A 38-year-old man sustained injuries in both lower extremities in a motor vehicle accident. Bilateral closed femoral shaft fractures were present, along with bilateral tibio-fibular fractures and severe soft tissue injury. The right leg was amputated at the above-knee level on an emergent basis, due to extensive comminution and loss of circulation caused by the crush injury. The bilateral femoral shaft fractures were fixed with plates. Reduction and external fixation of the left tibia was performed along with titanium plating. Initially, the size of the soft tissue defect was 20 × 15 cm, and the fractured tibial segments were exposed. The patient was treated in the emergency intensive care unit for 2 weeks due to multiple rib fractures and hemothorax. A bacterial culture from the tibial defect site revealed the presence of Pseudomonas aeruginosa. Wound care was performed with betadine, saline solution irrigation, and betadine-soaked dressings. The patient was shifted to the general ward 2 weeks after the trauma, at which time debridement and ostectomy of the necrotic tibia were performed. The tibial defect measured 17 cm. A vascularized bone graft was required to reconstruct the tibia; however, the contralateral fibula was lost due to the trauma and the ipsilateral fibula had multiple segmental fractures. We conserved the defect by external fixation of the tibia until complete union of the fractured fibula was achieved.
The P. aeruginosa infection did not resolve despite intravenous antibiotic injections and debridement. The patient was transferred to the isolation ward due to neutropenic fever, 7 weeks after the trauma. Carbapenem and other analgesics that were assumed to have caused the neutropenic fever were discontinued. After 1 week of observation, the patient returned to the general ward. Thorough sterile irrigation and dressing, as well as debridement, were performed under general anesthesia twice in order to eradicate P. aeruginosa without antibiotics. During the conservative management period, union of the fractured fibula was observed on serial follow-up radiographs.
Four months after the trauma, decrease of P. aeruginosa was observed in wound culture from a 20 × 15 cm skin defect, and a united fibula was noted on radiography (). We reconstructed the tibial defect using the ipsilateral fibula, including the united fracture segments.
First, computed tomography angiography was performed before surgery to check whether the three main arteries of the lower extremities were intact. The fibula was approached through a curvilinear posterior incision. The lateral head of the gastrocnemius was mobilized from the distal femur and the soleus was stripped from its origin from the posterior aspect of the fibula and the interosseus membrane. The popliteal vessels were dissected distally to the tibial vessels and the peroneal artery, which supplied the pedicle to the fibula. The fibula was completely exposed and ostectomy was done containing the flexor hallucis longus muscle and the peroneal artery. The 17-cm vascularized fibular flap was transposed through the intermuscular septum. After the transposition of the vascularized fibular flap, a bipedicled flap was transposed from the medial to the anterior side to cover the area of bone exposure. The harvested fibula was affixed to the tibial defect site using a plate and the external fixator system was maintained for stable alignment of the tibia (). The size of the soft tissue defect was 20 × 15 cm, and it was covered with the bipedicled skin flap.
Two months after surgery, the flap was stable and bony callus formation was observed on radiography. The patient started non-weight-bearing and muscle-strengthening exercises of the left lower leg. Three months postoperatively, hypertrophy of the transferred fibula and complete healing of the femur was observed on radiography. Weight-bearing training was started with a lower limb prosthesis on the amputated side. The patient learned to take steps with the prosthesis and the reconstructed lower leg with the help of a walker. Two weeks after the start of weight-bearing exercises, the patient had a fracture in the tibia at the weak point of the proximal plate fixation site. After confirming fracture union, weight-bearing training was restarted with a walker. Subsequently, the patient began to ambulate independently without the walker and returned to routine activities 6 weeks after the second tibial fracture. Two years postoperatively, good bony union and fibular hypertrophy were observed (). We confirmed the patient’s satisfaction in performing daily living activities with the reconstructed leg at a 12-year follow-up ().


Our patient was a 29-year-old male who presented with loss of vision in the right eye. He also reported fatigue, dyspnea, night sweats and left upper quadrant fullness for approximately one month before presentation. His initial complete blood count revealed a total leukocyte count of 380,000/mm3 with 66% blasts, 5% segmented neutrophils, 8% monocytes, 3% eosinophils, and other immature myeloid cells (). He was anemic and thrombocytopenic with a hemoglobin of 7.8 gm/dL and a platelet count of 35,000/mm3. A bone marrow biopsy was a diagnostic of AML with monocytic differentiation. There were no FLT3 nor NPM1c terminal mutations. Cytogenetic analysis showed a 3-way translocation involving chromosome 5 and both chromosomes 7 with breakpoints at 5q33.2, 7q32 and 7q11.2 [(46, XY,t(5;7;7)(q33.2;q32;q11.2)] as the only abnormality (). Interphase fluorescence in situ hybridization (FISH) was positive for PDGFRB rearrangement. Metaphase FISH with PDGFRB and with whole chromosome painting probes to chromosomes 5 and 7 confirmed the 3-way translocation and showed that sequences 5ʹ to PDGFRB were translocated to the derivative chromosome 7 with the 7q32 breakpoint (). For other PDGFRB fusions, the critical oncogenic fusion is on the derivative chromosome 5, which retains the 3ʹ portion of PDGFRB (). Thus, the partner gene for this unidentified PDGFRB rearrangement appeared to lie at the 7q11.2 breakpoint (). BCR-ABL1 fusion was not observed. The patient was started on induction chemotherapy with cytarabine and daunorubicin (7+3). Bone marrow biopsies on days 14 and 28 were negative for AML consistent with a successful induction.
When he presented for consolidation chemotherapy, the patient was found to have a total white blood cell count of 35,000/mm3 with 50% segmented neutrophils, 21% monocytes, and no blasts. Due to concern for disease relapse, a repeat bone marrow biopsy was performed which demonstrated 95% hypercellular marrow with granulocytic hyperplasia, monocytosis, eosinophilia, and megakaryocytic hypoplasia. Morphologically, the bone marrow was consistent with chronic myelomonocytic leukemia (CMML) with eosinophilia of 7%. Eosinophilia was not present in the peripheral blood. The patient had persistence of the same clonal rearrangement. The patient proceeded with high-dose ARA-C (HiDAC) for consolidation. Due to the PDGFRB translocation, imatinib 400 mg daily was added to his consolidation regimen.
A repeat bone marrow one month after initiation of imatinib therapy demonstrated continued morphological remission but persistent PDGFRB rearrangement. He subsequently underwent a matched unrelated donor alloSCT 3 months after initiation of imatinib with myeloablative conditioning. He has remained in a clinical and pathological CR 8 years later.


A 52-year-old female presented to the emergency department with a 5-day history of worsening abdominal pain, distension and vomiting. On examination she was peritonitic, tachycardic, hypotensive and tachypnoeic with a new oxygen requirement of 4 L.
She had a history of lung adenocarcinoma with brain metastases diagnosed 3 months prior to this presentation, for which she had a craniotomy with resection of metastases and cyberknife radiotherapy. In addition, she was on a weaning regime of oral dexamethasone for the brain metastases, on 2 mg three times a day at presentation, reduced from 8 mg twice daily over the prior 3 months since the diagnosis of malignancy. She had no other significant past medical history and the only other medications she took were lansoprazole for gastric protection whilst on dexamethasone and hormone replacement therapy for menopausal symptoms. She had not started other treatments for her malignancy prior to this presentation.
Imaging demonstrated perforation of the large bowel secondary to diverticulitis, and a coincidental finding of bilateral, patchy GGO of both the central and peripheral lung fields. This was reported to be a probable sign of COVID-19 [ and ]. Subsequently, she underwent an emergency subtotal colectomy and ileostomy formation.
Post-operatively, due to the chest CT findings, she was managed as COVID-19 positive. Five reverse transcriptase-polymerase chain reaction (RT-PCR) COVID-19 tests, however, were negative (nose/throat swabs on Days 1 and 10, bronchoalveolar lavage samples on Days 2 and 4, endotracheal secretions on Day 13). On Day 13, due to poor clinical progression preventing successful post-operative extubation, with increasing bilateral areas of ground glass changes on CT, persistently raised inflammatory markers and ongoing pyrexia (despite broad antibiotic cover), an alternative diagnosis was sought to explain the chest CT findings. A screening for alternative respiratory infections was carried out, which detected Pneumocystis jiroveci DNA in sputum and Beta D gluten antigen in serum.
Subsequently, the patient was commenced on 21 days of co-trimoxazole treatment for PCP, resulting in significant clinical improvement and hospital discharge.


An 11-year-old female child presented with a swelling in front of neck of 1 year duration; on examination, a solitary nontender globular swelling measuring 5 cm × 4 cm was noted in the submental triangle in front of hyoid bone with well-defined borders [], smooth surface, and nontender, it moved with deglutition and with protrusion of the tongue, thyroid gland was clinically normal, there was no lymphadenopathy, and oral cavity was unremarkable. Provisional diagnosis of the thyroglossal cyst was made. Routine investigations and thyroid function test were within normal limits; ultrasound of the neck showed midline swelling in the infrahyoid region with solid and cystic components with hypoechoic mural nodule and multiple foci of calcifications; thyroid gland was unremarkable. Fine-needle aspiration cytology of the swelling showed features of papillary carcinoma. Contrast-enhanced computed tomography scan revealed a lobulated cystic lesion measuring 55 mm × 44 cm × 35 mm in the anterior neck just below the hyoid bone with solid component showing intense enhancement (170–180 HU) []. With above findings, the patient was planned for Sistrunk procedure. Intraoperatively, there was swelling measuring 5 cm × 4 cm in the submental triangle with a stalk extending to the body of hyoid bone [], and going through it, there was no infiltration of surrounding structures and no obvious lymphadenopathy. Postoperative course was uneventful, drain was removed on the postoperative day (POD) 2, and the patient was discharged on POD3. Histopathological report showed a 5.5 cm × 4 cm × 5 cm swelling with solid and cystic components with multiple small papillary projections; on gross examination, microscopic picture showed papillary carcinoma-classical type [] with lymph vascular invasion, and two lymph nodes were identified of which one showed papillary carcinomatous deposits []. After multidisciplinary discussion, the patient was advised total thyroidectomy and bilateral lymph node dissection, but her parents refused surgery; after 2 years of follow-up, the patient is clinically normal and repeat ultrasound of the neck did not show any abnormality in the thyroid or lymphadenopathy.


A 57-year-old female hepatocellular carcinoma patient underwent liver transplantation from a living donor on April 15, 2019. She had no previous medical histories relevant to allergic diseases. There were no adverse events during hospitalization. She was discharged on a postoperative day (POD) 25 and readmitted because of a fever up to 38.4°C and a headache on POD 25. Based on results of neurological examination and spinal tapping, treatment was started under suspicion of viral meningitis. For persistent neuropathic pain, carbamazepine was prescribed by a neurologist from POD 32 to POD 40. She complained of drug rashes starting from hands and feet on POD 34. There were erythematous macular patches on the trunk and extremities, especially on the palmoplantar area. Laboratory findings were: white blood cell, 4,190/μL; hemoglobin, 8.2 g/dL; platelet, 89,000/μL; eosinophil, 0%; and lymphocyte, 18.4%. The drug was discontinued on POD 41 due to suspicion of drug rash caused by carbamazepine. From POD 41, she started taking trimethoprim-sulfamethoxazole (TMP-SMX) as a prophylaxis for Pneumocystis pneumonia (PCP) ().
She suffered from diarrhea, skin rash, and itching sense on POD 99. There was a fever over 39°C. From POD 105, piperacillin/tazoperan was administered for ten days as an empirical antibiotic for cholangitis after liver transplantation. Skin biopsy was performed on POD 106 for erythematous maculopapular rash in the arms, legs, and torso. Acute GVHD was confirmed in the biopsy (). She stopped taking Tacrolimus from POD 109. Steroid dosage was increased on POD 108 as we could not rule out Tacrolimus as the cause of skin problems. Concerning that piperacillin/tazoperan might lead to eosinophilia, drug rash, and drug fever, we changed Piperacillin/Tazoperan into Levofloxacin after ten days.
A liver biopsy was performed. There was no evidence of GVHD. However, acute hepatitis with reactive hemophagocytosis due to acute viral infection and drug/toxin-induced liver injury were observed (). The rash and itching sense of the trunk were exacerbated from POD 117. The rash was a generalized maculopapular rash that started from the neck and spread from the center to the peripheral. Oral mucositis and multiple ulcerative lesions in the oral cavity appeared. There were tingling, tearing, swelling around the eyes, hyperemia, blepharitis, and dry eyes. We suspected ocular GVHD Grade 1. Thus, steroid pulse therapy was done from POD 119. We retrospectively reviewed the clinical course of this patient and suspected DRESS syndrome due to several medications. TMP-SMX was discontinued on POD 119 and tenofovir was discontinued on POD 121. Skin biopsy was retaken on POD 121 due to itching sense, multiple bullae on both dorsum of the hand, both sole, and bullous pemphigoid. The biopsy result was drug eruption with superficial perivascular lymphocytic infiltration.
Afterward, most skin lesions in the torso () and limbs improved (). However, skin detachment of the abdomen and hands progressed further and onycholysis of nails appeared (). Multiple ulcerative lesions appeared over the near-total palate, lip, and buccal mucosa (, , ). There were erosions of the vulva and perianal areas (). On POD 224, we suspected PCP pneumonia because of fever and diffuse ground-glass opacity pattern on chest computed tomography. Clindamycin and primaquine were administered for three weeks. On POD 242, tenofovir was resumed as she was a hepatitis B virus carrier. After three days, urticaria, severe pruritus, erythema, and edema occurred. These symptoms disappeared after drug cessation. There was no further occurrence after follow-up for four months.


A 77-year-old woman was initially diagnosed with unruptured cerebral aneurysms by magnetic resonance angiography. She had a family history of subarachnoid hemorrhage. The aneurysms were located at the right middle cerebral artery (MCA) and the right basilar artery-superior cerebellar artery (BA-SCA) junction. Sixteen years later, routine magnetic resonance angiography examination showed no apparent change in the BA-SCA lesion []. By contrast, the size of the MCA aneurysm had increased and the patient was admitted for further examination. Catheter angiography was performed. Angiogram indicated two MCA aneurysms with bleb formation. Thus, we scheduled neck clipping of the MCA aneurysms. However, there was no apparent aneurysm at the BA-SCA junction and the lesion was actually an extremely tortuous SCA at the anterior pontine segment []. The tortuous SCA formed a mass of arterial loops with a coil-like appearance [].


A 64-year-old man, with no significant history except lumbar surgery with foot drop and sciatica, noted difficulty in right shoulder elevation, walking, and using chopsticks for a month. Physical examination revealed hyperreflexia and motor weakness in the upper limbs (shoulder elevation 3/5, elbow flexion 4/5, and finger extension 2-/3) and slow stream of urination without sensory disturbance. Bilateral Hoffman sign and Babinski sign were positive. Despite previous lumbar surgery, foot drop progressed bilaterally without numbness or sensory disturbance, implying the presence of motor neuron disease (MND). Imaging studies revealed canal narrowing at multiple locations with ossification of the posterior longitudinal ligament and spinal cord compression with T2 signal change at C4–C5 [ and ; and ]. Paralysis progressed to shoulder elevation 2/5- and standing difficulty in a few weeks, which required emergent spinal cord decompression as MND was excluded. He had no sensory disturbance but had a previous history of lumbar surgery for progressive foot drops. Although there were no cranial nerve symptoms, patient was referred to our acquaintance neurologist to exclude spinal-onset ALS. Motor and sensory nerve conduction velocity was examined, and the neurologist diagnosed the patient to be normal and recommended reconsultation if the patient showed dysphagia or dysarthria. We were unaware that at least needle EMG was required to exclude early-stage ALS.[] Moreover, ALS is reportedly associated with a higher incidence of spondylotic myelopathy compared with normal control.[] Unfortunately, the patient could not stand at all and required prompt decompression. After the patient was explained in detail, the patient selected surgery to prevent progression of paralysis; informed consent was obtained. He underwent C3– C7 laminoplasty and right C4–C5 foraminotomy [ and ]. Diffuse fasciculation appeared on the upper arms postoperatively [] with transient deterioration of shoulder elevation 2-/5-. Intravenous administration of methylprednisolone 500 mg on the first day, which was gradually reduced to 125 mg on the third day, improved shoulder elevation to 2/5-. Clumsiness in both hands persisted, but he could walk with a T-cane. The right shoulder showed improvement in elevation to 100° during hospitalization. Based on information on fasciculation and reconsultation even without cranial nerve symptoms, the acquaintance neurologist diagnosed the patient with ALS through needle EMG. The neurologist informed us that the upper limbs’ strength of the patient slowly deteriorated subsequently and he became totally debilitated at postoperative 8 months, with dysphagia being noted at 10 months. However, he refused artificial ventilation and died at 16 months.


A 71-year-old male patient visited Yeungnam University Hospital with abnormal chest computed tomography (CT) findings on a routine medical examination. The patient was taking medications for hypertension, diabetes mellitus, and hypothyroidism and had a history of traumatic abdominal aortic aneurysm surgery 40 years previously. Chest CT revealed a 3.5-cm oval mass in the posterior mediastinum at the level of the eighth to ninth thoracic vertebrae in addition to multiple pulmonary nodules (). Magnetic resonance imaging (MRI) of the abdomen showed multiple poorly enhanced masses in the liver. The liver masses were smaller than 2 cm and emitted a low T1 signal and a high T2 signal (). We considered the possibility that the lung and liver lesions were metastatic, but blood tumor marker tests were normal. Percutaneous needle biopsy (PCNB) of the pulmonary lesion failed to yield a meaningful sample. With the possibility of neurogenic tumor or pulmonary and hepatic metastases in mind, we decided to perform surgery. We planned to resect the mediastinal tumor and biopsy the pulmonary lesion for diagnosis. We decided to determine the treatment plan for the hepatic lesions based on the surgical pathology results. We performed surgery with the patient in the left lateral decubitus position via video-assisted thoracoscopic surgery. We made 3 ports (5 mm, 10 mm, and 12 mm) using a 10-mm telescope. We identified the mediastinal tumor as observed on chest CT. The mediastinal tumor was located in the posterior mediastinum, close to the sympathetic chain and supplied by feeding vessels from the intercostal vessel. The tumor was well-demarcated without invasion into adjacent structures. After ligation and division of the feeding vessel with a hemoclip, the tumor was removed without difficulty. A pulmonary examination revealed multiple well-circumscribed nodules of different sizes, diffusely distributed on the lung surface. We performed wedge resection to obtain tissue for diagnosis. After insertion of a 28F chest tube, the operation was terminated. On gross examination, the mediastinal tumor was a 3.5-cm oval nodular mass surrounded by hardened tissue. There were 5 lung nodules in the resected lung tissue, and the cut surface showed a honeycomb-like appearance. A microscopic pathological examination revealed at both sites dilated blood vessels that varied in size and were filled with varying amounts of blood components (). A histopathological examination of the specimens, lung nodules, and mediastinal tumor revealed cavernous hemangioma. Based on the liver MRI and histopathology of the mediastinum and lung, we decided that the hepatic nodules were also likely cavernous hemangiomas and decided to investigate further. The patient’s postoperative course was uneventful. The chest tube was removed on postoperative day 3, and the patient was discharged on postoperative day 4. One year after surgery, chest CT and abdominal MRI revealed no changes in the pulmonary and hepatic lesions (). The patient is doing well without any specific clinical symptoms. The patient provided written informed consent for publication of his clinical details and images.


A healthy eleven-year-old female with bilateral clubfoot was referred to our center by a charity outreach organization. She was from a remote area in Kalimantan, Borneo, and her childhood was handicapped by this deformity. Clinically, both her feet were grossly deformed and stiff with forefoot adduction, midfoot cavus, hindfoot varus and rigid equinus (). She had adapted to walking short distances on the talar heads and the dorsum of her forefoot, resulting in callus formation over the dorsum of both talar prominences ( and ). This was a limitation for her as she was unable to attend school regularly and was homebound.
Weekly changes of nine long leg casts were done in our dedicated outpatient clubfoot plaster room. We used a bolster under the popliteal fossa. An inner cotton stockinette and soft band was applied beyond the toes then up until midthigh. Sustained manual stretching of her feet was done for five minutes with twenty seconds rest in-between stretches before applying the cast in maximum stretch. This was done before every casting, and her discomfort gradually increased when we started stretching her feet into abduction. The discomfort was temporary, occurring only during the stretching and she was comfortable after the cast had set. The foot correction was held by one person; one hand grasping the forefoot under the stockinette, the other using thenar eminence over the talar prominence (this pressure was relieved intermittently to prevent skin necrosis), while avoiding the calcaneum to allow free rotation under the talus. Plaster of Paris was then applied over the foot to mid-leg, moulding was done in the corrected foot position of the week, the distal stockinette was then pulled over just until the tips of toes were seen, and the cast was allowed to set. The foot and distal tibia were then planted on the bed and positioned in external rotation with an assistant stabilizing the knee; the bolster was removed here, and the Plaster of Paris was completed to midthigh with the knee in 45° flexion. This method of keeping the heel static on the bed holds the knee in constant flexion and allows smooth cast application without creasing the popliteal area. Following this, a roll of 4-inch fiberglass cast was applied for cast longevity, as we expected her to move for wheelchair transfers, and bottom shuffling on the floor ( and ). This outer water-resistant layer was added to the original Ponseti cast to cater to local and cultural setting.
Cast 1 and 2 were aimed at midfoot cavus correction with the forefeet casted in supination to align the hindfoot with the forefoot (2 weeks). We started forefoot abduction at cast 3 through cast 9 (7 weeks). We found we could achieve only few degrees of abduction within the patient’s tolerance each week and the best final abduction at week 9 was at 30° with the head of talus satisfactorily covered and the heels in neutral (). This achievement in abduction is notably less than the original Ponseti method in an infant. The equinus deformity was rigid and we were unable to manually stretch the Achilles tendon. She had a Z-lengthening of both Achilles tendons with posterior tibio-talar and subtalar capsulotomy to achieve 15° dorsiflexion and was then immobilized in these knee casts for six weeks to allow healing of the Achilles tendon (with wound checks at three weeks and re-casting) ().
We applied foot abduction brace () for night-time and recommended regular shoes in the daytime. She had painless range with 10° dorsiflexion, 45° plantar flexion with 20° inversion and 10° eversion at the conclusion of casting. We lost her to physical follow-up following her return to her country. By electronic communication with her carers, we discovered she was able to walk to school on uneven terrain (pebbled, mud path) six months after seeing us, and even participated in her school dance performance six months thereafter ( and ). At eighteen months post-treatment, she had picked up bicycle riding and was walking barefoot with no discomfort or pain in her feet and ankles, despite her radiographs ().


A 31-year-old woman came to the Chiangmai University Hospital having felt a lump in her right breast for ~2 months prior to her presentation. She had no nipple discharge, no nipple retraction, or no skin changes. She did not have any medical history. There was no family history of breast cancer, ovarian cancer, FAP, or GS. She had not taken oral contraceptives, and there was no history of trauma or previous surgery of the breast or chest wall.
On physical examination, breast mass or any breast abnormality could not be identified. A bilateral breast ultrasound showed no abnormal breast lesions. She was advised to conduct breast self-examinations.
Four months later, she returned with right nipple retraction, but no nipple discharge or other palpable breast mass. This time, a bilateral breast ultrasound showed an ill-defined spiculated border hypoechoic lesion (13.6×7.4×12.4 mm) in the left breast and a subareolar hypoechoic solid lesion (13.6×7.4×12.4 mm) in the right breast without internal microcalcification categorized as BIRAD V. An additional mammogram showed a hyperdense breast ().
The cytology from fine needle aspiration under ultrasound guide yielded unsatisfactory specimens from both sides; therefore, an excisional biopsy was conducted on both breasts under ultrasound marks on the skin. An intraoperative review found an infiltrating, nonencapsulated mass at both sites.
The pathology results of both lesions included some bland-looking spindle cells in collagenous stroma. Immunohistochemical studies showed positive vimentin and actin with negative desmin and CD34 that was compatible with extraabdominal desmoid tumor. Free surgical margins were noted on the right side but focally approached the margins on the left side. Since this tumor is locally aggressive, the patient was advised to choose between a widened excision or a mastectomy to reduce the recurrence rate. She selected a bilateral mastectomy with silicone implantation. Her breasts were examined every 6 months at the clinic for the recurrence of the desmoid tumor for the first 5 years and then annually thereafter. At the time of this report, she has been disease-free for 7 years.


A 28-year-old woman presented with a palpable right breast mass and breast pain that started 2 months before she presented to the hospital. There was no family history of breast cancer, ovarian cancer, FAP, or GS. She had no other illness and no history of oral contraceptive use. There was no previous breast surgery or trauma to the breast or chest wall. Her physical examination revealed a well-defined mass at 6 o’clock on the right breast, which was of firm consistency, movable, and not tender.
A breast ultrasound showed two partially spiculated border hypoechoic masses at R9 (11×7×22 mm) and R12 (6×4×7 mm) and four circumscribed hypoechoic nodule-looking benign lesions at R7 (16×11×16 mm, corresponding to the palpable lump), R10 (8×5×9 mm), L2 (6×4×6 mm), and L4 (7×3×7 mm).
Fine needle aspiration was done under ultrasound guide at R9 and R12 (the spiculated masses); the result of R9 was an unsatisfactory specimen, and the result of R12 was clear fluid. A bilateral excisional biopsy was conducted using an ultrasound marker.
The pathology result from the right breast revealed an irregular lesion consisting predominantly of dense fibrous stroma, with some benign spindle cells (1.7×0.5 mm) with focally approached margins. Immunohistochemical studies showed positive vimentin and actin with negative desmin and CD34 that was compatible with an extra-abdominal desmoid tumor. Another part of the breast indicated fibrocystic changes and fibroadenoma. The left breast pathology showed fibrocystic changes with an increased amount of fibrous stroma.
At the patient’s 6-months follow-up, she had developed new irregular hypoechoic masses in her right breast R8 (18×2 mm) and R1 (0.8×11 mm; previously reported as R12: 6×7 mm), which were suspected to be recurrent extraabdominal desmoid tumors. A right subcutaneous mastectomy was scheduled, and the pathology result showed spindle cells among dense collagenous stroma that was compatible with a desmoid tumor (1.7 cm).
Six months later, she developed a new mass in her left breast. The ultrasound showed an irregular hypoechoic mass at L11 medial to the surgical scar of 6.5 mm. This was suspected to be a recurrent fibrous (desmoid) tumor. A left subcutaneous mastectomy with silicon implantation was scheduled. The pathology result was dense fibrous stroma with some spindle cells that were compatible with extra-abdominal desmoid tumor at the previous resected area. With free margin, the immunohistochemical studies showed positive vimentin and actin with negative desmin and CD34 (–). The patient was followed up every 6 months, and at the time of this report, the disease had not recurred.


A 68-year-old woman was referred to our hospital for long-standing thrombocytopenia without other symptoms. The thrombocytopenia was resistant to steroid therapy. A splenectomy was performed at another hospital, and histiocytic sarcoma of the spleen was diagnosed histopathologically. On physical examination, bilateral axillary lymphadenopathy was observed. Laboratory tests showed a low platelet count of 77000/µL. Liver enzymes were within normal ranges. Cytological and histological analyses of bone marrow aspirates and biopsy samples showed normal bone marrow. She had no history of blood transfusion.
After admission to our hospital, routine computed tomography (CT) revealed a wedge-shaped hypoattenuating area bounded by hepatic veins in the right liver lobe (, arrow). On the arterial phase of dynamic contrast-enhanced CT, faint enhancement was seen in this area (, arrow), and most of the area was enhanced, as was the liver parenchyma on delayed phase (, arrow).
On MRI with a 1.5T system, an ill-defined hyperintense signal area was found in wedge-shaped dark signal intensity area on fat-suppressed T2-weighted images using turbo spin echo (repetition time [TR]/echo time [TE] = 2284/64 ms, echo train length = 11) (). The signal intensity of the wedge-shaped area was lower on T1-weighted in-phase images (TR/TE = 3.9/2.4 ms, flip angle = 10°) () than on T1-weighted out-of-phase images (TR/TE = 3.9/1.2 ms, flip angle = 10°) (). Thus, iron deposition was suspected. Apparent diffusion coefficient (ADC) map calculated from diffusion-weighted images with motion-probing gradients along three orthogonal axes of b-values of 0, 50, and 800 s/mm2 indicated absence of restricted diffusion in the corresponding hyperintense signal area on fat-suppressed T2-weighted images (). The surrounding wedge-shaped area had low signal intensity on the ADC map, which was considered to be due to iron deposition, based on the combination imaging findings of in-phase and out-of-phase images.
After the injection of gadolinium-ethoxybenzyl-diethylenetriamine pentaacetic acid (Gd-EOB-DTPA), a dynamic study was performed using three-dimensional gradient echo (TR/TE = 4.2/2 ms, flip angle = 9°) with fat saturation. The area corresponding hyperintensity on fat-suppressed T2-weighted images was enhanced on the arterial phase, and was surrounded by a faint enhancement in the wedge-shaped area bounded by hepatic veins (, arrowheads). A well-defined wedge-shaped decrease of Gd-EOB-DTPA was found on hepatobiliary phase 20 minutes after the injection of contrast agent (). Corresponding portal vein branches were intact on both CT and MRI.
On ultrasound, a hyperechoic lesion was located in the ill-defined hypoechoic area. An ultrasound-guided liver biopsy was performed for diagnosis, and histopathological examination demonstrated atypical tumor cells with large pleomorphic nuclei infiltrating the liver sinusoids (). Tumor cells were positive for histiocytic markers, including CD68 and CD163 and negative for CD1a, Langerin, and S-100 protein. The final histological diagnosis was histiocytic sarcoma. Iron staining was performed, and the normal hepatocytes were positive for iron staining; whereas, tumor cells showed no iron deposition.


A 12-year-old girl was referred to the Department of Oral and Maxillofacial Surgery, Seoul National University Dental Hospital (Seoul, Korea), with a six-week history of recurrent pain and swelling of the right mandible, which had increased over the past month. Under the assumption that an infection was present, the patient had been treated with antibiotics for three weeks and underwent needle aspiration for drainage at a local dental clinic.
There was prominent right facial swelling, erythema, and tenderness at the angle and inferior border of the mandible.() No cutaneous fistula was present on the first day. Several days later, a cutaneous fistula had formed. There was regional lymphadenopathy, but sensation of lower lip and chin area was intact.
On intra-oral examination, the patient had full, well-maintained dentition with erupting second molars. There were no caries lesions or periodontal pathology. No intra-oral draining sinus was found. She denied fevers or chills and difficulty swallowing or talking. The maximal inter-incisal opening was 15 mm due to guarding secondary to pain.
A panoramic radiograph was taken as an initial diagnostic study. There was overall sclerosis on the body and angle of the right mandible. There was a lytic lesion (bony crypt) on the right mandibular third molar.() A prominent proliferative periosteal reaction was present on the angle, body, and sigmoid notch area of the right mandible.()
A two-dimensional computed tomography (CT) scan with contrast material was taken for evaluation of suspected osteomyelitis. The CT image showed widening of the follicular space of the right mandibular third molar with definitive cortical perforation on the buccal and lingual sides accompanied by an outer cortical periosteal reaction.() The attenuation of bone marrow on the body of the right mandible was increased. Pus formation was observed on the buccal side of the third molar germ. Based on the clinical findings and radiographic imaging, a provisional diagnosis of chronic suppurative osteomyelitis of the mandible with proliferative periostitis was made.
Surgical management including saucerization and germectomy under general anesthesia were planned. First, the lesion was drained through the cheek, and microbial cultures were collected.() An incision was made along the external oblique ridge, and a mucoperiosteal flap was elevated. We observed destruction of alveolar bone distal to the second molar. The right mandibular third molar tooth germ was surgically extracted, followed by debridement and saucerization of the extraction site.() Histological samples were collected, and a drain was inserted for further pus drainage and left in place for two weeks.
Antibiotic treatment with a first-generation cephalosporin and clindamycin was continued for two weeks after surgical treatment. Resolution of inflammation with decreases in high-sensitivity C-reactive protein (hs-CRP) rate and swelling and increased mouth opening was achieved one week after surgery. Microbiological culture yielded only commensal organisms. The histopathology demonstrated the presence of inflammatory granulation tissue around the tooth germ. After one month of follow-up, the swelling and infection had resolved, but the bony expansion remained due to residual proliferative periostitis. After six months of follow-up, the patient exhibited restored facial symmetry with normal bony contour of the mandible on radiographic examination.


An 11-year-old female was referred from the Department of Pediatric Dentistry with progressive swelling and abscess of the right mandible.() She had presented to the hospital with the same chief complaint two weeks prior. At that time, she was prescribed amoxicillin syrup 250 mg three times a day and ibuprofen syrup 200 mg three times a day for five days with chlorhexidine mouth rinse, which were very effective and rapidly relieved her symptoms. Her symptoms returned and were then interpreted as salivary gland inflammation and pariostitis by her pediatrician four days prior, and antibiotics were again prescribed. Her swelling and trismus worsened, and she was admitted and underwent enhanced CT scanning. A course of intravenous metronidazole and amoxicillin clavulanate was prescribed. She had tender swelling over the right mandibular angle and ramus. Her mouth opening was limited to 11 mm between her incisors.
Radiological examination revealed loss of the right mandibular third molar follicular cortex with peripheral diffuse bone destruction and new periosteal bone formation and an increase in bone marrow attenuation.() Focal abscess formation was also seen.() Under the diagnosis of osteomyelitis with cellulitis and focal abscess, immediate intraoral incision and drainage of the right mandibular posterior buccal vestibule were performed.
Three days after the incision and drainage, she had symptomatically improved, and her erythrocyte sedimentation rate and hs-CRP had decreased from 51 to 30 mm/hr and from 3.95 to 0.7 mg/dL, respectively. The next day, she underwent surgical extraction of the third molar germ and saucerization.()
The histology report confirmed the diagnosis of osteomyelitis of the mandible. She was discharged three days later. After two weeks of follow-up, there was dramatic resolution of her swelling.()


The 9-year-old otherwise healthy girl presented with minimal physical clumsiness and progressive mild left hemiparesis. Magnetic resonance imaging (MRI) revealed a giant deep AVM. This lesion was considered a grade-V AVM according to Spetzler–Martin scale. Surgical treatment of the AVM was declined as it was too risky. Radiosurgical treatment was instituted due to the size and location of the AVM. She received one course of radiotherapy (20 Gr) resulting in a slight reduction in the size of the AVM. Last follow-up digital subtraction angiography (DSA) at the age of 12 showed no change in AVM [Figure and ]. At the age of 18, she admitted to our hospital with loss of consciousness and left hemiparesis (mRS score 5). A computed tomography (CT) scan revealed large intracerebral hemorrhage [] due to ruptured arteriovenous malformation.
The decision on the surgical treatment of the AVM was made with a help of endovascular embolization. Onyx embolization was admitted with minor success. Treatment continued with microsurgery. The patient was taken to the operative theater for the removal of intracerebral hematoma and resection of the AVM. During the evacuation of hematoma major intraoperative bleeding (1 L) occurred from Onyx embolized intranidal aneurysm. Bleeding was effectively held by emergency clipping of some feeders originating from the middle cerebral artery (MCA). Resection of the AVM could not be performed because of the unstable medical conditions of the patient.
Postoperatively, the patient clinical condition was the same neurologically as during admission. However, 3 days after the first operation, the patient's condition deteriorated suggesting intracranial hypertension. The patient underwent a placement of external drainage catheter with the intracranial pressure monitor. Intracranial hypertension was eliminated. Further treatment continued with a new round of endovascular modality. A new attempt of Onyx embolization through the posterior cerebral artery (PCA) was made. On the same day, the patient underwent a second microsurgical operation using the same craniotomy. We used total temporary clipping under mild hypothermia (33°C) for almost 4 hours; P2 segment of PCA, A1 segment of anterior cerebral artery, anterior choroidal artery (AChA), and the supraclinoid segment of internal carotid artery (ICA) []. Most of the lateral part of AVM underwent removal []. A postoperative angiogram demonstrated remaining medial and some of the lateral parts of AVM []. MRI with diffusion-weighted imaging (DWI) performed next day showed a lack of global ischemia []. The next day the patient underwent the third operation using the same craniotomy. Remaining part of the medial AVM was completely removed, and only a small lateral part was left.
Three weeks after the third operation, shunt for cerebrospinal fluid diversion was inserted, and the patient remained conscious with left hemiparesis. The patient was transported to the department of neurology for rehabilitation. However, few days later, new minor bleeding occurred, adjacent to the residual part of the AVM. Two embolizations were performed through MCA and PCA. Residual part filling via MCA was completely occluded, however, small residual filling via PCA remained [Figure and ].
Shunt for cerebrospinal fluid diversion was removed. Ten days later, the patient underwent the last microsurgical operation. Eventually, AVM was completely occluded [Figure –]. The patient was discharged with strong left hemiparesis, visual changes, memory difficulties, and aphasia (mRS score 4). These symptoms gradually improved, and 5 years after her initial surgery, she could walk without assistance, however, visual changes and memory difficulties remained (mRS score 3) [].


The patient was a 40-year-old previously healthy male, who was referred to a general ophthalmologist 12 hours after experiencing firework-induced ocular trauma to his left eye (oculus sinister [OS]). The right eye was normal at the time of examination. The trauma resulted in a lacerated and perforated cornea that could not be sutured, with an associated severe traumatic cataract. A spontaneous positive result was obtained in the Seidel test. The patient’s injured eye had a shallow anterior chamber and exhibited visual acuity (VA) of hand motion. Written informed consent was obtained from the patient for publication of this case report and accompanying images.
A bandage contact lens (CL) was placed, and tobramycin 0.3% (Tobrex®; Alcon Brazil, São Paulo, SP, Brazil) and moxifloxacin 0.5% (Vigamox®; Alcon Brazil) were prescribed for a period of 4 weeks, until total closure of the cornea injury and negative Seidel test (). The eyedrops prescribed were the free samples available at the general ophthalmologist clinic. The patient had no financial resources for buying eyedrops. After 6 months, the patient underwent optical penetrating keratoplasty without complications (donor: 7.75 mm, recipient: 7.50 mm). On the 40th postoperative (PO) day, the patient returned with an intraocular pressure of 28 mmHg and was prescribed brinzolamide 1% (Azopt®, Alcon Brazil). At 4 months after the operation, the cornea was clear, and two loose sutures were removed.
During the seventh PO month, phacoemulsification was performed, and an “in-the-bag” intraocular lens was implanted. On the first PO day, the eye was calm and exhibited an uncorrected VA of 20/400. However, on the fifth PO day, the patient returned with complaints of mild pain, redness, tearing, and decreased vision. An infiltrate was found around a loose suture on the graft’s temporal interface. The infiltrate was whitish, had well-defined margins, measured 2×1 mm (a slit lamp scale was used to measure the infiltrate’s size), and affected up to 50% of the stromal depth. The patient also presented with a fibrin pupillary membrane and a VA of light perception. The infiltrate was not cultured due to the lack of a laboratory in the region where the patient was being treated. The following regimen of eyedrops was prescribed empirically: Vigamox® (Alcon, Brazil) every hour; moxifloxacin 0.5% + dexamethasone 0.1% (Vigadexa®; Alcon Brazil) four times per day; and nepafenac 0.1% (Nevanac®; Alcon Brazil) and brimonidine tartrate 0.2% + timolol 0.5% (Combigan®; Allergan Brazil, Guarulhos, SP, Brazil) two times per day.
On the fifth day of infection (DOI), the patient reported improvement in symptoms, although the infiltrate remained unchanged. At this time, treatment with Vigadexa®, Nevanac®, and Combigan® was maintained. However, on the 50th DOI, the patient experienced pain and a marked decrease in VA in his OS. Examinations revealed that the infiltrate had increased to a size of 3.6×2.5 mm. In addition, central pigmentation and feathery margins were found, as well as a shallow anterior chamber and a positive Seidel test ().
A bandage CL was placed, and Vigadexa® was discontinued because the ophthalmologist wanted to observe the infiltrate’s progression without the use of antibiotics or steroids. A marked increase in temporal limbal injection, a 2 mm hypopyon, and increased density of the infiltrate at the recipient border () were observed on the 60th DOI. Ultrasonography revealed no changes in the posterior segment. Fungal infection was suspected; thus, the patient was prescribed hourly natamycin 5% (Pimaricina®; Ophthalmos, São Paulo, SP, Brazil), daily fluconazole 150 mg (Medley, Campinas, SP, Brazil), and twice-daily Vigadexa®. On the 70th DOI, the continued persistence of a positive Seidel test and increased infiltrate density and size (up to 5.4×3.8 mm) were observed. In addition, the hypopyon occupied half of the anterior chamber ().
At this time, the treatment regimen was maintained by the ophthalmologist. On the 85th DOI, the patient experienced significant reduction in redness and pain, and there was a decrease in infiltrate density and size (to 4.6×2.5 mm), negative Seidel test results, and no hypopyon (). VA of light perception persisted. The bandage CL was removed, and the eyedrops were maintained.
After 4 months of infection, the patient had no complaints of pain, although examination revealed that the infiltrate remained unchanged. Natamycin was decreased to three times per day, and fluconazole was maintained at 150 mg/d. The lack of improvement in the infection motivated the referral of the patient to an ophthalmic reference hospital. At this hospital, the possibility of a bacterial etiology was reconsidered. Therefore, empirical therapy with topical vancomycin 5% (Ophthalmos) every hour, Vigamox® four times per day, and fluorometholone acetate 0.1% (Flutinol®; Latinofarma, Cotia, SP, Brazil) once per day was initiated, and the antifungal medication was discontinued. When the patient returned 19 days later, the size of the initial infiltrate had decreased to 3.8×2.5 mm2. However, a new infiltrate measuring 2.5×1.7 mm2, forming a white opacity with the aspect of small needles and without surrounding inflammation or edema, was observed in the anterior stromal third of the graft, which made the assistant ophthalmologist think on the possibility of ICK ().
Antibacterial treatment was held due to the fact that bacteria are the most common etiology of ICK. In addition, edema was present in the inferior third of the graft. At 6 months after the onset of the infection, an increase in the size and density of the infiltrate was observed, with the infiltrate measuring 6.8×4.5 mm. At this point, the medications were discontinued. Due to the clinical appearance of the lesion and the worsening of the patient’s condition after the use of antibiotics, an antifungal regimen of natamycin 5% every hour and fluconazole 150 mg/d was restarted.
After 8 months of infection, the injury remained unchanged, and a positive Seidel test and a shallow anterior chamber were observed again. Thus, another therapeutic keratoplasty was indicated. To remove the peripheral corneal infiltrate, this keratoplasty (donor: 7.75 mm, recipient: 7.50 mm) was performed in an off-center manner, with a deviation toward the temporal limbus. The excised cornea was sent for culture. The patient continued to be treated with Flutinol® once per day, Vigamox® four times per day, Combigan® two times per day, natamycin 5% every 3 hours, and fluconazole 150 mg/d. On the sixth PO day, the patient’s uncorrected VA was 20/400. The graft was clear and exhibited no signs of active inflammation or infection (). At the first PO month, the graft remained clear, and no signs of recurrence were observed. At the second PO month, natamycin and fluconazole were discontinued, although treatment with Flutinol® once per day and Combigan® two times per day was maintained. The culture results, which were obtained after 3 months, were positive for Cladosporium sp. The final follow-up, which occurred at the sixth PO month (), revealed a clear graft, no signs of recurrence, and an uncorrected VA value of 20/200. The patient was subsequently maintained without medications.


A 54-year-old female presented to the emergency department with complaints of gingival bleeding, hematuria, and bilateral lower extremity petechial rash that began 2 days prior to admission. Her symptoms started with gross hematuria for which she was evaluated at a prompt care and started on ciprofloxacin for a presumed urinary tract infection. The following day, she experienced gingival bleeding while brushing her teeth and a petechial rash on her lower extremities, prompting her to undergo further evaluation at the emergency department. Her past medical history was significant for idiopathic thrombocytopenic purpura diagnosed in 1995, TTP and acute cerebrovascular attack diagnosed in 1997, a relapse of TTP which resulted in a splenectomy in 1999, hyperthyroidism secondary to Grave's disease, and hyperlipidemia. Her family history was not significant. Her social history was negative for any alcohol use and she formerly smoked cigarettes.
On physical exam, she was afebrile and had normal vital signs with the exception of tachycardia with a heart rate of 103. She was alert, well appearing, and neurologically intact. She did not exhibit pallor or further evidence of gingival bleeding. Her lower extremities showed petechiae bilaterally. Initial laboratory studies were significant for mild anemia and thrombocytopenia with a hemoglobin 11.5 g/dL, hematocrit 34.4%, MCV 84.5 fL, and platelet count 17×103/mcL. Her white blood cell count was normal at 11.86×103/mcL. Her LDH was 1236 U/L, her haptoglobin was <8 mg/dL, and her reticulocyte count was 2.3%. Her peripheral smear showed poikilocytosis, helmet cells, and schistocytes. Her chemistry panel and liver enzymes were unremarkable with the exception of a total bilirubin of 2.4 mg/dL and a direct bilirubin of 0.7 mg/dL. Corticosteroid therapy was promptly initiated, and hematology and nephrology were consulted for presumed TTP. Direct and indirect Coombs testing was negative. An ADAMTS13 activity level was drawn and sent for evaluation. Due to her Grave's disease, thyroid peroxidase antibody and TSH were evaluated and found to be 180 IU/mL and <0.010 mIU/L, respectively. She was asymptomatic and requested outpatient follow-up with her endocrinologist for the initiation of hyperthyroidism treatment.
Without delay, she underwent temporary dialysis catheter placement and began therapeutic plasma exchange (TPE). Her platelet counts immediately improved following her first session of TPE, and she continued daily TPE with corticosteroid therapy until her platelet count was maintained greater than 150×103/mcL (). Her ADAMTS13 activity level resulted on day 4 after the admission and was decreased at 13%. Her ADAMTS13 inhibitor was found to be positive. In total, she received 5 days of TPE and corticosteroid therapy before ceasing TPE and initiating a 6-week oral steroid taper. Her platelet count at the time of discharge was 338×103/mcL. An ADAMTS13 activity was repeated 1 week after her initial draw, which resulted as 43% activity, and again 1 month later, with a value of 56% activity ().


A 26-year-old male patient with a history of spastic cerebral palsy, shunted hydrocephalus, and epilepsy was referred for his baclofen pump refill as well as increasing discomfort from spasticity. He was being treated with baclofen administered via an intrathecal baclofen pump that had been originally placed in subfascial position more than 20 years ago. His pump had been replaced twice previously. Specifically, he had undergone one previous end-of-service pump replacement and one revision to replace a fractured catheter, and the patient had not experienced any complications with any of his prior pump refills.
Because the patient’s baclofen pump could not be palpated in the created pocket and he continued to have signs of spasticity, an X-ray was performed, during his referral appointment at the clinic. The X-ray demonstrated a subcostal location of his baclofen pump, but it wasn’t clear from the X-ray whether the pump had migrated into the peritoneal cavity or not ().
The patient was subsequently admitted and taken to the operating room for revision of his baclofen pump. Upon opening the subfascial pump pocket, we saw the pump catheter, but unexpectedly noticed that the pump had migrated. The dissection was extended along the catheter until the peritoneal cavity was entered where the 40-mL baclofen pump was found ().
We removed his pump and inspected the bowel to ensure that no perforation had occurred. On further examination, we identified a fascial tear through which the baclofen pump had migrated. This defect in the fascia was in the right lower abdominal quadrant, on the posterior aspect of the subfascial pump pocket. For this reason, a smaller (20 mL) baclofen pump was filled and inserted instead subcutaneously.
Following the removal of his migrated baclofen pump and its replacement with a subcutaneous one, the patient was discharged home on postoperative day 7 only to return on postoperative day 9 with constipation and abdominal pain. Subsequently, he was readmitted and placed on a strict bowel regimen with milk of molasses enema treatments. As his diet was slowly advanced, he was monitored for any evidence of bowel distension. Once the patient was able to have normal bowel movements and tolerate oral food intake, he was discharged home on postoperative day 12. Additionally, on discharge it was noted that the patient would later need a proximal and distal revision of the ventriculoperitoneal shunt for his worsening hydrocephalus.
Due to progression of his hydrocephalus, the patient was then readmitted for a scheduled proximal and distal revision of his ventriculoperitoneal shunt. Although patient had significant adhesions, ventriculoperitoneal shunt revision was successfully performed. The patient was observed until postoperative day 7 to exclude the possibility of his ventriculoperitoneal shunt failure and sent home in good condition following this second procedure. However, on postoperative day 22, the patient began to develop evidence of shunt failure, including headaches and increasing ventriculomegaly. The patient was subsequently readmitted and returned to the operating room for a second revision of his ventriculoperitoneal shunt. Unfortunately, approximately one month later the patient passed away suddenly from what was believed to be sudden unexpected death in epilepsy. Of note, he was being managed with anti-epileptic drugs and intrathecal baclofen has no known effects on seizure exacerbation.


A 53-year-old healthy Romanian male, without prior medical disorders, was admitted to the hospital complaining of diffuse abdominal pain for the two weeks previous to admission and subfertility. No changes in bowel habits, weight loss or gastrointestinal bleeding were declared. No chronic use of medications such non-steroidal anti-inflammatory drugs (NSAIDS) or antihypertensive substances were noted.
At the time of admission, severe abdominal pain and signs of acute abdomen, with tenderness and guarding of the abdominal wall and no intestinal sounds was found. The computed tomography (CT)-scan of the abdominal cavity indicated suspicion of peritonitis as result of perforation of the terminal ileum. Multiple skip lesions were also described in the terminal ileum and cecum. An emergency laparotomy was performed. Blood analysis showed anemia [Hemoglobin 9.6 g/dL (normal values 12-17 g/dL), hematocrit 29.8% (normal values 36%-54%)], thrombocytopenia [platelets 87000/μL (normal values 150000-450000/μL)] and leukocytosis [total leukocytes: 16 × 109/L (normal values 3.6-10 × 109/L)].
The intraoperative exploration of the abdominal cavity revealed skip perforations of the terminal ileum and diffuse peritonitis with fibrin membranes that mimicked a CD. A right hemicolectomy with a terminal ileum resection was performed. The other gastrointestinal segments did not show modifications. The postoperative status of the patient deteriorated and, despite undergoing antibiotherapy, the patient died four days after surgery. The clinical diagnosis was septic shock and bilateral bronchopneumonia.
Gross examination of the surgical specimen revealed in the terminal ileum and cecum multiple skip transverse ulcerations 1-2 cm in length with strictures and multiple perforations (Figure ).
At autopsy, diffuse peritonitis was confirmed. The rest of the gastrointestinal tract presented no modifications. In the lungs, in the upper lobes, multiple small yellowish nodules were found bilaterally (Figure ). A few nodules were also found in the liver and spleen. No lesions of the kidney, adrenal glands or bone were detected.
Histopathological examination of the surgical specimen revealed tuberculous granulomas in both terminal ileum and cecum. These granulomas presented minimal central caseous necrosis surrounded by epithelioid cells and a few Langhans giant cells. Granulomas were also found in the mesenteric lymph nodes, lung parenchyma, liver and spleen (Figure ).
Based on the macroscopically and microscopically derived findings, the final diagnosis was “Miliary TB of the lung, liver and spleen, with transmural tuberculous ileotyphlitis”. The death was established as a result of peritonitis-related septic shock.


A 72-year-old female patient presented with a history of painful swelling over the head in the right temporal region for 5 months, and fine-needle aspiration cytology (FNAC) from the swelling revealed necrotic material. Contrast-enhanced computed tomography (CECT) thorax showed mass in the upper lobe of right lung. Sputum test for acid-fast bacilli was negative. For characterization of lesions, the patient was subjected to contrast-enhanced whole body 18F fluorodeoxyglucose (18F FDG) Positron emission tomography/computed tomography (PET/CT) that showed a soft tissue mass in the upper lobe of the right lung []. Necrosis was noted in most of the volume with increased FDG uptake only in the periphery. FDG avid enhancing lesions with central hypo-attenuation were also noted in multiple skeletal muscles and the left ventricle []. Increased FDG uptake was noted in the right temporalis muscle with underlying bone destruction. 18F FLT PET/CT was also done to characterize the lung lesion that showed increased tracer uptake corresponding to the FDG uptake suggestive of active proliferation. FLT uptake was also noted in the FDG avid myocardial and skeletal muscle lesions. Subsequently, fine-needle aspiration (FNA) was done from the tracer avid periphery of the right temporal soft tissue swelling that showed metastatic well-differentiated squamous cell carcinoma. A PET-guided biopsy from the lung mass was planned, but unfortunately the patient expired after 8 days of stay in the hospital due to ventricular fibrillation, despite the resuscitative efforts. A diagnosis of primary squamous cell carcinoma of the right lung with widespread metastases was made.


A 20 year-old male patient was referred to the maxillofacial surgery department with chief complaint of a rapidly growing swelling in the left upper back tooth region associated with pain. The patient had been noticing the swelling over a period of 2 weeks.
Clinical examination revealed an expansile swelling in the maxillary tuberosity region measuring 5 cm × 3 cm []. The swelling was tender on palpation and smooth in texture. Patient had parasthesia and pain in the involved areas. Facial asymmetry was evident. However, mouth opening was normal. Clinically, there was no regional lymph node involvement. No other relevant family and medical history could be elicited. Patient was advised an orthopantamogram and a cone beam computed tomography (CBCT) scan. A typical sun ray appearance was seen in the CBCT. The tumor was found to involve the entire maxillary sinus and superiorly extending onto the inferior orbital rim. Posterior extension was seen up to the pterygoid plates and medial extension to the lateral wall of the nose [].
An incisional biopsy was confirmatory of osteosarcoma. A Weber Fergusson incision was planned for access to the tumor. Adequate access was obtained and resection of the tumor with at least 1 cm clearance was done at all the margins, and the specimen sent for histopathologic examination. Because of the extensive defect and considering the psychological status of the patient, reconstruction of the defect was planned. An anterolateral thigh flap was harvested and used for reconstruction, which also created a lining for the oral cavity []. Patient was on Ryle's tube feeding for 3 weeks. Postoperative healing was uneventful. Histopathologic report confirmed the diagnosis of osteosarcoma which was of the chondroblastic variety. The anterior, posterior, and lateral margins were found to be free of tumor. The superior, inferior, and medial margins shows tumor involvement, and hence, as per the medical oncologist's opinion, postoperative radiotherapy was started. Anterior and lateral portal radiation in combination with chemotherapy was done. Patient has been reviewed for the past 1 year and no evidence of recurrence has been noticed in clinical and radiographic evaluation [Figures and ].


In May 2013, a 72-year-old white British lady with background of chronic obstructive pulmonary disease (COPD), hypertension, prior pulmonary embolism, oesophagitis and cataracts was admitted with a diagnosis of primary osteoarthritis for an elective right TKR. Her medications included spiriva18 mcg, uniphyllin 200 mg, ramipril 1.25 mg, folic acid 5 mg, amlodipine 10 mg, ipratropium 500 mcg, omeprazole 20 mg and salbutamol inhaler and she had no medication allergy. The patient occasionally also used home oxygen therapy for COPD exacerbations. Past surgical history included epistaxis needing cautery, duodenal ulcer surgery and gastroscopy. Family history was insignificant. She was a housewife and lived alone, a previously heavy smoker but stopped one year prior to her presentation and drank alcohol occasionally. Her clinical examination confirmed tenderness over the medial and lateral knee compartments as well as the retropatellar region with a range of movement from 0-120 degrees of flexion. There were no signs of neurological deficit or vascular compromise in the lower limbs. Radiographs taken pre-operatively showed tricompartmental osteoarthritis. Her ASA grading was 3[]. The surgical procedure was performed as per the surgeon’s routine practice under a spinal/epidural anaesthetic. Patient had three doses of perioperative cefuroxime as per hospital antibiotic prophylaxis guidelines. After elevation of the limb, a pneumatic tourniquet around the upper part of the thigh was inflated to a pressure of 300 mmHg. A midline skin incision and medial parapatellar approach were utilised to expose the knee joint. Standard surgical techniques for intraoperative haemostasis were used. A cemented Columbus® TKR was implanted. After all components were cemented into place, a thorough washout with normal saline was performed and the wound was closed in layers. No surgical drain was used. The tourniquet was released after the application of dressings. Tourniquet time was 90 min. Forty mgs of clexane (enoxaparin) was given on the day of surgery with the plan to continue for 4 wk. The patient returned to the recovery unit where an anteroposterior and lateral X-rays of her right knee were performed as per departmental protocol (Figure ). After an uneventful recovery, she was transferred to the ward to undergo rehabilitation. The following day she started complaining of pain in the right lower leg which by midday became excruciating and was exacerbated by any passive movement of the toes. The patient experienced tension across the entirety of the calf as well as paraesthesia. The distal pulses in the leg were palpable but weak. A clinical diagnosis of compartment syndrome of the leg was made based on these clinical findings and a Stryker Intra-Compartmental Pressure Monitor measured pressures of 26, 32 and 42 mmHg in the anterior, lateral and posterior compartments respectively (Figure ).
Therefore she was taken immediately to the operating theatre and a decompression of all 4 compartments was performed using lateral and medial longitudinal incisions. Post fasciotomies the blood supply to the muscles which were deemed viable at the time of surgery was restored and pain was markedly relieved. The patient had to go back to theatre three times subsequently in order to completely close the wounds. The lateral incision was closed 3 d postoperatively while the medial incision was closed 3 wk later after a period of vacuum pump application.
The patient was followed up as an inpatient until complete closure of all wounds. On further examination, she was mobilizing fully weightbearing with no restrictions and achieved a range of movement from 0-95 degrees of flexion. At 12 mo follow-up appointment, the patient maintained her good functional outcome with no neurovascular deficit in the right lower limb and hence got discharged. However, in December 2014, she died from respiratory failure secondary to lung cancer. Reviewing her case notes did not show any deterioration of her knee prior to her death.
Owing to the rarity of this complication, a comprehensive literature review aiming to identify contributing factors was conducted. The exploded MeSH terms “total knee replacement” and “Compartment syndrome” were used to search MEDLINE and EMBASE databases for relevant articles in English. Two authors independently reviewed the titles and abstracts and when in doubt the full articles were retrieved and reviewed to reach consensus. Following this irrelevant studies were excluded. We included only cases where a unilateral primary TKR was performed to provide direct comparisons with the current case report. This yielded 12 studies which were relevant to our case report[,-]. A PRISMA chart with the study selection process is outlined in Figure .
The literature review showed that compartment syndrome varies with regards to its onset and implications after TKR surgery. The time period from TKR to fasciotomy ranged from 14 to 192 h (Table ). Such variation may be attributed to the masking effect of spinal/epidural anaesthesia. Eight out of 25 patients who underwent fasciotomies had no complications. However, 15 patients developed complications 8 of whom had permanent foot drop; one patient underwent an above and another patient below knee amputation. It is therefore important to intervene in a timely fashion and have a low threshold of performing a fasciotomy to prevent such serious complications. On the other hand, a recent review of 6 cases of compartment syndrome post TKR surgery showed overall high complication rates associated with treatment in this group of patients[]. In particular, 2 patients developed periprosthetic infections one of whom ended up with an above knee amputation and 2 developed foot drops. As a result, authors suggested that surgeons need to maintain a relatively higher threshold for performing a fasciotomy following TKR in comparison to trauma patients who develop a compartment syndrome. However, it is worth mentioning that in this case series, all patients not only had a spinal anaesthetic but most of them also underwent vascular procedures prior to the fasciotomies. This may have adversely affected the result of the fasciotomies which were delayed in favour of such vascular interventions. In our case, had it not been for our low threshold for surgery and high index of suspicion, our patient may have developed complications. Therefore we disagree with the suggestion of delayed intervention in compartment syndrome developing after TKR surgery and support early treatment to avoid any complications.


A 68-year-old woman presented with decrement of visual acuity of the right eye 1 year ago. Magnetic resonance (MR) imaging revealed a tuberculum sellae meningioma (). The tumor was enhanced with homogeneous intensity after administration of a contrast material. After a year, she experienced complete loss of visual acuity of the right eye without light sense. Her visual acuity on the left side also began to decrease to 0.4 and the visual field reduced severely. At this point, we undertook surgery.
The tumor was removed through a right pterional approach. The subfrontal route was mainly used to resect the tumor. After retraction of the right frontal lobe, both the tumor and the superiorly displaced right optic nerve trunks penetrated by the tumor were exposed. However, we could not recognize the penetrated optic nerve for a while. It appeared as parts of the tumor because the tumor lay in the middle of the penetrated optic nerve and the two penetrated trunks were thin (). The tumor was attached to the tuberculum sellae, which was considered as the site of origin confirming the diagnosis of a meningioma originated from the tuberculum sellae. The tumor was dissected from the adjacent cortices and nerves by maintaining arachnoid planes. The whole course of the nerve could be visualized with the removal of most of the tumor.
To confirm the penetration, the operator biopsied the bulged tumor (black arrow, ) penetrating the optic nerve. The tumor around the optic nerve was resected gross totally without tumor enhancements in postoperative MRIs (). The remaining preserved optic nerve had a penetration after tumor resection (black arrow, ). Pathological examination revealed the absence of neural tissue in the bulging tumor between the nerve trunks. No neural or glial tissue was found in the biopsy sample, and the pathologies of the main tumor mass and the bulging tumor were identical-grade I meningothelial meningioma with a Ki-67 index of 2%. Postoperative course was uneventful and blindness in the right eye was unchanged. However, visual acuity of the left eye improved slightly to 0.6 and its visual field improved within 1 month post-operation (). No additional events or symptom aggravation were noted at the 1-year follow up.


A 73-year-old lady presented to our clinic with hip pain. Six months previously she had sustained a fragility fracture of her hip whilst on holiday in Canada. She was treated locally with an uncemented hemiarthroplasty (Zimmer press fit ML taper titanium alloy stem, Zimmer Versys Cobalt-chrome femoral head and neck adapter).
The patient reported persistent groin pain and a leg length discrepancy since the time of surgery. She was listed for an EUA, aspiration and injection of local anaesthetic. She had a normal white cell count, ESR and CRP. On aspiration a turbid, green, ‘pea-soup like’ liquid was extracted. Microbiology tests were negative. With no convincing evidence for infection the suspicion of ARMD was raised. Serum Cobalt and Chromium levels were 34 and 7 nanomoles per litre, respectively. An MRI scan revealed fluid around the femoral head and neck but no evidence of soft-tissue compromise or pseudotumour formation.
The injection of local anaesthetic afforded temporary relief and confirmed the hip as the source of pain. Subsequently, the decision was made to revise the hemiarthroplasty to a total hip replacement.
Intraoperatively a large amount of the ‘pea-soup like’ fluid was again noted together with some lysis of the proximal femur. The femoral head was explanted and corrosion was seen at the head neck junction with black staining around the trunnion and on the reciprocal surface of the adapter sleeve. Multiple soft tissue samples were taken and sent for both histology and culture. The femoral stem was found to be well-fixed and after cleaning the trunnion was left in situ. The area of lysis proximally was debrided and packed with reamings taken from the acetabulum. The hemiarthroplasty was revised to a total hip replacement.
Biopsies taken intraoperatively confirmed an adverse metal reaction with an ALVAL score of 8/10 (loss of synovial lining with attached surface fibrin [], numerous perivascular lymphohistiocystic aggregates [], loss of normal tissue arrangement with the presence of acellular hyalinised zones []) (Fig. .
A year post-operatively our patient is clinically improving and her metal ions are undetectable.


A 72-year-old man with a previous history of schizophrenia, acute subdural hematoma, duodenal ulcer and paralytic ileus was referred to our hospital for PEG. He has been hospitalized in a psychiatric institution for the past 40 years and has been bed-ridden for the past 5 years due to the subdural hematoma. His oral intake gradually decreased and recurrent episodes of aspiration pneumonia prompted his attending physician to recommend PEG.
Physical examination and blood laboratory markers were unremarkable and he was tube-fed with a nasogastric tube (NGT) for 7 days before undergoing the PEG procedure. Enteral feeding via NGT was well tolerated. Endoscopic imaging before the procedure revealed a deformed duodenal bulb with moderate stenosis () and gastric ulcer scars (). PEG was performed using the push technique on the anterior wall slightly towards the greater curvature of the stomach ().
Although the procedure ended uneventfully, emesis of liquid infusion into the gastrostomy tube occurred the following day, causing aspiration, asphyxiation and cardiac arrest. Cardiopulmonary resuscitation was successful and the patient received mechanical ventilation and total parenteral nutrition until he was stable. After receiving intensive care, the patient’s condition stabilized and he was weaned from mechanical ventilation on day 70 after PEG procedure. After multidisciplinary consultation and discussion, the legal guardian chose enteral nutrition via PEG-J over total parenteral nutrition (TPN) via a central venous access port.
PEG-J was performed on day 83 (post-PEG) using a 20-Fr size all-silicone jejunal tube with gastric decompression function (Cliny PEG-J Catheter by Create Medic Co., Ltd, Yokohama, Japan). The procedure was performed in an interventional radiology suite with the use of fluoroscopy. An ultrathin endoscope was inserted into the gastric lumen through the gastrostomy puncture site after removal of the PEG tube and the PEG-J tube was placed so that the tip of the tube goes beyond the ligament of Treitz. shows an abdominal X-ray image taken after the procedure, revealing the PEG-J tube’s position after placement. The green arrowhead indicates the entry point of the PEG-J tube through the gastrostomy puncture site, the yellow arrowhead points to the radiopaque marker before the pylorus and the red arrowhead shows the tip of the PEG-J tube in the jejunum.
Tube feeding resumed in a gradual manner until gravity-controlled drip feeding was achieved. As shown in , improvement of nutritional biomarkers such as total lymphocyte count (TLC), serum cholinesterase (ChE), total cholesterol (TC), triglyceride (TG), total iron binding capacity (TIBC), total protein (TP) and albumin levels (Alb) after PEG-J procedure was observed. After achieving stable tube feeding, the patient was discharged without further complications.


A 20-year-old male presented to the casualty with a history of an injury to the back of the head as a result of a fall. He complained of severe pain in the neck and shoulder region with difficulty in raising both arms and in gripping objects. On examination, Glasgow coma score was 15/15 and the vitals were within normal limits. There was no evidence of head injury, raised intracranial pressure, respiratory distress or paradoxical breathing.
Local examinations findings revealed tenderness in the upper cervical spinal level and severe spasm of the para- spinal muscles.
On neurological examination, he had weakness of both arms, more on the right, involving the C5 to T1 distribution and brisk reflexes. There was no sensory deficit. The only other abnormality on neurological examination was brisk reflexes in the lower limbs but power was intact. There was no involvement of the cranial nerve(s).
Open mouth odontoid view radiograph and a computed tomography (CT) scan () of the cervical spine showed a type III undisplaced odontoid fracture. MRI () showed a signal abnormality in the spinal cord at the level of the cervicomedullary junction extending up to the body of C2 vertebra. The patient was treated with traction in Gardner Wells tongs for six weeks. Upon removal of the traction a sterno-occipital-mandibular immobilizer immobilizer (SOMI) brace was applied for another six weeks. His neurological deficit improved in traction and upon discharge. He had no complications from the treatment. At three-month follow-up, he had achieved full neurological recovery. MRI at 3-months follow-up showed that the lesion had regressed to a cystic lesion adjacent to the base of the odontoid.


A 49-year-old male presented with a history of bomb blast injury to his left elbow 25 years back, when he had sustained an open and comminuted fracture of left distal humerus with extension into the elbow joint. The injury was treated with wound debridement and cast application for 6 weeks. Distal humerus progressed to atrophic non-union, with severe deformity and stiffness of the elbow. He also had partial involvement of radial and ulnar nerves with stiffness and deformities of the fingers of the left hand. He presented to us for correction of the elbow deformity and to gain reasonable elbow and hand function.
On local examination, there were multiple scars present medially, laterally and posteriorly around his left elbow suggestive of healed wounds by secondary intention after compound injury. There was fixed flexion deformity of the elbow of 900 with further flexion up to 120 degree was possible (, ). There was partial sensory loss in the little and ring fingers and the muscle power (as per MRC grading) of the finger flexors was 3/5; wrist and those of elbow flexors was 2/5. The motor power of left fingers, wrist and elbow extensors were 1/5.
Plain radiographs revealed atrophic non-union of the supracondylar fracture along with the deformity at the distal humerus due to malunited distal humeral fracture (, ). CT scan confirmed these radiographic findings and demonstrated a significant gap at the fracture site with grossly rotated distal humeral condylar fragments and marked disuse osteopenia.
In view of grossly distorted anatomy of the distal humerus, poor bone stock, bone gap at the non-united fracture, severe elbow deformity and stiffness we preferred to go for a mega prosthetic replacement of the left elbow. Under general anaesthesia, elbow was approached posteriorly by ’Wadsworth approach’ and the fracture site was exposed. Severe fibrosis was encountered between the fractured ends of the bone, which was excised. The distal humeral condylar fragments were found to be grossly mal-positioned, osteoporosed, destructed and were not amenable to osteosynthesis or conventional total elbow replacement. Hence, the distal humeral bone fragments were excised, leading to a large bone gap at the elbow. This gap was reconstructed by using elbow mega prosthesis. Preparation for mega prosthetic replacement was done by reaming the humeral and ulnar shafts. Cemented titanium prosthesis (XLO Tm) was used for reconstruction, with ulnar stem of 132 mm and humeral stem of 175 mm length. The implant had eyes in the ulnar component for the attachment of the triceps tendon. Common flexor tendons were attached on the medial side and the common extensor tendons were attached on lateral side of triceps tendon and finally the combined tendinous complex was attached at the eye of the prosthesis with the help of non-absorbable polyester suture (Ethibond™ No. 5). Intra operatively, we could achieve good correction of the elbow deformity (with ROM of 10-1500) with stable elbow joint.
The patient was given i.v. third generation cephalosporin and aminoglycoside for a period of three days post operatively. The antibiotics were shifted to oral at the time of discharge and were continued for a period of two weeks till suture removal. The limb was kept in a splint for 2 weeks followed by physical therapy to regain the movements and function of the elbow and hand (, ). The post-operative recovery was satisfactory and uneventful. Post-operative X-ray showed good placement of the prosthesis with adequate cementation (, ).
At 12 months follow up, the the patient had significant improvement in the post-operative Mayo elbow score of 80 compared to pre-operative score of only 50. The patient could now do many of his activities of daily living with left hand, which were not possible before, apart from significant improvement in the cosmetic appearance.


The patient, a 28-year-old English-speaking Caucasian female, presented for acute inpatient medical hospitalization following complaints of intractable pain originating in her lower back radiating down her left leg. Upon admission, she reported a complex medical history beginning with a diagnosis of acute promyelocytic leukemia at age 15. She was treated with multiagent chemotherapy but experienced a relapse 2 years later. Following additional chemotherapy and a bone marrow transplant, she achieved another remission but relapsed again at age 19 years. This was followed by additional chemotherapy and a second transplant.
In addition, the patient was diagnosed at age 25 years with cervical cancer treated with chemoradiation.
Other notable medical concerns that the patient reported on admission included: a resolved chemotherapy-induced cardiomyopathy (ejection fraction 60%), documented human papillomavirus infection, and hypothyroidism. There was also a significant familial discord with a strong family history of substance abuse in all of her nuclear family members (opiates, cocaine, and alcohol).
In addition to her current concerns related to pain, the patient reported that at the time of admission, she was experiencing increased worry and sadness, subsequently leading to a consultation request for behavioral medicine evaluation to assess for the presence of anxiety and mood disorders, as well as for drug seeking behavior (due to the patient's strong family history of substance abuse).
Upon initial evaluation, the patient reported frustration and worry related to her chronic medical concerns, as well as ‘feeling out of control’ and ‘being tired of being sick all of the time’. She stated that her medical teams had been unable to ‘find a reason for [her] pain’, and that ‘they think it is all in [her] head’. She expanded on her current experiences endorsing: hypersomnolence, increased appetite, significant weight gain, difficulty with concentration (‘that may be my chemo brain’), psychomotor retardation, fatigue, and social withdrawal; as well as ‘panic attacks two months ago’ with the following associated symptoms: shortness of breath, racing heart, diaphoresis, tingling in extremities, and a need to escape the situation.
Throughout the initial evaluation, the patient described consistent anxiety and dysthymic mood, as well as a congruent and reactive affect punctuated by frequent tearfulness. All aspects of her cognition and speech appeared grossly intact, and she evidenced linear and logical thinking, while denying present or past perceptual disturbances or suicidal/homicidal ideation and intent. It is important to note that she stated that she ‘was always a positive person’ prior to the onset of her new medical concerns.
The patient was assigned a diagnosis of adjustment disorder with mixed anxiety and depressed mood upon ruling out medical etiologies (e.g., hypothyroidism, infection, substance withdrawal/intoxication, electrolyte abnormalities, and others), while noting her recent report of panic attacks, which were in sustained full remission.
Regarding the psychosocial aspects of her care, the patient described that she had recently moved from her country of origin to receive medical care in the United States, and that she was presently separated from all of her support systems, without her nuclear family, with whom there existed considerable relational distress. This lack of social support had compounded her experiences of anxiety and depression as she found herself bereft of her typical coping mechanisms. Specifically, her physical interactions with her peers and romantic partner had typically mitigated prior subclinical psychological distress (i.e., self-reported anxiety and sadness), although due to her separation from them, these interpersonal relationships began to develop a conflicted bidirectional relationship with the patient's psychological distress (e.g., decrease in physical proximity led to increased relational distress with her partner, which then in turn led to increased distress in the patient). Amplifying this distress were increasingly tense relationships with her family, some of whom had relapsed and had begun abusing substances. The patient reported tense relationships with frequent arguments, and that she ‘was surprised both of [her] parents were in the same room with each other’ as they had experienced a tumultuous divorce during the patient's formative years.
She denied engagement in substance abuse (i.e., tobacco abuse, illicit/licit substance abuse, and alcohol abuse) citing her experiences with family members, although she was presently receiving opiate pain medication to manage her back pain. The patient did not meet criteria for a substance abuse disorder at the time of initial evaluation.
Following discharge, the patient transitioned to the IBM outpatient clinic, where she received ongoing psychological support, although shortly after her initial evaluation, she developed hydronephrosis secondary to ureter scarring from the radiotherapy (received at age 25 years), requiring the placement of bilateral nephrostomy tubes. The patient experienced considerable complications related to her nephrostomy tubes, leading to numerous inpatient hospitalizations as a result of infection, ultimately requiring multiple surgical replacements of the nephrostomy tubes over the span of several months.
Subsequent to her concerns related to her nephrostomy tubes, the patient continued to report increased lower back pain with significant radiation throughout her lower left extremity and foot, with new-onset swelling in her left leg and a left foot drop. Upon further examination, the patient was found to have developed a deep venous thrombosis behind her left knee. During this time, the patient evidenced a considerable decrease in mood, paired with decreased functional ability (e.g., difficulty walking with frequent falls, urinary incontinence, decreased ability to engage in activities of daily living), and an increasing need for opiate pain medication.
As a result of her increasing requests for pain medication, the medical teams’ concerns shifted to focus more on the possibility of a substance use disorder, with potential malingering, conceptualizing her report of increased pain as a ploy to secure higher doses of intravenous pain medication. Conventional imaging of her lower back had been attempted, although proved unsuccessful due to the presence of significant scaring related to the patient's radiation therapy.
In addition to increased physical concerns, there had been a significant increase in relational distress within the home, which was proposed to have been linked to prolonged hospitalizations as the patient had reported ‘I like being in the hospital, I don't have to be at home then’. Additionally, she described ‘when I am sleeping I don't have to deal with anything’, and was frequently noted to be unarousable in her hospital bed, especially in close temporal proximity to the administration of pain medication.
Despite the utilization of pain medication as a coping mechanism, continuous longitudinal behavioral medicine evaluation across multiple settings reinforced the likelihood of an anatomic etiology for the patient's lower back pain, with recommendations being made to further investigate medical explanations for the pain. Factors which led to this determination included frequent and substantial behavioral observations across settings and at different time points during the treatment process (e.g., before and after surgical intervention, morning vs. evening, prior to and after receiving pain medication, inpatient and outpatient settings, and observation of gait during ambulation to and from clinic rooms), as well as consistency of patient self-report and findings of psychological evaluations over the course of 9 months.
Upon insistence of considering a potential medical etiology, the patient underwent a PET scan which indicated an increase in size and metabolic activity of a previously ruled out left retroperitoneal lesion found to be causing erosive changes in the adjacent left sacrum. Upon further assessment, the patient was assigned a diagnosis of high-grade radiation-induced sarcoma and was scheduled for palliative radiation therapy.


Patient 1 is a young woman in her 20s who presented with a systolic blood pressure (SBP) of >200 mm Hg, seizure, and right hemiparesis in the setting of thunderclap headache. She reported several weeks of worsening headaches in the setting of marijuana use. MRI showed multiple bilateral infarcts and MRA showed diffuse beading of the intracranial vessels that during angiography responded to intra-arterial injection of verapamil. A lumbar puncture was unremarkable. Following the angiogram, she was started on intravenous magnesium and nimodipine without change in her TCD velocities, which were elevated on presentation; however, velocities decreased when nimodipine was changed to verapamil, and normalized further when the extended-release form was used (see fig. ). Verapamil SR was continued on discharge.
Outcome: discharged to acute rehabilitation with apraxia of speech and hemiparesis.
Follow-up mRS: unknown.


Patient 2 is a 54-year-old woman with a history of asthma, taking phenylephrine/dextromethorphan frequently for an upper respiratory infection. She developed 1–2 days of pain above her right eye, and presented to an outside hospital with thunderclap headache and left arm weakness. MRI showed a right parietal infarct and small subarachnoid hemorrhage. CTA showed poor filling in the bilateral ACA and MCA territories consistent with RCVS. A lumbar puncture was performed to rule out infection. She was started on nimodipine and transferred for further management. TCD revealed increased velocities bilaterally, most prominent in the left MCA. At day 4 after symptom onset, nimodipine was converted to long-acting verapamil dosed twice a day with subsequent improvement in TCD velocities. Verapamil SR was continued after discharge.
Outcome: discharged to acute rehabilitation with improving strength.
Follow-up 90 days: mRS: 0.


Patient 3 is a 36-year-old man with ‘occasional headaches’ who presented with a severe headache and right-sided weakness for 1 week, found to have a subacute stroke in the left basal ganglia. Blood pressure remained difficult to control throughout his hospitalization. Conventional angiogram showed only very mild irregularity of the left ACA. A stroke work-up was unremarkable including lumbar puncture. His blood pressure was controlled and he was discharged home on an ACE inhibitor and beta-blocker. Five days later, he re-presented with recurrent thunderclap headache and SBP of 230 mm Hg with a small subdural hematoma with subarachnoid along the right frontal convexity. He was started on nicardipine. RCVS was on the differential and TCD confirmed bilateral vasospasm. Two days later, long-acting verapamil was initiated and over the next 2 days, he reported an improved pain score (7 on admission to 0), correlating with a reduction in velocities on TCD (see fig. ). He was discharged on verapamil SR.
Outcome: discharged home with improved headaches.
Follow-up 90 days: mRS: 0.


Patient 4 is a 46-year-old previously healthy woman who presented to an outside hospital with thunderclap headache, nausea and subsequent generalized tonic clonic seizure. SBP was elevated (190 mm Hg). T2-weighted abnormalities suggestive of posterior reversible vasoconstrictive syndrome were seen on MRI. Lumbar puncture showed no evidence of inflammation and she was transferred for further workup. Conventional angiogram demonstrated mild proximal stenosis of multiple large vessels consistent with RCVS. TCD demonstrated mildly increased velocities, more pronounced on the left. Fibromuscular dysplasia was also seen and found to be present in the renal vessels, a potential trigger for her blood pressure elevation. She was initially started on nifedipine XL for blood pressure control without clinical improvement. After changing to oral verapamil (long acting administered once daily) her headaches began to improve though continued to occur immediately preceding her next scheduled dose. When twice a day dosing was initiated, her pain resolved. Verapamil SR was continued as an outpatient.
Outcome: discharged home with improved headaches.
Follow-up mRS: 0.


Patient 7 is a 37-year-old woman with a history of migraines, on celexa, who presented to an outside hospital with a thunderclap headache. A head CT showed no acute hemorrhage. She declined lumbar puncture. She received a triptan, after which time she became confused. Repeat imaging demonstrated a left frontal intracerebral hemorrhage with subarachnoid component and she was transferred for further management. A conventional angiogram was performed that was read as subtle beading in multiple vascular distributions. Elevated velocities were confirmed on TCD. She was started on twice daily extended-release verapamil and began to improve; however, there was disagreement as to the underlying diagnosis and verapamil was stopped. Velocities remained stable but did not improve and she was transferred out of the critical care unit. Four days later, her examination worsened. A CTA showed diffuse spasm throughout her intracranial vasculature, and TCD showed significantly increased velocities (see fig. ). Repeat MRI showed a new stroke within the left MCA territory. Though the diagnosis of RCVS was now accepted, there remained disagreement over the best course of treatment. Due to the significant vasospasm and perfusion-dependent nature of her exam, the decision was made to augment blood pressure to maximize cerebral perfusion. Intravenous magnesium was continued. Slowly over the next couple of weeks, her velocities stabilized and she was able to be downgraded to the floor and ultimately discharged.
Outcome: discharged to an inpatient rehabilitation facility with significant aphasia, speaking nonsensically and only occasionally mimicking.
Follow-up mRS: 4.