect in reducing the pain. In early February 2007, the patient sought evaluation in an outpatient clinic in the US due to aggravation of the pain in a lying position, and underwent blockage of the ganglion impar; the VAS decreased from 8/10 to 4/10. Because the effect was sustained for approximately 1 day, the patient could not ambulate for 2 months. During this period, the patient remained in a lying position. Thereafter, the patient underwent blockage of the ganglion impar on two occasions. Following this, the pain decreased to a VAS of 4/10. When the pain was severe, however, it was a VAS of 8/10. Every 3 months, the patient underwent blockage of the ganglion impar 4 times with the use of steroids 40 mg each, 4 times. In September 2008, the patient returned to Korea. At the time of his initial outpatient visit, the patient was recommended to undergo the blockage of the ganglion impar by radiofrequency ablation. A diagnostic blockage was first performed using 0.5% bupivacaine 2 ml because the patient was concerned about the destruction of the nerve ganglion. The patient was monitored clinically, and 3 months later, the VAS of 8/10 had decreased to 5/10. Approximately 1 week later, however, the pain recurred with a VAS of 7-8/10. The severity of pain was not significantly different from the pain which existed prior to treatment. The patient was therefore motivated to have blockage of the ganglion impar using BoNT-A. For blockage of the ganglion impar, the patient was placed in a prone position. A subcutaneous infiltration was performed in the superior area of the anococcygeal ligament. This area was chosen as a puncture point. Using a C-shaped image intensifier, a 22 G, 10 cm block needle in which the terminal part was bent at an angle of 30 degrees was advanced to a distance of 6 cm. Thus, attempts were made at the sacrococcygeal junction to reach the anterior surface. Following infusion of 2 ml of contrast media, the lateral and anterior-posterior views were evaluated (, ). Based on the spread pattern of the contrast media, the infusion was performed with a concomitant use of 0.5% bupivacaine 1 ml and BoNT-A 80 U. The VAS of 7-8/10 decreased to 3/10, but the VAS increased to 5/10 two months following the blockage. Accordingly, with the use of BoNT-A, 2 months following the blockage, the ganglion impar was blocked using 0.5% bupivacaine 2 ml and BoNT-A 100 U. When the patient underwent blockage using BoNT-A again, the VAS decreased to 2/10 and this was maintained for 6 months. The patient perceived the presence of perineal pain (a VAS of 5/10) during the sexual intercourse, which led to the third session of blockage of the ganglion impar at the same dose, which diminished the VAS to 2/10. At present, 3 months following the last treatment, other than perineal pain (a VAS of 4/10), the patient has had no problems in performing activities of daily living and work, including sitting and walking. The patient has maintained a VAS of 2/10. To evaluate the degree of disability due to chronic pain during activities of daily living, a pain disability index (PDI) [] was administered. The PDI has seven categories, such as activities associated with family/home responsibilities, recreation, social activities, occupation, sexual behaviors, self-care, and life-support activities, and scores are given with a scale ranging from 0 (no disability) to 10 (total disability). According to the PDI, scores were 10, 9, 10, 10, 10, 7, and 1, respectively, prior to treatment. Following the third session of blockage of the ganglion impar using BoNT-A, the PDI scores were greatly improved to 2, 2, 2, 2, 4, 3, and 0, respectively. These results indicate that the patient's condition had clearly improved. Currently, the patient has had a satisfactory outcome and is receiving follow-up observation. |
A 57-year-old man visited our hospital in April 2012 with the complaints of left hearing loss and left facial paralysis of approximately 3 weeks' duration. He had no significant previous medical history and no complaints of gastrointestinal symptoms such as nausea, abdominal pain, melena, hematochezia, or weight loss. In the abdominal examination, neither a palpable mass nor hepatosplenomegaly was noted. A neurological examination showed the loss of left-side forehead creases, the patient's inability to close his left eye, left facial muscle weakness, a rightward deviation of the mouth angle upon smiling, and a loss of the left nasolabial fold. His sensory and motor functions in the upper and lower extremities were normal. Cytological examination of the cerebrospinal fluid revealed no evidence of malignancy. Magnetic resonance imaging (MRI) of the brain showed a 1×1.5 cm tumor in the cerebellopontine angle that extended to the inner auditory canal. The tumor was clinically diagnosed as an acoustic neuroma (). Owing to the diagnosis of a benign tumor such as acoustic neuroma, the patient underwent surgery via the translabyrinthine approach, and the tumor was completely removed. In contrast with our initial diagnosis, however, the pathological examination of the surgical specimen revealed cells from a poorly differentiated metastatic carcinoma. Immunohistochemical staining did not help to identify the primary site. A further investigation to identify the primary lesion was performed postoperatively. Blood chemistry analysis indicated a serum carcinoembryonic antigen level of 1.34 ng/ml (reference range, 0-5 ng/ml). Other tumor markers were within normal limits. Abdomen/pelvic and thoracic computed tomography (CT) scans revealed no evident masses or regional lymphadenopathy that would suggest malignancy, except for a hepatic cyst, renal cyst, and mild splenomegaly. A positron emission tomography (PET)-CT scan revealed an abnormal hypermetabolic lesion in the stomach antrum but no other metastatic lesions. An upper gastrointestinal endoscopy examination found active ulcerative lesions on the anterior wall of the gastric antrum that were compatible with Borrmann type II gastric cancer. The pathological examination of the gastric antrum revealed cells from a poorly differentiated carcinoma. A total gastrectomy was performed and a pathological analysis of the surgical specimen revealed a poorly cohesive carcinoma that was histopathologically identical to that of the resected brain tumor (). On the basis of the results of the pathological examination, the case was finally diagnosed as gastric cancer with a solitary brain metastasis and was graded according to the TNM staging system as pT2 (invasion of proper muscle), N3b (involvement of 21 of 43 lymph nodes), and M1 (brain metastasis). According to the final pathology report, lymphovascular and venous invasion of gastric cancer were observed in the surgical specimen. The patient received palliative combination chemotherapy with intravenous oxaliplatin 130 mg/m2 of body surface area (BSA) on day 1 and oral capecitabine 2,000 mg/m2 of BSA on days 1 to 14 every 3 weeks. After 3 cycles of the combination chemotherapy, follow-up abdomen/pelvic CT scan and brain MRI showed no evidence of recurrence. The patient continued to receive the palliative combination chemotherapy on an outpatient basis, with a disease-free survival duration of 45 weeks. |
A 22-year-old male underwent penetrating keratoplasty for keratoconus in his left eye at another facility. He underwent intracameral air injection in his left eye at 1-week and 6-weeks postoperatively for an assumed diagnosis of DM detachment. Ten months later, the patient presented to our clinic with blurry vision in the left eye. On examination, his uncorrected visual acuity was 20/300 and intraocular pressure was 17 mmHg. Slit lamp microscopic examination indicated a clear graft with few folds and pigmented keratic precipitates. The anterior chamber showed mild flare and no cells. There were peripheral anterior synechiae and a mild fibrotic membrane over the crystalline lens []. The patient was diagnosed with a retrocorneal membrane that gave the appearance of a double anterior chamber.\nFurther work up included specular microscopy and anterior segment optical coherence tomography (OCT). Endothelial cell count was 850 cells/mm. The anterior segment OCT showed a retro-corneal membrane with double anterior chamber [].\nExtensive discussion occurred with the patient regarding the diagnosis as well as potential treatments. The use of the femtosecond laser was discussed. The patient was fully aware that the femtosecond laser was not indicated for such procedures and the proposed treatment was considered off-label and experimental. In addition, the patient was informed that the risks of femtosecond laser treatment for retrocorneal membrane were not fully understood. The patient consented to proceed with femtosecond laser-assisted surgical intervention for removal of the retrocorneal membrane. An informed consent was obtained from the patient.\nRemoval of the retrocorneal membrane with femtosecond laser was planned. The patient was taken to the femtosecond laser machine (LenSx®, Alcon Inc., Fort Worth, Texas, USA). The anterior capsulotomy was the only mode selected on the laser. After preparation of the patient with topical anesthetic and povidone-iodine solution, docking with good suction was achieved. The laser software automatically recognized the anterior lens capsule. The laser beam was manually changed and moved anteriorly to focus the laser spots on the retrocorneal membrane instead of the anterior capsule.\nDissection of the membrane was assumed to require more energy than a lens capsulotomy. Thus, the total energy was increased to 15 μJ. The laser settings were adjusted as follows: Circumference was 5.5 mm, spot separation was 3 μm, and line separation was 2 μm. Gates were set at 325 μm up and 375 μm down.\nAfter completion of laser treatment, the left eye was prepped and draped in the usual sterile fashion for ophthalmic surgery. The patient was transferred to the operating microscope. A 2 mm limbal corneal incision was made. An ophthalmic viscoelastic device (OVD) was injected into the anterior chamber. The retrocorneal membrane was removed with capsulorhexis forceps. The membrane was completely dissected and no tags were present. The OVD was removed from the anterior chamber with an irrigation and aspiration cannula. The wound was closed with a 10-0 nylon suture.\nPostoperatively, the patient was prescribed topical prednisolone acetate (1%) and moxifloxacin hydrochloride (0.5%) 4 times a day.\nOn the first postoperative day, UCVA was 20/125 and intraocular pressure was 16 mmHg. Slit lamp microscopy indicated a clear corneal graft; a well-centered 5.5 mm opening in the retrocorneal membrane, a deep anterior chamber with occasional cells and a clear lens. On the third postoperative week, UCVA improved to 20/50 and the intraocular pressure was 16 mmHg. The corneal graft was clear and the anterior chamber showed no cells or flare [].\nPostoperative investigations included tissue histopathology that established the diagnosis of retained DM. Ultrasound biomicroscopy showed retained segments of peripheral DM []. Specular microscopy showed a minimal decrease in endothelial cell count to 778 cells/mm. |
History and physical
A 67-year-old male former smoker with a history of prior occupational asbestos exposure and recurrent bronchitis presented with progressive dyspnea and thoracic pain to the point that he could not lie down in bed. A computed tomography (CT) scan of the chest was performed, which was interpreted as right-sided pneumonia with right parapneumonic effusion. He was sent to his local emergency department, where he was admitted for antibiotics and thoracentesis, the latter which demonstrated the presence of atypical mesothelial cells with inflammatory cells. He was readmitted two weeks later for progressive thoracic pain, was found to have a recurrent right-sided pleural effusion, and was managed with partial right pleurectomy with pleural biopsy, and talc pleurodesis. Right pleural pathology demonstrated atypical mesothelial proliferation at the pleural surface, without true invasion or definitive pathologic evidence of malignancy. Following surgery, he felt substantially better, such that he could sleep in the bed again, and he was able to return to his baseline activity levels. He underwent repeat chest CT five months later, which showed right pleural thickening and a small loculated pleural effusion, favored to represent a combination of calcification, pleurodesis, and atelectasis. He remained clinically well for another five months until he presented with cough and sinus congestion unrelieved by guaifenesin, dextromethorphan, and antibiotics. He underwent repeat chest CT that showed extensive mass-like pleural thickening completely encasing the right lung, with prominent involvement of the mediastinal pleura, and probable mediastinal extension into the right paratracheal and precarinal space, with pericardial effusion and probable pericardial metastases. There was no definite invasion into the right chest wall and no evidence of disease outside of the thorax.
He then established care at our institution’s mesothelioma and pleural disease multi-disciplinary program. Pathology review of the previously biopsied pleural tumor revealed that the pleural tumor cells were positive for Wilms' tumor-1 and calretinin, and negative |
A 30-year-old female patient reported to the department of oral and maxillofacial surgery with complains of growth in the left upper jaw at the site of extraction wound. History revealed she was apparently alright 15 days back; she visited a local dentist for pain in the left upper back teeth, which were found to be mobile and were extracted. Following extraction she noticed a small growth which was insidious in onset and gradually increased to the present size at the site of extraction after 15 days of teeth removal. She revisited the same dentist who referred to our institution. Her medical and family history was not contributory. She was happily housewife with two children. She did not have any abusive habits.\nOn examination a single, lobulated, sessile pink in color, nonpulsatile lesion was found to be present in 25, 26, 27 regions which extended beyond the occlusal surfaces of teeth both buccally and palatally of approximately 4 cm × 3cm in size []. On palpation, the swelling was fibrous and edematous which was interfering with the occlusion []. There were indentations of the lower teeth on the swelling. There was no regional lymphadenopathy. Differential diagnosis of squamous cell carcinoma and benign tumors were considered. Orthopantomogram (OPG) and PNS view revealed haziness in the left maxillary antrum with radiolucency extending in the alveolus of 25, 26, and 27 regions. Otherwise, her skeletal survey was normal. The CT scan of maxilla [Figures –] revealed break in continuity of buccal, palatal walls, and inferior wall of maxillary antrum. An incisional biopsy was carried out after doing routine blood investigations. Western blot test revealed that patient was HIV positive. The biopsy specimen revealed sheets of abnormally large lymphoid cells with high nucleus: cytoplasmic ratio, coarse chromatin, and inconspicuous nucleoli with abnormal mitotic []. The features were suggestive of large cell lymphoma of B-cell type. Immuno-histochemistry revealed positive CD20, LCA, and CD3, CK were negative, which proved conclusively the lesion to be as a NLM, diffuse large B-cell type. The HIV status was in the fourth stage which superseded non-Hodgkin's lymphoma as per WHO classification. History regarding the HIV-positive status did not reveal any mode of transmission of HIV infection.\nShe was referred to the oncologist. The patient was started on anti-retroviral treatment. Chemotherapy was advised by the oncologist and a total of three cycles were suggested at the gap of every 3 weeks. The treatment regimen followed was that of classical CHOP therapy which comprised of using cyclophosphamide, doxorubicin (hydrodoxorubicin), vincristine (oncovin), and prednisolone. At the end of two cycles the swelling started regressing in size and totally disappeared after three cycles. The growth completely disappeared after chemotherapy []. The patient died 6 months after chemotherapy. |
A 56-year-old female presented for the evaluation of gradual onset bilateral soft tissue masses involving the plantar surface of both feet, with the left foot being significantly more symptomatic. Her past medical history included hypertension and high cholesterol. Social history included gainful employment in a moderately labor-intensive type job and several-year history of smoking. Her physical exam was essentially normal with the exception of somewhat firm, slightly mobile nodular masses involving both plantar foot surfaces in the region of the plantar medical arch (Figure ). Tenderness was only noted involving the two masses on the left foot. Plain radiographs were negative. Conservative therapies were discussed and tried that included shoe gear and activity modification as well as analgesics. The patient declined injection therapy. An MRI was ordered that revealed two discrete soft tissue masses within the plantar fascia consistent with PF lesions without neurovascular encroachment (Figures , ). Surgery was proposed and she wished to proceed with surgery on her symptomatic left foot (Figure ). Risks and benefits were outlined for both complete fasciectomy and wide en bloc resection. The patient chose the latter technique and was advised to refrain from smoking that she did for several days both pre- and post-operatively. Surgery was completed without complications. Wound management included placement of a battery-powered ciNPT device (Prevena™) and light compression bandaging (Figure ). The device remained in place for one week and then was removed. No significant erythema or swelling was seen. Surgical pathology was consistent with two, benign PF lesions. Sutures remained in place for a total of three weeks and were then removed. At this time, the incision was found to be negligibly tender, flat and well coapted. She was non-weight bearing immediately post-operatively through suture removal at which time she was transitioned to a walking boot with instructions to gradually increase weight-bearing activities to tolerance. Rechecks were unremarkable with the exception of a small area of dehiscence that was seen one week after suture removal. Complete incisional healing was appreciated about a week after suture removal. She returned to normal, daily activities including unrestricted work just over two months with no symptomatology or recurrence at the one-year mark. |
The patient is a 24-year old male who was diagnosed with right distal thigh MLS in 2018. He had wide local excision with neo-adjuvant radiation (50 Gy/25 fractions).\nIn February 2019, an MRI of the femur was obtained for restaging. The MRI did not show any signs of local tumor recurrence but was highly suspicious for an intramedullary enhancing osseous lesion involving the proximal right femur. A follow-up positron emission tomography (PET)/computed tomography (CT) was performed and revealed a mildly hypermetabolic intramedullary lesion within the proximal shaft of the right femur that was suspicious for malignancy.\nIn May 2019 the patient was given 2 cycles of chemotherapy (ifosfamide and doxorubicin), and later in the same month, an MRI for the thigh showed further progression in the proximal right femoral osseous lesion.\nIn June 2019, a biopsy was performed and was negative for any features of malignancy. Definitive radiotherapy (70 Gy/35 fractions) to the right femoral lesion was delivered to the patient by October 2019. The patient was then observed by regular follow up.\nIn November 2020, the patient presented to the emergency department with a decreased level of consciousness, headache and vomiting. The patient also had a gross lesion in his forehead which he neglected for a while. CT scan was performed and showed a left frontoparietal mass measuring 6 × 5.4 cm with vasogenic edema. The patient was admitted and treated conservatively without any surgical interventions as he was COVID-19 positive with mild symptoms. Chest, abdominal and pelvic CT scans were done and were negative respectively.\nIn December 2020, the patient was re-admitted with lethargy, drowsiness and decreased level of consciousness. Brain MRI showed a large contrast-enhancing left frontal intracranial mass with mass effect and uncal herniation (). His Glasgow Coma Scale (GCS) score dropped during hospitalization to 6/15 and the pupil response was sluggish with left pupil dilation. He underwent left frontal craniotomy urgently with tumor resection including the affected bony involvement (), except for a small part invading the superior sagittal sinus, followed by cranioplasty with of the methyl acrylic bone.\nHistopathological analysis showed metastatic myxoid/round cell LPS. The tumor cells are negative for GFAP, S100, WT1, BCOR, SATB2, CD99 and cyclin D1.\nThe tumor had infiltrated the skull bone and extended the peripheral margins (). The case was discussed with the multidisciplinary clinic and the patient received stereotactic radiosurgery for the resection cavity. On week 6 follow up, there was no evidence of residual or tumor recurrence in the surgical bed. A new right parietal bone with soft-tissue component metastasis was treated with radiotherapy.\nA follow-up staging CT scan showed a soft-tissue mass at the suprasternal notch related to manubrium sterni and an anterior peritoneal with involvement of linea alba metastatic deposit was found. Palliative external beam radiotherapy to the sternu was completed by January 2021.\nFour months later, follow-up brain MRI revealed a right occipital condyle lesion, mostly correlating with osseous metastasis. There was no evidence of recurrence at the surgical bed (), and no significant change in the previously treated right parietal lesion. The patient was treated with radiotherapy for the right occipital condyle lesion.\nThe patient was observed by regular follow-up imaging. On his 6th month follow up, he was alive and all lesions were stable except the peritoneal lesion was slightly progressive. He was placed on single-agent dacarbazine. |
A 62-year-old female presented for the evaluation of a symptomatic soft tissue mass involving the right plantar medial arch. Her symptomatology was relatively insidious by history and what prompted her visit was gradual intolerance of a certain shoe gear. She reported the mass was present for over a year but symptomology escalated about four months prior to initial evaluation. Her past medical history included hypertension, thyroid disease, Factor V Leiden pathology with a history of deep vein thrombosis, degenerative joint disease, and morbid obesity. Social history was unremarkable. Her physical exam was largely unremarkable except for significant pes planovalgus changes in both feet and a painful mass involving the right foot. Specific examination of the soft tissue mass revealed a mildly firm, slightly mobile, tender soft tissue mass in the medial plantar arch. Plain films demonstrated no acute process. Both non-operative and operative treatment options were offered and the patient chose the latter, electing for wide en bloc excision instead of complete fasciectomy. Prior to surgery, an MRI was ordered that demonstrated changes consistent with a solitary plantar fibroma lesion (Figures , ). Her surgery was without compromise and included perioperative anticoagulation bridge management in light of her history of deep vein thrombosis. Wound management included placement of a battery-powered ciNPT device (Prevena™) and light compression bandaging. At bandage takedown, the incison was observed to be well coapted with no complications (Figure ). The device remained in place for one week and was then removed. No drainage strikethrough was seen (Figure ). Surgical pathology was consistent with a solitary benign plantar fibromatosis lesion. Sutures remained in place for a total of three weeks. A walking boot was used for two weeks after suture removal. Healing progressed uneventfully with gradual increase in weight bearing and activities. Dorsal foot swelling on the operative foot persisted but eventually resolved. She ultimately returned to normal shoe gear at three months and was discharged from care without symptoms or signs of recurrence at six months. |
A 20-day-old girl baby (weight: 3 kg; length: 48 cm) with D-transposition of great arteries with a large perimembranous ventricular septal defect (8 mm) and Type A aortic interruption underwent single-stage aortic arch repair with arterial switch operation and closure of the ventricular septal defect through a median sternotomy. Tracheal extubation was done after 36 h of mechanical ventilation. Before tracheal extubation, the baby was comfortable, generating about 5 ml/kg tidal volume on synchronized intermittent mandatory ventilation with a backup respiratory rate of 10 breaths/min, positive end-expiratory pressure of 5 cmH2O, fraction of inspired oxygen of 0.3, and a pressure support of 8 cmH2O. As the arterial blood gas reports were satisfactory on these settings, tracheal extubation was done, and within a short span of time, the child had complete left lung collapse. This did not improve despite administration of high-flow humidified oxygen-enriched air through a nasal cannula that was delivered at a rate of 2 L/kg. This flow through the nasal cannula was to generate a continuous positive airway pressure (CPAP) of about 5–6 cmH2O. The lung collapse was attributed to paresis of the left dome of the diaphragm as ultrasound of the chest suggested decreased diaphragmatic movements although they were not paradoxical.\nFollowing tracheal reintubation, fiber-optic bronchoscopy was performed, which showed left bronchial obstruction due to external compression [ and ]. The fiber-optic bronchoscope was advanced beyond the obstruction, and it was found that the bronchial lumen was patent with no significant secretions or mucus plugging. On withdrawal of the bronchoscope, posterior indentation of the left main bronchus was seen in the form of transmitted pulsations from the aorta []. This was due to the reconstructed descending aorta that had been pulled up and anastomosed to the distal ascending aorta, causing a pulsatile mass effect on the bronchus. A diagnosis of left lung collapse due to left main bronchial obstruction secondary to compression by the descending thoracic aorta was made. As a reoperation for surgical mobilization of the vessels was not easy, a trial at conservative management was planned. The steps in conservative management envisaged were: (a) conversion from invasive mechanical ventilation to noninvasive ventilation in the form of application of CPAP, (b) gradual weaning from CPAP support to high-flow oxygen-enriched air through nasal prongs and later to face mask oxygenation, (c) control of blood pressure and heart rate to reduce the mass effect of the aorta on the bronchus, (d) constant observation of the child's spontaneous ventilation capabilities, and serial blood gas analysis and urgent chest radiographs when needed. It was decided that any deterioration in the child's ventilation mechanics would warrant some kind of “release” surgery.\nAfter 3 h of mechanical ventilation, tracheal extubation was performed. The ventilator settings were similar to those that were used at the time of the first tracheal extubation. Chest radiographs before and after tracheal extubation showed complete reexpansion of the left lung []. As planned, noninvasive ventilation in the form of CPAP (about 9 cmH2O) was applied by nasal prongs with the help of an infant Flow SiPAP system (VIASYS Healthcare, Yorba Linda, CA 92887, USA) []. Baby improved over a period of 6 days on nasal CPAP and was subsequently transferred to high-flow oxygen-enriched air through nasal prongs []. The left lung remained completely expanded even after removal of CPAP []. The blood pressure and heart rate were controlled to age appropriate levels (systolic/diastolic blood pressure: 75–80/45–50 mmHg [approximately] and heart rate: 120–140 bpm) with oral propranolol (0.5 mg TID) and captopril (0.5 mg OD). Computerized tomography (CT) performed on the 10th day showed left bronchial narrowing with segmental collapse of the left upper lobe []. The lung collapse was not identified on the chest radiograph which continued to appear normal. As the baby was not exhibiting any features of respiratory distress and was clinically normal, the child was discharged home. During the 3-month follow-up, the left lung stayed fully expanded, and no further intervention was deemed necessary. |
A twenty-two-year-old male with extensive limb deformities reported to the department of Oral Medicine, Diagnosis, and Radiology with a chief complaint of unclear speech and difficulty in tongue movements.\nThe patient's medical history was insignificant. He was the fourth child of normal and unrelated parents. He had four siblings: one brother and three sisters all of whom were normal.\nHis prenatal history revealed that his mother had four episodes of fever during her first trimester and was medicated for the same. She was unable to remember the names of the medications. She had spontaneous vaginal delivery after thirty-nine weeks of gestation to give birth to a male baby with multiple limb deformities. Patient's family history for congenital abnormalities was negative.\nOn general examination, a healthy, moderately built male of normal intelligence presented to the clinic with an altered gait. He had anomalies affecting all the four limbs. His right hand showed brachydactyly (short digits) of second to fourth fingers with hypoplastic thumb and little fingers. Finger nails appeared normal (). In the left hand, hemimelia (missing half the limb) was observed below the elbow with five nubbins attached to its distal part. Radiograph of the right hand showed hypoplastic second and third metacarpals with deficient second to fourth phalanges. Absence of distal part of the radius and ulna was noted in the left hand radiograph with subluxation of the superior radioulnar joint. Lower limbs were also severely deformed. Right limb showed hypoplastic foot with adactyly (missing digits). Left limb had apodia (missing foot) with adactyly (). Radiographically, right limb revealed hypoplasia of the phalanges and first metatarsal bone; left limb revealed absence of tibia, fibula, and both the malleoli. Detailed systemic clinical examinations with appropriate investigations were also performed. Though patient was clinically normal, his chest radiograph showed prominent pulmonary bay. Further, Color Doppler ultrasonography revealed pulmonary artery dilatation with pulmonary regurgitation ().\nOn extraoral examination, his lips were competent; profile was convex with a deep mentolabial sulcus ().\nIntraorally, his tongue was hypoplastic; about two thirds the normal size with a rounded tip (). The tip was attached to the floor of the mouth by a short lingual frenum and positioned about a centimeter behind the lower incisors (). This restricted the protrusive and lateral movements of the tongue. But speech was affected minimally.\nMandibular anteriors were retroclined with congenitally missing right central incisor (). There was no radiographic evidence of impaction. Palate was V shaped, high arched, and narrow. Maxillary left lateral incisor was palatally positioned, thus blocking the regular alignment of mandibular anteriors. Anterior deep bite was observed (). Root stumps of maxillary left first premolar, mandibular first molars, and mandibular right second molar were present ().\nPresence of a small tongue in association with extensive limb deformities led to the diagnosis of oromandibular limb hypogenesis syndrome type IIB, hypoglossia-hypodactyly. Although patient complained of altered speech and difficulty in tongue movements, he refused to undergo surgical procedure for the correction of the same. Basic oral rehabilitation procedures like extraction of the root stumps and oral prophylaxis were performed. |
A 39-year-old woman, gravida 1, para 0, was diagnosed with invasive squamous cell carcinoma of the cervix following conization. Pathological findings showed carcinoma consistent with FIGO stage IA1 with lymphovascular invasion. She was referred to Keio University Hospital. The patient and her husband were informed of the treatment options, including AmRT and pelvic lymphadenectomy. The patient was told that the outcome of this procedure could not be guaranteed because an insufficient number of these procedures have been performed worldwide to yield reliable conclusions. The patient wished to preserve fertility, and she and her husband signed a written consent form agreeing to this treatment. Pathological findings after AmRT and pelvic lymphadenectomy showed no residual tumor and no lymph node metastasis. There was no finding of an ovarian tumor before surgery. A left ovarian cyst of 4 cm was identified during postoperative follow-up.\nAt 3 years and 6 months after surgery, the patient underwent IUI and then had fever and pain in her left lower abdomen 10 days later. At her first visit, her temperature was mildly elevated to 37.5°C. The patient's pregnancy was denied because a qualitative urine human chorionic gonadotropin (hCG) test was negative. A tumor with tenderness was palpated in the left adnexal area. A cystic tumor of 64x 41 mm was found by transvaginal ultrasonography (). Blood tests showed increases in white blood cell (WBC) count to 11900/μL and C-reactive protein (CRP) to 22.80 mg/dL. The patient was diagnosed with PID with ovarian cyst infection and hospitalized for treatment. Conservative treatment with antibiotics was initially used, but her symptoms did not improve. On hospital day 8, blood tests showed a further increase in WBC count to 23900/μL and CRP to 28.17 mg/dL, and pelvic CT showed that the ovarian cyst had grown to 10 cm in size ().\nWe decided to perform laparoscopic left ovarian cystectomy on day 8. Since the patient had a history of open surgery, adhesion was likely in the abdominal cavity. Before surgery, we asked the urologist to insert bilateral 6 Fr ureteral catheters because of possible difficulty identifying the ureters. The catheters were fixed to the thigh with tape (). The uterine manipulator was not inserted before surgery to avoid the risk of uterine perforation.\nCO2 pneumoperitoneum was established at 10 mmHg. Laparoscopic left ovarian cystectomy was performed using typical trocar placement. The left ovary was swollen to 10 cm and the fluid contents were purulent (). The left adnexa, posterior uterine wall, and retroperitoneum were firmly adhered (). The bilateral fallopian tubes were firmly adhered to the surrounding tissue and were unable to be identified. During surgery, by moving the catheter manually back and forth from outside the body, we were able to identify the ureters visually (). The 7 cm uterine manipulator was inserted during surgery under a laparoscopic view. The left ovarian cyst was excised, leaving the normal part of the ovary (). The operation time was 2 h and 58 min, and blood loss was 550 mL. No complications occurred during or after surgery. After the operation, symptoms improved rapidly and the patient was discharged 8 days after surgery. The excised specimen was pathologically an endometriotic cyst. A bacterial culture of the cyst fluid was positive for Prevotella bivia, Prevotella species, and Finegoldia magna. |
A 7-month-old girl was referred to our institution in December 2009 with a right femoral lesion, with concerns of neoplasm or osteomyelitis. Her parents noticed that she had shown a decrease in the use of her right lower extremity especially at about 3 months of life when the patient would not bear weight on her right lower extremity when held up in an upright position. The patient’s ability to sit upright was also somewhat limited because she preferred to have her right hip and knee extended. At age 7 months, her pediatrician obtained a roentgenogram that showed a lytic lesion in the right proximal femur. The past medical history was unremarkable. Clinical examination showed an afebrile infant who moved her right lower extremity much less than the left and preferred keeping her right hip and right knee extended with external rotation. There was no erythema, warmth or mass in the right lower extremity. The results of routine laboratory studies were normal.\nRoentgenograms of the right femur showed an osteolytic area in the intertrochanteric region, surrounded by a sclerotic rim and lamellar periosteal reaction (Fig. ). A magnetic resonance imaging (MRI) study showed a focal, 1.1 × 0.9 × 1.2 cm, high T2 signal lesion centered in the intertrochanteric region of the proximal right femur. There was marked lamellar periosteal reaction involving the proximal right femoral metaphysis extending to the mid diaphysis, with extensive, ill-defined intra- and extraosseous edema (Fig. ). The differential diagnosis included chronic osteomyelitis with a Brodie’s abscess, eosinophilic granuloma, osteoid osteoma, and small round blue cell tumor such as Ewing sarcoma or metastatic neuroblastoma.\nA CT-guided core needle biopsy of the lesion was performed. The axial CT scan at the time of biopsy showed a 1-cm lucent lesion eccentrically located within the medial aspect of the proximal femur with marked periosteal new bone formation (Fig. ). Cultures of the biopsy tissue were negative for fungal and bacterial organisms. Histological examination of the biopsy tissue showed an area of new woven bone formation with prominent osteoblastic rimming in a background of loose, cellular, fibrous stroma (Fig. ). There were no features of acute osteomyelitis, small round blue cell tumor, or eosinophilic granuloma. A diagnosis of OO was considered.\nThe infant subsequently underwent an RFA procedure, performed under CT guidance and general anesthesia. To reduce radiation exposure, only 20 mAs with 80 kVp was used, which resulted in markedly increased image noise, however, still allowing visualization of the lesion. Cefazolin sodium was administered during the procedure as a prophylactic to avoid osteomyelitis. After placement of the electrode-grounding pads and marking the entrance site on the skin, a 14G Bonopty penetration set (AprioMed, Uppsala, Sweden) was inserted and attached to the cortical bone. Then a 16G drill and extended drill (AprioMed) were advanced to the lesion.\nSubsequently, RFA was performed using a Cool-tip™ RFA system (Tyco Valleylab, Boulder, CO) in a noncooled mode. A 17G 15 cm RFA probe with a 0.7 cm active tip was inserted. Using this probe and a standard heating procedure by heating the tip of the electrode to 90°C for a period of 6 min, a tissue area of 0.5 cm around the active tip was ablated. No complications were observed during or immediately after the procedure. The patient was discharged the next day.\nAt the follow-up 1 week later, the patient was able to place weight on her right leg when held up in an upright position and sit, unassisted, for several minutes. The patient presented again 3 months later with signs of recurrence, including inability to bear weight and decrease in the use of her right lower extremity. The follow-up radiographs showed persistent osteolytic area in the intertrochanteric region but less prominent periosteal reaction as compared to the radiographs prior to treatment. A second CT-guided RFA was performed 4 months after the initial procedure by using again a 0.7 cm active tip RFA probe with the same standard technique. The CT images showed a decrease in size of the nidus, which measured 0.7 cm in diameter (Fig. ). Additionally, the periosteal reaction has improved when compared to the initial scans. One month after the second ablation, the patient still did not show signs of pain and began to stand on her legs. |
The patient is a 54-year-old Caucasian female, of height 170 cm and of weight 63 kg, who developed difficulties to open her feast at the age of 32 years. Until then she was working as a sport teacher and was performing high performance sport without problems. Since then she noted that particularly her explosive strength became impaired and she once fell when walking backward. Afterward, she recognized difficulties when climbing stairs and since age 48 years a feeling of muscle tension in the thighs was triggered by quick movements or voluntary contractions. She also noted that muscle stiffness improved during continuous exercise, being interpreted as warming-up phenomenon. Nonetheless, she was able to run marathons without major problems. Since age 51 years myotonia had affected the entire musculature, including the cervical and abdominal muscles, which she recognized only during sport as muscle burning without being handicapped. Cold or alcohol did not worsen myotonia. Her further history was noteworthy for right Achilles tendon rupture at the age of 20 years and ligament rupture of the left ankle also at the age of 20 years. Work-up for a paraneoplastic syndrome was negative. At the age of 45 years, struma uninodosa with hypothyroidism was diagnosed. Her mother and three second cousins also presented with myotonia. She was regularly taking only l-thyroxin (50 µg/day). At the age of 50 years, myotonic dystrophy had been suspected for the first time.\nClinical neurologic examination at the age of 51 years revealed mild atrophy of the temporalis muscles, mild myotonia and warming up after forced lid closure, and percussion myotonia of the thenar and the gastrocnemius muscles. Creatine kinase (CK) was mildly elevated to values between 300 and 600 U/L since at least age 32 years (). There was mild, constant leukopenia. Liver enzymes were mildly elevated since age 22 years (). Ophthalmologic investigations revealed an incipient cataract bilaterally. Nerve conduction studies revealed an increased latency and a reduced amplitude of the peroneal nerves bilaterally and a reduced amplitude of the right median nerve. Needle electromyography (EMG) of the right deltoid and right anterior tibial muscle was normal. EMG of the vastus lateralis muscle bilaterally revealed abnormal spontaneous activity in the form of positive sharp waves but normal muscle architecture. Magnetic resonance of imaging (MRI) of the thighs revealed a T2-hyperintensity of the semimembranosus and semitendinosus muscles bilaterally, being interpreted as edema. Cardiologic examination was normal. Ultrasonography of the abdomen revealed ovarial cysts bilaterally. Gastroscopy revealed gastritis and reflux. Genetic investigations disclosed no CTG-repeat expansion on the DMPK (Dystrophia Myotonica Protein Kinase) locus. Investigation of the CNBP/ZNF9 locus revealed a heterozygous CCTG-repeat expansion of 500–9500 repeats, why PROMM was diagnosed. Clinical neurologic examination at the age of 54 years was completely normal, particularly no clinical myotonia could be detected this time. The trapezius percussion sign was negative, there was no tremor, no calf hypertrophy, and she reported no restless-leg-syndrome or myalgias. She denied hypersomnia, cognitive deficits, or previous complications during general anesthesia. There was no autonomic involvement (normal heart rate response to Valsalva or change of posture, normal heart rate variability in time and frequency domain, no increased QT-variability). She reported recurrent infections. |
A baby boy was born to a 32-year-old primigravida at 34 weeks gestation. Antenatal ultrasound done at 28 weeks detected polyhydramnios and foetal anomaly, where a huge mass of mixed cystic and solid echogenicity was detected arising from the left side of his face, measuring more than 10 cm in its maximum dimension. The baby was born by Caesarian section after foetal distress was detected. He developed respiratory distress at birth which required intubation and assisted ventilation.\nPhysical examination revealed a large spherical mass at the left cheek measuring approximately 8 cm x 10 cm in size. There was significant displacement of his mouth to the right, his nose superiorly and his left eye superolaterally, due to the sheer size of the mass (). The overlying skin appeared healthy, with no ulceration or discolouration. On superficial palpation, the mass had a mixed soft to hard consistency. There was a haemangioma measuring 3 cm x 1 cm in size over his right nipple. Further examination showed that the other systems were normal. Maturity scoring of the baby was appropriate for age. The differential diagnoses from physical examination included teratoma, primitive neuroectodermal tumour (PNET) and embryonal sarcoma.\nHaemoglobin, white blood cell count and platelets were normal. Serum α-fetoprotein (AFP) measured 56852 IU (normal 0-8 IU) and β-human chorionic gonadotropin (β hCG) 14 IU (normal <10 mIU). The high levels were in keeping with a teratoma. A plain radiograph of the face and skull showed a wide opened oral cavity, poorly developed left temporomandibular joint and calcifications within the large soft tissue density mass (). On MRI, there was a large and lobulated tumour that demonstrated mixed signal intensities in keeping with fluid, fat and soft tissue components on both T1 and T2 weighted sequences. The left globe of the eye was displaced superiorly. The internal portion of the tumour effaced all normal anatomy whereby the left side of the oral cavity, oropharynx, nasopharynx, palate and left temporomandibular area had no clear margins from the tumour. There was extension of the tumour into the left temporal fossa ().\nSurgery on day 7 of life revealed findings which largely corresponded to findings on MRI. The tumour extended from the left temporomandibular joint to the nasal septum. It occupied the oropharynx and nasopharynx, caused elevation of the buccal mucosa and upper airway obstruction. There was extension superiorly into the base of the skull and inferiorly into the oral cavity, displacing the left mandible, as well as posteriorly to the lesser wing of the sphenoid where there was a defect covered by a thin membrane. The condyle and coronoid process of the left mandible were not well developed and no articulation existed between the left mandibular condyle and the temporal bone. The tumour was removed en-bloc with minimal difficulty and the cavity packed with surgicel and gelfoam. A skin flap was raised via circumferential dissection to cover the incision site over the left side of the face.\nCut section of the tumour showed multi-loculated cysts containing serous fluid and solid areas. Histopathology revealed tumour tissue consisting of a haphazard mixture of organoid mature tissue composed of skin adnexal tissue and microtubular structures. Calcifications and cysts lined by stratified squamous epithelium with rosettes were identified in one area of the section. These features were in keeping with a teratoma with immature neural elements. No mitotic or undifferentiated elements were seen ().\nPost-operatively, there was a significant decrease in serum α-fetoprotein to 4408 IU/ml. However, the left side of the face became increasingly swollen and there was difficulty extubating the baby despite clearance of tumour from the airway at surgery. A repeat MRI and CT scan done 2 weeks after surgery showed a mass of solid and cystic nature occupying the oral cavity with extension into the left temporal fossa. It showed avid enhancement with contrast and it extended posteriorly compressing the brain stem and causing obstructive hydrocephalus (). The endotracheal tube was displaced to the right side by the mass. The baby underwent another surgery. It showed recurrence of tumour in the oral cavity and oropharynx with extension into the left temporal fossa through the membranous covering. There was no further attempt to excise the tumour. A tracheostomy and external ventricular drainage was planned, but the baby succumbed 3 weeks later. |
Our patient is a 40-year-old Asian man with complaints of bleeding and discomfort in his anus of 2 months’ duration. He was an employee with average income who did not smoke tobacco or drink alcohol. He had no weight loss or urinary symptoms, and no substantial family history. He denied any significant medical or surgical history. His abdomen was soft, non-tender, and non-distended, with normoactive bowel sounds. In examination, a mass could be touched by finger tips. The mass was large and bleeding. In subsequent examinations, blood was detected in a stool sample. His vital signs were: blood pressure, 130.77 mm Hg; respiratory rate, 18 breaths/minute; heart rate, 83 beats/minute; and temperature within normal limits. Oxygen saturation was 98% on room air on admission. In colonoscopy, a large lobular tumor was diagnosed at 4 cm above the dentate line, which was suspicious for malignancy. Various samples were taken from the tumor. The rest of his large intestine did not show a clear pathologic lesion in the colonoscopy.\nHigh-grade adenocarcinoma was reported in pathological examinations. In subsequent diagnostic procedures, his carcinoembryonic antigen (CEA) level was normal. Computed tomography (CT) scans revealed that metastatic lesions were not detected in his liver, abdominal viscera, and chest. In CT scans with or without contrast, and magnetic resonance imaging (MRI) scanning, an ectopic kidney was detected incidentally on his right pelvis without any prior urinary symptoms. The left kidney was in its original location, and both kidneys were functional. Renal function tests provided normal results.\nIn subsequent investigations done by MRI scanning for staging the tumor, a pelvic rectum tumor was reported to be interfering with the T3 N1 mesorectal lymph nodes (Figs. , ). The case was discussed in a multidisciplinary cancer team; afterward, our patient was regarded as a candidate for neoadjuvant radiotherapy. He underwent 45 GY radiation in 25 fractions to the pelvis along with capecitabine. He underwent total mesorectal excision (TME) surgery to maintain the ectopic kidney 6 weeks later. After abdominal exploration, his abdominal viscera were examined. There was no metastatic lesion in his liver and abdomen (Fig. ).\nAfter mobilization of the left colon and the splenic flexure, and the closure of the inferior mesenteric artery (IMA), in the avascular plane, the mesorectum was separated from the fascia propria, and the mesorectal lymph nodes and hemorrhoidal vessels in the anterior and pelvic nerves were fully mobilized, and the distal rectum was removed by an appropriate margin (Fig. ).\nHis right kidney was completely inside the pelvis, and while the kidney was carefully protected by the retractor, an attempt was made to minimize the damage to the ectopic kidney because there was a possibility of damage to the pelvic nerve and nephrectomy.\nThe blood of the right kidney appeared to be supplied by the right superior iliac artery. During the surgery, hematuria occurred to our patient, which was resolved by hydrating him. Then, coloanal anastomosis and temporary ileostomy were performed on our patient. He was transferred to our intensive care unit (ICU). He underwent laparotomy again due to anastomosis leakage a week following the surgery. As a result, a colostomy was performed. Postoperatively, after the reappearance of symptoms, stabilization, and healing of the wounds, he was referred to medical oncology and started adjuvant chemotherapy with 5-fluorouracil, folinic acid, and oxaliplatin (FOLFOX). Follow-up testing (for a year) included routine medical history and physical examination (every 3–6 months), blood tests such as serum CEA, colonoscopy, and radiologic imaging. He was dissatisfied with the permanent colostomy after the end of the treatment. However, the satisfying result was that his kidney was preserved (Additional file ). Before the surgery, the potential risks and damage to his ectopic kidney and the possibility of its removal were explained to our patient and his consent was obtained. His general condition is appropriate after 1 year and his quality of life has been reported to be satisfying despite the permanent colostomy. |
A 59-year old female with a history of hypertension presented to the outpatient clinic in East Jeddah Hospital, Saudi Arabia, with sore throat, cough and arthralgia for 2 days. A nasopharyngeal swab for COVID-19 testing was performed as per the protocol during the current COVID-19 pandemic. The swab returned positive for COVID-19 and the patient was started on azithromycin (500 mg orally for 3 days) and paracetamol (1 g BID orally). Nine days later, the patient presented to the emergency department with drowsiness, loss of appetite and decreased activities. Patient was admitted and was initiated on prophylaxis enoxaparin 30 mg subcutaneously q12hr.\nPatient was conscious with normal vital signs on admission and CT brain was unremarkable (Figure ). Her chest radiograph showed bilateral infiltration with extensive peripheral consolidations (Figure ). Laboratory analysis showed markedly elevated D-dimer, fibrinogen and C reactive protein (CRP). Other laboratory values are presented in Table . On day 2 after admission, the patient deteriorated and became unconscious and developed acute renal shutdown. She was started on extensive medical therapy and serial CT angiography. Hematologist was consulted and the patient was shifted from prophylaxis enoxaparin into therapeutic enoxaparin 30 mg IV bolus dose followed 8hr later with maintenance dose enoxaparin 60 mg SC q12hr and monitored with the PTT and fibrinogen level. CT head was obtained on day 2 post admission demonstrating bilateral frontal lobe ischemia (Figure ). While in hospital, she became increasingly hypoxic and required intubation and mechanical ventilation. Serial CT brain showed diffuse ischemic insult involving left cerebral hemisphere, right frontal lobe and right cerebellum (Figure ). Her mental function continued to deteriorate with extensive involvement of anterior, middle and posterior cerebral artery complicated with brain edema (Figure ). The patient remained unresponsive and showed an abnormal breathing pattern on mechanical ventilation. The patient died on day 4 after admission due to severe post-ischemic brain edema, raised intracranial pressure (ICP) and uncal herniation. |
The patient is a 37-year-old man with a past medical history of hypertension, diabetes mellitus, extensive hypercoagulable history, and a remote history of exploratory laparotomy for an embolectomy of the superior mesenteric artery and a small bowel resection of the jejunum. He presented to the Emergency Department with an 8-hour history of worsening, crampy abdominal pain, nausea, vomiting, and multiple episodes of hematemesis. He had a bowel movement prior to arrival, but otherwise his review of systems was unremarkable. Physical examination was notable for hemodynamic stability and a mildly distended abdomen with epigastric tenderness but without guarding or rebound tenderness. Given his prior surgical history, a computed tomography (CT) of the abdomen was obtained, which showed dilated loops of bowel with a transition point in the region of the proximal ileum, just distal to his prior bowel anastomosis. The distended proximal small bowel loops contained fecalized material, and the distal small bowel loops were also decompressed without evidence of ascites, bowel wall enhancement, or free air ().\nThe patient was admitted and initially managed nonoperatively with fluid resuscitation, pain control, bowel rest with nasogastric tube decompression, and serial abdominal exams. Following return of bowel function, the nasogastric tube was discontinued on hospital day 2 but reinserted hours later due to persistent, new-onset vomiting, and worsening abdominal distention. Without significant improvement in symptoms, the patient was taken to the operating room urgently for an exploratory laparotomy. Intraoperatively, the small bowel was dilated to approximately the midjejunum, where an intraluminal obstruction was palpated and subsequently identified to be a phytobezoar. Consequently, a longitudinal enterotomy was performed to evacuate the phytobezoar measuring 4 cm by 3 cm and was closed in a transverse fashion in two layers (). The phytobezoar seemed to be composed of fibers, but material resembling skins and seeds were not identified. There was also no evidence that this obstruction was located at the suture line, and no other intraluminal pathology was noted that would have narrowed the lumen of the bowel. Final pathology revealed “vegetable matter and acellular debris.” On further questioning the patient, the patient endorsed a diet prior to hospitalization that was significant for large amounts of vegetables. However, because no finer detail was elicited, further conclusions about the content could not be drawn. The patient continued to progress well postoperatively with eventual return of bowel function and was discharged home without complications. The patient demonstrated good recovery from his surgery and was asymptomatic at his postoperative clinic visit and at 1-year follow-up. |
52 year old female presented to the department of otorhinolaryngology with complain of unilateral gradually progressive swelling over the left side of the nose below the eyes since 2 months. The swelling was painless to begin with, but patient gives history of pain over the swelling since 2 weeks.\nThere are no aggravating or relieving factors. No history of radiation of pain. No history of recent trauma. Patient also gives history of watering of left eye since 2 days. Not associated with nasal obstruction, nasal discharge. No history of visual disturbance. There was no history of significant weight loss or loss of appetite. No significant medical history (past history of tuberculosis, exposure to patients with tuberculosis), or family history.\nOn examination she was conscious cooperative and well oriented to time place and person. Vitals were stable.\nA swelling measuring 1.5 x 2 cm was noticed on the lateral aspect of the nasal bridge, inferior to the infraorbital ridge. Firm in consistency, irregular surface, ill-defined margins, non-tender, mobile in horizontal and vertical direction, no local rise of temperature, no skin changes and skin was pinch able.\nRest of the ear, nose, throat and neck examination were within normal limits. No palpable neck lymph nodes.\nOn investigation\nBlood parameters were within normal limits except for ESR, which was 76mm/hr.\nHIV, HbsAg and HCV were negative.\nFNAC was done from the lesion which showed multiple inflammatory cells- suggestive of chronic inflammation. A plain CT scan was done which showed- soft tissue density noted adjacent to anterior wall of left maxillary sinus. The lesion is not involving the anterior wall of sinus. ().\nChest x-ray PA view was normal\nPatient was planned for excision of the lesion under ga and was taken up for surgery after anaesthetic pre-op fitness. A cosmetically better approach was planned for this case i.e endoscopic sublabial approach to the swelling. Lesion was identified, it was a well encapsulated lesion present over the inferior orbital margin extending 1cm from the medial canthi laterally to the root of the nose. Lesion was removed in toto and sent for histopathology ().\nThe histopathology showed epithelioid cells with caseation suggestive of tubercular pathology ().\nLater the patient was subjected to Mantoux test and sputum for acid fast bacilli and was found negative for both test. Patient was started on category 1 antitubercular treatment. And patient has successfully completed 4 months with good compliance and no recurrence of swelling post-surgery. |
Following the appearance of a rapidly evolving jaundice, a 62-year-old Caucasian male was diagnosed with moderately differentiated cholangiocarcinoma localized at the level of the common biliary duct. This tumor was classified as cT3N0M0, or subtype I, according to the Bismuth classification. A surgical resection was performed with Whipple’s duodenocephalic pancreatectomy. Two years later, he presented with postprandial pain, and he had lost approximately 20 kg in 1 year. These symptoms were found to be associated with recurrence in the liver and lung. The patient began chemotherapy with cisplatin and gemcitabine (cisplatin at a dose of 30 mg/m2 and gemcitabine at 1,000 mg/m2 on days 1 and 8, respectively, every 3 weeks). Given the excellent therapeutic response after nine courses, the patient continued monotherapy with gemcitabine alone. Despite the stability of persistent lesions in the liver and lymph nodes, this medication was discontinued, due to the appearance of a discrete pulmonary infiltrate, which suggested gemcitabine toxicity. At the 5-month follow-up examination, the patient reported pain around his left shoulder without any other symptoms. A computed tomography (CT) scan showed an increase in the size of a lung nodule. F18 fluorodeoxyglucose (FDG) positron emission tomography (PET)/CT confirmed recurrence of the cholangiocarcinoma. Moreover, there were two other suspicious lesions: one was in the anterior portion of the vertebral body of L2 and the other was located intramedullary at the C4 level (Figure ). When the patient returned to learn the results, he reported that, within the last 36 h, he had developed a motor deficit in his left arm. The pain in the upper part of the left shoulder had also persisted. Therefore, magnetic resonance imaging (MRI) was performed, and the results confirmed an intramedullary metastasis at the C4 level with spinal cord compression (Figure ). The MRI results also revealed numerous parenchymal brain metastases (Figure ). The high number of brain metastases disqualified the patient for neurosurgery. In addition, the risk of operating on the spinal lesion was considered too high for either a complete resection of the lesion or a decompressive cranial laminectomy. The next day, radiotherapy of the whole brain and cervical spine to C5 was started, combined with corticosteroid therapy (64 mg methylprednisolone/day). A total dose of 30 Gy was administered in daily fractions of 3 Gy, dispensed with two lateral photon beams shaped with a multi-leaf collimator. After completion of the radiotherapy, systemic treatment with cisplatin and gemcitabine was reinitiated, due to the patient’s previous excellent response to this chemotherapy regimen. The clinical evolution of the symptoms rapidly became favorable in terms of managing pain and neurological deficit. By the middle of the radiotherapy treatment, the patient had completely recovered from the paresis in the left upper extremity. At the time of publication of the present study, he remained on chemotherapy without any signs of neurological or systemic progression. |
The patient was a 40-year-old woman, who had been diagnosed with a continuous heart murmur since junior high school. After being asymptomatic for many years, she became aware of angina on exertion for which she visited her family doctor. Chest X-ray revealed an enlarged heart and mild pulmonary congestion. Serology revealed a mild elevation of brain natriuretic peptide (BNP), at 77.7 pg/ml. The electrocardiogram was normal. No ischemic changes were noted on treadmill stress electrocardiogram. Transthoracic echocardiography showed normal left ventricular motion and no valvular disease. In addition, cardiac Doppler ultrasound showed continuous turbulent flow into the right atrium. Enhanced computed tomography (CT) showed a significantly dilated (13.2 mm) right coronary artery (RCA) at its origin, with a coronary artery fistulous tract draining into the posterior aspect of the right atrium. This fistula formed a large mass (21 × 29 mm) on the dorsal surface of the superior vena cava. The diameter of the RCA, distal to the fistula, was normal (). On right heart catheterization, intracardiac pressure data were within normal limits, but an O2 step up was observed in the right atrium, and Qp/Qs was 1.3. She was diagnosed with a right coronary aneurysm with a coronary artery fistula to the right atrium. Because of worsening of her symptoms and the presence of a large fistulous tract, the patient was considered a surgical candidate.\nThe operation was performed via median sternotomy. The RCA was markedly enlarged from its origin, and the fistula branched from the middle toward the dorsal side of the superior vena cava (SVC). The RCA distal to the fistula had a normal diameter (). After systemic heparinization, cardiopulmonary bypass (CPB) was established with ascending aortic cannulation, with venous drainage from bi-caval cannulation. The ascending aorta was cross-clamped and cardiac arrest was achieved with cardioplegic solution (CPS) infusion from the aortic root while compressing the coronary fistula. After snaring the SVC and inferior vena cava (IVC), the right atrium was opened. Inside the right atrium, a 9-mm tract opening draining dorsal to the SVC–right atrial junction was observed. The tract opening on the posterior wall of the right atrium was closed with 5-0 polypropylene continuous suture (). The dilated origin of the RCA was resected, and the stump was closed with 4-0 polypropylene mattress with continuous over-and-over suture to preserve the morphology of the sinus of Valsalva. (). The distal RCA was ligated at a normal diameter site and grafted to the right internal thoracic artery. The operative time was 230 min; the cardiopulmonary and cardiac arrest times were 118 and 58 min, respectively. Postoperative transthoracic echocardiography showed no residual shunt into the right atrium and no aortic regurgitation. Postoperative enhanced CT showed patency of right internal thoracic artery graft () and preserved morphology of the sinus of Valsalva (). The postoperative course was good, and the patient was discharged uneventfully on the 16th postoperative day. |
An 87-year-old Caucasian male was referred to our centre by a dermatologist, having undergone punch biopsy of a penile lesion with the initial histology reported as showing Bowen's disease. The patient gave a 6-month history of an enlarging lesion on the shaft of his penis prior to presentation to the dermatologist, which had been treated with topical agents and antibiotics. Nevertheless, the dermatologist was clinically suspicious of an invasive lesion prompting referral for wide excision. The patient had had a similar lesion at the same location 10 years earlier which was excised by his general practitioner but no histology report could be traced. He had no other lumps anywhere in the rest of the body and no family history of similar disease. His co-morbidities included ischaemic heart disease, Alzheimer's disease and venous ulcers.\nExamination revealed a 2.5 cm erythematous, fleshy, exophytic plaque at the base of the shaft of the penis (Figure ). There was a satellite lesion proximal to this. The patient had no palpable inguinal lymphadenopathy. A clinical suspicion of an invasive squamous cell carcinoma was made and the patient underwent a wide local excision of the penile and satellite lesions. Frozen-section examination was not performed. The scrotal skin was advanced and primary closure performed. The foreskin was retracted in order to achieve a tension-free closure.\nThe specimen measured 30 × 50 × 50 mm. Light microscopy showed intraepithelial proliferation of neoplastic; large, pale cells, located predominantly in the basal and parabasal layers of the epithelium (Figure ), with margins apparently clear. Immunohistochemical stains showed specific staining characteristics with strong positivity for epithelial membrane antigen (EMA), the cytokeratin (CK) CK7, CAM 5.6 and HER2 protein over expression. CK20 staining was negative. These immunohistochemical appearances supported the histological diagnosis of EMPD (Figure ). Immunohistochemical staining also revealed that there were occasional cells in proximity to the margins.\nThis patient's histology was discussed at our weekly multi-disciplinary cancer meeting and the consensus was not to screen for an underlying non-cutaneous malignancy in view of the patient's age and co-morbidities. Furthermore, a decision was made not to attempt wider excision. At 6-months follow-up, our patient had no local recurrence or palpable inguinal lymph nodes. |
A 59-year-old woman was referred to our department due to an abnormal shadow on chest computed tomography (CT). She had undergone right lower lid resection for a tumor of the eyelid 4 years earlier. The size of the lesion had been 11 mm, and HE staining showed that the tumor cells with atypical nuclei and foamy cytoplasm had expanded to form a net-like structure. The pathologic diagnosis was SGC (Fig. a). Two years after the first operation, she suffered from local relapse of SGC and underwent right lower lid resection for the tumor of the eyelid and radiotherapy with a total dose of 60 Gy of proton beam therapy after the operation. Four years after the first operation, she suffered from a recurrent subcutaneous tumor of the right lower eyelid and underwent orbital exenteration of the right eye. At the same time, chest CT revealed bilateral small pulmonary nodules located in segment (S) 6 and S10 of the left lung and S2, S4, and S10 of the right lung (Fig. ). A preoperative pulmonary function test revealed normal results (FEV1.0, 2600 ml; %VC, 123.6%).\nBecause the lung nodules were small, transbronchial or CT-guided biopsy was not indicated. We therefore decided to perform surgical biopsy. At the same time, we planned staged radical surgery for all nodules in both lungs in the event that the final diagnosis was pulmonary metastasis. She underwent two wide wedge resections of the left lower lobe (S6, S10). The tumor size of S6 was 8 mm × 4 mm, while that of S10 was 5 mm × 4 mm, and the surgical margin was negative. HE staining showed that pathological findings are similar to primary tumor (Fig. b). An immunohistochemical analysis revealed that the tumor expressed epithelial membrane antigen (EMA), androgen receptor (AR), and p53 and not expressed anti-epithelial antigen (BerEP-4) (Fig. ). The primary tumor also showed the same pattern of immunohistochemistry. The disease was therefore diagnosed as pulmonary metastasis from SGC of the eyelid.\nAfter the operation, 18F-fluorodeoxyglucose positron emission tomography/CT (FDG-PET/CT) was performed, which showed no metastasis other than that at the right lung. The remaining small nodules in the right lung did not show any uptake of FDG. Six months after the first pulmonary resection, CT showed that the nodules of right S2 and S10 had increased in size, and three small nodules had newly appeared at S3, S6, and S8 of the right lung. The patient therefore underwent six wide wedge resections of the right lung (S2, 3, 4, 6, 8, 10) through thoracotomy. The pathologic diagnosis was pulmonary metastasis from SGC of the eyelid.\nEighteen months after the first pulmonary resection, CT revealed a new nodule 7 mm in diameter in the left S1+2. She underwent wide wedge resection of the left upper lobe through thoracotomy. Forty-nine months after the first pulmonary resection, she underwent wide wedge resection of right lung to again treat recurrent pulmonary metastasis. The postoperative pulmonary function remained almost unchanged from the preoperative pulmonary function (FEV1.0, 2520 ml; %VC, 110.3%). Ninety months after the first pulmonary resection, the patient is doing well without disease. |
A 69-year-old Japanese woman was referred to our department in August 2015, complaining of swelling in the right preauricular region. She had shown a mass lesion of the right lung on a chest x-ray and was referred to the university hospital. Fluorodeoxyglucose-positron emission tomography (FDG-PET) examination showed accumulation indicative of a pulmonary lesion and a temporomandibular joint lesion. The temporomandibular joint lesion had been enlarging but was otherwise asymptomatic. Her medical history included treatment for pulmonary tuberculosis at the age of 13 and surgery to remove breast cancer at the age of 53.\nOn examination, a tender mass in the right preauricular region was palpable. The chin of the mandible was deviated to the right side during mouth opening (mandibular maximum mouth opening, 41 mm). Facial nerve function and mandibular nerve were intact. There was no indication of cervical lymphadenopathy.\nA panoramic radiographic examination showed resorption of the right mandibular condyle to the ramus (Fig. ). Computed tomography (CT) showed destruction of the right mandibular condyle and a large mass lesion with enhanced margin in the masticator space; a cystic lesion was present inside the tumor mass. Three-dimensional CT was useful to understand the bone resorption findings of the mandibular condyle. There was no finding of metastatic cervical lymph node (Fig. ). T1-weighted magnetic resonance imaging showed an enhanced mass lesion in the right masticator space. Because some portions of the mass lesion showed high intensity in T2-weighted images, cystic lesions were suspected to exhibit changes of blood flow or retention of high-protein liquid. Tumor development was not observed in the articular disk of the temporomandibular joint (Fig. ). FDG-PET revealed abnormal FDG uptake in the right submandibular condyle and masticator space.\nA malignant tumor was suspected after analysis by various modalities. Therefore, we performed an incisional biopsy via preauricular incision. Histopathologically, the tumor was largely composed of proliferative, atypical, spindle-shaped cells. Some tumor cells showed increasing mitotic change and extreme atypia (Fig. ). The histopathological findings of biopsy suggested spindle-cell sarcoma. Tumor resection was performed with the patient under general anesthesia via a combined preauricular and transmandibular approach to the masticator space and infratemporal fossa.\nAfter preauricular temporomandibular incision, the superficial temporal fascia and temporal fascia were elevated. The facial nerve (temporal and zygomatic branches) was protected by the fascia (Fig. a).\nThe facial nerve trunk was identified, and its branch was traced according to the conventional method.\nA midline lip-splitting incision was connected to the submandibular incision and accessed the anterior mandibular ramus. The lip-split incision traversed to the periosteum of the mandible; the periosteum dissector was used to elevate the soft tissues of the mandible, and the lateral mandible was exposed. The branch of the facial nerve was traced continuously with the skin flap, and the masseter muscle and deep parotid gland were dissected on the tumor side (Fig. c). The mandible was resected to within ≥ 20 mm from the primary tumor on the anterior of the mandibular ramus. The masseter muscle was divided at the lower edge of the zygomatic arch; further, the temporal muscle was divided horizontally at the height of the zygomatic arch to reach the side of the temporal bone. Excision was performed on the temporal bone to the base of the pterygoid process, and an osteotomy was performed continuously from the maxilla to the pterygoid process. Osteotomy was performed via ultrasonic scalpel. Then, deep excision was performed on the skull base until the foramen ovale was reached. The mandibular branch (VIII) of the trigeminal nerve was resected at the foramen ovale, and bone wax was used to fill the foramen ovale when hemostasis was achieved. The tumor was excised in bulk with the surrounding tissue (Fig. d). Especially, the glenoid fossa was close to the primary tumor site; however, it was excised including the joint disc, and the glenoid and infratemporal fossa were excised, including the periosteum of the skull base. For that reason, the margin was considered complete, and we did not perform the histopathological evaluation intraoperatively.\nThe surgical defect was reconstructed with a free vascularized fibula with skin paddle (Fig. e). The occlusion was performed with intermaxillary wire fixation for 1 week postoperatively. There were no abnormal findings during the postoperative course with complete healing. Right-sided facial nerve dysfunction appeared immediately after surgery.\nThe resected specimen exhibited nearly identical histological findings as observed in the biopsy. Because it involved a periosteal reaction corresponding partially to Codman’s triangle, the tumor was thought to have derived from bone (Fig. ). Immunohistochemical analysis showed positive staining for vimentin, MIB-1 index (40–80%), desmin, α-smooth muscle actin, Bcl-2, neuron-specific enolase, and S-100; it showed negative staining for AE1/AE3, caldesmon, and CD34. Thus, the final diagnosis was osteosarcoma (fibroblastic). The resection margin was negative for tumor. After excision of the mandibular tumor, excision of lung cancer was performed under thoracoscopy by a respiratory surgeon in our hospital. Although we recommended adjuvant treatment after surgery, the patient refused this treatment.\nThere has been no evidence of local recurrence or distant metastasis through 30 months of follow-up. The chin of the mandible deviates to the right side during opening (maximum mouth opening, 40 mm). Centric occlusion has not changed. Facial nerve dysfunction gradually improved and became mild according to House-Brackmann Scale evaluation (Figs. and ). |
A 5-year-old male child; the only child of his anxious parents, presented with right lower abdominal pain for 5 days, low-grade fever for 3 days and 2 episodes of nonbilious vomiting. Pain was of moderate intensity and localized to right iliac fossa without any shifting, radiation or referral. Fever was low-grade 99°F to 100°F without chills and rigors. He had 2 episodes of nonbilious vomiting immediately after forced feeding by mother. His bowel and bladder habits were normal. There was no past history of similar attacks. He had not undergone any surgical intervention before nor had any contact history with tuberculosis, drug intake or significant medical or surgical disorder. He was taking oral antibiotics and analgesics from their family physician.\nOn examination, the baby had fever of 100°F and tachycardia. Abdominal examination revealed tenderness with guarding in right iliac fossa.\nBlood investigations revealed leukocytosis (12,000/cc) with neutrophilia (85%). Rest of blood and urine investigations was normal. His X-ray abdomen was also normal. However, ultrasonography of abdomen was reported as acute appendicitis with inflammatory mass formation measuring 6.1 cm × 1.4 cm and minimal collection at right iliac fossa along with mesenteric lymphadenopathies. The appendix was not visualized sonologically. It was the mass formation that was interpreted as appendicular lump due to its position.\nWith the provisional diagnosis of appendicular lump, the baby was managed conservatively with intravenous antibiotics, intravenous fluids and bowel rest for 1 week. However, due to the persistence of pain in spite of the conservative management laparoscopic appendectomy under general anesthesia was planned after 1 week.\nOn laparoscopy omentum was seen in right iliac fossa. After retraction of the omentum from right iliac fossa, the cecum and ileum were visualized to be normal. There were small mesenteric lymph nodes, but the appendix could not be visualized even after mobilization of the ileocecal junction. Failing to visualize the appendix by laparoscopy, it was decided to convert to open appendectomy. Ileocecal junction then approached via right iliac fossa transverse skin crease incision. The point of convergence of the three teniae coli was delivered to the wound [], but the vermiform appendix could not be detected. The retrocecal space and ileocecal area were thoroughly explored to conform the absence of appendix []. A careful search for Meckel's diverticulum was also unproductive. The paracecal mesenteric lymph nodes were excised and sent for histopathology study which later revealed reactive hyperplasia.\nAbdomen was closed after discussion with the parents. The baby improved symptomatically and was discharged after 1 week. There was no recurrence of pain on follow-up. The cause of his condition was put to nonspecific mesenteric lymphadenitis. |
This case describes a 65-year-old Caucasian man who presented to our clinic with a six-year history of severe chronic lumbago with neurogenic claudication leading to the inability to walk more than a few steps. His past medical history included anxiety, atrial fibrillation after past ablation, coronary artery disease after percutaneous coronary angioplasty with three stents, gastroesophageal reflux disease, hypertension, obstructive sleep apnea, and hyperlipidemia. His pain was mainly axial and involved the lumbar spine with radiation to bilateral anterolateral thighs. He endorsed a history of over 100 falls during his years as an ice skater but described the onset of his pain as gradual. The patient had no history of any of the reported risk factors of OLF in literature, like nephrolithiasis, hypercalcemia, or kidney disease and his laboratory values for serum calcium, creatinine, and glomerular filtration rate were within normal limits. Prior interventions included physical therapy, acetaminophen, nonsteroidal anti-inflammatory drugs, pregabalin, and multiple caudal epidural steroid injections over the years with only marginal benefit. MRI revealed the etiology of his lumbar radiculopathy to be severe lumbar spinal stenosis.\nOn examination, the patient was sitting. He could only stand for a few seconds upright before leaning forward and taking just a few steps before he had to sit due to cramping in both legs attributed to neurogenic claudication. He exhibited 5/5 strength in his bilateral lower extremities with no signs of muscle wasting or atrophy. His deep tendon reflexes were 2/4 in the bilateral lower extremities. The facet loading test was positive bilaterally in the lumbar region. MRI of the spine revealed central canal narrowing and bilateral neuroforaminal narrowing from L3-5. At L4-5, there was a broad-based posterior disc bulge with annular fissuring of the nucleus pulposis and mild facet arthropathy. In retrospect, ligamentum flavum ossification was suggested with T2- and T1-weighted images (Figures -).\nThe patient underwent endoscopic intralaminar laminotomy with partial facetectomy and lateral recess decompression with immediate resolution of his symptoms. Intraoperatively, we identified OLF at the L4-5 that had not previously been noted on MRI.\nThe patient underwent uneventful induction of general anesthesia and neuromonitoring was performed via the quadriceps, anterior tibial, gastrocnemius, and hamstring on the right side. A true anteroposterior view of the lumbar spine at the L4-5 level was obtained by using fluoroscopy. A working channel was established and the spinal endoscope was introduced. The descending lamina was identified in the right L4-5 interlaminar window and a laminotomy was created over the right-sided descending lamina down to the descending facet. Once at the ligamentum flavum, the dorsal fibers were taken down, revealing ventral fibers of the ligamentum flavum that were unexpectedly found to be hardened and ossified across the interlaminar window at L4-5 (Figure ). The hardened ossified shell of the ligamentum flavum was carefully dissected. The dura was fully decompressed and freely mobile, without signs of ossification. Once adequate hemostasis was visualized, 40 mg of methylprednisolone was injected at the site and the incision was closed.\nThe patient reported immediate improvement of his radicular symptoms with no neurological deficits in the postanesthesia care unit. The patient presented to the clinic for a follow-up on postoperative day 9 with sustained improvement and was neurologically intact. |
A 75-year-old, 155 cm, 53 kg female patient presented for off-pump coronary artery bypass graft surgery. As a preoperative test, a coronary angiography was performed. The results revealed 86% stenosis of the left anterior descending artery, 83% stenosis of the left circumflex artery and 91% stenosis of the right coronary artery. The aortography revealed that only the right common carotid artery was aberrant in the aortic arch. The branching of the right subclavian artery could not be checked (), but delayed imaging of the right subclavian artery was observed (). ECG and other tests did not reveal other abnormal findings and there were no symptoms of dysphagia or respiratory difficulty.\nThe patient was pre-operatively administered 6 mg of morphine by muscular injection, was after which she was moved to the operating room. Five electrocardiogram leads were placed, and leads II and V5 were continuously monitored. The pulses of both radial arteries were palpated well and there was no difference in blood pressure between the two upper extremities. Because the left radial artery was going to be used as the graft vessel, a 20-gauge catheter was inserted into the right radial artery, and continuous monitoring of the arterial pressure was initiated. A pulmonary artery catheter was inserted into the right internal jugular vein to monitor cardiac performance parameters continuously.\nTo induce anesthesia, 50 µg sufentanil, 2.5 mg midazolam, and 10 mg vecuronium were administered and endotracheal intubation was performed. Anesthesia was then maintained using a continuous sufentanil infusion (0.5-1.5 µg/kg/hr) and supplemental sevoflurane (0.6-2.0 vol%). Next, a 20-gauge catheter was inserted into the right femoral artery to enable accurate blood pressure monitoring. The radial artery pressure was monitored along with the continuous monitoring of the femoral artery pressure. Hemodynamic conditions following the induction of anesthesia were stable, and a 14 × 12.5 mm TEE probe (6T probe, GE Vingmed Ultrasound, Horten, Norway) was inserted into the patient's esophagus. Immediately after insertion of the TEE probe, the arterial pressure waveforms of the right radial artery decreased significantly, whereas the right femoral artery pressure remained stable. The TEE probe was slowly removed from the patient's esophagus, after which the right radial waveform reappeared 28 cm from the upper incisor. After slowly advancing and retracting the probe twice, we observed the dampening of arterial pressure reoccur (). Based on these findings, it was suspected that the TEE probe caused compression of the right subclavian artery; therefore, the TEE probe was removed. Diluted blood was observed on the tip of the TEE probe after it was removed, indicating that the esophagus may have been damaged by the probe. Accordingly, a physician immediately conducted an esophageal endoscopy, which revealed an esophageal stenosis following pulsation on the posterior esophageal wall. With the use of the scope, we found the location of the stenosis to be 28 cm from the upper incisor, but no damage to the esophageal wall was observed. A 9.0 × 9.0 mm gastroscope (GIF-XQ260, Olympus Medical System Co., Tokyo, Japan) was used for esophageal endoscopy and easily passed through the area of the stenosis. No compression of the right subclavian artery was observed; therefore, we inserted a 10.9 × 8.4 mm pediatric TEE probe (9T probe, GE Vingmed Ultrasound, Horten, Norway) and proceeded with the surgery. No unusual conditions were observed during or after surgery. |
We present a case of a 14 year old boy with diagnoses of bipolar affective disorder, current episode mixed (ICD-10 F31.6), childhood autism (ICD-10 F84.0) and moderate mental retardation (ICD-10 F71). He had an 18-month history of gradual deterioration in his behaviour which on admission included aggression, periods of excitement interspersed with crying and head-banging, reduced need for sleep, and overactivity and was subsequently admitted to our inpatient unit. He was observed to present with disinhibition and at times displayed inappropriate behaviour towards fellow female inpatients and staff members.\nHis drug history includes a trial of risperidone, up to 2 mg per day which was partially successful but was associated with akathisia; a three week trial of quetiapine up to 200 mg daily was clinically ineffective and had to be prematurely discontinued due to clinical deterioration. Finally, brand-name olanzapine was added and titrated to 7.5 mg daily. In addition sodium valproate was titrated to 800 mg twice daily. This combination yielded the best improvement, in terms of mental state and functioning both at home and on the inpatient unit. At that point, after a 3-week period of sustained improvement, due to changes in our hospital budgeting pharmacy policy, generic olanzapine was given to the boy for the first time instead of the brand-preparation. This change was not apparent to the patient due to his neurodevelopmental problems. A noticeable deterioration in his mental state was observed within 2 days following the change to generic olanzapine. No other changes in medications were made. Symptoms that re-emerged included increased agitation, aggression, reduced sleep and disinhibition. This was not associated with any physical health problems, change in other medication or environmental factors. The generic olanzapine was increased to 10 mg daily with no positive effect on his mental state. Following this, the change from brand-name to generic olanzapine was hypothesised to account for the boy’s mental state deterioration. The brand-name olanzapine was restarted at 10 mg daily, with improvement in his mental state within 1 – 2 days. |
A 31-year-old Asian male visited the Department of Oral and Maxillofacial Surgery for evaluation of restricted mandibular opening. Maximal effort produced 20 mm of vertical interincisal opening, which could not be increased by external force. The extent of right and left excursions and protrusion was 4 mm. The patient’s main complaints was limited mouth opening, and he did not report pain. The limitation of mouth opening started when the patient was a middle school student, but he could not remember the exact time. The amount of mouth opening continued to decrease over time. Initially, he could eat anything, but now he cannot eat large volume food due to trismus. He denied a familial history of trismus and previous trauma to head and neck area. The patient was otherwise healthy and past medical history was non-specific. Laboratory findings were normal. There was no temporomandibular joint (TMJ) pain or tenderness and no muscular tenderness or spasm.\nPanoramic X-ray showed elongation of right and left coronoid process. On 3-dimensional computed tomography (3D CT) scan, the enlarged coronoid process overlay the zygomatic process and blocked mouth opening when the patient opened his mouth to approximately 20 mm (, ). Radiographs of the TMJ showed normal findings. A diagnosis of bilateral coronoid hyperplasia was confirmed from clinical and radiographic examinations.\nThe patient was admitted to the Department of Oral and Maxillofacial Surgery after one week and surgery was performed. Anesthesia was induced after a blind nasal endotracheal intubation. Because mouth opening was not improved after administration of a muscle relaxant (Rocuronium), trismus did not originate from a muscular problem. A full thickness mucoperiosteal incision was made along the external oblique ridge from premolar to the highest point on the anterior aspect of ramus of the mandible on both sides. Mucoperiosteum was reflected to expose the medial and lateral aspect of the ramus and coronoid process. The temporalis muscle insertion was detached from anterior portion of the ramus and coronoid process. A channel retractor was placed into the sigmoid notch to protect lateral soft tissue.\nA Seldin (Walter Lorenz Surgical Instrument; Biomet, Warsaw, IN, USA) retractor was used to protect medial soft tissues while sufficiently exposing coronoid process. The osteotomy cut was conducted from sigmoid notch to anterior aspect of the ascending ramus with straight saw. A bone Kocher clamp (Walter Lorenz Surgical Instruments) was used to remove coronoid process after removal of the remaining attachments of the temporalis muscle (). Immediately after coronoidectomy, the maximum mouth opening was 41 mm and smooth excursive movement was observed ().\nThe flap was closed with 3-O Vicryl acid suture. Post-operatively, the patient was advised to exercise the mouth opening with tongue blades. For the first eight weeks, mouth opening was slightly decreased to less than 30 mm, but thereafter, the maximum mouth opening was maintained at about 40 mm. At the one year exam, lateral movement and protrusion were symmetric and within normal range. The patient was able to open his mouth to 41 mm (). On post-operative panoramic X-ray and 3D CT, mechanical blocking by elongated coronoid process disappeared (). |
A 48-year-old African-American man with no significant tobacco history presented with a slow-growing mass in his neck and 11 kg weight loss over a 9-month period (Fig. ). On further history, he reported left arm pain and weakness, intermittent fever with chills, and had denied any dyspnea, shortness of breath, or cough. His past medical history was significant for diabetes mellitus, chronic obstructive pulmonary disease, and nerve impingement of his left shoulder. On physical examination, a mass was noted on the left side of his neck just superior to the sternoclavicular joint measuring 3 × 3 × 1 cm. He had ptosis and miosis of his left eye. His speech was hoarse, indicative of recurrent laryngeal nerve involvement. His breath sounds were decreased and coarse at the left apex. The strength of his extremities exhibited 3/5 weakness in his left arm, with the remainder of his limbs normal. There was positive muscle atrophy of his left upper extremity. Otherwise, the remainder of the physical examination was benign in nature.\nAn ultrasound performed on the neck mass showed an ill-defined heterogeneous mass lateral to his left thyroid lobe, resulting in mild mass effect. Further investigation was warranted with radiological studies including chest X-rays and a computed tomography (CT) scan of his chest. A chest X-ray exhibited a mass extending from the hilum of his left lung with gross deviation of his trachea and elevation of his hemidiaphragm. A CT scan of his chest showed a large multiloculated pleural-based mass in his lungs surrounding his left common carotid artery, left subclavian artery, which interfaced broadly with the aortic arch and extended into the left neuroforamina of C4/C5 through T1/T2 (Fig. ).\nPercutaneous needle core biopsy was performed on his neck and showed fibrohistiocytic tissue with mild chronic inflammation but was negative for malignancy. A left thoracotomy with biopsy was performed of the pleura and of the upper and middle lung lobes of his left lung evidencing a mass extending from the anterior mediastinum medially at the level of his pulmonary artery trunk superiorly into the superior sulcus and posteriorly into his chest wall. Tissue samples were obtained, all demonstrating a gross appearance of a pink to yellow gelatinous tissue for which frozen sectioning revealed soft tissue malignancy. Hematoxylin and eosin staining demonstrated a predominance of hyperchromatic spindle-shaped cells with acidophil cytoplasm (Fig. ). This is consistent with high-grade sarcomas [] typically with disorganized pleomorphic cells that can also include histiocytes and giant multinucleated cells []. A panel of immunohistochemical stains was performed of which only Wilms tumor protein (WT1), vimentin, and Ki67 were positive. This finding ruled out carcinoma, mesothelioma, melanoma, vascular tumor, synovial sarcoma, and Kaposi’s sarcoma as possible etiologies. Overall, immunohistochemistry revealed a high-grade undifferentiated myxoid sarcoma confirmed by the Mayo Clinic as fibromyxosarcoma versus MFS. |
A 66-year-old woman with a significant past medical history of well-controlled hypertension was admitted with complaints of microscopic hematuria and mild proteinuria for the past 3 years. Serum creatinine level was within normal range at that time and therefore the anti-GBM antibody was not tested. The first renal biopsy revealed mesangial proliferative glomerulonephritis with fibro-cellular crescents in one out of 18 glomeruli, excluding one global sclerotic glomerulus (Fig. ), and deposition of IgA and C3 in mesangial areas by immunofluorescence microscopy (Fig. ). Weak but significant IgG deposition was also observed in glomeruli in the distribution somewhat different from IgA or C3 (Fig. ). The electron-dense deposits were observed in mesangial areas by electron microscopy. Therefore, the diagnosis was IgA nephropathy. Antihypertensive therapy was initiated, mainly with an RAS inhibitor. Eight months later, the patient’s serum creatinine suddenly rose to 4.53 mg/dL (it was 1.04 mg/dL from the routine blood test 1 month before). Urinalysis showed 100 red blood cells per high power field and urinary protein excretion of 12.3 g/gCr (Fig. ). The serological tests that were performed to differentiate the cause of rapidly progressive glomerulonephritis revealed the presence of anti-GBM antibody at the titer of 116 IU/mL and the absence of anti-nuclear antibody and anti-neutrophil cytoplasmic antibody. Laboratory findings on admission are summarized in the Table .\nAfter admission, treatments with hemodialysis, plasma exchange, and intravenous methylprednisolone pulse therapy followed by oral prednisolone at the dose of 50 mg/day were initiated. The second renal biopsy was performed at 4 weeks after admission in order to assess the probability of renal recovery and to make the final diagnosis. It revealed cellular to fibrocellular crescents in 18 of 25 glomeruli, excluding six global sclerotic glomeruli by light microscopy. By immunofluorescence study, linear IgG deposition along the glomerular capillary walls and mesangial staining for IgA were observed. On the other hand, C3 deposition was observed in the mesangium as well as in the glomerular capillary walls (Fig. ). Electron-dense deposits were observed in mesangial areas, similarly as in the first biopsy, by electron microscopy (Fig. ). Based on the aforementioned findings, the diagnosis of anti-GBM glomerulonephritis and IgA nephropathy was confirmed. Plasmapheresis was performed eight times, anti-GBM antibody gradually decreased, and alveolar hemorrhage was prevented. However, her renal function could not be restored and she underwent maintenance hemodialysis (Fig. ).\nAdditional immunosuppressant was not given because the patient did not show any sign of pulmonary involvement and because the renal recovery was quite unlikely from clinical (continuous oliguria and hemodialysis dependence) as well as histological (crescent formation in most of non-sclerotic glomeruli) point of view.\nClinical and histological presentations from IgA nephropathy (at the time of first renal biopsy) and from anti-GBM disease (at the time of second renal biopsy) were summarized in the Table . |
A 38-year-old male patient, businessman by profession, presented to the outpatient department with a chief complaint of diffuse and continuous pain in the left ankle and difficulty in walking since the past 6 months. The pain was more on lateral aspect of ankle and more in the night time. There was no history of trauma, fever, loss of weight, and loss of appetite. He was taking tablet aspirin orally as when required, which helped him to get relief from pain. On examination, there was local tenderness and mild swelling at the anterolateral aspect of ankle. Ankle movement was full with dorsiflexion terminally painful. On radiological examination, X-ray showed osteosclerotic lesion around a central lucent area suspicious of OO with nidus in the medial aspect of distal fibula at the level of ankle joint []. CT scan showed the nidus with surrounding reactive sclerosis in the left distal fibula []. Three phase bone scan and SPECT-CT using Technetium-99m (Tc-99m) methylene diphosphonate were also done. Three phase bone scan showed increased tracer accumulation in the left ankle joint []. However, the increased tracer uptake was diffuse heterogeneous in pattern and was not specific to OO. Therefore, SPECT-CT was performed, which localizes most focal uptake to a sclerotic lesion with central nidus in the left distal fibula anteromedially [Figures and ]. The lower grade diffuse uptake localized to the ankle joint, likely due to articular changes because of pain. This confirmed the diagnosis of OO in the left distal fibula.\nWe decided to perform an open procedure using anterolateral approach to excise the lesion along with the reactive bone, sparing the syndesmosis, and articular cartilage. The patient was placed in supine position with a sandbag under the affected side buttock under regional anesthesia with pneumatic tourniquet at the thigh. A longitudinal incision of 5 cm was given at the anterior border of fibula the ankle joint, and distal fibula was exposed. The nidus location was identified by reactive cortical bone area of about 0.5 cm × 0.5 cm and further confirmed by fluoroscopy. The lesion was excised and sent for histopathology study. Adequate care was taken to prevent injury to syndesmosis joint and articular cartilage of fibula.\nPostoperatively, the patient was comfortable, and the earlier specific type of continuous pain was disappeared except for surgical site pain which was reducing gradually. The patient was advised for full weight bearing mobilization. At 2 weeks, stitches were removed, and at that time the patient was absolutely pain free. Postoperative X-ray showed complete excision the lesion. Histopathological examination confirmed the diagnosis of OO of distal fibula. The patient was followed up at 1, 3, and 6 months’ postsurgery and there was no evidence of any recurrence. |
62-year-old woman with medical history significant for aortic stenosis and chronic atrial fibrillation presented to the emergency department with fatigue and progressively worsening shortness of breath with minimal exertion. She was asymptomatic at rest and denied chest pain, orthopnea, paroxysmal nocturnal dyspnea, leg swelling, presyncope, or syncope. She was incidentally found to have a systolic murmur during her pregnancy 20 years prior to this presentation. Notably, she had been offered aortic valve replacement in the past but declined.\nVital signs were normal with blood pressure of 110/60 mmHg, pulse rate of 79/min, temperature of 97.2°F, and respiratory rate of 16 breaths/min with normal oxygen saturation of 100% on ambient air. Physical examination was significant for irregularly irregular heart rhythm, and ejection systolic murmur was loudest in the aortic area with radiation to the carotids. Her lungs were clear to auscultation, and no pedal edema was noted. Electrocardiogram revealed atrial fibrillation with voltage criteria for left ventricular hypertrophy. Her most recent transthoracic echocardiogram revealed a thickened calcified aortic valve with decreased excursion with 4.6 m/s velocity suggesting a peak of 86 mmHg and mean of 36 mmHg suggestive of severe aortic stenosis. No other significant valvular abnormalities noted. Cardiac catheterization revealed widely patent coronary arteries.\nBased on the presence of worsening symptoms and the risk of sudden cardiac death, the decision was made to proceed with surgical aortic valve replacement. Given her history of chronic atrial fibrillation, she was also planned for left atrial appendage exclusion and Cox Maze IV procedure simultaneously with the aortic valve replacement. Intraoperative transesophageal echocardiogram revealed quadricuspid aortic valves confirmed during surgical exploration (). The native stenotic quadricuspid aortic valve leaflets were excised, and a 21 mm Saint Jude Medical Trifecta valve was implanted. The left atrial appendage was excised, and Cox Maze IV procedure was performed. Postoperatively, she remained in junctional rhythm and underwent uneventful placement of the dual chamber pacemaker on postoperative day 3. She recovered without further complications and was discharged on the eight postoperative day. |
A four-year-old girl presented to the emergency service with painful left hip and fever. There was no previous relevant medical history. There were no other local or systemic symptoms, except for a cervical adenopathy. On physical examination, she walked with a limp, and movements of the left hip were painful (mainly external rotation), but not restricted. Blood exam revealed anemia (Hb 8.7 gr/dL), normal WBC, ESB of 123 mm, and reactive C protein of 149.7 mg/L. An initial X-ray to the pelvis revealed no changes. An ultrasound of the left hip was performed revealing small infusion and synovitis. Guided puncture was then performed being macroscopically compatible with reactive arthritis, and general and bacteriological tests were demanded. Because of the unusual characteristics of the pain, a CT scan to the abdomen and pelvis was performed revealing a left adrenal mass and retroperitoneal adenopathies in the celiac trunk and superior mesenteric artery ().\nDespite the painful complaints of the patient, no bone or articular involvement was found in the CT scan. No further alterations were reported in the thoracic CT scan or in peripheral blood smears. Bacteriological examination of the hip effusion was negative. MRI was also performed. The direct myelogram was compatible with infiltration from neuroblastoma. Bone marrow biopsy and cervical adenopathy specimens were collected to perform histological diagnosis. Skeletal scintigraphy demonstrated numerous points of osteoblastic activity compatible with metastatic activity, and the 12 iodine-123 metaiodobenzylguanidine scintigraphy concluded the following: “Abdominal mass with low expression of noradrenergic transporters. Diffuse bone metastasization with high expression of noradrenergic transporters. No other soft tissue involvement was detected.” In the histological report of the cervical adenopathy, the diagnosis of neuroblastoma NOS was performed. Immunohistochemistry revealed extensive expression for synaptophysin and CD56 (NCAM) and absence of expression of myogenin. ().\nBone marrow biopsy revealed extensive metastatic involvement. The patient started chemotherapy two weeks after admission, with 8 cycles of rapid COJEC protocol. After six months of follow-up, the primary tumor was still without criteria for resection, despite a decrease in the metastatic involvement. Given the chemotherapy-related renal toxicity, it was decided to proceed with irinotecan in combination with temozolomide (TEMIRI). After thirteen months of follow-up, no significant regression of the primary tumor occurred, so surgery was contraindicated and the patient was proposed for stem cell treatment. |
A 64-year-old man who had no symptoms was diagnosed with thoracic superficial esophageal cancer that was detected by screening upper endoscopy. He had a history of hypertension. He had also been found to have a vascular abnormality (DAA) as an adult and was observed in an asymptomatic state.\nPhysical examinations showed no unusual findings, and the laboratory examination data, including tumor markers, such as squamous cell carcinoma-related antigen and carcinoembryonic antigen, were all within normal ranges. Chest X-ray demonstrated a widening in the upper mediastinal silhouette, reflecting the superior right aortic arch. An endoscopic examination revealed superficial esophageal cancer located in the left side of the wall in the upper thoracic esophagus and the invasion of the submucosa (Fig. ). A histological examination of biopsy specimens confirmed the presence of squamous cell carcinoma. Enhanced computed tomography showed a swollen lymph node in the right upper mediastinum, which was diagnosed as metastatic (Fig. ). No distant metastasis was detected. Computed tomography also confirmed the DAA. The right aortic arch was dominant, and the descending aorta was located at the right side of the post-mediastinum, as is common in cases of DAA (Fig. ). The patient was therefore diagnosed with upper thoracic esophageal cancer of cT1bN1M0 Stage IIB (UICC-TNM 7th) and a DAA.\nHe underwent neoadjuvant chemotherapy prior to sub-total esophagectomy with three-field lymphadenectomy. The neoadjuvant chemotherapy regimen was 2 courses of 5-FU (800 mg/m2) and cisplatin (80 mg/m2) every 3 weeks.\nWe planned to perform radical subtotal esophagectomy with three-field lymph node dissection after neoadjuvant chemotherapy. We first planned to perform cervical procedure in a supine position before the thoracic procedure in order to identify the bilateral inferior laryngeal nerves and avoid causing them injury or inducing palsy. We also planned to perform upper mediastinal lymph node dissection during this preceding procedure because the DAA was expected to interfere with upper mediastinal dissection attempted via either side of a transthoracic approach. We then planned to perform lymph node dissection via a left-thoracoscopic approach below the left aortic arch, as we worried that the right-sided descending aorta might interfere with a right-thoracic approach (Fig. ). The laparoscopic procedure was planned to be performed via an abdominal procedure in a supine position. Reconstruction would use the gastric tube pulled up via the retrosternal route with cervical esophago-gastric anastomosis.\nIn the preceding cervical procedure performed in a supine position, we identified the bilateral inferior laryngeal nerves, which were thought to be recurrent at each side of the aortic arch (Fig. ). After upper mediastinal dissection was performed, the left thoracoscopic procedure in a prone position was performed for middle and lower mediastinal lymph node dissection below the left aortic arch. We first confirmed that the right-sided aortic arch and descending aorta would interfere with the usual right thoracic approach (Fig. a). Upper mediastinum dissection was also deemed impossible via a bilateral thoracic approach because of the bilateral aortic arches and subclavian arteries, as expected preoperatively (Fig. a, b). Postmediastinal reconstruction also seemed impossible. The port position for the left thoracoscopic procedure was set symmetrically to our normal right thoracoscopic procedure for middle to lower mediastinal dissection, as shown in Fig. . No major anatomical findings other than those noted preoperatively were observed during the left thoracoscopic procedure. We were unable to identify where the thoracic duct ascended because of the preservation of the thoracic duct. We were also unable to confirm the details concerning both recurrent laryngeal nerves around each aortic arch.\nThe abdominal procedure in a supine position was performed laparoscopically with the simultaneous cervical procedure for bilateral supraclavicular lymph node dissection. Reconstruction was performed with cervical esophago-gastric tube anastomosis. The gastric tube was pulled up through a retrosternal route as planned. Three-field lymph node dissection and complete resection (R0) were achieved. The operative time was 8 h 9 min, and the total bleeding was 70 ml. No vocal cord palsy was observed on flexible laryngoscopy after the operation.\nThe patient’s postoperative course included minor leakage that was cured conservatively after 2 weeks, and he was discharged at postoperative day 29. The pathological diagnosis was ypT1bN0M0 Stage IA (UICC-TNM 7th edition). The patient was followed for 2 years with no signs of cancer recurrence. |
A 20-year-old African American man was admitted to a psychiatric facility for psychosis. On initial presentation, the patient had an antalgic gait, which he attributed to his history of dopa-responsive dystonia. His mood was depressed and his affect was restricted. He had disorganized thought process and was slow to recall. He endorsed auditory hallucinations, paranoid delusions, depressive symptoms, frequent night awakenings, and persecutory nightmares. Per the ambulance report, the patient was wandering the streets in a confused state, so bystanders called 911. The patient stated that he had been homeless for the past 3 weeks. During this 3-week period, he admitted to not being complaint with his medications. Urine toxicology screen was negative.\nPer medical records, he was diagnosed with dopa-responsive dystonia at age 11 after a 2.5-year history of progressive abnormal gait. He was initially misdiagnosed with tight heel cords at age 10 and treated with serial casting that resulted in good improvement on the right but marginal improvement on the left. His toe walking became more pronounced overtime accompanied by worsening left calf pain and stiffness, increasingly frequent falls, and new onset of intermittent torticollis. These symptoms worsened over the course of the day. He was eventually taken to an urban teaching hospital, where he was diagnosed with dopa-responsive dystonia based on clinical presentation and marked improvement on a levodopa trial. Magnetic resonance imaging of the brain and spine was unremarkable at the time.\nAt age 15, he was diagnosed with schizoaffective disorder bipolar type. His psychiatric history is also significant for multiple psychiatric hospitalizations, history of previous suicide attempts with medication overdose, and history of trauma. He also endorsed marijuana use since age 15 and daily tobacco use since age 18. He denies using any other illicit drugs. Per collateral information from his mother, his schizoaffective disorder has never been well controlled given the conflicting effects of his medications. She also mentioned that he was placed in individualized education programs as a child due to learning disabilities. His family history is significant for bipolar disorder on his maternal side. His family history on his paternal side is unknown. In addition to carbidopa-levodopa, his outpatient medications included sertraline, divalproex sodium, aripiprazole, and benztropine.\nOn hospital day 1, he was started on carbidopa-levodopa 25/100 mg tablet three times daily for dopa-responsive dystonia. On day 2 of his hospital course, sertraline 50 mg once daily, benztropine 2 mg twice daily, divalproex sodium 500 mg twice daily, and risperidone 0.5 mg twice daily were added to his medication regimen. We started him on a low-dose risperidone to avoid exacerbating his dopa-responsive dystonia symptoms. Physical exams were also performed daily to assess for dystonia and parkinsonian symptoms. His initial physical exam revealed an antalgic gait secondary to left lower extremity dystonia, which improved by hospital day 2 and resolved by hospital day 3. On hospital day 3, he became agitated and aggressive with staff members, which led to intramuscular administrations of haloperidol 10 mg, diphenhydramine 50 mg, and lorazepam 2 mg. He continued to endorse auditory hallucinations, so risperidone was increased to 0.5 mg in the morning and 1 mg at bedtime. His auditory hallucinations resolved and then returned on day 6. He reported hearing “good” voices and “bad” voices. He also continued to endorse depressive symptoms, multiple night awakenings, and persecutory nightmares. As a result, his risperidone dosage was increased to 1 mg twice daily. On hospital day 7, the patient reported hearing “mumbling” voices only and improvement in his sleep and depressive symptoms. On hospital day 8, his auditory hallucinations fully abated. By hospital day 10, he slept throughout the night, no longer had depressive symptoms, and had normal spontaneous speech. His thought process was linear, logical, and goal-oriented. His mood and affect was euthymic and full range. No psychotic symptoms were noted. The patient was compliant with his medications throughout the whole hospital course and his daily physical exams were negative for dystonia or parkinsonian symptoms since day 3 of his hospitalization. He was subsequently discharged on hospital day 14 with appropriate outpatient follow-up. |
A thirty-two year old male presented with painful swelling on the nasal side of his left lower lid and complained medial canthal swelling, tenderness, chemosis, and epiphora. Oral antibiotics administered in another hospital did not relieve these symptoms. He was involved in a traffic accident fourteen years ago and at that time suffered from a left side facial bone fracture and left sided visual disturbance as a result of a choroidal rupture in his left eye. The patient underwent surgery to reconstruct the ethmoidal sinus. At the time of the initial visit, his uncorrected visual acuity was 20/20 in the right eye and counting fingers 10 cm from his left eye. The left lower lid showed severe erythematous swelling, particularly around the medial canthus, and there was a moderate degree of conjunctival injection and chemosis (). A complete blood count, urinalysis, and simple chest X-ray were within the normal limits. However, regurgitation of the purulent discharge from the lower lacrimal punctum upon lacrimal sac massage was observed. Accordingly, the patient was diagnosed with acute dacryocystitis and a bacterial culture examination was carried out. An endoscopic transnasal dacryocystorhinostomy was performed the next day.\nSurgery was performed under the general anesthesia and endotracheal intubation was held at the 45° position. Packing gauze soaked with a mixture of epinephrine and 2% lidocaine at a ratio of 1:100,000 was inserted one hour before the surgery, which was removed at the time of the surgery. A 20-gauge illuminator for the vitreoretinal surgery was placed into the lacrimal sac via the lacrimal punctum so an overview of the lacrimal sac and surrounding structure could be obtained. An electrocautery device tipped with a Colorado needle was used to cauterize and ablate the nasal mucosa, while a Freer elevator was used to expose the maxillonasal suture and adjacent bony structure. An osteotomy was made principally using a Kerrison punch and a microdrill. The surgical finding showed severe necrosis around the lacrimal sac and a 20×15-mm sized silastic sheet crumpled within the purulent discharge (). The sheet was removed, the lacrimal sac irrigated with an antibiotic solution (Gentamicin 80 mg/2 ml), and the medial half of the lacrimal sac was excised using a sickle knife and upward Blakesley forceps. Subsequently, gauze soaked with mitomycin C 0.4 mg/ml was placed into the osteotomy site for approximately two minutes and removed. The area was then irrigated with normal saline. A silastic tube (FCI bicanalicular intubation set®, France Chirurge instrumentation, France) with a 0.8-mm outer gauze was applied into the lacrimal pathway from the upper and lower punctum to the nasal cavity, which was then sutured with 4-0 nylon and positioned in the nasal cavity. A polyvinyl resin (Merocel®) pasted with antibiotic ointment was packed into the nasal cavity in order to prevent nasal bleeding, and to maintain the patency of the lacrimal sac opening.\nOne day after surgery, the silicone tube was positioned at the medial canthus. The painful swelling around the medial canthal area of the left lower lid was slightly decreased, and no pus-like discharge was found upon lacrimal sac massage. The patient was discharged with a prescription of topical antibiotics, steroid, oral antibiotics, and non-steroidal anti-inflammatory agent, and was followed up at the outpatient department. The painful swelling on the nasal side of his left lower lid resolved gradually, and there were no symptomatic complications after removing the silicone tube three months later (). |
A 28-year-old male patient was admitted in our hospital after a syncopal ventricular arrhythmia that requested external DC shock in the emergency room to restore sinus rhythm and hemodynamics.\nIn sinus rhythm, the patient showed a type 1 Brugada pattern at rest ECG.\nAccording to his young age and no need for pacing, we suggested an subcutaneous implantable cardioverter defibrillator (S-ICD) system implantation.\nIn June 2017, the S-ICD system (EMBLEM A219) was implanted creating an intermuscular pocket for the device, with the lead positioned vertically in the subcutaneous tissue, 2 cm sternal midline. left. We preferred to use a two incision technique in order to avoid the superior scar in this young patient. The system position was previously defined by positioning a demo over the patient's chest, in order to check by fluoroscopy the best position for can and lead, thus establishing landmarkers.\nAfter incisions closure, the S-ICD selected the secondary sensing vector as the best vector, according to the evaluation of signals only in supine position, immediately prior to the induction test. This vector was permanently programmed with an 1x gain, and the S-ECG template was acquired. The induction test was performed inducing ventricular fibrillation through the device itself, but the S-ICD was unable to restore sinus rhythm by a 65 J biphasic shock, in standard polarity, despite 69 Ohms shock impedance, and a 200 J shock by external defibrillator was required. A new attempt was performed programming the first shock modality in reverse polarity. Again, after induction, the S-ICD was unable to restore sinus rhythm by 65 J and a new 200 J external shock was required (the subcutaneous shock impedance was still 69 Ohms) (Fig. ).\nAt this point, we decided to reassess the system position by fluoroscopy. In posteroanterior projection, the system seemed in the same position with respect to the probe. Looking in latero-lateral projection, we noted that not all the coil lead was on the fascial plane (Fig. ).\nProbably, during vertical tunnelization, the tunneling tool was moved up, thus positioning the tip of the lead into the fat after under the skin. As showed by mathematical simulation , the fat is a poor current conductor and its presence between coil and sternal facial plane decreases considerably the defibrillation efficacy. Thus, a lead repositioning, keeping the tunneling tool down for all the way, using fingers on the surface of the sternum over the tip of the tunneling tool to help guide the tip and stay close to the facial plane, was performed. The new lead position was checked by fluoroscopy in latero-lateral projection (Fig. \n) and, after xyphoide incision closure, a new induction test was performed. The arrhythmia was induced by 2 sec alternate current delivery through the device. The S-ICD promptly restored sinus rhythm by a single 65 J shock in standard polarity (Fig. ). The shock impedance value decreased to 44 Ohms. |
A 3-year-old boy [] reported along with his mother to the Department of Pedodontics and Preventive Dentistry, AME's Dental College and Hospital Raichur, with the chief complaint of painful gingival growth in the upper and lower, right and left regions of jaw since 1 month. Pain was sudden in onset, intermittent in nature, nonradiating and aggravates on mastication and relieves on its own. Past dental history revealed that they visited to private dental clinic 1 month back with the similar chief complaint and routine analgesics and antibiotics were given. However, there was no improvement in patient's condition and was referred to AME's Dental College for treatment. There was no relevant history of trauma and medical history and family history were insignificant.\nOn general examination, patient was healthy and moderately built except the complaint. Extraoral examination revealed a diffuse swelling in relation to the left side of lower jaw, 4 cm × 2 cm in size extending from lower border of the body of mandible to the superior border of ramus of mandible. The swelling was firm in consistency and tender. There were palpable left submandibular lymph nodes 0.5 cm × 2 cm sized, firm and nontender. All inspectory findings were confirmed on palpation.\nIntraoral examination exhibited a diffused, erythematous swelling over attached gingiva with respect to 55, 65, 75 and 85 over buccal aspect and 55 and 65 with a palatal aspect. Mucosa over the swelling was ulcerated covered with necrotic slough with tiny bleeding spots with 55, 65, 75 and 85 [Figure –]. Grade II mobility was recorded with 55, 65, 75 and 85. Intraoral periapical radiograph of 55, 74 and 75 revealed a mild radiolucent lesion surrounding the root of 55, 74 and 75 [Figure and ].\nBased on the case history, a provisional diagnosis was made as localized chronic periodontitis with 55, 65, 75 and 85. The differential diagnoses were made as Papillon–Lefevre syndrome, Eosinophilic granuloma and cyclic neutropenia. Blood investigations revealed anemia (10.5 g%), and the peripheral blood smear revealed microcytic hypochromic anemia. The findings of complete urine and liver function tests were normal; creatine (0.3 mg/dl, normal = 0.4–1.4 mg/dl) and C-reactive protein (2.5 mg/dl normal ≤2.8 mg/dl) levels were in normal range. Patient was negative with HIV and hepatitis B surface antigen.\nOrthopantogram [] revealed multiple areas of bone loss in the left mandibular region. Axial and coronal computed tomography [CT] [Figure and ] revealed multiple soft tissue density lesions with irregular and punched out bony destruction noted involving left mandibular, left side of occiput, right maxillary and right temporal bone. Three-dimensional CT [] revealed multiple osteolytic lesions in relation to maxillary alveolar process, body and ramus of the left side of the mandible.\nHistopathology revealed hypercellular discohesive singly scattered Langerhans cells which are having abundant eosinophilic cytoplasm with characteristic retiform, convoluted nuclei with distinct longitudinal grooves. Also seen are binucleated and multinucleated cells with similar nuclear features. Background is showing a variable number of lymphocyte, eosinophils and plasma cells and occasional mitosis [].\nThe definitive diagnosis of LCH involving multiple bones was considered. It was correlated on the basis of clinical, radiographical and histopathological findings. The patient was referred to the nearby cancer institute for further treatment. |
A 51-year-old Caucasian left-handed housewife lady (weight 61 kg, height 159 cm) was admitted to our institution because of severe sudden onset headache followed by transient loss of consciousness and vomiting. On examination she was fully conscious and oriented and complained of severe headache. Nuchal rigidity was evident. No focal neurological deficit was found. Temperature was 38°C and other vital signs were stable. Past medical history was negative and she did not consume any medication.\nCT scan of the head revealed acute subarachnoid hemorrhage prominently occupying the right sylvian fissure. CT angiography was performed (figure ). The cerebral vasculature was found to harbor three saccular aneurysms at: (1) right MCA trifurcation, (2) left ICA bifurcation, and (3) distal basilar artery. The right MCA aneurysm was presumed to be the ruptured aneurysm due to location of subarachnoid clot and irregular shape of the aneurysm.\nA right pterional craniotomy was performed with the patient in the supine position and head rotated 30 degrees to left. After elevation of the craniotomy flap, the sphenoid ridge was drilled flush with the orbital roof to facilitate exposure of the basilar tip aneurysm. After duratomy, gentle frontal lobe retraction allowed CSF drainage from the optic and carotid cisterns. Right optic nerve and right ICA were identified and the Sylvian fissure was opened from medial to lateral, following the bifurcation of right ICA to MCA trifurcation. A saccular aneurysm was found in the MCA trifurcation projecting inferolaterally. After dissection of the neck it was clipped. Next, opening the membrane of Liliequist, let to the distal basilar aneurysm from the corridor between the right optic nerve and right ICA. The aneurysm was located between the right PCA and right SCA with a wide neck, and its dome projected laterally to right. Successful clipping was achieved. The left ICA bifurcation aneurysm was identified after following the right A1, anterior communicating and left A1 arteries with minimal brain retraction, and gaining benefit from favorable neuroanesthesia. The aneurysm projected superiorly and was also clipped (figure ).\nThe patient experienced an uneventful post-operative period and was discharge within 5 days of surgery. A follow-up CT angiography confirmed successful obliteration of all lesions and preservation of normal cerebral vasculature (figure ). |
The patient was a 76-year-old Caucasian male with a long history of recurrent multiple meningiomas. The patient's previous medical history includes hypertension, dyslipidemia, and lower acute myocardial infarction at age 44. At age 47, the patient was first diagnosed with right parietal parasagittal meningioma involving the falx cerebri, which was treated with surgery. Twenty years later, due to progression of residual left-sided hemiparesis, the patient was diagnosed with a recurrence for which he underwent a second surgery at age 68. This second surgical resection was incomplete, leaving a thin layer of tumour tissue in the posterior third of the sagittal sinus. Histopathology was compatible with meningothelial meningioma. Magnetic resonance imaging (MRI) of the posterior brain showed multiple early recurrences (2 parasaggital, 1 parietal, and 1 frontoparietal). Due to progression of these lesions, a third surgery was performed 3 years later, at age 71. This surgery resulted in multiple postoperative complications, the most important of which were a cerebrospinal fluid fistula requiring a ventriculopleural shunt, repeated failed graft reconstructions due to infections, and an intestinal perforation (due to a laparotomy performed to obtain a microvascular omental graft) leading to peritonitis and, subsequently, right hemicolectomy.\nSixteen months after the third surgery, a follow-up MRI showed clear signs of disease progression, with multiple extra-axial lesions involving the falx cerebri. After multidisciplinary assessment, the patient was referred for radiotherapy. Three months following radiotherapy treatment, radiological examination revealed a new progression, with growth in the maximal diameter of the sum of the lesions (from 8 to 8.4 cm). The patient's long history of recurrent meningiomas, along with three surgeries and radiotherapy, has left him with important sequelae, including left hemiparesis and partial seizures (since the second surgery), currently being treated with anticonvulsant therapy.\nGiven the patient's extensive history of recurrent meningiomas and progression after radiotherapy, various medical treatment options were considered. After much deliberation, we elected to initiate compassionate-use treatment with extended-release somatostatin analogues. This decision was based on the previously performed Octreoscan showing positive uptake on the somatostatin receptors, and published reports of clinical response to this medication, which is also reported to be well tolerated. We prescribed subcutaneous injection of lanreotide (Somatuline Autogel®) at a dose of 120 mg every 28 days due to the convenience of this delivery method. Brain MRIs performed every 6 months thereafter revealed a discreet but continuous radiological improvement. Given these findings, we maintained medical treatment without interruption to the present time (currently >2 years of treatment). At exactly 2 years from treatment initiation with lanreotide, the volume of the lesions had decreased by approximately 35%. Clinically, the patient continues to receive rehabilitation for neurological deterioration. |
We present a 48-year-old male who arrived at our academic level I trauma center complaining of left shoulder, back, and hip pain with corresponding flail chest. Initial workup identified several injuries, including a left pubic ramus fracture, a left sacral fracture, a left scapular fracture, a chronic right dislocated temporomandibular joint, a left basilar pulmonary contusion, a left hemopneumothorax, a small retroperitoneal hematoma without active extravasation, and flail chest. While flail chest was identified, the initial computerized tomography (CT) report did not identify all the patient’s rib fractures. He was not originally deemed an optimal surgical fixation candidate for several reasons related to the initial imaging: many of the fractures were too close to the corresponding transverse process for fixation, many of the fractures were in line with minimal displacement, and the costocartillage fractures were not readily discernible. Due to the extent of his injuries, the decision was made to admit him to the Surgical Trauma Intensive Care Unit (STICU).\nOn hospital day (HD) two, orthopedic surgery was consulted and recommended nonoperative treatment of his pelvic and scapular injuries. A thoracic epidural catheter was placed for pain management, and a chest tube was inserted for an enlarging hemothorax. The patient was transferred to a lower level of care but later developed respiratory distress and was readmitted to the STICU on BiPAP. On HD 10, he was intubated for further respiratory insufficiency and altered mental status. A repeat chest CT scan then revealed greater displacement and offset of the previously identified fractures as well as improved visibility of the costocartillage fractures (Table ). He was then deemed to be an appropriate surgical candidate on HD 13 and received surgical stabilization of rib fractures (SSRF) on the left side. He returned to the operating room on HD 15 for completion of SSRF. MatrixRIBTM plates and sternal locking screws (MatrixRIBTM Fixation System, DePuy Synthes, Raynham, MA) were used across six separate ribs in nine total locations (Table ). Other fracture locations were not deemed appropriate for fixation due to the patient’s body habitus and concern for damage to surrounding tissue. The procedures were without complications, and he returned to the STICU postoperatively.\nHe was able to be extubated on HD 16. On HD 40, the patient was reintubated for respiratory insufficiency but afterward became bradycardic with pulseless electrical activity (PEA) and required cardiopulmonary resuscitation (CPR) with the return of spontaneous circulation after two rounds of defibrillation. This corresponded to SSRF postoperative day 27. After his cardiac event, CT revealed hardware failure with one locking screw separating from a plate at the left costochondral junction and another along the sternum (Table ). The sternal plate had partial separation from the sternum as well (Figure ). An isolated hardware failure was also noted at the posterolateral segment of a rib close to the transverse process of the corresponding vertebrae. The screw in this instant was not freed to the same extent as was the sternal and costochondral segments and could potentially have been an imaging artifact. The patient ultimately died on HD 56 from PEA arrest. |
In August 2013, our 45-year-old female patient underwent a gross total resection of a 2.5 cm non-functioning pituitary adenoma with degenerative changes and recent hemorrhage, at an outside institute. Pathologic examination revealed small sheets of pleomorphic cells with positive staining for synaptophysin, chromogranin, CAM5.2, and AE1/AE3. She was subsequently followed regularly until March 2014, when repeat imaging showed her tumor had recurred in the sella turcica with asymptomatic invasion into the left cavernous sinus. The patient underwent a debulking resection of the sellar component of the tumor. Upon pathologic review, the recurrence specimen was found to now stain positively for ACTH as well as contain some atypical features including nuclear pleomorphism, large nucleoli, mitotic figures, and Ki-67 staining of 8–12%. Although lacking p53 nuclear expression, the tumor's increased mitosis, Ki-67, and invasive phenotype led to the classification of atypical pituitary adenoma []. She remained without Cushinoid symptoms at that time. She tolerated the surgical resection well and subsequently underwent postoperative CyberKnife radiation to a dose of 25 Gy given in 5 fractions to the residual mass completed in June 2014. On radiographic follow-up, a small amount of residual disease was observed in the left cavernous sinus. Over the next year and a half, the adenoma demonstrated slow, but persistent progression, and the patient began complaining of issues in her left eye concerning for CN III, IV, and VI palsies including lid ptosis and difficulty with left eye abduction and adduction. The diplopia progressed to persistent double vision, which was compounded by intermittent severe headaches causing nausea and vomiting.\nDue to her clinical and symptomatic progression, she established care with our institution in December 2015 to discuss repeat irradiation for her recurrent pituitary macroadenoma and undergo a full endocrine evaluation. At that time, her salivary cortisol levels were found to be approximately four times the upper limit of normal though she remained clinically asymptomatic. She initiated treatment with pasireotide 0.6 mg/mL subcutaneously twice a day and cabergoline 0.5 mg twice a week for this clinically-silent, ACTH-secreting pituitary adenoma with the intent to slowly titrate up her cabergoline dose to 2 mg twice a week. Repeat brain MRI two months later showed disease progression with her soft-tissue mass measuring 3.0 × 1.9 × 2.1 cm compared to 1.7 × 2.0 × 2.7 previously. The MRI also revealed persistent left cavernous sinus involvement with vascular encasement of the left internal carotid artery and newly-identified possible mild abutment of the left optic nerve. Of note, the patient's ACTH was stably elevated with cortisol levels maintained within normal range at this time.\nWith a lack of disease control with medical management, the patient elected for a repeat course of radiation therapy. In February 2016, the patient underwent a stereotactic radiosurgery (SRS) delivering a single fraction of 14 Gy using a head frame-based technique. Follow-up MRI one month after repeat SRS demonstrated the tumor had only a minimal increase in size, thought to likely represent pseudoprogression given the previously fast growth rate of the neoplasm that now appeared to be relatively stable post-irradiation. Repeat MRI three months later was unchanged and still demonstrated the pituitary adenoma extending into Meckel's cave, partially compressing the internal carotid, and abutting the left optic nerve. A five-month follow-up MRI showed the bulk of the disease to remain unchanged, however, a small, but obvious, enhancement in the oculomotor nerve concerning for progression was identified. Review of the SRS radiation treatment plan confirmed only low dose exposure to CN-III, not favoring but also not ruling out radiation-induced neuritis due to her previous history of fractionated SRS. Disease progression was high on differential. Due to the slow rate of growth and location in question, observation was deemed most appropriate over further treatment intervention. At the seven-month post-radiation follow-up, ACTH levels were found to have increased from 155 to 269 (norm. 0–46 pg/mL) over the month prior, but the salivary and urine cortisol levels remained within normal ranges. Repeat MRI imaging at seven, ten, and fourteen months after SRS all demonstrated a stable, large pituitary lesion with CN-III enhancement and elevated, but stable ACTH levels at seven and ten months after SRS.\nHowever, starting at twelve months post-radiation, her ACTH levels began to steadily increase each month, reaching 667 in June 2017. Following this rise in ACTH, the patient's cortisol levels began to rise despite taking 2 mg of cabergoline twice weekly. These endocrine abnormalities resulted in noticeable weight changes as well as an episode of psychosis secondary to Cushing's with acute anxiety and paranoia prompting hospital admission. On this admission, her ACTH and urine cortisol levels had spiked to 677 pg/mL (norm. 0–46 pg/mL) and 279 µg/dL (norm. ≤45 µg/dL) respectively, despite continued treatment, although serum cortisol levels remained relatively stable at 23.2 mcg/dL (norm. ≤10 mcg/dL). Repeat MRI revealed only mild primary tumor progression with a tumor size of 2.9 × 1.8 × 2.6 increased from 2.7 × 1.7 × 2.6 in April 2017 and 2.6 × 2 × 1.7 in December 2016. A multi-disciplinary, neuro-oncology tumor board suggested the use of PET-CT to identify a potential extra-pituitary source as an explanation for this atypical disease presentation. The board also recommended temozolomide (150 mg/m2 nightly for five consecutive nights) in an attempt to slow any new growth or invasion and added ketoconazole (500 mg BID) to her on going cabergoline treatment to manage her Cushing's disease. The PET-CT confirmed the presence of an FDG-avid pituitary mass with additional lesions in the liver and left occipital condyle-lateral C1 vertebra with associated bony destruction as well as potential involvement of the left adrenal gland. Biopsy of a liver lesion confirmed metastatic pituitary corticotroph carcinoma. In July 2017, the patient underwent RFA to her liver lesions and SRS (16 Gy in 1 fraction) to her C1 lesion.\nOne week after receiving RFA and the same day as undergoing SRS, the patient's ACTH levels had dropped dramatically to 188 (down from 677 measured three weeks prior) and displayed a decreasing trend with monthly levels measured at 128, 86, 78, 59, and 53 pg/mL. Serum cortisol levels have returned to normal ranges with a nadir of 2.9 mcg/dL (norm. ≤10 mcg/dL) four months following her RFA and SRS treatments and have remained stable since. It should be noted that ACTH did display suppression after dexamethasone administration, < 12 pg/mL suppression. Monthly repeat imaging (both CT and MRI) for three consecutive months following RFA and SRS showed the three ablated hepatic lesions had decreased in size, while the vertebral and pituitary masses had remained stable. No other areas concerning for disease have been identified, and per these findings, three-month imaging intervals were initiated. Her most recent scan (14 months following RFA/spine SRS) showed decreasing size in the both the primary and metastatic sites.\nSince receiving her most recent RFA and SRS treatment, fourteen months have passed, and the patient has finished her eleventh cycle of temozolomide (150 mg/mm2 given over 5 consecutive days every 4 weeks) and pasireotide 0.6 mg/mL subcutaneously twice a day. All diagnostic scans and lab tests suggest her disease is in remission. Moreover, function has begun to be restored in the patient's eye movement, and her diplopia has become less severe. |
A 22-year-old multiparous woman with a history of primary sclerosing cholangitis complicated by portal hypertension, esophageal varices, and thrombocytopenia was admitted at 29 0/7 weeks for evaluation and management of shortness of breath and lower extremity edema. Her obstetric history was significant for prior spontaneous preterm birth with placement of an ultrasound-indicated McDonald cerclage at 20 1/7 weeks of gestation in the index pregnancy. At the time of cerclage placement, the 5 mm Mersilene (polyester) tape was noted to be suboptimally placed along the posterior rim, distal to the internal os, so a second McDonald cerclage of 0-polyester suture was placed cephalad to the tape. She was admitted at 28 0/7 weeks for preterm labor and the cerclages were removed with no remarkable anatomic irregularities. She received antenatal corticosteroids and magnesium sulfate during her admission for preterm labor and was discharged on hospital day #3 with a cervical exam of 1cm dilation and 70% effacement. During her readmission at 29 0/7 weeks for shortness of breath, she began to have contractions and moderate vaginal bleeding on hospital day #3. Her cervical exam was 4 cm and 90% effaced. She was transferred to labor and delivery, where she continued to have a rapid cervical change to 6 cm. A brief fetal heart rate bradycardia occurred with spontaneous recovery to a normal baseline. At this time, findings were concerning for placental abruption or possible coagulopathy from decompensated liver failure with a total estimated blood loss of 500 mL. Amniotomy was performed to expedite vaginal delivery. However, cervical dilation did not continue and complete cessation of vaginal bleeding was noted. Careful visualization of the cervix revealed that the posterior rim of cervix was detached and traversing the presenting fetal head creating a tension band that prevented further dilation or descent. This band of cervical tissue was clamped and suture-ligated followed by rapid delivery. She delivered a male infant with a weight of 1325 g and Apgar scores of 5 and 7 at 1 and 5 minutes, respectively; arterial cord pH was 7.33. Following delivery of the placenta, vaginal exam revealed a partial posterior cervical detachment from 2 to 9 o'clock. The avulsed portion of the cervix did not appear necrotic and was bleeding briskly. Hemostasis was achieved with a single running locked suture. Her postpartum course was otherwise unremarkable, and she was discharged on postpartum day #2. At her six-week postpartum visit her cervical exam was remarkable for a normal appearing anterior lip; however the posterior lip was not visible and not palpable on digital exam (). Pathology was not available for the fragments of cervix that were excised and the placenta was remarkable for acute chorioamnionitis. |
A 62 years old male presented with recurrent SCC of the left nasal bulbar conjunctiva. The patient's previous medical history detailed multiple interventions at this site. Initially, the diagnosis of a left nasal pterygium (a wing-shaped growth that starts on the conjunctiva and can spread across the limbus to the cornea affecting visual function) was made 3 years prior to the reported presentation, and was surgically removed with local excision. The lesion recurred 1-year post-surgery, and was subsequently managed surgically with a wide local excision. At this time, pathology revealed positive margins for SCC. A further recurrence 6 months later was treated with Plaque Therapy to a dose of 50 Gy in 5 fractions. Subsequently, the lesion was re-excised with adjuvant cryotherapy 7 months later.\nOn presentation 3 months post-cryotherapy, a recurrent lesion (10 × 5 × 2 mm) was detected on the nasal bulbar conjunctiva (Fig. ). The patient declined enucleation following this SCC recurrence. Subsequently, multidisciplinary consultation offered SXRT as an alternative treatment option (Fig. ).\nThe patient also presented with a history of Crohns Colitis (treated with immunotherapy) and a previous excision of a non-melanoma skin cancer from the right temple, and a subsequent skin graft of the area. The patient was on no other medications and had no allergies.\nThe patient was prescribed a dose of 48.4 Gy in 22 fractions, to a depth of 3 mm. This resulted in a skin surface dose of 54.4 Gy. A 2.0 cm diameter direct applicator was used with a 3 mm margin on the treatment region (Fig. ). To allow for an acceptable treatment margin that encompases the target volume and accounts for set up variability, the left inner canthus and tear duct were included in the treatment field. A single en-face beam of 2.0 mm Aluminium energy (2 mm Al) was used to meet the previously articulated dose prescription. The risk of ulceration, scleral perforation, ischemia and nasolacrimal duct obstruction was explained to the patient. This risk was further exacerbated in this case due to re-irradiation of the previously treated area, and a particularly sensitive region post-cryotherapy, surgery and plaque therapy. Topical anaesthesia (Tetracaine eyedrops) was administered to the left eye prior to retractor positioning, to ensure eyelids remained open throughout SXRT delivery. After positioning of the retractors the patient was instructed to maintain gaze on a target positioned to the left side of the room, and the eye was monitored throughout treatment delivery by video surveillance. Lubricating eye drops were prescribed for use prior to treatment administration to prevent drying of the ocular surface, and for the patients self-use between fractions to relieve dry eye symptoms. Total set up and treatment time for this patient was approximately 20 min, with the retractors in place on average less than 10 min. The anaesthetic drops and ocular surface lubricants induced limited sensation and the patient found it mildly uncomfortable. As a precaution, the patient was informed to avoid contact with the anaesthetised eye and wear a protective patch for 60 min post-treatment delivery daily. |
An 18-year-old boy, born of a nonconsanguineous marriage, presented to the hospital emergency with a three-day history of dysphagia. The patient gave a history of two similar episodes of dysphagia that were relievedon treatment. He was under the care of a gastroenterologist who performed an endoscopy and a barium swallow that revealed a very tight stricture at the upper end of esophagus []. It was diagnosed asacongenital web and dilated after passing a guide wire. He was referred to dermatology departmentfor pigmentation of skin and nail dystrophy. A detailed history revealed that the patient developed splitting and dystrophy of his fingernails and toenails at 5 years of age. This was followed by pigmentation of the skin within a year. Pigmentation first appeared on the neck and lower limbs, followed by the extensive involvement of the entire body with sparing of the face. Around this time he started developing oral ulcers that were recurrent despite treatment. He gave no history of eye complaints, loss of hair, dental caries, or difficulty in keeping up with studies. Except dysphagia, patient gave no history of systemic symptoms. The patient's maternal uncle had a history of similar type of skin pigmentation and nail dystrophy, and died of unknown causes at 55 years of age.\nA thorough examination of the patient revealed generalized fine, lacy, reticulated, greyish-brown pattern of hyperpigmentation with areas of epidermal atrophy (poikiloderma), sparing the face [Figure and ]. Examination of the genitals revealed no signs of testicular atrophy. Splitting of nails with pterygium formation and dystrophy was present, more over the fingers than the toes []. Examination of the oral mucosa revealed the presence of a whitish, well-defined leukoplakic plaque on the dorsal surface of the tongue, interrupted by lingual ulcers []. Buccal and labial mucosa were spared. There were no significant systemic findings. Routine investigations along with skin biopsy were done. Histopathological examination of skin sample revealed melanophages in the upper dermis and vacuolization of basal cells. Routine investigations of the patient were normal. Bone marrow biopsy and lingual biopsy were refused by the patient. The classic clinical findings, family history and consistent histopathology enabled us to make a diagnosis of DKC. |
A four-year old girl, weighing 9 kg, presented with repeated lower respiratory tract infections and severe failure to thrive. Clinical examination revealed normal vital parameters with a 3/6 ejection systolic murmur in the pulmonary area. The splitting of the second heart sound was wide and fixed. The respiratory system examination was unremarkable. Her ECG revealed sinus a rhythm with QRS axis of +100 degrees. There was rsR' in lead V1. X-ray showed a cardiothoracic ratio of 55% with prominent pulmonary artery shadow. The lung fields were plethoric. Transthoracic echo (TTE) revealed a moderately large sized secundum ASD, measuring 15 mm, with adequate rims (> 5 mm) except the aortic rim which was almost absent. The ASD size was measured on 2D echo, without the use of color flow mapping. There was left superior vena cava (SVC) draining into the coronary sinus, which was dilated.\nThe child was taken for transcatheter closure under general anesthesia, with fluoroscopic and transesophageal echocardiographic (TEE) guidance. TEE confirmed the presence of a moderately large secundum ASD [] measuring 14 mm and having adequate rims all around, except the aortic rim, which was deficient. It was decided to close the defect using a 17 mm ASO (AGA Medical Corp., Plymouth, MN). The defect was not balloon-sized. The ASD was closed successfully using the routine technique of device closure.[] The position of the device was found to be optimum and secured on TEE [] and fluoroscopy [ and ] after the final release. Twenty-four hours later, prior to discharge, the child was subjected to a check TTE , as a part of a routine protocol. The device had moved from its previous position [] with a residual shunt seen superior and anterior to the device. This was confirmed on fluoroscopy []. In view of the unstable position of the device with an evidence of a residual shunt, it was decided to attempt percutaneous retrieval.\nThe procedure was done under general anesthesia. The right jugular vein was cannulated using a 5F hemaquit. A 10F straight long sheath, (Cook Medical Inc, Bloomington, IN), beveled at the distal end, was passed from the right femoral vein and Heparin 100 i.u/Kg was injected. Although the device was not free floating, a 5F bioptome (Cook Medical Inc., Bloomington, IN) introduced from the right jugular vein was used to stabilize the device []. An Amplatz goose neck snare (eV3 Endovascular Inc., Plymouth, MN) with a 15 mm diameter was introduced from the right femoral vein via the 10F sheath through a 0.038” tracker catheter, provided with the snare itself, and the device was held by its screw located on the right atrial disk []. Once the device was held at both the ends, the Amplatz goose neck snare was pulled with a constant pressure without any jerky movements. With a pull of gradually increasing intensity on the snare, accompanied by a gentle tension on the bioptome holding the LA disk, it was possible to slenderize the right atrial disk and retrieve it in the long sheath []. At this juncture, the bioptome was opened and the left atrial disk was released []. The entire device was subsequently pulled into the sheath []. It was then decided to balloon size the defect and proceed with the device closure. Balloon sizing revealed the stretched balloon diameter of the ASD to be 20 mm. Hence, a 20 mm ASO was used and the device was successfully deployed [], using the balloon-assisted technique.[] This technique is routinely used for closing all large ASDs in our laboratory. The child was discharged 24 hours later on aspirin (5 mg/kg/day) with the check echo showing an optimally placed ASO. On follow-up at six weeks, the device was stable with no residual shunt. The right ventricular volume had reduced significantly and the biventricular contractility was normal. The surrounding vital structures were functioning normally and there was no evidence of pericardial effusion or thrombus on the device. |
Patient 1 was a 38 years-old male. In May 2010, this patient was diagnosed with glioma soon after an episode of seizures. MRI showed intra-axial expansive and infiltrative lesions that were cortical and subcortical, and which affected the anterior half of the right temporal lobe and extending from the pole to the Sylvian fissure superiorly and to the right parahippocampal gyrus, posteriorly, and medially. Partial surgical resection was performed in August 2010 and the first pathologic diagnosis was astrocytoma grade II. He underwent chemotherapy with TMZ at a dose of 2,000 mg with cycles every 28 days for 5 days in the years 2011–2013, with no tumor regrowth until the beginning of 2015. At this time, he underwent MRI, which was used to compare the discrete extension of the signal alteration areas, especially the subinsular regions. In March 2015, he resumed chemotherapy with TMZ at a dose of 100 mg/day and the patient then lost 12 kg of body weight, which was associated with anorexia, insomnia, and depression. In May 2015, he suffered a seizure requiring hospitalization. In June 2015, the patient resumed the old chemotherapy regimen with TMZ (2,000 mg every 28 days for 5 days), and a follow-up with MRI; however, the tumor size continued to increase. In January 2016, the neuro-oncology team decided to discontinue treatment with TMZ considering the risk/benefit and planned a surgical re-approach. This was followed by chemoradiation and lasting 6 cycles of PCV associated with CBD. The CBD dosage was ranging from 300 to 450 mg/day.\nDuring chemoradiation, the patient had an excellent clinical performance, practiced sports and had few symptoms of fatigue and/or nausea.\nAt 1 month after the end of chemoradiation, control MRI (Figure ) was characterized by exacerbation and the ultra-precocious phenomenon of PSD with increased edema and inflammatory disease characterized by extensive areas of contrast enhancement associated with tissue hypoperfusion (not shown). MRI controls demonstrated the progressive reduction of these findings.\nThe result of a pathological study after the first surgery was astrocytoma grade II with Ki67 staining of 5%. After the second surgery, he progressed to GBM grade IV (Figure ), related to increased cellularity, frequent mitosis, presence of micronecrosis, microvascular proliferation/endothelial, Ki67 staining of 30%, and loss of ATRX expression. Biomolecular marker analysis indicated IDH-1 mutated and MGMT methylated. |
A 70-year-old gentleman presented with a painless swelling in the right thigh for 7 months, which was spontaneous. He had a history of femur shaft fracture, for which open femur nailing was done 10 years ago. Postoperatively, he had uneventful wound healing and fracture union. He has no recollection of any complications following the surgery and was able to walk full-weight-bearing without any aid. Clinically, he had a large diffuse swelling on the anterolateral aspect of the right thigh which was around 10 cm × 8 cm. The swelling was firm, non-tender, and non-pulsatile, with ill-defined margins. The presence of a longitudinal scar along the entire length of the lateral aspect of the thigh was noted, which had healed by primary intention (). The right hip and knee movements were normal. Routine hematological tests were done, including erythrocyte sedimentation rate and C-reactive protein. All the blood reports were normal.\nX-rays of the femur were done in anteroposterior and lateral views () which showed an irregular, hypertrophic cortical reaction with the bony remodeling in the proximal femur and an excavated lateral femoral cortex with a broken intramedullary nail in situ.\nMagnetic resonance imaging (MRI) of the right thigh was done, which showed a large, well defined, and heterogenous soft-tissue mass on the anterolateral aspect (T1 and T2 images). Post-contrast images showed a heterogeneous and patchy uptake (). These findings were consistent with a soft-tissue tumor. We, however, did not perform a computed tomography (CT) scan for this patient.\nAfter the MRI, we decided to do a closed needle biopsy along the previous surgical scar under local anesthesia in the operating theatre. The biopsy report was inconclusive, demonstrating fibrinous blood material and granulation tissue suggestive of a chronic hematoma.\nAs the patient was symptomatic, we decided to plan a surgical excision of the lesion. Under epidural anesthesia, in the supine position. The skin incision was taken along the previous scar on the lateral aspect of the thigh. Granulation tissue and fibrosed muscles overlying the mass were carefully separated. We noticed a mass adherent to the lateral aspect of the femur extending intramedullary up to the implant. Using a cautery, we separated the mass from the bone extending up to the intramedullary nail. The excised specimen measured 24 cm in length and 15 cm in breadth. An intraoperative frozen section analysis was done that revealed a chronic inflammatory reaction and no evidence of sarcoma. The defect after excision of the mass was filled with frozen allograft. The excised specimen was solid with a fibrotic wall; on gross examination, after dissecting the specimen, a mop was found ( and ).\nThe sample was sent for histological study which revealed fibrous strands, clots, hemolyzed pigments of red blood cells inflammatory cells, and multiple fiber strands. The foreign material was studied under polarized light () and confirmed the diagnosis of gossypiboma by demonstrating cotton fibers. The specimen was reported as an accumulation of blood products and their degraded components and not a tumor.\nThe patient had an uneventful surgical procedure and was mobilized and rehabilitation was started on day 1 postoperatively. |
A 46-year-old nondiabetic woman from a middle-class family, presented with discharging sinuses over the lower anterior abdominal wall for the past 2 years. There was no rise of temperature or weight loss. Her incision wound from laparoscopic appendectomy in 1998 did not heal properly and the surgical site was reopened after 1 month. There was no healing of the incision wound after reexcision. After that the whole area started to become erythematous and indurated and later on a keloid started developing over the scar. A large sinus developed over the operation scar and the other stitch sites also developed into sinuses discharging pus. However, there was no history of cough, haemoptysis, breathlessness, and anorexia. There was no history of intake of any immunosuppressive drugs/corticosteroids, any local trauma/injury, or tuberculosis. She had been treated off and on without having any definitive microbiological diagnosis with multiple injectable and oral antibiotics before consultation at this hospital. She did not have any contact with tuberculosis patients.\nOn examination, a previous-surgical scar was present over which a large keloid had formed, on the lower anterior abdominal wall in the midline (although a little more towards the right). There was no apparent mass over the abdomen, but 6 sinus openings were noted over the lower anterior abdominal wall (). At the time of presentation a single sinus was discharging pus. On palpation, tenderness was noted over and around the sinuses. The sinus opening was bluish, wide, and undermined and it was discharging a serosanguinous exudate admixed with pus. Complete haemogram and clinical chemistry were within normal limits including blood sugar. HIV ELISA negative by two kits, C-reactive protein normal and the ESR, was peaked at 22 mm. Pus from the discharging sinus was collected and processed using standard procedures. Gram stained smear showed plenty of pus cells with Gram variable bacilli (many of which were Gram positive) and on Ziehl–Neelsen staining numerous acid fast bacilli could be observed. The discharge was cultured on blood agar, MacConkey medium, and Lowenstein-Jensen medium and inoculated into a BacT/ALERT MP bottle, which was incubated in the BacT/ALERT 3D system (bioMerieux, USA). The blood agar and MacConkey medium were incubated under aerobic conditions at 37°C. The blood agar plate showed numerous dry, small nonhemolytic colonies after 72 hours but there was no growth on the MacConkey medium. The BacT/ALERT MP showed positive growth in 5 days for acid fast bacilli, which on further subculture onto L-J medium gave dry, rough, pigmented, and yellow to orange colonies after one-week period (). They were found to be scotochromogenic at 37°C. Speciation for the isolate was confirmed by the GenoType Mycobacterium CM/AS assay based on Mycobacterium DNA strip technology (Hain Lifescience, Nehren, Germany), [] following manufacturer's instructions. It showed a mixed infection caused by two NTM species, that is, Mycobacterium szulgai and M. intermedium. To confirm microbiological result, culture from repeat sample after one week showed the same dual infection caused by Mycobacterium szulgai and M. intermedium by the GenoType Mycobacterium CM/AS assay. Drug susceptibility testing (DST) was performed by standard disk diffusion method over Middlebrook 7H10 agar supplemented with OADC (oleic acid, dextrose, and citrate) and the following results were obtained: sensitive to linezolid, levofloxacin, clarithromycin, amikacin, and ciprofloxacin and found to be resistant to cotrimoxazole. DST for 1st and 2nd line antitubercular drugs were done by the GenoType MTBDRplus and MTBDRsl assay (Hain Lifescience, Nehren, Germany) which showed resistance to isoniazid and sensitive to rifampicin, fluoroquinolones, aminoglycosides/cyclic peptides, and ethambutol.\nThe patient was advised a combination of rifampicin 600 mg once daily, ethambutol 600 mg once daily, and clarithromycin 500 mg twice daily to be taken along with periodic follow-up based upon clinical response as well as microbiological response.\nShe was advised to continue the therapy and when she presented to the hospital after one month, there was no pus discharge from the sinus. She was advised to continue the therapy and report to hospital after another month. Again after one month of therapy, there was no discharge present and the sinus was almost completely healed. Thereafter, she was advised to continue the same therapy for another two months. Thereafter, patient was completely all right with no such discharge after completion of two-month antibiotic therapy. |
A 24-year-old unmarried male, pursuing graduate studies hailing from higher socio-economic urban background with family history of one manic episode in a second-degree relative and nil significant past history, presented with 4 years illness of insidious onset, continuous and deteriorating course characterized by repetitive behavior of arranging things (pens, books, shoes, ties, clothes etc.) due to ideas of perfection. Initially, for about 1 year, even if these symptoms were causing impairment in socio-occupational functioning, the patient did not consider them irrelevant. However, gradually he started feeling that these thoughts and behavior were excessive, irrelevant, unwanted, intrusive and consuming a lot of time. Yet, initially he did not seek treatment. He used to feel distressed whenever he tried to resist these acts or reduce the duration of acts.\nAfter 3 years of onset of illness, the patient started showing an unusual behavior of proposing love to girls impulsively. Whenever he would see a girl in any public place, he would have a strong desire to talk to her and propose her. Even though many times he felt that he should not repeat it, he could not control the impulse to talk and propose. Any hindrances in execution of this intention, for example, presence of own family members or the girl being involved in some other work would cause significant distress. Usually, he would approach the girl, introduce himself, praise her for physical characteristics and immediately express her liking to her. Most of the times, this sudden and unexpected way of proposing love would result in negative response from them; however, he would feel quite relaxed after doing so. In this manner, he proposed numerous girls in 1 year duration and many times, girls as well as authorities of his educational institute have complained his parents; subsequently, his classmate girls started avoiding him. Many a times, he missed his classes because of this behavior. When his parents asked the reason for proposing girls in this manner, his usual response was “just like that”. Many a times, he expressed to parents about his inability to control this behavior, parents, instead, blamed him of purposefully doing so. During interview, he expressed his inability to control the proposing behavior whenever he saw a girl and denied of any form of obsession associated with proposing behavior as well as had no intention of having sexual relationship or any plan of getting marriage. If due to any reason he was not able to propose girl, for example, if the girl left the place before he could approach her, he felt tensed and restless for a few minutes and then settled down.\nHis sleep, appetite, weight and libido were not disturbed. There was no history of repetitive washing or checking, any abnormal involuntary movements in any body parts, persistent elevated or depressed mood, increased psychomotor activity, substance abuse, delusions or hallucinations. Likewise, there was no history of stealing, fire setting, pulling hairs, excessive masturbation, skin picking and nail biting. As there was no temporal correlation between intake of anti-obsessive medicines and behavior of proposing girls, a chance of any hypomanic or manic switch, due to anti-obsessive medicine, was ruled out. This patient was diagnosed as having OCD with other habit and impulse disorders according to ICD-10.\nAlong with the behavior of proposing girls, various other repetitive behaviors in the form of arranging things were present. The patient and his family members felt about 40% improvements in his repetitive behavior, except the behavior of proposing girls, after around 8 weeks of treatment with 20 mg fluoxetine and 50 mg fluvoxamine. One month later in follow-up, carbamazepine-400 mg was added as anti-impulsive medication along with above medications, since there was no improvement in proposing behavior. In subsequent follow-up after 2 months, minimal improvement was seen in proposing behavior and about 60% improvement in other repetitive behavior and last follow-up subsequently. |
The first patient is a 14-year-old male who was admitted to Fuwai Hospital, Beijing, in March 2012 with intermittent syncope that had been ongoing over 2 years. Echocardiography showed multiple cardiac myxomas in the left atrium, left ventricle, and right ventricle (Figure A,B). The cardiac myxomas in the left atrium, left ventricle, and right ventricle were confirmed by MRI examination (Figure ). Upon physical examination, the patient had spotty skin pigmentation on his face (Figure ). The results of further evaluations are presented in Table .\nThe patient underwent surgery on 9 March 2012. The surgeon performed a median sternotomy and cardiopulmonary bypass using bicaval cannulation and ascending aorta cannulation with cardiac arrest. Myocardial protection was achieved by antegrade cardioplegia with St. Thomas solution. A 18 × 20 × 20 mm3 myxoma attached to the anterior mitral valve was removed through an interatrial septum incision (Figure A,B). Five myxomas were discovered in the right ventricle attached to cardiac muscle. Three myxomas measuring 18 × 20 × 20, 20 × 20 × 20, and 18 × 20 × 22 mm3 were in the inlet area or trabecular area and were removed through the tricuspid valve (Figure C). The remaining two myxomas measuring 6 × 8 × 8 and 8 × 8 × 8 mm3 were in the outlet area and were removed through a right ventricular outflow tract incision (Figure D). From the left ventricle, two myxomas (13 × 15 × 15 and 14 × 15 × 16 mm3) attached to the apical portion were removed through a left ventricular apical incision (Figure E,F). In total, eight myxomas were excised from the patient (Figure G).\nDuring the operation, the aortic cross-clamp time was 110 min and the cardiopulmonary bypass time was 146 min. The patient was in need of assisted mechanical ventilation for 15.55 h. Patient 1 remained in the intensive care unit for 20.80 h and had a postoperative stay of 10 days.\nThe patient’s recovery was uneventful. A postoperative echocardiograph indicated that no myxomas remained in any of the cardiac chambers. Follow-ups were performed at 3, 6, and 33 months after surgery. In each follow-up, echocardiography indicated no myxomas. The patient was evaluated as being in New York Heart Association functional class I.\nBeijing Ginkgo Wheat Biotechnology Co., Ltd, Beijing, 100084, China, provided genomic sequencing of the patient’s DNA in the PRKAR1A gene. The results indicated the presence of a c.491_492delTG mutation (Figure A). The genomic DNA was isolated from the peripheral blood lymphocytes and amplified by PCR using standard methods with the primers listed in Table . The gel purified PCR products were then sequenced in both directions on an automated sequencer (ABI 3700; GinkgoWheat Co. Ltd, Beijing, China). Nucleotides were numbered in accordance with the reference sequence for PRKAR1A (GenBank accession no. NG_007093.3). |
A 40-year-old Caucasian man with a background of severe seropositive RA affecting his shoulders, wrists and left hip was worked up for an elective total hip replacement (THR). He had a medical history of RA, diagnosed 14 years previously, for which he had been taking adalimumab (two weekly injections) for seven years, methotrexate 20mg weekly, salazopyrin 1g twice a day and prednisolone 1mg once a day. He had no other medical conditions and no family history of cancer. He was a former heavy smoker (quit four years ago, 40 pack-years) and drank 24 units of alcohol per week. On admission for his left THR, pre-operatively it was discovered that his entire left leg had been swollen for three weeks. He reported no other symptoms, including systemic symptoms of weight loss or night sweats. The operation was cancelled. An ultrasound scan of the left lower limb showed no deep vein thrombosis, but multiple abnormal looking lymph nodes in the groin were seen. He went on to have a computed tomography (CT) scan of his thorax, abdomen and pelvis. This demonstrated extensive lymphadenopathy affecting axillae, inguinal regions, mediastinum and retroperitoneum. The lymph nodes were particularly bulky in the left axilla, retroperitoneum and left inguinal regions. The CT scan also showed a left nipple mass.\nOur patient was referred to a hematologist and a breast surgeon for specialist assessment. The history was as noted above. On examination, added to the swollen left leg, bilateral axillary lymph nodes were palpable which were more bulky on the left, a 5 × 3cm hard left groin lymph node was palpable and a nodular grape-like pink lesion 2cm in diameter was seen over the left nipple. There was no hepatosplenomegaly palpable. Our patient’s adalimumab injections were stopped permanently after consultation with a rheumatologist. Our patient underwent core biopsies of the left nipple lesion, left axilla, left groin and bone marrow biopsy. Histology and immunostaining studies showed the left nipple lesion was an intermediate grade invasive ductal carcinoma of the breast with no lympho-vascular invasion. The groin biopsy showed a relatively homogeneous lymphoid cell population with mainly small lymphocytes demonstrating atypia suspicious for lymphoma. Test results revealed the cells were CD20+, CD5 negative, B-cell lymphoma (bcl)-6 positive and bcl-2 positive, in keeping with a B-cell lymphoma. The bone marrow biopsy was essentially normocellular except for a single paratrabecular cluster of lymphoid cells containing CD20+ B cells and CD3+ T cells suspicious for a follicular lymphoma. Fluorescence in situ hybridization (FISH) carried out on the groin lymph node core biopsy sample was inconclusive, with inconsistent translocations of bcl-2 and many cells showing an amplification of part of chromosome 18, the overall significance of which was uncertain, but could not confirm or exclude that this was a follicular B-cell lymphoma. The core biopsy of the left axilla sampled three separate lymph nodes which contained metastatic adenocarcinoma of the breast.\nOur patient went on to have a fludeoxyglucose (FDG) positron emission tomography (PET)-CT scan from the base of the skull to the mid-thighs. This showed a focal intense uptake in the left breast in the region of the known breast cancer, and increased uptake patterns consistent with extensive nodal disease above and below the hemidaiphragm, particularly bulky in the retroperitoneum and left inguinal region, consistent with the original CT scan. Increased uptake in the axilla and supraclavicular regions was seen, but it was not possible from the PET-CT scan to assess whether they represented nodes from lymphoma or metastatic breast cancer. All these results were discussed at the hemoncology and breast multidisciplinary team meeting and a decision was made to proceed with a left mastectomy and axillary clearance. The histology of this showed a 22mm low-grade invasive ductal carcinoma with prominent lymphatic invasion and numerous microcalcifications with clear excision margins and no evidence of Paget’s disease of the nipple. The tumor tested positive for estrogen receptor (8/8) and positive for progesterone receptor (6/8), but negative for human epidermal growth factor receptor 2 (Her-2). The left axillary contents contained 20 lymph nodes, five out of 20 nodes contained metastatic breast adenocarcinoma, all the others contained lymphoma. There was one lymph node, half of which was involved with metastatic breast cancer, and the other half with lymphoma (Figure\n). Our patient went on to have adjuvant chemotherapy to cover the breast cancer and lymphoma consisting of a regimen of fluorouracil, epirubicin and cyclophosphamide (FEC) chemotherapy with rituximab and thromboprophylaxis with enoxaparin 40mg subcutaneously once a day. |
A 28-year-old man presented at a traumatic surgery unit in an outlying hospital, where an external rotation of the left leg and a patellar dislocation were detected. Examination also revealed clinical signs of a neurofibromatosis-1 (Figure ). The patient had stumbled doing his work as a cook and had fallen on his flexed left knee. Conventional radiography of the knee demonstrated an osteochondral flake near the medial patellar margin, whereas the femoral fracture remained unnoticed (Figure ). After reposition of the patellar dislocation, the joint was stabilized with an orthosis, and the patient was sent home.\nTwo days later, because of increasing pain and swelling of the left knee, his family doctor arranged further radiographic examinations with MRI and CT scans, revealing the Hoffa fracture. The patient was then referred to a medical center for traumatic surgery. Surgical treatment consisted of diagnostic arthroscopy, open reduction of the fracture, and internal fixation with three 40-mm headless compression screws (Figure ). The screws were placed in posterior to anterior and caudal to cranial directions. The anterior horn of the lateral meniscus was fixed to the joint capsule using FiberWire 2-0, and the ruptured retinaculum was fixed to the medial patellar margin with two Mitek anchors. A 3 × 3 cm tumor close to the articular capsule turned out to be a lipoma and not, as initially suspected, a neurofibroma.\nAfter surgery, the knee was stabilized with a knee orthosis, and partial weight-bearing was prescribed for 10 weeks. Fracture healing was regular. In <4 months after the accident, the patient returned to work without any orthopaedic therapeutic appliances. In this case, the occurrence of a femoral fracture after a low-velocity trauma was probably influenced by reduced bone quality because of an underlying NF1, but NF1 did not influence fracture healing. Two years after the operation, the range of motion of both knees was unlimited, and no osteoarthrotic changes were noticed. |
A 22-year-old woman was transferred to the Oral and Maxillofacial Department for facial pain, oedema, and double vision, following extraction of the upper right third molar tooth three days earlier. The patient had initially visited an ear-nose-throat specialist because of diffuse pain of the midface a week previously. They had recommended a dental examination of the upper right molars and premolars. The general dental practitioner had decided to extract the patient's upper right third molar tooth, which was in close contact to the inferior wall of the maxillary sinus, assuming that the symptoms were odontogenic.\nThe general dental practitioner had prescribed amoxicillin postoperatively and advised on oral hygiene. However, the pain had gradually deteriorated after the extraction. The patient had been hospitalized for generalized facial oedema and pain in a regional hospital but was transferred to our institution twenty-four hours later, when she developed double vision due to paralysis of the right lateral rectus muscle. The patient's medical history included diabetes mellitus type I, which was poorly controlled. On clinical examination, she presented slight hemifacial oedema of the right side, inward gaze of the right eye because of paralysis of the abduscent nerve (VI cranial nerve), as can be seen in Figures and . Neurological examination also revealed hypaesthesia of the area of distribution of the maxillary nerve (2nd branch of the V cranial nerve). The right side of the palate was red with an ulcer of 1 cm diameter, near the second upper molar tooth ().\nGenerally, the patient was an undernourished young woman, with normal vital signs, and unwilling to provide information regarding her diabetes or the reason for dental care. Undertaken investigations at admittance included blood count, serum electrolytes, urea, kreatinin, glucose, and C-reactive protein. These revealed elevated blood glucose and mild dehydration. Furthermore, glycated haemoglobin (glycohemoglobin, HbA1c) was raised to 12.3%, with the normal range between 4–5.9% (). Opthalmological examination showed palpebral oedema of the right side, no lesions of the retina or optic nerve, but diplopia at the right outward gaze, which confirmed the paralysis of the right lateral rectus muscle.\nComputerised tomography revealed thickening of the mucosal lining of the paranasal sinuses. Further imaging with magnetic resonance imaging also showed involvement of all paranasal sinuses of the right side, however, without involvement of the central nervous system (). A biopsy of the palatal ulcer showed fungal infection by species of Mucorales (). The patient was initiated with intravenous antifungal agents (amphotericin B 300 mg qd and posaconazole 200 mg tid), and three days later she underwent subtotal right maxillectomy and reconstruction with an obturator. Thereafter, she continued the antifungal treatment, presenting gradual improvement. The paralysis of the right lateral rectus muscle progressively recovered, so the patient was dismissed from the hospital 2 months later, free of any infection, and has recently returned for definitive reconstruction (). During her hospitalization, our patient was also accustomed to more effective blood glucose management (). |
A 31-year old female patient attended to rehabilitate her maxillary right canine site. Her temporary canine was lost recently; the site was edentulous because the deciduous canine was impacted. She was wearing a provisional appliance and wished a fixed solution. Radiographic examination showed that the impacted canine was close to the alveolar ridge (Fig. ). For esthetic reasons, the patient vigorously refused to consider the orthodontic path prescribed by previous practitioners. The classical surgical approach was then explained; it consisted in removing the impacted canine, grafting the area and placing an implant after 6 months of healing and again waiting for the same amount of time. She was desperate for a shorter and less invasive solution.\nTo meet the needs of the patient, an alternative non-invasive protocol was then proposed; it was relying on a previous case that has been successful during 6 months until the canines were removed [,]. It was stressed that would this treatment fail, she would go for the conventional way at no additional cost. The patient accepted to cope with the risk.\nA large diameter tapered implant was planned (NT Osseotite, Ø 5 x 15 mm, 3i, Palm Beach Gardens, FL, USA). The impacted site was drilled following the manufacturer recommended drilling sequence, i.e. with the Ø 3.25 drill (Fig. ), the Ø 4 mm and Ø 5 mm. The Ø 6 mm drill was also used over the coronal half of the osteotomy; the aim was to hope keeping away the dental implant from direct contact with the root of the impacted canine. The coronal portion of the crown was totally removed and the implant was placed (Fig. ). Primary stability was achieved at implant seating but the palatal side was left with a bone defect; it was then filled with Bio-Oss® (Geistlich AG, Switzerland), a bone substitute of bovine origin. The gingiva was sutured over the implant.\nA 80-year old men attended with a failing mandibular tooth-supported prosthesis. The panoramic radiograph showed that all teeth needed extraction; in addition an impacted premolar was found (Fig. ). The impacted premolar was horizontal and the level of impaction was classified as level C [], i.e. the crown of the impacted tooth was beneath the root apices of the adjacent teeth. Impaction was not associated with a pathological image on the CT scan sections. The patient was seeking a global prosthetic solution in the mandible.\nImplant simulation showed that there was no way for the implants to avoid encroaching upon the impacted premolar. It was explained to the patient that the conventional treatment would require extraction of all the remaining teeth, removing invasively the globulous impacted premolar and grafting the created major bone defect. Implants would be placed after a healing period of 6 months. In addition, wearing a temporary prosthesis was strongly dissented for at least 8 weeks in order to protect the grafted sites. The patient asked for a shorter and less invasive alternative.\nA non-invasive 2-step solution was therefore discussed. It was proposed to extract all teeth but the canines and place 5 implants; 2 of them would be encroaching upon the impacted premolar in sites WHO # 32 (ADA # 23) and # 34 (ADA # 21). During the 3 months required to achieve implant stability, the canines would support a provisional bridge. At the 2nd-stage surgery, the canines would be extracted and 2 more implants would be placed in the extraction sites. The provisional bridge would be then further prepared to rely on the 5 integrated implants and the 2 newly placed ones.\nEventually, 3 out of the 7 implants did encroach upon the impacted premolar; all achieved primary stability. Implant at site WHO # 34 (ADA # 21), crossed the premolar root (Fig. ), its apical extremity was in contact with bone. At site WHO # 33 (ADA # 22), the implant apex was kept within the impacted crown without contacting bone (Fig. ). Implant at site WHO # 32 (ADA # 23) had its distal side in contact with the cuspidal edge of the crown (Fig. ). Implants were Osseotite Certain Ø 4/5 x 13 mm, full Osseotite NT Ø 5 x 11.5 mm and Ø 4 x 13 mm, respectively.\nA 85-year old women attended to rehabilitate her atrophic edentulous maxilla. The root of an ankylosed impacted canine was found outcropping the crest on the right side. Bone grafting has been previously performed to receive implants (Fig. ). The angulated root canine was occupying a position of strategic importance for implant placement. It was decided to maintain it in order to host an encroaching implant with sufficient primary stability. Implant simulation showed that at least 50 % of the implant surface would be in contact with bone. Nine implants were placed in position WHO # 11, 12, 13, 14, 16, 17, 21, 25, 26 (ADA # 2, 3, 5, 6, 7, 8, 9, 13, 14) and left to heal in a submerged way for 6 months (Fig. ). |
In 2006, a 22-year-old man was diagnosed with Crohn disease (CD). He first came to our unit 4 years later. At that time, he had stopped taking the medicine independently and presented with gastrointestinal symptoms. Mesalazine and azathioprine were initiated in 2010 at an age 26 years. After 2 years of treatments, the patient's gastrointestinal symptoms were uncontrolled, and an examination by colonoscopy revealed strictures in the descending colon (). Due to worsening CD, treatment with an anti-TNF-α agent, infliximab, was initiated. When the patient's treatment began, the tuberculin skin test was negative, chest radiography and interferon gamma release assay were normal, and no evidence of tuberculosis was found. After receiving the infliximab treatment (intravenous infliximab 5 mg/kg every 8 weeks), the patient began to experience symptom relief. Infliximab was injected 8 times, and the patients showed a very good response to the treatment, as his CD activity index indicated remission. At that time, the patient had a chest X-ray as part of regular outpatient follow-up. The identification of an abnormal pulmonary density was, therefore, an incidental observation. The patient looked healthy and had no respiratory symptoms, such as cough or sputum. The tuberculin skin test was negative, and the interferon gamma release assay results were normal.\nChest X-ray and computed tomography displayed clustered small nodules in both lobes and multiple enlarged lymph nodes (). The results of a fungus culture, an acid-fast bacilli stain, a tuberculosis polymerase chain reaction (PCR) assay, and a bacterium culture in bronchial aspirate were all found to be negative. Endobronchial ultrasound-guided transbronchial needle aspiration of the subcarinal lymph node was performed, and histology demonstrated noncaseating granulomatous lesions (). Cancer and tuberculosis may, like sarcoidosis, accompany mediastinal lymphadenopathy. That possibility was examined by using acid-fast staining, PCR, and cytology of the lymph node. All the results were negative. The serum angiotensin-converting enzyme level was normal (50 U/L; reference range, 20–70 U/L). At this stage, the diagnosis of sarcoidosis was made, and treatment with infliximab was discontinued. Lung lesions were improved 5 months after infliximab had been discontinued. Therefore, the anti-TNF treatment was thought to have triggered pulmonary sarcoidosis in this patient with IBD. Nevertheless, his paradoxical inflammations were not severe, and he wanted to continue anti-TNF-α therapy for his refractory CD, so infliximab was continued cautiously. Subsequently, the patient's lung lesions spontaneously regressed to normal (). Since that time, pulmonary sarcoidosis has not recurred over 19 dosing periods spaced 8 weeks apart. |
A 51-year-old man was referred to our hospital, with a dumbbell-shaped nodule measuring 40 mm in the right upper lobe of the lung (Fig. ). He was a current smoker and also had hypertension, diabetes mellitus, and bronchial asthma. Imaging findings suggested the possibility of a lung cancer, so transbronchial biopsy was performed. However, definitive diagnosis was not able to be obtained from the biopsy specimens. Bacterial culture of bronchial lavage fluid also yielded negative findings, including for tuberculosis. After eight months of observation, computed tomography showed that the tumour had slightly increased in size. Surgery was therefore planned to resect the tumour and reach a definitive diagnosis. Because of the size of the tumour, a lobectomy of the lung was scheduled with the patient's consent.\nThe patient was placed in the left lateral decubitus position under general anaesthesia. We then made a 1.2-cm skin incision for insertion of a flexible thoracoscope and endoscopic autosuturing devices in the seventh intercostal space along the midaxillary line. We used a surgical scalpel only for cutting the dermis, and then gently separated muscles with forceps to access the thoracic cavity. Next, we made a 1.2-cm skin incision for the main port in the fourth intercostal space along the anterior axillary line for endoscopic forceps and a vessel-sealing device. In addition, a 5.5-mm skin incision in the third intercostal space along midaxillary line and a 3.5-mm skin incision in the fifth intercostal space along the posterior axillary line were placed to achieve effective visual overview of the surgical site.\nThe surgical procedure was basically the same as for conventional MVATS (Video , Supporting Information). We completed a typical right upper lobectomy and lymph node dissection. The lobectomy took approximately 3 h and there was a small amount of bleeding.\nTo remove the specimen, we created a 3-cm longitudinal incision 1 cm below the xiphisternal joint. We subsequently incised the linea alba, and bluntly reached the right chest cavity using the index finger. The specimen was placed inside a tissue bag and removed through the subxiphoid incision (Video , Supporting Information). An intercostal nerve block with ropivacaine hydrochloride hydrate was performed at the end of the surgery.\nIntravenous patient-controlled analgesia (fentanyl citrate) was used for 18 h post-operatively instead of epidural analgesia. Numerical rating pain scale (NRS) at rest has consistently been 1/10 since 1 h post-operatively. The patient followed an uneventful post-operative course. The chest drain was removed 18 h post-operatively. He was discharged without any wound complications (NRS, 0/10).\nThe pathological diagnosis was tuberculoma, and antibacterial drugs were administered. The patient has not complained of any neuralgia or paraesthesia (NRS, 0/10) at the time of writing, six months post-operatively (Fig. ). |
In June 2016, a 9-year-old girl (elementary school student of Caucasian race, weighing 42 kg) was referred for consultation with an interdisciplinary team in the Vascular Anomalies Center of Guarneri Clinic (Rome, Italy) to address a painful and progressively increasing mass in the right thigh. The consultation team included vascular surgeons, interventional radiologists, hematologists, oncologists, plastic surgeons, and pathologists.\nThe patient had an unremarkable medical history.\nAt clinical examination, the girl showed functional impairment of the right knee (limited flexion of 30%), muscle contracture, and localized pain (). In the days before her hospitalization, the reported daily mean visual analog scale (VAS) score for chronic pain was 7 ± 1. Neurologic examination revealed normal patellar tendon reflex, full strength of the surrounding musculature, and normal superficial and deep sensitivities. Dermatological examination revealed normal skin characteristics.\nIt was reported that no therapeutic procedure had been carried out previously, and the pain symptoms had been controlled with pain relievers, when necessary.\nLaboratory blood tests for coagulation were performed, according to the high rate of variations in coagulation states that are found in patients with vascular anomalies. No abnormalities were observed in our young patient.\nIn addition to clinical signs, diagnosis of FAVA was based on both ultrasound and magnetic resonance imaging (MRI) examinations. Both imaging modalities showed a complex mass of 11 cm × 8 cm × 8 cm in the right thigh, a finding compatible with FAVA malformation, located in the vastus medialis muscle and marginally in the rectus femoral muscle. The T1-weighted MRIs showed heterogeneous and hyperintense signal related to the fat component (), and the injection of intravenous contrast clearly revealed the malformed venous component. The solid mass was found to have partially replaced the normal vastus medialis muscle fibers, with fibrofatty overgrowth and varied appearances of clusters of thick-walled muscular vessels; multiple soft-tissue planes were found to be involved as well, showing hypoechoic intralesional clots or development of phleboliths, with low-flow venous parameters that were also barely detectable by Doppler sonography.\nFollowing discussion and consensus by the interdisciplinary team, differential diagnoses of other commonly confused vascular lesions (i.e., venous or arteriovenous malformations or vascular tumors) were ruled out.\nThe primary end point of this patient’s treatment was radical FAVA treatment. The secondary end points were pain relief (assessed by VAS) and recovery of functional impairments [,].\nPrevious clinical experiences in the literature describe the frequent use of sclerotherapy and percutaneous cryoablation procedures for FAVA [,,] and the high risk of blood loss and nervous system damage related to primary surgery in FAVA []. Thus, the multidisciplinary team proposed an original hybrid treatment strategy of primary percutaneous ethanol embolization of the FAVA and a secondary surgical excision of the mass.\nInformed consent statement: Written consent was obtained from both parents of the patient, because she was a minor at the time of hospitalization.\nInstitutional review board statement: The Institutional Review Board of Guarnieri Clinic provided approval for this study (IRB No. 19/1721). |
A 6-month-old girl presented to our clinic with bilateral hip joint pain, which began 1 year prior to her admission. The pain drastically affected her physical activity and was more evident in the morning. No other symptoms were presented. She then received Plain MRI and enhanced scanning of the hip joints, which revealed a small amount of effusion within the bilateral joints. Both colored ultrasound examination of retroperitoneum and Plain CT scanning of the chest and abdomen showed no evident abnormalities; thus, no special treatment was given to the child at that time.\nThe child still complained of recurrent hip pain, and her parents treated her with physical therapy, with no significant improvement. In September 2019, the girl was admitted to our clinic for the first time, and the Plain MRI and enhanced scanning showed a small amount of bilateral hip joint effusion. Cytological examinations of the bone marrow were normal. However, her parents treated the child with irregular traditional Chinese medicine and still did not see any significant improvement.\nIn December 2019, the girl was admitted to another hospital and diagnosed with JIA. At that time, EBV-DNA quantification was normal. From then on, she received oral administration of methotrexate (MTX, 14 mg/m2, once per week), methylprednisolone pills (0.75 mg/kg/d), and subcutaneous injections of Recombinant Human Tumor Necrosis Factor-α Receptor II; lgG Fc Fusion Protein (0.8 mg/kg, once per week). The pain was relieved after 4 weeks of continuous treatment. Two months later, the child did not follow-up and stopped all treatment. On May 12, 2020, the pain in the bilateral hip joints became more severe and was accompanied by occasional headaches. The patient was then admitted to our hospital on May 14 for further examinations. No other symptoms were present ().\nThe girl was diagnosed with immune thrombocytopenia (PLT: 87 × 109/L) in 2017. At that time, cytological examination of the bone marrow showed active bone marrow hyperplasia, maturation disorders of megakaryocytes, and diffused distribution of platelets. The platelet count returned to a normal level after venous infusion of immunoglobulin. No history of organ transplantation or repeated infection was reported. In addition, no remarkable family history was reported.\nUpon physical examination, her vital signs were in a normal range with a body weight of 16 kg. The examination of her chest and abdomen showed no obvious abnormalities. No swelling or pressing pain was found in the joints of limbs. However, the 4-shaped sign showed positive results.\nDuring routine laboratory and blood examinations: C-reactive protein (CRP), IL-6, serum ferritin, ASO, and rheumatoid factors (RF) were all within a normal range. Autoimmune antibody (ANCA, ANA, ANA spectrum, PR3-IgG, MPO-IgG, GBM-IgG, and anti-CCP) and pathogenic examination (tuberculosis antibody, tuberculosis infection-specific T cells, mycoplasma antibody, HIV, and fungi) were all negative. EB-VCA-IgG and EB-NA-IgG levels were elevated; EB-VCA-IgM and EB-EA-IgG levels were normal. EBV-DNA quantification showed normal results. Examination of HBsAg, HBeAg, and HBcAb showed positive, and serum quantification of HBV-DNA was 3.38 × 107 IU/ml. The levels of immunoglobulins (A, E, G, and M) and complements C3 and C4 were all within a normal range (). Lymphocyte subsets were normal.\nColored ultrasound examination of the retroperitoneum showed no masses or abnormalities. Plain CT scanning of the lungs showed multiple nodules and stripe-shaped shadows bilaterally in the lungs. The largest was 0.63 × 0.74 cm. Plain and enhanced MRI scanning of the knee and hip joints revealed that the synovium of the bilateral knee and hip joints were slightly thickened and enhanced, with a small amount of effusion in the joints. Plain and enhanced CT scanning of the lumbar vertebrae showed the adnexa area left to the L2 vertebral body had osteolytic bone destruction and was accompanied by soft tissue masses. Plain and enhanced MRI scanning of the skull, pituitary, and spine showed nodular lesions at the parasellar and meninges of the right parietal lobe and bone mass destruction of the L2 vertebral body and left vertebral pedicle (). Electromyography (EMG) of bilateral lower limbs showed no abnormalities in nerve conduction or muscle contraction.\nBone marrow biopsy examination showed active bone marrow hyperplasia, substantially reduced erythroid hyperplasia, and elevated lymphocytes. Biopsy of the inguinal lymphocytes revealed that the microscopy and immunohistochemistry findings agreed with the changes of reactive hyperplasia of the lymph node.\nSo far, the diagnosis of the child is still not clear. Two biopsies were performed within the L2 vertebral lesion tissues. The first biopsy was not diagnosed because EBER in-situ hybridization was not performed. The diagnosis was not made until after the second biopsy of a positive EBER in-situ hybridization was performed ().\nThe girl's pathological examination showed EBER (+); thus, she was diagnosed with EBV-SMT. However, she was not previously infected with HIV or had any organ transplantation. In addition, the girl had no history of immunodeficiency-related diseases. In order to clarify the possibility of undiagnosed immunodeficiency or other causes, blood was obtained for whole-exome sequencing. The results showed no related pathogenic genes.\nThe patient was admitted to the hospital for arthritis. The imaging examinations after admission showed multiple lesions in the skull, lungs, and vertebral body. Other hematological tumors or Langerhans cell histiocytosis were considered during the diagnosis, which was not in agreement with the pathological and other examination results. Biopsy of the L2 vertebral body was then performed to clarify the diagnosis. Finally, the in-situ hybridization of the tumor of the lumbar vertebrae suggested non-HIV/transplantation-related EBV-SMT. The patient then received surgery (lumbar lesions resection + fusion bone grafting and internal fixation) without chemo or radiotherapy. The hip pain improved except for occasional headache. The patient is still under regular follow-up now. |
A 28-year-old woman, gravida 3 para 1, had a medical termination of a miscarriage at seven weeks, with no dilation and curettage, in 2008. In 2015, a baby was delivered by caesarean section in the breech position, weighing 3900 g. She had no significant past medical history, and her antenatal care had been uneventful. On August 9, 2018, at 19:15, she was admitted to our hospital due to a pregnancy of 9+ months and irregular contractions for 4+ hours. Periodic uterine contractions occurred every 6–8 min. The patient was not accompanied by abdominal pain or vaginal bleeding and had intermittent term after contractions. Clinical examination showed that her body temperature was 36.7 °C, blood pressure was 102/65 mmHg, pulse rate was 100 bpm, and oxygen saturation was 100%. Blood tests showed mild leucocytosis (16.61 × 109/L), normal platelet count, normal coagulation test, and haemoglobin of 102 g/L. Vaginal examination showed the cervix was tightly closed; no vaginal bleeding or fluid was found. The ultrasonography indicated that the foetal head was located above the uterine cavity, the foetal size was consistent with the gestational age, the placental position was normal, and the scar thickness of the previous caesarean section was approximately 0.2 cm. Uterine contractions declined after admission. During admission, the patient was clinically and biochemically stable, and daily cardiotocograms showed a reassuring foetal heart rate pattern. Because of the patient’s progressive anaemia (blood tests revealed a slow decline in haemoglobin to 93 g/L, 87 g/L) and sudden increasing abdominal pain, ultrasound was used but did not show ruptured abdominal fluid. An urgent laparotomy was performed and revealed a massive haemoperitoneum caused by the rupture of the uterine posterior wall. A haemoperitoneum with approximately 1 liter of blood was recovered. The lower uterine segment was intact and not ruptured. A boy with a body weight of 2900 g was delivered. Apgar scores were 9 at 1 min and 10 at 5 min. The amniotic fluid was clear, the placental was completely delivered, and no placental abruption occurred. The patient’s uterus was closed in two layers. After removing the blood and clots, a 12 cm-long tear in the posterior wall and active bleeding from the uterine rupture were found. Uterine tissue adhered to the bowel (see Fig. ). After separation of the adhesions between the bowel and the uterine wall, two layer of uninterrupted stitches restored the uterine integrity, and interrupted stitches closed the mesentery defect (see Fig. ). It was suspected that future conceptions would be dangerous, so bilateral tubal ligation was performed at the same time, under the permission of the patient and the patient’s family member. Our patient’s uterine and pelvis showed no abnormalities and, particularly, no evidence of endometriosis. Inspection of her liver showed no rupture. The placenta was sent for pathological examination. Syntocinon (oxytocin) (Ma an Mountain Company, China, SFDA approval number: H34020474) was administered intravenously. The operation was uncomplicated, and the estimated total blood loss was 2500 ml. Ten units of blood and 400 ml of blood plasma were transfused. The patient’s postoperative course was regular, and she was discharged 6 days later. |
A woman in her 70s was referred to our clinic complaining of redness and teariness in her left eye. She recounted that the sap of an E. trigona plant at home (Fig. ) had splashed into her left eye when she had cut the plant. Two days after the incident, she experienced pain and visual problems and visited a clinic in her neighborhood, where the physician immediately referred her to our hospital. She did not have a history of allergies and did not have any contact with chemicals. Her medical history included a cataract surgery performed 3 years earlier, and she was using a topical instillation for dry eye. Her last best-corrected visual acuity (BCVA) was 0 in terms of the logarithm of the minimum angle of resolution, and she had no other history of ophthalmological or medical problems.\nOn general examination, her condition was found to be stable. Slit-lamp examination revealed hyperemia with a thick and clouded cornea in her left eye (Fig. ). There was hypopyon inside the anterior chamber, but no keratic precipitates were observed (Fig. ). Corneal epithelial defects were not detected after fluorescein staining (Fig. ). The BCVA was 2.0 as measured under photopic conditions. The intraocular pressure was 17 mm Hg, as measured using a noncontact tonometer, and the central corneal thickness (CCT) was 812 μm, as measured using a contact ultrasonic pachymeter. The fundus of the left eye could not be seen because of the clouded cornea and intraocular inflammation; however, ocular ultrasound examination revealed no retinal detachment or vitreous opacity. There was no sign of general inflammation in the blood test results.\nWe started treatment with topical 1.5% levofloxacin and 0.1% dexamethasone 4 times a day. She responded well to treatment showing a reduction in hypopyon and corneal edema the next day. The measured CCT was 744 μm. She experienced no pain, her BCVA had improved to 1.0, and intraocular pressure was 14 mm Hg. A week later, the CCT was 621 μm, and BCVA increased to 0.22, despite the presence of intraocular cells (grade 3+). Corneal edema and Descemet membrane folds were still present (Fig. ), but no corneal epithelial defect was observed upon fluorescein staining (Fig. ). We were not able to assess the corneal endothelial cell density (ECD) by noncontact specular microscopy owing to corneal edema. The blood samples collected upon arrival tested negative for viral infections including cytomegalovirus, varicella-zoster virus, and herpes simplex virus. Therefore, we continued the topical instillation at the same frequency of administration for another week.\nTwo weeks after the injury, the corneal edema recovered with the CCT reaching 547 μm. The cornea became clear, and the BCVA recovered to 0.097. Hypopyon disappeared completely with a small number of intraocular cells floating in the anterior chamber (grade 1+). The ECD remained at 3,280 cells/mm2, the coefficient of variation of the cell area (CV) was 45%, and the proportion of hexagonal cells (6A%) was 44%. Therefore, the frequency of drug administration was reduced to 3 times a day for another week when the BCVA reached 0 and the CCT was 519 μm. The frequency of topical instillation of 1.5% levofloxacin and 0.1% dexamethasone was subsequently decreased to twice a day for another week. The CCT reached 528 μm, and the ECD remained at 3,233 cells/mm2 1 month after the injury; the CV was 46%, and the 6A% was 25%. The anterior segment showed no intraocular inflammation with a completely clear cornea (Fig. ), and palisades of Vogt were observed around the corneal epithelium (Fig. ). Hence, topical instillation of steroids and antibiotics was discontinued. The time-dependent changes in CCT and BCVA are summarized in Figure .\nA follow-up examination 6 months after the injury revealed a completely transparent cornea with good visual acuity (BCVA; 0); uveitis did not recur during the follow-up period. The ECD remained at 3,244 cells/mm2, the CV was 26%, and the 6A% was 66%, indicating no endothelial damage or complications. |
A 73-year-old man underwent uncomplicated phacoemulsification cataract surgery of his right eye under topical anesthesia with temporal corneal incision. He had no past ocular disease or trauma history. He had a medical history of diabetes and mild bronchial asthma. The patient was also taking oral medication for diabetes, but he was not taking any medication for bronchial asthma. His preoperative corrected vision was 20/40, and his intraocular pressure was 17 mmHg. On postoperative day one, his corrected visual acuity was 20/200, and his intraocular pressure was 16 mmHg. The patient's cornea was edematous with Descemet's folds. The anterior chamber was deep with cells 4+. He was instructed to use ofloxacin eye drops and 0.12% prednisolone eye drops every two hours for one week, and then four times a day thereafter. The cornea was still edematous when he visited our clinic one week later. Three weeks after surgery, his visual acuity was 20/100 and his intraocular pressure was 19 mmHg. The patient complained of a foreign body sensation and visual disturbances. The corneal edema had improved but the slit lamp exam revealed DMD at the superonasal area (). There was no direct trauma to the superonasal cornea during the surgery. Because the size of the DMD was small and the location was peripheral, we decided to observe the patient for a follow-up period without surgical intervention. Postoperative use of ofloxacin eye drops and 0.12% prednisolone eye drops was maintained four times a day. During the follow-up period, an intracameral air injection was not needed because the size of the DMD decreased and the patient's vision improved. Two months after the surgery, the DMD had completely reattached and the patient's corrected visual acuity had improved to 20/30. Ofloxacin eye drops and 0.12% prednisolone drops were maintained for two more weeks. Three months later, the cornea was clear, and his corrected vision was 20/20. |
A 65-year-old white woman was hospitalized for a right hypochondrium colicky pain radiating to the ipsilateral subscapularis region. Her pain was not related to food and posture. It appeared during the previous week and persisted despite nonsteroidal anti-inflammatory medication. A clinical examination showed that her abdomen was swollen in the right iliac fossa and the presence of voluminous palpable mass that was hard, fixed to the deep layers, and sore to touch.\nAn abdominal ultrasound (US) examination revealed her liver with gallbladder stones, a heterologous formation in her right iliac fossa with internal calcification, which compressed the iliac vessels, and a bilateral hydronephrosis more marked on the right than on the left.\nLaboratory tests revealed significant alterations (Table ). Her blood pressure was above 180 mmHg. A chest X-ray showed the presence of multiple metastases placed in both lung fields. A total body computed tomography scan (CT) without contrast confirmed the presence of lung metastases, bilateral hydronephrosis, and a mass in her right iliac fossa invading her ureter and her iliac artery.\nThere was also a mass in the right iliac mass but with a reduced size in the left iliac fossa invading her psoas muscle. The internal structure of the masses was homogeneous, apart from the centrally located amorphous high-attenuation areas. There was no fat inside the lesion.\nShe underwent hemodialysis and infusion therapy with rasburicase (Fasturtec ®; Table ).\nPrompt dialysis is necessary when treatment fails to normalize electrolytes or establish urinary flow. Hemodialysis removes excess circulating uric acid. Before the dialysis our patient was hyperuricemic and oliguric. After the dialysis, we observed that she developed diuresis, associated with the consequent resolution of hyperuricemia, hyperazotemia, hyperphosphatemia, and normalization of electrolytes.\nAfter 10 days she underwent surgery. An exploration of her abdominal cavity detected a retroperitoneal mass in her right iliac region which involved the last ileal loop besides compressing the iliac vessels and infiltrating her ureter. Another mass in her left iliac fossa caused a compression of the left iliac vessels and ureter without infiltration. As a result of the inoperability of the mass in her right iliac region, an ileotransversostomy was performed with enucleation of the left mass and cholecystectomy.\nAn histological examination performed on the mass diagnosed an ESOS infiltrating her psoas muscle, her internal iliac artery, and her bladder wall. On macroscopic examination the mass had a hard consistency, was whitish-gray in color, and there were large central calcified parts and areas of hemorrhagic-necrotic tissue. The mass was osteoblastic histological type, consisting of osteoblasts producing an osteoid material. The lesional cells showed great cytological atypia, high mitotic activity, and permeative growth pattern (Fig. ).\nThe tumor massively invaded our patient’s abdominal cavity and metastasized in both lung fields. According to these parameters, she was in a very poor prognostic subgroup, due to a very bulky and high growth rate tumor. This tumor was only partially resectable because she was over 60-years old and showed multiple metastatic lesions, multiorgan impairment, and increased alkaline phosphatase (ALP) levels. A CT scan showed large soft tissue masses with focal or massive areas of calcification and no osseous involvement. Magnetic resonance (MR) images showed intermediate signal intensity on T1-weighted images and a low-to-hyperintense signal on T2-weighted images.\nThe diagnosis of osteoblastic ESOS was confirmed by the tumor localization within the soft tissue, without attachment to bony structures, and the presence of abundant osteoid. There was no history of radiation or trauma in our patient, but there was an anamnestic finding of hysterectomy just 6 months before the diagnosis of ESOS.\nThe most common presentation of ESOS is a gradually enlarging mass that varies in size from 1 to 50 cm in diameter and does not always imply pain. Very large and bulky tumors often develop in the retroperitoneum before their detection as in our case. Thus the presence of metastatic lesions, the patient’s age, and the size were the major prognostic factors; in addition, patients with very large lesions have a worse clinical outcome. |
A 19-year-old male of Caucasian origin was admitted to our center as a polytrauma after a road traffic accident. He was previously fit and well, a nonsmoker with an alcohol intake of approximately 10 units per month. The accident, in which his motorcycle collided with an oncoming vehicle, caused him to sustain multiple significant injuries including unstable pelvic fractures and femoral fractures. He had bilateral pneumothoraces, extensive pulmonary contusion, and a splenic hemorrhage. He presented in extremis with signs of hypovolemic shock. He was intubated and resuscitated using local major hemorrhage protocols to achieve a blood pressure of 159/93, receiving ten units of packed red cells and four units of fresh frozen plasma in the emergency department.\nHe underwent an emergency laparotomy and splenectomy and was subsequently transferred to the intensive care unit, where he became increasingly hypoxic with features of adult respiratory distress syndrome (ARDS). This culminated in him receiving veno-venous extracorporeal membrane oxygenation (ECMO) from day 15 of his admission for 21 days. He returned to theater on day 21 for a massive haemothorax which required an emergency thoracotomy. After being decannulated from the ECMO circuit, he was stepped down to the general intensive care unit on day 36 and was transferred to the ward on day 55 before being discharged after a 4-month admission including a prolonged rehabilitation and recovery period.\nAfter presentation and commencement of ECMO, there was a relatively modest change in liver function tests. Alkaline phosphatase (ALP) increased from 55 to 143 IU/L between day 1 and day 6 of hospital admission and no persistent alanine transaminase (ALT) rise until after decannulation. Proceeding decannulation, there was a sequential increase in ALP peaking at 2335 IU/L on day 113. ALT rose to a lesser extent, peaking at 781 IU/L on day 52. The bilirubin did not rise above 57 μmol/L. The pattern of liver function tests is summarized in Figure . Autoantibody screen, immunoglobulins, and viral hepatitis serology were negative.\nSerial ultrasound, computerized tomography (CT), and magnetic resonance imaging excluded biliary stones and sludge. The liver on CT at day 1 showed normal liver and biliary structure (Figure ), and it was not until 10 months after the admission, the repeat magnetic resonance cholangiopancreatography (MRCP) demonstrated a multistenotic pattern of disease within the intrahepatic ducts (Figure ).\nIn the absence of significant casts within the biliary tree and no evidence to suggest biliary sepsis, endoscopic retrograde cholangiopancreatography (ERCP) was felt not to be helpful. A conservative management approach was taken, and the patient was instigated on ursodeoxycholic acid to help improve cholestasis. With bilirubin improving, the patient was closely monitored as an outpatient upon discharge for progressive liver disease and dysfunction. Repeat imaging and noninvasive fibrosis assessments were undertaken. Despite having evidence of SSC, the synthetic liver function has remained excellent, cholestasis markers have improved, and there have been no episodes of cholangitis or biliary sepsis for over 1 year. |
We report on a 7-month-old boy with Downs syndrome who was born with an unbalanced left dominant atrioventricular septal defect and aortic coarctation. Initially he underwent aortic coarctation repair. However, despite good restitution of the systemic obstruction he could not be weaned from mechanical ventilation and subsequently underwent pulmonary arterial banding, which reduced pulmonary arterial pressure to 50% of systemic pressure and the pulmonary-to-systemic flow ratio to 1.5 : 1. Also a plication operation was performed for left-sided diaphragmatic paralysis. Supportive pharmacotherapy consisted of digoxin, captopril, spironolactone, and high-dose furosemide. In addition, a gastro-duodenal tube (G-Tube) was placed for feeding because of vomiting and poor oral intake. The hospital course was complicated by more than 10 endotracheal intubations for recurrent respiratory failure, which appeared to be triggered by exacerbations of an inflammatory disease process of unknown origin. During this time the infant underwent multiple courses of antibiotic treatment. Despite multiple diagnostic efforts, the cause of the recurrent sepsis-like exacerbations could not be determined.\nThe hemodynamic status was characterized by good cardiac performance of the functionally single ventricle despite persistent pulmonary hypertension (). There was clear evidence for ongoing pulmonary vascular disease with pulmonary hypertension and need for oxygen supplementation. In this situation, stage 2 palliation (superior cavopulmonary connection, bidirectional Glenn shunt) of his single ventricle defect was considered contraindicated. Since we hypothesized that the recurrent clinical symptoms of heart failure were caused by neurohormonal and inflammatory dysfunction we started a trial of low-dose beta-blocker therapy with 0.3 mg/kg/day propranolol (0.1 mg/kg per dose) by G-Tube in accordance with the German guidelines for congenital heart defects in children [, ].\nDuring the next fever episode the infant developed signs of hepatic failure and pulmonary edema and required intubation with mechanical ventilation as well as intravenous antibiotics and aggressive diuretic treatment. Since the hemodynamic and cardiac function improved, propranolol therapy was not stopped. He recovered from this initial episode of systemic inflammatory response syndrome (SIRS) (see SIRS I in ) within 3 days. He was extubated without dyspnea and began to tolerate oral feeding. However the fever continued unabated and when we stopped antibiotic coverage to collect blood cultures he decompensated with signs of an exacerbation of the SIRS II (see ).\nFurthermore we switched the medication to the selective β\n1-blocker metoprolol. The patient stabilized quickly again but the fever continued. Since all blood cultures remained negative, we hypothesized that the source of recurrent endotoxemia may be gastrointestinal. One week later we decided to remove as many potential sources of infection as possible. We removed not only the central venous catheter but also the G-Tube. Subsequent blood cultures and the central venous catheter cultures were negative, but resistant E. coli was recovered from the jejunal end of the feeding tube. The infant improved with oral treatment with cotrimoxazole. His supplemental oxygen requirements ceased during his oxygen saturation rose to 90% and the pulmonary arterial pressure decreased to the normal range. After six months of intensive care, including 12 intubations for respiratory failure, he was discharged home. Two days later he was readmitted for fever and decreasing oxygen saturations due to bronchospasm. An etiology of the bronchospasm could not be ascertained. Despite intensive therapy with systemic and inhalational β\n2-adrenergic agonists, theophylline, and high-dose prednisolone, the bronchospasm persisted and the infant's condition deteriorated. Metoprolol was discontinued and mechanical ventilation was reinitiated. A subsequent event of pulmonary hypertensive crisis ensued and unfortunately the patient expired (SIRS III). Post mortem the results of a bone marrow aspirate showed hemophagocytosis and the diagnosis of HLH was verified by a highly elevated ferritin and soluble CD25 receptor levels. Especially the cytokine profiles confirmed the diagnosis of HLH. Epstein-Barr virus infection could be excluded by polymerase chain reaction. |
A 65-year old Hispanic male presented with complaints of chest pain and heaviness prior to the diagnosis of his disease. The patient had a history of tobacco use but had stopped smoking 20 years earlier. The patient had been otherwise healthy all of his life. Approximately 4 to 6 weeks prior to his diagnosis, he experienced an episode of chest pain and some mild shortness of breath. Initially, the patient underwent a routine chest X-ray and CT scan diagnostic procedures. Subsequently, a thorascopic biopsy was performed, and using H & E staining, tissue histology was carried out to detect the tumor development. In addition, the patient underwent a mediastinoscopy, thoracoscopy, and fine-needle biopsy to confirm the diagnosis of his disease.\nThe diagnostic evaluation revealed the presence of a stage IV malignant thymoma with multiple metastatic lesions involving the left peripheral lung and pericardium. Despite the presence of metastatic disease, these lesions are still oftentimes known to be amenable to surgical resection following treatment with induction chemotherapy. Therefore, to down- stage the tumor and to improve the patient’s chance of increased surgical resectability, neoadjuvant chemotherapy was initially employed. Since platinum with an anthracycline-based triplet or quartet regimen is currently the consensus treatment for malignant thymoma [-], this was chosen as the neoadjuvant chemotherapy in this patient. More specifically, the patient was initially administered five cycles of quartet regimen consisting of cisplatin, adriamycin, vincristine, and cytoxan. Subsequently, the patient received four cycles of cisplatin, adriamycin, and cytoxan followed by surgical resection of his residual disease. Despite the treatment with neoadjuvant chemotherapy and subsequent surgical resection the patient experienced recurrent chest disease within a year.\nIn attempt to salvage this patient, the patient underwent a course of post-operative and post-chemotherapy IMRT/IGRT-based three-dimensional radiation therapy to a dose of 7,440 cGy to the left chest wall, mediastinum, and left pericardium (Figure ). The patient was monitored for safety and efficacy using CT scans and a MUltiple Gated Acquisition (MUGA) scan prior to and after his chemoradiation therapy.\nInitial findings from the thorascopic biopsy indicated that the patient had multiple pleural-based masses with an associated small left pleural effusion. Assessment of tissue histology using H & E staining revealed a mixture of plum epithelial cells with both vesicular nuclei and distinct nucleoli and small lymphocytes indicating the presence of malignant thymoma (Figure ). Following mediastinoscopy and later a thoracoscopy, the patient was provisionally suspected of having lymphoma; however, the needle biopsy assessment showed lymph with aggregates that were negative for lymphoma. Subsequent diagnostic studies confirmed the presence of a stage IV malignant thymoma. Further assessment using a multi-slice CT scan indicated that the patient had multiple metastatic lesions involving his left peripheral lung and pericardium. In addition, the chest CT imaging revealed several mass structures with the largest size being 3.4 × 6.7 cm in the left hemithorax. Furthermore, there were also multiple mediastinal masses specifically located in the anterior mediastinal region with the largest mass size measuring 3.2 cm.\nThe initial five cycles of neoadjuvant chemotherapy with cisplatin, adriamycin, vincristine, and cytoxan was well tolerated by the patient. However, this induction therapy only resulted in reduction in the size of the tumor masses. However, a partial response was observed after the subsequent chemotherapy with four cycles of cisplatin, adriamycin, and cytoxan. The patient then underwent surgical resection of his residual disease. Despite treatment with chemotherapy and surgical resection, the patient experienced recurrent chest disease within a year. An attempt was then made to salvage the patient and he was treated with radiation therapy.\nA pre-radiation therapy chest CT imaging revealed several mass structures with the largest size measuring 3.7 × 3.0 cm in the left hemithorax (Figure A). In addition, there were also multiple mediastinal masses located in the anterior mediastinal region with the largest mass size measuring 3.2 cm. To control the disease, the patient then underwent a course of post-operative and post-chemotherapy definitive IMRT/IGRT-based three-dimensional radiation therapy to a dose of 7,440 cGy to the left chest wall, mediastinum, and left pericardium. A post-therapy chest CT imaging showed the presence of only small sub-centimeter right middle lobe pulmonary nodules with no discrete soft tissue mass or malignancy. Follow-up CT imaging of the chest 5 years after the patient’s chemoradiation therapy, revealed that the localized soft tissue thickening at the left upper lung anterior pleural space had resolved (Figure B). Seven years post-chemoradiation therapy, CT imaging of the chest also showed a resolving tumor mass and postsurgical and post-radiation changes and fibrosis (Figure C). The patient's post-radiation MUGA scan of the heart showed that the left ventricular ejection fraction was within the expected normal value of 61% 8 years after definitive three-dimensional IMRT/IGRT.\nMalignant thymomas are rare human neoplasms accounting for less than 0.5% of all malignancies. Due to their rarity, knowledge of the optimal treatment regarding these tumors is based on case reports or small retrospective series. Management of thymic carcinoma depends upon the clinical stage of the disease. Currently, surgical resection remains the mainstay of treatment for localized thymic malignancies []. However, the complete resection of the tumor is particularly important in the management of thymic carcinoma []. The resectability for stages I, II, III and IV of thymic carcinoma has been 100%, 43 to 100%, 0 to 85%, and 0 to 42%, respectively []. A study by Kondo et al. [] has shown that the 5-year survival rate is 66.9%, 29.8%, and 19.4% for completely resected, incompletely resected, and non-surgery groups, respectively.\nIn general, extensive mediastinal or lung invasion or metastasis is a frequent finding in most newly diagnosed patients. Despite complete surgical resection, over 50% of patients with advanced disease experience recurrence []. Consequently, treatment with multiple modalities such as repeat surgical resection, chemotherapy, and radiotherapy has been attempted but without general consensus on the optimal approach []. It is known that most thymic tumors are chemo- and radio-sensitive [-] and, thus, a multimodality treatment that integrates surgical resection with chemo- and radiotherapy has been advocated for advanced stages with the aim to improve both local and distal control of the disease and prolong survival [-]. In addition, preoperative (induction or neoadjuvant) chemotherapy has been successfully used to down-stage unresectable tumors for surgical resection and to prevent local and systemic recurrences [,,-].\nThe present patient was diagnosed as having an advanced stage IV malignant thymoma with multiple metastatic lesions involving the left peripheral lung and pericardium. Therefore, induction chemotherapy was employed, followed by surgery, and then post-operative definitive targeted three-dimensional IMRT/IGRT in this patient. Since cisplatin-based combination chemotherapy is effective against thymic tumors [,,], cisplatin was used in combination with adriamycin, vincristine, and cytoxan as induction chemotherapy to down-stage the tumor and to improve the surgical resectability of the patient’s unresectable tumor. This initial induction chemotherapy was found to be effective in reducing the sizes of the masses with an acceptable safety and patient tolerability; however, residual tumor was still present. Additional cycles of induction chemotherapy with cisplatin, adriamycin, and cytoxan yielded a partial response and the residual disease was managed with surgical resection.\nLittle has been reported on the role of advanced radiation therapy in the management of malignant thymoma. Until recently, no literature has demonstrated the superiority of one radiation therapy method over another. Despite the sensitivity of thymoma to radiation, the best use of radiotherapy remains controversial. Moreover, there remains no consensus on whether or not adjuvant radiation is of any benefit in completely resected thymoma. However, in this case study, we have demonstrated that by using targeted three-dimensional IMRT/IGRT, we were able to address the patient’s extensive recurrent disease and treat critical structures that were involved by the disease. In addition, our treatment approach spared the patient from any long-term detrimental side effects, especially to his myocardium and other critical organs. |
A 21 years old man, with no particular medical history, who presented a right leg painful mass was consulting at our hospital. There was no history of trauma or infection. On examination, a great warm mass measuring 12 × 21 cm was found at the end of the right tibia. Signs of inflammation were observed with a large swelling and oozing. He had no lymphadenopathy and no other mass. He just accused intermittent cough lasting for two months. His blood parameters were correct. An MRI (magnetic resonnance) was performed to establish the nature of this mass and its locoregional reports, it objectived abnormal signal intensities with a cortical destruction. A biopsy was then realised and the histopathological study revealed a chondroblastic osteosarcoma with an hypercellular neoplasm. He underwent an extension assessement by chest CT scan, scintigraphy, that revealed bilateral excavated pulmonary metastasis with different sizes and location. A pulmonary biopsy was performed to eliminate infectious diagnosis or vascularite, and revealed a metastasis of osteosarcoma. He had 3 courses of polychemotherapy API (doxorubicin 60 mg/m2 IV day 1, cis-platinum 75 mg/m2 IV day 1, and ifosfamide 7 g/m2 from day 1 to 5 IV with uromitexan to protect against hemorrhagic cystitis ) with partial response. Unfortunately, he died following a septic shock.\nLung represents a major metastatic site of the body as there's a seat about 30 to 50% of all secondary locations. The occurrence of excavated lung metastases is rarely observed only in 4% of cases (1). We report two cases with rare lymphoma of the gallbladder and osteosarcoma of the leg with pulmonary excavated metastasis emphasizing the role of CT in characterizing these lesions. Primitive tumor corresponds mainly to squamous cell carcinoma of the head and neck or cervix, rarely to colon (2,3). Metastatic sarcomas, especially osteogenic can disseminate as excavated pulmonary nodules with a high risk of pneumothorax. Excavation sometimes occurs after chemo-radiotherapy (4). A review of litterature shows that the primary site that metastasis to lung are excavated are bladder, kidney, breast, and ovary (2,5,6). CT scan is more sensitive than standard chest radiography for the detection of excavated metastatic lesions. It also allows aetiological diagnosis achieved by biopsy guided by the scan, as in our second observation. |
A 35-year-old woman (gravida 2, para 1) was referred to our hospital due to placenta previa at gestational week 31. Her medical history was unremarkable, and her previous pregnancy was an uncomplicated, normal vaginal delivery at gestational week 38. Her current pregnancy was uncomplicated except for the placenta previa. She denied abnormal genital bleeding before the current pregnancy. Cervical cytology performed during early pregnancy was negative for intraepithelial lesions. Vaginal ultrasonography revealed total placenta previa and one lacuna (Fig. a). Magnetic resonance imaging (MRI) at gestational week 31 revealed total placenta previa and loss of the myometrium between the placenta and bladder wall (Fig. b). Other MRI findings of PAS such as uterine bulging, heterogenous placenta, and T2 dark band were not observed. Based on these findings, we suspected PAS, and an emergency cesarean delivery was performed owing to antepartum bleeding (approximately 100 mL) at gestational week 35. An abdominal midline incision was made, and a healthy male infant weighing 2274 g (− 0.42 SD) was delivered with Apgar scores of 8 and 9, at 1 and 5 min, respectively. The placenta was not delivered within 30 min after fetal delivery, thus requiring hysterectomy for PAS. Estimated blood loss was 1000 mL. The postoperative course was uneventful, and the patient and baby were discharged on the 8th postoperative day.\nPart of the chorion and placenta were adhered to the uterus (Fig. a). The resected uterus was divided to 7 specimens in order to perform macroscopic and histopathological analyses. The surgical specimen showed a white polyp measuring 2 cm, which parted from the uterine fundus and the lower uterine segment (Fig. b). Histopathological examination of the tumor involving the lower uterine segment revealed endometrioid adenocarcinoma (Grade 1), with < 50% myometrial invasion and positive expression of estrogen and progesterone receptors, in addition to PAS (Fig. a and b). Notably, the tumor involving the uterine fundus did not show myometrial invasion. Histopathological findings were similar in both tumors located in the uterine lower segment and uterine fundus. A retrospective review of the MRI images obtained during pregnancy revealed the tumor involving the uterine fundus, although involvement of the lower uterine segment was difficult to detect (Fig. c). We performed a laparoscopic bilateral salpingo-oophorectomy and pelvic lymphadenectomy 102 days after cesarean hysterectomy and confirmed the absence of metastases. The tumor was a stage IA lesion based on the International Federation of Gynecology and Obstetrics system. Follow-up performed 4 years after cesarean hysterectomy revealed no recurrence. |
In May 2016, a 67-year-old woman came primarily to our hospital for a consultation about painless mass of the left lower gingiva. Intra-oral examination showed a 46 × 25-mm tumor with induration on the left lower gingiva (Fig. ). A submucosal mass, independent of the gingival tumor, was palpable in the left buccal region. Several cervical lymph nodes on the left side were also palpable. Pathological examination of a biopsy sample taken from the gingival tumor revealed a well-differentiated squamous cell carcinoma.\nA computed tomography (CT) scan with contrast showed a large gingival tumor, with destruction of the adjacent mandibular bone, and four metastatic left-cervical lymph nodes that were markedly enlarged, non-homogeneously enhanced, and partially necrotic. These lymph nodes included two left submandibular and two left upper jugular nodes. CT imaging showed no metastases to the lungs. Magnetic resonance imaging (MRI) showed a large primary tumor on the left side, with its epicenter located in the lower gingiva. The tumor appeared to extend into the sublingual space medially and into the buccinator muscle laterally. A non-homogeneously enhanced mass was identified in the buccinator space along the facial vessels, anterior to the anterior edge of the masseter muscle, and lateral to the buccinator muscle (Fig. ). This mass lay on the cranial side of the primary tumor. The mandibular ramus and pterygoid region that are on the cranial side of BN were not invaded by primary tumor (Fig. ). Moreover, T1-weighted MRI showed a thin layer with high signal, indicative of fatty tissue, between this mass and the primary tumor, indicating that the mass was independent of the primary tumor. Based on its anatomic location, the mass appeared to be metastatic disease to BN. Greyscale sonogram showed some metastatic cervical lymph nodes on the left, and metastatic BN. These cervical lymph nodes were markedly enlarged, round in shape, heterogenous hypoechoic, and without an echogenic hilus. Metastatic BN was round in shape, hypoechoic, with well-defined borders, and without an echogenic hilus.\nThe tumor was diagnosed as a cT4aN2bM0 squamous cell carcinoma of the lower gingiva. The patient received neoadjuvant chemotherapy, consisting of docetaxel 60–70 mg/m2 and cisplatin 70 mg/m2 on day 1, and 5-fluorouracil 700 mg/m2/day 96 h continuous infusion. Gross examination after two cycles of chemotherapy showed marked shrinkage of the primary tumor. A slight reduction in BN size was observed. According to the Response Evaluation Criteria in Solid Tumors (RECIST) guidelines, version 1.1 [], this patient showed a partial response to treatment.\nThree weeks after the end of neoadjuvant chemotherapy, the patient underwent surgery, consisting of suprahyoid neck dissection (levels I–II) on the right side, classical radical neck dissection (levels I–V) on the left side, segmental mandibulectomy, and oromandibular reconstruction with a scapular osteocutaneous flap. The primary tumor and buccinator space including BN were dissected in continuity with neck dissection. Histopathological examination of the segmental mandibulectomy specimens showed that the alveolar bone and part of the bone trabeculae of the mandible had been resorbed and replaced by fibrous connective tissue. This tissue contained a few nests of squamous cell carcinoma, composed mainly of necrotic tissue with a small number of viable cancer cells and remnants of keratin pearls. The surgical margins were free from tumor. Metastatic disease was detected in five cervical lymph nodes, including one left submandibular aggregated-node, three left upper jugular nodes, and one left middle jugular node. No metastatic nodes revealed extra-nodal extension. Metastasis to BN was also present (Fig. ). These metastatic regions contained few viable cancer cells and consisted primarily of necrotic tissue.\nFollowing surgery, the patient was treated with adjuvant radiotherapy (50 Gy/25 fractions) with concurrent oral chemotherapy (S-1, 100 mg/day for 5 days per week for 5 weeks) []. Two years later, there has been no evidence of tumor recurrence or metastasis. |
A 66-year-old African American male patient was diagnosed with MDS – refractory anemia with excess blasts 1 type. He received four cycles of decitabine, without significant improvement and remained dependent on allogeneic packed red blood (PRB) cells. Eight months later, he underwent bone marrow biopsy that showed a persistent increase in blast cells (8% blasts cells). He was started on fludarabine, busulfan followed by allogeneic PRB stem cell transplantation. Treatment was complicated by acute graft versus host disease (GVHD-with mild skin rash and elevation of liver function tests) that resolved immediately with an increase in tacrolimus dose. One year to the initial diagnosis, bone marrow biopsy showed no evidence of neoplastic cells and flow cytometry was negative and the patient achieved complete remission. The patient had a history of rising prostate-specific antigen (PSA) levels for one year, but the prostate biopsy was deferred due to his unstable hematological condition. Six months into remission, after stabilization of his hematological condition, the patient underwent a transrectal prostate biopsy (TRPB) that revealed Gleason 7 prostate cancer. He underwent robotic radical prostatectomy and histopathology specimen showed Gleason score 7, prostate adenocarcinoma involving <10% of prostate with no evidence of extraprostatic extension or seminal vesicle involvement and with negative surgical margins. Periprostatic tissue showed infiltrates of immature single cells resembling blast cells, but no blasts cells were noted in the prostate []. Immunohistochemical staining of periprostatic tissue identified neoplastic cells (T-cells and B-cells) positive for CD3, CD20, CD34, and CD117 suggesting myeloblasts consistent with a diagnosis of periprostate chloroma []. Subsequent bone marrow biopsy revealed low evidence of aberrant myeloid blasts (<5%). Repeat bone marrow biopsy 4 months following surgery was consistent with transformation to AML with 50% blasts. He received induction chemotherapy (FLAG regimen - fludarabine, high-dose cytarabine, and granulocyte colony stimulating factor) and donor lymphocyte infusion. The patient responded well to the treatment without any GVHD and bone marrow biopsy was hypocellular without evidence of leukemia and currently in remission. The patient is alive one year following the diagnosis of MS. |
A 40-year-old female, with 3-month history of nasal obstruction and tinnitus was admitted in August 2012. Nasopharyngeal endoscopy and biopsy already had been performed in another hospital, showing nonkeratinizing undifferentiated NPC. This histopathologic diagnosis was confirmed in our center. Magnetic resonance imaging (MRI) of the nasopharynx and neck revealed the tumor was confined to the nasopharynx and the bilateral locoregional cervical lymph nodes enlarged with its greatest dimension of 2 cm. Chest computed tomography (CT) scan, ultrasound of abdomen, and whole-body bone scan ruled out distant metastases. So clinical staging was determined to be T1N2M0, IIIA according to American Joint Committee on Cancer TNM Staging System for NPC (7th ed, 2010).\nThe patient was treated with definitive IMRT to 7050 cGy for primary tumor and 6600 cGy for infiltrated regional lymph nodes. Concurrent chemotherapy based on cisplatin and 5-flurorouracil was administrated for 2 cycles and then 2-cycle chemotherapy was given subsequently to consolidate the efficiency with the same regimen. At the end of therapy, she obtained clinical complete response by nasopharynx and neck MRI.\nIn the initial therapy, the patient had undergone abdominal ultrasonography for 4 times, and no hepatic lesions were noted during this period. Nevertheless, when she came to our hospital for 3-month conventional follow-up in April 2013, abdominal sonogram revealed a liver cystic lesion with thin wall and smooth margin of 18 × 16 mm in the right liver lobe, and the lesion was interpreted as a simple liver cyst (Fig. ). Therefore, intense follow-up was suggested. Five months later, the cystic lesion enlarged to be 59 × 46 mm, with thick wall, but no signal of blood flow. Further CT presented a low density and heterogeneous lesion taking irregular wall and incomplete septa with strong contrast enhancement, indicating liver abscess (Fig. ). However, the patient was asymptomatic, with no fever, no right up abdominal pain, and no palpable mass by physical examination. On the contrary, laboratory findings were negative, liver function was normal, the white blood cell count, C-reactive protein, and cancer-related antigen including α-fetoprotein were within the normal limits. Afterward the patient was transferred to another hospital for abscess drainage, and fluid culture was negative, but fine-needle aspiration was not performed. After drainage, the mass shrank significantly to one-third of the original size. However, it returned to previous size by CT 2 months later. Thus, the liver lesion was suspected to be malignant. Work-up examination including chest CT, pelvic MRI, emission CT for bones, gastroscopy, colonoscopy, and brain MRI excluded other lesion that may account for another primary tumor or extra-hepatic distance metastases from NPC. In addition, nasopharynx MRI showed no evidence of local relapse. On November 21, 2013, surgical resection was administrated both for histologic diagnosis and treatment, and the surgical margin was negative. Histopathologic examination definitely confirmed that the metastasis originated from NPC, since the cells of the surgical segment were similar to primary NPC on the morphology and they were positive in Epstein–Barr virus (EBV) encoded RNAs (EBERs) (Fig. ). No adjuvant chemotherapy was done after resection of the liver metastasis.\nHowever, relapse-free survival time lasted only for 8 months. In July 2014, 2 small cystic lesions were found on abdominal CT scan again, which were extremely similar to simple cysts (Fig. ). Further contrast-enhanced ultrasonography provided no sign of “fast in and fast out,” that is, a characteristic appearance in malignant carcinoma. As a result of multidiscipline team discussion, “watch and wait” strategy was recommended. However, the number of hepatic lesions increased to 4, and the size of previous 2 cysts enlarged to be 3 cm within 3 months. The multidiscipline team members reached a consensus that the cystic lesions were metastases from NPC and suggested a palliative systematic chemotherapy. Then the patient received chemotherapy with paclitaxel and cisplatin for 6 cycles. During the first 3 cycles, partial lesions diminished in size, but stayed stable within the later 3 cycles. Nevertheless, the intraliver metastases exhibited significant progress soon after the chemotherapy. Subsequently, she underwent chemotherapy with gemcitabine plus targeted therapy with nimotuzumab, then single navelbine both for 3 cycles, but neither protocols showed notable effects on hepatic lesions. In addition, during the phase of chemotherapy with navelbine, the patient complained of chest pain, an irregular and fixed lump was found on her chest wall, approximately 3 cm in diameter. After local surgical resection was performed, the lump histologically was demonstrated to be an extra-hepatic metastasis from NPC. Considering the patient's good performance status score and grade A liver function according to the Child-pugh grading system, transcatheter hepatic artery chemoembolization (TACE) was administrated for 5 times from December 2015 to April 2016. After the TACE treatment, all the lesions were obviously decreased in size, with the largest metastasis decreasing from 64 × 53 to 33 × 32 cm (Fig. ). Meanwhile, carbohydrate antigen 125 (CA125) decreased from 125 to 60.8 U/mL, and squamous cell carcinoma antigen decreased from 2.8 to 0.9 ng/mL. Then 4 cycles of gemcitabine plus cisplatin were offered to consolidate the clinical effects. In the next 6 months, her liver metastases maintained stable, but lung metastases were noted. In October 2016, the CT showed the liver metastases progressed. At last, she died of liver failure in March 2017. |
The patient is a 69-year-old woman. She was infected with pneumonia at the age of three and had a high fever for a long period. After the fever abated, she became aware of hearing loss. Because she lived in a mountain village, she rarely visited medical facilities. Since then, she had never worn a hearing aid, had gone to a school for the deaf from elementary school to high school, and communicated with people in sign language. Her husband also used sign language, so she had not used oral communication from elementary school to the present. In 2011, family stress triggered the onset of tinnitus. Because around the same time, she began suffering from sleep onset disorder (it took about one hour until sleep onset), nocturnal awakening, and palpitations, she went to a nearby psychosomatic medicine clinic. She received medication at the clinic but her tinnitus did not improve, so she was referred to our department in 2014.\nVarious questionnaires were given at her consultation. The result of THI (Tinnitus Handicap Inventory) [], which is used to evaluate the severity of tinnitus, was a high of 94, which indicates the most severe form of tinnitus. Also, an SDS (Self-rating Depression Scale) [] score, which is used to measure depression tendency, was as high as 61, showing that she had a tendency toward depression. The STAI (State-Trait Anxiety Inventory) [], which is used to measure anxiety tendency, returned a State Anxiety (STAIs) score of 69 and a Trait Anxiety (STAIt) score of 67, indicating that she had an extremely high anxiety tendency. As one of the personal features of her tinnitus, she had no concept of the “loudness of tinnitus” because she had been deaf since childhood.\nIn imaging tests, there were no abnormal findings except for a slight enlargement of the inner ear canals observed by temporal bone CT scan. Head MRI showed no abnormal findings within the skull or in the internal auditory canals.\nFor treatment, we started oral administration of a serotonin reuptake inhibitor (SSRI) (paroxetine hydrochloride, Paxil®, 12.5 mg, started as one tablet a day, increased to three tablets a day) and a sleep-inducing agent (suvorexant, Belsomra®, 15 mg, one tablet a day). One and a half months later, the sleep onset disorder and nocturnal awakening improved, but early morning arousal persisted. Then, a benzodiazepine anxiolytic was added (etizolam, Depas®, 0.5 mg, one tablet a day). At four months after the initial visit, THI was 84, SDS 43, STAIs 50, and STAIt 48, which showed that her psychological condition had improved, although the tinnitus distress level did not change.\nAt this time, there was no improvement in perceived palpitations, and “pulsatile tinnitus” that seemed to synchronize with the heartbeat became the chief complaint concerning tinnitus, which led us to suspect that she had autonomic disorders. Six months after the initial visit, she started to receive psychotherapy (autogenic training). After the start of the treatment, we treated the patient with psychotherapy once a month, which continued until the 7th therapy session was completed. At the end of psychotherapy, our test results showed THI at 60, SDS 45, STAIs 32, and STAIt 43, showing a further improvement trend. The THI score was still high at 60, but the subjective tinnitus distress became “not so annoying,” and the “echoing tinnitus” that was the cause of the patient’s discomfort at the time of the initial visit disappeared. Only the pulsatile tinnitus, which seemed to be related to palpitations, remained.\nOne year and seven months after the initial visit, palpitations and pulsatile tinnitus, as well as anxiety and insomnia, were aggravated due to work stress. She restarted psychotherapy. At the same time, SSRIs were replaced by noradrenergic and specific serotonergic antidepressants (NaSSAs) (mirtazapine, Reflex®, 15 mg, started as one tablet a day, increased to two tablets a day). As a result, improvement of the palpitations and insomnia gradually occurred, and two years and one month after the initial visit, our test results showed THI at 40, SDS 47, STAIs 40, and STAIt 46.\nBy three years after the initial visit, the symptoms had stabilized and the anxiolytic drug was discontinued, but oral administration of the NaSSA and sleep induction drugs continued. The subjective tinnitus and palpitations at the time of sleep almost disappeared, and the sleep onset disorder and nocturnal awakening rarely occurred.\nNow 4.5 years have passed and she is taking only a low dose NaSSA (mirtazapine, Reflex®, 15 mg, 0.5 Tablets a day). The latest test results were THI 0, SDS 43, STAIt 47, and STAIs 50, indicating that the tinnitus distress had disappeared completely. |
Mr. X was a 67-year-old man with nicotine dependence (45 packs-years), alcohol dependence in early full remission, and dysthymic disorder in remission. He was maintained on venlafaxine XR (225 mg per day) and quetiapine XR (50 mg per day + an additional 25 mg twice daily as needed) with no recent treatment regimen modification. Past psychiatric history was significant for recurrent unipolar major depressive episodes and cocaine abuse. Past substance use history included daily alcohol use since age 35. The number of standard drinks per day increased over the years but averaged 14–20 in the two years preceding his sobriety period. Cocaine was used once per month from the age of 35 to 45 and approximately two times per year thereafter until his sobriety period. He reported no other recent regular substance use. The patient had been stable psychiatrically with no alcohol or cocaine use for seven months. There was no personal or family history of bipolar disorder, but prior long-term substance use history remains a potential confounding factor.\nFor smoking cessation, bupropion SR coupled with nicotine replacement therapy (NRT) (14 mg patch daily) and therapeutic groups were used. In March 2012, bupropion SR (150 mg per day) was prescribed for three days and a preplanned quit date was set for day 4. As the treatment began, the patient noticed some feelings of excitement, which were amplified when the dose was increased to 150 mg twice daily on the fourth day of treatment. From that point on, he reported the onset of euphoria, racing thoughts, and decreased need for sleep. He subsequently relapsed to alcohol and cocaine use (on days 7 and 8) with minimization of the consequences of substance use. He mentioned no suicidality or psychotic symptoms. The patient met his therapist during the smoking cessation group on day 5, who noticed a change in the patient’s affect and describing him as more anxious, tense, and keyed up. Subjectively, on that day, the patient noticed being more anxious and having less ability to concentrate, which could be early nicotine withdrawal symptoms or early hypomanic symptoms. When seen by his psychiatrist 11 days after treatment initiation, the patient had decided to stop substance use and bupropion for two days. He was getting back to his baseline level, reporting some residual anxiety. Venlafaxine and NRT were maintained, while his quetiapine XR dose was increased to 100 mg per day regularly; bupropion was discontinued. One week later, the patient’s mood was stable, and he had not used any substances. |
A 51-year-old male patient, smoker, known to have diabetes insipidus type II, presented with a large deforming right hemifacial mass growing progressively over a period of 10 years, measuring 11 × 14 × 6 cm extending from the level of the right temporalis muscle above the zygomatic arch superiorly, down to the level of the mandible inferiorly, medially extending to the right lateral epicanthal fold, and laterally abutting the right external auditory meatus. The mass was firm, fixed but not tender, rubbery consistent with no overlying skin changes. Weakness was noted over the right eyebrow and right lower lip. No trismus or respiratory distress was present. Oral exam was unremarkable and a fiberoptic flexible laryngoscopy showed no narrowing of the nasopharyngeal, oropharyngeal, or hypopharyngeal walls. Narrowing of the right external auditory canal was evident on otoscopic exam. To note that the patient underwent a previous surgery in an outside hospital in attempt of removing the tumor bulk but the resection was minimal and a small portion of the tumor was removed, no pathological studies were taken at the time.\nCT scan of the brain and neck showed 11 × 6.2 × 14.5 cm heterogeneously enhancing right parotid mass with areas of necrosis, involving the deep lobe of the parotid gland. It is invading the lateral aspect of the masticator space, right temporomandibular joint, and external auditory canal causing narrowing of the latter and extending superiorly into the temporalis muscle and involving the masseter muscle (). The parapharyngeal space appears normal. There is erosion of the right zygomatic arch and a few subcentimetric lymph nodes surrounding the parotid mass posteriorly.\nA fine needle aspirate was done showing a picture suggestive of pleomorphic adenoma.\nThe decision was made to surgically excise the mass.\nAn extended right radical parotidectomy with excision of a portion of temporalis muscle, part of the masseter muscle, and zygomatic arch was made. Intraoperatively, the facial nerve was disappearing into tumor and was completely encased and invaded by tumor; a frozen section biopsy taken intraoperatively demonstrated high suspicion of malignancy, so the decision was made to sacrifice the facial nerve.\nPatient was scheduled for postoperative radiation.\nMass is composed of multiple components, with epithelial-myoepithelial carcinoma being dominant (80% of the tumor mass) with a minor component of solid variant of adenoid cystic carcinoma and basal cell adenocarcinoma (). The diagnosis of a hybrid carcinoma was made, in the presence of perineural invasion and absence of lymphovascular invasion. The surgical margins were negative for malignancy. |
A 33-year-old woman, gravida 4, para 3, live 3 at 35 weeks 4 days of pregnancy presented to the labor room with pain in abdomen for two days and something coming out of the vagina for one month. There was a history of difficulty in passing urine and stool for one month. All her previous deliveries were vaginal and in short intervals. There was no history of uterine prolapse in her previous pregnancies. She also had undergone right ovarian cystectomy one year back.\nOn abdominal examination, the uterus was found to be 34-36 weeks of size with mild contraction. The fetus was in the longitudinal lie with cephalic presentation and fetal heart sound was audible. On pelvic examination, stage 3 uterine prolapse with edematous cervix and parous size os was found (Figure ). Pelvic organ prolapse quantification (POPQ) was done and it revealed point C as the leading edge with other measurements being Aa-2, Ap-2, Ba-2, Bp-2, C+3, D-1, gh 6, pb 2, tvl 6. The reposition of the prolapsed uterus was tried but could not be achieved.\nUltrasound examination revealed a single live fetus with gestational age corresponding to 34 weeks and placenta lying in the anterior and upper segment of the uterus. A homogeneous mass of size 13 cm x 10 cm was seen arising from the posterior part of the cervix lying below the fetal head (Figure ).\nCesarean section was planned to deliver the fetus as the patient was progressing into obstructed labor. Preoperatively blood was cross-matched anticipating massive postpartum hemorrhage. Pfennaensteil abdominal incision was given and the uterine incision was put transversely avoiding the fibroid in the incision line during surgery. A cervical fibroid of size 10 cm x 10 cm partially covering the os was detected. A healthy child was delivered by vertex with a good APGAR score. The intraoperative blood loss was average in amount and we did not encounter any complications during surgery. Postoperatively, the prolapsed vaginal mass was successfully reposed and the rest of her hospital stay was uneventful. The patient was discharged with advice for further monitoring and management of the cervical fibroid and uterine prolapse in the postpartum period but she was lost to follow up. |
An 18-year-old male with a medical history of trigeminal neuralgia presented to the emergency department with complaints of severe epigastric pain. The pain was dull, started suddenly, and worsened gradually. The patient reported that he had this pain for the last 12 hours associated with nausea and three episodes of projectile vomiting containing food particles with no fever, rigors, and chills. He denied alcohol abuse and had no family history of any malignancy. However, the patient admitted having cocaine abuse for the last one week. The initial evaluation revealed a temperature of 98°F, blood pressure of 100/70 mmHg, respiratory rate of 22 breaths/minute, and heart rate of 102 beats/minute. The abdominal examination revealed normal bowel sounds with no distension.\nInitial laboratory analysis is given in Table and Table . His serum metabolic panel was unremarkable except for elevated serum lipase and amylase and mild elevation of serum creatinine and total bilirubin.\nAbdominal ultrasound revealed normal-sized liver and biliary ducts with no evidence of gallstone or biliary stone. A lipid panel was performed, and his LDL (low-density lipoprotein cholesterol) was 43 mg/dL, HDL (high-density lipoprotein cholesterol) was 55 mg/dL, serum cholesterol was 135 mg/dL, and serum triglyceride was 128 mg/dL. His urine drug screening confirmed cocaine abuse. He was admitted to the medical intensive care unit with a possible diagnosis of AP due to cocaine use as the patient had no other risk factor for AP.\nThe patient was managed conservatively. The patient was kept NPO (nil per os) and was resuscitated with isotonic fluids and analgesia. On the second hospital day, improvement in the patient’s clinical condition was observed and enteral nutrition was initiated. However, the pain did not improve. His pancreatic enzymes remained elevated. The patient was switched to parenteral nutrition again due to oral feed intolerance and pain in the epigastric region radiating to the back. His computed tomography (CT) revealed pancreatic enlargement with an ill-defined border (Figure ). Daily levels of lipase and amylase were analyzed (Figure ). The patient was symptom-free at hospital day 7 and was started on oral nutrition again. On day 8, he was discharged from the hospital with follow-up with his gastroenterology doctor as well as drug counseling service. |
A 70-year-old man with antecedent of follicular lymphoma in complete remission presented at the Timone University Hospital (Marseille, France) in 2016 for a squamous cell carcinoma of the hypopharyngeal region. The patient categorically refused any treatment, including preservative surgery, radiotherapy, chemotherapy or supportive care.\nOne year later, he was addressed to our palliative care unit by the hand-surgery department after attempting suicide. The patient explained his action by the fear of suffering. No depressive state was diagnosed by our psychiatrists. Despite persistence fear of suffering, the patient rejected the idea of suicide because of his family, but still wanted to die and asks for assistance. Information on Claeys-Leonetti law was given, especially on assisted-suicide banishment and on the possibility to relieve suffering with adapted treatments.\nOne week after discharge, the patient was readmitted to our department for dyspnea and anxiety. Symptoms were managed by appropriate treatments (oxygen and low dose of midazolam in an anxiolytic purpose). Despite stabilisation, the patient was afraid of dying suffocated and asked for deep and continuous palliative sedation until death. Apart from the fear he expresses, the patient has no symptoms of anxiety, depression or pain after the introduction of appropriate treatments. On the other hand, he clearly states that he refuses to live again knowing that his death is approaching and that he is apprehensive of suffering. He says he wants to rush his death. For us, this is a request for assisted-suicide (active help from a third party for the administration of a lethal product) or euthanasia (act of a third party which intentionally provokes the death of another to put an end to his sufferings), rather than a real demand for deep and continuous sedation. It seems important to note that patient’s requests for deep and continuous sedation until death are not registered officially. The law does not even impose a written request. Thus, the request is most often made orally in the presence of several doctors and clinicians.\nIn order to try to objectify this request and therefore our answer, the patient’s request was examined and denied by palliative multidisciplinary board, in accordance with by the French Oncology Coordination Centre guidelines. This situation did not fulfil the criteria requested by Claeys-Leonetti law. Indeed, prognosis appeared not short term committed (no visible clinical progression of the disease, which commits for sure the short-term vital prognosis), symptoms were managed with appropriate treatments and no life-sustaining treatment arrest could lead to potential unbearable sufferings. Regarding the short-term criterion of life-threatening prognosis, the patient was offered to have a Computed Tomography (CT) scan to measure the progression of the disease. Indeed, no imaging had been performed for one year (time of diagnosis of recurrence). The patient refuses this proposal. The request for deep and continuous sedation was reiterated several times by the patient, who was still refusing any investigations to define the progression of his cancer and wanted parenteral hydration to be maintained. Daily, he questioned each caregiver about the rationale for the refusal of his request. How can the medical staff be sure that his prognosis is not short-term compromise? Why his psychological distress could not be considered as refractory? One week after refusing further investigation, the patient finally agrees to undergo a CT scan. Three days after the exam he dies peacefully, according to our team (no specific questionnaires or objective elements to judge the quality of death exists), of a not predictable respiratory distress certainly linked to the evolution of his cancer of the hypopharyngeal region without introduction of deep and continuous sedation, but with introduction of midazolam for anxiety. Opiates were not introduced because the patient was saying not being painful. The CT scan results, unknown at the time of death, reveal nothing conclusive (pulmonary metastases, but no lymph node involvement) and would have required additional analyzes. |
The patient was a 32-year-old otherwise healthy female who was admitted to a tertiary care center with unexplained hypoglycemia and altered mental status. She was in her usual state of health until one month prior to hospitalization when she presented with morning drowsiness and loss of appetite. Due to the gradual worsening of symptoms, she was brought to the Emergency Department for further evaluation.\nUpon admission, the patient was found to have a glucose level of 35 mg/dL. She had recurrent episodes of hypoglycemia despite numerous intravenous glucose infusions which led to a work up for a possible pancreatic tumor. An ultrasound of the abdomen was performed showing several liver nodules along with a large pancreatic mass measuring up to 10.7 cm in the largest diameter (not shown). Computerized tomography (CT) scan of the abdomen was performed confirming the sonographic findings. The patient underwent a liver biopsy. Histopathology and immunohistochemistry results were consistent with low-grade neuroendocrine neoplasia (positive stain for chromogranin A) (). Insulin and C-peptide levels are depicted in .\nThe patient received continuous intravenous glucose because of recurrent episodes of hypoglycemia throughout the hospital stay (). Glucagon and hydrochlorothiazide failed to elevate glucose levels.\nOctreoscan was performed showing a high uptake in focal areas of the liver, left lower quadrant of the abdomen, and cervicothoracic area ().\nSandostatin LAR 30 mg IM was started on hospital stay day 43 on an every 28 days basis. The patient received 2 infusions of radiolabeled somatostatin analog lutetium (177LU) 8 weeks apart beginning on hospital day 56. By the second administration of the radiopharmaceutical, Octreoscan SPECT/CT had already shown objective metabolic and radiologic response to treatment (Figures and ). Serum glucose levels started to rise by hospital stay day 60 (). Intravenous glucose infusion was titrated progressively down onwards. The patient was discharged on a normocaloric diet on hospital day number 140. |
A 64-year-old man who had no symptoms was diagnosed with thoracic superficial esophageal cancer that was detected by screening upper endoscopy. He had a history of hypertension. He had also been found to have a vascular abnormality (DAA) as an adult and was observed in an asymptomatic state.\nPhysical examinations showed no unusual findings, and the laboratory examination data, including tumor markers, such as squamous cell carcinoma-related antigen and carcinoembryonic antigen, were all within normal ranges. Chest X-ray demonstrated a widening in the upper mediastinal silhouette, reflecting the superior right aortic arch. An endoscopic examination revealed superficial esophageal cancer located in the left side of the wall in the upper thoracic esophagus and the invasion of the submucosa (Fig. ). A histological examination of biopsy specimens confirmed the presence of squamous cell carcinoma. Enhanced computed tomography showed a swollen lymph node in the right upper mediastinum, which was diagnosed as metastatic (Fig. ). No distant metastasis was detected. Computed tomography also confirmed the DAA. The right aortic arch was dominant, and the descending aorta was located at the right side of the post-mediastinum, as is common in cases of DAA (Fig. ). The patient was therefore diagnosed with upper thoracic esophageal cancer of cT1bN1M0 Stage IIB (UICC-TNM 7th) and a DAA.\nHe underwent neoadjuvant chemotherapy prior to sub-total esophagectomy with three-field lymphadenectomy. The neoadjuvant chemotherapy regimen was 2 courses of 5-FU (800 mg/m2) and cisplatin (80 mg/m2) every 3 weeks.\nWe planned to perform radical subtotal esophagectomy with three-field lymph node dissection after neoadjuvant chemotherapy. We first planned to perform cervical procedure in a supine position before the thoracic procedure in order to identify the bilateral inferior laryngeal nerves and avoid causing them injury or inducing palsy. We also planned to perform upper mediastinal lymph node dissection during this preceding procedure because the DAA was expected to interfere with upper mediastinal dissection attempted via either side of a transthoracic approach. We then planned to perform lymph node dissection via a left-thoracoscopic approach below the left aortic arch, as we worried that the right-sided descending aorta might interfere with a right-thoracic approach (Fig. ). The laparoscopic procedure was planned to be performed via an abdominal procedure in a supine position. Reconstruction would use the gastric tube pulled up via the retrosternal route with cervical esophago-gastric anastomosis.\nIn the preceding cervical procedure performed in a supine position, we identified the bilateral inferior laryngeal nerves, which were thought to be recurrent at each side of the aortic arch (Fig. ). After upper mediastinal dissection was performed, the left thoracoscopic procedure in a prone position was performed for middle and lower mediastinal lymph node dissection below the left aortic arch. We first confirmed that the right-sided aortic arch and descending aorta would interfere with the usual right thoracic approach (Fig. a). Upper mediastinum dissection was also deemed impossible via a bilateral thoracic approach because of the bilateral aortic arches and subclavian arteries, as expected preoperatively (Fig. a, b). Postmediastinal reconstruction also seemed impossible. The port position for the left thoracoscopic procedure was set symmetrically to our normal right thoracoscopic procedure for middle to lower mediastinal dissection, as shown in Fig. . No major anatomical findings other than those noted preoperatively were observed during the left thoracoscopic procedure. We were unable to identify where the thoracic duct ascended because of the preservation of the thoracic duct. We were also unable to confirm the details concerning both recurrent laryngeal nerves around each aortic arch.\nThe abdominal procedure in a supine position was performed laparoscopically with the simultaneous cervical procedure for bilateral supraclavicular lymph node dissection. Reconstruction was performed with cervical esophago-gastric tube anastomosis. The gastric tube was pulled up through a retrosternal route as planned. Three-field lymph node dissection and complete resection (R0) were achieved. The operative time was 8 h 9 min, and the total bleeding was 70 ml. No vocal cord palsy was observed on flexible laryngoscopy after the operation.\nThe patient’s postoperative course included minor leakage that was cured conservatively after 2 weeks, and he was discharged at postoperative day 29. The pathological diagnosis was ypT1bN0M0 Stage IA (UICC-TNM 7th edition). The patient was followed for 2 years with no signs of cancer recurrence. |
A 67-year-old lady was admitted via the emergency department after being found unresponsive at home by her husband early in the morning. On presentation, her GCS was 9 with normal vital signs. Her glucose level was 8 mmol/L, and the blood gases were normal. On observation an hour later, her dextrose test was rechecked and fell to 1.5 mmol/L with blood glucose of 0.9 mmol/L. She was given intravenous dextrose and her condition improved.\nOf note, her past medical history included a two-year history of type 2 diabetes mellitus, recently diagnosed depression, and osteoporosis. Her oral hypoglycemic agents had been recently withdrawn as her blood sugar levels were well controlled on dietary changes she had made. Her family reports that she was increasingly anxious over the last few months with occasional confused periods. She also had a 4 kg weight loss over the last 12 months (10% body weight). Her daily medications include citalopram, bromazepam, and weekly bisphosphonate. There was no family history of diabetes mellitus, hypertension, or hyperparathyroidism. The patient denied taking any excess medication or oral hypoglycemic agents.\nOn examination, the patient was alert but appeared very anxious; her GCS returned rapidly to normal on intravenous dextrose. Cardiovascular, gastrointestinal, and neurological examinations were normal as well as her other laboratory investigations.\nThe patient underwent a 72-hour fast as part of the investigation but was interrupted at 2 hours due to hypoglycemic symptoms (sweating, feeling drowsy and unwell). During this episode, her blood glucose level was confirmed to be low (1.1 mmol/L), C peptide was elevated at 8.2 and insulin levels at 91. Her sulfonylurea screen was negative and her other pancreatic endocrine hormones were sent and came back as within normal limits.\nCT scan of abdomen revealed a 2.6 cm by 2.0 cm hypodense mass containing peripheral calcifications in the body of the pancreas (). Multiple low attenuation liver lesions, with the largest one measuring 5 cm, were seen in segment 8 of the liver. An endoscopic ultrasound (EUS) also found a 5- to 6-cm oval-shaped hypoechoic lesion () with irregular margins involving the left body and junction body/tail of the pancreas clearing invading the splenic artery with several hypoechoic large local lymph nodes. EUS-guided biopsies of the pancreatic mass and nodes were performed. A somatostatin receptor scintigraphy with octreotide showed intense uptake in the pancreas and the liver and no distant lesions. Due to the aggressive nature of the clinical presentation with anorexia and weight loss, an ultrasound-guided liver biopsy of hepatic metastases was also performed. Histopathology from all sites confirmed neuroendocrine differentiation with diffuse chromogranin A and synaptophysin staining; the Ki-67 proliferation index varied form <2% in the pancreas to 10 to 15% in liver metastases ().\nThe initial management consisted of intravenous dextrose infusions for multiple episodes of hypoglycemia with no particular diurnal variations, and these episodes were not relieved postprandially. Oral diazoxide was rapidly commenced without any appreciable effect on hypoglycemic episodes, and the patient was thereafter commenced on subcutaneous octreotide (100 mcg three times a day increasing to 600 mcg/day); the latter improved marginally the glucose levels, but she developed peripheral oedema and abdominal cramps. Following a multidisciplinary discussion, it was decided to attempt surgery using either a one-stage or two-stage strategy. At laparotomy, she underwent a distal pancreatectomy, splenectomy, and right hepatectomy. Wedge excision of segment 2 was also performed at the time of the procedure. The postoperative period was marked by difficult to control hyperglycemia, but the patient made a rapid recovery and was fit to discharge home after 14 days. All symptoms attributable to hypoglycemia completely disappeared with the patient experiencing a normal psychological state—no episodes of anxiety or unexplained confusion or stress as described prior to presentation. She is in good health with normal follow-up imaging, C-peptide levels measurement. She remains diabetic nine months postoperatively, and the diabetes is diet controlled. |
We describe the case of a 50-year-old woman who was admitted to a pheriferal department for heart failure (NHYA class III) in mild idiopathic dilated cardiomyopathy. She had chronic atrial fibrillation and hypertension in anamnesis. On admission to the hospital, the patient's blood pressure and heart rate were normal. Her oral temperature was 36.5°C. Examination of the head and neck revealed no abnormalities; the external jugular vein was distended; no adenopathy was present. Abdominal examination revealed mild hepatomegaly. Cardiac examination revealed a systolic murmur at the fourth intercostal space on the marginal sternal line. The laboratory results were normal; no increase in inflammatory index. Chest radiograph showed an enlarged cardiac silhouette and the ECG showed atrial fibrillation. The patient also underwent echocardiography, which revealed a small mass measuring about 1.3 × 1.0 cm adhering to the non-coronary cusp of the aortic valve, mild dilated cardiomiopathy and severe biventricular dysfunction. In order to the presence of AF, this mass had erroneously been considered a thrombotic lesion, so the patient was treated with thrombolysis and heparin e.v.\nAfter fibrinolysis, the dimension of this lesion did not change, so the patient was transferred to our department. Transthoracic and transoesophageal echocardiography was performed to define the mass. It showed a highly mobile, homogeneous echodense mass with a smooth surface from a pedicle to the closing margins of the noncoronary aortic cusp. All three aortic cusps had no signs of thickening and appeared normal as judged by echocardiography. Colour Doppler revealed no valvular incompetence. From echocardiographic findings, a tumour of the aortic valve rather than a thrombus or a vegetation was suspected (Figs. and ). Magnetic resonance imaging (MRI) was then performed to determine the character of the tumour tissue. It revealed a mobile nodular mass which was characterized by a slightly lower signal intensity than the myocardium at MRI with a single-slice breath-hold segmented turbo fast low-angle shot technique.\nTo prevent systemic embolization, the patient underwent excision of the tumor. The echocardiographic and MRI findings were confirmed during surgery. A 1-cm papillary mass was found attached to the noncoronary cusp of the aortic valve and was treated by simple excision of the tumour without aortic valve replacement. Histological examination revealed several fragments covered by flattened cells. The core was composed of dense fibrous tissue with hyalinization, consistent with a papillary fibroelastoma of the endocardium.\nIn the following days, the patient no longer showed signs of dyspnoea. An echocardiogram was performed at discharge without evidence of a mass attached to the noncoronary aortic cusp. |
A 33-year-old Somalian man initially presented with loss of neurological function of the left arm, hazy vision, and headache. A few weeks prior to this episode he had had an episode during which he experienced chest pain, nausea, and vomiting. Our patient does not drink alcohol. He smokes tobacco and chews two bundles of khat a week for more than 10 years. No other risk factors for atherosclerosis at young age were present. The blood levels of cholesterol and triglycerides were within normal range. Family history was negative for inherited thrombophilia. His ECG on admission showed a Q wave in V1 and V2 and 2 mm ST-elevations in V1, V2, and V3 and a terminal negative T wave in I, aVL, V2, V3, and V4, consistent with a recent, evolving anterior infarction (). On admission the highly sensitive troponin T was 18 ng/L (normal value < 14 ng/L). The transthoracic echocardiogram showed a poor left ventricular function with akinesia of the anterior wall and left ventricular dilatation. No thrombus was seen.\nThe noncontrast enhanced CT of the brain showed ischemia in the right middle cerebral artery vascular territory. The CTA of the carotids showed no stenosis or venous thrombosis.\nAn MRI of the brain was performed to rule out vasculitis or other vascular malformations. The MRI showed recent ischemia in the vascular territory of the posterior division of the right middle cerebral artery (Figures –).\nCoronary angiography showed a 70% stenosis with haziness of the proximal left anterior descending artery (LAD) (), which disappeared after intracoronary nitroglycerine injection (). The other coronary arteries were normal.\nMRI of the heart showed a poor left ventricular function, with a calculated ejection fraction of 29%. The left ventricle was dilated, with an end diastolic left ventricular diameter of 63 mm (normal value < 55 mm). There was thinning of the myocardium in the apex and the midcavitary anteroseptal, anterior, and anterolateral segments. There was hypo- to akinesia of these areas with subendocardial and transmural delayed enhancement (Figures and ) in these regions. No thrombi were seen. The signs on the MRI are consistent with infarction in the vascular territory of the LAD.\nScreening for thrombophilia (lupus anticoagulants, cardiolipin antibodies, factor V Leiden, and antithrombin III deficiency) was negative. A presumptive diagnosis was made of khat induced coronary spasm with myocardial infarction and khat induced cerebral infarction. Due to hypotension treatment with calcium blocking agents was not possible. He was advised to refrain from khat use. Furthermore he underwent a percutaneous coronary intervention of the proximal LAD with stent placement to prevent stenosis in case of possible future coronary spasm. His left ventricular function remained poor, which was a reason for ICD implantation for primary prevention. During admission all neurological complaints resolved. |
A 65-year-old man with a past medical history of diabetes mellitus developed sudden weakness of the right arm and leg. He presented the following day to our hospital; on physical examination, he was found to have a fever of 38.7 °C, mild right hemiparesis (Medical Research Council scale grade 4), right facial palsy, and dysarthria. He scored four points on the National Institutes of Health Stroke Scale. His cardiac examination findings, including echocardiography (EKG) and transthoracic echocardiography, were unremarkable. MRI of the brain was performed and multiple high signal intensity lesions were seen in the left frontal and parietal lobes on T2-weighted MRI and on diffusion-weighted imaging (DWI) with an associated low signal on the apparent diffusion coefficient (ADC) map (). We subsequently performed contrast-enhanced brain MRI and magnetic resonance angiography (MRA). There was no evidence of enhancement of the lesions on post-gadolinium images and there was no evidence of hemorrhage on T2 *-weighted images. The lesions were all felt to be in the left middle cerebral artery territory (although there was some question as to whether the most inferior lesion was in the posterior cerebral artery territory). However, contrast-enhanced brain MRA showed no steno-occlusion of the left middle cerebral artery. An initial diagnosis of acute cerebral infarction was made on the basis of these findings and the patient was treated with antiplatelet therapy. Because of the patient’s fever, an infectious work-up was performed and blood cultures were drawn. The complete blood count was unremarkable but chest X-ray suggested bronchitis. The patient was empirically treated with netilmicin and cefmetazole and his fever resolved within 2 days of admission to the hospital. On day five, Enterobacter aerogenes was found in his blood cultures.\nOver the next several days, the patient did well and was presumed to have experienced an embolic stroke as well as bronchitis complicated by septicemia. On the eleventh day after symptom onset, the patient experienced a focal motor seizure involving his right hand and follow-up MRI of the brain was performed. The T2-weighted MRI was notable for interval development of extensive vasogenic edema surrounding the left-sided brain lesions and the post-gadolinium T1-weighted MRI images demonstrated the ring enhancement of these lesions (). Based on these findings, the patient’s diagnosis was revised to multiple brain abscesses and he was switched to a 6-week course of triple antibiotic therapy with ceftriaxone, vancomycin, and metronidazole. The patient improved clinically and was discharged home without residual deficits after two months. Follow-up MRI at this time showed a marked decrease in the size of the lesions and associated edema (). |
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