text
stringlengths
746
31k
A 68-year-old male patient was transferred to our referral center for an abdominal ECF at the site of a mesh from a previous hernia repair. His past medical history includes coronary artery disease with coronary bypass surgery 12 years before the present events, a ventral hernia repair with mesh 22 years before the current episode, and a smoking history of approximately 3-4 pack-years.\nThe patient has been complaining of a superficial non-healing abdominal wound lasting for almost a year, which led him to consult at another hospital due to increased pain, local erythema, and fever. Imaging revealed the presence of a subcutaneous abscess (), for which large spectrum antibiotic therapy was initiated. After 2 weeks of intravenous antibiotherapy, the abscess did not resolve and enteric content started leaking from a skin opening, suggesting the progression to an ECF (). Due to subacute mild non-productive cough, the patient underwent as well a thoracic computed tomography (CT) scan that displayed bilateral cavitary nodules () which seemed to progress in size on a second thoracic CT scan performed a month later. The patient’s daily activities and occupation could not be linked to an increased risk of pulmonary infectious agents or inhalation injury, and he had no previous history of tuberculosis. Moreover, the patient complained of nasal crusting with epistaxis. Due to the complexity of the presentation, the patient was transferred to our university hospital for multidisciplinary management.\nAn infectious or a neoplastic process was suspected. Extensive microbiological investigations failed to identify a potential etiological agent at the ECF site or in the pulmonary lesions. Culture of the abdominal subcutaneous abscess revealed the expected presence of enteric pathogens, including Enterococcus sp., Enterobacter cloacae, Escherichia coli, and multiple species of Candida. No bacterial or mycotic agents were identified on bronchoalveolar lavage. The patient was thus maintained on total parenteral nutrition and on a large spectrum regimen of intravenous antibiotic therapy, consisting initially of piperacillin-tazobactam, then imipenem/cilastatin, vancomycin, and fluconazole, followed by ciprofloxacin and metronidazole and fluconazole. Antibiotic agents were based on antibiogram susceptibility tests and lasted for several weeks with no noticeable clinical improvement. The white blood count was normal since his arrival to our center (<11.0×109/L), but the C-reactive protein (CRP) remained high (>100 mg/L) and the patient had a persistent fever despite maximal antibiotic treatment. To rule out a neoplastic process, a surgical biopsy of the ECF opening was performed and revealed cutaneous ulcerations and inflammatory granulation tissue, while transbronchial lung biopsy revealed pulmonary inflammatory changes without dysplasia. The output of the fistula was estimated at approximately 2-3 liters/24 h and responded only slightly to octreotide and loperamide.\nTo confirm the etiology of the pulmonary nodules, a subsequent transthoracic biopsy was performed and revealed the presence of necrotizing damage affecting intraparenchymatous arterioles and venules, suggestive of a necrotizing vasculitis. Complementary blood analyses revealed positive proteinase 3 anti-neutrophil cytoplasmic antibody (PR3-ANCA/c-ANCA) at >8.0 antibody index and negative myeloperoxidase ANCA perinuclear p-ANCA. With negative infectious and neoplastic investigations, a diagnosis of an autoimmune disorder, specifically GPA, was highly suspected, which could have also been the cause of epistaxis and nasal crusting. No decline in kidney function, suggestive of renal damage, was noted.\nWith a high probability of an autoimmune vasculitis, and with the lack of improvement with antibiotic therapy, an immunosuppressive regimen of corticosteroid and intravenous pulse cyclophosphamide was initiated. On the initiation of this treatment, significant improvement was noted with a decrease in nasal manifestations and cough. Approximately 2 weeks after the initiation of the immunosuppressive treatment, a significant decrease in the size of the pulmonary lesions was noted, and antibiotic therapy was discontinued. Furthermore, the ECF output decreased significantly to <1 liter/24 h, and the levels of CRP normalized as well.\nAfter 2 months of immunosuppressive treatment, the patient underwent surgical excision of the ECF () with small bowel resection and anastomosis. He underwent an open cholecystectomy as well for a concomitant calculous cholecystitis, which was complicated by biliary trauma that further warranted a Roux-en-Y hepaticojejunostomy. After surgery, he developed an anastomotic leak that was controlled with intra-abdominal drainage and antibiotic therapy.\nThe patient was discharged after a hospital stay of 143 days, with close outpatient follow-up and treatment with azathioprine and decreasing doses of prednisone. No signs of ECF recurrence were noted 1 year after the episode of care.
A 63-year-old female patient in Medan, Indonesia, came to a dental clinic with a primary complaint of a lesion on the left dorsal surface of the tongue, which caused the difficulty in chewing and speaking. The lesion was of the size of a pea when the patient first noticed it but had grown to attain the size of 8 × 7 × 3 mm over a period of 2 weeks. The lesion had started to bleed when she was presented to the clinic. The patient's medical history was unremarkable. She mentioned that she previously went to a general practitioner and was prescribed amoxicillin, dexamethasone, and mefenamic acid. She consumed the medications for 5 days and reported no improvement. Intraoral examination revealed a single, spherical-shaped, and reddish pink lesion. The lesion had a distinct border and irregular surface (Figure ). The surrounding tongue mucosa appeared to be normal, and the growth was located on the left dorsal surface of the tongue. The lesion had a soft-to-firm consistency and blanched upon pressure.\nThe management of the lesion was a surgical excision, which was carried out under local anesthesia. A thread was tied around the base of the lesion. The lesion was then stretched upward to facilitate accessibility. It was subsequently excised out, and interrupted sutures were placed (Figure ). During the surgical procedure, minimal amount of bleeding from the site was observed. The specimen was then sent for a histopathological examination. After the surgery, the patient was instructed to keep the oral cavity clean by teeth and tongue brushing three times a day followed by chlorhexidine mouthwash. The patient was then asked about whether she had prior history of allergic reactions in relation to the use or consumption of beehive products such as honey, bee pollen, or propolis. She confirmed that she never had any allergic reaction to any beehive product. The patient was then instructed to also apply the commercially available hydroglyceric propolis extract to the surgical wound three times a day for a week. In addition, the patient was prescribed 500 mg mefenamic acid and instructed to consume when only the postoperative pain became unbearable. The partial healing was observed after a period of 1 week, and complete healing was observed a fortnight after the surgical procedure (Figure ). In our experience, the postoperative wound-healing period for this type of surgical intervention would usually be, on average, at least twice as long.\nThe histopathological section of the specimen showed the characteristics of an ill-defined intramuscular nodule with a pattern that was consistent with increased vascularity and inflammation. It was dominated by the appearance of epithelioid cells where the nuclei were in normal form. The predominant sub-epithelium consisted of tubular, proliferating blood vessels where the lumen was partially filled with red blood cells. The stroma was composed of fibrous connective tissue within the normal range which had a moderate amount of inflammatory white blood cells infiltrate. No signs of malignancy were found in the specimen. The histopathological diagnosis was given as lobular capillary hemangioma by a qualified pathologist (Figure ).
A 51 year old man presented to our outpatient department with an increasing swelling in the right distal upper arm. He reported about local pain without radiation. The patient´s medical history was without previous infections, surgeries or other diseases. The mass in the arm presented solid and relocatable. The examination showed full strength in all upper extremity muscles, especially in the forearm flexors, in M. pronator, M. abductor pollicis brevis, M. flexor pollcis brevis, M. opponens pollcis and Mm. lumbricales I and II. No sensory loss in the upper arm, the forearm, the palm and dorsum of the hand and the fingers could be found.\nMRI of the upper arm showed a spindle-shaped homogeneously contrast enhancing mass. It was located some centimeters above the crook of the arm within the medial sulcus bicipitalis. In the imaging it showed a relationship to the median nerve main branch of the forearm or seemed to originate from part of its fibres, respectively. Its diameter was about 11 × 4 centimeters (). The primary diagnosis from the radiologist was schwannoma.\nSurgical extirpation was indicated and performed. In its middle part the exposed tumour had a smooth capsule which was opened (). In its equator the surface had a good boundary to the surrounding tissue (). It did not extend to the muscles or tendons. In its distal and especially in its proximal ending the tumour showed a more infiltrative growth (). A feeding fascicle could be identified and was cut after ensuring by electric stimulation that it had no motor function. But with the intention to set no damage at the main nerve trunk approximately twenty percent residual tumour was left ().\nThe postoperative course was uneventful. The patient suffered a light hypesthesia in the forearm. This did not match to the supply territory of the median nerve which is the palmar hand. It rather corresponded to another skin nerve, possibly damaged by the approach. There was no new motor function deficit in the forearm flexors, in M. pronator, M. abductor pollicis brevis, M. flexor pollcis brevis, M. opponens pollcis and Mm. lumbricales I and II. A local upper arm pain vanished in the course of two weeks.\nThe final histological examination of the tumour showed typical criteria of the Castleman disease with an effaced architecture of a lymph node with regressed germinal centers and typical high endothelial venules (). Immunohistochemistry demonstrated regressed atrophic germinal centers () and aberrant network of follicular dendritic cells (). The combination of these features ensured the diagnosis.\nTo exclude a multicentric disease the patient was admitted to the internal medical department. Entire virus tests including HIV were negative. A bone marrow biopsy showed a normal hematopoiesis without evidence for an infiltration by pathologic cells. A staging PET-CT showed no further organ manifestations. An unicentric form was approved in synopsis of all findings. In regard to the tumour rest and the curative approach of an unicentric M. Castleman the patient finally underwent a selective radiation of the upper arm [].\nIn a 6-month follow-up, the partly sensory loss in the forearm had remained. Except for this, the patient had no nerve related problems or restrictions in everyday life except for the sensory loss in the forearm. In a 18-month follow up he reportet on full functionality of the arm. Currently, the area of the tumour is regularly examined with sonography.
A two-year-old boy was referred to the otolaryngology clinic by the pediatrics department for consultation regarding a suspected upper respiratory tract infection and rhinosinusitis and also to exclude the possibility of a hidden foreign body. History obtained from his mother revealed that he had purulent nasal discharge for about three weeks, with intermittent low grade fever since two weeks. This was associated with an occasional cough and the child had recently started refusing food. His parents consulted the local practitioner who prescribed antibiotics suspecting the condition to be a respiratory tract infection. However, although the fever subsided for the time being, the nasal purulence continued.\nUpon initial examination in our out-patients’ department, the child was playful, afebrile, with no apparent sign of respiratory distress, although there was copious amount of mucopurulent discharge in the nasal cavity as evident on anterior rhinoscopy. Examination of the oral cavity and oropharynx revealed postnasal drip and slight bulging of the soft palate.\nAfter interrogating the child’s mother it was made known that the child had been observed puting the cap of a small plastic bottle in his mouth about a month back. Immediately thereafter he had difficulty in swallowing and pain in his throat, but there was no paroxysm of cough or dyspnea. Several attempts were made by his mother to take it out with her fingers, but without success. However, the symptoms ameliorated soon, and with the child feeding well, no further medical attention was sought and the cap was presumed to be swallowed. It was only when the sick child was referred by the general practitioner to the pediatrics department of our institute that the clinical presentation mimicking upper airway infection was considered to be caused by a probable hidden foreign body. Leading questions to his parents further strengthened the suspicion. A formal referral to the otolaryngology department was made.\nConsidering the localized nature of the infection and mild bulging of the soft palate, we conjectured that the symptoms were more corroborative of a hidden foreign body that could have possibly been lodged in the nasopharynx. At the same time, we were also preparing for rigid esophagoscopy as the child was not feeding well. The clear lung fields on auscultation and chest x-ray and lack of suggestive history precluded us from considering a bronchial foreign body. However, a lateral view skiagram of the nasopharyngeal soft tissue revealed a radiolucent impression of some unknown round object that pushed the soft palate down (). It was presumed to be the lost cap, and we prepared for its removal.\nFollowing oro-tracheal intubation under general anesthesia, naso-endoscopy was performed and the foreign body was found in the nasopharynx, embedded between its posterior wall and the epipharyngeal surface of the soft palate. It could not be recognized initially as it was covered with granulations and discharge and it was too large to be delivered through the nose. A thin rubber catheter was passed through the left nasal cavity under endoscopic guidance and the other end was taken out from the oral cavity, retracting the soft palate anteriorly. The foreign body was now dislodged from its niche and delivered per-orally with the help of Tiley’s forceps (). It was a soft rubber cap measuring about 2 cm x 2 cm ().\nThe child was put on antibiotics for seven days along with regular nasal cleansing with isotonic normal saline. He recovered well without complications and was asymptomatic during his three month follow-up.\nThe present report has been prepared after obtaining permission from the Institutional Ethical Committee and informed consent from the child’s parents.
The 25-year-old Chinese female patient had been amenorrheic for 2 months and was referred to the hospital because of painless vaginal bleeding that had lasted for 15 days. Four years ago she had a missed miscarriage at 9 weeks of gestation, which was managed by surgical curettage. Two years prior to presentation she had delivered a baby by elective cesarean. Twenty days before presenting at the hospital she had been diagnosed as 40 days pregnant based on elevated urinary levels of beta human chorionic gonadotropin (β-hCG).\nThe study was approved by the Institutional Review Board at the First Affiliated Hospital, College of medicine, Zhejiang University. The procedures were conducted according to the principles of the Helsinki Declaration. On presentation the vaginal bleeding was scanty and her serum β-hCG level was 1200 mIU/mL. Transvaginal ultrasonography showed that the uterus measured 106 × 64 × 60 mm. There was an echo-free area above the inner cervical os, measuring 43 × 23 mm, without any blood flow signal, yolk sac or embryo present. A mixed echo mass measuring 29 × 15 mm was detected in the uterine cavity but no blood flow signal could be found in it. The patient was diagnosed as having had a missed miscarriage in the cesarean scar region of the uterus and curettage was performed with ultrasound monitoring. During the procedure, massive bleeding (~600 mL) occurred but this was stopped with an intravenous injection of oxytocin and uterine massage. Chorionic tissue was aspirated and proven as such by histopathology. When the curettage was finished, the uterine cavity was revealed as a clear thin line by ultrasound and was considered normal. Vaginal packing was performed subsequently.At 18 hours after curettage, the serum β-hCG level was 1164 mIU/mL. A cystic lesion with an uneven wall in the lower part of the uterus measuring 71 × 44 × 39 mm was detected with gray-scale ultrasonography (Figure ). Color Doppler ultrasonography showed a swirl of colors in the cystic lesion (Figure ), which was connected to an artery via a narrow neck in its posterior wall. The peak velocity in the artery was as high as 215 cm/s (Figure ).\nThe patient was examined by interventional radiologists; however, they lacked experience in treating such a giant lesion and thought that repeated treatment might be required. A persistently high serum β-hCG concentration suggested that there were retained chorionic villi present within the pseudoaneurysm. Thus, recanalization of the pseudoaneurysm by rapid recruitment of collateral vessels might occur even after arterial embolization. Repeated uterine curettage immediately after embolization, or methotrexate therapy, might decrease the likelihood of recanalization. Therefore, the patient sought admittance to an advanced institution for embolization treatment. However, while waiting to be admitted, massive bleeding occurred suddenly at day 10 and emergency surgery was performed.During the operation, a 60 × 70 × 50 mm pseudoaneurysm was located in the cesarean scar position (Figure ). Ideally, the lesion would have been completely resected and the uterus repaired after ligation of the uterine artery feeding the pseudoaneurysm. However, the hemorrhage was immediately life threatening. Therefore, to stop the bleeding quickly and save the patient’s life a hysterectomy was performed. The wall of the pseudoaneurysm was composed of clotted blood, decidual tissue and chorionic tissue.
A 54 years old man presented with ED secondary to a robotic radical prostatectomy in 2005. He had not responded to PDE5i inhibitors, intracavernosal injection therapy with PGE1 or vacuum therapy. He had a history of hypertension, diabetes, and chronic renal failure, all of which were well controlled. He also was incontinent, needing 4 pads per day and so initially underwent the placement of an artificial urinary sphincter (AUS) in 2008. This proceeded uneventfully with the balloon of the AUS placed in the right iliac fossa.\nTwo years later he had the insertion of an inflatable penile prosthesis (AMS 700 CX) using a 100 mL reservoir placed in the left retropubic space using a blind puncture through the external ring. He made an unremarkable post-operative recovery and was discharged with the implant deflated to be reviewed in clinic. Over the following 3 months he had problems inflating the device due to a “sticky pump” and pain in the right iliac fossa. To exclude a device leak an magnetic resonance imaging was ordered which showed the reservoir to be full and centrally placed within the pelvis (Fig. A).\nThe patient was then offered an exchange of pump but decided instead to have the device replaced with a malleable penile implant which was performed after an additional 3 months. The cylinders and pump were removed and a malleable Genesis implant inserted after a mini salvage washout. The reservoir was emptied and retained after the tubing was cut high. At follow up the malleable implant was excellently sited but the patient continued to experience right abdominal pain. An abdominal CT scan was ordered which revealed that the reservoir had migrated into the abdomen and had become wrapped around the caecum (Fig. B). Removal of the reservoir was planned initially by a laparoscopic approach although due to adhesions it was difficult to identify it. An open incision was then made in the right iliac fossa but the reservoir could not be found. A second midline upper abdominal incision was made and the reservoir retrieved for the left upper quadrant. A speedy recovery was made, the right abdominal pain resolved and the patient was happily having sexual intercourse.
A 56 years old healthy male patient was admitted to the Urology department for elective right inguinal hernia reparation (Table ). The urologists performed a standard operation of a sliding inguinal hernia on the right side. Due to the weakness of the lower AW, the urologist reinforced the inguinal wall with synthetic Prolene mesh. Postoperatively, the patient showed a clinical picture of an acute abdomen. At this point, the urologists performed a revision surgery of the operated inguinal hernia, during which they found only a hematoma, removed the Prolen mesh and performed adequate haemostasis. Unfortunately they did not notice the bowel perforation and did not perform an explorative laparotomy at that time. During the next 24 hours, signs of septic shock with crepitations on the AW and right flank region appeared in the clinical picture. Through the suture line of the inguinal canal a fecal collection was drained. Postoperatively, the patient received a combination of Penicillin G, Clindamycin, Metronidazol and Gentamycin. The native abdomen x-ray showed air under the diaphragm. Magnetic resonance images provided dramatic evidence of an inflammatory process infiltrating the deep fascial plane of the anterior AW. Systemic manifestations of SIRS with body temperature more than 39°C, heart rate more than 100 beats per minute, breaths less than 30 per minute, PaCO2 less than 32 mmHg and WBC account more than 18 × 109/L with a high number of immature forms, hypotension, hypoperfusion with a high level lactic acidosis, oliguria, and alteration of mental status and consciousness were indicators of severe sepsis and septic shock. The anesthesiologist introduced a central venous catheter and started intensive resuscitation.\nThe abdominal rigidity suggested a persisting peritonitis and an urgent laparotomy was done. Through a long midline incision we found a perforation of the caecum, necrosis of a great part of ascending colon, diffuse fecal peritonitis and signs of retroperitoneal NF. The surgical team executed extensive debridement, fasciectomy of the deep fascia on the AW, right orciectomy, right hemicolectomy, diverting colostomy on the descending colon and extensive debridement of the RS. The abdominal cavity and RS were extensively irrigated with hydrogen peroxide, saline and a solution of 1% povidone iodine, and drained on both sides. The structural and functional continuity of musculofascial system of the AW was obtained by component separation techniques (cite) and biological mesh. The wound was dressed with 1% povidone iodine solution. Dressing was controlled every 24 hours and serial debridements were performed. On the second postoperative day, the surgical team ordered adjuvant HBO therapy during the next 10 days. On the first day, the patient received two treatments of HBO therapy, followed by one treatment per day. HBO was given at 2.8 ATA for 90 minutes per day. In this case we needed five serial debridements to stabilize the wound. The results of microbiological analysis of the lower AW and retroperitoneal space showed a polymicrobial infection with Escerichia coli, Psudomonas aeruginosa, and Streptococcus fecalis, Streptococcus pyogenes, and the presence of mixed anaerobes, including Bacteroides fragilis and Clostridum spp. Blood cultures were positive for Escerichia coli and Pseudomonas aeruginosa. Methicillin-resistant Staphylococcus aureus (MRSA) was present in the second blood culture. Two weeks after the initial operation, the AW became stable and fresh granulation tissue appeared. At that point, we started closing the defects by using local advancement flaps, regenerative tissue matrix, and skin grafts. The closure of the diverting colostomy was performed three months postoperatively when the anterior abdominal has been strongly reinforced with a dermal matrix that was incorporated under the skin flaps. During long term follow up the colostomy was completely closed and regular bowel function was restored.
A 33-year-old healthy woman with a 1-year history of progressive sensory change on the left hand, visited the Department of Physical Medicine and Rehabilitation August 30, 2017. She had received bilateral open carpal tunnel release operation May 24, 2017, at a private orthopedic clinic. Tingling sensation and hypoesthesia of the thumb and index finger were relieved after surgery, but symptoms of the middle and ring fingers persisted and were aggravated. On physical examination, all deep tendon reflexes were normoactive in bilateral upper extremities, and no muscle wasting was noted including hand intrinsic muscles. Muscle strength was normal in bilateral upper extremities, including thumb abduction and opposition. Hypoesthesia was noted on the ulnar side of middle finger, and the radial side of ring finger, on the palmar side of the left hand. Tinel’s sign was positive, with proximal wrist crease tapping.\nAn electrodiagnostic study was performed September 5, 2017. On NCS, left median compound muscle action potential was within normal range. Left antidromic median sensory response with the middle finger recording was of low amplitude, compared to the sound side. To eliminate the possibility of volume conducted response, the orthodromic sensory conduction study was performed (). Orthodromically conducted sensory response was unobtainable at the wrist with ulnar side of middle finger and radial side of ring finger stimulation. But the response was normal, with the radial side of middle finger stimulation (, ). Needle electromyogram revealed large amplitude and long duration motor unit action potential, with reduced recruitment patterns in the left abductor pollicis brevis muscle. Normal motor unit potential was observed in all other muscles examined (). The patient was diagnosed with sensory neuropathy of palmar digital nerve of the left median nerve, supplying the ulnar side of the middle finger and radial side of the ring finger.\nUltrasonography was performed on the same day as the electrodiagnostic study. No abnormal findings such as nerve swelling and impingement were found at the palm and carpal tunnel. However, compression and swelling of the median nerve with fluid collection were noted at the distal wrist crease. Considering the above findings, compression of common digital branch of the median nerve at distal wrist crease was suspected ().\nThe patient underwent re-operation of open left carpal tunnel release October 26, 2017. Interestingly, a branching site of common digital nerves of the median nerve was identified not at the palm, but at a far proximal site around the distal wrist crease (). In addition, a traumatic neuroma approximately 0.5×0.5 cm was identified, on just the proximal site to the divergence of the median nerve. Neuroma excision and microscope-assisted direct end to end neurorrhaphy were performed. Severe tenosynovitis around flexor tendons at the left carpal tunnel was also identified, and tenosynovectomy of flexor tendons was performed.\nSix months after surgery, pain on the left middle and ring fingers reduced by 70%. A follow-up electrodiagnostic study was performed April 10, 2018 (, ). Previously unobtainable orthodromically conducted sensory responses, were detectable with low amplitudes. Aside from that, the left median sensory nerve action potentials (SNAPs) decreased in antidromic response with the middle finger recording, and orthodromically recorded sensory response in the radial side of the middle finger.
A 27-year-old male sustained penetrating trauma to his right eye as a child at 7 years of age, but unlike the other two cases had undergone primary repair. He developed sympathetic ophthalmia a few years later, and this was managed with oral steroids. He had also undergone cataract extraction to both eyes and used contact lenses to correct his aphakia. Like our other two cases, he too had undergone superficial keratectomy for band keratopathy in the sympathising eye. He was then offered and underwent a corneal graft, to the exciting right eye. This was performed under immunosuppressive cover of mycophenolate 1 g twice a day along with 40 mg of oral steroids which was then reduced to the dose 500 mg once a day and oral prednisolone 5 mg once a day. He developed glaucoma in his right eye, one year from the time of the graft and is on topical with brinzolamide and latanoprost. On examination, one year from the time of right corneal graft procedure, his BCVA was 3/60 in his right eye and 1/60 in his left eye. Anterior segment examination showed a clear right corneal graft and corneal degenerative changes in the form of band keratopathy and corneal decompensation in his left eye (). Ultrasound examination showed the retina to be attached and a single flash ERG showed a normal retinal response in his left eye. Penetrating keratoplasty was performed on the sympathizing eye, with the donor graft size of 7.50 mm and the host was 7.25 mm. Postoperatively the mycophenolate was increased to 1 g twice a day and was also on 30 mg oral prednisolone which was then tapered by 5 mg per week, and he is on the maintainance dose of 5 mg. He was also on topical steroids and antibiotics. At 10 months follow-up his BCVA was 6/36 and his graft was clear (). Fundus examination showed peripheral chorioretinal atrophy with scarring at the macula ().
A 25-year-old female, educated up to 10th std, unmarried, 22 weeks gravida came to OPD with complaints of gradually progressive low mood, decreased interest in daily activities, feelings of hopelessness, worthlessness, sleep disturbance, and loss of appetite over the span of 4 years with increased intensity for the past 6 months. The patient reported that when she was 17 years old, her stepfather tried to molest her sexually. She was able to save herself by running out of the house. Later, her father threatened her not to tell anyone about the incident with which she got frightened. A month later, her stepfather again tried to force her to engage in sexual activity which she resisted. She complained to her mother when she returned from work, but her mother did not believe her and thought that she was lying. After a month, due to frequent quarrels in the family, she started feeling low. The symptoms increased to such an extent that she had to leave her job. Then by mutual decision, her parents asked her to live separately. The patient shifted with one of her friends who was residing in a rented flat. She found another job where she met a man. They frequently met each other and fell in love with each other. During this period, the patient started feeling well and regained hope of a better and happy life in future. After around 1 year of relationship, she became pregnant. On being informed of this, her boyfriend refused to have anything to do with the child. He even left the job and stopped all communication with her. After this, the patient decided that she will give birth to the child without any support and look after her child on her own. Gradually she started having low mood, began taking frequent leave from job, and used to sit alone at home and do nothing. She had disturbed sleep and poor appetite. She felt hopeless about her future and inability to support her child. On MSE, the patient appeared sad with teary eyes. Talk was relevant and coherent. The mood was depressed; there was no suicidal ideation. She was diagnosed with a case of recurrent depressive disorder. She responded satisfactorily to pharmacological treatment and cognitive behavior therapy and delivered a healthy male child.
A 64-year-old gentleman with a past medical history significant for EGPA presented to our gastroenterology clinic after recurrent episodes of partial small bowel obstruction within a period of 6 weeks. The diagnosis of EGPA had been done 12 years before when he presented with asthma exacerbation associated with extremity petechiae, purpura and digital infarcts as well as amaurosis and temporal artery swelling. Two years previous to his presentation, the patient had several times undergone endoscopic retrograde cholangiopancreatography (ERCP) for recurrent choledocholithiasis with associated cholangitis. Biopsies had shown acute and chronic inflammation with reactive epithelial changes presumably secondary to stones. The vasculitis had been stable on methotrexate and prednisone with serial stable inflammatory markers and absolute eosinophils prompting a reduction in immune suppression 3 months prior to presentation.\nOn presentation, the patient was having recurrent, episodic abdominal pain associated with intermittent abdominal distention and new lower extremity purpuric lesions. Physical examination was otherwise within normal limits. Elevated inflammatory markers and eosinophilia of 0.69 × 109/l (13.5%) were detected. Magnetic resonance enterography was performed to further evaluate the cause of the recurrent small bowel obstruction. It showed bowel wall thickening in the distal small bowel, with mechanical small bowel obstruction proximal to the ileocecal valve (fig. ). Given his history of recurrent cholangitis, ERCP with cholangiogram was performed which showed moderate dilation of the biliary tree. Cholangioscopy showed a nodular common bile duct, with one stone in the left intrahepatic duct which was successfully removed. Common bile duct biopsies showed eosinophils and mononuclear cells with some associated fibrosis (fig. ).\nExtended enteroscopy was performed to evaluate the upper GI tract. Erythematous mucosa in the gastric fundus and body, nodular mucosa in the gastric antrum and multiple small duodenal polyps were identified. The esophagus, the remaining duodenum and the jejunum were normal. Biopsies of the normal and diseased mucosa showed a diffuse dense inflammatory infiltrate in the lamina propria including mononuclear cells and eosinophils (>50 eosinophils per high-power field). On immunohistochemistry, T and B cells showed normal immunophenotype, and in situ hybridization for Epstein-Barr virus was negative, findings that ruled out lymphoma.\nRetrograde balloon-assisted enteroscopy showed congested and thickened folds of the mucosa in the distal ileum biopsies with normal colon. Pathology showed blunted villi, with an inflammatory expansion of the lamina propria including the presence of increased eosinophils (fig. ). Table summarizes the macroscopic and pathologic findings.\nMagnetic resonance angiography was performed and no signs of significant mesenteric artery stenosis or other vascular abnormality were identified. All findings were consistent with Churg-Strauss syndrome involving the GI tract even though no vasculitis was shown on pathology. Although surgery of the ileal stricture was considered, medical management with increased dose of methotrexate and prednisone was elected. Unfortunately, after 2 months of being totally asymptomatic on higher-dose immune suppression the patient experienced a syncopal event and died. Clinically, this was attributed to known pre-existing heart disease likely due to EGPA involvement.
A healthy 18-year-old male patient attended a department of periodontics in a large teaching hospital, with a chief complaint of gingival swelling producing moderate discomfort for about one week, which was located in the mandibular right buccal region.\nClinical examination revealed obvious gingival swelling over the buccal region of tooth 46 (). The tooth was mildly uncomfortable to percussion with a normal response to thermal testing and no any apparent occlusal problems. The probing depths for all sites in the dentition did not exceed 3 mm except for a 10-mm pocket in the buccal furcation of tooth 46 (). However, an obvious worn-down abnormal central cusp was found on the occlusal central fossa of tooth 45, which had obvious discomfort on percussion, no response to thermal testing, and no mobility. The radiograph showed a well-defined round radiolucent lesion approximately 14 × 15 mm in diameter surrounded by a radiopaque line, around the root apex of tooth 45 and with a radiopaque margin involving the apical third of the mesial root of tooth 46 (). A small area of radiolucency was also present in the furcation of tooth 46 (). No obvious radiolucent lesions were seen elsewhere in the dentition. Based on the clinical history and clinical and radiographic examination, an initial diagnosis of a periapical cyst of tooth 45 accompanied by acute abscess draining through the periodontal ligament of tooth 46 was made.\nInitially, tooth 45 was treated with routine endodontic therapy. Both conservative and surgical treatment options were explained to the patient and he insisted on the surgical one for his limited time. A consent form was signed by the patient and the surgery was scheduled for the next visit.\nAt the second visit, periapical surgery was done. After standard disinfection, the operative region was anesthetized by block injection of Lidocaine HCl and infiltration injection of Primacaine Adrenaline (articaine hydrochloride with epinephrine tartrate injection). A relieving vertical incision was made on the distobuccal axial angle of tooth 44 and then a crevicular incision was made from tooth 45 to 46 along the buccal gingival sulcus. A full-thickness mucoperiosteal flap was reflected. The tissue of cystiform around tooth 45 was enucleated and then a 15 × 12 × 8 mm osseous destruction region was exposed. Further inspection found that the osseous destruction around the root apex of tooth 45 penetrated through periodontal ligament of the mesial root and involved the furcation area of tooth 46 (Figures and ). Then, root resection and root-end filling of tooth 45 were done and granulation tissue was removed thoroughly. After the surgical region was rinsed with normal saline, the bony wall of the cavity was scratched until it was filled with blood, and then Bio-Oss Collagen was placed into the bony destruction of teeth 45 and 46 (), as has been previously suggested []. Finally, the mucoperiosteal flap was repositioned and the surgical region was closed with 4-0 vicryl sutures. A periapical radiograph of teeth 45 and 46 was taken to observe and record the immediate postoperative situation (). After the procedure, the patient was advised to take amoxicillin (1500 mg per day) and ornidazole (1000 mg per day) orally for five days and gargle with 0.12% chlorhexidine gluconate for one week. A week later, the patient returned for postoperative examination and suture removal.\nAt 3 and 7 months of recall, clinical and radiographic examinations were performed to evaluate the healing status. At 3 months, oral examination revealed good healing of soft tissues and the furcal probing depth of tooth 46 had lessened to 4 mm (). A radiograph revealed a decrease in the size of the periapical radiolucency to 13 × 14 mm in diameter and some material implanted seemed to be absorbed (). At 7 months, the soft tissues were in healthy condition and the furcal probing depth of tooth 46 was about 2 mm (). A radiograph showed an apparent decrease in the size of the radiolucent area to 4 × 5 mm in diameter (). Additionally, tooth 45 had normal response to thermal testing at all appointments.
A 69-year old female patient, with a previous history of hypertension, thyroidectomy due to hyperthyroidism and hysterectomy for uterus myomas, presented with a progressive swelling of the dorsal aspect of the right side of her neck without signs of vascular obstruction or venous stases. No abnormalities of neural status of the head and neck were observed. There was no functional or sensory loss of the right upper extremity. No signs of Horner's syndrome, dysphagia, cough or dyspnoe were evident. CT scan demonstrated a retroclavicular soft tissue tumour with a cranio-caudal extension of up to 4.5 cm which partially displaced the trachea to the left and compressed the subclavian vein. An adjacent tumour of dimensions 3.5 × 3.5 cm not clearly separated from the before mentioned tumour was located at the inferior right thyroid lobe, compressing the internal jugular vein. Near the confluence of these vessels a subtotal occlusion of the brachiocephalic vein is revealed (Fig. ). The MRI scan added no further information on the origin of the tumour or the cause of venous occlusion. There were no clear signs of tumour infiltration of the brachial plexus, brachial artery, esophagus or trachea. The preoperative chest x-ray displayed a right sided upper mediastinal enlargement (Fig. ). Additional venous angiography indicated a filiform stenosis of the subclavian vein. Within the brachicephalic vein a longitudinal, irregular partial displacement of the vascular lumen was depicted. Extensive blood flow in cervical and supraclavicular collateral vessels was present. Neither MRI, CT nor angiogram allowed for clear distinction of the intravascular process whether it was caused by intravascular tumour growth or thrombosis. Incisional biopsy one month prior to the oncological tumour resection revealed the histopathological diagnosis of a leiomyosarcoma.\nSurgical exposure was obtained via a triangular incision running from behind the right ear, along the anterior axillary line and across the sternum. First, the brachial plexus was dissected, the phrenic and recurrent nerves identified and followed distally. The upper border of the tumour became visible at the upper thoracic aperture. The recurrent nerve was observed to run through the tumour capsule. Further preparation was carried out from the distal edge of the wound. The pectoralis major muscle was elevated and care was taken to preserve the vascular pedicle (thoracoacromial A.V.). It was further observed that the first intercostal space was invaded by the tumour. Subsequently a thoracic wall resection including a partial resection of the right clavicle, the right half of the sternum and the costal attachment of the first three ribs was performed uncovering the mediastinum. The vena cava was revealed and trachea dissected. In this area the tumour was in close proximity to the trachea displacing it to the left but without tracheal infiltration. Next, the carotic artery and the jugular vein were exposed.\nThe tumour, located in the right supraclavicular region/upper mediastinum, was found to surround both the subclavian and the internal and external jugular vein. Hence a resection of the subclavian vein proximal to its conjunction with the superior vena cava was required. The internal as well as the external jugular vein were incorporated into the tumour conglomerate (Fig. ). The tumour was resected en bloc. A partial resection of the clavicle, partial resection of the sternum with removal of the brachiocephalic, sublcavian and right jugular vein and the recurrent nerve was necessary to obtain clear resection margins. The defect coverage was achieved by a pedicled myocutaneous pectoralis major island flap.\nWithin the surgical specimen multiple nodular polypoid tumour masses of soft consistence with diameters of up to 3.6 cm, immediately adjacent to vascular structures of the subclavian, internal jugular and brachiocephalic vein were present. The tumour with its intravascular and extravascular components comprised a total area of 7.6 × 8 × 3.3 cm. The largest intravascular tumour sprout extended close to the resection surface of the vessel.\nThe macroscopic appearance resembled an intravascular tumour originating from the subclavian vein with infiltration of extravascular structures.\nMicroscopically the spindle-shaped cells of this mesenchymal neoplasm originated from the media of the venous vessel wall (Fig. ). The tumour cells formed various fascicles interwoven with other longitudinal cross sectional neighbouring fascicles (Fig. ). The tumour cells were characterized by an eosinophilic cytoplasm and cigar shaped nuclei. The mitotic rate was 19/10 HPF (per high power field). Some foci of tumour necrosis were present.\nThe neoplasm derived from the media of the vessel wall, disrupted the existing vascular architecture and formed an intravascular tumour sprout.\nImmunohistochemically the majority of tumour cells were positive for smooth muscle actin and desmin. A positive reaction for the proliferation marker Ki 67 was found in 25% of all tumour cells,\nThus confirming the diagnosis of an intravascular leiomyosarcoma (malignancy grading GII)\nPostoperatively only mild signs of mixed venous and lymphatic stases of the upper extremity following the resection of the subclavian vein were observed due to the well established collateral blood flow (as seen in the preoperative angiogram). These symptoms could be positively influenced by elastic compression dressings and physical lymph drainage. Owing to the resection of the right recurrent nerve, right sided vocal cord palsy occurred. Logopaedic training was initiated. The patient recovered well and was discharged two weeks later. Both pre- and post-operatively no symptoms of pulmonary embolism were detected.\nUnfortunately the patient declined the recommended radiation therapy.\nAfter an initial 5 month of tumour free survival without evident signs of either local or systemic metastasis a tumour relapse was detected. At this stage the patient refused further treatment apart from a palliative chemotherapy.
An 8-year-old girl fell from a height of around 1.5 m and injured her left elbow. She was examined by a physician in her village and diagnosed as having an elbow sprain for which limb was immobilized in a plaster cast for 3 weeks. The child sustained another injury to the same elbow following fall on her outstretched hand 4 weeks after removal of the cast. Again the elbow was immobilized in a long arm cast for 3 weeks. After removal of the cast, the child complained of persistent pain and discomfort in the elbow. She was then referred to our hospital 3 months after the initial injury.\nExamination revealed a stiff elbow with a painless range of motion from 45° to 90°, with only terminal limitation of pronation and supination. The ununited fragment was palpable separately from the rest of the distal humerus and freely mobile. Valgus instability was also noted. There were no signs of ulnar nerve irritability or deficit. An anteroposterior and lateral radiographs revealed a displaced fragment of the medial condyle involving the trochlea ().\nTo address the limitation of movement, articular incongruity and elbow instability, open reduction, and fixation of the fracture was planned. After obtaining consent from the parents, the patient was administered general anesthesia and positioned supine on the operating table with arm abducted on arm board. Limb was exsanguinated and surgery was commenced under tourniquet. Incision was made directly over the medial condyle fragment. The ulnar nerve was identified and protected with plastic tube. The medial condyle fragment was found displaced proximally and anteriorly. The fragment was rotated with its cancellous surface lying anteriorly. This surface was freshened with a curette. Fracture surface on the humeral side was identified and freshened. Medial articular edge of distal humerus was identified for accurate reduction. Without any undue soft tissue stripping, the fractured fragment was reduced to the best possible position with approximation of articular surfaces. Fixation with three smooth Kirschner (K) wires was done followed by suturing of surgical wound. Long arm cast was applied with elbow at 90° and forearm in mid-prone position. There were no post-operative complications.\nConsidering that the fracture was already 3 months old at the time of surgery, we anticipated that a longer duration of immobilization would be required; hence, the cast and K-wires were removed at 6 weeks under local anesthesia. Active and active-assisted elbow range of motion exercises were started after K-wire removal. Radiographs taken at 3-month follow-up show union of the fracture, with an irregular medial condyle. The patient regained an active flexion arc of 25° to 95°, with complete pronation and supination (). Passively 20° to 100° arc of motion had been restored. At 6-month follow-up range from 10° to 120° had been restored and there was no coronal plane deformity.
A 52-year-old right handed female presented with a chief complaint of left arm and left hand pain. The patient's symptoms began after a phlebotomist had a difficult time obtaining blood draws from the patient's left antecubital region for a cholesterol test. The patient described that immediately following the needle insertion it felt like the patient was being “electrocuted.” A few days later the patient went to the doctor due to this ongoing pain. The patient developed a shooting, burning, and constant neuropathic pain. The pain was originally in the left thumb finger and gradually extended to the indicis and middle finger with the relatively rapid expansion of the entire hand up to the wrist and sharp radiating pain distally from the elbow. The pain was somewhat diffused and was most sensitive along all the dermatomes below the elbow. Due to the continuation of her pain and with no benefit from initial pain medications regimen, her pain specialists decided to do stellate ganglion block, epidural steroid injections, along with physical therapy, occupational therapy, and multiple combined pain medications. She never experienced a tolerable pain level during the use of her medications even in the highest permitted dose. The patient had tried gabapentin, tramadol, baclofen, amitriptyline, hydrocodone, nucynta, savella, and lyrica, as well as lidoderm patches for her symptoms.\nThe patient presented to the pain clinic with pain and rated the pain as 8 out of 10 on visual analog scale and stated that the pain was perceived constantly around moderate to severe. The patient reported a constant warm and burning sensation. The patient had difficulty falling asleep, as well as interrupted sleep due to change of position and inadvertent pressure on the left arm. The patient is right handed but due to pain was able to use the left arm for daily activities.\nThe patient did not complain of any associated neurological symptoms. However, the patient stated that she has become weaker in her left upper extremity secondary to the lack of use from the pain. The patient's other symptoms are associated with redness, bluish discoloration, temperature disparity amongst contralateral upper extremities, and allodynia.\nAfter one year since the inciting event and trial of multimodality treatment we decided to try dorsal spinal neuromodulation. During seven days of a spinal cord stimulation trial, the patient was able to perform many daily activities with reasonable pain control. The patient reported the pain between 2 and 3 on the visual analog scale during the trial period. The patient was deemed to be a candidate for permanent implantation of dorsal column stimulator. The patient underwent the implantation of 8-contact-compact-lead percutaneously sensor rechargeable battery (2 × 8 octad leads, model #3778-75 Medtronic Co.).\nOn a six-month follow-up, the patient reported left foot pain and discoloration. The patient's CRPS symptoms had now spread to involve the right lower extremity. We administered an aggressive course of physical therapy with desensitization techniques along with occasional lumbar sympathetic blocks and pain behavioral modifications techniques with multimodal medication management. The symptoms remained unresponsive and had been refractory to the multimodality management. The patient reported constant pain in the right leg with severity of 8 on visual analog scale. Gradually skin discoloration and nail changes along with hair loss on the right foot created a clear picture of CRPS. Interestingly, the patient reported great pain relief on the upper extremity by using a cervical spine stimulator. The patient was able to use the upper extremity for daily activities. The patient's right leg pain was greatly disabling and due to the unresponsive CRPS symptoms we decided to proceed with thoracic dorsal column stimulator placement after the patient demonstrated a great response to 7 days of the trial. A thoracic spinal cord stimulation implant was done via percutaneous placement of 2 × 8 octad leads (Medtronic Co.) (see ()).\nWe did six-month follow-up after the patient's last surgery. The patient was 100% satisfied with the pain management result. We interrogated the spinal cord device. The patient was able to manage to go back to her school job using neuromodulation 100% of the time along with moderate consumption of gabapentin and tramadol. The patient rated her pain severity at both upper and lower extremities at a 2 on visual analog scale.
We report a case of a 58-year-old Caucasian male with no significant past medical history and also had no regular follow-up, came with slowly growing right sided inguinal mass. He did not remember any explanation or precipitating event to the swelling and he did not remember when swelling started to exist exactly but approximately over weeks. His surgical history included tonsillectomy. He was smoking for 40 years. He used to work in an office but recently retired and was living with family. On evaluation, there was no history of constitutional symptoms like fever, chills, and loss of energy or weight. Review of systems was completely negative. He came to the hospital. After physical examination, generalized lymphadenopathy was excluded after careful examination apart from that local right inguinal mass. The swelling was firm and nonfluctuant. It had an intact overlying skin with no redness or discharging tract. Blood work was unremarkable and HIV was nonreactive. CT scan of the abdomen and pelvis with oral and IV contrast was done () and showed necrotic appearing, enlarged right inguinal lymph node, measuring 5.0 × 7.1 cm. Additionally mildly enlarged right inguinal and external iliac lymph nodes were seen. Chest X-ray was unremarkable. Surgery was done with complete excision of the inguinal lymph node under general anesthesia. The iliac lymph nodes were not excised, being small and multiple. Pathology came back with malignant melanoma (Figures , , , and ). So the plan was to manage the remaining lymph nodes that were not excised with radiotherapy and (ipilimumab). He was discharged for follow-up in the clinic. Careful history and thorough physical examination were obtained, including examination of the lymph nodes and skin all over the body. Genital examination and digital rectal examination did not show any evidence for a primary lesion also. Patient noticed a fluctuant area in 3 the site of previous lymphadenopathy 1 week after the surgery. Using a 20-gauge needle, approximately 8 mL of seroma was evacuated. The fluid was serous in nature. There was a decrease in size in the fluctuant area. The fluid was drained with fine needle and aspiration fluid was sent for cytology coming back with malignant melanoma with the same pathology. PET scan was done and did not show any abnormal uptake all over the body. Patient was referred to a dermatologist for further evaluation. As a desperate trial, 2 lesions were excised. One was from his upper back of the trunk and the other was in the left thigh. Both looked like brown nonraised moles. Punch biopsy was obtained. The one from the back came back as junctional nevus and the other one from the left thigh came back as lentigo simplex. The case was then diagnosed as a metastatic melanoma of unknown primary. Patient received radiation therapy and then started on ipilimumab cycles.
An 83-year-old male with no significant past medical history presented to the clinic with a large, fungating scalp mass. The patient struck his head on an open cabinet door but did not seek immediate medical care as he did not have additional symptoms. He also avoided going to a physician’s office as the pandemic was on the rise. The patient presented to his primary care physician (PCP) for the first time approximately 4 weeks after the initial injury, for a persistent “lump on his head.” The mass was approximately 2 cm, fluctuant and friable with recurrent episodes of bleeding on the left aspect of frontal head above the left eyebrow.\nThis was initially diagnosed as a hematoma of the scalp, and he was advised to avoid self-manipulation, and to hold his aspirin at this time. Subsequently he was referred to a wound care clinic for possible drainage, where local wound care with a soft absorbent dressing consisting of a hydrophilic polyurethane foam sheet bonded to a semipermeable polyurethane film, with dry dressing was recommended. Due to the pandemic, his wound care follow-up was less frequent than normal circumstances. At the initial follow-up visit in a few weeks later, the scalp mass was found to become a large protuberant lesion without any evidence of healing, which raised significant concern for a possible malignancy. Although computed tomography (CT) of the head was recommended for further evaluation, he did not follow up with his PCP for several months due to fear for contracting COVID-19.\nHe was eventually referred to the surgical oncology clinic until 5 months later from the initial presentation, at which point he was noted to have a large, ulcerated mass on the left scalp. The mass was not fixated to the scalp, and mobile on physical exam (). Biopsy of the lesion was obtained in the clinic under local anesthesia, which demonstrated malignant spindle cell neoplasm favoring leiomyosarcoma. A CT head demonstrated a mildly enhancing exophytic, left frontal scalp mass measuring 3.4 × 3.3 cm with no evidence of invasion to the underlying bone (). Staging CT scan of the chest, abdomen and pelvis revealed no obvious distant metastatic lesions.\nWide local excision of the lesion was planned with possible myocutaneous rotation flap. He underwent a radical excision of left scalp lesion. The pericranium of the scalp was resected as a deep margin, and the 9 × 7 cm scalp defect was reconstructed using a full-thickness skin graft harvested from the left supraclavicular area (). The donor site was closed using an advancement flap measuring 15 × 7 cm.\nThe patient tolerated the procedure well and was discharged home from the recovery. Final pathology demonstrated 5.4 × 5.1 × 2.2 cm grade 2 malignant spindle cells consistent with leiomyosarcoma (T3). A mitotic rate was 28/10 high-power field (HPF), and all margins were negative ().\nOn his first postoperative visit, the patient was doing well with no surgical site occurrences. Subsequently, he is being considered for adjuvant radiation, given the tumor size, high mitotic rate, and grade 2 histology.
The current case report describes a retired, white, female patient who was 82 years old and from Spain. She was diagnosed with FL in August 2010 and presented with lymphadenopathy in the right femoral region with FL, grade 3A, a Follicular Lymphoma International Prognostic Index risk score of 1, low tumor burden, and no bone marrow involvement. A timeline of the patient history, interventions, and clinical findings is shown in Fig. . The patient had no relevant prior medical history. She was treated with radiotherapy (40 Gy in 20 fractions) for stage I localized disease and achieved a complete response (CR) with no major concerns.\nIn June 2012, approximately 18 months after CR was achieved, the patient experienced FL progression, presenting with a submandibular mass. A biopsy revealed grade 3a, stage Ia FL. The patient refused chemotherapy at that time, so she was treated with repeat radiotherapy (40 Gy in 20 fractions) and achieved CR with no toxicities. However, 9 months following the second CR, the patient experienced FL progression, presenting with lymphadenopathies in the left axillar region and splenic lesions, and biopsy revealed grade 1, stage IIIa FL. She was treated with six cycles of rituximab-chlorambucil in lieu of more toxic treatment options that the patient had refused. She achieved a partial response and refused further treatment at that time. Four months following the last treatment, the patient experienced FL progression (refractory FL) and presented with lymphadenopathies in the left axillar region and grade 3/4 lymphedema in the left arm. Biopsy revealed grade 1 FL. The patient also showed left pleural effusion (not investigated further in this case study). She was enrolled in a randomized, double-blind, phase 3 study evaluating rituximab in combination with an investigational therapy versus rituximab and placebo. The patient progressed after 4 months.\nBased on the refractory nature of the disease following two lines of chemoimmunotherapy (including an immunomodulatory drug treatment), a fourth relapse, and disease that was refractory to both rituximab and chlorambucil, the decision was made to initiate the patient on idelalisib monotherapy (150 mg orally twice daily). Trimethoprim/sulfamethoxazole prophylaxis was also initiated. Palliation was the alternative treatment option that was considered at this stage. The goal of treatment was resolution of lymphedema and dyspnea.\nResponse to idelalisib treatment (started on 19 May 2015) was observed at the 3-month follow-up in this patient, as indicated by the computed tomographic scans shown in Fig. . After 3 months of treatment, there was a significant reduction in lymphedema in the left arm, a partial response of the lymph nodes according to Lugano criteria [], and clearance of pleural effusion. At 6 months, the remaining lymphedema in the left arm was almost entirely resolved, and she remained in partial response (almost reaching CR) at the 9-month follow-up, with no evidence of pleural effusion. At 12 months, following initiation of treatment with idelalisib, the patient demonstrated a sustained partial response (almost CR), continued to have no pleural effusion, and lymphedema was resolved. Overall, the patient tolerated idelalisib well and reported good adherence to treatment. There were no hematological concerns or liver toxicity observed following the initiation of idelalisib. Hemoglobin, absolute neutrophil counts, platelet counts, and aspartate aminotransferase/alanine aminotransferase levels all remained within normal limits throughout treatment (Fig. ).\nAfter 11 months of treatment with idelalisib, the patient developed erythematosquamous papules and plaques, with some pustules at the periphery limited to the scalp, left forehead, back, buttocks, and over some scars on the abdomen and the right side; without accompanying symptoms. This was designated a grade 2 rash that was described as psoriasis-like, with T-cell infiltration based on skin histopathology (Fig. ). In line with suggested guidance, idelalisib dosing was interrupted for 4 weeks [], and the patient was treated with topical steroids. She experienced improvement to grade 1 but without complete resolution (Fig. ). Idelalisib was reinitiated at a lower dose of 100 mg twice daily. A physical examination revealed that the patient did not experience any worsening of any skin lesions, lymphoedema, and no palpable lymphadenopathy in the left axillar region after 1 month from re-initiating treatment. However, the patient then experienced a second recurrence of the psoriasis-like rash, which led to a second interruption of treatment for 5 weeks. These symptoms were well managed with topical steroids, and the patient then received a reduced dose of idelalisib 100 mg twice daily without a further recurrence.\nThe patient continued on idelalisib treatment and maintained a partial response up to the most recent visit (in October 2017), thereby demonstrating a maintained partial response over 30 months. No lymphoma progression was observed during the interruptions of treatment.
A 52-year-old male had an uncomplicated IPP procedure performed for vasculogenic impotence refractory to medical therapy. He had no medical history of keloids or other scarring problems. The procedure was performed through an infrapubic approach. An AMS 700 series CX prosthesis (21 cm with 3 cm rear tip extenders) was placed, for an intracorporal measurement of 24 cm. The patient's preoperative stretched penile length was 16 cm, which was similar to his penile length with the IPP fully inflated immediately after surgery. Photos in the immediate postoperative period demonstrated an excellent result (Figure A). The patient provided written informed consent to have his results published; all appropriate ethical guidelines were followed.\nThe patient was evaluated by the surgeon at 1 and 3 weeks after surgery per routine postoperative protocol and was found to be healing appropriately. The patient was instructed to start inflating his IPP 1 week after surgery and was given clearance to resume full sexual activity at 3 weeks, once he had demonstrated understanding of how to use the device, and all visible healing was completed. At 3 weeks after surgery, the patient had an excellent cosmetic and functional result similar to the immediate postoperative results. He was given instructions to maximally inflate his IPP daily for 15 minutes for 3 months until his next follow-up appointment, regardless of sexual intercourse frequency.\nThe patient suffered an unrelated dominant hand injury shortly after completing his 3-week follow-up visit and was unable to follow the postoperative instructions to maximally inflate his IPP daily. He reported inflating his implant only once every 1 or 2 weeks. He did not notify his surgeon of the injury until his 3-month postoperative visit. When the patient returned to the clinic after 3 months as scheduled, he complained of new onset curvature and difficulty fully inflating his prosthesis. There were no complaints of fever, chills, or pain. Physical examination demonstrated an obvious S-shaped deformity of the left cylinder and buckling of the right cylinder, without evidence of erythema, induration, fluctuance, or erosion (Figure B). No further imaging was obtained. The problem was discussed, and the patient decided to proceed with revision surgery.\nThe patient underwent exploratory penile surgery through his previous infrapubic incision. The previous corporotomy was opened, and the AMS CX cylinders were removed. Prior to antibiotic irrigation, aerobic and anaerobic culture swabs were taken from each corpus and were found to be negative for bacterial growth. The corpora were irrigated with copious amounts of antibiotic solution prior to re-measuring the intracorporal length with a Furlow instrument (American Medical Systems, Minnetonka, MN, USA). Both corpora measured 22 cm—2 cm less than measured at initial surgery. Using a #15 scalpel, intracorporal incisions were made on the lateral and ventral sides of the capsules to release the contractures and to prevent kinking of the cylinders (Figure C). After the old cylinders were removed, the blade was slid inside the capsule, inside the old cylinder space, and the walls of the capsule were incised. We replaced the previous AMS CX cylinders with new AMS CX cylinders and a 1-cm rear tip extender. Inflation of the implant confirmed correction of the S-shaped deformity with shortening of the penile length (Figure D).\nThe surgery was performed as an outpatient procedure. The implant was left fully inflated for 1 week, at which time the patient was instructed to start a maximum inflation protocol. The implant was to be inflated to maximum pressure and left inflated for at least 15 minutes, twice daily for 3 months. The patient was seen at 3 months after his revision surgery and had complete correction of his S-shaped deformity with results similar to his postoperative result. His penile length at maximal inflation was 14 cm.
An 8-year-old boy had admitted to our emergency department after falling on his left hand with outstretched upper extremity. The patient had admitted to our clinic 30 min after trauma. In the clinical examination of the patient, there was an open wound in the transverse antecubital region. Distal humerus and articular surface could be seen by looking through the wound (). Although neurological examination showed us no pathology, distal pulses could not be palpated in vascular examination. In the conventional radiographies, a posterior elbow dislocation without accompanying fracture was detected (). Because of the strong possibility of brachial artery injury, the patient had consulted to the vascular surgeon and an emergent surgical intervention had planned for this patient.\nThe patient received emergent surgery under general anesthesia. The transverse incision had lengthened 1 cm and the buttonhole dislocation had released. Following the exploration of the open wound in the elbow, open reduction was performed. Intra-articular debris was washed out. Brachial artery was identified in the open wound without continuity and distal pulses were still impalpable. The vascular surgeon had attended to the surgery and the brachial artery had repaired primarily. After the treatment of vascular pathology, the stability of the elbow was evaluated and the elbow was stable during 0–140° of flexion and extension. Varus and valgus stress tests were negative. Due to the stability of the elbow, we decided to follow this patient with splint instead of an external fixator. The wound was closed in a standard fashion with the approval of vascular surgeon; upper elbow splint was applied at 100°. We did not observe any postoperative vascular or wound complications in the follow-up. After 4 weeks follow-up with splint, using angle adjustable hinge elbow orthotics started controlled active assistive exercises. By the end of the 12th week, the orthotics was removed and movement was completely allowed.\nSix months after the operation patient had no any complaints about his elbow. The patient's elbow range of motion, in supination–pronation arc, has been detected as 80–85° and in extension–flexion arc; range of motion has been detected as 0–140° (). There was no varus–valgus instability. In conventional radiographies, Hastings class II heterotopic ossification was detected between the anterior capsule and the brachialis muscles ().
A 72-year-old Japanese man presented to the emergency department with dyspnea following swelling of the right lower limb. Physical examination was unremarkable, except for mild hypoxemia (SpO2 95%) and lower limb pitting edema. Laboratory findings showed elevated D-dimer levels (43.5 μg/mL). Enhanced CT images showed massive PE (Fig. ) as well as several nodules in the lungs. Moreover, an irregularly shaped tumor was also present from the retroperitoneum near the right ureter to the right pelvis, as well as DVT in the right iliac vein (Fig. ). He was transported to a nearby cardiovascular hospital and received anticoagulation therapy as well as inferior vena cava filter indwelling. From image inspection, retroperitoneal tumors, such as ureteral cancer, were suspected, although urine cytology did not show obvious atypical cells. A laparotomy biopsy of the tumor was scheduled. However, 2 months after onset, a re-examination of chest CT due to exacerbation of breathing difficulties showed a rapid deterioration of nodular lesions in the lungs. Although he was admitted to our hospital, respiratory failure combined with tumor metastasis, pneumonia, and alveolar hemorrhage made it difficult to perform a surgical biopsy. He died 8 days after admission due to progression of respiratory failure before the biopsy procedure.\nA general autopsy revealed a mass lesion in the retroperitoneal right caudal side, in contact with the right anterior part of the lumbar spine. The tumor macroscopically involved the right common iliac artery and vein, as well as the right ureter (Fig. ). However, there was no hydronephrosis in the right kidney, and no irregular lesions were observed in the ureteral luminal surface, indicating the absence of ureteral cancer. The tumor had a diameter of approximately 7 cm, and the reddish area due to bleeding was observed macroscopically on the dividing surface (Fig. ). Macroscopic observation of the transverse plane revealed that the tumor was continuous with the region of the right iliac artery and vein (Fig. ). Histological analysis using Elastica van Gieson staining showed that collagen and elastic fibers around the right external iliac vein were degenerated and disrupted due to the proliferation of spindle-shaped atypical cells (Fig. ). These atypical cells were observed in both the intraluminal and extraluminal areas, which caused vascular occlusion. Immunohistochemical staining showed that the atypical cells were positive for CD31 and Ets-related gene (ERG), which are endothelial cell markers (Fig. ). Based on these findings, we diagnosed angiosarcoma originating from the right external iliac vein as the cause of DVT and subsequent PE observed at the time of initial onset. Many tumor metastases with hemorrhage were observed in both lungs, as well as left pleural metastasis and bloody pleural effusion. These metastatic lesions and subsequent alveolar hemorrhages were considered to be the cause of fatal respiratory failure.
A 25-year-old female Irish patient presented with recurrent episodes of rhabdomyolysis since childhood. The first episode occurred at the age of 22 months following a respiratory tract infection. At that time, her creatine kinase (CK) serum concentration was noted to be 250 000 U/L.\nThe family history indicated that both parents were well. However, two siblings (brother and sister) had sudden death at the age of 2 and 4 years more than 20 years ago following a short infectious illness with sudden deterioration over a period of hours. In both cases, the children became progressively weak with severe muscle pain and had evidence of rhabdomyolysis and myoglobinuria of uncertain etiology. Skeletal muscle histology and electron microscopy studies at postmortem evaluation were normal in both children, cardiac evaluation demonstrated dilated cardiomyopathy. Notes pertaining to their clinical episodes were not available. On first presentation, our patient had no hypoglycemia and no ketosis during the acute illness, but was initially treated as a possible long chain fatty acid oxidation defect due to the family history.\nThe patient had numerous subsequent admissions with similar presentations of extremely elevated CK concentration (1 000 000 U/L at the age of 9) associated with muscle pain. This was despite aggressive carbohydrate supplementation including nocturnal cornstarch. Her fat intake from food was continually restricted to approximately 40 g/d. Her total fat intake was supplemented with the use of MCT Oil (medium chain triglycerides supplement) and essential fatty acids in the form of walnut oil. Coenzyme Q10 at a dose of 100-200 mg daily was provided on an ongoing basis. The CK concentration was also raised between the episodes (500-2000 U/L). She was advised to limit her exercise to 20 minutes per day and have high-calorie drink prior to any physical activity.\nExtensive investigations were performed over the presenting years with the lack of a definite etiology. Serial urine organic acid profile and the acylcarnitine profiles were normal. A fibroblast fatty acid oxidation study showed normal myristate and palmitate oxidation studies in fibroblasts. Genetic analysis for fatty acid oxidation defect (LCHAD, MCAD, CPT I, and CPT II) and McArdle disease was uninformative.\nAt the age of 16 years, mitochondrial respiratory chain activity measured in a muscle biopsy was normal, however morphological findings, such as intramyocellular lipid, were compatible with lipin-1 deficiency (Figure ). Her echocardiogram and electrocardiogram did not show any abnormal findings.\nAt the age of 19 years, DNA sequence analysis of the LPIN1 gene (all coding exons and flanking intron boundaries corresponding to the canonical transcript variant NM_145693.3) revealed the presence of a common pathogenic intragenic deletion within the LPIN1 gene (c.2295-866_2410-30del1763) encompassing exon 18 (HGMD accession: CG085181). However, a second mutation could not be identified.\nSubsequently, the LPIN1 coding region was analyzed by reverse-transcriptase PCR (RT-PCR) from total RNA isolated from muscle tissue and conventional DNA sequencing (Figure A,B). In addition to transcripts lacking exon 18 or exons 18-19 (corresponding to the allele harboring the genomic exon 18 deletion), an alternative exon spliced in between exon 5 and exon 6 was detected in a significant proportion of transcripts (Figure C,D). Because this alternative exon, named exon 5a, corresponded to an alternatively spliced in-frame exon annotated only in an N-terminal LPIN1 transcript variant (NM_001261428.2), further targeted DNA-based sequencing was performed. Indeed, this identified a second heterozygous variant (NC_000002.11:g.11916284C>A), which was formally regarded a nonsense mutation introducing a premature stop-codon within exon 5a (which would correspond to NM_001261428.2:c.942C>A, NP_001248357.1:p.[Cys314*]; Figure ). RT-PCR of total RNA isolated from healthy skeletal muscle detected exon 5a also in transcripts containing the first (noncoding) exon of the canonical isoform (data not shown). However, because exon 5a is currently not annotated to be contained in this main reference transcript (NM_145693.3), further studies will be needed to clearly establish pathogenicity of the novel variant.\nSegregation analysis in the patient's family revealed that her father and younger sister are heterozygous for the common pathogenic intragenic deletion (NM_145693.3:c.2295-866_2410-30del1763), while her mother was a heterozygous carrier of the variant within the alternative exon. Thus, the results of these molecular genetic analyses were formally consistent with the clinical diagnosis of LPIN1-associated rhabdomyolysis, due to compound heterozygosity for a known pathogenic deletion and potentially pathogenic novel mutation.\nAt the age of 25 years, the patient presented with acute muscle pain and weakness following prolonged fasting and strenuous exercise. A rhabdomyolysis crisis was confirmed with a CK of 500 000 U/L. She was admitted to intensive care unit (ICU) for a 2 week period over which period she lost a significant amount of weight. During her critical illness, she received an intravenous infusion of 10% dextrose at 1.5 times maintenance with added electrolytes, sodium bicarbonate, morphine, and dexamethasone. Intravenous carnitine was also provided as the patient had previously observed this to be clinically beneficial. She was treated symptomatically, with shortened fasting periods, corn-starch at night and an “unwell dietary regimes” with a mild restriction of fat (40 g per day) and supplementation with MCT oil and walnut oil for essential fatty acids and Liquigen 5 g daily. On day 11, her CK was monitored 4 hourly and was 1248 U/L. On discharge from ICU, this patient had generalized muscle weakness, stiff lower limb muscles (gastrocnemius), and bilateral drop foot requiring orthotic splints. Muscle weakness gradually improved after months of rehabilitative physiotherapy. Her drop foot has improved somewhat (power from 0/5 to 3/5 for dorsiflexion and extensor hallicis longus over 1 year) with areas of altered sensation anterolaterally below the knees consistent with bilateral residual common peroneal neuropathies (CPN). Serial nerve conduction/electromyography studies demonstrated bilateral CPN palsies and a background generalized myopathy. The most likely cause of this patient's weakness was a critical care neuromyopathy, which has improved with time, in addition to CPN palsies related to significant weight loss while critically ill. However, we cannot exclude the possibility that both defects were related to her lipin-1 deficiency. While myopathy has been reported in a few cases, CPN damage is not a known association of this rare metabolic disorder.
A previously healthy, 18-year-old female patient of African descent sought medical care with a history of weight loss of 14 kg during the last 3 months simultaneously with headache and vomiting. Two weeks after the onset of the symptoms, she started with diplopia and a burning sensation over the lower limbs that lasted for a month and progressed to face and scalp allodynia. She also had gait instability. A few days before hospital admission, the muscle strength of her lower limbs became impaired and ascended to the upper limbs. This was associated with sensory changes in the trunk and urinary incontinence.\nInitial examination showed an ill-looking patient with preserved cognitive functions, emaciated, pale, acyanotic, and with stable hemodynamic parameters. She denied dyspnea. Neurological examination showed slight asymmetric tetra paresis with muscular strength of grade 0 in the lower right limb and grade 3 in the upper right limb, and grade 1 in the lower left limb and grade 3 in the upper left limb. Deep tendon reflexes were absent in the upper limbs but were brisk with extensor plantar response in the lower limbs. All sensory modalities were compromised in limbs and trunk at T2 level. Cranial nerves were normal. Catheterization was necessary due to voiding dysfunction.\nInitial laboratory workup included ANA and other inflammatory markers; serology for hepatitis B and C, HIV, HTLV, and syphilis showed negative results; blood and urine cultures were also negative. The lumbar cerebral spinal fluid (CSF) analysis disclosed slight inflammatory changes () with negative results for China ink and cultures (aerobic, fungal, and acid fast bacilli). The brain and spine computed tomographies were normal.\nAfter 3 days in hospital, she rapidly evolved with plegia in all limbs, nasal voice and swallowing disturbances with preserved bulbar reflexes, vertical nystagmus, bilateral internuclear ophthalmoparesis, and acute respiratory failure that required mechanical ventilatory support. Magnetic resonance imaging (MRI) was not performed initially due to clinical instability and because transport to another institution was considered life threatening.\nBased on the clinical prodrome (weight loss, vomiting, headache with unknown origin), neurological presentation (transverse myelitis, quickly progressing to brainstem dysfunction), compressive cord lesion dismessed by tomographic study, CSF inflammatory abnormalities, and negative workup to more common infectious agents, the diagnosis of neuromyelitis optica (NMO) was highly considered. Pulse therapy with methylprednisolone (1g/day for 5 days), in addition to cyclophosphamide (1 g), was started as soon as her neurological status became worse. After 1 week of pulse therapy, her neurological impairment remained unchanged, except for muscle strength grade 3 at wrist extension in both hands. Therefore, intravenous immune globulin (400 mg/kg/day for 5 days) was also prescribed. Prednisone 40 mg/day was continued until the next pulse. From that time, her neurological deficits started to gradually improve. She received one more pulse before the hospital discharge. She was discharged without tracheostomy with normal ventilatory parameters, and independent for eating after 40 days of hospitalization. She was unable to walk, but was able to remain seated without aid. The muscle strength was grade 4 with distal predominance in the upper limbs, and was grade 3 proximal and grade 2 distal in the lower limbs. The deep tendon reflexes persisted as brisk in the lower limbs, and hypoactive in the upper limbs, but a brisk finger flexor response was obtained when bicipital reflex was searched in the left side. She also complained of visual blurring at the left eye with normal acuity, although a slightly pale optic disk was detected, and the vertical nystagmus was persistent. The sensory deficit remained at T2 level; however, there was an improvement of the vibratory perception at the knees. She regained the voiding control.\nDespite an MRI having been done 20 days after immunosuppressive treatment, areas of signal alteration in the brain stem, optic nerve and tract, left thalamus and basal ganglia with demyelinating features were found at brain imaging study ( and )\nDiffuse signal alteration throughout the cervical and thoracic segment, characterized by elongated hyperintense areas on T2-weighted and FLAIR images, was observed. These areas were predominantly located at the periphery of the cervical level, and centrally located at mid-thoracic level with small areas of vanished gadolinium enhancement. These alterations were continuous and confluent with slight expanding effect ().\nTested in a frozen (−20ºC) stored sample, serum IgG-NMO was negative.
A 9-year-old male child from a village of North India presented with complaints of inability to squat and sit cross-legged since the age of 2 years. The child had started walking at the age of 13 months before which he was able to crawl and sit on the floor. The mother (primary informant) also gives a history of febrile illness for which multiple injections were administered in both the buttocks following which the child gradually developed the disability. The parents initially noticed a mild limitation of hip motion which eventually progressed such extent that he has to left his school due to this disability and was having difficulty in maintain his personal hygiene (Video 1). The parents had not sought care for the child initially and he was being managed conservatively with physiotherapy, but the child was not showing any improvement (). There was no significant pre-, peri-, or post-natal history. The child has no other significant history of previous illness apart from that febrile episode. He has no history of previous surgery or history of any long-term intake of medications. On examination, there was puckering in bilateral glutei muscle. There was atrophy and flattening of both glutei with left significantly more wasted than right (). Hip flexion was only 0–40° on both sides. Apart of flexion adduction and internal rotation was also restricted on both sides (). There was no significant abnormality noted on plain radiograph (). Magnetic resonance imaging (MRI) showed marked atrophy of bilateral glutei (left> right) with low-intensity band within upper and middle 1/3rd of belly of the left gluteus maximus. Mild medial retraction of distal body of the tendons of gluteus maximus was also noted (). After thorough evaluation, a diagnosis of bilateral gluteus maximus muscle contracture was made and he was planned for open release of contracture. Open release of contracture was done in two separate settings at an interval of 1 week. The left side was operated before the right side. On the left side, we released the gluteal muscle from the iliac crest, but it did not improve the range of motion of hip joint, then we cut the gluteus maximus tendon and fibrotic band near greater trochanter, after cutting the tendon, we were able to flex the hip joint to >90°. On the right side, we did not attempt to release gluteus muscle from the iliac crest as we found it to be ineffective. Only the tendon and fibrotic band were released following which an improvement in flexion noted intraoperatively. Sciatic nerve was identified and protected in both procedures (). Post-operative period was uneventful and there was no complication related to wound healing. Gradual passive physiotherapy and range of motion exercise were started 2–3 days after surgery. After 1 year of follow-up, the child had good range of motion. He was able to squat and sit cross-legged. Hip extension was possible in both hips. There was hypertrophic scar formation on both sides, for which he was advised treatment, but the parents refused for treatment () (Video 2).
A 57-year-old woman presented with severe neck pain after a pedestrian traffic accident. The initial neurologic examination showed symmetrical motor power without any sensory changes in the extremities. Plane films and computed tomography of her cervical spine demonstrated a C2 fracture involving the bilateral (mainly left) C2 pedicle and a lateral mass extending into the base of the odontoid process. The preoperative magnetic resonance imaging (MRI) did not demonstrate any abnormal course and relationship of the VA and adjacent structures.\nThe patient underwent a bilateral pedicle screw fixation between C1, C2, and C3. The operative procedures through posterior approach were uneventfully completed. Six days after operation, she complained of an abrupt and severe nuchal pain and blurred vision. She deteriorated the symptoms and showed dysarthria, right facial palsy and drowsy consciousness. The diffusion MRI of the brain showed a multifocal acute infarction in the bilateral cerebellar hemisphere and the bilateral occipital lobes. The digital subtraction angiography (DSA) demonstrated high-flow VAVF between the V3 segment of the left VA above the pedicle screw located left C1 and the paravertebral venous plexus (). The VAVF was also fed by a backward flow from the right VA. The blood supplies from the right VA to posterior circulation were sufficiently accounted. Therefore, a complete occlusion of the left VA involving the VAVF using EVT was decided to prevent an aggravating ischemic symptom due to arterial steal and thromboembolism caused by VAVF.\nUnder local anesthesia, a single microcatheter approached via 5 Fr guiding catheter located the left VA was easily passed from the proximal part of the fistula to the distal V3. The V3 segment of the left VA was complete occluded by four detachable coils and ten pushable coils (). The angiography revealed a complete obliteration of the fistula and occlusion of the V3 segment of the left VA (). The patient had an unremarkable post-procedural course without further aggravation of the symptoms relating to a cerebral ischemia or VAVF. She was gradually improv-ed and was discharged two weeks after the procedure. Two months after the embolization, the follow-up angiography showed stable occlusion of the VAVF. During 32 months follow-up, there were no new symptoms and lesions at the imaging studies.
A 27-year-old Caucasian woman was referred to us by a general practitioner for prosthodontic evaluation and treatment in 2010. The patient's chief complaint stemmed from a car accident in 2002, in which she lost a few teeth. At that time, an iliac crest graft was performed, following which a dental bridge was placed. The bridge lasted for 8 years but had become unstable when the patient presented to us. In addition, the patient experienced some discomfort and bleeding. Based on a review of her medical history, the patient was classified as an American Society of Anesthesiologists Class I patient (i.e., a normal healthy patient), and she had no systemic disease and was not on any medications.\nClinical examination showed implants in the locations of missing teeth 9, 11, 12 and 13 with a fixed prosthesis. Hyperplastic tissues on the labial side and bleeding were noted, and suppuration was presented on palpation. The fixed prosthesis was infrapositioned, which resulted in an open bite situation []. The patient did not recall any parafunctional habit and did not have signs of occlusal wear. Remnants of cement were seen around the platform of the failed prosthesis. Head and neck examination revealed no cervical or submandibular lymphadenopathy as well as no facial deformities or asymmetries. The patient showed adequate interarch space at the approximate vertical dimension of occlusion. According to House's classification, the patient was classified as a Class I patient (i.e., accepts dentist's judgment and instructions and likely to best prognosis). The patient exhibited a Class III prosthodontic diagnostic index and a Class I residual ridge relationship and had reasonable expectations and desires about the anticipated dental treatment. Radiographic examination demonstrated moderate to severe bone loss around the dental implants [Figures and ].\nThe patient was referred to both oral and maxillofacial and orthodontic departments for consultation. Existing implants were judged to be failing, and minor orthodontic treatment was recommended. The treatment plan's benefits, risks and alternatives were discussed with the patient; she agreed for implant removal, bone grafting and a fixed hybrid prosthesis but rejected orthodontic treatment, and her signed consent for the treatment was obtained.\nPreliminary impressions were made for the fabrication of an immediate interim prosthesis. The implants and the implant prosthesis were removed under local anesthetic using gauze and hemostats. Given the substantial bone loss, multiple grafting procedures were planned and the same was discussed with the patient. The autogenous iliac grafting was performed first. After 9 months of healing, radiographic evaluation was carried out to document the vertical and horizontal bone gain. In the second grafting procedure, a 20 mm × 20 mm titanium mesh was fixed with 6- and 8-mm bone screws on the buccal and lingual sides of the ridge with a 0.5 cc of Geistlich Bio-Oss (Geistlich Pharma, Wolhusen, Switzerland) bovine bone, 0.5 cc Puros cortical particulate allograft (Zimmer Biomet Dental, Palm Beach Gardens, FL, USA) and a small amount of recombinant human bone morphogenetic protein-2 combined with an absorbable collagen sponge (INFUSE Bone Graft, Medtronic Sofamor Danek, Memphis, TN, USA). After 1 year of healing, radiographic evaluations were done [], and the titanium mesh was surgically removed. At this stage, alginate impressions were obtained for both arches, and a diagnostic wax-up was achieved to evaluate the prosthetic space. The radiographic template was fabricated, and a cone-beam computed tomography scan was completed while the patient wore the template. The scan was uploaded to in vivo 5 (Anatomage, San Jose, CA, USA) for implant planning, and the digital imaging and communications in medicine file was exported. A tooth-supported implant guide template was ordered.\nThe computer-aided design/computer-aided manufacturing implant guide was placed intraorally. Sequential osteotomies were performed using NobelActive surgery kit (Nobel Biocare AB, Göteborg, Sweden) in the sites of missing teeth 9 (3.5 mm × 11.5 mm), 11 (4.3 mm × 11.5 mm) and 13 (4.3 mm × 10 mm). Implants were placed with 40 N/cm of torque. All the surgeries were performed under local anesthesia. Then, after 8 months of healing, the implant uncovering procedure was performed under local anesthesia. Healing abutments were placed and hand torqued. Six weeks later, open-tray impression copings were placed and connected intraorally with dental floss (Oral-B, Procter and Gamble) and Filtek Supreme Ultra Flowable Restorative composite (3M ESPE, St Paul, MN, USA). Periapical radiographs were made to confirm the seating. Polyvinyl siloxane (PVS) impression material (Aquasil, Dentsply, Konstanz, Germany) was used to make impressions for both arches. Interocclusal records were obtained using PVS Regisil 2X VPS bite registration material (Dentsply). The custom abutments and framework were tried intraorally, and radiographs were made to confirm the seating. Later, Noritake porcelain system (Kuraray Noritake Dental, Tokyo, Japan) was used. Tooth shade B1 was selected for the cervical and middle thirds, and NW 0.5 was selected for the incisal third. For the gingival shade, a mix between G3 and G4 was used. The prosthesis was tried intraorally in bisque porcelain, and necessary adjustments were made. The prosthesis was finished and glazed. The hybrid prosthesis was cemented with TempBond (Kerr Dental Products, Romulus, MI, USA) []. Excess cement was carefully removed after making impression of the intaglio surface of the prosthesis. The hybrid prosthesis was cemented, and bitewing and periapical radiographs were taken. PVS impression material was used to make impressions of both arches for fabrication of an occlusal guard. The occlusal guard was placed, and the patient was instructed to maintain the prosthesis using Oral-B Superfloss and interdental brushes. At the follow-up appointment in 2015, the patient was satisfied with the treatment, with the occlusion, periodontal health and remaining teeth condition being stable. The patient was given home care instructions and a program for recall visits. In 2017, a panoramic radiograph revealed stable connection between implants and maxillary bone as well as no proximal bone or periapical changes were detected [].
An asymptomatic 73 year-old man with a remote history of surgically treated gastric cancer at the age of 40 presented with a 50 mm-sized mass on a chest radiography (). He had no significant familial history without any drug use nor allergic history. Chest contrast-enhanced Computed Tomography (CT) revealed an irregular homogenous mass in the anterior mediastinum, which extended along the left diaphragm without any specific enhancing effects (19–27 Hounsfield Unit) (ab). Magnetic Resonance Imaging (MRI) revealed a septate cystic lesion with a high intensity on the T2-weighted image (a). Fat-suppressed T1-weighted images revealed that the mass contained less fatty tissue (b). An invasive thymic epithelial tumor or soft tissue tumor were suspected mainly because of its location and a radiological extent, and a radical excision was planned via video assisted thoracoscopic surgery by an attending thoracic surgeon at our institute.\nWith the patient in the right semi lateral decubitus position, a total of 3 ports were placed on the 6th, 7th, and 8th left intercostal anterior axillary lines, respectively. Under Carbon dioxide insufflation at a pressure of 8 mmHg, the lesion was visualized adjacent to the pericardium. The lesion was also observed to have grossly invaded into the diaphragm and lower lobe of the left lung. He underwent a total tumorectomy with a combined resection of the pericardial fat, left lung, and diaphragm. Since a complete resection was achieved, no intraoperative frozen section examination was performed. The postoperative course was uneventful and the patient was discharged on the third postoperative day. The macroscopic findings of the specimen revealed a circumscribed mass embedded in the fat tissue (). The histopathological findings revealed dilated medium sized blood vessel proliferation in the adipose tissue. Those vessels were mainly muscular veins with a small number of arteries. Those findings were compatible with the diagnosis of a mediastinal hemangioma (a). Only fibrous adhesions were observed between the tumor and resected lung and diaphragm without any histological invasion (b).
A 33-year-old man who had undergone partial gastrectomy for gastric adenocarcinoma was undergoing adjuvant chemotherapy. His disease was in remission with no evidence of residual disease or recurrence on surveillance post-operative scans and endoscopic examinations. He presented to the emergency room two months after the start of adjuvant treatment with complaints of severe diffuse abdominal pain having a severity of 10/10 on the numeric pain intensity scale. His body mass index (BMI) was 24.5 kg/m2. His vital signs revealed tachycardia with a pulse rate of 118 beats per minute, fever of 38 °C, blood pressure of 120/80 mmHg, respiratory rate of 19 per minute, and oxygen saturation of 96% on room air. General and systemic examination was positive for generalized distress and severe diffuse abdominal tenderness upon palpation. Initial lab investigations revealed markedly increased white cell count of 23000/microliter and significantly increased serum lactate level of 43 mg/dL.\nHe was admitted to the high dependency unit and started on supportive therapy, antibiotics, and analgesia. An urgent CT scan of the abdomen was ordered to determine the cause of his abdominal pain.\nA contrast-enhanced CT of the abdomen was subsequently performed to evaluate the cause of abdominal tenderness. There was no evidence of local disease recurrence on the scan. However, the appendix was dilated in caliber measuring up to 10 mm. It had surrounding inflammatory changes with peri appendiceal localized fluid collection and fat stranding (Figures -). Upon a detailed look using different windowing levels, a note was made of a focal defect in the appendiceal wall integrity which was not disrupted on the serosal aspect. The appendix was enhancing and contained some intraluminal fluid. There were moderate ascites as well. Considering the overall appearances, a diagnosis of sealed off appendiceal perforation was made.\nThe patient underwent an emergency laparotomy and appendectomy. Per-operatively the appendix was mildly inflamed and a large volume of ascites was drained. The anastomotic site of prior gastric surgery was unremarkable. He was shifted to the intensive care unit and mechanically ventilated for two days. His post-operative course was on a continuous improving trend and he was subsequently shifted to the general ward and later discharged from the hospital in a stable, asymptomatic condition.\nThe histopathological examination of appendectomy specimen subsequently revealed inflammatory changes of the appendix. There were foci of poorly differentiated carcinoma as well. The immunohistochemical analysis was positive for cytokeratin and mucicarmine. A final diagnosis of metastatic adenocarcinoma with adjacent soft tissue involvement was made.
The patient is a 26-year-old man brought to our Emergency Center from the scene of a frontal crash in which the patient's vehicle impacted a wall at 30 mph. The patient was unrestrained and air bags did not deploy.\nUpon EMS arrival the patient was unresponsive and had a witnessed seizure. En route to the hospital the patient received Valium, Narcan, and IV fluids without a change in his mental status.\nUpon arrival in the Emergency Center the patient's airway was intact, his respiratory rate was 47 breaths per minute, blood pressure in the left brachial artery was 62/55 mmHg, and heart rate was 132 beats per minute. The lower extremities were mottled from the mid-thigh level with ischemic changes in both feet and absent pedal pulses. The left radial pulse and both femoral pulses were weak, but the right radial pulse was normal. Pupils were fixed and dilated bilaterally and Glasgow Coma Scale score was 7. No evidence of trauma was present on physical exam of the head, chest, abdomen, or extremities. Blood pressure was retaken in the right brachial artery and found to be 116/76 mmHg.\nThe patient was intubated and a right femoral arterial line was placed and confirmed the blood pressure of 60/50 mmHg at the femoral level.\nA presumptive diagnosis of blunt aortic injury with pseudocoarctation syndrome was made and the patient was rapidly transported for a head, chest, abdomen, and pelvis computerized tomography (CT) scan. The head CT was normal. The chest study showed a large left atrial filling defect attached to the atrial septum and an abrupt cutoff of the left axillary artery. Abdominal CT showed multiple splenic and renal infarcts and a thrombus at the aortic bifurcation extending into the inferior mesenteric artery and into both common iliac arteries ().\nThe patient was returned to the trauma bay where he underwent transthoracic and transesophageal echocardiography (). These revealed a 3-4 cm left atrial pedunculated mass. The mass was spherical with a large part missing, reminiscent of an apple with a bite out of it. The diagnosis of left atrial myxoma versus sarcoma with extensive embolization was made.\nAt surgery the patient underwent an axillary artery embolectomy with reestablishment of left upper extremity circulation. Bilateral femoral embolectomies were done. Though clot was adequately removed from the femoral popliteal systems, retrograde aortoiliac embolectomies were inadequate, and laparotomy with transaortic antegrade embolectomies as required to reestablish lower extremity flow. Pathology from femoral, brachial, and aortic embolectomies revealed organizing thrombus consistent with emboli from atrial myxoma. The small bowel and colon were viable at laparotomy. Cardiovascular surgery evaluated the patient preoperatively and the decision was made not to proceed with removal of the atrial mass until after the patient was more stable and showed signs of neurological activity.\nPostoperatively the patient lost gag and corneal reflexes. Repeat head CT scan showed diffuse cerebral edema with impending herniation. Supportive care was withdrawn at the family's request and the patient expired on postoperative day one. Autopsy confirmed a 7 × 4 × 1 cm left atrial myxoma (). Microscopic exam revealed a left atrial tumor with stellate or globular myxoma cells.
The patient was a 17-year-old female who had been a long distance runner for five years. Her height was 160 cm, her weight was 47 kg, and her body mass index (BMI) was 18.4. She had run about 210 km a week during her junior high school years and 140 km a week during her high school years on a tartan track. Her running form was the large stride type and her average time was 3 minutes and 40 seconds per kilometer. She had not reached menarche. There are two types of amenorrhea. Primary amenorrhea is the absence of menstruation in a woman by the age of 16 years. Secondary amenorrhea is where an established menstruation has ceased for three months in a woman with a history of regular cyclic bleeding, or nine months in a woman with a history of irregular periods. Her coaches had placed her on a strict diet to keep her weight low since she was 12 years old. In particular, she had never eaten dinner for two weeks before any track meets. She developed pain in the anterior part of the left thigh while training nine days before a prefectural track meet, but she resumed training two days later. Seven days later, she suddenly developed severe pain in the left hip while running, and she was thereafter unable to walk or run. She was taken to the hospital (Department of Orthopaedic Surgery, Kyoto Interdisciplinary Institute Hospital of Community Medicine, Kyoto, Japan) by ambulance. An examination revealed that her left hip was slightly flexed, and she could not move the hip joint actively or passively. Anteroposterior X-rays of the left hip showed a femoral neck fracture (Figure ). The Devas classification of the fracture was a displaced type []. The femoral head was adducted, and the shaft had been displaced upward by 2.5 cm. There was no continuity between the proximal and distal fragments. No obvious signs of sclerosis were seen in the femoral neck. Bone trabeculae were clearly seen in the femoral neck, indicating that there was no bone atrophy. The atrophy of the right femoral neck was classified to be grade 6 according to Singh's index [], which indicated no bone atrophy. The neck-shaft angle of the right femur was 138° and the alignment of the right lower limb was normal. Her serum biochemistry profile results were all within the normal range: Ca, 9.4 mEq/dl; P 3.7 mEq/dl; and ALP 250 IU/l. Her serum FSH, LH, and estradiol were measured to investigate her primary amenorrhea; the values (FSH, 3.0 mIU/ml; LH, 2.1 mIU/ml; and estradiol, 39.4 pg/ml) all decreased, thus indicating a pituitary dysfunction which thus had caused primary amenorrhea (Table ). The fracture was easily reduced by skeletal traction. Two days later, the patient had surgery. She was placed in the supine position on a fracture table, and post-reduction alignment was checked using an image intensifier. Internal fixation was performed with the Hansson Pin System® (Stryker) (Figure ). The patient was started on partial weight-bearing after 1 week; however, weight-bearing was restricted because the varus deformity of the femoral neck shaft angle increased by 3°. The varus deformity was subsequently stabilized. The bone mineral density (BMD) of the left calcaneal bone, which was used to predict of the generalized fracture [], was evaluated using dual energy X-ray absorptiometry (DEXA) 4 weeks postoperatively. The BMD was 0.870 g/cm$^2$, with a young adult mean of 103%, thus showing no bone loss. Callus formation was identified on X-rays two months postoperatively. The patient was started on full weight-bearing 3 months postoperatively. A plain X-ray of the left hip taken at 6 months confirmed bone union. T1-weighted MRI images obtained at that time showed an area of low signal intensity at the fracture site, but no low intensity band was seen in the femoral head (Figure ). Bone scintigraphy was positive at the fracture site and the femoral head, but did not show a 'cold-in-hot' appearance in the femoral head (Figure ). The Hansson Pin System was removed 12 months postoperatively because bone union was obtained and it might have become difficult to remove the implant over time. There was no evidence of necrosis of the femoral head on plain X-ray at 24 months after the injury (Figure ), and she returned to the same runner level before the injury with no complaints.
A 64-year-old man (height 160 cm, weight 62 kg) with a 10-year history of diabetes mellitus, who had undergone a left pneumonectomy due to squamous cell carcinoma in his left upper lobe, presented with pain along the incision site (corresponding to a T5-6 dermatomal distribution) for 20 days. The pain began 3 days before the appearance of vesicles, which appeared 20 days after the pneumonectomy, and a physical examination revealed allodynia and hyperalgesia at the incision site. Following the diagnosis of acute herpes zoster, he was treated with 1000 mg/day of acyclovir intravenously for 5 days and with 150 mg/day of pregabalin orally for 3 weeks. Three weeks after pain onset, he was referred to our department for treatment because the pain persisted after the skin lesions had healed. A thoracic epidural block at the T6-7 interspace with a single dose of 10 ml of 0.1% bupivacaine and 10 mg of triamcinolone resulted in complete pain relief and a bilateral sensory block of the T5-8 dermatomes as determined by the pinprick method. However, the pain relief lasted less than 3 days. At this point, he was scheduled to receive 4 cycles of adjuvant chemotherapy. Following the decision that no additional thoracic epidural blocks were possible during the chemotherapy, epidural catheter placement and the subcutaneous implantation of an injection port were recommended. The same procedure as that used in the previous case was performed at the T10-11 interspace with the catheter tip located at the T5 (). Immediately after the completion of the procedure, the epidural infusion device containing 20 mg of morphine, 20 ml of 0.5% bupivacaine and 210 ml of normal saline (a total volume of 250 ml) was programmed to deliver at a basal rate of 2 ml/hr and delivered through a port needle. The side effects and complications associated with the procedure and epidural medications were monitored closely in the general ward. Oral medications consisting of 150 mg/day of pregabalin and 10 mg/day of amitriptyline were maintained. A stool softener and a bowel stimulant were added to the routine medications to prevent opioidinduced constipation. The epidural infusion pump was changed, and the pain intensity was evaluated every 5 days. One week after the procedure was completed, 4 cycles of adjuvant chemotherapy consisting of the daily intravenous administration of paclitaxel (175 mg/m2 × 0.9) and cisplatin (75 mg/m2 × 0.9) began, with 3 weeks between each cycle.\nThe analgesic regimen decreased the visual analog scale pain score from 8 or 9 to 1 or 2. The lower pain scores were maintained until the removal of the subcutaneous port and the epidural catheter. Leukocytosis occurred during each chemotherapy cycle, but no epidural catheter infection-associated signs or symptoms presented. During the chemotherapy, no complications related to the subcutaneous port or the epidural catheter were noted. Due to the obstruction of the epidural catheter identified 120 days after the procedure, the catheter and subcutaneous port were removed. After removal, the patient reported a visual analog scale pain score of 1 or 2. Oral pregabalin and amitriptyline were tapered off uneventfully 1 month after the removal. Although the decreased visual analog pain scores of the zoster-associated pain were maintained during the 6-month follow-up period after the removal, opioid therapy (20 to 80 mg of oxycodone per day) was initiated after a new onset of cancer-related pain (left lower extremity pain). Until his death (16 months after the removal), no symptoms or signs of herpes zoster were reported.
A 12-year-old male patient visited our polyclinic with complaints of pain and swelling in the left knee. His medical record showed the pain had started after an impact to the lateral side of the left knee while playing football 2 months ago. Radiological and clinical examinations of the traumatic area were performed. While following the injury of the left knee suspected of ligamentous strain, swelling of the knee regressed. The pain was localized to a 2 cm2 area on the lateral side of the distal thigh. On the anteroposterior and lateral radiographs of the left femur, a 1-cm calcified mass was detected in the distal region of the vastus lateralis muscle, where the pain was localized. Before anesthesia, the painful region was marked with a marker. The calcified mass was monitored under fluoroscopic vision. For guidance, a ring-shaped wire was placed laterally on the marked painful region. Under fluoroscopic vision, the ring-shaped guide wire was placed on the calcified mass (). Under regional anesthesia, a tourniquet was applied to the left femur. The operation was started at 2 cm lateral to the femur through an incision. The tensor fascia latae was opened. Over the vastus lateralis, a bright-surfaced and coin-shaped cartilage-like mass with a size of 2×10 mm was observed through the incision line. The mass was mobile. It had no adhesion to adjacent tissues. There was no abnormal appearance in adjacent tissues. Pathological analysis of the mass was made during operation. The result of intraoperative consultation (frozen section) was the neoplasm with fusiform cell. It was suspicious in terms of malignity. In macroscopic examination of the excised mass, a grey-white sectional view of an irregular-shaped solid tumoral mass of 1 cm in diameter was observed besides firm regions at certain locations. In microscopic examination of the tumor, short and irregular bundle structures, which involve wide dystrophic calcification regions and consist of large fusiform cells with narrow cytoplasm and oval nucleus, were observed. In the tumor, under 100× magnification (high magnification field), there were 12 mitoses, nuclear pleomorphic structures at mild-medium level of severity. But, no tumor cell necrosis was observed. According to the classification of soft tissue tumors in the grading scale of Fedaration Nationale de Lutte Contre le Cancer, tumor differentiation of the mass was 3, mitosis was 2 (12/10 high magnification field), tumor necrosis was 0, and total score was 5. The histological grade of tumor was grade 2. In 10%–15% of the tumor, mildly differentiated regions were observed. The diameter of tumor at its largest point was 1 cm. Facial tissues were invaded, but no tumor at the surgical limits was observed. The distance to the closest surgical border was 0.6 cm. Furthermore, in immune-histochemical analyses of our tissue samples, Ki-67 proliferation index was found to be slightly high (range, 5% to 6%). Vimentin was positive. Cytokeratin 7 and EMA were focal positive. Pathological diagnosis was mono-phasic type SS (). At 1 month after the operation, patient had no complain regarding the lateral side of the knee. Late recurrence and metastasis to a distant organism were not found during the long-term follow-up (3 years).\nDuring active and passive flexion and extension movements of the left knee, the patient felt pain lateral to the knee, which restricted knee movement. While walking, stand phase of left-lower extremity was short. The patient was able to walk without support. There was a swelling throughout the lateral side of the left knee. There was sensitiveness that could be localized distal and lateral to the femur. Varus position of the knee provoked pain in the lateral collateral ligament region. Instability tests of the left knee were negative. Routine biochemical parameters were within the normal limits. Roentgenogram and magnetic resonance imaging were performed ().
A 53-year-old male presented to the surgical department with lower abdominal pain. An ultrasound revealed a well-defined 5.6 × 4.6 cm isoechoic lesion superior to the urinary bladder and connecting with the umbilicus via a duct, suggesting an urachal remnant lesion or an infected urachal cyst. Subsequently, he was referred to our urology department for further management.\nHe was diabetic and had undergone a laparoscopic inguinal hernia mesh repair 2 years previously. Physical examination revealed some abdominal tenderness. Urine sediment and culture showed no abnormalities, laboratory findings were normal and abdominal computed tomography (CT) scan confirmed a urachal cyst []. Preoperative cystoscopy showed normal bladder mucosa. The patient deferred intervention for 9 months for personal reasons. On laparotomy, a 10-12 cm fibrous nodular mass was seen arising from the dome of the urinary bladder, fixed to the right posterior surface of the rectus muscles. Because the procedure was performed late at night, no pathologist was available so frozen section could not be done. Because of its malignant appearance, we considered the risk of invasion and metastasis very likely, what resulted in discontinuation of the procedure. Incisional biopsy was taken and procedure was deferred until histopathological examination was done. Because the preoperative CT scan had become dated, we repeated an abdominal CT scan postoperative, which showed a lesion with heterogeneous solid components and thickening of anterior bladder wall []. Histopathological examination revealed fibroinflammatory tissue.\nRuled out malignant disease, 6 days after the initial procedure relaparotomy with frozen section was planned. During dissection, the tumor was identified infiltrating the posterior wall of the right rectus sheath. Frozen section was done to exclude a sampling error and to rule out an inadequate first sample. Further dissection revealed presence of the previously placed inguinal mesh []. Excision of ventral part of the bladder wall, total fibrous tissue, and mesh was performed and frozen section was suggestive of inflammatory pseudotumor. Histopathological examination confirmed the frozen section, without any signs of malignancy []. The postoperative period was uncomplicated and patient was discharged asymptomatically after 3 days.
A 39-year-old female was brought to our emergency room by a private vehicle after being shot. An evaluation revealed a wound at the lower left back and at the mons pubis. Exploratory laparotomy and resection of the sigmoid colon, left ovary, and fallopian tube with a colostomy was performed. Postoperative deep venous thrombosis prophylaxis in the form of enoxaparin, 30 mg every 12 hours, was given and then changed to heparin, 5,000 units subcutaneously every eight hours, due to worsening renal function. The patient did well until postoperative day 4 when she complained of increased abdominal pain out of proportion to the clinical findings. Her symptoms worsened the following day. A computed tomography (CT) scan of the abdomen and pelvis was obtained, the results of which were consistent with postoperative ileus and raising concerns for right colon ischemia.\nThe patient was managed conservatively, and bedside drainage of the superficial wound infection was done on postoperative day 6 with improvement in abdominal pain. On the following day, a repeat CT of the abdomen and pelvis raised more concerns for bowel ischemia; however, the patient refused reexploration (Figure ).\nA computed tomography angiogram (CTA) of the chest was obtained for worsening pulmonary symptoms on postoperative day 8 and was consistent with lower lobe pneumonia.\nFeculent drainage around the stoma was noticed on postoperative day 9, and an exploratory laparotomy was performed with resection of multiple small bowel ischemic loops, cholecystectomy, and right hemicolectomy. The rest of the small bowel appeared dusky and friable. After multiple visits to the operating room, she was left with only a few inches of the proximal jejunum. A CTA of the abdomen was done on postoperative day 13 from her initial surgery and was consistent with a superior mesenteric artery occlusion (Figure ).\nAdmission platelet count was 281, down to 82 on postoperative day 11 when she tested positive for heparin-induced antibodies. The pre-test probability for HIT score was high (2 for thrombocytopenia, 1 for timing, 2 for thrombosis, 2 for no other cause = 7). The heparin was stopped and argatroban was used. The patient did survive her injury and was referred to a small bowel transplant center.
A 54-year-old man presented to the emergency department complaining of a one-month history of non-specific abdominal pain in the left abdominal quadrant and a weight loss of about 10 kilograms in less than six months. Past medical history and physical examination were unremarkable. Laboratory tests were all within the normal range. A computed tomography (CT) scan of the chest, abdomen and pelvis revealed nodular formation (5.1 x 3.9 cm) of mixed nature with a posterior tissue component that captured the contrast product and an anterior component of a cystic nature, localized in the posterior margin of the great gastric curvature, without evidence of lymphadenopathies and no distinct planes with adjacent structures namely with spleen and left adrenal gland (Figure -). Upper gastrointestinal endoscopy was normal and biopsy showed superficial chronic gastritis with positivity for Helicobacter pylori. Endoscopy ultrasonography (EUS) was then performed showing a hyperechogenic formation with regular and well-defined limits, apparently unrelated to the gastric wall, with a cystic component. No changes in the morphology and thickness of the different layers of the gastric walls were identified. Biopsy puncture with 19G needle from EUS was done, but fragments obtained were inadequate for diagnosis. The patient underwent an exploratory laparoscopy converted to open surgery when a large tumoral mass adhering to the gastric fundus was found surrounding the spleen and left adrenal gland. He was submitted to an en-bloc resection of the mass with partial gastrectomy, splenectomy and left adrenalectomy. The patient’s postoperative course was uneventful. He was discharged 10 days after surgery. Histopathologic gross examination of the surgical specimen showed stomach with a cystic lesion (2 cm) developed in the gastric wall but not related with the gastric mucosa, with an underlying white and firm tissue (5 x 1.8 x 1.8 cm), with areas of serous content, infiltrating the splenic and left adrenal gland (Figure ). The lesion developed in the gastric wall, without communication with the gastric mucosa, which had no significant changes. Histologic evaluation revealed that the cyst had ciliated pseudostratified epithelium, underneath with a muscle layer continuous with the gastric muscular layer (Figure , ). The cyst exhibited ulcerated areas, where an epithelial malignant neoplasm was evident. The tumor was composed of glands of irregular contours, juxtaposed, widely infiltrative and with marked desmoplastic reaction (Figure , ). Phenotypically the tumor displayed strong staining for EMA (MUC1) and CK7, with heterogeneous staining for MUC5AC and MUC6 and no staining for MUC2 and CK20 were registered. Stained slides were observed in a light microscope, Nikon Eclipse 50i, and images obtained using a Nikon-Digital Sight DS-Fi1 camera (Nikon Instruments, Inc., Melville, NY). Eight lymph nodes were isolated without metastatic disease. Lymphovascular and perineural invasion were evident. These aspects were consistent with moderately differentiated pancreatobiliary adenocarcinoma developed in a gastric duplication cyst. The patient was evaluated in multidisciplinary team discussion (MTD) and was proposed for adjuvant chemotherapy (ChT) with modified FOLFIRINOX scheme (5-Fluorouracil 2400 mg/m2 46h continuous infusion; Levofolinate sodium 200 mg/m2 D1, Oxaliplatin 85 mg/m2 D1, Irinotecan 150 mg/m2 D1, 12 cycles, biweekly). At baseline of treatment, tumor markers were negative. Three months after completed ChT, the patient referred an abdominal left pain. The CT scan revealed a nodular lesion with 2.5 x 3 cm adjacent to pancreatic tail and suspect peritoneal implants. The biopsy confirmed that they were metastatic lesions. The patient was discussed again in MTD and was proposed for second-line palliative ChT with nabpaclitaxel (125 mg/m2) plus gemcitabine (1000 mg/m2), D1-8-15, 28/28 days. After 13 months of treatment, the patient had stable disease in the last response evaluation.\n​​​​​​​
A 10-year-old Chinese boy was referred to our Orthodontic Department at Hong Kong University with the chief complaint of delayed eruption of the upper left central incisor. The child was in good health and had no relevant medical history or previous dental trauma. Intra-oral examination showed that he had a mixed dentition, reverse anterior overjet with complete deep bite and clinically missing upper left central incisor (Figures and ).\nRadiography showed all the permanent teeth were present and the possibility of root resorption of the upper lateral incisors (12, 22), caused by mesially erupting position canines could not be ruled out (Figure ).\nA cone-beam CT scan was taken to assess the extent of resorption, if any, and to aid in the creation of a suitable treatment plan. The three-dimensional view from the CT scan (Figures and ) revealed that the crown of the upper right canine was in close proximity to the upper right lateral incisors. The crown tip of the upper left canine was touching the root of the upper left lateral incisors, causing some root resorption, which was confirmed in the sequential transaxial views of the CT scan (Figure ).\nThe treatment objective was to prevent and eliminate any root resorption of the lateral incisors that might be caused by the erupting canines. The treatment plan was extraction of the upper primary first molars and palatal root torque of the lateral incisors by using a specially designed upper fixed lingual appliance (Figure ).\nBecause our patient had a reverse overjet and 100% complete overbite, the conventional labial bracket system could not be used. Therefore, a specially designed upper fixed appliance with a torquing spring was constructed to apply torque to the upper lateral incisors from the palatal side. Tip-edge brackets were bonded to the palatal surface of the upper lateral incisors for tying the palatal arch-wire into the teeth.\nA 19 × 25 stainless steel (ss) wire was soldered to the fixed palatal arch and extended anteriorly to the palatal surface of the lateral incisors. Two torquing springs were attached to the wire to rest on the palatal surface of the lateral incisors.\nThe appliance was cemented onto the upper first molars, and fitted anteriorly into the bracket slot and secured in place by using ligature ties through the vertical slot of the bracket. Prior to cementation the torquing spring was activated to apply a palatal root torque to the upper lateral incisors.\nThen, three months after the treatment, radiography showed that the lateral incisors were in a more upright position and the upper canines were erupting in a more favorable distal direction away from the root of lateral incisors, and no further resorption was evident (Figure ). Our patient was further reviewed and a subsequent radiograph (Figure ) showed that the both canines had erupted further down from the roots of the lateral incisors, and there were no signs of root resorption. Later photographs (Figure ) show the successful eruption of both canines.
A 50-year-old woman had a hard navel mass. She regularly visited the Department of Internal Medicine at our hospital for the treatment of diabetes. In August 2011, she presented to the Department of Dermatology with a main complaint of an umbilical mass; however, she was sent to the Surgical Department because of the diagnosis of an umbilical lesion located deep in the abdomen. The hard mass, which was the size of a thumb, was palpable at her navel. She did not feel any pain.\nAn abdominal computed tomography (CT) scan showed the enhanced tumor; it was 2 cm in diameter and located in the pancreatic tail (Figure ). The contrasting effect was poor compared to normal tissue, which was a finding suggestive of pancreatic cancer. The para-aortic lymph node was not swollen; however, low, enhanced foci were scattered in the spleen, which was a finding suggestive of metastasis.\nThe fluorodeoxyglucose-positron emission tomography (FDG-PET) scan showed swelling in the pancreatic tail with abnormal accumulation (Figure ). This contrasting pattern along with the CT findings suggested pancreatic cancer. Accumulation in the spleen was noted, so we were unable to rule out the possibility of invasion. There was a small granular shadow with slight accumulation suggestive of lymph node metastases.\nSkin thickening and abnormal accumulation in the umbilical region were also noted. Consecutive accumulation was not observed in the peritoneum, which was suggestive of local inflammation, not dissemination. No other abnormality was found.\nThe patient was placed in a supine position for the operation. First, we used Kocher mobilization at the front part of the inferior vena cava, moving toward the anterior surface of left renal vein to back side of the superior mesenteric artery. We confirmed no para-aortic lymph node swelling. The greater omentum was resected from the spleen to the pancreas, transverse colon, and splenic flexure. The inferior mesenteric vein was set aside. The adhesion between the stomach and pancreas was opened. In addition, the posterior gastric vein was separated, and the coronary vein was preserved. The spleen was separated from the retroperitoneum. Next, the left adrenal gland was resected from the pancreas and preserved intact. The pancreas was cut at the anterior of the superior mesenteric artery, and the pancreatic tail, including the tumor, was extracted. The stump and tumor were quick frozen for pathological examination; the pancreatic duct stump was ligated with 5-0 prolene sutures. The patient was diagnosed as having pancreatic cancer, and the pancreatic stump was not malignant. The gastric posterior wall was transected to approximately 80% of the stump width. Single layer anastomosis was performed with 3-0 absorbable sutures (Figure -E). One soft drain was placed under the left diaphragm and the hiatus of Winslow after washing with 2000 mL of saline. The left side of the greater omentum was used to cover the stomach-pancreas anastomotic region. The operative time was 211 min, and the blood loss was 162 mL.\nThe invasive ductal carcinoma of the pancreatic tail, scirrhous, nodular, Infγ , ly0, v1, ne3, mpd(-), s(+), rp(-), PVsp(+), A(-), pcm(-), mdpm(-), and M1(umbilicus) carcinoma, formed the mass (30 × 25 mm) and showed serosa exposure and progress to the outer membrane of the spleen. This mass was a tub1(> tub2)-based tubular, scirrhous adenocarcinoma. It was accompanied by high neurologic and splenic vein invasion. Each excised stump was negative for malignancy.\nCytology of ascites showed that the umbilical region mass, the invasive ductal carcinoma, was a class V adenocarcinoma.\nThe drain was removed after the drain fluid amylase level decreased to 190 IU/L on day 3 from 1595 IU/L on day 1. The patient was discharged without any problems on postoperative day 11.\nWe have followed the patient’s pancreatic stump in the stomach postoperatively for 1 year using a gastric fiber scope. After 1 wk, the stump was massive; however, after 3 mo, the gastric mucosa covered almost the entire stump end. After 1 year, we could not detect the stump in the stomach (Figure -C).
A 42-year-old female, who underwent 18 years ago, extracorporeal shockwave lithotripsy for right kidney stones, presented with six months history of right lumbar region pain associated with dysuria without hematuria. Physical exam showed right-sided abdominal tenderness.\nComputed tomography (CT) urography () showed two masses in the midportion of the right kidney with distinct radiologic appearances. The external mass measuring 24 × 23 mm was a fat containing tumor suggesting AML. The internal tumor was fleshy and large measuring 49 × 40 mm. It deformed the renal contour and demonstrated a predominantly heterogeneous enhancement pattern. There was no evidence of lymph node metastases.\nThe other laboratory findings were within normal limits.\nA right radical nephrectomy was done.\nThe patient had an uneventful postoperative recovery, and no neoadjuvant treatment has been administered. Six months after surgery, the patient is healthy without signs of tumor recurrence or distant metastasis.\nOn gross examination (), the kidney was slightly enlarged weighing 280 g and measuring 130 × 70 × 65 mm. The cut surface revealed 2 masses in the midportion. The largest mass of 47 × 40 mm was encapsulated and firm with a heterogeneous appearance, composed of golden yellow soft areas with hemorrhagic changes. The smaller mass measuring 25 × 20 mm was well defined and had yellowish, glistening cut surface. It was located at 3 cm from the first lesion.\nMicroscopic examination of the largest mass showed a malignant proliferation composed of cells with distinct cell membranes, optically clear cytoplasm, and rounded nuclei slightly larger than a red cell with inconspicuous nucleoli (consistent with Fuhrman grade 1). The cells were mainly arranged in sheets and compact nests surrounded by a regular network of thin-walled blood vessels (). These features were consistent with the diagnosis of CCRCC.\nThe smaller lesion corresponded histologically to an AML composed of aggregates of thick-walled blood vessels, admixed with large mature fat cells and smooth muscle cells ().\nIn the immunohistochemical study, AML showed positive immunostaining for melanocytic markers (HMB-45 and Melan-A) and for smooth muscle markers (smooth muscle actin and H-caldesmon) ().
A 64-year-old male with multiple cardiovascular risk factors (arterial hypertension, dyslipidemia, former smoking habit and obesity, with a body mass index of 33 kg/m2) was admitted to our center for an elective coronary angiography after the onset of typical angina with a positive stress test.\nCoronary angiography revealed a significant multivessel disease (critical stenosis of the proximal left anterior descending artery and chronic total occlusion of the left circumflex artery, with collateral circulation from the right coronary artery), for which a surgical revascularization was planned [].\nAs preoperative workup, the patient underwent transthoracic echocardiography (TTE), with poor acoustic window, that showed a normal biventricular systolic function and a significant mitral regurgitation with an eccentric jet of unclear mechanism [].\nFor a better morphological definition and quantification of mitral regurgitation, in a patient candidate for a cardiac surgery, we performed a transesophageal echocardiography (TEE). This examination was inconclusive because of the lack of adequate visualization of the cardiac structures (only the descending thoracic aorta could be well seen).\nThus, to better quantify the degree of mitral regurgitation and for a further diagnostic investigation in order to clarify the inadequate TEE window, we decided to perform a CMR. Using phase-contrast flow sequences [], we documented a severe functional mitral regurgitation (effective regurgitant orifice [ERO] 0.36 cm2, regurgitant volume 32 ml), as a result of tethering of the valve leaflets in the presence of a scar in the basal part of the posterior wall []. In addition, in HASTE and VIBES sequence images, we documented a giant hiatus hernia with gastric sliding into the thorax []. Other additional findings were an azygos lobe in the right lung and multiple renal cysts (in particular a voluminous one in the upper pole of the right kidney) [].\nAfter the results of CMR, the initial surgical strategy changed and the patient underwent a combined intervention of coronary artery bypass grafting and mitral valve annuloplasty. Therefore, these results helped the surgeon to plan the ideal cardiac intervention and the echocardiographer to explain the difficult visualization of cardiac structures during TEE.
A 56-year-old man with a history of rectal adenocarcinoma presented for low anterior resection and creation of diverting loop ileostomy. The surgical service requested epidural analgesia for assistance with post-operative pain management. Following uneventful thoracic epidural placement and negative catheter aspiration, a test dose of 1.5% lidocaine with 5 mcg/ml epinephrine was administered. This resulted in a transient increase in heart rate from approximately 80 beats per minute (BPM) to 115 BPM. There was no change in non-invasive blood pressure measurement and the patient remained asymptomatic. Attempts to aspirate blood through the epidural catheter failed to demonstrate any return of any fluid. Given the transient rise in heart rate, lack of change in blood pressure, negative aspiration, and lack of symptoms reported by the patient, a repeat test dose created by the provider was administered. Following administration of a second test dose, the patient experienced supraventricular tachycardia with heart rates of 200-210 BPM for approximately 2 minutes. This rhythm then converted to atrial fibrillation with rapid ventricular response with heart rate of 150-170 BPM. Initial conservative treatment with esmolol boluses was ineffective and the patient's blood pressure decreased from approximately 140/70 mm Hg to approximately 90/50 mm Hg. He denied chest pressure or other cardiac symptoms, but 12 lead ECG revealed marked ST segment depression in the lateral leads. The patient was sedated with 1 mg midazolam and 30 mg propofol prior to 120 joule synchronized cardioversion at the bedside in the preoperative area. The patient returned to sinus rhythm and ST depression resolved. Later, evaluation of medication administration revealed that the second epidural test dose inadvertently contained 100 mcg epinephrine instead of the intended 10 mcg dose.\nThe surgeon and patient's family were updated, and surgery was postponed to allow for a formal cardiology evaluation. The patient received a transthoracic echocardiogram which revealed normal heart structure and function. The patient returned 3 days later and underwent an uneventful low anterior resection with diverting loop ileostomy. He declined pre-operative epidural placement but consented to post-operative bilateral quadratus lumborum blockade. The patient's post-operative course was complicated by ileus, but he had no further cardiac complications. He was discharged 8 days post the operation. Written informed consent for treatment and patient's approval for the publication of results were obtained.
Twelve-year-old boy presented with very disruptive symptoms of rhinitis with significant nasal obstruction as well as sneezing, rhinorrhoea and very disturbing nasal and ocular pruritus. These symptoms developed every year during the summer months and were persistent and severe, affecting his ability to sleep and his performance at school. His exam marks were lower in his summer examinations compared to those earlier in the year. He also felt that his nose problems were restricting his sport and social activities during the period when the weather was good; he liked playing outside. He was tested for different airborne allergens and both skin and specific IgE testing showed sensitization to grass pollen confirming grass pollen allergy. The previous years, he had been prescribed loratadine, intranasal mometasone furoate, montelukast and sodium cromoglicate eye drops, which he was taking. Despite good adherence, he continued to have poor disease control.\nGiven his continued symptoms, that were impacting on this quality of life, despite optimal pharmacotherapy, he was started on sublingual immunotherapy to grass pollen. He took the first dose in clinic and continued with the treatment at home. He initially had some local pruritus but this settled after a couple of weeks. One year into this treatment, he was already feeling some improvement and was able to reduce the medication he was taking to loratadine only.\nAllergen-specific immunotherapy (IT) is the only disease-modifying treatment for allergic rhinitis. It is able to change the natural history of this condition and to provide long-term remission [,]. It is indicated in patients over 5 years old with demonstrable IgE to clinically relevant allergens, particularly in patients where pharmacological treatment has failed to control symptoms []. Since he was having troublesome symptoms despite maximum pharmacological therapy and the symptoms were due to grass pollen exposure to which he had detectable IgE, he was a good candidate for this treatment. There are standardized extracts to grass pollen commercially available to administer via the subcutaneous or the sublingual route [,]. Although there are very few head-to-head studies comparing subcutaneous immunotherapy (SCIT) and sublingual immunotherapy (SLIT), both forms are effective if appropriately used [,]. In terms of safety, SCIT more frequently causes systemic adverse events while SLIT tends to cause more local side effects, which are usually mild and resolve with continuation of treatment [,]. Severe adverse events are commonly associated with uncontrolled asthma, high allergen exposure during therapy, concomitant diseases such as severe infections and inexperienced health care staff. Premedication with an antihistamine may decrease the rate of adverse effects []. The decision on whether to do SLIT or SCIT depends on a variety of factors, including patient’s preference about home-based versus hospital-based treatment, fear of injections, costs and concordance.[]. In children, SLIT is more widely accepted but may have lower patient adherence []. Although SLIT is given at home, the first dose should be given at the doctor’s office. This is also the opportunity to give detailed instructions about how to administer the treatment and about the precautions to be taken. Patients should be informed about possible adverse reactions and about the ways to treat them. Apart from the effectiveness in reducing symptoms and medication use, another potential advantage of allergen-specific immunotherapy is its preventative effects in reducing asthma and the development of further allergic sensitizations [-]. This is particularly important in the pediatric age groups. When clinically indicated, IT should be started early in the disease process, before significant remodelling and fixed airway obstruction has developed in the case of patients with asthma. As allergen-specific immunotherapy is the only disease-modifying treatment available for allergic rhinitis and respiratory allergy, it may be considered as a therapeutic option even before trying maximal therapy, depending on individual cases, clinical practice and finance.
The patient was a 57-year-old male, with ESRD secondary to diabetes and hypertension, on hemodialysis for 20 months. He was evaluated to undergo living unrelated donor kidney transplant. The donor was a 54-year-old female with unremarkable past medical history. She was medically and surgically cleared after full assessment. Preoperative computed tomography (CT) angiography for the donor revealed a small complex cyst (Bosniak IIF), a short right renal vein (2 cm), and a right renal artery with an early bifurcation of an upper pole artery. Surgical approach deemed best by the living donor selection committee was to remove the right kidney with the complex cyst via laparoscopic approach.\nThe risks of surgery and potential complications were explained to both donor and recipient. Written informed consent was obtained prior to surgery from both patients.\nA standard hand-assisted laparoscopic living donor nephrectomy (LLDN) was performed to retrieve the right kidney and the right ovarian vein for reconstruction. The length of the donor renal vein was reduced by 10-15 mm due to the standard use of vascular stapler device to control the renal vein stump.\nStatus after procurement upon visualization in the back table is that the length of the donor renal vein was 3 mm. In order to elongate the vein, the donor ovarian vein was dissected and used for reconstruction. It was opened longitudinally, folded over, and anastomosed to the donor renal vein using end-to-end anastomosis with an 8-0 Prolene ().\nThe donor kidney had a renal artery with early bifurcation of an upper pole artery. After mobilization of the inferior vena cava as much as possible, the endovascular stapler was placed after the bifurcation from the renal artery and the length of the vessel was reduced by 10-15 mm. At the back table the donor kidney presented two individual arteries: the main renal artery and the short upper pole arterial branch. Consequently, the recipient IEA was used as an extension graft to perform an end-to-side anastomosis between the upper pole arterial branch and the main renal artery using 8-0 Prolene ().\nThe complex cyst was dissected all the way down into the calyces and substantial margin of healthy parenchyma was removed. Distal margin was sent to pathology to rule out malignancy. The calyces and the renal parenchyma were oversewed with 5-0 PDS and the edges of the defect with U-stitch of 4-0 PDS using pledget ().\nThe extended renal vein and the main renal artery were anastomosed end-to-side to the external iliac vein (EIV) and artery (EIA) respectively, using 6-0 Prolene ().\nWe performed an extravesical ureteroneocystostomy and a Jackson Pratt drain was placed. The warm ischemia time was 33 minutes. Postoperative course was uncomplicated and the patient has maintained adequate renal function with stable serum creatinine (1.32 mg/dl) at 5-month follow-up. The final pathology report and the intraoperative frozen section of the margin were negative for malignancy.
An 83 year old female, who had been hospitalized for years due to schizophrenia, complained of vomiting and diarrhea for 2 days. She had no history of diabetes mellitus, neurogenic bladder, or previous antimicrobiotic therapy, but had a urinary catheter in place for a considerable period since she was bedridden for many years. The diagnosis was acute enterocolitis and intravenous fluid was administered. After she developed a chill and lower abdominal pain with muscular defense, she was diagnosed as surgical acute abdomen and transferred to our emergency department.\nOn arrival, her body temperature was 38.2°C, blood pressure was 150/70 mmHg with a regular heart rhythm of 110 beats/min, and her respiratory rate was 22 breaths/min. Physical examination revealed tenderness over the lower abdomen with muscular defense, rebound tenderness, and loss of bowel sounds. Laboratory examination revealed a normal white blood cell count with elevated C-reactive protein (24.9 mg/dL). Urinalysis was unremarkable. An X-ray of the abdomen showed gas collection in the pelvic cavity, and CT scans of the abdomen revealed intraperitoneal fluid collection and abnormal gas accumulation in the pelvic space. The distribution of this abnormal gas was between the abdominal wall and retroperitoneal cavity including the urinary bladder wall, and its association with the intraperitoneal cavity was unclear ().\nIn accordance with the history, physical examinations, and radiological findings, the patient was diagnosed as acute peritonitis with possible perforation or penetration of the lower intestine, and exploratory laparotomy was performed. At laparotomy, severe inflammatory changes were observed in the peritoneum from just below the umbilicus to the lower abdomen (). Ascites in the peritoneal cavity was slightly turbid, but without a stool smell and there was almost no intraperitoneal inflammation. Other abdominal structures were intact, however, the bladder was swollen. When the bladder wall was pushed from the peritoneal cavity side, an unusual crackling sensation was felt and purulent urine was excreted via the urethral catheter. Dull dissection of the retroperitoneal cavity toward the bladder wall showed markedly loose connective tissue around the bladder due to severe emphysematous change, and the boundary with the bladder wall was unclear. These findings indicated emphysematous cystitis. The patient was placed on 1.5 g/day of intravenous doripenem for broad antibiotic coverage. Afterwards, K. pneumoniae was isolated from cultures of peritoneal fluid, urine, and blood (). Antimicrobiotic therapy was changed to intravenous administration of ceftriaxone at 2.0 g/day for 2 weeks. The patient recovered uneventfully and follow-up CT imaging after 3 weeks demonstrated resolution of the bladder wall emphysema.
This 18-year-old male patient presented at the Oral and Craniomaxillofacial Surgery Clinic to consider surgical treatment options for reducing an enlarged tumor of the back. The patient had more than six café au lait spots on the trunk and extremities, axial and inguinal freckling and several cutaneous tumors that were slightly raised above the level of the skin. The patient had no physical discomfort, no motor or sensitive deficits. The patient stated that he had been operated 2 years earlier on a tumor of the back in another hospital. More detailed information was not available. Despite this previous treatment of the tumor, the remaining tumor mass disturbed him both physically and in his self-perception. The patient stated that the tumor had been growing again since the first operation.\nOn the back there was a tumorous protrusion of the intact skin with a maximum above the spine, which extended from the lower thoracic region close to the edge of the pelvis (Figure 1 A ). The tumor was clearly prominent under tight-fitting clothing. The skin in this area was darker pigmented throughout the lumbar region and showed hirsutism. The tumor was insensitive to touch and pressure, showed no fluctuation on palpation, and the covering skin moved with the tumor.\nB-scan ultrasound revealed an inhomogeneous mass with focal, partly string-like reflections inside the space occupying lesion. Borders were poorly defined and the tumor mass reached to the spinous processes of the spine. The tumor appeared as solid mass and contained no cavities suggestive of necrosis. The tumor was resected in general anesthesia. When the lesion was exposed, a black pigmentation became apparent, which was partially arranged in a stripe-like pattern and frayed at the edges (Figure 2 ). The tumor was resected and the contour of the back reshaped. Despite dense suturing of the wound margins a hematoma developed, which was emptied. Secondary wound healing took 21 days and led to a stable healed wound (Figure 1 B ). There was no movement restriction of the patient after the wound had healed.\nUpon neuropathological investigation a spindle-shaped, 22x9x2 cm3 large skin sample with centrally located 6 cm long scar was seen. Cutting the skin exposed white and slightly greasy tissue on both sides of the scar with spotty brown-black pigmentation.\nHistological examination revealed a diffusely grown neoplasia of medium to high cellular density in the subepidermal connective tissue, consisting of roundish and oblong cells with delicate cytoplasmic extensions and slightly pleomorphic, small, round-oval, sometimes comma-shaped nuclei. The cells showed different degrees of pigmentation. Repeatedly, pseudo-Meissner corpuscles were observed. There was no evidence of mitoses and no Turnbull-positive hemosiderin pigment was detected. Immunohistochemistry demonstrated labeling of the tumor cells with antibodies against S100-protein and melan-A and to a lesser extent also with antibodies against HMB45. The Ki-67-proliferation index was less than 3%.\nA subepidermal diffusely grown pigmented (melanotic) neurofibroma WHO grade I was diagnosed (Figure 3 ).
A 66-year-old woman presented with a non-tender mass of the right cheek, which slowly increased in its size over 3 years. Three years ago, she had visited with the same lesion. However, she had been lost to follow-up after computed tomography (CT), and the mass had gradually expanded over the time. Physical examination revealed a hard, mobile round mass with about 2 cm diameter. The overlying skin and cheek sensation was normal. She had a history of left frontal bone and orbital wall fracture due to traffic accident 7 years ago. Findings of fine needle aspiration biopsy showed clusters of epithelioid cells suggesting benign tumor such as salivary gland tumor or skin adnexal tumor. Nasal cavity was normal on endoscopic examination. Preoperative CT scan revealed a heterogenously enhanced mass in the subcutaneous fat layer of the right cheek, with well defined margin (). Tumor diameter has increased about 2 times compared to that of 3 years ago. There was no evidence of any intracranial mass lesion except encephalomalacia of the frontal lobe.\nUnder general anesthesia, she underwent complete mass excision via lateral rhinotomy incision. Intraoperative frozen biopsy was compatible with benign lesion. The tumor was surrounded by unclear and thin fibrous capsule, and tethered to the surrounding soft tissue. The relationships of the tumor with the infraorbital nerve was not identified. There was no postoperative morbidity such as cheek sensory defect. She was discharged three days after surgery. She had shown no signs of recurrence during a follow-up period of 18 months.\nMicroscopic examination revealed a poorly-defined tumor which is haphazardly admixed with mature adipose tissue and normal skeletal muscle. The tumor was composed of uniform, epithelioid cells showing delicate chromatin pattern, and characteristic meningothelial whorls were frequently observed (). There were several foci of psammoma bodies within meningothelial whorls. Neither mitotic figures nor significant nuclear pleomorphism was identified. Immunohistochemical studies revealed that tumor cells were reactive for epithelial membranous antigen (EMA), focally positive for S-100, but negative for HMB-45, smooth muscle actin, CD31, CD34, and CD68. Additionally, Ki-67 was found to have low expression index. These histological and immunohistochemical findings were compatible with meningothelial meningioma. This case report was approved by Institutional Review Board of Hallym University Sacred Heart Hospital.
A 34-year-old woman with a history of urachal carcinoma was referred to our department (Department of Liver and General Surgery) in January 2015 in order to surgically treat a disease relapse presenting with a liver metastasis. The patient was an otherwise healthy woman. The first symptoms of the disease occurred in December 2011 and included urinary frequency, dysuria, and episodic gross hematuria. In January 2012, the patient sought medical attention. The primary care physician diagnosed urinary tract infection and ordered antibiotics. After two months, an abdominal ultrasound was performed, however with an empty bladder, and the disease focus was not recognized. It was not until April 2012 that the patient came directly to the emergency department and was for the first time consulted by a urologist. At that point, the diagnosis of urinary bladder tumor was made. A CT scan showed a pathologic mass in the region of the bladder dome extending toward the umbilicus (Fig. ) which is a typical appearance of urachal carcinoma []. It was adherent to the sigmoid colon, and there were signs of the surrounding adipose tissue and peritoneum involvement. No sites of distant metastases on abdominal CT scan and chest X-ray were noted.\nIn June 2012, the patient underwent radical cystectomy including hysterectomy, bilateral adnexectomy, partial resection of the sigmoid colon, and partial resection of the rectus abdominis muscle with the fascia, skin, and umbilicus. A urinary diversion with a Studer-type orthotopic ileal neobladder was constructed. Intraoperative histologic examination of two ileal lymph nodes was negative. However, a post-operative examination of these lymph nodes revealed one metastatic focus. The full specimen was described in pathology report as a 22 × 19 × 17 cm in size, with tumor extending between the uterus and umbilicus involving the urinary bladder and extending beyond its wall. On microscopic examination, a mucous-producing adenocarcinoma consistent with urachal carcinoma was diagnosed. The involvement of the sigmoid colon was caused by an inflammatory response. Other resected organs as well as surgical margins were free of neoplastic infiltration. The patient received four cycles of adjuvant chemotherapy with paclitaxel and carboplatin.\nA follow-up CT scan performed a year after surgery (July 2013) showed no signs of disease recurrence. In November 2014, another CT scan revealed a hypodense mass in the right liver lobe of max 40 mm in diameter. A collection of fluid in the region of the right iliac vessels with calcifications was also noted (in a region where in previous imaging studies a simple lymphocele was described). A subsequent PET-CT scan of entire body proved a high probability of disease recurrence in the liver and in the region of the right iliac vessels (Fig. ). A biopsy of the liver mass confirmed a focus of metastatic disease. After presentation of possible therapeutic options to the patient, she chose a surgical treatment. The surgery took place in January 2015. Intraoperative findings with the use of ultrasonography included a tumor of max 80 mm in diameter in the right liver lobe with a small satellite focus on the liver phrenic surface. Despite the probability of local recurrence in the pelvis, a right hemihepatectomy was performed. This decision was based on a large tumor size and a possible expansion into the vena cava inferior and liver hilum (Fig. ). The pathology report revealed a metastatic urachal carcinoma and a positive surgical margin. Magnetic resonance imaging performed a month after the surgery confirmed enlargement of the pathologic mass in the pelvis. No other sites of the disease were noted. Patient was qualified to a second-stage surgical treatment and underwent an excision of the tumor mass in the region of the right iliac vessels in April 2015; however, complete resection has not been achieved.\nFollowing the surgery, the patient received three cycles of adjuvant gemcitabine-based chemotherapy. Subsequent imaging studies showed gradual disease progression. The patient died of the disease in March 2016.
A 92-year-old man was referred to the emergency department by his general practitioner because of suspicion of pneumonia. The patient reported increasing dyspnoea and bilateral pain at the thoracic base. Four weeks earlier he fell from the stairs and since then he suffered from mid-dorsal back pain. Physical examination of the lungs revealed tachypnoea, decreased breath sounds on the left side and unequal chest rise. Heart auscultation demonstrated regular rate tachycardia (110 bpm). The jugular venous pressure was raised. Abdominal examination showed a distended abdomen with hypoperistalsis, but no tenderness. On a chest x-ray a left tension pneumothorax was seen with pleural effusion on the left side and three recent basal dorsolateral rib fractures. Surprisingly a pneumoperitoneum was also visible on the chest x-ray (Figure ). Needle decompression was immediately executed. Subsequently an apical chest tube was inserted on the left side and approximately 500 ml of serous and bloody fluid was drained. A computed tomography was made in search of the origin of intra-abdominal air. A left posterolateral diaphragmatic rupture was found. In respect to the patient's age a conservative approach was chosen. He was admitted to the intensive care unit and a second basal chest tube was inserted on the left side and broad spectrum antibiotics were administered. The chest tubes were kept on suction (-10 cm H2O) to accelerate the rate of healing. On the seventh day brown liquid was observed from the basal chest tube. A new computed tomography was performed and this showed herniation of the transverse colon through the hernia defect in the left diaphragm (Figure ). The basal chest tube had perforated the colon, thus creating a left fecopneumothorax. A laparoscopic repair was planned. During this procedure the herniated and perforated part of the colon was removed, a transdiaphragmatic lavage was undertaken and the omentum was used to close the diaphragmatic defect (Figures and ). A mesh or sutures were not used since the abdomen was contaminated with feces. The 92-year-old-patient deceased on the fourth post-operative day due to respiratory insufficiency. Both the patient and family were in consent for abstinence from further invasive therapy.
A 34-year-old right-hand-dominant male presented to the emergency department with acute left wrist pain and diminished forearm rotation. The patient stated that he had been in an altercation the evening prior and had woken up the next morning without any recollection of the mechanism of injury. He had no relevant previous medical or surgical history. He denied any previous injuries to his left wrist or forearm.\nOn physical examination, the patient complained of pain over the ulnar aspect of the left wrist. The dorsal prominence of the ulnar head was no longer appreciated and was instead palpable volarly. There were no open skin lacerations or wounds. The patient was neurovascularly intact with the appropriate function of the anterior interosseous, posterior interosseous, and ulnar nerves as evident by intact cardinal hand movements. The wrist was mildly swollen, and passive and active range of motion was limited.\nInitial radiographs were obtained (Figure ), which demonstrated abnormal overlap of the distal ulna with respect to its normal articulation with the distal radius on posteroanterior view and clear volar dislocation of the ulna on the lateral view. Additionally, left forearm and elbow radiographs demonstrated no fractures or dislocations.\nA CT scan was also obtained to look for any associated injuries or fractures. CT confirmed the isolated volar dislocation of the ulnar head from the distal radial sigmoid notch. This was best seen on the axial cut at the level of the DRUJ (Figure ). A 3D reconstruction of the scans was also performed to obtain additional information on the injury and aid with closed reduction and treatment (Figure ).\nAfter receiving informed consent from the patient, conscious sedation was performed with the assistance of the emergency room provider. Closed reduction of the left volar DRUJ dislocation was successful by applying slight distraction to the radius and ulna and applying a dorsally directed force to the ulnar head. As the wrist was slightly pronated with continuous direct pressure being maintained, an audible click was heard, and reduction was appreciated. Once the DRUJ was reduced, the wrist was passively taken through the full normal range of motion including flexion, extension, and pronosupination. Post-reduction stability was assessed throughout motion and no instability was noted. Range of motion and stability was comparable to the contralateral uninjured wrist after reduction. The patient was immobilized in neutral forearm rotation and 90 degrees of elbow flexion using a well-molded sugar tong fiberglass splint. Post-reduction radiographs were obtained and confirmed the successful reduction of the DRUJ (Figure ).\nThe patient was seen for a follow-up in the orthopedic clinic the subsequent week, and repeat radiographs of the left wrist demonstrated a maintained reduction of the DRUJ. The patient's pain had improved, and he was instructed to keep the wrist immobilized for another four weeks. Two weeks later, the patient presented once again to the emergency department after his original fiberglass splint had got wet and loosened, requiring a new splint to be applied. Repeat radiographs again showed a concentric reduction. The patient’s splint got wet and loosened once again at six weeks from the date of his original injury, and he was transitioned to a prefabricated removable wrist splint. At that time, his pain had continued to improve and he had full range of motion of the left wrist. The radiographs from the outside emergency department during this last encounter demonstrating maintenance of reduction without recurrence of volar ulnar head dislocation are presented below (Figure ). Unfortunately, this patient was lost to long-term orthopedic follow-up due to a period of incarceration, but he has had subsequent medical encounters within our health system nearly three years after his original injury without noted residual left wrist pain, diminished range of motion, or any additional wrist complaints, and a satisfactory clinical recovery can be inferred.
We present a 64-year-old Hispanic male with Rai Stage II, IgH mutated, and trisomy 12 positive CLL, diagnosed 13 years prior to admission. He was initially observed for 3 years and then received first-line fludarabine, cyclophosphamide, and rituximab (FCR) for 3 cycles with good response. He remained on observation for 4 additional years and then received an additional 4 cycles of FCR followed by 5 cycles of maintenance rituximab. Five years later, he was found to have 17p deleted recurrent disease and he was started on ibrutinib with good response. He presented to an outside facility with acute onset altered mental status after stopping ibrutinib 2 months earlier due to financial constraints. Cerebrospinal fluid (CSF) analysis was initially concerning for CLL infiltration of the CNS with neoplastic-appearing lymphocytes identified by cytomorphology; however, serological tests for West Nile virus indicated acute infection based on positive IgM and negative IgG; however, PCR could not be performed. The patient received therapy for presumed CNS involvement by CLL with intrathecal methotrexate along with intravenous rituximab and methylprednisolone before being transferred to our institution. On admission, his complete blood count revealed leukocytosis with a normal differential and thrombocytopenia, after reviewing the patients' historical trends, we could discern that the platelet count had been within normal limits in the past and had likely decreased due to the acute illness; additionally, we can appreciate the increasing trend in WBCs until the start of treatment around 10 years prior to this admission (). Peripheral blood smear showed normal platelet morphology and moderate leukocytosis with lymphocytosis with coarse, block-like chromatin pattern concerning for prolymphocytoid transformation which had not been present previously. Bone marrow biopsy revealed hypercellularity with diffuse infiltrate of the medium to large-sized mononuclear cells with irregular nuclear contours and prominent nucleoli similar to that seen in the peripheral blood smear (). Repeat CSF studies at our hospital showed lymphocytes accounting for 49% of total events per flow cytometry, positive for CD19, CD20, CD5, and CD23, with lambda light chain restriction, and negative for CD10 and FMC-7 (). The morphology of lymphocytes in CSF was consistent with neoplasia; however, given the admixture of reactive lymphocytes and the presence of known CNS infection, it was deemed that this most likely represented peripheral blood contamination and reactive infiltration of neoplastic lymphocytes.\nHe was treated supportively during his hospital stay and improved without any further CNS-directed therapy. Ibrutinib was later reinstated after the resolution of thrombocytopenia and BTK mutational testing proved negative. The patient continued to improve during outpatient follow-up, prolymphocitoid morphology disappeared from peripheral smear, and brain MRI showed no evidence of CNS disease.
A 11-year-old boy presented to the outpatient clinic with complaints of pain, deformity and shortening of the left arm (Fig. ). Physical examination revealed pathologic motion at the proximal part of the left arm and 8 cm shortening of the left upper extremity compared to the opposite side. According to his past medical history, he had admitted to another institution 4 years before due to pain, swelling and deformity on his left shoulder and upper arm. An expansile, lobulated lytic lesion in the proximal metaphysis of the humerus had been detected on radiographic evaluation (Fig. ). He had been initially treated by en bloc resection and reconstruction with cortical strut allograft in this center (Fig. a). The histopathological results of the tissue samples had been found to be consistent with ABC. Serial follow-up radiographs showed failure of the fixation with nonunion of the proximal humerus (Fig. b). Second surgical intervention including excision of the graft material and implants had been applied in the same center, 1 year after the index operation. The defect area had been filled with calcium phosphate allografts, and patient’s arm had been immobilized in a sling. However, union of the humerus could not be achieved.\nRadiographs at the presentation to the author’s institution revealed evidence of proximal humerus nonunion with a large defect (Fig. a). His shoulder range of motion was completely restricted with unlimited elbow motion. His neurovascular examination was normal.\nSurgery was performed under general anesthesia. The patient was positioned at beach chair position, and deltopectoral exposure over the old scar tissues was used to reach the humerus. Curettage of the dead space, which included the previously inserted allografts, was performed carefully. However, it could not be possible to remove all allograft materials completely due to their adhesions to the surrounding tissues. Then, reconstruction of the defective area with fibular autograft was planned, and the length of the fibular graft was decided. After exposing the contralateral fibular bone, subperiosteal resection of the fibular graft was made under tourniquet control (Fig. c). Care was taken to preserve the periosteum in order to provide the opportunity of rebuilding the fibula in the future. The ends of the fibular graft were countered to the shape of the medullary canal of the humerus. The fibula was initially inserted into the distal medullary canal of the humerus and then was placed into the proximal humeral metaphysis. The fixation of the fibular graft was secured by one K wire that was inserted intramedullary from proximal to distal (Fig. b).\nHe had an uneventful postoperative course without any postoperative complications. Leg immobilization was not applied. During the postoperative follow-up period, the authors did not observe any nerve injury or palsy in the upper and lower extremities. Immobilization of the left shoulder and arm in a velpou bandage was applied for a period of 12 weeks. At the third-month follow-up, graft incorporation was observed in the distal part of the fibular graft; however, proximal part did not show adequate healing on radiographs (Fig. d). Therefore, additional immobilization in a sling for 3 months was advised to the patient and his family. However, the patient was lost to follow-up, and the family did not return to the callings.\nThe patient readmitted to the author’s institution at the 12th month postoperatively. Physical examination revealed the same findings at the initial presentation and radiographs showed failure of the fibular graft fixation and nonunion of the humerus (Fig. ). His family stated that they did not comply with author’s suggestions, and the patient started to move his arm without using the sling after 3rd month postoperatively. Second surgical procedure was offered to the patients and family. However, they declined surgery. Written informed consent was obtained from the patient’s family for the publication of this case report and any accompanying images.
A 64-year-old white male, resident in Rio de Janeiro, has been managing our hospital due to insulin dependent diabetes mellitus, arterial hypertension, chronic coronary disease and renal failure. In August of 2011, he began hemodialysis in our hospital as his renal glomerular function became worse. In March 2012, one month after implanting a permcath catheter he came for the emergency department with signs of infection to elucidate. The results of the microscopic examination of urinary sediment showed 15 to 20 white blood cells (WBC), and so cephalexin was begun to treat a possible urinary infection. However, three days after the antibiotic therapy was begun the urinary sample did not show any bacterial growth. The patient returned with signs of systemic inflammatory response syndrome. A clinical examination of the patient showed an inflammation at the insertion site of the hemodialysis catheter. The patient was admitted in our critical care unit on 7 March 2012 with suspicion of CRBSI. The catheter was removed and another was implanted in his right femoral vein. Two samples of peripheral blood were sent for culture and empiric therapy with vancomycin and piperacillin-tazobactam was initiated within one hour of admittance to the ICU. The microbiology analysis of blood samples revealed MRSA. The therapy was changed to daptomycin on 12 March 2012. After 30 doses of daptomycin intravenously based on his glomerular filtrate rate (GFR), the patient was discharged from the hospital on 18 April.\nOn 9 May 2012, 20 days after the last dose of daptomycin, he returned to the hemodialysis department with a respiratory syndrome. The chest computed tomography demonstrated signs of pneumonia. Therefore, three samples of peripheral blood were sent to the hospital laboratory. Antibiotic therapy (vancomycin, meropenem and trimethoprim-sulfamethoxazole) was initiated according to the microbiology data. The vascular catheter was withdrawn because there was free purulent secretion at the insertion site. Once again, a new catheter was implanted, this time, in the right femoral vein for hemodialysis therapy.\nThe new MRSA isolate from the blood samples showed a multidrug resistance pattern. The isolate was resistant to ciprofloxacin, rifampin, chloramphenicol, clindamycin and erythromycin but showed susceptibility to linezolid, quinupristin-dalfopristin, tetracycline and trimethoprim-sulfamethoxazole by the disk-diffusion test. The minimum inhibitory concentration (MIC) assessed by broth microdilution method showed MICs of >256 μg/mL, 4 μg/mL and 8 μg/mL for oxacillin, vancomycin and daptomycin, respectively. The SCCmec typing [], pulsed field gel electrophoresis [] and multilocus sequence typing (MLST) [] revealed that the isolate belonged to the SCCmec II/USA100/ST5 lineage. After 3 months in hospital, the patient died from a hemorrhagic stroke and pneumonia associated mechanical ventilation due to Acinetobacter baumanni.
A 43-year-old woman was admitted to a clinic with right hypochondriac pain in April 2012. Upper gastrointestinal endoscopy was performed and detected no abnormalities. Her symptom continued with weight loss. Abdominal ultrasonography was performed and found a pancreatic cancer. She was admitted to our hospital in October 2012. Computed tomography (CT) showed a pancreatic head cancer with multiple hepatic metastases. It also revealed irregular duodenal stenosis, suggesting direct cancer infiltration into the second portion of the duodenum (fig. ). Percutaneous needle aspiration was performed to the pancreatic tumor. The pathological diagnosis was adenocarcinoma (fig. ). Laboratory findings on admission showed mild anemia and coagulation abnormalities. Tumor markers were markedly elevated (table ); CA19-9 was extremely high (>100,000 U/ml).\nThe patient's prognosis was considered extremely poor. Comparison of the CT findings on admission with those at the primary clinic 2 weeks previously demonstrated rapid tumor progression; the hepatic metastases had increased in size by approximately 15%. We concluded that we might have no chance to treat her with second-line chemotherapy if first-line chemotherapy failed. Therefore, we selected combination therapy with gemcitabine and S-1 (GS therapy), currently the therapy with the highest antitumor effect in Japan.\nMelena occurred just before the initiation of chemotherapy. Subsequent upper gastrointestinal endoscopy was performed and revealed a giant ulcer 3 cm in diameter with oozing. We found neither arterial hemorrhage nor exposed blood vessels (fig. ). Her anemia did not deteriorate without any endoscopic hemostatic procedure and she was eligible for the scheduled chemotherapy. Melena occurred again on day 5 of chemotherapy with progressive anemia from 9.5 to 7.2 g/dl. Gastrointestinal endoscopy could not be performed because she rejected it. Total parenteral nutrition was started with a blood transfusion of 4 units of packed red blood cells. We presented the treatment option including gastrointestinal bypass surgery to her, but she selected to continue chemotherapy. The anemia did not worsen after all and a liquid diet was started on day 20. She showed no adverse events of grade 2 or greater severity, such as thrombocytopenia or neutropenia, according to CTCAE v4. She was discharged with a low risk of rebleeding on day 38.\nGS therapy was continued after discharge. CT was performed at month 2 and showed that the primary lesion and the hepatic metastases had been reduced, meeting the criteria for a partial response (RECIST v1.1). The CT findings at month 6 showed re-enlargement of the primary lesion, an increase in the number and size of hepatic metastases and the appearance of ascites. The patient took best supportive care. She died 7.5 months after the initiation of therapy.\nThe patient's course after admission is presented in supplementary figure (see ).
The 58-year-old female patient was hospitalized due to recurrent episodes of upper abdominal pain, anemia, weight loss, fatigue and fever attacks. Under suspicion of a duodenal perforation by a lymphoma or GIST, seen in an ultrasound examination, the patient was transferred to our clinic.\nPhysical examination of the patient with no history of preexisting diseases revealed a palpable mass in the right upper abdominal quadrant. Hemoglobin was 90 g/l.\nUpper endoscopy revealed a large necrotic cavity in the inferior part of the duodenum. Multiple biopsies taken from the tumor mass confirmed the suspicion of a duodenal GIST. PET-CT scan showed a 9 × 9 × 15 cm tumor mass arising from the duodenum with a maximal standard uptake value (SUV) of 15,5. The tumor had contact to the pancreatic caput and led to compression of the inferior caval vein and the inferior mesenterial vein. The portal vein as well as the common hepatic artery and the superior mesenterial artery showed no signs of infiltration or compression. Furthermore PET-CT did not reveal any signs of metastasis. According to a neoadjuvant approach preoperative therapy with imatinib (Gleevec, Novartis, Basel, Switzerland), 400 mg per day, was initiated immediately. Responder controll by PET-CT scan was planned to be performed 4 weeks after initiation of the therapy. After 2 weeks under ambulatory pharmacological therapy the patient presented in the emergency room with an acute upper gastrointestinal bleeding. CT confirmed a dramatic bleeding from the upper GI tract necessitating mass blood transfusion (Fig. ). Tumor size decreased to 7 × 8 × 12 cm within only 2 weeks of imatinib treatment. An angiographic CT showed the diffuse tumor bleeding supplied by the gastroduodenal artery and some branches of the superior mesenterial artery. The diffuse bleeding forbade a coiling of the vessels. During the emergency laparotomy an encapsulated tumor mass could be identified, originating from the descendent part of the duodenum and reaching both the pancreatic caput and the right flexure of the colon. Obviously the giant tumor had led to a bleeding by arrosion of peripancreatic vessels. After ligation of the vessels supplying the mass a partial pancreaticoduodenectomy (Traverso-Longmire) was performed to resect the tumor (Fig. ). Additionally a resection of the right hemicolon was performed due to tumor infiltration of the right curvature of the colon. Continuity was reconstructed by gastrojejunostomy (Traverso-Longmire) on the one hand and an end-to-side-pancreaticojejunostomy on the other hand. An ileotransversostomy was performed to reconstruct the gastrointestinal passage.\nUpon macroscopic examination the specimen showed a partially necrotic mesenchymal mass with a diameter of 9 cm, an infiltration of the duodenal wall leading to ulceration and perforation, an infiltration of the pancreas and two peripankreatic tumor islands (Fig. ). There were no signs of metastases in locoregional lymphnodes. Histological examination of the tumour tissue revealed the typical appearance of a GIST composed of cells with spindle-shaped nuclei (Fig.). Immunohistochemically the tumour cells showed an expression of Vimentin (Fig. ) and CD117 (Fig. ), a focal expression of CD34, smooth-muscle-actin (not shown) and a nuclear expression of the proliferation-associated Ki-67-antigen in approximately 5-10% of the tumour cells (Fig. ). The tumour was negative for S-100 and Keratin (not shown).\nTwo days after surgery the patient was weaned and successfully extubated. After an uneventfull recovery the patient is alive and without any signs of tumor recurrence. Up to the follow-up of 19 months the patient permanently received an adjuvant imtinib therapy (400 mg per day).
A 34-year-old female of Australasian ancestry, born in the UK, presented with subacute visual loss in the right eye preceded by retroocular pain. Vision deteriorated to no perception of light; vision in the unaffected (left) eye was 6/5 with normal perimetry. Magnetic resonance (MR) imaging performed 2 weeks after the onset of her symptoms showed hyperintensity on STIR images and Gadolinium enhancement of the intraorbital portion of the right optic nerve (Figures and ). The brain appeared normal. A lumbar puncture was performed: the cerebrospinal fluid (CSF) analysis was normal apart from minimal elevation in the number of lymphocytes (5/mm3). On CSF protein electrophoresis, one oligoclonal band was found in both CSF and serum and one or two further bands were found in the CSF only. Serological tests for NMO were negative. The diagnosis was made of non-MS optic neuritis.\nThe patient was treated with 1 g of Methyl Prednisolone intravenously for 3 days and with 60 mg of oral prednisolone for 3 weeks thereafter, at which point there had been good improvement in the right peripheral field. Her right visual acuity remained at 6/60 with a dense central scotoma, and the dose of prednisolone was steadily reduced as it was considered unlikely to improve further. Eventually, the daily dose was 7.5 mg and the signs were stable with no new symptoms. Repeat MR imaging was performed after one month at this dose and showed no persistent or recurrent enhancement. It was therefore considered reasonable to gradually reduce the corticosteroid dose further, that is, by 1 mg every two weeks. This proceeded uneventfully, and two months later the steroids were discontinued by which time the daily dose had been reduced to 1 mg.\nOne day after stopping the steroid treatment, the patient noticed pain on moving the left eye but no loss of vision. The patient had been instructed to attend immediately in this eventuality and hence presented to Eye Casualty within 48 hours where she was noted to have no change in her visual acuity, normal colour vision, and normal Goldmann perimetry. MR imaging showed STIR hyperintensity and enhancement of the left optic nerve (Figures and ). The patient was again treated with the same corticosteroid regimen. The pain resolved within a few hours, and there was no loss of vision, not subjectively, not on acuity and not on visual field examination. Her vision remains unchanged after 6 months follow-up and long-term immunosuppression with Azathioprine has been initiated.
A 67-year-old woman was referred to our hospital due to sudden onset of epigastralgia after dinner. The patient had undergone hysterectomy, bilateral oophorectomy, omentectomy, and pelvic and para-aortic lymph node dissection for her ovarian cancer 6 years before. Computed tomography (CT) on admission showed no dilatation of the small intestine. The patient was afebrile and had flat and soft abdomen with tenderness from the upper abdomen to the right lower abdomen. Blood gas analysis did not suggest intestinal necrosis, that is, neither the elevation of serum lactate level nor the metabolic acidosis. The patient, therefore, initially received gastrointestinal decompression and fluid therapy under the presumed diagnosis of adhesive ileus, unfortunately leading to the failure of symptom relief and the aggravation of ileus on follow-up CT (Fig. ). We, therefore, performed laparoscopic surgery to the patient on the 3rd day after admission. On laparoscopic inspection, we found minimum intra-abdominal adhesions, yellowish serous ascites without cancer cell dissemination, marked dilatation of the ileum, part of the terminal ileum passing through under the right external iliac vein without ischemic change, and no surgically available peritoneum left around the right external iliac vein (Fig. ). In addition, we had preoperatively found presumed thrombi in the right external iliac and femoral veins on nonenhanced CT (Fig. ) but no clinically significant swelling in the right leg. We, therefore, cut the right external iliac vein without vein reconstruction using an endoscopic stapler for the release of the strangulated ileum to prevent both the pulmonary embolism and the recurrence of this type of ileus (Fig. ). The patient recovered uneventfully with marked relief of the uncomfortable symptoms due to ileus. Ultrasonography on the 7th postoperative day showed thrombi extending from the distal external iliac vein to the femoral vein without further extension beyond the femoral vein. The patient began to wear an elastic stocking on the right leg during the admission period and was discharged on the 13th postoperative day. The patient has been doing well with no walking disturbance, no daily life impairment, and nominal lower limb swelling (Fig. , ).
A 65-year old male patient with early gastric cancer was transferred from Aruba to our institution. He had a 3-year history of black stools and anemia. His past medical history included multiple comorbidities: diabetes, chronic renal failure, alcoholic cirrhosis Child A, complete heart blockade and thrombocytopenia of unknown etiology. An upper endoscopy and biopsy revealed a well-differentiated intestinal type adenocarcinoma in the antrum. Endoscopic ultrasonography showed a hypoechoic, 3.2 cm neoplasm, without muscularis externa infiltration and reactive ganglia (). Endoscopic mucosal resection was chosen due to tumor size, stage and comorbidities of the patient. The tumor was fully resected without complications. At the end of the procedure the anesthesiologist had difficulty with ventilation and abdominal distention was observed (). He had a 128/91 mmHg blood pressure and 70 bpm heart rate. An endoscopic revision was done before finishing the procedure, without identification of any macroscopic perforation. A nasogastric tube was placed and therapeutic strategies to improve abdominal-wall compliance were instituted (changes in ventilation parameters, nasogastric suction, change to a supine position and removal of any strap over the abdomen). A plain abdominal radiography in the operating room showed a massive pneumoperitoneum (). Decision of a nonsurgical management was conducted and the patient was taken to the intensive care unit (ICU) for monitoring. The IAP measured by a trans-bladder catheter was 33 mmHg. Six hours after ending the procedure the patient developed dyspnea and anuria. The diagnosis of an abdominal compartment syndrome was established. Given the worsening status, interventional radiology evaluated the patient. A CT scan confirmed the massive pneumoperitoneum without intraperitoneal extravasation of contrast (a & b). A percutaneous decompression guided by CT scan was performed with a pigtail catheter G14 (c & d). Air was immediately released under pressure. Immediately after the procedure, the patient's symptoms and hemodynamic status improved. Diuresis returned after a few hours. The pigtail catheter was closed the first day after placement and taken out at the third postoperative day. Control CT scan revealed no evidence of pneumoperitoneum. Pathology report confirmed the resected specimen had free malignant cell margins and areas of high-grade and low-grade dysplasia. The patient was discharged from ICU at postoperative day 2 and discharged from hospital at postoperative day 5 without further complications.
A 46-year-old female presented in 2017 with symptomatic right leg pain and aesthetic complaints relating to the right calf. Medical history showed a previous head trauma (car accident) with brain hematoma drainage and a saphenous vein operation. Physical examination revealed edema in the perimalleolar area and painful varicose veins, in the right calf (with considerable aesthetic impact) and left calf (with minor aesthetic impact). Venous scores at the first visit to our clinic were the following: Venous clinical severity score VCSS 10 and Aberdeen quality of life questionnaire 27.7.\nDuplex examination conducted before the original venous operation (which had been performed in a different clinic in January 2016) had shown reflux in the left great saphenous vein and significant reflux in the right small saphenous vein. However, the operation actually performed was stripping of the right great saphenous vein. Both the left great saphenous vein and the right small saphenous veins were left in place untreated. After this procedure, symptoms had exacerbated progressively, and the aesthetics of the leg had deteriorated progressively.\nPreoperative evaluation was normal. We performed a complete duplex scan, according to our routine, as published elsewhere. The patient had type 1b+2a shunt in the right leg and 4+2d shunt in the left leg. We suggested operating to treat the small saphenous vein in the right leg and the great saphenous vein in the left leg. We treated the patient using the CHIVA technique to preserve the remaining saphenous veins.\nWe performed the CHIVA procedure on both legs during the same operation. Local anesthesia was provided with a solution containing 10 mg/mL 20 mL of ropivacaine and 2% lidocaine, using 20 mL and 60 mL of saline. We routinely have an anesthetist in the operating room to guarantee patient safety and comfort, who is always advised to avoid sedation as much as possible. When necessary, an opioid-free sedation technique is employed. In the right leg, we ligated the small saphenous vein at its junction with a calf vein and ligated two N3 collaterals, leaving the small saphenous vein draining through two perforators. In the left leg, we ligated a collateral draining to the great saphenous vein from the inguinal ligament and an N3 draining reflux from the great saphenous vein to the calf. A total of 5 small incisions were made. The patient was discharged two hours after the operation wearing compressive stockings and taking 40 mg enoxaparin per day for 3 days, according to our postoperative routine.\nOn the sixth postoperative day, duplex scanning was performed, showing minor continuous reflux in the small saphenous vein of the right leg and even less reflux in the great saphenous vein on the left. The right small saphenous vein had been 7.4 mm before the operation and was 3.8 mm after. The left great saphenous vein had been 4 mm before the operation and had not decreased in size during the initial postoperative period. The patient scored pain at 3 on a 0-10 pain scale and had taken one 750 mg paracetamol tablet during the entire postoperative period. We made a full photographic record before and after the operation ( \n ). There were no photographs or records of symptoms available from the original operation.\nIn relation to the wrong-site surgery, we comforted the patient and reported the case to both the previous surgeon and the patient safety surveillance team at the hospital where the operation had been performed.
A 20 year-old female presented to the dental clinics with the complaint of unaesthetic smile, generalized sensitivity, difficulty in chewing due to multiple cavities for several months and severe intermittent pain in left upper tooth for 5 days. The pain was spontaneous; aggravated on taking hot and cold fluids and during chewing. Pain was relieved temporarily after taking analgesics. Due to broken down posterior teeth and generalized sensitivity, she experienced extreme discomfort during mastication of hard or fibrous food, therefore, she relied more on soft, refined food for several years and used to skips regular meals. Most of her meals consisted of sweet drinks and refined carbohydrates. Her mother informed that she had suffered same problems with the deciduous dentition. Family history revealed that her grandfather had the same dental problem and most of his teeth were removed in early age. The oral hygiene history was also not satisfactory. The patient was otherwise healthy with no known allergies or co-morbids. She visited multiple dentists previously but was not satisfied with the given treatment plans. Most of them advised her extraction of most of her posterior teeth but she was willing to save her natural teeth. She was well motivated towards dental treatment and had no financial constraints. Therefore, wanted a durable solution with perfect aesthetics at the same time requested retaining her natural teeth.\nExtra oral examination revealed normal mouth opening with no tenderness, clicking or crepitus over the Temporo-mandibular joint. The lower facial height was reduced. Intraoral examination revealed poor oral hygiene with grossly carious posterior teeth. The occlusal plane was uneven with deep anterior bite and worn-down posterior teeth. The labial surfaces of all anterior teeth were stained due to irregular grooving. The overall enamel thickness was also reduced. The smile line was average but non-consonant. Marginal gingiva was red, inflamed and edematous. Deep periodontal pockets of 4mm were found around left mandibular first and second molar, and right mandibular first molar, however 2.5- 3mm pocket depth was found in all other posterior teeth. Tooth wear was uncompensated with freeway space of approximately 4-5mm.The panoramic radiograph revealed substantial loss of enamel from the occlusal surfaces of all teeth with multiple decayed lesions. Preoperative clinical pictures and panoramic radiograph are shown in Figure . Diagnostic casts were obtained, on which occlusion was evaluated. On the basis of clinical and radiographical findings, the most probable diagnosis was of hypoplastic type Amelogenesis imperfecta. The problem list consisted of:\n1. Poor oral hygiene with generalized gingivitis and localized periodontitis.\n2. Multiple carious lesions involving all posterior teeth.\n3. Generalized attrition with loss of occlusal vertical dimensional dimension.\n4. Compromised esthetics.\nThe aim of treatment was to provide a functional occlusion with realistic aesthetics and prevention of further tooth loss. The patient was asked to maintain a regular food diary to found out the frequency of cariogenic diet along with record of oral hygiene aids. This helped us in changing her diet to healthier and more fibrous diet. Mock build-up of full coverage crowns were initially done on upper and lower casts and showed to the patient. She was satisfied with the predicTable outcome. So the following treatment plan was made:\nImmediate management: Endodontic treatment of tooth #24 for relieving patient pain\nDefinitive treatment plan:\n1. Oral hygiene instructions, patient motivation and dietary modifications.\n2. Endodontic treatments of all natural teeth excluding of tooth #24 and third molars (#18, 28, 38, 48).\n3. Extraction of all third molars (#18, 28, 38, 48).\n4. Crown lengthening surgery on tooth #36, 37 and #46 for gaining ferrule.\n5. Restoration of the lost occlusal vertical dimension by provision of temporary bridges on all posterior teeth and freehand composite buildup on all palatal surfaces of upper teeth and lower canines. 6. Bisque trial and provision of all ceramic crowns.\n7. Provision of a Michigan type stabilization splint.\n8. Reinforcement of oral hygiene measures and periodic follow-ups.\nInitially, endodontic treatment was performed on #24 to relief patient symptoms, then it was done on all remaining natural teeth and post endodontic build-up was done with the composite ( 3M™ ESPE™ Filtek™ P60 Posterior Restorative System). All wisdom teeth were extracted and gingivectomy was performed on #36, 37 and #46 to gain ferrule for subsequent crown placement. After healing, diagnostic impressions were made using irreversible hydrocolloid (Tropicalgin, Zhermack Italy). Diagnostic casts were mounted on semi adjusTable articulator (Hanau Articulator, Teledyne Hanau Buffalo, NY, USA) using Hanau facebow. Diagnostic wax up was done according to optimum aesthetics and function followed by fabrication of a vacuum form stent (3A MEDES, EASY – VAC GASKET, KOREA). All posterior teeth were prepared and temporary acrylic bridges (Integrity™ Temporary Crown & Bridge Material, DENTSPLY, USA) were placed at an increased occlusal height. Composite buildups (3M™ ESPE™ Filtek™ P60 Posterior Restorative System, USA) on all palatal surfaces of upper teeth and lower canines as shown in Figure . Temporary bridges were left for three months to evaluate patient’s tolerance to increase occlusal vertical dimension (OVD). Periodic follow-ups were done after a week, one month and three months to assess the patients tolerance and any associated complication of temporary bridges. Patient was comforTable, no muscle tension was noted and TMJ examination revealed no tenderness or crepitus. Therefore, definitive impressions of maxillary and mandibular arch were taken with poly vinyl polysiloxane impression material (Aquasil Ultra Putty Soft Regular and Aquasil Ultra LV, Dentsply USA). Interocclusal records were taken in centric relation using bimanual manipulation method. Working casts were then articulated using interocclusal records on semi-adjusTable articulator (Hanau Articulator, Teledyne Hanau Buffalo, NY, USA). A mutually protected occlusal scheme was preserved to avoid stresses on posterior teeth during lateral excursions. All ceramic crowns (IPS e-max Ceram, Ivoclar vivadent, Leichtenstein. Germany) were fabricated and bisque bake trial was done for the evaluation of crown contours, contacts, occlusion and aesthetics. Crowns were glazed and bonded to the teeth with self-adhesive resin (RelyX Unicem 2 Clicker A2 Universal Self-Adhesive Universal Resin, 3M-ESPE, USA). A Stabilization splint was made after the delivery of final prosthesis and delivered to the patient. Post-operative extra-oral and intraoral pictures shown in Figure displays a significant improvement in esthetics and even occlusal plane with correction and increase in vertical dimension of occlusion. Postoperative panoramic view is shown in Figure displays nicely done endodontics and fixed prosthodontics. Pre and post-operative comparative clinical pictures are shown in Figure showing significant improvement in dento-facial aesthetics. Patient was asked to follow up in six months.
A 16-year-old male presents to the emergency department (ED) with complaints of pain, swelling, and difficulty bearing weight on the right lower extremity. A week prior to this presentation, the patient’s bike collided with a car resulting in an impact injury to the lateral aspect of his right knee. X-ray of the right ankle done at the local urgent care center was normal and he was discharged home with a diagnosis of a right ankle sprain. The pain and swelling persisted and progressively got worse over the next week, prompting the visit to our ED.\nIn our ED, the patient appeared to be in moderate discomfort, unable to bear weight and has an ankle brace wrapped around his right ankle. Vital signs on presentation: temperature = 99.3 F, heart rate = 113 beats per minutes (tachycardia), blood pressure = 117/73 mmHg , respiratory rate = 18 breaths per minute, pulse oximetry was 98% on room air. On physical examination, there was moderate swelling of the right foot and ankle area with significant edema extending to the lower third of the leg, along with severe tenderness that is exaggerated upon moving the toes. Evaluation of pulses in both lower extremities revealed a noticeable reduction in the right popliteal, posterior tibialis, and dorsalis pedis arteries (Figure ). The right foot felt colder and paler compared to the left foot without any erythema or bluish discoloration of the toes.\nThe surgery team was consulted due to the suspicion for possible compartment syndrome and vascular compromise and the patient was admitted to the in-patient unit for further workup and management. Doppler sonogram of the right lower extremity showed no evidence of deep vein thrombosis or vascular compromise.\nThe following day the patient started complaining of complete loss of sensation in the extremity from around the knee to the toes. CT angiography of the right lower extremity with contrast revealed contrast in the adjoining vein adjacent to the artery along the area of trauma on the right side compared to the healthy left lower extremity (Figure ). The next diagram shows multiple contrasts enhance receiving veins in the right lower extremity as compared to the left lower extremity (Figure ).\nThe interventional radiologist on a careful review of the CT angiogram raised the suspicion of a possible traumatic AV fistula and recommended selective IR angiography of the right lower extremity. This study is diagnostic and at the same time allows for therapeutic intervention if needed.\nThis study clearly defined the abnormal communication between the muscular branch of the right anterior tibial artery and the vein (Video ). No immediate vascular intervention was required at this time as this was a low flow of fistulous communication. The patient reported improvement of his symptoms following the procedure, manifesting less pain and return of skin sensation. He received physical therapy during his hospital stay, which also attributed to his recovery of weight-bearing and range of motion in the affected right lower extremity. He was subsequently discharged home with instructions to continue physical therapy and follow up with interventional radiology.
A 26-year-old Caucasian man was referred to the surgical service with an enlarging splenic mass and left upper quadrant pain. The pain was described as dull with no exacerbating factors. He had no nausea or vomiting and no other systemic complaints. He had a history of laryngeal ACC that was treated with total laryngectomy and adjuvant radiation three years prior to this clinical visit. Within a year of the original diagnosis and treatment of ACC, our patient developed a single right lung metastasis for which he underwent thoracotomy with resection. This was followed by recurrence in the ipsilateral lung, which was again resected. He was started on Tarceva (erlotinib), an EGFR inhibitor used to treat non-small cell lung cancer, but the therapy was discontinued secondary to an intolerable rash.\nDuring follow up a computed tomography (CT) scan of the chest demonstrated bilateral lung nodules, which were consistent with metastatic disease (Figure ). However, there were no symptoms attributable to these lesions. The CT scan also demonstrated a lesion in the spleen that measured 4cm and was approximately 1mm from the splenic capsule (Figure ). He was referred to the surgical service when a follow up CT scan of the abdomen three months later demonstrated progression in size of the lesion by 1cm and our patient complained of left upper quadrant abdominal discomfort. There was no evidence of other intra-abdominal disease. Due to the progression in size of the splenic lesion, its proximity to the capsule with potential complications of rupture or local advancement, and in light of our patient's symptoms the decision was made to proceed with laparoscopic splenectomy. He was given appropriate pre-operative immunization. At the time of surgery, there was no evidence of other intra-abdominal disease. The spleen (Figure ) was removed intact through an enlarged port site incision. Pathologic examination (Figure ) showed a 5.2 × 4.3 × 4cm single metastatic nodule within 0.1cm from the capsule. The mass shows mucoid areas in the center and microscopically had features characteristic of adenoid cystic carcinoma with a predominant cribriform growth pattern. He had a routine convalescence, and is currently alive and well.
The patient was a 55-year-old male entrepreneur, who had been experiencing pain of moderate intensity in the mid third of his left thigh for approximately 6 days. He sought emergency care at a hospital in response to a sudden increase in the intensity of the pain combined with swelling at the site of pain. He had a history of smoking equating to approximately 37 pack years. He did not have any other comorbidities. On physical examination he was slightly pale, with tachycardia (120 bpm) and blood pressure at 100 x 70 mmHg. Physical examination by segments was unremarkable for the head and neck, thorax, and abdomen. Vascular examination of the right lower limb found normal auscultation and visual inspection results, with all pulses present and normal. The left lower limb was well-perfused, but there was ecchymosis and a pulsatile swelling between the mid and distal thirds of the thigh, in the anteromedial region ( a). Inspection of the left foot also revealed signs of distal microembolization ( b) and popliteal and distal pulses were absent.\nUltrasonography of the left thigh showed an aneurysmal dilatation of the SFA measuring 5.8 x 5.3 cm and with associated mural thrombi and perivascular accumulations compatible with a ruptured aneurysm ( ). Since angiotomography was not available at the service providing care, the decision was taken to perform emergency surgical treatment.\nDuring the procedure, extensive hematoma was observed involving subcutaneous and muscle tissues in the anteromedial region of the thigh. Once this had been removed, the ruptured SFA aneurysm could be seen ( 3b). There were no obvious signs of active infection. Proximal and distal ligatures were performed and then the aneurysm was resected and samples collected for anatomopathological and microbiological analyses. Revascularization of the limb was then accomplished by interposition of the contralateral great saphenous vein in reverse, with end-to-side anastomosis – taking into consideration the diameter of the femoral artery and the significant destruction of its walls, as illustrated in c. The contralateral saphenous vein was used both because of the probability of associated damage to deep veins in the limb involved in rupture and because of the greater likelihood of injury during dissection, due to anatomic distortions. There were no intercurrent conditions during the procedure\nThe patient was prescribed prolonged, wide-spectrum antibiotic therapy until the results of the microbiological culture of the aneurysmal fragment were available, showing no evidence of growth of microorganisms. The anatomopathological analysis found true aneurysmal walls, with no specific abnormalities. Supplementary imaging exams did not identify any additional aneurysms or any evidence of valve vegetations suggestive of endocarditis.\nAt 1-month follow-up, the patient had palpable distal pulses and was free from pain or other problems.
A 73-year-old man was admitted with severe chest pain at rest for 3 hours. He had previous three myocardial infarctions with the last one occurring 14 years before this admission. At that time, he underwent coronary artery bypass grafting (CABG) with LIMA to left anterior descending artery and two saphenous vein grafts to the right coronary and first obtuse marginal arteries. Following surgery, his chest pain resolved, but he had frequent admissions with chest pain over the last four years which was misdiagnosed as coronary artery spasm. In addition, his past medical history included congestive heart failure and cardiac arrest twelve years ago. His coronary risk factors were hypertension, hypercholesterolemia, family history of ischaemic heart disease, and a 45 pack-year history of cigarette smoking.\nHis physical examination revealed a blood pressure of 124/63 mmHg in the right arm and 83/50 mmHg in the left, a regular pulse of 84 beats/minute, and respiratory rate of 18 breaths/minute. He had normal heart sounds and the lung fields were clear. The vascular examination revealed easily palpable right upper extremity and bilateral lower extremity pulses. Left upper extremity pulses were present but diminished. Carotid arteries were palpable with no audible bruits. No abdominal masses were palpated.\nA 12-lead electrocardiogram demonstrated ischaemic changes in lateral leads and a chest x-ray showed cardiomegaly. Cardiac enzymes revealed troponin of 0.05 μg/L. On diagnostic coronary angiography, there was 50% stenosis in left main stem and 50% stenosis in left anterior descending artery in mid-segment with good run off. Failure to pass the catheter through the left subclavian artery indicated possible stenosis raising suspicion of CSSS.\nCT Angiogram confirmed a 2 cm stenosis at the origin of left subclavian artery (). Stenting of left subclavian artery was considered hazardous due to the risk of occlusion of the adjacent vertebral artery. The patient was referred to undergo left common carotid to subclavian artery bypass. After exploration, the left subclavian artery was found to have significant atherosclerosis. Therefore, an infraclavicular incision was utilised for access to the left axillary artery. A 6 mm ringed Vascutek PTFE graft (Sulzer Vascutek Ltd.; Renfrewshire, Scotland, UK) was used to construct a bypass between the left common carotid artery and the left axillary artery tunnelled beneath the clavicle. Carotid shunt was not used while systemic heparin (5000 units) was administered during the operation. Postoperatively, a good graft pulse was felt in addition to easily palpable left upper extremity pulses. The patient made an uneventful recovery, the angina pain disappeared and he was discharged 4 days following the operation. Two months later, he reported improvement in symptoms and his blood pressure was equal in both arms. Control CT angiogram nine months later revealed an intact PTFE graft between carotid and axillary artery.
A 42-year old woman underwent LSG for class 1 obesity (BMI 31 kg/m2) in a private hospital in October 2018. Her past medical history was unremarkable, except for psoriasis and a previous laparoscopy for extrauterine pregnancy. The operation lasted 37 min and there were no obvious intraoperative complications. Immediately after the operation the patient started complaining about pain in the throat, mild difficulty swallowing and pain when moving her neck. During the night she complained about chest discomfort. Her vital signs were checked regularly and were normal. The next morning the pain was increasing together with swelling of the throat and marked sialorrhea. These symptoms led to further investigation with laryngoscopy, which was without remarks. A few hours later the patient developed subcutaneous emphysema and subsequently underwent an emergency CT scan, which showed free air outside the esophageal lumen, up to the neck and along the entire intrathoracic esophagus (Fig. ). The patient was still hemodynamically stable and in good general condition and was transferred to our hospital, which is a tertiary referral center for esophageal surgery, for further management.\nThe patient was taken directly to the operating room and a gastroscopy was performed under general anesthesia, revealing a 3 cm long perforation located 14–17 cm from the incisors on the posterior wall of cervical esophagus (Fig. ). Due to the proximity to the cricopharyngeal muscle, sealing of the perforation by placement of a fully covered esophageal stent was not possible. Additionally, use of Eso-SPONGE® Endoluminal Vacuum Therapy System (B. Braun, Germany) was considered not feasible because of the large size of the defect. Based on the gastroscopy findings, the perforation was estimated to be at exactly the level of the thoracic inlet. Nevertheless, the false lumen was extending more distally, and the prevertebral space was at the time of surgery filled with purulent fluid (Fig. ). Our assessment was that we needed access to the upper mediastinum to achieve satisfactory drainage and, hopefully, also repair the defect at the same time if possible. The patient was placed in the prone position and a right-sided thoracoscopy was performed. The intrathoracic esophagus was mobilized by incising the mediastinal pleura and a large mediastinal fluid collection was evacuated. The lower border of the perforation high up on the posterior wall of the esophagus at the level of thoracic inlet could be visualized but suturing was technically not possible thoracoscopically. After placing two drains in the thoracic cavity, the patient was placed on the supine position and the cervical esophagus was approached through a left-sided neck incision. The proximal esophagus was mobilized and rotated giving access to the posterior side, the defect was visualized and repaired with interrupted 4/0 PDS sutures (Fig. ). Further reinforcement was applied by a muscle flap constructed from the sternal head of the sternocleidomastoid muscle. Due to neck edema, delayed extubation was performed as a precaution, and the patient spent the first post-operative day in the Intensive Care Unit but could be discharged and transferred to the ward the following day. Further treatment with nil by mouth, broad-spectrum antibiotics and parenteral nutrition was carried out. The recovery was uneventful; the patient could start an oral diet on post-operative day 6 and was discharged from the hospital 2 days later, on postoperative day 8.
CJ was a 49-year-old man. He belonged to a family of Jehova’s witnesses. He had undiagnosed learning difficulties (LD) due to which he was never able to lead an independent life. He lived with his parents. No other agencies were involved in his care and he was not seen by the General Practitioner (GP) for years. He was brought in to be seen by the GP for a routine appointment by his father. He was frail and looked unwell but did not seem to be in any discomfort. Examination revealed a rectal mass and palpable inguinal lymph nodes. An urgent referral to the colorectal surgeons was made and blood tests were performed. He was found to be profoundly anaemic. Due to the parents’ belief, no blood products were accepted for CJ. The hospital also offered an inpatient care which was refused by his parents.\nAfter being seen by the consultant in the hospital, he had an MRI scan which revealed a locally advanced rectal tumour. A de-functional colostomy was performed for palliative purposes by the surgical team due to mechanical bowel obstruction secondary to tumour size and location. The specialist multidisciplinary team (MDT) decided to go ahead with aggressive chemotherapy to stop further tumour spread.\nOnce the patient was discharged back home, the district nurses were involved in providing stoma care to the patient. During this time, he had multiple chemotherapy sessions and two incidences of neutropaenic sepsis. A follow-up MRI scan few months later found metastasis to liver. Patient was commenced on palliative care pathway with the aim to reduce discomfort.\nThe patient’s health was deteriorating slowly. He was started on nutritional support and was frequently assessed by the community palliative care team. They were making regular contact to assess pressure areas, provide any stoma care or dressings if needed and helped parents out with his basic needs and medication. There was a little hospice or GP involvement during that time.\nHis end-of-life care was carried out in his parents’ house as they did not allow him to go elsewhere. Due to the accessibility issues and the size, the house and CJ’s room was unsuitable to deliver care in the best possible manner. The bedroom where CJ spent his time could only accommodate a bed where he would lie all day. There was no space for a chair or a recliner for him to sit to prevent bedsores. The parents did not allow any hoisting equipment or changes to their existing bathroom and showering facilities to accommodate CJ’s needs. It resulted in poor hygiene and lack of dignity for CJ towards his end of life. The discussions by the community healthcare team about these issues with parents were brief and when parents denied all help, colleagues like GPs and palliative care consultants in hospice were not involved to further explore the reasons of parents’ reluctance to accept help which might have enabled in improved outcomes for CJ. There was no decision around patient’s resuscitation status as parents refused this discussion on many occasions as well.\nHe had multiple visits to the emergency department (ED) during this time which could have been avoided. CJ used to be taken to hospital in an ambulance to the ED where he used to wait for a clinician on the trollies for hours. He used to get unnecessary investigations and intravenous cannulation.\nCJ died in his own home after 16 months of his diagnosis.
The patient was a 28-year-old woman who referred with the chief complaint of a swelling and stiffness in the left cheek. From a clinical perspective, there was a slight swelling in the masseter muscle. On palpation, a moving mass with a stiff area was felt; and in intraoral examination, its position was felt in the anterior ramus. The patient expressed no specific history of systemic disease. There was also no history of trauma incidence in the area. The only point in the patient's history was a course of laser therapy for skin rejuvenation in the left cheek and several other areas in her face.\nIn the medical history of the lesion, there were 3 times of triamcinolone injection in the area during the last 3 years to treat the lesion by another physician. The patient said that reduction in tumor size was seen for a while after these injections.\nAspiration was performed for the patient through intraoral approach, whose result was negative. Magnetic resonance imaging (MRI) radiography (with and without contrast media) was also prepared.\nMagnetic resonance imaging revealed a solid heterogeneous mass in the pterygopalatine fossa area with penetration and extension towards both buccal and masticator spaces on the left. The submandibular area and carotid space were normal and no abnormality was seen in the nasopharynx area. Paranasal sinuses were also completely normal [Figure and ].\nAccording to the results of clinical and radiographic examinations of the treatment plan, the excisional biopsy of the studied mass was selected under complete anesthesia.\nUnder general anesthesia, the patient underwent a surgery with intraoral access through a cut in the upper area of anterior ramus. After dissection in the upper-side direction, vascular lesion was seen in buccal extension of the BFP []. The BFP capsule was intact and the mass had offended no soft/hard adjacent tissue. A single firm nodule (probably calcified) was also seen in the lesion.\nGiven two points, first, “the possibility of a vascular lesion” and second, “for the purpose of liposuction for cosmetic goal and remove of the swelling on the patient's cheek,” the vascular mass was removed along with anterior lobe, as excisional biopsy through intraoral approach and hence that we did not enter into the vascular lesion []. The specimen was sent to a pathologist for histopathologic examination. The clinical swelling of the cheek was removed immediately after the surgery. In the macroscopic viewpoint, the lesion was a yellow and dark purple mass measuring 2 cm × 3 cm × 4 cm along with a hard nodule-like area. Microscopic results represented a vascular lesion composed of large amounts of small to large vascular structures covered with endothelial cells. Hemorrhage areas were also observed. Fatty tissues composed of lipocytes were seen in other areas. There was no sign of Malignancy [].
A 61-year-old male showed high levels of lactase dehydrogenase and alkaline phosphatase at a health checkup. He went to the clinic for an assessment of his general condition, and multiple lymphocytic metastases on an abdominal ultrasound and an elevation of the carcinoembryonic antigen level were detected. At the time of the medical visit, the patient only had back pain and did not report any inconveniences in his daily life activities. He had a history of hyperlipidemia and hypertension. When he came to our hospital, his peripheral blood examination showed multiple abnormal values (table ). In addition, we diagnosed the patient with DIC (based on the DIC score calculated according to the DIC diagnostic criteria issued by the Japanese Association of Acute Medicine). A total colonoscopy (TCS) showed a stenosis of the transverse colon (fig. ), and a signet ring cell carcinoma was detected on a biopsy. We found that the cancer was present throughout the transverse colon on a barium enema (fig. ). Furthermore, a CT detected multiple lymph node metastases. Fluorodeoxyglucose (FDG)-positron-emission tomography (PET) also detected multiple lymph node metastases in addition to multiple bone metastases (fig. ). We performed a bone marrow puncture in order to make a diagnosis of the tumor and ultimately diagnosed the patient with DCBM originating from colon cancer (fig. ). The same day, he was admitted to our department for treatment. We immediately initiated treatment with emergency chemotherapy consisting of XELOX + BV with an elemental diet. To treat the bone metastasis, denosumab was administered at an interval of 1 month for a total of 3 times before reevaluating the tumor. In addition, we administered anticoagulation therapy with rhTM to treat DIC (resulting from DCBM) for 1 week. We continued the chemotherapy for 4 cycles without any severe adverse events; we then reevaluated the tumor. The carcinoembryonic antigen level consistently decreased from 1,382 to 69.1 ng/ml. Although the primary lesion did not change in size on TCS or barium enema (fig. ), the multiple metastases in the bone and lymph nodes clearly disappeared on CT and PET-CT (fig. ). Furthermore, the patient recovered from DIC caused by DCBM following the administration of chemotherapy and rhTM. We considered performing a resection of the primary lesion in order to reduce the tumor volume and enable oral intake.
A seven month old baby boy with Trisomy 21 presented with growth failure (weight 5.1 kg) and respiratory symptoms. He had normal oxygen saturations, a long systolic murmur in the right sternal border, normal splitting of the second sound along with mild cardiomegaly on chest X-ray. His electrocardiogram (ECG) showed right axis deviation and prominent right ventricular forces usual for his age. Echocardiogram showed dilated right atrium and right ventricle. On parasternal short axis view, there was an abnormal trifurcation of the main pulmonary artery. [Figure and , Video and ] The pulmonary artery had the usual right and left pulmonary artery branches and a third abnormal tunnel that arose from the origin of the left pulmonary artery coursed over the roof of the left atrium in the transverse sinus of the heart towards the right and drained near the superior vena caval orifice of right atrium. The tortuous course of this channel and entry into the right atrium was well seen from the subxiphoid view in coronal plane [, ] and apical view [, ]. There was abnormal continuous flows into the right atrium through this tunnel. Contrast enhanced multislice computed tomography in axial and coronal slices [Figure and ] and volume rendered images [Figure and , ] confirmed the abnormal tunnel like communication from pulmonary artery to right atrium coursing in the transverse sinus of heart causing a circular shunt and dilatation of right atrium and right ventricle. Diagnostic pulmonary artery angiogram in cranial and caudal projections [Figure and , Video and ] delineated this 6 mm wide tunnel. The pulmonary artery pressure and right atrial pressures were mildly elevated. Two femoral venous access were taken, and the tunnel was crossed from the pulmonary artery with a guidewire, which was snared in the right atrium to get a veno venous railroad. A long 6F sheath was advanced through the right atrium into the tunnel. This tunnel was closed with a 8-6 Amplatzer duct occluder (St Jude Medical, Minnesota, MN) in the cardiac catheterization laboratory [Figure and , Video and ] and position was checked with pulmonary angiogram through the other venous access. Follow up echocardiogram showed cessation of flows in the tunnel and reduction in size of the right ventricle. While different cardiac lesions causing circular shunts have been reported in the past, this rare variation of circular shunt from a pulmonary artery to right atrium involving only the right sided cardiac chambers without causing systemic hypoxia is unreported so far.[]\nThis anatomy may be embryologically akin to the aortico-right atrial tunnel which often arises from the left side of the aortic root or from the undersurface of the aortic arch, courses in the transverse sinus of the heart and drains in to the right atrium and causes a left to right shunt.[] The anatomical location of the fistula in this patient was also similar in its course through the transverse sinus of the heart over the roof of the left atrium and drainage near the superior vena caval orifice of the right atrium. While the proximal origin of aortico-right atrial tunnel has been documented from the left side of the aortic root and also from the undersurface of the aortic arch, this patient had the proximal origin from the main pulmonary artery near the confluence. In the recent years, aortico-right atrial tunnels with significant left to right shunts have been managed with transcatheter interventions.[] We opted to close the tunnel in our patient too in the catheterization laboratory. If left alone, the obligatory shunt from the pulmonary artery to right atrium may result in progressive dilatation of the right sided cardiac chambers. Since this anomaly has not been reported in the past in literature, the natural history of an uncorrected pulmonary artery to right atrial tunnel is unknown.
A 37-year-old female who had undergone a total parathyroidectomy 4 months previously for a parathyroid adenoma presented in our clinic with high serum calcium (10.3 mg/dL) and high intact parathyroid hormone levels (iPTH; 1,378 pg/mL). A technetium-99m sestamibi scan was performed to determine whether there was any remnant parathyroid tissue or whether there was a satellite adenoma. A focal nodule with increased uptake of technetium was detected in the left lower paratracheal area and suspected to be an ectopic parathyroid adenoma (). The mass was located on the left side of the pulmonary trunk, and we decided to perform a surgical biopsy. Preoperatively, we performed a chest computed tomography (CT) scan to localize the mass more accurately without enhancement because of her impaired renal function. She had been diagnosed with chronic rejection following transplantation of both kidneys, and was on hemodialysis. The mass was located between the aortic arch and the pulmonary trunk, apparently adhering to the inferior side of the aortic arch in the CT scan ().\nBecause the nodule was located deep inside the lower paratracheal area and was thought to adhere to the aortic arch, and a recurrent laryngeal nerve was nearby, excision through a posterolateral thoracotomy was planned. After opening the left 5th intercostal space with one lung ventilation, the mediastinal pleura was opened in the aorto-pulmonary window. A firm and brownish mass about 2 cm in diameter was seen to be in contact with the lesser curvature of the aortic arch. During the operation, the recurrent laryngeal nerve was saved by snaring. The mass was carefully dissected free from the aorta, and it was sent for pathological examination. A frozen biopsy revealed a parathyromatosis. A 24 Fr chest tube was inserted and the wound was closed layer by layer. She was then transferred to a general ward with tolerable vital signs.\nThe immediately postoperative serum iPTH level was 32.9 pg/mL, and it had decreased to 5.1 pg/mL by the time of discharge. The postoperative course was uneventful and the patient was discharged on postoperative day 6 without any problems. The last follow-up took place 4 months after the excision, and the patient had been doing well, without any complications.
The patient was a 66-year-old male with ESRD due to chronic glomerulonephritis. Chronic haemodialysis was initiated in 1970 when the patient was 28 years of age and this modality was still rare in Japan. For the first 26 years, he received nocturnal haemodialysis for 12 h twice a week. After that he had received haemodiafiltration (HDF) for 4.5 h three times a week until transplantation. He had surgery for aseptic necrosis of the right femoral head at the age of 58 and suffered from cerebral infarction but recovered without any neurological defect. He was noted to have atrial fibrillation at the age of 63, and has since then been treated with an anticoagulant.\nAfter maintenance haemodialysis for 38 years and 2 months, he received a kidney transplant from a 54-year-old female who had been diagnosed as brain dead due to traumatic subarachnoid haemorrhage. At the time of emergent admission, screening was performed to evaluate the risk of transplantation. There was no critical problem regarding the cardiovascular system except for the anticoagulant treatment. Computed tomography (Figure a) showed minimal calcification of his iliac vessels in spite of the long-term haemodialysis.\nThe kidney was harvested after cardiac arrest and preserved in University of Wisconsin solution, with a total ischaemic time of 6 h and 48 min. The graft ureter was anastomosed to his bladder intravesically. A percutaneous transvesical ureteral catheter was inserted because of the small volume of his bladder (<50 ml).\nThe graft functioned slowly, and four sessions of haemodialysis were needed after transplantation (Figure ). Thereafter, the serum creatinine level gradually decreased to as low as 1.0 mg/dl. However, another operation was necessary on postoperative Day (POD) 13 due to bursting of the surgical wound caused by incisional hernia. Due to occlusion of the ureteral catheter for vesical rehabilitation starting on POD 20, he required frequent urination and was fatigued. On POD 35, the ureteral catheter was removed, and a transurethral Foley catheter was inserted. Cystography on POD 42 showed that the contrast medium infused via the Foley catheter went to the transplanted graft easily because of disuse atrophy of the bladder (Figure b). Although the Foley catheter was removed on POD 55, it was inserted again on POD 59 because of a rise in fever and mild elevation of the serum creatinine level, attributed to graft pyelonephritis caused by vesico-ureteral reflux. After reinsertion of the Foley catheter, he showed a very favourable course, and serum creatinine remained as low as 1.0 mg/dl. After the continuous rehabilitation of his native bladder by transient occlusion of the Foley catheter, the catheter was removed on POD 75. He was finally discharged from the hospital without difficulty in urination on POD 84. Now, he is free from haemodialysis with an improving vesical function.
A 76-year-old man with a past medical history of coronary artery disease, hypertension, and severe septal hypertrophy suspicious for hypertrophic cardiomyopathy, presented to the hospital with progressive symptoms of heart failure. He had been suffering from worsening dyspnea and lower extremity edema for several months. Lab work over the preceding months had shown deranged liver function tests, concerning for right heart failure. He was admitted for intravenous diuretic and inotrope therapy. At his cardiology clinic appointment prior to this hospital admission, an ECG showed typical atrial flutter and diffuse low voltage []. A right heart catheterization showed moderately elevated right greater than left filling pressures with a right atrial pressure 18 mmHg, pulmonary capillary wedge pressure 21 mmHg and a Fick cardiac index 1.6 liters/min/m2. The ScvO2 was 48%.\nOf note, a year prior to this admission, a transthoracic echocardiogram (TEE) had shown severe asymmetric septal hypertrophy (2.6 cm), suspicious for hypertrophic cardiomyopathy, but had not been further evaluated. A repeat TEE at the present admission showed a left ventricular ejection fraction (LVEF) of 35% as well as the aforementioned septal hypertrophy []. However, due to the significant low voltage on ECG, despite the significant LVH on echocardiogram and history of hypertension, a cardiac MRI was ordered to rule out infiltrative cardiomyopathy as opposed to hypertrophic cardiomyopathy.\nThe images showed normal left ventricular chamber size with severe, asymmetric left ventricular hypertrophy, primarily involving the septal wall with a maximum septal thickness of 27 mm []. The LVEF was measured at 40%. Also noted was diffuse, global late gadolinium enhancement of the left ventricular myocardium, consistent with a diagnosis of cardiac amyloidosis []. The only extracardiac clinical finding suggestive of systemic amyloidosis was carpal tunnel syndrome. Serum and urine electrophoresis did not detect a monoclonal protein, and serum free light chain ratio was low, which significantly lowered the suspicion for AL-amyloidosis. An abdominal fat pad biopsy was ordered which came back negative. Finally, an endomyocardial biopsy was performed which showed a pink amorphous interstitial infiltrate exhibiting apple green birefringence with Congo red stain, confirming cardiac amyloidosis. The subtype was likely wild-type ATTR or variant ATTR amyloidosis.\nIncidentally, the cardiac MRI also found a left upper lobe mass which, on further workup, was characterized as adenocarcinoma. No further differentiation of the subtype of ATTR amyloidosis was pursued, as this would not have changed management, especially in view of the poor prognosis portended by the adenocarcinoma.
A 48-year-old woman was referred to our emergency department with a chief complaint of severe abdominal pain. The patient's presentation was acute, accompanied by nausea, chills, and cold sweating. The patient appeared pale, and while her mental status was alert, systolic blood pressure was 60 mmHg, and diastolic blood pressure was 40 mmHg. Her heart rate was elevated at 119 beats per minute. On physical examination, the abdomen was distended with diffuse pain, and local tenderness was observed in the lower abdomen. The patient's medical history was unremarkable.\nSerum blood analysis revealed a hemoglobin level of 5 g/dL. The patient underwent transfusion of 3 units of packed red blood cells to compensate for the unstable hemodynamic status. Computed tomography (CT) revealed 10.1 cm × 9.5 cm and 7.6 cm × 7.0 cm heterogeneous masses in the uterus () and abundant fluid collection in the peritoneal cavity (). Ultrasonography showed the same finding, suggesting hemoperitoneum in the abdominal cavity. Unfortunately, because she was transferred from another hospital to our emergency room, we did not have any information about previous ultrasonography or specific clinical signs. The cause of bleeding was unclear, but owing to her unstable hemodynamic status, the decision was made to perform emergency laparotomy in order to explore and control the source of bleeding.\nThe patient was transferred to the operating room, and the peritoneal cavity was approached through a vertical incision on the lower abdomen under general anesthesia. On dissection of the subcutaneous fat and the underlying fascia, approximately 1,800 mL of blood and blood clots were evacuated from the pelvicabdominal cavity. After removal of the hematoma, the uterus was carefully examined to identify the potential source of the hematoma. During the inspection, multiple myomas were noted at the fundus of the uterus, which enlarged the uterus to the size of a watermelon. Using the International Federation of Gynecology and Obstetrics classification, the patient's fibroid could be categorized as type 7, which is a fibroid pedunculated on the subserosal surface. Active bleeding was noted at the fundus of the uterus, which originated from the vessel of a pedunculated myoma. Upon ligation of the bleeding vessel, excision of the pedunculated myoma was performed using an electrocautery device and open sealer (LigaSure; Medtronic, Minneapolis, MN, USA). The resected surface of the uterus was sutured using absorbable polysorb and monosyn sutures. Further exploration found no other bleeding sources.\nFrom the initial visit to the end of the surgery, the patient received 10 packs of platelets, 8 packs of fresh frozen plasma, and 8 packs of packed red cells. Following surgery and transfusion, her hemoglobin level increased from an initial 5 to 10.2 g/dL. Her vital signs returned to normal after surgery.\nPostoperative CT performed 3 days after surgery showed evacuation of the hematoma with the remaining uterus. The patient was discharged after 7 days in a healthy state. A follow-up visit was made 6 weeks after surgery, but no further complications were noted.
The patient was a 55-year-old male entrepreneur, who had been experiencing pain of moderate intensity in the mid third of his left thigh for approximately 6 days. He sought emergency care at a hospital in response to a sudden increase in the intensity of the pain combined with swelling at the site of pain. He had a history of smoking equating to approximately 37 pack years. He did not have any other comorbidities. On physical examination he was slightly pale, with tachycardia (120 bpm) and blood pressure at 100 x 70 mmHg. Physical examination by segments was unremarkable for the head and neck, thorax, and abdomen. Vascular examination of the right lower limb found normal auscultation and visual inspection results, with all pulses present and normal. The left lower limb was well-perfused, but there was ecchymosis and a pulsatile swelling between the mid and distal thirds of the thigh, in the anteromedial region ( a). Inspection of the left foot also revealed signs of distal microembolization ( b) and popliteal and distal pulses were absent.\nUltrasonography of the left thigh showed an aneurysmal dilatation of the SFA measuring 5.8 x 5.3 cm and with associated mural thrombi and perivascular accumulations compatible with a ruptured aneurysm ( ). Since angiotomography was not available at the service providing care, the decision was taken to perform emergency surgical treatment.\nDuring the procedure, extensive hematoma was observed involving subcutaneous and muscle tissues in the anteromedial region of the thigh. Once this had been removed, the ruptured SFA aneurysm could be seen ( 3b). There were no obvious signs of active infection. Proximal and distal ligatures were performed and then the aneurysm was resected and samples collected for anatomopathological and microbiological analyses. Revascularization of the limb was then accomplished by interposition of the contralateral great saphenous vein in reverse, with end-to-side anastomosis – taking into consideration the diameter of the femoral artery and the significant destruction of its walls, as illustrated in c. The contralateral saphenous vein was used both because of the probability of associated damage to deep veins in the limb involved in rupture and because of the greater likelihood of injury during dissection, due to anatomic distortions. There were no intercurrent conditions during the procedure\nThe patient was prescribed prolonged, wide-spectrum antibiotic therapy until the results of the microbiological culture of the aneurysmal fragment were available, showing no evidence of growth of microorganisms. The anatomopathological analysis found true aneurysmal walls, with no specific abnormalities. Supplementary imaging exams did not identify any additional aneurysms or any evidence of valve vegetations suggestive of endocarditis.\nAt 1-month follow-up, the patient had palpable distal pulses and was free from pain or other problems.
The patient was a male aged 6 years and 9 months, the only son of a 38-year-old mother and a 41-year-old father who measured 1.58 m and 1.76 m in height, respectively, and were healthy and nonconsanguineous. The parents reported no genetic conditions or birth defects in the family. The pregnancy was planned and uneventful. Delivery was by cesarean section at 39 weeks and 6 days. The patient’s birth weight was 3145 g, with a length of 49 cm and head circumference of 33.5 cm, which are all appropriate for the gestational age. There was no visible perinatal asphyxia. The postnatal course was characterized by psychomotor delay and muscular hypotonia.\nAt the first clinical evaluation at 20 months of age, he showed mild dysmorphic features. The first tooth erupted at 8 months of age. Beginning at 6 months of age, he received regular neurological and pediatric follow-ups. The first smile was noted at the age of 2 months, rolling from a ventral to a dorsal position was observed at 3 months, following objects with his eyes was observed at 6 months, maintaining a sitting position was observed at 8 months, maintaining a standing position was observed at 10 months, responding to his name was observed at 10 months, walking was observed at 20 months, and toilet training was achieved at 2 ½ years.\nAt 6 months of age, physical therapy and an interdisciplinary program of early stimulation were initiated. He was discharged from physical therapy at the end of the second year of life. Psychologic evaluation at 2 years of age showed cognitive delay (corresponding to 1 year and 6 months). He was subjected to speech therapy due to difficulties related to sensory-motor aspects and oral language. According to a clinical assessment at 4 years of age, despite having communicative intent, language expression was quite restricted. He also had deficits in psychomotor and cognitive executive function that improved with time, especially regarding sensory integration, and he presented an attention deficit. At 5 years of age, an evaluation of language and cognitive development was performed using the Protocol of Observation Behavioral []. The examinations revealed the following: limited oral language, with attempts to engage in dialogue; language disorder, especially in expressive language; deficit in motor skills and difficulties in the understanding and execution of tasks and games. With respect to activities of daily living, he showed autonomy in eating and dressing.\nAt 6 years and 10 months of age, he measured 125 cm in height (50th – 75th percentile), weighed 26 kg (50-75th percentile) and had an occipital frontal circumference (OFC) of 52 cm (25th – 50th percentile). Dysmorphological examination revealed low-set ears with prominent helices and lobules, hypertelorism (ICD 3.5 cm, >97th percentile), bushy eyebrows, short nose with anteverted nostrils, deep nasolabial furrows, small and open mouth with an open bite and a thin vermilion, a prominent Cupid’s bow of the upper lip and a prominent and everted lower lip. Mild micrognathia was also observed (Figure ).\nThe propositus attends a regular school with support. He communicates well with colleagues and teachers, and he is able to express his wishes through language and to understand simple conversation and demands. Further examinations revealed the following: Magnetic resonance imaging (MRI) showed an intact corpus callosum and a small region of ischemia in the glyptic cavity of the cerebellar region. Electroencephalography (EEG) and audiometry results were normal, and he showed no vision problems.
The patient is a 68-year-old woman who presented to the hospital with less than 24 hours of right upper-quadrant abdominal pain and nausea. She had a past history of alcohol and tobacco abuse, although she had been abstinent from both for five years at the time of presentation. No HIV risk factors were identified. She did not have a fever, and she had moderate abdominal tenderness on examination. Her chest X-ray was interpreted as showing a right lower-lobe infiltrate (), and she received intravenous ceftriaxone and azithromycin for suspected community-acquired pneumonia. Because of persistent pain, an abdominal ultrasound was performed, which was negative for gallbladder wall thickening, stones, or pericholecystic fluid. A hepatobiliary iminodiacetic acid (HIDA) scan was also normal. The antibiotics were continued. The third day in the hospital, her abdomen became more diffusely tender and she developed atrial fibrillation with a rapid ventricular response. Her heart rate was controlled with intravenous diltiazem. An abdominal CT scan was performed, and it revealed multiple dilated loops of bowel anterior and superior to the liver, with associated free fluid (). Laboratory studies demonstrated a leukocytosis of 13,000 white blood cells/mm3 with a left shift and two-dimensional echocardiography revealed a moderate pericardial effusion, without evidence of hemodynamic compromise. She was taken to the operating room, where she was found to have an internal hernia, with an ischemic loop of ileum incarcerated between the liver and the diaphragm. The hernia was reduced, and the ischemic segment of bowel was resected. She also underwent a cholecystectomy during the same procedure. Ciprofloxacin and metronidazole were administered postoperatively.\nOn the first postoperative day, control of her atrial fibrillation worsened. An echocardiogram was repeated, revealing an increase in size of the pericardial effusion. The cardiothoracic surgical service was consulted, and two days later she was taken back to the operating room and underwent drainage of the effusion and construction of a pericardial window. The pericardial fluid contained 2 white blood cells/μL, with a manual differential of 33% polymorphonuclear leukocytes, 44% lymphocytes, and 23% monocytes. The total protein was 2.9 g/dL and the glucose was 131 mg/dL. Microbiologic stains of the pericardial fluid were negative for bacterial, fungal, and acid-fast organisms, and cultures were ultimately negative as well. She improved following the procedure and was discharged home 6 days later, on no antimicrobial therapy.\nThe patient returned to the hospital 19 days later due to a complaint of worsening shortness of breath. Examination showed her to have a low-grade fever of 38°C and diminished breath sounds in the right chest. She had a white blood cell count of 24,500/mm3 with 18% band forms. The remainder of her CBC was normal. Her chemistries and electrolytes were unremarkable, except for a depressed albumin of 1.8 g/dL. Blood cultures were obtained and were negative. Her chest X-ray showed a right upper-lobe infiltrate with a large associated effusion (). She was started on piperacillin/tazobactam and azithromycin, intravenously. Review of her chest X-ray suggested that she likely had several intrathoracic fluid collections. A CT scan of the chest was performed, demonstrating a large, multiloculated fluid collection in the right chest, with multiple air-fluid levels (). There was a mass effect on the mediastinal structures. It could not be determined whether the fluid was contained above or below the diaphragm, so the patient was taken to the operating room, where an initial retroperitoneal approach showed that the fluid was in fact in the thoracic cavity. She then underwent thoracoscopy, which immediately encountered 1000 mL of thin, brown fluid and gas. Decortication was attempted via the thoracoscope, but ultimately was completed via open thoracotomy. Gram stain of the pleural fluid was negative, but anaerobic cultures yielded Clostridium septicum. The organism was identified using the RapID ANA II system (Remel Products, Lenexa, KS, USA). The organism did not produce β-lactamase. No additional susceptibility studies were performed. There was no growth in aerobic cultures. Her antibiotics were changed to aqueous penicillin G, 3 million units IV every four hours, and she was discharged home from the hospital on the sixth postoperative day to complete four weeks of parenteral antibiotic therapy at home. She made a full recovery, and a colonoscopy, performed to rule out a colonic neoplasm, was negative. She is still doing well, now over five years later.
A 45-year-old, gravida zero para zero, female presented with a one-week history of a fluctuant mass and erythema in the right superior breast. She had a history of seat belt injury to the right breast seven years prior, and had felt stable masses in the breast for two years prior to presentation. After admission to the hospital, intravenous antibiotic therapy was initiated for symptoms of infection. No family history of breast cancer was noted at that time. The work-up for presumed mastitis began with a bilateral diagnostic mammogram. The provided patient history included a possible diagnosis of cellulitis with imaging to rule out an abscess of the right breast. The ordering physician also emphasized the history of seat belt injury. The admission diagnostic mammogram revealed heterogeneously dense breasts, as well as the presence of fat necrosis in the upper outer quadrant of the right breast at the 12 o’clock position (Figure ).\nNo significant masses, calcifications, or abnormalities were noted in the left breast at that time. Ultrasound of the right breast demonstrated edema with no evidence of malignancy. The patient was diagnosed with cellulitis of the right breast and discharged with antibiotics.\nTwo weeks later, the same patient returned with exacerbated erythema, hardness, and tenderness in the right breast. In addition, she also noted a new lump in her left breast which she had not noticed before and mentioned this for the first time to the radiologist while ultrasound is being performed on the right side. The right breast showed redness, induration, and tenderness in the upper outer quadrant. Subsequent diagnostic ultrasound of the left breast revealed an irregularly shaped hypoechoic mass with microlobulated margins. The mass measured 21 x 18 x 14 mm and was located at the 3 o’clock position, 3 cm from the nipple (Figure ).\nUltrasonography of the right breast revealed only fat necrosis and edema consistent with the patient history. Overall, the imaging was given a BI-RADS assessment of 4C, which is a moderate concern for malignancy.\nUltrasound-guided biopsy of the left breast revealed invasive ductal carcinoma, a moderately differentiated nature, and a grade of two with components of ductal carcinoma in situ. Follow-up mammography was performed, showing proper placement of a marker in the clinically observable mass (Figure ).\nThe patient's care was transferred to an outside facility following a definitive diagnosis. The patient ultimately received bilateral mastectomies with sentinel lymph node dissections and adjunctive chemotherapy.
A 72-year-old female was initially found to have morphea on the skin 4 months prior to presentation with 3 months of progressive thickening of the skin on the ankle, chest, back, arms, and neck. The findings were diffuse, and there was no fever, myalgias or arthralgias. The skin changes were asymmetric and not associated with any pain or pruritis; however they had been progressively worsening over the past 3 months (). She reported decreased mobility of her extremities as a result. There were no other identifiable pertinent systemic manifestations outside of the skin. This presentation was associated with an elevated ESR, but negative antibodies for lupus, scleroderma and other autoimmune processes. As a result, a skin and muscle biopsy was done and the findings revealed a thickened fascial layer associated with a lymphocytic infiltrate. The patient was started on 60 mg of prednisone and was prescribed the medication from April 2013 to March 2014. The patient had improvements in skin thickening throughout the body and was started on topical fluocinolone acetonide 0.01% once oral steroids were discontinued.\nThe patient was re-evaluated in the year of 2018. Her skin thickening showed improvement and had a return of nearly normal ankle motion. She was able to walk downstairs normally and exercise comfortably. She took precautions such as limited sun exposure and decreased strenuous exercise. However she noticed that some areas of skin thickening had returned after 3 months on the posterior aspect of the right lower extremity. She attempted use of topical corticosteroid; however the lesion did not resolve and within a few months, this lesion became ulcerated (). The patient underwent biopsy which revealed SCC. After that, wide and deep excision skin biopsy involving muscular layer was performed (). Wound care was able to reduce the size to a superficial ulcer of about 2 × 3 cm diameter with good granulation tissue. She returned for routine follow-up without signs of recurrence of EF or carcinoma.
A 62-year-old male patient was admitted to the hospital with complaints of aggravated dyspnea during exercise and dizziness. Three years before his admission, he received an artificial pacemaker because he was suffering from sick sinus syndrome. A physical exam noted pitting edema on both of his legs. He weighed 80.45 kg at admission while his healthy body weight was 74.5 kg. Echocardiographic assessment revealed regional wall motion abnormality (RWMA) and an enlarged LA and LV and decreased LV systolic function with an EF of 47%. He was diagnosed with dilated cardiomyopathy. A cardiac positron emission tomography-computed tomography scan confirmed that he had cardiac sarcoidosis, so he was prescribed steroids. He was discharged from the hospital on day (HOD) #10 with plans to titrate steroid doses in an outpatient clinic.\nHe attended the group session for HF patients before being discharged and revisited the clinical dietitian twice after being discharged. His usual nutritional intake and anthropometric measurements are presented in and . He usually ate 3 meals regularly throughout the day but ate a large amount of food. His NQ score was 61.1, which was in the good range and his diversity score was 88.4, which was in the high range. He said that as much as he wanted of a variety of foods and did not consider his health when making eating choices. He usually ate 2,175 kcal/day and 85 g of protein/day, which were 128% and 113% of the recommended amounts, respectively. He selected 8 out of 10 in the checklist to assess his sodium intake patterns, which showed that he was at high risk of consuming a high amount of sodium. He was obese with a BMI of 26.8 kg/m2 based on his healthy body weight. His nutritional diagnoses were excessive food and beverage intake and excessive sodium intake.\nAt his first follow-up session, his food diary showed that he had reduced his food intake after discharge, but he still frequently ate kimchi stew and soybean paste stew, which are high-sodium foods. He ate more vegetables and nuts and less fruit; protein-dense foods, such as meat, eggs, and beans; and ramen and other instant foods. Analysis of his 3-day food diary revealed that he consumed 97% of the recommended amount of calories and 87% of the recommended amount of protein. The clinical dietitian instructed him to consume an adequate amount of lean meat and meat substitutes because the patient's protein intake decreased to less than 90% of the recommended amount as a result of the fact that he avoided most protein-dense foods. A clinical dietitian gave him individualized nutrition education and counseling to teach him how to choose foods and plan meals to reduce his sodium intake. The second follow-up session took place 4 months after the first follow-up session. He selected 4 out of 10 in the checklist to assess his sodium intake patterns, which showed that he was at moderate risk of consuming a high amount of sodium. He said that he was not adding table salt or extra sauces to foods and reduced his consumption of delivery food. His overall NQ score and each of his category scores had increased, but his food intake moderation score had increased the most. He consumed 103% of the recommended amount of calories and 96% of the recommended amount of protein. His BMI had decreased to 24.5 kg/m2, which was lower than the cutoff for obesity diagnosis as defined by the Korean Society for the Study of Obesity.
A 60-year-old male presented to the hospital following a syncopal event that was preceded by an ICD shock shortly after exposure to marijuana.\nThe patient has a past history significant for a large anterior myocardial infarction and severe three-vessel coronary artery disease (CAD). The patient was not deemed a bypass candidate and has received a single chamber ICD (Medtronic EnTrust D154VRC) for prevention of sudden cardiac death (SCD) in context of a left ventricular ejection fraction of 20%.\nThe patient presented to hospital on this occasion following a left eye orbital rupture sustained during a fall due to the syncopal event shortly after smoking 4 "bongs" of marijuana. The patient experienced an ICD discharge prior to the loss of consciousness. There were no other associated symptoms. At no time did the patient have clinical signs or symptoms of angina or cardiac failure. The patient gives a history of experiencing an ICD discharge while smoking the same amount of marijuana 6 months prior. With both occasions, the patient had used 4 bongs rather than his usual 2 to 3 bongs of smoking. He did not seek medical attention at that time. Other than these two episodes of ICD discharge following more than his usual marijuana exposure, he denied any other syncope or ICD discharge.\nThere was no family history of SCD. The patient was receiving at home enteric coated aspirin 81 mg daily, carvedilol 25 mg twice daily, ramipril 10 mg once daily, atorvastatin 20 mg once daily, spironolactone 25 mg once daily, furosemide 40 mg once daily, diazepam 10 mg once daily, rabeprazole 10 mg as needed for heartburn, all without any changes in the two months prior to this episode. He denied any other recreational drug, or alcohol use. Home oxygen was used at night during sleep. The patient reports use of marijuana daily using a water pipe. He typically uses 7 or 8 "bongs" throughout the day, usually 2 to 3 "bongs" at one time. On the occasions that he inhaled a fourth "bong" consecutively, his defibrillator discharged.\nInvestigations revealed no biochemical or electrocardiographic evidence of ischemia. Following repair of the globe rupture, the ICD was interrogated and revealed one event in the VF zone (rate > 188 bpm) which was adequately detected and treated with one appropriate shock. The device delivered anti-tachycardia pacing during charging and a 35 J shock (Figure ) that was successful to restore normal sinus rhythm. During the interrogation, the device was reprogrammed to decrease the VT/VF detection from 12/16 to 6/8. The device was found to be otherwise functioning normally. The patient made an uneventful recovery and was discharged home after 2 days of intravenous amiodarone therapy and was discharged with a prescription for oral amiodarone and strong advice of quit smoking.
A 77-year-old man with a past medical history of type 2 diabetes, hypertension, and ESRD underwent deceased donor renal transplantation. Two months following his renal transplant, the patient was admitted for an acute kidney injury discovered on routine follow-up laboratory testing. From a baseline creatinine of 1.3 mg/dl after the transplant, he was noted to have a creatinine of 2.7 mg/dl and a renal biopsy was obtained. Histopathology was suggestive of mildly active cellular rejection and acute tubular injury but with no concerns for antibody-mediated rejection. The patient received three days of methyl-prednisolone 250 mg daily intravenously and was subsequently transitioned to high-dose oral prednisone with a taper.\nThe patient was readmitted within three weeks of his renal biopsy with worsening renal functions, now with a creatinine level of 3.8 mg/dl. At this time, he was on prednisone five mg daily, tacrolimus four mg twice daily, and mycophenolate 500 mg twice daily. His family reported poor oral intake and that he had been taking furosemide at home. He received intravenous (IV) normal saline and diuretics were held. However, as his renal functions did not improve beyond a creatinine level of 2.1 mg/dl, the decision was made to perform a repeat renal biopsy. During ultrasonography for the renal biopsy, concerns were raised for a possible renal artery aneurysm. An ultrasound of the right lower quadrant and transplant kidney subsequently showed a 3 cm x 3.4 cm x 4 cm aneurysm proximal to the renal artery anastomosis to the right external iliac artery (Figure ). The right external iliac artery to renal artery anastomosis was patent and the renal vein was noted to be patent as well. No peri-transplant kidney fluid collections were noted and no hydronephrosis was seen.\nSubsequently, a pelvic arteriogram was performed, which showed patent pelvic and iliac arterial flow. However, a large pseudoaneurysm arising directly off the right external iliac artery was re-noted and the renal transplant artery was noted to be filling from the distal side of the aneurysm and remained patent, although with sluggish flow (Figure ). A second known renal transplant artery was not seen and was presumed to be thrombosed.\nWhile the interventional radiologist considered closing off the pseudoaneurysm by stent placement, the origin of the aneurysm was noted to be too close to the anastomosis to allow for stent placement. Hence, transplant surgery was consulted. The patient was taken to the operation room for a re-exploration of his transplanted kidney and revision of the arterial anastomosis. The patient underwent exploratory laparotomy, and intraoperatively, significant inflammation and scar tissue surrounding the hilum of the transplanted kidney was seen. Once the pseudoaneurysm was entered, necrotic tissue and purulence within the pseudoaneurysm were noted. It was felt that the arterial flow to the transplanted kidney could not be restored and, therefore, a decision was made to proceed with the resection of the infected pseudoaneurysm and transplant nephrectomy. The transplanted kidney was explanted. A portion of the pseudoaneurysm was sent to pathology for further characterization, and a portion was sent to microbiology for culture. The explanted kidney was also sent to pathology for further analysis. The patient was transferred to the intensive care unit (ICU) postoperatively for further management and close monitoring. During the surgery, the patient received crystalloids and multiple blood products.\nOn arrival to the ICU, the patient had temporary dialysis access placed and started on continuous renal replacement therapy (CRRT). Given concerns for an infected pseudoaneurysm, he was empirically started on renally dosed vancomycin, piperacillin-tazobactam, and fluconazole. All immunosuppressive agents were discontinued at this time. Due to persistent vasopressor requirements while in the ICU, the patient was switched to vancomycin, meropenem, and micafungin. The infectious disease team was consulted for recommendations. Blood bacterial and fungal cultures remained negative. However, fungal cultures from the pseudoaneurysm grew Aspergillus flavus on three separate samples. The patient was then transitioned to IV isavuconazonium 372 mg every eight hours for six doses, followed by 372 mg daily with a plan to continue for at least six weeks. Vancomycin and meropenem were discontinued. The pathology of the explanted kidney showed transmural necrosis of the renal artery, no evidence of rejection, and scattered micro-abscesses within the parenchyma. As the patient improved clinically, he was transitioned from CRRT to intermittent hemodialysis. He was subsequently transferred out of the ICU in stable condition. His hospitalization was complicated by colitis secondary to Clostridium difficile for which oral vancomycin was initiated. Preparation was made to discharge him to an acute rehabilitation facility; however, a day prior to discharge, he was found to be unresponsive by his nurse and despite prolonged resuscitation attempts for cardiac arrest, could not be revived. The exact etiology for this sudden demise could not be determined.
A South Indian boy aged 14 years, reported to the Department of Pedodontics and Preventive Dentistry, accompanied by his father with the chief complaint of broken upper front teeth. History revealed trauma 1 month before while playing and there was no loss of consciousness at the time of injury. There was history of bleeding from the teeth after the injury with sensitivity on taking hot and cold substance and pus discharge through the broken incisors 2 weeks later. Health history of the patient was not contributory.\nPatient showed bilaterally symmetrical face with competent lips. Intraoral examination revealed permanent dentition with fracture of both maxillary permanent central incisors () and pus discharge through the open pulp chambers of both centrals. Bilateral class I molar relationship was noted with localized gingivitis in relation to permanent first molars.\nProvisional diagnosis of Ellis class IV injury was made as involving maxillary permanent central incisors. Intraoral periapical radiograph taken for maxillary central incisor area showed periapical radiolucency in relation to central incisors, presence of an inverted supernumerary tooth in the periapical area of the right central incisor and the presence of blunderbuss canal for left central incisor (). The final diagnosis was Ellis class IV injury in maxillary permanent centrals and inverted supernumerary tooth in relation to maxillary right central incisor.\nTreatment planning included, completion of root canal treatment in maxillary right central incisor () followed by extraction of the inverted supernumerary tooth under local anesthesia and apexification for maxillary left central incisor. Palatal crevicular incision was placed from maxillary right canine to maxillary left canine and mucoperiosteal flap was raised exposing the bulge of inverted supernumerary tooth present at the apex of maxillary right central incisor. Bone is removed until the crown of the supernumerary tooth is exposed. The crown of the supernumerary tooth was sectioned with a fissure bur and removed as first piece and the root is elevated into the space vacated by the crown and removed (). A portion of the root apex of the maxillary right central incisor had to be sacrificed during the procedure as it was closely related to the root of the supernumerary tooth. After careful debridement, which included complete removal of the remnants, and achieving hemostasis the flap is repositioned and sutured interdentally. Apexification was planned for maxillary left central incisor with Mineral Trioxide Aggregate (MTA, Angelus®, Brazil). Maxillary left central incisor was isolated using rubber dam and working length was determined about 16 mm. MTA was mixed in the ratio of 1:1 on a sterile glass slab, placed into the canal and condensed using reverse ends of the paper points. Intraoral Periapical (IOPA) radiograph was taken after appropriate amount of MTA was condensed into the canal (). After 15 minutes interval obturation using gutta-percha points was completed in maxillary left central incisor. After 3 weeks of apexification composite build-up was done ().
A 12-year-old female child reported to the Department of Pedodontics and Preventive Dentistry with complaint of missing right eye (). Detailed and careful case history recording revealed that the patient had been diagnosed having retinoblastoma of the right eye and the affected eye had to be enucleated. Patient examination consisting of internal examination of the anophthalmic socket revealed a healthy epithelial lining. Following describes the procedure of eyeball prosthesis. Patient in erect position, seated, to allow the impression of the tissues involved in the defect to record in their natural drape during active posture.\nPatient instructed to gaze straight ahead while making the impression of the socket with light bodied rubber base impression material. The impression material was slowly injected into the socket taking care to avoid any air bubbles. The patient was instructed to make various eye movements to get functional impression of the eye. The impression material was reinforced with a syringe needle cover to hold it in place and for ease of removal after it sets (). After boxing the eye region, external facial impression was made with irreversible hydrocolloid (), allowing the material to combine with that of the extruded material, this facilitates the retrieval of the entire impression. In globe formation, a 2-piece dental stone mold was poured to immerse the lower part of the impression. After the stone had set, separating media was applied on the surface. Then the second layer was poured. Grooves were made on all four sides of the cast for proper reorientation of the cast. The impression was separated from the cast of the defect and lubricated the stone cast with a thin coating of vaseline. The lubricated socket of the working cast was filled with molten wax and after solidification; the retrieved wax form was smoothened and polished for try-in on the patient's face (). Vaseline was applied to the tissue surface of the wax pattern to avoid irritation to the tissues, was placed into the clinical defect by introducing it first under the upper lid, and then over the retracted lower lid. Several minutes are required for relaxation of the protective blepharospasm, which may occur when the wax pattern is first placed in the socket. With the wax form in place, and with the patient's eyes closed, both socket areas were palpated simultaneously to compare globe sizes. Modify the wax pattern and its corneal prominence, where necessary, to duplicate the shape of the natural eye and eyelid drape of both eyes was matched. Retract the eyelids and corneal surface was exposed to adjust the wax form to give the best duplication of globe contour. The corrected wax pattern was flasked and processed in tooth-colored acrylic prematched with natural sclera of the unaffected contralateral eye, selected using the tooth-colored acrylic shade guide. Processed resin globe was retrieved from the flasking matrix in a way that preserved the outer stone matrix and later allows reseating of the modified globe back into this matrix for eventual reprocessing of the globe's original contour. In iris characterization, a processed resin globe, with a high polish, placed in the patient's socket for evaluation, and made necessary adjustment to effectively simulate the normal corneal contour as accurately as possible. The patient was instructed to hold an erect position and to gaze straight ahead and observed from the side to determine the iris plane relationship with the normal eye. The distance from the pupil of the normal eye to the midline was used in establishing the horizontal position of the prosthetic pupil center and marked on the globe. The vertical position of the pupil center is determined and marked by the canthus relationships. The diameter of the iris was measured holding a ruler close to the normal steadied eye. The globe form with its marked pupillary center is removed and the iris size that is 1 mm smaller than the diameter of the measured iris is circumscribed with a compass from the established point. Return the globe to the clinical defect and the outlined iris evaluated in relation to that of the eyelids. Accurate iris positioning is critical in the establishment of a natural appearance. The ocular globe modified by cutting away resin within the circumscribed area providing a chamber to house a photographic digital image. In color characterization and globe completion, the iris photographic image of approximately 1 mm smaller than the diameter of the measured patient's iris was cut () as this will be compensated for, by the magnification caused by the overlay of clear acrylic resin in the completed prosthesis. If necessary, further customization and color modifications are performed using professional quality color pencils. The paper iris was covered with three light coats of water resistant spray and attached to the excavated recess of the globe. The remaining outer corneal surface was characterized by removing a thin layer of acrylic resin and using professional quality color pencils, scleral blood vessels were drawn along the outer periphery. Soft color tones of yellow and brown were added onto the medial canthal area to simulate the normal eye (). Evaluation was done in the patient and the characterized globe form was returned to its original position within the initial flasking matrix. The space created over the disk and between the reduced outer corneal surface of the globe and the stone matrix of the flasking was packed and processed with clear acrylic resin. The retrieved processed ocular globe was trimmed and polished to a high finish using pumice and was critically evaluated for lid drape, contour, iris color and dimension (). The patient was taught to properly insert and remove the appliance and the importance of careful cleansing and handling of the prosthesis was emphasized.
We reported a case of a 19-year-old male patient, who was initially admitted to our hospital with a picture of acute appendicitis. He was complaining of 1-day history of right side and lower abdominal pain. On examination at that time, there was tenderness and rebound tenderness at the right iliac region, with no palpable masses and intact hernial orifices. His blood tests showed elevated white blood cells. Thus, he was diagnosed with acute appendicitis and underwent laparoscopic appendectomy on the same day. His appendix was acutely suppurative inflamed and perforated at its middle, with purulent fluid in the pelvis. The appendix was cut and removed through the left iliac port, and the turbid fluid in the pelvis was sucked first, and then dried by a gauze. There was no evidence of irreducible inguinal hernia.\nAfter surgery, the patient was kept on intravenous (IV) antibiotics for 2 days.\nOn the third day after the admission, the patient’s condition improved. His vital signs were normal and his wounds were dry and clean. His abdominal examination was unremarkable, so he was discharged from the hospital on oral antibiotics (cefuroxime and metronidazole).\nSix days later after discharge from hospital, he presented again to our hospital complaining of a painful swelling at his right inguinal region extending from the groin to the base of the scrotum and reaching the right testis, which had appeared suddenly 3 days previously (3 days after hospital discharge). Apart from that, he had no gastrointestinal symptoms or fever or urinary symptoms. That was the first time for the patient to notice such swelling. Examination revealed a hard and tender swelling, which was extending from the right inguinal region to the base of the scrotum with red and edematous skin over. There was no impulse on cough. His abdomen was soft and the other examinations were unremarkable. His complete blood count, renal function test and urine analysis tests were normal.\nThe patient had soft tissue and scrotal ultrasound, which was reported: ‘There is a right inguinal hernia with omentum and heterogenous contents manifested by 2 large septate hematomas measuring 2x4 cm and 3x4 cm extending into the right scrotum. Both testis are normal in size’.\nThe patient was diagnosed with incarcerated right inguinoscrotal hernia, inflamed, omentocele with possible gangrene.\nHe underwent emergency open hernia repair surgery, which revealed irreducible oblique hernia containing omentum, which was inflamed and gangrenous at its tip with turbid fluid in the cord ( and ). The omental contents were excised and the hernia was repaired anatomically, without mesh insertion. The patient was kept in the hospital after surgery for 2 days on IV antibiotics and discharged home.
An 81-year-old woman with long history of gallstones was presented to the emergency department with persistent vomiting associated with progressive abdominal distension and inability to pass bowel motion or gases for the past 3 days. Examination showed stable vitals and distended abdomen. A computerized tomography (CT) scan showed high-grade small bowel obstruction in mid-ileum caused by a large impacted gallstone (). There was also a wide neck cholecystoduodenal fistula ().\nThe patient was taken to the operating room (OR) for diagnostic laparoscopy that revealed small bowel obstruction with a transition zone at the mid-ileum. An enterotomy proximal to the obstruction point was made and the stone was removed. The stone was round in shape, not faceted (). The rest of the bowel was examined from the Ligament of Treitz to the ileocecal valve with no other stones identified. No attempt was made to explore the right upper quadrant as there was no plan to take down the fistula during this surgery.\nPostoperative course was marked by a slow recovery evidences by persistently high nasogastric tube output and failure to open her bowel. On the third postoperative day, a trial of therapeutic Gastrografin was given, not much improvement was achieved, and the patient continued to be obstructed. A repeat abdominal CT scan was obtained on day 5 due to persistence of intestinal obstruction. It showed dilated contrast-filled small bowel loops with multiple filling defects, indicating recurrent GSI ().\nThe patient was taken back to the OR for diagnostic laparoscopy. Small bowel was markedly dilated and edematous, which made manipulation of small bowel loops difficult. Ascertaining locations of stones was not easy; therefore, laparoscopy was aborted and a mini-midline laparotomy incision was created. Palpation revealed multiple stones along the length of the small bowel. The previous enterotomy site was re-opened and the bowel was milked from the ligament of Treitz downward. A total of eight varying size stones were retrieved; largest measured 2 × 3 cm in size. Enterotomy site was used to perform a side-to-side stapled anastomosis. Postoperatively, the patient opened her bowels and tolerated diet. She left the hospital on the third postoperative day.
We report a rare case of IVC aneurysm in a 22-year old Afghan-Iranian male patient. The patient had a history of blunt abdominal trauma one week prior to his referral to the emergency department of our center. On his initial abdominal trauma, a complete physical examination and focused assessment with sonography for trauma (FAST) was done. The investigations were normal and the patient was discharged from the emergency department. The patient has had vague abdominal pain after his discharge.\nOn the referral of the patient to our center, we planned an abdominopelvic computed tomography (CT) scan with oral and IV contrast. The scan illustrated an IVC saccular aneurysm originating from right side of the IVC below the renal veins (). We assumed two possible etiologies. The aneurysm could incidentally and in another hand it could be related to the patient’s recent history of abdominal trauma. Magnetic resonance venography was also conducted and it also confirmed the diagnosis of a saccular type III IVC aneurysm ().\nWe planned open resection and repair of the aneurysm. A midline laparotomy was done. After thorough exploration of the abdominal and pelvic cavities, a right medial visceral rotation was conducted by mobilization of the right colon and a Kocher maneuver (The Cattel-Braasch Maneuver). The right kidney was left in situ. The entire sub-hepatic IVC was exposed. A saccular aneurysm with dimensions of 4*5 cm was found on exploration (). The aneurysm was located below the renal veins and the neck of the aneurysm was at the right side. The aneurysm was confined to the infrarenal IVC and there was not any associated venous anomaly. Thus, it was a type III saccular IVC aneurysm. A partial Satinsky clamp was applied posterior and left to the site of aneurysm origin on IVC and a longitudinal incision was done anterior to the neck of the aneurysm. Then, the entire aneurysm was resected. The neck of the aneurysm was closed with lateral venorrhaphy by running 6.0 polypropylene sutures ().\nThe patient had well recovery after the operation. Postoperative anticoagulation was administered by unfractionated heparin and warfarin. Warfarin anticoagulation was continued for three months to prevent venous thrombosis and probable pulmonary embolism. The patient’s follow-up did not reveal any morbidity. Postoperative CT scan was also conducted on seventh postoperative day. Postoperative appearance of IVC was normal ().
A 57-year-old female with widely metastatic high-grade serous adenocarcinoma of the ovary was referred to our tertiary palliative care clinic by her oncologist for management of severe cancer-related pain. Following her original diagnosis, she underwent a number of multimodal disease-directed therapies, including surgical resection and chemotherapy. She had significant ongoing mixed somatic and neuropathic pain in the left lower back as well as her pelvis, both sites of known metastatic disease. The back pain was noted to be a deep sharp pain without radiation to her extremities, located primarily around the region of her nephrostomy tube and into the abdomen. She would also have sharp lancinating neuropathic pain in her pelvis due to tumor burden with prolonged periods of sitting, walking, or activity.\nInitial treatment consisted of multimodal pharmacologic therapy including short-acting and long-acting opioids with nonopioid and adjuvant neuropathic agents. Despite these therapies, she had progressive pain and was ultimately increased to an opioid combination of morphine sulfate controlled-release (120 mg by mouth every 8 hours) and morphine sulfate immediate-release (30–45 mg by mouth every 3 hours as needed). Early changes yielded acceptable analgesia. Conversations and medication adjustments continued over the phone, given the distance between the patient's home and our institution. However, over several weeks' time, pain progressed despite further opioid increases. Given concern for escalating pain and poor response to several attempts at altering her opioid therapy, it was advised that she present for a consultation in the palliative medicine clinic. Fortunately, focused discussions with the patient and her family around the patient's goals of care were addressed with her palliative medicine team at every meeting. She was very clear that her top priority in her ongoing medical care was to achieve acceptable analgesia. During the interview in the clinic, the patient noted significant pain with generalized discomfort, a sense of restlessness, and new muscle fasciculations. Her husband accompanied her and noted intermittent confusion. Her physical exam featured delirium (Confusion Assessment Method positive) with diffuse myoclonus.\nOut of concern for opioid-induced neurotoxicity (OIN), the patient was admitted to the hospital for analgesic control and treatment of what was felt to be a toxic encephalopathy. Despite the known shared metabolic pathway (phase II metabolism) between morphine and hydromorphone, the patient's opioids were rotated from oral morphine to parenteral hydromorphone as the clinical picture continued to unfold. Further workup revealed an unremarkable head CT and EEG. Clinical evaluations and laboratory assessments suggested that, in the setting of her declining physical and renal function (creatinine 1.0 mg/dL with prior baseline 0.6 mg/dL and estimated glomerular filtration rate (eGFR) 55 ml/min/BSA with prior baseline >60 ml/min/BSA), the delirium could be the result of an accumulation of active neurotoxic morphine metabolites (morphine-3-glucuronide). In addition, she was dehydrated, constipated, and had not slept in several days. Unfortunately, her delirium persisted despite correction of metabolic derangements, hydration, and an aggressive bowel regimen.\nUrology was consulted, and a nuclear medicine dimercaptosuccinic acid (DMSA) scan revealed minimal function of the left kidney, despite the presence of a nephrostomy tube. Subsequent vascular radiological investigations revealed tumor-induced thrombosis with surmised infarction of her left kidney. It was concluded that the altered renal function due to infarction likely resulted in an inability to adequately excrete the morphine, and the accumulation of polar morphine metabolites resulted in OIN. As exemplified in this case, even with opioid rotation, centrally active metabolites of hydromorphone (hydromorphone-3-glucuronide) theoretically exist, given that it follows a similar phase II metabolic glucuronidation pathway as morphine, although the relative potency and effect of such hydromorphone metabolites are thought to be significantly less than morphine. The patient continued to have signs of OIN and was eventually transitioned to parenteral fentanyl, given that fentanyl is a unique medication that is largely hepatically metabolized into inactive metabolites. Despite common lore that fentanyl and methadone are agents that do no produce active metabolites and therefore do not elicit OIN, case reports of synthetic opioids eliciting OIN do exist in the literature [, ]. Unfortunately, despite aggressive supportive measures and conversion of parenteral hydromorphone to parenteral fentanyl without reduction for cross-tolerance, she continued to have persistent pain with minimal resolution of her systemic neurotoxicity.\nGiven the concern for poorly controlled cancer-associated pain, the palliative care and pain medicine teams collaborated from the initial visit with concerning symptoms of OIN. At our institution, the palliative clinic shares space within the pain clinic, and the collaboration required a simple conversation followed by same day pain consultation. Initially, the shared thought was moving directly to intrathecal (IT) TDD therapy, as this had been discussed over numerous palliative care visits as a potential option with her physicians and was in line with her goals to achieve enhanced pain control with limited side effects. However, given the underlying metastatic disease process and concurrent cancer treatments, she had a metabolic coagulopathy due to nutritional and micronutrient deficits that required correction with vitamin k to assist in the reversal of her international normalized ratio (INR), thus allowing for any type of procedural intervention.\nUltimately, given the persistent multifactorial delirium and rapidly changing situation, the pain medicine and palliative care teams convened a family conference with the husband, the patients' health-care power of attorney, to again address the current situation and make recommendations that seemed consistent with the patient's predetermined goals. Given the rapid progression of her symptoms despite aggressive attempts to correct potentially remediable factors, the shared medical decision-making discussion included moving forward with TDD, given that this therapy was thought to be the best chance for meeting the patients' goals for comfort and hope to have meaningful interaction with family by reducing the burden of systemic side effects from oral or parenteral opioid therapy. The patient's advanced directive was clear that she appointed her husband to make decisions on her behalf if she was ever in a situation where she was unable to provide consent. Informed consent was obtained through her appointed surrogate decision maker and designated health-care power of attorney.\nAfter correction of her metabolic coagulopathy yielding an INR <1.2, she proceeded with placement of an intrathecal TDD system (Medtronic SynchroMed™ II 40 mL pump and Ascenda catheter). The pump reservoir was placed in her right lower abdominal quadrant, and the catheter tip was placed at T10. Her TDD system was efficiently titrated to achieve acceptable levels of analgesia using a combination of opioid (hydromorphone 2 mg/mL) and local anesthetic (bupivacaine 10 mg/mL). The initial postoperative settings were 0.5 mg/day of hydromorphone in a simple continuous mode without bolus dosing (2.5 mg/day of bupivacaine). Within 24 hours of TDD placement, the patient experienced near complete resolution of her toxic encephalopathy and was able to engage in meaningful conversation with her family and health-care teams. There were no signs or symptoms of opioid withdrawal. In the early postoperative phase, she required additional dosing of oral hydromorphone, but was responsive to 2 mg orally every 3 hours as needed for breakthrough postsurgical pain totaling three to four doses per day. Given the robust response to TDD therapy, the patient was able to participate in goals of care discussions with her interdisciplinary palliative care team and elected to discharge closer to home under the provision of hospice services postoperative day (POD) two. Prior to leaving the hospital, the TDD rate was increased to 0.8 mg/day of hydromorphone, and she was provided with a personal therapy manager (PTM) allowing 0.1 mg/dose every six hours totaling four doses per day. The dose remained stable for seven days. As her oncologic disease continued to evolve, her TDD system was adjusted by her hospice provider to accommodate her daily use of oral opioid therapy. The pump was again adjusted on postoperative day nine, yielding a basal rate of 1.4 mg/day of hydromorphone with four PTM boluses of 0.15 mg/dose of hydromorphone every six hours. This dose remained for POD 9–13, and she passed away peacefully with acceptable analgesia at home with her family under the auspices of hospice two weeks after TDD implantation.
A 41-year-old female with a past medical history of migraines, hypertension, and EDS IV with multiple prior spontaneous vascular dissections (including superior mesenteric, splenic and bilateral iliac arteries) originally presented to the hospital with acute chest pain. CT angiography of the chest was performed and demonstrated occlusion of a 2 cm segment of the proximal right coronary artery (), which was likely due to dissection given her history and predisposition. Imaging also demonstrated a moderate-sized hemopericardium. During acquisition of the CT images, the patient suffered a cardiac arrest on the CT scanner table and a cardiopulmonary arrest code was initiated. The patient was found to be in ventricular fibrillation. Return of spontaneous circulation was achieved after 1 min of chest compressions and a single 200 Joule defibrillation. Cardiology work-up confirmed an acute ST elevation myocardial infarction (STEMI) complicated by pericardial hemorrhage likely secondary to the acute dissection. However, given her history of EDS IV, performing percutaneous coronary intervention was considered too high risk of a procedure. Conservative management was recommended with close clinical monitoring in the intensive care unit. A complete cardiologic work-up performed during her hospital stay found no further evidence of heart block or physiologic evidence of right ventricular infarction, and the patient suffered no further sinus arrest. The patient was monitored for two more days and discharged in stable condition.\nThe patient returned to the emergency department 2 days later with acute shortness of breath. Initial assessment revealed hypoxia and increased respiratory effort. In concert with the patient’s physical exam, these findings were concerning for pulmonary edema secondary to acute myocardial infarction. A CT angiogram of the chest was obtained. The pre-contrast-media images confirmed the clinical suspicion of pulmonary edema (). The patient was then administered 100 cc of intravenous nonionic iodinated contrast media at 4 cc/s with imaging timed for peak systemic arterial enhancement. However, the angiogram images, which were obtained within 3 min of the pre-contrast-media images, demonstrated a new, large-volume, high attenuation fluid collection occupying a portion of the parenchyma of the middle lobe, with most of the fluid extending into the right minor fissure, findings consistent with spontaneous parenchymal hemorrhage (). Further interrogation demonstrated air-fluid levels within the middle lobe parenchyma and within the pleural space subjacent to the minor fissure, findings consistent with traumatic air cyst formation and hemopneumothorax, respectively (). The CT angiography was not optimized for opacification of the pulmonary arteries and, as such, a source of active extravasation was not visible.\nInterventional radiology was consulted for possible embolization. However, as aforementioned, given the lack of suboptimal imaging parameters for the pulmonary arteries, thereby disallowing visibility of a possible target vessel, and the patient’s underlying EDS IV, conventional angiography and transcatheter embolization was not recommended. Subsequently, the patient and her family decided to pursue comfort care measures only. The patient ultimately expired later that evening due to cardiac arrhythmia secondary to tamponade from her hemopericardium.
A previously healthy male infant was thought to have a normal neurodevelopmental profile till the age of 20-month-old at presentation. He was born at term after an uneventful pregnancy and uncomplicated normal delivery. His family history was negative for any neurological or developmental disorders in close blood relatives. He was noted to have several worrisome observations around the age of 20 months old, including progressive loss of consistent eye contact, consistently irritable mood with inconsolable high pitch cry, and worsening loss of prior vocabulary and speech abilities. He was described as more clumsy and noted to have more frequent falling than usual as he started walking around the age of one-year-old. Multiple family members noticed overall worsening with decreased interaction with family members and usual social cues. Because of the numerous concerning observations, his primary care physician initiated an urgent referral to the neurology specialist service. Upon evaluation by neurology service, he was found to be well-looking with no signs of acute illness. However, he had multiple abnormal neurological exam findings such as poor eye contact, irritable mood, diffusely decreased axial and appendicular tone, along with brisk deep tendon reflexes throughout all extremities. No nystagmus, cranial nerve palsies, striking abnormal facial features, or neurocutaneous signs were noted. No joint contractures or deformities were found. With this concerning neurological decline and abnormal physical exam, a brain magnetic resonance imaging (MRI) with magnetic resonance spectroscopy was completed and interpreted as an unremarkable study. Basic and targeted metabolic screening was obtained, including lysosomal, thyroid, amino acid, fatty acid, and immune disorders, which were all found to be negative. DNA genomic micro-array sequencing test was then done and showed heterogeneous de novo p.L3101 pathogenic variant of GABRB3. Although his variant was not previously reported, it was thought to be pathological and likely represents the underlying etiology of his clinical symptoms and signs. The patient was started on an intensive rehabilitation program. Interestingly, his response to physical, occupational, and speech therapy services were positive and remarkable, and he continues to make developmental progress.
A 10-year-old boy child was referred for assessment of bilateral swelling. He is the fourth in a family of eight children. His previous medical history were uneventful. The parents gave no history of febrile illness, drug utilization or herbal plant. No findings suggest a particular risk period or an environment exposure. There was no history of similar complaint in any of the siblings and parents of affected child. No findings suggest a particular risk period or an environment exposure.\nThe history of present illness dates back about 6 years by gingival bleeding treated successfully by traditional healers in his village. One year after this episode, swelling started initially as smaller in size in left side of the face which gradually increases in left side of face following by right side.\nAt presentation in our center, physical examination revealed bilateral symmetric swelling of the face without pain (). On palpation, temperature of the overlying skin was normal and no tenderness was elicited. There was no lymphadenopathy. No abnormality was found on clinical examination of the chest, abdomen, cardiovascular system. Neurologic exam was normal. It reveals an active and alert child with normal interests and social curiosity. No extra-gnathic skeletal involvement was observed. No cutaneous pigmentation or congenital abnormality was associated. The intra-oral examination shows backward displacement of the tongue, dental eruption abnormalities with absence and displaced teeth, the rudimentary development of molars, abnormally shaped teeth, partially resorbed roots or delayed and ectopically erupting teeth.\nHematological and biochemical investigations for serum calcium, phosphorous, hepatic and renal tests were found to be within normal limits. In contrast, there was an elevation of the Serum alkaline phosphatase levels for age at 984 U/L. HIV and hepatitis serology were negative. No plasmodium was found.\nX-ray shows that the bones were involved with a multilocular radiolucency with thin and expanded cortices, including the inferior border. The condyle and the condylar neck were normal.\nA computed tomography (CT) scan revealed honeycomb-like lesions of the mandibular cortical bone with further progression in the size of the lesion.\nHistopathological examination of the biopsy specimen from the central area of both right and left rami shows loose fibrous stroma, proliferating fibrous connective with tissue interspersed with multinucleated giant cells, small thin walled blood vessels and scattered sparse mononuclear inflammatory infiltrate. The process is highly vascularized and hemorrhagic. No atypical or evidence of malignancy (). This description is suggestive of giant cell lesions of bone including cherubism.\nClinical feature, radiographic feature, histopathological examinations are suggestive of cherubism.\nNo genetic and molecular tests were made because of their unavailability.\nDuring his hospitalization, the child did not receive specific chemotherapy and was kept under follow up with no further surgical intervention and we prefer waiting until the end of puberty before performing a surgical intervention.\nDeath occurred 4 months after admission by a sudden cardio-respiratory arrest probably due to obstructive sleep apnea. Autopsy was refused by the family.
This is a 70-year-old female who is a known case of diabetes mellitus, hypertension, and coronary artery disease. She presented to our clinic in February 2013 with debilitating back pain secondary to degenerative lumbar scoliosis with a multilevel lumbar spinal stenosis. She had posterior spinal fusion from T12 to pelvis with a decompression of the stenotic levels. Two months following her surgery, she presented to our clinic complaining of disabling back pain after a history of falling on her back. This pain was exacerbated by activity and relieved by rest. However, she had no change in her neurological status. Clinical and radiographic findings confirmed T11 and T12 compression fractures. As the patient was symptomatic, the decision has been made to proceed with vertebroplasty and in this way avoid a more extensive surgery with rods and screws. Following general anesthesia, she was positioned in a prone position. Then, under direct fluoroscopy, the correct levels were confirmed followed by a standard percutaneous insertion of trocars into T11-T12 pedicles. High viscosity bone cement was injected into T11 and T12 vertebral bodies under fluoroscopic guidance. At T12, the bilateral trocars were placed using an in-out in technique aiming the tip of the trocar just under the pedicle screws at T12. A bipedicular approach was also used at T11 to inject bone cement. High viscosity bone cement was injected into T11 and T12 vertebral bodies under fluoroscopic guidance. The total volume of injected cement was 4 cc in each level. The patient tolerated the procedure and was discharged uneventfully. Six months following her vertebroplasty, she presented to our clinic with a history of trauma causing back pain, diminishing her ability to ambulate, and worsening neurological symptoms. On clinical examination, she had tenderness over the thoracolumbar spine and generalized weakness of the lower extremity. Radiographs demonstrated a significant 45-degree junctional kyphosis at T11-T12 with a loss of sagittal balance. Subsequent CT-scan showed an incidental finding of a cement pulmonary embolism in the pulmonary trunk and right and left main pulmonary arterial systems (). Patient's spine sagittal imbalance was treated surgically by extending her spinal fusion up to T2 to restore her sagittal alignment. Nine months following her vertebroplasty, the patient started to have a new onset of shortness of breath. Pulmonary embolism was the main working diagnosis. For this reason, a complete cardiorespiratory work-up was carried out. CT pulmonary angiogram demonstrated an unchanged cement embolus within the pulmonary trunk extending to both main pulmonary arteries. Echocardiography revealed an ejection fraction of 60% and a pulmonary artery systolic pressure of 35 mmHg, which is considered normal. Pulmonary function testing was normal. Dobutamine cardiac echocardiography was also unremarkable. There was no physiological explanation for her shortness of breath, and her cement embolism is just an incidental finding. Moreover, her shortness of breath was mainly after exertion and responds very well to nitroglycerine. Because there is no interval change in the patient's pulmonary or cardiac function tests, the respirology and cardiology consultants recommended close monitoring for any signs and symptoms suggestive of worsening embolism. Serial cardiac and pulmonary assessments will be carried out looking for increased pulmonary artery pressure as an indicator for the removal of the cement embolus with the aid of an interventional radiologist.
A 63-year-old man presented heel pain in the plantar face of the left foot. The obese but otherwise healthy man states that he has had the pain daily for months. He worked for more than 30 years as welder wearing safety boots for 8 hours a day. During the first medical examination, he referred to the clinician an unrelenting heel pain radiated along the Achilles tendon and posterior leg muscles, severe in the morning and increasing with load. In addition, he reported occasional swelling of the ankle but denied numbness or tingling. The patient indicated great difficulties to perform daily life activities (as walking, climbing stairs and getting up from chairs) due to the pain and felt worried about the stability of his ankle. On physical examination, his feet and ankle have no abnormalities, edema or skin changes. Sensibility was found to be conserved for the entire lower limb and there was no strength deficit. The patient presented strong pain during heel palpation, specially corresponding to the medial insertion of plantar fascia on the calcaneus bone. When he was asked to walk on his heel, the pain was too strong to be tolerated. Moreover, he reported a feeling of stiffness and articular limitation of the hip and knee during lower limb active mobilization. Both legs had no pulse or reflex impairment. Finally, the patient received a diagnosis of plantar fasciitis. The patient was addressed to a conservative treatment by using ESWT and a 3-session program was assessed. The clinical condition was investigated by using Foot and Ankle Outcome Scale (FAOS) and Foot Functional Index (FFI). FAOS is a self-administered survey instrument to assess the disability of the foot and ankle and associated pathologies based on 5 subsections (symptoms and stiffness, pain, daily living, sports and quality of life). FFI consists of 23 self-reported items divided into 3 subcategories based on patient values: pain, disability and activity limitation. The patient has to score each question on a scale from zero (no pain or difficulty) to 10 (worst pain imaginable or so difficult it requires help), that best describes their foot over the past week. The 2 scales were filled out before each session and 1 month follow up was scheduled: T0 was defined as the first session, T1 as the second session, T2 as the third session and T3 as the interview in the 1-month follow up. For each treatment, 3 or 4 point of stiffness and tenderness in the impaired lower limb and in the pelvis were chosen and 1500 shocks (Piezowave 2; 5 Hz, 0.167 mJ/mm2) for each one were given. This protocol has been chosen upon the observation that this amount of energy is needed to lose the densification in the fascial point. Moreover, very rare adverse reactions have been reported in the literature and the safety of the treatment has been widely demonstrated. The selection of points was based on Fascial Manipulation’s principles by Stecco that describes 3 different planes of treatment of the fascial system of the limbs and pelvis; each one is composed by 2 antagonist sequences of fascial points (center of coordination, CC) that may be found impaired or densified and can create tension. For each treatment, one among the three planes has been chosen. This decision was based on: the sensations of stiffness and articular limitation reported by the patient himself; the physical examination of the active and passive articular movements; the research in every segment of the pelvis and lower limbs, through the palpation with the fingertip, the knuckle or the elbow, of the most densified (and, usually, the most painful) points of the fascia’s sequences. During the three sessions, the following points of the left lower limb were selected: in T0 extra-pelvis, intra-genu, extra-pes, intra-pes; in T1 extra-pelvis, extra-talus, intra-talus; in T2 retro-coxa, retro-latero-genu, retro-talus (according with Stecco’s nomenclature). shows the points selected for this first report. In T0, FAOS score of 33 and FFI score of 85% were observed. Immediately after the application of SW, the patient referred a significant reduction of the hip and knee sensation of stiffness and articular limitation. In T1, an improvement in FAOS (77 vs 33) and FFI (53% vs 85%) score was found and the previous sensations of stiffness and limitation in the knee and hip were not reported anymore. As regard the last session (T2), a FAOS score of 76 and FFI score of 14% were registered and knee and hip mobilization was observed to be similar to the previous sessions. In T3, improvement of symptoms was found to be confirmed (FAOS score=79; FFI=6%). Subscales results are listed in and .
A 74-year-old female patient visited Inje University Haeundae Paik Hospital with a chief complaint of a pinkish mass on her right cheek of 3 months’ duration. A history of the presenting illness revealed that the growth was gradual in onset, and mass was initially small but grew to the present size. The growth measured approximately 0.3 cm×0.3 cm in diameter and was soft to firm in consistency (). The skin lesion was painless and asymptomatic, except for the slight discomfort of the patient due to the growth. There was no evidence of pus discharge from the lesion. Intraoral examination showed no other mass lesions, fistula, or gingivitis. No relevant medical history was obtained from the patient and her family.\nThe skin lesion was completely excised under local anesthesia and sent for histopathological examination. The subsequent skin defect was covered by bilateral V-Y advancement flaps. shows the immediate postoperative appearance, which confirms that the skin defect was covered without any tension. Histopathologically, hematoxylin and eosin staining confirmed the diagnosis of PG (). The healing period was uneventful, but 2 months after surgery, the patient returned to our clinic with persistent discharge from the previous wound site. A second surgery was performed to excise the recurrent skin lesion. A unilateral V-Y advancement flap was used for the coverage of the skin defect (). Similar to the initial surgery, there was no problem with wound healing. However, 1 month later, she returned to our clinic with a complaint of persistent discharge. A wound culture test was performed but no bacteria were identified. For histopathological examination, a tissue biopsy was performed and the results revealed chronic inflammation and abscess formation.\nIn order to identify the cause of the recurrent PG, intraoral examination was repeated. There were no findings suggestive of odontogenic infection. Subsequently, panoramic X-ray and computed tomography (CT) were performed. The panoramic radiograph revealed partial alveolar bone loss due to pulpitis and the presence of a periapical abscess of the right maxillary canine tooth (). However, there were no findings suggestive of chronic osteomyelitis, such as soft tissue swelling, mucosal abscess, cutaneous abscess, and draining fistulas, on the CT images (). For evaluation of the odontogenic infection, the patient was referred to the dental department. During dental evaluation, irreversible pulpitis was observed on the right maxillary canine tooth. Therefore, the tooth was extracted and systemic antimicrobial therapy was started to suppress the odontogenic infection. The patient visited our clinic 2 weeks after the extraction, and no secretions from the cheek flap site were found and wound healing was complete. After 3 months, all odontogenic infections had resolved and no further recurrence was observed (). The extracted tooth was replaced with a dental implant.
A 39-year-old woman with renal failure secondary to a congenital kidney malformation had been on renal haemodialysis treatment 3 days every week for the previous 17 years. She presented to the emergency eye clinic with a sudden onset bilateral reduction of vision occurring soon after waking in the morning. One day previously she had noticed visual obscurations in her left eye also after waking in the morning, which she described as temporary episodes of blurred vision lasting ∼2 min. She had attended a session for haemodialysis the day prior to when these visual symptoms started. She denied any headache. On examination her Snellen visual acuities were found to be 6/5 in the right eye and 6/9.5 in her left eye. Fundoscopy revealed bilateral swollen discs, more so in the left eye than the right (Figure ). Visual field testing showed an inferior altitudinal defect in both eyes (Figure ). An MRI scan of the brain was performed to rule out any space-occupying lesion or visual pathway pathology and was found to be normal.\nShe had also recently been diagnosed with episodes of hypotension associated with dizziness in the mornings. Two weeks prior, she was found to be anaemic with a haemoglobin of 7.5 mmol/L and subsequently received a blood transfusion of 1 unit. The patient was managed conservatively and monitored for the first few weeks and her anaemia improved to 9.5 mmol/L.\nOne month following her initial presentation she was admitted to hospital with a post-operative foot infection and was treated with intravenous clindamycin. Following this, her vision appeared to progressively decline and was measured at hand movements in her right eye and counting fingers in the left. On this occasion, the right optic disc was found to be swollen, consistent with NAAION. Her usual blood pressure had been measured at 110–120/80 and on this occasion had been found to be lower than her norm at 90/60 mmHg. She was placed on erythropoietin and Midodrine therapy to control her anaemia and hypotension. Her subsequent BP had improved and was measured in clinic at 101/61. Approximately 6 weeks later, her visual acuity improved in both eyes to 6/9 in both eyes; however, her visual field defect remained unchanged.
A 52-year-old man was transferred to our emergency room with the loss of consciousness and history of a first episode of seizure, 2 h earlier. He had a history of headache during the past several days. He had no previous history of seizure or neurologic signs. In the brief evaluation, his Glasgow Comma Scale was 11, without a focal neurologic deficit. The vital signs were within normal ranges. Emergent computed tomographic (CT) scan revealed SAH in the basal cisterns and both sylvian fissures and anterior interhemispheric region []. Dilated lateral ventricle and interstitial edema due to acute hydrocephalus were demonstrated. The patient was transferred to the operation room, and external ventricular drainage was placed in the right frontal horn of the lateral ventricle urgently. The patient was admitted to the Intensive Care Unit. Brain CT angiography and digital subtraction angiography of both internal carotid arteries (ICAs) and both vertebral arteries demonstrated a ruptured aneurysm of the left MCA and an unruptured fusiform aneurysm of right posterior communicating artery and an aneurysm at the site of the distal azygos anterior cerebral artery (ACA) []. Furthermore, another vascular variant reported in our patient was the azygos ACA in the A2 segment.\nThe patient underwent surgery, with the left pterional approach. A left frontotemporal craniotomy was performed, and an aneurysm was exposed through the sylvian fissure. The saccular aneurysm, with superior projection in the bifurcation of the left MCA, was clipped with a 7 mm straight clip. After dissection of the aneurysm, fenestration of the MCA was exposed immediately at the aneurysm's site []. The postoperative course was complicated with bacterial meningitis that was treated with antibiotic therapy. After 26 days, the patient was discharged without neurologic deficit.\nFollow-up angiography performed 3 months postoperatively showed complete obstruction of the previous left MCA aneurysms. The second surgery was done 6 months after first surgery. The neurosurgeons opted for a right interhemispheric approach and a saccular aneurysm, with superolateral projection beneath the cingulate gyrus and 15 mm before bifurcation of the pericallosal and callosomarginal arteries was clipped with a 6 mm mini-clip [].\nMonoparesis of left hand occurred as a complication of the second surgery. Patient muscle force in its left hand was 4/5, with mild spasticity at 4 months follow-up after surgery.
A 50-year-old woman presented to plastic and reconstructive surgery department with a chief complaint of right inguinal swelling and pain. Autologous bone grafts had been harvested on two previous occasions from the right anterior iliac crest for use in the reconstruction of multiple facial fractures incurred during a traffic accident 29 years ago. Two months before presentation, the patient accidentally sat on a piece of metal with her right buttock and developed pain in the right pubic region, and a mass appeared in the inguinal region. The pain continued to worsen while the mass grew, leading her to consult the surgery department at our institution.\nA contrast-enhanced computed tomography (CT) scan taken upon presentation at the surgery department suggested active hemorrhage in the right gluteal region, and emergency angiography was performed. Active hemorrhage was confirmed at the anastomosis between the right superior gluteal artery branch and the right medial circumflex femoral artery branch. Successful hemostasis was achieved with two attempts at transcatheter arterial embolization (TAE). Despite this treatment, the inguinal mass continued to grow, and the patient was referred to our department.\nA physical examination upon the patient’s initial presentation at our department revealed a large, tense mass (30×12 cm) in the right inguinal region (). The only symptom was mild tenderness. No apparent symptoms were noted in the right gluteal area, and palpation revealed no abnormalities. The vital signs and blood laboratory test data were also normal. A CT scan revealed a full-thickness bone defect in the right anterior iliac crest (). A mass was noted in the right gluteus minimus (), while a multilocular cystic mass extended from the right iliac crest defect to the right inguinal region (). Magnetic resonance imaging (MRI) showed that the gluteal lesions were septated, with low signal intensity on T1-weighted images (), while T2-weighted images and short TI inversion recovery (STIR) images were of iso-signal to high signal intensity ( and ). The inside of the lesions showed high signal intensity in the T1-weighted and STIR images ( and ), and low signal intensity in the T2-weighted images, suggesting hemorrhagic components ().\nThese imaging findings led us to suspect that the gluteal mass was a VM. The inguinal cystic lesions were suspected to be the result of inflow from the gluteal mass. With localized treatment comprising paracentesis and compression therapy, there was a high risk of recurrence. Since the patient had a history of TAE, we considered surgical resection to be a viable option, and decided to excise the inguinal and gluteal masses simultaneously. Both the inguinal mass and gluteal mass were removed under general anesthesia. A skin incision made immediately below the inguinal mass revealed a multilocular cystic mass on the inguinal ligament that extended from the iliac bone defect to the gluteal region (). This mass entered the gluteal region and formed another mass inside the gluteus minimus muscle. The border separating the gluteal mass from the surrounding tissue was distinct, and it was possible to excise it together with the inguinal lesion. Upon rupture of its membrane, the inguinal lesion produced a dark brown serous exudate ().\nHistopathological analysis revealed that the inguinal mass had no capsular epithelium, and numerous histiocytes and inflammatory cells covered the surface. Fibrin had adhered to the lumen. The gluteal mass was badly damaged, which we thought to be a result of the presurgical TAE, so it was difficult to determine the overall characteristics of the tumor. Most of it was made up of organized cysts and hematoma, but it also included an area of heterogeneously distributed, irregularly dilated, venous vasculature. Immunostaining of the vascular endothelium was positive for CD34 and negative for D2-40 (). These results were compatible with a VM. Six months after surgery, the patient had recovered with no signs of recurrence ().
A 32 year old man presented to an outside hospital with a one month history of left foot pain. He recalled that the pain started after he had twisted his ankle during a round of golf. Since that time, the pain had persisted and was most severe along the lateral aspect of his left foot. On physical examination, there was moderate swelling over the lateral aspect of the hindfoot, with point tenderness directly over the cuboid bone. The remainder of the foot exam was normal. A plain radiograph of the left foot showed a large oval, cystic mass within the cuboid bone that had relatively well-defined margins but with obfuscation of the lateral cortex (figure ). There was no significant periosteal reaction, and no soft tissue mass or calcifications were appreciated. A computed tomography (CT) scan showed an expansile lytic lesion, which was contained by an extremely thin shell of reactive bone (figure ). The margins of the lesion were circumscribed, but not sclerotic, and there were no internal foci of mineralization. There was also no evidence of ankle effusion.\nMagnetic resonance imaging (MRI) demonstrated a gadolinium-enhancing lesion, with increased signal intensity on the T2 weighted image (figure ). The lesion demonstrated slight heterogeneous enhancement, and there was no associated pathologic fracture or soft tissue mass identified. There was, however, extensive edema in the adjacent soft tissues. A two-phase technetium-99m-methylene diphosphonate (Tc99 M MDP) was obtained and demonstrated marked uptake corresponding to the left cuboid lesion, with no other foci of significant uptake (figure ). Based on the clinical and radiographic findings, the lesion was thought to represent a benign, but potentially locally aggressive neoplasm such as giant cell tumor of bone.\nThe patient's pain became progressively more severe and he no longer was able to weight bear. An open biopsy was performed and a frozen section revealed a giant cell rich lesion with atypical mononuclear stromal cells and areas of extracellular eosinophilic matrix. The differential diagnosis was an atypical giant cell tumor versus a variant of osteosarcoma. A discussion regarding immediate management concluded that it would be best to thoroughly curette the lesion and pack the defect with cement, which would stabilize the lateral column and be adequate therapy for a benign lesion. If, however, the histologic analysis of the additional tissue demonstrated an osteosarcoma, then an ampution would be performed at a later date and the curettage would not have compromised treatment for this type of tumor.\nEvaluation of the entire tissue specimen revealed a neoplasm that varied in morphology. The majority of the tumor was solid and in regions consisted of randomly interconnecting trabeculae of woven bone rimmed prominently by neoplastic osteoblasts. The plump osteoblasts had abundant eosinophilic cytoplasm and round or oval nuclei with fine chromatin. The intertrabecular spaces were filled with loose vascular connective tissue containing congested capillaries, extravasated red blood cells, and scattered osteoclast-type giant cells. The morphologic features were reminiscent of osteoblastoma (figure ). In other areas, however, the neoplastic osteoblasts grew in solid aggregates, were cytologically malignant with nuclear enlargement, coarse chromatin, and hyperchromasia, demonstrated many mitoses and produced coarse lace-like neoplastic bone typical of a high grade osteosarcoma (figure ). Based on the histologic features the tumor was diagnosed as a high grade osteoblastoma-like variant of osteosarcoma.\nA staging computed tomography (CT) scan of the chest showed no evidence of metastatic disease. The patient was started on an appropriate chemotherapy regimen and subsequently underwent a below the knee amputation (BKA). Dissection of the cuboid revealed residual small foci of osteosarcoma in the bone neighbouring the cement plug, as well as in the soft tissues immediately adjacent to the bone. No vascular invasion was identified and all margins of resection were widely negative.
A 16-year-old Bangladeshi girl presented to our hospital with hoarseness of voice which had been gradually worsening over the past 4 months. She was treated earlier by the local physician with antibiotics. On evaluation with a fiberoptic endoscopy, she was found to have a polyp in the anterior 2/3 of the right vocal cord with impaired right vocal cord mobility. She underwent excision of the polyp, and subsequent histopathology showed well-differentiated squamous cell carcinoma. She was then referred to the oncology clinic for further management.\nShe had no history of using tobacco or consuming alcohol. She had a history of exposure to passive smoke. She did not have a past history of any laryngeal surgery or exposure to radiation/carcinogenic agents; there was no family history of malignancy either. A computed tomography scan of the neck showed an irregularity of the right vocal cord, and no neck nodes were seen. Her biopsy slides were reviewed, and the diagnosis of carcinoma was confirmed (fig. ). Her disease was staged as T2N0M0 well-differentiated squamous cell carcinoma of the larynx.\nShe was planned for radical radiotherapy. The treatment fields were as per the standard guidelines for a T2 vocal cord. The superior border was 1 cm above the thyroid cartilage, the inferior border at the lower edge of cricoid cartilage, anteriorly in air, and the posterior border was kept at the anterior border of vertebral bodies (fig. ). Neck nodes were not prophylactically treated as the incidence of nodal positivity is <2% in T2 lesions. The organs at risk that were taken into consideration were the parotids and spinal cord. The dose received by breasts and thyroid glands was also estimated for documentation purposes. She did not receive any concurrent chemotherapy in view of early disease. She was then treated with radical radiotherapy (66 Gy in 33 fractions, 2 Gy per fraction, 5 days a week over 6 1/2 weeks) using 6 MV photons in a linear accelerator. During her treatment, lead apron was used to cover the thorax to prevent scatter dose radiation. She tolerated the radiotherapy well and is on a regular follow-up. She had no disease recurrence on her last follow-up.
A 46-year-old female presented in 2017 with symptomatic right leg pain and aesthetic complaints relating to the right calf. Medical history showed a previous head trauma (car accident) with brain hematoma drainage and a saphenous vein operation. Physical examination revealed edema in the perimalleolar area and painful varicose veins, in the right calf (with considerable aesthetic impact) and left calf (with minor aesthetic impact). Venous scores at the first visit to our clinic were the following: Venous clinical severity score VCSS 10 and Aberdeen quality of life questionnaire 27.7.\nDuplex examination conducted before the original venous operation (which had been performed in a different clinic in January 2016) had shown reflux in the left great saphenous vein and significant reflux in the right small saphenous vein. However, the operation actually performed was stripping of the right great saphenous vein. Both the left great saphenous vein and the right small saphenous veins were left in place untreated. After this procedure, symptoms had exacerbated progressively, and the aesthetics of the leg had deteriorated progressively.\nPreoperative evaluation was normal. We performed a complete duplex scan, according to our routine, as published elsewhere. The patient had type 1b+2a shunt in the right leg and 4+2d shunt in the left leg. We suggested operating to treat the small saphenous vein in the right leg and the great saphenous vein in the left leg. We treated the patient using the CHIVA technique to preserve the remaining saphenous veins.\nWe performed the CHIVA procedure on both legs during the same operation. Local anesthesia was provided with a solution containing 10 mg/mL 20 mL of ropivacaine and 2% lidocaine, using 20 mL and 60 mL of saline. We routinely have an anesthetist in the operating room to guarantee patient safety and comfort, who is always advised to avoid sedation as much as possible. When necessary, an opioid-free sedation technique is employed. In the right leg, we ligated the small saphenous vein at its junction with a calf vein and ligated two N3 collaterals, leaving the small saphenous vein draining through two perforators. In the left leg, we ligated a collateral draining to the great saphenous vein from the inguinal ligament and an N3 draining reflux from the great saphenous vein to the calf. A total of 5 small incisions were made. The patient was discharged two hours after the operation wearing compressive stockings and taking 40 mg enoxaparin per day for 3 days, according to our postoperative routine.\nOn the sixth postoperative day, duplex scanning was performed, showing minor continuous reflux in the small saphenous vein of the right leg and even less reflux in the great saphenous vein on the left. The right small saphenous vein had been 7.4 mm before the operation and was 3.8 mm after. The left great saphenous vein had been 4 mm before the operation and had not decreased in size during the initial postoperative period. The patient scored pain at 3 on a 0-10 pain scale and had taken one 750 mg paracetamol tablet during the entire postoperative period. We made a full photographic record before and after the operation ( \n ). There were no photographs or records of symptoms available from the original operation.\nIn relation to the wrong-site surgery, we comforted the patient and reported the case to both the previous surgeon and the patient safety surveillance team at the hospital where the operation had been performed.
An 83-year-old man underwent surgical excision of a malignant peripheral nerve sheath tumor (MPNST) at the right knee at another hospital and was then referred to our hospital for further chemotherapy. Three months after the start of chemotherapy, the patient complained of severe pelvic and low back pain. A PET CT was performed which showed multiple bony lesions with a variable (moderate to high) degree of metabolic activity raising suspicion that these were metastases. The lesions, however, had a very unusual appearance and evolution as seen on CT.\nInitially, the lesions presented as small rounded lytic lesions with a well-defined sclerotic margin (Figure ). They exhibited a rapid growth but the initial appearance of central lysis and surrounding sclerotic margin was preserved. Follow up PET CT scan was performed after seven weeks to evaluate the response to chemotherapy. It revealed a second concentric band (halo) around some of the lesions. This band (halo) had a ground-glass appearance and was surrounded by a second sclerotic rim. This rim was thinner and less sharply demarcated than the more central rim (Figure ). The multiple lesions were in different phases of evolution and as such had a different appearance at any given time.\nPresence of multiple lesions, a known primary tumor and moderate to high uptake of FDG tracer on PET scan (Figure ) suggested metastatic disease, despite the unusual appearance of the lesions.\nOther bony metastases were also evident on CT. Some were entirely sclerotic and a few had an ill-defined lytic appearance. Collapse of a vertebral body and fracture of the upper endplate of another vertebra due to metastases were also present.\nBone biopsy of a lesion with the above described doughnut appearance located in the left pubic bone was performed. A biopsy was obtained in the central lytic area and another biopsy in the peripheral halo (Figure ). Histological evaluation of both the samples showed infiltration of bone marrow by malignant spindle-shaped cells arranged neatly in bundles. Histological and immunohistochemical studies were compatible with metastases from a spindle cell tumor (in this case MPNST).
A 51-year-old woman was referred to the ENT department with a 2-week history of a lump on the right side of her neck. There was no history of change to her voice or dysphagia.\nShe is a para 4 with all normal vaginal deliveries and has had normal cervical smears in the past. Her periods were regular and she gave no history of intermenstrual or post-coital bleeding. She smoked about 20–30 cigarettes per day.\nOn further questioning in the clinic, she gave a history of increasing lethargy for the past 3 months and was also unable to report to work due to severe back pain.\nFive years before the present episode, she reported feeling unwell with significant weight loss and heavy periods. She was found to be anaemic and was given five units of blood. She was investigated for a possible colon cancer which proved to be negative. She was referred to a Menstrual Disorder Clinic but failed to attend the clinic twice.\nOn examination, multiple cervical lymph nodes were palpable on both sides of the neck. Ultrasound scan of the neck revealed two large supraclavicular lymph nodes with several abnormal looking lymph nodes in the right carotid chain.\nAn X-ray of the chest showed no abnormality. Fine needle aspiration of the lymph nodes yielded squamous carcinoma cells.\nMetastatic squamous cell carcinoma of an unknown primary tumour was suspected and investigations were performed to find a possible primary site. Clinical examination and endoscopy of the upper digestive tract did not yield an obvious primary tumour in the nasopharynx, larynx and hypopharynx.\nComputerised Tomography (CT) of the neck, chest and abdomen revealed marked mediastinal and para-aortic lymphadenopathy suggestive of spread of the known squamous cell carcinoma. There was evidence of dilatation of the collecting system bilaterally with dilatation of the proximal ureters suggesting an obstruction within the pelvis.\nA Positron Emission Tomography-CT (PET-CT) scan was performed which showed markedly increased uptake in the right cervical lymph nodes, as well as in the right paratracheal, anterior mediastinal, lower para-aortic, and bilateral iliac lymph nodes with an obturator node showing a photopaenic centre. In addition, there was a focal area of increased uptake in the pelvis, suggesting a lesion within the rectal wall or in the vaginal vault (Figures and ).\nGiven the histology of squamous carcinoma, the PET scan suggested that the uptake in the pelvis may represent a primary gynaecological problem rather than a second malignancy in the rectum. But given the distribution of the disease which was very unusual for cervical carcinoma, a review of the histology was suggested with a differential diagnosis of lymphoma to be considered. The histology from fine needle aspiration of the cervical lymph node confirmed it to be carcinoma cells of squamous origin.\nOur patient was then referred to the gynae-oncology team. On examination, the uterus was anteverted, mobile and bulky corresponding to about 14 weeks' size with no palpable adnexal masses. Her cervix appeared normal to the naked eye and a smear was obtained which was reported as normal.\nMagnetic Resonance Imaging (MRI) of the pelvis and abdomen was performed which revealed a highly abnormal cervix, diffusely infiltrated by an intermediate to high T2 signal intensity mass measuring approximately 3 × 4 × 3.5 cm. The mass involved the endocervical canal and the stroma with suspected early parametrial invasion anteriorly. There was no convincing evidence to suggest bladder involvement and the rectum was clear of disease. Several small intramural fibroids were demonstrated within the myometrium as well as a submucosal fibroid in the anterior body of the uterus (Figure ).\nThere was extensive lymphadenopathy along both pelvic side walls, common iliac regions and the para-aortic regions but with no evidence of inguinal lymphadenopathy. Bilateral hydronephrosis was noted. No bony deposit was seen. In conclusion, the MRI reported that the appearance was consistent with a cervical carcinoma with extensive lymphadenopathy and hydronephrosis, stage FIGO 3b.\nRoutine blood investigations before examination under anaesthesia showed her to be anaemic with a haemoglobin level of 6 g/dl. She was transfused with four units of blood. Her liver function tests and renal function tests were normal and serology showed her to be negative for HIV.\nShe had an examination under anaesthesia, cervical biopsy and an endocervical and endometrial curettage. Examination under anaesthesia showed the cervix to be bulky with an intact surface epithelium. There was no parametrial involvement and the rectum and bladder were free. Hysteroscopy revealed a pedunculated fibroid on the anterior wall of the uterus. Large biopsies of the anterior and posterior lip of the cervix were taken which identified a poorly differentiated squamous cell carcinoma of the anterior lip of the cervix. The endocervical curettings were positive for squamous cell carcinoma and the endometrial curettings showed proliferative phase endometrium.\nWith an impression of metastatic squamous cell carcinoma of the cervix, she was started on palliative chemotherapy with carboplatin and paclitaxel. She has responded well to the therapy with a reported decrease in the size of the neck nodes.
We report the case of a 61-year-old woman who was suffering from lower abdominal bulge formation, chronic constipation, as well as a feeling of permanent abdominal constriction and pain. These symptoms appeared eight months after bilateral breast reconstruction, which was performed following subcutaneous mastectomy that was necessary owing to ductal carcinoma in situ. The breast reconstruction was conducted using a non-muscle-sparing pedicled TRAM-flap transposition. The defect created at the donor site within the abdominal wall after harvesting the rectus muscle was closed using a continuous suture with resorbable suture material. An additional augmentation was performed by the implantation of a resorbable polyglactin mesh placed on the fascial suture.\nThe patient presented at the authors' outpatient clinic eight months after reconstruction. At that time her body mass index was 18.9 and she was suffering from a lower abdominal bulge formation (Figure ). An ultrasound examination revealed an abdominal wall defect measuring 18 × 20 cm, with no detectable rectus abdominis muscle remaining, resembling a large rectus diastasis. A preoperative endoscopy of the colon showed signs of adhesions in the colon sigmoideum and transversum, but no other pathologies; the laboratory values were normal. Apart from an appendectomy performed 20 years ago, the patient had undergone no other previous abdominal surgery. In addition to the annoying large bulge in this otherwise slim patient, the pain experienced during everyday movement and impairment of bowel function led to an explorative laparotomy and an attempt to reconstruct the abdominal wall.\nFollowing adequate preparations with intestinal irrigation, a re-incision through the midline scar was performed. On entering the peritoneal cavity, several dense adhesions of small intestine to the abdominal wall and interenteric to the colon were found. These were carefully dissolved without causing injury to the intestine. Further exploration revealed a near-total absence of both abdominal rectus muscles; residual muscle fibres could be detected only at the lateral side of the rectus sheath. The initially implanted absorbable mesh was not identified, and the ultrasonographic finding of a diastasis-like defect with lateralization of both lineae semilunares was verified. Following a wide-ranging mobilization of the epifascial subcutaneous tissue, the remaining parts of the anterior rectus sheaths and minimal lateral parts of the rectus muscles were exposed. The herniation sac was partly resected, leaving sufficient material to facilitate a peritoneal closure of the abdominal cavity. In order to reach an adaptation of both lateralized anterior rectus sheaths, a component separation of the abdominal wall (Ramirez procedure) was performed. In the absence of an intact rectus abdominis muscle and anterior rectus sheath, only a vertical incision lateral to the linea semilunaris and separation in the plane between oblique external and internal muscle was used. A two-layer closure of the fascia in the midline was performed using a non-resorbable single-stitch suture of the posterior wall, and a continuous suture with a slowly resorbable suture material for the remaining anterior rectus sheath. The lateral defects between the external oblique muscle and linea semilunaris were covered with a halfmoon-shaped lightweight polypropylene mesh (Ultrapro®; Ethicon, Norderstedt, Germany) on each side (Figure ). Punctual mesh fixation was achieved using resorbable 3/0 single-stitch sutures (Dexon®; Braun, Germany). A subcutaneous suction drain was placed on top of each mesh, after which wound closure was achieved with a continuous intracutaneous suture using non-resorbable material.\nThe patient's recovery was uneventful; during her hospital stay she wore an elastic abdominal belt and was provided with analgesics and physical therapy with intense respiratory training. The suction drains and suture material were removed on schedule, the postoperative ultrasonography was without pathological findings and minimal postoperative seroma resolved. The patient was discharged from hospital and made subsequent visits to the outpatient clinic. At 12 months after surgery she remained satisfied with the outcome.