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Case 2. In November 2014, a woman aged 69 years traveled to India for 4 weeks, returning on December 8, 2014. On December 11, 2014, she experienced a fever and headache. On December 21, 2014, she was admitted to the hospital with a diagnosis of malaria caused by P. vivax and was treated with quinine and doxycycline. She developed acute respiratory distress syndrome and remained hospitalized for 3 weeks before returning home. She was not treated with primaquine to prevent relapses. She remained in the United States and did not travel out of the country again. On November 11, 2015, her fever returned. On November 21, she received a diagnosis of an uncomplicated P. vivax malaria infection and was hospitalized. She was treated with quinine, doxycycline, and primaquine. Her fever resolved, and she appeared to be clinically stable. The patient was discharged from the hospital on November 23 but then died on November 25. No autopsy was performed, and no additional investigation occurred.\nCases 3 and 4. Two men aged 45 and 44 years who were residents of an African country where malaria is endemic had recently traveled for work to one or two other countries in Africa where malaria is endemic before traveling to the United States for a vacation. On May 1, 2015, they arrived in Chicago, Illinois, after traveling through Amsterdam, Netherlands. According to law enforcement officials and public health investigation records, the man aged 45 years was the first to feel ill, with onset of headache and body aches on May 1 while in Amsterdam. The man aged 44 years reported similar symptoms on May 2. The men were found dead in adjacent hotel rooms on May 9, 2015. The county medical examiner performed autopsies and sent tissue samples from multiple organs from both men to the CDC reference laboratory, which confirmed P. falciparum infections in both patients based on immunohistochemistry and PCR. Parasites from the men contained identical molecular resistance signatures, suggesting that the infections were acquired from the same region. The parasites contained the triple mutation in the dhfr gene and a single mutation in the dhps gene (pyrimethamine and sulfadoxine resistance markers), which are a common signature of parasites from Africa. However, microsatellite analysis revealed discrepant signatures at seven loci, and the man aged 45 years was infected with two different parasite strains. These data suggest that the men acquired the infections independently. No drug paraphernalia were found in either hotel room, nor was there evidence of a struggle or any disarray. No weapons or poisons were found. Law enforcement officials did not suspect foul play in their deaths.
A 31-year-old Korean female visited complaining of an abnormal facial sensation due to previous mandibular angle contouring facial plastic surgery at a local plastic surgery clinic. On panoramic view, the inferior alveolar canals on each side of the mandible did not look normal after both mandibular angle reduction procedures. The canal was cut from the area below the left mandibular second molar with discontinuation in the left mandible. The right inferior alveolar canal was intimately contacted to the inferior border of the mandible without marked mental foramen.()\nWe tested the degree of IAN injury using the nerve mapping method suggested by Lee et al., which consists of five checkpoints including the contact threshold, direction, two-point discrimination, pin prick, and thermal discrimination. The patient showed abnormal sensation in both the lower lip and chin areas as expected. She had mild to moderate paresthesia in both sides of the mandible, thus we predicted there was direct damage to the axon and epineurium, with a diagnosis of axonotmesis in the left mandible. Some damage to the epineurium in the right side resulted in a diagnosis of neuropraxia, which is a first degree injury according to Sunderland's classification.\nThe patient received facial contouring surgery with bilateral zygoma reductions and both angle reductions with mandibular shaving five years previously. She did not have any antipathy to the clinic nor to the surgeon. She had desperately asked the hospital staff for a small round face, regardless of any disruptions to anatomical structures, including IAN damage. But as time went on, she could not endure the continuous pain in both lower cheek areas in spite of many prescriptions for neuro-depressive and analgesic drugs.\nAlthough several consultations about her present symptoms predicted aggravated future outcomes and advised decompressive neurorrhaphy procedures to relieve her symptoms, the patient did not want any further operations not because of her fear for operation, but because of possible changes in the appearance of her face.
A 13-year-old male presented with a fungating lesion on the scalp. He had sustained flame burns at the age of three. The lesion was never grafted and failed to heal completely. The primary care centre continued to manage the wound conservatively with frequent dressings. Eventually, a biopsy was performed and a well-differentiated squamous cell carcinoma was confirmed. He was referred to our tertiary centre for further management.\nHis main complaint was pain and a foul-smelling ulcer with a mucopurulent discharge. Specifically, the patient did not give any history suggestive of epilepsy or personality change. He was noted to be underweight for his age. On examination the lesion occupied the entire central vertex of the scalp []. The tumour measured 12 × 15cm. The central portion of the lesion was pulsatile. Bilateral cervical lymphadenopathy was present. On neurological examination no deficits were identified. No abnormalities were noted on the cardio-respiratory and abdominal examination. Blood investigations revealed microcytic anaemia and hypoalbuminaemia. The human immunodeficiency virus serology was negative.\nInvestigations included a skull radiograph, computed tomography (CT) and magnetic resonance imaging (MRI). These revealed extensive tumour infiltration of the soft tissue with underlying full thickness bilateral parietal bone erosion []. The depth of the infiltration included the dura and the cortex of the left cerebral hemisphere with underlying oedema of the brain []. A filling defect in the superior sagittal sinus indicated a thrombus. A chest roentegram and an abdominal ultrasound excluded metastatic disease in the lungs and liver respectively.\nThe cancer was staged clinically as T4N1M0. With Neurosurgical assistance the resection of the lesion was planned.\nAt surgery, the scalp, the involved cranial bones and dura were excised with 2cm tumour free peripheral margins. A suction-assisted resection of the involved cerebral cortex was done to the depth of normal brain tissue. The soft tissue defect measured 20 × 17 cm []. Our plan was to cover the defect with the anterolateral thigh flap. The tensor fascia lata was to serve as vascularised dural substitute. The flap harvest was abandoned after suitable calibre perforators could not be identified. A latissimus dorsi musculocutaneous flap was then used for cover. Lyoplant® (bovine pericardium) was used as a dural substitute. The flap was anastomosed to the superficial temporal vessels.\nHistological diagnosis confirmed an invasive acantholytic squamous cell carcinoma, an aggressive pathological type which is poorly differentiated, extending into the deep dermis and subcutaneous tissue. The final histopathology result differed from the initial biopsy result due to a larger sample size of the former as well as poorly differentiated type cells were noted within the centre of the lesion. Abundant scar tissue and perineural invasion was noted with no vascular involvement. Brain and dura confirmed the presence of tumour in the deep resection margin. The interpretation of tumour clearance was inconclusive in view of the method of resection of the involved brain tissue.\nThe patient's postoperative recovery and wound healing was uneventful []. The patient had a near total right-sided hemiplegia of immediate onset following the surgery. Four weeks postoperatively, the bilateral cervical lymph nodes showed no signs of regression. Instead, the distribution and the size of the nodes had increased. A fine needle aspiration biopsy confirmed the presence of metastatic disease. Therapeutic bilateral selective cervical lymph node dissection, levels 1-5, was performed five weeks after the primary excision. Histology confirmed metastases to eight out of 90 lymph nodes. The patient was referred to the oncologists for further management. Three months later a large lymph node was detected in the left pre-auricular region which was excised and confirmed as metastatic disease. Chemotherapy was advised and six cycles of Taxotere and Cisplatin were administered. At nine months post surgical excision there was no evidence of local recurrence or metastatic disease. The patient defaulted on the subsequent planning of radiotherapy to the brain. At one year he presented to the oncologist with a history of seizures and weakness in the right upper and lower limb. A scalp recurrence was also noted. A CT scan of the brain showed left intraparenchymal parietal lobe mass lesion with solid and cystic components. The lesion was not amenable to neurosurgical intervention. The patient was offered palliative radiotherapy to the whole brain at a dosage of 3Gy each over nine cycles. The response was poor and the patient was discharged to the care of a hospice resulting in his demise three months later.
An 87-year-old woman with an intractable pain radiating over the left chest, suggestive of intercostal neuralgia, was referred to our spine center for further evaluation and management. Five weeks earlier, she experienced a ground-level fall, causing severe back pain. According to the initial evaluation conducted elsewhere, she was diagnosed as having OVF of the T10 vertebral body and managed conservatively with a brace and pain medication for one month. Her chief complaint gradually changed from bilateral back pain to an aching pain extending to the left thorax before her first visit to our hospital. What is noteworthy about the history of this case is that 8 years previously, she had had a painful OVF of the T12 vertebral body at the age of 79 years, which was treated with vertebroplasty using hydroxyapatite (HA) blocks and posterior pedicle screw instrumentation followed by iliac bone graft fusion from T11 to L1 at another hospital [].\nOn clinical examination at our hospital, the patient had no neurologic deficits, complaining of nothing but reporting a position-dependent severe chest pain on the left side, which intensified when sitting up from a supine position. Imaging studies revealed neither rib fractures nor abnormal findings of the chest cavity as a possible cause of the chest pain.\nOn the plain radiographs of the thoracic spine (), the anteroposterior (AP) views and the lateral views taken in the supine position showed the IVC within the T10 collapsed vertebral body as a gas-like radiolucent area, which disappeared on the lateral view obtained in the sitting position (i.e., the opening-closing phenomenon) most likely indicating an OVF of nonneoplastic and noninfectious origin [, ]. The lateral radiograph in the sitting position also demonstrated that the fracture line extended through the posterior fusion mass bone, grafted 8 years ago, indicating a potentially unstable flexion-distraction injury. Close observation of the AP radiograph obtained in the supine position revealed a gas-like radiolucency at the extravertebral space just lateral to the T10 collapsed vertebral wall ipsilateral to the side of her chest pain. The computed tomography (CT) scans, which have a higher sensitivity in detecting gas than radiography and magnetic resonance imaging (MRI) scans, more clearly showed an accumulation of gas located just below the head of the left 10th rib, the place radiologically termed the T10-T11 extraforaminal zone ().\nIn terms of the IVC contents of radiolucency on the plain radiographs, sagittal MRI revealed a gas-like signal void within the T10 collapsed vertebral body. Corresponding to the extravertebral area of radiolucency shown by the AP radiograph and the CT, both T1- and T2-weighted axial MRI showed combined gas-like and fluid-like signal intensities; i.e., a gas-like low signal intensity area with a fluid-like iso-signal intensity area on the T1-weighted image and a gas-like low signal intensity area with a fluid-like high signal intensity area on the T2-weighted image ().\nAll these findings from the imaging studies suggested that the patient's chest pain probably resulted from T10 nerve root compression at the T10-T11 extraforaminal zone by a mixed accumulation of gas and fluid, which most likely originated from the adjacent IVC within the collapsed T10 vertebral body as a result of the “force pumping mechanism,” as previously postulated [].\nBecause of the unstable nature of the fracture involving all structural components from anterior through posterior spinal columns, in addition to her persistent disabling pain not responding to conservative therapy, we carried out posterior spinal fusion from T7 to L2 with pedicle screw instrumentation followed by iliac bone graft (). Immediately after the surgical stabilization, the patient reported a complete resolution of the pain. This patient had no history of antiosteoporosis medications before the referral to our spine center. We administered pharmacological treatments postoperatively, consisting of teriparatide injections initially, followed by oral bisphosphonates. At a 2-year follow-up, she remained asymptomatic, and CT scans showed neither a gas accumulation nor a fluid collection both inside and outside of the T10 vertebral body.
A 49 year-old man received prolotherapy after suffering from pains at the right shoulder and elbow for five months at a local medical clinic one week before his visit to our clinic. Diagnosed with tendinitis, the patient got both physiotherapy and prolotherapy using a mixed solution of 15% dextrose and 0.125% lidocain onto the near right C5 nerve root, right shoulder and elbow. Right after prolotherapy, he suffered from a sensation resembling an electrical shock on both upper and lower extremities and weakness. Since then, the numbness and pain on the right upper and lower extremities persisted, leading to visit our outpatient care. His medical history showed no hypertension, diabetes mellitus, tuberculosis and trauma and got an appendectomy over acute appendicitis about 15 years ago. He complained of intermittent numbness and discomfort over the right upper and lower extremities. Though the pain was aggravated due to weather condition, there was no particular relieving factor. The visual analogue scale (VAS) checked 60 points. Neurological examination showed his consciousness was on alert mental state. Manual muscle test showed all extremities were normal and the sensory examination showed no indication of hypoesthesia despite the fact that he complained of numbness on the sensory dermatome at the level of C5-C7 nerve root. Vibration and proprioception tests showed no signs of abnormality. Perianal sense and anal sphincter's tones were well kept and a bulbocavernosus reflex was not unusual. His case was classified as American Spinal Injury Association (ASIA) impairment scale E level. Deep tendon reflex was within the normal range without spasticity and the ability to carry out activities of daily living and gait functioned independently. Laboratory test and cervical spine X-ray identified no sign of abnormality. A digital infrared thermal imaging study showed lateral aspect of right and dorsum of hand had a lower skin temperature compared with the left side by 0.6-0.8℃ ().\nSomatosensory evoked potential and magnetic motor evoked potential studies found no definite electrodiagnostic abnormalities and T2-weighted magnetic resonance images (MRI) of cervical spine showed about 0.7 cm sized oval spot with high signal intensity on the right side of the spinal cord at the level of C4-C5 disc. Other mass or signal changes from arachnoid membrane were not observed (). For neuropathic pain relief, the patient used oral administration of Gabapentin 100 mg once a day for two weeks. As part of physiotherapy, transcutaneous electrical nerve stimulation (20 Hz, 10-50 mA, 10 minutes) and infrared therapy (60 Hz, 770 nm, 250 W, 10 minutes) were conducted once a day for two weeks. Additionally, stretching exercise at neck and upper extremities along with strengthening exercises such as isometric flexion exercise and extension exercise of cervical paraspinalis muscles were carried out. After one month of outpatient sessions, his pain level was reduced from 60 to 20 points at VAS.
A 65-year-old male, smoker with a history of type 2 diabetes mellitus, hypertension, and hyperlipidemia, started to notice slowly growing lesions in his left cheek, posterior left shoulder, and left upper arm. The lesions were not tender or itchy. There was no history of shortness of breath. On physical examination, an indurated 10 × 10 cm lesion arising on the left cheek was identified (). Similar lesions were also identified in the right upper arm measuring 2 × 3 cm and posterior left shoulder measuring 5 × 5 cm. No palpable lymphadenopathy or organomegaly was noted. Subsequent biopsy and microscopic examination of the left cheek skin lesion revealed an infiltrate formed by sheets of foamy macrophages surrounded by a dense plasma cell infiltrate and lymphocytes with scattered neutrophils. There was focal granuloma formation. Special stains for organism were negative. Immunohistochemical studies showed diffuse staining of the histiocytes for S100 protein and CD68. They were negative of CD1a, ruling out histiocytosis X. The lymphocytes were a mixture of T and B cells. The plasma cell infiltrate was polyclonal and was confirmed by in situ hybridization for kappa and lambda. The combination of findings was consistent with cutaneous Rosai-Dorfman disease. CT scan of the chest was performed, which showed bilateral thin and mildly thick-walled pulmonary cysts. Some of them were rounded and some demonstrated bizarre shapes with multiloculation and internal septations. There were also numerous ground-glass nodules ranging from 1-2 mm up to a few centimeters. The findings had a characteristic upper lung zone predominance sparing the lung bases and the anteromedial parts of the middle lobe and lingula (). There was no intrathoracic lymphadenopathy and no pleural effusion. Initially, the skin lesion at the cheek was treated with radiotherapy (1500 cGy in 5 fractions); however, there was only minimal response. The patient was started on 50 mg oral prednisone daily, which resulted in improvement of the skin lesions. However, treatment with prednisone was complicated by worsening of diabetes and peripheral edema and hence was stopped. A follow-up chest CT five months later showed complete resolution of the ground-glass nodules and persistence of the lung cysts ().
We report the case of a 47 years-old female patient, who was referred to our unit in November 2007 for isolated pelvic pain. Clinical examination was without abnormalities. The abdominal pelvic ultrasound revealed a 60 mm hypoechoic and heterogeneous mass in the right iliac fossa. The abdominal pelvic computed tomography showed a tumor measuring 50 × 50 × 48 mm, attached to the anterior wall of the cecum and the right colon (Fig. ).\nThe multidisciplinary meeting decided to perform a diagnostic and therapeutic laparoscopy. The laparoscopic exploration showed a 70 mm tumor of the small bowel, 70 cm upstream the last ileal loop. The tumor was extracted through a small suprapubic incision, without the use of a wound protector. It was resected with a safety margin of 2 cm and an end-to-end anastomosis was performed. The postoperative course was marked by an infection of the extraction’s site. The pathology specimen findings revealed a stromal tumor with a high aggressive potential (five mitosis per field), positive to CD117 at the immunohistochemical examination. An Imatinib adjuvant therapy was indicated, but the patient did not receive it because of financial considerations.\nThe patient had a postoperative follow-up (clinical examination, abdominal ultrasonography and abdominal computed tomography alternately) every 3 months during the first year, then every 6 months during the second and the third year. After that, the patient stopped her follow-up on her own.\nSeven years after the surgical resection, the patient was hospitalized for a pelvic mass underneath the old scar. The abdominal examination found a mass measuring 60/70 mm. The abdominal pelvic CT scan showed a 100/75 mm solid mass of the abdominal wall alongside to the pubic symphysis (Fig. ).\nPercutaneous biopsies of the tumor were made and histologic examination showed an aspect of low-grade malignancy GIST. An enbloc surgical resection of the tumor was performed, with a safety margin of 1 cm. The wall defect measured 6 cm in diameter and it was closed by direct suture. The pathological examination revealed a tumoral proliferation of spindle atypical cells with mitotic signs. The immunehistochemical examination showed an important positivity to CD117 and DOG1. The patient received adjuvant chemotherapy (Imatinib).
A 32-year-old woman (gravida 5, para 3) at 32 weeks of gestation was diagnosed with placenta previa and was referred to our hospital from a private facility. She had a history of 3 cesarean deliveries. In her current pregnancy, the placenta was located along the entire anterior uterine wall. Ultrasonographic findings revealed multiple lacunae, and magnetic resonance imaging revealed the loss of uterine myometrium between the placenta and bladder wall (Fig.A and B). Therefore, she was considered to be at a high risk for placenta percreta. Fetal growth was appropriate for gestational age.\nAt 33 weeks, she experienced a sudden onset of vaginal bleeding. Blood loss was estimated about 100 mL and she was admitted to our hospital. She was received intravenous fluids and intramuscular betamethasone to stimulate fetal lung maturation. The day after admission, she experienced vaginal bleeding again and total blood loss was estimated to be over 300 mL. We decided to perform an emergency cesarean delivery using a transverse uterine fundal incision to deliver the fetus.\nAn ureteral stent was preoperatively placed by a urologist. Laparotomy revealed large blood vessels and the placenta was observed through the anterior uterine wall (Fig.A). Based on these findings, placenta percreta was suspected and separation of the bladder from the uterus was considered extremely difficult. We performed a transverse uterine fundal incision to avoid an incision into the placenta. After elevating the uterus outside of the abdominal wall, an ultrasound-guided transverse incision was made into the uterine fundus to avoid rupturing or producing a bulge on the fetal membrane (Fig.B). The incision produced minimal bleeding and avoided the placenta. A healthy male infant of 2122 g was successfully delivered.\nThe placenta was not spontaneously delivered because of abnormal uterine adherence, vaginal bleeding continued. We initially attempted to perform hysterectomy using the bladder-filling technique; but because of adherence between the anterior uterine wall and bladder, we could not strip the bladder from the uterus. Because of our attempt to separate the bladder, bleeding suddenly increased from the placental site and the total blood loss reached over 3500 mL. Conventional hysterectomy could have caused severe bladder injury and cystectomy. A retrograde hysterectomy and placental amputation were successfully performed (as shown in Fig.A).\nFollowing hysterectomy, bleeding continued and the remaining placental tissue was resected. During the resection, a 10-mm2 bladder injury was detected. The border between the bladder and placenta was visualized to separate the organs, and after complete resection of the placental tissue, we repaired the bladder. The total blood loss was approximately 7800 mL, including an estimated blood loss of 4000 mL from the placental site prior to hysterectomy; 2000 mL during hysterectomy; 1000 mL from the bladder wall after hysterectomy; and 800 mL from other sources. The patient was transfused with 3360 mL of red blood cells; 1800 mL of flash frozen plasma; and 400 mL of platelets. The excised specimens are shown in FigureB.\nThe patient was transfused with 560 mL of red blood cells after the operation. Postoperative blood pressure stabilized to 120/70 mmHg, and pulse rate was 90 beats/min. A complete blood count indicated a hematocrit level of 29.6% and a hemoglobin level of 10.6 g/dL. Because of the substantial hemorrhage, considerable blood transfusions were required; and she was subsequently placed in the intensive care unit. She had an uncomplicated postoperative course and was discharged 10 days later. The infant was discharged 30 days later in a healthy condition.\nA hematoxylin and eosin-stained section of the placenta (Fig.C) revealed the placenta invaded the serosa of bladder. These findings suggested the diagnosis was placenta percreta. As shown in FigureB, the lower uterine segment was resected from the uterine body and the residual tissues were resected step by step from the bladder. We considered the placental tissue to be completely resected, and this was confirmed by a negative qualitative human chorionic gonadotropin test result obtained after discharge.
An 89-year-old gentleman with no significant past medical history presented to the emergency department (ED) of a district general hospital with atraumatic right hip pain and inability to weight-bear. The patient had a long history of right hip osteoarthritis causing pain and functional limitation, for which he had been referred by his general practitioner to the orthopaedic team for consideration of total hip replacement three years previous. However, the pain experienced during the week preceding ED presentation was much more severe in character. Of note, he had been seen by his general practitioner in the week preceding ED attendance with intense right hip pain on weight-bearing and passive movement and had been referred to the elderly care team with a view to optimising pain management.\nPlain film radiography obtained in the emergency department () demonstrated a lytic lesion within the right femoral neck, intertrochanteric region, and proximal femoral metaphysis with evidence of cortical breach and progressive sunburst periosteal reaction.\nThe patient was admitted for further investigation and assessment. Computed tomography (CT) of the chest, abdomen, and pelvis () confirmed a bony lesion affecting both the right acetabulum and proximal femur with pathological intertrochanteric fracture and abnormal surrounding soft tissue. Magnetic resonance imaging (MRI) () findings were felt to be consistent with that of a primary bone tumor; tumoral necrosis was evident, and thigh muscles demonstrated oedema to the knee level. No lymphadenopathy was seen. Nuclear medicine scanning () demonstrated avid uptake in the right acetabulum and proximal femur with cortical breakthrough and marked extension to the surrounding thigh compartments but no uptake suggestive of distant disease. Fluorodeoxyglucose-positron emission tomography (FDG-PET) was not performed preoperatively.\nHistological analysis of needle core biopsies obtained via a direct lateral approach under ultrasound guidance of the right femur was undertaken; the findings of which were in keeping with that of diffuse large B-cell lymphoma (DLBCL). Sections through the cores demonstrated tissue infiltration by large round blue cells staining positively for leukocyte common antigen, CD20, BCL2, and BCL6 and negatively for CD10, CD3, CD5, and cyclin-D1. The Ki67 proliferation index was high at approximately 80%. Interface fluorescent in situ hybridisation detected a BCL2 translocation but no BCL-6 or c-myc translocations.\nThis gentleman was transferred to Northern Ireland's Regional Trauma Centre where he underwent proximal femoral replacement with acetabular reconstruction, owing to the presence of pathological fracture as visualised on CT. Pathological analysis of the operative specimen demonstrated a tumor present within the medullary cavity with cortical, periosteal, and soft tissue involvements. The osseous resection margin was clear; however, the soft tissue resection margin was involved laterally. Histological analysis confirmed DLBCL of germinal centre phenotype staining positive for CD20, BCL6, and BCL2 and negative for CD3, CD5, CD10, MUM-1, cyclin-D1, C-MC, and TdT. The proliferation index was again high with MIB-1 of over 90%.\nThe patient received postoperative care in the intensive care unit where he required inotropic support ahead of ward transfer. FDG-PET undertaken two weeks postprocedure demonstrated high FDG uptake at the surgical site, but metabolically active disease at other sites was not seen.\nHe was discussed at the haematological multidisciplinary meeting postoperatively, and the diagnosis diffuse large B-cell lymphoma (DLBCL), NOS (M9680/3), was agreed. He was deemed stage IEA due to the involvement of contiguous bones. The patient embarked upon six cycles of R-Mini-CHOP (a regimen of rituximab with decreased dose cyclophosphamide, doxorubicin, vincristine, and prednisolone []), a regimen which is in widespread use within the unit amongst those greater than 80 years of age.\nThroughout his R-Mini-CHOP treatment, the gentleman was able to fully weight-bear and made excellent progress with the rehabilitation team. Radiographically, implant alignment was satisfactory, and there was no evidence of prosthetic loosening. The patient subsequently declined radiotherapy treatment. Repeat FDG-PET was conducted five months following diagnosis; no further disease was demonstrated.
A 65-year-old male presented with complaints of gradual diminished vision that was greater in right eye. He gave history of blurring of vision with watery discharge bilaterally and was diagnosed with PUK and treated successfully 2 years prior to presentation. On examination his visual acuity was counting finger at 2 feet in right eye and 20/100 in the left eye. Intraocular pressure in both eyes was 16 mmHg with applanation tonometry. Slit-lamp examination showed both eyes with peripheral degeneration at the nasal limbus without thinning of cornea, with nuclear sclerosis grade III with posterior subcapsular opacity in the right eye and nuclear sclerosis grade II in left eye. Fundus examination was normal. The patient underwent uneventful manual small incision cataract surgery in the right eye. He was discharged with instruction to instill topical prednisolone acetate six times a day, moxifloxacin four times a day and cyclopentolate at night. At the 5 days postoperatively, the patient presented with scleral melting and thinning of the superior sclera from 11 o’ clock to 1 o’ clock at the incision site. The patient was diagnosed with SINS and systemic immunosuppression in the form of a pulse of methylprednisolone 500 mg followed by prednisolone 20 mg was started along with topical steroid drops. Ageneral physical examination was performed that was negative for systemic vasculitis or arthritis. A chest X-ray and blood tests including a full blood count, erythrocyte sedimentation rate, C-reactive protein, rheumatoid factor, antinuclear antibody, and anti-neutrophil cytoplasmic antibody tests were performed. These tests were normal. Urinanalysis was normal.\nSINS did not respond to steroid therapy and progressed further to create wound gap and a shallow anterior chamber. Simultaneously, the nasal corneal periphery showed PUK in the form of crescent shaped superficial corneal thinning at the previous site of corneal degeneration []. To prevent globe perforation, a multilayered AMT with conjunctival resection was performed at the site of scleral necrosis. PUK was superficial, hence no active surgical treatment was performed. Systemic steroids were continued along with topical steroid drops. Three days after AMT, scleral necrosis began to resolve with a well formed anterior chamber however, PUK increase in depth up to Descemet's membrane []. Again, to prevent perforation, a multilayered AMT was performed with conjunctival resection. Systemic steroids were reduced to 10 mg maintenance dose within a week. Topical prednisolone was continued. Steroids were tapered down and stopped within a month. Scleral as well as corneal melting improved after AMT. Complete healing took place within 15 days of AMT [. There was no recurrence of SINS and PUK after 6 months follow-up.
An 11-year-old girl was referred to our hospital in August 2010 with a lesion in the right cheek area which was progressively enlarging. The patient complained that her nose and mouth corner were crooked and that her face was swelling. In clinical examination, the patient had a slight asymmetry in the right midface as a result of buccal and palatal cortical expansion from the right maxillary canine to the molar region, resulting in depression of the nasal alar and mouth corner (Fig. ).\nA panoramic radiograph showed an increased bone density on the right maxilla and zygoma and obliteration of the right maxillary sinus. Computed tomography (CT) revealed a 4.5 × 4 × 4.5 cm, expansile ground-glass opacity lesion involving the right maxillary sinus, right maxillary alveolar process, zygoma, and hard palate. Bone scan revealed an irregularly shaped hot uptake in the right maxilla, and no abnormally increased uptake was observed at any other sites (Fig. ). The physical examination did not show any other lesions, and the patient had no history of pain, trauma, loosening of teeth, or trismus. Based on the typical radiologic findings, the patient was diagnosed with FD, and no additional biopsy was performed. The patient had regular follow-up every 6 months to monitor the lesion’s progress. At the 1-year follow-up, the development of tooth germ within the lesion was normal, and slight expansion of the lesion to the bucco-lingual side was observed. Because we thought the patient was still growing and increasing in height, we decided to conduct an ongoing progress observation.\nAbout 3 years later in December 2012, there were no significant changes of the FD lesion, but the distance from the mouth corner to the inner canthus was about 2 cm longer on the right side than on the left. Periodic observation was continued, and in August 2017, corrective surgery was planned because the maturation of the lesion was confirmed to be complete and there were no changes in the size of the lesion. At that time, the distance from the mouth corner to the inner canthus was 2.5 cm longer on the right side than on the left, and the distance from the occlusal plane to the outer canthus was 5 cm longer on the right side than on the left. Bone contouring surgery, the primary treatment for facial asymmetry and fibrotic bone lesions, was planned (Fig. ).\nThe patient wanted to improve asymmetrical facial appearance through the surgery. Therefore, we aimed not only to remove the FD lesion, but also to make the patient’s facial as symmetrical as possible. For this, direct visualization and surgical approach to the infraorbital rim and lateral area of zygoma were required, but the surgical approach through buccogingival incision had limited access to these areas. On the other hand, the midfacial degloving approach was expected to help reestablishing symmetric facial contour by allowing direct comparison of the lesion with the normal side. Moreover, this approach provides esthetically acceptable outcomes, leaving no scars and no functional disability. Therefore, we decided to perform the operation through the midfacial degloving approach.\nWith the patient under orotracheal anesthesia, the lesion was removed by the midfacial degloving surgical procedure. Local anesthesia with 2% lidocaine with epinephrine (1:100,000) was infiltrated into the maxillary vestibular mucosa and into the nose. The procedure is performed with a maxillary vestibular incision and three intranasal incisions to expose the entire midface skeleton that include (1) bilateral intercartilaginous, (2) complete transfixion, and (3) bilateral piriform aperture incisions (Fig. ).\nA buccogingival incision was made in the maxillary vestibule approximately 5 mm superior to the mucogingival junction and extended from the second molar to the contralateral second molar. Periosteal elevators were used to elevate the tissues in the subperiosteal plane fist over the anterior maxilla and then extending widely to encompass posterior tissues behind the zygomaticomaxillary buttress. The infraorbital neurovascular bundle was identified superiorly and dissected. Subperiosteal dissection along the piriform aperture stripped the attachments of the nasal labial muscularture to allow its complete release from the midface skeleton. The mucoperiosteal flap was elevated up to the piriform aperture.\nThe intercartilaginous incision divided the junction between the upper and lower lateral cartilages (Fig. b). An incision was made along the inferior border of the upper lateral cartilage, beginning at the lateral end and extending medially curved into the membranous septum anterior to meet transfixion incision (Fig. a). Laterally, the incision was sufficient that it extended to the piriform aperture. The lower lateral cartilage was eventually displaced superiorly during the degloving procedure, whereas the upper lateral cartilage remained attached to the midface skeleton. The transfixion incision was used to separate the membrane septum/columella from the cartilaginous septum. An incision was made along the caudal border of the septal cartilage from the medial end of the intercartilaginous incision toward the anterior spine (Fig. a). The intranasal incision was made by a full-thickness incision down through the periosteum of the piriform margin and the nasal floor.\nDissection through the intercarilaginous incision allowed access to the nasal dorsum and bones (Fig. b). Sharp subperichondrial dissection with a scalpel or a blunt dissection with scissors freed the soft tissues above the upper lateral cartilage as in a standard open rhinoplasty. The dissection should be within the subperichondrium plane to prevent injury to the overlying musculature and blood vessels of the nose. Elevation extended laterally to the nasomaxillary sutures and superiorly to the glabella. Retraction of the freed soft tissues allowed sharp incision to be made with a scalpel or with sharp periosteal elevators through the periosteum at the inferior edge of the nasal bones. Elevation of the soft tissue laterally to the piriform aperture was also performed so that the maxillary vestibular dissection was easily connected to this pocket.\nAfter the connection of the nasal and oral incisions, the midface was degloved. The midface skin was separated from the maxilla and the nasal pyramid. The upper lip and the intact nasal columella, nasal tip, and alar cartilages were then retracted by two Penrose drains introduced through the nostrils over the nose to the level of the inferior orbital rim. This approach provided visualization of the medial maxillary wall, pterygoid junction, nasofrontal suture, infraorbital rim, and laterally to the temporal process of the zygoma (Fig. d). Under direct visualization, the overgrowing bone lesion was then excised using osteotomes and saws. The right maxilla was drilled further at the orbital rim and laterally till zygomatic complex. The contour of the midface was reestablished using burr to give a cosmetically normal looking midfacial skeletal contour while protecting the infraorbital nerve (Fig. ). For the removed lesion, a biopsy was performed for the accurate diagnosis and histologically confirmed as FD. The soft tissues were then carefully redraped and the nasal tip brought back into position. The intranasal incisions were closed using 4-0 resorbable sutures (vicryl), and the transfixion sutures were precisely performed to determine the final position of the nasal tip and prevent vestibular stenosis. The cinch suture of alar base was used to prevent postoperative alar base widening. The intraoral incisions were closed using a 3-0 black silk. Nasal packing into the maxillary dead space with Vaseline gauze was done for 3 days in order to minimize the postoperative bleedings.\nThe patient’s postoperative course was generally uneventful. There was moderate nasal crusting for 3–4 days. Mild swelling with periorbital ecchymosis disappeared after 2 weeks, and transient paresthesia around the infraorbital nerve spontaneously resolved after 3 month. No postoperative complications such as epistaxis, vestibular stenosis, or esthetic problems of the nose were seen. Clinical and radiographic examinations obtained 4 months after surgery showed the anatomical structures were in a stable state without recurrence of FD (Fig. ). The esthetic result was satisfactory for the patient, and occlusal state was also well maintained (Fig. ). Therefore, no additional orthodontic treatment or orthognathic surgery was performed.
A 50-year-old male was brought to the emergency department after he jumped from a 5-meter bridge in an attempted suicide and fell on the hard concrete below. Upon admission, the patient was agitated, disoriented, and in intense respiratory distress. Examination revealted that the patient’s airway was clear, but there was a bilateral absence of breath sounds and hyperresonance on percussion. The patient was hemodynamically stable. He was intubated due to respiratory failure. Bilateral chest tubes were inserted based on a high clinical suspicion of pneumothorax. Thereafter, the patient developed a large subcutaneous emphysema, despite the fact that the chest tubes were functioning with his severe air leakage. Past medical history was unremarkable with no previous formal depression diagnosis. A social history check showed daily marijuana and tobacco use.\nThe patient was sent for a head, neck, thorax, abdomen, and pelvis CT scan. The scan revealed giant bullous emphysema on the superior lobes bilaterally, right pneumothorax with a collapsed lung, along with multiple rib fractures, and lung emphysema (, ). A hip dislocation was detected, and closed reduction was performed.\nThe patient was sent to the ICU, where he improved clinically after conservative treatment with continuous negative pressure suction using a 20 cm water column. He was extubated after 48 h, with persistence of the air leakage on both chest tubes.\nOn the day following extubation, he developed hypoxia associated with disorientation and agitation and had to be intubated again. Hypoxia was postulated from pulmonary contusion and ventilator-associated pneumonia worsening his already baseline compromised lung. He developed sepsis, and subsequently acute kidney injury with the need for dialysis.\nDuring the course of 5 days, the patient presented hypoxia and a severe mixed metabolic and respiratory acidosis, despite the use vancomycin and piperacillin/tazobactam. The treatment with bilateral chest tubes associated with continuous negative pressure aspiration did not correct the air leakage, which caused an important lost of tidal volumes on the ventilator. Changes in ventilator parameters (increases in PEEP, tidal volumes, etc.) did not improve his oxygenation or decrease his pCO2. In an effort to expand his lungs and improve his ventilatory function, we decided to perform a bilateral bullectomy.\nAs the patient had poor surgical status, only a right bullectomy was performed 8 days after the trauma. The right side was chosen over the left, because it showed more compression and a larger residual, healthier parenchyma. After surgery, the right side fistula was resolved (). Four days after surgery, the patient developed a right-sided empyema, right lung incarceration, and blood clots in the chest tube (). In order to resolve these issues, videothoracoscopy and decortication of the right lung were performed, which resulted in the resolution of these problems () and improvement in ventilatory parameters with conservative treatment for the left lung. Notwithstanding antibiotic treatment and successful surgical interventions, the patient died on the 25th day of hospitalization due to infectious complications.
An 18 year old Saudi unmarried male student had been attending the ophthalmology clinic for posterior uveitis for the last few weeks. He was also followed in urology clinic for testicular pain with benign hypo-echoic lesions, presumed to be trauma related, for the last few months. He presented to the emergency room with massive bleeding from mouth, (query hemoptysis versus hematemesis) and found to have cardiac arrest on arrival to the emergency room. The patient had no history of any allergies, surgeries in the past, sexual contacts, smoking, addiction or any familial illnesses. Cardiopulmonary resuscitation was done and he was intubated, ventilated and shifted to intensive care unit. His laboratory data on arrival revealed no significant abnormalities. His endotracheal tube and nasogastric tube continued to drain over 500 ml of bloody aspirate over the next 24 hours, putting the possibility of asphyxia from aspiration of blood as a likely cause of initial cardiac arrest.\nHe underwent esophagogastrodudenoscopy which showed coffee ground material in stomach but no signs of active bleeding. His toxicology screening and autoimmune profile (rheumatoid factor, antinuclear antibodies) and viral & Brucella serology were all negative. The patient remained intubated and mechanically ventilated with a baseline Glasgow coma scale of 7. His CT (computerized tomography) scan chest with contrast was negative for pulmonary embolism. His echocardiogram on the 2nd post admission was initially suspicious for probable mass versus vegetation over tricuspid valve which prompted IV antibiotics (vancomycin and amikacin) as well as antifungal (amphotericin B) as patient remained culture negative throughout his stay. However, the second echocardiography and cardiac surgery review refuted the need for any surgical intervention for suspected tricuspid lesion due to poor clinical condition of the patient and the paucity of chances for successful repair. The patient remained afebrile from the 3rd post admission day onwards, which prompted the primary physician to discontinue all antibiotics/antifungal after 14 days as all septic screening remained negative throughout. His initial CT brain revealed hypoxic brain injury with minimal cerebral edema which resolved on subsequent scans. He also remained under the auspices of neurologist for two episodes of myoclonic seizures treated with phenytoin and maintained on sodium valproate. An opinion regarding possible biopsy of the hypo-echoic lesion in the testicles was sought but the urologist’s impression remained that the lesions are likely secondary to a traumatic cause which require no further workup.\nAs the diagnosis remained elusive, the family was again called for obtaining additional points in history. On 14th post admission day, during re-interview with the family about recent oral ulcer that the patient had now developed in the mouth while in intensive care unit, they revealed that the patient has recurrent oral and genital ulcers on almost a monthly basis along with recurrent joint pains off and on. His Pathergy test was done which was inconclusive. His ophthalmological examination was significant for posterior uveitis. Rheumatologist’s opinion was sought and it was mutually agreed by the multidisciplinary team to treat the patient with steroids as a case of Behcet’s disease. A second CT chest with contrast was done with reconstruction imaging, which showed massive pulmonary arteriovenous malformations/aneurysm (see Fig. ) and left small pulmonary infarct without any evidence of thromboembolism. His steroids were changed to Methylprednisolone 1 gram intravenous pulse therapy for 5 days in addition to initiation of azathioprine 50mg. The family was counseled but did not agree to consent for cyclophosphamide therapy due to potential side effects. The steroids were then tapered down over 6 months and maintained on 10 mg prednisolone with azathiprine 75 mg daily along with osteoporosis prevention medications.\nThe patient was eventually extubated and tracheostomy closed but continued to have irreversible brain hypoxic injury with minimal responsiveness with eye contact and nodding and no motor response in the limbs. He remained under follow-up with cardiology, rheumatology, nephrology and neurology for over one year as family was unwilling to take the patient home. His biochemistry remained normal throughout the stay. His repeat ultrasound revealed that the testicular lesions had completely resolved while the repeat echocardiography revealed that the tricuspid lesion had also completely resolved, all indicative of possible vasculitic origin. His repeat CT scan chest showed all pulmonary arterial aneurysms resolved after almost one year of steroids and azathioprine (see Fig. ). The patient was discharged with home health care and over the last one year follow-up he has remained in an unchanged condition, bedridden with persistent hypoxic brain injury with no new lesions or other active problems.
A 35-year-old G4P2012 presented to labor and delivery at full term in early labor with spontaneous rupture of membranes. She had undergone living donor kidney transplant 8 years prior for ESRD suspected secondary to IgA nephropathy versus post-streptococcal glomerulonephritis. She was maintained on Prograf and prednisone throughout her pregnancy with a stable baseline serum creatinine 1.2 mg/dL. Obstetrical history was significant for two prior uncomplicated Cesarean deliveries, both prior to her kidney transplantation. It was unclear from her records whether she had undergone counseling regarding pregnancy post-transplant. She declined a of trial of labor after Cesarean section despite counseling on the risk of injury to the transplanted kidney given its location in the operative field, which was confirmed on transabdominal ultrasound prior to the case.\nThe patient was taken to the operating room for and indicated repeat Cesarean section. Routine dissection was performed down to the level of the fascia through a Pfannenstiel incision and the fascia was carefully transected horizontally in the usual fashion. When the superior fascial edge was lifted for traction to allow for dissection from the underlying rectus muscle, brisk pulsatile bleeding was noted. The transplanted kidney was noted to be densely adherent to the overlying anterior abdominal wall, and avulsion of a portion of the transplanted kidney was confirmed (Fig. ). Direct pressure was applied to the organ while the Trauma Surgery team was consulted. The avulsion was repaired using 3–0 pledgetted Prolene sutures in interrupted horizontal mattress fashion and using Evarrest compound. At this time, the decision was made to extend the skin incision in the vertical midline for improved access to the uterus given the concern for further shearing forces to the exposed transplanted kidney in the process of delivering the baby. The rest of the case was uneventful. No blood product transfusion was necessary. After the transplanted kidney was repaired, a viable male infant with Apgars of 8 and 9 weighing 2460 g was delivered.\nPostoperative serum creatinine levels remained stable between 1.1–1 .3mg/dL and the patient maintained adequate urine output. Renal ultrasound immediately postpartum and 1 month postpartum demonstrated normal arterial and venous flow with no evidence of ischemia or perinephric hematoma.
Case 1 is a 60-year-old man with a family history of “BD” in his elder brother who was treated with lithium and who died due to chronic kidney disease. Case 1 presented at our clinic ABC 25 years ago with a history of two “manic” episodes and 2 depressive episodes being treated with lithium. He was euthymic at the time of this first consultation at our clinic. His previous consultant referred him as patient shifted his residence to the present city. The patient had significant lithium-induced tremors for which he was put on propranolol which did not give him a satisfactory relief. During follow-up, over a period of years, he never had recurrence of manic episodes but a few milder depressive episodes for which he was treated with escitalopram 10–20 mg for brief periods of 3–4 months. The patient was happy over the control of his mood symptoms but had constant worry about his hand tremors as his job, a banker, involved lot of writing work. After about 10 years of follow-up, a decision was taken to challenge the course of illness with gradual withdrawal of lithium (reasons being – no manic episodes in 10 years and significant tremors) and maintain him on lamotrigine. Patient never had any manic episode. He had milder depressive episodes only, whose frequency did not change, and he was treated with brief antidepressant medication. After a period of another 8 years, lamotrigine was withdrawn gradually and he is maintained on about 10 mg of escitalopram. Patient's disease course did not worsen in the last 7 years while he is on escitalopram alone. Over past 25 years, he went through multiple personal stressors including marital separation for few years, intellectual disability in his son who later died at the age of 11 years. Currently, he works as an assistant manager in the banking sector. The clinical questions are – (1) Was the original diagnosis of BD correct? (2) Is he one of those rare patients who had only 1–2 manic episodes without any further episodes of mania in the follow-up period of 25 years! (3) Was there requirement of long-term maintenance of lithium and lamotrigine in this patient? The only reason the clinician continued “Mood Stabilizers” for almost two decades was because of the belief that these medicines were helping him immensely in preventing the manic episodes, which might not be true.[. Lithium was tapered and stopped due to intolerable hand tremors, which were causing disability at workplace. For the last 7 years, he is being treated with escitalopram 20 mg and is doing well.
A 41-year-old woman was referred for vaginal bleeding. The previous day, she underwent a TOT procedure in a private gynecologic clinic. She urinated without difficulty and was discharged from the clinic. However, she presented with abdominal pain and voiding difficulty at the next day. She went to original clinic, they inserted foley catheter into her bladder to drain, she urinated 1,000 mL after foley catheter insertion. The original surgeon found hematoma on the vaginal wall and undertook hematoma removal. However, the vaginal bleeding persisted. Her hemoglobin dropped from a preoperative level of 15 to 9.6 g/dL. After packing with vaginal gauze, she was sent to our emergency care center. After arrival, she appeared pale and acute illness. Her blood pressure was 104/66 mmHg with tachycardia at 100/min. The left vaginal wall was found to be edematous and active bleeding. However, it was difficult to identify focus of bleeding. On the laboratory findings, the hemoglobin was 8.1 g/dL after two units of packed red cell transfusion, and coagulation factors, electrolytes, chemical batteries were within normal range. The patient was taken to angiographic room for embolization. Angiography showed extravasation of contrast from the upper vaginal branch of left internal iliac artery (). After super selection via microcatheter, selective embolization with glue were undertaken. Then extravasation of contrast was stopped. After embolization, vaginal bleeding was decreased and her vital signs stabilized. On post operation day 3, no evidence of active bleeding after embolization was observed on abdomen and pelvis computed tomography (CT). On post operative day 4, she reported no vaginal bleeding, she was discharged. Twelve days after her completed operation, she went to the emergency room, complaining of lower abdominal pain. Through the abdomen and pelvis CT scan, it turned out that the size of residual hematoma had decreased and extravasation didn't show up on the contrasted CT. She was discharged from the emergency room after receiving pain modulation. Fourteen days after the operation, we conducted a follow-up consultation and her abnormal pain had been mitigated. She did not show signs of any of the specific abnormalities. Her stress incontinence symptoms also improved in a follow-up consultation conducted in 3 months later.
A 50-year-old male presented with complaints of chronic, intermittent abdominal pain. The patient had a 20-year history of mild-to-moderate amount of alcohol consumption. There was no history of jaundice, fever or weight loss. His laboratory tests revealed no significant abnormal findings. Serum amylase and lipase, and tumour markers [CA 19-9, carcinoembryonic antigen (CEA)] were within normal limits. The patient was subjected to a CT examination. On unenhanced CT image, a poorly defined soft tissue mass was seen in the pancreaticoduodenal (PD) groove with a hypodense cystic lesion within the mass (). On post-contrast study, the lesion showed minimal enhancement in the portal venous phase ( and ), but delayed imaging at 2–3 min showed mild persistent enhancement of the lesion compared with the pancreatic parenchyma that was consistent with scar tissue () and a non-enhancing cystic lesion within the lesion. The lesion showed poorly defined fat planes with the adjacent second part of the duodenum on its right side and pancreatic head on the left side. Mild thickening of the wall of the second part of the duodenum adjacent to the lesion with variable luminal narrowing was noted. The common bile and pancreatic ducts appeared grossly normal. The pancreatic body and tail were normal. In order to clearly delineate the ductal system and the periampullary region, and to know the extension of the lesion, the patient was also subjected to an MRI examination. Axial two-dimensional fast imaging employing steady-state acquisition () sequences showed soft tissue signal intensity lesion in the PD groove with a cystic lesion within the lesion and the aforementioned CT scan findings. MR cholangiopancreatography sequence () revealed smooth and regular tapering of the pancreatic and common bile ducts. The gallbladder was distended and the cystic duct was normal. Incidentally, a few simple cysts (Bosniak 1) were noted in both the kidneys. The patient was advised further surgical intervention but he refused and was managed with conservative treatment. At present, the patient is asymptomatic.
A 50-year-old male was brought to the emergency department after he jumped from a 5-meter bridge in an attempted suicide and fell on the hard concrete below. Upon admission, the patient was agitated, disoriented, and in intense respiratory distress. Examination revealted that the patient’s airway was clear, but there was a bilateral absence of breath sounds and hyperresonance on percussion. The patient was hemodynamically stable. He was intubated due to respiratory failure. Bilateral chest tubes were inserted based on a high clinical suspicion of pneumothorax. Thereafter, the patient developed a large subcutaneous emphysema, despite the fact that the chest tubes were functioning with his severe air leakage. Past medical history was unremarkable with no previous formal depression diagnosis. A social history check showed daily marijuana and tobacco use.\nThe patient was sent for a head, neck, thorax, abdomen, and pelvis CT scan. The scan revealed giant bullous emphysema on the superior lobes bilaterally, right pneumothorax with a collapsed lung, along with multiple rib fractures, and lung emphysema (, ). A hip dislocation was detected, and closed reduction was performed.\nThe patient was sent to the ICU, where he improved clinically after conservative treatment with continuous negative pressure suction using a 20 cm water column. He was extubated after 48 h, with persistence of the air leakage on both chest tubes.\nOn the day following extubation, he developed hypoxia associated with disorientation and agitation and had to be intubated again. Hypoxia was postulated from pulmonary contusion and ventilator-associated pneumonia worsening his already baseline compromised lung. He developed sepsis, and subsequently acute kidney injury with the need for dialysis.\nDuring the course of 5 days, the patient presented hypoxia and a severe mixed metabolic and respiratory acidosis, despite the use vancomycin and piperacillin/tazobactam. The treatment with bilateral chest tubes associated with continuous negative pressure aspiration did not correct the air leakage, which caused an important lost of tidal volumes on the ventilator. Changes in ventilator parameters (increases in PEEP, tidal volumes, etc.) did not improve his oxygenation or decrease his pCO2. In an effort to expand his lungs and improve his ventilatory function, we decided to perform a bilateral bullectomy.\nAs the patient had poor surgical status, only a right bullectomy was performed 8 days after the trauma. The right side was chosen over the left, because it showed more compression and a larger residual, healthier parenchyma. After surgery, the right side fistula was resolved (). Four days after surgery, the patient developed a right-sided empyema, right lung incarceration, and blood clots in the chest tube (). In order to resolve these issues, videothoracoscopy and decortication of the right lung were performed, which resulted in the resolution of these problems () and improvement in ventilatory parameters with conservative treatment for the left lung. Notwithstanding antibiotic treatment and successful surgical interventions, the patient died on the 25th day of hospitalization due to infectious complications.
A 1-year-old female presented to the Department of Pediatric Surgery with a progressively enlarging forehead swelling for the past 3 weeks. On examination, the swelling was erythematous, tender, fluctuant, 3 × 4 cm in diameter, and located to the right of the midline, consistent with the diagnosis of an abscess. Concurrently, a smaller lesion on the nasal bridge containing a few hair strands was present. The nasal lesion, per the mother's report, had been present since birth.\nA brain MRI was also performed to rule out any possible intracranial extension of the lesion, given its midline location. The MRI revealed an intracranial, extradural tumor, with radiological features suggestive of a dermoid cyst. Additionally, the lesion was complicated by the formation of two dermal sinus tracts, with one opening into the forehead and the other into the nasal region ().\nSubsequently, the patient underwent surgical drainage, with a small incision made at the medial aspect of the right eyebrow, revealing copious amounts of pus.\nThe decision to surgically excise the intracranial mass and debride both the frontal and nasal dermal sinuses was agreed upon by both neurosurgery and ENT departments. Intraoperatively, a sutar bitemporal incision of the scalp was performed, followed by bitemporal craniotomy, exposing the dura mater. The frontal mass, overlying the crista galli, was identified with one tract that led to the forehead and another extending to the nasal lesion (). Because the mass was fused with the parietal dura, extra care was taken to precisely excise it using a sharp surface cut in order to avoid cerebrospinal fluid (CSF) leakage. Although the mass was fused with the outermost layer of the dura, dural invasion was highly unlikely as ruled out by both radiological evidence and the absence of any symptoms in the patient suggestive of chemical meningitis. The mass was successfully excised, intact with no rupture or CSF leakage, and the dermal sinus that led to the forehead was removed. The lower nasal sinus tract was excised by ENT, and the overlying skin was sutured. Lastly, a Hemovac drain was applied and the bone flap was reconstructed. The subcutaneous layer of the scalp skin was approximated with absorbable sutures, and the overlying skin was closed with proline interrupted sutures.\nHistopathology reports on the specimen obtained intraoperatively confirmed the diagnosis of a dermoid cyst with a chronically inflamed dermal sinus tract. Postoperatively, the patient had full recovery with no complications. During subsequent follow-up visits to the neurosurgery clinic, there were no clinical features suggestive of possible recurrence.
A 54-year-old Caucasian female proceeded to our institution with epigastric pain, nausea and vomiting along with pain located around the lumbar area lasting for one week. No previous surgical history or commorbidities existed. Clinical examination did not reveal any palpable abdominal masses or abdominal tenderness and the patient’s vital signs were within the normal spectrum. Blood test detected hypercalcemia (serum calcium: 10.2 mg/dL) and parathyroid hormone level of 111.8 pg/mL. All the findings in conjunction with the clinical presentation lead to the assumption that the patient had primary hyperparathyroidism (PHPT).\nThen, an ultrasound was performed but it was negative for any thyroid or parathyroid abnormalities. Subsequently, the thoracic and abdominal CT revealed a soft tissue in the anterior mediastinum 7 × 1 cm. Additional Tc-99m-MIBI scintigraphy followed, which detected an ectopic adenoma located in the lower anterior mediastinum, on the left of the median line (). Following these, a mid-sternal thoracotomy was finally scheduled.\nDuring the operation, after the thoracotomy, surgeons attempted to detect deep into the mediastinum the parathyroid adenoma according to the preoparative localization. Indeed, the mediastinal mass was detected on the left of the median line, at the anterior mediastinum, in front of the anterior surface of the pericardium and close to the left pericardiophrenic vessels and the left phrenic nerve (). The adenoma was covered by a thin fibrous capsule. When surgeons removed the capsule, a dark red mass of 7 × 2.8 × 1 cm was finally revealed (Figs. and 4 ). The detailful preoperative localization of the present mediastinal adenoma which was in close relation with various anatomical structures of the thorax, reduced effectively the difficulty of the mass excision and the potentiality of accidental surgical injuries which may lead to thoracic bleeding and subsequent obstructive symptoms.\nThen, the operation continued in the usual fashion and a drainage was placed into the left side of the thoracic cavity. The patient was discharged the 5th postoperative day with instructions, when the drainage was finally removed.\nHistology of the mass confirmed the diagnosis of ectopic parathyroid adenoma that was composed predominantly of oxyphil cells arranged in an acinar pattern. Serum calcium level was 2.60 mmol/L and iPTH 17.6 pg/mL 12 h after the operation. Serum calcium and iPTH remained normal after 6 months’ follow-up.
A 63-year-old female was injured after falling off a bicycle and transported to a nearby emergency hospital by ambulance. On initial physical examination, tenderness and swelling were noted over the trochanteric region. She could not move her left leg actively because of severe left hip pain. She had no distal neurovascular deficits or other clinically apparent orthopedic or systemic injuries. Radiography revealed a trochanteric fracture in the left femur, AO type 31-A1.2 (). She underwent closed reduction and internal fixation with a short gamma 3 nail (Stryker, Tokyo, Japan) according to the standard technique (). The lag screw was inserted close to the subchondral bone, but eccentrically in the femoral head. No unusual events were noted during the operative period, and the early postoperative course was uneventful. The patient was mobilized and attained full weight-bearing status prior to discharge 1 month later. At 6 weeks after the first operation, she returned to the hospital with a 1-week history of progressive pain in her left hip, but no trauma. Radiography revealed redisplacement of the fracture and intrapelvic migration of the lag screw through the femoral head and the medial wall of the acetabulum, which was separated from the nail body (). She was referred to our hospital for revision surgery. Intra-abdominal organ injuries were excluded on contrast-enhanced computed tomography (). The lag screw was located deep in the pelvis, between the internal and external iliac vessels, and tangent to the bladder and the sigmoid, but no signs of pneumoperitoneum or hematuria were present. We scheduled a 2-stage operation. First, the migrated lag screw was removed without injury to other pelvic structures. Immediately after the surgery, the pelvis and abdomen were assessed by computed tomography, which revealed no intrapelvic damage due to removal of the migrated lag screw. In the second operation, 3 weeks after the removal surgery, cementless total hip arthroplasty with an autologous bone graft for the bony defect in the acetabulum due to lag screw penetration was performed ().\nThe patient tolerated the procedure well and the postoperative period was uneventful. At the most recent followup 2 years after the revision surgery, she had fully recovered activities of daily living and did not require a walking aid.
A 19-year-old, single, female was referred to the Dental College and Hospital, University of Kerala, India, by a general dental practitioner after treating her in vain with antibiotics for dental abscess for a period of ten days. The patient presented with a complaint of a diffuse swelling on the left side of the mandible, large enough to cause her aesthetic anxiety. She had mild tenderness on palpation of the swelling and slight discomfort in the last molar region of the affected side while chewing. History revealed nothing of significance. At the time of clinical examination, the swelling was of two weeks duration. On examination, the swelling was circumscribed, bony hard, and roughly about 6 × 5 cm in size at the angle of the mandible. The swelling showed diffuse borders. The skin overlying the swelling was of normal color but had a glossy appearance, probably due to tautness of the skin over the swelling (). Intraorally, the swelling was evident on the mandibular buccal vestibule adjacent to the second molar. There was apparent expansion of the buccal cortical plate. Lingual cortical plate also showed expansion but to a lesser extent. The mandibular third molar was missing, and the patient explained that both her mandibular wisdom teeth were extracted due to recurrent infection a couple of years ago.\nPremolars and the first molar on the affected side were healthy with no carious or periodontal involvement. The second molar showed grade I mobility. Bidigital palpation did not reveal any lymph node enlargement anywhere in the cervicofacial chain.\nPanoramic and lateral oblique views of the mandible were ordered. The orthopantogram showed a large radiolucency at the angle of the mandible on the left side, involving the second molar (). The tooth appeared to float in space with bony attachment apparent only mesially. The circumscribed cortical plate expansion showed a centrifugal growth pattern involving the angle and major part of ramus of the mandible (). The maxillary third molar of the affected side was present within the bone, but the mandibular third molar was absent corroborating the history and clinical finding. There was no widening of the periodontal ligament space and there was no sign of periodontal bone loss anywhere else. The inferior margin of the body of the mandible on the affected side had a moth eaten appearance in the lateral oblique view and a discontinuity in the inferior border suggestive of a pathologic fracture was apparent at the junction of the body and ramus.\nAn incision biopsy was performed. The histopathologic picture showed tissue lined with stratified squamous epithelium. Numerous proliferating spindle and oval-shaped mesenchymal cells with tumor osteoid and tumor bone formation were strewn subepithalially. Some areas showed highly pleomorphic cells with hyperchromatic nucleus and bizarre nuclear-cytoplasmic ratio with numerous vascular channels (). Without much difficulty a diagnosis of osteosarcoma, osteoblastic variant, of the mandible was arrived.\nPatient was recalled and interrogated for any paresthesia or numbness over the affected area. She admitted to a tingling sensation which had been present for a long time. Since it had not caused her any discomfort, she deemed it irrelevant to be mentioned. The serum alkaline phosphatase level was within normal limits. The patient was referred to the Regional Cancer Center for expert management. We followed up the patient's progress and learned that her CT scan did not show any metastatic lesion in the body and also her nuclear bone scan showed an increased isotope uptake at the lesional site. The patient was planned for a radical hemi-mandibulectomy, but before the scheduled date she died. Death was due to massive uncontrolled local disease. It had been a mere six weeks from the time she reported to a dental clinic with the complaint of swelling to her demise. At the time of her death, the swelling on her jaw had doubled in size and the skin over the swelling was stretched tighter and had a deep bluish hue to it.
A 35-year-old Caucasian parous woman presented to the clinic with symptoms of spontaneous and periodic bleeding from the umbilicus for four months. The bleeding would start two days before her menses and continue for the entire duration of her period. It was accompanied by pain and swelling in the umbilical area.\nThe patient had regular, heavy and painless menstrual periods and did not wish for any treatment for such. She had two previous spontaneous vaginal deliveries and had no history of abdominal pain, dyspareunia or infertility. She was not using any form of hormonal contraception. Her medical history was not significant and she never had any abdominal surgeries.\nClinical examination revealed that the patient had a 2 cm × 2 cm firm nodule at the umbilicus, which appeared to be covered by a reddish brown discharge. Suspecting that she had an infection, the patient was swabbed and given a five-day course of oral broad-spectrum antibiotics. She showed up on check up two months later with no relief of symptoms. She then underwent an ultrasound scan that showed a 15-mm thin-walled cyst, approximately 5 mm below the skin surface. The key clinical feature that led to the correct diagnostic hypothesis of umbilical endometriosis was the temporal association of the bleeding with her menstrual period.\nThe patient was offered both medical and surgical management and she opted to have depot injections of Zoladex (AstraZeneca UK, Goserelin acetate, 3.6 mg subcutaneously, monthly). The swelling continued to persist in spite of three doses of Zoladex, and the patient then requested surgical excision. The risk of recurrence and scar endometriosis were explained to her.\nThe patient successfully underwent excision of the nodule with accompanying umbilical reconstruction. Histology confirmed the diagnosis of endometriosis and revealed the presence of endometriotic glands with mucinous type metaplasia and extravasation of the mucinous secretion into the adjacent stroma (Figure ). No epithelial atypia was seen and the excision appeared complete. The patient was seen six weeks after the surgery and found to be asymptomatic with a normal umbilicus. Before being discharged, the patient was again reminded of the risk of recurrence.
A 70-year-old male was referred to our hospital complaining of left flank pain. At physical examination, nothing relevant was observed and laboratory findings were within normal limits. A CT examination of the abdomen and the pelvis was performed with a multidetector scanner, before and after contrast media administration. Portal phase images showed a large amount of solid tissue in the left perirenal space, infiltrating the renal capsule and the main renal vessels; the tissue did not show significant contrast enhancement. Similar findings were detected also in the right perirenal space (). CT images also revealed a partial stenosis of the common bile duct, with intrahepatic bile duct ectasia owing to hypervascular eccentric tissue (). Hence, a diagnostic integration with endoscopic retrograde cholangiopancreatography was performed to exclude an intraductal proliferation. A biopsy was also performed in the left perirenal space; the pathological samples were composed of connective and adipose tissues, revealing the histological features of a DT or abdominal fibromatosis and also showing immunohistochemical markers typical of muscular tissues, such as actine. The tumour was considered unresectable and medical therapy was started with tamoxifen (20 mg die–1); after an episode of thrombophlebitis, the patient asked to suspend tamoxifen and accepted a new therapeutic regimen (docetaxel 75 mg m−2 every 3 weeks); unfortunately, even this treatment was discontinued after only 4 weeks owing to neuropathy. A new CT examination was then performed to assess the results of the treatment. Unexpectedly, on venous phase images, the perirenal tissue showed a remarkable reduction on the left side and had almost disappeared on the right side (). The peribiliary tissue had equally decreased in size and thickness (). The treatment was then discontinued in accordance with the patient’s decision. 1 year later, both the left perirenal and peribiliary tissues demonstrated no progression and remained clinically stable on off-treatment; however, new tissue was visible surrounding the right renal pelvis and the calices (). The same therapeutic regimens (tamoxifen and docetaxel) were proposed to the patient based on the previous good response; unfortunately, the patient refused any treatment.
A 62-year-old male without neurofibromatosis presented with an enlarging, painful mass in the third web space of his left foot. The lesion was first noticed approximately five years earlier as a nodular lesion that was then diagnosed as an epidermal cyst of the sole at a dermatologic clinic. Neither excision nor biopsy was performed at that time. On examination, there was a firm, round mass measuring 9 × 7 cm in the third web space (). The mass was immobile against the surrounding bones and adhered to the overlying skin. Multiple swollen lymph nodes were palpable in the left inguinal region. An open biopsy was performed, which showed proliferation of epithelioid round cells with cellular atypia, indicating probable malignant melanoma. Radiological examination including fluorodeoxyglucose positron emission tomography showed no evidence of definite distant metastases except left inguinal region (Figures and ). Therefore, surgical treatment was planned under the diagnosis of T4aN2bM0, stage III-C malignant melanoma according to the American Joint Committee on Cancer classification.\nThe first operation was performed under general anesthesia. Left inguinal dissection was carried out (), and an adequately wide excision of the primary tumor with a 30 mm surgical margin (i.e., transmetatarsal amputation) was performed (). The resultant raw surface of the stump was temporarily covered with an artificial dermis. One week postoperatively, the stump was resurfaced with a free latissimus dorsi musculocutaneous flap with a split-thickness skin graft following histological confirmation of adequate surgical margins (). Although partial necrosis of the flap was observed postoperatively, application of an additional split-thickness skin graft under local anesthesia led to complete wound healing. The specimen showed nodular growth of the lesion measuring 70 × 43 mm between the dermis and subcutaneous layer without continuity to the epidermis. Histologically, the lesion consisted of two components: proliferation of spindle cells with some differentiation to nerve cells, reminiscent of conventional malignant schwannoma (), and epithelioid proliferation of round-to-polygonal or rhabdoid cells with hyperchromatic nuclei, reminiscent of malignant melanoma (Figures and ). Tumor cells stained positive for S-100 protein and negative for HMB45 (). All dissected lymph nodes were free of sarcoma. Based on these histological features, the lesion was diagnosed as EMPNST, of which histological grade was classified as grade 3 according to the FNCLCC system (tumor differentiation: score 3, mitotic count: score 2, and tumor necrosis: score 1).\nTen weeks after the first operation, computed tomography demonstrated the development of multiple small bilateral lung nodules (Figures and ). No further evidence of metastasis to other organs was observed. Systemic adjuvant chemotherapy consisting of doxorubicin and ifosfamide was then initiated. Doxorubicin (60 mg/m2) and ifosfamide (7.5 g/m2) with mesna (Uromitexan) were administered. After two courses of the adjuvant therapy, follow-up computed tomography revealed a good response to all abnormal shadows; however, two lesions in segment 8 and one in segment 10 of the left lung field were still observable (Figures and ). Because these three lesions were located just beneath the pleura, tumors were resected during thoracoscopic surgery five months after the first operation. Histological examination revealed complete necrosis of the tumor cells in one lesion and metastases of EMPNST in the other two lesions. At the 12-month followup after radical surgery of the primary tumor, during which time four courses of adjuvant chemotherapy and a thoracic surgery were added, no signs of local recurrence or metastasis were observed (Figures and ). The patient maintained ambulation without a brace ().
A 67-year-old man from India, who was recently diagnosed with latent tuberculosis, presented to an outside hospital and was admitted for six days with generalized fatigue and hypotension. On presentation, he reported a 2-week history of generalized fatigue with systolic blood pressures in the 60s, as recorded at home. He also reported a 30-pound weight loss which was attributed to diuretic use and dietary changes in the setting of congestive heart failure. With these complaints, his losartan (angiotensin receptor blocker) dose was reduced, which was ineffective in alleviating his symptoms. His history was notable for an LTBI diagnosed three months prior to presentation, for which he was being treated with INH (300 mg daily) and pyridoxine. He had been treated for 11 weeks by the time he presented, and was noted to tolerate the therapy well for at least four weeks without changes in dietary or sleeping habits, per documentation by his primary care provider. His medical history was otherwise notable for atrial fibrillation, beta thalassemia, and tachycardia-induced cardiomyopathy with reduced ejection fraction. He had no known history of liver disease or diabetes. His other medications included apixiban, metoprolol, furosemide, losartan, and hydroxyzine (for insomnia). He had no prior history of heavy alcohol consumption or recreational drug use, and he worked in the electronics and computer industry. There was no family history of cirrhosis or other liver disease.\nHis initial workup was notable for elevated liver function tests, as shown in . A computed tomography scan of the abdomen demonstrated no intrahepatic biliary dilation and no apparent fatty change. An abdominal ultrasound revealed a simple cyst and heterogenous echotexture, with mild subcapsular nodularity. Based on his workup, his liver dysfunction was postulated to be drug-induced liver injury from INH or apixaban. Both medications were discontinued. He was discharged for outpatient management. However, follow-up laboratory testing indicated an increasing bilirubin, which resulted in readmission to the hospital. A liver biopsy was considered but not performed, as his bilirubin levels began to downtrend.\nOne week later, the patient experienced a syncopal episode while having a bowel movement, and was admitted to a different hospital. He presented with altered mental status and generalized pruritis. Laboratory data revealed worsening liver function () and acute kidney injury, for which he was treated with albumin, midodrine, and octreotide. His pruritis improved with the introduction of cholestyramine.\nAutoimmune serologies, including antinuclear antibody, antimitchondrial antibody, and anti-smooth muscle antibody, were negative. Viral hepatitis serologies were negative. HIV infection was ruled out. The patient’s acetaminophen level, urine toxicology screen, and serum ferritin level were within normal limits. A transjugular liver biopsy was performed and demonstrated cholestatic hepatitis, thought to be drug- or toxin- related. His mental status initially improved with lactulose, but worsened thereafter, prompting a transfer to our hospital for consideration of liver transplantation for acute liver failure, about six weeks after initial presentation.\nPhysical examination at time of transfer was remarkable for jaundice, icteric sclera, and altered mental status. He was somnolent but arousable to noxious stimuli, oriented only to self, intermittently following some commands, and had notable asterixis. He had an irregular rhythm and a murmur on cardiac examination, vesicular breath sounds, and a benign abdominal examination. He was noted to have Grade III hepatic encephalopathy in the setting of acute liver failure, and was admitted to the intensive care unit before being listed for liver transplantation as Status 1A. On day four in the hospital, he underwent orthotopic liver transplant surgery, and was extubated on post-operative day one. He was transferred to the floor on post-operative day three, but subsequently suffered a sudden aspiration event resulting in a pulseless electrical activity and fatal cardiopulmonary arrest.
A 79-year-old woman, 145 cm in height and 37 kg in weight, presented with a pelvic fracture due after falling from a standing height when walking in her room. She was unable to walk with low back pain. The patient was moderately healthy with some comorbidities (hypertension, cerebral infarction). The paralysis of the cerebral infarction was slight, and she was able to walk independently before this injury). Her American Society of Anesthesiologists physical status classification [] was III.\nX-ray and CT taken in the previous hospital showed few dislocations of the sacral fracture (). After that, bed rest/conservative treatment and administration of PTH injection were performed in the previous hospital, but the pain did not improve over some weeks and walking was difficult. She was referred to our hospital because the pain persisted and the treatment was unsuccessful. CT taken at our hospital showed that the dislocation of the sacral fracture had worsened (). The diagnosis of type IVb FFPs, displaced sacral fracture, was made from CT. In CT, a sacral corridor for inserting the transiliac transsacral (TITS) screw existed, but the TITS bar was not be approved to be used in Japan, and in this osteoporotic case with dislocation of the sacrum, there was concern about fixation with the TITS screw. On the other hand, in this case, L5 and S1 were bonny fused, and we decided to perform SIRF with inserting a pedicle screw into L5 and S1, fixing the fractured bone directly, and connecting it to the iliac screw. The operation was performed 2 months after the injury and 4 days after the transfer to our hospital under general anesthesia with the patient in the prone position.\nAbout 5 cm incisions were made to the skin bilaterally placed medial to both the posterior superior iliac spines (PSIS). The fascia was peeled away from the surfaces of the PSIS to develop sufficient space for manipulation of the iliac screws (IS) and S1 pedicle screws (S1PS). First, the bilateral S1PS were aimed at the promontory. The right L5 pedicle screw was inserted (left L5 pedicle screw was not inserted this time because it was inserted from the fractured site in the case). The entry point of the IS was set 2–3 cm distal to the head of the S1PS. With image intensifier position for the “teepee” view, a guidewire was placed from the PSIS toward the anterior inferior iliac spines (AIIS). Iliac screws (φ9.5-90 mm) were inserted []. Constructs were made using spinal instruments (Solera, Medtronic Co., Dublin, Ireland) (). The surgery time was 2 h and 56 min, and intraoperative blood loss was 95 g. Immediate weight bearing as tolerated was allowed postoperatively.\nThe patient continued PTH injection treatment after the surgery. CT examinations (postoperative 6 months) were performed to determine the progress of bone union (). The patient experienced no pain waking with a walking stick and returned to most social activities including living independently within 6 months of the operation. The modified Majeed score was 94 (except sexual intercourse, which was 4 points out of a possible 96) at the visit of 6 months after operation. No particular implant-related complications such as skin irritation, screw loosening, or buck out have occurred.
A 14-year-old male had an extensive flame burn in the right side of neck, face, and scalp, anterior chest, and right upper limb at the age of 2 years.\nNo past medical or surgical history. No known drug history, no family history of any genetic disorder, patient and both parents are non-smokers.\nHe received multiple skin grafting procedures and an amputation of the right hand. The burn to his scalp was treated with a burr hole to the outer cortex to allow granulation tissue to cover the scalp, and then the wound was skin grafted.\nThe patient chronically continued to have unstable skin over the scalp and kept developing recurrent ulceration. On 29 March 2012, the patient was admitted throw the clinic due to his chronic condition of recurrent open wounds for many years.\nA plan was set for the removal of the unstable ulcer and scarred skin of the scalp and coverage with an omental free flap and split thickness skin graft ().\nUnder general anesthesia with endotracheal intubation, the patient was in supine position, with draping of the head circumflex, face, abdomen, and left thigh down to the knee.\nThe General Surgery team was consulted for laparoscopically harvesting the omental flap and was performed by Dr I.Anwar based on the left gastroepiploic artery. The right superficial temporal artery was identified to be the recipient vessel ().\nThe ENT team did a right neck exploration to explore the neck vessels as a backup in case the right superficial temporal artery was not suitable.\nThe free omental flap based on the left gastroepiploic artery was anastomosed to the right superficial temporal artery. The micro procedure of omental free flap was performed by DR F.Hashem, Plastic surgeon.\nThe patient was given heparin 2000 U intraoperatively. The flap was viable with bleeding with a positive Doppler signal.\nAfter establishing flap revascularization, all the excising unstable scarred and ulcerated skin covering the scalp was resected, and the underlying irregular skull was smoothed down using a flat burr. The omental flap then covered the scalp, and a split-thickness skin graft from the thigh was placed over the omental flap ().\nThe patient was monitored postoperatively in intensive care unit. Post-operative care for microsurgery and wound care for skin graft over omental flap.\nThe hospital course was uneventful. The patient was kept on IV anticoagulants (dextran and heparin) for 5 days.\nThe patient tolerated the procedure well. No wound complications were reported during his two years follow-up (). Re-Exploration nor revision of the surgery were needed
A 10-year-old girl was referred by her local hospital to a tertiary centre for an upper GI endoscopy and biopsy. The girl had been diagnosed with insulin-dependent diabetes mellitus (IDDM) 4 years previously, remaining clinically well until four months prior to referral, when she presented with a history of weight loss (approximately 10% of her weight). During an assessment at her local hospital it emerged that an antitissue transglutaminase antibody (tTG) blood test (a screening test for CD) performed at the initial presentation of her IDDM, had been positive. Now presenting with weight loss, she was referred urgently for further investigation.\nThe patient had no overt gastrointestinal symptoms, but interestingly did have a 4-month history of lymphadenopathy. She also had a recent infected insect bite on her arm, lived with cats and had had contact with a family member with suspected tuberculosis (TB). The lymphadenopathy, located in her left groin, was nontender, varied in size, and had improved when treated with antibiotics at her local hospital. An ultrasound scan performed at her local hospital was suggestive of infected lymph nodes. Clinically the patient had no other palpable nodes, and there was no hepatosplenomegaly. The initial differential diagnosis included borrelia, bartonella, and TB.\nThe case was complicated by the patient's significant needle phobia and it was decided that all haematological investigations would be performed whilst the patient had an upper GI endoscopy under general anaesthesia. An ultrasound scan was repeated, confirming the enlarged nodes in her groin, and also demonstrating para-aortic lymphadenopathy. A subsequent CT scan also demonstrated cervical lymphadenopathy as well as the abdominal lymphadenopathy, and at this stage a diagnosis of lymphoma was considered.\nDuring the general anaesthetic, a groin lymph node was removed for histopathology. Blood taken at this time showed normal haemoglobin and white cell count, but a mildly elevated platelet count (600 × 109/L) and inflammatory markers (CRP 29 mg/L and ESR 36 mm/hour). A peripheral blood film was normal. A repeat tTG was initially mildly elevated at 16 u/mL and subsequently negative at 0.7 u/mL (normal range 0–7 u/mL).\nDuodenal biopsy of small bowel confirmed the diagnosis of CD with villous atrophy, crypt hyperplasia, and increased intraepithelial lymphocytes (Marsh 3a-b according to the modified classification, ), and so the patient was commenced on a gluten-free diet.\nThe lymph node biopsy showed morphology and immunohistochemistry of an ALCL ().\nHLA-typing demonstrated that she was homozygous for HLA-DQ8, which is associated with higher risk for coeliac disease, more severe gluten sensitivity, and diabetes susceptibility. Treatment was given according to the European ALCL 99 protocol, with six courses of multiagent chemotherapy. The patient responded well to treatment and 4 years following chemotherapy remained in clinical and radiological remission.
A 67-year-old man from India, who was recently diagnosed with latent tuberculosis, presented to an outside hospital and was admitted for six days with generalized fatigue and hypotension. On presentation, he reported a 2-week history of generalized fatigue with systolic blood pressures in the 60s, as recorded at home. He also reported a 30-pound weight loss which was attributed to diuretic use and dietary changes in the setting of congestive heart failure. With these complaints, his losartan (angiotensin receptor blocker) dose was reduced, which was ineffective in alleviating his symptoms. His history was notable for an LTBI diagnosed three months prior to presentation, for which he was being treated with INH (300 mg daily) and pyridoxine. He had been treated for 11 weeks by the time he presented, and was noted to tolerate the therapy well for at least four weeks without changes in dietary or sleeping habits, per documentation by his primary care provider. His medical history was otherwise notable for atrial fibrillation, beta thalassemia, and tachycardia-induced cardiomyopathy with reduced ejection fraction. He had no known history of liver disease or diabetes. His other medications included apixiban, metoprolol, furosemide, losartan, and hydroxyzine (for insomnia). He had no prior history of heavy alcohol consumption or recreational drug use, and he worked in the electronics and computer industry. There was no family history of cirrhosis or other liver disease.\nHis initial workup was notable for elevated liver function tests, as shown in . A computed tomography scan of the abdomen demonstrated no intrahepatic biliary dilation and no apparent fatty change. An abdominal ultrasound revealed a simple cyst and heterogenous echotexture, with mild subcapsular nodularity. Based on his workup, his liver dysfunction was postulated to be drug-induced liver injury from INH or apixaban. Both medications were discontinued. He was discharged for outpatient management. However, follow-up laboratory testing indicated an increasing bilirubin, which resulted in readmission to the hospital. A liver biopsy was considered but not performed, as his bilirubin levels began to downtrend.\nOne week later, the patient experienced a syncopal episode while having a bowel movement, and was admitted to a different hospital. He presented with altered mental status and generalized pruritis. Laboratory data revealed worsening liver function () and acute kidney injury, for which he was treated with albumin, midodrine, and octreotide. His pruritis improved with the introduction of cholestyramine.\nAutoimmune serologies, including antinuclear antibody, antimitchondrial antibody, and anti-smooth muscle antibody, were negative. Viral hepatitis serologies were negative. HIV infection was ruled out. The patient’s acetaminophen level, urine toxicology screen, and serum ferritin level were within normal limits. A transjugular liver biopsy was performed and demonstrated cholestatic hepatitis, thought to be drug- or toxin- related. His mental status initially improved with lactulose, but worsened thereafter, prompting a transfer to our hospital for consideration of liver transplantation for acute liver failure, about six weeks after initial presentation.\nPhysical examination at time of transfer was remarkable for jaundice, icteric sclera, and altered mental status. He was somnolent but arousable to noxious stimuli, oriented only to self, intermittently following some commands, and had notable asterixis. He had an irregular rhythm and a murmur on cardiac examination, vesicular breath sounds, and a benign abdominal examination. He was noted to have Grade III hepatic encephalopathy in the setting of acute liver failure, and was admitted to the intensive care unit before being listed for liver transplantation as Status 1A. On day four in the hospital, he underwent orthotopic liver transplant surgery, and was extubated on post-operative day one. He was transferred to the floor on post-operative day three, but subsequently suffered a sudden aspiration event resulting in a pulseless electrical activity and fatal cardiopulmonary arrest.
A 68-year-old man presented for evaluation of a new asymptomatic bump on his left arm. His previous skin examination had been six months earlier and the lesion had not been present. He previously had three basal cell carcinomas (on the left temple, left side of his upper lip, and left mid back excised 32 years, four years and two years earlier, respectively) and one squamous cell carcinoma (on his right upper back that was excised seven years ago). He also had actinic keratoses that were treated with liquid nitrogen cryotherapy.\nHis past medical history was significant for severe acne vulgaris as an adolescent, hypercholesterolemia, hypertension, and prostate cancer that was diagnosed one year ago. He is currently with no evidence of malignant disease after treatment which included a robotic-assisted laparoscopic prostatectomy (with negative margins for tumor) and a bilateral pelvic lymph node dissection (with none of eight nodes positive for cancer). His current oral daily medications included amlodipine 10 mg and simvastatin 20 mg.\nCutaneous examination showed a six by six millimeter flesh-colored nodule on the extensor aspect of his left arm near the elbow (Figure ). A shave biopsy of the superficial portion of the nodule was performed. The site was treated topically with mupirocin two percent ointment, three times daily, until it had healed.\nMicroscopic examination of the hematoxylin and eosin-stained tissue specimen showed strands and nodular aggregates of atypical basaloid tumor cells in the dermis reaching the deep margin of biopsy; the overlying epidermis was thin with effacement of the rete ridges and sparse overlying orthokeratosis (Figure ). Deposition of amorphous material filled the dermal stroma between the tumor aggregates (Figure ); the amount of amyloid present was abundant (+++). The lateral aspect of the specimen showed solar elastosis in the upper dermis above the tumor and below the epidermis (Figure ).\nCorrelation of the clinical presentation and pathological findings established the diagnosis of a nodular basal cell carcinoma with extensive amyloid deposition and adjacent solar elastosis. The residual tumor was excised and a side-to-side layered closure was used to resolve the surgical wound. The surgical site was healed without recurrence at a follow-up examination nine months later.
Our patient was an otherwise healthy 43-year-old male who had intermittently taken prescription methadone, fentanyl, and oxycodone over a 14-year period of time for chronic low back pain. In the past 2 to 3 years, he used heroin after coming home from working his job during the night shift; he would inject heroin intravenously to relax and fall asleep, and later would join his spouse and daughter for dinner before going back to work. Aware of the severity and progression of his problem, the patient had reached out to several local addiction treatment programs. Upfront costs, required travel to a treatment center, and the inability to be away from work prevented him from participating in any treatment program.\nOn one occasion, the heroin he obtained was more potent than expected. He injected himself and fell asleep. When he did not answer his spouse’s calls, she came home from work and found him unresponsive and apneic; this was just moments before their pre-teen daughter would have come home from school. His spouse, a layperson, was unable to locate naloxone in their home and performed cardiopulmonary resuscitation until first responders arrived, even though she does not work in health care herself. First responders administered 2 intranasal doses of naloxone as the patient was transported to the emergency department (ED) of the critical access hospital in the same town. There, he was medically stabilized and monitored overnight. Coincidentally, his family medicine physician, who cared for his entire family and was familiar with the patient, was working in the ED that night. He was aware of Suboxone therapy for OUD being offered by his colleagues in the outpatient practice and made an urgent referral to the MAT provider. The following morning, the patient was seen in the family medicine clinic. He was actively in withdrawal with a COWS score of 16, indicating moderate withdrawal. Suboxone therapy was initiated according to the MAT protocol, 2 mg initially and 2 mg every hour thereafter for a total of 4 doses. He was stabilized over several days of follow-up at a dose of 8 mg of Suboxone twice daily. Follow-up consisted of frequent weekly visits for the initial 4 weeks, monthly visits for 6 months, and then continued office visits every 3 months thereafter.\nAt a follow-up visit after 6 months of MAT, the patient was motivated to share his positive experience with others and referred 2 people for MAT in our practice. One year after beginning MAT, he was still taking Suboxone at 8 mg twice daily and felt that he was ready to begin weaning to a lower dose. He was working, had received a promotion, was actively participating in family activities, and made it a point to attend all of his daughter’s school events. He and his family remain in our family medicine practice and are otherwise physically and emotionally well.
A 24-year-old healthy woman presented with difficulty breathing and dissatisfaction with her facial appearance. She had a history of childhood trauma resulting in nasal septum deviation and external nasal deformity. Four months after a successful and uneventful septorhinoplasty, she presented to the emergency department with blunt nasal trauma resulting in a septal hematoma, which was drained successfully; the patient was discharged with no adverse sequelae.\nFour months later, the patient sustained nasal trauma again, this time accompanied by clear nasal discharge, raising suspicion of cerebrospinal fluid (CSF) leak. The patient was discharged after managing the nasal injury, as the CT brain showed an intact cribriform plate with no evidence of a CSF leak. Ten days later, she presented at the emergency department with dizziness and an unstable gait. She also had complaints of paresthesia for the past two months, beginning in her right hand and progressing to the right shoulder, arm and leg, associated with some difficulty in the execution of movements in the first and second finger of the right hand. Her right leg was quite stiff with difficulty in walking. On close inquiry, she gave history of pain in the right eye and double vision many months back, which had resolved spontaneously. Examination showed a positive Romberg’s and Lhermitte’s sign, with right-sided sensory impairment.\nMagnetic resonance imaging (MRI) of the brain, cervical and thoracic spine demonstrated demyelinating lesions in the brain and cervical segment of the spinal cord (Figure ). Some of the lesions demonstrated enhancement on post gadolinium administration sequences, suggestive of active demyelinating diseases like MS. A lumbar puncture was performed which demonstrated the presence of oligoclonal bands in the CSF. The diagnosis of MS was confirmed by a neurologist and treatment was initiated.\nThe initial neurological symptoms have largely vanished with only persistent light paresthesia in the right hand. Two years later she has had no new symptoms and continues with the same medication with good tolerance.
A 59-year-old female with a medical history of chronic hepatitis C was referred for management of a right nonhealing neurotrophic ulcer due to Bell's palsy. One-year prior, she reported experiencing “lightning bolt” pains across the right side of her face. She was referred to a neurologist who did not note any abnormality of facial sensation or motor function at that time; a brain MRI was obtained and was unremarkable (this study was not dedicated to the fifth nerve, and no comment was made on structures inferior to the skull base in this report). She was diagnosed with trigeminal neuralgia and treated with carbamazepine. Subsequently, she noted a decrease in visual acuity and was evaluated by optometry where a serological workup for temporal arteritis was ordered and returned negative. She went on to develop right hearing loss and noticed right temporal wasting. She developed a right-sided facial paralysis and returned to her neurologist who now documented right fifth and seventh nerve palsies. As a result, she underwent a lumbar puncture with normal protein and cell counts as well as negative results for HSV, West Nile, and tuberculosis. At this time, her ophthalmologist independently documented right facial weakness, decreased facial sensation, and neurotrophic keratitis consistent with fifth and seventh nerve palsies. The patient was told her symptoms were a result of Bell's palsy. No repeat neuroimaging was obtained after the onset of her facial weakness. She was treated with pressure patching of the affected eye but, due to a lack of resolution, was ultimately referred for management of the neurotrophic ulcer.\nOn presentation to the oculoplastic service, visual acuity was count fingers and she demonstrated 6 mm of lagophthalmos, a poor Bell's reflex, and a large central corneal epithelial defect (Figure ) Incomplete facial and trigeminal nerve palsies were diagnosed on the basis of lagophthalmos and reduced corneal sensation, and additional workup with neuroimaging was urgently obtained to determine the underlying etiology. Maxillofacial computed tomography with contrast demonstrated a lytic right mandibular lesion at the level of the ramus (Figure ). Fluorodeoxyglucose (FDG)-positron emission tomography (PET) scan revealed uptake in the right mandible (Figure ). A biopsy of this lesion demonstrated spindle cell sarcoma with perineural spread along the trigeminal nerve. Following her diagnosis, she developed a right abducens nerve palsy concerning for further extension, and MRI of the orbit, face, and neck demonstrated involvement of the 5th and 7th nerve with intracranial spread and enhancement of the right pons and middle cerebral peduncle (Figure ). Her malignancy was deemed inoperable due to its extent, and she went on to receive palliative chemoradiation and then expired one and a half years after presentation.
A 42-year-old male presented with a history of swelling on the right side of the upper jaw in the molar area. A biopsy proved it to be low-grade mucoepidermoid carcinoma. An inferior maxillectomy with resection of maxillary alveolus distal to the canine tooth and adjacent hard palate was carried out. This resulted in a Class IIa defect according to classification of maxillectomy defects by Brown et al.[][]. The defect size was 3 cm × 4cm. The objective of reconstruction was to get an oro-antral separation and to provide a base for the dental prosthesis. The defect was reconstructed with a free radial forearm adiposo-fascial flap harvested from the left forearm.\nAdiposo-fascial flap harvesting involves few technical modifications from the conventional radial forearm fascio-cutaneous free flap harvesting. The flap was marked on the forearm skin. The margin of the flap was marked with a hypodermic needle dipped in methylene blue, the tip of the needle needed to reach the subcutaneous layer []. A lazy “S” incision was placed extending from a point 2 cm proximal to the wrist crease to a point 2 cm distal to the ante-cubital fossa. The skin flap was elevated in the subcutaneous plane to leave the layer of adipose tissue on the fascia []. The fascia with fat, incised along the dye markings, was harvested based on the radial artery []. The methylene blue marking helped to limit the dissection beyond the requirement. The width of the flap that can be harvested by this technique is similar to the conventional radial forearm flap. The skin over the flap site is raised at a superficial level to expose the adiposofacial tissue. Hence, large flaps may compromise the vascularity of this skin flap and lead to necrosis at the incision site. So it would be safer to restrict the size of the flaps to the width of the forearm. The donor site was closed primarily in two layers, the subcutaneous tissue with absorbable 3-0 Polyglactin 910 (Vicryl®) and skin with 3-0 nylon suture. The two layered closure allowed better approximation of the skin edges. A suction drain was used to prevent any subcutaneous hematoma. This was removed after 3 days. The donor site required no immobilization. Immobilization is usually required for 1 week after the harvest of the conventional fasciocutaneous flap and split skin graft for the defect. The flap was then sutured to the defect edges and the radial artery was anastomosed to the superior thyroid artery and the accompanying venae commitantes was anastomosed to a direct tributary of the internal jugular vein through a right neck crease incision. The flap was monitored by the color, by observing the nature of bleeding from the adiposal tissue by pinprick method, and from the Doppler signals of the artery. The patient was kept on nasogastric tube feeds for 5 days; after 5 days, a soft diet was started. Solid food was allowed after 2 weeks.\nThe primary site healed well with rapid re-epithelialization of the fascial subcutaneous layer to achieve a mucosal surface. The flap underwent some amount of contraction providing a taut immobile base for the prosthetic dental rehabilitation []. There was no restriction of mouth opening before or after the surgery. The defect did not crossover to the cheek, hence, there was no significant obliteration of the sulcus. It was limited laterally to the bony alveolus. This prevented the cheek from retracting in. Since this was a Stage II disease, the patient did not receive any adjuvant treatment. shows a view of the palate well mucosalized with no intraoral hair at the end of 2 year follow-up. Functionally, he could eat a normal diet, his speech was normal, and he had no nasal regurgitation. The donor site healed well. Primary and donor sites were aesthetically satisfactory.
A 20-year-old male referred to our neurosurgical clinic as a case of multiple periventricular mass, suspected tuberculomas, with obstructive hydrocephalus on anti-tubercular therapy for 2 months. The available previous records of the patients showed a history of difficulty in walking (generalized weakness) for 2 months and altered sensorium for 5 days; there was no other significant history. Magnetic resonance imaging (MRI) brain showed prominent nodular hyperintensities around the left occipital horn, bilateral frontal horns, right thalamic region, and altered signal intensities in the corpus callosum []. The patient was managed conservatively and was discharged (no details were available). The patient again presented, 3 months later, with complaints of altered sensorium and no motor deficit. Routine blood and CSF investigations were within normal limits. The available images showed progression of the lesion with diffuse involvement of the left occipital horn, third ventricle, and bilateral frontal horn with the appearance of new solid-cystic lesions around the frontal horn and pineal region. Magnetic resonance spectroscopy showed lipid peak []. The patient was started on anti-tubercular therapy with steroids on a suspicion of multiple tuberculomas as a differential diagnosis. The patient improved clinically and was discharged. Now, the patient again presented to the neurosurgery emergency of the same hospital with complaints of multiple episodes of vomiting and generalized weakness. Noncontrast computed tomography (NCCT) scan of the brain showed significant diffuse nodular densities around the lateral and third ventricle with an irregular ventricular enlargement []. At the current presentation, the patient was in altered sensorium for 5 days and on examination, he was drowsy and spontaneously moving all the four limbs equally with Glasgow Coma Scale E4V2M5; his bilateral pupils were reacting to light and fundus examination showed bilateral papilledema. A NCCT brain showed obstructive hydrocephalus along with previous lesions []. The patient was planned for urgent craniotomy and decompression. Right pterional craniotomy and tumor decompression was done. The lesion was heterogeneous in the consistency, mildly vascular, suckable, infiltrating into the 3rd ventricle and frontal horn, encasing the anterior cerebral artery without any plane of cleavage. The histopathological examination was suggestive of morphological features of Germinoma with immunohistochemistry features were also characteristic of Germinoma with oct3 and SALL4 positivity [].
A 50-year-old female with no ocular history and a medical history of diabetes mellitus presented to the oculoplastic service for management of right eye lagophthalmos due to Bell's palsy. The patient reported that 4 months prior to presentation her right face became swollen and painful following a tooth extraction. This facial swelling was attributed to a dental abscess, and she went on to require intensive care unit (ICU) level care at an outside hospital for cellulitis in the setting of diabetic ketoacidosis. Her swelling resolved with antibiotic therapy, and she followed with her primary medical doctor for the next four months (a total of eight office visits with no documentation of a cranial nerve examination) for persistent complaint of right-sided facial pain and weakness. Concurrently, she was followed by an ophthalmologist (three visits) for difficulty closing her right eye. Lagophthalmos and corneal exposure with inferior corneal scarring were noted but neither the patient's visual acuity nor the function of cranial nerves other than the facial nerve were documented at these ophthalmologic evaluations. Both providers documented concern for “Bell's Palsy,” and the patient was treated with oral corticosteroids. After 4 months, her medical doctor recommended neuroimaging (magnetic resonance imaging [MRI]) but she was referred to oculoplastics for further management of her persistent lagophthalmos prior to obtaining this study.\nOn presentation, she had no light perception visual acuity in the right eye. Her examination was also significant for House-Brackmann grade 4 palsy of the right facial nerve and loss of sensation in the V1 distribution of the right trigeminal nerve. (Figure ) The severity of keratopathy limited posterior examination, but the right optic nerve appeared pallid. Further workup was performed, and a dedicated MRI of the orbit with and without gadolinium contrast demonstrated optic nerve atrophy, abnormal enhancement along the trigeminal nerve with extension to Meckel's cave, and edema of the right temporal lobe concerning for perineural spread and cerebral parenchymal involvement of an invasive malignancy (Figure ) Subsequent transnasal endoscopic biopsy of the pterygopalatine fossa demonstrated fungal elements which were identified as Mucorales species, and she was diagnosed with chronic mucormycosis of the right muscles of mastication and skull base. She was treated with amphotericin and micafungin and has remained symptomatically and radiographically stable in follow up for over two years.
A 68-year-old male, farmer by occupation, presented with the complaints of nasal blockage and intermittent streaking of blood from left nostril for six months. There was no relevant past medical / family history. On examination, there was swelling of the left alar region. Anterior rhinoscopy showed a shiny polypoidal mass in the left nasal cavity which was friable and bled on touch. Patency was reduced and cavity was filled with blood stained discharge. Posterior rhinoscopy was normal. Examination of the throat, ears and larynx were normal. Evaluation of the case demonstrated only mild anemia with otherwise normal baseline hematological and biochemical profile. Urine and stool examinations were normal. Chest radiograph, ultrasonography of abdomen and all other relevant investigations were normal. Computed tomographic (CT) scan showed an enhancing soft tissue lesion in left nasal cavity with a suggestive diagnosis of a polypoidal mass not otherwise specified. CT guided aspiration cytology of the lesion was carried out. The smear showed large epithelioid and few spindle cells showing hyperchromatic nuclei, macronucleoli and both intra- and extra-cellular melanin pigments that led to a provisional diagnosis of malignant melanoma []. All other possible sites of primary malignant melanoma were excluded. The patient was operated and tumor was excised by lateral rhinotomy. The tumor was polypoidal and capsulated which ruptured during removal and hence it was removed in pieces. The tumor was attached with a broad base and was found on the inferior turbinate of left nasal cavity. After removal of tumor, the gross examination revealed several bits of blackish, irregular, soft and friable tissue, totally aggregating to form 5 cm × 6 cm mass. The hematoxylin and eosin stained section showed the tumor was composed of epithelioid and spindle cells which were medium to large in size, arranged in lobules and small bundles. The nuclei were pleomorphic, hyperchromatic with prominent nucleoli. Both extra and intracytoplasmic melanin pigment was noted []. Scattered mitoses and tumor giant cells were also seen. The overlying epithelium was ulcerated and only few intraepithelial atypical melanocytes noted. The case was diagnosed as primary mucosal malignant melanoma. HMB-45 and S-100 positivity further confirmed the diagnosis. Radiotherapy was given following complete wound healing. The patient is being followed up and till date there has been no recurrence.
Our patient was a 23-year-old Iranian man who complained of diffuse headache and several bursts of vomiting on the day of presentation. He told us that three days before, he had received a traumatic head injury after falling from his bicycle at a low speed due to an obstacle. He had no post-traumatic amnesia, loss of consciousness, seizure or visual blurring. Initially, his headache was mild and focal, but its severity gradually increased. He had been visited by a general practitioner and a skull X-ray had been performed, which did not show any fracture line. The general practitioner had prescribed an acetaminophen tablet every eight hours. After two days, despite the medication, the severity of the headache had increased, become generalized and was not responding to the analgesic. The quality of headache was pulsatile without remission. He also developed nausea and vomiting.\nOur patient had no history of seizure. He was uneducated but could cope with his daily life activities alone. He had been married for four years and had a two-year-old son. In a physical examination, macrocrania and asymmetry of his calvarium were evident, with a marked expansion of the left hemicranium. The deformity had been present for many years and our patient had been told that the bulging of his head was due to abnormal fluid accumulation; no neuroradiological investigation had been performed during his childhood.\nIn a neurological examination, his score on the Glasgow coma scale was 15 and his mental status was normal. In an ophthalmologic exam, he showed grade 2 papilledema. His pupils were normal size and the movements of his eyes were normal bilaterally. Motor and sensory exams were normal and his deep tendon reflexes were normal and symmetric bilaterally. A computerized tomography scan (CT scan) of his brain was performed, which revealed a very large extradural hematoma in the left frontotemporoparietal convexity, but surprisingly we also saw a very large cystic area over the frontoparietal convexity that extended into the left middle fossa. The overlying skull was bulged. The lesion appeared to be similar to an arachnoid cyst and was associated with the lentiform extradural hematoma, measuring 5.5 cm×7 cm, extending from the base of the middle cranial fossa to the middle part of the left parietal lobe with a total height of 6 cm. There was no evidence of a skull fracture and there was a left-to-right midline shift and the ipsilateral ventricle was compressed (Figure ). Routine laboratory tests, including a complete blood cells count, coagulative profile and serum electrolytes, were within normal limits. Since our patient showed symptoms of increased intracranial pressure we decided to proceed with surgery.\nWe performed a left frontoparietal craniotomy. No skull fracture was detected. We then evacuated the extradural hematoma, which was similar in appearance to motor oil liquid. The source of the hematoma was a tear in the middle meningeal vein, which was coagulated with a bipolar cautery. We opened the dura with a small incision and the use of a microscope. The arachnoidal cyst was identified by the marked atrophy of the underlying brain. We did not find any subdural hematoma or intracystic hemorrhage.\nWe decompressed the cyst and took a biopsy of the cyst membrane. After suspension of the dura with tack-up sutures, the wound was closed in layers. Our patient's postoperative course was excellent and uneventful. A postoperative brain CT scan of our patient showed evacuation of the hematoma and decompression of the cyst and underlying brain parenchyma (Figure ). The pathologist reported the specimen to be arachnoid membrane (Figure ). In the last follow-up visit, six month after surgery, our patient was symptom free, the papilledema was resolved, and he was working in a fast food kiosk.
A 68-year-old male presented with the right buccal abscess and oral cutaneous fistula. The obtained biopsy results confirmed buccal squamous cell carcinoma and the patient received preoperative chemotherapy (). Surgical procedures of the right modified radical neck dissection and segmental mandibulectomy were subsequently performed, resulting in three-dimensional defects involving outer skin, inner mucosa, and bony structure (). The authors harvested a fibula osteocutaneous flap based on a single septocutaneous perforator and resurfaced the full-thickness defects including bone, inner mucosa, and outer skin.\nAfter drawing a line connecting the most prominent points of the fibula head and lateral malleolus, the center of skin paddle was designed to be positioned approximately 3 cm behind the line. We tried to include as many perforator vessels as possible in the skin paddle, but there were limitations as confirmed by preoperative computed tomography (CT) and Doppler (). Through the anterior incision, the peroneal muscle was elevated from the fibula periosteum, exposed to the anterior compartment muscle, and detached from the fibula. The fibular osteotomy was performed, and the interosseous membrane was transected from the distal to proximal to the medial to lateral with the fibula traction in the lateral direction, and the tibialis posterior muscle was divided. The origin of the perforator was found and dissection was performed until the tibioperoneal trunk was reached. At this time, the posterior incision line of the skin paddle was drawn to complete the design of the skin paddle. Through the posterior incision, the soleus muscle was detached from the posterior septum and the flexor halluces longus muscle. After confirming that the perforator was septocutaneous, dissection was performed and the flap elevation was completed ().\nIn order to reconstruct the bony defect, the harvested fibula was segmented accordingly. We then folded the skin flap and used one-third of that area to reconstruct the inner oral cavity, while another third of that area was used to cover the outer skin defect, the middle portion was de-epithelialized to be folded and inset between the oral mucosa and the skin ().\nNo notable intraoperative events occurred during the surgery and the flap remained viable throughout the recovery period. The patient was discharged after 2 postoperative weeks without any significant complications. To date, 6 weeks after the surgery, the flap remains well-incorporated ().
A 33-year-old otherwise healthy female was admitted to our hospital initially for symptoms of generalized abdominal pain for 2 days, worst in the left lower quadrant and associated with diarrhea and fever. There was tenderness on palpation associated with rebound but without guarding. Vaginal and rectal examinations were unremarkable, without any tenderness or discharge.\nShe was hypotensive with a blood pressure of 84/51 mmHg and tachycardic with a heart rate of 100. She was successfully resuscitated in the emergency department. Her white blood count (WBC) was raised at 15 × 109 and C reactive protein (CRP) at 54 mg/L. A computed tomography (CT) scan revealed a left partially rim enhancing structure 2 × 2 cm suggestive of a ruptured corpus luteal cyst (). There was otherwise minimal free fluid in the pelvis and no evidence of other intra-abdominal pathology. Clinically, there were no other possible sources of sepsis found to account for the presenting symptoms and signs. There were no respiratory or urinary symptoms, and the urine pregnancy test was negative.\nOur patient was married with children and sexually active. Her last menstrual period was 16 days prior to this presentation, with no history of tampon or intrauterine contraceptive device (IUCD) use. She had a significant history of previous cervical dysplasia (CIN3) and had undergone treatment 5 years prior, with annual PAP smears being negative for recurrence.\nShe was then admitted under gynecology for further evaluation and treated with oral ciprofloxacin on account of the diarrhea with the primary diagnosis of gastroenteritis. She was discharged after 2 days with no further deterioration in her symptoms.\nShe was subsequently readmitted again 2 days later with recurring symptoms of similar abdominal pain that progressively worsened. She was febrile with a temperature of 38.4 °C and tachycardic with a heart rate of 133. There were no other symptoms of respiratory or urinary infection. On examination, her abdomen was guarding and exquisitely tender at all quadrants with features of generalized peritonism. No other localizing sources of infection were found on examination.\nHer WBC was 22 × 109/L and hemoglobin (Hb) was stable at 12.8 g/dL. CRP was 108 mg/L. Liver enzymes and amylase were within normal limits. An intra-abdominal source of sepsis was suspected. A repeat CT scan was performed, which showed extensive free fluid in the abdomen suspicious for either hemoperitoneum or exudative ascites with the persistent left adnexal lesion noted from the previous study (). No other intra-abdominal pathology was discovered on the scan with a normal appendix noted on both CT scans. In view of clinical deterioration and CT scan findings, a diagnostic laparoscopy was performed by the gynecologist on call to rule out possible ovarian cyst bleeding or ongoing intra-abdominal sepsis. Intraoperatively, we found generalized profuse seropurulent fluid. The uterus and ovaries were examined and showed no signs to suggest pelvic inflammatory disease (PID) or a bleeding cyst. On the table, a general surgery (GS) consult was made and the operation was taken over by the general surgeon. A thorough diagnostic laparoscopy was performed and the liver, spleen and rest of the alimentary tract (stomach, small and large bowels) were inspected to ensure no causative pathology. A normal appendix was identified and unlikely the cause of peritonitis, hence it was not removed. The bowels were coated with fibrin but with no sign of inflammation or perforation. In light of the unknown infection source with purulent peritonitis, abdominal drains were inserted, and the patient was continued on intravenous (IV) antibiotics in the ward.\nFluid cultures returned positive for group A Streptococcus sensitive to penicillin. Blood and urine cultures were otherwise negative. There was no history of upper respiratory tract infection (cough, sore throat and running nose) or signs of genitourinary sepsis prior to this event. A HIV screen was also done and found to be negative. A diagnosis of primary streptococcal peritonitis was made. She was treated with IV penicillin for 5 days and subsequently swapped to oral clindamycin 450 mg 3 times a day for a total duration of 2 weeks. A transthoracic echocardiogram did not show any sign of infective endocarditis. Patient showed improvement both clinically and biochemically during her stay. Drains were sequentially removed based on drain outputs with no bile or feculent discharge noted.\nPatient was discharged after 10 days of treatment in the hospital and continued with oral clindamycin upon discharge as per culture sensitivities. She was subsequently reviewed by the infectious disease team in clinic in view of this atypical presentation and repeat blood tests showed the total whites and CRP had normalized. She did complain again of some lower abdominal pain which spontaneously resolved with conservative symptomatic management.
A 15-year-old boy was referred to our unit with severe leg pain on a background of 1 week of claudication-type symptoms. On arrival, he had a dusky left lower leg with reduced sensation, reduced movements, and a capillary refill time of 7 s. Examination of the foot demonstrated an absence of the dorsalis pedis and posterior tibial pulses. Medical history included meningococcal septicemia at 2 years old with no long-term sequelae. There was no family history of vascular disease. Blood tests revealed a normal prothrombin time of 14 s and a prolonged thrombin time of 194 s. A CT angiogram demonstrated complete occlusion of the left popliteal artery with additional thrombus within the profunda femoris artery (). The apparent cause for this was a mixed density lesion arising from the distal femur, compressing the femoral vessels. As thrombus was seen in the profunda without an obvious cause (the tumor was in the distal femur), a diagnosis of deep venous thrombosis (DVT) and paradoxical embolus was considered. An echocardiogram excluded a patent foramen ovale and subsequent imaging excluded a DVT. The exact cause of the post-traumatic femoral (PFA) thrombus was never ascertained, but the suspicion was the whole SFA thrombosed initially with thrombus spilling into the PFA, then later embolized distally away from the PFA origin.\nInitial concern was that this lesion represented a malignant tumor with both osteosarcoma and chondrosarcoma within a previous osteochondroma considered. It was also considered that this could simply represent an osteochondroma with a vascular complication. Given the acute presentation, the patient was taken immediately to theater for attempted decompression of the tumor and popliteal vessels. Due to the concerns that this lesion may have represented a malignant tumor, an open biopsy was performed through a medial approach. Copious hematoma was evident which was compressing the popliteal vessels.\nFollowing decompression, the wound was irrigated and closed and attention transitioned to the thrombectomy through the common femoral artery. Initial femoral thrombectomy was successful in retrieving a large volume of fresh thrombus. Despite this, an on-table angiogram demonstrated little flow distal to the tumor and so a repeat thrombectomy was performed. Further, angiography again demonstrated poor distal flow with the vessel, this time appearing to empty into a false aneurysm (). At this point, it was decided that further thrombectomy would be futile, so a popliteal exclusion bypass was performed using ipsilateral reversed long saphenous vein to good effect. Given the ischemic time, prophylactic four-compartment fasciotomies were performed and the patient was transferred to the pediatric intensive care unit on an unfractionated heparin infusion.\nIntraoperative frozen sections demonstrated a predominantly fibrinous tumor with no bony or cartilaginous matrix. This was later confirmed to be an osteochondroma. Post-operative radiographs and a magnetic resonance imaging demonstrated a posteromedial cortical spur without evidence of a clear chondral cap which was thought to have been removed intraoperatively ().\nUnfortunately, the patient returned to theater on the first post-operative night due to hemorrhage from a slipped ligature on the graft managed with a single suture. The patient was converted to warfarin on post-operative day 11 and subsequently transitioned to clopidogrel following 3 months of anticoagulant therapy. He was discharged on post-operative day 18 having made significant progress with the physiotherapists. At his 5 month follow-up, the patient remained free from claudication and demonstrates good flow on duplex ultrasonography follow-up at 1 year.
A 27-year-old female visited our emergency room (ER) with intermittent abdominal pain and nausea. Her last bowel movement was 2 days before visiting. She had no notable past medical history, including no abdominal operation or growth abnormalities. On her physical examination, she was tachycardic, with a pulse of 136 bpm, but the rest of her vital signs were normal. Abdominal examination revealed distension and epigastric tenderness with peritoneal irritation symptoms. Her laboratory examination results showed slightly elevated C-reactive protein levels (1.8 mg/dL), but the other findings were within normal ranges. Enhanced computed tomography (CT) showed enlargement at the level of the ascending and transverse colon (Fig. ). The transverse colon had an obstructive point with a whirlpool sign. The colonic wall showed no ischemic signs, but the supply vein was dilated. The large intestine was located on the left side of the abdominal cavity, and the small intestine occupied the other side. The duodenum did not cross between the superior mesenteric artery (SMA) and the abdominal aorta but went down straight on the right side of the SMA. The SMA and the superior mesenteric vein (SMV) were located at inverted positions; the SMA was on the right side, and the SMV was on the left. The patient was diagnosed with transverse colonic volvulus, and nonrotation of the intestine was suspected.\nA transanal ileus tube was placed endoscopically at the dilated colon to decompress the enlargement. The mucosa of the colon was congestive, but there were no necrotic findings (Fig. ). She was treated conservatively and observed for the next 24 h. However, her symptoms remained, and CT revealed no improvement in the intestinal obstruction and showed increasing ascites. Conservative therapy was determined to be ineffective; therefore, emergency surgery was performed. The laparoscopic view revealed the enlarged transverse colon, and there was no space for the surgical procedure. Consequently, the operation was converted to laparotomy with a middle abdominal incision. The dilated transverse colon was pulled out from the abdominal cavity without any mobilization, and it was rotated with 180° clockwise along a bridging adhesion within the transverse colon itself. The dilated transverse colon was resected (Fig. ) and reconstructed with functional end-to-end anastomosis (FEEA). The surgical observation also showed that the mesenteries of the ascending colon to the transverse colon had mobility without fixation to the retroperitoneum. There was no ligament formation requiring Ladd’s procedure. The surgical diagnosis was transverse colonic volvulus, nonrotation type, due to the anomaly of bowel rotation. She was discharged uneventfully 12 days after the surgery.\nSix years after the first surgery, the patient present to the ER with abdominal fullness and lower abdominal pain. On physical examination, her vital signs were normal. Abdominal examination revealed distension and light lower abdominal tenderness without peritoneal irritation. Her laboratory data were within the normal range. Enhanced CT revealed the dilated colon with an obstruction at the ascending colon (Fig. ). Although a focal beak sign and narrowing of the vein were noted, there was no sign of ischemia. The appendix was located on the left upper side of the abdominal cavity, unlike in the previous surgery. Clinical diagnosis was volvulus of the cecum and intestinal nonrotation, and emergency laparotomy was performed. In the surgery, the tract from the dilated cecum to the remaining transverse colon was extracted from the abdominal cavity without any resistance (Fig. ). There was torsion of the terminal ileum to the remaining transverse colon with a 180° clockwise rotation. The dilated and twisted tract was removed, and ileocolic anastomosis with FEEA was performed. The anastomotic site of the previous surgery was adhered and fixed to surrounding tissue. She was discharged uneventfully on postoperative day 9. Although the patient was diagnosed with obstruction of the intestine caused by an operative adhesion 7 months after the surgery, conservative treatment relieved her symptoms in 3 days of hospitalization.
A 24-year-old healthy woman presented with difficulty breathing and dissatisfaction with her facial appearance. She had a history of childhood trauma resulting in nasal septum deviation and external nasal deformity. Four months after a successful and uneventful septorhinoplasty, she presented to the emergency department with blunt nasal trauma resulting in a septal hematoma, which was drained successfully; the patient was discharged with no adverse sequelae.\nFour months later, the patient sustained nasal trauma again, this time accompanied by clear nasal discharge, raising suspicion of cerebrospinal fluid (CSF) leak. The patient was discharged after managing the nasal injury, as the CT brain showed an intact cribriform plate with no evidence of a CSF leak. Ten days later, she presented at the emergency department with dizziness and an unstable gait. She also had complaints of paresthesia for the past two months, beginning in her right hand and progressing to the right shoulder, arm and leg, associated with some difficulty in the execution of movements in the first and second finger of the right hand. Her right leg was quite stiff with difficulty in walking. On close inquiry, she gave history of pain in the right eye and double vision many months back, which had resolved spontaneously. Examination showed a positive Romberg’s and Lhermitte’s sign, with right-sided sensory impairment.\nMagnetic resonance imaging (MRI) of the brain, cervical and thoracic spine demonstrated demyelinating lesions in the brain and cervical segment of the spinal cord (Figure ). Some of the lesions demonstrated enhancement on post gadolinium administration sequences, suggestive of active demyelinating diseases like MS. A lumbar puncture was performed which demonstrated the presence of oligoclonal bands in the CSF. The diagnosis of MS was confirmed by a neurologist and treatment was initiated.\nThe initial neurological symptoms have largely vanished with only persistent light paresthesia in the right hand. Two years later she has had no new symptoms and continues with the same medication with good tolerance.
A 22-year-old male presented with pinkish looking pit lateral to the left ala of nose with history of repeated purulent discharge with intermittent conjunctivitis since birth []. History of intermittent mild pain and tenderness in the left cheek improved with antibiotics. There was no history of excessive lacrimation from left eye. On clinical examination, a pinkish opening about 5 mm in diameter was seen inferolateral to the base of left ala, and on palpation there was no bony indentation or notching on the nasolacrimal and infraorbital region. However on moderate pressure over the lateral wall of nose there was increased discharge from the opening. The patient otherwise had a normally developed face with no evidence of any cleft. Injecting saline into this opening showed exit of saline from the punctum of inferior canaliculus confirming the nasolacrimal duct []. All the routine investigations were within normal limits. CT scan or MRI was not done because of resource constrains and the diagnosis was confirmed by injecting saline into the duct.\nThe patient was operated under general anesthesia. We cannulated the lower end of the nasolacrimal duct with no. 5 infant feeding tube. The alar facial groove was marked with methylene blue and infiltrated with 1:20000 adrenaline solution. A circular incision was placed around the swelling which was extended superiorly in the alar-facial groove taking care not to injure the nasolacrimal duct with the feeding tube in place as a guide The lower third of the duct was dissected circumferentially []. The lower part of the incision was deepened till the mucosa lining the lateral wall of nose so as to reach the inferior meatus. The distal opening of the nasolacrimal duct was cut 2 mm on the medial side and sutured with the opening in the nasal mucosa with 4.0 catgut. The incision was closed with 6.0 Prolene []. Patient was on intravenous ceftriaxone 1 g IV followed by oral cefixime for five days, Gentamicin eye drop for two weeks. The stent and the sutures were removed after five days. Patient was asymptomatic three months later when followed up.
A 63-year-old Caucasian woman complaining of insidious upper abdominal pain (unrelated to food ingestion) and nausea was admitted after an episode of intense acute abdominal pain. She denied having hematemesis or melena and had a history of untreated dyslipidemia. Her dietary habits included acid fruits, soft drinks, beer, chocolate, and high-fat foods. She was a smoker of 10 or more cigarettes per day and had no family history of cancer. She had systemic arterial hypertension that was being treated with 500mg of methyldopa per day. The results of a physical examination were normal except for a palpable and movable upper abdominal mass. The results of all laboratory tests were normal, and our patient did not have anemia. An abdominal ultrasound was promptly performed, and a large echoic mass compatible with an expansive lesion was found in the gastric antrum. Conventional endoscopy detected a large bulging mass in the posterior gastric wall and three ulcerated areas in the gastric mucosa, but none of them showed signs of recent bleeding (Forrest III). Owing to technical limitations, the procedure did not result in an adequate biopsy of the mass in the submucosa. The fragments acquired from the ulcers revealed nothing but necrotic mucosa. Abdominal contrasted computed tomography revealed a well-defined homogeneous oval mass that was located within the posterior gastric wall and that compressed the second portion of the duodenum. The lesion had fat tissue density and a suspicious central contrast enhancement with trabecular architecture but was noninvasive with respect to the peripheral layers (Figure ).\nFor this reason, as well as the tomographic dimensions (size of 10 × 6 × 11cm and volume of 660cm3), malignancy was suspected. Our patient then underwent an open laparotomy for abdominal exploration. During surgery, unexpected benign aspects of the mass, such as the envelopment by a fibrous capsule and no signs of lymphadenopathy or local metastasis, surprised the team (Figure ). Multiple septated cysts in the right ovary and multiple simple cysts in the left ovary were also found, and although the freezing biopsy failed to demonstrate tumor cells, a bilateral oophorectomy was done. In regard to the gastric mass, subtotal gastrectomy and D1 lymphadenectomy with a Roux-en-Y reconstruction were performed. The post-operative period was uneventful, and our patient was discharged by the seventh day.\nMacroscopic findings included a well-defined homogeneous yellowish lesion that was located in the submucosa and that comprised the entire gastric posterior wall and three ulcerated zones of 0.5, 1.0, and 1.4cm in diameter (Figures and ). Despite the dimension indicated by computed tomography, an actual size of 12 × 8 × 6cm (576cm3) was measured. Microscopy revealed a histological pattern of well-differentiated noninvasive mature adipose tissue surrounded by a fibrous capsule (Figures and ). The findings confirmed the benign intra-operative aspect of the lesion and the diagnosis of gastric submucosal lipoma. The greater omentum was free of tumor cells.
Towards the end of the year 2017 a then 25-year-old, 0-gravida of Asian origin first came to our outpatient ward. She had an MRI scan in her home country, in which a complex cyst in the left ovary had been detected, suggesting an endometriotic origin. She wished to have a follow-up on the cyst. But the findings in the MRI could not be reproduced by ultrasound. Four months later, however, in the subsequent follow-up ultrasound scan, both of the ovaries as well as the rectovaginal septum showed newly developed, possible endometriotic lesions ().\nIn consequence of these findings, a hormonal treatment with norethisterone acetate was initiated. In the following months, she submitted herself twice to the emergency ward of a different clinic because of severe pain in her lower abdomen. Both times no correlating pathology, other than the already suspected endometriosis could be found which could have explained the severe symptoms.\nBetween two subsequent scans in July and November 2018, made in the process of regular follow-up at our clinic, and under continuation of the prescribed hormonal treatment, a large, partially septated, endometriotic cyst had developed in the right ovary measuring a maximum diameter of 8 cm, showing a content of ground glass echogenicity as well as signs of fresh hemorrhages ( and ). There had also been an increase in the size of the left ovary, with multiple cystic findings with ground glass echogenic content (), one of which had a possible papillary structure lining the cyst wall ().\nOnly two days after her last regular check-up in November 2018, the patient presented herself in our clinic with acute abdominal pain. The ultrasound scan suggested a ruptured endometrioma of the right ovary, which was confirmed during emergency laparoscopic surgery. Intraoperative findings additionally revealed concomitant endometriosis on the cardinal ligaments as well as on the uterosacral ligaments and adhesions of both ovaries to the pelvic wall (rASRM IV°, ENZIAN B3). Besides being adherent to the left pelvic wall, the left ovary appeared to be enlarged and hard upon palpation. During enucleation of the endometriotic cyst on the right side (), unexpected papillary structures were found lining the bottom of the cyst (). Definitive histological examination revealed an endometrioid borderline ovarian tumor, pT1c2 (rupture of the cyst preoperatively), without stromal invasion ().\nBecause of the intraoperative appearance of the left ovary, an ultrasound follow-up after 4 weeks was concluded, prior to planning the staging operation, in case the left ovary needed surgical intervention as well. A fact that would have to be considered in the treatment planning. Unfortunately, after this period of 4 weeks, sonographic imaging showed further enlargement of the left ovary itself as well as the cystic lesions, along with a more prominently appearing central solid part, all in all suspicious of contralateral borderline malignancy entailing further surgical investigation ().\nThus, suspecting bilateral involvement of the ovaries in this now 26-year-old patient, the decision was made to complete the staging operation by laparoscopic reevaluation of the right ovary (), cystectomy of the left ovary for histological diagnosis ( and ) as well as peritoneal washing cytology, omentectomy (), peritoneal biopsies and endometrial sampling. Postoperative pathological examination revealed a borderline ovarian tumor of the left ovary as well, however of sero-mucinous differentiation, pT1c1. Omentum and peritoneal samplings showed no implants or invasive implants and endometrial sampling was unsuspicious for endometrial pathology. Peritoneal cytology showed very few slightly atypical cell groups with no further classification possible and no high grade atypia.\nIn conclusion, considering the bilateral involvement of the ovaries and the young age of the patient, the interdisciplinary tumor board recommended an ultraconservative, fertility sparing management with preservation of both ovaries and an aftercare including regular sonographic assessments of the ovaries. This treatment plan was discussed with the patient and she was informed of the higher risk of recurrence as a consequence of this management.\nAfter 18 months of follow-up, under continued hormonal treatment of the endometriosis with Dienogest, the patient remains recurrence-free and also asymptomatic regarding the endometriosis. Taking into account the higher risk of recurrence and the young age of the patient, quick pursuit of family planning or, alternatively, timely oocyte freezing was recommended to the patient.
The patient was a 45-year-old man with a chief complaint of discomfort in the maxillary right central and lateral incisors for one year. Mucosal swelling and spontaneous pain were seen at the labial gingiva of the maxillary right lateral incisor, and tooth mobility started at three months before the initial examination (). Oral examination revealed the mobility of the maxillary right lateral incisor as grade 2 and that of the central incisor as grade 1. In addition, the patient had both spontaneous and occlusal pain. Intraoral periapical radiographs showed radiolucency at the apical regions of the central and lateral incisors, although no dental caries were detected in these incisors (). Both teeth had periodontal pockets around 1–2 mm circumferentially in depth, and there was no bleeding on probing sites. Furthermore, the cold thermal examination revealed normal responses of the vital dental pulp at both incisors. Panoramic radiography showed numerous dental caries and apical lesions in the oral cavity (). The closest lesion to the site of the chief complaint was detected at the maxillary right first premolar, which was previously treated by root canal treatment. A connecting bridge was attached, linking from the first premolar to the first molar, and there was no occlusal pain on percussion. The adjacent maxillary right canine was intact without caries or periodontal disease. Intraoral periapical radiography revealed an apical lesion in the maxillary right first premolar (). However, the apical lesion appeared small on intraoral periapical radiographs, there was no swelling of the mucosa around the root, and the patient had no symptoms. CBCT was performed using Trophypan Smart Osiris 3D (Carestream Dental, Atlanta, GA) to obtain three-dimensional information. The CBCT images revealed marked apical radiolucency and bone resorption around the lateral incisor. We also found apical radiolucency with destruction of the cortical bone on the buccal side, measuring ≥12 mm in diameter on the lateral incisor, and an apical radiolucency measuring ≥8 mm in diameter on the central incisor (Figures –). Root canal filling material was found on the buccal root of the maxillary right first premolar, and no root canal filling agents were seen in the palatal root, with an apical lesion measuring more than 10 mm in diameter on the palatal side (). Furthermore, the apical lesion had spread extensively from the palatal apical lesion and reached the apical areas of the central and lateral incisors that were the sites of the chief complaint (). Based on these results, the clinical diagnostic was that the apical radiolucency of the chief complaint was derived from the apical lesion of the palatal root at the maxillary right first premolar. Therefore, we planned to retreat the root canal of the first premolar, which was considered as the causative tooth. To perform endodontic caries treatment, the connective bridge on the maxillary right molars was cut between the first and second premolars to remove the crown on the first premolar alone. Subsequently, we used a rubber dam and removed the gutta percha in the buccal roots using ProTaper Retreatment files D1 and D2 (Dentsply Sirona Endodontics, Ballaigues, Switzerland) and a #10 K-file (Dentsply Sirona Endodontics) to locate and measure the working length of the buccal and palatal root canals with an electronic apex locator (Root ZX; J. Morita Mfg. Corp., Kyoto, Japan). WaveOne Gold (Dentsply Sirona Endodontics) was then used to expand the root canal to have a large tip size. We used 6% sodium hypochlorite for all intermediary procedures. After root canal enlargement, we used 17% ethylenediaminetetraacetic acid (EDTA) for 1 min and ultrasound with an EndoActivator (Dentsply Sirona Endodontics) to irrigate the root canal wall dentin. The fluid inside the root canal was then aspirated and dried with a paper point, and calcium hydroxide (Fujifilm Wako Pure Chemical Corporation, Osaka, Japan) mixed with sterilized water was applied and given a temporary hydraulic seal with Caviton EX (GC Corp., Tokyo, Japan). On the second visit (four weeks later), root canal filling was performed using a resin sealer (AH Plus; Dentsply Sirona Endodontics) with the core carrier method (Gutta Core, Dentsply Sirona Endodontics). Abutment was prepared using a flowable resin composite, and a temporary crown was placed (). This treatment led to the disappearance of swelling at the maxillary right central and lateral incisors approximately two weeks postoperatively, and intraoral periapical radiographs showed a tendency of disappearance of the apical radiolucency approximately six months postoperatively (). After three years, intraoral periapical radiographs and CBCT images showed that apical radiolucency almost disappeared around the maxillary first premolar and incisors (Figures , , , , and ). The mobility of the central and lateral incisors improved to grade 0, and the dental pulp was preserved, leaving vital teeth with no symptoms ().\nThe apical radiolucencies of the maxillary right central and lateral incisors, the sites of the chief complaint in this patient, were large and required approximately three years until clear osteoanagenesis was achieved. CBCT shows bone destruction around the lateral incisor, also visible on the periapical radiography, and there is also a hypodense image between the roots of the premolar (, , and ), requiring a longer follow-up. Swelling of the mucosa diminished in approximately two weeks, but it took approximately one-and-a-half years for the patient's discomfort to completely disappear.
We present the case of a 30-year-old Nigerian male who was brought to the Surgical Emergency Department of the Lagos State University Teaching Hospital Ikeja 22 hours after he had inserted a constricting ring over his penis. He had developed a painful penile shaft swelling distal to the ring with suprapubic pain and swelling secondary to acute urinary retention. There was associated urethral bleeding.\nThere had been failed attempts at removing the ring by self and the resulting severe pain drew the attention of his relatives who brought him to the emergency room.\nHe had a history of a psychiatric illness and the patient claimed he was under a spell and had heard a voice that instructed him to insert a ring over his penis. He denied using the ring to sustain erection and claimed it was his first time of inserting a ring over his penis.\nThe patient had a history of deterioration in personal and general performance with underachievement dating back to 7 years prior to presentation when he voluntarily dropped out of the university and had done nothing tangible thereafter.\nTwo weeks prior to presentation, the patient's relatives had noticed some unusual behavior in him characterized by talking to self and rubbing salt over his body and the patient claimed he was being chased by unseen people.\nHe had a history of alcohol, cigarette, and cannabis abuse for about 15 years.\nOn examination, he was in acute urinary retention with a tender suprapubic distention up to the level of the umbilicus.\nThere was a thick constricting ring at the root of his penis. There was a markedly swollen oedematous penis distal to the ring with marked reduction in sensation over the penis and glans ().\nWe made a diagnosis of Constrictive Penile Injury (Bhat Grade III) with acute urinary retention [].\nHe had a suprapubic cystostomy done to relieve the acute urinary retention as a urethral catheterization was impossible.\nAttempts were made to remove the constricting ring by the use of aspiration, application of cold compress, and lubrication initially and later by the use of the string method.\nFollowing failed attempts at removing the device with these different manipulations and unsuccessful attempt at cutting with the manual saws available in the hospital coupled with the fact that the patient appeared to have imminent penile gangrene, a decision was made to call the fire service for a power driven saw.\nThe ring was successfully removed by cutting it at two different points () with a power driven arc saw () under conscious sedation at the emergency room.\nThermal injury was prevented by intermittent cooling with ice packs and injury to underlying tissue was prevented by insinuating a pair of artery forceps between the penis and the ring ().\nDressing of the resulting penile skin ulceration was done and the plastic surgery team was invited for possible additional wound care.\nThe patient was also reviewed by the psychiatric team who made a diagnosis of schizophrenia and commenced the patient on haloperidol. He was to be followed up on an outpatient basis in the psychiatry clinic.\nThe patient reported normal nocturnal erections while on admission. Further evaluation of the suspected urethral injury with urethrogram and a urethroscopy was planned but this was declined by the patient who opted to retain his suprapubic catheter.\nThe patient also declined any additional wound care by the plastic surgery team and the wound was healing satisfactorily by secondary intention as at 2 weeks after the initial presentation ().\nHe subsequently defaulted from care.
A 70-year-old female patient presented to us with complaints of being non-ambulatory since the past 10 days. She gave a history of difficulty in walking for the past 10–15 years associated with pain in the right hip which did not respond to analgesics and physiotherapy. She also complained of mild pain in the left hip and both the knees. She did not have any history of trauma or fall. There was no other medical or surgical illness. Family history was insignificant. On examination, the right hip had a 2 cm shortening with the joint movements being painful and restricted. A flexion deformity of 10° was noted in the same hip. In addition, the right hip had a restricted abduction and adduction compared to the left hip. There was also a bilateral knee flexion and varus deformity of 10 degrees each. Clinically, we provisionally diagnosed the case as arthritis of the hip joint under evaluation.\nThe radiological assessment revealed severe joint destruction of the right hip with a fracture at the neck of femur. Femur head demonstrated irregularity and significantly narrowed hip joint space ().\nThe left hip joint also showed narrowing of joint space. Both the knees revealed severe tricompartmental osteoarthritis (OA) (). The X-ray of the spine demonstrated fusion of vertebral bodies resembling a bamboo spine appearance ().\nOn magnetic resonance imaging, significant bony destruction of the right femoral head-and-neck regions with superior subluxation of the right greater trochanter and femur was seen (). Inflammatory marrow changes in the residual upper femur raised the possibility of an associated osteomyelitis. Inflammatory or hemorrhagic fluid collections were seen in the acetabular fossa. The sacroiliac joint appeared normal.\nThe erythrocyte sedimentation rate (ESR) and C-reactive protein were within normal limits. Rheumatoid factor and HLA-B27 were negative as well.\nUnder local anesthesia and aseptic precautions, aspiration of the involved hip was done in the operating room and joint fluid sent for culture sensitivity and GeneXpert. The aspirate did not show any growth on culture. The results of the GeneXpert turned out to be negative as well.\nA total hip arthroplasty was planned. Intraoperatively, on lifting the short external rotators, we found the joint capsule to be black. The capsule had lost its natural consistency and had turned hard and contracted. Moreover, the joint surfaces, ligaments, and tendons were blackish too with pieces of black cartilage. The capsule was excised posteriorly and the femur head was removed. Reaming of the acetabulum revealed poor bone quality. An uncemented acetabular cup was used (44 mm) with a ceramic liner (, ).\nAfter the reaming and broaching of the femoral canal, an uncemented femur stem (Smith and Nephew, cone 12/14, size 1) was inserted and the hip joint was reduced. There were no intraoperative or post-operative complications. The bone and soft-tissue specimens were sent for histopathological evaluation. The patient was mobilized full weight-bearing on the 1st post-operative day and was discharged on the 3rd day.\nPostoperatively, reexamination of the patient revealed brownish-black pigmentation of the ear pinna as well as the sclera (). The urine of the patient was also tested and it turned dark on standing for 24 h ().\nOn histopathological examination, the articular cartilage showed brownish-black pigmentation throughout, sparing only the part adjacent to the underlying bone. Soft-tissue demonstrated brownish-black fragments of pigmentation which were sharply defined and irregularly shaped. The surrounding tissue revealed fragments of non-viable bone, inflamed synovial tissue, foreign body type giant cells, and fibroblastic proliferation with few hyalinized areas. The synovial lining was denuded. All of the features mentioned above are consistent with the diagnostic findings of ochronosis ().\nThe follow-up of the patient at 1 year revealed that her right hip pain had completely subsided along with a full range of motion. The Harris Hip Score of the patient had shown significant improvement from 32 preoperatively to 81 at 1 year follow-up. The patient was also counseled for a bilateral total knee replacement.
A 58-year-old female presented to the emergency department with intermittent, crampy right-sided abdominal pain, nausea, and vomiting, which began approximately 18 hours previously. Her past medical history was significant for hypertension and her surgical history included a thyroidectomy for treatment of thyroid cancer and a Caesarean section. A contrast enhanced CT abdomen and pelvis was obtained, demonstrating multiple fluid-filled, dilated small bowel loops in the right abdomen, which were predominantly anterolateral to the ascending colon and cecum (). In addition, two transition points were identified in the right lower quadrant, with one transition point at the distal ileum just proximal to the cecum and a second transition point in the proximal ileum. The two transition points were in close proximity to each other, indicative of closed loop obstruction. Decreased wall enhancement of the dilated small bowel loops was concerning for ischemia. Mucosal hyperenhancement of the ileum at the proximal transition point was felt to relate to ischemia or decompressed state (). Given the patient's symptoms and findings of closed loop obstruction on CT, the patient was taken to operating room. In the operating room, an internal hernia with closed loop obstruction was confirmed and resulted from herniation of small bowel through an adhesion of a transverse colon epiploic appendage to the ascending colon mesentery. The herniated small bowel was nonviable and a total of 60 cm of small bowel was resected (). Retrospectively, kinking of the ascending and transverse colon could be seen on the initial abdominal CT and was felt to correspond with the site of adhesion ().\nAfter resection, the patient's small bowel was left in discontinuity and an abdominal wound-vac was placed. The following day, the patient returned to the operating room, at which time the terminal ileum was also found to be nonviable. An ileocecectomy with enterocolonic anastomosis was performed.\nThe patient had a complicated postoperative course, but was ultimately discharged approximately two weeks after the initial surgery.
A 66-year-old female presented to our head and neck clinic with a 2.5 cm left floor of mouth, biopsy-proven squamous cell carcinoma. The treatment recommendation was for primary surgery consisting of floor of mouth excision, bilateral selective neck dissections and radial forearm free flap (RFFF) reconstruction.\nHer past history was significant for a colonic perforation approximately 2 years prior to her presentation to our clinic. At that time she required a laparotomy and colostomy followed by an extended intensive care unit stay. She was right-hand dominant and denied any trauma or surgery to the left forearm or hand. She was a 50-pack year smoker and a heavy alcohol drinker. Pre-operatively she was assessed for RFFF harvest with a standard Allen’s test and pulse oximetry plythesmography, which were both normal. She had a palpable radial pulse and no visible evidence of trauma at the donor harvest site.\nShe was taken to the operating room and underwent resection of the primary site. A RFFF measuring 5 × 4 centimeters was designed overlying the radial artery with the distal aspect of the skin paddle placed approximately 3 cm proximal to the left flexor wrist crease. The flap was raised in standard fashion. During the flap elevation, significant fibrosis was encountered at the distal aspect of the pedicle as well as along the flexor retinaculum underlying the pedicle.\nAfter the tourniquet was released, the skin paddle of the flap was not perfused. Assessment of the radial artery with pencil Doppler confirmed a pulse in the proximal artery but this was lost at the proximal end of the skin paddle. No pulse was obtainable within the borders of the flap. The distal artery clip was removed and there was no flow-through observed. A Fogarty catheter was then inserted from the distal end of the radial artery, however there was significant resistance towards the proximal end of the flap suggesting stenosis of the vessel. The catheter was passed proximal to the stenosis and on inflation and withdrawal of the catheter flow through the flap was able to be re-established however this was not sustained. A decision was made at this time to abort the RFFF. A left anterolateral thigh flap was raised without complication and used as reconstruction.\nWe hypothesized that during the prior surgery and ICU admission for management of her colonic perforation she had a left radial artery intra-arterial catheter placed for hemodynamic monitoring. This likely resulted in occlusion of the left radial artery, which was not appreciated on pre-operative assessment. Interestingly the location and length of radial artery occlusion in our case corresponds to the length of a standard intra-arterial catheter from its usual insertion site at the flexor wrist crease, as illustrated in Fig. .\nFollowing the first case we encountered a 69 year-old patient with an oral tongue cancer that required free-tissue reconstruction. He had a history of multiple medical comorbidities including diabetes, chronic renal failure and peripheral vascular disease, in addition to a history of prior surgeries. He had an arteriovenous fistula in his right arm and a left leg below-the-knee amputation. The Allen’s test on the left hand was normal. We then assessed the patency of the radial artery by performing the Allen’s test in reverse. Just as the radial artery occlusion is maintained with digital pressure in a standard Allen’s test to assess the ulnar artery supply to the arches, palmar inflow through the radial artery is assessed in the ‘reverse’ Allen’s test by maintaining ulnar artery compression. With normal radial artery patency the tester can expect rapid return of color to the hands and fingers upon release of the radial artery as illustrated in Fig. . In this case there was no evidence of hand re-perfusion upon release of the radial artery. While maintaining ulnar compression the radial artery was assessed with the hand-held Doppler, which demonstrated that there was no Doppler signal of the radial artery at the flexor crease, however a Doppler signal was present more proximal on the artery, approximately 5 to 6 cm from the flexor crease. We therefore designed our flap in a more proximal location, an example of which is demonstrated in Fig. .\nFlap harvest in this way was successful with a well-perfused flap transplanted to the hemi-glossectomy defect. The long pedicle length of the RFFF is advantageous in this situation, whereby shortening the pedicle length by moving the donor harvest site more proximal on the arm still permitted arterial and venous anastomoses to be performed in the neck without the need for vein grafts or deferring to an alternate donor site.\nThe RFFF has established itself as a workhorse reconstructive option for a multitude of head and neck defects providing thin, pliable tissue supplied by a long, large caliber vascular pedicle. Furthermore its attractiveness is enhanced by it being a reliable donor site with flap failure rates reported to be less than 3 % and an anatomical location that permits two-team harvest [–]. There are, however, potential morbidities associated with this flap harvest []. These may include a cosmetically displeasing donor site closure, skin graft loss with subsequent flexor tendon exposure along with alterations in range of movement, strength and sensation in the donor hand and forearm []. Despite their rarity, the most feared complications of RFFF harvest are the ischemic hand complications [].\nThe vascular supply of the hand is derived from the superficial and deep palmar arches, which receive their arterial inflow from the radial and ulnar arteries. The ulnar artery is usually the dominant contributor to the superficial arch, anastomosing with the superficial branch of the radial artery over the thenar eminence []. Palmar digital arteries run distally from this arch to supply the fingers. A complete superficial arch is present in 84–90 % of patients, with considerable variation occurring. In contrast, the radial artery predominantly supplies the deep arch. It almost invariably forms a complete arch through anastomosis with the deep branch of the ulnar artery. The palmar metacarpal arteries arise from the deep arch and anastomose with the palmar digital arteries from the superficial arch. Hence the hand is supplied by an anastomosing network of arteries arising from both superficial and deep palmar arches, which in turn are supplied from a combination of the radial and ulnar arteries. These extensive anastomotic connections usually prevent the hand from ischemic damage in the face of injury to a single component of the network.\nFollowing harvest of the radial artery during the RFFF harvest, the hand is solely perfused by the ulnar supply to the arches and the distal anastomotic connections ensure the hand and fingers remain perfused. However, inadequate flow through the ulnar artery may result in either acute or chronic hand ischemia []. The adequacy of flow through the palmar arches is routinely tested pre-operatively with the Allen’s test to pre-emptively identify patients at risk of hand ischemia after radial artery sacrifice. Other tests such as Doppler ultrasonography and pulse oximetry with plythesmography have also been employed to improve accuracy of pre-operative clinical decision-making []. This dedicated assessment of the adequacy of the arch system reflects the seriousness of the morbidity should inadequate arch circulation be overlooked prior to RFFF harvest.\nIn contrast to the attention given to the potentially devastating donor site morbidity related to inadequate ulnar inflow, little has been published on the impact of radial artery occlusive disease in the setting of attempted RFFF harvest. The very nature of the arterial arcades within the hand may mask pre-existing radial artery damage as ulnar collateral circulation through the palmar arches can produce a palpable pulse on the radial side of the wrist crease in spite of radial artery occlusion more proximally. This may predispose to intra-operative flap failure. Performing the Allen’s test in the reverse direction can ensure the adequacy of flow through the radial artery and prevent this potential complication.\nRecently there has been increasing use of invasive monitoring during anesthesia and the intensive care setting as well as a dramatic expansion of therapeutic endovascular procedures. The radial artery has become an attractive option for arterial catheterization due to its superficial location and fewer access site complications. Whilst the complications of trans radial catheterization are reported to be lower than other sites, radial artery occlusion is the most frequently encountered []. The reported incidence of radial artery occlusion is 2 to 18 % [], however it is recognized this may be an under-representation because this condition is usually asymptomatic and only detected with ultrasound or plethysmographic assessment of the radial artery or when the radial artery is re-accessed for another endovascular procedure.\nAs with ulnar artery disease, radial artery occlusion may be a silent disease given the arcade-like nature of the palmar vascular anatomy. Quite rightly, the vascular literature concludes that this condition is associated with essentially no major clinical sequelae, however it does limit the future utility of the radial artery as an arterial access site []. It is important to recognize that this also extends to its use in a free tissue transfer. It is interesting to note that the length of an intra-arterial catheter from the usual insertion site at the wrist corresponds to the area most commonly used to harvest a RFFF (Fig. ).\nAs the use of the radial artery for diagnostic and therapeutic procedures continues to expand it is behest on the reconstructive surgeon to consider this potentially silent disease in planning head and neck reconstruction. To this end, we reiterate the importance of performing a detailed assessment of both ulnar and radial artery blood flow prior to RFFF harvest by performing the Allen’s test in both directions, as already described. When the ‘reverse’ Allen’s test demonstrates a complete lack of re-perfusion of the hand and digits then a lack of flow through the radial artery should be assumed. This is in contradistinction to the situation where there is reperfusion to the thumb and index finger, which is due to an incomplete arch with lack of communication with the ulnar system. In the latter situation a hand-held Doppler can be used to trace the flow in the radial artery in order to determine candidacy for a RFFF and when flow is present proximally it can aid in designing the flap more proximally over an area of skin that will be perfused by the radial artery. Alternatively, another donor site may be chosen, particularly if the injury to the radial artery extends proximally.
A 77-year-old woman was referred for the treatment of her severely worn dentition. Her chief complaint was that she could not eat anything because her teeth were worn too much. The patient had anticoagulant and analgesic agent due to hypertension and idiopathic headache. Intraoral examination revealed a generalized loss of dental substance that was greater in the maxillary left incisors and the mandibular right incisors. Maxillary left canine and mandibular right canine were worn to the gingival level, and had got root canal treatments (). The anterior teeth had sharp enamel edges, dentinal craters, and attritional wear due to the loss of posterior support. The mandibular posterior teeth were missing, but she said that she did not use her old removable partial denture (RPD) as it was not comfortable. The mandibular RPD lost its retention and support because the regions that functioned as the rest and retentive undercut of abutment teeth had been fractured. The facial type of patient was square and her lip seemed to be under strong tension. The patient did not have temporomandibular disorder history and soreness of the mastication muscles, but the discrepancy between centric occlusion (CO) and maximum intercuspal position (MIP) was found when she was guided to CR with bimanual technique. The transcranial view was taken to determine whether a temporomandibular problem exists. The right mandibular condyle was flatter than the left one, but any specific disorder was not found ().\nTo determine whether VDO had been altered, the following aspects were investigated:,,\nLoss of posterior support: mandibular posterior teeth were missing, and the patient did not use the mandibular partial denture. Posterior collapse resulted in excessive wear and fracture of anterior teeth. History of wear: Physiologic wear can be compensated by tooth eruption in general, but the accelerated wear may exceed the rate of eruption. The patient liked vegetables and acidic fruits. Her favorite food was tough and fibrous. In addition, maxillary posterior base metal prostheses might accelerate the wear of mandibular acrylic resin teeth and unbalance of wear rate. Phonetic evaluation: If the distance between the incisal edge of the mandibular incisors and lingual surface of the maxillary incisors is about 1 mm, it makes normal /s/ sound. The patient's increased space altered /s/ sound to /∫/. Interocclusal rest space: The patient's interocclusal rest space that was measured between nose tip and chin tip was 5 - 6 mm that was greater than the normal value, 2 - 4 mm. Facial appearance: Wrinkles and drooping commissures around mouth were observed.\nThe possible causes of patient's worn dentition that might include posterior interferences, parafunction, eating habit, and dental ignorance were explained to the patient. And the options of treatment plan were restoring mandibular edentulous posterior region with implants or removable partial denture, full mouth rehabilitation with metal ceramic restoration with or without crown lengthening procedure. The patient was scared of implant surgery, so the option of implant installation was excluded. Also the patient did not want to get multiple crown lengthening procedures and endodontic treatments to restore the worn teeth in the insufficient space. As there was clinical evaluation of reduced VDO, full mouth rehabilitation with increasing VDO was planned. Only severely worn teeth which were maxillary left canine and mandibular right canine were undergone crown lengthening procedures to obtain a sufficient clinical crown length and ferrule effect.\nThe patient's casts were mounted on a semi-adjustable articulator (Hanau™ Modular Articulator; Whip Mix Corp., Louisville, USA) using a face-bow record and an interocclusal record that was made with the aid of a Lucia jig and polyvinylsiloxane occlusal registration material (EXABITE II; GC Corp., Tokyo, Japan). The new VDO was set by 5 mm increase in the incisal guidance pin of the articulator. Because the patient's interocclusal rest space was 2 - 3 mm larger on the premolar area than normal distance, the actual increase were determined 3 mm in the anterior teeth and 1 - 2 mm in the posterior teeth. The splint was designed to offer bilateral contacts of all posterior teeth in centric relation and guides of the anterior teeth in excursive movement (). The anterior guidance disoccluded the posterior teeth in all jaw position except centric relation.\nThe adaptation of patient to the increased VDO was evaluated during 1-month trial period. No muscle tenderness and temporomandibular discomfort was found. The method of increasing VDO with the splint was used to determine desirable VDO of the fixed interim prostheses. After taking CR record using Lucia jig and wax-rim, diagnostic wax-up was performed (). Autopolymerizing acrylic resin (ALIKE™; GC America, ALSIP, USA) provisional crowns were fabricated using a vacuum formed matrix (Drufolen H; Dreve Dentamid GmbH, Unna, Germany) that was produced from the diagnostic wax-up, and mandibular provisional RPD was made to fit provisional crowns (). The provisional fixed restorations were cemented with temporary cement (FREEGENOL TEMPORARY PACK; GC Corp., Tokyo, Japan), and the patient's adaptation was monitored.\nFor three months, interim restorations were adjusted, and used as a guide for the definitive oral rehabilitation. During this period, the patient's condition and functions, such as muscle tenderness, discomfort of TMJ, mastication, range of the mandibular movements, swallowing, and speech, were evaluated. Improvement in mastication, speech, and facial esthetics confirmed the patient's tolerance to the new mandibular position with the restored VDO. The anterior guidance and posterior disclusion on excursive movement were established. Adjusted occlusion was transferred to customized anterior guide table, which was made with acrylic resin (PATTERN RESIN; GC Corp, Tokyo, Japan)().\nFinal preparation was performed, and definitive impressions were made with polyvinylsiloxane impression material (Extrude; Kerr Corp., Romulus, Germany). Bite registration was taken using provisional crown and occlusal registration material (StoneBite; Dreve Dentamid GmbH, Unna, Germany) by half and half (). Porcelain fused to metal restorations were made using customized anterior guide table and cemented with resin modified glass ionomer cement (FujiCEM; GC America, Alsip, USA). Because the patient's anterior guidance table was used in the production of definitie restoration, the amount of occlusal adjustment on the lingual surface of maxillary anterior teeth was minimal. Individual tray with polyvinylsiloxane (EXAMIXFINE; GC Corp., Tokyo, Japan) was used for the impression of mandibular RPD. The impression on posterior alveolar ridge was taken once more with the individual tray which is attached to the RPD framework, and the altered cast was made. After the adaptation of RPD framework and the trial of wax denture were done, the definitive mandibular RPD was fabricated and delivered with minor occlusal adjustment (). The prostheses were designed using mutually protected occlusion. The anterior teeth protected the posterior teeth from excursive force and wear, and posterior teeth supported the bite force. Oral hygiene instruction and regular check-up were administered.
This 49-year-old man was of New Zealand-British descent. He presented to cardiac services at 36 years of age with palpitations and exertional chest pain. For approximately 2 years prior he had episodes of calf pain, with increasing shortness of breath on exertion and lethargy. He had previously been very active. He had normal intellect, no history of recorded hypoglycaemia or symptoms to suggest this, a normal birth and childhood and no other medical problems. His mother and father died in their 70s of a cerebrovascular accident and bowel cancer respectively. He has an older brother and sister and two sons, all of whom are well. His echocardiogram at presentation revealed severe cardiac hypertrophy with speckling of the cardiac muscle and an ejection fraction of 44 %. A cardiac muscle biopsy was performed and glycogen accumulation was seen (see below).\nHe had no clinical evidence of skeletal muscle disease or weakness, but a mildly elevated serum creatine kinase (s-CK) on one occasion. Muscle biopsy showed an abnormal glycogen pattern (see below) and a liver biopsy showed microvesicular steatosis and glycogen accumulation with mild inflammation. He had no hepatomegaly and his liver enzyme tests and liver ultrasound scans were normal.\nHe progressed to develop increasing fatigue and lethargy, worsening cardiac hypertrophy with secondary dilatation and worsening ejection fraction (28 % at 44 years). At age 45 years a cardiac MRI (Fig. ) demonstrated a severely dilated left ventricle with moderate-severely impaired ventricular function. Right ventricular function was normal without any focal thickening or hypertrophy. Regional wall motion assessment showed a complex pattern of akinesis. There was delayed gadolinium enhancement showing an extensive pattern of scarring with a patchy appearance, mostly in the mid-myocardial or sub-epicardial segments. The myocardium was thinned out in the posterolateral wall with almost full thickness scarring. Coronary angiogram at 45 years was normal.\nHe developed increasing issues with cardiac arrhythmia and an ICD was implanted. He had many episodes of anti-tachycardia pacing and three shocks.\nHe was listed for cardiac transplantation at 48 years of age and underwent a cadaveric donor transplant 1 month later. He had no significant complications and no clinical evidence of multi-system disease at the last examination 6 months post transplantation.
A 27-year-old nulliparous woman was admitted to the obstetrics department with preeclampsia at 29 weeks' gestation. She was following a normal course of pregnancy and complied well with her antenatal schedule, with no abnormalities detected during her visits or ultrasound scans. She was similarly well prior to becoming pregnant with no risk factors that could potentially complicate the pregnancy. The patient had previously been pregnant once, which resulted in a miscarriage, and had a notable past medical history of polycystic ovaries. She did not have a partner and stated that she wished no further involvement of the father in the pregnancy or future care of the baby, wishing to act as a single parent. This pregnancy had come after over a few years difficulty conceiving.\nDuring the course of her investigations for preeclampsia, she was noted to have ascites on trans-abdominal ultrasound scan and a slight abnormality of her liver function tests (LFT): raised alanine transaminase (ALT). An ultrasound scan and magnetic resonance imaging (MRI) of her pelvis was thus organised which identified a large, well-defined right-sided adnexal mass measuring 15 × 18 × 13.5 cm extending into the upper abdomen, with a vascular pedicle extending from the broad ligament. A smaller mass was also noted in the left adnexa measuring 4.5 cm occurring concurrently with a thickened peritoneum and omentum (Figures , , and ). These findings prompted measurements of her tumour markers which demonstrated alpha-fetoprotein 104.3, human chorionic gonadotrophin 14007, lactate dehydrogenase 172, and cancer antigen 125 (CA-125) 1266.\nThe right-sided mass was connected via its vascular pedicle to the broad ligament/uterine wall but despite a clear spatial relationship, there was little evidence that the tumour was connected to the right ovary. Some images of the right ovary also seemed to be normal. Considering that the patient was largely asymptomatic, this generated a preliminary differential diagnosis of a benign broad ligament tumour with ascites, subserous fibroid with ascites, or a broad ligament fibroma with Meigs' syndrome. We were also mindful that this could be a malignant ovarian tumour.\nInitial treatment plans involved a review by the oncology team with repeat radiological assessments as well as awaiting delivery with the intention to perform ovarian cystectomies in order to obtain a diagnosis in the first instance. Paracentesis had also been considered; the benefits of this were not compelling as it would not alter patient management considering that debulking would be a management mainstay of tumours of gynaecological origin. However, shortly following the initial admission, the patient underwent a caesarean section at 31 weeks' gestation due to foetal distress on cardiotocography (CTG) in which the baby was safely delivered. This procedure allowed visual inspection of this pelvis which demonstrated that by this stage, the right mass had progressed into a large 25 cm cystic lesion attached to the broad ligament and posterior wall of the uterus, with an 8 cm irregular ovarian mass on the left. The inspection of the stomach during surgery was unremarkable. Large volume ascites of 3-4 litres was also noted which was drained and sampled for cytology assessment along with omental and peritoneal biopsies.\nFollowing delivery, the patient underwent a right salpingoophorectomy and partial left oophorectomy. Histological analysis of the peritoneum and omentum samples showed infiltration by an adenocarcinoma with areas of signet ring cell differentiation. Immunostaining revealed that the tumour cells were positive for CK7 and CK20. Frozen sections of the ovaries confirmed that this was not high-grade serous ovarian cancer.\nThe presence of signet cells indicates that the primary malignancy is most likely of gastrointestinal origin and hence that the ovarian masses detected are metastatic, most likely a Krukenberg tumour. This was further supported by the immunochemistry results as tumours that are immunoreactive to CK7 and CK20 suggest a gastropancreatobiliary origin []. This conclusion resulted in the decision not to perform a pelvic clearance. Blood results demonstrating deranged clotting and the presence of a large haematoma on caesarean section also made this patient an unsuitable candidate for hysterectomy.\nThe possibility of a colorectal or lobular breast carcinoma origin had been considered and discussed at both upper GI and gynaecology multidisciplinary team meetings. An endoscopy would ideally have been performed to confirm the working diagnosis, however the patient declined this investigation. As described above, the patient had presented in rapid decline, with life-threatening ascites and pleural effusions. Hence, we were mindful of the window of opportunity to treatment—this would undoubtedly have been narrowed with comprehensive endoscopic investigations. Furthermore, even following debulking, the patient was of Performance Stage 2. Working on the immunohistopathology report which was most in keeping with an upper GI primary, EOX chemotherapy was commenced. This allowed us to begin prompt treatment but also had the added benefit of activity against our differentials which could not, at that stage, be definitively ruled out—oxaliplatin is effective in colorectal cancer and capecitabine is effective in both breast and colorectal cancers.
This 47-year-old male presented with a non-healing tongue ulcer of 3 months’ duration and associated pain for 2 months. He had initially noticed the asymptomatic ulcer on the ventral surface of tongue which had been increasing in size for the last 1 month. He had smoked beedis for the last 20 years and had consumed alcohol daily for past 15 years. On examination, ulcers were noticed in the tongue and buccal mucosa. A solitary ulcer (3 cm × 4 cm) with undermined edges and minimal induration was seen on right ventral surface of the tongue extending from the midline past the lateral border, up to the dorsal area of the anterior two-thirds of the tongue []. The area adjacent to the tongue ulcer appeared to be lobulated. Another ulcer (0.5 cm × 0.5 cm), also with an undermined edge was seen on the dorsal surface of tongue. In the left buccal mucosa, a single ulcer (1 × 1 cm2) covered with pseudomembrane was present, extending 3 cm from the angle of the mouth and 5 mm below the occlusal plane []. The patient was not aware of this ulcer. A single firm, non-tender (<1 cm) sub-mandibular lymph node was present on the right side.\nProvisional diagnoses of squamous cell carcinoma and lichen planus were given for the lesions of the tongue and buccal mucosa, respectively. Incisional biopsy from the edge of tongue ulcer revealed proliferating epithelium with the underlying connective tissue exhibiting chronic inflammatory cell infiltrate without evidence of epithelial dysplasia or malignant invasion. Biopsy was repeated from a different area of the tongue ulcer for further review. On examining serial sections, giant cells with peripherally arranged nucleus resembling Langerhans cells were appreciated along with caseous necrosis []. Chest radiography revealed bilateral upper lobe infiltrates [], and sputum was positive for acid-fast bacilli. HIV was negative. With anti-tuberculous therapy the lesions began to heal on a follow-up visit a month later. He was referred to local primary health care center for continuation of therapy.
A 29-year-old man (BMI: 32 kg/m2) was admitted to our Emergency Department for nausea and intermittent abdominal pain. The pain had increased for the last 2 days in severity and was associated with episodes of emesis. The patient had a story of undetermined colitis, treated with medical therapy, actually in phase of remission.\nAt clinical examination, the abdomen appeared flat, with a generalized tenderness, painful to palpation, in particular in the left upper quadrant. Lab tests showed a white blood cell count of 15,400/mm3. Chest and abdomen X-Rays were normal and abdominal ultrasound was normal too. On the contrary, the CT scan with intravenous contrast medium revealed an aspecific thickening of a tract of small bowel with partial contrastographic enhancement near the left colon, with a modest dilatation of the lumen of small bowel upstream of the injury, as reported in .\nTherefore, in order to perform a resolute diagnosis, in presence of an inconclusive and nondiagnostic CT scan, we decided to perform an emergency exploratory diagnostic laparoscopy. In the operating theater, after the first laparoscopic entry in the abdomen according to the open Hasson technique, an exploratory laparoscopy was performed. All the abdominal cavity was explored and all the bowel was inspected: at 35 cm from the Ligament of Treitz, a jejunojejunal intussusception was found. We decided to reduce the jejunal intussusception in order to define the pathologic cause, suspecting the presence of a mass. In fact, a mass of 4 cm in diameter was discovered on the intestinal serosal surface of the antimesenteric side and the mesentery presented multiple lymph nodes. The wall of the bowel was normal and no signs of ischemia were present.\nIn order to treat the cause of jejunal intussusception and to perform a histological diagnosis too, we decided to carry out an 8 cm laparotomy in the left upper abdominal quadrant and perform a resection of the affected jejunum (including the mass) and subsequently a jejunojejunal anastomosis.\nThe postoperative course was uneventful and the patient was discharged on the 5th postoperative day. Histological examination showed that the mass was a jejunal ectopic pancreas and that dissected lymph nodes simply share a lymphoid hyperplasia. According to the Heinrich classification system, this was a type 2 EP containing acini and ducts but no islets.
Patient A is a six-year-old male. He presented initially at the age of three years and four months with a seizure after a fall in which he hit his head. The initial seizure consisted of right gaze deviation, nystagmus, and clonic movements in his right upper limb. The duration of the first seizure was unknown. He had two more seizures lasting 20 minutes and 45 seconds, respectively. It was thought that the seizures were due to the fall initially rather than the patient falling as a result of the seizures.\nOver the next two years, the patient experienced nine breakthrough seizures and was diagnosed with epilepsy. He was initially started on valproic acid, which required dose optimization to gain better seizure control. Additionally, at four years and two months of age, clobazam was added to improve his seizure control. His final seizure occurred around five and a half years of age. He has since remained seizure-free on two antiseizure medications.\nHe also has behavioural difficulties and developmental delay, especially with language acquisition. While slow improvement in his development has been noted, at the age of five years and ten months of age, he was still only able to use 50 single words and could not put two words together.\nThe patient was born to a G1P0 mother at 39 weeks gestation via caesarean section due to failure to progress and followed an uneventful neonatal course. He was born in the Philippines and immigrated to Canada at three years of age. There is no relevant family history of seizure or developmental delay.\nAt three years and eight months of age, weight was 15.3 kg (42nd percentile), height 99.1 cm (32nd percentile), and head circumference 53 cm (98th percentile). His cardiovascular, respiratory, and abdominal exam was unremarkable. He had no neurocutaneous stigmata or dysmorphic features. He had full extraocular eye movements, normal pupillary reaction to light, and normal facial movements. He had normal strength and reflexes but was hypotonic. He walked with an ataxic wide-based gait and required assistance to prevent him from falling. At three years and ten months of age, he displayed an intention tremor in his upper limbs bilaterally. Since then, his physical exam has remained unchanged.\nAt the age of three years and ten months, the patient’s electroencephalogram (EEG) showed frequent epileptiform activity over the left temporal-central region. A brain magnetic resonance imaging (MRI) completed at the same age showed left mesial temporal sclerosis but did not show any other abnormalities that could explain his ataxia. The patient also underwent extensive routine, metabolic, and genetic testing (including a microarray CGH), which were all unremarkable. Finally, at six years of age, the patient had whole-exome sequencing, which ultimately provided a diagnosis of CACNA1A-related epileptic encephalopathy following the discovery of a genetic mutation in the calcium channel gene, CACNA1A. Specifically, this patient was heterozygous for a de novo pathogenic variant in the CACNA1A gene designated c.4174G>A, p.Val1392Met. The mode of inheritance is autosomal dominant. The patient’s disease phenotype is consistent with his diagnosis.\nThe patient continues to display intention tremor bilaterally and has an ataxic wide-based gait. The patient’s DEE is associated with chronic ataxia. His ataxia does not wax and wane and is present independent of when his seizures were not controlled or when his antiseizure medication doses were changed. His antiseizure medications’ trough levels are in the therapeutic range.
A 55-year-old male patient with acute myeloid leukemia (AML-M2) underwent RIC aHSCT in 2004 from his full matched male sibling. He had had methotrexate for four doses (days 1, 3, 6, and 11) and cyclosporine A (CyA) as GVHD prophylaxis. At the end of the third month, he had elevated liver enzymes. A biopsy revealed acute hepatic GVHD. Steroid was added to continuing CyA therapy. Because of rising enzyme levels, CyA could never be stopped, but steroid was tapered because of steroid side effects. CyA was continued at 25–50 mg daily doses until the fourth year of aHSCT when liver enzymes returned to the normal range and stabilized; CyA was then stopped. For about 2 years, the patient was free of any cGVHD signs and symptoms. In this period, the patient has been followed up at the outpatient transplantation clinic every month.\nAt about 5 years and 7 months of aHSCT, the patient was admitted to the outpatient transplantation clinic with extensive maculopapular rash and deep sclerotic cutaneous lesions with thickening of the skin in over 50% of the body surface area (Figures and ). He had no pruritus and no involvement of eye, mouth, and nails. Skin biopsy was reported as lichen sclerosis which was consistent with cutaneous cGVHD. According to the modified organ specific scoring of GVHD in 2014 NIH consensus criteria, the patient had a skin score of 3 for GVHD. This score resulted in making the diagnosis of severe GVHD according to NIH global severity of score []. Methylprednisolone 1 mg/kg and CyA 200 mg daily were begun and topical sodium fusidate was applied to skin lesions. Six months later, since the patient had no benefit from that treatment, CyA was stopped, methylprednisolone dose was lowered, and tacrolimus was started at 2 mg per day. As there was no satisfactory response to oral immunosuppressive treatment, PUVA therapy was performed for 22 times in 4 months with oral psoralen. As there was no reducing in the intensity of the lesions, extracorporeal photopheresis was added to the therapy every two weeks for two consecutive days. At about 3 months of phototherapy, skin lesions began regressing. Then, methylprednisolone was stopped and tacrolimus dose was reduced to 1 mg per day. For the last 3 years until May 2015, the patient has been on low dose daily tacrolimus 2 mg and photopheresis once a month. Although regression of the skin lesions was noted during three years, the rate of regression dropped at the end of three years as observed clinically and we decided to administer thalidomide. In June 2015, thalidomide 100 mg per day was started for the still persisting skin lesions. Thus, it has been five years since cutaneous cGVHD was first diagnosed, and limited success has been obtained despite the use of a wide range of therapeutic agents.
A 42 year old female patient was referred to the Department of Periodontology, Faculty of Dental Sciences, Sri Ramachandra Institute of Higher Education and Research (SRIHER) for the excision of a painless swelling present in the maxillary right gingiva which was interfering with the denture given for replacement of maxillary anterior teeth [Fig ].\nOn enquiring the history, the patient reported that the swelling was present since childhood which had gradually increased in size. There was no associated pain or bleeding associated with the growth of the swelling. The patient also presented with a history of Type I Neurofibromatosis with multiple small tumours present all over the body for which she had previously undergone excision. No pertinent family history was reported [Fig ].\nIntraoral examination revealed a sessile, non-ulcerated, diffuse, unilateral swelling present in the maxillary right area involving the marginal and attached gingiva corresponding to teeth 14-17. The swelling was fibrous, painless and of a non-inflammatory origin. It measured around 17mm in diameter and was firm on palpation. While the probing depth for the teeth was less than 3mm, attachment loss was found to the evident in the teeth associated with the lesion. All the associated teeth were found to be vital, with no decay and no accidental trauma. The radiographs taken in that area did not reveal any alteration. Ultrasound of the swelling revealed it to be of neurologic origin. A provisional diagnosis of NF1 of the gingiva was made and the lesion was planned to be excised in entirety using laser. Prior to excision, we had obtained medical fitness from the Department of Neurology and the Department of Dermatology, SRIHER.\nUnder local anesthesia, using appropriate laser precautions, the outline of the swelling was marked using laser [Fig ] and the lesion as a whole was excised using diode laser of 940nm wavelength with 3W power along with 2mm of normal tissue so as to differentiate between the normal and diseased tissue [Fig and Fig ].\nUpon achieving hemostasis, the area was thoroughly irrigated with saline and Coe-Pak™ (GC America Inc) surgical dressing was placed. The patient was recalled on day 1 to assess the healing and no complications were observed. The excised tissue was seen histologically which revealed the presence of proliferating spindle cells with wavy nuclei. The underlying connective tissue showed richly cellular nerve tissue comprising of spindle shaped cells with wavy nuclei in collagenous stroma along with few chronic inflammatory cell infiltration and increased vascularity. Review on day 7, three months and six months post-op revealed good healing [Fig ].\nOn 6 months post-op, the area of the lesion had healed completely and no indication of recurrence was observed. The patient was referred for further prosthetic management.
A 22-year-old female patient of Indian origin reported to the out-patient department with a chief complaint of a painful swelling in the right posterior mandible of 6 months duration. The swelling was insidious in onset and had progressed slowly to cause facial asymmetry along with mild parasthesia. On examination, there was a diffuse hard swelling on the right side of face with smooth margins extending anteroposteriorly from anterior border of the masseter muscle to the posterior border of the ramus and superoinferiorly from tragus to the inferior border of the mandible []. The skin overlying the swelling was normal in appearance with no rise in local temperature and there was no associated cervical lymphadenopathy. Intraorally there was bicortical expansion in the posterior region of the right mandible with a firm, tender soft-tissue mass posterior to the second molar. The mucosa overlying the lesion was erythematous and the third molar was clinically absent. There was no other apparent abnormality in the oral cavity except for missing maxillary third molar on the right side. The hematological parameters of the patient were within normal range. An orthopantomograph was advised and it revealed a large unilocular lesion in the ramus of the right mandible extending supero-inferiorly from the superior border of the ramus, involving the coronoid process, to the inferior border of the mandible, leaving a thin rim of bone inferiorly. Postero-anteriorly the lesion extended from posterior border of the ramus up to the body of the mandible distal to the second molar and involving the entire width and the anterior border of the ramus []. Based on the clinical and radiographic findings a provisional diagnosis of ameloblastoma was made with other benign/malignant odontogenic neoplasm, keratocystic odontogenic tumor, calcifying cystic odontogenic tumor or any other intraosseous neoplasm being kept as differential diagnosis. Fine needle aspiration (FNA) was performed, which yielded fluid with some cheesy white material. Papanicolau and H and E stained smears prepared from the aspirate showed abundant poorly preserved superficial epithelial squames along with numerous nucleated and anucleate keratin flakes in a background of necrosis, debris and a moderate amount of leukocytes []. The cytological findings were suggestive of a keratinizing cystic lesion leading to a tentative diagnosis of odontogenic keratocyst (OKC). An incisional biopsy was done and referred to a general Pathologist and was reported as ameloblastoma. Following the diagnosis of ameloblastoma and the extensive nature of the lesion, wide surgical excision was planned. Segmental mandibulectomy up to the distal of right second premolar was performed with preservation of condyle followed by reconstruction using titanium reconstruction plate and iliac crest graft.\nThe resected specimen showed a creamy white, brittle soft-tissue mass involving the ramus with complete destruction of the lingual cortical plate. The histological sections from the tumor mass revealed proliferation of odontogenic epithelium in the form of sheets, broad interconnected plexiform ribbons [] and few follicles with peripheral layer of tall columnar cells having hyperchromatic palisaded nuclei showing reverse polarity in focal areas. The central cells were ovoid to stellate reticulum like in appearance. Large areas of squamous metaplasia with extensive keratinization were evident within sheets of odontogenic epithelium []. In few places “keratin filled cystic spaces” were also evident [], some showing Pacinian corpuscle like stacks of lamellated parakeratin []. The background stroma was scanty, loose and edematous with areas of cystic degeneration but relatively free of inflammation. Based on the histological findings a final diagnosis of keratoameloblastoma was made. Patient is currently kept under close follow-up and so far has not shown any signs of persistent or recurrent disease 24 months post-surgery.
A 4-year-old boy, born in Zhejiang Province of China, was admitted to Yuying Children's Hospital affiliated to Wenzhou Medical University in June 2014 with complaints of productive cough accompanied with high fever for 5 days. He was the first-born child to unrelated healthy parents, born at 38 weeks of gestation after an unremarkable pregnancy. His birth weight was 3.5 kg, and meconium was passed on the first day of life. The patient had no history of meconium ileus or diabetes mellitus and lacked family history of CF. Tracing back his medical history, the patient was formula feeding but failure to thrive with a weight of 6.8 kg at the age of 8 months and had intermittent diarrhea. For further evaluation of the condition of growth and development, the patient was taken to a local hospital at the age of 8 months, and received complete blood count and liver function tests. And the results indicated liver involvement with slightly elevated alanine aminotransferase (ALT) and aspartate aminotransferase (AST) with values of 78 and 82 U/L, respectively. The patient suffered from recurrently and slightly elevated ALT levels ranging from 70 to 92 U/L and AST levels ranging from 80 to 90 U/L. In addition, the common etiologies that easily lead to increased levels of ALT and AST were also excluded, such as cytomegalovirus and hepatitis B virus infection. Initially, these symptoms were not paid enough attention by the physicians or parents because the elevated levels of ALT and AST can recover to normal levels automatically without treatment or through the injection of magnesium isoglycyrrhizinate before four years of age.\nPhysical examination for the patient at the age of 4 years showed a weigh of 16.5 kg with a height of 104 cm. The patient had a BMI of 15.3, which was in the 50th percentile for his age. The physical examination also revealed tachypnea and a barrel-shaped chest. The liver was palpable ~2 cm below the right costal margin, and the spleen was palpable about 1 cm below the left costal margin. Clubbed fingers were absent. Laboratory examination indicated increased ALT and AST values of 93 and 92 U/L, respectively, whereas other markers such as γ-glutamyl transferase (GGT), bilirubin, bile acid, fasting blood glucose, albumin, and globulin were within normal limits. Other laboratory investigations including of complete blood count, serum electrolytes, urine, arterial blood gas, amylase, and lipase were normal. The sputum and bronchoalveolar lavage fluid cultures tested positive for Pseudomonas aeruginosa. The Sudan III dye test of fecal matter indicated fat droplet positivity. Pulmonary function tests failed to be performed because of the difficulty at this young age for the child. Additionally, the lack of laboratory facilities caused impracticability of the sweat chloride test. Utilizing computed tomography (CT), we identified severe bilateral paranasal sinusitis () and diffuse fatty infiltration of the liver () in the patient. In addition, the chest CT scan verified the presence of bilateral bronchiectasis and marked peribronchial thickening, especially in the middle and lower lobes (). Extensive sticky and purulent secretion were observed in the lungs by bronchoscopy (). Based on the aforementioned pathological findings, the patient was primarily diagnosed with CF.\nGenetic testing of the patient revealed a homozygous nonsense mutation from a C-to-T substitution (c.1657C > T) in the CFTR gene, which was inherited from both his father and mother (). This single-nucleotide variant changed an arginine at position 553 into a premature termination codon (p.R553X). Notably, CF screening using amniotic fluid of the mother during her second pregnancy also indicated the fetus (sibling) to be a p.R553X carrier.\nOther than hypertonic saline nebulization, high-frequency chest wall oscillation, expectorant administration, pancreatic enzyme replacement therapy, and supplementation with vitamins A, D, E, and K, the child was prescribed intravenous ceftriaxone to address the P. aeruginosa. Respiratory symptoms gradually improved after 7 days of treatment, and he was discharged on the 15th day after admission. Ursodeoxycholic acid was prescribed after confirmation of genetic diagnosis, but taken irregularly by the patient. Therefore, the medicine failed to bring about the desired effect. Remarkably, the patient was later re-hospitalized two times because of pulmonary infections and liver involvement. Liver function test showed that the levels of both ALT and AST ranged from 90 to 120 U/L. Further examination of abdominal CT and ultrasound have suggested the progression of hepatic cirrhosis. The final hospital admission in August 2016 was due to complaint of a stomachache for 3 days.\nAbdominal CT showed a wave-like margin of the liver and many areas of multifocal hypoattenuation in the liver, which indicated the occurrence of hepatic cirrhosis on the basis of diffuse hepatic steatosis (). Simultaneously, the patient presented with pancreatic atrophy and splenomegaly (). In addition, both chest CT scan and bronchoscopy showed the characteristics of bilateral bronchiectasis, marked peribronchial thickening, and extensive sticky and purulent secretion, similar to that observed in 2014 (). The abnormal prothrombin time (PT) and activated partial thromboplastin time were 20.2 s (normal control: 13 s) and 52.6 s (normal control: 36 s), respectively. The international normalized ratio (INR) was 1.85, which confirmed the diagnosis of liver failure.
An 11-year-old girl was referred to our hospital in August 2010 with a lesion in the right cheek area which was progressively enlarging. The patient complained that her nose and mouth corner were crooked and that her face was swelling. In clinical examination, the patient had a slight asymmetry in the right midface as a result of buccal and palatal cortical expansion from the right maxillary canine to the molar region, resulting in depression of the nasal alar and mouth corner (Fig. ).\nA panoramic radiograph showed an increased bone density on the right maxilla and zygoma and obliteration of the right maxillary sinus. Computed tomography (CT) revealed a 4.5 × 4 × 4.5 cm, expansile ground-glass opacity lesion involving the right maxillary sinus, right maxillary alveolar process, zygoma, and hard palate. Bone scan revealed an irregularly shaped hot uptake in the right maxilla, and no abnormally increased uptake was observed at any other sites (Fig. ). The physical examination did not show any other lesions, and the patient had no history of pain, trauma, loosening of teeth, or trismus. Based on the typical radiologic findings, the patient was diagnosed with FD, and no additional biopsy was performed. The patient had regular follow-up every 6 months to monitor the lesion’s progress. At the 1-year follow-up, the development of tooth germ within the lesion was normal, and slight expansion of the lesion to the bucco-lingual side was observed. Because we thought the patient was still growing and increasing in height, we decided to conduct an ongoing progress observation.\nAbout 3 years later in December 2012, there were no significant changes of the FD lesion, but the distance from the mouth corner to the inner canthus was about 2 cm longer on the right side than on the left. Periodic observation was continued, and in August 2017, corrective surgery was planned because the maturation of the lesion was confirmed to be complete and there were no changes in the size of the lesion. At that time, the distance from the mouth corner to the inner canthus was 2.5 cm longer on the right side than on the left, and the distance from the occlusal plane to the outer canthus was 5 cm longer on the right side than on the left. Bone contouring surgery, the primary treatment for facial asymmetry and fibrotic bone lesions, was planned (Fig. ).\nThe patient wanted to improve asymmetrical facial appearance through the surgery. Therefore, we aimed not only to remove the FD lesion, but also to make the patient’s facial as symmetrical as possible. For this, direct visualization and surgical approach to the infraorbital rim and lateral area of zygoma were required, but the surgical approach through buccogingival incision had limited access to these areas. On the other hand, the midfacial degloving approach was expected to help reestablishing symmetric facial contour by allowing direct comparison of the lesion with the normal side. Moreover, this approach provides esthetically acceptable outcomes, leaving no scars and no functional disability. Therefore, we decided to perform the operation through the midfacial degloving approach.\nWith the patient under orotracheal anesthesia, the lesion was removed by the midfacial degloving surgical procedure. Local anesthesia with 2% lidocaine with epinephrine (1:100,000) was infiltrated into the maxillary vestibular mucosa and into the nose. The procedure is performed with a maxillary vestibular incision and three intranasal incisions to expose the entire midface skeleton that include (1) bilateral intercartilaginous, (2) complete transfixion, and (3) bilateral piriform aperture incisions (Fig. ).\nA buccogingival incision was made in the maxillary vestibule approximately 5 mm superior to the mucogingival junction and extended from the second molar to the contralateral second molar. Periosteal elevators were used to elevate the tissues in the subperiosteal plane fist over the anterior maxilla and then extending widely to encompass posterior tissues behind the zygomaticomaxillary buttress. The infraorbital neurovascular bundle was identified superiorly and dissected. Subperiosteal dissection along the piriform aperture stripped the attachments of the nasal labial muscularture to allow its complete release from the midface skeleton. The mucoperiosteal flap was elevated up to the piriform aperture.\nThe intercartilaginous incision divided the junction between the upper and lower lateral cartilages (Fig. b). An incision was made along the inferior border of the upper lateral cartilage, beginning at the lateral end and extending medially curved into the membranous septum anterior to meet transfixion incision (Fig. a). Laterally, the incision was sufficient that it extended to the piriform aperture. The lower lateral cartilage was eventually displaced superiorly during the degloving procedure, whereas the upper lateral cartilage remained attached to the midface skeleton. The transfixion incision was used to separate the membrane septum/columella from the cartilaginous septum. An incision was made along the caudal border of the septal cartilage from the medial end of the intercartilaginous incision toward the anterior spine (Fig. a). The intranasal incision was made by a full-thickness incision down through the periosteum of the piriform margin and the nasal floor.\nDissection through the intercarilaginous incision allowed access to the nasal dorsum and bones (Fig. b). Sharp subperichondrial dissection with a scalpel or a blunt dissection with scissors freed the soft tissues above the upper lateral cartilage as in a standard open rhinoplasty. The dissection should be within the subperichondrium plane to prevent injury to the overlying musculature and blood vessels of the nose. Elevation extended laterally to the nasomaxillary sutures and superiorly to the glabella. Retraction of the freed soft tissues allowed sharp incision to be made with a scalpel or with sharp periosteal elevators through the periosteum at the inferior edge of the nasal bones. Elevation of the soft tissue laterally to the piriform aperture was also performed so that the maxillary vestibular dissection was easily connected to this pocket.\nAfter the connection of the nasal and oral incisions, the midface was degloved. The midface skin was separated from the maxilla and the nasal pyramid. The upper lip and the intact nasal columella, nasal tip, and alar cartilages were then retracted by two Penrose drains introduced through the nostrils over the nose to the level of the inferior orbital rim. This approach provided visualization of the medial maxillary wall, pterygoid junction, nasofrontal suture, infraorbital rim, and laterally to the temporal process of the zygoma (Fig. d). Under direct visualization, the overgrowing bone lesion was then excised using osteotomes and saws. The right maxilla was drilled further at the orbital rim and laterally till zygomatic complex. The contour of the midface was reestablished using burr to give a cosmetically normal looking midfacial skeletal contour while protecting the infraorbital nerve (Fig. ). For the removed lesion, a biopsy was performed for the accurate diagnosis and histologically confirmed as FD. The soft tissues were then carefully redraped and the nasal tip brought back into position. The intranasal incisions were closed using 4-0 resorbable sutures (vicryl), and the transfixion sutures were precisely performed to determine the final position of the nasal tip and prevent vestibular stenosis. The cinch suture of alar base was used to prevent postoperative alar base widening. The intraoral incisions were closed using a 3-0 black silk. Nasal packing into the maxillary dead space with Vaseline gauze was done for 3 days in order to minimize the postoperative bleedings.\nThe patient’s postoperative course was generally uneventful. There was moderate nasal crusting for 3–4 days. Mild swelling with periorbital ecchymosis disappeared after 2 weeks, and transient paresthesia around the infraorbital nerve spontaneously resolved after 3 month. No postoperative complications such as epistaxis, vestibular stenosis, or esthetic problems of the nose were seen. Clinical and radiographic examinations obtained 4 months after surgery showed the anatomical structures were in a stable state without recurrence of FD (Fig. ). The esthetic result was satisfactory for the patient, and occlusal state was also well maintained (Fig. ). Therefore, no additional orthodontic treatment or orthognathic surgery was performed.
Our patient is a 68-year-old white female with a two-year history of progressive dyspnea and a persistent non-productive cough. Past medical history included hypertension, lower extremity neuropathy of unknown etiology, and interstitial cystitis. She had a remote history of tobacco use but no exposures to other toxins. Family history was positive for stroke, diabetes, coronary artery disease, and gastric cancer. Physical exam was normal except for bilateral trace lower extremity edema. No jugular venous distension, heart murmur, or tumor plop was present.\nFour months previously, a CT scan of the chest, pulmonary function tests, and a pulmonary angiogram were normal. A sleep study showed no apnea but nocturnal hypoxia prompting a prescription for home O2 at night.\nAt the time of re-evaluation, a transthoracic echocardiogram (TTE) demonstrated a normal ejection fraction of 60% with normal chamber dimensions but showed a large right atrial mass with high flow velocity around the echodensity. A small patent foramen ovale (PFO) was also present. Estimated pulmonary artery pressure was 44 mmHg. A transesophageal echocardiogram confirmed the large immobile right atrial mass, which involved the IVC. A CT scan revealed a cystic dense mass in the IVC and the right atrium with no evidence of pulmonary emboli or metastases (Figure ). The abdomen and pelvis were negative with no evidence of renal cell carcinoma, which can also present with IVC involvement.\nThere was mild cerebral atrophy without evidence of intracranial masses on a CT scan of the head. A combined PET (positron emission tomography)/CT scan of the chest showed a partially contrast-enhancing intraluminal mass within the intrahepatic and suprahepatic inferior vena cava extending into the right atrium. There was no extension into the hepatic veins. The mass markedly narrowed the suprahepatic inferior vena cava and right atrium with only a small amount of residual lumen noted medially. There was diffuse heterogeneity of the liver without evidence of mass lesion, suggesting hepatic venous congestion. The PET imaging demonstrated uptake in the tumor mass consistent with malignancy but no evidence of metastatic disease. Magnetic resonance angiography (MRA/MRI) showed that the large mass centered at the confluence of the inferior vena cava and right atrium and extended into the right atrium (Figure ). The mass extended 2.5 cm below the diaphragm. The widest transverse dimension was 4.5 cm and the greatest length obliquely was 5.6 cm. There was collateral venous flow with dilatation of the azygous vein and lumbar collaterals.\nA fluoroscopically guided biopsy was preformed and histology revealed spindle shaped cells. Immunostains for smooth muscle actin and desmin were positive and those for melanoma and cytokeratin were negative, consistent with the diagnosis of leiomyosarcoma (Figure ).\nIntraoperatively, the tumor was noted to originate from the posterior aspect of the IVC and measured approximately 8 cm in length by 4 cm in width, with the widest part lying within the right atrium. Ten minutes of circulatory arrest was required and the tumor was excised along with a portion of the posterior inferior vena cava. The tumor mass was well demarcated with gross total resection. Of note, tumor margins at the time of resection were negative. The inferior vena cava was then closed primarily from the intraluminal side. Surgical clips were left in place to help identify the target for radiation therapy. In addition, the patient had her PFO surgically corrected. The patient tolerated the procedure well and was weaned from bypass without difficulty.\nPostoperatively, the patient received radiation therapy. The patient received a course targeting the resection bed of the origin of her tumor in the inferior vena cava. She was treated with photons of 18 megavolts (MV) at the isocenter at 2 Gray (Gy) per fraction for 25 fractions to a total dose of 50 Gy. This was followed by an additional boost to a small area for an additional five fractions to a total dose 10 Gy. The total dose to the tumor bed was 60 Gy. The patient tolerated the course of radiation well and reported no side effects, such as skin toxicity or gastrointestinal toxicity. There were no breaks during treatment.\nSubsequent clinical examinations every 6 months and imaging with yearly CT scans have confirmed that the patient has remained disease free 5 years after treatment.
A 47-year-old female visited the hospital with a sudden abdominal pain and swelling on the right side of the abdomen. The patient had no co-morbidities or no previous history of a liver disease. At another hospital, she underwent abdomen ultrasonography. She was diagnosed with mucinous cystic neoplasm of the liver. More than two litres of blood were aspirated from the cyst. She received blood transfusion. Subsequent transarterial embolization was carried out to tackle the bleeding. However, her pain persisted. A follow-up computed tomography was suggestive of a differential diagnosis of a 19-cm hemangioma with internal hemorrhage or mucinous cystic neoplasm along with venous congestion in the right liver ().\nShe was then offered a curative surgical treatment. On blood investigations, all her results were within normal limits except a slightly raised alkaline phosphatase level. An open extended right hemihepatectomy was planned. During the surgery, a huge cystic hepatic tumor with dilated and engorged arteries and portal veins at the porta was observed (). An intraoperative ultrasound was carried out. It showed a cystic lesion containing septations along with solid components. Using an anterior approach, extended right hemihepatectomy including middle hepatic vein was performed. Due to engorged vessels, division of the right Glisson pedicle was difficult. A decision was made to use vascular staplers. After completing liver resection, the right liver was mobilized and removed without rupture of the tumor. A Jackson Pratt drain was inserted. After specimen retrieval, the remnant common and left hepatic artery engorgement were slightly reduced. The surgery lasted 150 minutes with an estimated blood loss of 350 mL. The patient was transferred to the intensive care unit considering the possibility of hemodynamic changes due to the presence of engorged arteries. Post-surgery, the patient remained vitally stable without any hemodynamic instability or immediate postoperative complications. She was discharged on the 6th postoperative day.\nHistopathology showed a 15 cm × 13 cm × 6 cm hepatic AML with a combination of internal hemorrhage and 60% of epithelioid cells. Mild sinusoidal dilatation with no specific abnormality in microscopic arteries was found (). On follow-up at 6 months after the surgery, the patient was doing well with normal liver function. She had no complications. A follow-up computed tomography showed well regenerated remnant left liver and a further reduction of perihepatic arteries ().
Patient: a 55-year-old Japanese female. None of the factors contributing to lateral compartment knee disease, such as obesity, complications, or psychiatric disorders, were present in the patient. During hyaluronic acid (HA) treatment, although we prescribed strength training to the patient, she did not perform it frequently enough because of severe pain. As the knee pain was caused by tripping while playing tennis, the possibility of trauma cannot be ruled out. In 2013, due to pain in both knees, she visited our hospital and was diagnosed with OA of the knee. In December 2017, while playing tennis, the right knee developed a knee collapse. She was examined at the hospital, and an MRI of the right knee was performed. Horizontal dissection of the lateral meniscus and cartilage defect on the lateral condyle of the femur were observed. Since 2018, injections of hyaluronic acid have been administered every 2 weeks, but joint edema and pain recurrence have been remitted. The patient had already undergone HA treatment and received multiple steroid joint injections; however, these interventions were not very effective. Moreover, the patient was unwilling to undergo these treatments. The patient was aware that our hospital would start providing regenerative medicine treatments and had been waiting for a year before the start of the treatment. During that period, the patient received HA treatment, which proved ineffective. Therefore, the patient requested ASC treatment in April 2018. In April 2018, MRI of the right knee was performed. A cartilage defect was found in the external condyle of the femur, and the patient desired ASC transplantation. The range of motion of the right knee is 0–145. Anteroposterior (AP) and lateral radiograph of the right knee are shown in Fig. . On 28 April 2018, 20 mL ASCs were collected from the abdomen. On 11 June 2018, the first ASC transplantation was performed on the right knee. On 2 July 2018, although the effect was experienced after 1 week
This was a 40 year old, premigravida patient who initially presented to the infertility clinic at our institution for desired pregnancy. Her past medical history was significant for primary infertility. A successful intrauterine pregnancy was achieved via intrauterine insemination. Due to advanced maternal age, the patient underwent extensive prenatal testing and monitoring through the course of her pregnancy. All tests were normal, with the exception of a left ovarian mass that was incidentally detected by ultrasound during the third trimester. The patient was closely followed without prenatal surgical intervention. Her pregnancy advanced uneventfully, and labor commenced at 40-week gestation. Due to failure to progress, a caesarean section was performed resulting in the delivery of a healthy female infant. At the time of caesarean section, a left oophorectomy was performed.\nThe specimen was received fresh for intraoperative pathology consultation. On gross examination, it consisted of an intact, unilocular, thin-walled cyst measuring 15 × 12 × 5 cm and filled with clear fluid. Both the outer and the inner surfaces of the cyst were smooth. The cyst wall ranged from 0.1 cm to 0.8 cm in thickness and showed marked edema. Based on the gross findings, an intraoperative interpretation of benign ovarian cyst was made. No frozen section was performed.\nOn subsequent microscopic examination, the cyst was lined by single to multiple layers of large cells with abundant eosinophilic cytoplasm (Figure ). Most cells showed small, round and regular nuclei, but focal cells displayed enlarged and bizarre nuclei with hyperchromasia and occasional mitosis (Figure ). The outer fibrous wall of the cyst showed edema and nests of luteinized cells that were morphologically similar to the cyst lining cells. Special stain showed reticulin fibers around nests of luteinized cells in the cyst lining (Figure ) and in the outer cyst wall. Adjacent residual ovarian tissue exhibited a corpus luteum and numerous cystic follicles.\nThe patient has been free from disease for 7 years after surgery.
A 36-year-old Hispanic man presented to the emergency department complaining of right groin pain for one week. He described the pain as aching (6–8/10) in severity, localized to the right groin, precipitated by walking, and relieved by resting for approximately 10 minutes. The pain had progressively worsened over the previous week, and causing a painful limp at the time of presentation, he denied any morning joint stiffness, muscle aches, other joint pain, rash, fever, or back pain. He had taken a few tablets of nonsteroidal anti-inflammatory drugs every day for the last week, with minimal pain relief.\nUpon review of other organ systems the patient described that he had been feeling subtle weakness in the left upper and lower extremities which started three years earlier and became slowly progressive. He stated that the weakness was not causing any limitation of activity or falls and denied any history of urinary or bowel incontinence, numbness, or pain in the extremities other than the presenting complaint.\nWhen evaluated for the weakness two years earlier an MRI of the brain was ordered and reviewed by his primary care physician with reportedly normal results.\nOn physical examination, no erythema or swelling around the right hip or groin area, and no inguinal lymphadenopathy were noted. The passive range of motion of the right hip was limited and painful, especially forced internal rotation. Passive internal and external rotation of the extended leg “log roll test” and straight-leg raise test elicited pain. Bilateral hyperpigmentation from the knees to the toes was noted, with mild bilateral lower extremity pitting edema, and normal peripheral pulses.\nCranial nerves examination revealed a left spinal accessory nerve lesion as the patient was unable to shrug his left shoulder. Tongue fasciculation was noted with a slight deviation of the tongue to the left on protrusion, suggesting a left hypoglossal nerve lesion. The remainder of cranial nerves examination was normal with negative meningeal signs.\nMotor examination revealed left upper and lower extremity weakness with a power of 4/5, in the right upper and lower extremities the power was normal 5/5. Deep tendon reflexes were exaggerated in the left upper and lower extremities and normal on the right. Increased muscle tone in the left upper and lower extremities was also noted, with a positive Babinski sign and ankle clonus on the left side. No muscle atrophy or other muscle fasciculations were identified.\nSensory examination revealed loss of vibration and position sensation in the left lower extremity, pain and temperature sensations were impaired in the left arm, and in a cape-like distribution across the shoulders and upper torso, anteriorly to the level of T2 dermatome and posteriorly to L1 dermatome. On rectal exam, the patient had a normal sphincter tone, with no evidence of prostate tenderness or other pathologies. Serological tests for syphilis, that included serum RPR and VDRL, were negative, with normal serum chemistry, B12, folate level, and complete blood count.\nRight hip X-ray () appeared to be normal with no fractures or dislocations and a preserved articular space.\nRight hip joint MRI () showed a wedge shaped subcortical enhancement with adjacent bone marrow edema in the right femoral head posterior articular surface, suggestive of avascular necrosis or early radiological signs Charcot arthropathy considering the patients underlying neurological disease.\nMRI of the brain was normal, with no evidence of infarcts, atrophy, or any other intracranial pathology.\nMRI of the cervical and thoracic spine () showed a 9 mm inferior herniation of the cerebellar tonsils through the foramen magnum, compressing the craniocervical junction (Type I Chiari Malformation) and extensive cervical and thoracic spinal cord syringohydromyelia.\nThe patient underwent neurosurgical intervention, where cervical decompression with a suboccipital craniectomy and C1 laminectomy was performed. He was discharged to a rehab facility where regular physical therapy and follow-up in an out-patient clinic was arranged. Over the following two years the patient was able ambulate with assistance due to slight progression of left lower extremity weakness (opposite to the site of the hip neuroarthropathy); he had no recurrent right hip pain or joint deformities, and no orthopedic surgery was required.
A 63-year-old white man was referred to us with a solitary nodule on the right side of the neck that had appeared 2 months after the surgical removal of a SCC of the floor of the oral cavity.\nThis carcinoma was focused on the middle of the tongue on the right side with a limited extension to the adjacent oral floor. The neoplasm was >4 cm, and there was involvement of the right neck nodes at levels I, II, and III with skin adhesion and ulceration. One of these nodes had a diameter of >6 cm. According to the 2010 American Joint Committee on Cancer TNM Classification of Tumors of the oral cavity, it was cT3N3M0 (stage IVB).\nIn accordance with the National Comprehensive Cancer Network guidelines (2011), the surgical treatment was an excision of the primary tumor of the tongue and oral floor (a pull-through operation) and ipsilateral neck dissection (levels I, II, III, IV, and V) with ablation of the skin involved with the tumor. A secondary reconstruction with a myocutaneous pectoralis major muscle flap with 2 skin isles was used for reconstruction of the oral mucosa and laterocervical skin.\nThe histopathology was characterized by a poorly differentiated SCC (G3) of the oral cavity. The dissection of the neck nodes showed metastases with extracapsular spread and skin infiltration. The final histopathological classification was pT3N3M0 (stage IV).\nThe patient reported that the skin nodule was growing rapidly. He had a painless, erythematous, dome-shaped nodule with suppurative foci on the right side of the neck near the surgical suture. The nodule ranged from 4 × 4 cm in diameter (). An incisional biopsy specimen revealed small and round cells with infiltration of the underlying connective tissue. On the basis of the histopathological features, SCC was diagnosed.\nThe 2011 National Comprehensive Cancer Network guidelines advise surgery or chemoradiotherapy, and this case was discussed with the Oncological Tumor Board of the Department of Organs of Sense of the “Sapienza” University of Rome. The patient refused other major surgical treatments, as well as chemotherapy or radiotherapy. In this condition, we decided to treat the patient with ECT because a review of the international literature showed that it had good results and was indicated for all relapsing tumors of the skin regardless of the dimensions and histological types. If the treatment with ECT had failed, surgery or chemoradiotherapy treatments were possible as additional treatment options.\nThe patient was treated with ECT with intravenous sedation and local anesthesia. Eight minutes after 15 mg/m2 of intravenous bleomycin therapy was begun, a needle electrode-hexagonal array (length, 25 mm) was inserted directly into the tumor mass. A series of 8 pulses of 1000 V/cm was delivered at a frequency of 5 kHz and duration of 100 microseconds. To ensure that the lesional tissue received an adequate concentration of bleomycin, the treatment was completed 30 minutes after the end of the infusion. A sterile medication was applied on the treatment site.\nThe patient had no residual pain, fever, or discomfort after the treatment and was discharged the next day. When the sterile dressing was removed after 7 days, the tumor mass was detached (). Two months after treatment, the lesion was diminished in diameter and the absence of disease recurrence was confirmed by an incisional biopsy.\nThree months after the treatment, the lesion had disappeared and the skin was completely sheltered (). Two years after the treatment, the patient currently shows no signs of local or systemic disease recurrence.
A baby female, aged 4 months, was referred to our Orthodontics and Paediatric Dentistry Unit. The patient was receiving breast-feeding since birth. Her parents reported, during the first months of life, the spontaneous formation of a pink oblong vesicle in the left internal part of the lower lip (). The color [], the localization [], and the shape [] have been considered coherent with a mucocele lesion; however, a conclusive diagnosis requires histopathological examination. As spontaneous regression of these oral lesions has been reported [], the parents planned only a further control.\nAfter a month, the patient returned, and the lesion had a significant growth with a shape modification and appeared as a more regular bulla (). Therefore, the excision intervention was planned. The size of lesion in the moment of surgery was about 10 mm × 6 mm.\nThe objective of the treatment was mucocele treatment with topic anesthesia and laser-assisted excision.\nThe first possible alternative to the treatment was the delay of the intervention, thus planning only further controls, with the risk of lesion growth and feeding problems. Another alternative consisted in a conventional lancet surgical approach, with the consequent risks of intra- and postoperative bleeding and potential difficulties in wound healing. The last option was marsupialization that would allow lesion drainage without excision, but this technique is more fitted for larger lesions.\nWritten informed consent was obtained from the patient's parents to proceed with lesion excision with laser surgery. The patient and the whole staff wore protective glasses to prevent eye damage []. Local topic anesthesia was performed with lidocaine gel local application for one minute. Diode laser (Diode Laser, DMT Lissone, Italy) at 810 nm wavelength, continuous wave mode with a power output of 3 watt, and a 0.4 mm diameter fiber optic were set for excising the lesion. The tip was directed at an angle of 10 to 15°, moving around the base of the lesion with a circular motion [] (). It took 3 minutes to complete the procedure. The diode laser provided a combination of clean cutting of the tissue and hemostasis (). The patient was discharged with necessary postoperative instructions for maintenance of good oral hygiene and keeping the area clean. No additional analgesic or antibiotic was recommended.\nAfter excision, the lesion was immersed in formalin and then was sent to histopathologic service for evaluation.\nAfter excision, the patient had no signs of respiratory distress and no feeding difficulty was reported from the parents. The patient was visited with a follow-up of 2 weeks and 3 months. After 2 weeks follow-up (), the intraoral wound healed without complications and no signs of infection or mass recurrence were noted. After 4 months () follow-up, the lesion healed completely and the patient had a functionally and developmentally normal mucosa without lesion recurrence. The patient demonstrated age-appropriate weight gain.\nThe histopathological examination confirmed the initial clinical diagnosis of mucocele. Grossly, the lesion displayed a polypoid fashion and was covered by a smooth mucosal layer (). Histologic examination revealed a process deep seated within the submucosal connective tissue (), consisting of newly formed capillary vessels intermingled with a chronic, lymphohistiocytic inflammatory infiltrate and associated with deposition of extracellular mucin (), the latter which resulted Alcian blue positive (). Thus, the histopathologic picture was consistent with stromal reaction to extravasated mucin, possibly related to an injured, salivary gland mucous cyst (mucocele).
A 44-year-old Caucasian woman was referred to the ophthalmology unit four hours after sudden painless loss of vision in her right eye. She was in remission following six months of chemotherapy with cyclophosphomide and fludarabine for chronic lymphocytic leukaemia. Two months after her chemotherapy ceased (9 months prior to this presentation) she had severe right sided herpes zoster ophthalmicus with uveitis that was treated with topical steroids. She was a smoker and on hormone replacement therapy.\nHer visual acuity was count fingers at one metre in the right eye and 6/6 in the left eye. Pupils were equal and reactive with a right relative afferent pupillary defect. Dilated fundus examination at presentation was essentially normal showing healthy discs with some mild arteriolar attenuation in the right eye. Whilst the history was highly suggestive of a central retinal artery occlusion (CRAO) optic neuritis could not be excluded. Dual therapy was commenced with ocular massage and intravenous acetazolamide and high dose intravenous (IV) methylprednisolone (1 gram daily) and oral famciclovir (500 mg TDS) for a possible underlying vasculitic cause or optic neuritis. She continued the IV steroids for a total of three days and the oral famciclovir for three weeks.\nDilated fundus examination of the right eye the following day demonstrated a cherry red spot and retinal oedema, findings consistent with right CRAO (Figure ). The early and late phase fluorescein angiogram images showed reperfusion of the right central retinal artery with no signs of optic neuritis or retinal vasculitis.\nAn embolic screen for possible sources of emboli included routine blood tests, carotid doppler ultrasound and a transthoracic echocardiogram. Blood tests were normal with no active leukaemia and erythrocyte sedimentation rate of 19 and c-reactive protein < 3. The carotid doppler ultrasound revealed complete occlusion of the right ICA with a patent left ICA. CT angiogram the same day supported the finding of 100% right ICA occlusion with no signs of calcification and demonstrated a generalized diminished right intracranial circulation (Figure ).\nVascular surgery was not undertaken as the occlusion was total and stable and the right intracranial circulation was well collateralized. At one month follow up she was clinically stable with no visual recovery.
A 49-year-old male smoker with a history of squamous cell carcinoma was found to have a 4 cm left hilar mass on CT scan. An EBUS FNA performed of the mass demonstrated abundant mucinous material with clusters of pleomorphic cells []. The nuclei were enlarged and hyperchromatic, with coarse chromatin and dense basophilic cytoplasm. The background showed abundant mucinous material with entrapped inflammatory cells and bronchial cells []. The ROSE was malignant, favoring a nonsmall cell carcinoma; however, it was uncertain if the mucin was associated with the neoplastic cells or simply a contaminant. The immunohistochemical stains performed on sections of the cell block revealed that the tumor cells were positive for p63, CK5/6, and negative for TTF1. In addition, the tumor was similar in morphology to the patient’s prior lung biopsy with a diagnosis of squamous cell carcinoma. The final diagnosis was metastatic squamous cell carcinoma with the mucin attributed to bronchial contamination.\nThis case illustrates the discrepancy or mismatch that may exist between background material and the cells of interest in EBUS FNA. For example, a mucinous background usually leads us to infer that there is a mucin-producing adenocarcinoma []; however, similar to pancreatic EUS-guided FNAs, mucin can be a contaminant from the needle path and is not necessarily produced by the tumor cells present. This is an important aspect of EBUS FNA to be aware of, particularly because cytopathologists often rely on the background material to aid in making a diagnosis and for subtyping tumors.\nWhen looking at an EBUS FNA with a mucinous background, there are two cytomorphologic clues that can help to determine if the mucin is truly indicative of a mucin-producing adenocarcinoma. The first clue is the presence of columnar cells, or cells with voluminous mucinous cytoplasm or targetoid vacuoles with a central mucin droplet. A second clue is the presence of “dirty” mucin with entrapped inflammatory cells and debris, which usually signifies mucin contamination [, and ]. This is in contrast to mucinous adenocarcinomas [], which usually have a “clean” mucinous background with thick mucin that does not have entrapped inflammatory cells or debris. In our experience with EBUS FNA, we have seen several tumors, including small cell carcinoma [] and this case of squamous cell carcinoma [], associated with mucinous contamination. Other cystic mucinous neoplasms occurring in the lung or mediastinum should also be considered, such as mucinous cystadenocarcinoma, which has been described in the lung.[] The final thing to consider is mucinous contamination only, without associated tumor cells or lymphocytes [], which indicates a nondiagnostic specimen and the need to ask the surgeon for additional material. In some cases, the presence of reactive bronchial cells with cytologic atypia within a mucinous background, may present a diagnostic difficulty in determining whether the specimen is simply bronchial contamination (and therefore nondiagnostic) or truly diagnostic of a metastatic mucinous adenocarcinoma.\nIn addition to a mucinous background, other types of background material are associated with a particular differential diagnosis in EBUS FNAs, and the four main categories of background material with their associations are summarized in .
GN was a 5-month-old female patient at the time of her initial presentation to our clinic. She was a product of a nonconsanguineous union and born at 44 weeks via vaginal delivery. She had two older deceased male siblings, and it is unknown if these two brothers shared the same father. The oldest brother passed away at the age of 3 months of a suspected unspecified infection. Two weeks prior to the patient's initial presentation to us, her second brother passed away from sepsis at the age of five years. This brother was confirmed to have X-linked CGD at an outside institution where he was unfortunately lost to follow-up. The mother said that she was tested at the outside institution as well and was found to be a carrier for the same pathogenic mutation. She denied a personal history of significant or life-threatening infections or history of autoimmunity.\nAt the age of 2 months, GN started to develop recurrent oral ulcers. She also had a chronic waxing and waning rash on her face and extremities. The rash would flare after receiving vaccines and during bouts of viral respiratory tract infections. The patient had one episode of coxsackie virus and no history of bacterial or fungal infections. She had recurrent oral mucosal erosions, a violaceous periorbital rash, and malar edematous erythematous patches and plaques. Serial infectious workup of the oral lesions was negative. A skin biopsy showed an interface dermatitis with a primarily lymphocytic infiltrate at the dermoepidermal junction with perivascular involvement. Most of the cells in the infiltrate expressed CD68 and myeloperoxidase, features of immature myeloid cells. This was a primarily T-cell infiltrate as most cells were CD3+, and there were few CD20 + cells. The skin biopsy was negative for bacteria, acid fast bacilli, and fungi. Her oral and cutaneous lesions responded to fluocinonide 0.05% gel (mouth) and fluocinonide ointment to affected areas of skin. Interestingly, GN's recently deceased brother had a similar rash on his face and his skin biopsy identified interface dermatitis with a mild granulomatous infiltrate.\nGenetic testing confirmed the patient to be a heterozygous carrier of the pathogenic CYBB p.Ile248AsnfsX36 variant. She also had a CYBA mutation p.Glu135Lys of uncertain significance. Her neutrophil oxidative burst was initially 46% but normalized to 99% when rechecked.\nGN also had intermittent neutropenia with a nadir of 600 K/μL. Anti-neutrophil IgM was weakly positive. Over time, the neutrophil count trended up and stabilized at 1.4 K–2.2 K/μL. A bone marrow biopsy was considered but declined by the parent. She did not have hypocomplementemia, and aside for a mildly positive ANA (40 units, homogenous pattern) and a proteinase 3 (PR3) antibody positive at 1.2 AI (0–0.9), the remainder of her rheumatologic workup was negative including ds-DNA, aldolase, SSA/Ro, and SSB/La.\nLN, GN's brother, presented for evaluation two weeks after birth for genetic testing due to the significant family history. The DHR oxidative burst was 6%, and he tested positive for the same pathogenic CYBB variant as his sister. The patient was started on prophylactic antimicrobial agents with trimethoprim/sulfamethoxazole and voriconazole, which was then changed to itraconazole, as well as interferon gamma-1b. The serum level of the antifungal prophylactic agent continued to be subtherapeutic despite loading and weight-appropriate dosing, likely secondary to medication noncompliance. Despite the early referral for HSCT and counseling, the parent declined to pursue this curative option before 12 months of age. LN's maternal grandmother requested to have genetic testing be done for her as well in the hopes that she can serve as his bone marrow donor. Interestingly, she was found to not have the CYBB mutation implying that the patient's mother had a de novo pathogenic CYBB mutation.\nThe patient had a complicated infectious history with several bouts of pubic cutaneous lesions that grew Klebsiella pneumonia, MDR Enterobacter asburiae, and Serratia marcescens. He had a bout of unilateral bacterial conjunctivitis that was positive for Serratia marcescens and Neisseria sicca/subflava. LN also experienced recurrent Clostridium difficile colitis. His pretransplant course was further complicated by ESBL Klebsiella pneumonia, Enterobacter cloacae sepsis, and Klebsiella bacteremia. He was started on hydroxyurea and azathioprine in preparation for transplant, but this regimen was discontinued due to Candida Parapsilosis bacteremia. He commenced cytoreduction therapy with busulfan, melphalan, fludarabine, and ATG and received a T-cell depleted unrelated 9/10 matched HSCT at 19 months of age. He obtained 100% donor chimerism with a normalized oxidative burst and discharged with outpatient follow-up.
A 42-year-old female presented to the outpatient cardiology clinic for symptoms of chest pain. The patient complained of increasing episodes of chest pain radiating towards her back and neck along with having episodes of palpitations, dizziness, intermittent dysphagia, and recurrent headaches with near syncope. Physical examination revealed a regular heart rhythm with no vascular bruits. An exercise stress test taken a year ago was normal. An echocardiogram was also normal. An electrocardiogram (EKG) revealed normal sinus rhythm and incomplete right bundle branch block. Several non invasive studies for work up of chest pain including exercise stress test and myocardial perfusion test were negative. She remained symptomatic with a growing concern of underlying coronary artery disease and was subsequently scheduled for a diagnostic cardiac catheterization to delineate her coronary anatomy.\nThe patient was prepped and draped on catheterization table in a sterile fashion and right radial artery was accessed under ultrasound guidance. After insertion of a 6 French slender radial sheath a 0.035 J tipped was advanced but it failed to advance in aortic arch and ascending aorta. Instead wire kept advancing easily in descending aorta. After multiple failed attempts we decided to perform a nonselective aortic arch injection (Figure ).\nInjection revealed an aberrant right subclavian artery originating from the descending aorta. Given significant tortuosity (Figure ) and risk of complications with further advancing the catheter, we contemplated to abort radial access and move to femoral access. However, we then meticulously used a 6 French Multi Purpose catheter, a 6 French Williams Right catheter , a 6 French Judkins Left 4.0, and a 6 French Judkins Right 4 catheters along with a long exchange length 0.035 J tipped wire to negotiate the anatomical variations and completed the selective coronary angiograms successfully (Figures -).\nOnce the anomalous anatomy was confirmed and no structural ischemic etiology was noted, the procedure was completed without any complications.\nThe patient later underwent a CT scan of chest with contrast which confirmed the anomalous anatomy that was also seen seen during catheterization (Figure ).\nThe patient's symptoms of atypical chest pain and intermittent dysphagia were thought to be related to this anomalous anatomy. The patient was offered both surgical evaluation and medical therapy with beta blockers, calcium channel blockers, and GI anti-spasmodics (hycosamine). She opted for medical therapy and her symptoms responded very well to medical management.
One week prior to her first visit to our clinic, the 13-year-old Caucasian girl noticed muscle twitching in her right (dominate) hand or a fluttering sensation. The muscle twitching resolved itself spontaneously after one hour. On the following day immediately before taking an exam at school, she experienced a severe tremor in her right hand which spread to her left hand and persisted. Immediately after leaving school, the girl was hospitalized at the neurology ward of a pediatric clinic where she underwent a complete medical check-up. After a thorough examination, no neurological or medical explanation for the symptoms was found. A physiotherapist asked her to "stop pretending", a notion that caused prolonged anger and sadness in her.\nOur patient agreed to be transferred to the child and adolescent psychiatric department. The tremor continued after admission and it could only be interrupted by either grasping an object in her hand or clenching her hand. She was not able at this time to either write or perform activities requiring fine motor skills (for example, cutting something with a knife). In order to fall asleep our patient had to either lie on her hands or hold an object in her hands.\nOur patient was attending the 7th grade in an academic secondary school and was in danger of failing academically. There was no history of psychiatric, neurologic or other severe somatic disorder in her past. She lived with her parents, who had been fighting regularly for years. The question of a separation was an issue between them, but they reported never having talked about it openly with their daughter.\nOur patient was appropriately developed for her age. Her cardio-pulmonary status and the results of examinations of her head, neck, abdomen, skin and genitals were all normal.\nThe finger to nose test, motor proficiency tests, pupillary reactions and eye movement tests were all normal. Her reflex status showed normal tonicity for both sides of her body, no meningism was present, gross strength was normal and sensitivity was normal for both sides of her body. During the examination the tremor ceased. At rest a low frequency hand tremor could be observed (approximately 5/s) in both hands. By the end of the physical examination the tremor changed from a low frequency tremor to a diadochokinetic tremor (Additional file ).\nBlood tests revealed no abnormalities, including thyroid stimulating hormone. Ceruloplasmin, serum copper and urine copper levels were taken and an ophthalmological evaluation was performed to rule out Wilson's disease. The results of cranial magnetic resonance imaging and an electroencephalogram were normal, as were results of a cerebrospinal fluid abdominal ultrasound.\nPsychiatric diagnostic testing included German versions of the Anxiety Questionnaire for Children, which showed a high tendency toward social desirability and performance anxiety (schoolwork). Our patient showed above average intelligence (HAWIK-IV, German version of WISC: IQ = 124). A scale to measure depression in children showed no marked symptoms of depression. For observation purposes and for a more precise diagnosis, the patient was filmed.\nOur patient's symptoms were noticeably lessened when her attention was on something else, for example on mental arithmetic. Concentrated on her tremor resulted in it becoming intensified. During her examination, the tremor fluctuated greatly. An entrainment test [] showed that when our patient tapped a rhythm with her contra-lateral hand, the tremor matched this new rhythm and at times ceased altogether.\nOur patient completed a comprehensive medical-behavioral therapy which included individual and group therapy sessions (client-centered in orientation) and anxiety-reducing techniques, especially in the area of performance anxiety. She also learned progressive muscle relaxation techniques (PMR).\nOne important aspect of the treatment was to work with our patient to help her understand what triggered her tremors and kept them from diminishing. In particular, her performance anxiety and her avoidance of challenges relating to school can be seen as factors involved in the disorder. Every day, our patient trained to write for about a quarter of an hour. At the beginning of her stay in our clinic, her motivation to go back to school or to write on her own was very low (Additional file ).\nA customized wrist brace was adapted to her hand, making it easier for her to write (Figure ).\nFrom this time she was sent to school regularly and had to participate in every exam. Our patient participated in private lessons because of great difficulties in certain school subjects, which helped her to overcome her anxiety concerning school. The tremor intensity was strongly related to her individual stress level and parental conflicts. By the end of the period of hospitalization, our patient's tremor was still present, but noticeably reduced in degree (reduction of 80%). Despite the presence of a slight tremor, the girl was able to write with the aid of the customized wrist brace (Additional file ).\nAfter completion of the therapy, our patient described what had helped her most in dealing with the symptoms of her tremor. First was to become more physically active rather than retiring alone to her room to listen to music. In doing so, she was able to cope with her symptoms both on a physical and on a social (interpersonal) level as she had been taught in therapy. Secondly, identifying and relating her feelings to others. Thirdly, learning and practicing a relaxation method to reduce her stress, in this case PMR, which had initially proven difficult for her, but which she was able to master in the end. Finally, attending regular physical therapy sessions helped in ameliorating her symptoms.\nOur patient had her first check-up two weeks after being discharged from our clinic, after a total duration of in-patient hospitalization of three months. At this time the tremor had ceased altogether and reappeared only about once every two months in extremely stressful situations. She reported that her parents were still living together, but planned to split up. The total duration of her treatment was nine months (Additional file ).
A 39-year-old woman was scheduled to undergo split thickness skin grafts as part of a reconstructive program following extensive burns. She was burned in a house fire 2 years previously, when she suffered 93% total body surface area burns, with a burn index of 85. Her history was significant for having undergone 39 reconstructive operations over 2 years. Her neck was reconstructed using a graft from the latissimus dorsi, and both femoral veins were occluded due to multiple accesses and indwelling catheters. A subclavian venous catheter had been inserted once before under ultrasound guidance. Physical exam showed her weight was 49 kg, and her height was 155 cm. Most of her body was covered by hard contracted skin. There were no peripheral veins evident on inspection.\nTo obtain venous access for this operation, we searched for veins using a linear probe ultrasound device (6–15 MHz, SonoSite Edge, FUJIFILM SonoSite, Inc., Washington, USA) on the chest and arm. This revealed accessible veins, including the subclavian and axillary veins on the anterior chest, and the brachial vein of the right arm. An indwelling catheter in the right brachial vein was considered as the first choice to avoid possible complications of using the subclavian or axillary veins such as pneumothorax or hemothorax. The right brachial vein measured 3 mm by ultrasound imaging.\nThe right arm had restricted motion range due to extensive contractures. She was positioned in the right semilateral position to gain easy access to the medial side of the arm by supination and abduction. A pillow was placed behind her back to maintain this position. The skin of the arm was prepped with 1% chlorhexidine alcohol solution and covered with a sterile drape. The ultrasound probe was covered with a sterile plastic probe cover. The indwelling venous catheter was placed using sterile barrier precautions. Local anesthetic (5 mL of 1% lidocaine) was injected, and a 20G catheter-over-the needle (48 mm) was inserted. Ultrasound-guided venous catheterization was performed. To access the brachial vein, we used the short-axis out-of-plane approach to avoid mechanical complications of unanticipated artery or peripheral nerve injuries, and then used the long-axis in-plane approach to penetrate the anterior vein wall and cannulate the vein. After inserting the cannula, a guide wire was placed through the cannula using the modified Seldinger technique. We placed a single lumen polyurethane catheter (3 Fr., SMAC Plus, Nippon Covidien, Japan), a commercially available central venous catheter (there is no commercially available midline catheter in Japan). Successful insertion of the catheter was confirmed with ultrasound imaging (Fig. ). The catheter entered the vein 6 cm from the skin entry site (Fig. ). After placing the catheter, we checked venous flow using color Doppler imaging (Fig. ).\nSurgery was performed the following day with no adverse events. A split-thickness skin graft was harvested from the head, and grafted to the neck. The skin graft became infected several days later and the midline catheter was used for administration of antibiotics. A second skin graft was performed again postadmission day 16 and the catheter removed on postadmission day 24. She was discharged without complications.
A 56 year-old man was diagnosed with cancer of unknown primary, with metastases to the lung, liver, bone and brain. He received a full course of palliative whole-brain radiotherapy, and daily dexamethasone was initiated. The patient was recruited to the study post-radiotherapy, and a summary of the participant’s baseline assessment is provided in Table . His most common reported physical activity over the past week was climbing stairs within his home, in order to access his bedroom and bathroom on the top floor.\nThe participant was prescribed a daily walking plan of 5 minutes per day at low to moderate intensity, to progress up to a total of 30 minutes per day at the end of six weeks. All strength exercises were started at 1 set of 8 repetitions, slowly progressing up to 2 sets of 8 repetitions for most exercises. The patient was unable to progress beyond walking 10 minutes per day before experiencing severe fatigue. Modifications were made to the strength exercises, with adoption of seated positions where possible. The participant completed 16 out of the 18 prescribed strength exercise sessions, and experienced no adverse events over the course of the 6-week program.\nA summary of the participant’s post-intervention assessment is provided in Table . As monitored by the activPAL™ accelerometer, the average number of steps taken over the baseline week was 3714, with an average estimated total energy expenditure of 29.1 MET·hours; post intervention, the average number of steps taken during Week Six was 1471, with an average estimated total energy expenditure of 28.3 MET·hours. The majority of his steps were taken inside his home. It was noted that there was no change in dexamethasone dose over the course of the 6-week program. The patient reported significant total fatigue that likely impacted his endurance and mobility. The patient expressed high satisfaction with the physical activity program and identified one-on-one supervision of the strength training sessions as among its top advantages. The participant indicated that his least enjoyed program aspect was his decline in overall condition despite participating in the physical activity program. In follow-up, the participant passed away 77 days after completing the study.
We identified a 60-year-old male patient with an exceptional response to everolimus. He presented initially with gross hematuria and flank pain. CT and MRI demonstrated a large 12-cm mass replacing the left kidney with invasion of the left psoas muscle. There was no evidence of metastatic disease. He underwent radical nephrectomy 4 weeks after presentation. On pathologic examination, he was confirmed to have RCC with clear cell and papillary features, Fuhrman grade 3, with gross involvement of the renal vein. Eight lymph nodes were examined with no evidence of nodal metastases. He was treated with adjuvant sunitinib on a clinical trial. The patient was unable to tolerate therapy, and sunitinib was discontinued after 1 month.\nOne year after nephrectomy, surveillance imaging demonstrated new pulmonary and hepatic lesions, as well as abnormal tissue in the left nephrectomy bed and left adrenal gland. Liver biopsy confirmed metastatic RCC. The patient was started on pazopanib as first-line therapy for metastatic disease. He had mild toxicity but did not require dose reduction or interruption. Ten weeks after starting pazopanib, a mixed response was seen on imaging. Although his pulmonary metastases were stable to slightly improved, his disease had clearly progressed in the liver (Fig. ). The patient was started on everolimus at this time.\nDisease in the lung, liver, adrenal gland, and nephrectomy bed was significantly improved after 11 weeks of everolimus therapy. His lesions continued to regress at the 6- and 9-month time points and remained stable for 3 years (Fig. ). At that time, an MRI of the orbit was ordered to evaluate ptosis and proptosis of the right eye. A solitary site of metastatic progression was discovered in the right frontal sinus causing erosion of the calvarium with extension to the scalp, mass effect on the right frontal lobe, and extension to the right orbit and ethmoid sinus. The metastasis was surgically resected with pathologic examination confirming metastatic clear cell carcinoma. The resection was considered clinically complete, and no adjuvant radiation was recommended. Despite this recurrence, his other metastatic disease remained stable with only a sub-centimeter nodule in the lung and no disease detectable in the abdomen and pelvis. Given good tolerance and lack of progression elsewhere, everolimus therapy was continued.\nTwo years after resection, MRI was concerning for progression on the basis of several small areas of abnormal enhancement. He was treated with stereotactic radiosurgery to right nasal, right temporal, and right frontal dural lesions. There was no evidence of progression elsewhere, and he remained on everolimus. His disease remained stable for an additional 2 years. Imaging at that time demonstrated multiple lesions in the liver and spleen, as well as osseous metastatic disease. Everolimus therapy was discontinued, and the patient was started on nivolumab and denosumab. In total, the patient remained on everolimus for >7 years.\nTo identify potential molecular mechanisms for the extended benefit in response to everolimus, we performed WES on DNA from the primary (pretreatment) and metastatic tumor obtained at the time of surgical resection. The coding regions from approximately 25,000 genes were sequenced, with 90% of exons achieving 20x coverage or greater. Analysis of mTOR pathway genes revealed variants in several genes associated with mTOR signaling (Table ). The variants identified are present in the dbSNP (Single Nucleotide Polymorphism Database); however, only the PIK3C2G (P146L) mutation was found in the COSMIC (Catalogue of Somatic Mutations in Cancer) database. The alterations identified include 2 phosphoinositide 3-kinases (PI3Ks) that are involved in regulating Akt activation and missense mutations in PIK3R2 and PIK3C2G []. We also identified a missense mutation in diacylglycerol kinase zeta (DGKZ), encoding an enzyme that functions in mTORC1 activation []. In addition, we identified an alteration in protein kinase D (PRKD3) that activates Akt through diacylglycerol signaling []. A missense mutation was also detected in the insulin receptor (INSR) gene which encodes a receptor critical in activation of PI3K/Akt signaling and providing feedback regulation of mTORC1. Finally, we identified a missense mutation in eukaryotic translation initiation factor 3 (EIF3B) encoding an essential scaffolding component of the EIF3 complex activated by mTORC1 and functions in regulating protein translation [].\nTo identify a potential genomic driver of disease recurrence in this patient, we compared alterations discovered in the primary tumor to those found in the metastatic lesion from the right frontal sinus obtained at the time of recurrence. Our analysis focused on alterations in mTOR pathway genes as a potential mechanism of everolimus resistance. Unique to the metastatic tumor are mutations in INSR, PIK3R2, and DGKZ genes, all of which are involved in either PI3K/Akt signaling or mTORC1 activation. Common between both the primary and metastatic tumor was the EIF3C missense mutation, suggesting a clonal mutation, occurring early in the evolution of the tumor.\nIn addition to mTOR pathway gene analysis, we also examined the sequencing data for genes commonly altered in RCC. In both the primary and metastatic tumors, we identified nonsynonymous alterations in VHL, PBRM1, KDM3A, KDM5C, and ARID4A (Table ). The alterations identified in VHL and PBRM1 have been previously characterized as inactivating, loss-of-function mutations in RCC []. KDM3A, KDM5C, and ARID4A are involved in chromatin remodeling and are frequently altered in RCC [].
The patient was a 55-year-old woman hospitalized in the Department of Oncology, the 309 Hospital of the Chinese PLA on December 23, 2009 for 4 years of chest pain which was further aggravated for 8 months. Previously, she had paroxysmal dull pain in the chest for 4 years without obvious incentives; unfortunately, no attention was paid to it. The chest pain aggravated since April, 2009, accompanied by cough, white phlegm, hoarse voice, and cough after drinking water. No fever, night sweats, or weight loss was observed. Chest computed tomography (CT) done in another hospital in April, 2009 showed a mass localized in the superior mediastinum with a maximum diameter of 4.3 cm. Routine blood test, erythrocyte sedimentation rate, biochemical test, and tumor marker test were normal, as was abdominal ultrasonography. Thoracic operation was conducted, during which no clear boundary between the mass and surrounding blood vessels was found. As a result, the mass could not be excised and only an incisional biopsy was performed. Postoperative pathologic examination showed reactive hyperplasia of lymph nodes accompanied by charcoal particle deposit. The patient was clinically suspected to have mediastinal tuberculosis lymphadenitis and was given an anti-tuberculosis treatment for almost half a year. No obvious relief occurred and the patient suffered from somasthenia with a weight loss of about 10 kg in 6 months. A chest CT performed in November, 2009 found no obvious change in the superior mediastinal mass ().\nThe patient came to the 309 Hospital of the Chinese PLA for further treatment. Results of the tuberculin (PPD) test and MycoDot TM test were both negative. As a result, the Department of Tuberculosis excluded the possibility of tuberculosis and discontinued anti-tuberculosis treatment. The pathologic specimen of the thoracic mass incised in April 2009 was consulted by Departments of Pathology from several hospitals including the Peking Union Medical College Hospital. In the pathologic specimen, there was obvious diffuse infiltration of lymphocytes with blood vessels proliferated and infiltrated into the follicles; immunohistochemical assay for CD20 detected positive staining on the cell capsule inside follicles (). Lymph node hyperplasia was then considered, supporting the diagnosis of Castleman's disease. A whole body positron emission tomography (PET)-CT scan was performed on October 30, 2009, showing a soft tissue mass in the superior mediastinum with the maximal diameter of 4.4 cm. The fluorodeoxyglucose (FDG) uptake was slight and the maximum standardized uptake value (SUV) was 2.9. No other abnormality was found. In combination with the medical history, giant lymph node hyperplasia was considered ().\nThe patient underwent hormone therapy for 2 months with no obvious response, then she was transferred to the Department of Radiotherapy in December, 2009. Three-field intensity - modulated radiotherapy (IMRT) was delivered to the mediastinal mass since December 23, 2009. After radiotherapy of 30 Gy by 15 fractions, symptoms such as chest pain, hoarse voice, and cough after drinking water were markedly relieved, but the chest CT scan showed no obvious shrinkage of the mass. Radiotherapy was continued, and the total dose was increased to 60 Gy by 30 fractions, until February 4, 2010. A chest enhanced CT scan performed on May, 2010 showed that the mass was significantly smaller than before, indicating partial remission (). Upon follow-up until December 2010, the patient had no discomfort.
A 44-year-old woman with a history of antiphospholipid antibody syndrome (coumarin user) presented with traumatic brain injury after falling to the ground. Upon admission, she scored 12 points on the Glasgow Coma Scale, and a CT scan depicted a massive left frontotemporal subdural hematoma and brain midline shift. The patient underwent a craniotomy to remove the clot and was discharged 2 weeks later. She was conscious and oriented, and exhibited no motor deficit except for a slight difficulty in speaking. Four weeks later, she developed a wound infection. She was treated with antibiotics and the affected bone had to be removed.\nThe patient was reassessed 8 months later. The bone defect was 8.2 cm × 5.3 cm in size, and the scalp had sunk 2.2 cm from the surface of the skull. A CT scan showed no shift of brain structures, but sulci were enfaced next to the bone defect (). The patient presented with headache, tinnitus, dizziness with head movements, incision discomfort, and difficulty in speech. There were no motor deficits, and she received 29 points on the MMSE. The patient scored 2 points on the mRS (ranging 0–6) and 100 points on the BI (ranging 0–100). The patient underwent cranioplasty with methyl methacrylate and was discharged uneventfully on the second postoperative day.\nSix months after surgery, the patient reported complete resolution of symptoms, without changes in motor examination, and a subjective report of improvement in verbal fluency. Neuropsychological assessment before and after cranioplasty showed improvement in memory capacity, language, executive functions, and activities of daily living (). Postoperative MMSE showed a slight variation (down 1 point). No changes were observed in BI and mRS.\nRegarding the cerebral hemodynamic changes, postoperative TCD showed a 20% and 16% increase in the cerebral blood flow velocity in the middle cerebral artery in the supine and the sitting positions, respectively. There was no significant increment of CBF velocity on the contralateral middle cerebral artery.\nPerfusion CT study revealed an increase in CBF: 18 to 58 mL/100 g/minute ipsilateral to the cranioplasty and 19 to 70 mL/100 g/minute on the contralateral side. Interestingly, there was a reduction in the mean transit time on both sides: 14.7 to 5.2 seconds (ipsilateral to the cranioplasty) and 13.6 to 4.8 seconds (contralateral side). The increment in cerebral blood volume was less evident: 4.03 to 5.11 mL/100 g (ipsilateral to the cranioplasty) and from 4.34 to 5.46 mL/100 g (contralateral side). The values of blood pressure and heart rate were similar at the time of the tests ().
Our patient, a 33-year-old woman, presented to our hospital with a right lower abdominal mass 13 months previously. Two months later, the mass was surgically removed. Examination secured a diagnosis of stage I adenoid cystic carcinoma. One month later, the patient visited a medical oncology department for consultation regarding their future treatment plan. Radiological examination revealed no cancer metastasis. However, the doctors were not able to obtain sufficient information about the possible future progression of the malignancy, which increased the patient’s anxiety. The patient did not have any mental illnesses such as depression and schizophrenia, nor did she have any history of such illnesses. As her anxiety did not improve, she was prescribed lorazepam at an oral daily dose of 1.5 mg. However, her anxiety did not improve, and she began to experience sleep disorders, which seriously affected her subjective quality of life and overall daily function. One month later, she presented to our outpatient clinic for consultation concerning her psychological symptoms. At the first visit, she was tearful, stating “I’m not sure what to do. I understand that I don’t know much about adenoid cystic carcinoma, but I keep getting anxious.” She had lost 6 kg over the prior four weeks owing to anxiety-related appetite loss. She worried excessively about her future, and had fallen into a negative thinking cycle owing to a fear of death. Her immediate goal was to complete a medical examination to obtain a second opinion about her adenoid cystic carcinoma from a specialty hospital, hoping that expert medical advice would relieve her anxiety. However, because her anxiety affected her daily life, she was unable to travel far to visit a hospital. Because lorazepam had no effect, we decided to taper off this drug. We wanted to use an SSRI as the next treatment, but this drug class can possibly induce serotonin syndrome. Therefore, we prescribed 12.5 mg of quetiapine orally as required. To reduce the adverse effects of quetiapine (e.g., cerebrovascular events, neuroleptic malignant syndrome, metabolic changes, suicidal thoughts and behaviors), we titrated the dose down to a slightly safer amount. On a return visit seven days later, the patient was prescribed quetiapine three times a week. At this dosage, her sleep increased to approximately 6 h per night, and she had no obvious side effects. However, she reported that “Focusing on my housework or parenting helps keep my mind off adenoid cystic carcinoma. Out of nowhere, I’ll suddenly remember adenoid cystic carcinoma.” We told her that she did not necessarily need to stop thinking negatively to overcome her anxiety. On a return visit 14 days later, the patient said “I was so anxious at the thought of having to receive long-term medical examinations for 5 or 10 or more years. But I gradually began to think that at the moment I may be all right for 5 or 10 years.” Over the course of several consultations, she gradually became able to lead her daily life despite concerns about adenoid cystic carcinoma. The patient’s psychological symptoms stabilized to the point that she only required treatment with quetiapine a few times a month. Two months later, she obtained her desired second opinion, and she is currently being treated by that institution.
Our patient was an 11-year-old Japanese boy (height 141 cm, weight 36.5 kg) with acute lymphocytic leukemia. Since the onset of acute lymphocytic leukemia, he had received early-stage intensive chemotherapy, remission therapy, and maintenance therapy; however, because he had a positive test result for minor breakpoint cluster region, umbilical cord blood transplantation was performed. After transplantation, he complained of abdominal and articular pain; his abdominal pain was accompanied by frequent diarrhea. These were considered to represent gastrointestinal symptoms of graft-versus-host disease (GVHD); thus, opioid therapy was initiated. For long-term opioid therapy, few opioid medications provide information on the label regarding the safety and effectiveness of the drug in pediatric patients []. We selected fentanyl because chemotherapy for leukemia predisposes the patient to renal dysfunction, and it is considered effective because it has high selectivity for mu 1 receptors in the treatment of mucosal pain [, ]. However, the dose of fentanyl was gradually increased for pain management, resulting in dose as high as 2300 μg/day, which required intervention from the palliative care team.\nAt the time of intervention, the patient was isolated in a sterilized room after transplantation. In addition to the major complaint of pain in the lower left abdomen, upon palpation, the patient complained of pressure pain throughout the abdomen; he also complained of joint pain in the legs when the abdominal pain intensified. Computed tomography revealed changes showing pancreatitis and mild intestinal edema, which was considered to be a sign of GVHD (Fig. ).\nGiven the intense acute pain caused by GVHD, the fentanyl dose was increased again to 2700 μg/day; however, the frequency of the rescue dose for pain (equivalent to 1-h dose of continuous infusion) did not decrease below 15 times per day, and continuously increasing the dose did not reduce the frequency of the rescue dose. After the opioid was switched to 90 mg/day of oxycodone injections, the patient continued to experience pain, and there was no change in the frequency of oxycodone rescue doses (Table ).\nThe patient’s general condition improved, and he did not require isolation. Despite attending school in the hospital, there was no improvement in his complaints of pain, and just before entering junior high school, he expressed anxiety about friends, learning, and whether he would be understood by the teachers. Considering the possibility of opioid overdose in response to complaints of nausea and fatigue, dose reduction was planned; however, he exhibited strong resistance. Furthermore, he became irritable, and his mental instability became evident as exhibited by violent outbursts.\nComputed tomography revealed no findings that caused physical pain. His pain was considered to have alleviated; thus, health professionals involved in his care (that is, pediatrician, pediatric psychiatrist, palliative care team, ward nurse, child medical care support provider, and childcare worker) examined the possibility of mental anxiety resulting in the lowering of pain threshold and the possibility of chemical coping.\nExpecting to use less opioid, we initiated duloxetine, which exerts an antidepressive effect and adjuvant analgesic effect, at a dose of 10 mg/day. Furthermore, to address the patient’s mental anxiety, a meeting was held with the teacher whose class the patient was expected to attend. The new school staff cooperated so that the patient could attend the same class as his good friends. To address the patient’s drug use, upon suspicion that the sudden increase in blood concentration due to administration of rescue doses of opioid injections could have caused chemical coping, switching to oral opioids was attempted; however, on a pain scale (scale of 0 to 5), the patient assessed that the rescue doses of intravenous oxycodone had reduced his pain from 5 to 1.2 points, whereas the oral oxycodone immediate-release preparation had only reduced pain from 5 to 4.5 points; thus, switching to oral drugs was not successful.\nWe believed that the rescue dose of intravenous oxycodone resulted in a sudden increase in blood concentration, and the administration of the rescue dose could have been a coping behavior. An explanation regarding opioids in general and the possibility that the number of rescues will not decrease for purposes other than analgesia (such as antianxiety) was shared with the patient and his family members who provided the consent; thereafter, we decided to lower the concentration of intravenous oxycodone without informing the patient of the timing of dose reduction.\nAfter approximately 1 month, the intravenous oxycodone dose was gradually reduced to 3 mg/day; however, there was no major change in the frequency of rescue doses. After the patient was informed that the intravenous oxycodone had been reduced to a dose that had been ineffective as an analgesic, we prescribed 200 mg of acetaminophen and 100 mg of ibuprofen to be taken as needed. Subsequently, oxycodone infusion was discontinued, and the patient did not complain of pain. Thereafter, he expressed no desire for opioid use and was discharged. Currently, he is being treated on an outpatient basis and is opioid-free.
The patient was an 86-year-old woman who suffered neoplasia on the right cheek and right frontotemporal area for 2 years. The patient had a history of lacunar infarction, cerebral insufficiency, hypertension, and diabetes. The facial neoplasm grew slowly for 2 years and accelerated in the last month before she was seen, with ulceration and crusting. The size of the neoplasia was 4.0 cm × 4.0 cm, 3.0 cm × 3.0 cm and 2.5 cm × 3.0 cm (Fig. ).\nEnhanced CT and MRI of the maxillofacial region were performed preoperatively. The surgery area was simulated using 3D image technology for the virtual surgery design and operation to be implemented. A safe resection range of 1.5 cm on the outer edge of the lesion was designed. One day before the surgery, the shape and range of the perforating branches were determined by Doppler ultrasound. The size and shape of the SCAIFP were designed to be elliptical and 15 cm × 7 cm in size.\nThe maxillofacial tumour extended resection and supraclavicular flap repair operation was performed. The neoplasia was completely resected, and the facial nerve was preserved. The defect was approximately 18.5 cm × 7.5 cm. Pathological analysis of the frozen tissue showed low-medium differentiated squamous cell carcinoma with negative margins. Based on the position of the transverse carotid artery, the final flap size was 19.3 cm × 8.3 cm, which was larger than the initial flap design (15 cm × 7 cm). The larger size was a result of the defect being larger than initially expected and due to flap tension (Fig. ). On the surface of the deltoid muscle, the flap was dissected from distal to proximal, and the superficial layer of the deep cervical fascia was reserved. The transverse carotid artery was cut off and ligated to enter the deep branches of the trapezius muscle to extend the reach. We used fluoroscopy to identify and protect the perforating branches. After the flap was harvested, a warm saline gauze was used for cover and protection, and the blood supply of the distal end of the flap was evaluated.\nNo complications were observed after the reconstruction. The flap survived entirely, and the donor area healed well. The patient was discharged on postoperative day 7 (Fig. ), and at the 10-month follow-up coverage was stable with no tumour recurrence and the scar from the incision was natural. Upper limb abduction was 90° without dysfunction. Facial nerve function was well preserved, and facial expressions were natural and satisfactory for the patient (Fig. ).
In July 2018, a 63-year-old male patient received an emergency referral to our department from his primary care physician; on the previous day, the patient had noticed a feeling of numbness of the left lower lip and chin while eating his breakfast. The patient compared the sensation to that after being given anesthetic by the dentist. He reported that when getting up that morning, he had already experienced a feeling of uncertainty and a slightly unsteady gait, but that these had completely cleared after a short time. The patient’s medical history revealed Lyme disease with Bannwarth syndrome in 2017, a pulmonary embolism in 2017, a right vestibular neuropathy in 1998, arterial hypertension, nicotine consumption of 80 pack years, as well as a tonsillectomy in 1982. With regard to medications, the patient took 2.5 mg/d of ramipril for the treatment of arterial hypertension. The neurological examination revealed an approximately 2 × 3 cm area of hypoesthesia in the area of the chin and left lower lip. The patient’s sense of taste and smell were intact and the nerve exits were not painful on palpation. Otherwise, the cranial nerve status presented as normal and the muscles were normal in tone and eutrophic without paresis. The muscle reflexes were symmetrical and in the lower half of the normal range. The sensitivity to touch was normal, and coordination was intact. The cranial MRI revealed an acute ischemia in the right thalamus. Over the further course of the inpatient stay, further cerebro- and cardiovascular diagnostics were conducted with Duplex sonography of the brain-supplying arteries, long-term ECG and blood pressure monitoring, laboratory diagnostics, and transesophageal echocardiography. These revealed an increased risk of arteriosclerosis without indications of an embolic etiology of the ischemia. The patient was put on 100 mg of acetylsalicylic acid for cerebrovascular secondary prevention, a cholesterol-lowering treatment with 20 mg of simvastatin was initiated, and the patient was urgently advised to refrain from further nicotine consumption.
A 16-year-old Indian girl presented to our hospital with swelling on the scalp. She was born with a black-pigmented triangular patch over the scalp covered with hair as well as multiple black hairy patches on her extremities, back, and most of her anterior trunk. The patch on the scalp had increased in size over a period of eight years after birth. Within the past three months, swelling had progressed from the hairline to just above the left brow, leading to unilateral mechanical ptosis. Upon applying pressure, there was a scanty, yellowish, non-foul-smelling discharge that was occasionally blood-stained and was associated with intense itching without any pain. Her sleep, appetite, bowel, and bladder were unaltered. Her menarche had begun at the age of 15 years, and her menstrual cycle was normal and regular. Her birth and developmental history were normal. The family history was negative for congenital nevi or melanoma.\nThe patient's clinical examination revealed her to be afebrile, with a pulse rate of 64 beats/minute, a respiratory rate of 16 breaths/minute, and blood pressure of 120/70 mmHg. The examination of her cardiovascular system was normal. Her cranial nerve functions were normal, and no neurological deficit was found in the limbs. The plantar response in both the limbs was flexor. There was no evidence of meningeal irritation.\nA local examination revealed a single swelling of 10 cm × 8 cm on the left side of the scalp. The swelling extended anteroposteriorly from 3 cm in front of the left parietal eminence to just above the lateral part of the left supraorbital ridge. The transverse extension was from the line passing through the left parietal eminence to the right of the midline (Figure ). Her local temperature was not raised, and tenderness was absent. The surface was irregular, rough with multiple pits, and the margins were irregular with rounded edges. The swelling was firm in consistency and mobile. The anterior part of the swelling overlying the forehead could be lifted. The underlying skin appeared normal. It was non-pulsatile with no evidence of impulse on coughing. The regional lymph nodes were not palpable. Numerous hairy nevi were present over the scalp, trunk, and limbs.\nThe patient was referred to the Department of Neuro-ophthalmology and Otolaryngology for further assessment. Her fundoscopy and visual evoked potentials were normal. Her otorhinolaryngological evaluations were normal.\nRoutine blood investigations showed that her hemoglobin level was 11.6% and her total leukocyte count was 4200/mm3 with relative neutrophilia (80%). Her serum urea and electrolyte levels were normal. A lumbar puncture revealed clear cerebrospinal fluid with a normal cell count and biochemistry and no growth on culture. Her liver and kidney function tests were within normal limits. Her electrocardiogram did not show any abnormal features.\nAn X-ray of her skull (posteroanterior view) revealed a soft tissue swelling in the left temporofrontoparietal region. Ultrasonography of the swelling showed a large, homogeneous, hypoechoic, solid 9.4 cm × 6.8 cm × 1 cm lesion in her scalp in the left frontal region and partially extending into the parietal region on the left side. The underlying outer table of the skull and diploic spaces were normal with few hyper-reflective areas scattered throughout the lesion and distal shadowing raising clinical suspicions of tiny calcified foci. The interface between the lesion and the scalp was obscured. No significant color flow was noted on a color Doppler ultrasonogram. These findings were highly suggestive of a possible angiofibroma. Power Doppler ultrasonography showed a highly vascularized lesion.\nMRI of her brain (both plain and contrast images) was done by using a multi-echo, multi-planar technique, which showed the presence of a 8.99 cm × 2.26 cm abnormal signal intensity involving the scalp (subcutaneous plane within fat) in the left frontoparietal region. The signal intensity appeared isointense to hyperintense on T1-weighted MRI scans with few small tubers with cortical dysplasia in the left frontoparietal region and asymmetric dilatation of the left ventricle that were hypointense on T2-weighted MRI and fluid attenuated inversion recovery (FLAIR) images. Few foci were seen within the lesion, which appeared hyperintense on T2-weighted MRI and FLAIR images. The MRI impression revealed multiple intra-cranial lesions, which included a benign, homogeneously enhancing, subcutaneous scalp lesion in left frontoparietal region suggestive of angiofibroma; a few calcified subependymal nodules within the body of the lateral ventricles on the right side; a few small tubers with cortical dysplasia in the left frontoparietal region; and left maxillary sinusitis (Figure ). These radiological findings were highly suggestive of tuberous sclerosis. Magnetic resonance angiography revealed the absence of involvement of any underlying blood vessels.\nMicroscopic examination of the patient's scalp swelling revealed the presence of lining keratinized, stratified squamous epithelium and underlying dermis. The epidermis was thinned out with loss of rete pegs. Her dermis showed lobules and nests of nevi cells, hair follicles, sweat glands, and sebaceous glands. There was diffuse deposition of melanin pigment and the presence of melanocytes around and within the hair follicles and sebaceous glands extending up to deep subcutis and infiltrating the fat (Figure ). All of these findings were suggestive of congenital melanocytic nevi.\nOn correlating these imaging findings with the patient's clinical symptoms, surgery was considered. Excision with primary skin grafting was done (Figure ). The graft was taken from the medial aspect of her right thigh.
We report the case of a 70-year-old Caucasian man, with no significant clinical background, who presented to our casualty department with a three-week history of headache and maxillary pain on the right side, together with the recent appearance of dysphonia and dysphagia for solids. No urinary disorder was reported. A neurological examination revealed a paresis of cranial nerves IX and X, manifested by the displacement of the soft palate to the right and difficulty swallowing. Paresis of cranial nerves XI and XII was also observed, indicated by the lowering of the right shoulder and hypotonia of the right trapezius muscle, and was accompanied by displacement of the tongue toward the right, right-side hypotonia and muscle twitching (Figures and ). Results of the rest of the physical examination were normal.\nBlood analysis results revealed an alkaline phosphatase level of 350 UI/L (normal range 44 to 147 UI/L) but no other significant alterations. In view of the paresis of the four lower cranial nerves, a CT scan of the larynx and neck was performed; the CT scan revealed an asymmetrical union between the clivus and the right occipital condyle, adjacent to the jugular foramen, with increased ground-glass bone density. There was no visible lesion to the bone cortex or soft tissues. This study was complemented with an MRI scan, which revealed a bone lesion producing a hypointense signal at sequences T1 and T2. Administration of a gadolinium contrast agent produced a moderate degree of enhancement at the base of the skull, affecting the right occipital condyle and part of the right side of the basilar bone (Figure ). The image corresponded to a moderately space-occupying blastic lesion, visible in the CT scan, which slightly decreased the caliber of the jugular foramen and the condylar canal. Therefore, fibrous dysplasia, Paget's disease and metastasis were considered in the differential diagnosis.\nSubsequently, an additional radiographic examination of the lumbar column and pelvis was carried out; this examination did not reveal any lesions suggestive of Paget's disease. A bone gammagraphy was then requested, and images showed multiple pathological foci of tracer uptake in the right maxilla, the rib cage, right scapula, spine and pelvis. These foci were compatible with disseminated bone metastases. The blood analysis was repeated, and tumor markers were studied. The initial prostate-specific antigen (PSA) value was 21.30 ng/mL.\nA physical examination revealed an enlarged prostate with a hard consistency, destructured in the left lobe. Because there was a strong suspicion of prostate neoplasm, a biopsy was performed. The anatomic pathology findings were bilateral common adenocarcinoma, with a Gleason grade of 8 (4+4), affecting 60% of the tissue. There was no presence in the periprostatic adipose tissue and no perineural infiltration.\nFollowing the diagnosis of stage IV prostate adenocarcinoma by metastatic bone dissemination with Collet-Sicard or jugular foramen syndrome, hormone treatment was begun with an antiandrogen. Then, 15 days later, a luteinizing hormone-releasing hormone (LHRH) analog was added and a monthly dose of zoledronic acid was subsequently included. The PSA value during the diagnostic process, prior to the start of antiandrogen therapy, was 71.9 ng/mL.\nAfter three months of treatment, our patient was able to swallow normally, but the dysphonia remained. The rightward displacement of the palate, the lowering of the right shoulder and the atony of the right side of the tongue (paresis of cranial nerves XI and XII) remained unaltered. The latest PSA value was 0.11 ng/mL.
A 53-year-old gravida 1 woman was referred to our department with a tumor in her groin. Her previous medical history consisted of endometriosis treated by surgical cautery at the age of 38 years, abdominal myomectomy for uterine leiomyoma at 39 years, and a cesarean delivery at 41 years. Although the patient entered menopause at the age of 49 years, she did not have any discomfort or pain in the inguinal region associated with menstruation before menopause. She discovered a tumor in her right groin approximately 3 years before her hospital visit. She went to a private gynecological clinic because the tumor had gradually increased in size and become painful. The gynecologist suspected a soft tissue tumor arising from muscle or adipose tissue and referred her to an orthopedist in our hospital. Surgical biopsy was performed, and the findings obtained indicated that the tumor consisted of a papillary component and was suspected of being serous adenocarcinoma based on hematoxylin and eosin (HE) staining. The tumor appeared to be adenocarcinoma, similar to metastatic ovarian cancer, and not a soft tissue tumor. The patient was referred to our department.\nThe tumor was palpable in the groin and had poor mobility. An ultrasound indicated that the tumor was as large as 5 cm in diameter. Magnetic resonance imaging (MRI) revealed a multilobular tumor in the right inguinal region that was 55 mm in diameter and consisted of cystic and solid components (). 18F-Fluorodeoxyglucose-positron emission tomography/computed tomography (FDG-PET/CT) showed the accumulation of FDG at the tumor and right external and obturator lymph nodes, which was attributed to metastasis of the malignant tumor (). MRI also indicated uterine leiomyoma with calcification and a right ovarian cystic tumor that appeared to be benign endometrioma because a solid part was not found in the cyst. No other lesion involving peritoneal dissemination or distant metastasis was detected. Serum CA125: 21 U/ml, CEA: 1.0 U/ml, and CA19-9: 6 U/ml were not elevated. Serum FSH: 66.2 mIU/ml and estradiol <10 pg/ml were consistent with a menopausal patient.\nSince endometrioma was detected in the right ovary, the tumor was located in the groin, one of the sites of extrapelvic endometriosis, and the pathological findings of a biopsy specimen suggested serous adenocarcinoma or endometrioid adenocarcinoma, which is closely related to endometriosis, the tumor was suspected to be endometriosis-associated adenocarcinoma arising from endometriosis in the groin. Distant metastasis was not detected, and, thus, surgical resection of the tumor and lymph nodes was performed.\nAbdominal hysterectomy, bilateral adnexectomy, and pelvic lymphadenectomy were initially performed, followed by a skin incision to the right lower abdomen. Skin and adipose tissue around the tumor were divided and separated as much as possible. The tumor including a part of the abdominal rectus muscle and rectus sheath, subcutaneous fat, skin, and the right inguinal ligament was resected en bloc with a 1.5 cm surgical excision margin. A skin incision to the right femur was performed and a skin pedicle flap was constructed. The lower abdominal wall with skin and fascia defects was reconstructed with a fascia lata tensor muscle skin flap ().\nThe resected tumor was 11.5 × 9.5 × 6 cm and section surface was white. The tumor infiltrated fat tissue under the skin, inguinal ligament, and abdominal rectus ().\nMacroscopic findings showed that the tumor mainly grew in the cystic component and partially expanded outside of the cyst. Erosion, bleeding, and the accumulation of hemosiderin were observed in the cyst wall, while a small spindle cell-like endometrial stroma was detected below the cyst wall. Immunohistostaining revealed that estrogen receptors (ER) were positive in the epithelium and stroma of the cyst wall, while CD10 was positive in the stroma, suggesting that the cyst was an endometriotic cyst.\nThe tumor showed papillary, tubular, and cribriform growth in the fibrovascular stroma of the cyst. Tumor cells had a clear and eosinophilic cytoplasm with a round-shaped nucleus with nuclear atypia. Immunohistostaining showed that hepatocyte nuclear factor-1beta (HNF-1β) was positive, p53 was negative, ER was negative, and progesterone receptor (PgR) was focally positive, indicating that most of the tumor presented the features of clear cell adenocarcinoma ().\nThe tumor also invaded outside the cyst, in which an alveolar tumor with high-grade nuclear atypia grew in vesicular and sheet forms. Immunohistostaining of the tumor at this site showed that HNF-1β was negative, p53 was positive, ER was partially positive, and PgR was negative, indicating poorly differentiated adenocarcinoma. The tumor also involved 8 out of the 61 resected lymph nodes. Although endometriosis was detected in the right ovary and broad ligament of the uterus, no other malignant lesions were found in the abdominal cavity. Furthermore, the cytology of ascites was negative.\nBased on the pathological findings of (1) benign endometriotic cysts in the cystic lesion, (2) endometrial stroma around the cystic lesion, (3) transition from benign to malignant features in the epithelium of the cyst wall, and (4) no other primary lesion, the tumor was consistent with clear cell adenocarcinoma arising from extrapelvic endometriosis\nAfter the surgical wound had healed, we recommended chemotherapy with carboplatin and paclitaxel, which are commonly used in the treatment of ovarian cancer. The patient agreed with our proposal, but developed severe hypersensitivity to paclitaxel; therefore, gemcitabine and carboplatin were alternatively prescribed for 6 cycles. Although the patient developed right leg edema, she remained healthy and had no evidence of recurrence 20 months after the treatment.
The patient was a 60-year-old man. Abdominal ultrasonography indicated that he had an abdominal mass. He reported having perceived lower abdominal pain 7 months before the initial examination, but it spontaneously remitted. The patient had no fever nor any history of conditions such as hypertension. A blood test revealed that he did not have anemia, thrombocytopenia, or coagulation abnormalities. The patient demonstrated slightly elevated levels of carcinoembryonic antigen (4.7 ng/ml) and soluble interleukin-2 receptor (603 U/ml), tumor markers that are elevated in gastrointestinal cancers and malignant lymphoma. Contrast-enhanced CT showed a 33× 31 mm abdominal aortic aneurysm and a 106 × 81 mm mass peripheral to the abdominal aorta. The mass did not feature a contrast effect, and its margin was enhanced in the venous phase (Fig. ). Sigmoid wall thickening and inferior vena cava thrombosis were also observed (Fig. ). We suspected the patient had a neoplastic disease, such as a malignant lymphoma or sigmoid colon cancer. Detailed examinations were consequently performed, and direct oral anticoagulant therapy was initiated to treat the inferior vena cava thrombosis.\nThe FDG-PET revealed an abnormal accumulation of FDG in the margin of the mass and the sigmoid colon, but not in any other organs. Colonoscopy showed only mild inflammation in the sigmoid colon, and a sigmoid colon biopsy revealed no significant findings. Chronic inflamed fibrous connective tissue was harvested from the periaortic mass using CT-guided biopsy. There were no findings indicative of infection or neoplastic disease, thus precluding a pathological diagnosis.\nAt 1 month after the initial examination, intermittent claudication occurred, prompting the performance of follow-up contrast-enhanced CT scanning. On CT scanning, we found an arterial occlusion from the infrarenal abdominal aorta to the bilateral common iliac arteries, the right external iliac artery, and the right internal iliac artery (Fig. ). These findings indicated the potential progress of the tumor to the aorta. Via a midline incision, biopsies of the mass and the lymph nodes peripheral to the sigmoid colon were obtained. However, these biopsies showed only connective tissue with inflammation and fibrosis and did not yield any significant findings. Since the developments, we elected to resect the mass and perform bypass surgery for definitive diagnosis and treatment after 3 weeks from biopsy.\nSurgery was performed using a transperitoneal approach via a midline incision. Adhesion was observed peripheral to the abdominal aorta, the bilateral iliac arteries, and the retroperitoneal mass. The abdominal aortic lumen was completely occluded by an old thrombus and plaque. Removal of the thrombus revealed a 35-mm intimal defect consistent with the direction of the retroperitoneal mass (Fig. ). Communication was observed between the intimal defect site and the retroperitoneal mass, which was filled with a partially organized old thrombus. During the intraoperative rapid pathological examination, the tissue inside the mass was diagnosed as a fibrin clot. The tumor tissue was not assessed. The area from the infrarenal abdominal aorta to the right common femoral artery and the left common iliac artery was bypassed using a Y-shaped woven Dacron graft. Pathologic examination showed that the mass tissue was a partially organized fibrin clot, thus agreeing with our intraoperative rapid diagnosis. On the basis of the intraoperative and pathologic findings, the patient was diagnosed with CCR-AAA with abdominal aortic occlusion. His intermittent claudication improved, and he was discharged on day 19 after surgery. Six months after the operation, the retroperitoneal mass disappeared on CT.
A 73-year-old white man presented to our emergency department with a 3-day history of left lower extremity swelling and acute-onset shortness of breath. On evaluation, he was tachycardic with a pulse of 113, hypertensive with a systolic blood pressure of 130-170 mmHg, and demonstrated poor oxygen saturation of 81% on room air. He was supported with continuous positive airway pressure (CPAP) and supplemental oxygen while a computed tomography angiogram (CTA) was obtained, which revealed a saddle PE (Fig. ).\nTissue plasminogen activator (tPA) was administered and he was started on a heparin infusion and admitted to our intensive care unit (ICU) for management. He remained on the heparin infusion for 3 days, during which he continuously improved and was eventually weaned to 3 L oxygen via nasal cannula. On hospital day 2, he was transferred to intermediate level of care. Per hematology recommendations, he would have to be on indefinite anticoagulation due to the massive PE he had sustained, the source of which was a left lower extremity popliteal deep vein thrombosis (DVT). The plan was to transition him from the heparin infusion to enoxaparin twice per day with hematology follow-up in 1 month.\nOn the day of discharge, however, he had sudden onset of right leg numbness and weakness below the level of his hip. He had previously been working with physical therapy and had been able to walk 200 feet with the assistance of a walker during each session. A physical examination revealed decreased sensation to light touch, 2/5 strength in right hip flexion and right knee extension and flexion, and loss of right patellar reflex. Left leg physical examination was normal at that time.\nAn emergent head computed tomography (CT) scan was ordered due to concern for a possible stroke, and neurology was consulted. The head CT was negative for infarction or hemorrhage. Neurology was concerned for spinal cord infarction versus hematoma and recommended emergent magnetic resonance imaging (MRI) of his thoracic and lumbar spine. The MRI revealed a left psoas hematoma (Fig. ). A CT of his pelvis performed the same day also showed a right psoas and iliacus hematoma. Due to these findings, hematology recommended discontinuing enoxaparin and reverting to a low intensity heparin infusion, as well as placement of an inferior vena cava (IVC) filter. The following day his left leg began exhibiting the same symptoms as his right leg. There was concern regarding the risk of progressive and irreversible nerve damage due to compression if the hematomas were not promptly drained. IR was consulted and advised that if the drainage were to occur while our patient was on anticoagulation, the risk of rebleeding into the retroperitoneum would be high and potentially nullify any benefit from drainage. Our patient would also be at risk of hemodynamic instability if the current hematomas were acting as tamponades against further bleeding. An additional complicating factor was the risk of further thrombosis due to the presenting saddle PE. Hematology was consulted for recommendations on pausing anticoagulation, but they were hesitant to offer a timeframe as there was no established safe period to enable this type of procedure to take place. Eventually, a window period of pausing the heparin infusion for 3 hours pre-procedure and up to 6 hours post-procedure was decided upon in the event that our patient agreed to have the drainage performed.\nThroughout this sequence of events, our patient and his wife were aware of the plans and considerations on how to proceed. They were informed of the recommendations and concerns made by neurology, hematology, and IR, as well as the risks and benefits of intervention versus non-intervention.\nAfter speaking with his wife, our patient decided to undergo the procedure. The low intensity heparin infusion was stopped 3 hours beforehand and the IR team then performed drainage of the right retroperitoneal hematoma, placing two pigtail catheters in our patient’s right flank (Fig. ). In total, the hematoma was drained of 215 milliliters of blood, 10 milliliters of which were drained during the procedure itself. The left psoas hematoma was not found to be amenable to drainage.\nOur patient tolerated the procedure well, and the heparin infusion was restarted 6 hours after it was completed. A repeat neurological examination demonstrated improved lower extremity strength bilaterally as well as the return of sensation to light touch. Hip flexion improved to 3/5 bilaterally, and knee flexion and extension improved to 4/5 bilaterally. Deep tendon reflexes remained absent. Four days later, the pigtail catheters were removed. His recovery was complicated by anemia requiring blood transfusions totaling 4 units of packed red blood cells (PRBC). Other sources of potential bleeding were evaluated and not found. A repeat CT on hospital day 10 (Fig. ) showed a stable right-sided hematoma, and our patient did not experience any further neurologic deficits. He was transitioned again from the heparin infusion to enoxaparin after 3 more days. His hemoglobin and hematocrit remained stable. During this time, he worked with physical therapy, who recommended discharge to a skilled nursing facility where his strength began to improve somewhat. Follow-up was scheduled with neurology and hematology. On hospital day 18, he was safely discharged.
A 64-year-old male patient presented to his primary care physician complaining of a dry cough and recurrent bouts of bronchitis as well as frequent orthostatic dizziness. At the age of 20 years, he had undergone repair of CO-A through a left thoracotomy using an interposition Dacron graft between the left SCA and the mid thoracic aorta. His chest radiograph raised suspicion of AN of the thoracic aorta. Computed tomographic angiogram then confirmed the presence of two large anastomotic PANs at both ends of the graft (\n) as well as an occluded right SCA, stenosis of the left vertebral artery, and bovine origin of the carotid arteries (\n).\nIn view of this complex anatomy, the decision was made to use a hybrid approach to address these findings. In a first stage, a right carotid artery to right SCA bypass was done using a short segment of Goretex graft (\n). Next, using single lung ventilation, a right posterolateral thoracotomy was performed and the chest entered through the fifth intercostal space. With the diaphragm retracted inferiorly, the pericardium, posterior to the phrenic nerve, was gently lifted with a long clamp, thus allowing exposure of the distal most portion of the thoracic aorta, medial to the inferior vena cava (IVC). After heparinization, the aorta was partially clamped and a 22 mm Hemashield graft anastomosed in an end-to-side fashion to an appropriate aortotomy using a continuous suture of 5–0 Prolene (\n). The graft was then brought posteriorly to the IVC and anteriorly to the right hilum, then anastomosed in an end-to-side similar fashion to the partially occluded ascending aorta through a vertical pericardial opening (\n,\n).\nThe immediate postoperative course was uneventful, and 5 days later, the patient was brought to the hybrid operating room for completion angiogram. This confirmed patency of the extra-anatomic graft as well as the presence of a 4 cm proximal left SCA anastomotic PAN with close extension to the left vertebral artery origin, and a 4.5 cm distal anastomotic PAN. EVS of the proximal PAN was done using a 16 × 80 mm covered Medtronic stent deployed across the origin of the left vertebral artery (\n).The distal PAN was excluded using a 26 × 100 mm covered Medtronic stent extending from the level of the coarctation to the insertion of the extra-anatomic bypass graft (\n). Completion angiogram confirmed patency of the latter and occlusion of the left SCA to thoracic aorta graft as well as of both anastomotic PANs (\n). The postoperative course was uneventful and the patient remains asymptomatic 2 years later and will be followed up on a yearly basis.
A 54-year-old male presented with a history of root canal treatment to tooth No. 30, followed by periapical surgery. At the time of periapical surgery, a periodontal problem was noted on tooth No. 32. Two weeks after the surgery, the patient presented with right posterior swelling that distorted the right cheek. He stated that his lower lip was ‘numb’. On intraoral examination, a large gingival swelling associated with tooth No. 32 was noticed, which had a clinical appearance of a pyogenic granuloma. There was a class III mobility to tooth No. 32. The swelling appeared to progress mesially on the edentulous ridge distal to tooth No. 30. The buccal mucosal tissue and gingiva was firm extending to the bicuspid and the vestibule. Tooth No. 32 was extracted, and the associated gingival lesion was removed. During the extraction, a large area of necrotic bone in the socket of tooth No. 32 and on the ridge near tooth No. 31 was observed. The lesion also progressed to the apex of the distal root of tooth No. 30. The radiographic findings were that of a radiolucent destruction of bone with ill-defined margins around tooth No. 30 that extended posteriorly to tooth No. 32 (fig. ). The differential diagnosis based on clinical and radiographic finding was an acute abscess, acute osteomyelitis or a malignant tumor such as an osteosarcoma. The patient's medical history was significant for hypertension and hepatitis.\nTwo tissue specimens were submitted. The first was during the apical surgery and the second was 2 weeks later when tooth No. 30 and No. 32 were extracted. The diagnosis for the first specimen was a periapical granuloma. The histopathology of the second specimen showed multiple fragments of a squamous mucosa, soft tissue and bone with a dense lymphoid infiltrate (fig. ). The lymphocytes were intermediate-to-large and atypical with a moderate amount of cytoplasm. The nuclei had finely clumped chromatin with variably prominent nucleoli, and there were abundant mitotic figures throughout the lesion (fig. ). A panel of immunostains was performed, with cells exhibiting positivity for CD20 (fig. ) and CD79a, weak positivity for CD10 (fig. ) and variable staining for Bcl-6 (fig. ). All cells were negative for CD34, TdT, Cyclin D1 and CD23. CD3 showed a scattered positivity for T-cells (fig. ). Interphase fluorescence in situ hybridization (FISH) showed no evidence of c-MYC/IgH translocation. Based on the histopathology and immunohistochemical findings, the lesion was diagnosed as a high-grade B-cell lymphoma.
A 17-year-old otherwise healthy male with noncontributory past medical history presented to his orthopedic surgeon for evaluation of chronic unremitting right foot pain that had been bothering him for six months. He finally decided to visit his orthopedic surgeon after noting swelling along the medial aspect of his foot while playing lacrosse. The patient graded the pain as an 8/10 with nothing making the pain better or worse. No inciting event could be recalled by the patient that started the pain six months earlier. On physical exam, the patient was tender over the tarsonavicular region without any appreciable swelling. Range of motion around the foot was normal and the patient could bear weight. Blood results such as complete blood count, erythrocyte sedimentation rate, and C-reactive protein values were all within normal limits.\nThe orthopedic surgeon decided to order radiographs of the foot that initially were read as negative for fracture or any significant abnormality by the radiologist. In retrospect, the posterior medial aspect of the cuboid articulated abnormally with the plantar lateral aspect of the navicular (). Importantly, no talar beak was visualized on radiography. The orthopedic surgeon then ordered magnetic resonance imaging (MRI) of the right foot without intravenous contrast to further elucidate the etiology of the patient's symptoms. MRI revealed abnormal articulation between the cuboid and navicular as well as marrow edema on both sides of the coalition with cystic change along the cuboid (Figures , , , , and ), consistent with fibrocartilaginous coalition as no osseous connection was seen between the cuboid and navicular. The patient was treated conservatively with physical therapy that helped for three months and is scheduled to receive a cortisone injection into the coalition if necessary for further alleviation of symptoms. However, to date, the patient has not reported pain recurrence and cortisone injection has been deferred until the patient presents again with pain. The orthopedic surgeon never ordered computed tomography (CT) examination since surgical planning was not needed in this case. No further imaging has been performed on this patient after his baseline MRI foot examination.
Mrs. X is a 45-year-old unmarried female who is a known case of bipolar affective disorder of total duration of 15 years during which she had two manic episodes without psychosis, the last episode being 10 years back. She is off medication for the past 7 years as per her previous therapist's advice following absence of any psychiatric symptoms for 2 years after treatment of the second episode. She presented with symptoms of elevated mood, increased talk, overfamiliarity, and decreased need for sleep for 5 days following a financial stressor. There was no history of aggression or psychotic symptoms and the symptoms did not affect her work or conflicted with her family members or colleagues. There is no history of any medical illness, use of other medications, or substance-use disorders. She belongs to the low socioeconomic status, with a family history of mental illness in the first- and second-degree relatives, suggestive of a depressive disorder.\nOn mental status examination, she was adequately groomed with normal psychomotor activity. Her talk was increased in tone, tempo, and content, but there were no formal thought disorders. Her mood was euphoric and there were no ideas of guilt, depressive ideas/death wishes, or psychotic symptoms. Her abstract ability was impaired, and her insight was grade 2.\nHer physical examination and vital signs revealed no abnormalities. Laboratory investigations on the day of admission including hemoglobin, total and differential count, platelet count, serum sodium and potassium, erythrocyte sedimentation rate, routine urine examination, renal and liver function tests, random blood sugar, and lipid profile were within the normal limits.\nAs per the 10th revision of International Statistical Classification of Diseases and Health Related Problems 10, a diagnosis of bipolar affective disorder, current episode hypomanic, was made. Given her history and a chance of her symptoms evolving to fully fledged manic episode, she was admitted and started on olanzapine 2.5 mg tablet HS and lorazepam 1 mg tablet HS. The patient slept the same night, but on the next day, she was found to be drowsy, not oriented in time and place with impaired attention, concentration, and immediate memory. There was no history suggestive of excessive fluid intake by the patient, vomiting, or diarrhea. She was afebrile, with normal vital signs. There were no signs of dehydrations and generalized/pedal edema. Her neurological examination did not reveal any lateralizing signs and examination of her other systems did not reveal any abnormalities. Her laboratory investigations were repeated and serum sodium was found to be 128 mEq/L with normal serum potassium and renal function tests. Other investigations were within the normal limits. Chest X-ray and computed topographic scan of the brain did not reveal any abnormalities. Further investigations revealed low serum osmolality (260 mOs/kg), elevated random urine osmolality (110 mOs/kg), and random urine sodium levels (55 mEq/L), based on which a diagnosis of SIADH was made. Antidiuretic hormone (ADH) level estimation was not available at our institution. After consulting with the physician, olanzapine was stopped and she was advised to follow normal diet and restricted fluid intake. Lorazepam 1 mg tablet was continued in the night. The patient's general condition improved over the next 2 days, with the patient attaining clear sensorium and normal serum sodium level on the 4th day. Lithium 300 mg once daily was started on the same day for the patient's hypomanic symptoms along with lorazepam. Lithium was gradually increased to 600 mg in two divided doses over the next 2 days for control of symptoms. She was discharged on the 8th day after becoming euthymic with the same medications. Her serum sodium was normal on the day of discharge. Rechallenge test was not attempted in the patient.
A 22-year-old single lady was brought to the Centre for Addiction Medicine with a request for help for tobacco cessation. She had been on treatment for schizophrenia for the last 6 years which had responded well to treatment and she was on maintenance with risperidone 4 mg/day and trihexyphenidyl 2 mg/day.\nOnly following 4 years of regular follow-up, following pressure from family, she reported the use of beedies in the form of chewing. Her father was a habitual beedi smoker and her mother used areca nut and betel leaf with tobacco. The patient had begun chewing beedies at the age of 15 years, after having observed her father smoking beedies, which she was often sent out to procure. She said she had begun chewing as she could not smoke (female smoking is not socially acceptable in most sections). Initially, she would chew the stubs her father had dropped around, but later began procuring beedies for her own use. While buying the beedies, she would say it was for her father. The patient had developed a dependent pattern of tobacco use since the age of 18 years. On average, she was chewing up to one pack, which contains 24 beedis.\nAt the time of her consultation for tobacco cessation, the positive symptoms of schizophrenia were well controlled, and she had minimal negative symptoms. She had been able to work as an office assistant. A general physical examination did not reveal any abnormality. Routine biochemical and hematological parameters were within normal limits. The diagnosis of nicotine dependence syndrome along with undifferentiated schizophrenia[] was considered.\nDespite significant improvement in her psychotic symptoms and occupational work function, treatment for tobacco dependence was challenging since patient reported very severe intense craving early in the morning and a wavering motivation for abstinence. Both pharmacological and psychosocial treatment measures were used for supporting tobacco cessation. A trial of nicotine gum 4 mg up to 12 mg per day and behavioral counseling was not useful to contain her tobacco use. She was subsequently prescribed bupropion 300 mg and was subsequently abstinent from tobacco use. She has stopped chewing beedies since the last 8 months and is not using any other form of tobacco.
The patient was a 27-year-old gravida 7 para 4 who presented with vaginal spotting at 23 weeks and 2 days of gestation. Her obstetric history included 4 preterm deliveries and 1 spontaneous abortion at 16 weeks. Her cervix was 4–5 cm dilated with a bulging bag. Ultrasound showed a live pregnancy with breech presentation, as well as a placental mass with differential diagnosis of a chorioangioma measuring 10x40 mm. Cervical length was unable to be accurately measured secondary to the anatomical changes. The patient was counseled and she consented to undergo amniocentesis and rescue cerclage placement. The amniocentesis showed a karyotype of 46XX. A cerclage was placed using the McDonald technique. The patient also received betamethasone for fetal lung maturity secondary to the risks for preterm delivery. The patient was discharged in stable condition after 2 days. Tocolytics or progesterone was not used.\nShe returned with vaginal bleeding and premature rupture of the membranes at 25 weeks and 2 days. The potential benefit of retaining the cerclage in order to prolong latency and decrease complications related to prematurity was considered. The decision was made to remove the cerclage at 25 weeks and 6 days due to the presence of heavy vaginal bleeding and the suspicion of preterm labor. She went into spontaneous preterm labor and vaginal delivery of a viable female fetus weighting 625 grams. The delivery was uneventful; however, the placenta was partially retained after delivery. An exploration of the uterine cavity and cervix was performed. This was followed by curettage due to continued heavy bleeding. Grape-like structures were seen during the procedure.\nThe placenta weighted 200 grams. The tissue appeared to have two distinct areas, one with normal placental tissue and one mixed with vesicles. This vesicular placental tissue was reported by pathology to represent gestational trophoblastic disease. Today, the fetus discussed previously is a healthy 4-year-old girl without major medical problems, aside from mild intermittent asthma. Her mother had a beta-hCG level less than 5 mIU/ml at 8 weeks post partum and is full remission today.
A 25-year-old gravida 1 para 0 woman at 35.6 weeks of pregnancy who weighed 57 kilograms was admitted to our hospital because of irregular uterine contractions. The patient had been buried for 48 hours in the ruins after an earthquake. She had undergone more than 30 surgical procedures within the previous 6 years. Her legs and abdomen were severely injured in the earthquake. She underwent left, total leg and right below-knee amputations, left hip resection due to clostridial necrotizing fasciitis, transverse colostomy, cystic and vulvar reconstructions, and colostomy reversal surgery approximately 2 years before becoming pregnant. In addition, she also suffered from spinal shock after the accident. Her sensory and motor function below the level of T10 did not recover until 8 months later. In addition, the patient had progressed to a chronic stage in which her reflex activity was regained. This stage was characterized by disuse atrophy, flexor spasms, and exaggerated reflexes.[ To bear the weight of her body, she had to sit, lie down, or lie on her side. As a result, the uneven distribution of the stress caused compensatory changes in her spine, leading to severe scoliosis.\nThis patient had 2 episodes of congestive heart failure during her previous surgical interventions that required hospitalization in an intensive care unit (ICU). When she was admitted to our hospital, the fetal heart rate and movement were monitored closely. Dexamethasone was used to promote fetal lung maturation. At 36.4 weeks of gestation, her uterine contractions became more frequent, and preterm delivery was inevitable. Spontaneous vaginal delivery was not an option because of the patient's incomplete pelvis structure due to the previous left hip resection and extensive keloid tissue over the perineal region (Fig. ). A cesarean delivery was planned after multidisciplinary consultation and meetings with the patient.\nGeneral anesthesia was planned due to the patient's traumatic stress experience. Preoperatively, we ruled out deep venous thrombosis using Doppler sonography. The patient's right internal jugular vein was cannulated preoperatively due to difficult intravenous access. The patient inhaled oxygen in the operating room in the semidecubitus position. In preparation for a potentially prolonged operation, arterial access was also established according to the standard American Society of Anesthesiology monitoring guidelines. We used the central venous pressure (CVP) and pleth variability index (PVI) to guide fluid management. The bispectral index (BIS) and continuous total hemoglobin were also measured. The patient's blood pressure was 105/63 mm Hg, her heart rate was 102 beats/min, her oxygen saturation was 99% when breathing oxygen (>4 L/min), and her CVP was 6 cm H2O. After intravenous hydration with 300 ml of lactated Ringer solution (approximately 10 ml/min), she was positioned in the supine position with left uterine displacement to minimize aortocaval compression.\nAfter sterile preparation, general anesthesia was induced through rapid sequence induction with ketamine, propofol, and succinylcholine, following adequate preoxygenation. A surgical incision was made immediately after successful tracheal intubation. To deliver the fetus as quickly as possible, a high transverse abdominal incision and a low transverse segmental uterine incision were used by the obstetric team to avoid surgical scars and peritoneal adhesions. A male infant weighing 2940 g was delivered in 3 minutes. Apgar scores were estimated to be 10 at 1 minute and 10 at 5 minutes. Anesthesia was maintained with a reduced sevoflurane concentration and sufentanil. Warm blankets were used during surgery, and parts of the patient's limbs were protected. The patient was stable during surgery with no significant blood loss. The patient's intraoperative systolic blood pressure was 110 to 130 mm Hg, her heart rate was 100 to 110 beats/min, her BIS was 45 to 65, and her CVP was 5 to 8 cm H2O. The duration of surgery and anesthesia were 34 minutes and 1 hour and 42 minutes, respectively. Intraoperatively, the estimated blood loss was approximately 500 ml, 800 ml of lactated Ringer solution was administered during surgery, and 200 ml of clear urine was collected. The patient was extubated after surgery and was then transferred to the ICU. At 42-days of follow up, she exhibited no signs of any postoperative complications.
A 50-year-old male presented to the Department of Otorhinolaryngology with left facial asymmetry of two months duration. Clinical examination revealed an infranuclear facial palsy on left side with associated bilateral postauricular and occipital region scalp swellings. The scalp swellings were firm and nontender and margins could not be well identified. No neck nodes were palpable on clinical examination. Routine laboratory investigations were within normal limits. The patient was referred to the Department of Imaging to identify the cause of the facial palsy and determine extent of the scalp swellings. A noncontrast HRCT scan of the temporal bones and a contrast enhanced MRI scan of the temporal bones/brain was performed. HRCT of the temporal bones showed extensive irregular permeative osteolytic destruction of the right temporal bone and adjacent right occipital bone. Similar lesions were also noted involving the base of the left temporal bone (Figures and ). HRCT also revealed soft tissue opacification of the mastoid air cells on both sides with erosion of the intercellular septae. Internal and external bony cortical erosions were seen on both sides with erosion of the descending mastoid segment of the left facial nerve canal. The middle and inner ear structures were normal on both sides. MRI scan showed diffuse signal alteration in both temporal bones with associated lobulated, extradural, and subgaleal enhancing soft tissue lesions (Figures and ). The lesions were hypointense on both T1 weighted and T2 weighted images with heterogeneous postcontrast enhancement and showed restricted diffusion on diffusion weighted images (DWI). No calcification or hemorrhagic foci were noted. On the left side, the soft tissue was seen extending along the styloid process into the stylomandibular tunnel up to the deep lobe of parotid gland, involving the extracranial segment of the left facial nerve below the level of stylomastoid foramen. There was no enhancement of the facial nerve seen within the left temporal bone or left internal auditory canal. This finding ruled out retrograde perineural spread of the pathology. On the right side, the intracranial extradural enhancing soft tissue component was seen extending into the middle and posterior cranial fossa. Subgaleal extension of the soft tissue was seen through a defect in the right occipital bone. There was no significant cervical lymph node enlargement detected on the MRI scan. Based on the age of the patient, the clinical presentation, and examination as well as the imaging findings the differential diagnosis included multiple myeloma, metastases, and lymphoma. The clinical presentation and imaging findings were not suggestive of an infective aetiology and hence this diagnosis was not considered.\nAn excision biopsy of the right subgaleal swelling was performed. Microscopy showed sheets of medium to large lymphoid cells with hyperchromatic nuclei and scanty cytoplasm (Figures –). These cells stained positive for CD3 (), CD20 (), Ki67 (), LCA1, (), and LCA 2 () and negative for CyclinD1 (clone Polyclonal), CD5 (Clone 4C7), and CD138 (clone MI-15). The tumor was also positive for Mum-1 and Bcl6 and negative for EBVLMP-1. The above results are suggestive of diffuse large B-cell non-Hodgkin's lymphoma (DLBCL), activated B-cell phenotype. Other blood and bone marrow investigations did not reveal any abnormality.\nA whole body fluorodeoxyglucose (FDG) positron emission tomography-computed tomography study (PET-CT) was performed for staging purposes. The PET-CT scan revealed FDG-avid lesions (SUV max. 3.6) in both mastoids, in the extradural soft tissue mass on the right side and extracranial mass along left styloid process (Figures and ). No other FDG-avid lesions were detected in the rest of the body (). This confirmed a primary extranodal involvement of the temporal bones by DLBCL.\nThe patient was treated with six cycles R+CHOP chemotherapy (one cycle of 21 days). This consisted of rituximab at 375 mg/m2, cyclophosphamide at 750 mg/m2, doxorubicin at 50 mg/m2, vincristine at 1.4 mg/m2, and prednisolone at 100 mg/m2. This was supplemented by prophylactic intrathecal methotrexate at 12.5 mg on day 2 of each cycle and G-CSF (granulocyte colony stimulating factor) at 300 μgm from day 3 to day 7 of each cycle. After six cycles of chemotherapy, patient was treated with six cycles of radiotherapy (45 Gy, 20 fractions). A PET-CT scan was performed three months after the end of radiation therapy which showed significant resolution in the metabolic activity of the mastoid lesions. (Figures and ). The repeat MRI scan also showed regression in the size of the enhancing soft tissue masses on both sides (Figures and ).\nPatient also showed signs of clinical improvement and has been placed on routine surveillance protocol which includes a follow-up visit to the Oncology outpatient department once every 6 months for the first 2 years and then once every one year for next 3 years. At the time of the visit the patient will undergo a whole body FDG PET-CT scan, a 2D Echocardiogram (to look for cardiotoxic side effects of Adriamycin), and routine laboratory investigations. Till date the patient is in clinical remission as documented on the last outpatient department visit. The patient is alive and asymptomatic without disease progression for the last twenty months after initial diagnosis, without any evidence of local or systemic recurrence.
A 38-year-old African American woman came to emergency room with generalized body aches. She also reported three episodes of vomiting containing clear liquid and subjective fever. She denied respiratory, cardiovascular or neurological symptoms at the time of initial evaluation. Her past medical history was negative for any medical conditions. Her surgical history included right knee arthroscopy few years back. She denied tobacco, alcohol or recreational drug use. She denied any allergies to medications in the past.\nIn the emergency room she was found to have temperature of 100 degrees of Fahrenheit, heart rate of 72 per minute, systolic blood pressure of 164 and diastolic blood pressure of 64 millimeters of mercury respectively. On examination she was a well built woman without any distress. Her abdominal examination did not reveal any tenderness, organomegaly or clinically detectable free fluid. Her cardiovascular, respiratory and neurological examination was within normal limits. Her laboratory and imaging tests revealed abnormal liver chemistries () and dilated common bile duct (CBD) of 8 millimeters without any evidence of cholelithiasis or choledocholithiasis. She was hydrated with intravenous fluids and treated symptomatically with Ibuprofen 800 mg for body aches as needed. She was admitted to medical floor for further work up and management.\nOn day 2, gastroenterology evaluation was requested for abnormal liver chemistries. She did not report any further episodes of vomiting and her body aches slightly improved with ibuprofen. On further interviewing it was noted that recently she fell and injured her left knee. She was evaluated by orthopedician and noted to have anterior cruciate ligament, medial meniscus and medial collateral ligament tears on magnetic resonance imaging (MRI). She mentioned that for last one week she had been taking over-the-counter Ibuprofen 600 mg tablets as needed for her knee pain. Extensive laboratory work up aimed to diagnose viral, metabolic and autoimmune liver diseases was requested. Magnetic resonance cholangiopancreatography (MRCP) was requested to rule out biliary obstruction, as there was CBD dilatation noted on abdominal sonogram. It was also recommended to stop Ibuprofen as initial presumptive diagnoses included drug induced liver injury.\nHer liver chemistries showed alaninie aminotransferase (ALT) of 249 units/liter, aspartate aminotransferase (AST) of 201 units/liter, alkaline phosphatase (ALP) of 31 units/liter with normal total protein, albumin and bilirubin levels. Her other significant laboratory results include platelet count of 139,000/micro liter and potassium levels of 3 milliequivalents/liter. She was immune to hepatitis B and tested negative for hepatitis A, hepatitis C and human immunodeficiency (HIV) viruses. Her transferrin saturation was 16% and ceruloplasmin levels were within normal limits excluding the possibility of hemochromatosis and Wilson disease respectively. Autoimmune markers including anti-nuclear antibody (ANA), anti-mitochondrial antibody were negative except for anti-smooth muscle antibody titers of 1:20, which was not clinically significant. MRCP was done which showed dilated common bile duct of 9 millimeters without any evidence of choledocholitiasis or obstructing lesion.\nOn day 3, she became lethargic without any focal neurological deficit and was transferred to medical intensive care unit (MICU) for close monitoring. Computer tomography (CT) and MRI of brain ruled out acute intracranial pathology and lumbar puncture with cerebrospinal fluid (CSF) analysis was performed. Her CSF analysis showed elevated protein, normal glucose and lymphocytic pleocytosis. CSF was tested negative for common bacterial and viral pathology. Her blood and urine were tested negative for common toxicological agents. Her mental status gradually improved after cessation of Ibuprofen and transferred back to medical floor. She underwent CT guided liver biopsy which was uneventful. Liver biopsy (, ) was reported to have mild lobular hepatitis with eosinophilic infiltrate suggestive of drug induced liver injury.\nHer hospital course was significant for an episode of fever associated with leucocytosis. Complete septic work up including blood, urine cultures and x-ray of chest were done. She received broad-spectrum antibiotics and improved without any further febrile episodes. Her x-ray was negative for any lung pathology and body fluid cultures were negative. During this period she also developed acute renal failure with blood urea nitrogen of 32 milligrams/dL and creatinine of 3 milligrams/dL, which improved with conservative management. Subsequently her clinical condition improved and all her laboratory values including liver chemistries, electrolytes and platelets were normalized leading to her discharge from hospital.\nOn further follow up in clinic, she was asymptomatic and her all laboratory tests were within normal limits. She underwent endoscopic ultrasound (EUS) which did not reveal any CBD stone or obstructive lesion. She was subsequently evaluated by orhtopedician and underwent arthroscopic intervention for her knee injury.
A 65-year-old woman presented with abdominal pain and hypophagia for 2 weeks. She had a medical history of alcoholic pancreatitis. A computed tomography (CT) scan of her abdomen and pelvis revealed thickening of the transverse colon wall. Colonoscopy showed an ulcerated tumor in the transverse colon, and histological analysis indicated moderately differentiated adenocarcinoma. Serum carcinoembryonic antigen (CEA) levels were normal, and no sites of distant metastasis were reported on preoperative examination. She underwent a laparoscopic extended right hemicolectomy using the non-touch isolation technique. Histopathological examination of the specimen revealed a moderately differentiated adenocarcinoma invading the subserosal layer with low venous invasion (v1) and no lymphatic invasion (ly0). The surgical margins were negative. A total of 92 lymph nodes were removed, of which none showed metastases. The tumor was diagnosed as stage IIA (T3, N0, M0) according to the International Union Against Cancer tumor, node, and metastasis (TNM) classification (7th edition) []. The patient had an uneventful recovery and was on regular follow-up every 3 months without adjuvant chemotherapy.\nFifteen months after her colectomy, a CT scan demonstrated a tumor in the antero-lateral rectal wall that was 20 mm in diameter, and a right ovarian tumor that was 25 mm in diameter (Fig. ). The ovarian tumor was a cystic mass with a solid component on magnetic resonance imaging (Fig. ). Colonoscopy revealed a superficial elevated lesion in the middle rectum that was shown by histological examination to be moderately differentiated adenocarcinoma (Fig. ). Serum CEA levels were normal. These findings were indicative of rectal cancer with ovarian metastasis, or double primary cancer of the rectum and ovary. A diagnostic and therapeutic laparoscopy detected no disseminated peritoneal metastases or liver metastases. Intraoperative cytological examination of the peritoneal lavage was negative for carcinoma. An operative rapid pathological diagnosis of the resected right ovarian tumor indicated metastasis of the colorectal cancer. Thus, we performed laparoscopic low anterior resection preserving the left colic artery with partial resection of the vagina, and bilateral adnexectomy.\nThe resected rectal tumor measured 20 × 18 mm (Fig. ). Histological examination demonstrated a moderately differentiated adenocarcinoma that had invaded the vagina and formed a metastasis in one of 32 lymph nodes. High venous invasion (v3) and low lymphatic invasion (ly1) were observed. The adenocarcinoma was mainly present in the submucosa and muscularis propria with a small range of invasion to the vagina (Fig. ), while the carcinoma-involved region of the mucosal layer had mucosal colonization representing the spread of metastatic tumor cells along the basement membrane of preexisting crypts and/or villi (Fig. ) []. There was no adenomatous precursor at the edge of the tumor, and surgical margins were negative. The right ovarian tumor was moderately differentiated adenocarcinoma that was positive for cytokeratin (CK) 20 and negative for CK7 immunohistochemical staining, indicating metastasis of colorectal cancer. The rectal and ovarian tumors shared high similarities with transverse colon cancer in architectural and cytological atypia (Fig. ). Both adenocarcinomas of the transverse colon and rectum were negative for p53 immunohistochemical staining and RAS wild type in genetic assessment (Fig. ). These findings support a diagnosis of rectal and ovarian metastases from primary transverse colon cancer.\nThe patient recovered well after surgery, and adjuvant chemotherapy was decided after a multidisciplinary meeting. She was treated with eight cycles of oxaliplatin and capecitabine, and neither relapse nor metastasis has been observed 18 months after surgery.\nThe incidence of colorectal metastasis from primary colorectal cancer is rare, and distinguishing primary from metastatic colorectal cancer can be challenging. To our knowledge, only three reports in English and six in Japanese have described suspected cases of colorectal metastasis of colorectal cancer [–]. We reviewed a total of eight patients, including seven out of the abovementioned nine patients with detailed clinical information as well as our own (Table ). The patients were three males and five females, with a median age of 66.5 years (range 52–88 years). Ascending colon cancer was the most common primary tumor (n = 3), followed by tumors of the sigmoid (n = 2), transverse colon (n = 2), and cecum (n = 1). Metastatic colorectal lesions were located in the rectum in all cases. In terms of other metastatic sites, seven patients had other metastases (lung, liver, spleen, abdominal wall, axilla, and ovary) and one had no other metastases.\nA previous study of 278,208 malignancies in a nationwide Japanese pathologic autopsy database from 1990 to 2003 identified 18,252 case with metastatic colorectal cancer. Of these, 1302 (7.1%) were from primary colorectal cancer []. The database does not include information about the suspected metastatic pathway (hematogenous, lymphogenous, direct invasion, or dissemination), but most metastatic tumors in the database are attributed to direct invasion or disseminated metastasis, reflecting late-stage disease. However, the number of reported clinical cases might not represent the actual incidence of colorectal metastasis from colorectal cancer. One possible reason for the rarity of reported colorectal metastasis cases is that most cases occur as part of systemic advanced disease, for which surgical resection will not be performed []. The other reason is that diagnosing metastasis is difficult [, ]. Although gastrointestinal metastatic carcinoma usually represents a submucosal tumor, differential diagnosis (as primary disease or metastasis) becomes difficult if the tumor invades the mucosal layer. Estrella et al. argued that metastatic carcinomas involving the mucosal surface frequently mimic second primaries, so histologic features cannot reliably distinguish metastatic from primary carcinoma []. Additionally, primary colorectal cancers resembling submucosal tumors have been reported in previous studies [, ]. This explains why colorectal metastases may be misdiagnosed and treated as primaries.\nDistinguishing primary from metastatic colorectal cancer can be challenging, but a comprehensive evaluation of histological features, clinical history, and tumor distribution enables making the correct diagnosis and implementing the optimal treatments []. In this case, we suspected a metachronous metastasis for the following reasons. First, histological findings demonstrated that the adenocarcinoma was mainly present in the submucosa and muscularis propria with mucosal colonization, which is a supportive finding for metastatic carcinoma, as this indicates a low possibility of primary rectal cancer and implantation of the transverse colon cancer. Second, the rectal and ovarian tumors were very similar to the transverse colon cancer in terms of architectural and cytological atypia, further suggesting metastasis of primary transverse colon cancer. Third, there was no adenomatous precursor at the tumor edge, indicating metastatic cancer. Fourth, both adenocarcinomas of the transverse colon and rectum were negative for p53 in immunohistochemical staining and RAS wild type in genetic assessment, indicating metastatic rectal cancer from primary transverse colon cancer.\nIn reviewed cases, the exact mechanism of colorectal metastasis from primary colorectal cancer has not been fully elucidated; thus, the possibility of hematogenous or lymphogenous spread remains. We suspected hematogenous or lymphogenous metastatic pathways to the rectum from the primary transverse colon cancer because the carcinoma was mainly located in submucosa and muscularis propria; however, there was no additional evidence than tumor localization. In this case, the rectal tumor penetrated into a small range of the subserosal layer with invasion to the vagina; additionally, there was a metastatic ovarian carcinoma. Peritoneal metastatic invasion primarily toward the deep layer of the rectal wall cannot be ruled out. The metastatic colorectal lesions were located in the rectum in all reviewed cases, and this suggests that peritoneal spread to Douglas’s pouch or the rectovesical pouch is a possible pathway for colorectal metastasis from primary colorectal cancer.\nConsidering that the majority of reported cases had multiple metastases, the existence of other metastatic lesions might be a risk factor for colorectal metastasis. Therefore, more attention should be paid to colorectal lesions when other metastatic sites have been identified. Colorectal metastases usually represent late-stage disease and have poor prognoses; however, prolonged survival after surgery and complementary therapy can be achieved in some patients []. In this case, neither relapse nor metastasis has been observed 18 months after surgery. The follow-up periods varied too widely to evaluate patient prognosis in reviewed cases. Therefore, additional studies are needed to better understand this rare metastasis and to determine the optimal therapeutic strategies.
A 38-year-old female patient came with a chief complaint of difficulty standing up from squatting position since 2 years ago. The patient also suffered heaviness and numbness from her hips that radiated to both of her knees and ankles. The symptoms worsened steadily in the past 4 months with both lower limbs getting weaker. Laboratory results came out normal, with no signs of infection or positive tumor markers. Radiological examination showed no apparent abnormalities as well. An MRI was obtained, and a tumor mass in the intradural region level of T10–T12 was found (). At that time, the patient was offered surgery, but she chose to undergo treatment with a bone setter. Around two months later, the patient returned to hospital with profound weakness on her lower extremity. Her physical examination revealed paresis from her thigh on both lower extremities grade 1-2/5 power in left and right lower limbs, respectively. Increased patellar reflexes were found on both limbs. Another MRI was performed and showed that the mass had grown to lumbar vertebrae L2, accompanied with worsening of the neurological statuses and impaired sensibility, as well as defecating and urinating problems (). From the history, spinal manipulation procedure was performed by a bone setter, although no specific techniques were available for review.\nA surgical procedure was proposed for exploration and decompression to the patient. The operation started by opening the lamina on T10–T12 levels, followed with laminectomy and hemostatic procedure to stop the bleeding, until the dura was exposed (). A dense mass from T10 to T12 was palpable from the dura layer. After we exposed the lamina, we observed that the dura was tense from touch, and a solid mass underneath the dura was palpable from T10 to L2. Intradural tumor excision was performed by a sharp 3 mm incision in the midline of the dura and then continued with blunt dissection, opening the dura layer to caudal L2 and to cranial T10. Dura was then parted with stay thread until the vessel-rich tumor mass was exposed. The tumor mass was excised, and the surgical field was contaminated by blood from hematomas from tumor vessels (). Bleeding was found to originate from the anterior part and posterior part of the cord, no bleeding source from the cerebrospinal fluid nor the subarachnoid space. The tumor mass was successfully evacuated with fragments of hematomas and necrotic tissues. The dura layer was then closed with a continuous suture. The tumor mass was fixed and transported for histopathology examination ().\nAt the time of discharge, the patient did not regain the function on her lower extremities (1-2 out of 5 on neurological motor examination). After six months of follow-up, some improvement on her lower extremity function was noted. Motor strength was returned to 3-4 out of 5, and the patient was able to ambulate using a walker. No improvement of her bowel and bladder symptoms was noted.

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