text
stringlengths 746
31k
|
|---|
Patient is a 35-year-old male who was transferred to our hospital with a diagnosis of abdominal mass after presenting to an outside hospital (OSH) for abdominal pain. Patient reports a three-month history of abnormal bowel movements and a 3-week history of an enlarging, palpable abdominal mass. A CT scan obtained at the OSH revealed a 14 × 18 × 20 cm abdominopelvic mass causing ureteral obstruction and bilateral hydronephrosis, with no retroperitoneal lymphadenopathy (). Physical examination revealed the patient had an undescended left testicle, palpable in the left inguinal canal. Serum tumor markers for testicular cancer were obtained and beta HCG was found to be 10 mIU/ml (upper limit of normal), AFP was within normal limits, and LDH was elevated at >6x the upper limit of normal. The patient underwent a percutaneous biopsy of the mass by interventional radiology, and he subsequently underwent cystoscopy with bilateral ureteral stent placement and radical left inguinal orchiectomy. The percutaneous biopsy returned positive for metastatic seminoma (). The left radical orchiectomy specimen contained no tumor (). Ultrasound of the right testis revealed no lesions (). CT of the chest was negative for metastatic disease. The patient underwent 4 cycles of BEP. Two cycles were given without bleomycin, the first due to PFT abnormalities and the last due to rash development with bleomycin during the 3rd cycle. Follow-up imaging revealed decrease in mass size to 5 × 3.4 × 5 cm and an incidental pulmonary embolism (PE), which was asymptomatic. He was treated for the PE with therapeutic low molecular weight heparin for 3 months. PET imaging demonstrated pet avidity in the region of the residual mass, so the patient was taken back to the operating room, 5 months after completion of chemotherapy. Pathology from this specimen showed no viable tumor (Figures and ) but did contain testicular parenchymal tissue. Based on the totality of pathologic findings, we believe this to be a case of primary pure seminoma arising in a supernumerary testis rather than metastatic spread. The patient tolerated the procedure well and had no complications, pain, or evidence of recurrence at 1-month and 6-month follow-up appointments. |
Patient BC is a 61-year-old female with a past medical history of diabetes mellitus and hyperlipidemia who initially presented to the emergency room in June 2015 for periods of confusion and memory problems over the prior few weeks. Upon further questioning, the patient also reported having intermittent, diffuse headaches over the prior week. On initial examination, she was grossly neurologically intact and did not exhibit any noticeable short or long-term memory deficits. A noncontrast head computed tomography (CT) scan revealed a 4 cm right frontal hematoma with associated edema and sulcal effacement along with 3 mm of right-to-left midline shift []. Underlying neoplasm could not be excluded, and therefore, further imaging modalities were sought.\nMagnetic resonance imaging (MRI) of the brain with and without contrast done on the night of admission revealed stable acute hemorrhage in the right frontal lobe with underlying dural-based lesions in the right frontal lobe including a dominant hemorrhagic lesion measuring up to 2.4 cm []. Radiographic characteristics were most consistent with metastatic disease. A screening CT scan of the chest/abdomen/pelvis was done, which demonstrated mild hepatomegaly and calcifications in the right breast, but no signs of obvious malignancy. Positron emission tomography (PET) scan showed no evidence of extracranial disease.\nThe patient was started on dexamethasone and underwent imaging studies to fully evaluate her intracranial hemorrhage. Evaluation for a primary malignancy source found no other sites of disease. The decision was made to proceed with surgical resection, and the patient underwent a right frontal craniotomy with resection of the dural-based lesion with no adverse events. Postoperatively, the steroids were tapered and she was transferred to an acute rehabilitation facility. Postoperative MRI brain with and without contrast demonstrated enhancement along the margins of the resection cavity, compatible with a resolving residual hematoma [].\nHistologic examination revealed a mitotically active neoplasm with spindled and epithelioid components, conspicuous infiltration by chronic inflammatory elements, including many lymphocytes, and with associated hemorrhagic necrosis. On immunohistochemical staining, there was focal labeling for CD35 and more diffuse expression of clusterin []. The tumor cells focally expressed smooth muscle actin, minimally and focally expressed CK7, but not CK20 or CAM5.2, retained INI 1 expression, and did not express ALK, CD20, CD21, CD31, Chromogranin, EMA, ERG, GFAP, HMB45, Melan A, MyoD1, Myogenin, NeuN, SOX10, STAT6, or Synaptophysin. These findings are consistent with FDC sarcoma. The findings are not those of a melanoma, carcinoma, meningioma, tumor of neuroepithelial origin, other type of sarcoma or lymphoma. Foundation One report noted STAG2, PTEN mutation, MDM2 amplification, and DKC4 amplification.\nThe patient was sent to the emergency room from the clinic in August 2015 for confusion, urinary frequency, and hyperglycemia. She was still complaining of difficulty with her memory, as well as feeling drowsy, which was slightly worse than previous. She also complained of an episode of feeling shaking and confusion, which she was concerned was a seizure. On neurologic examination, she was once again grossly intact. She had not yet started radiotherapy or any adjuvant therapy for her brain tumor. CTH was performed in the ED, which demonstrated vasogenic edema in the right frontal lobe and 9 mm of the midline shift [].\nMRI brain with and without contrast on admission demonstrated increasing enhancement and edema in the region of the right frontal resection cavity, including a new 1.4 cm nodular focus of enhancement along the falx cerebri []. Radiographic findings were most suspicious for tumoral progression.\nThe patient's Keppra dose was increased and she was restarted on dexamethasone. She underwent repeat right frontal craniotomy for resection of both the frontal and midline dural-based masses with no adverse events. The patient received her first dose of fractionated frameless stereotactic radiosurgery, and was discharged on a dexamethasone taper and instructions to follow-up for further radiotherapy.\nThe specimen was similar to that of the previous resection, consisting of dura attached to granulation tissue, with associated large neoplastic pleomorphic cells in nests and areas of necrosis. The neoplasm once again expressed vimentin. Immunohistochemical staining was positive for p53 and CK7. Stains for AE1/3, BCL2, CAM5.2, CD21/31/34/35, Clusterin, CK20, and Synaptophysin were negative.\nThe patient presented again to the emergency room in December 2015 with dizziness and urinary urgency. She was found to have a UTI, but CTH on presentation demonstrated a new 1.5 cm hyperdense lesion in the right middle cranial fossa and a 5 mm lesion in the right high frontal convexity, with associated worsened vasogenic edema and midline shift, concerning for recurrent neoplasm. The patient once again complained of gradually worsening headaches and confusion, with difficulty in memory.\nMRI brain with and without contrast demonstrated increased size of the midfrontal enhancing lesion, as well as a new area of enhancement in the right anterior temporal lobe, with associated edema and mass effect [].\nThe patient was started on high dose steroids and antiepileptics and improved clinically. After discussion with the patient, the decision was made to not pursue further neurosurgical intervention. The patient was initiated on bevacizumab and irinotecan.\nBased on the patient and family wishes, the patient was made DNR/DNI prior to discharge with the decision for no further aggressive or invasive measures. She succumbed to her illness and expired in April 2016. |
A 40-year-old female presented with chronic headache with infrequent exacerbations. She presents with worsening headache for three months with associated vertigo, nausea, and vomiting not responding to analgesics or vestibular sedatives. Her symptoms initially started twelve years ago as a diffused mild headache, which persisted through the day. Gradually, the headache worsened to a severe headache episodically associated with vertigo, nausea, and vomiting. These episodes lasted for two to three days and got resolved. She was treated with flunarizine for suspected basilar migraine but did not show any response. From the last year, she had monthly exacerbations of headache associated with distressing vertigo, unsteadiness of gait, and right-sided body numbness. In between these episodes, she had a significant dull diffuse headache not responding to simple analgesia. She did not complain of fever or night sweats but had constitutional symptoms lasting for several months. All of these symptoms severely affected her daily activities and functionality.\nDuring the last 17 years, she had repeated episodes of neurological deficits. Even before the headache appeared, she has presented with visual impairment of the right eye and right lateral rectus palsy and was treated as retrobulbar neuritis with good response to methylprednisolone. One year later, she developed left-sided visual impairment, which fully responded to methylprednisolone. MRI imaging at that time revealed normal results. Few months after this event, she got admitted with right hemisensory loss with hemiplegia, and a demyelination disease or hemiplegic migraine was suspected. Second MRI was performed at this admission, and no abnormalities were detected again. Eight years ago, she had developed a left lower motor type facial nerve palsy, which was attributed to Bell's palsy. Within the last year, she was diagnosed to have depression and anxiety for which she was treated for few months. Other than the first two instances, she was not treated with steroids thereafter. She did not complain of weight loss and did not have constitutional symptoms or chest symptoms during these periods.\nOn examination, she is an averagely built female with a BMI of 23 kg/m2. She is afebrile, pale, and did not have lymphadenopathy. Her GCS was 15/15, and she was conscious and rational with normal pupillary response, visual acuity, visual field examination, and fundoscopy. There was no neck rigidity, and she had residual left lower motor VII palsy. She had an ataxic broad-based gait with unsteadiness. Upper and lower limb examination is clinically normal. Her respiratory, cardiovascular, and abdomen examinations were unremarkable.\nInvestigations revealed a hemoglobin count of 9 g/dL with normal white cells and platelets. ESR was elevated to 86 mm/1st hour. Renal- and liver-related biochemical investigations were normal with an alkaline phosphatase within the normal range. Initial MRI scans of the brain done 10 years ago did not reveal any abnormalities such as demyelination, optic nerve enhancement of focal lesions in the cerebrum, or cerebellum. CSF examination revealed an elevated protein level of 55 mg/dl with normal glucose and cells with negative oligoclonal bands or TB-PCR. Vasculitis investigations including ANA, ANCA (ELISA and Immunofluorescence), and RF were negative. Syphilis serology and HIV testing were also negative. Chest radiograph, ultrasound abdomen, and CT scan of chest and abdomen did not reveal any mediastinal lymphadenopathy or focal lesions in visceral organ or evidence of any malignancy. Serum ACE levels (19 µ/l) and ionized calcium levels were normal. We performed a new MRI scan of the brain with contrast, which revealed a diffuse and patchy meningeal thickening and enhancement mainly in the right frontoparietal and left occipital regions with a minor enhancement of bilateral optic sheaths (). Her NMO antibodies were normal, and the MRI did not show any areas of demyelination. Therefore, she underwent a dural biopsy from the thickened dura, which revealed large areas of caseous necrosis surrounded by epithelioid histiocytes and lymphoid cells with a few isolated giant cells in the adjacent vicinity (). There were no features of vasculitis or sarcoidosis. TB-PCR of tissue and acid-fast bacilli were negative. Final conclusion was necrotizing granulomatous inflammation suggestive of dural tuberculosis. This diagnosis was presumed by the presence of necrotizing granulomatous necrosis with caseation with a strongly positive Mantoux test of 25 mm (), and later was supported by a marked response to antituberculous medication.\nWe initiated her on antituberculous therapy (all four drugs for 3 months and 9 months of rifampicin and isoniazid) without streptomycin as she is already having vestibular symptoms. Steroids were added concurrently (1 mg/kg) and was continued for 6 weeks and was tailed off over a month. She experienced a marked improvement of her headache, and she could do her daily activities normally. After a year of anti-TB medication and follow-up, she did not complain of any worsening of symptoms. |
The proband, a 52-year-old Caucasian male with mild hypertension, presented to the hospital after his wife found him unconscious in his garage. He recalled doing some gardening work when he felt lightheaded before losing consciousness. The patient reported that he did not experience chest pain; however, he had a sense of foreboding before he collapsed. On physical examination, he was found to have a right-sided weakness consistent with a possible stroke; he was intubated and taken immediately for CT and MR of the head and neck. Imaging studies revealed a widespread dissection of the ascending portion of the thoracic aorta with extension into the common carotid arteries and left subclavian artery accompanied by high-grade stenosis bilaterally (secondary to the dissection). The patient was also found to have a dissection of the abdominal aorta, which extended into the iliac arteries, with apparent sparing of the renal arteries.\nEmergently, he underwent repair of the dissection of the ascending aorta. He had a hemiarch replacement, as well as a graft to the right axillary artery, an aortic valve repair, and valvuloplasty. Four days postoperatively, the patient developed acute left-sided hemiparesis. MRI of the brain showed predominantly right-sided cerebral hemispheric watershed infarcts with no evidence of hemorrhage. Additionally, it was found that he had a continued narrowing of the lumen of the common carotid arteries due to dissections. Because of the fragility of the vessels and the acuteness of the situation, vascular or neuroradiology intervention was not performed, and the patient was put under observation instead.\nThe proband underwent extensive physical therapy and rehabilitation and remarkably has recovered full physical abilities. In the course of his evaluation, he was found to have positive ANA titers up to 1 : 640. He had a homogeneous as well as a speckled pattern. He consulted with his local rheumatologist who performed an extensive serologic workup, all of which returned normal. The patient did not have any symptoms of inflammatory arthritis, rash, oral ulcers, alopecia, photosensitivity, Raynaud's phenomenon, skin thickening, skin hardening or tightening, dysphagia, cough, shortness of breath, or symptoms of serositis.\nThe family history does not include any known history of sudden vascular death, although his father did pass away at age 82 of a cerebral aneurysm. The patient did not display any traits of hypermobility and denies the history of recurrent joint dislocations, poor healing of the skin, or lens dislocations. He was on low-dose lisinopril for his hypertension, and he had taken prednisone long-term for his microscopic colitis, with doses of up to 30 mg per day, but usually not more than 10 mg per day.\nHeritable disorders of connective tissue (HDCT) sequencing and deletion/duplication panel was performed by a CAP-accredited/CLIA-certified laboratory to explore the possibility of a vascular form of a connective tissue disorder. This test analyzed the following genes via NextGen sequencing on an Illumina platform with concurrent exon-level oligo array CGH: ACTA2, ADAMTS2, ALDH18A1, ATP6V0A2, ATP7A, B3GALT6, B4GALT7, CBS, CHST14, COL11A1, COL11A2, COL1A1, COL1A2, COL2A1, COL3A1, COL5A1, COL5A2, COL9A1, COL9A2, DSE, EFEMP2, ELN, FBLN5, FBN1, FBN2, FKBP14, FLNA, LTBP4, MAT2A, MED12, MFAP5, MYH11, MYLK, NOTCH1, PLOD1, PRDM5, PRKG1, PYCR1, RIN2, SKI, SLC2A10, SLC39A13, SMAD3, SMAD4, TGFB2, TGFB3, TGFBR1, TGFBR2, and ZNF469 [UCSC hg19]. The test identified a heterozygous deletion of at least exons 21–34 of MYLK [3q21.1(123,332,644–123,386,568)x1 arr[GRCh37]]. The extent of the deletion was confirmed with whole-genome oligonucleotide array-based comparative genomic hybridization with single-nucleotide polymorphism analysis (CGH + SNP), arr[GRCh37] 3q21.1(123219342_123386568)x1.\nAll of our proband's living first-degree family members, mother, brother, and three daughters, were screened for the MYLK deletion. His 87-year-old mother was found to carry the same variant but had no history of aortic disease. She had completed cardiac evaluation due to the history of a myocardial infarction and placement of a pacemaker. The proband's daughter and brother also tested positive; they appear to be currently unaffected as well. His brother had completed recent cardiac imaging, which was negative. A child of the proband's brother plans to undergo further testing to see whether they carry the mutation.\nThe sequence of human, smooth muscle myosin light chain kinase (MYLK) (isoform 1) is a part of the muscle-contraction process that occurs through phosphorylation on myosin light chains. MYLK, which is a protein encoded by the MYLK gene, was taken from the NCBI Reference Accession Sequence: NP_444253: version NP_444253.3, which is encoded for the amino acid sequence and was used for computer-assisted modeling. Monte Carlo simulations were performed on the mutant to allow local regional changes for full-length 1914 amino acids and when the p.S1218del variant was introduced.\nThe refinement module for Monte Carlo was built using the in-house code (and YASARA) SSP/PSSM method [–]. The structure was relaxed to the Amber force field using knowledge-based potentials under the defined algorithms. The side chains and rotamers were adjusted with knowledge-based potentials, simulated annealing with the explicit solvent, and small equilibration simulations as described []. The entire full-length protein sequence was used for the structural models, and any gaps or unresolved portions from the X-ray data were filled in using our established methodology [–].\nRefinement of the finalized models was completed using either Schrodinger's MCMD search with the Monte Carlo-based module or NAMD2 protocols. Molecular refinements started with the in-house code (built on YASARA) generated initial refinement and mutant p.S1218del [–, ]. The superposition and subsequent refinement of the overlapping regions yield a complete model for MYLK. The final structures were subjected to energy optimization with the PR conjugate gradient with an R-dependent dielectric.\nAtom consistency was checked for all 1914 amino acids (29,429 atoms) of the full-length wild-type model and 1217 amino acids (18,546 atoms) for the p.S1218del variant, verifying correctness of chain name, dihedrals, angles, torsions, nonbonds, electrostatics, atom-typing, and parameters. Each model was exported to the following formats: Maestro (MAE) and in-house code (PDB). Model manipulation was done with Maestro (Macromodel, version 9.8, Schrodinger, LLC, New York, NY, 2010) or visual molecular dynamics (VMD) [].\nMonte Carlo dynamics searching (LCMOD-MC) was completed on each model for conformational sampling using methods previously described in the literature [, , , ]. In brief, each system was minimized with relaxed restraints using either steepest descent or conjugate gradient PR and then allowed to undergo the MC search criteria, as shown in the literature [, , , ].\nFor the wild type versus the variant p.S1218del, there is a clear loss of the kinase domain, which would have loss of function effect on the protein. Additional domain regions missing in the variant include Ig-like C2-type 8, fibronectin type-III, and Ig-like C2-type 9 domains. Taken together, this would constitute a negative variant as the large N-terminus may participate in binding and cause additional loss of function that a heteromeric individual's wild-type MYLK would be competing for binding [, , , , –] (); our modeling has previously supported such findings [–, –, ]. Additionally, significant loss of activity was seen due to the enzymatic region being cleaved from the protein's global structure (Figures and ). |
A medically free 14-year-old male who was obese sustained an injury to his left knee after jumping from 3 stairs. The patient mentioned that he predominantly landed on his left lower limb with his left knee in full extension and in external rotation. The patient started complaining of left knee pain limiting his range of motion and ability to bear weight immediately after the fall. He was brought to the Emergency Department (ER) of King Saud Medical City (KSMC) by his parents immediately after the injury. On physical examination, the left knee was profoundly swollen and bruised. There was tenderness over the tibial tuberosity and lateral joint line. He was unable to actively move the knee joint. The passive range of motion was painful. There were no signs indicating compartment syndrome or neurological or vascular injury. X-ray radiographs revealed a Watson-Jones type IIIB avulsion fracture of the tibial tuberosity apophysis (). A CT scan showed a step of the articular surface more than 2 mm extending to the posterior-lateral epiphyseal part of the proximal tibia (lateral tibial plateau) ().\nThe patient was admitted and was prepared for operative management. A procedure was planned and done on a radiolucent table under general anesthesia. A tourniquet was used to avoid excessive bleeding during the procedure. The tourniquet was inflated after pulling down the quadriceps to avoid blocking the reduction due to the extensor mechanism. The leg is prepped and draped according to the standard orthopedic protocol. Anterolateral approach of the knee was used with an incision starting from the lateral upper border of the patella to 10 cm down. Deep fascia was opened anterior to the iliotibial tract. The fracture line was identified; the reduction of the articular step was done using a reduction clamp and assured using a portable image intensifier on flexion and extension of the knee without arthrotomy. We avoided arthrotomy of the joint to not make it vulnerable to infection and possible scarring. Stabilization of the reduction was maintained using a k-wire. Definitive fixation was achieved with three 3.5 mm partially threaded cancellous screws placed under fluoroscopic guidance for the tibial tuberosity fracture. A proximal tibial plate was slid laterally and was used to buttress the lateral tibial column. Careful placement of the screws was done to not cross the physis with the help of a C-arm (). After fixation, good hemostasis was achieved, drain was placed, and the range of motion was assessed which was full. Closure was done layer by layer, then dressing after. The postoperative plan was to immobilize the knee in a cylindrical cast for 3 weeks with no weight bearing on the left lower limb with the use of crutches for ambulation.\nPostoperative knee CT scan is requested to ensure that the fracture is anatomically reduced. The patient received analgesia and antibiotics, and drain was removed 24 hours post-op. The patient was seen in an orthopedic clinic after 3 weeks, there were no signs of surgical site infection, and the clips were removed. The controlled range of motion was advised using a hinged knee brace throughout the day for 4 weeks. A follow-up X-ray () shows that the fracture is aligned with no loss of reduction or displacement. Physiotherapy is advised 7 weeks postoperative management focusing on the range of motion and strengthening. The patient was seen 6 weeks later, he had full range of motion with no deformity, and there were no complaints reported by the patient like locking or pain. |
A 77-year-old man was presented with cardiovascular pathologies that included severe mitral regurgitation (MR) and moderate tricuspid regurgitation (TR). He had no history of congenital venous disease. He had recent shortness of breath with exertion. His New York Heart Association status was class II. Echocardiogram results showed severe MR due to P3 prolapse, moderate TR, and pulmonary hypertension. The left ventricular function was normal with ejection fraction at 61 % and coronary angiogram findings revealed no significant coronary artery stenosis. Renal function was normal with creatinine at 0.69 mg/dl and preoperative hematocrit at 41 %. A preoperative nutritional assessment showed a normal serum albumin level.\nThe patient underwent a mitral valve repair with P3 resection and suture, followed by ring anuloplasty and tricuspid anuloplasty procedures using a prosthetic ring via a right mini-thoracotomy. Cardiopulmonary bypass was established with antegrade arterial flow through the right subclavian artery, and bicaval venous drainage through the superior vena cava and right femoral vein. We checked the position of venous cannula by transesophageal echocardiography (TEE). Also, we confirmed the position under direct vision when performing tricuspid annuloplasty. Cardiac arrest time was 219 min and cardiopulmonary bypass time until declamping aorta was 262 min. During main procedure, cardiopulmonary bypass flow, mean arterial pressure, and central venous pressure (less than 5 mmHg) were stable within normal range. The value of hematocrit during cardiopulmonary bypass was between 22 and 25 %, and base excess was around −5.0 mEq/L. Blood transfusion was also performed. We observed no sign of poor venous drainage from his lower body, such as severe edema of lower extremity and ascites. While weaning from the bypass, the patient became hemodynamically unstable and the weaning became difficult. Soon thereafter, hemodynamic status worsened even while under the cardiopulmonary bypass due to insufficient venous drainage. TEE showed that the heart appeared to be empty and hyperdynamic contraction of the left ventricle. There was no bleeding in the pericardium cavity or left pleural cavity, and no evidence of aortic dissection. Despite fluid infusion, we could not maintain a sufficient venous return to maintain blood pressure. The only abnormal finding at that point was abdominal distention. We suspected retroperitoneal bleeding but could not find any hematoma throughout the right groin or in transgastric TEE.\nSince high intraabdominal pressure was externally apparent, we decided to perform a decompression laparotomy, which was done by the general surgery team. A midline laparotomy revealed severe edema of the small intestine and mesentery (Fig. ). The abdominal cavity was decompressed by putting the small intestine into a transparent plastic intestinal isolation bag, which was secured to the laparotomy wound. As soon as the abdominal cavity was decompressed, central venous pressure dropped down from 15 to 4 mmHg, and the patient became hemodynamically stable under the cardiopulmonary bypass. Because of the long bypass time (501 min) and low oxygenation due to a large amount of fluid infusion, it was difficult to wean off the cardiopulmonary bypass, and the patient was sent to the intensive care unit with extracorporeal membrane oxygenation.\nPostoperatively, intestinal and mesenteric edema showed rapid dispersion with high urine output then promptly recovered after the decompression laparotomy. The general surgical team performed abdominal closure on postoperative day 1. Thereafter, the patient became hemodynamically stable and could be weaned from extracorporeal membrane oxygenation on postoperative day 8. Although pneumonia and acute renal failure later developed, he recovered well and was discharged on postoperative day 101. Twelve months after the operation, the condition was stable.\nThere are two types of ACS; primary with an intraabdominal origin and secondary. Secondary ACS has an extra-abdominal cause such as massive fluid resuscitation leading to intestinal and mesenteric edema []. Organ dysfunction seen in these cases typically includes hemodynamic instability, respiratory insufficiency with impaired gas exchange, and acute renal failure, which generally become apparent early in the course of the disease []. Therefore, ACS could be fatal in a patient under cardiopulmonary bypass, as increased intraabdominal pressure can cause a failure of venous return. However, only a few cases of ACS during cardiopulmonary bypass have been reported [, ], and there are no patients undergoing minimally invasive cardiac surgery.\nThe mechanism of ACS involves several factors such as shock, hypoxia, massive crystalloid resuscitation, and extreme hemodilution []. In addition, there may be some role for extracorporeal circulation in development of intraoperative abdominal hypertension. Previous reports have shown an increase in intraabdominal pressure that is dependent on the degree of hemodilution after initiation of cardiopulmonary bypass, which has a significant impact in such bypass cases with cardiac arrest on mesenteric circulation []. Hypothermia is also reported to be a predisposing factor in ACS []. Cardiopulmonary bypass is associated with systemic inflammatory response, which can cause increased capillary permeability. Furthermore, insufficient venous drainage frequently results in systemic interstitial edema. In the present case, we initially did not intend to perform transfusion during surgery, though we stored autologous blood obtained prior to the operation, which might have caused hemodilution and a low hematocrit value. Also, when an adequate flow could not be obtained, a large amount of crystalloid infusion was initially given.\nPrompt diagnosis is very important to reduce the negative effects of ACS, and once a diagnosis is made, the appropriate treatments should be immediately started. Various nonsurgical approaches, such as intraabdominal fluid drainage and neuromuscular blockade, have been shown to reduce intraabdominal pressure []. A decompression laparotomy should be considered to relieve pressure and restore capillary perfusion in the organs of critically ill patients [, ]. In the present case, a relatively long period of time was needed to make a diagnosis of ACS, as there were several points to check to determine the reason for instability of cardiopulmonary bypass due to the small incision and insufficient view of the heart, vessels, and other side of the pleural cavity, which might have caused massive fluid resuscitation and requirement of extracorporeal membrane oxygen. Once a decompression laparotomy was performed and intraabdominal pressure relieved, hemodynamic status dramatically improved, and massive urine output led to early abdominal closure. |
A 55 year old man was diagnosed with anemia by his family physician towards the end of 2017. In February of 2018, an upper GI endoscopy and colonoscopy were performed which showed a right colon cancer. CEA was 40.4. CT of the chest, abdomen, and pelvis was obtained in February of 2018. This showed a mass 7 cm in greatest diameter that was immediately adjacent to the undersurface of the right liver and contiguous with numerous loops small bowel and the lowest part of the second portion of the duodenum (). There were mildly prominent mesenteric lymph nodes but no evidence of distant metastatic disease.\nIn February of 2018, the patient underwent a single port laparoscopic right colon resection. The surgeon described the tumor as stuck to the undersurface of the liver with adhesions taken down without difficulty. On histopathologic examination, the tumor was PT3N0M0 with 0/16 positive nodes. Perineural invasion was identified. The cancer was moderately to poorly differentiated. Systemic chemotherapy was not recommended.\nIn June of 2018, the patient began noting problems with digestion and pain after eating. CEA had increased to 940 ng/ml. Repeat CT scan showed multiple nodules within the greater omentum compatible with peritoneal metastases. The liver, kidneys, and ureters were normal. There was marked stranding within the right colon resection site (). Bowel loops proximal to the prior ileocolic anastomotic site were moderately dilated and fluid-filled. A mass was noted in the abdominal wall at the laparoscopic port site. There was also a mass in the rectovesical space immediately adjacent or invading the right and left seminal vesicles.\nIn July of 2018, the patient underwent exploratory laparotomy. The posterior surface of the liver was layered by cancer. The undersurface of the right hemidiaphragm was covered by tumor nodules. The pelvis contained a 4 cm tumor mass invading into the seminal vesicles. The old ileocolic anastomosis was involved and needed to be resected. The umbilical port site was infiltrated by cancer. Near complete resection was possible by cytoreductive surgery.\nIntraoperatively, the patient was treated with HIPEC-melphalan []. This postoperative course was unremarkable. The histopathology showed adenocarcinoma compatible with the primary tumor in all tissues submitted. The peritoneal cancer index was 28 and the CC score was 2 []. The resection was judged to be a palliative effort and its major goal was to relieve intestinal obstruction and to prepare the patient for rapid initiation of palliative systemic chemotherapy.\nNeither of our patients was evaluated by the MDT preoperatively. Their preoperative and intraoperative management was evaluated in an attempt to improve the outcome of subsequent patients. |
We report a case of a 75 year old woman addressed to Allergology Department of our hospital in January 2014 for recurrent episodes of angioedema since the age of 66, with progressively increased severity and frequency. It was first considered to be induced by treatment with angiotensin-converting-enzyme inhibitors (ACEI) for mild hypertension, but she continued to have angioedema attacks for the next 6 years after discontinuation of ACE, with progressive aggravation during the last year. The previous multiple evaluations by many specialists in other hospitals did not succeed to give a clear diagnosis and treatment.\nThe patient had no relevant medical history and took no medication, except ACEI that was stopped some months after angioedema onset. No relation with possible allergic stimuli could be identified and she had no clinical manifestations between attacks. Angioedema was painful, not accompanied by urticaria or abdominal symptoms, located variably to neck, arms or buttocks, without facial involvement during 6 years. The attacks occurred at weeks or months intervals and usually lasted between 48 and 72 h, irrespective of corticosteroids and antihistamines treatment usually administered. The frequency of attacks had progressively increased from one at 2–3 months intervals to almost weekly during the last year before presentation. The last angioedema attack, determining emergency hospitalization in September 2014, was more severe and prolonged, accompanied for the first time by laryngeal edema and respiratory symptoms. The extended medical evaluation, including complete blood tests for inflammation, allergy, autoimmunity and cancer, were all negative (Table ). Full body CT scan and bone marrow examination were normal. No criteria for lymphoproliferative, mieloproliferative or autoimmune diseases could be found. Measurement of C1 inhibitor (C1INH) in plasma showed significantly decreased level at all measurements, with low activity ranging from 58 to 4% and constantly low C4 (Fig. ). Complement fraction C1q plasma level was measured twice and had normal value. Genetic tests were not performed, given the patient advanced age and lack of family history of angioedema, which are against HAE. A spontaneous mutation in SERPING 1 gene is noticed in up to 25% cases of HAE without family history, but we considered this probability very low in our case, due to late onset of angioedema.\nTreatment of angioedema attacks before hospitalization consisted of antihistamines and systemic corticosteroids, which proved to be ineffective. Since no pathogenic therapy with C1INH concentrate, antagonists of bradikinin receptors (icatibant) or selective inhibitor of plasma kallikrein (ecallantide) was available in 2014, we first initiated daily prophylactic treatment with tranexamic acid for 3 months, with no amelioration. During the more severe attack with laryngeal edema, in September 2014, we have switched to attenuated androgen danazol, given 400 mg the initial dose, reduced to 200 mg daily after 1 week and then to 100 mg daily. The clinical evolution was very good, no angioedema attack occurred since the introduction of danazol. C1INH and C4 plasma levels increased after 2 weeks of treatment and became normal after 1 month. After some months, the patient decided herself to discontinue danazol for short time, in order to check effects and to taper the minimal dose. Serum C1-INH and C4 plasma levels were measured after 2 and 4 weeks and showed significant lower levels, but no angioedema attack occurred during this period of time. She therefore restarted danazol prophylactic therapy at a minimum of 50 mg dose daily, ongoing after 3 years. The patient was closely monitored during the next 3 years, with complete clinical and laboratory control twice a year. We took into consideration the possible side effects of danazol, mainly dislipidemia, haematological and liver malignancies and any other complications or concomitant diseases. The clinical evaluation was very good, except two episodes of pulmonary cysts infection, remitted with broad spectrum antibiotherapy, which could not be related to danazol treatment. The clinical outcome of angioedema after 3 years of danazol treatment is very good, with no attacks or other related symptoms. No clinical or laboratory sign of any disease that could induce C1 INH deficiency occurred. No relevant side effects of danazol were noticed. The patient has an improved quality of life due to therapeutic compliance and general management plan. |
A 69-year old woman was admitted to the Inha University Hospital with complaints of mucous diarrhea and low abdominal pain with repeated relapses and remission of symptoms over 5 months. She was taking medication for hypertension and had a history of adjuvant chemotherapy after undergoing a low anterior resection due to rectal cancer 12 years prior to admission to our hospital. Five months ago, the patient was admitted to a local hospital due to diarrhea that developed after eating pork. She then visited our hospital's outpatient unit because her symptoms remained the same. Even though antidiarrheals and probiotics were administered to the patient in the outpatient unit, her symptoms were not improved.\nA colonoscopy performed two months prior to her visit revealed edema, hyperemia, and multiple whitish plaques from the transverse colon to the sigmoid colon (). Since pathologic findings included denuded epithelium covered with a mucoid exudate () and stool test results were positive for Clostridium difficile toxin, she was diagnosed with PMC. Her CRP level was elevated to 7.99 mg/dL and no other bacteria were detected from blood or stool cultures. Administration of metronidazole (500 mg), orally 3 times per day for 10 days, temporarily improved her symptoms, with a decrease of CRP to 1.58 mg/dL. However, diarrhea persisted and her symptoms were gradually worsened. Subsequently, vancomycin (250 mg) was given orally 4 times per day for 2 weeks, and symptoms were improved for a week, but diarrhea persisted.\nShe underwent colonoscopy again after being admitted to our hospital 1 month prior to her final admission for FMT. Colonoscopy revealed PMC, and re-elevated CRP levels (8.82 mg/dL). Hence, a daily dose of oral vancomycin was increased to 500 mg, 4 times per day for 2 weeks. However, mucous diarrhea persisted after discharge and the symptoms were not improved. Since her PMC was determined to be refractory to antibiotics, we decided to perform an FMT after obtaining consent from the patient and her guardian. At the time of admission for FMT, she had stable vital signs (blood pressure 146/78 mmHg, heart rate 80 beats/min, respiratory rate 18 breaths/min, and a temperature of 36.0℃) but was critically ill. She complained of systemic muscle weakness, abdominal pain and a feeling of incomplete defecation. The patient suffered from diarrhea occurring 5–8 times per days. Mild tenderness in the lower left abdomen was detected in a physical exam. Her heart and breath sounds were normal and skin rash was not observed.\nLaboratory tests revealed WBC 8,860/mm3, hemoglobin 13.3 gm/dL, platelet count 190,000/mm3, total protein 6.9 gm/dL, albumin 3.8 gm/dL, total bilirubin 1.3 mg/dL, AST 19 IU/L, ALT 15 IU/L, ALP 161 IU/L, BUN 9.4 mg/dL, creatinine 0.87 mg/dL, CRP 0.21 mg/dL, Na 138 mEq/L, K 2.9 mEq/L and Cl 105 mEq/L. Based on these results, she was suspected to have hypokalemia attributable to repeated diarrhea. No specific findings were observed on simple chest radiographs.\nThe patient's daughter, who had no underlying diseases or gastrointestinal (GI) symptoms, volunteered to be the donor. In addition, the daughter had no history of medication over the previous 3 months. Donor screening tests were carried out, including a parasite exam, stool culture, and a susceptibility test for Salmonella, Shigella, and Campylobacter species, a C. difficile toxin A/B test. In addition, several serum tests were done, including serum Ameobic Antibody, HAV IgG/M, HBsAg/Ab, HBcAb, HCV Ab, HIV Ag/Ab, vereneal disease research laboratory. The donor had no abnormal findings. Before the transplantation, the use of antibiotics was suspended and bowel cleansing was performed. Colonoscopy revealed edematous and hyperemic mucosal changes with multiple whitish patches from the transverse colon to the sigmoid colon ().\nOn the day of the procedure, 50 g of fresh feces were collected from the donor and then mixed and stirred with 500 mL of normal saline. Subsequently, the supernatant of the solution was filtered using a coffee filter or gauze (). Approximately 500 mL of the filtered fecal suspension was administered into the proximal ascending colon via a colonoscope (). After the FMT there were no complications and the patient was discharged 2 days after the procedure. At the first week follow-up visit, mild abdominal discomfort was present but diarrhea was improved. One month after being discharged, a follow-up colonoscopy was performed and found no relapsing symptoms. Mucosal edema and hyperemia were also dramatically improved (). The patient is currently being monitored and had no recurrence of symptoms at the last follow-up, 9 months after the procedure. |
A 19-year-old female from east Sudan presented 3 months after giving birth of her first healthy baby with huge multiple bilateral breast masses. She was very anxious about this bilateral tumor growth in her both breasts. This tumor started to grow 2 years prior to the presentation (age 17). Initially, the masses appeared in the right breast and shortly involved the left one. Lumps showed a gradual course initially, but a dramatic rapid growth was noticed during pregnancy. There was severe discomfort especially in the left breast, due to rapid growth and inflammation. There was no past history of breast trauma or family history of breast cancer. The patient started to menstruate at the age of 15 years, without reporting any abnormal events in her breasts. She was of good internal and external hygiene; no history of smoking, drinking, or any bad habits; and no allergies. Both breasts had multiple lumps; the right breast mass was measuring about 7 cm × 7 cm, hard and nodular with no skin changes or evidence of deep structure involvement by clinical palpation. The largest left breast mass was about 7 cm × 5 cm with other smaller ones occupying all four quadrants. There was large area of skin changes, which surrounds the left large lump with a large irregular inflamed ulcer below and lateral to the left nipple with no elevated edges. Both breasts were lactating, and milk discharge from the nipple was observed with normal color.\nUltrasound report of the left breast showed multiple bilateral echogenic breast enlargements; the largest one was about 7 cm × 7.55 cm. Also, there was a 7 cm × 4 cm well-defined, turbid, and cystic collection noted beneath the ulcerated area in the left breast (Figure ). Dilated right breast mammary ducts were noticed. The nipple and overlying skin were intact. Multiple enlarged bilateral axillary lymph nodes (LNs) were identified. The cytology report confirmed area of hyalinization with no malignant changes. Patient completed 6 months of conservative management (bromocriptine and antibiotics) locally without any signs of improvement. The size of the tumor steadily growing, inflammation, ulceration, and LN enlargement were getting worse with time. Consequently, left simple mastectomy had been done based on failure of conservative management and infiltration of other quadrants in the left breast with very huge ulcerated tumor. Active surveillance with watchful wait was management plan for the right breast mass. The excised specimen's histopathological report revealed macroscopically an exophytic growth measuring 7 cm × 6 cm lateral to normal looking nipple with skin ulceration. Cross sectioning of the specimen showed no breast tissue, only lobulated firm mass with central cyst behind the nipple measuring 10 cm × 7 cm × 5 cm in diameter. Microscopically, sections showed enlarged lobules, with complex glands composed of inner actively secreting epithelial cells with vacuolated cytoplasm and apical cytoplasmic blebs and outer myoepithelial layer (Figures and ). There was no cytological atypia, and stroma was scanty. There were two reactive isolated axillary LNs identified during histology; the right breast showed the same histological features (Figures and ). P63 and S100 were positive. The immunohistochemistry confirmed a diffuse tumefactive lactating adenosis with very complicated histological picture. Eventually, 6-month follow-up was arranged, and overall prognosis after surgery was satisfied. |
A 52-year-old woman presented with pain and active function loss in her left shoulder, and was admitted to our hospital in June 2017. Her shoulder problem had started 3 months earlier, and there was no history of trauma or fracture. At first the pain was intermittent and bearable, but then gradually increased. On examination, there were no positive signs except for localized pain. A radiologic examination on 3 April 2017 found no destruction of the shoulder (Fig. ). Pain at the shoulder joint became gradually aggravated, together with the appearance of shoulder joint dysfunction. Two months later, physical examination revealed mild swelling of the shoulder, and markedly restricted shoulder and elbow motion. Mild distal nerve function defects appeared gradually. Radiography on 3 June 2017 showed that the head of the humerus had disappeared within the past 2 months (Fig. ), which was confirmed by magnetic resonance imaging (Fig. a, b).\nThe patient is a healthy, active individual with no history of weight loss, anorexia, or fever during this period. General and systemic examination findings were within normal limits. Routine laboratory investigations were also normal, including levels of serum calcium, phosphate, alkaline phosphatase, high-sensitivity C-reactive protein, and erythrocyte sedimentation rate. An open biopsy of the lesion revealed that the bony tissue had been replaced by fibrous connective tissue, and small areas of bony trabeculae with occasional osteoclasts were visible (Fig. ). There was no evidence of malignancy or tuberculosis. Because of the lack of any clinical findings or supporting data for other causes, the features were confirmatory of GSD. Computed tomography of the shoulder joint (Fig. ) revealed a bony defect of the glenoid cavity.\nWe performed surgery involving glenoid cavity amplification with an autologous iliac bone graft and a reverse total shoulder arthroplasty. The grafts were from autologous iliac bone (Fig. , Fig. ). A deltopectoral approach was used on the shoulder, and necrotic and dissolving bone tissue was removed. Reconstruction of the glenoid was carried out with autologous iliac bone and installation of reverse shoulder prosthesis. Postoperatively, the arm was placed in a sling for 3 months. Passive elevation and external rotation were allowed 2 weeks after the operation. Three months later, sling use was discontinued, and active range of movement was initiated. Six months after surgery, the patient is pain-free with more than 90° of active abduction, 100° of forward flexion, and 30° of shoulder posterior extension. She also has good functional use of her shoulder (Fig. , Fig. ). |
A 56-year-old man presented with disturbance of consciousness after manifesting clouded consciousness, during which he could not open his eyes spontaneously without anisocoria. He was transported to the local neurosurgical department and diagnosed with intracerebral hematoma. He had no history of head trauma or hematological disorders. Computed tomography revealed a right ASDH of 1.5 cm in diameter and a 15-ml oval subcortical hematoma in the right occipital lobe associated with slight perifocal edema. The midline was slightly shifted to the left (fig. ). We planned our strategy of emergent removal of ASDH after diagnosis of the hemorrhagic source. Digital subtraction angiography was performed emergently and demonstrated a dural AVF located in the right occipital convexity. The main feeding arteries were the right middle meningeal artery, parietal branch of the superficial temporal artery and meningeal branch of the right occipital artery (fig. ). Shunt flow was not markedly high and drained into the superior sagittal sinus and vein of Galen via cortical veins on the occipital lobe associated with venous pouches and cortical reflux. The lesion was classified as type IV on the Cognard classification. ASDH removal was planned after obliteration of the hemorrhagic source of dural AVF by endovascular treatment because the consciousness level was not aggravated under hyperosmotic fluid administration, and endovascular embolization could be performed immediately after DSA. Transarterial embolization was performed via the right middle meningeal artery. Venous pouches were suspected ruptured points and were occluded with diluted n-butyl cyanoacrylate. Shunt flow disappeared after embolization. Craniotomy was subsequently performed and the subdural hematoma was evacuated with external decompression within 1 h after transarterial embolization. No hemorrhagic point was observed on the surface of the cortex. The shunting point on the surface of the dura mater was also removed. His consciousness level improved after surgery. The postoperative course was uneventful, and cranioplasty was performed after recovery from brain edema. He could communicate with conversation and walk with aid after 3 weeks of rehabilitation. |
A 17-year-old male patient attended the oral and maxillofacial surgery clinic in King Saud University requesting extraction of all third molars. The patient’s medical history was unremarkable; he was a non-smoker and revealed no history of previous facial trauma or surgery. There was neither pain nor swelling of the buccal and lingual sulci of the mandible and no cervical lymphadenopathy. Initial dental examination revealed a minimally restored permanent dentition with good oral hygiene. A panoramic radiograph showed that all third molars were present and impacted except on the upper right side where the tooth was missing, with the lower third molars in close proximity to the inferior alveolar canals (Figure ). The radiograph was otherwise unremarkable with no signs of pathology or abnormality.\nA CBCT of the mandible was requested for further assessment of the relation and proximity of the roots to the inferior alveolar canal. A survey of the CBCT sections of the mandibular body revealed bilateral sublingual depressions inferior to the premolars and first molars extending to the inferior border of the mandible that was not seen on the panoramic radiograph.\nTo assess the depth and width of these depressions, the CBCT images were reconstructed in planes aligned with the axes of the right and left mandibular second premolars in the mesiodistal dimension, buccolingual dimension, and the coronoapical dimension. Then, linear measurements representing the width of the depressions were made along a line drawn a tangent to the heights of contours (crest of curvatures) of the lingual surfaces of the mandible superior and inferior to the bilateral depression. The superoinferior width of the depressions was found to be 2.1 cm on the right side and 2.9 cm on the left side. Linear measurements representing the buccolingual depth of the depressions were made along a line drawn perpendicular to the above-mentioned width line to the deepest point of the depression; it measured 0.59 cm on the left side and 0.6 cm on the right side. The buccolingual thickness of the remaining mandible was 0.42 cm on the left side and 0.45 cm on the right side (Figures -).\nThe diagnosis was concluded to be bilateral sublingual depressions of the mandible. It is considered a normal anatomical variation that does not require any further investigation or intervention. The incidental finding was documented in the patient dental record for future implications in case an implant placement was needed. |
An 8-year-old thin built Indian boy belonging to lower socio-economic group presented to the orthopedic department with the chief complaints of pain in the left leg for the last 3 months. There was no fever at presentation. On examination there were three small and scantily draining sinuses in the proximal and middle third of his left leg. The ipsilateral inguinal lymph nodes were not significantly enlarged. There was history of high grade fever for a few days three months back with spontaneous acute onset pain and swelling around the upper left leg with inability to walk, which was managed by a local doctor with some medication that provided some symptomatic relief within a matter of a few days time. The three small non-healing wounds however persisted. After three months the patient attended our hospital for further treatment.\nThe child had no fever or palpable abscess on examination. Clinically, swelling was minimal in the affected site of the leg. Radiograph of the affected limb showed features of chronic osteomyelitis with a large diaphyseal sequestrum on the medial cortex of tibia with incomplete involucrum []. The total lymphocytic count was within normal limits and differential counts were unremarkable. Sedimentation rate was 38 mm per hour. Tuberculin test and fungal smears were negative. The patient could not afford to do magnetic resonance (MR) imaging or a computerized tomography (CT). Wound swab taken from the proximal draining sinus yielded methicillin sensitive Staphylococcus aureus (MSSA) sensitive to 3rd generation of cepahalosporin and aminoglycoside (Amikacin), which were started and continued for a period of six weeks. No surgery was performed; not even incision and drainage. The sinuses healed completely in 6 weeks time. Gradually, over a period of 8 months, the large tibial diaphyseal sequestrum got fully incorporated into the healthy diaphyseal bone indistinguishable from normal bony architecture [Figures and ]. The functional outcome also was excellent as demonstrated by full range of motion at the knee joint [Figures and ]. |
A 35-week-old gestational age male weighing 2,495 grams was admitted to our neonatal intensive care unit (NICU) shortly after he was born secondary to previously diagnosed gastroschisis. The child was born to a 17-year-old gravida 1 para 1 female. The pregnancy was uncomplicated other than a traumatic event that occurred at 17 weeks resulting in placental abruption but no loss of the pregnancy. The mother did receive appropriate prenatal care. The child was born via an uncomplicated vaginal delivery with APGAR scores of 9 and 9 at one and five minutes, respectively. There is no family history of gastroschisis or intestinal atresia. The mother's only medical history included celiac disease.\nUpon presentation to the NICU, the child was examined by the surgical service and deemed to be an appropriate candidate for reduction of the gastroschisis and primary closure of the abdominal wall defect. Only a small amount of bowel was protruding from the abdominal wall defect without signs of compromised bowel viability. The patient was subsequently taken to the operating room where a nasogastric tube was placed for decompression. Furthermore, the rectum of the patient was emptied of meconium by gentle anal dilatation and irrigation, providing standard of care prior to reduction and closure of the abdomen for the gastroschisis. The patient's gastroschisis was reduced and the abdominal wall was primarily closed without any complications. The extracorporeal bowel was found to be mildly edematous with minimal inflammatory peel and no signs of atresia. The patient was transferred back to the NICU where he was extubated within the first 24 hours postoperatively. A peripherally inserted central catheter was placed on postoperative day one and the patient was started on total peripheral nutrition (TPN), awaiting return of bowel function before starting enteral feeds. Postoperatively, the patient never developed any signs or symptoms of abdominal compartment syndrome or necrotizing enterocolitis. The patient was continued on IV antibiotics for a total of 72 hours from the date of surgery.\nThe patient remained hemodynamically stable with nasogastric decompression and parenteral nutrition. He continued to demonstrate high output from the nasogastric tube, which, over the course of his first three weeks of life, became more mucus-like and nonbilious. His abdomen remained mildly distended but, as Snyder et al. pointed out, patients can often suffer from severe ileus for up to 3 to 4 weeks after closure of the gastroschisis []. Further studies have also demonstrated prolonged ileus from closure of a gastroschisis. A study from 2000 by Driver et al. showed the median time to full oral feedings and resolution of ileus was 30 days (range: 5 to 160 days) [] and a 2011 study by Bradnock et al. showed a median duration of 21 days (range: 9 to 39 days) to reach full oral intake []. For this reason, we never became alarmed as we felt our patient was just suffering a prolonged ileus. However, as the patient approached four weeks since the initial gastroschisis closure, the concern for a possible bowel obstruction became evident as the patient never developed any further bowel function since the original passage of meconium at the initial surgery. Therefore, an upper gastrointestinal study with small bowel follow-through was obtained (). The study demonstrated multiple dilated loops of proximal small bowel, consistent with a small bowel obstruction. Therefore, on day of life 27, the decision was made to take the patient back to the operating room for exploratory laparotomy.\nAfter an extensive enterolysis, it was found that the patient had type III proximal jejunal atresia, which is when the blind ends of bowel are separated by a V-shaped defect of the mesentery []. Eight centimeters of the distal atretic jejunum was resected secondary to questionable viability; however, the remaining bowel appeared healthy and without inflammatory peel. A catheter was inserted into the distal atretic enterotomy and sterile normal saline was injected, demonstrating patency of the bowel through to the ascending colon. At this point, a primary end-to-end hand-sewn enteroenterostomy was created followed by a proximal jejunal plication secondary to the size difference of the two ends of bowel. The abdomen was primarily closed and the patient was transferred to the NICU in a stable condition.\nOnce again, the patient did well postoperatively. His TPN was continued and the nasogastric tube remained in place for decompression secondary to the anticipated postoperative ileus. Three weeks following the second surgery, there was again concern for an obstructive process, as the patient was still demonstrating high, nonbilious output from the nasogastric tube with chronic abdominal distension and no progression of bowel function. Furthermore, the patient also developed elevated bilirubin levels due to the extended period of time receiving TPN. At this point, we attributed the prolonged ileus to the previous two extensive surgeries experienced by the patient; however, in retrospect, we probably observed the patient too long this second time. The average postoperative ileus for jejunal atresia is approximately 5 days with a range of 3 to 10 days []. Additional imaging, such as an abdominal ultrasound, may have been beneficial during this time to help determine why the patient was not having bowel function. Nonetheless, a barium enema was obtained () after our allotted observation time demonstrating no progression of contrast beyond the midtransverse colon as well as significant microcolon, highly suggestive of colonic obstruction. Furthermore, an upper gastrointestinal study with small bowel follow-through was again performed (). It demonstrated patency of the previously created jejunojejunostomy and propagation of contrast into the distal small bowel and proximal colon but was also suggestive of a distal colonic obstruction. Due to a work-up and clinical presentation highly suggestive of a colonic obstruction, the patient was taken back to the operating room on day of life 48 for a second exploratory laparotomy.\nAgain, extensive enterolysis was performed after entrance into the abdominal cavity. The previous jejunojejunostomy with the proximal plication was found to be patent. The bowel was followed to the colon where we discovered type II transverse colonic atresia, which is when the blind ends of bowel are separated by a fibrous cord []. An enterotomy was made in the distal atretic segment and 60cc of normal saline was injected into the distal colon with conformation of patency demonstrated by fluid excreted by the anus. The decision was made to perform a hand-sewn end-to-end anastomosis by spatulating the distal atretic segment. Once this was completed, the abdomen was closed and the patient was transferred to the NICU in a stable condition. The patient tolerated the procedure well.\nThe patient was continued on TPN and his nasogastric tube remained in place. A few days after the final surgery, the patient finally had his first bowel movement. Enteric tubes feeds were introduced slowly as his TPN was weaned. With the introduction of enteric feeds and weaning of TPN, the patient's hyperbilirubinemia gradually resolved. |
A 51-year-old Caucasian male with 4-year history of MS was referred to our neuropsychiatry memory clinic by his family doctor for a cognitive assessment and intervention. His course is best described as relapsing and remitting in nature. He had left upper and lower limb spastic weakness, double vision, jerky eye pursuit, and some cognitive deficits mainly in attention. Depression and anxiety screening was negative, although patient reported frustration with his “slowed thinking.” Patient's MRI showed significant white matter involvement including left prefrontal and parietal white matter, areas that are relevant to attention (). On cognitive assessment using the Montreal Cognitive Assessment scale (MoCA) original English version [] patient scored 23 out of 30 missing one point on letter fluency, 4 points on delayed recall though he was helped with cueing, one point on copying the cube, and one point on sentence repetition. His categorical fluency was 12 animals in one minute while letter fluency was 7 words starting with letter “F” in one minute.\nBased on case reports of benefit of Cholinesterase inhibitors in MS patients [] we offered a trial of Rivastigmine patch which was ineffective. Patient inquired about TMS as an enhancer of brain function. We speculated that enhancing left prefrontal cortical activation may enhance attention. We offered a trial of five daily sessions of high-frequency rTMS applied to left dorsolateral prefrontal location. Although patient reported subjective improvement in “thinking,” there was no significant effect of this intervention on cognitive function except for reduction in the time needed to complete Trail A from 122 to 60 seconds. Patient spontaneously reported walking faster and with less effort. This was in keeping with the finding of faster completion of Trail A, which is largely a speed of processing task. He requested a trial of rTMS for his walking. After we discussed potential benefits and potential known risks including head discomfort, headaches, and seizures [], we obtained an informed consent from the patient as per our institution's standard policy.\nWe used a figure of eight air film-cooled coil attached to Magstim Super Rapid 2 TMS device (Magstim, UK). This coil produces a cone shape magnetic field with a 1 centimeter square peak that can penetrate around 2 cm into the cortex of the brain. Hundred percent of the machine output produces about 3 tesla magnetic field power. The magnetic field passes unimpeded to the cortex and induces a local current. It is thought that this current is frequency dependent and results in modulation of cortical interneurons. A higher frequency magnetic pulse (3 or more cycles per second) is usually excitatory (which enhances glutamatergic interneurons activity whereby it results in lower threshold of the cortical projection neurons) while low frequency magnetic pulse (below 3 cycles per second) is usually inhibitory in nature (which enhances GABAergic interneurons activity and results in higher threshold for firing of cortical projection neurons) [, ].\nWe used 10-20 international EEG lead localization system to identify F\n3 location which usually corresponds to left dorsal lateral prefrontal cortex (L-DLFC) []. Patient received high-frequency 6 Hz rTMS at 90% resting motor threshold, defined as the minimum amount of energy needed to induce a visible muscle twitch in the first dorsal interosseous muscle on more than 50% of the trials while the patient's hands are resting. Each session involved delivering 1200 pulses divided to several trains based on safety parameters built in to MAGSTIM Rapid2 machine (MAGSTIM, UK). In general, treatment was well tolerated except for some local scalp discomfort.\nPatient received one session and three consecutive daily sessions of rTMS treatment separated by 3 days. We administered rTMS using the same parameters for both sessions. Gait data were collected at 2 points of time: (a) before (baseline 1) and immediately after one rTMS session and (b) before (baseline 2) and immediately after 3 consecutive daily rTMS sessions. The gait pattern was assessed during three consecutive trials at a usual gait.\nGait performance was assessed using an electronic walkway system (GAITRite System ) that is 600 cm in length and 64 cm in width. As participants walk along the mat, imbedded sensors are activated by the pressure of their feet and deactivated when the pressure is released. A computer processed the footsteps, providing data for both spatial and temporal parameters. Start and end points were marked on the floor with tape 1 m from either end of the mat to avoid the recording of acceleration and deceleration phases. Participant performed 1 practice trial walking on the mat to familiarize himself with the protocol. Ambulation time (time elapsed between first contact of the first and the last footfalls, measured in seconds), gait velocity (cm/s), stride time (ms), cadence (number of full cycles taken by the pair of feet per minute), and stride time variability (percentage of coefficient of variation (%CoV)), the principal gait measures of interest, were measured over three trials which consisted of walking the length of the mat at a self-selected usual pace.\nA descriptive data analysis (means and proportions) was performed on quantitative data using SPSS software package 21.0 (SPSS Inc., Chicago, IL). We used t-tests repeated measures within subject for multiple comparisons of pre- and post-rTMS sessions. Comparisons were made for outcomes at each time point with respect to the baseline to examine any significant differences. We set the level of statistical significance at p < 0.05. The t-test for paired data was used for statistical analysis.\nOverall, the results indicated that patient had significant improvement in his gait parameters after being treated with rTMS. indicates the gait parameters measured before and after 1 rTMS session and before and after the 3 consecutive rTMS sessions.\nThere were no statistically significant differences before and after 1 rTMS session in ambulation time and velocity; however, cadence was significant (t(2) = −4.99, p < 0.05). Statistically significant differences were found before and after 3 consecutive daily rTMS sessions in ambulation time (t(2) = 8.32, p < 0.05), velocity (t(2) = −4.59, p < 0.05), and cadence (t(2) = −7.57, p < 0.05).\nAnalysis of data showed that stride time variability, measured as the time elapsed between the first contact of two consecutive footfalls of the same foot, was decreased after the 1 rTMS session from 5.02% CoV to 4.6% CoV. However, it was increased after patient received 3 consecutive rTMS sessions from 4.64% CoV to 5.34% CoV; see for graphic representation of the GAITRite data. |
A 55-year-old, Caucasian woman was referred to our tertiary women’s heart center for persistent chest pain, palpitations, and dyspnea. Her medical history included hypertension, dyslipidemia, chronic anxiety, and bilateral non-obstructive carotid atherosclerosis. She had no prior history of diabetes mellitus, tobacco smoking, alcohol or substance abuse, or adverse pregnancy outcomes. Her family history was significant for premature coronary artery disease. Her father had a history of hypertension and had a myocardial infarction (MI) and coronary artery bypass grafting at the age of 39. Her brother had a history of coronary artery disease and also had a MI at the age of 40. Her occupational history indicated that she had been working in the field of psychology and was still an employee in the same job at the time of the hospital visit and follow-up care.\nTable summarizes the general symptoms and characteristic signs of our patient for the onset of CMD and her progression to HFpEF. She had undergone an exercise treadmill test which revealed ischemic ECG changes and dyspnea. Her initial echocardiogram demonstrated a LVEF of 67%, mild diastolic dysfunction, mild left ventricular (LV) hypertrophy, no significant valvular heart disease, and no pulmonary hypertension. Subsequent invasive left heart catheterization was performed and it showed normal epicardial coronary arteries without angiographic evidence of atherosclerotic plaque. She continued to have exertional symptoms and angina-like chest pain and was subsequently referred to our center for further evaluation of suspected INOCA. During her evaluation and treatment she continued to experience stable angina and exertional dyspnea despite initial management with atorvastatin 20 mg daily, lisinopril 20 mg daily, aspirin 81 mg daily, and sublingual nitroglycerin as needed. She had a poor clinical response to sublingual nitroglycerin. Due to her persistent symptoms and abnormal stress testing, she was referred for coronary reactivity testing (CRT) to establish the diagnosis of CMD.\nOur patient underwent invasive CRT, as previously published []. Testing demonstrated normal coronary flow reserve (CFR) in response to intra-coronary adenosine (CFR 3.1; normal ≥ 2.5), abnormal macrovascular endothelial function to intra-coronary acetylcholine (− 6% change in coronary diameter, constriction; normal, dilation), abnormal microvascular endothelial function (coronary blood flow change 48%; normal ≥ 50%), and abnormal non-endothelial function to intra-coronary nitroglycerin (coronary diameter change + 0%; normal dilation) (Table ). She also underwent cardiac magnetic resonance imaging (CMRI) with perfusion imaging at rest and with adenosine stress (140 μg/kg per minute) which showed circumferential subendocardial perfusion defect at stress, normal LV end-diastolic volume indexed to body surface area (EDVi) of 56.4 mL/m2, LV mass index 42.3 grams/m2, and no LV hypertrophy (septum 7.2 mm and lateral wall 6.0 mm). The myocardial perfusion reserve index (MPRI) was 1.8 which was considered borderline abnormal [] (Table ). There was no evidence of myocardial scar.\nThe diagnosis of CMD was established by the coronary endothelial dysfunction observed with invasive CRT, and carvedilol and eplerenone 25 mg daily were added to her regimen. She was followed regularly in clinic with good control of her blood pressure and serum lipid levels. She reported improvement of her angina and dyspnea along with reduction in the duration and frequency of these episodes.\nTen years after her initial diagnosis of CMD, our patient was hospitalized due to symptoms of dyspnea. She was found to have elevated brain natriuretic peptide (BNP) levels of 406 pg/mL and normal LVEF. She had a computed tomography (CT) angiogram of her chest to evaluate for pulmonary embolism, which was negative but revealed bilateral pulmonary edema. She was treated with intravenously administered furosemide for pulmonary edema and diagnosed as having HFpEF. Subsequently, she was discharged with instructions to increase her eplerenone.\nShe continued to experience worsening dyspnea on exertion, orthopnea, and paroxysmal nocturnal dyspnea. A repeat echocardiogram demonstrated normal LV systolic function with an LVEF of 64%, and diastolic dysfunction as evidenced by decreased lateral E′ velocity (4.2 cm/s, indicating impaired myocardial relaxation) and elevated E/E′ ratio 12.9 (suggestive of increased LV filling pressure). She underwent coronary CT angiography which showed absence of coronary atherosclerotic plaque and a coronary calcium score of 0. She was diagnosed as having HFpEF based on clinical symptoms, preserved ejection fraction of 64%, elevated BNP, and evidence of diastolic dysfunction.\nAs part of the WISE – Coronary Vascular Dysfunction (WISE-CVD) Continuation Study (NCT00832702), she underwent a repeat rest-stress CMRI to assess myocardial structure, function, perfusion, and scar, and 13C magnetic resonance (CMR) spectroscopy. Compared to her prior CMRI 7 years ago, she had an increase in LV wall thickness in both the septum and lateral wall (Table ). On CMR spectroscopy, the myocardial triglyceride content was elevated (0.83%) compared to normal control women (mean 0.43%), suggesting myocardial steatosis which is consistent with an ischemia-induced metabolic shift and HFpEF phenotype []. Adenosine stress first pass-perfusion CMRI again showed circumferential subendocardial hypoperfusion (Fig. ) and her MPRI worsened from 1.8 to 1.1, consistent with severe CMD []. There was no evidence of scar on late gadolinium enhancement imaging. |
A 70-year-old obese woman with diabetes who underwent an abdomino-perineal resection due to rectal cancer 20 years ago was admitted to our hospital with a 20-year history of a huge irreducible bulge occupying her left abdomen () and presented with intermittent abdominal pain, abdominal distention, nausea, vomiting and no discharge from her colostomy stoma for 3 days. On physical examination, her abdomen was soft and distended. There was a huge irreducible bulge around the colostomy stoma in the left lower quadrant. A mild tenderness in the bulge was detected with no guarding and rebound tenderness. Abdominal computed tomography showed an abdominal wall defect 10 cm in diameter and a huge mass 25 cm in diameter containing loops of bowel (). Diagnosis of parastomal hernia with intestinal obstruction was established preoperatively. An emergency exploratory laparotomy revealed an incarcerated internal hernia due to an adhesive band extending from the hernia sac wall to distal colon in the huge parastomal hernia (). Two hernia rings were found in this patient: one was around the defect of the abdominal wall (primary parastomal hernia) and the other was composed of an adhesive band resulting in an incarcerated internal hernia. So diagnosis of "incarcerated internal hernia within primary parastomal hernia (hernia within hernia)" was confirmed during the operation. The lysis of the adhesive band and the removal of the distal colon were performed to relieve intestinal obstruction and the colostomy stoma was relocated into the left upper quadrant of the abdominal wall (). Abdominal wall defect repair with prosthetic mesh was not performed due to concerns of incisional infection and consequent removal of the mesh. The patient was discharged uneventfully 2 weeks after the surgery and was readmitted to have further laparoscopic intraperitoneal onlay mesh (IPOM) repair procedure 8 months later.\nMassive abdominal adhesion and two adjoined abdominal wall defects were found by diagnostic laparoscopy during the second procedure. One defect with about 12 × 10 cm in diameter located at the original stoma site while another defect with about 5 × 5 cm in diameter located at the new colostomy site (). A large mesh with a 5 cm orifice through which the stoma was able to go was used with the IPOM technique to cover both defects. Unfortunately, the patient presented with a right lower abdominal pain and high fever three days after the surgery. Some bile-like juice drained out from the abdominal drain tube. An emergency exploratory laparotomy was done revealing an intestinal perforation and an infected fluid collection in the right lower abdominal cavity confined by omentum and adjacent bowels. Fortunately, the intestinal fluid did not contaminate the mesh placed in the left side of the abdominal wall, so we were determined to keep the mesh in place. Resection of the affected portion of the small intestine and an end-to-end anastomosis were performed. After the third surgery, no other complications but incision infection developed in this patient. At 22-month follow-up, there were no evidences of recurrence. |
A 27-year-old African American male without any significant past medical history presented with intermittent palpitations and left ventricular hypertrophy on electrocardiogram (ECG). Two-dimensional (2D) transthoracic echocardiography showed an echodense mass in the left atrium. Further evaluation with Cardiac Magnetic Resonance Imaging (CMRI) demonstrated a heterogeneous broad base mass arising from the posterior superior wall and roof of the left atrium. The mass was located approximately at the expected location of coumadin ridge and measured 1.9 × 1.5 cm and demonstrated mild postcontrast enhancement (). It arises from a diffusely thickened superior posterior wall and atrial roof but did not obstruct the pulmonary venous drainage at this time. Mediastinal lymphadenopathy with similar signal intensity as cardiac mass was noted on fat-suppressed postcontrast axial images (). Lymphadenopathy was more marked in aortopulmonary region.\nPatient was further evaluated with Computed Tomography Angiography (CTA) of chest for better anatomical evaluation of the mass, which confirmed a cardiac region mass which is difficult to distinguish from the mediastinum and is located at the roof and posterior superior wall of the left atrium similar to the description on the CMRI (). The mass demonstrated an infiltrative appearance and may actually originate in the mediastinum, exerting mass effect on to the left atrium. Based on findings of MR and CT, possibility of lymphoproliferative disorder was the primary consideration. PET scan was done to further validate the diagnosis.\n18F-fluorodeoxyglucose positron emission tomography has proven to be a valuable imaging technique for distinguishing neoplastic lesions from benign lesions and evaluating the extent and processes of the disease. It not only demonstrates the complete staging of the disease but also can provide functional information about the disease activity to guide biopsy. FDG PET imaging in our patient demonstrates foci of increased uptake in the mediastinum and right perihilar region, corresponding to soft tissue findings on the previous CT study (). Focal area of uptake was noted near upper pole of left kidney, but, on further imaging by CT and MR of abdomen, no other definite lesion was identified. No other areas of uptake were noted on PET scan. PET scan is also a sensitive indicator for early prediction of treatment response in RDD.\nThoracoscopic-guided biopsy was inconclusive. Imaging done six months later showed no increase in size of mass lesion or mediastinal lymphadenopathy. Decision to surgically excise the mass was done. Left atrial mass demonstrated histiocytes which were immunoreactive to S100. Emperipolesis is engulfment of lymphocytes and erythrocytes by histiocytes which is considered diagnostic of RDD which was noted in the section from left atrial mass (). The left atrial mass showed histiocytes and lymphoplasmacytic cells infiltrates with fibrosis and numerous plasma cells. Patient was discharged home and will continue follow-up with annual CMRI. |
A 56-year-old male with history of AF, status after ablation, eight years ago at an outside hospital, presented with recurrent AF. Five years after his initial ablation, the patient developed symptomatic palpitations due to atrial fibrillation and he was prescribed flecainide and metoprolol. Despite antiarrhythmic therapy, the patient continued to have symptomatic atrial fibrillation with palpitations but no shortness of breath. The patient was taken for AF ablation. The preablation echocardiogram demonstrated normal ventricular function and pulmonary pressures. The preprocedural computed tomography (CT) scan along with the three-dimensional (3D) reconstruction was done on the procedural table prior to the transeptal puncture but did not pick up the pulmonary vein stenosis. Pulmonary vein potential mapping noted that there was a potential at the ostium of the left superior pulmonary vein. There was also difficulty advancing the catheter into the left superior pulmonary vein due to a possible obstruction. Direct angiography of the left superior pulmonary vein (LSPV) confirmed complete PV stenosis. The procedure was aborted in lieu of further diagnostic work up. A higher resolution cardiac CT angiogram () and left atrium 3D reconstruction () was used to confirm subtotal occlusion of the left superior pulmonary venous trunk. A lung perfusion scan revealed significantly decreased left upper lung perfusion () with the left lung contributing approximately 18% of the lung function and the right lung contributing 82% of the total lung function. A levophase angiogram of the right middle lobe pulmonary arterial system demonstrated venous return confined to the area of the lung supplied by the arterial vasculature. The levophase pulmonary angiogram demonstrated well developed collateral circulation from the left upper lobe to the mid segment of the left lung (Figures and , resp.). After cardiac workup, it was concluded that this pulmonary vein stenosis is chronically totally occluded and less amenable to percutaneous intervention for reestablishment of flow. Ultimately, the decision to intervene to relieve the stenosis and to repeat AF ablation on the remaining pulmonary veins was left to the patient. The patient initially opted for continuance of antiarrhythmic drug therapy. However, due to persistence of breakthrough atrial fibrillation despite antiarrhythmic therapy, the patient ultimately underwent repeat atrial fibrillation ablation procedure two years after his initial presentation to our institution. |
Case reports are exempt from institutional review board approval at our institution.
A 75-year-old woman was evaluated for long standing right-sided nasal obstruction, dependent mouth breathing, clear rhinorrhea, congestion, and hyposmia not relieved by intranasal steroids or nasal irrigations. She initially presented with a history of previous endoscopic sinus surgery in Russia more than 20 years ago followed by nasal polyposis treated with ambulatory cauterization. She was also noted to have a history of nonmelanotic skin cancers of the nose treated with radiation in Russia.
Physical exam findings included a fleshy intranasal lesion that, in the setting of previous nasal skin cancer treated with radiation, raised a concern for possible secondary carcinoma. Computed tomography showed complete opacification of the right maxillary sinus, obstruction of the right ostiomeatal complex, and soft tissue density in the right nasal passage ().
Initial biopsy revealed a nasal mass that originated in the right inferior meatus. Pathology showed multiple polypoid fragments lined by a respiratory type epithelium with underlying edematous stroma with mild chronic inflammation. There was invagination of the surface epithelium into the underlying stroma resulting in nested aggregates of bland glandular and mucinous cells and focally benign squamous epithelium (). These features were found to be consistent with a benign inverted papilloma.
The patient presented with continued nasal obstruction and was evaluated for definitive treatment. Given her diagnosis of inverted papilloma and chronic rhinosinusitis, complete excision and revision endoscopic sinus surgery was recommended. During endoscopic sinus surgery, an exophytic mass with abnormal maxillary mucosa was seen emanating from the left inferior meatus that was thought to originate from the right maxillary sinus, given that it was protruding through a bony dehiscence into the inferior meatus and nasal cavity. Right partial inferior turbinectomy was performed, along with right extended maxillary antrostomy and stripping of maxillary mucosa to remove the entirety of the presumed inverted papilloma base within the maxillary sinus. Intraoperative and postoperative pathology again showed inverted papilloma.
Given |
A-13 year old girl was referred to the radiology department of French Medical Institute for Mothers and Children (FMIC) for taking plain radiographs of the axial and appendicular bones. The patient had short left leg with abnormal swelling and deformity of fingers and left toes. The swelling were hard in palpation however no pain was noticed. The overlying skin appeared normal. In radiographic images, multiple expansile lytic lesions (enchondromas) were noted in the metacarpi and phalanges of hands with sparing of the index finger of right hand associated with multiple lytic lesions in the distal ulna and radius of both hands with sparing of left distal ulna (Fig. ). Chest plain image demonstrated multiple enchondromas in the anterior end of the ribs bilaterally as well as proximal metaphyses of both humeri, extending into right humerus diaphysis with multiple punctate like calcifications (Fig. ). Enchondromas were also noted in the metatarsi and toes of left foot, left distal tibia and fibula in plain image of left foot (Fig. ). Multiple lytic lesions with punctate calcifications were also seen in the left iliac bone and proximal metaphysis of left femur in anterior posterior radiograph of pelvis bone (Fig. ). Plain image of left calf showed multiple foci of calcifications with lytic lesion in the left distal femur as well as proximal and distal metaphyses of left tibia and fibula (Fig. ).\nAn 8 year- old boy was referred to our radiology department for taking radiographs of left leg and pelvis bones. The patient had left leg discrepancy with bowing deformity without specific pain. The lateral radiograph of the left leg demonstrates bowing deformity with enchondromas in the distal femur as well as proximal and distal ends of left tibia and fibula (Fig. ). The anterior posterior radiograph of pelvis and both lower extremities showed lytic lesions in the proximal and distal ends of left femur, tibia, fibula and left iliac bone with deformity and significant left leg discrepancy (Fig. ). |
An 88-year-old female presented to the emergency department with abdominal pain and vomiting. She was mildly tender in her right upper quadrant but was focally more tender in her right lower quadrant. Her blood results showed an elevated white cell count of 12.9 × 109 l–1 and a neutrophil count of 10.4 × 109 l–1. Furthermore her C-reactive protein level was 34 mg/L. An abdominal ultrasound showed multiple calculi within the gallbladder. Gallbladder wall thickness was not assessed. A non-contrast CT scan, due to the patients impaired renal function, was then ordered as the patient’s physical exam findings were not in keeping with a diagnosis of cholecystitis.\nThe CT showed abnormal dilatation of the proximal small bowel with a possible transition point in the left iliac fossa. Pneumobilia was also noted due to prominence of the hepatic biliary tree (). No evidence of a ductal calculus was observed. The CT scan was reported as showing features of a bowel obstruction with a transition point in the left iliac fossa. The pneumobilia was reported as being possibly an indicator of previous sphincterotomy. There was no evidence of appendicitis or another inflammatory process in the right iliac fossa.\nThe following day, while proceeding with conservative management, the treating team requested a small bowel follow through to investigate the obstruction. After further review and discussion with the treating team it was revealed that there was no history of an endoscopic retrograde cholangio-pancreatography or cholecystectomy. An addendum was added to the CT report, suggesting that the appearances were indicative of a gallstone ileus from a choledochoduodenal fistula () with a non-calcified gallstone possibly being present at the transition point in the left iliac fossa ().\nThe patient underwent a laparotomy and a gallstone was found obstructing the distal jejunum. A 41 mm gallstone was removed by vertical enterotomy, later confirmed on histological diagnosis. The patient had an uneventful recovery in hospital and was discharged a few days later to a regional hospital for rehabilitation. A cholecystectomy or fistula closure was not performed and she was not referred for further surgical follow-up on discharge, likely due to the patient’s advanced age and medical comorbidities. |
We report the unique case of a six-year-old female treated for an extensive venolymphatic malformation noted at birth. The lesion was centered around the left axilla as well as chest wall and also involved significant areas of the neck and posterior trunk (Figure , ). The patient also had lesions on bilateral posterior scapular regions and the left breast. At the age of two, she underwent combined surgery with pediatric and plastic surgery where the axillary/chest wall lesion was resected along with significant neurolysis off the brachial plexus and flap closure.\nSurgical pathology of the mass revealed a 25.5 cm x 17.5 cm x 7.0 cm combined venous and lymphatic malformation with areas of cystic formation, myxoid change, and focal sclerosis. The pediatric surgeon at our institution deemed the debulking surgery of the lymphangioma to be successful and recommended waiting until the age of five to resect the residual neck and back disease. This was advised due to the encasement of neurovascular structures within the residual malformation, including the brachial plexus. Additional recommendations included postponing resection of the left breast lesion until breast development was complete, to avoid disrupting the developing breast bud.\nThe patient presented again to our institution at the age of six for follow-up. Physical exam revealed bilateral, mobile, subcutaneous lesions in the scapular region raising suspicion for large lipomas (Figure ). In addition, asymmetry of the upper extremities was noted in the left forearm (Figure ) and hand twice as large as that in the right forearm (Figure ). Given the constellation of symptoms, a diagnosis of CLOVES was suspected. Preoperative magnetic resonance angiogram (MRA) was obtained of the neck, chest, and abdomen. MRA revealed multiple small foci of microcytic lymphatic malformations in the chest at the site of prior resection with irregularity of the left subclavian vein with absence of the left axillary vein and multiple collateral vessels in this area concerning for thrombosis. The patient underwent successful excision of the lipomatous overgrowths. Pathology demonstrated a 9.0 cm x 6.0 cm left and 7.0 cm x 4.0 cm right lipomatous overgrowth. Outpatient genetics consultation was scheduled.\nGenetic consultation revealed that the patient had a history of developmental delay and did not walk until 18 months of age or talk until age three. However, this has not significantly impacted the patient’s current school performance. There is no family history of significant genetic illness, consanguinity, lipomatous overgrowths, macrocephaly, or significant nevi. Physical examination showed appreciable immobility of the left-upper extremity and neck asymmetry with the left shoulder inferior to the right with bilateral shoulder sloping. Skin examination showed a left-sided breast mass, soft and mobile to palpation, with skin hyperpigmentation, one café-au-lait spot on the right flank, and raised areas of hyperpigmentation over the left breast mass, and surrounding scar of the previously resected lymphangioma. Her head circumference was normal for her age, and no nevi were noted aside from the café-au-lait spot.\nDefinitive diagnosis of CLOVES syndrome was made after genetic testing revealed a heterozygous, pathogenic, missense variant in the PIK3CA gene, with positive testing of the affected tissue and negative testing of her peripheral blood. The patient also underwent a postoperative cardiac workup, which was negative for any structural abnormalities of the heart. |
We present the case of a 62-year-old male with advanced melanoma who followed an unconventional treatment path (see ). The patient was diagnosed with stage III BRAFV600E LDH normal melanoma of the right chest in June of 2011. Upon diagnosis, 4 axillary lymph nodes were positive on axillary lymph node dissection. The patient initially decided to forego treatment; however, imaging 9 months later showed numerous subcutaneous and pulmonary metastases. The patient began treatment with BRAF inhibitor vemurafenib in April of 2012. Subsequent serial PET/CT scans two months later indicated mixed response to therapy, with resolution of pulmonary and numerous subcutaneous nodules with the development of multiple new nodal and subcutaneous lesions. Throughout the following two years of vemurafenib therapy, this pattern of new and resolving nodal and subcutaneous metastases continued. Due to an overall decrease in disease burden and patient preference, treatment was continued. In September of 2014, vemurafenib was discontinued due to CT and MRI indicating significant metastases in the brain and bones. The patient completed a course of radiotherapy to L4 and L5 lesions as well as radiosurgical and radiotherapy treatment for the brain metastasis with good response. He was then started on ipilimumab for systemic therapy. After 4 cycles, CT and MRI demonstrated stable disease. Immunotherapy was held due to a period of colitis, but on resolution, a maintenance dose of ipilimumab was given. However, due to significant cutaneous metastasis, ipilimumab was discontinued in late May of 2015, and the decision was made to proceed with wide excision of the subcutaneous masses and hold systemic therapy with reimaging in 6 weeks. At that time, there were no new cutaneous metastases and intracranial/osseous disease was stable. Follow-up imaging was scheduled for another 6 weeks, which again showed stable disease even with continued hold of systemic therapy. At this point, follow-up MRI brain and CT abdomen/pelvis were scheduled for 3 months later.\nFollow-up imaging was not completed until January of 2016 but showed stable intracranial and osseous disease with multiple new cutaneous metastases. The lesions were excised, and systemic treatment options were discussed at a tumor board. The patient was lost to follow-up until mid-June 2016, where repeat CT indicated significant progression of cutaneous metastasis. Given continued cutaneous progression, single-agent pembrolizumab was started in June of 2016. The patient had an initial mixed response to pembrolizumab with overall stable disease and remained on the therapy for 9 months. Subsequent PET/CT scan in March of 2017 showed an increased number of pulmonary nodules and approximately 7 new subcutaneous lesions on the patient's legs bilaterally, and pembrolizumab was discontinued.\nAfter discussion, ipilimumab/nivolumab combination therapy was started in April of 2017. After 4 cycles, a PET/CT demonstrated stable disease with the exception of a new subcutaneous metastasis on the ankle. Given the history of extensive treatment and lack of significant progression, single-agent nivolumab was continued and the lesion was treated palliatively with radiation therapy. Imaging in October of 2017 indicated a mixed response, with stable visceral disease but progression in the form of multiple new subcutaneous metastases in the back and right thigh. Lack of open slots in appropriate clinical trials led to continued therapy with nivolumab beyond progression. However, after imaging in December of 2017 showed continued subcutaneous progression, single-agent nivolumab was discontinued. The patient was then started on a clinical trial with intralesional SD-101+systemic pembrolizumab from January 2018 to March 2018 until progression.\nSince it had been over 3 years since the patient trialed BRAF inhibitor therapy and the patient had never been treated with BRAF inhibitor/MEK inhibitor combination therapy, dabrafenib/trametinib was initiated. The patient first began dabrafenib and trametinib in March of 2018 and had an impressive response with substantial shrinkage of subcutaneous lesions within a few days. In July of 2018, PET/CT showed near-complete response to treatment, with resolution of the pulmonary and subcutaneous nodules (). Several areas of hypermetabolic subcutaneous infiltration were seen throughout the body consistent with an inflammatory dermatologic reaction. Response persisted until unfortunately in November of 2018, CT showed evidence of progression with new nodal and soft tissue lesions as well as a single hepatic lesion concerning for metastasis. Dabrafenib/trametinib combination therapy was discontinued.\nGiven the fact that the patient progressed on all standard lines of treatment and there was no eligible clinical trial available at the time, we decided to proceed with encorafenib/binimetinib combination therapy. The patient was on encorafenib/binimetinib from December 2018 through April 2019 with partial response, until unfortunately imaging indicated progressive disease with new left external iliac nodal metastasis and soft tissue deposits.\nAs the patient had exhausted and progressed on all standard lines of treatment and no clinical trial was available for him at the time, we discussed potential treatment with nab-paclitaxel chemotherapy or retrial of ipilimumab/nivolumab immunotherapy. The patient had shown response to ipilimumab/nivolumab therapy in the past, with progression in the maintenance phase. This context was paired with data from studies suggesting the potential for immunotherapy after BRAF inhibition owing to favorable modulation of tumor microenvironment [, ]. The patient decided to proceed with combination ipilimumab/nivolumab immunotherapy and began treatment in April of 2019. PET/CT in late-July 2019 showed mixed response to therapy, with a significant decrease in nodal and subcutaneous FDG avidity with a stable small left hepatic lobe lesion that was previously noted in November 2018 (). After 5 treatment cycles, CT imaging in September 2019 showed a decrease in nodal metastasis size, a stable hepatic lesion, and no evidence of pulmonary disease. CT imaging completed 11/2019 showed no change in the nodal or hepatic lesion, indicating stable disease. |
This is the case of a 50-year-old female, with a family history of pancreatic cancer, who was found to have a chest lesion on chest X-ray carried out in 2006 because of intermittent cough since 2000. A subsequent CT scan of the thorax confirmed a 13-mm nodule on the upper right lobe of the lung, which did not seem suspicious for malignancy. Following a FDG-PET, which did not pick up on the nodule (SUV max 1.25), observation of the lesion was decided with yearly scans. Up until 5 years later, the lesion seemed to have remained unchanged. However, the imaging follow-up of December 2010 showed a new lesion in the right breast. In January 2011, she underwent right breast quadrantectomy for a lump histologically perceived as an infiltrating triple negative lobular carcinoma, with a negative sentinel node. This result was confirmed from a second pathologist later on. Surgery was followed by adjuvant chemotherapy and radiotherapy, completed in July 2011. In August 2012, a left breast nodule was detected on mammogram and confirmed on MRI. Biopsy of the lesion resulted in a triple negative invasive ductal carcinoma. A PET/CT with FDG was carried out which surprisingly showed a high uptake only on the old pulmonary nodule, which had increased in the meantime, becoming 3 cm in diameter (SUV max 5.9). Biopsy of the lesion was suspicious for a carcinoid. In June 2012, the patient underwent a right lobectomy and right hilomediastinic lymph node dissection. Histology reported a neuroendocrine neoplasm with Ki67 of 18%. Clinically, the patient showed PD from the left breast with subcutaneous nodules formation. It was decided to proceed to a bilateral mastectomy with subcutaneous nodules removal in July 2012. The histological result showed a neuroendocrine tumour, Ki67 of 12% and negative left sentinel node. A second pathology opinion was concluded for breast metastases and subcutaneous nodules from a moderately differentiated NEN of pulmonary origin, with Ki67 of 14% positive for CgA and TTF-1 and negative for calcitonin and somatostatin receptors (SSTRA2), concluding for an AC (). A follow-up 68Ga-PET/DOTATOC, CT with contrast medium and MRI showed further lesions on left and right femur. Biopsy of the left femoral lesion confirmed a metastasis from the same moderately differentiated NEN with Ki67 of 16% with negative somatostatin receptor (SSTR). In view of disease progression, it was decided to start chemotherapy in January 2013 with capecitabine (1,500 mg/m2/die for 14 days) and temozolomide (150 mg/m2 for 5 days). The patient tolerated well the first six cycles which were stopped due to thrombopenia (grade 3) and malaise and were switched to subcutaneous (SC) octreotide LAR 30 mg/die. A follow-up CT scan showed a new pulmonary nodule of 3 mm on the left and an ultrasound of the thyroid revealed nodule on the right lobe of 15 × 12 mm, which was aspirated and confirmed the metastatic nature of the primary pulmonary lesion. The patient was reluctant to undergo for further chemotherapy at that time, and thus decided to continue with the somatostatin analogue and re-evaluate the situation in few months. In August 2013, her disease was clinically and radiologically stable. |
A 52-year-old woman presented with pain and active function loss in her left shoulder, and was admitted to our hospital in June 2017. Her shoulder problem had started 3 months earlier, and there was no history of trauma or fracture. At first the pain was intermittent and bearable, but then gradually increased. On examination, there were no positive signs except for localized pain. A radiologic examination on 3 April 2017 found no destruction of the shoulder (Fig. ). Pain at the shoulder joint became gradually aggravated, together with the appearance of shoulder joint dysfunction. Two months later, physical examination revealed mild swelling of the shoulder, and markedly restricted shoulder and elbow motion. Mild distal nerve function defects appeared gradually. Radiography on 3 June 2017 showed that the head of the humerus had disappeared within the past 2 months (Fig. ), which was confirmed by magnetic resonance imaging (Fig. a, b).\nThe patient is a healthy, active individual with no history of weight loss, anorexia, or fever during this period. General and systemic examination findings were within normal limits. Routine laboratory investigations were also normal, including levels of serum calcium, phosphate, alkaline phosphatase, high-sensitivity C-reactive protein, and erythrocyte sedimentation rate. An open biopsy of the lesion revealed that the bony tissue had been replaced by fibrous connective tissue, and small areas of bony trabeculae with occasional osteoclasts were visible (Fig. ). There was no evidence of malignancy or tuberculosis. Because of the lack of any clinical findings or supporting data for other causes, the features were confirmatory of GSD. Computed tomography of the shoulder joint (Fig. ) revealed a bony defect of the glenoid cavity.\nWe performed surgery involving glenoid cavity amplification with an autologous iliac bone graft and a reverse total shoulder arthroplasty. The grafts were from autologous iliac bone (Fig. , Fig. ). A deltopectoral approach was used on the shoulder, and necrotic and dissolving bone tissue was removed. Reconstruction of the glenoid was carried out with autologous iliac bone and installation of reverse shoulder prosthesis. Postoperatively, the arm was placed in a sling for 3 months. Passive elevation and external rotation were allowed 2 weeks after the operation. Three months later, sling use was discontinued, and active range of movement was initiated. Six months after surgery, the patient is pain-free with more than 90° of active abduction, 100° of forward flexion, and 30° of shoulder posterior extension. She also has good functional use of her shoulder (Fig. , Fig. ). |
A 24-year-old healthy woman, without a significant medical family history, presented with a lump in the lower inner quadrant of her left breast 24 months prior to her referral to our center. At that time, the mass was diagnosed as a benign lesion after incisional biopsy. She became pregnant and about 1 year after child delivery, the left breast mass grew significantly larger. On clinical suspicion of malignancy with global infiltration of the left breast, she was referred to our center. Mammography showed an asymmetric increased density in the left breast. Multiple-site core needle biopsy was performed, but it led to no definite pathological diagnosis. Consequently, the patient was scheduled for open biopsy. Under general anesthesia, the mass was entered via an incision on the most prominent site. After the removal of a 2×2 cm tissue biopsy, a cavity was opened beneath the excised tissue which was full of blood clot. Following the removal of the clot, active bleeding occurred. Bleeding was diffuse and uncontrollable, such that within a few minutes, the patient lost about 1500 cm of blood. Effective packing was performed. The next day, after the patient had stabilized, she was retransferred to the operating room for the removal of the pack. However, due to severe bleeding after the removal of the pack, packing was reapplied. Chest computed tomography scan revealed a large, well-enhanced mass in the left breast without invasion to the skin or pectoralis muscles and also enlarged left axillary lymph nodes ().\nAccording to the final pathology report, histological sections of the mass demonstrated a vascular tumor with interconnecting blood channels lined by atypical, pleomorphic endothelial cells. Mitotic figures and areas of necrosis were also seen (). The tumor cells were immunoreactive for CD31, CD34, and vimentin but not for cytokeratin. The Ki-67 labeling index was about 30%.\nAs the pathology study of the sample was suggestive of an angiosarcoma, the patient underwent left-side simple mastectomy and then was planned to undergo adjuvant chemotherapy. |
RS, a 12-year-old female child was brought by her mother to dermatology outpatient department (OPD) with multiple monomorphic geometric erythematous lesions with sharp margins on anterolateral aspects of both her arms of sudden onset. There were no complaints of any pain or discharge from the lesions. There was no history of any physical or chemical injury. There were four lesions on both the arms at similar anatomical location with exactly similar morphological appearance [Figures and ]. The findings of histopathological examination were nonspecific. The lesions had appeared on 8–10 instances in last 1 year and had healed in 1–2 weeks time. The first episode had occurred, whereas the child was in school and she complained about this on coming back to home. She reported of utter ignorance about the cause of the lesions. Multiple medical consultations resulted in investigations such as hemogram, platelet count, fasting blood glucose, and all within normal limits. The nature of the lesions and course prompted a diagnosis of dermatitis artefacta and the child was referred to psychiatry OPD.\nThe parents were reluctant for psychiatry consultation asking about the relation of skin disease with psychiatry. The initial barrier was overcome when the child and her parents were reassured that she had been asked for psychiatry consultation for the evaluation and management of “stress” which might be manifesting as a skin eruption.\nRS was the younger daughter of a physics lecturer in a government college. Her elder sister was extremely bright in academics and preparing for board examinations. The father was engaged in teaching the elder sister as he had high expectations from her. He had little time left for RS. RS being a naughty child was not getting enough time and attention, her academic grades were persistently falling. RS was often rebuked by her father due to her poor scholastic performance which would make her extremely unhappy.\nThe birth history of RS was uneventful and developmental milestones were attained at age appropriate levels. RS talked of her difficulties in studies and feelings of loneliness and inadequacy on being scolded by her father for not performing well in studies. She talked about the difficulty in focusing on studies since her primary school days and inability to organize her tasks which would often result in late submissions of her work. The support which was provided by her father had also faded in the last 1 year which was adding to her problems. She denied pervasive feelings of sadness or anxiety. Her parents corroborated her difficulties. The child fulfilled criteria for ADHD, predominantly inattentive type as per Diagnostic and Statistical Manual of Mental Disorders Fifth Edition and with a total score of 32 on Vanderbilt ADHD diagnostic parent rating scale with positives on items about inattention.[] She was prescribed with tablet atomoxetine 10 mg and is under regular follow-up. |
An 88-year-old female presented to the emergency department with abdominal pain and vomiting. She was mildly tender in her right upper quadrant but was focally more tender in her right lower quadrant. Her blood results showed an elevated white cell count of 12.9 × 109 l–1 and a neutrophil count of 10.4 × 109 l–1. Furthermore her C-reactive protein level was 34 mg/L. An abdominal ultrasound showed multiple calculi within the gallbladder. Gallbladder wall thickness was not assessed. A non-contrast CT scan, due to the patients impaired renal function, was then ordered as the patient’s physical exam findings were not in keeping with a diagnosis of cholecystitis.\nThe CT showed abnormal dilatation of the proximal small bowel with a possible transition point in the left iliac fossa. Pneumobilia was also noted due to prominence of the hepatic biliary tree (). No evidence of a ductal calculus was observed. The CT scan was reported as showing features of a bowel obstruction with a transition point in the left iliac fossa. The pneumobilia was reported as being possibly an indicator of previous sphincterotomy. There was no evidence of appendicitis or another inflammatory process in the right iliac fossa.\nThe following day, while proceeding with conservative management, the treating team requested a small bowel follow through to investigate the obstruction. After further review and discussion with the treating team it was revealed that there was no history of an endoscopic retrograde cholangio-pancreatography or cholecystectomy. An addendum was added to the CT report, suggesting that the appearances were indicative of a gallstone ileus from a choledochoduodenal fistula () with a non-calcified gallstone possibly being present at the transition point in the left iliac fossa ().\nThe patient underwent a laparotomy and a gallstone was found obstructing the distal jejunum. A 41 mm gallstone was removed by vertical enterotomy, later confirmed on histological diagnosis. The patient had an uneventful recovery in hospital and was discharged a few days later to a regional hospital for rehabilitation. A cholecystectomy or fistula closure was not performed and she was not referred for further surgical follow-up on discharge, likely due to the patient’s advanced age and medical comorbidities. |
A 22-year-old male patient in excellent health reported with tooth fracture in maxillary anterior jaw region 11# and the clinical crown had been lost (). The patient’s medical history was unremarkable and on dental hard tissue examination there were no other significant findings. Periodontal status- oral hygiene was good. No marginal gingival inflammation was detected. No periodontal pockets were present, no other tooth mobility found. The patient desired fixed type replacement which preserved the neighboring healthy teeth.\nAfter the clinical examination and review of the initial periapical radiograph, the possibility of an extraction of the remaining root structure of tooth 11# and subsequent implant placement seemed feasible. The patient was advised of the potential benefits of a CBCT scan, this information would yield the information necessary to recommend the optimal treatment plan, based on a comprehensive assessment of the bone for the potential implant placement. Based on this decision was made to take CBCT scan.\nThe cross-sectional CBCT data was visualized which revealed that the thickness of the palatal and buccal plate were intact (). There were no deficits in the bony structure around the entire circumference of the residual root. Cross-sectional image dictated the existing tooth position in relation to the surrounding bone. When we viewed the proposed recipient site, it was realized that the anatomy was ideal for immediate implant placement, and the amount of available bone apical to the existing tooth which could be utilized to provide stability for the immediately placed implant was adequate.\nPatient’s consent was taken, and Study cast model was prepared.\nInitial phase 1 therapy was performed. Supragingival and subgingival scaling was done in all quadrants.\nAt the day of surgery, the patient was prepared and draped. The patient was appropriately anesthetized with local anesthesia. The remaining root 11# was atraumatically removed (). The socket was checked for any residual granulation tissue. Osteotomy site was marked. 2.0 diameter pilot drill was used to start the bone preparation, labiolingual and mesiodistal angulation was assessed using paralleling pins. The osteotomy was done in a sequential manner. Implant site was flushed with normal saline and betadine to remove any debris and 4.0 diameters and 13 mm length implant was placed in the osteotomy site (). The site was closed. Immediate RPD was delivered. CBCT was done after 4 months of implant placement, and 3D peri-implant osseointegration was analyzed (). The final prosthesis was delivered after 6 months of the implant placement (). |
An 18-year-old male patient having indwelling catheter in situ reported with the complaint of inability to move his legs and no sensation below his navel []. He also told that his legs seemed to be cold. He was having loss of appetite. He could not hold the urge of defecation.\nOn asking about the history of the same, he stated that he was alright till evening of 8 April 2010 and then he felt feverish and developed pain all over the body. He took some medicine for the same from his family doctor. He woke up late in the morning and was unable to stand up by himself. He stood with support and tried to walk. During the walk, he felt that his legs were not in his full control. He had not voided any urine since morning. In the evening, along with his relatives, he again consulted the other doctor who then referred him to higher center.\nHe got admitted to the hospital on the next day with the complaint of no sensation in lower half of the body as well as insensitivity to touch from lower part of neck to toe. He was also having the feeling of tightness around the abdomen.\nLumbar puncture was done on the same day, which showed increased polymorphs. On 15 April 2010, CT-head was done and it was normal. He also faced some respiratory problems and so was on intermittent oxygen inhalation therapy from 12 to 18 April 2010. On 17 April 2010, MRI cervico-dorsal spine with MRI brain was done, findings of which were as follows.\nLong segment T2 hyper intensity of the cervicodorsal spinal cord from the C3 level onward. Suggestive of myelitis.\nTherefore, the present case had been diagnosed as that of transverse myelitis and came to us after two months of acute attack. At that time, he was having total paraplegia, no sensation below umbilicus, and other typical features of transverse myelitis such as bladder–bowel dysfunction, feeling of tightness around abdomen, and a bed sore on the sacral region.\nSome of the above-mentioned presentations of the patient resembled the sign and symptoms of a disease named as Urustambha (spasticity of thighs) in Ayurvedic classics.[] On having a critical view, some of the features of transverse myelitis and Urustambha are found very close to each other and can be correlated[–] []. On the other hand, many other features of transverse myelitis and Urushtambha are also there that cannot be correlated[] []. |
The patient is a 52-year-old right-handed gentleman, who initially presented in 1998 with headaches that were thought to be related to sinus infections. Initial work up consisted of X-ray of the skull, which demonstrated a dense calcified lesion in the left frontotemporal region []. This study was followed by a magnetic resonance imaging (MRI), which demonstrated a densely calcified lesion in the frontotemporal region abutting the sylvian fissure. After neurosurgical consultation, conservative management was adopted. The lesion was deemed stable according to the patient, however, images and reports are not available for long periods of this interval.\nThe patient presented again in 2014 with similar headaches, again thought to be caused by sinus infection. At this time, an otolaryngologist ordered a computed tomography (CT) scan, which demonstrated growth of the mass []. This was verified by MRI, which also showed an increase in surrounding edema with transtentorial herniation and mass effect on the midbrain []. With the exception of headache, the patient remained otherwise asymptomatic. While surgery was advised, continued surveillance was adopted showing progressive enlargement, with the onset of very subtle circuitous speech. Surgery was strongly recommended, to which he ultimately consented.\nCT angiography (CTA) was obtained demonstrating close proximity of the mass to the left cavernous and ophthalmic internal carotid artery, as well as M2 branches of the middle cerebral artery (MAC), without obvious stenosis, involvement, or entrapment of these vessels. While his hearing was intact and face symmetric, there was an unclear relationship of the mass to the geniculate ganglion and cochlea on immediate preoperative imaging studies [].\nIn 2016, approximately 18 years after the discovery of this mass, the patient underwent surgery for resection of this lesion through a frontotemporal craniotomy and craniectomy, and zygomatic osteotomy to maximize our access to the middle fossa floor. We sectioned the zygoma and reflected the temporalis inferiorly after subfascial dissection to gain adequate access to the floor of the middle fossa. We used neuronavigation to identify the mass emanating from the inner table, placing burr holes immediately around this region and connecting these. This allowed us to separate the mass from the larger frontotemporal craniotomy flap, which we then elevated. We used a high-speed drill to remove the extradural portion of the mass which at the superficial depth of the sylvian fissure became intradural. The MCA branches were easily dissected from the lesion and the middle fossa dura found to be intact. After a complete resection we replaced the frontotemporal flap, reconstructing the central portion with titanium mesh. Postoperative imaging showed a complete resection [] and he was discharged in excellent condition on the second postoperative day. Despite analysis of the frozen section raising a concern for meningioma, permanent analysis showed FD []. |
A 58-year-old woman with no history of cardiac disease was introduced to our hospital with atypical chest pain at rest and before sleeping at night for a month. Her risk factors for coronary artery disease were obesity and dyslipidemia, and she was administered with statin by a local clinic. On clinical examination, she had no murmur, and both chest X-ray and resting electrocardiogram were normal. We performed TTE and 64-slice MDCT (Aquilion 64, Toshiba Medical Systems, Japan) since treadmill exercise test indicated positive finding for myocardial ischemia. TTE revealed continuous flow into the main pulmonary artery which had peak flow in the diastolic phase. MDCT was performed with a retrospective ECG-gated protocol and with a collimation of 64 × 0.5 mm, detector pitch of 11.2, gantry rotation time of 350 ms, tube current of 400 mA, and tube voltage of 120 kV. She received 2 mg propranolol hydrochloride and sublingual nitroglycerin before scanning, and 59 mL of contrast medium (370 mg iodine/mL) was used for MDCT angiography. Axial images demonstrated the leakage of contrast medium into the main pulmonary artery from the aberrant artery originating from coronary arteries (), and we could not detect any other leakages of contrast medium in the pulmonary artery. In addition, three-dimensional volume-rendered images revealed the network of aberrant arteries arising from both left anterior descending coronary artery and right coronary sinus (). From these TTE and MDCT findings, we were able to diagnose her disease as coronary to pulmonary fistulas. Furthermore, these fistulas proved to be dual fistulas originating from the left anterior descending coronary artery and right coronary sinus to the same site of the main pulmonary artery. Subsequently, ICA confirmed these fistulas (), but we could clearly demonstrate the course and the termination of the fistulas more with MDCT. She was not referred to surgical or percutaneous treatment, because the left-to-right shunt calculated by TTE and cardiac catheterization was not significant and the absence of pulmonary hypertension, heart failure, or myocardial ischemia was detected by radionuclide myocardial perfusion imaging. |
The patient is a 19-year-old girl, a known case of RRMS starting from 5 years ago. She was well until the time of the MS diagnosis, about 5 years ago. With the disease diagnosis, subcutaneous interferon beta 1-a was started three times a week for the first 3 years of her disease and after that switched to fingolimod 0.5 milligram daily owing to new clinical and radiological relapse. She was stable during fingolimod consumption without disease activity (clinical and imaging aspects). However; she was tiered of taking medicine and discontinued her drug on her own. About 2 weeks after cessation of fingolimod, she developed acute onset of hearing loss and tinnitus in her right ear after cessation of fingolimod for 2 weeks. There was no history of vertigo, headache or ear pain, recent trauma to the head, otitis media, upper respiratory or other viral infections, and family history of hearing loss. She also did not use new medication with ototoxicity side effect in the previous weeks. She was on the MS DMDs and was well since the time of the MS diagnosis, about 5 years ago, until 2 weeks before the onset of the hearing loss (subcutaneous interferon 1a three times a week for 3 years and then daily oral fingolimod because of new clinical and radiological relapse). General and neurological examination, especially of the head and neck parts, was not significant except for a decrease in hearing in the right ear at the time of the hospital admission. On otoscopic evaluation, she had intact bilateral tympanic membranes. After consulting with otolaryngologist and completing audiometric examination and work-up, SNHL was diagnosed. The results of the audiometric tests before and after treatment are demonstrated in (). Routine laboratory tests, autoimmune profiles and vasculitis markers all showed to be normal. New brain MRI showed one new T2 lesion in the right side of the pons and 2 small gad enhancing lesions in the supratentorial area ().\nThus, with impression of probable new MS attack resulting from fingolimod cessation due to both clinical and radiological activity, she received 5 grams of intravenous (IV) methyl prednisolone and improved significantly with the pulse of the corticosteroid therapy. |
A 22-years-old female who following a stressful family event and an abortion, had to move away from her family and lived under difficult situations, following that she noticed severe weight loss. This was treated with supportive measures and her general health improved. Then she developed severe fear, anxiety, irritability, and sleeplessness for which she was suspected as having reactive depression secondary to family stressors. While on treatment she came to NIMHANS with weakness of right sided limbs with inability to speak. Examination revealed thin built patient who was pale. Her blood pressure was 70/46 mm of Hg. She had right hemiplegia with global aphasia. Her fundus showed mild temporal pallor and she had pigmentation over both legs and sparse hair. There was history of mild intermittent fever on questioning, which improved with symptomatic measures. There was history of another episode of transient left sided weakness in the past which improved on its own and was ignored. Patient underwent investigation for vasculitis including skin biopsy. Her skin biopsy specimen showed oedema of Capillary dermis with scattered lymphocytes and histocytes that are also seen in the deeper dermis surrounding hair follicles and small venules. Pigment incontinence with melanin pigment laden macrophages seen just beneath the epidermis which is flattened with vacuolation of basal keratinocytes suggestive of vasculitis. Her Anti-Phospholipid antibody both IgG and IgM were positive. MRI done outside showed diffuse atrophy with slight increase in the mineralization of the basal ganglia. CT scan showed diffuse atrophy with old calcified granulomas []. CSF showed antimicrobial antibody positive which corroborates with the CT finding of calcified granulomas, with 1 cell/mm3, Chloride– 122 mEq/L, Glucose 35 mg/dl and Protein 155 mg/dl. Two months after diagnosis and initiation of treatment patient conceived again inspite of advice to the contrary. Therefore patient had to be maintained with steroids and Heparin throughout pregnancy. She had an IUD at 9 months of gestation. Following that her neurological status deteriorated with severe behavioral problems, recurrent seizures, Transient Ischemic attacks (TIAs) with skin changes over the face and limbs. The skin changes were in the form of strawberry tongue, cheilitis, hyper pigmented as well as erythematous rashes over the face and persistent fever with oral thrush, leucopoenia and anaemia. She was referred to multidisciplinary hospital for follow-up. |
A 64-year-old woman with psoriatic arthritis was found to have a raised ALT on routine DMARD monitoring blood tests. Her psoriatic arthritis was in remission on methotrexate 15 mg once weekly, folic acid 15 mg once weekly and paracetamol as required. She had recently returned from a holiday in Spain and was treated for a chest infection with a course of amoxicillin. Clinically she was icteric but examination was otherwise normal. Blood tests revealed a bilirubin of 65 μmol/L, ALP 501 IU/L and ALT 628 IU/L. These had been normal one month previously.\nThe differential diagnosis for this patient’s presentation was considered to be either drug related (secondary to antibiotics or methotrexate) or a viral infection. The methotrexate was withheld and she underwent a full liver screen. An abdominal ultrasound showed fatty liver infiltrates but no other significant pathology. She was found to be positive for hepatitis E virus (HEV) IgG and IgM antibodies. The LFTs normalized within 4 weeks after which she was recommenced on methotrexate and have subsequently remained normal.\nHEV is a ubiquitous single stranded RNA non-enveloped virus with four main genotypes. HEV1 and 2 are usually transmitted via contaminated water in developing countries whilst HEV3 and 4 are zoonotic, commonly infecting wild and domesticated pigs amongst other mammals []. The virus is well known for its severity in pregnant women, where mortality rates can approach 15–20% in contrast to healthy individuals where the infection is usually asymptomatic [, ].\nIn patients with compromised immune systems, such as patients on chemotherapy or transplant maintenance medication, chronic HEV infection, where HEV RNA persists for >3 months, is well documented []. Although frequently self-limiting these infections can rarely lead to liver cirrhosis [].\nPatients with inflammatory arthritis receive similar immunosuppression from DMARD therapy and therefore are at increased risk of chronic HEV infection []. Similar to healthy individuals HEV infected RA patients are frequently asymptomatic however common complaints include jaundice, right upper quadrant abdominal pain and fever [].\nA retrospective study, although low powered, showed good rates of clearance of HEV in these patients within three months. This was achieved either spontaneously or through DMARD withdrawal/dose reduction. The antiviral agent, ribavirin, is indicated when LFTs remained persistently raised [].\nFollow up virology testing, monitoring viral load and HEV IgM levels (positive in this patient), is important in these patients to confirm HEV clearance especially when LFTs remain abnormal [].\nHEV serology should be performed in patients on with DMARDS who develop abnormal liver function tests. |
A medically free 14-year-old male who was obese sustained an injury to his left knee after jumping from 3 stairs. The patient mentioned that he predominantly landed on his left lower limb with his left knee in full extension and in external rotation. The patient started complaining of left knee pain limiting his range of motion and ability to bear weight immediately after the fall. He was brought to the Emergency Department (ER) of King Saud Medical City (KSMC) by his parents immediately after the injury. On physical examination, the left knee was profoundly swollen and bruised. There was tenderness over the tibial tuberosity and lateral joint line. He was unable to actively move the knee joint. The passive range of motion was painful. There were no signs indicating compartment syndrome or neurological or vascular injury. X-ray radiographs revealed a Watson-Jones type IIIB avulsion fracture of the tibial tuberosity apophysis (). A CT scan showed a step of the articular surface more than 2 mm extending to the posterior-lateral epiphyseal part of the proximal tibia (lateral tibial plateau) ().\nThe patient was admitted and was prepared for operative management. A procedure was planned and done on a radiolucent table under general anesthesia. A tourniquet was used to avoid excessive bleeding during the procedure. The tourniquet was inflated after pulling down the quadriceps to avoid blocking the reduction due to the extensor mechanism. The leg is prepped and draped according to the standard orthopedic protocol. Anterolateral approach of the knee was used with an incision starting from the lateral upper border of the patella to 10 cm down. Deep fascia was opened anterior to the iliotibial tract. The fracture line was identified; the reduction of the articular step was done using a reduction clamp and assured using a portable image intensifier on flexion and extension of the knee without arthrotomy. We avoided arthrotomy of the joint to not make it vulnerable to infection and possible scarring. Stabilization of the reduction was maintained using a k-wire. Definitive fixation was achieved with three 3.5 mm partially threaded cancellous screws placed under fluoroscopic guidance for the tibial tuberosity fracture. A proximal tibial plate was slid laterally and was used to buttress the lateral tibial column. Careful placement of the screws was done to not cross the physis with the help of a C-arm (). After fixation, good hemostasis was achieved, drain was placed, and the range of motion was assessed which was full. Closure was done layer by layer, then dressing after. The postoperative plan was to immobilize the knee in a cylindrical cast for 3 weeks with no weight bearing on the left lower limb with the use of crutches for ambulation.\nPostoperative knee CT scan is requested to ensure that the fracture is anatomically reduced. The patient received analgesia and antibiotics, and drain was removed 24 hours post-op. The patient was seen in an orthopedic clinic after 3 weeks, there were no signs of surgical site infection, and the clips were removed. The controlled range of motion was advised using a hinged knee brace throughout the day for 4 weeks. A follow-up X-ray () shows that the fracture is aligned with no loss of reduction or displacement. Physiotherapy is advised 7 weeks postoperative management focusing on the range of motion and strengthening. The patient was seen 6 weeks later, he had full range of motion with no deformity, and there were no complaints reported by the patient like locking or pain. |
A 58-year-old lady, nurse by profession, residing in the United States, presented to us in the second half of 2019. Her history was remarkable for systemic hypertension, dyslipidemia, and chronic persistent asthma. She denied any history of smoking or tobacco product use. She did not have any environmental or professional exposure to agents triggering her asthma. Her asthmatic symptoms dated back to late adolescence and had progressed in severity since then, with significant worsening over the last 3 years. She was steroid dependent for the last 15 months. Despite being on a maintenance dose of 10 mg of prednisolone per day, she used to experience nocturnal symptoms and needed salbutamol inhalations on an average 3-4 times a week. She used to get exacerbations once a month needing hiking up of prednisolone dose to 40 mg per day for 5-7 days. Her detailed evaluation was done in the United States. Her spirometry showed persistent obstructive ventilatory defect. Her compliance to medications was ascertained, inhaler technique was supervised, and medications were optimised. Thyroid abnormalities, gastroesophageal reflux, allergic rhinitis, and vocal cord dysfunction were ruled out by appropriate evaluations. She was obese (BMI 33) with no snoring or excessive daytime sleepiness measured by the Epworth scale. Cardiac function as measured by ECG and 2D ECHO were normal. CT chest showed no parenchymal shadows, bronchiectasis, or mucoid impaction. She had no features of anxiety or depression ascertained during a formal psychology evaluation. Her IgE levels were <10 mg/mL on multiple occasions in the United States as well as during her current evaluation. She was considered for therapy with biological agents (omalizumab) but therapy with this agent was not instituted as her IgE levels were below the range approved for therapy. She was having significant steroid adverse effects including truncal obesity and osteopenia.\nWith this background, she presented to our outpatient department. Her blood counts revealed a normal total leucocyte count (7700 per cubic mm []) with 6 percent eosinophils. Renal functions, hepatic functions, serum electrolytes, fasting lipid profile, and thyroid functions were normal. She was evaluated at the difficult asthma clinic where her compliance to medications was ascertained, inhaler technique was supervised, and medications were optimised (budesonide 1600 mcg per day, tiotropium 18 mcg per day, addition of montelukast-levocetirizine combination, and sustained release theophylline). The reports of evaluations done in the United States were crosschecked. Her spirometry () showed persistent obstructive ventilatory defect (postbronchodilator FEV1 74% predicted). Serum IgE levels repeated at our institution revealed a value of 7.5 IU/mL. She had inadequate response at one month of our therapy with exacerbations needing escalation of systemic steroids. Considering the burden of her disease, adverse drug effects, and its impact on her life, she was given the option of off label omalizumab therapy which she readily accepted after detailed briefing regarding the uncertainty in efficacy. She was initiated on omalizumab 150 mg subcutaneously once monthly, while continuing her baseline drugs. She began to get subjective improvement in her nocturnal symptoms after the second dose and tapering of prednisolone was attempted after the second dose. She was totally off systemic steroids by the sixth month of therapy and her rescue use of beta 2 agonists reduced to one per week on an average. A total of 9 doses of omalizumab were given. The improvement in steroid dose, asthma symptoms, and early morning PEFR are shown in . She had only 2 exacerbations needing hiking up of steroids during these nine months of therapy as opposed to 7 in the 6 months prior to omalizumab initiation (). Her asthma control improved significantly (baseline ACT score of 13 improving to 22 at the end of 9 months). Her postbronchodilator FEV1 improved by 3% over the 9 months of therapy. She remains under reasonable control with 800 mcg per day of budesonide, tiotropium, sustained release theophylline, and montelukast. She plans to return to the United States in the next 2 months. |
A 68-year-old female underwent a partial left-sided chest wall resection, with partial removal of the 6th and 7th ribs and of the scapula angle for elastofibroma (). The chest wall defect was reconstructed by using a Mersilene mesh, secured by interrupted pericostal stitches, and covered by a sufficient volume of viable muscles. The postoperative course was uneventful; the radiographic aspect at discharge was normal (). The first symptoms in the form of pains in the region of the incision appeared five months after the operation, and computer tomography (CT) of the thorax showed a lung hernia in the region of the mesh covering the chest wall defect (Figures and ). The patient refused the proposed surgical correction, being only slightly limited in usual daily activities. During the next several months, the symptoms persisted with variable intensity under analgesic therapy, till the moment when pains significantly limited patient's daily activities, 22 months after the operation. The repeated chest CT showed a slight increase in hernia size, with no signs of tumour recurrence (), so that reoperation was planned.\nAfter the excision of the previous skin scar and the incision of the muscular layer, the mesh region was exposed, showing a lung protrusion (4 × 3 cm) along the anterolateral edge of the mesh (). The local situation is schematically presented on . The mesh suture line in the hernia region was completely disrupted, with a small piece of the herniated lung being completely detached from the mesh, the remaining lung surface under the mesh area being fully adherent to the mesh. By careful dissection, the mesh was separated from a firmly adherent lung and removed (). After adhaesiolysis and complete lung liberation, a wedge resection of the afunctional lung tissue of the superior segment of the lingula was done, just in the region of contact with the mesh. After the chest tube insertion, the chest wall defect was reconstructed by suturing a Mersilene mesh in two layers—single pericostal sutures for initial fixation and running suture for additional reinforcement (). A final chest wall stabilization was done by the fixation of two Synthes plates (DePuy Synthes J&J) over the 5th and 6th ribs (). The postoperative course was uneventful. The chest X-ray on discharge, on postoperative day 5, is presented in .\nAt the last contact with the patient, one year after the operation, the general condition was good, without the need for analgesics. |
A 26-year-old young female presented with a history of partial seizures with secondary generalization since childhood. She was born to a non-consanguineous Saudi couple. The patient's seizures started at age of one month associated with decreased serum calcium levels. All history and lab were negative for other causes of seizures. She was diagnosed with partial seizure with secondary generalization and started phenobarbital at the age of one year, then tapered and discontinued at age of four years. During her childhood, she only had mild learning difficulties started at intermediate school, her school performance was challenging compared to her colleagues; however, she was able to finish high school and did not pursue higher education. Brain CT was normal initially. At the age of 21, she was diagnosed as having major depressive disorder with psychotic features and controlled on citalopram and quetiapine. At the age of 22, the patient presented to the emergency department (ER) complaining of dizziness after suddenly losing consciousness lasting about 30 seconds noticed by her mother; she had up rolling eyes with muscular rigidity. There was no urine incontinence, memory loss, or salivation, and she was seizure-free for the past three years until one month before this visit as when she again experienced one episode of seizure. Furthermore, the patient gave a history of multiple teeth loss throughout her life, and this description was suggestive of the manifestation of chronic hypocalcemia and dental enamel hypoplasia. Family history was negative for epilepsy, endocrine disorders, intellectual disability, and genetic diseases, she was the only case in her family. Also, the mother was not known to have any chronic medical illness, the pregnancy and antenatal history were unremarkable, and no genetic studies were done.\nIn ER, the patient was conscious, alert, oriented and communicating, stable vital signs, she had tiptoe walking during gait inspection, positive Chvostek's sign and teeth abnormalities. The patient was admitted to the medical ward for managing hypocalcemia. During the admission, the patient had total serum calcium level 5.1 mg/dL (normal range 8.5-10.1 mg/dL), serum phosphate: 6.2 mg/dL (normal range 2.6-4.7 mg/dL), magnesium level: 1.60 mg/dL (normal range 1.8-2.4 mg/dL), parathyroid hormone: 0.71 pmol/L (normal range 1.58-7.2 pmol/L), creatine phosphokinase: 777 U/L (normal range 26-308 U/L), thyroid-stimulating hormone: 1.127 µIU/mL (normal range 0.35-4.94 µIU/mL), total triiodothyronine level: 0.65 ng/mL (normal range 0.58-1.59 ng/mL), and total thyroxine level:4.24 mcg/dL (normal range 4.87-11.72 mcg/dL). Her ECG revealed prolonged QT interval, no echocardiography was done. A 21 channels EEG revealed an abnormal study showing a mild diffuse slowing of background rhythm and active focal epileptic discharges. These findings are suggestive of focal onset seizure with or without secondary generalization and right temporal structural pathology. Mild global cerebral dysfunction could be the result of underlying untreated epilepsy. A single brain CT without contrast was done has revealed: extensive coarse calcifications involving the infra and supratentorial white matter, predominantly within the basal ganglia, thalami, and dentate nuclei of cerebellar hemispheres (Figures -). After receiving intravenous calcium gluconate and restoring the normal calcium level over 24 hours, she was discharged on calcium carbonate and alfacalcidol tablets.\nFahr's syndrome with primary brain calcification or secondary to treatment of hypoparathyroidism induced hypocalcemia was the provisional diagnosis; Based on her history, CT findings, and neuropsychiatric manifestation. Secondary causes of hypoparathyroidism were excluded, such as a history of thyroid surgery, congenital anomalies, history of neck radiation, Magnesium level was low during this visit, history of autoimmune disease, or history of malabsorption. |
Our case was a 14-year-old right-handed boy who complained of pain in his right middle finger. The finger was injured by a baseball impact, and the treating hospital performed splint fixation after diagnosing a fracture of the right middle finger. The patient failed to follow up his treatment on his own volition. However, pain in the right middle finger continued and the range of motion became restricted. He consulted our institution 5 months after injury.\nAt first consultation, there was swelling in the PIP joint of the right middle finger and displacement of that finger to the ulnar side at extension position (Fig. ). The range of motion of the PIP joint was limited between extension 0° and flexion 60°.\nX-ray images of posterior and anterior views showed bony defect in the articular surface of the PIP joint in the middle phalanx and displacement of the finger to the ulnar side. X-ray image of the lateral view showed depressed articular surface of the PIP joint (Fig. ). CT images showed a bony defect sized 5 × 6.5 × 2 mm in the articular surface of the PIP joint in the middle phalanx (Fig. ). From these imaging findings, we diagnosed the case as malunited intra-articular fracture of the PIP joint and decided to conduct surgical treatment. First, an incision was made by palmar approach and the PIP joint was exposed. A cartilage defect approximately 5 mm in diameter was seen in the articular surface of the middle phalanx, and a cartilage defect of 1 × 2 mm in size was seen in the palmar side of the articular surface of the proximal phalanx (Fig. ). After creating the drilled recipient hole at the osteochondral lesion of the middle phalanx, a cylindrical osteochondral plug of 4.5 mm diameter harvested from the left knee was inserted and press-fitted to the hole. The osteochondral plug was harvested using the mosaicplasty autogenous osteochondral grafting system (Acufex, Smith and Nephew, Andover, MA, USA) from a non-weight-bearing site on the upper lateral femoral condyle. The osteochondral plug was obtained with an obliquely angled cartilage surface along the long axis to facilitate insertion in the recipient hole (Fig. c). The cartilage defect in the proximal phalanx was left untreated as the range of damage was minimal.\nPostoperative splint fixation was done only on the day of surgery, and mobilization exercise was started from the next day by changing the splint fixation to buddy taping. The buddy taping was continued up to 3 months after surgery. After removing the buddy taping, the patient gradually resumed sports activity.\nAs of 1 year after surgery, the patient has no pain, and the ROM of the PIP joint has improved showing extension and flexion to 0° and 90°, respectively. Although slight displacement to the ulnar side remains in the PIP joint, instability is not noted, (Fig. ). There are no adverse effects in the donor site of the left knee. The patient resumed his previous level of baseball activity. Final follow-up X-ray and CT images showed bone union with no dislocation of the implanted osteochondral plug. Although slight displacement of the finger to ulnar side remained, the ulnar displacement of the axis improved from preoperative 14° to postoperative 8°. Also, MR images showed a well-maintained joint space by the transplanted cartilage (Fig. ). |
A 41-year-old woman was hospitalized for a left thyroid mass, which had been found using type B ultrasonography. There was no enlargement of the lymph nodes in the neck on physical examination, and the color of the skin surface was normal. The mass was located in the left lobe of the thyroid, with an irregular shape, which indicated a malignant tumor. The maximum diameter of the mass was 2.4 cm, detected by ultrasonic examination. Scattered clusters of atypical cohesive epithelioid cells were found in the mass of the thyroid by fine-needle aspiration. The hematoxylin-eosin staining of the specimen, together with frozen section and extensive immunohistochemistry, confirmed a diagnosis of paraganglioma of the thyroid gland with melanocytic differential. The patient underwent a mass excision, and no lesion was found using computed tomography or magnetic resonance imaging. Four lymph nodes, which showed no metastatic carcinoma (0 of 4), were isolated from the left neck.\nThe mass specimen was measured to be 3.5 cm × 2.3 cm × 2.0 cm. On the cut surface, all the visible area of the tumor was solid without cyst changes, with grayish white or black color, and the margins of the tumor were slightly irregular. Two distinct components were found in the black area of the tumor through microscopic observation. The first component, the majority of the mass, was composed of solid sheets and clusters of cohesive epithelial cells, with no pigment observed in the cytoplasm (, ). The second component comprised cells covered by pigment, with the intercellular substance and structure unclear (, ). The tumor cells in the first component were arranged in a nest-like distribution without any gland cavity. The tumor consisted of monotonous sheets of cells with fluent and light-dyed cytoplasm, but without significant atypia. Tumor cells were round or polygonal, and the cytoplasm was basophilic. No mitotic figure was observed. There was no tumor thrombus observed in the vascular tissue after multiple dissections. Using immunohistochemistry, the tumor cells in the first component tested diffuse strong positive for synaptophysin (Syn) (), but negative for chromogranin A (CgA), pan-cytokeratin, calcitonin and thyroglobulin. Ki-67 was positive in approximately 1% of tumor cells. A large quantity of pigments were within the stroma (). These pigments were negative for periodic acid-Schiff (PAS) and Prussian blue, but positive for Fontana-Masson. Of the cells surrounding the tumor, few were positive for HMB-45 and Melan A after bleaching by oxalic acid (, ). The stromal cells were positive for S-100 (). The tumor cells were negative for calcitonin and thyroglobulin (, ). The remaining tissue was free of tumor infiltration or metastasis.\nElectron microscopy showed that the tumor was composed predominantly of polyhedral cells that had the features of paraganglion chief cells, although they exhibited greater nuclear pleomorphism than normal chief cells. Half of the chief cells had abundant cytoplasm with dispersed organelles and secretory granules (); the other half had less cytoplasm and more closely approximated organelles and granules. Nucleoli, which were inconspicuous using light microscopy, were not prominent. The cytoplasm contained numerous mitochondria and many dense granules of the neurosecretory of endocrine type (). These granules were of uniform electron density, membrane-limited, and 100 to 150 µm in diameter. Most had halos or clear spaces between the granules and the surrounding limiting membrane. Although the cytoplasmic margins of adjacent cells were difficult to evaluate, they appeared to interdigitate with one another. As is typical of paragangliomas, the tumor cells were closely related to the capillaries present within the stroma. The stroma itself appeared to be composed of fibrocytes and collagen with occasional histiocytes. Amyloid was not identified. Cells with ultrastructual features of sustentacular cells were extremely rare. A few had less cytoplasm and fewer organelles, and these also had many round melanin bodies with greater electron density granules of nonuniform size ().\nThe pathologic diagnosis was of primary pigmentary paraganglioma of the thyroid gland without focal necrosis and vessel tumor thrombus. No recurrence or distance metastasis was observed after 19 months of follow-up.\nParaganglioma usually occurs in the area where paraganglia are distributed, such as the posterior peritoneum and the head and neck region. Paraganglioma of the head and neck region mainly occurs in the carotid body, jugulotympanic, vagus nerve body, and auris media. However, paraganglioma is rarely found in the thyroid. Previous articles have reported that no paraganglioma occurred in the thyroid and the precise pathogenesis of thyroid paraganglioma is unknown. We postulate that ectopic carotid body or jugulotympanic paraganglia could be a plausible origin of the lesion. Another possibility is that paraganglioma may originate from entrapped neuroendocrine progenitor cells of the dispersed neuroendocrine system, resulting from aberrant migration of the neural crest during embryogenesis.\nPigmental paraganglioma is a special and rare type of paraganglioma. Less than 38 cases have been reported since Tavassoli first reported two pigmental paraganglioma of the uterus. It featured multiple pigment particles in the cytoplasm. Pigmented paraganglioma has been reported across a wide age range (17-57 years), and no gender differences were noted. It has arisen in the bladder, posterior peritoneum, mediastinum, spine, and other sites. There is no functional symptom apparent, such as hypertension.\nAn unusual feature of this case was the presence of significant amounts of pigment. Based on histochemical staining or electron microscopy, the pigment has been classified as neuromelanin, lipofuscin, or true melanin. Herein, we present a case of thyroid paraganglioma with extensive melanin melanosis in the stroma. Using electron microscopy, the melanin globules were found in the chief cells. It was an interesting and unique case of paraganglioma with melanocytic differentiation without evidence of melanoma of the skin. Remarkable melanosis deposition was observed not only macroscopically but also microscopically. These pigments are negative for PAS and Prussian blue, but positive for Fontana-Masson. Electron microscopy showed that the tumor cells had large, pleomorphic granules with varying electron density, size, and shape, which are identified as neuromelanin. To the best of our knowledge, this is the first such case reported. Although primary melanoma of the thyroid gland has not been previously reported, paraganglioma with melanocytic differentiation was confirmed by morphologic and immunohistochemical staining, and melanoma was definitely excluded.\nThe diagnosis of paraganglioma mainly depends on the combination of morphology and immunohistochemistry. However, in previous reports, it was made by morphology, special staining, and electron microscopy, which could not distinguish paraganglioma and medullary carcinoma. Because the head and neck paraganglioma is a nonfunctional tumor, there is no obvious clinical presentation other than a cervical lump. Thyroid paraganglioma, medullary thyroid carcinoma, and hyalinizing trabecular tumor (HTT) are difficult to distinguish only by histological morphology, so they are usually misdiagnosed before immunohistochemistry. As a result, all thyroid paragangliomas were identified as medullary thyroid carcinoma. Meanwhile, some paragangliomas are extremely similar to medullary carcinoma, the histological type of which is called “paraganglioma-like medullary carcinoma.” It features high levels of serum calcitonin and more or less amyloid in tumor stroma. Immunohistochemistry shows that apart from neuroendocrine markers such as neuron-specific enolase, CgA, and Syn, the tumor also expresses calcitonin, carcinoembryonic antigen, and cytokeratins. Even S-100-positive cells occurred in medullary carcinoma around the tumor nest, which has similar presentation with paraganglioma. However, it is difficult to confuse paraganglioma-like medullary carcinoma and paraganglioma. Comparatively speaking, HTT seldom occurs in the thyroid. The tumor cells are arranged as funicular or acini, resembling paraganglioma, and rare thyroid collagens are observed. Nevertheless, immunohistochemistry shows tumor cells are positive for thyroglobulin and thyroid transcription factor-1, and may express cytokeratins, but are negative for calcitonin. All these illustrate that the tumor cells originate from thyroid follicular epithelium. Genetic features suggest HTT is closely related to thyroid papillary carcinoma and may be the early period or histological type of papillary carcinoma, so immunohistochemistry can differentiate HTT from paraganglioma.\nOf greatest concern to clinical doctors is whether paraganglioma is malignant or not because it relates closely to resection range and continuous therapy. However, with neuroendocrine tumors, benign and malignant are hard to differentiate for the pathologist. The prognosis of paraganglioma needs to be comprehensively assessed by surgical approach (biopsy, local resection, or complete resection), tumor size, operative margin, cell atypia, count of mitotic figure, necrosis, lymph node metastasis, vascular invasion, etc. Paraganglioma in the thyroid has been depicted as a benign progression in previous reports, although one reported that thyroid paraganglioma was malignant because it invaded the thyroid cartilage and penetrated through trachea wall into the tracheal mucosa. Its infiltrative growth mode seemed malignant, but others suspected it originated from cervical soft tissue rather than the thyroid. |
A 17-year-old Japanese woman, who had decreased vision in her right eye and severe ocular pain and headaches, was referred for management of vitreous hemorrhage. On a day prior to her visit the woman's younger brother had been playing with an umbrella, which subsequently caused the handle to detach and the metal tip of the umbrella (figure ) to strike the woman in her upper right eyelid. She reported that she only felt being hit with something hard on her right upper eyelid and the left eye. Following the incident, the umbrella was found on the floor. She visited a local clinic, and the eyelid injury was sutured on the same day because it was considered to be a surface injury. On the next day she had severe ocular pain, headaches, and blurred vision in the right eye and she visited our clinic.\nAt presentation the lid wound in her right eye was sutured and the lids were severely swollen. Her vision had diminished to light perception only in her right eye and was 20/200 in the left eye, but she could barely open her right eye because of the severe ocular pain. The eyelids were gently separated and slit-lamp examination revealed an intact globe with moderate mydriasis. There were 2+ inflammatory cells and fibrin in the anterior chamber of the right eye and corneal erosion in the left eye. Ophthalmoscopy revealed commotio retinae, and intraretinal hemorrhage at the superior quadrant with mild vitreous hemorrhage in the right eye, but no sign of a rupture of the globe.\nAlthough her lid wound was small the woman had severe headache, and CT was performed on the orbit and brain to determine the extent of the injury. Unexpectedly, the images revealed irregular signals between the right orbit and the third ventricle. The signals were interpreted as brain edema, and the high-density area, which was isodense with bone and located anteriorly to the third ventricle, represented orbital bone fragments; no other foreign body was detected on cross-sectional CT scanning (Figure ). On three-dimensional CT scanning a window defect became apparent in the superior orbital wall (Figure ). MRI on the same day demonstrated edema of the head of the caudate nucleus and putamen, and the edema extended to the hypothalamus, where the bone fragments had penetrated into the brain cavity through the inferior part of the frontal lobe (Figures ). We assumed that her headaches were caused by the leakage of cerebrospinal fluid, and she was transferred to the Neurosurgery Department.\nThe patient was given intravenous hyperosmotic solutions to reduce the brain edema and antibiotics to prevent bacterial meningitis. Her systemic condition improved, and the ocular pain and headaches disappeared within a week. However, several neurological tests showed that her memory was altered, indicating minor brain damage.\nAfter the vitreous hemorrhage had cleared, a retinal break was found superiorly where the intraretinal hemorrhage had been located, and the break was treated by laser photocoagulation. The retina remained attached in the right eye three months later, with full ocular movement and a minor scar of width approximately 2 cm at the superior eyelid (Figure ). Her vision improved to 20/50 in her right eye and 20/20 in her left. Decreased vision in her right eye was caused by subretinal fibrosis due to the choroidal rupture and subretinal hemorrhage caused by the blunt trauma. |
A 55-year-old woman visited our hospital with complaints of palpitations and discomfort during swallowing. Her medical and familial histories were unremarkable. A frontal chest radiograph showed a smooth round mass, and a lateral radiograph showed a smooth mass slightly larger than 7 cm in diameter in the middle mediastinum between the trachea and the vertebral bodies. A chest computed tomography (CT) scan showed a lobulated tumor (75 × 57 × 80 mm) in the upper to middle mediastinum, with a homogenous inner component, compressing the esophagus (Figure ). Magnetic resonance imaging (MRI) of the chest revealed no invasion of surrounding organs. On imaging studies, a mesenchymal tumor such as gastrointestinal stromal tumor was suspected. Upper gastrointestinal endoscopy showed a smooth elevated lesion, 22 cm from the incisor teeth. A mucous membrane was accompanied by the venous dilation (Figure ). A schwannoma was diagnosed based on the biopsy result for the lesion. Although there was no evidence of malignancy, the patient underwent surgery because of the large size of the tumor, dysphagia, palpitations caused by the tumor compressing the heart, and a suspicion of malignant potential. The patient was placed in the left lateral position and underwent a mini thoracotomy via the fifth right intercostal space with thoracoscopic assistance. A mass slightly larger than 8 cm in diameter was found adjacent to the mid-thoracic esophagus. From above, the tissue surrounding the esophagus was detached and the tumor was excised. The resulting defect in the adventitia and muscular layer was extensive. A direct anastomosis was considered to be difficult, such that subtotal esophagectomy and esophagogastrostomy in the right thorax were performed. The resected specimen showed normal esophageal mucosa. The tumor was well demarcated and elastic hard, had a globular appearance, and measured 75 × 57 × 80 mm. The cut surface was almost uniformly milky white. Histopathological examination revealed spindle-shaped cells in a fasciculated and disarrayed architecture and nuclei in a palisading pattern (Figure ). Immunohistochemical studies revealed S100 protein positivity (Figure ) and the absence of staining for α smooth muscle actin (αSMA), CD34 and CD117, establishing the diagnosis of benign schwannoma. Her postoperative course was uneventful and there has been no evidence of recurrence to date. |
A 74-year-old woman presented with right upper abdominal pain and poor dietary intake. She had no notable medical history or history of abdominal trauma. Abdominal computed tomography (CT) showed multiple liver cysts, with the size of the largest cyst in the right lobe measuring 155 × 113 × 106 mm (). In addition, a large cyst measuring 135 mm in the left lateral lobe strongly compressed the stomach (). T1-weighted magnetic resonance imaging revealed different findings in the right and left lobe cysts, and there were no enhanced structures in the liver cysts (). Positron emission tomography-CT did not show increased 18F-fluorodeoxyglucose metabolism in any of the liver cysts (). Moreover, hepatobiliary scintigraphy with CT showed no biliary communication with the liver cysts (). The patient was diagnosed with symptomatic and multiple liver cysts with no biliary communication or malignancy. Therefore, LF was performed.\nOne hour before surgery, the patient (body weight: 45.9 kg) was intravenously administered ICG (2.5 mg). A 12-mm trocar was placed at the umbilicus for scope entry, and two 5-mm ports were placed at the epigastric and right upper areas to manipulate the instruments (). A laparoscopic imaging system (Visera Elite II; Olympus, Tokyo, Japan) was used to detect ICG fluorescence. Large cysts were observed in the bilateral lobe, and large cysts in the left lateral lobe compressed the stomach (). ICG fluorescence was detected in the liver parenchyma and the biliary tract, but not in the cyst wall (). The left and right lobe cysts were punctured to drain fluid. Biochemical examination showed no elevation of bilirubin levels in the cystic fluid from either the left or right lobe cysts. ICG fluorescence imaging clearly distinguished the cysts from the liver parenchyma, and we could resect only the cyst wall as wide as possible under the guidance of white light and fluorescence imaging (). Intracystic bile ducts and bile leaks in the cysts were not detected during surgery. The large cysts were completely fenestrated, and the small cysts were conserved. The total surgical time was 73 min. The total cystic fluid volume was 1900 ml, and there was no blood loss during surgery. All cysts were histopathologically diagnosed as benign liver cysts lined with cuboidal epithelium.\nThe patient had no complications and recovered quickly; she was discharged on postoperative Day 6. The patient’s condition was good, and there was no recurrence of the preoperative symptoms after discharge. |
A 29-year-old female patient who was 29 weeks pregnant presented to another hospital complaining of shortness of breath, fever and epigastric pain. Her past history was remarkable for a caustic esophageal injury that was treated by esophago-gastrectomy and colonic interposition 8 years ago. Soon after her admission she went into a state of severe respiratory distress, was intubated and mechanically ventilated. A CT scan of the abdomen showed a dilated large bowel that was presumed to be related to a left-lower-lobe pneumonia. She was transferred to our hospital for further treatment. On admission the patient was sedated, mechanically ventilated, oliguric, tachycardic to 160 beats/min, hypotensive with a systolic pressure of 70 mmHg and had profound lactic acidosis. Due to severe fetal distress she was transferred to the operating room for emergency cesarean section. A 1,100 gram male fetus was delivered, intubated, ventilated and after stabilization was transferred to the neonatal intensive care unit (NICU). On exploration of the abdominal cavity, the patient's almost entire remaining colon and 130 cm of distal small bowel were necrotic as a result of an adhesion from the previous surgery that caused complete bowel obstruction. The necrotic bowel was resected and the ends stapled off without an anastomosis or a stoma. This was elected due to hemodynamic instability. The abdomen was temporarily closed and a planed second-look laparotomy to determine the fate of the remaining bowel was scheduled. The patient was transferred to the ICU for further stabilization. On the next day, 30 hours after the first operation, the patient underwent a second-look laparotomy. Surprisingly, an additional segment of 150 cm of distal small bowel was necrotic and was therefore resected. The patient remained with approximately 120 cm of jejunum, and even this segment looked somewhat pale and non-viable. Again, the abdomen was temporarily closed for a planned third laparotomy. At that point the patient was diagnosed as being positive for H1N1 influenza by real time PCR test, and began receiving appropriate treatment. On the next day she underwent another laparotomy during which and additional segment of 40 cm of distal jejunum was resected, and an end-stoma was fashioned. Gradually she recovered in the ICU, and was transferred to a general surgical ward one week after admission to the hospital. She now has approximately 80 cm of normal small bowel ending in a stoma, and is getting her nutritional support by total parenteral nutrition (TPN). Repeat testing for H1N1 was negative one week after the first positive result. |
A 5-year-old boy with a history of uncontrolled, moderate-persistent asthma presented to the pulmonology clinic for asthma management. The patient had a previously normal developmental history, up-to-date immunization record including Streptococcus pneumoniae and Haemophilus influenzae, and notable past medical history significant for recurrent otitis media due to poor compliance to oral antibiotics, which were subsequently successfully treated with parenteral antibiotics. Upon presentation, the patient recently had a left lower lobe community-acquired pneumonia that was treated with one dose of intramuscular ceftriaxone in the emergency department followed by a standard 10-day course of amoxicillin. The patient's family was instructed to open the capsule and mix the powder with food to improve compliance due to known history of poor palatability with previously prescribed antibiotics. The patient returned to the emergency department two weeks later with worsening of symptoms and was treated with oral cefdinir for unresolved pneumonia. He was subsequently seen in the pulmonology clinic four days later to establish care without acute complaint. He was started on fluticasone/salmeterol and a five-day course of oral prednisolone for management of an acute asthma exacerbation.\nOne month later, the patient presented to the emergency department with fever and increased cough and was diagnosed with right middle lobe pneumonia. He was treated with intramuscular ceftriaxone to be followed with a home course of oral antibiotics, which was reportedly completed. Two weeks later, the patient again presented to the emergency department with recurrent symptoms and diagnosed with right lower lobe pneumonia that failed outpatient treatment. The patient was subsequently admitted to the medical floor and managed with a single dose of intravenous ceftriaxone. He was discharged the following day in stable condition on cefdinir and azithromycin. The patient was to follow-up as an outpatient with his pulmonologist and primary care physician. Hospital laboratory work including CBC with differential, peripheral blood cultures, sweat chloride test, immunoglobulin assay, T cell count, and Aspergillus antibody was unremarkable. At the two-week follow-up, he had persistent fever, wheezing, daytime and nighttime cough, shortness of breath, exercise intolerance, and difficulty breathing. The patient was scheduled for further evaluation with high-resolution chest CT without contrast and flexible bronchoscopy with bronchoalveolar lavage and nasal cilia biopsy to evaluate for Primary Ciliary Dyskinesia (PCD). Repeat chest X-ray at that time revealed an improved right lower lobe consolidation without complete resolution, as expected. CT scan revealed a mild degree of bronchiectasis in the lower lobes, bilaterally, associated with consolidation on the right and linear atelectasis and scarring on the left with other nonspecific evidence of chronic inflammatory versus infectious process. Bronchoscopy identified multiple mucus plugs in the right bronchus intermedius (Figures –), right middle lobe (Figures and ), and right lower lobe (). Gross inspection of the bronchoscopy sample revealed a solid, rock-like mucus plug (). Airway clearance via a high-frequency chest wall oscillation vest along with nebulized therapies was initiated. The patient did not tolerate postoperative oral azithromycin and required mixing with grape juice.\nOne week following the procedure, he was readmitted for recurrent fever and a new rounded lucency on chest X-ray in the right lung base, suspicious for cavitation or pneumatocele. Chest CT demonstrated a 2.5 × 3.2 × 3.7 cm area of cavitation within a region of consolidation or bronchiectasis in the posterior aspect of the right lower lobe with mediastinal and right hilar adenopathy, suspicious for cavitary bacterial pneumonia or fungal disease (). Home treatment continued with the addition of intravenous ceftriaxone. Bronchoalveolar lavage culture from the previous week grew beta lactamase negative Haemophilus influenza and Moraxella catarrhalis. Fungal culture, mycobacterial culture, bronchial aspirate cell count, cytology, gastrin pepsin assay, and pathology report were all unremarkable. A PICC line was placed for 3 weeks of home antibiotics as a management for complicated cavitary pneumonia. Further workup included QuantiFERON-TB Gold, HIV assay, complement assay, tetanus antibody, diphtheria antibody, and pneumococcal antibody which were all unremarkable.\nAt 3-week follow-up post hospitalization, the patient was symptomatically improved without coughing or fever and he was reported to be back to his baseline. The mother reported adherence to antibiotic regimen through the PICC line. He continues maintenance therapy with fluticasone/salmeterol, montelukast, and vest therapy. He was started on oral azithromycin three times weekly. At the two-month follow-up, the patient remained asymptomatic, and repeat imaging at the nine-month follow-up revealed complete resolution of cavitating consolidation with residual bilateral mild bronchiectasis (). |
A 38-year-old unconscious female with left hemiplegia was admitted to out hospital. The neurologic evaluation on admission revealed that her consciousness level was drowsy (Glasgow coma scale 14, eye opening 3, verbal response 5, motor response 6), and motor grade was I on the left side. The patient had a history of TBM at the age of one, and she suffered from mental retardation as a consequence of the TBM. Except for mental retardation, no neurologic deficits were noted prior to the hemorrhage, and cerebral angiography had never been performed. Brain computed tomography (CT) scan disclosed a 35 mL hematoma in the right temporo-parietal lobe and an intraventricular hemorrhage (IVH) from the right lateral ventricle to the 4th ventricle (). CT angiography revealed a saccular aneurysm, presumed to be the origin of the hemorrhage, at the right temporal area and hypotrophy of the branches of the right middle cerebral artery with M1 occlusion. For further evaluation, digital subtraction angiography was performed. The right distal ICA was occluded proximal to the ophthalmic artery with basal collaterals on the right common carotid artery (). A small aneurysm was seen at the petrous branch of the right MMA. Angiography of the left common carotid artery revealed the left distal ICA and middle cerebral artery vessels to be patent, and the right middle cerebral artery and the anterior cerebral artery received blood supply through the anterior communicating artery channel ().\nUnder general anesthesia, the patient underwent an emergency operation to evacuate the massive hematoma and to control the acute hydrocephalus with IVH. As the dura was incised, an aneurysm-shaped vessel was identified penetrating into the dura from the MMA (). After coagulating the vessels of the dura and removing the hematoma, the temporal horn of the lateral ventricle was exposed. The wall was reconstructed with Tachosil (Takeda Inc, Osaka, Japan), and cranioplasty was performed with a mesh plate to facilitate ingrowth of the branches of the arteries. An external ventricular drainage tube was inserted, and the operation was concluded.\nA 6 × 6 × 4-mm-sized aneurysm was biopsied. Hematoxylin and eosin staining revealed fibromyxoid degeneration of the vascular wall with partial rupture, and pseudoaneurysmal rupture was confirmed. The vessel wall did not stain on elastic stain, a typical finding of pseudoaneurysm ().\nPostoperatively, the patient's consciousness was drowsy, although conversation was possible despite grade II weakness on her left side. Delayed communicating hydrocephalus developed, a ventricular peritoneal shunt was inserted, and the patient was transferred to the department of rehabilitation medicine. |
A 16-year-old female patient presented with a swelling over left lower orbital area which was present for last 3 years. On examination, the swelling was pinkish in color with a deep “T” shaped scar over it due to previous intervention []. On palpation, the swelling was soft and nontender, seemed to be fixed with the deeper tissue.\nTwo years ago, she was treated as a case of dacryocystitis with osteomyelitis of lower orbital rim (left maxillary bone) for which incision and drainage was done by an ENT surgeon in her local area; it took around one month for the wound to heal. Though there was an initial reduction of the size of the swelling for few months, the swelling started to grow again for last 8 months, for which she came to us.\nWe then referred the patient to the department of ophthalmology for detailed cheek up of the eye and lacrimal system. On examination, her visual acuity was found to be normal; conjunctiva was normal; syringing through both puncta of her left upper and lower eyelid proved the patency of lacrimal system.\nContrast-enhanced computed tomography scan of paranasal sinuses (PNS) and orbit showed an ill-defined, enhancing, soft tissue density mass in the left lacrimal sac region extending up to superior aspect of right maxillary antrum; disruption of medial wall of left orbit was noted; adjacent fat planes were blurred []. The impression of the radiologist was an inflammatory lesion of the lacrimal sac with pyocele. Her routine blood investigation along with erythrocyte sedimentation rate was normal. The patient was tested negative for HIV, and she did not have any other immunodeficiency. Her X-ray chest was done for routine preanesthesia check-up, which was found to be normal.\nHence, with a preoperative diagnosis of orbital swelling due to lacrimal sac pyocele, we decided to explore the area surgically in presence of an ophthalmologist. The operation was done under general anesthesia. An incision was placed over the old scar extending medially up to the medial canthal ligament. On exploration, we found an ill-defined soft tissue mass adherent with the surrounding soft tissues; meticulous dissection was done and the swelling was found to be extending deep into the orbit. As the lacrimal sac was intimately adherent with it, it was removed. There were also some erosion of medial part the bony orbit []. Extra-ocular muscles were not involved and intraoperative tests were done to check their functions. Electro-cauterization of the wall of the cavity was done and gelfoam was packed into the cavity to achieve hemostasis; the wound was then closed.\nHistopathological examination of the specimen was done. Sections from the orbital cyst and lacrimal sac showed tissue lined partially by stratified squamous epithelium and partially by columnar epithelium. Sub-epithelium showed sporangia with endospores. Surrounding tissues showed chronic inflammatory cell infiltrate []. Thus, a diagnosis of orbital rhinosporidiosis was made.\nThe patient made a good recovery and was discharged on the 5th postoperative day on oral antimicrobial therapy (dapsone 100 mg/day, for 6 days a week, for 6 months). Before discharge, a through ENT cheek-up was done with nasal endoscope and fiberoptic laryngoscope to look for any such lesion in the upper respiratory passage, but no such lesion indicating infestation by Rhinosporidium in that area was found. There was no sign of recurrence in last 6 months follow-up []. |
A 4-year-old female child came to the emergency of a medical college with the history of accidental impaction of the head of a toothbrush while brushing her teeth. The handle of the toothbrush was broken, and the head along with the adjacent part of the handle remained in her throat. She was unable to expel it out from her mouth. Instead, she tried to swallow it repeatedly and eventually got it stuck in her throat.\nHer parents took her to the emergency of a nearby health center but, unfortunately, no dentist was posted there. The patient would have been relieved immediately if the service of a dental surgeon was available. The general physician took no risk and after initial supportive treatment with parenteral analgesics and antibiotics, he referred the patient to a higher level medical institution, where proper medical facilities are available.\nOn the next day, she was brought to the emergency of a medical college with odynophagia, swelling on the left side of the neck and fever. As it was not a dental college, the patient was admitted under the Department of Otolaryngology. Joint effort of a dental surgeon and an otolaryngologist would have been the best, but due to infrastructural constraints, the otolaryngologist had no other option.\nShe was complaining of pain in the throat and was unable to open her mouth completely or take food and drinks. She was restless and was having mild respiratory distress. Her temperature was 102°F, and she was mildly dehydrated.\nOn examination, there was edema of the uvula and the left lateral wall of the oropharynx including both pillars of the left tonsil. Bloodstained saliva was found in the oropharynx, but there was no mucosal injury. There was a swelling on the left side of neck posteroinferior to the angle of the mandible which was extremely tender. As the patient was a child and was in distress, the indirect laryngoscopic examination was not possible. The patient was unable to cooperate for flexible pharyngolaryngoscopy.\nPlain radiograph of the neck and chest revealed no significant abnormality. Computed tomography (CT) scan of the neck [] revealed the head of the toothbrush with its bristles occupying the left parapharyngeal space.\nThe patient was administered intravenous fluid, broad spectrum antibiotics, corticosteroid, and others to improve her condition before the operation. Taking a decision about the approach for its removal was a little difficult. It was decided that the toothbrush should be tried to be removed by endoscopic approach first, and if it failed, the external approach would be adopted.\nThe patient was operated under general anesthesia. A Boyle Davis mouth gag was introduced. The oral cavity and the oropharynx were cleaned by suction. Initially, the toothbrush could not be located. There was a moderate degree of edema involving the left lateral wall of the oropharynx and hypopharynx extending toward the laryngeal inlet. After a thorough examination, the broken end of the handle, which was light yellow in color, was found to be protruding inside the left pyriform fossa from its lateral wall. However, the exposure of the foreign body was not satisfactory. The Boyle Davis mouth gag was removed, and a rigid hypopharyngoscope was introduced. The entry point of the toothbrush was located. The head along with the bristles was found to be embedded inside the left parapharyngeal space. The handle of the toothbrush was grasped with a foreign body removing forceps and pulled gently, but the head was found to be strongly impacted within the soft tissue of the neck due to the presence of the bristles in it. The entry point in the left lateral pharyngeal wall was carefully widened a little by using a blunt probe, and the head was gradually disimpacted. After that, the toothbrush was removed by gently pulling, as well as rotating it slightly in either direction to free it from the surrounding soft tissue. It was done slowly with utmost care to avoid injury to the adjacent important structures. On measurement, the toothbrush was found to be 5.4 cm long including the broken part of the handle and 1.8 cm broad at the head [].\nAfter removal of the toothbrush, the wound in the left lateral pharyngeal wall was carefully inspected. There was no significant injury to the other adjacent important structures. The wound was apposed by applying stitches with absorbable suture material.\nTo eliminate the risk of respiratory obstruction in the postoperative period, a tracheostomy was done as a preventive measure. A Ryle's tube was introduced for feeding in the postoperative period.\nThe patient was kept on intravenous fluid on the day of operation. Ryle's tube feeding was started from the next day. The patient recovered quickly. On the fifth postoperative day, the tracheostomy tube was removed, and the wound was closed. The Ryle's tube was also removed on the same day, and the patient was allowed oral feeding. The patient was discharged 7 days after the operation. |
A 29-year old male, formerly a competitive soccer player, presented with pain in the superior-lateral and posterior aspect of the left knee. He had a history significant for gout that was managed without medications. Physical examination revealed a moderate effusion and an extensor lag of 5 degrees. There was no laxity to the ACL or other ligaments. An initial MRI revealed soft tissue nodularity in the lateral and central aspects of the knee, most consistent with PVNS or gout (, ). A second MRI was performed to obtain sagittal gradient echo images on a 3 Tesla MRI unit to confirm the preliminary diagnosis of PVNS. This again revealed the soft-tissue nodularities with a few areas of blooming artifacts, but not to the extent expected for classic PVNS and therefore was thought to be an atypical presentation by our radiology colleagues (). Surgery was scheduled for an arthroscopic knee debridement and excisional biopsy of the lesion by the senior author (JA). During the arthroscopy, significant crystalline formation on the articular cartilage in all three compartments was noted, extending to the synovium (). A large mass was seen anteriorly in the notch surrounding the ACL and PCL (). The mass was resected and sent to pathology for formal analysis. Extrusion of calcified-appearing tissue and crystals from the mass were consistent with a gouty tophus. The mass was debrided in its entirety with care to avoid ACL or PCL injury. The majority of gouty deposits found along the cartilage was debrided carefully with the aid of a shaver to protect the cartilage from further mechanical injury. An extensive synovectomy was also carried out to aid in the reduction of the gout crystal load within the knee. Post-operative histological analysis revealed the excised mass was in fact a gouty tophus. Additionally, since gout was suspected, we performed post-operative uric acid levels which were revealed to be 717 μmol/L. The patient tolerated the procedure well and was ambulating under full weight immediately post-operative. Pain was managed with over-the-counter analgesics. Six- and twelve-month follow-up revealed good range of motion, no effusion and cessation of pain during sporting activities. At two-week follow-up the patient was referred to a rheumatologist for medical management of their gout. |
A 27-year-old male patient had an interpersonal fight and received a blow with a blunt object on the left side of the face. Patient presented to the Department of Oral and Maxillofacial Surgery with the chief complaints of inability to chew and pain in left retromandibular region. On presentation patient was fully conscious, oriented and vitals were stable. There was no history of loss of consciousness. Also there was history of bleeding after trauma form the left ear. On local examination of the head and neck region there was diffuse swelling with retromandibular fossa and lower third of the face in the mental region on the left side. There was extreme tenderness in the region between the left posterior border of the mandible and the mastoid process and condylar movements were indistinct. Intra-orally there was occlusal step present with the lower premolar region, which correlated well with the extra-oral tenderness with left inferior border of the mandible in the mental region.\nEven though it is uncommon to find ipsilateral condylar and parasymphysis fracture of the mandible. But the history of blunt injury with positive history of bleeding from the ear on the same side and indistinct condylar movements with preauricular swelling were suggestive of ipsilateral fracture of condyle and parasymphysis of the mandible. A clinical diagnosis of fracture of ipsilateral condyle and Parasymphysis of the mandible was made. But an orthopantamogram revealed intact condylar process on either side. It also showed moderately elongated styloid process (ESP) on both sides and a suspected fracture of left SP []. A computerized tomography scan was ordered which confirmed the presence of elongated SP on either side and an undisplaced fracture of left SP [Figures and ]. Pain and tenderness around the left ear was correlated with fracture of the SP on the same side.\nMandibular fracture was treated with open reduction and internal fixation. SP fracture was managed conservatively with neck collar and analgesics. Inter-maxillary fixation was maintained for 2 weeks post-operatively. Post-operative period was uneventful. Patient was asymptomatic and occlusion remained intact after 1½ year follow-up. |
A 73-year-old Caucasian woman was rushed to our Accident and Emergency department after discovering a large swelling in her mouth on awakening. She had intermittent bleeding from the site with mild pain. Her history consisted of a recently constructed set of complete acrylic dentures made one week previously by her general dental practitioner. It was reported these had been slightly uncomfortable ever since she had received them. Later questioning further revealed that our patient had worn her dentures continuously, even at night, but had not yet returned to her dentist for a follow-up. Her medical history was significant for rheumatic fever as a child, resulting in valvular heart disease for which our patient had mitral and aortic mechanical valve replacements in 1991 and 2002, respectively. Warfarin 5 mg had been initiated to obtain a target INR between 2.5 and 3.5. Our patient also had hypothyroidism, which was being treated with thyroxine replacement therapy. On arrival to the hospital, her full blood count was found to be within the normal range, however her INR was recorded as 5.5. A clinical examination revealed a large, soft, dark red swelling involving the anterior region of the floor of her mouth, indicative of sublingual hematoma. The Wharton's duct of the submandibular gland was distinctly visible on opening (Figure ). As a result of the hematoma, the tongue was displaced superiorly and there was mild limitation of tongue movement. Our patient was acyanotic at the time of admission and on general examination there was no signs of stridor or major airway restriction. Further flexible endoscopic examination did not reveal any edema or obstruction within the pharynx or larynx.\nOn palpitation there were firm, bilateral swellings noted in the submandibular and submental regions. Her mouth opening was approximately 7 mm on first presentation.\nSome hoarseness of her voice was appreciated, but our patient's vital signs were stable. Oxygen (5 L/minute) was delivered through a nasal cannula and our patient was advised to sit in an upright position at this time.\nOur patient was transferred to our Acute Assessment Unit where close monitoring of her airway was undertaken. Warfarin was stopped and 2000 IU of Beriplex® prothrombin complex concentrate supplemented with 2 mg intravenous vitamin K was administered to reverse the anticoagulation. In addition, 100 mg of intravenous hydrocortisone was provided to assist in decreasing any associated edema. At approximately two hours post-transfusion the INR was corrected to 1.0. Although the airway was patent on arrival, the hematoma was seen to be increasing in size during this time and our patient was at risk of airway occlusion.\nImmediate decompression of the sublingual space was performed under general anesthetic. A conscious intubation was carried out using a fiber-optic flexible nasal endoscope. Had this not have been successful a surgical cricothyroidotomy or tracheostomy would have been required to facilitate the surgery. After successful intubation, an initial horseshoe-shaped incision was made followed by blunt dissection above the mylohyoid muscle. The lingual nerve and Wharton's duct of the submandibular gland were identified and preserved. Bipolar diathermy was carried out, as well as packing of the area with absorbable oxidized cellulose agent (Surgicel®) to prevent any further bleeding. As the nasopharynx and hypopharynx were found to be clear with no obvious obstructions, a clinical decision was made to extubate our patient after surgery.\nOur patient was transferred to our Intensive Care Unit where she remained for four days. She was given 1 mg/kg (70 mg) of subcutaneous low-molecular-weight heparin (LMWH), which continued daily for 10 days. On day three, our patient's warfarin treatment was restarted at a dose of 2 mg initially in combination with LMWH. Daily checks of her INR were carried out and as a result, her warfarin dosage was gradually increased accordingly. During this time our patient experienced a few occasional bouts of mild bleeding from the surgical site that were easily stopped with 5% tranexamic acid mouthwash. Ecchymosis later appeared over the anterior surface of the neck.\nAdequate healing was soon noted in the area of the surgical site, with no evidence of further bleeding. Our patient was discharged on the 12th hospital day with a therapeutic range INR of 2.8, achieved with 4 mg of warfarin. She was advised to discontinue wearing her dentures until any necessary adjustments had been made by her general dental practitioner. At review two months later she is doing well with no signs of recurrence. |
A twenty-five-year-old female was injured after falling from the back bumper of a moving truck. Her past medical history was significant for unspecified anemia and her surgical history included four previous cesarean sections. The patient presented to the emergency department of our level 1 trauma center and was evaluated by the orthopaedic trauma service and the general surgery trauma service. She was found to have an isolated injury to the left knee. Plain radiographs and computer tomography (CT) scans of the knee showed a lateral split depression type fracture of the tibial plateau (Schatzker type 2), mostly in the anterior portion with a vertical split (Figures and ). Upon application of a knee brace in the emergency department, obvious posterolateral instability was noted. A detailed ligamentous exam in the emergency department was deferred due to the acute injury and the patient's significant discomfort. Based on the physical exam findings, Magnetic Resonance Imaging (MRI) of the knee was indicated. The results of the MRI showed no injury to the anterior cruciate ligament, posterior cruciate ligament, medial collateral ligament, or meniscal damage (Figures and ). However, the MRI demonstrated a concomitant injury to the posterolateral corner (Figures –).\nThe patient was taken to the operating room on the first day after her injury. Open reduction and internal fixation was performed through a standard lateral approach to the proximal tibia including a submeniscal arthrotomy. Surgical fixation was achieved using a precontoured lateral proximal tibia plate by the manufacturer Smith and Nephew. After the fracture fixation, a detailed ligamentous exam was performed. This physical examination showed joint laxity to varus stress, 1+ at zero degrees and 2+ at thirty degrees. In addition, she had a positive tibial dial test at thirty degrees. These findings confirmed the diagnosis of posterolateral knee instability and established the indication for a PLC repair. A formal lateral dissection was performed and the peroneal nerve was protected and identified. The LCL had completely avulsed off the fibular head but remained firmly attached to the femur. The biceps femoris was partially torn and had sheared from the fibular head as well. Two suture anchors (1.5 mm Biomet Juggerknots, Warsaw, IN) were placed on the fibular at the respective insertions of the LCL and biceps. Direct repair of each was performed passing the suture in a Krackow fashion, giving good apposition of the ligament and tendon back to the fibula. The popliteus tendon appeared to be slightly stretched but in continuity and surgical repair was not deemed necessary. Afterwards, the knee was stable to varus stressing at 0 and 30 degrees. The incision was closed and the patient was admitted. After an uncomplicated postoperative hospital course, the patient was discharged to home with a knee brace locked in extension and non-weight-bearing instructions. At the two-week follow-up visit, the knee brace was unlocked and range of motion exercises were initiated. The patient was kept non-weight-bearing to the injured lower extremity for a total of 12 weeks.\nThe patient was last seen in the orthopaedic trauma clinic sixteen months after the operation when she returned to clinic for a follow-up appointment. On physical exam, she had 5/5 strength in her leg, had full range of motion from 0 to 140 degrees, and had no signs of joint laxity with anterior, posterior, valgus, or varus stress. Plain radiographs of the knee were taken at her sixteen-month postoperative visit (Figures and ). |
A 30-year old married female patient from a semi-urban area presented with the complaints of pain in the lower part of face, behind the ears, back side of neck which was more on the left side. It had started insidiously without any precipitating factor about three years back. Initially the illness was progressive in nature for a year and then reached plateau phase. She reported clicking sound in her ears which was even audible to people at one meter distance when she opened her mouth. The complaints would increase with the increased rate of the clicking sound. The clicking sound was louder when she opened her mouth and was not under her control. The clicking sound was absent during sleep. The symptoms did not vary with stressors at home. She also complained of poor concentration and mild impairment in work because of pain symptoms. There was no difficulty in speech and to swallow. Sleep and appetite were normal. She did not report of pervasive depressive or anxiety symptoms but she was worried about the illness. The presence of co-morbid psychiatric disorders was ruled out using MINI scale (MINI international neuropsychiatric interview, English Version 6.0.0).\nGeneral physical examination (GPE) revealed symmetrical rhythmic flapping movements of the soft palate and the uvula. She did not have associated regional tremors. A detailed central nervous system (CNS) examination did not reveal any focal deficits. Magnetic resonance imaging (MRI) of the brain was normal and did not reveal any signal changes in olivary nucleus. She was diagnosed as having EPT. She was started on clonazepam 0.75 mg /day and gradually increased to 3 mg/day over the next two months. The improvement in her symptoms started within one week and by her third visit at the end of three months, she reported an improvement of 80% in her pain symptoms and the clicking sound. Her concentration improved and reached premorbid state in all her daily activities. She was followed up for 6 months during which she reported sustained improvement. Video of the palatal tremor was taken to compare the improvement before and after administration of the medication to assess improvement. |
We describe a case of a 29-year-old lady who was referred to our hospital at the 40th gestational week of her first spontaneous pregnancy for counseling regarding an elective cesarean section due to a pelvic mass presenting as a tumor previa.\nShe was aware of an abdominal ultrasound examination performed at her childhood in which bilateral pelvic kidneys were diagnosed. Her renal function tests were always within normal limits.\nOn the transvaginal ultrasound examination performed at the 12th gestational week an intrauterine singleton pregnancy with a fetus appropriate for gestational age was found. On this scan the maternal left kidney was demonstrated in the pelvis behind the uterus attached to the sacral wall (see ).\nLater in the pregnancy, the patient had two routine obstetrical sonographic scans at 15th and 24th gestational weeks. Both examinations were normal, concerning the fetal growth and anatomy. In both scans the bilateral pelvic kidneys were reported (see ). The kidneys had a normal sonographic appearance and showed no hydronephrosis. The patient was informed of the possibility that the ectopic kidneys may interfere with the vaginal birth and a near-term assessment was recommended. Lastly, she was examined at the 40th week gestation. The fetal heart monitoring and amniotic fluid index were normal and the estimated fetal weight was 3800 grams. On the digital vaginal examination the cervix was closed, 50% effaced, and posteriorly positioned. The fetal vertex was at the spina −5 station, floating outside the pelvic inlet. Below the fetal head, the left pelvic kidney was palpated as a large fixed mass bulging from the upper-posterior vaginal wall into the birth canal. A transvaginal sonogram showing the relation between the fetal vertex, pubis, pelvic walls, and left kidney is presented in . An anterior-posterior distance of 5.75 cm was measured between the most anterior margin of the kidney and the posterior aspect of the pubic symphysis, while the fetal biparietal diameter was 9.8 cm.\nBased on this information the decision was made to deliver the fetus by an elective cesarean section. At surgery a healthy male newborn weighing 3626 grams was delivered. The right maternal kidney was found in the lower paracolic gutter, and the left kidney was palpated as a retroperitoneal mass behind the lower segment of the gravis uterus. The surgery and postoperative period were uneventful. |
A 20-year-old female patient reported to the Department of Oral Medicine and Radiology with a chief complaint of inability to close her mouth since birth. Her mother stated that she was also unable to close both eyes since birth and there was difficulty in suckling. Her milestones were normal and there was no history of prenatal or postnatal trauma. Her medical history revealed that she had visited a medical hospital previously for augmentation of the upper lip at the age of 15 years and breast augmentation at the age of 18 years. Intelligence quotient (IQ) evaluation revealed normal IQ level of the patient. There was no history of consanguineous marriage between the parents.\nOn general examination, all the vital signs were within normal limits. No abnormality was detected in lower limbs whereas shortening of the index finger was noted in both the upper limbs (). Her sensory functions were normal. She was unable to close both eyes () and raise the eyebrows. There was absence of wrinkles on her forehead (). The patient was neither able to whistle or blow air between her cheeks nor was able to smile. She was not able to completely abduct her eyes and was unable to rotate her eyeballs upwards or downwards and could only partially move it in medial and lateral directions. Other ocular manifestations were the presence of ptosis of the right eye and strabismus. Other facial and intraoral features were wide nasal apertures, incompetent lips (), scar mark over the upper lip and a high-vault palate ().\nBased on history given by the patient and features shown on clinical examination, the case was diagnosed as Mobius syndrome. Routine hematological investigations were within normal limits. Panoramic view showed decays with respect to maxillary left second premolar, first molar and mandibular right and left first molar (). Wrist radiographs showed short middle phalanges in the index finger of both hands (Figure and ). A lateral cephalogram showed no abnormality (). Correction of incompetent lips was planned using a multidisciplinary approach. |
A 31-year-old married male, educated up to 12th standard from middle socioeconomic status and working as a technical assistant presented to the outpatient department (OPD) of a multi-specialty hospital with complaints of frequent abdominal pain and disturbed bowel habits for the last 6 years. These pains would get exacerbated soon after eating and/or taking tea and reduce only after defecation. Moreover, these pains would become worse after eating dosa, chapatti, green vegetables, spicy foods, and using tea or coffee. In order to control pain and associated bowel habits, the patient started eating less (including reduced consumption of vegetables) and would avoid eating several items. He would also avoid going and eating in public places due to the fear of getting abdominal pain and needing to defecate. On a typical day, he would use toilet 5–7 times. Over time he started avoiding going to various places, both known or unknown and had stopped travelling due to fear of pain and leakage. Gradually, he became partially house bound with marked impairment in social functioning. He was able to continue working despite abdominal pain and frequent visits to toilet that significantly affected his occupational performance. He used to feel helpless and worried about his gastroenterological condition. There was no past history of physical, neurological or psychiatric illness. Personal history revealed ongoing stressors due to financial losses in the recent past and associated worsening of the symptoms. He had fairly good support from immediate family members including the spouse. There was no family history of psychiatric problems and premorbidly he was noted to be well adjusted. The patient was noted to have worries regarding his health issues and anxious affect during the mental status examination. He had been visiting the gastroenterologists and had been diagnosed to be suffering from IBS. He had been on pharmacological interventions as prescribed but had experienced no significant improvement for the past 5 years. He was then referred to the clinical psychologist for intervention. At the time of initiating psychological intervention, he was not on any medication. To begin with, he was doubtful about the utility of nonmedical intervention for his problems. However, he was cooperative for the same and his motivation for undergoing psychological intervention improved following socialization to the CBT model and building of therapeutic alliance.\nThe patient received eight session of CBT. The CBT consisted of socialization of the patient to CBT model, exposure and response prevention, cognitive reappraisal and relapse prevention. CBT for IBS is based on the understanding of the links between symptom related stimuli, experience of fear, and development of avoidance behaviors. Fear is known to alter motility and avoidance behaviors can result in long term aggravation of symptoms owing to increased psychological salience of symptoms as well as generalized stress.[]\nIn the initial session, the patient was explained the rationale of CBT including principles underlying exposure and response prevention related procedures. A hierarchy of fears associated with consumption of different food items was prepared. It was noted that the patient was avoiding several kinds of food items on which there was no restriction as per the medical advice he had received. In other words, he was trying to follow an extremely restricted diet as self-prescribed. On occurrence of minor sensations of discomfort, he would use the toilet to experience some temporary relief. Either he passed a small quantity of stools or no stools during his visits to the toilet.\nTherapist-assisted gradual exposure started with patient's cooperation in the OPD. He was encouraged to expose himself to stimuli associated with his fears. In this process, he was advised to consume food items lower on the hierarchy (least anxiety provoking) before the session and observe the sensations and associated distress while not using the toilet. Initially, he was anxious and fearful that he might need to use the toilet or would not be able to control. However, proper education about exposure and response prevention helped in motivating him to tolerate the distress and thereby experience a reduction in his anxiety toward the end of the exposure sessions. This step was followed subsequently for food items somewhat higher on the hierarchy. Though he reported tightness in the abdomen during the exposure sessions, he was able to tolerate it without using the toilet. After three sessions, he become comfortable with the process and reported a significant reduction in his abdominal sensations. In the next three sessions, the patient was advised to eat normal food before coming to the sessions for therapist-assisted exposure. After the fourth session, he was able to practice such exercises at home with minimal pain. After 6 sessions, he was able to tolerate minor pain/discomfort without using the toilet and his bowel habits improved significantly (one or twice in a day).\nAt this juncture, he was encouraged to identify catastrophic thoughts and develop alternative more realistic thoughts to help him cope with IBS. This included a discussion of his avoidance of social functions/travel and how this avoidance was providing temporary relief and yet strengthening the fears and interfering with his quality of life. Gradually, he started going out and attending social functions which he had avoided since the past few years. He expressed satisfaction about regaining his independence and his quality of life improved dramatically according to his self-report. He was explained about a relapse of the symptoms and ways to deal with the same.\nAt 9 months follow-up, these gains were maintained and the patient was satisfied with the outcome []. |
A 46-year-old man also reported exercise/activity-related pain and fatigue from early childhood. As patient 1, he was always considered to be “a lazy child”. He was not able to run and physical activities such as walking or swimming were challenging. As a child he reported trying to build a good relationship with his physical education teachers, so they would feel pity for him and he could avoid any strenuous physical activities (PA). He experienced three severe episodes of rhabdomyolyses in his life. The first one happened during childhood, which followed vigorous physical activities. The second rhabdomyolysis episode was at the age of 18 following multiple squats, which he performed during a physical test for military service. Not qualifying for the military service, instead he had to complete a civilian service at the university hospital at the age of 22. As he was clearly weaker than his colleagues, a diagnostic investigation took place, which included a skeletal muscle biopsy. At that time, doctors advised him to avoid excessive physical effort. He was told about the second wind phenomenon; however, it was not explained how to reach it. Additionally, he was recommended to eat a maximum of 20% of his daily food intake in carbohydrates. However, the rest of the macronutrients recommendations were not specified. In the following years, his physical activity level decreased, leading to physical deconditioning, loss of muscle mass, and increase in fatty adipose tissue and body weight.\nThe third rhabdomyolysis episode was experienced one year ago and was nearly fatal. He was undergoing physical assessment for the insurance company and pushed himself too hard, resulting in a severe contracture of his lower back muscles. He was admitted to hospital. During the hospital stay he was also diagnosed with myocarditis. Following this episode, he decided to learn more about the condition to manage it better.\nHe began doing gentle aerobic exercises, which improved his ability to attain a second wind. He attended a conference where he met a Spanish team from whom he learnt about the strength training trial performed in Madrid and decided to try it himself in a local gym []. Initially he approached a personal trainer. However, he did not feel the trainer understood his condition and was pushing him too hard. He decided to write his own programme based on strength training principles and exercises he learnt at the conferences and meetings.\nWhen in the gym he reports always doing an aerobic “warm up” by cycling on a stationary bike for 20 minutes, he then exercises on resistance machines (chest press, seated row, butterfly, chest horizontal adduction, reversed butterfly, rare deltoids and trapezius, lat pulldown, leg press, leg adductors, and leg abductors). He finishes his sessions with more aerobic exercise by walking on a treadmill and/or cycling on a stationary bike. and illustrate his progress during the first three months of training (based on personal records). He completes between 5 and 15 repetitions of each exercise with one-minute rest in between sets. He stops a set of exercise earlier if he feels any discomfort in the muscle.\nHe did not report Delayed Onset Muscle Soreness (DOMS) or McArdle symptoms following initial sessions. After three months of resistance training, he found his sleep pattern, overall stamina and McArdle symptoms improved. He is now able to walk two kilometres stopping only once, as opposed to previously when he would have to stop multiple times. He has also found it easier to perform everyday tasks such as changing a car tyre. He has been eating a balanced diet. He autonomously chose not to take any supplements containing glucose pre- or intratraining session. |
A 6-month-old female infant had presented five times to medical services since birth with a history of discharge from the umbilicus. On days 2 and 4, she presented with bleeding from the umbilical stump, which was managed with a pressure dressing. At 2 weeks of age, she attended a general practitioner surgery with discharge of watery liquid from the umbilicus, and it was treated like an umbilical granuloma with silver nitrate application. She came to the paediatric assessment unit of the local district general hospital at 3 weeks of age because of ongoing discharge. The periumbilical skin area appeared raw with features of a chemical dermatitis. An umbilical swab was taken and treatment with oral flucloxacillin commenced. The child presented again with ongoing symptoms. This time, it was diagnosed as excoriated umbilical granuloma with possible tinea corporis infection and she was treated with miconazole and neomycin. The discharge was mainly serous fluid which occasionally turned serosanguinous but did not contain pus.\nAt 6 months of age, she was rereferred by the general practitioner for persistent umbilical discharge with intermittent blood-stained fluid whilst she had been on her third course of antibiotics. On this occasion a consultant paediatrician reviewed the infant. An ultrasound was requested to investigate for remnants of an omphalomesenteric duct because of the persistence of symptoms. The ultrasound showed a cystic structure of 5 mm diameter with echogenic walls situated deep to the umbilicus (see ).\nThere was no communication with the bowel. A fistula extended through the anterior abdominal wall to the umbilicus. She was referred to surgeons for further management. She had a surgical excision of the cyst and sinus tract at 7 months. On macroscopic analysis, it was found to be a hard cystic lesion below the umbilicus surrounded by thick chronic inflammatory tissue. A fistula extended through the abdominal wall with no obvious communication or tract to the peritoneal cavity except for its continuation into the ligamentum teres. Histology showed skin with exudates and ulceration overlying a cystic structure lined by gastric corpus type mucosa in keeping with an umbilical cyst lined by ectopic gastric mucosa (). Her postoperative recovery was uneventful, discharge and dermatitis resolved and she was discharged from hospital care. |
A 44-year-old Japanese male with no significant medical history other than migraines presented to a primary care clinic with 10 days of right neck pain that was not alleviated by treatment with 60 mg of loxoprofen sodium taken three times a day. He had had six episodes of this neck pain in the previous 5 years but had not visited his doctor because it had disappeared within a week each time. He had no history of chronic disease.\nOn physical examination, the patient was not pale, and his body temperature was 36.6°C. His initial blood pressure was 113/72 mmHg, his heart rate was 72 beats/min, his respiratory rate was 18/min, and his oxygen saturation was 99%. He had severe tenderness over his right carotid bifurcation but had no vascular bruit. The pain was constant and moderate in intensity and occasionally radiated to the inside of his ipsilateral ear and lower jaw. The pain was not made worse by swallowing, but it was exacerbated when he rotated his neck and opened his mouth widely. There was no tenderness over his thyroid or neck muscles. He had no pain elsewhere in his body, and there was no skin rash, lymphadenopathy, or inflammatory changes to his throat. His mucous membranes were moist, heart sounds were normal, lungs were clear, and abdomen was soft and nondistended. He had no peripheral edema.\nThe patient's laboratory values were within normal limits without any inflammatory findings (Table ). His blood cell count, liver and renal function tests, lipid tests, blood glucose, glycohemoglobin, and electrolytes were also in the normal ranges. There were negative findings for auto-nuclear antibodies and antineutrophil cytoplasmic antibodies. An ultrasound revealed a hypoechoic change of the outside wall of the carotid artery (Fig. ), slight outward extension of the vessel, mild thickening of the carotid wall, and mild narrowing of the vessel lumen (Fig. ). The maximum intima-media thickness of the right carotid bifurcation decreased in 2 weeks (Fig. ) over the course of 1 week with the outward extension also disappearing during this time period.\nBased on the findings described above, we made the diagnosis of TIPIC syndrome. We told the patient the details of this disease, and he consented to our continuing to observe him without any treatment and to follow his condition with ultrasonography. Over the following week, his neck pain improved spontaneously and completely disappeared without any treatment. An ultrasound 2 weeks later showed that the wall bulging, endothelial thickening, and hypoechoic change of the outside of the carotid artery had improved and nearly disappeared. |
A 75-year-old woman with a history of coronary artery disease (status post- coronary artery bypass graft seven years ago) and RA (diagnosed ten years back being treated with 10 mg/day of prednisone and 200 mg/day of Plaquenil) was admitted to the hospital for evaluation of new-onset seizure. She was followed by a rheumatologist when she was living in Texas several years back, and presently it is managed by her primary care physician. The patient has tried several medications for her RA, including Imuran and monthly injections, and reports that only prednisone helps her arthritis.\nA CT scan of the head done in the Emergency Room did not show any acute intracranial process but suggested the possibility of a cervical spine fracture. Dedicated CT of the cervical spine did confirm type II odontoid fracture (\n). The patient was evaluated by Neurosurgery and opted for conservative management with a cervical collar as the patient did not have any neurological symptoms. On the night of admission to the medical floor, the patient developed hypoxemia and was started on supplemental oxygen through the nasal cannula. However, her saturation continued to deteriorate, leading to the development of severe bradycardia was transferred to the Intensive Care Unit for further management. Urgent CT pulmonary angiogram did not show any embolism nor pleuro-parenchymal changes. In the intensive care unit, the patient was initially treated with nasal cannula oxygen and later high flow oxygen without any improvement in saturation. The patient reported some breathlessness but was not in distress. Clinical examination was normal. This newly diagnosed unexplained hypoxemia was attributed to the hard cervical collar leading to airway compromise. However, despite trying a different sized hard collar and soft cervical collar, she continued to show hypoxemia. The patient was later started on with Bilevel Positive Airway Pressure (BIPAP) with improvement in saturation. By evening, the saturation dropped to 70% despite the up-titration of BiPAP settings and developed sinus bradycardia with a heart rate of 30 beats/min. The patient was emergently intubated. Due to cervical cord fracture, intubation was done with a fibro-optic pediatric bronchoscope, and seven number endotracheal tube was placed without any difficulty. Arytenoid swelling was noted during intubation, but due to emergency procedure and lack of video monitoring, it was not explored further.\nThe next day, video bronchoscopy performed through the endotracheal tube did not reveal any endobronchial pathology. However, bronchoscopy through the right nostril showed evidence of significant arytenoid swelling compromising the upper airway space (\n). On reviewing the CT scan of the cervical spine done in the emergency room, there was evidence of arytenoid cartilage sclerosis and cricoarytenoid joint involvement secondary to RA (\n). Further collateral history was obtained from the patient's daughter, who denied any prior history of speech, swallow, or breathing problem. The patient has only multiple joint pain, stiffness, and deformity without any other organ involvement RA. The patient smoked a pack of cigarettes several years back, quit smoking seven years back when she had heart surgery. She has not been diagnosed with chronic obstructive pulmonary disease nor with any sleep-related problems before.\nBased on these findings, the patient was diagnosed with sudden onset of cricoarytenoid arthritis due to RA. After intubation, the patient did not show any evidence of desaturation but developed ventilator-associated pneumonia and was treated with antibiotics. The patient was treated with 60 mg/day prednisone for a week, along with antibiotics and bronchodilators. She did not show any improvement in upper airway obstruction without any air leak around the vocal cords during the air-leak test done on the ventilator. The patient underwent tracheostomy and was transferred to Long-Term Acute Care for further management after a week of hospitalization. She was later weaned from the ventilator but could not be decannulated from tracheostomy. She was discharged with 6 mm Shiley™ tracheostomy tube along with supplemental oxygen. During her follow-up visit to ENT clinic, a direct laryngoscope continued to show arytenoid swelling and the vocal cords fixed in the midline position. The patient wase also followed up with rheumatology and stared on Imuran therapy and being worked up for biological agents. Steroids were taperedd off and stopped completely. |
A 54-year-old male presented to surgical emergency room of Aleppo University Hospital with the complaints of sharp pain in the left lower extremity with pallor and coldness, in addition to the presence of coldness with no pulse in the lower right extremity, these symptoms are consistent with the presence of acute arterial obstruction in the left lower extremity.\nDuring the clinical examination of the abdomen, a large and pulsating mass was noted from the abdomen wall. Despite the presence of this very large pulsating mass, the patient did not complain of any digestive symptoms such as digestive discomfort, abdominal pain or bloody defecation. The clinical history indicated the existence of a previous surgical operation to repair anal hemorrhoids 8 years ago, in addition to the presence of orally treated systemic hypertension. Notably, the patient smoked an average of one pack per day for 40 years.\nInitial blood tests included a white blood cell count of 8.8 × 10^9/L, hemoglobin level 10.7, platelets count 171 × 10^3/UL. Laboratory tests for electrolytes, liver and kidney enzymes were within the normal ranges.\nA multislice CT scan with contrast was performed showing the presence of a dissected aneurysm of approximately 8 × 13 cm with complete obstruction of the abdominal aortic lumen below the renal arteries (, ). The open surgery was performed with the participation of four doctors from the vascular surgery department, an anesthesiologist and a nurse. We used halothane for general anesthesia. We removed a large aneurysm (), and installed a 16 × 8 double aortofemoral dacron joint (). The operation took 2 h, We did not face any difficulties during the surgery, but we were very careful to deal sensitively with aneurysms of this large size. A surgical drain was installed. Fortunately, it was within 24 hours, as no purulent or serous oozing was noticed. To avoid infection of the wounds after surgery, metronidazole was given with ceftriaxone intravenously, with intravenous paracetamol for pain relief. The patient was admitted to the intesive care unit for the first day, then he was transferred to the surgery department. He was discharged after 5 days. The patient's condition was followed up for 3 months by telemedicine, no complications were observed and the results were very acceptable. |
To illustrate this method, we present the case of a 14-year-old boy suffering from a denture-related erythematous stomatitis (). Denture-related stomatitis is defined as an inflammatory process of the oral mucosa underlying a removable dental prosthesis. In young patients, a denture-related stomatitis could be due to a complication of a pediatric prosthetic denture or can occur during long-term orthodontic treatment with a removable material. This pathology has been described among both children and teenagers, and diagnosis methods and treatment have been proposed [–]. Here, a removable orthodontic appliance was worn for two years to compensate a dental agenesis.\nA classical microscopical procedure typically involves removing a representative sample from the infected site (exfoliative cytology) which is transferred to a microscopic slide and treated with potassium hydroxide (KOH), Gram stain, or periodic acid-Schiff (PAS) stain. A sample collection was performed and sent to laboratory for cultivation and the result was positive for Candida albicans.\nThen we provided simplified, direct microscopy method as comparison. In this method, the patient's saliva was collected on the floor of the mouth. An intraoral mirror was placed horizontally beneath the tongue, in contact with mucosa. When saliva covers the mirror, it is removed from the mouth and laid on the slide (). The sample was subsequently collected with a sterile probe directly placed into the patient's saliva (), and a cover slip was mounted. An important point to note is that the sulcus area is the optimal site to collect the sample using this method (). The practitioner should press with his or her finger on the slide to spread the sample. The sample was then analyzed under a phase contrast optical microscope (). The most interesting magnification is ×1000 as it allows nonpathogenic yeast forms to be differentiated from opportunistic hyphal forms. In pathological conditions, several hyphae are visible on each screen, mixed with oral bacteria and cells (). Importantly, this method is valuable for determining the efficacy of treatment. The treatment consisted in 3 weeks of local treatment using antifungal agent (amphotericin B), and the modification of the prosthetic appliance for a fixed one. An other laboratory analysis by cultivation was negative. At the two years follow-up, the same procedure showed a normalization of oral flora, with absence of hyphae (), linked to a clinical improvement (). |
A 30-year-old female presented to our hospital with the chief complaints of left flank pain and dysuria since 9 months. Patient had no complaints of fever or hematuria. There was a history of occasional tobacco chewing since the past 10 years. There was no relevant medical history or history of any surgical intervention. Physical examination was unremarkable apart from poor dental hygiene and some missing teeth. Laboratory investigations revealed normal hemoglobin level, total leukocyte count, kidney function tests, and viral markers. The routine urine examination was negative for red blood cells, white blood cells, protein, and glucose.\nTransvaginal and transabdominal ultrasound scans revealed a 29 × 18 mm heterogeneously hyperechoic nondependent mass lesion [] arising from the right superior wall (dome) of the urinary bladder with multiple areas of calcification and dense posterior acoustic shadowing. Rest of the urinary bladder wall had normal thickness. Bilateral ovaries were normal in size and echotexture and were separate from the bladder wall []. Uterus was normal in shape, size, and echotexture. No fluid or collection was noted in the pouch of Douglas.\nCT scan was performed and showed a 35 × 36 mm relatively well-defined heterogeneous mass lesion in the urinary bladder on the right side of the dome with multiple specks of calcification and internal discrete hypodense areas within the mass lesion (CT attenuation of fat) on noncontrast scans. Multiple linear strands of calcification were noted extending anteroposteriorly on both sides of the mass lesion. On postcontrast images, no enhancement of the mass lesion was noted. The mass lesion was protruding in the lumen and was also seen to extend laterally out of the bladder contour []; the lesion was close to but separate from the right ovary []. Rest of the urinary bladder was normal in outline and wall thickness. No fat stranding was noted in the surrounding areas. Few calcific foci were incidentally noted in the spleen alongwith left small kidney with a 22 × 19 mm calculus in renal pelvis causing moderate hydronephrosis. Rest of the abdominal viscera was unremarkable.\nCystoscopy and transurethral resection (TUR) biopsy revealed a hard bladder mass having hair and calcifications on the surface and yellowish fat in the centre involving the bladder dome on the right side. TUR biopsy specimen was sent for histopathological examination and revealed skin comprising stratified squamous epithelium (epidermis) and dermis with adnexal structures including sebaceous glands [] and sweat glands []. Lobules of mature fat and densely fibrotic stroma were observed []. Based on imaging, cystoscopy, and histopathology, a diagnosis of dermoid cyst of urinary bladder was entertained. |
On Feb. 3, 1982, a 33 year old man, an industrial worker, was admitted to the general surgery department of Korea University Hospital because of intermittent claudication and longstanding ulceration of the right leg, which developed following acupuncture on his right leg. The perfusion was very poor and the arterial pulses were not palpable over the femoral arteries and down either leg. An aorto-femoral angiogram revealed occlusion of the right femoral artery with patent anterior tibial, posterior tibial, and peroneal arteries, and the occlusion of the left internal iliac artery with collateral arteries.\nFor the purpose of limb salvage, embolectomy and right femoro-tibial saphenous vein bypass graft surgery was attempted. There was no evidence of atherosclerosis in the leg vessels. Twelve hours postoperatively, the bypass graft stopped functioning. Reoperation was done and thrombectomy was carried out, but the patient finally had to undergo above-knee amputation on the right leg at a later date. During this admission, the patient experienced multiple episodes of precordial chest pain and an episode of colicky left flank pain.\nAccording to the past medical history, he was first admitted to Korea University Hospital because of severe chest pain, which awakened him early in the morning of July 23, 1976. The electrocardiogram on admission showed changes compatible with an acute inferior wall myocardial infarction (), the peak SGOT was 230 IU L and the peak LDH was 1500 U ml (Wrobleski). The hospital course was uneventful except for the intermittent episodes of abdominal pain. He was discharged on Aug. 16, 1976.\nBetween Aug., 1976 and Feb., 1982, the patient had to be amitted to the hospital three times. The first time was for the sudden development of dizziness, the second time for abdominal pain due to ileus, and the third time because of chest tightness and abdominal pain.\nHe had had an appendectomy at the age of 13 and had contracted typhoid fever at 14. According to his history his father and younger brother had hypertension. He smoked about two packs of cigarettes per day and consumed a little alcohol.\nThe chest X-ray revealed mild cardiomegaly with a prominent left ventricle. Occasional premature ventricular contractions were noted on the twelve-lead electrocardiogram. The hemoglobin was 14.0 gm dl; hematocrit 42%: ESR 30 mm hr: WBC 12,250 with 50% of segs, 22% of lymphs, 10% of monos, and 12% of eosinophils. The platelet count was 258,000 mm3. The bleeding time and the coagulation time were normal. The serum cholesterol was 167 mg dl and the triglyceride was 55 mg dl. The lipoprotein electrophoresis revealed that α-lipoprotein was 22.7%, prebeta 28.6%, and beta 48.7%, the RA factor was negative. The protein electrophoresis revealed albumin 50.9%, α1 3.2%, α2 10.9%, β13.8%, and γ21.2%. The patient was discharged on Apr. 14, 1982.\nOn July 3, 1982, the patient was admitted to the hospital because of abdominal pain. The abdomen was tender and distended, and the bowel sound was diminished. Under the impression of mesenteric artery thromboembolism heparinization and subsequent coumadinization was done. To rule out the possibility of embolization from the heart two dimensional echocardiography was done. This reveled a large, pedunculated mass which had its base in the apex of the left ventricle and its end freely movable in the left ventricular outflow tract (). The mitral valve leaflets were seen striking the mass during its motion. The mass was 7×3 cm. The mass reflected echoes which were dense compared with the lighter echoes from the myocardium. The outline of the mass was rather well defined and several daughter masses were noted to be budding from the main mass. The left ventricle was only slightly enlarged in size, and the apex and the distal septum were noted to be akinetic. Under the impression of either a left ventricular thrombus or a myxoma open heart surgery was performed on July 9, 1982.\nIn the operative field, the left ventricular apex was noted to be fibrotic and moderately adhered to the regional pericardium in an area of about 3×5 cm2. The left ventricle was opened through the infarcted area. The organized thrombus -like mass, which was pedunculated from the inner surface of the apex and intermixed with the muscular trabeculae was removed en bolc with the central infarcted apical wall to preserve the total continuity of the mass. The left ventricular wound was closed with heavy, interrupted, cross suture and pledgeted dacron flet velour.\nThe gross specimen measured 7.0×4.5×2.7cm and weighed 35 gm (): On pathologic examination the mass was confirmed to be a thrombus. The surface of the mass was irregular and grayish-pink colored with multiple hemorrhagic foci. On the cut surface could be seen whitish-yellow-to-pinkish-brown, soft tissue with interlacing bundles. The microscopic examination revealed fibrinous lamellated eosinophilic material mixed with coagulated RBSs, WBCs, platelets, cholesterol clefts, and focal calcifications. At the periphery of the mass, fibroblastic proliferation was seen, sections from the right autricle and the left ventricle revealed the coagulative necrosis of myocardial fibers and fibroblastic proliferation.\nOn July 23, 1982, he developed impending gangrene of the left leg. Thromboembolectomy of the left femoral artery was done without success. Finally, he had to undergo the above knee amputation on the left leg. During this admission, the patient experienced squeezing precordial pain and intermittent abdominal pain.\nOn Aug. 30, 1982, factor VIII concentration was 200% and factor V 100% of the normal control. The euglobulin lysis time was 3 hours. FDP was less than 10 μg/dl. The fibrinogen was 407 mg/dl. The serum antithrombin III level was within normal range. The platelet aggregation test revealed increased aggregability with ADP (patient, 91.3%; control, 77.5%), epinephrine (patient, 82.5%; control, 72.5%), and collagen (patient, 81.3%; control, 70.0%).\nThe two-dimensional echocardiography repeated on Oct 6, 1982 revealed no evidence of recurrence of thrombus in the left ventricle. The patient was discharged on Now. 16, 1982. He has been followed in the outpatient clinic and has been doing reasonably well so far, except for occasional mild left precordial chest pain. A thallium-201 myocardial imaging was done on June 29, 1983, by injecting 2 mCi of thallium-201 intravenously following the intravenous injection of 7 mg of dipyridamole. It showed a perfusion defect of the inferior wall, the site of the previous myocardial infarction. The perfusion defect did not improve with the delayed images taken four hours later. |
A 20-year-old woman presented to the emergency department with complaints of severe abdominal pain, multiple episodes of vomiting and obstipation for one day. She was a known case of abdominal tuberculosis and had received anti-tubercular treatment for two months. Vital signs showed a blood pressure of 100/64 mm Hg, heart rate of 110 per minute, respiratory rate of 18 per minute, oxygen saturation of 96% on room air and a temperature of 100.5°F. She was cachectic with a weight of 28 kg which was less than 5th percentile for gender and age matched normal population. Physical examination revealed diffuse abdominal tenderness and rigidity. Her erect abdominal radiography showed gas under right dome of the diaphragm. Clinical diagnosis of perforation peritonitis was made and the patient was wheeled to the operating room for emergent laparotomy. Intraoperatively, a 3 cm x 2 cm single ileal perforation was found 1 foot proximal to the ileocecal junction, with unhealthy bowel margins and multiple mesenteric lymphadenopathy. Resection of perforated bowel segment with proximal ileostomy and mucus fistula of distal ileum was performed. Later, histopathology report confirmed tubercular etiology showing caseous necrosis in mesenteric lymph nodes and epithelioid granuloma in ulcer edge biopsy. Two days after the surgery, the patient was started on oral feeds and was accepting meals orally subsequently. Ten days following surgery, the patient had multiple episodes of bilious vomiting with stoma output decreasing to less than 100 mL per day. Her abdomen remained flat with no increase in bowel sounds. Abdominal radiography showed dilated stomach with no air fluid levels. Oral feeds were stopped and a nasogastric tube was placed for drainage of gastric contents. A contrast enhanced computed tomography (CECT) abdomen was done to determine the cause of obstruction which revealed a decreased aortomesenteric angle of 15° (Figure ).\nAs a result of this decreased angle, an abrupt collapse of the third part of the duodenum was seen along with proximal duodenal and stomach dilation (Figure ).\nBased on the clinical features and CECT findings, diagnosis of SMA syndrome was made and the patient was managed conservatively. The patient was not tolerating enteral feeds and no relief of postprandial abdominal pain was noticed even on changing to left lateral decubitus or prone position. Considering the patient’s nutritional condition, total parenteral nutrition (TPN) was started. Anti-tubercular drugs were continued with oral sips. She was started on 1500 kcal/day (50 kcal/kg/day), taking care of refeeding syndrome and was gradually titrated up to provide maximal caloric support. Monitoring of weight, blood glucose, electrolytes, liver function test, and lipid profile was done to prevent any TPN complications. A good clinical response was seen after starting TPN. The patient’s weight gradually increased to 34 kg in three weeks and gradually the stoma output increased and nasogastric drainage decreased. TPN was tapered off gradually with concurrent starting of oral feeds. On discharge, the patient was advised a high caloric and protein diet with small frequent meals. A follow-up CECT was done after six weeks from discharge which showed an increase in aortomesenteric angle from 15° to 21° (Figure ) with the patient being asymptomatic and a total weight gain of 12 kg post-surgery. |
A 25-year-old gravida 3, para1 was admitted at 19 weeks of pregnancy with a history of vaginal bleeding for 1 week and pain in abdomen. She was told that she had a low lying placenta during her second trimester scan. A lower segment caesarean section for cephalopelvic disproportion was done 2 years back and the baby was alive and well. This was followed by a missed abortion at 8 weeks for which a curettage was done. At admission, her vitals were stable. Uterus was irritable. Hb was 7 gm/dL. Ultrasound done after admission showed a live fetus of 20 weeks and anterior placenta with a thin, bulging, and deficient lower uterine segment. The decidual interface between the placenta and myometrium was partially absent and there were large dilated vessels in the same area. These sonographic features were suggestive of a placenta accreta. Patient continued to bleed; 4 units of blood were crossmatched and injection of methotrexate 50 mg was given intramuscularly on the day of admission. Prophylactic uterine artery embolisation was done on day 2 but the bleeding continued. The next day she was taken up for hysterotomy under general anaesthesia. Entry into peritoneal cavity was difficult because of dense adhesions. There was no hemoperitoneum. Bladder was adherent to the lower uterine segment which was severely deficient. Placenta was encroaching on the left broad ligament and was covered by a thin layer of peritoneum. Bladder was dissected from the lower uterine segment and incision was given at the previous scar. Fetus was extracted out first and placenta was then clearly seen to be firmly adherent to myometrium at several sites. Most of it could be removed piecemeal. Hemorrhage was controlled by uterotonics and hemostatic sutures at the placental bed. The estimated blood loss was approximately 1 litre. Two units of blood were transfused intraoperatively. Her postoperative Hb was 8 gm/dL. Catheter was removed on day 5. Recovery was uneventful. She was discharged 1 week later on iron tablets and contraceptive advice. |
The patient was a 65-year-old woman without notable antecedents presented to our institution for progressive left hip pain for approximately 8 months. It was a mechanical pain of the hip well relieved by the usual analgesics. The appearance of walking distance and the poor response to analgesics forced her to consult in our center.\nThe BMI was 35,5. The walk was almost normal. There was no cutaneous scar on the lateral side of the left hip or on the ipsilateral buttock. There was a good trophicity of the abductors. Lateral rotation and abduction were markedly diminished. The rest of the exam was strictly normal. The pelvis AP () and lateral () left hip radiographs revealed signs of hip osteoarthritis. We concluded that it was a symptomatic left hip osteoarthritis that was more and more disabling in an obese woman of 71 years with no particular history. We indicated THA by posterolateral approach.\nIn the operating room, after the skin incision and subcutaneous haemostasis, we discovered in the adipose tissue about 5 cm thick a kind of well-circumscribed shell of about 2.5 cm of axis. Her incision gave rise to a whitish, thick color, looks a little oily collection (), resembling a purulent collection (). A sample for bacteriological investigation in a lab was carried out. The hull with its clear boundaries within the gluteal fat was resected and entrusted to the pathologist. All the neighborhood tissues were healthy (very localized lesion).\nIn front of this collection which appeared to be purulent, we limited ourselves to the resection of this hull, the cleaning of the wound, and the deferred implantation of the prosthesis.\nCytobacteriological examination of the specimen revealed its greasy appearance, epithelial and lymphocytic cells; there were no visible germs. Histological examination of the resected shell revealed a fibrous wall with chronic inflammatory remodeling made of lymphocytes and plasma cells with no necrosis centers.\nIn the light of these laboratory results, we conducted the interview of the patient, who reported a notion of malaria for about two months to the screen treated with an intramuscular injection on the right buttock of the compounds derived from artemisinin. We found the result of the thick drop before the injection which was positive and that of the injection which had not been negated; the patient was then successfully treated orally. The sample was sent to a lab for confirmation by artemether identification by thin layer chromatography (TLC).\nA sample of 40 g of human fat was treated with ethyl acetate (50 ml × 3) after filtration on Whatman paper, the solvent was evaporated, and the residue was taken up with acetone (40 ml) constituting the sample to be analyzed. Artemether was purchased from a local pharmaceutical company.\nImplementation of the TLC: solution to be analyzed: 20 μl of sample; control: artemether (80 mg/mL), 10 μl deposit; support: silica gel GF254; mobile phase (10 ml): dichloromethane, ethyl acetate (7/3); and developer: 25 ml anisaldehyde reagent, 5 ml concentrated acetic acid, 450 ml ethanol, and 25 ml concentrated sulfuric acid. Using a capillary tube, 20 μl of the sample was deposited on the plate (silica gel GF254), the control 10 μl. The plate is placed in a tank previously saturated with the migration or elution solvent (mobile phase) which covers the bottom of the tank at 5 mm height. The migration of eluting solvent causes the substances contained in the samples at various speeds; spots are formed characterizing the substances present in the sample.\nThe plate was removed from the tank as soon as the solvent front reached about 9 cm. The plate was dried and observed under a UV lamp at 254 nm and then revealed with the developer which will characterize the artemether in human fat.\nThe plate then shows an orange spot on the left side of the sample and a spot with the same color on the right side of the control; the two spots have the same front report as shown in . This indicates that there was artemether in this human fat sample. |
A 68-year-old male presented to the head and neck clinic with history of a progressive midline lower neck swelling which appeared suddenly, increased in size progressively and became prominent over 6 weeks. On examination, the swelling was about 5 × 2 cm in size, mobile and not adherent to the skin or underlying structures. The swelling was not prominent on tongue protuberance. Thyroid was impalpable and patient was clinically euthyroid with no evident Grave's disease features. It was neither associated with fever nor any other constitutional symptoms. His pressure symptom includes mild dysphagia to solids which compelled him to visit our hospital, but he did not encounter any respiratory complaint.\nPatient was not on any anticoagulants, but he was taking regular medications for diabetes and hypertension for many years.\nFor treatment decision, the patient was referred to radiology department. Ultrasound neck showed a large well defined thin-walled cystic neck lesion measuring about 5.1 × 2.6 cm (transverse x anteroposterior). There was no appreciable internal septation or solid tissue in it (). No hypervascularity was noted within or periphery of the lesion (). The internal content of the cyst was of 2 types, basal part was homogenously low echogenic with curved upper border while rest of the part was filled up with anechoic fluid (). Few tiny echogenic foci were also seen in its low echogenic part which were not giving any comet tail artifact to be confidently called as colloid content (). Thyroid gland was not easily visible due to lateral push by this large cyst. Thyroid isthmus was hardly visible clearly. Lobes were found to be normal, but their echotexture was heterogenous and micronodular with normal vascularity and intact echogenicity. According to the location and content of cyst, the closest sonographic differential in our patient was thyroglossal cyst, but it is usually smaller in size, moves with tongue protrusion and more cranial in position. Other differentials could be hemorrhagic or fourth branchial cleft cyst.\nLeft lobe was having a solid well-defined isoechoic nodule measuring around 2.5 × 2.0 × 1.5 cm with some internal vascularity on doppler ultrasound ().\nConsidering the differentials and ambiguous site of mass origin, referring team discussed the case with radiologist and planned to proceed for contrast enhanced computed tomography neck. Computed tomography (CT) showed a non-enhancing midline cyst in the region of isthmus measuring around 5.1 × 3.5 × 5.6 cm (transverse x anteroposterior x craniocaudal). It was pushing both lobes of the thyroid gland inferolaterally and strap muscles anterolaterally with no signs of aggression. The craniocaudal extension of the cyst was from the level of the thyroid cartilage to just above the clavicles (). The attenuation of the fluid content was varying between 27-51 Hounsfield Unit. No calcification was appreciated (). No worth noting mass effect or its connection with the airway was noted. On careful inspection of images, isthmus was unappreciable and we were able to see a “claw sign” in the thyroid lobes which helped us to discern the organ of origin (). The differentials of non-inflammatory thyroid cysts including atypical colloid and hemorrhagic cyst were given on CT scan with a suggestion of cyst aspiration to have definitive diagnosis. 3D volume rendered images for skin demonstrated clinically visible swelling (). 3D volume rendered airway images clearly showed no mass effect (). After observation of “claw sign” on CT scan we ruled out thyroglossal and branchial cysts from our differential list.\nLab results showed strongly positive antithyroid peroxidase antibodies (TPO), while thyroid stimulating hormone, triiodothyronine (T3) and thyroxine (T4) were normal. Rest of the blood examination findings were unremarkable.\nPathology assessment of the diagnostic aspirate showed, 18 ml of fluid having abundant colloid, few scattered foamy macrophages and hemosiderin. It was consistent with benign cystic colloid nodule with hemorrhagic changes, Bethesda Category II. It was found negative for malignancy. Fine needle aspiration (FNA) of the left thyroid nodule showed small groups as well as isolated follicular cells with focal Hurthle cell changes and rare collection of foamy macrophages in the background of thick colloid. Few clusters showed follicular epithelium with focal nuclear atypia including nuclear overlapping and grooving, putting it under Bethesda category III with further advise to have follow up and repeat FNA at appropriate interval.\nDiagnostic aspiration was later followed by therapeutic colloid aspiration. On follow up visit, symptom of dysphagia was resolved. Due to atypical cells on histopathology and high-risk age group for thyroid malignancy, left hemithyroidectomy was done on subsequent visit. Patient did not report further complaints. |
A 83-year-old African American female presented to the cancer clinic complaining of the right leg swelling. She has a past medical history of hypertension and dyslipidemia. She was diagnosed with melanoma in her right leg about 2 years ago in 2013. She subsequently had her first and second toe amputated. However, she did not receive any adjuvant chemotherapy or radiation treatment. She denies any weight loss, night sweats, fever, dizziness, headache, hemoptysis or visual problems. Physical examination was significant for right leg swelling and right inguinal lymphadenopathy of approximately 5 cm in diameter. She also had vitiligo skin changes limited to both hands. Doppler ultrasound of the right lower extremity was negative for any deep venous thrombosis.\nA whole body positron emission tomography (PET) and computed tomography (CT) was done. The PET/CT scan revealed hypermetabolic 6 mm lymph node behind superior vena cava/ascending aorta, 5 mm hypermetabolic lymph node anterior to descending thoracic aorta, and another 5 mm hypermetabolic soft tissue density posterolateral to the bifurcation of the aorta. However, the most significant finding was a 43 mm × 30 mm size right inguinal lymph node, which was markedly hypermetabolic with a maximum SUV of 44.5. Following the results of PET scan, biopsy of the right inguinal lymph node was obtained. The biopsy result showed malignant cells positive for melanoma. A BRAF mutation analysis was done which was negative. A c-kit mutation analysis was also negative.\nThe patient was then started on anti-PD1 therapy with nivolumab to treat metastatic melanoma. She clinically responded well to the treatment with minimal side effects. After 5 cycles of nivolumab therapy, a repeat PET scan was obtained. The results showed a decrease in metabolic activity of the lesion at the anterior aspect of the right leg with a resolution of the other hypermetabolic foci at the ankle. Decreased size of the metabolic activity of the right inguinal lymph node and decreased metabolic activity of the lesion at the aortic bifurcation. On the other hand, the lymph nodes within the mediastinum had persistent hypermetabolism and a new hypermetabolic lymph node at the bilateral hilar regions. There was the resolution of the focus hypermetabolism at the right thyroid lobe, but a new diffuse hypermetabolism which indicates thyroiditis. No treatment was initiated at this time because she was asymptomatic with normal thyroid-stimulating hormone (TSH) levels.\nAbout 5 months later, she presented to the hospital complaining of fatigue, forgetfulness and symptomatic bradycardia. Chest X-ray was normal. CT scan and magnetic resonance imaging of the head were unremarkable. At this time, further laboratory investigations revealed a TSH was 36.4 mU/l (normal 0.5–4.7) with free T4 of 0.25 (normal 0.73–2). The thyroid peroxidase antibody levels were >600 was diagnosed with acute autoimmune thyroiditis. The patient was started on levothyroxine supplementation which resulting in dramatic clinical improvement.\nThree weeks later, she was again admitted to the hospital after she was discovered to be hypersomnolent and severely lethargic. Her laboratory work showed a TSH level of 1.92 Mu/l, free T4 0.87. Cortisol level was drawn, and it was <0.5 μg/dl and thyroid peroxidase antibody levels >600. Insulin-like growth factor-1 and prolactin levels were normal. She was diagnosed with acute severe autoimmune adrenal insufficiency and acute autoimmune thyroiditis related to Nivolumab therapy. The adrenocorticotropic hormone (ACTH) level was <0.01 pg/mL confirming a central cause for the adrenal insufficiency of the patient and a possible diagnosis of Nivolumab associated hypophysitis. She was treated with intravenous (IV) hydrocortisone 100 mg every 8 h and IV levothyroxine 75 mcg daily supplementation. Nivolumab had to be stopped due to the immune-mediated side effects. After 2 days of therapy, the patient clinically improved and she became more arousable. She underwent aggressive physical therapy and was subsequently discharged to a rehabilitation center for further care. |
A 52-year-old male presented to us with progressive abdominal discomfort and swelling of 3 months' duration along with loss of appetite and weight. On examination, a huge nontender firm mass (20 cm × 15 cm) occupying the whole of the right side of the abdomen and crossing the midline up to the left midclavicular line was palpable.\nUltrasound showed a large retroperitoneal lesion of size 18.5 cm × 15 cm of heterogeneous echogenicity and encasing the right kidney with loss of fat planes with the right kidney.\nContrast-enhanced computed tomography of abdomen was done which showed a 17 cm × 17 cm × 20 cm heterogeneous right retroperitoneal lesion with peripheral enhancement. Central necrosis was noted with loss of fat planes with right renal cortex postero-inferiorly. The right kidney showed normal contrast enhancement and excretion. The right proximal and mid ureter were deviated medially with loss of intervening fat planes. The lesion was causing compression and superior displacement of the right lobe of liver and anterior and left lateral displacement of pancreas and bowel loops. There was also stretching and medial displacement of the inferior vena cava (IVC), aorta, and other vessels. The IVC was significantly compressed [].[] There was no evidence of any other mass lesion in the remaining retroperitoneum or the GI tract.\nIn view of radiological finding of involvement of the right kidney, a clinical diagnosis of right renal malignancy (leiomyosarcoma) was made and exploratory laparotomy for excision of the mass through a modified chevron incision performed.\nExploratory laparotomy revealed a huge swelling of variegated consistency with no evidence of metastasis to liver and no retroperitoneal lymph nodes. Caecum and ascending colon were adherent to the mass and pushed anteriorly and to the left across the midline. IVC was splayed and adherent to the mass posteromedially. The right kidney was not visible being entirely engulfed by the mass with the right renal vessels entering the mass. Posteriorly, the mass was adherent to the psoas fascia. Frozen section from the medial (left) margin was found to be negative for malignancy. Since the right kidney was engulfed by the mass, the mass was removed en bloc along with the psoas fascia and weighed 5.5 Kg. Additional slices of tissue from the margin of resection at the ascending colon were taken and send for the frozen section which was negative. The course during hospital stay was uneventful and the patient was discharged on day 3 postoperatively.\nHistopathology revealed an encapsulated retroperitoneal tumor composed of solid areas, necrosis and hemorrhage and was completely enclosing the right kidney with tumor cells showing diffuse C-Kit immunostaining [Figures and ]. The patient was started on imatinib mesylate and was doing well till 4 months after surgery on follow-up but was lost to follow-up after that. |
A 42-year-old, G3P2 L0 presented to the obstetrics outpatient department in the second trimester of her third pregnancy for a routine antenatal check up. She had two still births in the past, the details of which are not known. She is not a hypertensive or diabetic. There was no history of any drug intake except for iron and folic acid supplementation. Her routine hematological and biochemical investigations were within normal limits. A routine anomalous scan done at 2nd trimester showed a fetus with features of intrauterine growth retardation along with a large fluid-filled sac in the abdominal region which was compressing the thorax. Both upper and lower extremities were visualized. However, due to oligohydraminos no further comment could be made upon the fetal anatomy. Color flow showed two umbilical arteries along the wall of the cyst. The intraabdominal unilocular cystic mass was assumed to be the urinary bladder and in conjunction with the presence of oligohydraminos and Intrauterine growth retardation a proposed diagnosis of congenital megacystitis or Prune Belly Syndrome was made [Figures and ]. The parents were informed about the diagnosis and after counseling, they opted for termination of pregnancy. The pregnancy was terminated after obtaining the consent, and the abortus was sent for pathological examination.\nAt autopsy, the fetus weighed 75 g and on external examination a number of anomalies were noted. The genitalia were ambiguous. Encephalocoele was seen over the head of the fetus in the occipital region. The most striking abnormality was a left sided anterior abdominal wall defect, from which gastrointestinal organs were protruding. The organs identified included the liver and the intestine. Although these organs were not encased by a membrane, a membrane like structure was present near the anterior abdominal wall opening [Figures and ]. A defect in the posterior abdominal wall was also noted. A number of skeletal abnormalities were noticed. There were scoliosis and amelia of the digits of the right upper limb. The lower limbs exhibited malrotation and were pushed to the right lateral side by the protruding abdominal organs []. The umbilical cord was short and was found in a membrane like structure that was partially covering some of the protruding organs. A radiological examination was also done, which confirmed the gross skeletal anomalies and the encephalocele []. On internal examination, there was agenesis of anal canal along with agenesis of genitourinary tract, and the lungs were hypoplastic. The remaining organs appeared normal on gross examination and congested on microscopy. In view of the above combination of malformations, a diagnosis of LBWC was offered. |
A 42-year-old male presented to the Department of Head and Neck Surgery of a tertiary oncological centre because of right mandibular swelling and trismus. The patient had already been admitted to a secondary care hospital 3 months earlier with complaints of right mandibular discomfort and slight tumefaction. A biopsy was then performed and the diagnosis of ameloblastic carcinoma was made histologically. The patient was otherwise healthy with no significant past medical history, including alcohol, smoking or tobacco abuse.\nA complete head and neck examination revealed a painless, firm and fixed right mandibular mass with no cutaneous inflammatory signs. No ulcers or mucosal lesions were found in the oral cavity. Laboratory evaluation, chest radiograph and respiratory function tests were unremarkable. The patient underwent bronchofibroscopy, which revealed only mild laryngeal hyperaemia.\nBoth neck CT () and MRI () were performed, showing a large, solid tumour arising from the ramus and posterior body of the right mandible. The lesion extended to the surrounding soft tissues, with invasion of the masseter and medial pterygoid muscles and caused bulging of the buccal mucosa. The soft tissue component was hypointense on T1 weighted and hyperintense on T2 weighted MR images and showed avid enhancement after gadolinium administration on MR examination. CT scan disclosed striking sclerosis and irregularity of the mandibular ramus with some gas bubbles inside the medullary cavity and an expansive lytic component in the posterior body and angle with some bone-forming matrix inside. Prominent periosteal reaction was also identified, particularly in the outer cortical surface of the mandibular ramus with the typical pattern of a ruptured Codman triangle. No associated cystic lesion was found in the mandible. No enlarged lymph nodes were detected and the evaluation of the remaining cervical spaces was unremarkable. A thoracic CT scan was also performed, with no parenchymal lesions.\nThe patient underwent a right hemimandibulectomy and ipsilateral cervical lymph node dissection. Surgical resection also included the right submandibular gland and a segment of buccal mucosa that was swollen by the mandibular mass. Reconstruction was performed with free fibula graft. The surgical specimen included a large, white and solid tumour with 11 × 7.5 × 6.5 cm, corresponding to an invasive, moderately differentiated (G2) SCC (). Focal positive margins were found at the medial surface of the specimen. The resected buccal mucosa, submandibular gland and lymph nodes had no neoplastic tissue. Taking into account the imaging staging examinations and the post-surgical histological report, the final TNM stage was stated as pT3 N0 Mx. After surgery, the patient underwent adjuvant chemotherapy (cisplatin-based regimen) and radiotherapy. |
A 45-year-old female presented with macular extremity and facial rash from a rehabilitation center after having been discharged a month earlier from a revision surgery on her gastric bypass for anastomotic bleeding. The gastric surgery itself had been done four years prior to the presentation. On this admission she was noted to be lethargic for the past few days with neurological assessment revealing a Glasgow Coma Scale [] score at 4, necessitating intubation and mechanical ventilation.\nDuring this time, extensive investigations were done including imaging of the head. The Magnetic Resonance Imaging (MRI) of the brain showed symmetrically restricted diffusion (Figure ) was read as suspicious for hypoxic injury. This stood in contrast to any clinically documented hypoperfusion. Routine blood investigations did not offer a significant clue as her electrolytes, hepatic and renal functions were well within normal limits and the only thing that was seen abnormal was normocytic anemia, which in comparison to her past admissions, was found to be stable without an acute drop. Her thyroid profile, cortisol level, vitamin B12, and ammonia levels were checked for completion of the neurological workup and she was found to have elevated ammonia at 142 micromoles per liter (mmol/L) at the initial check. In the next few days of the critical care admission, she had progressive hyperammonemia to 432 mmol/L, despite treatment with lactulose and rifaximin. Ultrasound exams of the liver done during two different stages of the admission revealed no nodularity or fibrosis.\nContinuous Renal Replacement Therapy (CRRT) was initiated despite normal renal function with the express intent to clear the ammonia and observe for improvement in encephalopathy while awaiting the results of extensive laboratory testing including serum levels of amion acids, essential minerals and vitamins to reveal the etiology. MRI (Figure ) was repeated at that time showing worsening of diffusion weighted abnormalities. Laboratory investigation then revealed her to be deficient in zinc (Table ) along with many essential and nonessential amino acids. The deficiencies are highlighted in (Table ). On the third day of CRRT, supplemental nutrition was reinitiated, specifically fortifying her parenteral feeds with the essential amino acid combinations that were found deficient on testing.\nThis lead to a change in her responsiveness, first to pain, then to loud voice commands in the couple of days to follow. A week later the patient was awake with what had been a slow but progressive improvement in encephalopathy. Repeat MRI (Figure ) two weeks later showed substantial reversal of diffusion weighted changes and the patient herself was conversational and participating with physical therapy. |
A 25-year-old female patient referred to the Department of Periodontics and Oral Implantology at SGT Dental College, Hospital and Research Institute, Gurgaon with a chief complaint of sensitivity in her upper front teeth. On examination it was seen that the patient had generalized recession particularly in relation to the maxillary anterior teeth from the left to the right maxillary canines (). It was diagnosed to be Miller's class I recession with no interdental dental bone loss (). The clinical attachment loss was the most severe on both the canines being 4 and 5 mm on right and left maxillary canines, respectively. The soft tissue biotype was examined both visually and by seeing for transparency of a probe inserted through the gingival margin []. It was adjudged to be a thin tissue biotype.\nThorough scaling and root planning was done for the patient, and she was put on a comprehensive oral hygiene maintenance program. On the day of the surgery, lignocaine 2% was used to anaesthetize the maxillary anterior teeth and the palate bilaterally. Sparing the interdental papilla a partial thickness flap was raised from the left maxillary canine to the right maxillary canine (). Some undermining of the tissue was done distal to both maxillary canines, to allow movement of the flap and aid in placement of the subepithelial connective tissue graft.\nDue to the amount of connective tissue required to cover the defect, it was preplanned to take the graft bilaterally from the palate. Once the graft was procured, palatal sutures were placed. The connective tissue graft was then trimmed to fit the defect. Following that it was placed to cover the recession defects and sutured in place with Vicryl 4-0 sutures. A periodontal dressing was then placed over the surgical site to aid in uneventful healing.\nSutures were removed at 10 days postoperatively (), and the patient was examined every week for the first one month and every month for the following year. Almost complete root coverage has been achieved in all the teeth which appears to be stable at the end of one year (). We were also successfully able to modify the soft tissue biotype into a relatively thicker, more durable one. |
Since about a year ago visiting our hospital, A 73-year-old female patient visited our clinic with both hip pain more severe on the right side. The patient experienced pain while walking and the pain was getting more serious, she did not have a history of trauma. The patient has been taking drugs for diabetes and hypertension for the past 10 years. Approximately 8 years ago, she underwent percutaneous coronary intervention and total thyroidectomy for angina pectoris and thyroid cancer, respectively. Five years ago, the patient was also diagnosed with end stage renal disease (ESRD) and taking the required drugs. After the onset of symptoms, a simple pelvis X-ray image taken by another institute indicated osteolytic lesions on the right femoral head and neck (), computed tomographuy (CT) images of the pelvis also showed similar osteolytic lesions; both hip joints were swollen and the joint cavities were invaded (). In order to confirm the presence of lesions, non-enhanced magnetic resonance images (MRI) were taken of both hip joint and the right femoral head and neck showed osseous lesions. In both joint cavities, low signal intensity of lesion deposition were observed (). Approximately 1 year later, the patient was subjected to simple X-ray and enhanced MRI and we identified subchondral inflammation and that the femoral head and neck cysts were enlarged. Further, amount the lesion which shows low signal intensity in MRI that means deposition in cavities increased as well (, ).\nThe range of motion of the right hip joint, the flexion contracture, further flexion, internal rotation, and external rotation were measured and determined to be 10°, 90°, –10°, and 40°, respectively. In addition, adduction and abduction were 50°, and 30°, respectively. To make a more accurate diagnosis, ultrasound-guided biopsy was performed and amyloidosis was confirmed based on apple-green birefringence with Congo red stain and the presence of amyloid P positive cells when immunohistochemical analysis was conducted. When hospitalized, hematological analyses were conducted; erythrocyte sedimentation rate was 110 mm/hr and C-reactive protein was 2.95 mg/dL. In addition, it was suggested that the patient may have anemia (hemoglobin 9.5 g/dL). Despite of conservative treatment, the patient still complained of serious pain, thus total hip arthroplasty of the right hip was performed to relieve pain, prevent femoral neck fracture and the enlargement of lesions (). While performing surgery, the authors confirmed cystic formation of the femoral neck and head and massive joint effusion, synovial hypertrophy, and amyloid deposition throughout the joint muscles ().\nAccording to the biopsy, apple-green birefringence with Congo red stain and amyloid P positive cells were observed and amyloid fibrils were visible with an electron microscope (). No microbial contamination was observed with ultrasound-assisted biopsy and culture examination. To further test if amyloidosis invaded toward other tissues, esophagogastroduodenoscopy, sigmoidscopy, chest CT, and abdomen ultrasonic wave examinations were performed and the results suggested no additional amyloidosis. Serum and urinary protein immune electrophoresis were performed to elucidate the primary cause of amyloidosis; in this, lambda light chain (monoclonal gammopathy, lambda type) was found to be high and the bone marrow aspiration examination indicated increase in plasma cells (19.4%). Therefore, the patient was diagnosed with secondary multiple local amyloidosis due to multiple myeloma. The patient is now being treated for multiple myeloma and followed up. |
A 27-year-old woman presented with progressively worsening chest tightness and shortness of breath on exertion.\nSix years ago, the patient began to experience chest distress and shortness of breath after activity, which was relieved after rest. The patient had occasional tussiculation but no paroxysmal dyspnea. She had low energy and lack of appetite, but her sleep quality was fair. The patient’s growth and development were within the normal range. Over time, the symptoms of chest tightness worsened, and were accompanied by dizziness and numbness of the extremities.\nThe patient had a history of incomplete abortion one year ago and had been treated with induced abortion.\nThe patient had never smoked and had no family history of heart or lung disease. She had congenital heart disease since childhood without standard treatment. The patient stated that she had a "cold" once a month. Symptoms such as shortness of breath and weakness appeared after walking up one flight of stairs and the symptoms were relieved after rest.\nOn admission, her blood pressure was 100/64 mmHg in both arms and her heart rate was approximately 74 bpm. Physical examination revealed that her heart rate was regular, and there was a 3/6 systolic murmur on the aortic second auscultation area. She had cyanotic lips and clubbing of her fingers.\nRoutine laboratory examinations were within normal limits.\nThe patient underwent multimodal imaging. A transthoracic echocardiogram revealed that the main pulmonary artery was dilated with a diameter of 54 mm. There was an abnormal passage between the descending aorta and pulmonary artery with an inner diameter of 9 mm. The entire heart was enlarged. The continuity of the ventricular septal outflow tract was interrupted, and the defect size was approximately 14 mm. Doppler ultrasound evaluation found bidirectional reflux of abnormal channels between the descending aorta and pulmonary artery, which was thought to be a patent ductus arteriosus (PDA) in the interrupted region (Figure ). Chest radiography showed an enlarged heart shadow, a prominent pulmonary artery, and increased lung texture, which was consistent with pulmonary congestion (Figure ). Computed tomography angiography (CTA) showed enlargement of the right heart and absence of the aortic arch. The descending aorta originated from the pulmonary trunk, and the junction was 13 mm wide. The diameter of the main pulmonary artery and the right pulmonary artery were thickened. The main pulmonary artery was 54 mm wide, and the right pulmonary artery was 27 mm wide. The ventricular septal wall defect measured 17 mm at the level of the right ventricular outflow tract. The aorta was shifted forward and to the right, straddling the two ventricles (Figure ). Ven-triculography was performed via the left femoral arteries. Right ventriculography showed pulmonary artery dilatation with residual lung signs. Contrast medium was abnormally seen simultaneously in the left ventricle, ascending aorta, and descending aorta (Figure ). |
We report a case of a 75 year old woman addressed to Allergology Department of our hospital in January 2014 for recurrent episodes of angioedema since the age of 66, with progressively increased severity and frequency. It was first considered to be induced by treatment with angiotensin-converting-enzyme inhibitors (ACEI) for mild hypertension, but she continued to have angioedema attacks for the next 6 years after discontinuation of ACE, with progressive aggravation during the last year. The previous multiple evaluations by many specialists in other hospitals did not succeed to give a clear diagnosis and treatment.\nThe patient had no relevant medical history and took no medication, except ACEI that was stopped some months after angioedema onset. No relation with possible allergic stimuli could be identified and she had no clinical manifestations between attacks. Angioedema was painful, not accompanied by urticaria or abdominal symptoms, located variably to neck, arms or buttocks, without facial involvement during 6 years. The attacks occurred at weeks or months intervals and usually lasted between 48 and 72 h, irrespective of corticosteroids and antihistamines treatment usually administered. The frequency of attacks had progressively increased from one at 2–3 months intervals to almost weekly during the last year before presentation. The last angioedema attack, determining emergency hospitalization in September 2014, was more severe and prolonged, accompanied for the first time by laryngeal edema and respiratory symptoms. The extended medical evaluation, including complete blood tests for inflammation, allergy, autoimmunity and cancer, were all negative (Table ). Full body CT scan and bone marrow examination were normal. No criteria for lymphoproliferative, mieloproliferative or autoimmune diseases could be found. Measurement of C1 inhibitor (C1INH) in plasma showed significantly decreased level at all measurements, with low activity ranging from 58 to 4% and constantly low C4 (Fig. ). Complement fraction C1q plasma level was measured twice and had normal value. Genetic tests were not performed, given the patient advanced age and lack of family history of angioedema, which are against HAE. A spontaneous mutation in SERPING 1 gene is noticed in up to 25% cases of HAE without family history, but we considered this probability very low in our case, due to late onset of angioedema.\nTreatment of angioedema attacks before hospitalization consisted of antihistamines and systemic corticosteroids, which proved to be ineffective. Since no pathogenic therapy with C1INH concentrate, antagonists of bradikinin receptors (icatibant) or selective inhibitor of plasma kallikrein (ecallantide) was available in 2014, we first initiated daily prophylactic treatment with tranexamic acid for 3 months, with no amelioration. During the more severe attack with laryngeal edema, in September 2014, we have switched to attenuated androgen danazol, given 400 mg the initial dose, reduced to 200 mg daily after 1 week and then to 100 mg daily. The clinical evolution was very good, no angioedema attack occurred since the introduction of danazol. C1INH and C4 plasma levels increased after 2 weeks of treatment and became normal after 1 month. After some months, the patient decided herself to discontinue danazol for short time, in order to check effects and to taper the minimal dose. Serum C1-INH and C4 plasma levels were measured after 2 and 4 weeks and showed significant lower levels, but no angioedema attack occurred during this period of time. She therefore restarted danazol prophylactic therapy at a minimum of 50 mg dose daily, ongoing after 3 years. The patient was closely monitored during the next 3 years, with complete clinical and laboratory control twice a year. We took into consideration the possible side effects of danazol, mainly dislipidemia, haematological and liver malignancies and any other complications or concomitant diseases. The clinical evaluation was very good, except two episodes of pulmonary cysts infection, remitted with broad spectrum antibiotherapy, which could not be related to danazol treatment. The clinical outcome of angioedema after 3 years of danazol treatment is very good, with no attacks or other related symptoms. No clinical or laboratory sign of any disease that could induce C1 INH deficiency occurred. No relevant side effects of danazol were noticed. The patient has an improved quality of life due to therapeutic compliance and general management plan. |
The patient is a 21-year-old male who presented to our institution after being involved in a high-speed motor vehicle collision. He was the restrained driver, and his car rolled over and collided with a tree at highway speeds. The patient presented with isolated bilateral knee dislocations in a windswept pattern. Trauma workup performed at arrival revealed no other injuries. Within 90 minutes of injury, the patient underwent closed reduction of both knees with application of knee immobilizers. Given the obvious deformities of both knees, the bilateral knee dislocations were reduced prior to obtaining imaging, and X-rays were acquired postreduction. CT scans were also obtained to gain a better understanding of the bony involvement (Figures , , and ). Postreduction evaluation revealed no vascular or neurologic injuries with soft compartments bilaterally. Ankle-brachial index (ABI) measurements evaluated pre- and postreduction were >0.9 bilaterally and were repeated for 48 hours to ensure no vascular changes presented in a delayed fashion. Due to gross instability of the knees following reduction, the decision was made to apply external fixators (Figures and ).\nAfter initial stabilization, the decision was made to obtain an MRI to further evaluate the extent of soft tissue injuries. Even though the external fixator components were compatible with MRI, the patient complained of burning symptoms at the pin sites during initial attempt at obtaining the MRI. The decision was made to remove the external fixation devices, and the patient was subsequently placed in cylindrical casts to obtain MRI images. MRI was obtained and confirmed the injury patterns that were classified according to the anatomic classification of knee dislocation as a KD-V on the right and KD-IV on the left, respectively [] ( and Figures and ). The decision was made to proceed with bilateral knee multiligament reconstruction.\nThe procedures were performed at one and three weeks from presentation, with the left knee being reconstructed first. A combined arthroscopic and open approach was utilized for both multiligament knee reconstructions ( and Figures and ). Perioperatively, the patient received antibiotic prophylaxis with three doses of 2 g of Ancef and DVT prophylaxis with 30 mg of enoxaparin sodium administered every 12 hours for 6 weeks. Upon completion of each case, the operated knee was stable in both coronal and sagittal planes. Following each procedure, the respective knee was immobilized in a hinged knee brace in extension, and physical therapy was started at postoperative day one. The patient was briefly in an inpatient rehabilitation facility but opted to be discharged and received in-home therapy 1-2 times per week. The rehab protocol emphasized early prone range of motion from 0 to 90° for 2 weeks. The patient remained in a knee immobilizer for 2 weeks, was non-weight-bearing for 10 weeks, and then transitioned to weight-bearing as tolerated. Given the bilateral nature of the injuries, the patient remained non-weight-bearing for an extended period of time (10 weeks as opposed to 6 weeks), as he did not have a functioning contralateral leg to allow for full weight-bearing support.\nThe patient presented for initial outpatient follow-up four weeks following the injury. At this time, all wounds had healed appropriately with no erythema, drainage, or excessive swelling. The patient remained non-weight-bearing at this time and was utilizing a wheelchair. His pain was controlled with naproxen, acetaminophen, and Percocet 2-3 times per week. On exam, both knees were grossly stable; however, there was significant stiffness bilaterally. The right knee had passive range of motion (PROM) from 0 to 20°, and the left knee from 0 to 50°. The Knee Society Scores were 45 and 39 for the left and right knees, respectively []. The importance of physical therapy was emphasized to the patient at each follow-up visit, and he was made aware that an aggressive therapy regimen would be required in order to regain full motion of the knees. However, due to a multitude of factors, including the ongoing COVID-19 pandemic, the patient failed to progress through his rehabilitation protocol over the subsequent weeks.\nBased on his inability to regain full range of motion with physical therapy, the patient required manipulation under anesthesia (MUA) of bilateral knees to improve range of motion. MUA was performed twice on the left side (week 2 at time of right-sided surgery and week 12 after reconstruction) and once on the right side (week 10 after reconstruction). Manipulation under anesthesia was implemented rather than lysis of adhesions, as it was felt to be a less invasive procedure that would convey similar benefits for the patient.\nThe patient continued in-home therapy. At the last MUA procedure, both knees were ultimately able to obtain full flexion and extension under anesthesia. Both knees remained stable after the procedure. The patient continued with physical therapy focusing on ROM, gait mechanics, and strengthening. He required oxycodone on physical therapy days; otherwise, his pain was controlled with acetaminophen and naproxen. At four-month follow-up, the left knee had active range of motion (AROM) from 0 to 130° and PROM from 0 to 135°. The right knee had AROM from 5 to 80° and PROM from 0 to 90°. At six-month follow-up, the left knee exhibited full AROM from 0 to 135°, and the right knee had advanced to an AROM of 0-115° and PROM of 0-125°.\nAt nine months from initial injury, the patient was able to walk unassisted without difficulty.\nHe was continuing physical therapy for range of motion and strengthening. A 5° flexion contracture of the right knee persisted. He had not yet begun running or returned to sport.\nAt one-year follow-up, the Knee Society Scores were 90 and 89 for the left and right knees, which improved from 45 and 39, respectively []. The left knee demonstrated a full range of motion, while the right lacked the last 5° of flexion. All ligamentous repairs remained intact (Figures and ). The patient was able to walk for an unlimited distance and navigate stairs without the use of assistive devices. He had returned to the gym for exercise and resumed his regular activities and hobbies. He reported occasional anterior knee pain brought on by sitting in deep knee flexion for extended periods of time, which was relieved with ibuprofen. Overall, the patient was satisfied with his outcome at one-year follow-up. |
A 76-year-old female patient presented to the Department of Oral and Maxillofacial Surgery with the chief complaint of swelling in the left palatal region for the past 2 months. The patient had a history of loss of the upper left second molar tooth three years ago when the patient herself plucked it out due to pain and presumably damaging the alveolar socket in the process. Further, the patient had a history of extraction of the first molar tooth 5 months back elsewhere, supposedly a traumatic extraction of extended duration, following which the patient has been informed of possible involvement of the sinus floor after the procedure by the previous dental practitioner. The patient's past medical history revealed to be uncontrolled diabetic, hypertensive and under anticoagulants. There was no previous history of trauma, previous surgeries, or maxillary sinusitis.\nOn extraoral examination, mild swelling was evident in the left upper cheek region and the patient also had paresthesia on the left infraorbital region and left nose blockage for a duration of one month. On intraoral examination, palatal swelling of 2.5 cm × 3 cm was confined to premolar and molar region, not extending across the midline with obliteration of the left buccal vestibule and expansion of the alveolar segment over the molar region []. Skin overlying the swelling was of normal colour, and the swelling was slightly tender on palpation but not pulsating. The premolar teeth were not tender to percussion. Periodontal pocketing was absent and surrounding gingiva was of normal color and appearance. All teeth were vital. No intraoral or extraoral sinus openings were identified.\nPreoperative computed tomographic (CT) scan showed an extensive radiolucent unilocular cystic lesion within the left maxillary sinus measuring 35 x 31 x 34 mm, which obliterated the left osteomeatal complex superiorly till the left infraorbital foramen. Resorption of both anterior and posterior wall of the maxillary sinus was evident []. Expansion of palatal and buccal cortex is noted. Straw-colored fluid was aspirated from the lesion and the size of the lesion was reduced. An incisional biopsy was performed through the buccal vestibule, and the result suggested the presence of unspecified odontogenic cyst. Provisional diagnosis was made as an odontogenic keratocyst, and the decision was made based on the above findings to perform enucleation of the cyst under general anesthesia. On preoperative investigations, electrocardiogram reports of the patient showed abnormalities, and the patient was declared unfit for surgery under general anesthesia. Over the next 1½ months, her blood glucose and blood pressure were brought under control and the cardiologist opinion and fitness were obtained to perform surgery under general anesthesia. The size of the cyst remained the same.\nAs planned previously, enucleation of the cyst in toto with the sinus lining was done under general anesthesia. The fluid from the cyst was dark, dirty colored, and the specimen was sent for histopathological examination [Figures and ]. The bony cavity was packed with Bactigras to prevent hematoma formation or secondary infection. The wound was then closed primarily with a small opening left for removal of gauze after a week. Healing was uneventful.\nExcisional biopsy reported a cyst lined by thin epithelium composed mainly of psuedostratified ciliated columnar type, underlying dense connective tissue showed areas of hyalinization and entrapped squamous cells and few areas of chronic inflammatory cells which confirmed the diagnosis of surgical ciliated cyst of the left maxillary sinus [Figures and ]. The patient was reviewed on a regular basis. There was no abnormality detected both clinically and radiographically during the review visit after 6 months. |
A 48-year-old healthy male presented with complains of mild but persistent cough for about 6 months. The cough was dry, not related to position, and not associated with chest pain or hemoptysis. The cough had episodes of exacerbation that subsided after some time without treatment. He gave no history of fever or any other significant symptom. He was on cough syrups prescribed by his local doctor. He suffered from no other significant systemic or mental illnesses. He was a well-educated man with average intelligence. The patient gave a history of surgical hernia repair gone under general anesthesia about 6 months earlier.\nThe patient came to us with a chest radiograph () taken about a month prior, which showed a radiopaque shadow of 2 rings joined by a wire-like structure in the left main bronchus close to the carina. This was initially thought to be an artefact by a local practitioner he was visiting. After about 2 weeks, he was advised to repeat the radiograph () by another physician. The second radiograph showed a radiopaque shadow in his right main bronchus. This shadow was identical to that in the previous radiograph except for its location and orientation, and was diagnosed as a bronchial foreign body. The patient was then referred to us.\nWe were unsure as to the nature of the foreign body. The patient could not think of anything he had aspirated that resembled the shadows. He did not have a history of missing dentures. It was decided that flexible fiberoptic bronchoscopy would be performed to ascertain the nature of the foreign body and if safely possible, to remove it. The plan was explained, written consent taken, and awake fiberoptic examination was performed. The patient received nebulized 4% lignocaine and mouth gargles. Glycopyrrolate was given intramuscular 20 minutes before the procedure. A trained expert performed the bronchoscopy. On fiberoptic view, the foreign body was found to be a portion of denture that was longitudinally wedged just below the carina in the right main bronchus. Using a grasping claw, the foreign body was carefully secured and gently pulled up under vision. Continuous oxygen flow at 15 L/min was given through a nasal prong. There were no episodes of desaturation. The patient was cooperative and the denture was successfully extracted without much difficulty (). |
A 32 year old male in evidence with a diagnosis of tuberculosis 2 years prior was admitted to the pneumology clinic with repetitive left basal pneumonic processes. The most important symptoms that made the patient rush to the hospital were: irritative cough with medium quantity of muco-purulent expectoration, subfebrility and small haemoptysis. Standard thoracic radiological exam showed an enlarged left pulmonary hilum and sequelas of fibro-nodular lesions in the upper lobe of the same side. The bronchoscopic exam revealed a 5mm stenosis in diameter beginning with the 4th ring of the left main bronchus that did not allow the passage of the bronchoscope (). The surrounding bronchial mucosa was congestive and a purulent secretion was draining out of the stenotic lumen. Bacteriology exam certified the absence of Koch Bacillus. CT exam showed the quasi normal aspect of the left distal main bronchus and certified the presence of fibrous sequelas in both upper lobes. We proposed and performed a left main bronchial sleeve resection of about 2.5cm followed by a favorable postoperative evolution (). On the 7th day after surgery, the bronchoscopic exam revealed the integrity of the anastomosis (). The patient returned to the pneumology clinic to continue the tuberculostatic treatment.\nIn all four cases, the diagnosis was established by the bronchoscopic examination which allowed the description and biopsy of lesions. In 3 cases the CT examination correctly evaluated the status of pulmonary parenchyma as well as the status of local regional lymph nodes. The surgical approach was lateral thoracotomy in the 5th intercostal space, an approach that offers good and sufficient light for the safe dissection of the hilum. In the first case, the azygos vein was divided due to the adherences between the vein and the tracheobronchial angle following the previous procedure. On the left side, the arterial ligament (ductum arteriosum Botallo) was divided to enlarge the space under the aortic arch for the resection and the anastomosis. The anastomosis was performed using absorbable 3.0 threads (polidioxanone) for the anterior and posterior aspects of the anastomosis. The suture was covered with mediastinal pleura in the 1st and 2nd case (on the right side) and with pediculated pericardial fat for the 4th case (on the left side). The main bronchial sleeve resections were between 1cm (for typical carcinoid) and 3cm (for metachronous squamous cell carcinoma). A radiologic examination was performed daily and a bronchoscopic exam in the 7th and the 30th day after the procedure. |
The patient was a 65-year-old woman without notable antecedents presented to our institution for progressive left hip pain for approximately 8 months. It was a mechanical pain of the hip well relieved by the usual analgesics. The appearance of walking distance and the poor response to analgesics forced her to consult in our center.\nThe BMI was 35,5. The walk was almost normal. There was no cutaneous scar on the lateral side of the left hip or on the ipsilateral buttock. There was a good trophicity of the abductors. Lateral rotation and abduction were markedly diminished. The rest of the exam was strictly normal. The pelvis AP () and lateral () left hip radiographs revealed signs of hip osteoarthritis. We concluded that it was a symptomatic left hip osteoarthritis that was more and more disabling in an obese woman of 71 years with no particular history. We indicated THA by posterolateral approach.\nIn the operating room, after the skin incision and subcutaneous haemostasis, we discovered in the adipose tissue about 5 cm thick a kind of well-circumscribed shell of about 2.5 cm of axis. Her incision gave rise to a whitish, thick color, looks a little oily collection (), resembling a purulent collection (). A sample for bacteriological investigation in a lab was carried out. The hull with its clear boundaries within the gluteal fat was resected and entrusted to the pathologist. All the neighborhood tissues were healthy (very localized lesion).\nIn front of this collection which appeared to be purulent, we limited ourselves to the resection of this hull, the cleaning of the wound, and the deferred implantation of the prosthesis.\nCytobacteriological examination of the specimen revealed its greasy appearance, epithelial and lymphocytic cells; there were no visible germs. Histological examination of the resected shell revealed a fibrous wall with chronic inflammatory remodeling made of lymphocytes and plasma cells with no necrosis centers.\nIn the light of these laboratory results, we conducted the interview of the patient, who reported a notion of malaria for about two months to the screen treated with an intramuscular injection on the right buttock of the compounds derived from artemisinin. We found the result of the thick drop before the injection which was positive and that of the injection which had not been negated; the patient was then successfully treated orally. The sample was sent to a lab for confirmation by artemether identification by thin layer chromatography (TLC).\nA sample of 40 g of human fat was treated with ethyl acetate (50 ml × 3) after filtration on Whatman paper, the solvent was evaporated, and the residue was taken up with acetone (40 ml) constituting the sample to be analyzed. Artemether was purchased from a local pharmaceutical company.\nImplementation of the TLC: solution to be analyzed: 20 μl of sample; control: artemether (80 mg/mL), 10 μl deposit; support: silica gel GF254; mobile phase (10 ml): dichloromethane, ethyl acetate (7/3); and developer: 25 ml anisaldehyde reagent, 5 ml concentrated acetic acid, 450 ml ethanol, and 25 ml concentrated sulfuric acid. Using a capillary tube, 20 μl of the sample was deposited on the plate (silica gel GF254), the control 10 μl. The plate is placed in a tank previously saturated with the migration or elution solvent (mobile phase) which covers the bottom of the tank at 5 mm height. The migration of eluting solvent causes the substances contained in the samples at various speeds; spots are formed characterizing the substances present in the sample.\nThe plate was removed from the tank as soon as the solvent front reached about 9 cm. The plate was dried and observed under a UV lamp at 254 nm and then revealed with the developer which will characterize the artemether in human fat.\nThe plate then shows an orange spot on the left side of the sample and a spot with the same color on the right side of the control; the two spots have the same front report as shown in . This indicates that there was artemether in this human fat sample. |
A 14-year-old female patient reported to the Department of Prosthodontics with the chief complaint of pain and sensitivity in multiple teeth and aesthetics. Patient also gave a history of repeated vomiting and inability to swallow both solid and liquid foods.\nOn general examination, there was stunted growth and reduced vertical height of the face; on intraoral examination, multiple erosive lesions and multiple pulpally involved teeth with extensive destruction of enamel and dentin were present.\nPatient was referred the Department of Gastroenterology, and routine blood investigations were done which were found to be normal. Thyroid profile showed increased T4 levels. Endoscopy revealed dilated esophagus with no peristalsis. Based on these investigations, a diagnosis of achalasia cardia was made. After balloon dilatation, the patient was relieved of symptoms.\nIn order to preserve the remaining tooth structure, we planned to go for overdenture. Intraoral periapical radiographs and OPG were taken []. Primary impressions were made with irreversible hydrocolloid material, i.e., Alginate impression material and poured cast with Type III dental stone []. Extraction of 15 14 11 21 24 25 26 36 45 46 was done under antibiotic coverage as the teeth eroded to the level of crest of the ridge and were not suitable for using it as abutments to avoid secondary infections. Root canal treatment was done in relation to 16 13 12 22 23 35 34 33 32 31 41 42 43 44 47 for pulpally involved teeth [] and then tooth preparation was done in relation to 13 12 22 23 35 34 33 32 31 41 42 43 44. Final impression was made with polyvinyl siloxane light body consistency []. Then, metal copings were fabricated on working cast by using Co-Cr metal alloy. These copings were permanently cemented with glass ionomer cement on 13 12 22 23 in the maxillary arch and 35 34 33 32 31 41 42 43 44 in the mandibular arch []. On that conventional overlay denture was fabricated []. Then, the denture was relined at regular intervals with a soft liner []. |
A 51-year-old woman presented with left upper abdominal pain that had persisted for the past 6 months. The results of a physical examination were unremarkable. The results of a complete blood cell count, electrolyte count, eosinophil count, serum biochemistry, and urinalysis were within normal limits. The chest x-ray was normal and ultrasound examination revealed a heterogeneous cystic mass between the upper pole of the left kidney and the spleen. Computed tomography (CT) scan demonstrated the presence of a solitary cystic mass within the left adrenal gland with no enhancement suggestive of adrenal echinococcosis. No other intra-abdominal or intrathoracic masses were found (). Urinary catecholamines and metanephrine levels were within normal limits. The serology for E. granulosus was positive. On further enquiry, the patient confirmed a history of animal contact. On the basis of these findings, the patient was started on albendazole for 4 weeks and a plan for surgical excision was formulated.\nThe diagnosis was confirmed during the surgery. The cyst was approached laparoscopically by the transperitoneal route. The rest of the peritoneal cavity did not reveal any other lesions. The area around the cyst was carefully packed with gauzes soaked in betadine solution. The cyst was aspirated during the procedure and 10% betadine solution was filled inside the cyst and maintained for 10 minutes. An appropriate dissection plane between the cyst and the adrenal gland could not be found. Hence, a 10-mm trocar was introduced inside the cyst and the cyst contents were sucked out, including the germinal layer (). Partial excision of the cyst wall was done because the cyst was adherent to the renal vessels. Pathological examination of the specimen confirmed the diagnosis. The wall of the adrenal gland showed cells in clusters and also singly with necrosis in the background. The hooklet of the parasite was also demonstrated as an acid-fast structure on the pathology slide (). The patient's postoperative course was uneventful. At the 6-month follow-up, the patient was free of recurrence. |
A 76-year-old man with a past medical history of hypertension, coronary artery disease (focal stenosis of the first obtuse marginal branch, which was medically treated), and non-tuberculous mycobacterial left lung infection complicated by chronic severe left bronchiectasis necessitating a left pneumonectomy was referred to our institution for the management of symptomatic persistent AF. He was first diagnosed with persistent AF about a year prior and required cardioversion to normal sinus rhythm. He remained in normal sinus rhythm for about six months, followed by recurrence of AF. At that time, he was started on sotalol therapy, which was discontinued due to excessive fatigue. He was not a candidate for amiodarone or other anti-arrhythmic drugs due to his chronic non-tubercular mycobacterial lung infection and potential drug interactions, notably QT prolongation, with his chronic antibiotic therapy. Therefore, the decision was made to proceed with AF ablation.\nAs part of routine pre-procedural planning, the patient underwent contrast computed tomography (CT) imaging of the heart, which excluded an intracardiac thrombus ( and demonstrated a left mediastinal shift due to his pneumonectomy (. Intracardiac echocardiography (ICE) was also performed to help define the anatomy of the interatrial septum and guide transseptal puncture. Transseptal puncture was more challenging in this patient due to the rotation of the heart, but imaging helped to overcome this challenge. Pre-interventional imaging also helped define the PV anatomy. The patient had a remnant left common PV stump as a result of his history of left pneumonectomy and two widely patent right-sided PVs. The length of the left common PV stump was noted to be around 30 mm. The size of the veins is given in . Anchoring the CBA catheter in the PV stump can be difficult if the length of the pulmonary stump is short. After carefully reviewing the anatomy, it was concluded that the length of the left common PV remnant was sufficient to attempt CBA.\nFor the CBA, the patient was placed under general anesthesia and an esophageal temperature probe was inserted for close monitoring. We first performed voltage mapping of the left atrium using the PentaRay® mapping catheter (Biosense Webster, Diamond Bar, CA, USA) and the CARTO® electroanatomic mapping system (Boston Scientific, Natick, MA, USA). The latter demonstrated electrical activity in the left common PV stump and the two right-sided PVs (. We then advanced the Achieve catheter (Medtronic, Minneapolis, MN, USA) to the left common PV stump. Thereafter, we were able to successfully occlude the left common PV stump using a 28-mm Arctic Front™/Achieve cryoballoon catheter system (Medtronic) and achieve electrical isolation. Then, we successfully performed CBA of the right-sided veins while monitoring the phrenic nerve function. Each of the PVs was frozen once if isolation was seen by 30 seconds, and, if not seen, the veins were frozen twice. Local temperature, esophageal temperature, time to −30°, and thawing were monitored during each isolation procedure. Post-CBA, voltage mapping of the left atrium demonstrated isolation of all three PVs (. He was in normal sinus rhythm after the procedure and was discharged home. Unfortunately, at the one-month follow-up visit, he was found to have recurrence of AF. The patient was unable to tolerate atrioventricular (AV) nodal blocking agents due to hypotension, and anti-arrhythmic drugs were contraindicated due to harmful interactions with chronic antibiotic therapy for non-tubercular mycobacterial infection. After patient-centered discussion, he was treated with an AV node ablation and permanent pacemaker implantation. |
A 79-year-old female patient with dyspnea underwent computed tomography (CT) angiography to rule out pulmonary embolism in October 2017. There was only arterial hypertension, bilateral hip arthroplasty, and laparoscopically assisted cholecystectomy in the patient’s history. A mass on the right adrenal gland was incidentally detected on the CT scan. With this finding, the patient was referred to a urological department in a tertiary medical center. A lesion of the right adrenal gland was confirmed on the subsequent abdominal CT, and in addition, another incidental lesion was found on the right kidney. A tumor on the lower pole of the right kidney measured 45 × 38 × 34 mm, with significant heterogeneous enhancement; it was interpreted as a suspected renal cell carcinoma (). The mass on the right adrenal gland had dimensions of 41 × 38 × 24 mm (); its attenuation was 41 Hounsfield units (HU) in the unenhanced phase, 71 HU in the venous phase, and 64 HU in the late phase; the absolute percentage rate was 23.3% and the relative percentage rate was 9.9%. Neither the unenhanced attenuation nor the values of the wash-out rate indicated a typical adenoma; therefore, the lesion had to be classified as indeterminate. Endocrinological examination did not reveal increased hormonal activity. With regard to the finding of a tumor of the right kidney, metastasis seemed to be the most likely. Unfortunately, about 20% of patients with a malignant renal tumor are diagnosed in the metastatic stage []. A suspected malignant renal tumor should be removed with clear surgical margins [,]. In the case of cT1 renal neoplasm, partial nephrectomy or enucleation is recommended [,] in order to preserve renal function [] for eventual adjuvant therapy. Therefore, a combined surgical procedure was indicated to remove both the renal and adrenal mass in December 2017. First, a robot-assisted partial nephrectomy was performed on the right kidney using a 5-port transperitoneal approach; the warm ischemia time was 19 min. A right-sided robot-assisted adrenalectomy followed directly. The patient was lying in the left lateral decubitus position, the overall operating time was 95 min and blood loss was minimal. The postoperative course was uneventful and the patient was discharged on the fifth postoperative day.\nHistological examination of the right kidney tissue confirmed a clear cell renal carcinoma pT1b, N0, M0, grade I–II (). The removed adrenal gland was without any metastatic involvement, but the cortical layer of its medial limb was fused with a nodule surprisingly formed by the splenic parenchyma with a large proportion of red pulp with the presence of extramedullary hematopoiesis (). In the case of hematopoietic elements included inside an adrenal tumor, it is necessary to consider the differential diagnostic possibility of an adrenal myelolipoma, which is a benign tumor-like lesion composed of mature adipose tissue admixed with hematopoietic elements in various proportions []. In our case, the sample contained splenic white pulp; on the other hand, it did not contain a fat component, and therefore, it was possible to rule out a myelolipoma.\nThe patient underwent follow-up abdominal CT in July 2018; the finding was without recurrence of the tumor. At the last follow-up clinical check in March 2020, the patient felt in good health condition.\nRegarding the management of such a case, we believe that spleno-adrenal fusion is an extremely rare anomaly and could not be predicted a priori. In similar conditions that are more common in clinical practice, such as splenosis or accessory spleens mimicking a neoplastic lesion, the suspicion may be confirmed by 99m Technetium heat-damaged red blood cell scintigraphy combined with single-photon emission CT.\nThe patient provided an informed consent to all the diagnostic and therapeutic procedures. Further, the patient provided a general informed consent to the use of the results of the examination methods for the purposes of research and publication, which is included as a part of the informed consent for hospitalization in our department. |
A 7-year-old girl was referred to the ENT clinic by neurosurgery as she had been suffering from a painless, gradually progressive proptosis of the left eye for 6 months. Her vision was normal, and there was no history of nasal obstruction, trauma, or epistaxis. General and systemic examinations were normal. Transnasal endoscopic examination revealed the mass was hard and smooth covered with normal mucosa filling the left middle meatus. There was obvious axial proptosis, but ocular movements were normal, and pupillary reaction and corneal sensation were intact. Fundus examination and routine blood serum electrolytes, chest X-ray, and urine analysis were normal.\nComputed tomography (CT) of the nose and paranasal sinuses showed a well-defined expansile lesion of the mid ethmoid and frontal sinus with extensive bone remodeling and thinning of the adjacent lamina papyracea and superior orbital roof, with internal heterogeneous soft tissue component. Intracranial extension had a distinct cleavage line formed by thin bone separating the dura from the cyst component []. The magnetic resonance imaging (MRI) showed concentric soft tissue and multiple fluid levels of high signal intensity on T1 and T2 sequences with mild enhancement post contrast administration []. The carotid angiogram revealed no significant uptake [].\nThe patient underwent transnasal endoscopic approach with navigational guidance in the presence of a neurosurgeon. There was a hard and smooth mass with a bony shell covered by an intact mucosa in the left nasal cavity within the middle meatus, which pushed the middle turbinate medially and the lamina papyracea laterally.\nThe mass consisted of multiple bony shells and cysts filled with blood which was excised completely using the 30° and 45° endoscopes under navigational guidance, which assisted us in identifying and avoiding insult to vital structures such as the orbit and the skull base and also helped in confirming complete excision of the mass. The entire nasal and the intracranial component of the mass were removed endoscopically resulting in the intact thin bone separating the nasal cavity and the cranium []. The patient had an uneventful postsurgical course. The pack was removed on the first postoperative day, and she was discharged on the third postoperative day.\nHistopathological examination of the excised mass revealed numerous small ossicles or psammomatoid bodies embedded in the cellular fibrous stroma []. Large cystic spaces filled with blood and separated by fibrous septa were seen focally. These cysts were lined by fibroblasts and histiocytes []. Clusters of osteoclast-like multinucleated giant cells with loose spindly to cellular stroma and reactive woven bone were also noted. No malignant osteoid or atypia were identified. These findings are consistent with POF with underlying ABC. |
Here we report a case study on a 72-year-old male patient who was consulted for left shoulder pain and weakness upon hospitalization to the Rehabilitation Department of Cishan Hospital. The patient provided informed consent for the publication of this report. We presented a detailed analysis of the SZP disease course and review of the literature based on the CARE guidelines. The patient complained of burning-like pain from his left shoulder to left forearm, followed by a vesicular eruption at the same location 10 days prior to admission. The diagnosis of HZ was made by a neurologist. An oral antiviral therapy with acyclovir was administered. Approximately 1 week later, the pain in the left elbow and forearm subsided. However, the patient was unable to elevate his left arm to the shoulder level, and the pain over the left shoulder persisted. Therefore, he visited our neurologic outpatient department where prednisolone (40 mg) was administered 2 times per day. The patient was admitted for further evaluation and management. His medical history included hypertension, coronary artery disease, and benign prostate hyperplasia. There was no history of trauma related to the left shoulder joint, or any systemic illnesses, including diabetes mellitus, cancer, or immunological disorder.\nPhysical examination of the patient revealed hyperpigmented macular lesions from the left shoulder to left forearm (Fig. ) and most of the skin lesions were found on the left shoulder. Cervical spine motions were slightly painful with minimal limitations pertaining to lateral flexion. No radicular pain was provoked by the Spurling test. Left shoulder movements were painful and limited. The passive range of motion (ROM) of the left shoulder could attain the full range but in the presence of pain, whereas the active ROM of the left shoulder was severely limited according to the following conditions: flexion: 30°, extension: 30°, abduction: 15°; external rotation: 5°, internal rotation: 35°, and full adduction. Limited active flexion and abduction were partially compensated by scapulothoracic motion. The sulcus sign was negative in the left shoulder. There was no marked atrophy or fasciculation in the left shoulder muscles. ROM of the left elbow, wrist, and finger were normal and free of pain.\nNeurological examination revealed that the left biceps and brachioradialis reflexes were hypoactive and the triceps reflex was bilaterally normal. Marked weaknesses were found in the left deltoid, supraspinatus, subscapularis, and infraspinatus muscles [manual muscle testing (MMT) 2/5], whereas slight loss of muscle strength (MMT 4/5) was documented in the left biceps brachii muscles (Fig. ). The muscle strengths of the left triceps, wrist extensors and flexors, and finger extensors and flexors were not compromised (MMT 5/5). In addition, hypoesthesia was found in all dermatomes in the range of C5 to C8.\nLaboratory investigations revealed normal hemogram, erythrocyte sedimentation rate, C-reactive protein, and biochemical tests. Lumbar puncture was performed, and the cerebrospinal fluid was clear without any white blood cells or pleocytosis. Protein and glucose in the cerebrospinal fluid were within normal limits. Brain computed tomography revealed no peculiarities other than mild cerebral atrophy. There was mild spondylosis of the cervical spine with marginal spur formation and narrowing of the C5 and C6 disk space in the cervical spine radiograph. Further radiological investigation with MRI of the cervical spine revealed mild neural foramen stenosis at C4 and C5 to C6 and C7 without the evidence of nerve root compression. Left shoulder sonography revealed only spurring of the greater tuberosity without evidence of rotator cuff tendon injury. As the weakness in the left arm could not be explained using imaging studies or other investigations, an electrophysiological test was performed on the fourth day of admission.\nA bilateral nerve conduction study was routinely performed for the median and ulnar nerves.[ The brachial plexus was stimulated 2.5 cm above the clavicle at the level of the 6th cervical vertebra, at the triangular localization between the clavicle and posterolateral line of the sternocleidomastoid muscle. The motor response of the musculocutaneus nerve was recorded from the biceps muscle, and the axillary nerve response was recorded from the deltoid muscle using superficial electrodes.[ The amplitudes of the motor responses were decreased for the left median and left axillary nerves, while it was normal for the left musculocutaneus nerve compared with the nerve on the contralateral side. Sensory nerve conduction studies revealed relatively low-sensory nerve action potential amplitudes for the left median (recorded on the second digit) and left radial (record on the snuffbox) nerves with normal conduction velocities compared with the right side. No response was obtained from the lateral antebrachial cutaneous nerve. Normal motor and sensory responses were obtained from the peripheral nerves, which innervate the lower trunk (Table ). Needle EMG showed spontaneous denervation activity and few motor unit potentials in the left deltoid, supraspinatus, and infraspinatus muscles. Needle evaluation of the left biceps brachii muscle showed only a mildly reduced recruitment pattern without spontaneous denervation activity. Normal motor unit potentials and recruitment patterns were observed in the left cervical paraspinal muscles, left rhomboid, and other muscles innervated by the lower trunk of the left brachial plexus.\nThe history, physical examination findings, biochemical, imaging, and electromyographic studies led to the diagnosis of SZP. Acyclovir and prednisolone were administered to treat SZP. Meanwhile, the patient was administered analgesics and gabapentin to control his neuralgic pain. A physical therapy program for daily exercise was initiated along with ROM and strengthening exercises. The patient was instructed to perform a home exercise program before hospital discharge. He confirmed during the examination that the pain intensity decreased 2 and a half weeks after his discharge from the outpatient clinic. The examination revealed increased shoulder ROM without improvement of muscle strength. The skin lesions had regressed but were still present. Mild atrophy of the left deltoid and supraspinatus muscles was detected. Outpatient physical therapy programs with ROM, strengthening exercises, and extra electrical stimulation in the left deltoid and supraspinatus muscles for 15 minutes and low-level laser therapy over left supraclavicular region for 10 minutes were conducted 3 times a week. The muscle strengths of the left biceps brachii (MMT 4/5 to full) and left supraspinatus and infraspinatus (MMT 2/5 to 3/5) muscles showed improvement; however, there was no change in the muscle strength of the left deltoid (MMT 2/5), and no progression of the atrophy was observed in the left deltoid and supraspinatus muscles at the follow-up visit 2 months later. The patient complained of low-intensity pain, and indicated a slight improvement in his left shoulder ROM. |
A 40-year-old female presented with chronic headache with infrequent exacerbations. She presents with worsening headache for three months with associated vertigo, nausea, and vomiting not responding to analgesics or vestibular sedatives. Her symptoms initially started twelve years ago as a diffused mild headache, which persisted through the day. Gradually, the headache worsened to a severe headache episodically associated with vertigo, nausea, and vomiting. These episodes lasted for two to three days and got resolved. She was treated with flunarizine for suspected basilar migraine but did not show any response. From the last year, she had monthly exacerbations of headache associated with distressing vertigo, unsteadiness of gait, and right-sided body numbness. In between these episodes, she had a significant dull diffuse headache not responding to simple analgesia. She did not complain of fever or night sweats but had constitutional symptoms lasting for several months. All of these symptoms severely affected her daily activities and functionality.\nDuring the last 17 years, she had repeated episodes of neurological deficits. Even before the headache appeared, she has presented with visual impairment of the right eye and right lateral rectus palsy and was treated as retrobulbar neuritis with good response to methylprednisolone. One year later, she developed left-sided visual impairment, which fully responded to methylprednisolone. MRI imaging at that time revealed normal results. Few months after this event, she got admitted with right hemisensory loss with hemiplegia, and a demyelination disease or hemiplegic migraine was suspected. Second MRI was performed at this admission, and no abnormalities were detected again. Eight years ago, she had developed a left lower motor type facial nerve palsy, which was attributed to Bell's palsy. Within the last year, she was diagnosed to have depression and anxiety for which she was treated for few months. Other than the first two instances, she was not treated with steroids thereafter. She did not complain of weight loss and did not have constitutional symptoms or chest symptoms during these periods.\nOn examination, she is an averagely built female with a BMI of 23 kg/m2. She is afebrile, pale, and did not have lymphadenopathy. Her GCS was 15/15, and she was conscious and rational with normal pupillary response, visual acuity, visual field examination, and fundoscopy. There was no neck rigidity, and she had residual left lower motor VII palsy. She had an ataxic broad-based gait with unsteadiness. Upper and lower limb examination is clinically normal. Her respiratory, cardiovascular, and abdomen examinations were unremarkable.\nInvestigations revealed a hemoglobin count of 9 g/dL with normal white cells and platelets. ESR was elevated to 86 mm/1st hour. Renal- and liver-related biochemical investigations were normal with an alkaline phosphatase within the normal range. Initial MRI scans of the brain done 10 years ago did not reveal any abnormalities such as demyelination, optic nerve enhancement of focal lesions in the cerebrum, or cerebellum. CSF examination revealed an elevated protein level of 55 mg/dl with normal glucose and cells with negative oligoclonal bands or TB-PCR. Vasculitis investigations including ANA, ANCA (ELISA and Immunofluorescence), and RF were negative. Syphilis serology and HIV testing were also negative. Chest radiograph, ultrasound abdomen, and CT scan of chest and abdomen did not reveal any mediastinal lymphadenopathy or focal lesions in visceral organ or evidence of any malignancy. Serum ACE levels (19 µ/l) and ionized calcium levels were normal. We performed a new MRI scan of the brain with contrast, which revealed a diffuse and patchy meningeal thickening and enhancement mainly in the right frontoparietal and left occipital regions with a minor enhancement of bilateral optic sheaths (). Her NMO antibodies were normal, and the MRI did not show any areas of demyelination. Therefore, she underwent a dural biopsy from the thickened dura, which revealed large areas of caseous necrosis surrounded by epithelioid histiocytes and lymphoid cells with a few isolated giant cells in the adjacent vicinity (). There were no features of vasculitis or sarcoidosis. TB-PCR of tissue and acid-fast bacilli were negative. Final conclusion was necrotizing granulomatous inflammation suggestive of dural tuberculosis. This diagnosis was presumed by the presence of necrotizing granulomatous necrosis with caseation with a strongly positive Mantoux test of 25 mm (), and later was supported by a marked response to antituberculous medication.\nWe initiated her on antituberculous therapy (all four drugs for 3 months and 9 months of rifampicin and isoniazid) without streptomycin as she is already having vestibular symptoms. Steroids were added concurrently (1 mg/kg) and was continued for 6 weeks and was tailed off over a month. She experienced a marked improvement of her headache, and she could do her daily activities normally. After a year of anti-TB medication and follow-up, she did not complain of any worsening of symptoms. |
A 55-year-old, Caucasian woman was referred to our tertiary women’s heart center for persistent chest pain, palpitations, and dyspnea. Her medical history included hypertension, dyslipidemia, chronic anxiety, and bilateral non-obstructive carotid atherosclerosis. She had no prior history of diabetes mellitus, tobacco smoking, alcohol or substance abuse, or adverse pregnancy outcomes. Her family history was significant for premature coronary artery disease. Her father had a history of hypertension and had a myocardial infarction (MI) and coronary artery bypass grafting at the age of 39. Her brother had a history of coronary artery disease and also had a MI at the age of 40. Her occupational history indicated that she had been working in the field of psychology and was still an employee in the same job at the time of the hospital visit and follow-up care.\nTable summarizes the general symptoms and characteristic signs of our patient for the onset of CMD and her progression to HFpEF. She had undergone an exercise treadmill test which revealed ischemic ECG changes and dyspnea. Her initial echocardiogram demonstrated a LVEF of 67%, mild diastolic dysfunction, mild left ventricular (LV) hypertrophy, no significant valvular heart disease, and no pulmonary hypertension. Subsequent invasive left heart catheterization was performed and it showed normal epicardial coronary arteries without angiographic evidence of atherosclerotic plaque. She continued to have exertional symptoms and angina-like chest pain and was subsequently referred to our center for further evaluation of suspected INOCA. During her evaluation and treatment she continued to experience stable angina and exertional dyspnea despite initial management with atorvastatin 20 mg daily, lisinopril 20 mg daily, aspirin 81 mg daily, and sublingual nitroglycerin as needed. She had a poor clinical response to sublingual nitroglycerin. Due to her persistent symptoms and abnormal stress testing, she was referred for coronary reactivity testing (CRT) to establish the diagnosis of CMD.\nOur patient underwent invasive CRT, as previously published []. Testing demonstrated normal coronary flow reserve (CFR) in response to intra-coronary adenosine (CFR 3.1; normal ≥ 2.5), abnormal macrovascular endothelial function to intra-coronary acetylcholine (− 6% change in coronary diameter, constriction; normal, dilation), abnormal microvascular endothelial function (coronary blood flow change 48%; normal ≥ 50%), and abnormal non-endothelial function to intra-coronary nitroglycerin (coronary diameter change + 0%; normal dilation) (Table ). She also underwent cardiac magnetic resonance imaging (CMRI) with perfusion imaging at rest and with adenosine stress (140 μg/kg per minute) which showed circumferential subendocardial perfusion defect at stress, normal LV end-diastolic volume indexed to body surface area (EDVi) of 56.4 mL/m2, LV mass index 42.3 grams/m2, and no LV hypertrophy (septum 7.2 mm and lateral wall 6.0 mm). The myocardial perfusion reserve index (MPRI) was 1.8 which was considered borderline abnormal [] (Table ). There was no evidence of myocardial scar.\nThe diagnosis of CMD was established by the coronary endothelial dysfunction observed with invasive CRT, and carvedilol and eplerenone 25 mg daily were added to her regimen. She was followed regularly in clinic with good control of her blood pressure and serum lipid levels. She reported improvement of her angina and dyspnea along with reduction in the duration and frequency of these episodes.\nTen years after her initial diagnosis of CMD, our patient was hospitalized due to symptoms of dyspnea. She was found to have elevated brain natriuretic peptide (BNP) levels of 406 pg/mL and normal LVEF. She had a computed tomography (CT) angiogram of her chest to evaluate for pulmonary embolism, which was negative but revealed bilateral pulmonary edema. She was treated with intravenously administered furosemide for pulmonary edema and diagnosed as having HFpEF. Subsequently, she was discharged with instructions to increase her eplerenone.\nShe continued to experience worsening dyspnea on exertion, orthopnea, and paroxysmal nocturnal dyspnea. A repeat echocardiogram demonstrated normal LV systolic function with an LVEF of 64%, and diastolic dysfunction as evidenced by decreased lateral E′ velocity (4.2 cm/s, indicating impaired myocardial relaxation) and elevated E/E′ ratio 12.9 (suggestive of increased LV filling pressure). She underwent coronary CT angiography which showed absence of coronary atherosclerotic plaque and a coronary calcium score of 0. She was diagnosed as having HFpEF based on clinical symptoms, preserved ejection fraction of 64%, elevated BNP, and evidence of diastolic dysfunction.\nAs part of the WISE – Coronary Vascular Dysfunction (WISE-CVD) Continuation Study (NCT00832702), she underwent a repeat rest-stress CMRI to assess myocardial structure, function, perfusion, and scar, and 13C magnetic resonance (CMR) spectroscopy. Compared to her prior CMRI 7 years ago, she had an increase in LV wall thickness in both the septum and lateral wall (Table ). On CMR spectroscopy, the myocardial triglyceride content was elevated (0.83%) compared to normal control women (mean 0.43%), suggesting myocardial steatosis which is consistent with an ischemia-induced metabolic shift and HFpEF phenotype []. Adenosine stress first pass-perfusion CMRI again showed circumferential subendocardial hypoperfusion (Fig. ) and her MPRI worsened from 1.8 to 1.1, consistent with severe CMD []. There was no evidence of scar on late gadolinium enhancement imaging. |
Patient 3 is a 30-year-old male who presented to the ED from the field with extensive facial trauma secondary to a firework blast to the face. His airway was secured immediately upon arrival due to an altered mental status with a GCS of 3 and poor oxygenation. A glidescope intubation with a size 7 tube was performed, with almost immediate replacement of a size 8 tube when an air leak was found. Exchange of the endotracheal tube was guided with the help of a bougie. He was hypotensive initially and had episodes of bradycardia. The hypotension responded to fluids. He had extensive facial blast injury with a large amount of tissue loss of the eyes, forehead and nose (). Both of the eyes were clinically ruptured. The maxilla was unstable upon examination. Focused assessment with sonography for trauma was performed and no free fluid was found. Ophthalmology, neurosurgery, and plastic surgery services were consulted.\nCT head revealed diffuse cerebral edema with effacement of the 3rd and 4th ventricles, subarachnoid hemorrhage and bilateral frontal and temporal contusions. CT maxillofacial revealed multiple complex fractures as follows: frontal bone with a comminuted fracture involving the inner and outer table and displacement of both; large defect noted in the anterior and central skull base with destruction of the ethmoid complex and the sphenoid bone; left temporal bone fracture with involvement of the inner ear and mastoid sinus; bilateral orbital, pterygoid, zygomatic and maxillary sinus fractures; fracture of the hard palate with obvious diastases of the bone fragments; and a mandibular body fracture. In addition the CT of the face revealed bilateral globe ruptures with extensive edema of the ophthalmic nerves and extraocular muscles.\nThe patient was admitted to the ICU. Consultation of neurosurgery, ophthalmology and plastic surgery were obtained. Post injury the patient developed persistent high fevers and was treated with antibiotics, he developed central diabetes insipidus, and was minimally responsive with only a cough and a gag. Eventually he was able to intermittently move his upper and lower extremities with stimulus. No surgical intervention was planned for his injuries.\nOn hospital day six, the patient underwent tracheostomy, percutaneous endoscopic gastrostomy tube placement, and facial wound debridement, all of which were uneventful. On hospital day seven, the patient had an increasing pressor requirement and fevers. His responsiveness also diminished after the operating room and it was thought that he progressed to brain death. He was in the middle of a brain death workup when he went into cardiac arrest. After 3 cycles of cardiopulmonary resuscitation, the patient was pronounced dead. |
A 42-year-old female presented to the hospital with vaginal bleeding and lower abdominal pain of 3 months duration. On pelvic exam, cervix was necrotic with oozing friable tissue extending to the pelvic wall consistent with stage IIIb cervical cancer. Biopsy of the cervical mass showed small cell neuroendocrine carcinoma with positive immunohistochemical staining for chromogranin and synaptophysin ().\nStaging contrast enhanced CT scan of the chest, abdomen and pelvis showed a large heterogeneously enhancing cervical mass measuring 5.3 × 4.8 cm on axial imaging. There was a prominent right external iliac lymph node measuring 1 × 1.9 cm. Positron emission tomography (PET) scan was positive for malignancy at the cervical mass with suspicion of right external iliac node involvement with no abnormal fluorodeoxyglucose (FDG) uptake noted elsewhere.\nShe was treated with etoposide, cisplatin and radiation. Repeat CT scan after completion of chemoradiation showed significant interval reduction in size of cervical mass which now measured 3.4 × 2.7 cm. Previously identified right external iliac lymph node had resolved. On repeat whole body PET scan, there were two foci of activity in the breasts. On the left side, there was a focus of activity deep and superior to the left areola. On the right side, there was a focus of activity in the medial aspect of the right breast (). There was also evidence of multifocal metastatic disease involving the skeleton and liver.\nBiopsy of the right breast mass was done which showed small cell neuroendocrine carcinoma consistent with metastatic disease. Immunohistochemical staining for synaptophysin and chromogranin was positive (). The patient’s prior cervical biopsy was reviewed concurrently and the malignant cells present in that specimen were identical to the malignant cells seen in breast biopsy specimen. These cytomorphologic and immunohistochemical findings were felt to be consistent with metastatic small cell neuroendocrine carcinoma of the uterine cervix.\nShe received palliative radiation therapy to spine and liver. She had notable improvement in her back pain after a week of radiation therapy. Eventually, she was discharged with home hospice. |
A 28-year-old white male presented in our office with knee problems after he hit a gym wall with his right knee during sports. His major complaint was anterior knee pain and the feeling of instability. He already underwent MRI (Figure ) examination with the following report: "Complete, chronic tear of the anterior and posterior cruciate ligaments and chondropathia of the medial femoral condyle." No further abnormal findings were documented. He further was treated by diagnostic arthroscopy elsewhere with the following report: "Normal findings of the medial and lateral menisci, narrow notch with lack of an anterior cruciate ligament (ACL), insufficiency of the posterior cruciate ligament (PCL), chondropathia of the medial femoral condyle." Again no other abnormal findings were documented. The patient was now referred to our clinic for combined reconstruction of the anterior and posterior cruciate ligaments.\nOn clinical exam he had a free range of motion, no swelling and a slight valgus alignment. He had a positive posterior sag at 90° of flexion and a reduced medial step off when compared to the other side. His Lachman test was severely abnormal without a firm endpoint, pivot shift was slightly positive. His total anteroposterior laxity when measured with the Rolimeter (Aircast, Don Joy, Inc) was 6 mm and 22 mm with a resulting side difference of 16 mm. His collateral ligaments were stable. His further history revealed a status post medial growth plate closure at the medial femoral condyle at the age of 12 for a significant leg length discrepancy and a syndactylia of the second and third toe of his right foot.\nDue to the clinical, surgical and MRI findings the patient was scheduled to undergo ACL and PCL reconstruction.\nDuring exam under anesthesia the ligament findings were the same as during the clinical exam. Originally, it was planned to use ipsilateral and contralateral hamstrings as grafts. However due to abnormal appearance of the MRI (Figure ) it was decided to start with the diagnostic arthroscopy before tendon harvest on the contralateral side. At arthroscopy there was a hypoplasia of the medial trochlea, and a lateralization of the patella. The lateral compartment showed a small cartilage defect at the lateral femoral condyle. The trochleal groove revealed a bare bone region at the distal end as if it was an osteophytic bone formation in a chronic ACL case. The medial compartment was normal. The intercondylar notch was extremely narrow (Figure ). The tibia revealed a missing ACL footprint and a single bump with a complete coverage with articular cartilage (Figure ). The lateral condyle appeared to be enlarged. At the figure of 4 position a meniscofemoral ligament (MFL) could be identified connecting the posterior horn of the lateral meniscus to the medial femoral condyle. There was no room proximal to the MFL where an ACL graft would fit in. A PCL could not be identified from the view from anterior. The lateral meniscus appeared to be normal.\nDue to the findings at surgery the procedure was ended since a ligament reconstruction did not appear possible in this case. Postoperatively the patient was informed on the unexpected aplasia and notch deformity making ligament reconstruction impossible. The patient underwent further evaluation with computed tomography scans (Figure ) and three-dimensional reconstruction (Figure ) to characterize the degree of bony deformity. The images affirmed the hypoplasia of the medial trochlea and the extremely narrow intercondylar notch. Three-dimensional reconstruction visualized the single tibial spine (Figure ) as opposed to usually two tibial spines in a healthy knee joint (Figure ).\nThe patient was further treated conservatively and did well at a reduced activity level at last follow up. |
A five years and six months old African American male child presented to his pediatrician in the outpatient clinic with the solitary complaint of lower back pain. The back pain was located in the midline, in the lower lumbar region. Pain had set in two weeks prior to his clinic visit. Initially his pain was intermittent.\nIt seemed to be exacerbated when the child bended forward. The mother started noticing the child would sit with his back stiff in the chair. He would also walk slowly holding his back straight. He seemed hesitant to bend down to pick up his toys and would ask for assistance. Into the second week of illness, his back pain was more constant throughout the day. Acetaminophen and ibuprofen would relieve the pain temporarily with recurrence after. Pain was non-radiating, and intensity was described as 7/10 by the child. He did not have paresthesias.\nAt night, he started having discomfort maneuvering in bed while sleeping and would frequently wake up in pain. The child was afebrile through his course of illness. No trauma to that area of the body could be recalled by the child and parents. There were no overlying skin changes noted at home. He did not have a limp. The child did not complain of any extremity or other joint pain. He did not have any abdominal pain, history of constipation, or rash. He denied dysuria, and no hematuria was noted at home. He did not have any other accompanying symptoms. There was no history of recent travel. The parents could not recollect any known sick contacts. There was no known family history of rheumatological or autoimmune diseases.\nHe was evaluated by the pediatrician in the clinic, with physical examination being notable for decreased forward flexion and lateral rotation of back and tenderness on palpation of his lower lumbar region in the midline. He stayed afebrile. Complete blood count was within normal limits. Erythrocyte sedimentation rate (ESR) was noted to be elevated at 66 mm/hr. The child was directed to the emergency room for further evaluation. In the emergency department, physical examination was consistent with that of the pediatrician. C-reactive protein (CRP) was found to be elevated at 25.3 mg/mL. Urinalysis was unremarkable. Comprehensive metabolic profile, which includes liver and renal function tests, was within normal limits as well. Creatinine kinase was non-elevated. Blood and urine cultures were obtained.\nComputed tomography (CT) scan of his spine was performed, which showed prevertebral soft tissue thickening at L4-L5 with two well-formed defects involving the inferior and superior endplates of L4 and L5 (Figures -).\nMagnetic resonance imaging (MRI) with and without contrast was recommended by the radiologist, which was conducted after keeping the child in nil per os (NPO) status overnight for sedation needed for the MRI. MRI findings were reported as mild disc space narrowing at the L4-L5 level with thin fluid along the anterior margin of the disc, abnormal soft tissue thickening and edema anteriorly centered at the L4-L5 disc space level, and shallow disc bulging, which were consistent with osteodiscitis (Figure ).\nAn orthopedic was consulted, who agreed with conservative management with antibiotics. Intravenous clindamycin and ceftriaxone was initiated as the initial antibiotic regimen after drawing the blood culture. Antibiotic selection was based on local sensitivity patterns, and clindamycin achieves good drug concentrates in the bone. Purified protein derivative was placed during his admission, which was read as negative at 72 hours. Blood culture and urine culture did not show any growth. Per infectious disease recommendations, antibiotic was switched to a monotherapy of intravenous clindamycin, and after completion of five total days of intravenous therapy, he was switched to per oral clindamycin for three more weeks. Into the day of discharge from the hospital, the ESR had declined to 58 mm/hr and CRP had declined to 18.4 mg/mL. Pain during his course of admission was being well controlled with acetaminophen as needed, and after being discharged home he did not require medications for pain control. The child showed complete resolution of symptoms by three weeks since the onset of illness. ESR was rechecked after completion of four weeks of antibiotics, and due to continued elevation, it was checked at two-week intervals till the ESR was less than 20 mm/hr, which was the goal for discontinuation of antibiotic therapy. A repeat MRI was obtained 10 weeks from the initial MRI, which showed residual inflammation in L4-L5 with mild paraspinal enhancement. No drainable fluid collection was seen. The radiologist suggested a follow-up scan in two to three months to reevaluate for resolution. |
A 14-year-old Thai boy presented to the community hospital with a complaint of intermittent cough of two weeks duration. Four years previously, he had undergone a tracheostomy for laryngeal stenosis following prolonged intubation after a burr-hole craniotomy for subdural hematoma evacuation. A No. 5 stainless steel tracheostomy tube was put in place. The current tracheostomy tube had been used for one year.\nTwo months previously, the patient started coughing and during the daily cleaning session his parents found that the inner tube was missing. He was brought to the family physician immediately. The patient was diagnosed with acute bronchitis and a new tracheostomy tube of the same size was inserted. After discharge, the parents reported that their child still coughed off and on every week. He slept well during the night without any breathing difficulties and had no abnormal breath sounds.\nOne day prior to admission, the boy had more severe and persistent cough. He was sent to the community hospital again. The chest X-ray revealed a metallic density foreign body in his right main bronchus. Subsequently, he was referred to our university hospital for definite treatment.\nOn arrival, the patient had occasional cough with hyperpnea. His vital signs were: a body temperature of 38.0° Celsius; a pulse rate of 140 beats per minute; respiratory rate of 44 times per minute; and blood pressure of 120/80 mmHg. The chest auscultation revealed decreased breath sounds on the right side but no chest wall retraction. An X-ray of the chest was performed. Patchy infiltration of the right lower lung and a metallic foreign body in the right main bronchus were found. He was transferred to the operating room for bronchoscopic removal under general anesthesia. The foreign body (inner tube of the previous tracheostomy tube) was retrieved from the right main bronchus and removed through the tracheostomy stoma (Figure ). A fracture at the junction between the inner tube and connector was found (Figures and ). His pneumonia was treated with intravenous amoxicillin with clavulanic acid for three days before switching to oral form for 11 days. A follow-up chest X-ray showed decreased infiltration compared with the prior film. He was discharged with improvement of his symptoms. He had fully recovered at the one month follow-up and there were no signs of any late complications. |
The patient, a 66-year-old male, who had a long-standing history of gout under medications presented to the outpatient department with complaints of a mass on his left heel which became painful and ruptured 2 days earlier causing pain, swelling, and some bleeding. He had a history of painful masses over the multiple areas of hands, feet, elbows, and knees which progressed in size over the past 16 years. On presentation, the patient was not able to carry out the basic activities of daily living due to severe deformities of the hands and feet including holding a spoon or hairbrush due to tophi ().\nHis medical history revealed that the patient had initially developed non-specific pain around the right ankle 16 years ago which was mild, dull without radiation, shifting and he was able to carry out his daily activities without discomfort which were treated with medications at a local practitioner. He has had similar episodic pains over the past 16 years. In October 2012, he developed tophi over the base of the right thumb which got infected and also led to acute kidney injury which was managed with three rounds of hemodialysis along with daily dressing of the wound with betadine and normal saline for lack of availability of better options. After this episode, allopurinol was prescribed. He underwent tophi excision in the left index finger (), olecranon, and right toe in July 2014. He has not required dialysis since.\nThe patient had a history of hypertension for the past 16 years who was prescribed amlodipine. On presentation, blood pressure was within normal limits.\nHe consumed a non-vegetarian diet and reports a regular intake of alcohol. He was prescribed febuxostat to control his hyperuricemia. His father and two siblings had similar pains and swelling around the joints, for which they were taking medications.\nOn physical examination, flexion deformities of the metacarpophalangeal joints (), and proximal and distal interphalangeal joints of all fingers and toes were noted along with tophi. Active and passive range of motion was painful and restricted in hands and toes. Local temperature was not raised. Ulcerated tophi seen on the heel of the left foot. Tophi also noted on the helix of both ears (), elbows, and heel.\nLaboratory work-up revealed a total leukocyte count of 6400/mm3, serum uric acid of 10.4mg/dL, urea 56mg/dL, and creatinine of 1.7mg/dL. X-ray of hands showed evidence of asymmetrical joint narrowing and typical calcified tophi and bone erosions sclerosis and the “cookie cutter/rat bitten” appearance of bones ( and ) [].\nA diagnosis of chronic tophaceous gout with ruptured tophi was made and the patient underwent wound debridement for the heel tophus. Incision was made over the site of swelling, skin subcutaneous tissue resected, and the tophus excised and dressing done. Febuxostat was continued and dressing was done till the wound healed (). |
A 60-year-old right hand dominant male suffered a blast injury from a stick of dynamite. He was complaining of left hand, forearm, and elbow pain. Patient also endorsed significant numbness and tingling in the left hand. On physical exam, there was significant degloving of the palm and partial amputations of the second and third distal phalanges. Radial pulses were intact, with no evidence of an arterial bleed. A photograph of the hand on presentation can be seen in . Figures and are the initial radiographs of the hand and elbow, respectively, showing the extent of the injury.\nThe patient went for emergent washout and exploration by hand surgery. He was found to have complete obliteration of the superficial and deep palmar arches. Approximately half of the flexor tendons were intact. The thumb was attached via a single flexor tendon and remnant skin. The median nerve was completely severed at the level of the wrist. Open reduction and internal fixation (ORIF) of the distal radius and the distal ulna was not performed because of a lack of viable soft tissue coverage secondary to the traumatic injury from the blast effect as determined by the hand surgeon intraoperatively. The amputation level was partially based on the level at which viable soft tissue coverage was available for coverage of bone. The hand surgeon proceeded with a transradial amputation.\nClosed reduction of the ulnohumeral joint was performed by the orthopaedic team once hand surgery was done with their part of the procedure. The patient required 90 degrees of flexion at the elbow to maintain reduction due to gross instability. 3D reconstructions from the postreduction computerized tomography (CT) scan are seen in Figures and showing the terrible triad injury of the elbow.\nTwelve days after the initial injury, the patient returned to the operating room for definitive fixation. A Kaplan approach was used, which demonstrated rupture of the lateral collateral ligament (LCL) as well as a comminuted radial head fracture. An ulnar nerve decompression was performed because of preoperative ulnar nerve symptoms. A Hotchkiss “over the top” approach was used to expose the coronoid and medial collateral ligament (MCL), which demonstrated a midsubstance disruption. MCL was reconstructed and reattached to the medial epicondyle. The small coronoid tip fragment was excised. A cemented radial head arthroplasty was performed. LCL was then repaired. The remnant radial shaft and ulnar shaft were percutaneously pinned in neutral pronosupination due to instability noted intraoperatively.\nThe patient was seen in the clinic two weeks postoperatively. Radiograph () revealed maintained reduction of the ulnohumeral joint and satisfactory alignment of the radial head replacement. The patient was noted to have a stable arc of motion between 20 and 90 degrees. Five weeks after reconstruction, the distal forearm pin was removed. The patient had a flexion arc of 5-90 degrees, but limited pronosupination. Two months postoperatively, his arc of motion was 5-115 degrees, as seen in Figures and . He was able to attain 50 degrees of pronation and 50 degrees of supination. Radiographs at that time once again show intact hardware, seen in .\nThe patient was seen again at 12 months postop for prosthetic consult. The patient was fitted for prosthetic devices including the body-controlled hook prosthesis and the myoelectric hand prosthesis. The patient chose the myoelectric device due to the cosmetic appearance and fine motor capabilities. shows the myoelectric device prior to fitting. Figures and show the patient during his prosthetic fitting. We will continue to watch his progress and long-term outcome. |
Subsets and Splits
No saved queries yet
Save your SQL queries to embed, download, and access them later. Queries will appear here once saved.