Patient AM is a Caucasian female born at a community hospital by cesarean section to a 20-year-old gravida 1 para 0 mother of Western European descent. The mother had regular prenatal care, took prenatal vitamins and did not use alcohol, tobacco, or illicit drugs during the pregnancy. During prenatal visits, the fetus had reassuring vitals so an ultrasound was not obtained. The pregnancy was complicated by gestational diabetes and preeclampsia for which the mother had been placed on bedrest during the final trimester. At 42 weeks gestation, the mother was induced, but during labor, fetal distress was noted, requiring an emergency cesarean section to be performed. Birth weight was 7 lbs 4 oz. (3.29 kg) and length was 17″ (43.18 cm). After birth, our patient failed to spontaneously void and concern for acute renal failure resulted in her being transferred to a tertiary pediatric hospital for further evaluation. This later resolved during the hospitalization.
During the admission, radiographs of the chest, abdomen, and pelvis were obtained. The images demonstrated multiple vertebral segmentation defects in the thoracolumbar spine and agenesis of the distal sacrum and coccyx. Our patient was noted to have fusion of multiple vertebrae within the cervical spine consistent with a Klippel–Feil deformity and partial fusion of multiple ribs bilaterally consistent with spondylocostal dyostosis. She was referred to pediatric orthopaedics. Since our patient was healthy and without other congenital anomalies, she was conservatively followed with serial radiographic evaluations every 6 months (). On exam, she was noted to have a short neck with limited range of motion. Bowel and bladder function were normal. Motor responses were good, and there was full strength in the lower extremities.
She began walking at eight months old. At one year of age, she was noted to have a 40° thoracolumbar scoliosis. At age three, she was noted to have a disproportionately short trunk, but was otherwise well developed and wanted to participate in gymnastics. Over the next few years, our patient's congenital spinal deformity was stable. By age seven, she had developed an extremely protuberant abdomen and had prominences of her rib cage due to relatively little longitudinal growth of the spine. Her 3-year-old younger sister, who had no evidence of skeletal deformity, had surpassed her in height at this point. However, the patient was otherwise asymptomatic and doing well in school, subsequently participating in gymnastics, tumbling, and cheerleading with only minimal episodic back pain.
At age ten, radiographs began to demonstrate evidence of consolidation in the thoracic spine. Our patient continued to remain very active over the ensuing years with cheerleading and dance but had increasing back and chest wall pain with these activities. Due to her short truncal stature, she also began to have pain related to costo-iliac impingement that occurred when she bent over, sat, or coughed. She was managed conservatively with NSAIDs and core strengthening exercises which provided some relief. Notably, the patient did not have shortness of breath with her regular activities or frequent respiratory illnesses. By age 14, her spine had almost completely fused. She continued to follow-up with her orthopaedic surgeon every 3 years and had no notable progression of her spinal deformity.
At the age of 18, she became pregnant but subsequently terminated the pregnancy due to obstetrical concerns for compromised respiration secondary to limited thoracic volume. At the time, she also underwent a tubal ligation to prevent future pregnancies. At 21, she returned for follow-up. She was working a job which required her to remain standing throughout her shift that was causing her to experience increasing hip and knee pain. She also noted increasing back pain without neurological deficits that had progressed over the last 3 years. She had a notable leg length discrepancy due to pelvic obliquity, a result of her scoliosis. She was treated with a 5/8-inch shoe lift which provided some relief.
At the age of 25, she experienced an episode of severe back pain for which she visited the emergency department (ED). Radiographs were obtained which could not definitively rule out a vertebral compression fracture, so an outpatient bone scan was obtained. The scan demonstrated multiple foci of increased uptake in the vertebral bodies of the cervical, thoracic, and lumbar spine as well as the costovertebral region. These nonspecific findings were attributed to stress reactions from the patient's multiple vertebral anomalies. Several years later, she was involved in a high-speed MVA and was transported to the ED complaining of significant neck and back pain. At the time, computed tomography (CT) of the chest, abdomen, and pelvis and a posteroanterior (PA) chest film were obtained which illustrated her known vertebral, costal, and pelvic anomalies with no evidence of acute fractures (Figures –). She was next seen at age 30 after an accident on a “slip and slide” in which she dove and noted immediate pain in the lower right rib cage. Radiographs illustrated a nondisplaced fracture of the right 7th rib near the costochondral junction which was managed conservatively with NSAIDs (). The fracture subsequently went on to heal with resolution of symptoms at one month.
She is now 31 years of age and currently suffers from chronic back pain for which she takes naproxen daily. Two to three times per year she has exacerbations of mid-back pain, necessitating a visit to the emergency department for intravenous narcotic analgesics. She also complains of intermittent sciatic neurogenic symptoms including numbness and paraesthesias down the right lower extremity which are exacerbated by sitting in the car for extended periods of time. She occasionally has dyspnea with exertion after walking long distances or climbing the stairs. She feels that her shortness of breath has always been present, but only has limited her during certain activities in the past three years. Given the intermittent nature of her respiratory issues, she has not required a pulmonary function workup; however, this may be necessary moving forward. She currently smokes a half pack of cigarettes daily and has a 3-pack year history. She is being encouraged to quit. She is now 54 inches tall (4′6″) and weighs 138 pounds. She has been counseled on diet and exercise given the risk of abdominal obesity causing further restriction of lung volume. She completed a Scoliosis Research Society 30 Questionnaire (SRS 30) at her most recent follow-up and 5 years prior. Notably, she felt that she looked worse in clothes and had a more negative body image at present. However, she has increased her level of activity despite increasing back pain at rest.


A 26-year-old female presented with the complaint of painless and progressive swelling on the dorsum of the tongue for 1 year. It was associated with slight difficulty in swallowing and speaking. However, it was not associated with bleeding. There was no history of fever, cough, or significant weight loss. She denied any history of trauma. There was no history of similar swellings in other body parts. Her medical and surgical history was not significant.
On examination there was a single, pale to pinkish exophytic growth 2.5 × 2 cm in size, arising from the dorsum of anterior tongue with a pedunculated base (Figs. and ). It was soft, nontender with a smooth surface that did not bleed on touch. There was no pus discharge from the lesion. Tongue mobility was normal. Her oral hygiene was poor. There was no cervical lymphadenopathy. Based on the history and the physical findings, hemangioma and papilloma were considered as the differential diagnoses. Routine investigation including complete blood count, coagulation profile, serology, and biochemistry were within normal limits. Excisional biopsy was performed under local anesthesia. The histopathological examination(HPE) revealed multiple lobules of capillaries of varying caliber lined by endothelial cells, with an overlying stratified squamous epithelium showing focal ulceration. This was consistent with the diagnosis of pyogenic granuloma (Fig. ).


A 15-year-old girl was brought from a remote village with the complaint of painless and progressive swelling arising from the upper lip for 4-month duration. It bled occasionally, especially when touched. Her parents were mainly concerned about the cosmetic problem caused by the lesion. Her past medical and surgical history were not contributory. She denied any history of trauma to the upper lip. There was no palpable cervical lymphadenopathy.
On examination, there was a single pinkish mass with a sessile base (Fig. ),0.5 × 0.5 cm in size, arising from the upper lip. It was mildly tender with minimal bleeding on palpation. She had poor oral hygiene. A provisional diagnosis of hemangioma was made. Routine preoperative investigation, including coagulation profile, was within normal limits. Punch biopsy was taken from the lesion. The HPE findings were suggestive of pyogenic granuloma. Excision of the mass was performed under local anesthesia.


We report the case of a 9-year-old Saudi male who presented at King Abdulaziz University Hospital with a 3-day history of pallor, lethargy, shortness of breath, and headache. Along with these manifestations, the family noticed a yellowish discoloration of the sclera. The patient was in optimal health, with no significant medical complaints, until 2 weeks prior to hospital admission, a time when he developed symptoms of upper respiratory tract infection (i.e., a sore throat, cough, and mild subjective fever). One week later, he developed diarrhea with a frequency greater than 5 times daily. The diarrhea was yellowish in color and without blood. Furthermore, the diarrhea was associated with epigastric pain and vomiting, and it resolved a few days later; however, the epigastric pain became more severe, and his vomiting continued. Due to this situation, the patient had been unable to maintain his usual appetite and had lost some weight. The patient also reported a decrease in the amount of his urine and a change of the urine's color, which was described by the mother as tea-colored. No rash or edema was reported.
The patient was referred to our tertiary hospital by a dispensary after their results revealed red and granular casts in the urine and abnormal kidney function.
An initial assessment revealed that the patient had a rapid but regular heart rate of 118 beats/min. His respiratory rate was 20/min, his blood pressure was 119/61 mmHg, and his oxygen saturation was 100%.
Examination showed that the patient was conscious and oriented; he was pale and had yellowish sclera but exhibited no other significant signs (i.e., no periorbital or lower limb edema, skin rash, or petechia). His chest was clear, and the heart sounds were normal, with no additional sounds or murmurs. A neurological examination was unremarkable.
Based on the initial assessment and the laboratory work-up, (Table ) hemolytic uremic syndrome was strongly suspected due to the presence of the classical triad of the disease. The diagnosis of PSGN was unlikely for several reasons, although the patient had a high ASO titer; this only confirmed a previous exposure and not an infection. Additionally, another more probable disease, i.e., HUS, better explained the abnormal kidney function and urine analysis. Unfortunately, we were unable to obtain a throat swab results and get an evidence of a streptococcal infection. Thus, PSGN could not be completely ruled out.
There are few similar cases reported in the literature involving patients with SLE who initially present with HUS , , , , , . (Table )
Surprisingly, the patient also exhibited a positive immune profile for SLE (i.e., ANA and dsDNA).So far, a clear diagnosis of the acute kidney injury was difficult to be made. For this reason, a kidney biopsy was indicated.


An 82-year-old Japanese man suddenly developed dysstasia without back or neck pain, which made him unable to walk and barely able to stand. He was previously able to perform moving exercises of his neck every morning. Thus, he was admitted to our hospital. He had a medical history of cerebral infarction (treated using 100 mg/day aspirin), dementia, and hypertension but had no history of head and neck trauma. The patient was alert on admission, and his blood pressure was 163/57 mmHg. Neurological examination revealed the presence of astasia–abasia. The Romberg test was also positive. However, the rest of his physical examinations, such as the test for motor or sensory deficits, had unremarkable findings. Moreover, the patient had no coagulation disorder, based on blood test results. The cervical bone conditional computed tomography (CT) revealed no specific findings, although the soft tissue conditional CT showed a high-density mass at C2–C5. Furthermore, magnetic resonance imaging (MRI) of the cervical spine displayed a ventral extradural lesion at C2–C5, which was iso-/hyperintense and hyperintense to the cord on T1- and T2-weighted images, respectively (Fig. ). Based on these findings, we diagnosed the patient with SSEH, which extended from C2 to C5. Considering that his symptoms did not improve after 24 h, we performed hematoma removal and decompression by corpectomy from the front of C3–C5 at approximately 32 h after the onset of his symptoms (Fig. ). The surgery time was 4.4 h. Subsequently, he was able to stand and walk 3 days after the operation without fixation. Currently, the patient can walk without assistance and has not experienced recurrence for 1 year.


A 90-year-old Japanese man suddenly developed dysstasia without back or neck pain and was transferred to our hospital. He had a medical history of hypertension and dementia but had no history of trauma or previous antiplatelet medication use. The patient was alert on admission, with a blood pressure of 154/93 mmHg. Neurological examination revealed the presence of astasia–abasia. The Romberg test was also positive. The rest of his physical examinations, such as the test for motor or sensory deficits, showed unremarkable findings. Furthermore, the patient had no coagulation disorder, based on blood test results. The head MRI revealed no specific findings, although the soft tissue conditional CT displayed a high-density mass at C2–C5. In addition, the MRI of the cervical spine showed a dorsal extradural lesion at C2–C5, which was isointense and hyperintense to the cord on T1- and T2-weighted images, respectively (Fig. ). The cervical spinal cord was normal. Based on these findings, we diagnosed the patient with SSEH, which extended from C2 to C5. We started conservative treatment using methylprednisolone (500 mg/day), tranexamic acid, and mecobalamin. The patient's symptoms gradually improved after 24 h without further worsening. We decided to manage the patient using conservative treatment because of the observed improvements in his neurological deficits and advanced age (>90 years). He was discharged on day 19 without any neurological problems.


The patient was female who was 54 years old at the time of her first visit to the clinic. In the previous year’s screening, she had tested positive for fecal occult blood, but the condition remained untreated. As the symptoms of melena gradually developed, she came to the clinic.
Medical history shows cervical cancer after conization (at age 41).
No notable abnormalities were found. Tumor markers (CEA 0.8 ng/mL, CA19-9 12.13 U/mL, and SCC 1.5 ng/mL) were within normal ranges.
There was a type 1 tumor (25 mm in diameter) extending from the anterior anal canal wall to the lower rectum (Rb).
The tumor was diagnosed as invasive poorly differentiated carcinoma with a tendency for cornification and was suspected to be squamous cell carcinoma.
Contrast-enhanced, irregular thickening of the lower rectal wall was observed. Lymphadenopathy or distant metastasis was absent.
The preoperative diagnosis was cT1b(sm)N0M0 stage IA, and the patient required surgery.
Proximal D3 lymphadenectomy with laparoscopic abdominoperineal resection was performed. Because the preoperative diagnosis was cT1b(sm)N0M0, lateral dissection was not performed.
There was a type 1 tumor, measuring 22 mm in diameter, extending from the lower rectum (Rb) to the anal canal (Fig. ).
The diagnosis was adenosquamous carcinoma pT3(A) ly2 v1 pN1(2/16) sM0 stage IIIa.
The tumor was composed of an approximately 1:1 ratio of adenocarcinoma to squamous cell carcinoma components (Fig. –).
Lymph node metastasis was only found in #251, which was a result of metastasis of the squamous cell carcinoma component.
Due to wound infection on the perineum, incision drainage was performed and the patient was discharged on postoperative day 15.
The patient underwent postoperative adjuvant chemotherapy with S-1 (80 mg/day) for 1 year. On CT, performed 12 months after the first surgery, enlargement of the left lateral and inguinal lymph nodes was detected (Fig. ). Concomitant PET showed a high concentration of fluorodeoxyglucose in the left lateral lymph nodes, and the patient was diagnosed as having a recurrent tumor of the lateral lymph node. As there was no standard strategy for the treatment of recurrent rectal adenosquamous carcinoma, we expected tumor regression by induction chemotherapy followed by conventional lateral lymph node dissection, and chemotherapy with mFOLFOX6 + bevacizumab was indicated for the next 6 months. On CT, performed 18 months after the first surgery, the tumor exhibited an increased propensity to metastasize to the lateral lymph nodes of the left internal and external iliac regions (Fig. ), and no distant metastasis was detected. Because the tumor did not respond to the chemotherapy, extended radical surgery was necessary for the curative resection. Therefore, the patient was sent to a referral hospital.
As there was no sign of right lateral lymph node metastasis, unilateral extended left lateral and inguinal lymphadenectomy with combined resection of the external iliac vein, internal iliac artery and vein, obturator nerve, and pelvic nerve plexus was performed.
Metastases of the adenosquamous carcinoma were observed in 5 out of 9 of the resected lateral lymph nodes. The tumor was composed of approximately an 8:2 ratio of the squamous cell carcinoma to adenocarcinoma components. There were a number of necrotic cells in the adenocarcinoma component. Therefore, the response to chemotherapy was rated grade 2. However, there were only a few necrotic cells in the squamous cell carcinoma component. Consequently, the response to chemotherapy was rated grade 1a. There were no metastases to the inguinal lymph nodes.
The patient did not receive postoperative adjuvant therapy after the reoperation and remains alive without recurrence 90 months after the first surgery and 70 months after the reoperation.
Adenosquamous carcinoma of the colon was first reported by Herxheimer [] in 1907. According to the eighth edition of the “General rules for clinical and pathological studies on cancer of the colon, rectum and anus” [], adenosquamous carcinoma of the colon is defined as a tumor comprising adenocarcinoma and squamous cell carcinoma. Although the rules do not mention the proportions of the adenocarcinoma and squamous cell carcinoma components in the tumor in their definition, previous reports have defined adenosquamous carcinoma of the colon as a tumor with no less than 40% [] of a squamous cell carcinoma component. Because the tumor in our case contained 50% squamous cell carcinoma component, it was diagnosed as an adenosquamous carcinoma.
Adenosquamous carcinoma of the colon is a rare form of cancer with an incidence of about 0.1% of all colorectal malignancies reported in Western countries and in Japan [, , ]. Yokoi and colleagues [] have reported that the incidence of adenosquamous carcinoma of the colon is slightly higher in males (1.19:1) and that the tumor is more likely to occur in the right colon compared with adenocarcinoma. Furthermore, adenosquamous carcinoma of the colon is often detected at an advanced clinical stage, in which the tumor is already large and is characterized by a high incidence of lymph node metastasis, compared with adenocarcinoma [–]. Besides surgical resection, no effective treatment has been established to treat adenosquamous carcinoma of the colon, which is resistant to chemotherapy. Although there have been reports describing the effectiveness of radiation therapy for local recurrence of adenosquamous carcinoma of the colon following surgery [, ], the number of cases has been small. Moreover, adenosquamous carcinoma of the colon is known to be a highly malignant form of cancer with a poor prognosis compared with adenocarcinoma. Nishimura and colleagues [] reported that the 5-year survival rate was 43.3% among 61 patients with adenosquamous carcinoma of the colon. Since the 5-year survival rate of all colorectal carcinomas is 72.1% [], the prognosis of patients with adenosquamous carcinoma of the colon is worse than that of patients with other colorectal carcinomas.
The efficacy of surgical resection for liver and lung colorectal metastases has been reported. However, for distant metastases to other sites, the treatment guideline for colorectal cancer indicates that surgical resection is to be considered if the tumor is amenable to complete resection, and if curative resection is difficult, chemotherapy and/or radiation therapy should be considered as the options. Although there have been reports describing the efficacy of surgical resection for recurrent lateral lymph node tumor, there is no established treatment strategy for this cancer. Since the efficacy of chemoradiation therapy for adenosquamous carcinoma has not been established, it is considered necessary to insist on surgical resection when considering the treatment options.
Lymph node metastases of adenosquamous carcinoma of the colon are often a result of metastasis of the adenocarcinoma component. While there have been some reports indicating that the squamous cell carcinoma component is also present in the metastatic lesion, the latter type, composed solely of squamous cell carcinoma, is believed to be rare [, ]. In our case, the lymph node metastasis observed during the first surgery was a bona fide squamous cell carcinoma, but the small size of the metastatic lesion made it difficult to make an accurate diagnosis. Therefore, we expected tumor regression by induction chemotherapy followed by conventional lateral lymph node dissection, and chemotherapy with mFOLFOX6 + bevacizumab was indicated. However, in the lymph node metastases resected during the reoperation, the tumor was composed of an 8:2 ratio of squamous cell carcinoma to adenocarcinoma components, demonstrating the dominance of the squamous cell carcinoma component. In consideration of the fact that, in this case, the adenocarcinoma component showed a grade 2 response to chemotherapy, in retrospect, there might have been other treatment options besides surgical intervention, such as combining radiation therapy and/or incorporating combination therapies using drug(s) effective for the treatment of squamous cell carcinoma.


A 34-year-old woman, gravida 3, para 2, with a history of an intrauterine death at 32 weeks of gestation due to hydrops fetalis as a result of Rh alloimmunization in the previous pregnancy was referred to our center at 13 weeks of gestations. There were no pathologic findings in her physical examination, laboratory findings, and obstetric ultrasonography. Gray-scale and color Doppler ultrasonography evaluation was performed to detect the findings of anemia and hydrops with a two-week interval starting from the 18th gestational week in the Prenatal Diagnosis and Treatment Unit by a protocol defined by Society for Maternal-Fetal Medicine for the pregnant women complicated with Rh alloimmunization []. Her obstetric follow-up was unremarkable until 29 weeks of gestation. At 29 weeks of gestation, mid-cerebral artery peak systolic velocity (MCA-PSV) was detected as 80.6 cm/sn [>1.5 multiple of the median (MoM)] in her obstetric Doppler ultrasonography that suggests fetal anemia with ascites, cardiomegaly, and pericardial effusion. An intrauterine intravascular transfusion was performed, and the hemoglobin concentration before the procedure was detected as 2.9 g/dL. 40 ml O Rh(D)-negative red blood cell that was freshly prepared and underwent irradiation and leukodepletion was transfused to the fetus via the umbilical vein in the portion of the umbilical cord near its insertion into the placenta by a 22-gauge needle with no complication. Posttransfusion hemoglobin was detected as 8.1 g/dl. The initial and posttransfusion platelet count was detected in normal range (154000/μl and 163000/μl, resp.). A few days later, ultrasound examination revealed the presence of an echogenic collection involving right lateral ventricle and extending to the surrounding cerebral parenchyma compatible with grade IV intraventricular hemorrhage (). The couple was counseled, and they opted for the continuation of in utero therapy. The second IUT was scheduled after ten days. At 30 + 4 weeks of pregnancy, the second IUT was performed. The initial hemoglobin value was detected as 4.9 g/dl. Persistent fetal bradycardia was noted during the procedure, and an emergency cesarean section was performed. APGAR score 4/7, 1315 g, 43 cm male infant was delivered by cesarean section. Neonate was transferred to the neonatal intensive care unit. Exchange transfusion, phototherapy, and intravenous immunoglobulin treatment were applied. Postnatal cranial ultrasonography showed diffuse echogenicity extending from the inferior left caudate nucleus to the left ventricle that leads to left ventricular dilatation compatible with intraventricular grade IV hemorrhage. The intracranial hemorrhage was gradually regressed in the subsequent ultrasonographic examinations and completely disappeared at the end of the first month, and the neonate was discharged from the hospital after healing two months after birth. At the time of writing this paper, the baby was showing normal neurological development at 6 months.


We present the case of an 84-year-old female who presented with cough productive of clear phlegm, dyspnea on exertion, and right-sided chest pain. Her past medical history included systemic lupus erythematosus, thrombotic microangiopathy, deep venous thrombosis, coronary artery disease, hypertension, and hypercholesterolemia. She was a former smoker with a 60 pack-year smoking history. Family history was noncontributory. A physical examination revealed decreased breath sounds over the right lower lung field with associated scattered rales. A chest X-ray revealed right lower lobe opacity with right pleural effusion. A computed tomography (CT) of the chest showed right lower lobe consolidation with moderate to large sized right pleural effusion and associated right hilar and mediastinal lymphadenopathy, thought to be reactive in nature. Diagnostic and therapeutic thoracentesis was performed, and she was empirically started on antibiotics for possible pneumonia. The pleural fluid analysis was consistent with a transudate with no malignant cells on cytology. She was subsequently discharged to follow up as an outpatient. After a month, she was readmitted for worsening dyspnea, cough, and hemoptysis. Chest X-ray showed increased right-sided pleural effusion and CT chest revealed large right pleural effusion with a new appearing soft tissue density filling the bronchus with associated atelectasis (Figure ). The patient underwent another diagnostic and therapeutic thoracentesis in addition to bronchoscopy and bronchioalveolar lavage. A biopsy could not be performed because she was unable to tolerate the entire procedure.
Pleural fluid cytology showed an abnormal B cell population consistent with a B cell lymphoproliferative disorder. Of the 93% total viable cells, approximately 51% were T cells and 42% were B cells. Virtually all of the B cells expressed CD19, CD20, PAX5, CD43 and CD38 dim. Kappa light chain was equivocal (dim/negative). The cells were negative for CD5, CD10, CD11C, CD22, CD23, BCL6, cyclin D1, and surface lambda light chain. No abnormal T-cell population was identified. Pathology from bronchial brushings also revealed a monoclonal kappa B cell population lacking CD5 or CD10 expression.
With the presumed diagnosis of BALT lymphoma, the patient was started on bendamustine and rituximab. After four cycles of chemotherapy, a positron emission tomography/computed tomography (PET-CT) scan demonstrated increased fluorodeoxyglucose (FDG) uptake in the heterogenous right lower lobe pulmonary mass involving bronchus intermedius and right lower lobe bronchus (Figure ). Various possibilities were considered at this point including the progression of marginal zone lymphoma or a second primary lung cancer. Pathology from a subsequent CT-guided core biopsy revealed squamous cell lung cancer (SCLC), consistent with clinical stage 2A disease. Given the advanced age of the patient and comorbidities, she was not a surgical candidate. Radiotherapy was pursued and she received a total of 5000 cGy in 20 fractions with symptomatic improvement following which she developed a spontaneous pneumothorax deemed to be secondary to radiotherapy. It was managed by suctioning with pleurX vacutainer. Two months later, she developed loose watery diarrhea, increased lethargy, and a decline in overall functional status. Given the debility, multiple hospitalizations, and overall poor prognosis, a decision for hospice care was made.


A 75-year-old right-handed female was brought to the emergency department (ED) with complaints of dysphagia, increasing fatigue, upper extremity weakness more prominent on the left side, and upper extremity numbness for a one-week duration. She had a history of B-cell lymphoma and had received six cycles of chemotherapy (CHOP). Two weeks prior to presentation, she had presented with left-sided facial nerve palsy and left abducens nerve palsy. Magnetic resonance imaging (MRI) brain scan at that time had revealed a focus of increased diffusivity in the left caudate, with a hyperintense signal on diffusion-weighted imaging (DWI) without apparent diffusion coefficient (ADC) correlate. Her other medical comorbidities were congestive heart failure (ejection fraction <55%), depression, chronic headaches, history of pulmonary embolism, hypertension, hyperlipidemia, multiple transient ischemic attacks (TIA), and hyperhomocysteinemia. Her blood pressure (BP) at presentation was 160/83 mmHg. On examination, she had slurred speech with prominent nasal tone. Cranial nerve examination was significant for left abducens nerve palsy (restricted left eye abduction), lower motor neuron type of left facial weakness, and poor elevation of the right soft palate. Sensory examination showed decreased sensation on left medial forearm. Motor examination showed left-hand flexor and extensor weakness without loss of muscle bulk. MRI brain revealed an increase in signal intensity in the head of the left caudate nucleus compared to the scan from two weeks prior without any interval acute findings or contrast enhancement (Figures -).
MRI C-spine did not reveal any myelopathy to explain left arm weakness although moderate-severe neural foraminal stenosis from C3-C6 was noted. Computed tomography (CT) of the chest and neck did not reveal any findings to explain dysphagia. Electromyography (EMG) and nerve conduction studies (NCS) showed changes consistent with chronic sensorimotor axonal polyneuropathy along with carpal tunnel syndrome and ulnar neuropathy on the left. Direct laryngoscopy confirmed right sided vocal cord paralysis consistent with right vagal nerve palsy. Lumbar puncture with CSF analysis showed abnormal cells consistent with malignancy. Flow cytometry analysis showed CD19 and CD20 positive B cells, thus confirming CNS involvement of DLBCL. Hematology/Oncology was consulted for CNS involvement of DLBCL. The patient however refused further chemotherapy and decided to proceed with palliative care. She was eventually discharged to a nursing facility.


A 4-year-old girl presented at the Akdeniz University Dental Faculty Department of Oral and Maxillofacial Surgery with a painless sublingual swelling. Extraoral examination revealed no facial abnormalities or swellings. Intraoral examination revealed a large, compressible cystic mass in the midline of the anterior floor of the mouth, causing significant elevation of the tongue. The mucosa covering the mass was normal in color, while the mass itself was smooth, firm and ﬂuctuant at bi-manual palpation, with no evidence of inflammation (Fig. ). The patient’s parents were uncertain how long the swelling had been present, but stated that their child’s speech was below the level of her peers. The child’s medical history was unremarkable. No regional lymphadenopathy was noted. Magnetic resonance imaging (MRI) revealed contiguous, round, T2-hyperintense cysts with regular margins in the central and anterior parts of the floor of the mouth that were 22x22 mm and 25x21 mm, respectively, with T1-hyperintense areas observed in the anterior cystic lesion (Figs. a, b). Preoperative differential diagnoses included epidermoid or dermoid cysts, cystic hygroma, ranula and lymphangioma.
Under nasotracheal intubation, using an intraoral approach, a semi-lunar incision was made on the ventral surface of the tongue from left to right. Using blunt dissection, the first lesion was separated from the surrounding tissue, and the genioglossus muscle was separated from the lesion. The first lesion was then gently elevated from the mylohyoid muscle and removed in one intact piece (Fig. a). Next, the second lesion was accessed through a blunt dissection in the muscles of the floor of the mouth (Fig. b) and excised in the same manner as the first lesion. Hemostasis was established, the wound edges were closed, and a 0.25 inch Penrose drain was inserted. The drain was removed on the postoperative 3rd day, and the region was closed primarily via suture. Histopathological examination of the specimens taken during surgery revealed the first, anterior cyst to be a dermoid cyst and the second, posterior cyst to be a heterotopic oral gastrointestinal cyst (HGIC) (Figs. a. b). Postoperative healing was uneventful. In line with clinical protocols, the patient presented for regular follow-up visits.


A 53-year-old right-handed man was admitted to our hospital for acute onset of isolated hemianesthesia on the right side. He had no history of hypertension, diabetes, and hepatitis but had 35-year history of daily alcohol consumption of 150 to 250 mL of liquor greater than 50 degrees. On admission, he was normotensive, and, except for hemihypoaesthesia on the right side, a neurological examination revealed no dizziness, nystagmus, dysarthria, facial weakness, tongue deviation, limb weakness, ataxia, Horner syndrome, diplopia, and hearing loss. Deep tendon reflexes were symmetric, and plantar responses were normal bilaterally. Brain computed tomography (CT) demonstrated a hemorrhagic lesion in the dorsal and medial medulla oblongata with hemorrhage rupturing into the fourth ventricle (Fig. A). Brain magnetic resonance imaging (MRI) with T2 sequences showed hyperintensity in the medulla oblongata (Fig. B), whereas magnetic resonance angiography (MRA) showed no significant findings. Laboratory tests revealed mild coagulation disorders, including mildly elevated prothrombin time (PT) of 13.3 s, thrombin time (TT) of 26.4 seconds, international normalized ratio (INR) of 1.26, and decreased fibrinogen (FIB) at 0.97 g/L. Antigen, and antibody for viral hepatitis, anti-HIV, and syphilis were all negative. Liver function tests showed elevated aspartate aminotransferase (AST) (135 IU/L), alanine aminotransferase (ALT) (104.67IU/L), alkaline phosphatase (ALP) (201.36 IU/L), and gamma glutamyl transferase (GGT) (1547.58IU/L). Count of blood platelet was 57 × 109/L. Abdominal ultrasound, and CT confirmed a small, and nodular liver, and splenomegaly. The score from FibroScan was 18.8 kPa, and that of Child–Pugh was Class A. Oral glutathione was administered with dosage of 0.4 g 3 times daily and hemocoagulase injection with 2 U was started once a day. Fourteen days after the liver protection therapy and the management of coagulation disorders, he was discharged with mild hemianesthesia but with more improved parameters in laboratory tests. At a 6-month follow-up, MRI showed a malacic lesion in the medulla oblongata. Non-contrast enhanced MRI demonstrated no abnormal areas of enhancement, and results showed negative findings on follow-up MRA (Fig. C and D).
The patient signed the informed consent form for publication of this case report.


The patient was a 70-year-old man with history of hypertension and hyperlipidemia who presented with gait instability (2 weeks), chronic dry cough (4 months), weight loss (15-lb in 2 weeks), and fever (in 2016). His dry cough started 4 months prior to his presentation for which he underwent extensive work-up with no specific pathologic findings. He was prescribed different medications including prednisone for presumed COPD with no improvement.
Review of systems was significant for exertional dyspnea. Splenomegaly was noted on physical exam with no palpable lymphadenopathy. At the time of presentation, he had full strength (5/5) in upper and lower extremities with mild atrophy of proximal muscles. His gait was broad-based without difficulty turning.
Laboratory findings included prolonged PT and PTT along with mildly decreased factor VII level attributed to mild vitamin K deficiency or underlying liver disease; although transaminitis was noted, hepatic evaluation was negative for autoimmune and viral hepatitis. Chest CT-scan showed subsegmental left lower lobe acute pulmonary embolus (PE). Serum protein electrophoresis (SPEP) showed a discrete abnormal band, measuring < 0.2 g/dL identified to be IgM kappa by serum immunofluorescence studies (IFE). Electromyogram (EMG) and Nerve Conduction Studies (NCS) showed demyelinating axonal neuropathy concerning Guillain-Barre syndrome (GBS). A paraneoplastic autoantibody panel was negative. Serum-free light chains were normal.


The patient was a 31-year-old woman who presented with severe headache, dizziness, and facial edema in the beginning of her pregnancy (in 1997). Initially, it was attributed to her pregnancy, but due to persistence of the symptoms, she underwent an extensive work-up including lumbar puncture and MRI with no specific pathologic findings. She was started on corticosteroids with some improvement.
At 16-week gestational age, she developed abdominal pain followed by a spontaneous abortion; however, the pain was not relieved by pain medications. Further work-up revealed superior mesenteric vein thrombosis and subsequently the patient underwent resection of the infarcted bowel.


A 2-year-old male child presented with a painless slowly progressive mass in the inferolateral aspect of right orbit of three-month duration. There was no associated history of fever or any other systemic illness.
General physical examination and systemic examination were normal. Local examination revealed fixed, firm, nontender mass in inferolateral orbit of approximately 25 × 20 mm.
Differential leucocyte count revealed raised eosinophil count (13%). On radiological examination, CT scan showed 25 × 27 mm round well-defined smooth-outlined homogenously enhancing space-occupying mass arising from the zygomatic bone at the inferotemporal periorbital area of right orbit with bone erosion. The MR imaging showed an exophytic infiltrative mass with irregular margins arising from the anterolateral wall of right orbit. The mass was localized to the extraconal space sparing the intraconal compartment. The lateral rectus muscle was not involved and was distinctly visible and separate from the mass ().
Incision biopsy of the mass was done and histopathological examination of the specimen revealed characteristic Langerhans cells, 15–20 um in size with discrete nucleolus and homogenous eosinophilic cytoplasm. The immunohistochemical studies were positive for S-100 protein and adenosine deaminase (). CD1a positivity was also demonstrated while electron microscopy revealed Birbeck granules.
A diagnosis of LCH was made and the patient was subjected to PET-CT to identify any other foci. The PET-CT revealed a single focus of uptake in the lateral wall of the orbit (). No other foci of uptake were noticed anywhere else in the body.
The patient received 12 cycles of vinblastine 0.2 mg/kg body weight along with oral prednisolone 1 mg/kg body weight. On completion of three cycles of chemotherapy, a reduction in size of the mass was noticed (Figures and ).
A repeat PET scan was done 3 months after completion of chemotherapy. The PET scan did not reveal any activity noted previously.
The patient is currently being followed up for last 2 yrs without any recurrence.


A 47-year-old woman was referred to our hospital, with a 4-year history of anal itchiness and an erythematous eczematoid skin rash in the perianal region. The woman complained that the lesion had gradually increased in size and she suffered from severe itching. A perianal shave biopsy revealed an EMPD. The preoperative testing included a complete blood count, colonoscopy, abdominal and chest computed tomography, digital mammography, an abdominal and pelvic ultrasound, and pelvic examination. The test results were negative for cancer. The procedure included a wide local excision, resulting in a semicircular defect of 3.5 cm in diameter. The reconstructive technique involved a pedicle flap based on internal pudendal artery skin perforator, which had been identified by a handheld Doppler device preoperatively. The design of the flap was in a diamond-shaped pattern (Figs. , ), a modified V-Y advancement flap. A typical V-Y flap would not be practical because it would lead to anal stenosis. In contrast, the diamond-shaped flap was able to cover the defect, without causing anal stenosis. The perforator was identified (Fig. ), and the subcutaneous vessels. The flap was advanced to cover the defect and sutured in place. The diamond-shaped flap covered the defect fully, with 1 quarter of its surface used to cover the intra-anal portion of the defect. The donor side was closed directly. Six months after surgery, the patient is disease-free and has returned to her activities (work, swimming, jogging). The functional and aesthetic results are successful (Fig. ).


An 80-year-old female without any underlying disease had long-term follow-up in our outpatient department for cataracts of the bilateral eyes. Moderately dense (Grade 3 in The Oxford Clinical Cataract Classification and Grading System) nuclear cataracts were observed bilaterally. Due to progressive visual loss, we performed phacoemulsification cataract surgery for the left eye and the procedure was uneventful. On postoperation days 1 and 7, only mild corneal edema with minimal inflammation was noted. Spontaneous confluent DMD was observed on postoperation day 14 by slit-lamp examination, though not at the surgical site. The DMD was most severe over the superior and mid-periphery area and involved more than 1/2 area of the cornea including the visual axis. Descemet's membrane was separated from the posterior corneal stroma by about 1/2 to 2/3 corneal thickness at the most severe parts. According to Mackool and Holtz[ classification, it was a planar DMD. The best corrected visual acuity (BCVA) decreased to 0.2 compared to 0.3 preoperation (Fig. A and B).
We prescribed preservative-free 0.04% betamethasone eyedrops 8 times daily and 3% hypertonic saline 4 times daily. After 1 week of observation, the condition of DMD with cornea edema was still prominent. We decided to perform anterior chamber tamponade with air under topical anesthesia on postoperation day 21, and finally reattached Descement's membrane.(Fig. C and D) The BCVA improved gradually to 0.6, but left a small Descement's membrane scar (Fig. E and F).
Due to the improvement of the left eye condition, we decided to perform cataract surgery for the right eye 4 months later. Despite a smooth operation, the fellow eye encountered the same situation. On postoperation day 1, only mild corneal edema with minimal inflammation was noted; however, spontaneous significant DMD was observed by slit-lamp examination 5 days after cataract surgery. (Fig. A–C). Descemet's membrane was separated from the posterior corneal stroma about 1/2 to 2/3 corneal thickness at the most severe parts with visual axis involvement. According to Mackool and Holtz[ classification, it was still a planar DMD. We chose conservative treatment with preservative free 0.04% betamethasone with methyl cellulose 8 times daily and 3% hypertonic saline four times daily. Due to the area of DMD and the separation of DMD constantly decreasing during each outpatient follow-up visit, we decided to continue conservative treatment rather than surgical intervention. The detachment totally regressed 2 months after cataract surgery without surgical intervention. (Fig. D–F). No scar was left.
The final VA of this patient was 0.4/0.5 compared to initial preoperation VA 0.1/0.2. No recurrent DMD was observed during the following OPD visits.


A 25-year-old primigravida with no significant past medical history at 22 weeks and 0 days gestation presented to a local hospital with sudden onset of right-sided back pain radiating to her right lower quadrant that had persisted for less than one day. She denied nausea, vomiting, fevers, vaginal bleeding, and dysuria. Her pregnancy had been otherwise uncomplicated. Her pain became uncontrollable with intravenous medication and localized solely to her right lower quadrant. She was transferred to a tertiary care center for further management, given concern for appendicitis and possible need for surgical intervention.
Her pain worsened on transport, and on arrival to the tertiary care center, she demonstrated severe right lower quadrant tenderness with rebound and voluntary guarding without costovertebral angle tenderness. She was hemodynamically stable, but intravenous hydromorphone only provided transient and mild improvement in her pain. Her cervix was found to be closed on digital exam, and no abnormalities were noted on speculum exam. Initial laboratory evaluation demonstrated a normal comprehensive metabolic panel and coagulation studies. Hemoglobin was 10.7 gm/dL and white blood cell count was not elevated (9.9 × 103/μL). Urinalysis was negative.
She underwent an abdominal MRI showing a normal appendix. However, in a verbal read from the on-call radiologist, concern was communicated for right forniceal rupture given the constellation of radiologic findings of hydroureter combined with perinephric and retroperitoneal fluid, highlighted in . Her left kidney and collecting system were normal in appearance. Renal ultrasound was therefore performed, and it revealed right ureteral tapering between the gravid uterus and right iliac artery with no right ureteral jet visualized. Given these findings, the patient was subsequently managed by a multidisciplinary team consisting of maternal-fetal medicine, urology, and interventional radiology. Three strategies were discussed and included conservative management with close follow-up, placement of a ureteral stent, and placement of a percutaneous nephrostomy (PCN) tube. Patient preference was for PCN placement. Of note, urine culture collected prior to PCN placement was negative.
Following its placement by interventional radiology, her pain was relieved, and she was discharged with follow-up with maternal-fetal medicine and interventional radiology. Her pregnancy was subsequently complicated by readmission for recurrent pain and pyelonephritis with culture isolation of Enterobacter cloacae resistant to both nitrofurantoin and trimethoprim/sulfamethoxazole (TMP-SMX). She required placement of a midline IV for daily infusion of ertapenem at her local hospital. Her pregnancy was also complicated by fetal growth restriction, diagnosed at 35 weeks. Uncomplicated vaginal delivery occurred at 37 weeks and 1 day. Her PCN was removed postpartum at which point antibiotics were also discontinued.


A 65-year-old male presented to the emergency department with typical chest pain at rest since 2 hours before admission after eating. The patient denied shortness of breath and previous episode of chest pain before admission. He had a past medical history of poorly controlled hypertension and was taking captopril. History of diabetes, smoking, and previous stroke or myocardial infarction was denied. On physical examination, blood pressure was 150/90 mmHg, heart rate was 57x/minute, and respiratory rate was 20x/minute, and the cardiopulmonary examination was within normal limits. ECG showed sinus rhythm of 57 bpm and >1 mm upsloping ST depression with symmetric tall T in lead V2-3 characteristic of de Winter T-wave ECG pattern (). Routine blood examination, AST/ALT, BUN, and serum creatinine were within normal limits. Sodium was 137 mmol/L, and potassium was 3.4 mmol/L denoting that tall T was not due to hyperkalemia. Troponin at the 2nd hour after onset was negative. The patient was diagnosed with acute coronary syndrome with suspected de Winter T-wave ECG pattern. Aspirin 160 mg, clopidogrel 300 mg, sublingual isosorbide dinitrate, simvastatin 20 mg (atorvastatin was not available), and enoxaparin 0.4 mg subcutaneous were administered. The patient refused referral to the interventional cardiology capable hospital due to distance and socioeconomic causes. Repeat ECG was done 3 hours later showing Q-waves developing in V2–4, consistent with the final stages of acute STEMI accompanied with ST-segment elevation in lead V2–4 which barely meets the 2 mm threshold for those precordial leads as per the guidelines (). Elevated troponin at the 5th hour confirmed the presence of myocardial infarction. The patient still experienced sustained and severe chest pain although not as intense as initial presentation. Streptokinase (1.5 million units) was administered, and chest pain subsided with a resolution of ST-segment elevation (). The patient was discharged on the 5th day after admission.


A 53-year-old female presented to clinic with pain over the lateral aspect of the right foot which began after ‘rolling’ her right ankle three months prior. She reported that two previous attempts of conservative management with a fracture boot for six weeks, which was subsequently repeated for three weeks, did not improve her pain. Multi-planar, multi-weighted, 3 Tesla (3T) magnetic resonance imaging (MRI) of the right ankle revealed the following conditions: 1) chondral thinning over the medial margin of the tibial-talar joint, with underlying bone marrow edema at the level of the medial talar dome, 2) marked fluid distension and synovial thickening with distension of the common peroneal tendon sheath into the peroneus brevis and peroneus longus sheaths, and 3) reactive subcortical bone marrow edema along the lateral wall of the calcaneus. The peroneal tendons demonstrated tearing and degenerative flattening (Figure and Figure ). Inflammatory changes were noted along the posterior tibialis tendon and flexor digitorum longus. Given these findings, the initial assessment was a chronic ankle sprain with peroneal tenosynovitis.
The patient was taken to the operating room for exploration and planned tendon debridement and repair. A lateral ankle incision was placed along the fibula to allow access to both the attenuated anterior talofibular ligament (ATFL) area as well as the retrofibular space to evaluate the peroneal tendons. Careful dissection was performed after elevating skin flaps to avoid damage to neurovascular structures. The superior peroneal retinaculum was incised, and a copious amount of yellow fluid egressed from the distended peroneal tendon sheath. Upon entering the peroneal tendon sheaths, a significant amount of brownish-gray synovial proliferation was noted throughout the retrofibular space. Several samples were taken from the peroneal tendon sheath synovium, peroneus brevis, and peroneus longus, and sent to pathology to determine the cause of this proliferation. The peroneus longus tendon was noted to be torn, with >80% of its length disrupted and degenerated. At that time, a decision was made to excise the peroneus longus tendon and perform a side-to-side tenodesis. A Broström-Gould lateral ankle ligament repair was also performed.
At first follow-up, nine days postoperatively, the patient’s lateral ankle wound was healed and her pain was improving. She was referred for physical therapy but was unable to make the appointment due to personal issues. At three months postoperatively, the patient was called as she had missed her second follow-up due to continued personal issues. She declared that her pain prior to surgery had resolved completely and she was back to all of her activities without difficulty.
Specimens from the peroneus brevis, peroneus longus, and peroneal tendon sheath synovium were evaluated by pathology. Tissue from all tendons demonstrated marked lymphocytic inflammation with chronic and acute inflammation and development of germinal centers observed within the synovium (Figures -).


A 49-year-old man visited to our hospital in December 2016 with complaints of abdominal pain, anorexia, and rapid weight loss for 5 months. He had undergone Billroth-II subtotal gastrectomy 30 years earlier for treatment of a bleeding gastric ulcer. Abdominal CT scan revealed irregular thickening of the gastric walls and miliary calcifications. Histopathological examination of specimen obtained by endoscopic biopsy showed poorly differentiated signet-ring cell cancer with calcifications (Fig. ). Tumor markers were elevated (CEA 6.21 ng/mL, CA125 115.80 u/mL, and CA199 659.93 u/mL). Serum calcium and phosphorus levels were within the normal range. The clinical stage was T4N2M0. A tumor at this stage is inoperable, and so the patient was started on chemotherapy with docetaxel, cisplatin, and fluorouracil (DCF). He received three cycles of DCF, but in March 2017 the chemotherapy protocol was changed to XLOX (oxaliplatin and S-1) because of toxic response. After five cycles of chemotherapy (3 cycles of DCF + 2 cycles of XLOX) the symptoms of abdominal pain and anorexia were relieved, and CEA, CA125, and CA199 decreased to 1.90 ng/mL, 112.80 u/mL, and 344.32 u/mL, respectively. Abdominal CT showed reduction in gastric wall thickening. However, the calcifications had increased (Fig. ). The patient refused further chemotherapy and was lost to follow-up for 2 months.
In July 2017, the patient returned to the hospital with complaints of epigastric pain and abdominal distention. Abdominal CT showed progressive disease according to Response Evaluation Criteria in Solid Tumors (RECIST) criteria and increased calcifications (Fig. ). The patient was started on palliative oral chemotherapy with S-1.


A 34-year-old man had reduced vision in his left eye for about 15 years, and night blindness for about two years. He had a complete ophthalmic examination including measurements of the best-corrected visual acuity (BCVA) and intraocular pressure, slit-lamp examinations, indirect ophthalmoscopy, Goldmann perimetry (GP), perimetry with Humphrey Field Analyzer (HFA), optical coherence tomography (OCT), MP-3 microperimetry, and full-field ERGs. His visual acuity was 20/15 in the right eye and 20/50 in the left eye. The fundus did not show any obvious abnormalities, but optical coherence tomography showed partial loss of the interdigitation zone (Fig. ). The retinal sensitivity of the macular region was reduced in both eyes (Fig. ). His family ocular histories were unknown because both his parents and grandparents were dead.
The ERGs were recorded and analyzed with MEB-9402 (NIHON KOHDEN, Tokyo, Japan) and LS-100 (Mayo, Nagoya, Japan) as light emitting device. Before ERG recordings, we confirmed that the pupils were maximally dilated to approximately 8.0 mm following topical application of a mixture of 0.5% tropicamide and 0.5% phenylephrine (Sandol P; Nitten Pharmaceutical Co. Ltd., Aichi, Japan). ERG recordings performed binocularly with contact lens electrodes.
He was dark-adapted for 20 min, and the dark-adapted 0.01 ERG, dark-adapted 3.0 ERG, and dark-adapted oscillatory potentials were recorded in that order. The eye was then light-adapted for 10 min, and the light-adapted 3.0 ERG, light-adapted 30 Hz flicker (3.0 photopic cd‧s‧m− 2 stimulus luminance) were recorded. Then, ERGs elicited by long-duration stimuli (on and off responses: 63.0 cd/m2 stimulus intensity with 31.6 cd/m2 background intensity, 200 ms duration) were recorded. All of the recordings conformed to the International Society for Clinical Electrophysiology of Vision (ISCEV) standard for full-field clinical electroretinography [].
During the recording of the dark-adapted ERG series elicited by single flashes, there were no changes in the patient’s overall condition. However, as soon as the flicker ERG recordings were completed and long-duration flashes began, the patient reported that a paralysis had developed in both upper limbs. Therefore, the examiner immediately stopped the ERG recordings, and soon thereafter, convulsions of the lower limbs developed and he lost consciousness. The convulsions disappeared after an intravenous injection of diazepam. Examinations of the ERG recordings, no apparent decrease in amplitudes or extension of implicit times were observed as compared with the normal waveforms indicated by ISCEV. No abnormality of the a-wave / b-wave ratio at the maximum combined response was observed (Fig. ). Computed tomography of the head was performed on the same day, and no abnormality was found. Magnetic resonance imaging (MRI) of the head was also performed later, and the findings were normal. There was no abnormality in the electroencephalogram examination performed after the attack. After that incident, he reported that he had had conscious-loss seizures four years earlier.


A 23-year-old male with CF, genotype F508del and c.350G > A, presented to a scheduled CF clinic visit. He is a student from a local University.
He had minimal respiratory symptoms. He had methicillin-sensitive staphylococcal aureus isolated on a prior occasion with no pseudomonas identified. Chest X-ray showed early signs of bronchiectasis. He had no features of chronic sinusitis. He had impaired glucose tolerance with normal glycaemia and had no evidence of pancreatic insufficiency or any liver disease.
On presentation, he complained of four episodes of sore throat, each lasting up to 4–5 days, resolving spontaneously in the prior 3 months. He had no symptoms of nasal congestion, sinus pain, or postnasal drip and no cough or production of sputum. He had not had any treatment for it. At the time of review, he was systemically well with no fevers. His swallow was normal. He had not had any changes to his living environment or any close contact with other CF patients.
On examination, he was afebrile, and PR was 86 and BP 110/80 mmHg. His BMI was 22.
The throat examination revealed an inflamed pharynx with no purulent discharge, and his tonsils were not enlarged or inflamed. Cervical and submandibular nodes were not tender or enlarged. His lungs were clear. His FEV1 at that visit was 4.28 L/min (87% predicted).
A throat swab was obtained as part of assessment for standard bacterial culture, and a nasopharyngeal swab was also taken for respiratory viral PCR. He did not have any sputum to produce for testing at this visit. He did have a sputum sample taken 6 weeks later, which was negative for Pseudomonas.
The bacterial culture of the throat swab isolated non-mucoid Pseudomonas aeruginosa, which was sensitive to Ciprofloxacin, Gentamycin and Piperacillin, and Tazobactam. The respiratory viral PCR was negative. Based on these results, he was started on Pseudomonas eradication therapy, with oral ciprofloxacin and nebulized tobramycin. His symptoms of sore throat disappeared with treatment, but his repeat throat swab after intended eradication still grew Pseudomonas. At this point, he was referred to an ENT surgeon after CT sinuses, which returned features of mild chronic sinusitis.
He underwent tonsillectomy, septal reconstruction, turbinectomy, and full FESS. At surgery, a sample taken from the right maxillary sinus also grew Pseudomonas. He was then treated with intravenous Piperacillin, Tazobactam, and oral Ciprofloxacin for 4 weeks.
Almost 6 months after the initial isolation, his throat swabs returned negative for Pseudomonas. He did not have any more clinical features of pharyngitis.
He made good recovery clinically from pharyngitis and sinusitis and continues to follow up with our CF service. He remains free of Pseudomonas on sputum cultures to date but has isolated Staphylococcus in current sputum.


In 1997, female patient, 24 years old, presented intense lower back pain. Lumbar spine CT revealed multiple osteolytic lesions in lumbar vertebrae. Further investigation by attending physicians ruled out neoplastic infiltration. Clinical and laboratory exams were normal, except for slightly decreased calcium levels of unknown origin, with normal levels of PTH, so the patient was treated conservatively with NSAIDs and careful medical advice.
In May of 2004, the patient discovered a nodule in left thyroid lobe, which gradually increased in size. Biopsy revealed follicular thyroid cancer, and a total thyroidectomy was performed. Histological exam showed papillary and follicular thyroid cancer. Histological preparation did not consist of any parathyroid gland, although patient suffered from severe hypocalcaemia for many years postoperatively until utterly controlled by attending endocrinologist with administration of calcium and salmon calcitonin. In September of 2004, a large cystic mass was detected in anterior cervical surface, left of the clavicle. Ultrasound revealed a cyst with 4 cm diameter which was surgically removed. Histological exam suggested lymphatic cyst.
In 2007, a relapse was observed and was US monitored for the next months. In 2008, the patient was referred to our department for further investigation. Clinical examination showed the palpable mass in the lower anterior section of the cervix, with 3 cm diameter, increasing in size. Lab tests were normal apart from CPK (250 IU/L), serum phosphorus (5,6 mg/dl), and serum calcium (7,8 mg/dl). Thoracic and Cervical MRI reveal a substantial cystic mass (12,5 × 2,2 × 2,3 cm) in anterior mesothorax. Two similar masses were detected one paratracheal (2,6 × 4 cm) and one in the lower cervix (2,4 × 2 cm), all communicating. Lower and upper abdomen CT showed multiple cystic lesions of the spleen, while full MRI check revealed no change in cystic lesions of lumbar vertebrae, with several similar lesions in whole vertebrae column, the clavicle and both femurs. FNA procedure took place and the sample proved to be lymph. FNA in combination with CT and MRI results indicated Gorham–Stout disease.
Patient continued receiving conservative therapy with calcium and salmon calcitonin and after six months calcium levels became normal and bone density reached normal range, improving by far patient's quality of life. Mesothorax and spinal cystic lesions were reevaluated regularly and no change in size was noted.
The next two years patient underwent two incidents which were impossible to properly diagnose. The first consisted of an intense headache, which lasted for 15 days during patient's vacation, with following improvement and the second involved a vision disorder which was examined by an ophthalmologist with no pathology defined.
However, in 2010, the patient was admitted due to intense headache, gradually increasing, affecting patient's quality of life. Although examination revealed no abnormal findings, the patient was deteriorating. A lumbar puncture took place and showed low cerebrospinal fluid (CSF) pressure (<6). Intravenous fluids were administrated and, later, patient improved and was discharged, only to come two days later with severe temporal and cervical cephalalgia and vertigo. Otorhinolaryngological examination was not pathological and patient was treated conservatively with Sibellium, Lonarid-N, and Tramal tablets. Patient improved but further examination took place. Full-body 3D CT scan with intrathecal radioactive polymer infusion revealed the following: (1) incomplete herniation of the brain stem probably due to low CSF pressure; (2) dilation of endothoracic cyst, with more than 20 cm diameter, fully compressing left lung causing functional left pneumonectomy; and (3) possible communication of CSF with mesothoracic cystic mass (Figures , , and ).
Surgical excision was undertaken. Patient underwent thoracoscopic excision of the gargantuan cystic mass, with ligation of major lymph vessels, conserving major thoracic duct. No connection between the cyst and spinal cavity was detected, possibly because during the excision procedure the connection was ligated or shut. Patient had a smooth postoperative course and recovery.


A 54-year-old man presented with worsening sharp pain over his right upper back. The pain had started two weeks back immediately after getting a flu vaccination in his right shoulder. There was no radiation and no aggravating or relieving factors. It was associated with a localized sensation of warmth and occasional chills. He, however, denied any preceding trauma, fever, cough, weakness, numbness, or pain in any other part of the body. Rest of the review of symptoms was negative. His past medical history was significant for hypertension, type 2 DM, morbid obesity with a body mass index of 44 kg/m2, and depression. He was not compliant with his diet nor his medications. At presentation, he was afebrile but, subsequently, he developed a fever with a temperature of 102 F. His blood pressure was 137/75 mm Hg with a pulse rate of 113 beats per minute. His physical examination was significant for an extensive area of induration in the right upper back extending into the back of the neck, measured about 25 cm × 20 cm in size, and had a 3 cm × 4 cm × 3 cm area of fluctuation with erythema and tenderness (). His laboratory investigations were significant for leukocytosis with a white blood cell count of 26,000/μL, lactic acidosis with a serum lactic acid of 2.4 mmol/L (normal 0.5–1.9 mmol/L) and serum bicarbonate of 16 mmol/L (normal 22–32 mmol/L), and poorly controlled diabetes with serum glucose of 291 mg/dL (normal 74–118 mg/dL) and subsequent hemoglobin A1C of 10.3% (normal 4.8%–5.6%). Erythrocyte sedimentation rate was 104 mm/hr (normal 0–20 mm/hr), C-reactive protein was 345.7 mg/L (normal 0–4.9 mg/L), thyroid stimulating hormone level was 1.360 μIU/mL (normal 0.450–4.50 uIU/mL), and free thyroxine level was 5.1 μg/dL (normal 4.5–12 ug/dL). Serum protein electrophoresis (SPEP) showed a normal pattern with no monoclonal protein, and antistreptolysin O (ASO) titer was negative. Chest imaging showed bullous changes in the upper lobes of the lung, a large area of soft tissue density in the subcutaneous tissue of right upper back, and enlarged right trapezius muscle (). In this setting of fever, tachycardia, leukocytosis, and lactic acidosis, a presumptive diagnosis of sepsis likely due to cellulitis of the back with abscess formation was made. The patient was then started on broad-spectrum intravenous antibiotics and taken to the operative room for incision and drainage. His deep-seated abscess was drained and cultured, which subsequently grew Staphylococcus lugdunensis sensitive to methicillin, so the patient was started on intravenous Nafcillin. A skin biopsy taken from the back was sent for histopathology and showed fibroadipose tissue and skeletal muscle with focal acute and chronic inflammation with granulation tissue (). These histopathological findings were consistent with scleredema diabeticorum and was thought to have resulted from his longstanding, poorly controlled diabetes. However, he denied prior symptoms of tightness or discomfort over the shoulders and upper back. The patient was counseled extensively and started on insulin and metformin, and the dosage was optimized during hospitalization. He was also informed about the treatment options for scleredema diabeticorum including immunosuppressants and phototherapies, but he declined any treatment for the time being. And with the sepsis resolved, blood glucose better controlled, and his surgical wound healing, he was discharged home to follow up as an outpatient.


A 58-year-old woman with a history of chronic obstructive pulmonary disease, hypertension, and smoking presented to the emergency department two hours after the onset of severe left-sided weakness with facial droop, dysarthria, right gaze preference, and weakness. Her initial National Institutes of Health Stroke Scale (NIHSS) was 8 by the tele-neurology exam using the spoke and hub robot. The patient was administered intravenous rtPA (IV-rtPA) and transferred to our high-volume comprehensive stroke center for endovascular stroke therapy. The initial head computerized tomography (CT) demonstrated a dense MCA sign on the right (Figure ).
CT angiography confirmed an occlusion of the M1 segment of the right middle cerebral artery in addition to nonruptured anterior communicating (ACOM) and right superior hypophyseal aneurysms (Figure ).
An initial catheter angiography confirmed the occlusion of the M1 segment right MCA with good leptomeningeal collaterals (Figure ).
Given the presence of the clot at the MCA bifurcation and aneurysms of the right superior hypophyseal and anterior communicating locations, a decision was made to stay proximal and use ADAPT (Figure ).
This technique was chosen because it avoids the need for manipulating a stent retriever blindly through the MCA bifurcation in a patient with a higher risk of harboring an additional unidentified aneurysm at the MCA bifurcation. After successfully aspirating the large clot fragment using ADAPT, a bilobed irregular aneurysm was found at the right MCA bifurcation (Figure ).
Recanalization with ADAPT showed an improvement from 0 to 2b on the modified thrombolysis in cerebral infarction (TICI) scale. The patient showed dramatic improvement on the angiography table with a postoperative NIHSS score of 0. The patient was clinically suitable for discharge and sent home after five days with the plan to follow up with the department of neurosurgery to discuss possible future treatments of her multiple nonruptured cerebral aneurysms.


A 9-year-old boy was brought by his father due to disturbed behaviour with attempts to run in front of traffic over the 2 days preceding the presentation. He was overactive and aggressive, hitting and biting others. His sleep was disturbed.
He has been well until 2 months ago, at which time he refused to go to school citing somatic symptoms. He later reported being physically punished by a school teacher which appeared to have led to the school refusal. During this period, he was irritable and anxious. He reported suicidal ideation and threatened to jump into their well on one occasion, following an argument with his mother. He was socially withdrawn. His sleep was disturbed and he had poor appetite. He was diagnosed as having a depressive episode and commenced on sertraline 25 mg mane 3 weeks prior to the current presentation. His father reported some improvement with this in terms of his activity and dose was increased to 50 mg 2 weeks later. After about 5 days, he became very disturbed, attempting to run off. His father brought him to the hospital as they could not safely contain him at home.
He was the older of 2 siblings and lived with his parents and younger sister. There was a family history of bipolar affective disorder in one of his first cousins. His parents were supportive, but there were marital conflicts and he reported witnessing domestic violence between them. His birth and development history were normal and past medical and psychiatric history were unremarkable. His school performance has been average. Although his parents reported him as being hyperactive and stubborn, his school teachers claimed he was a well-behaved child who could concentrate and completed the tasks he was given. There was evidence of inconsistent parenting styles with his father being authoritarian and mother being very permissive.
On mental state examination, he was an average built boy dressed casually. During the initial interview, he attempted to run off and was restrained physically by his father. He was angry and screamed using obscene words. He was admitted to the in-patient unit. Sertraline was ceased. Clonazepam was used for sedation. As his agitation reduced, he was noted to be socially disinhibited. He had pressure of speech. He did not have flight of ideas. He was extremely irritable. But at times he sang and danced in the ward. There was no suicidal ideation. He expressed self-expansive ideas. He did not have any delusions or hallucinations. He was oriented in time, place, and person. He was distractible and could not engage with cognitive testing. He had poor insight. Physical examination including cardiovascular, respiratory, and nervous systems and the abdomen was unremarkable. He did not have any abnormal movements and bilateral fundi were normal.
Investigations including full blood count, C reactive protein, and thyroid function tests were normal. Electroencephalography recorded in the alert state was normal for the age. Contrast enhanced computerized tomography of the brain was normal.
His presentation met criteria for treatment-emergent mania []. He was commenced on risperidone and dose increased to 2 mg twice daily at which point he developed extrapyramidal side effects. As his symptoms persisted beyond one week and there was a positive family history, a diagnosis of bipolar affective disorder, currently manic episode without psychotic symptoms, was made. Sodium valproate was added and dose increased up to 200 mg twice daily. Risperidone was changed to olanzapine after 3 weeks as he continued to have significant emotional and behavioural disturbances; he was extremely irritable and physically aggressive. He showed slow improvement in his mental state after increasing olanzapine to 5 mg twice daily. Following 4 weeks of treatment with olanzapine and sodium valproate, he was discharged home after discussing extensively with his parents regarding the diagnosis, risks, and treatment.
Upon review after 1 week, his parents reported further improvement in his irritability and overactivity and he was continued on the same medication. He was reviewed every 2 weeks for the next month and continued to show improvement in mental state. However, he showed significant weight gain while on treatment. Metabolic screen was done at 4 weeks and investigations were normal. Dietary advice was provided and physical activities were encouraged. Switching to aripiprazole was discussed; however it is not available in the public hospitals in Sri Lanka and parents were not able to afford it. Olanzapine dose was slowly reduced. He then had a relapse of manic symptoms and was commenced on lithium carbonate. Sodium valproate was tailed off. The treating team continued to support parents and liaised with school to ensure smooth transition back to school. His symptoms improved and he was able to go back to school. Olanzapine was tailed off and lithium dose was optimized.


A 75-year-old Caucasian male was referred for evaluation with the following history: coronary artery bypass grafting (CABG) with left internal mammary artery (LIMA) to left anterior descending (LAD) and sequential saphenous vein grafting (SVG) to ramus, obtuse marginal, right posterolateral and right posterior descending arteries, repeat CABG two years later with revision of the SVG, severe peripheral arterial disease (PAD) with a history of left lower extremity surgical revascularization, right iliac artery occlusion, atherosclerotic carotid artery disease, and chronic kidney disease (CKD) Stage III. He had been hospitalized for the treatment of CHF and non-ST elevation myocardial infarction (NSTEMI) within the prior three months and continued to experience increasing angina and dyspnea on exertion (NYHA Class III), which was refractory to medications. He was noted to have had a decrease in LV ejection fraction by echocardiogram from 45% to 15% and underwent cardiac catheterization.
Cardiac catheterization revealed a patent sequential SVG and severe disease of the body of the SA, distal to the left vertebral artery, and involving the LIMA (). The lesion involved the LIMA and the SA, immediately both proximal and distal to the LIMA. Cardiac surgical consultation was obtained, and the patient was deemed a prohibitive risk for a third CABG. Vascular surgical treatment (subclavian-carotid transposition or bypass) was not an option as it would not address the compromised LIMA. Percutaneous therapy directed toward the SA/LIMA bifurcation was planned.
A 6 Fr FR 4.0 Medtronic Launcher coronary guide catheter was placed via the left femoral artery (LFA) into the proximal SA, and a 6-French IMA Medtronic Launcher coronary guide catheter was placed via the left radial artery (LRA) into the SA distal to the LIMA origin. Initial attempts to cross the diffusely calcific SA stenosis anterograde via the LFA, using multiple wire sizes and tips, were unsuccessful. A 0.018 Terumo Glidewire Advantage wire was then placed retrograde via the LRA across the SA stenosis and into the ascending aorta. A 0.014 Terumo Runthrough NS Hypercoat coronary guidewire was placed from the LFA into the LIMA (). The SA was predilated via the LRA. A 5 mm × 28 mm balloon expandable stent (Multi-Link Ultra, Abbott Vascular) was then placed into the SA crossing the ostium of the LIMA, trapping the LIMA guidewire (). This was followed by postdilatation of the stent with a 5 mm × 15 mm noncompliant balloon (NC Trek, Abbott Vascular). A second Terumo Runthrough NS Hypercoat coronary guidewire was then placed through the stent into the LIMA from the LFA, and the trapped guidewire was removed. Multiple unsuccessful attempts were made to pass a balloon through the stent struts into the LIMA from the LFA. Despite the use of small, low profile balloons and a guide extender (Guide Liner, Vascular Solutions), the LIMA could not be accessed from the LFA. At this point, the decision was made to change the angle of approach to the LIMA by attempting percutaneous intervention via the LRA.
A 0.014 Terumo Runthrough NS Hypercoat coronary guidewire was then placed from the LRA through the stent struts into the LIMA. The coronary guidewire which had been placed into the LIMA from the LFA was removed. A 0.035 Terumo Glidewire Advantage wire was then placed from the LFA into the SA.
The stent struts were then dilated via the LRA, and a 3 mm × 12 mm drug-eluting stent (Xience Alpine, Abbott Vascular) was easily passed through. A 5 mm × 15 mm noncompliant balloon (NC Trek, Abbott Vascular) was then placed into the SA stent via the LFA, and a small portion of the LIMA stent was pulled back into the SA stent in order to perform “T and small Protrusion” or TAP stenting [] of the SA/LIMA bifurcation (). A final kissing balloon angioplasty was accomplished, and post-op angiography demonstrated a patent SA/LIMA bifurcation ().
The patient was symptom free with improved LV ejection fraction to 30% by echocardiography at 6 months.


An 83-year-old Caucasian male with past medical history of hypertension, diabetes, and coronary artery disease presented to the emergency department with extreme fatigue, weakness, and disorientation. He reported a history of 10–15-pound weight loss over the previous month. Physical exam did not yield any focal signs. Laboratory evaluation revealed pancytopenia (Hgb 9.6 g/dL, WBC 3.0 bil/L, and platelets 169 bil/L) and increased liver function enzymes (AST 311 U/L, ALT 62 U/L, and ALP 237 U/L). The patient was admitted for generalized weakness and fatigue.
An unenhanced CT scan of the abdomen and pelvis obtained in the emergency department showed a nondistended gallbladder with diffuse wall thickening (). A small calcified gallstone was identified in the neck of the gallbladder. Neither a soft tissue mass nor lymphadenopathy was present. Although acute cholecystitis was not a leading differential diagnosis based on the clinical presentation, HIDA scan was recommended based on the unexpected CT findings.
The patient developed worsening alteration of mental status, metabolic acidosis, further increase in liver function tests, and pancytopenia. D-dimer was elevated (1830 ng/mL). There was initially clinical concern for pulmonary embolism versus disseminated intravascular coagulation. Nuclear medicine testing with ventilation-perfusion (VQ) scan was performed and low probability for pulmonary embolism was found. Coagulation testing remained stable.
The HIDA scan revealed cystic duct patency (). Subsequently, a targeted sonographic examination of the right upper quadrant was performed to further characterize the nonspecific findings on the CT scan. This revealed diffuse gallbladder wall thickening measuring up to 8 mm. The gallbladder wall had a striated appearance with a central hypoechoic stripe surrounded by an echogenic band on either side (). The sonographic Murphy's sign was negative. The gallbladder was decompressed and contained a few small stones. An MRI of the abdomen with contrast showed diffusely thickened gallbladder wall measuring up to 12 mm with intramural increased signal intensity on T2 weighted images. Contrast enhanced T1 weighted sequences showed no abnormal enhancement or nodularity of the gallbladder wall. The gallbladder did contain a few small stones, but there was no luminal distention, pericholecystic fluid, or lymphadenopathy (). Clinical concern further developed for septicemia with the gallbladder as the primary site of origin given the imaging findings. The patient underwent laparoscopic cholecystectomy.
Pathologic evaluation of the surgical specimen revealed findings of intravascular large B-cell lymphoma (IVLBCL), a rare variant of extranodal diffuse large B-cell lymphoma. Histologic evaluation with hematoxylin and eosin stain demonstrated diffuse gallbladder wall thickening with lymphatic ectasia (). Several blood vessels in the subserosal fat showed infiltration with large, atypical lymphomatous cells. The mucosa was uninvolved. These features explained all the imaging findings well in this particular case.
Flow cytometry of the peripheral blood revealed a small population of aberrant B-cells which correlate to the pathologic gallbladder wall findings. Subsequent bone marrow core biopsy and aspiration were performed. Pathologic evaluation demonstrated slight hypercellularity of the marrow and 10–15% involvement by diffuse large B-cell lymphoma. Immunohistochemical stain of bone marrow core biopsy sample was positive for CD79a, confirming B-cell origin.
Postoperatively, the patient's condition continued to deteriorate requiring respiratory support in the intensive care unit. He developed severe metabolic acidosis and hemodynamic instability. Given the patient's poor functional status and concomitant diagnosis of IVLBCL, the decision to withdraw supportive care was made by his family. The patient expired 7 days after initial presentation.


- Diagnosis and etiology
A girl aged 10 years 4 months presented with a slightly concave profile, lack of upper labial support, highly accentuated peribuccal sulcus, good lip seal and acceptable facial asymmetry. The patient had a good overall state of health with no history of systemic disease, trauma or deleterious habits. The intraoral exam revealed: ½ Class II molar relationship, four missing permanent maxillary incisors, anterior crossbite, permanence of deciduous maxillary canines and transmigration of permanent maxillary canines into the region of the maxillary incisors (Fig. A,B).
The patient sought treatment for poor anterior teeth esthetics and anterior cross-bite rendering the patient too embarrassed to smile. Analysis of the initial panoramic radiograph taken at 8 years 6 months disclosed the presence of 2 deciduous maxillary central incisors but absence of maxillary permanent incisor buds and transmigration of the maxillary canine buds mesially (Fig. C). The patient´s mother confirmed that the maxillary deciduous lateral incisors had always been missing. Panoramic radiographs and lateral teleradiography taken prior to orthodontic treatment disclosed almost complete eruption of the canines in the position of the maxillary central incisors and a poor relationship of the arches owing to missing anterior teeth, i.e., due to short maxillary arch, with no significant sagittal maxillomandibular discrepancy.
- Treatment objectives
The following treatment objectives were established: (1) to establish an esthetic facial profile; (2) to resolve the anterior crossbite; (3) to obtain a stable occlusal relationship; and (4) to improve dental esthetics, establishing an esthetic smile.
- Treatment alternatives
Three treatment options were presented to the parents. First, distalization of the maxillary molars using an extrabuccal arch to correct the molar relationship, extraction of the deciduous canines, moving of the permanent canines into the position of the deciduous canines, fabrication of temporary prostheses until the patient reaches sufficient bone maturity to allow placement of four implants and fabrication of prostheses in the region of the four maxillary incisors. This option was rejected by the parents for financial reasons and owing to the lengthy orthodontic treatment required until implant placement.
The second option involved mesialization of the maxillary molars to achieve a Class II molar relationship, whereby the first two maxillary premolars substitute the maxillary canines, the maxillary canines substitute the maxillary central incisors, with the required esthetic and functional rehabilitation and extraction of the deciduous canines, and space opening for provisional teeth in maxillary lateral incisors until the patient reaches sufficient bone maturity to allow placement of two implants and fabrication of prostheses in the region of the two lateral incisors. This option was also rejected, mainly owing to the orthodontic treatment time required until placement of prostheses on implants.
The third option involved mesialization of the maxillary molars to achieve a Class II molar relationship, whereby the first two maxillary premolars substitute the maxillary canines, the maxillary canines substitute the maxillary central incisors and the deciduous canines are moved to the region of the lateral incisors, with all these teeth submitted to the required esthetic and functional rehabilitation. This third option was elected.
- Treatment progress
Treatment commenced by bracket bonding with the MBT prescription, banding of the first molars, taking care not to bond any brackets to deciduous canines thereby avoiding the risk of overload and exfoliation. The MBT prescription was chosen for its incisor bracket angulation, which can help correct the anterior crossbite. Maxillary central incisor brackets were bonded to maxillary canines, taking into account end of treatment, to allow better positioning of these teeth for torque, angulation and height.
In the first phases of the treatment, the teeth were aligned and leveled using 0.012-in and 0.016-in nickel-titanium wires (Fig. A). Nickel-titanium open-coil springs were employed to obtain the ideal spacing equivalent to the four maxillary incisors for future rehabilitation of the teeth. After round nickel-titanium wires, 0.016 X 0.020-in stainless steel wire were used, interspersed with rectangular section 0.017 x 0.025-in nickel-titanium wire, finishing with 0.019 x 0.025-in stainless steel wire. During this phase, the patient was instructed to use Class III intermaxillary elastics.
The whole fixed apparatus was then removed and during the same session, a 3 x 3 retainer was fabricated in the lower arch anchored by the lingual aspect of the anterior teeth. A mock-up was first performed (Fig. B), and after approval of the results, teeth 13 and 23, previously moved to substitute the maxillary central incisors, were prepared and fitted with feldspathic porcelain laminate veneers. Teeth 53 and 63 were also fitted with feldspathic porcelain veneers, assuming the position and function of the maxillary lateral incisors. Teeth 14 and 24 were reshaped with direct composite resins and assumed the function and position of the maxillary canines, with adjustment for group function when performing laterality.
- Treatment results
At end of treatment, all goals were achieved. There was an improvement in the patient´s facial profile from slightly concave to straight. The crossbite problem was resolved. Final intercuspidation of the teeth was highly satisfactory. The esthetic rehabilitation of all maxillary teeth restored the patient´s self-esteem owing to the major esthetic improvement (Fig. ). The patient was asked whether the mandibular third molars could be extracted, since these were impacted and inclined mesially.
The patient was highly satisfied with the results obtained, particularly the esthetic and functional correction. However, the patient and her parents were advised about possible mobility of the deciduous canines and made aware of the likely need to replace these two teeth with implants at a later date.


A previously healthy 42-year-old woman with fever, headache and sore throat for three days developed sudden bilateral visual loss and dysarthria, and lapsed into confusion and severe irritability that required sedation. Examination at that time showed tachycardia (142 beats/minute), body temperature of 39.6°C, neck stiffness, and tender maculopapular rashes in both hands and feet (Janeway lesion, Figure A). Fundus examination disclosed multiple scattered retinal hemorrhages with a white center (Roth spots) in both eyes (Figure B). Hematologic assays showed leukocytosis of 12,700/μL (neutrophil 94%), hemoglobin of 9.6 g/dL (hematocrit at 29.9%), and platelet counts at 135,000/μL. CSF study disclosed pleocytosis (1,020/mm3, neutrophil 80%), RBC of 200/mm3, protein at 166.0 mg/dL, and glucose of 55 mg/dL (serum glucose at 122 mg/dL). Under the suspicion of bacterial meningitis, the patient was placed on 2.6 g of vancomycin and 4 g of ceftriaxone per day. A methicillin-resistant Staphylococcus aureus was identified on blood culture later. Subsequent transthoracic echocardiography revealed a hypermobile vegetation (1.7 cm × 1.1 cm) attached to the mitral valve, which led to an operation for a valve replacement 20 days after the symptom onset.
On regaining consciousness 1 day after the operation, she reported dizziness, and hearing loss and tinnitus in the right ear. Examination showed no SN nystagmus either with or without visual fixation, but horizontal head-shaking induced left beating nystagmus (Figure C). Horizontal saccades and smooth pursuit were normal. She tended to fall to the right on attempted standing or walking. Other neurologic examination was unrevealing without weakness, sensory changes, or cerebellar dysfunction. Video HITs were abnormal for the right horizontal and posterior canals. Bithermal caloric tests showed right canal paresis of 81% (Figure D). Pure-tone audiometry documented right hearing loss (Figure E). SVV was normal. Cervical and ocular VEMPs showed no responses during right ear stimulation. Brain MRIs disclosed gadolinium enhancements of the vestibule, cochlea, and semicircular canals on the right side (Figure F) as well as multiple scattered infarctions in the frontal and occipital lobes (Figure , 1). The findings of MR angiography were normal. These findings were consistent with multiple infarctions due to septic embolism from cardiac vegetation. After 6 weeks of IV vancomycin and anticoagulation, she was discharged with minimal dizziness left although the tinnitus and hearing loss remained unchanged.


A 73-year-old man with a history of adrenal insufficiency, operations for colon cancer, patent foramen ovale, and pericardiac effusion was referred for further evaluation of acute vertigo and acute infarction observed on MRIs. He had developed acute vertigo and nausea/vomiting on the way to the bathroom just after awakening in the morning 6 days before the referral. He denied associated tinnitus, aural fullness, hearing loos, or sensorimotor symptoms. The vertigo had lasted into the afternoon with severe imbalance, and subsided after symptomatic medication with a diagnosis of vestibular neuritis at a local hospital. He was then referred to our ENT department where the MRI and MR angiography were arranged, and acute infarction was identified.
On evaluation 6 days after symptom onset, he showed SN nystagmus beating leftward, upward, and counterclockwise without fixation (Figure A). Bedside HITs were positive for the right horizontal canal. Rightward ocular torsion was observed on fundus photos (Figure B). SVV was tilted to the right either monocular [13.9 with right eye (normal range = −3.1 to 3.0), 16.0 with left eye (normal range = −3.8 to 3.1), positive values indicate a rightward tilt] or binocular viewing (13.9, normal range = −2.4 to 2.6). Bithermal caloric tests showed complete right canal paralysis (Figure C). Ocular VEMPs showed no responses during right ear stimulation (Figure D), but findings of cervical VEMPs were symmetric. Audiometry was normal (Figure E). Brain MRIs disclosed a tiny acute infarction in the tail portion of left hippocampus (Figure , 3). The findings of MR angiography were normal.


A 33-year-old woman, G0P0, who complained of atypical vaginal bleeding and abdominal pain during a period of 4 months was referred from a local clinic. The patient was 165 cm in height, weighed 100 kg, and had a body mass index of 36.7 kg/m2. Her past medical history included schizophrenia treated with risperidone and an appendectomy at the age of 8. An endometrial biopsy revealed differentiated endometrioid carcinoma with trophoblastic components. Then, she was referred to our hospital. Physical examination revealed the presence of a solid mass measuring 8 cm in the left ovary and anther tumor mass in the uterus. Transvaginal ultrasonography showed a mixed echogenic mass in the left ovarian, size 138 x 90 x 65 mm; a mass invading the uterine, size 64 x 54 x 53 mm. Magnetic resonance imaging (MRI) of the pelvic revealed an ill-defined large heterogeneous soft tissue masses in the uterus and left adnexa area. Preoperative chest computed tomographic (CT) scan was negative. Because of the histologic and imaging results, both of the serum β-hCG and AFP were tested and found to be elevated (β-hCG: 238,418.35 U/L, normal value ≤ 4 U/L; AFP: 800.10 ng/mL, normal value ≤ 7 ng/mL). Other markers including carbohydrate antigen (CA)-125 and neuron-specific enolase (NSE) were within the normal range. She underwent abdominal surgical treatment, and the left ovary and fallopian tube were removed and sent for intraoperative consultation. On frozen section, a poorly differentiated ovarian malignant tumor was diagnosed. Then, total hysterectomy with contralateral salpongo-oophorectomy, total omentectomy, and systemic pelvic lymphadenectomy was performed.
Gross morphologically, the uterus measured 16 x 10 x 5 cm. Two protruding masses were found in the endometrial cavity. Both of the 2 polypoid masses (size 6 x 5 x 5 cm and 2 x 1 x 1 cm, respectively) were infiltrating into the myometrium. And the smaller one was extending from the fundus to the cervix. The left ovarian tumor (12 x 10 x 6 cm) was involved with large necrosis and hemorrhages.
Microscopically, both of the 2 masses in the uterus were composed predominately of intermediated-sized, poorly differentiated cells without obvious nested or trabecular patterns. The tumors had deeply (> 50%) infiltrated the myometrium and showed extensive necrosis (Fig. A). In addition, multinucleated choriocarcinoma-like cells were identified in the area of confluent necrosis. Involvement of undifferentiated carcinoma (UC) components was also identified in the left ovary (Fig. B). However, fewer syncytiotrophoblasts were presented in ovarian lesion than that in the uterine lesion. Lymphovasular invasion was found. The tumor was diagnosed as DEAC metastatic to the left ovary. According to the International Federation of Gynecology and Obstetric (FIGO) system, the case was considered FIGO stage IIIA. A panel of immunohistochemical (ICH) analysis was performed to confirm the histologic diagnosis (Fig. A–D). The results of ICH analysis are summarized in Table .[ In the uterus, focal positivity for cytokeratine (CK7), epithelia membrane antigen (EMA), and vimentin was detected in the UC components suggesting epithelia neoplasms. Markers such as β-hCG, human placental lactogen (HPL), and alkaline placental phosphatase (PLAP) were positive in the multinucleated choriocarcinoma-like cells. PAX8, estrogen receptor (ER), and progesterone receptor (PR) staining were absent in UC components. And, AFP and CD30 status were both negative.
Both the serum β-hCG and AFP were dramatically decreased postoperatively (Fig. ). Unfortunately, 3 weeks postoperatively, nodules were identified in the lungs by chest CT, with associated elevations in her serum β-hCG. No adjuvant chemotherapy was given since the patient declined. She was dead of extensive disease approximately 5 months after her primary surgery.