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A 40-year-old woman was found to have a Helicobacter pylori infection at a standard health check and was accordingly referred to our hospital. An upper gastrointestinal endoscopy demonstrated a 0-IIc-type lesion 15 mm in diameter located at the lesser curvature of the middle gastric body, without ulceration (Fig. ). Histological analysis of a biopsy specimen demonstrated a signet ring cell carcinoma, while computed tomography revealed neither enlarged lymph nodes nor distant metastases. Accordingly, undifferentiated-type mucosal gastric cancer was diagnosed, with the possibility of lymph node metastasis considered very low. As the lesion fulfilled the expanded indication for ESD according to the Japanese gastric cancer treatment guidelines [], we elected to perform ESD as recommended treatment. However, because the patient and her family ultimately requested surgical treatment, we therefore performed laparoscopy-assisted distal gastrectomy with D1+ lymph node dissection without any intraoperative complications. Macroscopically, the 0-IIc lesion without lymph node metastasis was identified in the resected specimen (Fig. ).\nRoutine histological analysis of the resected specimen with hematoxylin and eosin (H&E) staining revealed a predominantly signet ring cell carcinoma of 15-mm diameter limited to the mucosa, without lymphatic-vascular capillary involvement or ulcerative components. There were neither apparent findings outside the zone of the tumoral area nor another malignant lesion in the resected specimen. Multiple lymph node metastases of the perigastric area were unexpectedly identified in 15 of the 45 retrieved lymph nodes; therefore, the tumor was classified as pathological stage IIB (T1a N3 M0).\nBecause of multiple lymph node metastases, the present case was sufficiently unusual to prompt us to perform additional deep sectioning of the whole tumoral area at a thickness of 20 μm, and further analyses were performed using H&E and D2-40 staining. The results of these analyses demonstrated that all cancer cells existed completely limited to the mucosa, with immunohistochemical staining for desmin and vimentin revealing no evidence of fibrosis in the submucosal layer or deformity of the muscularis mucosae. However, we finally identified isolated lymphatic capillary involvement of the extremely superficial submucosa in a single histological section (Fig. ).\nThe postoperative course of this case was uneventful, and the patient received postoperative adjuvant chemotherapy with an S-1 oral agent. At this moment, no recurrence was observed following strict surveillance for 1 year postoperatively.\nIn general, further size and ulceration of early mucosal gastric cancer are considered as risk factors for lymph node metastasis. The rate of lymph node metastasis from mucosal early gastric cancer has been reported to be as high as 8 %, and the risk factors for lymph node metastasis were tumor size and having ulceration in 148 cases, who were diagnosed with early mucosal gastric cancer without adaptation of endoscopic resection and underwent laparoscopic gastrectomy []. Hirasawa et al. reported that tumor sizes greater than 21 mm, lymphatic-vascular capillary involvement, and submucosal penetration were considered as risk factors for lymph node metastasis in a series of 3843 cases diagnosed with undifferentiated-type early gastric cancer who underwent gastrectomy []. Recently, Pyo et al. demonstrated tumor sizes larger than 17 mm, elevated tumor type, and lymphatic-vascular involvement to be significantly associated with lymph node metastasis []. The present case would be a candidate for curative resection with expanded criteria according to the Japanese guidelines because of the identification of an undifferentiated lesion with a diameter of 15 mm without ulceration. However, postoperative histological analysis revealed as many as 15 regional lymph node metastases in our case, despite the absence of metastatic lymph nodes in either preoperative analysis or intraoperative observations.\nSynchronous lymph node metastasis has previously been reported in a case fulfilling the expanded criteria for endoscopic resection []. In this report, the lesion was sliced into 60 thin sections, and additional histological analysis was performed using D2-40, desmin, Masson, and vimentin immunohistochemistry. As the result, lymphatic vessel involvement was identified in the deep mucosal layer, and accordingly, the authors suggested that routine pathological analyses for undifferentiated-type gastric cancer are inadequate for determining curative resection. Sako et al. reported an improvement in the diagnostic accuracy of lymphatic vessel involvement by using an immunohistochemical method with D2-40 in the pathological analysis of early gastric cancer []. Furthermore, a correlation between lymph node micrometastasis and lymphatic invasion has previously been reported, with micrometastasis found to be more closely correlated with D2-40 than H&E staining [, ]. There are some immunohistochemical examinations for detecting lymphatic-vascular involvement. Actually, D2-40 staining is the most powerful tool to detect lymphatic-vascular involvement. As for other methods, lymphatic vessel endothelial hyaluronan receptor-1 (LYVE-1) and platelet and endothelial cell adhesion molecule 1 (PECAM1) are also available, but these are less common. Therefore, we conducted additional pathological analyses of deep-cut sections of the whole tumoral area at 20-μm thickness using H&E and D2-40 staining to identify cancer cells in unexpected areas. Despite tumoral depth in the remaining mucosa, we finally identified isolated lymphatic capillary involvement in the shallow submucosal layer in one histological section. Consequently, gastrectomy with lymph node dissection could contribute to a favorable outcome in this case. |
A 47-year-old female, Scottish Sheep farmer presented to Aberdeen Royal Infirmary Major Trauma unit with pain and discomfort from a lesion on her non dominant left ring finger distal phalanx. She accidently stabbed her hand when she was shearing a sheep this resulted in a laceration to her left ring finger. She presented 2 weeks following the incident with a soft well circumscribed mass on the ulnar aspect of her distal phalanx bordering the nail plate. The patient has a past medical history of protein c deficiency for which she requires lifelong warfarin. She is a non-smoker, works full time as a farmer and is physically fit able to perform all activities of daily living. She was not aware that any of her sheep were infected with the Orf virus infection and had not seen evidence of lesions around the mouths of her sheep.\nAberdeen Royal Infirmary run a dedicated tertiary hand service. The on-call orthopaedic hand registrar reviewed the patient. After taking a full medical history a through clinical examination was performed. The lesion presented on the left ring finger distal ulnar aspect bordering the nail a 4 mm by 4 mm discrete brown hemispherical lesion was noted (). The surrounding skin was slightly erythematous. The lesion was not warm, nor tender on palpation. The patient did not present any Kanaval’s signs to suggest pyogenic tenosynovitis or evidence of dorsal compartment infection. No fluctuance was noted in the lesion. The patient had a full range of movement in her left ring finger. The distal neurovascular status of the finger was intact. The patient had seen a clear fluid exudate discharge a day before she presented. The lesion was dry on presentation. The patient was prescribed oral antibiotics by her general practitioner which commenced a week before presenting to the hand unit. The antibiotics did not resolve the lesion. Radiographs taken did not show any evidence of foreign body within the distal aspect of the left ring finger ( and ).\nThe differential diagnosis included over granulation following trauma to the hand, pyogenic granuloma or a felon. Her observations and inflammatory markers were normal, this further ruled out infection. The patient presented to the hand trauma clinic 2 weeks after presentation to the hand on call service. Her hand was re-examined the lesion showed evidence of regression. The senior author recognised the lesion. The Orf virus infection diagnosis in a non-immunocompromised patient is self- limiting. The lesion eventually resolved in 10–12 weeks without any scars. |
A 41-year-old woman, para 3, with abdominal and back pain was referred to our hospital for the evaluation and treatment of a large cystic tumor. Magnetic resonance imaging (MRI) confirmed the diagnosis; the tumor was approximately 27 cm × 9 cm in area with a solid component inside the cyst []. Tumor markers for epithelial ovarian cancer were elevated as follows: Carbohydrate antigen (CA) 125, 150 U/ml; CA 19-9, 220 IU/ml. Ovarian tumor was suspected and the laparoscopic operation was performed using three port insertions. Based on the surface characteristics of the tumor, it seemed more likely to be peritoneal rather than ovarian in origin []. The tumor was connected to the uterine body by a narrow, 3-cm long band []. The tumor capsule ruptured easily when gently touched with forceps. Brown chocolate-like fluid and coagulative tissue leaked from the tumor [ and ]. Most of the cyst contents were removed by suction. Tumor attachments to surrounding structures were severed and then the tumor was packed into a specimen bag in the peritoneal cavity [], cut into small pieces inside the bag, and removed through the port site. Both adnexa were normal in size and shape, without any connections to the tumor []. Two intramural uterine myomas 5 cm in each diameter were located on the anterior wall of the uterine body, and myomectomy was performed laparoscopically []. We ultimately determined that the tumor had been connected only to the uterine body [] and to portions of the left [] and right [] uterosacral ligaments. There were no findings of another adhesions or endometriosis in the pelvic cavity. Since intraoperative frozen sections of the cystic tumor resulted in a histopathological diagnosis of the endometrial cyst without malignancy, the laparoscopic operation was concluded.\nAlthough the solid component was not clearly identified macroscopically inside the tumor, subsequent postoperative histopathological examination confirmed the presence of the tumor epithelium, showing an increased nuclear-to-cytoplasm ratio, several mitotic cells, papillary changes, stratification, and metaplastic alteration to squamous cells without necrosis or invasive growth into the stroma [– and ]. Immunohistochemical staining was negative for HNF1β [], p53, and MIB-1 and positive for ER. The postoperative histopathological diagnosis of the tumor was confirmed as the endometrioid borderline tumor in an endometrial cyst. Moreover, atypical endometriosis showing cytological atypia and architecturally, complex proliferative lesions were also identified [ and ].\nBecause the patient did not desire future childbearing, hysterectomy, bilateral adenectomy, partial omentectomy, and pelvic lymph node biopsy were subsequently performed as a staging laparotomy for ovarian borderline tumor. No remnant tumor cells were detected on postoperative histopathological examination. Based on laparoscopic findings, the large endometrial cyst was thought to have arisen in the peritoneum, whereas pathological findings indicated that the endometrioid borderline tumor probably originated from endometriosis. The patient provided informed consent for this case report and associated images. |
A 40 year-old male patient reported to the Department of Periodontology with a complaint of hypersensitivity to chilled drinks, in relation to the upper left and right back teeth region. He had no significant medical history. The patient gives a two-year prior orthodontic history. On clinical examination, multiple adjacent recessions were identified on the right and left posterior maxillary teeth. The bilateral recession defects, Miller Class I, were measured by calculating the distance between the cementoenamel junction (CEJ) and the gingival margin. It was recorded as the second premolar of 3 mm and first molar of 3 mm. A hard tissue abrasion defect was also present on these teeth and was measured to be less than 0.5 mm in dimension. Phase 1 therapy was completed with oral hygiene instruction reinforced. The surgical procedure was explained to the patient and informed consent was obtained. The use of an additive membrane was assigned by toss of a coin.\nThe operative site, that is, 15 and 16 was anesthetized using 2% Xylocaine with adrenaline (1:200,000). A coronally positioned flap technique was performed at the surgical site in relation to 15 and 16 []. This was performed by making two horizontal incisions with respect to the distal and mesial interdental papillae of 16 and 15, followed by a crevicular incision, two vertical releasing incisions at the mesial and distal aspects of 16 and 15. A full thickness flap followed by a partial thickness one was reflected []. A horizontal releasing incision was made in the periosteum, at the base of the flap, to facilitate tension-free coronal displacement. The exposed root surfaces were scaled and root planed. Following this, the cervical step at the CEJ was eliminated using an aerator and a diamond bur.\nAfter the recipient site preparation was completed, 5 ml of venous blood was drawn in test tubes without an anticoagulant, and centrifuged immediately. It was centrifuged for 12 minutes at 2700 rpm. The resultant product consisted of the following three layers: The topmost layer consisted of acellular Platelet-Poor Plasma (PPP), a PrF clot in the middle, and red blood cells (RBCs) at the bottom. After centrifugation, the PrF clot was obtained, separated from the RBC base using scissors, and placed in a sterile dappen dish. The PrF membrane was prepared by placing it into a petri dish. At the recipient site, the PRF clot was placed over the denuded root surfaces []. The flap was coronally advanced to cover the membrane as well as the defect and sutured []. A tin foil and periodontal dressing were placed over the surgical area.\nThe patient was advised to use 0.2% chlorhexidine digluconate mouth rinse, twice daily. Systemic analgesics were prescribed and he was advised to follow the routine postoperative instructions. The dressing and sutures were removed 10 days after surgery.\nFollow-up of seven months [].\nA similar surgical procedure was followed for the receipt bed preparation along with reduction of the cervical step in relation to 25and 26, similar to that for the PrF membrane [Figures –]. The commercially available amniotic membrane# was cut into the desired shape and length with scissors and placed onto the recession site []. The flap was coronally advanced and sutured []. A tin foil and periodontal dressing were placed over the surgical area.\nSimilar postoperative instructions were advocated as those for CAF with PrF.\nFollow-up of seven months [].\nPostoperative examination was done for one, three, six, and seven months. At the end of the seventh month, both the treatment procedures showed 100% root coverage and increased gingival biotype. However, the amniotic membrane-treated sites showed more stable results than the PrF-treated sites at the end of the seventh month. |
A 75 years old male patient, presented to the outpatient department with a 3-month history of vague abdominal discomfort (stretching sensation in the upper abdomen) and an intra-abdominal mass “about the size of his fist” which he believed had gradually increased in size over the previous 2 months. On examination the mass was found to be located above the umbilicus; it was mobile, smooth, approximately 6 × 7 × 5 cm and slightly tender on deep palpation. The baseline laboratory workup consisted of routine blood tests including hepatic and renal function tests which were all unremarkable. An abdominal CT scan with IV contrast revealed a heterogeneous, soft tissue, mesenteric mass with calcifications in the root of the mesentery, encasing the superior mesenteric vessel. Mesenteric fibrosis resulting in tethering of small bowel loops with extensive desmoplastic reaction and small mesenteric lymph nodes were also noted (Figures , , and ).\nWith high suspicion of bowel carcinoid tumor by the radiologist (although a differential diagnosis of fibrosing mesenteritis was also suggested), a diagnostic laparoscopy was performed. Intraoperatively, the mass was found to be located in the paraaortic region, at the root of mesentery. Because the friable lesion bled easily on touch, the procedure was converted to a minilaparotomy.\nA wedge of the mass was taken for histopathology examination which revealed extensive fibrosis along with a few areas of necrosis. Occasional collections of histiocytes were noted, but no evidence of granuloma or malignancy was identified (Figures and ).\nThe diagnosis of sclerosing mesenteritis was made and the patient was started on a regimen of immunosuppressive drugs which included prednisolone and azathioprine, along with tamoxifen. Over the past 30 months, the patient has been on tamoxifen and azathioprine. Tamoxifen has been shown to be useful in the treatment of desmoid tumors and idiopathic retroperitoneal fibrosis []. Another reason for combining tamoxifen with the immunosuppressive regimen was that this drug is relatively safe and simple to dose.\nPrednisolone has been tapered off and restarted twice over this time, depending on the symptoms which include feeling of stretch in the abdomen and lack of appetite. Follow-up CT scans of the abdomen at 2, 6, 8, and 15 months after the biopsy revealed no interval change in the appearance of the mass. The patient had been on the specified regimen until he developed herpes zoster along the left mandibular division of the trigeminal nerve. This required stopping azathioprine. However, over a period of 6 weeks, this led to worsening of symptoms and both steroids and azathioprine had to be restarted. Currently, he is stable clinically and his steroids are being tapered off. |
A 19-year-old Caucasian female with no past medical or surgical history. She presented to the orthopedic oncology clinic three months after a sudden onset of a traumatic left ankle swelling. An outside provider placed her in a CAM walking boot for a presumed ankle sprain; her swelling eventually subsided. After removing the boot and returning to unrestricted activity, the swelling returned. An MRI was done, which showed a large distal fibular mass with characteristics consistent for Ewing Sarcoma (). She underwent a CT guided biopsy on the same day of the initial consultation, which demonstrated a small round blue cell lesion that was CD99 positive, FLI 1 positive, and FISH positive for EWSR 1, confirming the diagnosis of Ewing Sarcoma (). The Hematology/Oncology service promptly saw her, and staging studies were negative for distant metastasis (). Pre-chemotherapy echocardiogram demonstrated an ejection fraction of 62 %. She was started on a neoadjuvant regimen of vincristine, doxorubicin, and cyclophosphamide. She also underwent a pre-operative radiation therapy regimen at a total dose of 4500 cGy broken up into 25 fractions. After completing her neoadjuvant chemotherapy and radiation treatment, a repeat MRI scan showed a significant decrease in the distal fibular lesion and soft tissue component (). She continued to be followed with surveillance MRI scans for local recurrence until a decision was made between the patient, family, and treating specialties to proceed with surgical treatment.\nJust over one year from the initial presentation, she underwent en bloc resection of the distal left fibula, neurolysis of branches of both the superficial peroneal and deep peroneal nerves, local flaps, and reconstruction of the lateral ankle ligaments using the peroneus brevis (). She was immobilized in the immediate postoperative period with a three-sided short leg splint and was instructed to be non-weight bearing.\nShe did very well ten months postoperative without evidence of recurrence and intact mortise on imaging studies (MR/CT/XR). She was ambulating in a high-top shoe without discomfort or major gait disturbance. Sensation was intact distally in the foot in the sural, saphenous, superficial peroneal, deep peroneal, medial, and lateral plantar nerve distributions. Gross motor was intact in the tibialis anterior, gastrocnemius, extensor hallucis longus, and flexor hallucis longus. She was encouraged to continue supportive shoe ware and limit ambulation on uneven surfaces until 15 months post-op. She will have continued to follow up with serial imaging. |
The patient was a 66-year-old man without a smoking history. The patient had been previously healthy until he was diagnosed with GERD a month prior, and began treatment with lansoprazole. He had a history of working at a dusty place several weeks before hospitalization, where he did not experience any respiratory symptoms. However, a few days after initiation of lansoprazole treatment, cough and dyspnea developed along with a febrile sense. After suffering from a progressively aggravating cough and dyspnea for a month, the patient visited our hospital.\nVital signs of the patient were stable and the laboratory studies including eosinophil count were all in the normal ranges. An initial chest radiograph showed diffuse ground-glass opacities in both lungs with upper lobe predominance, and we diagnosed differentiated diffuse interstitial lung disease or interstitial pneumonia, such as Pneumocystis carinii pneumoniae pneumonia (). High-resolution computed tomographic scans (HRCT) showed focal areas of diffuse ground-glass opacity in both lungs with upper lung predominance, similar to those of the plain radiographs (). The patient was hospitalized for further evaluation and treatment. A transbronchial lung biopsy was performed, and the histopathological result was acute lung injury with marked type II pneumocyte hyperplasia, interstitial edema and interstitial infiltration of lymphocytes. After only one day of symptomatic treatment, both the clinical symptoms and chest radiographic findings rapidly improved. Considering his recent history of working at a dusty place, we thought that the clinical, radiologic, and histopathological findings represented hypersensitivity pneumonitis and we decided to start prednisolone therapy (30 mg/day). However, on the third day of prednisolone therapy, the patient complained of sudden aggravation of dyspnea. Blood gas analysis showed hypoxemia (SaO2 80% on room air), and the chest radiographic findings showed aggravation of diffuse bilateral infiltration. We performed a video-assisted thoracoscopic lung biopsy to decide on further treatment planning. The specimen showed a NSIP pattern with frequent Masson's bodies, active type II pneumocyte hyperplasia and mixed interstitial inflammation without hyaline membrane formation (). The next day following the operation, the respiratory symptoms and chest radiographic findings all improved spontaneously, which was difficult to explain.\nIt was difficult to explain the cause of the sudden aggravation of symptoms and the imaging findings during the hospitalization of the patient. Therefore, we carefully reexamined the clinical course of the patient during the hospitalization. It was noted that the patient stopped taking lansoprazole immediately after admission in preparation for the transbronchial lung biopsy, and he restarted taking the drug along with prednisolone after the biopsy. The patient again stopped taking lansoprazole prior to the thoracoscopic lung biopsy. Such a temporal relationship between the ingestion of lansoprazole and the worsening of symptoms led us to conclude that the symptomatic, radiological, and histopathological findings in the patient actually represented a drug-induced interstitial disease. Therefore, we stopped giving lansoprazole to the patient and restarted prednisolone therapy. The clinical symptoms and the pulmonary opacities on the follow-up chest radiographs progressively improved. The follow-up HRCT scans performed two days after the restart of prednisolone therapy showed a marked improvement of the ground glass opacities in both lungs (). |
The patient is a 50-year-old female who had a 4-year history of left lumbar radiculopathy which was precipitated from a lifting injury that did not resolve following L4-5 microdiscectomy. She had previously been unsuccessfully managed with epidural steroid injections and multimodal analgesics. A nerve conduction study was unremarkable for any pathology. She was diagnosed with failed back surgery syndrome and counseled regarding her treatment options, including spinal cord stimulation. She elected to pursue spinal cord stimulation with the Nevro Senza HF10 system. As part of her evaluation for SCS placement, the patient was seen at the behavioral medicine clinic and underwent presurgical psychological testing and evaluation by a board-certified psychologist. Her psychological assessment profile suggested a high likelihood of a good postoperative outcome. She had a history of depression and anxiety, both of which were stable and under medical management. The patient underwent an uneventful SCS trial with near 100% relief of her back and leg pain and wished to proceed with permanent implant. Her permanent placement procedure was uneventful with leads placed at the same levels as her trial leads (.) with the tip of the right lead at the top of the T8 vertebral body and left lead tip at the mid T9 vertebral body. The device was activated in the post-anesthesia care unit with bipole setting on electrodes 10 and 11.\nThe first day following her procedure, the patient noted a buzzing or a chirping sound predominately in her left ear as well as vertigo, intermittent involuntary left facial twitches, and perioral numbness, all of which were new to her. She also reported nausea, vomiting, and diarrhea, which she has attributed to anxiety in the past. She was subsequently evaluated by ENT around 4 weeks postoperatively and underwent an audiological evaluation with no abnormal findings. There was concern that the tinnitus could be related to her stimulator. She also later complained of implanted pulse generator (IPG) site pain and stinging sensations. Attempts at reprograming her SCS system with a bipole settings on leads 4 and 5 failed to resolve her ongoing neurologic symptoms. The patient was subsequently advised to deactivate her system to see if her symptoms would improve. Two days after deactivation of her system, the patient reported complete resolution of her symptoms. She was again evaluated and spine radiographs were performed which demonstrated unchanged position of her SCS leads. She was not interested in reactivating her system to assess for return of symptoms. The SCS system was subsequently explanted. At the time of explant, there were no visible defects in the SCS system. |
A 28-year-old female presented to our clinic having had symptomatic gall stones for 2 years. All her laboratory investigations were within the normal range, however an ultrasound revealed multiple gall bladder stones.\nDuring the laparoscopic cholecystectomy there was a stone impacted at the neck of the gall bladder surrounded by thick tissue.\nDuring the dissection of Calot's triangle there was bile coming into the area ( and ) so careful dissection continued until we could clarify all anatomy.\nThere was a small injury to the right accessory hepatic duct.\nAfter completing the cholecystectomy and extracting the gall bladder, flashing of the common bile duct was carried out through the cystic duct stump, after which a drain was inserted at the subhepatic area and the surgery was complete. The patient was reviewed post-operatively.\nOn day 1 post-operation the patient had no abdominal pain. The total bilirubin was 18.3(5.1–17.1), direct bilirubin was 6(0–3) and the drain was 30 mL serosangeous.\nMRCP show minimal collection at the lesser sac and no obstruction in the common bile duct or dilatation in the biliary tree.\nFollow-up laboratory investigations show normal liver function and normal WBC.\nOn day 5 post-operation the patient was asymptomatic and the drain was 10 mL serous.\nAn ultrasound was carried out and showed a collection of fluid at the epigastric area ()\nA CT scan showed a 15 × 15 cm collection of fluid at the lesser sac with the drain tube in the gall bladder bed and no collection of fluid at the perihepatic area ()\nUltrasound-guided drainage was carried out, and revealed bile.\nThe drain was kept in place for 12 days until there was no more bile and an ultrasound showed no collection of fluid.\nAfter the removal of the drain the patient was discharged.\nThe patient was followed up weekly in an outpatients' clinic for 2 months. There was no further abdominal pain, a liver function test came back normal and a follow-up ultrasound showed no collection of fluid. |
44-year-old man had a history of trauma and splenectomy dating back to 2008 due to a high fall accident injury. The patient came to our hospital for routine follow-up health check for trauma. During the interview with the doctor, the patient had complaints of fatigue, flu-like symptoms and occasionally had the sense of thoracalgia for about a week. During the physical examination, a well healed laparotomy scar measuring about 10 cm in the upper left abdomen was identified. No spleen was palpable when palpation of left costal region was conducted by the doctor. No other significant findings were identified. All laboratory findings were within normal range. Hematology and biochemistry tests were within normal range. All other laboratory findings were within normal range too. Pulmonary function tests and cardiovascular examination showed the normal state. Plain chest computed tomography (CT) with an attenuation value 52HU was performed. There were multiple nodules under the left upper lobe tongue segment, lower lobe basal segment subpleural and right diaphragmatic, partial fusion showed wavy changes with uniform density. There were clear boundary and enhanced scanning lesions, measuring up to 18 mm, suggesting suspicious for primary lung cancer. There were old fractures of the left side 9th and 10th ribs, and no right sided pulmonary lesions or mediastinal lymphadenopathy were seen on CT. Contrast-enhanced CT scans showed obvious enhancement in soft tissue masses (Fig. ).\nBased on CT imaging, the surgery of left thoracic exploration and lumpectomy of the chest wall was planned to relief symptom, remove masses. After discussed with patient. Via video-assisted thoracic surgery (VATS) was performed to remove the masses. During the operation, we found mass on the surface of the upper lobe of the left lung, and a large number of nodules that scattered on the surface of left lung surface, diaphragm and mediastinal pleura. The largest nodule was tough in texture, regular in shape, and was measured about 1.82.0 cm on pleura. (Fig. ) We completely removed this biggest mass and other masses on left pleura through thoracotomy. After careful hemostasis, a chest tube was placed in the seventh intercostal space at the mid-axillary line and attached to an underwater seal. The left lung was re-inflated under direct vision. On the third day after surgery, the chest drain was removed after minimal drainage.\nThe patient had an excellent recovery after surgery and was discharged properly. Final pathological findings: (Fig. ) on H&E slide, the mass was consisted of red pulp and white pulp of spleen with splenic corpuscle and spleen trabecular structure, which showed viable splenic tissue. Immunohistochemically, cells of masses were CK(−)、CD3(T cell+)、CD20(B cell+)、TTF-1(−)、Syn(−)、Ki-67(+,5%)、CD34(sinusoid+)、CD8(sinusoid+)、CD1a(−)、S-100(−)、CD68(sinusoid+) (Fig. ). The final pathological diagnosis was splenosis without malignancy. |
A 58-year-old male diagnosed with Hodgkin’s disease 30 years ago received mantle field and para-aortic radiation without chemotherapy. A computed tomography (CT) scan done in August 2015 showed no evidence of disease. In Jan 2017, he presented with left shoulder pain which did not respond to medications and physical therapy. CT showed a large left upper lobe necrotic soft tissue mass protruding through the chest wall between the first and the second ribs with underlying expansion of the left first rib and extension into the pectoral space and neck (). The chest wall/lung mass was biopsied via core needle in June and showed spindle cells that immunohistochemically (IHC) stained negative for AE1/AE3, S100, desmin, smooth muscle actin, and CAM 5.2. It was classified as undifferentiated spindle cell sarcoma of at least intermediate grade according to FNCLCC (). Neoadjuvant therapy consisting of AIM: adriamycin 75 mg/m2 and ifosfamide 7500 mg/m2 with mesna was started. He received two cycles of AIM in July with side effects of anemia requiring transfusion and neutropenia. CT scan in August showed minimal decrease in the size of the tumor (). After recovery from his side effects, he was given one dose of liposomal doxorubicin 30 mg/m2 with plan for surgical resection. He tolerated the liposomal doxorubicin well and on 21 September 2017, he underwent uncomplicated left chest wall resection, left upper lobectomy, and chest wall reconstruction with prolene mesh. He was discharged in good condition without complications. The completely resected specimen showed negative margins and 95% necrosis within the remaining tumor measuring 9 × 7 × 4 cm3 (). He reported feeling well until he was admitted on 19 October 2017 for shortness of breath and diagnosed with an ST Elevation Myocardial Infarction which required three drug-eluting stents and a pericardial window for a large effusion. He subsequently underwent cardiac arrest on 29 October 2017 and was on ECMO until he was decannulated on 1 November 2017. He then had a pacemaker placed on 2 November 2017. CT chest on 10 November 2017 showed no evidence of disease. His chest wall mesh from the surgery became infected with MDR Pseudomonas which required a mesh explantation on 13 November 2017. Despite maximum ICU care with mechanical ventilation and vasopressors, the patient passed away several months later. |
A 36-year-old immunocompetent female without any medical comorbidity, vascular risk factors, or drug allergy presented to the emergency department with a history of hyperacute onset of flaccid paraplegia with double sphincteric incontinence of a day's duration. She stated that she attended a hospital for her routine monthly injection of 12 lakh units of benzathine penicillin for her rheumatic heart disease. She did the report of rheumatic fever at the age of 3 years and had been diagnosed with moderate mitral stenosis, mild aortic stenosis with mild aortic regurgitation since the age of 12 years and has been on benzathine penicillin prophylaxis without any untoward events in the past.\nOne day earlier, she attended her hospital for benzathine penicillin prophylaxis and was given the IM injection at 3 PM. Although she did not notice any difficulties during the IM injection to her right gluteal region, within few minutes (5 min), as she was stepping out of the hospital entrance, she experienced a sudden burning, stinging sensation at the site of injection that progressed to the periumbilical region, followed by an almost abrupt ascending numbness in both lower limbs to her hip level, only to buckle and collapse to the floor. She was fully conscious with no features of anaphylaxis and realized that she had complete weakness of both lower limbs, developed urinary retention and had to be catheterized. While in bed, due to the pain at the injection, her daughter noticed a bluish mottled discoloration on her right gluteal region.\nThe clinical examination during her presentation to our hospital 18 hours after the onset of the neurological catastrophe revealed an alert, conscious patient with normal vital signs. Her femoral, popliteal, tibial, and dorsalis pedis pulses were palpable with no evidence of skin lesions over the limbs or perineal region; no gangrene of the toes; and no evidence of rectal bleeding.\nInitial neurological examination revealed Grade 0/5 Medical Research Council paraplegia, with flaccidity, areflexia, bilateral Babinski sign with a nondissociated pan-sensory level at T6, and double-sphincteric incontinence without flexor spasm or other evidence of multifocal neurological or meningeal deficits. The skin over the site of her IM injection revealed nonblanching, nonindurated, area of mottled erythemato-violaceous patches without a “palpable purpura” over the right superior gluteal distribution, with a reticulate pattern extending to the lateral aspect of the thigh and right lumbar paraspinal area, and with satellite lesions; the maximum diameter measuring 23 cm ×18 cm [] which the patient had corroborated to have coalesced and increased in size.\nIn view of the apoplectiform “stroke-like” paraplegia in a young female patient and unaware of “a priori” NS, especially in view of having had her regular benzathine penicillin prophylaxis, we did reexplore her medical history for vasculitis, vascular risk factors, thrombophilia, connective-tissue disorders, history of optic neuritis, or opticospinal neurological deficits in an attempt to exclude other plausible and treatable etiologies of the “unexpected” hyperacute paraplegia.\nA complete blood count (hemogram) revealed neutrophilic leukocytosis, raised erythrocyte sedimentation rate of 62 mm/1st h with a normal coagulation profile (Coombs test, antiphospholipid, and anticardiolipin antibodies), and urine examinations. Her chest X-ray, electrocardiography, blood urea, serum electrolytes, serum creatinine, and creatine kinase were normal. Tissue destruction was indicated by elevated transaminases with serum glutamic-oxaloacetic transaminase (aspartate aminotransferase [AST]) of 1018 units, serum glutamic pyruvic transaminase (alanine aminotransferase [ALT]) of 413, and serum lactate dehydrogenase of 1075 units. The results of her Venereal disease research laboratory (VDRL) and Human immunodeficiency virus (HIV)-1 and HIV-2 tests were negative. Her arterial Doppler studies in the extremities were normal. Antinuclear antibody profile, cytoplasmic and perinuclear antineutrophil cytoplasmic antibodies, rheumatoid factor anti-cyclic citrullinated peptide, and serologies (Leptospira IgM, Dengue NS1 antigen, malarial parasite, HbsAg, and HCV) were unremarkable. The cerebrospinal fluid examination was normal with no oligoclonal bands. Her nerve conduction studies were unremarkable. Magnetic resonance imaging (MRI) revealed a longitudinally extensive, centromedullary cord involvement with altered signal intensity from T7 level to conus level in T2, fluid-attenuated inversion recovery, and short tau inversion recovery sequences without contrast enhancement with normal brain and optic nerve MRI sequences [].\nSections of the skin biopsy [–] revealed epidermal and papillary dermis infarction, with edema and extravasation of erythrocytes, the vessel walls showed fibrinoid necrosis and thrombotic occlusion of the vessel lumina with progression of vascular and perivascular necrosis, neutrophilic exocytosis, apoptotic nuclei, perivascular and transmural neutrophilic, and lymphocytic infiltrate associated with karyorrhexis in the dermal vessels all of which was consistent with leukocytoclastic vasculitis (LCV). LCV is the most common form of vasculitis of the skin and usually results from the deposition of immune complexes at the vessel wall, which was partially evident in the direct immunofluorescence (DIF) microscopy in this patient. We reiterate that a diagnosis of LCV should not be made solely on the presence of positive DIF findings nor should the diagnosis be excluded with a negative DIF test. The findings of DIF should be interpreted along with clinical, microscopic, and other laboratory findings.[] It is prudent to recognize that the DIF result may be negative in a significant number of cases especially when samples are taken more than 7 days after the onset of lesions.\nShe was initiated on combination therapy with 1 g pulse methylprednisolone for 5 days (followed by tapering short course of 1 mg/kg/day of prednisolone), 40 mg enoxaparin s/c Q12 h, and pentoxifylline infusion for 2 weeks after 48 h of onset of her neurological complication. However, she did not make any improvement in her neurological status and was discharged home after 3 weeks of hospital admission. |
A 67-year-old woman came to our varicose vein center complaining of varicosities as well as edema and eczema in her left lower leg. Upon duplex ultrasound (DUS), she was diagnosed of having primary varicose vein due to the incompetent GSV, with Clinical Etiological Anatomical Pathophysiological (CEAP) classification C2s C3s C4as Ep As PR (). DUS did not demonstrate thrombosis either in the deep veins or the superficial veins. She had no history of excessive bleeding or thrombosis on her conventional open appendectomy at the age of 15 or on her delivery of two children. There was no family history of bleeding tendency or thrombosis. Since she strongly desired to have varicose vein surgery for the cure of stasis edema and eczema, preoperative examination was performed, showing prolonged APTT to 180.0 seconds (). Both bleeding time and prothrombin time were within the normal range. To investigate the cause for abnormal APTT, she was referred to the department of hematology in a university hospital near our varicose vein center. The absence of medical history with bleeding tendency indicates that she does not have hemophilia (congenital or acquired) or von Willebrand disease. Further blood examinations demonstrated that FXII activity was 1% (normal range, 60%–140%) (). Other coagulation parameters were within the normal range. Based on these results, she was diagnosed of having severe (less or equal to 1% of normal) FXII deficiency. Our varicose vein center did not have any experience of surgery for patients with FXII deficiency. In addition, through literature search in PubMed, no reports are available on varicose vein surgery for patients with FXII deficiency. On the other hand, her attending hematologist approved for her to undergo varicose vein surgery without any treatment for FXII deficiency. Moreover, she had experienced conventional open appendectomy and vaginal delivery without any hemorrhagic or thrombotic complications. Taken together, because she strongly desired to have surgery for the varicose veins, we planned ETA for the incompetent GSV. Under local anesthesia combined with tumescent local anesthesia (TLA), ETA was performed with the use of a 1470-nm diode laser (ELVeS 1470, Integral Corp., Tokyo, Japan) and radial two-ring fiber (ELVeS Radial 2ring™ fiber, Integral Corp.). The ETA procedure was based on the reports of others. The optical fiber was inserted at the distal side of the GSV through a 6-Fr sheath by DUS guidance. After TLA, from the point 2 cm distal from the saphenofemoral junction, the GSV in the thigh portion was ablated. The length of the ablated GSV was 27 cm, and the average linear endovenous energy density was 81.1 J/cm. We did not perform concomitant phlebectomy for the varicosities in the calf. Immediately after the procedure, a compression bandage was applied to the thigh and calf. These procedures were performed as a day surgery. She was followed up on an outpatient basis, and DUS was performed 1 day, 1 week, 1 month, 3 months, 6 months, and 1 year after the operation. Either deep or superficial vein thrombosis was not found postoperatively. On the first postoperative day (POD), DUS showed class 1 endovenous heat-induced thrombus (EHIT), which disappeared thereafter. Her postoperative course was uneventful, except for the tiny ecchymosis in the TLA area on the first POD (). |
A 65-year-old Caucasian female had a past medical history significant for bilateral fibrocystic breast disease resulting in bilateral subcutaneous mastectomy, followed by bilateral cosmetic breast reconstruction with textured silicone gel implants at age 30 (). Subsequently, she had multiple complications from the implants including capsule contractures, infections, chronic seroma, and ruptured breast implants, leading to capsulectomy and implant replacements 15 and 22 years post original implantation. During these periods, the patient was noted to have several areas of calcifications in both breasts (L > R) that had been monitored with routine mammography. The patient noted that at 30 years post original implantation, her left breast became edematous; however, this self-resolved a few months later. Two years later, edema was noted again in the left breast and confirmed by MRI ()], resulting in a third replacement of the silicone gel implant. Recently, at 35 years post original surgery, the patient presented with swelling in the left breast which progressively worsened over 2–3 months. A targeted ultrasound examination of the left breast at the approximate twelve o'clock position, left axilla, and of the right breast at the ten o'clock position over areas of concern demonstrated no discrete cystic or solid abnormalities (). Unremarkable parenchyma was observed throughout the entire region. However, given the extent of edema in the left breast and associated pain, bilateral total capsulectomy was performed for a fourth time.\nBreast tissue obtained during surgery was sent for pathologic evaluation. The left capsulectomy specimen revealed a thickened fibrous capsule with chronic inflammation, consisting of small lymphocytes, eosinophils, plasma cells, and macrophages. The luminal surface of the breast capsule showed fibrin deposition with a thin row of highly atypical cells. The atypical cells were large and pleomorphic, with hyperchromatic nuclei and occasional prominent nucleoli noted along with abundant clear to slightly eosinophilic cytoplasm (). Immunohistochemical analysis demonstrated strong CD30, CD43, and MUM1 expression, while EMA was weakly positive (). The cells did not express ALK (), CD20, CD79a, or estrogen receptor. The overall morphology and immunohistochemical profile were diagnostic for breast implant–associated anaplastic large cell lymphoma.\nA bone marrow evaluation, including flow cytometry studies, showed unremarkable trilineage hematopoiesis without evidence of involvement by lymphoma or metastatic malignancy. Cytogenetic examination of the bone marrow revealed twenty metaphase cells with a normal female diploid karyotype with no consistent numerical or structural chromosome aberrations. Computed tomography (CT) scans of the neck, chest, abdomen, and pelvis with IV and oral contrast were essentially negative for any malignancy or lymphadenopathy except for the noted fluid collection in the left breast measuring 12.9 × 2.8 × 10.1 cm (). Whole body positron emission tomography–computed tomography (PET/CT) scan with fluorodeoxyglucose (FDG) radiotracer revealed increased FDG uptake along the anterior chest wall, slightly greater on the right than the left, with a maximum SUV of 4.7 and 4.1, respectively. No other area of increased FDG uptake was noted. Given the in situ subtype of ALCL noted in our patient, she underwent capsulectomy with no other local or systemic therapy. She remains clinically well after 12 months follow-up under close surveillance with our clinic. |
We describe the case of a 45-year-old Hispanic man who presented to the psychiatric emergency room on account of depressed mood and forgetfulness. He was found by his niece sitting in the bathroom batting away imaginary flies and crying, stating that he could not remember anything which prompted his niece to call emergency medical services (EMS).\nThe patient was emotionally labile and could not remember his name or address at the time of presentation. He was hyperverbal and difficult to interrupt, and his speech was disorganized. The patient stated that prior to admission, he left his home and suddenly could not remember how he got to the location he had traveled to. He then returned home and entered the bathroom to look for a belt to hang himself with, because he could not remember any of the evening's events. He stated that he felt lonely and helpless and that he had suicidal thoughts. The patient stated that his sleep had been poor. He endorsed a perceptual disturbance of seeing fleas that were trying to infest his body. He also endorsed an auditory hallucination of a male voice calling his name. Collateral information from his niece, who called the EMS, revealed that the patient had been acting bizarre with two previous episodes of new-onset wandering behavior in the past six months, both associated with heavy alcohol use. She also reported that the patient had a 15-year history of schizophrenia and that he had had similar episodes in the past, which were usually brief and resolved without the need for hospitalization. During a similar episode three years ago, the patient began attacking his family members and was hospitalized after the police were called. The patient also received a diagnosis of major depressive disorder five years ago. The patient was admitted to the inpatient psychiatry unit with a diagnosis of major depressive disorder. Urine toxicology at the time of admission was negative for controlled substances, illicit drugs, and alcohol. The patient's admission Complete Blood Count (CBC) and kidney liver function tests were within normal limits. Rapid regain test was negative. Serum sodium and potassium were 138mmol/L (136–144.0mmol/L) and 4.4mmol/L (3.6–5.1), respectively. Other routine urine analyses and coagulation profiles were also within normal limits as were routine chest radiograph and ECG. Serum thyroid stimulating hormone was below the lower limit of normal 0.409 uIU/ml (0.450–4,500 uIU/ml) and free T4 was 1.09 ng/ml (0.82–1.77 ng/dL). The patient had no symptoms of hyperthyroidism.\nOther chemical laboratory investigations were within normal limits except for dyslipidemia. Computerized tomography (CT) and magnetic resonance imaging (MRI) performed during admission revealed partial agenesis of the corpus callosum with the absence of the posterior body and the splenium as shown in .\nOn day 1 of hospitalization, the patient was hyperactive and restless on the unit. He was treated with escitalopram 10 mg PO daily and risperidone 2 mg PO BID. By day 2 of hospitalization, the patient was able to recall his name and his perceptual disturbances resolved, but he was still hyperverbal, with increased activity. By day 9 of hospitalization, the patient's condition had stabilized, and he was discharged.\nAccording to the patient's mother, the pregnancy was reported to be complicated at five months, and the patient was born at seven months. He had normal gross motor development, but language was delayed until the age of 7 years. The mother reported a history of cognitive developmental delay and intermittent behavioral disturbances that led to his dropping out of school in fifth grade. |
A 62-year-old woman was referred to our neurosurgical outpatient service with abnormal sensation in her trunk, arms and legs. The patient had a past medical history of a gastric ulcer, a right ankle plating after fracture 20 years ago and a headlice infection 6 months before first presentation. She was a smoker, social drinker of alcohol and was not taking any regular medication. She lived alone and had been struggling to manage independently.\nSince her symptoms commenced 3 years previously, the patient had presented to emergency department (ED) on 11 occasions. Her primary complaint was of dysaesthetic sensory symptoms including a feeling of water retention and a gel infiltrating the skin of her face, trunk, arms and legs, feeling there was something stuck on her skin and feeling her hair was stuck down. In the weeks before the onset of her symptoms, the patient started using an olive oil moisturising cream, to which she attributed her symptoms.\nWhile dermatological examination was conducted, comprehensive neurological examinations were not documented during the first presentations to the ED, which focused on the patient’s facial dysaesthesia.\nThe patient was frustrated that her symptoms were repeatedly dismissed as delusional by the ED staff. She refused assessment by liaison psychiatry and mental health review by her general practitioner. The patient was felt to have capacity throughout all consultations. The patient was not taking any psychiatric medications.\nOver time her symptoms progressed. She started to complain of back pain, multiple falls and episodes of her right leg giving way which had progressed to severely compromised walking and coordination. The patient also complained of episodes of urinary and faecal incontinence. This led to a lumbar MRI which ruled out cauda equina syndrome. In her final presentation before referral, she also complained of stiff legs, difficulty walking, difficulty passing urine, reduced manual dexterity and neck pain. A neurology assessment was finally sought and an MRI for suspected myelopathy was organised.\nOn presentation to our neurosurgical clinic, the patient’s complaint remained sensory dysaesthesia from her neck down, particularly affecting her hands and groins. She complained of her body feeling like a ‘wet gel-like substance’. In addition to the above symptoms, the patient also complained of a 3-year medical history of numbness and tingling in her upper limbs.\nOn examination in the neurosurgical clinic, the patient had hyperaesthesia from the neck down, most prominent in the hands and groins. The patient had reduced grip strength of 3/5, finger extension and abduction of 3/5, wrist flexion and extension of 3/5, elbow flexion of 4+/5, elbow extension of 4/5 and shoulder abduction of 4+/5 on the Medical Research Council power scale. Hip flexion was 3/5, with all other muscle groups in the lower limb at 4+/5. The patient had very brisk reflexes with pathological spreading in the upper and lower limbs. There was self-limiting clonus in the ankle bilaterally. Hoffmann’s and Babinski reflexes were positive bilaterally. There was a severe loss of coordination and balance. Gait was impaired; the patient required a frame to mobilise. The severity of the patient’s cervical myelopathy was scored as 1 (upper limb motor dysfunction) +3 (lower limb motor dysfunction) +1 (upper limb sensory dysfunction) +1 (sphincter dysfunction) =6 (severe myelopathy) on the modified Japanese Orthopaedic Association scale (mJOA). |
On January 4th, 2018, a 57-year-old woman was hospitalized in the department of Psychiatry, Sir Run Run Shaw Hospital because of pain and acid bilge in multiple sites of her upper body for more than 1 year. Over a year ago, the patient started feeling pain and discomfort in the upper left abdomen, and the pain got worse when coughing but with no other discomfort. Two months later, the upper left abdomen pain and acid bilge extended to the front chest, back, abdomen, and upper limbs. The symptoms persisted for months, and aggravated when changing body posture. Test results including cervical MRI, chest CT, abdominal B ultrasound of upper abdomen in a local hospital showed no abnormalities. Treated with Chinese medicine for more than 3 months, there was no significant improvement. About 6 months ago, the patient came to our hospital, expressing the symptoms above and worries about them, with weight loss of about 1–1.5 kg, but denying continuous depression, anxiety, and other symptoms (the score of 24 items of Hamilton Rating Scale for Depression was 12, and Hamilton Anxiety Rating Scale score was 11), and was diagnosed as “somatic symptom disorder.” After 4 months of treatment with 60 mg of duloxetine enteric-coated capsules twice daily and hypnotic drugs, the symptoms were obviously alleviated but not completely relieved and there was a significant weight loss of about 5 kg. Therefore, medication was adjusted to escitalopram tablets 20 mg once daily. Two months later, the patient felt no further improvement.\nWith hypertension history of more than 10 years, the patient claimed that it's not necessary for her to take any antihypertensive drugs to control blood pressure in recent 1 year. She had bronchitis for 12 years but no medicine was needed. She denied any history of diabetes, heart disease and other diseases and claimed there was no history of surgery and trauma. Also, the patient denied long-term chemical substances, drug or poison exposure history and had no history of smoking and drinking alcohol.\nAfter admission, due to long-term poor efficacy of the patient, we re-evaluated the patient's physical condition to rule out organic diseases. However, through Blood routine, Blood Biochemistry, Stool Routine, Urine Routine, Chest Film, Electrocardiogram, and so on, no specific abnormality was found. We found that the patient's tumor marker CA-153 was 61.2 U/mL (< 25.00 U/mL) and ferritin was 198.70 ug/L (13.00–150.00 ug/L), with no specificity. There was another finding of patient suffering from cholecystitis and gallstones through abdominal ultrasound examination; however, the surgeon suggested that it could not explain the patient's symptoms. When perfecting cranial MRI, we unexpectedly discovered below result: diffuse thickening of the skull and increasing signal intensity. Metastasis? Multiple myeloma? (Figure ).\nAnd lumbar MRI prompted lumbar vertebra, attachment and right iliac bone multiple bone changes, multiple myeloma? Transfer? (Figure ). Skull and pelvis plain radiographs prompted skull, maxillofacial bone, pelvis, and femoral bone changes, multiple myeloma? Transfer? (Figure ).\nAfter perfecting corresponding blood examination, the patient eventually underwent bone marrow aspiration and the results suggested that the patient was suffered from multiple myeloma (Figure ). The patient was finally referred to the hematology department and received appropriate treatment.\nHere we report on a case of a 57-year-old woman with pain and discomfort in multiple sites of upper body who was diagnosed as somatic symptom disorder after completing a partial examinations of relevant parts which turned out to be negative. Finished imageological examinations of all painful parts, she was eventually diagnosed with multiple myeloma after 6-month being misdiagnosed as somatic symptom disorder. This case highlights the importance of completing imageological examinations of all the painful parts of the patient to exclude the possibility of multiple myeloma especially when symptoms are associated with objective signs and treatment has been ineffective; and it is as well as significant to notice characteristics of symptoms and to pay excessive attention directed toward the symptoms in the diagnosis of somatic symptom disorder.\nMM is a disease which is characterized by the neoplastic proliferation of immunoglobulin-producing plasma cells. Most patients with MM present with signs or symptoms related to the infiltration of plasma cells into the bone or other organs or to kidney damage from excess light chains. MM accounts for ~1–2% of all cancers and slightly more than 17% of hematologic malignancies (). Worldwide, there are ~154,000 cases and 101,000 deaths per year attributed to MM (). MM is also slightly more frequent in men than in women (~1.4:1). The risk of developing MM increases with body mass index (, ). MM is a disease of older adults. The median age at diagnosis is 66 years; only 10 and 2% of patients are younger than 50 and 40 years, respectively (, ).\nMost patients with MM present with signs or symptoms related to the infiltration of plasma cells into the bone or other organs or to kidney damage from excess light chains. As an example, a retrospective analysis of 1,027 sequential patients diagnosed with MM at a single institution found the following symptoms and signs at presentation: Anemia-73%, Bone pain-58%, Elevated creatinine-48%, Fatigue/generalized weakness-32%, Hypercalcemia-28%, Weight loss-24%, one-half of whom had lost ≥9 kg ().\nIn American Psychiatric Association's Diagnostic and Statistical Manual, Fifth Edition (DSM-5) (), SSD is characterized by one or more somatic symptoms that are accompanied by excessive thoughts, feelings, and/or behaviors related to the somatic symptoms. It is estimated the prevalence in the general population is 4% (, ) and that among primary care patients is 17% (, ). An analysis of individual patient data from nine community studies (total n > 28,000) found that the most frequent burdensome symptom was pain (). SSD is not defined by the number of distressful physical symptoms that are present; however, patients who complain about multiple symptoms are more likely to have the disorder. In this case, the patient had a number of pains and acid bilge in multiple locations which are typically present in somatic symptom disorder, with no other symptoms of MM, for instance, anemia, elevated creatinine, fatigue/generalized weakness, hypercalcemia. These enhance the possibilities of misdiagnosing MM as SSD. The percentage of underlying somatic diseases in patients previously diagnosed with SSD is relatively small but unneglectable. A meta-analysis () reviewed six diagnostic evaluation studies (total N = 1,804 patients), 16 follow-up studies (total N = 2,440 patients), and the percentage of misdiagnosis with SSD was 8.8% (95% CI 1.0–22.2, p = 0.007) in diagnostic evaluation studies, 0.5% (95% CI 0.01–1.5, p = 0.03) in follow-up studies, while the correct diagnosis shall be diabetes mellitus, duodenal ulcer, Crohn's disease, polymyalgia rheumatica, carcinoma, herniated disc, and so on.\nImaging is a key part of the evaluation of all patients with suspected MM. In this case, we found some related negative imaging test results like cervical MRI, chest CT, abdominal B ultrasound of upper abdomen from another hospital, however, neglected to do examinations of other important parts where the patient reported discomfort, such a lumbar and pelvis imageological examinations. Pain and acid bilge in multiple sites are usually associated with musculoskeletal and nervous system disease, and MRI is the best imaging choice for the early stage of these diseases. In the diagnosis procedure of SSD, thorough physical examination, laboratory tests and imageological examinations are necessary to help clinicians and patients build confidence and ensure that no important diagnosis will be missed (–). Moreover, criteria for selective use of tests include objective signs rather than the volume of the concerns expressed by the patient, the presence of complex symptoms, and persistence of symptoms (). For instance, in our case, the pain of the patient aggravates when the body posture is changed or coughing. This characteristic probably points to a physical disease which is ignored during the early processes of out-patient treatment.\nSSD patients always have excessive thoughts, feelings, or behaviors associated with the somatic symptoms. The patient was also anxious because of her symptoms which now we can consider it as healthy anxiety. Clinicians taking a history should determine whether somatic symptoms trigger healthy anxiety or not, in addition, determine whether the patient manifests persistent thoughts and anxiety related to the somatic symptoms, and whether the patient devotes excessive time and energy to the somatic symptoms (–). In International Classification of Disease-10 (ICD-10) () which is currently widely used in the world, somatoform disorders are defined on the basis of failure to find physical causes rather than the presence of definite psychological and behavioral features. The notion of taking medically unexplained symptoms as the defining feature of ICD-10 somatoform disorders creates a major hindrance to the clinical utility of the diagnosis. There is evidence that the decision about whether symptoms are medically unexplained is unreliable and lacks validity. The inherent dualism in the notion of a lack of medical explanation for somatic symptoms that are cross-sectionally assessed is simplistic and ultimately unhelpful to patent care (). In ICD-11 (), excessive attention directed toward the symptoms is highlighted in the diagnosis of Bodily distress disorder.\nThere is evidence that antidepressants are effective for SSD (, ). However, SNRIs could relieve pain by inhibiting reuptake of serotonin and norepinephrine, and suppressing painful sensation uploading regardless of physical disease or psychiatric disorders. Therefore, pain of the patient was obviously alleviated after 4 months of treatment with 60 mg of duloxetine enteric-coated capsules twice daily. This phenomenon also could perplex the revision of the diagnosis.\nThis case indicates that imageological examinations of all the painful parts of the patient should be completed to exclude the possibility of MM, especially of those whose symptoms are associated with objective signs and treatment has been ineffective. Furthermore, diagnosis of SSD requires not only the elimination of somatic diseases, but also excessive thoughts, feelings, or behaviors associated with the somatic symptoms. |
A 12-year-old female patient presented to the Department of Orthodontics in with a chief complaint of impaired esthetics. She presented with a dental history of feeding problem due to oronasal communication. Cheiloplasty and palatoplasty were performed at 5 months and 12 months of age, respectively. Her prognosis was poor according to Goslon Yardstick.[] Extraoral examination revealed a concave profile with a Class III skeletal pattern having a maxillary hypoplasia. Intraoral examination revealed that there is a prominent oronasal fistula perforation seen on the left side within palatal scar tissue. She had a Class I molar relationship on the right and left sides, anterior crossbite with reverse overjet of 4 mm, Unilateral open bite on the left side, palatally positioned 12, partially erupted 22 and incisors are in Class III relationship []. The mandibular arch is relatively undisturbed.\nCephalometric analysis showed Class III skeletal pattern with relatively retrognathic maxilla, vertical growth pattern, clockwise rotation of maxilla and mandible, retroclined and retruded upper incisors, normally inclined lower incisors and Class III soft-tissue profile [ and ].\nThe main treatment objective was to improve the facial esthetics and profile by correcting the Class III skeletal pattern and alignment of maxillary and mandibular dentition. Anterior maxillary DO was planned using an intraoral device to obtain correct skeletal relationship and adequate arch length to align the maxillary teeth. The main advantage of this procedure is decrease in the amount of relapse, because of soft-tissue histogenesis along with incremental traction of anterior maxilla.\nLe Fort I osteotomy and anterior maxillary osteotomy are discussed as treatment alternatives but ruled out considering the amount of bone shift required to correct the crossbite.\nThe treatment was started when the patient is at 12 years 7 months of age. Surgery was aimed to provide alveolar bone support in the region of 22, 23 through autogenous bone graft raised from anterior iliac crest. The cleft region was untouched for 6 months for the graft take up []. After this secondary graft placement orthodontic correction was carried out to align upper and lower the teeth. The period of presurgical alignment and leveling was about 6 months. The surgical site for DO was decided in the interdental region between the maxillary first and second premolars on both sides. This surgical site provides the advantage of avoiding maxillary sinus exposure and taking more anchorage support from second premolars and molars on either side. Treatment simulation was made using dolphin digital imaging software (Paterson and Kelly, version 11.2) showed 7 mm advancement is required to achieve the adequate amount of overjet. To avoid the relapse after the surgery, it is planned to advance up to 9 mm [].\nAfter the treatment simulation was finalized, a tooth-borne distraction device was custom made using a hyrax screw positioned in anteroposterior direction. The posterior anchorage unit was modified into an occlusal bite plane with cold cure acrylic. Anterior segment was consolidated by making a cap splint to prevent the cleft segment from parting away [].\nOsteotomies for the anterior maxillary distraction were carried out under general anesthesia. Horizontal corticotomy cut was sited from premolar to premolar region 5 mm above the root apices. Vertical corticotomy cut was made interdentally between premolars on either side. Vertical cuts are extended to the palatal region on either side, and a greenstick fracture of the anterior maxillary segment had been done. Mucoperiosteal flap was closed primarily, custom-made distraction device was cemented to the teeth using glass ionomer cement and checked for its activation [].\nAfter a latency period of 5 days, distraction was started from the 6th day after the surgery and was carried out at a rate of 1 mm per day with a rhythm of 0.5 mm twice a day. Distraction is done for 9 mm and the consolidation period took 2 months. Bone formation in the distraction site was confirmed on radiographs. At the end of this consolidation period, the distractor is removed [].\nPostsurgical orthodontics was performed by placing the brackets on the maxillary teeth. Lateral open bite was developed in the region of 22, 23, and 24. This lateral open bite was corrected using the box elastics. The first premolar on either side is completely distalized and is included in the anchor unit. This is followed by distalization of canines on both sides. Palatally blocked out 12 is slowly aligned into this space. Left lateral incisor was in the cleft region; hence, very mild forces are used to derotate it [].\nRetention is provided using upper fixed retainer from premolar to premolar and a Begg wrap on retainer, lower retention is attained by fixed retainer from canine to canine. Superimposition of the pre- and post-treatment lateral cephalograms showed a linear movement of maxillary anterior segment by 7 mm [ and ].\nThe results showed an improvement in skeletal, dental, and soft-tissue parameters. Anterior crossbite has been corrected. Overjet is improved from −4 mm to +2 mm. ANB angle was increased from −4° to 1°. Angle of inclination increased from 74° to 87°. Superimposition of the pre- and post-treatment lateral cephalograms showed a linear movement of maxillary anterior segment by 7 mm. Upper incisor was proclined little more than the ideal inclination to achieve acceptable interincisal relationship. The follow-up retention after 24 months showed a stabilized skeletal pattern with no signs of relapse [Figures and ]. |
A 5-year-old boy with a height of 110 cm and body weight of 18 kg complained of a mass detected at the middle dorsum of the tongue, and thus, he was referred to the Department of Oral Surgery of Nagoya City University Hospital by a family dentist. The patient would snore when laid on his back while sleeping. Detailed examination revealed a 35 × 25 × 25-mm mass lesion on the dorsum of the tongue (Figures and ). His medical history showed that although he was being followed up by a local pediatrician for congenital adrenal hyperplasia, the follow-up had been completed at the time of this operation. The patient had no allergies or any family history of diseases, and preoperative examination revealed no particular findings. Under general anesthesia, resection of the mass lesion located at the dorsum of the tongue was planned.\nRegarding anesthesia management, these two concerns were considered: (a) a difficult airway due to the large mass present on the tongue and (b) the risk of airway obstruction associated with a swollen tongue postoperatively.\nBefore anesthetizing the patient, a peripheral intravenous route was secured on the left forearm to prepare for the difficult airway that was expected after the induction of anesthesia. Prior to the induction of anesthesia, oxygenation was administered with 100% oxygen for approximately 3 minutes. Anesthesia was induced with 6 L/min of oxygen and 6% sevoflurane. After the onset of anesthesia, mask ventilation temporarily became difficult due to the tongue mass and glossoptosis; however, mask ventilation was feasible after administering 15 mg of rocuronium and using an oral airway. Decreased peripheral capillary oxygen saturation associated with temporary dyspnea was not observed. Subsequently, 30 μg of fentanyl was administered, and after the muscle relaxant showed its effect, nasotracheal intubation was performed using the McGRATH™ MAC (McGRATH). The dorsal mass of the tongue was mobile. Hence, the tongue could not be completely removed from the field of view due to the mobile mass although the tongue has been removed from the field of view during intubation. However, the view with McGRATH showed a Cormack–Lehane Grade 2, and intubation was successfully performed during the first attempt. Anesthesia was maintained at 1 L/min of oxygen, 2 L/min of air, 2% sevoflurane, and 0.1-0.2 μg/kg/min of remifentanil. Intraoperatively, 2 mg of dexamethasone was administered to prevent postoperative tongue swelling, and 200 mg of acetaminophen was administered for postoperative analgesia. Intraoperative findings included no tongue swelling at the end of the surgery. Therefore, extubation was considered possible in the operating room. After completing the surgery, 50 mg of sugammadex, a muscle relaxant antagonist, was administered, and the patient was extubated after confirming body movement, eye opening, and sufficient spontaneous respiration. After extubation, no problems were observed with the patient's respiratory condition. The total surgical duration was 1 hour 27 minutes, with the time spent under anesthesia of 2 hour 13 minutes and amount of blood loss of 6 g.\nIn the postoperative intensive care unit (ICU), dexmedetomidine (0.4 μg/kg/hr) was administered for sedation, and additional dexamethasone was administered to prevent tongue swelling. The patient was managed in the ICU until the next morning, but no tongue swelling was observed. Then, the patient was discharged from the ICU due to stable respiratory status and vital signs. |
A 25-year-old married male came to the outpatient psychiatry clinic with his mother as an informant with a total duration of heroin use of about two years. He complained of runny nose, diarrhea, headache, and cramping pain in his legs in outpatient settings. The clinical opiate withdrawal scale (COWS) score was 13, indicating moderate opioid withdrawal. Interestingly, he also complained of insects crawling over his chest. On further questioning, he described insect color and size. He also reported that he kept hearing the voice of a female who lived in his neighborhood instructing him to do things, but his mother denied such instances and said no such female lived in their neighborhood. The patient was admitted to the psychiatry inpatient department. During his inpatient stay, care was provided according to the hospital protocol. In addition to three meals in the form of breakfast, lunch, and dinner, the patient was provided with snacks between the meals. Family visitation was granted during the day but they were not allowed to stay in the hospital. The patient and his family members complied with hospital policies and staff. Relationship between patients and family members was cordial before and during inpatient hospitalization. His blood investigations were within normal limits, but his electrolyte report showed hyponatremia with a serum sodium level of 131 mEq/L (normal range: 135-145 mEq/L). The patient was admitted and started on buprenorphine 4 mg tablets in divided doses, diclofenac 100 mg tablet on a pro re nata basis, and olanzapine 2.5 mg tablet at bedtime in the night. After two days in the ward, his COWS score reduced to four. At this time, he only complained of anxiety and yawning symptoms. However, he reported a continuance of psychotic symptoms. He reported that a female voice still ordered him to run from the ward and do some rituals for her. He also said that insects are all over his hair and chest. The patient also described the female wearing a blue saree on further exploration, which he saw mainly during afternoons only for a few minutes.\nDue to persistent hallucinations and delusions, olanzapine dose was increased to 10 mg. Within the next three days, visual hallucinations were not reported, but the patient reported that insects were still continuously troubling him by causing itching all over his body. Therefore, computed tomography scan of the head was scheduled to rule out the organic cause of psychotic symptoms, but the patient’s family denied consent due to financial constraints and low economic status. After stabilizing the patient, he was discharged on buprenorphine 2 mg tablet, olanzapine 12.5 mg tablet, and clonazepam 1 mg tablet once daily for ten days. After ten days of follow-up, the patient reported delusional parasitosis, with very few fragmentary auditory hallucinations. The patient’s mother reported excessive eating, sedation, and mild dizziness with no withdrawal symptoms; COWS score was zero. At this visit, olanzapine was discontinued, and the patient was started on 2 mg risperidone twice daily, and a two-week follow-up was advised. After two weeks, the patient reported about 50% improvement in the delusion of parasitosis. Risperidone dose was increased to 3 mg twice daily, with buprenorphine 2 mg once daily and clonazepam 0.75 mg once daily. A follow-up visit was scheduled after two weeks. The patient didn’t report for further follow-up, but the mother came to the outpatient clinic for his medications. According to the mother, the patient showed about 75% improvement on risperidone and complied with medications. The unique clinical finding of psychotic symptoms after opioid withdrawal is uncommon in literature and clinical practice compared to other substances. This case provides more clarity in terms of dual diagnosis in our routine practices. |
A 15-year-old female with a past medical history of obesity presented to the emergency department with a marked 90-degree flexion at the thoracolumbar region. She was able to walk only by using a walker in an unusual manner; she placed her axillae on the hand grips. The patient described constant back pain almost daily for which she took ibuprofen with moderate relief. These symptoms began three years ago following a sexual assault and have progressed significantly. As per the patient's mother, the only time the patient's spine straightened out was during deep sleep. She had no neurologic complaints, specifically denying any sensory or motor deficits, fatigue, ptosis, or any complaints consistent with any form of endocrinopathy. Her family history included siblings with epilepsy, ADHD, and mood disorders.\nThe patient denied any substance abuse, including use of alcohol or cigarettes, as well as being sexually active. She reported no history of mood or psychotic symptoms and no suicide attempts.\nDespite the pain, however, the patient was not concerned with her condition. The patient herself was not interested in physical therapy or additional diagnostic testing or counseling despite her mother's encouragement. She refused a gynecological examination due to anxiety following having been a victim of sexual assault; it was unclear the extent of counseling, if any, she received following the assault.\nThe patient was a seventh grader at a local school where she participated in extracurricular activities. She reported being well accepted by her peers. She stated having a good social support network including friends and family. Her parents were separated and she lived with her mother and brother.\nPhysical exam revealed an obese (BMI 39.2) Caucasian female, leaning forward with the spine nearly ninety degrees at the waist. Extremities showed normal strength, sensation, and range of motion. The patient was unwilling to attempt spinal extension. Tenderness to palpation of the paraspinal muscles was present. The rest of the physical examination was unremarkable.\nUpon Pediatric Neurology consultation, no evidence of neuromuscular disease was found. Plain radiography of the cervical, thoracic, and lumbar spine was limited due to her deformity, but no abnormalities were noted. Magnetic resonance imaging of the same was unrevealing. Psychiatric consultation was obtained; they concluded that the patient's symptoms were likely manifestations of Conversion Disorder, following exclusion of organic causes.\nWhile she is currently receiving social services support and physical therapy; she declines psychiatric outpatient care at this time. |
We present a case of a 51 year-old white female with a 5-year history of ulcerative colitis. She has been taking mesalamine 1.2 gram (2 tablets two times a day) for 1 year with no complications that were reported. No history of allergies and no history of smoking or alcohol abuse were present. Past medical history was only remarkable for ulcerative colitis and there was no significant past surgical or family history. Around March 2018, she started having increased watery diarrhea with occasional blood (10-12 bowel movements per day from a baseline of 1-2 bowel movements per day) as well as cramping abdominal pain. She went to see her gastroenterologist in clinic. On physical examination, she had diffuse tenderness to palpation of her abdomen. Stool studies including stool cultures, stool ova, and parasites were sent which were negative. ESR and CRP levels were elevated. Therefore, she was thought to be in a moderate to severe ulcerative colitis flare based on the current criteria and was prescribed budesonide multimatrix (MMX) 9 mg once daily. Her abdominal pain improved but the diarrhea persisted. She then received a course of oral prednisone 40 mg daily for one month without any improvement of her symptoms and was subsequently started on infliximab therapy. Prior to initiating infliximab therapy, an interferon gamma release assay, hepatitis panel, varicella zoster antibody, and HIV tests were done which were negative. On 4/13/2018, she received her first dose of infliximab 500 mg based on her weight of 100 kg (5mg/kg). Her symptoms got better during the first week after the infusion; however during her second week, she complained of nonradiating chest pain located at the midsternal region, shortness of breath, and worsening fatigue. She went to a hospital where she was admitted. Her initial vitals were significant for low to normal blood pressure and a persistent tachycardia of up to 110. EKG was negative for any acute changes and a CT-PE was also negative for pulmonary embolism but showed a moderate size pericardial effusion. She was given fluids with no change in the blood pressure, and she continued to remain hypotensive and tachycardic and was eventually transferred to another hospital for concerns of a cardiac tamponade. At the other hospital, a transthoracic echocardiogram was done that showed an ejection fraction of 65-70% and a small to moderate size pericardial effusion that was present more anteriorly and less prominently on the apical, inferior, and subcostal views (Figures and ). There were no echocardiographic criteria for cardiac tamponade. Based on the difficult anatomical location of the effusion, decision was made to medically manage the patient.\nShe underwent extensive workup to evaluate the etiology of her pericardial effusion. Viral causes including HIV, monospot test were negative. T-spot was also negative. Due to concerns for a drug-induced lupus from infliximab, ANA and ds-DNA were checked, which were negative. Antihistone Abs were 1.9 (positive). ESR was 70 and CRP was more than 190. There were no signs of serositis, oral ulcers, photosensitivity, blood disorders (leukopenia, anemia, and thrombocytopenia), neurologic disorder, or rash (malar or discoid).\nThe clinical presentation was not compatible with any other pathology and based on the specified time frame of the presentation, a diagnosis of infliximab induced lupus was made and patient was taken off infliximab therapy.\nHer infectious workup for diarrhea including stool culture, stool ova and parasite, and clostridium difficile were negative. A procalcitonin level was also negative. She was in a moderate to severe ulcerative colitis flare and was therefore started on IV methylprednisolone 60 mg daily for 3 days and then transitioned to PO prednisone 40 mg daily. Her shortness of breath and fatigue got better and, after discharge, her diarrhea frequency went back to baseline. After she completed her prednisone taper, she was planned for vedolizumab (antagonist to α4β7 integrin) therapy for her ulcerative colitis. Vedolizumab is not shown to be associated with drug induced lupus [] and that is why it was chosen for our patient. She got induction therapy with IV vedolizumab 300 mg at weeks 0, 2, and 6 and was then continued on maintenance therapy with IV vedolizumab 300 mg every 8 weeks. She did not receive infliximab therapy in the future. Post discharge, serum anti-TNF alpha antibodies were checked which were negative. |
A 27-year-old female, gravida 4 para 3, presented with a persistent umbilical mass from over a year. She had previously undergone three low-transverse cesarean sections and had one miscarriage. She reported constant sharp pain in the mass as well as associated increasing size and worsening discomfort, particularly with her menses. The mass was also reported to have sporadic drainage that ranged from cloudy to clear. Her medical history was remarkable for dyspareunia and intermittent pelvic pain. The patient agreed to participate and was explained the nature and objectives of this study, and informed consent was formally obtained. No reference to the patient's identity was made at any stage during data analysis or in the report.\nPhysical exam revealed a 2.5 cm firm and indurated subcutaneous mass in the center of her umbilicus, with extreme tenderness to palpation. Ultrasonography revealed an indeterminate solid and cystic lesion in the subcutaneous soft tissues at the umbilicus suggestive of a urachal cyst (Figure ).\nA provisional diagnosis of a urachal sinus tract with a cyst was made. The patient consented to an excision of the umbilical cyst with a diagnostic laparoscopy and plans to close off the urachal sinus tract.\nUnder general anesthesia, laparoscopic exploration revealed a uterus adherent to the anterior abdominal wall consistent with the patient's history of previous cesarean sections. The area around the umbilicus was examined, and no distinct connection to the bladder was noted. The surrounding tissue around the umbilicus was taken down using a tissue sealer. The umbilicus itself was examined, and no intraabdominal connections were noted. As a result, the laparoscopic approach was concluded, and attention was turned to the umbilicus externally where a circumferential incision was made around the mass. The firm mass was encountered subcutaneously and was circumferentially excised. Because of its adherence to the fascia, a 2 x 2 cm circle of fascia was excised along with the mass. This was sent to pathology for specimen analysis. The fascia was then closed, and the umbilicus was reconstructed. On microscopic examination, histologic sections revealed tissue consistent with endometriosis. |
A 36-year-old female P2L1 presented in October 2013 with the complaint of pain and swelling on the cesarean scar on and off for 11 years. In addition, she described cyclic bleeding along with pus from this cesarean scar for 11 years. She previously had one spontaneous vaginal delivery 14 years ago and cesarean section 12 years back in view of nonprogression of labor. She described pain over the cesarean scar at the lower end that increased during the menstruation and then noticed a swelling over cesarean scar at the lower end. She noticed mild bleeding from scar within the 1st days of her menstruation followed by pus. Examination revealed a well-healed cesarean scar, moderately pigmented area, tenderness at the lower end of the vertical infraumbilical scar on palpation. Ultrasound [] showed that ill-defined hypoechoic lobulated lesion 3.3 cm × 2.7 cm is seen in relation to the superior surface of the uterus till skin surface at the site of scar. A linear fluid-filled tract was also seen within it. Based on characteristic history and examination findings, the most probable diagnosis of scar endometriosis with uterocutaneous fistula was kept. The patient underwent exploratory laparotomy. Intraoperatively, an inverted Y-shaped fistula was identified with the help of a thin infant feeding tube connecting abdominal skin with the uterus and to the dome of the bladder. Fistulous tract excised out and the specimen was sent to the pathology department. Left salpingectomy was done in view of hydrosalpinx, and as bladder dome had some swelling attached to the fistulous tract, sleeve resection of the bladder with repair was done in single continuous layer. Uterus was also closed in two layers. Histopathology of the excised tract confirmed endometriosis. The patient's postoperative course was uneventful and her pain subsided. There was no bleeding and discharge from scar afterward. She conceived spontaneously in May 2014, she was a booked pregnancy in our hospital, and her antenatal period was uneventful. She underwent emergency lower segment cesarean section in view of fetal distress and preterm premature rupture of membranes at 34 weeks 2 days of gestation. She delivered a healthy male baby of birth weight 2.42 kg. The patient's postoperative period was uneventful. |
A 20-year-old man presented with mild dyspnea and was diagnosed with a common cold at the first visit to a clinic. However, severe dyspnea persisted after 3 days. Enhanced computed tomography (CT) scan revealed a mediastinal mass in the subcarinal space, which compressed the right pulmonary artery and a delayed right pulmonary vein and airway enhancement at the tracheal bifurcation (-A, B). The mass was diagnosed as a mediastinal cyst and bronchoscopy was attempted; however, the patient was unable to be placed in the supine position due to severe respiratory distress. The patient was then transported to our hospital by ambulance due to the need for emergent surgery. Despite the fact that transportation took only two hours, his white blood cell count increased rapidly and symptoms became progressively worse, which suggested that the patient’s condition was emergent. Signed consent was obtained for all procedures. To ensure that the mass was a simple cyst without septum and mural nodule, magnetic resonance imaging (MRI) was performed as the patient’s condition allowed; the image showed a two-layered simple cyst, which indicated an infection or bleeding inside the cyst (-C, D). Since the symptoms developed and exacerbated rapidly, we performed emergent surgery. After the induction of general anesthesia with “stand-by” extracorporeal membrane oxygenation, video-assisted thoracic surgery (VATS) was performed. First, we punctured the cyst and aspirated white pus; then, we performed cyst wall fenestration on the subcarinal lesion and superior mediastinum. The bottom layer fluid inside the cyst contained both pus and blood, which were compatible with MRI findings, while continuous bleeding from the cyst wall was not observed (). Performing complete cyst resection was difficult owing to severe adhesion of the cyst to surrounding organs such as both main bronchi and pericardium. After surgery, the symptoms resolved immediately and completely. The postoperative course was uneventful and the patient was discharged on the 15th postoperative day. Pathological examination of the cyst wall revealed an inflamed bronchogenic cyst with the findings of bronchial gland, cartilage with infiltration of inflammatory cells, and no malignancy (). Six months after the operation, no sign of recurrence was observed. |
A 40 year-old malnourished man with history of roux-en-Y-gastrojejunostomy for pyloric stenosis secondary to analgesia overuse, presented with septic shock and hepatic derangement. He developed refractory status epilepticus on day 2 of his hospital admission and received a loading dose of sodium valproate. Subsequently an ammonia level of 619 μmol/L was reported (normal in all 3 cases presented <52 μmol/L). Emergency dialysis normalized the ammonia level by the next day but he remained encephalopathic. An MRI brain was performed 5 days after presentation showing a diffuse increase in T2 and FLAIR signal within the cortex of both cerebral hemispheres (). There was sparing of the perirolandic cortex and occipital cortex bilaterally. This cortical high signal with sparing of the perirolandic cortex was also well visualised on B1000 images of diffusion weighted imaging () with no associated low signal on the ADC (apparent diffusion coefficient) map . The initial short TE (35ms) magnetic resonance spectroscopy () at presentation revealed a slightly elevated glutamine/glutamate peak with a slight decrease in choline and myoinositol peaks. These findings have been described in hepatic encephalopathy with elevated ammonia.\nAt the time of the MRI, the cause of the ongoing encephalopathy was thought to have been due to sepsis but the diagnosis of hyperammonemic encephalopathy was suggested after MRI was performed even though his ammonia level was at that stage normal. The possibility of the cortical signal change relating to status epilepticus or hypoxia was also considered but both were felt to be less likely particularly given the pattern of signal change and the previously very markedly elevated ammonia level.\nRepeat short TE MR spectroscopy was performed two months later (). Whilst it is difficult to directly compare these spectra due to the differences in the y-axis, using creatine peak as a reference, there is a reduced level of N-acetylaspartate (NAA). NAA is a marker of normal neuronal activity and the reduction of NAA is consistent with significant brain atrophy which was noted on other MR sequences obtained at that time and which can be seen on the planning images for MR spectroscopy () . Although more equivocal, there was also a slight decrease in the glutamine/glutamate peak, which may reflect resolution of the hyperammonaemia.\nA metabolic work up did not reveal any underlying urea cycle abnormality. His hyperammonaemia was attributed to a combination of malnutrition, acute sepsis, and poor hepatic reserve. Neurological recovery was poor at 6 months and he was quadriparetic and minimally responsive. |
A male patient, 26 years old, sought care at the dental clinic with fractures of the left maxillary central incisor resulting from a sudden strike three months earlier. The patient had no clinical symptoms during this period (Fig. ). A clinical examination revealed that the left maxillary central incisor was fractured in the middle third of the crown and that this fracture involved the enamel and dentin with no pulp exposure and no signs or symptoms of a concussion or contusion. A routine cold vitality test of the tooth revealed that it was associated with the same reaction as the reference tooth. Additionally, the patient had a defect in the incisal area of the right maxillary central incisor that resulted from eating melon seeds, and a routine cold vitality test of the tooth revealed a positive reaction. Finally, the relationship between the anterior teeth overbite and overjet was normal. A radiographic examination of the central incisors was conducted, and an analysis of radiography of the maxillary left central incisor revealed that there were fractures in the middle third of the crown, but no abnormalities, such as damage to the remaining roots, were observed (Fig. ).\nA 3D-printed template was fabricated using intra-oral scanning, CAD, virtual modeling and 3D printing. Briefly, a digital registration of the dentition was performed using a CEREC AC Omnicam intra-oral scanner (CEREC AC D3492, Sirona Dental Systems GmbH, Fabrikstr, Bensheim, Germany). The inlay in the machine was selected, and the system automatically generated a prosthesis using the contralateral tooth as a reference. From the analysis performed using the software, the occlusal contact of the intercuspal occlusion of the patient was concentrated in the middle third of the cervix, and it was therefore appropriate for composite resin restoration. An occlusal adjustment was made to eliminate anterior contact in the occlusion and to avoid contact with the prosthesis (Fig. ). We showed a picture of the result to the patient, and he was satisfied with it. The data were then imported into Freeform (Geomagic Freeform, 3D Systems, Morrisville, North Carolina, USA), a software program that is widely used to design 3D models. Using the Freeform program, a template can be designed through a process similar to drawing a picture, and a dentist can design a repaired palatal template in only minutes (Fig. ). The digitally designed template is prepared for export using the “stl check” command, exported as a stl-file and then sent to a 3D printer (3D System 3510HB, 3D Systems, Morrisville, North Carolina, USA). Finally, the 3D-printed template is fabricated (Fig. ).\nBefore treatment, the 3D-printed template was detached and soaked in disinfectant. Then, the template was positioned on the patient’s dentition, and a correct and reproducible fit was verified. Initially, the anterior teeth were isolated using a rubber dam (Hygienic Elasti rubber dam, Switzerland). The teeth were carefully cleaned using prophylaxis paste (SS white prophylaxis paste, England), dried, and submitted to minimal tooth preparation using a diamond bur (Mani SF-41, Japan) to produce an improved alignment for the bond. Both surfaces of the connection were etched using acid gel (Ultra-Etch® 35% Phosphoric Acid, Ultradent, USA), rinsed, and gently dried. Single bond (Adper™ Single Bond 2, 3 M ESPE, USA) was applied first. The surface was then air-dried for 5 s and exposed to light-activation for 10 s. Subsequently, the 3D template, which had been detached and soaked in disinfectant, was positioned on the back of the anterior teeth (Fig. ). It was convenient to construct the palatal surface using an opaque enamel shade (E2, Ceram*X duo, DENTSPLY, Germany) with the aid of a 3D printing guide. After polymerization, the palatal wall is sufficiently strong to support the next stratification steps. Reconstruction was performed using an opaque dentin shade (D2, Ceram*X duo, DENTSPLY, Germany) to construct the dentin body (Fig. ). The enamel shade E2 was used to match the superficial enamel, and each composite increment was light-cured for 20 s. Additionally, tooth 11 was restored using enamel shade E2 in the incisal area and on the buccal surface. The final step consisted of performing an additional 20 s of polymerization at each site. After excess composite material was removed, an occlusion test was performed using carbon paper, and the restorations were shaped to a proper anatomic morphology (Fig. ). Next, finishing and polishing procedures were performed using diamond fine coating burs and a polishing system (One-step diamond micro-polisher, Germany) (Fig. ). |
A 12-year-old boy presented to the Cornea Clinic of our hospital with a conjunctival mass lesion on his left eye, which had developed over the course of a few weeks. During a slit lamp examination, the lesion was found to be lobulated with corkscrew vessels that infiltrated the temporal, nasal, inferior and superior bulbar, and forniceal conjunctiva (Figures and ). He had a positive history of recurrent herpetic keratitis on the same eye and had undergone penetrating keratoplasty six years earlier. However, his corneal graft had failed with total opacity and vascularization due to recurrent herpes simplex keratitis (HSK) in the corneal graft. The right eye was normal.\nHis history revealed that he had suffered from eczematous skin lesions, recurrent skin bursts, and sinopulmonary infections since early childhood and recurrent generalized HSV infection. He had been diagnosed with HIES at the age of three based on elevated serum IgE levels and other clinical features. He did not have a positive family history of HIES or any other immunodeficiency.\nAt the onset of the lesion discussed here, a differential diagnosis of a conjunctival lymphoid tumor, conjunctival papilloma, and ocular surface squamous neoplasia was given. Because the patient was prone to recurrent HSK, recurrent herpetic lesions were not considered in the differential diagnosis because the patient presented a papillomatous mass lesion of the conjunctiva. Thus, the pathogenesis was suspected to be HPV because the dendritic lesion and stromal infiltration had not be seen in the failed graft. For a specific diagnosis, an incisional biopsy was obtained from the conjunctival lesion, and sections were prepared and stained with hematoxylin and eosin using the periodic acid-Schiff technique to observe the sections under light microscopy (Olympus BX43, Tokyo, Japan). Microscopic examination revealed conjunctival tissue with dense, diffuse, acute, and chronic inflammatory cells that infiltrated the lamina propria and were rich in eosinophil. Eosinophilic conjunctivitis with pseudoepitheliomatous hyperplasia was the pathologic diagnosis. An examination of the specimens for the presence of HPV was requested, and genotyping was performed by a DNA analysis using a line-probe assay method that revealed genotype 52 (high-risk) HPV.\nThe patient was treated with topical interferon-α2b (IFN-α2b) (3 MIU/cc of PDferon-B; Pooyesh Darou Co, Iran), 1,000,000 IU/mL, four times a day and oral cimetidine (Iran Daru, Iran, Tehran), 200 mg, four times a day for HPV and was given a low dose of oral acyclovir (400 mg/day) to prevent recurrence of HSK. There was no clinical response to this management at the three-month follow-up. In the next stage of treatment, the patient should have undergone excision and debulking of the mass lesion, but extensive herpetic lesions had recurred on the skin of the patient's head and neck. Therefore, the patient received intravenous (IV) acyclovir for systemic herpetic disease (600 mg/three times a day), prescribed by a pediatrician. The size of the conjunctival mass lesions decreased rapidly after this treatment, and the lesion completely resolved within a few days (). Due to this positive response to IV acyclovir, the presence of HSV in the lesion was suspected and a reexamination of the pathology sample using immunohistochemical staining for HSV revealed the presence of this virus and proved coinfection. |
An 18-year-old male college baseball pitcher and football player presented with right upper extremity pain and exertional fatigue with overhead throwing for 18 months. He also endorsed paresthesias in the right fourth and fifth fingers as well as loss of power manifested by slower pitching speeds, result in a decline in his athletic performance. Symptoms were largely absent at rest with little to no effects noticed with activity below shoulder level. He denied any shoulder pain or limitation of motion at the glenohumeral joint. There was no preceding trauma although he has a history of medial epicondyle fracture with a closed reduction 4 years prior and thoracic myofascial pain managed with ongoing physical therapy.\nHe was initially seen by Sports Medicine who performed an extensive work-up over a 6-month period including a negative EMG, shoulder radiographs, and MRI of the humerus and glenohumeral joint that suggested a posterior labral tear. Physical exam was unremarkable for shoulder tenderness, muscle atrophy, limitation of mobility around the glenohumeral joint or decreased strength. Noninvasive studies suggested TOS with positive costoclavicular and hyperabduction maneuvers, but due to his presentation, an injury to the glenoid labrum was suspected and they proceeded to manage his symptoms conservatively with intra-articular injections and physical therapy. Due to only transient improvements, he was referred to Vascular Surgery for evaluation.\nMRA of the chest showed a nonunion fracture of the right first rib with a large hypertrophic callus adjacent to the brachial plexus compressing the subclavian artery mildly (Fig. ). No cervical rib was present. With radiographic evidence suggesting TOS, surgical decompression was offered to the patient. After discussion, the decision was made to proceed with transaxillary first rib resection.\nUnder general anesthesia in left lateral decubitus, a transverse incision was made in the axilla with dissection carried down to the thoracic outlet. The anterior and middle scalenes were identified and resected. The large callus on the first rib was identified and located adjacent to the neurovascular bundle consistent with preoperative imaging (Fig. ). The rib was then circumferentially isolated and resected with a rib cutter, however the posterior-most portion of the rib was inaccessible. Therefore a rongeur was used to remove remaining bone. On inspection of the specimen, a large protuberance corresponding to the callus on MR was immediately obvious (Fig. ). The neurovascular bundle was identified and protected throughout the resection. There was obvious entrance into the chest so a chest tube was place. The patient was woken and had an intact neurological and vascular exam. Postoperative chest film showed adequate removal of the first rib.\nOn postoperative Day 1, his chest tube and drain were removed and he was discharged home without any issues. At his 5-month follow up visit, his paresthesias had completely resolved and he denied exertional fatigue. He had resumed pitching and endorsed marked improvement. |
An otherwise well 30-year-old female presented to her general practitioner complaining of intermittent right upper quadrant discomfort over the preceding months. Abdominal examination was suspicious for hepatomegaly and subsequent ultrasound demonstrated a large liver mass. The patient was referred to hospital and CT imaging confirmed a pedunculated giant hepatic haemangioma (13.8 cm × 10.2 cm × 7.4 cm) attached to segment 4 of the liver (Figs and ). Two smaller intra-parenchymal haemangiomas in segment 6 and 8 were also identified and measured 0.5 cm and 1.3 cm, respectively. Despite a negative urine Beta HCG test at the time of CT scanning, she was subsequently found to be pregnant. False negative results such as this are reported to occur, especially in the early luteal phase [] when the urine HCG may not have reached the diagnostic threshold. Approximately 1 year prior, she had ceased the combined oral contraceptive after taking it for 10 years and was planning to conceive.\nOn first review in our clinic the patient was 6 weeks pregnant. She was counselled by a consultant liver surgeon and obstetrician about the available management options and associated risks. The surgical options discussed included; close observation throughout pregnancy and elective resection post-partum, termination of pregnancy followed by resection or resection during the second trimester of pregnancy. Our patient decided to have the tumour resected whilst pregnant due to the concern of rupture, accepting the risks of surgery to herself and the foetus. The operation was scheduled for the second trimester as this is the period in which subsequent miscarriage or preterm labour are least likely [].\nThe resection was performed at 18 weeks gestation under general anaesthesia via a small upper midline incision. Upon entering the peritoneal cavity the exophytic haemangioma (Fig. ) was resected along its attachment to segment 4b using Harmonic ACE®+7 Shears. This allowed removal with minimal disruption of liver parenchyma, major vasculature or bile ducts. Minimal blood loss occurred and operating time was approximately 1 h. The smaller intra-parenchymal lesions were not resected as they were not the likely cause of her symptoms and represented a lower risk of rupture. The general anaesthetic was tailored to reduce any interruptions to uterine blood flow. Intraoperative foetal monitoring was not indicated due to the pregnancy still being in the previable phase.\nObstetric examination in the immediate post-operative period was consistent with a healthy foetus and the patient was discharged from hospital on the third post-operative day. Histopathological examination of the tumour confirmed a haemangioma weighing 412 g. The patient received outpatient follow-up and did not experience any surgical or obstetric complications. A healthy infant was delivered at term via an elective caesarean section due to maternal request. |
A 27-year-old Caucasian gentleman was referred to the plastic surgery outpatient clinic with a 7 month history of pain and swelling of his left middle toe. The cause of his symptoms was unclear, with the patient linking the episode to minor trauma. Initially, he had seen a chiropodist who diagnosed him with an in-grown toenail. The toenail was subsequently removed; however, his symptoms worsened. At this stage, he was noted to have subungual discolouration. A punch biopsy was performed with histology demonstrating a pigmented keratinous disc, and he was subsequently referred to our service as a possible case of malignant melanoma.\nHe has no other significant past medical history and did not take any regular medication. He smoked approximately 10 cigarettes per day since aged 15.\nUpon review in clinic, he was found to have an ulcerated area over the dorsum of his left middle toe with associated erythema and swelling, which was actively discharging pus but good peripheral pulses. He also reported a 6-week history of similar symptoms on his left index finger, over the dorsum of the proximal inter-phalangeal joint (PIPJ) which had appeared spontaneously. He had been treated with several courses of oral antibiotics by his general practitioner with no improvement. He was admitted acutely for intravenous antibiotics, surgical exploration and washout of both digits, and a biopsy of the left middle toe for microbiology and histology to rule out osteomyelitis and malignant melanoma. Initial biopsy of the nail bed excluded malignancy and instead showed acute inflammatory changes. The index finger defect was further debrided and closed with a local flap.\nFollowing this initial surgical intervention, the patient went on to have a long and protracted clinical course from complications of poor wound healing and necrosis, with multiple hospital admissions over a 15-month period. He was reviewed by the vascular team with full assessment of peripheral vasculature using ankle-brachial pressure index and computed tomography (CT) angiography which did not identify a specific cause for his necrotic digits. He developed osteomyelitis of both the left third toe and left index finger, which required amputation to prevent proximal spread of infection. A clinical diagnosis of TAO was suspected when he developed similar symptoms in the adjacent left second toe 2 months after initial presentation. Negative autoimmune, vasculitic and coagulopathy screening blood tests, including anti-neutrophil cytoplasm antibodies (ANCA), erythrocyte sedimentation rate, anti-cardiolipin antibodies, anti-thrombin, proteins C and S, supported the diagnosis. Genotyping for prothrombin mutations was negative; however, the patient was positive for homozygous factor V Leiden mutation. Verbal smoking cessation advice was reinforced frequently; however, the patient found complete abstinence difficult. This ultimately resulted in a Ray amputation of the second and third toes due continued non-healing (). Following this, he underwent a short period of remission. Unfortunately due to ongoing use of tobacco, symptoms recurred in the contralateral and dominant right hand affecting his index and middle finger tips ( and ). The right index finger became gangrenous and required amputation. During the procedure, a segment of digital artery was biopsied. Histopathologic analysis showed evidence of chronic inflammation with lymphocytic arteritis and thrombosis, confirming a diagnosis of TAO (). |
A 66-year-old male patient, with a history of smoking (30 pack-years) and no known medical or surgical history, was admitted in our department for a spontaneously resolved inferior ST elevation myocardial infarction (STEMI). The intra-hospital treatment included enoxaparin 0.6 ml twice a day, clopidogrel 75 mg once a day, aspirin 100 mg once a day, bisoprolol 2.5 mg once a day and atorvastatin 40 mg once a day. The coronary angiogram (performed at day 3 through the right radial artery) showed a severe thrombotic lesion of the distal circumflex. The patient underwent an ad-hoc successful angioplasty of the circumflex with a drug eluting (everolimus) stent. Initial laboratory tests at admission were normal except elevated troponin. Echocardiography showed a 65% left ventricular ejection fraction. The patient was discharged after 5 days of anticoagulation by low molecular weight heparin (enoxaparin). Laboratory tests were not controlled during the hospitalization. The discharge treatment included clopidogrel 75 mg once a day, aspirin 100 mg once a day, bisoprolol 2.5 mg once a day and atorvastatin 40 mg once a day.\nOne week later, the patient was referred again to our department for both chest and right lower limb pain. The electrocardiogram showed an inferior STEMI and the physical exam of the right lower limb found ischemic signs with absence of the femoral pulse. There was no history of aspirin or clopidogrel discontinuation. An urgent coronary angiogram (performed through the left femoral artery) showed total thrombosis of the circumflex stent (\n). The patient underwent a successful primary angioplasty of the circumflex by simple balloon (\n). Urgent lower limb contrast-enhanced computed tomography was performed immediately after the angioplasty, revealing total acute thrombosis of the right common femoral artery (\n). The patient underwent an urgent successful thrombectomy with Fogarty catheter. Immediate evolution was favorable with total regression of coronary and right lower limb ischemic signs. Laboratory tests showed a marked fall in the platelet count (68,000/L) which was normal (364,000/L) in the previous hospitalization. A diagnosis of concomitant coronary stent and femoral artery thrombosis due to HIT was strongly suspected (4T score = 8). Our therapeutic strategy was immediate discontinuation of low molecular weight heparin (enoxaparin), aspirin and clopidogrel with strict daily control of platelet count. During this period, no alternative anticoagulation was initiated because of the unavailability of direct thrombin inhibitors in our center. Anticoagulation with a vitamin K antagonist (acenocoumarol 4 mg once a day) and dual antiplatelet therapy with aspirin 100 mg once a day and clopidogrel 75 mg once a day were initiated at day 3 once platelet count had recovered. The in-hospital outcome was favorable and the patient was discharged after 15 days on acenocoumarol 4 mg once a day, aspirin 100 mg once a day and clopidogrel 75 mg once a day. The 3-month follow-up, with controlled blood tests and lower limb contrast-enhanced computed tomography showing total reperfusion of the right femoral artery (\n), was unremarkable. |
The second patient is a 53-year-old Asian male with a history of type 2 diabetes mellitus that was diagnosed in 2010. His medical history also includes hyperlipidemia, severe uncontrolled hypertension and advanced renal disease with Stage 4 CKD just before the need for intravitreal anti-VEGF therapy was noted. He had poor compliance with his prescribed diabetic diet. His hemoglobin A1C ranged between 10% and 11% at the time of initial diagnosis and remained consistency high for years afterwards. He was on oral agents for his diabetes mellitus since he declined insulin therapy despite elevated blood sugar levels and had a history of NSAID use earlier in his history that likely contributed to his CKD progression. As his renal function declined, he reduced his NSAID usage significantly and would only use NSAIDs during infrequent gout flares. His metformin was stopped in March 2017 and changed to a sulfonylurea due to his declining renal function.\nHe had an estimated glomerular filtration rate (eGFR) of 17 mL/min at the initiation of bevacizumab injections on 1 July 2017. He had a nearly linear decline in eGFR (mL/min) as measured by the Cockroft–Gault equation. His proteinuria had been stable at 2800–3000 μg albuminuria/mg creatinine (= 2.8 g albumin/g creatinine), but after starting bevacizumab injections for proliferative DR his ACR increased to 4500 μg albumin/mg creatinine.\nWithin the first 2 months of initiating therapy he received four injections of 1.25 mg of bevacizumab at an interval of every 2 weeks alternating between each eye. His injections were planned so that he waited at least 1 month between injections in the same eye. The total dose delivered over these four injections was 5 mg, and within that short interval, his renal function deteriorated to the extent of needing renal replacement therapy (hemodialysis).\nHis renal function had already been declining prior to anti-VEGF therapy, but after starting therapy his creatinine rose from a baseline of 338 μmol/L (3.83 mg/dL) to 688.8 μmol/L (7.79 mg/dL). His blood pressure at baseline was 180–200 mmHg systolic (SBP) and 100–120 mmHg diastolic (DBP). There was no discernible change in the SBP or DBP, though his blood pressure was already severely elevated. The increase in urine protein from baseline to higher levels after the anti-VEGF injections and renal function trends are depicted in . |
A 65-years old male patient came to the Department of Oral Medicine and Radiology with the chief complaint of deep aching and burning pain on the right side of the face region for the last two days. On clinical examination, few crops of vesicles were present on the right side of his lip region involving the vermilion border () and a single vesicle was present intraorally on the right side of the palate region. Past medical history was insignificant.\nBased on the history of a burning sensation on the right side of the maxillary dermatome region with a unilateral distribution of vesicles, it was provisionally diagnosed as herpes zoster with the involvement of the maxillary branch of the trigeminal nerve. The patient was kept under Tab. Acyclovir 800mg five times daily for seven days and recalled for follow-up after a week.\nHowever, the patient returned to the department after two days with hyperesthesia on the right side of his face causing him difficulty in performing daily chores as well as falling asleep. The patient also presented with multiple vesiculo-ulcerative lesions on the right side of the ala of the nose, columella of the nose, and the upper lip, involving the vermilion border with mild swelling of the upper lip (). Intraorally, multiple ulcers were noted on the right side of the hard palate region which was restricted to the midline. After a thorough history, it was revealed that the patient had not taken the full daily dose of the prescribed antiviral medication (Acyclovir) and rather took painkillers from a nearby pharmacy that did not subside his pain. The patient was further advised for some routine laboratory investigations such as complete blood count (CBC), random blood glucose test, CRP (C reactive protein) test, and enzyme-linked immunoassay (ELISA) test to rule out any asymptomatic human immunodeficiency virus (HIV).\nOn day eight, the patient came with his laboratory reports which showed an increased erythrocyte sedimentation rate of 32mm/hr with all other investigations such as CBC, random glucose test, and CRP test results within the normal limits. ELISA test for HIV was negative. On examination, multiple scab formations were noted extra-orally (Figure 2B,2C).and much-healed ulcers intraorally. According to the patient, there was a marked reduction in pain and the burning sensation. Complete resolution of extraoral ulceration and scab lesions are seen on the 15th day (). Nonetheless, the patient was made aware of the chances of recurrence of similar or worsening pain even after the resolution of lesions and was recalled for a follow-up visit after a week. |
We present a 43-year-old man with a past medical history only significant for a prior splenectomy who was admitted to the hospital due to right upper quadrant pain for two days. This was described as a dull “liver pain” in the right upper quadrant area. On physical exam, there was evidence of a midline scar, the abdomen was soft with mild tenderness to palpation of the right upper quadrant and the liver span was approximately 10 cm in the mid-clavicular line by percussion. A complete blood count and a basic metabolic panel were normal; however, alanine transaminase (ALT) and aspartate transaminase (AST) showed a mild elevation of 66 U/L and 51 U/L, respectively. Serum bilirubin levels and alkaline phosphatase levels were within normal limits. Due to the reported complaint of right upper quadrant pain and the associated abnormal liver function tests, an abdominal ultrasound (US) was ordered. This showed fatty liver disease and a left liver lobe isoechoic liver mass. A computed tomography (CT) triple phase abdomen scan was done demonstrating a 2.5 cm exophytic mass in the liver in segment 2 (Figure ). The next day of admission, the patient’s pain improved with analgesia. As no clear diagnosis was made, he was later discharged with an intention to perform an elective abdominal magnetic resonance imaging (MRI). This MRI revealed a single mass in segment 2 of the liver, with features of a hepatic adenoma (Figure ). The surgical team was consulted and evaluated the patient and an elective percutaneous liver biopsy was performed. Examination of hematoxylin and eosin (H&E) stained sections revealed histological evidence of splenic tissue with distinct red and white pulp areas, with evidence of passive congestion (Figure ). The red pulp included thin-walled venous sinusoids that were congested with red blood cells that were positive for CD8 stains (Figure ), with surrounding macrophages and few lymphocytes. The white pulp included thickened meshwork of cords showing arterioles sheathed by predominantly small T lymphocytes (CD3+) and scattered B-cell aggregates (CD20+), consistent with splenic Malpighian corpuscles (Figure ). On further questioning, the patient reported he had an exploratory laparotomy with subsequent emergent splenectomy at the age of 16 years due to a motor vehicle accident which caused a splenic rupture. As the patient was diagnosed with hepatic splenosis and was at this point asymptomatic, his benign diagnosis was explained, and no further workup was needed. |
A 73-year-old white man presented in 2014 with a chronic fistulation of his left mandible. He had a history of primary hypertension, type 2 diabetes mellitus, and hypercholesteremia. Furthermore, in 2010 the diagnosis of a poorly differentiated SCC of his oropharynx was made: tumor stage cT4 cN2 cM0 (TNM, 7th edition, 2010). Clinical findings displayed an exophytic tumor portion, which derived from the right lateral wall of his oropharynx, grew into his right epiglottic vallecula, infiltrated the lingual epiglottis and exceeded the midline in the region of his tongue base. Due to the size of the tumor and at his request a tumor resection was not made. Hence, primary therapy was performed as combined radiochemotherapy. External beam radiation was performed with a fractionation of five times 1.8 Gy per week up to an overall dose of 72 Gy. This was supplemented by a simultaneous chemotherapy with cisplatin and 5-fluorouracil in the first and sixth week of radiotherapy. During the oncological follow up there were no signs of residual tumor or lymph node metastases.\nDue to an increasingly painful restricted mouth opening in 2013 he sought medical help again. At that time an intraoral fistula of the mucosa in his left retromolar region and a pathological fracture of his left mandibular angle on the basis of an osteoradionecrosis were detected. These conditions required a complete resection of his mandible in the retromolar region and the temporary bridging of the bony defect with a reconstruction plate (Fig. ). Despite intensive wound care, wound healing only took place very slowly and remained insufficient with distinctive wound dehiscence.\nAfter this he firstly attended our hospital in 2014 with exposed osteosynthesis material and persisting suppurating fistulation intraorally and extraorally with the desire for a definitive reconstruction of his mandible. A first reconstruction attempt with a microvascular anastomosed graft from his right iliac crest failed due to an insufficient venous anastomosis. The resected bone of his defect-bordering jaw showed the typical pathohistological findings of an intraosseous PH (Fig. ). Five days later, after explantation of the iliac crest graft, the bony defect was successfully reconstructed with a microvascular anastomosed fibula graft along with an indicator flap from his right lower thigh (Fig. ). The wound healing process was again extremely prolonged. Once more in spite of targeted antibiotic therapy with ciprofloxacin and extensive local wound care some long wound dehiscences developed, which healed only very slowly. After covering the skin defect of his right lower thigh with full thickness skin from his right lower abdomen and closure of the tracheostoma, he was discharged after 6 weeks in a good general condition. Seven months later, the occurrence of a pseudarthrosis at the anterior osteosynthesis required another surgical intervention including debridement of the bordering bone and re-osteosynthesis. By means of close monitoring and continuous local wound care all dehiscences were closed. His osteosynthesis material was finally removed after the bony continuity of his mandible was clearly documented intraoperatively. He is well except for a moderate painless restricted mouth opening and he has largely kept symptom-free.\nHis bone tissue showed a chronic recurrent osteomyelitis. The trabecular bone was multifocally necrotic and surrounded by a mixed inflammatory infiltrate consisting of lymphocytes, plasma cells, and histiocytes. In addition, there were plenty of neutrophil granulocytes, focal formation of abscesses, and some bacterial colonies with features of Actinomyces. Furthermore, signs of bone reabsorption as well as bone remodeling and fibrosis of the medullary spaces were seen. The trabecular bone was partly covered by ribbon-like proliferations of regularly stratified squamous epithelium (Fig. ) without nuclear atypia, mitosis, and dyskeratosis. Immunohistochemical stainings were inconspicuous. There was no overexpression of p53, and the proliferation rate, determined with the antibody against Ki67, showed a physiological staining pattern in the basal cell layers of the squamous epithelium (Fig. ). Moreover, the squamous epithelium expressed p63 (Fig. ), while cytokeratin 7 remained negative as expected (Fig. ). Histological characteristics for the previously diagnosed SCC such as nuclear pleomorphism, increased mitosis, perineural invasion (Fig. ), and an infiltrating growth pattern could not be detected.\nIn summary, this case is a rare example of an intraosseous PH in response to a chronic recurrent osteomyelitis. |
The patient was a 35-year-old, gravida 2, para 1, woman who had undergone cesarean delivery by low transverse incision because of cephalopelvic disproportion 4 years previously. She was referred to another hospital with suspicion of a cesarean scar pregnancy because a wedge-shaped gestational sac (GS) was found at the scar in the lower uterine segment at 6 weeks and 1 day of gestation (). Three days later, a deformed GS at the previous uterine scar was confirmed and she was closely followed up due to the potential for miscarriage. At 9 weeks of gestation, the deformity had disappeared with growth of the GS into the uterine cavity. The ultrasound image was similar to that of a normal pregnancy and the gestation was allowed to continue. She was referred to our hospital with a diagnosis of total placenta previa at 24 weeks of gestation. Transvaginal ultrasonography revealed loss of hypoechoic appearance of the retroplacental zone, lacunas in the placenta, and bulging of the bladder (). Magnetic resonance imaging showed disappearance of the sonolucent zone between the myometrium and the bladder, a heterogeneous signal from the internal placenta, and an irregular bulge with a flow void on the surface of the bladder on T2-weighted images. Cystoscopy revealed normal bladder mucosa. Based on these findings, placenta previa-accreta was suspected. She did not desire to preserve her uterus. Therefore, she agreed to a cesarean hysterectomy without attempting placenta removal after delivery of her baby, if placenta accreta was strongly suspected. At 28 weeks of gestation, she was hospitalized because of warning bleeding, and administration of tocolytic agents was initiated.\nFive days later, preterm premature rupture of membranes developed with intense uterine contractions, necessitating an emergency cesarean section.\nWith consideration of a possible cesarean hysterectomy, a ureteral stent was placed after induction of spinal anesthesia. An intra-arterial balloon catheter could not be prophylactically placed, because it was an emergency operation. Intraoperatively, no myometrium was detected in the lower uterus and the placenta was visible through the uterine wall, findings consistent with a cesarean scar pregnancy (). Placenta percreta was diagnosed and cesarean hysterectomy was indicated. A viable baby was delivered after classical uterine incision followed by abdominal total hysterectomy without removal of the placenta. The bladder musculature strongly adhered to the incision scar of the previous cesarean section. There was massive hemorrhaging with detachment of the bladder, requiring partial resection of the bladder musculature. The bleeding volume was approximately 6.5 L and a massive blood transfusion was required. Her postoperative course was uneventful and the patient was discharged from the hospital 7 days after the operation. The pathological examination confirmed placenta percreta. |
A 54-year-old African American male initially presented to an outside hospital complaining of headache, which had been present for two months but in the last week was worsening per patient's family. Two months previously, he had been diagnosed with chronic myeloid leukemia (CML) initially diagnosed to be in the chronic stable phase and had been maintained on hydroxyurea. The patient was admitted to the hospital and order for head CT placed. While on the floor, the patient complained of sudden worsening of headache and eventually became nonresponsive, requiring emergent intubation. Head CT was obtained showing large subdural hematoma (SDH) for which the patient was given 100 grams of mannitol and he was transferred to our facility immediately.\nUpon intake, the patient was noted to have a fixed and dilated right pupil, reactive left pupil, and trace movement in his right upper extremity. Head CT () showed 1.5 cm right panhemispheric hyperdensity in the subdural space with significant midline shift, falcine herniation, uncal herniation, and loss of basilar cisterns. Given the mixed density on imaging and rapid progression symptomatically, outside hospital physician had assumed a spontaneous subdural hematoma secondary to coagulopathy. Interestingly, the patient's platelet count, PT, PTT, and bleeding time were all well within normal limits. The patient was taken emergently for hemicraniectomy and hematoma evacuation.\nTo the great surprise of the operating surgeon, upon dural opening, large clumps of confluent white material expressed themselves under pressure (). Very little hematoma was present; copious irrigation throughout both the subdural and subarachnoid spaces revealed more confluent material again with very little blood. Persistent irrigation of the subarachnoid/subdural spaces continued to yield additional confluent white material. After evacuation, the patient had intracranial pressure monitor placed and had a postoperative head CT ().\nThe patient was initially kept sedated in the ICU and had good ICP control for roughly 6 hours. Thereafter, he began having progressively worsening ICP spikes, at which point repeat head CT was done (), once again showing large accumulation of subdural mass. As his exam never improved from presentation, his family was counseled as to likely futility of reoperation, though eventually they requested evacuation. Second decompression again revealed confluent mass in the subdural and subarachnoid spaces with very little hematoma. After second evacuation, the patient continued to have malignant intracranial hypertension with no improvement in clinical exam. His family chose to withdraw care 3 days later. Pathology revealed myeloid cells with immunohistochemistry staining negative for CD34 and CD117, consistent with mature myeloid sarcoma (). |
A 3-year-old Caucasian girl accompanied by her mother was referred by her pediatric dentist to the outpatient stomatology clinic at the São José dos Campos Dental School, UNESP, because of the lack of eruption of some teeth and marked atrophy of the left posterior inferior alveolar border (Figure ). During investigation of her medical, dental and familial history, the mother reported the presence of maculae in various regions of the child's body. This was confirmed by physical examination, with the observation of bilateral, slate-brown maculae in the axillary region (Figure ), on the back, and in the hypochondrium and groin. With respect to clinical history, the mother reported that her daughter had suffered some episodes of seizures when she was approximately one month old, and that at that time small blisters had appeared that followed the same trajectory as the current maculae. During that time, the reported blisters were diagnosed as herpes zoster and treated with topical acyclovir, but the outcome was unsatisfactory. After a period of approximately one month, the mother noted remission of the bullous lesions; however, maculae started to develop in these regions and persisted until the time of the visit.\nThe physical and intellectual development of the girl was considered to be normal by pediatricians despite the presence of discrete strabismus. After extra- and intraoral examination, a panoramic X-ray was requested to evaluate tooth germ development (Figure ) and to determine the reason for the lack of some teeth in the region of the third quadrant and the presence of conoid teeth 71 and 82. The X-ray revealed the absence of some deciduous and permanent tooth germs in the third quadrant (74, 75, 34, 35), as well as the lack of tooth 62 and of various permanent tooth germs at other sites. The mother was asked about the existence of family members with similar signs, which she denied. Hematologic tests showed no alterations.\nBased on the clinical history and clinical and radiographic findings, the final diagnosis was IP []. At present, the girl is under complete medical follow-up, mainly taking into account alterations in the skin, eyes, CNS and mouth. |
A 63-year old male presented with a history of lumbar laminectomy and fusion seven months prior to his initial presentation. He recalls that two days following his prone lumbar operation he began experiencing severe bilateral pain along the respective anterolateral thigh. Lumbar magnetic resonance imaging (MRI) ruled out spinal nerve root-related pathology as causative and a definitive diagnosis of bilateral MP, secondary to LFCN compression during the prone spinal surgery, was rendered. Conservative measures with oral analgesics were initially recommended given the typically self-limited course of this pathology. Despite an increasing regimen, including NSAIDs, narcotics, and anti-neuropathic pain medications, the pain persisted over the course of several months. Additionally, traditional anatomically-guided local anesthetic injections were attempted without improvement in his symptoms. Thus, the patient elected to proceed with operative decompression after eight months of failed conservative therapy and worsening quality of life.\nThe history of failed local anesthesia to even temporarily alleviate symptoms suggested a possible non-classic nerve location and prompted preoperative ultrasound to outline the superficial course of the LFCN. The ultrasound technique utilized has been previously described in the setting of percutaneous injection guidance (Figure ) [].\nSkin markings in the inguinal region and upper thigh indicating the course of the LFCNs were made by the ultrasound technician prior to operative site preparation for surgery. Indeed, anatomically variant LFCNs were identified bilaterally on the preoperative ultrasound with neither nerve passing in a classic location medial to the anterior superior iliac spine (ASIS) (Figure ). On the right, the ultrasound identified the LFCN passing directly over the ASIS, while on the left, the nerve was localized lateral to the ASIS. Oblique incisions were made centered over the marked LFCN locations identified by ultrasound. Both LFCN anatomical variants observed on preoperative ultrasound were confirmed after intraoperative dissection. Fascia overlying the nerves was opened to effect decompression. The fascial opening was confirmed adequate by ensuring the absence of entrapment along its course out of the pelvis into the thigh (Figure ). The patient experienced immediate resolution of his preoperative pain and was discharged home on the same day without complication. |
A 62-year-old male weighing 75 kg and with a height of 162 cm complained of discomfort in the throat. Preoperative laryngoscopy revealed a large laryngeal polyp attached to the right vocal fold that synchronously swung inward into the trachea and outward to the larynx with inspiration and expiration, respectively (Fig. ). The space between the vocal cords was too narrow due to the presence of the polyp for an endotracheal tube to be inserted. In addition, a surgical procedure could not be performed if the polyp moved into the trachea after endotracheal intubation. Thus, we decided to reduce the volume of the polyp by using a flexible bronchoscope through the LMA and then to perform total removal of the polyp by using a rigid laryngoscope with endotracheal intubation. If the airway did not remain patent by such airway management, we planned to perform temporary tracheostomy for removal of the polyp using a rigid laryngoscope.\nThe patient was not given premedication, and routine noninvasive monitoring including blood pressure, percutaneous oxygen saturation (SpO2), and end-tidal CO2 was performed in the operating room. SpO2 was 97% on room air, and oxygen was delivered by a facemask at 7 L/min in the operating room. During anesthesia that was induced with incremental administration of propofol (total dose of 170 mg), spontaneous ventilation was manually assisted and was followed by manual positive pressure ventilation without any airway obstruction. However, complete airway obstruction occurred immediately after a size 4 LMA (LMA Supreme™, Teleflex Co., NC, USA) was inserted. Maneuvers including changing the LMA position and increasing and decreasing the volume of cuff air did not improve the difficult airway. A flexible laryngoscope revealed airway obstruction due to the polyp lodged between the vocal cords (Fig. ), and the polyp could not be moved by pulling with forceps through the LMA. Spontaneous respiration returned, but the airway was still not patent even though the maneuvers were repeatedly tried. SpO2 decreased to 88%, and the LMA was removed. Soon after the removal, patency of the airway was dramatically improved and SpO2 returned to 100%. Transient tracheostomy was then carried out under general anesthesia with 2.0% of sevoflurane in 40% oxygen with assisted spontaneous ventilation and injection of 250 μg fentanyl. Resection of the polyp was successfully performed using a rigid laryngoscope. The tracheostomy was closed on postoperative day 5, and the patient was discharged on postoperative day 7. |
Our patient is 67-year-old Turkish man, a primary school graduate, and a forest ranger who lives in a rustic area. He was fully informed about the study and written consent was obtained from him prior to examination. In his medical examination he explained that his gums started bleeding at a very early age. At age 24, he started to smoke since he thought smoking would help to stop bleeding. Currently, he smokes 1-1.5 packs a day. At age 30, he started to experience difficulty in eating and complained of tooth mobility and gum bleeding. He claimed that his teeth ached a lot and as a result the teeth were extracted by a non-dentist or himself. Consequently, at the age of 50 to 60, he lost most of his teeth. At age 25, he was diagnosed with periodontitis. Nonetheless, he had not seen a dentist for this problem or performed any personal oral hygiene.\nOur patient complained of pain in the oral cavity especially on the tongue, buccal mucosa and had difficulty in chewing and swallowing solid food for six months.\nHis past medical history was significant for a tonsillectomy as a child. All the symptoms in his medical reviews were negative. Spirometric pulmonary tests were normal. High resolution computed tomography (HRCT) showed minimal emphysematous areas over both apices and non-specific sequelae (Figure ). Head and neck examinations were normal. Magnetic resonance imaging of the tongue revealed no sign of abnormality. He underwent extensive tests to identify the etiologies associated with systemic amyloidosis. Tests such as rectal biopsy, bone marrow biopsy, echocardiogram, abdomen tomography, serum and urine protein electrophoresis as well as liver function test were all normal. However, our patient was diagnosed as chronic renal failure with proteinuria and hypoalbuminemia (Table ). Renal biopsy showed AA type amyloidosis.\nClinical examination revealed poor oral hygiene and heavy plaque accumulation. Our patient was almost completely edentulous. The mean periodontal probing depth was 9.10 ± 0.84 mm (range 8-10 mm) in his remaining teeth. The tongue was diffusely enlarged (macroglossia) and clear red appearance and bilateral white plaques bleeding easily by gentle removing, and irregular translucent papules were present (Figure ). Based upon our patient's history, these papules developed spontaneously or after minor trauma. The enlarged tongue has interfered with speech and swallowing, and caused sleep apnea. In addition, two painful, small flat-based ulcers with erythematous halos and a white ulcer bed were detected, localized on the right and left buccal mucosa.\nCultures from the plaques on the tongue and oral cavity were all negative for bacteria and fungi. The biopsies were obtained from the tongue, buccal mucosa and retromolar trigon. Secondary amyloidosis (AA type), was diagnosed by histological and immunohistochemical findings (Figure ).\nOur patient received comprehensive periodontal therapy, which included careful oral hygiene instructions, curettage combined with non-surgical and surgical therapy. Serum inflammatory markers and some laboratory data improved dramatically at the second week of the periodontal therapy more than at any other time interval (Table ). Restorative treatment started after his periodontal condition was stabilized (around three months after periodontal therapy). Intra-oral radiographs showed poor bone density (Figure ).\nThe Congo red method was used to detect amyloid in tissue sections []. Amyloid was identified as the AA type on immunohistochemical tests with the use of monoclonal antibodies specific to amyloid A []. |
The patient is a 49-year-old male with a body mass index of 22.15 and no significant past medical history who underwent a THA on the right side. He has no history of diabetes; however, he does smoke multiple cigarettes a day. He was positioned in the lateral decubitus position with an axillary roll for a posterior approach to the hip. The operation lasted approximately 2 h and 45 min and was without complication. The patient was recovering appropriately until post-operative day 1. At that time, examination revealed absent wrist and finger extension with pain and numbness on the dorsum of the hand. Anesthesia was consulted and helped to formulate the diagnosis of radial nerve palsy. The patient was placed in a wrist brace and began working with occupational therapy while in the hospital. At discharge, he continued to work with occupational therapy for the radial nerve palsy and was followed closely in the orthopedic clinic. At 2-week postoperation, he was progressing appropriately with his THA; however, he still lacked grip strength and could not extend his wrist or fingers appropriately. Therefore, an electromyography (EMG) was undertaken to evaluate the radial nerve further. EMG revealed normal sensory nerve action potentials in the median, ulnar, and radial nerves. However, compound muscle action potential showed slowing with conduction block in the spiral groove and decreased recruitment of the brachioradialis, extensor carpi radialis, and extensor indicis pollicis. The triceps were intact and unaffected. The results were consistent with radial nerve palsy in the spiral groove, or “Saturday night palsy” []. The patient has now been following in the orthopedic clinic for 2-month time, and the radial nerve palsy has been improving. The sensory deficits have decreased and now there is only pain and paraesthesia on the dorsum of the thumb. Weakness and inability to extend the wrist are still present; however, subjectively the patient feels that he is improving. At this time, the patient is following with neurology as well as the hand surgeons for further evaluation and treatment options. The hip arthroplasty is progressing appropriately as well and the patient has had no issues. At 6-month follow-up in the orthopedic clinic, the radial nerve palsy has fully resolved. Physical examination findings of the patient showed full wrist extension, wrist flexion, finger abduction, and finger flexion. Thumb extension and general strength continued to improve from the previous follow-up but was not fully resolved, rated at a 4/5. The thumb extension and general strength would continue to be monitored at the next follow-up visit. As symptoms were improving, no additional therapy or treatment was necessary. The hip arthroplasty progressed appropriately as well and the patient has had no issues since surgery. He is back to work without limitation and able to complete all activities of daily living. Finally, at 1-year follow-up, the radial nerve palsy continues to remain fully resolved with no additional complications. Physical examination findings of the left wrist showed full wrist extension and full finger extension with intact sensation to light touch throughout all radial nerve distributions. The patient no longer has any physical or sensory deficits due to the radial nerve palsy. Strength is now full, rated 5/5 in thumb extension. Evaluation of the patient was completed by the same physician throughout the course of treatment, and the patient will now continue to follow a normal post-operative total hip protocol for clinical visits. |
A 39-year-old female was referred to the orthopaedic clinic for a left hand mass that had been enlarging over the past 9 months (). Physical examination revealed a large, soft mass overlying the palmar and radial aspect of the thumb metacarpal with mild tenderness and intact thumb motion. Sensation and vascular status were normal. She had no fever or systemic symptoms and normal laboratory values.\nPlain radiographs revealed a large, noncalcified soft tissue mass adjacent to the thumb metacarpal with some metacarpal erosion (). T1-weighted MRI sequences showed a large heterogeneous mass encompassing the thumb metacarpal with focal signal change in the metacarpal (). On T2-weighted imaging, the mass had increased signal and was multilobulated (). There was enhancement of the flexor pollicis longus and extensor tendon sheaths.\nA CT of the chest was normal and there was no lymphadenopathy. An open biopsy of the tumor revealed a highly cellular proliferation of spindle cells in sheets consistent with a high grade synovial cell sarcoma.\nSurgical resection with a staged reconstruction was then performed. A staged reconstruction was chosen in this case to confirm negative margins prior to undergoing reconstruction. A vascularized autograft was chosen to optimize the chance of arthrodesis in the setting of planned adjuvant radiation therapy. The entire thumb metacarpal, trapezium, flexor pollicis longus, EPL, and first dorsal compartment tendons were resected and the wound was closed with provisional K-wire fixation of the thumb. Pathologic examination revealed negative margins. One week later, the patient returned to the operating room for reconstruction. The metacarpal reconstruction was performed with a 5.5 cm distal radial osteofasciocutaneous flap vascularized by the radial artery. The graft was fixed proximally to the index metacarpal with a 2.4 mm locking plate and lag screw and distally to the proximal phalanx with a separate 2.4 mm locking plate. The graft was positioned to provide for index finger to thumb opposition with the thumb IP joint in flexion. Full flexion of the index finger to the palm was obtained when the thumb IP joint was extended. Approximately one-half of the distal radial shaft and metaphysis were utilized, and the defect was prophylactically stabilized with a contoured volar locking plate (). An extensor indicis proprius to EPL tendon transfer was then performed to restore extension of the thumb.\nThe patient received adjuvant radiation to the area with a total of 50 grays of radiation. At 2 years, thumb IP range of motion was 0–70 degrees compared to 0–80 degrees preoperatively. She had functional pinch strength and no evidence of local recurrence (Figures and ). DASH and MSTS scores were 17.5 and 24, respectively. Clinical and radiographic arthrodesis has been achieved. |
A 60-year-old man was referred to our clinic presenting with 14-month history of persisting unilateral pulsatile tinnitus. He had no history of craniotomy, trauma and did not complain various other neurologic symptoms. Otoscopic examination, audiometry, and tympanometry showed normal finding. Physical examination by stethoscope revealed a pulsatile sound behind the left ear, about 2 cm above the mastoid process. Tinnitus Handicap Inventory (THI) quantitative questionnaire was performed by the patient, which scored 52 points. The patient complained high pitch sound like whistle regularly depend on his position. Manual compression in the left side of the neck and mastoid process led the noise to cease. An initial magnetic resonance imaging (MRI) with magnetic resonance angiography (MRA) demonstrated the suspicious left transverse venous sinus arteriovenous fistula (). Since MRA findings showed a suggestive DAVF, catheter angiography was obtained revealing abnormal vasculture along branches of posterior and middle meningeal artery, basal tentorial artery and early filling of the transverse/sigmoid sinus, which confirmed the diagnosis of DAVF (). Because there was no risk of neurological complications such as increased intracranial pressure, cerebral hemorrhage, focal neurological symptoms, we decided to wait until the fistula could be occluded spontaneously instead of endovascular embolization. Thus, we tried to compress the neck intermittently to help self occlusion. The patient was educated to conduct the dual compression of the carotid artery and jugular vein with seating position, above the neck carotid bifurcation with 2-4 fingers as a power of the flow sounds disappeared. The daily compression was applied 20-30 times for 10s each time. There were no side effects during manual compression, and the patient did not feel discomfort as he understood the manual compression well. And the patients were followed up every 3 months and the THI score and qualitative questionnaire were surveyed. After 1 month of external manual compression without further treatment, he reported resolution of pulsatile tinnitus and we could not observe any tinnitus sound by stethoscope. THI score decreased to 0 points at 3 months after treatment. And tinnitus did not recurred during 12 month follow up period. The patient was suggested for taking a follow-up imaging after symptom improvement. However, the patient already experienced symptom resolution and did not want further examination, so imaging work-up was not possible. |
A 43-year-old female was admitted to our hospital complaining of numbness in the left limb for one week. Past medical history was notable for the diagnosis of NMO and intracranial hemorrhage. The patient was diagnosed with NMO ten years ago due to repeated episodes of blurred vision and numbness and weakness in the limbs. Brain and spinal magnetic resonance imaging (MRI) at that time indicated lesions in the brain white matter as well as spinal cord involving cervical and thoracic regions. AQP4 antibody test was not performed. Considering the potential diagnosis of demyelinating disease, steroid pulse therapy was initiated and the patient improved after the treatment. After discharge, corticosteroid was gradually tapered and the patient was maintained at a low-dose corticosteroid and azathioprine in the long term. The patient was also diagnosed with left basal ganglia hemorrhage three years ago, presenting as right-sided hemiplegia and confusion. Brain angiography was not performed at that time, and the patient recovered after symptomatic treatment. Family members exhibited no sign of the case pathology. Upon examination, the patient appeared lethargic and sluggish, with normal vital signs. Visual acuity was impaired in the right eye with a score of 20/200 on testing. Pupils were 3 mm bilaterally, round and reactive. Regarding motor function, muscle strength was decreased in the left extremities (Medical Research Council strength score, grade 3). Hypoesthesia of the left side was also observed. Babinski sign was present bilaterally. Further investigations revealed a positive AQP4 antibody in the serum, with an elevated titer of 1:32. An initial diagnosis of NMOSD was made considering her medical history and radiological findings. Other tests including complete blood count, basic metabolic panel, serum glucose, and anti-nuclear antibodies were all normal. Brain MRI after admission indicated lesions with restricted diffusion in the right thalamus and hemosiderin deposition in the left basal ganglia (Figure ). Unexpectedly, brain magnetic resonance angiography revealed severe stenosis of bilateral anterior and middle cerebral arteries, as well as stenosis of the right posterior artery and the intracranial segment of the right internal carotid artery. Radiological findings, as a result, strongly suggested the diagnosis of MMD. Digital subtraction angiography further confirmed this diagnosis with findings of bilateral occlusion of distal internal carotid arteries and rich collaterals near the skull base (Figure ). After antiplatelet and other symptomatic therapies, the patient improved. She refused further treatment with vascular reconstruction surgery and received physical therapy at a local rehabilitation center. |
An 88-year-old woman had suffered from a cough for the past 2 years and had recently felt dysphagia. The dysphagia progressed to the extent that she was unable to tolerate solid foods. She also reported an episode of regurgitation of a mass into her mouth. Three months before, she was diagnosed with cervical esophageal cancer at the clinic near her home, at which point she was admitted to a local tertiary medical center. On the further examination, she was diagnosed with cervical esophageal cancer invading the hypopharynx (Figures and ). A histopathological examination revealed squamous cell carcinoma. Computed tomography and positron emission tomography (PET) revealed no lymph nodal metastasis or distant metastasis. She was recommended to undergo total pharyngolaryngoesophagectomy or chemoradiotherapy. However, she was elderly; she refused these invasive treatments and hoped to preserve her voice and swallowing function. She was therefore referred to our hospital for a second opinion. The present patient received a transnasal endoscopic examination. We have routinely performed the trumpet maneuver using transnasal endoscopy for patients with esophageal cancer since 2009, using the following procedure. First, the patient is asked to bow the head deeply in the left lateral position. We then place a hand on the back of the patient's head and push it forward. To examine the hypopharynx and the orifice of the esophagus, the patient is asked to blow hard and puff the cheeks while the mouth remains closed (trumpet maneuver). The image of the pharynx using the trumpet maneuver with transnasal endoscopy in the present patient is shown in . The postcricoid was difficult to visualize during esophagogastroduodenoscopy; however, the view could be improved by using trumpet maneuver. We therefore concluded that the tumor of the cervical esophagus had not invaded the hypopharynx. The pedicle of the tumor was located at the left-anterior wall of the pharyngoesophageal junction (). We therefore planned to observe it under general anesthesia. A specially designed curved laryngoscope was inserted into the anesthetized patient to create a working space in the pharyngoesophageal lumen. Initially, the tumor was invisible from the hypopharynx (), as most of it was located in the cervical esophagus. Using the forceps, the tumor was pulled up from the cervical esophagus to the hypopharynx (). The pedicle of the tumor was mainly located at the postcricoid area (). The flat superficial part and anal side of the tumor had spread to the cervical esophagus (). It was easy to grasp the pedicle with the forceps inserted transorally, and the lesion was moving well, so we concluded that the tumor had not invaded the muscle layer. |
A gravida 2, para 2 woman aged 44 years with a history of two cesarean sections was referred because of recurrent urinary tract infection and urinary leakage through the abdomen. In her past medical history, she had Sjogren’s syndrome and optic neuritis. In her past surgical history, she had a bladder injury in a caesarean section, which was repaired with a primary suture. One month later, she presented with a 10 cm subcutaneous hematoma at its largest dimension. The hematoma was drained surgically, and during the exploration, no source of bleeding was found. Four months later (in total, five months after the cesarean section), she was admitted to the hospital and referred for urinary leakage through the abdomen, about 5 cm below the level of umbilicus. The leakage was periodic and she had an episode of subcutaneous abscess, which was managed conservatively using antibiotics and non-steroidal anti-inflammatory drugs.\nA fistula tract was observed in a retrograde fluoroscopic examination; however, the distal end of the fistulous tract was not clear (). An ultrasound examination was also not helpful to clarify the lesion. Contrast-enhanced magnetic resonance (MR) urography () revealed a defect of 1.2 cm on the anterior wall of the bladder in the midline. Between this defect and subcutaneous fatty tissue, a fistula tract of 2 cm at its widest diameter was evident. In the subcutaneous fatty tissue, a 6x6.4x3.7 cm collection was directly related with the fistula tract. There was inflammation and possible infection surrounding the collection due to the dense contrast enhancement.\nThe patient underwent a laparotomy with the plan for excision of the fistula tract and repairing the bladder defect. A suprapubic incision parallel to the Langer’s lines of the skin was made with a 1.5-2 cm margin with the fistulous tract. The fistula tract was about 12 cm in length and 4 cm wide, and was totally excised with the involved skin (, ). The defect on the dome of the bladder, which is apart from the trigon, was closed in 3 layers. The rectus fascia was closed with 1-0 Vicryl Subcuticular tissue was repaired with interrupted 2-0 Vicryl and the skin was repaired with 3-0 Prolene sutures. Her two-month follow-up after the surgery was unremarkable. |
A 35-year-old Caucasian female patient presented to her local emergency department in November 2016 with a chief complaint of neck pain. The patient had a past medical history significant for Hodgkin lymphoma diagnosed in 1998 following excision of a neck mass at age 16. She underwent chemotherapy and mantle field radiation in 1998. The radiation targeted lymph nodes in the neck, axilla, and behind the sternum in order to encompass the nodal basin of her cancer and the common lymph node drainage areas. The patient denied any history of radiation to her face. She reported remission at the time of presentation for this complaint of neck pain and was not following with anyone for her history of HL. She had no notable past surgical history. Menarche was at age 13 and she gave birth to one child at age 18. The patient’s family history was unremarkable with the exception of ovarian cancer in her maternal great aunt. There was no family history of breast or thyroid cancer. The patient was a previous smoker, quitting after about 10 years of use. No drug or alcohol use was recorded.\nIn the emergency department, a neck CT revealed a subcutaneous mass over the mid-clavicle, a breast mass, and multiple nodules in the thyroid gland with the largest nodule measuring 1.5 × 1.6 × 2.0 cm. The breast mass had dimensions of 2.6 × 4.0 × 4.9 cm by ultrasound. The patient was instructed to follow-up in breast and thyroid clinics for these findings.\nThe patient followed the emergency department’s recommendations and was examined by a surgical oncologist. In the breast clinic, she stated that the large right upper-outer quadrant breast mass had been present for 1 year. She was unsure how long the mass overlying the clavicle had been present, as it had been asymptomatic. On physical examination, the patient appeared well developed and well nourished. Respiratory, abdominal, musculoskeletal, and cardiovascular systems were normal. An 8.0 cm mass was located in the upper-outer quadrant of the right breast centered at the 10:00 axis about two fingerbreadths on the nipple border. Nipples were normal bilaterally. There was no cervical, supraclavicular, or axillary lymphadenopathy. Directly overlying the clavicle about two fingerbreadths medial to the mid-clavicular line was a 0.6 cm mobile mass within the skin. It was not associated with any regional lymphadenopathy. Laboratory workup was negative and unremarkable.\nThe patient underwent a bilateral mammogram in December 2016, followed by ultrasound-guided core biopsy of the breast mass. Initial core biopsy performed at an outside institution of the right breast mass came back as fibrocystic change. The outside biopsy was not reviewed at our institution. Based on a high level of clinical suspicion, additional imaging and a repeat biopsy were performed at our institution in January 2017. The repeat biopsy of the right upper-outer quadrant breast mass showed a phyllodes tumor. Pathology results described a fibroepithelial lesion with hypercellular stroma, mild-moderate stromal cytologic atypia, increased stromal mitotic activity (4-5/10 HPF), and focal areas suggestive of phyllodes architecture. The nature of the margins (pushing or infiltrative) could not be determined from the biopsy material. MRI showed the phyllodes tumor in the right breast measuring 4.7 cm. An excisional biopsy of the clavicular mass was done in a separate operation. The biopsy result was a cutaneous adnexal adenocarcinoma with eccrine differentiation. Surgical excision was recommended for both the breast and clavicular masses (Figures and ).\nThe patient also followed up in thyroid clinic for the multinodular goiter seen on her CT scan of the neck in the emergency department. Review of systems in the thyroid clinic was negative for change in voice or positional dyspnea but was significant for difficulty swallowing that started roughly 3 months prior. The patient also had pain in the right lower neck. She described the pain as constant, with an intensity of 5/10, and alleviated by acetaminophen. Ultrasound revealed three complex nodules with the largest in the left lobe measuring 1.3 × 1.8 × 2.5 cm, and other smaller nodules. The patient was diagnosed with multinodular goiter at this time. Two nodules met criteria for FNA. Cytology for both nodules was benign. The patient elected to defer any intervention and did not continue to follow-up.\nIn early March 2017, the patient underwent wide local excision of the phyllodes tumor, wide local excision of the cutaneous adnexal adenocarcinoma and right axillary sentinel lymph node biopsy, and concurrent post-reduction bilateral oncoplastic reconstruction. Surgery entailed intra-dermal injections of Tc99m-filtered sulfur colloid 1-2 cm from the margins of the lesion located over the right clavicle. Lymphoscintigraphy revealed uptake in two right axillary nodes. Once in the operating room, a standard axillary incision was made and 2 “hot” and blue lymph nodes were identified and removed. The cutaneous adnexal adenocarcinoma was then resected with a 1.5 cm margin which created a 4 × 10 cm ellipse. A lumpectomy was performed through predesigned incisions to ensure a cosmetically favorable closure for the phyllodes tumor in the right upper-outer breast. After removal, the plastic surgery team completed a bilateral breast tissue rearrangement and left breast reduction for symmetry. All aspects of the operation went smoothly, and the patient recovered uneventfully.\nPathology confirmed a phyllodes tumor measuring 4.1 cm in greatest diameter and clear margins. The phyllodes tumor pathology showed a circumscribed border, mild to moderate stromal cellularity, mild stromal cytologic atypia, and a mitotic rate of 4-5/10 HPF. Necrosis and malignant heterologous elements or stromal overgrowth were not identified. Overall, features were consistent with a benign phyllodes tumor (Figure ).\nThe adnexal neoplasm in the right chest was resected with negative surgical margins, and 0 of 2 nodes were positive for metastatic disease. The pathology report noted the presence of mitotic figures and rare atypical mitotic figures, favoring the diagnosis of a malignant adnexal neoplasm. The report adds that since the breast is a modified sweat gland, it is impossible to distinguish a primary cutaneous adnexal neoplasm from a primary breast neoplasm based on histologic features and that no immunoperoxidase stains can distinguish these two entities.\nOn her first postoperative clinic visit, the patient was recovering well. Her incisions were clean, dry and intact without erythema, drainage, hematoma or seroma. The patient has since continued to follow-up and has not experienced any complication or recurrence. She is recommended to follow-up annually. |
The patient is a 50-year-old female who had a 4-year history of left lumbar radiculopathy which was precipitated from a lifting injury that did not resolve following L4-5 microdiscectomy. She had previously been unsuccessfully managed with epidural steroid injections and multimodal analgesics. A nerve conduction study was unremarkable for any pathology. She was diagnosed with failed back surgery syndrome and counseled regarding her treatment options, including spinal cord stimulation. She elected to pursue spinal cord stimulation with the Nevro Senza HF10 system. As part of her evaluation for SCS placement, the patient was seen at the behavioral medicine clinic and underwent presurgical psychological testing and evaluation by a board-certified psychologist. Her psychological assessment profile suggested a high likelihood of a good postoperative outcome. She had a history of depression and anxiety, both of which were stable and under medical management. The patient underwent an uneventful SCS trial with near 100% relief of her back and leg pain and wished to proceed with permanent implant. Her permanent placement procedure was uneventful with leads placed at the same levels as her trial leads (.) with the tip of the right lead at the top of the T8 vertebral body and left lead tip at the mid T9 vertebral body. The device was activated in the post-anesthesia care unit with bipole setting on electrodes 10 and 11.\nThe first day following her procedure, the patient noted a buzzing or a chirping sound predominately in her left ear as well as vertigo, intermittent involuntary left facial twitches, and perioral numbness, all of which were new to her. She also reported nausea, vomiting, and diarrhea, which she has attributed to anxiety in the past. She was subsequently evaluated by ENT around 4 weeks postoperatively and underwent an audiological evaluation with no abnormal findings. There was concern that the tinnitus could be related to her stimulator. She also later complained of implanted pulse generator (IPG) site pain and stinging sensations. Attempts at reprograming her SCS system with a bipole settings on leads 4 and 5 failed to resolve her ongoing neurologic symptoms. The patient was subsequently advised to deactivate her system to see if her symptoms would improve. Two days after deactivation of her system, the patient reported complete resolution of her symptoms. She was again evaluated and spine radiographs were performed which demonstrated unchanged position of her SCS leads. She was not interested in reactivating her system to assess for return of symptoms. The SCS system was subsequently explanted. At the time of explant, there were no visible defects in the SCS system. |
A 68-year-old woman presented with decrement of visual acuity of the right eye 1 year ago. Magnetic resonance (MR) imaging revealed a tuberculum sellae meningioma (). The tumor was enhanced with homogeneous intensity after administration of a contrast material. After a year, she experienced complete loss of visual acuity of the right eye without light sense. Her visual acuity on the left side also began to decrease to 0.4 and the visual field reduced severely. At this point, we undertook surgery.\nThe tumor was removed through a right pterional approach. The subfrontal route was mainly used to resect the tumor. After retraction of the right frontal lobe, both the tumor and the superiorly displaced right optic nerve trunks penetrated by the tumor were exposed. However, we could not recognize the penetrated optic nerve for a while. It appeared as parts of the tumor because the tumor lay in the middle of the penetrated optic nerve and the two penetrated trunks were thin (). The tumor was attached to the tuberculum sellae, which was considered as the site of origin confirming the diagnosis of a meningioma originated from the tuberculum sellae. The tumor was dissected from the adjacent cortices and nerves by maintaining arachnoid planes. The whole course of the nerve could be visualized with the removal of most of the tumor.\nTo confirm the penetration, the operator biopsied the bulged tumor (black arrow, ) penetrating the optic nerve. The tumor around the optic nerve was resected gross totally without tumor enhancements in postoperative MRIs (). The remaining preserved optic nerve had a penetration after tumor resection (black arrow, ). Pathological examination revealed the absence of neural tissue in the bulging tumor between the nerve trunks. No neural or glial tissue was found in the biopsy sample, and the pathologies of the main tumor mass and the bulging tumor were identical-grade I meningothelial meningioma with a Ki-67 index of 2%. Postoperative course was uneventful and blindness in the right eye was unchanged. However, visual acuity of the left eye improved slightly to 0.6 and its visual field improved within 1 month post-operation (). No additional events or symptom aggravation were noted at the 1-year follow up. |
A 43-years-old woman, born in Ukraine, living and working in Italy since 1999 (nine years), came to observation of the First Aid Unit of Ferrara Hospital in March 2008, due to an epileptic seizure with generalized convulsions which occurred while she was working as assistant for a patient of the Pneumology Unit, in the same Hospital. She was found by the nurses of the ward, prone on the floor, unresponsive and presenting diffuse muscular jerks. This episode had spontaneous resolution in a few minutes and the patient completely regained consciousness. No signs of sphincters relaxation or secondary trauma were evident. Her vital parameters never got altered during and immediately after the seizure and she had normal blood pressure and body temperature. The nurses reported appearance, a few hours before the seizure, of a brief, sudden episode of diminished awareness of environment and inability to respond to external stimuli; this was erroneously blamed to tiredness and lack of sleep.\nNeither medical therapy at home nor voluntary drug abuse or toxin exposure were reported. She never had epileptic events or other neurological symptoms before. In her past clinical history no significant diseases could be found, except for a surgical intervention of saphenectomy in February 2008. She did not report familiar or personal history of psychiatric or neurological disorders, or, in particular, of epileptic disease.\nShe was working as an assistant for a disabled lady and had always done an efficient and competent job.\nThe patient got married when she was twenty-five years old and divorced ten years ago. She had one pregnancy; her daughter is twenty years old. She doesn't smoke and she drinks alcohol only occasionally. She is 165 cm high and she weights 63 kg.\nFriends described her as a clever, polite, mild woman, able to speak a good Italian in a short time.\nTaken to First Aid Unit and after medical evaluation, the patient was addressed to Brain CT in order to exclude haematomas or vascular injuries; she was then taken to Neurological Unit.\nThe patient was admitted in the Neurological Unit of Ferrara Hospital due to an acute, apparently generalized, convulsive seizure\nPast medical history was not significant. A mild change in her emotional balance and behaviour control was reported in the past few months, when she felt more anxious than usual, with occasional compensative alcohol abuse.\nPhysical and neurological examinations and Brain Computed Tomography Scan (fig. Fig. ) on admission were unremarkable as well as the standard Electroencephalogram, which showed a bilateral, symmetrical 10 Hz Alfa rhythm with normal arrest reaction to eyes opening, and did not reveal any epileptic rhythms or figures.\nThe routine Electrocardiogram and laboratory tests (blood cells count and morphometry, plasmatic glucose level, seric electrolytes, coagulation, kidney and liver function indexes) didn't show any significant abnormalities, except for a mild red blood cells macrocitosis (MCV 97 fl). Blood pressure and temperature were normal.\nThe patient had no evident epileptic relapse during the subsequent clinical observation.\nAs the first line routine investigations did not reveal the cause of her epileptic episode, we planned to perform second level diagnostic tests, particularly sleep-deprivation sensitized electroencephalogram and cerebral MRI (Magnetic Resonance Imaging), in order to unravel the epileptic focus or possible organic brain pathology.\nNevertheless, we could not complete our diagnostic program: the patient progressively developed an intense anxious state, explained away with the fear to lose her job because she was ill. For this reason she left the hospital two days after the admission, against physician's opinion.\nA month later, the woman visited again the First Aid Unit, taken by her friends, upset by her sudden change in behaviour: she did not speak, even if she was able to understand and respond with gestures and signs; she was slow in all daily activities; she seemed uninterested and emotionless, lost in her world. Since her first visit to Hospital she didn't experienced any epileptic symptom.\nAfter medical examinations, psychiatric and neurological consultations, the patient was hospitalized in a Psychiatric Unit for acute inpatient.\nThe psychiatric diagnostic possibilities were: a psychotic acute crisis, a major depression episode or hysteria.\nDuring the first week of hospitalization, the major difficulty has been to establish a dialogue with her. She hardly communicated, she was very confused and she looked like she was experiencing visual hallucinations: her eyes were like following images in the space around her, and her hands were moving in front of her eyes, in a way similar as she had to get rid of webs. She couldn't speak Italian anymore, – although she was able to speak Italian before- but kept on understanding what we told her; she was still able to speak Russian, her mother language. Some symptoms could be reconnected to our diagnostic hypothesises, but, at the same time, others ruled out them [-]. During this period she took low doses, 3 mg/die, of Risperidone, an antipsychotic drug, without clinical improvement. During following days, the clinical development was very variable and characterized by fluctuations of the consciousness state, stereotypic movements, urinary incontinence and vomiting episodes. A significant episode occurred when we asked the patient to make her signature and she did it with syllable reduplications. This one and subsequent neuro-cognitive tests, such as the "clock drawing test" (fig. ), suggested a neurological diagnostic hypothesis.\nWe requested a new neurological consultation, where the patient showed any peripheral neurological signs, but several cognitive symptoms as apraxia, disturbance in executive functioning and partial amnesia, beside aphasia.\nA second Electroencephalography examination showed a slow basic activity in both brain hemispheres and marked focal epileptic type anomalies in right frontal area, with a tendency to spread on the left homologous region.\nFocusing on inflammatory or infective disease, we sent the patient, forty days after the first – negative – TC, to a new neuroradiological evaluation, and the specialist chose to undertake Brain Nuclear Magnetic Resonance: it evidenced a large lesion in the right frontal lobe, a smaller lesion in the left frontal lobe, a large irregular halo of altered signal on both sides, probably attributable to perilesional oedema.\nMost likely, a contrast CT would have highlighted such big lesions in a similar way, but MRI was first undertaken, thinking it was an inflammatory or infective disease.\nAfter Brain Magnetic Resonance, the patient was quickly transferred to the neurosurgery unit.\nA magnetic resonance imaging (MRI) showed two frontal focal lesions (4 × 3 cm on the left side and 5 × 4 cm on the right side) with peripheral contrast enhancement, surrounded by T1-ipointense and T2-hyperintense signal limited to frontal lobes (fig. Fig. ). Functional sequences detected increased microvascular permeability and cerebral blood volume, suggesting neoangiogenesis.\nHer neurological examination was characterized by lethargy, apathy, mutacism, visual hallucinations, impairment of 6th right c.n. and papilloedema.\nWe administered antiedemigenic therapy with high doses of mannitol and dexamethasone.\nIn consideration of the neuroradiological findings, of inability to distinguish between primary and metastatic lesions and of the absence of other primary lesions on the thoracic-abdominal computed tomography (CT) scans, we performed surgical removal of the left frontal lesion. Our choice to approach the left lesion was related to its important mass effect and cortical localization. The removal was radical as confirmed by the post-operative CT scans(fig. Fig. )\nAfter surgery, we assisted to rapid awakening and a gradual improvement of apathy and mutacism. Glioblastoma was histologically defined and then we performed the total removal of the contralateral lesion. Also in this case, histology reported the presence of a glioblastoma. A post-operative CT confirmed the radical tumors excision (fig. Fig. ).\nThree weeks later the patient received limited field irradiation radiotherapy (40 Gray in 15 fractions over a period of 3 weeks) and temozolomide.\nTwo months later, the patient was able to perform her daily life activities with a mild disphoria (KPS – Karnovsky Performance Scale 100). |
A 66-year-old woman complained of an intractable swelling of the right ankle and difficulty in wearing shoes on the affected side because of the swelling. She had a medical history of type 2 diabetes, hypertension, and dyslipidemia. The patient had become blind due to diabetic retinopathy at the age of 35 years. After an inversion sprain of her right ankle which occurred two years previously, which was treated conservatively by her local doctor, she began to feel discomfort in the ankle. She noticed that the anterolateral part of the ankle gradually got swollen. The patient visited a nearby orthopedic clinic about a year after the episode of ankle sprain and was diagnosed with lateral premalleolar bursitis. Conservative treatment including several aspirations and corticosteroid injections failed to reduce the size of the bursitis, and the patient was referred to our hospital for surgical treatment. Physical examination revealed a fluctuant mass 5 × 8 cm in size, over the anterolateral part of the right ankle (). There was no local heat or redness. A callus was formed just over the surface of the mass on the right foot and at the same position on the left foot. Tenderness was localized around the anterior talofibular ligament (ATFL), and instability and apprehension were evoked by the anterior drawer test. Clear yellowish fluid was aspirated from the mass and was cultured, but no organism growth was observed.\nPlain radiographs revealed no apparent abnormality except for a round soft tissue shadow corresponding to the lesion in the anterolateral aspect of the ankle. Varus and anterior instability were obvious with stress radiography (). Magnetic resonance imaging (MRI) of the right ankle displayed a homogeneous multicystic lesion in contact with the anterolateral capsule of the ankle joint that had isointensity on T1-weighted, high intensity on T2-weighted, and high intensity on short T1 inversion recovery (STIR) images, indicating fluid collection in the lateral premalleolar bursa. MRI also showed unstrained ATFL and fluid collection in the sheath of the extensor digitorum longus (EDL) tendon (). These clinical findings and her past history prompted us to consider that chronic ankle instability and the disrupted capsule due to the past ankle sprain might have contributed to the recalcitrant bursitis with the disrupted capsule working as a check valve.\nWe performed bursectomy and ATFL repair for this case. In the operating room, ankle arthrography was performed before the surgery. Contrast medium was injected into the right ankle joint and the joint was passively moved to spread the medium. The arthrogram showed contrast medium leaking from the anterior aspect of the joint into the tendon sheath of the EDL and the distal tibiofibular syndesmosis and from the posterior aspect of the joint into the tendon sheath of the flexor hallucis longus (FHL) and the tibialis posterior indicating ruptures of the joint capsule of the ankle joint (). No leakage into the bursa was observed. Following the arthrography, indocyanine green was injected into the lateral premalleolar bursa percutaneously so that the margin of the bursa to be resected could be easily visualized. The indocyanine green injection was performed under arthroscopic observation of the ankle joint; however, leakage of the indocyanine green into the joint was not observed. Arthroscopy also revealed the tear of ATFL on the fibular side and a disrupted posterior capsule leading to exposure of the FHL tendon. Next, we performed open bursectomy with a transverse skin incision just above the bursa. The thin cutaneous layer around the bursa was stripped off with particular attention to the dorsal cutaneous nerve. When the underside surface of the bursa was being dissected from the underlying extensor retinaculum, the bursa was found to communicate with the tendon sheath of the EDL through a fistula 5 mm in diameter (). The fistula and inner side of the tendon sheath were stained with indocyanine green confirming a communication between the bursa and the tendon sheath. The bursa was completely removed and the fistula was closed with a few absorbable sutures. We then repaired the ATFL according to the modified Broström-Gould method using suture anchors. Postoperatively, full weight bearing was allowed immediately with a soft ankle brace. Activity without a brace was allowed at 12 weeks after the surgery. The surgical wound healed without complications. Histological investigation of the resected specimen revealed hyalinized fibrous tissue with proliferation of microvessels and granulation tissue and the migration of inflammatory cells, compatible with chronic bursitis. The patient was followed up for 17 months without recurrence of bursitis. The patient was satisfied with the result and had no difficulty wearing shoes and no functional disability at the latest follow-up |
A 68-year-old male presented to the emergency department with vague complaints of right-sided flank pain. The patient had a history of nephrolithiasis and underwent a CT abdomen (Fig. A). The scan revealed a large heterogeneous mass in the left upper quadrant. Subsequently, an MRI was performed, confirming a 16 × 9 cm2 complex mass with both cystic and solid components (Fig. B). This appeared to extend from the gastric wall and resulted in mass effect on the pancreas; however, there was no evidence of lymphadenopathy or metastatic disease. While this lesion was difficult to definitively characterize by imaging studies, a diagnosis of mesenchymal origin tumor such as a GIST was initially preferred.\nThe patient underwent endoscopic ultrasonography with fine needle aspiration of the mass. The lesion appeared to arise from the submucosa, as the mucosa was intact overlying the mass. On cytology, a paucity of bland spindle cells was present (Fig. A) and the cell block contained several small clusters of cells which stained strongly for CD34, but failed to stain for DOG1 or CD117 (Fig. B). Given the small amount of material present, additional testing for PDGFR and succinate dehydrogenase testing was not able to be performed at this time to confirm the diagnosis of GIST. GIST remained the favored diagnosis given the morphology and location of the tumor. The options were to repeat the biopsy or start imatinib therapy with a plan of repeat imaging after short-term follow-up. Because of extensive necrosis of the tumor, repeat biopsy was felt to be a less favorable option and the patient chose to undergo treatment with short-term imaging follow-up. Accordingly, the patient began treatment with imatinib. However, after 4 weeks of therapy, there was no significant radiologic regression. A second biopsy was performed and the specimen was sent for STAT6 immunohistochemistry (Fig. C). This revealed diffuse strong nuclear positivity and a diagnosis of SFT was made.\nPatient underwent en bloc resection of the tumor along with partial gastrectomy involving the lesser curvature of the stomach. The mass was found to arise from the lesser curvature of the stomach with extension into the lesser sac (Fig. D). The patient’s postoperative convalescence was uncomplicated, aside from a mild self-limiting ileus. Histopathologic analysis revealed a predominantly spindle cell tumor with marked variability in cellularity with areas resembling fibrosarcomatous transformation. Furthermore, zones of frank necrosis, invasion into muscularis propria and regions of marked pleomorphism were consistent with an undifferentiated pleomorphic sarcoma. The tumor displayed brisk mitotic activity with up to 15 mitotic figures per 10 high power fields and the resultant French Federation of Cancer Centers Sarcoma Group (FNCLCC) was histologic grade 3/3. Notably, the resection margins were negative for malignancy. The patient was prescribed to undergo surveillance imaging in accordance with the National Comprehensive Cancer Network (NCCN) guidelines every 3–6 months for the first 2 years after surgery. The follow-up CT scan performed 4 months after surgery showed a new 2.3 cm hepatic lesion suspicious for metastasis; subsequent biopsy confirmed the diagnosis of metastatic disease. He later underwent left lateral segment resection with uneventful postoperative recovery. The pathology report confirmed metastatic high-grade sarcoma with margins negative for malignancy (Fig. ). |
A 76 year old woman presented with a 12 month history of hypoglycaemia symptoms. The patient underwent a series of tests including measurement of overnight fasting plasma glucose, measurement of plasma levels of glucose; insulin; and c-peptide during a hypoglycaemic episode, measurement of urinary sulphonylurea, and radiological imaging with a CT scan. Abdominal CT scan demonstrated a 13 mm insulinoma localized in the tail of her pancreas. She was commenced on diazoxide and later underwent surgery for enucleation of insulinoma when a small (< 1 cm) incidental tumour was discovered on her stomach wall. The small incidental tumour was an exophytic gastric wall lesion that had not been detected pre-operatively and revealed no suspicious features suggestive of invasion or spread on intra-operative inspection. For this reason the lesion was excised with diathermy off the surface of the gastric wall, the defect being closed with Polydioxanone suture, and no frozen sections were taken to assess invasion/spread. Macroscopically the lesion was completely excised. The nature of the lesion was unknown at this time. Recovery was uncomplicated and she was discharged home a week later.\nThe pancreatic lesion (figure ) was composed of homogeneous soft tissue that had the typical red brown colour of such lesions although paler than usually seen, measured 13 mm in diameter, and its histology showed a well differentiated neuroendocrine tumour with nesting and trabecular pattern. The tumour cells were regular, their nuclei were round with vesicular chromatin and no mitosis was identified. Numerous psammoma bodies were seen in tumour nests and there was abundant hyalinised stromal reaction with no remarkable inflammation or necrosis. Immunohistochemistry showed strong positivity with chromogranin A, synaptophysin, progesterone, oestrogen-β and insulin markers. Very occasional cells were stained by somatostatin antibodies whilst pancreatic polypeptide, gastrin, amyloid and CD117 (C-kit) immunostaining were negative. There was a low proliferation index with less than 1% of tumour cells being stained by Ki67 antibody. Also, no vascular invasion was noted. The tumour was encapsulated and looked completely excised. The insulinoma was classified as T1NxMx.\nThe gastric lesion was a 6 mm firm whitish nodule in the upper wall of her stomach and its histology revealed an epithelioid cell tumour. Mitotic rate was very low and no necrosis was noted. Immunohistochemistry showed uniform positivity with C-kit (figure ) and CD34 markers. Desmin, smooth muscle actin, S100, cytokeratin and neuroendocrine markers were all negative. Ki67 immunostaining revealed very low proliferation index. We attempted performing mutation analysis on the GIST tumour but the DNA obtained was not of sufficiently good quality for the assay. We were unable to comment on the nature of the tumour margin because the GIST lesion was excised without any adjacent gastric wall tissue. The overall appearance was that of an epithelioid kit positive incidental GIST tumour and the lesion was incompletely excised.\nThe patient was re-admitted 6 months later with weight loss, anorexia and vague symptoms. She had a CT scan which showed a 3.9 × 3.9 cm fluid attenuation mass superior to the tail of her pancreas (figure ) displacing the stomach anteriorly. Endoscopic ultrasound confirmed a solid/cystic irregular mass involving the stomach wall, and in close relation to the pancreatic tail.\nShe subsequently underwent oesophagogastroduodenoscopy-guided endoscopic ultrasound confirming an ill-defined mass extending into the stomach wall. Cytology revealed groups of spindly and epithelioid cells highly suggestive of recurrent/metastatic GIST but not indicative of a neuroendocrine tumour (figure ). She was managed symptomatically, discharged and had another abdominal CT scan 7 months later that showed that the lesion had not significantly grown in size, but did appear to have formed 2 well defined cystic components measuring 29 × 28 mm, and 21 × 24 mm. Owing to the small size of the primary GIST and the frail state of the patient, she was not treated with Imatinib (Glivec) or offered further surgery but has subsequently been regularly reviewed by CT scan surveillance of the lesion and has fortunately had no evidence of tumour size increase or metastases. |
A 61 year old Caucasian man presented to our Accident and Emergency unit with a day history of right iliac fossa pain associated with fever and rigors. The appetite was reduced but no nausea or vomiting. The pain was said to be constant and sharp in nature and exacerbated by movement and stretching. He denies any history of a recent altered bowel habit or urinary symptoms. The only significant past medical history were renal calculi and well controlled asthma.\nPhysical examination revealed mild dehydration and normal vital signs. His abdomen was full with tenderness in the right iliac fossa and associated with guarding and local peritonitis. Blood investigations showed haemoglobin level of 14.0 g/dl, total white blood cell count of 22.4 with neutrophilia of 20.0, platelet count of 326, C-reactive protein of 36 and normal electrolytes, urea, amylase and liver function tests.\nThe initial diagnosis of acute appendicitis was entertained and patient was prepared and consented for an open appendicectomy. The findings at operation included a 4 cm by 5 cm pericaecal abscess mass adjacent to the anterior tenia coli. Within the abscess mass was a perforated anterior caecal diverticulum with necrotic wall. There was a polypoid mass within the wall of the caecum. The appendix was macroscopically normal with no evidence of acute inflammation. There was a suspicion of a perforated caecal tumour. He then underwent a right hemicolectomy with an ileo-transverse anastomosis through a medial extension of the appendicectomy wound.\nThe histology of the right hemicolectomy specimen macroscopically showed an inflamed and perforated solitary caecal diverticulum with abscess formation and an isolated caecal pedunculated polyp. Microscopically no dysplasia or malignancy within the caecal diverticulum and the polyp was a tubulovillous adenoma with low grade dysplasia. The caecal diverticulum lacked mucularis propria and therefore was considered to be acquired [Figures and ].\nHis postoperative course was uneventful and he was discharged home within a week of admission with an outpatient colonoscopy planned to evaluate the rest of his bowel. His follow up colonoscopy revealed further left sided colonic polyps with histology showing tubulovillous adenoma with moderate dysplasia. |
The patient is a 50 year old male who received a LURT 8 years prior to presentation. He had previously undergone bilateral native nephrectomies 2 months prior to transplant for PKD. His early course was complicated by biopsy-proven acute cellular rejection, vascular type, 5 days after transplant, which was effectively treated with anti-thymocyte globulin and intravenous immunoglobulin. He subsequently went on to enjoy excellent graft function. Initially, he was maintained on standard triple immunosuppression with tacrolimus, mycophenolate mofetil (MMF) and prednisone.\nTwo years prior to presentation, he developed numerous squamous cell carcinomas of the skin treated with resection and radiation. One of these lesions was an invasive poorly differentiated SCC (Bowen’s type) of the left auricle, requiring auriculectomy and reconstruction. Tumor margins were negative. His immunosuppression was reduced by stopping his MMF.\nOne year prior to presentation he developed a parotid mass found to be SCC by fine needle aspiration. It was felt that this was a metastatic lesion from the auricular tumor. At this time, he was switched from a dual immunosuppressive regimen of tacrolimus and prednisone to sirolimus (SRL) and prednisone. He underwent a left parotidectomy and neck dissection with pathology showing invasive keratinizing squamous cell carcinoma, poorly differentiated. The tumor was 4.6 cm with lymphovascular and perineural invasion. Surgical margins were negative, but 5 out of 23 periparotid and cervical LNs were positive for metastasis with focal extranodal extension. He underwent radiation therapy and cetuximab. A surveillance PET CT performed 6 months after treatment revealed 5 bilateral pulmonary nodules, which grew over 2 months from 6 mm to 10 mm. He initiated systemic treatment with carboplatin, paclitaxel and cetuximab with minor improvement initially, followed by disease progression in the lungs and mediastinum after 7 months of treatment. He was then treated with gemcitabine, and imaging after 2 months of therapy revealed tumor growth.\nA complex discussion was then held regarding symptom-focused palliative care or consideration of novel therapies. Next-generation tumor sequencing was performed on his lung biopsy specimen. Although no clear primary tumor driver was found, 16 genetic abnormalities of possible oncogenic effect were demonstrated, including an EGFR amplification event and a ROS1 mutation of uncertain significance. He enrolled in a clinical trial of the ROS1 inhibitor, entrectinib, but had clinical and radiographic progression within 6 weeks. Other clinical trial options were limited by his history of solid organ transplantation.\nWith his young age and active lifestyle, the patient opted to proceed with nivolumab 3 mg per kg therapy, understanding the high risk of alloimmune kidney transplant rejection. In preparation, sirolimus was tapered off and prednisone was tapered to 5 mg daily, after which his allograft function remained stable with a creatinine of 1.4 mg/dL. His sirolimus level prior to discontinuation was 6.9 ng/mL.\nThirteen days after receiving the first dose of nivolumab, he presented with low-grade fevers, oliguria and fluid retention. The physical exam demonstrated an enlarged and tender renal allograft and significant lower extremity and peri-orbital edema. Laboratory testing revealed marked acute kidney injury with a creatinine of 4.4 mg/dL. His sirolimus level was noted to be 1 ng/mL and he was treated empirically for acute rejection with a 3 day methylprednisone pulse but without improvement. A renal biopsy was deferred, as he was not a candidate for T-cell depleting therapy with his active malignancy and hemodialysis was initiated for volume overload and electrolyte disturbances. Given the life-threatening nature of his metastatic SCC, the graft was sacrificed and he continued on nivolumab therapy every 2 weeks. Imaging after 4 weeks demonstrated a partial regression in tumor burden and lymphadenopathy. For continued fevers, hematuria and marked allograft pain, an allograft nephrectomy was performed 2 months after stopping his immunosuppression. Histologic evaluation revealed hemorrhagic infarction with features of acute and chronic vascular rejection (Fig. ).\nNow, he continues treatment with nivolumab and most recent imaging 18 months after treatment initiation shows stable tumor regression. He has been maintained on hemodialysis, but has been able to travel and return to an active lifestyle. |
A 77-year-old woman was attended to our hospital for evaluation of an esophageal submucosal tumor (SMT) and complained of progressive dysphagia for more than 2 months. She had first noted intermittent difficulty in swallowing solids 2 months before the visit. Her symptoms worsened progressively over the past 1 month with difficulty in swallowing both solids and liquids. She had no significant weight loss during this time period. Her past medical history included a right buccal mass that had undergone resection 1 year ago. She had no history of any immunosuppressive disease, alcohol abuse, or smoking. The findings of physical examinations showed no abnormalities, and the superficial lymph nodes, liver, and spleen were not palpable. Laboratory tests revealed normal levels of blood routine, liver function, kidney function, and blood electrolytes. No hepatitis B surface antigen and hepatitis C virus antibodies were detected. No elevation of tumor markers or autoimmune antibodies was observed. Upper gastrointestinal endoscopy revealed a large rounded esophageal submucosal mass covered by normal mucosa, located at the lower esophagus, 30–34 cm from the central incisors (). Endoscopic ultrasonography (EUS) showed a well-demarcated hypoechoic mass chiefly located in the esophageal wall (), clearly separated from the surrounding adventitia. Findings on chest contrast-enhanced computed tomography (CT) revealed a well-defined homogeneous mass in the lower esophageal region, with size 18 mm × 28 mm (). Despite her age, in consultation with the patient, we chose and performed an ESD by injection-and-cut technique to completely remove the large esophageal lesion to allow for accurate histological diagnosis.\nESD was performed for diagnostic and treatment purposes. In this case, we used ESD-derived technique of submucosal tunneling endoscopic resection (STER). The submucosal injection was performed from the oral side at a distance of 3–5 cm from the tumor. Fluids were injected beneath the mucosa by a submucosal injection needle through the endoscopic channel to create a cushion. The fluid was a normal saline solution combined with 1:10,000 epinephrine and 1% methylene blue. A 2-cm longitudinal mucosal incision for a tunnel entry was made using a Dual knife. The submucosal layer was dissected using Dual knife and IT nano knife. Carbon dioxide was used for insufflation. After accomplishing the dissection, the lesion was removed using a basket and processed for histological evaluation. The dual knife was used for treating the possible vessels during the inspection. The mucosal incision site was closed with endoscopic clips (). The resected specimen measured 4.3 cm × 2.8 cm × 1.5 cm ().\nESD was completed without any complications. A broad-spectrum antibiotic and proton pump inhibitor were administered intravenously for the next 3 days after the procedure. The patient was fasting and receiving fluid therapy for 3 days. She was discharged 5 days after the surgery, and an oral proton pump inhibitor was prescribed for the next 4 weeks. The histopathological findings of the resected specimen showed infiltration of small- to medium-sized lymphoid cells with slightly irregular dark nuclei and abundant cytoplasm (). Neoplastic cells infiltrated the lamina propria to the submucosal layers. Infiltration of neoplastic cells in the lateral margins of the resected specimen was not observed. However, vertical margins showed an R1 situation and mild damage to the muscularis propria. Immunohistochemical studies revealed that the lymphoid cells were positive for CD20 (), CD19 (), PAX5 (), as well as BCL2 (), and negative for CD3 (), CD5 (), CD10 (), and cyclin D1 (). The percentage of tumor cells positive for Ki-67-staining was <5%, indicating few mitotic cells. The diagnosis of esophageal MALT lymphoma was confirmed based on these pathological features. The patient was not tested for Helicobacter pylori during hospitalization. Therefore, she was not treated for Helicobacter pylori eradication. During the follow-up visit, the patient complained that her 13 C-urea breath test result was negative for Helicobacter pylori in the past.\nAfter 3 months, her dysphagia disappeared and a follow-up endoscopy showed no recurrence or complication at the ESD site, except for the presence of a scar. During the follow-up visit, the patient received additional radiation therapy according to the oncologist's suggestion. After 5 months, the patient was still under surveillance and free of recurrent disease. |
A 69-year-old gentleman presented for consultation after being diagnosed with prostate cancer. His past medical history was significant for hypertension, hyperlipidemia, stable 5 mm left pulmonary nodule, mitral valve prolapse with moderate regurgitation, and BPH for which he underwent a simple prostatectomy ten years earlier. The patient elected decision to undergo simple prostatectomy a decade ago for BPH after alpha-blocker therapy and a short course of finasteride were ineffective in resolving his urinary retention. On this most recent consultation, physical exam was notable only for an estimated 60 g prostate with no nodules appreciated, suggesting cT1c prostate cancer. MRI showed extension of neoplastic tissue into the prostatectomy field (Figures and ). MRI fusion prostate biopsy performed for elevated PSA of 5.7 ng/mL demonstrated 4/12 positive cores, 2 Gleason 4+3, and 2 Gleason 3+4. Surgical history was also significant for an open left inguinal hernia repair that took place seven years ago. Preoperative imaging with CT of the abdomen and pelvis and whole body bone scan did not demonstrate any evidence of metastatic disease.\nThe patient elected to undergo robot assisted laparoscopic prostatectomy (RALP). Cystoscopy was performed prior to the procedure to assess bladder anatomy. At the start of the RALP procedure, the bladder was adhered anteriorly, requiring extensive lysis of adhesions and gently dissecting the bladder off the anterior abdominal wall. Upon entry into the space of Retzius, the endopelvic fascia was not incised to maximize nerve-sparing technique as part of Samadi Modified Advanced Robotic Technique (SMART) []. When the bladder neck was opened, ureteral stents were positioned by passing a wire through the side trocar and into the ureteral orifice using the robot and then passing the stent over the wire and through the port []. Stents were inserted in order to identify the exact location of the ureteral orifices, considering the change in normal anatomy following the previous surgery. Due to the change in the anatomy of the prostate and the patient's thin posterior bladder neck, tissue recognition was vital in this case. Posterior dissection was performed using cold scissors to minimize rectal damage and the bladder neck was opened. Nerve-sparing procedures were conducted using athermal technique by blunt dissection with round-tip scissors and performed in an interfascial plane, as opposed to an intrafascial one. The dorsal vein complex was cut with cold scissors just before removal of the specimen and then suture-ligated. The bladder neck was then reconstructed in a posterior tennis-racquet fashion with a narrower diameter of 18 Fr. After completion of reanastamosis, bilateral pelvic lymph node dissection was performed with minimal difficulty by removing the lymph package anterior to the obturator nerve and inferior to the external iliac vein. Total operative time was 145 minutes.\nPathologic examination of the specimens revealed a 76 g prostate with bilateral, Gleason 4+3 pT3a disease with extraprostatic extension, involving 58% of examined slides. The right posterior margin was positive focally. Lymphovascular invasion and peri- and intraneural invasion were present. No lymph nodes (0/3) were positive for disease.\nVoiding cystogram performed 10 days post-op did not demonstrate any extravasation or abnormal findings. On the patient's most recent follow-up, approximately 9 weeks post-op, he was noted to be continent, with minimal difficulty voiding, and had a PSA of <0.01 ng/mL. |
A 46-year-old Japanese woman presented with right hemifacial spasm that had persisted for 2 years, and was admitted to our hospital for microvascular decompression. She had shown no medical abnormality during the perinatal period and had developed normally. Furthermore, she had no history of malignant diseases or severe head injury, or any remarkable family history. Findings of physical and neurological examinations were unremarkable except for right hemifacial spasm with synkinesis. A head computed tomography (CT) scan revealed a small intraosseous defect 6 mm in diameter in the right occipital bone. Magnetic resonance (MR) images obtained using heavily T2-weighted MR cisternography showed an isointense mass 6 mm in diameter outside the right cerebellar hemisphere, corresponding to the bone defect. Around the mass, there was a high-intensity area similar to cerebrospinal fluid (CSF), which was continuous with the intracranial subarachnoid space (Fig. ), indicating that the mass was located within the subarachnoid space.\nOur patient underwent a right suboccipital craniotomy to create an opening 3 cm in diameter. After craniotomy, there were several small dural openings, causing some CSF leakage. The outer table of the detached bone was normal while the inner table was partially lacking, creating a tiny cavity filled with fragile reddish tissue (Fig. ). The tissue which was obviously separated from the cerebellar tissue was resected to be a subject for pathological examination.\nAfter successful microvascular decompression, the wound was closed using the bone graft for cranioplasty. Her postoperative course was uneventful and she was discharged without facial spasms or any neurological complication.\nThe specimen exhibited a fragment of cerebellar tissue with gliosis, as well as necrotic bone and fibrous interstitial tissue. The cerebellar tissue consisted of cerebellar cortex and white matter. The cerebellar cortex clearly exhibited the molecular layer and the granular layer; however, Purkinje cells were depleted, and had been replaced by proliferation of Bergmann glia. The white matter was gliotic, and meninges were evident at the surface of the cerebellum. There were no features indicative of neoplasia. |
A 24-year-old man whose focal seizures began at age 7 presented for further evaluation. During his episodes, the patient was reported as having a surprised look on his face, covering his mouth with his hands, and laughing. These episodes happened several times per day and were followed by marching movements of the legs and grunting noises. The patient had no alteration of consciousness during his seizures. He also experienced hypermotor seizures arising out of sleep at least nightly and often had several per night. The patient sustained bilateral frontal lobe damage, intraparenchymal hemorrhages, and extra axial hemorrhages following a severe fall in 2006. He was seen by several neurologists before coming to WFBH.\nThe patient’s epilepsy was refractory to multiple antiseizure medications. His initial EMU admission in 2001 was unable to localize the seizure onset zone. The interictal EEG showed very frequent epileptiform discharges arising from the right frontal region, but PET showed mild decreased activity in the left medial temporal lobe which was consistent with an epileptogenic focus. Both ictal and interictal SPECT indicated slightly asymmetric activity within the temporal lobes with the right side greater than the left, also suggestive of an epileptogenic focus within the left temporal lobe. Because of the discordant findings, the patient proceeded to invasive monitoring. ICEEG pointed towards a right frontal lobe origin, but the pattern of spiking occurred almost simultaneously with patient’s clinical semiology. At that time, it was felt that the seizure focus was not clearly localized to warrant right frontal lobectomy. The patient then received a VNS in addition to medication, and this combination was effective for several years.\nThe patient began to experience more problems around 2012 and was recommended for a MEG scan. The patient’s MEG data were very noisy owing to metal hardware in his skull that covered the burr holes from his previous invasive monitoring, but SAM(g2) sufficiently removed these artifacts and revealed a single focus in the right middle frontal gyrus (A) with numerous, MEG-only spikes (B). (Because the head is magnetically transparent, MEG is not susceptible to breach effects from craniotomies or other defects.) The MRI obtained for the MEG scan also revealed a small focus of cortical thinning and irregularity with subtle T2 hypointensity in the right frontal lobe. Based on the MEG results, the patient underwent invasive monitoring with a stereo-EEG array placed over the MEG focus (C). The patient’s epileptiform activity (green arrowhead, D) was subsequently localized to the three ICEEG contacts (green arrows) nearest to the peak of the SAM focus (red sphere, C). The patient then received a right frontal lobe resection of the seizure focus and has been seizure free since. |
We describe the case of a 55-year-old, para 7, postmenopausal black African woman who was initially seen with a history of vulval itching of 3 years’ duration and at that stage there were no lesions to report and the itchiness was treated with topical steroids with an initial good response. Seven months prior to seeing a gynecologist she noted three warty-like lumps on her right labia majora. She then presented to a gynecologist and an excisional biopsy was done. Histology surprisingly showed infiltrating moderate to well-differentiated adenocarcinoma with malignant glandular structures that were lined by tall columnar epithelium with stratification and mucin production. The warty lesions eventually became florid as shown in Fig. .\nSince this was an unusual histological type for vulval cancer, immunochemistry was requested which showed mucin, strongly positive cytokeratin (CK) 20 and CDX2, and negative CK7. There was minimal patchy staining for p16 and features suggestive of large bowel or pancreatic primary tumor. Several years back, she had a hysterectomy for abnormal uterine bleeding and histology showed several leiomyomata and no evidence of uterine or cervical cancer. In view of the unusual histology, a mammography was done which showed a solitary benign lesion in her right breast. Computed tomography with contrast of her chest and abdomen showed multiple parenchymal shadows suggestive of metastasis in her lungs and there was rectal thickening of uncertain significance with no obvious large bowel or pancreatic lesions.\nShe underwent sigmoidoscopy; the findings were essentially normal. A biopsy of an enlarged left groin lymph node showed the presence of metastatic adenocarcinoma with an immunohistochemistry profile again consistent with spread from colonic or rectal adenocarcinoma. When she moved to Zimbabwe she presented to the gynecological clinic with a history of lumps on her vulva. These warty lesions were biopsied and sent for histology and immunohistochemistry. The results showed adenocarcinoma and immunohistochemistry again was suggestive of gastrointestinal malignancy as a primary. She was then commenced on chemotherapy; she received six cycles of oxaliplatin and capecitabine daily for 2 weeks with a 1-week break. After 4 months, the lesions, however, did not resolve, instead she developed a flare of the vulvar tumor associated with severe itchiness and pain.\nA multidisciplinary team meeting involving gynecologists, general surgeons, radiologists pathologists, and radio-oncologists agreed to proceed with a colonoscopy and vulvectomy to alleviate her debilitating symptoms. A colonoscopy revealed anorectal cancer, and histology confirmed invasive adenocarcinoma of villoglandular type in keeping with anal rather than vulval origin. A vulvectomy was done and she commenced on radiotherapy. On clinical examination she was doing well and was relieved of her symptoms. Despite the chest metastases, she has never had significant pulmonary symptoms and she has always been clinically well except for the vulval symptoms.\nFigure shows vulva preoperatively, Fig. shows the vulva immediately post operation, Fig. shows the vulva 1 month post operation and Fig. shows the vulva 2 months post vulvectomy. |
A 42-year-old Hispanic male presented to our hospital with worsening shortness of breath of 3 weeks' duration, cough with green color sputum, subjective fevers, decreased PO intake, and as per the patient a 40-pound weight loss. His weight documented on previous admission was approximately 175 pounds, and upon the current admission the patient weight approximately 158 pounds amounting to a weight loss of 9.71% in approximately 2 months. Four months prior to this presentation, the patient was admitted to our hospital with similar complaints and he was diagnosed with Klebsiella pneumoniae with parapneumonic effusion. The patient was given antibiotics and thoracentesis was done. Pleural fluid analysis is shown in Table . His past medical history including liver cirrhosis secondary to NAFLD, hypothyroidism, and morbid obesity for which he had undergone Roux-en-Y gastric bypass 7 years prior to the current presentation. He also developed achalasia, 6 years after gastric bypass surgery for which he got Botox injection with symptomatic improvement. His initial labs were essentially benign, physical exam was normal except for diminished lung sounds in the right lower and right middle lobes. Computed tomography of the chest was done which was consistent with a thick-walled fluid collection at the right lower chest cavity containing fluid and air, suspicious for empyema (Fig. , ). The patient was admitted to the general medical floors and started on meropenem and linezolid. A 14-Fr pigtail catheter was inserted in pleural cavity with removal of approximately 200 cm3 of thick purulent pleural fluid. The fluid was sent for evaluation including cytology which was suggestive of empyema (Table ). Due to decreased drainage of the pleural fluid through the catheter which was likely secondary to loculations and worsening of patient's respiratory status, surgery was consulted for video-assisted thoracoscopic surgery (VATS). The patient underwent VATS which showed extensive empyema with numerous adhesions and a thickened pleural peel. The patient underwent extensive lysis of adhesions, and right lower lobe wedge resection, and insertion of two 32-Fr chest tubes for continued drainage. During the VATS procedure, the pleural fluid was sent for analysis including anaerobic and aerobic Gram culture and stain which was positive for C. difficile. Intravenous metronidazole was started. The patient had no history of CDI, and blood cultures were negative for C. difficile but were positive for K. pneumoniae. A stool C. difficile toxin assay was sent which returned negative for toxin, antigen, and PCR testing. The chest tubes were removed and the patient recovered well. |
A 13-year-old female Motswana student, who is the first born in a family of two children and being raised by a single unemployed mother, was referred to a psychiatric hospital by her local facility. She presented with a 1 week history of calling out for people who were not there as if she were conversing with them and seeing things other people could not see. She was also reported to often appear anxious and was not sleeping well at night. The symptoms appeared to worsen daily, prompting the caregivers to seek help. There have been no preceding life events that may have precipitated the symptoms, and she had never been admitted or been on treatment for any psychiatric disorder before the current presentation.\nMs K was born via a normal vaginal delivery at 32 weeks gestational age, with a birth weight of 2.1 kg. The mother was Gravida 2, Para 2, and antenatal history was unremarkable. Her mother highlighted that the patient had delayed developmental milestones as she did not walk and talk until after age two. Regarding social development, Ms K was reported to prefer solitary activities, and if she interacted with others, she would choose children younger than her. She reportedly attended a normal stream school for 2 years but was transferred to a special needs school (i.e. school for children with learning difficulties) because of academic difficulties.\nOn medical history, she has been diagnosed with a complex congenital heart disease: pulmonary atresia, large ventricular septal defect, pulmonary regurgitation, right ventricular hypertension and failure. She underwent corrective surgeries for the cardiac conditions at ages 6 years and 12 years, an umbilical herniorrhaphy at 3 years and clubfoot repair at 2 years.\nTypical features of DGS on physical appearance were a broad flat nose, small ears and a thoracolumbar scoliosis, whereas the typical long face, hypertelorism and micrognathia were absent. Physical examination revealed a mediastinal scar and a pansystolic murmur. Her blood pressure was 113/83 mmHg, pulse rate was 114 beats/minute and temperature was 35.8 °C. Investigations such as full blood count, liver function test, urea and electrolytes were within normal ranges. A chromosomal analysis was positive for chromosome 22 deletion syndrome.\nMental status assessment on the index consultation revealed a well-nourished adolescent. She was very restless, pacing up and down the interview room, thus making it difficult to establish a rapport. There was no eye contact. She had a labile affect. She was socially inappropriate as she kept undressing during the interview. Thought process was mostly tangential. She had fixed belief that her family members were bewitching her. She reported that God was commanding her to take her clothes off and she admitted to seeing a short man in the interview room.\nA working diagnosis of acute schizophrenia-like psychotic disorder was made using the International Classification of Diseases-10 (ICD-10) diagnostic criteria. The patient was admitted on haloperidol 3 mg at night. Four days post admission, she was noted to have increased motor activity, restlessness and sialorrhea, whereas the psychotic symptoms persisted. Manic symptoms such as elated or irritable mood and increased energy were absent. On suspicion of akathisia, haloperidol was stopped and she was started on olanzapine, 5 mg once daily at night. The extra pyramidal side effects (EPSE) symptoms reduced after 2 days. Psychotic symptoms subsided on day nine post admission. She stabilised 2 weeks after admission and was discharged on olanzapine 5 mg once daily at night. She was reviewed in an outpatient clinic after 2 weeks and remained stable. She continued reviews as an outpatient for 4 months and medication dose was reduced to 2.5 mg nocte. A month later, she presented to OPD with history of poor sleep at night, laughing inappropriately and isolating self. Medication was reviewed upwards to olanzapine 5 mg nocte as it was on discharge. For long-term management, patient was to be enrolled in vocational training and behavioural therapy. A definitive diagnosis of early-onset schizophrenia in a patient with DiGeorge syndrome was made. |
A 24-year-old man whose focal seizures began at age 7 presented for further evaluation. During his episodes, the patient was reported as having a surprised look on his face, covering his mouth with his hands, and laughing. These episodes happened several times per day and were followed by marching movements of the legs and grunting noises. The patient had no alteration of consciousness during his seizures. He also experienced hypermotor seizures arising out of sleep at least nightly and often had several per night. The patient sustained bilateral frontal lobe damage, intraparenchymal hemorrhages, and extra axial hemorrhages following a severe fall in 2006. He was seen by several neurologists before coming to WFBH.\nThe patient’s epilepsy was refractory to multiple antiseizure medications. His initial EMU admission in 2001 was unable to localize the seizure onset zone. The interictal EEG showed very frequent epileptiform discharges arising from the right frontal region, but PET showed mild decreased activity in the left medial temporal lobe which was consistent with an epileptogenic focus. Both ictal and interictal SPECT indicated slightly asymmetric activity within the temporal lobes with the right side greater than the left, also suggestive of an epileptogenic focus within the left temporal lobe. Because of the discordant findings, the patient proceeded to invasive monitoring. ICEEG pointed towards a right frontal lobe origin, but the pattern of spiking occurred almost simultaneously with patient’s clinical semiology. At that time, it was felt that the seizure focus was not clearly localized to warrant right frontal lobectomy. The patient then received a VNS in addition to medication, and this combination was effective for several years.\nThe patient began to experience more problems around 2012 and was recommended for a MEG scan. The patient’s MEG data were very noisy owing to metal hardware in his skull that covered the burr holes from his previous invasive monitoring, but SAM(g2) sufficiently removed these artifacts and revealed a single focus in the right middle frontal gyrus (A) with numerous, MEG-only spikes (B). (Because the head is magnetically transparent, MEG is not susceptible to breach effects from craniotomies or other defects.) The MRI obtained for the MEG scan also revealed a small focus of cortical thinning and irregularity with subtle T2 hypointensity in the right frontal lobe. Based on the MEG results, the patient underwent invasive monitoring with a stereo-EEG array placed over the MEG focus (C). The patient’s epileptiform activity (green arrowhead, D) was subsequently localized to the three ICEEG contacts (green arrows) nearest to the peak of the SAM focus (red sphere, C). The patient then received a right frontal lobe resection of the seizure focus and has been seizure free since. |
Patient AM is a Caucasian female born at a community hospital by cesarean section to a 20-year-old gravida 1 para 0 mother of Western European descent. The mother had regular prenatal care, took prenatal vitamins and did not use alcohol, tobacco, or illicit drugs during the pregnancy. During prenatal visits, the fetus had reassuring vitals so an ultrasound was not obtained. The pregnancy was complicated by gestational diabetes and preeclampsia for which the mother had been placed on bedrest during the final trimester. At 42 weeks gestation, the mother was induced, but during labor, fetal distress was noted, requiring an emergency cesarean section to be performed. Birth weight was 7 lbs 4 oz. (3.29 kg) and length was 17″ (43.18 cm). After birth, our patient failed to spontaneously void and concern for acute renal failure resulted in her being transferred to a tertiary pediatric hospital for further evaluation. This later resolved during the hospitalization.\nDuring the admission, radiographs of the chest, abdomen, and pelvis were obtained. The images demonstrated multiple vertebral segmentation defects in the thoracolumbar spine and agenesis of the distal sacrum and coccyx. Our patient was noted to have fusion of multiple vertebrae within the cervical spine consistent with a Klippel–Feil deformity and partial fusion of multiple ribs bilaterally consistent with spondylocostal dyostosis. She was referred to pediatric orthopaedics. Since our patient was healthy and without other congenital anomalies, she was conservatively followed with serial radiographic evaluations every 6 months (). On exam, she was noted to have a short neck with limited range of motion. Bowel and bladder function were normal. Motor responses were good, and there was full strength in the lower extremities.\nShe began walking at eight months old. At one year of age, she was noted to have a 40° thoracolumbar scoliosis. At age three, she was noted to have a disproportionately short trunk, but was otherwise well developed and wanted to participate in gymnastics. Over the next few years, our patient's congenital spinal deformity was stable. By age seven, she had developed an extremely protuberant abdomen and had prominences of her rib cage due to relatively little longitudinal growth of the spine. Her 3-year-old younger sister, who had no evidence of skeletal deformity, had surpassed her in height at this point. However, the patient was otherwise asymptomatic and doing well in school, subsequently participating in gymnastics, tumbling, and cheerleading with only minimal episodic back pain.\nAt age ten, radiographs began to demonstrate evidence of consolidation in the thoracic spine. Our patient continued to remain very active over the ensuing years with cheerleading and dance but had increasing back and chest wall pain with these activities. Due to her short truncal stature, she also began to have pain related to costo-iliac impingement that occurred when she bent over, sat, or coughed. She was managed conservatively with NSAIDs and core strengthening exercises which provided some relief. Notably, the patient did not have shortness of breath with her regular activities or frequent respiratory illnesses. By age 14, her spine had almost completely fused. She continued to follow-up with her orthopaedic surgeon every 3 years and had no notable progression of her spinal deformity.\nAt the age of 18, she became pregnant but subsequently terminated the pregnancy due to obstetrical concerns for compromised respiration secondary to limited thoracic volume. At the time, she also underwent a tubal ligation to prevent future pregnancies. At 21, she returned for follow-up. She was working a job which required her to remain standing throughout her shift that was causing her to experience increasing hip and knee pain. She also noted increasing back pain without neurological deficits that had progressed over the last 3 years. She had a notable leg length discrepancy due to pelvic obliquity, a result of her scoliosis. She was treated with a 5/8-inch shoe lift which provided some relief.\nAt the age of 25, she experienced an episode of severe back pain for which she visited the emergency department (ED). Radiographs were obtained which could not definitively rule out a vertebral compression fracture, so an outpatient bone scan was obtained. The scan demonstrated multiple foci of increased uptake in the vertebral bodies of the cervical, thoracic, and lumbar spine as well as the costovertebral region. These nonspecific findings were attributed to stress reactions from the patient's multiple vertebral anomalies. Several years later, she was involved in a high-speed MVA and was transported to the ED complaining of significant neck and back pain. At the time, computed tomography (CT) of the chest, abdomen, and pelvis and a posteroanterior (PA) chest film were obtained which illustrated her known vertebral, costal, and pelvic anomalies with no evidence of acute fractures (Figures –). She was next seen at age 30 after an accident on a “slip and slide” in which she dove and noted immediate pain in the lower right rib cage. Radiographs illustrated a nondisplaced fracture of the right 7th rib near the costochondral junction which was managed conservatively with NSAIDs (). The fracture subsequently went on to heal with resolution of symptoms at one month.\nShe is now 31 years of age and currently suffers from chronic back pain for which she takes naproxen daily. Two to three times per year she has exacerbations of mid-back pain, necessitating a visit to the emergency department for intravenous narcotic analgesics. She also complains of intermittent sciatic neurogenic symptoms including numbness and paraesthesias down the right lower extremity which are exacerbated by sitting in the car for extended periods of time. She occasionally has dyspnea with exertion after walking long distances or climbing the stairs. She feels that her shortness of breath has always been present, but only has limited her during certain activities in the past three years. Given the intermittent nature of her respiratory issues, she has not required a pulmonary function workup; however, this may be necessary moving forward. She currently smokes a half pack of cigarettes daily and has a 3-pack year history. She is being encouraged to quit. She is now 54 inches tall (4′6″) and weighs 138 pounds. She has been counseled on diet and exercise given the risk of abdominal obesity causing further restriction of lung volume. She completed a Scoliosis Research Society 30 Questionnaire (SRS 30) at her most recent follow-up and 5 years prior. Notably, she felt that she looked worse in clothes and had a more negative body image at present. However, she has increased her level of activity despite increasing back pain at rest. |
A 32-year-old Chinese-Vietnamese nulliparous female presented to our clinic after trying to conceive for 1 year and 9 months without success. Her cycle length varied from 30 to 39 days. Body mass index was 19. Previous workup showed normal hormone levels and hysterosalpingogram. Antral follicle count was 16.\nThe patient's partner had normal semen analysis. Post-coital testing was reassuring with regard to mucous and presence of motile sperm.\nThe patient had previously undergone 3 cycles of letrozole and timed intercourse at an outside institution. She proceeded to undergo 4 cycles of letrozole and intrauterine insemination in our clinic, which were unsuccessful.\nThe decision was then made to move forward with IVF with embryo freezing after biopsy for aneuploidy screening. For her stimulation protocol, she began with 75 units of human menopausal gonadotropin and 125 units of recombinant follicle stimulating hormone, which was subsequently increased to 300 units. An antagonist was started on stimulation day 8. hCG trigger was administered on stimulation day 11, at which point the patient was found to have 15 follicles, 11 of which were larger than 12 mm. Oocyte retrieval occurred 2 days later where all follicles were aspirated, and 10 oocytes were obtained. Conventional IVF resulted in 4 embryos that were frozen.\nThe patient experienced vaginal bleeding 13 days after oocyte retrieval, which she believed to be a “heavier than usual period” lasting 4 days. She had a positive home pregnancy test about 29 days afterward, however. The patient had not yet undergone embryo transfer. Beta hCG level was 154,224 mIU/mL. Pelvic ultrasound 45 days after oocyte retrieval confirmed an intrauterine pregnancy with positive fetal cardiac activity consistent with 8 weeks 1 day gestation, suggesting conception around the time of oocyte retrieval. The patient and partner recalled having had intercourse 6 days prior to oocyte retrieval – at the cut off of when our program instructs patients to avoid intercourse, i.e., after the seventh day of stimulation. She proceeded to have an uncomplicated vaginal delivery of a healthy female newborn at 39 weeks' gestation weighing 3.09 kg. A case summary is provided in supplementary . |
Case 3. The third patient was 8-year-old boy who presented in the emergency room of our hospital for left ankle pain. Pain was permanent with ankle swelling and limping but without fever. The mobility of the ankle was painful at clinical examination. Radiography was performed and was found normal (). Echography revealed a joint effusion, synovial thickening, and reactive hyperemia of surrounding soft tissue. Diagnosis of inflammatory arthritis was retained but no blood biology was performed. He was first treated with oral NSAIDs (ibuprofen 3 times a day) and nonweight-bearing with crutches.\nFour days later the patient was seen in general pediatric clinic with relative pain improvement but with persistence of skin redness and ankle swelling. The blood biology and urinary sediments did not found any argument of inflammatory pathology. Borrelia, Cytomegalovirus, Epstein-Barr virus, and parvovirusB19 serologies were performed. Only EBV IgG and parvovirusB19 IgG index were found positive. The diagnosis of juvenile arthritis was retained and the treatment with oral ibuprofen was continued.\nHe was seen 10 days later by pediatric rheumatologist without significant symptoms improvement. He also concluded to idiopathic juvenile arthritis. The patient underwent an intra-articular methylprednisolone acetate injection under general anesthesia and two weeks later additional methotrexate was given 10 mg a week with folic acid. No symptomatology improvement occurred.\nConsidering the persistence of symptomatology, a CT-scan and a MRI () were performed and gave the diagnosis of talar neck OO four months after the onset of pain. The nidus size was 8 mm in the dorsal aspect of the talar neck ().\nThe patient had sustained a fracture of both forearm bones treated by elastic stable intramedullary nailing 6 months before. As the surgery of nails removal was already planned, he was operated at the same time by direct open curettage of the OO, under general anesthesia and in one-day surgery. Histopathology confirmed the diagnosis of OO. There was no postoperative complication and the pain disappeared quickly two days after surgery. One month after surgery the mobility of ankle was similar to the contralateral side and limping had disappeared. |
A 17-year-old male patient with neurofibromatosis (NF) type 1 and mental retardation presented with sudden ocular pain, during the ophthalmologic follow-up of neovascular glaucoma and vitreous hemorrhage in the left eye. Ten months before his emergency surgery for enucleation of left eyeball, the crescent-like intraorbital solid content measured approximately 20 mm × 10 mm in the posterior eyeball and generated a low (grayish) signal intensity on T2-weighted magnetic resonance imaging (MRI) (). There were neither apparent signs of optic nerve or surrounding orbital tissue involvement, nor any intracranial tumor lesions (). On gross examination of the surgical specimen, the sagittal cut surface of the left eyeball characteristically displayed an oval, well-demarcated nodular and solid lesion, yellow-whitish in color, measuring approximately 10 mm × 5 mm in diameter and likely floating in the abundant bloody to serous fluid, coexisted with a subsequent cystic component (). A gross connection between its solid tumor part and the optic nerve was not evident (). On its scanning magnification, this intraorbital tumor was found to contain a large volume of eosinophilic serous fluid with a number of red blood cells and showed a well-circumscribed but distorted nodule, uniquely accompanied by frequent, large to small cystic degeneration (). Resection was diagnosed as complete. After the thorough histopathological examination, retina was mostly detached and overtly separated by this intraorbital ancient PA of the optic nerve ( and ). The intraorbital ancient PA strongly compressed the vitreous body, resulting in severe lens luxation ( and ). The schema of each cystic or solid PA component was shown, involving the orbital portion of the nerve, and likely demonstrating a sequential degeneration with possible rupture ().\nMicroscopic findings showed that the solid PA component was noted in the optic nerve and that this solid part arising from the optic nerve uniquely permeated the retina and was protruded from the orbital portion of the nerve into the eyeball (). Foci of arachnoidal hyperplasia around the optic nerve were absent (). Medium-power view revealed that the tumor predominantly comprised a cellular to acellular (biphasic) proliferation of characteristic spindle-shaped, bipolar astrocyte-like cells having mildly enlarged, pleomorphic nuclei and elongated, hair-like processes with very few mitotic figures ( and ), associated frequently with cystic and hyalinized degeneration and eosinophilic fluid production (). Those pilocytic cells appeared as palisading features around cystic spaces or blood vessels (). Rosenthal fibers were rarely seen, but foci of other degenerative changes including calcification or fibrosis were evident. Interestingly, a high-power view detected scattered coagulative tumor necrosis with pseudopalisading features, and glomeruloid microvascular proliferation, reminiscent of high-grade gliomas (). However, there was no apparent infiltrative growth fashion. Immunohistochemically, these neoplastic cells were diffusely and strongly positive for glial fibrillary acidic protein (GFAP, diluted 1:40; Nichirei Bioscience, Tokyo, Japan; ), whereas completely negative for epithelial membrane antigen (EMA, diluted 1:1; Dako Cytomation Co., Glostrup, Denmark), isocitrate dehydrogenase 1 (IDH-1, diluted 1:100; Dianova, Lyngby, Denmark), and p53 (diluted 1:1000; Dako). In addition, the Ki67 (MIB-1, diluted 1:1; Dako) labeling index was much less than 2.2% in the proliferating tumor cells. All of the immunohistochemical stainings were conducted using the Dako Envision kit in accordance with the manufacturer’s instructions. Overall, these features could be diagnosed as optic nerve glioma, named intraorbital ancient PA of the optic nerve. To date, after approximately 7 months of post-operative follow-up, the patient remains well without recurrence. |
We present a case of a 16-year-old boy who while playing cricket on a road was hit by a car from behind; He was standing in knee flexed position approximately 10°, at the time of injury, his leg was fixed to the ground and his knee was pushed anteriorly and leg was fixed at the ground resulting in hyperextension injury at the left knee. He tried to bear weight following trauma but he collapsed.\nThe patient presented in emergency room with extremely swollen knee and soft tissue swelling (hemarthrosis), he was unable to lift his leg actively due to severe pain because of hamstrings spasm, and he had no wound over his left knee and had no other associated injuries.\nOn clinical examination, left knee was in hyperextension attitude, patient had edematous swelling and intra-articular effusion over the left knee with generalized tenderness over the knee, palpation revealed no fracture of the patella or disruption of quadriceps or patellar tendon, any movement at the knee level provoked severe pain so were restricted, patient had palpable distal pulses, and had no sensory or motor loss distal to left knee. Assessment of collateral ligaments, menisci could not be done as the patient was in extreme pain, but it did not reveal any significant abnormalities. Compartment pressures were evaluated clinically which did not reveal any significant increase.\nPlain radiographs were taken which revealed, separated proximal tibial epiphysis (salter harris Type 1 injury) The epiphysis was anteriorly displaced fracture line extending beyond growth plate through metaphysis and tibial tuberosity also displaced anteriorly. The proximal fibular epiphysis fragment was displaced anteriorly with no injury to femoral epiphysis and no patellar fracture, Radiograph also revealed no intra-articular fracture as joint appears congruent ( and ).\nPatient’s left lower limb was immobilized and he was immediately shifted to the operation theatre (4 h interval following trauma).\nUnder spinal anesthesia, under all aseptic precautions traction was applied for few minutes, the fracture was reduced closed as the hamstring spasm gave way, reduction was confirmed under image intensifier in both anteroposterior and lateral planes, and joint congruity was examined. Proximal fibular epiphysis also snapped back in place.\nTwo cross K-wires were inserted percutaneous from lateral and medial side and though mini-open approach from proximal epiphysis to metaphysis. The reduction was found to be stable enough, and proximal fibular epiphysis was also stable, distal pulses were felt post reduction, Above knee posterior and anterior slab was given in 15° knee flexion to avoid popliteal vessel compression ().\nPost-operative patient was immobilized with nil weight bearing for 4 weeks, check X-rays taken, he was mobilized in wheelchair. After 4 weeks slab was removed, K-wires were removed and partial weight bearing was started with rehabilitation for full range of motion of the left knee. At 6 weeks both the knee joints appeared symmetrical with no abnormalities or limb length discrepancy or instability with knee from 0° to 140°, with full weight bearing ( and , ).\nFollow-up at 1 year showed the full range of motion, no joint laxity, and no limb length discrepancy (-). |
An 8-month-old boy was referred to our pediatric urology department with concerns related to the anatomy of his penis. No history of previous penile surgery or trauma was reported. On examination, the glans looked well formed with a normal pediatric foreskin and a normal, wide glandular meatus. A large defect within the midshaft was present. The defect measured 3 × 2 cm and was lined with mucosa of the urethral groove similar to the lining of the urethral plate in patients with hypospadias. Two openings were seen which communicated to the distal and proximal urethra (\n). The patient underwent an examination under general anesthesia that revealed a wide patent distal urethra well covered with spongiosum; the proximal urethra was also normal on cystoscopic examination and did not demonstrate any obstruction nor the presence of any accessory, duplicated urethra. At the age of 11 months, the urethral groove was closed primarily using the Thiersch‒Duplay technique with a continuous 7–0 synthetic absorbable monofilament suture. A second waterproof layer was added using local ventral dartos tissue. The skin was then closed in two layers using 6–0 synthetic absorbable monofilament interrupted sutures. A 6 F feeding tube was used for stenting the repair. Dressing and catheter were removed 7 days postoperatively as per our usual protocol. The patient developed an early superficial skin dehiscence without signs of infection or inflammation. This was managed operatively by excising the margins, mobilizing the skin laterally, and closing it over the tubularized urethra in three layers. At the time of the second procedure, the urethral repair was found intact and did not require intervention. After the second repair a small area of the skin had a further minor dehiscence and was allowed to heal by second intention. From the functional point of view the boy had a successful repair and always demonstrated normal urinary stream. After follow-up of 7 years, the boy is fully continent and voids normally. |
The patient is a 16-year-old adolescent Caucasian male with a past history of diabetes mellitus type 1 (diagnosed at age 10) with history of diabetic ketoacidosis (DKA) with episodic hallucinations, gastroesophageal reflux disease (diagnosed at age 10, s/p fundoplication), cerebral contusion (at age 14) without any history of seizures, and history of occasional cocaine use. At the time of initial psychiatric evaluation, the patient was admitted for DKA with persisting visual hallucinations lasting for more than 48 hours, even after adequate glucose control. His symptoms included hallucinations of hearing screams and voices telling him to harm himself and others. A more detailed description of the patient's symptoms can be found in . The patient's family history included schizophrenia in paternal grandfather and two paternal aunts, substance use disorders in paternal grandfather, depression in mother, and stuttering in father which spontaneously resolved during his adolescence. Initial laboratory workup of the patient revealed no abnormalities other than poor glycemic control. A baseline MRI of the brain showed no acute or chronic intracranial pathology. Thyroid disease and other neurological and systemic conditions that may cause psychosis were ruled out and urine drug screen was also negative. The patient's psychotic symptoms resolved completely with a trial of haloperidol and the patient was discharged with a psychiatry clinic follow-up visit.\nFollowing the initial evaluation, the patient received psychiatric care on an outpatient basis for the ensuing couple of years. The patient's psychotic symptoms continued to worsen; he reported thought broadcasting on television and paranoia about hidden microphones in the walls at home which were there to spy on his thoughts. The patient's visual hallucinations also continued to worsen and he reported seeing his leg being cut off, seeing his brother being stabbed, and becoming violent and homicidal, which resulted in 2 additional psychiatric hospitalizations. He also became withdrawn and isolated, as well as not being able to attend or perform school work. He also developed depressive mood symptoms which ultimately resulted in the diagnosis of schizoaffective disorder. For a detailed timeline of the patient's clinical progression of his psychotic symptoms, see . During those years, until the start of clozapine therapy, the patient's psychiatric medication history included aripiprazole, risperidone, olanzapine, haloperidol, divalproex sodium, benztropine, clonazepam, fluoxetine, and citalopram. The reasons for each change in the medication regimen included poor tolerability, worsening symptoms, or insufficient response at the maximum tolerated dose. For a detailed timeline of the patient's clinical history, see .\nDue to the lack of improvement with other antipsychotics, the patient was started on clozapine. The patient's other medications at the time included lithium, citalopram, clonazepam, and atenolol. During the clinic visit at Day 38 of clozapine therapy, with the dose of 100 mg per day, the patient demonstrated a modest improvement in his visual and auditory hallucinations. He demonstrated no extrapyramidal symptoms and his Abnormal Involuntary Movement Scale (AIMS) score was zero. The patient's dose of clozapine was gradually escalated on an outpatient basis and the progress of the dose escalation is summarized in . At Day 77, with the clozapine dose of 350 mg per day, the patient developed rare infrequent orofacial dyskinesia with perioral twitching. Due to continuing improvement in the patient's symptoms of schizophrenia, the dose was continued to be escalated. However, due to the anxiety regarding potential worsening of the side effects, the patient and the parents requested that the dose escalation be carried out at a slower pace. Then at Day 109, with the clozapine dose of 400 mg per day, the patient developed persistent stuttering. The patient and the mother reported previous anxiety-related intermittent stuttering that occurred approximately once every several months, each with duration of a few minutes. However, his stuttering worsened significantly with clozapine 400 mg per day and the stuttering was now constant and no longer related to his mood. The patient had an especially hard time with pronunciation of letters “I,” “D,” and “T” and was minimizing verbal communication while in public places and increasingly relying on his parents to facilitate his communication. However, the patient demonstrated significant improvement in both positive and negative symptoms, no longer had suicidal ideations, and was socially engaged and able to continue his school work.\nAt Day 134, with continuation of mild intermittent perioral twitching and persistent stuttering, an electroencephalogram was performed. The study showed an abnormal waking and sleep EEG (). Several episodes of generalized spike and wave activity of 3 Hz that lasted 2-3 seconds during photic stimulation and hyperventilation suggested epileptiform activity. Due to the intermittent form of this activity, we will hereafter refer to it as a “microseizure.” In spite of the worsened stutter, clozapine was continued at the same dose as psychotic symptoms and suicidal thoughts were finally well controlled and the patient's functionality returned close to his baseline. Due to the possibility of potentiation of clozapine-induced side effect by lithium, a change of lithium to divalproex sodium was recommended. However, the patient and the parents refused the switch as the patient had not had such an excellent response to treatment since his initial diagnosis. The patient was referred to a speech language pathologist for evaluation, therapy, and support. In subsequent clinic visits, due to the worsening symptoms of stuttering, the patient and the parents decided to proceed with the tapering-off of lithium and the start of divalproex sodium. After 4 weeks of divalproex sodium 500 mg BID, the patient and the family reported improvement in perioral twitching and stuttering. The patient is currently on clozapine and divalproex sodium combination therapy and continues to receive coordinated outpatient based care from a pediatrician, child and adolescent psychiatrist, and neurologist and speech language pathologist. The patient continues to require close monitoring including the management of positive and negative symptoms of schizophrenia, as well as ongoing monitoring for any development of generalized tonic-clonic seizures. The patient has not had any further psychiatric hospitalizations since starting clozapine therapy. |
The patient is a 43-year-old female who developed end stage renal disease following an ANCA positive rapid progressive glomerulonephritis, for which she received a living donor renal transplant six years ago. The donor tissue was negative for Epstein Barr Virus (EBV serologies, while the recipient was positive for EBV IgG Antibodies only. She was managed on chronic immunosuppression with a combination of azathioprine and prednisone.\nThe patient presented initially after an exacerbation of chronic lower back pain following a non-traumatic injury while dancing. Radiographs and MRI of the lumbar spine were performed as part of the initial work-up. Radiographs were negative for acute injury. MRI of her L-spine incompletely captured irregular marrow signal at her left ischium and right sacral ala. This raised the concern for a primary or metastatic marrow replacing process and prompted further imaging work-up. MRI of the pelvis and sacrum performed at this time demonstrated multiple T\n2-hyperintense and enhancing osseous lesions involving the lumbar spine, sacrum, pelvis, and proximal left femur. Several lesions demonstrated aggressive features, including periosteal reaction and large extra osseous soft tissue components (). The multifocal nature of the bone involvement and the presence of lytic and aggressive features raised concern for osseous metastatic disease from a distant primary.\nCT of the chest, abdomen, and pelvis was performed at this time in attempt to locate a primary site of disease. The CT scan again demonstrated multiple bone lesions with areas of marked cortical destruction (). Numerous round pulmonary nodules measuring up to 1.8 cm, as well as a few enlarged axillary lymph nodes, the largest measuring 2.1 cm in the short axis, were also noted. However, there was no conclusive primary site of malignancy. Fluo-D-glucose positron emission tomography revealed markedly fludeoxyglucose (FDG) avid bone lesions with maximum standardized uptake value up to 35.8 (). Additionally, the rounded pulmonary nodules and prominent thoracic lymph nodes were found to be FDG avid as well, with the SUVmax of the nodules and lymph nodes measuring 23.6 and 30.9, respectively ().\nCT guidance was utilized in the biopsy of the large destructive sacral bone lesion. Pathological analysis revealed an EBV negative, diffuse large b-cell lymphoma with a high proliferation rate. confirming the diagnosis of PTLD. The additional thoracic findings were then presumed to be from PTLD given their rounded, mass-like appearance and marked FDG, and therefore biopsy of these lesions was not pursued. |
A 39-year-old male presented to an emergency department with a worsening headache of one-week duration. He was found on CT to have an acute SDH with almost 10 mm of midline shift and was therefore transferred to our level I trauma center. He denied any traumatic head injury when questioned extensively and stated that the only notable event was him forcefully blowing his nose the day his headache started. He denied any past medical history or use of antiplatelets or anticoagulants but admitted to daily tobacco use and frequent methamphetamine use. He denied alcohol use. He denied any other symptoms. He was mildly hypertensive with systolic blood pressure between 140 and 169 mmHg. On physical examination, he was neurologically non-focal without a pronator drift, his Glasgow Coma Scale (GCS) score was 15, and he did not have any findings suggesting trauma. His urine drug screen was positive for methamphetamine. An interval head CT was obtained (Figure ) with the addition of angiography and venography, given the lack of a history of a traumatic event. These were negative for vascular anomalies. A surgical evacuation was offered to the patient, and he provided informed consent for burr holes.\nThe patient was taken to the operating room for SDH evacuation, and two left-sided burr holes were created in a standard fashion. The anterior burr hole revealed hemosiderin-stained dura mater, and upon incision of the dura, thick subdural membranes were encountered. No acute bleeding could be found, and the blood products were copiously irrigated. A subdural drain was placed, and the incisions were closed. The patient was extubated and transferred to the intensive care unit, and a postoperative head CT demonstrated improvement of his midline shift. His subdural drain was removed on postoperative day 1. A final CT performed two days after drain removal (Figure ) showed significant improvement. He remained GCS 15 without neurologic deficit and was discharged on postoperative day 6. The patient’s family members took him to their home in another state, and he was lost to follow-up. |
A 22-year-old male presented to our institution's emergency room with decreased hearing on the right side, ataxia, nausea, and vomiting. On physical examination, an occipital painless subcutaneous mass was noted and audiogram revealed non-serviceable hearing on the right side. A magnetic resonance image (MRI) of brain with contrast demonstrated two separate lesions with similar signal characteristics. A subcutaneous occipital mass measuring 4.8 × 2.9 × 4.1 cm was noted as well as an intradural extraxial 2.6 × 2 × 5.2 cm CP angle mass. There was extension of the CP angle mass into the enlarged jugular foramen and invasion of the internal jugular vein. Both lesions were T1 hyperintense enhancing lesions with a mixed iso and hypointense signal on T2 [Figures and ]. A cerebral angiogram confirmed obstruction of the right sigmoid sinus and jugular vein at the level of the jugular foramen with recanalization distally []. Imaging of the spine by MRI did not reveal other lesions.\nSurgical resection of the two lesions was performed at our institution. The posterior fossa mass was approached through a right translabyrinthine approach and the subcutaneous lesion was resected in the same setting with a separate incision. Electrophysiologic monitoring with cranial nerve monitoring was performed. The extracranial lesion was resected first and did not show any continuity to the intracranial lesion.\nA posterior petrosal translabyrinthine approach was utilized for the intracranial lesion which permitted presigmoid access as well as exposure of the sigmoid sinus and jugular bulb. The dura showed extensive patchy areas of melanocytic coloration over the entirety of the surgical exposure. The intradural lesion was readily visible with invasion of the sigmoid sinus. The sigmoid sinus was opened and found to be occluded with tumor. The sinus was ligated on either side of the tumor invasion and sacrificed. The mass was debulked using a combination of ultrasonic aspiration and suction. The capsule was dissected-off of the lateral cerebellum, brainstem, and cranial nerve 7 despite its adherence. Dissection of the tumor to the lower cranial nerves could not be completely achieved and a subtotal resection was performed. Closure was performed with a dural onlay followed with the use of abdominal fat graft after waxing the surrounding bony structures. A lumbar drain was used postoperatively for 5 days. The patient postoperatively was noted to have nystagmus and a facial nerve function consistent with a House–Brackmann grade 2 which recovered within a month. The patient was discharged home on postoperative day 6. The patient was treated with stereotactic radiosurgery at 6 months (22 Gray in three fractions) and has been stable on MRI imaging for 12 months [].\nGross and microscopic examination of both of the lesions demonstrated similar histomorphological features and immunohistochemical profile confirming the melanocytic origin. The tumor cells were arranged in a nested pattern and showed mild pleomorphism, although rare mitotic figures were noted. Marked intracellular and extracellular melanin deposition was noted []. No evidence of high grade features were noted and both the lesions were classified as melanocytoma with intermediated grade.[] |
A 66-year-old male patient, with a history of smoking (30 pack-years) and no known medical or surgical history, was admitted in our department for a spontaneously resolved inferior ST elevation myocardial infarction (STEMI). The intra-hospital treatment included enoxaparin 0.6 ml twice a day, clopidogrel 75 mg once a day, aspirin 100 mg once a day, bisoprolol 2.5 mg once a day and atorvastatin 40 mg once a day. The coronary angiogram (performed at day 3 through the right radial artery) showed a severe thrombotic lesion of the distal circumflex. The patient underwent an ad-hoc successful angioplasty of the circumflex with a drug eluting (everolimus) stent. Initial laboratory tests at admission were normal except elevated troponin. Echocardiography showed a 65% left ventricular ejection fraction. The patient was discharged after 5 days of anticoagulation by low molecular weight heparin (enoxaparin). Laboratory tests were not controlled during the hospitalization. The discharge treatment included clopidogrel 75 mg once a day, aspirin 100 mg once a day, bisoprolol 2.5 mg once a day and atorvastatin 40 mg once a day.\nOne week later, the patient was referred again to our department for both chest and right lower limb pain. The electrocardiogram showed an inferior STEMI and the physical exam of the right lower limb found ischemic signs with absence of the femoral pulse. There was no history of aspirin or clopidogrel discontinuation. An urgent coronary angiogram (performed through the left femoral artery) showed total thrombosis of the circumflex stent (\n). The patient underwent a successful primary angioplasty of the circumflex by simple balloon (\n). Urgent lower limb contrast-enhanced computed tomography was performed immediately after the angioplasty, revealing total acute thrombosis of the right common femoral artery (\n). The patient underwent an urgent successful thrombectomy with Fogarty catheter. Immediate evolution was favorable with total regression of coronary and right lower limb ischemic signs. Laboratory tests showed a marked fall in the platelet count (68,000/L) which was normal (364,000/L) in the previous hospitalization. A diagnosis of concomitant coronary stent and femoral artery thrombosis due to HIT was strongly suspected (4T score = 8). Our therapeutic strategy was immediate discontinuation of low molecular weight heparin (enoxaparin), aspirin and clopidogrel with strict daily control of platelet count. During this period, no alternative anticoagulation was initiated because of the unavailability of direct thrombin inhibitors in our center. Anticoagulation with a vitamin K antagonist (acenocoumarol 4 mg once a day) and dual antiplatelet therapy with aspirin 100 mg once a day and clopidogrel 75 mg once a day were initiated at day 3 once platelet count had recovered. The in-hospital outcome was favorable and the patient was discharged after 15 days on acenocoumarol 4 mg once a day, aspirin 100 mg once a day and clopidogrel 75 mg once a day. The 3-month follow-up, with controlled blood tests and lower limb contrast-enhanced computed tomography showing total reperfusion of the right femoral artery (\n), was unremarkable. |
A 40 year-old married Pakistani woman came to our out-patient department (OPD) on 19 July 2017 with pain and dryness in her left eye for 7 months, she also complained of progressive loss of vision in her left eye for 6 months. She complained of joint pains and stiffness for the past 2 months and she had pain with blurring of vision and photophobia in her right eye for the past few days.\nShe said that she had severe pain in both eyes with gritty sensation, around 8/10 on Visual Analog of Pain Scale (VAPS); she associated this pain with dryness of eyes and she stated that there had also been pus discharge from her left eye and progressive loss of vision. She visited various physicians in her local area and she was kept on antibiotics for 7 months but she had complete loss of vision by the time she presented to our ward.\nShe also complained of dry mouth, oral ulcers, and there was a history of stillbirth in eighth month of gestational amenorrhea 1 year back. She also complained of pain in all small joints of her hands associated with morning stiffness of 30–45 minutes. She had severe pain around 7/10 on VAPS associated with joint swelling. On examination she was vitally stable and anemic. The examination revealed acute synovitis in the joints of her hands and feet.\nOn examination she had no light perception in her left eye and her right eye was 6/6. A slit lamp examination of the cornea revealed a melted left cornea with red eye and her right eye had inferior peripheral ulceration (Figs. and ). There was scleral thinning in lower temporal quadrant with visible uveal tissue. There was peripheral corneal thinning in lower temporal quadrant.\nOn investigation she was anemic with hemoglobin of 10.2 mg/dl and a mean corpuscular volume (MCV) of 66. She had an erythrocyte sedimentation rate (ESR) of 15. She was found to have a strongly positive RA factor value of 191.9 IU/ml with a positive C-reactive protein (CRP) and anti-citrullinated cyclic polypeptide (CCP) of 32 IU/ml.\nShe was started on methylprednisolone at a dose of 1 g intravenously administered once a day in her right eye for 3 days, methotrexate 10 mg/week, analgesics, proton pump inhibitor, and folic acid supplementation to which she responded really well. The dose of steroid was tapered to 5 mg after 3 months and hydroxychloroquine 400 mg/day was added. She was asked to attend follow-up. |
A 62 year old woman was diagnosed with B-cell CLL manifested as peripheral lymphocytosis only. After 3 years of a watchful waiting approach, treatment was initiated based on peripheral lymphocytosis and constitutional symptoms. FISH cytogenetic testing was performed on peripheral blood and no 17p or 11q abnormalities were identified. She was treated with FCR as standard first-line treatment and achieved clinical remission with normalization of peripheral lymphocytosis and resolution of B symptoms. Within 21 months she progressed, developing cervical and axillary lymphadenopathy and recurrent lymphocytosis, and was subsequently treated with bendamustin and rituxamab. The patient was only able to tolerate an abbreviated number of treatment courses due to pancytopenia and achieved a partial response. The FISH cytogenetic testing was repeated prior to second-line treatment and showed a new clone with an 11q deletion. The patient had no HLA-matched sibling but a 10/10 matched unrelated donor was identified at that time.\nWe recommend repeat testing for high-risk features at the time of each relapse or progression event due to the evolving biology of this disease and the acquired nature of these mutations. New cytogenetic abnormalities are acquired during follow-up in more that 25% of patients over a 5 year interval and are associated with short survival. If a patient acquires poor genetic features during the course of the disease or progresses within 24 months after initial therapy, the indication for alloHCT becomes more relevant compared to the standard choice of second and third line treatment.\nThis patient’s 2- month progression in combination with the acquired cytogenetic abnormality made her a candidate for allo HCT. The use of reduced intensity conditioning has become standard for patients with CLL and decreases transplant-associated toxicities, especially for older patients. The effectiveness of reduced-intensity HCT for the treatment of CLL has been reported in the literature since 2003. Sorror et al. report that 64 patients treated with a non-myeloablative protocol using low dose TBI have a 2-year OS of 60%, DFS of 52% with a TRM 22% and significant GVHD. Using a non-myeloabative FCR conditioning regimen employing early tapering of immunosupression and use of rituxan and DLI for immunomodulation, Khouri et al. estimate a 4-year OS of 48% and a current PFS of 44%. In 46 patients treated using a non-myeloablative regimen of fludarabine and low dose busulfan, Brown et al. report a 2-year OS of 54% and PFS of 34% with 17% TRM and a 2-year cumulative incidence of relapse of 48%.\nOur own data from COH were presented at the Rome Congress, “New drugs and hematopoietic stem cell transplantation in oncohematological diseases of the elderly” as an oral presentation in November of 2009. We presented an analysis of data from 27 CLL patients treated using alloHCT with fludarabine-based reduced intensity conditioning demonstrate overall survivals (OS) and progression-free survivals (PFS) of 80.0% and 72.8% at 1 year and 64.0% and 62.4% at 2 years. The relapse/progression rate was 15.4% and the non-relapse mortality (NRM) was 24.7%, at 2 years. The best response post transplant was complete remission in 19 patients (70.4%), partial response in 4 (14.8%) and stable disease in one (3.7%).\nFor patients such as this man, who do not have HLA-matched siblings, use of a matched unrelated donor (MUD) is a valuable option. Based on extensive registry data from the EBMT, the Center for International Blood and Marrow Transplant Research (CIBMTR) and multivariate analysis of data effecting OS after RIC and myeloablative HCT in patients with CLL, there is no evidence of the inferiority of a well-matched MUD (10/10) versus HLA-matched sibling donors. Therefore, for older CLL patients with good performance status MUD transplant with reduced intensity conditioning would be a reasonable choice with curative intent. |
A 67 years old Caucasian woman gravida 3, para 1 presented with three days history of right flank pain and difficulty in urination. Her medical history was significant for obesity, hypertension and previous salpingectomy due to ectopic pregnancy. 15 years previously a cervical cautery was performed due to cervical ectropion.\nOn admission her vital signs were stable. Physical examination revealed tenderness in both costovertebral angles and fullness in the lower abdomen. In gynecologic exam a huge soft cystic mass was bulging in the vagina to the level of the hymen. It was suspected to be a distended cervix. The external cervical os was not identified.\nComputed tomography of the abdomen revealed a distended urinary bladder with bilateral hydronephrosis and hydroureter. Adjacent to the bladder was a cystic mass of 10 cm in the pelvis that was suspected to be a dilated cervix (figure ). The uterus was extra pelvic on top of the mass and was of normal size with dilated cavity up to 2.5 cm (figure ). No ascites or pelvic lymphadenopathy were noticed. Urethral catheterization drained 2000 ml of clear urine. Blood and chemistry profile were normal.\nAn explorative laparatomy was performed; the uterine cervix was found to be distended with very thin walls and when was incised proved to be a large hematocervix and about 700 ml of old blood was drained. Due to cervical distention and difficulty differentiating the margins of the cervix from the vagina a modified radical hysterectomy was performed. Due to suspected malignancy, surgical staging was completed by omentectomy and lymph node dissection.\nHistological examination of the specimens revealed grade I adenocarcinoma of the uterus located in the fundus with more than two thirds myometrial invasion. There was no lymph-vascular space involvement. The lower uterine segment, cervix and omentum were free of disease. Two of the pelvic lymph nodes were positive for metastases; stage IIIC according to the International Federation of Gynecologists and Obstetricians staging system. Following surgery the patient was treated by pelvic radiation and was doing well one year after surgery with no signs of disease recurrence. |
A 51-year-old man with no significant past medical history was admitted to our hospital because of gait disturbance and dysuria. He had a 2-year history of low back pain and numbness in both legs. His symptoms worsened 1 week earlier and he suddenly developed gait disturbance associated with bilateral leg weakness. On admission, manual muscle testing revealed paresis with distal dominance. Patellar tendon and Achilles tendon reflexes were not exaggerated. He had urinary retention and his lumbar Japanese Orthopedic Association (JOA) score was only 2/29, indicating severe physical impairment (the lower the score, the more severe the impairment). MRI of the spine revealed a spinal lipoma at the L5 level and a flow void dorsal to the spinal cord at approximately the level of the conus medullaris (Fig. ). MRA revealed tortuous blood vessels within the spinal lipoma (Fig. ). Subsequent angiography showed an arteriovenous shunt from the right lateral sacral artery. The feeding artery entered from the dorsal aspect into the lipoma, and then passed the shun zone, and entered the spinal canal primarily via the juxtamedullary vein (Fig. ).\nDiagnosis was a sAVF within a spinal lipoma, and the patient underwent ligation of the draining vein combined with embolization. Under general anesthesia, a catheter was inserted into the lateral sacral artery and advanced to just before the shunt of the fistula. Because a provocation test with lidocaine hydrochloride (hereafter, xylocaine test) showed depression of the motor-evoked potentials (MEP) in the left anterior tibial and anal muscles, embolization using any liquid embolic agent was deemed to be high risk and, we performed only proximal artery occlusion with a coil. Angiography of the internal iliac artery after embolization revealed slight remaining density in the shunt. Therefore, ligation of the draining vein was additionally performed under microscopic view, with transection of two draining veins in the lipoma. Postoperative angiography confirmed there was no remaining shunt.\nPostoperatively, bilateral lower limb muscle strength was slightly improved, but there was no appreciable improvement in vesico–rectal disturbances. At 1 month after surgery, his lumbar JOA score had improved to 10/29, but angiography revealed an arteriovenous shunt via a collateral route from the right lateral sacral artery (Fig. ). The patient experienced no aggravation of symptoms thereafter. Although his low back pain, leg numbness, and bladder dysfunction persisted, intermittent claudication, muscle weakness, and the ADL score were improved at 1 year after surgery. His lumbar JOA score was further improved to 13/29. |
Our patient was a 4-year-old boy who was referred to the urology clinic because of a history of nonprogressive unilateral scrotal enlargement of a few months duration. He had no notable past medical history, and there was no history of genitourinary trauma or urinary symptoms. The child was well-appearing and physical examination revealed a left inguinal hernia. Right testis was palpable in scrotal sac and was normal. Left hemiscrotum was enlarged, and a solid nontender mass was palpable. There was no distinct border between testis and the mass.\nLaboratory investigations including urinalysis, alpha-fetoprotein and human chorionic gonadotropin were normal. Ultrasonography revealed a hernial sac in the left inguinal area (31 mm × 23 mm × 12 mm) filled with bowel. Furthermore, a vascular homogenous hypoechoic mass (27 mm × 18 mm × 12 mm) was found adjacent to the superior aspect of the left testicle (11 mm × 6 mm). Although the tumor markers were negative, however, there was a real risk of a malignant growth, so we decided not to do percutaneous biopsy and proceeded directly to open surgery with a presumptive diagnosis of testicular neoplasm. The patient was explored through a left-sided inguinal incision. Intraoperatively, we found a firm dark blue fleshy mass arising from the left testis and occupying most of testis volume []. There was also a separate smaller mass with the same characteristics located in the processus vaginalis [Figures and ]. As we did not have the facility of frozen section available and our clinical diagnosis was a tumoral growth originating from the testis, complete surgical removal with a safe margin was the best option. Radical orchiectomy was performed because there was hardly any testicular parenchyma to be salvaged; and then, surgery was finished by performing a herniotomy. Pathologic examination of the testicular mass revealed benign splenic tissue attached to a primitive nonfunctional testicular parenchyma []. Furthermore, the mass found in the inguinal canal was also found to be benign splenic parenchyma. Postoperative period was uneventful. The patient has been under follow-up for >1 year with no concerning issues. |
A 30-year-old Caucasian woman consulted for surgical correction of excess brachial skin and fat and breast ptosis. According to the Regnault’s classification, she presented a grade II ptosis when she had her arms along her body. She wanted her breasts to be enlarged but without implants. She had undergone bypass bariatric surgery in 2015 for morbid obesity [body mass index (BMI) 51]. She had no major history other than a malabsorption syndrome following surgery. She was a nonsmoker. The loss of 58 kg had caused significant excess skin and fat. After stabilizing her weight (BMI 29), she underwent surgery to repair her abdominal and back weight loss sequelae 2 years after her bypass in 2017 thanks to a body lift.\nOn clinical examination, there was breast ptosis with a significant loss of projection and bilateral brachial excess skin, which is classic in this post-bariatric context. A major lateral thoracic excess causing significant discomfort was noted. Excess skin and subcutaneous tissue in the lateral chest wall was assessed by means of a pinch test to determine the available width of this donor flap. Considering these findings, we proposed brachioplasty associated with breast augmentation and intramammary burial of the excess tissue in the lateral thoracic region. The patient was informed of the expected higher degree of scarring. She was informed that the scars would extend from the inside of the elbow to the inside edge of the arm (brachioplasty), from the lateral surface of the thorax to the breast base (removal of excess lateral thoracic tissue and mammary burial), and that she would have a long submammary scar (necessary for positioning the flap).\nA maneuver that must be performed to convince both surgeon and patient of the eligibility for true SLT flap or augmentation mammaplasty by superolateral thoracic (SLT) flap is reclination of the lateral thoracic skin in the breast test (Fig. ).\nPreoperative drawing must be performed in a standing position (Fig. ). Under general anesthesia, the patient is positioned supine on the operating table with both arms supported on arm boards abducted to 90°. The first step is classic brachial dermolipectomy. After a skin incision on the brachial drawing, de-epithelialization is performed with an electric scalpel. After hemostasis, closure is performed in two planes with 3/0 resorbable monofilament thread. This first step is carried out on both sides.\nThe second step consists of lateral thoracic skin resection, delimitation, and burial of the thoracic flaps. The shape of the resection must be confirmed by making a frame with staples. This frame allows the upper part of the design to be adapted and to create a continuity with the brachial dermolipectomy design with a cutaneous area preserved in an “S” shape between the two. This prevents a bridle when the arm is raised. The drawing is traced back to the frame, and the modeling staples can then be removed.\nDe-epithelialization of the area is carried out with coagulating forceps. The flap is detached but remains attached to the bank on the breast side (Fig ). During this maneuver, the external mammary vascular network must be preserved (Fig. ). The flap is detached from the thoracic and pectoral planes to about half of the outer side of the breast. This flap is then buried into the breast and sutured deep down with 2/0 resorbable stitches (Fig. ). Sutures are then made to reattach the submammary and external mammary groove, taking in the two edges and the periosteum of the corresponding rib with 2/0 resorbable stitches. This deep mooring is essential for the outcome to be satisfactory. Sutures are made between these pressure points with transparent resorbable 3/0 monofilament thread and an intradermal overlock with the same thread. This second step is carried out in the contralateral thoracic region. Care must be taken to ensure good symmetry at the end of the procedure (Fig. ). No drainage system is required. To ensure better postoperative analgesia, all scars are infiltrated with ropivacaine.\nThe patient was hospitalized for 1 day and was allowed to leave the day after surgery. The postoperative course was uneventful, with no breast skin necrosis, no hematoma, and no seroma or infections. At 15 days, the patient did not have any scarring disorders and she was already satisfied with the result. At 6 months, she no longer had areolar ptosis with an areola above the fold under the breast when she had her arms along the body. Under the inframammary fold, a persistent slight glandular descent was noticeable but allowed to keep a natural aspect, according to the patient. She was very satisfied with the result and reported feeling much more comfortable from a functional and body image point of view (Fig. ). |
A 24-year-old Moroccan man, an athlete, was admitted to the emergency department of the Ibn Sina University Hospital with complaints of relentless pain, discomfort, and inability to move his dominant right hand following a fall onto his right upper limb during a national cycling competition. He had no pathological antecedents and did not present any history of past surgery or trauma. He was unable to recall the exact mechanism of the injury. An initial clinical examination found that he was hemodynamically stable. An examination of his right upper limb showed a significant edema and a clear deformation at the dorsal face of his right hand with no signs of nerve compression. A vascular examination was normal. Radiographs of his hand showed a divergent CMC fracture-dislocation of the last four fingers, as well as associated fractures of the head of the second and the base of the fifth metacarpals. The last three metacarpals had palmar displacement while the second metacarpal was dislocated posteriorly (Fig. ). He was immediately admitted to our operating room and underwent open reduction with dorsal approach under aseptic precautions. Two longitudinal incisions were made in the second and fourth web space addressing adjacent respective joints. CMC joint and fractures were exposed and reduction was visually achieved using external maneuvers (traction in the axis of each finger with pressure on the base of the luxated metacarpal) followed by an axial pinning of the second, third, and fifth CMC joints under fluoroscopic control (Fig. ). Additional plastered immobilization by an intrinsic plus splint was applied for 6 weeks. Our patient underwent physiotherapy and assisted active exercises to increase strength of grip. Pins removal was done at the eighth week; approximately 10 weeks after removal of the cast, he returned to work. Clinical and radiologic examination at the 24-month follow-up visit showed no recurrence of the dislocation or arthritic phenomena. Grip strength and wrist mobility recovered, and there was no pain. |
A 65 year old female patient with a history of rare migraine attacks developed hyperacute severe global headache with accompanying symptoms of confusion and reduced short term memory. The headache was different from her previously known migraine, which had been asymptomatic for the last six years. The symptoms dissolved completely within 90 minutes. 2.5 weeks later she consulted a doctor, and was admitted to the hospital.\nOn neurological examination there were no focal neurological deficits. There were no paresis or numbness in the limbs, and cranial nerve examination and reflexes were normal. Physical examination was also completely normal.\nOn admission an unenhanced Computer tomography (CT) of the head was performed. There were no signs of haemorrhage or other pathology. CT angiography of the intracranial arteries revealed a 2-3 mm aneurysm in the right posterior communicating artery. There was an anatomical variant with fetal origin of the right posterior communicating artery and a hypoplastic P1-segment of the right posterior cerebral artery. This aneurysm was assumed to have caused “warning leak,” indicating an unstable aneurysm in need of treatment.\nThe aneurysm was successfully treated with carotid artery stenting and aneurysm coiling. Postinterventionally, the patient was clinically asymptomatic without any neurological deficits. The patient was treated with Acetylsalicylic acid and Ticagrelor, as Clopidogrel did not show adequate effect by platelet function tests. The patient was discharged to her home with a planned follow-up for Magnetic resonance imaging (MRI) of the brain three and twelve months postinterventionally.\nOne month after EVT, the patient was rehospitalized due to two weeks of right sided headache and a transient episode with flickering in the left visual field. A CT of the head and a CT angiography were performed and revealed small areas of subacute infarction in the right insula, interpreted to be due to a procedure-related thromboembolic event. There was no bleeding, and cerebral arteries were open. Neurological examination was normal. Except for soreness in the neck muscles, the medical examination was normal. The patient's symptoms were thought to be caused by tension headache, and the patient was discharged the day after.\nOne week after hospital dismissal, the patient was again rehospitalized due to persisting headache and a transient episode of anomic aphasia. Additional to a new CT of the head and CT angiography, a CT perfusion of the head and a brain MRI were performed. The examinations revealed no new findings. The patient had a slight persistent aphasia at admission which completely resolved within an hour. Blood samples were normal. The headache was still thought to be caused by tension headache, and the patient was dismissed after four days.\nFive months after EVT, a follow-up brain MRI was performed. On T2-weighted images, new hyperintense white matter signal changes consistent with vasogenic edema in the vascular territory of the right internal carotid artery were seen (). There were approximately 10 lesions located mainly subcortical in the vascular territory of the right internal carotid artery. The lesions had slight mass effect. There was no restricted diffusion. Contrast enhanced MRI was not performed.\nOne year after EVT, a new follow-up MRI was performed, showing only small remaining areas of signal changes (). The patient's neurological examination was still normal, and the headaches decayed about six months after EVT without specific treatment. The clinical and radiological presentation were interpreted to be suggestive of a foreign body reaction after neurointerventional treatment. |
A 39 years old male presented to the emergency department with a history of two episodes of seizure with altered consciousness. He was admitted in ICU with supportive management and relevant investigations were done. Detailed history taken from his father revealed he had a chronic psychiatric illness for last 20 years characterized by aggressive behaviour, muttering to self, fearfulness and decreased sleep. He also had disorganized behaviour in the form of not bathing and washing, drinking dirty water stored for the purpose of toilet use and collecting garbage without apparent reason. He had marked apathy and blunting of emotional responses that resulted in social withdrawal and lowering of social performance. He also had a history of irrelevant talk when trying to hold a conversation. Considering these symptoms, a DSM 5 diagnosis of Schizophrenia was made. Treatment history revealed he was maintaining well on tablet Risperidone 2 mg/day for last three years.\nApart from this, his father also gave a history of increased intake of water for last two years. He would drink around 5-6 liters of water per day that had increased to around 9-10 liters per day in the last few weeks. When asked, he wouldn't justify the amount of water intake but simply answered that he was thirsty and had an urge to drink water. He also had polyuria. On the day of presentation in our tertiary care center, he had two episodes of generalized tonic-clonic seizure. His investigation revealed values as shown in .\nAs per the laboratory reports he appears to meet the criteria for Syndrome of Inappropriate Antidiuretic Hormone Secretion (SIADH) []. However considering the presence of polyuria and no other secondary causes, a provisional diagnosis of psychogenic polydipsia with hyponatremia induced seizure was made and the differential of SIADH was considered. Hyponatremia was corrected over two days. Restriction of water intake was done at the hospital. He was continued on the same dose of Risperidone. Family members and patient were psycho-educated about effects of increased water intake and the need to restrict the same. Behavioral therapy for compulsive water drinking was started with the patient. The father was given a role of a co-therapist. The patient was advised to keep a log diary of intake and output of water. He was advised to decrease his water intake by observing the log. After discharge in a span of two months, two follow-up visits were made. The patient showed remarkable improvement in his water drinking, it reduced from 10 liters approx. to 5 liters/day as seen by input/output charting made by the patient and supervised by his father. The patient was maintaining well in one year follow up. |
A 53 year old Pakistani lady presented to the Medicine clinic of a local hospital in 2004 with a history of heel pain and lower back pain for 5 months. In this period, the patient had sustained a rib fracture and left humeral fracture. There was no history of diabetes, hypertension or any other chronic disease. She had not been on any form of medication, including steroids and traditional drugs widely available and prescribed in the region, prior to the onset of pain. At the time of the fractures, she had been placed on non steroidal anti inflammatory agents, acetaminophen and tramadol. There was no history of illicit drug use and she was a non smoker. Family history was unremarkable, particularly in the context of bone disease, and malignancy.\nInitial laboratory investigations had shown a mildly elevated total calcium level of 10.8 mg/dL {2.7 mmol/L}-(no albumin level result available from that time for correction). Parathormone levels (PTH) had not been determined. There was no vitamin d or renal function report available from that time. X-Ray pelvis revealed lytic lesions in the right iliac bone (Fig. ). A magnetic resonance imaging (MRI) of the lumbosacral spine showed some signal changes. The differentials based on the MRI were metastatic bone disease or multiple myeloma.\nSerum protein electrophoresis was normal. The patient then got lost to follow-up. Her work up was resumed 4 years later when her bone pains had started flaring up. Bone marrow examinations done back in 2007, and later in 2009, were negative for multiple myeloma. A bone scan in November 2009 showed generalized increased tracer uptake over the skull and both the axial and appendicular skeletons- findings in favor of metabolic bone disease (Fig. a). An initial planar parathyroid sestamibi scan requested by a general practitioner in November 2009 was negative for any functioning parathyroid adenoma in the neck or superior mediastinum. No serum PTH report was available from this time either. Following this workup, the patient was treated empirically for bone pains with calcium supplements, an empiric vitamin d injection, and intravenous zoledronic acid 5 mg (without prior bone mineral density assessment via DXA scan). This empiric treatment was instituted by an orthopedic surgeon whom she had been referred to. The patient experienced only a slight improvement in bone pains with this treatment and also developed nausea, vomiting and anorexia. Subsequently, she sought care at the National Institute of Diabetes and Endocrinology, Dow University Health Sciences, Karachi, Pakistan.\nAt presentation, the patient was well oriented and of functional class 3 (wheel chair bound, able to walk only with support). Her blood pressure was 110/70 mmHg. Neck examination revealed no mass or lymphadenopathy. She had a significant proximal myopathy as well as curved thighs. She had shortened fingers, and spinal scoliosis was evident. Severe generalized bone tenderness was elicited. There was no focal deficit. Laboratory investigations at this time showed a calcium level of 15.1 mg/dL{3.775 mmol/L}, (corrected for albumin of 3.6 mg/dL{36 g/L}); Vitamin D3 level of 33.92 ng/mL{84.664 nmol/L}; phosphorus 2.3 mg/dL {0.743 mmol/L}and alkaline phosphatase of 1298 IU/L {21.633 µkat/L}. Her 24 h urine calcium was 155 mg/day {3.875 mmol/day}, with urine calcium to creatinine ratio of 0.02. Her creatinine level was 1.3 mg/dL {114.92 µmol/L}(Table ). The estimated glomerular filtration rate (calculated through Cockcroft-Gault equation) was 50 mL/min {0.835 mL/second).\nFollowing these tests, the patient’s PTH level was ordered and determined to be 2105 pg/mL {2105 ng/L} [Table ]. Ultrasonography of the neck showed a solid hypo echoic, well-circumscribed mass lesion, measuring 1.8 × 1.2 cm at the lower pole of the right lobe of thyroid. There were no calcifications or lymphadenopathy. Appearances were suggestive of parathyroid adenoma. Both lobes of the thyroid appeared normal. A repeat planar sestamibi scan, (requested from a different institute in the city), revealed areas of tracer retention over upper and lower poles of the right lobe of thyroid. The intensity of retained tracer was more over the right inferior parathyroid gland. The findings were highly suggestive of hyperparathyroidism (Fig. ).\nA bone mineral density scan showed a T score of − 2.9 in the spine, − 3.8 in the hip and − 4.5 in the distal forearm, consistent with severe osteoporosis. The Z scores at the spine, hip and distal forearm were − 2.0, − 3.1 and − 3.6, respectively (Table ).\nUltrasonography of the kidneys revealed a single renal stone (0.6 cm) and no neprocalcinosis.\nBased on the biochemistry results of hypercalcemia, associated with elevated PTH levels, a diagnosis of primary hyperparathyroidism was made. Subsequent sestamibi scan and neck imaging facilitated us to localize the abnormal parathyroid gland. The DXA scan was useful for evaluation of the bone mineral density. In view of the phenomenally high levels of parathyroid hormone, (more than 10 times upper limit of normal), the pre-operative suspicion of parathyroid cancer was high [, ]. The patient was rehydrated with intravenous fluids. Subcutaneous calcitonin injections at a dose of 4 units/kg every 12 h were administered to tide her over until the surgery. Once her calcium levels had come down to 10.5 mg/dL {2.625 nmol/L}L, she was operated upon. At surgery, right hemithyroidectomy and inferior parathyroidectomy with level six lymph node resection was done. The lymphadenectomy was performed as there was evidence of enlarged lymph nodes at neck exploration. The size of the lesion was measured as 2.5 × 1.5 × 1 cm. Histopathology showed features consistent with parathyroid cancer (Fig. a–d). Capsular invasion and focal vascular invasion were noted. However, margins of excision were tumor free. The excised lymph nodes did not show evidence of tumour infiltration. The patient was not given external radiation therapy postoperatively. Literature review revealed that post operative adjuvant radiation therapy may only have a role in the management of patients with a histologically positive margin following en bloc resection, or in those with lymph node metastases [, , ].\nPostoperative PTH level, performed on the second day of surgery, was 59 pg/mL {59 ng/L} (16–87). On the third postoperative day, the patient’s serum corrected calcium declined to 6 mg/dL {1.5 mmol/L}. This was associated with paresthesias around her mouth and carpo-pedal spasm. There were no seizures, although there was some confusion in terms of time and place. Intravenous calcium (2 g calcium gluconate, equivalent to 180 mg elemental calcium, in 50 mL 5% dextrose water) was infused over 20 min. Re-monitoring of calcium levels revealed persistent hypocalcemia. A slow infusion of calcium was initiated at an initial rate of 50 mL/h. This was prepared by adding 100 mL of 10% calcium gluconate (equivalent to 900 mg elemental calcium) to 1000 mL 5% dextrose water. The infusion rate was adjusted, with a goal to maintain calcium levels at lower end of normal range. On the fifth post-operative day, the calcium level had risen to 9.0 mg/dL {2.25 nmol/L}. Neurologic examination was normal and she was tolerating oral diet. Oral calcium supplementation was initiated (Qalsan D four times daily-equivalent to 2 g elemental calcium per day). She was discharged on oral calcium and vitamin D supplementation with active vitamin D, (calcitriol) 0.25 µg twice daily, in a stable condition.\nAt follow-up, her appetite and mobility had improved significantly, although she continued to experience bone pains. Corrected calcium was 9.5 mg/dL {2.375 nmol/L}. A repeat skeletal scintigraphy done 3 months after parathyroidectomy did not demonstrate a significant change in the lytic lesions (Fig. a, b). A repeat DXA scan 2 years down the line revealed a significant improvement in bone mineral density at all sites, though more so at the spine and hip, than at the forearm (Table ). Thereafter, we followed her clinically, as she was not keen to have further radiologic testing done. We have been monitoring her calcium and PTH levels on an annual basis. They have remained within their normal range till date (2018). She is now functional class 2, (no longer wheel chair bound), and on regular calcium and vitamin D supplements (patient perspective, attached as Additional file ). |
A 20-year-old male with pain in the mandibular right first molar was referred to the department of conservative dentistry, and endodontics and patient gave a history of pus discharge from the upper front teeth region that was episodic since many years with no occurrence of pain. The tooth 46 had a deep carious lesion which was tender to the clinical tests. On examination of the remaining oral structures, a morphological alteration in the crown structure of maxillary left lateral incisor 22 was detected with no visible decay and color change []. The soft tissue above 22 disclosed a sinus tract opening which on the application of slight digital pressure discharged the purulent exudate. Sinus tracing was done with gutta-percha (GP) cone which was directed distolateral to the lateral incisor root and ended in the periapical lesion present around the apex []. Radiographic examination revealed the presence of apical periodontitis in relation to 46 and dens invaginatus in 22, the palatal invagination that ended at the middle third of the root and presented an immature root apex surrounded by a radiolucent lesion. The condition was diagnosed as a chronic apical abscess secondary to type II dens invaginatus in relation to 22.\nIn the first appointment, 46 was root canal treated and also access cavity preparation was done in 22 under surgical operating microscope (Labo America Inc, USA) which revealed three orifices-mesiolabial (ML), distolabial (DL), and palatal (P) []. The palatal invaginatus canal had an obvious apical stop with no communication with the main canal. Working length was determined for all the canals, files inserted in the ML and DL orifices were found to be crossing each other in the main canal beyond the compressed area by the invagination indicating that it is a continuous communicating channel []. Invaginatus canal was biomechanically prepared and obturated using GP and zinc-oxide root canal sealer by conventional lateral compaction technique [].\nThe patient was posted for surgical management of the dens invaginatus in 22 for the treatment of the main canal with open apex as there was limited accessibility through the small coronal orifices. Under local anesthesia, full thickness mucoperiosteal trapezoidal flap was elevated following which bony defect and a mass of granulation tissue was observed distolateral to 22 running toward the apex []. Curettage was performed and the apex located. Through the coronal orifices, manual biomechanical preparation was accomplished gently, and thorough irrigation was performed with 2% chlorhexidine solution and saline from the apical end followed by irrigation through the orifices which ensured optimal evacuation of the debris.\nAt the apex, the flat end of the warm plastic filling instrument was held by the assistant as an apical stop. Obtura tip (Obtura II, Spartan, USA) was inserted into the ML orifice to down-pack the entire canal with the shooting pressure, and the flow of the obturating material emerged through the DL orifice that ensured a three-dimensional obturation of the main wide canal with complex internal anatomy []. Retro-preparation was done in apical one-third with ultrasonic tip-S12-90ND (Satelec, France) and the root-end cavity was filled with mineral trioxide aggregate (MTA) []. Flap was approximated and sutured. Thorough intraoral betadine irrigation was done on the following day. Sutures were removed 1 week later, and the clinical evaluation revealed signs of healing. Follow-up periods were scheduled at 1 week, 1, 3, 6, months, and 1 year to observe the healing which revealed a reduction in the apical radiolucency [–]. Cone beam computed tomography (CBCT) imaging at 1-year follow-up revealed three-dimensional void free obturation and the complexity of the internal anatomy [–]. |
A 68-year-old lady was brought in by ambulance with a one-day history of bilious vomiting, severe colicky abdominal pain, and profuse watery diarrhoea. She had a background of primary lung adenocarcinoma, for which she had completed neoadjuvant chemotherapy 5 months ago. Surgery was abandoned after repeat staging PET/CT scan revealed sacral metastases. Her other medical comorbidities included thalassaemia, osteoporosis, and previous transient ischaemic attack. Her surgical history included an abdominal hysterectomy which was complicated by rectal injury requiring a Hartmann's procedure that was subsequently reversed. Her regular medications were erlotinib 150 mg daily, Asasantin SR, and alendronate. On examination, she had a low grade tachycardia of 90 bpm and blood pressure of 110/70 mmHg. Her abdomen was grossly distended and peritonitic with a palpable hernia containing bowel in the left lower quadrant. Urgent abdominal CT (computed tomography) was performed, which indicated a severe large bowel obstruction secondary to herniation of transverse colon into an incisional hernia at the previous colostomy reversal site (). The proximal large bowel was dilated up to 54 mm and the caecum was dilated up to 93 mm in maximum diameter (). Distal small bowel loops were also prominent. Given the CT findings of a markedly dilated caecum with clinical features of peritonism the risk of caecal perforation was extremely high. Therefore, an urgent needle decompression of the caecum was performed in the emergency department. Using sagittal and axial CT images, the vertical distance from the anterior superior iliac spine (ASIS) and the anterior abdominal wall skin to the caecum was measured (). At the level of the ASIS, the caecum was measured to be approximately 3 cm from the skin and there were no small bowel loops between it and the abdominal wall (). The patient was positioned in the partial left lateral position to utilise gravity to mobilise the small bowel away from the caecum. The needle entry point was measured and marked on the skin. Under aseptic technique and local anaesthetic (1% lignocaine), a 21-gauge needle (0.51 mm inner diameter) attached to a 5 ml syringe was inserted through the abdominal wall into the caecum. Correct placement was confirmed by the syringe filling with gas and a small amount of feculent fluid. The plunger of the syringe was then removed and the syringe was attached to low wall suction. This produced effective suction of gas and decompression with immediate improvement in pain and patient comfort. The patient remained haemodynamically stable and subsequently underwent laparoscopic adhesiolysis; reduction of the herniating bowel, mesentery, and omentum; and mesh repair of the abdominal wall defect. At laparoscopy, the caecum was decompressed and appeared pink and viable. There was no peritoneal contamination. As a small (21 G) needle was used for decompression, repair of the temporary caecostomy site was not required. The patient was monitored in the intensive care unit for one day postoperatively before being transferred to the ward, where she had an uncomplicated recovery. |
The patient was a 33-year-old Caucasian man without any specific personal or familial history. He was an active smoker without alcoholic intoxication. In 2009, he presented a lesion of the lower lip. He underwent resection of this lesion, and the first pathological analysis revealed a salivary ductal ecstasy.\nOne year later, he developed a peripheral facial paralysis associated with left trigeminal neuralgia. MRI showed perineural invasion of the trigeminal nerve within the temporal fossa (fig. ). Surgical biopsies performed in July 2010 identified secondary lesions from a well-differentiated SCC localized around the V and VII nerve sheaths. According to this diagnosis, a new pathological analysis of the previous resection of the lip was performed in a cancer reference center and diagnosis of SCC of the lip was made. The patient started radio-chemotherapy in September 2010. He received 70 Gy in 35 fractions, and five courses of an association of 5-fluorouracil and cisplatinum. First radiological assessments, 4 months after the end of radio-chemotherapy showed no residual disease.\nHowever, in November 2011, the patient presented with new neurological symptoms: paraparesis and back pain. MRI showed medullary myelitis without meningeal enhancement. Lumbar puncture revealed inflammatory cells without atypical cells within the cerebrospinal fluid (CSF). Cerebral MRI and positron emission tomography scan were normal with no evidence of visceral metastasis. Multiple sclerosis was suspected and the patient received several corticosteroid flashes who led to partial improvement of symptoms. Nevertheless, other explorations were negative, and it was not possible to certify the diagnosis of multiple sclerosis. In early 2012, the patient relapsed and developed paraparesis once again. Sequential lumbar punctures found no abnormal cells. He was given a symptomatic treatment combining corticosteroid and plasma depletion. In March 2012, CSF analysis showed intrathecal immunoglobulin syntheses with oligoclonal immunoglobulin bands. Medullary MRI revealed intramedullary hyperintensity without enhancement after gadolinium injection. The diagnosis of dysimmune myelitis was made and the patient received an immunosuppressive drug (mycophenolate mofetil). It is of note that his neurological symptoms partially improved under this treatment.\nIn August 2012, he subsequently developed progressive back pain, sphincter disorders, and lower limb weakness. A new MRI performed in November showed enhancement of the meningeal nodules and abnormal leptomeningeal contrast enhancement (fig. ). There was no evidence of brain lesions. New lumbar punctures revealed several atypical cells consistent with metastatic squamous cells (fig. ) associated with low glycorrhachia and high proteinorrhachia levels (1.25 g/l). These cells were well-differentiated with nuclear abnormalities consistent with meningeal infiltration by a well-differentiated SCC. Intrathecal immunoglobulin synthesis was no longer seen. Moreover, chest and abdominal CT-scans did not identify other secondary lesions, especially bone metastasis. Nevertheless, a PET scan showed an intramedullary spinal cord metabolism enhancement. All these data were consistent with the diagnosis of CM associated with a carcinomatous myelitis.\nThe patient underwent intrathecal weekly infusions of methotrexate (15 mg) and methylprednisolone combined with systemic treatment based on carboplatin every 3 weeks and weekly cetuximab (250 mg/m2). After two cycles, the disease was clinically stable with no extension of symptoms, the proteinorrhachia level was still high (1.5 g/l), and metastatic cells were still identified in the CSF. However, MRI showed a slight amelioration and cytological analysis revealed only isolated metastatic cells. A new chest and abdominal CT-scan showed no other secondary lesions. As the patient developed a febrile neutropenia, we decided to stop systemic chemotherapy. He received cetuximab 500 mg/m2 and intrathecal methotrexate 15 mg every 2 weeks. After three courses of treatment, clinical and MRI evaluation showed no neurological worsening and a stabilization of the medullar lesions. These observations were confirmed 6 months after treatment start. |
We here report the case of a 56 year old Caucasian female never-smoker, who was initially diagnosed with clinical stage IIIA adenocarcinoma of the lung in 2014. The patient underwent surgical resection of the primary and adjuvant therapy (4 cycles of adjuvant chemotherapy with cisplatin/vinorelbine). Two years after the resection of the tumor, the patient developed distant metastatic disease (Fig. ) and was eventually biopsied at the right iliac bone for pathological evaluation and genetic testing of druggable targets. We employed combined testing for fusions and mutations as described previously [], and detected fusion of EML4 exon 13 with ALK exon 20, which is one of the most common fusion variants in lung adenocarcinomas (variant 1) []. Consistent with this result, we neither detected mutated EGFR or KRAS, or fusions involving ROS or RET. In parallel, IHC staining for ALK was applied. While massive parallel sequencing showed 4163 fusion reads indicating expression of the fusion gene (Fig. ) IHC staining was largely negative except for very few intermingled cells that may show exceedingly faint ALK expression at high magnification, which cannot be reliably subtracted from background signal in routine diagnostics (Fig. , ). Repeated IHC staining for ALK yielded similar results (data not shown) and orthogonal FISH analysis showed impaired hybridization with only very few cells exhibiting probe signals including splits at low intensity (Fig. ). As the current guidelines require that at least 15% of the tumor cells show split (or single red) signals [, ], FISH data of the biopsy specimen were inconclusive and thus non-informative. HE-stained slides suggested crush and thermal artifacts that are known to influence IHC performance. These divergent findings prompted us to perform additional molecular profiling of the primary tumor (Fig. ) that was resected two years earlier. In keeping with the results observed in the biopsy specimen, we detected the very same gene fusion at even higher read counts (9639 fusion reads; Fig. ). In accord with these genetic data, we detected clear-cut split signals in all cancer cells by FISH (Fig. ). Corresponding IHC staining of ALK showed weak ALK expression (Fig. , ). Based on the integrated view of these results, we designated this case as positive for a fusion event involving ALK thus rendering the patient eligible to treatment with crizotinib. |
A 55-year-old woman with large size nasal defect () was referred to the Department of Prosthodontics, Career Post Graduate Institute of Dental Sciences & Hospital, Lucknow, Uttar Pradesh, India, for a possible prosthetic rehabilitation. Since her childhood, she was like this and apparently did not seek any treatment for the same. She used to cover her face with a piece of cloth and had nasal twang in her voice. She was very shy and silent and only after due encouragement and reassurance she gave her consent for treatment.\nHistory revealed that in name of tradition her parents took her for nose piercing to a rural agent where a chemical was applied to her nose and piercing was done. After that she suffered from some type of infection which starts with some pustule and ulcer and slowly it starts dissolving her nasal tissues. There was bad smell and little pain. As narrated by her relatives that to escape the social trauma her parents did not seek medical care and left her to the destiny. This results in slow and gradual disintegration of nasal tissue including bony skeleton also leaving behind large rhinectomy defects.\nOn examination, the patient was completely healthy, nasal site of interest appeared normal with healthy skin and mucosal coverage. There was no inflammation, abnormal discharge, or pain on palpation. The apex, right and left alae, lower half of dorsum of nose, and septal cartilages were absent. The mucosa over the anterior part of nasal floor and the remaining tissue margin of the defect was mobile. The mobility of the upper lip was also noted that could potentially compromise the stability of the prosthesis. The maxillary and mandibular arches were partially edentulous. The maxillary anterior teeth were missing and after implant insertion the removable partial denture was used to replace the missing teeth.\nAfter thorough evaluation implant supported silicone nasal prosthesis with O-ring attachments was planned for rehabilitation. Presurgical planning included plane radiographs (panoramic radiograph (), lateral cephalographs, maxillary occlusal view, and PNS view) of the region and reformatted computed tomogram (CT) for assessment of the bone condition, any pathology and determination of implant size and position. Patient was requested for frontal and profile views of the nose prior to the existing condition but unfortunately only a single frontal photograph was provided as reference.\nAfter facial moulage preparation, special tray with autopolymerising acrylic resin (DPI Self-Cure; Dental Products of India, Ltd.) was made for definitive impression and implant positions were identified. On the basis of clinical and radiographic findings anterior nasal floor (ANF) was selected as site for implant placement.\nPlanning and insertion of implants in the nasal floor is a complicated procedure when the patient has natural teeth in the anterior portion of the maxilla because of the risk of damaging the roots of the natural teeth during the surgical procedure while inserting the implants. When digitally planning the implants according to the technique of Van der Meer et al. [], the implants can be safely inserted in the nasal floor of dentate subjects.\nIn our case, the maxillary anterior teeth were missing so the level of complication was little less comparatively. We used various radiographs and Cone Beam Computer Tomography to determine the bone level and angulation of the premaxillary area, associated anatomical structures, and placed implants. It has been suggested that dental fixtures may be placed in the alpha sites to retain nasofacial prostheses. Alpha sites are 6 mm or greater in axial bone volume. The most common areas of the facial skeleton having this volume of bone are the anterior maxilla through the nasal fossa (floor of the nose) and the zygoma [].\nInitially two-stage implant placement was decided, following the techniques described by Lundgren et al. [], Stanley and Olsen [], and Parel and Tjellstrom []. But later, to avoid stage II surgery, it was decided to place the healing caps in place of cover screw, over the implants in stage I itself. Two conventional implants of 3.3 mm diameter and 10 mm length (Adin Dental Implant Systems Ltd.) were placed in the ANF parallel to palate. Surgical templates were used to assist in positioning the implants. Implants were allowed to heal for 6 months. After healing period a soft tissue with thickness of 2–4 mm and width of 2-3 mm was developed at the implant site. Now the healing caps were removed and the selected O-ring posts were screwed over the implants ().\nUtilizing special tray, definitive impression of the defect site was made using light body polysulfide impression (Aquasil Ultra Lv, Dentsply, Germany) material. The impression was boxed and poured in type IV gypsum product (Kalabhai Karson, Mumbai, India). The resulting master cast was sprayed with an oil free release agent (Silicone Spray, Dentsply Caulk) to facilitate the removal of wax pattern and prevent the wax from soaking into the gypsum cast. The prosthesis was sculpted in wax with the esthetic contours developed. Remaining anatomical landmarks and previous photograph provided by patient were used as reference.\nFor fabrication of substructure, mold was made by CAD/CAM. Patient's nasal defect area was scanned by CT to obtain the geometric data (Figures and ).\nThese data were further processed by a CAD system to generate both positive and negative replicas of the normal substructure for nose, and the resultant data were input into a CAM system (Spectrum 510, Z Corporation) for the fabrication of the resin models. This substructure was designed to fit within the confines of the nasal prosthesis and to hold the metal encapsulators of the O-ring attachments at a further stage. Following this try-on of wax pattern () and acrylic substructure try-in were done and verified for shape, extension, air way, and esthetics. Patient's approval and suggestions were taken into consideration.\nOn completion of try-on step, the wax sculpture was sealed into place on the master cast. Dental plaster was injected into the areas of the nares to maintain the air way passage and the entire wax pattern was invested. After the wax boil-out procedure, the acrylic substructure was positioned on the master cast ().\nThe medical grade room temperature vulcanizing silicone (Dow Corning Corp, Midland, Michigan) was mixed and packed in the mold after evaluating color from the patient. Layer wise packing was done to produce a multilayered intrinsically colored prosthesis. After packing, the mold was clamped under pressure at room temperature for 24 hours. Following complete curing, the prosthesis was retrieved from the flask and trimmed and finished carefully with the help of scissors and silicone finishing burs. Limited flash was preserved around the borders of the nasal prosthesis to facilitate transition of the borders to the adjacent tissue. The definitive prosthesis was checked critically for the silicone tags, color, shape, and incorporation of acrylic substructure and its exposure. To achieve the bonding between polyamide substructure and the silicone both chemical and mechanical methods were used, to be sure that debonding between two would not take place very early. The substructure was made hollow (as seen in and care was taken to maintain the space for metal encapsulator during packing of silicone) so that the silicone engages it from all around and adhesives were also applied on its surface to bond with silicone.\nThe silicone material had filled the space for the metal encapsulators of O-ring attachment, so it was cut off from the area and acrylic was roughened. Now on the O-ring posts over implants, the O-ring with 30–40 hardness in a Shore A scale was seated along with the corresponding metal encapsulator. The previously made polyamide acrylic substructure which was incorporated in the nasal prosthesis was repositioned over the implants in its accurate position. The prosthesis was removed and evaluated for the incorporation of metal encapsulator with O-ring in acrylic resin substructure ().\nNow the completed nasal prosthesis was delivered to the patient (). The patient was instructed for prosthesis placement, removal, and application of the medical grade skin adhesive (Pros-Aide; ADM Tronics Inc., Northvale, NJ) at the borders. Home care and maintenance included mechanical debridement with a soft toothbrush and proxy brush, irrigation with warn saline water and soap, and/or 2% hydrogen peroxide. The color match was satisfactory and appreciated by patient and her husband. Elevation in the spirit of patient was seen in her eyes and happiness of her husband could be judged by the action that he bought a new nose ring to his wife. Patient was observed regularly for 6 months which was found satisfactory and verified by radiograph (). |
A 13-year-old female of the Marshallese origin presented to the emergency department with complaints of nausea; nonbloody, nonbilious vomiting; and abdominal pain []. The patient denied any recent fevers, and no rash was reported. Review of systems was notable for the left ear drainage. Immunization status was unknown, and she was not reported to have been previously treated for any significant illness, though the history was limited by the patient's custodial circumstances. She lived in a small house in rural North Carolina with 21 other Marshallese immigrants and was cared for by relatives who had assumed care for her at the time of her immigration five years before.\nExamination during the initial hospital visit revealed dehydration and acute otitis media with rupture of the tympanic membrane. Laboratory results revealed leukocytosis, prerenal azotemia, elevated liver enzymes, and mildly elevated lipase (). Computed tomography (CT) showed scattered focal pulmonary infiltrates, splenomegaly, and a markedly distended stomach without an obvious focus of mechanical obstruction (). Cytomegalovirus and Epstein–Barr virus serology were requested with results suggesting prior exposure. She was admitted with a presumptive diagnosis of gastroparesis secondary to a nonspecific viral infection and possible mild pancreatitis. An NG tube was placed resulting in high volume output. Her symptoms gradually improved over several days with IV fluid support and bowel rest; her NG was successfully removed, and she was discharged home tolerating a regular diet.\nTwo days after discharge, the patient returned with recurrence of her prior symptoms. The patient appeared acutely ill with dehydration. On lung exam, scattered crackles were noted. She was also noted to have diffuse small nodular lesions most apparent on her hands, feet, lower legs, and face (). An advocate placed with the family reported her concern for additional symptoms of chronic weight loss and productive cough with posttussive emesis and reported the skin changes to have been present for months. Prior exposure to or testing for tuberculosis was unknown. The patient did not report symptoms of peripheral neuropathy.\nLaboratory results revealed a relatively increased white blood cell count (21.4 × 109 per liter) with significant worsening of her renal function (creatinine of 2.45 mg/dL which had previously been normalized with rehydration) and persistent mild elevation in liver enzymes and lipase. Respiratory viral screening was positive for rhino/enterovirus. Repeat CT imaging showed persistent gastric dilation and splenomegaly ().\nThe patient was placed on empiric antibiotics for community-acquired pneumonia and put on reverse isolation with concern for tuberculosis. Skin biopsies of multiple lesions were obtained and sent for pathology and culture (). Because multiple attempts to place an NG tube failed and vomiting failed to respond to conservative measures, endogastroduodenoscopy was pursued. The scope passed into the stomach and through the pylorus easily, reaching a normal-appearing proximal duodenum. However, a feeding tube could not be passed beyond the pylorus and was left just proximal to the pylorus for decompression. Stomach mucosa was described as having a “cobblestone” appearance in places, with one area of ulceration possibly due to prior nasogastric tube. Stomach biopsies revealed Helicobacter pylori and chronic active gastritis, but were negative for AFB.\nDue to persistent evidence of gastric outlet obstruction, an upper gastrointestinal study was performed which suggested gastric volvulus. The patient proceeded to gastropexy via open gastrostomy tube placement. The surgeons reported an extremely large and patulous stomach that had twisted mesenteroaxially. Gastric aspirates were negative for AFB.\nBiopsies of the skin lesions confirmed the presence of many AFB with changes consistent with lepromatous leprosy due to M. leprae (). Additionally, the serial sputum samples were positive for AFB, and culture of the skin sample for AFB showed evidence of growth within a few days, suggesting Mycobacterium tuberculosis (MTB) pulmonary infection and possibly disseminated MTB given selectivity of the culture medium.\nIn coordination with the health department and the infectious disease service, the patient was initiated on treatment for presumed disseminated MTB, noting that this would also provide coverage for M. leprae. However, polymerase chain reaction (PCR) testing from the sputum samples and skin samples failed to demonstrate MTB, while PCR testing performed at the Hansen's Disease Center of skin samples was positive for M. leprae. Retrospectively, it was felt that growth detected on the AFB culture was due to metabolism by an unusually large inoculum.\nGradually, the patient's oral intake improved. With hydration, her renal function again returned to normal. After several weeks of inpatient treatment, the patient discharged to complete therapy through the health department. Symptoms of gastric outlet obstruction have not returned. |
A 21-year-old Turkish man was admitted to our clinic for right knee pain, clicking and popping sensation in the affected knee for three months prior to his presentation. Difficulties in climbing stairs and disability during sports activities were the other symptoms of our patient. He reported no previous physical or surgical treatment for any other knee pathology and did not have any known history of trauma. On our patient's physical examination, atrophy of the quadriceps muscle was observed. Medial patellar apprehension test was positive and increased passive medial patellar mobility was observed when his patella was stressed medially. His gravity subluxation test was negative. His physical examination also revealed that medial subluxation of his right patella was more prominent in extension while weight bearing (Figures and ). We also noted patellofemoral hypermobility. He had no complaints related with his left knee. Conventional anterior to posterior, lateral and Merchant radiographs of our patient did not reveal any abnormality.\nOur patient was initially treated with physical therapy. A quadriceps strengthening rehabilitation program and neuromuscular stimulation focused on the vastus lateralis muscle and was continued for three months. After three months of rehabilitation, some improvement was achieved in his quadriceps strength and the medial translation of his patella was less than the pre-treatment level. However, he still suffered from right knee pain, which disturbed his gait pattern. As such, surgery was planned.\nUsing a lateral parapatellar approach, we first performed direct lateral retinacular imbrication. We evaluated provocative patellofemoral tracking during flexion and extension of the knee by pressing on the inferior lateral pole of his patella. The achieved patellar stability was not sufficient and his patella was still moving medially more than 50% of its width. We dissected a strip of his iliotibial band approximately 1 cm wide and 4 cm in length, thus leaving distal base of the strip attached to Gerdy's tubercle. Using this strip, we augmented the patellatibial ligament. As the medial subluxation of his patella was more prominent in extension, we tensioned the strip in extension.\nPost-operatively our patient's knee was immobilized using a brace for six weeks. He was allowed to walk with partial weight bearing after the first post-operative day until the fourth post-operative week. During the first week he was allowed to perform passive knee extension and active assistive flexion exercises within a range of 0 to 90°. Full active range of motion exercises and full weight bearing was allowed after the third post-operative week. Quadriceps strengthening physical therapy focused on the vastus lateralis with neuromuscular stimulator was continued for three months after the operation. A home exercise program was also given to our patient.\nHe was examined weekly for the first month. Follow-up visits were made by phone interview monthly and by clinical examination at intervals of three months. By the end of the sixth week of the surgery he achieved full range of motion. Meanwhile, full quadriceps strength was achieved at the end of the third post-operative month. Our patient did not experience any patellofemoral instability during his follow up examinations.\nDuring his last visit for follow-up examination, atrophy of his quadriceps muscle was completely resolved and both medial patellar apprehension and passive medial patellar mobility tests of our patient were negative. Gravity subluxation test was also negative. He was evaluated subsequently according to the clinical score and pain scale defined by Hughston et al. [] (Table ). Our patient's pre-operative functional level limited performance of his daily activities. At the end of the first year, however, he was categorized as vigorous recreational. While he used to have severe pain, he described mild pain with competitive sports in the post-operative period and no pain with daily activities. |
Our patient is a 67-year-old white man, who works as a teacher in a small city. He does not smoke tobacco; he has a history of arterial hypertension grade I to II and coronary heart disease. His mother died from myocardial infarction due to occlusive coronary artery disease. He was diagnosed as having CML in 2006. The treatment was initially started with imatinib (Glivec, Novartis) 400 mg administered orally once daily. Imatinib 400 to 800 mg per day was taken for 53 months. He lost major molecular response (MMR) and imatinib therapy was replaced with dasatinib 100 mg orally administered once daily and after 6 months MMR was achieved. He was also using torasemide 20 mg orally administered once daily and metoprolol succinate 50 mg orally administered once daily. In April 2015 he developed increasing dyspnea on exertion, fatigue, and peripheral edema. He consulted his family physician, and a chest X-ray was done, confirming pleural effusion. On admission his heart rate was 97 beats per minute and blood pressure was 143/90 mmHg. Fever was not present. He presented with peripheral edema and diminished breath sounds. Pleural friction rub was present. Deformity of the spine accompanied by lower back pain was noted during neurological check-up. The pleural fluid was drawn out several times via thoracentesis (1.5 to 2 liters of exudate in total) but cytological analysis excluded malignancy, a GeneXpert® tuberculosis test of a bronchial smear was also negative, therefore, the pleural effusion was suspected to be caused by congestive heart failure. A complete blood count was normal, but his creatinine levels were elevated (Tables and ). Over the course of the next 4 months his general condition deteriorated as he experienced multiple recurrences of pleural effusion requiring drainage of the built-up fluid (Table ). Dasatinib therapy was stopped in September 2015 after 42 months of treatment. A coronarography was done in September 2015; it did not reveal any hemodynamically important stenosis in his coronary arteries, thereby excluding coronary artery disease. An echocardiogram showed right ventricular dilation, estimated right ventricle systolic pressure of 125 mmHg, and severe tricuspid regurgitation suggesting PH.\nRHC performed on 12 October 2015 revealed severe PAH with mPAP of 53 mmHg and normal left ventricle diastolic pressure (Table ). A computed tomography scan confirmed the absence of pulmonary embolism; laboratory specific tests for HIV, rheumatoid factor, and anti-nuclear antibodies (ANA) were negative and dasatinib-induced PAH was diagnosed. The 6-minute walk test (6MWT) distance was limited to 165 m (Table ). He started PAH-targeted treatment with sildenafil 20 mg × 3 orally administered and restarted CML therapy with imatinib 400 mg orally administered daily on 19 October 2015.\nHis condition rapidly improved, a check-up RHC done a month later showed mPAP of 34 mmHg, decreased pulmonary vascular resistance, and increased cardiac output values (Table ). His 6MWT score was 2.1 times higher (Table ). Echocardiography done in February 2016 revealed right ventricle systolic pressure of 50 mmHg; a complete blood count and biochemistry showed no abnormalities (Tables and ). He has been asymptomatic since, but treatment for PAH with sildenafil is still necessary. The last hematological check-up was in January 2017, he was still on imatinib 400 mg daily and had normal complete blood count and MMR (BRA-ABL was negative). |
The first patient was a 24-year-old African American female with past medical history of congenital Human Immunodeficiency Virus (HIV) infection, compliant with antiretroviral therapy (ART) with a CD4 count of 275, recently diagnosed endometriosis, and childhood asthma. She was admitted to the hospital for an elective dilatation and curettage procedure. She was seen postprocedure by pulmonary medicine for acute onset of shortness of breath. She was noted to be in mild respiratory distress with oxygen saturation of 96% on 4 liters of oxygen via nasal cannula but, otherwise, had a normal exam. She improved after receiving nebulized albuterol. Upon further questioning, the patient revealed that she was diagnosed with asthma as a child. She was treated with various inhaled and nebulized medications throughout her childhood. During early adolescence, she required home oxygen for about 1-2 years. She had multiple hospitalizations for presumed asthma exacerbation during her childhood and teenage years. She did not recall having a pulmonary function test or any imaging of the chest. She had never been placed on noninvasive or invasive mechanical ventilation. Over the last 5-6 years, she had no hospitalizations for shortness of breath. She did not use oxygen at home. She had a desk job as a receptionist in a doctor's office so was able to perform her duties without experiencing any respiratory distress. However, she did get short of breath after walking 3 blocks and after climbing 1 flight of stairs. She used an albuterol inhaler which she said brought only mild relief of her symptoms. She had never smoked cigarettes or any other illicit drugs. However, she was exposed to second-hand smoke as her grandmother and several of her friends smoked in her presence.\nHer hemoglobin on presentation was 8.9 g/dL, stable as compared to previous levels. She had a normal white blood cell, platelet count, and renal function. A liver function test was normal except for a low albumin level of 3.0 g/dL. Her lactic acid level was normal. An arterial blood gas showed pH of 7.38, pCO2 of 37 mmHg, and pO2 of 70 mmHg on room air. Her pulmonary function test showed FEV1 of 0.9 liters (33% of predicted), FVC of 1.9 liters (61% of predicted), and diffusion capacity of 8% predicted. A CT () showed severe bilateral cystic changes involving peribronchial thickening and cylindrical bronchiectasis at the bases. There was no evidence of pulmonary emboli. There were no masses or enlarged lymph nodes.\nThe patient's evaluation for cystic lung disease included a negative folliculin gene test, ruling out Birt-Hogg-Dubé syndrome, and a normal serum vascular endothelial growth factor-D (VEGF-D) level arguing against lymphangioleiomyomatosis. Her autoimmune testing was negative as well.\nShe then underwent right-sided video-assisted thoracoscopic surgery with right upper, middle, and lower lobe wedge resections and biopsy. Tissue samples were negative for fungal growth and tuberculosis. Pathologic examination of the lung revealed marked follicular bronchiolitis and centrilobular and subpleural cystic changes. There was no evidence of a malignant lymphoproliferative disorder (Figures and ).\nShe was started on 60 mg of prednisone daily. However, after two months of treatment, she had no improvement in respiratory symptoms and began to develop complications from steroid use such as weight gain, myalgias, and hyperglycemia. The prednisone was tapered slowly, and a trial of azithromycin was started; however, she failed to respond to that as well. She was still able to perform her daily activities without the need for supplemental oxygen. She was referred to the lung transplantation clinic for further evaluation. |
A 68-year-old male patient was referred by the public health service of his city for specialized care to the Oral and Maxillofacial Surgery Service of the Federal Hospital of Rio de Janeiro State Servers/Brazil for the evaluation of volumetric increase on the right side of the face. Medical history has reported systemic hypertension and gastritis, both conditions under drug control. He also reported having had surgery for an appendectomy at 25 years of age and denied allergies. The patient reported frequent use of alcohol and smoking for 35 years and having stopped smoking 4 years ago. The patient reported a progressive and painless growth of lesion in the genian region in the last four months.\nExtraoral examination revealed a tumor-like lesion, firm consistency, well-defined contours, and significant expansion near the parotid region (). On intraoral examination, a slight elevation in the ipsilateral jugal mucosa was noted, corresponding to the expansion of the medial growth tumor ().\nFor an incisional biopsy, computed tomography (CT) images of the face and preoperative laboratory tests were requested. The results showed normal serum levels of most of the requested blood fractions, except for slightly elevated urea and creatinine. The axial CT image () revealed an expansive lesion (approximately 6.0 × 6.0 cm) in the soft tissue of the mixed aspect and regular contours. In the coronal CT image, hyperdense foci of the lesion were observed (), suggestive of calcification or bone lysis. In the three-dimensional reconstruction of the tomography (), bone destruction in the mandibular branch was evidenced by possible tumor compression.\nAn intraoral incisional biopsy was performed. In the first 72 hours after the biopsy, the patient developed significant local edema and volumetric expansion of the lesion, which became inaccurate (). On gross examination, three fragments of smooth brown tissue were observed (), which were sent for histopathological analysis. After anamnesis and clinical and radiological examinations, our diagnostic hypotheses were Warthin's tumor, pleomorphic adenoma, mucoepidermoid carcinoma, and soft tissue sarcoma.\nMicroscopically, large neoplastic cells often eosinophilic and pale, sometimes clear, with reticulated cytoplasm, centralized oval nucleus and perinuclear halo, and prominent nucleoli were observed (). The diagnosis was strongly suggestive of oral metastasis of chromophobe renal cell carcinoma.\nThe patient underwent primary CT scan of the abdomen, which showed the presence of lesion involving the right kidney cortex and parenchyma (). The patient was referred to the Referral Oncology Service, where renal cell carcinoma was effectively diagnosed, and then began treatment with chemotherapy for kidney tumor and radiotherapy for orofacial metastatic tumor. The patient died four months after starting treatment. |
A 44-year-old woman visited our out-patient clinic with dizziness in December 2013. She had previously been diagnosed with a 5 cm phyllodes tumor in her right breast and underwent right lumpectomy with axillary lymph node dissection in August 2008 (), which was followed by radiotherapy. In July 2011, she underwent right total mastectomy because of local recurrence of a 2.5 cm phyllodes tumor in the treated breast. In August 2013, at 5 years after the initial diagnosis, chest computed tomography (CT) revealed a 4.7 cm lung mass. Right lower lobectomy was performed, and the pathology results revealed a metastatic phyllodes tumor. As the risk of another recurrence was considered high, adjuvant chemotherapy was recommended, but the patient refused to undergo this treatment. Four months later, multiple liver nodules were found upon chest CT, and a liver biopsy revealed metastatic phyllodes tumor cells identical to those from the primary breast phyllodes tumor. After confirming the liver metastasis, we recommended palliative chemotherapy using an ifosfamide-containing regimen to the patient, but she refused this treatment and selected only palliative symptomatic care.\nWhile receiving the symptomatic care, she visited our outpatient clinic because of dizziness and was admitted for further evaluation. Blood tests revealed severe anemia (hemoglobin levels of 6.7 g/dL), and we subsequently performed esophagogastroduodenoscopic evaluation, which identified a large gastric mass (approximately 7 cm in diameter) with active bleeding and several related masses (). Therefore, based on an initial impression of multiple metastatic gastric tumors, we performed endoscopic hemostasis with cauterization and biopsy. The biopsy confirmed that the tumors were metastases from the breast phyllodes tumor (). Two days later, we performed endoscopic hemostasis again for re-bleeding at the site of the gastric metastases. After the bleeding had been stopped, we considered total gastrectomy for complete bleeding control. However, we judged the patient as having a very high perioperative risk based on the rapid progression of multiple metastases involving the liver and her poor general condition. Therefore, we selected treatment using a proton pump inhibitor instead of total gastrectomy. We did not observe any evidence of re-bleeding for > 1 week; thus, she was discharged.\nApproximately 1 month after discharge, she visited our emergency room for massive melena. Esophagogastroduodenoscopic evaluation confirmed that there was active bleeding in the bed of the gastric metastasis, where we had performed the earlier hemostasis. Thus, we re-performed endoscopic hemostasis and resumed treatment with the proton pump inhibitor. The patient did not experience any other bleeding events, although she ultimately died on April 2, 2014 because of irreversible liver failure related to rapid progression of the liver metastasis. |
A 21 years old male, presented with pain and recurrent nodular swelling over right foot for last four years. He was farmer by occupation. Following the onset of painful swellings, after two years, there was history of a sinus from the foot discharging pus with dark coloured granules. There was history of close contact with an active case of pulmonary tuberculosis (wife). Following the onset of the discharge, the patient visited a local practitioner who sent the pus for culture and sensitivity. The patient was started on oral antibiotics on the basis of sensitivity. The discharge mildly decreased on oral antibiotics but persisted and the patient was advised local dressing. Due to the persistent discharging sinus, patient finally presented to the outpatient department of our hospital.\nOn local examination, multiple nodular swellings were seen over dorsum, medial and lateral aspects of the mid foot. There was a discharging sinus over the dorsum of the midfoot. The sinus was adherent to the bone and the margins were not undermined. There was diffuse tenderness on the mid and forefoot and the movements of the mid and forefoot were painful and restricted.\nBlood investigations revealed marginally high ESR (32) and raised C-reactive protein levels (20ng/dl). Plain radiographs of the foot showed multiple lytic lesions involving the metatarso-cuneiform joints and inter-tarsal joints () and were associated with gross osteoporosis. On the basis of the history and examination, number of diagnostic possibilities arose. Since the patient was a farmer by occupation, the possibility of foreign body prick (like thorn) was considered. Due to the endemic nature and history of close contact with a case of tuberculosis, pedal tuberculosis was also kept as a differential. The other differential diagnoses included rheumatoid, neuropathic and neoplastic involvement of the foot. To rule out the possible differentials and reach the final diagnoses, incision biopsy was planned.\nThe incisional biopsy was performed from the dorsal aspect of mid foot along with sinus tract excision. The incision revealed pus which was interspersed with dark black coloured granules on gross examination. A big seqeustrum was removed from the tarso-metatarsal junction, with multiple black pigmented granules ().\nThe histo-pathological examination of the tissue revealed colonies comprising of dense tangled meshwork of slender hyphae interspersed with ovoid to round spore like structures. The colonies had brown pigmentation at the periphery (). The fungal culture did not grow any organisms.\nOn the basis of the histo-pathology findings, the patient was started on oral Itraconazole 200 mg twice a day and Terbinafine 250 mg twice a day for a period of 12 months.\nOn follow up, the sinus on the foot healed and there was significant improvement in pain. The radiographs did not show any improvement in the lytic lesion in the initial follow ups and the only sign of recovery was the improvement in osteoporosis. The sequential radiographs of the foot revealed resolution in the lytic lesions in the foot as well (Fig 5). The pain improved dramatically. The treatment was finally discontinued after the resolution of the lytic lesions. |
We report a case of a 60-year-old Sinhalese man from a rural area of central Sri Lanka who presented to a teaching hospital with on-and-off fever of 4 months’ duration and pain in the lateral aspect of the right upper thigh of 2 months’ duration. He had been admitted to a local hospital 3 weeks prior to admission to our hospital with a history of nearly 3 months of on-and-off fever and a painful lump at the lateral aspect of the thigh. He had been treated for multilobar pneumonia at the local health facility with intravenous meropenem and teicoplanin for 14 days, and he required intensive care unit management during the course of his illness. Although his pain and the size of the lump at the lateral aspect of the thigh decreased with treatment, the lump and pain over the area persisted even after he was discharged from the local hospital. Further, he experienced on-and-off fever again, though he was afebrile upon discharge from the local hospital. With time, he experienced difficulty in walking with increasing severity of pain in the lateral aspect of the right upper thigh, for which he sought medical advice. On admission to our hospital, he had high-grade fever, and he denied any respiratory or urinary symptoms. His bowel motions were normal with normal color and contour. He was a farmer and had a history of exposure to surface water. He was a nonsmoker and never used alcohol. He was diagnosed for the past 8 years with type 2 diabetes mellitus, which was treated with oral hypoglycemic drugs. He was not receiving any medication other than oral hypoglycemic drugs, and he denied recent use of steroids. He did not have any high-risk behaviors.\nOn examination, he was febrile (102 °F) and mildly pale but not icteric. He did not have enlarged peripheral lymph nodes or skin rashes. He had a small induration over the lateral aspect of his right upper thigh, which was tender. On further examination, lumpiness was detected deep in the indurated area, measuring 0.5 cm × 0.5 cm, which was firm and tender. Further, there was surrounding muscle tenderness. He did not have evidence of thrombophlebitis. He was hemodynamically stable, and the results of his respiratory and cardiovascular examinations were perfectly normal. His abdomen was soft, and he did not have hepatosplenomegaly. The result of his neurological examination, including bilateral fundi, was normal.\nHis initial full blood count showed a white blood cell count of 15,500/mm3 with 80% neutrophils. His blood picture showed normochromic, normocytic anemia (hemoglobin 8.0 g/dl) with neutrophil leukocytosis, suggesting anemia of chronic disease but without any evidence of bone marrow infiltration. His initial C-reactive protein (CRP) level and erythrocyte sedimentation rate (ESR) were 170 mg/L and 70 mm in the first hour, respectively, and he had an elevated serum creatine kinase level. His liver enzymes were marginally elevated with alanine transaminase of 111 U/L and aspartate transaminase of 87 U/L, but his liver and renal function were normal, and his hemoglobin A1c was 7%. His antibodies for human immunodeficiency virus types 1 and 2 were negative. The findings of his chest x-ray, 2D echocardiogram, and ultrasound scan of the abdomen were normal. He was started on intravenous flucloxacillin and meropenem after blood cultures and serum were taken for melioidosis antibodies. Ultrasound scan of the right lateral thigh showed a lesion that extended up to the right-side femur, but an x-ray of the right femur was normal. Contrast-enhanced computed tomography of the right thigh showed an ill-defined, heterogeneously enhancing, hypodense area in the vastus lateralis, vastus intermedius, and quadratus femoris, suggestive of infective myositis but without abscess formation. The findings of contrast-enhanced computed tomography of the chest and abdomen were normal. The patient underwent ultrasound-guided muscle biopsy, which showed coagulative necrosis in the muscle/subcutaneous tissue; infiltration with neutrophils, lymphocytes, and plasma cells in the adjacent tissue; focal suppuration; granulomata with Langhans-type giant cells; and focal fibrosis in the muscle (Fig. ). The histopathological features were suggestive of melioidosis. A muscle biopsy specimen was sent for bacterial culture and antibiotic sensitivity testing as well as culture for tuberculosis and melioidosis, and all results were negative. The results of an indirect hemagglutination assay for melioidosis antibodies were highly positive with a titer > 10,240. The patient received intravenous meropenem for 2 weeks and was started on eradication therapy with oral cotrimoxazole 960 mg 12-hourly after the intensive phase and continued for 6 months. He was clinically improving with reduced pain over the lateral aspect of the right thigh and was fever-free by day 7 of treatment. He was discharged after 14 days of treatment with meropenem. On discharge, the patient’s ESR was 40 mm in the first hour, and his CRP level was 15 mg/L. At his follow-up appointment after 2 weeks, he reported resolution of symptoms. |
A 74-year-old male with a past medical history significant for chronic obstructive pulmonary disease, cerebral vascular accident, hypertension, peripheral vascular disease, and type 2 diabetes mellitus presented to the emergency department with hematemesis versus hemoptysis 2 months after undergoing emergent TEVAR for a ruptured thoracic aortic aneurysm. A computed tomography (CT) angiogram illustrated an AEF.\nThe patient was admitted to our Surgical Intensive Care Unit (SICU) and resuscitated, then the patient was promptly taken to the operating room for flexible bronchoscopy and esophagogastroduodenoscopy (EGD). Bronchoscopy was negative for endobronchial lesions in all lung lobes. On endoscopy, a large thrombus was noted in the mid-esophagus and significant blood was present in the stomach. Following irrigation and suction, no active bleed was discovered in the stomach, and attention was given to the large esophageal thrombus believed to represent the connection to the aorta. The vascular surgery team who placed the initial endograft was consulted, and the decision was made to stent the esophagus as a temporizing measure to tamponade the fistula. Fluoroscopy was utilized to measure the length of the thrombus to determine the size of the stent needed to fully cover the area. The large clot measured around 5 cm, extending from the level of about 30 cm from the incisors to 35 cm. In order to ensure that the entire defect was completely covered, a 15-cm stent was chosen. The area was delineated with radiopaque markers, and under direct vision, a guidewire was placed through the endoscope which remained in the esophagus. Under fluoroscopic guidance, the 15 cm stent was deployed, extending above and below the region of the thrombus.\nThe interim between esophageal stent placement and definitive surgical repair allowed adequate time for medical resuscitation and optimization of the patient’s condition. Seven days after esophageal stent placement, the patient returned to the operating room for definitive surgical repair of the AEF including left muscle-sparing thoracotomy, partial esophagectomy and high cervical esophagostomy, and resection of the infected thoracic aortic stent graft under femoral-femoral cardiopulmonary bypass. The thoracic aorta was reconstructed utilizing a rifampin soaked 24 mm × 30 cm gel weave tube graft. The surgery was completed without complication and the patient tolerated the procedure well.\nPost-operatively, the patient was transitioned to enteral feeds via a J-tube. IV ampicillin-sulbactam was given for 6 weeks after tissue cultures grew Enterococcus faecalis. After a 22-day hospital course, the patient was discharged to a skilled nursing facility for further rehabilitation. |
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