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We present the case of a 75-year-old male whom initially presented to our service with a mass on his right upper back. The patient's medical history included coronary artery disease and hyperlipidemia. Further history revealed he had received (2) stents in the past and undergone multiple heart catheterizations (up to seven) approximately seven years prior at an outside facility. During his last catheterization and stent procedure, which took over 4 hours (approx. 33 mSv), he developed a radiation-induced injury to his right back around the T10 dermatome (). During the following years, the burn was closely followed at an outside facility, and the area developed a chronic nonhealing ulcer which continued to evolve over time eventually growing outward from his back and developing a foul odor on his presentation to our facility in 2017. He reported increasing pain at the periphery of the tumor margin. Up to a year prior to presentation, the area was flat and biopsies revealed no evidence of malignancy. Upon presentation to us, however, the physical exam demonstrated a fungating mass approximately 5 × 9 cm with central purulent necrosis and induration surrounding the periphery (Figures –). No additional nodularity or adenopathy was found on physical exam initially. At this time, punch biopsies demonstrated undifferentiated pleomorphic sarcoma along with frankly necrotic debris.\nInitial staging showed only localized disease by MRI with no evidence of metastasis by CT scan (Figures and ). Approximately one month following diagnosis, the patient underwent a radical excision with a 20 × 9 cm elliptical excision of the site. In this case, the patient chose human dermal matrix reconstruction, as primary closure was not possible due to the size of the site. Our patient did not want an additional wound of autologous skin procurement.\nPathology of histologic sections revealed a highly cellular spindle cell neoplasm. Focal areas of necrosis comprised less than 50% of sampled tissue. Mitotic figures were readily evident (17 per 10 high-power microscopic fields), including atypical forms. Marked pleomorphism was present with vesicular nuclei, irregular nuclear contours, and scattered prominent nucleoli (Figures and ). The immunohistochemical staining pattern was supportive of sarcoma. The neoplastic cells are positive for CD68 and vimentin (). The neoplastic cells were negative for markers of melanocytic origin (S100 protein, sox10, and melan A), epithelial origin (pancytokeratin AE1/AE3), vascular origin (CD31), neural origin (S100 protein), and muscle origin (smooth muscle actin, myoD1, and desmin). MDM2 gene amplification by FISH testing was negative. Overall histologic and immunohistochemical staining features were those of undifferentiated pleomorphic sarcoma (pleomorphic malignant fibrous histiocytoma). The final pathology of the mass demonstrated a grade 3 undifferentiated pleomorphic sarcoma-staged pT2a. All margins were negative for malignancy obtaining an R0 resection. Furthermore, there was no evidence of angiolymphatic invasion. After discussion by our institutional multidisciplinary tumor board, adjuvant therapy was not recommended. The patient's postsurgical course was essentially benign with the exception of not unexpected wound healing issues. The patient is under continued observation with clinical follow-up in accordance with NCCN guidelines. Follow-up CT in October 2018 demonstrated pulmonary metastases, and he passed away from disease in June 2018.
The patient, a 31-year-old woman, was admitted to the department of neurosurgery at The First Hospital of Jilin University due to a “right occipital mass for 5 months”. The patient was in good health, and her family members did not have similar lesions. A physical examination showed that the mass was in the right occipital region at the upper margin of the sternocleidomastoid muscle and exhibited slight tenderness without obvious pulsation or vascular murmurs during auscultation.\nA head CTA examination at admission suggested that the lesion was approximately 3 × 5 cm in size, was mainly composed of vascular components, presented as a “wool mass”, was located in a subcutaneous region, involved the muscles, and showed obvious enhancement. The lesion was mainly supplied by the occipital and muscular branches of the vertebral arteries. The venous drainage of lesion communicated with the suboccipital venous plexus and the paravertebral venous plexus, and the venous drainage communicated with the subcutaneous cervical superficial veins (Figs. and 2).\nA further DSA examination revealed that the blood supply of the lesion was mainly from the occipital artery and less from the muscle branches of the vertebral arteries (Fig. ). The lesion was considered an AVM based on its imaging characteristics and was recommended for surgical removal. The surgery was carried out under general anesthesia. The lesion could be touched when the occipital skin was incised during the operation. The lesion showed a clear boundary. The occipital artery touched the anterior margin of the lesion, was exposed and ligated, and was removed around the lesion. The lesion consisted of massive blood vessels, and the surrounding muscles were swollen, indicating IH.\nAfter the lesion was removed, the normal muscle tissue around the lesion was also removed. The specimen was sent to pathology, and postoperative pathology confirmed that the lesion was IH accompanied by thrombolytic recanalization. HE staining showed that the lesion almost purely consisted of capillaries with only rare cavernous components among the muscles. CD31 staining was positive in the vessel endothelium, indicating that the lesion was an IH (Fig. ). The patient achieved a good recovery after surgery and was discharged from the hospital. A head CTA review performed one year later showed partial residual IH (Fig. ). The patient had no obvious discomfort, and the hemangioma could not be touched from the surface, and the patient was recommended for radiation therapy. The patient refused radiation therapy. Follow-up 2 years later showed no enlargement of the IH.
An 18 year old girl with no past or family history of psychiatric illness as well as a bleeding disorder, presented to the surgical emergency department with abrupt onset bleeding from the nose. She was admitted to the department of otorhinolaryngology and evaluated for bleeding disorders like factor VIII deficiency and platelet abnormality and underwent neurological imaging and abdominal ultrasonography but investigations were non-contributory. Investigations like total count, differential count, renal function test, liver function test, thyroid function test, and electrolytes were within normal limits. Epilepsy was ruled out in consultation with the department of neurology. In view of non-response to medication, the patient was sent to the psychiatric out patient service for a consultation. Patient’s BMI was 25. Vitals were stable with vesicular breathing sounds and normal S1 S2 heart sounds. There were no organomegaly and tenderness over abdomen. Central nervous system and peripheral nervous system parameters were within normal limits. For genital examination, gynaecological consultation was asked for. A gynaecology consultation revealed that she had multiple cut marks over the unexposed areas of her body. Injury report was prepared that showed 1cm by 5cm cuts marks over her thigh and upper leg mostly in the medial region. All cuts were superficial skin cuts. Most of them were dry and few were recent like near to the upper thigh. She denied any physical or sexual abuse. On instruction from us, the mother kept an eye on the patient unobserved and discovered the patient injuring herself with sharp objects in unexposed areas and mixing it with her nasal secretions to simulate epistaxis.\nAfter a detailed evaluation, the patient revealed that she was undergoing significant ongoing stress due to parental conflict. Since her childhood she has been exposed to severe mental turmoil due to the unhealthy relationship between her parents. Though there was no growth restriction to her social, motor and language milestones, she had developed very poor attachment behavior towards parents and friends. She also mentioned a lack of confidence about decision making and asking validation in everything she had to take part which exposed her dependent personality traits.\nAlthough a high achieving student, her grades had recently been slipping. She claimed to be unable to recall what had happened during the periods of epistaxis. After mental status examination and suggestion, she revealed that she had been having low mood and loss of interest in academics but denied suicidal ideation and vegetative dysfunction. She did not have any intention to obtain medical attention. Her main motive seemed to be to get attention from her parents.\nShe was started on fluoxetine and cognitive behavioral therapy and then we persuaded the family to attend family therapy to resolve the parental conflict. She was followed up for 1 year with monthly telephonic consultation and three monthly outpatient visits. Since the last consultation, she was doing well with excellent academic achievements. Fluoxetine was tapered and stopped after 9 months of therapy. Positive reappraisal, seeking social support and problem solving strategies were taught and mindfulness techniques were demonstrated to her.
A thirty-nine-year-old woman who was thirty-six weeks pregnant sustained a left ankle injury during a mechanical slip and fall, resulting in significant pain and deformity. Her pregnancy had been uncomplicated up until that point. She had no significant past medical or surgical history and did not take any medications at the time of injury. On initial presentation to the emergency department at an outside institution, the patient was diagnosed with a trimalleolar left ankle fracture dislocation (). The fracture pattern was consistent with a Lauge-Hansen supination-external rotation IV injury. The patient's neurovascular status was intact. She was closed reduced and transferred in a splint from the outside hospital to our institution for further care.\nUpon arrival at our emergency room, we reviewed the radiographic images, which revealed an incompletely reduced tibiotalar joint and proceeded to remove the splint in order to fully examine the site of injury. At this time, the ankle was noted to be highly unstable. Attempts at closed reduction and splinting yielded the same incomplete restoration of the ankle mortise with persistent subluxation of the talus. The patient was counseled that her injury was unstable, incompletely reduced, and that it would require surgical intervention. The risks and benefits of surgery, not only to the patient but also to the fetus, were discussed with her in conjunction with the obstetricians. The decision was reached to proceed the following day with open reduction and internal fixation of the left ankle.\nA fetal heart rate monitor was used for continuous fetal assessment while in the operating room. Spinal anesthesia was performed with the patient in the lateral decubitus position, as this was deemed more appropriate than general anesthesia with relation to the pregnancy. Following the induction of anesthesia, the patient was placed in the supine position with a bump under her right side to displace the uterus. Sterile draping was begun. During this time, the fetal monitor began to show prolonged heart rate decelerations to 50–60 beats per minute, which did not correct with changing the patient's position. The obstetrical team, who had been previously notified about the case and was present in the hospital, was immediately called. Upon their arrival, the obstetricians decided to perform an emergent cesarean section. Though the fetal heart rate had stabilized by the time they completed their evaluation, they were committed to an emergent delivery in order to avoid further distress to the fetus.\nThe patient was again placed in the lateral decubitus position in order to receive an epidural catheter. After this was placed, the patient was returned supine, and a caesarian section was performed. A healthy infant female was successfully delivered, with APGAR scores of eight at one minute and nine at five minutes. The obstetricians closed the uterus and abdomen without incident.\nAfter the anesthesiologists and obstetricians reassessed the patient and determined that she was stable for further surgery, we proceeded with open reduction and internal fixation of the ankle (). The patient tolerated the procedure well, and there were no further complications. Postoperatively, the patient was transferred to the postpartum unit, where she progressed well over the ensuing days in terms of rehabilitation and physical therapy. The patient was changed from a plaster splint to a fiberglass cast after several days. She was discharged from the hospital on postoperative day four in good condition. Clinical followups in the twelve months since the operation have shown no complications, with both the patient and her daughter doing well and progressing appropriately.
A 72-year-old Caucasian female who was initially diagnosed with ‘saddle pulmonary embolism’ based on an outpatient computed tomography (CT) chest angiogram approximately 2 months prior to the current presentation and subsequently placed on warfarin for anticoagulation presented with persistent dyspnea. Her dyspnea had been progressively worsening during this time, and serial CT scans showed cardiomegaly with pulmonary emboli involving the right and left main pulmonary arteries with extension into the right and left upper and lower lobe branches (). The CT scans also showed a 47×42 mm right upper lobe paratracheal mass that had significantly increased in size over the 2-month period prior to this presentation. An echocardiogram shortly after admission showed severe pulmonary hypertension with a pulmonary artery pressure of 82.9 mm Hg, and a severely enlarged right ventricle. The patient developed respiratory distress and was placed on mechanical ventilation. Multiple laboratory values at this time indicated multiorgan failure with worsening right ventricular function. Subsequently, the patient became hypotensive and bradycardic. Despite aggressive resuscitative measures, the patient expired shortly thereafter. A chest-only autopsy performed with the family's consent revealed biatrial dilatation, and the right ventricular cavity was dilated with a thickened wall. The interventricular septum was distorted and pushed toward the left ventricular cavity (). The lungs were markedly congested. A lobulated, pale, yellow mass occupied the entire volume of the right and the left main pulmonary arteries, loosely adherent to the pulmonary artery at multiple locations and measuring 13 cm in greatest dimension. The pulmonary artery mass extended into the parenchyma of the right upper lobe, 6 cm in greatest dimension (). On microscopy, this mass showed a highly malignant neoplasm, characterized by a sheet-like growth pattern with alternating areas of higher and lower cellularity; highly pleomorphic nuclei with abnormal mitotic figures, over 10 mitoses per 10 high-power fields; and extensive areas of necrosis. These cells were positive for desmin and vimentin and showed focal reactivity for actin. Overall, this was consistent with high-grade primary pulmonary artery leiomyosarcoma ().
A 15-year-old male, presented with a Class I incisor relationship on a mild Class II skeletal base with increased vertical proportions (). This was complicated by heavily restored upper first permanent molars, restored lower first permanent molars, reduced overbite, moderate upper arch and severe lower arch crowding, and a lower centreline discrepancy to the right side (). The maxillary central incisors showed white opacities. His dental history revealed repeated and major restorative work associated with his MIH-affected FPMs. The patient also presented with signs and symptoms associated with the heavily restored upper FPMs. His Orthopantomograph (OPT) showed signs of molar stacking of the upper right and left second molars, and distally tipped lower right and left second molars (). His paediatric dentist referred him to explore the potential for fixed appliance therapy and, if indicated, whether or not extractions of FPMs will be planned as part of orthodontic treatment.\nWith a view to help the patient avoid a course of root canal treatment to his upper FPMs, treatment involved the extraction of the upper and lower first permanent molars, together with upper and lower fixed appliances ().\nAlthough the timing of FPMs’ extraction is most favourable around a chronological age of 8–10 years old, this older case illustrates the advantages of FPMs’ extraction at a later stage. The extraction pattern in this case was dictated primarily because of the pain, large restorations and the questionable long-term prognosis of the upper FPMs, and because of the large occlusal restorations on the lower FPMs. Furthermore, extraction in the lower arch was indicated, because of the severity of the crowding and space requirements.\nIn this case, the extraction of FPMs increased the prognosis of the remaining dentition and relieved the signs and symptoms associated with upper FPMs. If the FPMs had not been of poor prognosis, the extraction pattern might have been the extraction of the first premolars. Although the response of the second permanent molars is variable following extraction of FPMs and acceptable positions would also be achieved irrespective of the timing of extraction [], a favourable mesial eruptive position of second permanent molars was predictable, to some extent, in this case due to a number of reasons:The upper second permanent molars were still within bone at the time of extraction; The OPT revealed signs of molar stacking of upper right and left second permanent molars, and distally tipped lower right and left second permanent molars reflecting posterior molar crowding; The distinct vertical growth pattern and presence of a steep mandibular plane, which encouraged molar mesial movement.\nMoreover, the OPT showed favourable positioning of third molars following space closure () Perhaps, in this case, if elective extraction of FPMs was prescribed around the optimum time, provided the third molars were radiographically detectable, the repeated restorative work might have been avoided. On the other hand, this approach could still be deemed cost effective, as it obviated the need for further dental treatment i.e., root canal treatment ().
A 54-year-old Japanese female visited an oral surgery clinic with a complaint of swelling in the inner region of the left cheek for the past one month. On clinical examination, a mobile swelling (size, 1 cm x 0.75 cm) with a clear boundary was observed on the left buccal mucosa. No associated pain was reported and the overlying mucosa was normal in appearance. The swelling was clinically diagnosed as benign buccal mucosa tumor. The tumor was excised under local anesthesia and was diagnosed as acinic cell carcinoma (AcCC) after histopathological examination. The margins were still positive for the tumor and further resection was advised. The patient reported to the Health Sciences University of Hokkaido Hospital for resection of the residual tumor two months after the initial surgery. Clinically, the patient was asymptomatic. The level 1B lymph nodes on both sides were palpable, bean sized, mobile, elastic, and soft. Intraorally, a surgical scar of about 7 mm was present on left buccal mucosa. There was no pain on pressure in the region of the scar (). The patient had a history of noninvasive ductal carcinoma (ductal carcinoma in situ [DCIS]; Tis N0M0) in the right breast, which was treated by excision and 57 Gy of radiotherapy five months ago. On investigation for oral lesion, no obvious abnormalities were detected on the computed tomography- (CT-) scan, contrast MRI, and ultrasonogram. Positron emission tomography- (PET-) CT did not suggest transition to and from any of the distant organs. The margin was resected under general anesthesia and sent for histopathological examination (). No relation to the parotid gland was found at the time of surgery.\nHistopathologically, the excised margin appeared as a fragmented tissue with no encapsulation. The tumor tissue was composed of cells with dominant microcystic structure with eosinophilic cytoplasm and eosinophilic secretory material. Papillary and tubular pattern of cell arrangement were also found but were limited to small area. A few vacuolar cells and some areas with hemosiderin deposition were observed. Furthermore, normal muscle tissue and atrophied salivary gland tissues were also seen ().\nThe secretory material was positive for diastase digested Periodic acid-Schiff (d-PAS), Mucicarmine, and Alcian Blue staining. No zymogen granules were found in the tumor cells. Immunohistochemistry (IHC) revealed strong positive reactions to vimentin, cytokeratin-19, and S100 protein. Mammaglobin was strongly positive, whereas discovered on gastrointestinal stromal tumors 1 (DOG-1) showed a negative reaction (). The histological sections of breast carcinoma were examined in suspicion of metastases; however, features of ductal carcinoma in situ that appeared completely different from those of buccal mucosa tumor were noted. Based on these histomorphologic and IHC profiles, the case was diagnosed as SC of the minor salivary gland in the buccal mucosa.
In the year 2000, a 30 year old, otherwise healthy male presented with pain, swelling and redness in his left third toe. Radiological findings showed a pathological fracture of the proximal phalanx of the third toe on the basis of an intra-osseous lesion with a spotted and sclerotic aspect. An MRI was made showing a clear abnormality of the proximal phalanx of the third toe with oedema of the bone marrow and minimal reaction in the adjacent soft tissue. There was no extra-cortical expansion. Osteomyelitis or Ewing's sarcoma could not be excluded. (Fig. )\nIn January 2001 a biopsy was performed. Histopathology showed cortical and cavernous bone tissue with reactive changes and damaged cells of which identification was not possible. The bone abnormalities were considered to be reactive in nature and the diagnosis was set as osteomyelitis.\nThe patient's complaints diminished spontaneously. No antibiotic treatment was prescribed. In April 2001, a control radiograph showed a normal structure of the third proximal phalanx of the left foot. The patient was discharged from follow-up in the outpatient clinic.\nAt the time of the second presentation, in 2008 with the patient being 38 y, the same symptoms occurred. They were present since six weeks. Physical examination showed clear swelling and tenderness of the left third toe. There was no ulceration.\nThe conventional anteroposterior radiograph (Fig. ) of the left foot showed a moth-eaten aspect of the proximal phalanx of the third toe with cortical destruction. There is an infiltration in, and swelling of the soft tissue surrounding the lesion. The head of the third metatarsal bone is intact.\nThe total-body bone scintigraphy showed high uptake of TC-99M in the left third toe and was suggestive of osteomyelitis. There were no other hotspots.\nThe clinical diagnosis was set at osteomyelitis and the patient was surgically treated as such. In august 2008 the proximal phalanx of the left third toe was surgically removed. Macroscopically there was a large mass in the surrounding soft tissue with calcifications. The findings were highly suggestive for a malignancy. Due do the initial diagnosis of osteomyelitis the surgical margins were intralesional.\nHistological examination followed. Microscopically there are large fields of atypical small blue round cells. The cells are highly undifferentiated and are very uniform in appearance. The nuclei are dark and round to oval, there is little cytoplasm. There is osteoid formation by the tumor cells. (Fig. ) The pathological diagnosis is small cell osteosarcoma of the phalanx.\nMetastatic disease was not found on a CT-scan of the thorax and abdomen. An additional MRI of the left foot was made in September 2008. This showed the initial post-operative situation. (Fig )\nFrom November 2008 the patient was treated according to the EURAMOS-1 trial []. In February 2009 additional surgery was performed. The third and second rays of the left foot were amputated. The histological specimen showed one small residual lesion in the soft tissue of the second web space. There was a good response to induction chemotherapy as shown histologically by a high rate of necrosis within the tumor. The patient was randomised in the EURAMOS-1 trial and treated with 4 cycles of Adriamycin and Cisplatinum. Methotrexate was abandoned after 2 cycles due to severe mucositis. At time of submission of this paper, the patient is living and well. At 6 months follow-up there are no signs of recurrent of systemic disease.\nIn retrospect, the initial abnormalities in 2001 are verified as infectious by the pathologist.
An 11-year-old male patient presented to our institution after sustaining trauma to his left lower extremity. On the day of presentation, there had been high winds in the vicinity resulting in the corrugated iron roof of a garage he had been walking nearby blowing off and landing on his left leg. The patient was trapped under the sheet of metal until freed by attending paramedics. In the emergency department, primary and secondary survey assessments of the patient revealed injuries to the knee and distal fibula only. A full formal examination of the knee was not possible due to pain and a tense haemarthrosis. Subsequent plain radiographs revealed an intra-articular avulsion fracture (), as well as fracture of the distal fibular diaphysis. A working diagnosis of displaced tibial spine avulsion fracture was made, and the patient was scheduled for surgery the following day to assess and fix the injury. Examination under anaesthesia revealed a ten-degree block to neutral extension of the knee and a positive Lachmann test with a soft end point. Arthroscopic examination of the knee revealed a lesion at odds with the preoperative diagnosis of tibial spine avulsion. A femoral end osteo-chondral avulsion fracture of the ACL was revealed (). Due to the unexpected findings and uncertainty about the optimal method of fixation in a skeletally immature patient, the fragment was reduced back into its normal position, the arthroscopic portals were closed, and the knee was splinted in extension in a plaster of Paris splint. With the benefit of hindsight, the treating team acknowledged that if a bone fragment this large had been avulsed from the tibial insertion of the ACL, this should have been reflected in the architecture of the tibial spine footprint on the preoperative X-ray. But as the appearances of this region appeared normal, this avulsion was unlikely to have occurred from the tibial end of the ACL, rather occurring from the less common femoral origin. A CT scan was subsequently obtained (), and the literature was scrutinised to aid preoperative planning of definitive fixation. Subsequent definitive fixation was perfomed nine days following the index procedure using a part arthroscopic and part open approach. Initial arthroscopic manoeuvres (including the use of a direct anterior portal in addition to the two standard portals) involved gentle curettage of the residual crater over the medial aspect of the lateral femoral condyle from where the ACL had been avulsed. Then two 2.4 mm Beath pins were passed in transosseous manner from the lateral supracondylar ridge into the centre of the defect (crossing the physis) using a posterior cruciate ligament targeting guide. 2 loops of 1 PDS suture were passed via these tunnels from the outside of the knee to the centre of the defect. Then a medial parapatellar approach to the knee joint was performed to formally expose the knee, two No. 2 Vicryl Bunnell sutures were passed across the femoral end of the ACL, and these sutures were placed into the PDS loops that had previously been passed into the knee. Pulling on the PDS sutures allowed the No. 2 Vicryl sutures to be passed through the tunnels previously drilled with the Beath pins and to be delivered to the lateral supracondylar ridge of the femur. The sutures were then tied to one another over a bone bridge thereby reducing the avulsed fragment back into its normal anatomical position (). Postoperatively, the patient was splinted in extension for the first four weeks. At this stage, the patient was placed into a range of motion knee brace (allowing up to sixty degrees of flexion) and allowed to partially weight bear with crutches. At six weeks following surgery, the patient's brace was slackened to allow a full range of motion. Radiographs performed nine weeks following definitive fixation showed union of his fracture (). He was allowed to ambulate free of his brace at this stage. At six-month follow up, the patient was able to ambulate without walking aids with a normal gait pattern and had a negative anterior drawer and Lachmann test and full restoration of knee flexion. No deformity or leg length discrepancy was noted at this follow up interval.
A 15-year-old right-handed female (VF) presented in 2012 with a history of weekly headaches over the right side of her head and with a sudden onset of diminished auditory acuity in her right ear when she was wearing headphones. She perceived music in the right ear as a noise rather than music. There was no history of developmental, psychological or school difficulties. Audiological assessment found normal neurological responses to sound stimuli bilaterally suggesting normal hearing function (10 dB at 1 kHz and 20 dB at 4 kHz). A subsequent brain MRI showed the presence of an expanded right mid-frontal gyrus with changes extending from the cortex into the subcortical white matter consistent with a possible low-grade tumour (Fig. ). Initially, VF was followed up with serial imaging until slight growth of the lesion was observed 14 months after diagnosis. She underwent a right-sided craniotomy and resection of the progressive right frontal lesion in February 2014. Histopathological analysis confirmed a WHO grade II diffuse astrocytoma.\nPostoperatively, VF reported profound retrograde amnesia for a range of memory components, in particular, autobiographical memory and semantic memory. For example, she could not recall her parents, life history or friends. She could not recall her way around school and reported loss of knowledge such as her time table or how to make a cup of tea. Imaging demonstrated a good radiological resection of the lesion with no evidence of surrounding brain injury (Fig. ). VF underwent no additional therapy following her surgery as follow-up imaging demonstrated a good resection and no tumour recurrence. Interestingly, her subjective hearing loss normalised after surgery.\nVF underwent comprehensive neuropsychological assessment at a month, 6 months and 18 months after surgery. VF reported no improvement in autobiographical memory; however, significant relearning of semantic knowledge took place and VF’s academic performance was found to be in line with expectations for her age. VF reported ongoing difficulties in attention, prospective memory, poor organisational skills and emotional lability. VF’s last assessment indicated general intellectual abilities, executive skills and memory abilities in the average range, demonstrating age-appropriate skills in new learning and laying down new memories. Significant difficulties were identified in attention (particularly sustained, switching and dual-task attention).
The patient was a 48-year-old woman who had occasionally felt pain and swelling in her left forearm since childhood, but she had not visited a clinic because the pain resolved naturally. She had no medication history or surgical history, and no relevant family history. She had felt increasing pain and numbness in her left finger for about 1 month, and she was referred to our hospital after the pain increased further.\nHer forearm were broadly distended () and the circumference of the left wrist was 1.2 cm longer than that of the right wrist. Grip strength was reduced by 11.4 kg on the left. Pain in the dorsiflexion of the wrist joint was noted, and the patient complained of numbness in the fingers. Electromyography showed a decreased amplitude of motor nerve conduction of the median nerve compared to the healthy side, but there was no delay in latency. Sensory nerve conduction velocity was undetectable on the affected side in the median nerve. The visual analogue scale for pain was 30/100.\nImaging findings showed no abnormalities on plain X-rays. MRI revealed hyperintensity on T2-weighted imaging and suggested that the vein had expanded from the proximal forearm to the palm (). The size of the lesion was 16 × 2.2 × 3.3 cm. The median nerve was compressed by the vascular malformation invading the carpal tunnel.\nPregabalin was given for pain, but there was no improvement. Resection of the vascular malformation was considered to be invasive and to have a risk of incomplete resection. Therefore, carpal tunnel release was performed as an alternative treatment. A skin incision was made from the distal forearm to the palm, and the flexor retinaculum was opened. The veins were present on the ulnar side of the median nerve () and were not touched during the procedure to avoid damage. Postoperatively, pain gradually diminished. One year after the operation, numbness of the finger disappeared and the patient was able to return to work. She has had no recurrence of symptoms due to the vascular malformation to date.
A 3-year-old Middle Eastern boy presented with a defect in the midline of his neck. He was born at full term by normal vaginal delivery and had no significance in his past medical history. There was no family history of congenital defects or consanguinity. The anomaly was located in the ventral midline of his neck (Fig. ). The superior aspect was composed of a skin tag leading to a short mucosa-like raw surface. Inferiorly, there was a sinus present with a greenish, thick residue occluding the opening. There was no contracture of the neck. He did not appear to be troubled by the lesion and a full examination was otherwise normal, except for adenoidal hypertrophy.\nHe had an MRI done elsewhere, indicating a soft tissue mass without any fistula tract. Despite contrast material being injected through the opening at the caudal end of the lesion, the diagnosis of MCC was established. No evidence of any other neck anomaly was found (Fig. ). The sinus, less than 1 cm in length, was found to extend caudally to the suprasternal notch. There were no attachments to underlying structures.\nA surgical removal and immediate closure with multiple Z-plasties were performed. Surgical removal was done with an incision 1–2 mm from the periphery of the lesion, deepened down to the supraplatysmal plane (Fig. ). During the surgery, the sinus at the caudal end of the lesion was probed and followed caudally until it ended, which was found to be approximately 2 cm long. This underdeveloped fistula tract ended right above the thymus gland. The cranial end of the defect had a fibrous band extending up to the mandible and this band was resected together with the cervical lesion. The midline lesion was found to be superficial and hence the excision was done at the subdermal level. A double Z-plasty was found to be sufficient for the closure. Closure was done with 5–0 vicryl interrupted sutures at the subcutaneous level and 6–0 rapid vicryl interrupted sutures for skin closure (Fig. ).\nA pathological examination of the specimen confirmed our clinical diagnosis. The findings were consistent with stratified squamous epithelial cells covering the cleft with few adnexial structures at the dermal level (Fig. ).\nOne month follow-up examination revealed an uneventful healing period, with redness along the incision scar and some nodularities, which were most probably due to the subcutaneous suture material. He was able to move his head in all directions without any restriction or pain (Fig. ).\nA 14-month follow-up examination showed an acceptable level of scarring causing no restriction of neck movements (Figs. and ).
A 10-year old African-American female with Down syndrome was evaluated by our pulmonary service for history of chronic daily cough and recurrent pneumonias for eight and a half years duration. Cough was worse at night, in supine position and during exertion. Cough also worsened during viral respiratory tract infections. There was history of frequent vomiting of undigested food but not necessarily associated with the cough.\nPatient was diagnosed with asthma exacerbations and pneumonia and treated as such several times in her lifetime. She had history of transient clinical improvement with antibiotics, bronchodilators and oral corticosteroids. Daily inhaled corticosteroids failed to completely control cough. Past medical history was significant for small ventricular septal defect and chronic constipation.\nOn examination, she was between 75th and 90th percentile for weight, and between 10th and 25th percentile for height. Chest examination was unremarkable. Chest roentgenograms showed predominantly patchy peribronchial air space consolidation with more involvement of the right middle lobe. A Barium swallow study was first done at 5 years of age. There was no frank aspiration or laryngeal penetration and the esophagogram revealed normal anatomy of the esophagus with mildly delayed hypopharyngeal contraction but no contrast retention.\nFlexible bronchoscopy showed normal airway anatomy. The BAL fluid contained 16% neutrophils, 35% lymphocytes, 35% macrophages with 14% percent epithelial cells. Cultures grew only mixed flora. Lipid laden macrophages were present with Colombo index of 110 which is consistent with aspiration[].\nCT scan of the chest showed diffuse lung infiltrates that seemed worse on right middle lobe and left lower lobe. The esophagus was enlarged along its entire length but with no obvious tracheal compression (figure ). In view of the CT scan findings, a repeat Barium swallow study was done which showed severe esophageal dysfunction with multiple waves of tertiary contractions throughout a markedly dilated thoracic esophagus. the distal esophagus appeared tapered caudally with severe narrowing at the gartroesophageal junction. Small food particles with air bubbles were seen within the mid esophagus. Only small amount of contrast passed to the stomach (figure ).
Our patient, a 58-year-old Caucasian male, presented to the emergency department with the acute onset of quadriplegia extending from C5 throughout the rest of the pan neuroaxis while awakening from bed the morning of presentation. Upon initial physical examination there were no signs of trauma noted that were significant to the presenting symptoms, additionally, patchy sensation was noted in the upper and lower extremities with clonus in the legs only and hyperreflexia in both arms and legs. Initially, our patient was administered one intravenous dose of methylprednisolone and allowed to enter a state of permissive hypertension which improved our patient's arm strength bilaterally to 2/5, but had no positive effects on the lower extremity paraplegia. As a result of minimal improvement, immediate imaging of the cervical spine was ordered and highlighted a severe cervical stenosis from C3 to C7, as seen in Figure , due to extra-axial posterior compressive spinal mass with cord signal change.\nAdditional imaging of the brain was completed as a precautionary measure and was normal. Upon obtaining full patient history and medical records from nearby hospitals, it was found that our patient was on a prolonged regimen of warfarin for many years due to a prior diagnosis of congestive heart failure without regulation and regular INR/prothrombin evaluation. Our patient also did not follow up with his primary care physician throughout the duration of the warfarin regimen. At presentation, our patient's INR was found to be 5.0 necessitating the prompt correction with intravenous injection of fresh frozen plasma (FFP) and vitamin K. Additional magnetic resonance imaging (MRI) of the thoracic and lumbar spine revealed large compressive acute epidural hematoma posteriorly compressing the thoracic spinal cord from T6 to T10, visualized in Figure , with cord signal change, as well as L4-S1 posterior acute epidural hematoma compressive of the cauda equine, visualized in Figure .\nUpon a complete review of the patient history, presenting symptoms, and radiological imaging results our patient was deemed a prime surgical candidate that would significantly improve both motor function, sensation, and ultimate restoration of our patient's quality of life. Our patient was emergently taken to the operating room for stage 1 of a two-part surgical procedure. Stage 1 involved a posterior T3 to L1 decompression, epidural hematoma evacuation and instrumented fusion. Three days later, our patient was taken to the operating room again for stage 2 of the planed procedure which involved a posterior cervicothoracic C2 to T2 decompression and instrumented fusion with evacuation of acute epidural hematoma.
A man aged 56 was included in the study. He complained about brief stabbing pain in the innervated right areas of the infraorbital nerve that had lasted for five years, and the pain was defined to be aggravated by the stimuli of eating, laughing, and being touched. The intensity of the pain was 5 out of 10 on the visual analog scale (VAS, with 0 indicating no pain and 10 the worst pain imaginable). He also suffered from infrequent exacerbations which lasted about half an hour and the pain intensity of which was 9/10 on the VAS.\nBefore consulting our pain service, both patients had also been assessed by the departments of neurology, otorhinolaryngology, and maxillofacial surgery. The patients had first consulted to the department of neurology, where they were clinically examined and their diagnostic tests were performed. Magnetic resonance imaging and facial computed tomography scans had also been performed, yielding no pathological findings. Both of them stated that they had never previously experienced any trauma and undergone any face or tooth operation. Then, consultation was asked from the departments of otorhinolaryngology and maxillofacial surgery. These departments were also not able to identify any pathology. Thereupon, the neurology clinic diagnosed these patients with idiopathic infraorbital neuropathy and pharmacological treatment was started, from which neither patient could benefit.\nWhen both these patients presented to our pain clinic, the case was as follows: the patients reported that they could not get rid of their pain although they received carbamazepine, diphenylhydantoin, and baclofen therapy. They also stated that it was confirmed by a dentist that they did not have any dental problems. The neurological examination of both patients revealed that the pain could be caused by the palpation of the nerve in the infraorbital notch. During the examination, no other concomitant data of interest were observed. It was concluded that, as the pains were in the region innervated by the infraorbital nerve, the patients had idiopathic infraorbital neuropathy. Consequently, by taking the condition of the patients into consideration, we had the same diagnosis with the neurology clinic. However, the patients had not benefited from the pharmacological treatment, which was the first option in such cases. Therefore, based on our previous experience with such patients [], we decided to perform alcohol neurolysis of the infraorbital nerve on the patients for the treatment of facial pain.\nWe administered 2 ml of 0.5% lidocaine hydrochloride over six times percutaneously to these patients. It was seen that the patients were free from pain until the local anesthesia lost its efficiency, but then the pain resumed at its usual intensity. Thus, in order to ease the pain definitely, the patients were recommended the application of neurolysis and informed on the side effects and complications that might occur. Then, upon the receipt of confirmations of the patients, application of neurolysis was started. After identifying the location of infraorbital foramen on the patient's face using thumb nail, a 22-gauge needle was forwarded down to the point where it touched the periost. In the infraorbital foramen, nerve, artery, and vein are found together. In order not to destroy the perfusion of this area, the needle was withdrawn 1 mm immediately after it touched the periost. After the negative result of the aspiration test, 2 ml of 0.5% lidocaine was injected. The needle was held up still for a whole minute until when the patient confirmed that the pain was gone, and then 0.5 ml of 50% ethanol was injected.\nFor the first patient, the same procedure was repeated 8 months after the first time, when the patient suffered from mild pain. For the second patient, however, the same procedure was repeated twice: one after six months and the other after a year as he still suffered from moderate pain. During the patients' clinical follow-up, they were scheduled for an evaluation once in six months for the following two years. Besides, they were advised to call in immediately in the event of any pain. At the end of the clinical follow-up, these two patients reported that they did not experience such pain any longer.
A 10-year-old boy presented to the emergency department (ED) one day after sustaining a closed right forearm fracture due to a fall. He was admitted in another hospital the day prior, where he underwent closed reduction and received an above-elbow slab. He was referred to our hospital due to the inability of the former to achieve a good reduction. Examination at our ED revealed intact vascularity and nerve function. No signs of compartment syndrome were evident in the form of excessive pain or tense swelling. Radiography showed malreduction (). Re-reduction and casting were successfully performed (). Assessment of the ulnar nerve post-reduction was not possible as the boy was uncooperative due to pain. The patient was discharged and scheduled for a follow-up visit in the outpatient clinic 2 days later. The visit was rescheduled, and he was seen 7 days after discharge.\nAt the follow-up visit in the clinic, the patient still complained of sharp pain. Physical examination showed no swelling suggestive of compartment syndrome, pulses and capillary filling were normal, and median and radial nerves were intact; however, signs of ulnar nerve palsy in the form of decreased sensation in the little finger and weak abduction and adduction of the fingers were present. Radiography showed loss of reduction. Redisplacement could be explained by the fact that the patient was 10 years old, and as described by the study by Bowman et al stating that reduction of both bone forearm fractures in children 10 years and older are more likely to fail. The patient was admitted and closed reduction with percutaneous ESIN under general anesthesia was performed. Two 2.5-mm elastic nails were used, retrogradely for the radius and anterogradely for the ulna. The postoperative radiograph is shown in .\nNo improvement in the patient’s sensory or motor deficit was seen after the surgery. As there were no available radiologists with experience in musculoskeletal ultrasonography in our hospital, and an electrodiagnostic study was not readily available, a non-contrast magnetic resonance (MR) image was obtained a day later, revealing a continuous right ulnar nerve without evidence of bony entrapment or surrounding edema. The patient was then discharged.\nOn follow-up 2 weeks post-operatively, the slab was removed, and radiography revealed excellent alignment. The patient was followed up on a regular basis ( and B). At the 5-month follow-up, the patient showed complete recovery of the ulnar nerve (normal sensation and range of motion, complete adduction and abduction of the fingers, and negative Froment’s sign). The elastic nails were removed 1 month later ().\nWritten informed consent was obtained from the patient for publication of this case report and accompanying images. No ethical approval is required for case reports as per university regulations.
A 22-year-old female of South Indian ethnicity with a 3-year history of acrofacial vitiligo presented for consultation in July 2013. She had been born, raised and lived exclusively in the United States. There were no previous medical concerns or history of colitis until asymptomatic areas of depigmentation appeared in June 2010 on the lateral side of the left eye. A few weeks later, symmetrical lesions were detected around the right side of the eye. Depigmentation progressed over a period of 6 months, both superiorly and inferiorly over both eyes, to converge at the medial aspect of the orbit. During this time, additional facial lesions were noted on the nose. Nine months later, lesions started appearing over the acral aspects of both upper and lower limbs and continued to progress. Review of systems was pertinent for the absence of any gastrointestinal or systemic symptoms. There was no personal or family history of celiac disease or of any other autoimmune disorders. The maternal grandmother and father had diabetes. Physical examination revealed extensive crenated depigmented lesions over the face and extremities with sparing of the torso (fig. ). There were no signs suggestive of other concomitant autoimmune disorders. Biochemical evaluation, including a complete metabolic profile, sedimentation rate, blood count and thyroid-stimulating hormone, did not indicate any thyroid or metabolic abnormalities.\nTopical therapy had been initiated within the first year with tacrolimus ointment, and subsequently calcipotriene, with no response, following a diagnosis of vitiligo by a local dermatologist. Topical steroids had been equally ineffective. Dapsone had been initiated at 100 mg three times a week with simultaneous phototherapy, after consulting a dermatologist with extensive expertise in vitiligo in India. The light therapy had been discontinued due to inconvenience and lack of response. Based on anecdotal improvement of vitiligo with elimination of gluten from other patients and the lack of any significant impact of conventional therapies, we advised her to try complete elimination of gluten from her regular diet to determine whether this would result in any potential changes. She was maintained on oral dapsone. Within 1 month, profound and significant repigmentation was evident (fig. ). Of note, the areas of repigmentation were distinctly darker than the native skin, which normalized over the subsequent month. Improvement continued for the next 3 months and maximal improvement was seen by 3 months, with no further repigmentation (fig. ). None of the other lesions showed similar changes or progress of the disease. The elimination diet was well tolerated, though restrictive, and compliance was not difficult to maintain once benefits were evident. Long-term follow-up is planned.
This British 12-year-old girl was originally referred because of a mild developmental delay particularly affecting her speech and language. She also had recurrent infections, failure to thrive and short stature.\nShe is the middle of three children born at term weighing 2.9 kg. Both her siblings were well and there was no contributory family history. The pregnancy was largely uneventful but she was noted to be small for dates. Her growth remained slow in the neonatal period although there were no particular feeding difficulties. She had an operation for bilateral inguinal hernia at 8 weeks and was extensively investigated for her failure to thrive (celiac screen, thyroid function tests and a cystic fibrosis screen were all normal). She was prone to recurrent respiratory tract infections and was found to have a mildly low IGA level. At 18 months she was noted to have a soft ejection systolic murmur which was investigated and proved to be a functional murmur with a structurally normal heart. Subsequently, at the age of 13 years, Patent Ductus Arteriosus (PDA) was diagnosed and surgically corrected. She was also noted to have an apparent convergent squint which remains under review. A formal assessment showed her to have no manifest squint with normal refraction and optic discs. At age 10 years she had a poplideal bursa cyst surgically removed from her leg.\nAt 8 months, her mother was concerned about her hearing as she was not babbling. The tympanograms were flat. She was due to have grommets inserted when the situation improved and she eventually passed a hearing test.\nHer motor milestones were normal: she sat at 6 months, walked at 14 months and had some babble at the age of a year. She was noted to have very few single words by 18 months and concerns regarding her poor speech and slow language acquisition remained.\nOn examination, she was a petite child with growth parameters between the 2nd and 9th centiles. She was not overtly dysmorphic but had a few distinctive features with slightly deep set eyes, narrow palpebral fissures, a small mouth and somewhat prominent low-set ears ().\nAssessment aged 6 years, at a time of continuing concern about communicative competence, with WISC 3 and CELF3 showed particular problems with all language based tasks, receptive score 70, expressive 59 but her performance and verbal IQ were 80 and 73 respectively (all tests mean 100 SD 15). The clinical finding was of “mainly expressive language difficulties but other problems that will affect all aspects of increasingly abstract reasoning and thinking.” She also showed over-anxiety, social anxiety, sensory sensitivities and some rigidity of behaviors.\nLater assessment (at age 12 years) using the WISC showed global learning difficulties (FSIQ64), CELF language assessment was in line with the IQ both receptively and expressively. Socially she was immature, in line with IQ and had no autism or ASD clinically or on ADOS.
A 79-year-old male with a history of chronic obstructive pulmonary disease, type 2 diabetes, chronic kidney disease, and persistent atrial fibrillation was brought to hospital after being successfully resuscitated following a brief pulseless electrical activity (PEA) cardiac arrest. During his convalescence, he had a second PEA arrest, from which he was again successfully resuscitated. Telemetry revealed atrial flutter with atrioventricular conduction that slowed markedly to ventricular rates as low as 34 beats per minute. A balloon-tipped temporary pacing catheter was floated in via left internal jugular vein until adequate ventricular capture was observed. Fluoroscopy was not used during insertion. A follow-up chest X-ray confirmed appropriate placement of the lead, which revealed the lead in the right ventricle with redundant lead slack forming a loop ().\nThe patient subsequently had a PPM implanted. The PPM was implanted without complication using a standard left cephalic vein access. The lead was advanced under fluoroscopic guidance. Implantation of the PPM lead was carried out without difficulty with active fixation to the right ventricular septal wall. The lead pin was attached to a pacemaker pulse generator which was then placed in a pocket located in the prepectoral plane below the left subclavicular fossa.\nThe TVP was then removed under fluoroscopic guidance. The large loop of redundant wire was noted, but it exited the cardiac silhouette without any resistance or interaction with the newly implanted PPM lead. However, significant resistance was noted when attempting to pull the tip of the TVP lead out through the introducer sheath. This resistance was only encountered when the TVP lead tip was at the level of the skin; fluoroscopy was not performed at this stage to determine the cause of resistance. The resistance was overcome and the TVP lead was successfully removed. It quickly became evident that the reason for the resistance was that the TVP lead had looped and formed a knot around the RV PPM lead at the junction of the left subclavian and left internal jugular vein. Continued traction had thus dislodged the RV lead tip from the endocardium, resulting in inadvertent removal of the RV lead through the left internal jugular vein (). No dislodgement of the PPM lead had been apparent fluorocopically during removal of the TVP wire from the cardiac silhouette as the ensnarement occurred at the level of the thoracic inlet when fluoroscopy was no longer being employed, and no loss of capture was noted as the patient was in their intrinsic rhythm. The patient remained hemodynamically stable.\nIn order to place a new RV PPM lead, left axillary venous access was obtained and a new lead was successfully placed, which was connected to the original pulse generator. The ensnared RV lead was cut within the pocket, and the lead pin was removed from the pulse generator directly. The external portion of the RV lead () was then removed without complication from the left internal jugular vein. The new PPM assembly functioned well, and the procedure was completed without any further complication.
A 56-year-old woman, with a past medical and surgical history significant for obesity (body mass index: 43), arterial hypertension, hysterectomy 15 years ago, and ovarian adenocarcinoma treated by ovariectomy and adjuvant chemotherapy 1 year ago, underwent elective uneventful total thyroidectomy, using cautery, and bilateal upper parathyroidectomy preserving the recurrent laryngeal nerves and inferior parathyroid glands by an experienced senior surgeon under general anesthesia. Thyroid vesicular adenocarcinoma (pT1N0M0) was confirmed after histological analysis. Tracheal intubation using a 7 mm tracheal tube was easy, and tracheal cuff was inflated with air until no leak was detected. Postoperatively, she was weaned from mechanical ventilation but an acute respiratory distress immediately appeared, requiring immediate endotracheal intubation of the patient's trachea and consecutive mechanical ventilation. A left recurrent laryngeal nerve paralysis was diagnosed and confirmed by a postoperative ENT check. The patient was admitted into intensive care unit, and a systemic corticotherapy (1 mg/kg/day) was introduced. A new attempt of ventilatory weaning was performed 4 days later with a second acute respiratory distress and a new endotracheal intubation. On the sixth postoperative day, the patient presented a productive cough, a temperature of 38.2°C, and a suspicion of wound infection of the operative site and developed rapidly an increasing emphysema of the neck and the upper torso. Computerized tomodensitometric cervical and thoracic examination showed a pneumomediastinum and a leak from the anterior part of the trachea secondary to localized tracheal necrosis confirmed by fiberoptic bronchoscopy. Prompt surgical exploration of the neck revealed a necrotic anterior part of the trachea involving the second to the fifth tracheal rings and a perforation of the third and fourth rings (). The lateral parts and the posterior membranous part of the trachea were intact. The necrosis of the tracheal wall was not located in the area of the cuff extending. The necrotic trachea was excised, leaving a large anterior defect that spanned 4 rings (). There was an inability for surgeons to perform a sleeve resection with primary anastomosis, a muscle flap reconstruction of the anterior trachea, or a partial tracheal closing around a Montgomery T-tube or tracheotomy canula because there was a too high risk causing the acute septic process to spread and because the patient presented both life-threatening cardiovascular and respiratory failures. The endotracheal tube was left long, and the cuff was advanced beyond the tracheal necrotic area. At the time of the first aggressive tracheal and cervical wound debridement, a vacuum-assisted closure device (VAC therapy) was used to manage the wound (). A polyurethane sponge cell foam dressing ((VAC GranuFoam), recommended for stimulating granulation tissue and wound contraction, was cut to be slightly smaller than the wound (), placed directly onto the wound (), and loosely covered with a clear occlusive drape to encompass the entire wound (). A separate small piece of foam was place in the defect to keep a hole to be refashioned into a formal tracheostomy allowing the later insertion of a tracheotomy canula (Figures and ). Placing the drape loosely allowed the suction to create a tight seal along the perimeter of the wound and decreased periwound maceration. The suction tubing was placed into the sponge at the end of the application of the dressing () and covered with an additional piece of OpSite (Smith & Nephew), making sure to pinch the drape around the tubing to create a complete seal (). The occlusive dressing was applied lightly over the sponge and wound, trying to lay the adhesive dressing down like a blanket instead of a tent. This helps to reduce any free air spaces which can make a complete seal difficult. This step over irregular surfaces such as the neck often requires multiple sheets of dressing. Using a scalpel, the occlusive dressing and the outer portions of the sponge were incised to facilitate insertion of the VAC line. The dressing was then connected to a vacuum pump (VAC ATS), providing a continuous subatmospheric pressure setting to 75 mm Hg. Bacterial examination of the cervical damaged area revealed strains of Escherichia coli, Streptococcus C, and Staphylococcus aureus. Antibiotics were administered for 3 weeks. The dressing change interval at our center was 72 hours and was performed in 15 to 20 minutes. Each time, the dressing was changed at bedside and the wound was debrided to decrease the amount of fibrotic and nonviable tissue (). The surrounding tissue was cleaned and completely dry until granulation tissue partially covered tracheal structures (). There was no complication associated with utilization of VAC. After two weeks of VAC therapy, 3 debridements, and dressing changes, there was no evidence of ongoing infection and the trachea was partially closed around a no. 7 tracheotomy canula. The use of the VAC therapy improved the soft tissue component of the neck wound and likely impacted the tracheal wound. The patient was discharged with the canula in place for 3 months. An endoscopic examination was then performed showing a complete closure of the trachea around the tracheotomy canula, no sign of local infection, tracheal stenosis or malacia, and no more left recurrent laryngeal nerve paralysis. The tracheotomy canula was then removed. At 2 years, the patient is well with no tracheal symptoms. There is no immobilization of the trachea but the resultant cervical skin scar-tissue adheres to the trachea over two inches in height.
Patient 1 was a 51-year-old Caucasian male who underwent wide excision of a FDCS of the duodenal wall with negative margins. This was a spindle cell malignancy with immunohistochemistry positive for CD21, CD23 and vimentin and negative for CD20, CD34, CD35, CD117 (c-kit), DOG 1 and smooth muscle actin. EGFR expression was negative by immunohistochemistry and mutation analysis was wild type. One year after surgery, he was diagnosed with multiple liver metastases confirmed by biopsy and received six cycles of CHOP chemotherapy at another medical center with partial response. However, four months after completing CHOP he had marked worsening of hepatic metastases with numerous lesions affecting all hepatic segments up to 7 cm diameter. Prior to transfer of care to our center, he went untreated for two additional months during which time liver metastases coalesced with the largest becoming 15 cm in diameter, but the lungs remained clear. He had moderate right upper quadrant pain and fullness, but surprisingly no constitutional symptoms such as fatigue, anorexia or weight loss. Due to limited tissue availability, only two genes were sequenced to determine potential clinical trial eligibility, and both BRAF and PI3KCA were wild type as has been reported in FDCS. He received gemcitabine by fixed dose rate infusion and docetaxel since he had already approached his lifetime limit of doxorubicin. After three cycles, he achieved a RECIST 1.1 partial response with the largest hepatic metastasis decreasing from 15 to 9.5 cm diameter, the second largest decreasing from 10.3 to 7.0 cm and symptomatic improvement. After six cycles, the hepatic metastases continued to respond with the largest mass decreasing to 6.4 cm and the second largest to 5.9 cm. Docetaxel was discontinued after six cycles due to lower extremity edema and bilateral pleural effusions, which subsequently resolved. Gemcitabine was continued for a total of 12 cycles with the largest hepatic mass decreasing to 5 cm and the second largest to 4.7 cm. Performance status and quality of life remained excellent throughout treatment, and a RECIST 1.1 partial response is continuing at the time of this report.
A 46-year-old Javanese woman presented with urinary incontinence following an abdominal hysterectomy with bilateral salpingectomy 3 months earlier. She is a housewife with no history of routine drug use and no prior history of hypertension, diabetes, allergies, or other chronic disease. She does not smoke tobacco and does not consume alcohol.\nA physical examination revealed that her general condition was good and her vital signs were: blood pressure 112/74 mmHg, heart rate 89 beats per minute, respiratory rate 18 times per minute, and temperature 36.6 °C. There were no abnormalities in her chest and abdomen, or in musculoskeletal and neurological examinations. In a genitalia examination using a speculum, we identified fistulae above her vagina wall that were 1 cm in size. All laboratory findings (that is, complete blood count, liver functions, renal functions, and urine analysis) were within normal limits.\nAfter discussion with our patient regarding the risks and benefits of an open abdominal procedure and laparoscopic approach, we discussed the similarities and differences between the two procedures were her. We chose surgical management using laparoscopic approach with the considerations that it could facilitate precise dissection, offer good visualization, and be minimally invasive, thereby enabling faster recovery.\nOur patient was placed in the lithotomy position and received general anesthesia. A cystoscopy was performed to confirm the fistulae orifice and a stent was inserted into the fistulae tract from her bladder to her vagina. A tamponade was inserted into her vagina up to the vaginal apex, to be able to identify the vagina and prevent loss of pneumoperitoneum. A transperitoneal approach was performed with trocars distributed as follows: The camera was placed through a 12 mm port with 30° down lens located superior to the umbilicus. Two ports for the surgeon were placed on the right side (Fig. ).\nShe had adhesions; therefore, adhesiolysis was performed, using a combination of sharp and blunt dissection to expose the vaginal stump and the superior aspect of her bladder (Fig. ). A simple cystotomy was performed and extended to include the fistulae site, and the fistula tract was excised until viable fresh tissue was exposed. Later the defect was repaired by using a running stitch of 3–0 Vicryl. Knots were tied intracorporeally. A second layer of closure was performed in an imbricating fashion with the same suture. The vagina defect was not closed separately but covered with an omental flap (Fig. ). A vascularized omental flap was made using a scalpel, which was placed in the plane of dissection between her bladder and her vagina, and it was secured with two attachment points.\nThe ureteral stents were removed without difficulty. A urethral catheter was placed for adequate postoperative urinary drainage. This procedure takes approximately 2.5 hours; the estimated blood loss for our case was minimal and there were no intraoperative complications.\nOur patient was given intravenously administered ceftriaxone 1 gram per 12 hours postoperatively for prophylaxis and orally administered diclofenac for pain control on an as-needed basis from the following day. At postoperative day 1, she was able to eat as usual and complained of minimal abdominal pain during mobilization. The surgical wound was good and there was no urine leakage from her vagina. After that, she was discharged while still using urethral catheter for adequate postoperative urine drainage for 2 weeks.\nShe returned for a follow-up 2 weeks after surgery and reported that she experienced no recurrent incontinence and urination was normal. She continued to do well at 1-month, 3-month, and 6-month postoperatively.
Patient 2 (40–45 years old) presented with severe agitation. He was threatening, screaming, scratching, and spitting, and refused a conversation. He thus showed a similar clinical picture as patient 1 but also appeared to experience auditory hallucinations and to actively talk to them. Patient 2 was in a state of poor hygiene. Taking into consideration his manner of response, one could assume that patient 2 has experienced psychiatric treatment in the past. When the nurse asked him if he had any experience with psychiatric medication, he yelled at her and clarified his wish to refuse haloperidol. He seemed to feel especially threatened by the police and the psychiatric staff, not only due to psychotic symptoms but also due to previous aversive experiences with psychiatric treatment. Once again, the staff members involved in the situation discussed the clinical case in a multiprofessional setting and weighed out every possible option. The team suspected that patient 2 suffered from an acute exacerbation of a disorder that persisted for a longer period of time or a psychotic relapse. In this case, the team decided that patient 2—due to his previous aversive experiences—would have extraordinarily suffered from compulsory treatment, which may exacerbate previous traumatic experiences. Also, he calmed down a bit when given a space to withdraw and did not immediately endanger himself or others; however, he remained tense for several days and threw objects whenever members of staff tried to engage him in a conversation or offered oral medication. When left alone, he did not appear aggressive or present improper handling, showed a regular food intake, and welcomed the possibility to smoke. Somewhat later, he was seeking a medical consultation and expressed the need for a low-dosage medication. To this day, 6 years later, he regularly receives outpatient care and short-term crisis intervention treatment on a psychiatric ward, although he has felt threatened and deprived of his identity by the state and the psychiatric system of another city for more than 25 years.
A 29-year-old male was involved in a motorcycle collision. He was immediately brought to a level-one trauma center for advanced trauma workup. Examination showed no gross deformity to the extremities or pelvis and no significant ecchymosis or edema of the pelvic region. Immediate assessment revealed no obvious concern for neurovascular injury.\nSoon after arrival, the patient began experiencing hemodynamic instability and received three units of packed red blood cells. Initial pelvic radiographs were not performed as the patient went straight to computed tomography (CT) per the trauma protocol at our institution. CT scans showed active arterial extravasation into the pelvis and he remained unstable, prompting immediate transfer to interventional radiology. Embolization of bilateral anterior internal iliac arteries, in combination with a resuscitative endovascular balloon occlusion of the aorta, was successful in halting bleeding and reestablishing hemodynamic stability. CT scans of the pelvis also demonstrated a pelvic ring disruption consistent with an anteroposterior compression (APC) type II injury (Figure ).\nBy the time the patient was available for orthopedic consultation, he was hemodynamically stable and additional pelvic radiographs were obtained. It was felt that as the reason for internal bleeding had been addressed and his hemodynamic parameters had stabilized, external fixation was not necessary. He was deemed fit for open reduction and internal fixation of his pelvis three days later after adequate resuscitation. Additionally, on retrospective evaluation of the CT scan, there was evidence of left testicular dislocation that was not read as such by the radiologist and not identified on exam (Figure ). It was difficult to assess the viability of the testicle on CT.\nNext, the patient was brought to the operating room and placed supine on the table. Further clinical examination was performed under anesthesia, with no significant external edema, ecchymosis, scrotal injury, and open wounds noted. He was then prepped and draped in sterile fashion. The anterior pelvic ring was exposed utilizing a Pfannenstiel approach. After dissecting through a midline rectus split, significant diastasis of the pubic symphysis was clear. Disruption of the left rectus abdominus muscle insertion on the pubic tubercle was observed and remaining abdominal muscles were elevated off the pubic rami bilaterally. The patient’s left testicle was discovered extruding from the scrotum adjacent to the pubic symphysis (Figure ).\nThis prompted immediate urology consultation. Appearing fully intact and highly mobile, the testicle was protected, and pelvic stabilization continued while awaiting urology. Anatomic reduction was achieved utilizing a Jungbluth clamp and two screws on either side of the pubic symphysis through a six-hole 3.5 mm pubic symphysis plate (Synthes; West Chester, PA, USA). After final alignment, all three screws on either side of the plate were filled.\nAttention was turned to the bilateral sacroiliac joint widening. O-arm and Stealth navigation systems (Medtronic; Minneapolis, MN, USA) were utilized to achieve percutaneous stabilization. After guidewire positioning in both S1 and S2 sacral segments, three 7.3 mm cannulated screws were used to stabilize the posterior ring (Synthes; West Chester, PA, USA) (Figures ).\nAfter satisfactory reduction and stabilization was confirmed with fluoroscopy, urology successfully reduced the testicle to the hemiscrotum and secured it with vicryl suture. The urogenital structures were without compromise and the testicle and spermatocord appeared viable. The anterior wound was thoroughly irrigated, 2 g of vancomycin powder were placed deep in the pelvis, and the wound was closed in layers.\nFollowing five weeks in a skilled nursing facility, the patient was discharged home with the assistance of home health and physical therapy. During his first follow-up visit, the patient reported continued pelvic pain, while denying urogenital complications including sexual dysfunction or difficulty voiding. Pelvic radiographs confirmed a well-aligned pelvic ring without evidence of hardware breakage or loosening. The decision was made at this point to progress his non-weight-bearing status to as-tolerated.\nThe patient had his final follow-up at 13 months and was complaining of bilateral hip discomfort but minimal pain anteriorly or posteriorly in his pelvic ring. Repeat radiographic evaluation demonstrated fracture union with maintenance of reduction and no evidence of hardware failure (Figure ).\nSpecific etiology of his bilateral hip pain was unclear at the time of follow-up but did not appear to be directly related to his pelvic ring fixation as this had healed uneventfully. He was likely significantly deconditioned and noted persistent psychosocial stressors which may explain some of these complaints. The scrotum and external genitalia remained intact at follow-up, without issue. He continued to deny sexual dysfunction or genitourinary complaints, suggesting that complications due to his associated testicular dislocation had been avoided.
A 4-year-old girl was previously diagnosed with asplenia, single right ventricle, and supracardiac TAPVC. She underwent pulmonary artery banding at the age of 1 month, followed by a bidirectional Glenn procedure and TAPVC repair at the age of 5 months. She did not demonstrate symptoms of PVO, and there was no evidence of delay in the excretion of contrast media in cardiac catheter examination before Fontan procedure. Extracardiac Fontan palliation was subsequently performed at the age of 19 months. At the age of 3 years, although right lower PVO occurred because of the compression of the vertebra, she did not demonstrate symptoms of PVO (). At the age of 4 years, she underwent a cardiac catheterization to evaluate her hemodynamic parameters after Fontan procedure (). She was taking oral aspirin, warfarin, and enalapril. After a routine hemodynamic assessment, NO and oxygen tests were performed. A pigtail catheter was placed in the single ventricle, and a wedge catheter was positioned in the pulmonary artery. We performed the pulmonary vasodilator examination in the same way as when doing the examination in patients with pulmonary hypertension []. Subsequently, 20 ppm NO was administered via face mask. After 5 minutes of NO administration, her hemodynamic parameters were measured. Following a washout period of 5 minutes, 100% oxygen was administered, and after 5 minutes of oxygen administration, her hemodynamic parameters were again measured. To measure the pulmonary arterial wedge pressure and pulmonary arterial pressure accurately, the waveform was monitored carefully (Figures and ). After the administration of NO, the single-ventricle end-diastolic pressure, bilateral mean pulmonary arterial pressure, and cardiac index did not change. Oxygen saturation did not change at 95%, and pulmonary resistance index decreased from 2.1 to 1.0 Um2. However, the right lower pulmonary arterial wedge pressure increased from 8 to 12 mmHg, and the left lower pulmonary arterial wedge pressure increased from 7 to 9 mmHg. The cardiac index remained almost the same. Similarly, following the administration of oxygen, the single-ventricle end-diastolic pressure did not change; however, the right lower pulmonary arterial wedge pressure increased from 8 to 14 mmHg and the left lower pulmonary arterial wedge pressure increased from 7 to 12 mmHg. The bilateral mean pulmonary arterial pressure increased from 13 to 15 mmHg. Although angiography was performed after the inhalation of NO and oxygen, there was no evidence of left pulmonary venous obstruction or delay in the excretion of contrast media (Figures and ). A systemic-to-pulmonary shunt was also nonexistent. On echocardiography, there was no acceleration of blood flow at the left pulmonary vein or surgical anastomosis between the pulmonary venous confluence and the atrium. There was also no stenosis of the atrioventricular valve. Despite the inhalation of NO and oxygen, there was no acceleration of blood flow, and stenosis was not identified at the surgical anastomosis between the pulmonary venous confluence and the atrium ().
A 45-year-old female presented at the Oral and Maxillofacial Surgery Department of a public hospital in the city of Recife, Brazil, complaining of an increase in volume on the right side of her face and being unable to open her mouth adequately for more than a year. She reported not having suffered facial trauma or infections in the region. The extraoral examination revealed an increase in volume of the right zygomatic arch and palpation revealed hard tissue mass [], which was not evident in the intraoral inspection. The patient had limited mouth opening (8 mm), with an inability to perform protrusive or lateral movements. No pain symptoms were reported.\nThe tomogram revealed altered anatomy of the coronoid process, with a radiopaque mushroom shaped characteristic developing in an upward and lateral direction, invading the infratemporal space and causing deformity of the zygomatic arch. The upper region of the tumor had a growth on the zygomatic arch exceeding the limit of the infratemporal cavity and causing limited mouth opening with the inability to perform excursive movements. The lateral growth had caused deformity and fracture of the right zygomatic arch. The fracture line was evident in the three-dimensional reconstruction [].\nOn the basis of the clinical and imaging features, a preliminary diagnosis of pseudo-ankylosis of the right coronoid process was established. The patient was hospitalized and underwent a coronoidectomy under general anesthesia. Osteotomy of the coronoid process was performed using an intraoral incision. The removal of the fibrous tissue between the tumor and zygomatic arch and excision of the tumor were performed using a coronal incision [].\nThe specimen measured about 3 cm at its largest diameter and had a shape similar to a mushroom []. The microscopic analysis revealed sclerotic, lamellar and dense bone similar to the cortical bone, allowing the diagnosis of osteoma of the mandibular coronoid process [].\nPostoperative recovery was satisfactory. No occlusal changes were observed and mouth opening had increased to 25 mm. Signs of injury to the temporal branch of the facial nerve were also observed. The patient was referred to a physiotherapist for the recovery of the full range of mandibular movements and the stimulation of nerve function. After 1 year of clinical and X-ray follow-up, the patient had experienced recovery of her normal facial contour, full recovery of nerve function, the return of excursive mandibular movements and maximum mouth opening had increased to 37 mm [Figures and ]. Moreover, there were no signs of recurrence.
A 46-year-old African-American man presented with an enlarging, tender, left-sided neck mass. The initial evaluation consisted of magnetic resonance and computed tomographic (CT) imaging that demonstrated a 7-cm mass with T1 signal and T2 signal contrast enhancement []. On the CT scan, the mass was revealed to be hyperdense. A presumptive diagnosis of nonsecretory paraganglioma was made after catecholaminergic products were absent in serum and urine analyses. Given the size and progressive growth of the mass and symptomatic status of the patient, resection was planned.\nAfter informed consent was obtained, digital subtraction angiography and balloon occlusion testing were performed. Angiography confirmed a 7-cm vascular tumor with very thin vascular channels arising directly from the walls of the common and internal carotid arteries. There was no pedicle large enough to be suitable for embolization []. This pattern was unusual because paragangliomas are extremely vascular with very well developed vascular channels that usually arise off the external carotid artery and have a pattern of arborization that is highly amenable to endovascular embolization. In this case there was the appearance that the vascular channels were direct offshoots of the vasa vasorum of the affected vessels. In addition, there was irregularity and dilatation in the region of the carotid bulb suggestive of carotid wall involvement, which is possible but rare in paragangliomas. The patient tolerated the balloon occlusion test; and because the tumor was considered to be invading the wall of the carotid artery, we elected to endovascularly sacrifice the internal carotid artery (ICA) in preparation for surgical resection. The purpose of this sacrifice was to starve the tumor of its blood supply and minimize the degree of intraoperative bleeding. The endovascular sacrifice of the ICA was accomplished without incident. One-week later, the patient was taken to the operating room for resection.\nSurgical exposure of the carotid sheath revealed a large mass in the region of the carotid bifurcation. Exposure from the skull base to the omohyoid muscle allowed sequential separation of the jugular vein and the vagus, accessory, and hypoglossal nerves from the mass. The entire carotid bifurcation was dilated without separation of planes between the internal and external carotid arteries, carotid bulb, and tumor. The common, internal, and external carotid arteries were subsequently ligated away from the tumor; and the tumor and neighboring arterial segments were resected en bloc and sent for pathologic examination. Four distinctive lymph nodes appreciated during neck dissection were additionally submitted for examination. After resection of the mass, the sympathetic chain ganglia below the carotid sheath became apparent and appeared uninvolved.\nThe patient woke up without deficits; however, on the second postoperative day, he developed mild hoarseness and swallowing difficulty with an enlarging neck mass. He was taken emergently for the evacuation of a neck hematoma. No active bleeding sites were noted. Thereafter, the patient made an uneventful recovery and was discharged home without evidence of cranial neuropathies.\nGross examination of the en bloc specimen revealed a segment of artery surrounded by a mass measuring 4.8 cm × 4.0 cm × 2.4 cm. The tumor had a tan, myxoid surface with yellow tissue, and hemorrhagic changes next to the thrombosed arterial segment. Microscopic examination revealed a large circumscribed cervical lymph node with a large elastic artery coursing through it. The lymph node showed distinctive features including follicles composed of concentric rings of mantle zone lymphocytes around germinal centers as well as prominent vascularity of the germinal centers, often showing a single penetrating arteriole [Figure and ]. Immunostaining patterns from the lymph node revealed CD23, cyclin D1, BCL2, CD20, CD3, CD138, CD56, kappa/lambda, CD30, and CD15, which excluded the diagnosis of lymphoma; and Congo red staining excluded amyloidosis. Additional adjacent benign lymph nodes submitted for microscopic examination showed some of the above-mentioned features of Castleman's lymphadenopathy.
This is the case report of a 24-year-old female patient, who is para three with a previous history of one cesarean section in her second delivery, after which she had a copper T 380A IUCD insertion in a family planning center, with no recorded complications. A follow-up abdominal sonography done after 1 month confirmed that the IUCD was inside the uterus in its correct position. Surprisingly, during her checkup for period irregularity 9 months later, another sonography was performed and showed the presence of a 7-week intrauterine gestational sac along with the IUCD inside the uterine cavity. The patient was offered removal of the IUCD, but she preferred to complete the pregnancy despite its presence. Her pregnancy went smoothly and without complications; the only mentioned symptom was an increased frequency of vomiting in the past 3 months in comparison with the previous two pregnancies. Knowing that the IUCD was difficult to be identified in the second half of her pregnancy, however, the IUCD was not retrieved at the time of delivery. Accordingly, the patient was investigated to find the IUCD which was confirmed to be in the right iliac fossa outside the uterine cavity by a plain abdominal X-ray [].\nAs long as the patient was asymptomatic, the plan was to do a laparoscopic surgery after complete uterine involution. The surgery was done as a classical laparoscopic procedure, with main entry through umbilicus, two ancillary ports on the left side and one on the right side for the assistant. Exploring the loop of bowel that was adherent to uterine fundus aided in finding the IUCD thread. Removal of adhesions between bowel and uterus was first done, after which pulling the thread of the IUCD resulted in retrieving the vertical arm, and showed that the horizontal arms were inside the cecum []. The IUCD was pulled out, and primary closure of the cecum was achieved, using four interrupted stitches by vicryl 0–2 [].\nThe surgery went smooth, and the patient was discharged 2 days later without any complication.
A 21-year-old female patient attended the Department of Conservative dentistry and Endodontics, 2 days after an automobile accident. The patient had multiple lacerations on the face and fractured crown. After the general medical, dental, and traumatic incident histories were reviewed, clinical and radiographic examinations were conducted. A complicated crown fracture was observed in relation to left maxillary central incisor, tooth no. 21 (). The crown fragment was mobile but still attached on the palatal surface by the junctional epithelium and connective tissue. The patient was in severe pain as a result of a large pulp exposure. Adjacent teeth had slight mobility with redness at the marginal gingiva suggestive of some tooth displacement. Radiographic examination revealed horizontal fracture line at cervical third of the crown in relation to tooth no. 21, and no root fracture (). A detailed explanation about the treatment plan was given to the patient, including emergency root canal treatment, reattachment of the fractured crown, splinting of the anterior teeth, followed by reinforcing the attachment by fiber reinforced post. The treatment plan was accepted by the patient and informed consent was obtained.\nLocal anesthesia was administered and fractured segment was gently removed and stored in saline. One visit root canal treatment was performed. The fractured segment was reattached as described in case report 1. Since adjacent teeth had mobility, they were splinted with 19-gauge stainless steel wire and light cure composite resin (). At the following visit the canal was prepared to receive the fiber reinforced post, by removing the obturated gutta percha till apical third. The canal walls were etched, rinsed, dried, and treated with bonding agent and fiber-reinforced post was cemented into the canal as described in case report 1 (). The patient was advised to report after a week for the removal of the splint (). Patient was recalled after 1 month for followup. The patient had no signs or symptoms and wanted to go for orthodontic consultation. One-year followup showed stabilization of the fragment, and orthodontic treatment in progress ().
A 21-year-old man presented with fever, weight loss of 6 kg, myalgia, and polyarthralgia for 3 months. There was no response to anti-inflammatory drugs. He had a good relief of symptoms with an oral medication (possibly steroids) for 2 months, but as soon as he stopped it; a month ago, he developed additional symptoms of cramp and pain in both feet while walking. The pain used to subside with rest but used to recur again on walking the same distance, suggestive of claudication. His sleep was disturbed due to burning pain in both the forefeet, which used to decrease on dangling the feet but used to increase on raising the legs. The symptoms had markedly increased for last 10 days with continuous burning pain, coldness of feet, and inability to walk.\nAt this time, he presented to the emergency department of our hospital. His blood pressure at presentation was 150/100 mmHg. Both feet were cold, pale, and had absent pulses (bilateral posterior tibial and dorsalis pedis). There were few skin infarcts on the left sole. There was no toe gangrene. He was a nonsmoker and had no history of oral or genital ulcers. He had a history of left testicular pain 20 days back which lasted for 2 days. During that period, minimal free fluid was present in tunica vaginalis on ultrasound. The pain subsided spontaneously and did not recur.\nThus, the patient had constitutional symptoms that responded to corticosteroids. This was followed by a subacute involvement of distal arteries of both the lower limbs manifesting as foot claudication. He finally presented to us with an episode of acute on chronic limb ischemia. Doppler ultrasound of the lower limbs arterial system showed complete occlusion of anterior and posterior tibial and peroneal arteries in both the legs with multiple collateral channels. Digital subtraction angiography (DSA) of the lower limbs and abdomen visceral arteries showed symmetrical narrowing and irregularity of the mid and distal part of superficial femoral artery [] and popliteal artery with delayed contrast opacification, narrowing and occlusion of anterior tibial, posterior tibial, and peroneal arteries on both sides []. There was no aneurysm or stenosis of abdomen visceral arteries. Based on 1990 American College of Rheumatology classification criteria,[] the diagnosis of PAN was made. He was treated with intravenous methylprednisolone (1 g daily) pulse for three consecutive days and had a marked relief in pain by the 2nd day. He had a gradual resolution of other symptoms and started walking by the end of a week. Urine examination was normal. Erythrocyte sedimentation rate was 61 mm at 1 h, C-reactive protein was 21.4 mg/dl, and platelet count was increased to 419,000/m2. Antinuclear antibody, antineutrophil cytoplasmic antibody, antiproteinase-3, and antimyeloperoxidase antibodies were negative. Serum complements were normal. Hepatitis-B surface antigen, antihepatitis-C antibody, and HIV by ELISA were also negative. Further, he was treated with pulse intravenous cyclophosphamide and oral prednisolone as per the EUVAS protocol.[] He had no tissue loss of the toes and his claudication pain subsided. The skin infarcts healed with a scar. He was followed up regularly and was in remission on azathioprine. Prednisolone was tapered and stopped after 1 year.
Index patient, a 25 years old female hailing from rural West Bengal, India, was referred to Psychiatry unit from Medicine inpatient department of our institute with complaints of mood symptoms for last one month. Further review of referral notes in case record file revealed a provisional diagnosis of steroid induced Cushing’s syndrome by Medicine inpatient unit. History of her past illness did not reveal any medical and psychiatric illness. On enquiry, she reported that she was taking oral dexamethasone 0.5 mg twice daily for last 2 years as over the counter (OTC) medicine without any prescription. She elaborates that she was too thin to get married. She started taking medication as a way to put on weight to increase her credibility for marriage. She was told regarding dexamethasone as an effective medication to increase body weight by her peers. One of her friends also used to accompany her in doing so. Her weight increased. She did not report any mood symptoms during the period she was taking dexamethasone. As days progressed, she noticed she became overweight. Her skin got discolored at places. She started to have pain and burning sensation in abdomen. Her sleep was disturbed with erratic appetite. Due to these, she stopped taking dexamethasone and attended Medicine outpatient unit on the following day. Her physical examination revealed cushingoid features. She was admitted at Medicine inpatient unit and was treated accordingly. Dexamethasone was restarted at a dosage she was taking and was tapered over a period of two weeks. Her cushingoid features started to improve but she became more irritable with crying spells. For these she was referred to psychiatric unit for management. She complained of irritability, loss of interest in all works, tiredness throughout the day, disturbed sleep, burning sensation in abdomen. Mental status examination revealed depressive affect with prominent anxiety symptoms, hopelessness and idea of guilt. Neither suicidal ideas nor psychotic symptoms could be elicited. A diagnosis of severe depressive episode without psychotic symptoms (F32.20) and abuse of non-dependence-producing substances (F55.5) were made. Her mood symptoms were considered as a part of steroid withdrawal syndrome (SWS) though it could not be confirmed with biochemical investigation. Escitalopram was started 10 mg/day with gradual tapering benzodiazepines. She was scheduled for a follow-up visit after 3 weeks. After 10 days she came back to psychiatry outpatient department (OPD) with complaints of increased burning sensation in abdomen without minimal improvement of her mood symptoms. As gastrointestinal (GI) irritation is a common side-effect of all selective serotonin reuptake inhibitors (SSRIs), escitalopram was stopped. Mirtazapine was chosen for its GI friendly characteristics. It was started 7.5 mg/day and then gradually titrated up to 22.5 mg/day over a period of 4 weeks. She was advised for 1st follow-up visit after 3 weeks. After 3 weeks she reported improvement in her irritability and other mood symptoms. Her biological functions including sleep and appetite were improved. Notably she did not report any worsening of pain abdomen. Also, she did not indulge herself taking non-prescription OTC steroid. She is maintaining well without any fresh complaints till her last follow-up visit at 12 weeks after her treatment started at our OPD.
A male patient in his fifties presented to his local hospital having awoken with numbness and weakness of the right leg which left him unable to mobilise. The patient was a known historic intravenous drug user who had maintained a period of abstinence over the preceding 12 months before relapsing and injecting heroin the previous morning. This was followed by a period of immobility, as he lay in a comatose state on the floor. On rousing he experienced pain in the gluteal region and an inability to walk, prompting him to seek medical attention. On arrival at the A&E department his condition was diagnosed as acute limb ischaemia secondary to intravenous drug-induced thromboembolism. The patient was commenced on treatment dose low-molecular weight heparin and transferred to a tertiary centre for an urgent Computed Tomography Angiogram (CTA, ). The angiogram showed patent vessels below the common iliac artery and was reported as normal. Detailed neurological examination revealed weakness in hip flexion and extension (1/5 on the Medical Research Council [MRC] scale) with complete paralysis in knee flexion and extension (0/5 on the MRC scale) and ankle plantar and dorsiflexion (0/5 on the MRC scale). Sensation to pinprick, vibration, and light touch was globally reduced and there was a loss of proprioception in the limb. Lower limb reflexes could not be elicited and the patient was in severe pain requiring regular high-dose oral morphine for analgesia. Blood tests revealed acute kidney injury with raised creatinine kinase, prompting treatment for rhabdomyolysis. In the context of these findings an orthopaedic referral was made with the suspicion of gluteal compartment syndrome. The patient was seen by the on-call orthopaedic team that confirmed the above examination findings and reviewed the CTA image with a musculoskeletal radiologist, who agreed that it showed significant swelling of the muscles in the gluteal compartment. The clinical picture together with the imaging findings was thought to be sufficient evidence for a diagnosis of gluteal compartment syndrome. Given the prolonged history not to measure compartment pressure was decided, as the results would not alter the management strategy and would be more likely to be normal than in an acute presentation, and taking measurements could cause a delay in treatment. The patient was subsequently taken to theatre for emergency fasciotomy, 56 hours following the onset of symptoms.\nFasciotomy was performed through a posterior approach with decompression of all gluteal muscles. The fascia lata was tense overlying an engorged, actively bleeding but contractile gluteus maximus, which was not debrided. Deep to this the gluteus medius contained some areas of friable, nonviable tissue which amounted to approximately half of the muscle. This tissue was progressively debrided down to contractile, bleeding tissue. Interestingly, the sciatic nerve was found to be intact with no obvious direct compression, other than slight impingement from piriformis which was released. The fasciotomy was left open and a negative pressure dressing was applied with the aid of nylon sutures. Examination on return to the ward revealed increased power in the limb with knee flexion and extension 3/5 MRC and ankle plantar and dorsiflexion exhibiting 4/5 MRC. Sensation gradually returned to the limb, becoming subjectively normal 36 hours after decompression, at which time the patient was requiring only oral nonsteroidal anti-inflammatory analgesia with oral morphine for breakthrough pain. A second and final debridement was performed 48 hours following the first procedure, with small areas of residual necrotic tissue removed from gluteus medius prior to washout with six litres of normal saline. A new negative pressure dressing was applied, prior to delayed closure 48 hours later.\nThe patient's recovery was complicated by worsening acute renal failure which resolved following haemofiltration (). At discharge the patient was mobilising with a Trendelenburg gait due to loss of his hip abductors and 4/5 strength in all lower limb muscle groups. Pain was controlled by oral nonsteroidal anti-inflammatory medications. At eight-week follow-up, the patient had made a strong functional recovery with no residual sciatic nerve palsy. A full lower limb neurological examination revealed 5/5 MRC in all movements at all joints. He was mobilising unaided. Functionally, he was able to perform his activities of daily living unaided and reported full independence. Pain was not reported in the affected limb.
A 33-year-old man presented to our clinic with complaints of watering, redness, and purulent discharge in the right eye. The patient reported seeing a physician a year earlier in Georgia due to fatigue, nausea, vomiting, and diarrhea. After his diarrhea and vomiting had resolved, he had swelling of the lymph nodes on the right side of the neck. After returning to Turkey for treatment, he had received cephalosporin and penicillin for suspected pharyngitis. When night sweating and weight loss were added to his complaints, he had presented to another hospital where his treatment was changed to amoxicillin-clavulanic acid 1 g 3 times a day and ciprofloxacin 750 mg twice a day, and incisional drainage was performed on the lymph nodes of his neck. When his symptoms failed to resolve completely, he had presented to the department of infectious diseases of a different university hospital. Serum agglutination test was positive for F. tularensis at a titer of 1/1280 and he was prescribed streptomycin 1 g per day for 9 days followed by 1 g twice a day for 5 days for a total of 14 days, followed by doxycycline 100 mg twice a day for 1 week. Ultrasound examination of the neck had revealed multiple abscesses in the right submandibular region and pathological lymph nodes including multiple calcifications in the right cervical chain, while magnetic resonance imaging of the neck showed retropharyngeal abscess narrowing right nasopharynx and oropharynx and submandibular lymphadenopathies (LAP) including cystic and necrotic areas (). He reported that the LAPs had resolved after a few months with no recurrence, but complaints of watering, swelling in the lacrimal sac area, hyperemia, and pain in the right eye developed a few weeks later. The patient presented to our clinic with recurrent swelling around the lacrimal sac, hyperemia, and purulent discharge.\nOn examination his best corrected visual acuity was 20/20 in both eyes. Intraocular pressure measured by automatic tono-pneumometry was 15 mmHg in each eye. On slit-lamp examination, epiphora was noted in the right eye and the left eye was normal. There was swelling in the area of the right lacrimal sac (). Fundus examination was normal in both eyes. In nasolacrimal lavage, the patient’s right nasolacrimal duct was occluded and the common canaliculus was patent. Discharge of purulent material from the right lower punctum was noted after lavage. A sample of the purulent discharge was collected and sent to the microbiology laboratory for culturing and the patient was started on oral amoxicillin-clavulanic acid 1 g twice a day and topical ciprofloxacin drops 4 times a day. Antibiotherapy was discontinued because the culture was negative. Consultation from the otorhinolaryngology (ENT) department was requested to rule out any intranasal pathology. The patient underwent ENT examination, followed by nasal endoscopic examination. In addition, to rule out intranasal pathologies that may present an obstacle to surgery, the paranasal sinuses were examined using computed tomography. No intranasal pathologies were detected in ENT evaluation. Dacryocystorhinostomy surgery was recommended to the patient, but he refused the procedure.
A 13-year-old male reported to the Department of Paediatric and Preventive Dentistry with a chief complaint of dental pain in right mandibular posterior region. Pain was mild continuous, vague aching pain. Patient presented no significant medical history and history of trauma. On extra oral examination, the patient was found to have a straight profile and a symmetric face. Clinical intraoral examinations revealed good oral hygiene with arrested caries in the right first permanent molar. The molar relationship was Angle class I on both sides.\nClinically, the right mandibular second molar was partly visible (). There was no tooth mobility and no tenderness to percussion in relation to the right mandibular first permanent molar. An intraoral periapical radiograph was advised. Radiograph revealed a mesially inclined right mandibular second molar which was partly underneath the cement-enamel junction of the adjacent tooth and partly above it. The roots of the second mandibular molar were immature with an open apex () and no resorption facets were detected on the first molar. Correlating clinical and radiographic findings, the right mandibular second molar was diagnosed as ectopic.\nThe first step in treatment is to expose the permanent second molar completely and to have better access. Under topical anaesthesia the mucosa or the pericoronal flap overlying the second molar was excised, using a diode laser (). In order to place the elastic separators in the same appointment, laser was preferred over surgical excision, as it provides better visibility and a blood free field. The second goal of the treatment was to place an elastic separator. The elastic separator acts as a wedge and aids in pushing the molar distally to gain space. Initially a single Dynaflex posterior blue elastic separator with a diameter of 2.5 mm was stretched and placed. Patient was called for review after 3 days (). Mild distal movement of the molar was observed ().\nOne more separator was added to overcorrect it and the patient was asked to revisit in 3 days. On the second recall visit, elastics were removed as the tooth was upright and free to erupt (Figures and ). No further treatment was advised as the tooth was expected to move into occlusion with a normal eruptive force. After 1-month followup, the patient was found to be asymptomatic.
A 53-year-old man with no significant past medical history was admitted to our hospital because of pain in the lower-extremities and gait disturbance. His gait disturbance noticed by his family when he was 30 years of age. At age 51, he was found to have a spinal lipoma at a neighboring hospital and was referred to our institution. His lower extremity pain and gait disturbance worsened while he was under observation and he was admitted for further examination. Manual muscle testing revealed lower limb paresis that was more pronounced on the right side. The patellar tendon reflex was not exaggerated on either side, but the Achilles tendon reflex was hyperactive and the Babinski sign was positive on both sides. There was no evidence of bladder or bowel disturbance, and the lumbar JOA score was 10/29. MRI of the spine revealed a spinal lipoma at the L2/3 level and a flow void dorsal to the spinal cord at the level of the conus medullaris (Fig. ). MRA revealed tortuous blood vessels within the spinal lipoma, as in Case 1 (Fig. ). Subsequent angiography revealed an arteriovenous shunt from the right second lumbar artery. The radiculopial artery entered the spinal canal from its ventral aspect to form a arterio-venous shunt within the lipoma. A vein from the shunt region drained into the juxtamedullary vein within the spinal canal, and formed a congestion of the venous circulation (Fig. ).\nDiagnosis was sAVF arising within the spinal lipoma, and embolization and possible surgery was scheduled. First, embolization was performed for the second lumbar artery, the feeding artery for the shunt. A xylocaine test at the feeding artery showed transient depression of MEP (as in Case 1) in the right leg muscles. However, we carried out embolization therapy as we judged the risk would be low as long as embolization was confined solely within the shunt. Because of the presence of multiple feeding arteries, the minor feeder was embolized with a coil first and then N-butyl-cyanoacrylate was injected through one of the main feeders into the shunt zone to occlude it. This did not achieve complete embolization, and surgical excision was deemed necessary. The shunt-containing lipoma was resected as far as possible. An abundance of blood vessels was confirmed within the lipoma. Intraoperative angiography confirmed there was no remaining shunt after the resection.\nAfter surgery, lower limb pain and muscle strength were improved on both sides. At 1month after surgery, lumbar JOA score was 14/29. At 1 year, angiographic examination showed no vascular abnormalities. Although the patient’s low back pain and leg numbness persisted, muscle strength and ADL score were improved. His lumbar JOA score was further improved to 17/29.
A 67-year-old man visited the emergency department of our hospital due to sudden paraplegia of both lower extremities caused immediately after slip down. On presentation to the department, both his lower limbs were observed to be almost paralyzed, with a motor grade of 2 or lower in both limbs. This paralysis showed a gradual aggravation. The patient had a history of renal cell carcinoma diagnosed 7 years prior, and achieved remission without any recurrence after total nephrectomy and adjuvant chemotherapy. He was also diagnosed with papillary thyroid carcinoma 5 years ago, and yet again achieved remission after surgical treatment. The patient signed an informed consent statement, and the study was approved by the Institutional Review Board of the Yeungnam University Medical Center.\nThe plain radiograph revealed the collapse of the T12 vertebral body. CT showed an acute burst fracture at the T12 vertebral body with an osteolytic mass-like lesion within the vertebral body and pedicle, causing severe encroachment of the spinal canal (Fig. ). Magnetic resonance imaging (MRI) revealed a bulging posterior cortex, with an acute fracture of the vertebral body and severe compression of the spinal cord (Fig. ). Considering his medical history and the MRI findings, a pathologic fracture with a tumorous condition was suspected. Therefore, the evaluation of the malignant potential and metastasis in the remote area was necessary. Hence, 18FDG PET/CT was performed, and 18FDG uptake at the T12 level was measured. A maximum standardized uptake value (maxSUV) of 1.7 was noted, with a central FDG defect on the vertebral body (Fig. ); this was interpreted as a benign fracture by a senior radiologist. Additionally, there was no positive uptake in the other body parts on PET/CT. Although there was a discrepancy between the interpretation of the spine fracture lesion on MRI and PET/CT, the patient required emergent surgical treatment owing to acute paraplegia caused by spinal cord compression, and we thus planned an initial surgery for the main purpose of cord decompression, temporary stabilization, and tissue biopsy. The surgical intervention was as follows. First, we performed T11-12 posterior laminectomy for the spinal cord decompression, followed by T9-L2 posterior instrumentation without a bone graft. Finally, tissue biopsy was obtained at the T12 pedicle and vertebral body. Histological examination confirmed the presence of a malignant solitary bone plasmacytoma (SBP) (Fig. ). Seven days later, a definite surgery with pedicle screw fixation and posterolateral bone graft from T8 to L2 without vertebral body corpectomy was performed.\nImmediately after the initial surgery, the patient's lower extremity motor function showed gradual improvement. After the surgery, the patient wore a thoracolumbar rigid brace for 2 months, and underwent continuous gait rehabilitation. Three weeks postoperatively, the motor function in his lower extremities had recovered sufficiently, and ambulation was possible with the assistance of a cane. Four weeks after the definite surgery, the patient underwent radiation therapy for 2 months. One year postoperatively, spine radiographs showed that the surgical segment had healed fully, and spine MRI revealed complete decompression of the spinal cord with no evidence of local recurrence (Fig. ).
A 54-year-old woman presented to her primary care physician complaining of epistaxis, hematochezia, headache, and a seizure. She had a prior history of seizures. The patient was on warfarin for an unknown reason. Her physician ordered an international normalized ratio (INR), which returned with a result of 13.4 after the patient had gone home. She had been told to hold her warfarin during her office visit. The patient was directed to go immediately to the emergency department (ED) but did not present until the next day. She had a history of very labile INRs in the past, with and without compliance. Multiple prior ED visits with extremely high levels in the past had been treated successfully with vitamin K and fresh frozen plasma (FFP) without complications.\nOn presentation to the ED the patient complained of a headache. She had no epistaxis or evidence of nasal bleeding. Her skin exam was normal. A neurologic exam was normal. A rectal exam was heme negative and the stool was normal color. The patient had right lower abdominal pain and tenderness. The emergency physician (EP) attending who was board certified in emergency medicine (EM) (seven years experience) supervised an intern (who had just begun residency training) in rendering patient care. A computed tomography (CT) of the brain was done with normal results. An INR lab test returned with a result of greater than 10. Due to abdominal tenderness, the possibility of appendicitis was entertained. A CT of the abdomen was ordered and the surgical service consulted.\nThe attending EP then discussed the case with the intern and a decision was made to give Profilnine (factor IX). There was no documentation of medical decision-making or discussion with the patient. The decision to administer factor IX was made prior to completion of the surgical consultation or CT result. Subsequently the EP attending left for home before the patient’s consultation or care was completed. He had never administered the drug before and instructed the intern to look up the dose on the Internet and order it. A hospital guideline specifically discussed indications for use of the drug: The patient must have either (1) a serious or life-threatening bleed; or (2) require emergency surgery.\nThree hours after administration of the medication the patient developed signs and symptoms of an acute myocardial infarction (MI). An electrocardiogram showed marked ST elevations, which resolved after the administration of tPA. Troponin was elevated as well. Cardiac catheterization performed after resolution of the ST-segment elevation revealed no thrombosis. The patient suffered a cardiac arrest and was subsequently resuscitated. Experts opined that a stroke had also occurred. She was left in a minimally conscious state with a seven-year life expectancy. A jury rendered a plaintiff verdict for $15 million.
A 54-year-old Japanese female visited an oral surgery clinic with a complaint of swelling in the inner region of the left cheek for the past one month. On clinical examination, a mobile swelling (size, 1 cm x 0.75 cm) with a clear boundary was observed on the left buccal mucosa. No associated pain was reported and the overlying mucosa was normal in appearance. The swelling was clinically diagnosed as benign buccal mucosa tumor. The tumor was excised under local anesthesia and was diagnosed as acinic cell carcinoma (AcCC) after histopathological examination. The margins were still positive for the tumor and further resection was advised. The patient reported to the Health Sciences University of Hokkaido Hospital for resection of the residual tumor two months after the initial surgery. Clinically, the patient was asymptomatic. The level 1B lymph nodes on both sides were palpable, bean sized, mobile, elastic, and soft. Intraorally, a surgical scar of about 7 mm was present on left buccal mucosa. There was no pain on pressure in the region of the scar (). The patient had a history of noninvasive ductal carcinoma (ductal carcinoma in situ [DCIS]; Tis N0M0) in the right breast, which was treated by excision and 57 Gy of radiotherapy five months ago. On investigation for oral lesion, no obvious abnormalities were detected on the computed tomography- (CT-) scan, contrast MRI, and ultrasonogram. Positron emission tomography- (PET-) CT did not suggest transition to and from any of the distant organs. The margin was resected under general anesthesia and sent for histopathological examination (). No relation to the parotid gland was found at the time of surgery.\nHistopathologically, the excised margin appeared as a fragmented tissue with no encapsulation. The tumor tissue was composed of cells with dominant microcystic structure with eosinophilic cytoplasm and eosinophilic secretory material. Papillary and tubular pattern of cell arrangement were also found but were limited to small area. A few vacuolar cells and some areas with hemosiderin deposition were observed. Furthermore, normal muscle tissue and atrophied salivary gland tissues were also seen ().\nThe secretory material was positive for diastase digested Periodic acid-Schiff (d-PAS), Mucicarmine, and Alcian Blue staining. No zymogen granules were found in the tumor cells. Immunohistochemistry (IHC) revealed strong positive reactions to vimentin, cytokeratin-19, and S100 protein. Mammaglobin was strongly positive, whereas discovered on gastrointestinal stromal tumors 1 (DOG-1) showed a negative reaction (). The histological sections of breast carcinoma were examined in suspicion of metastases; however, features of ductal carcinoma in situ that appeared completely different from those of buccal mucosa tumor were noted. Based on these histomorphologic and IHC profiles, the case was diagnosed as SC of the minor salivary gland in the buccal mucosa.
The patient in this case was an otherwise healthy 6-year-old male who presented to the emergency room with a right elbow deformity from an injury sustained while playing football. He reported being tackled and falling back, leading to a direct impact onto his flexed right elbow. Examination demonstrated his right arm to be neurovascularly intact despite having a closed gross deformity at the elbow joint. X-ray evaluation revealed an anteromedial elbow dislocation with fracture of the radial head (). Two attempts were made at closed reduction in the emergency room under ketamine sedation with no appreciable reduction or improvement in alignment. Given the lack of success with closed reduction, the decision was made to take the patient to the operating room urgently for closed versus open reduction and possible fixation. Once under general anesthesia and with muscle relaxation employed, another closed reduction maneuver was attempted again with no success (). A direct posterior approach to the elbow was then performed with exposure carried around to the subcutaneous border of the ulna. It was noted that the olecranon apophysis remained in its anatomic position despite the ulna being anteriorly dislocated. Interestingly, the proximal ulna was found to be buttonholed through the anterior joint capsule, thus preventing reduction of the joint. The radial head was also noted to have an angulated Salter-Harris II fracture which was realigned with direct pressure application. No epicondylar fracture was noted with direct inspection. Once the entrapped proximal ulna was freed from the anterior capsule, it was easily reduced into its anatomic position (). Braided suture was used to reattach the olecranon apophysis to the proximal ulna through two drill holes in a figure of eight fashion. Final clinical exam and fluoroscopy images through a full range of motion revealed stable reduction of both the elbow joint and olecranon apophysis. The patient was splinted for approximately four weeks followed by progression of gentle passive and active elbow range of motion. At his most recent 7-month follow-up, the patient was doing very well with 0o–135o of elbow extension and flexion and 75o each of forearm pronation and supination. Radiographs revealed well aligned and healed fractures with maintained physes ().
Patient 2 (40–45 years old) presented with severe agitation. He was threatening, screaming, scratching, and spitting, and refused a conversation. He thus showed a similar clinical picture as patient 1 but also appeared to experience auditory hallucinations and to actively talk to them. Patient 2 was in a state of poor hygiene. Taking into consideration his manner of response, one could assume that patient 2 has experienced psychiatric treatment in the past. When the nurse asked him if he had any experience with psychiatric medication, he yelled at her and clarified his wish to refuse haloperidol. He seemed to feel especially threatened by the police and the psychiatric staff, not only due to psychotic symptoms but also due to previous aversive experiences with psychiatric treatment. Once again, the staff members involved in the situation discussed the clinical case in a multiprofessional setting and weighed out every possible option. The team suspected that patient 2 suffered from an acute exacerbation of a disorder that persisted for a longer period of time or a psychotic relapse. In this case, the team decided that patient 2—due to his previous aversive experiences—would have extraordinarily suffered from compulsory treatment, which may exacerbate previous traumatic experiences. Also, he calmed down a bit when given a space to withdraw and did not immediately endanger himself or others; however, he remained tense for several days and threw objects whenever members of staff tried to engage him in a conversation or offered oral medication. When left alone, he did not appear aggressive or present improper handling, showed a regular food intake, and welcomed the possibility to smoke. Somewhat later, he was seeking a medical consultation and expressed the need for a low-dosage medication. To this day, 6 years later, he regularly receives outpatient care and short-term crisis intervention treatment on a psychiatric ward, although he has felt threatened and deprived of his identity by the state and the psychiatric system of another city for more than 25 years.
A healthy 11-year-old boy was referred to the clinics of Department of Pediatric Dentistry, following a severe facial trauma as a result of automobile accident. He complained of pain in his jaw and was unable to open his mouth. Initial physical examination showed abrasions and lacerations on the facial skin and the lips (). Limited opening of the mouth and lateral deviation of the mandible toward the right side on mouth opening were noted. Intraoral examination revealed a missing maxillary left permanent lateral incisor, an uncomplicated crown fracture of his maxillary right central incisor, and subluxation of his maxillary left central incisor. CBCT examination revealed a nondisplaced mandibular body fracture in the right second molar region, an oblique greenstick fracture on the left lingual side extending between the canine and first molar tooth, and a unilateral medially displaced subcondylar fracture on the right side (Figures -). The vertical height of the ramus was decreased in fractured side. Avulsion of the maxillary left lateral incisor was verified by radiograph and a horizontal root fracture of the maxillary right central incisor was also detected (Figures and ). The maxillary lateral incisor was lost at the site of the accident.\nClosed reduction techniques were chosen to manage the mandible fractures. A vacuum formed splint was fitted in the lower arch for functional repositioning of the mandible (). Arch bars that were cut to the appropriate size were bonded to vestibuloposterior parts of the splint using acrylic, and brackets were attached to the maxillary posterior teeth (). Orthodontic elastics (1/4 inch, medium strength) were used to prevent uncontrolled movements of the jaw within the first week of treatment (Figures -). The splint helped the child avoid doing excessive mouth opening and closing movements. The patient was instructed to wear the splint continuously during 24 h removing it only for eating and cleaning. A soft diet was also recommended. Analgesics and antibiotics were prescribed and the patient was instructed to gargle gently with chlorhexidine mouthwash for a week. One week later, the splint's height was increased to about 3 mm on the fractured side of the condyle and this remained for 5 weeks until a stable occlusion had been achieved. After two months, the right central incisor did not respond to the electric pulp test on clinical examination. Endodontic therapy was performed in the coronal fragment only and mineral trioxide aggregate (MTA) was used for the permanent root canal filling.\nClinical and radiological examination after 18 months revealed uneventful healing with reduction of the condylar head and remodeling of the condylar process following the conservative treatment and also complete healing of the mandibular body fractures (). The patient was not available for the follow-up controls. At the age of 17 years, he visited our clinics again with a complaint of discoloration on his maxillary right central incisor. Clinically, the maxillary central incisors were in infraposition, being more prominent in the left incisor than the right incisor (). Radiographic examination showed internal resorption on the maxillary right central incisor and ankylosis on the maxillary left central incisor (). The height of the ramus in the fractured side was similar to that of unfractured side and the mandible showed no deviation during mouth opening and closing movements. The ongoing treatment plan for the patient involves prosthetic rehabilitation of the maxillary incisors subsequent to extraction of the maxillary right central incisor and implant placement.
A 68-year-old gentleman presented to A&E with a 3-day history of nausea and faeculent vomiting. He was also reported to have not opened his bowels for 4 days, which was normal for him. He had no abdominal pain and had no fever or rigours.\nHis background was that he had cerebral palsy with severe learning difficulties. He has also had transient ischaemic attacks in the past. He is a nursing home resident and mobilizes with sticks.\nOn examination he was found to be mildly distended with a soft painless abdomen, per rectum examination was refused. A provisional diagnosis of constipation was made and he was referred to the Medical Assessment Unit. He was reviewed by the medical team who agreed with the previous diagnosis, and they also began treatment for biliary sepsis based on his blood results which suggested obstructive jaundice.\nThe following day his constipation had not resolved despite laxative treatment; thus, an abdominal X-ray was ordered which revealed dilated small bowel loops with collapsed large bowel suggestive of small bowel obstruction. This resulted in referral to the general surgery team who arranged a CT scan to determine the cause of his apparent obstruction.\nCT abdomen revealed the following:\nDilated jejunum and proximal ileum with no obvious transition point. Inguinal hernia with a closely related small bowel loop, raising the possibility of resolved obstruction (Fig. ).\nThis was then re-reviewed by a specialist GI radiologist who added the following:\nPneumobilia with cholecystoduodenal fistula secondary to gallstone passage. Small bowel obstruction with transition in the distal ileum probably due to obstructing gallstone (Fig. ).\nGiven the paucity of information elicited from the patient in terms of history, the differential diagnoses were based on the results of the CT scan. The patient has small bowel obstruction the causes of which appeared to be either secondary to inguinal hernia or gallstone ileus. The treatment of which is very different, hernia being treated with an inguinal repair and gallstone ileus being treated with either laparoscopic resection or laparotomy. The decision was made to proceed to laparotomy on the basis that if the obstruction was secondary to hernia, a resection may well need to be performed, the same approach would also allow the resection for gallstone ileus. In addition, gallstone ileus was felt to be the more likely diagnosis given the presence of pneumobilia.\nHe was taken for laparotomy with the intention to perform an enterotomy to remove the gallstone and relieve the obstruction. On opening the abdomen, he was found to have a grossly dilated small bowel with a 3-cm gallstone which was freely mobile in the distal ileum. This was abutting a large caecal mass in keeping with a tumour, which had not previously been reported on a CT scan (Figs and ).\nIleocaecectomy was performed with double-barrelled ileo-colostomy formation. A formal right hemi-colectomy was not performed due to the presence of a cholecystoduodenal fistula making mobilization of the hepatic flexure hazardous.\nHistology of the specimen confirmed a mucinous adenocarcinoma of the caecum extending into the distal ileum arising from an area of extensive carpet villous adenoma. The lesion was extending into the muscularis propria with no extramural spread. On this basis it was staged as a Dukes A pT2pN0 tumour.\nThe gentleman has recovered well postoperatively and was transferred back to his nursing home following a 10-day postoperative stay.
A 53-year-old Caucasian female with Ehlers Danlos syndrome presented with abdominal pain and vomiting to the Emergency Medicine Department. The pain was described as aching in character, initially beginning in the left iliac fossa and radiating to the left upper quadrant. The patient was unable to keep food down and had several episodes of vomiting over the course of the previous 24 hours. She felt distended but the bowel motion was unchanged with normal stools on the day of admission and no rectal bleeding. The patient recounted feeling feverish prior to admission. She had a previous history of diverticular disease and had seen her general practitioner (GP) four days earlier with abdominal pain. Her GP had started her empirically on oral Cefalexin and Metronidazole. Her past medical history consisted of diverticulosis within both the small and large bowel diagnosed on contrast studies (Figure ). She also had oesophagitis. The patient had also had two previous caesarean section deliveries which were uncomplicated and a right-sided inguinal hernia repair. The patient was neither a smoker nor drinker. The only history relating to her Ehlers Danlos was of thin skin and an increased bruising tendency, with mild difficulty in using her hands since childhood with occasional finger locking and decreased hand strength. There was no significant family history and none of connective tissue disorders.\nOn examination the abdomen was soft and minimally tender with no guarding, rigidity or rebound tenderness. Bowel sounds were present. Rectal examination revealed soft stool in the rectum. Chest radiograph was normal and abdominal films showed distended bowel loops. The patient's bloods on admission showed a normal white cell count of 6.9 and a raised C - reactive protein of 491. The other bloods were normal. The surgical team organised admission and converted her to intravenous antibiotics and fluids.\nOver the next 48 hours, the abdominal pain continued to increase and was associated with intermittent vomiting with an inability to eat or drink. C - reactive protein remained elevated. The patient continued to feel distended yet bowels continued to open normally. A repeat abdominal radiograph revealed progressive bowel distension so a CT scan was requested. This was performed the same day and suggested malrotation of the large bowel with a dilated caecum lying under the diaphragm on the left hand side and the ascending colon lying to the left of the midline (Figure ). Multiple loops of dilated jejunum and ileum were present. Engorged vessels were present in the root of the mesentery and tortuosity of the mesenteric vessels was also noted. There was no ascites or free intraperitoneal air and no obvious ischaemia of the bowel.\nIn view of the continuing deterioration in conjunction with the imaging, a laparotomy was performed. Malrotation of the bowel was found as per the CT findings and the caecum was fixed in the left hypochondrium (Figure ). Numerous diverticulae in the duodenum were also noted (Figure ). Multiple perforations sealed with omentum were seen within a six-inch segment of distal ileum without perforations of diverticulae as such. The perforations occurred within relatively normal bowel (Figure ). There was massive dilatation of the small intestine with huge multiple diverticulae especially in the jejunum and proximal ileum (Figure ). Adhesions were then divided with sharp dissection and the bowel wall was noted to be extremely fragile. Serosal tears were evident with minimal handling (Figure ).\nThe small intestine segment with the multiple perforations was resected. A hand sutured double-layer anastomosis was performed. The mesenteric defect was also closed. Haemostasis was obtained and an abdominal lavage performed with two litres of warmed normal saline. A corrugated drain was brought out through the right iliac fossa. Mass closure was performed with loop 0 Nylon. Wound lavage was performed and clips used to appose skin.\nPost-operatively, intravenous antibiotics were prescribed, and the patient was admitted to the intensive care unit. Total parenteral nutrition was started and the patient kept nil by mouth. Her bloods showed a decreasing CRP from 175 to 32 and her WCC remained stable. The patient's haemoglobin dropped to 6.0 gm/dl post-operatively while her platelets climbed to 1532. The patient refused a blood transfusion. Also during the post-operative period there was a developing mild reduction in peripheral oxygenation noted incidentally with accompanying blue hands and lips.\nThe patient made a good post-operative recovery with discharge to the ward on day 4 with commencement of a normal diet by post-operative day 7. Resumption of normal bowel habit began and patient was discharged home. Follow-up over the next three months confirmed no other complications.\nHistology revealed a segment of small intestine measuring 16 cm in length × 3 cm in diameter. The serosal surface was noted to be haemorrhagic, with several perforations, the largest measuring 1.0 × 0.5 cm with associated white exudate on the serosa. The mucosa shows a submucosal haemorrhage measuring 1.0 × 1.0 cm, which was 4 cm from its closest margin. The second specimen revealed an omental mass with an appearance of white tissue measuring 2.5 × 1.5 × 1.0 cm.\nOn microscopy, the sections of small bowel show submucosal oedema and vascular dilatation with focal haemorrhage and perforation and organising inflammation in the serosal surface. The mucosa was preserved and showed no significant abnormality. The resection margins showed viable small bowel. There was no dysplasia or malignancy and there was no thrombus in the sections of the mesenteric vessels.\nFurther sections of the omental mass show organising inflammatory debris and granulation tissue. No malignant cells were seen. Within the small bowel resection a submucosal haemorrhage and focal perforation was noted. On microscopy of the omental mass, inflammatory debris was seen.
A 70-year-old white man developed right knee pain and swelling followed by left ankle pain and swelling over a week. Over the next 4 months, his symptoms progressed to include both knees, both feet, and both hands. Due to the severity of his symptoms he was unable to ambulate or carry out normal activities of daily living. He initially took ibuprofen 800 mg three times daily with some mild improvement, but at the time of presentation, it offered no relief.\nIn addition, he endorsed morning stiffness that persisted for most of the day. Due to the stiffness in his joints, he could no longer ambulate and presented to our clinic in a wheelchair. He previously was fully functional and independent in his activities of daily living. He was an avid fisherman and was unable to pursue his interests at all.\nHis past medical history was significant for metastatic melanoma initially diagnosed 2 years ago. His initial lesion was located over the left side of his neck and he had a Mohs procedure with negative margins. He was monitored closely for 1.5 years until he was found to have new right lower lobe lung nodules on positron emission tomography (PET)/computed tomography (CT) with increased fluorodeoxyglucose (FDG) uptake. Wedge resection of the right lower lobe revealed metastatic melanoma with wild type BRAF and no C-KIT mutations. Continued surveillance demonstrated an increasing number of right pulmonary nodules over the next 6 months. Dual therapy nivolumab (1 mg/kg every 3 weeks for four doses followed by 240 mg every 2 weeks) and ipilimumab (3 mg/kg every 3 weeks) immunotherapy was started. After the second cycle of his immunotherapy he developed severe non-infectious colitis requiring hospitalization. His immunotherapy was stopped and his colitis resolved with supportive care and glucocorticoids. Without further immunotherapy, he developed new left pulmonary nodules within 6 months that were increasing in size. Single agent immunotherapy with nivolumab (240 mg every 2 weeks) was started 4 months before his presentation to Rheumatology. With single agent immunotherapy, the pulmonary nodules receded fully and no further metastatic disease was seen on subsequent PET/CT imaging 3 months later.\nHis medical history was also notable for hypertension and benign prostatic hypertrophy.\nHe was treated with hydrochlorothiazide, aspirin, and nivolumab. He had no known drug allergies.\nThere was no family history of connective tissue disease or inflammatory arthritis. His mother died from colon cancer in her 80s and his father had coronary artery disease. He was married with three living children. He served in the Navy during the Vietnam War and worked as a mechanic after his military service until retirement. He denied any history of recreational drug or alcohol use. He reported a 20-pack year tobacco smoking history, but quit over 10 years ago.\nHe denied having any chest pain, shortness of breath, rashes, oral or nasal ulcers, alopecia, Raynaud’s disease, fevers, chills, night sweats, or unintended weight loss. He did endorse feeling weak because of his chronic condition.\nHe appeared his stated age and in no apparent distress. His temperature was 37 °C, blood pressure 116/78, heart rate 70 beats per minute, and oxygen saturation 100% on ambient air. His musculoskeletal examination was significant for tender boggy synovitis of his bilateral metacarpophalangeal joints (MCPs), proximal interphalangeal joints (PIPs), wrists, elbows, knees, ankles, and metatarsophalangeal joints (MTPs). There was no palpable effusion in any joint but he had significant soft tissue pitting edema present over his extremities. There was +3 pitting edema over the dorsum of both hands and feet extending up to his wrists and mid-shins respectively. There was mild erythema and warmth present over his joints, most notable over his MCPs (Fig. ). There was decreased range of motion in his hands, feet, ankles, elbows, and knees. There also were extensor tendon rubs noted on range of motion of his MCPs bilaterally by palpation and auscultation. The remainder of the musculoskeletal examination and general physical examination was unremarkable. There were no rheumatoid nodules noted on examination.\nResults of the laboratory evaluation are shown in Table . Our patient’s erythrocyte sedimentation rate and C-reactive protein (CRP) were quite elevated. The remainder of his laboratory tests was unremarkable. Ultrasound and X-ray imaging of his hands were obtained (Figs. and ) demonstrating soft tissue swelling and extensor tenosynovitis. There were no erosions present.\nHe was started on prednisone 40 mg (0.5 mg/kg per day) and tapered gradually over the course of 6 weeks to 10 mg daily. He had a very rapid response to the prednisone with almost complete resolution of his symptoms. Once his prednisone was decreased below 10 mg he began noticing a steady return of his symptoms. During this time period he continued treatment with nivolumab and on surveillance imaging he had complete resolution of metastatic disease. Due to the marked response of his melanoma to immunotherapy, it was felt that paraneoplastic RS3PE was unlikely. Although at the time there were no published reports of nivolumab or other checkpoint inhibitors causing a RS3PE picture, it was felt that because of the temporal relationship between the nivolumab and the acute onset of his symptoms that they were related. The numerous previously described autoimmune conditions associated with checkpoint inhibitors raised the possibility that this presentation of RS3PE was another rheumatological manifestation. The clinical dilemma we were left with was that our patient had previously demonstrated a very rapid relapse of his stage 4 melanoma when off treatment, yet was incapacitated with the side effect of the treatment. After a careful discussion with his oncologist, we elected to maintain a steady dose of prednisone of 7.5 mg daily to control rheumatological symptoms and continue nivolumab. At 9 months, he demonstrated minimal pitting edema, no inflammatory arthritis, and continued full response from nivolumab therapy.
A 56-year-old woman presented to the emergency department 24 h after undergoing abdominal liposuction in another aesthetic centre. Few hours after the procedure, she experienced severe, diffuse and persistent abdominal pain. There was no relief despite a high dose of non-steroidal anti-inflammatory drugs. Later she was transferred to the surgical intensive therapy unit for “septic shock syndrome.” She presented with large necrotising cutaneous lesions of abdomen and flank areas. She was electively intubated, and initial therapies were resuscitation and hemodynamic stabilization. She needed vaso-pressors to maintain her blood pressure. The diagnosis of intestinal perforation had been missed intra-operatively as well as in the immediate postoperative period.\nA computed tomography (CT) scan was done, which showed extensive subcutaneous air in the abdominal wall from the xiphoid process down. Pockets of free air and inflammatory changes also were present within the peritoneal cavity surrounding the region of the caecum and ascending colon as seen on Figures and . Bilateral congenital lumbar herniae of the abdominal wall were identified [].\nHer vital functions were too unstable to perform an immediate debridement of the necrotizing tissue or bowel repair. Therefore, only releasing incisions up to muscular layer in flanks and infra umbilical areas were made [].\nAfter a period of 12 days, her condition stabilized enough for us to perform surgery. We performed extensive debridement of all necrotizing tissue injuries. In the area of the right lumbar hernia, there were multiple perforations in the ascending colon and the caecum []. A colostomy was made in the remaining healthy skin, in the right hypochondrium. We next dissected an omental flap to repair the right lumbar hernia []. During left lumbar debridement, in the area of the left lumbar hernia, we found multiple perforations in the descending colon []. There was no reliable flap on hand to cover this area and hence we decided to close this perforation. She had no sign of peritoneal infection. Negative pressure large wound therapy (NPWT) with 2 motors was initiated [].\nWe repeated serial debridement and NPWT dressing twice a week during the next 10 weeks. The wound healed gradually in 12 weeks [].\nFinally, this large wound was covered with a 2:1 meshed skin graft in a single stage. The back and the right thigh were the donor site. On the left lumbar area, sutures to repair the colon repeatedly failed to hold. We then decided to convert the perforated colon into a colostomy [Figures and ]. When the patient was well enough to be ambulatory, she was transferred to a rehabilitation centre.
This patient is a 43-year-old gravida 5, para 4, and abortus 1 with a history of uterine fibroids and two prior cesarean sections. The patient was referred by her obstetrician to Los Angeles County-University of Southern California (LAC+USC) Medical Center in 2019 for increasing pelvic pain and amenorrhea for seven months after her second cesarean delivery. Upon presenting to our institution, she reported that her pain was severe and had been gradually worsening over time with increasing intensity five weeks prior to presentation. The patient described the pain as constant and dull, localized to the pelvis and radiating to the lower back. Her pain was initially controlled with ibuprofen; more recently, she had required opioid medications for adequate pain control. Prior to receiving care at LAC+USC, the patient was initially evaluated for pelvic pain five months following her cesarean delivery in an emergency department. No imaging was performed at that time, and the provided diagnoses were uterine fibroids and incisional scarring. The patient then presented to other urgent care settings for worsening pain. At her first urgent care visit, she was treated empirically for endometritis without improvement in her symptoms. At another urgent care visit, a computed tomography (CT) scan of the abdomen and pelvis was performed and revealed an enlarged uterus with multiple fibroids and an enlarged cavity with suspicion of hematometra. She then presented to an outside outpatient clinic for follow-up and a stenotic cervical os was visualized on exam; she was subsequently referred to our institution.\nThe cesarean delivery operative report was obtained and revealed that the cesarean delivery was performed for persistent category two fetal heart rate tracing in the setting of one prior cesarean delivery. Upon entry into the abdomen, the surgeon documented a uterine scar dehiscence and multiple fibroids. The location and size of the fibroids were not described. After delivery of the infant, multiple supracervical and vaginal sulcal lacerations were encountered. These were repaired abdominally with a running 0-vicryl suture, beginning at the apex of the tear and suturing towards the hysterotomy. No other complications were noted. The patient was subsequently discharged home on postoperative day number two after meeting all postoperative milestones. At her postpartum visit, her provider documented her exam as limited due to an enlarged uterus and uterine fibroids. She ceased breastfeeding after two months and had not been using any hormonal or other methods of contraception at the time she was first seen at our institution.\nUpon presentation to LAC+USC Medical Center, she had no urinary symptoms, and urinalysis was negative. A pelvic exam revealed an enlarged and tender uterus, approximately 22 weeks in size. Digital exam revealed a likely blind ending of the vaginal canal. The cervix was not palpated on exam nor clearly visualized on speculum exam. Transvaginal and transabdominal ultrasound were performed. The uterus measured 21.8 cm × 9.4 cm × 1.3 cm (). The ultrasound showed a heterogeneous fluid-filled upper vagina and endometrial canal (), as well as hypoechoic structures in the adnexa suggestive of hydro/hematosalpinges (). Overall, the ultrasound findings were concerning for hematometrocolpos.\nDue to her severe pain and suspected outflow tract obstruction, the patient was taken urgently to the operating room. During exam under anesthesia (EUA), a bulging obstruction was visualized within the upper vagina. An indentation was identified and a small opening created with a scalpel. This opening was then expanded using Hegar dilators under transabdominal ultrasound guidance with efflux of a large volume of old blood. Transabdominal ultrasound then revealed a persistent fluid collection above the level of dissection. A vaginoscopy was performed with passage of the hysteroscope through the narrow opening that had been entered with the Hegar dilators. Multiple pockets of fluid were serially identified, probed gently with the Hegar dilators, and then drained. Although visualization of the portio vaginalis was suboptimal, the cervix was deemed to be dilated and without stenosis. Vaginoscopy and ultrasound revealed that there was a small fluid collection contiguous with the uterine cavity above the level of the cervix, but no further blood was noted to be draining. The procedure was then terminated. Immediately postoperatively, the patient reported improvement in her pain and was discharged home the same day. She was seen in clinic two weeks later, where she reported improvement in her symptoms. However, on sterile speculum exam, she was again noted to have a bulge in the upper vagina. Ultrasound confirmed a persistence of her hematometrocolpos. She was therefore offered planned repeat vaginal exploration with scar excision versus hysterectomy and upper vaginectomy. She elected for scar excision.\nThe patient subsequently returned to the operating room and underwent an EUA, cystoscopy, vaginoscopy, and excision of vaginal scar. EUA and cystoscopy were performed first for surgical planning. The cystoscopy showed no fistulae or masses. Attention was then turned to the vagina, where the vaginal scar was palpated, and a small cruciate incision was made in the midline with a scalpel with efflux of a moderate volume of old blood products. This opening was serially expanded with Hegar dilators followed by placement of a 30 cc Foley balloon over a catheter guide. The scar was then placed on tension and estimated to be about 2 cm in thickness. The vaginal epithelium proximal to the scar was undermined, and the scar tissue was removed circumferentially by dividing it into quadrants, until the dilated cervix was visible. The denuded vaginal tissue at the base of the scar was covered by reapproximating the distal vaginal epithelium to the proximal vaginal epithelium in a circumferential fashion with 2-0 polyglactin suture. Intraoperative ultrasound showed complete resolution of the hematometrocolpos, and the procedure was terminated. The patient was then discharged home the same day.\nShe was subsequently followed in the outpatient setting. The patient had return of regular menses and complete resolution of her pain within two months of the surgery. She reported continued normal menses seven months after the scar revision.
C is a 14-year-old girl who came to the emergency room in a bizarre state, with altered consciousness. It was just after spring break. Her parents came with her.\nC was living with her parents and her 16-year-old sister. C comes from an upper middle class Catholic family. Both parents are teachers in an elementary school. The only psychiatric background in the family concerned one aunt (one of her mother's sisters) who had been undergoing treatment for depression for two years with a complete remission. C was a premature child (33 weeks) at birth but her psychomotor development was normal. No psychological or medical problems have been reported in the early childhood. C had her menarche when she was 13 years old. Neither C nor her sister had medical or psychiatric previous record. No evidence of recent traumatic events have been found in the family. Her learning skills have always been average. She was described by her parents as too serious and highly concerned by her school results while not experiencing any academic difficulties. She was also described as being shy and dependent.\nBefore Easter vacation, one of C's aunts (the wife of one of her father's brothers) died of lung cancer three weeks after she was diagnosed but the parents didn't inform C right away because she was taking her school exams. At the burial, C was very sad but there were no pathological manifestation. Her behavior and reactions were considered normal.\nThe Easter vacation started a few days later. The first week of vacation C went to Prague with her school. Every pupil stayed in a Czech host family. No problem occurred during this week. Soon after she came back, her parents discovered that she lied to them. They had asked her to bring back crystal glasses for which they had given her some money. C came back with the glasses, but she did not tell her parents they had been a present from the family she visited, and she used the money for other purposes. C was very upset when her parents discovered the truth, even if they didn't consider this event relevant.\nThe day after a discussion concerning her untruthfulness, C suddenly presented the first symptoms, in the form of insomnia, logorrhea, anxiety with incoherent discourse and amnesia of recent events, such as her aunt's death and the recent holiday in Prague. She said that she was her dead aunt, she wondered about her origins: "I am the daughter of my aunt", she said to her father: "You are not my father". While watching a film on television, she said she recognized places where she spent her childhood. Her parents took turns to stay with her night and day. She would sleep only four hours a night. There was neither agitation nor aggressiveness but several fluctuations a day from mutism to logorrhea with incoherent ideas about her origins.\nThe following days, no change occurred but her parents still hoped she would recover spontaneously. She was unable to return to school. Then the parents referred her to a physician who sent her to our emergency room.\nIn the emergency room, C was quiet. She only complained about sleep disturbance. She also answered questions with approximate answers. When asked who she was, she said: "Anne". When asked where she was, she replied: "At my place". When asked who were the people who came with her, she replied: "My uncle and my aunt". She also gave wrong answers to questions involving simple arithmetic, for example "three plus three equals seven". C wondered about her filiation: "What if my father was not my father? I may be Jacques Chirac's (former president of France) daughter or your daughter." There was no other delusion. Her discourse was diffluent, going from questioning her origins to her aunt's death and finally to the holiday in Prague. She also presented visual hallucinations. She was scared, pointing to snakes on the walls of the emergency room. Her behavior varied from one interviewer to another: with one, she could be confused giving approximate answers, with another, she could be mute and opposing. The clinician reported no other signs of disintegration of thought processes and of emotional responsiveness.\nIn view of the atypical presentation, an organic etiology was looked for. Physical and neurological examination results were normal. A computed tomographic (CT) head scan and a EEG showed no abnormalities. Blood test results were also normal and the drug screen was negative. There was no history of head injury or trauma, or other illness.\nC was therefore admitted to our child and adolescent psychiatric unit. The two first days of the hospitalization, C received 25 mg a day of cyamepromazine. She started sleeping normally again within 48 hours. She did not seem to suffer from hospital life. No behavioral disorder was reported. The first week, we observed fluctuations in the symptoms during the day, alternating phases of coherence with a good adaptation and phases of confusion with approximate answers. By the second week of hospitalization, stable recovery was complete. Delusions and hallucinations didn't last; she presented amnesia of the episode.\nThe psychological tests produced results within the normal range.\nAfter staying 15 days in hospital, she came back home without treatment and was able to return to school.\nUntil the end of the summer vacation C did not present any disturbances. In September, C went back to school for the beginning of the school year without any trouble. But in the beginning of November, after a school holiday, she started to cry a lot, with irritability, decreased concentration, loss of appetite, negative self perceptions, suicidal ideation, telling her parents she wanted to die, and never go back to school. She refused to consult her psychiatrist during this period and the general practitionner of the family advised to wait.\nAt the beginning of December, she presented insomnia, expressed intensive negative self-perception, refused to go to school and isolated herself at home. Her parents reported that at the end of December for a few days she seemed to be "in a numbed state, talking nonsense". On New Year's Eve, she suddenly started dancing on her own in the middle of the family meal, talking about another dancer dressed in black. Her parents, alarmed by this behavior and by the visual hallucinations, decided to bring her back to our emergency room.\nAt the emergency room she arrived with a very sad look on her face, she only spoke when called upon to do so. She presented a delirious discourse on her filiations, and approximate answers; there were moments of disturbed consciousness and visual hallucinations. She though she was in "something like a cemetery". She cried a lot, and between periods of confusion she said she wanted to die. No other symptom of a psychotic disorder has been reported. No stress, drug consumption or any other precipitating factor have been found linked with this episode.\nThe next day, on the ward, the psychiatrist made the decision to treat her with fluoxetine at 20 mg/day, on account of the chronology that had been noted: appearance of a depressive state, in November, which was not treated, and deterioration of the depressive state, followed by appearance of Ganser symptoms (clouding of consciousness, approximate answers, and visual hallucinations). She also received 25 mg/day cyamepromazine at bedtime for the first three days. The Ganser symptoms improved from the start of the third week of fluoxetine administration; there was no further clouding of consciousness, no visual hallucinations, and depressive symptoms (insomnia, irritability, suicidal ideation and depressed mood) disappeared at the same time. She was hospitalized one month total and then returned to school.\nFluoxetine treatment was prescribed at 20 mg/day, but after two months C started to refuse to take any medication and stopped taking it. The school year ended uneventfully; there were no further disturbance. C continued with a combination of psychiatric follow-up and interpersonal psychotherapy for another year. No further episode occurred. Then C decided to end up to the follow-up.
A 34-year-old man (height 172 cm, weight 87 kg, body mass index 29.4 kg/m2) had been followed for purpura nephritis since he was 18 years of age. Since 32 years of age, he had been treated for hyperlipidemia with 20 mg of atorvastatin (cholesterol-lower agent). He had no history of alcohol abuse or smoking. Because of the gradual progression of renal failure, he was started on corticosteroid therapy (prednisolone at 30 mg/day) for the first time at 34 years of age.\nThe time course of the corticosteroid therapy is shown in Fig. . Two months after the initiation of corticosteroid therapy, he underwent tonsillectomy followed by corticosteroid pulsed treatment (500 mg of methylprednisolone for 6 days) for purpura nephritis. The prednisolone dose was gradually decreased, declining to 10 mg/day at 6 months after its initiation. He was not on any diet or exercise program during the corticosteroid therapy, even though he was overweight.\nEight months after the initiation of corticosteroid therapy, he complained of left hip pain with no antecedent triggering activity. T1-weighted MRI scans obtained 2 weeks after the onset demonstrated a low-intensity band lesion in the left femoral head (Fig. ). On the other hand, there was no obvious abnormality in the right femoral head. On short tau inversion recovery (STIR) images, an associated bone marrow edematous lesion was confirmed distal to the band lesion on the left side, indicating the occurrence of femoral head collapse (Fig. ). The patient was diagnosed with unilateral osteonecrosis of the left femoral head and was referred to our department.\nAlthough osteonecrosis of the left femoral head had collapsed already, joint preserving procedures, such as osteotomy and bone grafting, were considered to be more suitable than total hip arthroplasty since the patient was relatively young. For the purpose of evaluating the indications of osteotomy, he underwent MRI again 6 weeks after the initial MRI. This time, a low-intensity band newly appeared in the right femoral head on T1-weighted images (Fig. ). STIR images did not reveal a bone marrow edema (Fig. ). The second MRI scans demonstrated the new development of osteonecrosis in the right femoral head. As the right hip was asymptomatic and the patient did not will additional treatments, we decided to observe conservatively without restricting weight-bearing unless the right hip became symptomatic. Thereafter, his left hip underwent transtrochanteric anterior rotational osteotomy. Postoperatively, the patients started to perform active range of motion exercise 5 days after the operation and began to use wheelchairs at the same time. His left hip was maintained non-weight-bearing status until 5 week after the operation, followed by partial weight-bearing walking using two crutches. Full weight-bearing walking was permitted 4 months after the operation. His right hip had remained asymptomatic as of 8 months after the second MRI scans (7.8 months after the operation). At that time, the low intensity band was observed more obviously on T1-weighted images.\nThe time courses of the patient’s serum lipid levels (triglycerides, total cholesterol, low-density lipoprotein cholesterol, high-density lipoprotein cholesterol) are shown in Fig. . The serum level of triglycerides showed transient elevation twice during the period of observation (2.5 and 8.5 months after the initiation of corticosteroid therapy). The first elevation occurred immediately after corticosteroid pulse treatment, while the second occurred without obvious trigger events including the increase in the dose of corticosteroids. No other parameters showed significant elevations during the period of observation. There were also no remarkable changes in his coagulation parameters, hepatic enzymes, or C-reactive protein level.
Our experience reports the case of a 41-year-old male patient who presented to our outpatient department for severe back pain. The patient had been emergently treated with a zone-2 TEVAR with distal PETTICOAT (provisional extension to induce complete attachment) limited to the thoracic region for complicated Type-IIIB aortic dissection performed due to a worsening of pleural effusion and to a not responding arterial hypertensive state. The left subclavian artery was neither revascularized nor occluded at the origin. The intervention was complicated by left cerebral ischemia; the stroke resulted in a right hemisyndrome with mild walking impairment. Patient followed a neurological rehabilitation program both in hospital and after discharge. A 2-month computed tomography (CT) scan observed a Type-IC endoleak and complete reperfusion of the false lumen from distal reentry tear with enlargement of the false lumen associated with intermittent pain. The subsequent treatment entailed a stent–graft extension from the previous TEVAR to just above the celiac trunk origin followed, 2 weeks after, by embolization of the origin of the left subclavian artery and occlusion of the distal false lumen with a candy plug version II.\nThe immediate postoperative course was characterized by a postimplant syndrome treated with steroids. Nonetheless, a few months after these procedures, the patient returned with worsening back pain that was hardly responding to any common pain killers. Repeated hospitalizations and complete vascular and neurological evaluations did not show any technical and anatomical issues to justify the recurrent pain. Follow-up CT scans, performed to rule out an aortic etiology of the pain, revealed a progressive complete thrombosis of the false lumen with progressive shrinkage of its portion in the thoracic region and stable transaortic diameter in the abdominal region with the residual dissection (46 mm;\n). The patient was referred for algological therapy with a mild regression of the visual analogue scale (VAS) from 8 to 6. After 18 months, since the first procedure, the patient was under transdermal oxycodone treatment. A Short Form 12 Health Survey Scale (SF-12) was administered underlying severe physical and social impairment because of back pain and drug therapy. During a new hospitalization, in our hospital, new angio-magnetic resonance imaging (MRI), electroencephalogram, electroneuromyography, and somatosensorial-evoked response tests excluded any neurologic defects. Lung and pleural evaluations showed normal results. In the end, despite the technical success, the aortic nature of the pain was suspected and zooming the bulk of endovascular material inside the thoracic aorta as a trigger for complex regional pain syndrome (CRPS).\nTherefore, the candy plug removal was considered to reduce the radial force working inside the aorta. The patient was approached in two stages. First, a left carotid subclavian artery bypass was performed to increase the collateral network inflow considering that preoperative MRI and CT scan failed to detect patent intercostal arteries arising from the stent–grafted region. Ten days after cervical bypass, a semiconservative open conversion was performed through a left thoracoabdominal incision in the eighth intercostal space; the progressive shrinkage of the thoracic false lumen allowed treating the patient as a Type-IV thoracoabdominal aortic aneurysm (TAAA). Cerebrospinal fluid drainage and permissive hypothermia (lower rectal temperature of 33°C) combined with the previous left subclavian artery revascularization were employed as adjuncts for spinal cord protection. The thoracoabdominal aorta was prepared from approximately 10 cm above the aortic hiatus down to the aortoiliac bifurcation. Before opening the aorta, the region where “candy plug” imprinting the aortic adventitia was evident (\n; available in the online version). “Candy plug” bulged out from the aortic false lumen through a 10-cm long longitudinal incision (\n; available in the online version). After aortic cross-clamping, the false lumen was longitudinally opened, the candy plug was removed (\n), and the true lumen endograft was partially resected to both perform the proximal anastomosis in a dissection-healed thoracic region and resect the residual and aneurysmal abdominal aorta. Visceral and renal protection was obtained with local hypothermic perfusion with 4°C Mannitol and Ringer Lactate solution.\nProximal aortic graft was sutured end to end with the endograft and distally a beveled anastomosis, including visceral and renal vessels, was performed.\nRecovery was uneventful, and patient was discharged on postoperative day 15. After 4 months, the patient reported VAS 2 with no need of opiates as painkiller.
The patient, a 31-year-old woman, was admitted to the department of neurosurgery at The First Hospital of Jilin University due to a “right occipital mass for 5 months”. The patient was in good health, and her family members did not have similar lesions. A physical examination showed that the mass was in the right occipital region at the upper margin of the sternocleidomastoid muscle and exhibited slight tenderness without obvious pulsation or vascular murmurs during auscultation.\nA head CTA examination at admission suggested that the lesion was approximately 3 × 5 cm in size, was mainly composed of vascular components, presented as a “wool mass”, was located in a subcutaneous region, involved the muscles, and showed obvious enhancement. The lesion was mainly supplied by the occipital and muscular branches of the vertebral arteries. The venous drainage of lesion communicated with the suboccipital venous plexus and the paravertebral venous plexus, and the venous drainage communicated with the subcutaneous cervical superficial veins (Figs. and 2).\nA further DSA examination revealed that the blood supply of the lesion was mainly from the occipital artery and less from the muscle branches of the vertebral arteries (Fig. ). The lesion was considered an AVM based on its imaging characteristics and was recommended for surgical removal. The surgery was carried out under general anesthesia. The lesion could be touched when the occipital skin was incised during the operation. The lesion showed a clear boundary. The occipital artery touched the anterior margin of the lesion, was exposed and ligated, and was removed around the lesion. The lesion consisted of massive blood vessels, and the surrounding muscles were swollen, indicating IH.\nAfter the lesion was removed, the normal muscle tissue around the lesion was also removed. The specimen was sent to pathology, and postoperative pathology confirmed that the lesion was IH accompanied by thrombolytic recanalization. HE staining showed that the lesion almost purely consisted of capillaries with only rare cavernous components among the muscles. CD31 staining was positive in the vessel endothelium, indicating that the lesion was an IH (Fig. ). The patient achieved a good recovery after surgery and was discharged from the hospital. A head CTA review performed one year later showed partial residual IH (Fig. ). The patient had no obvious discomfort, and the hemangioma could not be touched from the surface, and the patient was recommended for radiation therapy. The patient refused radiation therapy. Follow-up 2 years later showed no enlargement of the IH.
A 45-year-old man presented with a 6-month history of progressive dyspnea with productive cough and wheezing. The patient had a history of smoking (1 pack per day for 20 years), hypertension and asthma, which was under regular medical control. He also had a history of tongue cancer (squamous cell carcinoma, pT2N0M0, stage II) for which he underwent wide excision of the right side of the tongue and modified neck lymph node dissection five years prior to this presentation. Chest plain film showed a protruding mass shadow in the left hilar region. Costodiaphragmatic angles were clear. There was increased density over left lung field with elevation of the left side of the diaphragm. These features were indicative of a hilar mass obstructing the bronchus with collapse of the upper left lobe of the lung (Fig. ). Contrast enhanced computed tomography (CT) showed a hilar mass measuring approximately 35 mm × 28 mm × 15 mm and a collapsed left upper lobe of the lung. There was weak enhancement in the arterial phase. The endobronchial part of the tumor had clear margins along the bronchus of the upper left lobe of the lung. The distal part of the tumor had indistinct margins along the lung parenchyma. The distal bronchus was dilated and filled with secretions. There was no mediastinal lymphadenopathy (Fig. ). Bronchoscopy revealed a whitish tumor obstructing the left upper bronchus (Fig. ). Biopsy specimens of the tumor taken during the bronchoscopic examination showed evidence of smooth muscle cell proliferation with focal abnormal mitosis. A smooth muscle cell tumor of malignant potential was considered. Positron emission tomography (PET) showed increased fluorodeoxyglucose (FDG) uptake in the lesion (Fig. ).\nThe tumor involved the upper left lobe of the lung and obstructed the bronchus. The patient underwent a thoracoscopic lobectomy under general anesthesia with double lumen endotracheal tube placement. The vessels of the left upper lobe were divided and ligated using an endoscopic autostapling device. The bronchus of the upper left lobe was opened by endoscissor. The cutting margin was checked by examination of frozen sections to ensure that the resection was clear. The upper left lobe of lung was removed through a port with extended skin incision 5 cm at the anterior 5th intercostal space. The orifice of the bronchus was sutured with standard instrumentation through the utility incision.\nThe resected tumor was white and elastic, measuring 3.5 cm × 2.5 cm × 1.5 cm in size. It impacted the whole bronchus of the left upper lobe (Fig. ). Microscopic examination revealed a mixture of spindle cells showing fibroblastic and myofibroblastic differentiation arrayed in fascicles, or with storiform architecture. The spindle cells had oval nuclei, fine chromatin, inconspicuous nucleoli, and bipolar, lightly eosinophilic cytoplasm (Fig. ). Admixed with the spindle proliferation was an inflammatory infiltrate containing lymphocytes, plasma cells, and eosinophils. Immunohistochemical analysis showed positive staining for vimentin (Fig. ) and desmin, and focal positive staining for smooth muscle actin and cytokeratin (Fig. ). The tumor had a low Ki-67 proliferative index. In contrast, the tumor cells were not reactive to CD34, CD99, or S-100 antibodies. The surgical resection margins and all regional lymph nodes were tumor free. Inflammatory myofibroblastic tumor was diagnosed. At the most recent follow-up (12 months after operation), the patient was symptom free and there was no evidence of tumor recurrence on chest CT scan.
A 41-year-old man with no significant medical history presented with a chief complaint of pain in his left leg. The top of his left foot had been run over by a car tire. No fractures or joint injuries were noted in the toes. He was diagnosed with complex regional pain syndrome (CRPS).\nHe underwent twice-weekly lumbar epidural blocks at his local hospital and a month-long continuous epidural block each winter, which allowed him to return to work. The needle used for a single epidural block was 20 gauge, and the catheter used for the continuous epidural block was 20 gauge. The degree of pain in his left leg according to the numerical rating scale (NRS) was 8 during activities of daily living and 3 after a lumbar epidural block was performed.\nFive years after the initiation of epidural blocks, greater resistance was felt while injecting the medication into the epidural space, and it was difficult to insert the catheter. The degree of pain in the leg after the epidural block became NRS 6, indicating that effects of the epidural block were insufficient. The degree of pain in the leg during activities of daily living was not exacerbated, remaining at NRS 8. Adhesions in the epidural space caused by frequent epidural blocks were suspected, and the patient was referred to our department.\nThe patient had undergone epidural blocks many times at his local hospital. These comprised single epidural blocks with 10 ml 0.375–0.75% ropivacaine at the L4/5 and L5/S1 levels performed over 200 times as well as continuous epidural blocks using 0.2% ropivacaine for approximately 1 month performed multiple times. During examination, the patient exhibited spontaneous pain from the left leg to the foot accompanied by allodynia. Muscular atrophy and edema were also noted at the same site. Manual muscle testing results ranged from 2 to 3 in the tibialis anterior muscle, triceps surae muscle, gastrocnemius muscle, long extensor muscle of the thumb, and flexor hallucis longus muscle. There was no decreased perception and no other abnormal neurological findings. In the area from the left leg to the foot, plain X-rays revealed mild bone atrophy and magnetic resonance imaging (MRI) revealed mild bone and muscle atrophy. No abnormalities were noted in the lumbar spine on plain X-rays, and MRI did not reveal any abnormal findings in the intervertebral discs, vertebrae, spine, or nerve roots. T1-weighted fat-suppressed images showed uneven contrast effects of gadolinium throughout the back of the epidural space from L4 to S1 (Fig. ). Epidurography performed from the sacral hiatus revealed blockage in the L5/S1 vertebral area. Lower limb thermography revealed decreased temperature throughout the entire left leg. We diagnosed the pain in his left foot as CRPS because it applied to continuing pain which was disproportionate to any inciting event, hyperesthesia, allodynia, temperature asymmetry, motor dysfunction, and trophic changes among the diagnostic criteria for CRPS by International Association for the Study of Pain, 2005 []. However, the attenuated effects of the epidural block were determined to be due to adhesions in the epidural space rather than CRPS exacerbation.\nRegarding further treatment, the patient was advised that, although it might be possible to regain the effect of the epidural block by performing adhesiotomy in the epidural space, this was not a radical cure for CRPS of the left leg, and it would be difficult to achieve good-quality continuous epidural blocks over long periods if adhesions in the epidural space recurred following adhesiotomy. Therefore, he was advised to undergo spinal cord stimulation rather than long-term epidural blocks as a means of treating the pain in his leg. However, the patient requested to undergo adhesiotomy in the epidural space.\nTherefore, epiduroscopy was performed. We used video-guided catheter and 0.9 mm fiberopticscope (Kobamed system, Japan Medicalnext Co., Ltd., Osaka, Japan). Contrast enhancement of the area from the sacral hiatus to the epidural space performed before epiduroscopy revealed blockage of the L5/S1 vertebral area, with no contrast of the epidural space any further toward the cranial side of this area (Fig. a). Endoscopic findings revealed adhesions in the epidural space in the L4 to S1 vertebral area. The adhesions were mainly formed from the soft connective tissue, and little hard scar tissue was observed. The adhesions were distributed throughout the L4 to S1 vertebral area, with many adhesions observed on the left side of the L4 and L5 vertebral area. There were few areas of hyperemia, reddening, or blood vessel proliferation (Fig. a). Adhesiotomy was carefully performed using the catheter tip while injecting the physiological saline solution. We were able to easily detach the adhesions, and contrast effects were achieved in the epidural space (Fig. b). In the epidural space toward the cranial side from the L3 vertebral area, dura mater and fatty tissue could be clearly observed and appeared to be normal (Fig. b). The operation time was 58 min, and the physiological saline solution used for adhesiotomy was 250 ml.\nAfter epiduroscopy, although the degree of pain in his leg after an outpatient epidural block had recovered to NRS 4, the effects of once-weekly epidural blocks were only sufficient for 1 month. At this point, the patient realized that the treatment of the pain in his leg with epidural blocks would be difficult and requested to undergo spinal cord stimulation.\nFor spinal cord stimulation, an 8-pole electrode (Octrode™, Medtronic, Inc., Minneapolis, MN, USA) was percutaneously placed into the epidural space so that the tip was in the Th9 vertebral area and a nerve stimulator (Eon Mini™, Medtronic, Inc) was placed in the right hypogastric region. Thereafter, the degree of pain in his leg during activities of daily living decreased to NRS 5.\nIn our patient, the effects of repeated epidural blocks gradually decreased. MRI, epidurography, and epiduroscopy indicated adhesions at epidural block puncture sites. Furthermore, no spinal disorders were observed on imaging. Therefore, our patient appeared to have adhesions caused by repeated epidural blocks.\nMRI and epidurography are generally used to diagnose adhesions in the epidural space. When adhesions are present in the epidural space, MRI shows enhanced contrast effects in the connective tissue in the epidural space and disappearance of epidural fat [, ]. Although MRI is noninvasive and can be used for extensive observation of adhesions, its diagnostic precision is low. Epidurography shows blockages at the adhesion sites. It can be used for extensive observation of adhesions and offers high diagnostic precision. However, the diagnosis of adhesions located further toward the epidural space on the cranial side from the blockage requires another puncture site. Although MRI and epidurography are sufficient for the diagnosis of epidural adhesion, it may be possible to obtain findings of epidural space by epiduroscopy which are difficult to obtain by other radiological tests.\nEpidural blocks result in connective tissue growth in the epidural space due to microbleeding caused by the punctures, local inflammation caused by high concentration of local anesthetics, and healing process of the inflammation []. In our patient, it appeared that multiple epidural blocks and continuous epidural catheter insertion have contributed to this connective tissue growth. Most of the adhesions in our patient resulted from connective tissue growth, and there were few areas with strong adhesions, scarring, hyperemia, or reddening. In contrast, standard endoscopic findings of lumbar degenerative diseases such as lumbar disc herniation, lumbar spinal canal stenosis, and failed back surgery syndrome have been reported [, ]. In case of lumbar disc herniation, inflammatory findings such as hyperemia and reddening are common and adhesions are often mild. In case of lumbar spinal canal stenosis, inflammatory findings and connective tissue growth are observed due to the degree of stenosis, and adhesions are often strong. In cases of failed back surgery syndrome, the connective tissue in the epidural space is known to form scars and exhibit strong adhesions around the nerves. Comparing our endoscopic findings to previous reports, the connective tissue that developed in our patient because of epidural blocks was milder than any of other lumbar degenerative diseases.\nNormally, lumbar and sciatic nerve pain due to lumbar herniated disc, lumbar canal stenosis, and failed back syndrome are indications for epiduroscopy []. We performed adhesiotomy in the epidural space in our patient to enable him to undergo subsequent epidural blocks as treatment. Although adhesions in the epidural space could be detached with epiduroscopy, adhesions recurred because of repeated epidural blocks following adhesiotomy. Therefore, it appears that performing adhesiotomy and subsequent epidural blocks cannot be recommended for such cases of iatrogenic adhesions of epidural space even when the patient asks to perform.
We report the case of a 24-year-old man who presented progressive precordial pain and dyspnea that had worsened in the last six months prior to the consultation with no previous history of arterial hypertension, dyslipidemia, smoking habits, or infectious diseases, such as syphilis or immunodeficiency syndrome. The physical examination showed no signs of congenital syndromes and revealed only severe systolic murmur in the aortic focus. Preoperative examinations were conducted as routine. Laboratory tests as well as the chest radiograph were unchanged. The electrocardiogram showed sinus rhythm, incomplete right bundle branch block and moderate left ventricular hypertrophy. The echocardiogram revealed aortic stenosis caused by a thickened aortic valve, which determined a gradient of 65 mmHg, left ventricular ejection fraction of 0.63, ascending aortic diameter of 30 mm, and systolic and diastolic diameters of 32 and 48 mm, respectively. Then, we proposed the surgery for aortic valve replacement. The procedure was performed by median sternotomy. After the pericardiotomy, we noticed an uncommon aspect of the ascending aorta, with an irregular surface and thinned dilated areas with soft superficial texture (). Aortic cannulation was performed above and medial to the innominate artery, and right atrial cannulation was performed through the right atrial appendage. A left ventricular vent was placed via the right superior pulmonary vein. After total heparinization, cardiopulmonary bypass was then established, the aorta was cross-clamped and a transverse aortotomy was performed. The internal aspect of the vessel was thickened, with multiple focal cavities of different sizes corresponding to the external dilatations; no thrombi were found ().\nAntegrade cold blood cardioplegic solution was infused every twenty minutes. The three thickened aortic leaflets, as well as the anterolateral convexity and anteromedial concavity of the aorta were excised, leaving the posterior one third, macroscopically normal, to serve as support tissue to the vascular prosthesis that would be placed. Samples were submitted for histological study, and the procedure was completed. The prosthesis implanted was a mechanical no. 21; a Dacron tube prosthesis (no. 24) was positioned in replacement of the ascending aorta above the coronary ostia. De-airing was performed through a catheter placed in the tube; the aortic cross-clamp was removed. As the heartbeats became effective, the cardiopulmonary bypass was ended, and the surgery was finalized as usual. The patient was sent to the intensive care unit, where he remained for one day. He was then discharged by the tenth day in good health. He recovered and has lived until the present moment without complications.\nThe histopathological findings were ascending aorta with areas of intimal fibrosis with dystrophic calcification consistent with arteriosclerosis. Areas of medial cystic degeneration with saccular dilations were observed to be diffusely distributed; inflammation was absent (-A,B,C). The aortic leaflets were fibrous with myxomatous degeneration and dystrophic calcification. The loss of smooth muscle cells was markedly important, as was the deposition of extracellular matrix following the degeneration.\nAngiotomography of the aorta was performed post-operatively and showed no other injuries in the aorta. Furthermore, the prosthetic graft in the ascending aorta and the mechanic valve prosthesis in the aortic position were properly positioned ().\nSome laboratory tests were performed in order to try to identify infectious diseases, such as syphilis and HIV, and autoimmune diseases, such as vasculitis, with no positive results.
A 53-year-old man was referred to the oral surgery clinic affiliated to Isfahan University of Medical Sciences complaining of painful exposed oral lesion and bone extraction from right lower jaw in the mouth. He has declined any surgical or other dentistry procedure on his mouth recently. In his medical history, RCC with lung metastasis has been a diagnosis for him in March 2016, and he underwent a left total nephrectomy on April 2016. For the previous 5 months, he has been treated with cycles of 50 mg sunitinib (Sutent®) once a day for 4 weeks followed by 2 weeks drug free. In fact, he had received almost 4 cycles of sunitinib before lesion exposure in October 2016. Neither mandibular pain nor bone or mucosal lesions were present at the time of sunitinib initiation. During the past 5-month sunitinib therapy, the patient has encounter to intermittent mucositis and gingivitis in oral cavity several times. He had no other positive history for any chronic conditions such as hepatic or renal failure. He declined using any other medications such as glucocorticoid's drugs. Unfortunately, due to complex condition at the time of cancer presentation and misdiagnosis with multiple myeloma, our patient had received zoledronic acid (4 mg intravenously 2 times) on March 2016, and after diagnosis confirmation, it was discontinued quickly. Hence, more than 7 months before lesion exposure, our patient had a history of BP administration. After, 4 course of chemotherapy with sunitinib, oral lesion with persistent pain and progressive mandibular bone exposure has been emerged. In following, patient complained of chewing problem led to our patient refer to oral surgery clinic at the end of October 2016.\nClinical examination revealed a painful exposed bone (around 10 mm) at the left hemimandible [] on the contralateral side. The panoramic radiograph [] showed irregular sclerotic bone at these regions, and the fusion of computerized tomography (CT) scans and bone scans demonstrated locally elevated nucleotide activity there. Axial CT scan showed a single osteonecrotic process involving the mandible, associated with increased bone marrow density and initial signs of bone sequestration and periosteal reaction.\nFinally, Grade 1 of ONJ was diagnosed. In this grade, there is a necrotic or exposed bone in patients while the patients are asymptomatic and have no evidence of infection. It supposed to be happened in the patient who received sunitinib therapy while he had a history of BP administration. As the main accused reason was sunitinib therapy, the treatment was withheld and unnecessarily oral amoxicillin (500 mg tid) and metronidazole (500 mg tid) for 7 days were prescribed for the patient. He also was recommended to use chlorhexidine mouthwash twice daily. We followed him for more than 4 weeks, his lesion was Grade 1 of ONJ and the symptom recovery occurred after 2 weeks without any further complication. After 1 month, therapy with sunitinib was resumed for other remained courses. He is taking his medication under the supervision of our team, and the teams frequently monitor him for any other predicament.
A 75-year-old woman was referred to our department with a diagnosis of pneumonia with a high fever. She had no significant medical history except for diabetes mellitus, and no abnormality on screening chest X-ray had ever been detected. A computed tomography (CT) scan showed fluid collection in a large emphysematous lesion. At first, pulmonary sequestration of the right lower lobe was suspected, but it was eventually diagnosed as pulmonary bullous emphysema because there was no aberrant artery from the aorta on enhanced computed tomography. The patient was treated with antibiotics for infected bullous emphysema. There was no clear communication between the bronchus and the bulla on bronchoscopy, but the presence of infection suggested the possibility of a communication. After the infection was cured, the localized emphysema had not decreased in size, and she continued to have repeated infection in the bulla. Therefore, it was decided that surgery to completely remove the bullous emphysematous lesion was needed. The large bullous lesion was located by CT scan from segment 9 to segment 10 in the right lower lobe, and it extended longitudinally to the border between segments 6 and 10 (Figure \n).\nVideo-assisted thoracoscopic surgery (VATS) was performed, and the bullous lesion was seen both by the scope and directly by the naked eye. However, the border of the bullous lesion was not clear in the deflated lung. ICG (0.5 mg/kg) was injected into a right arm vein, and the lung was observed using the PDE imaging system through the 8-cm thoracotomy site. About 8 seconds after injection, ICG entered the lung, and the bullous lesion was detected as a defect of fluorescence contrasting with the normal lung fluorescence. The contrast remained for 30 seconds, and the ICG reached the bullous area, which was visualized as white (Figure \n). While the contrast was present, the border between normal lung and the bullous lesion was marked by electric cautery. During the marking, a scope from a 10-mm port assisted in visualizing the lung because the PDE system shows a black and white view without normal light support. It appeared that the bullous emphysematous lesion could be resected completely by basal segmentectomy, and this was performed. There were no complications postoperatively, and the patient was discharged 10 days postoperatively. At follow-up 12 months after surgery, the patient had no infections and no bullous lesions in the lung. The patient was provided informed consent to use ICG and the Ethics Review Board on Clinical Research of Japanese Red Cross Nagasaki Genbaku Hospital approved the study protocol (#175).
A 46-year-old Javanese woman presented with urinary incontinence following an abdominal hysterectomy with bilateral salpingectomy 3 months earlier. She is a housewife with no history of routine drug use and no prior history of hypertension, diabetes, allergies, or other chronic disease. She does not smoke tobacco and does not consume alcohol.\nA physical examination revealed that her general condition was good and her vital signs were: blood pressure 112/74 mmHg, heart rate 89 beats per minute, respiratory rate 18 times per minute, and temperature 36.6 °C. There were no abnormalities in her chest and abdomen, or in musculoskeletal and neurological examinations. In a genitalia examination using a speculum, we identified fistulae above her vagina wall that were 1 cm in size. All laboratory findings (that is, complete blood count, liver functions, renal functions, and urine analysis) were within normal limits.\nAfter discussion with our patient regarding the risks and benefits of an open abdominal procedure and laparoscopic approach, we discussed the similarities and differences between the two procedures were her. We chose surgical management using laparoscopic approach with the considerations that it could facilitate precise dissection, offer good visualization, and be minimally invasive, thereby enabling faster recovery.\nOur patient was placed in the lithotomy position and received general anesthesia. A cystoscopy was performed to confirm the fistulae orifice and a stent was inserted into the fistulae tract from her bladder to her vagina. A tamponade was inserted into her vagina up to the vaginal apex, to be able to identify the vagina and prevent loss of pneumoperitoneum. A transperitoneal approach was performed with trocars distributed as follows: The camera was placed through a 12 mm port with 30° down lens located superior to the umbilicus. Two ports for the surgeon were placed on the right side (Fig. ).\nShe had adhesions; therefore, adhesiolysis was performed, using a combination of sharp and blunt dissection to expose the vaginal stump and the superior aspect of her bladder (Fig. ). A simple cystotomy was performed and extended to include the fistulae site, and the fistula tract was excised until viable fresh tissue was exposed. Later the defect was repaired by using a running stitch of 3–0 Vicryl. Knots were tied intracorporeally. A second layer of closure was performed in an imbricating fashion with the same suture. The vagina defect was not closed separately but covered with an omental flap (Fig. ). A vascularized omental flap was made using a scalpel, which was placed in the plane of dissection between her bladder and her vagina, and it was secured with two attachment points.\nThe ureteral stents were removed without difficulty. A urethral catheter was placed for adequate postoperative urinary drainage. This procedure takes approximately 2.5 hours; the estimated blood loss for our case was minimal and there were no intraoperative complications.\nOur patient was given intravenously administered ceftriaxone 1 gram per 12 hours postoperatively for prophylaxis and orally administered diclofenac for pain control on an as-needed basis from the following day. At postoperative day 1, she was able to eat as usual and complained of minimal abdominal pain during mobilization. The surgical wound was good and there was no urine leakage from her vagina. After that, she was discharged while still using urethral catheter for adequate postoperative urine drainage for 2 weeks.\nShe returned for a follow-up 2 weeks after surgery and reported that she experienced no recurrent incontinence and urination was normal. She continued to do well at 1-month, 3-month, and 6-month postoperatively.
A 2-year-old female presented a systolic murmur between the 2nd and 3rd sternal ribs during a routine examination before kindergarten admission. This child was referred to a cardiologist at our institute. The patient had a normal growth in height and weight and was not known to have any cyanotic, recurrent respiratory infection or heart failure symptoms. In addition, there was no past medical history of cardiac surgery, endocarditis, trauma, or cardiomyopathy. The echocardiography confirmed an abnormal shunt between the left ventricle and right atrium located in the very superior part of the ventricular septum close to the septal tricuspid valve and anterior mitral valve, with a diameter of 2 mm. Electrocardiography and Holter monitoring had been performed to demonstrate that there was no arrhythmia before the procedure. In addition, dilation of the right atrium (40 mm) was identified, which was not a common clinical manifestation of typical ventricular septal defect (Fig. A and B). Therefore, a rare congenital LV-RA shunt type I was suspected. In consideration of the small size of the LV-RA shunt and some previous case reports indicating that transcatheter closure would be an alternative to surgery, an invasive procedure was scheduled. During the procedure, angiography demonstrated that the right atrium first appeared with contrast medium, which then flowed into the right ventricle and pulmonary circulation (Fig. A). As a long ductus from the left ventricle to the right atrium was suspected, the ADO II was chosen for this case. The ADO II device is a type of self-expanding nitinol mesh device for the occlusion of patent ductus arteriosus. Its most special feature is that the ADO II device presents a great flexibility, which can result in little harm to valves and has been applied to some superior location ventricular septal defect closures in consideration of the interaction with aortic valves. Finally, an AGA ADO II 3/4 mm device was delivered from the 4F sheath, and the defect was closed successfully (Fig. B). One week after the procedure, echocardiography revealed completed closure of the shunt with normal movement and function of tricuspid valves, mitral valves, and aortic valves (Fig. C). One month after that, enhanced computed tomography scanning confirmed that the occluder was in the appropriate position (Fig. C and D). Echocardiography was repeated at 3 months post closure and revealed normal device position, heart structure, and function. In addition, as arrhythmia can occur after transcatheter closure, ECG was performed at 1 week, 2 weeks, 4 weeks, and 3 months post closure, while an additional Holter monitoring series was completed at 4 weeks, which indicated that there was no arrhythmia occurrence. According to our follow-up data, there is no impairment of tricuspid valvar movement due to the implantation of the ADO II device. Thus, the highlight of this technique is that it allows the patient to receive an transcatheter cardiac interventional closure and maintain normal valvar function, avoiding open heart surgery.
A 43-year-old Asian man presented to the emergency department in our institution due to generalized weakness in April 2018. One month prior to admission, his family noted that he showed poor oral intake and consistently complained of epigastric discomfort. He was diagnosed as having impaired fasting glucose and hyperlipidemia at the age of 42 on routine medical checkup. Eight months ago, he underwent total thyroidectomy with both central and sentinel lymph node dissection due to papillary thyroid carcinoma and the pathologic stage was diagnosed as T3N1bM0 on the permanent pathologic report. After that, the first radioactive iodine (RAI) therapy was conducted and an iodine [–] whole body scan was planned to determine whether to perform the second RAI that was on the next day of visiting the emergency room, therefore, he had to stop the thyroid medication for 3 weeks to prepare for the examination.\nAt the time of admission to the emergency room, he was noted to be somnolent and had a decreased level of consciousness. He opened eyes to pain, showed inappropriate verbal response and flexion withdrawal from pain, which suggested that Glasgow Coma Scale (GCS) was 10 out of 15. On physical examination, there was no pretibial edema and his pupils were equal in size and normally reactive to light. His abdomen was slightly distended with decreased bowel sound and his extremities were cold. His blood pressure was 127/96 mmHg, heart rate was 101 beats per a minute, and respiratory rate was 25 breaths per a minute with oxygen saturation 97% on room air. He was in a hypothermic state and his tympanic temperature was approximately 34.0 °C. Chest radiography revealed the findings of gastroparesis and paralytic ileus as presented in Fig. . An electrocardiogram at presentation showed sinus tachycardia with QT prolongation by 537 ms of corrected QT interval (Fig. ).\nArterial blood gas analysis revealed a state of metabolic acidosis: a pH of 7.00, partial pressure of carbon dioxide in arterial blood (PaCO2) of less than 10 mmHg, bicarbonate (HCO3) of less than 10 mmol/L, and base excess of − 34.6. Laboratory findings suggested hyperglycemia with glycosuria and ketoacidosis, which are presented in Table . Considering the history of total thyroidectomy and planned schedule for RAI, a thyroid function test (TFT) was conducted and revealed severe hypothyroidism. He was found to have a thyroid-stimulating hormone (TSH) of 34.126 uIU/mL (0.55–4.78 uIU/mL) and free thyroxine (T4) of less than 0.01 ng/dL (0.82–1.76 ng/dL) and triiodothyronine (T3) of less than 0.01 ng/mL (0.6–1.81 ng/mL). Even though he did not have any history of diabetes mellitus, we checked his glycated hemoglobin (HbA1c) due to hyperglycemia. Finally, the value of HbA1c was 16.5% which met the criteria for a diagnosis of diabetes.\nHe was admitted to the intensive care unit (ICU) for the management of DKA and myxedema coma. He received intravenously administered fluid with electrolytes and an immediately applied insulin pump. For hormonal replacement, liothyronine 5 mcg two times per day and levothyroxine 175 mcg once daily were administered via a nasogastric tube. He instantly responded to the therapy with a favorable clinical improvement. His mental status started to improve several hours after treatment and at the third day of hospitalization he showed a GCS of 15/15; his body temperature increased from 34 °C to 36.5 °C approximately 10 hours after admission. The metabolic acidosis was corrected 6 hours after administration of intravenously administered fluid with insulin pump and hyperglycemia was also improved; the insulin pump was discontinued then and switched to subcutaneous insulin 1 day after hospitalization. Repeated TFT before discharge revealed TSH of 21.798 uIU/mL (0.55–4.78 uIU/mL), free T4 of 1.02 ng/dL (0.82–1.76 ng/dL), and T3 of 1.04 ng/dL (0.6–1.81 ng/mL). The clinical course of this patient was summarized in Table . During the hospitalization, a workup for diabetes mellitus was performed and there was no evidence of pancreas mass or pancreatitis on abdominal computed tomography (Fig. ). Results from investigations for diabetes mellitus showed a fasting c-peptide of 1.08 ng/mL (0.48–3.30 ng/mL), anti-islet cell antibodies (ab) negative, and glutamic acid decarboxylase (GAD) ab of 0.01 U/ml which suggested that a diagnosis of type 2 diabetes mellitus would be appropriate.\nHe was discharged from surgical ICU after 2 days, stayed for a further 8 days on the general ward and was discharged on the 11th hospital day with tolerable status. The dose of thyroid hormone medications was subsequently reduced at our out-patient clinic after he was discharged and an endocrinologist recommend insulin with orally administered hypoglycemic agents.
A 67-year-old man visited the emergency department of our hospital due to sudden paraplegia of both lower extremities caused immediately after slip down. On presentation to the department, both his lower limbs were observed to be almost paralyzed, with a motor grade of 2 or lower in both limbs. This paralysis showed a gradual aggravation. The patient had a history of renal cell carcinoma diagnosed 7 years prior, and achieved remission without any recurrence after total nephrectomy and adjuvant chemotherapy. He was also diagnosed with papillary thyroid carcinoma 5 years ago, and yet again achieved remission after surgical treatment. The patient signed an informed consent statement, and the study was approved by the Institutional Review Board of the Yeungnam University Medical Center.\nThe plain radiograph revealed the collapse of the T12 vertebral body. CT showed an acute burst fracture at the T12 vertebral body with an osteolytic mass-like lesion within the vertebral body and pedicle, causing severe encroachment of the spinal canal (Fig. ). Magnetic resonance imaging (MRI) revealed a bulging posterior cortex, with an acute fracture of the vertebral body and severe compression of the spinal cord (Fig. ). Considering his medical history and the MRI findings, a pathologic fracture with a tumorous condition was suspected. Therefore, the evaluation of the malignant potential and metastasis in the remote area was necessary. Hence, 18FDG PET/CT was performed, and 18FDG uptake at the T12 level was measured. A maximum standardized uptake value (maxSUV) of 1.7 was noted, with a central FDG defect on the vertebral body (Fig. ); this was interpreted as a benign fracture by a senior radiologist. Additionally, there was no positive uptake in the other body parts on PET/CT. Although there was a discrepancy between the interpretation of the spine fracture lesion on MRI and PET/CT, the patient required emergent surgical treatment owing to acute paraplegia caused by spinal cord compression, and we thus planned an initial surgery for the main purpose of cord decompression, temporary stabilization, and tissue biopsy. The surgical intervention was as follows. First, we performed T11-12 posterior laminectomy for the spinal cord decompression, followed by T9-L2 posterior instrumentation without a bone graft. Finally, tissue biopsy was obtained at the T12 pedicle and vertebral body. Histological examination confirmed the presence of a malignant solitary bone plasmacytoma (SBP) (Fig. ). Seven days later, a definite surgery with pedicle screw fixation and posterolateral bone graft from T8 to L2 without vertebral body corpectomy was performed.\nImmediately after the initial surgery, the patient's lower extremity motor function showed gradual improvement. After the surgery, the patient wore a thoracolumbar rigid brace for 2 months, and underwent continuous gait rehabilitation. Three weeks postoperatively, the motor function in his lower extremities had recovered sufficiently, and ambulation was possible with the assistance of a cane. Four weeks after the definite surgery, the patient underwent radiation therapy for 2 months. One year postoperatively, spine radiographs showed that the surgical segment had healed fully, and spine MRI revealed complete decompression of the spinal cord with no evidence of local recurrence (Fig. ).
A 52-year-old female with achondroplasia presented with an 8-month history of progressive weakness in the upper extremities with numbness and a gait disturbance. A neurological examination revealed weakness (grade 3/5) in the bilateral intrinsic muscles and hyperreflexia in the bilateral upper extremities with a positive Hoffmann sign; the knee and ankle reflexes were also increased bilaterally. She had decreased sensation in the bilateral upper extremities and a spastic gait with clumsiness of her hands, although she had no bowel or bladder difficulties. Lateral radiographs showed hypoplasia of C1. Computed tomography (CT) disclosed bony canal stenosis caused by the C1 posterior arch and edge of the occipital bone [], and magnetic resonance (MR) imaging demonstrated atrophy of the spinal cord and a high-intensity intramedullary lesion at the C1 level [].\nThe patient underwent posterior decompression of the CVJ. We resected the C1 posterior arch and the rim of the occipital bone (approximately, 7 mm from the edge of the foramen magnum). Intraoperative ultrasonography confirmed sufficient resection of the bony structure. At this stage of operation, the dura matter did not expand significantly. The thickened reactive fibrous tissue band was noted between C1 and the occipital bone []. Because sparing this fibrous tissue band carefully from dural matter did not improve compression at CVJ, the band and the dura mater underneath were carefully dissected longitudinally to achieve decompression, and the dura mater was left open without a patch graft. The arachnoid membrane was preserved []. The patient's motor weakness and gait disturbance improved immediately after surgery. Although the arachnoid membrane appeared intact during the surgery, the output of suction drainage indicated CSF leakage. One week after the surgery, she complained of a severe headache and nausea. Postoperative MR imaging revealed a moderate collection of CSF at the surgical site. Brain CT demonstrated bilateral infratentorial subdural fluid collection []. As the neurological condition of the patient was stable, she was managed conservatively with intravenous hydration. On the 17th day, after the surgery, her consciousness level suddenly deteriorated, and she became drowsy and unresponsive to verbal stimuli. In the first place, overdose of hypnotics was suspected. An emergency brain CT scan revealed an increased volume of infratentorial fluid collection and marked hydrocephalus with an obstructed cerebral aqueduct [Figures and ]. Following rapid deterioration of the patient's neurological status, she underwent emergency external ventricular drainage and subsequent re-exploration of the surgical site. Additional wide craniotomy and Y-shaped durotomy were performed as in FMD. Subdural hemorrhage and hygroma were noted in the subdural space, which was suctioned cautiously. Duraplasty using the occipital periosteum as a patch graft was performed, and the patient's consciousness level improved to normal immediately after the revision surgery. The external ventricular drain was removed 5 days after the revision surgery, and follow-up imaging studies demonstrated the complete resolution of hydrocephalus. At the 12-month follow-up visit, she had regained her full strength and was able to walk without assistance.
A 56-year-old male visited our hospital with New York Heart Association functional class IV dyspnea and pitting edema in his lower extremities. The patient had been diagnosed with tricuspid valve regurgitation and atrial fibrillation at an outside institution 10 years ago. However, the patient had refused surgical treatment at that time. Physical examination and laboratory tests demonstrated severe hepatomegaly, ascites, azotemia, and cardiac cirrhosis (Child-Pugh class B). Echocardiography and cardiac magnetic resonance imaging showed severe tricuspid valve regurgitation, with a persistent left superior vena cava (SVC) draining into the coronary sinus and absence of the innominate vein. Magnetic resonance imaging revealed left and right ventricular ejection fractions of 31.5% and 34.2%, respectively. Heart transplantation rather than a tricuspid valve surgery was recommended because of the biventricular dysfunction.\nPreoperative computed tomographic venography of the neck and brain was performed to evaluate the probable risk of a left SVC ligation. The diameters of the right and left SVCs were 17 mm and 16 mm, respectively. Abundant collaterals were built up at the sigmoid sinus, which was considered adequate for draining venous blood from the left part of the head to the right SVC. This finding suggested that the risk of cerebral venous congestion after left SVC ligation was minimal.\nWhen opening the pericardium during heart transplantation, the recipient heart showed a large dilated distal part of the left SVC draining into the coronary sinus. Under moderate hypothermia, orthotopic heart transplantation was performed with the standard aorto-bicaval cannulation technique using an additional venous cannula on the left SVC. The donor heart was implanted using the standard bicaval technique. Despite the preoperative evaluation suggesting left SVC ligation would be safe in this patient, the left SVC was anastomosed to the right atrial appendage using an interconnecting conduit (). A tubular conduit 16 mm in diameter and 4 cm in length was constructed using the resected coronary sinus tissue to avoid size discrepancy and kinking. After the orthotopic cardiac transplantation was completed using the bicaval anastomosis technique, the tubular conduit was anastomosed to the distal part of the left SVC and to the right atrial appendage using continuous 5-0 polypropylene sutures. The cardiopulmonary bypass and donor ischemic times were 354 minutes and 125 minutes, respectively. The postoperative course was uneventful. A computed tomography angiogram at one month after the surgery demonstrated a patent conduit between the left SVC and right atrial appendage (). The patient was discharged without any complications and did not show any sign of upper-body venous congestion at the six-month follow-up visit.
A 6-year-old girl, who happened to have swallowed a 100 FCFA coin (diameter 22 mm, ) on April 2014, was taken immediately to an ENT physician because of esophageal symptoms. An attempt (by the physician) to remove the coin with the aid of a rigid esophagoscope was unsuccessful and was rather complicated by perforation of the esophagus. The child was taken to a tertiary hospital in Douala (Cameroon) three days after the accident, where a chest radiograph and CT scan revealed a massive right empyema (). A chest tube was placed and she was transferred to the intensive care unit, where she was placed on broad spectrum antibiotics. She was transferred to Yaoundé 12 days after the incident because her clinical state did not improve. On arrival, she was in severe respiratory distress and septic shock due to mediastinitis and empyema. The chest radiograph done on the day she arrived our casualty unit is shown in . Immediate resuscitation was performed and the child was taken to the operating room, a few hours later. A right thoracotomy was done entering the chest at the level of the 5th intercostal space. Findings were massive empyema in the fibrinopurulent stage. The tear could not be located because of the magma of fibrin tissues. The coin was impacted just above the lower esophageal sphincter; thus it could not go through the sphincter. The esophagus was diffusely inflamed and as such very fragile. We removed as much fibrinopurulent tissue as we could and washed the pleural cavity until it became visibly clean. We then gently pushed the coin upwards and did a small longitudinal incision 3 cm below the azygos vein, through which the coin was removed. The hemodynamic condition of the child and our working environment did not permit for a longer procedure. We thus choose to be conservative. A nasogastric tube was inserted and then obstructed. A local pleural flap was used to buttress the sutured area. We washed the pleural cavity again and did a decortication. The corresponding lower lobe was rather inflammatory and void of pus. We left a single chest drain in place using the simple drainage system as we have reported []. The surgery took about 80 minutes from skin to skin. Postoperatively, she was taken back to the intensive care unit and placed on imipenem and metronidazole (the child had received amoxicillin/clavulanic acid, cefotaxime, and ceftriaxone in the preoperative period). Extubation was done on the same day of surgery and she was placed on 3 liters of nasal oxygen/min for 48 hours.\nThe patient was discharged from the intensive care unit on the 3rd day after surgery. The drainage remained purulent for 2 weeks. Daily care included antibiotics, high protein diets, respiratory physiotherapy, and wound care. She was fed exclusively through the NGT for the first 10 days after surgery. Oral feeding was resumed on the tenth day following surgery. However, the NGT tube was left in place for a further ten days for complementary feeding and removed on the twenty first day after surgery. She remained febrile for three weeks and was maintained on antibiotics. The drain was removed after 18 days. She stayed in the hospital for 1 month and was discharged on oral antibiotics after 5 days of restoration of strength and the absence of fever.\nShe was followed up every week as an outpatient. Two weeks after discharge, she began running a low-grade intermittent fever for which we requested an esophagogram (Figures and ). The findings were normal (and without any leak). She was readmitted 2 months after surgery because of the persistence of fever (this time of high-grade). A chest radiograph revealed a right lower lobe abscess. She was replaced on imipenem and was taken back to the operating room on the fifth day of admission because of the persistence of sepsis despite broad spectrum antibiotics. A thoracotomy, through the previous incision, was reconducted. We found a lower lobe abscess with destruction and gangrene of the right lower lobe. The esophagus was normal and without any signs of inflammation. We carried out just decortication and a lobectomy. The postoperative period was uneventful. Fever subsided on the day after surgery; meanwhile the drain was removed 5 days after surgery and she was discharged 10 days after the operation. She is followed up regularly as an outpatient. The chest radiograph one year after the surgery is shown on and is almost normal. She is currently healthy and doing well.
A 54-year-old man with a past medical history significant only for hypertension presented to our clinic with a large soft tissue growth on hislower back which had been present for the past 20 years. Over the past three years it had been rapidly enlarging, nearly doubling in size over that time frame. He had recently re-established medical care after having not seen a physician since childhood. At the time of presentation, he denied any pain or tenderness over the mass and denied any systemic symptoms such as fever, night sweats, and weight loss. The patient had an unremarkable physical exam except for the large soft tissue mass over the lower back, with the maxiumum dimension measured to be 38cm () .\nAfter evaluation by the surgical oncology and radiation oncology services, an abdominal CT scan was obtained and thisdemonstrated a large (35 cm, x 38 cm x 17 cm), heterogeneous soft tissue mass. A differential diagnosis consisting of teratoma versus liposarcoma was established based on the radiologic imaging. Subsequently, several core biopsies of the mass were performed, all of which revealed fat necrosis with calcifications.Surprisingly, given the size and rapid growth of the mass, a diagnosis of benign giant lipoma was made.\nFour weeks after presentation, several surrgical teams performed a six hour operation to remove the 14 kilogram mass. After the patient was widely prepped and draped, the skin overlying the central portion of the tumor was shaved and harvested as multiple split thickess skin grafts (). Subsequently, an incision was made in the skin overlying the tumor in an area outside the skin graft donor sites, preserving significant flaps in all dimensions to permit primary closure (). Numerous, large variceal vessels feeding the tumor were ligated as the tumor was dissected off of the paraspinous muscles, which constituted the deep margin.\nThe specimen was sent for frozen section analysis,which was consistent with a lipoma, and was confirmed on final the pathology. The defect was able to be closed primarily with the preserved skin flaps, which measured greater than 200 cm × 40 cm (). The skin flaps were de-epithelialized and imbricated to achieve a multi-layered closure of the entire back wound, obliterating as much of the deadspace as possible. Two subcutaneous closed-suction drains were placed prior to the final closure. Postoperatively, the patient did well without complication (). After a brief and uneventful hospital stay postoperatively, he was discharged home in good condition. On follow-up, his drains were sequentially removed and the incision line has healed without problems. He has not had any evidence of recurrence or infection at six months postoperatively.
A 17-year old male professional runner was transferred to our musculoskeletal tumor centre on recommendation from his team doctor. Currently a member of a national biathlon junior team, the patient was one of the best track and field athletes of his age group for mid-distance running disciplines (1500 m & 3000 m) on a competitive level at the time of initial diagnosis. Besides daily workout in the gym for strentgh and endurance, his training schedule included up to 4 h running 6 days per week. However, ongoing pain in both of his shins prevented the young athlete from training and competition despite intensive conservative treatment for 5 months. Before admission to our hospital, symptoms were treated for shin splints without success until MRI was conducted. Extensive cyst-like bone lesiosn were found in both diaphyseal tibiae (Figs. and ). Imaging was completed by plain radiography of both tibiae (Figs. and ).\nAs multimodal conservative treatment for MTSS demonstrated unsuccessful and MRI revealed the most likely reason for the patient’s symptoms, surgical therapy was indicated after thorough analysis of all clinical and radiological findings. In favour of a fast return to a competitive sports level we abandoned our standard surgical procedure for UBC consisting of open curettage and autologous cancellous bone grafting from the iliac crest. Due to the dimensions of both tibial lesions, this would have been associated with a long recovery and potential major donor site morbidity both seriously jeopardizing the young athlete’s competitive sports career. Based on promising reports of minimally invasive surgery for treatment of UBC of the humerus from Hou et al. [, ] we performed a customized surgical technique for the first time at the lower extremity beginning with the more symptomatic left lower leg. Surgery was conducted in a supine position without use of a pneumatic tourniquet to prevent possible muscular impairment. The exact dimension of the cyst and its distance from the lateral knee joint line and the tibiotalar joint line were marked on the skin. Intraoperative fluoroscopy was used to confirm correct localization. After a skin incision of two inches (Fig. ) the soft-tissue layers were dissected bluntly in a longitudinal direction down to the fascia of the anterior tibial muscle. The muscle was stripped from the antero-lateral facet of the tibia with its fascia, leaving a narrow strip of fascia on the edge of the tibia allowing easy later refixation. After detachment of the periost, a small bony fenestration was created by 2 mm K-wire cortical corner drillings and connecting oscillating saw cuts. Sawcuts were placed slightly tangential to hinder the bony lid to fall through the window after curettage and grafting. Before completing all four sides of the bony window further drill holes on each side of the cranial and caudal osteotomy site were placed for additional suture cerclages for later reattaching the lid. Then the content of the cyst was aspirated and sent for cytological analysis, followed by a routinely smear test for microbiological analysis. The membrane lining and any septa within the cyst were disrupted and removed with a long flexible arthroscopic ring curette (Arthrex, Germany). Specimen of the membrane were sent for histoptahologic analysis. Fluoroscopy ensured treatment of the entire cyst. Hou et al. (Taiwan) [] proposed the use of custom-made curved curettes and impactors. In accordance with current medical laws, we converted several surgical instruments for our needs: A 2.0 mm K-wire was fixed in a universal chuck with T-handle (Synthes, Switzerland) and its sharp end bent 60–90° to use as a scraper. Furthermore a 6.0 mm sponylodesis titan rod was bent and utilized as impactor. After repeated chemical cauterization with 95 % ethanol and intermittent irrigation with normal saline solution, grafting was performed. For grafting, autlogous bone material was not the preferred choice for obviuos reasons. Bone substitutes like calcium sulfate pellets, as suggested by several other authors, have not met our expecetations in the past, can show poor osteointegration in adult patients and can entail adverse effects []. Thus allogenic cancellous bone (Tutoplast©, RTI Biologics/Tutogen Medical, Neunkirchen/Germany) was administered and firmly impacted within the cyst space. Finally, the fenestration was closed by reattaching the cleaned bone cover and secured with transosseous sutures. A cannulated 6.5 mm screw was inserted caudally the small fenestration under fluoroscopic guidance to provide continuous decompression.\nHistopathologic examination confirmed the diagnosis of UBC. Microbiology results of the smear test showed no signs of bacterial infection. After complete wound healing the patient was allowed to resume modest physical activity with cycling and swimming to regain muscular strength and decrease muscle atrophy. Six months after initial surgery on the left tibia and 3 months after last surgery on the contralateral right side the athlete had regained his full training capacity on a competive level and has been free of symptoms eversince. Clinical and radiographic follow-ups showed desirable bony consoldidation of the tibiae without signs of recurrence (Figs. and ). Three years after initial surgery, both cannulated screws were removed percutaneously. Proving the therapeutical succes of our less-inavsive procedure and showing a desirable healing process, the athlete qualified for this years Biathlon Junior World Championships in Belarus 8 month after final surgery.
This 20-year-old woman had no other medical history. The patient had been suffering motor weakness in both upper extremities and radiating pain in both hands since 3 weeks ago. She experienced progression of pain, sensory changes, and lopsided motor weakness to the left side of the body. At the initial physical examination, a motor grade of both upper extremities was determined as grade 4 and no weakness was found in lower extremities. She was generally mildly hypertonic, although there was no Babinski sign or clonus. She complained of mild radiating pain in both hands, which was not one-sided with inexplicit dermatome. The patient underwent brain and whole spine MRI, which revealed a heterogeneously enhancing mass in the central posterior aspect of the spinal cord at the C1-6 level (). Brain MRI showed no specific findings. She was suspected of a tumorous condition, particularly ependymoma. Following this, she was admitted to our hospital for further evaluation of neurologic change. On the second hospital day, her motor function in both upper extremities suddenly deteriorated from grade 4 to 3. Cervical MRI was performed again, which revealed further extension of the hyperintense lesion on T2 weighted image and more intramedullary enhancing lesion on the T1 weighted image than that on the initial MRI image (). We hypothesized that the origin of neurologic deterioration was spinal cord compression by tumor bleeding. We immediately started intravenous steroid therapy and carried out total laminectomy C2-6 for decompression with surgical biopsy.\nWe performed C2-6 laminectomy and after opening the dura, the spinal cord was edematous but otherwise normal (). In the operation field, there was no tumor like lesion, discoloration or hypervascularization. We obtained frozen biopsy from a site near the lesion which was obtained consistent with astrocystic cell proliferation. Due to the gross normal finding and no tumorous frozen biopsy, we stopped the removal procedure of the mass lesion to prevent another potential spinal injury. The operation was closed by lateral mass screw fixation and fusion C2-6.\nOn the first postoperative day, her vital signs were stable, and suffered from nuchal pain, but her motor weakness remained the same. However, from the second postoperative day, motor weakness in both upper extremities became gradually better along with improvement of other sensory symptoms. On the twelfth postoperative day, permanent biopsy revealed abundant histiocytic collection, perivascular lymphocytic inflammation and reactive astrocytic proliferation. There was no evidence of a neoplastic process and no oligoclonal bands. The pathologist strongly suggested the diagnosis of neuromyelitis optica. Only then did we investigate her ophthalmic symptoms with a suspicion of another disease. She recalled her visual disturbance 5 years ago. Since then, there was no symptom until visit our department. We referred her to ophthalmology and revealed optic atrophy with disc pallor with optic neuritis on her left eye. Finally, her diagnosis was determined on NMO.\nOn the fourteenth postoperative day, her motor grade was improved to grade 5 and the patient could perform her daily tasks including eating food. We continued postoperative steroid treatment and started rehabilitation. After rehabilitation, she regained most of her upper-extremity motor functions and her ability to walk. Her pain significantly disappeared. When she was discharged from the hospital, there remained only mild nuchal pain. After 3 month from surgical intervention, her follow-up cervical MRI showed dramatic improvement ().
A 76-year-old woman sought treatment in the USPUH Emergency Department with a 3-day history of hypoactivity and loss of appetite. The patient reported that she had experienced a drop in the level of consciousness on the previous day. She also reported that she had fallen from standing height 15 days prior to the onset of the symptoms.\nThe patient had a personal history of chronic obstructive pulmonary disease and had been on home oxygen therapy for the last six years. She reported that she did not have arterial hypertension or diabetes and had experienced no cardiovascular events. The patient had a family history of cancer, her father having died at age 52 years from carcinoma of the oropharynx and her mother having died from cancer, the primary site of which was unknown to the patient.\nPhysical examination revealed dehydration, pallor, cyanosis, and extensive ecchymosis on the right side of the face. The patient presented with spatiotemporal disorientation (Glasgow coma scale score, 13). Her temperature was 36 °C, her blood pressure was 90/50 mmHg, her heart rate was 90 bpm, and her peripheral oxygen saturation was 68% on room air. There were no signs or symptoms of heart disease, lung disease, or abdominal disease. In addition, there were no signs or symptoms of upper- or lower-limb disorders. Her capillary blood glucose level was 80 mg.dL–1.\nA presumptive diagnosis of hypoactive delirium was made, and tests were ordered (). A bedside chest X-ray was taken. Although it is technically difficult to interpret chest X-rays taken under such conditions, the findings were suggestive of a focus of infection in the right lung base (). Because the information in the medical chart of the patient had been constantly updated, it was possible to compare the results of the arterial blood gas analysis performed at admission with those of that performed in 2004.\nThe patient underwent aggressive hydration with saline solution. She was diagnosed with pneumonia (a PORT score of 236, risk class V) and exacerbation of chronic obstructive pulmonary disease. She was started on empirical treatment with 2 g/day of ceftriaxone. On the following day, clarithromycin (500 mg every 12 hours) was added. Her level of consciousness improved, as did her renal function and leukocyte count.
Our patient is a 53-year-old male who worked as a coal miner. In spring of 2017, he noticed enlarging scalp nodules and right hip pain. He also complained of fatigue but was otherwise asymptomatic. Because of the hip pain and nodules, he came to the emergency department where a CT scan showed an enlarged right frontal scalp mass that measured about 4 cm (). MRI also revealed a right frontal scalp mass and extensive vasogenic edema localized to the right frontal lobe with a 5 mm midline shift at the foramen of Monroe (). A subsequent CT chest, abdomen, and pelvis was performed and showed a lytic lesion in his ilium and a single enlarged right hilar lymph node (not shown). A follow-up nuclear medicine bone scan demonstrated multiple areas of increased uptake concerning for metastasis (). One of the largest areas was in the right frontal scalp. After discussion with the patient and the need for a definitive diagnosis, he was consented for subtotal resection of the right frontal scalp mass.\nHe was taken to the operating room where a right frontal scalp incision was performed for subtotal resection of likely metastatic cancer. The mass was primarily located within the right frontal scalp but extended through the skull into the right frontal cortex. It did not however appear to be involved with the brain parenchyma. The resection consisted of removing scalp mass and taking a core biopsy from the specimen. Histopathology of the biopsy showed a diffuse and monotonous infiltrate composed of small cells with irregular nuclei, condensed chromatin and inconspicuous nucleoli (). On immunohistochemistry, the cells are CD20 positive B-cells and co-express CD10 and Bcl-2 but do not express CD3, 5, 23, 43 and cyclin D1 (). CD21 was present highlighting a disrupted follicular dendritic cell meshwork. Ki-67 proliferation rate was low (less than 20%). By flow cytometry, a dim kappa light chain-restricted B-cell population was identified expressing CD10. The FISH assay revealed t(14;18) IGH (immunoglobulin heavy chain locus)-Bcl-2 fusion. These findings support the definitive diagnosis of follicular lymphoma, WHO grade 1, diffuse pattern stage IV.\nAfter definitive diagnosis was made, the patient was then referred to radiation oncology for follow-up. By time of presentation to radiation oncology, the patient was showing symptoms of lower extremity numbness, personality changes, and some memory loss. The radiation oncologist discussed the diagnosis with the patient and his family and came up with a treatment plan to combat the unusual presentation of follicular lymphoma with the intracranial extension from the calvarium. The treatment plan consisted of repeat MRI’s; local (palliative) XRT (4 Grays over two fractions) and systemic chemo-immunotherapy (6 cycles) followed by rituximab maintenance. Lumbar puncture was done a month after resolution of vasogenic edema to assess leptomeningeal spread later proven negative for malignant cells. MRI showed complete resolution of the right scalp mass post-radiotherapy and after the patient had already underwent 3 cycles of chemo-immunotherapy. PET/CT done approximately 3 months since therapy initiation showed no suspicion for abnormal hypermetabolic activity, which suggested the absence of active malignancy.
A 47-year-old female presented to an outside institution with chronic low back pain and right L5 radiculopathy, and an MRI of the lumbar spine was performed (not shown). It showed a mass-like lesion at the right L5–S1 foramen and a diagnosis of hernia or tumour was considered. Over a 1-month period, the patient was managed clinically with analgesics with partial resolution of symptoms.\nThe patient then presented to the emergency department of our hospital owing to her persistent symptoms and an MRI of the lumbar spine (T1 and T2 weighted sequences) was obtained (). At this point, no contrast injection was performed as it is not included in the low back pain/radiculopathy investigation protocol in the emergency department. It showed the previously described lesion at the right L5–S1 foramen, as well as other ipsilateral lesions in the posterior paraspinal muscles and the anterior epidural space. Most of the lesions had a mass-like appearance and low signal intensity in all MR sequences. Interestingly, one lesion in the posterior paravertebral muscle showed mixed signal intensity on T2 weighted sequence, defining a fluid–fluid level. All the lesions were unchanged compared with the outside scan. The radiology team suspected that the lesions were calcified and suggested a contrast-enhanced CT scan of the lumbar spine ( and ) to confirm their initial suspicion and rule out possible soft tissue components associated with the lesions. The CT scan confirmed the calcified nature of the lesions and also showed no contrast enhancement. It also contributed to an additional finding of interstitial lung disease, as seen in a few images at the base of the lungs in the thoracolumbar region. A CT scan of the chest was suggested () and showed signs of pulmonary fibrosis and oesophageal dilatation. At this time, the hypothesis of paraspinal tumoral calcinosis secondary to SSc was suggested.\nAdditional clinical investigation showed that the patient had a history of dyspnoea, generalized weakness, arthralgia and gastro-oesophageal reflux disease. On physical examination, Raynaud’s phenomenon with a distal phalanx skin ulcer of the left third digit, sclerodactyly and multiple facial telangiectasias were also observed. Laboratory analysis indicated that creatinine, calcium and phosphorus levels were normal. In fact, the patient had an established diagnosis of lcSSc over a period of 15 years. However, this information was not provided to the radiology department, making the diagnosis of lumbar paraspinal tumoral calcinosis secondary to lcSSc challenging.\nAfter a multidisciplinary team meeting, it was initially decided that the patient’s radiculopathy should be managed clinically with non-steroidal and steroidal anti-inflammatory drugs and follow-up MRI of the spine should be performed within 6 months. In case of non-resolution or worsening of the symptoms during the follow-up interval, it was decided that surgical decompression could be considered depending on the patient’s general clinical status.
A 54-year-old man with a past medical history significant only for hypertension presented to our clinic with a large soft tissue growth on hislower back which had been present for the past 20 years. Over the past three years it had been rapidly enlarging, nearly doubling in size over that time frame. He had recently re-established medical care after having not seen a physician since childhood. At the time of presentation, he denied any pain or tenderness over the mass and denied any systemic symptoms such as fever, night sweats, and weight loss. The patient had an unremarkable physical exam except for the large soft tissue mass over the lower back, with the maxiumum dimension measured to be 38cm () .\nAfter evaluation by the surgical oncology and radiation oncology services, an abdominal CT scan was obtained and thisdemonstrated a large (35 cm, x 38 cm x 17 cm), heterogeneous soft tissue mass. A differential diagnosis consisting of teratoma versus liposarcoma was established based on the radiologic imaging. Subsequently, several core biopsies of the mass were performed, all of which revealed fat necrosis with calcifications.Surprisingly, given the size and rapid growth of the mass, a diagnosis of benign giant lipoma was made.\nFour weeks after presentation, several surrgical teams performed a six hour operation to remove the 14 kilogram mass. After the patient was widely prepped and draped, the skin overlying the central portion of the tumor was shaved and harvested as multiple split thickess skin grafts (). Subsequently, an incision was made in the skin overlying the tumor in an area outside the skin graft donor sites, preserving significant flaps in all dimensions to permit primary closure (). Numerous, large variceal vessels feeding the tumor were ligated as the tumor was dissected off of the paraspinous muscles, which constituted the deep margin.\nThe specimen was sent for frozen section analysis,which was consistent with a lipoma, and was confirmed on final the pathology. The defect was able to be closed primarily with the preserved skin flaps, which measured greater than 200 cm × 40 cm (). The skin flaps were de-epithelialized and imbricated to achieve a multi-layered closure of the entire back wound, obliterating as much of the deadspace as possible. Two subcutaneous closed-suction drains were placed prior to the final closure. Postoperatively, the patient did well without complication (). After a brief and uneventful hospital stay postoperatively, he was discharged home in good condition. On follow-up, his drains were sequentially removed and the incision line has healed without problems. He has not had any evidence of recurrence or infection at six months postoperatively.
A 30-year-old female with a past medical history including bipolar disorder, major depressive disorder, severe alcohol use disorder, alcoholic hepatitis, chronic pancreatitis, and gastric ulcers presented to an outside hospital with hematemesis and malaise. She was found to have acute alcoholic hepatitis, and treatment was started with supportive therapy, prednisone, and pentoxifylline. After failing to show signs of improvement, the patient was transferred to our hospital for extracorporeal liver assist device evaluation. Her Model for End-Stage Liver Disease score was 26. She had significant comorbidities including pancreatic insufficiency, candida esophagitis, and positive blood cultures for Klebsiella. Her condition continued to deteriorate until she developed acute respiratory failure with encephalopathy requiring endotracheal intubation and intensive care unit admission. A computed tomography scan was performed emergently, and it showed subarachnoid hemorrhage secondary to coagulopathy associated with her liver disease. She continued to have persistent muscular weakness and was unable to wean from the ventilator. After 2 weeks, she was scheduled for a bedside percutaneous tracheostomy.\nThe patient was stabilized hemodynamically but continued to have ongoing coagulopathy with thrombocytopenia, elevated INR, and PT. The initial tracheostomy placement was complicated by false passage with a left pneumothorax that required a chest tube placement. Eight days after the initial tracheostomy placement, a persistent cuff leak was noted and required emergency reintubation with an oral endotracheal tube. The patient then underwent a surgical tracheostomy revision complicated by a right-sided pneumothorax, which also required a chest tube placement.\nSix days after her tracheostomy revision, she was noted to have massive bleeding from the tracheostomy site with severe occlusion of airway anatomy. Initially, bleeding was noted at her tracheostomy site. Pressure was held at the tracheostomy site to control bleeding, but then she was noted to have pooling of blood into the oropharynx and nose. There was a high suspicion of tracheo-innominate fistula.\nKnowing the patient's ongoing coagulopathy with worsening INR and thrombocytopenia in the setting of acute bleeding, the patient was taken to the operating room (OR) emergently to control bleeding and secure the airway. The patient had an arterial line in place, and other standard ASA monitors were available continuously throughout the resuscitation. Our anesthetic management focused initially on verifying a patent airway despite profuse bleeding through the tracheostomy site while maintaining spontaneous ventilation with inhaled agents, small doses of midazolam/fentanyl, ketamine infusion and maintaining hemodynamics with transfusion of blood products and norepinephrine/vasopressin infusion.\nUpon arrival to the OR, surgeons began exploring the tracheal site and found significant bleeding from the distal tracheal site, which was presumed to be from the innominate artery. The region was packed, and acute bleeding temporized. At this time, a bronchoscopy performed by the ENT surgery team through the tracheostomy site showed a right mainstem insertion of the tracheostomy tube. It was noted that the patient had a low tracheostomy site, being only 1.5 cm from the carina.\nAfter further verifying that the majority of the bleeding was from the inferior region of the trachea, thoracic surgeons were called for help. It was not possible to control the bleeding through the neck incision, and it was noted that the posterior aspect of the artery was friable and lacerated.\nAnticipating sternotomy and the need for a secured airway and use of paralytics, an oral endotracheal tube (ETT) was placed with aid of a video-laryngoscope just above the tracheostomy site. Then, a bronchoscope was passed through the oral ETT to visualize the distal trachea beyond the tracheal defect. At this point, a Cook catheter was placed through the tracheostomy tube and into the mainstem bronchus to allow a track in case of a lost airway situation. The ETT was then advanced under direct visualization into the distal trachea. ETT placement was confirmed under visualization with the bronchoscope, and then, the Cook catheter was removed.\nThe patient was paralyzed at this point. The surgeons performed a sternotomy for better exposure of the innominate artery. With the upper sternum open, surgeons dissected the anterior mediastinum towards the trachea and immediately noted that the bleeding was coming from a TIF fistula. The defect was a 5 × 2 mm hole in the innominate artery. It was repaired in a stepwise fashion so as to not fully cut off the blood supply to the distal innominate artery. At first, primary closure of the tracheal defect was attempted; however, it was unsuccessful. Therefore, the thoracic surgeons were once again involved, and they opened the abdomen to perform an omental flap for closure of the tracheal defect. For the rest of the surgery, the patient's airway was adequately maintained and she was transferred to the ICU intubated for further stabilization. Overall, the patient received 8 units of PRBC, 5 units of FFP, and 1 unit of platelets to address her bleeding and coagulopathy.\nPostoperatively, the patient's clinical status remained tenuous with ongoing requirements for vasopressor support, mental status decline, and renal failure. The patient died several days later after a decision was made by the family to withdraw the ventilator and hemodynamic support.
A 23-year-old male collegiate football player, who experienced a sharp pain in his left leg during practice, presented to an orthopaedic surgeon for evaluation 18 hours after the incident. The athlete was reportedly participating in cutting drills when he experienced a sharp, snapping pain in his left leg just distal to the fibular head. He reported no significant trauma nor was there any twisting or indirect injury. He confirmed working through the pain and continuing the drills. The pain worsened throughout practice, which prompted him to approach the athletic training staff. Ice, a compressive wrap, and nonsteroidal anti-inflammatory drugs (NSAIDs) were administered and the patient was advised to go home and elevate the extremity. Due to escalating pain through the night, he was brought to the local emergency department by a team trainer. An ultrasound revealed no evidence of deep venous thrombosis. After being prescribed a narcotic for the pain, he was discharged to go home. However, the pain continued throughout the night and he began having paresthesias in the superficial peroneal nerve distribution of his left foot. The trainer was contacted again the next morning and the patient was immediately brought to the orthopedic operating center for further evaluation.\nPast medical and surgical histories of the patient were unremarkable. A review of systems was notable for extreme pain in the left leg, but otherwise negative for any clinical illness. He confirmed taking NSAIDs and the pain medication from the emergency department, but denied intake of any other medications, including supplements.\nOn examination, the patient appeared to be in significant distress. Visual inspection of his extremity revealed swelling over the lateral compartment, most notably approximately 5 cm distal to the fibular head. There was no ecchymosis or open wound. Palpation revealed a tense and firm lateral compartment. He held his foot in a plantarflexed and inverted position. There was no pain with the passive range of motion of his toes, as well as with passive dorsiflexion and plantarflexion of the ankle. However, he experienced pain with both passive eversion and inversion of his ankle, which he localized to his lateral compartment. The patient had gross numbness on the dorsum of his foot, including the first dorsal web space, which was consistent with the superficial peroneal and deep peroneal nerve distributions. Sensation remained intact in the lateral, medial, and plantar aspect of his foot. Capillary refill was less than 2 seconds and palpable dorsalis pedis and posterior tibialis pulses were present. X-ray studies showed no evidence of a fracture.\nHe was immediately taken to the operating room for fasciotomy; this was approximately 19 hours postinjury. The preoperative diastolic blood pressure was measured as 86 mmHg. In the operating room, pressure was measured using a Stryker handheld compartment pressure monitor (Stryker, Mahwah, NJ, USA): the lateral compartment measured 139 mmHg (delta pressure = -53), the anterior compartment measured 89 mmHg (delta pressure = -3), the superficial posterior compartment measured 30 mmHg (delta pressure = 56), and the deep posterior compartment measured 48 mmHg (delta pressure = 38).\nThe patient underwent fasciotomies involving a release of the anterior and lateral compartments. A linear incision was made midway between the tibial crest and the fibula. The lateral compartment was then released, which exposed a large tear involving the proximal aspect of the peroneus longus muscle belly, which was believed to be the cause of this compartment syndrome (). Approximately 20% to 25% of the peroneal musculature was dark burgundy, friable, and unreactive to electrocauterization. Nonviable tissue was debrided until bleeding commenced and responsive muscle was obtained (). A significant hematoma, which was present at the exit of the superficial peroneal nerve from the lateral compartment into the anterior compartment, was removed. Next, an incision was made over the anterior compartment; it exposed viable musculature that was reactive to electrocautery; therefore, no debridement was performed. Vertical mattress stitches were used to distally close the skin and cover the peroneal nerve. Proximally, the incision was left open and a KCI V.A.C. Ulta Negative Pressure Wound Therapy System device (San Antonio, TX, USA) was applied with a pressure of 125 mmHg on "continuous" mode ().\nPostoperatively, the ipsilateral foot was placed at 90° in a posterior splint to prevent contracture. The initial recovery was unremarkable with immediate improvement in pain. On postoperative day two, the patient was taken back to the operating room for repeat irrigation and debridement, removal of any nonviable muscle tissue, and partial closure of the wound. Minimal debridement was necessary and 75% of the wound was closed while the wound VAC device was applied to the remaining section. On postoperative day five, repeat irrigation, minimal debridement, and final closure of the wound were performed.\nInitially, the patient was advised to bear weight as tolerated in a controlled ankle motion boot with eventual transition to crutches by 2 weeks postoperatively. During this time, he worked on ankle range of motion and conditioning using a stationary bike. By 3 weeks postoperatively, sensation had returned to the dorsum of his foot. From postoperative weeks three to eight, he began manual resistance training, and was transitioned to regular shoes. Eight weeks postoperatively, he was pain free with full range of ankle motion. He was cleared for complete athletic participation at 12 weeks after surgery.
A 65-year-old single Japanese man was referred to our hospital with tremendous enlargement of the penis. There was no particular psychiatric or family history, and the patient had never travelled to a tropical region. Approximately 4 years prior to admission, he began to roll a commercially available rubber band (4.5 cm in diameter) around the penile root and foreskin, and used it throughout the day and evening to prevent urinary incontinence. Two years after beginning such rubber band use, the patient noticed gradual swelling and deformity of the penis, but continued to use the rubber band, resulting in progressive enlargement.\nA physical examination revealed brown colored hypertrophic skin that was very large (44 cm in length, maximum circumference 50 cm) though painless and completely covering the penis, with only the penile orifice barely observable (). A normal scrotum and testes could be identified after lifting the mass (). The thickened skin had no ulceration or infection, though loss of sensation for urination and erection were noted. Magnetic resonance imaging of the genital area showed an intact penis covered with edematous soft tissue. Most laboratory findings including filarial worm egg inspection were within normal limits, though urinalysis confirmed pyuria. Based on these findings, we diagnosed secondary giant penile lymphedema due to long-term chronic penile strangulation from use of a rubber band.\nSurgical treatment was performed to improve quality of life (QoL) and cosmetic issues. After placing a Foley urethral catheter, surgical excision of the subcutaneous hypertrophic tissues was made with carful haemostasis to expose the corpus cavernosa, that was found to be not deformed, while skin near the shaft of the penis was also found to be normal and used to cover and preserve the penis (). Finally, we removed the infiltrated tissue (4.8 kg, 30 × 21 × 10 cm) and penile skin reconstruction was performed using absorbable sutures ().\nA histopathological examination revealed that most of the excised mass consisted of proliferated collagen fibers, as well as enlarged lymphatic and capillary vessels with lymphocyte infiltration. Two months after surgery, wound healing along with acceptable cosmetic results and improvement of QoL was noted, though the patient required continuous use of a Foley urethral catheter because of loss of sensation to urinate.
An 88-year-old female presented to the emergency department with abdominal pain and vomiting. She was mildly tender in her right upper quadrant but was focally more tender in her right lower quadrant. Her blood results showed an elevated white cell count of 12.9 × 109 l–1 and a neutrophil count of 10.4 × 109 l–1. Furthermore her C-reactive protein level was 34 mg/L. An abdominal ultrasound showed multiple calculi within the gallbladder. Gallbladder wall thickness was not assessed. A non-contrast CT scan, due to the patients impaired renal function, was then ordered as the patient’s physical exam findings were not in keeping with a diagnosis of cholecystitis.\nThe CT showed abnormal dilatation of the proximal small bowel with a possible transition point in the left iliac fossa. Pneumobilia was also noted due to prominence of the hepatic biliary tree (). No evidence of a ductal calculus was observed. The CT scan was reported as showing features of a bowel obstruction with a transition point in the left iliac fossa. The pneumobilia was reported as being possibly an indicator of previous sphincterotomy. There was no evidence of appendicitis or another inflammatory process in the right iliac fossa.\nThe following day, while proceeding with conservative management, the treating team requested a small bowel follow through to investigate the obstruction. After further review and discussion with the treating team it was revealed that there was no history of an endoscopic retrograde cholangio-pancreatography or cholecystectomy. An addendum was added to the CT report, suggesting that the appearances were indicative of a gallstone ileus from a choledochoduodenal fistula () with a non-calcified gallstone possibly being present at the transition point in the left iliac fossa ().\nThe patient underwent a laparotomy and a gallstone was found obstructing the distal jejunum. A 41 mm gallstone was removed by vertical enterotomy, later confirmed on histological diagnosis. The patient had an uneventful recovery in hospital and was discharged a few days later to a regional hospital for rehabilitation. A cholecystectomy or fistula closure was not performed and she was not referred for further surgical follow-up on discharge, likely due to the patient’s advanced age and medical comorbidities.
Mr. K is 35-year-old married bangle seller. He was brought to the psychiatry outpatient department with a history of sudden onset of amnesia for the recent past with an alleged new identity for himself. Apparently well till a day before the presentation, in the morning hours he complained headache and slept for some time. After awakening, he started behaving in odd manner to the family members. He refused to accept the identity of his wife and other family members. He declared another name and residential address in Hyderabad for himself which is far away from his residence. He was stopped forcibly by the family as he was trying to travel to Hyderabad. When interviewed in the outpatient clinic he was conscious, alert, and well oriented. During the initial and repeated interviews, which were carried out over 5 days he remained firm on his new identity. He refused to accept that accompanying members were his relatives. He was telling that the accompanying lady and other persons were trying to act as his relatives. When he was shown the family pictures carrying his photo taken in the past, he said that they were of a similar person and not his own. When he was asked about how he reached the home where he was living, he remained puzzled and answered that he would like to know that. He was saying that his residence lies in Hyderabad, he was married and having two daughters. When he was asked to provide the address of his home in Hyderabad and his phone number he said that he forgot and he would like to know the address. Further, he stated that once if he is taken to the old city of Hyderabad he would recognize his residence. His family members have provided the history that his last visit to Hyderabad was many years back and the address and the names he was telling were related to his bangles business. As part of his business, he buys bangles in old city Hyderabad and sells them in his town. He stated few names as his brother in laws. Family members have provided the information that those names were of personnel who were in business. He was stopped by his family members forcibly from traveling as they have a similar experience in the past. Family members provided the history of three incidence of altered behavior in a span of 4 years preceding the presentation.\nIn the first instance about 4 years back there was a history of sudden onset of one episode of behavioral disturbances characterized by unresponsiveness to the external stimuli. This was preceded by financial stress in his business. He was observed to be withdrawn and brooding for few days prior to this. He remained stuporos for 3 days during which he was hospitalized in a nearby tertiary care hospital, and it was said that there was no apparent aetiology for his stupor. He was treated symptomatically and on the 3rd day suddenly he started talking normally as if nothing has happened. Family members did not bother much to evaluate this as they felt he was doing fine whatever the reason was.\nIn the second one, which was 2 years prior to the current episode he has traveled to Mumbai without any intimation to his family. His family members tried to locate him as he suddenly disappeared from home. About 2 days later the family members were called by him after his reaching to Mumbai which is about 1000 km from his residence. He called them to tell that he was not sure why he was in Mumbai and he could not recollect how he traveled to reach Mumbai. However, he reported a vague recollection that he traveled to Mumbai in train without any ticket. Soon the family members brought him back from Mumbai. This was preceded by financial hurdles during his house construction. During this episode also he was observed to be withdrawn and dull for 2 days prior to his travel.\nThe third episode was 2 months prior to the current episode. He has called family members saying that he was in a train to Chennai and he did not know how and why he was on that train. In this incidence, he himself came back to home. Wife has reported a similar financial problem in his family 2 days prior to this episode. There was no history of any substance intoxication or usage in the dependence pattern. There was no history of seizure disorder in the patient and his family. There was no significant head trauma history. There was no history to suggest any first rank symptoms or mood syndrome. Premorbidly, he was described as having anxious avoidant traits.\nHis physical examination including neurological examination did not reveal any significant findings. His routine blood investigations, computed tomography of the brain, and electroencephalogram were normal. He was diagnosed as having dissociative fugue and tablet lorazepam 1 mg twice a day was prescribed on an outpatient basis with specific instructions to the family to prevent any travel. On the 5th day suddenly he regained his memory saying that he could not recollect what happened in the 5 days. However, he recollected that he met the therapist. He revealed that he was under intense pressure to repay his debts related to his business.
A 47 year-old man was admitted to our emergency department after a motorcycle accident with his right hip in a fixed position of 90° flexion, 90° abduction and externally rotation. The patient had a history of hitting an electric pole with his right thigh with abduction force during collision. The patient complained of severe pain during movement of his right hip joint. His vital signs were stable. There were no neurovascular deficits in the injured limb. An anteroposterior radiograph revealed an inferior, obturator-type dislocation of the right hip joint (). Computed tomography (CT) scan was performed before any manipulation to evaluate any associated injury. There was no additional damage to the femoral head, acetabulum or trochanteric area (). After appropriate consultations, manipulation under anesthesia was taken.\nGeneral anesthesia was administered with patient in supine position. The dislocation was reduced as per the recommended techniques. We applied continuous traction in line with femur with gentle flexion and lateral push was given on the inner thigh with the pelvis stabilized by an assistant. However, this was unsuccessful. The hip was then internal rotated and adducted. Forceful resistance was abruptly reduced with audible "clunk" and we checked stability and range of motion of the hip. Reduction of the hip joint was confirmed under fluoroscopy and skin traction was applied for maintenance. Post-reduction CT was taken in order to confirm the congruent reduction. Although the post-reduction X-ray had shown minimal contour change of the greater trochanter (), post-reduction CT scan revealed the fracture of the greater trochanter (). The authors considered the possibility of further displacement of the greater trochanter, because the fracture gap was more than 2 mm on CT image. The fracture of the trochanter was then fixed through the incision under fluoroscopy control with cannulated screws and washers (). The patient was discharged with advice for strict bed rest for three weeks, followed by crutch walking for next three weeks. At the final follow-up after two years from trauma, the patient had a pain-free hip joint with active full range of motion ().
A 43 year old man with one month history of generalized seizure, and upward gaze and recent mild headache, and a past medical history of grade II glioma of 7 years ago, has come to the hospital with an evidences of a left temporal tumoral lesion, with mild enhancement and the size of 44 × 64 × 42 mm () in his routine subsequent brain MRI and. For more details, 3DCSI MRS with color coded map has obtained which revealed remarkable cholin rise with respect to NAA, which prompted him to the plan of surgical debulking and intraoperative radiation therapy as the known case of recurrence glioma with a history of previous receiving a total of 5.5 Gy radiation therapy. At the time of admission he has seemed alert, oriented with the Karnofsky score of 80 and Barthel index of 90. Pre operation CBC, LFT and RFT were normal.\nAfter performing a left temporal lobectomy, we have successfully resected more than 98% of the tumoural cavity, by using the conventional frameless neuronavigation-guided microsurgical tumor resection, tumoural bed has prepared for the placement of radiation source. The size of resection cavity in 3D was 4 × 5 × 3.5 cm. The craniotomy margin that has surrounded the tumor was 7 × 8 cm. Frozen section has confirmed high grade glioma. After achieving the favorable homeostasis, right before subjecting the tumor bed into the focal irradiation, has potentially involved risk structures and the expected doses to the risk organs have calculated by the team of neurosurgeons and radiation oncologists by considering the cavity borders using the neuronavigation software, the cavity geometry. Potential risk structures have identified. The most suitable applicator size has considered 3.5 cm in this patient. Then any skin or bone that could receive direct or scattered irradiation has shielded using sterile cutton pieces. IORT has applied in a straight forward way in the surgical procedure using the 50 Kv INTRABEAM® system (Carl Zeiss Meditec AG, Germany) and 10.3 Gy of focal irradiation therapy has administered for 12 minutes (). Lateral side of the spherical probe had 0.5 cm distance to the resection bed, while for the posterior, anterior and medial sides it was zero. Optic Nerve with the distance of 8 mm and Optic Chiasma with 15 cm have considered as risk structures, and have paid attention to be safe with the calculation of probable expose of 3.7 Gy and 1.8 Gy, respectively. The intraoperative radiation therapy procedures have gone uneventful. After completion of the procedure, absolute hemostasis has maintained, duraplasty has performed with the pericranial patch. Cranioplasty has made with microplate and the wound has closed in layers. Post-operative MRI has shown gross total tumor removal ().\nThe patient has gradually improved 48 hours after procedure, with the GCS of 15. Follow up pattern started with the early 48 - 72 hours post-operative MRI () and neurocognitive tests, in which he has shown functionally independent with the Barthel’s score of 100 and Karnofsky score of 90. Post operation CBC, LFT and RFT have remained normal. Follow-up brain imaging () has only shown some areas of encephalomalacia. Four days after the procedure he has presented with sign of aphasia which improved and disappeared within the next 3 days. He has discharged a week after the procedure and has currently under gone our regular follow-ups every 3 months, based on the combination of imaging studies (MRI), clinical presentation (physical examination, KPS, current medication) and a neurological assessment using the late effects in normal tissues subjective, objective, management and analytic (LENT-SOMA) scales.
The patient was a 24-year-old pregnant woman (20 weeks of pregnancy) who suffered from auditory hallucinations. She had never been diagnosed with any impairment in intelligence or communication skills and had no adverse childhood experiences such as abuse. She had never been diagnosed with epilepsy or any other physical illness. She had been drinking small amounts of alcohol twice per week. She also had no history of smoking or using other drugs such as cannabis or stimulant drugs. Although she was naturally nervous, she had no history of psychiatric disorders. When she was 22 years old, she became pregnant for the first time. She was admitted to the obstetrics clinic in our hospital twice for severe hyperemesis gravidarum. Her family blamed the condition on her physical and mental fragility. After admission, she developed mild auditory hallucinations of strangers blaming her. However, she was able to distract herself and not worry about the existence of the auditory hallucinations by talking to others such as the nurses or other patients. After her first child was born, the auditory hallucinations disappeared spontaneously, and her mental condition remained stable.\nWhen the patient became pregnant again at the age of 24 years, she developed general malaise, insomnia, and vomiting during the early stage, similar to the first trimester of her first pregnancy. Starting at 15 weeks of pregnancy, she heard the doorbell when there was no one at the door and her cellphone ringtone even though it was switched off. From 20 weeks of pregnancy, she started to hear a voice criticizing her actions, even though there was no one nearby. Therefore, she visited the Department of Psychiatry in our hospital for psychiatric evaluation and treatment. She seemed to tire easily; however, her communication was smooth and did not show evidence of thought disturbances. The malaise, insomnia, and vomiting associated with hyperemesis gravidarum were more severe than in her first pregnancy. However, obvious depressive mood and loss of the sensation of pleasure were not observed. She said that the auditory hallucinations were clearer and longer than during her first pregnancy. She would suddenly hear a stranger's voice criticizing her several times a day, as well as voices of performers speaking accusatory words about her while she was watching television. The auditory hallucinations had become a distraction even when talking to someone. There was no obvious disturbance of consciousness, and no obvious neurological findings such as loss of tendon reflexes, gait disturbance, or eye movement disturbance were observed. There were no obvious abnormal findings in the blood test. Magnetic resonance imaging and electroencephalogram could not be performed because she was unable to rest for a long time due to hyperemesis gravidarum. At the first visit, she was prescribed zolpidem (5 mg/day) for her insomnia. The insomnia improved; however, moderate auditory hallucinations persisted. Then, we administered 4 mg/day of an atypical antipsychotic agent, perospirone, to treat the auditory hallucinations. Subsequently, the frequency of the auditory hallucinations decreased. When the dose of perospirone was increased to 12 mg/day, her auditory hallucinations disappeared. In the final month of pregnancy (36 weeks), the patient's auditory hallucinations temporarily relapsed due to the increase in stress and anxiety. Then, when we increased the dose of perospirone to 16 mg/day, her hallucinations disappeared again. However, her hyperemesis gravidarum persisted. The patient safely gave birth at 39 weeks. After childbirth, we discontinued perospirone treatment. Her mental condition was stable, and she had a normal daily life without relapse of auditory hallucinations.
History: The patient was a 32 years old female from Addis Ababa, Ethiopia. She presented to one of the private clinics at Addis Ababa on January 18, 2017, with weakness on the right lower limb and numbness and tingling on the left lower limb of three days' duration. She had also associated urinary urge incontinence with a band like feeling on the right trunk below the breast fold. She had lost her right eye vision progressively seven months before the first visit. On the first visit, she was treated with five days of methylprednisolone intravenously and followed by oral prednisolone for 2 months and showed significant improvement. However, after six months of the first visit, the patient presented with another attack with weakness on the right lower limb of five days' duration with urinary urge incontinence. She was married and a mother of three children. Regarding past medical history, she was hypertensive on treatment with good control in the last 2 years. Otherwise, there was no personal or family history of diabetes or cardiac illness. No history of cough, night sweat, weight loss, or skin rash was recorded. She had no history of exposure to toxins and travel history out of Ethiopia..\nPhysical examination: On physical examination, she was noted to have a visual loss of the right eye with pale optic disc but no background change noted on fundoscopic examination. The right eye visual acuity was only light perception; left eye visual acuity was 6/6. Power on lower limbs was 3/5(right) and 4/5(left), reflex ¾ both on ankle and knee, plantar responses were up going bilaterally. Vibration and position sense decreased on the right body below the thoracic sixth vertebral spine and decreased pain, touch, and temperature sense on the left body below the thoracic sixth vertebral spine. The rest of the systemic and neurologic examination was unremarkable.\nAuxiliary examinations: Her complete blood count, erythrocyte sedimentation rate, liver function test, renal function test, serum electrolytes, and plasma glucose levels were normal. Serum VDRL was non-reactive and serology for retroviral infections was also negative. Serum Antinuclear antibody (ANA) was also negative. Serum AQP4-IgG titer (using AQP4-IgG serologic assay techniques) was also determined on the second attack and reviled 1:200+++ (normal value <1:10). Chest X-ray and abdominopelvic ultrasound results were also normal. Her brain MRI finding was normal except for the presence of the right side atrophied optic nerve (). Thoracic MRI () revealed hyperintense ill-defined lesions from the thoracic four (T4) to the thoracic seven (T7) spinal cords. However, the limitation of this case report was, contrast (gadolinium) was not administered during MRI study. Visual Evoked Potential (VEP) of the patient also revealed left side moderate demyelinating optic nerve neurophysiologic impairment (Optic neuritis, P100 latency of 128 milliseconds) and absent response on the right eye suggesting severe optic nerve neurophysiologic impairment (). However, a cerebrospinal fluid analysis was not done considering that the case was a patient with classic NMOSD.
A 20-year-old systemically healthy female patient was referred to our clinic. The clinical evaluation of the patient revealed low (<20%) full-mouth plaque score and full-mouth bleeding scores []. There were no periodontally diseased sites other than the lower-left posterior region of the patient. Clinical and radiological evaluation showed mesial tipping and vertical bone loss caused by impaction of the lower-left second molar. Blood on probing and excessive plaque accumulation were present on distal sites of the first molar and mesial sites of the second molar teeth. The initial probing depth was 8 mm on the mesial surface of the second molar. The distal surface of a neighboring first molar also had a probing depth of 8 mm (Figures –). The malposition of the lower-left second molar was suspected to be the reason of the localized bony defect.\nAt first visit, nonsurgical periodontal therapy was performed, and the patient was given oral hygiene instructions to maintain plaque control at the defect site. Three weeks after the initial therapy, a removable appliance with a T-loop was designed to upright the lower-left second molar tooth (Figures -). The uprighting force was transferred to the teeth by a bonded button on the occlusal surface of the lower second molar tooth (Figures –).\nThe patient was scheduled for follow-up every two weeks to maintain the patient's oral hygiene and the continuous orthodontic force by activating the uprighting spring. Because of the compromised periodontal status of the second molar, approximately 150 grams was applied to the button by activating the spring about 1 mm distally in each visit. The open end of the loop in the removable appliance caused the distal tipping of the tooth as expected. The appliance was used for three months. As the patient's growth and development period had not been completed, the extraction of the third molar was delayed because of the possibility of an acceptable eruption. After the finalization of orthodontic treatment, reevaluation of the third molar showed that a successful eruption was not possible. Thus, the patient's third molar was extracted after orthodontic treatment.\nAt the end of the orthodontic treatment, the patient showed a significant improvement in the periodontal tissues (Figures -). Gingival tissues around first and second molar teeth healed successfully, showing no signs of inflammation and bleeding on probing. The tooth was positioned in a physiological location, and the probing depth measurements were reduced from 8 mm to 3 mm. Also the remodeling of the bone was achieved by a distal tipping movement (Figures -).
A 40-year-old previously healthy woman underwent a curative resection of the medical compartment of the right thigh for a malignant spindle cell tumour. Due to clear resection margins and absence of any metastatic deposits in imaging, she was not given any adjuvant therapy. She was followed up for 5 years, and she was otherwise well and asymptomatic without any evidence of local recurrence.\nAfter a period of 5 years, she developed an acute onset generalized severe abdominal pain and collapsed. She did not have any external bleeding manifestations such as haematemesis or melaena. There was no history of abdominal trauma. She was immediately admitted to the emergency department, and she was found to be in class III haemorrhagic shock with a pulse rate of 125 beats per minute and a blood pressure of 90/60 mmHg. Her abdomen was distended and diffusely tender. She was immediately resuscitated with intravenous crystalloid boluses and blood transfusion. Her serum beta-Human Chorionic Gonadotropin (beta-HCG) was negative, excluding a ruptured ectopic pregnancy. Her full blood count revealed a haemoglobin level of 5 g/dl, and a massive transfusion protocol was initiated. Her other basic investigations and clotting profile were within normal limits. Despite resuscitation, her vital parameters did not improve and her abdominal girth was gradually increasing. The abdominal ultrasound scan revealed moderate amount of free fluid with a mass around the proximal part of the pancreas. There was no evidence of abdominal aortic aneurysm, and other solid organs were normal. Hence, a decision was taken to proceed with an emergency exploratory laparotomy. A computed tomography was not done as the patient was haemodynamically unstable.\nA midline laparotomy was performed. There was 4.5 litres of haemoperitoneum, and a linear tear in the gastrocolic omentum with a haematoma in the lesser sac. Active bleeding was noted from a nidus which was situated posterior to the body of the pancreas, and it had ruptured through the inferior border of the pancreas. The rest of the pancreas and other abdominal organs looked normal. Bleeding was controlled by ligation and local application of haemostatic agents. A biopsy was taken for histopathological analysis. The pancreas and the rest of the organs were macroscopically normal.\nThe histological analysis revealed an infiltrating tumour composed of fascicles of spindle cells with oval, vesicular nuclei with the scanty eosinophilic cytoplasm. There were areas containing cells with moderate to severe nuclear pleomorphism and increased mitotic activity (13/10 high power field). The prominent storiform pattern was evident (). The focal haemangiopericytomatous vascular pattern was present. There was no tumour necrosis. Entrapped atrophic pancreatic acini were seen within the tumour. CD 99 and PS 3 markers showed moderate cytoplasmic positivity and nuclear positivity, respectively. SMA showed nonspecific positivity. CD 34, BCL 2, S100, EMA, CK7, CK19, and Desmin were negative. Therefore, the diagnosis was compatible with an undifferentiated spindle cell sarcoma involving the pancreas. Furthermore, the histological patterns were compatible with a metastatic deposit of the previously diagnosed spindle cell sarcoma.\nFollowing surgery, the patient was managed in the intensive care unit for 4 days, and thereafter, the patient recovered and was discharged on day 7. She was offered pancreatectomy with excision of the tumour but she refused surgery. She was referred to the oncologist and was commenced on adjuvant chemotherapy. She received a course of Ifosfamide and Doxorubicin. Contrast-enhanced computed tomography was performed before the commencement of chemotherapy which showed a 7 × 4.8 × 4.9 cm mass lesion with a thin wall containing hypodense material, attached to the inferior border of the pancreas (). There were no other metastatic lesions identified. She was given 6 months of chemotherapy and finally succumbed to the disease 18 months after the presentation.
A 7-year-old girl, student of second class, belonging to a high socioeconomic status family living in an urban locality was brought with complaints of academic decline, irritability, and abnormal behavior for the past 9 months. The child was born out of a nonconsanguineous marriage, is first in order, and was a wanted child. Maternal health during pregnancy was normal, but the period of labor was prolonged beyond 18 h, so a lower segment caesarean section was performed. There was no history of birth-related complications and the child's birth weight was 2.80 kg. The child attained developmental milestones as per age. The child had a temperament characterized by high activity levels, below average threshold of distractibility, average ability to sustain attention and persist, easy to warm up, adaptation to new situations, and regular bowel and bladder habits. She was enrolled in school at the age of 4 years and progressed well till 9 months back when a decline in her academic interest was observed by her class teacher. Deterioration of her handwriting skills and avoidance of group activities in school were observed. Similarly, at home persistent irritable behavior was seen and her play activities with her siblings reduced. However, her biofunctions were normal during this period.\nOne month prior to visiting us, she started insisting on wearing the same dress. She wore the same colored or at times the same dress which she would not take off even at bed or bath time. In addition, a change in her mood from largely irritable to cheerful was noted. Her activity levels were increased and it would be difficult to make her sit quietly in class. Her speech output was more than her usual self and she talked incessantly. Her sleep duration also decreased and she started getting up 3–4 h earlier than her usual routine. In view of these symptoms, her family made first contact with us. Her physical examination was normal and mental state examination revealed her to be cheerful, overactive, and difficult to interrupt. She sang and danced during the interview. We diagnosed her with acute mania on the basis of clinical evaluation and assessment on MINI Kid 6.0.[] The details of her physical examination are depicted in . She was initially treated with olanzapine 5 mg/day which was later on increased to 10 mg/day. However, no response was observed with it in the next 2 weeks, so it was cross tapered with sodium valproate which was built up to 400 mg/day. She improved by nearly 50%, but her mood still remained cheerful/irritable. She did not resume her school and was brought irregularly for the follow-up. Within the next 2 months, she also started muttering to herself and made certain abnormal gestures. She often feared staying alone, or while going to bed insisted the lights to be kept on and ask someone to accompany her in the toilet unlike her previous self. When asked, she reported seeing a lady in white clothes, with no other details. She stopped asking for food on her own and remained lost in her fantasy world. However, her interest in dressing and appreciating herself in mirror persisted. Her mood during this period was mostly labile and often changed from cheerful to sad or irritable. As per the family, the medications were continued as advised. So in view of the emerging picture, the diagnosis was revised to schizo-affective disorder, and in addition to hike in dose of sodium valproate to 500 mg/day, risperidone 2 mg/day was also added. However, even after 8 weeks of treatment with this combination with hike of risperidone to 4 mg/day, there was no relief. The child is still symptomatic, does not go to school, and has significant dysfunction. Psychosocial intervention in the form of psychoeducation, activity scheduling for the child, and occupational therapy has been instituted in addition to the existing treatment regimen, but results are yet to be seen.
A 25-years-old woman with an autoimmune hypothyroidism since 2 years, treated with levothyroxine 200 mcg daily, was admitted with fever (37.9 oC). She had neck pain and a rapidly growing goiter, mainly affecting the left lobe. Inflammatory parameters were elevated (ESR 140 mm/h, CRP 119 mg/L, normal <20 and <5, respectively). Based on this clinical presentation together with an initial FNA that had showed a purulent material with granulocytes and fibrin, acute thyroiditis was suspected and antibiotics were initiated. Two days later the diagnosis was reevaluated to subacute thyroiditis because of the clinical appearance and a new FNA showing a mixture of histiocytes, lymphocytes, and granulocytes. Prednisolone 40 mg once daily was initiated, and the dose were tapered to 7.5 mg/day, as the volume of the goiter decreased with clinical improvement. After 1 month a clinical deterioration was noted. A third FNA was performed from the right lobe showing granulocytes, eosinophils, and a large number of partially destroyed muscle cells that was judged to be a late phase of a subacute thyroiditis, and the prednisolone dose was increased to 30 mg/day. During the next 4 years, the patient went through different periods of activation of the disease, which responded, well but transitory to glucocorticoid therapy. The FNAs showed every time an inflammatory process with a mixture of granulocytes, histiocytes, plasma cells and fibroblasts and fragments of muscle cells. A biopsy of a lymph node from the neck showed reactive changes with an increase of plasma cells.\nAfter 4 years of evolution total thyroidectomy including extirpation of some lymph nodes was performed, and RT with extension outside the thyroid gland was the final pathological diagnosis.\nEight years after surgery, and 12 years after initial presentation, the patient still had exacerbations of the general and local symptoms that always were treated with prednisolone for different periods of time. Finally a biopsy was taken from the right maxillary sinus secondarily to symptoms related to the sinus. The pathological diagnosis was IgG4 sclerosing disease. No serum increase of IgG4 was ever detected. Azathioprine was initiated but was stopped secondary to an increase of the liver enzymes. During the following 10 years she was treated with mycophenolate mofetil combined with Rituximab because of lack of effect of mycophenolate mofetil alone, and at last follow-up 22 years after the initial presentation she still has periods of inflammation.
We report a case of a 32-year-old male, who sustained a severe crush trauma resulting in open fractures of right tibia and acetabulum, multiple rib fractures, and closed fracture of both forearms. The patient was firstly treated in another hospital: fractures were reduced, and right tibia was covered with a free left latissimus dorsi muscular flap. After 5 days a complete necrosis of the flap occurred, the flap was removed, and a topical negative pressure therapy was applied.\nThe patient was then referred to our department. He presented a wound of 30 × 12 cm on the anterior surface of right leg with exposed fracture of tibia and ischemic sufferance of the distal bone segment.\nWe agreed that the best option was a debridement of all nonvascularised tissues and coverage with a free muscular flap, and we planned a reconstruction with the right latissimus dorsi muscular flap.\nThe patient was studied preoperatively with an arteriography of the right leg, that documented an obstruction of the anterior tibial artery in its middle third and was distally perfused by a branch of the interosseus artery; the posterior tibial artery was open, and the dorsalis pedis artery was frail and thin.\nSurgery consisted in a removal of cortical bone from the fracture margins and distal fibular osteotomy and a soft tissues debridement. Posterior tibial vessels were isolated through a posterior leg access. The right latissimus dorsi muscular flap was harvested, transferred to cover the wound and vascularized by end-to-end venous and end-to-side arterial anastomosis between thoracodorsal and posterior tibial vessels. The muscle was covered by a mesh split thickness skin graft.\nAfter 24 hours we observed a flap congestion, so we took back the patient to the operating room and explored the anastomosis; the venous anastomosis appeared thrombosed. The thrombus was removed and both arterial and venous anastomosis were reperformed. After surgery the flap appeared viable.\nAfter 48 hours a flap congestion was newly observed and anastomosis exploration revealed a thrombosis of both artery and vein. We decided for the removal of latissimus dorsi muscle flap and coverage with a cross-leg fasciocutaneous flap from contralateral leg.\nThe flap was designed on the posteromedial surface of the left leg, and the dimension was 12 × 10 cm.\nThe incision was made on the posterior, proximal, and distal side of the flap. The dissection proceeded in a subfascial plane from the posterior side to the medial one. The flap was left attached on the anterior edge, and the posterior one was sutured to the medial aspect of the bone exposure on the right leg. The other two sides were partially sutured to the wound borders (). The remaining wound on the right leg, the under free surface of the flap, and the donor site were covered with a nonadhesive dressing. The patient was positioned using a custom-made valve for the two legs together. Moreover he presented an external fixator applied before the first surgery in another hospital It provides the necessary strength for immobilization and overhead suspension aiding greatly in wound care, as well as for general ease of patient mobility and positioning. At the same time a better immobilization is very helpful to avoid partial or total flap necrosis.\nThe dressing was changed every two days, and antibiotic and antithrombotic prophylaxis therapy were administered. The postoperative X-ray of the leg demonstrated a good alignment of the fracture.\nAfter one month the flap was separated from the donor site. The donor area and the remaining wound on the right leg were covered with a skin graft (Figures and ). No complications were observed in the postoperative period and the patient was discharged after 6 days.\nAt the 6-month followup we assessed an overall satisfactory result. No neurological problems were reported, except for dysesthesia of the area surgically treated. The patient can walk, and the X-ray demonstrates a good recovery of the tibia fractures. The cosmetic result is acceptable ().
The patient was a 48 year-old male academic public health physician who had just returned to the Middle East after spending a week with his family in Saskatoon in Canada. His journey to the UAE was via Calgary and Heathrow airport in London, UK. He started feeling lethargic, and developed a sore throat, with cough and high fever for around 10 hours since the night of his arrival in Dubai, UAE. This led him to consult the on-duty infectious disease consultant at the Emergency Department of a local hospital at around 8:00 am the following day.\nThe consultation included a discussion of any possible exposure to H1N1 since Canada was recognized then as experiencing a large number of cases of the infection. A combined influenza A&B antigen screen on a nasopharyngeal swab was positive, and an additional swab and a blood sample were then sent for further confirmatory testing. He was prescribed Oseltamivir 75 mg orally twice daily for five days, azithromycin 500 mg daily and paracetamol 500 mg three times daily for three days, and advised to remain at home until the confirmatory test results were available.\nBy the next morning, the patient's fever and sore throat had subsided and he was feeling better. Despite the very low, but nevertheless real risk of having 'swine flu', the patient had to make some important difficult decisions regarding his state of health and his work deadlines. His work place was a university campus and as he had no lectures that day, he had no need to be in contact with any students. All scheduled appointments on his calendar for the day were cancelled, but he decided to proceed with a ten-minute scheduled presentation to six of his peers regarding a large research grant proposal. A mask was not worn during the presentation, and he returned home immediately after the event. The patient was alone at home but one of his relatives came to visit him unannounced, accompanied by his wife and a ten year old child from a neighboring town. They stayed at his home for that night, as the distance for return travel was considerable.\nOn the next day the patient received a call from the Health Authority confirming Influenza A (H1N1) infection and he was therefore in the unenviable although historical position of being the first reported case of H1N1 infection in the UAE. The patient was admitted to hospital with airborne and contact isolation, where he completed the rest of the maximum recommended 10 days quarantine period. The visiting couple and child also had to stay at the patient's home for 10 days of quarantine and all also received prophylactic medicine (Oseltamivir). No lab tests were advised.\nAs a public health physician, the index case had considered the H1N1 situation before commencing his travels to Canada. At that time (May 8 2009), the World Health Organization (WHO) did not recommend restricting travel, although some individual national authorities were advising against non-essential travel. The advice on the various websites seemed very pragmatic: observe basic hygiene, hand-washing and cough etiquette; do not travel when ill and seek medical advice if you become ill after your return. The patient's route to Canada took him through London (34 cases reported in the UK at that time) and Toronto (15 cases in Ontario) to Saskatoon (2 cases). By the time he was due to return to the UAE from Saskatoon via Calgary, the number of cases in Canada had increased from 242 to 496 with 19 in Saskatchewan and 67 in Alberta. During his stay in Saskatoon he did not recall meeting anyone with respiratory symptoms and he was quite well on his journey back to Dubai. He was therefore not certain where and from whom he caught the infection.\nThis case raised several issues related to workplace and general public health. Measures taken by the UAE government to prevent an influenza epidemic include the installation of thermal scanners at Dubai, Sharjah and Abu Dhabi airports (three major international airports in the United Arab Emirates). The individual was afebrile and symptom-free on arrival at the airport, and so was not detained for further enquiry. The thermal scanners will detect individuals with fever from whatever cause, but will not necessarily detect those with early H1N1 infection, especially if they are afebrile [].\nEffective and timely communication is essential to allay unwarranted concerns from the public and at the workplace. Queries from the media were channeled to a senior member of the administration from the office of the Dean - to ensure consistency in the information provided. He was briefed by public health physicians, occupational health physicians and hospital clinicians dealing directly with the case. A central news release was provided to staff and students on H1N1 reiterating the importance of hygiene in regards to limitation of transmission. The workplace was a university campus. This case did not have any lectures or meetings with students. Contact with a few coworkers was transient (not more than 15 minutes in the same area). These contacts were counselled on the low likelihood of acquiring the infection. They were informed about seeking medical advice if they had any other reasons for concern or if they developed H1N1 symptoms. Doctors, nurses and ancillary healthcare workers looking after the case while in hospital were briefed on hygiene and infection control procedures. N-95 masks, gloves and gowns were provided to health-care staff.\nThe Health department took prompt action. Family members with close contact were quarantined at home. They were given a prophylactic course of Oseltamivir. Adequate supplies of food and provisions and maintenance of phone communication was confirmed. The public health department dealt with general queries from the public. Official release of information and contact with the WHO was through the federal Ministry of Health. The airline that transported the case from Canada to the UAE sought to contact passengers in the rows adjacent to the passenger's allocated seat. None of those who were traced developed any flu-like illness within the incubation period following the timing of the flight.
A 27-year-old lady presented to our OPD with a 6-month history of recurrent ulcers on extremities and upper back, the cause of which she could not explain. Her social and medical history revealed that her in-laws abandoned her after the death of her husband in a road traffic accident. On clinical examination, we found many symmetrical superficial excoriations on anterior aspect of both upper and lower extremities, which she claimed appeared overnight, interestingly many, as she narrated, appeared on her way to hospital. Almost all the lesions were similar in appearance (about 0.5 cm wide and 2.5 cm long), dimension and were positioned in a linear manner and all present only in accessible parts of body []. Though few were fresh erosions with little serous oozing, many were old marks indicating past healed-up lesions. Barring one or two over upper back and left flank [], almost all the lesions were on anterior aspect of lower limb and extensor aspect of forearm. DA was suspected and the patient was hospitalized for further evaluation and treatment.\nPhysical examination and laboratory work-up were negative. Fresh symmetrical and identical skin lesions started appearing while she was in hospital. Interestingly, patient could tell the exact time when a particular lesion appeared. Only occlusion dressing was advised with systemic antibiotics and lesions healed in 2 to 3 days. After repeated interviews, it became obvious that she was depressed following death of her husband and was in conflict with her parents and confessed to having produced the lesions by own nail. A clinical diagnosis of DA was made. No fresh lesions appeared during two days of observation. She was referred to a psychiatrist and few sessions of psychotherapy produced marked improvement. She was discharged and was advised by the psychiatrist for further sessions. At follow-up visit two weeks later, the ulcers had recurred and were still present at two subsequent visits. She refused further admission and did not return for follow-up visits.
Case 2 was a 51-year-old man who suffered from end-stage liver disease caused by Budd-Chiari syndrome, diagnosed in 2005. He had 6 cases of esophageal variceal bleeding treated with rubber band ligation, but had no history of ascites drainage or SBP. He had a subtotal gastrectomy and Billoth-II anastomosis due to adenocarcinoma detected from the varix ligation specimen in 2012. He underwent living donor LT due to recurrent variceal bleeding with a left lobe graft from his daughter on August, 2014. During operation, fibrotic change of the peritoneum was not observed. His maintenance immunosuppressive agents were FK, MPA, and prednisone. His early posttransplant course was complicated by portal vein stenosis and a large amount of ascites that was treated with angiographic portal vein balloon dilatation and percutaneous drainage of the ascites. Klebsiella pneumoniae was cultured on the drained ascites and he was treated with ertapenem for 2 weeks. He was discharged 4 weeks after transplant without any complications. Three months after transplant, he was readmitted with nausea, vomiting, and abdominal pain without rebound tenderness. His upper gastrointestinal series revealed a distended stomach and jejunum with delayed passage. Abdominal CT revealed diffuse thickening of the visceral peritoneum of the small bowel loop with suspicious adhesions and decreased mural enhancement of the terminal ileum (). Tamoxifen 20 mg was administered twice a day and FK was replaced with mTOR inhibitor (sirolimus, target trough level: 5–10 ng/mL). Prednisolone was restarted at 4 mg twice a day. Two weeks after medical treatment, he complained of aggravated abdominal pain and a subjective fever. Follow-up CT revealed terminal ileum perforation, which was suspected due to decreased mural perfusion on the prior CT. He was taken back to the operating room and received a terminal ileum resection and anastomosis. Dissection of the fibrotic membrane and adhesiolysis were also performed (). He was able to start oral feeding 6 days after the operation and was discharged 4 weeks after the operation. Tamoxifen was maintained for 3 months, and he was continued on sirolimus and prednisone. He has maintained an oral diet without nausea or vomiting for 20 months after operation.
A 42-year-old lady, gravida 2 para 0, presented to our emergency department with complaints of vaginal bleeding and lower abdominal pain for 1 day, with a positive urine pregnancy test. She had no past medical history, and surgical history comprised of one previous termination of pregnancy. She was unable to recall her last menstrual period but reported taking abortion pills 2 weeks ago. Physical examination revealed an enlarged uterus of 12 weeks size, with mild tenderness on palpation of the suprapubic region but no rebound or guarding. Speculum examination revealed a closed cervix with minimal vaginal bleeding.\nBedside trans-vaginal ultrasound revealed a pregnancy with a crown rump length of 47 mm corresponding to 11.4 weeks, with no fetal heartbeat detected. She was provisionally diagnosed with a missed miscarriage and underwent a second scan for confirmation. The repeat scan by a different operator was concordant with the initial scan (). The patient was counselled on the diagnosis accordingly and consented to a surgical evacuation of the uterus. Laboratory investigations revealed a serum beta human chorionic gonadotropin (bhCG) level of 45,195 IU/L and hemoglobin level of 6.5 g/dL, prompting initiation of a packed cell transfusion. At that time, her anemia was attributed to vaginal loss from the miscarriage and physiological anemia from the pregnancy. Her vital signs remained stable.\nTaking into consideration the history, clinical examination and ultrasound findings, our working diagnosis was that of a missed miscarriage. Another possible differential was an ectopic pregnancy, more commonly tubal in origin. However, there was a low clinical suspicion for this condition in view of the fact that the fetus was entirely visualized in its gestational sac as an intrauterine pregnancy, confirmed on two ultrasounds done by different operators. Clinical examination of the patient also revealed a soft abdomen with only mild tenderness in the suprapubic region and no overt signs of peritonism.\nThe patient underwent evacuation of the uterus under general anesthesia as planned. A repeat vaginal examination was done intra-operatively and there was an unusual finding of a mass felt in the Pouch of Douglas (POD). A Hegar dilator was used to gently dilate the cervical os, but it was only able to be advanced to a cavity length of 6 cm. Minimal products of conception were obtained. She was quickly reassessed with an intra-operative ultrasound. This time, the ultrasound clearly demonstrated that the Hegar dilator was in the uterine cavity, but it was not in continuity with the gestational sac and fetus (). This prompted revision of the diagnosis to an abdominal ectopic pregnancy and we proceeded to perform a diagnostic laparoscopy.\nUpon entering the abdominal cavity there was frank hemoperitoneum with adhesions, limiting access to and visualization of the pelvis. Therefore, the decision was made to convert the operation to an exploratory laparotomy. This revealed a large and inflamed left tubo-ovarian complex with inflamed omentum covering the uterine surface. The entire fetus and placenta had been expelled into the POD. Blood clots were evacuated and the origin of the ectopic was identified to be the fimbrial end of the left tube, which was actively bleeding. The uterus was otherwise normal. We performed adhesiolysis and left salpingectomy, and fetus and placenta were removed in its entirety (). The total estimated blood loss was 1.8 L.\nThe patient recovered uneventfully after surgery and was discharged on the second postoperative day. Histology showed products of conception within the lumen of the left fallopian tube in keeping with a ruptured tubal ectopic pregnancy. Final diagnosis was that of a secondary abdominal ectopic pregnancy, with implantation in the POD after tubal rupture. The patient was given an outpatient follow-up appointment. However, she defaulted on the appointment as she had made plans to return to her home country.
A 55-year-old man with history of pulmonic stenosis (postvalvuloplasty in 2004) and chronic thromboembolic pulmonary hypertension due to antiphospholipid antibody syndrome, presented with two months of progressive chest pain and exertional dyspnea. A CT scan of the chest was remarkable for massive dilatation of the proximal PA () but did not reveal evidence of acute pulmonary embolism. Our subsequent differential diagnosis included progressive pulmonic stenosis with right ventricular failure, progressive pulmonary hypertension with right ventricular failure, LMCA compression from massive PA dilatation, and left mainstem bronchus compression from PA dilatation. These potential diagnoses were evaluated through a series of diagnostic tests. First, a cardiac MRI revealed a bicuspid pulmonic valve with a peak gradient estimated at 18 mmHg and an impaired right ventricular ejection fraction (27%). Subsequently, a right heart catheterization revealed pulmonary arterial pressure of 93/38 (52), pulmonary capillary wedge pressure of 18 mmHg, right atrial pressure of 18, Qp:Qs = 1:1, and a gradient across the pulmonic valve of 15 mmHg without significant regurgitation. Next, as attention shifted to the potential mechanical complications caused by this massive PA dilatation, pulmonary function testing was performed but did not show obstructive lung disease as would be expected if left main bronchus narrowing contributed significantly to his symptoms. Last, to evaluate the LMCA, a CT angiogram of the coronary arteries was performed (). The CT angiogram showed effacement of the LMCA, but no compression or flow limitation. The most significant finding from these diagnostic tests was severe pulmonary hypertension with elevated pulmonary vascular resistance and without either a significant gradient across the pulmonic valve or physiologic evidence of significant mechanical compression of the surrounding structures. Therefore, we started the patient on bosentan (an endothelin receptor blocker) as his clot burden is distal and not amenable to thromboendarterectomy. Six weeks after the initiation of treatment, the patient had resolution of chest pain, improvement in functional class, a decrease in his BNP to 43 pg/ml, and a decrease in his right ventricular diastolic diameter on transthoracic echocardiogram from 6.2 cm to 5.2 cm. These improvements have sustained over the past year.
A 73-year old woman was referred to our surgical department from a breast out-patient clinic, with a 1-year history of observation for subareolar tumor of right breast. Her medical history included arterial hypertension and osteoporosis. On clinical examination the tumor was palpable, approximately 2 cm in diameter, movable in relation to surrounding breast tissue and chest wall, axillary lymph nodes were not involved. Control mammography confirmed subareolar localization of the tumor, ranging from 11 to 15 mm in diameter. Biopsy revealed infiltrating ductal carcinoma, ER-75%, PR (-). A modified right radical mastectomy with level I and II lymph node dissection was performed without any complications. The wound was drained with two 16Fr, low pressure suction, silicone drains. One of the drains was placed in the axilla, a second was placed along the wound between skin flaps and greater pectoral muscle. Additionally external compressive dressing was placed on the wound site. Drainage volume declined from 220 ml on first post operative day to 50 ml on the sixth postoperative day and drainage was removed, total drained volume was 780 ml. Postoperative shoulder movements were not restricted and rehabilitation exercises started on the third postoperative day. The patient was discharged on the 7-th postoperative day. On control visit, 4 days after discharge, for skin sutures removal, patient showed with a palpable and symptomatic seroma in the wound site. She has undergone puncture with evacuation of 240 ml of clear serous fluid. Since then punctures were performed 2–3 times a week. Indication for puncture was symptomatic seroma causing pain and impairment of shoulder movement. Volume of clear serous fluid evacuated from wound site ranged from 120 ml to 260 ml per puncture. Additionally compressive dressings with elastic bandage were applied on the wound site. During the time of postoperative chemotherapy, the patient developed deep veins thrombosis of the lower extremities. Treatment with low molecular weight heparin (enoxaparin), increased the volume of evacuated fluid. On control USG there was no impairment of flow in axillary vessels, but USG of the wound showed spindle like container with thickened wall. Because volume of drainage didn't decline, after having finished chemotheraphy patient was qualified for operation as soon as symptoms of deep vein thrombosis diminished. Due to the large volume of drainage from the wound, we took into consideration not only inflammatory exudation as a cause, but also lymphatic origin of effusion. In this case surgical excision with closure of dead space may cause arm swelling in postoperative course. Because the patient didn't agree for lymphangiography, we decided to inject 2 ml of Patent Blue V dye subcutaneously in the right arm and fore arm 30 min before operation, in order to stain upper extremity lymph vessels confluence. The skin incision was done in the line of previous wound. The spindle-like serous pouch was located in the subcutaneous tissue on the greater pectoral muscle, with its lateral end forming vessel like structure and penetrating deep in to posterior site of axilla. (figure ). After opening the reservoir, since there was no sign of stain in the lumen and containing fluid was clear, we assumed that there is only a little risk of arm swelling and lymph edema after resection of reservoir and closing of supporting vessel. After resection of reservoir, we visualized the route of the vessel supplying the reservoir by injection of Patent Blue V dye in to its lumen. The stained vessel branched on the lateral side of trunk and penetrated deep into intercostals of chest. All branches of stained vessel were closed separately. The wound was closed without suturing the dead space between the skin and greater pectoral muscle. The low pressure suction drain 16Fr was placed into axilla. Volume of drainage declined from 150 ml on first postoperative day to 50 ml on the fourth postoperative day, and drainage was removed. The patient was discharged on sixth postoperative day. She required two more punctures of serous fluid of total volume 140 ml, collected in the wound site. On the postoperative histological examination the seroma wall appeared to be composed of fibrous tissue with marked eosinophilic hyaline degeneration of collagen and weak predominantly lymphocyte infiltration. No epithelium was present on the inner surface of the seroma capsule. Despite accurate examination of the seroma wall no evidence of connection to lymphatic vessels was found, either macro- or microscopically (figure .)
A 31-year-old male patient has a history of a multiple fracture involving the left femur shaft, the middle and distal segment of the left tibiofibula, as a result of a traffic accident in October 2008. Subsequently, the patient underwent an open reduction and internal fixation (ORIF), using titanium alloy plates and screws. Ten months after ORIF, an X-ray examination showed no displacement of the fracture site, and the bridging callus was visible on two standard views with partial obliteration of the fracture line. However, the patient did not undergo reoperation to remove the metal implants due to financial difficulties.\nIn August 2011, the patient complained of a 3-month history of a gradually enlarging left thigh mass. Physical examination revealed a 12 × 10 cm firm, tender and fixed mass in the anteromedial left thigh, with local superficial venous engorgement and skin temperature increment. Percussion pain in the axial direction of the left lower limb was absent. The inguinal lymph nodes were not palpable. Routine chest radiograph and abdominal ultrasound examination showed no abnormalities. Other routine tests, including plasma biochemistry, liver function tests and urinalysis, were normal.\nThe X-ray examination demonstrated an ill-defined osteolytic lesion with disappearance of the medial cortical bone in the middle of the left femur shaft, as well as an ambiguous image of a soft tissue mass. Computed tomography (CT) images clearly showed a partial bone defect approximately 9.7 cm in length on the medial border of the left femur shaft. A 9.7 × 11 × 19 cm irregularly shaped soft tissue mass was observed around the left femur, with the surrounding tissues compressed and shifted significantly (Figure \n). CT findings also showed that the soft tissue mass was heterogeneous in density with CT value of 36 HU, in which small pieces of slightly high-density lesions were found. These imaging findings suggested that the mass was neoplastic.\nTissue biopsy was performed, and histopathological examination reported that the tumor was predominantly composed of a large number of diffuse fusiform cells and myxoid matrix. The fusiform tumor cells were arranged in a multi-nodular pattern, with indistinct cell margins, slightly eosinophilic cytoplasm, and hyperchromatic atypical nuclei. Mitoses were infrequent. In addition, many elongated curvilinear capillaries were observed (Figure \n). These cytological findings suggested a malignant tumor of mesenchymal origin.\nOn 3 September 2011, the patient underwent operation for the metal implants removal and tumor resection. Intraoperative findings indicated that the tumor infiltrated into the middle of the femur shaft and the surrounding tissues including the rectus femoris, sartorius, pectineal muscle, long adductor muscle, gracilis muscle, vastus medialis, vastus lateralis, vastus intermedius, and biceps femoris. The tumor and bone tumor segment approximately 15 cm in length were resected, and the surgical margin was microscopically free of tumor. Sequential bone defect reconstruction after tumor resection was performed by reimplantation with the alcohol inactivated bone tumor segment, in which the vascularised autologous left fibula approximately 18 cm in length was inserted. Then, an internal fixation with titanium alloy plates and screws was once again performed, and a closed vacuum drainage system was used.\nMacroscopically, the excised specimen was a grayish-white or grayish-brown mass measured 7 × 14 × 10 cm. On the cut section, a fish-like appearance with central necrosis area was observed. Microscopic features were identical to that of the biopsy. Immunohistochemical studies showed that the tumor cells expressed strong immunoreactivity for vimentin (VIM) and negative for cytokeratin (CK), suggesting a mesenchymal histogenesis (Figure \na and b). Labeling index of Ki-67 (the number of Ki-67 positive tumor cells divided by the sum of Ki-67 positive and negative tumor cells) was 25% (Figure \nc). Muscle-specific actin (MSA), smooth muscle actin (SMA) and desmin (DES) were negative in the tumor cells, eliminating the possibility of a muscle-derived tumor (Figure \nd-f)\n[]. The negative expression of S-100 protein eliminated a tumor of neural or adipose tissue origin (Figure \ng). The tumor cells were negative for cluster of differentiation 34 (CD34) (Figure \nh), B cell lymphoma/lewkmia-2 and anaplastic lymphoma kinase, which eliminated the possibility of a solitary fibrous tumor or inflammatory myofibroblastic tumor\n[]. Interestingly, the positive staining of CD34 in vascular endothelial cells highlighted the curvilinear capillaries, which was an important histological feature of myxofibrosarcoma. In addition, positive expression of murine double minute 2 (MDM2) was observed in the tumor cells. Although MDM2 immunostaining is useful adjunct in diagnosing well-differentiated and dedifferentiated liposarcoma subtypes, overexpression of MDM2 has also been reported in a small number of myxofibrosarcomas\n[-]. The combination of clinical and pathological features revealed a low-grade myxofibrosarcoma.\nThe patient received three cycles of postoperative adjuvant chemotherapy with a combination of ifosfamide, doxorubicin, cisplatin and mesna, and he experienced no special discomfort during chemotherapy. In June 2012, the patient underwent the third operation for the removal of metal implants due to implant breakage in an accidental fall, followed by external fixation.\nHowever, in June 2013, only 22 months after tumor resection, a firm and painless mass approximately 5 cm in diameter was palpated in the medial left thigh. Magnetic resonance imaging (MRI) showed an irregular soft tissue mass with ill-defined margin in the anteromedial left thigh, accompanied by surrounding soft tissue swelling and normal muscle structure disappearance. Additionally, bone destruction in the left femur shaft was also observed (Figure \n). Tissue biopsy was performed and histopathological examination showed that the tumor exhibited the classical histological features of myxofibrosarcoma, including a nodular appearance, prominent myxoid matrix and elongated curvilinear capillaries. The morphology of the tumor cells varied from small and bland to enlarged, bizarre, pleomorphic and multinucleated, suggesting increased atypia and pleomorphism compared with those in the primary tumor (Figure \n). The combination of clinical and pathological features revealed a recurrent myxofibrosarcoma with higher malignancy. There was no evidence of distant metastasis. The patient eventually underwent left hip disarticulation due to the inability to control the tumor progression.
A 25-year-old active young woman (weight 57 kg, height 1.64 m) suffered a left patella fracture due to a slip and fall. She had a medical history of right knee arthroscopy in 2001 due to a cruciate ligament injury. Complete blood count, electrolyte panel, coagulation screening, electrocardiography, and plain radiographic findings were all within normal limits. A physical examination revealed moderate tenderness and swelling in the left thigh and knee area. We could not check motor grade of the left knee due to severe pain. Open reduction and internal fixation with a tension band wiring was performed under spinal anesthesia three days after admission to the emergency room. A pneumatic tourniquet was inflated to 370 mmHg at the onset of the procedure and was deflated 80 minutes after surgery. Her leg was elevated to 30 degrees immediately after surgery, and an ice bag was applied to the operation site. Immediate full weight bearing and range of motion were allowed. There were no signs of infection. The patient was discharged with a crutch four days after the operation.\nThe patient returned the following week for consultation. She had marked pain in the left knee area, difficulty in extending the knee, and weakness of the quadriceps muscle. She was referred to a rehabilitation physician and a neurologist for electromyography-nerve conduction velocity (EMG-NCV) testing, but she refused the test.\nEight weeks after being discharged, she was readmitted because of persistent pain and neurologic symptoms. At that time, manual muscle testing of the left lower limb showed grade IV hip abduction, grade V adduction, grade III hip flexion, grade II knee extension, grade V ankle dorsiflexion, and grade V plantar flexion. Pinprick sensation of the femoral nerve and lateral cutaneous nerve in the thigh area was decreased to 8 points on a scale from 0 to 10 points. Reflexes at the left patella were not assessable. Knee pain was measured as 5 on a visual analogue scale. MRI of the knee showed no evidence of abnormalities of the anterior cruciate ligament, posterior cruciate ligament, lateral collateral ligament, medial collateral ligament, or meniscus abnormality, except for slight increase in the amount of joint effusion. However, evaluations of the patella, infrapatellar fat pad, quadriceps tendon, patellar tendon, and anterior horn of medial/lateral menisci were limited due to severe metallic artifacts. Diffuse T2 high signal intensity was observed in the anterior compartment of the left thigh, suggesting an acute denervation change related to left femoral neuropathy. Furthermore, the whole volume of the muscles of the left thigh was reduced, suggesting combined disuse atrophy (). MRI of the lumbar spine depicted a normal spinal cord, L4 nerve roots, and lumbar plexus, except for bulging discs with an annular tear at L3-4, L4-5, and L5-S1. In addition, mildly increased echogenicity was noted in the left anterior compartment muscle with decreased volume by joint ultrasonography, suggesting disuse atrophy or a denervation change.\nAn EMG study was performed on the left tibialis anterior, gastrocnemius medialis, vastus lateralis, vastus medialis, rectus femoris, adductor brevis, gluteus medius, and lumbar paraspinal (L3 and 4) muscles. Positive sharp waves and fibrillation potentials were observed in the vastus lateralis, vastus medialis, and rectus femoris muscles by needle electromyography. Furthermore, a sensory nerve conduction study (NCS) revealed reduced conduction for the left saphenous nerve. One week after the test, a follow-up EMG study was done by another neurologist, and the results were the same.\nAccordingly, the study established the presence of partial peripheral neuropathy of the left femoral nerve. The patient was referred for electrical stimulation therapy and steroid therapy but refused any further treatments. The internal fixation device was removed at two months after surgery due to severe pain, and subsequently, the knee pain scale improved to visual analogue scale 3 five days after the device removal.
A 42-year-old man visited our outpatient clinic presenting with both leg weakness. Eight months before the visit, he had been diagnosed with elevated intraocular pressure accompanied by continuous headache and eyeball pain. Despite treatment for the glaucoma, the symptoms persisted and became aggravated. A week before admission, he experienced a seizure attack for several seconds, and a computed tomography scan at another hospital revealed hydrocephalus without intracranial causes. He complained of progressive both leg weakness, and sensory change below the T2 dermatome was found. He was then transferred to our institute. He had no other medical history. He had lost weight of more than 10 kg for the last 7 months. He complained of difficulties in urination and defecation. The neurologic examination showed hypoesthesia below the T2 and hyperactive deep tendon reflexes of the lower extremities.\nSpinal magnetic resonance image (MRI) was done with contrast dye enhancement (). An intradural extramedullary (IDEM) mass lesion was found at the T2 level, ventral side to the spinal cord. The mass demonstrated a lower signal intensity than the spinal cord, and well enhancement of gadolinium in the T1-weighted image. The dorsal portion of the dura mater was slightly enhanced. There was no other lesion on the whole spine image. A brain MRI was performed, which showed no definite identifiable lesions.\nSurgery was performed with respect to the benign IDEM tumor. A posterior midline approach was performed followed by a T2 laminoplasty. The dura matter was dark gray in color and was opened (), which showed a dark gray-colored mass that was severely adhered to the spinal cord and roots. After dissection, normal spinal cord was identified and the mass was removed as much as possible grossly. The black colored arachnoid membrane was also removed, but total resection was impossible. After decompression of the spinal cord, the dura was closed and sealed. There was no adverse event during the operation.\nHistopathologic examination revealed a black or dark gray colored fragile mass in the gross view. Hematoyxlin and eosin stain of the tumor showed melanin pigmentation of tumor cells (). Immunohistochemical stain for human melanoma black revealed positive results for malignant melanoma. The MIB-1 index of 2.6%, atypia, and mitosis (0/10 in high power field) indicated that the melanoma was of intermediate grade.\nAfter surgery, positron emission tomography (PET) was performed for evaluation of the primary lesion but there were no demonstrable metastatic or other primary melanoma lesions beyond the spinal cord. There were no skin lesions on physical examination. During the hospitalization period after surgery, a seizure attack occurred which was a generalized tonic and clonic type. The cause for the seizure was suspected to be hydrocephalus, so a ventriculo-peritoneal (VP) shunt was planned. CSF study was done but no malignant cells were observed. However, the patient refused further treatment and was discharged. He also refused further adjuvant treatment. The patient revisited our hospital and was admitted again 22 months after the initial surgery with progressive both leg we-akness. During the period of follow-up loss, a VP shunt was performed in another hospital but the patient still had not received chemotherapy and radiotherapy for the melanoma. An MRI scan was done which showed that the spinal cord had swelled with dorsal side adhesion to the dorsal surface of the dura mater. The surface of the entire spinal cord was enhanced and this finding was regarded as leptomeningeal dissemination (). Revision surgery for decompression of the spinal cord was performed. There was more aggressive dissemination of the melanoma in the arachnoid and pia mater. There was severe adhesion between the melanin-pigmented tissue and neural tissues. Bleeding continued during removal of the melanoma and it was difficult to control. Total resection of the melanoma was impossible but decompression of the spinal cord was achieved. After the revision surgery, weakness of both legs had slightly improved but the patient refused additional treatment again and was discharged finally.
A 35-week preterm male neonate was born to a 29-year-old healthy mother. Antenatal ultrasound at the 32-week gestation showed left-sided CDH with no additional anomalies noted on scan. After delivery, the baby was intubated and further evaluation confirmed left CDH and no other associated malformations. On the 3rd day of life, after stabilization, he was taken to operating room for repair of his left CDH through transabdominal approach. Operative findings revealed classical posterolateral CDH with herniation of the stomach, small bowel, colon, and spleen into left hemithorax, with no malrotation of the bowel. Further examination revealed a 1 × 1 cm wide base MD over the antimesenteric border of the ileum almost 40 cm from the ileocecal junction. The palpation of MD did not reveal any nodule/mass within it. In addition, there was an isolated 3 × 1 cm yellow, well-circumscribed, lobulated mass over the antimesenteric border of jejunum approximately 15 cm distal to the duodenojejunal junction. Close inspection of this mass was suggestive of an ectopic pancreatic tissue, which was extending from serosa to the lumen of jejunum and had no anatomical and vascular continuity with the main body of the pancreas. The pancreas and hepatobiliary tract were anatomically normal in shape and position.\nThe diaphragmatic defect was closed primarily after reducing the contents from left hemithorax. Later the jejunal pancreatic nodule was gently dissected from serosa to mucosa and removed in a single piece. Through enterotomy, jejunum was examined and noted to be healthy with no any more ectopic tissue inside the jejunum. The enterotomy was closed followed by digital examination of MD and it was confirmed to be empty with no contents inside; hence, it was not excised. Patient tolerated surgical procedure very well and his postoperative recovery was smooth. A repeat abdominal ultrasound on the 7th postoperative day was performed, which documented normal pancreas and no other associated anomalies. Histopathological examination of resected specimen showed benign pancreatic exocrine acinar tissue (typical of pancreatic heterotopia) as shown in . In a regular followup after 18-months of surgery, he has been free of any complications.
A 47 year-old-man, human immunodeficiency virus negative, with a recent diagnosis of central nervous system lymphoma presented with perianal skin lesions and nonbloody diarrhea. The skin lesions began as nodules that progressed to a roughened plaque and in the minority, ulceration. The lesions began eight weeks prior to presentation, were multiple, scattered in the perianal area and upper thigh bilaterally, and three centimeters at the widest diameter (). A more distinct lesion with irregular border was present over the sacrum consistent with a decubitus ulcer. The patient had been diagnosed with a CNS lymphoma six weeks prior to admission when he presented to another hospital with lower extremity weakness, saddle anesthesia, and fecal incontinence, and was found to have a L1-L2 intramedullary spinal mass. He had emergent surgical resection of the mass and was treated with high-dose dexamethasone and further radiation therapy. He was additionally found to have a right temporal lobe mass that was resected and pathology was consistent with a B-cell lymphoma with T-cell infiltration. He had no other significant past medical history or immunocompromising condition. The lower extremity weakness persisted and primarily confined him to bed. For ongoing fecal incontinence and the nonhealing sacral decubitus ulcer, he ultimately had a diverting colostomy. Two weeks prior to presentation, his bowel movements became more liquid in consistency and increased in frequency. He denied abdominal pain. The perianal skin lesions were not painful, but the initial loss of sensation from the compressive spinal mass had not recovered. He remained on a dexamethasone taper and was transferred to this hospital for further evaluation for chemotherapy. His complete blood count was notable for a normocytic anemia, and his liver function tests for an elevated alkaline phosphatase. An excisional biopsy of one of the skin lesions from the upper thigh was obtained and sent for pathological examination.\nSections of the pathological specimen revealed a dense neutrophilic crust with numerous bacteria distributed in varying sized aggregates, and the underlying epidermis was acanthotic with artefactual subepidermal clefting. However, the superficial dermis demonstrated a distinct perivascular lymphocytic infiltrate, and the endothelial cells were markedly enlarged (). Some endothelial cells contained magenta-colored nuclear inclusions consistent with cytomegalovirus (CMV) infection which was confirmed by a positive CMV immunostain (). Stains with periodic acid-Schiff and for acid-fast bacilli were negative.\nThe patient was treated with intravenous ganciclovir at five mg/kg every twelve hours for 21 days followed by transition to valganciclovir. A serum CMV viral load was 16,400 copies/mL at treatment initiation and was undetectable after four weeks of treatment. The diarrhea resolved within two weeks, and the skin lesions had completely healed at the time of switch to valganciclovir. Based on pathological findings and response to treatment, the diagnosis of cutaneous CMV was made. The primary mode of pathogenesis was thought to be local inoculation from fecal shedding. The diarrhea appeared to be a manifestation of CMV colitis, but given the commitment to CMV treatment and the response to therapy, no further investigation was performed. Unfortunately, the patient later died secondary to complications of an intracranial hemorrhage at the site of the temporal lobe resection.
A 60-year-old man presented with the history of progressive dysphagia, regurgitation and an offensive smell of fermented food from his mouth since his surgery for achalasia cardia 32 years ago. The precise details of the surgery were not available. It was also not known whether an anti-reflux procedure was done at the previous surgery although a recent CT scan had shown a partial wrap. He claims that his dysphagia and regurgitation were immediately improved after his first operation. But after a year or so he started having dysphagia again which got progressively worse over the years. On examination, his general health was satisfactory. The previous Heller was done by open transabdominal approach and he had an upper midline laparotomy scar []. His routine work-up revealed the following findings:\nEndoscopy: The esophagus was dilated and contained fluid residue, wall was hypertrophied with multiple contraction ring. There was no stricture at the lower end and scope passed easily through to the stomach.\nBarium meal: A persistent bird's beak image of cardia with a dilated sigmoid esophagus [].\nCT chest and abdomen: Dilatation of esophagus with air fluid level seen up to gastroesophageal junction. Dilatation at its maximum was 7.8 cm (grade IV). Below the diaphragm a small paragastric air fluid level was seen due to previous surgery [].\nUnfortunately, the pH and manometry services were not available to study the lower esophageal sphincter pressure and the reflux. The radiological and endoscopic findings were highly suggestive of recurrent achalasia cardia. He underwent three courses of progressive balloon dilatation but showed no improvement in his symptoms and was referred for surgery.\nThe port positions were same as for laparoscopic hiatus hernia repair []. The liver was completely adherent to the diaphragm and retraction of liver was not necessary requiring only four ports []. The surgery was difficult because of loss of planes, distorted anatomy, excessive scarring and severe adhesions in the esophagogastric region. After adhesiolysis around the hiatus area, it became clear that a posterior fundoplication was attempted at previous surgery, which had disrupted. We chipped away slowly and steadily and the hiatus and the crure were cleared. The esophagus was mobilized circumferentially in the posterior mediastinum and was straightened. The posterior vagus nerve was identified after undoing the fundoplication, the anterior vagus nerve was seen proximally in the mediastinum to the left of the previous myotomy and was lifted away to complete the myotomy, excessive care was taken not to injure the vagi. The proximal myotomy was extended to 8-9 cm and the lower limit of the cardiomyotomy was extended up to 3 cm on the stomach to completely divide the lower esophageal sphincter.[] Myotomy was performed using ultracision shears [Figure , ]. Per-operative endoscopy was helpful during the surgery. Completeness of the myotomy was confirmed on table endoscopy and at the same time air leak test was done to rule out any mucosal perforation. The posterior fundoplication was converted to floppy Dor fundoplication after dividing the short gastric vessels [Figure , ]. The Surgery lasted for 180 minutes. There were no per-operative complications and no blood transfusion required. He was fed liquids on first postoperative day. His dysphasia and regurgitation improved considerably and postoperative barium meal done at one month and six months showed the cardia diameter of more than 1 cm, significant reduction in the esophageal diameter and free passage of barium in the stomach.
A 52-year-old male patient presented with the complaint of sudden vision loss in his left eye 3 days earlier. Past medical history was significant for chronic kidney disease, secondary hypertension, chronic hepatitis C virus infection and arrhythmia. Ophthalmologic examination revealed best corrected visual acuity of 10/10 in the right eye and 4/10 in the left eye from the temporal field. Confrontation test revealed inferonasal visual field loss in the left eye. Direct and indirect light reflexes were normal in both eyes and there was no relative afferent pupillary defect. Anterior segment examination was normal and intraocular pressure was 13 mmHg in both eyes. Dilated fundus exam demonstrated soft exudates consistent with hypertensive retinopathy in the right eye. Fundoscopy of the left eye revealed an area of pallor in the superotemporal quadrant and the macula with macular cherry red spot, which were consistent with occlusion of the superotemporal branch of the left retinal artery (). On OCT, peripapillary retinal nerve fiber layer (RNFL) thickness was within normal limits (). In the patient’s visual field, there was an inferonasal defect in the left eye corresponding to the occluded region (). The patient was treated with a single dose of 500 cc intravenous dextran-40 and 200 mg intravenous pentoxifylline. In etiologic studies, Doppler ultrasonography revealed an atherosclerotic stenosis in the right and left main carotid arteries and a calcified plaque causing luminal narrowing in the left internal carotid artery. Transthoracic echocardiography revealed second- to third-degree aortic valve regurgitation and first-degree tricuspid valve regurgitation. There was no improvement in visual acuity or visual field despite treatment. At follow-up 7 months later, OCT showed thinning of the superior, inferior and temporal peripapillary RNFL (). On the thickness map, ganglion cell layer was thinner in the superior and temporal areas (). Decreased vascular density in the superficial and deep capillary plexus consistent with ischemia in the regions supplied by the superotemporal branch of the retinal artery was observed in a 6x6 mm macular field on OCTA (). The borders of the ischemic area were more clearly seen in en face images (). In optic disc OCTA, capillary density was reduced in the superotemporal region and collateral vessels were present in the area (). When compared to the fellow eye, there was a decrease in the macular deep and superficial capillary density in the superior and temporal quadrants () and a decrease in peripapillary capillary density in the superior quadrant (). Visual field loss persisted in post-treatment threshold perimetry ().
A 60 year old man, who was under regular haematological follow-up because of myelofibrosis, presented at a routine clinic visit complaining of increasing shortness of breath, weight loss and lethargy. Clinical examination of the chest was normal but a chest x-ray (CXR) showed multiple lung lesions consistent with metastatic deposits (figure ). A staging computed tomography (CT) scan done shortly afterwards showed marked splenomegaly, causing displacement of the left kidney medially. In the left kidney, there was a 5 cm soft tissue mass arising from the middle of the kidney with characteristics of primary renal cancer (figure ). The staging CT chest showed multiple pulmonary metastases in both lungs.\nThe patient underwent laparoscopic cytoreductive nephrectomy without complications and agreed to have immunotherapy with alpha-interferon. Histology revealed clear cell renal cell carcinoma. Six weeks following the operation, just prior to commencing immunotherapy, he attended the haematology clinic as routine follow-up. His initial symptoms had completely resolved and a repeat chest radiograph on that day showed clear lungs with no evidence of metastatic deposits (figure ). Although no histological confirmation of the metastatic nature of the lung lesions was obtained, it is highly likely that his pulmonary metastases had regressed spontaneously as the patient had not received any immunotherapy in the meantime. The patient remains well five months after the operation.\nRenal cell cancer accounts for 2% of all cancers and its incidence is steadily rising. It usually presents in late adult life and is more common in males than females. Although they are associated with Von Hippel-Lindau disease, adult polycystic kidney disease and multicystic nephroma, most renal cell cancers develop spontaneously [].\nThe patient may present with urological symptoms such as haematuria or flank pain or with an abdominal mass or alternatively with systemic manifestations, such as anaemia and fever, or symptoms of metastatic disease and other rare phenomena [].\nApproximately 20% to 30% of patients with renal cell carcinoma present with metastatic disease, and 20% to 40% of patients undergoing nephrectomy for clinically localized disease will develop metastasis []. If the tumor cannot be completely resected, the course is generally relentlessly progressive, with median survival of 12 to 18 months after metastasis. 85% of relapses occur in the first three years [].\nHowever, a group of patients with advanced disease have experienced improvements in survival, which is partly related to the introduction of immunotherapeutic approaches and a better understanding of the role and timing of cytoreductive nephrectomy. Although the benefits of immunotherapy have been displayed repeatedly by several studies, controversy has existed as to the need for adjunctive nephrectomy in treating metastatic patients. Removal of the malignant kidney may be of palliative benefit in some settings of metastatic renal cell carcinoma []. There have been studies to demonstrate that the benefits of nephrectomy are in addition to and probably greater than the benefit resulting from the interferon-alpha that all patients would receive. Others argue that immunotherapy as a modality has had disappointingly little proven impact on the survival of patients with advanced renal cell carcinoma compared to a variety of other options with less toxicity [].\nThere have been case reports in the literature that describe spontaneous regression of metastatic renal cancer [,-].\nBumpus described the first reported case of spontaneous regression of metastatic renal cell carcinoma in 1928 []. Metastatic sites include brain, bone, hilar adenopathy and most commonly pulmonary metastases. The clinical pattern of the improvement is not uncommonly the complete disappearance of disease, and often the regression is long-lasting. Many of these cases are associated with surgical removal of the primary tumor, but regression can also occur in association to radiation or embolization of the primary tumor [].\nThe rarity of the observation and the heterogeneity of the clinical circumstances in which spontaneous regression of disease occurs do not provide the opportunity for insight into the pathophysiologic mechanism or into the capability for the identification of potential candidates for regression. Although no single mechanism can completely account for this phenomenon, it can be speculated that resection of the primary tumour may result in removal of a prometastatic or growth factor secreted by the tumour and/or promotion of apoptosis might be involved. Immunologic factors almost certainly play a role in some cases of spontaneous tumour regression and perhaps removal of bulk tumour enables or stimulates the immune system to control residual disease. Other theories include hormonal changes, trauma and changes in blood supply (via inhibition of angiogenesis by cytokines) [].
A 48-year-old female patient was admitted to the emergency department due to a stab wound of the neck region (). She was fully conscious and her vital signs were in the normal range. The rescue team leader informed us that the patient had tried to commit suicide.\nAfter the completion of the initial medical history, a thorough physical examination was conducted to detect any major vital organ damage to the neck and concomitant injuries. There was no presence of active bleeding, no bruit, no pulsatile hematoma, no sign of central neurological deficit, hemoptysis, stridor, hoarseness, dysphagia or subcutaneous emphysema. The patient, with full cardiopulmonary viability, was transported to the radiology department to conduct a complete computed tomography (CT) scan of the neck and thorax.\nWe were quite astonished with the results, which showed that the knife reached completely through the central mediastinum to the posterior mediastinum, attaining the aortic arch, gently leaning on the vessel's adventitia, and not damaging in the process any vital structure (). The CT scan revealed a bilateral pneumothorax, due to which bilateral drainage of the pleural cavity was administered through the second intercostal space in the midclavicular line using Thorax 24 F drains (). After completing the preliminaries mentioned above, the patient was transported to the operating room.\nA left-sided anterolateral thoracotomy was conducted in the supine position. The access limited the direct view into the posterior mediastinum, but our idea was to reduce any patient rotation to a minimum, so that the injury to the vascular wall of the aorta was not compounded.\nAfter ensuring that the tip of the blade did not penetrate completely through the aortic vessel wall, but only bordered the surface of adventitia of the aortic arch, the first assist removed the knife.\nSubsequently to the removal of the blade, no hemorrhage to the pleural cavity was noted, and at this stage the decision to leave an additional drain in the left pleural cavity and to close the thorax was made.\nAfterwards the neck injury exploration was begun. To extend the post-injury wound a cut along the medial edge of the right sternocleidomastoid muscle was applied. During the dissection towards the trachea no damage to the vital anatomic structures was noted. However, after the deep layers behind the trachea were explored, tangential damage to the tunica adventitia of the esophagus was discovered. The esophageal injury was attended to with simple monolayer interrupted stitches. A Redon drain was left in the attended injury site, which was removed on the second day after the surgery. The upper drains from the pleural cavity were removed on the third day after surgery, and the left lower drain on the second day. The patient was discharged to a psychiatric hospital on the seventh day after the surgery. During the follow-up visit on the tenth day the stitches were removed, and no complications were noted.
An 88-year-old female presented to the emergency department with abdominal pain and vomiting. She was mildly tender in her right upper quadrant but was focally more tender in her right lower quadrant. Her blood results showed an elevated white cell count of 12.9 × 109 l–1 and a neutrophil count of 10.4 × 109 l–1. Furthermore her C-reactive protein level was 34 mg/L. An abdominal ultrasound showed multiple calculi within the gallbladder. Gallbladder wall thickness was not assessed. A non-contrast CT scan, due to the patients impaired renal function, was then ordered as the patient’s physical exam findings were not in keeping with a diagnosis of cholecystitis.\nThe CT showed abnormal dilatation of the proximal small bowel with a possible transition point in the left iliac fossa. Pneumobilia was also noted due to prominence of the hepatic biliary tree (). No evidence of a ductal calculus was observed. The CT scan was reported as showing features of a bowel obstruction with a transition point in the left iliac fossa. The pneumobilia was reported as being possibly an indicator of previous sphincterotomy. There was no evidence of appendicitis or another inflammatory process in the right iliac fossa.\nThe following day, while proceeding with conservative management, the treating team requested a small bowel follow through to investigate the obstruction. After further review and discussion with the treating team it was revealed that there was no history of an endoscopic retrograde cholangio-pancreatography or cholecystectomy. An addendum was added to the CT report, suggesting that the appearances were indicative of a gallstone ileus from a choledochoduodenal fistula () with a non-calcified gallstone possibly being present at the transition point in the left iliac fossa ().\nThe patient underwent a laparotomy and a gallstone was found obstructing the distal jejunum. A 41 mm gallstone was removed by vertical enterotomy, later confirmed on histological diagnosis. The patient had an uneventful recovery in hospital and was discharged a few days later to a regional hospital for rehabilitation. A cholecystectomy or fistula closure was not performed and she was not referred for further surgical follow-up on discharge, likely due to the patient’s advanced age and medical comorbidities.
A 14-year-old boy, educated up to class 6, belonging to a family of middle socioeconomic status and residing in an urban area was brought with complaints of academic decline since 3 years and hearing voices for the past 2 years. The child was born out of a nonconsanguineous marriage, an unplanned, uneventful, but wanted pregnancy. The child attained developmental milestones as per age. From his early childhood, he was exposed to aggressive behavior of his father, who often attempted to discipline him and in this pursuit at times was abusive and aggressive toward him. Marital problems and domestic violence since marriage lead to divorce of parents when the child attained age of 10 years.\nThe following year, the child and the mother moved to maternal grandparents’ home and his school was also changed. Within a year of this, a decline in his academic performance with handwriting deterioration, and irritable and sad behaviour was noted. Complaints from school were often received by the mother where the child was found engaged in fist fights and undesirable behavior. He also preferred solitary activities and resented to eat with the rest of the family. In addition, a decline in performance of daily routine activities was seen. No history suggestive of depressive cognitions at that time was forthcoming. A private psychiatrist was consulted who treated him with sodium valproate up to 400 mg/day for nearly 2 months which led to a decline in his irritability and aggression. But the diagnosis was deferred and the medications were gradually tapered and stopped. Over the next 1 year, he also started hearing voices that fulfilled dimensions of commanding type of auditory hallucinations. He suspected that family members including his mother collude with the unknown persons, whose voices he heard and believed it was done to tease him. He eventually dropped out of school and was often found awake till late night, seen muttering to self, shouting at persons who were not around with further deterioration in his socialization and self-care. Another psychiatrist was consulted and he was now diagnosed with schizophrenia and treated inpatient for 2 weeks with risperidone 3 mg, olanzapine 2.5 mg, and oxcarbazepine 300 mg/day with some improvement in his symptoms. Significant weight gain with the medication lead to poor compliance which further led to relapse within 3 months of discharge. Frequent aggressive episodes over the next 1 year resulted in multiple hospital admissions. He was brought to us with acute exacerbation of symptoms and was receiving divalproex sodium 1500 mg/day, aripiprazole 30 mg/day, trifluperazine 15 mg/day, olanzapine 20 mg/day, and lorazepam injection as and when required. He was admitted for diagnostic clarification and rationalization of his medications. He had remarkable physical features of elongated face with large ears. Non-cooperation for mental state examination, and aggressive and violent behavior were noted. He was observed to be muttering and laughing to self. His mood was irritable, speech was laconic, and he lacked insight into his illness. We entertained a diagnosis of very early-onset schizophrenia and explored for the possibilities of organic psychosis, autoimmune encephalitis, and Fragile X syndrome. The physical investigations done are shown in . Further special investigations in the form of rubella antibodies (serum IgG = 64.12 U/mL, IgM = 2.44 U/mL) and polymerase chain reaction for Fragile X syndrome (repeat size = 24) were normal. His intelligence quotient measured a year ago was 90, but he did not cooperate for the same during present admission. Initially, we reduced the medication and only kept him on aripiprazole 30 mg/day and added lurasidone 40 mg twice a day and discharged him with residual negative symptoms only. However, his hallucinations and aggression reappeared within 2 weeks of discharge and was readmitted. This time eight sessions of bilateral modified electroconvulsive therapy were administered and he was put on aripiprazole 30 mg/day, chlorpromazine 600 mg/day, sodium divalproex 1000 mg/day, and trihexyphenidyl 4 mg/day. The family was psychoeducated about the illness, and mother's expressed emotions and overinvolvement was addressed by supportive psychotherapy. Moreover, an activity schedule for the child was made, and occupational therapy was instituted. Dietary modifications in view of weight gain were also suggested. In the past 6 months, no episodes of violence came to our notice, though irritability on not meeting his demands is persistent. However, poor socialization, lack of motivation, apathy, weight gain subsequent to psychotropic medications, and aversion to start school are still unresolved. Influence of his multiple medications on bone marrow function is an impending issue of concern.

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