text
stringlengths 746
31k
|
|---|
Our patient was a 58-year old female, diagnosed with CLL in 2002. Abnormal lymphocytes showed expression of CD5, CD19, CD20, CD22, CD23 and immunoglobulin kappa light chain by flow cytometry. Ubiquitous enlarged lymph nodes were detected. The patient was asymptomatic. First line treatment was required 2003 due to increasing leukocytosis and lymphocytosis accompanied by advancing anemia and thrombocytopenia. The patient was treated with chlorambucil and prednisone (Knospe protocol) according to local standards and therapeutic possibilities at that time. After achieving a partial remission persisting approximately one year, the patient was retreated with continuous chlorambucil for one month but showed no response. Four cycles of oral fludarabine were administered achieving a partial remission for four years. The following two relapses of the disease were treated again with fludarabine, of which the latter course was mainly due to patient’s preference. After documenting resistance to fludarabine the patient agreed to administration of five cycles rituximab in combination with bendamustine. A partial remission could be achieved. Rituximab and bendamustine were used for treating the following relapse 1.5 years later, achieving a partial remission for another eight months. Afterwards the patient received ibrutinib within a clinical trial, but showed progression of disease after only four months of treatment. Massive progression of lymphadenopathy was apparent at that time. Therefore, a lymph node biopsy was done showing a diffuse infiltration with small lymphocytic cells partially resembling centroblasts or immunoblasts, though transformation to an aggressive lymphoma could not be demonstrated. According to the clinical behavior of the disease, rituximab plus CHOP were administered but progression occurred after three cycles of treatment. Alemtuzumab was then administered achieving stabilization of the disease for another year. Ultimately, the patient was treated with lenalidomide but showed no significant response and died 2014 due to pneumonia. Informed consent for studies performed and for publication of the results was obtained from the patient. All methods used are described in detail in Additional file .\nPatient material was first sent to our laboratory eight years after the initial diagnosis of CLL. In the next four years, karyotyping and FISH studies were performed seven times in intervals of six to twelve months. The detailed cytogenetic findings in the seven samples of the patient, analyzed between 2010 and 2014, are summarized in Table . Consistent findings in all patient probes included the t(12;14)(q23.2;q32.3), a partial trisomy 12 due to duplication of der(12) chromosome (Fig. ) and a submicroscopic deletion of the 13q14 region. Signal splitting of the Cytocell IGH Breakapart probe confirmed the involvement of the IGH locus on chromosome 14 in the translocation (Fig. ). The duplication of der(12) indicates that the t(12;14)(q23.2;q32.3) preceded trisomy 12. Since trisomy 12 is considered to be an early driver clonal event in CLL [], we propose that the translocation occurred early in CLL evolution. Nevertheless, it is not possible to experimentally confirm that, since no sample was available at the time of diagnosis.\nSequencing of LDI-PCR-generated IGHJ bands varying from the expected germline bands revealed a productive VDJ recombination with an unmutated V1–69 gene (100% sequence homology) fused to D3–3 and J5 sequences and a D-J recombination between D2–21 and J5 on the other allele. Sequencing of the aberrant IGHS bands revealed sequences from chromosome 12 integrated into the Switch μ (Sμ) region. A second round of sequencing with a reverse primer from chromosome 14 (IGH der12 Rv) was necessary to read over the breakpoint on der(12), which was located 86.5 kbp downstream of the achaete-scute family bHLH transcription factor 1 (ASCL1) gene. Primer sequences are listed in (Additional file : Table S1). The IGHJ-Cμ enhancer was translocated in the proximity of ASCL1, while the more distal gene C12orf42 was translocated to der(14). The breakpoint on der(14) was localized within the pentameric repeat region of Sμ. There were no deletions or insertions of sequences at the breakpoints of both chromosomes (Fig. ).\nThe expression of ASCL1 in the BM of the patient bearing the translocation (90% infiltration) was compared to that in normal and CLL BM samples (mean infiltration >70%). CLL samples were subdivided in four groups according to their cytogenetic findings (Table ). ASCL1 was highly overexpressed in the sample of the patient bearing the translocation as opposed to all other groups with average fold change (FC) values greater than 5600 in all samples (ANOVA p-value = 5.12E-10) (Fig. ). Immunohistochemistry with a monoclonal anti-ASCL1 antibody on peripheral blood cytospins of the patient and two CLL control samples confirmed the ASCL1 overexpression at the protein level (Fig. and ).\nASCL1, also known as hASH1 or mASH1, is the human homolog of the Drosophila achaete-scute complex. It encodes for a basic pioneer helix-loop-helix transcription factor (TF), which is a master regulator of vertebrate neurogenesis []. In order to further explore the possible downstream effects of the ASCL1 upregulation in the aberrant B-cells of the patient, we compared the gene expression of these cells to that of sorted B-cells from seven healthy donors, using the GeneChip® PrimeView™ Human Gene Expression Array (Affymetrix, Santa Clara, CA). We found 176 significantly deregulated genes (FC ≥ 2, FDR p ≤ 0.01) (Additional file : Figure S1) and (Additional file : Table S2). Deregulation of 55 genes in our gene set was concordant with at least two CLL expression studies comparing CLL cells to peripheral CD19+ B-lymphocytes of healthy individuals (see also Additional file ) [–].\nWe then focused on the genes with the strongest deregulation in our gene set (FC ≥ 10, FDR p ≤ 0.001) (Table ). Seven of the top 18 deregulated genes (ABCA9, KCNJ11, FHDC1, KSR2, EBF1 and RGS2) were part of the above-mentioned CLL gene expression signature. The deregulation of three further genes from this list (RGS1, APP, GABRB2 and FGF2) was concordant with CLL versus normal comparisons from the Oncomine Database [–]. Among the residual eight highly deregulated genes the overexpression of ASCL1 and also PAH, localized 40 kbp proximal to the ASCL1 locus, could be explained by their proximity to the IGH enhancer due to the translocation. PAH encodes for phenylalanine hydroxylase, an enzyme involved in phenylalanine catabolism. To our knowledge, no oncogenic properties have been assigned to the PAH gene so far. Binding of ASCL1 on promoter sequences of the MRO, EDNRB and RNF150 genes has been demonstrated by ChIP in adult hippocampus-derived neural stem cells []. The overexpression of GLDN and PAX9 has not been previously described in CLL and these genes are also not listed among the direct ASCL1 targets. INSM1, the gene with the highest upregulation and the third most significant in our gene set, is a well- established direct ASCL1 transcriptional target in neural and neuroendocrine tissue as well as in SCLC [–]. |
A 74-year-old Greek man presented in June 2008 with gross hematuria. Our patient had been previously treated for non-muscle invasive pTa, G2 transitional cell carcinoma of the bladder (TCC). The first transurethral resection of bladder tumour (TURBT) had been performed ten years before, while the last was performed three years before. The last follow up cystoscopy had been performed twelve months before. Our patient was asymptomatic thereafter, until June 2008.\nThen, at cystoscopy, a large solitary polypoid, yellow-white tumor was found on the superior surface of the bladder. A computed tomography (CT) scan revealed a heterogeneous mass arising from the bladder dome with evidence of invasion of the bladder wall (figure ). There was no evidence of pelvic or abdominal lymphadenopathy. Subsequent transurethral resection was extremely difficult and incomplete because of hardness of the tissue. Pathological examination of retrieved specimen revealed a highly pleomorphic and hypercellular neoplasm composed of spindled sarcomatous cells with characteristic formation of malignant osteoid, as well as an abundant chondroid matrix.\nImmunohistochemical analysis performed on paraffin-embedded tissue sections was negative for pan-cytokeratin 7 and 20, epithelial membrane antigen (EMA), as well as a smooth muscle actin, desmin, CD34 and CD68. Vimentin and p53 were strongly expressed in more than 95% of the neoplasmatic cells. No transitional cell carcinoma or carcinosarcoma elements were evident (figures and ). These findings supported the diagnosis of primary osteogenic sarcoma of the urinary bladder. Other localization studies including a chest X-ray and a bone scan were unremarkable.\nDue to the significant comorbidity of our patient, who had a coronary artery disease and restrictive respiratory disease, he was offered partial cystectomy. However, he refused this option and underwent external beam radiation therapy (EBRT) instead. Our patient declined to receive adjunctive chemotherapy. In the following months, he had two episodes of proven urinary tract infection. Seven months after diagnosis, he developed ascites. A CT scan at that point showed extravesical tumor extension and bowel infiltration (figure ). Our patient died eight months after diagnosis, experiencing severe lower urinary tract symptoms (LUTS) and abdominal pain the last months of his life. |
A 50-year-old female patient reported to the Department of Physical Medicine and Rehabilitation of Ewha Womans University Mokdong Hospital as an outpatient with lymphedema in the right upper extremity. The patient had undergone breast-conserving surgery, axillary lymph node dissection, chemotherapy, and radiation therapy for breast cancer on the right side. After these treatments, secondary lymphedema occurred, and bandage therapy was administered as symptoms improved. However, edema from the forearm to the hand had been worsening for the previous month. The circumference of both arms was measured 5 cm above and below the elbow crease connecting the medial and lateral epicondyles. Compared to the left arm, the right arm had larger circumferences of 3.5 cm and 4.7 cm at 5 cm above and below the elbow, respectively. In addition, Stemmer sign and peau d'orange changes were observed. The range of motion of the right shoulder was limited. Flexion, abduction, and external rotation were 170°, 150°, and 80°, respectively. According to these results, the patient had stage 3 lymphedema.\nA CT scan of the upper extremities was performed using SOMATOM Sensation 16 (Siemens, Forchheim, Germany) in order to determine the location and volume of lymphedema and to establish a treatment plan. The patient underwent a CT scan with a slice thickness of 10 mm from the aortic arch to the tip of the fingers in the supine position with the shoulder at 180° flexion, elbow extended, forearm pronated, and fingers extended. According to the results of the CT scan, the right medial forearm showed the most severe honeycomb pattern. When the right and left sides were compared, it was found that the thickness of the skin and subcutaneous fat had increased on the right side (). The patient had severe skin hardening and underwent ESWT around the right forearm, and bandage therapy was continued. The ESWT was performed with Dornier AR2 (Dornier MedTech, Wessling, Germany), which is the electromagnetic defocused type. During one session of treatment, stimulation was applied 2,000 times. Stimulation was applied 1,000 times to the right medial forearm, which showed the most severe honeycomb pattern, and to the rest of the right upper limb. ESWT was performed twice a week for eight weeks, and a total of 16 sessions were administered. The intensity of treatment was 0.040-0.069 mJ/mm2 energy flux density and the frequency was 5 Hz.\nAfter 16 sessions of ESWT, a CT scan of the upper extremity was performed to determine the therapeutic effects (). The volume of the upper extremity was measured using a manual tracing technique with PiViewSTAR software (INFINITT Co. Ltd., Seoul, Korea) in order to examine the changes in volume in the areas with edema (). The cross-sectional area observed in each section of the coronal CT scan was multiplied by the slice thickness (10 mm in this study) of the CT scan to obtain the volume of each section. The volume in the ROI was measured by adding up the volume of each section in the ROI. The volume of the subcutaneous fat layers was obtained by subtracting the volume of the bones and muscles from the volume of the entire upper extremity. The volume of the entire upper extremity was measured by summing up the volumes of the regions from the distal radial and ulnar styloid process to the acromion process. The volume of the areas between 5 cm below and 5 cm above the elbow was calculated and compared before and after treatment. The volume of the entire right upper extremity and the subcutaneous fat layers was reduced from 210,878.4 mm3 to 205,970.1 mm3 and from 122,562.2 mm3 to 113,154.2 mm3, respectively. The volumes of the entire areas at 5 cm above the elbow and subcutaneous fat layers were reduced from 25,014.7 mm3 to 24,735.3 mm3 and from 14,732.6 mm3 to 13,315.2 mm3, respectively. The volumes of the entire areas at 5 cm below the elbow and subcutaneous fat layers were reduced from 28,799.8 mm3 to 28,535.9 mm3 and from 15,243.2 mm3 to 13,935.6 mm3, respectively (). In addition, when the circumferences of both arms were compared, differences of 2.8 cm and 4.3 cm at 5 cm above and below the elbow, respectively, were noted. These circumference measurements were smaller than the initial measurements. However, the presence of Stemmer sign and peau d'orange changes persisted after therapy. |
Abdominal computed tomography for regular monitoring of uterine fibrosis in a 49-year-old woman found a hypodense lesion 7 cm in diameter with a clear boundary near the gallbladder (Fig. a, b). No obvious change in size and internal density of uterine fibrosis had been observed during these 5 years (Fig. b). She had no previous history of alcohol or drug abuse. Ultrasound revealed a well-defined, non-calcified tumor between the gallbladder and liver (Fig. ). Magnetic resonance imaging demonstrated a hypointense tumor that compressed the gallbladder and liver on precontrast T1 mapping (Fig. a). T2-weighted images revealed hyperintense tumor with delayed enhancement on arterial phase and portal venous phase (Fig. b) followed by a delayed washout on the hepatocyte phase. Clinical evaluation and laboratory results were nonspecific, and serum tumor markers including carcinoembryonic antigen, cancer antigen (CA) 19-9, alpha-fetoprotein (AFP) and squamous cell carcinoma antigen were within their normal ranges. The findings were consistent with a gastrointestinal stromal tumor (GIST) or another benign tumor originating from the liver or gallbladder. Accordingly, we planned firstly laparoscopic partial hepatectomy concomitant with gallbladder resection. Lymphadenectomy among the hepatoduodenal ligament were also planned under the situations where malignant tumor was suspected intraoperatively. Under the laparoscopic view, a soft tumor had expansively progressed behind the gallbladder, with dorsal compression of the liver (Fig. ). The tumor was easily separated from Laennec’s capsule of the liver at the gallbladder neck and body without adhesion, which indicates the tumor is benign. Therefore, operative policy changed to tumor enucleation with cholecystectomy. Frozen sections including the surgical margin of the cystic duct were negative for tumor cells. As the tumor was moderately attached to the liver at the fundus of the gallbladder, the liver parenchyma was partially resected en bloc to ensure that the surgical margins were free of tumor tissue.\nThe resected specimen included soft and white tumor tissue with clear boundaries that was located between the liver and gallbladder (Fig. ). Pathological examination following Hematoxylin and Eosin and immunohistochemical staining of tumor specimens was consistent with SFT. The specimens included spindle-shaped tumor cells with elongated nuclei (Fig. a), present in both tangled and patterned arrangements in the subserosal layer of the gallbladder (Fig. b, c). The tumor tissue had a storiform pattern with alternating hypocellular and hypercellular areas with some showing myxoid degeneration. The tumor cells were positive for CD34, CD99 and B-cell lymphoma (BCL)-2 and negative for S100 and alpha smooth muscle antigen (αSMA) staining (Fig. d), and few cells were positive for the cell proliferation marker Ki-67/MIB-1. The tumor cells were negative for SMA, keratin, cytokeratin (AE1/AE3), CD117, epithelial membrane antigen, and desmin. The pathological diagnosis was SFT originating from the cystic plate.\nThe postoperative course was uneventful. She was discharged on postoperative day 9. Chemotherapy was not considered necessary, and postoperative follow up at 18 months found the patient well without any sign of recurrence. Periodical check-up for uterine fibrosis by gynecologist is also continued. |
A 22-year-old woman, right dominant hand, visited our Department complaining of the presence of several small swellings localized under the skin of the pulp of the index finger (Figs –). She reported an increase in the number of swellings in a period of about 2 years. She noted the first one 3 years before. She never felt pain. An X-ray of the index finger showed multiple not well-defined round-like calcified lesions, without involvement of the bone or of the distal interphalangeal joint. There were no other such lesions in the body. The patient was apyrexial and in good general health. There were no signs of tenosynovitis, palmar space or flexor sheath infection. She had no lymphangitis or regional lymphadenopathy.\nESR, cell blood count, electrolytes, uric acid, cholesterol and triglyceride levels were all normal.\nThe results obtained from a DXA examination reported a value of −1.5 at lumbar spine and a value of −1.8 at the femoral neck. There was no evidence of any underlying disease.\nFor this clinical presentation we decided to treat this young patient with a surgical excision of the calcific deposits. A wide excision of the volar surface of the distal phalanx including the calcified nodules was performed. Skin affected by tumoral calcinosis was also excised preserving the neurovascular bundles and FDP insertion. The masses appeared yellowish and chalky, with a maximum size of 0.5 × 0.5 × 0.3 cm (one of them).\nThe soft tissue defect was reconstructed by harvesting a reverse homodigital artery flap from the ulnar side of the index and transposing the flap into the defect. The flap was based on ulnar digital artery reverse flow. A full thickness skin graft was also harvested from the forearm to cover the flap donor area.\nPostoperative recovery was uneventful with no loss of flap or skin graft. The patient was discharged 4 days post-surgery and resumed normal activity by 6 weeks.\nThe specimen was sent for histological examination. Histology confirmed the presence of granules and deposits of calcium in the soft tissue. |
In 2017, a 52-year-old male presented at the hospital with complaints of progressive chest pain. Chest imaging revealed an anterior mediastinum mass with enlarged lymph nodes and a left pleura metastasis. A small needle biopsy of the anterior mediastinal mass revealed poorly differentiated squamous cell carcinoma of the thymus (). Immunohistochemistry showed that 70% of the cells were positive for PD-L1. The patient was administered first-line treatment with paclitaxel liposomes plus carboplatin; however, the disease continued to progress. Next, immunotherapy was initiated with pembrolizumab as second-line treatment, and the patient showed progressive disease after 2 cycles of immunotherapy. Subsequently, the treatment was modified to include combination therapy with albumin paclitaxel as a third-line treatment, which led to significant disease remission after 2 cycles. Upon examination, the patient showed only slight progress after the 3rd cycle (), and was switched to treatment with pembrolizumab plus anlotinib, which subsequently resulted in progressive disease. This was followed by the introduction of palliative radiotherapy, and concurrent administration of targeted therapy with anlotinib. Combination therapy with anlotinib plus pembrolizumab was continued for 16 cycles as a part of a sequential regimen, resulting in the maintenance of stable disease until December 2019 (), when an enlarged pleural tubercle and new bone metastases were observed (). As a consequence of these findings, the third-line chemotherapy was judged to be ineffective, and the patient was switched to one cycle of combination therapy with pembrolizumab plus gemcitabine. To our surprise, the patient exhibited a partial response (). Furthermore, during the entire course of pembrolizumab immunotherapy and immunocombination therapy with albumin paclitaxel/anlotinib, the patient experienced only grade 1 nausea without any additional immunotherapy-related side effects. However, the patient did experience a grade 3 skin reaction that manifested as rashes on the trunk and limbs, accompanied by itching during treatment with pembrolizumab plus gemcitabine combination therapy. Later, a marked improvement in the skin reaction was observed after initiation of hormone therapy. In summary, the duration of anti-PD-1 single therapy and immunotherapy in combination with chemotherapy/antiangiogenic therapy lasted for a total of 23 months. |
Ms. N is a 23-year-old nulliparous woman with morbid obesity (body mass index (BMI) 40.3 kg/m2) who presented to the emergency room at our local community hospital. Her concerns were progressive exertional shortness of breath for two weeks and tachycardia for three days. She had access to a home pulse oximeter and had noted heart rates in the 120s with normal oxygen levels. Ms. N did admit to some mild shortness of breath at baseline but stated that the recent progression was much worse. The patient noted recent past medical history significant for COVID-19 infection that occurred six weeks prior to presentation, diagnosed by nasal swab. Of note, she was an employee at a local nursing home and believes she contracted the virus at the facility. Ms. N stated that the acute illness was treated at home with symptomatic treatment and that her COVID-19 symptoms of cough and fever had completely resolved following the acute illness. Her past medical history was otherwise significant for asthma, depression, and migraines. Her only regular medication was an oral contraceptive. She did relate a family history of heart disease but was not able to specify further.\nAt the time of the emergency room presentation, Ms. N was noted to have sinus tachycardia and tachypnea. Her brain natriuretic peptide (BNP) was elevated at 1082 pg/mL while serum myoglobin and troponin-I were normal. The patient’s COVID-19 immunoglobulin G (IgG) was positive. Pertinent presenting labs are presented in Table . Computed tomography (CT) of her chest revealed the absence of pulmonary embolism but the presence of multifocal lymphadenopathy throughout the chest. A representative image is shown in Figure .\nEKGs revealed a normal sinus rhythm and were unchanged during her hospital stay. She was admitted to the hospital with a provisional diagnosis of acute decompensated systolic congestive heart failure, and the cardiology service was consulted. Presumptive heart failure treatment was initiated with valsartan, metoprolol succinate, and furosemide. Echocardiography revealed severely decreased systolic function with an ejection fraction of 10%-15%, grade I diastolic dysfunction, and severe global hypokinesis. Cardiac catheterization demonstrated normal coronary arteries and elevated left ventricular end-diastolic pressure of 38 mmHg. The consultant cardiologist believed Ms. N’s cardiomyopathy to have been due to her recent COVID-19 diagnosis.\nMs. N was managed as an inpatient with diuresis with an improvement in her presenting symptoms. She was discharged to home with prescriptions for furosemide, metoprolol succinate, and valsartan. The interval echocardiogram at one month redemonstrated an ejection fraction of 10-15%, and the patient was referred to cardiopulmonary rehab. Ms. N was also referred to a specialized heart failure clinic in our healthcare system; the cardiologist there believed that she had a pre-existent cardiac remodeling process that was made worse by her COVID-19 infection. The heart failure clinic ordered a repeat CT of the patient’s chest, which redemonstrated the bilateral hilar and mediastinal lymphadenopathy. Cardiac MRI performed two months after hospital discharge demonstrated a dilated left ventricle without ventricular wall hypertrophy; this was interpreted by the consultant radiologist and cardiologist as consistent with nonischemic cardiomyopathy. |
Our patient was a 27-year-old Japanese woman, gravida 1 para 0 (G1P0010). Her obstetric history was not significant except for a spontaneous abortion requiring curettage 10 years previously. There was no history of any other pelvic or abdominal surgical intervention.\nShe was admitted to our hospital with premature rupture of the membranes and had undergone induction of labor at 39 gestational weeks. Two days later, she had an uncomplicated vaginal delivery of a 3244-g male infant with a good Apgar score. However, her placenta was not delivered. We tried manual removal of the placenta, but felt resistance during traction of the umbilical cord. Subsequent Doppler ultrasonography (US) examinations showed a progressive vascular flow to the placental mass in her uterus. We suspected placenta accreta, so we left the whole placenta in her uterus. Fortunately, postpartum bleeding was not severe and the total amount of bleeding was 850mL, even though the umbilical cord was torn during delivery.\nWe cared for the bleeding and possible infections. At 7 days post-partum, her serum human chorionic gonadotropin (hCG) level was 12,656IU/L. Gadolinium-enhanced MRI also indicated deep myometrial invasion by placental tissues, and the whole placenta was strongly enhanced (Fig. ). A general physical examination revealed no abnormalities. Her medical and gynecological histories were unremarkable.\nOur patient hoped to preserve her uterus for a future pregnancy so we decided to administer 50mg MTX by intravenous injection after obtaining informed consent relevant to surgical and other treatment strategies. Hematological, hepatic, and renal functions were evaluated and found to be normal before the MTX injections. Our patient experienced no untoward effects following MTX administration, and her vaginal bleeding diminished gradually over four weeks. To avoid intrauterine manipulation with surgical equipment and subsequent infection (that is, diagnostic hysteroscopy), follow-up MRI using an identical technique was performed every four weeks to assess the treatment response. MTX therapy (50mg/week) was administered 13 times. During the course of this therapy, our patient had an intrauterine infection and took three separate course of antibiotics. Complete resolution of the uterine lesion was confirmed by MRI scans (Fig. ). Her serum hCG level was undetectable (<5IU/L) 97 days after the first MTX injection (Fig. ). At 117 days post-partum, she had a labor-like pain every three minutes and delivered the remnant of the placenta with a little bleeding. The vestigial mass weighed 80g (10×6×4cm) and histopathology indicated placental tissue with necrosis. Her menses resumed 181 days after the first MTX injection, and a follow-up hysteroscopy showed no abnormal findings (Fig. ).\nTwo years later, our patient (G2P1011) became pregnant spontaneously. We did not detect any placental lacunae or clear spaces on transabdominal US, nor did we see any depletion of decidua in her uterus on MRI. She underwent an uncomplicated vaginal delivery of a 3304-g female infant with a good Apgar score. Postpartum bleeding was not active and the total bleeding was 910mL. We did not find any thinning of the posterior uterine muscular layer on transabdominal US, and there was no bleeding with the dislodging of the placenta. The placenta had histopathological features of marginal infarction and chorioamnionitis stage 2 by the Blanc category. Two independent pathologists did not note any deficiency in the decidua. |
A 59-year-old plastic surgeon was diagnosed with a lung tumor on 31 March 2009, when he was examined for persistent upper dorsal spinal pain, refractory to TENS and physiotherapy. He had smoked 20 cigarettes a day for the last 40 years. He felt healthy except for a lack of energy in the last two weeks and he had a good performance status. His tumor was classified as a Stage IV right lung, poorly differentiated, large cell adenocarcinoma. It was a T2 (1,6 cm nodule in the right parahilar region), N2 (4,3x3, 1x5,3 cm bulky conglomerate of subcarinal nodes) and M1 (left suprarenal metastasis of 2,7 cm diameter). The carcinoembryonic antigen (CEA) had a high value of 203,4 ng/ml. He was scheduled for four cycles of platinum doublet based chemotherapy with cysplatinum and premetrexed at every 21 days, to start on May 11. This patient requested treatment with intravenous PRF. This was done on April 11, 2009, 3 weeks before the start of chemotherapy. He reported a subjective feeling of wellbeing starting during treatment and continuing thereafter. Over the 3 weeks following treatment, his dorsal pain with an NRS score of 8 gradually disappeared completely. Also, on a second PET-CT on May 8 2009, before the start of chemotherapy, there was no tumor progression of the parahilar nodule. He was then treated with chemotherapy and later with chemoradiotherapy, and the adrenal metastasis was removed endoscopically. After the chemoradiotherapy, ending on October 9, there was no more active treatment. He kept doing well and a PET-CT made on February 3, 2010 failed to show any evidence of disease. The last checkup was done in May 2012. He was again tumor free and the CEA was 7 ng/ml.\nA spontaneous complete remission of an NSC lung carcinoma is extremely rare (). We have been able to find only one single case of adenocarcinoma of the lung stage I, with a complete remission (). A complete and durable result of chemotherapy and chemoradiotherapy is equally rare (). In this case, the complete remission of pain and the stable image on the scan in the period before chemotherapy was started suggest that possibly PRF treatment may have played an additional role in the therapeutic process.\nA 50-year-old construction engineer had to stop working at the age of 31 because of increasing dyspnea and coughing. A diagnosis of α 1 anti-trypsin deficiency was made. He was not dyspneic in rest, but he had very little tolerance for exercise. He could walk up a flight of stairs but he had to do it slowly.What bothered him most was the occurrence of intercurrent infections. Especially in the winter season this happened frequently. There was then increased and productive coughing, moderate fever and a feeling of sickness. It often took him 2 to 4 weeks to recover and the next infection might occur very soon afterwards. He requested treatment with intravenous PRF. He was treated on March 13, 2011. He has had no intercurrent infections since, and he was very pleased with the result. To put it in his own words: “I know that I have the disease, but I’m not sick anymore”. On his urgent request the treatment was repeated as a precautionary measure on October 17, 2011 and on March 11, 2012. His pulmonologist saw him last on July 5, 2011 for a checkup. He was very pleased with the physical findings, on auscultation the lungs were dry and the lung function showed no deterioration compared to a test in 2001. He encouraged him to continue the PRF treatment.\nA 42 years old female nurse had a history of major depressive disorder since the age of 19 years. Since then she had been under continuous psychiatric treatment, involving continuous anti-depressive medication. Lately she was medicated with clozazolan 2 mg and venlafaxine 75 mg daily. Mid December 2011 she developed an acute depressive episode. That was not a new phenomenon; acute crises had been recurrent, occurring without any warning, provocation or specific time schedule. They had sometimes required the need for hospitalization, but they had been responsive to medication in the past. This time the dose of clozazolan and venlafaxine were doubled and trazodone 150 mg was additionally prescribed. The medication was not efficacious and she also became too drowsy. After consultation with her psychiatrist she was treated with intravenous PRF on 11 April 2011. The dispersive electrode was placed over the contralateral carotid artery. Before the procedure hs-CRP was 9,4 mg/l. On the day after treatment she reported that she felt more alert but that her mood was unchanged. After the first week she started improving progressively. After 2 weeks she felt no longer depressed and she started reducing the medication to venlafaxine 75mg. At 4 weeks the hs-CRP was again determined, it had dropped to 3,8 mg/l. In June, two months after treatment, she stopped taking all medication – for the first time in 23 years - and she started working again. She has been doing very well for 9 months. Then suddenly on 11/01/2012 she had a recurrent acute episode. She reinitiated the medication and she requested a new intravenous PRF treatment. This was done on 25/01/2012. She is now doing well again without medication.\nA 46 year old, healthy female secretary, a relative of one of the authors (AT), had a sudden episode of apraxia and right hemiplegia on 30/10/2009, while she was resting at home. She was immediately transferred to the hospital where she arrived unconscious. A diagnosis of ischemic stroke in the left middle cerebral artery encompassing the insula, frontal and temporal lobes was established and appropriate treatment was implemented. No cardiac or carotid vessel disease was identified and the etiology was undetermined. She survived the acute episode with a right hemiparesis, aphasia and apraxia. A rehabilitation program was initiated with physiotherapy and speech therapy on the fifth day post stroke. She was discharged on 18/11/2009, having by then fully recovered from the hemiplegia and partially from the apraxia. She continued the rehabilitation program as an outpatient. Over the first month there was some progress in recovery from the apraxia and the aphasia, but then she stopped improving further and she abandoned the program. At the time her aphasia was classified as a mild to moderate Broca’s aphasia. In January 2010 she requested intravenous PRF treatment. At that time her situation was as follows. Verbal expression was limited to a small repertoire vocabulary and short sentences. Auditory comprehension was well preserved but only if her interlocutor talked at a slow rate. She could gesticulate and write. Her reading capacity was also limited to words and sentences; she was unable to read paragraphs. She was alert and cooperative but anxious and mildly depressed due to her clinical condition. In making the decision we considered that spontaneous improvement had come to an end. We expected that it might be possible to improve her condition by reducing the perilesional inflammatory component with a positive effect on the neuroplasticity and that possibly her mood would improve. She was treated on January 1, 2010. A high sensitivity CRP was taken immediately before the procedure and repeated 1week after. It had dropped from 1,05 mg/l to 0,33 mg/l. The immediate outcome was a subjective feeling of well-being. Thereafter the symptomatology progressively improved, starting in the first week and continuing for 8 months. By the end of April 2010 she restarts cooking and driving her car. By then she was capable of reading magazines. Her speech repertoire had also improved and she could maintain a conversation with several interlocutors if they talked at a slow pace. She and her husband referred that she improved the most from July to September. After that period there was stabilization and she asked for a repeat treatment. This was done on 29/12/2010 and the result was a further improvement of the aphasia. She and her husband commented that she improved more in the 4 months after the second treatment than in all the previous year. Her sexual life, which had come to a stop, returned to normal in this period. She resumed her job with some limitations in June 2011. Actually she lives an almost normal life now with a mild expressive aphasia. |
A 44-year-old white man experienced painless bilateral blurring of vision during and after an ultramarathon event in Park City, Utah, in April 2011. He noticed the visual impairment primarily in the right eye during the last few hours of the ultramarathon and presented to our clinic 5 h after completion of the event. This was his first ultramarathon and he had not had any similar episodes of visual impairment in previous athletic activities. Past medical history included hyperlipidemia, controlled by atorvastatin (Lipitor). Family, social, surgical and ophthalmologic histories were non-contributory. He presented to the clinic the evening after the event. The patient involved provided written informed consent to having his data used for research purposes, in accordance with the tenets of the Declaration of Helsinki.\nOn eye examination, the patient had visual acuity of 20/200 OD and 20/30 OS. Intraocular pressure was 17 mmHg OD and 18 mmHg OS. Extraocular muscle movements were intact. The orbits and eyelids revealed no evidence of trauma. On slit lamp examination, the right eye had diffuse 3+ corneal edema with a few Descemet’s folds (Figs. and ) while the left eye had trace to 1+ corneal edema located on the nasal periphery of the cornea and dissipated at the temporal side. The conjunctivae had no injection and there was no corneal epithelial abrasion in either eye. The anterior chamber, iris and lens were normal in both eyes. There was no evidence of pigment dispersion. A dilated pupil fundus exam revealed a normal optic nerve, vasculature and macula on the left side, and only red reflex was appreciated in the right eye due to corneal edema.\nThe patient was prescribed prednisolone acetate 1% ophthalmic suspension QID for both eyes. His vision subjectively recovered to his baseline 12 h later, and an eye exam performed 48 h after the episode showed complete recovery of both corneas without sequelae. Endothelial microscopy/counting was not performed. |
A 35-year-old woman, gravid 5, para 3, abort 2, was referred to our clinic because of continuous vaginal bleeding from 2 months ago after uncomplicated curettage with missed abortion at the first trimester (GA: 12 w + 4 d). Her obstetric history was notable for two previous low-transverse cesarean section deliveries and two curettages were without any complication. The pathology report after curettage was confirmed to be decidual tissue.\nPhysical examination was normal. The gynecologic examination of the vulva and vagina was normal, the cervix was closed, the uterus was larger than normal at about 10-12 weeks of gestation, retrovert axis and she had mild bleeding. In the clinical test, her Hb was 12.4, U/A was NL (without hematuria), BHCG was 112 mUI/mL and other coagulation tests, hepatic and renal tests and electrolytes were normal. Transvaginal sonography was performed as requested: UT > NI, retrovert uterus, endometrial thickness = 2.6 mm, hypoechotic mass about 8 cm in the lower uterine segment with necrotic change and increased vascularity with low resistance was seen around it []. Differential diagnosis included invasive mole, chorio carcinoma and uterine tumors, including degenerating myoma. Total abdominal sonography and chest X ray were normal. Surgical intervention was suggested and arranged to confirm the diagnosis and to remove the lower uterine segment mass. Surgery was performed with the patient under general anesthesia and in the 15-degree Trendelenburg position. A Foley catheter was inserted preoperatively to empty the bladder. She received suction and curettage. After removing the debris and much necrotic tissue, vaginal hemorrhage could not be controlled during operation. The decision fell on laparatomy. The lower segment uterine mass appeared as a large, friable, highly vascularized mass that occupied the whole thickness of the uterine. Hysterectomy was performed immediately for treatment of hemorrhage-induced hypotension, fluid replacement and packed blood cells and blood products were administered []. For more than care, she was transferred to the Intensive Care Unit. On the day after surgery, the BHCG level decreased to about 13 mUI/mL. The patient was discharged on the third postoperative day after an uneventful recovery. Definitive pathology of the surgical specimen led to a diagnosis of necrotic and hemorrhagic placental tissue, consistent with placenta previa and placenta increta []. |
A 78-year-old male patient with TA was referred to our hospital for the treatment of a BCA aneurysm. He had been treated for TA with 10 mg/day oral administration of prednisolone for 24 years. He was also treated for hypertension at a local hospital and had no history of cardiovascular diseases. He had moderate pulmonary emphysema due to a long history of smoking. Furthermore, though there were no obvious sequelae, he had a history of brain infarction. Computed tomography showed a BCA aneurysm measuring 32 mm in diameter with a thickened aortic wall ranging from the orifice of the BCA to the bifurcation of the right common carotid and right subclavian artery (Fig. a). Computed tomography also showed severely atherosclerotic changes in the arch vessels (Fig. b). C-reactive protein level was 0.2 mg/dl, and erythrocyte sedimentation rate was 6.0 mm/hour. Taking into consideration of the patient’s comorbidities as mentioned above, partial arch replacement with ICP was scheduled. Following median sternotomy, cardiopulmonary bypass was established with femoral artery cannulation (Fig. a) and bicaval drainage with a low flow (0.5 L/min). When the cardiopulmonary bypass got stabilized, direct cannulation of bilateral carotid arteries was added with a proximal clamp not to flush debris to the distal site (Fig. a). Total cardiopulmonary bypass was gained by additional cannulation through the ascending aorta and an 8-mm graft anastomosed to the right subclavian artery (Fig. a). The chronic inflammation changes compatible with TA, such as wall thickening and increased hardness, were observed in the ascending aorta (Fig. b). When the body temperature reached to 25 °C, lower body circulatory arrest was introduced. The orifice of the right subclavian artery was closed with a continuous 4–0 polypropylene suture through inside of the aneurysm. The orifice of arch vessels presented with severe atherosclerotic changes. Partial arch replacement with 28 mm quadrifurcated graft (Triplex®, Terumo Corporation, Tokyo, Japan) was performed with an open distal anastomosis technique. After distal anastomosis, the neck vessels including right subclavian artery, right carotid artery, and left carotid artery, were reconstructed with each branch of the artificial graft individually (Fig. c). An artificial graft anastomosed to the right subclavian artery was used for branch reconstruction (Fig. c, d). Both distal and proximal anastomosis were reinforced with Teflon felt strips at inner and outer side of the aortic wall. Weaning from cardiopulmonary bypass was uneventful, and the patient recovered without any neurological deficits. The time of operation, cardiopulmonary bypass, aortic cross-clamp, cerebral perfusion, and circulatory arrest were 442, 229, 128, 133, and 60 min, respectively. There were no significant changes in the continuous bilateral cerebral regional oxygen saturation values between the right and left side during the surgery as monitored using INVOS™ Cerebral/Somatic Oximetry Adult Sensors. Postoperative brain imaging detected no significant cerebral infarctions. Postoperative computed tomography demonstrated no anastomotic aneurysms and no abnormal flow in the aneurysm (Fig. d). Pathological results reported that the intima of the aorta had focal and raised plaques. During the perioperative period, management with additional prednisolone infusion was done, and the patient has never experienced episodes of exacerbation of inflammation. The patient remained free from any major adverse aortic and cerebrovascular events 1 year after surgery. |
A 68 year old caucasian female with a pancreatic cystic neoplasm diagnosed on ultrasonography (US) presented at our Institute in December 2013. Her past medical history was silent. No abuse of ethanol or smoking was reported. The patient reported upper abdominal discomfort for 2 months; nausea, vomiting or weight loss were not referred. The physical examination revealed a palpable mass in the epigastrium; scleral icterus was absent. No other clinical abnormalities were detected.\nThe US showed a 5 × 6 cm pancreatic macrocystic lesion localized in the body of the gland. Laboratory tests showed that blood cell counts and C-reactive protein were normal. Levels of serum glucose, creatinine, albumin, aminotransferase, gamma-glutamyl transferase, alkaline phosphatase and lipase were all within normal limits. The serum levels of the following tumor markers were elevated: carcino-embryonic antigen (CEA) was 196 ng/ml (normal range <5 ng/ml) and carbohydrate antigen 19-9 (CA 19-9) was 66 U/ml (normal range <33 U/ml).\nThe patient underwent a triphasic multidetector computed tomography (CT) scan and a T1, T2-weighted and DWI contrast enhanced magnetic resonance imaging (MRI) with intravenous Gadolinium administration.\nThe CT scan showed a large complex cystic and solid mass measuring 5 × 3 × 6 cm involving the neck and the body of the pancreas (Fig. ). The tumor had a predominant central component characterized by multiple large cystic spaces (up to 3 cm in diameter) separated by thick septa. A 2 cm area of low attenuation solid tissue was located between cyst walls in the caudal portion of the lesion. The main pancreatic duct was enlarged in its entirity (diameter 12 mm) with a more marked dilatation in the body and tail region. Irregular calcifications were evident in the peripheral portion of the tumor. There was compression of superior mesenteric vein by the mass with a minimal infiltration of the splenic vein. The CT scan of the chest and abdomen did not show any lymphadenopathy, hepatic and pulmonary metastasis.\nMRI confirmed the complex pancreatic lesion featured by a large cystic portion with inner irregular septa and a peripheral solid area measuring 2 cm in the lower portion of the tumor (Fig. ). Cholangiopancreatographic images did not demonstrate a communication between the enlarged main pancreatic duct and the cystic mass; ectatic branch ducts were present in the body and tail region.\nAt laparotomy a large cystic mass involving the body and the neck of pancreas was confirmed. Further exploration showed an infiltration of the head of the gland but ruled out neoplastic spreading to adjacent organs and lymph nodes; the splenic vein was infiltrated by the mass at superior mesenteric veinous junction. Patient underwent total pancreatectomy with splenectomy and lymph node dissection. A US performed on 7th post-operative day ruled out any intra-abdominal fluid collection. Patient developed post-pancreaectomy diabetes which was difficult to control by insulin therapy. The patient was discharged 14 days after the operation.\nMacroscopically the pancreas was involved by a 6 cm well-circumscribed round mass localized in the neck and the body with infiltration of the head; stomach, duodenum, common bile duct and spleen were not infiltrated. Externally the tumor presented a smooth reddish surface and the pancreatic parenchyma not involved by the lesion appeared fibrotic. On cut-section the lesion showed multilocular macrocystic spaces separated by fibrous septa and filled by a clear viscous mucoid fluid. The main pancreatic duct was ectatic but it was not possible to recognize a communication with the cystic lesion. A 2 cm single yellow solid area was located in the caudal portion of the mass.\nMicroscopically the tumor was principally composed of a cystic mucinous part characterized by a columnar mucinous epithelium with atypical nuclei, copious mitoses and stromal invasion. It was not possible to find an ovarian-like stroma (Fig. ). In the caudal part of the neoplasm there was a solid area containing mononuclear spindle cells, associated with pleomorphic giant cells (PGCs) and scattered multinucleated osteoclast-like giant cells (OGCs) (Fig. ).\nThe surgical margins were negative for neoplastic infiltration. No lymph node metastases were shown. There was no evidence of perineural or vascular infiltration.\nThe mononuclear spindle cells and pleomorphic giant cells were immunoreactive for epithelial markers including CK8/18 and CK19, and for vimentin and expressed a weak immunoreactivity for CD68 and a focal immunoreactivity for actin and CD10. The proliferative index (Ki-67) of the mononuclear spindle cells and pleomorphic giant cells was high (approximately 30 %) (Fig. ). Multinucleated osteoclast-like giant cells intensely expressed histiocytic marker (CD68) and vimentin but they were negative for epithelial markers (CK8/18 and CK19) and actin. In these cells Ki-67 immunoreaction was negative (Fig. ).\nThe histopathological findings supported the diagnosis of indeterminate mucin-producing cystic neoplasm with a component of osteoclast-like giant cell carcinoma.\nTwo months after surgery serum CEA and CA 19-9 decreased to 83 ng/ml and 31 U/ml, respectively. Nevertheless after 4 months serum CEA increased to 144 ng/ml and hepatic metastases were founded at follow-up CT scan. Gemcitabine single agent chemotherapy was started (1,00 mg/m2 weekly × 7, then weekly × 3 every 4 weeks). CT scan performed 8 months after surgery showed the progression of liver metastases and appearance of lung metastases. The patient expired 10 months after surgery. |
A previously healthy 6-year-old boy presented to a pediatric hospital with a 3-week history of torticollis. He had symptoms of an upper respiratory tract infection four weeks prior and had 2 days of documented fever at home during that time. He had been treated with a 7-day course of amoxicillin by the primary care physician for suspected streptococcal pharyngitis. Four days into the course of antibiotics, he woke up from sleep with pain on the left side of his neck. Despite taking ibuprofen and acetaminophen, he presented to the Emergency Department 3 weeks later due to persisting torticollis. Pain was worse with movement. There was no history of head/neck trauma. At the time of presentation, the infectious symptoms had resolved. Some fatigue was noted but he remained generally active, continuing to play hockey. There was no history of rash, peripheral joint pain, or weight loss. Past medical history and family history were unremarkable.\nOn examination, the patient was afebrile with normal blood pressure for age and a maximum heart rate of 110 beats per minute. The patient's head was tilted to the right with chin rotation to the left. No lymphadenopathy or masses were noted on palpation of the neck. There was no tenderness to palpation of bilateral sternocleidomastoid muscles. There was a limited range of motion in all planes of rotation of the neck secondary to pain, particularly in lateral flexion. Bilateral injected conjunctivas were present. The oropharynx was normal with no erythema or mucus membrane changes. Cardiovascular exam revealed normal peripheral pulses, a quiet precordium with normal heart sounds, and no murmur. Respiratory exam was normal. The abdomen was soft with no distension, tenderness, or hepatosplenomegaly. There were no bruits heard on auscultation of major vessel regions. There were no rashes or desquamation of the skin. Neurological exam was normal.\nAt the time of presentation, laboratory investigations revealed an elevated white blood cell count of 17.4 × 109/L with a neutrophil count of 14.1 × 109/L. Hemoglobin was normal for age at 110 g/L. Inflammatory markers were elevated including platelet count of 860 × 109/L and CRP of 38.5 mg/L. Renal function (BUN and creatinine) and liver function (ALP and ALT) were normal for age. Because of the unexplained elevated white blood cell count and evidence of inflammation, a chest X-ray was performed which revealed normal lung fields but an enlarged cardiac silhouette. X-ray of the cervical spine was normal with no atlantoaxial rotary subluxation demonstrated. Ultrasound of the neck revealed mild thickening of the left sternocleidomastoid muscle and no lymphadenopathy. Abdominal ultrasound with Doppler was normal.\nAdditional investigations included a normal throat swab for group A streptococci and a negative anti-streptolysin O antibody titer. High-sensitivity troponin was elevated to 176 ng/L. Creatinine kinase was normal. ANCA was normal. Electrocardiogram showed normal sinus rhythms without evidence of chamber hypertrophy. The patient underwent an echocardiogram to further characterize the enlarged cardiac silhouette identified on the chest X-ray. This revealed massive ectasia and aneurysmal dilatation of the right coronary artery, left main artery, left anterior descending artery, and circumflex arteries, as seen in . Left ventricular function was normal. The aortic arch was normal as were the proximal neck vessels.\nBecause of the dilated coronary aneurysms, the patient was diagnosed with KD. Despite lack of fever, given the evidence of ongoing inflammation and initial presence of bilateral nonsuppurative conjunctivitis, in addition to the coronary artery changes, the patient was treated with high-dose IVIG (2 g/kg) and started on daily low-dose aspirin. Low-molecular-weight heparin was started as antithrombotic therapy and once stabilized, daily atenolol was initiated. Activity was restricted as much as possible.\nInflammatory markers were followed. Platelets revealed a peak of 952 × 109/L and CRP a peak of 54.6 mg/L. After treatment, both platelet and CRP levels normalized.\nThe patient's neck pain and the limited range of movement resolved immediately after treatment, as did the bilateral conjunctivitis. The patient was stable and appeared well at time of discharge. His aspirin, low-molecular-weight heparin, and atenolol were continued. The CT angiogram performed after discharge revealed massively dilated and aneurysmal coronary arteries, as shown in .\nIn follow-up cardiology and rheumatology clinics, he has been doing well with no further neck pain or stiffness. He did not develop desquamation during follow-up, and the repeat echocardiogram one month after discharge was unchanged. He will continue long-term anticoagulation therapy with low-dose heparin with a target level greater than 0.5 IU/ml. He will also continue low dose aspirin and atenolol. His family was advised to have the annual influenza vaccine. |
A 70-year-old woman from Sale, with no history of previous hydatid disease, was admitted to our cardiology department for dyspnea NYHA class IV evolving in a febrile context for 2 days. Its hemodynamic profile showed a tachycardia at 120 bpm with a systemic blood pressure at the lower limit 95/55 mmHg. The clinical examination found the signs of right heart failure. Echocardiography revealed a pericardial effusion of great abundance in pre-tamponade with an intramural honeycomb pattern in the right ventricular wall.\nThe honeycomb pattern as a specific image usually found in hydatid disease prompted the investigation of hydatid serology which was explored by the ELISA technique with the ELISA DRG Diagnostic kit. The test came back positive up to 31.90 OD.\nThe diagnosis of isolated cardiac hydatidosis involvement was established, and CT scan assessment showed a massive pericardial effusion with hydatid cyst compressing the right ventricle and mild bilateral pleural effusion (Fig. ) with no other organ involvement.\nSurgery as indicated was realized through a median sternotomy with off-pump (Fig. ). First, we proceeded to the aspiration of a pericardial liquid with whitish rock water appearance and to pericystectomy to remove the false membrane and cystic formations.\nWe proceeded after that to an off-pump treatment of the right ventricle cysts: we first used aspiration with a 10-G lumbar puncture needle connected directly to the aspirator, and then we removed the cystic membranes, the residual cavity was cleaned with gauze saturated with hypertonic sodium chloride and povidone-iodine solution, and was left open.\nPart of the piece was sent to the parasitology laboratory. Examination under an optical microscope (× 400) had revealed rare hooks of Echinococcus granulosus (Fig. ). The whitish rock water appearance of the pericardial liquid confirms the theory of intrapericardial cyst rupture. The patient was put on medical treatment with albendazole 10 mg/kg/day for 3 months with a favorable clinical course. |
A 32-year old man with no significant past medical history presented with a several month history of a non-healing ulcerative lesion under his right axilla. On examination, multiple palpable subcutaneous nodules were present in the axilla, groin, face, neck and chest wall. Excisional biopsy of the axillary lesion showed a poorly differentiated HA (). The diagnosis of carcinoma was supported by positive immunohistochemical staining for keratins (AE1/AE3 and CK7). In addition, the tumor was strongly positive for estrogen receptor (ER), but negative for Her/2neu overexpression. Staging with positron emission tomographic computed tomography (PET CT, ) scan showed multiple enlarged hyper-metabolic nodes in the right axilla and bilateral supraclavicular lymph nodes. A lytic lesion in the posterior right 5th rib was also seen. The patient was not thought to be a surgical candidate secondary to metastatic disease. A single case report in the literature found prolonged response to paclitaxel and carboplatin, and therefore, we offered our patient palliative cytotoxic chemotherapy with this regimen. The patient completed three cycles of chemotherapy and restaging scans revealed progressive disease in the bilateral axilla. Given the progressive disease, chemotherapy was stopped and the patient received palliative radiation to his bilateral axilla, to which he had a significant response. Upon completion of radiation patient began tamoxifen, an estrogen receptor antagonist based on the ER positivity of his tumor and some success with tamoxifen in other ER positive hidradenocarcinomas. At 13 months of starting tamoxifen, the patient remains without clinical progression.\nIn parallel to initiating treatment, an axillary lymph node was biopsied, the presence of metastatic tumor was confirmed, and the tumor tissue was sent for genomic profiling using Foundation One testing (Foundation Medicine, Cambridge MA, USA) as well as Baylor’s whole exome sequencing (Baylor College of Medicine, Houston, TX, USA). The Foundation Medicine panel identified four genetic alterations including FGFR1 amplification, CDH1 splice mutation, MYST3 amplification and ZNF703 amplification (). The Baylor whole exome analysis identified no germline mutations, two cancer related and actionable genes, seven tumor associated genes, and approximately 180 variants in non-cancer associated genes (). The actionable cancer related genes identified in the Baylor panel included PTCH1 and TCF7L1, and the mutations in cancer genes without identifiable targets included ARID1A, CDH1, FBXO11, FNBP1, IL6ST, MYC, and WHSCIL1. |
The patient was a three-month-old female, the second child of healthy parents with consanguineous marriage. She was referred to the pediatric neurology department with a history of weak cry, poor feeding, and progressive peripheral hypotonia. She was born full-term by normal spontaneous vaginal delivery and had no history of neonatal intensive care unit admission. Growth parameters at birth were within the normal range. Prenatal history revealed decreased fetal activity during the third trimester but was negative for polyhydramnios and urinary tract infection. She had a history of two previous admissions secondary to chest infections but did not require mechanical ventilation. She had one healthy brother and negative family history of similar diseases.\nThe patient was evaluated for the first time at the age of two months. She was awake, alert, with stable vital signs, without dysmorphic features or neurocutaneous stigmata. Growth parameters showed normal weight and length, but large head circumference, as follows: weight, 4 kg (above 15th percentile); length, 52 cm (at 50th percentile); and head circumference, 46 cm (above 99th percentile). Signs of active hydrocephalus were observed, including a bulging anterior fontanelle, sunset eyes, and dilated scalp veins. Occasional tongue fasciculation was seen. The patient was hypotonic, with flickery movements of the upper limbs and no movements in the lower limbs. Deep tendon reflexes were absent. Apart from paradoxical breathing, the systemic examination was unremarkable with no organomegaly noted.\nType I SMA was suspected and molecular genetic testing was requested. Regarding macrocephaly, computer tomography (CT) was done and it showed picture of active communicating hydrocephalus (Figure ). Tumors, infection, and bleeding were ruled out as possible causes of the hydrocephalus and the patient was referred for urgent surgery. Later on, the molecular genetic study confirmed type I SMA, revealing a homozygous pathogenic deletion encompassing exons 7 and 8 of the SMN1 and extending to the neuronal apoptosis inhibitory protein (NAIP) gene, with two copies of the SMN2 gene.\nA ventriculoperitoneal shunt (VPS) was surgically implanted. Nusinersen treatment was planned and requested.\nThe patient was followed up by a multidisciplinary team comprising neurology, neurosurgery, genetic, pulmonary, and physiotherapy specialists. However, she died at the age of five months secondary to aspiration pneumonia, prior to receiving the first dose of nusinersen. |
A 61-year-old man presented with a low-density, round-shaped mass with well-defined margins measuring 5.5 cm on his preoperative chest radiograph (Fig. A). A computed tomography (CT) scan of the chest revealed a mass measuring 6.0 × 5.0 cm in the lower lobe of the left lung and a linear band of 2.0 cm behind the LA (Fig. B). Transthoracic echocardiography (TTE) showed a thin, linear hypoechoic band of 0.9 cm in the left inferior pulmonary vein ostium and also revealed a highly mobile embolus extension 8 to 10 cm in length protruding into the LA (Fig. C). Since the extension prolapsed into the left ventricle toward the mitral valve in diastole and into the LA with the retrograde motion of systole, a small amount of blood flowed backward past mitral orifice due to the embolus-leading mitral regurgitation. There was no evidence of enlargement or injury of the LA. This study was approved by the Institutional Review Board of PLA Navy General Hospital. The patient has signed a written informed consent form.\nA left anterolateral thoracotomy was carried out under general anesthesia. After the lung mass was isolated from the lower lobe of the left lung, the veins, arteries, and bronchial tube to the area of the mass were cut and the mass was removed from the left lower lobe (Fig. A). After which, the blood vessels and bronchial tube was sutured and the patient was checked for any bleeding in and around the lungs. Following lobectomy, embolectomy was executed under cardiopulmonary bypass. A free-floating embolus of 16 cm in length was removed from the LA together with the excision of the embolus extending up to the left inferior pulmonary vein around its base. A grape-like cluster of soft tissue was observed in the extension (Fig. B). Pathological examination of the lung mass showed spindle-shaped fascicular proliferation with nuclear palisading (Fig. C) indicating moderately differentiated squamous cell carcinoma (SCC). Immunohistochemistry was used to confirm SCC. The cells from the lung tumor and the embolus extension showed cytokeratin (CK) AE1/AE3 (+++) and p63 (+++). The patient recovered uneventfully and was discharged on postoperative day 15. |
A 56-year-old male with a past medical history of end-stage renal disease on hemodialysis, congestive heart failure, groove pancreatitis, chronic atrial fibrillation, valvular heart disease, and obesity, who was status post four gland parathyroidectomy for hyperparathyroidism seven years prior presented to the hospital for chronic bilateral lower extremity nonhealing wounds which had been worsening. He was found to have a parathyroid hormone level of over 3,200 pg/mL (normal range 10-65 pg/mL) as well as a soft right forearm mass located at the site of previous autotransplantation. The mass was further characterized with a CT scan of his right forearm under the indication “distal forearm tumor”. The CT scan showed a fat containing mass at the lateral aspect of his forearm measuring 4.6 x .9 x 5 cm with soft tissue nodularity suspicious for an atypical lipomatous tumor or liposarcoma (Figures , ).\nFollow-up imaging with a nuclear medicine Technetium-99m Sestamibi scan showed retained activity posterior and inferior to the left lobe of the thyroid gland and focal increased activity at the right forearm on a two-hour delayed image (Figure ). A 4D-CT scan of the parathyroid showed a 1.5 x 2 x 1.5 cm nodule posterior to the lower pole of the left lobe of the thyroid gland, suggestive of a parathyroid adenoma or thyroid nodule (Figure ).\nBecause of his markedly elevated parathyroid hormone (PTH) level and concern that the mass on his right forearm may be a lipomatous tumor due to the CT scan interpretation, the decision was made to resect the mass. Upon resection, the operative report stated that the mass did have the appearance of possible lipomatous features given that it had a different characteristic than the surrounding subcutaneous fat. The surgical pathology report came back as nodules of hyperplastic parathyroid tissue involving fibroadipose tissue and skeletal muscle (Figures , ).\nA repeat Technetium-99m Sestamibi study performed three days later showed no uptake at 20- and 60-minute delayed imaging to suggest residual parathyroid tissue (Figure ). Intraoperatively his PTH level was still markedly elevated and he eventually underwent additional parathyroid tissue removal with a left thyroid lobectomy several months later. |
Case 1 is a 63-year-old morbidly obese man weighing 280 kg who presented with septic shock secondary to worsening bilateral lower limb cellulitis. His medical history included obstructive sleep apnoea and chronic recurrent lower limb cellulitis. His clinical course in the intensive care unit (ICU) was further complicated by development of a sacral pressure ulcer and atrial fibrillation. After 9 days in intensive care, he was transferred to the medical ward, where his renal function normalized and he did not require further dialysis.\nCase 1 is a retired truck driver who lived with his wife in a single-storey house with 4 access steps. Prior to hospital admission, he ambulated with a walking stick indoors and outdoors and was independent with all activities of daily living (ADL).\nAfter 41 days in the acute ward, the IR team was consulted. At the time of initial review, the patient was severely deconditioned and required 8 staff for bed mobility, 4 staff for hoist transfers, 4 staff for assistance with sit-to-stand, and full assistance with personal care.\nThe initial goals of IR were to reduce the care burden to 3 staff for transfers and to begin sit-to-stand training with a forearm support frame (FASF), motivate the patient to lose weight, and coordinate sacral pressure care management. The medium-term plan was to enable smooth transition to a subacute rehabilitation facility once feasible.\nA strict schedule for physical therapy sessions was enforced. Case 1 was encouraged to comply with dietitian recommendations, including meals with no added salt, low calorie intake, and high-protein diet. The dietary goal was to facilitate wound healing and promote weight loss.\nAfter the second week of IR on the acute ward, the patient’s functional status had improved to requiring 3 staff to assist with bed mobility and transfers (no longer hoist dependent), one staff to assist with sit-to-stand, and he was able to mobilize 20 m with 5 seated rests using a FASF. However, he still required full assistance with ADLs.\nBy the end of 4 weeks, he required 2 staff to assist with bed mobility, 1 staff for sit-to-stand with a 2-wheel frame, and walked 80 m with 4 seated rests with 2 staff members. The patient was then transferred to a subacute inpatient rehabilitation unit for ongoing therapy. At the time of discharge from hospital, his body weight was 236 kg, a loss of 44 kg from admission. There was no FIM gain at the time of transfer to subacute rehabilitation facility despite demonstrated functional improvement. |
A 63-year-old male, weighing 82 kg (BMI 28), was scheduled for abdominoperineal resection (APR) and right partial hepatectomy for metastatic colon cancer. His past medical history was significant for hypertension, recently diagnosed non-insulin dependent diabetes, a cerebral vascular accident 9 years prior to surgery, an 80-pack year smoking history (quit 20 years prior to surgery), and an episode of acute congestive heart failure with preserved ejection fraction approximately one year before surgery.\nAt preoperative evaluation, the patient described his functional capacity as excellent (able to climb 2 flights of stairs multiple times a day with no symptoms; worked more than 60 hrs. a week as a mechanic) and denied any symptoms of HF. The physical examination did not reveal any signs of congestive heart failure. Transthoracic echocardiogram (TTE) showed normal left ventricular (LV) size and function (EF of 55%) and no significant valvular pathology. Since the patient was scheduled for an intermediate risk surgery, had no active cardiac conditions, and had good functional capacity, a decision was made to proceed with surgery without further work-up based on guidelines established by the American College of Cardiology and the American Heart association (ACC/AHA).\nGeneral anesthesia was induced with propofol and maintained with isoflurane plus an air-oxygen mixture of equal parts. Standard ASA monitors were applied along with invasive hemodynamic monitoring using a right radial arterial line. Patient had a preexisting tunneled central venous catheter that was accessed and 2 large bore IVs were inserted for intravenous access. He received muscle relaxation with intermittent doses of intravenous (i.v.) rocuronium titrated to keep the train of four to less than 2 twitches. He received a total of 2 mg i.v. hydromorphone in divided doses and 1000 mg of i.v. acetaminophen for analgesia.\nThe surgery lasted approximately eight hours. His estimated blood loss was 400 ml and urine output was 300 ml. He received 7 liters of crystalloid and 1 liter of albumin. His intraoperative fluid management was guided by measurement of stroke volume variation (SVV) via Vigileo™, serum lactate levels, and urine output. He received fluid boluses to keep the SVV less than 13, lactate levels less than 2 mg dl−1, and urine output greater than 0.5 ml kg−1 per hour. He remained hemodynamically stable throughout the surgery. He did not have any significant electrolyte abnormalities as determined by hourly arterial blood gases (ABGs) during the surgery. The patient's trachea was extubated at the end of the procedure after ensuring adequate reversal of neuromuscular blockade and responsiveness to commands.\nImmediately after extubation, he developed hypertension and tachycardia with a high blood pressure of 273/100 mm Hg and heart rate ranging between 120 and 130 beats/minute. His oxygen saturation declined to a low of 89% despite supplemental oxygen and airway recruitment maneuvers. He progressively became agitated and less responsive. Pink frothy sputum was noted upon suctioning of his oropharynx. The patient's trachea was promptly reintubated, and 20 mg of i.v. furosemide was given. He was then transferred to the surgical intensive care unit for further management.\nThe immediate postoperative chest X-ray revealed bilateral vascular congestion. The electrocardiograph (ECG) showed nonspecific inferior lead changes without ST elevations. The serial troponin I levels were obtained and peaked at 0.5 ng ml−1 (normal < 0.12). He received an additional dose of i.v. furosemide and over the next 12 hours his respiratory status improved. After meeting all the necessary criteria, his trachea was extubated. His home doses of aspirin, atorvastatin, and metoprolol were restarted. A repeat TTE done on postoperative day 1 (POD 1) showed an ejection fraction of 60% without any wall motion abnormalities. Despite this, he had another episode of acute pulmonary edema on POD 1 followed by atrial fibrillation (AF) with rapid ventricular response (RVR). He was successfully treated with noninvasive continuous positive airway pressure (CPAP) of 5 cm H2O and a dose i.v. furosemide for acute pulmonary edema. For his AF with RVR, he received intermittent doses of i.v. metoprolol to achieve rate control with a target heart rate of <100 beats/minute. His dose of oral metoprolol was also increased from 25 mg every 12 hours to 50 mg every 12 hours.\nOn POD 4, he developed a third episode of respiratory distress with minimal response to diuretics and antihypertensives. His ECG now showed ST elevations in anterior and inferior leads and his troponin I level was 0.74. He underwent emergent cardiac catheterization with placement of two bare metal stents in the mid-left anterior descending and left circumflex vessels for 80% and 85% occlusion, respectively. The remainder of his hospital course was uneventful and he was discharged home on POD 12. |
A 28-year-old Asian woman (G3P1) who had undergone emergency cesarean delivery owing to a compound presentation at full term was referred to our institution with a suspicion of abnormally located gestational sac. She had undergone laparoscopic cholecystectomy and open appendectomy previously. She did not have any medical, family, or psychosocial history. She had missed her menstrual period without any other symptom and visited a private obstetrical clinic to confirm the pregnancy. However, she was diagnosed as having an abnormal pregnancy such as cervical or CSP by USG.\nAt our institution, she reported that her last menstrual period was just 5 to 6 weeks prior. However, USG revealed a gestational sac in the anterior lower uterine segment with a fetus measuring 4.83 cm crown-rump length (CRL) with positive cardiac activity, corresponding to 11 weeks and 6 days of gestation. Color/power Doppler images depicted a hyperechoic rim of a choriodecidual reaction with excessive vascularity (Fig. ). Although we could observe a definitive abnormally located gestational sac, our patient did not have any pain during the physical examination. She admitted that her last menstrual period was different from her usual menstrual periods. Because CSP or cervical pregnancy was suspected, we performed computed tomography (CT) for a definitive diagnosis. The CT scan showed an intrauterine gestational sac in the lower uterine segment bulging through the anterior uterine wall at the site of the cesarean scar. No invasion of the urinary bladder was observed (Fig. ). On presentation, her β-human chorionic gonadotropin (β-hCG) level was 66,536.8 IU/L (Day 1). Initially, we injected 50 mg of methotrexate (MTX) mixed with 9 mL of normal saline in the amniotic sac through a 22-G needle transabdominally under USG guidance. Simultaneously, 2 ml of amniotic fluid was aspirated for termination of the pregnancy. However, fetal cardiac activity was still observed 2 days later (Day 3), without significant changes in the serum β-hCG levels (65,342.5 IU/L). We decided on laparotomy instead of laparoscopy because of the large CRL (Day 4). The intraoperative finding showed bloody amniotic fluid, blood clot, placenta, and a fetus at the lower segment of the uterus. A transverse uterine incision was made at the lower segment of the uterus (Fig. ). The gestational sac was removed, as well as most of the trophoblastic tissues that were adherent and invading the wall of the lower uterine segment. The fetus and placenta showed no definitive abnormalities (Fig. ). The estimated blood loss was 1.2 L at intra-operation, without immediate complication. The uterine defect was repaired into two layers by using 2–0 Vicryl sutures. Our patient received 3 units of packed red blood cells (PRBC) at the ward postoperatively. The serial β-hCG level was 1958 IU/L at 4 days after the surgery (Day 8). She was discharged in good condition 5 days after the operation (Day 9). After 1 month (Day 39), her β-hCG levels returned to normal (2.8 IU/L). She was very satisfied with the fact that she had recovered well without the need for intensive care or further treatment without the need for hysterectomy. |
A 60-year-old man with a past surgical history of C5–C7 anterior arthrodesis for cervical fracture 17 years ago was found on the floor after an unwitnessed ground-level fall, resulting in a head strike. He was taken to the local hospital in an Aspen collar with a GCS of 15 and no neurologic deficits. Routine ECG and laboratory evaluation was unremarkable except for elevated serum alcohol level. Full cervical spine computed tomography (CT) scan was performed which showed an Anderson and Montesano classification (20) type III left occipital condyle fracture (), a Levine and Edwards classification (21) type III (Jefferson) fracture with mild lateral subluxation of bilateral C1 masses (), and an asymmetric widening and slight anterior subluxation of the right AOJ (). A head CT was performed which showed no acute intracranial abnormalities.\nThe patient was immediately transferred to a level I trauma center for further management. In the emergency department, he reported severe midline neck pain, worse with movement, and unchanged paresthesia to bilateral upper extremities which he states is his baseline. Physical exam was unremarkable except for a right forehead hematoma with overlying abrasions and tenderness to palpation over the midline posterior neck. He exhibited full strength and unchanged baseline sensation to all extremities. He denied bowel or bladder incontinence and had strong rectal tone and intact perianal sensation. Through his clinical course, he became increasingly altered and agitated, refractory to medication. He was unable to maintain spinal precautions due to his agitation, and in an attempt to protect his cervical spine, he underwent endotracheal intubation.\nMRI of the cervical spine was performed and showed ligamentous injury at the craniocervical junction (CCJ) (Figures and ), asymmetric left odontoid-lateral mass widening, widening and subluxation of the right AOJ (), and edema of the paravertebral soft tissues around the fracture. CT and MRI established the diagnosis of OCD in a neurologically intact patient. Due to the unstable nature of the fracture, the patient was taken to the operating room for emergent occiput-C2 posterior instrumentation and fusion ().\nAn Aspen collar was in place at all times until surgical stabilization. Immediately, after removing the collar, Mayfield tongs were applied. Prior to patient positioning, baseline somatosensory evoked potentials (SSEPs) were obtained. Using a Jackson table turning frame, the patient was rotated into prone position. Fluoroscopic imaging was used during manual manipulation of the Mayfield apparatus to ensure no further displacement of the fracture.\nOcciput to C2 posterior instrumented arthrodesis was performed. Neuromonitoring remained stable during the procedure. The patient was discharged to home five days after presentation. |
A 56-year-old male presented to the emergency department (ED) with significant substernal chest pain starting 30 minutes prior to arrival. The patient was immediately brought back to an exam room after an electrocardiogram (ECG) was performed and was seen by a provider within 10 minutes of registration (Figure ).\nThe initial ECG revealed atrial fibrillation with a rapid ventricular response, rate of 147, with minimal ST depression within the lateral leads but was without apparent ST-segment elevation. On initial assessment, the patient had point-of-care labs immediately drawn (a basic metabolic panel and troponin), and a chest X-ray performed to evaluate for a possible aortic dissection which revealed no gross abnormalities (Figure ).\nWith a non-diagnostic chest X-ray alternative diagnoses were pursued. The ultrasound fellow in the department was consulted for an immediate cardiac ultrasound for evaluation of right heart strain secondary to pulmonary embolism. During the bedside cardiac ultrasound, the patient experienced ventricular fibrillation (Video ), and CPR was immediately started.\nThe ACLS algorithm was followed for pulseless ventricular fibrillation, and the patient received multiple rounds of epinephrine, 450 mg of amiodarone (300 mg and then 150 mg), and three conventional defibrillations with increasing joules at 150 J, 200 J, and 200 J (the departmental defibrillators are biphasic and have a maximum output of 200 J). The patient continued with ventricular fibrillation throughout the ACLS algorithm, and the decision was made to attempt DSD. The patient had a second set of pads applied in the anterior-posterior orientation in addition to the conventional right upper chest and left lateral chest with successful conversion of the ventricular fibrillation. The patient was additionally given Lidocaine, 100 mg, due to a wide-complex tachycardia and apparent non-responsiveness to the previously given amiodarone (Figure ). An improvement was noted after the use of Lidocaine, and a Lidocaine drip was started.\nOnce the patient was stabilized, he was taken for computed tomography (CT) imaging to further evaluate for the possibility of a pulmonary embolism. In the CT room, he developed bradycardia and subsequently lost his pulse. CPR was again started, the patient was given atropine, and return of spontaneous circulation (ROSC) was achieved shortly after. The CT scan did not reveal any evidence of aortic dissection or pulmonary embolism and a repeat ECG was performed which showed a persistent wide complex tachycardia with no obvious ST-segment changes. Due to the morphology of the QRS complexes and length of resuscitation time from initial arrest (nearing 90 minutes), tissue plasminogen activator (tPA) was used as a thrombolytic for what was presumed to be a large vessel occlusion myocardial infarction. Hundred milligram of tPA was administered (50 mg as a bolus and 50 mg as a drip given over 60 minutes) with an apparent reperfusion rhythm followed by a "normal" appearing sinus tachycardia. Another ECG was repeated which revealed sinus tachycardia, at a rate of 114, now with ST-segment elevations present in aVR, V1, V2, V3, and V4 with depressions in leads II, III, and aVF (Figure ).\nGiven the patient's persistent elevations despite thrombolytic therapy, interventional cardiology was consulted, and the patient was transferred to a tertiary care facility for cardiac catheterization revealing a thrombotic occlusion in the proximal left anterior descending coronary artery.\nAfter transfer to the tertiary care facility for cardiac catheterization, the patient developed cardiogenic shock. The patient was started on ionotropic medications with no improvement, and he was placed on venous-arterial extracorporeal membrane oxygenation (ECMO) therapy (~20 hours after his cardiac arrest). Before initiation of ECMO, the patient was awake, alert, and following simple commands (although still intubated). Unfortunately, despite ECMO support, his cardiac function did not improve, and the patient was not a candidate for cardiac transplantation. Seventeen days after the patient's initial presentation to the ED, the family decided to withdraw care. The patient was extubated, had ECMO discontinued, and time of death was documented shortly after. |
A 25-year old woman with no pertinent past medical history presented intoxicated after a mechanical fall down a flight of 15 stairs, and was found to have fractures of the left temporo-parietal bone, with extension into the sinuses and acute epidural hematoma with midline shift. She emergently underwent left decompressive hemicraniectomy with duroplasty. Her initial post-surgical course was uncomplicated and she was discharged home after 5 days.\nShe returned 1 month later with acute onset expressive aphasia. At that time, she denied any fevers, chills, or night sweats, however she reported clear fluid drainage from her operative site. MRI with and without contrast of the brain revealed a collection concerning for subdural empyema at the site of the recent craniectomy. She underwent a cranial wound washout and purulence was noted in the subdural plane. Intraoperative specimens were negative on Gram stain and grew low quantities (single or few colonies) of normal skin flora including Staphylococcus capitis, Cutibacterium acnes (formerly Propionibacterium acnes) and Staphylococcus epidermidis. Initially she was treated empirically with IV vancomycin, however she was transitioned to daptomycin after failure to achieve therapeutic vancomycin troughs. Her aphasia resolved, and following her clinical improvement she was discharged on post-operative day 4 with a plan to complete a 6-week course of daptomycin.\nTwo days after her discharge, she was readmitted with fever, weakness, recurrent aphasia, and edema at her recent surgical site. MRI brain showed focal dehiscence along the temporal bone, leptomeningeal enhancement along the left cerebral hemisphere and a complex extra-axial fluid collection along the left cerebral convexity. There was no clinical or radiographic concern for osteomyelitis in several CT and MRI images enhanced with gadolinium. She was taken to the operating room urgently, and a large amount of purulent drainage was noted in the epidural, subdural, and subarachnoid spaces, without any adjacent bony involvement. These findings were consistent with a complex recurrent empyema and associated cerebritis. Daptomycin, cefepime and metronidazole were started empirically, however she failed to improve and developed worsening aphasia and right-hand weakness. Repeat MRI brain on post-operative day 4 demonstrated extensive leptomeningeal enhancement in the left cerebral hemisphere consistent with meningitis as well as swelling in the left frontoparietal region favoring cerebritis {}. Continuous EEG monitoring was consistent with subclinical seizures. Therefore, the patient was initiated on a short steroid taper and anticonvulsant medications.\nGram stains from the intra operative specimens on this readmission revealed white blood cells (WBC) but no organisms and cultures showed no growth for the first 3 days. On day 4, possible pinpoint colonies were noted on anaerobic cultures (CDC anaerobic agar), however Gram staining of the possible colonies was negative. Colonies on CDC anaerobic agar from approximately day 6 of culture are shown in . Upon further review of cultures from the previous admission (collected 1 week prior), these pinpoint colonies were also identified. These findings of fastidious growth and inability to stain organisms with Gram stain raised suspicions for Mycoplasma or Ureaplasma infection and she was started on levofloxacin and doxycycline. Culture growth morphologically consistent with Mycoplasma species was recovered from all 9 intraoperative specimens (brain tissue, scalp, fluid aspiration) submitted for culture on readmission, and Mycoplasma hominis was eventually identified using 16 s ribosomal DNA sequencing. Susceptibility testing was requested, but unable to be obtained.\nHer aphasia improved significantly 2 days after starting appropriate therapy. She was ultimately sent home to complete an 8-week course of levofloxacin 750 mg daily and doxycycline 100 mg every 12 h, and a 6 week course of daptomycin 10 mg/kg IV via PICC line. A follow-up MRI brain 1 month after completion of antibiotics revealed improvement in left cerebral edema and leptomeningeal enhancement. Her symptoms completely resolved and she completed her antibiotic regimen without complication. She did well, and 11 months later (delay due to COVID-19 pandemic) she underwent left autologous cranioplasty. She was seen 6 weeks post-operatively, and was doing well. |
MDMR, a 15-year-old male patient was presented to urology outpatient clinic about 2 weeks after alleged fall. The patient claimed that he has fallen due to a slippery floor at his school. Unfortunately, his penis hit the edges of staircase nearby. Following that, he sustained bruises at the base of the penis which slowly increased in size and associated with pain. After day 4 of the fall, he noticed he had no morning erection, apart from that, he could pass urine as usual with no history of passing out hematuria and no dysuria.\nDuring initial examination, circumferential hematoma at the base of penis, nontender, and no obvious defect along the penile shaft was noted. Three weeks post-trauma, he was again followed up in the outpatient clinic and at that time the swelling remained the same. The patient reported that he had morning erection, but only proximal 1/3 was hardened and distal 2/3 of the penis was flaccid. Penile examination then showed small firm-to-hard swelling at the base of the penis, size about 1 cm × 1 cm, and there was no scrotal swelling. Following examination, the patient was subjected to Doppler ultrasonography (USG) of penis followed by an urgent MRI of the penis after that in view of late presentation and clinical examination findings.\nHis blood investigations which were taken earlier were in normal range. His USG penile results revealed that focal discontinuity of proximal tunica albuginea suggestive of a penile fracture. The discontinuation was measure 0.9 cm in length; there was heterogeneous lesion suggestive hematoma of the left corpus cavernosum of penis adjacent to penile fracture measures 0.5 cm × 1.1 cm. As shown in the [Figures -].\nHowever, his penile MRI result showed no spongiosum tear; however, there was a small left corpus cavernosum discontinuity with small hematoma and with final MRI diagnosis was penile hematoma in view of intact circumferential enhancement of tunica albuginea. Illustrated in [Figures -].\nHe was following up again in urology clinic after 6 weeks posttrauma, the swelling already resolved and was able to have a full erection. Clinical examination showed no obvious abnormality along the penis. |
Patient is an otherwise healthy 50-year-old female adult, without any past medical history. She presented to the emergency room with fever and epigastric pain, having experienced 3 months of an intermittent pain in the right upper quadrant that was related to fatty food intake. Abdominal examination showed upper abdominal tenderness and a positive Murphy sign.\nLaboratory data found elevated white blood cell count and a positive C-reactive protein. Bilirubin, liver function tests and amylase were normal. Due to pain, an abdominal ultrasound was requested that revealed two gallbladders. Two piriform images, with anechoic content, were detected. The left one had gallstones and right one had biliary sludge, also the right gallbladder had a thickened echogenic 4.5 mm wall (Fig. A). A magnetic resonance cholangiopancreatography confirmed this diagnosis. It revealed a normal liver and a gallbladder duplication with inflammation signs in one of them (Fig. B), they both had independent cysts ducts that joined in a common cystic duct before entering a normal diameter common bile duct (Fig. C).\nGallbladder duplication with cholecystitis in one of them was suspected. Due to clinical, laboratory and images findings, surgery was decided.\nAt laparoscopy, multiple adhesions from omentum to the gallbladder fossa were seen and released, revealing the Calot’s triangle structures. After achieving critical view of safety, a gallbladder duplication was identified, the right gallbladder had purulent bile with thickening of its walls, the second gallbladder appeared normal. Both of them had independent cystic ducts, but only one cystic artery was detected (Fig. A and ). From there surgery was straightforward, clips were applied to the common cystic duct and cystic artery respectively and they were divided between the clips without any complications. After that, the gallbladders were dissected off the gallbladder fossa using electrocautery and retrieved in a latex bag via the umbilical port.\nPathology reported two permeable cystic ducts, and two separated gallbladders divided by thick inflammatory tissue. Multiple 0.5 cm gallstones were discovered in the left gallbladder and biliary sludge in the right one (Fig. B). Microscopically, chronic exacerbated cholecystitis was detected in the right gallbladder (Fig. C). A Y-shaped gallbladder duplication with cholecystitis due to cholelithiasis in one of them was the final diagnosis\nThe postoperative course of the patient was uneventful and she was discharged after full diet was tolerated.\nPatient underwent full recovery, on follow-up controls patient is doing well. |
A 25-year-old woman presented to the acute surgical team with a 24-hour history of sudden onset generalized abdominal pain, worsening in severity. She had an associated fever, malaise and anorexia. She did not complain of any urinary symptoms, dyspareunia or vaginal discharge. At the time of presentation, she was approximately midway through a regular menstrual cycle. She was otherwise fit and well with no previous medical history and on no regular medications. She did admit to a diarrheal illness one month previously during which an out-patient stool culture had grown Campylobacter jejuni.\nOn examination, she was noted to be tachycardic with a heart rate of 110 beats per minute and fever at 38.0°C. She was tender throughout the lower abdomen, mostly in the suprapubic area with guarding. Urinalysis was negative for blood, nitrites and leukocytes and a urinary pregnancy test was negative. Her blood investigations revealed a raised white cell count of 19.1 (normal range 4 – 11 109/L) and a C-reactive protein of 3 (normal range 0 – 10 mg/L). An ultrasound examination was not performed. She was discussed with the gynecology team who agreed with our management plan to perform a diagnostic laparoscopy and was available to attend theater if required.\nDiagnostic laparoscopy was performed via a 10 mm infraumbilical camera port and a 5 mm left iliac fossa instrument port. A small collection of pus was noted in the pelvis although the uterus, ovaries and appendix were macroscopically normal. The pus subsequently showed pus cells on microscopy but no organisms were cultured. On inspection of the small bowel, primarily in search of a Meckel’s diverticulum, a 160 mm length of cystic and nodular change involving the mid-ileum and neighboring mesentery was found. The rest of the small bowel was normal. One cystic lesion on the posterior aspect of the mesentery had ruptured with evidence of surrounding induration []. The left iliac fossa stab incision was lengthened to 40mm, creating a mini-laparotomy incision to enable resection of the effected length of small bowel and a hand-sewn end-to-end bowel anastomosis was performed. The resected bowel was opened along its antimesenteric border and cystic disease affecting both the mesentery and bowel wall was confirmed []. Her post-operative course was uneventful and she was discharged home 5 days after surgery.\nHistology identified these changes as localized intestinal and mesenteric lymphatic cyst disease. Dilated lymphatic channels were noted to extend throughout the full thickness of bowel wall and into the mesentery []. |
A 69-year-old man was admitted with history of multiple episodes of syncope. He was diabetic, hypertensive and known case of chronic kidney disease. He was receiving ramipril 10 mg daily as anti-hypertensive. His ECG revealed sinus arrest, tall peaked T wave with heart rate of 16 bpm (). On admission, his blood sugar was normal. Complete hemogram was normal except for mild anemia and hypocalcemia. However, he was in metabolic acidosis with pH 7.3. Blood urea and creatinine were 112 mg% and 4.9 mg% respectively. His potassium level was 7.5 mEq/L. After proper consent, a 7 Fr transvenous temporary pacing wire was inserted into right ventricle (RV) via the left internal jugular vein under aseptic condition (Pacel Biplar Pacing catheter, St Jude Med, NM, USA). ECG showed pacemaker spike with regular capture and pacing with left bundle branch block (LBBB) pattern. On next day, his ECG showed pacemaker spike with regular capture and pacing but with right bundle branch block (RBBB) pattern (). Chest X-ray in antero-posterior view showed the tip of the pacing lead into left ventricle (LV) (). Fluoroscopy showed the tip of the pacing lead was posteriorly directed facing toward LV on lateral film (). Echocardiography revealed normal LV systolic function and pacing lead coursing from RV through the interventricular septum into LV (). Therefore, septal perforation with LV pacing was diagnosed by ECG, anatomical position appearing in chest X-ray, fluoroscopy and confirmed by echocardiography. Patient was hemodynamically stable. The patient was then taken for re-implantation which was successfully done with another temporary lead which was inserted under fluoroscopy through right femoral vein and placed into RV apex and the migrated lead was removed without need for surgical exploration. Patient underwent hemodialysis for few days. His potassium came down to 3.4 mEq/L. Blood pH got normalized. Blood urea and creatinine level were 42 mg% and 2.4 mg% respectively predischarge. After few days, he attained normal sinus rhythm (). Temporary pacing wire was removed and was discharged in stable condition and is under regular follow-up since then. |
A 49-year-old Korean male patient presented with a palpable nodule of the right cheek (). The nodule was a firm, nontender, subcutaneous mass. His medical history was significant for a motorcycle collision 13 years ago, from which he suffered a right-sided facial injury and loss of vision in the right eye. More recent than this, the patient suffered a bicycle collision 2 years prior to presentation and underwent open reduction of a right zygomaticomaxillary fracture. One year after the bicycle accident, the patient began to notice a small nodule in the traumatized area and slowly grew.\nInitially, we believed the lesion to be an epidermal cyst or another mass of benign origin. The mass was excised under local anesthesia without much consideration to tumor margins. Intra-operatively, the tumor was situated just underneath the dermis with capsulation. The mass was dissected from the skin, and no skin excision was necessary to isolate and resect the mass. Grossly, the 6×5×4 mm tumor was yellow-brown in color with a smooth encapsulating surface (). Histopathologic examination revealed the tissue to contain mucinous lakes separated by fibrous septa (). Neoplastic cells were round to cuboidal with abundant cytoplasm and minimal atypia. Cribriforming and small glandular structures were also noted. On immunohistochemistry, the neoplastic cells were strongly positive for cytokerain 7 (CK-7), gross cystic disease fluid protein 15, and mucin 1 protein. The cells were negative for cytokeratin 20 (CK-20) and mucin 5AC. These morphologic and immunohistochemical features were consistent with mucinous adenocarcinoma.\nBecause of the unexpected finding of mucinous carcinoma, an extensive oncologic evaluation was performed to evaluate for any extracutaneous metastatic primary (i.e., breast or gastrointestinal tract). The evaluation included computed tomography of the head and neck, a positron emission tomographic scan, and an upper and lower gastrointestinal tract endoscopy. No extracutaneous source was found, and the diagnosis of a PCMC was confirmed.\nAt 3 weeks after the first operation, the patient underwent a wide local excision with a safety margin of 5 mm, and was found to have clear surgical margin on frozen section. The resulting 1×1.5 cm defect was covered with a croissant -shaped modified V-Y advancement flap (). Considering the lack of any evidence-based data regarding the treatment and follow-up of this rare tumor, the patient was followed every 6 months for early detection of local recurrence or metastasis. The follow-up workup included physical examination, computed tomography (CT) scan, and CA 72-4, which is a tumor marker for detecting tumor-associated glycoprotein 72 (TAG-72), a mucin-like molecule found on the surface of cancer cells []. The patient had no evidence of recurrence or metastasis during a 14-month follow-up (). |
A 76-year-old man presented with right-sided paralysis. Cerebral magnetic resonance imaging revealed a watershed infarction in the left hemisphere, and cerebral angiography revealed severe stenosis of the left intracranial ICA (distal cavernous-infraclinoid portion), without stenosis of the cervical ICA [ and 2a]. Collateral flow through anterior communicating artery was absent, but through posterior communicating artery was confirmed insufficiently. Single-photon emission computed tomography (SPECT) demonstrated that his left cerebral hemisphere reserve was reduced. He immediately started conservative treatment with antiplatelet drugs and rehabilitation, and extracranial-intracranial bypass was planned within 3 months after the ictus. However, he presented with severe visual acuity disturbance in the left eye 1 month after onset, and ophthalmic evaluation revealed a central retinal artery obstruction in the left eye, and the ocular circulation time evaluated by arm retinal circulation time (A-R time) was prolonged.\nDecreased blood flow to the ocular artery due to stenosis progression of the left ICA was suspected. Urgent cerebral angiography performed 2 days after the occurrence of visual impairment demonstrated that the left ICA was almost completely occluded, and the ocular artery was confirmed through anastomosis from the external carotid artery []. Percutaneous angioplasty (PTA) was performed to improve visual acuity. The ICA flow improved immediately after PTA but decreased thereafter. After several attempts at PTA, an intracranial stent (Wingspan, Stryker Neurovascular, Salt Lake City, Utah, USA) was placed to maintain the improved flow of the ICA []. After stent placement, the antegrade ocular artery flow recovered, and his vision improved immediately.\nThe A-R time was reduced from 54 s to 11 s, and retinal angiography also showed improvement in the visualization of blood vessels [ and ]. In addition, SPECT showed improvement in the left cerebral blood flow reserve [-], and he was discharged 2 weeks later without deficits. During the 1-year follow-up, his visual acuity and ICA flow remained stable. |
A 30-year-old Japanese man with no personal or familial psychiatric history was admitted to a psychiatric hospital for manic episode. His medical history included head trauma by traffic accident at the age of 6 years. He experienced severe impaired consciousness for 2 weeks after the head trauma. After discharge he showed a flat affect, which resolved after 3 months. Thereafter, he showed an inability to control his emotions for some time, but this eventually resolved, and he lived a stable life without major problems.\nAt the age of 12 years, the patient passed an entertainment office audition and subsequently performed on TV programs and on the stage. During high school, he studied in Australia and obtained tattoos on his whole back, which is uncommon behavior for a Japanese student. During college, he was the captain of a dance team. At 21 years, he had a child. Thus, his behaviors from the ages of 12 to 21 could be interpreted as showing a disinhibited tendency. After college graduation, he initially worked on a motor race rescue team for 4 years before switching to work in his family’s company. At 29 years, he experienced a problem with his relationship with his sister and became estranged from his family.\nThe patient began working as an apparel retailer and was taking lessons for Japanese classical dance and classical ballet. He frequently went dancing in nightclubs, reversing his day/night cycle. According to the patient’s wife, the patient stated, “I communicate with the universe” during this period. In June of 2016, at the age of 30, he was scouted in a night club by a record company, and he ended up paying the company several thousand dollars to produce his music video. In addition, his schedule grew busier because of training in both Noh theater (Noh is a form of traditional Japanese dance-drama) and ballet. In August, after seeing a Noh performance, he showed symptoms of megalomania by claiming “Because Noh is a world heritage, I would not be guilty even if I killed a man.” and “The world goes around me.” At his grandmother’s funeral at the end of September, he stated, “I worship nature and attend as God”. After the funeral, he suddenly went to a shrine and prayed to cleanse his unclean body. When he returned home, he quarreled with his mother, yelling and behaving incoherently.\nOn October 1, he dashed naked out of the house and repeatedly made emergency calls to police to ask, “Who am I?” He also brazenly took many books out of a bookstore without paying. Because of these incoherent behaviors, he was voluntarily admitted to a hospital. Although pharmacological treatment with aripiprazole was initially adopted, his symptoms continued to deteriorate. The patient purchased an expensive Japanese sword and telescope via a telephone service and displayed sexually disinhibited behavior in the hospital. The admission was changed from voluntary to involuntary. On November 8, the patient was referred to our hospital to have a more detailed examination. On admission to our facility, the patient was apathic rather than hyperactive. Electroencephalography and brain computerized tomography (CT) revealed no notable results. Magnetic resonance imaging was not available due to his tattooed back, but single photon emission computed tomography (SPECT) revealed low blood flow in the OFC and high blood flow in the PCC (Fig. a). According to the Wechsler Adult Intelligence Scale–Third (WAIS-III), the patient’s total IQ was 85 (verbal IQ: 90, performance IQ: 82). In particular, his WAIS-III scores for letter–number sequencing, picture completion, digit symbol-coding, and symbol research were low. He scored an 81/85 (low average) on the Behavioral Assessment of the Dysexecutive Syndrome (BADS), and his score on the Wisconsin Card Sorting Test (WCST) was normal. His performance on the trail-making test was accurate, but he processed slowly. Together, these tests revealed that the patient had a slow processing speed and lowered attention (Table ).\nAfter admission to our hospital, the patient was treated with carbamazepine in addition to aripiprazole. He gradually became coherent enough to claim that he did not have any fantastical feelings, and finally his megalomania and irritability disappeared. 9 months after discharge, in February 2017, he reported that his life had returned to how it was before the onset of mania. Reflective of this recovery, he showed a WAIS-III total IQ of 96 (verbal IQ: 97, performance IQ: 95). Other tests further confirmed his improvement, although he still showed a low processing speed and low attention (Table ). Hypo-activity in the OFC and hyper-activity in the PCC were no longer detected by SPECT (Fig. b). |
A 75-year-old woman with sudden right hemiparesis was admitted to our hospital with a diagnosis of acute ischemic stroke. She was treated with intravenous tissue plasminogen activator and thrombectomy in the left M2 segment of the middle cerebral artery, after which her symptoms improved remarkably (modified Rankin scale 0). The patient met the criteria of ESUS, and a thorough examination was performed to determine the cause. Transesophageal echocardiography showed minor plaque formation in the aortic arch (a potential cause of aortogenic embolism) and absence of a patent foramen ovale (a potential cause of paradoxical embolism). The patient had the HAVOC score of 4 (hypertension, age). Although CPAF was not identified by telemetry at admission, it was suspected and we decided to implant an ICM device. However, SIT was observed by chest X-ray. Thus, the ICM device was implanted into the right chest in a symmetrical position (Figure ).\nWe used fluoroscopy to detect the precise location of the heart. In order to detect the P waves clearly in ICM, we did a simple premapping with surface ECG. During premapping, ECG was recorded at the angle of 0 degrees, 30 degrees, 60 degrees, and 90 degrees in 3rd and 4th intercoastal space respectively, and the site was chosen with the highest QRS/T ratio and relatively higher P wave. ECG at the angle of 60 degrees in 3rd intercoastal space had the best ECG, and an ICM (BIOMONITOR IIITM; Biotronik) was then inserted into the right margin of the 3rd intercostal sternum along the long axis shadow of the heart at 60 degrees angle using the provided tools. The procedure duration was 5 minutes, and the fluoroscopy time was 15 seconds. There were no complications during the procedure.\nThe first AF episode was successfully detected at 28 days after ICM insertion (Figure ). Within 6 months, the AF lasting for more than 1 minute was detected 28 times and AFL was detected 15 times in total. Figure shows the sudden drop rate alarm, the feature unique to the BIOMONITOR III™ device. It shows the moment when rhythm changed from AF to sinus rhythm. P wave can be clearly seen during the sinus rhythm. AF was then detected on a number of occasions.\nAspirin was used initially for prevention of recurrent ischemic stroke. However, we changed to edoxaban after CPAF was detected. |
This is a 38-year-old woman – with no history of pregnancies – with a cutaneous form of SLE diagnosed 12 years ago. Five of the criteria of the American Rheumatism Association from the year 1992 are present: butterfly rash; discoid lupus lesions; arthritis/arthralgias; immunological abnormalities (ANA, ADNA). Oral therapy with methylprednisolone and hydroxychloroquine has been administered for 12 years. SLE has been in remission – according to the clinical and laboratory results – for the last 2 years, and patient has been on maintenance therapy with reduced doses of the medical treatment. PAP smear 3A, and visible from colposcopy gross leukoplakia area (L2) were found on a prophylactic examination of patient; the leukoplakia area was with a section of mosaic (M2), and sizes of 1.5 × 2 cm in the region of exocervix, extending into the cervical canal. A loop excision of the described section was performed with histological result of adenocarcinoma of uterine cervix and involvement of the resection lines. A repeated examination of histological preparation was accomplished, which confirmed the initial result – villoglandular adenocarcinoma with infiltration to the native squamous epithelium of uterine cervix. Patient was admitted at this Clinic for continuation of treatment ().\nIn view of the preceding disease (SLE) and histological result, a decision was made – after a thorough discussion – for performing of robotic radical hysterectomy with bilateral adnexectomy, combined with pelvic lymph node dissection (DaVinci Si®, Intuitive Surgical). Based on the assessment of the attending rheumatologist, a preoperative preparation with Methylprednisolone 60 + 60 + 40 + 40 + 20 mg was appointed, which began two days before the operation. Magnetic resonance imaging (MRI) of lesser pelvis, and pelvic and paraaortic lymph node chains, was performed, in which changes in uterine cervix were found with diameter of less than 10 mm () and lymph nodes enlarged within referent range of up to 10 mm that were not suspected to be metastatic (). The operative intervention passed with no complications, with a total surgery time of 185 minutes, console time of 160 minutes, and total blood loss of 50 ml. The histological examination showed a high grade of glandular endocervical dysplasia (HCGIN), with no presence of residual carcinoma after the conization performed ().\nSeventeen lymph nodes were removed on pelvic lymph node dissection – two of them were sent for frozen section analysis due to their enlarged sizes, with no metastases found in them (). The final pathological examination did not reveal any lymphatic metastases.\nThe early postoperative period passed with significant lymphorrhea – respectively, 300 ml, 500 ml, 600 ml, 500 ml, 600 ml, 700 ml, 700 ml from the first to seventh day – which required a more prolonged hospitalization. Patient was discharged on seventh postoperative day, after which the abdominal drain was removed, and therapy was appointed for improvement of the condition of lymphedema. Within one month the lymphorrhea was significantly limited. In view of the histological result and according to the decision of the Oncological Committee, radiotherapy was not appointed. In the course of follow-up, presence of lymphocele was found three months after the intervention, in the area of left and right external iliac lymph node chains, with sizes of respectively 3 × 2 cm and 2 × 2 cm; there was reduction of their sizes, with one centimeter each, on the sixth month, as a result of only medicinal treatment. Their drainage was not required due to the absence of symptoms. On the last follow-up examination, 9 months after the operation, patient felt well, with no data of persistence of disease. |
A 15-year-old boy with leucoderma was admitted to our service reporting pain while chewing, bulging in the left mandibular angle and aesthetic discomfort because of face asymmetry, but no relevant clinical facts existed.\nPhysical examination revealed a facial asymmetry, a palpable mass in the angle region of left mandibular, pain, limitation of oral opening and bulging in region of teeth 36 and 37, tooth 38 was lacking. The diagnosis exam, the panoramic radiographic and computed tomography of the face showed suggestive images of injury involving the body and left mandibular ascending ramus ( to ).\nPatient’s familiar medical history did not show significant data. Reconstruction possibilities were discussed with patient and his family including the use of autogenous bone grafting of iliac crest and/or off-label use of rhBMP-2. Advantages and disadvantages of both possibilities were presented and patient and family decided for rhBMP-2 off-label. After evaluation of surgical risk and treatment of teeth 36 and 37 canals had are done, the patient underwent surgery in a hospital environment under general anesthesia and nasotracheal intubation. We performed an incision with extension of mandibular ascending ramus to vestibular region to first lower left molar, being elevated the mucoperiosteal graft, therefore achieving a partial exposition of the injury. An osteotomy was need using a 701 drill for higher exposure of the injury (). The dissection was simple because the tumor was encapsulated ().\nRecipient site was prepared and, by using the bone grafting technique, the rhBMP-2 was prepared associated with Bio-Oss® (Geistlich Pharma AG, Wolhusen, Switzerland) and fairly adapted to surgical site with subsequent adaptation of titanium mesh 1.0 system that promoted a mechanical barrier. A plate 2.0 system was used on external oblique line to obtain higher stability ( and ).\nA polyglactin 910 wire suture was used in deep plans and on the mucosa. Material obtained was forwarded for anatomopathological examination, and the definitive diagnosis was unicystic ameloblastoma, intraluminal variant with plexiform pattern ().\nImmediately after surgery, the patient had an edema compatible with the procedure and discreet paresis of left inferior alveolar nerve. We observed a late inflammation reaction. Clinical and radiographic follow-up was done after 1, 3 and 6 months with satisfactory aesthetic and functional result ( and ). |
A 57-year-old man was admitted to the cardiology clinic with a 20-year history of gradually increasing breathlessness on exertion and, in the previous 10 days, orthopnoea and paroxysmal nocturnal dyspnoea. Physical examination found irregular pulse with diastolic rumbling murmur. The electrocardiogram revealed atrial fibrillation with a ventricular rate of 120 beats/min and accompanying T wave abnormalities and minimal ST depression in inferior derivations. Chest radiograph revealed double shadow on the right cardiac silhouette and prominent pulmonary trunk with increased vascular markings. Transthoracic echocardiography revealed a mitral stenosis with a mitral area of 0.7 cm2, ejection fraction of 68%, and normal segmental wall motion, mild aortic regurgitation, severe pulmonary hypertension and moderate left atrial enlargement. Severe pulmonary hypertension was confirmed by Doppler (pulmonary arterial pressure = 106.8 mmHg). The patient received 20 mg furosemide, 40 mg spironolactone, 0.125 mg digoxin and low molecular weight heparin by subcutaneous injection. With the relief of dyspnea, coronary angiography was performed for preoperative evaluation of mitral valve replacement. Right anterior oblique caudal view showed coronary artery fistulae between the left circumflex artery and the left atrium. One large fistula originated from the first obtuse marginal branch and the second obtuse marginal branch, and another fistula originated from the third branch of the second obtuse marginal branch (). Antero-posterior oblique projection of the left coronary angiogram revealed the coronary artery fistulae drained into the left atrium with two terminal orifices ( and ). The fistulae were hemodynamically significant, and closure was indicated. In the operation, utilizing cardiopulmonary bypass, the mitral valve was replaced by a 27-mm ATS Open Pivot Bileaflet Heart Valve (ATS Medical Inc., Minneapolis, MN). At the time of mitral valve replacement, the fistulae were successfully ligated through not only left atrium but also the left circumflex coronaries’ side. Postoperatively, the patient made an uncomplicated recovery. At 3 months postoperatively, the transthoracic echocardiography was performed, which revealed pulmonary arterial pressure of 62 mmHg, and the closure of the fistulae was confirmed by 128-slice computed tomography ().\nCoronary artery fistula is a rare anomaly connecting coronary arteries to cardiac chambers or great vessels, which are rarely detected during routine angiographic evaluation. The frequency of congenital coronary fistulas is reported at approximately 0.1%[. Mitral isthmus ablation, which is an important component of catheter ablation for persistent atrial fibrillation and mitral isthmus-dependent flutters, become one of the reasons to cause a fistula between the left circumflex artery and the left atrium[. The main sites of origin are the right coronary artery (55%), the left coronary artery system (35%), and both coronary arteries (5%). The main termination sites are right ventricle (40%), right atrium (26%), and pulmonary arteries (17%). Less frequently, they may drain into the superior vena cava or coronary sinus and less frequently into the left atrium or left ventricle[. Although a similar fistula is reported with one entrance draining into the left atrium in the literature[, to our best knowledge, this is the first case of the circumflex to left atrium fistulae with two terminal orifices.\nAlthough asymptomatic in the vast majority, coronary artery fistula may cause chronic myocardial ischemia and angina, congestive heart failure, myocardial infarction, pulmonary hypertension, rhythm disturbances, subacute bacterial endocarditis, thromboembolism, rarely aneurysmal segment rupture, and sudden death[. Small fistula usually does not cause hemodynamic impairment. However, high volume shunts via left circumflex coronary artery to the left atrium may result in increasing volume load of the left atrium and pulmonary arterial hypertension. In our reported cases, coronary fistulae and mitral stenosis appeared together may just be a coincidence, and pulmonary arterial hypertension may be due to the chronic left atrial volume overload caused by congenital coronary-left atrial fistulae and mitral valve stenosis. The best way to manage coronary cameral fistulae is not well known due to the rarity of the condition. Surgical repair of coronary artery fistulae is safe and effective, with low risks and favorable late outcomes[, as described in this case. |
A 70 year old female with chronic obstructive pulmonary disease (COPD) on home oxygen with no history of previous surgeries presented with lower abdominal pain for a year which has become constant and severe for 2 days. She had nausea, vomiting and was not passing flatus for 3 days. On physical exam vital signs were within normal limits. Abdomen was mildly tender in the suprapubic region with no peritoneal signs. There are no palpable hernial swellings or lymph nodes. On adduction and medial rotation of left thigh, patient complained of increased pain in her medial left leg. Laboratory test showed increased lactic acid level. Abdominal CT scan () was done which showed dilated small bowel loops and findings suspicious for incarcerated small bowel containing left obturator hernia. Patient refused nasogastric tube gastric decompression. Patient was taken to the operating room for surgical management of the incarcerated hernia. In the operating room, after achieving penumoperitoneum, a 5 mm 30 ° laparoscope was introduced and dilated small bowel was noted with free peritoneal fluid. Two 5 mm port was placed on each side of the abdomen. After displacing distended small bowel loops, a loop of small bowel herniating into the left obturator canal was observed. Attempt to reduce left obturator hernia by gentle traction was successful (). A Richter’s type (partial enterocele with protrusion or strangulation of only part of the circumference of the intestine's anti mesenteric border) of Obturator hernia was noted and gangrenous portion of approximately 3 cm of small bowel was noted.\nA small transverse pfannensteil incision (5 cm) was made just above the pubic symphysis and the loop of gangrenous small bowel was delivered out and resected with a GIA stapler. After that peritoneum and the fascia was closed and pneumoperitoneum recreated. Subsequently a Vicryl woven mesh cut to appropriate size was tacked around the left obturator canal to buttress the hernial defect. The neurovascular bundle and the obturator foramen was clearly visible laparoscopically and was protected. No entrapment of bowel or omentum was observed at the end. Pneumoperitoneum was desufflated and ports were removed. Post operatively patient made an uneventful recovery. |
A 60-year-old Caucasian man was transferred from a regional hospital to a tertiary referral centre for the emergency management of hypercalcaemic hyperparathyroid crisis. He initially presented with a two-week history of weakness and lethargy and a one-week history of vomiting, polyuria and polydipsia. He became acutely confused in the 24 hours prior to his admission to our hospital and registered 12 on the Glasgow Coma Scale. On examination he was normotensive with a regular pulse of 70 beats per minute. There was a left-sided mass in the anterior triangle of his neck measuring 3 × 3 cm. The mass was firm, regular, non-tender and mobile. See Figure and Table for results of the initial laboratory investigations.\nAn ultrasonography of his neck showed a 4 × 3 cm large cyst in the left lobe of his thyroid gland. His parathyroid glands were not visualised. A 99 mTc-sestamibi scintigraphy scan was performed, and no evidence of a parathyroid adenoma was found. An electrocardiogram revealed acute changes with ST depression in leads II, III, aVF and V2 to V6. An echocardiogram showed good left ventricular failure (LVF) with an ejection fraction of 63%.\nInitial management included aggressive fluid resuscitation, cardiac monitoring and the administration of intravenous bisphosphonates. A left thyroid lobectomy and left lower parathyroidectomy were performed. At the time of operation, a haemorrhagic cyst with a parathyroid gland within it was visualised. A biopsy was taken from the left upper parathyroid gland. Histology revealed a 4 cm parathyroid carcinoma within the cyst which was fully excised. The cyst had a macroscopic measurement of 6 × 6 × 5 cm. The wall of the cyst contained a well-circumscribed, unencapsulated soft tissue mass measuring 4 × 2.5 × 0.7 cm. It was light yellow-tan in colour and firm in consistency. There was a small amount of normal thyroid parenchyma within the specimen. The biopsy of the left upper parathyroid gland revealed normal parathyroid parenchyma without diagnostic abnormality.\nOur patient remained intubated and ventilated overnight in the intensive care unit. His metabolic laboratory profile resolved quickly following the surgery [Figure , Figure , Figure ]. A lower respiratory tract infection delayed his initial recovery. He was discharged home on oral calcium supplementation 24 days after surgery. He has been followed up for 6 months so far without any complications or disease recurrence. |
A 12-year-old boy presented with the signs of raised intracranial pressure including a progressive headache, nausea/vomiting, and lethargy following a minor head trauma. The patient had a history of prematurity (gestational age of 30 weeks) due to premature rupture of the membranes. Diagnosed with hydrocephalus in the early infancy, he had undergone ventriculoperitoneal (VP) shunting at the age of 6 months and had several shunt revision surgeries since then. The medical records including previous brain imaging and the reports of surgeries performed in other institutions were not available. There was no notable family history. Physical examination revealed delayed filling of the shunt's pump. Neurological examination was unremarkable.\nA brain MRI with and without contrast showed dilation of the cerebral aqueduct and enlargement of the fourth ventricle. A cystic area existed within the fourth ventricle, extending through the cerebral aqueduct toward the third ventricle. The cyst contained a fluid with the signal intensity similar to the cerebrospinal fluid (CSF) and was not enhanced after administration of the intravenous contrast. These findings were compatible with an intraventricular arachnoid cyst.\nBased on these findings, we scheduled two procedures: Replacement of the pump of the shunt and endoscopic fenestration (ECF) of the cyst. A midline suboccipital craniotomy was made. Cerebellar tonsils were identified and separated, and a straight rigid endoscope (Gaab®, KARL STROZ©, Tuttlingen, Germany) was inserted into the fourth ventricle through the median aperture. Subsequently, the inferior wall of the cyst was visualized and was delicately punctured; a clear CSF-like fluid was drained from the cyst to the ventricular cavity. The endoscope was then gently introduced into the cyst, which allowed inspection of the interior cavity as well as the superior wall of the cyst. There was no solid or nodular tissue within the cyst, and no signs of infection or hemorrhage were noted. The cyst had extended to the cerebral aqueduct, entering the third ventricle, and both internal cerebral veins were visible through the superior wall of the cyst. An opening was made in the most anterior and superior part of the cyst wall, resulting in a free flow of CSF from the third ventricle. After the surgery, the patient recovered favorably and was discharged in a good condition.\nThe patient remained asymptomatic during the subsequent follow-up visits. In a visit 4 years postsurgery, evaluation of cognitive function revealed an impairment; the patient was not able to proceed more than the fifth grade. The function of VP shunt was satisfactory on examination. MRI study with and without contrast revealed the persistence of the cyst albeit with a reduced size as compared to before the ECF. The cyst was not associated with any significant mass effect []. |
An 84-year-old Caucasian female presented to the local emergency department with a recent history of severe headaches, myalgia and shoulder and calf muscle pain. She had been recently diagnosed with polymyalgia rheumatic in the context of a three-month history of fatigue, weakness and fluctuating fever and was already on tapered dose of corticosteroids. The patient was otherwise known for atrial fibrillation on anticoagulation, atherosclerotic coronary heart disease, hypertension, and dyslipidemia. Past ocular history was unremarkable except for remote bilateral phacoemulsification cataract surgery.\nOn presentation at the ER, the patient was afebrile. There was no temporal artery tenderness or jaw pain, and C-reactive protein was mildly elevated at 52 mg/L. Platelets count was 313 x 109, white blood cells count was 19.9 x 109. No erythrocyte sedimentation was done. The tests were otherwise unremarkable.\nThe patient was prescribed oral prednisone 50 mg once daily and admitted. Twenty-four hours later, solumedrol 1g IV was prescribed once daily for 48 hours for a presumed diagnosis of polymyalgia rheumatica and atypical temporal arteritis as the symptoms worsen and C-reactive protein increased to 197 mg\L. At this point, the patient had no ocular symptoms.\nDespite initial improvement, the patient relapsed after three days with worsening of symptoms and a new retro-orbital pain of the right eye. On examination by the ophthalmologist, the patient had 20/20 vision bilaterally, with normal adnexal structures and extraocular movements. Intraocular pressure was within normal limits and the visual field exam showed a superonasal quadranopsia. Anterior segment examination showed no sign of inflammation and fundus examination of the right eye showed a white, vascularized chorioretinal mass in the inferotemporal quadrant. The examination was unremarkable for the left eye. The diagnosis given by the general ophthalmologist was a possible malignant tumor and the patient was referred to a retinal specialist for further evaluation.\nInvestigations by an infectious disease specialist led to a concomitant diagnosis of emphysematous cystitis. Piperacillin tazobactam 3.3 g IV q 6 hours was prescribed to the patient and given the partial response to antibiotics, the corticosteroids were gradually eliminated. Piperacillin tazobactam was changed for ampicillin 2 g IV q 6 hours.\nOne day later, a PET scan was performed with results suggesting an inflammatory or infectious etiology. It showed two pulmonary opacities of unknown etiology and hypermetabolism in the right calf muscle, thought to be a hematoma. Large vessel arteritis was absent.\nThree days later at follow-up, the patient’s vision in the right eye was significantly decreased from 20/20 to counting fingers. Panuveitis was present with a stage 4 vitreal haze on fundus exam. A B-scan was performed and revealed that the mass had increased in size and was extending into the vitreous. A diagnosis of endogenous endophthalmitis with a probable fungal etiology was made. A vitrectomy with a chorioretinal biopsy of the lesion was performed the next day. Intravitreal amphotericin B 5 mg/0.1 mL, vancomycin 1 mg/0.1 mL and ceftazidime 2.25 mg/0.1 mL were administered during the procedure. A biopsy of the left calf mass was made by needle biopsy.\nThe culture of the vitreous () and biopsy of the left calf mass () showed high quantities of Nocardia nova. Disseminated Nocardiosis was therefore the final diagnosis.\nAmpicillin was stopped and changed for meropenem 2 g IV q 8h and TMP-SMX 400 mg IV q8h for 4 doses. A dose of 2 mg of intravitreal ceftriaxone was also planned. After the intraocular injection and the parenteral antibiotics, a regression of the intraocular mass, right calf mass and two pulmonary opacities was observed. A considerable improvement of the patient’s general condition was also noted. However, the patient decided to cease treatment given her age and other comorbidities. She was transferred to palliative care and died 18 days later. |
A 66-year-old male with a history of Hepatitis C treated in 2009 with Ledipasvir/Sofosbuvir (Harvoni®) initially presented to the Medical Oncology team in Hackensack University Medical Center, NJ with abdominal discomfort and distention for 45 months associated with an approximate 15 pounds of weight loss. He underwent CT scan of the abdomen with contrast and was found to have a large 15.0 × 11.0 × 13.0 cm heterogenous mass attached to the left lobe of the liver, with compression of surrounding intra-abdominal contents including the stomach and transverse colon, as well as the abdominal wall (Figure ). Subsequent percutaneous biopsy was performed for genetic testing, and results demonstrated hepatocellular carcinoma with extensive necrosis. He was then referred to the Surgical Oncology team for further workup and management. Follow up MRI demonstrated two additional satellite lesions consistent with multifocal hepatocellular carcinoma without evidence of invasion into surrounding vital structures. Surgical intervention was deemed necessary given the size of the mass and symptomology. A baseline AFP was 6.1, he was designated as a Child Pugh Score of 8 and he had a MELD Score of 9.\nIn the operating room, two large-bore intravenous catheters were placed but fluid was kept at a low rate along with placement of a foley catheter. A laparotomy was performed; making a midline sub-xiphoid incision, which was extended down to the pubic symphysis due to the size of the tumor (Figure ). Multiple friable blood vessels were noted at the umbilicus. The omentum as then resected, freeing the anterior abdominal wall. The transverse colon was then carefully separated away from the tumor using a GIA stapler. The transverse colon and its mesentery were preserved and protected from the dense mass. It was also noted that the distal stomach was adhered to the mass along the greater curvature. A sleeve gastrectomy was also completed, requiring three firings for completion.\nAt this time, the retroperitoneal attachment to the duodenum was carefully dissected, followed by separation of the pancreatic head. This allowed the tumor to be separated from the retroperitoneum and lifted out of the abdomen. A Pringle maneuver was performed using a vascular clamp, and the satellite lesions were transected using parenchymal ablation using the NeuWave Microwave Ablation system. The midline laparotomy was closed in the usual fashion.\nOn gross inspection, the resected specimen was a 21.0 × 20.0 × 10.7 cm, tan-yellow mass weighing 1948 g (Figure ). The tumor is partially adhered to a portion of the stomach and surrounded by adjacent background liver. Microscopically, the tumor shows solid and trabecular growth pattern with round nucleus, prominent nucleoli, abundant cytoplasm and distinct cell border typical for hepatocellular carcinoma (Figure ). Mitotic figures are frequent and tumor necrosis is also seen. Multiple satellite nodules are seen adjacent to the main tumor and extensive small vessel invasion is present (Figure ). The tumor also invades into the gastric wall. The background non-tumor liver shows well-formed cirrhotic liver with mild to moderate activity. The attached portion of the stomach demonstrated negative margins (R0). The specimen from the partial hepatectomy, which was the satellite lesion of segment 3, demonstrated a similar pattern.\nThe post-operative course was significant for urinary retention. Given his history of benign prostate hyperplasia (BPH), a foley catheter was reinserted. He had also developed an ileus requiring a nasogastric tube, which was removed four days later. The patient developed serosanguineous drainage from his incision site after a bout of heavy coughing. A Prevena VAC was placed but was discontinued one week later when the output was reduced to zero, and he was discharged from the hospital later in the day. |
A four-year-old boy with acute lymphoblastic leukemia (ALL-L2) was admitted to the hospital with diagnosis of febrile neutropenia with an absolute neutrophil count of zero. He was at the end of remission induction phase of ALL-BFM 2003 protocol. Neck stiffness and acute mental status changes were observed on his physical examination. His cranial MRI showed generalized hydrocephalus; lumbar puncture was performed and analysis of the cerebrospinal fluid (CSF) specimen revealed 312 white blood cells/μL, 23 mg/dL of glucose, and 126 mg/dL of protein. Vancomycin and meropenem were initiated empirically with the diagnosis of meningitis. Repeated lumbar puncture performed since his symptoms persisted despite the five days of antibacterial treatment; CSF analysis revealed 160 white cells/μL, 53 mg/dL of glucose, and 160 mg/dL of protein. Candida albicans grew in CSF culture in 2 days; therefore, intravenous liposomal amphotericin B with a dose of 5 mg/kg/day was added to the antibacterial treatment regimen. Because the signs and symptoms of hydrocephalus continued to progress, an external ventricular drainage device was inserted. During the follow-up, he showed significant clinical improvement in addition to improved CSF profile. Liposomal amphotericin B was discontinued at the 30th day of treatment, because CSF cell count and glucose and protein levels returned to normal levels and the last three CSF cultures kept sterile. The drainage device was replaced by a ventriculoperitoneal shunt and the patient was discharged.\nTwenty five days after discharge, he was readmitted to the hospital with signs and symptoms of hydrocephalus. Cranial MRI revealed generalized hydrocephalus and ventriculitis. Candida albicans grew in the CSF culture again. Liposomal amphotericin B was given both intravenously (5 mg/kg/day once daily) and intraventricularly (1 mg/day, dissolved in 3 mL of 5% dextrose, and shunt was closed for 4 hours after 4 administration). The patient also underwent extraventricular drainage to treat the hydrocephalus. Neurological or nonspecific side effects like vomiting, headache, or paralysis were not observed during intraventricular amphotericin B treatment. Intraventricular liposomal amphotericin B treatment was discontinued at the 12th day because the sterilization of CSF was achieved in addition to normalization of CSF WBCs, glucose, and protein levels. Intravenous liposomal amphotericin B treatment was continued for six weeks. Ventricular drainage device was replaced by a new ventriculoperitoneal shunt before discharge. |
Herein we present a case of a 13-year-old girl who was admitted to the Department of Paediatric Surgery, Traumatology, and Urology in Poznan. She reported chest discomfort, difficulty breathing, and a cough. A month prior to admission she had suffered from a respiratory tract infection. She was delivered at term as a firstborn child with an Apgar score of 10 and a normal birthweight of 3360 g. At the age of three years, she was diagnosed with erythrocyturia. Clinical examination on admission revealed pallor, left side rib prominence, and diminished vesicular breath sounds on the left (from base to shoulder blade angle). X-ray showed atelectasis of a large part of the superior left lobe and obstruction of the bronchus leading to the third segment of the left lung ().\nComputed tomography indicated a soft tissue lesion within the bronchus ().\nBronchoscopy was performed, which revealed a pathological mass filling the left main bronchus ().\nDespite the initial recanalisation of only the inferior lobar bronchus, the condition of the patient improved, and a week later the procedure was repeated to clear the superior lobar bronchus. Histopathological examination of the mass indicated an inflammatory myofibroblastic tumour. In the following two months, the tumour recurred and caused symptoms of breathing difficulty. Seven subsequent bronchoscopic decongestions were performed. Due to recurrence, a tissue biopsy was repeated and a second pathomorphological centre was involved in the histopathological interpretation of the biopsy sample. Cytomorphology showed typical Touton cells, and immunohistochemistry was negative for CD1a, s-100, and langerin and positive for CD163. With these results, the patient was diagnosed with juvenile xanthogranuloma. She was referred to an oncological centre for consideration of systemic chemotherapeutic treatment with vinblastine or cytarabine. The patient did not undergo chemotherapy due to the risk of adverse effects, and because the pathological mass was of a stable diameter. The tumour will be monitored, and chemotherapy considered at each future evaluation. |
A 35-year-old African American woman with a past medical history of an episode of pericarditis presented to the Emergency Department complaining of chest pain and fatigue of a weeks' duration. She reported a sudden onset of pleuritic chest pain associated with cold-like symptoms but no complaints of any fever or chills. In addition, she had reported difficulty lying supine, which caused some dyspnea along with some discomfort under the left breast. She had gone to the Emergency Department of another hospital, two days ago, and was discharged with the diagnosis of a viral syndrome. Upon further investigation, she reported a possible diagnosis of pericarditis when she went to another hospital in 2010. She then went on to give a history of hair loss and arthralgia in her hands. There was no other past medical history and family history was significant for SLE in a half-sister. She had 3 children and a 1st trimester miscarriage. She denied alcohol and drug use.\nOn admission, physical examination revealed a young woman with mild distress with tachycardia of 102. Other vital signs were within the normal limit. Only other significant findings on physical examination were a pericardial friction rub and nonscarring alopecia. Electrocardiogram (ECG) showed concave ST elevations in the inferior and lateral leads (). Chest X-ray (CXR) showed no abnormalities (). Labs including complete blood count and comprehensive metabolic panel were within normal limits. The first two sets of cardiac troponins were within normal limits. She was initially diagnosed as having pericarditis and was started on Indomethacin with Pantoprazole, but she could not tolerate NSAIDs. Hence, she was started on Colchicine with Prednisone. Additional labs, including antinuclear antibody (ANA), antidouble stranded DNA (Anti-ds DNA), and complement C3 and C4 were sent for further evaluation.\nLater during the day, she complained of increasing shortness of breath with chest pain and found to be desaturating down to 87% on room air. She was noted to have crackles and trace pedal edema on physical examination. Arterial blood gas revealed hypoxemia. Her oxygen saturation improved after placing her on a nasal cannula with 2 liters of oxygen. A repeat chest X-ray showed an interval development of small bilateral pleural effusions (). Repeat labs including Beta natriuretic peptide (BNP) and troponin were sent. A transthoracic echocardiogram (TTE) revealed moderately reduced left ventricular systolic function with an Ejection Fraction of 35–40% and a moderate pericardial effusion without any signs of tamponade ().\nThe patient was transferred to the intensive care unit (ICU) for closer monitoring and started on lasix, metoprolol, and lisinopril. Troponin trend was 0.60 ng/ml > 0.49 ng/ml > 0.74 ng/ml, which was expected for myocarditis, and no heparin drip was indicated. She was very weak with marked dyspnea on exertion and could not get out of bed. Creatinine phosphokinase (CPK) was 435 U/L and BNP was elevated at 488 pg/ml. ANA returned positive at 1 : 160 with low C3 of 74 mg/dl and C4 of 25 mg/dl. SM/RNP, dsDNA, RPR, HIV, and HBsAg were negative. She was subsequently diagnosed with SLE, according to the new criteria, with pericarditis, myocarditis, and small pleural effusion but also ANA, low C3, and hair loss with alopecia. Prednisone was held and methylprednisolone was started. Colchicine was also held, as the patient developed diarrhea. Vitals remained stable with improvement in overall clinical status while the patient was in ICU but continued to feel weak. She was then transferred to a tertiary care center and had a cardiac magnetic resonance (CMR) imaging 3 days later, which showed normal biventricular size and systolic function, edema, and subepicardial enhancement in the lateral wall and confirmed the presence of myocarditis. |
A 10-year-old male castrated domestic shorthair cat presented to the author's hospital for progressive wheezing, increased respiratory effort, and dyspnea of 3 month's duration. On the day of presentation, the owners noted the cat occasionally had open mouth breathing and was breathing harder after walking up/down the stairs. The cat was taken to the primary care veterinarian 2 months prior, where radiographs revealed a soft tissue opacity in the trachea at the thoracic inlet. Tracheal collapse was suspected, and the cat was prescribed doxycycline (5 mg/kg PO q12h) and theophylline (100 mg PO q24h) which had no immediate effect on the clinical signs. The cat had an adverse reaction to the medications, causing hypersalivation, vocalization, and hyperactivity causing the owners to discontinue the medications. Following this, the primary care veterinarian recommended a surgical consultation.\nOn initial presentation, the cat was bright, alert, and responsive with pink and moist mucous membranes and a body condition score of 4/9. There was increased inspiratory and expiratory effort throughout the full respiratory cycle with wet sounding respiratory noises consistent with a partial tracheal obstruction. Cardio-thoracic auscultation revealed only moderate referred tracheal noises. The rest of the physical examination was unremarkable. The primary care veterinarian's radiographs were reviewed and were found to be most consistent with a soft tissue partial obstruction of the trachea at the thoracic inlet. Unfortunately, the thoracic limbs were positioned over the area of interest, making the radiographs difficult to fully assess.\nRecommendations included a computed tomography of the cervical and thoracic region for surgical planning as well as tracheoscopy to obtain a small cup biopsy for histopathologic diagnosis with the possibility for debulking under the same anesthesia. Based on the result of the histopathologic diagnosis, either chemotherapy (for lymphoma) or surgical tracheal R&A would be recommended. Alternatively, a tracheal R&A with histology of the mass could be performed without first obtaining a definitive diagnosis. Additionally, tracheal stenting was offered as a palliative maneuver. After discussing the risks versus benefits of different options, the owners decided to proceed with repeat radiographs and a tracheal R&A, forgoing any preliminary advanced diagnostics.\nThe cat was hospitalized and placed in an oxygen cage overnight in preparation for surgery the following day. A complete blood count and a chemistry profile showed a mild metabolic alkalosis with a total CO2 of 26 mmol/L (likely compensation to chronic hypoventilation and hypercapnia), a mildly elevated total protein (9.1), and a mild leukocytosis (27.1 k) due to a moderate eosinophilia (6 k), mild monocytosis (800), and mild neutrophilia (16.5 k). The remainder of the biochemistry panel was unremarkable. Vitals were normal (T: 100.5 F, P: 150 bpm, R: 24 bpm), and the cat's breathing pattern remained static overnight. The next morning, an IV catheter was placed, and the cat was premedicated with a bolus of fentanyl (4 mcg/kg IV once), induced with Valium (0.25 mg/kg IV) and propofol (4 mg/kg IV to effect). The patient was intubated routinely, using a 5.0 mm endotracheal tube, terminating rostral to the tracheal mass. Anesthesia was maintained with Isoflurane and a fentanyl continuous rate infusion (4-8 mcg/kg/h IV). When the trachea was not intubated during the procedure (see below), propofol was used IV for total intravenous anesthesia (TIVA) as needed to maintain an adequate plane of anesthesia until reintubation. The cat was placed on a mechanical ventilator with the pressure set to 5 cmH2O and a rate of 5 breaths per minute. The cat was given prophylactic antibiotics of cefazolin (22 mg/kg IV q90 minutes) twice during the surgery. Intravenous isotonic crystalloids were run at 5 ml/kg/h during the surgery. Monitoring and support included capnography, pulse oximetry, esophageal temperature, blood pressure (doppler and oscillometric), and telemetry as well as a forced warm air circulator and a fluid line warmer for thermal support. Preoperative radiographs were obtained () which showed an approximately 2 cm intraluminal tracheal mass at the level of the thoracic inlet obstructing >50% of the tracheal lumen.\nA ventral, cervical, midline incision with a partial cranial sternotomy was performed. The cranial half-sternotomy was made using a battery-operated oscillating saw. The trachea was freed from surrounding tissues using blunt dissection. Upon inspection of the trachea, there was a 2 cm lymph node on the left lateral aspect of the trachea just cranial to the thoracic inlet. At the thoracic inlet, there was a firm, multinodular mass associated with approximately 2.2 cm of the trachea that was obstructing greater than 50% of the lumen. The local cervical lymph node was removed and submitted for histopathology. After placing vessel loops, stay sutures, and preplaced appositional sutures, the trachea was transected caudal to the mass. A sterile 4.5 mm tube was then placed through the surgical site and into the caudal tracheal segment to allow for continued mechanical ventilation and maintenance with isoflurane and oxygen. Approximately, 3 cm (corresponding to 5 tracheal rings) was resected, creating approximately 4-5 mm visual margins cranial and caudal from the gross tumor. The mass and associated trachea were submitted for histopathology. The oral endotracheal tube was exchanged for a sterile 4.5 mm endotracheal tube. The sterile endotracheal tube entering the caudal segment of the trachea through the surgical site was then removed, and the oral endotracheal tube was advanced past the resection site into the caudal segment. The preplaced 4-0 prolene appositional sutures were tightened and were tied off in order to obtain tracheal segment apposition. 4-0 polypropylene sutures were used in a simple interrupted pattern to complete the anastomosis. The dorsal tracheal membrane was friable and did not hold sutures well creating a small tracheal tear. A 1 × 2.5 cm segment of the sternohyoid muscle was harvested and placed over the dorsal aspect of the trachea to reconstruct this defect. This was sutured in place using simple interrupted 4-0 polydioxanone. The endotracheal tube was deflated, repositioned cranial to the tracheal incision, and reinflated. This was used to pressure check the trachea which pressure checked appropriately up to 20 cmH2O. A 12Ga × 20 cm single lumen radiopaque polyurethane thoracostomy tube with multiple fenestrations was placed on the right hemithorax and sutured in place. The surgical site was then routinely closed using 3-0 polydioxanone, 3-0 poliglecaprone, and skin staples. Liposomal bupivacaine was injected along the incisional line during closure. The thoracostomy tube was aspirated until subatmospheric pressure was obtained. Postoperative radiographs were taken and revealed mild tracheal narrowing at the location of the anastomosis ().\nThe cat was extubated and recovered routinely with pulse oximetry (SpO2) readings of 98%. Supplemental oxygen was provided for the first 2 hours after extubation as a precaution. In the oxygen cage, the patient was breathing easily with SpO2 monitoring remaining >98%. The patient was then weaned to room air with SpO2 monitoring remaining >98% overnight with a normal breathing pattern and effort. Postoperative analgesia consisted of a fentanyl CRI for 4hours, a fentanyl transdermal patch (12mcg/h) placed upon recovery, robenacoxib (2 mg/kg SQ), and gabapentin 10 mg/kg PO q8h. Isotonic crystalloids were continued at 1x maintenance (45 ml/kg/24 h) overnight. Cold compressing of the incision continued q4h during his hospital stay. To avoid anxiety-related respiratory complications, the cat was administered trazodone at 5 mg/kg PO q8h postoperatively. This caused excessive sedation, so trazodone was discontinued the following day, and gabapentin was decreased to 5 mg/kg PO q8h. Thoracostomy tube aspirations yielded minimal fluid (<0.25 ml/h) for over 24 h and was removed 24 hours after surgery. Robenacoxib was transitioned to the oral formulation (6 mg PO q24h) the day following surgery. Forty-eight hours after surgery, the cat was alert, eating, and comfortable and was breathing easily with minimal referred upper airway noise. The cat was discharged to the owner on the third day with 3 days of robenacoxib (6 mg PO q24h), 12 mcg fentanyl transdermal patch, 2 weeks of liquid gabapentin (5 mg/kg PO q8-12h), and 2 weeks of liquid amoxicillin/clavulanic acid (13.75 mg/kg PO q12h).\nHistopathological analysis of the submitted tissue revealed an incompletely excised squamous cell carcinoma originating from the tracheal mucosa with neoplastic cells noted at the cut edges cranially and caudally. The mass itself is composed of many irregular lobules of neoplastic squamous epithelial cells with central cystic areas within many lobules containing amorphous eosinophilic debris and neutrophils. The neoplastic tissue is noted to have 14 mitotic figures seen per 10 high power fields. This tissue is invading the lamina propria as well as into and through the tracheal cartilage. No lymphatic emboli were noted on the histopathology report. Histopathology of the local cervical lymph node showed no evidence of metastasis. These results were relayed to the owner, and further options including revisional surgery, radiation therapy, or chemotherapy were discussed.\nSeventeen days postoperatively the cat was presented for staple removal. The owners stated that the cat was acting normally at home, vocalizing normally and eating/drinking readily. Physical examination revealed a well-healed skin incision. The cat was eupneic with no tracheal sensitivity nor discomfort. No abnormal noise was noted on thoracic and tracheal auscultation. It was recommended to keep the cat's activity restricted for one more week and then reintroduce him back to his normal routine. A consultation with an oncologist was recommended with the option for a revisional surgery vs radiotherapy or chemotherapy being revisited. At the time of last phone recheck (120 days postoperatively), no further treatments have been initiated and a consultation appointment with the oncologist was canceled. The owners were contacted just prior to article submission, and the cat was acting normally at home with no clinical signs of recurrence. |
A 65-year-old man presented to the emergency department with fever, chills, nausea and abdominal pain. A week prior to this admission the patient had been worked up for hematuria, a computed tomography (CT) scan of the abdomen showing multiple rim-enhancing liver lesions. The differential diagnosis at that time included metastatic colon carcinoma, amebic abscess and bacterial abscess seeded from sigmoid diverticulitis. Five days prior to admission, the patient underwent colonoscopy with biopsies, which showed acute inflammation but no evidence of malignancy. On the day of admission, repeat CT scan showed interval increase in size of his rim-enhancing liver lesions (fig. ) as well as acute cecal diverticulitis, chronic pancreatitis and a large thrombus in the portal vein. Based on the interval increase in size of the liver lesions and the colonoscopy findings, an infectious etiology was favored and four drains were placed for presumed abscesses (confirmed with drainage of purulent fluid) and empiric treatment with ciprofloxacin and metronidazole was initiated. The patient was also started on heparin for his portal vein thrombosis 3 days after admission (transitioned to Lovenox twice daily 6 days after admission). On day four, the causative organism for the patient's abscesses remained unidentified and a liver biopsy was performed to re-evaluate for the possibility of neoplasia or an unusual organism.\nThe liver biopsy showed neutrophil-rich debris (fig. ), consistent with abscess, and liver parenchyma embedded in fibrous tissue and scattered mixed inflammation consistent with abscess wall; no peliosis or viropathic changes were appreciated. Immunohistochemical staining for CD34 and CD31 showed no evidence of a vascular neoplasm, and S100 was negative, arguing against a neural crest-derived tumor (not shown). No bacteria were identified on Gram stain (fig. ), and no fungal organisms were seen on periodic acid-Schiff diastase or Gomori methenamine-silver stains. Also, there were no mycobacterial organisms on an acid-fast bacilli-stained slide (not shown). Warthin-Starry (WS) stain, however, showed filamentous bacteria within the necrotic debris (fig. ) consistent with Fusobacterium infection []. A week after admission, detection of Fusobacterium sp. in cultures of the patient's hepatic abscess fluid confirmed the diagnosis of Fusobacterium liver abscess.\nIn the interval between the biopsy and identification of the causative organism, ciprofloxacin was empirically changed to ceftriaxone because of concern for Streptococcus milleri infection; the patient improved on the combination of ceftriaxone 1 g daily and metronidazole 500 mg three times daily, his drains were removed, and he was discharged 8 days after admission on antibiotics as described above. |
We report a 24-year-old Filipina woman who was recently diagnosed with hypertension and hypothyroidism. She presented to our hospital with generalized fatigue and weakness that had been going on for three months prior to presentation. She had increased abdominal girth along with acne and occasional difficulty while getting up from chairs or walking. On examination, she was found to be thinly built with a slightly round face containing numerous acne. A slight hump on her back was noted. She also had proximal bilateral muscle weakness, mainly in the lower limbs. Her blood pressure was found to be 163/67 mmHg. Laboratory tests revealed an 8 AM cortisol level to be high with high ACTH. A low-dose dexamethasone suppression test failed to suppress cortisol (Table ). At this point, pituitary magnetic resonance was performed, and it showed a pituitary microadenoma. Then, the high-dose dexamethasone suppression test showed high cortisol levels (Table ), which meant failure to suppress cortisol, suggesting the cause to be an ectopic source, rather than central.\nChest, abdomen, and pelvic CT scans were taken; they showed findings of a small thymic neuroendocrine tumor (Figure ). (Axial contrast-enhanced CT study at the level of midthoracic regions shows a small peripherally enhancing solid abnormality within the thymus (circle)) associated with bilateral adrenal hypertrophy.\nFigure . (Axial contrast-enhanced CT study at the level of the upper abdomen shows a smooth enlargement of both adrenal glands in keeping with hypertrophy (arrows)). This further confirmed the suspected diagnosis of Cushing's syndrome secondary to a thymic neuroendocrine tumor. She underwent surgical excision of the tumor by right video-assisted thoracoscopic surgery (VATS) with en bloc excision of the mediastinal mass and total removal of anterior mediastinal fat.\nPathological examination of the resected thymus revealed a well-circumscribed tumor sharply demarcated from the surrounding atrophic thymic tissue (Figure A). The tumor cells are arranged in a nested pattern. The cells are characterized as having round to oval nuclei, salt-and-pepper chromatin, and eosinophilic cytoplasm. The interstitium between the tumor cells is fine with small capillaries (Figure ). By immunohistochemistry, the tumor cells are positive with CKAE1/AE3 (paranuclear dot-like pattern), synaptophysin, and ACTH (Figure ), and negative with chromogranin, TTF-1, and PAX8. The Ki-67 proliferative index is about 1%. The features were consistent with typical carcinoid tumor (ACTH expressing) confined to the thymus. Following the surgery, her ACTH dropped to 6.1 pg/m and cortisol to 399 nmol/L, as shown in Table , along with improvement in her symptoms and blood pressure and titration down on her antihypertensives with no postoperative complications. |
A 65-year-old man was referred by the otolaryngology department to our outpatient clinic due to sudden swelling and mild pain around the right eye. On examination, the patient exhibited what appeared to be severe edema encompassing the upper and lower lids of the right eye (). Crepitus was clearly audible on palpation of the eyelids. An attempt to open the lids was unsuccessful. Visual acuity and intraocular pressure could not be measured due to extreme lid swelling. The patient reported that he had undergone transnasal endoscopic nasal polypectomy through the right nostril 2 days earlier. He said he had been instructed not to cough or strain after the endoscopic nasal surgery and the sudden swelling occurred immediately after severe coughing and straining. We suspected that the sinus wall was weakened due to his endoscopic surgery and the increased pressure caused by straining had forced air in the nose into the periorbital area. B-mode ultrasonography showed trapped air in the periorbital area ().\nConsidering the patient’s anxiety, the severity of periorbital emphysema, inability to conduct a full ophthalmologic examination, and the risk of complications such as compressive optic neuropathy, the patient was re-evaluated for a surgical intervention. After consultation, it was decided to evacuate the air using a 21 gauge needle inserted in the subcutaneous tissue of the upper and lower lids. In sterile conditions, the eye area was cleaned with 10% povidone-iodine. A 21-gauge needle was passed through the skin and subcutaneous tissue of the upper and lower lids parallel to the tarsus about 1.5 cm from the lid margin. Evacuation of subcutaneous air was evident from a significant reduction in lid swelling during the procedure (). The patient’s vital signs were stable and the procedure was concluded. He was discharged with systemic antibiotics (cefuroxime axetil 500 mg twice daily) and moxifloxacin drops four times daily.\nOn follow-up examination the next day, the periorbital emphysema was substantially reduced and the globe could be examined (, ). He had full visual acuity in both eyes; intraocular pressure was 17 mmHg in the right eye and 16 mmHg in the left eye. Dilated fundus examination was normal. No restriction in eye movements was observed. Follow-up examinations at 1 week and 1 month revealed no pathological findings. |
An 18-year-old male presented to our institution in 2014 with headaches and vomiting for two weeks. A magnetic resonance image (MRI) of his brain showed a complex extra-axial dumbbell-shaped lesion with the epicenter in the left Meckel’s cave, extending anteriorly to the cavernous sinus and the cerebellopontine (CP) angle posteriorly with mass effect over the brainstem. He underwent a left retromastoid craniectomy and decompression of left CP angle lesion elsewhere. The histopathology was suggestive of malignant melanoma, and he was referred to our institution for further management.\nHis general physical examination revealed a deep gray-blue nevus over the left upper eyelid, extending to the frontal and temporal region. He had dysarthria, left upper motor neuron facial nerve palsy, and right hemiparesis with Grade 4 power in his right upper and lower limbs. The biopsy of the nevus over his left eyelid was reported as superficial, and the deep dermal dendritic melanocytosis with histological features was suggestive of a blue nevus.\nThe MRI of his brain showed a large contrast-enhanced extra-axial mass with solid and cystic components measuring 4 cm x 2 cm x 3 cm in the left cavernous sinus extending through the Meckel’s cave into the posterior fossa (Figure ). A whole-body positron emission tomography-computed tomography (PET-CT) scan confirmed no extracranial disease. He underwent a left temporal craniotomy and zygomatic osteotomy, and we took an interdural middle cranial fossa approach for the radical excision of the tumor.\nPerioperatively, we noted the blue nevus on the left side of the forehead in the ophthalmic distribution of the trigeminal nerve. The pigmentation extended into the subcutaneous tissue and galea. The diploe of the temporal bone was also pigmented. The entire temporal dural convexity was pigmented completely black as were the dural root sleeves of the trigeminal nerve and the lateral and medial walls of the cavernous sinus. The tumor was localized in the cavernous sinus and had a well-defined capsule surrounding the divisions of the fifth cranial nerve. It extended into the posterior fossa through Meckel’s cave. The tumor was completely removed via the cavernous sinus through an interdural approach.\nThe surgical specimen revealed a tumor composed of sheets of moderately large polygonal cells with markedly pleomorphic nuclei with evidence of mitotic activity with foci of necrosis, and occasional cells with intracytoplasmic melanin (Figure ). We also saw small segments of nerve containing ganglion cells with perineural deposits of melanin. The tumor cells showed diffuse positivity for S100 protein and Melan A. Occasional cells were positive for human melanin black (HMB)-45. The ki67/mib-1 was 20% to 25%.\nGiven the coexistent skin lesion reported as a blue nevus, neurocutaneous melanoma was considered as the provisional diagnosis. The postoperative hyperacute MRI showed no residual tumor. His symptoms gradually resolved after surgery. He received postoperative intensity modulated radiotherapy to the tumor bed (4950 cGy in 22 fractions to the planning target volume with a biologically effective dose [BED] of 60.39 Gy and an equivalent dose in 2 Gy fractions [EQD2] of 50.32). He was closely monitored during the follow-up period.\nHe presented again about 20 months later with recurrent symptoms of headaches for two months and diplopia and vomiting for two days. On examination, sensations over the V1, V2, and V3 dermatomes were reduced on the left side. There was masseter and temporalis muscle wasting on the left side with impaired blinking in both eyes. His spino-motor system and higher mental functions were found to be normal.\nA lobulated heterogenous signal intensity mass lesion was seen in the left CP angle and Meckel’s cave. The mass is predominantly isointense and weighted T1, and T2 images were hypointense (Figure ). Various other similar intensity lesions were seen along the left tentorium suggestive of recurrent meningeal carcinomatosis.\nThe brain MRI with contrast showed a recurrent mass in the left Meckel’s cave extending into the CP angle compressing the brainstem. Another lesion of a similar nature was seen in the lateral aspect of the cerebellum. There was evidence of thickening and abnormal enhancement of the adjacent pachymeninges and leptomeninges suggesting disease recurrence with leptomeningeal spread. He was further evaluated with a CT of his thorax and abdomen which ruled out extracranial disease.\nHe underwent a left retromastoid suboccipital re-exploration and subtotal excision of the tumor. A postoperative CT scan of his brain showed no residual disease. However, the patient died five months after the second surgery. |
An 11-year-old boy presented with a swelling of the right side of his face for one month. The patient’s medical history was unremarkable. Physical examination showed a painless and circumscribed swelling of the right maxillary, but skin color was normal. Panoramic radiograph demonstrated a well-defined unilocular radiolucent lesion at the apices of the roots of the right maxillary from the lateral incisor to the first molar. Due to the expansive growth there was deviation of the roots of right maxillary lateralis, first and second premolar (), and of the roots of right mandibular canine and first premolar (). The clinical impression was benign odontogenic tumor. Based on these features, we considered a differential diagnosis of odontogenic cysts and tumors.\nWe took a preventive approach to preserve the developing canine. Therefore, we planned marsupialization of the lesion through the extracted socket of deciduous canine to create a window allowing continuous drainage of the tumor content. We treated the patient by marsupialization under local anesthesia, extracting the deciduous canine affected by the tumor. We made a pouch-like opening within the tumor. We then enlarged the extraction socket to form a bony window. Care was taken to avoid disturbing the developing tooth buds. During the marsupialization procedure, we took a biopsy sample to confirm the histopathologic diagnosis. Finally, we inserted a vaseline gauze into the cyst cavity to keep it open and to achieve hemostasis.\nAntibiotics were administered for seven days to prevent postoperative infection. After one week, we inserted an obturator using prosthetic putty. The putty was replaced every week. A space maintainer was used to maintain the spacing from the adjacent teeth. Panoramic radiographs were taken at three month intervals until the tumor-associated teeth erupted.\nAfter six months, there was further occlusal movement of the developing tooth with reduced radiolucency. The root formation of maxillary canine had continued, and bone formation was evident in the tumor ().\nFollow-up examination revealed occlusal movement of the developing tooth bud, but no apparent reduction in the radiolucency. So we performed enucleation and curettage, exempting the impacted canine, 12 months after marsupialization and decompression ().\nSix months after marsupialization, a multibracket appliance was placed in the maxillary arches, and to reinforce anchorage, a Nance appliance was placed. We created space in the canine location. A small orthodontic button, threaded with soft twisted ligature wire of 0.012-inch gauge, was bonded during the partial enucleation and curettage. The ligature was immediately engaged by the Nance appliance. We did follow-up activation every month thereafter. After 20 months of traction, the canine erupted and orthodontic treatment was finished after 37 months. Although we did orthodontic treatment only at the maxillary arch, Class I occlusion was achieved with an acceptable interincisal relationship. The root of the impacted canine showed normal morphology without root deformation (), but there was decalcification on the labial surface of canine. An electric pulp test was normal (25/60). Optimal intercuspation of teeth was achieved without recurrence (). We continued to observe the patient, and the tumor did not recur as of one year postoperatively ().\nMicroscopic examination revealed an extremely vascular encapsulated lesion showing multivariate patterns of cellular arrangements ranging from sheets of polygonal cells arranged in ductal patterns, rosettes to solid sheets of cells. In the center of these ducts, eosinophilic amyloid-like material was also seen. The solid lobular masses showed numerous spindle to columnar hyperchromatic cells with interspersed deposits of eosinophilic hyaline-like material. The above features were consistent with the diagnosis of AOT ().\nAt low magnification, the most striking pattern is that of various sizes of solid nodules of columnar or cuboidal epithelial cells forming nests or rosette-like structures with minimal stromal connective tissue (). |
The patient (Fig. ) was a 15-year-old Chinese girl. She was born as the younger one of twins at 38 weeks gestational age by planned caesarean section delivery, with a birth weight of 2.3 kg and length of 46 cm, and the 1,5 min Apgar scores were 10. There was no consanguinity between parents. Her elder identical twin sister was clinically hypothesized died of BS at the age of 6 months. Other family members had no histories of hereditary diseases. At 4 months old, she was transferred to a tertiary referral center as she presented with frequent vomiting, dehydration, hypokalemia and concomitant metabolic alkalosis. Plasma renin and aldosterone were markedly elevated, while blood pressure was within the normal range. She was clinically diagnosed with CBS. Oral Spironolactone, indomethacin and potassium supplements were started. During follow-up, despite the appropriate therapy and generally normalized serum electrolyte, the girl showed failure to thrive. At the age of 6 years, her height was 97 cm(<3rd percentile) and weight was 13 kg(<3rd percentile). There was no abnormality in renal ultrasonography and magnetic resonance imaging of pituitary gland. GH stimulation tests revealed GH deficiency, and recombinant human GH replacement therapy (0.1 IU/kg per day) was started (Table ). After 6 years of treatment, the annual increase in her length had reached 11 cm on average. Ostium secundum type ASD was diagnosed by echocardiography. Proteinuria was first indicated when she was 12 years old from the results of a urinalysis during the follow-up but had not been noticed.\nAt 14 years, serum creatinine and blood urea nitrogen levels were elevated and she was admitted to our hospital for further evaluation of renal function. On physical examination, her height was 155 cm, body weight was 45 kg, blood pressure was 120/74 mmHg, cardiac auscultation revealed a grade 3/6 systolic blowing murmur at the second and the third left intercostal space. Biochemical analyses showed normal serum pH (7.45) and normal levels of blood sodium, chloride, bicarbonate (HCO3−), calcium, phosphorus and magnesium. However, serum potassium was low (2.99 mmol/L, reference range: 3.5–5.3 mmol/L). The plasma renin activity and AngiotensinII were high both in decubitus (plasma renin activity 1.5 ng/ml and AngiotensinII 149.58 ng/ml; reference value 0.5–0.79 ng/ml and 28.2–52.3 ng/ml) and upright position (plasma renin activity 8.67 ng/ml and AngiotensinII 149.58 ng/ml; reference value 0.93–6.56 ng/ml and 55.3–115.3 ng/ml). She had moderate renal dysfunction [BUN 13.49 mmol/L; Cr 175 umol/L (19.79 mg/dl); 24-h creatinine clearance 43 ml/min per 1.73 m2 body surface area, indicating moderate CKD (Grade 3b) (2012 KDIGO guidelines)], severe proteinuria (urinary protein 8.861 g/day, serum total protein 54.2 g/L; reference value 65–85 g/L, serum albumin 30.9 g/L; reference value 40–55 g/L, urine β2-microglobulin 3.16 mg/L; reference value < 0.23 mg/L) and normal urine calcium excretion (0.11 mmol/L). Neither nephrocalcinosis nor nephrolithiasis was detected by renal ultrasonography. However, renal dynamic imaging (scintigraphy with 99mTc-DTPA) revealed glomerular filtration rate remarkably decreased [total glomerular filtration rate (GFR) about 49.7 mL/min per 1.73m2, left GFR about 26.9 mL/min, right GFR about 22.9 mL/min]. The transthoracic echocardiography revealed a 22-27 mm secundum atrial septal defect with left-to-right shunt. While the left ventricular ejection fraction (57%) and diastolic function were normal, the left ventricular volumes decreased (left ventricular end-diastolic volume:48 ml, left ventricular end-systolic volume:20 ml). Electrocardiogram was normal.\nAfter obtaining the informed consents from the patient and her parents, direct sequencing of known BS genes was performed. The sequencing procedure were performed by KingMed Diagnostics Test Laboratory (Shenyang, China) which provides the third-party inspection services. While the genetic studies for SLC12A1, KCNJ1, BSND, CASR and SLC12A3 were all negative, two novel compound mutations in CLCNKB were detected. The results showed one is a heterozygous mutation c.1696delG in exon 16 of CLCNKB, resulting in p. Glu566fs amino acid frameshift mutation. The one inherited from her mother. The other one is a heterozygous deletion of exon 2–3, which was confirmed by multiplex ligation-dependent probe amplification (MLPA) of CLCNKB (Fig. ). Neither of these two mutations have been described before or detected in 100 control samples (reference sequence: NM_000085.4). Because the predicted devastating effect on protein structure of the 2 alleles and the patients’ clinical features, we speculate these mutations are pathogenetic.\nBecause of the patient’s severe proteinuria, a percutaneous renal biopsy was performed and 17/26 of the results showed glomeruli revealed glomerulosclerosis, 8/26 of the glomeruli revealed FSGS which were located near the vascular pole, the other one was slightly enlarged with mildly increased mesangial cellularity. The microscopic examination of renal tissue showed hyperplasia of cells at the juxtaglomerular apparatus, focal tubular atrophy involving approximately 25% of the cortex, tubulointerstitial fibrosis with infiltration of inflammatory cells and a few foam cells were presented, vascular wall without obvious pathological changes. These findings are compatible with renal histology findings for BS. The immunofluorescence examination of 2/26 of the glomeruli demonstrated dominant granular staining for immunoglobulins (IgM +, IgA +/−) and complements (C3 +/−) in the mesangium and capillary wall. Staining for C1q was negative. Electron microscopy of one sclerotic glomeruli revealed glomerular basement membrane thickened, immune complex deposited in mesangial matrix, vacuolar degeneration of tubular epithelial cells, renal interstitial fibrosis and inflammatory cells infiltration appears (Fig. ). |
A 65-year-old man with a history of bilateral THAs for ankylosing spondylitis presented with right hip pain of a duration of 1 month. Bilateral hip arthroplasties (Trapezoidal-28 design, Zimmer, Warsaw, IN) had been performed 30 years earlier. The patient was pain-free and walking independently before onset of symptoms. There was no history of injury. The hip pain started insidiously and progressed with time. It was mechanical and radiated to the anterior aspect of the right thigh. He required regular analgesics for pain relief. Despite the pain, the patient could tolerate level-ground walking using one cane for 10 minutes. A radiograph showed fracture of the cemented femoral stem (Fig. ). A revision of the THA was planned.\nHis medical history was significant for ankylosing spondylitis and posttuberculosis tracheal stenosis. The long-standing ankylosing spondylitis led to spontaneous fusion of the spine from the cervical to the lumbar regions (Fig. ). The ligamentum flavum also was ossified (Fig. ). He had shortness of breath and wheezing on exertion for many years. CT showed stenosis of the distal trachea at the level of the aortic arch. The transverse tracheal diameter was only 3.4 mm and the length of the stenotic segment was 1.6 cm. There was another narrowing at the right main bronchus origin. The intraluminal diameter was only 2.6 mm and the stenotic segment measured 1.4 cm in length. Complete collapse of the right upper lobe with calcified granulomata also was seen, in keeping with posttuberculosis changes. As a result of the tracheal stenosis, lung function tests showed a severe obstructive pattern with a forced expiratory volume in 1 second to forced vital capacity (FEV1:FVC) ratio of only 44%. The FVC and FEV1 were 68% and 40% of predicted values, respectively. Previous attempts at bronchoscopic tracheal dilatation and stenting had failed because of a rigid cervical spine and failure to insert a rigid bronchoscope by cardiothoracic surgeons.\nAnesthetic assessment revealed a Mallampati Class IV airway [], very limited neck movement, and marginal mouth opening of only 2 cm. Owing to the difficult airway access, tracheal stenosis with the impossibility of intubation with even a small endotracheal tube, and poor lung function, we considered general anesthesia to be contraindicated. Conventional neuraxial anesthesia also was not possible because of the ossified ligamentum flavum and lack of interlaminar space for entry. After discussion with various teams including our consultant spine surgeon, we decided the best option was a minilaminotomy and insertion of an intrathecal catheter for neuraxial anesthesia.\nThe patient was informed of his situation and our recommendation: attempting conventional neuraxial anesthesia first and if that failed to proceed to minilaminotomy. The patient declined an initial attempt at neuraxial anesthesia owing to his concerns about a high chance of failure and complications such as breakage of spinal needles. He elected to proceed directly to the minilaminotomy.\nOn arrival in the operating room, routine monitoring was applied and equipment for difficult intubation was checked and on standby. Two intravenous lines and one arterial line were secured under local anesthesia. The patient was placed in the left lateral decubitus position. He was sedated using a loading dose of dexmedetomidine (1 μg/kg body weight over 10 minutes) followed by continuous infusion. A 11.5-mL dose of 2% lignocaine with 1:200,000 adrenaline was infiltrated locally. Then a posterior longitudinal incision was made at the L3/L4 level, which was judged by palpation. Subperiosteal stripping of the paraspinal muscle was performed on the right side to expose the lamina. A 5-mm hole was made in it using a high-speed burr. The underlying ligamentum flavum was completely ossified. After exposing the dura, an 18-gauge Tuohy needle was introduced in the subarachnoid space through the exposed dura. Free flow of cerebrospinal fluid was observed and an 18-gauge spinal catheter was inserted and secured. The tip of the catheter was at the L2 level. Two milliliters 0.5% plain bupivacaine and 25 μg fentanyl were injected through the catheter. Complete sensory block was achieved up to the T10 level bilaterally, which was adequate for the subsequent part of the surgery.\nWe used a posterior approach to the hip with an extended trochanteric osteotomy. The loosened femoral component was revised successfully to a cementless porous-coated bow stem (Solution®; DePuy, Warsaw, IN). The acetabular component also was revised to a cementless cup (Duraloc®; DePuy, Leeds, UK). Vital signs including pulse rate, blood pressure, and oxygen saturation were stable throughout the surgery, which lasted for 3 hours. At the end of the operation, the spinal catheter was removed after injecting an additional 1 mL 0.5% plain bupivacaine to prolong the block and provide adequate postoperative analgesia. We did not seal the hole in the dura. The patient did not complain of headache and there was no sign of cerebrospinal fluid leakage during the postoperative period. The patient was given an intravenous self-controlled analgesic device with morphine for pain control after the sensory block wore off. Power and sensation of his lower limbs were completely normal 4 hours after the operation.\nThe patient made an uneventful recovery. He was allowed to bear weight as tolerated and was discharged home 3 weeks after the operation. At last followup at 3 years, the patient was pain-free and walked with one cane. Radiographs showed well-fixed implants (Fig. ). |
A 46-year-old super morbidly obese male (BMI: 72) in renal failure requiring hemodialysis was admitted to the intensive care unit (ICU) following herniorrhaphy for an acute ventral hernia and incarcerated transverse colon. On post-operative day #2, CVC placement into his left subclavian vein with an 18-gauge access needle of 6.35cm length was attempted at the bedside in the intensive care unit. Due to the patient’s excessive body mass it was necessary to use the entire length of the needle.\nFollowing successful venous puncture with minimally applied pressure, the 18-gauge access needle fractured and separated from the hub device. At this time it was unknown if the foreign body was in the vascular space or in the left chest wall.\nInitial attempt at retrieval took place at the bedside. The incision site was immediately extended in an attempt to track and retrieve the needle, which was palpated running parallel to the clavicle. Given the proximity to the great vessels and poor visualization, bedside intervention was stopped.\nA portable AP chest x-ray taken just after needle fracture showed a curvilinear density inferior to the left clavicle (Figure ).\nOn post-operative day #3 the patient was taken to the operating room for needle retrieval under fluoroscopic guidance. The needle was visualized medial and deep to the previous chest wall incision. Muscles of the chest wall were divided and mobilized to expose the clavicle. Repeat fluoroscopy revealed the needle to be deep and medial to the costoclavicular ligament, approaching the chest (Figure ).\nTo approximate the needle, the costoclavicular ligament was divided, and the subclavian vein was exposed. The original puncture site was encountered in the middle segment of the subclavian vein. Using this reference point, oblique images on the c-arm were then taken, and the retained needle was seen deep to the clavicle, still in the subclavian vein.\nAn endovascular approach was then used under fluoroscopy to retrieve the needle through the thoracoacromial vein. Ultimately, a snare device engaged the needle. The needle and snare were controlled with a sheath, and the needle was retracted successfully in one unit through the venous thoracoacromial branch (Figure ). |
A 40-year-old gravida five, para four woman presented for workup and management of abnormal uterine bleeding. Her past medical history was significant only for hypertension and anemia. On review of her social history, she admitted to drinking six packs of beer on the weekends but denied further substance use. She denied previous treatments for her bleeding including any previous intrauterine device usage.\nUltrasonography revealed a 7 cm fundal fibroid with otherwise normal pelvic anatomy. She was initially offered medical management of her bleeding. She declined any medical treatment and strongly desired definitive surgical treatment. She then underwent a total vaginal hysterectomy with adnexal conservation. Due to the large size of the uterus, a myomectomy was performed to facilitate vaginal removal. Her postoperative hospital course was relatively uncomplicated and she was discharged home on postoperative day three.\nOn postoperative day ten, she presented to the Emergency Department (ED) for fever, worsening abdominal pain, and new onset of nausea and vomiting. In the Emergency Department, she was tachycardic and tachypneic but afebrile. Her exam was significant for abdominal tenderness to minimal palpation, vaginal cuff erythema, and significant tenderness to palpation of the vaginal cuff. Lab work showed an elevated white blood cell count. She was admitted for management of presumed pelvic infection.\nA CT of the abdomen and pelvis was obtained and showed a 6.2 x 9.7 cm pelvic abscess adjacent to the vaginal cuff (Figures and ).\nInterventional Radiology placed a drain into the abscess and the patient was started on IV piperacillin/tazobactam. She was transitioned to oral amoxicillin/clavulanate potassium after four days on intravenous antibiotics and her drain was removed on hospital day 5. Vaginal wound cultures remained pending; however, due to continued clinical improvement on the oral antibiotic regimen, she was discharged home on hospital day 5 with a two-week course of amoxicillin/clavulanate potassium.\nThe patient then returned for her outpatient visit approximately one week later. The results of the vaginal wound cultures revealed a large growth of Actinomyces meyeri. The patient's case was discussed with an Infectious Disease (ID) specialist who recommended an additional two-week course of amoxicillin/clavulanate potassium.\nThe patient then returned to the ED on postoperative day 25 for pleuritic chest pain with mild cough but denied gynecologic complaints. She reported compliance with the oral amoxicillin/clavulanate potassium regimen. Exam and lab work were unremarkable. A chest X-ray showed left basilar heterogeneous opacities, likely subsegmental atelectasis. A CT angiogram was obtained due to concern for a possible pulmonary embolism (PE). The imaging was negative for a PE; however, it was concerning for possible pneumonia. The patient was discharged home with a five-day course of levofloxacin for treatment of pneumonia.\nOn postoperative day 27, the patient represented to the ED with worsening shortness of air and chest pain. Again, she reported compliance with her antibiotic prescriptions. Exam and lab work were again unremarkable. A repeat chest X-ray showed a slight progression of right basilar heterogeneous opacities thought to be infectious. Her antibiotic regimen was again discussed with ID specialists and an intravenous antibiotic regimen was felt preferable to an oral antibiotic course. She then completed an outpatient two-week course of IV ampicillin/sulbactam as recommended.\nOn postoperative day 37, a repeat CT of the abdomen and pelvis showed near complete resolution of the previous pelvic abscess. HIV testing was obtained and returned negative result. She reported significant improvement of her symptoms. She was placed on a six-month course of oral amoxicillin per ID recommendations with plans for continued follow-up in their clinic, as well as with gynecology. She has not shown any signs of recurrent infection after approximately 1 year of follow-up. |
A 50-year-old female patient who had a mass in the lacrimal sac area for more than 3 months is presented in this report. The mass exhibited slow but progressive swelling. She experienced occasional epiphora and pain, without blood discharge or impaired vision. After 6 months, the patient underwent dacryocystectomy for the left lacrimal sac mass. Postoperative pathological examination confirmed the presence of malignant melanoma. Three months after the operation, a lump in the lacrimal sac area had gradually increased. Local recurrence was suspected and she was referred to ophthalmology department for further treatment.\nA physical examination revealed a scar from the previous operation. In addition, a 1.5 cm × 1.5 cm firm, localized and relatively clearly defined mass was observed in the left medial canthus. There was no cervical lymphadenopathy. A CT scan demonstrated a partially well-defined soft mass in the fossa of left lacrimal sac extending into the nasolacrimal duct and anterior ethmoid sinus (Fig. a). CT plain scan revealed that the lesion had a CT value of 49HU. The CT value of the tumor was similar to that of the extraocular muscle and wall of eyeball. Enhanced CT scan detected a CT value of 103HU for the arterial phase and 95HU for the venous phase (Fig. b). There was no eyeball involvement. Bone windows showed that the left nasolacrimal duct was enlarged and partial bone destruction of the nasolacrimal duct was confirmed. There was no regional or distant metastasis.\nMRI plain scan demonstrated a partly well-demarcated mass of 1.2 cm × 1.3 cm × 2.3 cm in the lacrimal sac lesion. The tumors had infiltrated the septa orbital and subcutaneous adipose tissue. MRI revealed intermediate signal intensity on T1 and iso-or hyper-signal on T2 weighted images (Fig. c, d). The lesions displayed slight hyperintense signal on DWI. A dynamically enhanced MRI scan showed intense enhancement of the lesion margin and inhomogeneous enhancement of the lesion center (Fig. e).\nA biopsy was obtained from the patient before the operation. Histopathological examination on the biopsy confirmed fusiform malignant tumoral cells with hyperchromatic nuclei. The tumor had high number of pigmented cells, inhomogeneous pigment granules and massive hemorrhage (Fig. f, g). Immunoreactivity to the S-100 protein, Melan-A, HMB-45, Nestin, and CD56 was positive (Fig. h, i). Therefore, a diagnosis of malignant melanoma was confirmed. Further examination of the whole body, including computed tomography scanning and B ultrasound revealed that there was no metastasis in the head and neck, chest, abdomen, and pelvis regions. Thus, total excision of tumor was performed. The patient refused to undergo radiotherapy and chemotherapy. |
A 21-year-old female presented with complaints of progressive NYHA Class III breathlessness with episodes of paroxysmal dyspnoea and intermittent claudication Rutherford Class II for 2 months. The referring physician assumed the presence of the cardiovascular disease, as all of her four limbs had absent peripheral pulses. On examination, she was thin built and undernourished with muscle wasting of the four limbs. We could not record accurate blood pressure. Heaving apex was discovered by cardiac examination, 2 cm outside the midclavicular line, and laterally displaced. Grade II mid-diastolic murmur with regular heart rhythms could be heard on auscultation.\nLaboratory parameters were normal except for elevated acute phase reactants. Electrocardiography demonstrated features of left ventricular hypertrophy with strain pattern like poor ‘R’ wave progression. Computed tomography (CT) angiography of aorta revealed the complete occlusion of the infrarenal abdominal aorta and bilateral iliac arteries. It also demonstrated the occlusion of bilateral subclavian arteries with good collateralization [Figure –]. Complete occlusion of the right renal artery and 80% stenosis of the left renal artery were also delineated. Echocardiography showed dilated left ventricle, global hypokinesia, concentric left ventricular hypertrophy, and moderate mitral regurgitation, with small apical clot [].\nThese features were suggestive of hypertensive heart disease. Based on the results of clinical examination and investigations, a diagnosis of TA was made.\nMedical therapy in the form of newer oral anticoagulant, aspirin, diuretic, spironolactone, and empirical antihypertensives was started. Rheumatologist prescribed her steroids, once a weekly dose of methotrexate and mycophenolate mofetil. Her symptoms improved to NYHA class II over 2 weeks and could perform her routine activities.\nAfter 9 months of immunosuppressive treatment, the intervention was planned for the revascularization of the lower limb vessels to alleviate their symptoms after acute phase reactants had plummeted to normal levels. The vascular surgeon recommended against surgical revascularization because the extensive dissection and high risk of graft restenosis would have been involved. The patients and their families chose less invasive solutions against the possibility of repeat revascularization.\nThe percutaneous intervention was planned along with interventional radiologists. Under ultrasound guidance, both groins were punctured using a micro-needle to reduce the risk of injury to the arterial wall. Digital subtraction angiography (DSA) has demonstrated the occlusion of bilateral iliac arteries. The left iliac artery had a distal end tapered, while the right iliac artery had a flush occlusion after the origin of the internal iliac artery [Figure and ].\nBoth iliac arteries initially crossed with Gaia 2 guidewire that entered into the false lumen [Figure and ], then PROGREAT microcatheter and hydrophilic guidewire (0.018̎) system used to re-enter into the true lumen. True lumen entry was confirmed by injection in the abdominal aorta and the visualization of the inferior mesenteric artery branch [Figure and ]. Predilatation of stenotic segments was done using a 4 × 40 mm Mustang 0.035̎ balloon dilatation catheter system [Boston Scientific Corporation, MA, USA] at 6-8 atm. Angiography of abdominal aorta revealed long-segment stenosis of the infrarenal aorta with osteal occlusion of the right renal artery and left renal artery stenosis []. Bilateral kissing balloon angioplasty of the distal segment of the abdominal aorta and the proximal iliac arteries was performed using two 7 × 60 mm Mustang non-compliant balloons [Boston Scientific Corporation, MA, USA] at 8 atm []. 14 × 60 mm Wallstent [Boston Scientific Corporation, MA, USA] was deployed into the abdominal aorta across the renal and other mesenteric arteries to end just before the aortic bifurcation []. Bilateral 10 × 80 and 10 × 100 mm LIFESTAR self-expanding Nitinol vascular stents [Bard Peripheral vascular, AZ, USA] for both iliac arteries were deployed simultaneously [].\nFinal angiography demonstrated brisk flow into the aorta and bilateral iliac arteries. Both peripheral pulses are palpable [Figure and ]. True intraarterial blood pressure was 200/120 mm Hg and anti-hypertensive medication doses were escalated. Dual antiplatelet therapy in the form of aspirin and clopidogrel required for at least 12 months but can be extended up to 36 months to prevent restenosis as per the recommendations. She has completed a 1-year follow-up and now in NYHA class I symptoms. In the first year of follow-up, ultrasonography and Doppler study of iliofemoral vessels were performed every 3 months and recommended every 6 months after 1 year to detect early signs of restenosis. |
A 53-year-old male pedestrian was hit by a car 20 minutes before arriving at the hospital. Upon the arrival of the emergency medical services, the patient was drowsy but following commands. While being taken to the hospital, the patient became hypotensive. He was brought by ambulance to our trauma center under ongoing resuscitative measures. Anywise, no intravenous access was established yet. After the primary survey following the ATLS® protocol, there were distended abdomen and left fibular fractured. The extended Focused Assessment Sonography in Trauma (eFAST) revealed free fluid in the peritoneal cavity. His pulse rate was 142 beats per minute, and his blood pressure was 66/43 millimeters of mercury by the upper arm cuff at ER.\nThe patient was intubated, and a massive transfusion protocol was activated immediately. The right common femoral artery was cut down, and insert a 12-French gauge introducer vascular sheath (). The conventional (over the wire) aortic balloon was inserted via introducer vascular sheath over the guidewire (). The length of the balloon was measured by the distance between the right groin and mid sternum (). After inflation of the aortic balloon, blood pressure was raised to 118/78 millimeters of mercury. Confirmation of the aortic balloon was made by the physiologic response of the patient with there is no pulse palpated in both lower extremities and the blood pressure in upper extremities is raised. We used complete aortic occlusion technique in this patient.\nThe patient was sent to the operating room for immediate laparotomy. There was a sizeable mesenteric avulsion and 10 centimeters of jejunal contusion, causing 2500 millimeters of hemoperitoneum (). Segmental small bowel resection with end-to-end anastomosis of jejunum and suture to stop bleeding from mesenteric vessels were performed. At the same time, the patient was resurrected with medication and blood components by the anesthesiologists. The intraabdominal procedures took 55 minutes, and then we tried to deflate the balloon. However, when we deflated the balloon, the blood pressure dropped immediately. So, we inflated the balloon for the anesthesiologist to resuscitated the patient. After that, we deflated the balloon again. The total aortic occlusion time took 90 minutes. The patient’s blood pressure was approximately 120/75 millimeters of mercury by arterial-line after the operation.\nAfter the hemodynamic was stable, the right common femoral artery was repaired, and the wound was sutured. Both dorsalis pedis pulses were palpated.\nThe total operative time was 140 minutes. The patient received ten units PRBCs, eight units of fresh-frozen plasma, and six units of platelets.\nAll of the procedures were performed by a trauma surgeon trained in vascular procedures on duty that day and covered by the vascular surgical staff.\nThe patient did well and was discharged on hospital day 5. He required rehabilitation and ambulation training. The telephone followed up to track symptoms and found that the patient did not have any eating problems. |
A 43-year-old female with a past medical history of severe allergy-induced asthma and chronic nasal drip presented to the clinic with worsening cough and chest tightness for 10 days and was treated for bronchitis with steroids and antibiotics. She then presented to the emergency department with severe and acutely worsening shortness of breath, sudden onset pleuritic chest pain, and sharp radiating pain between both shoulder blades with deep inspiration. At time of presentation, the patient was afebrile with vital signs within the normal limits. Lab work was insignificant with negative leukocyte count and negative cardiac enzymes. A chest X-ray demonstrated an abnormal right cardiomediastinal silhouette with large opacity over the right mediastinum adjacent to the right atrial border. A follow-up CT scan revealed a large right-sided mass adjacent to the right atrium and extending into the right chest measuring 5.1 cm × 9 cm × 4.3 cm (). Her last imaging study was a fluoroscopy study 10 years ago that showed no indications for a mediastinal mass. An echocardiogram revealed a normal ejection fraction (55–59%), no wall motion abnormalities, and a cyst near the right atrium. The patient had continued pleuritic chest pain and difficulty breathing and the decision was made to perform video-assisted thoracoscopic surgery (VATS) for pericardial cyst removal. The patient underwent general anesthesia with a 37 French left-sided double-lumen tube. Standard ASA monitors were applied. Two large-bore peripheral IVs and an arterial line were placed for continuous blood pressure monitoring. Intraoperatively, a large cystic lesion was adherent to the pericardium (). There was no solid component and no obvious communication with the pericardium. Part of the cyst wall was left on the phrenic nerve to preserve it. The patient tolerated the procedure well, had no postoperative complications, and was discharged home on postoperative day number two. The final pathology report revealed benign, acute inflammatory pericardial cyst. |
Patient is a 73 year-old right hand dominant female who initially presented to the office complaining of atraumatic right shoulder pain with activity and limited range of motion of longstanding duration. On physical exam, she was found to have significantly limited active range of motion of the right shoulder and clinical signs of impingement. Radiographs at that time demonstrated superior escape of the humeral head with impingement of the greater tuberosity on the acromion and early acetabularization of the acromion (Fig. ). MRI findings were consistent with her x-ray and also demonstrated a lack of contiguous supraspinatus or infraspinatus tendon. At this juncture, the patient was diagnosed with rotator cuff arthropathy and elected to proceed with reverse total shoulder arthroplasty.\nThe patient was brought to the operating room and placed in the beach chair position. An incision was made from just lateral to the coracoid to the medial border of the proximal humeral shaft in line with the axillary fold. Subcutaneous tissue was dissected and the cephalic vein was identified. As the cephalic vein was mobilized and the clavipectoral fascia was incised, a discrete, branching, fascicular nerve was identified lateral and deep to the cephalic vein within the deltopectoral groove (Fig. ). The nerve was further dissected and traced both proximally and distally. Distally, the nerve and all branches were found to be diving into the anterior deltoid muscle. Proximally, it was found to run deep to the conjoined tendon, towards the brachial plexus. The nerve was freed from the deltoid muscle belly, allowing enough excursion to access the glenohumeral joint via a small deltoid window. The remainder of the operation concluded without complication and the wound was closed primarily (Fig. ). The patient was neurovascularly intact post-operatively with intact sensation in the axillary nerve distribution and able to fire her deltoid muscle. She healed without complications. At 4-month follow-up, she was doing well and able to actively abduct and forward flex her right shoulder to approximately 120 degrees (Fig. ). |
A 12-year-old boy presented with a history of hematemesis and melena from 10 days. The child had blunt abdominal trauma due to road traffic accident 1 month earlier for which he was operated at an outside hospital for hemoperitoneum and at laparatomy a large laceration of right lobe of liver was seen. Hemostasis was achieved by packing, which was confirmed on re-look laparotomy on the 2nd day, and a drain was kept in the perihepatic space; no attempt for primary repair of liver laceration was done. The child stabilized hemodynamically and was discharged 10 days after surgery; his parents were advised to monitor the color and volume of the drained fluid. After surgery, although asymptomatic, he continued to drain some 100–200 mL of blood containing fluid from the drain daily and then developed GI bleeding.\nOn examination at presentation with GI bleed, the child was pale but afebrile; scleral conjunctiva showed icterus. His pulse was 108 beats/min and blood pressure on admission was 90/50 mmHg. Liver and spleen were not palpable. Hemoglobin was 6 g/dL, alkaline phosphatase was 400 IU/L, gamma-glutamyltransferase was 180 IU/L, serum bilirubin was 4 mg/dL, and aminotransferases were slightly elevated. Platelet count, prothrombin time, partial thromboplastin time, electrolytes, blood urea nitrogen, creatinine, and amylase were all normal.\nThe child continued with vomiting of blood and passage of black tarry stools. Upper GI endoscopy was performed after blood transfusion and showed bulky clots in the stomach and fresh blood in the duodenum; no varices or mass lesion were seen in the esophagus or stomach. The major duodenal papilla was not examined satisfactorily because of the presence of a lot of blood. Abdominal ultrasonography (US) revealed a septate fluid collection around the right lobe of the liver; intrahepatic and extrahepatic bile ducts were slightly dilated, no calculus or worm or debris was seen in the ducts.\nContrast-enhanced CT (CECT) study of abdomen was performed with a 64-slice multidetector-row-CT (MDCT), which revealed a defect in the right lobe of the liver with adjacent perihepatic fluid collection with drain tip more anteriorly; enhancing lesion was seen anterior to the right branch of the portal vein []. CT angiography reconstructed as three-dimensional (3D) volume-rendered and thick multiplanar reformation (MPR) images from the arterial phase of CECT revealed the diagnosis [Figures and ]. An irregular shaped structure showing same enhancement as adjacent celiac artery and aorta was seen deep to the old laceration in the right lobe, arising from the right branch of the hepatic artery suggestive of pseudoaneurysm [Figures and ]. Celiotomy was planned for the treatment as embolization was not available. Surgical resection of pseudoaneurysm was done. The patient tolerated the surgery well and made an uneventful recovery; his liver function tests gradually returned to normal. Hematemesis and melena subsided after surgery. He was discharged on the 10th postoperative day and was seen doing well in the follow-up 3 months after operation. |
The patient is a 50-year-old African American female with a history of bilateral breast reduction twelve years ago, iron deficiency anemia, and obesity, who presented to the surgeon's office complaining of tenderness of her right breast. The patient reported that recently she had been developing keloids along the scar of the right breast with some areas having a blue hue; her left breast was unremarkable. She noticed that after wearing a sports bra there was increased pressure and abrasions to the keloid, leading to cellulitis and edema. She was previously treated with two courses of antibiotics for what was presumed to be an infected keloidal scar of her right breast but with minimal improvement. On exam, she had a large 10 cm diameter keloidal region on the inferior and lateral aspect of the right breast with edema and cellulitis. The keloidal area had no palpable fluctuance; she exhibited no nipple discharge or palpable adenopathy of the right axilla ().\nThe patient had a benign-appearing mammogram 8 months prior, and all of her screening mammograms since her breast reduction have been without signs of malignancy. Another mammogram was ordered but was not performed due to patient discomfort. An ultrasound of the breast was preformed and suggested marked edema and skin thickening suggestive of infection but no definitive fluid collection or underlying suspicious mass was observed.\nThe patient underwent a right breast partial mastectomy for cosmesis and resection of the infected keloidal area. Intraoperatively, the mass was highly vascular, firm, but not fixed to the chest wall. Postoperatively, the pathology revealed a high-grade primary angiosarcoma of the breast with negative margins.\nPatient underwent a computed tomography of the chest, abdomen, and pelvis, which did not show any evidence of gross metastatic disease. The patient then underwent completion mastectomy and scheduled for adjuvant chemotherapy with combination gemcitabine and Taxotere, followed by radiation. |
A 23-year-old man presented in 2011 with increasing abdominal pain. In October 2011, he developed obstructive symptoms and underwent exploratory laparotomy. He was found to have a poorly differentiated adenocarcinoma of the transverse colon with multiple peritoneal implants. It was consistent with a pT4 pN2 (4/28) pM1 adenocarcinoma, K-RAS wild type. From January to July 2012, he received 12 cycles of FOLFIRI plus bevacizumab at another institution and had complete clinical response. Therefore, it was decided to stop his chemotherapy completely. In September 2013, he again developed abdominal pain. A PET-CT scan revealed FDG-uptaking lesions in the peritoneum as well as in the colonic anastomosis. From June 2014 to June 2015, he received 24 cycles of FOLFIRI and cetuximab with initial response followed by stable disease. In September 2015, he was first seen at our institution, and during a multidisciplinary meeting, it was decided to pursue CRS with HIPEC (oxali-platin 300 mg/m2), which occurred on October 5, 2015. He stayed at the ICU for only 72 h, and had the first bowel movement 6 days after surgery. On the 7th postoperative day, he developed fever with no identified origin, and was started on piperacillin-tazobactam and vancomycin. At this time, the patient was also complaining of left scrotal pain and swelling. An ultrasound of the testis was performed on October 17th and showed normal testicles and increased volume of the left epididymis with heterogeneous echogenicity. In addition, analysis of the epididymal waveform revealed a low-resistance pattern suggestive of acute epididymitis (fig. ). Although all cultures remained negative, it was decided to switch antibiotics to amikacin for 10 days and doxycycline for 14 days and treat as infectious epididymitis. The patient was discharged home on October 22, 2015, to complete antibiotics at the outpatient unit. As of November 23, the patient was still facing left scrotal discomfort, although less in intensity. |
Our patient is a 54-year-old female with a past medical history of hypothyroidism and very severe obesity (BMI 48 kg/m2). She underwent laparoscopic gastric sleeve surgery in the year 2012. Results were non-satisfactory in terms of weight loss with a difference of 6 kg/m2 in BMI post-procedure. So after six years, she underwent a laparoscopic biliopancreatic diversion with a duodenal switch. She had an uneventful postoperative recovery period. An upper gastrointestinal (GI) study contrast post-procedure did not reveal any evidence of obstruction or leak. The patient was discharged home two days after the procedure. A few days later, she started experiencing three episodes of nausea with brown-colored vomitus. She was found to be septic, with a heart rate of 110 beats per minute and temperature of 100.2oF. Her white blood cells count was 12/mm3.The source of infection was presumed to be intraabdominal considering her symptoms. Computed tomography (CT) of the abdomen and pelvis showed mildly dilated proximal small bowel loops. The patient was started on empiric antibiotic therapy with ceftriaxone 1 gm intravenous (IV) daily and metronidazole 500 mg IV every eight hours. Symptoms did not improve, so she was taken back to the operating room for diagnostic laparoscopy. Partial small bowel obstruction was noted along with ischemia of a segment of the ileum that was part of the duodenoileostomy due to mesenteric dissection. She underwent an open revision of the small bowel anastomosis with resection and anastomosis for the obstruction revision of the duodenoileostomy. Her hospital stay post-surgery remained uneventful. Diet was advanced gradually throughout the hospital course and a week later, the patient was discharged home with outpatient follow-up. Three weeks after that procedure, she noticed a productive cough with thick, yellow, foul-smelling phlegm and shortness of breath. She saw her primary care physician. A chest X-ray performed showed a right lung infiltrate with a right-sided pleural effusion. She was started on treatment with augmentin 500 mg/125 mg every eight hours. Her symptoms became worse so she came to the emergency room. Her vitals showed blood pressure 129/79 mmHg, heart rate 86 beats per minute, respiratory rate 20 breaths per minute, and temperature 98.6oF. Pulse oxygen saturation was 97% on room air. Mild leukocytosis was evident (white blood cells count 11.4/mm3 with no bands or left shift). A chest CT showed loculated, right-sided hydropneumothorax with almost total collapse of the right lung (Figure ).\nThere was a fistulous connection evident, extending from the surgical anastomosis in the stomach/bowel in the right upper quadrant through the right hemidiaphragm to the right hemithorax. These CT scan findings were new as compared to a CT scan obtained for this patient six months prior to the duodenal switch when she presented to the emergency department for non-specific left-sided chest pain. To analyze the anatomy of the fistula further, an upper gastrointestinal fluoroscopic contrast study was performed that showed a large fistula from the distal stomach prior to the duodenal bulb opening to the right pleural cavity (Figure ).\nConsultations from gastroenterology and cardiothoracic surgery teams were obtained. Chest tube drains were placed with the plan of eventually performing a video-assisted thoracoscopic surgical decortication. Post-procedure CT showed patent chest tubes draining the right pleural cavity. The drained fluid was exudative in nature as per Light’s criteria (fluid lactate dehydrogenase > 12,000 u/L and total protein ratio = 0.7) and culture from the right lung empyema grew Escherichia coli, Klebsiella pneumoniae, Pseudomonas aeruginosa, and Candida albicans. An infectious disease consultation was placed at this time. The patient was started on levofloxacin 750 mg IV daily for two weeks as per the sensitivity result obtained for Escherichia coli, Klebsiella pneumoniae, and Pseudomonas aeruginosa. Micafungin 100 mg IV daily was started for the infection with Candida albicans. This was later switched to Diflucan 400 mg IV daily for a total of two weeks. Repeat cultures from the draining fluid were negative toward the end of the second week.\nFor treatment of the fistula, the patient was transferred to another facility for esophagogastroduodenoscopy (EGD) and possible clipping of the fistula due to the unavailability of that particular service in our hospital. As a result, there was a delay of 16 days from admission to the treatment of the fistula. When the EGD was performed, it showed that there was no anastomotic leak from the previous surgery. No evidence of any stricture was identified at the previous anastomosis. A small fistulous tract was noted in the distal part of the antrum likely secondary to ulcer formation that was noted in very close proximation to the fistula tract. An endoscopic clipping was performed. A post-procedure contrast study performed on the same day revealed complete closure of the fistulous opening. The patient was transferred back to our facility after the procedure. An upper GI contrast study performed two days later showed residual leakage from the distal stomach to the right upper quadrant. A repeat EGD was performed along with fulguration of a fistulous opening with argon beam coagulation and repeat orthoscopic clip application with complete obliteration of the fistula tract. This was confirmed by an upper gastrointestinal contrast study showing no persistent fistulous communication between the post-bulbar duodenum and pleural space (Figure ).\nAfter confirmation of fistula obliteration, a video-assisted thoracoscopic surgery was performed followed by the washout of the right pleural space with the placement of a right-sided chest tube. The patient was discharged home after the procedure and has been followed by multidisciplinary teams on an outpatient basis. |
A 48-yr-old man presented with left chest pain, abdominal pain, and dyspnea of three days duration. Five days before admission, he had been involved in a minor motorcycle accident, but was asymptomatic for two days. The patient also had a history of chronic alcoholism and uncontrolled diabetes mellitus. He was a nonsmoker.\nOn admission, he was acutely ill-looking. However, his vital signs were stable; heart rate 70/min, respiration 37/min, blood pressure 110/80 mmHg, and body temperature 36.8℃. A physical examination also revealed no remarkable finding with normal bowel sounds and a soft, flat abdomen with no general or rebound tenderness. Laboratory tests revealed; WBC 3,200/µL, C-reactive protein 31.68 mg/dL, and serum glucose 201 mg/dL, and chest radiography on admission showed mediastinal widening and bilateral pleural effusion (). The patient underwent endoscopy on the admission day to exclude esophageal rupture, and diffuse thickening of mucosal folds with decreased distensibility and an 1 cm sized mucosal ulcer in upper thoracic esophagus were observed with scattered patches of hemorrhage in the gastric mucosa of the body and antrum.\nOn the evening of first hospital day, the patient was became febrile with a body temperature of 39℃, and thus, empirical treatment with broad spectrum antibiotics was immediately started under the suspicion of empyema or secondary infection. On the second hospital day, the dyspnea worsened and the amount of left pleural effusion increased on chest radiography. Left closed thoracostomy was performed with pus drainage. On the forth hospital day, a contrast-enhanced chest CT scan was performed and showed diffuse and marked circumferential wall thickening of the entire thoracic esophagus, extending to gastric cardia and associated with diffuse intramural low density and a peripheral enhancing rim (). In addition, multiloculated bilateral pleural effusion and mild pleural thickening were evident. A CT diagnosis of acute phlegmonous esophagogastritis was suggested and bilateral open thoracotomies were performed immediately. Pleural fluid analysis revealed exudates and a surgery was decided due to worsening of clinical condition of the patient and radiologic findings.\nDuring surgery, bilateral multiloculated pleural effusions were evacuated through open thoracotomies and the esophagus was freed from adjacent tissue. The adventitial and muscular layers of the esophagus were intact and no perforation was identified. Several separate esophageal myotomies were performed and the submucosal layer was found to have been filled with thick, cheesy materials, which were removed from the mucosa by scraping. Blood and sputum cultures of the patient and a microbiologic examination of pleural fluid demonstrated Klebsiella pneumoniae.\nA follow-up chest CT scan performed on the 49th postoperative day showed reduced diffuse esophageal wall thickening and bilateral pleural effusion (). The patient was successfully discharged on the 73rd postoperative day. |
A 60-year-old man with a history of metastatic prostate cancer (treated with docetaxel ~2 weeks prior to presentation), hypertension, hyperlipidemia, paroxysmal atrial fibrillation (on rivaroxaban) and right knee osteoarthritis (status post total knee replacement) presented to the emergency room with left sided neck pain and swelling. He first noted left sided neck pain 1 to 2 weeks prior to presentation. Initially, he noted some discomfort and a small nodule on his left neck. However, the nodule grew over time and the pain progressed to the point where he noted discomfort with swallowing and breathing. Accordingly, he presented to our medical center for care. On the day of admission, he was febrile (38.7°C), tachycardic (122), hypertensive (152/94). Fortunately, despite the tender neck swelling, there was no airway compromise.\nThe physical exam revealed a tender non-pulsatile mass on the left neck extending to the angle of the mandible. A computed tomography (CT) scan of the neck with contrast revealed a focal outpouching of the left carotid artery (). There also appeared to be a mass encircling the left carotid artery (4.3 cm × 2.5 cm × 3 cm) with lateral displacement and compression of the left jugular vein.\nA vascular surgery consultation was obtained and given concern for a contained ruptured aneurysm, the patient was taken to the operating room (OR). During the surgery, an aneurysm with a posterior perforation and contained rupture was noted (). Pathological specimens from the surgical site were notable for significant atherosclerotic disease. Also, purulence was noted along with extravasated blood and cultures taken from intraoperative specimens revealed pan sensitive S. enterica ser Dublin. The affected and ulcerated portion of the carotid artery was excised and replaced via end-to-end anastomoses with a bovine mesenteric vein graft. Interestingly, all peripheral blood cultures were negative for bacterial growth. He was treated with ceftriaxone 2 grams IV daily for at least 6 weeks. |
The patient is a 61-year-old male with a history significant for NF-1, hypertension, esophagitis and intermittent gastrointestinal bleeding, who initially presented to an outside institution for acute onset of bleeding per rectum. The patient noted bloody bowel movements with diarrhea for several days, which had increased in frequency over the past 24 hours. The patient reported mild abdominal pain and denied nausea, emesis, chest pain, shortness of breath, syncope or other symptoms at the time. He was not on anticoagulation and denied excessive alcohol or nonsteroidal anti-inflammatory drug (NSAID) use. His family history was significant for NF-1 diagnosed in his mother and brother. He had no significant surgical history other than a total hip replacement.\nOn admission at the outside hospital, the patient’s hemoglobin was 6.6 g/dl, with normal platelets, partial thromboplastin time and prothrombin time. Diagnostic workup at the outside institution included a nuclear medicine gastrointestinal scan (mTc-labeled RBCs), which was negative for abnormal bleeding. On hospital Day 2, his hemoglobin failed to respond appropriately to transfusions and a computed tomography angiogram (CTA) was completed, which showed a 6.9 × 5.8 × 6.8 cm hyper-vascular mass in the left pelvis; abutting the left border of the bladder medially, iliac arteries and veins posteriorly, rectus sheath anteriorly and the small and large bowel (). Vascular supply of the mass was identified to originate from branches of the superior mesenteric artery (SMA) and appeared to be contiguous with the adjacent jejunum. The mass was noted to also abut, but not involve, the sigmoid colon. The CT scan further noted a 9 mm enhancing tumor in the second portion of the duodenum anteriorly (). After administration of five total units of packed red blood cells with limited improvement in hemoglobin, the patient was transferred to our institutions for further management.\nThe patient arrived hemodynamically stable and underwent esophagogastroduodenoscopy (EGD). The EGD was unremarkable with no visualization of the previously mentioned duodenal mass. Colonoscopy was performed, which was notable for bright red blood present throughout the entire colon as well as blood 5 cm proximal to the ileocecal valve (). As the patient continued to have bloody output per rectum, with insufficient response to transfusion, interventional radiology was consulted and proceeded with mesenteric angiography with intervention (). The hyper-vascular mass was found to be supplied by ileal branches of the SMA, which were successfully embolized (). After conversations with the interventional radiology team and the patient, the decision was made to proceed with an exploratory laparotomy and resection of the identified mass.\nExploratory laparotomy was notable for a 7 cm mass originating from the distal jejunum (). This mass was mostly exophytic and a single firing of a GIA load was used to excise the mass with preserved adjacent normal wall of the jejunum. Kocherization of the duodenum revealed a single sub-centimeter duodenal mass at D2. The mass was excised with subsequent primary closure of the defect. Taking down the Ligament of Treitz allowed us to identify two additional proximal jejunal lesions that were resected and resulting defects were primarily closed. The remainder of the jejunum was evaluated and a stretch of proximal jejunum, about 10 cm from Treitz, containing 10 small (0.5 to 2 cm) lesions was identified (). The decision was made to resect a 30 cm segment of jejunum with creation of a side-to-side functional end-to-end anastomosis using a linear stapler. This allowed complete resection of macroscopic disease. The mesenteric defect was closed using multiple interrupted silk sutures. The remainder of the bowel was examined and found to be normal. The patient returned to the intensive care unit and recovered over the course of the coming days without immediate complications and without requiring additional transfusions.\nFive surgical specimens were sent to pathology, including the large intra-abdominal mass, a segment of proximal jejunum (30 cm), the duodenal lesion and two proximal jejunal lesions. Microscopic examination revealed a total of 13 foci of tumor. All tumor margins were negative and 12 regional lymph nodes were examined and revealed no evidence of nodal metastases. All tumors underwent H&E staining () and immunohistochemical staining for DOG-1 (), which was strongly positive, confirming a diagnosis of GIST for all tumors.\nGenetic analysis of the tumors samples was performed on paraffin-embedded tissue using the Tempus XT next generation sequencing (NGS) platform, which includes 648 cancer-related genes. No KIT or PDGFRA mutations were detected (both were wild type). Instead, NF-1 was found to harbor a loss-of-function single nucleotide alteration (c.654 + 1G > T splice region variant).\nThe patient recovered appropriately from his surgery and was discharged one week after the operation. He continues to do well and has returned to full-time work. |
A 24-year-old man was brought to the emergency department (ED) of our hospital because of suddenly started abdominal pain, altered mental status, and agitation for the past 2 h. He complained of a severe persistent abdominal pain predominantly in the epigastric area. He was so agitated that apart from abdominal pain, no other history could be obtained. His colleague stated that he last noticed that the patient was “completely well” and was walking to the office about 3 h earlier. Then, he added that he was called by the patient's co-workers as they found the patient agitated while complaining of severe abdominal pain, nausea, stool incontinence, and diarrhea.\nPast medical history of the patient was completely unrevealing, and no chronic medicine use or chronic medical condition was reported by his colleague. Social history was also unremarkable, except for occasional cigarette smoking. No illicit drug use was reported.\nInitial physical examination showed a young thin man who was in severe distress. His heart rate was 110 beats per min, blood pressure (BP) was was 90/60 mmHg, and respiratory rate was 24/min. Oxygen saturation in ambient air was 89%. Auscultation of the heart revealed a moderate tachycardia with muffled heart sounds. Abdominal examination showed hyperactive bowel sounds and tenderness over the epigastrium with mild distension. No evidence of previous surgical scar was found.\nUltrasonography, according to the RUSH protocol, revealed mild pericardial effusion as well as moderate pleural and peritoneal effusions. ECG, performed about 10 min later, showed ST segment elevations in the leads II, III, and aVF. A presumptive diagnosis of internal hemorrhagic shock was made based on the abovementioned findings.\nThe patient underwent emergency contrast-enhanced CT angiography in a try to find any vascular lesion, especially dissection of the aorta. It revealed no evidence of aortic dissection but unexpectedly showed the presence of a radiopaque substance in the stomach, despite no administration of oral contrast (Fig. ). Plus, CT angiography revealed RV (right ventricular) failure as indicated by the contrast media filling the right side of the heart without evidence of contrast in the left side of the heart, small caliber aorta without contrast enhancement, and dilatation of the pulmonary veins, IVC (inferior vena cava), and hepatic veins with early enhancement due to contrast reflux. A contrast-fluid level during the arterial-dominant phase of the CT study was seen in the IVC; Figure .\nThere was increased pulmonary and systemic venous pressure resulting in bilateral pleural effusions, ascites, and submucosal edema in the intestines and gallbladder wall. Attenuation of the nephrogram in cortical phase had been decreased in both kidneys as a result of decreased renal blood flow. A large subcapsular fluid collection was seen in the right kidney (Fig. ). Paracentesis of abdominal ascites showed a clear yellowish fluid without any evidence of intra-abdominal hemorrhage.\nAt this stage, clinical and radiologic findings were in favor of possible oral intoxication with a radiopaque poisonous substance which resulted in RV functional impairment and multiorgan failure. With this presumptive diagnosis in mind, further detailed questioning from his co-workers revealed that the patient had ingested a suspicious bottle of “unknown” fruit juice shortly before the initiation of his symptoms.\nDespite aggressive resuscitation efforts made, the patient developed severe metabolic acidosis 2 h after admission which was refractory to bicarbonate therapy. Meanwhile, his BP started to decline progressively despite receiving intravenous fluids and vasopressor therapy. He was intubated, and mechanical ventilation was initiated. Unfortunately, the patient did not respond to the resuscitative efforts and died about 4 h after admission. Autopsy was performed which confirmed the diagnosis of poisoning with zinc phosphide probably in a suicidal/homicidal scenario. |
Patient B was a 37-year old male of Turkish descent referred for immunological investigation by the treating nephrologists because of IgM deficiency. Asymptomatic renal insufficiency was detected at the age of 28 years when a cirrhosis of the left kidney and mild hydronephrosis of the right kidney were found. Serum creatinine was 3.2 mg/dl (normal range 0.6–1.2 mg/dl), proteinuria was 2.5 g/d. He reported no increased susceptibility to infections, and his chronic renal insufficiency caused only mild clinical symptoms (development of fatigue and tachycardia upon physical strain).\nThe mRNAs encoding the membrane-bound and secreted immunoglobulin heavy chains are produced from identical primary transcripts, which are differently processed at their 3′ ends. Regulation of membrane-bound vs. secreted forms of the immunoglobulin heavy chains depends on the competition of 2 mutual cleavage polyadenylation sites (pAs/pAm) (). In mice targeted deletion of the mu heavy chain cleavage polyadenylation site pAs leads to deficiency of secreted IgM with intact expression of surface IgM and normal secretion of other immunoglobulin isotypes (). Therefore, we sequenced mu heavy chain gene including the polyadenylation sites in both patients with sIgMD and found no alterations (data not shown). Both patients' B cells were able to express precursor, secreted and membrane IgM mRNA (Figure ). Furthermore protein expression of monomeric and native pentameric IgM (Figure ) and surface expression of IgM on the B cell membrane (data not shown) was comparable to healthy controls.\nTo elucidate the genetic basis of the patients' selective IgM deficiency we used a targeted resequencing approach to sequence potential candidate genes. In both patients, we identified defects within the intrinsic B-cell receptor signaling pathway. Patient A harbored a c615G > T missense mutation in exon 8 in the tyrosine kinase BTK. The G > T transition resulted in a glutamic acid to aspartic acid substitution at position 205 within the highly conserved proline-rich (PRR) region located at the C-terminus of the TEC homology (TH) domain (Figures ). Proline rich regions are involved in protein-protein interactions, including interactions with G proteins and intramolecular association with the SH3 domain (). Mutations within the proline rich regions have been shown to abolish SH3 domain binding and result in functional impairment of BTK, pointing toward a potential biologic relevance of the BTK mutation found in patient A (). |
In September 2013, a 50-year-old man from Vietnam with an unremarkable medical history presented at our outpatient clinic with pain in his right flank after a fall. He also complained of voiding difficulty and nocturia. He had smoked one pack of cigarettes (20/pack) per day for 30 years, but did not have any respiratory symptoms. The physical examination was normal except for mild costovertebral angle tenderness on the right side. In the initial laboratory tests, there were no red or white blood cells in urinalysis and the results of the biochemistry tests were within normal limits. Chest radiography showed an old tuberculosis scar, and plain radiography of the kidneys, ureter, and urinary bladder showed a large radio-opaque mass in the right kidney (Fig. ). Abdominal computed tomography (CT) showed a hyperdense mass of 2.62 × 5.70 cm in the right renal pelvis with severe hydronephrosis and cortical thinning. Diuretic-enhanced 99mTc DTPA renal scanning showed that the relative function of the right versus the left kidney was 20 versus 80 %. On the basis of imaging findings, kidney dysfunction due to ureteropelvic junction (UPJ) stenosis with a large renal pelvis stone was initially diagnosed.\nWe created a percutaneous nephrostomy (PCN) to decompress the hydronephrosis. Interestingly, the drained urine volume was almost zero and gelatinous material was aspirated via PCN catheter.\nUrinary cytopathology of the gelatinous material indicated no malignancy. We reviewed the preoperative radiological images and noted that the stone did not obstruct the UPJ directly, although the stone was located in the renal pelvis. The upper ureter, located below the stone, was dilated (Fig. –), and a transverse view of the abdominal CT showed dense lines and unclear enhancement in the dilated right renal pelvis, suggesting septa (Fig. ). Although the urinary cytopathology was negative for malignancy, we could not exclude the possibility of other disease or hidden malignancies of the kidney. We performed a radical nephrectomy with a grossly safe resection margin of the ureter without an intraoperative frozen section study in the resected kidney and resected the margin of the ureter.\nPathological examination of the kidney after surgery revealed a mucinous cystadenocarcinoma in the renal pelvis. Endoscopy showed no other tumours in the gastroduodenal tract, and a bone scan and positron emission tomography-computed tomography (PET-CT) showed no evidence of other malignancies, metastasis, or remnant cancer.\nThe patient did not receive any post-operative radiation therapy or chemotherapy. He was followed up via urinalysis, biochemistry, urinary cytopathology, cystoscopic examination, and abdominal CT scan at 3-month intervals postoperatively for the first year and every 6 months thereafter and a yearly PET-CT. He has been well, without evidence of tumour recurrence or metastasis, for 20 months post-surgery.\nGrossly, the kidney showed a cystically dilated pelvi-calyceal system with thinning of the parenchyma and contained mucoid material. At cross section, there was no definite mass-like lesion, and the cut surface had a soft consistency with a gelatinous appearance. The large stone was noted in the dilated lower calyx adjacent to the UPJ and did not obstruct the UPJ directly. The stone seemed to float in a mucin pool.\nOn light microscopic examination, the renal pelvis was composed of pools of mucus with clumps or strands of neoplastic glandular epithelium (Fig. ), and the pelvic mucosa consisted of tall columnar cells that tended to stratify into two or more layers, with perfuse irregular infolding and protrusions into the surrounding stroma (Fig. ). The tumour cells extended into the peripelvic fat tissue and were not found in the resection margin of the ureter.\nThe pathogenesis of primary mucinous adenocarcinoma in the renal pelvis is unclear. A possible mechanism involves glandular metaplasia of the urothelium that develops in response to injury (e.g. chronic irritation, inflammation, infection, hydronephrosis, or urinary calculi) and progresses to dysplasia and adenocarcinoma [, , , ]. In our case, we believed that the renal pelvis stone may have caused chronic inflammation in the renal pelvis, which then spread to the entire pelvis and ureter. This also induced stenosis of the ureter, resulting in hydronephrosis of the right kidney. Over time, this condition might have given rise to glandular metaplasia of the urothelium, leading to development of this tumour.\nIn previous reports, no characteristic symptoms of primary mucinous adenocarcinoma were noted. Most patients simply reported flank discomfort. A palpable abdominal mass may be a sign of late-stage disease [, , ]. In our patient too, there was no specific symptom except pain over the right flank and mild costovertebral angle tenderness on the right side on physical examination. We believe that it is impossible to suspect or diagnose this tumour through history taking and physical examination preoperatively.\nRadiological studies including abdominal ultrasonography, intravenous pyelography, and abdominal CT may not be able to identify a malignant tumour []. According to Sisoda et al. [] and Abbas et al. [], no radiological features define a primary mucinous adenocarcinoma. In most reported cases, a non-function kidney, hydronephrosis, and renal calculi were noted in abdominal CT scans; these features are consistent with, but not conclusive of, malignancy. Moreover, most primary mucinous adenocarcinomas of the renal pelvis are diagnosed only after pathologic analysis of the resected specimen. Similarly, in our case, severe hydronephrosis with cortical thinning and a large renal pelvis stone were observed in abdominal CT scans, and the decreased kidney function was identified with diuretic-enhanced 99mTc DTPA renal scanning, which led to the initial diagnosis. However, in the review of the preoperative radiological images after indentifying gelatinous material aspiration without urine drainage via PCN catheter, the stone did not obstruct the UPJ directly, and the upper ureter, located below the stone, was dilated. Generally, in kidney dysfunction due to renal pelvis stone, the UPJ is directly obstructed by the stone. Therefore, we hypothesize that hydronephrosis without direct obstruction of the renal pelvis by a stone and ureter dilatation below the level of the stone without definite obstruction causes could be signs of this tumour.\nIn view of the presence of cysts containing large pools of mucin and gelatinous areas in most documented cases of mucin-secreting adenocarcinoma, Raphael et al. [] and Abbas et al. [] suggest that diagnosing carcinoma in this circumstance requires a strong clinical suspicion and that an intraoperative frozen section study or cytology may help confirm the diagnosis and planning the appropriate surgery. Unfortunately, cytopathology of the gelatinous material via preoperative PCN did not help to diagnose this tumour preoperatively in our case. However, we suggest that the preoperative Tru-Cut biopsy may help to detect this tumour and to decide the range of surgical treatment.\nThe recommended treatment for tumours in the renal pelvis is radical nephrectomy and total ureterectomy, including the intravesical area []. In our case, without an intraoperative frozen section study in the resected kidney and resection margin of the ureter, we performed a radical nephrectomy with a grossly safe resection margin of the ureter.\nAlthough fortunately there was no tumour in the resection margin of the ureter, we believe that surgical treatment based on reasons of absence of malignancy in the preoperative cytopathology and decreased kidney function was an error in a patient who could have had other disease or hidden malignancies. We should have performed an intraoperative frozen section study of the kidney, along with a total ureterectomy including the intravesical area. We also suggest that the radical nephrectomy with total ureterectomy is necessary in patients who are suspected or diagnosed with adenocarcinoma of the renal pelvis or ureter.\nDespite reports of good prognosis without recurrence even 3 or more years after surgery [], the overall prognosis of patients with primary mucinous adenocarcinoma is poor, with ~50 % of the patients dying within 2 years of surgery [, ]. Local recurrence due to both spillage of tumour cells during surgical manipulations and downward seeding in the distal ureter has been reported [, ]. Fortunately, in our case, the patient has been well, without evidence of tumour recurrence or metastasis, for 20 months after surgery.\nThe guidelines regarding follow-up and surveillance are not yet established in primary mucinous adenocarcinoma of the renal pelvis and ureter. However, we suggest that the European Association of Urology (EAU) guidelines on urothelial carcinomas of the upper urinary tract [] may help to evaluate the recurrence or metastasis of this tumour. |
A male patient aged 47 years presented with a chief complaint of worn-out lower front teeth. A complete oral examination followed by a detailed medical and dental history was done and recorded. The oral hygiene status of the individual was fair [Oral hygiene index (OHI) score=1.5]. Medical history revealed that the patient was a known diabetic under medication Glipizide once daily (OD). He presented with a history of occasional grinding of teeth.\nIntraoral examination revealed, congenitally missing teeth in relation to 31, 41. All the remaining teeth showed signs of attrition, without any specific symptoms. Most of the posterior teeth showed class V fillings, which suggests that the lesions could be due to stress caused by the parafunctional habits like bruxism and clenching. Lower anterior teeth in relation to 32, 33, 42 and 43 were severely attrited with inadequate clinical crown length. Teeth numbers 11, 12, 21 and 22 were slightly palatally tilted, and the palatal aspects of these teeth also showed gross attrition, due to deep bite. Teeth numbers 21, 22, 32 and 33 were already endodontically treated as he had a history of pain and recurrent infections in relation to these teeth [Figures and ].\nEvaluation of vertical dimension of occlusion did not reveal any signs of loss of vertical dimension of occlusion (VDO). Occlusal analysis was done intraorally, and it revealed that there was no definite anterior guidance; and, thereby, absence of any mutually protected occlusion. Lateral movements of mandible showed that on the left working side, there was group function; and on the right nonworking side, there were interferences in the molar regions (46, 47 and 48). On the right working side, there were canine and premolar occlusions, with nonworking side on the left showing interferences in the second and third molars (37 and 38). Even with these clinical signs, the patient did not present with any symptoms of Tempero Mandibular Joint (TMJ) dysfunction or disorder.\nThe treatment was planned in three phases.\nScaling and root planing\nCrown-lengthening (in relation to lower anterior teeth) Porcelain-fused-to-metal (PFM) crowns (in relation to 11, 12, 21, 22; and 32, 33, 42, 43)\nFollow-up and oral hygiene instructions Use of a night guard to minimize effects of bruxism and also to act as a habit-breaking appliance\nFirst, the patient’s maxillary and mandibular impressions were made with irreversible hydrocolloid impression material, and two pairs of diagnostic casts were prepared.\nA complete oral prophylaxis, including root planing, was done as a part of initial therapy. The patient was recalled after a week for evaluation and was found to maintain satisfactory oral hygiene.\nThe pre-surgical analysis consisted of the following[]\nDetermination of the finish line prior to surgery[] Transcrevicular circumferential probing prior to surgery, to establish the biologic width.\nPrior to the procedure, evaluation of the tooth and periodontium was done clinically and radiographically. The clinical measurements included probing sulcus depth, biologic width, osseous crest, pulpal involvement, gingival health, loss of mesial/distal occlusal space, anticipated final margin placement.\nRadiographic assessment included level of alveolar crest, pulpal involvement, root length, root form, crown-to-root ratio (pre-treatment or post-treatment).\nThere was a generalized mild loss of attachment (1-2 mm). The crown and root lengths were evaluated from the radiographs. The findings were as follows:\nCrown length was 6 mm, 6 mm, 5 mm and 5 mm in relation to 42, 43, 32 and 33, respectively. Root length was 12 mm, 14 mm, 10 mm and 11 mm in relation to 42, 43, 32 and 33, respectively.\nThe amount of crown-lengthening planned was about 2 mm more than presently available crown length. Anticipated crown-root ratio following completion of the procedure, on an average, would be 1:1.2, which is considered favorable.\nThe patient was scheduled for an apically repositioned flap to provide adequate clinical crown length to facilitate prosthesis.\nInverse bevel incisions were placed to reduce the bulky tissue. The flaps were extended one tooth distal of both the lower canines to permit adequate access to perform osseous surgery. Maximum preservation of keratinized gingiva was recommended. The scalloping of the flap was performed anticipating the final underlying osseous contour. A full-thickness flap was reflected up to the mucogingival junction and split apically [Figures and ].\nFollowing proper degranulation, osteoplasty followed by ostectomy was performed to obtain at least 4 mm of healthy tooth structure above the alveolar crest.\nOstectomy and scalloping of the bone buccally and lingually were performed not only on the affected tooth but also onto the adjacent teeth for blending and gradulization of osseous architecture.\nSince the amount of keratinized gingiva was around 3 to 4 mm, the flap was positioned at the crest of alveolar bone and sutured using a black braided silk suture []. Routine postoperative instructions were given. The medications prescribed were, amoxicillin 500 mg tid for 5 days and Paracetamol qid for 3 days.\nThe patient was recalled after 1 week for suture removal [], following which the patient was referred to the Department of Prosthodontics for the fabrication of PFM crowns in relation to 11, 12, 21, 22, 32, 33, 42 and 43.\nThe main aim of this treatment was to create esthetic and functional crowns and thereby develop mutually protected occlusion by generating an anterior guidance to disocclude posteriors, thereby generating group function/canine and premolar occlusion on working side and removing nonworking side interferences.\nAnterior-bite plane splint was fabricated with a self-cure acrylic resin to disocclude the posterior teeth in centric and eccentric jaw positions, so as to allow for the passive supraeruption of all the posterior teeth. Only the lingual surfaces and incisal edges of lower anteriors were covered with the bite plane. splint Labial surface was kept open so that the post-surgical maintenance in the region of the surgery was not hampered. The patient was instructed to wear the splint through the day for at least4 to 6 weeks, so as to allow for passive supraeruption of posterior teeth.\nThe patient was recalled after a month and evaluation of occlusion was done. It was observed that there was no significant supraeruption of posteriors, but there was a good amount of tooth exposed in relation to 32, 33, 42 and 43 for the abutment height post-surgery. Therefore, teeth preparation to receive PFM crowns was scheduled in relation to 11, 12, 21, 22, 32, 33, 42 and 43.\nUpper and lower elastomeric impressions (3M ESPE – Express XT putty soft and light body) were made for the fabrication of final definitive restorations. Provisional restorations for both upper and lower teeth were fabricated with self-polymerizing acrylic resin (DPI – self-cure – tooth-molding powder). Protrusive wax record was taken. Provisional restorations were cemented with non-eugenol temporary cement (3M ESPE – Relyx Temp lute). Casts were made from the elastomeric impressions and mounted on semi-adjustable articulator (Hanau, non-arcon) with the help of a face bow and interocclusal records [].\nWith the help of protrusive record, horizontal condylar guidance angle was generated, which was 45°. Lateral condylar guidance angle was calculated based on the Hanau formula, L = (H/8) + 12, which was found to be 17°. Articulator was programmed according to these values.\nWax patterns for the fabrication of PFM restorations were made and cast with metal. In case of upper incisors, labial ceramic facing with palatal metal backing was planned; whereas for the lower anteriors, full ceramic coverage was planned. In the final crowns, overjet was slightly increased to compensate for the deep bite. Metal try-in was done to check the fit of the crowns on the natural teeth. It also gives an idea about overjet and overbite. After the fabrication of PFM crowns, refining of occlusion was done in the patient intraorally [Figures and ].\nCare was taken so that the occlusion on the working side was not canine guided, as canine is restored with a crown and if the habitual grinding continues, it can lead to ceramic fracture in relation to the canine 33, 43. Therefore, if canine is disoccluding the premolars and molars on the working side, the length of 33, 43 is so adjusted that there is group function / canine and premolar guidance. On the left side, group function; and on the right side, canine and premolar guided occlusion were generated (on the working sides) as planned. All the non working interferences on both the sides were removed [Figures , and ].\nAfter refining the occlusion, the crowns were glazed and cemented with glass ionomer cement (3M ESPE -Easy mix). After cementation, alginate impressions of upper and lower arch were made, and casts obtained were used for the fabrication of night guard. Both upper and lower night guards were made and dispensed to the patient with instruction to wear them every night, so as to break the habit of grinding and at the same time protecting the crowns from fracture [Figures and ]. |
A 59-year-old female with a medical history of alcoholic cirrhosis who had undergone uncomplicated elective splenectomy and distal pancreatectomy for splenic artery aneurysm 2 months ago was admitted to the intensive care unit with acute hypoxic respiratory failure for two weeks. She was found to have increased respiratory rate, diminished breath sounds, and abdominal ascites with shifting dullness. The patient had hypoxia, which was attributed to hepatopulmonary syndrome and compressive atelectasis from hepatic hydrothorax. Common causes of hypoxia, including pneumonia, congestive heart failure, and pulmonary embolism, were excluded during work-up.\nThe patient underwent diagnostic and therapeutic paracentesis as well as thoracentesis. Both ascitic and pleural fluid chemistries revealed elevated triglyceride levels (268 mg/dL in the ascitic fluid); therefore, a diagnosis of CA was made. Microbiological work-up, including gram stain and cultures, were negative. No malignant or suspicious cells were seen in the cytological analysis. She was started on albumin and diuretic therapy with furosemide and spironolactone for portal hypertension. The patient underwent multiple paracentesis procedures with removal of about 2-3 liters of chylous fluid every 72-96 hours. Due to the recurrence of effusion and need for frequent paracentesis, with poor prognosis, goals of care discussion were planned, and a temporary peritoneal drainage catheter was placed for symptom relief with a plan to discharge the patient on hospice care.\nDespite the above measures, accumulation of ascitic fluid was very challenging to control. She was started on treatment with octreotide 100 micrograms scheduled three times per day. She received it for five days. There was a remarkable improvement in the collection of ascitic fluid during octreotide therapy. The patient’s ascitic fluid collection improved from about 600 mL per day to 25 mL per day, as shown in Figure . Her supplemental oxygen requirement improved significantly, and the patient was discharged on diuretic therapy. She underwent TIPSS (transjugular intrahepatic portosystemic shunt surgery) after her clinical improvement. With this, there was a significant improvement in her overall functional status. The patient was referred for liver transplantation. |
A 3-year-old male patient was assessed in the pediatric emergency department on 3 June 2020 for a 3-day history of fever and shortness of breath and a 2-day history of productive cough. His mother denied having any history of chills or hemoptysis. Review of systems was unremarkable. There was no history of travel outside Damascus city in the recent past. He had no exposure to birds or contact with sick persons. He had no pets at home. There were no smokers in the home. His mother said that he had a history of frequent colds and repeated respiratory infections. He also suffered repeated pneumonic infections. There was no history of allergy, sleep apnea, and weight loss. No family history of asthma or atopy was present. Family history revealed that his sister was once diagnosed with right-sided heart by a clinician on routine examination. She had no history of repeated respiratory infections or repeated pneumonic infections. His parents were healthy and not related. His mother reported no problems during pregnancy.\nIn the emergency department (ED), the patient was reported to be in severe respiratory distress. Vital signs revealed temperature of 38°C, respiratory rate of 50 breaths per minute, heart rate of 120 beats per minute, blood pressure of 110/60 mm Hg, and oxygen saturations of 75% on room air. The infant appeared acutely ill. He was diaphoretic. Physical exam revealed nasal flaring, with severe suprasternal, intercostal, and subcostal retractions. On cardiovascular examination, apex beat was felt on right fourth intercostal space along midclavicular line. Heart sounds were best audible on the right side of his chest. On chest auscultation, there was diminished air movement in both lungs, bilateral wheeze, coarse crackles at the bases of both lungs were audible, with coarse rhonchi present in both upper lobes. There was no evidence of otitis media with effusion as confirmed by otoscopy. The palatine tonsils were observed to be grade 3 hypertrophic (Brodsky Grading Scale), but with no active infection.\nOther physical exam findings were unremarkable. Chest X-ray revealed cardiac apex and aortic arch on right side, suggesting dextrocardia, and right-sided stomach air suggesting a case of situs inversus, with normal lungs fields (). A chest computed tomography (CT) scan showed dextrocardia with no radiographic evidence of bronchiectasis (). Ultrasound examination of the abdomen showed a normal liver and gall bladder on left side and a normal spleen on right side, suggestive of situs inversus. A CT of the paranasal sinuses revealed sinusitis. The examination showed opacified maxillary and ethmoidal sinus cavities (). A CT scan of the abdomen showed liver on the left and spleen on the right, suggestive of situs inversus (). Electrocardiogram showed signs of dextrocardia, inverted P waves in lead I, right axis deviation and QRS complexes get progressively smaller in leads V 1–V 6. Two-dimensional echocardiography was consistent with dextrocardia; all four chambers were normal. Doppler study confirmed situs inversus of aorta and inferior vena cava. Sweat test for chloride was also undertaken to rule out cystic fibrosis and it was negative on two occasions. The tuberculin skin test was negative. Immunoglobulins tests were normal.\nA diagnosis of KS was made based on clinical presentation and imaging features. The case represented a classical KS. The current diagnosis was consistent with severe acute bronchitis.\nThe patient was initially hospitalized in the ED where he received nebulized salbutamol and ipratropium, intravenous methyl prednisolone, and intravenous magnesium sulfate. The patient was placed on a facial mask with partial rebreathing reservoir, 10 L/min O2 was administered with it, and his oxygen saturation was measured as 93% with pulse oximetry. Initial arterial blood gas (ABG) analysis revealed partially compensated respiratory acidosis with Type 2 (hypercapnic) respiratory failure, pH 7.26, PaCO2 82 mmHg, PaO2 83 mmHg, HCO3− 36 mEq/L, and SaO2 94%. Serum electrolytes, renal and liver function tests, and complete blood counts were normal. Two hours after administration of drug treatment, ABG analysis was conducted again. The analysis showed no improvement in the patient’s condition. There was no clinical improvement, so the patient was moved to the PICU, where noninvasive ventilation (NIV) was initiated using a full face mask and bilevel positive airway pressure with inspiratory positive airway pressure of 8 cm H2O, expiratory positive airway pressure of 4 cm H2O and a FiO2 of 50%. SpO2/FiO2 ratio was 188. The ABG values are summarised in .\nIn view of worsening dyspnea and falling saturation, the pressures were gradually increased to 16 and 6 cm H2O respectively, and FiO2 was gradually increased to 90%. ABGs repeated after 2 h revealed no improvement. As the patient was not improving on NIV and was becoming hypoxemic and agitated, he was given 2 mg/kg of propofol, and the trachea was intubated in a single attempt using a cuffed tube of internal diameter 4.5 mm. The ventilator was set as synchronized intermittent mandatory ventilation (SIMV) mode with pressure control 16 cmH2O, FiO2 0.5, positive end-expiratory pressure (PEEP) 5 cmH2O, frequency 25 times/min, inspiratory time to expiratory time ratio 1:3, pressure support 10 cmH2O, PEEP 5 cmH2O. He was sedated intermittently with intravenous midazolam. Ketamine and intravenous salbutamol were added to meet optimal treatment. ABGs measured after 2 h of mechanical ventilation were as follows: pH 7.30, PaCO2 65 mmHg, PaO2 112 mmHg, and SaO2 99%. A nasogastric tube was inserted for enteral feeding and intermittent oral suctioning was performed. He was continued on scheduled intravenous methyl prednisolone and intravenous magnesium sulfate in the PICU as well. The patient was treated with a combination of antibiotics. The ABG analysis conducted 6 h after the beginning of the mechanical ventilation found a pH 7.38, PaCO2 44 mmHg, PaO2 115 mmHg, and SaO2 99%. Serial ABGs were done and showed marked improvement. On day 4, he had clear consciousness with adequate cough reflex and stable cardiovascular function. Oxygen saturation remained 98% or higher after 24 h had passed since the beginning of ventilation using a T-piece, and 3 L/min of oxygen was provided to the patient. PaCO2 was 40 mmHg. Thereafter, extubation was done.\nAfter extubation, O2 5 L/min was applied via facial mask with partial rebreathing reservoir and he was observed closely. Thirty minutes later, the ABG analysis was pH 7.45, PaCO2 40 mmHg, PaO2 120 mmHg, and SaO2 99%.\nThe patient had no complication during the PICU stay and was subsequently discharged from the PICU on day 5, with pH 7.48, PaCO2 42 mmHg, PaO2 102 mmHg, HCO3− 33 mEq/L, and SaO2 99%, and blood test results were in the normal range. Chest X-ray showed no infiltrates. Then, he was treated with orally administered antibiotics, mucolytic, bronchodilator and chest physiotherapy. He was symptomatically better with the above therapy and started on long-term low-dose prophylactic antibiotic. He was advised for influenza and pneumococcus vaccines. He was then referred to regular follow-up in otolaryngology, pulmonology, and general pediatric medicine. After 2 months of follow-up, the patient is doing well. |
A 3-year-old girl was referred to a tertiary metabolic bone disease unit for premature loss of primary teeth with roots intact and low serum ALP activity (123 IU/L; reference range: 230–700 IU/L) []. Routine genetic testing revealed compound heterozygosity (c.350A > G, p.Y117C, c.400_401AC > CA, p.T134H) for different TNSALP missense mutations in exon 5 of the ALPL gene, confirming the diagnosis of HPP. On presentation, radiologic assessment of the left hand and arm showed tongue-like lucencies projecting into the metaphyses consistent with childhood HPP. She did not have any clinical features of skeletal involvement of the lower limbs and no motor developmental delay except for a mild waddling gait as a younger child. The patient had a relatively asymptomatic clinical course until she presented at age 11 years with swelling and tenderness of the left ankle that was nonresponsive to paracetamol or ibuprofen. An MRI scan of the ankle suggested a diagnosis of chronic recurrent multifocal osteomyelitis, which was subsequently confirmed by biopsy. The symptoms of pain and swelling of the lower limb joints showed spontaneous transient improvement at age 13 years. Recurring at age 14 years, the symptoms fluctuated and caused significant pain and disability. These symptoms eventually stabilized when the patient was transitioned to adult care at age 17 years. At age 18 years, she successfully underwent radiofrequency ablation for Wolff-Parkinson-White Syndrome, a cardiac disorder unrelated to HPP. The patient is now 27 years of age and has experienced an episode of metatarsal stress fracture; she also suffers from generalized aches and pain.\nOver 22 years, this patient was hospitalized 3 times for a total of 19 days (Table ). Only 1 hospitalization exceeded 3 days, when the patient was admitted for 14 days to receive intravenous antibiotics for suspected osteomyelitis (Table ).\nOutpatient specialist visits, outpatient procedures, and day case procedures represent the majority of healthcare resources used by this patient (Fig. ). Seven specialists provided care for the patient; a pediatric dentist was seen on 40 occasions. Dental procedures, including restorative dentistry (performed on 3 occasions), were the most common of these. This patient was also seen by a pediatric rheumatologist and psychiatrist (Table ). |
A 75-year-old man was referred to our hospital for abdominal fullness and nausea since 2 months. He had a medical history of hypertension and hyperlipidemia and a surgical history of the right inguinal hernia. The patient's laboratory findings were within normal limits. Abdominal computed tomography (CT) revealed a well-demarcated oval isodensity mass of 25 mm at the tip of his appendix. Contrast-enhanced CT revealed a lesion with gradual homogeneous contrast enhancement from the arterial phase to the equilibrium phase (). No abnormal findings were found in the root to the middle of the appendix. Abdominal ultrasonography (US) revealed a well-demarcated hypoechoic tumor. The tumor size was 22 mm × 18 mm × 18 mm, with some cystic area and blood flow (). Colonoscopy findings were normal. The patient's symptoms naturally alleviated during examination period.\nPreoperative diagnosis indicated appendiceal neuroendocrine tumor (NET) G1 or gastrointestinal mesenchymal tumors, such as GIST. Malignancy could not be ruled out; therefore, laparoscopic ileocecal resection with D3 lymph node dissection was recommended. Intraoperative findings revealed a well-demarcated tumor at the tip of the appendix, with no invasion into the surrounding tissue. This observation was similar to the preoperative imaging findings. According to another intraoperative finding, dissecting the adhesion between the terminal ileum and the peritoneum, which was the effect of the past herniorrhaphy, was necessary. The operation time was 167 min, and the amount of blood loss was 100 ml.\nPathological findings revealed a well-demarcated tumor originating from the muscular layer at the tip of the appendix and spindle-shaped heterotypic cells proliferating in a bundle. Vascular invasion and lymph duct invasion were not detected. No tumor cells were found in the dissected lymph node. Immunohistochemical studies revealed negative values for KIT and CD34 and positive values for S-100 protein (), which confirmed the schwannoma of the appendix. The patient was discharged on the 9th day after surgery without any complication requiring medical treatment. The patient is presently doing well without any evidence of recurrence at 3 months after surgery. |
Patient presentation
A 21-year-old female college student with a history of asthma presented to the neurosurgery office for consultation complaining of mass on the left side of her skull associated with increasing size over the past two days and intermittent headaches for the past two to three weeks. The left-sided headache included her upper jaw. She also reported a history of cellulitis and urinary tract infections, in addition to surgical removal of an impacted wisdom tooth in 2016. Family history was positive for diabetes mellitus (DM) type II in both her father and her grandfather and colon cancer and coronary artery disease in her other grandfather. She admitted to drinking alcohol one to two times per week but denied use of tobacco and drugs. At the time, she was taking Viorele birth control to regulate her menses. Review of systems was otherwise negative.
Clinical findings
Physical examination revealed a well-developed, well-nourished female in no acute distress. She was awake, alert and oriented to person, place and time with a Glasgow Coma Score (GCS) of 15. A soft left frontal lesion associated with tenderness to palpation, without erythema or drainage, was palpated slightly off midline. Her cranial nerves II-XII were intact. Strength in both upper and lower extremities was five out of five bilaterally. No pronator drift was noted. Sensation to light touch was intact bilaterally in V1-3, upper extremity, and lower extremity distributions. Her reflexes were symmetric. Her gait was within the normal limits.
Imaging
CT of the head without contrast (Figure ) revealed an expansive soft tissue mass with beveled edges and dimensions measuring approximately 3.5 x 2.1 x 2.3 cm in the left frontal calvarium. Bony destructive changes of the inner and outer table of the left frontal calvarium were apparent. Extension of the mass into the dura was noted. The mass did not extend into the |
A 78-year-old female nonsmoker had previously been examined, a CT scan of the chest having shown a left upper lobe pulmonary mass (A). At that time, she underwent bronchoscopy with BAL and bronchial biopsy. The BAL fluid and the biopsy sample were both negative for neoplastic tissue. She subsequently dropped out of follow-up. Two years later, she was referred to our hospital with a six-month history of shortness of breath, dry cough, and weight loss. A chest X-ray and a chest CT scan (without contrast) identified a 10 × 7 cm lesion in the left upper lobe without forming cleavage plans with the aorta and the pulmonary artery (Figures 1B and 1C), with a small left pleural effusion and small mediastinal lymph nodes. An 18F-fluorodeoxyglucose positron emission tomography-CT (FDG PET-CT) scan (D) showed increased metabolic activity in many organs, greatest in the mass in the upper left lobe, the maximum standardized uptake value (SUVmax) of which was 22. Metabolic activity was also increased in the mediastinal lymph nodes (SUVmax = 7), as well as in the right and left suprarenal glands (SUVmax = 16 for both); the aortic lumbar lymph nodes (SUVmax = 11); and the right inguinal lymph node (SUVmax = 10). In addition, there was a mesenteric focus, together with subcutaneous tissue nodes and multiple locations in bone.\nThe bronchoscopic examination revealed a mass occluding the left upper lobe bronchus (), and a bronchoscopic biopsy was performed. Before the results of the tissue sample examination had been obtained, she developed right hemiparesis and a new subcutaneous nodule arose. A CT scan showed multiple brain lesions. She was started on corticosteroids. However, she showed no clinical improvement and brain radiotherapy was proposed.\nThe histological examination of the biopsy specimen showed a soft-tissue neoplasm with a sheet-like or enclosing cleft arrangement of spindle and epithelioid cells with prominent nucleoli, with some multinucleation; mitotic figures were conspicuous, as were extensive necrosis and hemorrhage (A). Immunohistochemical staining of the tumor specimen revealed that it was strongly positive for CD31 (B), factor VIII-related antigen (C), and vimentin; weakly positive for the nuclear transcription factor Fli-1 and cytokeratin AE1/AE3; and negative for CD34, desmin, and smooth muscle actin. Therefore, the histology and immunohistochemistry were both consistent with epithelioid angiosarcoma.\nTwo weeks after the histologic diagnosis had been made, the patient died. Her death was attributed to the progression of the disease, the brain metastases in particular. |
A 43-year-old male () presented at our hospital with abdominal pain and abdominal mass. Results of his blood chemistry and routine blood tests were within normal range. Urine cytology showed no signs of malignancy, but the patient had an elevated CA-125 at 345.7 kU/L. The abdominal CT and US findings were reviewed. The case underwent unenhanced and enhanced CT scans (arterial, venous, and excretory phases scanning) and coronal and sagittal multiplanar reconstructions in the venous phase. The unenhanced CT scan showed a unilateral large solid renal infiltrative mass completely replacing the left kidney (). The mass had a maximum diameter of ~12 cm. The margin of the tumor was poorly defined with a lobulated contour. Tumor necrosis and hemorrhage were detected in the case. The mass showed weak or moderate heterogeneous enhancement. The contrast-enhanced CT showed that the tumor contained multiple irregular septa-like structures. The patients had renal vein thrombosis and a metastasis to the regional lymph nodes (). A lung metastasis was detected in the patient at the initial diagnosis (). Extracapsular extension was also detected in both, but invasion to the adjacent organs was not detected. US showed an ill-defined, irregular and slightly echogenic mass without acoustic shadowing, which replaced the left kidney completely. The patient had renal vein thrombosis and had extension of thrombosis into the inferior vena cava and right atrium. CDFI showed moderate blood flow within the mass (). The patient underwent chemotherapy after a biopsy; 2 months later, the size of the tumor decreased significantly, and the patient underwent a radical nephrectomy, but 3 months later, the patient had tumor recurrence including lung, liver, lymph node, and mesenteric metastases. The histopathology reports also revealed a small round blue cell tumor (). On immunohistochemistry, the tumor cells stained positive for CD99 (), CD117, Syn and EWS rearrangement (EWS-FLI1 translocation) on fluorescent in situ hybridization. |
A 65 year-old white female patient had been previously evaluated an outside facility for recent development of edema and CKD. She had a history of hypertension diagnosed at age 58 on treatment with angiotensin II receptor blocker. At time of her initial evaluation (at an outside facility) her serum creatinine was 1.1–1.2 mg/dL and urine analysis demonstrated proteinuria +++. She underwent a kidney biopsy that showed a FSGS lesion and presence of focal myelin figures within podocytes (zebra bodies). GLA sequencing for FD was negative. She had no history of prior exposure to silica, amiodarone or hydroxychloroquine, which can be associated with lesions resembling zebra bodies on EM. Her mother was diagnosed with FSGS at age 69 years, then developed end-stage renal disease, 2 years later, and was on dialysis until her 80s when she died. The patient’s father had died due to liver cancer. She has two healthy brothers, ages 56 and 63, and two healthy sons who are 39 and 40 years old.\nDue to the inconsistency between the EM findings and the genetic analysis, and to obtain further clarification regarding the diagnosis, the patient was referred to Mayo Clinic 4 years after the first evaluation. She had no history of acroparesthesias, angiokeratomas, transient ischemic attacks, hypohidrosis, parapelvic cysts or any other FD-related signs or symptoms []. One son had negative biochemical testing and the other had negative genetic testing for FD. Physical examination was unremarkable including no evidence of corneal abnormalities in ophthalmological evaluation. Laboratory evaluation is presented on Table . Cardiac and brain magnetic resonance imaging were unrevealing. To further evaluate the cause of her renal disease, she underwent a repeat kidney biopsy. On light microscopy (LM) there were 30% globally sclerotic glomeruli and one glomerulus showed segmental sclerosis with podocyte capping. Few glomeruli showed duplication of the glomerular basement membranes. There was mild tubular atrophy and interstitial fibrosis (Fig. a) involving 25% of the cortex sampled accompanied by mild chronic interstitial inflammation. Some podocytes with bubbly cytoplasm were seen on LM (Fig. b). No glomeruli were seen in the immunofluorescence. There was 3+ focal tubular casts staining for IgA, kappa, and lambda with 2+ IgM. The medullary tissue was negative for IgG, C1q, C3, albumin, and fibrinogen. On EM, several podocytes contained myeline figures resembling zebra bodies (Fig. c and d) with podocytes displaying mild to moderate foot process effacement involving approximately 30% of the total peripheral capillary surface area. No myelin figures were seen within endothelial cells, mesangial cells, peritubular capillaries, or tubular cells. No beaded collagen fibrils were seen within the glomerular basement membranes. GLA deletion/duplication testing for FD gene was repeated and was negative. Due to the inconclusive biochemical and genetic testing, she was referred to the Department of Clinical Genomics for further evaluation. To further evaluate the cause of her renal biopsy findings, RNA sequencing of kidney tissue was performed through a research program in the Center for Individualized Medicine []. No variants, aberrant expression, aberrant splicing changes, or allelic imbalance was detected within GLA by RNA analysis. Moreover, there was no sign of skewed X chromosome expression. To search for other possible genetic explanation for her symptoms, whole exome sequencing was performed on the kidney tissue and revealed a pathogenic variant in LMX1B (NM_002316.3:c.737G > A, p.Arg246Gln), which is associated with NPLRD in multiple families. The variant was Sanger confirmed to be germline on DNA extracted from the patient’s blood. Her renal disease progressed and 2 years after presentation to the Mayo Clinic the patient received a kidney transplant from a deceased donor. |
A 6-year-old boy from a rural area came in to the hospital with a chief complaint of a right axillary mass with progressive growth for 1 month. He denied inflammatory signs or pain, but had limitation in abduction of the right upper limb. Initially he was diagnosed with pyogenic adenitis and treated with cephalexin, without much improvement. He also complained of other enlarged lymph nodes in the neck and inguinal regions with spontaneous resolution and intermittent articular pain of the wrists, ankles and knees. His past medical history was unremarkable and his immunizations were up to date. He had frequent contact with cattle and raw milk, but not with cats. He denied any previous sick contacts. On his first medical evaluation, a neck ultrasound showed multiple enlarged lymph nodes; therefore, a diagnostic lymph node biopsy was done. Chronic granulomatous inflammation was reported; however, special staining of the specimen was not performed due to local administrative issues. Upon admission to our hospital he appeared well with normal vital signs. He had small palpable lymph nodes in the posterior cervical chain and in the right supraclavicular region. In the right axillae he had a well-defined, round lymph node, 4 cm in diameter, without inflammatory changes (Fig. ). The rest of the physical examination was normal. Laboratory workup included a normal CBC, acute phase reactants, renal and liver function tests within normal limits and negative antinuclear antibodies. Chest X-ray and abdominal ultrasound were normal. However, chest CT showed multiple lymphadenopathies in the right axillae. Tuberculin skin test and gastric aspirates for acid fast bacilli staining and Mycobacterium tuberculosis cultures were all negative. Serologies for Cytomegalovirus, Toxoplasma gondii, Epstein–Barr virus, Brucella and HIV were also nonreactive. A new biopsy from the right supraclavicular lymphadenopathy was performed. Histopathology reported liquefaction necrosis with evidence of bacilli compatible with Bartonella spp on Warthin Starry staining (Fig. ). AFB, silver methenamine and PAS staining were all negative. Other studies were not performed because these are not available locally. A diagnosis of CSD was made and the patient was discharged and treated with azithromycin for 5 days. One month later he returned with a significant decrease in lymph nodes, without new symptoms and without fistulous tracts. |
A 32 years old female who was diagnosed in the Department of Orthodontics in the University of Alabama at Birmingham. On clinical examination, the subject had a convex profile on a lateral view and occlusal cant. She had Class III molar relationship on the right side and Class I on the left side. An anterior open bite involving the lateral incisor on the left side and a retained primary canine was noted. Posterior open bite involving the right first molar was also documented. She had a lower midline shift to the left side in centric occlusion due to a functional shift. The subject had also spacing in the upper and lower arch [].\nPanoramic radiograph was taken and no pathological abnormalities were detected. The subject had four missing third molars. She showed retained primary left upper canine with fully resorbed root and presence of permanent successor [].\nCephalometric findings showed maxillary and mandibular excess. ANB was − 0.5 which showed Class III tendency. The subject had proclined upper incisors, retroclined lower incisors, and steep mandibular plane [].\nNonextraction of permanent teeth and nonsurgical approach were recommended for the subject. The retained primary left upper lateral was extracted and nonsurgical extrusion of the permanent successor was done. Asymmetric elastics approach was used to treat the midline deviation. Class III elastic on the right side and Class II elastics on the left side were used with cross elastics anteriorly.\nKodak 9500 cone beam 3D system device (Carestream, Atlanta, GA) was used to take two CBCTS in two times frames: Initial (T1) and progress (T2). The radiation dose was 90 kV in a pulsed mode and frequency of 140 kHz. The tube focal spot was 0.7 mm with the sensor of a flat panel detector. Voxel size was (300, 300, 300) µm for the full field 3D image taken. The exposure time was 24 seconds, X-ray pulse time was 30 ms, and the image reconstruction took 2 min and 30 s.\nIn this case report, facial asymmetry was defined as the measurement resulted from subtracting the right side of an anatomical structure from its left counterpart. In order to do this, a 3D facial analysis using 3D imaging software using InVivo Dental 5.2.3 (Anatomage Company, San Jose, CA, USA) software was used. The facial analysis was composed of five parts: Upper face asymmetry analysis, maxillary analysis, maxillary cant analysis, mandibular cant analysis, and mandibular asymmetry analysis. The five components of the facial analysis were applied in the initial CBCT (T1) for diagnosis. Maxillary analysis, maxillary cant analysis, and mandibular cant analysis were applied to measure the progress of the orthodontics treatment (T2). A coordinate system was set for the MSP, Frankfort horizontal plane (FHP), and frontal plane (FP). Nasion, sella, and anterior nasal spine were chosen as landmarks for the MSP because it was found that the nasion and anterior nasal spine falls almost over the MSP.[] FHP was connecting portion right, orbitale right, and orbitale left. FP was perpendicular on the MSP and FHP.\nTwenty-two linear measurements bilaterally and sixteen angular criteria were used to analyze the facial structures using different anthropometric landmarks []. The upper one-third of the face will be analyzed using the following landmarks bilaterally: Lateral cranium, lateral scalp, lateral zygoma, exocanthus, endocanthus, and enocanthus. This will segment the superior one-third of the face into (1) cranium width, (2) outer orbital width, (3) inner orbital width, and (4) zygomatic width. This will be applied on T1 CBCT [].\nThe maxillary analysis will have the following landmarks plotted: Lateral maxillary alveolus and mesiobuccal cusp of maxillary first molar. These will segment the maxillary analysis into (1) horizontal position of the first molar, (2) vertical position of the first molar, and (3) maxillary width. The distance between the mesiobuccal cusp of the first molar and the two planes mid-sagittal and FHP will be measured, respectively []. This will give an indication of the position of the first molar in the vertical and horizontal access. The distance between the lateral part of the maxillary alveolus and the mid-sagittal will give the maxillary width.\nThe maxillary cant analysis will be composed of (1) maxillary alveolar ridge cant which is an angle between a line connecting the lateral right maxillary alveolar ridge and its counterpart with the MSP (2) maxillary dental cant which is an angle between a line connecting the mesiobuccal cusp of the first molar on the right side and its counterpart with the MSP []. This will be applied on T1 and T2 frames [Figures –].\nThe mandibular arch of the face will be analyzed using the following landmarks: Condylion_R, Condylion_L, Gonion_R, Gonion_L, and Menton. This will segment the mandible into four parts: (1) ramus length on the right side, (2) ramus length on the left side, (3) body of the mandible on the right side, and (4) body of the mandible on the left side. Linear and angular measurement between each part of the mandible and three planes (mid-sagittal, FHP, and FP) will be acquired in order to compare each line from a 3D aspect. Menton deviation from the MSP will be measured []. This will be applied on T1 frame [Figures and ].\nThe mandibular cant analysis will be analyzed using lateral mandibular alveolus and mesiobuccal cusp of mandibular first molar. This divided the mandibular cant analysis into (1) mandibular alveolar ridge cant which is an angle measured by a line connecting the lateral right mandibular alveolar ridge and its counterpart with the MSP. (2) Mandibular dental cant which is an angle between a line connecting the mesiobuccal cusp of the first molar on the right side and its counterpart with the MSP. This will be applied on T1 and T2 frames [Figures and ]. |
A 56-yr-old man collapsed without chest pain after playing badminton on January 29, 2007. His friend witnessed the collapse and promptly performed cardiopulmonary resuscitation. The Emergency Rescue Service was called, and on arrival, after determining the presence of ventricular fibrillation, applied an automatic external defibrillator.\nDefibrillation shock was delivered twice and sinus rhythm recovered. After admission, his mental state was markedly disoriented with retrograde amnesia. His speech was appropriate but unable to perform 7 serial subtractions. Physical examination was unremarkable. A profile of routine chemistry did not reveal any specific abnormalities except for slightly increased cardiac enzymes (CK: 1289 IU/L, CK-MB: 13.6 ng/mL) which might be due to repeated cardioversions. The electrocardiogram revealed normal sinus rhythm with left ventricular hypertrophy by voltage criteria and small Q waves in inferior leads. There was no ventricular preexcitation or QT prolongation. He had been on medication (Losartan, Thiazide, Glimepiride, Metformin) for hypertension and diabetes over the last 5 yr. According to detailed history, the patient have had exertion angina in the last 2 yr but did not seek for medical advice. The prolonged telemetry monitoring revealed an episode of non-sustained monomorphic ventricular tachycardia (8 beats, right bundle branch block morphology, cycle length 340 ms). Echocardiography and coronary angiogram were performed to evaluate the structural heart disease. An echocardiography revealed inferior wall akinesia and an ejection fraction of 42%. A coronary angiography demonstrated a chronic total occlusion of the proximal right coronary artery and patent left coronary artery without stenosis. Percutaneous coronary intervention with stent was performed in the right coronary artery without complications. During an electrophysiologic study, programmed electrical stimulation with single or double ventricular extrastimuli reproducibly induced polymorphic ventricular tachycardia with hemodynamic compromise. In view of the inducible ventricular tachycardia observed during electrophysiologic study and aborted sudden cardiac death without an acute coronary event, we decided to implant a single chamber ICD for the secondary prevention of sudden cardiac death. However, while the ICD was being implanted, a left subclavian venogram failed to visualize the left subclavian vein, and the retrograde femoral catheter could not be advanced to the vein (), which was attributed to likely prolonged indwelling of the left subclavian sheath for venous access. Accordingly, the right subclavian vein was punctured, and a defibrillating ventricular dual coil lead for an ICD (Vitality VR 1870, Guidant, St. Paul, MN, USA) was inserted. Lead measurements revealed ventricular sensing at 9.0 mV, a pacing threshold of 0.8 V/0.5 ms, and an impedance of 420 Ohms. Subsequently, a defibrillation test using a biphasic waveform was conducted in the dual coil system configuration (conventional configuration; RV-→SVC++CAN+).\nInitially, an active Can was positioned at the right pectoral site. A DFT test of the active Can that revealed high energy (31J) to terminate the induced ventricular fibrillation. Polarity reversal, different shock configuration (RV-→CAN+) and lead repositioning were not helpful at reducing DFT (31J).\nTherefore, after fixation of the lead sleeve at left pectoralis fascia, the ICD lead was diverted from the right side area to the ICD in the left pectoral area via tunnel made by tunneling tool (Medtronic, Minneapolis, MN) over the sternum. Adaptor of lead extension was not necessary because lead length was long enough. A repeat defibrillation test revealed a lower DFT of 11J, and the active Can was successfully implanted in a pocket on the left pectoral side without complication (). During 2 yr follow up after ICD implantation, patients had a no episode of tachyarrhythmia and recent lead measurements revealed ventricular sensing at 10.2 mV, a pacing threshold of 0.8 V/0.5 ms, and an impedance of 439 Ohms. |
A 45 year old female with the diagnosis of 10 years' progression of definite () bilateral type I Menière's Disease, according to the Lopez-Escamez classification (), with recurrent vertigo and hearing loss attacks symptomatically controlled with sulpiride (50 mg) on demand, was referred to the cochlear implants unit of a tertiary hospital center as a possible candidate for cochlear implantation, because of profound hearing loss (1997 BIAP) in the left ear and fluctuating moderate-severe (1997 BIAP) hearing loss in the right ear. The most recent vertigo episode occurred 10 days prior to her visit to the cochlear implant unit. No visual impairment or other alterations were found on otoneurotologic examination; normal ocular movements, normal smooth pursuit, and saccadic movement, absence of ocular misalignment on skew deviation test and no spontaneous nystagmus were also found, despite the recency of the last vertigo attack. Other neurological examinations and standard cerebral MRI were also normal for this patient.\nIn addition to the same tests as for case I, because of the bilateral MD diagnosis, the patient had received 3 months prior a 3 Tesla MRI examination of the inner ear using the HYDROPS MRI sequence (): this sequence is based on the digital subtraction of images produced by the different time diffusion of gadolinium along the inner ear fluids. This MRI showed bilateral cochlear and vestibular endolymphatic hydrops with left side hydrops predominating, as can be observed in Figure .\nThe instrumental vestibular testing was performed by the same senior neurotologist using a vHIT ICS Impulse™ USB hardware version with software version 4.1: this device was a different unit from the device used in case I. Figure shows enhanced vHIT eye velocity responses for both sides, with an aVOR gain value of 1.14 on right horizontal canal function test and 1.05 for left side. In this case, the vHIT calibration was repeated four times with similar enhanced gain values obtained, and a fifth calibration was done with the default system calibration parameters, also yielding similar aVOR gain values. The VVOR test was also performed on this patient using the vHIT ICS Impulse™ device, finding an enhanced eye velocity response during the VVOR test with a measured VVOR gain () value of 1.39 for left side and 1.35 for right side.\nFor both cases presented in this paper, the patients gave written consent to publish the results obtained from their clinical examinations and instrumental tests. |
To our knowledge, this is the first reported case of an index finger MCP joint dislocation surgically treated by a lateral approach.\nThe authors describe a case of a 16-year-old male who suffered a fall onto his outstretched right hand during a soccer game. The patient presented to the ER with pain and deformity of the index finger MCP joint. Volarly, the prominence of the second metacarpal head was evident ().\nRadiographs confirmed a dorsal index finger MCP joint dislocation and showed a small dorsal osteochondral fragment (Figures and ).\nAfter multiple unsuccessful reduction attempts under ring block by different physicians, the patient was referred to surgery.\nUnder general anesthesia, a lateral surgical approach () was performed on the MCP joint. A straight longitudinal incision was made over the lateral aspect of the MCP joint; the volar neurovascular bundle and the dorsal branch of the digital nerve were identified and retracted with Farabeufs.\nInterposition of the volar plate () preventing the reduction was observed. Applying gentle traction and flexion, the MCP joint was reduced, and proximal volar plate reinsertion with a 4-0 Vicryl suture was performed.\nThe posterior joint capsule was identified and split longitudinally, above the collateral ligament. Once adequately exposed, a small osteochondral fragment was found (). Reduction and retrograde fixation of the osteochondral fragment with a 1.7 mm screw were performed, burying the screw head in the cartilage.\nThe joint capsule, subcutaneous layer, and skin were closed using appropriate sutures. Reduction was confirmed by intraoperative fluoroscopy.\nThe patient was placed in a volar splint with approximately 45° of flexion and discharged on postoperative day zero without any complications.\nImmobilization was removed by week 3. Radiographic control revealed joint congruence, and the patient was encouraged to actively mobilize the finger.\nAt week 6, the fracture was consolidated (Figures and ). The joint was painless and presented slight stiffness (ROM 0-70°). The patient could return to competition with protective syndactyly.\nOne year postoperative, there was no pain, growth disturbance, or joint stiffness, with full ROM of the index finger. |
A 48-year-old Caucasian woman presented to a neurology clinic for progressive right upper limb weakness that had started 3 months earlier. Her major complaints were frequently dropping things from her hands, worsening handwriting, difficulty holding a spoon, and inability to do her job as a desk clerk. She also noticed some twitching in the muscles of her right upper and lower extremities. She denied numbness or tingling in the extremities and weakness of the bulbar muscles (dysphagia, dysarthria, and shortness of breath). She denied any neck pain; trauma; and constitutional symptoms such as fever, night sweats, and weight loss. Her past medical history included rosacea and dry eyes. She had undergone a cesarean section and plantar fasciotomy in the past. Her father had died as a result of heart failure. She reported no neurodegenerative diseases, neuromuscular diseases, or sudden deaths in other family members. She had no history of smoking or alcohol or illicit drug abuse and no known drug allergies.\nOn examination, she did not have any cognitive dysfunction, and the result of her cranial nerve examination was normal. Her motor examination revealed decreased strength in both proximal and distal muscle groups, predominantly in the intrinsic hand muscles with wasting and in the shoulder abductors. Fasciculations were noted in the right upper and lower extremities in various muscle groups. Her deep tendon reflexes were brisk with positive jaw jerk, Hoffman’s sign on the left, and bilateral ankle clonus. Her plantar reflexes were mute bilaterally. Her sensory system, coordination, and gait were unremarkable. Magnetic resonance imaging of the brain and the cervical and thoracic spine was unremarkable. The result of her autoimmune and paraneoplastic workup was negative, although the result of her malignancy workup was positive for papillary thyroid cancer, which was subsequently resected. An electromyogram with nerve conduction studies showed denervation in three body regions, consistent with the diagnosis of ALS. During the next 3 months, her pulmonary function tests showed a decrement by more than 20% in measurements of predicted forced vital capacity and forced expiratory volume in 1 second. One month later, she had an acute episode of shortness of breath and was diagnosed with pulmonary embolism, for which she was started on anticoagulation.\nNine months into her diagnosis of ALS, she presented to the emergency department of our hospital with acute on chronic shortness of breath and was hypoxic and hypercapnic with a negative inspiratory pressure of −10 mmHg, requiring bilevel positive airway pressure ventilation (BiPAP). In further evaluation for cardiopulmonary causes, her chest x-ray was unremarkable; a computed tomographic angiogram of the chest ruled out pulmonary embolism; and an electrocardiogram showed sinus rhythm with J-point elevation, saddle-shaped ST segment elevation predominantly in V1 and V2 with no significant QTc prolongation and negative troponinemia (Fig. ). Eventually, she became lethargic with a worsening Glasgow Coma Scale score and increased work of breathing, and she was unable to protect her airway, requiring intubation. She was continued on BiPAP until induction for intubation. Etomidate and succinylcholine were administered, and within 1 minute into induction, she developed pulseless ventricular tachycardia and ventricular fibrillation. Advanced cardiac life support was initiated with defibrillation, three rounds of epinephrine, and a loading dose of amiodarone prior to return of spontaneous circulation. After some time, she became responsive and started following simple commands, and a decision was made not to use a hypothermia protocol. She was admitted to the cardiac intensive care unit for close monitoring. Her echocardiogram showed that her left ventricular ejection fraction was 70%, and asymmetric hypertrophy of basal segments of the left ventricle was noted. She was diagnosed with type 2 Brugada syndrome by cardiology services as the cause of her ventricular tachyarrhythmia, and she was started on quinidine. The results of her genetic panel for Brugada syndrome was negative. There were no recurrences of ventricular arrhythmias during the rest of her hospitalization. She declined automatic implantable cardioverter-defibrillator placement and was later discharged to home hospice care with a tracheostomy. |
A 37-year-old, Chinese man had a 3-month history of intermittent low grade fever and a painful, non-healing ulcer in the left gluteal region. The left gluteal ulcer initially started as a pimple-like swelling 9 years ago. Later, it ruptured and slowly increased in size. The patient had sought treatment from numerous General Practitioners, who only prescribed oral antibiotics and daily dressing. Three months prior to admission, the ulcer begun to enlarge and was increasingly painful. No symptom suggestive of tuberculosis, however, was present. There was no history of discharging sinus. There was also no associated numbness or weakness in the left lower limb. He denied history of weight loss or high risk behaviour, such as, drug abuse or sexual promiscuity. He was not a known diabetic or immunologically compromised. He was also not taking any immune-modulating medication. His past medical history was completely uneventful.\nPhysical examination revealed a mildly pale, febrile young male with a temperature of 37.5 degree Celsius. Examination of the gluteal region revealed a 4 x 4 cm ulcerated, infected, bad-smelling lesion in the left gluteal fold. The ulcer had a fungating edge with a fixed base and its depth was about 4-5 cm. Mild tenderness was noted on palpation. No neurological deficit was detected in the left lower limb. Palpable left superficial inguinal lymphadenopathy was found consistent with secondary involvement of inguinal lymph nodes. The rest of the systemic examination showed no abnormal findings. Laboratory evaluation revealed mild anaemia with haemoglobin count of 10.4 g/L and a mildly raised white blood cell count of 11,900/ul. The erythrocyte sedimentation rate (ESR) was not elevated. Serology confirmed he was negative for retrovirus as well as Hepatitis B and C viruses. No bacterial cultures were performed from the ulcer here.\nPelvic radiograph () demonstrated area of marked sclerosis with wide zone of transition and thickening of the left ischial tuberosity. Cortical irregularity was noted, but no calcification was seen. There were also associated lucencies in the adjacent soft tissue. The appearances were suggestive of an aggressive lesion, and the differential diagnoses included chronic osteomyelitis of the left ischial tuberosity with abscess formation and malignancy of the bone. Contrast-enhanced axial CT scan of the thorax, abdomen, and pelvis was performed. This showed a heterogeneously enhancing rim of soft tissue mass in the left gluteus medius and maximus muscles extending inferiorly down to the posterior aspect of the left mid thigh measuring 4.5 x 5.3 x 10 cm (). Air pockets and bony fragments were noted within the mass. There were sclerosis and cortical irregularity in the adjacent left ischial tuberosity. Left superficial inguinal lymphadenopathy was noted.\nIncisional biopsy of the lesion was performed, and histopathological examination confirmed an invasive, well-differentiated squamous cell carcinoma surrounded by acute inflammation. Wide surgical excision and debridement with distal flap reconstruction and split-thickness skin graft was performed. This confirmed invasion into the ischial tuberosity (which was osteotomised), cartilage, ligaments, and fibromuscular tissue as well as metastasis to the inguinal nodes. The hamstring muscle was not involved and the left sciatic nerve was preserved. The anus and rectum appeared free of tumour involvement. Post-operative recovery was uneventful. Post-operative radiotherapy was given to the primary and draining nodal sites, with a generous margin, because of the ill-defined extension of the tumour. So far, follow-up has not demonstrated any recurrent disease. |
A previously healthy, 52-year-old Caucasian man presented to his family physician a week after having a tonic-clonic seizure. A magnetic resonance imaging (MRI) scan showed a 10 cm left frontal tumor, which was confirmed as an atypical meningioma following craniotomy and resection (Figure ).\nPostoperatively, he took 400 mg of phenytoin PO once a day. He had no seizures postoperatively or afterward. The patient uneventfully received 60 Gy of adjuvant radiation therapy to the postoperative bed in 30 fractions. Three months after the resection of the tumor, the patient began a trial of phenytoin but nine days later, he developed symptoms consistent with a generalized seizure. He resumed his daily phenytoin prophylaxis with good effect.\nTwo months later, he complained of blood in the stool and after an evaluation was diagnosed with a locally advanced nonmetastatic adenocarcinoma of the low rectum (Figure ). A curative-intent dose of 50.4 Gy in 28 fractions of neoadjuvant radiation therapy was prescribed, with 2000 mg PO BID of concurrent radiosensitizing capecitabine []. After 20 of the planned 28 fractions, he began to feel unwell and experienced new, right-sided upper and lower limb dysfunction and an unsteady gait. A contrast-enhanced computed tomography (CT) scan of the brain showed no suspicious findings but his phenytoin level was dramatically elevated at 138 µmol/L, compared to 49 µmol/L just prior to neoadjuvant therapy (normal range: 40-80 µmol/L). His albumin level from a few weeks prior to these symptoms had also been normal at 39 g/L (normal range: 34-46 g/L), and he was taking no other medications other than an occasional stimulant laxative. Capecitabine was discontinued, and the patient was treated with charcoal and admitted for observation. Phenytoin was temporarily discontinued and then reintroduced at the previous dose of 400 mg PO per day once levels began to normalize. His symptoms quickly resolved and he showed no further toxicity.\nHe resumed radiation therapy a few days later without concurrent capecitabine. It was believed that he had developed phenytoin toxicity secondary to impaired clearance as a result of his capecitabine. His phenytoin levels were monitored during the following weeks and his phenytoin dose was bridged with lacosamide and titrated down gradually and then discontinued, with no further symptoms of toxicity. The patient remained on 200 mg PO per day of lacosamide. He underwent a surgical resection with clear margins followed by adjuvant capecitabine and showed no signs of a recurrence of rectal adenocarcinoma thereafter. Three years later, the patient passed away from recurrent meningioma. |
A 61-year-old female patient was diagnosed in August 2018 with neck metastasis of unknown primary origin. She underwent left radical neck dissection and was staged as pT0N2b squamous cell carcinoma (SCC) with sarcomatoid differentiation (according to American Joint Committee on Cancer (AJCC) 8th edition). She was treated by adjuvant concomitant cisplatin and radiation therapy (64 Gy: left neck level 1b-2, 60 Gy: tumor bed and left neck level 3, 57 Gy: left neck level 4-5, 54 Gy: right neck level 2-3-4 in 32 fractions with 6 MV photons using the intensity-modulated radiation therapy (IMRT) technique). During follow-up in December 2018, a recurrence was detected in the left parotid region outside the RT field. She underwent left parotidectomy, and the surgical margins were positive. She received adjuvant RT to the left parotid bed to a total dose of 40 Gy in 5 fractions with 6 MV photons using the IMRT technique. In May 2019, MRI showed an enlarged lymph node at the right neck level 1b, which was not previously irradiated, and the biopsy confirmed metastatic disease. Thereupon, 60 Gy RT was applied in 30 fractions with 6 MV photons using the volumetric arc treatment (VMAT) technique to the right neck level 1b with concomitant carboplatin. While the patient was followed up without medication, in January 2020, a 2.5 cm diameter erythematous soft tissue lesion developed on the left neck lateral skin. Excisional biopsy was performed, and pathologic examination revealed sarcomatoid carcinoma metastasis with a 0.6 cm surgical margin. 40 Gy RT in 5 fractions with 6 MV photons using the VMAT technique was performed to the tumor bed at the left neck skin. After three months of the last RT session, she was started on nivolumab due to the metastatic disease at the retrosternal lymph node and right lung. Four weeks later, erythematous plaque-like lesions were formed starting from the bilateral temporomandibular region and spreading to the anterior chest, which corresponded to the previously irradiated area (Figures , ). A biopsy was performed with the differential diagnosis of skin metastases which revealed subacute spongiotic dermatitis. The lesions completely regressed in two weeks using topical steroids (hydrocortisone butyrate %0.1, twice daily) and antihistamine tablets (cetirizine dihydrochloride, once daily). Thus, nivolumab treatment was not interrupted, and no reaction was observed during or after the next cycle. |
A 56-year-old female patient presented with neck and left shoulder pain radiating to the left upper extremity. However, no objective loss of sensory or motor function was observed. Her pain had been aggravated for 10 days. Her visual analogue scale (VAS) score was 7 of 10. Although conservative treatments, including as resting, traction, non-steroidal anti-inflammatory drug and muscle relaxant were properly done for two weeks, her pain was not improved. The neurosurgeon requested a selective left C6 nerve root block. A cervical spine plain film radiograph showed degenerative cervical spondylosis (). The radiologist reported that the cervical magnetic resonance image (MRI) revealed a left-sided foraminal stenosis at the C5-6 level. Before performing the procedure, the author re-examined the MRI meticulously. Although the radiologist did not mention the VA, the author detected a tortuous anatomical variation in the VA at the left C5-6 level on the MRI (). The author ordered a CT angiogram to show the anatomical variations in the VA more precisely. The CT angiogram clearly showed bilateral tortuous anatomical variations in the VA (). In particular, the 3D reconstruction image showed a unilateral atypical entrance into the foramen transversarium (FT) of the fifth cervical vertebra and a hypoplastic abnormality of the right VA (). The diameters of the left and right VA were 4.86 mm and 2.47 mm at the lower endplate level of C5, respectively. The diameter of the left VA was 2.39 mm greater than that of the right at the C5 lower endplate. The maximum diameters of the tortuous component of the left and right VA were 9.80 mm and 6.64 mm, respectively. The maximum diameter of the tortuous component of the left VA was 3.16 mm greater than that of the right. The left VA entered the FT of the sixth cervical vertebra, whereas right entered the fifth. To minimize adverse events, the procedure was performed with dexamethasone (non-particulated steroid) and under CT guidance. The patient was placed in the supine position on a CT table with her head tilted slightly to the right. Before scanning, a wire was placed on the lateral side of her neck to use as a marker. The patient was scanned to identify a suitable approach point for the 23 G spinal needle. A path that avoided contact with the periosteum and deep cervical plexus was chosen to minimize patient discomfort. The scanning level for the CT fluoroscopy (CTF) image was selected and the target lesion was scanned to a thickness of 1 mm from the middle of the C5 body to the middle of the C6 body. The skin entry point and target point for the spinal needle was determined on the CT scan view () and marked on the neck. Before the CSTE was performed, 2% lidocaine was aseptically infiltrated into the skin and subcutaneous tissue. The needle was then advanced toward the target point using intermittent CTF guidance. The needle was advanced slowly into the left C5-6 foraminal area while avoiding the large tortuous VA. It was confirmed that the 23 G spinal needle was located exactly within the left-sided C5-6 foraminal area using 0.3 ml of radio-contrast dye (). After confirmation of appropriate needle placement, the author injected a mixture of 0.5% lidocaine 2 ml and dexamethasone 5 mg. Pain was relieved immediately after completion of the procedure and two weeks later, VAS score was decreased to 1 or 0 of 10. The patient's symptoms were relieved almost completely and did not recur in the following 3 months. |
A 68-year-old woman was referred to our department after gastroscopy, which showed a mass in the stomach. She had undergone total abdominal hysterectomy with bilateral salpingo-oophorectomy because of an ovarian serous carcinoma (stage IIIa) nine years ago. On routine workup, gastroscopy showed a round mass, approximately 3 cm in diameter, in the upper body on the greater curvature side of the stomach ().\nA biopsy was performed and histologic examination indicated a poorly differentiated adenocarcinoma. Tumor markers including carcinoembryonic antigen and carbohydrate antigen 19-9 were within the normal ranges. Contrast-enhanced computed tomography showed focal, eccentric, enhancing wall thickening in the greater curvature of the stomach body and no significantly enlarged perigastric lymph nodes (). We diagnosed the patient with gastric adenocarcinoma and performed a laparoscopic-assisted total gastrectomy with D2 lymphadenectomy. No ascites or peritoneal lesion was observed. Reconstruction was performed with a Roux-en-Y anastomosis.\nThe resected specimen contained a polypoid tumor with central ulceration 3 cm in diameter (). Tumor cells had spread through the submucosal layer of the stomach with clear resection margins (). None of the 44 resected lymph nodes were positive for metastasis, but lymphatic tumor emboli were detected by immunostaining with CD-31 and D2-40 antibodies (). The tumor cells showed atypical hyperchromatic nuclei () and accompanying melanin distribution within the tumor nest (). Immunohistochemical examination revealed a positive reaction to HMB-45 antibodies and S-100 proteins and a negative reaction to cytokeratin antibodies ().\nBased on these findings, we diagnosed the patient with malignant melanoma. In the pathologic examination, the depth of tumor involvement was to the submucosal level and no metastasis showed in 44 lymph nodes. Therefore, the final pathologic tumor's TNM stage was pT1bN0M0. Postoperatively, further examinations were performed to identify the primary tumor or other metastatic foci. Ophthalmologic, dermatologic, and oral examinations showed negative findings. Positron emission tomography/computed tomography was performed, but no hot spots were detected (). These results indicated a final diagnosis of primary malignant melanoma of the stomach. After total gastrectomy, a follow-up study was performed with abdominal computed tomography scans at 3, 6, 12, 18, and 24 months (5 times) over the course of 2 years and there was no recurrence. The TNM tumor stage was early (pT1bN0M0); therefore, we did not perform any adjuvant chemotherapy, but the patient has taken oral immunotherapeutic medication for 4 months. |
A 3-year-old south Indian girl was reported to the department of pediatric dentistry with a complaint of pain in lower right and left back tooth region since 1 week. Pain was spontaneous, intermittent, non-radiating, aggravated during night and subsided on medication. The medical history revealed that child was born to non-consanguineous parents with non-contributory health history.\nIntraoral examination showed complete set of primary dentition with mesial step molar relation and carious lesions of varying severity in relation to left maxillary first and all four mandibular primary molars.() An intraoral periapical radiograph of the symptomatic teeth revealed caries involving the pulp in relation to left mandibular second as well as right mandibular first and second molars with an abnormality in tooth anatomy showing abnormally extended pulp chamber with no constriction at the CEJ , large root canal ending at the apex resembling single or pyramidal root for all the molars suggestive of taurodontism. ()\nThe panoramic radiograph revealed the involvement of maxillary primary molars with similar pulp chamber anatomy of the mandibular molars and no other obvious anomalies were noticed. ()\nFrom these radiographic findings, it was diagnosed to be a cuneiform type of taurodontism according to classification given by Shaw in 1928. [] A diagnosis of dental caries was made for all the five involved teeth based on clinical and radiographic findings with chronic irreversible pulpitis in relation to mandibular left second as well as both right molars. As there was no apical constriction proceeding with conventional obturation technique posed a dilemma. Hence, three teeth were treated by multivisit pulpectomy procedure followed by placing a small piece of a synthetic collagen material at the level of the apex (CollaCote®; Zimmer Dental, Carlsbad, USA), and using hand plugger as a barrier. Final obturation was done with a combination of iodoform and calcium hydroxide (Metapex® Meta Dental New York; Elmhurst, USA) paste and a semi- permanent restoration with stainless steel crowns. As left maxillary first primary molar was having multisurface caries, it was restored with stainless steel crown and left mandibular first primary molar with composite restoration. ()\nSuspecting a familial inheritance, panoramic radiograph was advised for the parents, which revealed no obvious anomalies. |
A previously healthy 39-year-old woman was referred to our hospital because of a cystic lesion in the liver demonstrated by abdominal ultrasonography (US). Laboratory studies, including liver function tests, and tumor markers were also within the normal limits. Serological markers for hepatitis B or C viral infection were undetectable. Abdominal US revealed a well demarcated, heterogeneously low-echoic mass 170 mm in diameter in right lobe of the liver. Abdominal computed tomography (CT) during hepatic arteriography (CTHA) revealed early ring enhancement in the peripheral area in the arterial phase and slight internal heterogeneous enhancement in the delayed phase (Figures and ). Magnetic resonance imaging (MRI) showed that the tumor had low signal intensity on T1-weighted images and some foci of high signal intensity on T2-weighted images. Gadolinium ethoxybenzyl (Gd-EOB) MRI revealed no uptake in the corresponding area (Figures , , and ). Abdominal angiography demonstrated a large avascular region in the liver corresponding to the tumor, although no typical features of cavernous hemangioma were evident (). 18-Fluorodeoxyglucose positron emission tomography (FDG-PET) revealed no abnormal FDG uptake. With these radiological findings, malignant liver tumor could not be excluded, such as biliary cystadenocarcinoma, cholangiocarcinoma, mesenchymal tumors, and hepatocellular carcinoma associated with cystic formation.\nThe patient underwent posterior sectionectomy. Intraoperative examination revealed a relatively soft dark red tumor (); the resected specimen weighed 1.1 kg and measured as 170×100×80 mm. The cut surface of the tumor revealed a white, solid, and cystic mass that was elastic, soft, and homogeneous with a yellowish area considered to be myxoid degeneration (). Histological examination showed that the tumor mostly consisted of sclerotic area and cavernous hemangioma area is partly observed (). Sclerotic area presents diffuse fibrosis () and the typical histology of cavernous hemangioma was confirmed in some parts. In addition, marked increase and dilation of medium sized veins with cavernous form were frequently noted in the surrounding areas of tumor (). The increased and dilated veins show positivity of CD31 immunostaining being a marker of endothelium (). The pathologic features were consistent with sclerosing hemangioma. The postoperative course was uneventful, and the patient was discharged on postoperative day 10. |
A 29 year old Sinhalese Sri Lankan female, who was apparently well, presented with incidentally detected high blood pressure. She is a mother of two children, but had no history of pregnancy induced hypertension. She denied any family history of hypertension. She also complained of loss of appetite and subjective weight loss during the past few months.\nHer physical examination revealed a blood pressure of 180/120 mmHg on two separate occasions, and was equal in both arms. Her pulse rate was 88 beats per minute, with no radio-radial or radio-femoral delay. There were no renal masses, and no carotid, renal or femoral artery bruits. Her cardiovascular and central nervous system examination was unremarkable, and had no evidence of retinopathy on fundoscopy. She had no peripheral stigmata of atherosclerosis, or endocrinopathies.\nHer renal function tests, serum electrolytes, urine full report, full blood count, erythrocyte sedimentation rate, and liver function tests were all normal. Her electrocardiogram and transthoracic echocardiogram were unremarkable.\nAs she was a recent onset young hypertensive, she was investigated with ultrasound scan of the abdomen which showed non visualization of the left kidney. Therefore, computed tomography angiogram (CTA) and diethylene triamine penta acetic acid (DTPA) renogram was indicated. DTPA renogram showed a small left kidney which was suggested to be either congenital, or due to RAS, with normal perfusion and function of the right kidney. CTA revealed significant stenosis of the left main renal artery, which was suggested to be due to FMD, and an accessory renal artery supplying the lower pole of the left kidney was detected (Figs. , ). After evaluation of her renovascular hypertension, she was referred to a vascular surgeon and underwent left sided nephrectomy, and histology revealed features of FMD of left renal artery. She achieved full recovery with normalization of blood pressure following surgery, and is currently not on any antihypertensive medication. |
The patient was a 59-year-old Japanese man who visited the Division of Oral and Maxillofacial Surgery, Ebina General Hospital, in December 2012, with a chief complaint of a mass in the upper lip, which had increased in size over several years. A mobile, elastic, and relatively soft mass without tenderness was palpable in the upper lip. The mucosa in the upper lip covering the mass was normal. The patient had no relevant medical history. The mass in the upper lip region measured 1.0 × 1.0 cm (). The clinical diagnosis was suspected to be pleomorphic adenoma arising in the minor salivary gland of the upper lip.\nIn December 2012, tumor extirpation was performed under local anesthesia. There was no adhesion to the surrounding tissue and the postoperative course was uneventful with no recurrence. Macroscopically, the surgical specimen consisted of an ovoid, nodular mass that measured 1.0 × 1.0 × 0.8 cm. The mass was solid, lobulated, and brownish-yellow in appearance (). The tumor was surrounded by a thin capsule and did not involve any normal salivary gland tissue.\nHistopathologically, the tumor was encapsulated by fibrous connective tissue and demarcated from the surrounding tissues (). It consisted of monomorphic epithelial cells with a trabecular or tubular pattern (Figures and ). The solid nests were composed of almost uniform epithelial cells that were columnar or cuboidal in shape with scanty eosinophilic cytoplasm and round to ovoid nuclei. The stroma [] surrounding the epithelial tumor nests was composed of thin fibrous tissue and was well demarcated from the solid nests (Figures and ). Further analysis showed a glandular structure containing a mucinous substance that was positive in Periodic Acid-Schiff (PAS) staining () and deposition of abundant PAS-positive basal lamina material within and around the tumor nests ().\nImmunohistochemically, the inner tumor region of tubuloductal structures stained strongly positive for cytokeratin 7 (CK7) and the outer tumor cells were weakly positive for CK7 (). The outer tumor cells also showed cytoplasmic staining for α-smooth muscle actin (α-SMA) and CD10 and nuclear staining for p63 (Figures and ). The MIB-1 (Ki-67) labeling index was extremely low (). These results led to a diagnosis of BCA. |
Patient is a 73 year-old right hand dominant female who initially presented to the office complaining of atraumatic right shoulder pain with activity and limited range of motion of longstanding duration. On physical exam, she was found to have significantly limited active range of motion of the right shoulder and clinical signs of impingement. Radiographs at that time demonstrated superior escape of the humeral head with impingement of the greater tuberosity on the acromion and early acetabularization of the acromion (Fig. ). MRI findings were consistent with her x-ray and also demonstrated a lack of contiguous supraspinatus or infraspinatus tendon. At this juncture, the patient was diagnosed with rotator cuff arthropathy and elected to proceed with reverse total shoulder arthroplasty.\nThe patient was brought to the operating room and placed in the beach chair position. An incision was made from just lateral to the coracoid to the medial border of the proximal humeral shaft in line with the axillary fold. Subcutaneous tissue was dissected and the cephalic vein was identified. As the cephalic vein was mobilized and the clavipectoral fascia was incised, a discrete, branching, fascicular nerve was identified lateral and deep to the cephalic vein within the deltopectoral groove (Fig. ). The nerve was further dissected and traced both proximally and distally. Distally, the nerve and all branches were found to be diving into the anterior deltoid muscle. Proximally, it was found to run deep to the conjoined tendon, towards the brachial plexus. The nerve was freed from the deltoid muscle belly, allowing enough excursion to access the glenohumeral joint via a small deltoid window. The remainder of the operation concluded without complication and the wound was closed primarily (Fig. ). The patient was neurovascularly intact post-operatively with intact sensation in the axillary nerve distribution and able to fire her deltoid muscle. She healed without complications. At 4-month follow-up, she was doing well and able to actively abduct and forward flex her right shoulder to approximately 120 degrees (Fig. ). |
This was case report of a 23-year-old male patient who reported to the Department of Orthodontics with the chief complaint of flower like upper front teeth causing difficulty in speech. Clinical examination revealed a normal patient without any signs of syndromic features. The patient had full complement set of teeth, which had yellowish fluorosis stains []. Two mesiodens were present, one palatal and the other labial to 11 and 21 resulting in proclination and rotation of the maxillary central incisors [Figures and ]. The occlusion and arrangement of anterior teeth was disturbed both in maxillary and mandibular arch due to the presence of mesiodentes [Figures and ]. Patient also complained of difficulty in pronunciation of certain words due to proclination of his front teeth. Patient's self-esteem was low, which was affecting his social life. Soft-tissue profile was normal. Radiographic diagnosis included orthopantomograph (OPG), lateral cephalogram and intraoral periapical (IOPA) radiograph. No other supernumerary teeth or abnormalities were observed in the OPG examination []. The lateral cephalogram examination was explored to rule out the possibility of crossover of mesiodens from labial to palatal side and also the direction of eruption of mesiodens []. The IOPA radiographs revealed two separate twin mesiodens which are conical in shape. The root formation was complete in both teeth. The periodontal ligament shadow was clear around both teeth ruling out ankylosis []. Cephalometric findings indicate that it is a class I skeletal case with overjet of 6-7 mm, bimaxillary dentoalveolar protrusion with crowding of upper and lower teeth.\nAfter a detailed examination and case discussion it was decided to extract the two mesiodens as an initial step and align the maxillary permanent central incisors. Mesiodens were extracted under local anesthesia and uneventful. It was decided to evaluate the case after alignment to make a choice between first and second premolars for extraction to improve the esthetics as well as function []. |
First case
A 44-year-old man attended our outpatients’ clinic for persistent right knee swelling after direct trauma occurred three months earlier. MRI, performed in the emergency room, showed extensive edema of the trabecular bone of the medial femoral condyle, without fractures lines. The patient was discharged with a plaster immobilization of the knee, protected weight-bearing and oral NSAIDs. During the immobilization period, the patient reported an increase in pain and paresthesia of the knee. At the first follow-up evaluation, the patient presented significant joint effusion and limited knee range of motion (ROM) without signs of knee instability. On the anteromedial aspect of the knee, the skin was atrophic and discolored, cold and with a peculiar area of allodynia. Under the impression of post-traumatic CRPS, MRI scan was performed to detect possible trabecular fractures, showing a large area of edema of the medial femoral condyle which was diagnosed as an algodystrophic modification (Figure ).
All diagnostic tests were performed to rule out other possible causes. A combined treatment with low dose oral prednisone, gabapentin 300 mg three times a day, daily calcium carbonate 1250 mg-cholecalciferol 400 UI and intramuscular clodronate 200 mg daily for 15 days was prescribed. Additionally, a rehabilitation program was initiated. The treatment was discontinued after four months due to the lack of any symptom’s improvement. Afterward, the patient underwent TPT (20 mcg day) and calcium carbonate/cholecalciferol (1250 mg/400 UI) supplementation. After one month, the patient reported a marked subjective improvement in terms of both pain and joint mobility. On clinical examination, no joint swelling was noticed, with almost complete recovery of range of motion and disappearance of the cold skin overlying the medial femoral condyle. The patient started physical training and the knee function was completely recovered after one month. After three months, MRI showed almost complete resolution of the joint effusion and bone edema (F |
We examined a 32-year-old man who was diagnosed with bilateral anterior uveitis almost a year ago. His symptoms consisted of left eyeball pain and a decrease in vision in his left eye. He was treated with 1% topical prednisolone bilaterally three times a day by the referring ophthalmologist. He had experienced recurrent oral aphthous ulcers and papulopustular skin lesions for almost 10 years. He also had a history of lower extremity deep vein thrombosis. We found that the best-corrected visual acuity was 20/20 in the patient’s right eye and 20/40 in the left. A slit-lamp examination revealed trace cells in the anterior chamber and vitreous of the right eye, and in the left eye, tender scleral nodules with a purplish hue () and grade 2+ cells in the anterior chamber and vitreous.\nThere was posterior synechia at 1 o’clock in the right eye and at 2 o’clock in the left eye. The intraocular pressure was within the normal limits bilaterally. While the right fundus was normal, there was grade 3 vitreous haze, according to the University of Miami Scale for Photographic Grading of Vitreous Haza (), and occlusive vasculitis with intraretinal hemorrhages in the lower temporal quadrant of the left eye (). A fluorescein angiogram showed mild macular edema with findings related to occlusive vasculitis in the left eye (). The fluorescein angiogram of the right eye was normal. Optical coherence tomography indicated that both eyes had normal macular architecture.\nThe patient underwent a detailed assessment by an experienced rheumatologist, which involved a systemic examination and a laboratory work-up. Varicose veins were observed on his lower extremities. The patient was diagnosed with Behçet’s disease associated with nodular scleritis and occlusive vasculitis in the left eye. Subsequently, the patient was treated with azathioprine (150 mg) and oral prednisolone (60 mg) along with 1% topical prednisolone bilaterally six times a day. The nodular scleritis resolved rapidly within a few weeks () and, 3 weeks later, the patient’s visual acuity in the left eye was improved to 20/25, with a slight improvement in the appearance of fundus. |
A 14-year-old previously healthy girl presented 2 weeks after an uncomplicated laparoscopic appendectomy for non-perforated acute appendicitis in a regional hospital. The girl complained of gradually reducing urinary frequency to twice per day and prolonged hesitancy. The micturition stream was initially weak and slow before becoming interrupted. Straining did not produce stronger urinary stream. She had never suffered from urinary tract infections (UTIs) or constipation and opened her bowels daily.\nFollowing an episode of acute cystitis 2 months later, she completely lost her ability to void. She was put on indwelling Foley urinary catheter, and her cystitis was successfully treated with antibiotics. After every attempt to remove the urinary catheter, she had to be catheterized again with 300 to 1200 mL of urine volume registered. She noted loss of urge to urinate and felt only dull pain in suprapubic region and right iliac fossa on extreme bladder distention. The girl was kept on indwelling urethral urinary catheter and referred to a tertiary center to determine the etiology of her urinary retention.\nShe was examined with normal clinical findings and no obvious pathology on abdominal and pelvic ultrasound scan (USS). A pediatric neurologist found nothing abnormal, and magnetic resonance imaging (MRI) of the brain and spine, electromyography (EMG) of the lower extremity, somatosensory-evoked potentials (SEP) of tibial nerve, electroencephalogram (EEG), and lumbar puncture were with no pathology. On USS, the gynecologist described multiple follicular cysts on ovaries bilaterally and found no pathology explaining her urinary retention.\nOur pediatric urologist performed an examination under general anesthesia including a free calibration of the urethra up to 26F followed by normal findings on cystoscopy. Videourodynamic study (VUDS) showed an asensitive and hypotonic bladder. The bladder filling had to be stopped at 360 mL due to the patient's discomfort. Maximum intravesical pressure achieved 11 cmH\n2\nO. When pulling the urodynamic catheter out of the bladder manually, the maximum urethral pressure measured was 120 cmH\n2\nO. On vesicocystourethrogram (VCUG), there was no vesicoureteral reflux, a smooth bladder wall and closed bladder neck (\n).\nPsychologic and psychiatric evaluation identified no major problem. During the following 2 years of repeated admissions to several regional and university hospitals, many of the tests described above were repeated, including an MRI of brain and spine with identical conclusions.\nClean intermittent catheterization (CIC) was recommended to the patient. However, because of poor tolerance of CIC due to frequent macroscopic hematuria and pain, a suprapubic catheter was placed. Thereafter, she suffered recurrent symptomatic afebrile UTIs caused by multi-resistant bacterial strains, e.g.,\nKlebsiella\n,\nPseudomonas\n, or\nEscherichia\n. Finally, after 2 years, based on the history, symptoms, and urodynamic findings, she was diagnosed with Fowler's syndrome (FS).\nFor the treatment of FS, the patient was indicated for S3 neurostimulation. The implantation of two Medtronic S3 neurostimulators, type Interstim II, bilaterally in the upper gluteal region was performed under general anesthesia in two phases. The first phase was a transcutaneous implantation of the electrodes into S3 foramina and their connection to externalized neurostimulators. The first procedure took 30 minutes. As the patient restored her voiding completely back to normal when switching on the neurostimulators and experienced no side effects, she could undergo the second phase 4 weeks later—permanent subcutaneous implantation of the neurostimulators (\n). The second procedure took 15 minutes under general anesthesia.\nWith a transcutaneous remote control, she was able to modify the intensity of stimulating current to avoid any discomfort (\n). On the last follow-up, 4 months after the implantation, she voided four to six times per day with post-void residuals up to 50 mL on USS. Unfortunately, she suffered two prolonged episodes of burning on micturition even after the operation. On both occasions, she was diagnosed with acute cystitis by\nE. coli\n107 that was treated with antibiotics after sensitivity testing. |
A 57-year-old Greek housewife, who was 165 cm tall and weighed 65 kg, was admitted to our hospital after a closed injury of her right femur. She was a married mother with one 18- year-old daughter who was a non-smoker and who did not drink alcohol. She was suffering from an ipsilateral dysplastic hip []. As a child she had undergone an unsuccessful operation for a non-defined femoral osteotomy. She had no other significant medical history and received no medication except pain killers. Her right leg was fixed in a flexed and internally rotated deformity. She had been walking with great difficulty for more than 10 years, due to hip and knee stiffness with concomitant severe hip arthritis.\nShe had fallen in her house while walking. On clinical examination, the leg was in fixed flexion with adduction and internal rotation. X-rays revealed an isolated spiral mid-shaft fracture of the right femur (Figure ): type 0 according to the Winquist-Hansen classification [] or 32-A1 according to the AO-OTA classification [].\nWe performed a total hip arthroplasty via a posterolateral incision, using a long cementless Wagner stem [] and a porous tantalum monoblock acetabular cup to address both morbidities. The fixed deformity meant that straight forward hip dislocation was impossible and, therefore, the femoral neck had first to be osteotomised. The cup was placed in the anatomic position. Part of the native head was used as a morselised autograft at the true acetabular bed. The superolateral part of the head was used as a structural graft and secured with one screw. A cup was then inserted in a press fit manner, basing the initial stability on the periphery of the cup. After an additional small incision at the fracture site, the fracture was initially reduced anatomically. Reduction was secured with five cerclage wires and the stem was inserted under direct vision. The operation took 95 minutes. Tissues were sent for culture and histological analysis: the results were negative for tumor or infection, revealing that the fracture was not pathological. The patient received three doses of prophylactic antibiotic and was given low molecular weight heparin for six weeks. There was no leg length discrepancy post-operatively and no complications were recorded. She was mobilized with partial weight bearing the second postoperative day. Full weight bearing was allowed after three weeks, due to the concomitant presence of acetabular graft and diaphyseal cerclage wires. Three months postoperatively, the fracture had healed, the cup showed no signs of migration (Figures ,,,), there was a normal hip range of motion and patient was walking and free of symptoms. |
A 11-year-old boy presented with a three-month history of recurrent pain on the right knee with squatting, jumping and swimming breaststroke style. He did not report any trauma. In the last several years, he also complained about recurrent painless “clunks” on both knees especially during squatting. He used to practice martial arts, but he had to stop because of the pain. At physical examination both knees were not swollen and showed a complete and painless range of motion. A “clunk” was audible and it was visible and palpable during active and passive motion. McMurray test was negative. Left knee scored fair and right knee scored poor according to Ikeuchi scale []. X-rays showed squaring of the lateral femoral condyle and hypoplasia of the lateral tibial spine bilaterally (Figure ). MRI revealed bilateral incomplete discoid lateral meniscus with normal medial menisci and open physes (Figure ).\nTo relieve pain and restore function, arthroscopic partial resection of the lateral meniscus of the right knee was performed by the senior author, who is trained in pediatric knee arthroscopy, leaving a functional residual rim of 8 mm (Figure ). Physical therapy was begun immediately with isometric exercises, partial to total weight bearing was allowed as tolerated. The postoperative course was uneventful, after 2 months the patient returned to sports activities without any restriction and was followed-up clinically at 1, 2, 3, 6 and 12 months after surgery and then yearly.\nTwenty-nine months after surgery the patient returned to our clinic for a non-scheduled follow-up visit complaining about the same symptoms as before surgery, again only on the right knee. The onset of the symptoms was subtle and the patient did not report any trauma. In the meantime, he was going through a growth spurt and he had grown about 15 cm since the first surgery. MRI of the right knee showed an incomplete discoid lateral meniscus, with a different signal intensity compared with the original one, and open physis (Figure ). Arthroscopy of the right knee was done again by the same surgeon, confirming the discoid re-growth of the lateral meniscus, with a horizontal tear in the posterior horn. Partial resection was performed again into a functional residual rim of 8 mm (Figure ). After surgery the patient reported complete relief of symptoms. Physical therapy was begun immediately with the same protocol. The patient returned to sports activities without any restriction after 3 months.\nWe are still following-up the patient. At the time this manuscript was submitted he was 15 years old and 18 months had been elapsed since the second operation, and reportedly doing well.\nThere are several issues to consider in the case presented. Why did the lateral meniscus re-grow after surgery? Why did it return to its original incomplete discoid shape? Why was the patient symptomatic only on the right side?\nMenisci in children have an increased vascularity and cellularity that are progressively lost with aging. They can be found throughout the inner parts of the menisci in patients aged 10 to 11 years []. Furthermore, during the growth spurt there may be some influence of growth and maturation of all tissues, including menisci. To the best of our knowledge there are no reports in literature about the vascularization of the inner part of discoid lateral meniscus in children, but we assume it is similar to a normal meniscus of the same age. During the 29 months that elapsed between first and second arthroscopy, our patient had undergone significant physical growth which may have had an impact on meniscal regeneration. One may argue that the surgeon did not resect enough of the meniscus during first arthroscopy. Surgeons treating meniscal lesions in children are concerned about removing too much tissue, because this could promote degenerative osteoarthritis. On the other hand, the aim of surgery in discoid meniscus is to restore its crescent shape. In our patient, the resection performed during the first operation was judged adequate (Figure ) by the performing surgeon who is trained in pediatric knee arthroscopy, also demonstrated by the lack of symptoms referred by the patient during very active sport activities sustained in the period of 29 months between the first and the second arthroscopy.\nThe growth of the patient involves all structures of the knee and likely the lateral menisci. Normally the proportion and the shape of menisci are maintained from the fetal phase to adult age [], but in our patient after surgery the right lateral meniscus re-created the previous condition of an incomplete discoid shape, as this would be its natural shape.\nThere is still debate about the etiology of discoid meniscus. Smillie [] first proposed that this condition is the persistence of the normal stage during fetal development. However, most of the authors believe it is anomalous also during prenatal development, and arises through variant morphogenesis [,]. On the other hand, comparative data favor a phylogenetic origin because it represents, at least in some cases, the persistence of an ancestral character []. We believe that the re-growth of the discoid lateral meniscus in our patient favors the hypothesis of variant morphogenesis. In consideration of this condition, it is also often associated to other musculoskeletal abnormalities [].\nAlso the left lateral meniscus was discoid-shaped, but the patient did not report any complaints on that side. In fact, stable discoid menisci are often an incidental finding and commonly asymptomatic []. |
Subsets and Splits
No community queries yet
The top public SQL queries from the community will appear here once available.