Datasets:

meowterspace42
/

clinical_notes

Dataset card Data Studio Files Files and versions Community

Split (1)

train · 30k rows

text stringlengths 746 31k
A 24-year-old right-handed Arab man, working as a computer engineer, presented with a painful mass on his right wrist that had appeared 2 years ago. The mass had slowly grown over the past 2 years and the pain had increased over time. It started affecting the range of motion of his right wrist, specifically during flexion, but without a significant effect on the range of motion of his fingers. He described experiencing a tingling sensation at the tip of the second and third fingers of his right hand. The mass started to affect his daily activity, especially at work while using a keyboard for long periods. He had no history of trauma to his right hand or of any chronic medical illnesses.\nHe initially visited a local clinic, where he was diagnosed as having a lipoma-like tumor on his right forearm. He was referred to our hospital for further evaluation. On examination, we noted an ill-defined firm fusiform mass along the volar aspect of his distal forearm, approximately 3-cm proximal to his wrist joint. The mass was approximately 2 × 3 × 3 in volume and was not pulsatile. It became more prominent during flexion of the wrist (Fig. ). Moreover, there was no focal tenderness or changes in the skin covering the mass.\nSimple radiography of his forearm performed initially revealed no bony abnormalities. Ultrasonography showed a superficial soft tissue mass at the level of the subcutaneous plane. It was isoechoic to the muscles of the forearm (Figs. and ). The mass was clearly observed in Doppler imaging and did not show any cystic changes or abnormal vascularity (Fig. ).\nMagnetic resonance imaging of his right forearm was performed. T1-weighted images and T2-weighted images in the axial, sagittal, and coronal positions revealed signal intensities of the mass similar to those of the adjacent muscles (Fig. ). Thinning of the superficial fascia and subcutaneous fat was also observed.\nThe mass was located medial to the tendon of the flexor carpi radialis and in the region of the tendon of palmaris longus. The muscle appeared tendinous in the upper (proximal) portion and muscular in its lower (distal) portion. The sagittal plane images showed the longitudinal fusiform extent of the mass, which reached the upper margin of the flexor retinaculum at its distal extent (Fig. ).\nThe mass was located close to the median nerve, as observed in the axial images. We diagnosed our patient as having reversed PLM. We detected marked hypertrophy of the belly of the muscle extending almost up to its point of attachment with the flexor retinaculum and restricted movement of its tendon over the flexor retinaculum. In addition, the median nerve near the wrist joint was being compressed. The pressure on the median nerve was the most probable cause of the wrist pain experienced by our patient.\nWe planned to treat our patient conservatively. A systemic anti-inflammatory medication and a wrist splint was the first line of treatment with regular follow-up. Furthermore, we referred him to a physiotherapy department to start stretching exercises. During his follow-up visits, he was able to tolerate pain. One year later, he eventually regained full function of his right hand, and it no longer affected his daily activities.
A 59-year-old Thai man was successfully treated with chemoradiation for stage III glottic cancer 3 years prior to presentation. Unfortunately, the tumor recurred and the patient was sent to our clinic for salvage surgery involving a total laryngectomy. Two months after this salvage surgery, the patient developed radionecrosis of the soft tissue of the neck. This necrosis resolved after hyperbaric oxygen treatment (HBOT); however, a large pharyngocutaneous fistula remained in the upper neck ().\nA free flap reconstruction was not suitable for this patient because of his surgical history and severe neck fibrosis. Thus, a two stage, medially based deltopectoral flap reconstruction was chosen. Because the distance requirement was relatively long, we opted for a surgical delay for the distal two-thirds of the flap. The flap was completely elevated and placed on the fistula 10 days after this delay.\nOn postoperative day 5, the flap developed necrosis at its distal tip and was completely separated from the fistula (). The flap tip was trimmed to ~2 cm; however, this postdebridement flap did not have a sufficient length to reach the fistula. After discussion with the patient, we decided to re-operate using this shortened flap rather than harvesting a new flap from the contralateral chest.\nThe revision operation was divided into three stages. In the first stage, we created a temporary recipient site just below and lateral to the fistula. After debridement, the distal part of the flap was placed on this temporary recipient site (). After this stage, the patient underwent another course of HBOT.\nBecause the temporary recipient site was located in a previously irradiated area, the second stage was delayed for 8 weeks, at which point the flap was divided from its origin. The proximal part, which had a reverse blood flow from the temporary recipient site, was transposed upwards to the fistula (). The patient then underwent a third course of HBOT.\nApproximately 6 weeks after the second stage of the operation, the wound was completely healed without saliva leakage (). During the third stage, excess flap tissue was divided and removed (). No further HBOT was administered. By about 1 month after the operation, the patient had resumed regular oral intake.
A 10-year-old girl presents to the emergency department after jumping on an in-ground trampoline and slipping onto the surrounding grass and twisting her right ankle. This was followed by immediate swelling over the lateral aspect of her ankle of approximately 3x4 cm. She presented immediately to the emergency department and her examination was notable for compartment syndrome. Although there was significant swelling around the ankle, she had good pulses in dorsalis pedis and posterior tibial vessels and normal sensations in her foot. A radiograph demonstrated an undisplaced fracture of medial malleolus with possible disruption of lateral ligament complex of the ankle (, ).\nShe was admitted overnight for observation, elevation, and pain relief. The following morning, the swelling was now circumferential around the ankle and the lower leg, with significant increase in pain levels and foot turned cold and purple with weak pulses. She had a delayed capillary refill time and reduced sensation in common peroneal nerve distribution over lateral aspect of foot and tense anterior and lateral compartments of the leg, these symptoms appeared approximately 20 h after the time of injury. She had no motor deficit preoperatively.\nDifferential diagnosis at this time consisted of a high-grade ankle sprain, hematoma, and compartment syndrome. A formal diagnosis of compartment syndrome was made based on high clinical suspicion; the patient had neurovascular compromise, tense compartments, and pain on passive toe stretching. The patient then underwent an urgent fasciotomy approximately 24 h after time of injury. The radiographs had not shown proximal fibular fracture but examination under anesthetic showed ankle joint to be very unstable in varus and on anterior drawer testing suggesting disruption of the syndesmosis and lateral ligament complex. Compartmental pressure measurements showed 44 mmHg in anterior and 33 mmHg in lateral compartments (measured using Stryker intracompartmental pressure monitoring system), normal compartment pressures should be between 11 and 13 mmHg. A standard open fasciotomy of the leg was performed through an anterolateral longitudinal incision extending halfway between the crest of the tibia and the fibula and anterior and lateral compartments were decompressed. Once the intermuscular septum was incised, a large hematoma revealed itself in the anterolateral ankle extending around 10 cm above the ankle and an avulsed perforating branch of peroneal artery. The hematoma was evacuated, and the perforating peroneal artery was ligated. The anterior capsule of the ankle was avulsed with the anterior tibiofibular ligament (ATFL) and the calcaneofibular ligament (CFL), was completely disrupted. Peroneal muscles were dusky in appearance but pinked on fascial release. Anterior, deep, and superficial compartment muscles were healthy ()\nPostoperatively pain improved, passive toe stretching was no longer painful and she was immobilized in a below-knee plaster cast. Forty-eight hours postoperatively, she was taken to the operating room and her lateral ligament complex, that is, ATFL and CFL was repaired. Intraoperatively, syndesmosis felt grossly unstable and a single syndesmotic screw engaging four cortices was inserted to stabilize ankle mortise. Fasciotomy wound was closed without any skin grafts. Her 6-week follow-up showed normal sensations and motor power in the common peroneal distribution and a good recovery.
A 31-year-old Malay gentleman presented to our Ophthalmology clinic with history of progressive bulging of the left eye associated with redness and pain for 2 months. He has no known medical illness although his blood pressure was reported slightly abnormal on his last visit to general practitioner. For the past five years, he has been having recurrent nasal blocked which resolved by nasal decongestion spray that he bought over the counter. He started to have recurrent headache since his last episodes of nasal congestion which was three months prior to his visit to us. In the past one month, he has reduced vision in the left eye and noticed double vision on turning to the left.\nHis visual acuity in the left eye was 6/18 and 6/9 in the right eye. Ocular examination revealed congestive nonpulsating 7 mm exophthalmos of the left eye, measured by Hertel exophthalmometer (). There was no restriction of eye movements in all direction of gazes but there was diplopia in left lateral gaze. There was no restriction of eye movements in all direction of gazes. At primary gaze intraocular pressure was 21 mmHg in the right and 29 mmHg in left eye, with no difference in other gaze. Fundoscopy of the left eye showed slightly dilated and tortuous retinal vessels (). The right fundus was normal. There was no optic disc swelling on either eye. OCT pupillometry was normal. Colour vision and visual field on each eye were normal. Systemic examinations were normal except a borderline blood pressure. There was no sign of thyroid disease. Cranial nerves were intact. He is moderately overweight but there was no sign of increased intracranial pressure.\nCT scan was performed and showed right temporal arteriovenous malformation with right middle cerebral artery as feeding artery. The left superior ophthalmic vein was tortuous and dilated measuring 0.9 mm in diameter. The left cavernous sinus was enlarged (). Extraocular muscles of left eye were relatively larger than the right eye. MRA and carotid four vessels angiogram confirmed right temporal arteriovenous malformation with a nidus of 3.8 cm × 2.5 cm (). Arterial feeder is middle cerebral artery with no supply from external carotid artery. There was no aneurysm and no carotid-cavernous fistula. The right temporal AVM is grade 4 based on Spetzler Martin grading system. There was severe venous hypertension with most of the intracranial drainage being via left cavernous sinus. Venous drainage of the AVM is via the dilated and tortuous right temporal cortical vein into the right transverse sinus. There was a tight stenosis at the right sigmoid sinus. Hence from right transverse sinus, there was retrograde flow into superior sagital sinus and straight sinus that reach the left cavernous sinus and finally into the left superior orbital vein and left petrosal sinus. Left sigmoid sinus is occluded.\nHis proptosis dramatically improved following successful embolisation of the arteriovenous malformation. Follow-up after one year showed that the uncorrected visual acuity of 6/9 improved to 6/6 after refraction. Following cessation of antiglaucoma eye drops, his IOP remains between 14 mmHg and 16 mmHg on several visits. The proptosis has completely resolved.
Our patient is a morbidly obese (375 lbs) 58-year-old woman who presented to the head and neck surgery clinic with a right parotid mass and intact facial nerve function. She underwent a fine needle aspiration which was consistent with a Warthin's tumor. CT imaging demonstrated a 5.8 cm right parotid mass. She was scheduled for surgery, but was lost to followup. She presented again to the head and neck surgery clinic one year later with significant interval growth of the mass and a partial right facial paresis (Figures and ). A CT scan demonstrated a 11.2 × 9.4 × 10.7 cm mass centered in the right parotid effacing the jugular vein and abutting the mandible and skull base with extension along the facial nerve to the geniculate ganglion (Figures and ). She was taken to the operating room and underwent a radical parotidectomy with facial nerve sacrifice, radical neck dissection, parapharyngeal space resection, and lateral temporal bone resection (Figures , , and ). The tumor was found to be invading the jugular foramen requiring occlusion of the sigmoid sinus and packing of the jugular foramen for vascular control. The tumor was also noted to be extending medially to the geniculate ganglion of the facial nerve. Gross tumor removal was accomplished (). We were not able to obtain a negative margin on the proximal facial nerve; thus, the ipsilateral masseter nerve was grafted to the buccal and marginal mandibular branches of the distal facial nerve. Her face was further rehabilitated with a static palmaris longus sling and temporary tarsorraphy, which was later replaced with a gold weight. The defect was reconstructed with a large cervicofacial rotation flap and a radial forearm free flap (). Pathologic examination revealed a 14 cm low-grade acinic cell carcinoma with extensive perineural and lymphovascular spread. The lymph node dissection yielded 29 lymph nodes, all of which were negative for malignancy. Postoperatively, she received 6000 cGy in 30 fractions using intensity modulated radiation therapy (IMRT) to the primary site and neck. During and after radiation, the patient experienced massive weight loss, losing approximately 200 lbs. Follow-up imaging one year after treatment revealed no evidence of local or regional recurrence; however, there was interval development of multiple new bilateral lung nodules up to 0.9 cm highly suspicious for metastases (Figures and ). They were deemed too small to be biopsied percutaneously. The patient was referred for consideration of palliative chemotherapy; however, as she was asymptomatic the decision was made to follow her with serial imaging.
A 36-year-old female presented with a recurrent right upper extremity STS. Two years prior to initial presentation at our facility, she was diagnosed with an 11-cm right axillary, intermediate-grade pleomorphic spindle cell neoplasm. She underwent neoadjuvant chemoradiation therapy followed by a resection of the tumor. Microscopic margins were positive following the surgical resection and adjuvant chemotherapy was initiated. She developed a local recurrence 3 months after resection which ultimately progressed to invasion of the brachial plexus with paralysis of the right upper extremity as well as a pathologic humeral fracture. She was placed on low molecular weight heparin after developing a right subclavian vein thrombosis. Additionally, she developed distant metastatic disease in the left upper lobe of the lung. She perused several experimental modalities that were unsuccessful at reducing the tumor burden.\nShe presented to our institution 2 years after the initial diagnosis with debilitating pain of the right upper extremity. An MRI revealed a 25-cm shoulder mass involving the proximal arm muscles with encasement of the axillary artery, vein, and brachial plexus (Fig. ). After a multidisciplinary discussion, a palliative forequarter amputation was performed. Tumor thrombus was evident within the axillary artery and vein during ligation.\nTen hours after completion of the operation, she developed an acute onset of dyspnea with a resultant increase in her supplemental oxygen requirement. A chest x-ray was obtained which revealed bilateral atelectasis. Suspicion of a pulmonary embolism remained high and confirmatory testing was performed with a computed tomography (CT) chest (Fig. ). A pulmonary saddle embolism was present on the CT scan and she was started on an intravenous heparin infusion. A bilateral lower extremity venous duplex study did not reveal a deep vein thrombosis. Her blood pressure subsequently decreased, and she was unresponsive to crystalloid intravenous administration; therefore, she was placed on vasopressor support. A transthoracic echocardiogram (TTE) was performed and revealed an elevated pulmonary artery pressure (49 mmHg), tricuspid regurgitation, right heart dilation, and reduced right heart systolic function consistent with acute cor pulmonale. The patient declined a median sternotomy with pulmonary artery embolectomy. She went into cardiopulmonary arrest and expired within 24 h of the operation. An autopsy was declined but TE was presumed to be the source of PE due to the presence of tumor invasion into the vein.\nThe final pathology of the primary tumor revealed a 25 × 16 × 13 cm high-grade myxofibrosarcoma. The tumor invaded the bone, skin, and neurovascular bundle and contained tumor thrombus.
The second case presents a 60-year-old man with a history of hypertension. He was brought to the emergency room because of several generalized tonic-clonic seizures, and a head CT showed a frontal intracerebral hemorrhage. In the following weeks, two MRI scans had to be cancelled because the patient suffered from claustrophobia and could not cooperate. Contrast CT scan was not performed, even though it would have been relevant. A cerebral angiography was performed and excluded a vascular cause of the hemorrhage. A frontal tumor was found when the patient had an MRI four months after the initial intracerebral hemorrhage.\nThe patient suffered from several subsequent seizures. An operation was scheduled for removal of the tumor. The patient was brought to the emergency room a few days before the scheduled surgery with decreasing consciousness, and an acute CT scan revealed a new bleeding from the tumor. He therefore underwent emergency surgery during which there was extensive bleeding during removal of the tumor. The patient had no early postoperative MRI because the hypothesized diagnosis was metastasis from an unknown malignant melanoma. The microscopic examination revealed a malignant astrocytoma with numerous mitoses, microvascular proliferation, and pseudopalisading necrosis. The tumor cells stained positive for GFAP, map2, and olig2. p53 demonstrated weak staining. Immunohistochemical stainings did not reveal IDH1- or ATRX-mutations. Ki67 was high. The findings were compatible with glioblastoma, WHO grade IV. The average MGMT promoter methylation was 42%.\nDue to the new intracerebral bleeding, the patient spent one month at an intensive care unit and after this the patient and his family decided not to go through radiation. At this point, the patient was at performance status 4 and was treated at a palliative care unit until his death 10 months after his first hemorrhage. An autopsy was performed, and this revealed well demarcated solid metastasis in the liver. The solid metastasis measured 1 × 1 × 0.5 cm. Microscopic examination revealed a metastasis composed of spindled cells with scattered mitoses. Necrosis or microvascular proliferation was not observed. Immunohistochemical staining revealed strong and uniform staining for GFAP () and S-100 but not IDH1-mutation, and they were negative for map2, olig2, melan A, pancytokeratin, desmin, and actin. P53 staining was weak. These findings were compatible with metastasis from glioblastoma. The MGMT promoter methylation was 37%. There was no suspicion or complaints during the course of the disease that could lead to suspicion of liver metastasis.
A 53-year-old male presented to the hospital with shortness of breath. His past medical history was significant for poor baseline functional status, obesity hypoventilation syndrome, and obstructive sleep apnea on bilevel positive airway pressure ventilation nightly and with naps, chronic obstructive pulmonary disease on 4 L supplemental oxygen at home, chronic diastolic heart failure, paroxysmal atrial fibrillation, chronic kidney disease, history of pulmonary embolism, morbid obesity with a body mass index of 72 kg/m2, and a large right chest wall mass (). He had multiple admissions in the past year for dyspnea secondary to heart failure exacerbation. Prior pulmonary function testing for dyspnea evaluation was suggestive of a restrictive disease process with a functional vital capacity (FVC) of 17% of predicted with a normal forced expiratory volume in one second (FEV1)/FVC. His chest wall mass had been present for about 15 years, gradually increasing in size with accelerated growth in the preceding 6 months. It had previously been estimated to be 10 cm × 20 cm × 15 cm in maximum dimensions but had increased to about 55 cm in the largest diameter at the time of presentation. He reported the mass to have been diagnosed as a lipoma by biopsy several years ago and had opted against surgery at that time as it was benign.\nHe underwent further evaluation of his dyspnea in the hospital and his chest radiograph was suggestive of compressive atelectasis of the right lung with a large right-sided pleural effusion. An ultrasound of the chest wall revealed a nonspecific echogenic lobulated soft tissue mass with extensive subcutaneous edema and interdigitating hypoechoic septations in the right chest wall (). He was initially treated with diuretics for suspected exacerbation of heart failure due to his anasarca and elevated proBNP of 3391 pg/mL, but subsequently became hypotensive. He was progressively more lethargic during his hospital stay and a venous blood gas analysis revealed hypercapnia with pCO2 >102 mm Hg. A repeat chest radiograph showed complete opacification of the right hemithorax with nonvisualization beyond the right main bronchus (). He was initially supported with noninvasive positive pressure ventilation on the medical floor but persistent hypercapneic and hypoxemic respiratory failure led him to the medical intensive care unit.\nFurther investigation with a computed tomography (CT) scan () of the thorax, showed complete collapse of the right lung, extensive nonspecific soft tissue edema in the right anterolateral chest wall, right axilla, and right shoulder with a large right pleural effusion. The patient was electively intubated for a bronchoscopy, which revealed mucus plugging and Pseudomonas spp. growing in tracheal aspirates, treated with frequent suction and antibiotics. There was evidence of extrinsic compression of the right mainstem bronchus on bronchoscopy. The patient was unable to be weaned off the ventilator and thus a tracheostomy was performed. Due to the large chest wall mass compressing vital structures, the patient experienced intermittent episodes of syncope during repositioning and out-of-bed efforts, becoming largely bedbound and ventilator-dependent.\nA core needle biopsy with histopathology of the soft tissue chest wall mass showed largely adipose tissue, a fragment of fibrous tissue, edematous changes, and focal chronic inflammation ( and ). Immunohistochemistry for CDK4 and MDM2 were negative to rule out an atypical lipomatous tumor (ALT). Beta-catenin staining to rule out fibromatosis was absent in the fibrous component (). Over his approximately 3-month hospitalization, multiple surgeons determined that the patient was a very poor surgical candidate for mass resection given his comorbidities and size of the mass.\nHe had a prolonged stay in the hospital due to multiple complications associated with lung compression. He experienced multiple episodes of ventilator-associated pneumonia from the tracheostomy and renal failure from repeated courses of high-dose antibiotics and diuresis. The patient was determined to have a poor prognosis, and an informed decision was made to transition to comfort care measures only. He died within a day of being disconnected from the ventilator.
A 51-year-old female with a diagnosis of treatment-resistant schizophrenia was admitted to her local hospice. Historically her schizophrenia had been resistant to a number of antipsychotic agents. She had been functioning relatively independently in a supported living center for five years and, from a psychiatric viewpoint, had remained stable on her oral medication regimen, which consisted of aripiprazole 10 mg daily and clozapine 50 mg in the morning and 125 mg at night.\nShe had been diagnosed with SCC of both tonsils and uvula (T3 N3 M0), which was being conservatively managed. The N3 node was located in the left mandibular region and presented as a large painful mass causing a pressure effect on local structures. Upon admission to her local hospice, it was evident that her swallow was deteriorating due to the local effects of her cancer and that consistent administration of oral antipsychotic agents could not be sustained.\nThe patient had capacity to make the majority of decisions regarding her care. She had a supportive family who liaised with the medical team regularly and were invaluable in establishing the patient's typical behaviors and mental health status. The placement of an enteral feeding tube (both percutaneous endoscopic gastrostomy and nasogastric tube) and the possibility of total parenteral nutrition were discussed, but it was agreed by all not to proceed with such interventions due to the risks of harm, lack of long-term benefit (especially since no active or palliative treatment options were available for her SCC), and the distress it would likely cause the patient. Despite a deteriorating swallow, the patient was able to intermittently consume ice cream and nutritional supplements but had been eating limited amounts, even before the deterioration in her swallow, due to anorexia–cachexia syndrome—an archetypal and familiar phenotype of advanced malignant disease.\nThree weeks after admission, as a consequence of both the local and systemic effects of her advancing cancer, the patient's swallow had deteriorated to the point wherein consistent oral administration of her antipsychotics was no longer viable. Having excluded other enteral routes, we sought a suitable parenteral alternative. Continuation of antipsychotics was of particular importance, as her history of treatment-resistant schizophrenia and expected prognosis extending into several weeks meant she was at high risk of relapse.\nWe considered administering both long- and short-acting intramuscular antipsychotic agents. The patient, however, was averse to receiving injections that could be painful. On multiple occasions throughout the admission, the patient had refused the placement of an intravenous cannula, and had refused venipuncture for blood sampling, as she found these procedures both painful and prohibitively distressing. This, combined with reduced muscle mass as a consequence of anorexia–cachexia syndrome, meant that intramuscular administration would not be a viable solution, particularly for repeated injections. The use of long-acting injectable (LAI) antipsychotics also presented additional challenges. If adverse effects to an LAI antipsychotic occurred, it would not be possible to withdraw the medication, and ongoing adverse effects would have had the potential to significantly compromise quality of life. In addition, LAI antipsychotic agents, compared with standard release products, do not allow for rapid dose titration or de-escalation in response to changes in clinical condition and therapeutic response. The ability to optimize medications promptly at the end of life is essential to be able to maximize therapeutic efficacy and minimize adverse effects, to ultimately improve quality of life in situations wherein time may be short. Lastly, experience regarding rotation from oral clozapine and aripirazole to an LAI antipsychotic is lacking. With all other viable routes exhausted, the team was pleased to find that the patient fortunately did not find subcutaneous injections painful, and so would contentedly receive medications through this route.\nConsequently we opted for a standard-release antipsychotic agent that could be administered subcutaneously. Previous exposure to a phenothiazine antipsychotic had caused severe muscle spasms. Thus subcutaneous levomepromazine was avoided and haloperidol had previously been ineffective. Subcutaneous olanzapine was thus chosen and trialed initially at a dose of 10 mg once a day. This dose was selected based upon manufacturer recommendations and supported by a systematic review of 10 studies that demonstrated that negligible further improvement in psychotic symptoms was conferred when doses surpassed 10 mg per day.\nFor four weeks, the patient was able to continue interacting with her family and the ward team, without resurgence of psychotic symptoms. Four weeks after commencing the olanzapine however, her family noticed that she was exhibiting signs that they identified as relapse of her schizophrenia. She had become more withdrawn, distressed, and suspicious of her family and the staff. Her mental state was carefully assessed and, after the exclusion of all other reversible causes, her symptoms were attributed to an exacerbation of her existing psychiatric disorder rather than a new delirium. Therapeutic drug monitoring for olanzapine was not performed, as the patient had declined any further blood sampling. Consequently, olanzapine was carefully titrated according to clinical response and tolerability up to a dose of 10 mg twice a day in a stepwise manner over the course of a week, and all of her psychiatric symptoms resolved.\nThe patient remained on olanzapine 10 mg twice daily, as subcutaneous bolus injections for a further eight days. Over the following days, however, she began spending more of her time in bed and sleeping for longer periods. We considered if this may have been an adverse effect of the olanzapine, yet the multidisciplinary team assessed that her rate of deterioration, diminishing appetite, and significant weight loss to be more congruous with the expected disease trajectory of a patient dying from progressive cancer. To minimize disturbances, avoid recurrent bolus injections, and further simplify the regimen, we switched to administering olanzapine 20 mg for 24 hours as a continuous subcutaneous infusion through a syringe driver.\nThe patient died peacefully 14 days later with her family by her side. Over the course of the admission, olanzapine was administered subcutaneously for a total of 56 days. Administration of olanzapine in this manner was well tolerated with no injection site reactions or systemic toxic effects being observed or reported by the patient or her family. These findings suggest that olanzapine at a dose of 20 mg per day was both efficacious and well tolerated in the management of her treatment-resistant schizophrenia in a palliative care setting. The resolution of her paranoia allowed the patient to interact with her family, thus improving her quality of life. No trigger was ever identified for her deterioration in mental health, but it was felt to be a combination of disease progression, pain, and interruption to her usual antipsychotic regimen.
A 55 year old man was admitted to hospital with a head injury that resulted in hearing loss on the left side with pulsatile tinnitus and a vague feeling of disorientation. A CT scan showed an irregular extra-axial solitary lesion lying adjacent to the falx on the right of the midline measuring 3 × 4 × 6 cm with significant mass-effect and peritumoral oedema involving most of the right frontal lobe. There was evidence of underlying bone remodelling with vascular dural supply and heterogeneous enhancement after contrast administration with a deep cystic component. There were fringes of tumour interdigitating with the brain substance indicating probable brain invasion (Fig. ). The findings were consistent with an aggressive frontal meningioma. Partial surgical removal of the lesion in the right frontal lobe was undertaken. On histological examination, the lesion was classified as an atypical (WHO Grade II) meningotheliomatous meningioma.\nA year later, the patient experienced an episode of seizures and a CT scan revealed three new lesions at the site of the previously excised meningioma with extensive perilesional oedema. An MRI confirmed the CT findings indicative of tumour and also detected invasion and occlusion of the anterior part of the superior sagittal sinus (Fig. ). Partial resection of the recurrent mass was again undertaken. Histology of the tumour specimen showed features of an anaplastic (WHO Grade III) meningotheliomatous meningioma with lobules of tumour containing cells with round or oval vesicular nuclei. Focally, there were cells which showed more prominent nuclear pleomorphism. There was increased mitotic activity and extensive tumour necrosis was noted (Fig. ). Post-operative follow-up revealed residual tumour in the anterior part of the superior sagittal sinus, along the falx and the right frontal convexity. Follow-up imaging also confirmed interval progression of the residual meningioma.\nThe patient completed a course of radiotherapy and, nine months later, a mobile soft tissue lump on his back was noted. A thoracic CT scan revealed a soft tissue lesion superficially located in the inferior portion of the left trapezius muscle. The lesion was lobulated, had a low attenuation centre, showed peripheral enhancement and did not exhibit evidence of matrix calcification (Fig. ). The lesion increased in size and a subsequent MRI scan performed 6 months later confirmed the presence of a solid soft tissue mass up to 5 cm in diameter in the medial left trapezius muscle. The lesion indented the underlying paravertebral muscles but remained well-defined and did not invade the deeper musculature. The lesion returned isointense T1-W signal relative to skeletal muscle and heterogeneous high T2-W signal. There was no internal calcification, haemorrhage or cystic degeneration. Peripheral feeding vessels were present (Fig. ). A PET-CT scan demonstrated that the intramuscular lesion was extremely FDG avid with an SUVmax of 22.1 (Fig. ). PET-CT also revealed disseminated, markedly FDG avid metastases in liver, bone and pleura and residual meningioma in the right frontal lobe.\nThe lesion was biopsied and showed a largely lobulated tumour containing collections of tumour cells, many of which showed similar features to those seen in the frontal lobe tumour. There were a few whorled collections of tumour cells with round or oval vesicular nuclei (Fig. ). There was prominent nuclear pleomorphism, a high mitotic rate and focal tumour necrosis. Focally there were solid areas of proliferation of pleomorphic tumour cells, some of which had vacuolated cytoplasm. Immunohistochemistry showed strong expression of epithelial membrane antigen (EMA) and P63; there was also focal expression of cytokeratin (CK7+, CK20−), nuclear staining for IN-1, and a high Ki-67 fraction. There was no expression of desmin, S100, CD10, CD30, CD68, myogenin, smooth muscle actin, estrogen receptor, progesterone receptor, chromogranin, carcinoembryonic antigen or TTF1. There was initially some difficulty in establishing the pathological diagnosis as the previous history of recurrent atypical/anaplastic meningioma was not provided to the reporting pathologist whose initial differential diagnosis included a soft tissue metastasis of carcinoma and a primary soft tissue sarcoma expressing the epithelial markers. However, once the history of meningioma and the radiological findings were taken into account, it was clear that the soft tissue mass represented a metastasis of the previously diagnosed anaplastic meningotheliomatous meningioma.\nA wide local excision of the lesion, which measured 5 × 3 × 2 cm, was performed; this included a cuff of normal muscle around the lesion and a 1 cm margin of skin around the previous biopsy scar. Histological findings in the resection specimen were similar to those of the biopsy. Further follow up of the patient by CT scan showed no recurrence of the soft tissue lesion but progression of disease in the brain.
A 43-year-old female was seen in consultation at our thrombosis clinic. She had a stroke at age 14 and had presented with collapse and left sided hemiparesis. Her thrombophilia work-up was positive for a prothrombin G20210A gene mutation in heterozygous form. She had been on aspirin 81 mg daily since age 14.\nPrior to being diagnosed with a renal infarct at age 42, the patient presented with nausea, vomiting, hematuria, and left flank pain and was initially diagnosed as renal colic. She subsequently had a computerized tomography scan of the abdomen and pelvis, which showed evidence of a wedge-shaped area in the lower pole of the left kidney consistent with a renal infarction. She was not on an oral contraceptive. We started treatment with intravenous heparin and transitioned to warfarin for 15 months without any further thromboembolic events.\nGiven that cardioembolic sources are well-documented causes of renal infarction [], the patient had loop monitoring for two weeks and electrocardiograms, which did not detect atrial fibrillation. She also had two echocardiograms, none of which showed any evidence of cardiac thrombus. A transthoracic echocardiogram was performed with agitated saline at rest and after valsalva maneuver, which showed mild to moderate degree of shunting at rest that increased significantly with the release phase of a valsalva maneuver. This was suspicious for a PFO. A follow-up transesophageal echocardiogram showed a small left to right shunt due to a small ASD rather than a PFO. The patient had device closure of the ASD with no evidence of any remaining shunt on a transthoracic echocardiogram. It is likely that the patient's renal infarction was related to paradoxical embolism caused by small deep vein thrombosis migrating through the ASD shunt.\nAfter 4 months of being off of anticoagulation, patient had a D-dimer test, which was positive at 591 μg/L. There were no other reasons for the elevated D-dimer. Based on an annual risk of recurrence of approximately ten percent in females with a first unprovoked venous thromboembolism (VTE) event and a positive D-dimer, the patient was restarted back of warfarin [].
Case 2 was a 75-year-old Japanese man who presented at our hospital with acute abdominal pain, nausea, and cold sweat. His CT scan showed retroperitoneal bleeding (around the pancreas and the dorsal side of the ascending colon). His general condition was stable, but he was admitted to our hospital as a conservative measure. His angiography (6 days after admission) showed an aneurysm of the pancreaticoduodenal artery without active bleeding.\nOur experience treating MAL syndrome in case 1 enabled us to diagnose the disease accurately in case 2. MAL syndrome was the cause of the aneurysm in this patient too (). We selected laparoscopic surgery based on the MAL syndrome and the benefits of this surgery. The patient's posture for the surgery was the lithotomy position. Intra-abdominal pressure of 12 mmHg was maintained. The points of the trocars were as follows: a 12 mm trocar at the navel for the camera, two 12 mm trocars at the right upper abdomen, and a 12 mm trocar and a 5 mm trocar at the left upper abdomen ().\nFirst, we lifted the liver umbilical ligament by surgical sutures and put in an organ retractor to the crus of the diaphragm in order to improve the field of vision. After opening the omental bursa, we lifted the stomach with a snake retractor and observed the dorsal side of the stomach. We confirmed the left gastric artery and tied it with tape. The tape was taken out from the right outside trocar, and an assistant pulled it to provide traction of the surgical field. Following the celiac artery to the root, the artery was fastened by the MAL. We cut the MAL away little by little with a vessel-sealing system until the running direction of the celiac artery was clearly confirmed. Using a blood flow meter, we confirmed the improvement of blood flow of the left gastric artery (from 5 mm/min to 69 mm/min). A drainage tube was placed in the left subphrenic area, and the wound of the abdominal incision was closed. The operation time was 3 hours 35 minutes, and the blood loss was minimal at 15 ml. The patient's CT scans have shown no recurrence of the aneurysm for 2 years.
An 81-year old female with a history of coronary artery disease, hypertension, and thrombocytosis suffered a witnessed trip and fall onto a nightstand. The patient took 75 mg of clopidogrel daily in addition to an 81 mg aspirin tablet. She reported a mild headache however had no change from her baseline mentation per family members with no evidence of obvious injury aside from a small area of ecchymosis near a small forehead laceration. She remained up and ambulatory with no further complaints. Ten hours after her injury the patient presented to the Emergency Department with stridorous and agonal respirations with a profoundly decreased level of consciousness. She was noted to have developed extensive ecchymosis on the anterior portion of her neck and chest. Her symptoms had begun rapidly shortly prior to arrival while lying in bed. Family reported that she had been in the constant company of her husband with no further falls or injuries that had occurred since her fall. The patient was intubated upon hospital arrival due to respiratory extremis with obvious swelling and crepitus noted on neck examination. A noncontrast CT scan of head was unremarkable while there was demonstration of a large retropharyngeal hematoma measuring 3.6 cm by 5.3 cm by 20 cm on a CT of the cervical spine with no evidence of fracture. Her hemoglobin was 9.5 gm/dL and platelets were 1234 per deciliter, with an INR of 3 and a slightly below normal and activated partial thromboplastin time of 23.9 seconds (reference range 25-35 seconds). A CT angiogram of the neck was subsequently obtained demonstrating active bleeding from the anterior ligaments of the vertebral column that was not felt to be amenable to embolization (). Given the extent of the hematoma intraoral surgical evacuation was performed with bleeding from the anterior vertebral spine controlled with Bovie cauterization, placement of topical thrombin, and drain placement. No reaccumulation of hematoma was noted during her hospital course. The patient unfortunately expired 12 days from the date of admission from presumed aspiration pneumonia and multisystem organ failure.
A 21-year-old male with dissociative convulsions and fugue. When Mr. F first visited us in March 2013, he was a 21 years old single student into his 2nd year of graduation. There was no past or family history of neuropsychiatric morbidity or substance use. He presented with episodes of tonic stretching of his limb and neck muscles. These episodes occurred in a clear consciousness without any fall, incontinence or injury in a frequency of once-twice a month starting from 2004. These episodes had increased to ten-twelve per day since April 2013 with some correlation of increasing stress after working for a brief period of time in a call center; each episode lasting for around 10-20 min with Mr. F being well aware of the surrounding around him and able to recollect conversations around him during the episode. There was one episode for around half an hour in which Mr. F had wandered out of the house to an unknown location and had to be traced and when found, was unaware of his whereabouts and how he had got there. He underwent an awake EEG and a computed tomography scan brain which showed normal results. He had been treated with a combination of antiepileptics and antidepressants with no improvement in his condition. He was admitted in April 2013 on an involuntary basis and treatment was initiated with a major focus on nonpharmacological strategies, elicitation of stressors, and all his medications were tapered and stopped. The episodes continued during an inpatient stay. He wondered out of the ward during one of his episodes and was not traceable for 24 h. He returned on his own and recollected that he had boarded a train and had gone to an unknown location, but was unable to recall what he did at that place or how did he maintain himself in those 24 h. He recollected that he was restless and had to go. The resistance to therapy continued for nearly 3 weeks
A 59-year-old male underwent uneventful DDLT for decompensated hepatitis B-related liver cirrhosis with a MELD score of 19. The transplant surgery and postoperative course were uneventful. He was discharged on the 11th posttransplantation day with stable liver function. Immunosuppressive protocol was as per standard institution protocol. The patient was admitted after 6 weeks in an emergency department with hypotension, vomiting, and altered sensorium associated with oliguria. Liver functions were grossly elevated, and he had a systolic hypotension of 70 mmHg. Emergency abdominal ultrasound scan showed mild hepatomegaly and an echogenic thrombus in the retrohepatic IVC near the suprahepatic anastomosis () which was confirmed by computed tomography (CT) angiography that also revealed renal vein and iliac vein thrombosis as an incidental finding.\nThe patient was admitted in ICU and started on anticoagulation therapy, and a digital subtraction inferior venacavogram was done. This showed a focal severe stenosis approximately 70% in the inferior vena cava at the level of the T12 vertebra. There was an associated thrombus 6 × 3 cm within the retrohepatic and suprahepatic inferior vena cava with complete cut-off of the inferior vena cava 3 cm proximal to its junction with the right atrium. Intravascular thrombolysis using urokinase 50,000 IU was immediately instituted, and the patient was maintained on 100,000 IU/hour of urokinase infusion in the IVC with repeated mechanical thromboaspiration. The patient remained stable during the thrombolysis, and there was reestablishment of blood flow across the previously occluded part of the IVC. Post procedure abdominal ultrasound and Doppler ultrasound were done after 48 hrs, and both still showed the presence of a thrombus. Abdominal CT scans also showed severe stenosis in the suprahepatic inferior vena cava just proximal to the right atrial junction with mild to moderate ascites and splenomegaly. The patient had another angiography done which confirmed a 2 cm short segment severe stenosis of about 95% in the IVC at its junction with the right atrium (). The IVC was twisted along its long axis in this region.\nHe then underwent inferior vena cava venoplasty, and an endovascular stent was inserted whereby a balloon-mounted stent was deployed across the stenosis site to a size of 18 × 32 mmm (). Post stenting, there was improvement in the calibre of the IVC and it was untwisted (). There was no pressure gradient proximal and distal to the stenosis.\nFollowing the procedure, the patient was given low molecular weight heparin 2500 IU subcutaneously for 3 weeks. The patient made uneventful recovery and was discharged 2 weeks post stenting on warfarin with a target international normalized ratio between 2 and 2.5. The liver functions were normal at the time of discharge.\nFollowing the procedure, the patient was given low molecular weight heparin 2500 IU subcutaneously for 3 weeks. The patient made uneventful recovery and was discharged 2 days post stenting on warfarin with a target international normalized ratio between 2 and 2.5. The liver functions were normal at the time of discharge.\nSix weeks after discharge, he was reviewed and had no complaints. An abdominal ultrasound done showed no thrombus in the inferior vena cava and the rest of the abdominal organs were normal as well as his liver and renal function tests.
A 48 years old female patient was admitted to our institution because of the difficulties in swallowing food and the swelling of the tongue. The otorhinolaryngology exam showed bluish, localized, approximately 35 × 25 mm big swelling on the upper side of tongue, with vessels shining through the glossal mucosa. The lesion was localised on the right side of the tongue in the middle part. The radix of the tongue was free. After the biopsy the histological examination of the tissue was performed and cavernous haemangioma was diagnosed.\nContrast enhanced MRI with time resolved imaging of contrast kinetics sequence (TRICKS) was performed, so that the best method of treatment could be selected (, and ). The maxillofacial surgeon planed a surgery, so we were asked to perform a preoperative intra-arterial embolization. Because of the superficial localization of the lesion, we chose a direct puncture as the most appropriate approach.\nThe procedure was performed under general anaesthesia. This provided us the complete analgesia and prevention of tongue movement during the puncture and the injection of the ethanol and the contrast media.\nWe performed parenchimography of the lesion using several projections to reach an optimal view of the lesion and to evaluate the potential extravasation of contrast from haemangioma (, ). This enabled us a more controlled injection of ethanol and gave us a possibility to avoid the reflux to the venous side. For the puncture we used 10 to 12 G needle (Terumo, Tokyo, Japan). After the puncture the proper position of the needle was verified with a continuous reflux of blood. Then the cannula was flushed with a 5% glucose solution. Under fluoroscopy control 96% alcohol diluted with Lipiodol (ratio 1:5) was injected. The cannula was removed and next puncture was performed. All together we performed six punctures and injected approximately 6 ml of ethanol. There were no technical complications during the procedure (). No major bleeding was observed after the procedure. After the procedure the patient received analgetics and antioedematous therapy. After the sclerotization the planed surgery was abandoned. Control MRI examinations 6 and 12 months after the procedure showed only a small remnant of haemangioma and no signs of a larger relapse ().
A 25-year-old female referred to the author by the private clinician. The patient had undergone surgical placement of 2 implants in mandibular anterior region 2 months back. No postoperative pain or swelling was mentioned in case sheet. No history of any trauma in immediate postoperative phase was noticed. Patient general status was healthy, and she was not suffering from any bone disorders. The clinician mentioned about mobility with a dental implant placed in the mandibular right central incisor region. Intraoral examination revealed no soft tissue dehiscence noted in the concerned area. Periodontal health of adjacent teeth was normal. Mucoperiosteum covering the dental implant in 41 regions was so thinned that implant outline can be easily appreciable in transmitted light (). The implant was Grade I mobile. The marginal bone loss was seen in the adjacent implant.\nThe case discussed with the patient and depicted management options of implant removal or bone reinforcement to support the implant. The mandibular grafting technique was explained to patient and complications explained. The patient was ready for the surgery under local anesthesia.\nBilateral mental nerve blocks were given. Local anaesthesia with adrenaline administered in the mandibular vestibule. Crestal incision with two vertical releasing incisions placed. Full thickness mucoperiosteal Trapezoidal flap raised. Care was taken to raise mucosa in contact with the implant. Flap raised until the lower border of the mandible.\nAfter hemostasis, the examination revealed completely denuded buccal aspect of the implant in relation to 41 (). The defect measured to be about 18 mm mesiodistally. The thickness of the graft needed was assessed to be 2-3 mm, in order to compensate for the buccolingual loss of bone.\nUsing these measurements, the graft was marked 5-6 mm away from the apical margin of the implants. The safe distance of 5 mm from mental neurovascular bundle and inferior border of the mandible was maintained. The graft was marked using postage stamp method (). The graft was procured using chisel and a mallet and stored in sterile water. Some amount of cancellous graft was procured from the same site.\nTen milliliters blood was drawn from patient and sent for the preparation of platelet rich plasma. The graft was contoured from lingual aspect in order to fit according to implant contour. Margins of the graft were smoothened to prevent tearing of the flap. The graft was stabilised over the buccal aspect of implants using titanium screw away from implants ().\nCancellous bone was placed in between graft and implant. Platelet-rich plasma was placed over the graft. Gelfoam was packed at defect created at graft site. Periosteal scoring was done to achieve tension free closure. Hemostasis achieved and tension free closure was done using resorbable sutures.\nPressure dressing was placed over the chin to reduce edema. Antibiotics and analgesics were prescribed to the patient.\nWound healing found satisfactorily, and no any postoperative complications seen. Implant mobility was reduced to zero. Implants were loaded successfully after 6 months of surgery. No prosthetic or esthetic problems observed. Most importantly, the patient was very happy and thankful after the prosthetic replacement!
A 14-month-old boy presented to our hospital with developmental regression and severe dystonia. He was born at term by spontaneous vaginal delivery following an uneventful pregnancy with no history of perinatal asphyxia. He is the first baby of first-cousin parents with no family history of metabolic disorders or early neonatal deaths (). He developed neonatal jaundice on the second day of life, and was treated with phototherapy for 5 days. He was noted to be floppy since early infancy with delayed gross and fine motor development. He achieved head control after the age of 8 months, and started to sit unsupported at 10 months of age. However, he lost some of the developmental skills that he had gained after an acute gastroenteritis at the age of 11 months. This acute illness was complicated by encephalopathy and seizures. Extensive workup was carried out including cerebrospinal fluid analysis that was negative for viral and bacterial infections. He remained with severe spasticity, not responding to extensive physiotherapy and Baclofen therapy. His initial evaluation at our hospital showed a spastic child with severe dystonic posture and failure to thrive. His investigations revealed normal serum lactate and ammonia with no evidence of metabolic acidosis. His brain magnetic resonance imaging (MRI) showed abnormal high signal intensity at basal ganglia and widened Sylvian fissure (Figures ). Based on clinical presentation along with the MRI findings, the diagnosis of GA1 was considered. He was started on intravenous dextrose 10%, oral carnitine, and low protein diet. Few days later, his urine organic acids result showed elevated 3-hydroxyglutaric acid, while serum amino acids were normal. Acylcarnitine profile revealed high glutarylcarnitine, consistent with the diagnosis of GA1. The deoxyribonucleic acid extracted from lymphocytes was used to amplify the 11 coding exons, and the corresponding flanking sequences of the GCDH gene. The polymerase chain reaction products were analyzed by sequencing in both forward and reverse directions. Sequence analysis identified 2 copies of a missense mutation, c.482G>A in the coding region of the GCDH gene. This homozygous mutation predicts an amino acid change of arginine (R), glutamine (Q) at codon 161 of the dehydrogenase protein (p.R161Q). Also, both parents were found to be carriers of the same mutation. This mutation has been previously reported in patients with GA. Our patient was maintained on low lysine diet with supplementation of L-carnitine 100 mg/kg/day. His follow-up showed complete resolution of the extrapyramidal signs with significant improvement in motor skills and cognition.
A 46-year-old female with a history of diabetes mellitus, morbid obesity, peripheral vascular disease, and dense peripheral neuropathy was initially treated in another facility (private outpatient clinic) for a calcaneal puncture wound with multiple incision and debridement procedures. The patient had a history of smoking for many years and also sustained a calcaneal osteomyelitis secondarily to the original infection with Staphylococcus aureus and Pseudomonas aeruginosa organisms ().\nIn our facility, the patient had immediate non-invasive vascular studies that were followed with a lower extremity angiography that showed a vascular occlusion of the popliteal artery around the popliteal fossa (). The patient had agreed to quit smoking and was educated on a diabetic diet regimen with tight control over her blood sugars. At that time, the vascular surgery team performed a lower extremity bypass surgery (femorotibial) with the use of a saphenous vein graft while our team performed a simultaneous aggressive debridement of all the necrotic bone and soft tissue. Intraoperative bone and soft tissue cultures and biopsies were also obtained.\nA postoperative regimen of deep vein thrombosis prophylaxis (low molecular heparin) and intravenous antibiotics based on the culture results (Keftazidim: 2 g ×3 doses and Vancomycin: 500 mg ×3 doses) were initiated and the patient's vascular status of the lower extremity was monitored for approximately 3 weeks. A significant improvement of the skin temperature and blood perfusion of the limb was observed and it was confirmed with further Doppler non-invasive vascular studies.\nAt that time, the patient then had further surgical debridement and closure of the large calcaneal defect with a reverse flow sural neurofasciocutaneous flap. At the beginning of the surgery, a revisional resection of the calcaneal osteomyelitis was performed. The exact bone resection was defined with a macroscopical inspection under loop magnification (6×) combined with blue methylene fast staining and successive immediate multiple irrigations with normal saline solution. The procedure was followed with the flap dissection of the sural artery and associated neurovascular structures that were identified and ligated proximally. The flap's pivot point was located distally over the defect area and approximately 5–7 cm proximal of the lateral malleolar area. The flap was innervated with end-to-end coaptation of the sural nerve at the distal end of the lateral plantar nerves with epineural sutures. The sural flap was then rotated to the defect area and was secured without any skin tension (). The donor area was covered with a split thickness skin graft that was harvested from the ipsilateral thigh. The neurovascular bundle was covered primarily with an approximation of the angles of the ‘Z’-initial incision in order to avoid skin tension. The rest of the uncovered areas were grafted with split thickness skin from the ipsilateral thigh. The sural flap dimensions were approximately 6.5×15 cm in diameter.\nDuring the first postoperative week, the flap sustained a superficial venous congestion and approximately 5% of the flap coverage was lost due to skin necrosis. The venous congestion was managed with the use of a vasodilating agent Buflomedil Hydrochloride (600 mg ×1 over 24 h) and an oral micronized purified flavonoid fraction (MPFF; Daflon, a vasoprotector and venotonic agent). The epidermal necrosis was healed after a minor surgical revision and loose reattachment of the flap margins. During the postoperative period the patient continued the intravenous antibiotic therapy for 2 months, followed by oral antibiotic therapy for more than a 1-month period. Serial postoperative pedal radiographs had shown no evidence or recurrence of the calcaneal osteomyelitis. The patient was also kept non-weight bearing for approximately 3 months after the initial surgery. The flap was completely healed and the patient was ambulatory with a custom molded shoe at 4 months postoperatively ( and ).
A 10-year-old girl brought by her mother to the emergency department presented with the complaint of sudden onset blurring of vision. She had a history of hospital admission two days before this episode and was admitted for the management of viral meningitis. The patient at the time presented with a history of fever and bilateral swelling of the parotid region for a week with associated headache and vomiting. Pertinent examination findings included bilateral parotid gland swelling accompanied by cervical lymphadenopathy. On central nervous system examination, signs of meningeal irritation were positive. Cerebrospinal fluid (CSF) analysis was subsequently done, and it showed increased white blood cell count, normal glucose, and slightly increased protein levels, which were consistent with viral meningitis. Based on CSF analysis and clinical presentation, the diagnosis of mumps meningoencephalitis was confirmed. She received supportive therapy during the hospital stay and was discharged 10 days later.\nThe general physical examination now revealed an irritable child with intact orientation. Her vitals were normal. Ophthalmology consult was taken, which showed visual acuity of 6/6 in the right eye and 6/60 in the left eye. There was a mild relative afferent pupillary defect in the left eye, whereas the color vision of both eyes was also defective. Anterior segment examination was within the normal range. Fundoscopy revealed bilateral optic disc edema with the blurring of the optic disc margins accompanied by few splinter hemorrhages around the discs. These findings were more prominent on the left side as compared to the right side. The central nervous system examination was unremarkable. Magnetic resonance imaging (MRI) of the brain without contrast revealed thickening of both optic nerves (Figure ). The diagnosis of bilateral optic neuritis secondary to mumps meningoencephalitis was confirmed.\nThe patient was immediately started on intravenous methylprednisolone 750 mg/day for three days, followed by oral prednisolone 5 mg at a dose of 1 mg/kg. Her follow-up a week later showed gross vision improvement in the left eye from 6/60 to 6/6, although clinically, there were still signs of optic disc edema; in resolving stage. After 11 days of treatment, clinical signs of optic neuritis disappeared, with no residual disc pallor. Steroids were tapered gradually over the next 20 days. The patient has since recovered with no clinical signs of optic neuritis in both eyes and has resumed her routine daily life.
A 73 year old female with a history of four months of intermittent bright red blood per rectum and thirty pound weight loss presented to our hospital with abdominal cramping and pain. In the Emergency Department she was afebrile with a heart rate of 100 beats per minute. Initial laboratory studies revealed a hemoglobin of 6.3 g/dL and a normal white blood cell count with a normal automated differential. Colonoscopy demonstrated severe diverticulosis in the sigmoid, descending, transverse, and ascending colon.\nShe had a fever of 38.4 degrees centigrade following one of several transfusions of packed red blood cells. Blood cultures were drawn that yielded four out of four bottles of gram-positive cocci in chains, identified as Granulicatella adiacens. An infectious disease consultation was then requested. Trans-esophageal echocardiogram demonstrated a mobile, echodense lesion measuring 0.9 by 0.6 cm attached to the aortic valve with associated severe aortic regurgitation. The cardiothoracic surgery department was consulted and recommended that the patient may eventually require aortic valve replacement but recommended initially implementing conservative management with intravenous antimicrobials.\nSusceptibilities by E-testing for ampicillin and ceftriaxone alone and then in combination at standard concentrations which demonstrated synergy was performed by our microbiology laboratory (). The patient required daily intravenous and oral furosemide to control her congestive heart failure and we deemed her high risk for nephrotoxicity with prolonged aminoglycoside use. After consulting with the patient and her family, we decided to treat her with double beta-lactam therapy with combination of ampicillin 2 g intravenously every 6 h and ceftriaxone 2 g intravenously every 24 h, based on her creatinine clearance and serum albumin, levels for 6 weeks. She was discharged to a nursing home to complete her treatment.\nSeven months later she presented to our Emergency Department with asthma exacerbation. She had no signs or symptoms of IE and her blood cultures were negative. She was then transferred to another institution for aortic valve replacement. Eleven months after treatment for her IE she had recovered well from her valve replacement (personal communication).
The patient is a 14-year-old African American boy with a history of severe factor VIII deficiency. He has been previously treated for intra-articular bleeds in his knees, and was maintained with 3 time per week doses of 1250 U recombinant factor VIII. The patient presented to the emergency department after missing 2 such doses. He was in his usual state of health until the evening before admission, when he noted pain and swelling of his left forearm and ankle. The pain initially responded to nonsteroidal anti-inflammatory drugs, but the following morning, the patient woke to severe pain and could not move his fingers of the left forearm. He was taken to the hospital and seen in consultation by hematology, plastic surgery, and orthopedic surgery.\nAt the time of admission, the patient had a factor VIII activity of 1% (normal range: 50%-200%). Recombinant factor VIII was administered to normalize factor levels. He was alert, but in considerable pain, and denied a history of trauma. Physical examination revealed a tense and swollen left forearm with limited elbow and wrist motion secondary to pain. Pain was diffuse but centered about the volar-radial left forearm and radial hand. Sensation was diminished along the same area, as well as the median and radial nerve distributions of the hand. The left radial pulse was palpable. A diagnosis of compartment syndrome was suspected and the patient was taken to the operating room for emergency fasciotomy.\nIn the operating room, compartment pressures of 72 mm Hg and 10 mm Hg were measured on the left and right volar forearms, respectively. Intraoperative ultrasonography showed diffuse mild edema of the left forearm musculature, with no focal fluid collection or hematoma. Surgical decompression was obtained with generous release of the volar forearm fascia. The compartment was tense; the muscles were swollen and a hematoma of 5 cm3 was evacuated from the volar forearm (). The fasciotomy preceded ipsilateral carpal tunnel release, after which no tenseness was appreciated in the forearm. An anteroposterior radiograph confirmed that there was no bony involvement, and the wound was closed with vascular loops in “Jacob's ladder” fashion. The arm was placed in a dorsal splint extending from the fingertips to the proximal forearm. Postoperatively, the arm was kept suspended, and factor infusions were continued overnight at 35 U/kg every 6 hours.
A 29-year-old male presented with a painful swelling over his right knee for 4 months duration in 2011. The plain radiograph showed an eccentric lytic lesion over the epiphysis of right proximal tibia []. Open biopsy confirmed chondroblastoma extended curettage and bone using Bone cement [Polymethyl methacrylate (PMMA)] was performed. The final diagnosis remained as chondroblastoma [Figure -]. He was followed up monthly for the first 3 months, then 3 monthly for a year and 6 monthly until he developed local recurrence. At each followup, clinical palpation and X-ray were performed to look for local recurrence. He was symptom free for the next 4 years. In 2015, he presented to the followup clinic with pain and swelling over the right knee. Plain radiograph revealed a lytic lesion just adjacent to previous PMMA cement in keeping with local recurrence []. Magnetic resonance imaging showed a homogenous lesion adjacent to the previous bone cement []. Chest X-ray at this point was clear with no evidence of lung metastasis. Alkaline phosphatase and lactate dehydrogenase were within normal limit. Repeat curettage with bone cementation was done [] for the lesion. Within a few weeks, his knee pain and swelling worsened. He was in pain, especially while weight bearing and the pain did disturb his sleep. His knee range of motion was restricted from 15° to 110° of flexion. The histopathology report from the curettage unfortunately came back as conventional osteosarcoma. Further systemic staging showed multiple small lung nodules in keeping with distance metastasis. The bone scan, however, did not pick up any skeletal metastasis. He further underwent proximal tibia wide resection with endoprosthesis reconstruction and medial gastrocnemius flap followed by adjuvant chemotherapy []. Postoperative, his wound healing was uncomplicated, and he started regaining good function of his right lower limb with physiotherapy. The resected specimen was send for histopathology and margins and revealed a high-grade conventional osteosarcoma [Figure and ] with close margins. The histology was reviewed and confirmed by two bone and soft-tissue pathologist as the local pathologist was well aware of the rarity of this case. The patient was started on adjuvant chemotherapy consisting of doxorubicin 25 mg/m2 and cisplatin 50 mg/m2. Sadly, he succumb to lung metastasis after his 3rd cycle of chemotherapy.
A 49-year-old female was referred to our department with persistent pain in left mandibular angle. The patient had been experiencing this symptom for 3 months after biting on a peanut. The medical history of the patient revealed no systemic, endocrine, or metabolic disorders. On extraoral examination, no obvious abnormality was detected except restricted mouth opening. Intraoral examination revealed the malocclusion and the mandibular left first molar was restored with a crown. The patient declared that the mandibular left first and second molars were endodontically treated 5 years ago. An orthopantomograph (OPG) showed a unilocular lesion with a relatively well-defined border extending from the mandibular left second molar to the lower third molar. The dimension of the cystic lesion was 2.3 cm×1.7 cm×2.1 cm. The mandibular left third molar was horizontal impacted and its crown was located in the cystic lesion area. The lesion was associated with an obvious fracture in the inferior border of the mandible (Fig. ). It was even more obvious by 3-dimensional computed tomography (CT). The patient was tentatively diagnosed as having a pathologic fracture in the left mandible. The medical records of the patient revealed that the patient underwent endodontic treatment because periapical inflammation in the mandibular left first and second molars 5 years ago. In addition, there was no cystic lesion in the mandibular left molars 5 years ago. The patient admitted that she had never returned to the dentist for follow-up in the past 5 years. The Ethics Committee of School and Hospital of Stomatology, Wuhan University gave approval for this case report. Written informed consent was obtained from the patient for publication of this case report and any accompanying images.\nThe patient underwent surgery for treatment of the cystic lesion and the fracture. Curettage of the cyst and the extraction of the third molar were performed via an intraoral approach and afterwards, open reduction and internal fixation of the fracture was carried out via an extraoral approach. The histopathology examination confirmed the cystic lesion as a radicular cyst (Fig. ). The postoperative OPG and CT show the cavity of enucleated cyst and the internal fixation of the fractures (Fig. ).\nAt the 4-month follow-up visit, the patient's symptoms resolved completely, and the OPG revealed that the fracture healed already, combing with bony healing in the cavity of enucleated cyst (Fig. ). At the 1-year follow-up visit, the patient did not have any complaints, and the radiographs indicated the consolidation of the fracture without recurrence of cyst (Fig. ).
A 77-year-old woman visited our hospital with no history of trauma and complained of right thigh pain. Although her pain initially occurred late during activity and was resolved with rest, it finally became more constant until it was present at rest (Table ). She received right cementless bipolar hip hemiarthroplasty (BHA) in her local hospital 18 years earlier due to a right femoral neck fracture and had a history of 2-year alendronate (35 mg/wk) use. Radiography showed a radiolucent space surrounding the distal portion of the stem with reactive lines, accompanying localized lateral cortical thickening and an obscure transverse lucent line just above the stem tip (Figure ). At 3 years prior to this episode, she visited our hospital for left-sided sciatic pain with magnetic resonance imaging only indicating mild spinal canal stenosis of the L5-S1. She presented no pain or other symptoms in her right leg during that time. Therefore, we accessed the conventional anteroposterior radiographs of the hip joint taken at the time of the lumbar magnetic resonance imaging scan. Reactive lines and radiolucencies were already apparent, and there were no signs of progressive component migration during these past 3 years (Figure ), even though no beaking of the lateral cortex was observed in this earlier radiograph (Figure ). One possible cause of her severe thigh pain could be aseptic loosening of the stem as plain radiography showed a periprosthetic zone of radiolucency around the bone-implant interface. Another possibility was that the incomplete stress fracture with specific features reflective of atypical femoral fractures (AFFs) was responsible for the painful hip arthroplasty. Tam et al reported that comparing the pattern of uptake with previous bone scintigraphy studies is important to determine loosening. A higher intensity around the prosthesis compared with the previous studies may raise the suspicion of aseptic loosening. Conversely, should no increased uptake on delayed bone scintigraphy exist, significant loosening is unlikely. Furthermore, acute fractures or unstable and unfused old fractures were reported to show increased uptake on bone scintigraphy. Furthermore, we examined her right thigh with [18F] NaF PET/CT to investigate the cause of the pain. NaF PET/CT demonstrated focal intense tracer uptake at the beaking of the lateral cortex; however, only mild radiotracer uptake was observed even in the radiolucent space around the distal portion of the stem (Figure ), suggesting that the cause of the pain was probably due to AFF-like incomplete stress fracture. Hence, we performed open reduction and internal fixation (ORIF) using a locking plate. We chose a reversed locking compression plate for the distal femur (LCP distal femur plate; Depuy Synthes) for the contralateral side and an LCP cable system (Figure ). Alendronate treatment was discontinued and teriparatide (20 µg/d) was started following surgery. We prescribed a rehabilitation program including immediate, gradually augmented weight-bearing, and she reported alleviation of pain a couple of weeks following surgery. Unfortunately, bone healing such as callus formation or fracture line disappearance could not be confirmed on radiograph due to overlapping incomplete fracture site and LCP plate. One and half year following surgery, we reperformed NaF PET/CT scan again, and it showed a markedly reduced NaF uptake at the lateral cortex near the distal stem tip (Figure ). Unexpectedly, the high intensity around the distal screws was observed in contrast to low uptake along the distal stems. In addition, anteroposterior radiography of the right femur performed simultaneously, also showed radiolucent zones around the distal screws (Figure ). These findings indicated that hardware micromotion may have induced high bone metabolic activity, resulting in high concentrations of NaF on PET around the distal screws. She is currently (2.5 years postoperatively) under observation and ambulates independently with no complications (Table ).
34 years old patient, Gravida 6 Parity 3, previous 2 miscarriages (18 weeks & 12 weeks), was seen first at 23 weeks 4 days of pregnancy. She had undergone previous 3 cesarean sections and an evacuation of retained products of conception by curettage in 2013 for partial hydatidiform mole. At 27 weeks 5 days, she was admitted for vaginal bleeding. On further evaluation by ultrasound (), the diagnosis of placenta percreta was made (later confirmed by MRI). At 29 weeks, she had constipation with 2 episodes of urinary retention and she was put on continuous bladder drainage. She developed urinary tract infection and treated with appropriate antibiotics based on culture sensitivity. She continued to have repeated bouts of vaginal bleeding of varying amounts and severe constipation from 31 weeks of gestation.\nAt 32 weeks 4 days, patient underwent cystoscopy, which had shown signs of cystitis with no definite infiltration. She underwent classical cesarean section under combined anesthesia (Epidural + General). The umbilical cord was tied near insertion and the placenta was left in situ because there was no spontaneous separation. Then, the uterus was closed. Prophylactic temporary bilateral internal iliac artery balloons were inserted and inflated earlier. Uterine artery embolization was performed post cesarean section and selective angiograms confirmed adequate positioning. The patient required large volume of particles and still had incomplete embolization with the lower part of the uterus still showing some unblocked branches on both sides.\nPost-operatively, she was transferred to labor ward and within 4 hours, she developed clinical features of pulmonary embolism (PE). Some of her symptoms included drop in O2 saturation to 81%, tachycardia, chest pain, peripheral cyanosis, and signs of respiratory distress. Then, she was transferred to ICU and was initiated on heparin infusion. On chest X-ray, she had no atelectasis, pneumothorax, or pleural effusion. An immediate CT scan did not show any PE. There was no Doppler evidence of venous thrombosis in the femoral and popliteal venous systems. Later on day 1 post-operative, she had focal patchy consolidation left base and was started on parenteral meropenem, linezolid and fluconazole for the next 5 days. She had two consecutive CT scans on post-operative on days 2 and 3, which were negative. On ECG, there was right heart strain. She was now on enoxaparin. On the post-operative day 5, she was prescribed parenteral piperacillin-tazobactam for 5 days and she was shifted out of ICU next day. She had 500ml vaginal bleeding on the 9th post-operative day. 2 units PRBC were transfused. She was switched to oral cefuroxime and metronidazole and planned to continue on long-term low dose antibiotic. On post-operative day 11, she received methotrexate. On day 12, the MRA had shown the placenta was still enhancing with some areas of infarct and separation, fluid collection in the uterine cavity (present from day 1 post op, not increasing), with large ovarian veins, hugely distended and extensive pelvic varices, R>L, extensive collaterals. Her CRP was 12.7 mg/L.\nOn post-operative day 13, she underwent total abdominal hysterectomy. Intraoperatively, the bladder was densely adherent, drawn up, with large vessels in the broad ligament. The lower segment was bulging due to the presence of the placenta. The uterus was about 24 weeks’ size with adherent omentum. There was 100 mL of old blood in the cavity and the placenta was partially infarcted. The total blood loss was 2000 mL.\nPost-operatively, she was in ICU for 2 days receiving anticoagulation treatment (bridging treatment with enoxaparin + warfarin) and patient controlled analgesia. She had a bout of severe cough on day 4 and loose motions on day 5. She was diagnosed with vault hematoma, which was retro-vesical, about 120 ml in volume, treated conservatively. On day 10 she had been discharged from the hospital.\nShe presented to the ER on the post-operative day 16 and was diagnosed with chronic pulmonary embolism. Patient had a pulmonary embolus within the right middle lobe pulmonary artery; areas of sub-segmental embolus within the right lower lobe pulmonary arteries. She had no pleural effusions or consolidation and no mediastinal lymphadenopathy. She was readmitted for 4 days. She was started on therapeutic enoxaparin + warfarin. She was continued on 6 mg warfarin for 4 weeks after discharge.
Our patient is a 42-year-old male who initially presented to our clinic with a 5-year history of intermittent right knee pain.His medical history was significant for gout, managed with allopurinol, and he had previously undergone a nephrectomy to treat chronic renal disease. He denied any prior trauma to his knee but has worked in an oil field for his entire adult life. He reported a 2-month history of acute worsening of his pain as well as catching and locking symptoms that preceded his initial orthopedic assessment. He presented to our emergency room before orthopedic assessment due to sudden onset of right knee pain; blood tests revealed a uric acid level of 7.4 (normal) and a knee aspiration by the emergency room physician showed the presence of negative birefringent crystals. Pre-operative clinical examination of his right knee examination was significant for an effusion with medial greater than lateral joint line tenderness to palpation. His active and passive range of motion was 20—°, limited by pain. He had pain medially with McMurray testing and was ligamentously stable. He had no palpable gouty tophi within the knee and there was no evidence to suggest gouty tophi present anywhere else on his body. Weight-bearing knee radiographs showed mild tricompartmental degenerative changes with osteophyte formation. Magnetic resonance imaging (MRI) of the right knee displayed tears along the posterior horn of both menisci and mild thinning of the articular cartilage of the medial and lateral femoral condyles and mild osteophytoses. Given the patient’s presentation and new onset of mechanical symptoms, a diagnosis of symptomatic meniscal tearing in the setting of chronic degenerative osteoarthritis was made. The possibility of a gout flare was also discussed but thought unlikely due to the combination of his new mechanical symptoms with a negative prior history of knee involvement. After counseling of management options was completed, a trial of conservative care was initiated. He was given a combined injection of a hyaluronic acid viscosupplementation and corticosteroid to treat his pain and inflammation and a guided course of physical therapy was started. Topical anti-inflammatory medication was also prescribed as oral NSAIDs were contraindicated post nephrectomy. At his 4-week follow-up appointment, he reported improvement in pain but was still experiencing mechanical symptoms and pain limiting his ability to adequately perform his job. His right knee examination showed improved range of motion but continued swelling and tenderness centered over the joint lines. At this point, the decision was made to proceed with arthroscopic treatment with the goal being to return him to full duty and normal activities. During routine arthroscopy, extensive gouty tophi were noted diffusely throughout the knee including widespread involvement of both femoral condyles and menisci. The meniscal lesions were on the surface of the menisci as well as within the meniscal tears (, , , , , ). Furthermore, noted were the expected posterior horn medial and lateral meniscal tears with tophaceous deposits within the tears. Partial meniscectomies were completed, followed by extensive debridement and excision of the tophi from the articular surface and menisciutilizing a standard arthroscopic shaver and a curved liberator typically used during shoulder arthroscopy. At his initial 10-day post-operative visit, he reported significant pain relief and resolution of his mechanical symptoms. Aggressive physical therapy was initiated as well as a rheumatology referral for medical management of his severe gouty arthritis. By his 6thweek after surgery, he had full range of motion compared to the contralateral side and his activity-related soreness was well-managed with over the counter analgesics, and he was returned to full duty at his job. At final follow-up at 3 months, he continued to have full range of motion with no pain or issues with while working his job in the oil field.
We present a case of 40-year-old building and construction male worker who slipped and fell from a height of three (3) meters and sustained a deep penetrating wound on the right side of the anterior neck a week prior to presenting at our facility. He was apparently working from the above height when he slipped and fell on a sharp piece of iron rod which penetrated deep into the right anterior neck. He quickly pulled the sharp iron rod out when he got up from the floor. According to him, the bleeding was not profuse and stopped when he arrived at the local hospital to search for remedy (). He did not have hemiplegia, paraplegia, or quadriplegia when we saw him. He is not known to be hypertensive. He did not take alcohol prior to the fall although he takes alcohol occasionally. He had a left femoral fracture at the age of 24 and a right femoral fracture at the age of 32; both incidences were operated on successfully. On examination at our facility we saw a middle aged man who was conscious and alert but however acutely ill with his neck fixed in cervical collar. General as well as systemic examination did not yield much. All the systems where grossly normal. Neurological examination revealed normal pupils which reacted normally to light. Cranial nerves examination was unremarkable. Power on four limbs as well as reflexes was normal. Digital rectal examination revealed a normal spinster tone. Routine laboratory as well as other ancillary (ECG, CXR, etc.) investigations were normal.\nNeck CT-scan done at the local hospital revealed C2-C4 transverse process fractures on the right side, fracture at the right lamina of C3, and right common carotid artery dissection. CT-scan of the head showed no abnormalities (Figures and ). Explorative three-dimensional reconstruction plain and enhanced scan imaging of the cervical spine, chest, and abdomen done at our facility revealed two segmental stenoses of the right common carotid artery with very pale V1 and V3 segment of the right vertebral artery as well as blockage at V2 segment (Figures –) as well as fracture at the right lamina of C3 and C2-C4 transverse processes with free bone fragments and peripheral soft tissue swelling (Figures –). The skin at the right anterior cervical region is discontinuous, with adjacent soft tissue swellings and gas accumulation. The bilateral carotid artery sheath lymph nodes slightly enlarged. At the upper lobe of the right lung there were multiple calcifications, some of which were adjacent to the pleura. There was also slight thickening of the left pleura. The heart was not enlarged but we observed slight accumulation of gas in the anterior mediastinum. Multiple low-density lesions were seen in the liver which we think are constant cysts. A working diagnosis of right common carotid artery dissection with C1-C4 fractures was made.\nAfter preoperative education and counselling of the patient as well as the relatives, surgery was scheduled the next day. Intraoperative cerebral angiography showed right carotid artery dissection and right vertebral artery occlusion. There was some reparation at the distal end of the right vertebral artery. The left vertebral artery was however normal. We introduced the guiding catheter guide wire to the proximal end of the right common carotid artery with continued infusion of heparinized saline, after which we introduced a guide wire with a Cordis stent (10 ∗ 60mm) to completely cover the right common carotid artery dissection site with stenosis and released the stent gradually until it completely filled the stenosis area (Figures –)). We delivered contrast agent into right common carotid artery to make sure it was patent before removing the guiding catheter followed by withdrawal of the femoral arterial sheath. Control contrasted angiograph done revealed stenting was successful (Figures and ). The patient recovered markedly and was discharged home a week after. Scheduled outpatient visit every 6 months for 2 years revealed no neurological complications.
An 11-year old female came to the clinic after her parents complained of missing lower left teeth. No pain or previous discomfort was reported. The overall patient’s dental and physical health was good with non-specific general medical history and no contra-indication to dental treatment. A signed informed consent from the patient’s mother was obtained before the patient participated in the study.\nExtraoral examination revealed a symmetric face with no deficit in the lower left part of the face. Intraoral examination revealed a Class II incisor relationship and a Class II molar relationship from the right side in a late mixed dentition. At the left side, the first mandibular molar was clinically absent, and the overlying mucosa was normal in color and texture. The adjacent deciduous second molar had a large amalgam restoration with no signs of secondary caries.\nThe panoramic radiographic examination (PanRad) revealed the presence of six permanent molars in the upper jaw and five permanent molars in the lower jaw (). From the size of the teeth, the stage of the root formation, the location of the teeth buds, and the angulation of the impacted molar, it was assumed that the impacted tooth was the first mandibular molar, and the adjacent tooth bud was the mandibular third molar. A well-circumscribed unilocular radiolucent lesion in the body of the mandible was noticed, associated with the crown of the vertically impacted mandibular left first molar. The roots of the impacted molar were completely developed with closed apexes. The cephalometric X-ray confirmed a skeletal Class II malocclusion (). The clinical diagnosis was dentigerous cyst associated with the impacted molar.\nThe main objectives of the treatment plan were to eliminate the cystic lesion and establish a functional occlusion. The latter should include expansion of the upper arch, leveling and alignment of both arches, closure of any residual spaces of missing teeth, and establishment of a functional molar relationship. After taking into consideration the age of the patient, the missing mandibular left molar and her occlusal status, a combined surgical-orthodontic approach was decided upon. Both lower deciduous molars were extracted under local anesthesia. Two metal bands were cemented to the maxillary first permanent molars, and a Quad-Helix appliance was inserted in the palatal tubes of the bands. Eight months later, all mandibular permanent premolars had fully erupted, and a new panoramic examination showed that the impacted molar was still at the same height ().\nFixed orthodontic appliances (MBT, Dynaflex®, 10403 International Plaza Drive, St.Ann., MO, USA) were placed in the mandibular arch. After three months of active orthodontic treatment, initial alignment was achieved, and exposure of the impacted tooth was performed. A muccoperiostal flap was raised under local anesthesia, and a communication was established between the cystic cavity and the oral cavity. A specimen of the cyst was sent for biopsy. The crown of the impacted tooth was exposed, and a conventional molar attachment was bonded on the buccal side perpendicular to the long axis of the second molar with a self-etching adhesive. To upright the second molar, elastic traction was applied.\nHistopathological examination confirmed the diagnosis of a dentigerous cyst.\nAfter two months of orthodontic extrusion, radiographic examination showed a significant reduction in the size of the cystic cavity and a more favorable position of the first molar (). Orthodontic traction continued for 16 more months, while the upper dental arch was also treated with fixed orthodontic appliances. Eighteen months after the surgical procedure, the first molar had taken a very favorable position in the dental arch, thus debonding of the fixed appliances was decided due to the patient’s unwillingness to continue with the fixed appliance treatment. The previously impacted molar was left to erupt spontaneously, while the upper left molars were stabilized in order to prevent over-eruption ().\nThe patient reported for follow-up appointments every two months, and finally, 14 months after the debonding of the fixed appliances, the molar had fully erupted (). A stable occlusion was achieved, both arches were well aligned, and there were no residual spaces.\nRadiographic examination revealed an almost vertical position of the mandibular first molar and normal trabecular bone surrounding the previously impacted tooth ().
A 26-year-old female patient with chronic RA visited the hospital because of chronic joint pain in both knees and limited knee joint motion induced by severe flexion contracture and valgus deformity. She had RA diagnosed 10 years ago and had been undergoing conservative treatment. The pain in both knees was aggravated since 5 years ago, and a severe flexion contracture deformity developed gradually, resulting in inability to walk. The range of motion (ROM) of the right and left knees was limited to 85°–135° and 90°–130°, respectively, which was accompanied by a valgus deformity. Severe flexion contracture and valgus deformity were observed on the plain radiographs and scanogram (). Both knees had severe joint destruction and deformity caused by RA, which was the indication for TKA. Operation was decided for the left knee first due to the presence of more severe pain and greater flexion contracture deformity than the right knee. Although preoperative application of serial casting and physical therapy may be more effective for severe flexion contracture in both knees, we decided to perform TKA and proceed with serial casting and physical therapy postoperatively to correct the remaining flexion contracture. Furthermore, considering the possibility that she might need additional revision knee arthroplasty in the future, as well as her young age, the goals of surgery were to perform a proper soft tissue procedure and minimal osteotomy for one-third correction of the flexion contracture deformity.\nSurgery was performed under spinal anesthesia and the 90° flexion contracture of the left knee measured in the preoperative test was confirmed in surgery (). Using the general medial parapatellar approach, the hyperplastic synovium was removed. Femoral posterior condyle osteophyte removal was carried out and a proper posterior soft tissue release was performed. Then, additional distal femoral resection of more than 2 mm was performed to treat the flexion contracture by widening the extension gap. The posterior cruciate ligament (PCL) was resected to balance the flexion/extension gap and a PCL-substituting implant (e.motion; B. Braun-Aesculap, Tuttlingen, Germany) was inserted.\nImmediately postoperatively, plain radiography of the left knee was performed: the anteroposterior and lateral views of the left knee joint revealed that an additional extension of approximately 29° was attained (). Quadriceps setting exercise was begun immediately postoperatively, and serial casting and physical therapy were performed to correct the remaining flexion contracture deformity. Gradual stretching and correction of the posterior capsular structure of the knee joint was also initiated. Three months after the operation, the left knee pain was considerably relieved and full extension was achieved with 0°–100° of ROM (). Six months after the operation, the patient underwent a TKA for the right knee using the same method, which was minimal ostectomy for one-third correction of the flexion contracture deformity, using the same implant. Immediately postoperatively, anteroposterior and lateral radiographs of the right knee joint revealed an approximately 17° of increase in knee extension (). Postoperative treatment was done in the same way as in the opposite side. Three months after the operation, the knee joint pain was considerably relieved and the ROM of the right knee was between 0° and 100°, indicating restoration of full extension. During the follow-up period, full extension was achieved in both knees, and the thigh quadriceps muscles and hip and trunk muscles rapidly gained strength by performing standing exercises. Sixth months after the surgery, the patient was able to walk independently without using crutches, and no abnormalities were observed on anteroposterior and lateral radiographs of the knee joints ().
A 36-year-old China male, who was previously healthy, was found to have a left-sided abdominal mass on physical examination. An abdominal ultrasound was preformed and revealed the presence of a large mass, The mass was located inferior to the kidney and measured, approximately 15.5 × 12.3 × 5.2 cm, The patient also had portal vein expansion (inner diameter of about 18 mm) a dilated the splenic vein (inner diameter about 16 mm). An abdominal CT examination confirmed the presence of a large and soft tissue mass in the left lower abdomen and spleen enlargement. CTA demonstrated that the tumor was located in the free abdomen below the left kidney with abundant blood supply and periphery in focus. The nourishing artery branched directly from the abdominal aorta under the celiac axis and the reflux vein was grossly distorted. The latter divided into 2 to 3 branches which drained into the portal vein posterior to the kidney. The portal vein was also widened. The branches of the splenic veins were distorted and expanded into the tumor.\nAbnormalities were not seen in blood routine examination, biochemistry indexes, and the examination of coagulation routines. There were also no obvious abnormalities in the examination of electrocardiogram and chest radiograph.\nLaparotomy was performed with general anesthesia and the mass was completely excised. In the operation, a solitary hemorrhagic tumor of 15.5 cm∗12.3 cm∗5.2 cm was found in the left lower abdomen. The surface of the mass was dark red and with a several large blood vessels. Most of the blood vessels were veins and the thickest one was about 1 cm in diameter. One of artery connecting to the abdominal aorta was considered as a nourishing artery according to CTA examination Several large distorted veins merged into the portal vein posterior of the kidney and spleen. The portal vein had a significantly wider lumen.\nIts macroscopic inspection showed that the cut surface of the tumor was dark red with calcified areas. The nuclei of the tumors were markedly heteromorphic, with thick chromatin. The markers BCL-2 (+), CD34 (+), CD31 (+), and Vimentin (+), were positive, and the mitotic count was low. The proliferative index counted by Ki67 was weakly positive. Furthermore, elastic fiber staining demonstrated an arteriovenous short circuit inside the tumor. The final diagnosis for these manifestations was solitary fibrous tumor. Recovery was complete without a need for further treatment. The patient underwent abdominal ultrasound 10 months post-operation without recurrence, and the portal vein and splenic vein dilatation were alleviated (the inner diameter of the portal vein was about 17 mm). The symptoms of portal hypertension were also relieved.
We describe the case of a 27-year-old white woman who had experienced an emergency caesarean delivery at 39 weeks for fetal distress with no postpartum complications. As part of our ongoing study “Vaginal delivery after caesarean section”, she underwent saline contrast sonohysterography 6 months after the caesarean section. The caesarean scar had a small indentation and the remaining myometrium over the defect was 7.5 mm (Fig. ).\nIn the current pregnancy, she had a dating scan at around 11 weeks with no remarks. She came for a transvaginal ultrasound examination at around 13 weeks as part of our study. This scan revealed a duplex pregnancy with one viable intrauterine fetus with normal anatomy and placenta located high on the anterior wall and a small gestational sac (8 mm) with a yolk sac without embryo was located in the caesarean scar (Fig. ). There was no extensive vascularity surrounding the sac. One corpus luteum was found in each of the two ovaries. She was asymptomatic.\nShe was informed that not enough evidence existed to advise a specific management of this condition. After discussion with her and her husband, expectant management was chosen with a new ultrasound examination after 5 weeks.\nShe came to our ultrasound department at 18 weeks, 22 weeks, and 30 weeks of gestation. She remained asymptomatic. The ectopic gestational sac was not visualized with transvaginal or transabdominal scans at the 18 weeks examination (Fig. ). The niche in the scar and the thickness of the thinnest part of the remaining myometrium appeared unchanged at all visits. The intrauterine pregnancy developed normally with no signs of abnormal placentation. At 30 weeks of gestation the ultrasound appearance of the scar area did not indicate any contraindications for vaginal delivery. The thickness of the lower uterine segment (LUS) was 4.9 mm (Fig. ). In agreement with our patient, vaginal delivery was planned. The staff of the labor ward was fully informed.\nShe was admitted to the labor ward with irregular contractions in week 37 + 0. Her cervix dilated to 3 cm with no further progress. Due to that oxytocin augmentation was administered for 3 hours. The duration of active labor was 6.5 hours. A healthy male neonate weighing 2985 g was delivered, with Apgar scores 9–10 at 1 and 5 minutes and umbilical cord pH 7.27. The placenta delivered spontaneously and total blood loss was 250 ml. The postpartum period was without any complications, and she was discharged home the next day.\nAt a follow-up visit 6 months postpartum, saline contrast sonohysterography showed no signs of the previous CSP, and the remaining myometrium over the hysterotomy scar defect was 5.7 mm (Fig. ).\nEthical approval for the ongoing study was obtained by the Ethics Committee of the Medical Faculty of Lund University, Sweden, reference number 2013/176. Our patient has given permission for publication of this case report in a scientific journal.
A 40-year-old man, a temple priest by profession, presented with stiffness in his right knee for eight years. He had a motor vehicle accident (MVA) in 2011 when the auto-rickshaw in which he was traveling toppled and he sustained a fracture of both bones of his right forearm, fracture of the shaft of the right femur as well as basicervical fracture of the neck of right femur. He had undergone plating of both bones of the forearm, dynamic hip screw (DHS) for the neck of femur fracture and supracondylar intramedullary nailing for fracture shaft of the femur. The femoral neck and forearm fractures went on to heal uneventfully. However, he developed an infection of the femoral shaft fixation with a persistent discharging sinus from the thigh for six months after the surgery. The fracture went into non-union but the sinus healed and hence he had another surgery for bone grafting. The femoral shaft fracture united six months after bone grafting and one year after MVA. At this visit, he presented with stiffness in the knee and a short right lower limb.\nOn examination, he had a puckered scar on the lateral aspect of his thigh indicating a healed infected sinus, in addition to two surgical scars - one proximal for the DHS and the other distal for the interlocking screw proximally and bone graft surgery. The knee was stiff with flexion of 30 degrees from flexion deformity of 10 degrees, with a very firm endpoint. The right knee examination revealed laxity of the posterior cruciate ligament. Anterior cruciate ligament and both collaterals appeared intact. There was no distal neurovascular deficit. The right thigh was short by two centimeters.\nA computed tomography scanogram of the right thigh and knee showed a united femoral shaft fracture with no evidence of loosening of the implant or osteomyelitis. The DHS was intact with no evidence of loosening (Figure ).\nTwo-dimensional computed tomography scan of the femur revealed heterotopic bone formation in the knee just posterior to the infrapatellar fat pad region at the tip of the nail (Figure ).\nArthroscopy of the knee revealed heterotopic bone formation inferior to the lower end of the nail, adherent to the tibia (Figure ).\nThe fragment was impinging on the anterior aspect of the femoral notch, where a soft-tissue nodule arising from the femur had formed (inverted cyclops) (Figure ).\nThe heterotopic bone, as well as the inverted cyclops nodule, were excised. Arthroscopic release of fibrous adhesions followed by gentle manipulation of the knee was done. Supracondylar nail was removed after the arthroscopy and manipulation. A knee range of motion of 0-120 degrees was obtained. Extensive post-operative rehabilitation was given to improve the range of motion. At the last follow-up at three months, he had no flexion deformity and further flexion was 120 degrees. He did not have instability of the knee with regular daily activities and was not keen on proceeding with the reconstruction of the posterior cruciate ligament.
A 54 year old woman with OI type 1 presented in October 2014 with acute back pain without any history of trauma or fall. Computed tomography (CT) scan of the thoracic spine demonstrated a burst compression fracture of the 11th thoracic vertebra (T11), as well as a 2.6x1.3x1.5 cm soft tissue density involving the left transverse process of the 7th thoracic vertebra (T7) (). A CT-guided biopsy of the T7 lesion revealed a heavy infiltration by kappa restricted CD138 expressing plasma cells. Further investigations confirmed the diagnosis of kappa light chain myeloma with lytic lesions involving the right parietal bone and right iliac bone, normal renal function and 30% light chain restricted plasma cells in the bone marrow. A radiographic skeletal survey demonstrated a radiolucent line at the left femur diaphysis with localized periosteal reaction compatible with an incomplete insufficiency fracture, consistent with the diagnosis of AFF (). There was no involvement of the contralateral femur, but both femurs were noted to be significantly bowed. The cortices were not thought to be thickened. Correlation with radionuclide bone scan demonstrated focal increased radiotracer uptake at the left femur fracture site. The patient denied thigh or groin pain. Orthopedic consultation was sought and the consultant recommended against prophylactic femoral nailing in view of the absence of symptoms and of the concurrent diagnosis of MM that might negatively affect the post-operative course.\nThe patient had been diagnosed with OI type 1 in early childhood and never received bisphosphonates until the age of 40 years, when alendronate was initiated for ongoing bone loss at the time of menopause and a patellar fracture. Calcium and Vitamin D supplementation was begun, and low dose transdermal estrogen (estradiol 0.025 mg/day twice a week) was prescribed to alleviate menopausal symptoms. Alendronate was stopped after 10 years of continuous therapy since her bone density had stabilized and the patient had remained fracture free. Four years later, in March 2014, the patient sustained a right scapular fracture following a fall. Risedronate was initiated but was discontinued in October 2014, upon diagnosis of MM and the discovery of the left diaphyseal AFF.\nThe patient subsequently underwent chemotherapy with cyclophosphamide-bortezomib-dexamethasone (CyBorD), resulting in clinical, biochemical and hematological remission. Intravenous pamidronate was administered simultaneously to reduce myeloma-related skeletal complications. In view of the presence of AFF, dosage and frequency of pamidronate was reduced from 90 mg per month to 60 mg every 2 months, and planned for a shorter duration of 1 year instead of 2 years. Dexamethasone was also reduced from 40mg to 10mg per week given her overall increased fracture risk. Pamidronate was stopped after 8 months of treatment at the patient’s request. Bortezomib administered every two weeks was continued as maintenance therapy for an additional 24 months. In addition, the patient received radiation therapy to the T7 lesion for pain control.\nAt follow-up in July 2017, the patient remains on maintenance bortezomib, and free of fractures and detectable myeloma. On questioning, she does report occasional low-grade pain in the left groin upon walking or standing. Radiograph of the left femur () shows no progression of the small fracture line and persistence of the endosteal-periosteal reaction. Laboratory results reveal suppressed bone turnover markers 20 months following discontinuation of pamidronate ().
A 59-year-old male underwent uneventful DDLT for decompensated hepatitis B-related liver cirrhosis with a MELD score of 19. The transplant surgery and postoperative course were uneventful. He was discharged on the 11th posttransplantation day with stable liver function. Immunosuppressive protocol was as per standard institution protocol. The patient was admitted after 6 weeks in an emergency department with hypotension, vomiting, and altered sensorium associated with oliguria. Liver functions were grossly elevated, and he had a systolic hypotension of 70 mmHg. Emergency abdominal ultrasound scan showed mild hepatomegaly and an echogenic thrombus in the retrohepatic IVC near the suprahepatic anastomosis () which was confirmed by computed tomography (CT) angiography that also revealed renal vein and iliac vein thrombosis as an incidental finding.\nThe patient was admitted in ICU and started on anticoagulation therapy, and a digital subtraction inferior venacavogram was done. This showed a focal severe stenosis approximately 70% in the inferior vena cava at the level of the T12 vertebra. There was an associated thrombus 6 × 3 cm within the retrohepatic and suprahepatic inferior vena cava with complete cut-off of the inferior vena cava 3 cm proximal to its junction with the right atrium. Intravascular thrombolysis using urokinase 50,000 IU was immediately instituted, and the patient was maintained on 100,000 IU/hour of urokinase infusion in the IVC with repeated mechanical thromboaspiration. The patient remained stable during the thrombolysis, and there was reestablishment of blood flow across the previously occluded part of the IVC. Post procedure abdominal ultrasound and Doppler ultrasound were done after 48 hrs, and both still showed the presence of a thrombus. Abdominal CT scans also showed severe stenosis in the suprahepatic inferior vena cava just proximal to the right atrial junction with mild to moderate ascites and splenomegaly. The patient had another angiography done which confirmed a 2 cm short segment severe stenosis of about 95% in the IVC at its junction with the right atrium (). The IVC was twisted along its long axis in this region.\nHe then underwent inferior vena cava venoplasty, and an endovascular stent was inserted whereby a balloon-mounted stent was deployed across the stenosis site to a size of 18 × 32 mmm (). Post stenting, there was improvement in the calibre of the IVC and it was untwisted (). There was no pressure gradient proximal and distal to the stenosis.\nFollowing the procedure, the patient was given low molecular weight heparin 2500 IU subcutaneously for 3 weeks. The patient made uneventful recovery and was discharged 2 weeks post stenting on warfarin with a target international normalized ratio between 2 and 2.5. The liver functions were normal at the time of discharge.\nFollowing the procedure, the patient was given low molecular weight heparin 2500 IU subcutaneously for 3 weeks. The patient made uneventful recovery and was discharged 2 days post stenting on warfarin with a target international normalized ratio between 2 and 2.5. The liver functions were normal at the time of discharge.\nSix weeks after discharge, he was reviewed and had no complaints. An abdominal ultrasound done showed no thrombus in the inferior vena cava and the rest of the abdominal organs were normal as well as his liver and renal function tests.
A 42-year-old female patient received inpatient treatment for acute pyelonephritis in 2003. In 2009, she was diagnosed with gastroesophageal reflux disease and began taking prescription medication for that condition. During a regular follow-up examination, a chest X-ray revealed a mass lesion in a portion of the left lower lung field. Chest computed tomography was then performed, and the patient was suspected of having a neurogenic tumor or bronchial cyst in the left posterior mediastinum (). The patient was then admitted for surgical diagnosis and treatment.\nAt the time of admission, laboratory tests showed no remarkable findings. Thoracoscopic resection of a portion of the posterior mediastinal mass was performed under general anesthesia in the right lateral decubitus position under single lung ventilation on the right side. The lesion was a sessile cyst-like mass fixed to the posterior mediastinum. During dissection, the cyst wall was opened and a milky-white leaking fluid was observed. No serious adhesion to the surrounding tissue was found, and the lesion seemed benign during intraoperative observation. The operation was finished after a pathology report on the resected lesion was requested. The pathology report indicated that the lesion was composed of interconnected and dilated lymphatic spaces, lined by a single attenuated layer of endothelial cells ().\nPost-surgical thoracic drainage remained high, with 420 mL of drainage on the first postoperative day. On the second day, 110 mL of the milky pleural fluid was drained. Laboratory examination of the pleural fluid revealed that the fluid was chylothorax, containing 842 mg/dL of triglycerides and 75 mg/dL of total cholesterol. Thereafter, conservative treatment was started, combining fasting and total parenteral nutrition. However, after one week of conservative treatment, the chylothorax continued to leak. More than 900 mL of pleural effusion was drained daily. The resected mediastinal mass was confirmed to be a cystic lymphangioma in the final pathology report. Considering the clinical presentation of chylothorax after the operation and the pathologic confirmation of the resected mediastinal mass, the mediastinal mass was diagnosed as a thoracic duct cyst. Seven days after the initial operation, the patient underwent reoperation to ligate the thoracic duct by video-assisted thoracoscopic surgery and talc pleurodesis under general anesthesia (). The patient ingested 200 mL of olive oil to identify the thoracic duct leaks before reoperation. After reoperation, normal meals were administered, and no chyle drainage was observed. The patient was discharged seven days after the reoperation.
A healthy 12-year-old boy was referred by an orthodontist with swelling of the palate. The patient did not have any symptoms, and he had no pain or difficulty with speech or swallowing and no history of trauma, fever, fatigue, anorexia, respiratory or gastrointestinal symptoms, or weight changes. The swelling had been present for approximately 5 years, and the dentist observed it during the regular dental control visits, but no further action was taken. The swelling had never bothered the patient. The patient used Ritalin for attention deficit hyperactivity disorder (ADHD). Upon physical examination, a 2 cm nonfluctuating, nontender, immobile, firm nodular swelling was observed with intact overlying mucosa on the left side of the hard palate in the bicuspid-molar region (). Clinical and radiographic examination of the dentition did not reveal any pathology. The patient had no palpable cervical lymphadenopathy. MRI showed a well-demarcated mass of the left side of the hard palate without bone invasion (Figures and ). Pathological diagnosis was established by incisional biopsy performed under local anesthesia; histological examination was consistent with a pleomorphic adenoma of the minor salivary glands of the palate. The tumor was excised under general anesthesia with nasotracheal intubation. The mucosa around the tumor was marked and incised with an adequate margin, followed by dissection, with removal of the whole encapsulated mass and the mucoperiosteum (). With a bur under copious sterile saline irrigation, the superficial part of the underlying bone was removed to ensure that no remnants of the lesions could cause recurrence. The defect on the palate was not reconstructed (). An iodine tampon dressing was placed, and the wound covered with a fabricated palatal acrylic plate (). The postoperative recovery was uneventful and histopathological assessment of the operation specimen confirmed the original diagnosis of pleomorphic adenoma of the palate. Examination showed mucosal tissue with a multinodular lesion consisting of two components in the stroma. There were solid fields of cells with round-oval nuclei and poorly delineated eosinophilic cytoplasm. There was also a spindle cell component located in the myxoid matrix with perivascular condensation. The tumor itself was sharply delineated (Figures –). The wound gradually healed by secondary intention, and complete healing was observed 4 months after surgery (). Follow-up 12 months after surgery did not reveal any recurrence. The patient remains under surveillance.
A 105-year-old Caucasian woman was admitted to our department complaining of diffuse abdominal pain with recurrent vomiting for a day. Her past surgical history included the repair of an incarcerated umbilical hernia 3 weeks prior and an open cholecystectomy 13 years before. Her past medical history included ischemic heart disease, atrial fibrillation with a cardiac pacemaker and hypertension. Despite her age, she was clear-minded, and she managed to fulfill her daily activities in her own home with the help of a nursing assistant. On physical examination her abdomen was distended and tympanic with tenderness in the postoperative scar near her umbilicus. A computed tomography of her abdomen demonstrated small bowel obstruction. A midline laparotomy through the previous scar revealed numerous adhesions of the small bowel. During adhesiolysis, one small bowel loop was opened and a resection with primary anastomosis was created. Her abdominal fascia was closed with polydioxanone loop and her skin was closed with tension nylon sutures.\nThere was a total eventration of her bowel through the suture line 9 days after surgery. She underwent a second laparotomy that revealed necrosis in the fascia edges. No signs of peritonitis or turbid fluid in peritoneal lavage were observed. Due to the fascial necrosis the field was believed to be contaminated. As a result, fascial edges were resected and instead of using a regular synthetic mesh, we decided to close her abdomen with a 15×10cm Permacol™ implant (Covidien) sutured sublay with prolene sutures. Her abdominal skin was closed with several single nylon sutures, leaving an interval of a few centimeters between one suture and the next, due to contamination. After the second surgery, the postoperative period was devoid of complications. As part of the conservative treatment approach, she was gradually returned to oral nutrition that was accompanied by normal bowel movements. Her abdominal skin was closed with close nylon sutures 7 days after the second surgery, and she reported feeling well, with no fever. Her abdomen was soft and non-tender without any signs of surgical site infection. Discharged later that day, she returned to her house as clear-minded and functional as she was prior to her admission to hospital.\nDuring a follow-up period of 3 years and 2 months, there was no sign of either recurrent hernia or wound contamination. The patient reported that she felt good, and she had no complaints of abdominal pain. She died, 3.5 years after surgery, at the age of 108 of cardiac arrest unrelated to the abdominal surgery. For clarification, since the report is retrospective and obviously we cannot call the patient for a follow-up examination, all the data for the follow-up was collected from medical records, as the patient was hospitalized several times due to other reasons, not related to the surgery, and underwent physical examination by a doctor.
A 10-month-old female infant of height 67 cm and weight 6.4 kg was scheduled for endoscopic third ventriculostomy under general anesthesia to treat progressive hydrocephalus. She was born via emergency cesarean section at 33 weeks of gestation and had a low birth weight (2,200 g). Her past medical history included treatment with surfactants because of respiratory distress syndrome evident at birth, and admission to the neonatal intensive care unit for prolonged ventilator care, off which she was weaned on day 38. Although self-respiration was achieved, a need for intermittent oxygen support persisted thereafter. Echocardiography at the time of birth revealed a small atrial septal defect and pulmonary hypertension, for which she had received nitric oxide treatment. She underwent a ligation operation on day 8 to treat patent ductus arteriosus. Neonatal brain sonography revealed bilateral intracerebral hemorrhage, which resolved almost completely on follow-up. Apart from the respiratory difficulty, the patient also showed persistent weakness of muscle tone and a significantly low activity level overall. Genetic and metabolic screening test results were all normal. Electromyography and nerve conduction velocity examinations yielded no definite electrophysical evidence of myopathy or neuropathy. She was finally diagnosed with congenital myotonic dystrophy via a chromosomal study which confirmed the presence of the DMPK mutation with > 1,000 repeats of CTG (). The patient's sister and mother were also tested for this gene mutation, and the results were positive.\nThe patient presented with persistent and progressive hydrocephalus caused by aqueductal stenosis evident upon magnetic resonance imaging of the brain, and was scheduled for endoscopic third ventriculostomy under general anesthesia. On physical examination, the patient had nystagmus and strabismus. Apart from hypotonia, no notable findings were made upon preoperative work-up. Her recent medication history included cough syrup prescribed for symptomatic relief of dry cough. Her head circumference at the time of admission was 49.5 cm.\nThe patient was not premedicated on the day of the operation. Prior to induction of anesthesia, a three-lead pediatric electrograph, a pulse oximeter, a non-invasive hemodynamometer, a nerve stimulator, and a bispectral index (BIS) sensor (BIS VISTA™ Monitoring System, Aspect Medical Systems, Newton, MA, USA) were placed and confirmed to be functioning. Preoperative vital signs included blood pressure of 86/52 mmHg, a heart rate of 130 beats/min, oxygen saturation rate of 98%, body temperature of 37.1℃, and sinus rhythm on the electrocardiogram. After adequate denitrogenation using 100% oxygen, anesthesia was induced with 30 mg thiopental sodium, 10 µg fentanyl, and 1 mg vecuronium. Mask ventilation employed 100% oxygen, tracheal intubation proceeded without any complications, and stomach decompression was achieved by careful suctioning using a catheter. The patient was mechanically ventilated in the pressure control mode, and end-tidal carbon dioxide levels was monitored and maintained at the range of 30-32 mmHg. Anesthesia was maintained with oxygen (1 L/min), medical air (1.5 L/min), and continuous infusion of 50-150 µg/kg/min of propofol and 0.1 µg/kg/min of remifentanil using an infusion pump (AS50 Auto Syringe Infusion Pump®, Baxter International Inc., Deerfield, IL, USA). The train-of-four ratio (TOF) was monitored throughout the operation to confirm that muscle relaxation was adequate and to guide the choice of additional doses of muscle relaxants. An additional 0.5 mg of vecuronium was given at the time of incision, because the patient showed signs of self-respiration. A TOF ratio of about 20% was maintained during operation. The BIS was maintained in the range of 40-60. Temperature was monitored using an esophageal stethoscope, and was 36.9℃ at the end of the operation. Vital signs remained stable during the course of anesthesia. The total amount of fluid administered was 40 ml of dextrose saline. The total duration of anesthesia was 2 hours and 45 minutes. At the end of the operation, muscle relaxation was reversed with 1.8 mg pyridostigmine and 48 µg glycopyrrolate. The patient regained self-respiration and a TOF ratio of over 90% was confirmed at this time. She was transferred to the pediatric intensive care unit while still intubated. The total amounts of propofol and remifentanil infused were 60 mg and 150 µg, respectively.\nIn the intensive care unit, the patient was initially ventilated in the CPAP mode with a fraction of inspired oxygen (FiO2) of 25%. Initial oxygen saturation was 97%. Two hours later, she was extubated after suctioning of the endotracheal tube, with no sign of respiratory distress. An epinephrine nebulizer was applied. When breathing room air she showed oxygen saturation levels of 92-96%. After monitoring for appearance of complications for 24 hours, she was transferred to the general ward on postoperative day (POD) 1. Vital signs were stable throughout her hospital course. Follow-up computed tomography showed that ventricle size had been reduced, and bilateral hygroma was evident. Oral intake was satisfactory and there were no signs of vomiting or regurgitation. Postoperative computed tomography of the brain confirmed that the hydrocephalus had decreased in extent. The patient was discharged on POD 3, by which time her head circumference had decreased to 48 cm. She was scheduled for outpatient clinic follow-up 5 weeks later.
A 38-year-old male presented to our outpatient clinic with the complaints of swelling and pain around the posterior of the ankle for the past 3 months. We revealed that he underwent an open repair of the Achilles’ tendon after it was cut by a piece of glass at 3 years of age. The operation report indicated that silk was utilized for the repair of the Achilles’ tendon.\nOn physical examination, a quasi-solid soft tissue mass, almost 3 cm × 3 cm in size, painful with palpation, was noted on the former incision scar of the ankle (). There was neither fistula nor drainage. Muscle power of the plantar flexion was graded as 5/5 in addition to a slightly limited range of motion in the ankle due to pain. The infection parameters, white blood cell count, erythrocyte sedimentation, and C-reactive protein were normal in the laboratory tests (8 × 103/mL and 5 mm/h, 2 mg/L, respectively). Magnetic resonance imaging (MRI) displayed a lesion of 3 cm × 3 cm × 3 cm with low signal on both T1 and T2, and the zone around the lesion demonstrated low signal on T1 and high signal on T2, consistent with granulomatosis formation with reactive tissues around (). Similarly, an ultrasound revealed a heterogeneous cystic mass with echogenic material associated with the Achilles’ tendon. Together all pointed to the possibility of a chronic infection at the ankle. Aspiration of the mass showed a serohemorrhagic fluid which had negative cultures. Due to normal laboratory results and no bacteria in the culture, our initial diagnosis was that the patient had a foreign body reaction. As a result, an operation was undertaken by the senior author based on the preliminary diagnosis, physical, and radiological examination.\nA prophylactic single dose of cefazolin administered 1 h before spinal anesthesia. The patient was put into sleep in prone position under spinal anesthesia, and a knee above tourniquet was fastened. An incision approximately 8 cm which included the former one was longitudinally made just medially to Achilles’ tendon. There was no sign of infection at the ankle. After the enough exposure, the granulomatosis tissue was excised. Suture materials were detected inside the mass (). After excision of the mass, the defect about 3 cm on the tendon was repaired with polyethylene non-absorbable sutures. At the operation, the mass was dispatched to pathology and culture. After 1-wound drain was put inside the wound, the subcutaneous tissue and skin were primarily closed. Finally, a below knee circular cast was applied with the ankle at 10° of plantar flexion.\nHistopathological examination indicated classical findings of the foreign body reaction: Hemosiderin-loaded macrophages, giant cells, and eosinophilic infiltration. No microorganism was derived from the wound culture. 3 weeks after the operation, the circular cast was removed and the patient was followed up with an air cast until the 6th week.\nAt the 12th month follow-up, the patient had no complaint and functional evaluation was excellent based on AOFAS ().
The patient was a 65-year-old male with a history of cutaneous SCC involving the right upper chest diagnosed in 2009 and initially treated with resection followed by Mohs surgery. Two years later he had a cutaneous recurrence confirmed to be carcinoma in situ. In October 2014, right axillary adenopathy was noted demonstrating poorly differentiated metastatic SCC. CT of the chest, abdomen, and pelvis showed bulky right axillary and retropectoral lymphadenopathy. Lymph node dissection in November 2014 confirmed 7 of 22 positive nodes for metastatic disease. Postoperative PET scan demonstrated mildly FDG-avid right pectoral lymph nodes believed to be reactive and a small area of increased uptake in the transverse colon of uncertain significance. Colonoscopy was recommended based on the PET scan but deferred by the patient as he began chemoradiation using radiosensitizing doses of carboplatin and paclitaxel, including the right subpectoral lymph node in the treatment field. This therapy was completed in February 2015, and subsequent imagining including PET/CT scans in August 2015 and February 2016 demonstrated low-grade metabolic uptake in the region of the right shoulder and axilla, consistent with posttreatment changes without any evidence of active disease, including no evidence of increased colonic uptake. On February 22, 2016 the patient underwent a routine screening colonoscopy, which did not demonstrate any lesion in the transverse colon, but a small area of nodular polypoid tissue within a diverticulum of the sigmoid colon was identified (Fig ). Multiple biopsies and a partial snare excisional biopsy were obtained which demonstrated SCC. These findings were confirmed on histology and immunohistochemical stains (pancytokeratin AW1/AE3 and p63 positive, CK20 negative). The subsequent management decisions were discussed at several specialized cancer centers, and it was ultimately recommended that the patient undergo local resection of the colonic metastasis, as this was the only identified site of recurrent neoplasm. Preoperative marking of the site was accomplished on repeat endoscopic evaluation with repeat biopsy confirmation of the site. In June 2016, laparoscopy with a robotically assisted low anterior resection was performed and a previously unidentified small bowel metastatic lesion was noted in the jejunum. The small bowel metastasis was resected and confirmed to be SCC with lymphovascular invasion. The sigmoid resection confirmed metastatic SCC at the base of a deep diverticulum with 3 of 4 positive lymph nodes and clear margins of resection. Postsurgically in July 2016 the patient was enrolled in a trial with pembrolizumab and is currently undergoing systemic immunotherapy. Repeat CT/PET imaging in October 2016 demonstrated unremarkable colocolonic and small bowel anastomotic sites without evidence of any new metastatic disease.
A 52-year-old woman was referred to our department with a growing calcified mass in the left shoulder. She had been previously diagnosed with interstitial lung disease and progressive systemic sclerosis eight and five years prior, respectively. Four years before admission, chest computed tomography first revealed a small calcified lesion in the sub-scapular area (). During regular follow-ups, the calcified lesion increased in size, eventually becoming a large tumor-like mass ().\nAt presentation, the patient exhibited slightly limited motion in the affected joint and complained of mild pain and tenderness when leaning against a wall. The patient had no family history of tumoral calcinosis and no history of trauma, with the exception of a previous thoracoscopic lung biopsy of the right lower lobe. On physical examination, no skin lesions were noted on the body surface, but the mass was palpable on the neck and back. Laboratory test results revealed that serum calcium, phosphorus, and vitamin D values were within normal limits. Autoantibody test results were positive for antinuclear antibodies and negative for anticentromere antibodies. X-ray imaging of the chest revealed a large, lobulated, and trapezoidal calcific plaque extending from the tip of the left scapula to the lower neck ().\nWhen the patient was referred to us, she was being treated with a steroid (methylprednisolone, 2 mg) and an immunosuppressive drug (azathioprine, 100 mg) for interstitial lung disease. However, the patient received no specific therapy to control the progression of the calcification in the shoulder. Although the pain and disability associated with the affected joint were not severe, we surgically excised the chest wall mass because the mass continued to grow for several years, showing a tendency for more rapid growth in the years immediately before the decision to perform a surgical excision. This was particularly problematic because the patient was completely dependent on a wheelchair due to polio-induced paralysis, and therefore, the preservation of arm function was essential for maintaining her quality of life. Moreover, we were concerned that further growth of the tumor was likely to produce skin ulceration or compression of the brachial plexus and axillary vessels, which could potentially lead to psychological trauma to the patient and the ultimate loss of shoulder function.\nIn the operating room, the patient was positioned in a semi-prone position, and a skin incision approximately 15 cm in length was made along the medial border of the left scapula. The mass was located between the rib cage and the scapula, and was tightly attached to the first and second intercostal muscles. The mass was carefully dissected without damaging the neurovascular structures and completely excised (). Pathological examination of the resected tumor revealed calcifications consistent with tumoral calcinosis ().\nThe patient was discharged on the sixth postoperative day without any complications. In outpatient follow-up appointments, she appeared well, and no recurrence was observed on a computed tomography scan 10 months after the surgery ().
A 47-year-old man with a history of acute Stanford type A dissection requiring open ascending aortic repair and subsequent need for redo aortic valve replacement presented to vascular surgery clinic with aneurysmal degeneration of a residual chronic type B aortic dissection. His prior repair consisted of a Bentall with hemiarch without need for total arch replacement, and there was no evidence of residual arch dissection at presentation. The anatomy of the type B dissection was as follows: a large proximal entry tear distal to the left subclavian artery (, A), a distal re-entry tear proximal to the celiac axis (, B), and a maximum aneurysm diameter of 6.2 cm. The dissection extended through the visceral segment into the right common and left external iliac arteries, with the superior mesenteric, renal, and inferior mesenteric arteries arising off of the true lumen and the celiac artery arising off of the false lumen. The aneurysm was limited to the descending thoracic aorta, with adequate access vessels and a proximal seal zone in native, nondissected aorta of at least 20 mm in length. The proximity of the distal re-entry tear to the origin of the celiac artery (<5 mm) raised concern about the risk of a stent-induced new entry tear as well as possible dissection into the celiac artery. Therefore, the decision was made to land the endograft in the true lumen cephalad to the distal re-entry tear. In the midthoracic aorta, the true lumen appeared to be either severely compressed or occluded on preoperative computed tomography angiography (, C). During the initial TEVAR attempt, a short-segment intervening occlusion of the thoracic aorta was confirmed on diagnostic aortography. Unsuccessful attempts were made to traverse the short-segment occlusion with hydrophilic guidewires and catheters. This occlusion was located in zone 3 over a short distance, and the true lumen in zone 4 was preserved by retrograde flow from the distal re-entry tear (, A). Because of the patient's comorbid disease burden (chronic kidney disease, heart failure, obstructive sleep apnea, atrial fibrillation, obesity), two prior sternotomies, and prior aortic surgery, an endovascular approach was favored.\nThe procedure was performed under general anesthesia with cerebrospinal fluid drainage, arterial pressure monitoring, bilateral percutaneous ultrasound-guided common femoral arterial access with ProGlide preclose percutaneous closure (Abbott, Santa Clara, Calif), and systemic heparinization. Through the left-sided access, a hydrophilic Glidewire and Glidecath (Terumo, Somerset, NJ) were used to select the true lumen to the level of the occluded true lumen proximal to the celiac artery (, A). Through the right-sided access, the false lumen was selected through the natural fenestration proximal to the celiac artery and confirmed with intravascular ultrasound (IVUS). The natural fenestration was balloon dilated with 16-mm XXL (Boston Scientific, Marlborough, Mass) and 25-mm Tyshak (B. Braun Medical, Bethlehem, Pa) balloons. However, it was determined that there was inadequate length to land an aortic endograft in the true lumen between the fenestration and the origin of celiac artery. Therefore, from the left-sided access, a Rösch-Uchida transjugular intrahepatic portosystemic shunt kit (Cook Medical, Bloomington, Ind) was advanced through the true lumen up to the level of the most proximal portion of the reconstituted true lumen in the midthoracic aorta. Under IVUS guidance (right-sided access), the aortic dissection septum was crossed using the Rösch-Uchida (left-sided access). Wire access was thus established from the common femoral true lumen to the level of the newly created aortic fenestration, across the dissection septum into the false lumen in the mid-descending thoracic aorta, through the proximal entry tear back into the true lumen at zone 3, and into the aortic arch. This was confirmed with angiography and IVUS. The newly created septal fenestration was balloon dilated with a 16-mm XXL balloon (, B), with post-septoplasty angiography demonstrating opacification of both lumens (, C).\nNext, three sequential Gore C-TAG devices (W. L. Gore & Associates, Newark, Del) were deployed, effectively excluding the aneurysm and bypassing the occluded portion of the aortic true lumen and re-establishing in-line true lumen aortic circulation (37-mm × 10-cm proximal descending thoracic aorta, 37-mm × 20-cm mid-descending thoracic aorta, 37-mm × 20-cm distal descending thoracic aorta). The distal portion of the most distal device that crossed the newly created aortic fenestration was gently dilated using a 25-mm Tyshak balloon. Completion angiography demonstrated patency of the stent graft, preservation of arch and visceral vessels, and exclusion of the aneurysm without evidence of endoleak including any retrograde flow that might suggest a type IB leak (, D and E). IVUS was then performed to evaluate the ostia of the mesenteric vessels, which were free from obstruction. Wires and catheters were removed, access vessels were closed without complication, and heparin was reversed with systemic protamine administration. The patient was extubated, with normal neurologic examination findings on conclusion of the procedure, and he was discharged home on postoperative day 4. On follow-up computed tomography angiography performed at 1 week, 1 month, and 6 months postoperatively, the stent grafts were in good position without migration (, A) with decreased aneurysm sac size from 6.2 cm to 5.6 cm (, B and C) and no evidence of a type IB endoleak. There was an intercostal artery-based type II endoleak (, D), but the endograft at the distal seal zone had expanded from 15 mm to 17 mm, suggesting remodeling of the chronic aortic dissection septum (, E and F).
A 34-year-old Caucasian female, who had originally undergone an uneventful laparoscopic adjustable gastric band 4 years ago, presented to the bariatric surgery clinic with inability to tolerate solids. A work-up revealed that the laparoscopic band remained in good position but the patient had oesophagitis and gastritis, causing swelling of the mucosa at the band site. The fluid was removed from the reservoir, and the patient was treated conservatively with anti-reflux medication and a full liquid diet. After 2 weeks of treatment the patient’s symptoms improved. After careful consideration, she wished to undergo revisional surgery converting the laparoscopic adjustable gastric band to a laparoscopic vertical sleeve gastrectomy. The patient moved through the appropriate multidisciplinary team approach and was found to be an appropriate candidate for surgery. She underwent laparoscopic removal of the adjustable gastric band and conversion to a laparoscopic vertical sleeve gastrectomy without complications. Her post-operative course was uncomplicated and she was discharged on post-operative day 3.\nOn post-operative day 12, the patient was readmitted to an outside tertiary care hospital for lightheadedness and shortness of breath and was found to have leukocytosis, with white blood cell count of 18,000 cells μl–1. The work-up included a CT scan with intravenous contrast of the chest, abdomen and pelvis, and the patient was diagnosed with a pulmonary embolism. The patient was immediately transferred to our centre for definitive care. When the patient arrived at our centre, the CT films from the outside hospital were reviewed by our radiologists and there was concern that there was air and a faint suggestion of oral contrast outside of the suture line (). Given this finding, an UGI evaluation was ordered. During the early phase, no leak was observed, owing, in part, to the slow passage of 30 ml oral non-ionic contrast (). Some residual contrast from the outside hospital CT was present in the transverse and descending colon. Only after delayed imaging and with administration of additional non-ionic contrast for a total of about 65 ml (approximately 2 h after the start of the fluoroscopic examination) was there a faint suggestion of extravasated contrast, best seen below the left hemidiaphragm (). Follow-up CT scan with oral contrast confirmed the obvious leak ().\nThe patient was treated definitively with endoscopic stent placement and clipping using an Ovesco clip (Ovesco Endoscopy AG, Tubingen, Germany) to close the leak. After an extended hospital course, she was discharged and is presently doing well.
This 66-year-old African American woman presented with insidious onset of headaches followed by acute onset right-sided weakness and somnolence. She had a history of hypertension and a remote history of breast cancer with prior mastectomy.\nThe patient presented to the emergency department of Houston Methodist Hospital and was intubated on arrival for airway protection. Her pupillary exam revealed bilateral reactive pupils with symmetric size of 4 mm. Her motor exam revealed monoplegia of her right arm. She exhibited spontaneous movement of her left side and to a lesser extent her right leg.\nAdmission CT scan showed a large (8 × 4.5 cm) parenchymal hematoma in the left temporal lobe extending into the frontal lobe, basal ganglia, and insula. There was evidence of a blood-fluid level within the hemorrhage and midline shift of 8 mm from left to right. Subfalcine and uncal herniation were also present []. An MRI of the brain with and without contrast was obtained and showed a lobulated enhancing hemorrhagic mass in the left anterior temporal lobe attached by a stalk to the left insula with surrounding vasogenic edema. Acute hemorrhage posterior to the mass was also seen with blood-fluid levels filling a thin-walled cystic space in the left temporal lobe and left inferior frontal lobe [Figure –]. CT angiograms of the head and neck were performed showing no evidence of a vascular lesion.\nThe patient underwent a left frontotemporal craniotomy for evacuation of the hematoma and associated mass. The dura was opened in a c-shaped fashion and a corticectomy was performed through the inferior temporal gyrus as we were on the dominant hemisphere. A large clot was immediately encountered and evacuated to decompress the surrounding brain and relieve mass effect. This continued until all evident clot had been removed; there was no gross evidence of tumor at the time of surgery due to the significant amount of hemorrhage.\nMicroscopically, there were perivascular sheets of markedly atypical epithelioid cells with pleomorphic nuclei and frequent multinucleation. Brisk mitotic activity and abundant apoptotic bodies were seen. Prominent microvascular proliferation and many blood vessels with organizing thrombi were also noted. Recent hemorrhage was seen in the background [].\nImmunohistochemically, the tumor cells were positive for glial fibrillary acidic protein (GFAP) [] and S-100 protein. Pancytokeratin, HMB-45, and Melan-A were negative. These histological features as well as immunohistochemical profile were consistent with a diagnosis of epithelioid glioblastoma.
A 15-year-old obese boy visited our clinic with a painful hip on the left side. The complaints arose one year earlier after he had fallen on his hip. At that time a mild epiphysiolysis was diagnosed on presentation in the emergency room. He was admitted with bed rest and three days later an in situ fixation was performed with one cannulated screw (Figure ). The postoperative recovery was without complications and he was pain free after a few weeks. As it was thought that the fracture had consolidated, the screw was removed 11 weeks after initial placement. After this procedure had been performed his hip became increasingly painful and he experienced reduced mobility. Ten weeks after the screw removal, he fell again, complaining once again of severe pain in his hip. Plain radiographs were performed and a progressive abnormal position of the head of the hip with callus formation was seen. Initially the conservative treatment consisted of physiotherapy. Because of the persistence of disability he was referred to our orthopedic children’s clinic several months later.\nAt that time he had a painful gait with a severely limited left hip function with 70 degrees of flexion. His left leg was externally rotated, with an internal and external rotation in extension of 0–30–50 degrees. The X-rays depicted a severe SCFE with a slip of 70 degrees and an open growth plate (Figure ). Given the seriousness of the slip and the open growth plate, a re-(screw) fixation of the epiphysis was performed with an additional subtrochanteric correction osteotomy (according to Southwick). The postoperative course was uncomplicated (Figure ). After an initial period of six weeks of unloaded mobilization, weight bearing was supervised by the physiotherapist. During the last outpatient appointment, two years postoperatively, he was still found to be limping slightly, but he was pain free. On examination there was a leg length difference of 2cm with a hip motion of 100 degrees of flexion and an internal and external rotation of 25–0–45 degrees. The Harris Hip Score was 97. The X-ray showed a cam lesion due to the deformity, no signs of avascular necrosis (AVN) or chondrolysis and a Southwick angle of 25 degrees.
A 68-year-old Caucasian woman was referred into secondary care by her GP with abdominal bloating and postmenopausal bleeding of several weeks' duration. She was otherwise fit and well. As per NICE guidelines she had an urgent abdominopelvic ultrasound [], which revealed a 32 × 39 mm lobulated heterogeneous mass from the posterior wall of the endometrial cavity and an incidental 61 × 29 × 59 mm well-defined mass, superior to the right kidney, which was suspicious for an adrenal mass. Her physical examination was unremarkable. Urgent cross-sectional imaging and direct visualisation of the uterine cavity was advised. The patient underwent hysteroscopy, and endometrial biopsies, which showed a grade 3 endometrial adenocarcinoma. A pelvic MRI confirmed the presence of a large endometrial tumour extending into the posterior myometrium and a 5.9 cm complex mass in the Pouch of Douglas. The patient had a full body CT, which showed bulky bilateral adrenal masses, which were not in keeping with benign adrenal adenomas ().\nThe case was discussed at the central multidisciplinary team meeting (MDT) and the recommendation was to have radiological guided biopsy of the adrenal mass. The histology of the right adrenal mass showed a poorly differentiated adenocarcinoma in keeping with primary endometrial cancer (). A panel of representative immunohistochemistry was undertaken, but unfortunately in the antibodies of interest, the tumour was cut out. Therefore, consensus was reached by two external pathologists who agreed on the identical morphology between the adrenal tumours and the primary endometrial tumour. To resolve initial concerns about two synchronous primary lesions, a PET-CT scan had been requested, which reported the two adrenal metastases being hypermetabolic and suspicious for malignancy, showing increased FDG uptake (). A diagnosis of Stage 4b, Grade 3 EC with adrenal metastases was made, and the patient was referred to the medical oncologists for consideration of neoadjuvant chemotherapy.\nThe patient commenced on neoadjuvant chemotherapy with Carboplatin and Paclitaxel. She tolerated the chemotherapy reasonably well. Following her third cycle, she developed neutropenic sepsis and was treated with intravenous antibiotics and G-CSF injections. She then had a repeat CT scan, which showed size reduction in the primary tumour and metastases. A decision for further three cycles of Carboplatin and Paclitaxel with a 20% dose reduction was made as it was still felt that surgical intervention would be of no benefit at that point. Nevertheless, the option of palliative radiotherapy was discussed with the patient if she were to develop heavy vaginal bleeding. Following six chemotherapy cycles, a repeat CT showed further good response of the primary tumour and the adrenal metastases (). She was rediscussed at the Gynaecological Oncology MDT and a repeat PET-CT was requested. The uterine mass was still prevalent, but the adrenal metastases were no longer PET-avid (). No extrauterine disease was identified. She was commenced on alternating hormonal therapy with Megace/Tamoxifen three-weekly. The adrenal MDT recommended a bilateral adrenalectomy following pelvic surgery. The patient underwent a total laparoscopic hysterectomy, bilateral salpingo-oophorectomy and adhesiolysis followed by a bilateral retroperitoneal laparoscopic adrenalectomy two months later. The histology of the adrenal glands showed complete pathological chemotherapy response and no evidence of viable metastatic cancer in either adrenal gland. The patient was reviewed in clinic three and six months after her adrenalectomy with no clinical evidence of recurrent disease. She has been discharged from the endocrine clinic and remains on maintenance hydrocortisone.\nWe searched the MEDLINE and EMBASE databases for articles published from inception to April 2019 using medical subject heading (MeSH) terms. Key terms included “endometrial cancer” and “adrenal metastases”. The search was limited to the words “humans and adult female”. Publications were cross-referenced from reference lists to obtain additional citations. Only case series published in English language but with no geographical restrictions were included in the literature review. The electronic search initially yielded 14 citations. All reports were published in English language. Four studies were unrelated after screening titles. Nine publications were finally included in the literature review. The main characteristics of those case report studies are shown in .
A 43-year-old woman with no remarkable medical history underwent diagnostic arthrography of her right shoulder due to ongoing symptoms following a traumatic injury. Ten days later, the patient was admitted to the same general hospital with Staphylococcus Aureus sepsis. Ultrasound examination showed a fluid collection in her right shoulder. Repeated arthrocentesis was performed in order to drain the fluid and antibiotics were started. Three days after admission, she developed respiratory failure for which she was intubated and mechanical ventilation was started. The CT scan showed severe pneumonic infiltrates. Due to further deterioration, the patient was transferred to an academic hospital.\nOn transfer the patient was ventilated in the prone position []. During the first week, a spontaneous right-sided pneumothorax developed for which a chest tube was placed. Surgical debridement of the right shoulder was performed in order to eliminate the persistent infection. After 7 days she developed refractory respiratory failure and VV-ECMO was initiated. Consecutive CT scans showed a large bronchopleural fistula of the right lung, multiple abscesses and lung parenchyma which at that time was regarded as being destroyed (Fig. a). The patient continued to deteriorate and due to the ongoing septic profile, she was referred to our center where the proposal was made to perform a bilateral pneumectomy and then clear the infection, whilst keeping the patient on ECMO until such time as bilateral lung transplantation was possible [].\nAt the time of admission to our ICU, 35 days after intubation, the patient was in respiratory distress despite maximum VV-ECMO settings and tracheostomal high-flow oxygen therapy. She was not mechanically ventilated due to the impossibility of achieving adequate tidal volumes with lung protective settings. Successful donor matching for lung transplantation was considered highly unlikely due to the presence of multiple HLA antibodies (Panel-reactive antibody test = 100%). Furthermore, the patient was immobilized and had critical illness polyneuropathy, providing a further contraindication to transplantation. In order to facilitate lung recovery, we applied the TCAV™ method to set and adjust the APRV mode [, ] (Dräger Evita® V800). The TCAV™ method applies an extended high pressure CPAP Phase with intermittent very brief Release Phases for CO2 removal. We set the auto-release at 75%, resulting in termination of expiration at 75% of the peak expiratory flow rate, which initially yielded high frequencies and small tidal volumes (9). Our goal was to stabilize the lung with the very short Release Phase to minimize repetitive alveolar collapse-induced atelectrauma. The bronchopleural fistula and corresponding significant air leak persisted and initially worsened, however, because the patient’s collapsed lung parenchyma recruited increasing lung volume, tidal volumes were able to be increased and FiO2 decreased. A tracheostomy was performed eleven days after admission. After a month of APRV and while still on VV-ECMO, her arterial blood gas (ABG) normalized (see Table ). This strategy enabled the patient to be weaned off VV-ECMO and subsequently mechanical ventilation. After three months the patient was discharged from ICU (Fig. b), her tracheostoma had been closed but the chest tube was still in situ.\nTwo months after ICU discharge, surgery was performed to close several remaining bronchopleural fistulas and the patient was readmitted to ICU. Due to drain dysfunction, the fistulas unfortunately reopened. She was extubated the day after surgery, however, due to weakness, atelectasis and ongoing significant air leakage, the patient had to be reintubated after 3 days. One day later, a repeat tracheostomy was performed. Traditional pressure support and control ventilation modes were insufficient and resulted in significant atelectasis so the TCAV™ method was restarted. PHigh was reduced very slowly. Her clinical situation improved and attending physicians switched to pressure support ventilation, which was regarded as being more comfortable for the patient. However, hypercapnia occurred and after 2 days the patient was exhausted and the TCAV™ method was again restarted. Due to clinical suspicion of infection, antibiotic therapy was broadened. After this infection had been cleared, weaning from ventilation was initiated which was successful after a further 20 days (Fig. d).\nAfter less than 2 months, the patient was discharged home with a portable chest tube. The tube dislocated but this did not result in pneumothorax. In October 2020, the patient tested positive for COVID-19, but she only had minor symptoms and there was no need for admission to hospital.
A six-year and 8-month old boy (height: 98 cm, weight: 13.4 kg) with Ebstein’s anomaly and intellectual disabilities; who had undergone the Glenn surgery at 10 days old to connect his superior vena cava to the pulmonary artery, and the Fontan surgery at 4 years old to connect his inferior vena cava to the pulmonary artery; began to experience sudden vomiting, a loss of consciousness, and facial spasm on August 8. He was taken by ambulance to a special medical institute at a children’s hospital, where he was immediately admitted into the intensive care unit.\nThe patient was previously diagnosed with rampant caries at the age of 5, and received conservative dental treatments for his 16 teeth with AP under general anesthesia because of high risk of IE, followed by regular checkups once every 3 months. At the regular dental checkup 3 months prior, his left lower deciduous central and lateral incisors were rigid without any mobility, although it was noted that the permanent successors existed under the gingiva behind these teeth. No dental problems were found except for fair to poor oral hygiene. The Plaque Index Silness and loe of the patient was 1.8 in average. It was reported that the patient began to frequently touch his left lower deciduous central and lateral incisors and he had self-extracted those teeth 2 weeks prior to the appearance of the reported symptoms. A week before his hospital admission, he complained of pain in his right ear and developed a continuous fever (> 38.0 °C). However, his parents did not consult his attending cardiologist because they had perceived these symptoms to resemble those of a common cold. In this time, they were not informed about the risk of IE associated with dental events or procedures.\nBoth magnetic resonance imaging and computed tomographic (CT) scans of the brain were performed on admission and showed a ring-shaped abscess of 18 mm in diameter in the transitional area of the right temporal lobe (Fig. a). Echocardiography showed an incomplete tricuspid atresia with moderate regurgitation--predisposing heart conditions and minor echo criteria for the diagnosis of IE, but no oscillating intracardiac mass on valve or supporting structures, in the path of regurgitant jets, verrucous endocardial lesion, or thrombus in the heart--positive echo criteria of IE (Fig. ). An electrocardiogram showed complete right bundle branch block and right axis deviation. A chest X-ray showed right ventricular hypertrophy (CTR = 58%).\nAs an initial therapy, the patient received 1) cefotaxime (200 mg/kg/day) and meropenem (120 mg/kg/day) intravenously from August 8 to16. However, in a brain scan on August 13, the abscess was found to have enlarged to 21 mm in diameter. In response, the attending doctor administered 2) rocephin (200 mg/kg/day) and meropenem (120 mg/kg/day) from August 17 to 21. On August 22, a third brain scan measured the size of the abscess to be 29 mm and showed that wall thickness of the abscess and the surrounding edema became more prominent (Fig. b) with continuous spasm. Subsequently, since the size of the abscess was still increasing and exceeding 2.5 cm while on antibiotics for 2 weeks, the doctor administered 3) panipenem/betamipron (95 mg/kg/day) from August 22 to 23, and a brain surgeon performed surgical drainage - craniotomy followed by approaching the brain mass and draining the pus out of the abscess by using CT-guidance to enhance the efficacy of antibiotic therapy and reduce raised intracranial pressure [] on August 23, when the drugs effect reached its maximum. To find out the causes of insufficient effects from previously administrated antibiotics on a growing abscess, we obtained materials from the abscess. A Gram stain of them revealed gram-positive coccus, and blood and abscess culture tests detected intraoral bacteria of the Streptococcus milleri group. Based on the detection of these bacteria and possible involvement of the Methicillin-resistant Staphylococcus aureus, the doctor then administered 4) vancomycin (45 mg/kg/day) and rocephin (100 mg/kg/day) from August 23 to 29. Thereafter, the patient recovered steadily and began carbamazepine, an antiepileptic medication, to avoid post-operative epilepsy. Finally, he was discharged from the hospital after 2 months of treatment.\nAt a regular checkup after the reported incident, we informed the patient’s mother about the necessity of early extracting deciduous teeth with AP in her child, as oppose to waiting for their natural loss, to avoid self-extraction of his teeth before root resorption without AP. Afterward, the upper left and right deciduous central incisors and lateral incisors were extracted on January 31 and March 7 in the next year, respectively, under the use of AP with amoxicillin. We repeatedly disinfected sockets and checked for the appropriate healing process for 2 weeks after extractions. Although the rest of the deciduous teeth were extracted in the same way before their natural loss, no aberrant symptoms were observed during the course of that treatment.
A 57-year-old male was brought to the emergency room after being found unresponsive following an episode of massive hematemesis. The patient had undergone cardiopulmonary resuscitation prior to arrival in the hospital. His past medical history was significant for severe pulmonary fibrosis, chronic back pain and gastrointestinal reflux disease (GERD). His past surgical history was significant for open Nissen fundoplication five years prior. Based on clinical history and physical examination, the diagnosis of hemorrhagic shock due to upper gastrointestinal bleeding was made. An emergent esophagogastroduodenoscopy (EGD) identified a large amount of blood clot and a bleeding ulcer along the lesser curvature of the stomach. Endoscopic thermal coagulation was used to treat active bleeding from the ulcer. Over the course of the next several hours, his condition continued to deteriorate and a chest radiograph now showed new findings of pneumoperitoneum. An emergent exploratory laparotomy was performed which revealed a large amount of hemoperitoneum in addition to a gastric perforation along the proximal greater curvature. In addition, an area of active bleeding was seen in the region of the posterior gastric fundus and a 1–1.5 cm gastric ulcer incorporated in the posterior portion of the patient’s fundoplication wrap was found. Palpation around the ulcer revealed a large amount of thrombus, which upon removal resulted in massive hemorrhage. Manual compression revealed a fistulous tract running between the gastric fundus and abdominal aorta, which had developed from a penetrating chronic ulcer posterior to the fundoplication wrap. Control of the supraceliac aorta was difficult due to significant inflammatory changes, adhesions, and scar tissue. To facilitate proximal control, a left anterolateral thoracotomy was done and the distal descending thoracic aorta was clamped. Following incision of the diaphragm, the fistula was isolated and stomach separated from the aorta. The distal esophagus directly above the gastroesophageal (GE) junction was stapled off, and the devitalized proximal stomach removed. An attempt was made to repair the involved aortic segment using pledgeted prolene sutures. Due to the friability of the native aorta, a definitive repair was performed by replacing this aortic segment with 20 mm diameter Dacron graft.\nPost-operatively, the patient remained in critical condition and suffered from acute renal failure, necessitating continuous renal replacement therapy (CRRT). He went on to develop abdominal compartment syndrome requiring a second emergent laparotomy. On re-exploration, the remaining portion of the patient’s stomach was noted to have large ischemic patches extending into the pylorus, cecum, transverse and descending colon. Additionally, there were ischemic patches throughout the liver. A complete gastrectomy and subtotal colectomy were done. Despite multiple operations, his overall condition continued to deteriorate. Multiple conversations took place with the patient’s family who ultimately decided to withdraw care, given the poor overall prognosis and need for additional gastrointestinal reconstructions and lung transplantation.
A 74-year-old woman presented to our hospital with a 2-year history of left shoulder pain and persistent joint effusion. She had previously visited a nearby hospital and had been conservatively treated with intra-articular injection of a mixture of local anesthetic and corticosteroid. Radiography of her left shoulder at that time looked normal (). However, some collapse of the humeral head was found 5 months after symptom onset (). Despite the radiological findings, she desired conservative treatment. However, her pain persisted, even at night, and she was referred to our hospital for further treatment, 2 years after symptom onset. A physical examination conducted at our hospital revealed that her left shoulder was not swollen, with an active range of motion of 170° in flexion and 10° in external rotation; the internal rotation level was at L5. Neurological abnormalities were not noted and her blood test results did not show evidence of infectious disease or rheumatoid arthritis. Her synovial fluid was blood-stained but negative for crystals or infection. Additionally, her lumbar spine BMD (%YAM) was 67%.\nRadiography demonstrated that the humeral head was slightly displaced inferiorly, but its shape was maintained at the time of symptom onset (). Subsequent radiographs, taken 5 months later, revealed collapse of the humeral head (). Upon the patient's first visit to our hospital, 2 years after symptom onset, the radiographs demonstrated bone defects of the glenoid (). At symptom onset, oblique coronal MRI showed joint effusion and slight collapse of the humeral head (). An axial T1-weighted MRI showed a subchondral serpiginous pattern of low signal intensity with associated bone marrow edema (). At our hospital, oblique coronal MRI demonstrated the collapse of the humeral head, but the rotator cuff remained intact. Tumors, synovial hyperplasia, and intraosseous cystic lesions were not demonstrated ().\nA total shoulder arthroplasty was carried out because the rotator cuff was intact. The excised humeral head had detached articular cartilage and collapsed subchondral bone. Histologically, the articular cartilage was lost. In the subchondral area, the bone trabeculae were mostly vital but were focally necrotic. New bone formation was seen around the necrotic bone trabeculae ().\nAt the 4-year follow-up, the patient was free of symptoms, with an active range of motion of 170° in flexion and 70° in external rotation; the internal rotation level was L5. Additionally, her shoulder prosthesis had not failed, and further progressive bone destruction had not occurred in her left shoulder.
An 18-year-old lady presented to ENT clinic with a discharging sinus on the left side of her neck. She gave a history of recurrent neck abscess with pus discharge for over 4 years. She had to undergo incision and drainage for about 10 times in the past and had taken repeated course of different antibiotics. Her infection however subsided only temporarily and recurred each time with pus discharge, pain and swelling. She did not have any ear symptom. On examination, there was a sinus opening on her upper neck at the level of hyoid bone anterior to sternocleidomastoid muscle ().\nSome pus was draining out from the sinus at the time of first presentation to us and the surrounding skin was scarred. Otoscopic examination revealed an opening on the floor of the external auditory canal directed inferiorly ().\nTympanic membrane was found to be normal. A clinical diagnosis of infected collaural fistula was made. Fistulogram was attempted but we could not inject the contrast which could be due to fibrosis from recurrent infection. Imaging studies were not done due to long waiting time in a resource-poor setting. Excision of the sinus tract was performed under general anaesthesia after the acute infection has settled with a course of oral antibiotic. Circular skin incision was made encompassing scarred skin around the sinus opening. A small size nasogastric tube was used as a guide probe and dissection was done with a wide incision for good exposure ().\nThe sinus tract was traced upwards towards the opening at the external ear canal. We found the sinus tract running superficial to the facial nerve. The upper end of the sinus tract opened at the cartilaginous part of the external auditory canal. We ligated the upper end of the tract, and the sinus tract was excised completely ().\nThe wound was closed with a drain. Post operatively, the surgical wound healed well and she did not have further recurrence of infection at 1-year follow-up.
Case 2 was a 75-year-old Japanese man who presented at our hospital with acute abdominal pain, nausea, and cold sweat. His CT scan showed retroperitoneal bleeding (around the pancreas and the dorsal side of the ascending colon). His general condition was stable, but he was admitted to our hospital as a conservative measure. His angiography (6 days after admission) showed an aneurysm of the pancreaticoduodenal artery without active bleeding.\nOur experience treating MAL syndrome in case 1 enabled us to diagnose the disease accurately in case 2. MAL syndrome was the cause of the aneurysm in this patient too (). We selected laparoscopic surgery based on the MAL syndrome and the benefits of this surgery. The patient's posture for the surgery was the lithotomy position. Intra-abdominal pressure of 12 mmHg was maintained. The points of the trocars were as follows: a 12 mm trocar at the navel for the camera, two 12 mm trocars at the right upper abdomen, and a 12 mm trocar and a 5 mm trocar at the left upper abdomen ().\nFirst, we lifted the liver umbilical ligament by surgical sutures and put in an organ retractor to the crus of the diaphragm in order to improve the field of vision. After opening the omental bursa, we lifted the stomach with a snake retractor and observed the dorsal side of the stomach. We confirmed the left gastric artery and tied it with tape. The tape was taken out from the right outside trocar, and an assistant pulled it to provide traction of the surgical field. Following the celiac artery to the root, the artery was fastened by the MAL. We cut the MAL away little by little with a vessel-sealing system until the running direction of the celiac artery was clearly confirmed. Using a blood flow meter, we confirmed the improvement of blood flow of the left gastric artery (from 5 mm/min to 69 mm/min). A drainage tube was placed in the left subphrenic area, and the wound of the abdominal incision was closed. The operation time was 3 hours 35 minutes, and the blood loss was minimal at 15 ml. The patient's CT scans have shown no recurrence of the aneurysm for 2 years.
Our case was an 11-year-old white male who presented to our emergency department after a high-speed motor vehicle collision with a Glasgow coma scale score of 5. On arrival, the patient was intubated and was hemodynamically unstable. He had a large scalp laceration that was not actively bleeding. FAST examination was negative for free fluid, but the orientation of the spleen was felt by the radiologist to be unusual and the possibility of a splenic injury could not be ruled out. Chest x-ray was concerning for an acute, traumatic DR.\nThe massive transfusion protocol (MTP) was activated and the patient was taken urgently to the operating room for an exploratory celiotomy. There was no significant blood in the abdomen and the stomach and other upper abdominal organs were found to be within the abdomen. The spleen was not injured. The diaphragm was examined and no gross defect/injury was seen (peritoneum was intact in all directions), although the diaphragm did appear to be thin and stretched out (elevated). Postoperatively the patient underwent CT imaging that was concerning for diaphragmatic rupture (), but due to the patient’s severe head injury he was deemed too unstable for re-exploration from a neurosurgical standpoint.\nAs his neurological status began to stabilize, as demonstrated by his ICP measurements, imaging was repeated and was again felt to be conclusive for diaphragmatic rupture (). The patient was then returned to the operating room where laparoscopic examination of the diaphragm revealed a 4.5 cm laceration in the diaphragm from the 11 o’clock position, at the diaphragmatic crura adjacent to the esophagus and extending towards the central tendon with herniation of a portion of the stomach into the chest (). The procedure was converted to open due to poor tolerance of the pneumoperitoneum required for laparoscopy, and the laceration was primarily repaired.\nThe possibility that we may have missed a small diaphragmatic injury that later expanded to a detectable size (DDDR) or that a delayed diaphragmatic rupture (DDR) could have actually happened in this patient is a matter of speculation. Regardless of such possibilities (DDR versus DDDR), our case is an example that even a negative initial exploratory celiotomy in an injured patient does not rule out the possibility of a delayed DR.
A 29-year-old female patient presented to our outpatient department with a 15 years history of painful swelling over the sole both foots(R>L). The patient gives an alleged history that she was apparently normal 15 years ago when she developed a small nodular swelling over the sole of her foot. The swelling was gradually progressive and was multiple in number over her right foot. The swelling was painful and prevented the patient from weight-bearing and even walking for small distances was affected. There was no associated trauma. The patient gives no history of diabetic mellitus and epilepsy. There was no significant familial history of the disease. On examination of the patient, the feet were in plantigrade and no deformities were noted. The right foot revealed multiple nodular swellings which was tender to touch and firm in consistency with no local warmth. The skin over the swelling appeared normal. The biggest nodule measured about 2 cm in diameter. No neurovascular deficits were noted. Range of motion at the ankle and foot was within normal range. Examination of the left foot revealed a smaller nodule measuring 1 cm in diameter with local tenderness. The patient has no similar swelling elsewhere in her body. X-rays revealed no significant pathology(). Magnetic resonance imaging (MRI) was done to evaluate further(). MRI revealed a nodule in both the plantar aponeuroses. A diagnosis of bilateral foot plantar fasciitis was made and the patient was started on conservative management in the form of analgesic and anti-inflammatory drugs, physiotherapy (stretching exercise), cryotherapy, and footwear with soft insole were advised. The patient showed better results regarding pain on the left foot. However, there was no difference in pain or function of the right foot even after 6 months. Persistence of symptoms indicated surgical excision of the nodule. After obtaining necessary fitness for surgery, the nodule over the skin was palpated and marked. A small surgical incision was planned and marked onto the skin with relevance to the clinical anatomy () of the nodule. The skin and soft tissue dissection were made just adjacent to the weight-bearing surface of the foot [] and the nodule as identified (). We had raised skin flaps to aid in exposure. After careful dissection of the fascia, the nodule was exposed and excision of the nodule was done in toto ()andtissue sample is sent to the pathologist for evaluation. The skin flaps were sutured and there was no post-operative complication with wound healing. Sutures were removed after 2weeks. The patient was mobilized non-weight-bearing with walker support for 3weeks following which she was started on full weight-bearing with a soft insole footwear. The healed skin did not show any discharge, inflammation (). The histopathology of the excised nodule was discussed with the pathologist and revealed bland spindle-shaped cells with abundant collagen in a fibrous stroma background (Figs. and ). This was consistent with our diagnosis of plantar fibromatosis. On follow-up, the patient reported no pain and improvement in function of the foot.
An 81-year old female with a history of coronary artery disease, hypertension, and thrombocytosis suffered a witnessed trip and fall onto a nightstand. The patient took 75 mg of clopidogrel daily in addition to an 81 mg aspirin tablet. She reported a mild headache however had no change from her baseline mentation per family members with no evidence of obvious injury aside from a small area of ecchymosis near a small forehead laceration. She remained up and ambulatory with no further complaints. Ten hours after her injury the patient presented to the Emergency Department with stridorous and agonal respirations with a profoundly decreased level of consciousness. She was noted to have developed extensive ecchymosis on the anterior portion of her neck and chest. Her symptoms had begun rapidly shortly prior to arrival while lying in bed. Family reported that she had been in the constant company of her husband with no further falls or injuries that had occurred since her fall. The patient was intubated upon hospital arrival due to respiratory extremis with obvious swelling and crepitus noted on neck examination. A noncontrast CT scan of head was unremarkable while there was demonstration of a large retropharyngeal hematoma measuring 3.6 cm by 5.3 cm by 20 cm on a CT of the cervical spine with no evidence of fracture. Her hemoglobin was 9.5 gm/dL and platelets were 1234 per deciliter, with an INR of 3 and a slightly below normal and activated partial thromboplastin time of 23.9 seconds (reference range 25-35 seconds). A CT angiogram of the neck was subsequently obtained demonstrating active bleeding from the anterior ligaments of the vertebral column that was not felt to be amenable to embolization (). Given the extent of the hematoma intraoral surgical evacuation was performed with bleeding from the anterior vertebral spine controlled with Bovie cauterization, placement of topical thrombin, and drain placement. No reaccumulation of hematoma was noted during her hospital course. The patient unfortunately expired 12 days from the date of admission from presumed aspiration pneumonia and multisystem organ failure.
A 36-year-old Chinese man presented with 26 months of progressive motor aphasia and cognitive decline. Fourteen months after the initial symptoms, he began complaining of mild right-sided weakness. He was admitted to our hospital for acute exacerbation of confusion and weakness of limbs lasting 3 days. Upon further questioning after admission, the patient indicated that he had been experiencing slight vision loss for 11 months, particularly the right one, which had not attracted any attention due to the ametropia of both eyes. Neurological examination revealed bradyphrenia, motor aphasia, normal pupillary response but decreased visual acuity of bilateral eyes (visual acuity could not be measured using the standard logarithmic visual acuity chart due to the poor consciousness state of the patient), right central facial palsy, dysarthria, and quadriplegia.\nHis medical history included papillary thyroid cancer for over 1 year without any special treatment and chronic hepatitis B virus infection and cirrhosis for 10 years under regular anti-virus therapy. The patient had no siblings. Family medical history revealed that his father had developed a brain mass of unclear etiology and died at the age of 34. While his mother did not suffer from any neurological disorder.\nThe first MRI and brain biopsy were conducted 22 months ago in another hospital. We observed a T2 hyperintense massive subcortical lesions with rim enhancement in the left frontal lobe (Fig. a and d) and a non-enhanced nodular lesion in the left cerebellum. Histopathology of the former lesion indicated focal necrosis and reactive gliosis, along with vessel wall hyalinization and inflammatory cell infiltration. The patient was tentatively diagnosed with TDLs 18 months ago. Three courses of high-dose intravenous methylprednisolone and two courses of intravenous cyclophosphamide had been given subsequently during the following 16 months. The patient received repeated brain MRIs every six months. Cerebral edema reduced while contrast enhancement still existed and a surgical trail was left in the frontal lesion. The new lesions appeared in the left temporal-occipital lobe and the right temporal lobe on serial images (Fig. b, c, e, and f) and gradually deteriorating clinically indicated these treatments failed to prevent relapse.\nCurrent laboratory examination revealed mild anemia, mildly elevated liver enzyme levels, and elevated thyroid autoantibodies. Cerebrospinal fluid (CSF) analysis showed normal pressure and only slightly elevated protein. No antibodies associated with ganglioside or autoimmune encephalitis (against NMDA-R, CASPR2-R, AMPA1-R, AMPA2-R, LGI1, GABAB-R, DPPX) or paraneoplastic neurologic syndromes (against CV2/CRMP5, PNMA2, Ri, Yo, Hu, Amphiphysin) were detected in CSF and serum. CSF oligoclonal band, serum AQP4-IgG, and MOG-IgG were not detected. Whole-body 18F-fluorodeoxyglucose-positron emission tomography/computed tomography showed no evidence of malignancy. Repeated non-contrast computed tomography (CT) of the head showed hypodense lesions and several punctate calcifications (Fig. a). A second brain biopsy of the lesion in the left temporal-occipital lobe was obtained but only revealed a similar result to the first time (Fig. b and c). We confirmed retinal vasculopathy using fundus photography and fluorescein angiography (Fig. d), leading us to suspect hereditary vasculopathy. Whole exome sequencing identified a heterozygous GTCA insertion (c.741_742insGTCA) in the TREX1 gene (NM_033629) resulting in a frameshift mutation (p.T249Sfs*14). Nucleotide change was confirmed by Sanger sequencing (Fig. a). Therefore, we diagnosed the patient with RVCL-S. Genetic testing of the mother, aunt (father’s sister), and son showed that all lacked the mutation (Fig. b, c, and d).
A 4-month-old male infant born at 38 weeks gestation by normal vaginal delivery with birth weight of 2.5 kg was admitted in our institute. The child was asymptomatic until 3 months of age when he had an episode of fever and fast breathing for which he presented to the pediatrician. The child's mother gave history of pulmonary tuberculosis 2 years ago for which she was treated for 6 months. A cardiac mass was discovered on a chest X-ray taken for evaluation of the baby's condition. Subsequent computed tomography scan showed a large lobulated heterogeneously enhancing mass lesion in the right pericardial region closely abutting the lateral margin of the right atrium. A part of the mass extended inferiorly and caused focal indentation of the liver. No calcification or evidence of thickening of the pericardium was seen []. At admission, the baby was dyspneic, with history of intermittent feeding. Echocardiogram showed large pericardial collection with features of cardiac tamponade with right atrial and right ventricular free wall collapse. There was a large mass within the pericardium which extended with tongue like projections into the liver [Figure and ]. Pericardial tapping was done as emergency palliative measure. Cytology examination of pericardial fluid revealed no evidence of malignant cells. Two days later, a right anterolateral thoracotomy was performed to remove the mass. A lobulated mass was found arising from the right side of pericardium anterior to the phrenic nerve. The inferior aspect was going through the diaphragm. The healthy pericardium was dissected and still the stalk was found to to be attached to the diaphragm the diaphragm firmly, but was free from the liver. The central part of right diaphragm was excised along the mass, which could be removed in one piece. The defect in the diaphragm was closed with a dacron patch. The histopathology report was positive for MIC-2, pan cytokeratin (immunohistochemistry (IHC)). Microscopic examination revealed a tumor composed of sheets of cells with hypersarcomatic nuclei and moderate cytoplasm []. Interventing blood vessels were seen. Frequent mitosis was seen. Excisional margin was clear of tumor. The child had a smooth postoperative course and was discharged in 4 days. Postoperative echocardiogram done after 1 month showed no residual mass lesion.
The patient is a 76-year-old woman with a two-year history of left elbow pain empirically diagnosed as gout. When her symptoms failed to improve with appropriate management, radiographs were obtained, demonstrating a lesion in the proximal radius () characterized as a mildly expansile lucent lesion with a thin zone of transition but no sclerotic rim. Internal osseous septations were present and there was cortical thinning but no visible cortical breakthrough, periosteal reaction, calcified matrix, or soft tissue mass. The initial differential diagnosis included metastasis, multiple myeloma, and other less common entities such as a primary sarcoma of bone or atypical infectious process. She was referred to our tertiary care hospital to consult with an oncologic orthopedic surgeon. Further history obtained at that clinic visit elicited that 3 years previously she had incidentally discovered lytic lesions in her skull and left clavicle that were evaluated in another medical system. Biopsy of both lesions performed at that time was inconclusive showing a mix of inflammatory and fibrous cells per report. The pathologic specimens were not available for further review. Physical exam at her clinical visit was unremarkable with no palpable lymphadenopathy and no visible abnormality at the symptomatic left elbow. SPEP and UPEP tests were negative.\nHer initial imaging work-up included CT of the chest, abdomen, and pelvis; contrast-enhanced MRI of the left forearm; and nuclear medicine bone scan. Her CT scan showed no findings of primary malignancy and—pertinent to her eventual diagnosis—showed no lymphadenopathy or vital organ abnormality. Bone scan demonstrated marked radiotracer uptake at the site of the lytic lesion in the proximal left radius as well as at the previously biopsied skull and left clavicle lesions (). The MR scan of the left forearm showed a marrow replacing lesion within the proximal diaphysis of the radius (). The lesion was T1 isointense, T2 hyperintense and demonstrated avid enhancement. Cortical thinning and small areas of cortical breakthrough not visible on the radiographs were apparent on the MRI. No associated soft tissue mass or perilesional edema was present.\nAt the request of the orthopedic oncologist, a fluoroscopy-guided percutaneous biopsy was performed by Musculoskeletal Interventional Radiology. This rendered only tiny fragments of tissue that were nondiagnostic at histologic review. The patient then underwent open biopsy and curettage of the lesion with Orthopedic Surgery for both diagnostic and treatment purposes. Lesion histology demonstrated features diagnostic of RDD including emperipolesis (engulfment of intact lymphocytes contained with the cytoplasm of histiocyte cells) and positive S100 immunohistochemical staining (). At her follow-up clinic visit 8 weeks after surgery, the patient reported resolution of her left elbow pain, and repeat radiographs demonstrated partial filling in of the lesion with healing bone (). She was discharged from clinic and instructed to follow up if she developed recurrent left elbow symptoms or similar symptoms at a new site. One year later, she has not sought further care at our institution.
The patient is a 74-year-old male with comorbidities of coronary artery disease, congestive heart failure, hypertension, and renal insufficiency who presented to an outside hospital with back pain and left upper extremity weakness associated with high fevers and urinary incontinence. Outside spinal imaging showed a large spinal abscess; therefore, he was transferred to our institution for a higher level of care. On presentation, the patient was in septic shock requiring fluid boluses and inotropic agents to stabilize him. Further history was obtained from the family as follows:On November 2016, he underwent an elective right TKA complicated by an early MRSA PJI associated with bacteremia On May 0f 2017, he underwent resection of the hardware in the knee, incision and drainage, followed by 12 weeks of daptomycin therapy Five months later, on October of 2017, due to relapse, he had a second debridement of the right knee for source control as well as left ankle incision and debridement followed by another 8 weeks of daptomycin for this relapse of infection A daptomycin-susceptible, vancomycin-susceptible MRSA was isolated from blood and both surgical sites, knee and ankle, on both occasions He had ongoing thoracic back pain since 2016 which was monitored radiographically by his local providers, until the development of spinal epidural abscess with upper extremity weakness, which prompted his current hospitalization in January of 2018\nReview of systems on presenting to our institution was significant for general weakness and malaise, right shoulder and thoracic back pain, and constipation from narcotics. He was hemodynamically unstable requiring inotropic support. He was awake and oriented, following commands with intact speech. There were no cranial nerve deficits. On motor testing, he had normal muscle bulk with generalized hypotonia. There was no movement of his left upper extremity. He had 2/5 strength on his right upper extremity and 2/5 strength on his bilateral lower extremity. There was decreased sensation to light touch on his left side. Reflexes were globally decreased with negative Hoffman and Babinski signs. The white blood cell count was 30,000/L, and procalcitonin was 4.88 ng/ml. Blood cultures grew MRSA rapidly. Repeat imaging of the brain and spine at our institution showed extensive epidural phlegmon throughout the cervical, thoracic, and lumbar spine with intracranial expansion into the posterior fossa beneath the cerebellum with pockets of possible early organizing abscess within the phlegmon (). Brain imaging identified no discrete abscess or leptomeningeal enhancement.\nNeurosurgery immediately evaluated the patient and promptly performed a cervical spine decompression of C1–C7 and thoracic spine decompression of T5–T7. Operatively, a large epidural abscess was found, drained, and washed out. He was started on vancomycin every 12 hours with trough vancomycin levels being therapeutic. The patient subsequently underwent irrigation and debridement of the right knee, left ankle, and left great toe as well at our institution; all surgical sites grew MRSA with vancomycin MIC of 1 mcg/ml. Despite attempts at source control and optimal pharmacokinetic dosing of vancomycin with a trough level of 20.5 mcg/ml on day 5, he had refractory MRSA bacteremia. Infectious disease deemed he had failed daptomycin therapy; therefore, ceftaroline 600 mg every 8 hours (MIC of 0.38 mcg/ml) was added to vancomycin. Repeat blood cultures showed clearance of bacteremia after 48 hours of initiation of the combination therapy. His left ankle and right knee continued to yield MRSA. Due to his multiple comorbidities and need for more aggressive source control of his infection, i.e., amputation of the leg, palliative care was sought by the family, and he died a few days later.
A 30-year-old African-American male patient presenting to the hospital for trauma injuries to his abdomen. He had sustained a gunshot wound, which necessitated an exploratory laparotomy along with a small bowel resection. The patient had to have an ileocecostomy done. He initially improved but on the third day post operatively he became very short of breath and hypoxic. High flow cannula was initiated and a work up for the possible etiologies of hypoxemia was undertaken. Computed tomographic (CT) scan of the chest with intravenous contrast was performed and was notable for a pulmonary embolism (PE) in third and fourth order segmental branches of the right upper lobe pulmonary artery. Heparin infusion was started for treatment.\nThe usual protocol with aPTT monitoring, for the dose appropriation of heparin infusion, was initiated. During the following period it was found to be difficult to achieve a therapeutic range for anticoagulation based on the aPTT. During this time he had only been therapeutic once following the transfusion of one unit of fresh frozen plasma. The heparin was titrated up with increasing dose of heparin. However, suspicions for heparin infusion became a concern when the dose required approached 50,000 IU/24 h without aPTT being therapeutic. Haematology was consulted for evaluation and guidance.\nAt the time of evaluation patient was in no acute distress without any significant physical findings except for tachycardia and abdominal distention with midline scar closed with mesh. The former was likely explained with the PE the latter was secondary to the surgery. The trend for aPTT was noted to be in a sinusoidal pattern ranging between 51 and 27. Since the patient had multiple factors because of the acute situation, it was decided to check for factor 10 levels. Also factor VIII levels were checked. Because of the acute trauma to the abdomen it was suspected the factor VIII might be abnormal. His factor VIII levels were 400% (normal range of 50–200%), ATIII 64% (normal range 75–135%), and anti-factor 10a 0.1 IU/ml (normal therapeutic range goal for P.E 0.3–0.7 IU/ml).\nAnalysis of the factor levels showed that though the levels of the factor VIII were high, which can contribute to apparently low aPTT, the patient was in fact subtherapeutic, even with 50,000 IU/24 h of heparin. This was supported by lab value for the AF 10a, which was subtherapeutic for the treatment of PE. It was also established that levels of aPTT were not the ideal method of monitoring for heparin dose appropriation. Rather the monitoring of the AF 10a was a better indicator of the dose requirements for heparin. In his situation, the intervention was simple and required increasing the dose of heparin and monitoring AF 10a to the therapeutic range of 0.3–0.7 IU/ml. His dose was further increased and therapeutic levels achieved soon after.
A 67-year-old male was admitted to the emergency department of our hospital due to severe head trauma. The patient was in a coma with a Glasgow Coma Scale (GCS) score of 9 upon admission. A head CT scan revealed a bilateral frontal contusion and a large left frontal lobe hemorrhage. A left cranioectomy and hematoma evacuation was performed and the postoperation clinical situation kept stable. Eight weeks later, the patient underwent another surgery for cranioplasty and was then discharged for further rehabilitation.\nDuring the next couple of months, the patient initially did quite well. He could walk without assistance and was able to take care of his own affairs. However, 10 months after the first surgery, the patient developed symptoms of urinary incontinence and gait instability. An MRI scan demonstrated marked ventricular dilatation when compared to his previous images. He was then hospitalized again with a diagnosis of hydrocephalus. After admission, a lumbar puncture showed the initial CSF pressure was 140 mmH2O, with positive CSF tap test. A VP shunt was undertaken with an Aesculap Adjustable Valve System (opening pressure 100 mmH2O). Correct placement of the ventricular catheter was accomplished at the first attempt. There was no observance of ventricular cannulation produced blood in the cerebrospinal fluid. The postsurgery course was uneventful. The patient did not have a history of hypertension and his blood pressure was within the normal range after surgery. Neither anticoagulation nor antiplatelet therapy was indicated in the perioperative management. A routine CT scan was administered on the 8th day following surgery and showed similar ventricle sizes when compared to the presurgery images (Figure A, B). The valve was reprogrammed to 60 mmH2O before the patient was discharged the same day.\nHowever, he was sent back to our hospital again 8 h after being home for sudden decrease of consciousness and urinary incontinence. The neurological examination showed that the patient was in a coma with a GCS score of 10. The CT scan revealed a large right frontal intraparenchymal and intraventricular hematoma (Figure C, D). Emergency surgery was suggested but was declined by his relatives. Palliative care was then offered and the patient was discharged 3 days later according his relative's decision.\nInformed consent was given by relatives of the patient. The institutional review board of the Sir Run Run Shaw Hospital approved the report.
A 78-year-old male presented to the hospital with the history of fever and breathlessness. After initial assessment and investigations, the diagnosis of chest infection was made, and the patient was admitted to short stay unit for further management. The patient was a known case of myasthenia gravis and had history of asbestosis in the past. Whilst being on ward, the patient suddenly collapsed and required resuscitation. The anaesthetist was called to intubate the patient and was then transferred to the Intensive care Unit (ICU) for further management. After initial stabilisation, the patient was investigated to find the cause of sudden collapse. The ECG showed the right bundle branch block. The patient had raised Troponin T level, and the echocardiogram revealed well-preserved left ventricular function with reduced right ventricular function and bright mass in pulmonary artery, thus confirming the diagnosis of massive pulmonary embolism.\nThe patient was thrombolysed using 100 mg of tissue plasminogen activator (tPA) over two hrs and Heparin 1000 units per mL at 1 mL/hr given in separate lines. The nor-adrenaline was used to maintain mean arterial pressure of around 85. The patient had femoral arterial line after numerous failed attempts to have a right radial artery line. The patient responded very well to the thrombolytic treatment leading to stable haemodynamic condition. Eight hours following the initiation of thrombolytic therapy, the right forearm of the patient was noted to be very swollen and tight. The orthopaedic team was called immediately to assess the forearm in view of compartment syndrome. As patient being intubated, it was difficult to diagnose a compartment syndrome on just clinical ground, and hence a universally accepted, calibrated handheld device (Stryker, Kalamzoo, Michigan) was used to measure the compartment pressure in the involved forearm compartments (). The pressure was measured to be 45 mmHg. The decision of an emergency fasciotomy of the involved forearm was taken following this measurement and high index of suspicion.\nThe fasciotomy of the forearm was performed by extensile Henry's approach along with decompression of carpel tunnel and abductor compartment of the hand (). The muscles within both superficial and deep compartment were bulging and bloodstained. During this procedure, the massive blood clot was found in the volar compartment of the forearm, close to the radial artery puncture mark and was carefully evacuated. Flexor digitorum profundus was found to be partially necrotic and was therefore debrided till bleeding muscle identified. The wounds of carpel tunnel and adductor compartment release were closed primarily, and volar forearm wound was left open. After 72 hrs, the fasciotomy wound was then evaluated again and closed satisfactorily without any tension on the suture lines (). The patient made remarkable recovery postoperatively and was then commenced on vigorous physiotherapy.\nAt the end of 3-month followup, the patient had full range of movements in elbow, terminal restriction of movement in wrist and hand. He had functional muscle power in his intrinsic muscles of the hand. Apart from mild tingling in the hand, there was no sensory deficit.
A 76-year-old man with a mandibular pathologic fracture visited our department after referral for mandibular reconstruction from the dental department. Upon presentation, the alveolar bone of the right mandibular body was partially exposed through a mucosal defect, but the lesion was nonsuppurative due to the administration of antibiotics at a local hospital before the patient arrived at our medical center (). Approximately 2 months earlier, dental extraction of the right lower first molar was performed at a local dental clinic, and the symptoms began at that time. Computed tomography (CT) revealed an irregular bony fracture with sequestrum in the right mandibular body accompanied by mild soft tissue thickening of the chin, and several reactive lymph nodes were noted at level I in the right neck (). Chronic osteomyelitis of the mandible was suspected. However, the range of pathologic fracture was too large to consider osteomyelitis as the primary cause, and based on the daughter’s claim of his medical history of osteoporosis, the possibility of bisphosphonate-related osteonecrosis of the jaw (BRONJ) was considered. Surgical resection of the lesion and reconstruction using a fibula osteocutaneous flap were planned to manage the wide bony discontinuity after resection.\nTo increase the reconstruction accuracy and reduce the operation time, a three-dimensional (3D) printed modeling method was used. Before surgery, a 3D model of the patient’s skull was printed based on CT imaging, and the area expected to be resected from the symphysis to the right angle was cut off. The part to be reconstructed with a fibula free flap was composed of resin and fixed on both sides. Using this model as a template, prebending of the reconstruction plate was performed, and its position was checked in advance based on the cutting edge, angle, and inferior border of the printed mandible ().\nDuring surgery, the resection margin was set to ensure that enough viable bone was preserved on both sides, and surgical resection of the mandibular segment, including the extensive osteonecrotic lesion and the pathologic fracture, was performed between the symphysis and right angle (). After harvesting the right fibula osteocutaneous flap, osteotomized fibula segments were fixed to the prebent plate. The reconstruction plate was then fixed to both residual mandibular ends with three screws each and additional four-hole miniplates. The ipsilateral facial artery and vein were chosen as the recipient vessels. Microvascular anastomoses were performed with end-to-end sutures in arteries and vein couplers for venous anastomosis. The intraoral mucosal defect could be completely covered with the harvested skin paddle. On postoperative day 2, flap congestion was observed, and venous thrombosis could be found upon exploration immediately in the operating room. Venous reanastomosis was performed end-to-side to the external jugular vein. After revision surgery, the viability of the flap was adequately maintained. The resected segment included sufficient healthy bone around the necrotic lesion; therefore, we did not perform any tissue culture. Instead, we used third-generation cephalosporin as a prophylactic antibiotic therapy.\nWe checked the patient’s local hospital records and medication history, but we could not find any evidence of him taking bisphosphonates. Histological examination of the resected bone segment showed necrotic bone tissue with empty osteocyte lacunae and abscesses with bacterial colonies (). Considering the abovementioned findings along with the preoperative CT results, the final diagnosis was pathological fracture following chronic osteomyelitis of the mandible.\nCT performed on postoperative day 50 showed successful osseous continuity (). Although the postoperative course was complicated by his poor condition, at 2 months after surgery, the patient was discharged with good occlusion and a stabilized skin flap ().
We present a 37-year-old Caucasian female referred to our hospital for cardiac resynchronization therapy due to left ventricle dysfunction. The patient had a history of congenital atrioventricular septal defect which was repaired surgically at the age of 2. Limited medical records had been available. At the age of 8, the patient underwent mitral valve repair. Postoperative complete AV block was present which required implantation of a pacemaker. The patient came to the clinic with complains of exertional dyspnea at minimal physical activities. Holter ECG proved short episode of ventricle tachycardia. Concomitant arterial hypertension with poor control was also present. Transthoracic echocardiography revealed dilated globular left ventricle, with diffusely decreased ejection fraction/ EF 34%/ and septal dyskinesia. End diastolic volume was of 195 mL, and end systolic volume of 125 mL. Peak diastolic gradient of the mechanic mitral valve prosthesis was 11 mm/Hg. Aortic valve was with normal function. Normal right heart chambers with indirect systolic right ventricle pressure 30 mm/Hg. The patient was referred for cardiac resynchronization therapy. During the procedure, the coronary sinus was not possible to be cannulated and only right ventricular defibrillation lead was implanted (Figure ). Therefore, the patient was referred for computer tomography. Gated cardiac CT was performed with Aquillion One, Toshiba machine, following a standard protocol. (Figure ) The CT showed normal anatomy of coronary arteries. Coronary sinus was visualized in the posterior atrioventricular groove measuring 12 mm. Toward the right atrium the sinus was with saccular dilatation and then stopped abruptly without connection with the right atrium. Along the upper border of the terminal part of the sinus, a defect measuring 15 mm was noted and a communication with the lying cranially left atrium. A small vessel measuring 1.5 mm in diameter connected the coronary sinus with the right atrium was detected. Dilated anterior cardiac veins drained into the anterior wall of the right atrium. Also, numerous tortuous small venous vessels were seen in anterior wall of the heart draining into the right atrium and ventricle. No persistent superior vena cava was presented.\nDue to lack of accessibility to cannulate the coronary sinus and the entire cardiac venous system, the patient was referred for surgical lead implantation (Figure ).
Case 2 was a 75-year-old Japanese man who presented at our hospital with acute abdominal pain, nausea, and cold sweat. His CT scan showed retroperitoneal bleeding (around the pancreas and the dorsal side of the ascending colon). His general condition was stable, but he was admitted to our hospital as a conservative measure. His angiography (6 days after admission) showed an aneurysm of the pancreaticoduodenal artery without active bleeding.\nOur experience treating MAL syndrome in case 1 enabled us to diagnose the disease accurately in case 2. MAL syndrome was the cause of the aneurysm in this patient too (). We selected laparoscopic surgery based on the MAL syndrome and the benefits of this surgery. The patient's posture for the surgery was the lithotomy position. Intra-abdominal pressure of 12 mmHg was maintained. The points of the trocars were as follows: a 12 mm trocar at the navel for the camera, two 12 mm trocars at the right upper abdomen, and a 12 mm trocar and a 5 mm trocar at the left upper abdomen ().\nFirst, we lifted the liver umbilical ligament by surgical sutures and put in an organ retractor to the crus of the diaphragm in order to improve the field of vision. After opening the omental bursa, we lifted the stomach with a snake retractor and observed the dorsal side of the stomach. We confirmed the left gastric artery and tied it with tape. The tape was taken out from the right outside trocar, and an assistant pulled it to provide traction of the surgical field. Following the celiac artery to the root, the artery was fastened by the MAL. We cut the MAL away little by little with a vessel-sealing system until the running direction of the celiac artery was clearly confirmed. Using a blood flow meter, we confirmed the improvement of blood flow of the left gastric artery (from 5 mm/min to 69 mm/min). A drainage tube was placed in the left subphrenic area, and the wound of the abdominal incision was closed. The operation time was 3 hours 35 minutes, and the blood loss was minimal at 15 ml. The patient's CT scans have shown no recurrence of the aneurysm for 2 years.
A 76-year-old Brazilian male was admitted to the emergency room complaining of two-weeks epigastric abdominal pain, sporadic fever and weight loss accompanied by nausea and vomiting. The patient had a history of type II insulin-dependent diabetes mellitus, chronic hypertension and non-dialytic chronic renal failure. He denied alcohol or smoking abuse. Two months ago, he was hospitalized with the diagnosis of hepatic and splenic abscesses with blood culture positive to Staphylococcus aureus. On physical examination the patient was eutrophic and looking well. He was afebrile, pulse rate = 92 beats per minute, blood pressure = 150/80 mmHg and slight edema was palpable on lower limbs. Pulmonary examination showed rales on both lung bases. Abdominal examination revealed a generalized tenderness more pronounced on the epigastrium and left upper quadrant. The liver was palpable up to 4 cm below the right costal margin. Heart examination was unremarkable.\nThe results of laboratory tests on admission are summarized in , showing pancytopenia with slight elevation of amylase and lipase.\nAbdominal ultrasonography ruled out the presence of hepatic abscess. The gallbladder wall was thick and micro calculi were evidenced in its interior. The pancreas was diffusely enlarged showing a heterogeneous texture compatible with an acute inflammatory process. A peripancreatic hyper echogenic tissue, which extended diffusely through the retro peritoneum, was also evidenced. This examination was complemented by a total abdominal and pelvic computed tomography (CT), which revealed a diffuse enlargement of the pancreas surrounded by a solid infiltrative tissue, extensive to the splenic, hepatic hilum as well as to the retro peritoneum, which was interpreted as being of inflammatory nature. The presence of a considerable amount of peritoneal fluid also was noted ( and ). Considering the clinical and radiological findings, treatment for acute pancreatitis was undertaken.\nAs no clinical improvement was achieved after four days of appropriate treatment, the patient was submitted to a paracenthesis. The cytological analysis of the peritoneal fluid showed suspicious cells for malignancy along with a high number of necrotic or degenerated lymphoid cells. On the thirteenth day of hospitalization the patient underwent a needle retroperitoneal biopsy. One hour after this procedure, he experienced acute dyspnea, lung congestion, hypotension, cardiac arrest, and died. An autopsy was performed.
A 77-year-old female visited hospital due to a right thigh mass. The mass had been found as small nodule about 50 years ago with no specific treatment, but recently it grew rapidly after applying moxa treatment to the site. She had a medical history of diabetes mellitus, hyperthyroidism and angina and received bipolar hemiarthroplasy 4 years ago due to right femur neck fracture; the mass was distant from the previous operation site. On physical examination, there was a large, firm, nontender mass on the anterolateral side of thigh, measuring about 6 cm in the greatest dimension (). The mass seemed to be movable over the deep lying tissue. The overlying skin showed hardening of surface, but any color change to suggest melanocytic or other skin cancer or abscess was not noted. Other mass-like lesions suspicious for metastasis were not found around the lesion, especially at the right inguinal area. Radiologically, enhanced CT on the thigh revealed an approximately 4.6 × 4.3-cm sized, oval shaped soft tissue lesion in the dermis and epidermis of the right thigh with no identifiable metastatic node at the inguinal area, showing slightly high attenuation on precontrast image with internal faint calcification and heterogeneous enhancement after contrast injection (). Having an impression of soft tissue sarcoma or unknown metastatic tumors, a wide excision was done under the supine position with general anesthesia. The mass was completely excised with a gross minimum of 1 cm safety margin and primary skin closure was done with polypropylene 2-0. Hemovac was placed and removed at 3rd postoperative day. She was discharged at the sixth postoperative day without complication. She refused adjuvant treatment considering her old age and much comorbidity, but there was no sign of local recurrence and metastasis at 3 months, to date.\nMicroscopically, the mass was relatively well defined but focally infiltrative and the tumor cells arranged in a tubular, cribriform, nested pattern (). Most lumens contain bloody and necrotic secretion (). Pleomorphic tumor cells had abundant eosinophilic cytoplasm, round to oval nuclei, dispersed chromatin and inconspicuous nuclei; frequent mitoses were noted including atypical mitosis (). Some fibrotic portions in the deeper portion of the mass showed infiltrative growth of tumor nest that resembled metastatic adenocarcinoma (). There were multiple small calcifications that were noted radiologically, but necrosis was not found. Tumor cells were positive for CK7 and CK19, suggesting origin of skin appendage (). Ki-67 proliferation index was estimated over 50% ().
A 70-year-old male patient visited our pain clinic in December 2017 with gastrostomy site pain that started after percutaneous radiological gastrostomy was performed in May 2017.\nThe patient did not have any previous medical history, except for esophageal cancer located 26–32 cm inferior to the upper incisors. He received concurrent chemotherapy for a month in January 2017 but exhibited worsening of dysphagia and a consequent tendency of aspiration. For proper feeding, he underwent percutaneous radiological gastrostomy in May 2017.\nAlthough he experienced constant pain near the gastrostomy site immediately after the procedure, oncologist continued the use of the percutaneous endoscopic gastrostomy (PEG) tube due to the persistent tendency of aspiration. In June 2017, the patient was treated with a fentanyl patch 75 µg/h and short-acting fentanyl buccal Tab 400 µg for pain management and required hospitalization due to features of delirium suspected to originate from opioid treatment.\nWhen he visited our pain clinic in December 2017, he was being fed via the PEG tube. There were no abnormal findings on the abdominal computed tomography images, and physical examination did not indicate infection of the gastrostomy site. The patient also underwent PEG tube exchange under image guidance to ensure proper positioning of the tube, prior to visiting our pain clinic.\nThe pain was localized to the gastrostomy site and nearby abdominal wall located left and inferior to the xiphoid process. In addition, the pain was dull, with no signs of tenderness or local inflammation (). The patient was given a combination of acetaminophen 325 mg and tramadol HCl 37.5 mg 4 times a day for pain management. Although the pain control was effective (numerical rating scale [NRS] score of 5/10) for ~2 h after drug intake, the patient sometimes experienced severe pain with an NRS score of 9/10.\nIn addition to the oral administration of acetaminophen and tramadol, we locally applied a lidocaine patch and lidocaine 2.5% and prilocaine 2.5% cream to the site, but this was not effective. The use of 10% lidocaine spray was effective for ~20 min after the use, and therefore was utilized together with other drugs.\nNevertheless, there was no persistent improvement in pain. In January 2018, we attempted left-sided subcostal TAP block using 0.8% mepivacaine 4 cm3, and triamcinolone 10 mg and additional local anesthetics infiltration at the gastrostomy site using 1% lidocaine 4 cm3, under ultrasonography guidance. Immediately after the procedure, the pain near the gastrostomy tube was alleviated by ≥ 50% but quickly became aggravated again. After 2 weeks, we performed additional left-sided subcostal TAP block and lidocaine local infiltration at the gastrostomy site. Previous procedures were repeated. However, substantial pain relief was unclear after the procedure, and we were forced to provide oral administration of acetaminophen 325 mg plus tramadol HCl 37.5 mg 3 times a day and short-acting oxycodone HCl 5 mg in case of severe pain. However, the pain control was not effective, and the patient visited the emergency center several times due to pain.\nSubsequently, the patient’s dysphagia symptoms improved and the PEG tube was removed in May 2018. However, the patient continued to experience persistent abdominal wall pain near the gastrostomy site. This dull pain had an atypical tendency to worsen at night (NRS score of 9/10). As the pain relief from traditional medications (i.e., oxycodone HCl or acetaminophen 325 mg plus tramadol HCl 37.5 mg) was not effective, we considered additional opioid usage.\nWe explained to the patient that appropriate intake of the prescribed medication for pain control and dose titration are crucial. However, the patient strongly refused to use further opioid due to fear of using strong opioids—he reported a history of severe delirium caused by opioid usage (including high-dose fentanyl patch) prior to visiting our pain clinic, as well as side effects (i.e., constipation and drowsiness) after oral opioid usage after admission to our pain clinic. The patient continued to suffer from gastrostomy site pain and had difficulties in rehabilitation and daily activities (i.e., deep breathing and supraglottic swallowing) due to pain.\nThe effectiveness of oral medications, such as opioids, and of topical treatments, such as lidocaine patch, cream, and spray, was inappropriate. Whereas TAP block and local infiltration of lidocaine provided short-term pain relief by ≥ 50% without any side effects.\nTherefore, we decided to perform left-sided TAP prognostic block and subsequent neurolytic block. We explained possible side effects (i.e., neuritis, deafferentation pain, or abdominal muscle weakness) and unclear long-term outcomes. The patient still wanted to undergo neurolytic TAP block.\nWe performed left-sided subcostal TAP using 0.5% bupivacaine 5 cm3 under ultrasonography guidance. The patient exhibited temporary pain relief (~5 h after procedure) and the pain worsened again, indicating positive outcome of prognostic block.\nIn June 2018 (7 days after), we performed left-sided subcostal TAP neurolysis. We identified the abdominal muscle layer and rectus sheath under ultrasonography guidance and injected 0.8% mepivacaine 3 cm3 using a 26-G spinal needle. We confirmed concordant pain relief after 5 min and injected 6 cm3 of 100% ethanol with the spinal needle (). The total ethanol concentration for neurolysis was assumed to be 66%.\nThe patient did not experience any side effects except slight discomfort at the injection site immediately after alcohol injection and started to experience pain relief. The patient barely experienced any pain (NRS 0/10) immediately after neurolysis. On the outpatient visit 1 week later, he still experienced mild pain (NRS 3/10) near the medial tip of the gastrostomy site but had no pain in other sites. Pain relief was continuously observed until the 2-month outpatient follow-up, and we discontinued the use of opioids and utilized pregabalin 75 mg for abdominal pain control. However, 3 months after neurolysis, the patient started to experience abdominal pain at the medial tip of gastrostomy site (NRS 6/10), and we were forced to increase the pregabalin dose and use short-acting opioids such as oxycodone HCl.\nThe patient refused to use opioid again due to constipation and wanted other treatment option. We performed left-sided rectus sheath block using 0.5% bupivacaine 5 cm3 under ultrasonography guidance, and a positive outcome (pain relief) was observed.\nOn the 3rd month after the first neurolysis procedure, we performed rectus sheath neurolysis by injecting 0.8% mepivacaine 3 cm3 and then injecting 100% ethanol 7 cm3 (). The total ethanol concentration for neurolysis was assumed to be 70%.\nThe patient did not experience discomfort during the procedure and started to experience pain relief. Pain at the medial tip of the gastrostomy site substantially reduced from NRS 6/10 to 2/10. Currently, he is under 4-month follow-up, with pregabalin 150 mg 2 times a day and no opioid usage.
An asymptomatic 54-year-old man was referred for endoscopic colorectal cancer screening. A bulging mass covered by normal mucosa was identified in the rectum [].\nAn endoscopic ultrasonography (EUS) with fine needle aspiration (FNA) was performed []; between 5 cm and 8 cm from the anal verge, it was found to be an extrinsic compression of the rectal wall in relation with a heterogeneous lesion, 43 mm × 35 mm, cystic in the major part with various anechoic cavities separated by septa of different thickness; some of them was very thick that mimicked echogenic solid components; at the luminal border some parts of the muscular layer of the rectal wall were involved, and it was possible to identify that this layer was duplicated as well and involved the lesion partially; the submucosa and mucosa of the rectal wall were preserved. The lesion was well-delimited, although, parts of the contralateral borders were irregular. It did not involve any perirectal structure. No adenopathies were identified.\nOur first diagnostic possibility was a rectal duplication cyst; but we could not exclude an eventual malignant degeneration because of the existence of solid components, that is, thick septa. Consequently, we performed FNA by EUS with a 22-gauge needle (30 min after the administration of 200 mg ciprofloxacin intravenously). The aspirated material was white colored and thick. Cytological examination revealed the presence of mucus, containing isolated cells with vacuolated macrophage-type cytoplasm and groups of cylindrical epithelial cells without features of malignancy compatible with colorectal mucosal cells [].\nThe patient was submitted to surgery. We found that the lesion was involving the lateral wall of the rectum and an en bloc excision of the lesion and the lateral wall of the rectum was performed. The patient recovered without complications.\nThe macroscopic examination of the excised specimen, with 5 cm × 5 cm × 2 cm, showed the presence of the rectal mucosa, in a small area of its external surface; dissection revealed an irregular cavity with mucus-type material; no communication between the external surface and the cavity was found.\nThe histological examination revealed [] the presence of mucus and, in part of the internal surface, a colorectal mucosal lining, which lay on a smooth and well-defined muscular layer. This layer was in contiguity with the rectum muscular layer, only separated by a thin connective tissue. Externally, the rectal mucosa was identified. In another part of the lesion's internal surface, we found a stratified epithelium and some acinar glandular structures that could be considered heterotypic finding. There was no evidence of malignancy. The diagnosis of rectal duplication was made.
The patient was a 30-year-old G2P0010 who presented to our fertility center seeking fertility treatment. She had a medical history of polycystic ovarian syndrome (PCOS) and her partner had a diagnosis of male factor infertility. She had no prior surgical history, no known allergies, and medications included prenatal vitamins. She denied any history of sexually transmitted infections and had a normal hysterosalpingogram and saline sonohysterogram. Her first IVF cycle with an elective single embryo transfer resulted in a negative pregnancy test. Her second IVF cycle used a GnRH antagonist stimulation protocol and she was triggered with Ovidrel on stimulation day 12. Twenty-two oocytes were retrieved. On day five a single fresh blastocyst was transferred using a pass through technique under ultrasound guidance. A stiff outer sheath was introduced through the cervix and past the internal os. A soft tipped catheter containing the embryo was advanced through the outer sheath and the embryo was expelled into the uterine cavity approximately 1.5 cm from the uterine fundus with good visualization. Beta hCG was positive on post-transfer day 9 and serial beta hCG values were monitored and continued to rise appropriately (Table ). On day 28 after embryo transfer, the patient underwent a transvaginal ultrasound (TVUS) in the office that did not identify an intrauterine pregnancy (IUP) or any abnormal adnexal structures. She was asymptomatic with no vaginal bleeding or abdominal pain. The patient was sent for a more comprehensive ultrasound evaluation at the associated Maternal Fetal Medicine unit and another beta hCG value was obtained. Repeat scan similarly failed to identify an IUP or visualize an ectopic pregnancy. The beta hCG was 12,400 pg/mL. Given the high beta hCG value in the absence of an IUP, the patient was counseled and advised to take methotrexate treatment for presumed ectopic pregnancy of unknown location. One day later (day 29), she received an intramuscular dose of 83 mg (50 mg/m2 body surface area) methotrexate with plans to follow up with repeat beta hCG and TVUS.\nFour days after methotrexate administration, repeat beta hCG level continued to rise (20,000 pg/mL) and an ultrasound performed 1 day later demonstrated a right adnexal mass with a yolk sac, fetal pole, and fetal cardiac activity. The decision was made to proceed with diagnostic laparoscopy for treatment of ectopic pregnancy after failure of methotrexate therapy. The patient continued to be asymptomatic with no vaginal bleeding or abdominal pain. Diagnostic laparoscopy was performed on day 34 post-embryo transfer. The operative findings were significant for minimal hemoperitoneum (<50 mL) and products of conception were noted to be implanted on the peritoneum of the posterior cul-de-sac medial to the left uterosacral ligament (Fig. ). The products of conception were removed using graspers without difficulty and hemostasis was obtained with electrocautery and surgicel. All other pelvic organs including uterus and bilateral ovaries and tubes appeared grossly normal in appearance.
A fifty one year old Sri Lankan woman, presented with fever for three days and constitutional symptoms such as arthralgia, myalgia and headache. There was no specific focus for an infective process such as a urinary or a respiratory tract infection. She was a diagnosed patient with tight mitral stenosis (following rheumatic carditis), for which a metallic mitral valve replacement had been done eight years ago. She was on lifelong anticoagulant therapy with target INR (International Normalized Ratio) maintained with 7 mg of warfarin per day. Her other medication included; digoxin for atrial fibrillation, penicillin prophylaxis for rheumatic valvular disease and a combination of captopril, spiranolactone and furosemide for heart failure. Physical examination showed pallor, dental caries and ankle oedema without any peripheral stigmata of infective endocarditis. On admission, she was hemodynamically stable with a pulse rate of 82 beats per minute and a blood pressure of 110/60 mmHg. There was no postural drop in blood pressure or a narrowed pulse pressure to indicate intravascular volume depletion. On auscultation, the metallic first heart sound and a loud pulmonary component of the second heart sound were heard. Lungs had a few bilateral basal crackles. Rest of the examination was normal.\nThe provisional diagnosis was dengue fever as the clinical picture was typical of the infection. However, measures were taken to exclude an alternative infective process such as infective endocarditis. Her admission coincided with a dengue epidemic in the area. Strict monitoring of vital parameters and appropriate fluid management was initiated according to the national guidelines on dengue [].\nThe initial full blood counts showed a leucopenia and a trend of dropping platelet counts confirming our suspicion. The dengue antibodies assessed by IgM antibody capture ELISA (MAC- ELISA) were positive indicating an acute infection. On day 3 after admission (date of admission taken as day 0), her fever subsided but there was evidence of a small right sided pleural effusion indicating the onset of plasma leakage and the start of critical/leakage phase. The platelet count that was already low was expected to hit a nadir between days 4–5 (within 48 hours after the start of critical phase). The patient was at high risk of internal haemorrhage. The usual management protocol at this time calls for fluid restriction and observation for bleeding manifestations. Prophylactic platelet transfusions are discouraged. However, this particular patient had the complication of a mitral valve replacement needing mandatory anticoagulation. Stopping anticoagulation had the potential life threatening complication of valve thrombosis while continuing warfarin had the risk of a torrential internal haemorrhage aided by an already low platelet count. In the absence of guidelines or even previous case reports with similar experience, we managed the patient with institutional expert opinion.\nFever and vomiting was treated symptomatically with paracetamol and domperidone. Spiranolactone, captopril and furosemide were temporarily withheld since admission. Once the platelet count dropped below 100 × 103/μl, warfarin was withheld. Digoxin was continued at its usual dose of 0.125 μg three times daily. The rationale for stopping warfarin was to keep the patient off it during the critical phase until the platelet count picked up. However, as warfarin has a long half life, it was necessary to stop it at least two to three days before the anticipated onset of critical phase. The plan was to restart warfarin as soon as the platelet count was back within ‘safe’ margins. It was anticipated that the critical phase would only last for 48 hours (which is the typical clinical picture). It was a race against time and she was closely monitored both clinically and echocardiographically for evidence of valve thrombosis.\nThree days after stopping warfarin she entered the critical leakage phase (heralded by the detection of the pleural effusion). During this time she developed gum bleeding, petechiae and haemorrhagic blebs reaching a maximum size of 2 × 2 cm at venepuncture sites in both arms. During the critical phase, the platelet count dropped to 5,000/μl. It was decided to transfuse her with one unit of red cell concentrate and four units of platelet concentrates at this time. After the transfusion the patient was haemodynamically stable without any further evidence of bleeding. Rest of the critical phase was uneventful. However, the platelet count did not rise as expected immediately after the critical phase. It barely passed 50 × 103/μl on day 11 (6 days after the end of critical phase) at which point warfarin was restarted at a dose of 5 mg daily (Table ). This was not accompanied with heparin due to the risk of bleeding and heparin induced thrombocytopaenia. The patient did not develop a valve thrombosis despite being off anticoagulation for 10 days (assuming the period of retained warfarin activity due to long half life since stopping warfarin is equal to the time to regain full anticoagulatory effect after resumption). The target INR was reached within 3 days of restarting warfarin and the patient was discharged on day 15. She was free of symptoms with a platelet count of 260,000/μl when reviewed in clinic one week later. Further follow up for her cardiac condition was arranged at the Institute of Cardiology, National Hospital of Sri Lanka.
The patient is a 26-year-old woman with a 13-year history of chronic abdominal pain that started after an episode of presumed "food poisoning." The pain typically lasted 5-6 hours per day, was triggered by eating or bowel movements, and was often severe enough to induce vomiting. It was causing significant interference with her daily activities, both from the distraction of the pain itself and from the side effects associated with pain medication. The patient found an article about PMM performed on a series of six cancer patients and requested evaluation and consideration for the operation. At the time of our first evaluation, the patient had already undergone multiple colonoscopies with no abnormalities identified other than some nonspecific inflammation. She had also undergone endoscopy with biopsy of her gastrointestinal tract, in addition to a pill camera study. These revealed only small intestinal overgrowth. She even underwent a diagnostic laparotomy that did not show any abnormalities. She carried a diagnosis of severe irritable bowel syndrome with constipation (IBS-C) with visceral hypersensitivity syndrome. Regarding prior therapies, she had tried a multitude of diets without relief, and had even gone on total parenteral nutrition (TPN) for months for bowel rest with no resolution of her symptoms. Notably, her insurance company had denied other surgical options including a spinal cord stimulator and an intrathecal morphine pump. On exam, she was neurologically intact other than diffusely reduced deep tendon reflexes, more so in the upper extremities. Given the nature and severity of the pain, the extensive and unrevealing prior workup and therapeutic strategies, and the patient’s wishes, we offered a PMM as a treatment option for her clearly visceral origin pain. We discussed the risks in detail, as well as the uncertainty given the paucity of clinical experience with PMM in non-malignant pain. Despite these cautions, the patient and family wished to proceed with surgery.\nThe patient underwent an uneventful PMM via a T7 laminectomy. The technique used was that of a simple transverse crush injury to the PSDC system as first described by Nauta and colleagues in 2000 []. Figure depicts the post-operative magnetic resonance imaging (MRI) of the lesion.\nIntraoperatively, she had no decrement in her somatosensory evoked potential (SSEP) monitoring. Anticipating difficulty with post-operative pain control from the patient’s tolerance to opioids, liposomal bupivacaine (EXPAREL®) was injected intraoperatively into the paraspinal muscles and wound edges. Over the first two post-operative days, she was uncertain of any benefit. Then on post-operative day three, she had a bowel movement which was the first in many years without associated severe pain. By two-week follow-up, the patient had experienced no episodes of abdominal pain since the procedure and had weaned her pain medication from 2 mg of oral hydromorphone every 4-5 hours pre-operatively to 2 mg every 12 hours for incisional pain. On exam, she was motor intact with preserved light touch and proprioception in her lower extremities. The only detectable deficit was diminished sensation to vibration on her great toe pads bilaterally. Her gait was normal and Romberg testing was negative. Regarding sensory changes, she reported only mild numbness of the vulva but not of the vagina and some numbness on the insides of her feet. By her eleven-week follow-up, she was pain free off all opioid medication. She was able to eat whatever food she liked and was having normal pain free bowel movements. Also, the previous numbness/tingling in her vulva had resolved, and she had regained full sensation. She was having no balance difficulties, nor was she having bowel or bladder function issues. Moreover, she was again able to exercise and succeeded in losing over 10 pounds since surgery. Her only perceptible abnormality was some tingling in her toes bilaterally and around her incision on her back. Notably, her sexual function was normal, and she was able to achieve orgasm as pre-operatively. Overall, the patient was extremely pleased with the results of the operation.
A 48-year-old woman with neck pain and motor weakness was referred to our emergency room. The patient's symptoms developed when she rose from her bed. The patient had no previous history of anticoagulant therapy or hematological coagulopathy, and was in good health before this episode. On physical examination, she was alert and fully oriented. No neurologic abnormalities were found in the cranial nerves and cerebellar system. However, sensory testing of right arm and leg demonstrated loss of touch along with pin-prick and vibration sensitivity. Motor examination revealed hemiparesis on right side (Grade III on arm and Grade I on leg).\nBrain computed tomography (CT) scans did not reveal any abnormal findings. The patient's coagulation profiles including platelet count, prothrombin time, and partial thromboplastin time were within normal ranges. Emergency MRI scan of the cervical spine was performed. The sagittal T1 and T2 weighted images revealed a dorsolateral subdural hematoma extending from C3 to C5 presenting as high signal in both sequences. The axial images showed left displacement of the spinal cord at this level (). The epidural fat was preserved, confirming the location of the hematoma. There was no signal void area, indicating that there were no abnormal vessels in the lesion. Based on these MR findings, the patient was diagnosed with an acute cervical spinal SDH in the right side at C3-C5 level. For differential diagnosis to distinguish this case from other conditions such as cervical epidural hematoma, a lumbar spinal puncture was performed. About 20CC of CSF was drained and cerebrospinal fluid (CSF) analysis revealed 190,000 red blood cells/mm3 and 267 white blood cells/mm3 (). An urgent laminectomy and hematoma removal was planned, but the patient's motor weakness gradually resolved during surgical preparation. At 5 hours after the symptom onset, her right side hemiparesis improved to Grade IV. The next day, her muscle power on the right side had fully recovered and she was able to walk without any assistance. MR images obtained 10 days after admission revealed that the spinal SDH had completely resolved (). The patient was in good health and free of neurological deficits during the 10 months follow-up period.
A 5-year-old male patient reported to a general dental practitioner with a chief complaint of a pain and swelling in his left back teeth region for 10 days. His parents noticed mild swelling on the lower left side of the child's face for 1 week. Clinical examination revealed a diffuse firm tender swelling on the lower left side of the face and also intraorally in relation to mandibular posterior teeth with obliteration of the buccal vestibule. On palpation, there was mild expansion of the buccal cortical plate in relation to deciduous mandibular left second molar. Primary second molar on the involved side was also tender on percussion. Orthopantomograph showed a circumscribed globular radiopaque mass in continuity with the mesial root from which it arose. The tumor mass was surrounded by a thin, uniform radiolucent halo. The second premolar tooth bud was displaced distally by the tumor mass []. On the basis of the clinical and radiographic features, cementoblastoma, osteoblastoma, osteoma and odontoma were included in the differential diagnosis of this lesion. Considering the attachment to the tooth root on the radiograph, cementoblastoma seemed to be the appropriate diagnosis which needed the histopathological confirmation. Following written consent from the patient's parents, the tooth with the attached tumor mass was extracted under local anesthesia and was fixed in 10% formalin. The specimen was sent to the department of oral pathology and microbiology for histopathological examination. Postoperative period was uneventful.\nThe specimen consisted of a left deciduous second mandibular molar with the mesial root completely embedded within a smooth, globular mass of hard tissue []. The tumor mass was creamish white to tan brown in color and measured 1.5 cm × 1.5 cm × 1 cm in dimensions. The specimen was bisected in mesial–distal direction using a low-speed rotating disc. Examination of the bisected surface showed partial resorption of both roots with mesial root embedded within the tan hard tissue and the other root lying on the surface of the hard tissue mass []. The continuity of the tumor mass with cementum was well appreciated in the stereomicroscopic photomicrograph of the specimen.\nHistopathologically, examination of decalcified hemisection showed areas of radicular dentin with cementum-like tissue attached to it. The tumor mass was seen in continuity with root cementum []. It revealed abundant irregular trabeculae of basophilic mineralized tissue with prominent reversal lines, suggestive of cementum, interspersed with fibrovascular connective tissue. Toward the periphery of the hypocellular mineralized tissue, large cells within lacunae spaces were seen suggestive of cementoblasts []. A capsule-like band of fibrous connective tissue covering was apparent at few areas. Peripherally, characteristic perpendicular arrangement of the trabeculae to the external capsule was noted in the stereomicroscopic picture of the decalcified section []. All these radiographic and microscopic features confirmed the diagnosis of cementoblastoma.
A 73 years old female with significant past medical history of hypertension, hyperlipidemia and Type 2 diabetes mellitus was admitted to the hospital with complaints of diarrhea, hematochezia, lower left quadrant abdominal pain and significant weight loss of 25lbs in 6 weeks. The patient denied vomiting, any recent change in her diet, sick contacts, recent travel or any other active complaints. Physical exam was significant for dry oral mucosa, diffuse abdominal tenderness more prominent in left lower quadrant and sluggish bowel sounds. Her laboratory findings were significant for Hemoglobin: 10mg/dl, hematocrit: 34, white blood cell count: 15 x 103, sodium: 131, bicarbonate 20.2 with normal anion gap, albumin of 2.1 and globulin of 3.9. Clostridium Difficle PCR and Giardia Lamblia antigen were negative on the stool smear. Computerized tomography scan of the abdomen and pelvis showed wall thickening of the distal descending colon and sigmoid colon concerning for colitis (), Hence, a colonoscopy was performed for further evaluation.\nColonoscopy showed ulcerated mucosa, and punched out ulcers throughout the colon. Pathology results revealed inclusion bodies as shown in the bottom picture of concerning for CMV. Hence, the patient was started on ganciclovir and after improvement, was discharged to a subacute rehabilitation center.\nThe patient was readmitted a few weeks later with altered mental status and significant abdominal pain. Physical exam demonstrated guarding, rigidity and absent bowel sounds concerning for bowel perforation. CT scan of abdomen and pelvis was done which showed extensive air in mesentery and abdominal cavity consistent with perforation with the most likely site being the distal colon. The patient underwent subtotal colectomy and tissue was sent for biopsy. The differential considerations at that point included inflammatory bowel disease and ischemic colitis.\nHistology of the terminal ileum and colon revealed chronic active colitis including crypt distortion and crypt abscess as shown in the left and right pictures () involving the entire colon with extensive ulceration and pseudo polyp formation with terminal ileum sparing consistent with active ulcerative pancolitis.
A 9-month old boy presented at a hospital in a south western state of Nigeria, with a swollen left upper arm adjoining the chest, low-grade continuous fever (38.1 °C), frequent passage of loose watery stool and persistent cries for more than 3 h. Child had been immunized about 24 h earlier. The mother reported that the symptoms were observed 2 h after the child was vaccinated with the measles vaccine at a private hospital. The child was one of three children reported to have been vaccinated with measles vaccine at a private hospital during the immunization clinic session.\nOn examination, he was mildly pale, febrile and anicteric. He was moderately dehydrated; mildly dyspnoeic with normal heart sound, heart rate of 148 beats/ min, breath sound was vesicular and respiratory rate of 54 cycles per minute. He was well nourished as the weight was appropriate for age. There was extensive swelling with skin discolouration (hyperemia) involving the entire left upper arm, sparing the distal third of the forearm and hand. There was also swelling of the upper part of the anterior chest wall. The swelling was firm and mildly tender. There was no history of adverse reaction to immunization or any form allergic reaction.\nA day after admitting the child, extensive erythema of the left upper arm and anterior area of the chest was observed with extensive scalded skin lesion involving the deltoid area, the upper chest wall and arm (Fig. ). Desquamation of the affected areas was observed presenting like severely burned skin from a hot liquid. There was darkening and hardening of the skin over the affected area on the arm with eventual severe necrosis up to a depth of about 5 mm thereafter (Figs. and ). A diagnosis of severe necrotizing fasciitis was made.\nRadical debridement of necrotic tissues was carried out under general anaesthesia. Child was also transfused with blood. Daily dressing of the wound was done and antibiotics administered were intravenous metronidazole (20 mg/ kg/ day in 3 divided doses) and ceftazidime (100 mg/ kg/ day in 3 divided doses). Child was referred to University College Hospital, Ibadan, a teaching hospital in a neighbouring state where skin grafting was performed. Presently, child have recovered and he is fully healthy.\nA causality assessment was conducted by the state AEFI committee using the detailed AEFI investigation forms using WHO AEFI causality assessment methodology [, ].Visits were made to the private hospital where the child was reported to have received the vaccine. The routine immunization focal person in the facility was interviewed. Assessment of available cold chain devices for vaccine storage was also carried out. The knowledge and skills of health workers in vaccine handling, management and administration were assessed [–]. In addition, the caregivers of two other children immunized during the session were recalled and interviewed. The case of interest was the first child to be vaccinated with measles vaccine during the immunization clinic while the second child, a 9 months old female who received vaccination from the same measles vial had fever and abscess formation at the site of immunization only however, the third child who was also vaccinated during the immunization clinic was healthy and without symptoms. The third child was found to be vaccinated with measles vaccine from a newly reconstituted measles vaccine vial different from the measles vaccine vial used for the other two children on the day of the immunization clinic. Incision and drainage procedure was carried out for the second child with wound dressing conducted for two weeks who thereafter recovered fully.\nThe findings from the investigation indicated that a programmatic error may have been responsible for the reactions.We found that two children were vaccinated with a measles vaccine that have been reconstituted for a period of > 6 h. The measles vaccine administered to these children was reconstituted 7 days ago and used during the previous immunization clinic with the left-over stored in a refrigerator within the hospital. This was due to poor knowledge and skill in vaccine management and administration among health workers who administered the vaccine. Other key issues identified includes poor documentation of vaccination activities using the recommended data management tools resulting in difficulty to tracked other children vaccinated with other vaccines for further investigation and poor vaccine storage system at the private hospital as the hospital lacks the recommended Solar Direct Drive (SDD) refrigerator for proper vaccine storage. Also, effort to retrieve the samples of the left-over doses of the vaccine in the opened vials for laboratory investigation proved abortive as the used/empty vial of the vaccine was said to have been discarded by the health workers immediately after the immunization clinic. Furthermore, blood samples collected from the child with NF by the attending physician during the preliminary case management at a local hospital for microbiological culture investigation shows contamination of culture plate as samples were not properly stored during the culture process due to lack of the required facility to perform the test at the hospital.
In 2006, a 3-month-old male infant was examined by Ioannis G. Koutlas for progressive enlargement of the middle aspect of the mandible. Initially, relationship of the infant to any of the siblings described above was unknown to the examiner. Upon further discussion of the striking resemblance of the lesion to the reported patients by El Deeb et al. () and the rarity of this congenital lesion, a direct familial relationship to the previous cases was established. Prior to being seen in the clinic, the infant had radiographic evaluation and incisional biopsy at a local hospital diagnosed as fibrous dysplasia. Histologic preparations revealed an essentially similar benign fibro-osseous process (Figure ).\nClinical evaluation of the patient's mother (patient II-3) did not reveal any clinical lesions. There was only transposition between the right mandibular canine and first premolar. There was no evidence of radiographic abnormality. A follow-up of the male infant was recommended. At 12 (Figure ) and 36 months the lesion was still present, albeit smaller. The decrease in size was apparently relative to the normal increase in size of the mandible. In addition, at 12 months, left sided limb enlargement was noticed (approximately 1 cm leg size discrepancy) as well as slight asymmetry between the right and lefts hands and right and left feet. The patient underwent a complete skeletal survey at age 13 months. Besides the mandibular lesion there were no other osseous abnormalities present to suggest a similar dysplastic process in other bones. The left femur and tibia were minimally larger than the right but with no intrinsic bone abnormality. Follow up radiographic evaluation of the chest, pelvis, hands and feet including bone length studies have been undertaken from 2007 to 2011. The last bone length study confirmed the right lower extremity measuring 54.4 cm from the superior femoral head to the tibial plafond while the left lower extremity measured 55.3 cm. The left femur was 0.5 cm longer than the right, and the left tibia also 0.5 cm longer than the right. During this period of 5 years, bilateral coxa valga deformities were also appreciated; however, they showed improvement with time. Chest evaluation did not reveal any abnormalities of osseous structures. Since other affected members did not report or reveal, after clinical evaluation, such clinical limb anomalies, these findings are most likely unrelated to the jaw lesion.\nIn order to examine if BMP binding protein TWSG1 was present in either bone cells or fibrous tissue within the lesion, sections of lesional tissue obtained from this patient were evaluated by immunohistochemistry with monoclonal antibody against human TWSG1. Immunoreactivity was observed in a mosaic pattern within the lesion with some groups of cells stained strongly and other groups exhibiting low to no staining (Figures ). The staining was cytoplasmic and detected in fibroblasts, osteocytes and the strongest staining was seen in osteoclasts.
A 53-year-old male is admitted late morning for severe chest pain, with interscapular irradiation, associated with nausea and diaphoresis. He had been complaining of the same pain for a month and a half prior to presentation. Symptoms occurred sporadically during the day, lasting from several minutes to sometimes a bit less than an hour, at rest with no association with effort. He is known to have coronary artery disease (CAD), for which he underwent percutaneous angioplasty (PTCA) 1.5 years earlier on a tight lesion of the proximal LAD. A cardiac catheterization was performed 2 weeks prior to admission for the same complaints and revealed nonsevere coronaries lesions. We note that the patient reported the same quality of pain prior to his PTCA with relief of symptoms after revascularization at that time. An electrocardiogram (ECG) was performed during pain episode and was normal with no ST-segment changes (). Due to the high clinical suspicion of an angina and the low clinical probability of alternative diagnosis, the patient was given 5 mg of sublingual NTG, resulting in complete relief of symptoms after about 5 minutes. Cardiac enzymes were drawn and reported as normal, with no other significant blood analysis findings. The patient was admitted to the hospital for investigations.\nSeveral hours after admission, the patient suffered from another identical episode of chest pain, with normal ECG during the pain crisis, and again pain was relieved after administration of 5 mg of sublingual nitroglycerin. Cardiac enzymes were also reported as negative 8 hours after admission.\nSubsequently, a cardiac catheterization was opted for, due to high suspicion of an acute coronary syndrome (ACS), keeping in mind that our patient had already undergone a catheterization 2 weeks earlier.\nCoronarography was performed, showing nonstenotic coronary lesions at first, but after several contrast dye injections, the atrioventricular (A-V) groove branch of the circumflex artery showed a near total occlusion. The patient was asymptomatic at that time, and no ST-segment changes were noted the continuous ECG monitor in the catheterization lab. An angioplasty was elected for to revascularize the critical lesion. A guiding wire was inserted into the left coronary, and 2 mg of intracoronary NTG were given before further advancing the wire. This led to a complete relief of the previously detected lesion; so, the procedure was halted, and the diagnosis of vasospastic angina (VSA) was retained (Figures and ).\nThe patient was discharged on maximal dose of calcium channel blockers (CCBs) and sublingual NTG as needed. He was followed up at 2 weeks and then after a month with total resolution of symptoms.
A 63-year-old man was hospitalized for poor general condition accompanied by intensifying constipation and was referred for treatment of severe lower abdominal pain. His visual analog scale score was 9 out of 10. He had terminal pancreatic tail cancer, and comorbidities included diabetes mellitus, bronchial asthma, and hyperlipidemia. It was difficult for him to lie prone because of a surgery on the right hip that he had undergone 15 years ago. Unlike most patients with pancreatic cancer, the patient complained of lower abdomen pain, especially on the left side. He described his pain as achy and crampy and diffusely located in his left lower abdomen. The abdomen was distended slightly, and bowel sounds were significantly decreased. He was taking a lot of narcotic analgesics, which made him drowsy and lethargic and worsened his constipation. Simple abdomen X-ray imaging showed gas and abundant feces in the colon loops. Compared with abdominal CT findings obtained 50 days prior, hepatic metastasis and peritoneal seeding were still present, infiltration to the tissues around the pancreas and retrogastric area was increased, and most of the abdominal aorta was encased. In addition, metastatic lymph nodes were identified in several areas on the left including the left para-aortic area. However, on CT images, we could not identify the lesion that caused the pain. Nevertheless, owing to the patient's condition, we had to find a way to reduce the pain while reducing the dose of narcotic analgesics. Therefore, according to the patient's complaints, we decided to administer an inferior mesenteric plexus block instead of a celiac plexus block. However, the patient could not lie in the prone position at all.\nAt the outset, it was important to reduce the patient's lower abdominal pain by first performing epidural PCA so that the prone position was possible. Epidural PCA was performed under fluoroscopy guidance in the left lateral recumbent position. Fortunately, the patient's pain somehow decreased after epidural PCA, but epidural PCA alone did not control the patient's pain to a tolerable level. After 2 days of epidural PCA, it was still impossible for the patient to lie prone completely, so we proceeded with the procedure by using a cushion and blanket to make the patient feel less uncomfortable during the procedure. For safety, we started the procedure after confirming that the patient's posture would be maintained during the procedure. In addition, because of the considerable anatomic deformation owing to the extensive growth, invasion, and metastasis of the tumor in the abdominal cavity, we proceeded with CT guidance, instead of fluoroscopy.\nPrior to the procedure, enhanced abdominal CT confirmed that the target site, inferior mesenteric artery, was located at the L3 level (Fig. ). To find the most optimal skin entry point for the procedure under monitored anesthesia care, a metal wire was attached to the back, and CT was performed from the midportion of the L1 vertebral body to the midportion of the L5 vertebral body (Fig. A). On the axial CT fluoroscopy image, the skin entry point was selected, and a virtual line was drawn to the inferior mesenteric artery, which can be reached safely by avoiding the kidney (Fig. B). The skin entry point was marked at approximately 2 cm away from the metal wire toward the midline of the body, and the skin was sterilized aseptically and anesthetized using 2% lidocaine. Under the guidance of an intermittent axial CT fluoroscopy image, a 15-cm-long 22-gauge Chiba needle was slowly advanced toward the target point (Fig. C). After confirming that the Chiba needle arrived at the target point, 1 mL of radiocontrast was slowly injected, and CT scan was then performed to confirm that the needle tip was not located in the blood vessel. After an additional 3 mL of radiocontrast was slowly injected, CT scan was performed again to confirm whether the radiocontrast completely covers the area around the inferior mesenteric artery. If chemical neurolysis was required, we pre-emptively used 10 mL of 1.0% lidocaine to check whether the motor block was possible and 10 mg of triamcinolone to prevent a sudden increase in blood glucose in this patient with diabetes. The mixture of these 2 agents was slowly injected while checking the patient's response. After the administration of all the necessary drugs, CT scan was performed again to check the extent of the drug spread. The extent of the drug spread is shown in Figure . The patient was moved to the recovery room, where he was observed for 2 hours. There were no abnormal findings; therefore, the patient was sent back to the hospital room and the procedure was completed.\nThe patient did not show any side effects, and his visual analog scale score was reduced to 4. After the procedure, the patient's pain became tolerable using the fentanyl patch 75 mcg/hour only, except for the intermittent use of intravenous narcotic analgesics to control breakthrough pain. If the patient's pain increased and became worse within a few days, chemical neurolysis with alcohol was planned, but the patient did not complain of the same extreme pain as before for 1 week after the procedure. Both the patient and his family wanted to go to a nursing hospital near their house, which was more convenient for patient care. Because the patient's lifespan was not expected to be long, we only explained that neurolysis might be necessary, but did not implement it, and sent the patient to the desired hospital. The patient died after 3 weeks without complaints of severe pain as before.\nApproval of this study was waived from the Ethics Committee of Kyungpook National University Chilgok Hospital, based upon their policy on case reports. The authors obtained written consent from the patient to publish this case report.
A 9-month old boy presented at a hospital in a south western state of Nigeria, with a swollen left upper arm adjoining the chest, low-grade continuous fever (38.1 °C), frequent passage of loose watery stool and persistent cries for more than 3 h. Child had been immunized about 24 h earlier. The mother reported that the symptoms were observed 2 h after the child was vaccinated with the measles vaccine at a private hospital. The child was one of three children reported to have been vaccinated with measles vaccine at a private hospital during the immunization clinic session.\nOn examination, he was mildly pale, febrile and anicteric. He was moderately dehydrated; mildly dyspnoeic with normal heart sound, heart rate of 148 beats/ min, breath sound was vesicular and respiratory rate of 54 cycles per minute. He was well nourished as the weight was appropriate for age. There was extensive swelling with skin discolouration (hyperemia) involving the entire left upper arm, sparing the distal third of the forearm and hand. There was also swelling of the upper part of the anterior chest wall. The swelling was firm and mildly tender. There was no history of adverse reaction to immunization or any form allergic reaction.\nA day after admitting the child, extensive erythema of the left upper arm and anterior area of the chest was observed with extensive scalded skin lesion involving the deltoid area, the upper chest wall and arm (Fig. ). Desquamation of the affected areas was observed presenting like severely burned skin from a hot liquid. There was darkening and hardening of the skin over the affected area on the arm with eventual severe necrosis up to a depth of about 5 mm thereafter (Figs. and ). A diagnosis of severe necrotizing fasciitis was made.\nRadical debridement of necrotic tissues was carried out under general anaesthesia. Child was also transfused with blood. Daily dressing of the wound was done and antibiotics administered were intravenous metronidazole (20 mg/ kg/ day in 3 divided doses) and ceftazidime (100 mg/ kg/ day in 3 divided doses). Child was referred to University College Hospital, Ibadan, a teaching hospital in a neighbouring state where skin grafting was performed. Presently, child have recovered and he is fully healthy.\nA causality assessment was conducted by the state AEFI committee using the detailed AEFI investigation forms using WHO AEFI causality assessment methodology [, ].Visits were made to the private hospital where the child was reported to have received the vaccine. The routine immunization focal person in the facility was interviewed. Assessment of available cold chain devices for vaccine storage was also carried out. The knowledge and skills of health workers in vaccine handling, management and administration were assessed [–]. In addition, the caregivers of two other children immunized during the session were recalled and interviewed. The case of interest was the first child to be vaccinated with measles vaccine during the immunization clinic while the second child, a 9 months old female who received vaccination from the same measles vial had fever and abscess formation at the site of immunization only however, the third child who was also vaccinated during the immunization clinic was healthy and without symptoms. The third child was found to be vaccinated with measles vaccine from a newly reconstituted measles vaccine vial different from the measles vaccine vial used for the other two children on the day of the immunization clinic. Incision and drainage procedure was carried out for the second child with wound dressing conducted for two weeks who thereafter recovered fully.\nThe findings from the investigation indicated that a programmatic error may have been responsible for the reactions.We found that two children were vaccinated with a measles vaccine that have been reconstituted for a period of > 6 h. The measles vaccine administered to these children was reconstituted 7 days ago and used during the previous immunization clinic with the left-over stored in a refrigerator within the hospital. This was due to poor knowledge and skill in vaccine management and administration among health workers who administered the vaccine. Other key issues identified includes poor documentation of vaccination activities using the recommended data management tools resulting in difficulty to tracked other children vaccinated with other vaccines for further investigation and poor vaccine storage system at the private hospital as the hospital lacks the recommended Solar Direct Drive (SDD) refrigerator for proper vaccine storage. Also, effort to retrieve the samples of the left-over doses of the vaccine in the opened vials for laboratory investigation proved abortive as the used/empty vial of the vaccine was said to have been discarded by the health workers immediately after the immunization clinic. Furthermore, blood samples collected from the child with NF by the attending physician during the preliminary case management at a local hospital for microbiological culture investigation shows contamination of culture plate as samples were not properly stored during the culture process due to lack of the required facility to perform the test at the hospital.
An 85-year-old Caucasian male was admitted after sudden onset of expressive aphasia and weakness in both legs lasting 20 seconds. He was athletic, self-reliant and had no cognitive impairment. During the last 28 years, he had experienced 8-10 heterogeneous episodes of acute neurological symptoms, such as central facial palsy, hemiparesis, and non-fluent aphasia, lasting from seconds to 3-4 hours. Precerebral duplex and electrocardiography (ECG) were performed several times with normal results, and EEG registration and 24-hour Holter monitoring had been normal. Previous MRI scans showed no abnormal restricted diffusion, as seen in acute cerebral infarcts, but infarct sequelae in the left temporal lobe and both thalami. Several years later, three additional infarct sequelae were detected in the cerebellum. The patient was treated with platelet inhibitors, and medications and dosages were adjusted after new episodes. There was no suspicion of lack of compliance. Except from age, migraine, and previous smoking, with cessation 35 years ago, he had no known risk factors for cerebrovascular disease. On the current admission, he presented with reduced motor speed in his left arm and leg. Electrocardiography and Holter monitoring showed no signs of atrial fibrillation. CT and MRI revealed multiple, cortical infarct sequelae in the anterior and posterior circulation territories of both hemispheres, and MRI also detected two acute embolic infarcts in the right occipital lobe and one in the left parietal lobe (Figure ). CT and MRI angiograms and duplex sonography did not show significant plaques or stenoses, and pre- and intracerebral flow were normal with asymmetrical vertebral arteries, which were considered a normal anatomical variant. Cortical infarcts in several vascular territories strongly suggest cardioembolic etiology, but transthoracic echocardiogram showed no cardiac sources of emboli, and there was no sign of left atrial enlargement, which may be seen in the presence of atrial fibrillation. The patient concurred to further diagnostic tests aiming to determine the cause of recurrent cerebral emboli, although he was informed that the results would not necessarily alter treatment recommendations. We performed a transcranial Doppler (TCD) bubble test with 10 mL air-mixed saline injected into the left cubital vein while the left middle cerebral artery was insonated with a 2-MHz probe. Injection at resting state produced no microembolic signals, while injection after Valsalva maneuver resulted in a shower of microembolic signals followed by single signals persisting for over 30 seconds. The result implied the presence of a latent right-to-left shunt, and transesophageal echocardiography verified a large patent foramen ovale (PFO; Figure ). In agreement with the patient, we decided on non-operative treatment. Due to previous failure of antiplatelet treatment, we changed to a direct oral anticoagulant (dabigatran 110 mg twice daily), intended as a lifelong treatment. He had no subjective complaints at discharge.
A 62-year-old man presented at the Neurology consultation with a six-week history of a severe, strictly left orbitotemporal headache, with a frequency of three attacks per week, occasionally more than one at the same day. Most of them occurred in the first half of the night, waking him up, and lasted between thirty minutes and one hour. He used to take ibuprofen as acute treatment, with unsatisfactory response, since he did not notice a significant difference between treated and untreated attacks in terms of duration and pain intensity. To relief the pain, he used to open the window to get some fresh air. The headache was always associated with ipsilateral conjunctival injection and lacrimation. Pain triggers were not identified by the patient. He had no personal or familial history of headaches. His medical history was remarkable for hypertension and asthma, with a past surgical history including septoplasty and bilateral middle turbinectomy and uncinectomy due to nasal respiratory insufficiency. By the time of medical evaluation he was asymptomatic and neurological exploration was unremarkable. The clinical picture was suggestive of a CH and the patient was medicated with verapamil 120 mg daily. A MRI scan was performed, which revealed a sphenoid sinus mucocele, without secure expansion of the sinus. Two weeks later the patient came to the Emergency Department with complaints of horizontal diplopia that he noted when he woke up in that morning. He maintained the headache attacks, with similar characteristics, despite prophylactic therapy. Neurological examination revealed left eye adduction palsy and ptosis. A brain CT scan was performed and excluded lesions other than the mucocele. Paranasal sinus MRI revealed molding of the medial wall of left cavernous sinus by the sphenoid mass (). A paranasal sinus CT scan was also performed to allow for a better characterization of the lesion, showing sclerosis and interruption of the roof and posterior wall of the left sphenoid hemisinus (). The patient was submitted to surgical drainage of the mucocele by transnasal-transphenoidal approach, with complete resolution of the adduction impairment, persisting a mild left eye ptosis. After the surgery the attacks stopped, and in the six-month follow-up he reported no further attacks.
The patient is a 76-year-old woman with a two-year history of left elbow pain empirically diagnosed as gout. When her symptoms failed to improve with appropriate management, radiographs were obtained, demonstrating a lesion in the proximal radius () characterized as a mildly expansile lucent lesion with a thin zone of transition but no sclerotic rim. Internal osseous septations were present and there was cortical thinning but no visible cortical breakthrough, periosteal reaction, calcified matrix, or soft tissue mass. The initial differential diagnosis included metastasis, multiple myeloma, and other less common entities such as a primary sarcoma of bone or atypical infectious process. She was referred to our tertiary care hospital to consult with an oncologic orthopedic surgeon. Further history obtained at that clinic visit elicited that 3 years previously she had incidentally discovered lytic lesions in her skull and left clavicle that were evaluated in another medical system. Biopsy of both lesions performed at that time was inconclusive showing a mix of inflammatory and fibrous cells per report. The pathologic specimens were not available for further review. Physical exam at her clinical visit was unremarkable with no palpable lymphadenopathy and no visible abnormality at the symptomatic left elbow. SPEP and UPEP tests were negative.\nHer initial imaging work-up included CT of the chest, abdomen, and pelvis; contrast-enhanced MRI of the left forearm; and nuclear medicine bone scan. Her CT scan showed no findings of primary malignancy and—pertinent to her eventual diagnosis—showed no lymphadenopathy or vital organ abnormality. Bone scan demonstrated marked radiotracer uptake at the site of the lytic lesion in the proximal left radius as well as at the previously biopsied skull and left clavicle lesions (). The MR scan of the left forearm showed a marrow replacing lesion within the proximal diaphysis of the radius (). The lesion was T1 isointense, T2 hyperintense and demonstrated avid enhancement. Cortical thinning and small areas of cortical breakthrough not visible on the radiographs were apparent on the MRI. No associated soft tissue mass or perilesional edema was present.\nAt the request of the orthopedic oncologist, a fluoroscopy-guided percutaneous biopsy was performed by Musculoskeletal Interventional Radiology. This rendered only tiny fragments of tissue that were nondiagnostic at histologic review. The patient then underwent open biopsy and curettage of the lesion with Orthopedic Surgery for both diagnostic and treatment purposes. Lesion histology demonstrated features diagnostic of RDD including emperipolesis (engulfment of intact lymphocytes contained with the cytoplasm of histiocyte cells) and positive S100 immunohistochemical staining (). At her follow-up clinic visit 8 weeks after surgery, the patient reported resolution of her left elbow pain, and repeat radiographs demonstrated partial filling in of the lesion with healing bone (). She was discharged from clinic and instructed to follow up if she developed recurrent left elbow symptoms or similar symptoms at a new site. One year later, she has not sought further care at our institution.
The patient was an 83-year-old South Korean woman with locally metastatic gastric cancer and a gastroduodenal ulcerative mass. Along with the primary diagnosis, the patient also had significant comorbidities. These included peripheral vascular disease status post prior stroke and an enlarged abdominal aortic aneurysm. At the time of presentation, the patient was chronically weak, had a poor appetite, and chronically bled from her gastroduodenal tumor. Following admission, the patient was planned to undergo a laparoscopic surgery for the placement of feeding jejunostomy tube and gastrostomy tube. This was followed by chemoradiation therapy for palliation of bleeding and to prevent gastric outlet obstruction.\nBesides these interventions, the patient had also been evaluated by the vascular surgical service for her abdominal aortic aneurysm. The patient had previously undergone endovascular stenting, but, had a contained endoleak related to her aneurysm and stent procedure. The patient was at relatively high risk for continued leak and rupture. However, due to patient’s comorbidities and advanced cancer process, the treatment options were limited, thus, neither open repair nor repeat endovascular repair was considered indicated or feasible at that time.\nCode status was discussed extensively with the patient and her son, who was a cardiologist. The patient had clearly expressed her preference in favor of DNR status, including avoid attempts at resuscitation if she went into cardiopulmonary arrest. The patient consented to electrical cardioversion, but she did not consent to chest compressions and prolonged orotracheal intubation if these were to be deemed futile by her treating physician. The patient’s son expressed his support for his mother’s previously expressed wishes in favor of electrical cardioversion despite DNR status and stated that the two of them had had prior conversations about this issue and her feelings had not changed. Therefore, in accordance with the patient’s wishes and in line with the professional recommendation of the attending doctor, a DNR order was signed. Following hospital discharge, the patient had visited the ED multiple times to receive blood transfusions for her chronically bleeding ulcerative malignant mass. In the following months, code status was again discussed with the patient and her family members, wherein, they all remained resolute that their wishes for DNR order had not changed.\nThis case was challenging because the patient and her physician son demanded cardioversion and other aggressive treatment measures, while, not wanting his mother to have chest compressions or intubation in case of emergency. In practice, aggressive treatment measures such as advanced cardiac life support algorithms are difficult to undertake without intubation and are rarely effective []. The goal of electrical cardioversion is to preserve life, restore health, and limit disability. None of those goals could be achieved given her advanced disease status. It was very difficult to convey this to the elderly patient and her grieving son. Despite the fact that the patient’s son was a physician, he was too emotionally invested to acknowledge or accept the futility of the requests of his mother and himself to the medical team.
The patient is a 76-year-old woman with a two-year history of left elbow pain empirically diagnosed as gout. When her symptoms failed to improve with appropriate management, radiographs were obtained, demonstrating a lesion in the proximal radius () characterized as a mildly expansile lucent lesion with a thin zone of transition but no sclerotic rim. Internal osseous septations were present and there was cortical thinning but no visible cortical breakthrough, periosteal reaction, calcified matrix, or soft tissue mass. The initial differential diagnosis included metastasis, multiple myeloma, and other less common entities such as a primary sarcoma of bone or atypical infectious process. She was referred to our tertiary care hospital to consult with an oncologic orthopedic surgeon. Further history obtained at that clinic visit elicited that 3 years previously she had incidentally discovered lytic lesions in her skull and left clavicle that were evaluated in another medical system. Biopsy of both lesions performed at that time was inconclusive showing a mix of inflammatory and fibrous cells per report. The pathologic specimens were not available for further review. Physical exam at her clinical visit was unremarkable with no palpable lymphadenopathy and no visible abnormality at the symptomatic left elbow. SPEP and UPEP tests were negative.\nHer initial imaging work-up included CT of the chest, abdomen, and pelvis; contrast-enhanced MRI of the left forearm; and nuclear medicine bone scan. Her CT scan showed no findings of primary malignancy and—pertinent to her eventual diagnosis—showed no lymphadenopathy or vital organ abnormality. Bone scan demonstrated marked radiotracer uptake at the site of the lytic lesion in the proximal left radius as well as at the previously biopsied skull and left clavicle lesions (). The MR scan of the left forearm showed a marrow replacing lesion within the proximal diaphysis of the radius (). The lesion was T1 isointense, T2 hyperintense and demonstrated avid enhancement. Cortical thinning and small areas of cortical breakthrough not visible on the radiographs were apparent on the MRI. No associated soft tissue mass or perilesional edema was present.\nAt the request of the orthopedic oncologist, a fluoroscopy-guided percutaneous biopsy was performed by Musculoskeletal Interventional Radiology. This rendered only tiny fragments of tissue that were nondiagnostic at histologic review. The patient then underwent open biopsy and curettage of the lesion with Orthopedic Surgery for both diagnostic and treatment purposes. Lesion histology demonstrated features diagnostic of RDD including emperipolesis (engulfment of intact lymphocytes contained with the cytoplasm of histiocyte cells) and positive S100 immunohistochemical staining (). At her follow-up clinic visit 8 weeks after surgery, the patient reported resolution of her left elbow pain, and repeat radiographs demonstrated partial filling in of the lesion with healing bone (). She was discharged from clinic and instructed to follow up if she developed recurrent left elbow symptoms or similar symptoms at a new site. One year later, she has not sought further care at our institution.
A 40-year-old Sinhalese woman who was previous healthy presented with a history of intermittent colicky left-sided abdominal pain for 2 months’ duration. There were no other lower gastrointestinal symptoms, loss of weight or loss of appetite. She was hemodynamically stable. There was no palpable abdominal mass or evidence of peritonitis, and the rest of the history and examination were unremarkable. She did not have a significant family history of illnesses. She was unemployed. Basic biochemistry, which included complete blood count and renal and liver profile, was within normal parameters. Colonoscopy revealed a narrowing of the lumen of the descending colon a few centimeters distal to the splenic flexure. Biopsy showed evidence of resolving infective colitis, with no evidence of malignancy. Computed tomography of the abdomen and colonogram showed a circumferential thickening of the wall and narrowing of the lumen of the descending colon extending from 3 cm below the splenic flexure downwards, with evidence of extraluminal spread through the serosa and infiltration of adjacent parietal peritoneum and abdominal wall, suggestive of a stage IV neoplasm. There was no evidence of abdominal metastasis.\nSurgical exploration and excision of a probable malignancy was planned. Exploratory laparotomy revealed a mass lesion with multiple abscesses attached to the transverse and descending colon with a narrowed segment at the proximal descending colon. The mass was attached to the omentum and pericolic fat. Multiple enlarged lymph nodes along the inferior mesenteric artery were noted. The patient underwent extended left hemicolectomy with end-to-end colocolic anastomosis.\nThe surgical specimen consisted of transverse colon and descending colon together measuring 370 mm in length and 50 mm in diameter. The lumen of the descending colon was narrowed in a region 130 mm from the proximal resection margin. A mass lesion with multiple abscesses measuring 90 x 70 x 45 mm was attached to the transverse and descending colon at the narrowed segment.\nMicroscopic examination of sections of the mass lesion showed multiple abscesses surrounded by a fibroblastic reaction and a mixed inflammatory infiltrate with foreign body type giant cells. Occasional abscesses contained a few basophilic colonies suggestive of actinomycosis. The lymph nodes showed reactive changes. There was no evidence of tuberculosis, inflammatory bowel disease or malignancy (Figs. , ).\nThe patient received intravenous penicillin treatment for 10 days followed by oral penicillin for 6 months after reviewing with histology. There were no complications related to surgery. Follow-up assessment after 1 year revealed that the patient was free of symptoms, with no clinical evidence of bowel obstruction.
A 58-year-old man was found to have intrahepatic and extrahepatic bile duct dilatation on B ultrasound on 12 June 2009, and was hospitalized for further examination and treatment on 20 June 2009. Our patient was 171 cm tall and weighed 86 kg. There was no anemia or jaundice in the palpebral or bulbar conjunctivas. The superficial lymph nodes were not palpable. The abdomen was flat without any palpable mass. He had had diabetes and hypertension that had been under regular medical control for the past 20 years. Abdominal ultrasonography was carried out and a 4.3 × 5.2 cm protruding tumor was found in the lower pole of the right kidney. The laboratory test results showed total bilirubin was 20.6 μmol/l and direct bilirubin was 10.3 μmol/l, the results of a magnetic resonance imaging (MRI) scan showed (1) a lesion in the lower pole of the right kidney, (2) cysts in the left renal area and (3) abnormal signals in the ampulla of Vater. Intrahepatic bile duct dilatation could be seen on the MRI scan, but the head of the pancreas and distal bile duct showed no tumor signals leading to a diagnosis of periampullary carcinoma and right renal carcinoma. Considering the trauma of pancreaticoduodenectomy combined with renal resection operation is greater, and laparoscopic resection of renal tumors is feasible with fast recovery, after weighing the risks of endoscopic retrograde cholangiopancreatography (ERCP) we carried out the laparoscopic right renal radical resection first without carrying out ERCP. After general anesthesia, our patient was operated on via a 2 cm skin incision under the costal margin on the right posterior axillary line; a retroperitoneal CO2 artificial pneumoperitoneum was established, the anatomical landmarks were observed under endoscope, the right renal artery was dissected, clipped with a blood vessel clip and cut. Then, the renal vein was dissected and cut, the adrenal gland was retained and the ureter was cut; the right kidney was then resected. Pathological examination results were as shown in Figure\na. The clinical stage and TNM staging of the renal tumor was T1N0M0 and Robson stage I. Our patient recovered well and was discharged on the ninth postoperative day. Then, our patient presented with abdominal pain, fever, and body weight loss of about 10 kg the following month. He was hospitalized for further examination and treatment on 31 July 2009.\nERCP showed that an infiltrative ulcerative mass was visible in the duodenal descending part and papilla involving the lumen half cycle; the mucosa was ulcerative and the intestinal wall was stiff. No nipple and wrinkled wall structure could be seen. We tried to carry out nipple angiography but were not successful. Papillary carcinoma of the duodenum was diagnosed (Figure\nb). Our patient presented fever and other symptoms of cholangitis at this time. Percutaneous transhepatic cholangiography drainage was carried out under the guidance of computed tomography (CT) (Figure\na,b). Endoscopic views of the duodenum are shown in Figure\n. Postoperative anti-infection treatment (perazone sodium and sulbactam sodium) was applied. Our patient recovered well, then 30 days later the pylorus-preserving pancreaticoduodenectomy was carried out; the type was determined to be pancreatic duct jejunum anastomosis. However, postoperative intra-abdominal infections occurred on the sixth day and bleeding occurred in the peripheral arteriolar branch around the anastomosis on the eighth day; our patient improved after vascular interventional microcoil embolization for the treatment of hemostasis. The second operation was performed 14 days latter. A large number of dark red blood clots were seen on abdominal cavity exploration, mainly concentrated in the liver, gastric and pancreatic body and tail. Celiac necrotic tissues and blood clots were eliminated. At the same time, the gastrointestinal anastomosis had an approximately 1 cm split and bile was leaking from the biliary intestinal anastomosis, so jejunal fistulization was performed and peritoneal lavage and drainage was carried out repeatedly. The clinical stage and TNM staging of the pancreatic tumor was stage 1B and T2N0M0. Our patient improved gradually without undergoing chemotherapy and was discharged on the 58th postoperative day. There has been no tumor recurrence after a follow-up of 26 months.
A 53-year-old woman presented to our department in June 2011 with a left preauricular swelling. Her chief complaint was that the swelling had been slowly growing for about 5 months. Additionally, she complained of pain in the left TMJ area; the ability to open her mouth was limited, and each time she opened her mouth widely, she felt a clicking sensation in the left preauricular area. The patient had no history of trauma, rheumatoid arthrosis, or septic arthritis. She revealed that her left TMJ had become dislocated about 2 years previously, and the patient had repositioned it herself; however, there had been no subsequent recurrence. Before she presented to our department, she visited a local hospital for treatment and underwent a computed tomography (CT) scan of the TMJ region (). Because this was the first time the clinicians at that hospital had encountered such a case, the patient was referred to our hospital. Physical examination revealed a preauricular immovable and tender mass measuring approximately 20 × 30 mm, with medium to somewhat soft firmness. Her maximal mouth opening was limited to 25 mm, with slight deviation to the left side. No facial nerve paralysis or hearing disturbance was detected.\nA CT scan showed multiple small radiodense particles surrounding the left condyle, especially in the anterior region of the left TMJ capsule. Magnetic resonance imaging (MRI) was performed in our hospital to check the condition of the soft tissues surrounding the lesion. This examination showed an expanded high-signal area, including the anterior and lateral space of the left condyle, containing multiple signal-void particles (). The TMJ disc was in the clinically normal position and no perforation was suspected, and the surface of the left condyle was smooth. According to the physical examination and radiological findings, the preoperative diagnosis was SC of the left TMJ. The patient consented to undergo hospitalization and surgical treatment. After being admitted to the inpatient department, the patient underwent arthrotomy under general anesthesia; the preauricular approach was chosen to gain access to the left TMJ. When the TMJ capsule was opened, it discharged a viscous fluid containing many similarly sized opalescent, glistening loose bodies (). Most of the loose bodies were collected, although some were initially sucked into the suction unit. The disc was in the correct position without perforation, and the condylar head was smooth; therefore, only a partial synovectomy procedure was performed at the affected region of the synovium. The cavity was irrigated with a large volume of saline solution. After surgery, we counted up to 400 calcified bodies (), with the largest measuring approximately 8 × 5 mm.\nHistological examination revealed that the excised synovial membrane was chondrometaplastic and that the calcified loose bodies were composed of hyaline cartilage covered by fibrous connective tissue (). The histological findings confirmed the diagnosis of SC of the left TMJ. A postoperative CT scan showed no remaining loose bodies (). The patient recovered well after surgery, and no facial paralysis or hearing loss was noted. At the 3-month follow-up appointment, physical examination revealed no mandibular deviation, malocclusion, or preauricular tenderness, and her maximal incisal opening was 35 mm. We conducted follow-up every year after surgery for 9 years and found no signs of recurrence.
A 50-year-old man presented with difficulty in swallowing and nasal speech. First he had difficulty swallowing solids, which later progressed to ingestion of liquids.\nHe mentioned three episodes of nasal regurgitation of liquids. His consort noticed incomplete closing of his eyes during sleep.\nThe muscles of jaw closure were involved and produced weakness with a prolonged meal time (fatigable chewing). For evaluation of dysphagia, he was referred to an internist for a barium swallow and video fluoroscopy was performed. Benign narrowing of the hypopharynx and pharyngoesophageal junction was detected. He was advised follow-up and was not offered any treatment for stenosis.\nThe symptom of the patient progressed and he was referred to a neurologist. The neurologist recommended magnetic resonance imaging (MRI) of the brain, an electrophysiological test, and prescribed an acetylcholinesterase inhibitor (AChEi), pyridostigmine 60 mg twice a day. The brain MRI was normal and the electrophysiological findings were reported as a bulbar onset of amyotrophic lateral sclerosis. The acetylcholine receptor antibody titer was normal and in a pharmacological testing with neostigmine no improvement in the bulbar symptom was seen.\nAfter three months, gait disturbance and generalized weakness was added to his symptoms and low dose pyridostigmine was continued due to lack of response.\nSeveral months after the start of the symptoms, he was urgently admitted to the Emergency Department of our University Hospital because of respiratory failure and intubated. In the first neurological examination hyperreflexia was detected and in the light of his previous history, motor neuron disease was suspected and supportive care and neuroprotective drugs were started. The patient was referred to the Internal Medicine Service with an impression of aspiration pneumonia. A few days after admission, a neuromuscular consultation and electrodiagnosis was requested. On the first neuromuscular visit, the patient was intubated and mechanically ventilated. He was extremely cachectic due to several months of dysphagia, and profoundly ill on account of the intercurrent infection. Hyperreflexia in the upper and lower limbs was prominent. Nerve conduction studies (NCS) and electromyography (EMG) of the limbs was normal and no evidence of motor neuron disease (MND) was seen. A repetitive nerve stimulation test revealed a significant decremental response in the proximal and facial muscles, with post activation facilitation and exhaustion (23% decremental response in the trapezius muscle and 32% in the nasalis muscles) [Figures and ]. Short-duration polyphasic motor unit action potentials (MUAPs) were detected in the bulbar and proximal muscles, without any neurogenic finding. The electrical findings were consistent with a neuromuscular junction defect. The patient was transmitted to the Neuromuscular Service and treated with plasma exchange, pyridostigmine, and prednisone.\nThe patient recovered dramatically and the bulbar symptoms and limb weakness improved rapidly. He was extubated in a few days and discharged with significant improvement in his functional state. In a follow-up visit there was no evidence of respiratory or bulbar weakness. Ptosis and facial weakness were the prominent signs and the Cogan and peek signs were positive. The acetyl choline receptor antibody and anti-MuSK (muscle-specific Kinase) antibody titers were normal. A chest CT scan revealed an anterior mediastinal mass [] and the patient was referred for a thymectomy. Thymoma type B2 with capsular invasion was detected in the pathological specimen.
This patient is a 37-year-old female who presents to the pain clinic with a chief complaint of right side facial pain and numbness. The patient reports that the pain becomes more and more significant during the day until she feels the pain radiating from the right side of the entire face to the upper neck.\nThe patient has a history of an acoustic neuroma surgery with near total tumor removal. The surgery was performed 10 years ago. She underwent stereotactic radiosurgery for the tumor remnant shortly after surgery. The patient developed right facial palsy immediately after the surgery. She reported that she was having right facial pain and gradually was losing touch feeling on the face area after the stereotactic radiosurgery course was finished. The pain and numbness have persisted ever since. The right face numbness did not improve and the pain intensity did not change during the past nine years.\nThe patient characterizes her pain as a sharp, constant pain and rated it as an 8 out of 10 on numerical pain rating scale (NPR). The pain is occasionally exacerbated with chewing. Recently there was an MRI image performed that showed a small, stable-sized remnant lesion of the prior tumor along the CPA region. The stable size of the tumor during the past nine years did not warrant surgical intervention or further radiation therapy.\nOn physical examination, the patient had complete loss of sensation to light touch and pinprick on the right ophthalmic (V1), maxillary (V2), and mandibular (V3) dermatomes. She had a right side blepharoplasty due to right side complete facial palsy. She was under the multimodality treatment strategy that included a combination of antiepileptic antalgic medication, antidepressant antalgic medication, and opioid medication management with occasional rotation along with applying psychological techniques such as coping, biofeedback, and hypnosis. All the prior efforts including sphenopalatine ganglion block, trigeminal nerve blocks, and trigeminal nerve radiofrequency were unsuccessful in her treatment. With the diagnosis of anesthesia dolorosa, a complicated deafferentation centralized pain, we may consider deep brain stimulation in the future.
A 29-year-old male patient had reported to oral and maxillofacial surgery (OMFS) department with the complaint of bleeding from the oral cavity after the extraction of the mobile right maxillary second molar, for last 10 days []. He had extracted his tooth as it was loose and causing difficulty in chewing. Immediately after extraction, a significant amount of bleeding was noticed through the socket which was unusual to a routine extraction. However, it was arrested with pressure pack.\nOn the fourth day of the extraction, when he was eating he noticed severe bleeding from the socket, and subsequently, he was transferred to a local hospital and was managed conservatively by a pressure pack. After that he was referred to the surgery department of MKCG Medical College, Berhampur. The bleeding was arrested with pressure pack and he was treated with blood transfusions and styptics. All his investigations toward the cause of bleeding were within normal limits. As the bleeding continued, he was transferred to the OMFS department of SCB dental college and hospital.\nWhen he reported to the OMFS department, he was in a low general condition due to prolonged bleeding. On examination of the socket, there was a pulsatile clot present. Even on slight provocation of the socket, bleeding occurred so severely that the entire oral cavity got filled with blood within few seconds. The bleeding was arrested with a ribbon gauge soaked in Whitehead varnish packed into the socket and biting pressure. The patient was admitted for definitive treatment. Over a period of 2 days he was treated with antibiotics, blood transfusions, and palliative measures while diagnostic investigations were being carried out.\nThere was no past and family history of bleeding and coagulative disorders except that the patient had epistaxis on many occasions which went unreported. All hematological investigations performed were within normal limits. An orthopantomogram was performed which revealed a faintly and ill-defined radiolucent lesion extending from the right maxillary central incisor to the posterior maxillary region. A computerized tomographic (CT) scan was also performed which showed a multilocular radiolucent lesion involving the right posterior maxillary region. Finally, an angiogram of the right external carotid artery was performed and a diagnosis of an AVM involving the posterior superior alveolar vessels in the region from the right maxillary canine to the tuberosity area was established [].\nThe patient was planned for external carotid artery ligation with partial maxillectomy under GA. The right-side external carotid artery was explored in the carotid triangle of the neck and was ligated above the level of the superior thyroid artery []. It was followed by partial maxillectomy of the right side through an extraoral approach (by Weber-Ferguson incision). Maxillectomy was carried out with an oscillating saw sparing the zygoma, zygomatic arch, and lateral nasal bone []. On exploration, the anterolateral wall of the maxilla was found to be with small perforations presenting with copious bleeding. Partial maxillectomy was done from canine to the molar region. Subsequently, bleeding was arrested. Intranasal antrostomy was performed and the maxillary antrum was packed with an antibiotic-soaked ribbon gauge. After primary closure, a prefabricated acrylic splint was given and the specimen was sent for a histopathology study. The postoperative recovery was uneventful and the patient was discharged after 7 days of follow-up []. Subsequent follow-up revealed satisfactory wound healing and the histopathology report came as AVM.\nThough an AVM occurs in the orofacial region but this type of malformation involving maxilla is rare and is a challenging entity. Usually the AVM occurs in the soft tissues but intrabony involvement is rare. Four cases of mandibular AVM involving the mental foramen have been reported which were treated by transvenous embolization through the mental foramen with the coils and NBCA (N-butyl-2-cyanoacrylate).[] Another case of intraosseous venous malformation of zygoma was reported by Srinivasan.[]\nVascular malformations are often present at birth (though they may not appear till the later years) and grow insidiously with the patient throughout life and are characterized according to the main vessel type and flow characteristics. In contrast to hemangiomas, they never proliferate or involute, but expand slowly and relentlessly throughout life. Trauma, puberty, and pregnancy can cause periods of accelerated growth.\nVenous malformations are characterized by an abnormal collection of veins with no demonstrative mitotic activity in the endothelial cells or pericytes and often lack a uniform muscle layer. The distinction between a proliferative phase hemangioma and a venous malformation is relatively straightforward, but the distinction between a late stage hemangioma and a venous malformation on the basis of routine histology is difficult. Recent advances in immunohistochemistry, especially the use of GLUT-1, have made the more accurate characterization of these lesions possible. Juvenile hemangiomas consistently express GLUT-1 immunoreactivity whereas vascular malformations consistently fail to express GLUT-1. In the present case, we did not perform GLUT-1 immunoreactivity.\nThe management of these lesions depends on symptomatic control and for aesthetic concerns. In the present case, a decision was made to remove the lesion as it caused severe bleeding. Though various therapeutic options have been described for soft tissue venous malformations, surgical excision remains the mainstay for purely intrabony lesions. If necessary, it may be augmented with preoperative embolization. There have been no reports of recurrence or increased intraoperative bleeding when these lesions have been removed with a margin of normal tissue.
We present a case of a nine-year-old girl who experienced intermittent swelling of her right cheek associated with pain. After being evaluated by her family doctor, dentist, and a private ear, nose and throat (ENT) specialist, she was referred to the ENT Department at the Karolinska University Hospital where clinical examination ruled out parotid gland pathology and laboratory tests did not reveal any specific findings. A CT scan showed signs of osteomyelitis in her right mandible which rendered a referral to the Unit of Cranio- & Maxillofacial Surgery at Karolinska University Hospital where a mild swelling of the right cheek, trismus and a dull pain in the same area was found (). She described two- to three-week-long exacerbations of swelling and pain during the previous year, that recurred every two or three months. Radiology demonstrated increased mandible width, periosteal thickening and no clear difference between cortical and cancellous bone (). Altogether, these findings lead to the decision to obtain a bone biopsy and bacterial culture from the right mandibular body. The biopsy was retrieved under general anesthesia using an intraoral approach with as much isolation as possible to avoid contamination. Adequate isolation was verified by the absence of bacterial pathogens within the bone.\nHistopathological examination showed an appearance of the mandibular bone in accordance with primary chronic osteomyelitis. Culture of harvested bone revealed no presence of bacterial agents ().\nAs depicted in , the patient was prescribed ibuprofen at the time of diagnosis at the Unit of Cranio- & Maxillofacial Surgery for symptom release which had a moderate effect. She was instructed to take this regularly (200 mg 3 times daily) for at least 2 months. Then instructed to take it whenever needed.\nParallel to developing jaw pathology, pubertal signs including breast development and growth acceleration were reported at 8 years of age. This led to a referral to the Pediatric Endocrinology Clinic at the Astrid Lindgren Children's Hospital where clinical evaluation confirmed Tanner stage 3 breast development, growth acceleration, two years accelerated bone age, and pubertal levels of estradiol, luteinizing hormone (LH) and follicle stimulating hormone (FSH). Altogether confirming that the patient had entered puberty at a relatively young age. As requested by the patient and her parents, pubertal suppression treatment was initiated at 9 years of age by administering monthly intramuscular injections of an agonist analogue of gonadotropin-releasing hormone, triptorelin (3.75 mg), a treatment well known to reversibly suppress the hypothalamic-pituitary-gonadal axis. At clinical follow-up six months after starting triptorelin injections, the patient reported that all symptoms of PCO including pain and swelling of the jaw had disappeared within two-three months after initiating the treatment. At follow-up visits 11 and 18 months after initiating the triptorelin treatment, no new periods of swelling or pain from the jaw were reported and no clinical signs of jaw inflammation were noted. Radiology performed 18 months after starting the triptorelin treatment confirmed complete remission of the osteomyelitis ().\nAfter 24 months of pubertal suppression, the triptorelin injections were stopped according to the initial treatment plan. Clinical check-up six months after the last injection with triptorelin revealed no sign of recurrence of the PCO, neither clinically nor radiologically, and sporadic menstrual periods were reported starting approximately four months after the last injection. Hence, one can assume that her serum estradiol had increased to normal pubertal levels six months after the last injection of triptorelin while she showed no signs of recurrence of PCO (). illustrates the series of events.
A 27-year-old female with a previous diagnosis of systemic lupus erythematous (SLE) was admitted to the hospital for management of acute on chronic renal failure diagnosed as lupus nephritis on biopsy. She developed acute respiratory failure on hospital day 18 requiring intubation and mechanical ventilation. Subsequent bronchoscopy supported a diagnosis of diffuse alveolar hemorrhage secondary to her SLE. Plasmapheresis was recommended by the consulting medical services in addition to her standard medical therapy. The patient developed severe acute onset angioedema of the face and oral cavity following completion of her second of five planned apheresis sessions. The consulting rheumatology service attributed the angioedema trigger to administration of fresh frozen plasma (FFP) as part of her apheresis that was later diagnosed as an acquired C1 esterase inhibitor deficiency secondary to SLE. Subsequently, albumin was substituted for FFP in her three remaining apheresis sessions without appreciated aggravation of her edema. Antihistamines were added to the IV Solu-Medrol she was receiving as part of her standard medical therapy. Resolution of her facial angioedema was noted with these conservative measures within a few days; however, her tongue remained severely edematous and protuberant ().\nTracheostomy was completed seven days following onset of her angioedema given her persistent lingual edema and the anticipated prolonged course of mechanical ventilation. The otolaryngology service was contacted 16 days following surgery due to persistent tongue edema for consideration of possible partial glossectomy. Mild bite trauma was noted on the ventral aspect of the tongue but no significant trauma was noted dorsally. Her tongue was not able to be manually reduced. Bite blocks, fashioned out of silk tape and tongue depressors, were placed bilaterally between the premolars and molars at bedside. Vaseline gauze was then applied to the protruded tongue to help prevent desiccation.\nNo improvement in her lingual edema was noted after three days despite placement of the bite blocks. A lingual compression wrap was devised and initiated in an effort to avoid the need for partial glossectomy. First, a light wrap of moistened Kerlix was wrapped around the tongue from the distal tip proximally to the teeth. This was followed with a layer of self-adherent wrap also applied from the tip proximally to the teeth under light tension. A second pass of self-adherent wrap was then added with increased pressure titrated to patient tolerance (). The wrap was replaced once a day and left in place for 12 hours at a time to allow direct inspection of the tissue and help mitigate potential pressure injuries. Significant improvement in the macroglossia was noted after only the first wrap and by day four the tongue was able to be reduced completely within the oral cavity with minimal effort. At this point, the compression wraps were discontinued and the bite blocks continued for two more days until the tongue was able to be retracted into the oral cavity by the patient without external effort. No evidence of lingual trauma secondary to the wraps was observed. The patient's respiratory status eventually recovered and she was decannulated without incident. She was noted with no long-term lingual sequela on subsequent outpatient follow-up visits several months later.
An 81-year-old male patient was referred to our department with dysphonia. There was no history of smoking. A status past multiple myeloma was known in his medical history that was in complete remission at the time of presentation. The patient denied dyspnea, dysphagia, pharyngalgia, and fever. Laryngoscopy revealed a diminished mobility of the right vocal cord and a thickening of the right vestibular fold so that a microlaryngoscopy with tissue sampling was performed. The histological examination of specimens obtained from this region revealed fibrosis. Computed tomography (CT) scans of the neck and the thorax were without any pathologic findings. The patient was discharged to outpatient care.\nThree months later, the patient was admitted with progressive dyspnea along with inspiratory stridor. The clinical examination revealed now a complete paralysis of the right vocal cord and a remaining glottic cleft of only 1 mm due to a supraglottic protrusion of the right vestibular fold. The CT scan () showed now a tumor of the right vocal cord extending to the right piriform sinus.\nAfter tumor debulking in order to expand and secure the airway, the excised material that consisted of several red brown elastic tissue fragments measuring together 24 × 12 × 10 mm was sent for pathological examination. Histologically, one could see tight lymphoid infiltrates. The cells had large nuclei and were irregularly shaped, and the proliferation was strongly enhanced in the staining for Ki67 (50%). The immunohistochemical analyses showed a negative result for CD20 and CD3, and a positive staining for CD138. BCL2 and CD10 were coexpressed (). The clonal light chain restriction for lambda chains substantiated the diagnosis of a multiple myeloma. These results were consistent with laryngeal involvement from the patient's previously diagnosed multiple myeloma.\nThe patient was referred to the Department of Hematology and a systemic therapy with the proteasome inhibitor Bortezomib was discussed. Ultimately, instead of that, a local radiation therapy with 60 Gy was performed. In a control laryngoscopy with tissue sample taken after the radiotherapy, the myeloma could not be verified anymore. The patient is in continuous otorhinolaryngological and oncologic follow-up. To date, almost two years later, no recurrence of the myeloma has occurred so far.
A 67-year-old man with a history of treated prostate cancer, alcohol abuse and alcoholic cirrhosis presented to an urban, academic hospital with new low back pain. The pain had started 2 weeks prior to his presentation, had gradually worsened, and then became associated with bilateral lower extremity shooting pain and weakness. He denied bowel or bladder incontinence or saddle anaesthesia. He also denied subjective fevers or chills or any other bothersome symptoms. He was found to have normal vital signs, poor dental hygiene, tenderness over the lumbar spinous processes, and normal neurological and prostate exams. Magnetic resonance imaging (MRI) of the spine () showed mild compression fractures, with small lytic lesions throughout the thoracic and lumbar spine, and a large lytic lesion at the L5 vertebral body. The patient had a normal prostate specific antigen (PSA) level and a normal white blood cell count. C-reactive protein was mildly elevated at 30 mg l−1 (normal range 0–10 mg l−1).\nThere was concern for metastatic cancer given the patient's prior history of prostate cancer. His normal PSA level was not reassuring, because his prior prostate cancer had been diagnosed in the setting of a normal PSA level. He was started on intravenous dexamethasone 4 mg every 6 hours given the concern for metastatic spinal lesions and a neurosurgical consult was obtained. The neurosurgery team did not feel that urgent surgical intervention was needed and thus a biopsy of the lesion was obtained by the interventional radiology team for diagnostic purposes. Surprisingly, the vertebral biopsy result showed no evidence of malignancy, but did show findings consistent with osteomyelitis and was sent for culture. Dexamethasone was discontinued and blood cultures were drawn. Antibiotic therapy was withheld while culture data was pending, as the patient was clinically stable.\nThe patient was observed while the cultures were pending, and 2 days later Veillonella species started growing from the broth of the tissue biopsy and from one of two blood cultures. After a great deal of prompting for additional history, the patient reported a mechanical fall 4 weeks prior to the onset of his pain, at which time several teeth had been dislodged. A trans-thoracic echocardiogram obtained to evaluate for possible endocarditis was negative. Additionally, repeat blood cultures obtained to ensure clearance of the bacteraemia after the initiation of antibiotics were also negative. Culture sensitivity results were obtained, but were delayed as the sample had to be sent out to a reference laboratory for further testing. In the meantime, ceftriaxone 1 g intravenous daily was started and the patient was discharged to a nursing home for further rehabilitation, with close clinical follow-up scheduled. The sensitivity testing was performed by broth microdilution and showed that there was no β-lactamase inhibition. The MIC results showed sensitivity to meropenem, ampicillin/sulbactam and piperacillin/tazobactam, and borderline sensitivity to metronidazole and penicillin. The results were delivered with the caveat that there are currently no Clinical and Laboratory Standards Institute guidelines in the USA for the performance and interpretation of susceptibility testing for anaerobes other than Bacteroides fragilis. Thus, the advice of the reference laboratory was to interpret these results with caution. At his follow-up appointment, the patient appeared to be having a good clinical response to ceftriaxone. Given the lack of β-lactamase inhibition in the culture data and the fact that once daily dosing of antibiotics decreases disruption of therapies, ceftriaxone was continued. The patient completed a 6 week course of antibiotics and had complete resolution of his symptoms.
An otherwise healthy 15-year-old female soccer player presented to the emergency department with the complaint of bilateral anterolateral leg pain during exercise that has increased in intensity over the past year. Pain typically developed within minutes and remained for long periods of time, sometimes hours, after exercise. The patient stated that the pain has become increasingly worse even with the moderate exertion of walking or climbing steps at school and was consistently worse in the right leg. She experienced numbness previously during exercise as well as zingers radiating through the anterior and lateral compartments, again more noticeable in her right leg. She has been limited in recreational exercise and has stopped playing high school soccer due to increased discomfort. Activity modification and stretching failed to relieve pain.\n(1) Clinical Examination. Upon examination, the patient was 158.75 cm (62.5 in) tall and 72.6 kg (160.1 lbs) and showed stable vital signs. There was no tenderness along any of the four leg compartments; however, she exhibited woody compartments. Upon palpation there was a noticeable fascial defect present in the right anterior leg but not the left. The patient was able to walk with a normal gait, as well as heel and toe walk. She was also able to jump evenly with a full range of motion without pain. She had no signs of knee or ankle instability. Forward bend test was normal, her spine showed no curvature, and her upper extremities were supple.\nBilateral X-rays of ankles and feet revealed no evidence of fracture or dislocation. Bone mineralization was normal and there was no ankle joint effusion. The ankle mortise was intact and there was no evidence of focal soft tissue swelling. She exhibited strong bilateral pedal pulses both during and after exercise.\nShe was referred to the pediatric orthopedic clinic. Lack of improvement with exercise modification along with prolonged pain after exercise led us to consider chronic exertional compartment syndrome (CECS) in addition to medial tibial stress syndrome (MTSS), stress fracture, and popliteal artery entrapment syndrome (PAES) (). Strong pedal pulses with no calf tenderness during or after running allowed us to rule out PAES without the use of ultrasound. Lack of resting pain or evidence of fracture on X-ray ruled out a stress fracture. To further differentiate CECS and MTSS, we performed pressure measurement in clinic using ethylene chloride (Gebauer Company, Cleveland, OH) as a local anesthetic. We used a Stryker Pressure Monitor System wick catheter (Stryker, Kalamazoo, MI) in the anterior compartment after 10 minutes of jogging. Postexercise examination revealed both anterior leg compartments as woody to touch and firmer than the superficial posterior leg compartments. The palpable fascial defect was even more prominent after exercise. Anterior compartment pressure in the right leg was measured to be 99 mmHg (, ), considerably higher than diagnostic indication for fasciotomy which is ≥15 mmHg resting, ≥30 mmHg 1 minute after exercise, or ≥20 mmHg 5 minutes after exercise [].\n(2) Surgical Technique. Prior to operation while the patient was under general anesthesia, a resting anterior compartment pressure measure was taken on the right leg, which indicated a resting pressure of 29 mmHg (, ). This is well outside the normal range for resting compartmental pressure []. A 2.5-inch incision was made over the fascial defect on the right anterolateral leg after the leg was elevated and exsanguinated and the tourniquet was set. Blunt dissection allowed access to the fascia and the intermuscular septum was identified as well as the fascial hernia ().\nThe superficial peroneal nerve was identified as it entered the 2 cm fascial defect (). The nerve was gently dissected distally and proximally until we found normal nerve. The fascial defect was gently opened distally and proximally until there was a release of the lateral compartment to the fibula tip distally and about 3 cm to the upper fibula proximally (). An arthroscopic instrument was used to visualize the fascia release under the skin.\nThe subcutaneous tissues were gently dissected anteriorly until the anterior compartment was identified (). The anterior fascia was opened and then a release of the anterior compartment was done distally to the top of the ankle and proximally to within 3 cm of the knee joint.\nThe superficial peroneal nerve was allowed to fall back into fatty tissue. The subcutaneous tissues were closed with vicryl. The fascia was left open on the anterior and lateral compartments and her compartmental pressure was remeasured and noted to be normal at 6 mmHg (). The skin was closed with monocryl subcuticular running stitch and a sterile compressive dressing was applied.\nThe patient was advised to bear weight as tolerated and to use crutches as needed in the immediate postoperative period. We followed up with our patient at 5 days, 4 weeks, and 3 months. At 5 days following operation, the surgical site was healing well with no signs of infection. She regained full range of motion and appeared neurovascularly intact. At 4 weeks she continued to improve without complications. The surgical site was healing well and without bruising or swelling. Our patient regained strength and self-reported a noticeable improvement in her ability to walk and climb stairs compared to preoperation. The patient was allowed to walk, swim, and ride a bike without great exertion. She was allowed to run once again at 8 weeks' postoperative time. At 3 months following operation, our patient was noticeably better with greatly improved exercise capacity. She has returned to recreational running and playing high school soccer.
A 9-year-old male patient was evaluated at the endodontic clinic of the National University, School of Dentistry, Bogota, Colombia, on March 19, 2014. The patient had no prior medical or dental history. The trauma to his upper front teeth occurred 2 years previously (May 2012), and the initial autoradiogram revealed multiple root fractures at the level of teeth #11 and #21 (). One week after the first appointment, a flexible splint was placed between these 2 teeth, which remained present for approximately 7 months, based on the rationale that the teeth were very mobile and could be lost at any time. The parents were then informed that the prognosis of the case was poor and that extraction was recommended. They decided to request a second opinion (private practice); the splint was removed and endodontic treatment was started using intracanal medication, with calcium hydroxide paste placed on the fracture line in both teeth. This treatment was continued for approximately 1 year; however, the patient continued to have a sinus tract and pain in response to the percussion test, likely associated with necrosis of a coronal fragment, regardless of whether the apical fragment remained vital, which could be assumed due to the continuous apical growth of the teeth ( and ). Given the lack of positive response to treatment, the patient was referred to our endodontic clinic.\nThe first appointment occurred on March 19, 2014. The patient's clinical appearance can be seen in . Both teeth showed a mobility grade of 3, with no periodontal pockets or pain to horizontal or vertical percussion. Sinus tracts were present in both teeth. The pulp vitality, vertical percussion, and horizontal percussion tests were negative.\nRadiographically, multiple fracture lines were observed at the cervical and middle levels of the root, as well as an increase in the periodontal ligament space, a periradicular lesion, and inflammatory external root resorption. These clinical and radiographic findings confirmed the diagnosis of horizontal root fracture with chronic apical abscesses in teeth #11 and #21. Considering the poor prognosis for both teeth, an informed consent form was signed, and a revascularization treatment protocol was initiated using autologous PRP in tooth #11 and conventional revascularization treatment with a blood clot in tooth #21.\nLocal anesthesia was administered with the infiltration technique, using 2% lidocaine with 1:100,000 epinephrine, temporary cement was removed from both teeth, proper isolation and access to the root canals were ensured, and the working length of each tooth was extended up to the fracture line with an apex locator (Root ZX, Morita, Tokyo, Japan) and confirmed with a periapical radiograph (). Profuse irrigation was performed for each tooth with 10 mL of 1.25% sodium hypochlorite (NaOCl) using Endo-Eze Irrigator Tips (Ultradent Products Inc., South Jordan, Utah, USA) without instrumentation. After drying the canals with sterile paper points, a triple antibiotic paste consisting of amoxicillin (500 mg), metronidazole (500 mg), and ciprofloxacin (500 mg) (triturated and mixed in a 1:1:1 ratio with sterile water to obtain a paste-like consistency) was introduced into the canal using a 4 Lentulo Spiral 25 mm (Dentsply Maillefer, Tulsa, OK, USA). After condensing the paste on the orifice of the canal, the access cavity was temporarily restored with a glass ionomer (Ketac Molar Easymix; 3M ESPE, St. Paul, MN, USA).\nThe patient remained asymptomatic, and the intraoral sinus drainage had disappeared. Therefore, we conducted the second part of the revascularization protocol. PRP was obtained by drawing blood from the patient into sterile tubes with 3.8% sodium citrate that were centrifuged for 10 minutes at a speed of 3,000 rpm in a standard laboratory centrifuge (ROTOFIX 32; Andreas Hettich GmbH & Co., Tuttlingen, Germany). Subsequently, in a laminar flow chamber, the obtained fractions were carefully separated with a 500 μL sterile pipette to isolate the PRP in the middle of the tube, distinct from the red blood cells at the bottom and the platelet-poor plasma at the top of the tube. The PRP obtained was then transferred to a 10 mL hypodermic syringe to be prepared for injection into the canal. Following local anesthesia (2% lidocaine without a vasoconstrictor), and rubber dam isolation, the root canals were accessed and irrigated with 5 mL of 5.25% NaOCl to remove the antibiotic paste and then irrigated with 5 mL of sterile saline solution. Both canals were dried with sterile paper points, and the final Irrigation was carried out with 17% ethylenediaminetetraacetic acid for 1 minute in each tooth and then dried again []. We used a size 60 K-file to extend the working length until bleeding was achieved at the cementoenamel junction, and then waited for 20 minutes until hemostasis occurred and the fibrin clot stabilized. We only applied PRP on tooth #11, leaving the blood clot in place in tooth #21 with no further treatment (). Finally, the entrance of both canals was sealed with a 3-mm-thick layer of MTA Pro Root (Dentsply Maillefer) mixed according to the manufacturer's instructions. The MTA coronal barrier was sealed with a temporary filling glass ionomer (Ketac Molar Easymix; 3M ESPE). After completing the revascularization protocol, the patient was scheduled for periodic follow-ups. An autoradiogram at the end of this appointment was taken to verify the location of the coronal seal ().\nThis visit took place on June 27, 2014. An autoradiogram did not show any changes in relation to the initial autoradiogram (). Cone-beam computed tomography (CBCT) showed a seal with MTA in both upper central incisors. shows the axial view, shows the coronal view, shows the sagittal view of tooth #11 (PRP), and shows the sagittal view of tooth #21 (blood clot). The final restoration was performed with a composite (Filtek Z 250, 3M ESPE) at this time.\nThis appointment was on October 10, 2014. An autoradiogram showed apparent mineralization in the distal wall of the canal of tooth #11 (); unfortunately, no more autoradiograms were taken at that time to confirm the aforementioned mineralization. There were no symptoms or signs of inflammation, infection, or sinus tract. Cold tests were performed, and the patient reported a delayed stimulus in tooth #21.\nOn March 19, 2015, an autoradiogram of tooth #11 showed greater calcification in the root canal space and the fracture lines at the middle third (). There was no presence of periodontal pockets or sinus tracts. Cold tests gave a delayed response in teeth #11 and #21.\nOn April 20, 2017, periapical radiography was taken in the orthoradial position for teeth #11 and #21. This image demonstrated calcification of the canal space of tooth #11 (PRP-treated). Preservation of the crestal alveolar bone of both teeth and the process of healing with interposition of calcified tissue in the fracture line of tooth #21 were observed (). There were no periodontal pockets and no presence of sinus tracts. The response to the cold test was positive in tooth #11 and negative in tooth #21. A CBCT scan was taken, on which it was possible to clearly observe the seal with MTA and the calcification that had occurred in the root canal space of both the coronal () and the apical fragments of tooth #11 ( and ). In tooth #21, the apical fragment had been mineralized, and the coronal fragment still had a defined root canal space (), but there was no evidence of periapical pathology ().\nOn April 19, 2018, periapical radiography was taken in the orthoradial position (). The pattern of calcification into the root canal space of tooth #11 (PRP-treated) was still observed (), and it was also possible to see the displacement of the apical fragment of tooth #21 (). It is noteworthy that periodontal ligament formation was observed in the apical zone of the coronal fragment of tooth #21, as well as preservation of the level of the crestal alveolar bone of both teeth. The mobility of both teeth was grade 3. CBCT was again performed, showing that the calcification process continued in both fragments of tooth #11 (). In tooth #21, displacement of the apical fragment was confirmed (). Finally, at this follow-up there was no presence of periapical pathology in either tooth ().
The patient was a 21-year-old Chinese woman diagnosed with a tumor behind the left peritoneum on computed tomography (CT) during her 33rd-week pregnancy examination in July 2016. The tumor remained clinically silent. All physical examination results were normal except for lumbar scoliosis. The patient did not have problems in daily activities nor experienced neurological symptoms. Her family members were in good condition.\nThe patient underwent abdominal enhanced CT after the caesarean operation in September 2016. The CT results were as follows. The scoliosis was located at the lumbar segment (Fig. ). Several soft tissues suspected to be tumors were found at the apex of the scoliosis. Tissues in front of the left psoas major muscle were enhanced irregularly in the image and adhered to the aorta ventralis and wrapped around the left accessory renal artery. The maximum cross-sectional area of the tissues was 4.7 cm × 4.7 cm (Figs. A, B and ).\nWe considered the tissues as a type of neoplastic disease that can lead to scoliosis, but the nature of the tumor remained unknown. Whether the tumor grows continually was also difficult to confirm. Several tumor markers, such as alpha-fetoprotein and carcinoembryonic antigen, were at normal levels. Finally, the patient underwent resection of the retroperitoneal tumor after all physical examinations and routine laboratory studies showed no contraindication. We selected left lateral rectus incision. During the surgical operation, 3 parts of the tumor (approximately 6 cm × 7 cm × 6 cm, 3 cm × 3 cm × 3 cm, and 3 cm × 3 cm × 4 cm, respectively) were connected by fibers. Parts of the tumor were hard and lobulated in shape but did not extend to the spinal canal. The tumor was exceedingly close to the main vessels and psoas, and it was difficult to separate them. Nevertheless, the nearby lymph nodes were in their normal form. The masses and fibers were excised entirely during the operation, whereas the normal tissues including the lymph nodes were preserved and all specimens were diagnosed as GN after pathological examination. The pathologists finally provided the following immunohistochemistry report: S100 (nerve fiber +), Syna (ganglion cell +), NeuN (−), and Ki67 (MIB-1) (−). No adverse and unanticipated events occurred during the treatments. The patient was followed up for 19 months and did not show tumor recurrence. However, the condition of the scoliosis did not improve.
We describe the case of a 57-year-old white American man with a history of weakness who presented for genetic confirmation of a myotonic disorder. He had been evaluated and diagnosed as having a myotonic disorder at age 27 years when he presented for complaints of stiffness. This diagnosis was made after an electromyography (EMG) test was performed. Over the ensuing 30 years, he developed proximal muscle weakness more marked in his legs than arms with no other significant neurological complaints. A neurological examination at age 57 years showed no abnormalities of his mental status; a cranial nerve examination and cerebellar testing showed no abnormalities, and a sensory examination testing proprioception, vibration, light touch, and pin prick sensibility showed no abnormalities. He had normoactive reflexes with flexor plantar responses. An examination of power revealed Medical Research Council (MRC) grade 4/5 weakness in tests of internal and external rotation, and in deltoid, biceps, and hip flexion. In the remaining muscles, he had normal strength. His muscle bulk was normal and although he had no percussion myotonia, there was a delay in releasing a handshake consistent with action-induced myotonia. Tests of gait were normal.\nThere was no family history of anyone with a similar disorder but his father died at age 56 years from heart disease and there was no other history available on the paternal side. His paternal ancestors came from Romania but originally were of German descent. His mother died at age 94 years from breast cancer and had no clinical features to suggest she was affected by a myotonic disorder.\nMultiple recent EMGs had been performed showing the presence of myotonic discharges with no convincing evidence of myopathy. Genetic testing was performed confirming a diagnosis of DM2, an additional mutation was also detected in his CLCN1 gene (refer to Genetic testing subsection).\nHe was seen in follow up and several years after the confirmation of the diagnosis, he presented with four episodes of mild weakness of his right arm, leg and face associated with numbness and tingling in the same distribution. He also developed slurring of his speech during these episodes which lasted for approximately 2 minutes following a return to his baseline in 10 minutes. He initially went to our emergency room where a diagnosis of transient ischemic attack was made and investigations were performed including a cerebral magnetic resonance imaging and magnetic resonance angiography, carotid ultrasound, and an echocardiogram. All these tests and routine serum chemistries were normal or negative. These episodes recurred three more times over a course of 6 months. The episodes were stereotypical with no clear initiating event and with full recovery to baseline within 10 minutes. A repeat neurological examination showed no significant change from that initially performed. A routine electroencephalogram (EEG) was done 1 month after the last episode showing slowing in his left cerebral hemisphere with no other abnormality detected. He was diagnosed with focal seizures. Approximately 4 months after the last episode, a repeat routine EEG was done and was normal. This was followed up with a 48-hour EEG recording which was also normal. Over the course of the next 24 months, no further seizure episodes were noted without him taking an anticonvulsant medication.\nGenetic testing was performed by a commercial company and analysis for expansions in the DMNK and CNBP genes. However, since he had a prior diagnosis of myotonia congenita, analysis of the skeletal muscle chloride channel, CLCN1 gene was also performed. The results showed no genetic evidence for DM1; testing for DM2 showed a mutant-expanded intronic allele of greater than 372 CCTG repeats with the wild-type allele of 140 repeats in CNBP gene. In addition, a heterozygous mutation in the CLCN1 was also detected, rs149729531, c.501C>G, p.F167L.

Subsets and Splits

No saved queries yet

Save your SQL queries to embed, download, and access them later. Queries will appear here once saved.