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Ms. N. was a 79-year-old patient. Her main medical history included high blood pressure, cholelithiasis, and a lumpectomy of the left breast for benign adenosis. She was primiparous and menopausal from the age of 50 years and did not take any hormone replacement therapy. Her only family medical history was breast cancer in a sister.\nThis patient had consulted her general practitioner following the spontaneous appearance of a bruise on the left breast. A mammogram was then performed, which showed diffuse calcifications of the two breasts associated with a left breast cyst. Breast ultrasound confirmed the presence of the left cyst and showed a solid lesion of 11 mm in the left para-areolar region. The biopsy found a NOS grade II invasive carcinoma, triple negative, with a Ki-67 proliferation index of 15%.\nThe patient was referred to our service for further care. She presented a purplish skin lesion in the inferior internal quadrant of the left breast. This lesion had appeared spontaneously with no notion of trauma or taking of any anticoagulant (Fig. ). The patient's biological examination was normal and made it possible to eliminate idiopathic thrombocytopenic purpura and meningococcemia. In this context of an atypical presentation (ecchymotic purpura of unknown etiology), we performed a skin biopsy. The anatomopathological examination revealed the presence of triple-negative carcinomatous lymphatic emboli (Fig. , ) with a Ki-67 proliferation index of 30%, which may have corresponded to a breast origin, classifying the cancer as T4 according to the TNM classification.\nA second ultrasound was performed, showing an aspect of pseudocystic and partial tissue modification extending over 40 mm. In addition, axillary lymphadenopathy was visualized, and cytology confirmed the malignancy.\nA bone scan and whole-body CT scan were performed. They showed discrete hyperfixation foci in the ribs, spine, and pelvis, suspicious in this context, and also left axillary lymphadenopathy. After consultation with a dermatologist, we describe the lesion as follows: homogeneous purplish pseudo-ecchymotic infiltration of 65 × 100 mm of the left breast located in the internal para-nipple area extending to the upper and lower levels bypassing the nipple. It was associated with a left lateral extension with brownish-to-orange appearance, which may correspond to an inflammatory cancer.\nAfter discussion in a multidisciplinary meeting, the indication for neoadjuvant chemotherapy was retained. At that date, the ecchymotic lesion, initially located, now extended from the internal quadrants to the lower left quadrant. There was still a palpable lymphadenopathy in the left axillary region. The contralateral breast was free from any suspected clinical lesion.\nNeoadjuvant chemotherapy was started with 4 cycles of doxorubicin and cyclophosphamide combined with primary prevention with Granocyte 34 every 3 weeks. Following these well-tolerated treatments, we had observed a decrease in the purpuric lesion as well as a decrease in breast induration and a free axillary lymph node area (Fig. ).\nThereafter, the patient received 12 Taxol injections weekly. At the end of the treatment, there was a disappearance of the cutaneous involvement with simple persistence of hyperpigmentation without palpable mass. The lymph node area remained free.\nA re-evaluation after neoadjuvant chemotherapy was performed with an FDG-PET scan. Scintigraphy revealed discrete progression of the infracentimetric osteocondensations of the axial skeleton, without significant scintigraphic translation but remaining doubtful. The FDG-PET scan made it possible to confirm a complete metabolic response with absence of visualization of a suspected hypermetabolic focus over the entire explored volume of pelvic-spinal osteocondensations of scar appearance.\nClinically, we observed the almost complete disappearance of the purpuric lesion after the various neoadjuvant chemotherapy treatments (Fig. ).\nA total left mastectomy was then performed with a wide cutaneous excision associated with axillary dissection and an abdominal advancement flap, allowing a tension-free closure. The anatomopathological examination found a grade II plurifocal NOS invasive adenocarcinoma with high-grade ductal carcinoma in situ and abundant dermal lymphatic carcinomatous emboli.\nIn total, 3 tumors were found: in the upper internal quadrant (9 × 6 mm), in the lower internal quadrant (4 mm), and at the union of the outer quadrants (9 × 8 mm). Immunohistochemistry confirmed the triple-negative character with a Ki-67 proliferation index of 1%. Twenty lymph nodes were assessed, of which 12 were invaded and 10 presented a capsular rupture classifying the neoplasia as ypT4ypN1.\nThe medical file was again discussed in the multidisciplinary meeting, offering adjunct locoregional radiotherapy, maintenance chemotherapy with capecitabine (Xeloda), and PET-FDG monitoring. A new evaluation by FDG-PET scan was performed after radiotherapy, and 6 treatments with capecitabine confirmed the maintenance of a complete metabolic response.\nAfter the radiotherapy, there persisted a thoracic post-radiation hyperpigmentation and a grade 1 post-chemotherapy neuropathy. Two new treatments with Xeloda were performed, before the appearance of signs of poor tolerance required the treatment to be stopped. The last FDG-PET scan performed 17 months after the start of treatment confirmed the persistence of a complete metabolic response. We are continuing to monitor this patient by FDG-PET scan every 3 months. |
A 45-year-old female presented to our hospital with rapidly progressive double vision. She had a history of a right CCA (15 mm in diameter) [] that was diagnosed at our hospital 5 years earlier when she presented with transient right third nerve palsy. She had been treated with strict blood pressure control in our outpatient clinic without recurrence of symptoms. Annual magnetic resonance (MR) imaging had not shown any morphological change in the aneurysm. Seventeen days before the present admission, she experienced onset of acute intractable headache accompanied by a “swishing sound” in the right side of her head, which worsened gradually and which was not responsive to analgesics. Three days prior to admission, her headache subsided abruptly, and she developed double vision and worsening visual acuity of her left eye. On admission, she was distressed. Neurological examination revealed left third nerve palsy, left ocular bruit, left chemosis, slight left exophthalmos, and subjective reduction in visual acuity in the left eye. Ophthalmologic examination showed reduced visual acuity in the left eye (20/67) when compared with the right eye (20/50). Computed tomography and MR imaging revealed a remarkable enlargement of the left superior orbital vein (SOV), and cerebral angiography confirmed direct CCF caused by CCA rupture. The fistula tracked toward the contralateral (left) cavernous sinus and drained into the left SOV [Figure –]. Given the acutely worsening neuro-ophthalmologic symptoms as well as the robust direct CCF with the resultant engorged left SOV, urgent treatment was indicated to prevent devastating permanent neuro-ophthalmologic deficits. Endovascular treatment was not available on an urgent basis at our hospital. Given her young age and her stable systemic condition, we elected to proceed with open surgical trapping of the aneurysm in conjunction with high-flow bypass using a RA graft after obtaining informed consent.\nStandard neuroanesthesia with somatosensory evoked potentials (SSEP) of the left extremities was induced. With right cervical carotid bifurcation exposure, a curvilinear fronto-temporal skin incision was made, and the superficial temporal artery (STA) was meticulously prepared under a microscope. The RA graft was harvested concurrently by another surgeon. A fronto-temporal craniotomy was performed, and a subzygomatic tunnel was made for the RA graft. The Sylvian fissure was fully split under microscope, and the M1, M2, and M3 portions of the middle cerebral artery (MCA) and the supraclinoidal internal carotid artery (ICA) were exposed. First, an “insurance” STA-M4 bypass was made distal to the M2 portion for RA graft anastomosis.[] Then, the harvested RA graft was gently pulled through the subzygomatic tunnel. The distal end of the RA graft was anastomosed to the M2 (inferior trunk) of the MCA, and the proximal end was anastomosed to the external carotid artery (ECA). The patency of the anastomosis was confirmed with microvascular Doppler assessment, and the right cervical ICA was then clamped. At this stage, due to bleeding from the cavernous sinus, we incompletely trapped the aneurysm to control the bleed by clipping the ICA just distal to the origin of the ophthalmic artery, leaving only retrograde flow from the ophthalmic artery into the cavernous aneurysm. Then, extradural anterior clinoidectomy was performed. We exposed the ICA proximal to the ophthalmic artery as well as the distal dural ring, and some bleeding from the cavernous sinus was controlled with packing with Gelfoam. The C3 portion of the ICA was finally clipped []. Microvascular Doppler exploration through the lateral wall of the cavernous sinus did not detect any flow signal. We also confirmed anterograde flow into the ophthalmic artery via retrograde ICA flow provided by the RA graft. Significant SSEP change was not observed at all throughout the operation.\nImmediately after the operation, auscultation over the left eye confirmed disappearance of the ocular bruit. Furthermore, chemosis and exophthalmos resolved. Postoperative angiography confirmed patency of the high-flow bypass and complete occlusion of the CCF. MR imaging revealed no significant cerebral infarction in the revascularized distal right ICA area and showed marked shrinkage of the formerly engorged left SOV [Figure –]. An examination by an experienced ophthalmologist confirmed total resolution of the deficits. The patient returned to her daily life without any permanent neurological sequelae. |
A 74-year-old female presented to the emergency department (ED) with upper abdominal pain and melanotic stools. She had an elective open juxtarenal abdominal aortic aneurysm repair a month before her index presentation. She was hemodynamically stable. Her pertinent initial labs showed a hemoglobin of 6.7 g/dl (baseline 9.6 g/dl) with a hematocrit of 23%. Patient did not have any fever or leukocytosis. A CT abdomen with contrast done in the ED for abdominal pain showed nonspecific findings, i.e, irregularity of the “aneurysmal sac” with a small amount of fluid around the sac (see ) which was read by the radiologist as early postsurgical changes. She was admitted and was started on proton pump inhibitors. An esophagogastroduodenoscopy (EGD) was performed that revealed mild duodenitis. Her hemoglobin remained stable the next couple of days, and she was discharged home with a 6-8-week course of proton pump inhibitors. Two months later, she presented again with similar complaints with a drop of hemoglobin. A repeat EGD was performed that did not reveal any obvious source of bleeding, and she was discharged home after stabilization.\nA month later, she came for the third time into the ED with abdominal pain, hematochezia, and profound hypotension. Her pertinent laboratory findings include leukocytosis, low hemoglobin and hematocrit, thrombocytopenia, and transaminitis. She was resuscitated with IV fluids and blood transfusions. She was started on broad spectrum antibiotics after blood cultures were drawn. A CT abdomen and pelvis was performed which showed tiny foci of air at the anterior aspect of the native aneurysm wrap just inferior to the location where duodenum crosses (see ). At that time, a decision was made to perform push enteroscopy instead of simple EGD to evaluate second and third portion of duodenum which showed an aortoduodenal fistula with infected graft adherent to the bowel wall and extruding purulent exudate (see ). She underwent emergent surgical excision of the infected graft and bypass grafting to restore vasculature. Her blood cultures and cultures from the graft revealed methicillin-resistant Staphylococcus aureus (MRSA) and Streptococcus agalactiae. Aggressive management was continued with proper antibiotics in the intensive care unit, but her condition deteriorated, and she expired within several days. |
A 44-year old female patient presented with a clinical history of breast cosmetic augmentation surgery by saline-filled silicone implant, about 14 years ago. She had recently experienced pain, tenderness, breast enlargement and discomfort associated with rather firm indurations around the implant of the right breast, radiating into the right arm, for few months. There was history of radiation of pain into the right arm.\nThe Sonography was non-diagnostic because of the presence of breast implant. On systemic examination, neither lymphadenopathy nor hepatosplenomegaly were detected. There was no evidence of cutaneous involvement. Imaging studies also ruled out the possibility of any systemic disease.\nDuring the operation, large amounts of unusual fibrinous material were found around the saline-filled breast implant, mainly located in the lower inner quadrant LIQ. Then the suspicious tissues were biopsied and submitted for histological examination. The necrotic debris and surrounding tissue were fixed in 10% buffered formalin. Representative sections of the whole specimen were embedded in paraffin blocks, processed and stained with hematoxylin and eosin (H&E) for routine histological examination; also serial sections for immunohistochemical studies were prepared. Immunohistochemical staining was as performed by using antibodies, summarized in .\nUnder light microscopy, sections revealed predominance of necrosis intermingled with particles of neoplastic tissue of lymphoid origin, composed of many large anaplastic lymphoid cells with large lobulated nuclei, showing rather irregular nuclear membrane and one or more prominent basophilic nucleoli. The cytoplasm was slightly basophilic with prominent Golgi region. Mitotic figures were frequent. There were also many histiocytes, containing abundant pale cytoplasms. Some sections included portions of a dense fibrous capsule, associated with diffuse marked infiltration of eosinophils.\nThe anaplastic cells exhibited positive immunoreactions for leukocyte common antigen (LCA), CD3, CD30 () and granzyme B (). However, they were negative for AlK (), epithelial membrane antigen, cytokeratin, CD 5, CD 7 and CD20. The final diagnosis was ALK-negative ALCL (). |
A 67-year-old man complained of left shoulder pain for 2 weeks without any history of trauma. Bloody effusions were aspirated 5 times at a local orthopedic clinic during a 2-week period before the patient was referred to us. He had no previous history related to hemorrhagic factors. On examination, he had slightly limited range of motion, with active forward flexion to 160°, abduction to 120°, external rotation to 30°, and internal rotation to the fifth lumbar spine with the arm at the side. Passive range of motion was the same as active range of motion. Neer's impingement test and Hawkins test were positive. The greater tuberosity was seen irregular on radiography. MRI demonstrated a hematoma in the anterior portion of the subdeltoid bursa and bony erosion of the lateral acromion, similar to case 1 ().\nArthroscopy was performed to determine the cause of the hemorrhage. A hematoma was not observed in the glenohumeral joint. The intra-articular portion of the long head of the biceps tendon had completely disappeared. A bursal-side partial tear of the rotator cuff was noted. The size of the tear was 25 mm in the anteroposterior direction. Crater formation on the undersurface of the acromion and irregularity of the greater tuberosity were observed, which seemed to be kissing lesions (Figures and ). Active bleeding was seen at the exposed bone marrow of the acromion when the pressure of irrigation was lowered. The undersurface of the acromion was coagulated and the greater tuberosity was abraded to avoid impingement (). Acromioplasty was not performed in this case. The bursal-side partial tear was not repaired at that time ().\nOne month later, the patient underwent arthroscopic rotator cuff repair. The patient regained full range of motion 3 months after rotator cuff repair and returned to work as a taxi driver. At the final follow-up, 14 months after the first arthroscopy, he had no functional deficit. The UCLA score was improved from 14 points preoperatively to 35 points at the final follow-up. Hemarthrosis did not recur in the 14 months postoperatively. |
A 40-year-old ethnic Han Chinese female, who used to be healthy and had no history of disorders related to the nervous system, respiratory system, circulatory system, digestive system, rheumatic immune system, and also had no history of mental disorders, from a family without history of genetic disorders, hypertension, coronary heart disease, diabetes, genetic inherited diseases, etc., forming a happy family with her beloved husband and two healthy daughters, initially presented with intractable nausea and dizziness for 9 weeks. She went to local hospital. Full blood count, serum biochemical tests, gastroscopy and head CT examination showed no obvious abnormalities. She felt that her nausea and dizziness did not improve after symptomatic therapy and even followed by dysphagia, limb weakness, blurred vision, ghosting in the right eye, and fatigue. Two weeks later, she went to another local hospital. A blood test was positive for AQP4 antibodies and brain MRI found high-signal lesions (). She was diagnosed with NMO and treated with high dose of intravenous methylprednisolone. Two days later, she felt shortness of breath, hard to swallow and difficulty in walking. She was transferred to the ICU and received a tracheotomy and mechanical ventilation due to severe breathing difficulties, and a nasogastric tube feeding due to difficulty swallowing. Her condition did not improve over 2 weeks despite treatment with high-dose intravenous methylprednisolone (0.5 g per day for 3 days, 0.25 g per day for 3 days, 0.12 g per day for 2 days) and immunoglobulin (20 g/day for 5 days) followed by 3 rounds of plasma exchange.\nShe was transferred to our hospital at midnight, and physical examination revealed a temperature and blood pressure of 37.1°C and 96/68 mmHg, respectively. She was conscious on mechanical ventilation, with blurred vision of the right eye, shallow right nasolabial fold, upper limb muscle strength level 1, and lower limb muscle strength level 0. Laboratory tests revealed a white blood cell (WBC) count of 28,240 cells/μL, serum albumin 32.3 g/L, prothrombin time 16 s, activate partial thrombin 38.6 s, and D-dimer 2.24 μg/mL. Rheumatoid, lupus, and vasculitis-related immune indicators, and biochemical indicators related to the heart, liver, and kidney function showed no obvious abnormalities. Tuberculosis-specific enzyme-linked immunospot assay (T-SPOT), hepatitis B virus, hepatitis C virus, HIV, CMV-DNA, CMV-IgM, and Epstein-Barr virus (EBV)-IgM were negative; however, the CMV-IgG antibody was positive. Alpha-fetoprotein, carcinoembryonic antigen, and carbohydrate antigen (CA) 19-9, 125, 153, 50, 724, and 242 were negative. Blood and CSF smears and cultures were negative.\nOn day 2 of admission, the patient developed 150 ml suspicious menstrual blood, accompanied by a high fever, and marked increase in C-reactive protein (CPR) and procalcitonin (PCT); however, WBC count, hemoglobin concentration (1, 3), -β-D-glucan and galactomannan (GM) tests, and liver and kidney function were not significantly altered. Chest CT showed new glass-like lesions on both upper lungs. Multiple blood, sputum, and urine specimens were taken for pathogenic cultures (all final culture results were negative). Considering the hospital stay and administration of high doses of hormones in the external hospital, the patient was diagnosed with pulmonary infection and underwent an empirical broad-spectrum antibacterial and fungal treatment with meropenem, linezolid, and caspofungin. On day 3 of admission, the patient's body temperature peak began to decline; however, she suddenly passed 640 ml dark red bloody stool painlessly, resulting in shock. Blood transfusion and drug hemostasis treatment were administered to treat the shock. Meanwhile, bedside gastroscopy, colonoscopy, and full abdominal CTA scan were performed to identify the cause of bleeding. Abdominal CTA found a high-density shadow in the colon and rectum, which was considered an accumulation of blood; however, no bleeding vessels were found. Gastroscopy found no lesions in the upper digestive tract. However, colonoscopy revealed a large ulcer-like lesion at 10 cm above the anus and a large amount of feces and dark red bloody fluid (). Biopsy of ulcer-like lesions could not be performed due to coagulation dysfunction. On day 4 post-admission, the patient's body temperature returned to normal. On day 7 post-admission, the WBC count, CRP, PCT, and chest CT were normal; therefore, the anti-infective agents were removed; however, the daily excretion of 200–800 ml dark red bloody stool of this patient continued, and her muscle strength and vision loss did not improve.\nBased on the patient's bleeding characteristics and lesions found via colonoscopy, it was speculated that the cause of the patient's acute lower gastrointestinal bleeding was most likely AHRU. The pathogenesis of AHRU remains largely unknown. Therefore, we speculated that the presence of AHRU in this patient may be linked with her NMO.\nTo assess this link, we reviewed literatures and only found one reported case of an acute NMO with rhabdomyolysis in an immune-competent patient following CMV infection (). Infectious pathogens may trigger and exacerbate NMO by prompting AQP4-IgG production (). AQP4-IgG+ NMO is refractory to glucocorticoid treatment. In this case, the patient did not respond to high-dose intravenous methylprednisolone and immunoglobulin followed by plasma exchange. Therefore, we speculated that NMO in this case was caused by the CMV infection.\nThereafter, we wondered whether CMV infection could induce AHRU. Enteritis () caused by CMV in immune-competent individuals had been reported, although very rarely. Interestingly, no relevant literature about CMV-AHRU was found. However, ARHU may be caused by CMV infection from a monistic point of view. To verify this, on day 23 post-admission, CMV inclusions of pathology of a mucosal tissue specimen from the edge of the patient's rectal ulcer was carried out using routine HE staining and IHC. These results confirmed the presence of CMV infection (). In addition, CSF pathogen gene sequencing also detected CMV gene copies on day 25 post-admission () and serum AQP4-IgG was still positive.\nAfter 2 weeks of treatment with ganciclovir and sodium phosphinate, the patient's nausea, dizziness, visual impairment, and limb weakness gradually improved. After 3 weeks of antiviral therapy, her lower gastrointestinal bleeding stopped, and the CMV staining of rectal biopsies (), CSF CMV gene sequencing, and serum AQP4 all became negative. Further, high-signals on the brain MRI were significantly reduced (). After 4 weeks of antiviral therapy, the colonoscopy revealed a smooth intestinal wall at the original lesion (). In addition, her right eye visual acuity was resumed to counting fingers at 2 m, the muscle strength of her upper and lower limbs recovered to level 4 and 3, respectively. Following this, the antiviral drugs were stopped and the patient was discharged back to her local hospital for further rehabilitation. |
A 59-year-old otherwise healthy Caucasian man was diagnosed with a mucosal melanoma during hemorrhoid evaluation in August of 2009. Histopathological examination revealed a polypoid tumor occupying lamina propria and submucosa of the anal canal with intraepithelial lentiginous component in the center of the lesion. The tumor cells were epithelioid and showed clear cell change. Immunohistochemical studies showed the tumor cells to be positive for S100 and Melan-A. A diagnosis of a 15-mm thick mucosal melanoma with ulceration, 6 mitotic figures per mm2 and perineural invasion in the anal canal was made (Figures , and ). Molecular analysis showed the melanoma harbored a GNAQ mutation with wild-type BRAF, KIT and NRAS genes. The GNAQ gene mutation of the patient was the substitution of glutamine to proline in codon 209 (Q209P) which has been reported in uveal melanoma at a frequency of 20.8% but not in cutaneous melanoma or other subtypes of the disease [,].\nThe patient underwent a wide local excision of the primary melanoma with subsequent adjuvant radiation therapy. He was without relapse until January of 2010, when he had locally recurrent disease for which he underwent another wide local excision. He again remained free of disease until July of 2010 when he was found to have metastatic lesions in the perinephric lymph nodes, the liver, and lung, for which he received 2 doses of ipilimumab (3 mg/kg intravenous Day 1) and temozolomide (200 mg/m2 by mouth Days 1–4) [] with further disease progression and new metastatic lesions in hilar and mediastinal lymph nodes and in the right adrenal gland. Subsequently, he received two cycles of the combination of carboplatin, paclitaxel and bevacizumab before he had further disease progression in January of 2011. He started imatinib at 400 mg twice a day in February of 2011. Due to further disease progression with imatinib, ipilimumab (3 mg/kg) was re-introduced in April of 2011 and he completed 4 cycles of ipilimumab. However, his disease progressed further with multiple metastatic lesions and he expired in August of 2011. |
A 15-year-old boy attended the Emergency Department (ED) with a short history of a pale left arm. He denied pain but complained of some altered sensation throughout his left hand. He had a significant recent medical history, having suffered a fractured midshaft of his left clavicle (see ) managed conservatively with a broad arm sling 3 months previously. Furthermore, he had 3 recent attendances to the ED with pain and “pins and needles” which felt like “a trapped nerve,” the first of which was a sudden onset of clavicular pain and altered sensation on throwing a ball. At each ED visit he was assessed, reassured, and discharged with documentation stating “no neurovascular deficit.”\nPhysical examination on his final visit revealed a warm but pale arm with no radial, ulnar, or brachial pulses palpable, but a normal capillary refill time of 2 seconds, and a reduced power in his intrinsic muscles of his left hand, but normal peripheral neurovascular examinations in remaining limbs and an electrocardiograph showing sinus rhythm. Chest X-ray confirmed no cervical ribs.\nA duplex ultrasound scan showed a loss of flow in the brachial artery below the left elbow, with thrombus partially occluding the artery. Also noted was a 3 cm abnormality in the left subclavian artery that was thought to be a subclavian aneurysm that contained thrombus.\nSubsequent Computed Tomography (CT) of the thorax (Figures and ) confirmed a diagnosis of a 4.5 × 3.5 × 2.5 cm false aneurysm of the subclavian artery lying on the superficial aspect of the anterior second rib and also showed evidence of the previous mid-clavicle fracture that had healed but malaligned. The CT also showed the distal end of the proximal shaft protruding posteriorly into the false aneurysm.\nTransfer was arranged that day to the local acute vascular surgeon on call, where he underwent a subclavian-axillary bypass and brachial embolectomy. After 4 days of recovery as an inpatient including a heparin infusion he was discharged, symptom-free. |
A 37-year-old gentleman was referred to see a general surgeon with a 4-week history of right groin pain on a background of a previous right inguinal hernia repair. He was otherwise well with no major systemic comorbidities. A computed tomography (CT) scan of the abdomen and pelvis had been organized, which did not demonstrate an abdominal wall defect. Incidentally, there was a 3 cm lobulated cystic mass below the perineal floor on the right side abutting the rectal wall (). He denied any symptoms of perineal pain or alteration on bowel habit. On examination, his abdomen was benign with no groin cough impulse felt bilaterally. Digital rectal examination (DRE) was normal with no palpable mass.\nA magnetic resonance imaging (MRI) was performed demonstrating a multiseptated perianal cystic collection on the right side ~3 cm from the anal verge extending craniocaudally (). The working diagnosis at the time was a complex congenital cyst. A thin rim of T2-hypointense material was present on its medial wall indicating a solid component, which raises the possibility of malignant potential () []. A fluorodeoxyglucose (FDG)-positron emission tomography (PET) scan was performed accordingly and while the majority of the cyst was poorly FDG avid, the solid component was difficult to assess due to surrounding artefact from the adjacent rectum (). The decision was made to proceed with surgical excision.\nA trans-sacral approach was adopted. The patient was placed in the prone position and a midline incision was made over coccyx and lower sacrum. The muscles were retracted to access the right ischiorectal fossa and the lobulated cyst was dissected off the right sphincter complex to retrieve the specimen. A coccygectomy was subsequently performed. The pelvic floor was repaired with 0-vicryl sutures, the rectum was intact on DRE and the skin was subsequently closed.\nMacroscopically, the specimen consisted of a 45 × 35 × 23mm fluctuant cystic mass with serial sectioning indicating it was filled with sebaceous material (). Microscopically, haematoxylin-and-eosin staining was indicative of an SCT containing squamous, transitional and respiratory epithelium (). No immature or malignant elements were visible. Interestingly, while the coccyx was macroscopically normal, on microscopy there was adjacent fibrous tissue containing prostatic epithelium later confirmed with immunohistochemical staining ().\nThe patient’s post-operative recovery was uncomplicated. He was discharged from hospital the following day with a plan for 6-monthly surveillance imaging. |
A 41-year-old man was presented to the emergency department with progressively worsening suboccipital headache, ataxia, and diplopia. The patient had headache for 4 weeks, and it gradually increased in severity and persistence during this period. No clear precipitating and alleviating factors were identified. He also noticed that his walking gait became more unsteady and he tended to lean toward his left side. Diplopia had also been noticed a week before this presentation. No speech and language deficits, personality change, and difficulty in performing complex tasks were apparent. The patient was otherwise healthy without significant past medical or surgical histories.\nOn examination, the patient was alert and oriented to the place, time, and persons. He was in moderate distress due to his severe suboccipital headache. His body temperature was 37°C, blood pressure was 140/80 mmHg, heart rate ranged from 60 beats to 70 beats per minute, and his respiratory rate was 18 breaths per minute. Cranial nerve examination showed diplopia at all gazes and a left horizontal nystagmus. Cerebellar disturbance was found in the left upper and lower limbs, including dysdiadochokinesia and dysmetria in a finger-to-nose test and a positive heel–shin test. A wide-based standing posture and walking gait as well as a positive tandem walking test were also noticed. Other examinations were unremarkable.\nA computed tomography (CT) scan of the head using an intravenous contrast revealed a solid lesion of 2.5 cm in diameter with lobulated cysts and mild contrast enhancement in the fourth ventricle (). A nearly complete obstruction of the foramen magnum by cerebellar tonsillar herniation and a dilatation of the third and lateral ventricles with subependymal edema were also demonstrated ().\nThe patient was then transferred to the intensive care unit (ICU), treated with dexamethasone, and then prepared for an urgent magnetic resonance imaging (MRI) study and neurosurgical intervention. Two hours after the ICU admission, paroxysmal sinus bradycardia was found and patient heart rate oscillated between approximately 30 beats and 70 beats per minute. The occurrence of transient bradycardia made it difficult to capture blood pressure using a non-invasive blood pressure monitor. The patient complained about intensified headache during the bradycardia period.\nHe was then transferred to an operating theater for the insertion of an external ventricular drain. Intracranial pressure was 32 cm of water, and CSF was intermittently drained to stabilize this pressure. Later, an MRI of the brain confirmed the CT findings of hydrocephalus and cerebellar tonsillar herniation (). The extension of the lesion to the left foramen of Luschka was further demonstrated. An MRI of the spine revealed a syrinx extending from the C2 to T9 vertebrae and no leptomeningeal seeding (). The tumor was subsequently removed via suboccipital approach, and histopathological studies demonstrated classic medulloblastoma.\nThe patient’s presenting symptoms were resolved subsequently, and no postoperative focal neurological deficits were found. He was discharged a week later after undergoing outpatient radiation therapy, which involved a complete dose of craniospinal radiation with a boost on the primary tumor site. Two years after treatment, repeated magnetic resonance images showed the resolution of the syrinx and no clear radiological evidence of disease relapse (). |
A 10-year-old boy attended with his left condyle fractured, caused by a soccer accident. The patient was diagnosed with painful facial swelling localized over the left condylar region, and lateral deviation of the mandible to the left side resulting in a facial asymmetry. His medical history was found to be non-significant. Intraoral examination revealed an anterior crossbite and excessive crowding. Limited mouth opening and mandibular deviation during opening and closure were observed, and mastication and speech were both affected. The clinical diagnosis was supported by panoramic radiography and 3D computed tomography (CT) views. Panoramic radiograph and CT views confirmed the green-stick (incomplete) fracture on the left condyle in which one side of the bone was fractured and the other was bent (Fig. ).\nClosed reduction was chosen as a treatment method to allow to initial fibrous union of the fracture segments and obtain remodeling with normal functional stimulus. Alginate impressions of both jaws were taken and stone working models poured. A 3 mm thick removable acrylic mandibular splint was made by our laboratory technician. Then, a non-rigid mandibular splint was applied in order to remove the direct pressure on the fracture side (left) of the mandible for one month with functional repositioning of the mandible (Fig. ). Soft diet and rest were suggested. Instructions were given to his parents as for the cleaning of the mandibular splint with a soft toothbrush.\nClinical and radiologic examination at 1, 3, 6, 12 and 27 months revealed uneventful healing with reduction of the condylar head and remodeling of the condylar process following conservative treatment (Fig. ). During these follow-up periods, it was observed that the patient was cooperative and used his splint in a proper way. At 1, 3, 6, 12 and 30-month CT follow-ups, neither deviation nor pain was observed. It was also observed that the patient’s occlusion was normal and mouth opening was within normal limits. After initial treatment, reduction of the condylar head in the fossa, correct positioning of the ramus (repositioning) and new bone modeling (i.e., remodeling) of the submandibular condyle observed by CT scan resulted. Bone callus was observed on the anterior surface of the condyle, and the condylar axis was flattened. TMJ disorders such as ankylosis and dysfunction, or malocclusion as well as the limitation of the mouth opening were not observed after 30 months (Fig. ). It was noticed that the patient has a Class I occlusion with anterior crowding. However, since orthodontic tooth movement may precipitate dysfunctional symptoms, orthodontic teeth alignment was postponed until complete healing had occurred. |
A 70-year-old male farmer presented in skin outpatient department with multiple firm, erythematous, and ulcerated skin nodules all over his body for 35 days. The patient had no history of fever, chest pain, cough, cold, or any previous major medical illness. A provisional clinical diagnosis of cutaneous T-cell lymphoma was considered and skin biopsy with other basic investigations was planned. Suddenly patient developed altered sensorium and respiratory distress and was brought to emergency. On systemic examination, bilateral chest had rhonchi. Hematological and biochemical investigations were found to be within normal limits. Oncology reference and radiology workup were asked for but the patient succumbed to his illness within 24 h of admission. A complete autopsy was performed. External examination exhibited multiple skin nodules all over his body ranging from 1 to 10 cm in diameter []. A few nodules had central ulceration. Systemic examination revealed multiple transmural nodular deposits in stomach, small and large intestines along with pancreas []. Lungs exhibited features of bronchopneumonia. The histopathological examination of the skin nodules and nodular deposits from GIT revealed a malignant tumor comprising of large monomorphic tumor cells arranged in sheets. The cells were large with round to oval nucleus with prominent nucleoli and moderate amount of eosinophilic cytoplasm [ and ]. Mitotic activity was brisk. Initial immunohistochemistry (IHC) workup revealed weak positivity for CD45 and S100 in tumor cells with negative staining for CD 20, CD 3, and AE1-AE3. Ki 67 index was 70%. These findings ruled out carcinoma. Additional markers for lymphoma, melanoma, and histiocytic tumors were performed. The tumor cells were negative for CD5, PAX5, CD30, CD 56, HMB 45, CD 23, CD 38, CD138, Alk 1, and MPO but showed strong positivity for CD 68 []. CD 68 positivity suggested histiocytic nature of tumor cells and further IHC for CD 1a, lysozyme, and CD 163 was added for confirmation. The tumor cells were diffusely and strongly positive for lysozyme [], focally and strongly positive for 163 and negative for CD 1a, confirming the diagnosis of HS. Since this was a medical autopsy performed with relatives’ permission, the body was handed over to the relatives after autopsy as per hospital rules. |
A 57-year-old white male with history of Graves' disease with ophthalmopathy and coronary artery disease status after 3-vessel CABG presented to the ER after a syncopal episode during a doctor's office appointment. He had been having severe frontal headaches for several months with workup prior to this admission revealing an elevated ESR and bilateral temporal artery biopsies which were negative for giant cell arteritis. A lumbar puncture revealed elevated protein, increased opening pressure, elevated WBC, IgG index, and oligoclonal bands. A bone marrow biopsy performed for microcytic anemia was unremarkable.\nGiven his history of syncope, a CT angiogram of the chest was performed to exclude a pulmonary embolism. No evidence of pulmonary embolism was seen; however, soft tissue attenuation around the coronary arteries, bypass graft, and pericardium raised concern for vasculitis (Figures and ). Further workup with cardiac MRI demonstrated enhancing soft tissue around the graft and coronary arteries with a nodular appearance of the pericardium (). Conventional coronary angiogram revealed complete occlusion of LAD and right coronary artery although the bypass grafts were patent and there was no evidence of vasculitis ().\nA pericardial biopsy demonstrated dense scar tissue associated with a mononuclear infiltrate comprised mostly of nodular aggregates of monocytes and macrophages (). Within the nodular aggregates, myeloperoxidase-positive neutrophilic infiltration was localized to very small venules and capillaries with associated leukocytoclasis (). This granulomatous capillaritis with leukocytoclasis and peculiar mononuclear infiltrate was suggestive of GPA (). Although not as sensitive and specific as c-ANCA, positive p-ANCA serologies as in our patient have been associated with GPA.\nMyeloperoxidase associated ANCA vasculitis with orbital pseudotumor and pericardial involvement was considered to be the most likely diagnosis. He was discharged after being placed on prednisone and rituximab. Subsequent contrast-enhanced chest CT 3 years later showed significant improvement of the soft tissue thickening around the coronary arteries, bypass graft, and pericardium (). |
This patient is a 14-year-old male, who felt a popping sensation and significant right knee pain while jumping and colliding with another player during a basketball game the previous day. Following the injury, he was evaluated in an outside emergency department, where anterior, posterior, and lateral radiographs obtained in the emergency department demonstrated a tibial fracture consisting of two primary components (). He was placed in a cast and sought a second opinion regarding findings and management.\nUpon presentation to the clinic the following day, he reported mild pain (3/10) and noted no normal function of his leg. A physical exam was performed but was limited due to pain. Following the review of radiographic imaging, an MRI was performed, which demonstrated a type IIIB tibial tubercle avulsion fracture and complete tear of the patellar tendon from its distal attachment site, as well as a hematoma at the fracture site (). After discussing the findings with the family, the patient was scheduled to undergo open reduction internal fixation of a type IIIB fracture and repair of the patellar tendon three days following the initial injury.\nAn 8-centimeter anterior incision was made at the superior aspect of the tibial tubercle and extended distally. At the patellar tendon insertion site on the tibia, the tendon was noted to be completely avulsed from the bone cortex distally, while proximally, the tendon remained attached to the displaced tubercle. The tendon remained attached to the inferior pole of the patella. The anterior tibial plateau fragment was anatomically reduced using two fully threaded noncannulated screws (Arthrex, Naples, FL), while the tibial tubercle fragment was reduced via bicortical fixation with a 50 mm fully threaded 3.5 mm cortical screw (Arthrex, Naples, FL).\nThe distal patellar tendon was completely avulsed through two-thirds of its length. To restore the native footprint of the patellar tendon, a 4.5 mm PEEK (polyetheretherketone) corkscrew anchor (Arthrex, Naples, FL) was placed slightly lateral to the anatomic insertion site to avoid a stress riser on the anterior tibial cortex. The anatomic repair of the patellar tendon was completed with two mattress sutures and tied.\nIn addition to the avulsion of the patellar tendon and periosteum, it was noted that fascial tissue with tibialis anterior muscle belly avulsed through the injury site causing subacute extensive bleeding within the anterolateral compartments (). This scenario raised concern for impending compartment syndrome, and an anterolateral compartment release was planned.\nThree 3-centimeter incisions were made along the anterolateral aspect of the leg. The first was located 3 centimeters distal to the neck of the fibula, the second was located 10 centimeters above the distal fibula tip, and the third was located at the midpoint between the two. Under endoscopic visualization, the intramuscular septum was identified and Metzenbaum scissors were used to cut through the fascial compartment beginning in the anterior compartment and extending proximally then distally to the midtibia (). The fascial incision was extended posteriorly into the peroneal compartment and then was extended proximally and distally to the midtibia. These steps were repeated for the midpoint and distal incision sites. Distally, the course of the superficial perineal nerve was identified and the nerve itself was protected during the distal release of the anterior compartment. It was believed that the impending compartment syndrome occurred due to damage to the surrounding bony and muscular tissue. A medium Hemovac drain was placed along the length of the lateral compartment, exiting in the posterolateral proximal leg. The patient was placed in a hinged knee brace which was locked in extension. He was discharged home later that day.\nOn postoperative day number two, the patient's Hemovac drain was removed by a family member. The patient was seen 1 week postoperatively and noted moderate pain (6/10) and 0% normal function. On physical examination, incisional sites were clean, dry, and intact and a small fracture blister was noted on the posterior aspect of the knee—which was cleaned and redressed. Radiographic imaging revealed well-positioned screws, no evidence of new fractures or foreign bodies, and early evidence of callus formation. Two and a half weeks after surgery, the patient presented to the clinic for evaluation. He reported that he had no pain (0/10) and had 5% of his normal function at this time. On physical examination, he noted no tenderness to palpation of the knee joint, and he had 40 degrees of knee flexion. Anterior-posterior and lateral X-rays were taken which showed evidence of callus formation in the bone (). At this time, it was recommended that the patient begin gentle active range of motion exercises with extension and light flexion. He was also encouraged to become full weight-bearing with the brace until its removal two months postoperatively.\nFive months postoperatively, the patient reported no pain (0/10) and possessed 95% of his normal function at this time. On physical examination, he was nontender to palpation along the joint line. There was no laxity with varus or valgus stress. He demonstrated 5/5 quadriceps strength with no evidence of an extensor lag. He had an active range of motion from 0 to 130 degrees of flexion, and there was no lag with straight leg raise. Repeat anterior-posterior and lateral X-rays demonstrated a well-reduced tibial tubercle fracture as well as well-positioned and nondisplaced hardware (). |
A 24-year-old Caucasian man presented with a several month history of muscle pain, fatigue and insidious onset of pitting edema to his lower extremities. His symptoms progressed to include bilateral arm swelling, muscle pain to the thenar eminence of both hands, and paresthesias to his hands. He did not have a rash, joint pain, Raynaud phenomenon, oral ulcers, fever, hardening of the skin or weight loss, and he denied any cardiac, respiratory, genitourinary, or gastrointestinal symptoms. Prior to symptom onset, he had traveled to South America and reported possible ingestion of undercooked meat as well as swimming in a river and lake. He had frequent visits to the Northeastern United States and had recently spent an extended period of time outdoors in Rhode Island. Medical, surgical, and family histories were unremarkable, and he was not taking any medications. He had a history of mild alcohol intake, no history of smoking, and some marijuana use. On examination, his vital signs revealed a blood pressure of 104/57 mm Hg, pulse of 56 bpm, and normal temperature. He had no significant findings on head, neck, cardiovascular, respiratory, or abdominal exam. He had no cervical, axillary, or inguinal lymphadenopathy. He had significant pitting edema on his feet and legs extending up to his knees as well as non-pitting edema on the dorsum of both hands. Although there was edema, the skin was soft without any significant hardening and was without any overlying erythema. There were no signs of skin dimpling or grooves. His neurological examination revealed normal strength.\nHis initial laboratory work was significant for a mild eosinophilia of 700 with a normal white blood cell count, hemoglobin, and platelets. He had normal calcium, creatinine, and electrolyte levels. His alanine aminotransferase was slightly elevated and total protein slightly low, but he had normal albumin and bilirubin. His thyroid-stimulating hormone was slightly increased, but his free T4 and total T3 were normal. His urinalysis was normal. He had a normal level of creatine phosphokinase, sedimentation rate, and C-reactive protein. Further workup revealed a positive anti-nuclear antibody with a titer of 1:160 with a speckled pattern. His extractable nuclear antigen panel was negative, including Scl-70. His ANCA, myeloperoxidase and proteinase-3 serum studies were also negative. His ACE level and complement levels were normal. Infectious workup revealed negative stool studies for culture, ova, and parasites. Antibodies for HIV, CMV, and Trichinella were negative. Serum for histoplasmosis, cryptosporidium, coccidiomycosis, and interferon gamma release assay were negative. A blood smear for parasites was negative. An ELISA IgG/IgM test for Lyme disease was positive with subsequent testing with Western blot strongly positive for IgG (eight out of ten bands positive) and also positive for IgM (two out of three bands positive).\nThe patient had extensive imaging done with a normal CT scan of his neck, chest, abdomen, and pelvis. An echocardiogram was also normal. Due to a previous negative workup along with persistent pain and swelling, an MRI of his right lower extremity was performed (see Figure ). Imaging demonstrated extensive circumferential edema with enhancement of the superficial soft tissues, superficial fascia, and, to a lesser extent, deep fascia of the lower leg. Taking into account the mild peripheral eosinophilia and the imaging findings, a diagnosis of eosinophilic fasciitis was considered. To complete the workup, a biopsy of the fascia, muscle, and adipose tissue of the left calf was taken (see Figure ). The biopsy did not include the dermis. Surprisingly, there was no evidence of eosinophilic fasciitis. Instead, the specimen illustrated a striking granulomatous fasciitis and vasculitis. The fascia showed exuberant granulomatous inflammation (Figure A) with an inflammatory infiltrate that was made up predominately of histiocytes and CD3-positive T cells with very rare eosinophils (Figure B). The granulomatous inflammation centered primarily on small- to medium-sized blood vessels and was non-necrotizing. While the vessels did not display overt fibrinoid necrosis, they did appear damaged with loss of endothelial cells confirmed with CD31 immunostaining. The inflammatory infiltrate was seen extending into adipose tissue and particularly around blood vessels within the fat. The adjacent skeletal muscle also showed perivascular inflammation and vasculitis in both the perimysial and endomysial compartments. There was no endomysial fibrosis, fatty infiltration, or inflammation surrounding muscle fibers. AFB (acid-fast bacteria) and Wade-Fite stain were negative for mycobacterial organisms. GMS (Grocott-Gomori's methenamine silver) stain was negative for fungal organisms. Due to the positive Western blot for Lyme, a Warthin-Starry silver nitrate stain was performed to evaluate for spirochetes; however, no definitive organisms were seen. A Borrelia PCR analysis of the tissue was performed as well, but no DNA was detected.\nThe patient was given a diagnosis of granulomatous fasciitis along with a diagnosis of Lyme disease. He was first treated with doxycycline for 42 days straight due to initial Lyme serology being positive. The calf biopsy revealing fasciitis was not performed until the patient was 3 weeks into the doxycycline course. Once fasciitis was diagnosed, he was started on a prednisone taper starting at 1 mg/kg/day for a week with taper by 10 mg every 2 weeks. After 2 months of treatment with prednisone, the patient had near resolution of symptoms. A repeat MRI was performed 82 days after the initial MRI with the previously seen changes consistent with fasciitis nearly completely resolved with only a thin sliver of edema over the superficial fascia. On re-evaluation of the patient, he reported skin changes to his left upper arm at 20 mg of prednisone per day and skin changes to his left forearm at 7.5 mg per day. Examination of the upper arm revealed an atrophic patch with some overlying erythema and examination of the forearm showed indurated, bound-down, tense skin with a slight groove and minimal overlying hyperpigmentation. The rest of the dermatological examination was within normal limits. Skin biopsy revealed marked septal thickening with sclerosis, sparse lymphoplasmacytic infiltrate along the dermal subcutaneous junction, and swollen, homogenized collagen fibers with diminished spaces between the fibers (see Figure ). The clinical examination along with pathology revealing dermal and subcutaneous sclerosis was consistent with a diagnosis morphea profunda. Lyme serology was repeated, but Western blot for IgG was negative. A scleroderma antibody panel was negative as well. He was started on methotrexate and a higher dose of prednisone. |
A 22-year-old woman was admitted to our hospital with a history of intermittent colicky right hypochondrial pain not relieved by any medications for the past 3 months. Initially, she was given proton pump inhibitors, but her pain was not relieved. She had no other symptoms. Her past medical and family histories were not significant. The result of her clinical examination was normal. Upper gastrointestinal (GI) endoscopy showed globular swelling at the medial wall of D2 (Fig. ). The ampulla was situated at the summit of swelling.\nComputed tomography (CT) with oral contrast agent showed dilation of the intramural part of the common bile duct (CBD). A cyst of size 2.4 × 2.3 cm was noted in the second part of the duodenum (Fig. ). On the basis of the above findings, it was reported as type 3 choledochal cyst. The patient was further investigated with magnetic resonance imaging, which showed dilation of the intramural part of the distal CBD. A 2.4 × 2.3 cm cyst was noted in the ampullary region, again consistent with a type 3 choledochal cyst (Fig. ). Then the patient was planned for endotherapy, but owing to difficulty, it could not be negotiated into the ampulla. Finally, the patient was referred to the surgical gastroenterology department for operative management. After multidisciplinary team discussion, an abdominal ultrasonogram (USG) was done, which showed a clear thick wall cyst measuring 4.6 × 2.6 cm between the second part of the duodenum and the head of the pancreas with gut signature (Fig. ). Opening of the CBD into the duodenum was seen just distal to the cystic lesion. There was active peristalsis noted all around the cyst, suggestive of duodenal duplication cyst.\nAfter the diagnosis was confirmed, the patient was taken for elective laparotomy. Intraoperative findings were a 5 × 3-cm cyst over the medial wall of the duodenum extending proximal and distal to the ampulla and displacing it posteriorly (Fig. ). The cyst was communicating with the ampulla by a small opening in its medial wall (Fig. ). So, cyst secretions were drained via the ampulla, which avoided retention of cystic fluid. Part of the cyst wall was shared with duodenal musculature. Upon needle aspiration, the cyst wall contained bile due to communication with the ampulla. The duodenum was Kocherized, and a longitudinal duodenotomy was made for about 5 cm. Partial excision of the cyst was done (Fig. ) because it was closely associated with the ampulla. Marsupialization of the remaining cyst wall was done. The duodenotomy was closed horizontally. Feeding jejunostomy (FJ) was done using a modified Witzel method. A flank drain was kept in place. The postoperative period was uneventful. The patient was started on oral medications on the third day after surgery. The FJ tube was removed after 6 weeks. Histopathology showed the cyst wall was lined by duodenal mucosal epithelium with focal areas of ulceration and composed of tall columnar cells with goblet cells on either side of a common (shared) muscular layer. The submucosa showed lymphoid aggregates with Brunner glands. The common muscular layer showed congested vessels. Histopathological features were suggestive of duodenal duplication cyst (Fig. ). At her 9-month follow-up visit, the patient had no complaints. |
Patient GN is a 68-year-old right-handed woman without previous psychiatric or neurological episodes, and a normal seven-years education. She was admitted to our neurological clinic because of spontaneous complaints about mild episodic memory impairment and about a generalized and compelling feeling of familiarity and even intimacy for unknown people’s faces. This erroneous face familiarity recognition involved people met personally during daily life activities as well as people seen on television. HFF started about six months before admittance and gradually increased up to become severe and with direct consequences in daily life. In fact, HFF was spontaneous and immediate, and happened constantly throughout the day to the extent that for many (unknown) persons met or seen, GN engaged in effortful memory searching, trying to remember the circumstances, episodes and reasons that made the person’s face look familiar. The failure to recall such circumstance, and the stress related to it, leaded her to social detachment, as she stopped leaving the house or watching TV. By contrast, her ability to recognize truly familiar faces was unimpaired and correct recognition was associated with retrieval of the specific identity and correct name. GN never misidentified a person for another and never believed that people were disguised. Therefore, GN’s HFF appears as a selective disorder, qualitatively different from other misidentification syndromes, such as the Capgras (the pathological belief that a patient’s family member has been replaced by an identical impostor) or Fregoli syndrome (the delusional belief that different persons are in fact a single person in disguise) [, ].\nMedical and neurological examination was entirely normal as well as standard laboratory blood tests. During the psychiatric examination the patient was cooperative, well groomed, oriented and with a euthymic mood. No evidence of a formal thought, language and mood disturbance or of other psychiatric symptoms was detected. She never reported epileptic disorders. GN was treated with donepezil (5 mg/die for the first month, and then 10 mg/die for other 5 months) with relieve from the mild memory impairment but not from the HFF, which persisted substantially unchanged until the second follow-up (six months after discharge) and then gradually abated until spontaneous remission.\nA positive family history for dementia was reported, as GN’s mother had an early-onset AD. The presence of mild episodic memory deficits and the familiarity for dementia, suggested to further evaluating the possibility of a prodromal stage of AD. Therefore, additional metabolic and genetic analyses were carried out. There is indeed evidence that early stages of dementia are related to the increase of specific biomarkers in the cerebrospinal fluid (CSF) and to genetic factors. Analysis of the CSF (cells count: 1 element/mm3) was performed to assess the presence of biomarkers with high levels of sensitivity and specificity for amyloid load and tangles in the brain, which are characteristic of dementia. These markers are amyloid β1–42 (Aβ-42) (mean sensitivity: 86%; specificity: 90%), total tau (t-tau) (mean sensitivity: 81%; specificity: 90%), and phosphorylated-tau (p-tau) (mean sensitivity: 80%; specificity: 92%). Concentrations of the Aβ-42 were significantly reduced (298.40 pg/ml; normal values > 500 pg/ml), whereas concentrations of the t-tau were in the normal range (207 pg/ml; n.v. < 450 pg/ml) and concentrations of the p-tau were slightly increased (61.6 pg/ml; n.v. < 61 pg/ml). As far as the genetic factors related to dementia is concerned, GN has been found to carry the ε2–ε3 alleles of the apolipoprotein E (APOE), whereas higher risk to develop dementia and AD has been found in carriers of the ε4 allele of the (APOE) genotype. In consideration of the clinical history and of the assessment results, GN was diagnosed with atypical AD.\nThe neuropsychological examination took place during the first week after admittance, while the fMRI experiment and SPECT scan were performed three weeks after admittance. |
A 27-year-old male presented to urology clinic with the chief complaint of persistent feelings of impending orgasm. He reported a sensation similar, but not identical, to sexual arousal that did not occur in the setting of sexual thoughts or desire. During these episodes his penis would be extremely sensitive. With manual stimulation he would achieve orgasm, which alleviated the arousal for only a short time after which the symptoms would return. This had become quite bothersome to him.\nThe onset of symptoms was sudden. This was associated with a change of bowel habits with a sensation of fecal urgency and inability to have a bowel movement without straining. He also complained of an ache in the area of his coccyx. The need to strain resolved with fiber supplementation and did not return, but the fecal urgency persisted. His arousal symptoms persisted as well. He denied any urinary frequency, hesitancy, or urgency. The patient reported normal erections and ejaculation.\nHis past medical history was significant intermittent neck pain and chronic back pain from a herniated disk at L5-S1, for male pattern baldness, for which he was taking Propecia, and Peyronie's Disease for which he had seen an urologist in the past, but had declined treatment because he was not sexually active. Past surgical history was significant for appendectomy.\nGenitourinary exam revealed a circumcised phallus with a 1.5 cm plaque on the dorsum of the penis at the base, otherwise the penile and perineal exam were normal. There was a grade 3 varicocele on the left. No inguinal hernias were present. Digital rectal exam revealed good sphincter tone and a small, smooth, nontender prostate.\nThe patient was referred to an orthopedic spine surgeon and MRI of the brain and cervical, thoracic, and lumbosacral spinal cord was obtained. The MRI showed the previously diagnosed L5-S1 herniation which was stable and a synovial cyst at L4-5, but no other cysts or abnormalities that would explain the symptoms. He began physical therapy with resolution of his back pain. His arousal, however, persisted.\nHaving been unsuccessful in finding any obvious neurologic abnormality, the patient was started on paroxetine 20 mg p.o. daily. On follow-up, the patient reported complete resolution of his symptoms. He has since continued on paroxetine without return of symptoms for the past eight years. He has also seen significant improvement in his anxiety level as a result of the medication. |
A 19-year-old male presented to the emergency room as a level 1 trauma with a self-inflicted GSW to the abdomen. The patient was attempting to put his 380 caliber handgun on safety when it discharged. On physical exam, the patient's abdomen was rigid and tender with a presumed entry point in the periumbilical area () and a presumed exit point in the left posterior superior buttock (). No other injuries were present. The patient's measurements included a height of 165 cm and a weight of 60.1 kg (BMI 22.1). The vital signs upon arrival were BP 132/66 mmHg, pulse 106 bpm, and GCS 15/15. The patient's blood pressure decreased slightly to 101/45 mmHg; however, this corrected with intravenous (IV) fluids.\nComputed tomography (CT) was performed and showed a mild amount of free fluid and air in the abdomen and pelvis (). There was no evidence of great vessel injury. Given the known trajectory of the bullet and lack of severity in the CT findings, diagnostic laparoscopy was pursued with readiness to convert to laparotomy if necessary.\nAn initial periumbilical incision was made, and the abdomen was entered through a 5 mm port utilizing the Optiview technique. Pneumoperitoneum was obtained to 15 mmHg. Upon laparoscopic entrance, a small amount of blood and stool was noted in the left lower quadrant (). Additional ports were placed in the left lower quadrant (5 mm port) and right upper quadrant (11 mm port), both using the same optical trocar insertion method. Initial inspection of the sigmoid colon revealed a perforation with minimal contamination from stool spillage (). This was cleared and washed out followed by intracorporeal repair using 2-0 V-Loc™ suture in a running fashion done in 2 layers (). A second sigmoid perforation was found in the 4-5 cm distal to the first perforation as was repaired in a similar fashion (Figures and ). The sigmoid was followed systematically to the pelvis, and a rectal wall laceration was noted (). It was found to penetrate the serosa and muscle layer but lack mucosal involvement. This was again repaired using 2-0 V-Loc™ suture in 2 layers (). After advancing down the rectum, a laceration was noted in the peritoneum over where the bladder was located (). Of note, preoperative urinary catheterization yielded hematuria and this laparoscopic finding confirmed bladder injury. The on-call urologist was contacted intraoperatively, and it was agreed upon for the primary surgeon to repair it in 2 layers with 2-0 V-Loc™ sutures and leave a Foley in place for 2 weeks postoperatively (Figures and ). The colon was then systematically examined proximally including the rest of the sigmoid, descending colon, transverse colon, ascending colon, and cecum with no additional injuries noted. The ileocecal valve was identified; the small bowel was then run from the terminal ileum proximally. Another perforation was found approximately midsmall bowel with an enterotomy measuring less than 1 cm in size (). This was closed in 2 layers using 2-0 V-Loc™ suture (). The rest of the small bowel was run up to the ligament of Treitz with no additional injuries found. Of note, all the mesentery was examined while running the bowels with no significant injuries noted. All solid organs were intact on inspection. After abdominal and pelvic washout was performed, a size 19 French Blake drain was placed in the pelvis exiting through the left lower quadrant port site.\nThe GSW to the abdominal wall itself was examined. The bullet trajectory was angled in such a way that when it penetrated the skin, there was about a 5 cm difference from the presumed skin entry point and peritoneum entry point. Given this tangential path, the wound was not closed due to a low risk of herniation. The tract was thoroughly irrigated. Port sites were closed and dressings were placed. The patient tolerated the procedure well and was transferred to the postanesthesia care unit following extubation.\nPostoperatively, the patient was placed in the surgical intensive care unit. There he progressed well and remained NPO with a NG tube in place. A urologist recommended the Foley catheter remains in place for 7 to 10 days with a cystogram done prior to removal. The patient was started on ertapenem upon his arrival and remained on IV antibiotics throughout his admission. Repeat CT with oral, rectal, and IV contrast on POD 5 was negative for any occult injury or contrast leak. Following this, the patient was started on a clear liquid diet with progression to full liquids. The patient was tolerating this well and was having normal bowel movements. He was advanced to a soft diet the following day. The patient's drain remained serosanguineous in nature and was able to be removed. The patient was discharged home on POD 6 with a Foley catheter in place, which was removed by a urologist 5 days later. |
A 54-year-old female patient without any symptoms, admitted to our clinic because of history of CRC in her mother. There was no palpable mass on physical examination, no evidence of anemia and elevation of tumor markers in laboratory parameters. In colonoscopy, we have seen ulcerating tumor approximately 15th cm from the anal verge []. Multiple biopsies were taken and pathological examination revealed moderately differentiated adenocarcinoma. A computed tomography (CT) scan of the abdomen showed a huge mass (10 cm × 9 cm) in the pelvis and bowel wall thickening in left colon []. The wall of the cystic mass contained areas of calcification and there was a 9-mm area of calcification in the mass which is looking like an ossification area. There was no relationship to the adjacent tissues and the mass had regular margin. This pelvic mass was interpreted as a teratoma by CT scan. Gynecological evaluation with transvaginal ultrasonography showed the cystic mass did not originate from ovarium. We have made preoperative mechanical bowel cleansing and applied antibiotic prophylaxis by given 1 g cefazolin and 500 mg metronidazole intravenous. We used da Vinci® Surgical System for the robotic surgery. After induction, the patient was placed in a modified lithotomy position with the legs apart. A 12-mm trocar was placed through an incision 3-cm right and 2-cm above umbilicus after achievement of pneumoperitoneum. A 30° standard 12-mm robotic endoscope then was inserted through this trocar. The first 8-mm trocar was placed on the right spinoumbilical line at the crossing of mid-clavicular line. The second 8-mm trocar was inserted on the left spinoumbilical line at the crossing of mid-clavicular line. The third 8-mm trocar was inserted in the left upper quadrant. The 12-mm trocar was placed in the right upper quadrant for assistant port. During surgery, we deducted a rectosigmoid junction tumor, which does not exceed serosa of the bowel. In the exploration of the pelvic mass, we have seen the mass had regular margin, ıt was mobile and associated with only the left ovary []. The mass was resected with left ovary and salpinx and led out in endobag []. Robotic anterior resection was performed and colorectal anastomosis was performed as intracorporeal with 31-mm circular stapler []. There was no anastomotic leak was shown that with the air test. Intraoperative frozen section of the mass was defined as metastatic adenocarcinoma. Right salpingo-oophorectomy was performed for the possible bilateral ovarian involvement. Operation time was lasted 165 min, considering 25 min for robotic system set up. Postoperative 2nd day oral feeding was started. The postoperative 5th day the patient was discharged. Eighteen-reactive lymph nodes and one-metastatic lenf node found by as a result of pathological examination. Tumor diameter was 4-cm and there was no tumor proximal, distal, and circumferential radial margin. The tumor is advanced to subseroza layer, but had not infiltrated the serosa. The pelvic mass defined by pathology the isolated colorectal adenocarcinoma metastasis to the only left ovary. |
A 64-year-old male presented to our outpatient clinic for additional care after having undergone surgery for a distal femur fracture elsewhere. He had sustained a Hoffa fracture (Letenneur type II) of his right medial femur condyle after a motor vehicle accident 4 months ago. In this case, the medial approach was used during surgery, and the fragment was reduced and stabilized with two screws and plates. Unfortunately, 4 months later, the patient continued to have pain, and the computed tomography (CT) scan showed nonunion with implant loosening (Fig. ). The range of motion was (flexion/extension) 60°/0°/0°. A revision procedure was planned to provide stable fixation and promote the healing of this intra-articular fracture.\nThe patient was positioned as described above for case 1. The prior medial approach was used for exposure of the nonunion. The nonunion site was debrided after the old fixation instrumentation was removed. Again, at this time, the ununited condylar fragment was noted to be “free-floating” in the knee. The overlying articular cartilage on the fragment was not intact (Fig. ). The fragment was repositioned as described above, and stabilization was achieved with two compression screws placed from the posterior to anterior direction. However, a small gap persisted at the nonunion site, probably due to bone loss; for this reason, two 3.5 mm reconstruction plates were placed as buttress plates for additional stability. The fixation procedure did not cause damage to the articular cartilage. Again, the gap was filled with a cancellous bone graft from the patient's iliac crest.\nThe postoperative radiographs showed the restoration of the femoral condyle surface (Fig. ). The patient was allowed to bear partial weight, and he started range of motion exercises of the knee joint. He was free to walk with full weight-bearing after 3 months. No early complications, such as infection or loss of reduction, were noted after the revision surgery. After 1 year, he ambulated without pain, and his function was excellent, with a range of motion of (flexion/extension) 120°/0°/0° (Fig. ). |
A 26-year-old female presented to the emergency department with chief complaints of acute-onset weakness of all the four limbs for the last 3 days. The weakness was progressive, and she was not able to walk at the time of hospitalization. There was one episode of similar weakness 15 days back, for which she was admitted and treated with potassium supplementation. There was no other significant illness in the past. There was no history of vomiting, diarrhea, fever, or altered behavior. Drug history was not significant. General physical examination revealed mild pallor. Neurological examination revealed motor weakness (power 2/5 in the lower limbs and 3/5 in the upper limbs). Deep tendon reflexes were diminished in all the four limbs. There was no sensory deficit. Rest of the systemic examination was unremarkable. Her laboratory analysis revealed normal anion gap hyperchloremic metabolic acidosis []. In the absence of gastrointestinal loss or diuretic use, we suspect RTA as a probable cause of metabolic acidosis. Urinalysis revealed pH 7.45, which was failed to lower with ammonium chloride test (0.1 g/kg), which further consolidate our diagnosis of distal RTA. Ultrasonography abdomen was unremarkable, and there was no evidence of obstructive uropathy. On further evaluation of distal RTA, her autoimmune profile showed positive antinuclear antibody and anti SS-a/SS-b antibodies in high titer. Thyroid functions were normal, and viral markers were also negative (hepatitis B, C, and HIV). After ruling out the other causes, the possibility of SS was considered. Our patient was admitted to have dry eyes and dry mouth for the last 6 months. Schirmer test was also positive (4 mm in the right eye/5 mm in the left eye). A final diagnosis of hypokalemic paralysis with distal RTA associated with SS was made. She was started on potassium chloride (intravenous) and sodium bicarbonate. After 1 week, her muscle power recovered completely with normalization of all laboratory parameters. At discharge, the patient was put on oral potassium citrate. At 3 months of follow-up, she has been symptom free with no further episode of hypokalemia. |
A 51-year-old male patient with sudden onset of acute right lower abdominal pain and gross hematuria was admitted to the Emergency Department on 1 June 2019. An abdominal examination showed tenderness in the right side of pelvic cavity, as well as a huge mass. The pain radiated to the right side of the lower back, along with percussion pain over the right kidney region. A routine blood test showed normal findings. Urinalysis showed that the red blood cell count was 61.50/µL. Tumor biomarkers, including α-fetoprotein, cancer antigen 125, and carcinoembryonic antigen, were within normal limits. The patient had no medical history of splenectomy or trauma.\nDuring the first check-up, an ultrasonographic examination showed a pelvic mass of 7.0 × 6.5 cm in size and it was situated directly in front of the iliac blood vessels. The mass was grossly well circumscribed by a capsule, and the interior echo of the mass was solid and homogeneous. Color Doppler ultrasonography showed large vessels entering the mass, but tracing the origin of these vessels was difficult because of the influence of intestinal gas (). A calculus in the lower segment of the right ureter was also detected, as well as mild hydronephrosis of the right renal pelvis (pyelic separation of 18 mm, ). The spleen was in the regular site and was normal in size in the left upper abdomen.\nOn the third day of admission, magnetic resonance imaging (MRI) (1.5T; Signa GE Healthcare, Milwaukee, WI, USA) showed a 6.8 × 6.0-cm round mass on the right side of the pelvis (). On the fourth day of admission, a computed tomography (CT) scan showed a well-defined, homogeneous, round mass in the pelvic space (). The mass was approximately 6.7 cm in diameter and adjacent to the right iliac blood vessels. A normal-sized spleen was detected in the left upper abdomen. CTA clearly showed large vessels originating from the mass and ascending to the splenic hilum. CTA also showed the presence of a long vascular pedicle with an artery originating from the splenic artery and a vein that joined with the splenic vein (). On the fifth day of admission, a second check-up by ultrasonographic examination showed that the calculus had disappeared, but mild hydronephrosis remained (pyelic separation of 12 mm, ). On the seventh day of admission, laparoscopy was carried out, and it showed a solid pediculated mass covered with omentum in the right lower abdomen (). The vascular peduncle was isolated and ligated during surgery and the mass was then removed. A definitive histological examination subsequently confirmed accessory splenic tissue ().On the tenth day of admission, a third ultrasonographicic examination showed no hydronephrosis. The patient had an uneventful postoperative course and was discharged 2 days later.\nThe study was approved by the ethics committee of The First Affiliated Hospital of Wannan Medical College on 4 September 2019. The patient provided consent for publication of this case report. All data have been de-identified. |
A 23-year-old healthy female was admitted to our hospital with a new-onset tender bulge in her left groin, associated with subjective fevers and chills. Four days prior to presentation, she noted a sudden protrusion in her left groin with associated pain. Given worsening pain and intermittent nausea, she presented to our emergency room. On admission, physical examination revealed a soft, non-distended abdomen that was tender to palpation in the left lower quadrant. A soft, mobile, exquisitely tender, and non-reducible mass was appreciated in her left groin, concerning for an inguinal hernia. She had an unremarkable medical and gynecologic history and no prior surgical history. Laboratory data were notable for a leukocyte count of 6.7, a hematocrit of 36.9 and a lactate of 1.4 in the setting of normal electrolytes. An outside ultrasound showed an avascular complex cystic lesion measuring 2.1 cm in the left groin. A subsequent computed tomography scan of the abdomen and pelvis revealed a tubular cystic structure along the left inguinal canal, coursing along the expected path of the round ligament, without surrounding fat stranding or herniation of bowel loops. Given her symptoms and the unclear etiology of the hernia, she was admitted to the hospital and taken to the operating room for exploration (Fig. ). In the operating room, a 6-cm dark purple cystic structure was visualized within the sac of an indirect inguinal hernia, travelling along the round ligament down toward the mons pubis (Fig. ). The cystic structure was dissected away from the round ligament and the cyst was opened and noted to contain clear liquid. Ultimately, the patient underwent left indirect inguinal hernia repair, with Gore Bio A absorbable mesh, and excision of a left inguinal hydrocele of the round ligament. Final pathology revealed fibromuscular tissue lined by mesothelium consistent with a hernia sac and a hydrocele with concordant endometriosis. The patient's post-operative course was uneventful and she was discharged home on post-operative Day 1. |
The patient was a 36-year-old healthy man without any past history. An esophageal submucosal tumor was discovered during a periodical medical examination. Endoscopic examination revealed the presence of an esophageal submucosal tumor (4 cm in diameter) in the middle thoracic esophagus (fig. ). A computed tomography scan demonstrated a 4.5 × 3.5 cm-sized tumor in the middle thoracic esophagus (fig. ). Leiomyoma of the esophagus was suspected in the preoperative examination.\nThoracoscopic enucleation in the prone position was planned. Surgery was performed under general anesthesia with a single-lumen endotracheal intubation and a bronchial blocker. A 6-mm Hg artificial pneumothorax using carbon dioxide was induced with left one-lung ventilation.\nFive trocars were used, three of 12 mm and two of 5 mm. One of the 12-mm ports was placed at the 7th intercostal space (ICS) at the middle axillary line for the 30° endoscope. The other 12-mm ports were placed at the 7th and 9th ICS at the posterior axillary line. The 5-mm ports were placed at the 3rd ICS at the middle axillary line and at the 5th ICS at the posterior axillary line (fig. ).\nThe muscularis propria of the esophagus was cut using an electrosurgical knife to expose the capsule of the tumor, and a well-defined solid tumor with an intact capsule was enucleated (fig. ). The divided muscularis propria was closed by interrupted sutures under a thoracoscopic approach.\nPostoperatively, a nasogastric tube was kept in place for 2 days. Contrast radiography of the esophagus on the 4th postoperative day showed intact smooth esophageal mucosa with no leakage. The patient started peroral intake on the 4th postoperative day, and the chest drain tube was removed on the 6th postoperative day. There was no remarkable event after surgery, and the patient was discharged from hospital on the 12th day after surgery.\nThe final pathological diagnosis was leiomyoma of the esophagus without any malignant feature (fig. ). |
A 44-year-old Caucasian male presented to the Emergency department with a 3-day history of increasing abdominal pain, melaena, coffee ground vomit and left hip pain with difficulty in walking. The patient was a known IVDU, with a history of hepatitis C. He previously underwent multiple incision and drainage procedures for left groin abscesses and on one occasion required an emergency fasciectomy for suspected necrotizing fasciitis. The patient subsequently developed chronic osteomyelitis in the left proximal femur with avascular necrosis of the femoral head. Whilst under the care of a specialist bone infection unit he underwent a left Girdlestone resection arthroplasty 1 year prior to this presentation.\nOn admission, the patient was afebrile, but tachycardic. He had epigastric tenderness without guarding. Scrotal oedema and a necrotic patch of skin in the perineum were noted. Digital rectal examination demonstrated an empty rectum with no blood. Examination of the left hip revealed indurated tender skin to the upper outer thigh without discharging sinus. All passive and active movements of the left hip were irritable and reduced. He was unable to weight bear on that side.\nBlood investigations showed elevated inflammatory markers (CRP 213 mg/l and WCC 13.5 × 109/l). The rest of his blood profile and urinalysis were normal. He was commenced on intravenous antibiotic therapy (pipperacillin and tazobactam) for sepsis and proton pump inhibitors for a presumed upper gastro-intestinal bleed and admitted under the care of the physicians.\nThe patient's condition deteriorated and complained of increasing left sided lower abdominal and hip pain. Despite vigorous administration of intravenous fluids, serial lactate levels demonstrated a rapid increase. A computed tomography (CT) scan of the abdomen and pelvis demonstrated a moderate collection involving the musculature surrounding the left hip with gas within the soft tissue, extending to the left ischiorectal fossa and left obturator internus muscle, suggesting a communication between the bowel and hip joint. There were no features of IBD, DD or bowel carcinoma (Fig. ).\nFollowing review by the orthopaedic team, the patient underwent an urgent open hip washout. Surgery was performed under general anaesthesia with the patient in the lateral decubitus position. An anterolateral approach to the hip joint was utilized. On opening the hip capsule, copious amount of faeculant fluid was found inside the joint. A colorectal surgeon was called to theatre and identified a fistula between the hip joint and the bowel through the obturator foramen, from which faeculent fluid could be expressed. The hip joint was washed thoroughly, packed and a negative pressure wound dressing was applied. Fluid cultures taken intra-operatively grew Escherichia coli and Streptococcus anginosus. Following discussion with the microbiologist, the patient was commenced on piperacillin and tazobactam with good response.\nThe patient underwent contrast enhanced magnetic resonance imaging of the pelvis. This revealed areas of gas and an abscess in the upper thigh with extensive oedema and collections surrounding the rectum but due to significant movement artefact a fistula could not be visualized. Forty-eight hours post-operatively, a diagnosis of Fournier's gangrene was made and the patient was taken back to theatre for debridement of the perineal region. An open transverse loop defunctioning colostomy was fashioned to control the faeculent drainage. The hip joint had further washouts and the wound was treated with a negative pressure dressing.\nSubsequent OGD demonstrated oesophagitis, with two small duodenal ulcers that were not actively bleeding. Following surgery, the patient reported significant improvement in his left hip pain over the course of 10 days. He demonstrated a good range of hip movement and was able to weight bear and mobilize pain free. He was discharged home on oral antibiotics for 4 weeks. |
A 2 month old male baby was admitted with fever, cough and cold of 4 days duration and breathing difficulty since the past 2 days. He had bluish discoloration of the extremities. The child was apparently well till 4 days back when he developed cough and cold. He later developed breathing difficulty and poor feeding and was drowsy. There was no history of diarrhoea, convulsions or bleeding from any sites. He was irritable for the past 2 days with excessive crying. There was no history of rash, vomiting, photophobia, ear discharge and diarrhoea. Parents were consanguineous. He had no significant past history. There is history of asthma and cataract in the elderly members of the family. No definite history of cataract could be obtained in young children of the family.He was born normally at home, at term. There is no definite history suggestive of birth asphyxia or resuscitation. He was on breast feeds. He had been immunized for age.\nExamination of the patient revealed a weight of 4.2 kg, heart rate of 176 per minute, respiratory rate 70 per minute. Oxygen saturation was 94 %. He was irritable with respiratory distress. All peripheral pulses were felt, he had pallor, but no cyanosis, oedema, clubbing or lymphadenopathy. The anterior fontanelle was at level. He had many dysmorphic markers, including triangular facies and flat occiput. Red reflex was absent.Eye examination showed lenticular opacities in both eyes. There were no neurocutaneous markers. He was using accessory muscles of respiration. There was no chest wall deformity. He had marked intercostals and sub costal retraction. The trachea was central. There was decreased air entry into lungs with extensive wheeze and crepitations.\nPrecordium was normal. The apex beat was felt in the left 5th intercostals space just medial to the mid clavicular line. The first and second heart sounds were normally heard. There were no murmurs or additional heart sounds heard. Abdomen was soft. The liver was felt 3 cm below the costal margin and spleen 2 cm below the costal margin. There were no other masses felt. Bowel sounds were normally heard. The child was drowsy and lethargic. He was irritable. The tone and power were normal in all four limbs. The deep tendon reflexes were normally elicitable. The spine was normal and there were no signs of meningeal irritation. |
A 16-year-old nulligravid woman was referred to our clinic for the management of a cystic uterine lesion associated with acute worsening of chronic dysmenorrhea. She started menarche at the age of 14 years. Her periods were always regular at an interval of 28 days and associated with severe dysmenorrhea. She was prescribed oral contraceptive pills for dysmenorrhea, with partial relief. Before her referral to our clinic, she presented to the emergency room twice for worsening of pain in the lower quadrant. She underwent CT and ultrasound imaging, which revealed a cyst in the right lateral myometrium, consistent in its echotexture with that of an endometrioma. A CT of the abdomen and pelvis revealed a 4.7 × 3.8-cm subserosal cystic mass on the right side of the uterus, which appeared to be discrete from the endometrium. Pelvic ultrasonography was performed on the same day, which revealed a cystic lesion measuring 5.1 × 3.6 × 4.8 cm, with an echotexture consistent with that of an endometrioma, either within the cornua or in the fallopian tube; however, the exact location was difficult to identify using ultrasound.\nThe patient underwent diagnostic laparoscopy, which confirmed a bulge arising from the right uterine cornua and the presence of a cystic mass, indicative of a Müllerian anomaly. Appendectomy was concurrently performed because of a rigid and hyperemic appearance of the appendix, and minimal endometriosis was fulgurated. She was referred to us for further care and worsening of the pelvic pain after she underwent the abovementioned laparoscopy. We performed laparoscopic excision of the uterine cyst following a procedure similar to that used for case 1. The intraoperative findings () were a 5-cm bulging mass arising from the right cornual region and unremarkable bilateral adnexae. The final pathology report confirmed JCA. The patient experienced proper recovery and pain relief immediately after the laparoscopic resection of the adenomyoma. During follow-up ultrasonography, a cystic area in the left cornual region was noted, concerning to be a remnant of JCA. She now requires continuous oral contraceptive pills for the pelvic pain and suppression of endometriosis.\nsummarizes a selected review of the literature related to JCA. In a report by Takeuchi et al. (), the timing of the onset of severe dysmenorrhea was a mean of 6.6 years after menarche, ranging from 1 to 13 years later. These lesions are commonly 3–4 cm in size, although it may vary from 2 to 8 cm. The most common location is the lateral uterine wall under the round ligament insertion, without interruption of the fallopian tubes, and the uterine cavity is typically unaffected. Coexisting diffuse adenomyosis was identified using MRI in 4 cases of JCA. Five patients in the case series by Takeuchi et al. () had peritoneal endometriosis, similar to our patients, and 1 patient had ovarian endometrioma. The effect of the diagnosis of JCA and surgical resection of these lesions on future reproductive potential has not been well described. Takeuchi et al. () reported that 2 of 3 patients who desired pregnancy had successful live births after resection. |
A 32-year-old male with no significant past medical history presented to our facility with a history of worsening pain and swelling in the medial aspect of his proximal left lower extremity over the past several years. Anteroposterior (AP) and “frog leg” lateral radiographs of the left lower extremity revealed a dense lesion consistent with dense matrix, adjacent to the medial aspect of the femur (). An MRI of the left lower extremity revealed that the lesion was of extraskeletal origin, arising from the adductor magnus, without any involvement of the femur (, ). The solid heterogeneous mass was located in the posterior compartment of the proximal-to-mid thigh and measured 9.6 × 7.3 × 13 cm in the transverse, AP and vertical dimensions, respectively. Multiple tiny hypointense foci, which corresponded to the calcific density noted on the prior plain radiographical studies, were noted centrally. The mass was otherwise of intermediate soft-tissue intensity on T\n1 weighted images and moderately hyperintense to muscle on T\n2 weighted images. Following intravenous administration of gadolinium, moderate enhancement of the mass, excluding the central portion, was observed. Superiorly and inferiorly, the margins of the mass were indefinite, and the mass demonstrated peripheral vasculature, which was most prominent at the cephalad and caudad margins. The mass was centred within the adductor magnus muscle fibres that were displaced around the mass. Anterolaterally, the mass was very closely approximated to the posterior cortex of the femur and the fascial margins that separated the quadriceps and the posterior compartments (Supplementary videos). No obvious signal abnormality was noted within the femur to indicate invasion or primary osseous origin. Post-excision pathology confirmed the presence of mesenchymal chondrosarcoma.\nIn the interval, surveillance imaging for metastatic disease with CT scan of the chest, abdomen and pelvis was performed. At 18 months, multiple bilateral non-calcified pulmonary nodules concerning for metastasis were identified (). The most prominent pulmonary nodule was present within the posterior right base, measuring 2.3 cm in the greatest dimension and abutting the pleura (). With regard to the abdominal and pelvic series, a new right adrenal lesion appearing as a hypodense pedunculated mass measuring 1.6 cm in the greatest dimension was noted. There was no evidence of osseous metastases. |
A 4-month-old female infant was referred to the Department of Pediatrics with a history of persistent chest indrawing since birth. She was born at full term by cesarean section, cried immediately after birth, and had a birth weight of 2700 g. She was found to have respiratory distress with chest indrawing on the first day of life and required admission in the neonatal intensive care unit for 2 weeks and needed supplemental oxygen. She was reported to have persistent chest indrawing since then. She had two episodes of respiratory infection subsequently with worsening chest indrawing, which were treated with oral medications. There was no history of seizure in the past or family history of skin disorders. The child was referred to us at 4 months of age for the evaluation of unresolved chest indrawing. On examination, she weighed 4320 g with length of 54 cm and head circumference of 36 cm. Respiratory rate was 70/minute. Other vital signs were normal. Diffuse hypopigmentation, mainly streaks and few whorls on the skin were found on the left side of the chest, abdomen, and upper and lower extremities [Figure and ]. Consultation with dermatologist confirmed these skin lesions as HI. Respiratory system examination revealed bilateral subcostal retractions with no added sounds on auscultation. Other systemic examination was normal. Chest radiograph was normal []. Echocardiography revealed patent foramen ovale, mild pulmonary artery hypertension, and normal biventricular systolic functions. Gastroesophageal nuclear scan was negative for gastroesophageal reflux. Neurosonogram was normal. As no etiology could be identified for persistent chest indrawing, computed tomography (CT) scan thorax was ordered. CT thorax revealed hypoplasia of the inferior lingular and posterior segment of the left upper lobe [Figure and ]. The child was treated symptomatically for viral superinfection and discharged with an advice to have close follow-up. The cytogenetic studies could not be carried out due to financial constraints. |
In 2016, a 30-year-old woman with a 3rd relapse of the left orbital HPC was presented to our Tumor Board. She had received her first treatment from November 1999 to March 2001, which was incomplete surgical resection of the tumor, histologically reported as a benign HPC. Surgery was followed by RT, 60.0 Gy in 30 Fx.\nIn 2009 the tumor recurred in the same place, and the patient was treated with Cyberknife SRT, 24 Gy in 3 Fx. After 6 months, the patient lost the vision in her left eye, though it was a complete response. In 2013 another recurrence occurred. The patient again received SRT with 30 Gy in 6 Fx.\nIn April 2016, the patient came to our hospital with a local unresectable 3rd tumor relapse (shown in Fig. ).\nThe treatment options were limited due to the unresectability and benign nature of the tumor. Because no MRI indications of radiation brain tissue necrosis were reported and there was high conformity with previous stereotactic RT, long-term disease history, and gaps between RT courses, we decided to irradiate the patient again. The case was discussed at the Tumor Board, and the Institutional Ethical Committee approved the treatment strategy.\nThe 4th course of irradiation was done in May to June 2016. A dose of 60 Gy with 2.0 Gy/Fx was delivered via fixed horizontal spot-scanning proton therapy (PT) in a seated position supported with image guidance with built-in CBCT []. Standard immobilization equipment (thermoplastic mask) was used to ensure the reproduction of the patient position.\nMaximum high conformity and temporal lobe sparing were chosen as the main goals of treatment planning. The patient was blind in her left eye, so we did not need to spare the left optical nerve, which was wholly involved in the tumor, but the other goal was to reduce the dose to the chiasma and contralateral optical nerve as much as possible. GTV was delineated based on MRI and CT co-registration. The planning volume was done by adding a 3-mm margin to GTV. The GTV volume was 8.2 cm3, and the final PTV was 19.4 cm3.\nThe maximum dose to the PTV was 112.9%, and the minimum dose was 75.9%. The V95 (volume receiving 95% of the prescribed dose) was 92.4%, and D95 (dose to 95% of volume) was 56.3 GyRBE (relative biological efficacy = 1.1). The dose coverage was compromised to spare the temporal lobe. The right eyeball and right lens received 0 GyRBE. The dose to the left temporal lobe was 56.5 GyRBE (maximum dose), and to the right temporal lobe, the dose was 0 Gy (maximum dose). The maximum brainstem dose was 16.1 GyRBE, the maximum chiasma dose was 29.8 GyRBE, and the maximum dose to the right optical nerve was 7.8 GyRBE. The mean dose to the hypophysis was 3.0 GyRBE, and the midbrain dose was 1.0 GyRBE. We made a two-field plan with a perpendicular setup as the shortest way to the tumor. The PT dose distribution is shown in Figure .\nThe partial response (tumor shrinkage of 79.3%, from 8.2 to 1.7 сm3) was registered after 12 months. After 24 months, the next MRI reported a small (2.8 cm3) lesion in front of the left temporal lobe with peripheral contrast enhancement, suspected to be a radiation injury. 18F-tyrosine PET/CT confirmed the adverse event. A combination of steroids and bevacizumab (BVZ), which is known for curing radiation necrosis, was administered as a treatment []. In total, the patient received 12 cycles of BVZ, after which the necrosis was finally reported as successfully cured.\nIn August 2019, another relapse at the same location was reported without signs of brain damage (shown in Fig. ). We proposed a combined treatment strategy for the patient: PT with BVZ 5 mg/kg every 2 weeks concurrently. The 5th course of radiation therapy was done in August to September 2019. We chose a total dose of 54.0 GyRBE in 30 Fx. The main planning goals were high conformity and normal tissue sparing. The GTV was delineated with MRI and PET co-registration. The final PTV was 12.9 cm3.\nThe maximum dose to the PTV was 112.2%, and the minimum dose was 86.0%. The V95 was 94.4%, and D95 was 53.7 GyRBE. The target coverage was compromised to reduce the dose to brain tissue (temporal lobe). The right eyeball and right lens again received absolutely no dose. The left temporal lobe got 53.7 GyRBE (maximum dose), and the dose given to the opposite temporal lobe was 12.8 GyRBE (maximum dose). The maximum brainstem dose was 21.2 GyRBE, the maximum chiasma dose was 0.5 GyRBE, and the maximum dose to the right visual nerve was 14.3 GyRBE. The mean dose to the hypophysis was 14.9 GyRBE, and the midbrain dose was 1.3 GyRBE. Compared to our first PT plan, doses to organs at risk were higher, but this is the result of the field's overlap-sparing strategy. The results of the dosimetry are shown in Figure .\nAfter the treatment, 9 months later, another follow-up MRI (June 2020) showed promising results with tumor shrinkage to 0.4 cm3, without evidence of radionecrosis or any other adverse effects (shown in Fig. ).\nIn August 2020, 1 year after the last treatment, the patient unexpectedly died. The emergency CT scans (shown in Fig. ) showed a brain abscess in the left hemisphere, confirmed later by the pathology report. Officially, the adverse event was not related to the irradiation, as it has never been associated with post-RT brain toxicity in the literature. Nevertheless, we can assume that this situation is an indirect treatment side effect. The patient had persistent chronic sinusitis, and the skull base bones exceeded their dose tolerance, which could potentially be open gates to the brain infection.\nThe patient worked as a nurse until her death and did not demonstrate significant neurocognitive dysfunction. As shown in Table , the cumulative doses to the organs at risk were exceeded. |
A 49-year old man with history of psoriasis, on methotrexate for 3 years, presented with microscopic hematuria. The patient was asymptomatic otherwise (ECOG Performance status 0). His methotrexate treatment was held and an ultrasound of the abdomen and pelvis showed a urinary bladder mass. Further evaluation with a cystoscopy disclosed a tumor in the dome of the bladder, but no other mucosal abnormalities on inspection. Pathologic evaluation of a biopsy specimen of the tumor was consistent with mucinous adenocarcinoma (). Immunohistochemistry (IHC) was positive for cytokeratin 20 (CK20) and CDX2 and negative for CK7 and GATA3. No urothelial carcinoma in situ was observed. Staging with a CT scan of chest abdomen and pelvis showed an exophytic lobulated tumor mass with coarse calcifications in the dome of the bladder extending superiorly. The mass measured 3.6 cm by 2.8 cm by 6.2 cm in diameter (). A right enlarged pelvic lymph node with a diameter of 2 cm was also present () but no extension of the disease beyond the pelvis was observed. No evidence of any other primary was seen in the CT scans. A colonoscopy was negative. After multidisciplinary consultation and given the local extent of the disease, a decision was made to proceed with neo-adjuvant chemotherapy. The patient received three cycles of gemcitabine 1250 mg/m2 on day 1 and 8 and cisplatin 75 mg/m2 of a 21-day cycle which he tolerated well, with only grade 1 fatigue. Six weeks after completion of neo-adjuvant chemotherapy, he underwent a partial cystectomy with en bloc resection of the umbilicus and pelvic lymph node dissection. The surgical approach was suprapubic with dissection of the umbilicus, medial rectus muscle fibers, urachus and mobilization of the bladder circumferentially. The tumor was felt in the dome and dissected with large margins through a suprapubic cystostomy, together with the urachus and umbilicus in one specimen. The lymph node dissection started from the node of Cloquet and carried proximally to the bifurcation of the common iliac vessels. External iliac nodes were dissected en bloc with internal iliac nodes and the nodes of the obturator fossa. Surgical pathology disclosed a well-differentiated urachal adenocarcinoma invading perivesical tissue. The enlarged lymph node, measuring still 2 cm in gross pathology, was positive for metastatic adenocarcinoma but 10 additional lymph nodes resected were negative. Thus, the overall TNM stage was ypT3ypN1cM0. Margins of resection were all negative. A post-operative CT scan showed no abnormal findings. Post-operatively the patient received three additional cycles of the same gemcitabine and cisplatin regimen, starting 10 weeks post-operatively, with excellent tolerance. He is followed expectantly since, with cystoscopies and yearly scans and remains free of recurrence more than five years from his diagnosis. He has resumed methotrexate therapy with good control of psoriasis. |
A 37-year-old woman was admitted due to muscle weakness in her left thumb and a mass in her left forearm. The patient reported having had surgery, the details of which she could not remember, on her left wrist when she was a child. Since then, she had developed thumb abduction weakness and experienced dysesthesia. Physical examination confirmed the 3 cm post-surgical incision wound alongside the right palmer side of the thenar crease. Severe atrophy of the thenar muscle and a 4.5×10 cm mass on the palmar side of the distal forearm was observed. The left thumb, index, and middle fingers had hypoesthesia. Tinel’s sign test was positive on the carpal tunnel. There was a weakness of thumb opposition.\nSimple radiography showed an increased shading of soft tissues. An electromyogram test indicated CTS of a severe degree in the AAEM classification. An MRI was performed to confirm the mass properties. In the sagittal and coronal section of the MRI scan, ‘fusiform swelling’ of the median nerve was observed. In addition, in the axial T1-weighted image at the distal forearm level, a nerve fascicle with low signal intensity was located eccentrically inside the mass, and the fat tissue with high signal intensity protruded from the nerve and expanded to adjacent muscles ().\nSurgical treatment was determined under the diagnosis of severe CTS caused by a mass in the median nerve resulting in a disorder of thumb opposition. A 14-cm zigzag longitudinal incision was made alongside the mass, and then peeled to expose the palmar fascia. The palmaris longus tendon was dissected up to the midpalmar portion, while leaving the palmar aponeurosis attached for the longest length possible. After dissecting the subcutaneous tissues, the mass was identified distally along the normal median nerve in the proximal region. In the area of carpal tunnel, we made an additional curved incision parallel to the thenar crease. The transverse carpal ligament was carefully released along the ulnar border, avoiding damage to the median nerve and its recurrent branch. Care should be taken to avoid injury to the superficial palmar artery arch. Then, an internal neurolysis and debulking operation was performed to remove the yellow tumor tissue as much as possible by peeling off the mass while paying special attention not to damage nerve fascicles. Subsequently, the palmaris longus tendon was extruded through the subcutaneous tunnel to the radial side of the metacarpophalangeal joint of the thumb and sutured to the abductor pollicis brevis attachment (). While the thumb was abducted, the first metacarpal and the metacarpal bone were fixed with Kirschner wire and splint was applied. The final histological examination of the resected tumor tissue confirmed the diagnosis of LFH. After the surgery, the patient wore a thumb splint for 4 weeks and a brace for 4 weeks. The Kirschner wire was removed 8 weeks after the surgery. No increase in the size of the mass showed 10 months after the surgery. The patient said that thumb abduction was improved greatly after the operation, however, the hypoesthesia of the thumb and index finger persisted. |
A 47 year-old barber lady with three-year history of paroxysmal atrial fibrillation was admitted on 2 June 2013 for recent incremental attacks of palpitation. Physical examination and laboratory tests were all unremarkable. On 4 June 2013, the electrophysiological study using EnSiteTM VelocityTM (St. Jude Medical One St. Jude Medical Drive, St. Paul, MN 55117 USA) through the transseptal approach confirmed that the paroxysmal atrial fibrillation was originating from the right superior and inferior pulmonary veins. Subsequently, 20–30 Watts (45° Celsius for 30 to 40 s) of radiofrequency energy was applied to atrial locations of the four pulmonary veins utilizing a 7 F quadripolar irrigating ablation catheter. The successful ablation was confirmed by complete antegrade and retrograde electrophysiological study using incremental pacing and extrastimulus technique during isoproterenol administration. The patient was discharged six days later with uneventful course until 10 September 2013, a febrile episode associated with productive cough and mild dyspnea happened. Right lower lobe pneumonia was found by chest roentgenography and computed tomography. The patient was discharged in improved condition after antibiotic treatment for two weeks.\nHemoptysis and chest discomfort occurred 23 November 2013. An emergent chest roentgenogram revealed right lower lung consolidation. The bronchoscopic study was unremarkable. A cardiac computed tomographic angiogram was performed on the presumption of iatrogenic pulmonary vein injury and revealed right inferior pulmonary vein occlusion on 28 November 2013 (Fig. and ). Because of the totally occluded pulmonary vein, stenting was aborted due to high technical demand, high re-stenosis rate, and need of almost life-long anticoagulant therapy. On 10 December 2013, six months since the radiofrequency ablation of the paroxysmal atrial fibrillation, thoracoscopic right lower lobectomy was performed with four-port technique. Severe inflammatory adhesion of the right pulmonary hilar region and totally fibrotic right inferior pulmonary vein were noted. The postoperative course was uneventful and the patient was discharged on eleventh postoperative day. Chest roentgenogram was followed every three months and cardiac computed tomographic angiogram was followed every six months during the follow-up period. The patient has been free from both pulmonary and cardiac events 18 months postoperatively and 24 months after the radiofrequency ablation of the atrial fibrillation.\nOn the microscopic examination, extensive hemorrhagic necrosis with acute and chronic inflammatory cell infiltration, interstitial fibrosis, and vascular thrombosis were noted around the right inferior pulmonary vein. |
A 26-year-old Caucasian female with junctional EB presented to the Dentistry Unit of Bambino Gesù Children's Hospital for alterations in the structure of her teeth. Her medical history included alopecia, acne, lymphedema, erosion of her scalp, and oligomenorrhoea. At the time of presentation, she had blood- and fluid-filled bullae on her hands and feet and there was absence of nails. The intraoral manifestations were the absence or minimum presence of bullae, edematous gingival tissue, and generalized enamel hypoplasia (). We decided to remove the damaged enamel on maxillary incisors using the Er:YAG laser, because the patient complained for esthetic reasons. We used a traditional technique of operatory field isolation with a rubber dam and clamps because her intraoral conditions allowed the use of this tool without negative effects. The parameters used with the Er:YAG laser were 265 mJ and 25 Hz. The contact quartz tip was 80° curved and its diameter was 600 μm, with a fluence of 93 J/cm² (). We managed the laser beam which had selective action over a small amount of damaged enamel. The water cooling avoided intraoperative hypersensitivity and increased the detergent action. The effects of the Er:YAG laser were checked clinically and also with chemical caries detector. The final appearance of working area was cratered and irregular and it improved adhesive retention in reconstructive phase. The use of rotating instruments could have removed both damaged and not damaged enamel; in fact, the Er:YAG laser allowed selective ablation of damaged tissue promoting enamel-dentin decontamination and without any signs of thermal damage. Reconstruction of teeth was carried out using template indexes based on a wax-up to build incisal margins. Vestibular surfaces, interproximal emergence profiles, macro- and microsurface textures, and chromatic features were reproduced with a free hand technique (). During the procedure, the procedure was interrupted few times upon request of the patient. During the 6-month follow-up period, we clinically evaluated the stability of the dental restorations and the patient stated her satisfaction for esthetic results and the absence of postoperative hypersensitivity. |
A 68-year-old Japanese man with a history of distal partial gastrectomy for gastric cancer 10 years earlier was admitted for surgical treatment of intrathoracic esophageal cancer (T3, N2, M0, stage III). He underwent subtotal esophagectomy with three-field lymph node dissection and removal of the remnant stomach with the abdominal lymph nodes. The alimentary continuity was reconstructed with a pedicled jejunal limb through the antethoracic route. When we separated the diaphragm from the esophagus and removed xiphoid surgically to prevent a pedicled jejunal limb injury, the pericardium was opened. Anastomosis of the esophagus and jejunum was carried out instrumentally with a circular stapler. A postoperative enteral contrast examination showed smooth passage and no deformity of the reconstructed jejunum. The patient was discharged about 4 weeks postoperatively. About 6 months after the esophagectomy, the patient was readmitted to our hospital because of abdominal discomfort and vomiting. His vital signs were stable and unremarkable. He was thin, with a body mass index of 18.6 kg/m2. Physical examination revealed that the pedicled jejunum through the antethoracic space was markedly dilated and the abdomen was soft and flat (Fig. ). Laboratory data showed only leukocytosis with no other remarkable findings. A chest roentgenogram revealed an increased cardiothoracic ratio of 70%, and an enteral contrast study showed a bird’s beak deformity. The swallowed barium remained static in the reconstructed jejunum. Computed tomography of the thoracoabdominal region showed the reconstructed jejunum within the pericardium anterior to the heart (Fig. ). We diagnosed the patient with IPDH after esophagectomy and performed an emergency laparotomy. Upon opening the abdominal cavity, we found that the reconstructed jejunum had herniated into the pericardium through an approximately 4-cm-diameter diaphragmatic defect without a hernia sac (Fig. ). The herniation was easily reduced, and the congestion of the incarcerated jejunum resolved. There was no evidence of bowel ischemia. Primary closure of the diaphragmatic defect was accomplished using vertical mattress sutures with 1–0 silk (Fig. ). Because the diaphragm adhered to the pericardium around the diaphragmatic defect, we closed these together. Moreover, to reinforce the closure of the diaphragmatic defect, we used a graft harvested from the rectus abdominis posterior sheath. Interrupted sutures with 3–0 nylon were placed to fix the 8 × 6 cm graft of the rectus abdominis posterior sheath to the diaphragmatic defect, preventing recurrence of the hernia (Fig. ). Postoperatively, an upper gastrointestinal study confirmed free flow of contrast medium from the cervical esophagus into the intra-abdominal jejunum through the pedicled jejunum. The postoperative course was uneventful, and the patient was discharged on the seventh postoperative day. |
A 57-year-old woman came to the office on June 19, 2019 seeking relief from her tinnitus and hearing loss. Her vital signs, including blood pressure and pulse rates, were within normal limits. Her current medical history included severe sinusitis, insomnia, fatigue, and stress.\nOne year ago, patient started to feel fullness in her left ear and experienced a high pitch ringing and vertigo. She felt very congested in her frontal sinuses and both ears, with the left ear being worse. She then developed a very loud high-pitch noise with hearing loss in her left ear at an SUD scale of 8/10. The noise interrupted her sleep at night. Patient went to her ear, nose, and throat (ENT) doctor, an audiogram was performed and showed significant hearing loss in her left ear, registering 40 dB within the range of 250 to 4,000 Hz, with rapid reduction beyond it.\nA main Neuropuncture tinnitus prescription was performed at the first treatment. Patient reported that hearing began to return slowly afterward. After 3 treatments, she reported that she was able to hear a different “layer” of sound (she is a musician) and began to enjoy listening to music again. A total 12 treatments twice a week, including Tinnitus Main and Modified Protocols, were conducted. Patient reported having tremendous “draining” from her ears and the ringing changed from high pitch to a different lower tone with much less intensity with an SUD scale of 4/10. Her relationship with her husband improved because she did not have to constantly ask him to repeat himself in conversation.\nThe patient saw her ENT doctor for her 1 year follow-up in June, 2020. The result of the audiology test showed a significant improvement in hearing of her left ear. She was able to hear low frequency at 250 Hz clearly and high frequencies ranging from 4,000 to 6,000 Hz at 15 dB. Eighteen months follow-up in the office showed the tinnitus was occasionally noticeable; her SUD scale was at 1/10 and her hearing continued to improve. |
A 48-year-old morbidly obese woman with a history of metastatic endometrial carcinoma who was status post-total abdominal hysterectomy and bilateral salpingo-oophorectomy presented for evaluation of a large right-sided goiter found incidentally on computed tomography (CT) performed as part of her metastatic cancer evaluation. CT revealed a 10 cm right thyroid mass with extension deep to the right clavicle and associated tracheal compression (). Positron emission tomography revealed no evidence of metastatic disease but displayed diffuse thyroid hypermetabolism.\nAt initial presentation, she was undergoing chemotherapy but recently discontinued one of the agents due to thrombocytopenia. Initial work-up included thyroid-stimulating hormone and free T4 which were within normal limits. A surgeon performed thyroid ultrasound revealed a large 8.9 × 5.0 × 9.7 cm solid, isoechoic, homogeneous nodule without calcifications encompassing the right thyroid lobe and extending into the substernal/subclavicular location (). Fine needle aspiration of the large nodule revealed colloid and follicular groups consistent with colloid nodule or goiter. Given the large size and associated decreased diagnostic accuracy as well as tracheal deviation, excision was recommended after completion of her course of chemotherapy.\nApproximately 7 months after the initial evaluation, the patient returned to clinic to discuss surgery since recent completion of chemotherapy. Repeat surgeon performed thyroid ultrasound revealed slight growth of the large right nodule, measuring 9.4 × 5.2 × 9.9 cm. Her platelets were 50 000.\nAn experienced, high-volume endocrine surgeon proceeded with right thyroid lobectomy using intraoperative nerve monitoring. On the morning of the case, her platelets were 62 000, an acceptable preoperative level per well-established preoperative guidelines [, ]. As the strap muscles were swept off the right thyroid lobe, extremely large vessels (approximately the size of normal internal jugular veins) were encountered coursing through the thyroid capsule. The middle thyroid vein was at least 1.5 cm in diameter and the superior thyroid artery measured >0.5 cm in diameter. Tedious dissection and mobilization of the lobe proceeded for 2 hours without blood loss. However, the nodule was not able to be elevated or delivered to expose the recurrent laryngeal nerve (RLN). The non-nodular isthmus was divided to allow for additional medial to lateral mobilization. On the first spread with the dissecting instrument in the (typically) avascular pretracheal plane, considerable bleeding was encountered. The isthmus was divided with the ultrasonic dissector, and direct pressure was applied. The bleeding originated from a high-pressure arterial tracheal perforator that was flush with the tracheal surface. There was no vessel or soft tissue to grasp/ligate, and the bleeding was too significant for electrocautery. A second faculty endocrine surgeon scrubbed to assist. Bleeding was slowed and hemostasis eventually achieved using multiple figure-of-eight prolene sutures on the tracheal surface. Bleeding from additional vessels in the thyroid bed and from the right lobe remained problematic but not high volume. The additional mobilization allowed for successful completion of the right thyroid lobectomy. While visual identification of the RLN is standard for the operating surgeon, identification was not possible due to the degree and pace of blood loss. A meticulous capsular dissection technique was used to prevent inadvertent injury to the unidentified RLN. Once the lobe was removed, the vagus nerve was identified and it stimulated appropriately, indicating appropriate RLN function.\nEstimated blood loss was unprecedented at 2 L. Notably, all the bleeding encountered during the case was surgical in nature and all bleeding stopped following lobectomy (no concern for platelet-based oozing). The patient preemptively received a six-pack of platelets in the operating room when significant bleeding was encountered. The patient remained hemodynamically stable, requiring no other blood products. She recovered from surgery without incident.\nPathologic examination of the right thyroid lobe revealed a dominant adenomatous nodule with Hürthle cell and degenerative changes. A proliferation of large arterial and venous structures was seen predominantly at the periphery of the nodule and in the adjacent soft tissue (). The findings were consistent with an AVM. Consultation with an institutional expert on AVM syndromes resulted in recommendation for vascular imaging of the head and neck to evaluate for other possible associated AVMs. Follow-up imaging revealed no additional head or neck AVMs. |
An 81-year-old male presented for consideration of vertebral augmentation due to diagnosis of stage IV, metastatic prostate adenocarcinoma, and worsening back pain. Lupron therapy was initiated at diagnosis four months prior. Docetaxel treatment was planned for six cycles but was subsequently stopped after the first cycle secondary to side effects. No radiation therapy was previously given. PSA level was 120.73 at diagnosis and 0.6 before radiofrequency ablation.\nAt the first appointment, the patient reported mild back pain and required a walker but was able to ambulate without difficulty. He did have pain upon palpation of the thoracolumbar junctional level. He did not have any neurologic deficit at presentation. Computed tomography (CT) scans showed 40% compression deformity of T12. Magnetic resonance imaging (MRI) showed pathologic involvement of T12 and L1 and metastatic involvement of the epidural component, resulting in 40% spinal canal stenosis (Figure ). At this time, vertebral augmentation was recommended although it was believed the epidural component would not be addressed and Radiation Oncology would need to be consulted. In a short period of two months, the patient’s condition deteriorated where he was wheelchair bound due to severe pain, not controlled with NSAIDS or opioids. In addition, repeat studies showed further tumor infiltration involving T11, prompting augmentation of T11, in addition to T12 and L1.\nThe procedure was performed under monitored anesthesia care (MAC) and fluoroscopic guided imaging. Under this image guidance, 10-gauge introducer needles were advanced into the T11, T12, and L1 vertebral levels using a bilateral transpedicular approach (Figure ). A drill and osteotome were used to create cavities at the anterior aspect of the vertebral bodies. Bilateral 17-gauge bipolar radiofrequency probes were advanced into the vertebral cavities and simultaneous application of radiofrequency energy was performed as part of the protocol for volumetric ablation of the vertebral bodies. These were done in serial at T11, T12, and L1 vertebral bodies for approximately 15 minutes for each level. Lastly, methylmethacrylate was injected into the vertebral bodies of T11, T12, and L1 (Figure ) for vertebral stability. No complications occurred during the surgery and the patient was discharged the same day.\nThe patient reported no pain at the three-week follow-up and he was able to ambulate without assistance and continued to increase daily activities. He also no longer required any pain medication. He continued to be pain-free at the eight-week follow-up and repeat MRI showed stable vertebral changes and complete resolution of epidural disease at the T12 and L1 level (Figure ). At nine-months post-op, the patient still had no pain and returned back to his normal activities. |
A 51-year-old female presented to the department with complaints of chronic fatigue, laboratory findings of microcytic hypochromic anemia, and hemoglobin level of 66 g/l. The patient neither presented with additional GI complaints nor had signs of GI hemorrhage. The esophago-gastro-duodenoscopy (EGD) revealed a pedunculated polyp in the descending part of the duodenum with a small area of superficial ulcerated mucosa (Figure ). The endoscopic biopsy showed duodenal mucosal epithelium with minor signs of dysplasia, solitary mucous glands, eosinophilic inflammatory infiltrate, and granulation tissue proliferation. This result neither suggested epithelial origin, nor could rule out malignant origin and the patient was scheduled for endoscopic ultrasound and CT.\nEndoscopic ultrasound revealed a lesion in the second sonographic layer of the duodenal wall without distinct margin and a heterogenous isoechoic echo pattern (Figure ).\nThe CT confirmed pedunculated polyp sized 51 x 27 x 22 mm in the horizontal and descending part of duodenum with peripheral enhancing of 18-20 HU and hypodense core (Figure ). No abdominal lymphadenopathy was found.\nThe patient had significant comorbidities such as ischemic heart disease with stenting of two coronary branches, hypertension, and chronic obstructive pulmonary disease. Risk factors include smoking over 40 years, uncontrolled obesity with body mass index (BMI) of 31.0. The patient was scheduled for elective surgery, but due to the COVID-19 pandemics and the cancelation of elective surgical procedures, the treatment was postponed.\nThe patient failed to show up at appointments and sought medical attention eight months later with the progression of the initial symptoms and pronounced fatigability. Furthermore, the lack of physical activity led to an increase of the body weight and BMI to 33.7. The laboratory test confirmed again anemia and hemoglobin level 91 g/l. EGD and CT were repeated and demonstrated growth of the tumor which doubled its size to 85 x 30 x 28 mm. EUS showed intramural cystic transformation. Additional fluorodeoxyglucose (FDG)-positron emission tomography (PET) was performed, which did not establish increased metabolic activity of the lesion. Elective surgery was rescheduled.\nThe patient underwent laparotomy, duodenotomy, and excision of the polyp. The defect was closed with hand-sewn Heineke-Mikulicz duodenoplasty. No postoperative adverse effects and complications were registered.\nThe gross pathological description was of a lesion of the duodenum with dimensions 55 x 40 x 30 mm with irregular, lobulated surface and swollen, thickened wall, in the submucosa there was a rounded mass with light, tan-white color (Figure ).\nHistological examination showed a wall of the duodenum with preserved mucosal layer; in the lamina propria, there was a presence of moderate infiltration of lymphocytes, plasma cells, and single eosinophils with slightly hypertrophic muscularis mucosa (Figure ). In the submucosa, there was a proliferation of small caliber thin-walled blood vessels, elongated spindle-shaped cells with a moderately pronounced infiltration of eosinophils, plasma cells, and lymphocytes with the formation of several lymphoid follicles, edematous granulation-like stroma with lipomatosis, and hypotrophic, reduced muscularis propria. There was no cellular atypia. Immunohistochemistry was performed. Immunostaining for CD117 showed positive expression in mast cells and negative expression in the elongated spindle cells which excluded gastrointestinal stromal tumor (GIST) (Figures -). Immunostaining for smooth muscle actin (SMA) revealed positive expression in the wall of blood vessels and in some of the spindle cells (Figure ). There was no positive expression with antibody against desmin (Figure ). Immunostaining for CD34 showed positive expression in the blood vessels and in the spindle cells (Figure ). |
A 26-year-old man with a history of 3 years of excessive alcohol intake was admitted to the emergency department for 48 h of abdominal pain, mainly in the left hypochondrium associated with fever and vomiting. He had a history of 1 year of intermittent abdominal pain that intensified over the last month, which led him to stop alcohol consumption. He was hemodynamically stable upon admission. Laboratory test showed C-reactive protein 176 mg/dl, white cell count 16.7 × 109/L, platelet count 617 × 109/L and serum amylase level 109 U/L. The computed tomography (CT) showed a cystic lesion in the pancreatic tail of 5.7 × 9 cm in size and significant splenomegaly with hypodense areas consistent with a pseudocyst with splenic extension and rupture ().\nDue to patient stability, conservative management was decided. This included analgesics, antibiotics and nutritional optimization. During the first 48 h, the patient was stable, with little pain and without fever. On the fourth day of hospitalization, he developed tachycardia and severe pain. The abdominal examination showed signs of peritoneal irritation and laboratory tests with a white cell count of 41 × 109/L. Ultrasound assessment showed diffuse abdominal free fluid. An emergency laparotomy was performed, finding 1.5 L of dark serohematic and purulent fluid in the peritoneal cavity. The amylase level of this fluid was 3000 U/L. A large pancreatic tail pseudocyst was identified in addition to abundant clots in the left subphrenic space because of a ruptured spleen (). Due to extensive peripancreatic inflammation, dissection was difficult. Splenectomy was performed, extracting the shattered splenic parenchyma (). The amylase level of pancreatic cystic fluid was 13 000 U/L, confirming the diagnosis of a pseudocyst. We performed a double layer hand-sewn gastrocystic anastomosis with silk 2.0. One drain was located close to the anastomosis and two in the splenic bed. The patient had a satisfactory recovery, with drains fluid amylase level in the normal range, and was discharged on the 11th postoperative day. |
A 77-year-old female patient with invasive ductal breast cancer presented with MRONJ of the mandible in April 2014 in our department. After initial diagnosis in November 2000, she developed bone metastases and had a pathological right femoral neck fracture. In February 2001, she received adriamycin and taxotere in a neoadjuvant setting, followed by breast conserving surgery and lymphonodectomy and finally cyclophosphamide, methotrexate, and fluorouracil (CMF) chemotherapy combined with exemestane. Further, she developed recurrent sintering fractures of thoracic and lumbar vertebral bodies caused by severe osteoporosis followed by multiple stabilizing surgeries.\nAs oral antiresorptive therapy, she received bondronate from March 2001 to August 2003, followed by intravenous application until November 2013. This was followed by subcutaneous application of 120 mg DNO monthly from December 2013 to July 2014 and in September 2014 as a single application. In April 2014, she showed exposed oral jawbone in region 38 with a size of 4 × 3 mm and region 37 with a size of 2 × 1 mm at the lingual rim of the mandible. She complained about pain and showed signs of moderate infection (stage 2 after Ruggiero). After 3 days of antibiotic therapy, she reported pain relief. Antibiotics were then continued for 3 weeks. After 3 weeks, no signs of local infection were evident (stage 1). Additionally, local mouth rinses with chlorhexidine 0.1% once a day and green tea were recommended.\nBecause of necessary oncological treatment, DNO was continued in November 2014 with exemastane medication, again. Until 2018, she continued with local mouth rinses. Due to acute pain and signs of boosted infection, sultamicillin short-term therapy was conducted. For a total of nine instances, a short-term antibiotic therapy was necessary, however, the patient still preferred conservative treatment over surgical therapy.\nIn April 2018, clinically confluent exposed bone of the lower left jaw and a fistula at the right molar region was visible (). The panoramic radiograph revealed a mandibular involvement with diffuse radiopaque areas between radiolucent areas (). For preoperative planning, we performed a 18F-fluorine-PET/CT of the jaw, showing stage 3 after Ruggiero ().\nThe patient was admitted to the hospital and received 3 g of ampicillin/sulbactam three times a day combined with metronidazole 500 mg three times a day. After 3 days, pain was relieved and the patient further denied surgery. After 4 days, antibiotics were applied orally (sultamicillin) and the patient was discharged. Antibiotics were continued for 45 days in total. A follow-up 18F-fluorine PET/CT was performed in September 2018 at clinical stage 1 (). Conservative treatment with local mouth rinses was continued until April 2019 (1 year), when an extraoral fistula appeared at the left mandible. Observation followed until June 2020 and follow-up was paused because of COVID-19 infection risk. So far, the clinical situation was stable with low infection signs and moderate purulent discharge at the extraoral fistula. DNO was paused in November 2019, exemestane continued.\nThe first 18F-fluoride PET/CT in May 2018 showed a substantially increased 18F-fluoride uptake in the regions 38 to 47 of the mandible with a focal gap of 18F-fluoride uptake in region 36 (area of clinically exposed bone) (). CT revealed diffuse medullary sclerosis and cortical thickening with confluent periosteal reaction and focal cortical erosion in the regions 37 to 42. The regions 43 to 47 were only slightly sclerotic without cortical thickening, periosteal ossification, or cortical erosion. Sequestration was not found.\nFollow-up 18F-fluoride PET/CT imaging was performed in November 2018. Whereas no changes in both sclerosis and 18F-fluoride uptake were observed in the regions 38 to 42; the regions 43 to 47 showed a slight reduction of 18F-fluoride uptake and a slightly increasing diffuse medullary sclerosis and periosteal thickening ().\nAt baseline 18F-fluoride PET/CT in May 2018, we measured 906 ± 221 HU in the MRONJ-affected regions 38 to 47, whereas 949 ± 173 HU was measured at follow-up. The mean HU of the healthy jawbone was 232 ± 76 HU at baseline 18F-fluoride PET/CT and 220 ± 63 HU at follow-up 18F-fluoride PET/CT, respectively. Using the sum of all MRONJ-affected regions and healthy bone regions, we calculated a semi-quantitative HU index of 3.9 at the baseline examination and 4.3 at the follow-up examination.\nThe SUV of the MRONJ-affected jawbone was 12.4 ± 1.5 at baseline 18F-fluoride PET/CT and 9.9 ± 1.4 at follow-up. The mean SUV of the healthy jawbone was 2.0 ± 0.3 at baseline examination and 2.0 ± 0.4 at follow-up examination. In PET, the calculated semi-quantitative index was 6.20 at the baseline 18F-fluoride PET/CT and 4.95 at the follow-up 18F-fluoride PET/CT. |
A 44-year-old Japanese woman with a 6-year history of insulin-dependent diabetes mellitus and an 11-year history of central diabetes insipidus presented with a pain in the genital area worsening over 2 weeks, general fatigue, and loss of appetite. Two months earlier, patient underwent a urinary catheter insertion as a management for urinary frequency, but it was removed during the previous hospital stay, a month before her recent hospitalization, for possible urinary infection. She has had frequent hospital admissions (6 times/year) and was hospitalized 3 months before her recent admission because of edema of the pelvic area and lower limbs. The patient developed diabetes mellitus after undergoing total pancreatectomy for nesidioblastosis, a surgical procedure which involved the removal of patient’s pancreas including the spleen and gallbladder. Her sister was also diagnosed with idiopathic central diabetes insipidus; thus, a family etiology was suspected. The patient had a surgery for suspected tongue cancer 2 years ago and was also suspected of non-alcoholic steatohepatitis with episodes of hepatic encephalopathy. Although she was on multiple medications including subcutaneous insulin injections and desmopressin tablets, her glycemic and hydration status were poorly controlled.\nFour days prior to the present admission, she visited a gynecologist for her inguinal pain. No uterine tenderness or exudate was observed, and she was prescribed gentamicin and lidocaine ointments for possible local infection. She developed edema in the pelvic area with loss of appetite, and her home doctor consulted the university department 1 day before the present admission.\nUpon admission, the patient appeared weak but was alert and had low-grade fever (37.4 °C) under a regular use of acetaminophen (1500 mg/day) and diclofenac (75 mg/day). Her blood pressure was not significantly different from previous measurements (88/42 mmHg) but a sinus tachycardia (heart rate 125/min) was noted. She complained of continuous abdominal pain and tenderness in all four quadrants. No abdominal guarding or rigidity was observed, but she had severe edema in the pelvic and bilateral femoral areas without necrotic skin discoloration. Laboratory investigations revealed a white cell count of 16,310/μL with neutrophilia (90.8%), elevated C-reactive protein of 22.18 mg/dL, and no serum sodium or potassium abnormalities. Serum aspartate aminotransferase and alanine aminotransferase were elevated at 466 U/L and 148 U/L, respectively. The patient’s international normalized ratio was high (2.26), but disseminated intravascular coagulation score did not meet the criteria. The patient’s HbA1c level was 8.8%, and blood sugar at admission was 316 mg/dL. She had low serum albumin concentration (1.7 g/dL), elevated serum ammonia concentration (154 μg/dL), and elevated lactate level (10.3 mmol/L). No ketonuria was noted, but significant pyuria was observed.\nThe abdominal ultrasound was unrevealing; thus, an intravenous treatment with ceftriaxone (1 g every 8 h) was initiated empirically after obtaining the blood and urine culture samples. A CT scan performed the following morning revealed the presence of air in the soft tissue of the inguinal and pelvic areas, such as pectineal and psoas major muscles (Fig. ). Immediate infectious and surgical consultations were made, and the antibiotics were changed to meropenem (1 g every 8 h), vancomycin (1 g every 12 h), and clindamycin (600 mg every 8 h). Gram-positive cocci and gram-positive rods were found in the initial blood cultures. In the evening of hospital day 2, a surgical debridement of the extraperitoneal pelvic tissue with colonostomy was performed, and the CT image after the operation suggested a complete resection of the affected tissue. However, hypernatremia (a serum Na concentration of 160 to 170 mEq/L) ensued as the use of nasal desmopressin could not effectively control the patient’s central diabetes insipidus after the operation.\nNo bacteria could be cultured from the debridement tissues (Table ). The blood culture isolates were finally identified as Streptococcus constellatus using superoxide dismutase A sequencing and C. ramosum by 16S ribosomal DNA sequencing []. The minimum inhibitory concentrations (MICs) of various antibiotics were tested using Etest for C. ramosum [] and the broth microdilution method [] except imipenem and meropenem (Etest) for S. constellatus (Table ). These results were interpreted using the Clinical & Laboratory Standards Institute M11-A8 document [] for C. ramosum and M100-S24 document for S. constellatus. Both strains were susceptible to penicillin, meropenem, and clindamycin. Despite the continued use of susceptible antibiotics and intensive care, disseminated intravascular coagulation and pancytopenia developed, and the patient died on hospital day 8. |
A 57-year-old female was presented to the ED with swelling on the left face for eight months as well as unintentional 10 lb weight loss over the same time. Additional symptoms included frequent bouts of acute nervousness and tinnitus. She denied fevers and night sweats and had no pain or erythema associated with the swelling. Initial laboratory results demonstrated normal white blood cell and platelet count. The patient's kidney function and red blood cell count were normal. CT of the neck with intravenous contrast performed in the emergency department showed asymmetric enlargement of the left parotid gland (Figure ). Of note, her submandibular glands were atrophic. The report recommended further evaluation with MRI.\nThe patient was discharged and she saw her primary care provider one week later. An MRI of the face without and with intravenous contrast was ordered and performed two weeks later. It was at this time where her history of Sjogren syndrome was described in the medical record. The MRI revealed a left parotid space mass with ill-defined borders which had decreased T1 and increased T2 signal and homogeneous contrast enhancement with ill-defined margins (Figure ). Diffusion-weighted imaging (DWI) demonstrated significantly restricted diffusion with very low apparent diffusion coefficient (ADC) values (Figure ). The right parotid gland as well as both submandibular glands were atrophic. These findings were highly suggestive of a malignant parotid neoplasm, with the favored diagnosis of primary parotid lymphoma.\nThe patient was referred to otolaryngology where tissue sampling was performed which revealed primary MALT non-Hodgkin lymphoma of the left parotid gland (Figures , ). Once the diagnosis was confirmed, the patient went on to have a whole body fludeoxyglucose (FDG) F-18 positron emission tomography (PET) scan which showed high metabolic activity of the primary parotid neoplasm as well as nodal involvement of level II cervical lymph nodes on the ipsilateral side (Figure ). |
A 55-year-old Caucasian male, with a past medical history significant for tobacco abuse (41 pack-years), presented with shortness of breath accompanied by chest and back pain for two months. Blood workup showed a WBC count of 68,400 cells/µL, with an AEC of 27,360 cells/µL. A computed tomography (CT) pulmonary angiogram was performed, as he was hypoxic, and revealed a 3.6-cm speculated mass within the anterior right upper lobe, partially invading the anterior chest wall. It also revealed mediastinal and hilar adenopathy, an extensive osseous lesion (including compression fracture at T7), and a small pericardial effusion (Figure ). A CT of the abdomen and pelvis with contrast was performed and revealed a diffuse metastatic disease involving the liver, adrenal glands, spleen, and the bones. Magnetic resonance imaging (MRI) of the thoracic spine did not reveal spinal cord compression, but it did show the compression fracture at T7 and multilevel thoracic spondylosis. An MRI of the brain revealed a 5-mm lesion in the left occipital lobe, without edema or mass effect.\nThe hematology-oncology team was consulted for an evaluation of the metastatic disease and the eosinophilia. A core needle biopsy was obtained from a liver lesion and the result came back as poorly differentiated adenocarcinoma of the lung (cytokeratin 7, TTF1, and napsin-A were positive, while cytokeratin 2 and CDX2 were negative). Given his functional status, the decision was made to hold on systemic therapy and start on palliative radiation to the spine for pain control. The plan was to complete radiation sessions and then evaluate his functional status before starting systemic therapy.\nHe continued to have a high WBC count during the admission (Figure ). Therefore, a bone marrow biopsy was performed to rule out a hematologic malignancy and it revealed metastatic adenocarcinoma of the lung with no evidence of a myeloproliferative disorder. The flow cytometry from the bone marrow showed a CD5-positive clonal B-cell population, which was similar to the blood flow cytometry. Blood tests, including tests for Janus kinase 2 (JAK-2), calreticulin (CALR), MPL, BCR-ABL, and platelet-derived growth factor receptor (PDGFRA), were negative. The blood smear showed microcytic anemia with leukocytosis with absolute neutrophilia and eosinophilia. The serum immunoglobin E (IgE) was high at 377 IU/ml, and the tryptase level was low at 1.8 µg/L. Given these findings, his eosinophilia was related to a paraneoplastic process rather than a primary bone marrow disease.\nDuring the following days, the patient completed 13 sessions of radiation without improvement in his functional status, pain, or breathing. The case was discussed with the patient and his family; he decided that he would go with comfort measures, so he was discharged to the hospice facility. |
A healthy looking, 50-year-old Nepali man came to our clinic with a complaint of multiple growths on his scrotum for 15 years. The growths started as a single lesion on the right side of his scrotum, with the gradual appearance of similar lesions on other parts. Several of these lesions coalesced at various places to form large-sized nodules. The condition was mostly asymptomatic with an occasional complaint of itching. There was no history of pain, burning sensation, trauma, ulceration, or discharge. The lesions did not interfere with urination or sexual activities. He was worried because of the increasing size of the growth and hence came to us for advice. He did not give a history of any systemic illness including metabolic, autoimmune, or malignant disorders. There was also no history of a similar complaint in his family.\nOn physical examination, multiple pink to brown nodules ranging in size from 0.5 × 0.5 × 0.5 cm to 3 × 3 × 1 cm involving almost half of his scrotum were noticed (Fig. ). The skin over the nodules was shiny with several yellowish points indicative of underlying calcium deposition. The skin surrounding the nodules, testis, and penis was normal on palpation. The nodules were painless and firm in consistency. On laboratory examinations the following were found to be within normal limits: serum calcium, phosphorus, parathyroid hormone, and vitamin D hormone levels; uric acid; alkaline phosphatase; and lipid profile. Based on clinical features and laboratory reports, a diagnosis of ICCS was made.\nHe was advised a scrotectomy under spinal anesthesia, which he refused. The nodules were excised under local anesthesia in several sittings. The skin was sutured using chromic catgut (4–0). His postoperative period was unremarkable with good cosmetic result and no evidence of recurrence in a 1-year follow-up period. The cut section of nodules showed solid white to yellow homogenous areas. Histopathological examination revealed skin tissue lined by keratinized stratified squamous epithelium. The underlying dermis had areas of fibrosis and calcification (Fig. ). Numerous multinucleated giant cells were also seen (Fig. a and b). An obvious cystic structure was absent. |
A 42-year-old man with cholecystolithiasis had an open cholecystectomy under epidural anesthesia at a local hospital 2 months prior to admission. He weighed 72 kg and was 173 cm tall, and had no significant medical history. During the operation his vital signs were stable and no hypotension was observed. The epidural catheter was inserted via the T7–T8 interspace, and passed upward to the upper thoracic levels. During insertion of the catheter he felt a shooting pain over the right lower leg, which disappeared immediately. Five milliliters of 2% lidocaine was injected as a test dose. Five minutes after the test-dose injection, 15 mL of 2% lidocaine (with adrenaline 1:200,000) was given incrementally, obtaining a T4 sensory level. The operation was uneventful. At the end of surgery, the epidural catheter was removed.\nOn the fifth postoperative day, the patient complained of numbness and a paresis of the right leg, which spread to the left leg. Thereafter he was evaluated by an anesthetist and a neurologist. Neurologic examinations showed that sensory deficit was below the T7 level, muscle power was grade 2/5 over the right lower limb and 4/5 over the left lower limb, deep tendon reflexes of both lower limbs were increased, and the Babinski sign was bilaterally positive. He had bladder incontinence and constipation, and his positional sense of the left leg was impaired. Emergency thoracic computed tomography (CT) ruled out disk herniation, epidural abscess, and epidural hematoma. Both thoracic magnetic resonance imaging (MRI) studies and motor-evoked potentials recorded from the lower limbs were normal. Therefore, transverse myelitis was diagnosed and the patient was administered dexamethasone and γ-globulin. Two months after initiation of therapy, the patient had not yet shown any neurologic improvement, and was transferred to our hospital for further treatment.\nAfter admission the patient was re-examined. MRI of the thoracic spine showed a hyperintense lesion expanding the cord from T6 through T7 on T2-weighted images, and on T1-weighted images there was an isointense lesion expanding the cord from T6 through T7 with ring enhancement after gadolinium diethylenetriaminepentaacetic acid (GDTA) administration (Figure A–C). Results of cerebrospinal fluid (CSF) analysis were normal and the CSF culture did not grow any bacteria. A tuberculin skin test was negative. Chest radiography revealed no abnormality. Therefore, spinal cord infarction (SCI), intramedullary inflamed granuloma, or intramedullary neoplasm was considered to be the cause of paraplegia following epidural anesthesia. The patient was treated with dexamethasone, antiviral medications, and rehabilitation training. These therapies apparently relieved his neurologic symptoms. However, 20 days after administration his sensorimotor functions suddenly deteriorated. Numbness and paresthesia sensation extended up to the T5 levels. Muscle power of the lower limbs decreased to grade 0/5 over the right lower limb and 2/5 over the left lower limb. MRI studies at this time showed an enlargement of the intramedullary lesion (Figure A, B). Because of the patient’s neurologic deterioration and the uncertain diagnosis, complete surgical excision of the intramedullary mass was performed. Histopathologic examination revealed a tuberculous granulomatous lesion (Figure ). Antituberculous treatment was initiated with rifampicin, isoniazid, and pyrazinamide after surgical intervention and was continued for 6 months. At the last follow-up visit, 7 months after laminectomy, his motor and sensory functions had gradually improved over time. He was able to walk with assistance and had regained bladder control.\nParaplegia is a rare but tragic complication following epidural anesthesia. When postoperative paraplegia occurs, the anesthetic technique, surgical procedure, and coexisting disease should all be considered. In this case, the postoperative paraplegia was related to intramedullary spinal tuberculoma. However, we were unable to ascertain the exact cause of paraplegia before histopathologic examination. From clinical symptoms, laboratory tests, and imaging studies, the possible causes of postoperative paraplegia in this patient included acute transverse myelitis, SCI, and intramedullary inflamed granuloma or neoplasm.\nAcute transverse myelitis (ATM) represents a major subset of acute noncompressive myelopathies characterized by acute or subacute dysfunctions of motor, sensory, and autonomic nerves [], with an incidence of 1–4 per 1 million persons per year. Individual sporadic cases of transverse myelitis have been reported following spinal, epidural, or general anesthesia, but the causative agent has not been identified []. Because the clinical symptoms and abnormal gadolinium enhancement of the spinal cord at MRI were noted in our patient, the diagnosis of ATM could not be excluded despite normal CSF analysis.\nSCI is a rare disorder characterized by flaccid paraplegia with absent deep tendon reflexes, but with preserved proprioception and vibration sense. It has been reported that the causes of SCI following epidural anesthesia include compression of the spinal arteries by the volume of epidural solution, systemic hypotension, pre-existing vascular disease, the local vasoconstrictor effect of epinephrine, and possible acute thrombosis of the anterior spinal or radicular arteries []. In this case, introducing an excessive amount of anesthetics into the epidural space and using epinephrine in the local anesthetic solution were possible contributory risk factors. However, the absence of hypotension during the operation, the loss of positional sense in the patient’s left leg, and the characteristics of signal abnormity on MRI examination do not completely support the diagnosis of SCI.\nIntramedullary spinal tuberculoma is a rare form of central nervous system tuberculosis, which occurs usually in young people and in the thoracic spinal cord. Although it frequently presents signs of subacute spinal cord compression, variable clinical manifestations including Brown-Sequard syndrome and episodes of paraplegia have also been reported []. The MRI of intramedullary tuberculomas shows gadolinium ring enhancement, with or without central hyperintensity on T2-weighted images, and gadolinium to isointense rings on T1-weighted images []. In our patient there was an isointense area with cord expansion on T1-weighted images and a hyperintense area on T2-weighted images, with ring enhancement after contrast administration. Unfortunately, the diagnosis of intramedullary spinal tuberculoma was delayed despite the typical imaging features, for several possible reasons. First, the patient had no history of tuberculosis. It has been suggested that intramedullary tuberculomas are almost always associated with active pulmonary tuberculosis []. Second, the tuberculin skin test, chest radiography, and CSF examinations were all normal. Lastly, postoperative paraplegia accidently occurred following epidural anesthesia, and the puncture site was adjacent to the lesion location of intramedullary spinal tuberculoma. Therefore, it was necessary to identify anesthesia-related complications.\nThe optimal treatment of intramedullary tuberculoma remains debatable. Both surgical and medical treatments have been reported to achieve reasonable efficacy in different studies [–]. Medical treatment includes antituberculous chemotherapy and steroid therapy. The addition of corticosteroids with single or multiple tuberculomas resulted in an improved clinical outcome, presumably by reducing edema. For this reason, the neurologic function of our patient was improved temporarily after dexamethasone treatment. Surgery is generally indicated when (1) there is no response to chemotherapy, (2) the diagnosis is in doubt, and (3) there are large lesions with rapid deterioration in neurologic function []. Because of the uncertain diagnosis and deterioration of neurologic status, surgical intervention was performed in our patient. Despite subsequent antituberculous therapy, the patient retained some disability. |
A previously healthy six-year-old female presented with complaint of fevers up to 101 degrees Fahrenheit, as well as dry cough, intermittent periumbilical abdominal pain, and night sweat. She was taken to her primary care physician, who diagnosed the patient with a urinary tract infection and she was sent home with a 5-day course of oral amoxicillin. The patient returned to the emergency room 2 days after completion of the antibiotics with persistent fevers and cough. Urinalysis and complete blood count were unremarkable. A chest radiograph showed perihilar peribronchial thickening without focal consolidation. The patient was discharged home with a presumed diagnosis of atypical pneumonia and given a 5-day course of oral azithromycin. Following completion of the azithromycin, the patient had improvement in her cough but still had persistent fevers, prompting another visit to the emergency department the day after completing her antibiotic course. A repeat chest radiograph, complete blood count, urinalysis, and renal function panel were normal. C-reactive protein was elevated at 6.65 mg/dL (normal < 0.80) and erythrocyte sedimentation rate was elevated at 70 mm/h (normal 0–13). The patient was hospitalized for further evaluation of her fever.\nBlood and urine cultures on the date of admission did not demonstrate bacterial growth. Further investigation into the patient's history did not demonstrate any sick contacts but did have exposure to a family member who had been recently incarcerated. The patient did also have recent contacts with new dogs and kittens prior to the onset of fever, and the patient did suffer a scratch to the chest from one of the kittens which did not require medical care.\nOn hospital day 2, a complete abdominal ultrasound demonstrated an echogenic liver which was slightly enlarged to 11 cm in the craniocaudal dimension at the right midclavicular line. The spleen was also enlarged to 7.8 cm and contained several small hypoechoic foci (Figures and ). A contrast-enhanced CT was then recommended for further evaluation which showed small, poorly defined hypoattenuating lesions that were seen throughout the spleen suspicious for microabscesses (Figures and ). Additionally, small, poorly defined hypoattenuating lesions were seen within the bilateral renal cortices, findings which were also compatible with microabscesses (Figures and ).\nEpstein-Barr virus, cytomegalovirus, toxoplasmosis, HIV, histoplasmosis titers, and purified protein derivative tests were all negative. On hospital day 3, the Bartonella henselae IgG was positive at >1 : 1024, consistent with presence of IgG antibody to Bartonella henselae, suggestive of current or prior infection. Bartonella henselae IgM was also positive at 1 : 128, suggestive of current or recent infection. Given the CT and ultrasound findings, the constellation of findings was consistent with an active Bartonella henselae infection with involvement of the spleen, kidneys, and liver.\nFollowing these results, the patient was treated with oral rifampin 150 mg by mouth twice daily for fourteen days and trimethoprim-sulfamethoxazole 150 mg by mouth twice daily for fourteen days, which was well tolerated. The patient's fever and other symptoms resolved during her hospital stay and she was discharged on continuation of the oral antibiotic regimen. At the time of discharge on hospital day 5, the patient had defervesced and her inflammatory markers had decreased, with a C-reactive protein of 2.61 at the time of discharge. Her subsequent outpatient follow-up in the infectious disease clinic 11 days after discharge demonstrated complete resolution of her symptoms. |
The third case is of a 68-year-old Irish woman who presented to the MMUH in April 2016 with acute laryngitis. She had a background of bipolar affective disorder which had been stable for the past 30 years on monotherapy with lithium. There had been a recent history of lithium toxicity secondary to a deterioration of her renal function, which had been managed at her local psychiatric hospital. After the episode, she had been restarted on a low dose of lithium as well as a low dose of valproate.\nOn presentation to the MMUH she was initially treated jointly by the ear, nose, and throat (ENT) team and medical team and was managed in an ICU environment due to respiratory compromise. She had no oral intake for multiple days. Once stabilized she was transferred to an acute medical ward but an acute onset confusional state with bizarre behavior was noted over a period of 2 days. Due to her psychiatric history the Liaison Psychiatry service was consulted. On review she was severely thought disordered and confused. She was only able to produce a word salad and showed echolalia. She had motor retardation, increased tone, negativism, and posturing on examination. The impression was that she was suffering from acute catatonia. Brain imaging did not reveal acute abnormalities. She was diagnosed as having bipolar I disorder with catatonia as per DSM-5 (Table ).\nAdvice was given to treat her with paliperidone. Her mental state improved slightly as a result, but she remained severely thought disordered and confused for 2 weeks. Eventually, lithium was cautiously reintroduced under close monitoring of her renal function. The reintroduction of lithium was well tolerated and she improved significantly over a 2-week period. At discharge she was no longer thought disordered, she was well orientated, and back to her fully independent baseline. She continues to live independently to date. |
A 47-year-old male was admitted to our institution with a history of intellectual disability for more than 30 years and behavioral abnormalities for the past 3 mo. He had a poor academic performance, having dropped out after completing elementary school. The patient could talk with others in daily life, but he was unable to do housework.\nThree months ago, he underwent an operation for renal calculus, after which he showed progressive delusion (he believed unreasonably that he had a serious disease and was going to die), combined with slow response and speech reduction. At 1 wk before admission, he was unable to answer questions and developed an eating difficulty, incontinence, fever (T ≤ 38.2 °C) and severe weight loss. Chronic diarrhea was absent.\nThe proband is the 4th child of 5 in a non-consanguineous Chinese family. His family history was negative for symptoms related to neurological disorders. He was divorced twice and had no children, and did not have a history of smoking or drinking. The proband’s parents had died and his mother had a history of uremia. All of his four sisters are healthy (Figure ).\nThe patient had dark skin and a poor mental state (Figure ). Neurological examination indicated cervical rigidity and Kernig sign. Deep tendon reflexes and limb muscle strength were normal. Babinski sign was negative. Enlarged Achilles tendons and nodules on the bilateral tibial tubercles were observed (Figure and ). There was no evidence of cataract or xanthomas at other sites such as the eyelids.\nThe laboratory results (Table ) were as follows: Increased leukocyte count, neutrophil ratio and high erythrocyte sedimentation rate. There was also a significant increase in concentration of free fatty acid compared to normal range. In contrast, levels of high-density lipoprotein cholesterol were low. Serum cholesterol, glucose, electrolytes, and adrenocorticotropic hormone (ACTH) levels were all normal, as were the liver function tests. Doppler ultrasound of the urinary system showed multiple left kidney stones, a right kidney cyst, and a hypoechoic area in the bladder which could move with body position (Figure ). Cerebrospinal fluid protein was mildly elevated: 680.4 mg/L (normal range: 150-400 mg/L).\nX-ray of the lower limbs showed soft tissue swelling above the tibial tuberosities bilaterally (Figure ). Sagittal and axial magnetic resonance imaging (MRI) of the right ankle joint showed apparent enlargement of the right Achilles tendon and upper medial malleolus flexor tendon (consisting of low signal intensity on both T1- and T2-weighted images), abnormal thickening of the plantar fat, and a small amount of exudation around the fascia in front of the Achilles tendon (Figure ). The electroencephalogram (EEG) showed increased slow activities than normal and electrophysiological examination found mixed sensorimotor neuropathy of the upper and lower limbs. Cerebral MRI suggested white matter (WM) demyelination and slight cerebral atrophy (Figure and ).\nGenomic DNA was isolated from blood samples of the patient and his four healthy sisters. Mutation screening of all exons and flanking regions was performed on the patient’s sample by targeted sequencing as previously reported[]. Targeted sequencing revealed that the proband had compound heterozygous mutations in the CYP27A1 gene and that his four sisters were mutation carriers (Figure -, and Table ). Both of the variants found are known pathogenic mutations of CTX. The variant in exon 2 (c.435G>T, p.Gly145Gly), a functionally silent nucleotide substitution, has been reported to activate a 5’ splice site, leading to alternative pre-mRNA splicing[]. The other variant (c.1263+1G>A) is a splice site mutation that disrupts normal mRNA splicing and results in exon 7 being skipped in the transcript[]. In Italian and Japanese patients, the variant has been found to cause loss of a heme binding domain, a vital part of hydroxylase, and to damage bile acid synthesis[,]. |
A 27-year-old man was referred to our hospital for lung transplantation evaluation. His history included acute myeloid leukemia at age 20 years, for which he underwent bone marrow transplantation (BMTx) 1 year after the diagnosis with fully matched human leukocyte antigen typing. Three years later, he reported dyspnea on exertion and was diagnosed with bronchiolitis obliterans caused by chronic graft-versus-host disease (Fig. a). He suffered from invasive pulmonary aspergillosis during his treatment with high-dose inhaled steroids, bronchodilators, and antimicrobial drugs. Home oxygen therapy was initiated because of type II respiratory failure. Four years after BMTx, his condition worsened, requiring mechanical ventilation. He was eventually discharged home with a tracheostomy and mechanical ventilation. At the time of referral back to our hospital, his condition was relatively stable, and he could undertake moderate activities of daily living. He was fully committed to his rehabilitation. A multidisciplinary transplantation selection committee at our hospital deemed him suitable for lung transplantation. After obtaining his consent, we enrolled him on Japan’s national lung-transplantation list.\nAfter about a year at home, he developed a right pneumothorax that eventually required thoracotomy. Four months later, he developed a left pneumothorax, which exacerbated carbon dioxide retention. Although the pneumothorax was managed with chest drainage and pleurodesis, the carbon dioxide retention and resulting acidosis were barely manageable with conventional mechanical ventilation, which ultimately necessitated VV-ECMO. ECMO was established via the right internal jugular vein (using a 23-Fr cannula) and the left femoral vein (with a 21-Fr cannula) (Fig. ), after which he was transferred to an ECMO center at Nippon Medical School Hospital to await lung transplantation.\nAbout 2 weeks later, he developed another left hemopneumothorax. Chest tube drainage resulted in a massive hemothorax, necessitating open thoracotomy for hematoma removal and hemostasis. He attained relative stability despite a few septic episodes caused by Bacillus cereus.\nOn day 111 of ECMO, however, he developed hemoptysis. Computed tomography revealed intrapulmonary bleeding in the right lung (Fig. ), requiring catheter embolization of the right bronchial artery. On day 129 of ECMO, there were signs of acute inflammation, including an elevated C-reactive protein level (25 mg/dL). Although blood cultures remained negative, the ECMO cannulas were moved to the left jugular vein (19-Fr cannula) and right femoral vein (23-Fr cannula) (Fig. b). On day 136 on ECMO, with his general condition continuing to deteriorate, a suitable donor was identified, and the patient was readmitted to our hospital. During the pre-transplant waiting time on VV-ECMO, he had been treated with continuous unfractionated heparin. His activated partial thromboplastin time (APTT) was maintained at around 40–60 s. When bleeding occurred, the APTT was controlled at around 35–40 s. His activated clotting time was also monitored and maintained within the range of 160–200 s. After stabilization, he could eat and drink to some extent but still required mechanical ventilation (Fig. a). His rehabilitation was limited because a double-lumen cannula for VV-ECMO was not available in Japan.\nAt admission, enhanced computed tomography had revealed limited access to the patient’s major veins because of previous cannulation. We therefore consulted with a cardiologist, who inserted a Swan–Ganz catheter through the left subclavian vein (chosen because it appeared to be the only accessible major vein) preoperatively.\nBilateral lung transplantation began with continuous VV-ECMO. Severe adhesions in both thoraces made the procedure challenging. In addition to his history of bilateral thoracotomy, his right lung was inflamed and hard, making it impossible to collapse and difficult to mobilize. After dissecting as much of the intrathoracic adhesions as possible, we switched to veno-arterial ECMO by cannulating the ascending aorta, using two already existing venous cannulas for drainage. Standard pneumonectomy and graft anastomosis were performed with a reasonable ischemia time (left lung: total ischemia 8 h 21 min, warm ischemia 42 min; right lung: total ischemia 11 h 50 min, warm ischemia 52 min). Total blood loss was 34,930 mL.\nAfter reperfusion of the bilateral lungs, we planned to maintain central veno-arterial ECMO because of the massive bleeding and transfusion, the long-term ECMO, and the patient’s unstable preoperative condition. However, the diffuse intrathoracic bleeding was so difficult to control that a half-dose of protamine was administered. Although it resulted in reasonable hemostasis, the membranous oxygenator clotted, with suddenly decreased ECMO flow. Fortunately, the patient’s vital signs, including oxygenation, remained stable despite the diminishing ECMO support.\nHis postoperative course was uneventful, and 94 days after transplantation he was transferred elsewhere for rehabilitation. He has remained in good condition for 2 years 8 months since the lung transplantation without significant complications (Figs. c, b). The long-term non-ambulatory VV-ECMO therapy, however, seems to have interfered with his left leg movement, although it is slowly improving. |
A 29-year-old otherwise healthy woman who had frequently suffered from tonsillitis was admitted for tonsillectomy. The preoperative physical examination revealed no other abnormalities. Tonsillectomy was performed under general anaesthesia with orotracheal intubation. The tonsils were removed by electrodissection with bipolar scissors, and haemostasis was achieved using bipolar cautery. There was no remarkable bleeding during the procedure. There were some adhesions between the tonsils and tonsillar beds that were more remarkable on the left side. After the procedure, the patient was monitored in the recovery room and she was discharged a few hour later.\nAbout 14 hours after the procedure, the patient telephoned the emergency department because some swelling had suddenly appeared on the left side of her face, some two hours earlier. She was advised to come to the emergency department immediately. The patient felt some pressure in the facial region and had a sore throat. She had neither a cough nor difficulty in breathing, and she was able to swallow normally. Physical examination by the on-call otolaryngologist revealed left facial swelling and crepitus that extended to the temporomandibular joint region on the same side and also in the upper neck region (see Figure ). There was neither redness nor signs of cellulitis in the swollen area. Inspection of the tonsillar fossa revealed neither mucosal tear nor other abnormal findings. In indirect laryngoscopy, the larynx and hypopharynx appeared normal. In blood tests, the value of the white blood cells (WBC) was 12.7 × 103/μL and the value of C-reactive protein was 5.1 mg/l. The body temperature was 37.2 degrees Celsius. A chest x-ray was taken the same night and no pathological findings were found. Based on clinical examination, there was no suspicion of abscess or serious infection, and therefore neck computer tomography (CT) imaging was not carried out. Although there was no radiological confirmation the patient was thought to have facial subcutaneous emphysema that developed after the tonsillectomy (see Figure ).\nTreatment was started with a cephalosporin antibiotic (cephalexin 750 mg ×2 per os) and the patient was admitted to the ear and oral diseases ward. The following day, the swelling had extended slightly to the left orbital and frontal areas. The clinical examination of the neck and larynx did not, however, reveal any deterioration. Crepitus was still apparent in the cheek area and also on the mandibular angle. The patient did not have any general symptoms. In blood tests, the WBC was 9.3 × 103/μLand the C-reactive protein was 18.1 mg/l. The antibiotic treatment was continued and the patient was also given oxygen 2 l/min through a nasal cannula for about 24 hours, although she did not have problems with breathing at any point during her stay in hospital.\nThe next day, the swelling had decreased slightly. The patient was feeling well, and she was therefore discharged. The cephalosporin antibiotic was continued for a week. The patient was also forbidden to smoke.\nIn the follow-up examination five days after the onset of symptoms, the crepitus and swelling were resolved. In addition, normal post tonsillectomy status was revealed in the pharynx examination without any pathological findings. |
In June 2006, a 13-year-old girl was referred to Kyoto University Hospital with dyspnea on exertion and wheezing. There was a history of pneumonia in infancy and asthma during early childhood, which had remitted after she started primary school. She did not have atopy or allergies. Both the chest X-ray and the chest computed tomography showed a giant bulla in the right upper lobe and hyperlucency of the right lower lobe because of diminished vasculature due to a hypoplastic right pulmonary artery. There was no evidence of bronchiectasis. The X-ray taken before referral to our hospital was not available. She was admitted to Kyoto University Hospital in March 2007 for further evaluation.\nThe exhaled nitric oxide level (27.9 ppb) was elevated compared with that of normal children (<20 ppb). The sputum eosinophil count was increased to 2.5%. Respiratory function tests (performed in July 2006) showed a severe obstructive pattern (Table ).\nA methacholine challenge test was not performed because of severe airflow limitation, but peak expiratory flow (PEF) monitoring showed diurnal variation of 19% from 210 l/min in the morning to 250 l/min in the evening. A pulmonary perfusion scan demonstrated that arterial flow was decreased on the right side (right: left ratio = 1:3).\nBased on the detection of unilateral pulmonary hyperlucency and the absence of other possible causes, including congenital cysts, congenital pulmonary artery abnormalities, and embolization of the main pulmonary artery, she was diagnosed as having Swyer-James syndrome with a giant bulla. In addition, her response to an inhaled bronchodilator, the diurnal variation of PEF, and the detection of eosinophilic airway inflammation suggested the recurrence of her asthma. Although surgical resection of the giant bulla was considered, medical treatment of the patient's asthma was given precedence to maximize her respiratory function. After treatment with an inhaled corticosteroid preparation and tulobuterol patch, her exertional dyspnea showed marked improvement, and the exhaled nitric oxide level and PEF also improved. From August 2009, however, she complained of right-sided chest pain and her exertional dyspnea recurred. Simultaneously, the giant bulla in the right upper lobe enlarged further and the patient's inspiratory capacity decreased (Figure ). Because her chest pain and exertional dyspnea continued to worsen despite higher doses of asthma medication, surgical resection of the giant bulla was performed in January 2011.\nUnder general anesthesia, the patient was placed in the left lateral position. A massive bulla with a small pedicle in the right S1 was completely resected using an Endo-GIA 45-mm stapling gun (Covidien, Tokyo, Japan) and three cartridges via three access ports: (1) an 11.5 mm port in the seventh intercostal space at the posterior axillary line, (2) a 5.5 mm port in the sixth intercostal space at the inferior angle of the scapula, and (3) an 11.5 mm port in the fifth intercostal space at the anterior axillary line. Histopathological examination of the resected giant bulla and the surrounding lung parenchyma showed mild emphysematous change and proliferation of smooth muscle in the peripheral airways. There were no constrictive changes of the bronchioles or dysplasia of the pulmonary vessels and inflammatory changes were minimal.\nAfter surgical resection, her exertional dyspnea improved dramatically and the right-sided chest pain resolved. In August 2011, she was being treated with a leukotriene receptor antagonist alone. |
A 7-year-old boy complaining of dyspnea and fatigue was admitted to the Cardiology Division of our hospital with a previous history of right atrial myxoma. He had been operated on 2 years prior for right atrial myxoma. The myxoma had been resected from the interatrial septum with its pedicle, and the defect had been repaired with a Dacron patch. He had no familial history of cardiac tumors.\nOn physical examination at arrival, blood pressure was 90/50 mmHg, pulse rate 115/min, and he was tachypnoeic. His liver was palpable at 6 cm below the costal margin, and his jugular venous pressure was slightly elevated. Auscultation revealed a mild systolic murmur suggestive of both mitral and tricuspid insufficiency, and a prominent pulmonary component to the second heart sound was also noted. There was evidence of ascites in the abdomen. The skin was normal.\nElectrocardiography showed sinus tachycardia, and right ventricular hypertrophy. Chest X-ray showed minimal cardiac enlargement. Other laboratory tests were within normal limits.\nEchocardiographic examination revealed a large left atrial myxoma (approximately 40 by 34 mm). The myxoma arose from the interatrial septum of the left atrium. It was mobile during systole and diastole, almost completely obstructing the mitral orifice ().\nThere was little remnant on the septal surface of the right atrium (), and mild enlargement of all cardiac chambers was present. Colour Doppler examination showed mild mitral and tricuspid insufficiency.\nA large myxoma along with its pedicle were totally excised at open-heart surgery () through a right atrial approach. The atrial septum was reexcised and reconstructed with a Dacron patch. The dimensions of the excised septum were 40 by 40 mm. There were no further tumors within rest of the cardiac chambers. The operation and subsequent recovery were uncomplicated.\nHistologically, the tumor had the typical appearance of a benign myxoma composed of gelatinous appearing material with few cells and no mitotic activity. |
A 60-year-old man with a history of asthma, benign prosthetic hypertrophy, and hyperlipidemia presented 1.5 years after an uncomplicated primary right TKA done by an outside surgeon. He had been complaining of 4 months of increased pain in his right knee. An aspiration had been attempted, yielding 1 mL of sanguinous fluid which had not been sent for analysis. The patient continued to have swelling and increased pain in the knee, and an MRI was obtained demonstrating “pseudotumor” (Figure , A–C). He was then referred to our orthopaedic oncology office for further evaluation and management.\nAfter review of initial radiographs (Figure , A and B) and CT (Figure , A–C), the patient underwent an open biopsy of his right tibial lesion adjacent to the tibial baseplate one week after presentation to the office. Pathology from his initial biopsy was consistent with GCTB. One week following his open biopsy, the patient underwent a complex reconstruction of his proximal tibia as well as patellar tendon (Figure , A and B).\nIntraoperatively, complete destruction of the medial cortex of the tibia was noted, with the area infiltrated extensively by tumor. After the initial anterior exposure through the previous TKA incision, the area was extensively curettaged. A high-speed burr and argon beam coagulator was then used to complete the resection at the edges of the cavity. Following the removal of the mass, we noted that the tibial baseplate was mechanically stable even after the extended curettage. An intraoperative determination was made to preserve the primary arthroplasty components and to reinforce the tibia with cement and Steinmann pin fixation. Steinmann pins were fired distally into the tibia, which allowed buttressing of the tibial baseplate proximally. The entire excisional cavity was then packed with polymethyl methacrylate (PMMA) cement. Intraoperative examination demonstrated that the construct had excellent stability and strength afterward.\nFollowing reconstruction of the proximal tibia, attention was turned toward the patellar tendon. We noted that the destructive process had eroded much of the patellar tendon and reconstruction was required. Marlex mesh was used in the technique described by Browne and Hanssen. The mesh was layered into a construct with approximate width as the patellar tendon and then weaved into the remnant of the native patellar tendon into normal tendon tissue. #5 Ethibond suture was used to reinforce the closure and attachment of the Marlex mesh to the tendon, avoiding the placement of mesh adjacent to skin.\nBefore discharge, the patient was placed in a long leg bivalved cast. Three weeks postoperatively, the patient was transitioned into a hinged knee brace, which is locked in extension while upright. The patient was then instructed to allow for bed dangles with the knee. At 6 weeks post-op, the patient began physical therapy for gentle range of motion of the knee, still with brace locked in extension while ambulating. At 7 weeks, the patient was placed on Keflex for 1 week after he noticed a small amount of discharge from his distal incision site after a scab was removed, with resolution of symptoms. Three months post-op, the patient was allowed to weight bear as tolerated on his extremity. At this time, he was started on a trial of denusumab (Amgen Manufacturing Limited) adjuvant chemotherapy under the medial guidance of his oncologist. The patient developed a rash after two doses and was changed to zoledronic acid (Zometa; Novartis Pharmaceuticals Corporation) for a total of 6 months of diphosphonate therapy. He completed the course without further incident. Radiographs taken at 16 months demonstrated maintained alignment without evidence of component subsidence or implant failure (Figure , A and B). At a 20-month follow-up, the patient was weight bearing on the extremity without assistance, using a cane only for long distances. |
A 26-year-old male presented to our institution with three to four months of epistaxis, bilateral nasal obstruction, hyposmia, and headaches. Physical examination revealed a large bilateral intranasal mass emanating from the superior nasal vault and extending into bilateral ethmoid sinuses. Magnetic resonance imaging (MRI) with gadolinium contrast revealed a 5.1 cm × 3.3 cm × 3.4 cm enhancing soft tissue mass centered in the ethmoid sinuses with invasion through the ethmoid plate abutting bilateral frontal lobes (Figure ). There was no gross invasion of frontal lobe parenchyma or dura. Primary surgical excision was performed by otolaryngology and neurosurgical teams with a combined endonasal and bifrontal craniotomy approach. The patient underwent bilateral endoscopic ethmoidectomy and frontal sinusotomy, frontal sinus obliteration, and cranialization via coronal approach with pericranial flap. Intraoperatively, there was extensive bleeding and the patient required intraoperative transfusion of four units packed red blood cells. All gross disease was removed at the time of surgery; he recovered without complications and was discharged home on post-operative day 4. Pathological diagnosis of SNRCLA was made based on morphologic characteristics and immunohistochemistry (Table ). Due to piecemeal resection via endoscopic approach, margin status was unclear. PET scan two months post-operatively suggested residual tumor along the nasal cavity and right orbit. The patient was referred for radiotherapy (RT) where he received 6000 cGy in 30 fractions with a boost of 600 cGy in three fractions to treat residual disease.\nAfter completion of RT, PET imaging at seven months post-op showed persistent disease that had a partial response to RT. Salvage surgery was pursued via combined open and endonasal excision where negative margins were eventually obtained. After salvage surgery, his treatment course was complicated by nasal wound healing complications. He required multiple reconstructive procedures including a skin graft and paramedian forehead flap reconstruction. He is currently without evidence of disease at 16 months after diagnosis. |
An 18-year-old woman without any significant history of previous illnesses visited an outpatient clinic with a chief complaint of recurrent vulvar abscess for 5 months. On inspection, the vulva was diffusely swelling, tender, red and warm (Fig. ). The patient had no other complaints apart from perineal pain and difficulty in sitting for a long time. She had previously visited another hospital with the same complaint, and on 3 occasions received incision and drainage (I&D) of the abscess. She visited a secondary hospital, where abdominal and pelvic computed tomography (APCT) showed a labia major abscess and laboratory results showed no abnormalities. She received antibiotic treatment, comprising of cephalosporin and metronidazole for 2 weeks, but her symptoms did not improve. A magnetic resonance imaging (MRI) of the pelvis was performed which revealed a 9 × 7 mm sized abscess in the right labia major (Fig. A). Under the impression of persistent vulvar abscess with positive culture results implicating Citrobacter freundii, Corynebacterium striatum, and Eschericia coli, I&D with local excision of inflamed tissues was performed under general anesthesia. The pathology results showed acute and chronic inflammation with granulation tissue formation, without indication of caseation status. Intravenous antibiotics were administered for 2 weeks and the vulvar inflammation subsided. However, inflammation and painful swelling of the contralateral labia major arose 2 weeks postoperatively.\nShe was referred to our institution, a tertiary care hospital. At the pediatric and adolescent gynecology clinic, a follow-up MRI was performed because the features of the abscess were not typical of a gynecological abscess. A deep pelvic abscess, at a higher level than Bartholin abscess, was suspected. The MRI results showed diffuse inflammation of the perineum around the posterior vaginal wall with abscess formation along both vestibular glands as well as both labia major and minor. There were also features suggestive of a rectovestibular fistula at the 12–1 ‘o’clock position (Fig. B). The patient was then clinically evaluated for Crohn’s disease because of the above MRI findings. Her laboratory tests, including inflammatory markers, were normal.\nThe patient was then referred to the pediatric gastroenterology department where she underwent further blood tests, gastroduodenoscopy, and colonoscopy to rule out other differential diagnoses, such as sarcoidosis, pyoderma gangrenosum, hiradenitis suppurativa, cellulitis, tuberculosis, and contact dermatitis. Stool calprotectin levels was elevated to > 300 µg/g, which suggested a diagnosis of inflammatory bowel disease. A more detailed history revealed that the patient had no history of diarrhea or hematochezia. On gastroduodenoscopy, gastric mucosa was noted to be erythematous which was suggestive of reflux esophagitis and chronic superficial gastritis. Colonoscopy revealed multiple ulcers on the mucosa of the terminal ileum and the rectum (Fig. ). Pathological evaluation of tissue specimen retrieved showed mild chronic superficial gastritis and ulceration with ill-defined noncaseating granulomatous lesions in the mucosa of the terminal ileum, which were consistent with a diagnosis of Crohn’s disease or tuberculosis. Further AFB staining and Tb-PCR performed on biopsy samples taken from the terminal ileum were negative, which ruled out tuberculosis. The rectal biopsy specimen was within normal limits. The simple endoscopic score for Crohn’s disease was 11 []. The result of the stool culture was positive for Clostridium difficile and the patient received oral metronidazole therapy.\nMRI enterography after oral contrast ingestion showed segmental and uneven wall thickening with ulcerative lesions from the distal to the terminal ileum and distal rectum with increased mucosal enhancement and diffusion restriction (Fig. ). Diffuse bilateral perineal soft tissue infiltration and increased enhancement with features suggestive of a rectovaginal (11 ‘o’clock) and a vaginoperineal (bilateral anterior) fistula were also observed. The abscess in both vestibular glands and both labia were smaller in size compared to those in the pelvic MRI performed the previous month at the gynecology department. The Pediatric Crohn’s Disease Activity Index (PCDAI) was 12.5, which indicated clinical response (≤ 12.5), but not inactive disease (< 10) []. The modified PCDAI score was 2.5, indicating remission (< 7.5) [].\nShe was placed on elemental diet (2000 kcal/day) four times daily for 12 weeks, azathioprine 50 mg daily, and mesalazine 1 g three times daily. Azathioprine was increased to 75 mg daily after three weeks. The elemental diet was stopped after the prescribed 12 weeks, and she was maintained on 75 mg daily of azathioprine. The vulvar lesion completely resolved and her white blood cell (WBC) count was 3470/µL during her follow-up visit after 6 weeks of treatment. Inflammatory markers were normal, and the PCDAI was 10. Oral Azathioprine was increased to 100 mg daily, with continuous WBC monitoring on follow-up.\nThe patient revisited the emergency room with vulvar pain after taking medication for Crohn’s disease for 10 weeks. On examination, there was right labia major swelling, tenderness, and pus discharge (Fig. ). She underwent a Seton procedure for rectovaginal fistula (Fig. ). While her symptoms initially improved, a recurrence required second operation. About 4 months after re-operation, her symptoms finally resolved. |
A 76-year-old man was incidentally diagnosed with a left CIAA by abdominal ultrasonography, with computed tomography (CT) demonstrating a left CIAA measuring 4.5 cm in diameter (). The patient was referred to our hospital for surgical treatment. He had a medical history of liver cirrhosis and traumatic abdominal injury. Thus, he underwent EVAR using a GORE EXCLUDER AAA Endoprosthesis to embolize the left internal iliac artery. The postoperative course was uneventful, and postoperative CT showed no endoleak (). Follow-up CT scans at 6-month, 1-year and 2-year intervals post-EVAR revealed a reduction in the size of the aneurysmal sac. However, the 3-year interval CT demonstrated an increase in the sac size (). Contrast-enhanced CT and angiography revealed no endoleak but showed a dilatation of the CIAA. Therefore, the patient underwent open aortic repair of the CIAA.\nThe infrarenal aorta up to the right common iliac artery and the left external iliac artery were exposed via a retroperitoneal approach. Proximal control was achieved by clamping midway between the renal arteries and the aortic bifurcation, and distal control was achieved by inserting an occlusion balloon into the right common iliac artery and clamping the left external iliac artery. The sac of the CIAA was opened, and many thrombi were noted around the stent graft. No backbleeding of the lumbar arteries or junctional bleeding of the stent was detected, and the entire endograft was removed easily. Abdominal aortic replacement with a Y-shaped vascular prosthesis (J Graft SHIELD NEO, bifurcated vascular prosthesis) was performed (). Proximal and distal anastomoses were performed at the level of midway between the renal arteries and the aortic bifurcation where the endograft was fixed, and at the level of the right common and left external iliac arteries, respectively.\nThe patient recovered uneventfully, however, 1 year after open conversion, CT revealed a significant increase in the size of the unreplaced infrarenal aorta (, ). Reoperation was performed to treat the new aneurysm and on intraoperative findings, it was a true aneurysm. Abdominal aortic replacement was performed, however, infection of the vascular prosthesis occurred as a complication of this second open surgery. The infection was not controlled, and unfortunately, the patient died. |
A 31 year-old female who had undergone excision of a stage IIIB melanoma of the right heel with inguinal node dissection at an outside hospital presented to the emergency department with a 9-day history of intermittent temporal headaches. Postoperatively, she had been enrolled in an open label research protocol for which she was randomized to high dose ipilimumab (10 mg/kg). At time of presentation she had received 3 doses of this agent at 3 week intervals. Her most recent dose was administered 2 weeks prior to her ER presentation. Her only side effect with the first two doses was pruritus.\nIn the emergency department, she described her headaches as nonradiating and initially relieved by nonsteroidal agents. Two days prior to presentation, the headaches became more severe (described as 8/10 in intensity), constant, and unrelieved by nonsteroidal agents. Her review of systems was positive for weight gain of 15 pounds over nine weeks and previous history of sore throat and palpitations. She denied changes in vision, galactorrhoea, temperature intolerance, anxiety, or depression. Her only other medication was a Mirena intrauterine device (IUD). On physical exam, she was afebrile with a normal blood pressure (126/83 mmHg), pulse of 92 beats/minute, and respiratory rate of 16 breaths/minute. She was alert and in no distress. Visual field testing by confrontation was normal. Aside from right inguinal and right heel scars from her recent surgery, her exam was normal.\nWith the exception of a mild leukocytosis (12.9 K), initial laboratory findings demonstrated normal chemistry and hematology panels. Hormonal studies were pending, although she was noted to have a suppressed TSH and elevated free T4 2 weeks prior to admission (). Given the severity of her symptoms as well as awareness of possible hypophyseal involvement with anti-CTLA-4 therapy, the decision was made to obtain an MRI of the pituitary. This demonstrated a gland size at the upper limit of normal, with a measured increase in size from 3 × 15 mm to 9 × 21 mm when compared to a study done 2 months earlier as part of restaging workup (). Based on these findings, a presumptive diagnosis of hypophysitis was made and therapy with high dose intravenous methylprednisolone was initiated. |
A 68-year-old male was admitted to hospital with a history of dyspnea at rest for 2 d, a cough producing mucus, loss of appetite and diarrhoea. Pneumonia was suspected, and the patient received intravenous antibiotic treatment. During the patient's second night in hospital, he was found groggy and walking near his room by the nurses. They took him back to his room and connected a new antibiotic dose to his venous catheter. A few hours later, he was found unconscious, lying on the floor next to his bed. His peripheral venous catheter was connected to a nasal cannula delivering O2 from the wall, with a flow rate of approximately 2 L/min, and the antibiotic dose was found in the garbage can. As post-mortem lividity was present, no resuscitation attempts were performed by the clinicians and the death was pronounced. The body was immediately brought to the University Center of Legal Medicine (Lausanne, Switzerland).\nBefore any manipulation of the corpse, a native CT scan was carried out at around 10 h post-mortem using an eight-row CT unit (CT LightSpeed 8, GE Healthcare, Milwaukee, WI). All scanning parameters are detailed in . A forensic pathologist immediately viewed the native CT images. To evaluate the distribution of gas because of physiological changes in the body after death, the radiological alteration index (RAI) was used as proposed by Egger et al. []. The RAI was based on the analysis of samples from seven sites (heart cavities, liver parenchyma and vessels, left innominate vein, abdominal aorta, kidney parenchyma, L3 vertebra and the subcutaneous pectoral tissues).\nAll images were interpreted in a consensus reading by one board-certified radiologist and one forensic pathologist who were trained in forensic imaging. A post-mortem radiological report was prepared and described all findings from the native CT scan. The native CT scan revealed the presence of subcutaneous, intramuscular and intravascular gas, a pneumoperitoneum, a bloated heart with the right cavities filled with gas and a left pneumothorax ((a–d)). The maximum RAI score is 100, and RAI scores greater than 50 are usually seen in cases of severe changes []. Therefore, given the short post-mortem interval (<12 h), the elevated RAI obtained in this case (75) strongly suggests an exogenous source of gas in the tissues.\nCT-guided gas samples were immediately taken from multiple sites (carotid artery, jugular vein, right auricle, thoracic aorta, pectoral muscle, thoracic cavity, abdominal cavity, scrotum and gluteal soft tissues, (e,f)) according to the protocol for gas analysis described by Varlet et al. [].\nThe external examination revealed massive subcutaneous emphysema with audible crepitations on the entire surface of the body. Additionally, there was a bruise on the left part of the forehead, a small contusion near the left eyebrow surrounded by a purplish-blue bruise and some bruises of different ages on the inferior part of the thorax and on the legs. On the left arm, a venous catheter was still in place.\nSamples for toxicological and biochemical investigations (blood and urine) were collected in S-Monovette® tubes with sodium fluoride or ethylenediaminetetraacetic acid as a preservative (Sarstedt, Nümbrecht, Germany). Biological samples were collected as soon as possible on arrival of the body at the morgue (vitreous humor) and during autopsy (femoral blood, pericardial fluid and urine). During sampling of femoral blood by incision with a scalpel, multiple gas bubbles were visible in the blood ((a)).\nToxicological analysis of the collected samples revealed acetone at a physiological level in the blood, as well as caffeine and paracetamol in both blood and urine. Post-mortem chemistry analysis of the serum samples collected during the autopsy revealed elevated values of C-reactive protein (175 mg/L), consistent with an inflammatory state and a procalcitonine level compatible with a bacterial infection (0.69 μg/L). The results also revealed signs of cardiac dysfunction with a very high level of N-terminal prohormone of brain natriuretic peptide (>35 000 ng/L) and cardiac necrosis with a very high troponin-T level (>56 000 ng/L).\nThe autopsy was performed by one board-certified forensic pathologist and one forensic pathologist in training. Opening of the thoracic cavity revealed numerous gas bubbles in the fatty tissue covering the heart. The pericardium was opened and filled with water, and the heart floated. An incision was made in the right ventricle with a scalpel, and blood with gas bubbles escaped. This technique was performed mainly for training purposes and not for gas sampling, as the gas from the cardiac cavities had already been sampled during CT imaging. The autopsy revealed the presence of numerous gas bubbles throughout the vascular system, even in the small vessels of the brain ((b)). Additionally, changes to the pulmonary parenchyma were consistent with pneumonia. No other major findings were made.\nAn Agilent 6890N GC (Agilent Technologies, Palo Alto, CA) combined with a headspace gas autosampler and equipped with an Agilent Select Permanent Gases column arrangement was used. This column arrangement is specially designed for gas analysis and contains a molecular sieve 5 Å PLOT capillary column (10 m × 0.32 mm i.d.) and a Porabond Q column (50 m × 0.53 mm i.d.) in parallel, which allows for separation of carbon dioxide (CO2). The column temperature was maintained at 45 °C for 13 min. The injector temperature was 100 °C, and the injection was conducted in splitless mode. Helium was used as the carrier gas at a constant flow rate of 8 mL/min. The gas detection and quantification were performed with a thermal conductivity detector set at 150 °C. The system was calibrated for each gas with standard gases of H2S (Multigas, Domdidier, Switzerland), O2 and N2 (from laboratory air), and CH4 and CO2 (Carbagas, Lausanne, Switzerland). With this system, all the gases could be detected in the same run. The gas compositions for the different intracadaver sampling sites are displayed in .\nSamples of the brain, heart, lungs, liver and kidneys were taken during autopsy and stained following a standard haematoxylin and eosin protocol. The heart samples were taken from both ventricles, and the interventricular septum exhibited epicardial vessels deprived of red cells and small intramyocardial haemorrhages. The lung samples taken from each lobe contained numerous clusters of leucocytes, mainly neutrophils, and, to a lesser extent, some macrophages, predominantly in the left lower and right upper lobes. These findings are consistent with the pneumonia suspected on admission to the hospital. There were no significant findings from the other organ samples. Samples of the heart were prepared and analysed for deposition of the plasma antigen fibronectin and the terminal complement complex C5b-9 to look for early cardiac damage, especially the right ventricular ischemia. None of the samples showed reaction for C5b-9, and only small groups of cells stained for fibronectin. Based on these results, cardiac ischemia was not likely in this case.\nIn light of the different results, the cause of death was attributed to a fatal O2 embolism. This was caused by the infusion of pure O2 from the wall into the vascular system through a venous catheter connected to a nasal cannula. Although from a medico–legal point of view the circumstances of the death (accident, suicide and homicide) remained unclear, police investigations led to the suspicion that the patient had connected the nasal cannula to the venous catheter. The wound on the forehead and the bruises observed during the external examination could be explained by a fall from or next to the hospital bed, as suggested by the police. |
A 61-year old white British lady was referred to the local ENT department (day 0) with an acute history of photophobia, diplopia and right-sided facial numbness, preceded by rhinorrhoea and right maxillary sinus pain and swelling. Her past medical history included chronic obstructive pulmonary disease, with recent treatment for an infective exacerbation with a short course of prednisolone and doxycycline, as well as poorly-controlled T2DM (HbA1c: 134mmol/mol). The patient had no previous history of sinusitis.\nOn examination, she was noted to have a right-sided facial droop, facial swelling, numbness over the maxillary division of the trigeminal nerve and weakness of muscles innervated by the marginal mandibular nerve. Reduced visual acuity and ophthalmoplegia of the right eye were also noted. On initial flexible nasoendoscopy, pus and crusting of the right nasal cavity with oedema of the maxillary meatus, but no tissue necrosis, were observed.\nMagnetic resonance imaging (MRI) of her head and neck on day 0 showed fluid in the right maxillary antrum with distortion of the orbital floor and elevation of the inferior rectus, with CT scan confirming right maxillary sinusitis but also showing extension into the orbit through the orbital floor (). An inflamed inferior rectus was noted with right-sided proptosis and inflammatory changes tracking into the pterygopalatine fossa. The patient was commenced on intravenous (IV) ceftriaxone 2g twice a day and metronidazole 500mg three times a day in addition to topical chloramphenicol 0.5% drops twice a day for a presumed right maxillary sinusitis with orbital cellulitis, given for a total of 7 days.\nOn day +2, the patient deteriorated with a decrease in conscious level, resulting in a GCS fluctuating between 13 to 15. She underwent maxillary sinus exploration and debridement, at which point endoscopic examination showed the presence of a fungus ball (; Video in Supplementary material section) with bony invasion of the skull base. Medial maxillectomy, anterior and posterior ethmoidectomy, right sphenopalatine artery ligation and medial orbital wall decompression were carried out. A diagnosis of invasive fungal sinusitis with skull-base osteomyelitis was made. After tissue samples were obtained, liposomal amphotericin B (L-AmB) was instilled into the maxillary sinus peri-operatively, and IV L-AmB) was then commenced at a dose of 5mg/kg daily. Euglycaemia was ensured through a variable-rate insulin infusion.\nSupplementary video related to this article can be found at\nThe following is/are the supplementary data related to this article:\nMicroscopy from tissue samples confirmed the diagnosis of mucormycosis, with the causative agent being identified as Rhizopus arrhizus (). This was identified by macroscopic and microscopic morphological examination with Lactofuchsin from cultures grown on Sabouraud's Dextrose agar supplemented with chloramphenicol (SABC, Oxoid). Species-level identification was confirmed by matrix-assisted laser desorption time of flight mass spectrometry (MALDI-ToF MS) using established methods [] on the Bruker Biotyper Sirius, using Compass software (V4.1) with filamentous fungus database (V3). A Biotyper score of 2.17 was achieved, validating the identification to species level.\nThe following minimum inhibitory concentrations were established in the National Mycology Reference Laboratory using Clinical and Laboratory Standards Institute broth microdilution methodology: amphotericin B 0.06mg/L, posaconazole 0.25mg/L, isavuconazole 1mg/L, voriconazole 8mg/L, itraconazole 0.5mg/L.\nThe patient deteriorated further with decreased visual acuity of her right eye on day +10, with repeat flexible nasoendoscopy showing necrotic tissue and MRI revealing persistent intraorbital oedema involving both intra- and extra-conal spaces, with perineural enhancement of the right optic nerve extending from intra-orbital to intracranial part of the nerve, in keeping with the clinical suspicion of cerebral involvement. In view of this deterioration, a trial of dual-therapy involving the addition of IV posaconazole 300mg once daily was undertaken between days 8–13. This was subsequently stopped on day 5 of therapy in view of deranged liver function tests (Bilirubin: 30μmol/L; Alanine aminotransferase: 89 U/L; Aspartate aminotransferase: 224 U/L; Alkaline phosphatase: 1435 U/L; Albumin 16g/L) attributed to an acute drug-induced liver injury, with reversion to L-AmB monotherapy.\nFurther deterioration was noted on day +34, with repeat imaging showing intra- and extra-conal right orbital abscess formation, as well as new abscess formation in the right masticator space, associated with erosion of the right lateral maxillary wall. Given the extent of infection and lack of any alternative surgical options to achieve source control, exenteration of the right orbit was performed on day +36. Multiple abscesses were observed, necessitating further debridement of the upper lip, nasopharynx and oropharynx. Tissue samples from theatre yielded Pseudomonas aeruginosa, which was treated effectively with IV piperacillin/tazobactam 4.5g three times daily for 6 weeks. No fungal elements were observed on microscopy, with no further moulds being isolated from these samples despite prolonged incubation.\nHistological examination of intraoperative tissue samples revealed an inflammatory infiltrate consisting of a mixture of lymphocytes, histiocytes, neutrophils and multinucleated giant cells, with multiple foci of caseating granulomata seen in association with fungal hyphae, with focal intraneural and vascular invasion by fungal hyphae (). Acute osteomyelitis secondary to invasive mucormycosis was also confirmed histologically through the finding of foci of fungal hyphae and acute inflammatory infiltrate with bony necrosis in a tissue sample from the floor of orbit.\nSatisfactory appearances were noted on subsequent examination under anaesthesia on day +38 & +42, with further local application of L-AmB onto wound packing. Her ongoing medical management was complicated by a gradual deterioration in her renal function secondary to L-AmB treatment. L-AmB was therefore stopped and isavuconazole 200mg once daily was introduced on day +37. Subsequent clinical deterioration led dual antifungal therapy being restarted, with further surgical debridement performed on day +54 and day +58. Tissue samples in these instances revealed glycopeptide-resistant Enterococcus faecium (GRE), treated with IV daptomycin at 10mg/kg once daily for a total of 6 weeks.\nThe patient's clinical condition slowly improved, albeit with ongoing renal impairment. L-AmB was stopped and isavuconazole was converted to enteral formulation on day +85. Her progress was complicated by an intravascular catheter-related bloodstream infection secondary to Klebsiella pneumoniae on day +90, which was managed effectively with 7 days of IV meropenem 1g three times daily. Her insulin regimen was converted to a twice daily subcutaneous insulin regime on day +105. Repeat MRI sinus on day +121 showed no significant disease progression, and the patient was discharged on day +138 on a prolonged enteral course of isavuconazole 200mg once daily. On last outpatient review, the patient was clinically well with no evidence of disease recurrence. Facial reconstruction by the Maxillo-Facial team was subsequently undertaken with a satisfactory outcome. |
A 46-year-old male with a history of severe developmental delay, hydrocephalus, and seizure disorder presented to the hospital with blunt head trauma after a ground level fall. Work-up revealed bilateral acute subdural hematomas for which an external ventricular drain was placed. Despite intensive care management, the patient deteriorated to brain death. He was subsequently evaluated for organ donation. Abdominal computerized tomography (CT) scan () revealed an “elongated structure with metallic components in the upper portion of the IVC that extends into the right atrium.” There was no medical history of a prior procedure, or symptoms, to explain the incidental finding. The radiologist's interpretation and presumptive diagnosis were a retained atrial pacing wire.\nHe subsequently underwent procurement for organ donation after brain death. At the time of cross-clamp, the previously identified foreign body was transected when the right atrium was incised for exsanguination. During the back-table dissection, it was apparent that the foreign body had eroded into the posterior wall of the IVC, extending down the retrohepatic IVC (Figures and ). It also created a calcified reaction at the junction of the suprahepatic IVC and right atrium, adjacent to the left hepatic vein (LHV) and middle hepatic vein (MHV). We removed the foreign body () and performed a venoplasty () of the posterior wall of the IVC and of the common wall of the LHV and MVH, so that the outflow of the LHV and MHV was not compromised after transplant. We discovered that the foreign body was, most likely, a fractured CVC due to the overall appearance and interval markings.\nThe liver recipient was a 65-year-old woman with cirrhosis due to alcohol abuse; her Na-MELD score was 40 at the time of transplant. She underwent caval-sparing total hepatectomy and deceased donor liver transplantation via piggyback technique: the donor suprahepatic IVC was anastomosed to a common orifice of the recipient's right and middle hepatic veins. We did not alter our immunosuppressive therapy or prophylactic antibiotic regimen. Additionally, we did not initiate any anticoagulants or antiplatelet agents beyond our standard postoperative protocol. Postoperative imaging showed normal velocities and waveforms on ultrasound () and unremarkable appearance on axial CT () of the hepatic vein anastomosis. The patient otherwise had an uneventful postoperative course and has had stable allograft function with no venous outflow issues for >8 months after transplant. There were no reported complications in the other organ recipients. |
A 55-year-old woman presented to our Dermatologic Service informing a previous diagnosis of PXE and looking for treatment for her condition. During the anamnesis, she reported that her condition appeared when she was 16-year old. According to her, at that time, progressive, coalescent yellow papules appeared first on the axillae and after on flexural sites including elbows, neck, and groin (Figures , , , and ). In respect to the PXE diagnosis, the patient also reported that it was made by a general practitioner. However, no other detailed information was gotten from her during the anamnesis. Moreover, we could not have access to her medical records to confirm all information foregoing. Neither visual loss nor gastrointestinal alterations were noticed by her, except for arterial systemic hypertension, and dyslipidemia, which have been treated by using specific medication. The patient's family history was unremarkable with none of her siblings and first- and second-degree relatives affected by the disease. Clinically, the skin lesions revealed yellowish plaques on the flexural locations including axillary and neck regions. Likewise, oral examination also revealed a yellowish macula on the inner aspect of her lower lip (). After dermatological and oral examinations, fragments from the skin and lower lip were taken, routinely processed, and stained with hematoxylin and eosin to confirm the PXE diagnosis. Also, a small piece of the lip lesion was cut out to be analyzed by transmission electron microscopy trying to identify ultrastructural changes in the connective tissue that could suggest PXE.\nAs the microscopic features of the skin lesion were similar to the oral one, we will describe herein only those seen in the oral lesion. Microscopically, the lip lesion showed a well-circumscribed, noncapsulated lesion composed of scattered deposits of fragmented and basophilic fibers among collagenous fibers of the connective tissue (Figures and ). Neither inflammation nor signals of any causative agent were observed. Orcein and Von Kossa staining revealed a large amount of shortened, fragmented elastic fibers and deposits of black bodies throughout the lesion, confirming the presence of calcium and phosphorus (Figures and ).\nIn addition, by using transmission electron microscopy, numerous aggregates usually large, but sometimes small, electron-dense calcified bodies in the cores of the elastic fibers were identified, resulting in the rupture of some elastic fibers (Figures and ). Based on the clinical features and histopathological and ultrastructural aspects, a diagnosis of oral PXE was carried out.\nNext, the patient was referred to the ophthalmologic department at our institution to be submitted to a funduscopic examination, which in turn revealed the formation of angioid streaks without visual loss. She was also sent to the cardiology and gastroenterology departments to be systemically evaluated, but no abnormalities were found. After that, it was explained to the patient about the nature and expected evolution of her condition, as well as the impossibility of curative treatment. She was also advised to be monitored regularly in an effort to detect early signals of the loss of acuity or secondary choroidal neovascularization (another severe ocular complication), as also to prevent other complications that are likely to develop over the progression of the disease, especially those affecting the gastrointestinal and cardiovascular systems. After three years of followup, both skin and oral lesions have remained stable, as also her ophthalmic condition. Additionally, cardiovascular and gastrointestinal monitoring has not revealed any alterations so far. |
A 3-year-old female child with no significant past medical problems and unknown immunization history presented with a history of dry cough and gradual onset of dyspnea for 2 weeks without fever or evidence of upper respiratory tract infection. She began to develop significant weight loss and progressive fatigue. Chest X-ray showed a heterogeneous opacity involving most of her right lung (Figure . The initial diagnosis was pneumonia, for which she was she was kept on antibiotics (amoxicillin) and bronchodilators. However, this treatment failed to improve her cough or dyspnea. Several days later, the patient developed weakness of both lower limbs which then progressed to a complete inability to walk, with flaccid weakness of her lower limbs over 20 days in addition to dyspnea. She was admitted to a local hospital. The main differential diagnosis at that time was Guillain-Barré syndrome, depending on history and clinical findings, only because the facility for other sophisticated investigation was unfortunately unavailable at that peripheral hospital. Her condition rapidly worsened, including further worsening of her dyspnea and the development of central cyanosis. She was referred to our tertiary center for immediate mechanical ventilation.\nUpon arrival, the patient was severely dyspneic with central cyanosis, respiratory rate of 50 cycles/minute, and peripheral capillary oxygen saturation (SpO2) of 85% on room air. Neurological examination showed loss of sphincter control such that the bladder was palpable up to the level just below the umbilicus; therefore, she underwent catheterization. Further examination showed 2 palpable small right-side supraclavicular lymph nodes. Chest auscultation showed poor air entry in most of her right lung. Neurological examination of her lower limbs revealed flaccid paraparesis with loss of tone and reflexes and equivocal Babinski reflex on both sides. Sensory examination was difficult and non-conclusive and opsoclonus-myoclonus movement of both eyes were observed; however, her pupils were normal in size and reacted to light with no periorbital ecchymosis.\nThe patient was admitted to the respiratory care unit and ventilated with synchronized mechanical ventilation (SMV) for her declining oxygen saturation and worsening dyspnea.\nThe patient’s serum electrolyte levels were normal and other blood test results were unremarkable except for respiratory acidosis. Emergency magnetic resonance imaging (MRI) of the chest showed a large right posterio-superior mediastinal mass that had pushed the trachea to the left with heterogeneous enhancement associated with pleural effusion, as well as 2 supraclavicular lymph nodes, direct invasion of the dorsal spine, and canal stenosis. The liver was normal no metastasis (Figure .\nWhole-spine MRI showed extension of the tumor to the intraspinal canal at the level of D1-D8 (Figure . The initial differential diagnosis was neuroblastoma. Thereafter, assessment of urine vanillylmandelic acid (VMA) showed high levels. Bone marrow biopsy confirmed infiltration with neuroblastoma tumor cells.\nDue to spinal compression, which is an oncologic emergency, the patient underwent thoracotomy and total resection of the mass with spinal decompression and relief of the canal stenosis with pedicle screw instrumentation to fix the spine after long-segment decompression.\nHistopathological examination detected a poorly differentiated neuroblastoma as a small, blue, round-cells tumor with a fine chromatin pattern and high mitotic rate with nests of cells (pseudorosettes). Supraclavicular lymph node biopsy detected features of round blue cells consistent with neuroblastoma. According to the revised Shimada grading system, the tumor was a poorly differentiated neuroblastoma. Therefore, according to the International Neuroblastoma Risk Group Staging System (INRSS), the patient was classified as having high-risk neuroblastoma.\nThe postoperative period passed smoothly with significant improvement of the dyspnea followed by weaning from the ventilator but with residual lower limb weakness, opsoclonus myoclonus eye movement, and loss of bladder control. Then, the patient was administered a course of induction of intensive chemotherapy including the combination of cyclophosphamide, etoposide, and vincristine; however, the patient unfortunately died during the induction phase due to severe pancytopenia, overwhelming septicemia, and renal impairment despite intensive care and management. The timeline of patient case history and follow-up is shown (Figure 4). |
A 52-year-old male presented with a chief complaint of painful swelling of the left upper jaw; he had had the swelling for 2 months. The swelling was associated with decreased vision and watering from the left eye for this period. The patient's past medical history and family history were non-contributory. He had had the habit of smoking and alcohol consumption for 30 years.\nExtraorally, a solitary swelling was observed involving the left middle third of the face, which was non-tender and hard in consistency with no changes in surface temperature (). Intraorally, a well-defined swelling was located on the alveolus of the left maxilla in the region of the first, second, and third molars with obliteration of the buccal vestibule. On palpation, the swelling was non-tender, and firm to hard in consistency ().\nPeriapical radiographs () showed a characteristic "ground glass" appearance and symmetric widening of the periodontal ligament space in relation to the left maxillary canine and the first and second premolars. A panoramic radiograph () revealed complete destruction of the left maxillary antral walls and zygomatic process of the maxilla, and the center of the lesion exhibited numerous calcifications.\nThe coronal section of the CT scan (bone algorithm; ) revealed a hypodense mass involving the left maxillary antrum with complete destruction of the superior, inferior, medial, and lateral walls, with invasion of the mass into the nasal fossa, orbit, and the hard palate. There was also destruction of the conchae of the left nasal fossa. The inferior aspect of the lesion showed a micro-multilocular pattern, whereas the rest of the areas exhibited calcifications distributed haphazardly.\nAxial sections of CT (soft tissue algorithm) showed calcifications at the left maxillary alveolar process as well as in the left maxillary antrum. Complete destruction of all walls of the left maxillary antrum was observed with diffuse calcifications ().\nBased on the radiological findings, differential diagnoses of osteosarcoma and osteoblastic metastatic malignancy such as prostate cancer were considered. An incisional biopsy was taken, and histopathology () revealed solid nests of malignant tumor cells (epidermoid, intermediate, and occasional mucous cells); many of these tumor islands showed central necrosis with dystrophic calcification and a few cystic spaces. The fibrous stroma consisted of blood vessels and inflammatory cells. Histopathology confirmed high-grade MEC. Based on these findings, a final diagnosis of a central MEC of the left maxilla was made. |
A 34-year-old gentleman with mild dysmorphic features presented to the emergency department with a sudden onset loss of consciousness which lasted approximately 1 minute, during which he sustained tonic-clonic movements which self-terminated. A 1 year history of episodic carpopedal spasms and upper limb paraesthesia was elicited. A head computed tomography (CT) scan was normal. A corrected calcium level was 1.17 mmol/L.\nHis past medical history included a febrile seizure at 2 years of age, with no history suggestive of absence seizures or myoclonic jerks. He was operated for testicular torsion, acute appendicitis, a congenital epigastric hernia, a supraumbilical hernia, and a removal of an accessory digit in the left hand, the latter of which was also present in relatives from his paternal side. His developmental history included gestation at term with a birth weight of 3.2 kg and a delayed growth spurt. Until the age of 13 years he had regular physiotherapy for recurrent chest and sinus infections, with multiple hospital admissions. At that point he was thoroughly investigated and a diagnosis of cystic fibrosis was suspected, with equivocal sodium sweat tests. Heaf tuberculin skin test (Sterneedle test) in 1985, in spite of previous TB vaccination, was negative. During his adolescence he also reported incapacitating muscle cramps, which later resolved. Learning difficulties were attributed to his frequent absence from school, but the patient was now in full time employment.\nOn examination there was mild facial dysmorphism with a short philtrum. He was haemodynamically stable, with an early diastolic murmur. Chest was clear. Examination of the neck revealed a grossly enlarged right thyroid lobe, with deviation of the trachea to the left.\nThyroid function tests and antithyroid antibodies were normal. Serum parathyroid hormone levels were low. Serum calcitonin levels were elevated. Other routine blood investigations, including magnesium levels, were normal. A CT scan of the neck and thorax showed a grossly enlarged, inhomogeneous thyroid gland which was extending deep into the upper mediastinum and associated with enlarged cervical lymph nodes (Figures and ). An incidental right-sided aortic arch was also noted (). In view of the malignant features on CT, a total thyroidectomy and lymphadenectomy was performed. Histopathology reported the presence of two separate follicular adenomata and a nodular goitre.\nThe patient was discharged on calcium supplements, alpha-calcidol and antiepileptic drugs. The latter were initiated in view of tonic carpo-pedal spasms, which were initially considered to be suggestive of partial seizure activity. A brain magnetic resonance (MR) scan was unremarkable. No further seizures were reported during the follow-up period with continuation of calcium supplementation and the antiepileptic treatment was stopped.\nFluorescence in situ hybridisation (FISH) analysis later confirmed the diagnosis of DiGeorge syndrome with a deletion on the long arm of chromosome 22-22q11.2 deletion. |
A 75-year-old woman with no significant history of liver disease or neurologic disorder was evaluated for altered mental status. Her initial ammonia level was 33 mmol/L (normal range 11-32 mmol/L), and liver function tests (LFTs) were within normal range. A CT of the abdomen was performed which demonstrated an intrahepatic shunt between the right posterior portal vein and the right hepatic vein (Figure A) as well as enlargement of the right hepatic vein (type II IPSVS). No additional vascular abnormalities were identified. Given the lack of prior surgery or biopsy, the etiology of this shunt was deemed to be congenital in nature.\nAfter consultation with the interventional radiology service, angiography with endovascular embolization of the shunt was planned. The right hepatic vein was catheterized from a right internal jugular venous approach, and a 4-French angled glide catheter (Terumo Corporation) was manipulated through the shunt into the main portal vein. Prior to embolization, a temporary balloon-occlusion test of the shunt was performed using a 12-mm Berenstein occlusion balloon catheter (Boston Scientific). The test did show a portal pressure of 9 mm Hg before and 16 mm Hg after balloon occlusion, and this lasts remaining unchanged after several minutes. The findings were interpreted as no significant hemodynamic changes or risk of portal hypertension that would preclude shunt occlusion. Therefore, the shunt was then successfully embolized with Penumbra POD coils (Penumbra). Due to the high flow nature of the shunt, it was necessary to use coils that were approximately 50% oversized for the targeted vessel diameter to prevent potential coil migration and nontarget embolization. Following POD embolization, an Amplatzer Vascular Plug II (St. Jude Medical) was deployed as a safety measure to further avoid nontarget coil migration into the right atrium and pulmonary vasculature.\nFollowing embolization, a contrast-enhanced CT of the abdomen and pelvis was performed which demonstrated successful exclusion of the venous-venous malformation with thrombosis of the posterior segment branch of the right portal vein (Figure ). Follow-up at 6 and 12 months later yielded a normalized ammonia level and over time, improvement and objective resolution of the patient's hepatic encephalopathy. Abdominal color Doppler ultrasound did not show recurrent shunt recanalization. |
A 33-year-old man reported to the department with a chief complaint of pain and swelling in the lower left jaw region for 3 months and had no significant medical history. The patient gave a history of slip and fall on the floor 3 months back after which swelling and pain developed. On extraoral examination, mild asymmetry was noted with no trismus or lip paresthesia. Diffuse solitary swelling was seen in the left lower third of the face measuring 5 cm × 5 cm. Anteroposteriorly swelling extended from 1 cm from the corner of the mouth till the angle region of the mandible and superoinferiorly extending 2 cm below the ala tragal line extending up to the inferior border of the mandible.\nTwo submandibular lymph nodes were palpable measuring around 0.5 cm × 0.5 cm oval in shape, fixed to the underlying tissue, and tender on palpation.\nOn intraoral examination diffuse swelling measuring around 4 cm × 5 cm was seen extending from mesial aspect of mandibular left first premolar and extending up to the retromolar region. Mucosa over swelling was normal and mildly tender on palpation. Vestibule was obliterated. The patient gave a history of spontaneous exfoliation of teeth in that region [].\nCone-beam computed tomography revealed radiolucent lesion with ill-defined border extending from mandibular left first premolar up to the angle region []. Chest X-ray did not reveal any metastatic deposits.\nIncisional biopsy was performed under local anaesthesia. Histopathology suggested malignant fibrous histiocytoma. Immunohistochemical stains showed strong reactivity to vimentin. Ultrasound of the neck showed large soft-tissue mass on the left side of the mandible, showing heterogeneous texture with increased flow measuring around 3.5 cm × 3 cm. Two or three enlarged lymph nodes were seen in the left submandibular region measuring 0.8–1.2 cm.\nSegmental mandibulectomy with supraomohyoid neck dissection was planned with reconstruction with fibula osteomyocutaneous flap. Midline split incision was given extending till the left neck crease []. Segmental mandibulectomy was done from midline extending up to ramus. Left level IIa and III neck nodes were cleared []. Fibula flap was harvested and used to reconstruct the left side of the mandible with reconstruction plates and microvascular anastomosis was carried out [].\nHistological examination [] of the primary tumour revealed highly cellular mesenchymal component that consisted of numerous monomorphic spindle-shaped fibroblast and histiocyte-like cells in varying proportions. Multinucleated giant cell was seen interspersed in the connective tissue stroma. At the periphery, the tumour cells were invading the osseous tissue. Postoperative orthopantomogram was taken []. The patient was referred for adjuvant radiotherapy. |
A 74-year-old woman visited our station with squamous cell carcinoma (SCC) on the right buccal mucosa. Her past medical history included chronic obstructive airways disease, hypertension, and diabetes mellitus. The patient is a current smoker, with a history of 20 pack-years. Preoperative chest radiography, electrocardiogram, full blood count, and serum biochemistry were within the normal range. After being diagnosed with SCC as a result of incisional biopsy, the patient underwent the resection of SCC on the right buccal mucosa of the mandible, modified radical neck dissection, and primary reconstruction with a fibula-free flap. Tourniquet pressure was 300 mm/Hg, and its application time was 60 min. Total on-table time was approximately 7 h. Upon admission to the SICU after the 7-h operation, hypothermia and hypotension were noted. On the first postoperative day, the patient exhibited oliguria and proteinuria and elevation of CK, AST, ALT, and LDH. Together with the nephrology and neurology staff, we tried to figure out our patient’s symptoms and clinical findings. We thought that her clinical picture was based on an impression in which acute renal failure was diagnosed as secondary to rhabdomyolysis. Thus, she was managed with high-dose loop diuretic therapy. Additionally, we gave her hepatotonic to recover her liver function. The patient was supplemented with 150 to 250 mL/h of lactated Ringer’s solution and 0.9% NaCl. When the volume was full, urine output of above 100 mL/h was maintained by 20 mg intravenous injection with furosemide.\nHer urine output for the first hour is at 20 mL/hour, but after the medication, her urine output began to improve on day 4 with a corresponding reversal in the serum creatinine. After postoperative day 4, the muscular enzyme showed a downward trend. We treated the patient with medication and hydration, and then the result became favorable. In the end, she was able to recover fully from the symptoms. Figures and show the change in serum enzyme levels during hospitalization (Figs. and ). |
Case 1 was an 8-year-old boy who was admitted to the hospital for seizures and developmental delay. He had severe mental retardation and was unable to walk. He was born prematurely at 36 weeks through spontaneous vaginal delivery to consanguineous parents as a first child. There was no history of maternal diabetes or exposure to any teratogenic agents of the mother during the pregnancy. There were no information on his birth weight, length, and head circumference. Although the neonatal period was normal, a global delay of development was prominent at 4 months of age. At that time, the child was often irritable and had feeding difficulties. At 5 months of age, he had suffered from frequent tonic seizures requiring antiepileptic drug (AED) therapy. Moreover, cerebral magnetic resonance imaging (MRI) revealed white matter lesions. He was diagnosed with spastic cerebral palsy in another hospital, due to the history of prematurity and MRI findings. At the time of admission to our department, his neurological examination revealed severe mental retardation, microcephaly, and spasticity, but the cranial nerves were normal. There were increased tones in the upper and lower limbs and hyperreflexia with bilateral positive Babinski sign. Ophthalmologic examination was normal. He was unable to sit, walk, and speak. There was no hepatosplenomegaly. Laboratory parameters revealed normal complete blood count, serum electrolytes, liver and renal function, and thyroid function tests. The serology for toxoplasmosis, rubella, cytomegalovirus, and herpes simplex was all negative. Urinary amino and organic acids and metabolic screen were all normal. The cranial computed tomography (CT) scan showed calcifications in the basal ganglia and periventricular white matter []. The brain MRI revealed brain atrophy and demyelination of the white matter []. He was found to have a cardiomyopathy by echocardiography.\nClinical and neuroimaging findings have suggested AGS, and the mutation analysis of the TREX1 gene revealed a homozygous novel mutation, p.R169H (c.506G>A). |
Mr. F is a 27-year-old Caucasian male with a history of depression and schizophrenia with prominent negative symptoms. Before presenting to our outpatient clinic, Mr. F had been treated with multiple atypical antipsychotics including aripiprazole, olanzapine, quetiapine, and ziprasidone. An adequate trial was given for each agent, but the hallucinations and paranoia continued to be socially impairing. Aripiprazole was discontinued due to a poor response at a dose of 45 mg; olanzapine and quetiapine were discontinued secondary to excessive weight gain, and the patient had an acute glossopharyngeal dystonic event after taking the first few doses of ziprasidone about one year before presenting to our clinic. At that time, an acute allergic reaction was ruled out as the patient maintained blood pressure, lacked any skin involvement, and had no other laboratory abnormalities indicating systemic involvement. The symptoms of reduced air movement and tongue swelling were rapidly reversed with 50 mg of intravenous (IV) diphenhydramine.\nMr. F came to our clinic approximately one year after the initial dystonic event and had not taken medications since that time due to the adverse reaction. At this visit, he had notable social isolation, paranoid ideation, and auditory hallucinations. Due to his psychotic symptoms, it was difficult for Mr. F to perform some of his activities of daily living, including using public transportation and going to the grocery store. He was also requesting treatment for his depression symptoms. Fluoxetine was started and titrated over the next few months to 40 mg. Mr. F's depression partially responded to fluoxetine, but the auditory hallucinations continued to be problematic. Due to the history of weight gain from other antipsychotics, lurasidone was chosen to target his psychotic symptoms. After the first dose of lurasidone 40 mg, Mr. F reported difficulty in swallowing and speaking. En route to the emergency room, Mr. F was given IV diphenhydramine, and the reaction dissipated within minutes. An acute allergy was again ruled out due to lack of systemic involvement, lack of a rash, stable vital signs, and normal laboratory values. Mr. F was discharged home a few hours later with a diagnosis of glossopharyngeal dystonia. |
We report the case of a 58-year-old man who presented to the Emergency Department (ED) with bilateral leg pain, swelling and erythema. It had been reported by the care home staff that his mobility had decreased over the past few days secondary to the pain. The patient himself was a poor historian and we were unable to elicit the start of his symptoms but there was no history of trauma.\nHe was admitted under the care of the medical team the same day. A diagnosis of suspected bilateral cellulitis of the lower leg was made and he was commenced on intravenous flucloxicillin 2g QDS.\nLaboratory tests on admission were: haemoglobin 13.2 g/dL, white cell count 16.8 × 109/L, sodium 122 mmol/L, potassium 3.8 mmol/L, urea 2.4 mmol/L, creatine 80 mmol/L C-reactive protein of 23 mg/L and a creatine kinase level of 6,966 IU/L. Although compartment syndrome was not initially suspected the creatine kinase was performed as part of the accident and emergency pre-set blood profile for a patient attending with leg pain. The initial rise in creatine kinase was attributed to reduced mobility the previous day and the patients acute kidney injury.\nOn day 2 his renal function remained stable and his c-reactive protein had increased to 169mg/L. Interestingly his creatine kinase level had raised substantially to 33,250 I U/L. His fluid input and urine output were closely monitored and intravenous hydration continued. Daily urea and electrolytes were performed which remained static. The same treatment continued for a further 5 days, with his c-reactive protein rising further, and his creatine kinase remaining grossly elevated. Blood cultures taken were negative at 48 hours. On day 5 the general surgical team saw the patient as he had developed a distended abdomen and had clinical features of small bowel obstruction.\nThe general surgical consultant suspected bilateral anterolateral compartment syndrome, with secondary abdominal distension due to ileus as a result of rhabdomyolysis. An abdominal computerised tomography (CT) scan was performed which demonstrated high-grade small bowel obstruction with no identifiable cause radiologically. The patient was promptly reviewed by the orthopaedic consultant on call and a decision made to take the patient to theatre for bilateral anterolateral compartment fasciotomies and a laparotomy as a combined procedure.\nFasciotomy revealed muscle necrosis bilaterally in the antrolateral compartment. Decompression and debridement of the anterolateral compartments of both lower limbs took place. The laparotomy was negative with still no identifiable cause for the small bowel obstruction, and no evidence of bowel ischaemia.\nThe patient was managed in the intensive care unit postoperatively. A second-look and debridement with cutaneous approximation was performed 48 hours post-operatively. Recovery following this was uneventful, his bowel obstruction resolved and we discharged the patient after 3 weeks and rehabilitation was commenced. |
A 44-year-old man was admitted to the Intensive Care Unit for acute pulmonary edema. He referred worsening exertional dyspnea, paroxysmal nocturnal dyspnea, and orthopnea during the last four weeks; he also complained of intermittent low-grade evening fever during the last days. His past medical history was unremarkable, and he denied any common cardiovascular risk factor. On admission to hospital, the patient presented a blood pressure of 110/60 mmHg. The chest examination found diffuse pulmonary crackles; the cardiac auscultation revealed a suspected opening snap and a diastolic murmur with presystolic accentuation that seems to vary with changes in position. The ECG was normal. Because of the high suspicion for mitral valve stenosis, transthoracic (TTE) and then transesophageal echocardiography (TEE) were performed.\nA giant floppy mass (64 × 37 mm) implanted on the left side of the interatrial septum with diastolic movement through the mitral valve into the left ventricle was detected (Figures and ; see supplementary material available online at ). The colour Doppler revealed high turbulence due to a diastolic transmitral flow obstruction (), and the continuous-wave Doppler confirmed the obstruction with an extremely high transmitral gradient pressure (medium 21 mmHg; maximum 42 mmHg) (). Three-dimensional TEE was performed in order to properly evaluate the mass and its relationship with the heart structures (Figures and ).\nThe left side interatrial septum localization near the fossa ovalis and the echogenicity were highly suggestive for an atrial myxoma. The patient received massive doses of diuretic therapy initially and underwent successful surgical mass excision two days later. The mass (70 × 41 mm) was solid and smooth in appearance, attached via a pedunculated base. The surface predominant colour was red due to multiple and diffused areas of haemorrhage.\nHistologically, there were cords and syncytia of stellate shaped cells with abundant eosinophilic cytoplasm, within glycosaminoglycan-rich myxoid stroma. These cells formed rings around the small multiple vessels. There were also extravasated red cells and multiple foci of recent and old hemorrhage with hemosiderin deposition. These histopathologic features were consistent with the diagnosis of myxoma.\nAfter 3 months, the patient is in good general condition, and the control TTE was negative. |
A 65-year-old man with severe chronic obstructive pulmonary disease (COPD) was admitted with a four-day history of chest pain and worsening shortness of breath. He explained the chest pain started suddenly when he tried to reach out for something on his computer table. It was located on the right anterior chest, sharp in nature, 7/10 in intensity, pleuritic, and worse with coughing and deep breathing. He had a past medical history of severe COPD with frequent exacerbations recently necessitating multiple antibiotics and steroid courses, coronary artery disease, gastroesophageal reflux disease (GERD), and hypertension. He had a 30-pack per year smoking history and quit about 10 years ago. He was a retired fireman living with his family.\nVitals signs in the emergency department (ED) were stable; he was breathing on ambient air. The physical examination demonstrated decreased breath sounds bilaterally without any wheezing or Ronchi. Moderate tenderness was present in the mid-axillary line in the fifth intercostal space, but no other abnormalities were noticed. Laboratory investigations were negative for any leukocytosis, troponin, or any other abnormalities. The electrocardiogram (EKG) showed a normal sinus rhythm. Computed tomography (CT) angiography was done to rule out pulmonary embolism (PE). The CT was negative for PE but showed mild peribronchial infiltrate in the right middle lobe and posterolateral lung herniation between the seventh and eighth ribs, with minimal subcutaneous emphysema along the right chest wall (Figure ).\nThe patient was admitted to the hospital and managed conservatively on broad-spectrum antibiotics, including vancomycin, levofloxacin, and 40 mg per day of prednisone. Two days later, his face swelled up suddenly with a change in the quality of his voice while he was eating dinner. An examination showed a swelling in the neck, diffuse crepitations on his body involving the face, all the way down to the buttocks. Repeat CT chest and neck showed extensive subcutaneous emphysema in the face, neck, chest, and mediastinum with a right-sided pneumothorax at the level of the previous lung herniation (Figures -).\nThe prevertebral and retropharyngeal air was demonstrated as compressing the oropharynx (Figure ). His oxygen requirement went up to 6 liters nasal cannula. A blowhole incision was made on the anterior chest wall, and he was observed in the medical intensive care unit (MICU). Surgery to close the defect was deferred due to his other comorbidities and the higher risk of post-operative complications. He improved gradually over the course of the next few days, completed the course of antibiotics with steroids, and was discharged to a rehabilitation center and did well post-discharge. |
A 21-year-old male patient was admitted to our emergency department due to painful swelling of his left (nondominant) hand after crushing his hand against a wall while riding his motorbike. Both upper extremities were neurovascularly intact. Two small wounds were recognized dorsally over the second and third metacarpophalangeal (MCP) joint (Figure ).\nPlain radiographs of the injured hand revealed a nondisplaced fracture of the proximal phalanx of the index finger, a fracture of the styloid process of the ulna, a fracture of the styloid process of the radius, and an uncommon fracture of the head of the fourth metacarpal with complete horizontal displacement of the distal fragment (Figures , ).\nThe patient consented to surgery, which was performed on the same day. Following a longitudinal dorsal incision and subcutaneous dissection, the metacarpal head was found lying through the extensor tendon. The capsule and the sheath of the extensor tendon were disrupted from the metacarpal head. The tendon was mobilized radially which surprisingly revealed no capsular attachment neither on the volar nor on the dorsal side, whereas the collateral ligaments of the MP joint were intact (Figure ).\nThe fracture was reduced and stabilized via two Kirschner wires and the capsule and the extensor mechanism were restored. In addition, a wire was placed to the ulna’s styloid process and two wires to the radial styloid process after open reduction of the wrist joint (Figures , ).\nConservative treatment was undertaken for the phalangeal fracture. A volar splint was placed; after postoperative day three, the patient was allowed to remove the splint and perform active flexion and passive extension exercises. At four weeks postoperatively, the splint was removed and the patient resumed progressive range of motion (ROM) and strengthening exercises. The wires were removed between the fifth and sixth week postoperatively. All fractures healed during the same period except for the ulna styloid fracture, without any functional restriction or any patient complaints (Figure ).\nSubsequently, the patient underwent intensive physiotherapy, and vitamin C supplementation was also prescribed. The patient regained his grip strength and full ROM gradually and returned to his daily routine three months after the initial trauma (Figures , ).\nFollow-up at nine months revealed partial avascular necrosis of the metacarpal head at the ulnar and superior side; this was, however, only a radiographic finding as the patient reported no complaints about pain or tenderness and there were no limitations in his hand functionality (Figure ).\nHis grip strength was excellent and his ROM was normal. At the final 12-month follow-up, the patient reported excellent grip strength and normal ROM. In addition, the radiographs revealed unchanged avascular necrosis of the metacarpal head; hence, the patient was released from our care. |
A 63-year-old man underwent PpPD and Child reconstruction with Braun anastomosis for lower bile duct carcinoma at another hospital. Two weeks after surgery, he vomited several times due to DGE, and a nasogastric tube was inserted into the stomach. On the 32nd POD, however, DGE improved and the nasogastric tube was removed, as dysphagia persisted. On the 41st POD, gastrointestinal endoscopy was performed, revealing stricture of the middle esophagus. PPI did not improve esophageal stricture; the patient then underwent balloon dilation on the 70th POD, but the esophagus was perforated. The esophagus recovered with fasting, antibiotics, and PPI without surgical treatment. As the stricture still remained, he was referred to our hospital for further treatment on the148th POD.\nThe upper gastrointestinal series revealed a long stricture extending from the middle esophagus to just above the cardia portion, the length of which was approximately 10 cm, and the sliding esophageal hiatal hernia (Fig. ). Gastrointestinal endoscopy showed circumferential stricture of the middle esophagus with longitudinal esophageal ulcer scars (Fig. ). The narrow lesion was biopsied, and the result showed no malignancy. Preoperative gastrointestinal endoscopy before PpPD revealed a sliding esophageal hiatal hernia and mild esophagitis (Fig. ). We speculated that postoperative DGE, hiatal hernia, and gastric hyperacidity exacerbated the patient’s reflex esophagitis. The patient was treated with an H2 blocker for 2 weeks just after the surgery and with PPI from the 14th POD until the 140th POD. PPI was replaced to the H2 blocker due to the decreased numbers of white blood cells to less than 2000/μl from the 141th POD. The number of white blood cells recovered to normal level soon. Severe extensive stricture remained observed. We suspected that this patient had resistance to PPI; thus, we performed simultaneous 24-h pH and bilirubin monitoring to estimate the extent to which gastric acid secretion was inhibited by omeprazole (20 mg/drip/twice a day). Proximal and distal pH sensors were positioned in the narrow lesion and in the stomach, respectively, and a bilirubin sensor was positioned just beyond the narrow lesion. In the stomach, a pH < 4 was observed 89.3 % of the time (Fig. ). Usually, in patients with GERD or intermediate and poor metabolizer CYP2C19 genotype, the proportion of time for which the stomach is characterized by pH < 4 decreases to approximately 50 % with PPI [, ]. In this study, after treatment with omeprazole for 6 days, the white blood cell count decreased from 4000 to 1700/μl; this level increased to normal levels soon after the course of medication had been completed. We recognized that this patient was resistant to PPI. Neither an H2 blocker nor PPI could prevent the exacerbation of reflux esophagitis. We therefore concluded that medication therapy could not suppress gastric acid and considered performing total gastrectomy to prevent gastric acid from injuring reconstructed organs and the remnant esophagus.\nOn the 158th POD, we performed resection of the middle-lower esophagus and total gastrectomy through a right thoracoabdominal approach. The middle-lower esophagus was hard; we therefore cut the esophagus just beyond the azygos arch. The length of the jejunum was not sufficient to pull up to the cut end of the esophagus due to Child reconstruction after PpPD, and we performed reconstruction using the transverse colon (Fig. ).\nThe resected specimen showed wall thickening of the middle-lower esophagus, an ulcer scar on the 8-cm oral side of the cardia, an ulcer at the esophagogastric junction, and a petechial hemorrhage in the stomach. Pathological examination revealed no malignancy.\nMinor leakage of the esophagocolonostomy developed postoperatively; the patient recovered rapidly under conservative treatment. He started oral intake on the 23rd POD and was discharged on the 34th POD in good condition. The CYP2C19 genotyping test performed after the second operation showed that the patient had a rapid metabolizer genotype. Two and a half years after the second surgery, gastroendoscopy and simultaneous 24-h pH and bilirubin monitoring were performed. There was no sign of either esophagitis or Barrett’s esophagus. Simultaneous 24-h pH and bilirubin monitoring revealed that the rate of fraction time bilirubin absorbance >0.14 was 0.7 % in the remnant esophagus; there was little bile reflux to the remnant esophagus. Nine years after surgery, the patient has good oral intake with good nutrition, without reflux esophagitis, symptom, or any evidence of recurrence. However, he has undergone gastrointestinal endoscopy once a year to detect early stage colon cancer which can be treated by endoscopic submucosal dissection in preventing surgical resection of reconstructed colon.\nDGE is one of the major postoperative complications after PpPD, and the incidence has been reported to be 22–45 % []. The main pathogenesis of DGE after PpPD has been thought to be preservation of the pylorus ring without innervation. Conventional PD and subtotal stomach-preserving pancreatoduodenectomy (SSPPD), in which the pylorus and duodenum are removed and more than 90 % of the stomach is preserved, has recently been reported to reduce the incidence of DGE compared with PpPD [–]. In this patient, a sliding hernia was detected by preoperative gastroendoscopy. If DGE happened after surgery, substantial gastric acid reflux to the esophagus was expected to lead to severe esophagitis. Simultaneous 24-h pH and bilirubin monitoring revealed the reflux of not only gastric acid but also bile, resulting in severe esophagitis (Fig. ). Conventional PD may reduce the risk of acid reflux because an antrectomy induces the reduction of the gastric acid secretion. Therefore, conventional PD or SSPPD with Roux-en Y anastomosis should have been selected to reduce the gastric acid secretion or the risk of DGE, and prevent bile reflux; otherwise, fundoplication such as Nissen and Toupet should have been added.\nIn this case, 24-h pH monitoring revealed that omeprazole could not sufficiently suppress gastric acid. The CYP2C19 genotyping test performed after the second operation showed that the patient had a rapid metabolizer genotype. Several studies have reported on the effects of CYP2C19 genotypic differences on PPI-mediated cure of GERD []. We suggest that the rapid metabolizer CYP2C19 genotype was one reason why extensive esophageal stricture happened so rapidly. In this patient, the decrease of white blood cell occurred due to the administration of PPI after the initial surgery and on the 24-h pH and bilirubin monitoring. We considered that PPI could not be administered after the second surgery due to the decrease in the white blood cell count caused by PPI. The results of simultaneous 24-h pH and bilirubin monitoring and the decrease of the white blood cell due to PPI could allow us to decide to perform total gastrectomy to protect the reconstructed organ and remnant esophagus from gastric acid. Simultaneous 24-h pH and bilirubin monitoring were very valuable in evaluating the pathogenesis of this case and choosing the operative procedure most likely to preserve the stomach.\nEsophageal stricture of GERD is generally treated by endoscopic balloon dilation and continuous PPI administration; surgical resection is rarely performed due to the associated high morbidity and mortality []. Herein, we considered less invasive procedure such as balloon dilation or temporary stent replacement; however, because these treatments were expected to cause perforation again and because this patient had medication-resistant GERD, we decided to perform esophagectomy. There were some problems regarding surgical procedures, including the approach, the reconstructed organ, and the reconstructive route. We thought that mediastinitis due to perforation during balloon dilation might scar the tissue surrounding the esophagus and noted that it was difficult to separate the esophagus form adjacent tissue. As the transhiatal approach requires a blind separation maneuver, the right transthoracic approach was chosen. Esophageal stricture extended the middle esophagus, and the esophagus was cut just beyond the bifurcation of the trachea. Alimentary tract reconstruction was performed using the transverse colon (not the jejunum) because of the shortage of useful jejunum due to Child reconstruction after PpPD. With respect to the route of reconstruction, we elected to perform intrathoracic anastomosis because the patient refused the percutaneous route due to the poor cosmetic consequences. Roux-Y anastomosis was also added to prevent bile reflux to remnant esophagus. In fact, postoperative simultaneous 24-h pH and bilirubin monitoring revealed little bile reflux to the remnant esophagus. |
A 26-year-old Thai woman presented with a foreign body in her left orbit that had been retained for 8 months. Eight months previously, she had sustained a gunshot injury to her left eye. There was only a small wound on her left eyelid (Fig. ); she had normal eye movement, a normal visual field, and no pain. A plain radiograph was performed at that time; it detected a round-shaped metallic foreign body located medially in her left eye globe. She was then diagnosed as having a retained foreign body in her left orbit and she was advised to have clinical observation. However, 8 months later, she developed pain in her left eye without any sinonasal symptoms. After discussion about the risk of surgery and retention of an orbital foreign body, an ophthalmologist referred her to our department for the minimally invasive procedure option of removal of the foreign body using an endoscopic transnasal approach. On examination, the movements of her left orbit were not restricted and there was normal visual acuity (20/20) with no proptosis or chemosis. A computed tomography (CT) scan of her left orbit revealed a round-shaped metallic foreign body in the medial intraconal space, and lateral attachment of posterior ethmoid sinus, measuring 6 mm (Fig. ). A transnasal endoscopic approach, with navigator assistance (Brainlab), was used to remove the bullet. Uncinectomy and anterior-posterior ethmoidectomy were performed. The location of the intraconal metallic foreign body was confirmed with a navigator system, then part of the lamina papyracea was removed and the periorbita incision was done. The defect was enlarged and the fibrotically encapsulated bullet was found lying in the orbital fat (Fig. ). The fibrotic capsule was dissected, and the bullet was delivered through her left nostril with probes and curetted (Fig. ). No intraorbital bleeding or damage to any soft ocular structures was noted. A relative afferent pupillary defect was found in her left eye during removal of the foreign body, but it resolved and no postoperative complications were observed. |
A 19-year-old Chinese adolescent complained that her left breast was larger than the right breast and she had been able to feel a mass in her left breast for 2 months. She was admitted to our hospital on February 19, 2017, with no history of trauma, nipple discharge, fever, or weight loss. On physical examination, enlargement of the left breast with one palpable mass was observed (Fig. ). The mass was about 6 × 6 cm, movable, solitary, firm, and well defined. It was not tender and did not adhere to the skin or the underlying structures. The local temperature of the overlying skin was normal. There was no axillary lymphadenopathy.\nBreast ultrasound demonstrated a homogenous, hypoechoic solid mass with no phyllodes. Mammography showed a large mass with high density and well definition (Fig. ). Based on the clinical and experimental findings, the mass was considered a giant fibroadenoma. Due to the history of progressive enlargement of the left-sided lesion, she underwent an excision biopsy instead of needle biopsy. To avoid a disfiguring scar, we planned to use the 8-gauge Mammotome to shrink the mass and then perform the lumpectomy through a tiny circumareolar incision.\nLocal anesthesia (200 mg of 2% lidocaine, 75 mg of ropivacaine, 0.5 mg of 1:1000 epinephrine, and 20 mL of sodium chloride injection) was given in the operation area. A 3-cm circumareolar incision was made, which allowed the surface of the tumor to be separated. Under ultrasound monitoring, the tip of 8 G Mammotome needle was placed in the center of the tumor. We first removed the inner tissue to reduce the tumor to an appropriate size and avoided to cut through the capsule (Fig. ). A purse string suture was made to close the portal of the Mammotome needle. After the inner tissue was removed, the diameter of the tumor was reduced to about 3 cm. Then, the tumor could be completely removed through the incision completely (Fig. ). There was no residual tumor remains. The operation time was <60 minutes and the mean blood loss was about 5 mL.\nPostoperatively, there was no pain, hemorrhage, or hematoma formation, and the patient's recovery was uneventful. The specimen was submitted to the pathology department, and the diagnosis of juvenile fibroadenoma was confirmed (Fig. ). The patient returned to the clinic 2 years later, with good cosmetic results and no recurrence (Fig. ). |
A 4-year-old girl presented to our casualty with upper abdominal pain, distension, and nonbilious vomiting for the last two days. Her mother gave a history of recurrent episodes of similar symptoms every month for the last six months, and each episode lasted for 4-5 h.\nThe symptoms used to diminish spontaneously. Her perinatal history was unremarkable. A preliminary work up for her past symptoms in another hospital revealed that she had a suspected left sided eventration as seen on the chest X-ray []. On examination, the child was lethargic, obtunded, had cold peripheries with tachycardia, hypotension, and feeble pulse. Her abdomen was distended, tense and tender especially in the upper half. A nasogastric tube insertion was attempted that failed initially but subsequently drained hemorrhagic gastric contents. A skiagram [] at this stage revealed a gastric bubble, with a paucity of distal bowel gas and a raised left hemi diaphragm.\nAfter adequate resuscitation, the child was explored, and the intra-operative findings revealed a mesoaxial gastric volvulus. After successful reduction, it was noted that the stomach was grossly healthy except the greater curvature, which was congested. There was a large defect in the posterolateral aspect of the left hemi-diaphragm, which was covered with a sac and the diaphragm had a reasonably well-developed rim of muscle along the anterior segment [].\nTo repair the defect the sac was excised and it was noted that the cephalic surface of the sac had a lobular 6 cm × 3 cm fleshy pedicled mass flimsily attached to it [].\nThe pedicle of the blood supply was traced and it was found to be entering the mediastinum. The pedicle was separate from the inferior pulmonary ligament. The lung was well developed and was anatomically unrelated to the mass. The pedicle was ligated, and the mass excised and sent for histopathological examination. The repair was completed by primary interrupted sutures, and a three-point gastropexy was done to prevent recurrences. The child recovered uneventfully and is currently under our follow-up. The histopathology report of the mass indicated that it was an extra-pulmonary BPS. |
A 55-year-old Japanese woman was referred to our hospital because of hepatic tumors detected by abdominal ultrasonography during a screening examination. Her medical and family history was unremarkable; her occupational and residential history showed no apparent exposure to asbestos. She received no medical drugs, and neither smoked tobacco nor drank alcohol. On admission, her general status was unremarkable; her temperature was 36.2 °C and her blood pressure was 110/70 mmHg with a regular heart rate of 80/minute. A physical examination revealed no abnormal findings; neurological examinations were unremarkable. All laboratory data, including levels of the serum tumor markers carcinoembryonic antigen, cancer antigen 19-9, alpha fetoprotein, protein induced by vitamin K absence or antagonist-II, and cancer antigen 125 were within normal ranges (Table ). An abdominal enhanced CT scan revealed four hypervascular, round hepatic tumors. One tumor, which was 32 mm in diameter, was in contact with the lateral liver segment and the hepatic hilus; the other three tumors were in contact with the diaphragm and measured 7 mm in segment 4, 17 mm in segment 7, and 15 mm in segment 8 (Fig. a–d). There was no pleural effusion or ascites, and no other nodular lesions in her chest or abdomen were observed. Similar to the CT findings, magnetic resonance imaging (MRI) revealed a tumor of lower intensity than the liver on T1-weighted images, and with higher intensity than the liver on T2-weighted and diffusion-weighted images (Fig. a–c). Upper and lower gastrointestinal endoscopic examination findings were normal. We hypothesized that the tumor arose from her liver, and diagnosed our patient as having multiple hepatic hemangiomas, at first. However, imaging findings were not typical of hepatic tumor: all tumors were on the edge of the liver and the tumor margins were clear. Moreover, the tumor in segment 7 appeared continuous with the diaphragm with similar high-density contrast medium (Fig. d). We then performed CT angiography to obtain further details.\nCT findings during arterial portography showed enhancement in none of the tumors (Fig. a and b), but CT during hepatic arteriography revealed strong enhancement in the tumor in the lateral segment and that this tumor was fed by the left gastric artery. In contrast, the remaining three tumors had no enhancement and were fed by the right inferior phrenic artery (Fig. c and d). Imaging findings indicated that the liver was not the origin of these tumors. Moreover, all tumors exhibited increased uptake of 18F-fluorodeoxyglucose, with a standardized uptake value of 7.8 with positron emission tomography (PET)-CT (Fig. ). We considered these tumors to be peritoneal malignant tumors, preoperatively, and performed surgical resection. Laparotomy revealed that the tumor in the lateral segment was clearly in contact with the hepatoduodenal ligament, and that the other three tumors were on the diaphragm (Fig. a–c). We performed tumorectomy with left liver lobectomy (Fig. ) and partial diaphragmatic resection. Histopathological and immunohistochemical examinations of the resected specimens were positive for CD34, CD31, D2-40, and calretinin, which confirmed epithelioid mesothelioma (Fig. a–h). Our patient was not given any medication except a general drip infusion and prophylactic antibiotics; she was discharged 10 days after surgery without complications. CT and PET-CT revealed no recurrence 20 months after surgery without adjuvant therapy. |
A fourteen-year-old girl presented with a one-year history of an asymptomatic slowly enlarging mass on the right side of her neck. There was no history of hoarseness, nasal regurgitation or associated pain, fever, or trauma. Her medical history was significant for recurrent tonsillitis in early childhood, but the mass was found to be unresponsive to over three courses of oral antibiotics before being referred to our specialist centre for evaluation. On examination, she had a large six-by-four centimetre immobile solitary right-sided neck mass deep to the right sternocleidomastoid muscle with a smooth regular border. There was no associated palpable cervical lymphadenopathy. The patient's oropharynx revealed no displacement of the peritonsillar structures, and a further physical examination was non-contributory. Routine blood tests including LDH were normal. Ultrasound showed a solid, well-circumscribed homogeneous mass arising from within the fascial planes with low level Doppler venous flow. The mass was displacing the great vessels to the right (). She went on to have an MRI ().\nThis patient was discussed at our joint radiology/surgical MDT, and a consensus was made to proceed with surgical resection of the mass. During surgery, there were two lymph nodes above the right internal jugular vein identified which were excised for histopathology. These were later confirmed to be reactive hyperplasia. The mass was identified posterior to the right internal jugular vein and mobilised with blunt and bipolar dissection medially off the carotid artery (), and laterally off the scalene muscles. It was arising from the cervical sympathetic chain. The nerve was divided to facilitate complete resection of the mass; however, the remainder of procedure was unremarkable.\nPostoperatively the patient developed anisocoria and ptosis of her right eye. A diagnosis of Horner's syndrome was made, the description of which forms a vital part of the informed consent process prior to surgical resection. The final histology report confirmed a palisaded spindle cell lesion consistent with a nerve sheath tumour (). |
A 55-year-old Chinese female with a history of hypertension was evaluated by the cardiologist. She had a normal pulse rate and a regular rhythm, and her blood pressure was 150/85 mmHg. No murmur or thrill was present. The chest radiograph was normal. Echocardiogram showed a mass in the right atrial free wall, measuring around 30 mm × 15 mm in diameter (Figure A). She was admitted to our hospital for further evaluation.\nTo obtain better definition of the mass, a 64-slice computed tomography (CT) was performed. An evaluation by means of CT demonstrated that the mass, which contained several thin septations, was consistent with lipoma. The mass occupied a part of the right atrial free wall (Figure B). But, we were uncertain whether the fatty tumor was epicardial, intramural, or endocardial.\nSurgical treatment was indicated. After routine median sternotomy, the patient was started on moderate systemic hypothermic (34°C) cardiopulmonary bypass with cannulation of the aorta ascendens and both venae cavae. The superior vena cava cannula was inserted very high in the superior vena cava so as to be away from the tumor. The inferior vena cava cannula was also inserted very low near the diaphragm. To reduce the risk of complications, the patients underwent open heart operation on beating hearts. After the right atrium was opened, there was a large, yellowish rubbery mass (32 mm × 17 mm × 7 mm) constituting part of the free wall of the right atrium (Figure A). After total tumor excision, the defect of atrial free wall was closed directly without any patch.\nGross examination revealed a 32 mm × 17 mm × 7 mm mass comprised predominantly of mature adipose tissue of an intramyocardial lipoma (Figure B). Histologically, the excised specimen showed many fatty structures lined by myocardium, suggesting that the tumor had indeed originated within the atrial wall (Figure C).\nThe patient recovered well on the ward and was discharged 8 days after the operation. On 1-year follow-up, the patient remained asymptomatic, with good clinical and echocardiography evaluations. |
A 60-year-old woman with a history of hypertension was admitted to a local hospital complaining of severe chest pain lasting for more than one hour. She was diagnosed as suffering from acute anterior MI and received streptokinase, but her symptoms persisted. She was then transferred to our hospital for cardiac catheterization. ECG showed sinus rhythm, elevated ST-segment with relevant inverted T waves in leads V1–V5, and pathological Q patterns in leads III and V1–V4, suggesting anterior wall MI. Transthoracic echocardiography (TTE) revealed akinesia of the apical and lower anterior ventricular septum with formation of an apical LVA, and ID located in the apical-septal segments (). Cardiac function was moderately dysfunctional (ejection fraction of 38%).\nCoronary angiography demonstrated that typical three-vessel lesions were also present including total median-distal occlusion of the left anterior descending artery without visible established collateral circulation, diffuse moderate-severe stenosis of the left circumflex artery, and mild narrowing of the right coronary artery, eliminating the opportunity for percutaneous coronary artery intervention. Biochemical blood test results revealed serum levels of high-sensitivity C-reaction protein and troponin of 14.79 mg/L and 2.26 ng/mL, respectively, significantly higher than the normal ranges. Considering that the optimal timing of emergency operation had passed (less than 6 h from initial acute event), and the progressive ischemic myocardial edema, exudation and inflammatory reaction, the effectiveness of surgery was considered to be low. Therefore, pharmacological treatments were commenced to stabilize the patient's condition during the preoperative period. Three months later, the patient was admitted to our cardiac surgery department for surgical treatment. Cardiac function was grade III according to the New York Heart Association Classification. Repeat TTE indicated that the ejection fraction was 45%. Unfortunately, ID had also expanded into the right ventricular chamber as a tortuous tract present at several horizontal levels, which led to VSR followed by a small left-to-right shunt (). Serum levels of all cardiac markers were normal. After full physical evaluation, the patient underwent cardiac surgery with beating-heart cardiopulmonary bypass grafting (CABG). CABG was performed first. ID and VSR were repaired second as follows. First, two traction sutures were placed at the apex. Second, a 2 cm incision was made between the two traction sutures parallel with the left anterior descending artery. Finally, 2-0 polypropylene sutures were passed through a strip of Dacron felt, the left ventricular ID entry point, apical septum, and the other end of the strip of felt, using a horizontal mattress pattern. The LVA was corrected in a similar way using 2-0 Prolene suture and two strips of Teflon felt in a continuous horizontal mattress pattern (). Linear plication paralleled the left anterior descending artery, eliminating the chance of an abnormal contracting scar and restoring normal ventricular size and shape. Postoperative TTE confirmed disappearance of the echo-free space at the dissecting cavity, and no shunting. The patient recovered uneventfully without surgical complications and was asymptomatic at discharge. During a follow up of 6 months, patient has been free of angia pectoris. |
A 64-year-old man with an underlying diabetes mellitus type 2, hypertension and erythrodermic psoriasis which is under control with medications presented with progressive bilateral lower limb weakness for 1-week duration. The weakness was extended up to the knee level and caused him difficulty to walk. He denies any numbness, sciatica or lower back pain. There was no preceding history of fall or trauma and no symptoms suggestive of hyperthyroidism. His list of medications includes oral metformin 500 mg once daily, oral losartan 50 mg once daily, oral nifedipine 10 mg three times per day and oral methotrexate 5 mg weekly. He denies taking any over-the-counter nor traditional medication and only drinks alcohol occasionally. On physical examinations, his vital signs showed a low-grade fever with a temperature of 37.7°C, blood pressure of 148/86 mm Hg, pulse rate of 74 beats/min and respiratory rate of 20 breaths/min. Examination of the lower limb revealed weakness over the knee and ankle bilaterally with the muscle power of 3 over 5. The muscle power over the hips and upper limbs was normal. His reflexes were normal, there were no cerebellar signs present, and all his sensory modalities were intact. Examination over the neck region noted there was a diffuse swelling that was more prominent over the right side, which was not tender, firm in consistency, and move upward with swallowing but not with protrusion of the tongue. His abdomen was soft, not tender, and there was no palpable mass or organomegaly. Examinations of the cardiovascular and respiratory systems were normal.\nInitial blood investigations were taken and showed marked hypokalaemia. Otherwise, blood pH, random blood glucose, other electrolytes, and renal and liver function were normal (as shown in ). In view of the goitre, his thyroid function test was tested and reveals subclinical hyperthyroidism with the thyroid-stimulating hormone level of 0.30 IU/mL (normal range: 0.5–4.95 IU/mL), free T4 level of 17.05 pmol/L (normal range: 9–19 pmol/L) and free T3 level of 1.28 nmol/L (normal range: 1.08–4.14 nmol/L). The ECG shows sinus rhythm with no prolonged QT interval, no U-wave and no acute ischaemic changes.\nHe was diagnosed with acute lower limb paralysis secondary to hypokalaemia and was treated with 2 g of potassium chloride in 200 mL of 0.9% saline via intravenous infusion over 2 hours. His muscle power improves, and the repeated serum potassium level was 2.9 mmol/L. He was then started on oral potassium chloride 1.2 g three times per day thereafter. Throughout his admission, his potassium level was persistently below 3.0 mmol/L despite on oral potassium supplements. His 24-hour urine potassium was normal (12 mEq/24 hours, normal range: <15 mEq/24 hours). A CT scan of the neck and thorax was done and showed diffuse goitre with a retrosternal extension over the right side (as shown in ). He was subjected to ultrasound-guided fine-needle aspiration for cytology of the thyroid, which reveals cystic contain with no malignant cells. His anti-thyroglobulin antibody was negative. In view of persistent hypokalaemia despite taking regular oral potassium replacement with the background of normal blood pH and urine potassium level, HPP secondary to subclinical hyperthyroidism was suspected. |
A 54-year-old gentleman presented with a 7-year history of right neck swelling which had progressively increased in size without compressive or infiltrative symptoms. He complained of a pulling discomfort exerted by the weight of the mass, accentuated by gravity in the erect position. He denied history of malignancy in the family or exposure to radiation. On examination, he was clinically euthyroid. The 10 × 10 cm swelling was located at the right anterior triangle of the neck extending from the submandibular region to the level of the cricoid cartilage and medially not crossing the midline (Fig. ). The swelling was non-tender, soft in consistency and had a smooth lobulated surface. It did not move with deglutition or tongue protrusion and there were no changes to the overlying skin. Biochemical thyroid function was within normal limits. Ultrasonography revealed an ill-defined, homogenous, hyperechoic mass arising from the right submandibular space. Magnetic resonance imaging (MRI) showed a atypical lipomatous tumour with a deep extension into the right parapharyngeal space (Fig. ). A fine needle aspiration cytology (FNAC) was performed but results were inconclusive. Therefore, we decided to proceed with surgical excision without further attempts to obtain preoperative biopsy in order to reduce the risk of tumour seeding along the biopsy tract and also because the lesion looked well encapsulated on MRI except at the right parapharyngeal fossa region.\nA multidisciplinary team discussion between general surgery, radiology and otorhinolaryngology team was undertaken to discuss the MRI images and possible approaches to excise this lesion. The patient subsequently underwent excision of the lesion with the otorhinolaryngology team on standby for radical surgery if findings were suggestive of malignancy. Intraoperatively, a single, lobulated, lipomatous tumour measuring 10 × 12 cm in size with multiple engorged vessels on its surface was found to be located at the right parapharyngeal fossa (Fig. a - c). It was well-encapsulated with minimal adhesions to surrounding structures except at the superior part which was densely adhered to a part of the right submandibular gland. The right submandibular gland was excised en-bloc as it was most likely the origin of the tumour (Fig. d). There were no enlarged loco-regional lymph nodes to suggest malignancy. A small calibre vacuum drain was placed at the submandibular fossa to prevent sialocoele for a day before removal. The patient had an uneventful recovery and was discharged home on day 2 with no complications upon follow up.\nHistopathological examination revealed a fairly circumscribed lesion composed of lobules of various sizes and mature univacuolated adipocytes separated by fibrous septae (Fig. ). It measured 9.5 × 12.5 × 8.5 cm. There were multiple congested blood vessels of varying sizes and nodules composed of benign salivary gland ducts and acini with foci of squamous metaplasia. No fibrin thrombi were seen. An unremarkable salivary gland tissue was identified with no microscopic evidence of malignancy. No atypical stromal cells or lipoblasts were seen. These features were consistent with a benign adipocytic tumour of sialolipoma variant. During follow up at 1 month post-operatively, he was well without any evidence of complications or recurrence. |
The patient has provided informed consent for publication of the case, and the study design was approved by the Shengjing Hospital of China Medical University Institutional Review Board (IRB No.1698342)\nA 73-year-old woman was diagnosed with iNPH after presenting with memory impairment and gait disturbance for more than 1 year. A head computed tomography (CT) scan indicated ventricular enlargement and obvious paraventricular edema (Fig. ). The Tap test was positive, and she was indicated to receive a VPS using a Braun shunting system (proGAV + gravitational unit SA) (Fig. ). With the operating pressure set at 2 cmH2O, she achieved clinical improvement within 1.5 years. Two months before her second admission, the patient suffered from drowsiness and was unable to walk. Her family found that she presented with poor appetite and was bloated. Chronic cholecystitis was confirmed through abdominal CT, which showed flatulence and a swollen, enlarged gallbladder (Fig. ). After anti-inflammatory treatment, the patient's condition was stable, but her state of consciousness did not improve. A physical examination performed at the second admission revealed that she was in deep coma, was unresponsive to pain stimuli, and was unable to move her extremities. Abdominal distension was remarkable without tenderness. Relative to the CT findings obtained upon her discharge, a head CT scan indicated an enlarged ventricular system and paraventricular edema (Fig. ). While these findings seemed to indicate that the opening pressure of the shunt did not match the intracranial pressure (ICP), the condition of our patient did not improve even after the operating pressure was decreased to 0 mmH2O.\nAfter we identified the absence of an underlying infection, laparoscopic cholecystectomy was performed under general anesthesia 7 days following drainage from the reservoir. Intraoperative exploration revealed the shunt tube to have been partially covered and severely curled by the omentum majus around the gallbladder (Fig. ). We also observed elevated pelvic effusion but normal – albeit slow – cerebrospinal fluid outflow. The gallbladder was carefully and completely removed without bile leakage or bleeding. Antibiotics were administered regularly as suggested by the general surgery expert. The patient maintained wakefulness and could speak a few words with slowed speech. Her memory and cognitive ability were slightly impaired without a positive sign in the abdomen. A head CT scan showed the attenuation of ventricular enlargement and paraventricular edema (Fig. ). No catheter or abdominal infection signs were observed during the subsequent 3 months of follow-up. |
The case was of a 95-year-old man with a history of hypertension and dyslipidemia who was on hydrochlorothiazide and simvastatin. He presented to our hospital with abdominal pain and frequent vomiting. The patient was suffering from longstanding constipation. He used to habitually insert a rubber hose into his anal canal and use a hydrostatic water tap to relieve his constipation. He gave a history of performing this habit 24 hours before his presentation.\nThe patient was distressed with a temperature of 36.5°C, a respiratory rate of 30 per minute, a heart rate of 130 per minute, and blood pressure of 130/65 mmHg on physical examinations. Abdominal examination revealed a tender, non-distended abdomen with guarding allover. A soft stool with an enlarged, firm prostate was revealed during a digital rectal examination.\nIntravenous fluid, analgesia, and broad-spectrum antibiotics were used to rapidly resuscitate the patient. The patient could not tolerate standing for an upright chest X-ray, and a supine abdominal X-ray showed a suspicion of free air ().\nHe was found to have a low white blood cell count of 1.5 × 103/μ L, a hemoglobin level of 10.2 g/L, a platelet count of 194 × 109/L, and an international normalized ratio of 1.4.\nThe diagnosis of bowel perforation with fecal peritonitis was made. Various causes were considered including stercoral perforation. However, based on the clinical context, self-induced hydrostatic colonic irrigation was the most likely cause of perforation.\nThe patient was stratified as an ASA IV and taken to the operating room at high risk of death and perioperative complications. An emergency midline laparotomy was performed, which revealed fecal matter all over the abdomen and a 2-cm free perforation of the sigmoid colon in addition to multiple diverticula in which some of them were included in the tear ().\nAbdominal washout and Hartmann’s procedure were carried out. He was shifted to the intensive care unit for critical continuous monitoring. Unfortunately, the patient died of multi-organ failure two days after surgery.\nPathological examination of the resected sigmoid colon confirmed the presence of sigmoid diverticula with a 2-cm tear. |
A 79-year-old man presented with high-grade muscle-invasive bladder cancer and underwent robot-assisted laparoscopic radical cystoprostatectomy with urethrectomy, extended pelvic lymph node dissection, and intracorporeal urinary diversion. One week after surgery, clear fluid started leaking from the external urethral orifice. The leakage output was 300 mL per day. CT revealed a medium amount of ascites. Biochemical examination of the ascitic fluid was suggestive of lymphorrhea. A low-fat diet was started, but the leakage output did not decrease. Two months after surgery, intranodal lymphangiography was performed to detect the site of leakage and reduce the amount of lymphatic leakage. Under local anesthesia, bilateral inguinal lymph nodes were punctured with a 23-gauge cathelin needle under ultrasound guidance (). Lipiodol was injected slowly under fluoroscopy, showing a network of lymphatic vessels in the pelvis. Lymphatic leakage from the left pelvic wall into the peritoneal cavity was detected (). Although the therapeutic effect of lipiodol lymphangiography was expected, the amount of lymphatic leakage did not change after lymphangiography. Thus, laparoscopic surgical repair of lymphorrhea was performed two weeks later. During the repair, a dent was found on the left wall of the pelvis just above the external iliac artery, from which the serous fluid slowly leaked into the peritoneal cavity. Although the dent was suspected to be the lymphatic leakage point, the laparoscopic findings alone were not conclusive. We decided to perform intraoperative lymphangiography to confirm that the dent was the leakage point and to exclude other sites of leakage. A left inguinal lymph node was punctured under ultrasound guidance and lipiodol was slowly injected, showing leakage from the left wall of the pelvis. Then, indigo carmine diluted 10-fold with normal saline was slowly injected. In total, 1 mL of lipiodol and 1 mL of diluted indigo carmine were used. Three minutes after administering lipiodol injection, laparoscopy revealed extravasation of lipiodol and indigo carmine from the dent (Figures and and Supplementary Video). We also confirmed the absence of other leakage sites. The lymphatic leakage point on the left wall of the pelvis was close to the external iliac artery, and suturing the leakage point was a risk factor for vascular injury. Thus, the leakage point was cauterized using an electric scalpel. Additionally, a defect was observed at the pelvic floor region that was sutured at the completion of the previous surgery, and the lymph was considered to have leaked from the external urethral orifice through the defect. The defect was closed with 3-0 Vicryl sutures. No complications were observed. After surgical repair, lymphatic leakage from the external urethral orifice was no longer observed. Abdominal ultrasound revealed a decrease in ascites. One month later, the patient was discharged home. |
A 65-year-old immunocompetent man presented to the respiratory clinic with recurrent chronic productive cough and gradual loss of weight for 2 years. He denied any fever, haemoptysis, urinary symptoms, or contact to any patients with pulmonary tuberculosis. His initial vital signs were unremarkable (blood pressure = 130/70 mmHg, pulse rate = 76 beats per minute, oxygen saturation = 98% at room air, and temperature = 36.5°C). At the first encounter, he was underweight with a BMI of 17 kg/m2. On examination, there was reduced air entry in the left upper lung. The abdomen was soft and nontender. His sputum sample was sent for tuberculosis workup; however, the results were negative. Routine blood investigations including full blood count and urine profile were within normal limits. The inflammatory makers were not elevated with a white cell count of 11 g/dL. His past medical history included benign prostatic hyperplasia and remote history of healed scrotal abscess.\nThe initial chest radiograph revealed fibrotic lung changes in the upper zones, more so on the left than the right. Subsequently, a contrast-enhanced computed tomography (CT) of the thorax with high-resolution CT reconstruction was performed and showed extensive scarring in both apical regions, again more so on the left. In addition, there were nonconfluent clusters of micronodules () in the right lung with a few smaller regions of tree-in-bud nodularity. There was no significant mediastinal or hilar lymphadenopathy. In the upper part of the abdomen included in the CT, there were also incidental findings of the partial duplex system of the left kidney with mild-to-moderate hydronephrosis in the lower moiety with proximal hydroureter (). To further investigate this urinary finding, a multiphasic renal CT scan was then performed and did not reveal any calculus, suspicious ureteric mass or external compression from lymphadenopathy. The culture of his urine sample grew mycobacterium tuberculosis (MTB) complex, and he was diagnosed with active genitourinary and pulmonary tuberculosis. He was commenced on antituberculous medication (Akurit-4). His symptoms improved, and he started to gain weight. A repeated CT scan at 4 months revealed significant improvement of the aforementioned clusters of micronodules, tree-in-bud changes, and left hydronephrosis. Given the positive urine culture for tuberculosis with concurrent respiratory and constitutional symptoms, and improvement of the lung changes and respiratory symptoms following antituberculous treatment, the above-described nonconfluent clusters of micronodules were likely to represent active pulmonary tuberculosis. |
A 56-year-old male with a history of left testicular cancer treated with chemotherapy (Bleomycin, Etoposide, Cisplatin; BEP), radiotherapy, retroperitoneal lymph node dissection, and partial left nephrectomy in 1992 presented to hospital with complaints of left lower abdominal mass for 3–5 years, associated with ulceration and active drainage. He was asymptomatic with β-hCG and alpha-fetoprotein (AFP) levels within reference ranges (<2.39 and 1.4, respectively). On examination, inspection of abdomen revealed well-healed midline scar with bilateral incisional hernias and a 10 × 8 cm mass in the left lower quadrant () firm and fixed to the abdominal wall. An open lesion characterized by yellow, non-purulent drainage was noted in the left lower quadrant. CT imaging of abdomen and pelvis with contrast revealed a multiloculated large cystic mass in the left inguinal canal measuring 11 × 7 × 7.7 cm () along with a similar soft tissue nodule in the right inguinal area. A right retroperitoneal soft tissue mass in the mid abdomen was also identified, measuring 3.3 × 4.2 × 5.7 cm (). Changes of prior left nephrectomy and orchiectomy consistent with treatment of previous left-sided testicular cancer were also noted.\nBiopsy of right retroperitoneal mass revealed squamous cell-lined cystic lesion followed by left lower quadrantabdominal mass excision and complex wound closure. An elliptical incision was made in the left groin incorporating the lesion, and subcutaneous flaps were created. Dissection was carried down to fascia where the mass extended into inguinal ring and the lower edge of the external oblique aponeurosis, extending into the abdomen. The mass was excised and noted to be multi-loculated and heterogeneous in consistency. The fascia and peritoneum that was adhered to the mass were then resected. A superficial inguinal lymph node was excised and sent to pathology as a separate specimen, and no other inguinal or femoral enlarged lymph nodes were found. Final pathology results revealed a mucinous cystic neoplasm with a negative lymph node for malignancy, consistent with potential spermatic cord mucinous cystadenoma (). The presence of retroperitoneal and contralateral inguinal masses, however, with history of teratoma raised suspicion of bilateral mucinous cystadenoma vs. residual/recurrent teratomatous component of testicular tumor. With this patient’s history and laboratory findings, diagnosis of recurrent teratoma was favored. The patient was asked to follow up with surgery two weeks after the procedure. At follow-up, patient denied any new onset of symptoms and was deemed to be experiencing appropriate postoperative recovery. |
This is a case report of a 77-year-old male patient who presented with generalized weakness, confusion, somnolence, recurrent falls and tea-colored urine for 3-4 days. His past medical history was significant for hypertension, chronic obstructive pulmonary disease and coronary artery disease with a remote history of MI. Physical examination revealed a confused and lethargic elderly male. His vitals showed blood pressure 97/42, heart rate of 51, respiratory rate of 12 and oxygen saturation of 93% on room air. There were extensive ecchymoses in different stages of healing on all 4 extremities and on the torso. Laboratory data were consistent with rhabdomyolysis and acute renal failure. ECG upon presentation revealed sinus rhythm with no abnormalities [].\nThe patient was rehydrated with intravenous normal saline and nephrotoxic medications were withheld. His renal function improved and on the 2nd day, his chest X-ray revealed patchy bilateral infiltrates. On the 3rd day, he developed respiratory distress with an increase in oxygen requirements. The chest computed tomography revealed bilateral ground glass opacities with consolidation consistent with acute lung injury/acute respiratory distress syndrome. After failing a trial of non-invasive positive pressure ventilation, he was intubated and mechanically ventilated for hypoxic respiratory failure. He developed intermittent supraventricular tachycardia, atrial flutter and atrial fibrillation were treated with intravenous esmolol and diltiazem after which he converted to sinus rhythm. On the 4th hospital day, he developed increasing oxygen requirements and asynchronous volume control ventilation; his ventilator settings were switched to pressure control ventilation. An echocardiogram revealed left ventricular ejection fraction of 45-50% with grade II diastolic dysfunction and no wall motion abnormalities. Patient developed fever and broad-spectrum antibiotics were initiated. He later became progressively hypoxic and tachypneic on the ventilator during the next couple of days. He was paralyzed using vecuronium. His clinical status worsened and his ventilator settings were changed; the positive end-expiratory pressure (PEEP) was increased to 13 mm Hg. ECG at the time showed ST segment and T wave changes concerning for ST elevation MI. Careful review of the ECG revealed bizarre, broad-based apparent ST-segment elevation in the precordial leads where the upward shift of the baseline started slightly before the onset of the QRS complexes. A more obvious dome and spike pattern was seen in the inferior leads []. These findings are not consistent with true ST-elevation MI. His central venous pressure decreased from 12 to 7 mm Hg and tidal volumes were increased from 550 to 750 cc during this period. An urgent bedside echocardiogram showed preserved left ventricular ejection fraction with no wall motion abnormalities. The ventilator settings were changed and the PEEP was discontinued. Following this change, the ECG no longer showed the findings noted above []. The patient passed away on the 11th day of hospitalization from acute respiratory distress syndrome. |
The 70-year-old male patient was admitted to the Department of Neurology with isolated left upper extremity weakness and clumsiness. He had wrist drop and was unable to grip with the fingers. He complained of no sensory loss or numbness. His past medical history included long-term oral anticoagulation due to recurrent lower extremity deep vein thrombosis, glaucoma, and a non-significant aortic valve stenosis, with vascular risk factors including a 5-year history of treated hypertension, hypercholesterolemia, and a history of non-significant stenosis of the left anterior descendent coronary artery. On admission, no abnormality could be detected by physical examination, apart from the neurological signs, which included a wrist drop on the left side with decreased grip strength (Fig. ). Pronation and wrist dorsiflection were lost, as well as the ability to form a ring with the thumb and the index. The reflexes of the left upper extremity were slightly brisk, with not pathological reflexes present and no sensory deficits. Laboratory parameters were without abnormal findings except for a slightly elevated fasting blood glucose level. Cranial CT revealed contrast-enhancing, irregularly shaped lesions with diameters of 7, 10, and 9 mm (in the temporal, parietal, and frontal lobes, respectively), surrounded by perifocal edema. Carotid duplex ultrasonography did not demonstrate signs of focal atherosclerotic plaques, circulatory disturbance or stenosis on either side. In accordance with the findings of the CT scan, the cranial MRI confirmed the tumor in the right precentral gyrus, corresponding to the ‘hand knob’, accompanied by further similar lesions posteriorly in the parietal and the temporal lobes, and in the left frontal lobe (Fig. ). Electromyography and nerve conduction studies did not support a peripheral origin underlying the paresis. The neuropsychological examination revealed a deficit in the Luria three-step test as a single alteration, which performance could, however, be remarkably improved by verbal clues. Chest X-ray performed as part of the search for primary tumor revealed no abnormality. Non-contrast and contrast-enhanced chest CT detected an irregularly shaped mass in segment 10 of the left lung, with inhomogeneous enhancement of the contrast agent and multiple associated lymph node conglomerates, suggesting lung cancer as the primary tumor. Abdominal ultrasonography did not detect malignancy. The patient received palliative steroid therapy, with no improvement in the paresis during the observation period. Based on the recommendation of the tumor board, the patient was transferred to the Department of Pulmonology for bronchoscopic tissue sampling. The verification of the diagnosis by this means, however, was unsuccessful. The diagnostic process had to be suspended due to an acute bleeding duodenal ulcer, and the patient received blood transfusion and local hemostatic treatment in the Department of Surgery. In association with the antibiotic treatment, the patient developed pseudomembranous colitis caused by Clostridium difficile. In addition to the persisting colitis, fever occurred accompanied by hemoptysis and coughing. The subsequent chest X-ray did not confirm pneumonia. Despite the applied antibiotic and oxygen therapy, respiratory failure developed, and the patient passed away 2 months after the onset of the neurological symptom. The autopsy and the histopathological analysis identified an adenocarcinoma (mucinous tubular adenocarcinoma with solid anaplastic parts) both in the brain and the lung; (Fig. ). |
A 44-year-old man presented to his primary care provider for a right proximal clavicular mass and enlarging neck for six months that he attributed to an injury from Jujitsu. The patient endorsed mild pain in the area but denied dysphagia, dysphonia, choking, or shortness of breath. The patient reported 20 lbs of weight loss. He denied hypoesthesia or motor deficits. On physical examination, a firm 8 cm mass was fixed to his right clavicle. There was palpable thyromegaly, the right lobe more prominent than the left, but there was no cervical lymphadenopathy and no significant motor or sensory deficits. Initial workup with an X-ray of his right clavicle revealed a large soft tissue density mass extended to the midline of the right proximal clavicle. A subsequent soft tissue neck ultrasound confirmed a 5.4 × 3.6 cm mass extending from the thyroid with features of increased vascularity and calcification extending from the thyroid (). CT of the neck showed extension of the mass into the adjacent sternoclavicular junction with osteolysis of the middle third of the clavicle and the superior aspect of the sternum body (). Due to suspicion of thyroid malignancy versus osteosarcoma, fine-needle aspiration of the mass was completed that showed a cohesive nest of neoplastic cells, and the nuclei were enlarged with pleomorphism. Focal calcification was noted, but there was no evidence of colloid or macrophages on multiple passes. Immunohistochemical stains were performed on the cell block, and the neoplastic cells were immunoreactive for TTF-1, PAX8, CK7, and thyroglobulin, and they were negative for CK20, calcitonin, synaptophysin, or chromogranin (). The overall findings supported the diagnosis of papillary thyroid carcinoma. To assess the extent of the mass for total thyroidectomy, a chest CT scan with contrast confirmed marked enlargement of the right thyroid gland, measuring 5.3 × 7 × 4.9 cm, with invasion into the right proximal clavicle, adjacent strap muscles, and first rib as well as tracheal deviation and extension into the mediastinum.\nThe oral and maxillofacial surgery team performed a composite resection of the tumor, including a segmental osteotomy of approximately two-thirds of the medial clavicle. In continuity with the suprasternal and clavicular portion of the mass, the cardiothoracic surgery team performed a sternotomy to access and resect the substernal component, dissecting it off the first rib and manubrium without a segmental osteotomy. The great vessels were dissected and preserved with dissection of the mass off the superior mediastinum and thoracic inlet. The maxillofacial surgery team continued cephalad to perform a total thyroidectomy. Left levels 2–4, right levels 2–5, and bilateral central compartment selective neck dissections were completed. The mass was then delivered en bloc. The right sternocleidomastoid and strap muscles were sacrificed due to tumor involvement, but all other structures were dissected and preserved, including bilateral recurrent laryngeal nerves and the brachial plexus ().\nPostsurgically, the patient's serum calcium was low 7.9 mg/dL with simultaneously low PTH 9 pg/mL and low 25-hydroxyvitamin D 16.8 ng/mL. Thyroglobulin was markedly high at 15655.0 ng/mL (confirmed on dilution), and thyroglobulin antibody <1.0 IU/mL. Pathological examination of the right thyroid and clavicle showed PTC with extrathyroidal extension and clavicle involvement (staged pT4a pN0) with margins and lymph nodes negative for carcinoma (). The tumor showed a predominantly solid pattern with areas of the follicular pattern, but there were no cribriform areas identified. Immunohistochemical stain for beta-catenin showed no evidence of nuclear staining, which is not compatible with the morular pattern of papillary thyroid carcinoma. Next-generation sequencing revealed KRAS Q61 R mutation, and the following mutations were not detected: BRAF, HRAS, TP53, PTEN, TERT, RET, NTRK, and PPARg. The left thyroid showed benign pathology. Due to the perceived need for treatment with radioiodine therapy, the patient was not discharged home on levothyroxine, but was given supplemental calcium and vitamin D with subsequent follow-up with endocrinology.\nPostoperatively, the patient's course was complicated by a large expanding left neck hematoma that developed after a fall at home. The patient was readmitted and underwent an emergent hematoma evacuation without further incidence. He was able to follow up with endocrinology with laboratory studies at that time showing serum calcium was 8.6 mg/dL, TSH > 150.00 mIU/L, and free T4 0.2 ng/dL. He was started on calcitriol 0.5 mcg daily), vitamin D 50,000 units weekly, and calcium 1000 mg 4 times daily. Levothyroxine was held at this time with a plan for radioactive iodine treatment. On follow-up, an iodine-123 whole-body scan noted increased uptake over the lower lumbar spine that was suspicious for skeletal metastasis. A positron emission tomography (PET) scan confirmed a high fluorodeoxyglucose (FDG) avid lytic lesion along the posterior element of S2 with a maximum standardized uptake value (SUV) of 13.32. Magnetic resonance imaging (MRI) also revealed an enhancing sacral mass measuring 3.4 cm × 3.3 cm × 2.7 cm at the level of posterior elements of S1 extending to the sacral canal, causing severe sacral canal stenosis. There was a mass effect upon the traversing bilateral S2 nerve roots. These findings were consistent with metastatic disease. |
A 29-year-old female presented to the surgical outpatient department (OPD) at our hospital with the complaint of a lump in both the breasts for 7 days associated with pain. The lumps were felt around the nipple, tender to touch with the overlying skin warm. Pain was progressive in nature, sharp and shooting type. There was intermittent discharge of puslike substance from the nipples, milky in consistency. She complained of fever for the past 1.5 months. Fever was high grade with a peak of 39°C and touched baseline only with the intake of antipyretics. She also had occasional episodes of loose motions in this 1.5-month period. Two weeks prior to the development of breast pain and lump, the patient had visited the medical outpatient department with the complaint of fever. She was empirically started on amoxicillin plus clavulanic acid 625 mg bid for 7 days, but did not respond and continued to have fever. Although the blood culture had grown Salmonella typhi and the isolate was resistant to ampicillin, she did not visit the hospital for follow-up and so the treatment could not be modified accordingly. Her personal and family history was unremarkable. She was a mother of two children, but at presentation she was neither pregnant nor lactating.\nOn examination, the patient was afebrile but her pulse rate was 90/min. There was no organomegaly or macules on skin. Rest of the general physical and systemic examination was unremarkable. There was a centrally located lump in the left breast measuring around 8 × 8 cm and another lump in her right breast just below the areola measuring around 3 × 3 cm. A palpable axillary lymph node around 2 cm in size was present on the left side. Pus was drained from both the sides, which on culture grew non-lactose-fermenting colonies. The organism was identified as Salmonella typhi by biochemical reactions and agglutination test with specific antisera. The isolate was sensitive to chloramphenicol, cefotaxime, ciprofloxacin, and co-trimoxazole but resistant to ampicillin and nalidixic acid. The patient had a report of a positive blood culture for Salmonella typhi from our laboratory 2 weeks prior to the presentation, which had a similar sensitivity pattern. The Widal test showed a negative result with the titer of TO=160 and TH and AH=40. Her hemogram revealed normal values [haemoglobin (Hb): 10.4 g/dl, total leukocyte count (TLC): 10,000/mm3, differential leukocyte count (DLC); neutrophils: 80%; lymphocytes: 18%; eosinophils: 2%]. Biochemistry investigations were also within normal limits except that her serum glutamic oxaloacetic transaminase (SGOT) and serum glutamic pyruvic transaminase (SGPT) were raised with values of 96.59 and 113.78 U/l, respectively. The patient was started on oral ciprofloxacin 500 mg bid for 2 weeks to which she responded favorably with regression of the lesion. The stool and urine cultures were negative for Salmonella typhi during presentation and subsequent visits. |
A 68-year-old male was admitted to hospital with a history of dyspnea at rest for 2 d, a cough producing mucus, loss of appetite and diarrhoea. Pneumonia was suspected, and the patient received intravenous antibiotic treatment. During the patient's second night in hospital, he was found groggy and walking near his room by the nurses. They took him back to his room and connected a new antibiotic dose to his venous catheter. A few hours later, he was found unconscious, lying on the floor next to his bed. His peripheral venous catheter was connected to a nasal cannula delivering O2 from the wall, with a flow rate of approximately 2 L/min, and the antibiotic dose was found in the garbage can. As post-mortem lividity was present, no resuscitation attempts were performed by the clinicians and the death was pronounced. The body was immediately brought to the University Center of Legal Medicine (Lausanne, Switzerland).\nBefore any manipulation of the corpse, a native CT scan was carried out at around 10 h post-mortem using an eight-row CT unit (CT LightSpeed 8, GE Healthcare, Milwaukee, WI). All scanning parameters are detailed in . A forensic pathologist immediately viewed the native CT images. To evaluate the distribution of gas because of physiological changes in the body after death, the radiological alteration index (RAI) was used as proposed by Egger et al. []. The RAI was based on the analysis of samples from seven sites (heart cavities, liver parenchyma and vessels, left innominate vein, abdominal aorta, kidney parenchyma, L3 vertebra and the subcutaneous pectoral tissues).\nAll images were interpreted in a consensus reading by one board-certified radiologist and one forensic pathologist who were trained in forensic imaging. A post-mortem radiological report was prepared and described all findings from the native CT scan. The native CT scan revealed the presence of subcutaneous, intramuscular and intravascular gas, a pneumoperitoneum, a bloated heart with the right cavities filled with gas and a left pneumothorax ((a–d)). The maximum RAI score is 100, and RAI scores greater than 50 are usually seen in cases of severe changes []. Therefore, given the short post-mortem interval (<12 h), the elevated RAI obtained in this case (75) strongly suggests an exogenous source of gas in the tissues.\nCT-guided gas samples were immediately taken from multiple sites (carotid artery, jugular vein, right auricle, thoracic aorta, pectoral muscle, thoracic cavity, abdominal cavity, scrotum and gluteal soft tissues, (e,f)) according to the protocol for gas analysis described by Varlet et al. [].\nThe external examination revealed massive subcutaneous emphysema with audible crepitations on the entire surface of the body. Additionally, there was a bruise on the left part of the forehead, a small contusion near the left eyebrow surrounded by a purplish-blue bruise and some bruises of different ages on the inferior part of the thorax and on the legs. On the left arm, a venous catheter was still in place.\nSamples for toxicological and biochemical investigations (blood and urine) were collected in S-Monovette® tubes with sodium fluoride or ethylenediaminetetraacetic acid as a preservative (Sarstedt, Nümbrecht, Germany). Biological samples were collected as soon as possible on arrival of the body at the morgue (vitreous humor) and during autopsy (femoral blood, pericardial fluid and urine). During sampling of femoral blood by incision with a scalpel, multiple gas bubbles were visible in the blood ((a)).\nToxicological analysis of the collected samples revealed acetone at a physiological level in the blood, as well as caffeine and paracetamol in both blood and urine. Post-mortem chemistry analysis of the serum samples collected during the autopsy revealed elevated values of C-reactive protein (175 mg/L), consistent with an inflammatory state and a procalcitonine level compatible with a bacterial infection (0.69 μg/L). The results also revealed signs of cardiac dysfunction with a very high level of N-terminal prohormone of brain natriuretic peptide (>35 000 ng/L) and cardiac necrosis with a very high troponin-T level (>56 000 ng/L).\nThe autopsy was performed by one board-certified forensic pathologist and one forensic pathologist in training. Opening of the thoracic cavity revealed numerous gas bubbles in the fatty tissue covering the heart. The pericardium was opened and filled with water, and the heart floated. An incision was made in the right ventricle with a scalpel, and blood with gas bubbles escaped. This technique was performed mainly for training purposes and not for gas sampling, as the gas from the cardiac cavities had already been sampled during CT imaging. The autopsy revealed the presence of numerous gas bubbles throughout the vascular system, even in the small vessels of the brain ((b)). Additionally, changes to the pulmonary parenchyma were consistent with pneumonia. No other major findings were made.\nAn Agilent 6890N GC (Agilent Technologies, Palo Alto, CA) combined with a headspace gas autosampler and equipped with an Agilent Select Permanent Gases column arrangement was used. This column arrangement is specially designed for gas analysis and contains a molecular sieve 5 Å PLOT capillary column (10 m × 0.32 mm i.d.) and a Porabond Q column (50 m × 0.53 mm i.d.) in parallel, which allows for separation of carbon dioxide (CO2). The column temperature was maintained at 45 °C for 13 min. The injector temperature was 100 °C, and the injection was conducted in splitless mode. Helium was used as the carrier gas at a constant flow rate of 8 mL/min. The gas detection and quantification were performed with a thermal conductivity detector set at 150 °C. The system was calibrated for each gas with standard gases of H2S (Multigas, Domdidier, Switzerland), O2 and N2 (from laboratory air), and CH4 and CO2 (Carbagas, Lausanne, Switzerland). With this system, all the gases could be detected in the same run. The gas compositions for the different intracadaver sampling sites are displayed in .\nSamples of the brain, heart, lungs, liver and kidneys were taken during autopsy and stained following a standard haematoxylin and eosin protocol. The heart samples were taken from both ventricles, and the interventricular septum exhibited epicardial vessels deprived of red cells and small intramyocardial haemorrhages. The lung samples taken from each lobe contained numerous clusters of leucocytes, mainly neutrophils, and, to a lesser extent, some macrophages, predominantly in the left lower and right upper lobes. These findings are consistent with the pneumonia suspected on admission to the hospital. There were no significant findings from the other organ samples. Samples of the heart were prepared and analysed for deposition of the plasma antigen fibronectin and the terminal complement complex C5b-9 to look for early cardiac damage, especially the right ventricular ischemia. None of the samples showed reaction for C5b-9, and only small groups of cells stained for fibronectin. Based on these results, cardiac ischemia was not likely in this case.\nIn light of the different results, the cause of death was attributed to a fatal O2 embolism. This was caused by the infusion of pure O2 from the wall into the vascular system through a venous catheter connected to a nasal cannula. Although from a medico–legal point of view the circumstances of the death (accident, suicide and homicide) remained unclear, police investigations led to the suspicion that the patient had connected the nasal cannula to the venous catheter. The wound on the forehead and the bruises observed during the external examination could be explained by a fall from or next to the hospital bed, as suggested by the police. |
Our patient was a 60-year-old Hispanic female with a history of alcoholic cirrhosis and long-standing tobacco abuse who presented to our facility with complaints of a one-day history of acute onset of dyspnea and dry cough. She had never experienced these symptoms with this level of severity, and her symptoms began suddenly without any pre-existing strenuous activity. She described the pain as a sharp sensation, worse with deep inspiration, and located in the left anterior portion of her chest. She rated the pain as a 7/10 in severity, which was largely unchanged since symptom onset. She had not attempted to treat the pain with any over-the-counter medications. She denied fever, weight loss, night-sweats, lymphadenopathy, sore throat, hemoptysis, abdominal pain, rash, or joint pain.\nIn the emergency department, she was found to have an oxygen saturation of 79% on room air. The patient was placed on supplemental oxygen by nasal cannula with subsequent improvement in her oxygen saturation to greater than 90%. Physical exam was significant for decreased breath sounds and hyperresonance to percussion over the left lung fields. There was no tracheal deviation noted. Cardiac examination was unremarkable upon auscultation, and there was also no evidence of decompensated cirrhosis, such as ascites or hepatic encephalopathy. A chest x-ray revealed a large left-sided pneumothorax with nearly complete collapse of the left lung (). A chest tube was placed in the emergency department with adequate placement confirmed on chest x-ray with improvement of the pneumothorax. She was then admitted to the general medical floor. The following day, a repeat chest x-ray was performed which showed only a mild decrease in size of the pneumothorax from the prior day. A computed tomography (CT) scan of the chest without contrast revealed the presence of a left upper lobe cavitary lesion measuring 0.7 × 2.7 cm with a bronchopleural fistula (). Persistent air-leak was noted from the patient’s chest tube since admission. Cardiothoracic surgery was consulted, and video-assisted thoracic surgical bleb resection was performed due to persistent air-leak from chest tube. The surgical specimen was sent for bacterial, acid-fast bacilli, and fungal cultures. All cultures from the specimen were negative except for the fungal cultures, which were reported as growing Histoplasma capsulatum. Of note, three different sets of sputum acid-fast bacilli cultures were obtained and were negative, effectively ruling out active pulmonary tuberculosis. The infectious disease service was consulted and felt that the patient’s pneumothorax was likely due to chronic cavitary pulmonary histoplasmosis. Itraconazole was started at a dose of 100 mg by mouth daily for treatment of the infection. The patient was asked extensively about risk factors for exposure to Histoplasma capsulatum and denied recent travel, history of foreign travel, exposure to bird or bat droppings, construction sites, farmhouses, wood cutting or gathering, and cave spelunking. During admission, serial daily chest x-rays were performed to assess for re-expansion of her lung. Despite bleb resection, the lung did not fully expand, putatively because of entrapment due to an inflammatory rind. The patient then underwent a thoracotomy with decortication in hopes of improving lung expansion. During the thoracotomy, she was noted to have adhesions of both the upper and lower lobes of the left lung to the mediastinal pleura which were taken down. The patient’s pneumothorax persisted despite surgical efforts. She was discharged after a prolonged hospital stay to a long-term acute care facility after serial chest x-rays showed stable residual pneumothorax. Itraconazole was continued, with plans to continue for a total of 1 year of antibiotic therapy. Two months following discharge, a repeat chest x-ray showed resolution of her left-sided pneumothorax (). |
We present a 59-year-old woman of Belgian ancestry with negative past medical history, who was diagnosed with cutaneous malignant melanoma in the sole of the left foot. At the time of diagnosis, clinical examination and computed tomography scan of the chest, abdomen, and pelvis did not reveal findings suggestive of metastatic disease. The primary lesion was resected and histology demonstrated a nevoid melanoma of Breslow thickness 3.4 mm with ulceration. A second operation was then performed to have a wider margin width, as well as to resect a satellite lesion that had been found to be infiltrated by melanoma cells, and to examine sentinel lymph nodes, which were free of tumor. The pathology stage by AJCC (7th edition) was pT3bN2cM0 (stage IIIb).\nThe patient agreed to be enrolled in a randomized placebo-controlled phase III trial (CA209238) comparing Ipilimumab 10 mg/kg every 3 weeks for 4 doses, then every 12 weeks, to Nivolumab 3 mg/kg every 2 weeks as adjuvant treatment in high risk completely resected melanoma. Two months after enrollment, the patient developed grade 3 diarrhea due to autoimmune colitis. The treatment was stopped and she was started on oral methylprednisolone 1 mg/kg daily. Due to the severity of diarrhea, the patient was placed off protocol, and subsequent unblinding showed that she had been allocated to the Ipilimumab arm.\nNine months after discontinuing treatment the patient developed melanoma distant relapse with 2 subcutaneous lesions. Molecular tumor analysis showed a BRAF V600E mutation. Treatment with pembrolizumab 200 mg every 3 weeks was initiated. Clinical and imaging examinations on weeks 12 and 24 demonstrated partial tumor response, whereas on weeks 21 and 24 her treatment was deferred due to pancreatitis grade 2 that was well controlled with prednisolone 7.5 mg daily.\nOn week 30, the patient presented pain and edema of the right knee that occurred the day after the last pembrolizumab infusion. Clinical examination confirmed knee arthritis with swelling, increased temperature, and accumulation of intra-articular fluid. The knee movements were restricted due to pain. The patient was in good general condition with normal body temperature. Blood examinations showed grade 1 anemia (Hemoglobin [Hb] = 11.2 g/dL), a slightly elevated serum lactate dehydrogenase (247 U/L, upper normal limit 220 U/L), elevated erythrocyte sedimentation rate (100 mm/h), and serum C-reactive protein levels (63 mg/L, upper normal limit = 6 mg/L), while the white blood cell and platelet counts were within normal limits. A computed tomography of the right knee confirmed the presence of fluid in the suprapatellar bursa. A magnetic resonance scan of the right knee confirmed the presence of fluid mainly in the suprapatellar bursa and additionally showed degenerative lesions of both menisci with bucket handle tear of the medial meniscus. A synovial fluid analysis revealed an inflammatory effusion (7.200 cells/mm3). The Gram stain was negative, no crystals were detected, and the cultures for common pathogens and mycobacteria were negative. Also, the peripheral blood and urine cultures were negative. Immunological serum tests revealed a borderline titer of antinuclear antibodies (1:80), serum C3 levels were normal (161 mg/dL, normal limits 90–180 mg/dL), serum C4 levels were slightly elevated (52.5 mg/dL, normal limits 10–40 mg/dL), and rheumatoid factor (RF) and anti-cyclic citrullinated peptide (CCP) antibodies were negative. An intra-articular injection of 8 mg dexamethasone was performed and the arthritis improved. The patient was discharged with the prescription of lornoxicam 8 mg po daily.\nTwelve days after discharge the arthritis relapsed with deterioration of the pain and swelling in both knees. The clinical examination suggested the presence of intra-articular fluid in both knees. Following a rheumatological consultation, the patient received etoricoxib 90 mg daily without improvement and finally responded to methylprednisolone 16 mg po daily on week 36.\nAt that time, a restaging computed tomography scan confirmed that the tumor was still in partial remission. Because of the BRAF mutation, it was decided to proceed to a second line systemic treatment with dabrafenib and trametinib. In spite of this treatment, the tapering of the methylprednisolone dose resulted in the relapse of joint pain; therefore, leflunomide 20 mg po daily was initiated. A gradual methylprednisolone tapering was then feasible and leflunomide was replaced by hydroxychloroquine 400 mg po daily because of mild elevation of the liver enzymes. The arthritis resolved and the patient is free of complications and disease activity 20 months after the initiation of the second line systemic treatment. |
The patient is a 19-year-old man. The painless palpable mass in the right chest wall was approximately 20 mm in diameter, and he reported that the mass had persisted for the past 4 years. The patient qualified for a biopsy in a local county-level hospital. The pathologist then confirmed an adenoid cystic carcinoma. The specimen examination showed a mixed tubular and cribriform pattern. The estrogen receptor (ER) was positive at 3%. The sample was negative for progesterone receptor (PR) and c-erbB2. Then, he came to the thoracic surgery department of our hospital in February 2009. Physical examination revealed a 20 mm × 21 mm size subareolar mass which was irregularly shaped without fixation to the skin or muscle. There was no clinical evidence of regional lymphadenopathy. The patient had no past medical history or family history of cancer, and the patient did not smoke or drink alcohol.\nA breast ultrasound and mammography were performed. The ultrasonography (USG) revealed an irregular, mixed echoic, partial compressible mass(21 mm × 20 mm × 9 mm) in the subareolar region of the right breast (Figure A). Mammography (Figure B) revealed a spiculate hyperdense lesion (30 mm × 15 mm) in the right breast in accordance with the findings of the ultrasonography. Serum tumor markers and other routine blood tests were normal. The chest X-ray, bone scan, axillary lymph node, and abdominal ultrasound were normal. Following on from the diagnosis, he underwent a radical mastectomy (RM) with axillary lymph node dissection. The pathological examination of the resection specimen revealed a 30 mm × 20 mm × 15 mm tumor. The histopathological examination specimen revealed (Figure C) that surgical margins were negative; none of the 41 axillary lymph nodes excised were positive for malignancy. Microscopic examination revealed that the tumor is composed of pseudocysts, filled with basophilic mucin, surrounded by basaloid cells. Neoplastic basaloid cells were arranged in cribriform and tubular. The neoplastic cells were negative for estrogen receptor expression, progesterone receptor expression, and HER2/neu staining by immunohistochemistry. The tumor was pathologically staged as pT2N0M0. Since the estrogen receptor was 3% positive in the biopsy, it was decided for the patient to be treated with tamoxifen at a daily dose of 20 mg for 5 years. But tamoxifen was terminated after 13 months because of sexual dysfunction. Neither chemotherapy nor radiotherapy was performed in the present case. After 67 months of post-operative follow-up, our patient remains free of loco-regional recurrence or distant metastases. |
A 12-year-old boy was seen in outpatient department with complaints of severe right sided a headache, altered sensorium, and recurrent vomiting. He was having central cyanosis, clubbing since childhood. On clinical examination he was deeply cyanotic with room air saturation 74%, gross clubbing of finger and toes. Left upper and lower limb showed decreased motor function (3/5) and sensation. His hemogram revealed polycythemia with Hemoglobin of 20.2 g% and hematocrit value of 82%. Contrast enhanced computed tomography (CECT) head showed multiloculated ring enhancing lesion in the right frontotemporal region and Magnetic resonance imaging head suggestive of multiple pyogenic abscess. Diagnostic Echocardiography for heart was normal, but contrast echocardiography was highly suggestive of PAVM. X-ray chest PA view showed large lesion in the right middle and lower lobe []. CECT chest was done to confirm large centrally located PAVM in the right lower lobe [Figure and ].\nThe brain abscess was successfully removed by craniotomy and aspiration followed by systemic administration of antibiotics. The patient recovered well, but residual left hemiparesis remained. He was scheduled for elective right lower lobectomy considering the huge size of the malformation, central location and its effect of chronic hypoxia, recurrent brain infections with neurological consequences. Right posterolateral thoracotomy was done in fifth intercostal space, and unanticipated dense lung parenchymal adhesion to chest wall was found. During dissection multiple thin-walled large systemic collateral vessels were damaged with profuse bleeding and patient became hemodynamically unstable. As lung was adherent to the chest wall and bleeding, limited resection of lower lobe was not feasible. As a desperate attempt to salvage the patient, as he was bleeding profusely, right pneumonectomy was performed. His blood saturation improved to 100% once clamp was applied to pulmonary artery which was 78% earlier with Fio2 of 100.\nThe patient was resuscitated with multiple units of blood transfusion during surgery. After completion of pneumonectomy, he started oozing from all raw surfaces of chest wall. The chest cavity was packed with sponges and patient shifted to recovery. Despite multiple blood transfusions in the Intensive Care Unit (ICU) his hemoglobin level was falling and remained hemodynamically unstable with significant inotropic support. He was re-explored in the ICU and in a desperate attempt left internal mammary artery was ligated at its origin.\nHe became stable gradually but received a significant blood transfusion in the perioperative period (13 units packed red blood cell (PRBC)). On the second postoperative day, he was extubated and started orally. In the evening, he developed fever and oliguria followed by anuria. Inotropic support was also stepped up. Hemodialysis could not be instituted due to low blood pressure, so peritoneal dialysis was started. His condition further deteriorated and succumbed to acute renal failure on the fivth postoperative day. |
Our patient was 16 years old and he was a student with no particular medical history. The patient and his parents gave their informed consent to report this case. He presented a left upper limb trauma when he was 12 years old that was orthopedically treated by a cast during 3 weeks. Since then, he presented pain in his wrist, especially at sport and when he was leaning on his left hand. His pain was quoted at 7/10 (visual analog scale) and he reported a severe handicap at sport. Clinical examination showed a normal range of motion of his wrist with a normal but painful pronosupination motion. It revealed distal radioulnar instability with a protrusion of the ulnar head () without instability of the extensor carpi ulnaris tendon. The radioulnar ballottement test of the radioulnar joint, the fovea sign, and the chair test were positive. The clunk test was negative. The symptomatology was marked by a severe functional concern when leaning on his hand during gym classes or when playing basketball. The patient-rated wrist evaluation (PRWE) score was at 51/100 (pain = 31, function = 20), the QuickDASH score was at 43/100. The pain was specifically localized on the ulnar side of the wrist by the patient. No nerve compression or vascular signs were detected. X-rays of the wrist revealed a shortening of the radius with a high radioulnar index (11mm on the anteroposterior view) and an ulnocarpal impingement (). This was highlighted on the magnetic resonance imaging T1sequence of the wrist that revealed overpressure cysts of the triquetrum (). The radioulnar joint surface on the radial side was flat[]. A lengthening of the radius with an iliac crest autograft was indicated as a reconstructive surgery to recover the normal length of the radius. A bilateral wrist computed tomographyscan was performed with a 3D reconstruction to evaluate the needed lengthening according to the contralateral side. The position and the orientation of the metaphyseal osteotomy were also calculated. A lengthening of 10mm in the longitudinal axis was calculated. The final position of the plate, the length of its screws, and the dimensions of the graft were also generated (). A patient-specific guide was 3Dprinted (Allians Radius®, Newclip) to guide the osteotomy during the surgery. The 3D-printed material of the guide was done in PA2200 uspcl6 (polyamide). Under locoregional anesthesia and with a tourniquet, a volar Henry approach was performed to expose the distal radius. The distal part of the brachioradialis tendon was released at its radial insertion. The guide was applied on the metaphysis of the radius; a K-wire was positioned to evaluate the good position of the guide on the metaphysis with a fluoroscopy control. The holes of the plate were drilled previously. The osteotomy was performed with a 0.8mm saw blade and completed with an osteotome. A specific distractor with 1.8 pins was used to spread the two fragments. To avoid the buckling of the wire and the rotation of the fragment under the distraction stress, a Meary distractor was used and left in place. This allowed the stretching of the soft tissues during the iliac crest taking and it facilitated the insertion of the graft. A tricortical iliac crest bone graft of 10mm length was then taken on the homolateral side. The tricortical graft was then positioned into the osteotomy site flush to the anterior radial surface. The plate was screwed on the radius using the previously drilled holes (). The tourniquet was deflated (50 min). A cautious hemostasis was done and the approach was closed as usual. A removable sling was used to immobilize the patient during 1 month and a passive mobilization of the wrist was started the day after surgery. At 6-month post-operative(), the radius was consolidated with no more clinical deformation. The radioulnar index was improved (5mm on the anteroposterior view) with no more ulnocarpal impingement. The extension was symmetric at 65°, the flexion was at 80° versus 90° on the contralateral side, the radial and ulnar inclinations were at 5° and 40°, respectively, on both sides. The PRWE was at 2/100 (pain = 2/50, function = 0/50), the Quick DASH score was at 2/100. The patient presented no more pain on the ulnar side and was back to sport. No neurologic symptoms were found. |
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