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A 45 year-old woman came to our department complaining of an episodic headache for more than half year and worsened for 2 weeks. A typical headache attack would start with a pulsating pain in the two pterions of the bilateral temporal regions. The bilateral pain would synchronously spread backward in a coronal way and converged at a midline point of the occipital area, the occipital protuberance, within ten minutes. After converging of the bilateral pain, the head pain would become fixed and restricted within this pain spreading trajectory, i.e. a line-shaped area of 5–10 mm in width linking the two pterions of the bilateral temporal regions and the occipital point. The pain, pulsating in characteristic, reached its peak in severity within 10–20 min and was described as moderate to severe, and the patient denied this pain epicranial but complain it intracranial. The headache attacking was often triggered or precipitated by anxiety but not related with menstruation. Dizziness, head heaviness, photophobia and phonophobia but not nausea or vomiting usually accompanied the headache attacking which would last for half day or remitted over a sleep. The headache usually attacked 3–4 times a month in the past half year, but attacked almost every day with occasional 1 day remission in recent 2 weeks. In recent 2 weeks, a typical coronal line-shaped head pain started with left side tinnitus which usually lasted for 10 min, otherwise the head pain and the left side tinnitus occured simultaneously, and the headache became more severe (7–8 in scale of 10) compared to those of the past half year (4–6 in scale of 10). Analgesics, a compound consisting of aminopyrine, phenacetin, caffeine and phenobarbital, in combination with flunarizine (5 mg once), may partially relieve the coronal line-shaped head pain in 5 min, but trial of the analgesics alone failed to relieve the pain. She had a history of migraine without aura attacks in a frequency of once per 2–3 months for more than 10 years while she was 20–30 year old, beginning after giving birth of her son and remitting 15 years ago. The migraine headache attacking was prone to be triggered by anxiety or dysphoria, and usually accompanied with dizziness and head heaviness, but with no nausea or vomiting. Partial relief of the headache could be achieved after taking analgesics, a compound consisting of aminopyrine, phenacetin, caffeine and phenobarbital, but a complete elimination of the headache achieved over a sleep.\nThere was no family history of migraine or other recurrent headaches, except for her son. Her son of 25 year old age, the first and the only one child, had two episodes of abrupt most severe (10 in scale of 10) lancinating pain over the whole head 4 and 2 years ago respectively. The episodes lasted for about 30 min and remitted spontaneously. According to the International Classification of Headache Disorders (ICHD-III, beta version) (IHS ), the headache was retrospectively diagnosed as thunderclap headache (TH). On the other hand, her son had recurrent severe vertigo attacks, a feeling of spinning around of the background, for 12 years in a frequency of 1–2 attacks per week, beginning at his age of 13 year old. Each episode of vertigo attacking would be preceded or accompanied with 1–2 min of amaurosis and the duration of the vertigo attacking would be 5–10 min. The vertigo attacking was usually accompanied with photophobia but with no phonophobia. No nausea or vomiting was noticed during the vertigo attacking and no headache attack found around the vertigo attacking. And he had a feeling of head heaviness and could not walk steadily while the vertigo was attacking. According to the International Classification of Headache Disorders (ICHD-III, beta version) ((IHS). ), the diagnosis of the episodic vertigo attacks could be grouped under “episodic syndromes that may be associated with migraine” in ICHD-III, beta version.\nA complete physical and neurological examination was performed to exclude rhinitis, otitis media or cranial neuritis revealing normal. Inspection, palpation and sensory examination of the pain area, as well as the areas innervated by the lesser occipital nerve (LON), greater occipital nerves (GON) and auriculotemporal nerve (ATN) were all normal. Routine blood work-up with erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), blood electrolyte and brain magnetic resonance imaging (MRI) and magnetic resonance venography (MRV) were all normal either.\nThe patient accepted prophylactic treatment with sodium valproate (500 mg twice a day) and her coronal line-shaped headache was dramatically alleviated leaving with only 1–2 times of mild headache attacks per month during the next three-month follow-up. Her son also accepted treatment with sodium valproate (500 mg twice a day) and his vertigo did not recur during the next 3 months follow-up. |
A 78-year-old female with past medical history of hypertension, chronic obstructive pulmonary disease, gastroesophageal reflux disease, dyslipidemia, and panic disorder was admitted to an outside hospital after falling in her home. Cranial CT revealed an intraparenchymal hemorrhage with intraventricular extension within the right frontal segment of her brain. A subsequent CT angiogram revealed an aneurysm of the anterior communicating artery. The patient was transferred to our hospital for further care and evaluation.\nThe hemorrhage was categorized as world federation of neurosurgeons (WFNS) grade 2 and Fisher grade 4. Following admission to the neurological intensive care unit (ICU), the patient underwent endovascular coiling of the aneurysm after general endotracheal anesthesia was initiated. An attempted intraoperative nasogastric tube placement was traumatic, resulting in bleeding from bilateral nasal passages and blood pooling into the oropharynx. An orogastric tube was then placed successfully. After the procedure, on postoperative day 1, the patient remained ventilated and was transferred to the ICU where the ear, nose, and throat (ENT) service repaired the oropharyngeal trauma.\nOur critical care service was initially consulted on hospital admission day five for acute respiratory failure secondary to a new left lower lobe atelectasis and small left pleural effusions as indicated from a chest X-ray. Upon initial bronchoscopy inspection, the distal tip of the ETT was found to be 1 cm above the carina; therefore, the cuff was deflated, and the ETT was withdrawn 2 cm and secured in place. The bronchoscopy further revealed copious old/clotted blood in the distal hypopharynx and between the wall of the ETT tube and lining of the trachea. Additionally, mild-to-moderate tracheobronchitis in the right mainstem bronchus and the left lung was found to be nearly completely occluded with a “cast” of old blood and mucus outlining the entire length of the left bronchus.\nFour days later, our service was consulted for PCT placement. After confirmation of informed consent and patient identification, the patient was medicated with 200 mcg of fentanyl, 4 mg of midazolam, and 150 mg of rocuronium. A flexible fiberoptic bronchoscope was advanced into the ETT before starting the procedure; no abnormalities were observed. The ETT cuff was deflated and slowly moved directly above the vocal chords. A second bronchoscopic view revealed mild-to-moderate persistent nonpurulent tracheobronchitis and an ulcer on the posterior tracheal wall noted 3-4 cm above the carina extending 2 cm in length and 1 cm in width (). Due to the length of the ulcer, obtaining a proper seal with the balloon of the Shiley tracheostomy tube was expected to be difficult. Therefore, the tracheostomy procedure was aborted, and the ENT service was consulted for evaluation. The ETT was changed to a reinforced ETT following bronchoscopic guidance. FiO2 was weaned from 100% to 40%, oxygen saturation was kept greater than 92%, respiratory rate was reduced to maintain a PaCO2 between 35 and 45 mmHg, and a bronchodilator treatment was recommended.\nLater that evening the patient was transported to the operating room for an open tracheostomy by the ENT service. After the incision (approximately 2 cm below the cricoid cartilage) and dissection (superior to the thyroid isthmus), the second tracheal ring was identified. The ETT was removed just superior to the tracheal window under direct visualization with a flexible bronchoscope. The before-mentioned ulcer was identified inferior to the tracheal window and confirmed with its distal end 3 cm above the carina. Upon careful fiberoptic evaluation, the surgeon suspected the ulcer to be a tracheoesophageal (TE) fistula. At this time, the decision was made to place another reinforced ETT through the tracheal stoma, sutured to the skin, with the tip 0.5 cm above the carina and the cuff inferior to the TE fistula. The severity and location of the ulcer warranted an esophagogastroduodenoscopy to rule out a TE fistula. After evaluation with a rigid esophagoscope, a small amount of petechial type hemorrhage next to the tracheal ulcer, but no fistula, was identified.\nFour days later, the ENT service replaced the reinforced ETT with a size six cuffed TracheoSoft XLT Extended-Length Tracheostomy Tube to bypass the ulceration. Over the following week, the patient was weaned from mechanical ventilation, determined stable, and discharged with the tracheostomy cannula sutured in place. |
A 38-year-old woman who initially presented for evaluation of left-sided proptosis was found to have a left orbital mass depressing the globe inferiorly on MRI. There was an extension of enhancement in the left sphenoid along the orbital, lateral frontal, and temporal dura (Figures -). A biopsy was performed that identified the mass as an orbital meningioma, WHO grade I. An orbital-zygomatic craniotomy with lateral orbitotomy was performed for resection of the tumor. Over the next three years, the patient developed episodes of intermittent headaches and seizures, after which she began developing blurriness and a sensation of fullness in the left eye, as well as worsening of her headaches. An MRI was performed that demonstrated an enhancing extra-axial mass along the left greater sphenoid wing with extension into the left aspect of the left orbit measuring 4.0 × 2.7 cm, consistent with recurrence of her meningioma. A left frontotemporal craniotomy was performed and the tumor was resected followed by postsurgical radiation.\nThe first orbital resection specimen in 2013 was most consistent with meningioma, CNS WHO grade 1. The microscopic evaluation demonstrated meningothelial cells infiltrating bone and muscle with bland cytology. No mitotic figures were identified. However, there were areas of fibrous change consistent with transitional meningioma. The tumor demonstrated a Ki-67 proliferation index of 4-6% with focal EMA and S-100 positivity. PR immunohistochemistry was negative. The second orbital resection specimen was most consistent with atypical meningioma, WHO grade 2. The microscopic evaluation demonstrated islands of cells with round to oval nuclei with poorly defined cell borders and nuclear pseudo-inclusions adjacent to muscle. Up to seven mitotic figures were identified in 10 HPFs. The tumor demonstrated a Ki-67 proliferation index of 12-15%. The tumor cells were positive for EMA and focally for PR.\nDuring her one-year follow-up after the second resection, the patient denied any worsening of her vision. However, her headaches, which she had been experiencing for several decades, persisted; this was attributed to a medical history of trigeminal neuralgia. |
A 32-year-old G3P2 woman at 14 weeks’ gestation was brought to the emergency department by ambulance for sudden-onset syncope. She had 2 previous cesarean deliveries. The patient and her husband were elementary school teachers with 2 children, aged 5 and 3 years. The patient had a small ventricular septal defect that did not require treatment. She had no history of other past illness, including seizures or syncopal episodes, and she took no medication. On the day of her syncopal episode, she consumed the same evening meal as her husband and 2 children. She then took a bath. The episode occurred suddenly, while she was putting her children to bed and was actually in bed with them. Her 5-year-old child immediately called his father, who called an ambulance. On arrival to the emergency department, her Glasgow Coma Scale score was 6/15 (E1V2M3), and she was uttering strange sounds and exhibiting restlessness, with tonic extension of both arms. We treated her in accordance with the Immediate Cardiac Life Support protocol, developed by the Japanese Association for Acute Medicine and launched in 2002 as a part of the introductory training course for cardiopulmonary resuscitation []. Both her pupils were 6 mm in diameter and nonreactive to light. She had no history of ingestion or exposure and no clinical signs associated with poisoning. We therefore did not perform toxicology screening. Her initial vital signs were: blood pressure, 137/65 mmHg; pulse, 97 beats/min; body temperature, 36.7 °C; and oxygen saturation, 95% on 10 L/min oxygen administered by face mask. Her oxygen saturation on room air was unknown because she arrived at the hospital with a face mask in place. There were no other cardiopulmonary resuscitation interventions performed before her arrival at our hospital. Blood samples were collected intravenously and by femoral artery puncture, and a complete blood count, biochemistry profiling, and blood gas analysis were performed. Her hemoglobin level was 13.5 g/dL, and her platelet count was 150, 000/μL. The blood glucose concentration was 143 mg/dL, and her liver enzymes were slightly elevated, with an aspartate aminotransferase level of 72 IU/L and an alanine aminotransferase level of 15 IU/L. Her serum pH was 7.353, blood lactate was 6.1 mmol/L, and arterial partial pressure of oxygen was 173 mmHg. As her blood samples did not coagulate, neither prothrombin time nor activated partial thromboplastin time was measurable, suggesting a deficiency of coagulation factors. She had no genital bleeding, but transabdominal ultrasonography revealed a subchorionic hematoma (SCH), approximately 8 × 3 cm. This had been observed 1 week prior and was essentially unchanged in size. The fetus was living. Computed tomography (CT) and magnetic resonance imaging of the head and thoracoabdominal contrast-enhanced CT revealed no evidence of pulmonary embolism, venous thrombosis, or intraperitoneal bleeding. Extravasation of contrast into the SCH was observed (Fig. a).\nTwo hours after her arrival, the patient exhibited a drop in blood pressure to 75/49 mmHg together with a decrease in her Hgb level, from 9.8 g/dL to 6.3 g/dL, and a decrease in her platelet count, from 94,000/μL to 70,000/μL. We started transfusing packed red blood cells (PRBC), fresh frozen plasma (FFP), and concentrated platelets under the presumed diagnosis of DIC. She gradually regained consciousness and ultimately responded to her name. The DIC resolved, and her fibrinogen level increased, from unmeasurable at 2 h after arrival to 36 mg/dL at 5 h and 109 mg/dL at 8 h. Despite these gradual improvements, her blood pressure decreased again and her condition became unstable. Pelvic CT performed 11 h after arrival revealed an increase in the size of the SCH and a large retroperitoneal hematoma (Fig. b).\nWe suspected that the intrauterine hemorrhage and retroperitoneal bleeding, presumably originating from the uterus and the femoral artery puncture site, respectively, might worsen her general condition and coagulation status. We provided additional blood-component transfusions and subsequently preformed a supracervical hysterectomy. The ratio of the preoperative transfused products, PRBC: FFP: platelets, was 1:1.4:1. We observed a massive retroperitoneal hematoma and noted that the uterus was larger than expected but not ruptured, indicating that the retroperitoneal bleeding did not originate from the uterus. The amount of intraoperative blood loss was approximately 2400 g. On postoperative day 6, she had lower abdominal pain with a fever of 37.7 °C and an intrapelvic hematoma was detected by CT; infection was suspected. Drainage and flushing of the hematoma with antibiotic treatment improved her condition. She was discharged from the hospital 22 days after the hysterectomy. She is now back to her job as an elementary school teacher. She has had no sequelae and requires no medication.\nConsidering our patient’s clinical course and laboratory and imaging data, we felt that AFE was the most likely cause, despite the absence of severe hypoxia, because AFE provokes both DIC and maternal collapse resulting in reduced or absent consciousness [, ]. We therefore performed a detailed pathologic examination of the hysterectomy specimen, revealing an edematous myometrium. The presence of amniotic fluid inside a uterine blood vessel was evidenced by positive staining for zinc coproporphyrin-1 (Zn-CP1), which is reactive with fetal and amniotic components [] (Fig. a). In addition, many complement component 5a (C5a) receptor-positive cells were present in the myometrium (Fig. b), suggesting the presence of an anaphylactoid reaction []. Serum levels of Sialyl Thomsen-nouveau antigen (STN), specific for amniotic fluid, were elevated to 280.0 U/mL (reference value, < 45.0 U/mL), and her interleukin 8 (IL-8) level was elevated to 494.0 pg/mL (reference value, < 2.0 pg/mL). These findings collectively and strongly suggested the presence of AFE, in particular DIC-type AFE []. |
A 70-year-old woman was admitted to our hospital with a 3-day-old acute myocardial infarction. Although the patient reported adherence to the prescribed medication regimen, she developed heart failure with hypotension and oliguria the next day. Coronary angiography performed under intra-aortic balloon pumping demonstrated total occlusion of the proximal left anterior descending artery (LAD). Subsequent percutaneous coronary intervention achieved successful revascularization of LAD. The patient recovered steadily and gradually. However, four days later, her condition deteriorated suddenly and she went into shock. Her echocardiography results revealed cardiac tamponade with substantial pericardial effusion. Pericardiocentesis was performed, resulting in massive continuous drainage, and she was referred to us for emergency surgery.\nThe patient was markedly cyanotic and in cardiogenic shock with systolic blood pressure of 70 mm Hg. A large dose of dopamine had been administered. She was intubated immediately, and the results of blood gas analysis showed marked metabolic acidosis with a pH of 7.251 and a base excess of −13.2 mmol/l. Emergency surgery was undertaken via a median sternotomy. Upon opening the pericardium, a blowout rupture of the LV free wall was found. A large volume of fresh blood was expelled rapidly from the tear at the LV base, between LAD and its diagonal branch. We were unable to measure the size of the tear, because we had to cover the area quickly with TachoComb® sheets to achieve hemostasis. The LV apex was dyskinetic. A total of three TachoComb® sheets (5 × 5 cm each) were applied to the bleeding point and the surrounding area of fragile necrotic tissue. The major source of bleeding was controlled, but a small amount of blood continued to flow out the lower part of the sheet (Figure \n). Four 3–0 polypropylene (SH) horizontal mattress sutures were then used to secure a pair of Teflon felt strips over the TachoComb® sheets. The sutures were placed approximately 1 cm from the perforated myocardial region. Extreme care was taken to avoid LAD and its diagonal branch. After complete hemostasis was achieved, an additional TachoComb® sheet and fibrin glue were applied (Figure \n). The entire LV repair was performed without CPB. The patient was transferred to the intensive care unit with dramatically improved hemodynamics. The postoperative course was uneventful, and she walked out of the hospital on day 35. The patient was followed up until 3 months, when she died because of cerebral bleeding. |
A previously fit and well 15-year-old female presented via the emergency department in 2011 with a very short history of dyspnoea and biphasic stridor following unsuccessful treatment for presumed asthma. FNE revealed subglottic stenosis with mobile vocal cords and an urgent CT scan confirmed concentric soft tissue narrowing of the subglottis to 4 mm. She subsequently underwent emergency tracheostomy and laser MLB&D with symptomatic relief. Histology from the initial procedure had demonstrated chronic inflammation without underlying granulomatous disease, and an autoimmune screen at the time was negative.\nDespite oral prednisolone, repeated MLB&D every 6 to 8 weeks provided only transient relief from restenosis, and a decision was made to perform a laryngotracheal reconstruction with posterior rib graft augmentation. Airway diameter augmentation was achieved using autologous rib graft posteriorly and a sternohyoid flap with split skin graft. This significantly improved her airway and she was discharged on prednisolone with the tracheostomy in situ. A postoperative baseline CT was performed which demonstrated extension of disease to involve the right hemilarynx, perilaryngeal fat and arytenoid cartilage. Histology from surgical specimens demonstrated inflammation with marked plasmacytosis and polyclonality on immunostaining, although immunocytochemistry for IgG4 was within normal limits (IgG:IgG4 less than 25%, 27 IgG4+ cells/high power field). Serological IgG and IgM levels were noted to be raised although IgG4 subclass have been within normal limits. With a presumptive diagnosis of IgG4-RD, she was managed with azathioprine and courses of rituximab by co-author AS.\nSurveillance in ENT clinic has demonstrated stable appearances in her airway reconstruction, with a partial return of right vocal cord mobility, and she was decannulated the following year. Over the last 5 years peristomal tracheal scarring has warranted laser resection and the insertion of serial tracheal stents with adjuvant sirolimus therapy. Histologically, this recurrent stenosis has been demonstrated to be granulation and scar tissue unrelated to the previous IgG4-RD. She remains well with a good voice, receiving intermittent stent changes, and is presently studying medicine. |
A 32-year-old male patient reported to the Department of Prosthodontics with a complaint of discolored teeth, generalized sensitivity, chipping off of teeth, and difficulty in chewing ().\nNo abnormality was detected in temporomandibular joint movements. Intraoral examination revealed unaesthetic appearance, generalized attrition, decreased vertical dimension, dehiscence in relation to 13, sharp incisal edges and anterior edge to edge relation. Discrepancy between centric relation and maximum intercuspation was also found. Radiological examination revealed generalized loss of enamel (). Based on the above features, the patient was diagnosed with AI hypoplastic type.\nRoot canal treatment was advised in relation to 11, 13, 14, 21, 22, 23, 24, 25, 33, 36, 41 and 42. Pankey Mann Schyuler philosophy of full mouth rehabilitation was planned for this patient.,\nDiagnostic casts were obtained. Following this a facebow transfer was completed. The centric relation position was recorded using aluwax and the casts were mounted on a semi adjustable articulator. The vertical dimension was increased by 3 mm. A heat cure clear acrylic occlusal splint of 3 mm was fabricated, which was to be used by the patient (). The patient was examined on the first week and every 2 weeks for 4 months, the adaptation of the temporomandibular joints and muscles was carefully observed during this time.\nDiagnostic wax up was completed with an increased vertical dimension (). The wax up helped in assessing the outcome of the final prosthesis and it also helped in fabricating the temporary restorations. The maxillary and mandibular anterior teeth were prepared and restored with provisional restorations. The provisional restorations helped in assessing the esthetics and establishing the customized anterior guidance (). Mandibular anteriors were restored followed by maxillary. The splint was modified by cutting the anterior portion, so that it could be used by the patient.\nThe maxillary and mandibular anterior final restorations were fabricated and luted in the patient’s mouth (). The next step was the restoration of the mandibular posterior teeth for which the occlusal plane was established using a Broadrick’s occlusal plane analyzer., Diagnostic wax pattern was fabricated for mandibular posteriors and verified using the Broderick’s occlusal plane analyzer (). After the patterns were fabricated a silicon putty index was made, which acted as a guide in the ceramic build up. Once fabricated the restorations were luted ()\nThe luting of the mandibular posteriors was followed by the fabrication of the maxillary posteriors for which the functionally generated path (FGP) technique was used. After the preparation of the maxillary posterior teeth a master impression was made to obtain a maxillary master cast. A facebow transfer was done following which a clear acrylic base was fabricated which extended bilaterally on to the prepared teeth providing cross arch stabilization. The base was kept very thin on the occlusal surface, so that it would not contact the opposing teeth in either centric relation position or any lateral excursive movements. A softened functional wax (inlay wax) was added onto the acrylic base for recording the FGP, and the patient was asked to perform centric and eccentric movements (). The FGP should be checked for all movements during excursions to make sure all pathways have been recorded in sufficient functional wax. After confirmation of the movements the wax is chilled with ice water to make it firm, and a smooth creamy mix of fast setting stone is jiggled into the depressions of the functional wax. The fast setting stone must cover at least one unprepared tooth (restored canine) in front that served as a definite vertical stop and a positive key to the master dies used in the laboratory (). The mandibular cast was removed from the articulator, and the FGP base with the stone core was placed against the maxillary cast and mounted. The functional core is a 3 dimensional record of the border pathways and so the articulator serves merely as a devise to maintain the relationship of the dies to the recorded paths of movement of the lower teeth. The denture base with the functional wax was removed; the wax patterns were fabricated and casted. After the metal trial was verified the ceramic build up was completed with the help of the stone core. The stone core acted as a guide in removing the centric and eccentric interferences. The bisque trial was done in the patient’s mouth. After correcting the interferences the restorations were glazed and luted. A group function type of occlusal scheme was provided to the patient () and regular follow-up with good oral hygiene maintenance was advised (). |
A 69-year-old Caucasian male was referred to our hospital with 3 weeks of abdominal distension and worsening right lower quadrant pain. He was diagnosed with IgG kappa multiple myeloma four years prior to presentation. He was initially treated with bortezomib/dexamethasone with monthly zolendronic acid with good response initially; however, a year after diagnosis, he was found to have disease progression which manifested as a right radius fracture. His regimen was switched to lenalidomide with dexamethasone with good response and clinically depressed levels of paraproteins. After completion of 9 months of therapy, he underwent autologous stem cell transplant with high-dose melphalan. 7 months after bone marrow transplant, his disease progressed with involvement of pericardial fluid. Salvage therapy was initiated with pomalidomide, bortezomib, and dexamethasone which was discontinued a year later due to peripheral neuropathy; however, at the end of treatment, there was no evidence of ongoing disease.\nWhen the patient presented to our hospital, he had an acute abdomen. Initial blood work revealed a normocytic anemia with hemoglobin of 8.4 g/dl and elevated ESR of 44. He also had acute kidney injury with creatinine of 3 mg/dl (baseline of 1.9 mg/dl). CT scan of the abdomen and pelvis revealed extensive stranding seen throughout the abdomen within the peritoneal space with edema in the mesentery ().\nHe underwent an exploratory laparotomy which revealed induration of the entire base of the mesentery and retroperitoneum. He had an IgG level of 4407 units with predominantly kappa light chains whose level was 4833 units (kappa to lambda ratio 540). Pathology revealed extensive mesenteric infiltration by kappa restricted plasma cells positive for CD138 on immunohistochemistry, without evidence of amyloidosis. Bone marrow biopsy revealed a 30% involvement by plasma cells (Figures –). Cytogenetics showed 1q22 duplication, trisomy 7 and 15, and gain of 8q24.1. The skeletal survey revealed lytic lesions in the left femur and skull (Figures and ).\nHe was started on carfilzomib and dexamethasone therapy for relapsed multiple myeloma. Unfortunately, he died within one day of start of the chemotherapy from surgical complications of bowel obstruction. |
A 32-month-old Middle Eastern boy was born full term at a community hospital in Michigan with birth weight of 3135 g (15.0 percentile). He had normal prenatal ultrasounds. He passed meconium at birth and had no other complications including prolong neonatal jaundice or dehydration. His CF NBS showed serum IRT 139 ng/ml and was negative for the 40 gene mutations panel. At 1 month of age, he developed a wet cough without any other symptoms. He was followed by his primary care provider (PCP), and no treatment was given at the time. His symptoms continued on and off until 1 year of age. At 1 year, the mother noticed increased frequency of productive cough, lack of appetite, and poor weight gain. His weight-for-age percentile ranged from 0.3 to 5.0. His stools were reportedly normal. He had no excessive sweating. He was referred to an outside asthma/allergy specialist for evaluation of asthma. He was prescribed budesonide without any improvement. He had frequent pharyngitis and otitis media that were treated with oral antibiotics that reportedly helped treat acute infection, but the cough persisted. He was also prescribed a H2 blocker for possible gastroesophageal reflux disease, but no improvement in symptoms was noted. Family history was negative for CF.\nAt 30 months of age, he was seen by his PCP for one week of cough and fever. He was treated with amoxicillin. His symptoms continued to worsen despite oral antibiotics, and he had two episodes of small-volume hemoptysis. He was subsequently admitted for community-acquired pneumonia and influenza B. Chest X-ray showed diffuse ill-defined opacities in the perihilar area and diffuse bronchiectasis. During the hospitalization, pediatric pulmonary consult was obtained. Given the negative NBS, it was stated that CF was unlikely and no sweat chloride test was recommended. He had a normal videofluoroscopic swallow study. Immunodeficiency workup revealed elevated immunoglobulin levels, protective vaccine titers, and normal lymphocyte counts and response to phytohaemagglutinin, concanavalin A, and pokeweed mitogen. HIV test was negative. Pediatric gastroenterology was consulted for failure to thrive and recommended to continue high-calorie diet. He was discharged home on augmentin.\nTen days following discharge, he was seen at the immunology clinic. He was noted to have digital clubbing, worsening tachypnea, and crackles. With the concerning physical exam findings, a sweat chloride test was done with a result of 90 mmol/L (normal 0–29 mmol/L; intermediate 30–59 mmol/L; abnormal ≥60 mmol/L) []. He was referred to pediatric pulmonary clinic the same day. He was then admitted and treated for a CF exacerbation. Throat culture grew Pseudomonas aeruginosa and methicillin-sensitive Staphylococcus aureus (MSSA). Fecal elastase-1 was <50 mcg E/g stool (normal >200 mcg E/g stool). Lab results including comprehensive metabolic panel and vitamin A and E levels were normal. He completed two weeks of cefepime and tobramycin.\nAfter notifying MDHHS with the false-negative NBS results, the blood spot that was available at the NBS lab was retested using the new and expanded mutation panel (60 mutations). He was found to be homozygous for R1066C (c.3196C > T; p.Arg1066Cys) mutation. His care was transferred to our CF center, as per parents' request. Two weeks later, he was admitted for worsening respiratory symptoms and treated for a CF exacerbation. Vitamin D level was low at 25 ng/ml (normal ≥30 ng/ml). High-resolution computed tomography of the chest showed diffuse bilateral bronchiectasis (). Flexible bronchoscopy showed airway erythema and significant thick green secretions () that was positive for MSSA. |
The second patient is a 23-year-old female who was on the first floor of a four-storey building when it collapsed. She was trapped for five hours by a large beam; she sustained crush injuries to her pelvis and lower limbs. On arrival at hospital, a crush injury syndrome was diagnosed with resultant severe metabolic acidosis and hyperkalaemia. She was unable to move her lower limbs and reported no sensation below the knees to light touch, pain, or temperature. A CT scan of the pelvis showed bilateral fractures of the superior and inferior pubic rami with distraction of the pubic symphysis. There were no spinal injuries and no lower limb fractures.\nDue to the high demand on the local intensive care service, the patient was transferred to another hospital. She remained in intensive care for two weeks and required dialysis for her crush injury syndrome.\nThere was profound weakness of both legs for a week after the injury; this then slowly improved, although persistent patchy numbness and a right foot drop remained. The patient developed neuropathic pain in the right foot soon after the injury that was described as burning and stinging in the right sole. The pain was constant and severe (reaching 9/10 to 10/10 on a verbal rating scale). It was accompanied by allodynia; even light touch felt exquisitely painful. She could not tolerate physiotherapy; she required a 50% nitrous oxide/50% oxygen combination inhalation for splint application. The pain spread to involve the left foot as well.\nThe neuropathic pain and allodynia slowly reduced in intensity; the severe flareups, however, persisted. Autonomic features developed; these included a dusky appearance of the right foot accompanied by mild swelling, decreased sweating, and abnormal skin texture. Electromyography and nerve conduction studies demonstrated a severe lesion of the right sciatic nerve.\nThe patient's analgesic regime consisted of paracetamol, gabapentin, amitriptyline, tramadol, transdermal clonidine, and a fentanyl PCA. The patient was later converted to a fentanyl patch with oral immediate release oxycodone as needed. Ketamine was trialed early but caused unpleasant hallucinations and was stopped. An intravenous low-dose lignocaine infusion proved ineffective.\nIn hospital, the patient experienced low mood as well as some features of PTSD. On discharge, symptoms such as flashbacks and distressing dreams occurred when exposed to cues that triggered recall of events. She did not report significant anxiety and continued to adjust well. She resumed part-time work eight months after the injury. The neuropathic pain has persisted, particularly in the right foot, but flareups of pain have reduced in frequency and intensity. |
We illustrate a case of a 21-year-old, previously healthy, right-handed female who presented after a sudden onset of headache and vomiting. Her Glasgow Coma Score was 15, and there were no focal deficits on neurological examination. Computed tomography of the head (CTH) demonstrated intraventricular hemorrhage that appeared to arise from the anterior horn of the right lateral ventricle (Figure ). Her intracerebral hemorrhage score was 1 for intraventricular hemorrhage []. Computed tomography angiography and magnetic resonance imaging (Figures -) demonstrated a large medial right frontal lobe AVM. The lesion was in the region of the supplementary motor area (SMA) and cingulate gyrus.\nFurther imaging with DSA demonstrated that the right frontal AVM nidus measured 36.8 × 25.4 mm and was supplied predominantly by the right pericallosal and callosomarginal arteries (Figures , ). The right pericallosal artery and one of its larger branches were running en passage. Furthermore, the distal right callosomarginal artery appeared to empty directly into the AVM without evidence of en passage vessels. There was evidence of an intranidal aneurysm (Figure ), but this was not the source of the hemorrhage. The AVM also received a small arterial contribution from a distal right posterior cerebral artery branch (Figures , ). There was a large nidal draining vein that extended superiorly along the right side of the cerebral falx draining into the superior sagittal sinus (Figure ). The lesion was classified as S-M grade II AVM (2 points for size).\nThe patient remained stable preoperatively, and after discussing multiple therapeutic approaches, she opted for surgical resection. She underwent a right frontal craniotomy with an interhemispheric approach for surgical resection. After resection, which was thought to be a surgical cure, she remained under general anesthesia and underwent an immediate cerebral DSA. The DSA revealed residual nidus with two sources of deep venous drainage: one short draining vein entered the inferior sagittal sinus and another entered the right basal vein of Rosenthal (Figures , ). Thus, the AVM was reclassified as S-M grade III. We believed we could safely resect the residual nidus; therefore, we returned to the operating room for additional resection. On DSA after additional resection, there was no evidence of residual arteriovenous shunting (Figures , ).\nPostoperative CTH demonstrated expected postoperative changes that were stable on repeat imaging (Figure ). On postoperative examination, she demonstrated antigravity resistance in her left upper and lower extremities with weakness most pronounced in her left upper extremity. Given the lesion’s involvement of the right SMA, this was thought to be SMA syndrome. The weakness continued to improve throughout her postoperative stay. After drainage of the remainder of the intraventricular hemorrhage, she was weaned from the external ventricular drain. She was discharged home on postoperative day 12 with outpatient physical, occupational, and speech therapies for rehabilitation. At her one-month follow-up appointment, she was neurologically intact with full resolution of her right-sided SMA syndrome. CTH at this time demonstrated expected postoperative changes and interval resolution of intracranial blood products, without evidence of hydrocephalus. |
We report a case about a 65-year-old male patient with pseudoarthrosis of the first MTPJ, who suffered from a nonunion 12 months after a fusion made of two crossing screws (). Postoperative x-rays showed a correct position of the two crossing screws and a correct position of the dorsiflexion (). The x-ray revealed a nonfusion during follow ups; we recommended the possibility of an extracorporeal shockwave therapy but the patient refused. The blood samples which we had taken before the revision surgery didn’t show any infection signs; additionally, there was no fever or any clinical sign of infection. The patient is a high demand patient, who does sport (hiking and cycling up to 20 km) and gardening quite often. Twelve months after the fusion he still had severe pain and was not satisfied at all with the result of the surgery. At this time the x-ray showed a persistent nonunion. We discussed the pros (definitive solution, a greater load-bearing capacity for our active high demand patient) and cons (a long follow-up treatment) of a revision fusion. On the other hand we informed him about the stability, function, the faster healing and mobilization of the prosthesis. Our patient refused a revision because of the previous failed MTPJ fusion; for this reason we decided to use this new device.\nThe two screws and the connective tissue were removed. We took care that enough resection of the bone and a capsular release of the metatarsal head was made in order to achieve a dorsiflexion of more than 90 degrees intraoperative. Afterwards a hemicup Biopro prosthesis (Biopro, Farmingdale, NY, USA) 18.5 mm was implanted. Intraoperative the final check up showed a dorsiflexion of 80 degrees and a good stability of the joint.\nImmediately, the patient received postoperative-physiotherapy treatments. During hospitalization he learned how to do exercises for mobilization of the great toe, which the patient continued at home. The patient was allowed to bear weight as tolerated with comfortable wide shoes. The suture was removed 10 days post-operatively. After 6 weeks the hemicup prosthesis was radiologically controlled. It showed a correct fitting of the prosthesis. In the clinical examination an active range of motion of 40/0/30 degrees was achieved by the patient.\nSeventeen months postoperative the patient was evaluated using the American Orthopaedic Foot and Ankle Society (AOFAS) hallux metatarso-interphalangeal scale and a visual pain rating scale from 1 to 10, where 0 s indicates the absence of pain and 10 indicates the worst pain imaginable. The patient rated the pain with 2 and reached 95/100 points of the AOFAS score at the last follow-up. Preoperatively, the AOFAS score was 52 points with a pain of 8 points. The range of motion was scored –5 points because of a range of motion of the first metatarso-phalangeal joint of 60 degrees (40/0/20 degrees). Our patient was very satisfied with the postoperative result of the procedure and would undergo the surgery again (). |
An 88-year-old man was referred to our hospital for emergent massive hemoptysis. His medical history was remarkable for chronic heart failure, moderate mitral regurgitation, atrial fibrillation, and chronic kidney disease. He was undergoing treatment with apixaban and pilsicainide for atrial fibrillation. He was severely hypoxic (SpO2: 80 under O2 15 L/min by oxygen mask) and hypotensive (systolic blood pressure: 80 mmHg) on admission. In the emergency department, we performed intubation into the right main bronchus through guided bronchoscopy; this was followed immediately by right side one-lung ventilation as portable chest radiography showed consolidation in the left upper lung. Bronchoscopy showed that the trachea was almost obstructed by haemorrhage and haematoma. He experienced cardiopulmonary arrest immediately after the airway was maintained. However, spontaneous circulation was restored by cardiopulmonary resuscitation. Contrast computed tomography (CT) demonstrated an aortic aneurysm at the aortic arch, which penetrated the upper lobe of the left lung (Fig. A,B). We suspected that it would be difficult to perform emergent surgery because of the patient’s poor general condition. Furthermore, we believed that there was no indication for endovascular stenting due to the following reasons: (1) the root of the left brachiocephalic artery was close to the penetrating portion of the aneurysm, at a distance of 12 mm. Thus, there might have been a high risk of obstructing blood flow to the brachiocephalic artery; (2) a risk of aortic injury might have been induced by stenting because the aortic arch was highly calcified. He was admitted to the intensive care unit, and we controlled blood pressure using nicardipine and discontinued anticoagulation therapy and performed platelet and fresh frozen plasma (FFP) transfusion for haemostasis. Bleeding from the APF decreased gradually due to astriction by haematoma. On the 17th hospital day, we performed bronchoscopy for the suction of haematoma, except in the bronchi of the left upper lobe, and adjusted the intubation tube for conversion to bilateral lung ventilation. On the 18th hospital day, we performed endobronchial occlusion with EWS to prevent fatal hemoptysis despite the risk of atelectasis. We inserted the EWS into each target bronchus with haematoma, with consideration of the risk of re-bleeding due to the removal of the haematoma. EWS sizes were as follows: (B1 + 2a: 7 mm; B1 + 2b: 7 mm; B3b + c: 7 mm; and B3: 7 mm) (Fig. A). B1 + 2c did not undergo EWS insertion because this bronchus was not responsible for bleeding on CT findings. There was no massive hemoptysis after bronchial occlusion with the EWS; subsequent mild hemosputum was controlled by a haemostatic drug. The patient was successfully extubated on the 22nd hospital day and was discharged on the 47th hospital day without complications and free from oxygen. Radiography showed that EWS had promptly fixed each bronchus (Fig. B). |
A 74-year-old male with past medical history of atrial fibrillation, cardioembolic stroke, parkinson disease, chronic obstructive pulmonary disease (COPD), and recent cellulitis presented to the emergency department with a chief complaint of fever and cough. The patient had a full workup, including routine labs and chest x-ray, which were nonrevealing. The patient was discharged home under the suspicion that this was an exacerbation of his COPD. The patient went home on oral antibiotics. The patient returned to the emergency room (ER) within 24 hours with worsening symptoms, including headache, altered mental status, fever, and cough. The patient is originally from the Netherlands and presented to our hospital seven days after arriving to the United States. The patient was admitted to the hospital for further workup. All protective measures and precautions for suspected COVID-19 infection were taken. The patient was placed in isolation. Repeat chest x-ray demonstrated small right pleural effusion with bilateral ground glass opacities (see Figure ). CT chest revealed patchy bibasilar consolidations and subpleural opacities. Both throat sputum and nasopharyngeal cultures were negative for strep. Blood cultures were negative, and urine analysis was negative. Influenza A and B tests were negative.\nDue to the severe alteration in mental status, neurology was consulted. Upon examination, the patient was found encephalopathic, nonverbal, and unable to follow any commands; however, he was able to move all his extremities and was reacting to noxious stimuli. No nuchal rigidity was noted. A CT scan of the head and EEG were ordered immediately. The CT scan of the head showed no acute abnormalities. There was the presence of an area of encephalomalacia in the left temporal region, consistent with the prior history of embolic stroke (see Figure ). The EEG showed bilateral slowing and focal slowing in the left temporal region with sharply countered waves (see Figure ).\nThe patient was started on antiepileptic medication prophylactically given the possibility of subclinical seizures in this patient with an area of encephalomalacia and epileptiform discharges in the right temporal region. The patient was treated empirically with vancomycin, meropenem, and acyclovir. A lumbar puncture did not reveal any evidence of central nervous system infection (see Table ). Due to his progression in symptomatology, he was then tested for COVID-19 and found to be positive. The patient developed respiratory failure and required intubation and was transferred to the ICU. Based on anecdotal experience from other medical centers, the patient was started on hydroxychloroquine and lopinavir/ritonavir, and was continued on broad-spectrum antibiotics. The patient currently remains in the ICU, critically ill with poor prognosis. |
A 75-year-old male patient was admitted to Zhejiang Provincial People’s Hospital on 23 November 2018 after two-week-history of post-exercise dyspnea and chest pain. Physical examination revealed decreased breath sound, increased tactile fremitus on the affected side, and dullness to percussion of the left lung. Chest computed tomography (CT) showed diffuse thickening and calcification of the pleura on both sides, pleural effusion and atelectasis on the left side ( and ). After hospitalization, the patient received a thoracentesis on November 26, 2018. The pleural fluid was bloody exudate, and no tumor cells were found in the exfoliated cells of the pleural fluid sample. According to the imaging findings, we tracked the patient’s occupational history and learned that the patient had a history of close contact with asbestos for 30 years. The diagnosis of pleural mesothelioma needs to be ruled out clinically, and it is planned to be transferred to surgery for open pleural biopsy. At the same time, laboratory examination revealed elevated plasma D-dimer (26190Ug/L), combined with the patient’s chest pain, dyspnea symptoms, and a large amount of pleural effusion, the possibility of pulmonary embolism should be considered. Then, the computed tomography pulmonary angiography (CTPA) was performed for the patient, and the results showed that there was embolism in the right pulmonary artery branch (). After the diagnosis of pulmonary embolism, we immediately used anticoagulant drugs for the patient. Because anticoagulants are easy to cause massive hemorrhage, no open pleural biopsy was performed. After anticoagulation therapy, the patient’s symptoms of pleural effusion and dyspnea were improved and the patient discharged on December 18, 2018.\nHowever, after being discharged from the hospital, the patient was still admitted to the hospital several times due to chest pain and dyspnea, and the reexamined chest CT still showed a large amount of pleural effusion on the left side ( and ). When he was hospitalized again on July 8, 2019, there was no obvious abnormality in plasma D-dimer and CTPA, which ruled out the possibility of recurrent pulmonary embolism. We also considered the possibility of pneumonia and tuberculous pleurisy, but repeated pleural effusion and sputum culture did not show any positive results and there was no evidence for tuberculosis. Because of the long-term bloody pleural effusion, the possibility of lung cancer is also considered. The patient’s pleural fluid was sent for pathological examination for many times, but no tumor cells were found. PET-CT examination showed diffuse thickening of the bilateral pleura with calcification, mainly on the left side. FDG metabolism was increased, and malignant tumors were considered (). We consider that this patient may have malignant mesothelioma. Later, the patient underwent an ultrasound-guided pleural biopsy, and the pathological examination revealed fibroblast-like spindle cells arranged in bundles or chaotic shapes ( and ). The results of immunohistochemistry were: CK(Pan)(+), WT1(+), GATA3(+), P63(-), TTF-1(-), EMA(-), CD117(-), DOG1(-), Calretinin(-), CK5/6(-), CD34(-), ERG(-), SMA(-), Desmin(-), S100(-), MiTF(-), HMB45(Melanoma)(-), MyoD1(-), Caldesmon(-), D2-40(-), CEA(-), Vimentin(-) (). Combined with the above examination results, the final diagnosis was sarcomatoid pleural mesothelioma. However, two days later, the patient suddenly developed a coma, facial cyanosis, and sudden cardiac arrest. And the blood pressure, oxygen saturation cannot be measured at that time. We consider that the cause of cardiac arrest may be an acute myocardial infarction or acute pulmonary embolism. After tracheal intubation, electric defibrillation and chest compression, the patient discharged voluntarily. The patient died after being discharged from the hospital 2 days later. |
A previously healthy 29-year-old African-American female presented for evaluation of bilateral progressive breast enlargement, persistent palpable nodules in bilateral breasts, left breast tenderness, left upper extremity pain, and back pain for approximately a year. Previous treatment with antibiotics failed to alleviate symptoms. Physical examination revealed bilateral macromastia, asymmetry, with the left breast being larger than the right, and multiple palpable mammary nodules. Bilateral diagnostic mammograms performed at an outside hospital showed diffuse skin thickening and edema throughout the left breast parenchyma. Left breast sonographic study the same day showed retroareolar ductal prominence with multiple cysts. A skin biopsy was performed by a dermatologist, the result of which was noncontributory. A breast magnetic resonance imaging (MRI) was done at the same outside institution which demonstrated innumerable homogeneously enhancing masses throughout both breasts, greater on the left, with the largest one located on the left measuring up to 3.0 × 2.0 cm. No axillary or internal mammary lymphadenopathy was noted. Differential diagnoses based on imaging studies and clinical presentation included inflammatory breast cancer, severe mastitis, severe fibrocystic changes, and, less likely, phyllodes tumor. The patient reported no family history of breast or ovarian cancer. Two excisional biopsies of the left breast were performed at an outside hospital and were diagnosed as fibroadenoma and lymphangiectasia with associated mild lymphoplasmacytic infiltrate, respectively. The patient was subsequently referred to our institution for further evaluation. The slides of the previous excisional breast biopsies and skin biopsies were obtained and reviewed and were diagnosed as PASH. Sono-guided biopsies of the palpable nodules in the right breast were performed, which revealed fibrosis, duct ectasia, and apocrine metaplasia with no evidence of malignancy. Repeat MRI confirmed the previous findings and demonstrated worsening asymmetry and macromastia (). Antihormonal therapy with tamoxifen was recommended; however, the patient declined this treatment option. Due to disease progression, decision was made to perform bilateral mastectomy with immediate reconstruction.\nLeft total mastectomy specimen weighed 1770 grams and measured 25.0 × 20.0 × 7.1 cm. Serial sectioning revealed diffuse nodularity, marked edema, and multiple cysts filled with clear fluid (). The nodules were rubbery, gray/white in color, and relatively well-circumscribed, with the size of these nodules ranging from 9.0 × 2.7 × 2.0 cm to 0.5 × 0.5 × 0.3 cm. Right total mastectomy specimen weighed 1001 grams and measured 23.0 × 16.8 × 5.2 cm. Serial sectioning of the right breast revealed similar findings. Microscopic examination of the bilateral mammary tissue showed characteristic changes of PASH including stromal hyperplasia and bland spindle cell-lined slit-like open spaces (). Additionally, marked stromal edema, dilated lymphatic channels, and mild lymphoplasmacytic infiltrate were also present. Immunohistochemical staining showed that the stromal cells were positive for estrogen receptor (ER) and progesterone receptor (PR) and negative for CD31, supporting the diagnosis of PASH. |
A 62-year-old female patient was recently referred to our hospital with a 3-months history of an episodic, but progressively worsening stridor, exertional dyspnoea and dry cough. Clinical examination of the right side of the neck revealed a hard 3 cm × 5 cm solitary thyroid nodule with ipsilateral level III and IV cervical lymphadenopathy. No neck tenderness or dilated veins could be identified. Serum thyroid function tests were within normal limits (TSH 1.8 Miu/L, Free T3 4 pmol/L, Free T4 16.3 pmol/L), but thyroglobulin level was >115 IU/ml. Subsequent neck ultrasonography (US) confirmed a solitary thyroid nodule showing cystic changes and calcification with cervical lymphadenopathy. The ipsilateral IJV was also noted to be distended but the ultrasonographer was not able to comment on any intravascular masses. Fine-needle aspiration cytology of the thyroid nodule revealed a papillary thyroid carcinoma, and papillary thyroid cells in the enlarged lymph nodes. A fibre-optic laryngeal examination revealed a narrow glottis area with a postnasal drip, the vocal cords appeared normal, but with diminished mobility. Computed tomography (CT) imaging showed a large hypo dense nodule with areas of cystic degeneration and calcification occupying the right lobe of the thyroid gland and invading the left lobe (a) and a large, well defined, heterogeneously enhancing mass in the right anterior jugulo-digastric region representing a pathological lymph node partially compressing the right IJV (b). Surgery was considered for treatment and a total thyroidectomy with right neck dissection revealed a locally advanced thyroid neoplasm with superficial invasion of the trachea. Intraoperative palpation of the distended right IJV showed a 2 cm × 3 cm hard intraluminal mass. Longitudinal venotomy revealed a tumour thrombus with no endothelial adhesion and was easily removed (). The vein was repaired using 6-0 prolene and the tracheal invasion was shaved successfully.\nHistopathology and immunohistochemistry analysis of the right thyroid lobe mass and the tissue shavings of the trachea revealed stage (PT4bN1M) undifferentiated anaplastic carcinoma (a and b respectively). Histology of the right IJV intraluminal mass revealed a papillary thyroid carcinoma (c).\nThe surgery was followed by ablative radioiodine plus external beam radiotherapy to the neck. The patient is alive and functional 16 months later with no signs of local recurrence or distant metastasis. |
A 44-year-old woman with an unremarkable medical history visited our outpatient clinic because of the SCD of her younger brother and her younger sister. One day, at the age of 31 years, her previously healthy brother was unexpectedly found dead in bed, and post-mortem evaluation did not reveal a cause of death. Several years later, her sister was found dead in bed at the same age as her deceased brother, and also, she had previously been considered healthy. Notably, this sister consulted a cardiologist because of palpitations 1 month before her death. On cardiac examination, she had one non-sustained ventricular tachycardia on 24-h Holter monitoring, but her ECG, echocardiography and exercise test were normal. Her ECG is shown in Fig. . The family history further revealed that an uncle died unexpectedly at the age of 48 years late in the evening while he was out to inspect his cattle, and a great grandfather also died suddenly at a young age. Because of this impressive family history, the 44-year-old woman underwent a cardiac examination, which was unremarkable. Her ECG is shown in Fig. . Because no abnormalities were found, it was discussed that prophylactic treatment was not readily indicated, but her family members were asked to visit a cardiologist in a search for an inheritable arrhythmia syndrome. A few months later, while further cardiac examinations of her two remaining brothers were carried out, she suffered from VF while drinking coffee in the morning. Fortunately, she was successfully resuscitated. Subsequently, an implantable cardioverter-defibrillator (ICD) was implanted, which to date has not discharged. However, only a few months after her resuscitation, her nephew (her deceased brother’s son) was also resuscitated from VF at the age of 16 years while climbing stairs. After an unremarkable complete cardiac examination, he too had an ICD implanted, and he has received appropriate discharges on several VF recurrences in the past years. |
An 11-year-old girl reported to the Department of Pedodontics and Preventive Dentistry, Dr. D.Y. Patil Dental College and Hospital, Pune, with the chief complaint of discolored teeth since childhood.\nThis little girl's parents did not seek any treatment previously, thinking that since the condition was not resulting in any other systemic manifestations and since she had inherited the condition from her father, there was little they could really do about it and accepted it as part of her appearance. It was only now, when they realized that the girl avoided hard food substances, they got her to a dentist.\nOn enquiry, it was revealed that her deciduous teeth were also similarly discolored, and her father, younger brother of eight years, and younger sister of six years, also suffered from the same condition. The pedigree chart could be constructed as in .\nApart from this, her past medical history was noncontributory. The history did not reveal any eruption disturbances. From a functional point of view, she had been avoiding hard food substances; at the same time, remaining caries free, except for a sole proximal carious lesion in 36, involving the enamel and dentin.\nOn intraoral examination, it was found that she had a normal complement of teeth. The thickness of enamel was reduced on the teeth and was completely chipped off from some teeth exposing the dentin. The surfaces of the teeth were rough. The teeth, in general, exhibited a yellowish brown discoloration, with diffuse pitting present on the exposed tooth surfaces, more prominent on the labial and buccal aspects. The emergence pattern and timing of teeth seemed to be within the normal range. No open bite was present. Examination of the periodontium revealed the presence of chronic, generalized, marginal, and papillary gingivitis, with calculus deposition and unsatisfactory oral hygiene [Figures –].\nA provisional diagnosis of hypoplastic, rough autosomal dominant AI was proposed along with a differential diagnosis of environmental enamel hypoplasia, dentinogenesis imperfecta, dentin dysplasia, regional odontodysplasia, and the tricho-dento-osseous syndrome.\nRadiographic investigations included an orthopantomogram (OPG) and full mouth intraoral periapical (IOPA) radiographs. The OPG showed the presence of all unerupted third molars, as well as a seemingly normal pattern and timing of eruption of teeth []. Examination of the IOPA radiographs revealed a normal pulp chamber and root canal spaces with no signs of obliteration. The enamel was almost half its expected thickness, but was more radiodense than the dentin.\nThe ground section of an over-retained deciduous molar extracted from the girl showed that the enamel was thin and was composed of laminations of irregularly arranged enamel prisms. No abnormality was apparent in the dentin []. shows a ground section of a normal tooth, where regularly arranged enamel prisms along with the normal thickness of enamel can be appreciated [].\nThe diagnosis of hypoplastic, rough, autosomal dominant AI was confirmed on the basis of typical, clinical, radiographic, and histopathological features.\nEsthetics along with functional limitations were the reason the patient's parents brought her to the hospital for treatment. The treatment proposed for her ranged from bonded veneer restorations to tooth preparations for the placement of a full crown. Restoration of the carious lesion in 36 would be accompanied by a full coverage restoration. The coronal pulp in these children tends to recede faster than in normal teeth and hence crown preparations for full coverage crowns can often be done for relatively young patients.\nAttrition of teeth is a major problem in cases of AI. Therefore, the use of stainless steel crowns as a preventive measure is advocated. Hand scaling was advocated and oral hygiene instructions were given to the patient. Regular brushing of the teeth using the modified Bass technique was taught to the patient to be practiced twice a day (morning and at night before going to bed) using a soft toothbrush and a fluoridated toothpaste (containing 1000 ppm of available fluoride) followed by rinsing with 2% w/v of chlorhexidine gluconate for 15 days. |
A 28-year-old lady presented to us with history of decreased vision in her right eye of one month duration. Patient had episodes of headache associated with her visual complaints. Rest of the patient's complaint history was not contributory. One month prior to her presentation to our clinic, the patient had visited two different ophthalmologists, who diagnosed her as having papilledema. Her vision in the right eye was 20/200 (refractive error of − 1.00 spherical − 1.50 cylinder axis 180) and the left eye vision was 20/40 (−1.25 spherical − 2.00 cylinder axis 165) at the time of presentation. Slit lamp biomicroscopy of the anterior segment was unremarkable in both eyes. She had a relative afferent pupillary defect in her right eye. Dilated pupillary examination in the right eye showed optic disc edema, more obvious in the inferior half of the disc with faint splinter hemorrhage in the parapapilary area. The arterioles over the disc showed attenuation in their caliber. Further refined examination of the right optic disc same disc showed a circumscribed pale yellow lesion buried in the disc suggestive of disc drusen []. The optic disc in the left eye showed an irregular disc margin with minimal elevation and disc vessels showed anomalous branching pattern []. Visual fields in the right eye showed superior altitudinal defect [] and left eye was normal. Ultrasound B scan confirmed the presence of ONHD in both eyes []. A diagnosis of ONHD in both eyes, with anterior ischemic optic neuropathy in the right eye was made. Patient was treated for her headache (tension type) symptomatically. Our patient was seen again after 6 weeks and right eye disc showed pallor suggestive of optic atrophy [] and spectral domain optical coherence tomography also confirmed the presence of ONHD with retinal nerve fiber layer (RNFL) loss []. Visual acuity remained at 20/200 in the right eye. |
A previously healthy 25-year-old Haitian female, suffering only from headaches and back pain since delivery after an uneventful pregnancy 1 month previously, was admitted for sudden visual loss and impaired consciousness. She had no motor or sensory disturbance, but had complete visual loss with fixed, dilated pupils. Her body temperature was 38℃. CT scan and MRI of the head disclosed enlargement of the entire ventricular system and a calcification in the right occipital lobe (). Insertion of external ventricular drainage resulted in dramatic improvement of consciousness and visual recovery. Her ventricular CSF biology was normal (protein, 0.4 g/L; leukocyte count, 12/mm3). Her body temperature rapidly rose to 40℃ and a lumbar puncture was performed. The CSF level of protein was 8.5 g/L, and the leukocyte count was 680/mm3, among which 61% were lymphocytes and 18% were eosinophils. These discrepancies between ventricular and lumbar CSF samples led to MRI of the spine, which disclosed a heterogeneous mass located in the cauda equina from L4 to S2. There was neither leptomeningeal enhancement nor subarachnoid cysts suggestive of arachnoiditis at the thoracic, cervical, or cranial levels (). Since there was no other obvious explanation for the hydrocephalus (no obstructive lesion of the intracerebral CSF pathways nor at the craniocervical junction, and no abnormality in the spinal subarachnoid spaces at superior levels), this lumbosacral mass was considered to be responsible for the hydrocephalus, due to obstruction of the subarachnoid spaces by the mass itself and/or surrounding arachnoiditis. Surgery performed via a lumbosacral laminectomy allowed subtotal removal of an intradural, poorly limited, pearly white mass with small cystic formations surrounding the nerve roots, with thickening of the arachnoid layer. Histological examination revealed parasitic cysts. No scolex was found, but immunological blood and CSF tests for cysticercosis were positive. Although surgery led to disappearance of the symptoms, the patient was provided with adjunctive albendazole. There was no recurrence during a 15-year follow-up, with the patient leading a normal life. |
In case 2, a 24-year-old woman had presented with 65 days of delayed menstruation and a 2-month history of lower abdominal fullness, initially. She had a poor appetite and had lost 2 kg in weight. No abdomen ache, diarrhea, or melena was noted. Her physical exam demonstrated a hard, ill-defined, and tender mass in the upper abdominal region and shifting dullness was positive. CT showed lumps of varying sizes in the pelvic and abdominal cavity. Massive ascites and pelvic fluids were found (). No enlarged lymph nodes could be seen in the whole body. Carcinoembryonic antigen and CA19-9 levels were normal. The CA125 level was slightly increased. The patient was diagnosed with ovarian cancer with abdominal and pelvic cavity metastasis initially and received cytoreductive surgery. Macroscopic surgical exploration revealed an encapsulated nodular mass measuring 15×15×10 cm at the lesser curvature of the stomach, and other tumor nodules were dotted around the ovary, anterior wall of the uterus, mesentery, omentum, and liver capsule. Multiple sections were taken from all tumor nodules. An excisional biopsy was performed to confirm the diagnosis and the pathologic findings were consistent with FDCS. Based on the histopathological and immunohistochemical findings, the patient was diagnosed with FDCS of extensive abdominal and pelvic cavity involvement.\nShe received postoperative sequential chemotherapy. Four cycles of CHOP were initiated in July 2012. After three courses of chemotherapy, a subsequent CT scan was performed, which showed the patient’s disease had improved significantly. Two months later, CT revealed the disease had progressed and the regimen was changed to six cycles consisting of ICE (ifosfamid, carboplatin, and etoposide). This regimen was completed in July 2013. When a subsequent positron emission tomographic scan revealed persistent disease, the patient received enterolysis and cytoreductive surgery. After operation, she elected not to receive adjuvant therapy. Unfortunately, her disease could not be controlled and she died 5 months later. |
The subject described in this case report is a 21-year-old male who suffered a concussion 356 days prior to the physical therapy evaluation. The injury occurred during a fall out of a moving golf cart onto his left shoulder/neck region. The subject reported loss of consciousness and post-traumatic amnesia following the injury. The subject was taken to a local emergency department and CT scan results appeared negative for cerebral bleeding but identified a left temporal bone fracture not requiring medical intervention. The subject received no education on symptomology or symptom duration, and no referral or follow-up was scheduled at the time of discharge. The subject reported a history of four previous concussions, three of which were sport-related, but otherwise had no co-morbidities or significant past medical history. Only minor residual symptoms from his previous concussions were reported that all gradually resolved. The subject had constant, persistent symptoms since the injury that impaired his abilities in school as well as his social life and had restricted athletic or recreational activities, reducing his overall quality of life. The subject visited his primary physician a few weeks prior to evaluation; his cervical spine was cleared via radiographs, and he was referred to a metropolitan outpatient physical therapy clinic.\nThe subject was given the Rivermead Post-Concussion Questionnaire (RPQ) to assess symptomology. The RPQ is a subjective, self-report measure that encompasses 16 items, each of which is scored from 0-4 in increasing severity, assessing separate cognitive, emotional, and somatic physical factors. The RPQ demonstrates high test-retest and inter-rater reliability for both total score and individual items via spearman rank correlation coefficients (r = 0.91, r = 0.87 respectively). The subject’s chief reported symptoms during the initial evaluation were neck pain and stiffness, bilateral radicular symptoms that were worse in the left shoulder and upper extremity, lightheadedness, nausea, dizziness, blurred vision and diplopia, sensitivity to light, impaired balance, slurred speech, trouble sleeping, fatigue, slower thinking, and frustration. The subject’s primary complaint was his headache symptoms, which the subject reported were brought on by reading, scanning, driving, or cervical movements. The subject also reported a period of gradual worsening during the weeks and months following his injury. The subject’s goals for physical therapy were to reduce the severity of symptoms experienced since the injury, primarily concerning his headache, fatigue, and neck pain symptomology, as he reported these symptoms have impacted his abilities as a college student and decreased his participation in his social life.\nThe subject described in this case report provided informed consent for the study and was informed that the data collected would be used for publication. This study has been approved by the Mercer University Institutional Review Board and Office of Research Compliance. The primary author providing patient care and clinical decision making was a student physical therapist at the time of subject interaction and was supervised by a licensed physical therapist. |
An 88-year-old man was referred to our hospital for emergent massive hemoptysis. His medical history was remarkable for chronic heart failure, moderate mitral regurgitation, atrial fibrillation, and chronic kidney disease. He was undergoing treatment with apixaban and pilsicainide for atrial fibrillation. He was severely hypoxic (SpO2: 80 under O2 15 L/min by oxygen mask) and hypotensive (systolic blood pressure: 80 mmHg) on admission. In the emergency department, we performed intubation into the right main bronchus through guided bronchoscopy; this was followed immediately by right side one-lung ventilation as portable chest radiography showed consolidation in the left upper lung. Bronchoscopy showed that the trachea was almost obstructed by haemorrhage and haematoma. He experienced cardiopulmonary arrest immediately after the airway was maintained. However, spontaneous circulation was restored by cardiopulmonary resuscitation. Contrast computed tomography (CT) demonstrated an aortic aneurysm at the aortic arch, which penetrated the upper lobe of the left lung (Fig. A,B). We suspected that it would be difficult to perform emergent surgery because of the patient’s poor general condition. Furthermore, we believed that there was no indication for endovascular stenting due to the following reasons: (1) the root of the left brachiocephalic artery was close to the penetrating portion of the aneurysm, at a distance of 12 mm. Thus, there might have been a high risk of obstructing blood flow to the brachiocephalic artery; (2) a risk of aortic injury might have been induced by stenting because the aortic arch was highly calcified. He was admitted to the intensive care unit, and we controlled blood pressure using nicardipine and discontinued anticoagulation therapy and performed platelet and fresh frozen plasma (FFP) transfusion for haemostasis. Bleeding from the APF decreased gradually due to astriction by haematoma. On the 17th hospital day, we performed bronchoscopy for the suction of haematoma, except in the bronchi of the left upper lobe, and adjusted the intubation tube for conversion to bilateral lung ventilation. On the 18th hospital day, we performed endobronchial occlusion with EWS to prevent fatal hemoptysis despite the risk of atelectasis. We inserted the EWS into each target bronchus with haematoma, with consideration of the risk of re-bleeding due to the removal of the haematoma. EWS sizes were as follows: (B1 + 2a: 7 mm; B1 + 2b: 7 mm; B3b + c: 7 mm; and B3: 7 mm) (Fig. A). B1 + 2c did not undergo EWS insertion because this bronchus was not responsible for bleeding on CT findings. There was no massive hemoptysis after bronchial occlusion with the EWS; subsequent mild hemosputum was controlled by a haemostatic drug. The patient was successfully extubated on the 22nd hospital day and was discharged on the 47th hospital day without complications and free from oxygen. Radiography showed that EWS had promptly fixed each bronchus (Fig. B). |
A 42-year-old woman presented to our emergency department with a painful swelling in the suprapubic region persisted for 3 days. She had a history of LC 10 years back at our center for symptomatic gallstones and had no history of comorbidities. The patient had a history of cesarean section twice. She had a pulse rate of 92/min, blood pressure of 110/60 and a temperature of 37.4°C. Physical examination revealed abdominal tenderness in the suprapubic region, right and left lower quadrant, and exhibited signs of peritoneal irritation, muscle guarding and rebound tenderness. Laboratory tests resulted with white blood cell: 15 200/mm3, hemoglobin: 11.9 g/dl, C-reactive protein: 24 mg/dl, and other biochemical parameters were also normal. On oral contrast-enhanced abdominal computed tomography (CT) performed in the emergency room (), the mesenteric adipose planes were inflamed and contaminated. Minimal free fluid was observed in the periphery of the intestinal loop in the pelvic area. At first, it was not stated on tomographic interpretation that there were gallstones in the abdomen. Considering that the patient had signs and symptoms of acute abdomen, she underwent diagnostic laparoscopy. In exploration, it was observed that the small intestines were edematous, the omentum was inflamed in the pelvic region and the omentum was attached to the anterior abdominal wall, bladder and uterus by gato. Since a clear diagnosis could not be made in the patient for etiology, abdomen was opened with a midline incision under the umbilicus. Infected reactive fluid located between the omentum and the anterior abdominal wall and pelvic region was aspirated. Adhesions due to previous cesarean sections were removed. During the adhesiolysis, stones the largest of which was ~2 cm in size, and abscesses were detected in the omental granuloma/cake (). These stones were thought to remain in the abdomen due to the previous LC. Partial omentectomy with abscesses drainage was performed. The abdomen was irrigated and the stones were retrieved. No other pathology was detected in exploration and no additional surgical intervention was performed. The postoperative period was uneventful, and she was discharged on the fourth postoperative day. Spilled cholesterol gallstones were determined to be the cause of the acute abdomen in this case. Histopathological examination confirmed the diagnosis. Written informed consent was obtained from the patient for the anonymized information to be published in this article. |
A 62-year-old female patient who is heavy smoker presented with a burning sensation and discomfort in her left breast that has been recurring over a month prior to admission to the hospital. No fever, chills, or any other symptoms were described. She reported a past medical history of hypertension and a surgical history of hemorrhoidectomy, dilation and curettage surgery, colonoscopy, and gastroscopy.\nPhysical examination revealed a palpable left breast mass (measuring approximately 3 × 3 cm) in the upper quadrant with no overlying skin changes. The right breast exam was normal. No palpable locoregional lymphadenopathy (axilla and supraclavicular lymph nodes) was noticed. Routine blood tests (complete blood count with differential, electrolytes, prothrombin time, partial prothrombin time, and international normalized ratio), chest X-ray, and electrocardiogram (ECG) were all normal.\nMagnetic resonance imaging (MRI) of the left breast showed an ill-defined deep retroareolar spiculate lesion extending over 3 × 1.5 cm revealing early enhancement peak with associated architectural distortion. There were no axillary lymph nodes or abnormal bone signal intensity. No cutaneous thickening or retraction was seen. Findings were suggestive of BIRADS type IV lesion ().\nAn excisional biopsy was performed and revealed breast tissue with extensive lymphocytic infiltrate intermixed with neoplastic epithelial cells (). Immunohistochemistry results were positive for CK AE1/E3 antibody in the neoplastic epithelial cells with no expression of estrogen or progesterone receptors, and HER2/neu was not overexpressed (). The lymphocytes in the background stained positive for both CD3 and CD20 (Figures and ).\nThe patient underwent a left modified radical mastectomy. Eleven lymph nodes were dissected and free of tumor. The mastectomy specimen showed a 3.5 × 3 × 3 cm cavity at the site of the previous excisional biopsy. On histological examination, apocrine metaplasia was identified but no residual tumor was detected. To note, apocrine metaplasia is a very common incidental benign finding that is considered part of or associated with fibrocystic changes, and hence, does not affect prognosis and management []. Accordingly, no adjuvant hormonal therapy, chemotherapy, or radiotherapy was given to the patient.\nNo evidence of recurrence was noted on a 2-year follow-up. |
A 36-year-old Caucasian woman was evaluated with chief complaint of gluteal pain radiating to her leg. Her medical history was remarkable with gunshot injury to the affected leg with multiple pellets dispersed into her pelvis and proximal part of the thigh, as shown in Figs. and . She had gunshot injury 20 years ago. She was previously diagnosed as having lumbar disc herniation at L4–5 level. She underwent a previous discectomy outside our institution 2 years ago. A radiological examination revealed the presence of recurrent disc herniation, as well as multiple shotgun bullets in her pelvis and thigh. One of those bullets was deep into the sciatic nerve inside her quadratus femoris muscle.\nElectromyography (EMG) showed the presence of chronic sciatic nerve injury. Since it was clinically impossible to distinguish lumbar disc herniation from the sciatic injury, we decided to proceed with removal of the foreign object and neurolysis of the sciatic nerve followed by L4–5 discectomy and fusion. We decided to perform those procedures in two different settings. The first surgery included access to the sciatic nerve in the upper portion of her thigh and exposing the nerve fibrotic bands around the nerve. The dissection proceeded deep into the nerve within a muscle, where a bullet was found and removed. The distance from the bullets to the nerve was approximately 2 cm. Muscle tissue around the bullets was excised for analysis. For comparison, another specimen was obtained from the gluteal muscle, superficially away from the nerve and all the bullets. Two weeks later, she underwent scheduled L4–5 discectomy and fusion. Her postoperative course was uneventful. On follow-up examination at 6 months, she was essentially symptom free.\nA scanning acoustic microscope (AMS-50SI) developed by Honda Electronics (Toyohashi, Japan), whose schematic setup is shown in Fig. , was used in AI mode. It has a transducer with quartz lens, a pulser/receiver, an oscilloscope, a computer, and a display monitor. An 80 MHz transducer is installed within the microscope, which generates the signals and collects the reflected acoustic waves. Water is the coupling medium between the quartz lens and the substrate. For two-dimensional scans, an X-Y stage, controlled by a computer, is used. An oscilloscope analyzes the reflected signals from both the reference and target material after being collected by the transducer. As a result, acoustic intensity and impedance maps of the region of interest with 300 × 300 sampling points are obtained.\nThe principle of SAM in AI mode is demonstrated in Fig. . Distilled water is widely used as reference. The signal reflected from the target is\nwhere, S0 is the generated signal by the 80 MHz transducer, Ztarget is tissue’s AI and Zsub is the polystyrene substrate’s AI (2.37 MRayl). The tissue’s AI is calculated by combining the reflected signals from the tissue and the reference. The signal reflected from the reference iswhere Zref is the AI of water (1.50 MRayl). Then, the target’s AI is written aswith a constant signal S0 [] generated by the transducer.\nElectron microscopy-based imaging and chemical analysis studies were performed in a JEOL JIB-4601 focused ion beam scanning electron microscope (FIB-SEM) multi-beam platform coupled with an Oxford X-MaxN EDS system, as shown in Fig. .\nBlood samples were collected in test tubes containing ethylenediamine-tetraacetic acid (EDTA) and no anticoagulant on the day of the first surgery (foreign object removal) prior to the procedure. Then, 2 ml of 20% trichloroacetic acid (TCA) was supplemented into the blood samples to release the red blood cells (RBC) and other ingredients. The supernatant part was received from blood with TCA by centrifugation at 4000 revolutions per minute (rpm) for 20 minutes for the analysis of Pb and cadmium (Cd) within total blood. Coagulation of blood samples enabled serum trace element analysis: chromium (Cr), Fe, Cu, magnesium (Mg), manganese (Mn), selenium (Se), and Zn. The serum specimen was prepared using Hettich Universal centrifuge by centrifugation at 3000 rpm for 15 minutes, separating from cells immediately after and storing at − 20 °C until the analysis [].\nAfter weighing the left sciatic nerve tissue samples, they were digested with 2 ml of 65% nitric acid (HNO3) at 180 °C in the incubator for 1 hour. Then, 2 ml of 65% perchloric acid (HClO4) was added into the cooled mixture. Then, the mixture was digested at 200 °C in the incubator until the volume was halved. Digested materials were vortexed and diluted in water to a total volume of 10 ml. Concentrations were given in micrograms per gram (μg/g) wet tissue weight [].\nAll glassware were maintained at 10% (volume/volume; v/v) HNO3 before use, cleaned with deionized water, and dried in an incubator at 100 °C overnight. Pb, Cd, Cu, Cr, Fe, Mn, Se, and Zn elements were detected by inductively coupled plasma optical emission spectrophotometer (ICP-OES 6000, Thermo, Cambridge, United Kingdom). Measurements for each element were done three times and averaged. The ICP-OES was operated with argon carrier flow rate of 0.5 L/minute, plasma gas flow rate of 15 L/minute, sample flow and elusion rate of 1.51 L/minute, and peristaltic pump speed of 100 rpm, selecting the suitable wavelength for Pb, Cd, Cr, Cu, Fe, Mn, Se, and Zn, which were 220.353 nm, 228.802 nm, 267.716 nm, 324.75 nm, 285.213 nm, 357.610 nm, 196.090 nm, and 206.200 nm, respectively. Transport lines were obtained using 1.25 mm internal diameter polytetrafluoroethylene tubing. Element levels were indicated in micrograms per deciliter for serum (μg/dl) and μg/g for wet tissue. The standard concentrations for standard graph calibration were arranged from standard stock solutions of 1000 μg/ml for each analyzed element [].\nThe tissue samples were investigated by using AI mode of SAM. Figure shows the AI map of the tissue obtained away from the gunshot. The map was constructed by collecting the reflections of acoustic signals, generated by the transducer within SAM, from surfaces of the reference (water) and the tissue sample on the polystyrene substrate. At specific locations within the sample, the AI was calculated to be higher than 2 MRayl, indicating accumulation of elements with different elastic properties. Figure shows the AI map of the tissue obtained close to the gunshot. As can be seen in this image, almost everywhere had an AI of greater than 2 MRayl.\nSEM images of the tissue far away from the gunshot were obtained at magnifications of 5000 × and 500 ×, as shown in Figs. and , respectively. Similarly, SEM images for the tissue close to the gunshot were obtained at magnifications of 5000 × and 500 ×, as shown in Figs. and , respectively. The images were acquired at 5 keV energy for both tissue samples.\nThe SEM images show that the tissue far away from the gunshot keeps its original structure, whereas the tissue close to the gunshot seems to be deformed and torn up. These results demonstrate the degree of damage the impact of gunshot causes on soft biological tissues.\nTable represents the EDS measurements in SEM, carried out for determining the elemental distribution differences in the deformed tissues. The measurements show the percentages of the residue elements detected on tissues far away from the gunshot and close to the gunshot. According to the results, among all residue elements, Pb, Cr, Fe, and Mn are found to be higher in weight content in the region close to the gunshot, when compared to distant region. Cd and Cu levels do not differ much; however, Zn level is lower in the tissue close to the gunshot.\nWe determined Pb, Cr, Cd, Cu, Fe, Mn, Zn, and Se levels in both tissue samples. Pb, Cr, Fe, Se, and Mn levels were higher in the tissue close to the gunshot, conversely, Zn level was lower in this sample (Table ). Blood Pb and blood Cd, and serum Cr, Cu, Fe, Mn, Se, and Zn levels of our patient were also analyzed and the results are shown in Table , however, we did not observe significant differences when compared to reference values. |
A 64-year-old male presented with a two month history of difficulty urinating and was found to have a fungating penile mass involving 50% of his penis. The mass was hard and fixed and extended from the glans proximally up the shaft. He also had bilateral palpable inguinal lymphadenopathy. There were no associated constitutional symptoms. Given there was a high suspicion for malignancy, the patient underwent partial penectomy within a month of presentation. Biopsy results confirmed a pT2 tumor with invasive keratinizing squamous cell carcinoma, poorly differentiated, and tumor size of 5 × 4 × 2.5 cm, with corpus spongiosum and lymphovascular involvements.\nFollowing the procedure, the patient had PET-CT for staging, and imaging revealed enlarged hypermetabolic bilateral axillary lymph nodes concerning for metastatic disease. In addition, there was a large centrally necrotic lymph node conglomerate in his left groin that had increased FDG avidity. The patient had left inguinal and bilateral pelvic lymph node dissections revealing metastatic squamous cell carcinoma in multiple lymph nodes. The left inguinal mass was also found to be metastatic well-differentiated SCC. His diagnosis was staged at T2N3M0.\nAfter his surgical procedures, patient was started on adjuvant chemotherapy. He began first line chemotherapy with paclitaxel, ifosfamide, and cisplatin (TIP). He underwent 4 cycles of TIP but eventually developed disease progression on repeat imaging. At this point, the patient was started on cetuximab given EGFR amplification on tumor analysis with the FoundationOne testing platform. However, the patient had an allergic reaction to cetuximab, so his treatment was changed to panitumumab. The patient had stable disease and a progression-free survival of 6 months with anti-EGFR treatment, which is clinically significant given that this treatment was given in the second-line setting for an aggressive tumor type that other than chemotherapy there is no other approved drug to date.\nThe patient was ultimately started on the PD-1 inhibitor nivolumab. He had initial response to immunotherapy followed by stable disease, so he had a disease control rate of an additional 6 months with this investigational agent at that time. Ultimately, he was placed on hospice and passed away two years from the the time of diagnosis. |
A 17-year-old man was involved in a road accident in which he suffered the open fractures of the right femur and tibia. At the arrival to the Emergency Dept (ED), he was alert and hemodynamically stable and the Glasgow Coma Scale (GCS) was 15; the initial alignment of the fractured ends was performed in the ED with a gentle traction performed under sedation with iv. ketamine; a total body CT did not demonstrate other injuries. Approximately two hours after the admission the patient was taken to the surgical theatre for the external fixation of the fractured bones; at entering the operating room, the GCS was 8, the arterial pressure was 115/80 mm Hg, the heart rate was 115 bpm, and the arterial oxygen saturation (SPO2) was 85 at room air; the procedure was performed under general iv anesthesia with propofol and remifentanyl; the standard monitoring included the ECG, the noninvasive arterial pressure, the SPO2, and the end-tidal CO2 (ETCO2); during the intervention, the SPO2 rose to 100% at a FIO2=40% and all the other variables remained stable throughout the procedure after the 3-hour-long intervention in which the complete alignment of the bony ends was achieved; the patient was transferred to the Intensive Care Unit (ICU) still intubated and mechanically ventilated; the iv anaesthetics were gradually tapered until the complete suspension. Two hours later, the SpaO2 and the ETCO2 slightly decreased and anisocoria was observed; and an urgent CT scan of the head demonstrated a diffuse cerebral edema and the herniation of the cerebellar tonsils (Figures and , respectively). At this time, the pupils became bilaterally mydriatic and the EEG was almost isoelectric; due to the severity of the conditions, a MR scan was considered unnecessary. On the basis of the clinical and radiologic findings repeated boluses of iv. mannitol and steroids were given in the following hours aiming to reduce the intracranial pressure. An echocardiogram demonstrated a severe right ventricular depression with an ejection fraction of 20%. On the following day, the patient was declared brain dead according to the current Italian law.\nAt the autopsy, the cerebral microvascular network appeared diffusely plugged with BME (Figures –) and ischemia-related microcalcifications were scattered throughout the brain (); other organs were less extensively involved; no PFO was demonstrated. |
M.A. is a 20 months old male infant who presented with a pulsatile swelling in the anterior abdominal wall. He is the only child of a 29-year old housewife and a 34-year old man who is a primary school teacher in a non consanguineous marriage. They drink alcohol occasionally but do not smoke.\nM.A. was born at term at a clinic in the outskirts of Yaounde with a birth weight of 3,000 grams. The health personnel who took care of the newborn noted the presence of a pulsatile mass on the anterior surface of the abdomen which was covered with a hyperpigmented skin. The mass was thought to be an umbilical hernia requiring no active intervention.\nA family friend advised the parents to consult our services. On physical examination, his anthropometric measurements were normal for his age (weight 12.5kg, height 85 cm, head circumference 48 cm). We noted a pear-shaped midline abdominal mass extending from the epigastric region to the umbilical region. It measured 20 cm long and 12 cm in diameter (). The mass was completely covered by hyperpigmented skin.\nIn the epigastric hollow, there was an oblong mass measuring 5 x 7 cm, extending into the thorax, firm to palpation, pulsating at the rhythm of the patient’s radial pulse at a rate of 105 beats per minute. The rest of the mass had a soft consistency. On auscultation, bowel sounds were heard. The heart sounds were heard over the precordium and they were normal. Examination of the lungs revealed no abnormalities. There were no dysmorphic features or other abnormalities noted.\nA chest X-ray has shown a slightly enlarged heart. An abdominal ultrasound revealed no intraabdominal organ abnormality. There was a herniation into the abdominal cavity through the diaphragm, containing a pulsatile mass representing a portion of the left ventricle.\nEchocardiography showed an alteration in the cardiac morphology. The left ventricle appeared thin and elongated, measuring 41.1 mm x 15.2 mm compared to the right ventricle , 44.6 mm x 24.3 mm. No other structural abnormality of the heart was noted and cardiac function was satisfactory. The child was transferred for surgery to a European hospital. |
The second case refers to a 48-year-old woman, a busy manager with a history of depression and sleep disturbance. She has had three terminations of pregnancy and one delivery by cesarean section. She smokes approximately ten cigarettes per day and has high cholesterol serum levels. She takes several medications: a selective serotonin reuptake inhibitor (escitalopram), two benzodiazepines (delorazepam and clonazepam), and a statin. She reports a four-year history of urinary symptoms: daily UUI episodes, mild stress urinary incontinence (SUI), and two episodes of nocturia per night. She wears pads every day. The urology consultation revealed some degree of pelvic pain, especially during vaginal examination. The urine dipstick was negative and there was no PVR. No specific causes of the symptoms such as urine tract infection were identified. The patient also complained of mild dyspareunia and occasional constipation. The urine culture turned out to be sterile, with no blood in urine, and the pelvic ultrasound scan and urine cytology were also negative. The cystoscopy, which was performed as a result of the presence of storage symptoms and to rule out a bladder tumor in this current smoker, was normal.\nIn OAB patients, it is of utmost importance to consider all comorbidities. Anxiety and depression may play a role, feeding a vicious circle. Moreover, medications to treat neurological or psychiatric disorders can influence OAB and be responsible for side effects [, ]. Gastrointestinal disorders are frequently associated with OAB, such as constipation in this case, but patients rarely raise the topic. An overlap exists between irritable bowel syndrome and OAB [].\nThe patient was prescribed a β3 agonist, pelvic floor muscle training (PFMT) and bladder retraining. Four months later, she noticed some degree of improvement, but had stopped the treatment as she felt that she had no time for PFMT. She was not compliant with the bladder drill either, and soon stopped the β3 agonist because she did not sense any real improvement. She also felt that she did not have the time to complete a bladder diary. She was prescribed fesoterodine 8 mg for three months. In parallel, her general practitioner asked for vaginal and urethral culture swabs, which were negative. After three months, her urinary urgency improved, but she said that the few remaining episodes of urgency were “killing her life” and that she did not want to be on pills for her whole life. Therefore, she refused to continue the treatment and requested an “easy fix”. Her reaction highlights the need for careful consideration of the consequences of incontinence in terms of QoL. A publication from Vaughan et al. [] reported that OAB and incontinence synergize to reduce QoL, especially in the domains of sleep, elimination, usual activities, discomfort, distress, vitality, and sexual activity.\nConsistent efficacy on urgency symptoms with a significant decrease in UUI and urgency episodes has been reported with fesoterodine at doses of 4 and 8 mg compared to placebo () [, , , ]; however, some patients may react differently. Patient satisfaction is an important driver of treatment success []. Patient expectations should be considered carefully in the context of OAB management. The achievement of patients' goals was measured in the Study Assessing FlexIble-dose fesoterodiNe in Adults (SAFINA study) [], a 12-week multicenter open label study with 331 OAB adults, using the Self-Assessment Goal Achievement (SAGA) questionnaire. Fesoterodine treatment resulted in 81.3% of patients declaring that their goals were “somewhat achieved/achieved” or that the result “exceeded/greatly exceeded their expectation”.\nOur case patient had very specific expectations; she refused to have an implant (neuromodulation), saying “I'm not going to be an android!” She accepted botox injections, and so a first set of injections was performed under local anesthesia. She found the injections “a little painful” and “a big annoyance”, but at the one-month follow-up visit after botox injection she reported no more UUI episodes and an improvement in frequency and the number of urgency episodes, as well as in QoL. Even though she stated that she did not like the idea of being a patient for the rest of her life, she accepted subsequent injections.\nThe clinical points that can be learned from this case are as follows:All OAB cases are different, and a thorough evaluation is mandatory to adequately address each case. It is important to assess other aspects, such as functional and psychological disorders that may influence symptoms, and to consider nonneurogenic OAB as a multifactorial disease. The major goal of initial therapy is to meet the patient's expectations regarding the reason for their visit, to improve their satisfaction, and their QoL. Due to fesoterodine's characteristics and flexible dosage, improvement of symptoms and achievement of the patients' goal are usually high with this medication. When patients have specific requirements, all options should be discussed and the patient's agreement obtained. A customized approach is a crucial factor for treatment success. OAB management should be personalized; beware of a simplistic application of a standardized treatment algorithm. |
A 26-year-old female sustained an injury in the neck after fall from the bullock cart in her native village. She was treated with local quacks for some time but experienced stiffness of her neck for which she presented to us after 4 months. When we examined, we found neck was stiff, motor and the sensory test revealed the American spinal injury association (ASIA) E grade neurology without radiculopathy or myelopathy signs. A cervical spine X-ray showed bilateral facet dislocation with almost toppling of C4 over the C5 vertebrae []. Computed tomography of her neck revealed bilateral perched facets with early callous between C4 and 5 body and no other associated fractures. Magnetic resonance imaging of her cervical spine revealed the absence of disc compression anteriorly through the spinal cord was displaced posteriorly. In view of normal neurology and absence of myelopathic/root compression signs, a thorough discussion was done regarding the mode of treatment. The patient was fully explained the risks involving surgery, including a postoperative deficit. After the patient consented for surgery, she was planned for the posterior first approach. No preoperative traction was applied. She was positioned prone with Gardner-Wells tongs in place and weights of 4 kg. Posterior exposure revealed a visible cord between the separated lamina of C4-5. Partial laminectomy of superior of C5 and inferior lamina of C4 was done along with facetectomy. Manual extension maneuver was tried to reduce the dislocation under vision, which failed. Lateral mass screws were placed in C4-5 and compression was done, which reduced the dislocation up to 50% []. The posterior wound was closed, and the patient flipped supine. An anterior approach was taken, and pins applied in C4-5 that appeared as a cross []. Now, the pins distracter was placed, which made the pins parallel []. Next, the discectomy was done [] and a cage was placed with bone graft in situ harvested locally from the inferior lip of the C4 body []. Finally, a plate was applied to reinforce the construct and wound closed. Postoperatively the patient was neuro-intact after extubation and shifted toward after overnight stay in intensive care. The postoperative check X-ray was satisfactory [] and the patient was discharged subsequently with the Philadelphia collar. She is doing well. |
A 76-year-old woman with no prior significant medical or surgical problems was referred to the University of California San Diego Medical Center for evaluation of a 5-cm right solid renal mass diagnosed on computed tomography urogram (CTU) in the evaluation of new-onset gross painless hematuria. She denied any history of tobacco smoking or illicit drug misuse. The results of a clinical examination were unremarkable for any abdominal or flank masses, organomegaly, or lymphadenopathy. Hematological and biochemical laboratory values were within the normal range (blood urea nitrogen, 8 mg/dL; serum creatinine, 0.73 mg/dL; and glomerular filtration rate, >60).\nPreoperative CTU demonstrated a heterogeneous parenchymal right renal mass suspicious for RCC, along with right ureteral and renal pelvis filling defects (). Rigid urethrocystoscopy and a right retrograde pyelogram were performed and did not demonstrate any mucosal lesions or any filling defects in the bladder, ureter, or renal pelvis. Cytology of urine obtained during cystoscopy revealed epithelial cell clusters with atypia without overt features of malignancy. Some reactive urothelial cells were noted in the background. Further imaging showed no evidence of abdominal or retroperitoneal lymphadenopathy, organomegaly, or distant metastasis.\nGiven the clinical presentation and imaging findings, robotic-assisted laparoscopic right nephrectomy was offered. The risks, benefits, and potential complications were discussed thoroughly with the patient. Subsequently, robotic-assisted laparoscopic right nephrectomy was successfully performed with no complications. The patient had an uncomplicated postoperative recovery course. However, on postoperative day 4, she developed a low-grade fever and productive cough consistent with an upper respiratory tract infection (URTI) secondary to testing influenza A positive. This URTI completely resolved with an oral course of azithromycin. The patient was discharged home on postoperative day 6 in a comfortable and stable condition.\nPathologically, the right kidney specimen showed unclassified RCC with extensive extracellular but intraluminal mucin production with a maximum diameter of 5 cm and with renal sinus fat involvement consistent with pT3a with negative margins of resection. Grossly, the right kidney measured 12.5 cm×10 cm×5.5 cm and contained an upper pole solid mass that when bisected had a firm whitish yellow and glistening cut surface. The mass had ill-defined borders and was present in the superior pole and encroached upon the mid aspect of the kidney. The mass measured 5 cm×4.2 cm×4 cm. It involved mainly the cortex of the kidney and appeared to extend to the superior calices, the medullary pyramids, and the renal sinus fat. The renal pelvis appeared gray-white, glistening, and uninvolved by the tumor. One of the main branches of the main renal vein appeared to contain tumor; however, the renal vein margin was grossly clear of tumor. A smooth-lined unilocular cyst measuring 4 cm×3.5 cm×3.5 cm was found in the inferior to mid aspects of the kidney, which abutted the lateral aspect of the renal capsule and the perinephric adipose tissue. The cyst contained clear serous fluid and the lining was smooth without papillary excrescences. The wall thickness measured up to 0.1 cm. The remaining uninvolved parenchyma appeared tan-brown and smooth with a distinct corticomedullary junction, and the inferior calices appeared gray-white and glistening.\nHistologically, the tumor displayed variable architectural patterns including areas of compact alveoli of clear cells consistent with clear cell carcinoma (). However, islands of cribriform sheets of clear cells with glandular lumens predominated. Many of the gland lumens contained blue mucin that was positive on mucicarmine and periodic acid-Schiff diastase stains (, ). Other areas consisted of small clusters and acini of clear cells embedded in fibrous stroma. Additional microcystic areas lined by a single layer of cuboidal clear cells were noted.\nAfter 12 months of clinic follow-up, the patient remained clinically well and her abdominal wounds were well healed. Her renal function was preserved as indicated by blood urea nitrogen of 10 mg/dL and serum creatinine of 0.84 mg/dL. Follow-up computed tomography of the thorax, abdomen, and pelvis did not reveal any evidence of disease recurrence. |
A 23-year-old, gravida 0 para 0 woman presented with focal left-sided convulsions, talipes equinus position of both lower limbs, and gait disturbance at 11 weeks of gestation. She was diagnosed with limb dystonia and referred to our hospital. She had varices and a port-wine stain (capillary malformation) on the left leg (), and at the age of 15, she had undergone tibial epiphyseal line suppression surgery because of right foot hypertrophy. In the first trimester of this pregnancy, ultrasonography including Doppler flow measurements of the uterus and the pelvic vessels did not show any abnormality.\nAt 27 weeks of gestation, ultrasonography demonstrated tubular echolucent spaces throughout the myometrium (), and color Doppler showed blood flow within some of the cystic lesions (). T2-weighted magnetic resonance imaging (MRI) showed a greatly enlarged uterus and diffuse myometrial thickening with a visible junctional zone between the endometrium and large high-intensity myometrium at 30 weeks of gestation, (Figures and ). The border with the surrounding peritoneum was distinct, and there was no suggestion of extrauterine abnormalities. Because we thought that it had a considerable advantage to evaluate the blood flow of the lesion for accurate perinatal risk assessment, dynamic contrast-enhanced- (DCE-) MRI using gadolinium with diethylenetriaminepentaacetate (Gd-DTPA) was performed at 37 weeks of gestation, just before cesarean section, to assess vascularity of the myometrial lesion. DCE-MRI revealed that the myometrial lesion was enhanced slowly and showed homogeneous enhancement even on a 10 min delayed image, whereas the placenta demonstrated marked arterial enhancement and subsequent rapid washout (). These findings indicated the abundant slow bloodstream in the lesion, leading to a consideration of diffuse venous malformation. Taken together with unilateral foot hypertrophy, varices, and port-wine stain capillary malformation, the patient was diagnosed as having KTS complicated with diffuse venous malformation of the pregnant uterus. The hemoglobin, hematocrit, platelet count, fibrinogen, D-dimer, and FDP levels just before the delivery were 10.5 g/dL, 29.7%, 12.3 × 104/μL, 194 mg/dL, 14.2 μg/mL, and 30 μg/mL, respectively.\nAt 37 weeks of gestation, the patient underwent elective cesarean section under general anesthesia because of severe dystonia. There were no abnormal vessels in the central zone of the uterine corpus, but in the lateral aspects of the uterus, many dilated vessels were present. The lower uterine segment was thickened to approximately 5 cm, and heavy venous blood flow was observed at the incision. A biopsy sample was taken from the myometrial surgical margin and the incision of the lower uterine segment was closed. A normal female infant weighing 2,198 g was delivered with Apgar scores of 4 and 7. Total operating time was 70 minutes and estimated blood loss was 3,792 g. During the procedure, the uterus contracted and the fundus was located at the navel level. The patient received a blood transfusion with 6 units of red cell concentrate. Enoxaparin sodium was administered for 6 days after the surgery to prevent thrombosis. The patient was discharged on day 8 postpartum without any considerable complication. Multiple cystic lesions in the myometrium were not observed by ultrasonography on day 7 after the cesarean section.\nHistological examination of the myometrial biopsy showed numerous, variably sized, and thin-walled vessels distributed throughout the myometrium. The endothelial lining on the vessel wall was confirmed by strong immunoreactivity for CD31 and CD34, and lack of elastic fiber layer was observed by immunostaining with Elastica van Gieson (). The histological diagnosis was venous malformation. |
A 59-year-old gentleman with end-stage kidney disease due to hepatitis C with focal segmental glomerulosclerosis, on hemodialysis for seven years, underwent deceased donor renal transplantation. The donor kidney had a KDPI of 60%. There was a 4 antigen HLA mismatch with Class II panel reactive assay (PRA) of 62.31%. Class I PRA was 0%. His donor specific antibody testing was positive for an antibody to DQ7. The donor had died as a result of anoxic brain injury following cardiopulmonary arrest resulting from anaphylactic shock. The cold ischemic time was 12 hours, 7 minutes and the warm ischemic time was 51 minutes. Basilixumab was used for immunosuppression induction and the patient underwent early steroid withdrawal. His maintenance immunosuppression was tacrolimus (targeting trough levels 5-8) and mycophenolate sodium 720 mg BID.\nThe patient's postoperative course was complicated by delayed graft function, necessitating continuation of renal replacement therapy. He was hemodynamically stable throughout his hospital course. A renal allograft ultrasound showed good blood flow to the entire kidney with a resistive index of 0.64. A repeat flow crossmatch was negative, but he remained positive for a persistent low-level donor specific antibody to DQ7 (1000 MFI). He underwent a renal transplant biopsy on his 4th day postoperatively. This showed extensive acute tubular necrosis with associated peritubular capillaritis and interstitial nephritis (). Oxalate crystals were seen in several tubules. One large caliber artery showed active endothelialitis, but no tubulitis or glomerulitis seen. C4d staining was negative in the peritubular capillaries. Electron microscopy revealed minimal effacement of podocyte foot processes. The patient received methylprednisolone 500 mg x 3 doses to treat moderate acute cellular rejection. His tacrolimus dose was also optimized as his trough levels had been running low at between 3.5 and 6. He continued to take mycophenolate sodium at a dose of 720 mg BID. A decision was made not to treat for antibody-mediated rejection given that no glomerulitis was seen and that there was minimal capillaritis with a negative C4d stain.\nHe remained dialysis dependent with minimal urine output for three weeks after transplantation. A urine protein : creatinine ratio was elevated to 1070 mg/g when assessed after hospital discharge when his urine output started to gradually improve. His urine microalbumin : creatinine ratio was 450 mg/g. A decision was ultimately made to readmit the patient on day 12 after transplantation for IV thymoglobulin 1.5 mg/kg to treat his Banff Type IIa T cell mediated rejection, given his lack of response to pulsed IV steroid treatment. Given lack of improvement in renal function and urine output by day 16 after transplantation, a second renal allograft biopsy was performed. This revealed residual acute tubular necrosis with associated mild peritubular capillaritis and interstitial nephritis (). Extensive oxalate crystals were now visible in many tubules. His plasma oxalate level was concurrently elevated to 19.3 μmol/L (reference range ≤ 1.9 μmol/L). No signs of persistent acute antibody- or cell-mediated rejection were seen. Again, there was minimal segmental effacement of the podocyte foot processes seen on electron microscopy and no evidence of recurrent focal segmental glomerulosclerosis present.\nHe commenced calcium citrate along with dietary oxalate restriction to manage his hyperoxaluria and his serum creatinine improved to a nadir of 1 mg/dl (88 μmol/L), with a concomitant gradual reduction in his proteinuria and microalbuminuria to undetectable levels. Of note, the patient had no history of malabsorptive intestinal disease and denied any GI symptoms throughout this time period. He had never suffered from renal calculi. At three weeks after transplantation, his urine output and creatinine clearance had recovered sufficiently to enable him to become dialysis independent. A decision was made to continue low-dose oral steroids in the long-term given the presence of early acute cellular rejection on his first allograft biopsy. An interval renal biopsy performed 2 months later showed no ongoing evidence of oxalate deposition, tubular necrosis or cellular rejection. A concurrent repeat plasma oxalate level showed interval reduction to the normal range. |
In the 1998 report on the treatment of B, he was 20 years old. B had been a child with a classical gender dysphoric development and fulfilled the current criteria for GnRH analog treatment eligibility. He was interviewed shortly after his legal gender change. This meant that he had had puberty suppression, cross-sex hormone treatment, a mastectomy, ovariectomy, and uterus extirpation. From his assessment during the diagnostic phase, he appeared to be an intelligent person (IQ = 128) with excellent problem-solving capabilities with respect to emotional matters. Vulnerable aspects of his psychological functioning were his insecurity about himself and moderately depressed feelings. In all other areas, his scores on a broad range of measures were in the average range, as compared to Dutch normative samples. Shame concerning his transsexualism made him highly selective in his friendships and guarded in his contacts with new people. At the assessment, 1 year after his ovariectomy but prior to his metaidoioplasty, he no longer reported feelings of gender dysphoria. He had adjusted easily to the male role and expressed no doubts on the adequacy of his masculine behavior. After the development of his secondary male sex characteristics, nobody had ever approached him as a woman. He never felt any regrets about his decision and had never contemplated living as a girl again. Knowing what gender reassignment implied, he would do it all over again. B was happy with his life and did not feel lonely. He showed a significant pre-post treatment decrease in his feelings of inadequacy. At the time, he was attending medical school.\nB attended the pediatric endocrinology clinic for the first time when he was age 13.7 years. His medical history was uneventful. Puberty had started 1–2 years previously, but menarche had not occurred. On physical examination, a healthy girl was seen with a height of 157.3 cm and a Tanner pubertal stage of B3, P3. Bone age according to Tanner and Whitehouse was 13.5 years. Target height, as a female, was estimated at 171 cm, based on the parental height. Puberty suppression was induced by the GnRH analog Triptorelin, in a dose of 3.75 mg every 4 weeks intramuscularly. On this dosage, B steadily kept on growing.\nAt age 18.6 years, his height was 165.8 cm. Induction of male puberty and accompanying secondary male sex characteristics was initiated by administration of 100 mg testosterone-ester mixture intramuscularly every 2 weeks. This was, after 6 months, increased to 250 mg testosterone-ester mixture, every 2–3 weeks intramuscularly. At the start of the androgen treatment, the Triptorelin treatment was discontinued.\nWhen B was 20 years old, he had a subcutaneous mastectomy by way of an infra-areolar approach, a few months later followed by a hysterectomy and gonadectomy. Two years later, metaidoioplasty and testes implantation were performed.\nFifteen years after the first interview and testing session, at age 35, B was seen again at the clinic. He lived on his own and he worked as a family doctor in private practice with two colleagues. All family members knew about his transitioning as did some of his friends. He was very satisfied about his circle of friends and had never been treated negatively because of his transsexualism.\nB was still satisfied about his (minor) breast surgery, his ovariectomy, and his hysterectomy, but no longer about the metaidoioplasty he underwent one year after the first follow-up session. He did not like its size and shape and he could hardly urinate in a standing position. He was able to have orgasms, but he could not have sexual intercourse. Because of his desire to have more convincingly male appearing external genitals and his wish to be able urinate in a standing position, he considered having a phalloplasty. Despite his good looks and very masculine appearance, he had not had many steady girlfriends, which may have resulted from the guardedness he already had as an adolescent. At age 29, he had a serious relationship with a woman, which lasted for 5 years. However, he chose not to live together when the opportunity to do so arose. After his choice to continue living apart, his girlfriend ended the relationship, a few months before his interview at the clinic. This made him very much regret his lack of commitment. B considered it likely that his need to distance himself from her had been related to his shame about his genital appearance and his feelings of inadequacy in sexual matters. Additional factors, such as serious illness of his father and a suicide among his sisters’ in-laws, made him rather sad at the time of the interview. Although his psychoneuroticism score on the Symptom Checklist-90 (Arrindell & Ettema, ) was in the normal range, his depression subscore was high, indicating depressed feelings. However, on the Beck Depression Inventory, he scored in the “minimal range” (van der Does, ), indicating that he did not fulfill criteria for clinical depression. On the Adult Self-Report, assessing adaptive functioning and problems in adults aged 18-59 years (Achenbach & Rescorla, ), all his scores were in the normal range.\nIt seems, therefore, that B functioned well in most aspects of life, but that he was still struggling with the question, how to handle the dissatisfaction and shame about his genital appearance.\nAt age 35, on physical examination, we saw a healthy and well virilized person. Blood pressure was 120/85 mmHg, final height was 169.5 cm and weight was 73 kg, which resulted in a body mass index (BMI) of 25.4 kg/m2. Sitting height was 88 cm. Skull circumference was 55.5 cm. For a comparison of his anthropometrical measurements to the normal reference values of Dutch males and females, see Table .\nA fasting venous blood sample was drawn. General health markers, like hematological parameters (Hb, ht, erythrocytes), renal function, and liver enzymes, were all within the normal reference value ranges. His lipid profile showed a total cholesterol level of 228.3 mg/dl (<251.5 mg/dl), LDL-cholesterol 154.8 mg/dl (<193.5 mg/dl), HDL-cholesterol 55.0 mg/dl (>34.8 mg/dl), and triglycerides 97.4 mg/dl (<177 mg/dl). His glucose value was 97.3 mg/dl (<144.1 mmol/l) with an insulin level of 21 pmol/l (12–96 pmol/l for a fasting sample). His HbA1c was 5.4% (4.3–6.1%). These values were in the normal range.\nWith respect to the gonadal axis, a normal-low serum testosterone level of 236.6 ng/dl (231–866 ng/dl) was found. This relatively low level was probably due to the fact that the blood sample was taken just before the next testosterone-ester mixture injection. Gonadotropins were elevated as a consequence of the gonadectomy. LH and FSH were 16 U/l and 50 U/l, respectively. Additionally determined endocrine parameters, such as thyroid and adrenal function, were all within normal reference ranges.\nWith respect to bone mineral density (BMD), a dual energy X-ray absorptiometry (DEXA) scan was performed to determine B’s BMD in the lumbar spine, the non-dominant hip, and (further) total body. The observed values were related to the bone peak mass for white females and for white males, resulting in T-scores. For all values, the z-score was determined as well (see Table ). |
A 49-year-old man with a history of acromegaly was admitted to our hospital with the concern of recurrent shortness of breath and dyspnea on exertion during the previous 2 years, and he had experienced an episode of presyncope 2 weeks prior without any further evaluation. He was a chef in a local restaurant for almost 30 years. He had no family history of any diseases and no past history of hypertension, diabetes mellitus, sleep apnea, or sudden cardiac death. He did not smoke or consume alcohol. The patient provided a history of stereotactic radiosurgeries twice in a decade or so and adherence to treatment with a somatostatin analog (octreotide given 40 mg once per month through intramuscular injection) at the time of diagnosis 20 years before. The patient was overweight and moderately nourished. He was 1.85 m (73 inches) tall, weighed 134 kg, and had a body mass index of 39 kg/m2. His blood pressure was 110/60 mmHg, and his heart rate was 92 beats/min with sinus rhythm. He had distinct skeletal features that included prominent superciliary arches and nose bridge, enlargement of the tongue and lip, and large hands and feet. Cardiac auscultation revealed irregular premature beats and pathological third heart sound, and a systolic murmur was discovered over the apex and aortic area. Bilateral extensive borders of cardiac dullness were noted. His physiological reflexes were present without any pathology. An electrocardiogram demonstrated sinus rhythm with wide (160 ms) QRS duration of left bundle branch block (LBBB) (Fig. ). The patient’s condition was classified as New York Heart Association (NYHA) stage III–IV.\nOn admission, magnetic resonance imaging showed pituitary macroadenoma. Given the symptoms described, we arranged blood testing of myocardial injury markers showing an elevated brain natriuretic peptide level of 740 pg/ml indicating cardiac failure (Table ). Hormone laboratory tests performed subsequently demonstrated excessive secretion of GH and IGF-1, twofold greater than the reference normal upper limit, which was consistent with pituitary macroadenoma (Table ). Other routine analyses of liver and renal function were roughly normal.\nA Holter monitor was ordered for underlying arrhythmias to explain the patient’s dyspnea, chest discomfort, and presyncope. It demonstrated sinus rhythm with an average heart rate of 68 beats/min, frequent ventricular premature beats, and nonsustained ventricular tachycardia (up to 2200 ms) (Fig. ).\nA chest x-ray showed a cardiothoracic ratio (CTR) of 78%. Echocardiography showed diffuse impairment of left ventricular (LV) systolic motion, reaching an LVEF of 16%. We noted hypertrophy of the ventricular septum at 18 mm, ventricular dilation, with LV diameter of 72 mm. The right ventricle and atrium and the left atrium were also dilated with moderate mitral regurgitation and mild tricuspid regurgitation. There was no associated systolic anterior motion (SAM) of the mitral valve. Dyssynchrony of the biventricular systolic motion was apparent.\nGiven an exertional component to the symptoms together with echo presentations in order to better exclude ischemic cardiomyopathy, coronary angiography was performed, which showed normal coronary arteries without stenosis, and left ventriculography applied simultaneously revealed an EF of 20% with diffuse LV hypokinesis.\nGiven the patient’s previous medical history of acromegaly, the absence of obstructive coronary artery imaging findings or segmental dyskinesia, family history of hypertrophic cardiomyopathy (HCM), symmetric hypertrophy, as well as absence of SAM of the mitral valve, acromegaly-induced cardiomyopathy was confirmed, which was absolutely opposed to coronary heart disease (CHD) and HCM.\nThese results indicated that it was probably not a case of hereditary cardiomyopathy; therefore, we diagnosed the patient as having secondary dilated cardiomyopathy due to acromegaly, even taking it a step further progressing to congestive heart failure secondary to acromegaly-induced dilated cardiomyopathy.\nChronic excess of GH and IGF-I secretion affects cardiac morphology and performance [], so etiological treatment for acromegaly-induced cardiomyopathy is crucial to suppressing GH secretion or blocking GH action for the sake of reversing acromegaly-induced cardiomyopathy. The mainstay of treatment acknowledged globally is surgical resection of the pituitary adenoma [], which was unfortunately considered high-risk given our patient’s cardiac condition (NYHA stage III–IV). Although stereotactic radiosurgery combined with somatostatin analogs and GH antagonists administrated previously were effective in suppressing hormones, they could not help his cardiac function. Therefore, we carefully administered diuretics, vasodilators, angiotensin-converting enzyme inhibitor (ACEI), β-blockers, and spironolactone for management of heart failure following the current guidelines []; in the meantime, octreotide (200 μg/day) was administered for the control of GH excess. After good compliance of pharmacotherapy and a regular medical examination regimen for nearly half a year, the serum GH and IGF-1 concentrations decreased from 32.50 ng/ml to 1.98 ng/ml and 627.00 ng/ml to 229.10 ng/ml, respectively, but the patient was hospitalized again because of uncontrollable cardiac failure. Accompanied by the normalization of GH and IGF-1 levels, the patient’s cardiac function did not seem to take a favorable turn upon readmission. Though echocardiography showed a recovered EF value from 16% to 28%, a significant ventricular mechanical dyssynchrony was detected as formerly. Electrophysiological study was performed using a nonaggressive stimulation protocol, which revealed a nonsustained ventricular monomorphic tachycardia []. In the presence of overt ventricular dyssynchrony, complete LBBB, LVEF< 35%, inducible ventricular tachycardia, and symptomatic heart failure despite guideline-directed medical therapy, surgical indication was rarely assessed by neurosurgeons, and stereotactic radiosurgery together with pharmacotherapy produced infinitesimal effects. Therefore, we boldly recommended cardiac resynchronization therapy with defibrillator (CRT-D) implantation based on device implantation official guidelines [, ]. The patient underwent CRT insertion finally and was discharged to home 5 days later, pharmacotherapy continued as usual (Fig. ).\nTelephone follow-up was arranged, and the patient claimed symptom improvement following the device insertion 1 month later and was basically back to normal life. We required that he return for follow-up at 1 month, 3 months, and 6 months after the interventional therapy. The patient has been followed in our outpatient clinic for nearly half a year now. During his last visit, echocardiography identified improved LVEF of 54%, and a chest x-ray showed reduced CTR of 60%. The patient was in NYHA functional class II (Fig. ). |
A 54-year-old male patient presented with a two-day history of severe left-sided, lower back pain which disappeared with the appearance of left-sided lower limb pain felt deep inside the whole lower limb and described by the patient as intense internal pressure (VAS 8/10). On examination, power was 5/5 with no sensory deficit and normal reflexes. The patient received NSAID painkillers and active bed rest was advised. However, on the patient's insistence, a lumbar spine X-ray and MRI were done which showed mild IVD protrusion. The patient was reassured and sent home. A week later the patient presented to the ER with an 18-hour history of heaviness and difficulty in raising the left foot when walking, with numbness along the lateral part of the leg and dorsum of the foot. The pain was moderate (VAS 5/10). No sphincter-related symptoms were observed. According to the patient, the decreased pain caused the delay in presentation, against the instructions on first evaluation. On examination of the foot dorsiflexion was 2/5 (movement on gravity alleviation) with decreased sensation along the left L5 dermatome. There were normal reflexes and sphincters. The patient underwent immediate, new lumbar MRI which showed a large disc sequester with disc migration. His status was fully explained; he was admitted and underwent microdiscectomy L4/5.\nThe initial back pain lasted for 2 days, mostly caused by stretching and pressure on the weakened annulus fibrosis. This type of pain disappears or decreases once the annulus opens and nucleus pulposus leaks to the spinal or root canal. Initial left lower limb pain is a typical description of painful radiculopathy where mass pressure and inflammatory irritation of the nerve root and dorsal root ganglion cause neuropathic pain. This is different from nociceptive and referred pain types. Dorsal root ganglion hosts the cell bodies of sensory nerves with bidirectional connections (to the periphery and spinal cord). Injury to the nerve root motor fibers causes weakness.\nThe majority of patients with lumbar disc and radicular pain improve with conservative treatment. Surgical indications are acute or progressive motor weakness, sphincter dysfunction, intractable pain not responding to analgesia, and pain affecting patient daily life, not responding to 6 weeks of conservative treatment in the absence of acute surgical indications. In all cases, there should be a correlation between the clinical picture and MRI findings. In the current case, the patient has left L5 nerve root symptoms and signs (dorsiflexion weakness, normal reflexes, and dermatologic radiculopathy). The expected IVD prolapse occurred on either the left posterior-lateral L4/5 or left extreme lateral L5/S1 disc. Although there is a debate about dermatologic sensory distribution in root-originating symptoms and variations in clinical presentation (i.e., the L4/5 disc causing S1 root symptoms via compressing the root in higher position, groin pain with lower lumbar discs due to paravertebral sympathetic ganglion pathway entering at L1 or L2 nerve), we presented the commonly encountered scenarios. |
We report a 20-month-old female child with a VP shunt and a gradual distension of abdomen since three months, respiratory distress, and bilious vomiting for two days. She was previously operated for lumbar meningomyelocele with associated hydrocephalus. Right-sided VP shunt insertion was done on the seventh day of life and meningomyelocele repair on the 15th day of life. At the age of three months, there was shunt malfunction for which the right-sided VP shunt was removed and a VP shunt was inserted on the left side.\nAt the time of index admission, she presented with a history of gradual distension of abdomen for 3 months and respiratory distress and bilious vomiting for 2 days. There was no history of fever or abdominal pain. The shunt chamber was compressible. On examination, there was a large, 15 cm × 12 cm, nontender, cystic intraperitoneal lump with a smooth surface and ill-defined margins, occupying almost the entire abdomen; moreover, fingers could be insinuated between the costal margins and the lump.\nX-ray of the abdomen showed VP shunt in the left side of the abdominal cavity having ground glass opacity around the shunt, with the displacement of bowel loops to the periphery of the opacity []. Abdominal ultrasonography (USG) revealed a large multiseptate pseudocyst with the presence of the shunt within. As the baby had considerable respiratory distress, an ultrasound-guided aspiration of 200 ml of a straw-colored fluid was done. The bacteriologic study of the collected CSF showed normal results. However the fluid recollected immediately with increasing abdominal distension and the symptoms persisted; hence, we decided to operate on the child.\nWe went ahead with two-port laparoscopy; one 5-mm primary epigastric camera port above the cyst placed under vision and one 5-mm port in the right iliac fossa. The placement of the second port was difficult, as there was a large CSF pseudocyst predominantly on the left side of the abdomen, with multiple adhesions of bowel loops around the pseudocyst. Gentle maneuvering and dissection with the scope itself helped identify a window in the right iliac fossa where the second port could be placed. A grasper was used to gently free all adhesions of the cyst wall to parietes. The cyst was incised with a hook cautery and 1,100 ml of the CSF fluid was drained. The incision in the cyst wall was extended carefully so as to avoid injury to the adherent bowel loops, and the cyst was then deroofed. The shunt was repositioned in the right subdiaphragmatic space over the liver after confirming the patency of shunt with a free flow of the CSF. The postoperative period was uneventful. On sixmonth follow-up, the child is recovering well and is asymptomatic. |
A 82-yr old male was admitted to our department for sudden onset acute abdominal pain. This patient clinically appeared chronically ill with bed-ridden status. He also has a significant history of urinary incontinence which became progressively worse and an insertion of Foley catheter was recommended at a private healthcare institute. Briefly following this, a 14 Fr Foley catheter was placed after a second attempt by a non-trained orderly at the private healthcare institute. Two days later, slightly blood tinged urine was observed on his urinary drainage bag. He had a past medical history of hypertension for 10 yr, brain surgery for intracranial hemorrhage 5 yr ago and total left hip arthroplasty 10 yr ago.\nClinical examination revealed direct and mild rebound tenderness on his lower abdomen. His body temperature was 36.7℃. There was mildly left-shifted leukocytosis (WBC: 9,300/µL) on complete blood count but it was within upper normal limit. Although the bladder integrity was checked with bladder irrigation and aspiration, we observed a significant discrepancy between input and output fluid volumes. Hence, bladder rupture was suspected and retrograde cystography was immediately performed. The retrograde cystography showed extravasation of the contrast material into the peritoneal cavity and the Foley catheter balloon which was placed in peritoneal cavity (). Subsequently, emergency laparatomy was performed. However, there was no bladder perforation noted and the tip of the Foley catheter was noticed at the rectovesical pouch. There was no evidence of pathologic lesions like cancer or inflammatory mass. Given the location of the Foley catheter, we considered the possibility of false passage of the urethral catheter and subsequently removed the entire catheter. To further examine the extent of urethral injury, on-table, intraoperative urethrocystoscopy was performed. The urethrocystoscopical examination revealed a tunnel-like false passage extending from the verumontanum into the rectovesical pouch between the posterior wall of the bladder and the anterior wall of the rectum. Furthermore, a small hole into the peritoneum was also identified during cystoscopy (). Following the identification of the extent of the urethral injury, a 16 Fr open ended nephrostomy tube was indwelled and safely secured into the bladder under guidance of Amplatz guide wire after full cystoscopic examination. In addition, a 16 Fr cystostomy tube was placed into the bladder suprapubically to ensure adequate urinary drainage. Then, the perforated rectovesical pouch was repaired transperitoneally with simple continuous suture and a closed suction drain was left into the peritoneum. The patient was discharged to the private care institution with only suprapubic cystostomy tube 7 days after the surgery. |
A 37-year-old Caucasian male with a known history of aplastic anemia (AA), presented to a rural hospital after a ground level fall. AA was diagnosed 10 months earlier after he was investigated for pancytopenia. A bone marrow biopsy showed cellularity of only 10% and the presence of a small paroxysmal nocturnal hemoglobinuria clone (less than 0.2%). He received standard combination treatment for AA with cyclosporine 225 mg orally twice daily, horse anti-thymocyte globulin (ATG) 40 mg/kg daily for 4 consecutive days, and prednisone 1 mg/kg daily. His other medications included daily Pantoloc 40 mg orally, daily Valtrex 500 mg orally, and daily Dapsone 50 mg orally for Pneumocystis jirovecii prophylaxis due to a reported allergy to trimethoprim/sulfamethoxazole. He had recently quit smoking and denied alcohol use but actively used other recreational drugs, including marijuana, cocaine, and methamphetamine. He was unemployed. He had no known other medical co-morbidities and was taking no other medications prior to developing AA. The etiology of AA was felt to be idiopathic because he had no improvement after an initial trial of sobriety. AA improved following immunosuppressive therapy and, although human leukocyte antigen typing was performed, a subsequent bone marrow transplant was deferred not only because of the medical therapeutic response but also due to his ongoing recreational drug use. Although he was no longer transfusion dependent a month after starting immunosuppressive therapy, his treatment compliance waned overtime due to regular ongoing recreational drug use of cocaine and methamphetamines. He routinely used unsterilized tap water for illicit drug injections, but he denied other exposure to fresh or salt water sources at home or in the community.\nOn presentation to the emergency department he was not in distress, with a heart rate of 90 bpm and a blood pressure of 116/59. Severe pallor was noted upon examination, as well as a petechial rash and mild ecchymoses (Fig. ). The rest of his physical assessment was normal, including a neurological examination. Admission bloodwork revealed severe pancytopenia with hemoglobin of 22 g/L, a platelet count of 1 × 109/L, a white blood cell count of 3.7 × 109/L, and an absolute neutrophil count of 0.2 × 109/L (reticulocytes were not sent at admission, but 2 weeks into his hospitalization his absolute reticulocyte count was 12 × 109/L with a reticulocyte percentage of 0.5). All other admission blood work was normal, including liver function tests (total bilirubin 9 μmol/L (reference < 21 μmol/L), alanine aminotransferase 13 μmol/L (reference < 41 μmol/L), alkaline phosphatase 66 U/L (reference 30–130 U/L)) and renal function tests (creatinine 63 μmol/L (reference 59–104 μmol/L), glomerular filtration rate 120 mL/min (reference < 59 mL/min)). He was stabilized and transferred to a tertiary care center where he was restarted on treatment for relapsed AA with a regimen that included cyclosporine (5 mg/kg/day) and prednisone 30 mg daily in addition to five doses of ATG. He remained transfusion dependent throughout his hospitalization.\nOn day 10 after admission, he developed generalized, mild (3/10), colicky abdominal pain with an associated fever > 38.5 °C. He was started empirically on piperacillin-tazobactam (PTZ) 3.375 gm intravenously every 6 hours. Two sets of blood cultures, each consisting of an anaerobic and aerobic BacT/Alert bottle (bioMérieux, Laval, Quebec), were collected peripherally and from his central line. E. coli grew in each bottle set at 10 and 11 hours, respectively. He then developed watery, non-bloody bowel movements, 3–4 times a day, associated with rectal pain. Real-time PCR for Clostridium difficile A/B toxin on a stool sample was negative. Computerized tomography of the abdomen and pelvis was also unremarkable. Repeat blood cultures were negative at 24 and 48 hours after the initial positive set. He improved dramatically after 7 days of intravenous PTZ and was stepped down to oral ciprofloxacin 500 mg orally twice daily to complete a further 7 days of therapy.\nOn day 19 of admission he developed acute continuous severe (9/10), non-radiating dull rectal pain, associated with a high-grade fever (40.4 °C). Vancomycin 1.5 g intravenously every 12 hours and metronidazole 500 mg orally twice daily were empirically started and ciprofloxacin was continued in the same dosage. Blood cultures that were collected from peripheral venipuncture and a peripherally inserted central catheter line grew A. hydrophila at 11 hours. The peripherally inserted central catheter line was immediately removed the next day (day 20 after admission). The same day he also began to complain of vague, mild, bilateral leg pain. Delayed serum sickness due to recent ATG administration was considered a possible cause for his new symptoms because clinical examination did not show erythema, edema, or deformities on either of his legs. However, sustained bacteremia was diagnosed by recovery of A. hydrophila from repeat blood cultures (i.e., one anaerobic and aerobic bottle set from two peripheral venipunctures) positive after 11 and 16 hours of incubation. Bilateral leg pain steadily worsened in intensity (10/10) over the next 48 hours, and the area of distribution of pain extended to the lateral aspect of the right thigh although physical examination remained unremarkable. Creatinine kinase was increased at 470 U/L (normal range for males, 0–195 U/L). Ultrasound venous Doppler of both legs also showed no evidence of deep venous thrombosis. However, magnetic resonance images of both legs showed extensive bilateral patchy multi-compartment muscular and fascial inflammatory changes highly concerning for NF (Fig. , ).\nUrgent initial surgical debridement was performed that evening. An extensive four-compartment fasciotomy, debridement, and myomectomy were performed on both legs. Extensive ‘dishwater’ purulent material was found in multiple compartments of both legs, including (1) the superficial posterior compartment between the gastrocnemius and soleus muscles, and (2) the lateral deep compartment. There was also clinical evidence of severe muscle necrosis of the tibialis anterior muscles in the anterior compartment of both legs. He was admitted to the Intensive Care Unit post-operatively. After consultation with the Infectious Diseases service and review of the antibiotic susceptibility profile of the previously isolated A. hydrophila strain, antibiotics were changed to meropenem 1000 mg intravenously every 8 hours and clindamycin 600 mg intravenously every 8 hours. High dose intravenous immunoglobulin (2 g/kg) was also given. All prior antibiotics were discontinued.\nGram stain of tissue samples from the right tibialis anterior muscle showed no neutrophils but that gram-negative bacilli were present, and subsequently grew a heavy amount of A. hydrophila. Gram stain and anaerobic culture from the right vastis lateralis muscle also did not show the presence of neutrophils or organisms but grew scant amounts of A. hydrophila. A genus-level identification as Aeromonas was obtained for all isolates from blood and tissue samples by matrix-assisted laser desorption ionization-time of flight (MALDI-TOF) mass spectrometry using a VITEK MS (bioMérieux, Laval, Quebec, Canada); since this technique has an accuracy of identification rate of 80–90% for species-level identification of Aeromonas [], all isolates were also analyzed using in-house bi-directional 16S rRNA gene cycle sequencing of the V1-V3 (approximately first 500 bp), as previously described []. Broth microdilution susceptibility panel testing was performed and interpreted using published guidelines []. All isolates were multidrug resistant to ampicillin, ceftriaxone, ciprofloxacin, and trimethoprim/sulfamethoxazole but susceptible to meropenem and tetracycline. The isolates were confirmed to produce an extended-spectrum β-lactamase (ESBL) using published guidelines and the Mast Disc Test (Mast Group Ltd., Merseyside, UK) []. Production of an AmpC β-lactamase was shown by resistance to cefoxitin disk (30 μg) testing and the Mast Disc test (Mast Group Ltd.).\nTwo additional extensive surgical procedures for removal of necrotic tissue from both legs were undertaken in the next 24 hours. Bilateral above-knee amputations were performed during the last debridement as a life-saving measure because of extensive rapid progression of bilateral leg necrosis, and the patient’s rapid clinical deterioration with severe unremittent hemodynamic instability during the operation. Post-operatively, he required aggressive resuscitation for septic shock in the Intensive Care Unit with intractable hyperkalemia and severe acidosis, and anuric acute kidney failure (creatinine 210 μmol/L; normal range for males, 50–120 μmol/L). Despite all therapeutic interventions, the patient went into cardiac arrest and passed away within 2 hours after the final surgery.\nPost-mortem examination at autopsy revealed findings related to the underlying AA, and evidence of septic shock secondary to extensive bilateral lower limb necrotizing myofasciitis. The bone marrow was markedly hypocellular and there was splenic enlargement at 331 g. The heart was enlarged (536 g). Cardiomegaly was likely a compensatory response to the AA due to the absence of atherosclerotic and hypertensive cardiovascular disease. In keeping with the patient’s severe septic shock, there was marked centrilobular necrosis of the liver, as well as petechial hemorrhages of the skin, heart, pleural surfaces, kidneys, and liver capsule. Histologic examination of skin and muscle from the left thigh showed necrosis of the muscle and deep subcutaneous adipose tissue, admixed with dense collections of gram-negative bacilli (Fig. , ). However, in keeping with the AA, there was notably an absence of an acute inflammatory response. |
A 14-year-old Thai girl was born via cesarean section due to premature rupture of the membrane with a birth weight of 2500 g. She is the first child of a consanguineous (second-degree relatives) couple. Both parents are healthy and have never had fractures. During her first year of life, she had delayed motor development and growth failure. At one year of age, she could not sit by herself and weighed 7.5 kg (< 3rd centile). She presented to our hospital at 14 months of age with fractures of both femora without a history of significant trauma. She was found to have ptosis of both eyes with normal teeth but no blue sclerae. She was small for her age. Her weight was 7.8 kg (3rd centile) and her length was 68 cm (< 3rd centile). Skeletal survey showed diffuse osteopenia, multiple healed fractures of the right humoral shaft, both tibiae and fibulae. Spine radiograph showed flattening and indentation of vertebral bodies (Fig. ). A diagnosis of OI was made and intravenous bisphosphonate therapy (pamidronate 1 mg/kg/dose for 3 days) was initiated and given every 3 months. However, she sustained 1–2 long bone fractures per year from minor trauma. She required multiple corrective osteotomies to correct her deformities. At the last follow-up, she was 14 years old, weighing 20 kg. She could not walk due to her long bone deformity (Fig. ). Remarkably, although she was in a special education class due to physical disabilities, her cognition was appropriate for age. She could talk fluently and do mathematics properly.\nPrenatally, her younger sister was found to have a dilated fourth ventricle by an ultrasonography. She was born at term via cesarean section because of previous cesarean section and was diagnosed with hydrocephalus at birth. At 4 months of age, she had her first fracture without a history of a significant trauma, leading to a diagnosis of OI. Physical examination revealed a head circumference of 38 cm (> 95th centile) with a wide anterior fontanelle (3 × 3 cm.) and blue sclerae. She had global developmental delay (could not hold her head) and hypotonia. MRI of the brain demonstrated a large posterior fossa cyst connecting with the fourth ventricular system, moderate hydrocephalus, hypoplasia of cerebellar hemisphere with absence of cerebellar vermis, and hypoplasia of corpus collosum. She was also diagnosed with vesicoureteral reflux grade V and gastroesophageal reflux requiring tube feeding. The patient had multiple hospitalizations because of recurrent urinary tract infections and pneumonia. She expired at the age of one year.\nSixteen known OI genes, BMP1, COL1A1, COL1A2, CREB3L1, CRTAP, FKBP10, IFITM5, LEPRE1, PLOD2, PPIB, SERPINF1, SERPINH1, SP7, TMEM38B, WNT1, and MBTPS2, were amplified from 200 ng of genomic DNA using the Truseq Custom Amplicon Sequencing kit (Illumina, San Diego, CA). 286 amplicons which covered all the 226 exons (28 kb) of the target genes were sequenced by Miseq (Illumina, San Diego, CA) using 2 × 250 paired-end reads. SNVs and Indels were detected by Miseq reporter software. The proband was found to harbor a homozygous mutation, c.6delG, p.Leu3Serfs*36 in WNT1. The mutation has never been reported in Human Gene Mutation Database (HGMD; ) (Fig. ). The mutation was subsequently confirmed by PCR-Sanger sequencing. Segregation analysis was performed by using primers, WNT1-E1F: GGT TGTTAAAGCCAGACTGC and WNT1-E1R: ACCAGCTCACTTACCACCAT. The results revealed that the patient was homozygous, while her mother was heterozygous for the mutation (Fig. ). |
A 35-year-old healthy male with complaints of a gradually increasing growth of 9 months duration on right wrist presented to Dermatology OPD. The lesion became more prominent on movement of wrist. There was a history of similar growth in the father. No abnormality was detected on general physical examination. Local area examination revealed a 3 × 3 cm, soft to firm, hemispherical, non-tender, mobile lesion on the medial aspect of right wrist []. Clinically, a diagnosis of lipoma was made and confirmed by FNAC. The patient was not open to any surgical intervention and was given the option of injection lipolysis. After discussing the possible side effects like hyperpigmentation, skin loss, prolonged pain, swelling, or tenderness, late onset hives or itching and skin contour irregularities in detail, an informed written consent for three sessions at 6-8 weeks gap was taken.\nThe lesion was cleaned and draped. The area was divided into small grids (1.5 cm apart) []. Solution of phosphatidyl choline and deoxycholate (50 mg/ml) was filled in insulin syringes (4 injections of 16 units of formula). The pinch and pull technique was used. The lipoma was pulled away from the underlying structures and four units were injected in each of four grids (total of 16 units). Care was taken not to inject any material while withdrawing the needle to avoid any intradermal deposition of the drug. The injections were not perceived as painful by the patient. Immediately after the injections there was swelling, redness and burning sensation which persisted for around half an hour []. No external compression was used and gradually the swelling settled. In the next 48 h, the lesion shrunk to half of its original size and disappeared completely within next 3 weeks, after the first injection itself. The patient is under follow up for the last 9 months with no evidence of any recurrence []. |
A 10-year-old male child presented with complaints of cyanosis since age of 1 year and currently with New York Heart Association (NYHA) class II dyspnea on exertion. He had no history of cyanotic spells. On examination he was averagely built with central cyanosis and grade 2 clubbing. His pulse rate was 90 per minute with blood pressure of 110/76 mmHg. The cardiovascular system examination revealed normal S1, single S2 and continuous murmur best heard in third parasternal area in left intercostal space. The baseline arterial oxygen saturation was 85%. Chest roentgenogram showed decreased pulmonary blood flow with right ventricular type of apex, presence of pulmonary bay and large aorta. Electrocardiogram revealed normal sinus rhythm with features of right ventricular hypertrophy and right axis deviation. There was no evidence of myocardial ischemia. The transthoracic echocardiography showed large subaortic ventricular septal defect (VSD) with 60% aortic override, right ventricular hypertrophy with severe infundibular and pulmonary valve stenosis.\nCatheterization and angiography study confirmed diagnosis of tetralogy of Fallot (TOF) with large VSD, right ventricular hypertrophy and aortic override. There was only trickle of flow to the pulmonary artery in right ventricular injection. The aortic root injection showed presence of large communication from left coronary system opacifying pulmonary trunk. Selective coronary angiogram confirmed presence of fistulous communication arising from left anterior descending (LAD) coronary artery opacifying the main pulmonary artery []. At surgery the fistulous communication was ligated and intracardiac repair performed with Dacron patch VSD closure, infundibular resection, pulmonary valvotomy and right ventricular outflow tract augmentation using autologous glutaraldehyde treated pericardial patch. The patient had an uneventful recovery and discharged on day 10.\nThe congenital coronary artery to pulmonary artery fistula is a rare anomaly with a reported prevalance of 1 in 50000.[] However, congenital coronary artery to pulmonary artery fistulas are reported to occur commonly in patients with VSD and pulmonary atresia with reported incidence of 10%.[] The cause of congenital coronary arteriovenous fistula is believed to be a failure of obliteration of the intramyocardial trabecular sinusoids with anomalous development of the intratrabecular spaces, through which blood is supplied to the myocardium during intrauterine life. Small coronary to pulmonary artery fistula in setting of TOF had been described.[] However, the reports of large communication between pulmonary artery to coronary opacifying the whole trunk from the coronary injection have been recent only.[] In one report there was a fistulous communication reported between left main coronary artery to main pulmonary artery in a single coronary artery system.[] The other report there was collateral from left circumflex artery to the right pulmonary artery.[]\nThe surgical importance of coronary artery fistula to pulmonary artery lies in the fact that the communication needs to be identified and ligated before commencing cardiopulmonary bypass to prevent loss of volume to pulmonary vascular bed. In case of failure to close such communication prior to application of aortic cross clamp there will be loss of cardioplegia solution to pulmonary circulation and incomplete myocadial protection during administration of cardioplegia. Physiologically such communications in TOF helps in increasing pulmonary blood flow and reduction in degree of cyanosis. In our case the infundibular stenosis was so severe so as only to allow hegar dilator of size 3 with difficulty, yet the saturation was 85%. Other importance of major coronary artery to pulmonary artery fistula could be myocardial ischemia, though not present in our case.\nThe identification of such communication is important by preoperative investigations so cineangiography, computed tomography angiography or magnetic resonance angiography is necessary in each patient of TOF especially when echocardiography reveals severe infundibular stenosis but the patient is not so blue. |
A two-month-old girl was admitted to a pediatric university hospital because of convulsions (Table ). Twenty days prior to admission she was seen by her local doctor because of a 1st to 2nd degree burn of the chest (<10% total body surface). According to the caregivers this resulted from a leaking bottle cap. Although the pattern could be consistent with this explanation there was a delay in presentation at that time which, in retrospect, should have warranted further evaluation.\nOn admission cranial ultrasonography showed diffuse hyperechogeneity of the brain parenchyma with increased demarcation of the white matter—cortical border; flow reversal as a sign of increased cranial pressure was also seen (Fig. ). Computed tomography performed 1 day after admission showed diffuse severe ischemic brain injury (Fig. ). Skeletal survey findings are shown in Table . Fundoscopy showed bilateral intra- and pre-retinal hemorrhages. MRI on day 7 showed a post-anoxic brain with a subdural hematoma. A subsequent MRI performed on day 38 showed MCE (Fig. ).\nThe clinical, ophthalmological and radiological findings were all highly suggestive of AHT. The girl died at the age of five-and-a-half months, and as the death was related to child abuse a judicial autopsy was performed.\nNeuropathological examination of the brain and the spinal cord revealed a unilateral, several month old neo-membrane, consistent with an old subdural hematoma. The neo-membrane showed complete organization with moderately cellular connective tissue, normal size blood vessels and extensive iron deposits. There was evidence of several small recent rebleedings in the neo-membrane as well. The brain was extremely atrophic, weighing 200 g (normal weight for age approximately 490 g) []. Atrophy was due to a diffuse loss of the cerebral cortical neurons and mainly subcortical necrosis of the white matter. In these areas small to medium sized cavitations occurred (Table ). The deep white matter revealed extensive reactive gliosis but no cavitation. The ventricular system was moderately enlarged. The cerebellum was less affected; loss of the Purkinje cells and the small neurons of the granular layer were observed in the depths of the cerebellar folia, whereas more superficially neurons were mainly spared. The brainstem and the spinal cord showed no major abnormalities apart from secondary degeneration of the corticospinal tracts due to the loss of cortical motor neurons.\nSevere MCE was considered to be related to a generalized hypoxic accident which must have occurred several months prior to the patient’s demise. The cause of death was ruled as a result of brain dysfunction. |
A 21-years-old Caucasian woman presented to a private dental clinic with a chief complaint of asymptomatic swelling in the gingiva observed four years prior. A gradual increase in size and no history of previous treatment were also reported during the anamnesis. The patient signed the informed consent, which represents the ethical approval of the faculty committee. Her medical and socio-economic histories were not contributory. The extra-oral evaluation did not reveal changes. The intraoral examination revealed a sessile nodule with a color similar to that of the mucosa and a focal erythematous area with a fibro-elastic consistency measuring 1.5 cm in the largest diameter extending from the inferior right lateral incisor to the inferior right first premolar. The lesion involved the vestibular and lingual gingiva, causing displacement of the inferior right canine (Fig. ).\nPanoramic reconstruction and parasagittal slices of the Cone Beam Computed Tomography (CBCT) showed a slightly superficial hypodense area between the inferior right lateral incisor and inferior right canine with reabsorption of the alveolar crest (Fig. ). Based on the clinical and immunological aspects, the main diagnosis hypotheses included peripheral ossifying fibroma, peripheral giant cell lesion, and ancient pyogenic granuloma. The peripheral odontogenic tumors were also included as a differential diagnosis. An excisional biopsy was performed and a clear separation was noted between the lesion and mandible bone during the trans-surgical approach. The histopathological analysis revealed a well-circumscribed proliferation comprising numerous islands and strands of epithelial polyhedral cells with well-defined borders and marked round nucleus in the connective tissue under the mucosal epithelium. Numerous nests, cords, and small islands of polyhedral cells with clear and vacuolated abundant cytoplasm were observed interspersed with the amorphous eosinophilic deposits (Fig. ). Immunohistochemistry was performed, which yielded positive results for CK-19 in the epithelial cells, except for the clear cells. Congo red staining showed the presence of amyloid-like deposits with apple-green birefringence under polarized light (Fig. ). A final diagnosis of a peripheral CEOT rich in clear cells was reached. No complications were observed in the postoperative appointment and a follow-up schedule was established. The patient has had no recurrence after 22 months (Fig. ). |
Sixty-three-year-old woman presented with multicentric left breast cancer requiring mastectomy. She is 69 inches tall with a weight of 215 pounds and a corresponding BMI of 31.8 kg/m2. She has a history of inferior pedicle breast reduction surgery performed 20 years ago (Fig. 1). Despite her previous reduction, she has a very large breast volume and footprint that will be difficult to replicate on the reconstructed left side. She desires nipple preservation and wants to keep her native right breast. We proceed with a left nipple-sparing mastectomy through her previous vertical limb and immediate prepectoral reconstruction using a full-height variable-projection tissue expander (width = 16 cm, height = 16.5 cm, projection = 6.8 cm, and volume = 850 ml) with anterior coverage using an acellular dermal matrix. Twelve weeks later, we exchange her tissue expander for the largest anatomical implant available, a full-height extra-projection 775 ml implant (width = 15.5 cm, height = 16 cm, and projection = 7.1 cm). She also undergoes a contralateral reduction of 300 g to achieve better symmetry. The final result is shown in Figure . Despite using the largest and tallest implant available and reducing the right breast by an additional 300 g, the entire upper pole of the left breast was depleted with significant size asymmetry between the breasts. We discussed multiple sessions of lipofilling to fill this defect but felt that an autologous flap would be more definitive. The LICAP flap was chosen to reconstruct the upper pole of the left breast. An intraoperative photograph is shown in Figure , where an extended flap is dissected based off the known perforators that arise anterior to the latissimus muscle at the level of the inframammary fold as previously described. This flap is rotated on its pivot point and used to reconstruct the upper pole of the breast by suturing it to the underlying pectoralis muscle. The final result 6 months after surgery is shown in Figure , where we have reconstructed the upper pole of her breast with the LICAP flap and have acceptable symmetry between the 2 sides. The patient is discharged the same day, and drains are removed on postoperative day 4. |
A 15-year-old female patient (bodyweight 50 kg, height 160 cm) presented with syncope. Severe arterial hypertension was diagnosed (178/147 mmHg left arm, 102/83 mmHg left leg). She reported previous episodes of exercise independent headaches and nose bleeds. Auscultation revealed no cardiac murmurs, but an accentuated second heart sound and reduced pulses in the lower limbs. There were no clinical signs to suggest Alagille or William’s syndrome or clinical evidence of neurofibromatosis. Blood tests showed normal inflammatory markers and normal creatinine. The urine dip showed no markers for blood or protein. No regular medication was taken at the time she presented. The electrocardiogram showed normal sinus rhythm, no significant signs of left ventricular (LV) hypertrophy or abnormal repolarization. Echocardiography detected a severe concentric LV hypertrophy (14 mm diastolic septal diameter, 15 mm diastolic diameter of the LV posterior wall) and a long hypoplastic segment in the abdominal aorta with massive arterial collateralization. Cardiac catheterization confirmed the diagnosis of a midaortic syndrome with a minimum diameter of <1.5 mm and a gradient of 50 mmHg between the aortic arch and the femoral arteries (Figure ). The narrow part began directly under the diaphragm above the truncus coeliacus and continued down to the bifurcation of the arteriae iliacae including the renal arteries, with reversed flow in the caudal aorta up to the renal arteries. Because of the extraordinary length of the hypoplastic segment of 23 cm, we decided for a stepwise interventional therapy with a paclitaxel covered balloon. No additional computed tomography scan or magnetic resonance imaging (MRI) was performed. Consultation with our surgeon, supported the interventional approach. The proposed procedure was reviewed and approved by our institutional review board, extensively discussed with the patient’s family, and informed consent was obtained before proceeding.\nAfter balloon interrogation of the long stenotic abdominal aorta using a 6 × 20 mm Tyshak balloon (NuMED Inc., Hopkinton, NY, USA) to differentiate between rigid stenosis and hypoplastic parts, serial balloon dilation with a 7 × 40 mm paclitaxel covered In.Pact balloon (Medtronic, Minneapolis, MN, USA) (3 µg/mm2 Paclitaxel on the balloon) was performed (Figure ) from end to end of the stenosis. Planned redilations, to stepwise improve the aortic compliance and reduce the risk of dissection, were performed after 2, 4, and 8 months with Elutax balloons ranging from 8 mm to 12 mm diameter (Aachen Resonance, Düsseldorf, Germany) (2 µg/mm At the end of the last interventional procedure, a mild endothelial lesion at the former narrowest point of the aorta was noticed (Figure ). This lesion healed and after 2.7 years MRI showed a stable and adequate result (Figure ). Today the girl is in unrestricted physical condition. The blood pressure has markedly improved (139/64 mmHg right arm and 127/65 mmHg right leg) under therapy with atenolol and amlodipine.\nThe patient and patient’s family consented to the publication of this case’s history and the images presented. |
A 32-month-old Middle Eastern boy was born full term at a community hospital in Michigan with birth weight of 3135 g (15.0 percentile). He had normal prenatal ultrasounds. He passed meconium at birth and had no other complications including prolong neonatal jaundice or dehydration. His CF NBS showed serum IRT 139 ng/ml and was negative for the 40 gene mutations panel. At 1 month of age, he developed a wet cough without any other symptoms. He was followed by his primary care provider (PCP), and no treatment was given at the time. His symptoms continued on and off until 1 year of age. At 1 year, the mother noticed increased frequency of productive cough, lack of appetite, and poor weight gain. His weight-for-age percentile ranged from 0.3 to 5.0. His stools were reportedly normal. He had no excessive sweating. He was referred to an outside asthma/allergy specialist for evaluation of asthma. He was prescribed budesonide without any improvement. He had frequent pharyngitis and otitis media that were treated with oral antibiotics that reportedly helped treat acute infection, but the cough persisted. He was also prescribed a H2 blocker for possible gastroesophageal reflux disease, but no improvement in symptoms was noted. Family history was negative for CF.\nAt 30 months of age, he was seen by his PCP for one week of cough and fever. He was treated with amoxicillin. His symptoms continued to worsen despite oral antibiotics, and he had two episodes of small-volume hemoptysis. He was subsequently admitted for community-acquired pneumonia and influenza B. Chest X-ray showed diffuse ill-defined opacities in the perihilar area and diffuse bronchiectasis. During the hospitalization, pediatric pulmonary consult was obtained. Given the negative NBS, it was stated that CF was unlikely and no sweat chloride test was recommended. He had a normal videofluoroscopic swallow study. Immunodeficiency workup revealed elevated immunoglobulin levels, protective vaccine titers, and normal lymphocyte counts and response to phytohaemagglutinin, concanavalin A, and pokeweed mitogen. HIV test was negative. Pediatric gastroenterology was consulted for failure to thrive and recommended to continue high-calorie diet. He was discharged home on augmentin.\nTen days following discharge, he was seen at the immunology clinic. He was noted to have digital clubbing, worsening tachypnea, and crackles. With the concerning physical exam findings, a sweat chloride test was done with a result of 90 mmol/L (normal 0–29 mmol/L; intermediate 30–59 mmol/L; abnormal ≥60 mmol/L) []. He was referred to pediatric pulmonary clinic the same day. He was then admitted and treated for a CF exacerbation. Throat culture grew Pseudomonas aeruginosa and methicillin-sensitive Staphylococcus aureus (MSSA). Fecal elastase-1 was <50 mcg E/g stool (normal >200 mcg E/g stool). Lab results including comprehensive metabolic panel and vitamin A and E levels were normal. He completed two weeks of cefepime and tobramycin.\nAfter notifying MDHHS with the false-negative NBS results, the blood spot that was available at the NBS lab was retested using the new and expanded mutation panel (60 mutations). He was found to be homozygous for R1066C (c.3196C > T; p.Arg1066Cys) mutation. His care was transferred to our CF center, as per parents' request. Two weeks later, he was admitted for worsening respiratory symptoms and treated for a CF exacerbation. Vitamin D level was low at 25 ng/ml (normal ≥30 ng/ml). High-resolution computed tomography of the chest showed diffuse bilateral bronchiectasis (). Flexible bronchoscopy showed airway erythema and significant thick green secretions () that was positive for MSSA. |
We describe a 78-year-old woman who underwent partial resection of the left lower lobe due to lung carcinoid 20 years previously. Details of the surgery were unavailable. Five years before the time of this report, when she underwent an annual medical check-up, an abnormal shadow was noted in the left lower lung on chest radiography. A chest computed tomography (CT) scan revealed partial atelectasis in the left S8. Two years later, CT showed similar findings (Figure ). CT was repeated 2 years subsequently and it showed no obvious changes (Figure ). However, CT performed 1 month before the patient presented to our hospital revealed that the atelectasis had developed into a mass, suggesting malignancy. Thus, she was referred to our hospital for further investigation and treatment. In our institution, a CT scan showed a 45 × 37 × 32 mm mass with fine calcification in the left S8 (Figure ). Contrast-enhanced CT showed enhancement around the mass (Figure ). There were no suspicious findings of malignancy or infection in other lung fields or other organs. The patient had no subjective symptoms or previous episodes of pneumonia or other infections. White blood cell count and C-reactive protein level were within normal limits, and the levels of tumour markers for lung cancer (carcinoembryonic antigen, cytokeratin 19 fragment and pro-gastrin-releasing peptide) were also within normal limits. Bronchoscopy was unable to visualize the mass due to B8 obstruction. FDG-PET/CT showed accumulation consistent with the mass, with a maximum standardized uptake value (SUVmax) of 8.91 (Figure ). Based on these results, we performed surgery to confirm pulmonary malignant tumour, including the recurrence of carcinoid, although it had been a long time since carcinoid resection was performed. After intraoperative diagnosis through fine-needle biopsy, the mass was suspected to be epithelioid cell granuloma; thus, the S8 segment was resected. Pathological examination revealed a large mass of 25 mm diameter and a 10-mm suture granuloma centred on the suture (Figure ). Histologically, the mass was an epithelioid cell granuloma with necrosis (Figure ). No fungi were detected by periodic acid-Schiff or Grocott's methenamine-silver staining. Although no organism was identified by acid-fast staining of tissue sections and no acid-fast bacillus was cultured, it was suspected that the granuloma formation was due to an atypical acid-fast bacillus infection because of a high serum anti-MAC antibody level. The suture granuloma, with a braided non-absorbent suture at its core, was formed by minimal inflammatory cell infiltration, without an abscess (Figure , ). The epithelioid cell and suture granulomas were discontinuous. |
A 53-year-old woman of Hispanic descent presented to the emergency department at our institution complaining of foul-smelling discharge and pain at the left breast, as well as fatigue and a 24-lb weight loss over the prior 2 months. The patient reports that she first began to experience pain within her breast 3 months ago, at which time she began to treat herself with “alternative therapies.” On examination, she was noted to have a large, ulcerated, fungating breast mass with foul-smelling purulent drainage and bleeding (a). Her hemoglobin level on admission to the hospital was 4.2. The patient reported that she avoided seeking medical treatment of her breast mass until this admission because of fear of treatment. Her 44-year-old sister died of breast cancer after a long treatment course and multiple operations. At the time of her presentation, the patient lived at home with her mother, husband, and son.\nAfter receiving 4 units of packed red blood cells, the patient underwent MRM with removal of the pectoralis major fascia and axillary lymph node dissection. Pathologic stage was found to be T4bN4p3a with a mass size of 19 × 15 × 12 cm. Reconstruction was performed in 2 stages, the first of which involved closure of axillary contents with the application of a negative pressure wound therapy dressing on the day of initial resection (b). The second stage of reconstruction involved a 240-cm2 split-thickness skin graft (c). The patient had a 15-day hospital course at our institution. She refused discharge to hospice and was instead discharged home.\nThe patient refused postoperative radiation therapy and missed multiple chemotherapy appointments with medical oncology. When contacted via telephone, the patient cited “excessive breast pain” as the reason for her poor follow-up. She never returned to our clinic for a postoperative examination. She subsequently revisited the emergency department 2 months after her discharge complaining of a recurrent left breast mass and discharge from the breast. Examination revealed a large fungating breast mass protruding through the graft site (d). At this point, the patient was discharged home without further surgical or medical intervention for her disease. |
The patient was a 70-year-old man with COPD. The patient had a history of numerous hospital treatments for acute exacerbation of COPD and had been maintaining his condition on home O2 (1-1.5 L) therapy for shortness of breath. The patient underwent lung volume reduction (LVR) on the right upper lobe (RUL) on August 15, 2012, had a valve removed from the existing RUL, and underwent LVR on the left upper lobe on 2012-12-18. After the LVR, the dyspnea worsened and performance was maintained at a level that only allowed movement around the house. Oxygen was used nearly every day and the patient did not leave the house. The dyspnea worsened 4-5 days prior to the hospital visit and reached a severe level on the day the patient visited the emergency room (December 27, 2013). After receiving intravenous treatment of levofloxacin and methyl prednisolone, the patient was admitted to the general ward (GW).\nImmediately after admission, the patient was transferred to the intensive care unit with drowsy mentality, and for 5 days after admission, 1600 kcal was administered via enteral tube feeding through an L-tube. When the patient became alert, the patient was transferred back to the GW on January 10, 2014 and the diet was switched to an oral soft diet. However, due to low consumption, 2 bags of medical food supplement were prescribed starting on January 21, 2014. On January 24, 2014 (27 days after admission), nutrition management was requested due to a continued low oral intake. This process is shown in .\nAt the time of the first nutritional training on January 24, 2014, the patient's height and weight were 157.5 cm and 40.8 kg, BMI was 16.8 kg/m2, and percentage of ideal body weight was 75.8%, indicating that the patient was severely underweight. The patient's usual weight was 49 kg, and the patient had experienced an extreme weight loss of 14% during the 1 month. The patient's triceps skinfold measurement was 60% and mid-arm muscle circumference was 79%, figures accompanied by loss of both fat mass and FFM. No bioelectric impedance analysis (BIA) or bone mineral density (BMD) measurements were taken, and no parenteral nutrition, medical food supplement (Encover), or additional nutritional supplements (vitamins, omega-3 fatty acids) were prescribed.\nThe patient was a senior who lived alone without a caregiver and had diminished masticatory function that made chewing foods difficult despite dentures. Although the patient did not have dysphagia, the patient had constipation but showed normal bowel movements or frequent small amounts of stool. Despite complaints of severe fatigue and dyspnea, the patient maintained a 3-meal-a-day diet and showed no changes in diet patterns. The patient tended to eat mainly rice and leave other foods nearly untouched, and the patient's daily consumption consisted of 1,500 kcal, including 45 g of protein (C:P:F = 75:12:13) and one packet of soymilk and an oral nutrition supplement. During the first nutritional education session, the diagnosis was determined to be severe malnutrition as a result of chronic disease. The nutrition diagnosis and intervention are shown in .\nAt the first F/U 3 days later, the patient expressed at the interview, "I take breaks during the meal and finish everything including the side dishes. I drink one Encover. If I snack, I cannot eat much during the meal". With no significant changes in bowel movements, the patient's weight increased to 43.2 kg. There were no other changes to the patient's objective data. However, although the patient previously showed an activity level of barely being able to go to the washroom, the patient started walking around the ward supported by a walker. The patient demonstrated a diet exceeding the targeted nutritional requirement, but the patient's activity level increased. Since the patient's oxygen saturation was well maintained, the patient has not received a F/U after the measurement of 1/9 pCO2 44.3 mmEq/L (normal range, 35-45), and no adverse effects of the excessive energy supplementation were observed. Hence, the nutritional requirement was reconsidered and increased, and the continuous maintenance of meal intake was encouraged. Another department suggested that if the current oral intake level is maintained, the nutritional supplement drinks should be discontinued. The nutrition diagnosis and intervention contents at the first follow-up encounter are shown in .\nAt the F/U 13 days later, the patient expressed at the interview, "I eat all that was provided, but it seems that the quantity of meals got smaller the last couple of days. In terms of feces, it is not diarrhea, but the frequency of bowel movement is similar". Since February 7, 2014, due to the change in prescription to the regular diet, the patient's dietary satisfaction decreased. The patient's weight was maintained at 42.2 kg with no changes, and the activity level was maintained at the walking level since the use of the walker. However, a slight increase in albumin and improved complete blood count indicators were observed; thus, any changes to the consistent diet maintenance were observed through objective indicators. Therefore, nutritional intervention was once again performed to ensure consistent diet maintenance and the patient was put on a high protein diet again. The nutrition diagnosis and intervention contents at the second follow-up encounter are shown in .\nAt the time of discharge 1 week later, the patient's weight had increased to 44.8 kg and laboratory findings improved, demonstrating the positive effects of active nutritional support. The objective data of the patient is shown in . |
The patient was a 61-year-old man with multiple left rib fractures (1–6 ribs), left pneumothorax, left lung contusion, and left thoracic subcutaneous emphysema due to a fall injury. The examination showed a partial depression in the left front rib and abnormal breathing (see Fig. ).\nAdmission chest CT examination: 1–6 rib fractures on the left side (of which 3, 4 ribs are long comminuted fractures (see Fig. )); left pneumothorax, left traumatic wet lung; a small amount of liquid pneumothorax on the left side.\nPatient was given early chest straps, multiparametric monitoring, analgesia, and oxygen therapy. The chest pain was still severe. The visual analogue scale scored 7–8 points for the pain at rest and 9 points for the cough.\nPhysical examination revealed that the left chest wall was recessed and abnormally breathed. The CT scan of the rib showed a long comminuted fracture of 3 and 4 ribs. The key to successful operation was the reduction and fixation of these two rib fractures. A preoperative CT scan was performed to reconstruct the 3D model based on the scan results (see Fig. ), and 3D printing technology was used to prepare 3 and 4 rib models (see Fig. ). The three D print models of each fracture segment of the two ribs were adherently reconstructed.\nThe two rib metal plates were separately shaped according to the reconstruction model (see Figs. and ).\nThe patient is scheduled to have a open reduction and internal fixation of 3–6 rib fracture. After general anesthesia, right side lying position, small incision about 8 cm was performed under the edge of 4th rib underarm. The skin was sequentially incised and the subcutaneous tissue was freed layer by layer. The front of the latissimus dorsi muscle and the anterior serratus were exposed. The tunnel was established on the 3rd and 4th rib surfaces from the back of the chest and small muscles to the back of the scapula. The special long hooks lifted the scapula and exposed the scapular operation space. With assistance of endoscope, the electrocautery is useful to expose 3 cm outside the outermost fracture lines of the 3 and 4 ribs. The locking plate was molded on the surface of the third rib before operation, and the broken end of the non-fracture at the anterior and posterior portions of the third rib was well fitted. The distance between the two ends of the metal bone plate exceeded the fracture line to 3 nail holes distance. Under the thoracoscope, the metal plate and the ribs were temporarily fixed with long-angled forceps. The MIPO system was used to drill the holes. Two screws were implanted and locked at both ends to firmly fix the metal plate. In turn, each fracture segment was reset and drilled and secured to a metal plate. The fourth rib is fixed in the same way. Intraoperative image (Figs. , and ). 5, 6 rib fractures given to fix the ribs, not the content of this article, not elaborated. Sufficient to stop the bleeding, the wound was given to leave a negative pressure drainage tube. After a routine thoracoscopic probe of the chest cavity, a closed thoracic drainage tube was placed posterior to the 7th intercostal space and the incision was closed layer by layer. After the chest wall is well-shaped. Three days after surgery review the map (Fig. ). |
A two-year-old boy presented to the outpatient department at a tertiary care hospital in Karachi, with a complaint of multiple swellings on his chest since birth. He was also suffering from a fever and cough since three days and complained of symptoms that indicated respiratory distress for a day. According to the patient’s mother, the swellings were noticed by her when he was born. However, there was no cause for concern until he had turned one year old. In the last three days, the patient developed a fever and cough, and due to worsening condition, he was brought to the outpatient department. On further questioning, we established that the patient’s respiratory complaints were of a recurrent nature. The patient had already presented and been treated for respiratory infection and distress twice the same year, though further evaluation of his chest swellings had not been carried out. Furthermore, the family was told that the child could have a congenital heart disease, but no relevant work-up had been done. The patient had no contact history with tuberculosis (TB), which is a major contributor to such symptoms in our country. The patient did not have any significant surgical, drug, allergy or blood transfusion history. Interestingly, the patient had a positive family history when it came to the bony outgrowths, with the father, a paternal uncle and his paternal grandmother having similar deformities at different sites over their bodies. However, none of them had the outgrowths over their ribs, nor did they have any features of recurrent respiratory infection. The patient’s appetite, sleep and bowel habits seemed to be normal as well. The review of systems was unremarkable.\nOn presentation, the child was irritable, and his respiratory rate was 50 breaths/min. His temperature was also raised, measuring 102°F. Other vital signs were normal. On examining the chest, multiple non-tender swellings were noticed bilaterally, which seemed to be originating over the ribs, anteriorly and posteriorly (Figures -). There was dullness to percussion in the lower zones of the lung, and crepitations were also noticed on auscultation in the same region. Owing to the recurrent nature of his respiratory condition, and the yet to be evaluated chest swellings, the patient was admitted for further evaluation.\nThe patient’s sputum and blood were sent to be cultured to identify the causative organism, which was leading to pneumonia. A chest X-ray was also carried out to aid us in reaching a definitive diagnosis, which revealed bilateral consolidation patches, left-sided lung fibrosis, left lateral chest wall indrawing, lymphadenopathy and mediastinal widening (Figure ). We carried out the relevant tests to rule out the differentials. For TB, the sputum was sent for culture and staining, and a GeneXpert was carried out as well. Primary immunodeficiency syndromes were ruled out by measuring serum immunoglobulin levels. The recurrent pneumonia also urged us to work up the patient for cystic fibrosis. However, both the sweat test and the genetic studies came out negative. Type 1 diabetes was also ruled out based on random and fasting blood sugar levels. To assess the patient for congenital heart disease, we carried out an echocardiogram. This did not show a congenital abnormality but demonstrated an ejection fraction of 40%, along with a dilated left ventricle, which could be due to the recurrent infections. To assess the bony outgrowths on the chest, we ordered a CT scan which revealed exostoses on the left third, fifth and sixth ribs, and right second, third, fourth and sixth ribs as well as on the medial border of the scapulae, bilaterally. The CT scan supported the chest X-ray findings which revealed multiple homogenous opacities, along with hyperinflated lung fields. Based on such evidence, we were able to diagnose this case as being one of HME, leading to recurrent respiratory infections.\nA diagnosis of HME leading to recurrent respiratory infections was made based on the previously mentioned investigations. After admission, the patient was administered a penicillin antibiotic given eight-hourly. The patient was nebulized every six hours as well, as managing his respiratory distress due to the respiratory infection was our primary goal. The patient was examined regularly to note his progress and by the fifth day, our patient was free of fever, cough and chest congestion. The relevant investigations were carried out, and once the diagnosis was reached, our patient was referred to the orthopedic department where his resection would be managed. The operation was carried out within a month of his admission and three months later, he showed no signs of developing a respiratory infection again. Informed written consent was obtained from the patient and his family, granting us permission to report on his condition and the relevant images. |
The 41-year-old female referred to the medical toxicology department of Noor Hospital in Isfahan on January 18, 2019. The patient was alert and complained of headache, nausea, and vomiting. The patient stated that about 11 h ago, she took 20 tablets of colchicine 1 mg, 20 tablets of chloroquine 250 mg, and 40 tablets of Telfast 120 mg for suicide attempt. The patient had a history of addiction to opium and had used methadone syrup recently. She also had a history of gout, hypothyroidism, fatty liver, nephrolithiasis, and pulmonary surgery due to empyema and had been treated with levothyroxine, Telfast, and colchicine. The patient had no previous suicide attempts. On examination, the pupils were normal in size and symmetrical. Heart and lung examinations were normal. She complained of mild pain in the epigastric area. The vital signs were normal. The patient's laboratory values were in the normal range at admission and on the 1st day. The patient underwent conservative treatment with hydration and cardiopulmonary monitoring. Twelve hours after admission, large–bore IV access was taken, and normal saline was started due to decreased blood pressure.\nThen, 24 h after admission, the pain was suddenly exacerbated in the epigastric area and radiated to the hypogastric part. She again complained of headache, nausea and vomiting, burning sensations in the limbs, and diarrhea. In addition, 36 h after admission, the patient initially complained of visual loss, and after 48 h of hospitalization, her blood pressure dropped sharply so that the radial pulse was not detectable, followed by a decrease in blood sugar and saturation of peripheral oxygen. She was intubated. Electrocardiographic abnormalities included the right bundle branch block and left posterior hemiblock, and then developed to cardiac arrest. The patient died 2 h later despite the cardiopulmonary resuscitation.\nDecrease in platelet count (109 × 103/mm3), serum sodium (128 mEq/L), and blood sugar (25 mg/dl), severe respiratory acidosis (pH: 6.557), and increase in serum potassium (4.8 mEq/L), creatinine (3.5 mg/dl), partial thromboplastin time (>120 s), international normalized ratio (>5.7), aspartate aminotransferase (513 U/l), and alkaline phosphokinase (2536 U/l) were the most important laboratory findings of the patient on the 2nd day of hospitalization. |
A 13-year-old boy came to our hospital with history of intermittent knee pain, swelling, and with minimal limitation of movements for the past 1 year, no history of the limitation of mobility and no history of injury noted. On examination, he had tenderness over lower end of femur. No signs of infection were observed, and laboratory data were within normal limits. The radiographs revealed an ovoid radiolucent lesion with sclerotic margins over posterolateral aspect of epiphysis in distal end of the left femur with epiphyseal breach and transphyseal extension ().\nMagnetic resonance imaging (MRI) showed a 4.5 cm × 3.7 cm × 3.7 cm well-defined T1 intermediate and mildly hyperintense lesion in the right distal femoral epiphysis. It had lobulated margins with narrow zone of transition, thin arc-like calcification within the lesion suggestive of the chondroid matrix. The lesion is fairly centrally located within epiphysis (more to the lateral half), superiorly transphyseal extension into metaphysis, and inferiorly thinning of cortex with the focal cortical breach (). Computed tomography-guided biopsy was taken, and histopathological reports showed oval nuclei with few showing evidence of longitudinal grooving and moderate eosinophilic cytoplasm. Interspersed in between is multinucleated osteoclastic types of giant cells and also seen are foci of chondroid areas with eosinophilic matrix and foci showing characteristic deposits of chicken wire type of calcification ( and ).\nWe planned to proceed with intralesional curettage and bone grafting. The procedure was carried out under general anesthesia. Midline incision with a medial parapatellar approach used. Intraoperative picture presented as a breach through intercondylar fossa with thinning of cartilage (). Plan for a thorough curettage was carried out by making a small window over the non-weight-bearing area. The cartilage window was elevated and the lesion was reached. Intralesional resection of the cyst contents was performed. Thorough curettage was done under direct vision and made sure that no lesions left out, which was confirmed under the guidance of fluoroscopy intraoperatively and sample was sent for histopathological examination. The cavity was treated with hydrogen peroxide followed by normal saline [] and packed with ipsilateral iliac crest bone graft and synthetic bone substitutes (Allogran R-porous calcium phosphate). Since the cartilage window had adequate cover, we do not experience any spillage of graft and cartilage window was closed and sutured (). Post operative radiographs confirmed the entire removal of lesion ()\nThis technique helped in protecting and preventing further damage to the cartilage. It also gave a good approach to the lesion for a complete curettage. We avoided the lateral approach because, during curettage, it can perforate intra-articularly or causing more damage to physis. Post-operatively, the patient was immobilized with a knee brace. Post-operative biopsy reports also confirmed that the features were consistent with chondroblastoma. Isometric quadriceps muscle and straight leg raising exercises were initiated on the second post-operative day, following which non-weight bear walking was encouraged. The brace was removed 3 weeks after the surgery. Active and active-assisted range of motion exercises were initiated at that time. Two months after the surgery, the patient was pain-free, ambulating full weight-bearing with a full range of movements and without support. Follow-up was done at 6 weeks, 3 months, 6 months, 9 months, and 1 year after the curettage. There was no recurrence of the lesion with a resolution of knee symptoms. Knee movements were not affected. No radiological sign of recurrence was found at 1-year follow-up (). |
A 73-year-old female was referred to our Trust by her general practitioner with a 5-month history of a painless vaginal mass, which extruded from the introitus on straining, but was otherwise asymptomatic. This was originally thought to be a vaginal prolapse; however, examination revealed a soft, well-defined pink mass occupying the upper vagina and an MRI of the pelvis was requested for further characterization.\nMRI was performed using a 3.0 T system utilizing axial T1 weighted fast spin echo; small field of view axial, coronal and sagittal T2 weighted fast spin echo; and T1 weighted fat-saturated sagittal images before and after gadolinium contrast administration, obtained in the arterial and portal venous phases. Diffusion-weighted imaging was also acquired. The images demonstrated a 47 × 40 × 44 mm well-circumscribed, oval mass in the upper vagina. On the T1 weighted images, the signal intensity of the abnormality was intermediate, similar to that of the skeletal muscle (). However, on T2 imaging, there were discrete zones within the lesion; the anteroinferior aspect was of high T2 signal with no enhancement, whereas the posterosuperior aspect was of low T2 signal with avid enhancement (–). There was no restricted diffusion. The posterior wall of the retroverted uterus was demonstrated to abut the superior surface of the lesion and the vaginal lumen was deviated anteriorly. Normal vaginal wall was seen to extend around the lesion’s anterior and posteroinferior surfaces. The lesion appeared to be arising within the left posterolateral vaginal wall and there were areas of loss of definition of the outer margin of the vagina. There was no involvement of the rectum, urethra or bladder; however, there were hazy low T1 and T2 signal changes in the left paravaginal fat.\nThrough a MDT discussion, it was agreed that owing to the suspicious imaging features of enhancement and tissue inhomogeneity, a staging portal venous phase CT scan was required to look for evidence of metastatic spread. Again, the vaginal lesion demonstrated fluid and soft tissue attenuation areas with regions of enhancement (). Significantly, there was no evidence of distant spread or lymph node enlargement. After further MDT discussion, the mass was still thought to be suspicious for malignancy and the patient underwent surgery.\nThe uterus, ovaries, cervix and upper vagina were removed en bloc and macroscopic examination revealed a well-circumscribed 45 mm polypoid mass arising from the paracervical upper vaginal tissue. The cut surface of the lesion was fleshy grey and white in colour, and was mainly solid in nature.\nMicroscopic examination () revealed an unencapsulated lesion with a spindle cell morphology arranged occasionally in fascicles. Beneath the surface epithelium, there was a grenz zone. The spindle cells were set within finely collagenized stroma and were bland in nature, with no conspicuous mitoses identified. Areas of oedema and myxoid change were also present, with no evidence of haemorrhage or necrosis.\nImmunohistochemistry demonstrated that the lesional cells expressed desmin, vimentin, oestrogen and progesterone receptors. The Ki67 proliferation index was low. Immunohistochemistry for MNF116, alpha smooth muscle antigen, smooth muscle myosin, h-caldesmon, S100 and CD34 were negative in the lesional cells.\nThe morphological and immunohistochemical profile was considered consistent with a superficial cervicovaginal myofibroblastoma, which is also known as superficial myofibroblastoma of the lower female genital tract. |
A 23-year old Caucasian nonsmoking man with no past medical history presented to the ED of Kanta-Häme Central Hospital, Southern Finland, in October 2011 with bilateral numbness of the lower limbs. His walking had deteriorated over a period of 2 months. There was no history of recent trauma or infection, although the patient mentioned hearing an abnormal crack in his left scapular area while lifting a crate in June 2011. There was no family history of note. A symmetrical loss of sensation had risen up to lumbar area, and cold sensation in the lower extremities was absent. Power in the lower limbs was significantly reduced so much so that the patient had trouble rising from bed the day attending hospital.\nOn admission to ED, clinical examination revealed that the patient walked slowly, and there was evidence of atrophy in the left scapula. Power in the lower limbs, particularly on the left side, was significantly reduced, and atrophy was also visible in the thighs and calves. The patellar reflexes were exaggerated, especially on the left side. A weak extensor response to the plantar reflex (Babinski sign +/+) was found bilaterally. The upper limbs did not show any loss of power or sensory function. Laboratory tests were normal. Analysis of the cerebrospinal fluid showed only a moderate rise in proteins, 773 mg/L. Thoracic magnetic resonance imaging and computed tomography (CT) showed severe kyphosis with severe spinal stenosis as a result of destruction of thoracic vertebrae III–IV and left ribs IV–VI (Fig ). Radiologically, there were no significant soft-tissue findings.\nThe patient was hospitalized onto an orthopedic ward, and 2 days later underwent spondylodesis and decompression. Posterior spondylodesis was made bilaterally to thoracic vertebrae II–III and VI–VIII and laterally vertebrae IV with monoaxial and polyaxial screws. Operation proceeded with laminectomy to vertebrae IV–V and lateral decompression of medulla. Kyphosis was corrected with chrome-cobalt rods, and the operation was ended with a bone graft from the patient’s left pelvis. Biopsy confirmed the diagnosis of Gorham’s disease. There were no signs of other diseases that affect vertebrae. Postoperatively, the power in the lower limbs recovered and the patient was able to walk normally. The patient returned home 11 days after the operation. Six weeks postoperatively, the patient developed dyspnea, productive cough, and vomiting, and was found to have bilateral pleural effusions (Fig ). First pleural aspiration was exudate; analysis showed proteins 48 g/L, lactic dehydrogenase (LD) 128 U/L, leukocytes 7700 × 106/L, and erythrocytes 137,800 × 106/L. Culture showed no bacteria or tuberculosis. Osteolysis in the pelvic area was identified on CT. Frequent pleural aspirations were performed, and the patient developed postpunctional fever. Macroscopically, pleural fluid showed no typical chylothoracic findings. Steroids and antibiotics were started. The patient was then treated with biphosphonates and interferons, which were paused during radiation therapy. Pleural effusion was reaccumulated, and the patient was malnourished, with body mass index (BMI) decrease from 24.6 to 17.1. Albumin was as low as 22 g/L.\nTwo months after the operation, a thoracic CT showed moderate disease progression in the thoracic vertebrae and profuse pleural effusion. The pleural fluid did not show any pathological or bacterial findings. Biphosphonates, interferons, and radiation therapy were continued. Six months after the operation, pleural effusion proceeded. It was treated with a left chest tube. Pleural fluid was exudate, analysis showed proteins 43 g/L, glucose level was normal, and triglyceride level was negative. Because of re-existing pleural effusion, thoracoabdominal shunt was placed. This time, pleural fluid analysis showed Propionibacterium acnes, and the patient was treated with antibiotics. His symptoms and signs, mainly dyspnea, hypoxia, and hypotension, disappeared. Afterwards, the patient was able to walk normally, and fixation in the thoracic spine was appropriate based on imaging.\nApproximately 3 years after the operation in January 2015, the patient presented to the ED because of back pain after a loud snapping sound in his back. X-ray and CT showed destruction of the fixation materials without worsening of spondylodesis (Fig ). The patient underwent a surgery with repeat spondylodesis (Fig ), and there was no evidence of progression of the Gorham’s disease. Since then, he has not visited the ED because of this disease. |
A 64-year-old male with an insitu pacemaker needed for sick sinus syndrome for 8 years was presented to our institution with persistent fever for 2 months. He had been re-implanted with a dual chamber cardiac pacemaker 6 months ago on the opposite infraclavicular region, following erosion and pus discharge from previous implant site. His blood investigation showed total leucocyte count 4000/dl and platelet count 85,000/dl. Blood and urine culture showed growth of Pseudomonas aeruginosa, which was treated with appropriate intravenous antibiotics based on sensitivity report (amikacin and piperacillin- tazobactam) for 14 days. Ultrasonography examination of thorax showed a 5 mm fluid filled area along the lead in the left infraclavicular region. Transthoracic echo reported both leads in situ and left ventricular ejection fraction of 55-60%. The 18F-FDG PET/CT study showed increased focal tracer uptake along the pacemaker lead in the right atrium suggesting localized infection in the right atrium []. He was planned for surgical removal of the pacemaker and infected lead followed by epicardial pacemaker insertion via median sternotomy under cardiopulmonary bypass (CPB).\nIn the operating room after instituting standard monitors and invasive radial artery monitoring, anesthesia was induced using intravenous administration of fentanyl, titrated doses of propofol. Injection vecuronium was used to achieve muscle relaxation. Tracheal intubation was achieved using flexible fibreoptic bronchoscope in view of limited neck mobility. Pre CPB, TEE confirmed pacemaker leads in the right atrium and right ventricle, with no extraneous mass in either of the chambers, normal valves and biventricular function []. Detailed TEE examination in the modified ascending aortic short axis view and modified bicaval view showed an irregular echogenic mass (0.6 cm) around the lead in the superior vena cava (SVC). The SVC was narrowed (maximum diameter 1.4 cm). Colour flow Doppler examination showed turbulent flow pattern and continuous wave Doppler demonstrated increased flow velocity in the SVC [Figure and ; Videos and ].\nThe surgery proceeded with exploration of bilateral infraclavicular pacemaker pockets followed by midline sternotomy and placement of temporary epicardial pacing lead. An attempt to cannulate the SVC high up above the lesion for institution of CPB failed. Innominate vein and right subclavian vein were also of inadequate size for cannulation due to severe adhesions. The CPB was instituted with inferior vena cava cannulation and using sucker bypass for SVC return after right atriotomy. Surgery was accomplished using on CPB and beating heart technique. The pacemaker leads were removed from the right atrium. Vegetation was found attached to the pacemaker lead in the SVC, which was removed in piecemeal using traction on the lead. The SVC cannulation could be accomplished only after the removal of leads and vegetation. Pericardial patch augmentation of the SVC was done [Figure and ]. Total CPB time was 244 minutes.\nTermination of CPB was done using combination of epinephrine and norepinephrine. Total urine output during intra operative period was 1300 ml, without any obvious feature of hemolysis. In the postoperative period patient developed vasoplegic syndrome and sepsis. He succumbed to the resulting multiple organ dysfunction syndrome on the 3rd post-operative day. |
A Turkish 13-year-old boy was referred to our clinic fifteen days after a car accident. Clinically, the cooperation level of the child with the dentist was high, but oral hygiene was poor. After assessing medical history, the patient was examined for extraoral signs. He had no facial swelling, facial asymmetry, limited mouth opening, and pain on palpation. Intraoral examination revealed severe intrusive luxation in tooth 12 and moderate intrusive luxation in tooth 11. The tooth 11 was not mobile and did not interfere with the occlusion (). The patient was in the mixed dentition, had a deep bite, and tooth 13 was still erupting.\nAt the first appointment, pulp sensitivity of all anterior teeth was evaluated with an electric pulp test. Teeth 21 and 22 gave positive responses, while teeth 11 and 13 gave negative responses. Radiographs confirmed a complete root formation in all permanent anterior teeth, and periodontal space was absent (). Given that multiple severe luxation traumas, it was decided to perform surgical re-positioning. A photograph was requested from the family to see the anterior teeth positions. The patient and his parents were informed about treatment options and the expected prognosis of each procedure.\nThe difficulties encountered during surgery were i) reaching stability after re-positioning in adjacents multiple luxation teeth on the arch; ii) re-positioning of intruded teeth 11 and 12 in the alveolar socket due to the start of remodeling in the bone in 15 days. The teeth 11 and 12 were surgically extruded and stabilized in a position similar to the symmetrical teeth by 0.5 mm stainless steel splint (). Because the tooth 13 continued erupting, it was left to the spontaneous eruption and not included in the splint.\nAfter surgery, Augmentin (625 mg, 2*1), a penicillin-derived antibiotic, a mouthwash containing chlorhexidine, Parol, an analgesic agent, were prescribed for 7 days to the patient against the risk of pain and infection. Eating habits and vigorous oral hygiene maintenance were instructed to the patient and his parents.\nOne week after the surgery, the root canal treatment for teeth 11 and 12 was initiated, the pulp tissue was removed and was irrigated with 2.5% sodium hypochloride (Cerkamed). The root canal was filled with calcium hydroxide for 15 days. The root canal treatment of teeth 11 and 12 of the patient was completed without removal of the splint after four weeks (). Tooth 13 was also scheduled for root canal treatment because it was non-vital. In the first month after surgical reposition, the patient was called for clinical examinations at first, second and fourth weeks. Clinical and radiographic controls of the treatment condition were examined in months 3, 6, 9 and 12. In control sessions, clinically, there were no clinical symptoms such as mobility, sensitivity, periodontal bleeding, periodontal pocket, and colour change. The radiographic examination revealed the absence of a periapical lesion and inflammatory root resorption of the treated teeth (Figs. , ). |
A 10-year-old girl had reported to our hospital with the chief complaint of pain in her upper front tooth region. History revealed that the patient had traumatic fall 2 years before, leading to fracture and mild mobility of maxillary right central incisor, but no treatment was taken. Clinical examination of the patient revealed extruded and laterally displaced 11 with fracture involving enamel and dentin of the mesial incisal edge. The tooth showed normal mobility, discoloration, and no sign of vitality for electric pulp test. Radiographic examination (intraoral periapical radiograph) revealed fracture in the apical third of the root with open apex and associated periapical radiolucency [].\nTreatment option of either root canal treatment followed by peripaical surgery, root resection and root end filling or repair of root fracture and apexification using MTA was explained to patient's parent. As the parents were not willing for surgery, we opted for the apexification and repair of root fracture using MTA. After achieving adequate local anesthesia, access cavity was prepared and working length was determined. The canal was instrumented 2 mm short of radiographic apex using endodontic files with a gentle circumferential filing motion. This is done to avoid overinstrumentation of the fragile dentinal wall near the open apex and to prevent displacement of the apical root fragment and injury to the periapical tissues. An intracanal medicament of calcium hydroxide was placed in the canal, and the access cavity was sealed with temporary filling material for 2 weeks.\nIn the next visit, root canal was cleaned off the intracanal medicament with saline irrigation and dried with paper points. MTA (white MTA-Angelus, Brazil) was mixed according to manufacturer's instruction and placed in the Canal using MTA Endo Carrier (Dentsply) and condensed using endodontic plugger to the apical end. Radiovisiography (RVG) was taken to confirm the correct placement of MTA and the apical seal []. MTA was added and condensed into the root canal to create 4–5 mm apical plug []. A moist cotton pellet using sterile water was placed over the material, followed by a dry cotton pellet, and the access cavity was sealed with intermediate restorative material.\nAfter 24 h, the set MTA was assessed and final obturation of the root canal was done with gutta-percha using lateral condensation technique and access cavity was sealed with Glass-ionomer restorative material (GC, Type II glass-ionomer cement). Coronal restoration of the discolored and fractured crown was done with the acrylic jacket crown. The child was recalled at 3-, 6-, 9-, and 12-month periods, and the teeth were assessed clinically and radiographically. The patient was asymptomatic, and the radiographs showed a reduction in the size of periapical radiolucency []. Radiographic follow-up at the 3rd year showed complete absence of periapical radiolucency in relation to 11 []. Radiographic follow-up at the 5th year revealed the complete healing of the root fracture with calcified tissue and normal periodontal space around the MTA apical seal and the root fragment []. |
A 0-day-old boy with no abnormalities at the time of delivery was referred to the neonatal intensive care unit (NICU) due to low blood glucose level for 1 hour after birth. His mother underwent regular medical examinations, including ultrasonography, from the 14th week of pregnancy; however, no problems were detected. He was born in the Obstetrics Department by a scheduled Cesarean section at a gestational age of 38 weeks and 3 days and weighed 3534 g (+1.54 SD), Apgar score of 9/10 (1/5 minute) at birth. The hypoglycemia improved immediately after NICU admission with continuous administration of 10% glucose; thereafter, the patient’s course was uneventful without any hypoglycemia or other symptoms. Vital signs were normal at the time of admission (heart rate, 135 bpm; respiratory rate, 54 bpm; blood pressure, 59/37 mmHg). Front chest/abdominal radiography was performed as indicated for screening patients with hypoglycemia without respiratory symptoms. No abnormalities were found from the physical findings, and the breathing sound was normal, so no images were taken from lateral side. The mass shadow that appeared in the right superior lung field seemed reminiscent of the thymus at first glance because the contour and trachea/cardiac shadow were not displaced. However, on further consideration, a silhouette sign that would normally be impossible would be negative if it were a thymus (). Ultrasound was performed from the back, and a solid, uniform, high-echogenicity mass was observed. At the same time, pulsatile blood vessels flowing into the mass were observed; however, visualization was difficult due to the thoracic spine and the origin was not identified (). The next a magnetic resonance imaging (MRI) scan revealed a mass clearly, however even with this, the inflowing blood vessels could not be visualized (). Additional contrast-enhanced computed tomography (CT) examinations identified EPS based on the feeding arteries from the aorta () and the draining veins into azygos vein, the trachea without communication. No complications such as lung hypoplasia and congenital heart malformations were found. The patient gained weight during hospitalization and was discharged without signs of infection or respiratory problems. A follow-up examination will be planned in the future to determine further management. |
A 13-year-old female patient visited our institute complaining of an asymptomatic swelling in the chin region, which she noticed one month back.\nOn extra oral examination, mild facial asymmetry was seen in the lower third of the face. A swelling was observed in the chin region extending superiorly to one cm below the angle of the mouth and inferiorly to two cm below the angle of the mandible with adequate mouth opening.\nIntraoral examination revealed a well circumscribed swelling extending from the distal aspect of 31 to mesial aspect of 33 with normal overlying mucosa. The swelling was soft to firm in consistency and mild tender on palpation. Buccal and lingual vestibules were obliterated and Lower left lateral incisor was clinically missing. Grade II mobility of 31, 33, 41, and 42 was observed. Tongue movements were normal and there was no paresthesia of the mental region.\nPNS view [] showed a well-defined radiolucency extending from 33 to 42 regions, with a thin sclerotic border, associated with unerupted 32; 41 and 42 were displaced and 31 showed root resorption.\nProvisional diagnosis of dentigerous cyst was made and incisional biopsy was done.\nHistopathology revealed a 2-3 layer thick stratified squamous non keratinized epithelium with a fibrous connective tissue wall and few inflammatory cells []. The findings were suggestive of a dentigerous cyst.\nThe cyst was enucleated under general anesthesia and 41, 42, 43, 31, and 33 were extracted. There were no operative complications and the wound healed uneventfully.\nA well encapsulated, soft tissue mass attached to the neck of a tooth was received. The soft tissue mass was grayish in color, soft in consistency and smooth surface with regular borders. Cut section showed a cystic lumen with creamish cauliflower like proliferations from the periphery of the mass into the lumen and the crown of the tooth submerged into the lumen [].\nStratified squamous to cuboidal epithelial lining of 2-3 cells thickness gradually transforming into elongated cuboidal and spindle shaped epithelial cells arranged in whorls was seen [Figures 4–]. Cyst wall showed fibrous connective tissue stroma arranged circumferential to the lumen with few chronic inflammatory cells.\nDeeper sections were made which showed cords, whorls and few duct like patterns of round to ovoid cells in a scanty fibrillar connective tissue stroma with few areas of irregular basophilic calcifications towards the lumen [Figures and ].\nThe findings were suggestive of an AOT arising from the lining of a dentigerous cyst. |
A 19-month-old female child presented to the hospital with complaints of constipation since 6 months of age and difficulty in passing urine for last 6 months. Parents had noticed lower abdominal fullness for last 1 year. The child was born out of non-consanguineous marriage between young parents at full term by an uncomplicated vaginal delivery. Antenatal ultrasound screening had not been performed. There was no history suggestive of birth asphyxia or trauma. The parents gave history of increasing constipation for which laxatives and enemas had been advised but did not provide relief. The child passed small amounts of urine at short intervals.\nThe child weighed 7.2 kg and other anthropometric parameters including head circumference were appropriate for age. Chest examination revealed multiple rib defects on both sides but air entry was normal without any added sounds. Abdominal examination revealed a cystic mass in the suprapubic region arising out of pelvis and reaching up to umbilicus. Digital rectal examination showed normal tone of the normally placed anus but a large cystic mass was felt posteriorly which precluded higher examination. Examination of the spine at the back did not show any bony defects. There was no neurological deficit.\nHematological investigations showed hemoglobin of 11.2 g/dL, TLC of 12,000 per cc and platelets of 117×103 per mm3. Kidney function tests were normal.\nX-ray chest [] showed multiple segmentation anomalies of cervicodorsal vertebrae along with fusion anomalies of ribs on both sides. Echocardiogram did not show any associated cardiac anomaly. Ultrasound of abdomen demonstrated a cystic lesion posterior to urinary bladder measuring 9×4 cm with well-defined dome shaped upper margin and tapering inferiorly. No internal echoes were seen within the lesion. Urinary bladder was pushed anteriorly. There was no evidence of hydroureteronephrosis. Barium enema demonstrated Scimitar sign along with increased presacral space, displacement of the rectum anteriorly and sigmoid colon to the right [Figure and ]. Computed tomography [Figure and ] showed an anterior sacral dysraphic defect with large cystic mass in presacral region displacing the urinary bladder anteriorly and to the right side. Posteriorly, mass was seen entering the spinal canal through large sacral bone defect. There was no hydrocephalus. Both the kidneys were displaced and rotated through their axis but there was no evidence of hydronephrosis.\nAn exploratory laparotomy was performed by a suprapubic transverse incision with intent to excise the meningocele from anterior route. A cystic lesion measuring about 8×4 was seen emerging within the leaves of sigmoid mesocolon from the anterior part of sacrum in the pelvis. The sigmoid colon was pushed to the right while urinary bladder was pushed to the left side []. The sac was dissected till the base. The sac was opened at the apex, clear cerebrospinal fluid (CSF) was aspirated and the sac was excised with watertight closure of the tapering base saving the nerve roots and controlling epidural veins. Closed dural defect was checked for leakage of CSF and hemostasis and then covered with local tissue. CSF was sent for culture and sac was sent for histopathological examination (HPE). The CSF culture was reported as sterile while HPE report showed fibrocollagenous and fibrofatty tissue with few entrapped blood vessels and occasional nerve fibers.\nThere was no neurological deficit in the immediate postoperative period. There was no evidence of constipation, urinary complaints or hydrocephalus at 6 months of follow-up. Although the rib defects have not caused any clinical problem till now, the child remains in follow-up to manage if need arises. |
A 62-year-old male underwent LT for multifocal HCC, combined with hepatitis C related cirrhosis. According to preoperative CT scan images, a massive lesion with greatest extent over 8 cm was found in the right lobe and 2-3 small lesions were found in segment 2. AFP level was elevated at 278 ng/mL. Histological examination of the explanted liver revealed disseminated HCC with numerous nodules in all segments and widespread vessel infiltration. In the posttransplant period the patient received immunosuppressive therapy with tacrolimus, prednisolone, and mycophenolate mofetil (MMF).\nNine months after LT a routine CT scan revealed an extrahepatic, 2 cm lesion adjacent to segment 6 (). Ultrasound guided biopsy verified HCC. The case was determined suitable for laparoscopic resection.\nPneumoperitoneum was established through an open approach via right pararectal region, supplemented by three trocars: a 12 mm through the scar from previous incision and two 5 mm in the right flank. After complete mobilization of the right lobe, the tumor was found retroperitoneally between the right kidney and adrenal gland. Intraoperative ultrasound was carried out to determine the borders of the tumor. Despite close relation with renal capsule, no signs of infiltration to the kidney were revealed. Division of Gerota's fascia and exposing the upper part of the kidney allowed mobilization of the tumor laterally and cranially, separating it from the renal capsule. No evidence of infiltration to adjacent structures was found and the tumor was removed. No significant bleeding occurred during the surgery and the operation time was 131 min.\nHistological findings confirmed the presence of two (40 mm and 12 mm) extrahepatic recurrences from HCC. The postoperative course was uneventful.\nSurveillance for the first 8 months revealed no signs of disease recurrence, but one year after the reresection, metastases in liver, lungs, and adrenal gland were detected. Subsequent therapy with sorafenib was considered as the only possible active treatment. |
An 86-year-old man was admitted to our hospital with a 3-day history of acute abdominal pain. The patient had no previous medical history. A physical examination revealed marked right upper quadrant pain with normal bowel sounds. Murphy’s sign was positive. His vital signs were within the normal range. Abdominal ultrasonography revealed an enlarged gallbladder with surrounding fatty tissue inflammation. The blood biochemistry was essentially normal, including C-reactive protein (1.9 mg/dL) and total bilirubin (1.4 mg/dL) levels. An enhanced computed tomography examination revealed an enlarged gallbladder and incarcerated gallstone. Gallbladder wall enhancement was discontinued, and the fundus of the gallbladder was located centrally beyond the round ligament (Fig. ). The round ligament was attached to the right umbilical portion, which was associated with the anomaly of the intrahepatic portal vein system (Fig. ). Magnetic resonance cholangiopancreatography demonstrated the root of the cystic duct, while the middle portion of the cystic duct was unclear (Fig. ).\nWith the preoperative diagnosis of grade II acute gangrenous cholecystitis according to the 2018 Tokyo guidelines or gallbladder torsion, LC was planned. The first port was inserted into the umbilicus, and an enlarged and reddish gallbladder was observed. The gallbladder was swollen; however, torsion was not detected intraoperatively. The second port was placed in the epigastric area, while others were at the right hypochondriac and right lumbar regions. The gallbladder was attached to the left side of the hepatic round ligament (Fig. ). The cystic duct and the cystic artery were located in the normal positions. Severe inflammation and the narrow working space between the epigastric port and the gallbladder made it difficult to dissect Calot’s triangle; however, the cystic duct and the cystic artery were resected after the critical view of safety was confirmed. Due to the severe inflammation, a subtotal cholecystectomy was finally performed. The operative time was 178 min, and intraoperative blood loss was 50 mL. The pathological diagnosis was acute cholecystitis with a mucosal ulcer. The patient was discharged on the fifth day after surgery without postoperative complications. |
An 11-year-old boy presented with a traumatic injury to the right eye from homemade fireworks. Following initial assessment involving maxillofacial CT and suturing at the Emergency Department, he was transferred to our department for further evaluation because of his poor sight 1 day later. On examination, it was noted that several superficial wounds had been sutured before the child presented to us. His vision was limited to light perception, and the intraocular pressure was 21 mm Hg. A 60% hyphema was present. A skin laceration beneath the right eyebrow was noted, but the superior orbit was not fully visible on the maxillofacial CT performed 1 day previously. Therefore, an orbital CT scan was carried out on the second day, which again showed a hyperdense ring (fig. , arrow); this magnetic metal ring, measuring 18 × 12 mm and embedded in the superior border of the orbital wall, was surgically removed (fig. ). On postoperative day 7, the blood clot in the anterior chamber had decreased in size, and a 1-mm-layered hyphema was present. The patient's visual acuity had improved to 20/400. A fundus examination revealed resolving vitreous hemorrhage, retinal hemorrhage (fig. , short arrow), blunt traumatic retinal detachment superior to the macula (fig. , long arrow), and a large retinal tear parallel to the corneoscleral limbus located in the nasal superior fundus quadrant. The patient and his parents refused to take surgery for retinal detachment into consideration. Therefore, we opted for starting on oral steroids for 1 month and careful observation. After 2 months' observation, discrete areas of retinal pigment epithelial hypertrophy and atrophy, retinal hemorrhage, and epiretinal membrane formation (fig. , short arrow) were seen in the right eye. The large retinal tear had healed and white fibrous scar tissue had developed (fig. , long arrow), and the retinal detachment superior to the macula had reattached itself spontaneously. His vision had further improved to 20/200. During 1 year of follow-up, he remained clinically stable. |
A 7-month-old girl presented to the pediatric outpatient clinic with developmental delay and abnormal eye movements. Abnormal eye movements were noted shortly after birth and involved episodic deviation to lateral extremes of gaze, usually alternating and lasting for a few seconds. The movements were not accompanied by any change in color and activity and were present throughout the day. In between these movements, the child was unable to fixate and follow objects visually. Parents also noticed that the child was not able to keep up with developmental milestones. She had social smile at 3 months and head control at 5 months of age and was unable to sit even with support.\nThere was no history of seizure, abnormal breathing pattern, feeding or swallowing difficulty. She was born at term to non-consanguineous parents and suffered no significant perinatal asphyxia. She was the only child of her parents.\nOn examination, she appeared awake, alert, only inconsistently focusing visually. Intermittent movements of eyes to extremes of gaze were noted throughout the examination. She interacted with her parents and had social smile. She had no neurocutaneous markers. Ocular examination was normal. She showed mild facial dysmorphism in the form of forehead prominence, deep-set eyes, bilateral epicanthic folds and low frontal hairline. There was no organomegaly. Heart and lungs were normal on auscultation. Neurological examination revealed normal cranial nerves and fundus. Motor examination revealed hypotonia with normal tendon reflexes. Head circumference was normal for age.\nThe axial T1-weighted and T2-weighted [] Magnetic resonance (MR) images showed abnormally oriented and thickened superior cerebellar peduncles that resulted in a molar tooth configuration. The more caudal T2- and T1-weighted axial MR images [] showed the fourth ventricle shaped like a bat wing. Furthermore, T2-weighted axial MR images showed hypoplasia of the vermis which resulted in median approach of the two cerebellar hemispheres but without evidence of a posterior fossa cyst []. Based on clinical and magnetic resonance imaging (MRI) findings, diagnosis of JS was made and parents were counseled. Follow-up at 9 months of age revealed that she is able to sit without support and has no truncal ataxia or titubation. Hypotonia has diminished. |
A 64-year-old Caucasian male with a medical history of hypertension and symptomatic bradycardia status-post pacemaker implantation presented to the emergency department with the chief complaint of swelling of the right upper extremity for three weeks. It progressed to a generalized swelling of the right shoulder and right side of the neck, restricting his arm movements. He also voiced the concern of worsening shortness of breath which started with the swelling. He did not report any personal or family history of thrombophilia. There was no previous history of any excessive upper extremity exertion or catheterization in the neck. Vital signs on presentation were a blood pressure of 127/61 mmHg, pulse rate of 79/minute, good volume, regular rhythm with no radio-radial or radio-femoral delay, respiratory rate of 19/min with a saturation of 93% on 3-liters nasal cannula, and temperature of 97.5oF. Physical examination revealed right upper extremity swelling without any sensory or motor deficits, right-sided neck swelling, and right-sided facial plethora. Examination of the contralateral arm, as well as the cardiovascular and respiratory systems, was normal. Biochemical investigations were within normal limits. The patient underwent a right upper extremity duplex ultrasonography which revealed an acute non-occlusive thrombus in the proximal right internal jugular vein (Figure ) and right subclavian vein (Figure ) at the cephalic vein confluence. At this point, Factor V Leiden, anti-thrombin III, protein C, and protein S levels were ordered which were normal. The patient was admitted to the medical ward and an intravenous heparin infusion was initiated. Chest radiography did not reveal any cervical rib, and computed tomography (CT) pulmonary angiography showed no evidence of pulmonary embolism. The swelling improved over the course of two days, and the patient was switched to oral apixaban for anticoagulation. He was discharged on apixaban for six months, and a complete resolution of his signs and symptoms was noted at his three-month follow-up examination. |
A 16-year-old male child presented with history of headache, vomiting, and impaired vision for last 5 months. Headache was moderate to severe in intensity and associated with off and on vomiting. The patient also suffered an episode of generalised tonic clonic (GTC) seizure one month ago. He also had complaints of fever and swelling over right mastoid and peritubal cerebrospinal fluid (CSF) leak along the tract of VA shunt for the last 15 days.\nPatient was in altered sensorium with Glasgow Coma Scale (GCS) = E4 V4 M5. He had no vision in right eye whereas in left eye only hand movements could be perceived. He had bilateral papilloedema. No other neurological deficit detected. There was boggy swelling in the neck with minimal tenderness along the tract. His abdomen showed several scars of previous operations [].\nPatient was operated elsewhere at the age of 7 months in the year 1987 for congenital hydrocephalus. He had presented with increasing size of the head (51.5 cm) with bulging anterior fontanelle. Right VP shunt (Chhabra; Surgiwear India) was performed, which worked well till the age of 3 years. From year 1990 to 1999, VP shunt was revised 6 times for blockage at the abdominal end. In 2000 he had 2 shunt revisions 6 months apart.\nIn 2001 within a small duration of 5 months patient had 6 shunt revisions for shunt malfunction. In all, the shunt tube was placed in almost all quadrants of abdomen. In 2001 patient presented with headache, vomiting and intestine obstruction due to large infected intra abdominal pseudocyst. Laparotomy, adhesionolysis, and exteriorization of shunt tube were done. Later shunt tube was reinserted in suprahepatic space but it was again blocked very soon. After 15 days right ventriculo-atrial shunt was done. This VA shunt worked for nearly 9 years before patient presented to us. In all, he had sixteen operations in his life for hydrocephalus and sequel of shunt.\nThe computed tomography (CT) scan showed marked dilatation of lateral and third ventricles with aqueductal stenosis. Intraventricular shunt tube was visualised within the ventricle [].\nAs the patient had shunt placement at almost every part of the abdomen and VA shunt was also malfunctioning, the option of ETV was considered in him with informed consent.\nThe ETV was performed with standard techniques in February 2010. The CSF was clear under marked pressure. There were some adhesions distal to floor of third ventricle, which were broken with Fogarty catheter. The VA shunt was removed.\nAfter ETV, there was a remarkable improvement in level of consciousness and there was some improvement in vision (6/18) in both eyes as well. Patient was able to do finger counting at a distance of 3 feet one month after the surgery. At a follow-up of six months, there were no signs of any clinical deterioration. The post op CT scan showed presence of CSF in subarachnoid spaces without a significant reduction in size of ventricles. |
A 60-year-old Caucasian woman with no significant past medical history presented to our gastroenterology ambulatory clinic with complaints of chronic lower abdominal pain and diarrhea for the past six months. She had no personal history of malignancy, and her family history was significant only for a brother with prostate cancer. She described her abdominal pain as crampy in nature, exacerbated by food, radiating to the left side and relieved by defecation. She had been having significant diarrhea up to 10-20 episodes daily which were non-bloody and watery. In an attempt to control her bowel movements, she had been taking up to four Imodium tablets daily with some improvement. She also noted a weight loss of 30-35 pounds in the past three months which she attributed to early satiety and the fact that she had been self-restricting her diet to include liquids only. She reported subjective fevers in the evenings and having night sweats over the past six months but denied any nausea or hematochezia.\nLaboratory values were unremarkable without any new electrolyte imbalances, leukocytosis and thrombocytopenia, and her hemoglobin was at her baseline of 10 g/dL. Her carcinoma embryonic antigen (CEA) and carbohydrate antigen (CA 19-9) values were normal. She underwent esophagogastroduodenoscopy (EGD) and colonoscopy which were normal, along with normal duodenal and gastric biopsies. Plans were made to proceed with abdominal imaging and return to clinic afterwards.\nWhile waiting for further investigation, approximately two weeks later, she presented to our hospital with complaints of two days of melena and her hemoglobin was found to be 7.6 g/dL while all her other labs remained unremarkable. On physical exam, her abdomen had become diffusely tender to deep palpation and a firm mobile mass could be felt superior to the umbilical region. There was no obvious cervical or axillary lymphadenopathy. An EGD was repeated and revealed grade II lower esophageal varices with red wale signs, suggestive of recent bleeding, and endoscopic variceal band ligation was done (Figure ).\nA CT scan of the abdomen and pelvis was performed and showed a 7.2 cm x 2.5 cm heterogeneously enhancing soft tissue mesenteric mass with necrosis surrounding the celiac trunk and the superior mesenteric artery. It also revealed an additional extensive tumor thrombus extending from the mesenteric mass to involve the superior mesenteric, splenic and portal veins (Figures , ).\nShe underwent a CT-guided biopsy of this mass which showed high-grade diffuse large B-cell lymphoma (DLBCL) NHL with a Ki-67 of 80%. Morphologically, no granulomas or Reed-Sternberg cells were seen and further immunostaining performed showed positivity for LCA, CD20, PAX-5 (weak and focal), CD10 (diffuse), BCL-2 (diffuse) and BCL-6 (about 30%), while being negative for CD3, CD5, CD20, CD23, cyclin D1, MUM1, c-MYC, AE1/AE3, CAM 5.2, CK7 and CK20, suggesting NHL DLBCL (Figure ).\nA positron emission tomography (PET)-CT was performed and demonstrated an 18 F-fluorodeoxyglucose (FDG) avid mass with surrounding mesenteric lymphadenopathy and no evidence of disease elsewhere (Figure ).\nOur patient was started on R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine and prednisone) chemotherapy immediately. After three cycles of her chemotherapy regimen, her symptoms have subsided without any further evidence of gastrointestinal bleeding. A repeat CT scan showed that her mesenteric lymphadenopathy has begun to shrink, though the PVTT remains unchanged. She has been maintained on propanalol for secondary prophylaxis of esophageal varices and has experienced no further gastrointestinal bleeding. She continues to follow with hematology oncology for the management of NHL. She is also scheduled for a repeat EGD to assess for any remaining varices needing further obliteration. |
A 42-year-old female patient was referred from the Department of Plastic Surgery for the fabrication of ear pressure clip for the left auricular keloid. She complained of swelling on the left ear lobe since 2 years. There was a history of first ear piercing of the lobe of the ear at the age of 12 years without any swelling following piercing. Additional ear piercing was done at age of 38 years, which was 1 cm above the previous site. A very small swelling appeared on the posterior aspect of the pinna of the left ear after 1½ month at the second ear piercing site, which gradually increased in size. The lesion was excised surgically after 1 year. The swelling recurred after 1½ month of the surgery and increased progressively to a size larger than the previous one. Later, surgical excision was done after 1½ year again. Swelling recurred third time at the same site after 1 month of the second surgery and continued to grow until it reached the present size in 1½ year duration [].\nOn examination, a firm, nontender, nonpedunculated, lobulated swelling with a smooth surface and irregular margins, measuring 37 mm superoinferiorly and 35 mm anteroposteriorly was present at the posterior aspect of the pinna of the left ear. There were no accompanying symptoms except it caused severe emotional stress due to impaired esthetics. A clinical diagnosis of keloid was given. A custom made methyl methacrylate pressure appliance was planned to reduce the presurgical size of the keloid.\nPatient's skin over the ear and keloid was lubricated with petroleum jelly, and external auditory meatus was blocked with gauze pack tied with a thread []. Putty rubber base impression material was used to make beading for the confinement of the impression material. Impression was made using light body vinyl polysiloxane impression material (3M ESPE Express, VPS Impression Material, USA) with backing of putty addition silicone impression material [Figures and ]. Completed impression was retrieved after the impression materials were set. The impression was poured in dental stone (Kalstone, Kalabhai Karson Pvt. Ltd., Mumbai, India) with adequate land area to make the diagnostic cast. A modified configuration of the spring was planned using 21gauge stainless steel wire that was used to make a V-shaped loop with a helix at its apex and adapted over the swelling on the cast []. A custom made pressure appliance was fabricated incorporating this loop in clear methyl methacrylate and characterization was done to match the color of the appliance with that of the adjoining skin to make the appliance more esthetically acceptable and unnoticeable to others. Care was taken to ensure adequate space for acrylic resin between the wire loop and the skin to avoid direct contact of stainless steel wire with the skin and a wax spacer was used to provide this adequate space. The design of the appliance involved covering the whole of the surface of the swelling with the gap between the two almost equal halves to permit activation to ensure sustained pressure. With this design, there is no need for fabrication of a new appliance after reduction in size of the keloid as the gap between the two halves permits adjustment by closing the helix.\nThe appliance was finished, polished and adjusted to remove any sore spots []. It was delivered, and the patient was instructed to wear it 24 h a day 7 days a week. She was also instructed about use, maintenance of hygiene and regular follow-up for periodic activation of the appliance. During follow-up, a 2 mm reduction in all over size (Superioinferiorly and anteroposteriorly) of the keloid was achieved after 2 months [] and more 2 mm reduction in superior-inferior dimension at 4 months follow-up []. The adjustment was done in prosthesis by gradual addition of acrylic encompassing the mass without relief. No complication was observed during treatment. |
Patient 1 was a 27-year-old Japanese male (height, 168 cm; weight, 153 kg; BMI, 54) with a complaint of falsetto disorder. He worked as a pop singer and had a history of diabetes and fatty liver. At the time of his visit to our clinic, he had lost his falsetto after overusing his voice in singing practice a month previously. The patient was diagnosed with bilateral vocal fold polyps and underwent microlaryngeal surgery under general anesthesia.\nBefore induction of anesthesia, we conducted a preoperative simulation on the anesthesia table with the cooperation of the patient. The patient himself was asked to lie on the operating table in the operating room where microlaryngeal surgery was performed. Due to the thickness of the chest, it was not possible to set up a stand to hold the laryngoscope. Therefore, we chose to place a towel on the chest and the laryngoscope suspension directly on the towel (Figure ), and we explained the possibility of postoperative chest pain to the patient. The patient was then intubated in the ramp position for induction of general anesthesia (Figure ). Because of the ramp position, all microlaryngeal surgery procedures, which are usually performed in the sitting position, were performed by the surgeon in the standing position (Figure ). After a scalpel incision was made at the base of the bilateral vocal fold polyps, the lesions were removed with forceps. The postoperative course was good, and there was no pain in the trunk region, which was compressed by the base of the laryngoscope holder. The operation time was 11 min and the anesthesia time was 69 min, and no major problems were observed in the vital signs during the operation. The patient is currently under outpatient observation, and the head voice disorder is improving. Maximum phonation time (MPT) improved from 20.0 seconds to 37.5 seconds after one month. |
A 54-year-old female (weight, 46 kg; height, 153 cm), was transferred to Tri-Service General Hospital on account of an unexpected large pancreatic tumor. The clinical history of the patient included paroxysmal headaches, mildly elevated blood pressure (BP), diaphoresis and occasional palpitations. The patient was previously diagnosed with ventricular arrhythmia by cardiovascular departments in numerous hospitals, without any other significant findings. The patient was not administered regular treatment for the headaches or hypertension as the symptoms were considered insignificant. One month prior to surgery, the patient underwent a detailed health checkup and an abdominal mass was identified using abdominal sonography. A large, well-encapsulated pancreatic tail tumor, measuring 9 cm in length, was observed on abdominal computed tomography (). The patient was consequently transferred for surgical intervention.\nOn admission, mildly elevated BP (138–160/80–90 mmHg) with a heart rate (HR) of 70–90 beats per minute (bpm) was noted. The ECG revealed a normal sinus rhythm with two ventricular premature contractions (VPCs). Other laboratory tests showed no significant abnormalities. An exploratory laparotomy with a resection of the tumor was scheduled. Thoracic epidural anesthesia was initially performed without adverse events, followed by general anesthesia. When the pancreas was approached, no any abnormal lesions were identified, with the exception of a bulging mass from the retroperitoneal region. The mass originated from the adrenal gland and presented as a capsulated, vessel-rich tumor. The systolic BP surged to 260 mmHg abruptly with fluctuations and the HR increased to 150 bpm during the manipulation of the tumor. The concentration of the anesthesia was increased along with an additional administration of 100 μg intravenous (i.v.) fentanyl. The fentanyl was ineffective and 5 mg i.v. labetalol was administered twice. However, the hypertensive crisis remained. The surgeon made a temporary stay of surgery until the vital signs were under control and then the tumor was removed.\nThe BP dropped (75/50 mmHg) once the tumor was removed. Aggressive fluid replacement and vasopressors were administered until the patient was hemodynamically stable. The endotracheal tube was then removed. At one day post-surgery, the patient was completely asymptomatic and no sequelae were identified. The pathological report confirmed a diagnosis of pheochromocytoma () and the patient was discharged five days later. |
A 34-year-old systemically healthy Malay woman was referred to the Periodontics Specialist Clinic of the Kulliyyah of Dentistry, International Islamic University Malaysia, with a chief complaint of bleeding gums and mobility of her upper anterior teeth. Complete medical and dental histories were taken, and a full periodontal chart was recorded, including all the clinical periodontal parameters, plaque scores and bleeding on probing (BOP), probing pocket depth (PPD), clinical attachment loss (CAL), recession, mobility, and furcation involvement. The chart revealed grade II mobility of the UCI and pus discharge with deep probing depths and severe CAL >6mm. The three-dimensional picture in Fig. shows the circumferential bone resorption surrounding the UCI. The rapid rate of bone destruction affected the sites of the first and second molars, resulting in furcation involvement and mobility ranging from grade I to grade II with recorded PD and CAL greater than 6mm as well. The radiographic interpretation showed an angular bone defect mesial and/or distal to the first molars (Fig. ). Because the patient was systemically healthy except for the presence of periodontitis, and based on the patient’s history, clinical periodontal records, and radiographic findings, she was diagnosed with localized aggressive periodontitis []. A combination of oral metronidazole 400mg and amoxicillin 500mg were prescribed for 7 days as an adjunct to scaling and root planing (SRP). Six weeks after the NPT, the affected sites were reevaluated. The results revealed slight reductions in the patient’s BOP, PD, and CAL. However, the range of residual bleeding pocket depths was from 5mm to 6mm with no obvious clinical attachment gain. Therefore, the decision was made to provide regenerative therapy, and a surgical treatment was scheduled for the patient in spite of the questionable prognosis that had been assigned at baseline. The patient was informed verbally regarding the details of the surgical treatment plan and signed a written informed consent form before each periodontal surgical procedure as part of the clinical protocol of our institute.\nThe surgical procedure on the UCI included the use of a papilla preservation technique, which was applied to preserve the papilla and obtain primary closure of the inter-dental space (Fig. ). The technique of papilla preservation [] that we applied, compared with the modified technique [], provides better access to the palatal defect because it is applicable on the buccal and palatal aspects. Therefore, the incorporation of papillae with the facial part of the flap (Fig. ) was achieved through crevicular incisions made around each tooth without splitting the inter-dental papilla, and a semi-lunar incision was made across the palatal aspect of at least 5mm from the papillary crest, taking advantage of the keratinized palatal mucosa in protecting the graft during healing. Additionally, this palatal approach overcomes the post-operative labial scar formation in the aesthetic zone.\nAs a result of the intra-bony defect location, which is associated with two adjacent central incisors, significant supra-crestal components of the defect presented at the palatal area (Fig. ). The circumferential intra-bony defects were thoroughly debrided (Fig. ), and cerabone bovine xenograft granules (botiss biomaterials, Zossen, Germany) were used to fill the defects. An OsseoGuard resorbable bovine collagen membrane (Collagen Matrix/BIOMET 3i, Palm Beach Gardens, FL, USA) covered the bone substitute, with delicate trimming of the membrane done to extend inter-proximally (Fig. ) using sling sutures of absorbable material to adapt the membrane well and prevent its collapse over the bone graft. The flap was sutured using an internal horizontal mattress suture, which has the advantage of providing a tension-free flap (Fig. ) and a stable support for the grafting area. Simple interrupted sutures were applied at the rest of the flap sites. The sutures were removed 10 days after the surgical procedure. A fixed retainer using twisted wire (177.8mm; 3M Unitek, Loughborough, UK) was constructed palatally to splint the anterior teeth and stabilize the wound healing (Fig. ). This type of wire allows long-term stability with physiologic tooth movement.\nStrict oral hygiene instructions were given; however, the patient was asked to avoid normal brushing and flossing in the treated area for 4–6 weeks and replace it with the use of 0.12% chlorhexidine mouthwash. The modified Stillman brushing method was recommended to prevent the interference beneath the gingival margin until complete resorption of the membrane was achieved. During the healing process, professional plaque control was scheduled every 2 weeks to prevent bacterial contamination of the surgical area. The patient was also instructed not to chew on the treated area for the first 4 weeks.\nThe application of the graft and barrier membrane resulted in remarkable bone gain and support for more than two-thirds of the root length of the UCI, as shown in the radiograph taken at 2 years (Fig. ), which confirmed the resolution of the defect present at baseline (Fig. ). There was absence of BOP, significant PD reduction up to 2mm, and greater than 5mm attachment gain. Additionally, there was almost an equivalent amount of attachment gain and bone fill. The intra-oral photographs in Fig. show the clinical improvement in the gingival tissue from edematous bleeding gingiva (Fig. ) to the closely adapted, firm, non-bleeding sites (Fig. ). Furthermore, Fig. depicts a good post-operative result without any inter-proximally soft tissue crater formation, which explains the pre-requisite of the papillary preservation technique.\nThree more regenerative periodontal surgeries were performed for regions of teeth 46, 26, 27, and 36. In these procedures, we used different xenograft bone granules of bovine origin (Endobone from Collagen Matrix/BIOMET 3i and Bio-Oss from Geistlich Biomaterials, Wolhusen, Switzerland) and achieved successful results in respect to bone fill (Fig. ) compared with baseline (Fig. ). The patient was covered with systemic antibiotics for all periodontal regenerative surgical procedures. A decision was made to extract the partially erupted and angulated third molars because these teeth were either without antagonists or compromising the adjacent second molars. They also presented difficulties in self-performed plaque control and acted as a local factor for plaque accumulation in deep periodontal pockets. The surgical extractions were performed during the surgical periodontal therapy to minimize the required treatment time and healing process.\nDuring the phase of supportive periodontal therapy, the patient was treated with a schedule of professional plaque control with adjunct use of photodynamic therapy (PDT). The recall system of the maintenance phase was arranged every 3 months. At 2 years after baseline, the full periodontal chart revealed a marked improvement in the clinical periodontal records, as the percentage of sites with BOP had decreased from 40% to 16% and there was a substantial decrease in the number of deep pockets, characterized by the absence of PPD greater than 4mm compared with 22.22% before the commencement of treatment. This PPD reduction was accompanied by an average CAL gain of 3.07mm. |
A 54-year-old previously healthy Caucasian female with otherwise unremarkable past medical history presented to emergency department with one-day history of hematochezia and abdominal pain. The patient described crampy left lower quadrant pain with no aggravating or relieving factors. She had a total of five bowel movements since symptom onset with the first bowel movement containing stool mixed with bright red blood followed by predominantly bloody stools. She took no medications on a regular basis and denied having a screening colonoscopy for colorectal cancer at age 50. She reported symptoms of upper respiratory tract infection (cold, sneeze, and cough) for which she took three doses of 120 mg pseudoephedrine purchased from a local grocery store for 1 day prior to symptom onset. Her maternal grandfather had prostate cancer but there was no significant gastrointestinal tumor history in the family. She was a nonsmoker and reported drinking socially (roughly one standard drink) once a week.\nHer admission vitals were within normal limits. Physical examination was consistent with mild tenderness on the left side of abdomen and hypoactive bowel sounds. Rectal examination showed bright red blood without any stool in the rectal canal. Her laboratory values were significant for mild anemia with hemoglobin of 11.5 mg/dl, hematocrit of 34.5%, erythrocyte sedimentation rate 31 mm/hr, and C-reactive protein 2.15 mg/dl. A computed tomography scan revealed mild to moderate mural thickening of the descending/sigmoid colon consistent with colitis without pericolonic abscess, ascites, or free air (). An infectious workup was obtained including blood cultures, stool cultures, gastrointestinal panel for Clostridium difficile, and gastrointestinal viruses but was negative. She was resuscitated with intravenous fluids.\nThe patient underwent colonoscopy which demonstrated segmental moderate inflammation in the sigmoid colon, descending colon and splenic flexure along with internal and external hemorrhoids. There was evidence of submucosal hemorrhages with mild edema in the aforementioned segments of the colon (). Endoscopic findings were highly suspicious of ischemic colitis. Several biopsies were obtained from the inflamed areas which exhibited focal lamina propria eosinophilic change with mild crypt attenuation and loss of goblet cells consistent with mild ischemic changes. There was no evidence of chronic inflammation.\nShe was observed in the hospital for 3 days and her diet was progressed slowly. Her bloody bowel movements ceased after 1 day in the hospital and patient was counseled and educated regarding avoidance of pseudoephedrine and over the counter medications for symptomatic management. |
An 11-year-old boy had visited a provincial hospital because of frequent epistaxis and nasal obstruction persisting for one year. A tumorous mass was found in the nasopharynx, so computed tomography (CT) and magnetic resonance (MR) imaging studies were performed. Juvenile angiofibroma was suspected, so he was referred to our hospital for further examination and treatment.\nPhysical examination found a smooth reddish mass in the nasopharynx. The imaging studies performed at the previous hospital were reexamined. CT scans showed a homogeneously enhanced soft tissue mass in the nasopharynx without bone destruction (). The T1-weighted MR image with contrast medium demonstrated an isointense mass with homogeneous enhancement (). These findings elucidated the diagnostic impression of juvenile angiofibroma, as suggested by the previous physicians. Angiography detected faint tumor staining, but no obvious feeding artery (). This result suggested the possibility of tumors other than juvenile angiofibroma because of the unexpectedly poor vascularity. Histological examination of the specimen obtained by transnasal biopsy was highly suspicious of malignant neoplasm, such as follicular dendritic cell sarcoma, malignant melanoma, or mesenchymal tumor because of the high cellularity, large nuclei, and prominent nucleoli.\nTransnasal endoscopic extirpation of the tumor was performed under general anesthesia one week after the biopsy. The tumor was a red mass with a smooth surface originating from the posterior end of the nasal septum. The tumor was totally removed en bloc by resection at the posterior edge of the nasal septum. The surgery was completed with little blood loss and no complications.\nHistological examination with routine hematoxylin-eosin staining demonstrated proliferation of spindle cells with enlarged nuclei and infiltration of lymphoplasmacytic cells (Figures and ). Mitotic figures were rarely detected, and no necrosis was identified. Immunoreactivity for vimentin and smooth muscle actin (SMA) was detected in the cytoplasm of the tumor cell, but not for desmin or cytokeratin. This immunoprofile revealed myofibroblastic differentiation of this tumor. Additionally, anaplastic lymphoma kinase (ALK) was weakly expressed in the tumor cells (). These findings confirmed the final diagnosis of IMT.\nThe patient showed no evidence of recurrence at 8 months after surgery. |
A 13-year-old girl diagnosed the previous year with CVID in the setting of pneumonia, low serum immunoglobulin levels, and absent antibody responses to immunizations, presented with worsening dyspnea. Whole exome sequencing did not reveal an underlying genetic explanation for the immune deficiency. A chest CT scan at the time of CVID diagnosis revealed only mediastinal lymphadenopathy. She was placed on monthly IgG replacement therapy and did well until she reported gradual worsening of dyspnea over 3 months. The dyspnea first manifested during competitive sports and progressed to an inability to walk up a single flight of stairs. Pulmonary function testing results () showed a restrictive pattern, and she was unable to complete the maneuvers for diffusing capacity of the lungs for carbon monoxide (DLCO). A follow-up chest CT scan revealed mediastinal and hilar lymphadenopathy, peripheral interlobular septal thickening, peripheral consolidation, and ground glass opacities (). Bronchoalveolar lavage obtained by bronchoscopy did not show any evidence of infection. Specific testing included bacterial, fungal, mycobacterial, and viral cultures along with PCR assays for influenza, respiratory syncytial virus, parainfluenza, human metapneumovirus, adenovirus, cytomegalovirus, Epstein-Barr virus, human herpes virus-8, and Pneumocystis jirovecii. She underwent a right lower lobe wedge resection biopsy via thoracoscopy. The biopsy () showed non-caseating granulomatous inflammation with aggregates of small lymphocytes, scattered multinucleated giant cells, scattered foci of organizing pneumonia, interstitial fibrosis focally in the subpleural space but not prominent or diffuse, and airway luminal compromise from adjacent lymphoid hyperplasia, confirming the diagnosis of GLILD.\nShe was treated with 4 doses of rituximab 375 mg/m2 given 4–6 months apart, based on clinical symptoms and pulmonary function testing, and azathioprine 50 mg daily for 18 months as IgG replacement therapy was continued. She responded well to the new therapy regimen with complete resolution of exercise intolerance and normalization of pulmonary function testing parameters (). She also had remarkable improvement of the CT scan abnormalities () with the follow up CT scan after completion of therapy demonstrating resolution of all abnormalities. |
A 22-month-old male was admitted to our Department of Gastroenterology because of a more than one-year history of abdominal distension with anaemia. His symptoms of abdominal distension was obvious after eating and improved slightly after defecation and flatulence. Meanwhile, the patient’s stool appeared yellow and loose with visible food residue and was voided twice to four times a day.\nIn the past, the patient was once admitted to the local hospital with cervical lymphadenectasis and mild anaemia. At that time, he received oral iron treatment at home, but his abdominal distension showed no remission. The patient’s first admission to our hospital was due to recurrent fever for over half a year in May 2018, and he was diagnosed as bronchopneumonia with pleural effusion and liver and cardiac damage according to the examination results. The patient was born through a full-term natural delivery and showed no abnormalities in the perinatal period. He had repetitive respiratory tract infections, and fever always occurred after vaccination. Perianal abscess resection was performed at the ages of 6 months and 8 months. His parents and sister were healthy.\nPhysical examination indicated that the patient’s weight was 10 kg (z score: − 1.55), and his height was 80 cm (z score: − 2.05). Hardened, enlarged lymph nodes were found on the neck and bilateral sides of the groins. Abdominal distention extended from the xiphoid to the bilateral sides of the groins where the vena epigastrica was exposed. The patient’s abdominal circumference was 50.5 cm. The soft liver and spleen extended 4 cm below the rib cage. We also found that he had two surgical scars because of a perianal abscess.\nThe abdominal CT suggested hepatosplenomegaly, a slightly thickened and strengthened intestinal wall of part of the abdomen (the left abdomen was more obvious), and multiple lymph nodes in the mesenteric, retroperitoneal and bilateral inguinal region (Fig. , in the Supplementary file). The patient’s routine examination results are shown in Table . His bone marrow aspiration revealed that the proliferation of granulocytes, erythroid cells and megakaryocytes was obviously active, and platelet clusters could be seen, ruling out haematological diseases. To determine the cause of abdominal distension, we performed endoscopy and two colonic biopsies were taken from different parts of the intestine, which identified mucous hyperaemia in the membrane with ulcers and erosions from the ileocaecum to the rectum (Fig. ). The pathologic changes are shown in Fig. . However, the patient began to present fever with a temperature peak of 39.5 °C on the third day after endoscopy; treatment with meropenem controlled this condition of fever, but his gastrointestinal symptoms did not improve. Considering that he may have immunodeficiency, we tried the empiric treatment of immunoglobulin replacement. To identify the underlying disease, exome sequencing was performed after obtaining written informed consent from the patient’s parents. A novel frameshift mutation c.888-892delTAAAG (p. Asp296Aspfs*12) in exon 3 of the XIAP gene was identified (Fig. ). This deletion results in a shift in the reading frame and formation of a premature stop codon at the 888–892 position of the DNA strand, which corresponds to the 296-protein chain codon. As a result, peptide breakdown occurs earlier than the normal, which indicates the pathogenicity of this mutation. The patient’s healthy mother and sister were heterozygous carriers (Fig. ). For future monitoring, we recommend that the patient should conduct regular hospital follow-up, recheck the gastroenteroscopy regularly to observe the progression of gastrointestinal inflammation and injury, and histopathological examination will be conducted to keep abreast of the progress of the disease. However, his parents decided to pursue no further therapy (including HSCT) because of the expense, and the patient is currently experiencing recurrent infections again and undergoing follow-up at the outpatient clinic. |
A 38-year-old woman, G2, P2, presented with a 6 month history of metrorrhagia and dyspareunia. Prior to referral, she had undergone a hysteroscopic resection of an 8 cm endocervical mass. Histopathology revealed a poorly-differentiated endocervical adenocarcinoma which was confirmed by immunohistochemical stains. The physical examination was otherwise within normal limits, and the body mass index was 16.4. The patient was staged clinically as IB2 cervical adenocarcinoma. Subsequent positron emission tomographic scan showed a hypermetabolic focus in the left external iliac region.\nThe patient returned to the operating room for planned robotic assisted lymph node dissection. Examination under anesthesia revealed a grossly normal cervix, and a palpable 3 cm left pelvic sidewall mass. A robotic-assisted laparoscopic pelvic and para-aortic lymph node dissection was performed. The palpable 3 cm left external iliac lymph node was identified just distal to the bifurcation of the common iliac artery. The adjacent lymphatics of this enlarged node were first secured with hemoclips and the node was resected using sharp dissection and monopolar electrosurgery. Pathology revealed poorly-differentiated metastatic adenocarcinoma with focal mucinous features. Complete bilateral pelvic and paraaortic lymph node dissection was completed and no additional metastatic sites were identified (1/12). She was discharged home on the same day of surgery.\nOn postoperative day 9, the patient was readmitted to the hospital due to abdominal distention and early satiety. Vital signs were within normal limits. On physical exam, the abdomen was soft and distended with a positive fluid-wave sign. There was mild tenderness on deep palpation but no rebound tenderness. Computed tomography (CT) scan revealed free fluid with several hemoclips in the left pelvic area corresponding to the site of resection of the enlarged lymph node. There was no hydronephrosis and an intact urinary bladder was noticed (). Diagnostic paracentesis was performed and 400 mL of milky-fluid was obtained. Ascitic fluid analysis was significant for lymphocytes of 98%, and triglycerides of 236 mg/dL, confirming the diagnosis of chylous ascites. The patient was initially placed on IV hydration, total parental nutritional (TPN) support and restricted from oral intake (NPO). She was later managed with low fat diet, medium chain free fatty acids, and octreotide treatment. The patient was discharged home on the same treatment and followed with serial imaging studies until the ascites resolved six weeks after the initial diagnosis of chylous ascites.\nAfter resolution of the chylous ascites, the patient completed chemoradiation therapy, during which she developed diarrhea but no grade 3-4 hematologic toxicity. The patient has remained well with no evidence of disease 40 months after the diagnosis of her metastatic IB2 cervical adenocarcinoma (AJCC, IIIB; TNM, T1BN1M0). Recent CT scan of the abdomen and pelvis showed no evidence of disease with only trace amount of pelvic fluid. |
A 45-year-old male referred to Shahid Beheshti Maxillofacial Pathology Department with a two year history of generalized proliferative gingival maxillary lesions with palatal right side swelling and mandibular labially gingival lesions ().\nThe lesions were asymptomatic, without any bleeding or purulent discharge. The right submandibular lymph node was palpable and the patient noticed this swelling after the extraction of the third molar one month prior to his visit to our department. The gingivally lesions were red and soft with irregular surfaces, and the palatal swelling had a purple-gray appearance with intact overlying mucosa ().\nRadiographic examination revealed a moderate bone loss similar to a periodontal disease.\nLaboratory test results were normal, so the patient’s diagnosis was stated as inflammatory and reactive hyperplastic lesions.\nThe patient’s teeth were extracted. Only 2 maxillary central incisors were preserved for esthetic. Tissues needed for histopathologic evaluation were obtained from the gingivectomy of gingivally proliferative masses and with a full thickness flap from the palatal swelling.\nSoft tissue specimens were fixed in 10% neutral buffered formalin and embedded in paraffin blocks. For standard pathological examination, the sections were prepared with H&E stain.\nThe slides of both gingival and palatal specimens showed a dense cellular infiltration under the epithelial layer in deeper portions.\nThe cells were mononuclear which showed pleomorphism with moderately amount of cytoplasm and round to oval nucleus with prominent nucleoli. These cells did not have a characteristic phenotype. Therefore, a series of differential diagnosis such as large cell lymphoma, plasmacytoma, poorly differentiated carcinoma and lymphoblastic leukemia was suggested (-).\nFor the final diagnosis, a panel of antibodies was applied using the Immunohistochemistry (IHC) method. The tumor cells were diffusely positive with antibodies against LCA, negative with CD20, CD3 and CD79a and positive with C-kit. Therefore, according to the IHC results in combination with morphological features, the final diagnosis was made as granulocytic sarcoma.\nThe patient was then referred to the Hematology Department. Bone marrow biopsy and bone marrow aspiration were negative for malignant cells, and the laboratory tests revealed only an increase in the monocyte population.\nThe treatment protocol was 5 courses of induction chemotherapy with cytarabine, idarubici and doxorubicin and adjuvant whole brain radiotherapy.\nDuring the chemotherapy, the patient had neutropenia and thrombocytopenia. Although we tried to manage the complications, the clinical outcome became worse, and the patient died after a heart attack 10 months post diagnosis. |
A 14 year old boy was admitted to the intensive care unit with suspected staphylococcus toxic shock syndrome. He presented with multiple skin abscesses, fever, headache, macular rash and low blood pressure at 102/49. He was treated with intravenous cefazolin and clindamycin. Five minutes after starting his clindamycin infusion, he complained of throat tightening and dyspnea. Physical examination revealed angioedema, conjunctival hyperemia, generalized hives and wheezing. Saturation decreased to 88%. He was immediately treated with epinephrine, diphenhydramine, salbutamol, hydrocortisone, and symptoms were rapidly controlled except for remaining low diastolic blood pressure. Clindamycin was suspected and discontinued. However, he continued to be febrile and the addition of clindamycin to his penicillin treatment was suggested to inhibit the production of exotoxin associated with toxic shock syndrome, as currently recommended. Considering that infectious episodes can simulate drug allergic reactions, that clindamycin allergy is rare, and that he had never received clindamycin in the past to explain sensitization, we decided to perform a graded drug provocation test. After infusion of 380 mg, he developed throat tightness, hand pruritus, dyspnea, wheezing, and his oxygen saturation went down to 84%. Symptoms were rapidly controlled with epinephrine. The patient was subsequently treated with vancomycin and as his clinical evolution was favorable, there was no indication to proceed with clindamycin desensitization. At follow-up 2 months later, skin prick test (SPT) and intradermal tests (IDT) were performed, and both were positive. Undiluted SPT with clindamycin (150 mg/ml)) was positive with an 8 mm wheal diameter and a negative saline control. IDTs were positive at dilutions of 10−5 and 10−3, with respective wheals of 10 mm and 12 mm with surrounding erythema compared to a negative IDT saline control. These dilutions were reported as non-irritating []. Because it was the first time that he received clindamycin antibiotherapy, we asked the parents to look for other sources of exposure to clindamycin. After verification, the mother reported the use of clindamycin gel for acne on one or two occasions in the previous year. Otherwise, this patient was known for previous asthma and peanut allergy during infancy, both of which completely resolved. He also presented a history of AD since childhood, for which he continues to apply daily moisturizing cream, tacrolimus 0.1% ointment and desoximetasone cream to maintain the control of his AD. |
A 37-year-old woman, para 4 presented to the emergency department 3 times over 20 days for sudden onset of heavy vaginal bleeding after a LEEP procedure. The patient underwent a LEEP for CIN3 in a nearby local hospital. There was slightly more bleeding than expected during the procedure, however routine haemostatic measures sufficed. The LEEP specimen was 22 mm in length × 17 mm in diameter. In the days following the procedure, recovery was uneventful with only vaginal spotting.\nThe patient first presented to the emergency department one month after the LEEP procedure with a sudden onset of heavy bleeding and syncope. Her blood pressure was 88/45 mmHg and heart rate 80 beats/min on arrival. The abdomen was soft and non-tender. Speculum examination revealed no active bleeding with an expected appearance of the cervix post LEEP. The patient’s haemoglobin level was 9.2 g/dL and was transfused one pack of red blood cells (RBCs). Her urine pregnancy test was negative and coagulation results were also unremarkable. As her bleeding had ceased, she was discharged from the hospital 2 days later with a haemoglobin of 12 g/dL. The patient was commenced on tranexamic acid.\nThe patient then represented one week later with another sudden onset of heavy bleeding and hypotension. Her blood pressure was 75/40 mmHg and heart rate 80 beats/min. Similarly, on examination, her abdomen was soft, speculum revealed no further active bleeding with a normal cervix which was fully epithelialised. Her haemoglobin was 7.5 g/dL and coagulation screen was normal. The patient was transfused 2 packs of RBCs with an initial diagnosis of abnormal uterine bleeding and was counseled on the need for further work-up in the outpatient clinic. She was given one dose of intramuscular progesterone, then continued on oral norethisterone and tranexamic acid on discharge.\nFour days later, she returned the third time to the emergency department with another episode of sudden onset of massive bleeding per vaginum with hypotension and syncope. The patient’s blood pressure on arrival was 80/60 mmHg and heart rate was 100 beats/min. Her haemoglobin was 7.2 g/dL and transfusion of RBCs was commenced once more. Examination was unremarkable with no further bleeding once the patient arrived.\nIn view of the repeated episodes of sudden onset of heavy vaginal bleeding, a vascular malformation was suspected. A pelvic ultrasound was initially performed which showed a normal uterus with a thin endometrium of 3.3 mm. Ovaries were normal and torturous dilated vasculature were seen over the cervix, appearing more prominent over the paracervical region. Decision was made for a CT Angiogram as the pelvic ultrasound was non diagnostic. A CT Angiogram was then performed which revealed a 0.5 cm focal arterial hyperdensity at the utero-cervical junction which was suspicious for a pseudoaneurysm (Fig. ). The patient then underwent a transcatheter angiogram which confirmed the presence of the right uterine artery pseudoaneurysm. It was not bleeding on initial angiograph. However, during the angiogram there was inadvertent rupture of the pseudoaneurysm with gentle passage of a microguidewire, confirming the diagnosis that this was the cause of repeated bleeding (Figs. , ). Embolisation was then performed using a combination of Gelfoam® slurry and also a 0.3 cc of a liquid embolic Phil™ 25. Post-embolisation angiograms showed successful embolization of the pseudoaneurysm (Fig. ). The left internal iliac and the left uterine artery were also cannulated and angiograms were performed, demonstrating no supply to the pseudoaneurysm. The patient tolerated the procedure well with minimal pain. She was observed inpatient for complications and subsequently discharged from the hospital two days after the embolization without incidents. There were no further episodes of vaginal bleeding thereafter.\nA follow-up CT angiogram was also performed a month later which showed successful treatment of the pseudoaneurysm with no evidence of recurrence (Fig. ). |
The third patient was a 62-year-old male with multiple medical problems included HCV genotype 1a, coronary artery disease, hypertension and type 2 diabetes mellitus. He developed liver cirrhosis and HCC secondary to HCV infection. With exceptional MELD points granted for HCC the patient received a liver from a brain-dead donor. Prior to transplantation, the patient failed treatment with pegylated interferon and ribavirin.\nAfter two months of transplantation, the patient had worsening jaundice and was frequently complaining of abdominal pain. AST and ALT were elevated and serum TB was reached 22.1 mg/dL. The patient underwent ERCP that showed a focal biliary stricture in the post-transplantation anastomosis. A liver biopsy at this time demonstrated focal mild portal and lobular inflammation with mild ductular reactive without changes of cellular rejection. Additionally there was no definite hepatocellular injury or fibrosis. Biliary sphincterotomy with dilatation and stenting of the anastomotic stricture were performed but did not alleviate patient’s symptoms. He continued to have a worsening jaundice despite a repeated ERCP and successfully replacing the stent. A liver biopsy performed four months post-OLT revealed diffuse hepatocyte swelling with associated hepatocyte injury, and no evidence of endotheliitis. The trichrome stain confirmed the presence of periportal and perilobular fibrosis with rare fibrous bridge. These features were consistent with fibrosing cholestatic hepatitis C. His HCV viral load was >69,000,000 IU/mL.\nTreatment for HCV was started with SOF-SMV. HCV viral load decreased to 3200 IU/mL at week two and became undetectable at week six of the treatment. His liver biochemistries improved markedly at week six of the treatment. However, the patient developed recurrent abdominal pain, nausea and vomiting that required two ER visits. Liver ultrasonography was concerning for hepatic artery stenosis. Hepatic angiogram showed marked stenosis suggesting transplanted hepatic artery thrombosis. Paracentesis was performed for new ascites and fluid analysis showed severe leukocytosis and cultures later grew E. coli. He went into respiratory failure, required endotracheal intubation and stayed in the ICU for three days. He died of sepsis and multiorgan failure, which complicated his ICU stay. |
A 62 year-old male had displayed bradykinesia and tremor of his right limbs for one year, during which he was able to perform limited fine movements such as dressing himself, lacing up his shoes and brushing his teeth. His tremors were aggravated by nervousness and relieved when asleep. He had had a history of hypertension and took a daily dose of 5 mg amlodipine. The patient had no history of any other chronic illnesses and was not on any other type of medication. Neither the electrocardiogram nor the Holter monitor showed any abnormalities. His baseline recumbent-upright blood pressure (BP) and heart rate (HR) were normal prior to treatment with piribedil, as shown in Table . He was diagnosed with PD based on the Movement Disorder Society clinical diagnostic criteria []. Initially, he received dopamine replacement therapy of 50 mg piribedil per day. Although there was no significant improvement in symptoms neither did he feel any discomfort. Therefore, starting the first dose change of piribedil, he added extra 50 mg to his dose. About two hours later after the first change in dose, the patient experienced symptoms of dizziness and sweating; he collapsed half an hour later. Whilst in a sitting position, the patient’s BP and HR were measured immediately. The BP reading was 85/48 mmHg and HR was 45 beats/min. His symptoms continued for the duration of the day with sitting BP fluctuating between 80–95 mmHg to 45–68 mmHg. Because his head computerized tomography examination found no abnormalities, the patient received 500 mL of 0.9% sodium chloride solution, after which his symptoms improved. Due to adverse drug reactions (ADRs), the patient was started on a second dose change of piribedil, i.e. an extra dose of 25 mg piribedil was to be taken in the afternoon in addition to the existing 50 mg taken in the morning. After two and a half hours, the patient experienced dizziness, sweating, nausea and vomiting, with a BP reading of 70/45 mmHg and a HR of 47 beats/min when sitting. His BP recovered to 105/65 mmHg and HR to 60 beats/min after elevating his lower limbs and resting for 20 mins. As a result of these incidences, the patient was switched to pramipexole to replace piribedil. After taking pramipexole 0.125 mg or 0.25 mg three times a day (tid), the symptoms of hypotension and bradycardia disappeared but a reduced amplitude of his right arm swing was still observed. Finally, after the pramipexole dose was increased to 0.375 mg tid, the patient showed marked improvements in motor symptoms. The changes in BP and HR are shown in Fig. . |
A 37-year-old Caucasian male with a known history of aplastic anemia (AA), presented to a rural hospital after a ground level fall. AA was diagnosed 10 months earlier after he was investigated for pancytopenia. A bone marrow biopsy showed cellularity of only 10% and the presence of a small paroxysmal nocturnal hemoglobinuria clone (less than 0.2%). He received standard combination treatment for AA with cyclosporine 225 mg orally twice daily, horse anti-thymocyte globulin (ATG) 40 mg/kg daily for 4 consecutive days, and prednisone 1 mg/kg daily. His other medications included daily Pantoloc 40 mg orally, daily Valtrex 500 mg orally, and daily Dapsone 50 mg orally for Pneumocystis jirovecii prophylaxis due to a reported allergy to trimethoprim/sulfamethoxazole. He had recently quit smoking and denied alcohol use but actively used other recreational drugs, including marijuana, cocaine, and methamphetamine. He was unemployed. He had no known other medical co-morbidities and was taking no other medications prior to developing AA. The etiology of AA was felt to be idiopathic because he had no improvement after an initial trial of sobriety. AA improved following immunosuppressive therapy and, although human leukocyte antigen typing was performed, a subsequent bone marrow transplant was deferred not only because of the medical therapeutic response but also due to his ongoing recreational drug use. Although he was no longer transfusion dependent a month after starting immunosuppressive therapy, his treatment compliance waned overtime due to regular ongoing recreational drug use of cocaine and methamphetamines. He routinely used unsterilized tap water for illicit drug injections, but he denied other exposure to fresh or salt water sources at home or in the community.\nOn presentation to the emergency department he was not in distress, with a heart rate of 90 bpm and a blood pressure of 116/59. Severe pallor was noted upon examination, as well as a petechial rash and mild ecchymoses (Fig. ). The rest of his physical assessment was normal, including a neurological examination. Admission bloodwork revealed severe pancytopenia with hemoglobin of 22 g/L, a platelet count of 1 × 109/L, a white blood cell count of 3.7 × 109/L, and an absolute neutrophil count of 0.2 × 109/L (reticulocytes were not sent at admission, but 2 weeks into his hospitalization his absolute reticulocyte count was 12 × 109/L with a reticulocyte percentage of 0.5). All other admission blood work was normal, including liver function tests (total bilirubin 9 μmol/L (reference < 21 μmol/L), alanine aminotransferase 13 μmol/L (reference < 41 μmol/L), alkaline phosphatase 66 U/L (reference 30–130 U/L)) and renal function tests (creatinine 63 μmol/L (reference 59–104 μmol/L), glomerular filtration rate 120 mL/min (reference < 59 mL/min)). He was stabilized and transferred to a tertiary care center where he was restarted on treatment for relapsed AA with a regimen that included cyclosporine (5 mg/kg/day) and prednisone 30 mg daily in addition to five doses of ATG. He remained transfusion dependent throughout his hospitalization.\nOn day 10 after admission, he developed generalized, mild (3/10), colicky abdominal pain with an associated fever > 38.5 °C. He was started empirically on piperacillin-tazobactam (PTZ) 3.375 gm intravenously every 6 hours. Two sets of blood cultures, each consisting of an anaerobic and aerobic BacT/Alert bottle (bioMérieux, Laval, Quebec), were collected peripherally and from his central line. E. coli grew in each bottle set at 10 and 11 hours, respectively. He then developed watery, non-bloody bowel movements, 3–4 times a day, associated with rectal pain. Real-time PCR for Clostridium difficile A/B toxin on a stool sample was negative. Computerized tomography of the abdomen and pelvis was also unremarkable. Repeat blood cultures were negative at 24 and 48 hours after the initial positive set. He improved dramatically after 7 days of intravenous PTZ and was stepped down to oral ciprofloxacin 500 mg orally twice daily to complete a further 7 days of therapy.\nOn day 19 of admission he developed acute continuous severe (9/10), non-radiating dull rectal pain, associated with a high-grade fever (40.4 °C). Vancomycin 1.5 g intravenously every 12 hours and metronidazole 500 mg orally twice daily were empirically started and ciprofloxacin was continued in the same dosage. Blood cultures that were collected from peripheral venipuncture and a peripherally inserted central catheter line grew A. hydrophila at 11 hours. The peripherally inserted central catheter line was immediately removed the next day (day 20 after admission). The same day he also began to complain of vague, mild, bilateral leg pain. Delayed serum sickness due to recent ATG administration was considered a possible cause for his new symptoms because clinical examination did not show erythema, edema, or deformities on either of his legs. However, sustained bacteremia was diagnosed by recovery of A. hydrophila from repeat blood cultures (i.e., one anaerobic and aerobic bottle set from two peripheral venipunctures) positive after 11 and 16 hours of incubation. Bilateral leg pain steadily worsened in intensity (10/10) over the next 48 hours, and the area of distribution of pain extended to the lateral aspect of the right thigh although physical examination remained unremarkable. Creatinine kinase was increased at 470 U/L (normal range for males, 0–195 U/L). Ultrasound venous Doppler of both legs also showed no evidence of deep venous thrombosis. However, magnetic resonance images of both legs showed extensive bilateral patchy multi-compartment muscular and fascial inflammatory changes highly concerning for NF (Fig. , ).\nUrgent initial surgical debridement was performed that evening. An extensive four-compartment fasciotomy, debridement, and myomectomy were performed on both legs. Extensive ‘dishwater’ purulent material was found in multiple compartments of both legs, including (1) the superficial posterior compartment between the gastrocnemius and soleus muscles, and (2) the lateral deep compartment. There was also clinical evidence of severe muscle necrosis of the tibialis anterior muscles in the anterior compartment of both legs. He was admitted to the Intensive Care Unit post-operatively. After consultation with the Infectious Diseases service and review of the antibiotic susceptibility profile of the previously isolated A. hydrophila strain, antibiotics were changed to meropenem 1000 mg intravenously every 8 hours and clindamycin 600 mg intravenously every 8 hours. High dose intravenous immunoglobulin (2 g/kg) was also given. All prior antibiotics were discontinued.\nGram stain of tissue samples from the right tibialis anterior muscle showed no neutrophils but that gram-negative bacilli were present, and subsequently grew a heavy amount of A. hydrophila. Gram stain and anaerobic culture from the right vastis lateralis muscle also did not show the presence of neutrophils or organisms but grew scant amounts of A. hydrophila. A genus-level identification as Aeromonas was obtained for all isolates from blood and tissue samples by matrix-assisted laser desorption ionization-time of flight (MALDI-TOF) mass spectrometry using a VITEK MS (bioMérieux, Laval, Quebec, Canada); since this technique has an accuracy of identification rate of 80–90% for species-level identification of Aeromonas [], all isolates were also analyzed using in-house bi-directional 16S rRNA gene cycle sequencing of the V1-V3 (approximately first 500 bp), as previously described []. Broth microdilution susceptibility panel testing was performed and interpreted using published guidelines []. All isolates were multidrug resistant to ampicillin, ceftriaxone, ciprofloxacin, and trimethoprim/sulfamethoxazole but susceptible to meropenem and tetracycline. The isolates were confirmed to produce an extended-spectrum β-lactamase (ESBL) using published guidelines and the Mast Disc Test (Mast Group Ltd., Merseyside, UK) []. Production of an AmpC β-lactamase was shown by resistance to cefoxitin disk (30 μg) testing and the Mast Disc test (Mast Group Ltd.).\nTwo additional extensive surgical procedures for removal of necrotic tissue from both legs were undertaken in the next 24 hours. Bilateral above-knee amputations were performed during the last debridement as a life-saving measure because of extensive rapid progression of bilateral leg necrosis, and the patient’s rapid clinical deterioration with severe unremittent hemodynamic instability during the operation. Post-operatively, he required aggressive resuscitation for septic shock in the Intensive Care Unit with intractable hyperkalemia and severe acidosis, and anuric acute kidney failure (creatinine 210 μmol/L; normal range for males, 50–120 μmol/L). Despite all therapeutic interventions, the patient went into cardiac arrest and passed away within 2 hours after the final surgery.\nPost-mortem examination at autopsy revealed findings related to the underlying AA, and evidence of septic shock secondary to extensive bilateral lower limb necrotizing myofasciitis. The bone marrow was markedly hypocellular and there was splenic enlargement at 331 g. The heart was enlarged (536 g). Cardiomegaly was likely a compensatory response to the AA due to the absence of atherosclerotic and hypertensive cardiovascular disease. In keeping with the patient’s severe septic shock, there was marked centrilobular necrosis of the liver, as well as petechial hemorrhages of the skin, heart, pleural surfaces, kidneys, and liver capsule. Histologic examination of skin and muscle from the left thigh showed necrosis of the muscle and deep subcutaneous adipose tissue, admixed with dense collections of gram-negative bacilli (Fig. , ). However, in keeping with the AA, there was notably an absence of an acute inflammatory response. |
The patient is a 23-year-old African American female student, single, and domiciled with family, with no reported prior past psychiatric history and past medical history who was brought to the psychiatric emergency room of a sister hospital by the emergency medical services (EMSs) in the context of suicidal attempt after she swallowed 20 pills of sitagliptin-metformin (Janumet). After the initial primary workup of hypoglycemia and other medical complications, the patient was transferred to the Interfaith Medical Center psychiatric emergency room for further psychiatric evaluation and admission.\nUpon initial evaluation, she reported that she was in her usual state of health until 1 day before presentation when she started feeling overwhelmed and overrun by life and being frustrated with coronavirus quarantine, doing the same thing over and over for months, tired of getting up every day, eating and sitting at home. The patient reported low energy, hopelessness about her life, poor sleep, sleeping 2 h per night with early morning awakening of 1-month duration. The patient stated that she and her twin sister planned to kill themselves, and both of them took about 20 pills of Janumet. Patient reported excessive worrying since the coronavirus pandemic and stated that life of being in the house with five people, not doing anything, not in a romantic relationship was not worth living. The patient reported smoking marijuana with her twin sister on the day she overdosed on Janumet pills. She identified her main stressors as not being able to go to Manhattan daily as has always been done before the COVID-19 pandemic lockdown in New York City. The patient reported studying Fashion and Arts and missing her school, nature experiences, and city life, which are heavily weighing on her. The patient denied any suicidal ideation or attempt in the past, a family history of suicidality, and any history of psychiatric illness or in the family. She also denied any history of psychological trauma before the pandemic lockdown. Per collateral was obtained from the patient’s mother who reported that the patient was usually outgoing with liking for movie theaters. The mother reported that the patient has been isolated at home and stressed-out due to coronavirus. She corroborated the patient’s story of no previous suicidal attempt, psychiatric illness, history of childhood trauma, a complication in pregnancy or childbirth, and no family history of mental illness.\nOn initial investigations, the COVID polymerase chain reaction (PCR) test was negative, and the chest X-ray finding was clear lung fields with no infiltrates. Electrocardiogram (EKG) showed normal sinus rhythm with QTc as 431 ms. Urine toxicology was positive for cannabinoids and thyroid-stimulating hormone (TSH) was 6.750 µIU/mL. The potassium level was 3.4 mmol/L during admission which was below the expected range, it was supplemented and a repeat 9 days after was 4.5 mmol/L. There were no other pertinent findings as complete blood count, complete metabolic panel, urinalysis, and daily blood glucose checks were all normal. She was started on Zoloft 50 mg daily for depression and trazodone 50 mg HS for sleep. Patient was placed on 1:1 constant observation for being actively suicidal and safety reasons.\nOn the first day in the inpatient unit, she was agitated, demanding to be discharged and exhibiting poor impulse control, insight, and impaired judgment with maladaptive coping skills. Patient refused to take Zoloft medication stating she was not depressed. On the second day, the patient continued to be agitated, demanding to be discharged and exhibiting poor impulse control/insight and impaired judgment. The patient was offered group and individual therapy; however, she refused. On day 3, she took Zoloft 50 mg and complained of nausea. The dose of Zoloft was reduced to 25 mg due to gastrointestinal (GI) side effects. From day 4 to day 6, the patient continued to refuse medication and remained minimally cooperative with the treatment team, and continued to exhibit poor impulse, insight, and judgment. She continued to request for discharge stating that she was not depressed but was only overwhelmed by the COVID-19 pandemic situation and did not need medication. On day 7, the patient became minimally cooperative, became more engaged with the treatment team, and started coming for individual and group therapy sessions. Milieu and art therapy were provided in the unit. During her individual-supportive therapy, she was taught the basic coping skills to deal with difficult life stressors. Patient was observed to be engaging with peers, participated in individual-supportive therapy, and identifying healthy coping strategies. She was observed to be well engaged in art therapy sessions, and participated in coloring, drawing, writing, and playing puzzles. Her impulse control and judgment became fair and she continued to engage with the treatment team. Based on her clinical improvement, the treatment team decided to discontinue 1:1 constant observation. On days 8 and 9, the patient continued to show improvement in her behavior, impulse control, verbalized understanding of coping strategies she needs during stressful situations. By day 10, she showed good impulse control, well behaved on the unit, and exhibited good insight and judgment. The patient denied suicidal ideation, intent, or plan and was regretful and remorseful of her actions. She was discharged after the treatment team ascertained that she was no longer a danger to herself. Before her discharge, the treatment team had a family meeting with her mother, and psychoeducation was provided on the importance of compliance with aftercare appointments. The patient’s diagnosis was revised from major depressive disorder to adjustment disorder with mixed anxiety and depressed mood. She was discharged home with outpatient treatment plans. The patient was evaluated by the medical team daily throughout her 10 days of inpatient hospital course. The patient was supplemented with potassium due to low level on admission and she was referred to the outpatient endocrine clinic for subclinical hypothyroidism and repeat of TSH and free T4 within 2 weeks of discharge. |
A 13-year-old female patient reported to the Department of Pedodontics with chief complaint of pain in mandibular right posterior region. Patient's medical and family history were inconclusive. Intraoral examination revealed the presence of occlusal caries on mandibular primary right second molar. The radiographic examination revealed the presence of occlusal caries involving enamel, dentin and approaching pulp Figures and . There were multiple small teeth like radioopaque structures at the apex of distal root of mandibular right primary second molar. They were surrounded by a thin radiolucent zone measuring approximately 1.5 × 1.0 cm.\nAn OPG revealed the same findings as those of the intraoral periapical radiograph . Based on the clinical and radiographic examination, a provisional diagnosis of compound odontoma was made. Surgical excision of the lesion by curettage and radiographic follow-up were done.\nAfter achieving adequate local anaesthesia, a mucoperiosteal flap was reflected from the distal surface of the mandibular right primary first molar to the mesial surface of mandibular right permanent first molar on the labial surface. A layer of bone overlying the lesion was removed using a round surgical bur under constant irrigation with saline solution (). The calcified teeth like structures were removed along with the fibrous capsule, without disturbing the unerupted permanent premolar ().\nThe surgical site was curetted and irrigated with povidone iodine-saline solution. After hemostasis was achieved, the flap was approximated and closed primarily with 3.0 silk sutures and postoperative radiograph was taken (). Sutures were removed one week postoperatively. Histopathological examination confirmed the provisional diagnosis of compound odontoma, showing transverse and longitudinally cut dentinal tubules. Sections revealed are of amorphous basophilic masses resembling cementum. Dense fibrocellular connective tissue noted resembling periodontal ligament. Few sections also revealed numerous trabeculae with osteocytes in lacunae (). These features are similar to those of odontomas which comprise varying amount of enamel and pulp tissue, enamel organ, and cementum. Odontogenic epithelium and mesenchymal pulp tissue also may present in some cases. The connective tissue capsule is similar to that of dental follicle. Ghost cells are often seen along with spherical dystrophic calcification, enamel, and sheets of dysplastic dentin [–].\nSix months postoperatively, OPG was advised to check for the eruption of second permanent premolar () and revealed a continuous eruption of the tooth into the oral cavity. |
The patient was a 75-year-old right-handed female former shop owner with 5 years of formal education and a negative family history for neurodegenerative disease. She had sought our attention after a 4-year history of slowly progressive impaired articulation and mild forgetfulness that sneakily emerged after mourning for a family member. All previous specialist evaluations had framed her condition as functional and reactive to her sorrow. She had a brain MRI taken at symptom onset showing diffuse and nonspecific cerebral atrophy and a carotid ultrasonography that was normal. Moreover, she presented a past history of hypertension and dyslipidemia, for which she was taking medications, and rheumatoid arthritis that was now quiescent. On admission to our ward for an initial diagnostic workup, her speech was slurred, fragmented, and presented with occasional paraphasias while neurologic examination failed to evidence further abnormalities. A brain 18F-FDG PET was taken, documenting moderate hypometabolism of precentral and postcentral gyri and temporal lobes bilaterally, although hypometabolism was more marked on the left hemisphere (Fig. ). After workup, she was discharged with the diagnosis of slowly progressive anarthria and mild nonamnesic cognitive impairment and was referred to our outpatient memory clinic for follow-up. Along the next years, her dysarthria slowly worsened, but no other consistent deficits emerged during repeated clinical examinations or were reported by either caregivers or the patient.\nSeven years into the disease, her MRI profile was markedly asymmetrical between hemispheres with a more severe involvement of the left structures in particular involving the left parietal and temporal lobes (Fig. ) without involving brainstem structures such as the midbrain. In addition, also a second brain 18F-FDG PET showed frontal, parietal, and temporal hypometabolism that was more marked on the left (Fig. ), and a DaTSCAN SPECT evidenced dopaminergic degeneration mostly involving the left putamen. Clinically, her most prominent impairment was speech production, which left her almost mute, and she also presented marked orofacial apraxia. At this stage of the disease, her muscle tone was still normal, no falls were reported either by the patient or the caregivers, and her eye movements showed no abnormalities. However, she now exhibited also a marked reduction of mental flexibility and started to unexplainably flee from home. Nonetheless, she was self-sufficient in daily life activities. Her comprehension was intact, and she could still express herself in written form although her writing was littered with marked phonemic errors mainly in the form of substitutions and omissions with slight notes of agramattism (Fig. ). A lumbar puncture was performed with normal glucose, protein, and cells. CSF beta-amyloid was 655 pg/mL (normal values >500 pg/mL), total tau was 153 pg/mL (normal values <500), and phospho-tau was 28 pg/mL (normal values <61 pg/mL). Genetic testing was performed: GRN, C9ORF72, and TARBDP were all negative for mutations.\nHer last visit at the outpatient clinic was more than 7 years into the disease, and she evidenced extinction upon double touch, notes of limb apraxia, ideomotor slowing, pronounced mental inflexibility, and perseveration. The last time she was assessed, 8 years into the disease, she was at home for she was bedridden due to marked postural instability and asymmetric limb rigidity. The patient had to be fed and helped with daily life activities. Eventually, she developed dysphagia and died of ab ingestis pneumonia. |
A 19-year-old male presented in the emergency department with one episode of melena per day, for one week. It was associated with vomiting, shortness of breath and palpitations. His hemoglobin level on initial complete blood count was 5.80 g/dL, signifying severe anemia according to WHO guidelines []. His lab parameters on admission are presented in Table .\nImmediately packed red blood cells (RBCs) were requested from the blood bank. On forward typing his blood group was labeled as O positive and his serum showed strongly positive indirect Coomb’s test with a negative direct Coomb’s. On extended 11 cell panel antibody testing, his serum demonstrated pan-agglutination which matched with monoclonal panel cells having anti-Kell, anti-Lub, and anti-Kpb antibodies. On cross match with four O negative and four O positive packed RBCs, +4 incompatibility was seen with all. Meanwhile a detailed history of the patient revealed two distinct episodes of epistaxis in childhood and a family history of his paternal grandmother having an increased bleeding tendency. In view of his past history of fresh frozen plasma infusions, it was interpreted that the patient may have multiple alloantibodies in blood leading to gross incompatibility. Considering the urgency of the situation, one unit of the least incompatible (O negative) packed RBCs was issued after washing with normal saline thrice, to the emergency department. Transfusion was started under strict monitoring by the emergency department physicians. After slow transfusion of around 10 ml blood, the patient started shivering and his temperature spiked to 101°F with tachycardia and hypotension. The transfusion was stopped immediately and the patient was given intravenous antihistamine and hydrocortisone. Meanwhile, he was transferred to the intensive care unit (ICU) where he received intranasal desmopressin and intravenous factor VIII.\nTransfusion reaction workup revealed a grade 4+ pan agglutination in his serum. During repeat blood grouping, forward typing did not demonstrate any reaction to anti-A and anti-B antisera, like a normal O blood group. However, on reverse typing, his serum showed strong agglutination with group O pooled control cells. His post saline wash incompatibility with O negative red cell concentrate showed minor difference from grade +4 agglutination (pre-wash) to grade +3 clumping (post-wash). A fresh RBCs sample from the patient showed negative direct Coomb’s test, while fresh serum sample remained positive for indirect Coomb’s test. This workup strongly raised the suspicion of Bombay phenotype and his red cells were tested with anti-H lectin, which showed no agglutination. This confirmed his blood group as Bombay phenotype. The reactions observed with Bombay phenotype compared to other blood groups, on forward and reverse typing, are illustrated in Table .\nImmediately, voluntary donor pools were contacted in blood banks throughout the country. Overnight, a donor with Bombay negative blood group was arranged from Karachi. The packed RBCs were airlifted to Islamabad maintaining the cold chain. After crossmatching with recipient’s blood showed no reaction, the donor blood was transfused to the patient. Meanwhile, a distant relative of the patient from a nearby city, with Bombay positive blood group, consented to donate blood at our blood bank. Two days later, another unit of packed RBCs was transfused to the patient. His hemoglobin after two transfusions rose up to 7.40 g/dL. As his melena settled down on supportive therapy, an endoscopy was performed that suggested an underlying hiatal hernia. After surgical consultation, the patient was advised to reduce weight and discharged from the hospital, with a scheduled follow-up visit.\nIn view of the patient’s past medical history and family history, during the follow-up visit, a von Willebrand factor antigen, von Willebrand factor functional activity and factor VIII levels were ordered. His von Willebrand factor antigen level was <2.0%, von Willebrand factor functional activity was <4.0% and factor VIII level was 18.5%, consistent with type 3 von Willebrand disease. The patient and his family were counselled accordingly and referred to the hematology clinic. |
A 44-year-old woman was admitted for chronic cough syndrome and refractory asthma criteria. The patient had a history of hypertension and morbid obesity with body mass index of 42 despite gastric reduction surgery 11 months earlier by means of sleeve gastrectomy. Since surgery, she had reported chronic cough with 10 recurrent exacerbations of dyspnea and wheezing requiring visits to the emergency services. In one of the visits, pneumonic infiltrates were observed in lung bases; after 12 days, they reappeared at another site and were therefore defined as migratory (see Figs. , ). Blood tests revealed a total IgE of 5.54 kU/L (n = 150 kU/L), and blood eosinophils of 225 (n < 400/mm3). Given the repeated asphyxial episodes, and despite inhaled corticosteroids and high-dose long-acting beta-adrenoceptor agonists, she was readmitted for further study. During her stay, she presented several asthma attacks with declines in peak flow exceeding 20%, and two sudden decreases of 40% and 60%, respectively. During the periods between attacks, the spirometric pattern remained within normal limits. Brittle asthma was diagnosed [].\nUsing laryngeal fibroscopy, the reflux finding score was 8 points (n < 7). Given these results and the lack of improvement with the addition of proton pump inhibitors (40 mg/bid), impedance–pH monitoring was performed. This test detected the existence of a severe mixed acid–nonacid esophageal reflux, with 75% of the events reaching the upper third of the esophagus and a significant increase in nonacid or weakly acid GER episodes. For all refluxes, there was a symptomatic association probability of 100% (n < 95%) and a symptom index of 100% (n < 50%; see Table ).\nGiven the evidence of uncontrolled GER with a clear temporal association between the onset of extraesophageal symptoms and the gastric reduction surgery performed 11 months earlier, the digestive tract was reoperated by open gastric bypass with incisional surgical repair and conversion from sleeve to bypass. A few days after surgery, and at 2 and 6 months after surgery, the patient presented no respiratory symptoms. |
A 47-year-old female patient was referred to our hospital for the management of postoperative bleeding following vaginal hysterectomy done elsewhere for third-degree uterovaginal prolapse. She was conscious but drowsy on examination and tachypneic. Heart rate was 120 beats per minute and blood pressure (BP) of 90/60 mm Hg in supine position. Peripheries were cold and clammy. Laboratory investigations were normal except for low hemoglobin (6.2 g/dl.). She was posted for emergency laparotomy and re-exploration. In view of low blood pressure and the need for inotropic support, a triple lumen central venous access was secured through right subclavian vein. Fluid resuscitation was done with one liter of colloid and two units of packed red blood cells. Exploration was planned under general anesthesia. Before induction, left radial artery was cannulated with 20 ga PVC Jelco® cannula and invasive hemodynamic monitoring was instituted. Surgery involved ligation of bilateral internal iliac arteries because of continuous ooze. Intraoperatively, she received four units of fresh frozen plasma and three more units of packed red blood cells. Inotropic support with dopamine 10 to 15 μg/kg/min was required to maintain a mean BP of 65 mm Hg. Patient was admitted in intensive care unit (ICU) for monitoring and elective ventilation after the procedure. In the ICU, she developed disseminated intravascular coagulopathy on the second day which was managed with blood products and cryoprecipitate. Inotropic support with dopamine up to 20 μg/kg/min was continued. On the third day, there were features of acute respiratory distress syndrome and she required increasing ventilatory support to maintain acceptable oxygenation. Tracheostomy was done on the seventh day and ventilatory support was continued for the next 15 days and gradually weaned off successfully.\nOn the third postoperative day, dusky discoloration of the digits involving both hands were noted. The extremities were cold to feel. In view of unstable hemodynamic status and due to technical difficulties in securing arterial cannulation at alternate site, we deferred decannulation immediately. On the fifth day, the left thumb, index and middle finger showed worsening of discoloration. The left radial artery cannula was removed and vascular surgery opinion was sought then. Doppler ultrasound of the radial artery showed partial thrombosis at the cannulation site, even though the arterial pulsation was well felt. Injection Clexane (enoxaparin), a low molecular weight heparin, was started in a dose of 0.4 ml subcutaneously for seven days and IV low molecular weight Dextran was also started at 40 ml/hr for the next two days. Stellate ganglion block was also attempted on the same side using bupivacaine 0.25% 20 ml. Nitroglycerine ointment was applied for the dusky discoloration in both hands from the time it was noted.\nThe discoloration of the digits involving the right hand improved but the left hand digits, especially the left thumb, index and middle finger, did not improve with the conservative management and was slowly turning dark. Over the next three days, the digits had become blackish in color with clear line of demarcation suggesting dry gangrene []. Patient required pulmonary and psychiatric rehabilitation after recovering from the acute event. After adequate counseling, she underwent amputation of the gangrenous digits under brachial plexus block four weeks later. |
A 27-year-old Caucasian woman was admitted to the Emergency Department of our Institution because of bloody diarrhoea – up to 10 bowel movements per day – during the last month, 3 weeks after quitting smoking. Physical examination showed no abnormalities but confirmed haematochezia on digital rectal examination. Colonoscopy showed continuous severe colonic inflammation with small ulcers from the anus to the descendent colon, classified as grade 3 in Mayo endoscopic sub-score and 3 points in Ulcerative Colitis Endoscopic Index of Severity (UCEIS); complete examination was not performed because of the risk of perforation. Empirical antibiotic treatment with ciprofloxacin and metronidazole, as well as oral and rectal mesalamine were started and partial symptomatic improvement was achieved. Venous thrombosis prophylaxis with subcutaneous enoxaparin, 40 mg per day, was started. At admittance, haemoglobin, white cell count, platelets, fibrinogen and C reactive protein (CRP) were within the reference range. Stool cultures were negative. Cytomegalovirus (CMV) infection was also ruled out in colonic biopsies (polymerase chain reaction – PCR – and, later, immunohistochemistry). As bloody diarrhoea persisted 48 h later, and histopathological examination of colonic biopsies showed crypt distortion, a mixed inflammatory infiltrate of the lamina propria and crypt abscesses suggesting the diagnosis of UC, intravenous methylprednisolone (1 mg per kg of weight, daily) was started. After 3 days of corticosteroids the patient achieved partial clinical response (6 bowel movements per day, Edinburgh index 2 points, CRP within the normal range); nevertheless, 2 weeks later infliximab therapy (5 mg/kg of weight) was started due to sustained clinical activity, with 10 bloody bowel movements per day and a progressive increase of CRP levels, up to 10 mg/dL. Three days after the first dose of infliximab, the patient presented a massive lower bleeding with haemodynamic instability and severe anaemia; CT scan showed active arterial haemorrhage from ascendant colon; a subsequent arteriography demonstrated active arterial bleeding from a colic branch of the superior mesenteric artery; selective transcatheter embolization with platinum microcoils (MicroNester©, Cook Medical) was performed with immediate technical success; nevertheless, the patient persisted with rectal bleeding 2 days after embolization, requiring laparoscopic subtotal colectomy and ileostomy. Pathological evaluation of the colon confirmed the diagnosis of UC. Eight days after surgery the patient was discharged. |
A 59-year-old man was presented in our hospital because of respiratory distress since 3 days before admission, which had been gradually sever. He was admitted with impression of pulmonary edema. He had a history of uncontrolled diabetes mellitus, systemic hypertension and hyperlipidemia.\nOn physical examination, he was blind, with blood pressure of 210/140 mmHg, pulse rate about 150 beat/min and respiratory rate about 40 cycle/min. The pulses were equal in upper limbs. Both femoral pulses were equal but weak. There were diffuse moist rhales in both lungs and cardiac examination systolic ejection sound and murmur was audible in the aortic area and with less severity in apex and lower left sternal border.\nElectrocardiography showed sinus tachycardia with complete left bundle branch block. After several hours of aggressive medical treatment, the patient’s condition became relatively stable.\nEchocardiography showed sever aortic valve stenosis (mean pressure gradient = 60 mmHg) and sever LV systolic dysfunction (LV ejection fraction = 25%).\nAfter initial stabilization with conservative treatment, coronary angiography was done and revealed three vessels coronary artery disease.\nCardiac catheterization from right femoral artery showed occlusion of the distal thoracic aorta to the left subclavian artery and angiography from right brachial artery proved interrupted aorta (type A). We were not able to pass through aortic valve to the left ventricle because of valvular stenosis, and it seems that left internal mammary artery plays role in collateralization but we did not engage through it ().\nThe descending thoracic aorta was supplied by extensive collateral vessels from the vertebrobasilar system down to the posterior chest wall and the spine (). Successful surgical correction including coronary artery bypass graft and aortic valve replacement and repair of interruption of the aorta was performed without any complication. Three weeks later the patient died due to gastrointestinal (GI) bleeding which was not controllable by aggressive treatment and hospital acquired pneumonia. |
A 26-year-old male presented with complaints of the sudden occurrence of slurred speech, weakness on the right half of the face, right upper limb with subsequent involvement of the right lower limb of 4 days duration. Three hours prior to the development of these symptoms he had smoked three wraps of street marijuana. He denied use of other street drugs and alcohol.\nOn examination, he was fully conscious and alert. Blood pressure was 120/70 mmHg and pulse rate 80 beats/minutes, regular with good volume. He had paralysis of right facial nerve upper motor neuron lesion type. There was complete motor paralysis (power 0) on the right upper and lower limbs and loss of sensation to pinprick and temperature. The pupils were normal bilaterally and react normally to light.\nFasting blood sugar was 97 mg/dl. All hematologic parameters were within normal values (full blood count, platelet count and prothrombin time and partial thromboplastin time). The serum chemistry and the lipid profile were also normal. The HIV screening test for type I and II was negative as well as VDRL test. No abnormality was detected on electrocardiogram and echocardiogram. There was no evidence of a patent foramen ovale. Urine toxicological screening on admission was positive for cannabis and negative for amphetamines, cocaine, methadone, opiates.\nA computed tomography (CT) brain scan done 4 days after the onset of symptoms showed left basal ganglia infarct []. He subsequently had a magnetic resonance examination which showed a hypointense lesion in the left corpus striatum and insular cortex on T1W, [] and correspondingly high T2W consistent with infarcts []. Magnetic resonance angiography (MRA) of the intracranial vasculature showed reduced flow in the left internal carotid artery and absence of flow in the left and anterior middle cerebral arteries consistent with his deficit [].\nPart of treatment, the patient received was aspirin therapy, vitamin E and physiotherapy. The patient made minimal improvement in his neurologic deficit and was discharged home after 2 weeks. He has since been lost to follow up, a common problem in developing countries. |
A 5-year-old boy born of nonconsanguineous marriage was brought for recurrent fissuring and ulceration involving both great toes of 2-year duration, associated with palmoplantar hyperhydrosis. There was no associated pain or motor weakness and there was no history of preceding trauma. Family history was negative for similar disorder, diabetes mellitus, and leprosy. The boy had a normal birth and normal cognitive development. Examination revealed an ulcer on the dorsum of right great toe extending on to plantar aspect of size 3 cm × 2 cm, covered with healthy granulation tissue and nontender []. The deep fissure was noted at the base of left toe []. A small ulcer was also noted on the dorsal aspect of the right ring finger. There were hyperpigmented, hyperkeratotic plaques on pressure points, that is, knuckles of both hands []. There was no evidence of ainhum or auto amputation involving fingers and toes. Pain and touch sensations were absent on both feet and hands. Vibration and joint sensations were intact. Peripheral nerves were not thickened and not tender. Motor power was normal and equal on both sides. Peripheral pulses were equal on both sides. Hearing and vision was normal. Biceps and triceps jerks were normal but knee and ankle jerk were absent on both sides. No bladder or bowel disturbance. Based on the clinical features, the child was provisionally diagnosed as HSN. However, leprosy was considered in the differential diagnosis and evaluated. On investigation, he was found to have normal blood chemistry, skiagram of feet and magnetic resonance imaging scan of the spine. Nerve conduction study revealed severe diffuse sensory neuropathy. Slit skin smear for acid fast bacilli was negative. Karyotyping showed normal 46XY pattern. However, specific mutations could not be identified due to nonavailability of specific DNA probes. Sural nerve biopsy could not be done as his parents refused. Serology was negative for HIV. |
A 67-year-old man with a history of chronic obstructive pulmonary disease, cerebral vascular accident, necrotizing pancreatitis complicated by pseudocyst requiring splenectomy and heart failure with preserved ejection fraction was transferred to our hospital following one month of treatment for pneumonia. He was a distant alcoholic but had since gone through rehabilitation and admitted to drinking one time per week and smoking four cigarettes a day. He had previously presented to his primary care physician with fever and malaise and was diagnosed with community-acquired pneumonia. He was treated with five days of azithromycin. He continued to worsen, and was admitted to an outside hospital with hypoxemia and right lower lobe pneumonia for which he was started empirically on vancomycin and piperacillin-tazobactam. His hospital course was complicated by respiratory failure requiring intubation for three days and a recurrent exudative right lung loculated effusion that required decortication and placement of a catheter that remained in place for two weeks. All blood and pleural fluid cultures were negative.\nOn transfer to our hospital for physical rehabilitation, the patient complained of mild shortness of breath. He denied hemoptysis, chest pain, orthopnea, nausea, chills or night sweats. Physical exam was significant for bilateral rhonchi with signs of consolidation in the right lower lobe. His labs were notable for a white blood cell count (WBC) of 17,000 cells/mcl with 87% neutrophils, and a chest radiograph revealed a right middle lobe infiltrate. He was continued on intravenous (IV) vancomycin and piperacillin-tazobactam at admission. Over the next two days his WBC climbed to 21,000 cells/mcl. Computed tomography scan of the chest revealed a right-sided empyema with extensive bilateral airspace disease consistent with severe pneumonia. A new chest tube was placed, which drained dark brown exudative fluid with gram-positive cocci on gram stain. The fluid was cultured and grew E. faecium resistant to ampicillin and vancomycin but sensitive to linezolid, gentamicin and streptomycin. The patient was started on linezolid and improved over the next two weeks, with resolution of the chest tube drainage. |
A 17-year-old man was involved in a road accident in which he suffered the open fractures of the right femur and tibia. At the arrival to the Emergency Dept (ED), he was alert and hemodynamically stable and the Glasgow Coma Scale (GCS) was 15; the initial alignment of the fractured ends was performed in the ED with a gentle traction performed under sedation with iv. ketamine; a total body CT did not demonstrate other injuries. Approximately two hours after the admission the patient was taken to the surgical theatre for the external fixation of the fractured bones; at entering the operating room, the GCS was 8, the arterial pressure was 115/80 mm Hg, the heart rate was 115 bpm, and the arterial oxygen saturation (SPO2) was 85 at room air; the procedure was performed under general iv anesthesia with propofol and remifentanyl; the standard monitoring included the ECG, the noninvasive arterial pressure, the SPO2, and the end-tidal CO2 (ETCO2); during the intervention, the SPO2 rose to 100% at a FIO2=40% and all the other variables remained stable throughout the procedure after the 3-hour-long intervention in which the complete alignment of the bony ends was achieved; the patient was transferred to the Intensive Care Unit (ICU) still intubated and mechanically ventilated; the iv anaesthetics were gradually tapered until the complete suspension. Two hours later, the SpaO2 and the ETCO2 slightly decreased and anisocoria was observed; and an urgent CT scan of the head demonstrated a diffuse cerebral edema and the herniation of the cerebellar tonsils (Figures and , respectively). At this time, the pupils became bilaterally mydriatic and the EEG was almost isoelectric; due to the severity of the conditions, a MR scan was considered unnecessary. On the basis of the clinical and radiologic findings repeated boluses of iv. mannitol and steroids were given in the following hours aiming to reduce the intracranial pressure. An echocardiogram demonstrated a severe right ventricular depression with an ejection fraction of 20%. On the following day, the patient was declared brain dead according to the current Italian law.\nAt the autopsy, the cerebral microvascular network appeared diffusely plugged with BME (Figures –) and ischemia-related microcalcifications were scattered throughout the brain (); other organs were less extensively involved; no PFO was demonstrated. |
A 64-year-old Asian female was presented to our hospital with a recurrence of liver nodular lesions after multiple surgical resections.\nThe patient had received lung lobectomy due to lung cancer in the right upper lobe 11 years ago (). Postoperative pathology showed a 4.0×3.9 × 3.0 cm moderately differentiated adenocarcinoma, with one positive mediastinal lymph node among all the 10 dissected lymph nodes (). She quit adjuvant chemotherapy after one cycle due to intolerance. No elevation of tumor markers had been reported perioperatively.\nSeven years ago, the patient developed jaundice. CT assessment showed a pancreatic head mass with involvement of the distal common bile duct (). Results of tumor markers including CA19-9 were negative. She was then treated with radical pancreaticoduodenectomy, where a 2.5×2.0 × 2.0 cm moderately differentiated adenocarcinoma with negative lymph node metastasis was removed (). Afterward, she received adjuvant S-1 chemotherapy (consisting of tegafur 40 mg, gimeracil 11.6 mg, and oteracil 39.2 mg, administered orally twice a day) for 3 cycles (2 weeks per cycle followed by a one-week rest period), which was also discontinued due to adverse events.\nThe patient was then put on surveillance with an annual imaging assessment. Four years after the Whipple procedure, a follow-up examination showed a solitary liver lesion in segment VII (). She refused interventional treatment or chemotherapy and chose cytoreductive surgery to resect the segment VII lesion. Postoperative pathology demonstrated a 3.0 cm segment VII adenocarcinoma and suggested the diagnosis of liver metastasis ().\nRecently, the patient was admitted for the recurrence of a liver lesion, along with multiple lung nodules (). Recurrence of the previous adenocarcinoma in the liver and lungs was the first impression. The tumor board reviewed her past medical history and proposed the possibility of metastases of the lung adenocarcinoma to the pancreas, liver, and lungs. Liver biopsy confirmed adenocarcinoma, with similar morphology, such as the acinar and tubular structures, to the previous specimens (). Further immunohistochemical studies demonstrated that the lung adenocarcinoma eleven years ago, the pancreatic adenocarcinoma seven years ago, the liver adenocarcinoma three years ago, and current liver adenocarcinoma all shared features of positive thyroid transcription factor-1 (TTF-1, clone SPT24) and Napsin A expressions ( and ), indicating the exact pathological origin of the lung adenocarcinoma.\nWe then tested the adenocarcinoma specimen for mutations of driver genes in lung cancers as well as in the metastatic liver adenocarcinoma, and found an epidermal growth factor receptor (EGFR) exon 19 non-frameshift deletion, which was associated with favorable prognosis, in both samples. The patient was then administrated with gefitinib, a tyrosine kinase inhibitor for lung cancers with EGFR exon 19 mutations. Imaging reassessment one month later indicated a status of partial response, with the lung nodules disappeared and the liver metastasis significantly shrunk. |
A 74-year-old woman was admitted to the hospital with progressive exertional dyspnea in the past few weeks. On admission, echocardiography revealed severe PH with a TR-PG of 100 mmHg. Enhanced CT showed a small defect of fresh thrombus in the bilateral pulmonary arteries at the segmental or subsegmental levels and signs of CTEPH including intimal thickening and abrupt narrowing and occlusion in the bilateral pulmonary artery (). Laboratory investigations showed a D-dimer level of 9.1 μg/mL. Although anticoagulation therapy with heparinization was initiated, her condition rapidly deteriorated, leading to severe hypoxemia and hypotension. On the day after admission, ECMO was initiated. She was managed with heparinization and was attempted to be weaned off ECMO. Four days after the initiation of ECMO, mPAP was still 55 mmHg and it was impossible to wean off ECMO without any additional interventions. Therefore, we decided to perform rescue BPA. Pulmonary angiography revealed only a small fresh bloody thrombus in the right A1 and left A9 and chronic thromboembolic lesions of webs, subtotal occlusion, and total occlusion in the bilateral pulmonary arteries at segmental levels (). We performed BPA in the right (A3, A5, A9, A10) and left (A8, A9, A10) pulmonary arteries (). Immediately after BPA, her condition stabilized; however, hemoptysis was observed 1 hour later, requiring frequent tracheal suction. Small-vessel injury was suspected despite the careful wiring approach and ballooning, and bleeding was difficult to manage for 2 days. After hemostasis, her cardiorespiratory status improved and ECMO was successfully discontinued 2 days after rescue BPA. The results of RHC performed 16 days after BPA were as follows: mean PAP 35 mmHg and PVR 7.7 wood units. She was discharged with anticoagulation therapy 1 month after rescue BPA. After an additional four sessions of BPA, her hemodynamics showed further improvement with a mean PAP of 18 mmHg and World Health Organization functional class I. |
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