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Our patient, a 16-year-old girl of Middle East origin, arrived in Sweden with her family as a refugee in 2016. The family had lived under traumatic circumstances and had been forced to flee due to the political situation in their country of origin. On arrival in Sweden, our patient was under treatment with lithium, levothyroxine, and haloperidol, and was enrolled in our out-patient care unit for pediatric bipolar and psychotic disorders. Her parents showed a medical document saying she was diagnosed with BD and OCD by a psychiatrist in a major city in the Middle East. However, the parents and our patient had difficulties in accounting for her psychiatric history and showed little knowledge about the disorders and why she was taking the prescribed medicines.\nShe had no family history of psychiatric diseases. Her psychomotor development in childhood was reported normal. She had been physically active, successfully participated in martial arts, and was described as helpful, responsible, and having friends, but with no academic schooling. The first psychiatric symptom observed in our patient, according to her parents, was an episode of altered mood at 14 years of age. During this episode she was unable to get up from bed for 1 month, stopped eating and drinking, and needed help to visit the bathroom. She was prescribed psychopharmacological treatment and recovered after approximately 3 more weeks. This episode was followed by a more active episode with disruptive behavior, including self-harm by cutting her ankles. There were no reports of suicide attempts.\nDuring her first months in Sweden, our patient’s main psychiatric problems were irritability, aggressiveness toward herself and her sister, mood shifts, and sleep difficulties. Her mood stabilized somewhat after an increased dose of lithium, even though she was under pressure from the process of seeking asylum and waiting for decisions from the authorities. She had great difficulties in recalling any previous episodes of mania or depression, but could describe periods of obsessive thoughts, mainly of sexual and religious character, since childhood. She felt shameful about these thoughts and about previous reckless behavior.\nAdditional treatment with quetiapine was initiated at a low dose of 25 mg daily. After a conflict with her parents, our patient stopped taking her medications, including lithium, for a few days. Shortly after this episode of altered compliance, she was exposed to several additional psychosocial stress factors. These included interviews at the migration office and deportation of an elder brother and his 4-year-old son, at the same time as she started school. Our patient presented severe anxiety, disturbing sinful thoughts, and depressive and suicidal ideations. She was subsequently admitted for emergency care at the Department of Child & Adolescent Psychiatry in Malmö, 7 months after arriving in Sweden.\nDuring the intake interview, our patient expressed a strong sense of guilt and distress because of her obsessive thoughts, with fear that she might harm herself or others, show inappropriate sexual behavior, or offend religious objects and thereby dishonor her religion. At the ward, compulsive behavior was noted, including excessive hand washing, showering procedures, and a need to line up different items on her bed with great accuracy. She did not wish to continue treatment with lithium, in part because she experienced adverse effects such as tremor and because of the need for repeated blood sampling. Her serum lithium level was 1.3 mmol/L (0.5 to 1.2 mmol/L) 2 days after admission. Our patient’s negative attitude, possible side effects, and the clinical picture dominated by depressive mood and OCD symptomatology, led to the decision to phase out lithium. Over the following 2 weeks, lithium was removed from her treatment and simultaneously quetiapine was up-titrated to 600 mg daily.\nOur patient underwent magnetic resonance imaging (MRI) of her brain that showed no abnormalities. Routine laboratory tests indicated mild under-treatment of her hypothyroidism and there were no suspicions of illicit drug use. The Children’s Yale-Brown Obsessive Compulsive Scale (CY-BOCS) showed a total score of 18 points, indicating moderate OCD severity.\nDuring the third and fourth week of in-patient care, our patient experienced a certain degree of mood stabilization and less anxiety. However, during the fifth week, her mood switched to a manic state with mixed and psychotic features. Further care was given according to the Swedish Compulsory Mental Care Act. Our patient showed a complex symptomatology, with rapid switches from euphoria with elevated energy to episodes of dysphoria with depressed mood, feelings of worthlessness, and fatigue. Overlapping manic and depressive symptoms such as psychomotor agitation, irritability, and distractibility were present, as well as paranoid delusions such as fear of being poisoned. Her pattern of sleep was severely impaired with a decreased need for sleep. In her room, she tore away some of the base-boards, on one occasion using them as a weapon against the staff. At times she was also aggressive toward fellow patients, leading to recurrent periods of seclusion. At other times she planned a wedding to take place between herself and members of the staff. Occasionally, she sang and laughed inappropriately or excessively, at other moments she kicked and punched the walls. For a few days, she refused oral medication. In this severe state, forced injections with haloperidol, zuclopenthixol acetate, and diazepam respectively were needed on various occasions to prevent self-harm and injury to others.\nDespite reinstatement of lithium and further elevation of quetiapine to 800 mg daily, our patient did not improve during the following 2 weeks. Blood samples showed serum lithium concentrations of 0.7 to 0.9 mmol/L. At the beginning of the eighth week, haloperidol, which had previously shown a stabilizing effect in our patient, was reinstated and increased to 4 mg daily, while quetiapine was successively lowered to 150 mg daily. However, our patient’s behavior was continuously labile, with episodes of hyperactivity, agitation, and delusions.\nClinical discussions ensued regarding further psychotropic drug trials versus ECT; ECT was initiated with a first administration during the ninth week of hospitalization. ECT was administered every second or third day for 4 weeks with a total of 11 treatments. Unilateral ECT according to d’Élia [] was used with a dose of 96.0 to 156.8 millicoulombs (mC) and a pulse-width of 0.3 to 0.35 milliseconds (ms). The average seizure length was 23 seconds (range 16 to 37 seconds). Propofol was used as an anesthetic agent in ten sessions and thiopental was used in one session. Our patient showed a prompt and marked positive response to treatment, with a dramatic decrease in her severity of illness, as described by using the Ziegler Young Mania Rating Scale (YMRS) and Clinical Global Impression (CGI) scores (Fig. ). She experienced no evident negative side effects. Fourteen weeks after admission, our patient could be discharged to stay with her family. Her base medication at discharge was lithium, with a serum concentration of 0.7 mmol/L, haloperidol 4 mg daily, and quetiapine 150 mg daily, with a plan of further tapering of antipsychotics.\nAfter discharge, our patient was reviewed by our specialized out-patient care unit and monitored by home visits twice a week. The lithium concentration was controlled once a week and kept at 0.9 to 1.1 mmol/L. Psychosocial interventions including psychoeducation about BD and OCD were conducted. Psychotic and OCD symptoms were meliorated and new mood episodes have not yet been observed during the current 3-month follow-up period. Our patient has restarted school and is actively taking part in some physical activities. The family is still waiting for final decisions about permanent residency in Sweden.\nAfter discharge, we established contact (by telephone and email) with our patient’s physician in the family’s previous country. We were informed that our patient had been treated for approximately 2 years in their out-patient clinic, and that her OCD symptoms most probably were related to her BD, as decreased obsessions and compulsions had been noted when her mood was normalized. Our foreign colleague emphasized that there had often been a high level of expressed emotions within the family.
A 61-year-old female presented with Gustilo-Anderson Grade 3C compound fractures of the distal femur and Shatzker five tibial plateau fracture. She underwent initial debridement and a spanning external fixator in the acute setting and subsequently definitive fixation with a retrograde femoral intramedullary nail and lateral and posteromedial proximal tibia plates, with a bipedicled gastrocnemius flap and split skin grafts inset over the large skin defect in the same setting. The healing was complicated by tibia plateau fracture and she underwent a planned bone grafting 3 months later.\nBone union was achieved at 5 months post operatively, but the knee range of motion was limited from 5 to 20 degrees as shown in . Aggressive physiotherapy was commenced but failed to improve the range of motion. A Judet quadricepsplasty and removal of implants was subsequently performed 12 months after the initial surgery and 9 months after the secondary bone grafting surgery.\nThe anaesthetic team inserted an epidural catheter for continuous epidural anaesthesia prior to surgery and was retained in situ for the first week. Intravenous Cefazolin was given 1 hour prior to initiation of surgery and continued on for 24 hours. In supine position under general anaesthesia, the leg was cleaned and draped above the level of the anterior superior iliac spine (ASIS) to the foot. A sterile tourniquet is applied and the initial skin incisions are marked out; along the lateral intermuscular septum to the lateral aspect of the patellar tendon and a medial parapatellar S-shaped incision as depicted in and .\nThe lateral incision was made first directly through the skin, fascia and muscle preserving the inter-connective tissue between the fascia and the skin to preserve dermal blood supply. The knee joint was entered via a lateral parapatellar capsular incision and the intra-articular adhesions between the femoral condyles, tibia and patella were released.\nA medial parapatellar approach was then utilized for adhesiolysis of the medial aspect of the knee. A simultaneous partial release of the medial collateral ligament (MCL) and medial capsule was performed. Under radiological guidance, the posteromedial tibial plate was removed to prevent possible impingement during flexion. At this point of time, the knee was ranged and flexion had improved to 60 degrees as shown in .\nThe tourniquet was then removed and the lateral incision was then extended to the level of the Greater Trochanter. The rectus femoris origin was released at its origin via the same lateral incision, a modification of the original Judet’s procedure.\nThe quadriceps muscle slide was then performed by elevating the quadriceps muscle off the femur along the intramuscular septum using an extra-periosteal approach as shown in . Careful cauterization and ligation of the perforating vessels was performed at this juncture to prevent significant blood loss.\nFractional lengthening of the fascia lata and anterior fascia of the thigh was then performed by making multiple transverse incisions at multiple levels. The intramedullary femoral nail was then removed. At this point, maximal knee flexion of 0 to 120 degrees was achieved as shown in . The medial and lateral incision’s subcutaneous tissue and skin were then closed over vacuum drains with the capsule intentionally left open to prevent recurrent capsular adhesion formation.\nAn epidural catheter provided analgesic relief for 5 days post-surgery before gradually being weaned off and converted to oral analgesia.\nContinuous Passive Motion (CPM) was initiated in the postoperative care unit and continued for 24 hours for the first week. Electric muscle stimulation of the quadriceps muscles, aggressive physical therapy and cycling exercises were started on the 3rd post-operative day and the patient was allowed to ambulate on the 6th day post-surgery, after the epidural catheter had been removed.\nHer drains were removed on post op day 4 and 5 when the drainage amount was minimal. Regular monitoring of her haemoglobin levels showed a drop of 1.0 g/dL on the first post-operative day requiring blood transfusion. The haemoglobin levels were subsequently maintained.\nRemoval of sutures was performed at 3 weeks where the wounds had healed well and there was no evidence of dehiscence or skin edge necrosis. At 3 months follow up, the patient was able to ambulate and had knee range of motion from 0 to 90 degrees without extension lag as seen in . This represented an increase in range of motion of 75 degrees and the patient was able to climb stairs and return to daily activities.
A 61-year-old woman was admitted to National Cancer Center with a persistent headache. Her headache started suddenly about one week before being admitted to the hospital, after a cough. Right frontal area headache was the only symptom, her vital signs were normal and the neurologic exam showed no abnormalities. She wasn't on any medications and had a past history of ovarian cancer (stage IIIc, histologic type: adenocarcinoma, mixed endometrioid/mucinous) that was diagnosed 9 years ago. She underwent an operation and multiple cycles of adjuvant chemotherapy for 2 years from diagnosis. During her follow-up, recurrence was found in the pelvic lymph nodes 2 years ago and she received radiotherapy for 3 months. Since then there was no evidence of disease and she was having regular check-ups on outpatient clinic basis.\nA computed tomography scan showed a lesion in the right frontal area with a 5 cm sized conglomerated high density lesion and a cresenteric low density lesion in the ipsilateral subdural area (). Magnetic resonance imaging revealed a lobulated, contoured, well-defined dural based mass with a central portion of iso-to-high signal intensity in T1-weighted image and low signal intensity in T2-weighted image suggesting acute- to subacute intratumoral hemorrhage (). The cresenteric subdural lesion appeared as a low signal intensity in T1-weighted image and high signal intensity in T2-weighted image which is consistent with a liquefied subdural hemorrhage. On gadolinium enhancement, the mass showed strong homogenous enhancement except for the central portion representing hemorrhage (). Our first impression from these findings was acute an intratumoral hemorrhage and subacute stage subdural hemorrhage in a dural metastatic tumor due to her past history and presentation of hemorrhages which occurs more frequently in malignant or metastatic tumors than in benign tumors []. Meningioma was also the differential diagnosis because of the tumor location and homogenous enhancement. We planned a curative resection of the tumor and adjuvant therapy was undetermined until we received pathology results.\nAn elective operation was planned and performed without any complications. Bicoronal incision was done and an enlarg-ed middle meningeal artery heading towards the tumor was made visible by opening the frontal lobectomy bone flap. Dura mater was incised and the subdural hemorrhage was evacuated (). The subdural hemorrhage was composed of mixed components ranging from a liquefied hemorrhage to a dense acute hematoma (). The surface of the brain contacting tumor was intact and not been invaded by the tumor. At the deepest portion of tumor, a capsule attached to pial surface and cortical vein was observed (). Intraoperative findings suggested that this cortical vein adhered to the tumor arachnoid capsule might be a focus of bleeding in this case. A frozen biopsy of the main mass turned out to be a meningioma but the histologic subtype was uncertain. The pathologist reported tumor involvement of the pia attached to cortical vein. Thus, the cortical vein and underlying pia mater was removed with negative margins. The subdural hemorrhage had a membranous structure and the frozen biopsy suggested tumor involvement (). Therefore, the membranes surrounding the hemorrhage were removed totally and all resection margins were tumor-free, indicating Simpson grade I operation. She experienced an immediate postoperative partial seizure which was controlled with 'add-on' anti-epileptics to preventive one. She recovered without any neurological deficit. Final pathology results revealed a conventional meningioma of fibrous type (World Health Organization grade I) with intratumoral hemorrhage revealed among the thin-walled dilated capillaries (). She was discharged without further event and is now being observed on an outpatient basis.
A 36-year-old gentleman came to our emergency department (ED) complaining of weakness over the left side of his face. He had received the second dose of the mRNA-1273 severe acute respiratory syndrome coronavirus 2 vaccine one day before the onset of his symptoms in his left deltoid muscle. His symptoms had been ongoing and progressive for the past three days. He mentioned slight difficulty in speaking and eating. He also had mild numbness and tingling of the left arm along with subjective weakness of the left upper limb. He had never experienced such symptoms earlier. There was no prior history of any medical illnesses. No preceding fever or upper respiratory symptoms were present. He denied ear pain, increased sound sensitivity, or a change in taste sensation.\nThe patient was alert and oriented at the time of examination. He was speaking in full sentences and no slurring of speech was noted. Initial vitals were a heart rate of 66 beats per minute, a temperature of 37°C, maintaining oxygen saturation of 97% on room air, and blood pressure of 120/90 mmHg. Cranial nerve examination showed isolated VII nerve palsy of lower motor neuron type. The patient was unable to close his left eye completely. There was flattening of wrinkle lines above the left eyebrow and drooping of the left corner of the mouth. There was no objective weakness in all four limbs. He had slightly decreased sensations on his left upper limb which spared the lower limbs. His ear examination was normal bilaterally with no vesicles or scabbing and no parotid swelling in front of the ears. The rest of his physical examination was unremarkable.\nA neurology consult was obtained in the ED due to his left upper limb numbness and weakness along with facial palsy to rule out brainstem stroke. CT and MRI of the brain were done which were unremarkable (Figures , ). A diagnosis of Bell’s palsy was made, and the patient was discharged home with 60 mg of daily oral prednisolone for one week, artificial tears, eye care instructions to prevent corneal injury, and neurology follow-up. On follow-up after two weeks, the patient’s arm symptoms had resolved completely and his facial symptoms had improved but still present.
A 26-year-old female presented with complaints of posterior cervical headaches for many years with on and off tingling and numbness involving right arm, trunk, and leg. There was no history of bowel or bladder dysfunction. There was no significant past medical history or preceding trauma. The general physical examination was within normal limits. Neurological examination revealed presence of subtle spastic hemiparesis on the right side (Medical Research Council grade 4+/5) with exaggerated deep tendon reflexes as compared to left. She has positive right Hoffmann's sign. Computed tomography scan of the cervical spine revealed slight kyphosis and degenerative changes within the subaxial cervical spine with an expanded hyperostotic right posterior arch of C1 (). Magnetic resonance imaging of the cervical spine showed constriction of the dural sac with presence of possible right posterior arch of C1 lesion which was hypointense on T1 and T2W images and no contrast enhancement with a small area of increased signal within the spinal cord on the T2 weighted imagesb (). The patient was referred to us with a diagnosis of a benign bony tumor of C1 and a surgical removal was performed. Tc-labeled bisphosphonate nuclear bone scan performed did not reveal any abnormal focal uptake by the lesion which has been described in osteoid osteomas of the spine. Considering the dubious nature of the lesion with equivocal diagnosis, surgical resection was planned. Posterior exposure of the cervical spine in the C1-C2 regions was performed. There was no obvious anomaly seen externally with a normal appearing ligamentum flavum. The right posterior arch of the atlas was hypertrophied with no other abnormality suggestive of neoplastic lesion. The lesion was excised with performance of right hemilaminectomy of the atlas carefully preserving the C1/C2 joint. Final histopathology of the lesion showed the presence of normal bone tissue with no evidence of tumor confirming the diagnosis of hypertrophied posterior arch of atlas. Patient had an uneventful postoperative course with CT showing good decompression and excellent clinical outcome which was maintained at 3 months follow-up.
A 30-year-old primigravida booked at 11-week gestation. She has no relevant medical or surgical history. Her pregnancy was uncomplicated until she received a diagnosis of gestational diabetes mellitus (GDM) at 29 weeks through an abnormal oral glucose tolerance test. She was commenced on insulin at 33-week gestation after failing to control her blood sugars through diet and exercise alone. Her pregnancy was further complicated with diagnosis of obstetric cholestasis at 36-week gestation and she was admitted to hospital due to persistent pruritus and reduced fetal movements. A decision was made to induce labor at 37 weeks due to worsening liver enzyme profile and further progression of her pruritus. Induction of labor was achieved with 2 mg of dinoprost per vaginum. Her initial examination revealed a cephalic presentation, with a soft posterior cervix 1 cm dilated. Spontaneous rupture of membranes occurred 5 h after vaginal prostaglandin administration. Palpable contractions commenced within 20 min of rupture of membranes and reached a peak frequency of 7 in 15 min, lasting 45–60 sec each. Vaginal examination was performed 45 min after rupture of membranes due to maternal distress. The patient was found to be 4 cm dilated, vertex -2 and was transferred to a dedicated delivery room with one to one midwifery care. Within 2 h, the patient began involuntary bearing down and was found to be fully dilated, vertex +1 and rapidly advancing. Pushing was commenced.\nIn the second stage of labor, after pushing for 16 min, a 4 cm defect of the perineum between the vaginal orifice and the rectum was noted. The vaginal orifice itself remained intact. The fetal head was visible through this perineal defect as the head crowned. A right mediolateral episiotomy was performed to prevent an anal sphincter injury; however, the patient sustained a third-degree tear. Total time in labor from contractions starting to delivery of the baby was 2 h 45 min.\nAfter examination under anesthesia, a 3b tear was diagnosed – namely, a tear that has severed the external anal sphincter. This was successfully repaired under general anesthesia with a combination of 3.0 polydioxanone monofilament synthetic absorbable suture in an overlapping technique to the anal sphincter, and a 2.0 serapid®, OLM Limited, O'Leary Medical, Dublin, Ireland polyglycolic acid braided multifilament to the perineal muscle and skin. Particular care was made in the re-anastomosis of the perineal body to the underlying muscle. The patient was given a course of broad-spectrum antibiotics according to the local protocols and discharged home well after 4 days. She had no symptoms of flatus or fecal incontinence on discharge. A review at 3 months post delivery in the hospital's specialized perineal clinic revealed no abnormality in anal sphincter tone, an anatomically normal perineum and an asymptomatic patient with no fecal incontinence. The patient was counseled that her next child should be born by elective cesarean section to prevent a recurrence of the injury. She was also counseled on the possibility of developing a rectocele prolapse later in life.
A 51 years old man with a 5 years history of mild ED and Peyronie disease presented to our attention. He reported poor response to PDE5i and a stable plaque along the lateral aspect of the penis for the previous 12 months. He did not present significant comorbidities but reported a significant alcohol consumption and smoked 20 cigarettes per day. He underwent circumcision in childhood. A penile Doppler US showed arterial deficiency (peak systolic velocity = 20 cm/s) and a 25 × 17 mm calcified plaque of the mid-shaft causing significant penile shortening and a slight lateral deviation of about 45o. The patient was counseled regarding the possibility of a combined procedure to improve his erection and restore penile length and deviation. The “sliding technique” offered to the patient previously described by Rolle et al[ consisted of a penile prosthesis implantation together with a penile elongation thanks to a double dorsal-ventral patch graft. The aim of this surgical choice was both to restore the length lost due to PD and to guarantee the rigidity necessary to engage in penetrative sexual intercourse. The patient was fully aware about the complexity and the potential risk associated with the procedure, which is quite complex when compared to prosthesis implantation alone. Considering the severe level of distress caused by his double condition, the patient choice was to have the surgery recommended.\nThe surgical technique was conducted using a combined penoscrotal and subcoronal incision followed by a complete degloving of the penile shaft. Buck's fascia was incised and the neurovascular bundle (NVB) completely mobilized from the tunica albuginea down to the origin of the suspensory ligament to maximize lengthening. In case of sliding technique, the length of the NVB represents the limiting factor in the lengthening process because it cannot be stretched excessively to avoid blood supply compromising. The urethra was then dissected off the corpora cavernosa and the penis partially disassembled (Fig. A). Two longitudinal incisions of the tunica albuginea of about 4 cm in length were then made and the edges of these 2 incisions were then joined by 2 semi-circumferential transverse incisions. The proximal transverse incision was made on the ventral side of the penis at the level of the penoscrotal junction, to allow the insertion of the cylinders and connecting tubing of the inflatable penile prosthesis, avoiding in this way the need to make a second proximal ventral corporotomy, while the distal incision was carried out on the dorsal side of the shaft. This manouvre, transecting the corporas, literally led to the sliding of the distal portion away from the proximal aspect of the shaft along the two previously performed longitudinal incisions. At this stage, the maximum elongation of the NVB indicates how much the two sections of the shaft could be slid apart, as the spongiosum of the urethra could elongate significantly more than the NVB. When the maximum tension on the NVB and the urethra was obtained, the two segments of the shaft were fixed laterally along the two longitudinal tunical incisions with resorbable sutures. The sliding of the 2 segments of the shaft led to the formation of 2 rectangular tunical defects on opposite sides of the shaft penis, which were covered with an autologus graft (Fig. B).\nThe cylinders of an inflatable penile prosthesis were then implanted through the ventral albugineal defect; the cylinders were left semi-inflated to reduce the risk of hematoma and to allow the formation of a capsule around the cylinders. An adequate straightening of the shaft was documented intraoperatively with a 3 cm lengthening of the penile shaft (Fig. C). A compressive dressing was then applied to the scrotum and to the penile shaft and left in situ for 3 days. The patient was discharged 3 days postoperatively with oral antibiotics and was instructed regarding wound care.\nTwo weeks postoperatively the patient attended the outpatient appointment complaining pain of the distal shaft associated with a dusky area along the sub-glandular suture line and purulent discharge coming out from the wound (Fig. D). The skin of the penile shaft was under clear tension and it could have been a concurrent factor implicated in the distal necrosis. At this stage the decision to surgically review the wound was made.\nThe patient was admitted to hospital for an elective review. Preoperative broad-spectrum antibiotics were given at induction and prolonged for 7 days postoperatively. The distal penile shaft appeared ischemic and a small defect of the lateral aspect of the right corpora that was elongated during the first stage was observed (Fig. E and F). Considering the high risk of worsening of the necrotic damage, the implant was explanted to improve the blood supply, the defect on the corpora was repaired and overlapped with the dartos available (Fig. G). The main limiting aspect for the successful closure of the wound was the small amount of penile skin available. For this reason a full-thickness skin graft was harvested from the lower abdomen and used to recreate a decent thickness penile skin without tension. A gentle compressive dressing of the shaft was left in situ for 7 days.\nThe following outpatient appointments arrange twice-weekly revealed a poor graft adhesion on the ventral aspect of the penile shaft and, at a later stage, a progressive necrosis of the area involving the proximal part of the penile urethra resulting in a large fistula (Fig. H and I).\nTwo months later the patient underwent to a first stage penile urethroplasty and malleable penile implant covered in 2 dacron sleeves to reinforce the corpora cavernosa extremely flimsy and partially necrotic. An adequate urethral plate was created using a buccal mucosa graft quilted onto the remaining albuginea of the corpora with interrupted sutures (Fig. J). The corporas were reconstituted using two dacron grafts surrounding the malleable rods (Fig. K). A tension-free closure in multiple layers was performed at the end of the procedure. The patient was discharged three days after surgery with a compressive dressing and an indwelling catheter, both removed 2 weeks later (Fig. L). At 3 months of follow-up no early postoperative complications have been recorded with a good health of the penile shaft and the urethral plate. The second-stage urethroplasty was scheduled 6 months after the last procedure.
A 20-year-old female patient reported to our unit complaining of pain in relation to her right back tooth region of the lower jaw. She gives a history using medication for pain relief since few days, but the pain did not relieve on medication. Extraoral examination revealed no positive findings, and intraorally both the soft tissues and hard tissues were absolutely normal. Except the third molars, the patient had all the other dentition in a very good healthy condition. The lower right third molar was unerupted, and there was a deep pocket noted distal to the right lower second molar.\nThe patient was advised Orthopantomograph (OPG) which revealed a solitary unilocular radiolucent lesion associated with an impacted third molar in relation to the right posterior region of the mandible. There was another solitary unilocular radiolucent lesion noted on the left posterior region of the mandible situated between the roots of the canine and first premolar as shown in . Pulp vitality test for 43 and 44 revealed that both the teeth were vital. Fine-needle aspiration of both the lesions was negative. Based on the history, clinical presentation, and radiological evaluation, a diagnosis of dentigerous cyst in the right angle region of the mandible and LPC in the left body of the mandible was made. Patient consent was taken and following thorough general physical evaluation, the patient was posted for surgical enucleation of both the lesions located in the mandible under Local Anesthesia (LA).\nA modified Ward's incision was given distal to 47 and following elevation of a mucoperiosteal flap, distal bone overlying the impacted tooth was removed, and the lesion was enucleated along with the impacted tooth as shown in . A crevicular incision with an anterior release was given in relation to 35–33 and following the elevation of a mucoperiosteal flap, surgical enucleation of the lesion was done as shown in . Hemostasis was achieved and wound closure was done. The specimens were sent for histopathological evaluation which revealed an OKC in the right angle of the mandible and LPC in the left body of the mandible as shown in . The patient was kept on periodic follow-up. One year post surgery, the patient is asymptomatic with good soft-tissue healing and adequate intraosseous bone fill at the surgical sites.
An 82-year-old highly functional female at baseline without significant comorbidities presented to the emergency department with generalized body aches, paresthesia, and difficulty walking. She received her first dose of the Pfizer COVID vaccine two weeks prior to presentation (see Figures , ). The patient reported generalized malaise and body aches during the first week after receiving her vaccination. However, during the second week, she had worsening symptoms and noticed increased difficulty in walking to the point where she had to use a walker for ambulation. The patient then subsequently sustained a fall due to her weakness, which prompted her visit to the emergency room. Her physical examination on presentation revealed normal mental status and speech. She had an unremarkable cranial nerve examination and no visible facial weakness or asymmetry was appreciated. Motor examination demonstrated normal bulk and tone in bilateral upper and lower extremities, strength in bilateral upper extremities was noted to be 5/5 in both proximal and distal muscles, she had full extension and flexion of her wrists. Although she was able to sustain her bilateral lower extremities against gravity for over five seconds, the examination of muscle group strength testing showed muscle weakness of 4/5 in hip flexors. Her sensation to light touch was intact in bilateral upper and lower extremities but decreased to pinprick in bilateral lower extremities up to the knees. No dysmetria was noted on finger to nose testing. The patient had areflexia in both upper and lower extremities. CT of the brain was normal. Routine labs were unrevealing. Coronavirus Cov-2 PCR was negative. A lumbar puncture was performed and cerebrospinal fluid analysis showed albuminocytologic dissociation (protein of 88 and WBC of 4), consistent with the diagnosis of GBS. The patient was admitted to the Neurology unit and started on intravenous immunoglobulin (IVIG). The patient developed labile blood pressure on day 2 that resolve before discharge. She developed back pain and MRI lumbar spine demonstrated the enhancement of cauda equina nerve roots also consistent with the diagnosis of GBS (see Figure ). The patient did not show any signs of respiratory compromise and clinical improvement was appreciated after three doses of IVIG, the patient completed five days of IVIG. No complications were observed during or after the treatment. The patient received physical therapy during the hospital stay and was discharged to acute rehabilitation facility. She was referred to the outpatient Neurology Clinic for follow up Nerve Conduction Study (NCS) and electromyography (EMG) to be done in three to four weeks. The Centers for Disease Control (CDC) was notified about this complication associated with the vaccination.
A 19-year-old gravida 1 diabetic mother with no family history of seizures or tuberous sclerosis was noted to have, on fetal echocardiogram at 25 weeks gestation, two large, homogenous, oval echogenic mass in the LV []. One mass obstructed mitral valve inflow [Figure and ], and there was no flow in the left ventricular outflow tract (LVOT) to the aortic valve. The additional CR was noted in the ventricular septum beneath the aortic valve (). Mild LV hypoplasia with moderately diminished LV systolic function was noted (). The tricuspid and pulmonary valves measured normal for gestational age and demonstrated normal Doppler patterns. However, the mitral and aortic valves measured small for gestational age and there was appropriate right to left shunting across the ductus arteriosus, but inappropriate retrograde filling of the ascending aorta consistent with HLHS physiology. Fetal Echo at 32 weeks gestation showed a hypoplastic LV and inappropriate left to right shunting across the foramen ovale []. There was no history of fetal arrhythmia or hydrops and the fetus was delivered by inducing labor at 38 weeks gestation. Prostaglandins were started for ductal patency and a postnatal Echo confirmed the diagnosis of HLHS. A large LVOT CR causing complete LVOT obstruction [Figure and ] and additional small rhabdomyomas in the LV apex and free wall were noted (3c). No neuro-cutaneous markers of tuberous sclerosis were noted. On day 11 of life, he underwent a successful Norwood-Sano procedure followed by right bidirectional Glenn procedure at 5 months of age. The large LV tumor occluding the LV inflow and outflow tract had resolved by 5 months of age, however, several smaller tumors in the interventricular septum and free wall were still present. Repeat brain MRI performed a few months after Glenn palliation showed no evidence of cranial tumors. At age of 2 years, he underwent a fenestrated lateral tunnel Fontan with left pulmonary arterioplasty. Follow-up Echo showed resolution of the multiple small tumors in the interventricular septum and his most recent echocardiogram at 6-years of age shows resolution of tumors in LVOT [Figure and ]. The patient did not have any evidence of tuberous sclerosis by examination or neuroimaging at his most recent follow-up.
This case study reports a 47-year-old Middle Eastern woman, married for 17 years with primary subfertility conceived with in vitro fertilisation in India. She was pregnant with dichorionic triamniotic triplets. Her body mass index at initial visit at 10 weeks was 28 kg/m2. She refused fetal reduction in her first trimester and continued as triplet pregnancy. She had irregular follow-up to our tertiary care hospital throughout the pregnancy.\nAt 10 weeks, she was started on low-dose aspirin to aid placentation and alpha methyldopa for her hypertensive disorders of pregnancy. She was on low molecular weight heparin from early pregnancy. Her glucose tolerance test done at 16 weeks had abnormal fasting and 2 hour values, so she was started on diabetic diet, glucophage and then insulin to achieve glycaemic control. She had normal anomaly scan for the three babies. Her gestational weight gain was 17 kg. At 32 weeks, she received betamethasone for fetal lung maturity and was planned for elective caesarean section at 35 completed weeks of gestation, as her home glucose monitoring values and blood pressure were in control.\nTwo days before her scheduled caesarean, she arrived by ambulance to the emergency department (ED) at 02:00 hours with epigastric pain. She did not have headache or blurring of vision but was complaining of severe chest pain and difficulty in breathing. She was in left lateral position to help perfusion. Blood pressure on arrival was 202/127 mm Hg and before any antihypertensive and magnesium sulfate could be administered, she had an eclamptic fit for 1 min, which progressed to cardiac arrest. The patient had collapsed while being transferred to labour suite from ED on the ED bed.\nThe patient was made supine, manual uterine displacement and CPR started immediately. Meanwhile, code was announced so that the crash call team could be assembled. Manual uterine displacement, resuscitation and intubation continued but as the patient was not responding and remained pulseless, immediate decision to perform urgent and immediate hysterotomy on site of collapse was taken by the obstetrician present. The incision was taken at approximately 4 min postcollapse.\nResuscitative hysterotomy was performed with the small scalpel blade that was at hand to prevent wasting any time. Abdomen was opened by transverse (Joel-Cohen) incision as the obstetrician was more comfortable with it rather than vertical midline incision. Meanwhile, operative room staff was called for the instruments. On-call neonatologists were alerted to expect three preterm babies. Meanwhile, the chest compressions were continued as the hysterotomy was being performed. Babies delivered one after another were handed over to the midwives and neonatologist present.\nCPR continued and the mother was intubated and given two doses of epinephrine. She regained spontaneous circulation around 15 min after incision. She did not have noticeable bleeding during the procedure and we had almost finished closing the abdomen when circulation had returned and so did not require shifting to theatre. Oxytocin drip was started and uterus was contracted by the time we shifted her to intensive care unit (ICU). The babies weighing 1.9 kg, 1.98 kg and 2.1 kg each initially had low APGAR scores and recovered well in neonatal intensive care unit.\nThe patient was given triple antibiotics (ampicillin, gentamicin and metronidazole), started on labetalol infusion and magnesium sulfate for 24 hours postdelivery. On the second day, the patient was extubated and weaned off sedation. The patient received feeds via nasogastric tube. She needed dialysis for 3 days. She had slurred speech and difficulty walking for 10 days, but with intense chest and limb physiotherapy, she recovered.\nThe patient was shifted from the ICU to the postnatal ward on day 7 and her antihypertensives were gradually tapered. Prior to her discharge, she was mobilising well and able to breastfeed her babies. She went home with her babies in good condition after 18 days postarrest.
A 35-year-old male presented with a swelling in the right lower abdomen, reportedly existing for three months. He was apparently well till three months ago, when he developed pain over the right lower abdomen, radiating down to the right lower limb. Pain increased with exertion and was partially relieved with rest and analgesics. He subsequently noticed a swelling in the right iliac region which increased in size progressively. There was no history of trauma. Examination revealed a swelling over the right iliac fossa which was smooth, immobile, and firm in consistency. He had right hip fixed flexion deformity of 30 degrees with further flexion up to 110 degrees. Routine blood investigation results were normal.\nPlain radiograph showed a lytic lesion in the right iliac wing, measuring 5 × 6 cm with minimal periosteal reaction (). Ultrasound showed a 12.5 × 12.8 cm large solid mass in right iliac fossa with destruction of underlying iliac bone suggestive of chondrosarcoma or round-cell tumor. Bone scan was done which showed a primary lesion of the right iliac bone. MRI showed a large lytic lesion involving the right iliac bone with a large soft tissue component, with heterogeneous signal intensity with lobulated margins and hypointense capsule with no encasement of the neurovascular bundle. Involvement of the gluteal muscles and iliopsoas muscles was also noted (Figures and ). These features were more in favor of an aggressive neoplastic lesion. A trucut biopsy was done, and the sample being inadequate a repeat biopsy under ultrasound guidance became necessary. Ultrasound guided biopsy of right iliac fossa soft tissue mass lesion was done under local anesthesia which showed friable and necrotic tissue.\nSince the diagnosis was inconclusive, the patient was planned for excision biopsy of the swelling. On lateral position, the tumor was exposed. It had eroded the ilium and extended into the gluteal area in a dumbbell fashion. The well-encapsulated tumor was excised and sent for histopathological examination. Microscopically, it consisted mostly of organized hematoma, with central signs of recent haemorrhage. No signs of malignant degeneration were seen. The tumor consisted mainly of organized hematoma with a fibrous capsule (). The pathology report confirmed diagnosis of a pseudotumor. The complete haematological workup is listed in .\nPostoperatively, flexion deformity of hip improved and there was delay in wound healing due to serous collection which resolved spontaneously. At 2 years follow-up, there was no recurrence of the symptoms and the patient had returned to work.
A 31-year-old gravida 4 para 0 African American woman at 22-weeks gestation presented with vaginal bleeding to an outside hospital. Her obstetric history was significant for 2 therapeutic abortions and 1 spontaneous abortion. During prenatal care, the fetus was noted to have a unilateral dysplastic kidney. The patient’s family history was significant for two family members with cervical cancer and two family members with endometrial cancer. She was placed on bedrest with inpatient admission. 48 h after admission she developed pelvic pain and uterine contractions. She expelled a mass vaginally measuring 11 × 9 × 5 cm with no fetal contents. The pathology from the outside facility showed a showed a highly cellular tumor composed of spindle-shaped cells and bizarre multinucleated giant cells with focal myxomatous change with mitotic count is greater than 50 per 10 high power field (Positive for CD10, SMA, ER, PR, EMA) with the differential diagnosis including endometrial stromal sarcoma and undifferentiated uterine sarcoma. The patient underwent examination under anesthesia, demonstrating a 3 × 3 cm defect of the posterior vaginal wall with active bleeding, which was sutured for hemostasis. The cervix was long, closed, and high with no evidence of bleeding and the fetus was intact with normal heart tones. She was discharged in stable condition.\nAt 27-weeks, the patient was referred to our institution for maternal-fetal medicine, and gynecology oncology consultation. On initial evaluation, she reported no prior abnormal gynecology history and no history of infertility issues. She denied dyspareunia and bulk symptoms prior to or during pregnancy, and her periods before pregnancy were normal. A pap smear at 27-weeks gestation was normal and negative for human papilloma virus. Her intake physical exam at our institution revealed a normal pelvic exam with no evidence of residual vaginal mass, and an ultrasound revealed no intrauterine myomas.\nGiven the differential diagnosis included an endometrial stromal sarcoma, an undifferentiated uterine sarcoma, and a primary vaginal sarcoma, the patient underwent magnetic resonance imaging (MRI) without contrast of the chest, abdomen, and pelvis at 27 weeks. MRI findings were notable for a peri-centimeter cyst in the cervix consistent with a Nabothian cyst, a multiloculated cystic structure in the fetal abdomen consistent with dysplastic kidney, and an anterior placenta. There was no evidence of metastatic disease.\nDue to the inability to ascertain whether the cancer had originated in the uterus, cervix or vagina, the patient desired definitive management for possible uterine sarcoma. In consultation with maternal fetal medicine and gynecology oncology specialists, the decision was made to proceed with a cesarean hysterectomy at 36 weeks. Betamethasone 12 mg intramuscularly was given for two doses 24 h apart with the first dose at 35 weeks and 6 days gestational age.\nAt 36 weeks and 1 day, she underwent planned exam under anesthesia of the vaginal canal with cesarean hysterectomy, bilateral salpingectomy, and peritoneal biopsies. She delivered a male infant weighing 2520 g with Apgars of 9 and 9. Operative findings were notable for normal appearing pelvic organs. There were no palpable or visibly enlarged pelvic/periaortic lymph nodes or evidence of metastatic disease throughout the peritoneal cavity. The neonate was admitted to the NICU post-delivery for prematurity. Neonatal US confirmed a right multi-cystic dysplastic kidney. He was transferred out of NICU on day 2 of life, and discharged home with his mother.\nThe final surgical pathology report was benign: no malignant or neoplastic cells were seen. The patient was seen at her 6-week postpartum visit and was doing well with a normal pelvic exam. At a follow-up surveillance visit 3 months postpartum, she was noted to have a 1 cm posterior vaginal wall lesion; biopsy results showed a high-grade sarcoma. The tumor was estrogen receptor and progesterone receptor positive. Computer tomography (CT) with contrast of the chest, abdomen, and pelvis showed no evidence of metastatic disease. The patient underwent an upper vaginectomy and proctoscopy. Operative findings were notable for 1.5 cm polypoid lesion in the posterior vagina wall 5 cm distal to the posterior vaginal apex. A 1 cm surgical margin was obtained circumferentially around this lesion where feasible, and surgical pathology showed high-grade sarcoma () of the polyp lesion with negative surgical margins. The tissue removed from this excision showed residual sarcoma, morphologically similar to the original pathology from the outside hospital pathology. All other vaginal biopsies were also negative for malignancy. Marker seeds were placed in the proximal and distal margins of the areas where the sarcoma was excised. She was treated adjuvant high dose rate vaginal brachytherapy using a multi-channel vaginal cylinder. She received a biologically equivalent 2 Gy dose (EQD2) of 45 Gy to the whole length of the vagina and 60 Gy to the post-operative bed (). A CT scan 36 months after surgery continue to show no evidence of disease. She remains disease free 58 months after completion of vaginal brachytherapy.
A 49-year-old female patient presented with a 2-year history of progressive right lumbar pain radiating to her right lower extremity. The pain, which originated in the right buttock, extended down the posterior aspect of the right thigh and the calf to the ankle. She was operated on for the right ovarian hydatid cyst 16 years before. There was no history of prior trauma.\nOn admission, the neurological examination revealed hypoesthesia, a slight weakness and atrophy of the abductor and extensor of the hip and the flexor muscles of the knee, and areflexia and radicular pain of the right leg. Treatment that included anti-inflammatory drugs and exercise did not alleviate the pain. Therefore, a possibility of lower lumbar disc herniation was considered. An MRI examination that was intended to detect lumbar disc herniation was performed. The MRI of the lumbar spine showed no nerve-root compromise, but on the very right parasagittal images, a globular, septated cystic lesion in the right side of the pelvis was noticed ().\nA review of the axial computed tomographic (CT) scan of the pelvis showed a multiloculated cystic mass located in the right presacral area (). According to the pelvic MRI, the multiloculated cystic lesion was located anterior to the right sacral foraminae. The lesion had low T1 and high T2 signal intensities, without contrast enhancement. Besides this right-sided cyst, there was another lesion with the same imaging characteristics in the posterior left side of the rectum (). The right-sided lesion was in close proximity to the sacral nerve fibers. On the oblique coronal views, the displacement and compression of the lumbosacral nerve trunk could be well seen (). There was no expansion or destruction in the bony structure of the foraminae.\nBecause of the previous history of the right-sided oophorectomy due to hydatid disease, the patient was operated on with the presumptive diagnosis of hydatid disease. The diagnosis of a hydatid cyst was confirmed histologically after surgical removal of the lesion. At the time of the operation, the sacral nerve trunk was found to be displaced and compressed by the right-sided presacral cyst. Mebendazole (100 mg twice daily for 4 months) was prescribed.
A 66-year-old, 70 kg male patient with a BMI of 33 presented for laparoscopic cholecystectomy. He was a chronic smoker with chronic obstructive pulmonary disease which required salbutamol MDI. He was a hypertensive on treatment with oral ramipril and losartan. He had one episode of acute coronary syndrome 18 months back which required percutaneous intervention with stenting of right coronary artery. Subsequently, the patient was advised to take aspirin and clopidogrel orally which had been stopped 5 days prior to the surgery. History pertaining to other organ systems was unremarkable. On preoperative evaluation he was NYHA class II with decreased effort tolerance (<4 METs). His ECG showed normal sinus rhythm with left anterior fascicular block [] while his resting echocardiography showed regional wall motion abnormalities in the right coronary artery territory with an ejection fraction of 40%.\nIn the operation theatre ECG, non-invasive blood pressure and SpO2 monitor were attached. After intravenous access and radial artery cannulation, anaesthesia was induced with 150 mcg of fentanyl and 100 mg of propofol. 35 mg of atracurium was given to attain muscle relaxation to facilitate tracheal intubation. There was a fall in blood pressure to 70/40 mmHg after induction which was managed with 6 mg of ephedrine. The haemodynamics recovered to normal limits after endotracheal intubation. However, immediately after creation of pneumoperitoneum, the patient developed regular WCT with a ventricular rate of 150/min and a mean blood pressure of 80-90 mmHg. The abnormal heart rhythm immediately reverted back to normal on deflation of pneumoperitoneum. An open cholecystectomy was planned in consultation with the surgeons. Following skin incision the same pattern of WCT recurred. The arrhythmia reverted spontaneously within 3 to 4 min of stopping the surgery. The surgery was abandoned and the patient immediately shifted to ICU without reversal of anaesthesia. In the ICU, the patient had multiple episodes of WCT with severe hypotension which required synchronised DC cardioversion with 100-150 joules biphasic energy. An infusion of dopamine was also started at the rate of 10 mcg/kg/min. Cardiologist was urgently summoned who diagnosed the WCT as interfascicular VT on 12 lead ECG [Figure , ] and thus a loading dose of 150 mg of amiodarone was administered over 10 min intravenously followed by infusion at 1 mg/min for 6 hours and 0.5 mg/min for subsequent 18 hours. Despite the treatment the patient had multiple episodes of WCT which required cardioversion again with 150 joules of biphasic energy. All his biochemical parameters like serum electrolytes, renal and liver function tests were normal, and no increase in cardiac enzymes occurred thus ruling out possible myocardial ischemia. An echocardiography could not be done at as facilities of in-bed echocardiography were not available with us. The patient was weaned off inotropes over a period of 6 hours, the tracheal tube was subsequently removed, and he could be discharged from the hospital after 3 days.
A 51-year-old male presenting with abrupt onset drowsiness was admitted to our hospital. A neurological examination revealed mildly confused orientation, gaze limitation to the left side in both eyes, mild palsy of the left face, and right hemiparesis. The patient had a history of hypertension which was being medically managed. Brain computed tomography (CT) revealed a hematoma in the posterior portion of the lower pons with minimal mass effect (). After 2 months of conservative management, his mental status and gaze limitation were completely recovered while the facial palsy and hemiparesis were improved but still remained.\nMagenetic resonance angiography (MRA) was performed to evaluate for a possible lesion causing an intracranial hemorrhage (ICH); a fusiform aneurysm was found in the right VA. Although the aneurysm was located adjacent to the pontine hematoma, it appeared unlikely to be causing an ICH because the hematoma was totally encased within the pons and there was no evidence of subarachnoid hemorrhage on serial brain CT and MR. On angiography, the fusiform aneurysm was located distal to the origin of the posterior inferior cerebellar artery (). Because there was no neurological deterioration during a thirty minute balloon test occlusion, we performed proximal ligation of the VA via a retromastoid suboccipital craniotomy. The procedure was performed without any complications and the patient was discharged and received physical therapy in local clinics.\nEight months after the operation the patient visited our hospital complaining of involuntary left facial twitching. The spasm had initially begun in the left orbicularis oculi muscle during physical therapy. Despite two years of medication, the frequency of the spasms increased, the severity of the spasms increased, and the spasms spread to involve other ipsilateral facial muscles. During careful consideration of the cause of these spasms we identified a hemodynamic difference between the pre- and post-ligation MRAs. The left VA, which was compressing the facial nerve REZ, did not change architecturally but enlarged in diameter (). Although pontine ICH is also considered a potential cause of HFS, the long interval between the ICH and the onset of HFS prompted us to perform MVD in this patient. The facial nerve REZ was subsequently found to be compressed by an elongated VA (). Immediately after decompression the patient was free from symptoms and has not had a recurrence during 2 years of follow-up.
A 50-year-old, 5'1" tall, 140-pound Caucasian female was seen at a specialty outpatient orthopedic practice with a chief complaint of severe left knee pain. She had a medical history significant for prolonged glucocorticoid exposure for the treatment of ulcerative colitis. She had undergone colectomy in 1994, following which daily corticosteroid administration had been replaced with intermittent, pulse-dosed glucocorticoids, as required. The onset of left knee pain had occurred in March of 2019 with a rapid increase in pain severity. She had developed difficulty in walking, necessitating the use of a cane for ambulation, and had started taking ibuprofen on a regular basis. Three months later, she had been evaluated by a rheumatologist and found to have bone infarcts of both the femur and tibia. This had led to the diagnosis of ON of the knee, presumably corticosteroid-induced due to exposure history. At that time, she had started alendronate and calcitonin. However, the pain had persisted and she had been prescribed oral morphine 15-30 mg every eight hours as needed by her primary care physician (PCP), following which she had been transitioned to a 25 mcg/hr fentanyl patch six months after the initial presentation due to worsening pain.\nDespite additional analgesics, the pain had progressed with new-onset radiation into the distal tibia, ankle, and foot. This had prompted an evaluation with an orthopedic surgeon, who did not recommend surgical intervention. A second surgical opinion had been obtained with the recommendation to undergo intraarticular corticosteroid injections (CSI) for symptom management until the degree of progressive collapse necessitated TKA. A third surgical opinion had recommended against TKA since the location of the lesion in the tibia was too distal to the joint line and had instead considered transfemoral amputation as the best surgical option. A CSI had also been performed in September 2019 with no improvement. In October 2019, as her debilitating pain had continued, her PCP had increased her fentanyl patch dose to 37.5 mcg/hr, with oral morphine for breakthrough pain. At our evaluation in November 2019, her pain was 8/10 with ambulation and 5/10 with rest on the visual analog scale (VAS).\nThe patient arrived in a wheelchair for the clinical exam. Unassisted ambulation demonstrated an antalgic gait. She was tender to palpation over the left tibial tuberosity and had moderate laxity with a clear stopping point upon varus testing. She demonstrated 3/5 strength in left hip flexion, knee extension and flexion, and 4/5 strength in bilateral extensor hallucis longus muscles. No other significant findings were noted in the physical exam. MRI review from five months prior demonstrated medullary infarcts of the distal femoral metaphysis extending into the proximal aspect of the medial femoral condyle and of the proximal tibial metaphysis with extension into the tibial plateau (Figure ). X-rays from three months prior to the visit were notable for osteopenia and Modified Ficat and Arlet stage II ON (Figure ). Due to the failure of previous conservative management and the patient's desire to avoid amputation, an in-depth discussion was held about nonsurgical interventions. The patient elected to proceed with intraosseous treatment to the proximal tibia and distal femur with BMC, DBM, and leukocyte-poor 10x concentrated PRP in November 2019. Prior to undergoing bone marrow aspiration, corticosteroids and nonsteroidal anti-inflammatory drugs (NSAIDs) use were restricted for the patient for a minimum of six weeks and 14 days, respectively.\nOn the day of the procedure, bone marrow was harvested from the iliac crest under sterile conditions with ultrasound guidance. Following local anesthesia with 1% lidocaine and 0.25% ropivacaine, an 11-gauge bone marrow biopsy needle was utilized to obtain 45 cc of bone marrow aspirate (BMA) from four locations along the posterior superior iliac spine (PSIS) on each side of the pelvis, for a total of 90 cc. The BMA was hand-processed in a sterile ISO-7 cleanroom under ISO-5 laminar flow cabinets. The buffy coat was first isolated through centrifugation, producing 1-5 mL of BMC. This was transported sterilely to the procedure room for use. The PRP was prepared with 200x g centrifugation to separate the plasma and buffy coat from the red blood cells (RBC). The subsequent remaining supernatant was RBC- and leukocyte-poor. On the same day, with the patient under monitored anesthesia care and after full surgical prep, 15-gauge and 11-gauge 1.5-inch intraosseous needles were manually placed into the lesions in the medial femoral condyle and medial tibia plateau, respectively, under fluoroscopic guidance (Figure ). The tibial lesion was injected with 7 cc of BMC, 1 cc of Arthrex AlloSync Demineralized Bone Matrix Putty (Arthrex, Naples, Fl), and 8 cc of autologous 10x leukocyte-poor PRP. The femoral lesion was injected with the same injectates, but with a volume of 3 cc of BMC (12 cc total volume), as it was a smaller lesion. The BMC had a total nucleated cell count of 3.5 billion and viability of 89%, as determined by a Bio-Rad TC-20 automated cell counter (Bio-Rad Laboratories, Inc., Hercules, CA). After the procedure, the patient was advised to continue weight-bearing as could be tolerated while wearing a knee unloader brace until her first follow-up at six months post-procedure.\nThe patient tolerated the procedure well and continued with minimal activity following the injection. At two months post-procedure, she initiated physical therapy with a focus on aquatic therapy but was unable to tolerate it due to severe pain. However, at the three-month mark, she began to report a gradual improvement in pain and function. Nine months following the procedure in August 2020, she was able to participate in aquatic therapy without pain, with progression to land-based physical therapy after three sessions. Her VAS was noted to be 0/10 pain with rest and household ambulation at her nine-month follow-up. She no longer required a wheelchair, unloader brace, or any assistive devices other than a knee sleeve. Her narcotic load had also decreased. Though still requiring the fentanyl patch at 37.5 mcg/hr, she no longer needed breakthrough oral morphine for pain control. Her most recent MRI obtained at nine months post-procedure showed an unchanged size of the bone infarcts in the distal femur and proximal tibia; however, there was a noted interval decrease in the extensive surrounding bone edema (Figure ). At 15 months post-procedure, she was no longer taking any opioids for breakthrough pain and was slowly titrating down the fentanyl patch. Her pain was still a 0-1/10 at its worst, intermittent in nature, and most notable with increased activity. She had resumed all of her pre-morbid activities without any assistive devices, including ambulation.
A previously healthy 24-year-old Caucasian woman presented to her primary care physician with right-sided odynophagia, sore throat, and subjective fevers following an upper respiratory infection. The patient was given an oral steroid course and azithromycin for presumed pharyngitis. Over the next four days she developed progressive neck swelling prompting her presentation to the emergency department. Due to worsening odynophagia and neck swelling, she was unable to tolerate solid foods.\nOn exam, the patient was noted to have tenderness, induration, and mild erythema along the right lateral neck. Flexible laryngoscopy revealed a normal larynx and upper aerodigestive tract, with no airway compromise or impingement. The patient had normal true vocal cord mobility bilaterally. Computed tomography with intravenous contrast was performed and showed a large cystic lesion with air and fluid extending from the right larynx laterally and inferiorly toward the thyroid. The patient underwent a needle decompression of the cystic mass, producing approximately 10 milliliters of purulent debris. She was admitted to the hospital for intravenous antibiotics and observation.\nWhile in the hospital the patient improved clinically, but her cystic lesion reaccumulated fluid and ultimately necessitated decompression with a pigtail catheter drain placed by interventional radiology. The drain provided adequate decompression of the neck mass, allowing for improved swallowing ability.\nDuring her hospital stay the patient underwent a fluoroscopic sinogram of the pigtail catheter drain and her cystic neck mass to further explore its origin (). An aberrant connection of the cystic mass to the apex of the right pyriform sinus was discovered, suggesting the presence of a right third or fourth branchial cleft cyst and fistulous tract.\nThe patient was discharged home with a prolonged course of oral clindamycin and a drain in place. She returned to clinic five days later with decreased drain output and significantly less turgor around the area of the neck mass. Two weeks later she was taken to the operating room for direct laryngoscopy, esophagoscopy (Figures and ), and chemocauterization of the fistulous tract with 40% trichloroacetic acid (TCA).\nMicropledgets soaked in TCA were carefully placed into the fistulous tract (). This was repeated for a total of three times, and the area was then rinsed with saline and inspected ().\nPostoperative examination ten days after laryngoscopy and cauterization revealed no evidence of fistula patency, and the patient's neck mass had largely resolved. Similar findings persisted in follow-up one month later. Repeat esophagram four months later confirmed fistula closure ().
A 4-year-old boy presented to the radiology department as a referred case from the department of pediatrics, where he was primarily admitted for cough, bilateral nystagmus, and gaze instability. His past medical history revealed his difficulty in walking and maintaining an upright posture. These symptoms emerged at six months of age and gradually worsened. Further questioning of the patient’s mother revealed poor and delayed developmental milestones. He achieved rollover at the age of five months and social smile at the age of three months. Currently, he is barely able to stand on his own and requires permanent support. He was delivered via an uncomplicated cesarean delivery and weighed 2.6 kg at birth. The postnatal history was negative for prolonged admission at the neonatal intensive care unit. Apart from a mild cough, there was no history of asthma, feeding difficulty, or respiratory problems. Detailed family history was taken, revealing a consanguineous marriage with a single male child. No other prior members in their family were affected.\nOn physical examination, the child appeared extremely thin and fragile. Mild facial dysmorphia was noted with a depressed nasal bridge and deepened orbital sockets. He weighed less than the 25th percentile (6.0 kg) on the pediatric growth chart for his age. He appeared to be awake and alert. However, when instructed, he was unable to focus his gaze on specific objects. Ocular examination revealed bilateral horizontal pendular nystagmus without myopia. Grade 1 ptosis that was more prominent in the right eye than the left was noted. The cardiovascular examination proved to be normal. A pulmonary examination revealed a prominent expiratory wheeze without chest indrawing. Examination findings of the cranial nerves, apart from the oculomotor nerve, were normal. The motor exam revealed hypotonia and ataxia with normal tendon reflexes.\nA full series of magnetic resonance imaging (MRI) scans were conducted at our radiology division to determine the cause of the delayed milestones and to figure out the source of the hypotonia and bilateral nystagmus. The axial T2-weighted MRI revealed total aplasia of the cerebellar vermis with prominent, thickened, and elongated superior cerebellar peduncles forming a characteristic molar tooth appearance. Furthermore, the fourth ventricle appeared enlarged and triangular, giving it a slight batwing appearance (Figures -). Based on these clinical findings, MRI scans, and family history, a diagnosis of JS was forwarded to the clinical team.
Case reports are exempt from institutional review board approval at our institution. A 75-year-old woman was evaluated for long standing right-sided nasal obstruction, dependent mouth breathing, clear rhinorrhea, congestion, and hyposmia not relieved by intranasal steroids or nasal irrigations. She initially presented with a history of previous endoscopic sinus surgery in Russia more than 20 years ago followed by nasal polyposis treated with ambulatory cauterization. She was also noted to have a history of nonmelanotic skin cancers of the nose treated with radiation in Russia. Physical exam findings included a fleshy intranasal lesion that, in the setting of previous nasal skin cancer treated with radiation, raised a concern for possible secondary carcinoma. Computed tomography showed complete opacification of the right maxillary sinus, obstruction of the right ostiomeatal complex, and soft tissue density in the right nasal passage (). Initial biopsy revealed a nasal mass that originated in the right inferior meatus. Pathology showed multiple polypoid fragments lined by a respiratory type epithelium with underlying edematous stroma with mild chronic inflammation. There was invagination of the surface epithelium into the underlying stroma resulting in nested aggregates of bland glandular and mucinous cells and focally benign squamous epithelium (). These features were found to be consistent with a benign inverted papilloma. The patient presented with continued nasal obstruction and was evaluated for definitive treatment. Given her diagnosis of inverted papilloma and chronic rhinosinusitis, complete excision and revision endoscopic sinus surgery was recommended. During endoscopic sinus surgery, an exophytic mass with abnormal maxillary mucosa was seen emanating from the left inferior meatus that was thought to originate from the right maxillary sinus, given that it was protruding through a bony dehiscence into the inferior meatus and nasal cavity. Right partial inferior turbinectomy was performed, along with right extended maxillary antrostomy and stripping of maxillary mucosa to remove the entirety of the presumed inverted papilloma base within the maxillary sinus. Intraoperative and postoperative pathology again showed inverted papilloma. Given
The patient is a 15-year-old Caucasian female who was diagnosed with CRMO in 2007 at 5 years in the context of right thigh pain. provides a timeline of the patient's symptoms and course of disease. Magnetic resonance imaging (MRI) showed multifocal abnormal bone marrow signal in the right femur, the left femoral neck, and the proximal epiphysis and metaphysis of the right tibia, which was associated with osteolysis and callus formation (). Radio-nucleotide bone scan found increased uptake involving the left sacrum, left proximal femur, and femoral neck region as well as the midshaft of the right femur and the proximal right tibia. Bone biopsy of the lytic lesion involving the midshaft of the right femur was not consistent with malignancy and showed red blood cells and scattered neutrophils and lymphocytes. Over the next several years (2007–2015), she was followed by orthopedic surgery and was treated only with intermittent ibuprofen as needed for pain. She was noted to have a leg length discrepancy at the age of 7 years, and in 2014, at the age of 12 years, she had surgery to fuse the growth plate to prevent right leg growth (right leg was 4.5 cm longer than her left leg at that time).\nShe established Rheumatologic care in March 2015 at age 13, and right quadriceps muscle atrophy and failure to thrive with a weight and height under the third percentile were noted at this time. Bone scan revealed increased uptake in the right femur and asymmetry of activity in the growth plates of the knees and ankles with decreased activity in the right side compared to left. She was treated with naproxen 250 mg twice daily (8.7 mg/kg BID) and prednisone 20 mg per day (0.7 mg/kg), tapered by 5 mg weekly. Two months later, prednisone was discontinued, and she continued to have good control of her leg pain on NSAID monotherapy. After a few months, she developed new diarrhea with vomiting and weight loss. Her blood pressure was elevated, and a renal ultrasound and electrocardiogram were unremarkable.\nShe was referred for Gastroenterology evaluation and was found to have positive stool occult blood along with a perirectal skin tag. Laboratory studies revealed anemia with a hemoglobin of 7.1 g/dL (nl 12–16 g/dL), thrombocytosis with platelets of 744 k (nl 150–450 k), erythrocyte sedimentation rate (ESR) of 69 mm/hr (nl 0–10 mm/hr), and C-reactive protein (CRP) of 129 mg/L (nl 0–3 mg/L). There was no family history of autoimmune disease, and tuberculin testing was negative. Colonoscopy revealed pancolitis with crypt inflammation and crypt abscesses with no granuloma consistent with ulcerative colitis (). NSAID therapy was discontinued, and treatment for UC with prednisone 1 mg/kg/day (40 mg) tapering by 5 mg weekly along with sulfasalazine was initiated. Infliximab 3 mg/kg infusions were added the following month.\nAt the visit for her second infliximab infusion, she presented with tachycardia, a blood pressure of 230/190, and headache and was admitted to the pediatric intensive care unit for hypertensive emergency requiring nicardipine infusion. Her examination was notable for right-sided Horner's syndrome, decreased right leg pulse pressure, and an abdominal bruit. Echocardiogram showed a small pericardial effusion with reduced left ventricular ejection fraction. Laboratory studies revealed negative ANA and ANCA screens, normal C3 and C4, and normal von Willebrand factor antigen. CT angiogram (CTA) of the abdomen and pelvis showed narrowing of the mid-aorta, proximal renal arteries, celiac artery, and superior mesenteric artery (Figures –). CTA of the chest showed marked descending thoracic and abdominal aortic wall thickening with progressive luminal narrowing and wall thickening of the visualized portion of the right common carotid artery and celiac trunk, enlarged left atrium, left ventricular hypertrophy, and a small pericardial effusion (). MRI/MRA brain showed anterior and posterior circulations of the brain were without occlusion or aneurysm with the patent carotid and vertebral arteries of the neck (Figures and ). Given these findings, she was diagnosed with TA complicated by middle aortic syndrome. She was started on metoprolol 50 mg daily (1.5 mg/kg daily), amlodipine 5 mg daily, famotidine 20 mg twice daily, aspirin 81 mg daily, and increased dose and frequency of infliximab from 3 mg/kg every 8 weeks to 5 mg/kg IV every 4 weeks along with prednisone 20 mg daily (0.6 mg/kg daily) and mesalamine 1 g BID. The patient continued infliximab every 8 weeks rather than every 4 weeks as recommended.\nMagnetic resonance (MR) angiography of the chest, abdomen, and pelvis and Cardiac MR three months later showed luminal narrowing of the distal thoracic and upper abdominal aorta similar to previous CT studies, stenosis of origin of celiac axis, stenosis of proximal superior mesenteric artery (SMA), and moderate stenosis of bilateral proximal renal arteries. Echocardiogram noted concentric LVH with mildly reduced function, measuring 45%, and normal coronary arteries.\nAt this point, the patient transferred her care to our rheumatology clinic, and despite treatment with infliximab 5 mg/kg every 8 weeks, she continued to report right thigh pain and developed new inflammatory arthritis of the left ankle and increased inflammatory markers with an ESR of 55 mm/hr (nl 0–20 mm/hr) and an elevated CRP of 73 mg/L (nl < 3 mg/L). Additionally, interval imaging found new wall thickening around the right common carotid artery. This was concerning for uncontrolled TA and CRMO activity. There was an unfortunate delay in treatment escalation due to social circumstances. Two months later, she started treatment with parenteral methylprednisolone 1 gram weekly for 8 weeks, and her dose of infliximab was increased from 5 mg/kg to 10 mg/kg every 4 weeks. Based on adult data demonstrating a positive response to higher doses of infliximab, the decision was made to increase the dose of infliximab instead of trying another TNF inhibitor.\nMultidisciplinary evaluation at Boston Children's Hospital, Center for Middle Aortic Syndrome by neurosurgery, nephrology, and rheumatology, led to recommendations of a prednisone dose increase and the addition of methotrexate 15 mg/m2 weekly to infliximab 10 mg/kg every 4 weeks. At this time, her echocardiogram revealed moderate left ventricle dilation and mildly depressed left ventricular systolic function. One month later, repeat head and neck CTA showed progression of her right carotid artery stenosis to 80%. Her ESR had normalized, and CRP decreased to 7.8 mg/L at this time. Given her worsening carotid artery stenosis, infliximab was increased from 10 to 15 mg/kg every 4 weeks. On a combination of moderate dose prednisone, weekly methotrexate, and infliximab, she denied joint pain, swelling, abdominal pain, diarrhea, or blood in stool. Follow-up brain MRI/MRA three months later was normal. Repeat chest and abdomen MRA showed all of the areas of stenosis appeared to be stable and inflammatory markers had normalized.\nCurrently, her clinical course is stable without further anatomic progression, and she has normal inflammatory markers. Thus, she has continued on her current regimen with infliximab 15 mg/kg every 4 weeks and methotrexate 15 mg/m2 once weekly. Prednisone decreased gradually and discontinued. She has not developed any infectious sequelae on this regimen. Her blood pressure is stable on carvedilol alone. We continue to assess blood work monthly. Given her use of prednisone, vitamin D level was followed and found to be low, and she has started on a vitamin D supplement. Dilated ophthalmologic exam was normal. Repeat MRI of the lower extremities and bone scan show that her CRMO lesions are inactive. Repeat MRI/MRA of the brain, chest, abdomen, and pelvis in June of 2018 show stable changes without the need for stenting. Given the predisposition for autoinflammatory conditions in this patient, the authors are considering genetic testing in search of a monogenic cause that may support a unifying diagnosis.
A 29-year-old multigravida visited at 18-week gestation for prenatal genetic counseling. Her first two children delivered by cesarean section have normal developmental milestones till date. The third pregnancy was an unsupervised one when she delivered vaginally at home. The baby did not cry at birth and was kept on ventilator support for 10 days. He was diagnosed with hypoxic-ischemic encephalopathy Grade 3 and developed spastic cerebral palsy and global developmental delay later in life. The parents were not satisfied and went to some fetal medicine center for the evaluation of that baby. They were advised to get clinical exome sequencing of the baby when he was around 4 years of age. On clinical exome sequencing [], the child was found to have a deletion “AG” and insertion of “TT” between nucleoside “19” and “20” causing change in amino acid from serine to phenylalanine at codon 7 which was confirmed by Sanger validation. The child had defective mitochondrial and peroxisomal fission 2 (AR-617086) and was homozygous for this mutation. His ophthalmic examination and brain stem-evoked response audiometry test were normal. He had no history of seizure till his present age of 5 years. The magnetic resonance imaging of the brain showed symmetrical hyperintensities in bilateral thalami. The biochemical analyses including serum lactate levels were normal.\nThe pedigree chart is shown in . Surprisingly, on testing the parents, the mother came out to be homozygous and the father heterozygous for the same variant [ and ]. The mother was phenotypically normal, with normal intelligence quotient and without visual or neurological abnormalities. In the present pregnancy, she came for genetic counseling at 18 weeks and had normal genetic sonogram. The dilemma was whether to go for invasive prenatal diagnosis or not and what if the current fetus also turns out to be homozygous. We counseled the couple that this mutation could be a variant of unknown clinical significance, but they were adamant to get the invasive testing for confirmation. Amniocentesis was, however, done on the patient's request and Sanger sequencing revealed homozygous deletion in exon 3 of the MFF gene [].\nThe couple was again confused and finally decided to continue the pregnancy, and an elective cesarean section was done at 39 weeks, delivering a female child with weight of 3245 g and Apgar score of 8/9 at 1 and 5 min. The baby is under follow-up and has normal milestones at 11 months of life.
A 28-year-old south Indian native female presented with a complaint of localized pus discharge and large swelling on palatal aspect of right maxillary incisors of about 3 weeks’ duration. The symptoms started as occasional numbness in relation to the right upper lateral incisor since about 1 year and there was no history of trauma. Clinically, the tooth was normal in appearance without any mobility or pain; no discoloration, cracks, or fracture lines were detected for any of the anterior teeth. It was further noted that both right and left maxillary lateral incisors had prominent cingulum resembling a form of dens invaginatus []. Diagnostic intraoral periapical radiograph showed large irregular radiolucency of size 2 × 3 cm size involving the apices of right central and lateral incisors. Moreover, all the incisors had two separate canals arising in pulp chamber and reuniting in the root canal to have a single exit []. Both central and lateral incisors on right side were found to be nonresponsive for vitality test using EndofrostTM refrigerant spray (Coltene/Whaledent, Germany). Results of electric pulp testing were not consistent and a test cavity preparation was done for the teeth in question without local anesthesia under air water spray and the response was null. Based on the clinical and radiographic findings, it was decided to proceed with endodontic treatment on both central and lateral incisors.\nThe presence of two canal pattern was made sure with tube shift method radiography. The access cavity preparation was proceeded under direct vision and locating the orifices of central incisor was relatively effortless compared to lateral incisor; and the orifices were located mesiodistally []. For lateral incisor, one lingual pit was noticed in access cavity in addition to the buccolingual canal orifices, suggesting this tooth as a mild form of dens invaginatus. After locating the canals and instrumenting to an approximate working length, infected tissue fluid started expressing through the orifices of the lateral incisor (and never through central incisor) suggesting this tooth as the focus of infection. Sodium hypochlorite (5.25%) has been used as intracanal irrigant and cleaning and shaping was completed for all the canals. Because of the very rare nature of the case complicated by internal Type II canal configuration and external morphology of dens invaginatus, it was decided to take a spiral CT for confirmation of internal anatomy and comparison with contralateral incisors. This was necessitated, as we were planning for the nonsurgical healing of the lesion. The CT examination revealed that for both the central incisors, canals were located mesiodistally [], whereas, it was located buccolingually for both lateral incisors [].\nAfter drying the canal, calcium hydroxide medicament was coated on the walls of root canals with the help of paste carrier. Coronal orifice was sealed with zinc oxide eugenol restoration. In the subsequent appointment, after 1 week a fresh calcium hydroxide medicament was placed inside the root canals of both central and lateral incisors. In spite of the canal debridement and weekly renewal of the calcium hydroxide in three subsequent appointments, expression of infected tissue fluid through the orifice of lateral incisor had not regressed. Hence, the treatment plan was modified and a combination of minocyclin, metronidazole, and ciprofloxacin antibiotic paste was placed in the draining canal. Following this, the palatal swelling decreased in size and the canals of lateral incisor appeared dry without fluid discharge. Obturation was performed with conventional Gutta-percha points using lateral compaction technique with zinc oxide-eugenol cement as sealer. Access cavity was sealed with glass ionomer cement over which composite restoration has been given. The patient was recalled at regular intervals of 1, 3, 6, and 12 months and review radiograph 1 year after surgery showed excellent healing of periapical pathology as evidenced by decrease in size of the lesion with appearance of bony trabeculations [].
A 42-year-old man with a history of well-controlled hypertension presented to the hospital complaining of progressively worsening headaches associated with dizziness and blurry vision for three days. The patient denied any history of fever, chills, vomiting, head trauma, or loss of consciousness. He also denied any history of alcohol, vitamins, or anabolic drug use. Upon presentation, the patient was hemodynamically stable with normal physical and neurological examinations. All blood tests were within normal limits. CT head without contrast revealed acute right crescent-shaped subdural blood collection measuring up to 6 mm in thickness with mild falx herniation and right to left midline shift of 4 mm (Figures and ). CT cerebral angiogram showed no aneurysm and no active bleeding within the hematoma with prominent cortical veins on the right peri-sylvian region. CT angiogram of the neck revealed no abnormalities, and CT cervical spine reported no evidence of acute spine fracture. Diagnostic angiogram revealed no evidence of vascular malformation. The patient underwent a successful right craniotomy for evacuation of his atraumatic acute right-sided SDH with coagulation of the bleeding veins and placement of a subdural drain and a bone flap to the skull. A postoperative CT head showed successful evacuation of the SDH (). The patient remained hemodynamically and neurologically stable. The drain was removed, and the patient was then discharged home with outpatient follow up plan.\nHowever, one week later, the patient returned to the hospital complaining of persistent dizziness and headaches not improving with pain medications. A repeat CT head showed mixed density right hemispheric SDH that has increased in size with increased mass effect and midline shift suggestive of chronic reaccumulating SDH. Neurosurgery team recommended a repeat CT head after 24 hours or earlier if any change in his neurological exam. CT head was subsequently done and showed stable hematoma with mild decreased midline shift for which the patient was started on steroids and ultimately discharged home with follow-up in outpatient clinics.\nTwo weeks after discharge, the patient started reexperiencing severe headaches with subjective fever of one-day duration for which the patient returned to the hospital. In ER, the patient was febrile with normal vital signs. Blood test was significant for leukocytosis with neutrophilic shift and elevated CRP and ESR. CT head without contrast revealed decrease in size of previous right SDH with a new area of diminished attenuation in the right frontal lobe suggestive of vasogenic edema (). MRI brain was performed confirming the CT findings with presence of right subdural empyema (Figures –). The patient underwent a right craniectomy for removal of previously placed bone plate and for exploration washout of the subdural collection. During the procedure, a yellowish purulent material was seen in the subdural space from which multiple specimen swabs were taken and sent for culture. The subdural space was well debrided. The patient was started on broad-spectrum antibiotics with intravenous vancomycin, cefepime, and metronidazole, awaiting cultures result. The previous bone flap was not placed back due to underlying infection. Swab culture remained negative for 96 hours before growing P. acnes bacteria. Antibiotics were then de-escalated to aqueous crystalline penicillin for a total treatment duration of 6 weeks.\nTwo months later, after completion of the full course of antibiotics and during a follow-up visit to the infectious disease clinic, the patient reported improvement of symptoms. Repeat blood test was negative for any sign of persistent infection. Appointment was then set with neurosurgery office for cranioplasty scheduling.
A 67-year-old woman was brought to the emergency unit after a traffic accident. She suffered multiple traumatic injuries including liver laceration, left femoral shaft fracture, pelvic bone fractures, and multiple rib fractures. Roughly three weeks later, she developed dyspnea and swelling of the left upper extremity. A contrast-enhanced chest computed tomography (CT) revealed a pulmonary embolism in the right pulmonary artery and thrombosis in the left brachiocephalic vein (). On a lower extremity CT angiography and Doppler ultrasound taken after the diagnosis of pulmonary thromboembolism, a lower extremity DVT was not detected. A left upper extremity venogram revealed complete obstruction of the brachiocephalic vein with lack of collaterals, implying the development of acute upper extremity DVT ().\nTo prevent the recurrence of a pulmonary embolism from the left upper extremity venous thrombi, and to reduce her left arm swelling, removal of venous thrombi was necessary. Because anticoagulation and thrombolysis were contraindicated due to her recent major trauma history, we decided to perform a thrombus aspiration. To prevent a further possible pulmonary embolism during the procedure, we planned temporary filtration in SVC.\nOne day following the diagnosis of upper extremity DVT and pulmonary thromboembolism, the Tempofilter II was placed in the SVC percutaneously, via the right femoral vein. After a right femoral vein puncture, a guide wire was threaded through the IVC, right atrium, and up into the SVC. The subcutaneous puncture site was enlarged by about 10 mm with an incision and an approximately 20-mm-sized subcutaneous pocket was created by tissue dissection. Next, the introducer system (dilator and sheath) was inserted along the guide wire into the SVC under fluoroscopic guidance. After the dilator and guide wire were removed, iodinate contrast agent was injected to obtain a superior vena cavogram to clarify the exact location of the thrombus and to confirm the optimal landing zone for a filter in the SVC. Next, a filter unit was introduced into the SVC via the sheath. By pushing and pulling the tethered catheter, the filter was deployed correctly in the supra-azygos SVC. After checking the position of the filter, the anchoring device was attached to the tethering catheter just around right femoral vein, and the excess length of the catheter was sectioned immediately above the olive shaped button. After burying the anchoring device into the subcutaneous pocket, the incision was sutured ().\nAfter placement of the Tempofilter II in the SVC, thrombus aspiration was performed through left basilic vein with a 100 cm length 8-Fr guiding catheter (Guider SofTip; Boston Scientific, Natick, MA). Thrombus aspiration via a catheter was performed with a 20 cc syringe. During the thrombus aspiration, anticoagulation or thrombolysis was not performed. A follow-up venography performed after thrombus aspiration showed recanalization of the left brachiocephalic vein, regression of collateral veins (), and didn't show any thrombus capture within the unit. A CT angiography obtained two weeks after Tempofilter II placement demonstrated a patent SVC with no thrombus around the filter, as well as resolution of the pulmonary embolism in the right pulmonary artery. Moreover, no additional anticoagulation or thrombolysis was noted. Just one day after a follow up CT angiography, we successfully removed the filter without complication. Retrieval of the filter was achieved by making a skin incision around the palpable anchoring device under local anesthesia. The tethered catheter and filter were removed by simply pulling the tethered catheter outward.
A 71-year-old man was admitted to our hospital with chief complaints of abdominal pain and vomiting.\nA 71-year-old man was on treatment for chronic hepatitis B for 22 years when he was diagnosed with HCC. He was noted to have tumor nodules of size 20 mm in diameter, located in segment 8, on a follow-up abdominal computed tomography (CT) (Figure ) and had undergone partial liver resection 7 years previously. Based on the 8th Union for International Cancer Control classification of HCC, the tumor was classified as pT1N0M0 stage 1. Seven months after the first surgery, abdominal CT revealed recurrent HCC with nodules 10 mm in diameter, in segment 6 of the liver (Figure ). When laparotomy was performed, a peritoneal mass was found that was not apparent preoperatively; therefore, partial liver resection and peritoneal tumor resection were performed (Figure and ). The peritoneal tumor with peritoneal metastasis of HCC was diagnosed based on histopathological findings. Fourteen months after the first surgery, abdominal CT revealed a tumor nodule 32 mm in diameter in the pelvis, which was diagnosed as a peritoneal recurrence of HCC (Figure ). We determined that the recurrent tumor was solitary and decided to perform tumor resection. However, many small peritoneal nodules were found at the time of laparotomy, and radical resection was impossible (Figure ). Subsequently, the patient was followed up by the department of gastroenterology of our hospital, and 16 mo after the initial resection of HCC, administration of sorafenib of 400 mg/d was started. Since he developed a grade 2 hand-foot syndrome, the dosage was reduced to 200 mg/d. The administration was continued for 54 mo without any other major adverse events, and the disease was well controlled. Seventy months after the first surgery, he was admitted to our hospital with chief complaints of abdominal pain and vomiting.\nThe patient’s history was significant for extensive gastrectomy for duodenal ulcer at the age of 22 years. In addition, he had a history of hypertension since the age of 65, for which he was on treatment with amlodipine besilate (10 mg/d) and azilsartan (20 mg/d).\nThe patient’s social history consisted of a 40-pack year history and an alcohol intake of 350 mL beer per day. He had discontinued smoking and drinking alcohol 10 years previously. There was no history of cancer or liver disease in his family.\nThe height and weight of the patient at admission were 172 cm and 52 kg, respectively. There were no abnormalities in the vital signs. The abdomen was soft and slightly swollen. Tenderness was noted in the right lower abdomen.\nNo abnormal findings were found other than a high C-reactive protein level (4.95 mg/dL) in blood biochemical tests. Liver function tests revealed a class A Child-Pugh score.\nAbdominal contrast-enhanced CT revealed a well-defined, rounded, enhancing endoluminal tumor in the small intestine, leading to intussusception and small bowel obstruction (Figure ). An ileus tube was inserted to decompress the small intestine.
A Caucasian 6-year-old boy with no relevant medical history went to the emergency department after suffering trauma in his left knee after fell off his bicycle. He complained of pain in the left knee and clinical examination showed functional disability of the joint and hematic joint effusion. In the performed X-ray (), no osteoarticular injury was diagnosed and a pattern of massive joint effusion was found. Arthrocentesis with drainage of hematic fluid was performed and the joint was immobilized with cast for conservative treatment.\nAfter removing the immobilization, the initial evolution was positive with no pain on mobilization or joint effusion.\nA few months later, he went again to the emergency department due to new left knee trauma after pivot-type rotation and worsening of the pain. He admitted that symptoms had become persistent after the first knee trauma and he had complained of pain and some limitation on knee extension. On examination, he had pain in the mobilization of the left Knee, especially in knee extension which was not entire.\nThe performed X-ray revealed avulsion fracture of the anterior tibial spines (). The fracture was classified as modified Meyers and Mckeever Type II. Taking into account, the age of the patient and the apparent positioning of the bone fragment in relation to the bed led to the decision of conservative treatment with cast immobilization with weekly follow-up and serial imaging control. In X-ray performed () 6 weeks later, an evolution to non-union of the anterior tibial spine was noticed. Besides, joint instability was detected by Lachman test and pivot shift test. The magnetic resonance imaging (MRI) of the knee () confirmed the non-union of the avulsion fracture and excluded lesion of the ACL or the meniscus. A surgical treatment with reinsertion of the tibial spines was decided. At arthroscopy, non-union of tibial spines was confirmed, with elephant paw bone fragment with loss of its right to domicile. Based on these findings, conversion to open surgery was chosen for bed reshaping and anatomical reinsertion of the bone fragment in view of adequate ACL tension.\nThe patient was prepared to open reduction. A paramedian arthrotomy was performed. The fracture was identified and debridement, cruentation, and fixation of the bone fragment to the tibia with a 3 cm screw were done (). The patient had favorable post-operative evolution with fracture consolidation seen on X-ray (), preserved mobility (symmetrical to contralateral), no instability of the joint, and no limitation in daily and school activities. Four years after the initial surgery, a second procedure was performed for the removal of the screw ().\nAlthough the screw had penetrated the physis (), there was no repercussion in the growth of the affected limb as can be seen in the serial extra long X-ray performed on follow-up (). Besides, the MRI of knee performed 6 years after the surgery showed consolidation of the fracture and integrity of the anterior crucial ligament ().
A 57-year-old Hispanic female presented with altered mental status, fever, and headache of 3 days' duration. Prior to that, patient was in her normal state of health; patient was able to ambulate on her own and able to perform all activities of daily living independently. The patient retuned from Mexico 3 days ago when she became ill and started experiencing her symptoms. She points to the back of her head and neck area when asked to locate her pain. The patient is in obvious distress due to the pain. The family notes that, when she is febrile, her mental status worsens. At the time of evaluation, she was oriented to person but not to time or place. She was lethargic. She denied any numbness, dizziness, weakness, neither seizures, nor changes in vision. She denied exposure to sick contacts, rhinorrhea, and itchy eyes and denied any mosquito bites.\nOn neurologic exam, she was oriented to person but not to time or place. Patient was able to walk with no abnormalities in her gait. She was able to move all extremities with no sensory deficits. Cranial nerves 2–12 were formally tested and were intact. No visual defects were present. There was no pain present on knee extension when the patient's hip was flexed at 90 degrees. There was no flexion of the hips or knees present when the patient's head was lifted while she was lying supine. CT brain done on arrival revealed no evidence of acute intracranial pathology. MRI of the brain was performed and it revealed no evidence of acute infarct or any other intracranial pathology as demonstrated on .\nA lumbar puncture was performed. CSF studies showed a white blood cell count of 127 with 85% lymphocytes, 6 red blood cells, protein count of 177, and glucose of 54. Coccidioides antibody, Cryptococcus antigen, and VDRL were all negative. West Nile antibodies in the CSF were detected via ELISA and the results were as follows: IgM 6.10 (normal < 0.89) and IgG 2.08 (normal < 1.29).\nThe patient was admitted and provided with supportive treatment. Her neurologic status slowly improved. Patient had EEG monitoring, which did not reveal any seizures. On the 16th day of her hospitalization, the patient's clinical picture worsened. Per nursing staff, patient had a facial droop and left sided weakness. On examination by the physician, facial droop was not noticed. She was following commands but quite lethargic. Patient was able to move both hands and able to move the toes on both feet. MRI was performed, which showed moderate periventricular white matter changes. This is demonstrated in . The patient's clinical picture subsequently improved with continued supportive treatment. On the day of discharge, she was alert and oriented to person, place, and time. She was able to walk without any issues. She was able to follow commands and appropriately answer questions. She was subsequently discharged home.
A 51-year-old man reported to the department with a complaint of soft tissue growth in the mandibular left second and third molar region since about 15 days. The growth was small when he first noticed it and was associated with mobility of mandibular left third molar. He showed to a local dentist who extracted the tooth with excision of the mass. No histopathological examination was conducted on the excised tissue mass. Post extraction, the growth rapidly increased in size to reach the current size. Past medical history was insignificant. The patient also did not present with any habit of smoking and tobacco or alcohol consumption. General physical examination was conducted which revealed an otherwise healthy individual with a short and thin built, normal gait, and no history of any fever, headache, or weight loss in the recent past. The left submandibular lymph nodes were enlarged, tender, and fixed to the underlying tissues.\nIntraoral examination revealed a 2.5 cm × 2 cm irregularly shaped, reddish-white, lobular, soft gingival mass on the left mandibular alveolar ridge in the region of mandibular left third molar which was tender on palpation and occasionally bled. There was no ulceration or surface erosion (). A detailed hard tissue examination revealed a poor dental hygiene with multiple root stumps and decayed teeth. Routine hematological tests were conducted which were within the normal range except ESR which was elevated. Panoramic radiograph revealed a well-defined arc-shaped osteolytic lesion with noncorticated borders extending from the distal aspect of mandibular left first molar to anterior border of ascending ramus.\nThe gingival growth was biopsied under local anesthesia, the findings of which were suggestive of proximal variant of epithelioid sarcoma (ES) (Figures –). Immunohistochemistry performed on the tissue showed diffuse strong cytoplasmic positivity for pancytokeratin and vimentin (Figures and ). EMA was strongly positive with membranous staining of the tumor cells in >75% of the tumor cell population (). All other markers of S100 (), desmin, CD45, CD31, and CD34 () were negative for the tumor cells.\nTo rule out the possibility of a primary elsewhere in the body, advanced imaging modalities like ultrasound abdomen, contrast enhanced CT (CECT) of head and neck region, and positron emission tomography (PET) scan were carried out, all of which revealed the gingival growth to be the primary lesion (Figures and ). A surgical approach to management was considered as appropriate which included supraomohyoid neck dissection (levels IA, IB, IIA, IIB, and III), excision of submandibular gland, and tail of parotid gland under general anesthesia. Mandible was exposed and resected till mandibular left premolar region along with the soft tissue growth with 1.5 cm safe margin. A recon plate was admitted and fixed using three 2.5 × 10 mm screws. Negative margins were confirmed using the frozen section. Following the surgery, the patient was subjected to chemotherapy.\nHistopathologic examination following excision revealed overlying parakeratinized stratified squamous epithelium at either end with ulceration and discontinuity at the centre (Figures and ). The ulcerated area showed abundant epithelioid cells in loosely held stroma showing highly dysplastic features of pleomorphism, altered nucleocytoplasmic ratio, and atypical mitoses (). At areas, the adjacent epithelium was showing dysplastic features with invasion of these cells into the stroma along with the epithelioid cells. This was associated with a dense chronic inflammatory cell infiltrate. The epithelioid cells were highly undifferentiated and admixed with few spindle-shaped cells with mitotic figures (). Abundant rhabdoid cells with typical eccentric nuclei and cytoplasmic inclusions were seen scattered throughout. Invasion of the tumor cells into blood vessels and underlying muscle was also seen. The final diagnosis for the excisional tissue was determined as epithelioid variant of SC since the epithelioid component predominated over the spindle cells. The dilemma in diagnosis was resolved because the invasion of the overlying atypical epithelial cells into the stroma was clearly evident in the final tissue received and hence confirming the epithelial origin of the tumor.
A 65-year-old woman presented to the orthopaedic office on a wheelchair. The patient had suffered an O5 fracture 35 years ago which was treated conservatively, and produced a cauda equine syndrome. The symptoms of urinary incontinence, diminished sensation in both legs distally to the middle of both thighs and deep tendon reflexes were remaining. Furthermore, she presented with muscular weakness (2+/5) of both feet, and she used feet orthoses. The quadriceps strength was uneventful. The patient had no notable medical history concerning diabetes mellitus, syphilis, lacunar infarcts, rheumatologic disease and osteoarthritis, and she denied tobacco and alcohol use. She had suffered a right middle tibia fracture 5 years ago, which was treated with intramedullary nailing and fused uneventfully. After a period of 2 months she regained her prior walking ability and was able to walk using a walker. The last 2 years she gradually lost her walking ability due to gross instability of both knees.\nThe main complaints were progressively swollen knees with mild pain and increasingly instability with valgus deformity and crepitus for the last 2 years. Since then the patient was unable to weight bear due to gross instability, and she used a wheelchair. During her admission to the orthopaedic office both knees had similar clinical deterioration and radiological abnormalities. Gross abnormal mobility was present on clinical examination in both sagittal and coronal planes. Anterior and posterior drawer test were positive. Varus and valgus instability test were also positive.\nThe diagnosis of Charcot arthropathy was established using knee X-rays, which showed marked destructive changes, sclerosis, dislocation, and subchondral fractures of the knee joints (, ).\nDue to the progressive inability to ambulate the patient decided to be treated with total knee arthroplasty (TKA). She underwent removal of the intramedullary nail first. Two months later she was operated on her right knee using cemented rotating hinged knee prosthesis, in order to accommodate bone loss and ligamentous insufficiency. On postoperative day 2, the patient could partially weight bear. Wound healing was uneventful, and on postoperative day 20 knee range of motion (ROM) was 115°/0°/0° (flexion/extension/hyperextension). Knee stability was restored, and she was allowed to weight bear. Three months later the patient underwent TKA at her left knee using identical technique. Postoperative course was uncomplicated, and on day 20, ROM was 120°/0°/0°. Six weeks later she was able to walk with the aid of a walker and was satisfied with the result.\nWe assessed the clinical outcome using the Knee Society Score (KSS) [], which was calculated preoperatively and at the final evaluation 12 years postoperatively. There was an improvement from 25 to 90 points in knee score and from 0 to 30 points in function score, although she was continued to use walker due to her muscular weakness of both feet. Radiological evaluation also took place at the final follow-up, using radiographs. The position of components was evaluated for proper placement and loosening, with no apparent sings of malposition and progressive loose lines ().
A 29-year-old multi-gravida was referred to our hospital with an antenatal diagnosis of a large cystic hygroma diagnosed at 38 weeks of gestation. Fetal MRI revealed a large multiseptated cystic mass (measuring 12 × 8 cm) involving both sides of the neck with compression over the trachea []. Hence a difficulty in securing the airway was anticipated and EXIT procedure was planned. The parents were counseled and informed written consent was obtained. A multispecialty team comprising of obstetricians, anesthesiologists, pediatric surgeons, neonatologists, and radiologists were involved in planning and executing the procedure. Under general anesthesia elective Cesarean section was performed. Two teams of anesthesiologists were present, one for the mother and the other for the fetus. Apart from the routine monitoring, radial artery cannulation was done for invasive blood pressure monitoring. Patient was positioned in the supine position with left lateral tilt to prevent compression on the aorta. The legs were slightly abducted in order to allow easy access to the fetus for intubation. General anesthesia was commenced and maintained with higher concentration of isoflurane (for complete uterine relaxation), oxygen, nitrous oxide, and muscle relaxants. Before skin incision, ultrasound was done to locate the placental edge which revealed two loops of umbilical cord around the fetal neck. After skin and uterine incision, the fetal head and neck with the mass was delivered []. Warm Ringer lactate was infused into the uterine cavity to maintain the uterine volume. Fetal heart rate was monitored with an ultrasound probe and manually by umbilical cord pulsation. Initial attempts at intubation failed. Ultrasound-guided aspiration of the cyst was done but since most of the cysts were microcystic, adequate decompression could not be obtained. The neck was delivered up to the sternal notch [] and then intubated. Intubation could be accomplished in 3 minutes and 47 seconds and airway was secured with a 3-mm ID oral endotracheal tube and then ventilated with 100% oxygen. There was no fetal bradycardia and cord was clamped and handed over to the neonatologist for further resuscitation. Isoflurane was stopped and oxytocin 20 units in 500 ml Ringer Lactate solution was administered. Hemostasis was achieved and patient was extubated at the end of surgery. The neonate was taken up for definitive surgery after 4 hours of stabilization in the NICU. The lesion was excised and elective tracheostomy was done due to tracheomalacia. Second sitting of excision was done after 4 months and tracheostomy has been decannulated.
A 55-year-old man with a previous diagnosis of a gastric SMT visited our hospital 10 years after the identification of a gastric SMT that was detected in the posterior wall of the upper gastric body during a medical check-up (). The tumor had not been followed up in the intervening 10 years because during that time he had developed myocardial infarction and chronic heart failure. However, 10 years after the original diagnosis, a follow-up gastroscopy in another hospital revealed an enlarged SMT with a rugged surface. An endoscopic fine-needle aspiration biopsy had been obtained, but the SMT could not be histologically identified. Therefore, he was referred to our hospital for further investigation.\nGastroscopy at our hospital revealed a whitish elevated lesion in the posterior wall of the upper gastric body (). No atrophic changes were evident. A histopathological examination of further biopsy specimens showed adenocarcinoma mimicking fundic glands and the tumor was diagnosed as a GA-FG. An endoscopic ultrasound study revealed a lowly echoic tumor and a thickened muscular layer consistent with a T2 invasion depth according to the JCGC 15th edition []. No enlarged LNs or other evidence of distant metastases were detected on a computed tomography scan.\nThe patient underwent a total gastrectomy with D2 lymphadenectomy. An intra-abdominal abscess developed postoperatively but resolved completely with percutaneous drainage.\nGrossly, the tumor was a locally thickened lesion with indistinct borders measuring 4.7 cm in its largest dimension (). The gastric folds surrounding the lesion were slightly thickened.\nOn low-power magnification, the submucosal layer was also thickened and the mucosa pushed up into the gastric cavity (). Most of the tumor cells in the mucosal and shallow submucosal layers resembled mucous neck or chief cells with some intermingling eosinophilic cells resembling parietal cells (). These cells formed densely aggregated glands with anastomoses. The tumor glands in the middle of the submucosal layer gradually transitioned into irregularly shaped tubular glands with surrounding desmoplastic reactions and lymph follicles (). Some of these glands contained intraluminal necrotic debris (). In the muscular and subserosal layers, the tumor cells formed predominantly isolated tubular glands or small clusters that diffusely and widely infiltrated these layers (). Neural and venous invasion was detected within these layers () and lymphatic invasion was identified at the tumor's periphery. Among the 37 dissected LNs, a metastasis was identified at station No. 3a ().\nImmunohistochemically, the mucous neck and chief cell-like cells in the mucosal and submucosal layers were widely positive for pepsin A; additionally, about half of these cells co-expressed MUC6 (). The cells that resembled parietal cells were positive for H+/K+-ATPase but consistently negative for pepsin A and MUC6 (). MUC5AC positivity was detected in only a few cancer cells (). In contrast, the cancer cells in the muscular and subserosal layers were strongly and diffusely positive for MUC5AC and partially positive for MUC6 (). Only a few cells in these layers were positive for pepsin A or H+/K+-ATPase (). No aberrant expression of TP53 was identified. An attempt was made to identify the LN metastasis; however, no cancer cells were identified in the additional sections because the focus was very small.\nThe Ki-67 labeling index of the tumor was approximately 1% within the tumor in the submucosa but as high as 5% within the tumor in the muscular and subserosal layers (). These findings suggest that the GA-FG component in the mucosa and submucosa had transformed, forming an infiltrative tubular adenocarcinoma with foveolar epithelial differentiation that subsequently metastasized to a LN. Importantly, there was an obvious transition area between these two components in the submucosa, ruling out the possibility that the tumor was a collision cancer.\nThe pathological diagnosis was T3N1 according to JCGC criteria []. Nine weeks post-gastrectomy, the patient was started on adjuvant chemotherapy using S-1 alone. At the last follow-up 18 months later, there was no evidence of cancer relapse.
A 71-year-old man with a history of FXI deficiency presented to the orthopedic clinic with complaints of progressively worsening pain in his right hip. Of note, the patient had previously undergone cephalomedullary nail fixation of a right proximal femur fracture 3 years prior by another local orthopedic surgeon. The patient subsequently went onto heal this fracture; however, he did develop severe degenerative changes in his right hip along with significant post-traumatic arthritis (). Surgical options along with their risks and benefits were reviewed with the patient who ultimately elected to undergo conversion THA.\nOf note, the patient had a history of significant post-operative bleeding following a right-sided hernia repair done 19 years prior. At that time, his FXI deficiency was treated with only a single dose of fresh frozen plasma (FFP). Since then, he had undergone cephalomedullary nail fixation of both his left and right hips 3 and 5 years prior, respectively. He did not experience any post-operative bleeding or thromboembolic complications following either hip fracture surgery. The patient’s hematologist devised the following perioperative management strategy before his first hip fracture surgery: Preoperatively, a 5-g IV bolus of aminocaproic acid would be administered followed by aminocaproic acid as continuous venous infusion of 5 g in a 250 mL solution at 50 mL/h throughout the perioperative period. Postoperatively, tranexamic acid (TXA) would be administered as a 1 g bolus followed by 1 g every 8 h for 72 h for a total of nine doses. The patient would then be discharged on oral TXA at 1300 mg every 8 h for the first 7 days, with the frequency decreased to every 12 h for the subsequent week. He would also be administered subcutaneous enoxaparin 40 mg daily for venous thromboembolism prophylaxis.\nBefore conversion THA surgery, the patient was seen in the hematology clinic so that his perioperative plan could be reviewed before this surgery. The patient had been previously seen in this clinic at the time of this right hip fracture. At that time, the patient’s FXI deficiency was confirmed, with his factor XI assay at 1.9%, well below the normal value of 50–150% activity. Given the patient’s success with the aforementioned hematologic plan, it was agreed that the same protocol would be used for his THA conversion surgery.\nThe patient was taken to the operating room for removal of the cephalomedullary nail and subsequent conversion to a THA. Pre-operative hemoglobin and hematocrit were 12.4 g/dL and 35.6%, respectively. Using the previous surgical incision, removal of the cephalomedullary nail was completed with some difficulty as the distal locking screw was stripped at the time of removal. Once this was completed, attention was turned to completing the THA. Utilizing a standard anterior approach, the proximal femur was cut in appropriate position, the acetabulum was reamed up to a size 57, and a size 58 cup was press-fit with excellent purchase. The cup was fixed with two screws into the ilium. Next, the femur was prepared. An 8”, size 18 Solution (Depuy, Warsaw Indiana) stem with a 36 + 5 head was deemed to provide adequate stability, and the final components were implanted. Blood loss was estimated at 700 mL. The patient was made toe-touch weight-bearing to the right lower extremity. There were no intraoperative complications experienced.\nThe patient was placed on the pre-established protocol during and after the surgery. He was cleared by physical therapy on post-operative day 1, and plans were made for discharge to home with a rolling walker. The patient was observed closely for signs of bleeding, and he remained asymptomatic throughout his hospital course. The patient’s hemoglobin and hematocrit did decrease to 8.0 g/dL and 24.6% before stabilizing, and no transfusion was necessary. Ultimately, the patient was discharged to home in stable condition on post-operative day 3, shortly after finishing nine doses of IV TXA per the previously established protocol.\nFollow-up was obtained at 2 weeks, 2 months, 5 months, and 10 months, with post-operative radiographs obtained at 2 weeks and 2 months (). The patient continued to progress to weight-bearing as tolerated without issue. Follow-up X-rays demonstrated stable and well-aligned components. His pre-operative pain improved dramatically. Despite some mild stiffness and a small leg length discrepancy, the patient’s overall clinical status improved significantly and he remains very satisfied with the outcome of his surgery. He did not have any bleeding-related complications throughout his perioperative course. At 10 months, the patient reported no complications, and he noted that he was back to full activity including hiking and snowshoeing without pain or other noticeable symptoms.
A 16-year-old boy presented to our outpatient clinic with upper right quadrant pain. He explained that he had presented to our hospital's pediatric surgery clinic with skin redness, impaired general status, and cyanosis three years earlier, when a perforated AHC was diagnosed in radiological studies and treated surgically. Upon review of his previous medical reports, it was noted that the contents of the cyst in the right adrenal gland were removed, followed by the resection part of the cyst's anterior wall and the irrigation of the abdominal space with 10% polyvinylpyrrolidone iodine solution. He stated that he had always resided at a city center and had no animal contact ever before. On physical examination, no abnormality was noted apart from a right-sided subcostal incision. His hemogram and liver and kidney function tests were within normal limits. His serological tests revealed an ELISA IgG: 15.66 (positive). A contrast-enhanced computed tomography showed a lesion with lobulated contours and a size of 70 × 70 mm that was compatible with a hydatid cyst. The lesion originated from the hepatic segment V-VI and gave off exophytic extensions in the posterior-inferior direction, and it contained hypodense areas (daughter vesicles) (Figures –). The patient was operated on after a 2-week prophylactic albendazole treatment at a dose of 400 mg b.i.d. The old subcostal incision was used to access the abdominal cavity. It was noted during the exploration that the cystic lesion that reportedly located in the liver actually originated from the right adrenal gland and externally compressed the inferior vena cava. After dissecting adhesions between the right lobe of the liver and the cystic lesion, the location of the cystic lesion and its right adrenal origin were revealed. Since dense adhesions between the cyst and surrounding tissues did not permit excision of the cyst in isolation and since the disease had recurred, the cystic lesion was excised en bloc with the right adrenal gland, without its integrity being disrupted. The patient was then discharged without any problem. Albendazole was not postoperatively administered due to total excision of the lesion. No recurrence was observed at one-year follow-up.
A one month old baby girl was brought by her mother to the lactation clinic for further evaluation due to the difficulty of breastfeeding. The mother complained of pain in the nipple which was present throughout the entire breastfeeding session and usually recurred with each episode of breastfeeding. Two weeks after delivery, the mother noticed a tooth on the baby’s lower left gum. A week later, she noticed an ulcer under her baby’s tongue, which occasionally bled.\nIn light of the continuous pain during breastfeeding, the mother became reluctant to continue and infant formula milk was given as a substitute. Self-examination by the mother did not reveal any nipple crack or breast injury. Her antenatal history was uneventful. There was strong family history of natal teeth, and this baby’s siblings had similar problems.\nOn examination, the mother’s breast appeared to be normal and consistent with a lactating breast. There was no inflammation or engorgement. Some mild eczema was noted around the nipple, but there was no infection. Cervical and axillary lymph nodes were not palpable.\nExamination of the oral cavity of the baby revealed a neonatal tooth over the left anterior region of the mandibular ridge. The tooth measured 2 mm × 1 mm in size, was whitish opaque, and had Grade II mobility (Fig. ). There was also a whitish ulcer over the ventral aspect of the tongue measuring 1 mm × 1 mm (Fig. ). The parents declined radiological investigations and tongue biopsy for the baby. Hence, the clinical diagnosis was neonatal tooth associated with Riga-Fede disease causing difficulty in breastfeeding.\nThe baby was then referred to the pediatric dental surgery department. After a thorough discussion with the parents, the neonatal tooth was extracted under local anesthesia. The tooth had a crown but lacked a root. Following extraction, the baby did not have any complication such as bleeding and infection. The wound healed well within 2 days and she successfully resumed taking breast feeds.\nCurrently, at 10 months post-extraction, the child is growing well and still breastfeeding. Examination of the oral cavity revealed that only one central lower incisor tooth was present (Fig. ).
A healthy 51-year-old man presented to the emergency department with progressively worsening left shoulder pain of four-month duration that had been acutely exacerbated three days earlier by a ground-level fall. The patient reported that he first noticed a dull ache when playing golf. Radiographs obtained in the emergency department showed moderate degenerative changes but no acute abnormalities. A diagnosis of rotator cuff tendinitis was established. The patient was subsequently provided with an arm sling and discharged for outpatient follow-up.\nThe patient was evaluated in clinic approximately three weeks after his initial presentation in the emergency department. He reported minimal pain relief despite daily use of the arm sling and oral naproxen sodium. Physical examination revealed significant tenderness to palpation over the left glenohumeral joint. Range of motion of the left shoulder was restricted with abduction and extension. The Hawkins-Kennedy test and external rotation lag sign were positive. A presumptive diagnosis of rotator cuff tendinopathy vs tear was established. The patient was referred for magnetic resonance imaging (MRI) and outpatient physical therapy. However, a trial of physical therapy was required for health insurance authorization of imaging; the first session was scheduled for one week after the clinic visit.\nThe patient began physical therapy as planned. However, three days after his first session, he presented to the emergency department complaining of testicular swelling. An ultrasound was performed and revealed hydrocele. The patient was diagnosed with epididymitis and prescribed oral ciprofloxacin. The swelling failed to resolve after a two-week course of antibiotics. He was subsequently evaluated by urology, who recommended continuing ciprofloxacin for eight weeks. Symptoms had partially resolved following two months of treatment.\nThe left shoulder pain failed to improve after three months and twelve physical therapy sessions. An MRI without contrast was ultimately obtained and revealed severe tendinopathy of the supraspinatus tendon as well as a large tumor replacing the marrow of the proximal humerus (Figure ). Extensive adenopathy was also noted. Four days later, the patient was seen by the hematology/oncology team; providers suspected osteosarcoma or chondrosarcoma and ordered an urgent lymph node biopsy. Computed tomography (CT)-guided biopsy of a left axillary lymph node showed a B-cell lymphoma with high-grade features. The immunophenotype supported an activated B-cell (ABC, nongerminal center) subtype. Immunohistochemical analysis demonstrated diffuse positivity of CD20, PAX5, and Ki-67; focal positivity of Bcl-6; and negative CD10, CD30, and Bcl-1 markers (Figure ). The CD21 immunohistochemical stain highlighted dendritic cell meshworks associated with follicular structures. cMYC stained less than 40% of cells, and therefore, the lymphoma was not a double expresser. The fluorescence in situ hybridization (FISH) panel was negative for a double/triple-hit lymphoma. A definitive diagnosis of ABC-type DLBCL was therefore established.\nCT scans of the neck, chest, abdomen, and pelvis were subsequently acquired. Notable findings included splenomegaly, an enlarged right submandibular lymph node (Figure A), a lytic lesion in the C3 vertebral body, and left hydronephrosis secondary to distal urethral obstruction by retroperitoneal lymphadenopathy (Figure A). MRI of the brain was normal, and cerebrospinal fluid analysis showed no malignant cells. Repeat scrotal ultrasound showed moderate right testicular hydrocele as well as left varicocele. Correlation of the CT and ultrasound findings indicated that the scrotal swelling represented secondary varicocele due to the retroperitoneal lymphadenopathy; therefore, testicular radiation or surgery was not pursued. The patient was started on rituximab (375 mg/m2), cyclophosphamide (750 mg/m2), doxorubicin (50 mg/m2), vincristine (1.4 mg/m2), and prednisone (R-CHOP) every three weeks. There were no risk factors for central nervous system disease, as testicular involvement was excluded, and thus, central nervous system-directed prophylaxis was not administered.\nThe patient tolerated the therapy well. After the first cycle of R-CHOP, the patient reported that his shoulder pain was modestly improved and that his scrotal swelling had resolved. Repeat CT scans were obtained after ten weeks and three cycles of R-CHOP. Lymphadenopathy was markedly improved in the submandibular (Figure B) and retroperitoneal region (Figure B). The patient continued the R-CHOP regimen for another ten weeks, completing a total of six cycles. A positron emission tomography-computed tomography (PET-CT) scan of the skull base to mid-thigh was obtained after the last cycle of R-CHOP and revealed fluorodeoxyglucose (FDG) activity of the retroperitoneal lymph nodes and left humeral lesion; imaging was otherwise normal (Figure ). Notably, the retroperitoneal lymph nodes were decreased in size as compared to the previous CT scan. The patient subsequently began radiation therapy to the left shoulder at a dose rate of 250 centigrays (cGy) with a final external beam dose of 3750 cGy. At his most recent follow-up, the patient had returned to work and complained only of mild, intermittent left shoulder pain.
A 19-year-old woman who presented to the orthopedics outpatient clinic with weakness and atrophy in her right hand was diagnosed with advanced stage carpal tunnel syndrome and recommended surgery. She was then referred to the electrophysiology laboratory of the hospital’s neurology department for electroneuromyography (ENMG). She stated that her symptoms had begun 2 years ago; she occasionally had numbness and chilling sensation in her right arm; and she had long been unable to carry anything in her right hand. Her past and family history was unremarkable. Her physical examination was also normal except for neurological examination which revealed atrophic appearance of the right hand ( and ) and a muscle strength of 4/5 in the distal part of the right arm. It was also noted that the distal interphalangeal joint of the first finger of the right hand had a muscle strength of 1/5 and the proximal one had a muscle strength of 4/5. Muscle strength was 2/5 in the distal and proximal interphalangeal joints of the second finger. Thenar atrophy of the right hand was also found. The wrist circumference measured 5 cm proximal to the wrist was 15 cm on the right side and 16 cm on the left side. The arm circumference measured 10 cm proximal to the elbow was 26 cm on the right side and 26.5 cm on the left side. All biochemical and blood count parameters were within the normal range. Thyroid function tests and creatine kinase levels were also normal. Cranial and cervical vertebral magnetic resonance imaging (MRI) studies performed at an outside center were normal.\nAn ENMG was performed, which demonstrated an absent right median and ulnar nerve compound muscular action potential (CMAP) at the motor conduction study. The sensory conduction study revealed normal sensory action potential (SAP) conduction velocities and amplitudes in the right median nerve, right ulnar nerve, right radial nerve, right lateral antebrachial cutaneous nerve (right median nerve second finger SAP amplitude 56.6 µV, conduction velocity 63.8 m/s; right median nerve third finger SAP amplitude 34.7 µV, conduction velocity 59.3 m/s; right ulnar nerve fifth finger amplitude 12.1 µV, conduction velocity 56.3 m/s; right radial nerve amplitude 31.2 µV, conduction velocity 59 m/s; right lateral antebrachial cutaneous nerve amplitude 11.1 µV, conduction velocity 66.7 m/s). A needle ENMG recorded very large amplitude single oscillation pattern and diffuse spontaneous denervation potentials (positive sharp waves and fibrillation potentials) in the right first dorsal interosseous muscle, right abductor digiti minimi muscle, and right abductor pollicis brevis muscle. Electrophysiological study of forearm muscles was normal. HD was diagnosed as the definitive diagnosis.
A 30-year-old gravida 2, para 2 woman admitted to our hospital with complaints of headache, nausea, vomiting, and right-sided blurred vision. She did not report any previous medical history or malignancy. Her neurologic examination revealed a right optic disc edema suggesting a posterior orbital mass. Her cranial computerized tomography (CT) scan showed multiple lesions that are a 6 mm mass on the right parietal lobe, a 16 mm mass on the left occipital, and another 7 mm mass on the left temporal lobe (Figures and ). All the lesions were hyperintense and surrounded by edema which suggests a metastatic cancer. Her cranial magnetic resonance imaging (MRI) also confirmed similar findings suggestive of a metastatic cancer to the brain.\nFor identification of the primary malignancy, she initially underwent thoracic and abdominopelvic CT. Upper abdominal CT scan revealed indistinctly bordered and heterogeneously contrast accumulating multiple lesions less than 15 mm in the liver. There was a pelvic tumor of 62 × 53 mm sized and solid cystic in nature located in the right adnexa. The pelvic mass was round shaped and thin capsulated that contains irregular septations and a solid component of 38 × 24 mm on MRI scan (). The right pelvic lymph nodes were slightly enlarged. No ascites were detected in the pelvic cavity. The endoscopic evaluation of the upper and the lower gastrointestinal tract and the ultrasonographic evaluation of the breast was found to be normal. The Pap test was reported to be negative for malignancy. Her serum tumor marker levels (CA 125, CA 15-3, CA 19-9, CEA, and AFP) were within normal limits.\nThe patient underwent a diagnostic laparoscopy for further evaluation of the adnexal mass. Biopsy of the adnexal mass and the liver was obtained and the pathologic examination revealed an ovarian mucinous cystadenocarcinoma with metastatic lesions of liver. The patient then was subjected to a laparotomy for a surgical staging procedure including total abdominal hysterectomy, bilateral salpingooophorectomy, bilateral pelvic and para-aortic lymphadenectomy, and infracolic omentectomy and appendectomy. Her postoperative course was uneventful and she was discharged at the 6th postoperative day. The patient was determined to have a stage IV ovarian carcinoma with brain metastasis. An adjuvant therapy including whole brain irradiation (a total dose of 30 Gy in 10 fractions and 3 Gy per fraction) with simultaneous dexamethasone and systemic chemotherapy (two lines of six cure 400 mg/m2 carboplatin plus 175 mg/m2 paclitaxel with three weeks interval) was administered postoperatively. While documenting the patient, she was still alive 1.5 years after the initial diagnosis.
A 23 years old female patient was consulted to neurology department with left hemifacial atrophy with the involvement of the zygomatic area extending to the left jaw, mouth, and ipsilateral temporo-parietal area (). The patient did not have any other significant medical or traumatic history in the past, and there were no history of dental procedures or infections either. The onset of the manifestations was at the age of eight with hyperpigmentation in the left zygomatic region and left hemifacial atrophy, and it resulted in severe bone, fat tissue and muscle atrophy of the aforementioned regions after a steady progression during the following 2 years. The disease first developed since the patient was at 8 years old. The diagnosis of PRS was made at the age of 8 by a skin biopsy. Until then, the patient had not received any medications related to this.\nIn addition to the left hemifacial atrophy, the patient ( and ) was characterized by atrophy of the ipsilateral upper limb with hypoplasia of the fifth finger of the left hand, left enophthalmos () as well as eyelid retraction without involvement of the eye movements. Additional cutaneous manifestations included linear scleroderma and bandlike alopecia (). Regarding dental manifestations, the patient presented with radicular resorption of dental organs within the left superior hemiarch without headache or arthralgia. The laboratory tests including complete autoimmune panel showed no abnormal results. Since 2006, the patient was performed seven surgeries for multiple muscle and fat grafting.\nThe patient was referred to the neuro-epilepsy clinic for her neurological manifestations, which included seizures that initiated at the age of eighteen. It was characterized by a blank stare, supraversion, and tonic posture with extension of the four limbs, followed by tonic tremor and dystonic posture of the hands, sialorrhea, tongue biting, for about 1–2 minutes, and occasionally postictal confusion. The frequency of seizures was three episodes each month, which did not respond to the first line of anticonvulsants (carbamazepine and valproic acid) for more than 3 months, while adequately controlled by levetiracetam and lacosamide afterwards. The brain magnetic resonance imaging (MRI) revealed focal cortical dysplasia in the left frontal and temporo-polar regions (). The video-electroencephalography (EEG) monitoring for more than 72 hours showed abnormal epileptiform activities in the left fronto-temporo-occpital areas. The cognitive function test was not performed, but the patient reported emotional changes without cognitive dysfunction. The patient eventually diagnosed as major depression.\nThe patient provided her consent to report this case including all of the images in this article.
A 31-year-old gravida 4 para 0 African American woman at 22-weeks gestation presented with vaginal bleeding to an outside hospital. Her obstetric history was significant for 2 therapeutic abortions and 1 spontaneous abortion. During prenatal care, the fetus was noted to have a unilateral dysplastic kidney. The patient’s family history was significant for two family members with cervical cancer and two family members with endometrial cancer. She was placed on bedrest with inpatient admission. 48 h after admission she developed pelvic pain and uterine contractions. She expelled a mass vaginally measuring 11 × 9 × 5 cm with no fetal contents. The pathology from the outside facility showed a showed a highly cellular tumor composed of spindle-shaped cells and bizarre multinucleated giant cells with focal myxomatous change with mitotic count is greater than 50 per 10 high power field (Positive for CD10, SMA, ER, PR, EMA) with the differential diagnosis including endometrial stromal sarcoma and undifferentiated uterine sarcoma. The patient underwent examination under anesthesia, demonstrating a 3 × 3 cm defect of the posterior vaginal wall with active bleeding, which was sutured for hemostasis. The cervix was long, closed, and high with no evidence of bleeding and the fetus was intact with normal heart tones. She was discharged in stable condition.\nAt 27-weeks, the patient was referred to our institution for maternal-fetal medicine, and gynecology oncology consultation. On initial evaluation, she reported no prior abnormal gynecology history and no history of infertility issues. She denied dyspareunia and bulk symptoms prior to or during pregnancy, and her periods before pregnancy were normal. A pap smear at 27-weeks gestation was normal and negative for human papilloma virus. Her intake physical exam at our institution revealed a normal pelvic exam with no evidence of residual vaginal mass, and an ultrasound revealed no intrauterine myomas.\nGiven the differential diagnosis included an endometrial stromal sarcoma, an undifferentiated uterine sarcoma, and a primary vaginal sarcoma, the patient underwent magnetic resonance imaging (MRI) without contrast of the chest, abdomen, and pelvis at 27 weeks. MRI findings were notable for a peri-centimeter cyst in the cervix consistent with a Nabothian cyst, a multiloculated cystic structure in the fetal abdomen consistent with dysplastic kidney, and an anterior placenta. There was no evidence of metastatic disease.\nDue to the inability to ascertain whether the cancer had originated in the uterus, cervix or vagina, the patient desired definitive management for possible uterine sarcoma. In consultation with maternal fetal medicine and gynecology oncology specialists, the decision was made to proceed with a cesarean hysterectomy at 36 weeks. Betamethasone 12 mg intramuscularly was given for two doses 24 h apart with the first dose at 35 weeks and 6 days gestational age.\nAt 36 weeks and 1 day, she underwent planned exam under anesthesia of the vaginal canal with cesarean hysterectomy, bilateral salpingectomy, and peritoneal biopsies. She delivered a male infant weighing 2520 g with Apgars of 9 and 9. Operative findings were notable for normal appearing pelvic organs. There were no palpable or visibly enlarged pelvic/periaortic lymph nodes or evidence of metastatic disease throughout the peritoneal cavity. The neonate was admitted to the NICU post-delivery for prematurity. Neonatal US confirmed a right multi-cystic dysplastic kidney. He was transferred out of NICU on day 2 of life, and discharged home with his mother.\nThe final surgical pathology report was benign: no malignant or neoplastic cells were seen. The patient was seen at her 6-week postpartum visit and was doing well with a normal pelvic exam. At a follow-up surveillance visit 3 months postpartum, she was noted to have a 1 cm posterior vaginal wall lesion; biopsy results showed a high-grade sarcoma. The tumor was estrogen receptor and progesterone receptor positive. Computer tomography (CT) with contrast of the chest, abdomen, and pelvis showed no evidence of metastatic disease. The patient underwent an upper vaginectomy and proctoscopy. Operative findings were notable for 1.5 cm polypoid lesion in the posterior vagina wall 5 cm distal to the posterior vaginal apex. A 1 cm surgical margin was obtained circumferentially around this lesion where feasible, and surgical pathology showed high-grade sarcoma () of the polyp lesion with negative surgical margins. The tissue removed from this excision showed residual sarcoma, morphologically similar to the original pathology from the outside hospital pathology. All other vaginal biopsies were also negative for malignancy. Marker seeds were placed in the proximal and distal margins of the areas where the sarcoma was excised. She was treated adjuvant high dose rate vaginal brachytherapy using a multi-channel vaginal cylinder. She received a biologically equivalent 2 Gy dose (EQD2) of 45 Gy to the whole length of the vagina and 60 Gy to the post-operative bed (). A CT scan 36 months after surgery continue to show no evidence of disease. She remains disease free 58 months after completion of vaginal brachytherapy.
A 60-year-old male patient came with complaint of pain and swelling over the left hip for 30 days. The patient had a history of fall in the bathroom 30 days back sustaining a trivial trauma to the left hip. The patient was weight-bearing for 1 week, after which pain was increased on walking. The patient was a known case of hypertension and diabetic on regular medications. On examination, tenderness was present at left hip region, left lower limb was externally rotated, shortened with no wound and distal neurovascular deficit.\nPlain radiographs of the left hip with femur anteroposterior and lateral views revealed left subtrochanteric fracture with diffuse irregular cortical and trabecular bone thickening from the femoral head to lower third of the left femur (, ). The fracture was a complete transverse through both cortices without comminution at the subtrochanteric area of the femur. Right-sided femur appeared normal. Blood investigations showed increased ALP.\nMagnetic resonance imaging of pelvis with femur showed pathological fracture secondary to Paget’s disease with significantly distorted bone matrix, profound hypertrophy and thickening of the bony trabeculae with significant obliteration of intervening medullary canal ().\nAs the patient was elderly, needed early mobilization and had displaced pathological subtrochanteric fracture, we planned for surgical management with intramedullary nailing. The patient was operated under spinal anaesthesia, using traction table in the supine position. Incision of around 5 cm was taken from the greater trochanter extending proximally. The entry point was made over the tip of greater trochanter after dissecting the soft tissues. The guide was passed in the center of the medullary canal till the distal femur, gradual progressive reaming was done using flexible reamers. Long proximal femoral nail (PFN) was inserted (size 11 mm × 38 cm) with proximal and distal locking (). The intraoperative sample was taken from the medullary cavity and was sent for histopathological examination. The wound was closed in layers.\nThe post-operative X-ray showed acceptable fracture reduction (, ), the patient was mobilized off the bed from the 2nd post-operative day with partial weight-bearing. Histopathological examination confirmed the diagnosis of Paget’s disease with no evidence of malignancy. Full weight-bearing was started at 6 weeks. The patient became completely symptomless by 6 months. Patient was able to wight bear initially with crutches, latter he started walking independently by 6 months with no pain. At present 1-year follow-up, the patient is comfortable with no pain and difficulty in walking and having good hip and knee range of motion. The X-ray showed the union at the fracture site (, ). We are still following up the patient regularly to check for implant failure and refracture.
An 18-year-old man in otherwise good health presented complaining of the right midfoot pain and swelling for 7 months, worsening over the preceding 3 months. He stated that he could walk, but this was associated with pain. A 2 × 3 cm firm, tender mass was palpated on the medial aspect of his right midfoot ().\nHe had full range of motion at his ankle and limited inversion and eversion secondary to pain. A roentgenogram () showed an expansile, lytic lesion in the navicular bone. Magnetic resonance imaging (-) shows altered signal intensity over navicular bone of size 2.1 × 2.8 cm with mild edema and infiltration over surrounding soft tissues.\nNo other associated lesions were seen. Under strict aseptic precautions, biopsy was taken, it was a thick column of blood aspirate, and results () were consistent with ABC. In view of high recurrence rate after curettage of ABC, the primary procedure of intralesional sclerotherapy was planned. Under the aseptic condition, hypodermic needle inserted into the lesion with the help of image intensifier, aspirated to confirm the site followed by intralesional injection of polidocanol, a sclerotherapy agent at a dosage of 2 mg/kg, with the patient being 60 kg a total of 120 mg, was given. It was performed as a daycare procedure and carried out under local anesthesia. Early rehabilitation was performed with no mode of immobilization. Four such doses were given periodically at an interval of 1 month under aseptic precautions using the same aspiration and injection technique. The response was regularly observed with roentgenograms in each visit. The outcome seems to be markedly appreciable since the first injection and at the end of all the four injections, lesion found to be remitted completely and the patient improved greatly in view of symptoms. No complications were reported. At 3 months post-therapy (, ), roentgenogram was done and the patient was able to walk without pain and range of movements was full and painless in ankle and subtalar joint. The patient is symptom-free and shows no signs of recurrence at 1-year follow-up ().
A 55-year-old female presented for a laparotomy with abdominal exploration, small bowel resection, and lysis of adhesions secondary to sclerosing mesenteritis. The patient had a history of chronic pain syndrome. In 1997 she had an intrathecal drug delivery device implanted to treat refractory chronic back pain in the context of four prior lumbar laminectomies and fusion at an outside medical facility. Her chronic abdominal pain began in 2009 after a small bowel obstruction required an exploratory laparotomy, at which time fibrotic strictures were found which required small bowel resection at the ileum. The patient's pain continued and she went on to undergo cholecystectomy in 2010. In 2011 she underwent evaluation by a local gastroenterologist, with upper endoscopy revealing signs of bile reflux gastritis and retained food. Concomitant motility testing revealed gastroparesis and she was ultimately diagnosed with narcotic-related gastroparesis. In an attempt to decrease the amount of opioids she was receiving from her intrathecal device, she had a spinal cord stimulator (SCS) placed as a therapeutic adjunct. Her pain was marginally improved with the stimulator, though this device ultimately failed and stopped working. Despite the SCS failure, the patient elected not to have the stimulator explanted. Throughout this time course, the patient was also treated concurrently with multiple medication regimens including gabapentin, long acting morphine, fluoxetine, and benzodiazepines.\nIn 2013, due to continued abdominal pain and increasing intrathecal opioid requirements, a repeat CT scan of the abdomen showed enhancement of the terminal ileum and rectosigmoid colon with a new mass-like area of mesenteric inflammation. The diagnosis of sclerosing mesenteritis was considered and she presented to our institution for further evaluation and management.\nDuring preoperative evaluation, the patient stated her abdominal pain was constant, 24 hours a day, seven days a week. Utilizing the Agency for Healthcare Research and Quality's numeric pain intensity scale (NPIS) her pain was always an eight out of ten. Additionally, it was exacerbated with food, alcohol, the cold, and stress. She noted that she went to sleep and awoke with the same level of pain. Her current medication regimen included trazadone 200 mg orally at night and probiotics. The intrathecal drug delivery system (IDDS) included fentanyl (25,000 mcg/mL) and Bupivacaine (15 mg/mL), infusing at continuous infusion doses of 6000 mcg and 8 mg per day, respectively. Additionally, there were 11 pre-set boluses for fentanyl at 1200 mcg each (given over three-minute durations) per day. In summation, the patient was receiving a total daily dose of 19,702 mcg of fentanyl intrathecally when accounting for both continuous infusion and bolus dosing; this correlates with an oral morphine equivalent (OME) of 591 grams per day. This amount was calculated using a 100 : 1 conversion between intravenous and intrathecal fentanyl, a 100 greater fold potency of fentanyl, and a 3 : 1 conversion between oral and intravenous morphine.\nPreoperatively, regional analgesia with an epidural was extensively discussed with the patient; however given her multiple back surgeries and chronic back pain, she declined. As previously mentioned, the patient was treated with gabapentin in the past without any benefit and, as such, she declined preoperative administration. Her operative course was unremarkable. Anesthesia was induced with propofol and succinylcholine, endotracheal intubation was uneventful, and maintenance was provided with isoflurane in air and oxygen. Intraoperative opioids were rotated to hydromorphone totaling 15 mg and additional analgesia consisted of a ketamine infusion of 0.2 mg/kg/hr, acetaminophen 1000 mg IV, ketorolac 15 mg IV, and local infiltration of the wound with 20 cc of liposomal bupivacaine. Antiemetics included droperidol, ondansetron, and dexamethasone while ciprofloxacin was administered for surgical site prophylaxis due to a penicillin allergy. Her hemodynamics were satisfactory throughout the case and she was extubated at the end and transferred to the postanesthetic care unit (PACU) for recovery.\nIn the PACU the patient's pain was extremely difficult to control. She received escalating boluses of hydromorphone totalling an additional 6 mg and 40 mg of ketamine in addition to an increase in her ketamine infusion to 0.3 mg/kg/hr. The inpatient pain service, who manages both acute and chronic pain, was contacted to assist with management. The patient was hypertensive and tachycardic with a normal temperature, elevated respiratory rate, and oxygen saturations of 98% on 2 L nasal cannula. Examination revealed an unremarkable cardiopulmonary exam with a soft but diffusely tender abdomen to palpation. She noted her pain was progressively becoming worse over the course of her PACU stay and was some of the worst pain she had ever experienced. Her hemoglobin was 10 g/dL down from 11.7 g/dL preoperatively; electrolytes and coagulation profile were all within normal limits. After discussion with the surgical service, no intra-abdominal process was felt to be the etiology and she was diagnosed with an acute pain crisis complicated by OIH. Given the use of high dose intrathecal opioids, substantial ketamine infusion, and current multimodal regimen, we elected to initiate a dexmedetomidine infusion. Per institutional protocol, she was transferred to the progressive care unit (PCU) for monitoring during this infusion.\nWhile in the PCU her pain continued to be difficult to control. She required a dexmedetomidine infusion of 0.8 mcg/kg/hr, ketamine infusion of 0.7 mg/kg/hr with 10 mg IV boluses every 30 minutes as needed, lidocaine patches around her incision, an additional dose of ketorolac, and scheduled acetaminophen. A narcotic patient controlled analgesia (PCA) system was not prescribed given signs and symptoms of OIH. Boluses of 1 mg of hydromorphone every 30 minutes as needed were available and she had continued use of her personal therapy manager (PTM) to provide the previously prescribed intrathecal fentanyl boluses. This regimen provided adequate postoperative analgesia. Additionally, the patient was found to be hemodynamically stable throughout her admission without any overt signs of pain-sedation mismatch.\nOver the course of next 48 hrs the dexmedetomidine and ketamine infusions were weaned while the interval of IV boluses of hydromorphone and ketamine was progressively increased to every four hours. On postoperative day three she was transferred to the floor and transitioned from intravenous opioids to oral hydromorphone. At the request of the patient, oral medications were chosen rather than making adjustments to the IDDS. Throughout her hospitalization she experienced no opioid related adverse events such has constipation or sedation. She was discharged on hospital day seven with a prescription of hydromorphone 4 mg orally as needed every 6 hrs and plans to follow-up with her primary pain physician. At her three-month follow-up, her chronic abdominal pain was noted to be better and her intrathecal fentanyl usage had been reduced to 11,000 mcg per day. Furthermore, she was to be evaluated by a pain rehabilitation center at home to address her ongoing pain syndrome.
A 7-year-old female patient admitted to the pediatric intensive care unit (ICU) with a known medical history of GT was referred to a pediatric dentist for opinion and management for bleeding gums. The patient presented with a chief complaint of severe bleeding from the upper left back tooth region since the previous night. History of presenting illness revealed that the patient was apparently normal the previous day morning, after which spontaneous bleeding started by afternoon leading to severe blood loss and hypovolemic shock for which she was admitted to the ICU. Bleeding was continuous; there were no aggravating or relieving factors. Previous hospitalization history revealed she was admitted twice earlier within a period of 30 days, with a similar complaint of bleeding from the oral cavity leading to a significant decrease in the hemoglobin (Hb) levels. No dental treatment was performed in the earlier visits, as it was advised by the pediatrician to wait for a month for the complete recovery of the patient. The parent also gave a history of a similar episode of bleeding from gums, vomiting containing clots of blood 1 year back with decreased Hb level at the time of hospitalization. The patient was discharged without being referred to the dentist one year back. A family history of consanguineous marriage was present. On extraoral examination, purpuric spots were seen in the left leg along with crusting and bleeding from the lips. Intraoral examination revealed root stump in relation to 54, 65, dental caries in relation to 53, 74, 36, and mobile crown structure in relation to 75, 55. Spontaneous bleeding was evident from 54, 55, 21, 11, 12, 22, and 46 and the patient also had anterior tooth crossbite in relation to 11, 21 (). The provisional diagnosis was given as gingivitis and chronic irreversible pulpitis in relation to 55, 54, 65, 75.\nLaboratory investigation revealed a hemoglobin level of 1.3 g/dL on the night of admission. The next day the prothrombin time was 18.2 seconds, partial thromboplastin time was 22.8 seconds, international normalized ratio (INR) was 1.70, and hemoglobin was 6 g/dL. On the day of performing the dental procedure (fourth day of admission) as mentioned below, the hemoglobin level was 11 mg/dL, platelet count was 141,000, and bleeding time was 16.5 seconds. Orthopantomogram (OPG) revealed root stump in relation to 54, 65, dental caries involving enamel, dentin, and approximating pulp in relation to 53, 74, and resorbed roots in relation to 75, 55 ().\nConsent was taken from the pediatrician and hematologist. Treatment planned was oral prophylaxis followed by extraction of 55, 54, 65, and 75. Placement of soft splint with absorbable gelatin sponge (Ab gel, Sri Gopal Krishna Lab Private Limited, India) after extraction. Restorative treatment included deep caries management in relation to 53 and 74, followed by stainless steel crown in 74, glass ionomer restoration in 36, pit and fissure sealants in 16, 46, 26.\nAfter being admitted to the ICU, two units of random donor platelet and two units of packed cell volume transfusions were performed and the patient was shifted to the ward after an increase in the Hb levels. Two units of random donor platelets transfusion were performed on the next day. The patient was advised to use tranexamic acid mouth wash (500 mg tablet mixed with 10 mL of water—5%) 6 times a day for 2 days before the procedure. Instructions regarding maintenance of good oral hygiene and use of a soft-bristled toothbrush with fluoridated toothpaste and brushing using the Fones technique were emphasized. The dental procedure was planned for the fourth day. Half a unit of single donor platelets was infused pre- and postoperatively. Impression was made using putty and a soft splint (1.5 mm thick) was prepared. Following buccal infiltration, extraction of 55, 54, 65, and 75 was done. Tranexamic acid (500 mg tablet) was then powdered using mortar and pestle, mixed with saline, and packed into the socket. A paste of tranexamic acid was made, placed on gauze, and held with pressure for ten minutes on the socket after which the gauze was removed. The patient was advised to swallow the remaining tranexamic acid paste. An absorbable gelatin sponge (Ab gel, Sri Gopal Krishna Lab Private Limited, India) was then packed into the socket () followed by splint placement. Post extraction instructions were given and the patient was advised to keep the splint in place. The patient has advised a liquid diet until the next day and intravenous (IV) paracetamol and tranexamic acid (200 mg) 6 hours once were prescribed to the patient. After 2 hours, the patient did not show any signs of active bleeding. The splint was removed, the next day. No active bleeding was evident from the extraction socket. The patient was advised to wear a soft splint for two more days except during meals.\nThe patient was instructed to stop using the splint from the seventh day of admission (3 days after the dental procedure) and no active bleeding was evident from the extraction sockets. Few hours after removal of the splint, there was spontaneous bleeding from 12, 11, 21, 22, 46 and an extraction socket of 55. One unit of random donor platelets was infused on that day followed by infusion of half unit of single donor platelets the next day. Intermittent spontaneous bleeding was evident from the 11, 12, 21, 22, and 46 region. On the tenth day (sixth day of dental procedure), the remaining dental procedures were performed after infusion of one unit of random donor platelets pre- and postoperatively. Oral prophylaxis followed by deep caries management in 53, 74 with stainless steel crown in 74, glass ionomer restoration in 36, and type VII glass ionomer sealant in 16, 26, 46. Postoperatively, oral hygiene instructions were reinforced and IV tranexamic acid 200 mg once in 6 hours was prescribed. Spontaneous bleeding was still evident from 11, 12, 21, 22, and 46 the next day. The pediatrician advised stopping the oral bleeding to discharge the patient. Hence, on the eleventh day (the seventh day after the dental procedure), it was decided to use tranexamic acid paste (crush 500 mg of tranexamic acid and mix with saline) in the area of bleeding on the splint () and then place the splint intraorally. The patient was advised to keep the splint in place for 2 hours after which they were instructed to remove the splint and swallow the remaining tranexamic acid paste. The parent was advised to do the same whenever spontaneous bleeding occurred. The parent was also advised not to use more than two tablets of tranexamic acid (500 mg) each per day based on its maximum permissible dosage. Bleeding was completely reduced after 2 days of using this technique and the patient was discharged from the hospital.\nDiet counseling was done. Stringent oral hygiene instructions were advocated by the patient. The patient was advised to use a soft-bristled toothbrush, fluoridated toothpaste, and gargle after every meal. Also, the parent was advised to use the soft splint with tranexamic acid paste whenever bleeding occurred.\nPostoperatively, a 1-week follow-up revealed no active bleeding from gums. The patient has been reviewed every month for 4 months. The parent gave a history of using soft splint with tranexamic acid paste in the first 2 months during the eruption of the premolars with no history of hospitalization. Sealants were placed in relation to the premolars in the subsequent follow-up visits. At 7 months follow-up, there was no active bleeding and no history of hospitalization (). Loss of sealants was evident in relation to 16, 15, and 26 which was replaced.
A 52-year-old woman presented with pain and active function loss in her left shoulder, and was admitted to our hospital in June 2017. Her shoulder problem had started 3 months earlier, and there was no history of trauma or fracture. At first the pain was intermittent and bearable, but then gradually increased. On examination, there were no positive signs except for localized pain. A radiologic examination on 3 April 2017 found no destruction of the shoulder (Fig. ). Pain at the shoulder joint became gradually aggravated, together with the appearance of shoulder joint dysfunction. Two months later, physical examination revealed mild swelling of the shoulder, and markedly restricted shoulder and elbow motion. Mild distal nerve function defects appeared gradually. Radiography on 3 June 2017 showed that the head of the humerus had disappeared within the past 2 months (Fig. ), which was confirmed by magnetic resonance imaging (Fig. a, b).\nThe patient is a healthy, active individual with no history of weight loss, anorexia, or fever during this period. General and systemic examination findings were within normal limits. Routine laboratory investigations were also normal, including levels of serum calcium, phosphate, alkaline phosphatase, high-sensitivity C-reactive protein, and erythrocyte sedimentation rate. An open biopsy of the lesion revealed that the bony tissue had been replaced by fibrous connective tissue, and small areas of bony trabeculae with occasional osteoclasts were visible (Fig. ). There was no evidence of malignancy or tuberculosis. Because of the lack of any clinical findings or supporting data for other causes, the features were confirmatory of GSD. Computed tomography of the shoulder joint (Fig. ) revealed a bony defect of the glenoid cavity.\nWe performed surgery involving glenoid cavity amplification with an autologous iliac bone graft and a reverse total shoulder arthroplasty. The grafts were from autologous iliac bone (Fig. , Fig. ). A deltopectoral approach was used on the shoulder, and necrotic and dissolving bone tissue was removed. Reconstruction of the glenoid was carried out with autologous iliac bone and installation of reverse shoulder prosthesis. Postoperatively, the arm was placed in a sling for 3 months. Passive elevation and external rotation were allowed 2 weeks after the operation. Three months later, sling use was discontinued, and active range of movement was initiated. Six months after surgery, the patient is pain-free with more than 90° of active abduction, 100° of forward flexion, and 30° of shoulder posterior extension. She also has good functional use of her shoulder (Fig. , Fig. ).
A 57-year-old woman was referred to the Oral and Maxillofacial Pathology Department with swelling over the buccal aspect of her right mandible. The swelling had been present for the last one year and had increased gradually in size over that period. The patient denied any trauma or pain but reported an unusual feeling in that area. Her medical history was unremarkable; she was not taking any medications and had no history of known drug allergy.\nExtra-oral examination did not reveal any obvious facial swelling or asymmetry and there was no cervical lymphadenopathy. Intraoral examination demonstrated a round, painless swelling of about 2 cm in diameter in the buccal cortex of right mandible at the apical region of the lower right second premolar. This bony hard swelling showed no history of parasthesia or surface changes. The overlying mucosa was pink and appeared normal. Radiographic examination [] revealed a round radiolucency with a well-defined margin at the previous extraction site of first premolar, close to the apex of the lower right second premolar. The first premolar had been extracted more than 10 years ago and the patient could not remember any specific reasons for its extraction. The second premolar showed a normal response to vitality test and there was no root resorption.\nConsidering the size and clinico-radigraphic features of the lesion, cyst enucleation was done under local anesthesia and the specimen was submitted for histopathological examination. Its gross examination revealed a spherical, yellow cyst measuring 1 cm in diameter containing a milky, semi-translucent viscous fluid. The thickness of the cyst wall was about 1 mm.\nMicroscopically, hematoxylin and eosin stained sections showed a cyst lined with stratified ameloblastic epithelium consisting of relatively palisaded basal cell layer with plump epithelial cells, granular cytoplasm, small nuclei and distinct borders. These granular cells were closely packed cells distributed in groups throughout the epithelium. The lumen of the cyst was filled with granular cells and connective tissue wall of the cyst was well developed in most areas and not prominently vascular; small areas of hemorrhage and inflammatory cells were also present [Figures and ]. The granular cells showed variation in both size and shape. Most of them were round; but, angular, oval and polyhedral forms were also common. The nuclei of the granular cells were small, pyknotic and often darkly stained [Figures and ].\nPost-operative follow up of 12 months was uneventful and the patient was continuing to receive routine followup at the time of this study.
The patient was a 48 year-old female who underwent renal transplantation in another institution because of uremia in 1998. After the surgery, the patient was on an immunosuppressive regimen including mycophenolate mofetil, cyclosporin A, and methylprednisolone. Although the serum creatinine levels fluctuated at the upper limit of the normal range in the first few years postoperatively, it increased gradually and arrived at a climax of 570 μmol/L in 2007. At the same time, the patient’s highest blood pressure reading was 180/105 mmHg, subsequently, the patient received hemodialysis therapy five times every 2 weeks, with nifedipine and benazepril hydrochloride to control her blood pressure. In 2011, the patient was admitted in a local hospital for intermittent gross hematuria with right lower abdominal pain. There were no symptoms such as frequent urination, urodynia or lumbago. Serum creatinine level was 839 μmol/L. The diagnosis of rejection of transplanted kidney was made, and the patient received transplant nephrectomy. Histological examination confirmed the diagnosis of chronic renal allograft rejection. However, gross hematuria persisted after the surgery. Further examination by computed tomography (CT) showed a suspicious lesion with a diameter of 1.0 cm in the upper pole of the left kidney, so the patient received radical left nephrectomy and postoperative pathological results confirmed the diagnosis of cystic RCC. The symptom of hematuria disappeared after the surgery for 10 months, but then the patient was referred to our institution with a complaint of gross hematuria again. Serum creatinine level was 1,393 μmol/L. Urinalysis showed hematuria and proteinuria (3+). The cystoscopy examination showed bleeding from the right transplant ureteral orifice, with clots in the bladder. Open exploration was subsequently performed through a Gibson incision in the lower right abdomen. Transplant ureteral stump was located near the right wall of the bladder and had adhered to the external iliac vein tightly (). It was difficult to separate the transplant ureteral stump from the external iliac vein, so we decided to remove the transplant ureteral stump including part of the wall of the external iliac vein. The patient recovered with no complication postoperatively. Pathological analysis showed a fistula between the transplant ureteral stump and the external iliac vein (). At present, 20 months after the operation, the patient is free of hematuria. There are also no signs of recurrence of RCC.
The patient was a 45-year-old man with a chief complaint of multiple masses on his fingers and hands. The first mass was noticed at 26 years of age, and although the patient was diagnosed with gout by his doctor, it was not treated. His first examination in our hospital was at 31 years of age, and drug treatment for hyperuricemia was administered. However, about five years later, he stopped this treatment by himself, and there are no records of consultations since then. At 45 years of age, the patient was reexamined at our hospital wishing to undergo surgery for tophi on both hands.\nPhysical examination revealed multiple tophi on both hands () and similar tophi bilaterally on the elbows, knees, toes, and ankle joints. At that time, the PIP joint of the right index finger was contracted. MP joint dislocation was suspected on the palmar side, but the joint could be extended fully. Blood tests at the time of reexamination showed the uric acid (UA) level was elevated to 11.2 mg/dl. Plain radiography showed multiple soft tissue shadows, indicative of tumors and bone erosion in the PIP joints of the four fingers, and the MP joint of the index finger (). Palmar dislocation of the MP joint of the index finger was observed as well. Based on the clinical findings, the patient was diagnosed with gouty tophi and oral administration of Febuxostat (40 mg per day) for hyperuricemia was started to reduce the level of serum uric acid. It was expected that decreasing the uric acid would reduce the tophi.\nAfter about two months, the patient complained about his index finger disorder and was referred to our clinic. His right index finger was difficult to extend actively, but it could be extended passively. An extensor tendon rupture of the index finger was suspected, and surgery for resection of the mass and repair of the extensor tendon was planned. Perioperative findings showed that the extensor digitorum communis of the index finger in the MP joint was invaded by a mass, and its margins were identified proximally and distally. The extensor indicis muscle tendon was found on the ulnar side of the mass and was not ruptured (). Following resection of the mass, the extensor indicis muscle tendon was returned to its original position, and the patient was able to extend his index finger. Therefore, it was not necessary to repair the extensor digitorum communis. We also resected the masses in the PIP joints of the index, middle, and little fingers, as well as in the MP joints of the middle and little fingers. Macroscopic findings showed the masses to be like gouty tophi. Pathologically, crystalline masses surrounded by palisades of multinucleate giant cells and lymphocytes, together with fibrosis, also were found (). The hand was immobilized with a splint for two weeks, followed by motion training. In addition, the patient continued to be administered Febuxostat (40 mg per day) for hyperuricemia, and the UA level six months after surgery was 5.5 mg/dl. Six months after surgery, there was no recurrence of the tophi, and while there was some limitation in the extension of the MP joint of the index finger, which had an arc of motion of 60° (range −20° to 80°), the patient was satisfied with the cosmetic and finger motion improvements ().
A 60-year old Japanese woman was diagnosed with gastric cancer at another hospital. A staging laparoscopic examination was performed at that hospital and peritoneal metastasis was diagnosed. The patient was referred to our hospital to receive IP chemotherapy for peritoneal carcinomatosis of gastric cancer.\nThe upper endoscopy showed scirrhous-type gastric cancer (Fig. ) with the biopsy result of moderately to poorly differentiated adenocarcinoma. A staging laparoscopy was performed also in our institution showing the tumor exposure to serosa of the stomach and the massive peritoneal carcinomatosis involving the greater omentum (Fig. ). Peritoneal disseminated nodules were found in whole intraabdominal space with a peritoneal cancer index (PCI) score of 23 (Fig. ). Intraperitoneal washing fluid cytology was positive for free cancer cells. An IP access port was placed in the subcutaneous space.\nThe regimen of the intraperitoneal chemotherapy is oral intake of S-1 for 14 days, IP PTX (20 mg/kg) and IV PTX (50 mg/kg) on day 1 and day 8 with 7 days’ rest. This treatment was performed as a clinical study approved by the institutional review board of the University of Tokyo. After our patient received nine courses of chemotherapy in 7 months, a second-look laparoscopy was performed to assess the effect of the intraperitoneal chemotherapy. The disseminated peritoneal nodules had disappeared (Fig. ) and the washing cytology results had turned negative.\nAfter confirming the effectiveness of intraperitoneal chemotherapy, a total gastrectomy with D2 lymph node dissection was performed. Three of 14 harvested lymph nodes were found to have metastasis. The pathological grading of regression was 2.\nAfter gastrectomy, our patient received the same regimen of intraperitoneal chemotherapy. A follow-up CT scan, which was performed 4 years and 3 months after the IP chemotherapy started, revealed bilateral ovary enlargement. In order to reevaluate the conditions of the peritoneal disseminated lesions, a third-look laparoscopic examination was performed after our patient underwent a total of 67 courses of intraperitoneal chemotherapy in 4 years and 6 months, which revealed that the peritoneal carcinomatosis had not recurred. However, bilateral enlarged ovaries were observed, which were considered to be Krukenberg metastasis (Fig. ).\nA bilateral adnexectomy was planned but not actually performed because bone scintigraphy revealed multiple bone metastases. The chemotherapy was then changed to the regimen including CPT-11. After the IP chemotherapy was stopped, the peritoneal carcinomatosis recurred. Our patient survived 5 years and 2 months after her diagnosis of peritoneal carcinomatosis of gastric cancer.
A 34-year-old male patient suffered compound fractures with severe crush injuries and burns in the right ankle joint from a traffic accident. The patient was first brought to the emergency room and was treated using negative-pressure wound therapy (NPWT); however, trauma site infection appeared after 4 days and he was transferred to our department (). Examination of the patient revealed a 20 × 15 cm skin defect around the right malleolus medialis, along with denuded tibialis posterior tendon (TPT) and flexor digitrum longus tendon (FDLT), and an active site of Pseudomonas aeruginosa infection. Skin ulceration was also present in the area and on the dorsum pedis. Orthopedic surgery was recommended to cover the TPT and FDLT denudation in addition to extensive debridement around the ankle joint. Debridement of the necrotic tissue caused by the burn injury was performed by our department 5 weeks after the initial accident and wound cleaning was continued for an additional period of time. The lymph node transfer surgery was planned 8 weeks after the accident using a lymph-node-containing free superficial circumflex iliac artery perforator flap from the left inguinal area. Since the patient suffered an open ankle wound and prolonged denudation of the TPT and FDLT, functional impairment was a risk; therefore, debridement around the joint was kept to a minimum in order to focus on preserving joint functionality. Subcutaneous indocyanine green (ICG) injections were performed pre-operatively at two sites around the left anterior superior iliac spine area. ICG lymphography was then performed to identify the lymph flow and the inguinal lymph nodes in the left inguinal region (). An 8 × 15 cm free tissue flap was harvested containing two lymph nodes () and the flap was anastomosed to the branches of the anterior tibial artery and vein. Additionally, a split-thickness skin graft was harvested from the right gluteal region and was used to cover the remaining skin defect in the wound area. An arterial thrombus appeared the day after the surgery and thrombectomy followed by blood vessel reconstruction was performed. No other post-operative complication was observed and the flap and skin graft had attached successfully (). After a post-operative period of 2.5 years, the ankle joint function and toe flexion movements suffered only minimal impairment () and the patient had recovered to a point where he could play soccer without any problems. No recurrence of wound infection appeared in the right ankle and the donor site did not present any lymphedema-like symptoms. Post-operative ICG lymphography confirmed the accumulation of ICG in the transplanted lymph nodes within the flap, indicating the lymph node had successfully attached to the recipient area ().
A female patient, 45 years old, with BMI 80, arterial hypertension, dyslipidemia, waist circumference 135 cm, with medications for T2DM, underwent scheduled LL-RYGB (biliopancreatic limb 200 cm, alimentary limb 150 cm). On the fifth postoperative day leakage of the enteroenteric anastomosis was diagnosed, small bowel resection (approximately 100 cm) with a new anastomosis was performed and she was discharged in good condition. 60 days after the surgery, the patient had an excess weight loss (EWL) of 38% but she gradually showed deterioration in the values of hepatic function and lipids (). Jaundice was presented and she was readmitted to the Surgery Department for support and better investigation of this impairment. First a computed tomography scan and blood cultures were obtained in order to exclude sepsis. The results were negative and the ultrasound did not show biliary obstruction but revealed serious liver steatosis and perihepatic fluid. Despite the nutritional support with special supplements, total bilirubin reached 20 mg/dL and signs of liver failure and clinical deterioration were noted. A liver biopsy was obtained and revealed steatohepatitis with bridging fibrosis (S3A3F3, steatosis activity fibrosis).\nUltrasound guided catheterization of the bypassed stomach was decided in order to place a gastrostomy tube for activating the blinded biliopancreatic limb and reverse the vicious cycle of hepatic impairment. Catheterization with ultrasound guidance did not succeed, so the patient had general anesthesia and a gastrostomy was placed, with the patient recovering well after the operation. Enteral feeding was started from the gastrostomy in the bypassed stomach and after 60 days the hepatic function improved, with better values and resolution of jaundice. Now the patient is in the 6th month postoperatively with EWL 61%, no longer having diabetes or hypertension, and having nearly normalized hepatic values (). The gastrostomy tube was removed and the patient feeds orally. Hepatic impairment after bariatric surgery remains a rare complication and such cases should be recorded in order to evaluate the risk factors and better understand the underlying mechanisms.
A 33-year-old right-handed male was seen for evaluation of swelling in front of the left wrist and palm and intermittent tingling in the thumb, index finger, and middle finger that was noticed by the patient 6 months prior to seeking advice. Though the swelling had been present for a few years, the onset of tingling drew his attention.\nUpon examination, there was fullness over the wrist and in the interthenar area of the palm []. Another swelling was at the base of the index finger. It was soft, non pulsatile, non compressible, and tender to touch. Tinel's sign was positive over the wrist swelling. There was no thenar wasting or sensory deficit. A radiological examination was normal. An ultrasound examination revealed a soft tissue oblong lump extending from the wrist to the palm and another separate mass at the base of the index finger. Nerve conduction studies were not performed.\nSurgical exploration was performed in February 1999 under brachial block anesthesia with an upper arm tourniquet. The median nerve was found to be grossly enlarged from 4 cm proximal to the wrist crease line to the level of its division into distal branches in mid palm. Common digital nerves were not involved in the thickening. The radial-side digital nerve of the index finger was found to be merging with another fibrofatty mass at the base of the digit []. Carpal tunnel release and epineurotomy of the enlarged median nerve were Performed. A biopsy was obtained from the enlarged median nerve and the fibrofatty mass at the base of the index finger. The latter was only partially debulked for fear of damaging the digital nerve. We did not use intraoperative nerve stimulation. The wound was closed after placement of a small suction drain. The drain was removed on Day 3 and the sutures were removed on Day 14. The patient was kept on a regular follow-up schedule. Even after 5 years, the patient revealed no tingling in the thumb, index finger, and middle finger. He was able to carry out all his routine work. Upon examination, Tinel's sign was negative. Sensations were normal in the index finger and the swelling remained unchanged in size [].\nThe epineurotomy specimen with the biopsied strip of tissue showed minimal hypertrophy of nerve fibers. It was closely associated with a noncapsulated fibrolipomatous hamartoma, composed of a haphazard mixture of mature fibrous tissue, adipose tissue, a few blood vessels, and occasional small mature nerve twigs. The debulked mass from the base of the index finger also revealed identical features as seen in the enlarged median nerve proving both lesions to be lipofibromatous hamartoma.[] This mass was infiltrating the subcutaneous tissue resected along with the lesion. There was no neural hypertrophy or true macrodactly.
The 39-year-old female patient visited the hospital as an outpatient, complaining of pains from backache and the right leg. The patient had a lumbar MRI scan for back pain at another hospital three years ago, but there was no unusual diagnosis. For pain control, an epidural nerve block was performed at another hospital, but the symptom had not improved yet. After the operation, the patient gradually got pains at the back and right leg, and the symptom became worse starting a year ago. A physical examination showed pains during the silent period, referred pain to the right leg, and radiating pain from the lumbar 5th segment. From the straight leg raising test, the patient complained of pains from both legs at an 80 degree angle. However, any disorder or decompression of motor nerve and sensory nerve was not detected. The lumbar X-ray picture scanned on the same day showed a small metal foreign body ().\nWith the MRI was scanned at the hospital, doctors diagnosed degenerative spondyloisthesis at the lumbar 4-5th segment, and degenerative change from the facet joint as well as a foreign body (). The hospital diagnosed spinal stenosis and a foreign body at the facet joint, and decided to perform posterior lumbar interbody fusion and foreign body removal from the right facet joint of the lumbar 4-5th segment.\nThe patient was 158 cm tall and weighed 62 kg. Her liver function examination was normal before operation, except it was positive for the antibody for hepatitis C. Except that, there was no special diagnosis before operation. For the treatment before anesthesia, glycopyrrolate 0.2 mg was injected into the muscle on the very day of operation. After arriving at the operation room, 18 G intravenous route was additionally secured at the right side of the upper arm, and an ECG, a noninvasive blood pressure monitor, pulse oximetry, and capnogram were established. After identifying stable blood pressure and pulse rates, 100% oxygen 6 L/min was provided through a mask for 1-2 minutes. For the induction of anesthesia, propofol 2 mg/kg was injected into the muscle for 20 seconds, and after identifying the loss of lid reflex and the stability of blood pressure and pulse rates, rocuronium 0.9 mg/kg was injected into the muscle. After sufficient muscle relaxation was identified after forced breathing with the mask was performed for 2 minutes when there was no spontaneous breathing, intubation was performed. For the maintenance of anesthesia, oxygen 2 L/min, nitrous oxide 2 L/min, and isoflurane 1.5-2.0 vol% were used. With the favorable vital signs of the patient, the posture was changed to the prone position, and the surgery was performed after finishing all necessary preparations. The operating surgeon removed the metal foreign body stuck in the right side of the facet joint at the lumbar 4-5th segment, and finished the planned posterior lumbar interbody fusion safely. After the operation, the patient recovered well and left the hospital after one week. The foreign body removed from the operation was a stuffed needle with a sloping side made from a metal in silver color; its diameter was less than 1mm and its length was 0.7 cm (). Based on the previous epidural block history of the patient and the shape of the foreign body, it is considered to be the broken stylet of an epidural needle tip.
A 32-year-old male presented to us following an acute attack of severe right upper abdominal pain for two days. Upper abdominal ultrasonography revealed a septate edematous gallbladder with stones; the extra and intrahepatic biliary tree was otherwise normal. Hematological investigations including liver function tests were normal. Laparoscopy using a standard four-port technique showed a more laterally placed thick-walled gallbladder and an edematous cholecystohepatic ligament. [] Fundus first dissection of the gallbladder was carried out after aspirating the contents of the gallbladder; initially mucoid content and then frank pus was noted. During dissection at the neck of the gallbladder and in an attempt to separate it from the adjacent structure, bile leak occurred from a small rent in what appeared to be a distended duodenum. Suspecting a duodenal injury, a Fr 8 infant feeding tube was inserted into the rent and contrast injected. A well-distended gallbladder with a normal cystic duct was recognised []. This second gallbladder had no communication with the cephalic gallbladder and was separated from it by edematous connective tissue. The second gallbladder was edematous, inflamed and covered by omental adhesions. A careful search in the liver bed showed no evidence of bile leak or ductal structure and it was assumed that the enlarged cephalic gallbladder either had no cystic duct or the cystic duct was completely obliterated. A separate artery was seen arising from the right hepatic artery and supplying the cephalic gallbladder with the artery coursing in the edematous cholecystohepatic ligament. The distal gallbladder was dissected off the duodenum and after carrying out an intraoperative cholangiogram cholecystectomy was completed. The IOC showed a normal biliary tree and no leakage of dye. Careful inspection of the gallbladder bed showed no biliary leak and no ductal structure could be identified. The patient recovered well and was discharged on the third postoperative day.\nGross examination of the gallbladders [] showed two separate entities with no communication. The cephalic moiety showed features of a pyocoele with severe inflammation of the entire wall. The distal gallbladder showed acute inflammatory changes with cystadenomatous changes. No obvious calculi were seen in either of the gallbladders.
In May 2011, a 41-year-old Caucasian male was referred to the orthopedic department of our institution for evaluation of a 6-month left proximal humeral pain, increasing in severity over the last 2–3 weeks. His medical history was unremarkable and was under no medications at the time. He was an ex smoker and a social drinker and worked in constructions. Family history was notable for a grandfather with lung cancer and an aunt with breast cancer.\nUpon physical examination, a limited range of motion of the left shoulder was noted (decreased flexion, extension, abduction, and external rotation). Plain radiograph of the left shoulder demonstrated a lytic lesion with intralesional calcifications and cortical remodeling, suggestive of a chondrosarcoma. MRI of the left shoulder confirmed this finding. Biopsy of the lesion was positive for grade 2–3 chondrosarcoma. A full diagnostic workup was carried out, including a CT of the chest and upper abdomen and a bone scan, which demonstrated increased uptake in the left upper arm but no additional bone lesions. Hematological and biochemical investigations were within normal limits.\nIn June 2011, the patient underwent a limb sparing surgical operation, and pathology was positive for conventional chondrosarcoma grade 2–3, that had destroyed the medullary and cortical portions of bone reaching into adjacent soft tissue. Distal bony resection margin and skin were both free of tumor infiltration. The patient did not receive any adjuvant therapy.\nThe patient remained free of disease for 4 years following surgery. In 2015, the patient complained of increasing pain in his left resected arm and shoulder. Imaging confirmed local recurrence. The patient subsequently underwent amputation of his left arm with resection of scapula. Pathology confirmed diagnosis of a grade 2/3 conventional chondrosarcoma. In August 2016, he started to experience significant substernal chest pain with shortness of breath and was admitted to the hospital. A CT scan of the chest revealed multiple pulmonary lesions compatible with metastases. The patient was discussed at the oncology tumor board and it was decided that chemotherapy should be administered. He was treated with chemotherapy combination of doxorubicin 20 mg/m2, Ifosfamide 2.5 g/m2, and Mesna 2.5 g/m2 IV on days 1–3 every 3 weeks. After 3 months of treatment, chemotherapy was discontinued due to disease progression, which included presence of new pulmonary nodules.\nAt that time, we decided to treat the patient with pazopanib as second-line treatment. The drug was well tolerated and after 6 months of treatment, the patient had partial response on imaging according to RECIST criteria (Figure ), with improvement of symptoms. At the last follow-up in August 2017, clinical, biochemical, and imaging evaluation showed no evidence of disease progression. The patient continues to be on treatment with pazopanib. Written informed consent was obtained from the participant for the publication of this case report.
An 11-year-old male patient from the rural background consulted the ophthalmology OPD with loss of sensations over the left half of the face and the cornea since birth and reduced visual acuity.\nThe patient also complained of occasional deviation of the tongue during speaking and chronic pulsatile headache especially in morning. On examination, the vitals of the patient were stable, and general examination of the patient was uneventful except for pallor. Neurological examination revealed hypoesthesia along the left side of the face corresponding to ophthalmic, maxillary and mandibular divisions of the trigeminal nerve. There was corneal anesthesia with corneal ulceration. Rest of neurological examination was normal. The patient was then referred for contrast-enhanced computed tomography scan of the head, which revealed abnormally dilated and tortuous left vertebral artery, with an aberrant course. The caliber of the vertebral artery was 7.2 mm and it was seen compressing the left lateral aspect of the brainstem, with relative atrophy of the anterosuperior part of the left cerebellar hemisphere leading to dilation of ipsilateral cerebellopontine angle cistern and crossing toward the right side []. The ectatic vessel was also seen compressing the fourth ventricle with mild dilatation of bilateral lateral ventricles.\nNo definite evidence of thrombosis was seen, and the rest of the brain parenchyma was normal at the time of the scan. For further work up of the disease etiology, patient underwent magnetic resonance imaging (MRI) of the head with magnetic resonance angiography (MRA). MRI of the brain showed the abnormally dilated vessel to be the left vertebral artery, with evidence of compression of the left inferior olivary nucleus causing its flattening and the brachium pontis [Figures and , ]. Application of constructive interference in steady state sequence revealed that the ectatic vessel was associated with left-sided trigeminal nerve hypoplasia, with marked thinning of the preganglionic cisternal segment of the trigeminal nerve. The Meckel's cave and the porus trigeminus were empty []. Also noticed was mild displacement of the left facial nerve by the ectatic vertebral artery []. Rest of the visualized cranial nerves appeared normal. MRA showed that the left vertebral artery was unduly prominent, with an aberrant course having an anomalous high union with the left branch of the right vertebral artery at the level of brachium conjunctivum []. There was associated atrophy of the lingula and lobulus centralis on the left side leading to prominent cerebellopontine angle cistern []. The patient was reassured and was advised keratoplasty for correction of corneal ulcer. Being congenital in nature, the pathology was deduced to be nerve hypoplasia rather than nerve atrophy.
A 37-year-old male patient with previously diagnosed Sturge Weber Syndrome presented to the outpatient clinic inquiring about reconstructive treatment for a congenital vascular malformation that engulfed a considerable portion of his face. Past medical history was significant for a lifelong seizure disorder secondary to intracranial vascular angioma for which he was placed on several medications with poor symptom control. The patient was also medically treated for depression and anxiety and visited a psychologist regularly for the social strain his disease has caused him with family and peers.\nDuring the preoperative physical examination, the patient was noted to have a tuberous hemangioma of the right face and scalp with significant involvement in the frontal region (Figure ). The patient had an intact vision at the time, was able to grow a beard over the affected side, and did not show any lesions on the oral examination. The patient was deemed a candidate for surgical intervention and scheduled to undergo excision of the facial vascular malformation with staged full-thickness skin graft reconstruction.\nProcedure no. 1\nA right deltopectoral flap was raised through the area of the perforators in the mid-chest, turned into a pedicle, and inserted into a donor site in the right postauricular area. In addition, a forehead flap was delayed, outlining the flap to involve the complete left forehead site. Postoperatively, the patient was assigned a home health nurse for daily wound care and dressing change. At one month follow up there was a dark area on the deltopectoral pedicle with crusting behind the ear indicating tissue damage, but the flap was otherwise healing well with no further deterioration and was deemed viable for future reconstruction (Figure ).\nProcedures no. 2 and 3\nThe hemangioma on the right forehead, cheek, and nose was excised. The tube pedicle was then divided from the chest, opened longitudinally, and tailored to the defect in the cheek and upper lip. The previously delayed left forehead flap was elevated from the forehead and rotated to the nasal area with the pedicle maintained in the left medial brow area. Two weeks later, the postauricular portion of the deltopectoral flap was divided and the flap was untubed. The remaining area of right cheek malformation and the right eyebrow was excised, and the flap was trimmed and tailored to fit the defect in the right cheek. Then, the scalp flap was placed into position to cover the defect over the right eyebrow. Postoperatively, the skin on the nose showed evidence of self-induced trauma indicated by a linear area of excoriation (Figure ).\nProcedure no. 4\nThe patient underwent sculpting and recontouring of the flaps to create an anatomically appropriate eyebrow, upper eyelid, nasal bridge, and upper and lower vermillion borders on the right side of the face. Postoperatively, the patient continued to pick and scratch at large portions of his scalp flap and right cheek despite numerous warnings and safety measures including shielding the flaps with heavy dressing, prescribing antibiotics and anti-inflammatory medications to alleviate the associated pain and pruritus, and coordinating care with his psychologist to address these actions during therapy sessions. This resulted in local wound breakdown and necrosis which required repeated debridement and dressing during consequent visits (Figure ).\nProcedures no. 5 and 6\nA tissue expander was inserted in the posterior scalp to promote the growth of additional skin to be used in the reconstitution of the anterior hairline. During the follow-up period, the patient missed several appointments for wound care and tissue expander fills. The tissue expander eventually became exposed, requiring prompt removal and further facial reconstruction (Figure ). Postoperatively, the patient developed areas of necrosis and granulation tissue in the parietal and temporal regions as well as mummification of the scalp. Follow up remained poor and self-mutilation behaviors continued (Figure ). Additionally, the patient began experiencing increased seizure activity with frequent hospital admissions which made it increasingly difficult to maintain a controlled environment for healing of the wounds. The overseeing neurologist managing his medication regimen described his seizures as both petit mal and tonic-clonic, with the latter type involving episodes of rapid left-sided contractions lasting up to two hours in duration. The prolonged and frequent tonic-clonic activity contributed to the patient's growing frustration and poor compliance with wound care. His psychologist voiced concerns regarding his internalized anger and tendency for impulsive behavior but advocated for continuing with surgical intervention as the patient was eager to improve his appearance. The focus shifted towards addressing the significant residual right vertical orbital dystopia and acquired scalp defects.\nProcedure no. 7\nThe patient underwent correction of his right vertical orbital dystopia. Due to the large size of the frontal sinus discovered on imaging, an extracranial approach was chosen with orbital exploration, orbital unroofing into frontal and ethmoid sinuses, intraconal lipectomy, and medial canthopexy to establish symmetry with the contralateral unaffected side. Excision of multiple pyogenic granulomas in the right scalp was also performed followed by debulking and revision of the right facial flap, scalp flap advancement, and split-thickness skin graft for coverage of the remaining defects. Though the immediate postoperative period was well-tolerated, the patient continued to exhibit scratching/picking behavior of his facial wounds, causing severe tissue degeneration. The patient was seen at the office multiple times per week but attempts to prevent further self-injury were unsuccessful (Figure ).\nProcedure no. 8\nApproximately 16 months after procedure No. 7, the patient returned to the operating room for multiple revisions including reconstruction of the facial flaps, right lateral and medial canthopexies, and additional skin grafts to the face. One week following surgery, the skin grafts on the scalp, forehead, and portions of the eyebrow and nasolabial area were reportedly removed by the patient. Examination revealed poor hygiene and total loss of the skin graft on the right scalp and forehead, and partial loss of the right eyebrow and nasolabial fold (Figure ). The wounds were extensively cleaned with Dakin's solution, which was successful in preventing infection and encouraging the formation of granulation tissue (Figure ). The patient was advised and urged to have additional surgery for an immediate skin graft to the area to avoid infection and malignant transformation of the tissue, but he did not comply.\nAfter months of encouragement, the patient agreed to undergo surgery and was admitted to the hospital but ultimately left against medical advice prior to the operation. From 1993 to 1996, the patient continued to follow-up at the office, expressing interest in further surgical intervention but not following through. The last photographic documentation from 1995 portrays the significantly poor outcome following his 10-year treatment (Figure ).
A 35-year-old Indian male who was previously healthy, presented to the emergency department with epigastric pain for 5 days. This was associated with intermittent episodes of black stool, fatigue, and loss of appetite for 2 weeks prior to presentation and a significant weight loss of 4 kg in the preceding 6 months. He was found to have microcytic anemia with a hemoglobin of 5.6 g/dL and his iron profile was suggestive of iron deficiency anemia. The platelets and the coagulation profile were normal. A blood transfusion was arranged to correct his anemia, but he developed a transfusion reaction on 2 separate occasions with breathing difficulty and restlessness a few minutes after starting the blood transfusion. This was accompanied by hypotension on the second attempt. The blood transfusion was stopped. There was no prior history of blood transfusion or allergy, and since he was young and from an Asian ethnicity, IgA deficiency was suspected. IgA level was sent and came back as low titer (<0.50 g/L), with a negative Coombs test. Washed packed red blood cell units were arranged for transfusion. US abdomen showed thickening of the gastric wall measuring up to 1.5 cm at the pyloric region. CT abdomen with IV contrast showed a circumferential wall thickening of the distal gastric body and pyloric region, with a fungating mass having an exophytic component arising from the posterior aspect of the pylorus with central breakdown suggestive of gastric malignancy.\nGastroscopy was done after 2 days and a circumferential, ulcerating, friable mass that bleeds to touch was found occupying all the antrum, the pylorus, and the distal body of the stomach (shown in Fig. ). The biopsy from the mass showed invasive gastric adenocarcinoma of the mixed type. Positron emission tomography (PET) scan was done and showed multiple peritoneal, abdominal lymph nodes and bilateral small pulmonary metastases. The patient was subsequently treated with a palliative intent. Other workup showed eosinophilia, and stool microscopy showed Strongyloides species larvae. Stool H. pylori antigen was positive and was confirmed by Warthin-Starry stain on biopsy.
In October, 2007 a 61-year-old woman presented to Saint Vincent's Medical Center with a 5 cm subcutaneous growth on her left upper extremity. Histological examination after surgical excision of the mass revealed a clear cell neoplasm consisting of polygonal cells with abundant clear cytoplasm, containing faint granular material. Immunohistochemical analysis demonstrated positive CD10 and AE1/AE3 staining. Pathologic interpretation of the mass was highly suggestive of metastatic RCC of the clear cell type. There were no lesions present anywhere else by physical examination or CT scan.\nThe patient was closely followed in an attempt to locate a primary renal source of disease with multiple imaging studies negative for a renal primary or other sites of metastasis. However, repeat CT scan 9 months later revealed an asymptomatic pancreatic mass. Endoscopic evaluation was performed with endoscopic ultrasound and fine needle aspiration (EUS/FNA). The study demonstrated a 2-cm hyperechoic, well-defined lesion in the body of the pancreas. The remaining pancreatic parenchyma was otherwise normal without ductal dilation or evidence of pancreatitis. Histomorphological analysis of the core biopsy samples yielded similar findings to those of the upper extremity mass. Additionally, an immuno-profile was focally strong for both CD10 and PNRA, which was again highly suggestive of renal cell carcinoma. A central pancreatectomy was performed in August 2008 and tissue samples were positive for PRNA, Vimentin, and CD10, correlating strongly with RCC. The patient continued periodic surveillance to identify a renal primary and further metastasis at three month intervals. Six months later, physical exam revealed left parotid gland enlargement and an MRI revealed a 1.6 cm enhancing mass in the left parotid gland. No other lesions were found on surveillance PET/CT scan at that time. The patient had a superficial parotidectomy and again, pathological analysis demonstrated a clear cell carcinoma that was identical to the previous subcutaneous and pancreatic specimens. The sample was sent for expert verification at an outside institution, which corroborated our findings. Currently, the patient is doing well and is undergoing surveillance at 6 month intervals. To date, a renal primary has not been found.
This report is based on the informed consent of the patient and the approval of the appropriate ethics committee.\nWe report a case of a 56-year-old man with an olfactory neuroblastoma. The patient's chief complaint was nasal obstruction and left epistaxis. Magnetic resonance imaging showed that the tumour occupied mainly the left olfactory cleft and was isointense on T1 and high intense on T2 and showed no thickening of the left dura mater or invasion of the middle cranial fossa (shown in Figures and ). The left frontal sinus was filled with secondary mucus, and no tumour component was observed. However, both sides of the frontal sinuses were hypoplastic, with thickening of the septum of the frontal sinus and a difference in the anteroposterior (AP) diameter of the frontal sinus between the right and left sides (shown in ). The AP diameter was 9.6 mm on the right and 5.9 mm on the left, and the skull base-frontal sinus angle (SBA) was 126° on the right and 107° on the left (shown in Figures and ).\nThe patient underwent endoscopic anterior cranial resection for treatment. To determine the anterior tumour resection line, the outside-in EMLP was selected. First, the first olfactory fibre was identified on the right side (non-tumour side) (shown in ), and the depths of the right frontal sinus and the right anterior skull base were confirmed by Draf 2B (). Subsequently, Draf 2D [] was used to approach the left frontal sinus from the right frontal sinus via the septum of the frontal sinus (shown in ). At this time, the first olfactory fibre was identified on the left side (tumour side), and a space was observed on the left side at the same depth as the right frontal sinus, and the space was opened as if it was the frontal sinus. After opening the space by 4 mm, CSF leakage was observed, and the skull base was judged to be damaged (shown in ). When the position of the frontal sinuses was reoriented and the bilateral frontal sinuses were converted to a single sinus, the left anterior skull base protruded more than the right anterior skull base, and it was confirmed that the protruded region was damaged (shown in ). Fortunately, the injured area of the skull base overlapped with the resected area of the tumour (shown in ), and the skull base was reconstructed to be water-tight by suturing the dura mater to the fascia with 11 stitches of 6-0 proline (shown in ). Additionally, the fascia and nasal septal cartilage were in-layed between the dura mater and the skull base (shown in ). Finally, the nasoseptal flap was over-layed to complete the operation (shown in ). The postoperative course was good without CSF leakage or intracranial haemorrhage, and the patient was discharged one week later. There was no recurrence or higher-order functional disability observed 1 year after surgery.
An 11 year-old boy was first seen with the chief compliant of pain and swelling at the lateral aspect of the left ankle of two months’ duration. There was no history of injury, fever, loss of appetite or weight loss. Local examination revealed an illdefined swelling of 5cm x 4cm at the lateral part of left ankle which was warm and tender to touch. There was terminal restriction of movement at the ankle joint with no evidence of distal neuro-vascular deficit. Radiographs revealed an expansile lytic lesion involving the distal third of fibula with epiphysis with some patchy sclerosis and cortical breach. The proximal extension of the marrow and cortex is about 22.5cm from the tip of the fibula ().\nMRI showed an aggressive lesion involving the distal third of the right fibula with involvement of distal epiphysis, and with cortical breach and soft tissue extension (). Chest CT scan revealed no evidence of metastasis. A diagnosis of malignant neoplastic lesion of distal fibula was made. Core biopsy revealed histopathological features of osteosarcoma with tumour cells demonstrating high pleomorphic and many nuclei and prominent nucleolus. The tumour cells were surrounded by haphazardly arranged dense pink matrix. This was typical osteoid produced by malignant tumour cell of osteosarcoma. (). A decision to perform wide fibular resection was made as tumour was localized to lateral compartment with no metastases. Three courses of neoadjuvant chemotherapy were given at three week intervals. Post chemotherapy MRI revealed cortex and marrow involvement of the left fibula measuring 10.7 cm from the lateral malleolus.\nPatient underwent distal fibulectomy through a direct lateral longitudinal incision. At operation, the lesion was about 11 cm in diameter with involvement of flexor hallucis longus, peroneus brevis and the peroneal artery (). About 15 cm of fibula was resected above the tip of the lateral malleolus and marginal excisions of flexor hallucis longus and peroneus brevis were carried out leaving behind the distal tendons intact (). Peroneal artery was ligated. Ankle reconstruction was done using soft tissue and tested clinically for stability. Haemostasis was achieved and the wound closed in layers. External fixator was applied and sterile dressing done ().\nSutures were removed after two weeks and patient was mobilised with non-weight axillary crutches. At 12 weeks, the external fixator was removed and fibreglass cast was applied and full weight bearing started. Post-operatively, chemotherapy with Doxorubicin and Cisplatin was administered every three months for four cycles. Patient was followed up at intervals of three weeks for the first three months, three monthly for one year, six monthly for two years and every year for five years. During his last follow up in December 2016, there is no pain or swelling and the ankle joint was stable. Radiograph showed valgus and talar shift but no sign of joint degeneration. () However, the varus and valgus stress tests at ankle was negative (). He was able to participate in regular sports including running, cycling and playing basketball.
A 29-year-old male patient with no significant medical or surgical past history presented to our emergency room (ER) with complaints of gradually progressive abdominal pain of two-week duration associated with recent development of nausea and vomiting. Abdominal pain was located in epigastrium, intermittent, 6/10 in severity, nonradiating, and was dull in nature. Patient also stated that he took some unknown over-the-counter antacid to relieve the pain but it did not help. Patient also complained of nausea and vomiting which started after his moderate alcohol intake 2 days ago. Patient denied taking any other medications. He also denied any history of trauma. Physical examination was negative except for presence of tenderness in epigastric area. Laboratory exam was completely normal including amylase and lipase levels (). A diagnosis of alcoholic gastritis was made and patient was discharged from ER with a proton pump inhibitor.\nThis patient again came to ER after about 72 hours with complaints of increased abdominal pain and intractable nausea and vomiting. On examination patient had generalized abdominal fullness and tenderness. Bowel sounds were sluggish on auscultation. Rectal examination was normal with stool negative for occult blood. Laboratory examination was abnormal with elevated levels of amylase and lipase. Laboratory examinations for day 1 and day 2 of presentation to ER are elaborated in .\nUltrasonogram of the abdomen demonstrated a 3.5 × 3 cm heterogenous, well-defined lesion anterior to the right kidney in the location of second part of duodenum (). This lesion failed to show any vascularity on Doppler sonogram. Computed tomography (CT) scan of abdomen without intravenous (IV) contrast showed a circumferential hyperdense lesion in the wall of the second part of duodenum with no definite mass with presence of minimal peripancreatic fat stranding. There was associated gastric outlet obstruction (). CT scan with IV contrast could not be performed as patient had acute renal failure at the time of presentation.\nPatient was started on nil per oral and aggressive hydration therapy. Continuous gastric suction was performed by using a nasogastric tube. Laboratory blood tests were monitored at regular intervals and significant elevations in lipase, amylase, BUN, creatinine, and WBC were observed initially which started to trend down later. A graph depicting the levels of lipase is shown (). Serial abdominal CT scans without IV contrast were done (Total 3) and patient was managed symptomatically. Patient's hematoma slowly started to regress and pancreatic enzymes levels came down. Patient was admitted in the hospital for total of 12 days. His hematoma completely resolved at the time of discharge and he was completely asymptomatic.
A 81-year-old female patient, living with her nephew, who is absent most of the time. One of her primary caregivers is her neighbor, who looks after her daily and makes sure that she is in good health and that she does not need anything. The neighbor and the patient, upon being interviewed, indicated that the patient usually has a good cognitive level, and that there are no major difficulties with her superior mental functions. Moreover, they claimed she has no problems doing housework or other activities. They consulted with a doctor because they were worried the patient may be developing early symptoms of dementia.\nThe patient has a history of chronic hyperthyroidism and hypertriglyceridemia. At the moment, these conditions are under control, as her primary caregiver treats the conditions with 0.1 mg of levothyroxine medication and 600 mg of gemfibrozil, administered daily. She denies having a family history of psychiatric illness and consuming any kind of substance or medication, other than those indicated by her primary physician.\nThe patient, at the moment of her mental examination, was dressed according to her age and gender, exhibited a good attitude and was very collaborative with the interviewer. She used an adequate tone of voice, normal verbal language, was capable of rational thought and adequate course and content, had sensorial perceptions without alterations, was conscious, seemed well oriented and alert, showed a slight difficulty with calculations, proved to have an adequate memory and proper capacity of abstraction, had adequate judgment and adequate illness perception. She displayed no reason to doubt her story.\nThe patient claimed that a month ago she suffered an episode of Senile Dementia, which lasted for 3 days, by that name she refers to cognitive impairments and alterations of consciousness, the list of signs and symptoms shown was corroborated by her neighbor. Previously she was seemingly healthy. The symptoms were apparent because she was leaving her activities unfinished for no apparent reason; for example, if she was eating she would stop all of a sudden with no explanation. She wanted to sleep only during the day, presented serious memory defects: she did not remember what had just been said to her and did not recognize her life-long neighbors. She forgot to shower, even though she used to do it daily, and she would have difficulty thinking clearly. She was not speaking clearly and others could not understand her. These symptoms would not last all day, but rather would present themselves in certain periods during these 3 days.\nThe patient mentioned she stopped taking levothyroxine weeks before that period claiming she had forgotten where she stored her medication and that her symptoms went away when she resumed her treatment as indicated in a first consultation in the emergency medical service, a Mini-Mental State Examination (MMSE) practiced in the same query showed a score of 22 points. She seemed worried this episode might repeat itself and might become permanent in the future.
A 78 year-old male presented to the emergency department with a ten day history of mild abdominal pain, nausea, and distention, worsening over the last day. He did not recall having a bowel movement for at least three days. His past medical history was significant for hypertension, gout, osteoarthritis, and an eighty-pack year smoking history. He had no prior surgeries and took only a blood pressure medication. He guarded during physical exam, and his abdomen was noted to be quiet, distended, and tender to palpation, but without rigidity or peritoneal signs. His laboratory evaluation was unremarkable. The emergency department obtained a CT scan of his abdomen and pelvis, which demonstrated a large amount of free air and fluid, and a mass could be visualized within the lumen of the cecum. A surgical consultation was emergently obtained (Figure ).\nAfter fluid resuscitation, he was brought to the operating room for exploration, where we encountered a minimal amount of fecal contamination and perforation of the cecum. A six centimeter non-obstructive mass and a hard mesenteric nodule were found in the cecum just distal to the site of perforation. The remainder of the colon was palpated and noted to be full of hard stool but otherwise without obvious abnormalities. We proceeded with copious irrigation of the peritoneal cavity and performed a right hemicolectomy with primary anastamosis without difficulty.\nPost-operatively, the patient was extubated but progressed slowly. Final radiologic interpretation of the CT obtained on arrival additionally noted a mass in the tail of the pancreas, a finding the surgical team did not detect on the CT prior to surgery or grossly at exploration. Pathologic evaluation found that the cecal mass contained only tubulovillous adenomatous components and the perforated area demonstrated localized mucosal ischemia but had relatively sharp margins. Well-differentiated metastatic adenocarcinoma was found within the mesenteric nodule, without lymphatic components (Figure ). The primary tumor responsible for this metastatic nodule was not contained within the surgical specimen. The patient had a slow return of bowel function consistent with ileus, but his abdominal distention increased dramatically overnight on post-operative day five. A repeat CT demonstrated colonic distention proximal to a now apparent mass at the splenic flexure, with distal colon decompression, concerning for a large bowel obstruction (Figure ). The patient returned to the operating room for reexploration and resection of the obstructive distal colon mass missed at initial operation.\nAt the second exploration, the entire colon was mobilized, and this time, a mass could clearly be palpated at the splenic flexure of the colon. Continued mobilization revealed that this mass involved the splenic hilum and tail of the pancreas. An en bloc resection of the pancreatic tail, spleen, and left colon was completed without difficulty, and primary anastomosis was completed (Figure ). The patient was extubated postoperatively and progressed more quickly this time. Final pathologic evaluation of the second specimen was surprising, consistent with mucinous pancreatic adenocarcinoma, extending into the splenic flexure of the colon (T3, N0, M1). At three-month follow up, the patient is doing well living in an extended care facility.
47-year-old female presented to our Emergency department in 2012. She complained of progressive intermittent abdominal pain. Clinical exam revealed bilateral pitting edema and an elevated jugular venous pressure, with no history of familial polyposis or colorectal cancer. Laboratory work-up was noncontributory. CT revealed an ill-defined hypoattenuating solid mass in the small bowel mesentery (), with slight displacement of adjacent small bowel loops and mild enhancement.\nThe mass was resected surgically along with a right hemicolectomy and pathology reported it as a desmoid tumor. Mass was noted to have clear margins on pathology. No additional treatment was given. The patients immediate postoperative course was uneventful.\nFour years later, the patient returned to the Emergency department with a small bowel obstruction. There was no additional change in medical history. CT was repeated showing a recurrent irregular solid hypoattenuating mass in the mesentery of the right lower quadrant (). The mass was located in close proximity to adjacent loops of ileum. The small bowel was distended with little gas in the colon consistent with a small bowel obstruction.\nMRI advised for further characterization of the right lower mesenteric mass. MRI () shows the mesenteric mass to be of heterogenous signal intensity on T2 weighted imaging, low in signal of T1 before contrast and slight enhancement after contrast. The findings suggest desmoid recurrence in the right lower quadrant.\nIn the four-month interval between most recent scans the mass had grown significantly from 5 × 4 cm on CT to 8 × 7 cm on MR (Figures and , resp.). The patient underwent another laparotomy for definitive diagnosis. On surgical resection, the margins of the mass were noted to push against the edge of the serosal peritoneum. The mass was adherent to small bowel and involved 25–30 cm of the small bowel mesentery but was mobile.\nOn gross pathology, the mass was noted to be adherent to small bowel and firm. The mass was circumscribed and measured 9.2 × 7.3 × 7.2 cm. Focal areas of hemorrhage were noted near the periphery. The tumor did not invade surrounding fat or bowel (). Microscopically the lesion was composed of fibroblasts arranged in sweeping fascicles (). No atypia or pleomorphism was identified. Beta-catenin was diffusely positive ().\nFollowing surgical resection, no adjuvant therapy was administered. Follow-up with radiological imaging was planned.
A 60-year old male was admitted in the emergency ward with the history of massive hematemesis the previous night. Patient had difficulty in swallowing and retrosternal discomfort for the past 2 months for which he was being treated. Once in the hospital there was no fresh bout of hematemesis. On admission the patient's pulse rate was 124/minute and blood pressure was 100/70 mmHg. Hemoglobin level was 9.9 g/dl and the white blood cell count was 13,200/cmm. The chest X-ray showed bilateral emphysematous bullae. Upper GI endoscopy showed esophageal ulceration at 32 cm.\nContrast enhanced chest CT images acquired at thoracic region showed a penetrating aortic ulcer into the thoracic esophagus and focal anterior aortic defect with pseudo aneurysm formation [Fig , , ]. There was no pleural effusion. A diagnosis of aortoesophageal fistula due to penetrating aortic ulcer was made and the patient taken up for an emergency surgery.\nHe was intubated with double lumen endotracheal tube. Left radial and dorsalis pedis arteries were cannulated for the arterial pressures monitoring of upper and lower body respectively. A pulmonary artery catheter was introduced. An epidural catheter was placed to facilitate cold saline irrigation of the epidural space during aortic cross clamping. Injection Methylprednisolone 1 G was given intravenously for spinal cord protection. While securing of the femoral artery and vein in the left groin was being done, thoracotomy was performed through a posterolateral incision. There was no hematoma or effusion or pus in the pleural cavity. Descending thoracic aorta was enlarged but not to aneurismal proportions [Fig ], the medial portion of the lower lobe of the left lung was adherent to the esophagus in the region of the fistula. Aorta was mobilized from the left subclavian artery to the diaphragm except for the area with dense adhesions that contained the fistula. Heparin was given at 1 mg/Kg and after obtaining an ACT of > 400 seconds and aortic cannulation was done distal to the left subclavian artery and also the left femoral artery cannulated using appropriate arterial cannulae and were connected by an 1/4th inch polyvinyl chloride [PVC] tube establishing an aortofemoral shunt. The aorta was cross-clamped and the intercostals arteries were controlled with micro bulldog clamps. The distal aortic pressure maintained between 60 to 70 mmHg. Aorta was opened with a longitudinal incision and the aneurismal mouth was seen in the anteromedial aspect measuring 3 cm × 2 cm in size and oval in shape [Fig ]. The base of the ulcer was filled with clots. Rest of the aorta appeared normal and edge of the defect appeared healthy. The defect was closed with a gelatin sealed Dacron vascular prosthesis [Vascutek Gelweave woven Dacron graft manufactured by Sulzer Vascutek USA, Inc., Austin, TX78752] cut into an oval shape measuring 3 cm × 3 cm using 4/0 polypropylene continuous suture technique. The aortotomy was closed with 4/0 polypropylene sutures reinforced with Teflon felt on either side. Aortic cross-clamp was released and aortofemoral shunt clamped. The total cross-clamp time was 65 minutes. Heparin was reversed with protamine. Decannulation was performed and the femoral artery repaired with 6/0 polypropylene continuous sutures. Due to dense adhesions present between the pseudoaneurysm, the esophagus and the hilum of the left lung, further dissection of the esophagus was abandoned. A Ryle's tube was introduced and its' position in the distal esophagus confirmed by palpation. Chest and the mediastinum were copiously irrigated and drained with two 36 F chest tubes. Patient maintained stable haemodynamics with adequate urine output and was able to move both lower limbs. He was kept nil orally for the next 5 days with continuous Ryle's tube aspiration. Total parenteral nutrition was begun in the immediate postoperative period and he was maintained on peri-operative antibiotic coverage of Vancomycin, cefpirome, Metranidazole and Amikacin. The per-operative culture from the thoracic cavity revealed no growth of organisms 48 hours later. On the sixth postoperative day gastrograffin esophagography revealed no esophageal leak. A contrast enhanced CT scan of the chest revealed neither aortic nor esophageal leak [Fig. , ]. Patient was encouraged to take sips of sterile water.\nThe patient was afebrile, tolerating soft diet and ambulatory on the ninth postoperative day when he was discharged from the hospital on an antihypertensive drug.
The patient was a 65-year-old woman without notable antecedents presented to our institution for progressive left hip pain for approximately 8 months. It was a mechanical pain of the hip well relieved by the usual analgesics. The appearance of walking distance and the poor response to analgesics forced her to consult in our center.\nThe BMI was 35,5. The walk was almost normal. There was no cutaneous scar on the lateral side of the left hip or on the ipsilateral buttock. There was a good trophicity of the abductors. Lateral rotation and abduction were markedly diminished. The rest of the exam was strictly normal. The pelvis AP () and lateral () left hip radiographs revealed signs of hip osteoarthritis. We concluded that it was a symptomatic left hip osteoarthritis that was more and more disabling in an obese woman of 71 years with no particular history. We indicated THA by posterolateral approach.\nIn the operating room, after the skin incision and subcutaneous haemostasis, we discovered in the adipose tissue about 5 cm thick a kind of well-circumscribed shell of about 2.5 cm of axis. Her incision gave rise to a whitish, thick color, looks a little oily collection (), resembling a purulent collection (). A sample for bacteriological investigation in a lab was carried out. The hull with its clear boundaries within the gluteal fat was resected and entrusted to the pathologist. All the neighborhood tissues were healthy (very localized lesion).\nIn front of this collection which appeared to be purulent, we limited ourselves to the resection of this hull, the cleaning of the wound, and the deferred implantation of the prosthesis.\nCytobacteriological examination of the specimen revealed its greasy appearance, epithelial and lymphocytic cells; there were no visible germs. Histological examination of the resected shell revealed a fibrous wall with chronic inflammatory remodeling made of lymphocytes and plasma cells with no necrosis centers.\nIn the light of these laboratory results, we conducted the interview of the patient, who reported a notion of malaria for about two months to the screen treated with an intramuscular injection on the right buttock of the compounds derived from artemisinin. We found the result of the thick drop before the injection which was positive and that of the injection which had not been negated; the patient was then successfully treated orally. The sample was sent to a lab for confirmation by artemether identification by thin layer chromatography (TLC).\nA sample of 40 g of human fat was treated with ethyl acetate (50 ml × 3) after filtration on Whatman paper, the solvent was evaporated, and the residue was taken up with acetone (40 ml) constituting the sample to be analyzed. Artemether was purchased from a local pharmaceutical company.\nImplementation of the TLC: solution to be analyzed: 20 μl of sample; control: artemether (80 mg/mL), 10 μl deposit; support: silica gel GF254; mobile phase (10 ml): dichloromethane, ethyl acetate (7/3); and developer: 25 ml anisaldehyde reagent, 5 ml concentrated acetic acid, 450 ml ethanol, and 25 ml concentrated sulfuric acid. Using a capillary tube, 20 μl of the sample was deposited on the plate (silica gel GF254), the control 10 μl. The plate is placed in a tank previously saturated with the migration or elution solvent (mobile phase) which covers the bottom of the tank at 5 mm height. The migration of eluting solvent causes the substances contained in the samples at various speeds; spots are formed characterizing the substances present in the sample.\nThe plate was removed from the tank as soon as the solvent front reached about 9 cm. The plate was dried and observed under a UV lamp at 254 nm and then revealed with the developer which will characterize the artemether in human fat.\nThe plate then shows an orange spot on the left side of the sample and a spot with the same color on the right side of the control; the two spots have the same front report as shown in . This indicates that there was artemether in this human fat sample.
A 70-year-old woman complaining of transient numbness in the upper right and lower limbs was referred to our hospital. Physical and neurological examinations revealed no definite abnormalities. However, MRI indicated a large mass lesion in the posterior fossa. The maximal diameter was 22 mm. The mass severely compressed the medulla oblongata and caused a high signal intensity lesion, suggesting a vasogenic edema. Both T1- and T2-weighted MRI showed a flow void signal and mixed signal intensity area inside the mass, suggesting a partially thrombosed vertebral aneurysm (fig. ). MRA disclosed a thrombosed large aneurysm of the right vertebral artery (VA) (fig. ).\nThe proximal VA was exposed through right suboccipital craniotomy and was clipped with one titanium aneurysm clip in order to reduce the pressure inside the aneurysm. The postoperative course was uneventful. No neurological deterioration occurred. Postoperative MRA showed the disappearance of the flow void signal inside the aneurysm. One month later, however, cerebral angiography revealed a partial recanalization of the right VA, probably because one titanium aneurysm clip was not enough to obliterate the sclerotic VA. She then underwent coil embolization of the right VA just proximal to the aneurysm clip. Subsequently, the right VA was completely obliterated. She was discharged without any neurological deficit. However, a couple of weeks later she complained of mild hoarseness and right shoulder pain. Physical and neurological examinations showed the atrophy of the right trapezius and sternocleidomastoid muscle, leading to the deepening of the right supraclavicular fossa (fig. ). No other cranial nerve deficits were observed. The symptoms were considered to result from right isolated accessory nerve palsy. Radiological examinations showed no newly developed lesion such as cerebral infarction in the brain stem. There was no significant change in size of the VA aneurysm. As a result, the isolated accessory nerve palsy was considered to be a delayed complication due to aneurysmal compression on the intracranial portion of the accessory nerve.\nFollow-up MRI showed that the VA aneurysm gradually decreased in size over a period of several months. The deformity of the medulla oblongata markedly improved and its high signal intensity lesion completely disappeared (fig. ). At the same time, the patient's hoarseness completely resolved. The deepening of the right supraclavicular fossa also disappeared completely (fig. ).
A 42-year-old male presented to the Department of Head and Neck Surgery of a tertiary oncological centre because of right mandibular swelling and trismus. The patient had already been admitted to a secondary care hospital 3 months earlier with complaints of right mandibular discomfort and slight tumefaction. A biopsy was then performed and the diagnosis of ameloblastic carcinoma was made histologically. The patient was otherwise healthy with no significant past medical history, including alcohol, smoking or tobacco abuse.\nA complete head and neck examination revealed a painless, firm and fixed right mandibular mass with no cutaneous inflammatory signs. No ulcers or mucosal lesions were found in the oral cavity. Laboratory evaluation, chest radiograph and respiratory function tests were unremarkable. The patient underwent bronchofibroscopy, which revealed only mild laryngeal hyperaemia.\nBoth neck CT () and MRI () were performed, showing a large, solid tumour arising from the ramus and posterior body of the right mandible. The lesion extended to the surrounding soft tissues, with invasion of the masseter and medial pterygoid muscles and caused bulging of the buccal mucosa. The soft tissue component was hypointense on T1 weighted and hyperintense on T2 weighted MR images and showed avid enhancement after gadolinium administration on MR examination. CT scan disclosed striking sclerosis and irregularity of the mandibular ramus with some gas bubbles inside the medullary cavity and an expansive lytic component in the posterior body and angle with some bone-forming matrix inside. Prominent periosteal reaction was also identified, particularly in the outer cortical surface of the mandibular ramus with the typical pattern of a ruptured Codman triangle. No associated cystic lesion was found in the mandible. No enlarged lymph nodes were detected and the evaluation of the remaining cervical spaces was unremarkable. A thoracic CT scan was also performed, with no parenchymal lesions.\nThe patient underwent a right hemimandibulectomy and ipsilateral cervical lymph node dissection. Surgical resection also included the right submandibular gland and a segment of buccal mucosa that was swollen by the mandibular mass. Reconstruction was performed with free fibula graft. The surgical specimen included a large, white and solid tumour with 11 × 7.5 × 6.5 cm, corresponding to an invasive, moderately differentiated (G2) SCC (). Focal positive margins were found at the medial surface of the specimen. The resected buccal mucosa, submandibular gland and lymph nodes had no neoplastic tissue. Taking into account the imaging staging examinations and the post-surgical histological report, the final TNM stage was stated as pT3 N0 Mx. After surgery, the patient underwent adjuvant chemotherapy (cisplatin-based regimen) and radiotherapy.
A 42-year-old female patient presented within 1 h of receiving direct trauma to her maxillary area. The medical history of the patient revealed no systemic disease. Clinical examination revealed that the right and left maxillary central incisors presented mobility (grade 2) and sensitivity to percussion and palpation but no sensitivity to thermal stimulations. Occlusal displacement with extrusion in the left maxillary central incisor and luxation in the right maxillary central incisor was observed. The crown of the left maxillary central incisor was slightly dislocated in the buccal direction. The remaining anterior incisors were clinically normal, and no signs of alveolar bone fracture were detected; however, gingival bleeding was present ().\nTwo periapical radiographs with different vertical angles of the maxillary anterior teeth and an additional occlusal radiograph were taken. Radiographic examination revealed horizontal root fracture at the apical third of the left maxillary central incisor. Root fracture in the right maxillary incisor was not observed. The periodontal spaces of both maxillary central incisors were enlarged (Figures and ).\nThe treatment plan comprised reduction, repositioning, and rigid splinting of the coronal fragment of the left maxillary central incisor. Following administration of local anesthetics, the coronal fragment was repositioned with digital pressure and the success of repositioning was confirmed radiographically before the rigid splint was applied with composite resin material (Filtek Supreme XT, 3M Espe, USA) (). The patient was instructed to maintain good oral hygiene, follow a soft diet, and avoid masticating in that area. Right and left maxillary teeth were examined at 2-week intervals for 2 months.\nAt the end of the second month, the patient presented with a complaint of severe pain right and left maxillary teeth. Clinical examination revealed that these teeth showed pain to percussion and palpation. Sensitivity to thermal stimulations was not observed. It was decided to continue with endodontic treatment.\nAfter isolating the teeth with a rubber dam, the access cavities were prepared, the necrotic pulps were extirpated, and the working lengths were obtained. Apex locator (Morita, Kyoto, Japan) was used in determination of the working length and was confirmed by radiographs. Left and right maxillary central incisors working lengths were, respectively, 17 and 20 mm. The root canals were prepared with nickel titanium rotary system (ProTaper Universal, Dentsply Maillefer, Ballaigues, Switzerland) until the finisher F4 instrument was completed. 5.25% sodium hypochlorite (NaOCl) irrigation solution was used for the cleaning and shaping procedures. After instrumentation, the root canal was flushed with 5 mL of 5.25% NaOCl and 5 mL distilled water, respectively, and dried with paper points. Calcium hydroxide paste was applied in the root canal space and the cavity was temporarily filled with temporary restoration (Cavit-G, 3M Espe, USA). The patient was scheduled for follow-up after 2 weeks.\nAt the next visit, clinical and radiographic examinations revealed no signs of pulpal infection in either of the maxillary incisors; therefore, the root canals were prepared for obturation. Composite resin splint was removed from teeth. Then the calcium hydroxide paste was removed from the root canals and MTA (ProRoot MTA, Dentsply Maillefer, Ballaigues, Switzerland) was prepared according to the manufacturer's instructions. An endodontic plugger adequate (American Eagle, Missoula, MT, USA) for the length of the root canal was used and the stopper was fixed 1 mm short of the working length. MTA was inserted into the canal with Messing gun and compacted further with the plugger. Then a moistened cotton pellet was placed on the MTA surface, and the endodontic access cavity was filled with temporary restoration (Cavit-G, 3M Espe, USA) (). During the following visit, the cotton pellet was removed, and the remaining portion of the tooth was filled with composite resin. Next, the patient was referred to a restorative clinic.\nThe clinical oral examination for restorative treatment revealed the following aesthetic and appearance problems: malposition and displacement in left maxillary central incisor, unsuitable occlusion, unsuitable proportions and size of teeth, lingualisation of right maxillary canine and left maxillary first premolar teeth, absence of left mandibular central incisor, mal-texture and unsuitable appearance and colour of the teeth, and unequal and disproportionate gingival zenith level size of teeth (Figures , , and ).\nResin build-up and composite laminate veneers were planned for treatment of the mentioned aesthetic problems. Before any aesthetic treatments, all caries were removed and cavities, including endodontic coronal cavities, were restored with composite resin restorations (Filtek Supreme XT, 3M Espe, USA).\nA wax model was fabricated, establishing an ideal incisal edge position and form. This ideal wax model defined the final three-dimensional tooth positions. A silicon index was made using the study cast to determine the index during composite treatment (Figures and ).\nLeft maxillary central incisor was treated using composite build-up without an endodontic post. The other teeth were treated with direct composite veneer restorations performed using a polychromatic composite layering technique. Because A2 was the final restoration shade selected, the A3D shade (Filtek Supreme XT, 3M Espe, USA) was used to give the restoration a natural depth of shade (chroma) in the core of the filling. A2B and A1E shades (Filtek Supreme XT, 3M Espe, USA), which have medium transparency and chroma, were layered over this dentine shade to produce natural-looking shades. The C shade (Filtek Supreme XT, 3M Espe, USA), which has the greatest transparency and the least chroma, was used in combination with enamel and dentine shades to imitate the incisal edges. The buccal arch was labialised to achieve a better appearance of the buccal arch and reduce the carvings in the surface of the teeth. Pink coloured composite resin (Amaris Gingiva, Voco, Germany) was used to maintain the gingival embrasures and the gingival architecture of left maxillary lateral and canine.\nA composite Maryland bridge cemented with dual cure composite resin cement (Panavia F2.0, Kuraray Medical Inc.) was used to permanently replace left mandibular central incisor and temporarily replace right maxillary first premolar. To improve the surface texture, the restorations were finished and polished with a mixture of coarse diamonds (837LH014, Meisinger, Germany), composite finishing carbides (HM245012, HM244010, Meisinger, Germany), and various grits of polishing discs (Sof-Lex, 3M Espe, USA) (Figures , , and ).\nEndodontic and aesthetic restorative treatments were completed, and the patient's oral hygiene motivation was improved significantly (Figures and ). The clinical condition of the restorations and the patient's oral hygiene were in good condition at the 15-month follow-up examination ().
The patient is a 15-year-old Caucasian female who was diagnosed with CRMO in 2007 at 5 years in the context of right thigh pain. provides a timeline of the patient's symptoms and course of disease. Magnetic resonance imaging (MRI) showed multifocal abnormal bone marrow signal in the right femur, the left femoral neck, and the proximal epiphysis and metaphysis of the right tibia, which was associated with osteolysis and callus formation (). Radio-nucleotide bone scan found increased uptake involving the left sacrum, left proximal femur, and femoral neck region as well as the midshaft of the right femur and the proximal right tibia. Bone biopsy of the lytic lesion involving the midshaft of the right femur was not consistent with malignancy and showed red blood cells and scattered neutrophils and lymphocytes. Over the next several years (2007–2015), she was followed by orthopedic surgery and was treated only with intermittent ibuprofen as needed for pain. She was noted to have a leg length discrepancy at the age of 7 years, and in 2014, at the age of 12 years, she had surgery to fuse the growth plate to prevent right leg growth (right leg was 4.5 cm longer than her left leg at that time).\nShe established Rheumatologic care in March 2015 at age 13, and right quadriceps muscle atrophy and failure to thrive with a weight and height under the third percentile were noted at this time. Bone scan revealed increased uptake in the right femur and asymmetry of activity in the growth plates of the knees and ankles with decreased activity in the right side compared to left. She was treated with naproxen 250 mg twice daily (8.7 mg/kg BID) and prednisone 20 mg per day (0.7 mg/kg), tapered by 5 mg weekly. Two months later, prednisone was discontinued, and she continued to have good control of her leg pain on NSAID monotherapy. After a few months, she developed new diarrhea with vomiting and weight loss. Her blood pressure was elevated, and a renal ultrasound and electrocardiogram were unremarkable.\nShe was referred for Gastroenterology evaluation and was found to have positive stool occult blood along with a perirectal skin tag. Laboratory studies revealed anemia with a hemoglobin of 7.1 g/dL (nl 12–16 g/dL), thrombocytosis with platelets of 744 k (nl 150–450 k), erythrocyte sedimentation rate (ESR) of 69 mm/hr (nl 0–10 mm/hr), and C-reactive protein (CRP) of 129 mg/L (nl 0–3 mg/L). There was no family history of autoimmune disease, and tuberculin testing was negative. Colonoscopy revealed pancolitis with crypt inflammation and crypt abscesses with no granuloma consistent with ulcerative colitis (). NSAID therapy was discontinued, and treatment for UC with prednisone 1 mg/kg/day (40 mg) tapering by 5 mg weekly along with sulfasalazine was initiated. Infliximab 3 mg/kg infusions were added the following month.\nAt the visit for her second infliximab infusion, she presented with tachycardia, a blood pressure of 230/190, and headache and was admitted to the pediatric intensive care unit for hypertensive emergency requiring nicardipine infusion. Her examination was notable for right-sided Horner's syndrome, decreased right leg pulse pressure, and an abdominal bruit. Echocardiogram showed a small pericardial effusion with reduced left ventricular ejection fraction. Laboratory studies revealed negative ANA and ANCA screens, normal C3 and C4, and normal von Willebrand factor antigen. CT angiogram (CTA) of the abdomen and pelvis showed narrowing of the mid-aorta, proximal renal arteries, celiac artery, and superior mesenteric artery (Figures –). CTA of the chest showed marked descending thoracic and abdominal aortic wall thickening with progressive luminal narrowing and wall thickening of the visualized portion of the right common carotid artery and celiac trunk, enlarged left atrium, left ventricular hypertrophy, and a small pericardial effusion (). MRI/MRA brain showed anterior and posterior circulations of the brain were without occlusion or aneurysm with the patent carotid and vertebral arteries of the neck (Figures and ). Given these findings, she was diagnosed with TA complicated by middle aortic syndrome. She was started on metoprolol 50 mg daily (1.5 mg/kg daily), amlodipine 5 mg daily, famotidine 20 mg twice daily, aspirin 81 mg daily, and increased dose and frequency of infliximab from 3 mg/kg every 8 weeks to 5 mg/kg IV every 4 weeks along with prednisone 20 mg daily (0.6 mg/kg daily) and mesalamine 1 g BID. The patient continued infliximab every 8 weeks rather than every 4 weeks as recommended.\nMagnetic resonance (MR) angiography of the chest, abdomen, and pelvis and Cardiac MR three months later showed luminal narrowing of the distal thoracic and upper abdominal aorta similar to previous CT studies, stenosis of origin of celiac axis, stenosis of proximal superior mesenteric artery (SMA), and moderate stenosis of bilateral proximal renal arteries. Echocardiogram noted concentric LVH with mildly reduced function, measuring 45%, and normal coronary arteries.\nAt this point, the patient transferred her care to our rheumatology clinic, and despite treatment with infliximab 5 mg/kg every 8 weeks, she continued to report right thigh pain and developed new inflammatory arthritis of the left ankle and increased inflammatory markers with an ESR of 55 mm/hr (nl 0–20 mm/hr) and an elevated CRP of 73 mg/L (nl < 3 mg/L). Additionally, interval imaging found new wall thickening around the right common carotid artery. This was concerning for uncontrolled TA and CRMO activity. There was an unfortunate delay in treatment escalation due to social circumstances. Two months later, she started treatment with parenteral methylprednisolone 1 gram weekly for 8 weeks, and her dose of infliximab was increased from 5 mg/kg to 10 mg/kg every 4 weeks. Based on adult data demonstrating a positive response to higher doses of infliximab, the decision was made to increase the dose of infliximab instead of trying another TNF inhibitor.\nMultidisciplinary evaluation at Boston Children's Hospital, Center for Middle Aortic Syndrome by neurosurgery, nephrology, and rheumatology, led to recommendations of a prednisone dose increase and the addition of methotrexate 15 mg/m2 weekly to infliximab 10 mg/kg every 4 weeks. At this time, her echocardiogram revealed moderate left ventricle dilation and mildly depressed left ventricular systolic function. One month later, repeat head and neck CTA showed progression of her right carotid artery stenosis to 80%. Her ESR had normalized, and CRP decreased to 7.8 mg/L at this time. Given her worsening carotid artery stenosis, infliximab was increased from 10 to 15 mg/kg every 4 weeks. On a combination of moderate dose prednisone, weekly methotrexate, and infliximab, she denied joint pain, swelling, abdominal pain, diarrhea, or blood in stool. Follow-up brain MRI/MRA three months later was normal. Repeat chest and abdomen MRA showed all of the areas of stenosis appeared to be stable and inflammatory markers had normalized.\nCurrently, her clinical course is stable without further anatomic progression, and she has normal inflammatory markers. Thus, she has continued on her current regimen with infliximab 15 mg/kg every 4 weeks and methotrexate 15 mg/m2 once weekly. Prednisone decreased gradually and discontinued. She has not developed any infectious sequelae on this regimen. Her blood pressure is stable on carvedilol alone. We continue to assess blood work monthly. Given her use of prednisone, vitamin D level was followed and found to be low, and she has started on a vitamin D supplement. Dilated ophthalmologic exam was normal. Repeat MRI of the lower extremities and bone scan show that her CRMO lesions are inactive. Repeat MRI/MRA of the brain, chest, abdomen, and pelvis in June of 2018 show stable changes without the need for stenting. Given the predisposition for autoinflammatory conditions in this patient, the authors are considering genetic testing in search of a monogenic cause that may support a unifying diagnosis.
A 13 year old female patient is referred to an emergency service with a mostly bilateral retroocular headache after 2 years of evolution. the pain increased in frequency and intensity for two months which caused the patient to woke up in the mornings. Due to this, in the remission site, a cranial computed tomography (CT) scan was performed. It showed an infratentorial lesion, so the initial emergency service physician sends us the clinical record and the patient for integral management. When the patient came, a cranial nuclear magnetic resonance imaging (MRI) was performed [Figures –].\nA large infratentorial mass with robust enhancement, heterogeneous dilation of the lateral ventricles, and other signs of hydrocephalus such as horizontalization of the temporal horns was found. Taking into account the patient's findings and symptoms, it was considered necessary to perform a tumor resection through occipital craniectomy. During the procedure, we did not have any complications []. Furthermore, considering the high risk of hydrocephalus, we performed a system of the fifth ventricle. The patient was sent to the pediatric intensive care unit.\nThe outcome of the 1st day became torpid, with a high rate of production of xanthochromic cerebrospinal fluid (CSF), for which it was considered necessary to perform a ventriculoperitoneal shunt.\nAfter several days of satisfactory evolution, the patient became drowsy again, for that reason it was considered necessary to perform a new cranial CT scan [], in which significant ventricular dilatation was evidenced. With a diagnosis of hydrocephalus due to shunt failure, the patient was taken back to surgery.\nIn the surgery, it was found a high quantity of detritus in the porosities of the proximal catheter shunt, so a review of its functionality and a cleaning was performed. Two hours after the procedure, the patient was awake without neurological deficit. A control CT scan was performed on the 3rd day [].\nFifteen days postoperatively, the pathology reported choroid plexus papilloma, and after a follow-up at 3 months, 6 months, and a year later, the patient did not present any clinical deterioration or complication.
Mrs D was a 36-year-old female patient diagnosed with metastatic triple negative breast cancer (stage T4b N3 M1). The patient had undergone 7 cycles of chemotherapy before being referred to and managed by the pain and palliative care team. Metastases had spread to her cervico-dorsal, lumbar and sacral vertebrae and bilateral pelvic bones. On presentation to the pain control out patients department (OPD) she had severe pain in her head and neck, and was admitted to the inpatient palliative care unit for analgesic titration and control of nausea and vomiting.\nThe patient was already on anti-sickness medication (Ondansetron); further management of nausea and vomiting included the introduction of a proton pump inhibitor (Pantoprazole) as well as advice on diet and patterns of eating.\nThe management of the patient's pain was more complex, and can be framed in the context of total pain [].[]\nPhysical pain control was managed as per the WHO pain ladder.[] The patient was already on step 3 of the ladder, so her morphine dose was increased from 20 mg every 4 h to 30 mg every 4 h. Due to her head and neck pain, an MRI was done. This was discussed at a cross-disciplinary meeting with the radiologists to clarify what the progression of the disease was and what would be the most appropriate way to move forward with management. Although the progression of disease was such that treatment was no longer an option, a decision to give radiotherapy was taken as a palliative measure in order to prevent spinal cord compression and its complications, as well as to relieve pain and preserve function.[] As an adjunct to this, the physiotherapists provided advice on movements of the head and neck, and also a neck brace.\nThe patient's social problems had started when she first fell ill. She had to leave her job of making clothes and therefore lost some of the family income. Her brother and husband were also missing work to bring her to the hospital and spend time with her there, resulting in a further loss of income. Housing problems arose when the family's landlord found out that the patient was in the terminal stages of illness and asked the family to vacate the property. The children went to stay with another family but the patient was left without a home to go to once she was discharged from the palliative care unit. In this situation a non-governmental charity organization was called upon, who run a hospice. The hospice provides care for patients who have nothing further to benefit from cancer treatment, providing symptomatic care for pain and other distressing cancer symptoms (nausea, restlessness, and respiratory problems).\nPsychological pain was clearly evident upon speaking with the patient, and support was provided by all members of the palliative care team. I found there was an emphasis on communication and empathy above and beyond what is seen in other medical disciplines in the hospital. The patient's family (daughter, brother and husband) also spent lots of time with her in the hospital and were an ongoing source of psychological support.\nI did not witness any interactions with health professionals in the hospital that were explicitly spiritual. However, the approach taken when discussing the patient's prognosis and coming to terms with things implicitly involved some level of spirituality, to a greater degree than is common in the UK. The hospice has more space for spiritual matters; spiritual counselors or leaders can come to the hospice to see the patients, with each religion given equal respect. The hospice state that the sisters themselves interact spiritually with the patient whenever needed and is possible, while maintaining that there is strictly no indoctrination.\nThe care of the above patient involved doctors from different specialties (anaesthetics, medical oncology, surgical oncology, radiology), as well as other health professionals (nurses, physiotherapists), health professionals working in other sectors (NGOs), and also the patient's family who play a key role in palliative care in India. I will discuss the roles and interactions of different members of the team in turn.\nDoctors from different specialties worked closely with each other, sharing discussions on ward rounds and between times, so that there was collaboration rather than disciplines working independently on what they believe to be their remit of a patient's care. There was also a smooth system in operation between the OPD management of pain, and the small (6 bed) ward that was reserved for the most urgent cases of pain control. When patients presented to the OPD, if they were suffering from pain that could not be remedied with a simple change in prescription, they were admitted to the ward for titration of analgesics or for an episode that required some other management in the hospital.\nThe different health professionals worked side by side. It was a hierarchical system, with doctors being in charge of patient care and nurses and physiotherapists involvement arising as instructed by the doctor, but the role of the physiotherapist and nurses was highly valued by the doctors. In outpatient clinics the different professionals were working in the same room so could work closely together.\nInvolvement of the NGO sector was complementary to the care given at the hospital. Rather than being two separate entities, the government hospital and NGOs worked together. In the above case, the hospital suggested hospice care and the suitability of this was considered between the doctors, the hospice, the family and the patient. Hospices are far less common than in the UK, and the doctors of this hospice described themselves as acting in a way as a safety net, providing care for people who did not have the option of family care at home as per the norm for Indian society.\nIn other cases, I saw a further role for NGOs. There was another NGO that provided home support to patients being cared for by their families. Contact with the NGO was usually made by doctors from hospitals, and many patients were both receiving home visit care and visiting outpatient pain clinics. The home support team was able to spend more time with individual patients in their own environment, and also provide practical and emotional support to the families. For this they had a counselor in their team as well as a doctor and a nurse. Because of this emphasis there was a less hierarchical structure, with many patients requiring the counseling services over and above the medical ones, and doctors were not present on all visits. There was communication between the home team doctors and leads of the hospital pain unit, which ensured that both services were operating with the same principles and allowed discussion of difficult cases.\nThe home care team also collaborated with the hospice, keeping it as an option to suggest for patients or families who were not coping well at home. The role that the home care team NGO provided is somewhat analogous to the Macmillan nurses in the UK. Macmillan nurses are also funded partly by charity, although after a Macmillan nurse role has been operating for three years, the National Health Service takes over the funding. A systematic review of studies from western countries showed that home care can reduce symptom burden as well as making patients more likely to die at home (as many of them wish). The set up is very different in India to that of the western countries in the study, but it shows the potential value of such home care teams, particularly where even more focus is on families caring for dying patients in the home.[]\nIn palliative care worldwide the importance of the family is emphasized.[] In India this importance is manifold, as most patients are cared for in the family home, and die at home. The family then take on a role not just as part of the patients' social and emotional life, but also as carers and therefore part of the healthcare team. The daughter of the patient discussed was doing most of the caring for the mother, cooking and cleaning in the house and helping her to get around as she needed. This requires a different kind of collaboration with families, involving education as to the patients' physical needs as well as emotional.\nLinked with this central role of the family in India seemed to be a subtly different reality with respect to the four pillars of medical ethics (autonomy, beneficence, non-maleficence, justice).[] In the UK there is a strong emphasis on autonomy, and patients' right to knowledge about their health and disease as well as decisions arising from this.[] Indian law also emphasizes autonomy and consent, but in clinical practice there is sometimes a different reality, with patients not always aware of their diagnosis or prognosis.[] The philosophy of the palliative care team I was working with was to be open with the patients about their disease status and prognosis and to encourage them to accept this, often addressing these issues with patients for the first time. Even within this strong culture of openness there were still some cases in OPDs where families wanted to shelter their relatives from this information and the family's wishes were upheld. The above patient had full knowledge of her condition and prognosis, and the doctors took time with the relatives when the patient was in the ward to discuss the prognosis and difficulties in uncertainties of not knowing exactly how long the patient had to live.\nDuring my time at IRCH it was World Palliative Care day. Many patients and relatives of patients told their stories of being cared for under this palliative care team. The overall theme to come out of their accounts was the positive effect on both the patients and the families' experience of the end of life. An emphasis on the human aspects of the care received was made, so it is clearly not just the imposition of a structure of individuals from different disciplines working well together, but the nature of those individuals which may be a more difficult or more gradual thing to instill in a department.
A 32-year-old woman presented to the clinic with fever of unknown origin and a progressively increasing cough for the past 3 months. She also had hoarseness of voice and complained of odynophagia for the past 3 weeks.\nFurther history revealed that her fever had started intermittently approximately 10 months ago. A month later, she became pregnant but it was an ectopic pregnancy that ruptured after 6 weeks. She was admitted to the hospital and underwent a laparoscopic exploration. Cultures taken from the fallopian tube were positive for Mycobacterium tuberculosis. During her 10 day hospital stay, she developed sepsis and an adult respiratory distress syndrome. She was shifted to Intensive Care Unit and a central line was placed in her left subclavian vein. Her tracheal cultures revealed growth of Pseudomonas aeruginosa. Her blood cultures revealed the presence of Enterobacter cloacae. Bronchial lavage confirmed diagnosis of pulmonary tuberculosis (TB) and the absence of any malignancy. Antibiotics and anti-TB medications were started. The patient subsequently improved and was discharged home.\nThe patient was a mother of two children, both born of vaginal deliveries without complications. She had no other comorbidities and had no family history of TB.\nShe presented again 2 months later complaining of vomiting, headache, exertional dyspnea, inability to talk, and difficulty opening left eye. There was also a history of significant weight loss over the past month. A brain magnetic resonance imaging revealed disseminated central nervous system TB. Her anti-TB regiment was adjusted by increasing the number of drugs. She was noted to have a left-sided hemiparesis but gradually recovered and was discharged home.\nApproximately 2 months later, the patient represented with a complaint of hoarseness of voice and odynophagia. A chest radiograph revealed a superior mediastinal mass on the left. A follow-up CT scan of the chest showed a large subclavian artery pseudoaneurysm measuring 74 mm × 57 mm size on the left not involving the aortic arch but adjacent to the aortic knuckle. A CT angiogram [] confirmed a large left subclavian artery pseudoaneurysm.\nHer hoarseness and odynophagia were attributed to compression effects of the aneurysm on the trachea, esophagus, and recurrent laryngeal nerves. It was also noted that her radial artery blood pressures on the right side were higher than that on the left. Upon speculation, cause of the aneurysm could have been because of either TB or injury by central venous pressure line.\nThe preoperative diagnosis was left subclavian artery pseudoaneurysm possibly mycotic or related to central line injury.\nRepair of the aneurysm was performed through a left posterolateral thoracotomy incision and the fourth intercostal space was entered. Pleural adhesions were excised and a large mass was visible. CT angiogram had revealed a 1 cm distance of the mass's origin from the aorta so dissection around left subclavian artery was done to achieve the proximal control. The aneurysmal sac was opened and found to be a pseudoaneurysm []. Distal control was achieved. A pericardial patch was used to repair the 3 cm longitudinal defect in the left subclavian artery wall. Multiple mediastinal lymph nodes and the excised wall were sent for histopathology. Lymph nodes were reported as showing chronic granulomatous inflammation without necrosis (most likely due to Tb) and were negative for fungal hyphae or any malignancy. The excised wall showed a completely necrosed tissue.\nThe patient tolerated the procedure well. She had an uneventful recovery and was discharged within a week. She was followed for another 2 months and was doing well when last seen.
We report the case of a 12-year-old boy who was referred to the pediatric clinic at our institution with a history of painful oral and genital ulcers for one month before the presentation. The patient was seen by several general practitioners for stomatitis and was prescribed symptomatic treatment but showed no improvement. He had an unremarkable past medical history. No family history of autoimmune diseases was noted.\nSince the patient was not tolerating oral feeding, he was admitted for management and further investigation. He was started on broad-spectrum antiviral and antifungal therapy for two weeks. Considering that the patient had difficulty swallowing, he underwent an upper gastrointestinal endoscopy, which revealed severe gastroesophageal reflux disease with gastric and esophageal ulcers. Subsequently, he underwent colonoscopy, which had signs of mild colitis. In light of the clinical and endoscopic findings, a diagnosis of Behçet’s disease was made. Hence, the patient was started on colchicine and systemic corticosteroid for two weeks. No improvement was noted, and the patient lost around 15 kg since the disease onset. Further laboratory investigation revealed normal basic hematological and biochemical profiles. However, he had an elevated erythrocyte sedimentation rate (77 mm/hour) and mild elevation of fecal calprotectin. The viral serology panel, including Epstein-Barr virus, cytomegalovirus, and human herpesvirus 8, was normal. The patient’s condition progressed, and he developed multiple crusted hemorrhagic ulcers covering the oral mucosa (Figure ).\nDuring the hospital course, the patient developed severe abdominal pain associated with nausea and vomiting. Abdominal ultrasonography revealed a large lobulated hyperechoic mass lesion compressing the gallbladder. An abdominal CT scan was performed for further evaluation of this mass. It demonstrated a mass lesion located superior to the kidney but was not crossing the midline. There was no evidence of thrombosis or lymphadenopathy (Figure ).\nBiopsies were taken from the buccal mucosa and the abdominal mass by an image-guided procedure. Histopathological examination of the buccal mucosa revealed a benign salivary gland with a sparse chronic inflammatory cell infiltrate. The specimen obtained from the abdominal mass showed a benign lymphoid tissue with no evidence of malignancy. The patient underwent laparotomy for the resection of the abdominal mass. Histopathological examination of the mass was consistent with the diagnosis of unicentric Castleman’s disease. Despite the surgical resection of the mass, the patient’s symptoms persisted. Subsequently, the patient was given intravenous methylprednisolone (20 mg/kg) for five days in addition to azathioprine, which showed remarkable improvement (Figure ).\nThe medications were tapered gradually over a period of two weeks. The patient was discharged in a good condition on low-dose prednisolone and cyclosporine. He sustained remission for 18 months without any disease flare.
A 19-year-old male patient reported to an outpatient Department of Oral Medicine and Radiology in a dental college and hospital, with a 2-month history of rapidly enlarging painful swelling in the left cheek region. There was a history of trauma on the left side of the face before a couple of months due to an external force and had immediate pain. A small swelling was noticed around 15 days later by his friends and neighbors, and this gradually grew to reach the present dimension. The swelling seems to increase in size after waking up from the bed and while bending downward and mildly reduced in size while under heavy work. The patient consulted various local physicians, and there was no reduction in the size of the swelling in spite of taking medications prescribed by them. His past medical, surgical and dental history was not contributory.\nOn extraoral examination, there was a diffused swelling on the left side of the face [], which measured about 8 cm × 6 cm that extended superoinferiorly from temple region to 1 cm short of the base of the mandible and anteroposteriorly 1 cm short of ala of the nose to tragus of ear, which was mildly warm, tender and had variable consistency, i.e. firm to hard in center and soft to firm at the periphery. The right submandibular lymph node was palpable and tender. On intraoral soft-tissue examination, erosive areas were noted on the posterior part of buccal mucosa and vestibule from 25 to 28 regions and obliteration of buccal vestibule with tenderness evident in the 25–28 regions. On hard-tissue examination, grade I mobility was present from 24 to 27.\nRoutine hematological investigations were found to be within the normal limits.\nUltrasonogram [] of the left cheek showed a hypoechoic focus of size 4 cm in intramuscular plane in masseter muscle extending up to the buccal space. Minimal color uptake was noted on color Doppler.\nContrast-enhanced computed tomography (CT) [] showed a lobulated, 6 cm soft-tissue dense mass in the left masticator space, abutting lateral pterygoid and masseter muscles, with extension craniocaudally from the infratemporal fossa to the buccal space, anteriorly to the lateral wall of orbit and zygomaticus major, medially to the pterygomaxillary fissure and para maxillary space; posteriorly, it is confined within the masseter muscle. The mass showed heterogeneous enhancement with minimal central necrosis in the inferior aspect. Bowing of lateral wall of the left maxillary sinus was noted (Holman–Miller sign).\nMagnetic resonance imaging (MRI) [] performed a month later showed a relatively well-defined 7 cm isointense mass with T1-weighted images, isotense to hyperintense in T2-weighted images and short tau inversion recovery (STIR). The epicenter and extent of the mass were almost the same as noted in CT. There were multiplanar linear flow voids within the lesion. Postcontrast, lesion shows intense heterogeneous enhancement. On digital subtraction angiography, the left superficial temporal artery was prominent and few branches from it supplied the lesion.\nFine-needle aspiration was nondiagnostic as it showed only formed elements of blood.\nAfter obtaining the consent, photographs were taken and diagnostic and therapeutic excision was executed on July 2015 under general anesthesia through extraoral approach, and the mass was removed in toto by applying countertraction [Figure –]. Owing to massive bleeding which was met intraoperatively of about 300 ml, a unit of fresh blood transfusion was made. Postoperative recovery was uneventful.\nHistopathological examination showed parts of tumor composed of thin- and a few thick-walled ectatic vascular channels lined by attenuated endothelial cells set in a fibroblastic stroma composed of uniform spindle-shaped cells. There is no increase in mitotic figures or evidence of atypia [Figure and ], and the features were compatible with the pathohistological diagnosis of angiofibroma.\nImmunohistochemistry showed positive for vimentin [], and CD 34 highlights the vascular channels [].\nThe patient is under regular follow-up and no recurrence was noted.
A 69-year-old female was referred to Samsung Medical Center for progressive dyspnea and chest discomfort. A year before her referral, she had developed exertional dyspnea and general weakness. She had been diagnosed with severe aortic stenosis with regurgitation, moderate mitral regurgitation, and severe tricuspid regurgitation (TR) before presenting at Samsung Medical Center. Her medical history revealed that a single chamber transvenous permanent pacemaker (VVI mode) had been implanted 14 years prior due to sick sinus syndrome. Repeat echocardiography confirmed severe aortic stenosis, moderate aortic regurgitation, moderate mitral regurgitation, and severe TR. On her aortic valve leaflet, thickening, calcification, and retraction with motion limitation were present. The aortic valvular area was 0.87 m2 and the mean pressure gradient was 41 mmHg. In addition, the posterior mitral valve leaflet showed limited motion accompanied by moderate mitral regurgitation. These findings suggested a rheumatic etiology of the dysfunction in the two valves. However, the severity of this patient’s TR was not fully understood, since only annular dilatation secondary to left-sided lesions was observed (). A pacemaker lead around the tricuspid valve was found on echocardiography, but we were not able to determine its significance. Her severe TR was seemed to be related to coaptation failure in the tricuspid valve, but the coaptation failure was not serious enough to explain the severity of the TR. Further surgical analysis of her tricuspid valve was necessary. Nevertheless, echocardiography results suggested triple valve disease, for which surgical correction was indicated.\nThere were no abnormal values in her preoperative blood tests, but electrocardiography showed atrial fibrillation with a slow ventricular response and an intermittent ventricular pacing rhythm. Her native heart rate was 30 to 60 beats per minute. Cardiomegaly was apparent on a chest X-ray, and a coronary computed tomography angiogram showed no signs of coronary artery disease.\nThe operation was carried out through a median sternotomy with cardiopulmonary bypass support. After the induction of cardioplegic arrest, tricuspid valve analysis was performed. The pacemaker lead was found inside the right atrium, stretching from the tricuspid annulus toward the right ventricular cavity. The mechanism of severe TR resulted from three processes: impingement of the pacemaker lead, entrapment, and tricuspid annular dilatation (). Tricuspid annular dilatation was noted together with the impingement of the pacemaker lead into the right third of the septal leaflet. As well, lead entrapment was noted on the anterior papillary muscle, limiting the motion of the septal and anterior leaflets. Two decisions had to be made at this point. First, we had to decide whether to leave the pacemaker lead inside the heart chamber or implant new epicardial pacemaker leads. Second, we had to decide whether to repair her tricuspid valve or replace it. Fundamentally, this patient needed to maintain a permanent pacemaker due to her intractable sick sinus syndrome. With this in mind, it seemed significantly preferable to retain her transvenous pacemaker, because epicardial pacemakers have a relatively high stimulation threshold and require frequent battery changes. Moreover, her tricuspid valve morphology seemed repairable; as such, repairing her tricuspid valve was an obvious choice in light of the morbidity and mortality involved in triple valve replacement. Therefore, we decided to leave the pacemaker lead in place and to repair the tricuspid valve.\nFirst, we had to release the pacemaker lead from the valve and subvalvar apparatus. The pacemaker lead that impinged on the septal leaflet was detached by leaflet slicing without injuring the leaflet. The pacemaker lead attachment on the anterior papillary muscle was also released (). Subsequently, the pacemaker lead was moved to the poster-oseptal annulus and placed between two Lembert-type pledge-buttressed interrupted horizontal mattress sutures, in order to ensure that it was located inside the posteroseptal annulus (). Tethering of the leaflet did not take place after this procedure; instead, the size of the annulus was reduced. However, the pacemaker lead was still not firmly in place and wriggled from the tricuspid opening toward the right atrial cavity. Therefore, we pushed the pacemaker lead inside the ventricle and anchored it loosely to the anterior papillary muscle, using a 5-0 polypropylene simple interrupted suture (). A TR test using saline confirmed that the motion of the leaflet was not limited, so we proceeded to place an annuloplasty band using a 29-mm Duran AnCore Annuloplasty Band (Medtronic Inc., Minneapolis, MN, USA) (). Another TR test using saline confirmed the absence of TR. In turn, mitral valve replacement and aortic valve replacement were performed and the patient was weaned off cardiopulmonary bypass. After terminating the cardiopulmonary bypass, intraoperative transesophageal echography confirmed that no TR was present. The operation was completed and she was transferred to the intensive care unit. The day after surgery, she was extubated, and she was moved to the general ward on the second postoperative day. Postoperative echocardiography was performed five days after surgery, finding minimal TR and good functional recovery. She was discharged nine days after surgery after warfarinization was completed. Currently, she remains asymptomatic in outpatient follow-up examinations.
A 68-year-old male was referred to our institution for surgical management of a large, 7 cm, recurrent basal cell carcinoma (BCC) involving the upper face. Clinical examination revealed an ulcerative lesion impinging on the left canthus and lateral orbit with extension to within a centimeter of the helical root posteriorly. Computed tomography (CT) confirmed extensive invasion of underlying tissues including bony invasion of the zygomatic arch and posterior body of the zygoma (Fig. ).\nA wide local excision of the primary tumor was performed including the entire temple from the left lateral canthus and orbit to the route of the helix and tragus. The tumor was found to penetrate extensively into the underlying tissue, and thereby required a limited parotidectomy with further dissection of the temporal fossa. Ultimately, the entire lateral canthus, large portion of the left lower eyelid, entire zygomatic bone, left lateral orbit and sections of mid-facial muscles were resected leaving a large facial defect.\nA composite submental island flap (6 cm vertical X 8 cm transverse) was harvested with the inferior border of the mandible (1 cm vertical X 7 cm transverse) (Figs. and ). A level I neck dissection was performed with preservation of the submental branches of the facial artery and vein. The marginal mandibular branches of both facial nerves were identified and preserved during flap elevation. A perforator of the mylohyoid muscle supplying the mandibular segment was included alongside the left anterior digastric muscle to ensure adequate blood supply. The pedicle was completely dissected with a single submental artery and vein. Soft tissue and bone perfusion were confirmed with a doppler probe. The flap was transposed into the facial defect through a subcutaneous tunnel in the lower face. The zygomatic arch and lateral orbit were reconstituted with the vascularized mandibular bone using mini-plate fixation. The lateral canthus was reconstructed by suspending the lower lid remnant from the periosteum of the remaining orbit superiorly. The great majority of the skin defect was reconstructed with the SIF. However, a small superior temporal defect remained, and this was addressed with a local (superiorly based scalp) flap as well as a full thickness skin graft from the unused portion of the original submental flap.\nThe patient remains disease-free with no complications on 2-year follow up. The flap was robust throughout adjuvant radiotherapy. The patient required one revision procedure to address lower lid ectropion after radiation, as well as an unrelated BCC. The latter was done with a concurrent mid-brow lift to address a pre-existing brow ptosis and to provide a full thickness skin graft for the lower lid malposition.
We present a 67-year-old female admitted with dyspnea and new onset atrial fibrillation with rapid ventricular rate. She was diagnosed with Graves' disease 3 weeks earlier and had been started on 20 mg of methimazole daily. Thyroid function tests showed suppressed TSH with elevated Free T3 and Free T4. Details of lab results are given in . Pro-BNP was elevated at 1762. Chest X-ray revealed unilateral right pleural effusion which was subsequently drained by thoracentesis and was consistent with transudative effusion. She was managed as a case of congestive heart failure. Her heart rate control was optimized and the dose of methimazole was increased to 30 mg once daily. She was discharged home but readmitted 2 days later with recurrent dyspnea. A repeat chest X-ray showed recurrence of the right pleural effusion. Transthoracic echocardiogram was done showing normal ejection fraction and diastolic function. Her heart rate was controlled. Repeat thyroid function tests showed a normal free T3 and almost normal free T4. Patient was diuresed, and the unilateral right pleural effusion was drained again. The fluid characteristics showed a transformation from transudative to exudative likely in the setting of recent diuretic use.\nShe was then discharged home after she had improved clinically only to be readmitted again with worsening dyspnea attributed to reaccumulation of the right pleural fluid a few days later. A repeat drainage with pleural catheter was performed, and the fluids revealed an exudative process with elevated LDH. This was further evaluated with a CT scan of the chest with contrast (Figures and ) which showed mediastinal lymphadenopathy and a diffuse ground glass process involving the right lower lobe suggestive of pneumonitis. A bronchoscopy was also performed. The bronchoalveolar lavage showed neutrophil predominant fluid, and cytology and adenosine deaminase were negative. Patient also had an endobronchial ultrasound-guided biopsy of the lymph nodes (EBUS). A work-up at this time was begun for drug induced vasculitis. She was treated empirically with steroids 40 mg for 10 days, and the methimazole was also discontinued. The antinuclear antibodies (ANA) came back positive with a speckled pattern; antineutrophil cytoplasmic antibody (c-ANCA) and antimyeloperoxidase MPO were also positive, but anti-double stranded DNA (DS-DNA) was negative. The clinical picture was in keeping with a drug-induced vasculitic picture which is unusual with the use of methimazole. The patient improved remarkably without recurrence of the pleural effusion. She was restarted on methimazole as it was not definite it was the cause of the pleural effusion. On a subsequent outpatient visit a couple of weeks after restarting the methimazole, the effusion recurred but did not require drainage. Methimazole was discontinued. She was referred for urgent thyroidectomy. Repeat chest X-ray showed complete resolution of the pleural effusion after stopping the methimazole. Repeat ANCA and antimyeloperoxidase antibody were both negative one and a half weeks after methimazole was discontinued.\nSee .\nSee Figures and .
A 22-year-old female presented to the ear, nose and throat (ENT) department with an 18-month history of persistent clicking tinnitus and left facial pain. The patient's tinnitus and sensation of involuntary palatal movement developed about one or two months after starting the use of a wire type orthodontic appliance. There was accompanying involuntary tremor-like dystonia on the left side of her face and buccal area, which was unnoticeable to other people. Clicking tinnitus and involuntary muscle contraction of the face and buccal area prompted the patient to visit several dental, ENT and neurologic clinics; a diagnosis had still not been made after more than a year, and this caused the patient stress.\nThe tinnitus and dystonic spasm with painful sensation on the left facial area were aggravated over time. Stressful events in her everyday life made the symptoms worse. The patient felt being rejected, sad, frustrated, depressed and even anxious because the painful symptoms remained undiagnosed for a long time. The patient's attempts at relief included herbal medicine, acupuncture and massage, but none of these treatments were successful. The patient also experienced symptom-related depression, anxiety and insomnia, which also created much anxiety among her family members. The patient visited a local neurological clinic where she received brain magnetic resonance imaging (MRI) and an electromygraphic study of her face. Clonazapam was prescribed without a proper diagnosis by one neurologist, but the symptoms were not relieved. The patient identified that the tinnitus was exacerbated by touching the tongue to the palate.\nMore than 18 months had passed without any definitive diagnosis when the patient first presented to our department. After a careful and long history taking under the suspicion of palatal myoclonus with orofacial buccal dystonia, we thoroughly examined her palate and face. When the patient touched the palate with the tongue, clicking tinnitus was clearly audible and this could be heard by people next to her as one or two rapid clicks per second. Symmetric bilateral contraction of the anterior margin of the soft palate that was synchronous with the patient's clicking tinnitus was observed on both the oral and nasopharyngosocopic examinations. Facial examination revealed weak blepharospasm on the left side of her eyelid. However, we could not detect any noticeable contractions on the buccal area, which is where the patient felt the most severe dystonic movement and pain. We again used a fiberoptic laryngoscope to detect the movement of the buccal muscle and finally found the subtle contraction of the muscles in the left upper lip and buccal mucosal area. The patient also felt an involuntary movement of the muscles around the left nose and chin and even in the deep part of the left anterior neck, but there were no definite tremor-like motions evident on physical examination. The pure-tone, speech and impedance audiometry, including assessment of the static compliance, were normal. Brain MRI did not show any definite abnormal brain findings or brainstem lesion. Consultation with the neurologic department ruled out other neurologic disorders and this was confirmed by the absence of any other accompanying neurologic deficits. All of this led us to diagnose the symptoms as essential palatal myoclonus associated with orofacial buccal dystonia and this was possibly triggered by the orthodontic appliance. The patient refused medication for a long time as she had already unsuccessfully tried different types of muscle relaxants, 5-HT1 agonist, a Levo-dopa agent and anticonvulsants, which were all prescribed by another neurologist.\nFollowing an ineffective 1-month course of medical therapy with a combination of muscle relaxant (Baclofen®, Pacific Pharma, Seoul, Korea), anticonvulsant (Rivotril®, Rhoche Korea, Seoul, Korea) and anxiolytic agent (Xanapam®, Myung In Pharm, Hwasung, Korea), the patient was treated with an injection of botulinum toxin A (Dysport®, Ipsen Korea, Seoul, Korea) under the guidance of electromyography (EMG). EMG of the tensor veli palatine, orbicularis oris and orbicularis oculi muscles showed bursts of abnormal electrical activity (). Botulinum toxin A was injected into both sides of the tensor veli palatini muscles bilaterally via the mouth (15 U each) and by a nasal route (10 U each) (). The two most painful and dystonic movement sites on the patient's face (the orbicularis oculi and oris) were selected first and EMG-guided botulinum toxin injections were performed (10-15 U).\nTwo days later, she felt decreased clicking tinnitus and a decreased painful sensation with dystonia on her left face. Therefore, botulinum toxin A was injected into every point of the abnormal muscle contraction on the face where the patient pointed and this was confirmed by EMG to have been done. The injected muscle area and the dose of botulinum toxin A are demonstrated in . On the review at week after injection of botulinum toxin A, her tinnitus and muscle dystonia were markedly decreased. She developed a hypernasal voice and mild facial weakness, but she was satisfied with the treatment. Indeed, the patient wanted to be treated again to have complete cession of her tinnitus and palatal muscle contraction. So, two more consecutive injections into the palatal muscles (30 U in total to each side of the tensor veli palatine muscle) and one more injection into the left buccal area (15 U) were done. At the same time, the patient received counseling, which included general information about brain reorganization and behavioral modification designed to avoid triggering tinnitus and to reduce focusing on the tinnitus and facial muscle dystonia as well. Psychiatric consultation proved beneficial in easing the patient's feelings of depression and anxiety.\nAt the 2-month follow-up, the palatal myoclonus tinnitus had completely stopped and this was confirmed by nasopharyngoscopic examination. The orofacial buccal dystonia and pain had decreased and what remained was tolerable. Her hypernasal voice and velopharyngeal insufficiency had completely disappeared. Her symptoms did not reappear over the next 4 months. Nonetheless, additional botulinum toxin injections are planned if her symptoms recur.
A 50-year-old woman, with a history of breast cancer and a course of radiotherapy and chemotherapy, had a Permcath emplacement in right subclavian vein in august 2008 for her chemotherapy. Permcath is a flexible double lumen cuffed plastic tube that can be put into a vein just below the neck. The tube can then be used for long term purposes. The patient was otherwise in a good health condition and did not have any other positive medical or drug history. The treatment for cancer seemed to be successful and her oncologist performed routine follow-up measurements. The Permcath had remained in its position without any complication for three years followed up by routine chest X-ray study ( and ). However, the Permcath was found broken totally from its base and embolized to right ventricle and the left main pulmonary artery ( and ) after three years of insertion.\nThe chest X-ray (dated a year before our evaluation) revealed that the Permcath was intact and in its normal position. Furthermore, there was no history of physical or traumatic injury or accident in the precise history review for the last three years. The patient did not report any use of her Permcath during the last investigation and did not remember any obvious complaint regarding embolization, migration, or lodging the catheter. There was not any positive chief compliant at the time of detection and physical examinations. Furthermore, electrocardiography and echocardiography investigations were normal or unchanged comparing with the previous ones.\nWe hypothesized that sticking the catheter between her clavicle and first rib was the probable cause of its breaking (pinch-off syndrome). In consultation with colleagues and experts, and following discussion of probable symptoms and potential complications, considering the lack of any sign or symptom and the risk of open surgery to removal of the embolized catheter, we decided to leave it in its position, and the patient was advised to perform regular consecutive follow-up studies. There was no change in the patient condition and the catheter position during a one-year follow up.
A 60-year-old male patient was presented to our outpatient clinic with the complaints of swelling and pain in the second finger of his right hand. BD had been diagnosed 33 years earlier. He had experienced swelling in his right knee about 30 years earlier, but he was symptom-free after joint aspiration. Then, 20 years ago, he had a swollen left ankle, which improved spontaneously within 10 days. At that time, he used colchicine for 2 years, but stopped this drug himself due to gastrointestinal adverse effects. At the time of presentation to our clinic, the patient had not used colchicine or other medication for the last 10 years. He mentioned that he had been having difficulty using his hand due to swelling and pain in the second finger for the last 3 months. On physical examination, there was pain and swelling in his second MCP joint. He had no oral or genital aphthae. The fundus examination revealed no uveitis. The erythrocyte sedimentation rate was 9 mm/h and C-reactive protein was 8.5 mg/liter (normal range is 0–5). Rheumatoid factor and antinuclear antibody were negative. HLA-B51 antigen was positive. The kidney and liver functions and other blood tests were within normal limits. Since we suspected destructive changes in the second MCP joint of the right hand on plain radiograph (Fig. ), we evaluated the patient by GE Logiq 5 ultrasonography (US) in our department. The US examination was performed from dorsal aspect of the second MCP joint with longitudinal and transverse scans. A step-down contour defect of the erosion was observed in the second MCP joint on longitudinal image. The normal and erosive articular surface of the second MCP joint were indicated, on longitudinal images in Fig. and on transverse images in Fig. . After the diagnosis of the bone erosion, the patient was prescribed a weekly dose of 10 mg methotrexate. At the three-month follow-up, his complaints had almost completely been resolved. Written informed consent was obtained from the patient for the publication of this report.\nWe searched PubMed, MEDLINE, EMBASE, Scopus, and Web of Science using the keywords, ‘Behçet’s disease [AND] erosive arthritis’ and ‘Behçet’s disease [AND] destructive arthritis’. The original articles, letters and case reports published in English between 1985 and December 2019 were identified. Publications containing abstracts with limited information, comments or papers written in languages other than English, irrelevant articles, and duplicates were excluded.\nWe carefully reviewed 10 articles and the references therein. Five articles were not further evaluated because their main text was in French. Eight of the 10 articles were case reports/letters and two were original articles/reviews. Most case reports were published after the year 2000. Table presents the characteristics of the 19 patients with erosive arthropathy reported in the evaluated articles. The mean age of these patients was 43.89 (range 19–72) years, and 10 were female. The main presenting symptoms were usually monoarthritis or asymmetrical oligoarthritis.
A 77-year-old Caucasian man was admitted to our emergency department with abdominal pain and nausea that had occurred for the previous 24 hours; the patient had a one-year history of intermittent abdominal pain and chest tightness during physical effort. He had been evaluated for these chest problems approximately one year ago at another center and was determined to have no cardiac issues. The plain chest X-rays that were taken at that time revealed consolidated areas in the left basal lung segments, and the patient was treated for a diagnosis of pneumonia. The patient did not have a significant history of trauma.\nWhen the patient presented at our hospital, his abdomen was mildly distended and hyperactive bowel sounds were heard. A physical examination revealed muscular rigidity in the left upper quadrant of the patient's abdomen without rebound tenderness or signs of peritoneal irritation. The rectal examination revealed no masses or bleeding. The patient had a temperature of 37.5°C, a heart rate of 100/min, and a blood pressure of 160/90 mmHg. Laboratory analysis revealed an elevated white blood cell count of 19,000/mm3. The other laboratory results were normal. These signs were interpreted as a partial bowel obstruction of unknown origin because the patient was passing stool and flatus. An urgent computerized tomography (CT) scan with oral/rectal and intravenous contrast was performed; the scan revealed a left-sided BH, with a large segment of incarcerated transverse colon (-).\nThe patient was taken to the operating room for laparoscopic repair of an incarcerated BH. The patient was placed in a modified lithotomy position, and five trocars were used. The intra-abdominal pressure was maintained at 10 mmHg after the pneumoperitoneum was achieved via the open technique. The head and the left side of the patient were raised to 30°. The omentum and the distal part of the transverse colon were found to be attached to the left-sided BH (). The diaphragm was divided by a few centimeters to enable the reduction of the bowel. The dimensions of the defect were measured to be approximately 6×6 cm after the reduction of the omentum and colon into the abdomen. No signs of perforation or ischemia were present.\nThe defect was repaired with non-absorbable “0′' polypropylene sutures using a round-bodied 30-mm needle (Prolene; Ethicon, Bracknell, UK). An increased tidal volume was used before the last suture was placed to decrease any residual pneumothorax. A monofilament polypropylene (Prolene; Ethicon, Bracknell, UK) mesh was used to reinforce the repair, and a laparoscopic tacker was used for fixation of mesh.\nThe patient had an uneventful recovery, and he was discharged on postoperative day two. He remained well at his six-month follow-up, without evidence of clinical or radiological recurrence.
The patient is a 15-year-old Caucasian female who was diagnosed with CRMO in 2007 at 5 years in the context of right thigh pain. provides a timeline of the patient's symptoms and course of disease. Magnetic resonance imaging (MRI) showed multifocal abnormal bone marrow signal in the right femur, the left femoral neck, and the proximal epiphysis and metaphysis of the right tibia, which was associated with osteolysis and callus formation (). Radio-nucleotide bone scan found increased uptake involving the left sacrum, left proximal femur, and femoral neck region as well as the midshaft of the right femur and the proximal right tibia. Bone biopsy of the lytic lesion involving the midshaft of the right femur was not consistent with malignancy and showed red blood cells and scattered neutrophils and lymphocytes. Over the next several years (2007–2015), she was followed by orthopedic surgery and was treated only with intermittent ibuprofen as needed for pain. She was noted to have a leg length discrepancy at the age of 7 years, and in 2014, at the age of 12 years, she had surgery to fuse the growth plate to prevent right leg growth (right leg was 4.5 cm longer than her left leg at that time).\nShe established Rheumatologic care in March 2015 at age 13, and right quadriceps muscle atrophy and failure to thrive with a weight and height under the third percentile were noted at this time. Bone scan revealed increased uptake in the right femur and asymmetry of activity in the growth plates of the knees and ankles with decreased activity in the right side compared to left. She was treated with naproxen 250 mg twice daily (8.7 mg/kg BID) and prednisone 20 mg per day (0.7 mg/kg), tapered by 5 mg weekly. Two months later, prednisone was discontinued, and she continued to have good control of her leg pain on NSAID monotherapy. After a few months, she developed new diarrhea with vomiting and weight loss. Her blood pressure was elevated, and a renal ultrasound and electrocardiogram were unremarkable.\nShe was referred for Gastroenterology evaluation and was found to have positive stool occult blood along with a perirectal skin tag. Laboratory studies revealed anemia with a hemoglobin of 7.1 g/dL (nl 12–16 g/dL), thrombocytosis with platelets of 744 k (nl 150–450 k), erythrocyte sedimentation rate (ESR) of 69 mm/hr (nl 0–10 mm/hr), and C-reactive protein (CRP) of 129 mg/L (nl 0–3 mg/L). There was no family history of autoimmune disease, and tuberculin testing was negative. Colonoscopy revealed pancolitis with crypt inflammation and crypt abscesses with no granuloma consistent with ulcerative colitis (). NSAID therapy was discontinued, and treatment for UC with prednisone 1 mg/kg/day (40 mg) tapering by 5 mg weekly along with sulfasalazine was initiated. Infliximab 3 mg/kg infusions were added the following month.\nAt the visit for her second infliximab infusion, she presented with tachycardia, a blood pressure of 230/190, and headache and was admitted to the pediatric intensive care unit for hypertensive emergency requiring nicardipine infusion. Her examination was notable for right-sided Horner's syndrome, decreased right leg pulse pressure, and an abdominal bruit. Echocardiogram showed a small pericardial effusion with reduced left ventricular ejection fraction. Laboratory studies revealed negative ANA and ANCA screens, normal C3 and C4, and normal von Willebrand factor antigen. CT angiogram (CTA) of the abdomen and pelvis showed narrowing of the mid-aorta, proximal renal arteries, celiac artery, and superior mesenteric artery (Figures –). CTA of the chest showed marked descending thoracic and abdominal aortic wall thickening with progressive luminal narrowing and wall thickening of the visualized portion of the right common carotid artery and celiac trunk, enlarged left atrium, left ventricular hypertrophy, and a small pericardial effusion (). MRI/MRA brain showed anterior and posterior circulations of the brain were without occlusion or aneurysm with the patent carotid and vertebral arteries of the neck (Figures and ). Given these findings, she was diagnosed with TA complicated by middle aortic syndrome. She was started on metoprolol 50 mg daily (1.5 mg/kg daily), amlodipine 5 mg daily, famotidine 20 mg twice daily, aspirin 81 mg daily, and increased dose and frequency of infliximab from 3 mg/kg every 8 weeks to 5 mg/kg IV every 4 weeks along with prednisone 20 mg daily (0.6 mg/kg daily) and mesalamine 1 g BID. The patient continued infliximab every 8 weeks rather than every 4 weeks as recommended.\nMagnetic resonance (MR) angiography of the chest, abdomen, and pelvis and Cardiac MR three months later showed luminal narrowing of the distal thoracic and upper abdominal aorta similar to previous CT studies, stenosis of origin of celiac axis, stenosis of proximal superior mesenteric artery (SMA), and moderate stenosis of bilateral proximal renal arteries. Echocardiogram noted concentric LVH with mildly reduced function, measuring 45%, and normal coronary arteries.\nAt this point, the patient transferred her care to our rheumatology clinic, and despite treatment with infliximab 5 mg/kg every 8 weeks, she continued to report right thigh pain and developed new inflammatory arthritis of the left ankle and increased inflammatory markers with an ESR of 55 mm/hr (nl 0–20 mm/hr) and an elevated CRP of 73 mg/L (nl < 3 mg/L). Additionally, interval imaging found new wall thickening around the right common carotid artery. This was concerning for uncontrolled TA and CRMO activity. There was an unfortunate delay in treatment escalation due to social circumstances. Two months later, she started treatment with parenteral methylprednisolone 1 gram weekly for 8 weeks, and her dose of infliximab was increased from 5 mg/kg to 10 mg/kg every 4 weeks. Based on adult data demonstrating a positive response to higher doses of infliximab, the decision was made to increase the dose of infliximab instead of trying another TNF inhibitor.\nMultidisciplinary evaluation at Boston Children's Hospital, Center for Middle Aortic Syndrome by neurosurgery, nephrology, and rheumatology, led to recommendations of a prednisone dose increase and the addition of methotrexate 15 mg/m2 weekly to infliximab 10 mg/kg every 4 weeks. At this time, her echocardiogram revealed moderate left ventricle dilation and mildly depressed left ventricular systolic function. One month later, repeat head and neck CTA showed progression of her right carotid artery stenosis to 80%. Her ESR had normalized, and CRP decreased to 7.8 mg/L at this time. Given her worsening carotid artery stenosis, infliximab was increased from 10 to 15 mg/kg every 4 weeks. On a combination of moderate dose prednisone, weekly methotrexate, and infliximab, she denied joint pain, swelling, abdominal pain, diarrhea, or blood in stool. Follow-up brain MRI/MRA three months later was normal. Repeat chest and abdomen MRA showed all of the areas of stenosis appeared to be stable and inflammatory markers had normalized.\nCurrently, her clinical course is stable without further anatomic progression, and she has normal inflammatory markers. Thus, she has continued on her current regimen with infliximab 15 mg/kg every 4 weeks and methotrexate 15 mg/m2 once weekly. Prednisone decreased gradually and discontinued. She has not developed any infectious sequelae on this regimen. Her blood pressure is stable on carvedilol alone. We continue to assess blood work monthly. Given her use of prednisone, vitamin D level was followed and found to be low, and she has started on a vitamin D supplement. Dilated ophthalmologic exam was normal. Repeat MRI of the lower extremities and bone scan show that her CRMO lesions are inactive. Repeat MRI/MRA of the brain, chest, abdomen, and pelvis in June of 2018 show stable changes without the need for stenting. Given the predisposition for autoinflammatory conditions in this patient, the authors are considering genetic testing in search of a monogenic cause that may support a unifying diagnosis.
A 62-year-old male with a past medical history significant for a coronary artery bypass grafting 10 years prior presented with 3 weeks of persistent left epigastric pain. He denied dyspnea or chest pain. On exam, he was hemodynamically stable, in no apparent distress, and no audible murmur. EKG showed Q wave on the inferior leads suggest ing old myocardial infarct, which was unchanged from a previous EKG performed 3 months prior. A complete series of cardiac enzymes ruled out acute myocardial infarction. Because of the previous coronary bypass surgery and his atypical symptom, the patient underwent cardiac catheterization which showed patent grafts (left internal mammary artery to the left anterior descending artery and saphenous vein graft to the obtuse marginal artery) and intact right coronary system. Left ventriculography was not performed at that time. Transthoracic echocardiography showed severely decreased left ventricular function. Because of persistent pain in the left epigastric area to the left flank, a contrast CT scan was obtained to role out intraabdominal pathology. The 16 slice spiral CT scan with intravenous contrast demonstrated a large LV pseudoaneurysm (Fig. ). There was no other intraabdominal lesion was observed. Left ventriculogram was subsequently performed and confirmed the LV pseudoaneurysm (Fig. ).\nThe patient was then scheduled for resection of the LV pseudoaneurysm. After dissection of the anterior wall of the heart, further dissection of the lateral and posterior wall of the heart was performed under cardiopulmonary bypass because of dense adhesion. The LV pseudoaneurysm was located in the posterolateral wall of the LV (Fig. ), adherent to the posterior pericardium. After complete immobilization of the LV, the pseudoaneurysm was opened under aortic cross clamp. The wall of the pseudoaneurysm was thin and filled with clots. The neck of the pseudoaneurysm was identified and closed with a Gore-Tex patch. The ventriculotomy was then closed with multiple buttressed sutures. The postoperative course was uneventful and he was discharged to a rehabilitation facility 10 days after surgery.
A 72-year-old female was admitted to our hospital with acute cerebellar infarction and signs of nausea, vomiting, ataxia, and subsequent anorexia. The medical history of the patient included hypertension and arrhythmia, although it was unclear whether the patient ever experienced atrial fibrillation. The patient had no diabetes, coronary heart disease, hyperlipidemia, and no history of smoking and alcohol abuse. During physical examination, the patient was conscious, fluent in speech, with both eyeballs showing horizontal nystagmus. The limb muscle tone of the patient was normal, with signs of ataxia in left upper and lower limbs. Other items of the physical examination showed normal results. The routine blood test, liver and kidney function, electrolytes, blood glucose, and blood coagulation results of the patient were also normal. The head magnetic resonance imaging (MRI) of the patient showed bilateral cerebellar infarction (Fig. ).\nDuring hospitalization the appetite of the patient became worse. After 1 month of hospitalization, the dizziness, nausea, and vomiting of the patient were not alleviated. The patient then received another head MRI examination. No other abnormalities were found in this head MRI, except the marks of previous cerebral infarction. After being treated with benzaisol and ondansetron, the above symptoms of the patient gradually improved. Nevertheless, the appetite of the patient got worse and hence intravenous administration of nutritional support was needed. The patient eventually developed hyponatremia after 1-week administration of parenteral nutrition supplement, including high glucose, although the supplement of vitamins was not sufficient. The blood sodium level of the patient was 121 to 128 mmol/L despite the use of an appropriate sodium supplement. The blood osmolality and urine osmolality of the patient were 256 and 685 mOsm/Kg, respectively. On day 14 of parenteral nutrition supplement, the patient presented with hypotension and apathy. The blood pressure of the patient dropped to 95/60 mmHg and stayed there after all antihypertensive drugs were stopped. The patient developed consciousness disorder on day 20 of parenteral nutrition supplement and was uncooperative in examination. Neurological examination of the patient showed slower pupillary response to light and more obvious signs of nystagmus with paralysis of bilateral abduction nerves. Blood tests showed that the white blood cells, C-reactive protein, and procalcitonin of the patient were all elevated, although the signs of fever were absent. The analysis of arterial blood gas indicated respiratory alkalosis and lactic acidosis. The patient showed a pH value of 7.31, an oxygen partial pressure of 157 mmHg, a carbon dioxide partial pressure of 13 mmHg, and a lactic acid concentration of 6.2 mmol/L. Chest computed tomography showed signs of mild inflammation in both lungs. Routine urine test and routine stool test did not show signs of infection. The patient received head MRI again for fear of cerebral infarction relapse. The MRI showed symmetrical lesions in T2-weighted imaging images and FLAIR high signal intensity in the periaqueduct, hypothalamus, thalamus, mammiliary bodies, medulla oblongata and vermis cerebelli, which were consistent with the diagnosis of WE (Fig. ). On the same day, the patient quickly fell into lethargy. At the time, the blood pressure of the patient reached as low as 60/40 mmHg, with a heart rate of 130 bpm, a pH value of 7.31, an oxygen partial pressure of 65 mmHg, a carbon dioxide partial pressure of 13 mmHg, and a lactic acid concentration of 6.2 mmol/L in the arterial blood. Subsequently, the patient was intubated and sent to intensive care unit, where an electroencephalograph examination showed diffuse low-amplitude theta waves.\nThe patient was given 300 mg of intramuscular thiamine per day for two weeks, followed by 100 mg of intramuscular thiamine per day for another two weeks and then 300 mg of oral thiamine tablet per day. At the same time, the patient received anti-infection treatment and vasopressors. Within 3 days, the patient became alert and her blood sodium level returned to normal without the use of sodium supplementation. The blood pressure of the patient was stabilized after one-week administration of vasopressors, although her signs of nystagmus did not disappear until 45 days of thiamine supplementation was given. Mini-Mental State Examination indicated impaired memory functions. Unfortunately, the patient was not examined with head MRI again after thiamine supplementation, so it is not clear whether the intracranial lesions have improved. Nevertheless, the signs of severe pneumonia of the patient were alleviated more than 1 month later.
A 30-year-old female with a history of bronchial asthma and pregnant at 14 weeks of gestation presented with hyperemesis gravidarum to one of the private hospitals in the city. Abdominal ultrasound was performed showing a large cyst in the liver measuring 12 × 7 cm in size, which was diagnosed as a hydatid liver cyst. On general examination, signs of dehydration were detected. Abdominal examination revealed mild, diffuse upper abdominal tenderness. She was given symptomatic treatment and referred to the surgical clinic for followup, which she failed to attend. After delivery and during the routine follow-up visit, abdominal ultrasound was done showing evidence of a bilocular cyst with internal septa measuring 15 × 7 cm situated in the right lobe of the liver with an extension to the undersurface. CT scan of the abdomen and pelvis revealed a septated cyst arising from the right lobe of the liver (). A calcified septation within the cyst and some degree of calcification of the superior aspect of the cyst were detected. The left adrenal gland, spleen, and both kidneys were all normal. However, the right adrenal gland could not be delineated. All other investigations including chest X-ray, renal function tests, liver function tests, other biochemical tests, electrolytes, hematocrit, and thyroid function tests were normal. Urine culture and sensitivity and viral serology were all normal. Antibodies for a hydatid cyst were negative (160, reference range < 360). Due to the large size of the cyst, surgical intervention was considered, laparotomy was performed, and the cyst was excised.\nMacroscopic examination of the specimen showed a 12 × 9 × 7 cm partially collapsed multilocular brownish glistening cyst, weighing 210 grams and containing serous fluid (). The outer surface showed a 4 × 2 × 0.5 cm yellowish fatty adrenal tissue attached to the cyst. The average wall thickness of the cyst was 0.2 cm; the inner lining was largely smooth with focal roughened papilloid areas. Microscopically, the multilocular cyst showed a focally calcified fibrotic cyst wall lined by low cuboidal to flattened epithelium which showed strong positivity for factor-8 immunohistochemistry (Figures , , and ). Congested blood vessels and clusters of lipidized adrenal cortical cells were also seen in the stroma. The included adrenal gland was within normal limits. No pleomorphism or mitosis was seen. The overall appearance was consistent with a benign adrenal cyst of endothelial type.
A 42-year-old Saudi female patient, not known to have any chronic medical illness was referred to the Department of Oral and Maxillofacial Surgery in Prince Sultan Medical Military City, Riyadh, Saudi Arabia by a consultant dermatologist with a submandibular skin fistula, that was treated by antibiotic and local creams for 3 months with no improvement. Referral was to rule out an odontogenic cause. She was seen in the Oral and Maxillofacial Clinic complaining of recurrent pus discharge from her neck for the past 6 months with no history of dental pain. Examination showed an extraoral fistula in the left submandibular region with pus discharge upon palpation, intra oral examination showed no soft tissues abnormalities in the ipsilateral area with all molar teeth missing, panoramic radiograph showed a round radiolucent lesion in the left body of the mandible with presence of radiopaque foreign body inside the lesion resembling a remaining root (). The cone beam computed tomography evaluation showed a 1×1 cm round radiolucent lesion causing displacement of the inferior alveolar canal medially with presence of an endodontically treated root inside the lesion (Figures ). She mentioned later that she underwent teeth extraction on the same side 8 years earlier in a private dental clinic in Taif, and was not informed of any intraoperative complications. Following patient consent and under general anesthesia a lateral cortical window was reflected through an intraoral approach; the remaining root was exposed then removed, and the surrounding infected tissue was excised completely with preservation of the inferior alveolar nerve that was dissected and preserved medially. The cortical window was fixed to the original place by a microplate (1.5 mm) to enhance the stabilisation of the bony segment (Figures ). The fistula was traced and excised completely with elliptical excision and closed primarily (). Postoperatively, she was cleared from the infection but had temporary hypoesthesia in the left cheek area, which fully recovered 3 weeks after surgery. No further complications were reported.
A 69-year-old male with a past medical history of tobacco use disorder presented to the emergency department with a one-week history of worsening headache and right temporal vision loss. The patient worked as a boat mechanic, and stated that one week ago he was working on a boat engine that was leaking diesel fuel. He worked on the engine for several hours in an enclosed room with it running intermittently. Shortly after leaving work, he noticed a throbbing, dull headache located across his forehead and temporal region, as well as loss of peripheral vision in his right eye. At presentation, the patient described that the headache and vision changes had worsened, but denied any focal neurological deficits. He stated that he first went to an urgent care facility where he was diagnosed with sinusitis and prescribed amoxicillin, which did not relieve any of his symptoms.\nIn the emergency room, the patient was initially hypertensive with a blood pressure of 181/92 mmHg, but it improved to 141/80 mmHg without medical intervention. Neurologic examination revealed a right monocular temporal hemianopia upon visual field confrontation, resulting in an NIH stroke score of one. The patient was admitted for further evaluation of his headache and peripheral vision loss of the right eye.\nOn admission, magnetic resonance imaging (MRI) of the brain showed multifocal acute infarcts in the left occipital lobe, indicating left PCA disease and no hemorrhagic transformation (Figure ). Given the signal on diffusion and enhancement, it was estimated the infarct was between three and seven days old. Computed tomography (CT) angiogram of the head pre-contrast demonstrated a small area of infarct in the left occipital lobe, corresponding with the MRI findings (Figure ). After contrast administration, the left PCA demonstrated severe stenosis in the P1 segment. The distal branches of the PCA were still opacified and the posterior communicating arteries were faintly seen. This demonstrated severe stenosis of the PCA.\nThere was no indication for angiography or intervention due to the time that had passed since the estimated occurrence of the PCA infarct. A cardiac workup including electrocardiogram and echocardiogram with bubble study were performed to monitor for causes of cardioembolic stroke, and these studies returned with no abnormalities. The patient was advised to obtain a loop recorder to monitor the presence of atrial fibrillation but the patient denied. Medical management for the infarction was recommended. Modifiable risk factors such as blood pressure, glucose, and cholesterol were addressed with the addition of clopidogrel, atorvastatin, and lisinopril.
A 29-year-old woman initially presented with a 2-week history of nonmigratory right iliac fossa pain with subjective fever. The patient's history of complaint was not typical of acute appendicitis. After unremarkable inflammatory markers and pelvic ultrasound that did not visualize the appendix, she was discharged home with a provisional diagnosis of pelvic inflammatory disease and was given oral antibiotics for 7 days.\nHowever, she represented to our emergency department (ED) with worsening abdominal pain, ongoing fever and mild nausea. A computed tomography (CT) was organized and it showed a perforated appendicitis with retrocecal abscess and a calcified appendicolith. She then had an ultrasound-guided drainage of the abscess and was discharged home with a view of performing an interval laparoscopic appendectomy. After discharge, the patient returned to ED for persistent recurrence of a right pelvic collection involving the right iliacus and lateral abdominal wall muscle. This was again radiologically drained. A colonoscopy was performed during this presentation to exclude any primary tumors before surgery. The entire colon was examined and showed normal mucosa and appendiceal orifice.\nThe patient then underwent a semi-emergent laparoscopic appendectomy. During the procedure, thorough abdominal wash was performed and a drain tube placed in the right iliac fossa. The patient had an uneventful postoperative recovery, with normalization of inflammatory markers and the drain tube removed. She was then discharged home with oral antibiotics. During her follow-up in the general surgery clinic, the histopathology reported chronic appendicitis with granulomas but the fecalith was not within the specimen. On review, it was noted that she had intermittent abdominal discomfort and a repeat of C-reactive protein (CRP) showed that it has elevated to 41. A repeat CT scan was performed which showed re-accumulation of the right pelvic and lateral abdominal collection with the retained fecalith (Figures , ).\nAlthough the abscess was intramuscular, the fecalith and the pelvic abscess were going through the abdominal wall into the subcutaneous fat (Figure ). There was a brief discussion with an interventional radiologist for a hook-wire insertion preoperatively for direct localization of the fecalith but given the location of the abscesses, intraoperative ultrasound was used instead. The patient underwent exploration and surgical removal of retained fecalith through a right lateral hip approach (Figure ). A drain was inserted into the cavity and the wounds were closed with interrupted sutures.
A 19-year-old male patient came to the department of pediatric dentistry of our hospital in order to get prosthetic treatment for his right mandibular posterior teeth. The patient was given a temporary bridge after preparation of the right mandibular posterior teeth under general anesthesia at our hospital about 2 months ago. On the day of presentation, final preparation and impression for the final bridge were scheduled. The patient had autism, with no other relevant medical history. His weight was approximately 90 kg; height was approximately 180 cm. He was overweight for his age. The degree of autism was so severe that he could not stand still and stubbornly resisted from entering the clinic. In addition, the muscular strength of the patient made it tougher to control his behavior. We decided to administer 10 mg of midazolam via the intramuscular route. However, the patient's resistance to intramuscular injection made it difficult to do so. As the patient was overweight, sedation via the oral route could not be maintained, and he resisted taking oral pills. Moreover, even intravenous injection was difficult, and there were no professionals and monitoring equipment to perform sedation via the intravenous route. Hence, as an alternative, we decided to try sedation via the submucosal route. First, protective stabilization for the patient who had strong involuntary movements was arranged using physical restraint. Then, local infiltration anesthesia was administered with about 0.6 ml of 2% lidocaine (1:100,000 epinephrine) to the left maxillary vestibule, and midazolam was injected into the submucosa at the same spot. The total dose of midazolam was 9 mg (5 mg/ml; 0.1 mg/kg). After about 5 minutes, the patient was under sedation. The level attained was that of conscious sedation, and the airway remained under the patient's voluntary control. During the treatment, he turned his head or moved his body, both of which did not interfere with the treatment. SpO2 was continuously monitored during the treatment, and O2 was continuously supplied through the nasal mask. The duration of the dental procedure was about 40 minutes, and the treatment was safely completed without any side effects of midazolam under good coordination. Approximately 30 minutes after the completion of the treatment, the patient recovered from calm state. After confirming that there was no physical problem, we explained the remarks, and allowed him to go home.
A 26-years-old male patient reported to our institution with a complaint of swelling in the right side of the mandible and in the right supraorbital region since 5 years. History revealed that the onset of these swellings were slow and progressive. Patient developed a dull aching pain in the right side of lower jaw since few days and subsequently developed numbness in the lower lip of same side. Patient had undergone root canal treatment on 26 and 36 four years ago and there was no relevant medical history. Extraoral examination revealed facial asymmetry with widening of the inferior border of mandible on the right side as well as a bulge over the right supraorbital region []. Intraoral examination revealed a swelling extending from midline to ramus of mandible on the right side measuring about 14 × 7 cm. Mucosa over the swelling was normal in color. The swelling was hard in consistency, sessile and non- tender and not fixed to the underlying structures.\nRadiographic examination reveals enlarged body of the right side of mandible. An ill-defined radiolucency measuring about 7.5 × 2 cm is seen extending from the condylar rod to the right angle of the mandible. There is also an ill-defined radiolucency of size 2.5 × 1.5 cm extending from the distal side of 48 to the mesial side of 46. A multilocular diffuse radiolucency can also be seen at lower border of mandible [].\nThe plain computed tomography (CT) scan study shows the bilateral involvement of frontal bone with complete obliteration of frontal sinuses and right sphenoid sinuses, partial obliteration of right ethmoid sinus and bilateral maxillary sinuses. Few expansile lytic lesions in mandible with break in the outer cortices, largest measuring 2 × 1.8 cm (TD × AP) in the region of right lower third molar causing break in the inner cortex and predominantly involving bilateral condylar process and ramus of right mandible [].\nThere was high elevated serum alkaline phosphatase level, that is 825 Bodansky units. Incisional biopsy was done on the right side of the mandible near external oblique ridge below third molar. Moderate bleeding was encountered.\nHistopathological report showed bony trabeculae with numerous prominent basophilic reversal lines and entrapped osteocytes. Bony trabeculae are lined with osteoblasts and there was also evidence of few multinucleated osteoclasts. Marrow spaces were filled with fibro-vascular connective tissue with large number of blood vessels filled with RBCs [Figures and ].\nBased on the clinical, radiographic, CT scan, biochemical and histopathological findings, diagnosis of PDB was rendered.
In February 2007, a 14-year-old Caucasian girl was admitted to our Ear, Nose and Throat department. Since the beginning of 2006 she has been monitored for gradual loss of vision in her right eye. At the out-patient eye clinic of our hospital, she was diagnosed with reduced vision in her right eye due to partial atrophy of her optic nerve. Her vision ranges from 2 to 4m; the use of optical correction fails to improve the condition. The position of her eyes is parallel, her eyeballs are movable in all directions, the anterior segment is intact, optic media are transparent, and pale atrophic papillae are seen in the right fundus; the findings are unchanged without progression. The findings in the left fundus are physiological. Baseline perimetry revealed absolute central scotoma up to 25 degrees, which coincides with the blind spot. Eye findings correspond to optic nerve compression. In May 2006, magnetic resonance imaging (MRI) scanning was performed with the conclusion of suspected tumour of the prechiasmatic segment of her right optic nerve. In the summer of 2006, a temporary improvement in clinical symptoms occurred; however, during the autumn of the same year her symptoms worsened again and she developed headaches in addition to visual problems. In December 2006, a follow-up MRI scan was performed and revealed significant enlargement of the subarachnoid space of her right optic nerve (Figures and ). An enlarged bony structure was also seen laterally in the optic canal area, which was surrounded by solid tissue, probably compressing the optic nerve in the optic canal. An additional computed tomography (CT) scan was performed, which revealed a robust anterior clinoid process (Figures , , and ) and otherwise no further pathology.\nEndonasal endoscopic decompression of her optic nerve was performed (Figures and ). Medial and posterior ethmoidectomy and sphenoidotomy were performed under general anaesthesia using endoscopic techniques; subsequently bone was removed from the medial wall of her orbit and her optic canal was gradually approached. The optic canal narrowing was seen about 13mm behind the tip of her orbit, which enlarged again in the direction towards the chiasm. A part of the bone in the narrowed area was removed using punches, shaver and diamond bur to release the optic nerve. The histology report described the bone formation as a lamellar compact bone. She was subsequently referred to the care of an ophthalmologist and underwent regular eye examinations. Her condition markedly improved, with persistent central scotoma in her right eye.\nIn May 2008, her vision worsened again in her right eye, and she complained of blurred vision without diplopia. A CT scan revealed narrowing of the right optic canal and more robust optic nerve in her right eye, without other obvious pathology in the canal or sella turcica. An eye examination revealed reduced vision in her right eye, with non-progressing atrophy of her optic nerve; otherwise the findings were unchanged compared to the previous evaluations. She was admitted for revision endonasal endoscopic surgery, during which narrowing of her right optic canal was revealed due to bone apposition similar to bone exostosis. This bony process was removed using a cutter and her optic nerve was released. After surgery, her visual acuity gradually improved.\nIn January 2010, she presented for recurrent visual impairment in her right eye, without obvious pathology according to the CT scan. Revision endonasal endoscopic surgery was performed, during which her right optic nerve was not compressed over its course in the optic canal, but was more robust than her left optic nerve. New pathology was ruled out and an MRI scan was recommended, which confirmed a robust right optic nerve compared to the thickness of her left optic nerve. It is probable that the thickening of the nerve developed as a result of pathological processes that took place. The thickening of the nerve has a stationary character. Currently, he is stabilized and followed up by her ophthalmologist. She achieved a significant improvement in visual acuity, which is stable at 4m, follow-up perimetry in 2014 revealed a persistent central scotoma in her right eye, including partial scotoma in the upper temporal quadrant. Perimetry of her left eye provided physiological findings, without any failures in her visual field.
A 65-year-old woman with a medical history significant for anal cancer presented for mediport removal. Past surgical history included an elective thyroidectomy two years prior for multinodular goiter. The port was placed 11 years prior for chemotherapeutic treatment of anal cancer, which subsequently cleared with complete clinical response following treatment with the Nigro protocol. Thereafter, the patient failed to continue her scheduled postoperative follow-up evaluations. The patient was referred from her primary care physician for port removal, which was proceeded with as requested. The port-a-cath had been inserted into the left internal jugular vein with the catheter tip in the superior vena cava, and the port was secured in the subcutaneous tissue of the left chest wall along the left upper breast border. Under monitored anesthesia care and local anesthesia, an incision was made over the port site, approximately 2 cm inferior to prior incision site. The port and catheter were identified covered with chronic capsule. Subcapsular dissection allowed for complete freeing of the port, but significant resistance was felt upon pulling the catheter. To reduce subcutaneous tunnel resistance along the catheter tract, a second incision was made superior to the primary incision, closer to the site of left internal jugular vein catheter insertion. A clamp was placed on the proximal catheter end and the catheter transected. The explanted metallic port (measuring 2.5 x 2.5 x 1.3 cm) and transected proximal catheter (3.5 cm) were removed. We continued to dissect along the catheter tract to the point of insertion into the left internal jugular vein, followed by venotomy and temporary local tonsil clamp placement. At this point, no reduction in resistance was felt despite sustained traction, suggesting a possible intravascular central vein adhesion to the catheter. A cardiothoracic surgeon was consulted, and the patient was converted from monitored to intratracheal general anesthesia care. A guidewire was inserted followed by no change in resistance of the catheter tip, and there was a confirmed absence of backflow from the catheter. Fluoroscopic imaging was completed (Figure ), indicating the catheter tip was fixed to the sidewall of the superior vena cava. This fixation made the complete removal of the catheter by traction unsafe. We concluded that further attempt to retrieve the stuck catheter may result in vascular injury, and opted instead to shorten the distal catheter end while leaving the adherent tip in place. Three large surgical clips were placed and the distal catheter was transected (transected piece measuring 11.5 cm in length). All incisions, affected subcutaneous planes, and skin were closed in anatomic planes.
A 27-year-old post-partum female presented to the clinic with severe lower back pain radiating to the left lower limb for one month. The visual analog scale (VAS) was used to measure pain intensity and pain affect and the Oswestry Disability Index (ODI) was used to measure the degree of disability due to lower back pain [-]. The patient's initial scores on the VAS and ODI scales were 9 and 46 respectively.\nThe lower back pain developed suddenly during labor. It was initially masked by labor pain and was unrecognised for a few days post-partum. The pain gradually increased over a few weeks and radiated to the dorsum of her left foot. She was given a trial of conservative management for three weeks following which she failed to show any signs of improvement. She had no history of lifting heavy weights, previous surgery, injuries to the back, fever, weight loss, abnormalities of bowel or bladder movements, weakness or loss of coordination of upper or lower limbs.\nOn examination, vital parameters were within normal limits. Her gait was slow, limited by lower back pain radiating to the dorsum of her left foot. Inspection of the lower back revealed normal anterior curvature of the lumbosacral spine without overlying skin abnormalities. Her range of motion was limited in a forward flexion. There was moderate tenderness present over the lower back. Straight leg raise (SLR) was 20 on the left side. This test is done with the patient in supine position. The examiner lifts one leg upward keeping the knee fully extended. If the patient experiences sciatic pain during the leg raise, the test is positive and suggests herniated disc as a possible cause of pain. Power in the left extensor hallucis longus (EHL) was 3/5 and the left ankle reflex was absent. All sensations were intact. Peripheral pulses were well felt bilaterally in both lower limbs. A systemic examination did not reveal any significant abnormality.\nA magnetic resonance imaging (MRI) scan at this point showed a large herniated disc bulge at the level of L4-L5, causing a large radiculopathy (Figure ).\nThe patient was then given a trial of conservative management in the form of bed rest, physiotherapy, and analgesics. However, with conservative management, the patient did not show improvement and decided to undergo ozone disc nucleolysis.\nThe procedure was done under local anaesthesia in the catheterization laboratory on the Siemens rubber tired gantry (RTG) single-plane digital subtraction angiography (DSA) suite. With the patient in prone position, the C-arm was rotated such that the lateral one-third of the disc remained free from the lateral margin of the facet joint. Under local anaesthesia, a 22 gauge 17-cm long needle was introduced percutaneously via paraspinal approach into the disc via the transforaminal route. The intradiscal position of the needle was confirmed in the antero-posterior and lateral view. The ozonator was used to generate ozone on site by partly converting medical grade oxygen. 10 ml of ozone-oxygen mixture (concentration of 30 micrograms per ml) was injected intradiscally and in the periganglionic epidural space. Additionally, a mixture of local anaesthetic agent lidocaine 2%, hydrocortisone 100 mg, triamcenolone 45 mg, and hyaluronidase 1500 IU was also injected in the periganglionic epidural space.\nPost-procedure, the patient was advised to remain in supine position for one hour, following which she was gradually mobilised and advised light activity for the following three weeks. She showed significant improvement immediately after the procedure and there was a further improvement in symptoms over the next six weeks.\nPost-procedure follow-up at three months revealed a VAS score of two and an ODI score of 10. The axial and sagittal T2-weighted (T2W) images on the MRI scan at three months showed significant resolution of the large postero-central disc protrusion at the level of L4-L5 following ozone disc nucleolysis (Figure ).\nPost-procedure follow-up at six months revealed a VAS score of 1 and an ODI score of 4. The patient was counselled to avoid strenuous activity for six months. She was also advised physiotherapy to strengthen core muscles. There were no complications following the treatment.\nThe MacNab criteria was used to determine the satisfaction and well-being of the patient post procedure []. Her outcome on MacNab was in the excellent category.
A 40-year-old male was referred to the Department of Oral and Maxillofacial Surgery five months after a severe motor vehicle accident for treatment of pain and inability in closing the mouth. The patient had sustained severe head injury for which he had been hospitalized in the trauma department for 45 days. Craniotomy had been carried out and a cerebral shunt inserted to reduce intracranial pressure. In extra-oral examination, a deviation was present in the orbital axis, which was a sequel of the head injury. The mouth was found open and the mandible was anteriorly displaced (). Depressions anterior to the tragus were visible bilaterally and the condyles were fixed and palpable in front of the ears. Intraoral findings included loss of several teeth; anterior displacement of the lower jaw, and anterior open bite with an inter-incisal distance of 40 mm. Anterior displacement of both condyles was seen on the panoramic radiograph () and confirmed by CT views. According to the findings from the history and physical examination, a diagnosis of irreducible long-standing condylar dislocation was established and the patient was scheduled for treatment under general anesthesia. The occlusal models were prepared to check the postoperative occlusion. The procedures were explained to the patient and a detailed consent form was obtained. General anesthesia was administered via naso-endotracheal tube using intravenous midazolam, phentanyl, muscle relaxants, and propofol. Under general anesthesia, noninvasive methods such as different modalities of jaw manipulation and controlled tractions failed to reduce the jaw. Subsequently, the angles of mandible on either side\were exposed via bilateral Risdon approaches to apply traction using bone hooks in the sigmoid notch and heavy traction wires passed through bur holes at the angle (). These procedures also failed to bring the condyles into their appropriate position. Therefore, bilateral vertical osteotomies were carried out and the mandible was guided to the normal occlusion. Intermaxillary fixation was applied with arch bars after removing the throat pack and kept for 10 days; subsequently, active mouth opening exercises were ordered. Proximal and distal segments were checked and the incisions were closed in three layers.\nThe postoperative period was uneventful and the patient was discharged from the hospital four days after surgery. The patient was followed regularly every week for the first month and then every 3 months. One year after surgery, the patient had normal occlusion with an inter-incisal opening of 40 mm without open bite (). Despite the extra-glenoid position of the condyles on the postoperative panoramic view, the proximal segments revealed a good relationship with distal segments (Figure & ).
Case reports are exempt from institutional review board approval at our institution. A 75-year-old woman was evaluated for long standing right-sided nasal obstruction, dependent mouth breathing, clear rhinorrhea, congestion, and hyposmia not relieved by intranasal steroids or nasal irrigations. She initially presented with a history of previous endoscopic sinus surgery in Russia more than 20 years ago followed by nasal polyposis treated with ambulatory cauterization. She was also noted to have a history of nonmelanotic skin cancers of the nose treated with radiation in Russia. Physical exam findings included a fleshy intranasal lesion that, in the setting of previous nasal skin cancer treated with radiation, raised a concern for possible secondary carcinoma. Computed tomography showed complete opacification of the right maxillary sinus, obstruction of the right ostiomeatal complex, and soft tissue density in the right nasal passage (). Initial biopsy revealed a nasal mass that originated in the right inferior meatus. Pathology showed multiple polypoid fragments lined by a respiratory type epithelium with underlying edematous stroma with mild chronic inflammation. There was invagination of the surface epithelium into the underlying stroma resulting in nested aggregates of bland glandular and mucinous cells and focally benign squamous epithelium (). These features were found to be consistent with a benign inverted papilloma. The patient presented with continued nasal obstruction and was evaluated for definitive treatment. Given her diagnosis of inverted papilloma and chronic rhinosinusitis, complete excision and revision endoscopic sinus surgery was recommended. During endoscopic sinus surgery, an exophytic mass with abnormal maxillary mucosa was seen emanating from the left inferior meatus that was thought to originate from the right maxillary sinus, given that it was protruding through a bony dehiscence into the inferior meatus and nasal cavity. Right partial inferior turbinectomy was performed, along with right extended maxillary antrostomy and stripping of maxillary mucosa to remove the entirety of the presumed inverted papilloma base within the maxillary sinus. Intraoperative and postoperative pathology again showed inverted papilloma. Given
An 11-year-old girl was referred to the pediatric assessment unit due to a report of a lesion on her distal tibia. The X-ray was performed a few days prior to presentation and was reported and flagged up by the radiologist, which subsequently led to calling the girl in for a review on the weekend. The X-ray showed a lesion extending into the distal tibia (Figure ).\nThis X-ray triggered an urgent assessment of the patient by the general practitioner (GP) and an urgent MRI request was made to identify any bone tumors. Following is the chronological history of the case.\nThe patient originally presented in the accidents and emergency department (A&E) three years ago with the complaint of ankle pain. An X-ray was done at the time of original presentation with only the ankle and the foot visible (Figure ). No abnormality was detected and the patient was sent home on analgesia.\nAs seen in the original X-ray above, a complete assessment of the limb is not possible from this image as it does not contain a full view of the affected extremity.\nThe patient kept on having intermittent complaints of right lower leg pain over the course of two to three years until it exacerbated considerably in the last year leading to multiple visits to the GP. In the absence of any other alarming feature and any preceding or current history of fever, infection, trauma, or superficial skin changes, the pain was managed symptomatically.\nThe pain continued to worsen despite symptomatic management and a new X-ray was requested. This time, the X-ray included the distal tibia. Following is the radiolucent lesion visible very clearly on the new X-ray (Figure ).\nThis alarming image prompted the need for an MRI scan of the lower limb and her case was escalated to the paediatric orthopaedic consultant. Following are the images from the MRI (Figures , ).\nThe radiologist after reviewing the above images raised concerns of a suspected bone tumor on his report. In light of the report and concerns of the radiologist, the patient was asked to present in the paediatric assessment where she was reassessed. Worsening of the intermittent pain in the lower limb along with mild swelling was noted around the ankle in the history and examination. As mentioned earlier, there was no complaint of fever, and the patient was well otherwise. Blood tests were reviewed which were also normal. However, the new point of concern in her reassessment was that her symptoms had worsened to a point where she had started experiencing difficulty in weight-bearing. Therefore, an urgent referral was made to the regional centre for expert advice regarding diagnosis and further management.\nA diagnosis of Brodie's abscess was made and the patient was booked for a semi-elective surgery to treat the abscess. When the patient presented for the surgery, the abscess had extravasated from the skin and was protruding outwards (Figures , ).\nThe abscess was incised and drained. Intra-operative images are attached below showing the extent of invasion of the abscess into the growing tibia of the 11-year-old girl (Figure ).\nHoles were drilled in the bone to encourage blood supply. Curettage was done and Vancomycin 1g was instilled in the cavity. The wound was subsequently approximated and closed (Figure ).\nPost-operation recovery remained uneventful and the pain was managed with simple analgesia. She was also started on long-term antibiotics therapy (two weeks of IV followed by two weeks of oral doses) and follow-up was arranged with the paediatric orthopaedic team. Cultures taken intra-operatively grew Staphylococcus aureus, which, as mentioned previously, is one of the typical organisms associated with Brodie’s abscess.\nThe patient was seen in the clinic in six weeks where she was assessed to be doing well clinically and also showed evidence of good healing on her new X-rays (Figure ).
A 21-year-old male presented to the primary physician with complaints of low back ache with radicular pain to both legs without any neurological deficit eleven years ago. He was empirically started on Anti tuberculosis drugs by the primary treating surgeon. After 5 months of treatment he developed worsening of symptoms and was referred to us for further management. He came with low back ache and bilateral lower limb radiating pain. On examination he had hypoesthesia at L4 and L5 dermatome with no motor and autonomic disturbances. Computed tomogram showed bony destruction of L4 vertebrae () and magnetic resonance imaging (MRI) scan with contrast showed well defined lesions involving the L4 vertebral body, more on the left side and lower end plates of L3 and upper end plates of L5 vetebrae. The lesion measured 4×3 cm and had fluid intensity with no septation, hypo intense on T1 weighted sequence, hyperintense on T2 weighted sequences and did not show any enhancement with gadolinium (). The differential diagnosis was of giant cell tumor, aneurysmal bone cyst and hydatid cyst of L4 vertebra Chest radiograph and ultrasound abdomen was done to rule out involvement of the lungs and liver. Anterior debridement of L3-L5 vertebral body and L3 to L5 tricortical iliac crest bone grafting via anterolateral retroperitoneal approach was done. Then posterior stabilization with mono-axial screws and bone grafting of L3 to L5 was done under general anesthesia. Intra operatively the wound was washed with betadine and 20% hypertonic saline. Histopathological examination showed viable and necrotic lamellar bone enclosing fibrocellular tissue with fibrosis and necrosis. Fragments of laminated membrane lined focally by degenerate germinal layer were seen. Post surgery he recovered without complication. He was started on oral albendazole 400 mg twice a day for four weeks and this regimen was continued for 3 more cycles with 2 week interval between each cycle [] with regular monitoring of Liver Function test. Patient was reviewed at 3rd month and 6th month and thereafter annually and was asymptomatic, implant was in good position with full recovery from hypoesthesia. Eight years after the surgery he presented with low back pain and bilateral leg pain, more on the right side aggravated by walking (). He had motor power of 2/5 of the right L4, L5 myotome. Since the stainless steel implant was in situ, computerized tomogram (CT) myelography was done (). This showed soft tissue lesion involving L3, L4, and L5 vertebrae extending posteriorly into the spinal canal impinging the thecal sac and exiting L3, L4 nerve root on the right side and L4 nerve root on the left side. Soft tissue mass with a thin calcified capsule and few hypodense areas were suggestive of recurrence of the hydatid disease. Implant removal, debridement and wash out of infected tissue and posterior stabilization with poly-axial screw from L1, L2, S1, and iliac were done. He was started on oral albendazole 400 mg twice daily for 4 weeks. One month post implant exit, MRI () was done to delineate the extent of soft tissue lesion. A month later anterior retroperitoneal debridement, L4 corpectomy, L3 to L5 fusion with titanium polyaxial screws and cages and postero-lateral bone grafting was carried out. Histopathology showed that in some areas bone and marrow was replaced by amorphous granular eosinophilic necrotic tissue containing few structures resembling degenerated hydatid cyst wall with fragments of laminated membrane lined focally by degenerate germinal layer. Presence of hooklet bearing scolices confirmed the diagnosis of hydatid cyst. Postoperatively he recovered without complications and his neurological deficit improved completely. Postoperatively patient was started on oral albendazole 400 mg twice a day for four weeks, repeated after 2 weeks for two years, monitoring Liver Function once in three months. Patient was reviewed every year after surgery and at the end of 3 years plain radiograph showed implants () in good position and CT scan showed sound posterolateral fusion. MRI showed no signs of residual lesion. In view of the possibility of further recurrence patient has been advised a yearly follow-up.
A one month old baby girl was brought by her mother to the lactation clinic for further evaluation due to the difficulty of breastfeeding. The mother complained of pain in the nipple which was present throughout the entire breastfeeding session and usually recurred with each episode of breastfeeding. Two weeks after delivery, the mother noticed a tooth on the baby’s lower left gum. A week later, she noticed an ulcer under her baby’s tongue, which occasionally bled.\nIn light of the continuous pain during breastfeeding, the mother became reluctant to continue and infant formula milk was given as a substitute. Self-examination by the mother did not reveal any nipple crack or breast injury. Her antenatal history was uneventful. There was strong family history of natal teeth, and this baby’s siblings had similar problems.\nOn examination, the mother’s breast appeared to be normal and consistent with a lactating breast. There was no inflammation or engorgement. Some mild eczema was noted around the nipple, but there was no infection. Cervical and axillary lymph nodes were not palpable.\nExamination of the oral cavity of the baby revealed a neonatal tooth over the left anterior region of the mandibular ridge. The tooth measured 2 mm × 1 mm in size, was whitish opaque, and had Grade II mobility (Fig. ). There was also a whitish ulcer over the ventral aspect of the tongue measuring 1 mm × 1 mm (Fig. ). The parents declined radiological investigations and tongue biopsy for the baby. Hence, the clinical diagnosis was neonatal tooth associated with Riga-Fede disease causing difficulty in breastfeeding.\nThe baby was then referred to the pediatric dental surgery department. After a thorough discussion with the parents, the neonatal tooth was extracted under local anesthesia. The tooth had a crown but lacked a root. Following extraction, the baby did not have any complication such as bleeding and infection. The wound healed well within 2 days and she successfully resumed taking breast feeds.\nCurrently, at 10 months post-extraction, the child is growing well and still breastfeeding. Examination of the oral cavity revealed that only one central lower incisor tooth was present (Fig. ).