Search

Objective: To study the relationship between consumption of milk and milk products, calcium, lactose and vitamin D and occurrence of colorectal cancers. Design: Prospective cohort study. Subjects: A total of 9959 men and women aged 15 y or older without history of cancer at baseline. During a 24 y follow-up, 72 new cancers of the large bowel (38 in the colon and 34 in the rectum) were detected. Results: Consumption of milk and total milk products was suggested to be inversely related to colon cancer incidence, whereas no similar association was seen for rectal cancer. The relative risk between the highest and lowest quartiles of intake adjusted for potential confounding factors was 0.46 (95% confidence interval 0.14–1.46, P for trend 0.09) for milk and 0.37 (95% CI=0.12–1.39, P for trend 0.06) for total milk products. Lactose intake showed a similar inverse relationship with colon cancer: the relative risk was 0.31 (95% CI=0.08–1.15, P for trend 0.03). Intake of vitamin D or total dietary calcium was not significantly related to colorectal cancer risk, whereas calcium provided by fermented milk products was associated with increased colorectal cancer incidence; in the highest quartile the multivariate adjusted relative risk for colorectal cancer was 2.07 (95% CI=1.00–4.28). Conclusions: Our results indicate that individuals showing high consumption of milk have a potentially reduced risk of colon cancer; however, the association does not appear to be due to intake of calcium, vitamin D, or to specific effects of fermented milk. Sponsorship: This study was supported by a grant from the Swedish Cancer Foundation. European Journal of Clinical Nutrition (2001) 55, 1000–1007

Objective: To demonstrate sexual dimorphism in serum leptin levels not only during puberty, but also in childhood in Japan. Design: Cross-sectional study. Setting: Hamamatsu-Hokuen study in Japan. Subjects: Body weight and height were measured in normal-weight Japanese children and adolescents (143 boys, 178 girls), and 161 boys and 129 girls whose percentage of overweight for the standard (%Wt) was more than+25%. Serum leptin levels were compared with %Wt. Subjects were divided into group 1 (6–10 y of age) and group 2 (11–15 y of age) according to their age. Results: In overweight subjects, leptin was more highly correlated with %Wt in boys of group 2 (r=0.67, P

Sex differences are commonly observed in behaviors that are closely linked to adaptive function, but sex differences can also be observed in behavioral "building blocks" such as locomotor activity and reward processing. Modern neuroscientific inquiry, in pursuit of generalizable principles of functioning across sexes, has often ignored these more subtle sex differences in behavioral building blocks that may result from differences in these behavioral building blocks. A frequent assumption is that there is a default (often male) way to perform a behavior. This approach misses fundamental drivers of individual variability within and between sexes. Incomplete behavioral descriptions of both sexes can lead to an overreliance on reduced "single-variable" readouts of complex behaviors, the design of which may be based on male-biased samples. Here, we advocate that the incorporation of new machine-learning tools for collecting and analyzing multimodal "big behavior" data allows for a more holistic and richer approach to the quantification of behavior in both sexes. These new tools make behavioral description more robust and replicable across laboratories and species, and may open up new lines of neuroscientific inquiry by facilitating the discovery of novel behavioral states. Having more accurate measures of behavioral diversity in males and females could serve as a hypothesis generator for where and when we should look in the brain for meaningful neural differences.

PURPOSE Cisplatin plus gemcitabine is a standard regimen for first-line treatment of advanced non–small-cell lung cancer (NSCLC). Phase II studies of pemetrexed plus platinum compounds have also shown activity in this setting. PATIENTS AND METHODS This noninferiority, phase III, randomized study compared the overall survival between treatment arms using a fixed margin method (hazard ratio [HR] < 1.176) in 1,725 chemotherapy-naive patients with stage IIIB or IV NSCLC and an Eastern Cooperative Oncology Group performance status of 0 to 1. Patients received cisplatin 75 mg/m2 on day 1 and gemcitabine 1,250 mg/m2 on days 1 and 8 (n = 863) or cisplatin 75 mg/m2 and pemetrexed 500 mg/m2 on day 1 (n = 862) every 3 weeks for up to six cycles. RESULTS Overall survival for cisplatin/pemetrexed was noninferior to cisplatin/gemcitabine (median survival, 10.3 v 10.3 months, respectively; HR = 0.94; 95% CI, 0.84 to 1.05). Overall survival was statistically superior for cisplatin/pemetrexed versus cisplatin/gemcitabine in patients with adenocarcinoma (n = 847; 12.6 v 10.9 months, respectively) and large-cell carcinoma histology (n = 153; 10.4 v 6.7 months, respectively). In contrast, in patients with squamous cell histology, there was a significant improvement in survival with cisplatin/gemcitabine versus cisplatin/pemetrexed (n = 473; 10.8 v 9.4 months, respectively). For cisplatin/pemetrexed, rates of grade 3 or 4 neutropenia, anemia, and thrombocytopenia ( P ≤ .001); febrile neutropenia ( P = .002); and alopecia ( P < .001) were significantly lower, whereas grade 3 or 4 nausea ( P = .004) was more common. CONCLUSION In advanced NSCLC, cisplatin/pemetrexed provides similar efficacy with better tolerability and more convenient administration than cisplatin/gemcitabine. This is the first prospective phase III study in NSCLC to show survival differences based on histologic type.

Purpose To investigate the efficacy and safety of bevacizumab plus carboplatin and paclitaxel in patients with advanced or recurrent non-small-cell lung cancer. Patients and Methods In a phase II trial, 99 patients were randomly assigned to bevacizumab 7.5 (n = 32) or 15 mg/kg (n = 35) plus carboplatin (area under the curve = 6) and paclitaxel (200 mg/m2) every 3 weeks or carboplatin and paclitaxel alone (n = 32). Primary efficacy end points were time to disease progression and best confirmed response rate. On disease progression, patients in the control arm had the option to receive single-agent bevacizumab 15 mg/kg every 3 weeks. Results Compared with the control arm, treatment with carboplatin and paclitaxel plus bevacizumab (15 mg/kg) resulted in a higher response rate (31.5% v 18.8%), longer median time to progression (7.4 v 4.2 months) and a modest increase in survival (17.7 v 14.9 months). Of the 19 control patients that crossed over to single-agent bevacizumab, five experienced stable disease, and 1-year survival was 47%. Bleeding was the most prominent adverse event and was manifested in two distinct clinical patterns; minor mucocutaneous hemorrhage and major hemoptysis. Major hemoptysis was associated with squamous cell histology, tumor necrosis and cavitation, and disease location close to major blood vessels. Conclusion Bevacizumab in combination with carboplatin and paclitaxel improved overall response and time to progression in patients with advanced or recurrent non-small-cell lung cancer. Patients with nonsquamous cell histology appear to be a subpopulation with improved outcome and acceptable safety risks.

PURPOSE Patients with malignant pleural mesothelioma, a rapidly progressing malignancy with a median survival time of 6 to 9 months, have previously responded poorly to chemotherapy. We conducted a phase III trial to determine whether treatment with pemetrexed and cisplatin results in survival time superior to that achieved with cisplatin alone. PATIENTS AND METHODS Chemotherapy-naive patients who were not eligible for curative surgery were randomly assigned to receive pemetrexed 500 mg/m2 and cisplatin 75 mg/m2 on day 1, or cisplatin 75 mg/m2 on day 1. Both regimens were given intravenously every 21 days. RESULTS A total of 456 patients were assigned: 226 received pemetrexed and cisplatin, 222 received cisplatin alone, and eight never received therapy. Median survival time in the pemetrexed/cisplatin arm was 12.1 months versus 9.3 months in the control arm ( P = .020, two-sided log-rank test). The hazard ratio for death of patients in the pemetrexed/cisplatin arm versus those in the control arm was 0.77. Median time to progression was significantly longer in the pemetrexed/cisplatin arm: 5.7 months versus 3.9 months ( P = .001). Response rates were 41.3% in the pemetrexed/cisplatin arm versus 16.7% in the control arm ( P < .0001). After 117 patients had enrolled, folic acid and vitamin B12 were added to reduce toxicity, resulting in a significant reduction in toxicities in the pemetrexed/cisplatin arm. CONCLUSION Treatment with pemetrexed plus cisplatin and vitamin supplementation resulted in superior survival time, time to progression, and response rates compared with treatment with cisplatin alone in patients with malignant pleural mesothelioma. Addition of folic acid and vitamin B12 significantly reduced toxicity without adversely affecting survival time.

Key Points Question Are results of universal and selective screening for suicide risk implemented in a pediatric emergency department associated with future suicidal behaviors? Findings In this cohort study of 15 003 youths aged 8 to 18 years, positive screens were significantly associated with subsequent suicide-related hospital visits compared with standard emergency department procedures. Screening also more than doubled the detection of suicide risk compared with treatment as usual. Meaning These findings suggest that screening for suicide risk in pediatric emergency departments is an effective approach to identify risk for subsequent suicide-related emergency department visits., This cohort study examines the association between results of universal and selective screening for suicide risk in a pediatric emergency department using the Ask Suicide-Screening Questions (ASQ) instrument and subsequent suicide-related outcomes., Importance According to National Patient Safety Goal 15.01.01, all individuals being treated or evaluated for behavioral health conditions as their primary reason for care in hospitals and behavioral health care organizations accredited by The Joint Commission should be screened for suicide risk using a validated tool. Existing suicide risk screens have minimal or no high-quality evidence of association with future suicide-related outcomes. Objective To test the association between results of the Ask Suicide-Screening Questions (ASQ) instrument in a pediatric emergency department (ED), implemented through selective and universal screening approaches, and subsequent suicide-related outcomes. Design, Setting, and Participants In this retrospective cohort study at an urban pediatric ED in the United States, the ASQ was administered to youths aged 8 to 18 years with behavioral and psychiatric presenting problems from March 18, 2013, to December 31, 2016 (selective condition), and then to youths aged 10 to 18 years with medical presenting problems (in addition to those aged 8-18 years with behavioral and psychiatric presenting problems) from January 1, 2017, to December 31, 2018 (universal condition). Exposure Positive ASQ screen at baseline ED visit. Main Outcomes and Measures The main outcomes were subsequent ED visits with suicide-related presenting problems (ie, ideation or attempts) based on electronic health records and death by suicide identified through state medical examiner records. Association with suicide-related outcomes was calculated over the entire study period using survival analyses and at 3-month follow-up for both conditions using relative risk. Results The complete sample was 15 003 youths (7044 [47.0%] male; 10 209 [68.0%] black; mean [SD] age, 14.5 [3.1] years at baseline). The follow-up for the selective condition was a mean (SD) of 1133.7 (433.3) days; for the universal condition, it was 366.2 (209.2) days. In the selective condition, there were 275 suicide-related ED visits and 3 deaths by suicide. In the universal condition, there were 118 suicide-related ED visits and no deaths during the follow-up period. Adjusting for demographic characteristics and baseline presenting problem, positive ASQ screens were associated with greater risk of suicide-related outcomes among both the universal sample (hazard ratio, 6.8 [95% CI, 4.2-11.1]) and the selective sample (hazard ratio, 4.8 [95% CI, 3.5-6.5]). Conclusions and Relevance Positive results of both selective and universal screening for suicide risk in pediatric EDs appear to be associated with subsequent suicidal behavior. Screening may be a particularly effective way to detect suicide risk among those who did not present with ideation or attempt. Future studies should examine the impact of screening in combination with other policies and procedures aimed at reducing suicide risk.

Cystic hydatid disease or cystic echinococcosis (CE) is a globally endemic zoonosis caused by the larval cyst stage of the tapeworm Echinococcus granulosus. Concomitant presence of CE and hepatocellular carcinoma (HCC) is a rare clinical scenario. A 70-year-old male patient presented with acute abdominal pain to the surgical outpatient department. On evaluation, a cystic lesion with solid components and free fluid in the abdomen was observed, which led to multiple differentials in the working diagnosis. A CT showed the mass to have a delayed enhancement. Surgical exploration revealed a partially ruptured hydatid cyst with daughter cysts in the abdominal cavity and a solid-component mass lesion. We proceeded with a right partial hepatectomy. Pathological evaluation revealed a pale mass lesion with a large collapsed cyst. HCC with unusual dense fibrillar fibrosis and cystic interface with normal parenchyma was observed. This case connects the multimodal assessment of radiology, surgery and pathology.

Objective To compare outcomes after surgery between patients who were not prescribed opioids and patients who were prescribed opioids. Summary of background data Postoperative opioid prescriptions carry significant risks. Understanding outcomes among patients who receive no opioids after surgery may inform efforts to reduce these risks. Methods We performed a retrospective study of adult patients who underwent surgery between January 1, 2019 and October 31, 2019. The primary outcome was the composite incidence of an emergency department visit, readmission, or reoperation within 30 days of surgery. Secondary outcomes were postoperative pain, satisfaction, quality of life, and regret collected via postoperative survey. A multilevel, mixed-effects logistic regression was performed to evaluate differences between groups. Results In a cohort of 22,345 patients, mean age (standard deviation) was 52.1 (16.5) years and 13,269 (59.4%) patients were female. About 3175 (14.2%) patients were not prescribed opioids, of whom 422 (13.3%) met the composite adverse event endpoint compared to 2255 (11.8%) of patients not prescribed opioids (P = 0.015). Patients not prescribed opioids had a similar probability of adverse events {11.7% [95% confidence interval (CI) 10.2%-13.2%] vs 11.9% (95% CI 10.6%-13.3%]}. Among 12,872 survey respondents, patients who were not prescribed an opioid had a similar rate of high satisfaction [81.7% (95% CI 77.3%-86.1%) vs 81.7% (95% CI 77.7%-85.7%)] and no regret [(93.0% (95% CI 90.8%-95.2%) vs 92.6% (95% CI 90.4%-94.7%)]. Conclusions Patients who were not prescribed opioids after surgery had similar clinical and patient-reported outcomes as patients who were prescribed opioids. This suggests that minimizing opioids as part of routine postoperative care is unlikely to adversely affect patients.

The formation of an intraperitoneal pseudocyst as a complication of ventriculoperitoneal shunts is well known. However, the formation of a pseudocyst at the subcutaneous extraperitoneal abdominal space is unusual and likely secondary to the migration of the peritoneal catheter. We present a 53-year-old male who had placement of a ventriculoperitoneal shunt for hydrocephalus secondary to a vestibular schwannoma. Five months later, he presented with an enormously distended abdomen. Investigations showed the peritoneal catheter in the extraperitoneal space within a large right lower quadrant abdominal wall pseudocyst. The patient was taken to the operating theatre, and the shunt was externalised at the original abdominal incision. Approximately 3 L of cerebrospinal fluid were aspirated from the distal peritoneal catheter. After negative cultures, a new peritoneal catheter was placed intraperitoneally at the contralateral lower abdominal quadrant. The contralateral quadrant was utilised to prevent fluid accumulation into the old extraperitoneal cavity.

Mandibular fractures are rare in infants, and diagnosis can be easily missed due to the difficulty in obtaining an adequate history and the subtle signs. A high index of suspicion and detailed history taking from the caregiver are mandatory to pick up these cases.There are a plethora of management options that have been reported in dealing with such fractures. They range from conservative management to internal fixation by absorbable plates. While conservative management does not interfere with mandibular growth and teeth development, any surgical intervention can carry this risk. Nevertheless, a severely displaced fracture may need anatomical reduction and fixation to allow early nutrition.This study reports a 3-month-old male infant with a fracture in the mandibular symphysis who underwent reduction of the fracture and circummandibular fixation using immobilisation by an acrylic splint for 4 weeks. His long-term follow-up after 20 months showed adequate dentition with proper healing of the fracture site.

Tension gastrothorax is a form of obstructive shock resulting from increased intrathoracic pressure due to a distended herniated stomach. The clinical features of tension gastrothorax are similar to the clinical features of the more common tension pneumothorax. Clinical recognition of this trauma has remained difficult especially in the tropics where most responders are not specialists. We managed a 31-year-old male who, in addition to typical features of obstructive shock secondary to increased intrathoracic pressures, had a recent meal prior to the trauma and a scaphoid abdomen at presentation. We argue that a history of a recent meal before trauma and an unusually scaphoid abdomen could be suggestive of tension gastrothorax and may help to differentiate it from tension pneumothorax. We recommend improved emergency preparedness to help recognise and treat this pathology.

Adrenoleukodystrophy (ALD) is an X linked recessive genetic disorder caused by an abnormality in the ABCD1 gene on the X chromosome, that affects 1 in 20 000 people. In X linked adrenoleukodystrophy (X-ALD), a defect in lignoceroyl-coenzyme A ligase causes pathognomonic tissue accumulation of very long chain fatty acids (VLCFA) in the adrenal cortex and nervous system. The phenotypic variability ranges from cerebral inflammatory demyelination of childhood onset, leading to death within 5 years, to adults remaining presymptomatic through more than five decades. Our case is that of a man who was previously diagnosed with bipolar affective disorder presented with dystonic posturing. During transit, he had an episode of generalised convulsive status epilepticus. He presented with spasticity and exaggerated reflexes. Three important signs of adrenal insufficiency were observed: hypotension, hyperpigmentation and comatose state. The diagnosis of X-ALD should be considered in young men presenting with gradually progressive unexplained cognitive and behavioural problems, a strong family history, adrenal insufficiency, bilateral upper motor signs with absent ankle reflexes.

Primary amyloidosis is a rare systemic disorder often associated with multiple organ dysfunction. The most common form, light chain amyloidosis, has an estimated age-adjusted incidence of 5.1–12.8 cases per million person-years. Spine involvement is extremely uncommon. We present the case of a young Asian man with newly diagnosed amyloidosis involving the lumbar spine among multiple organs with a pathological vertebral fracture that required urgent spine surgery. We believe this is the first reported case to discuss the perianaesthetic challenges in the management of lumbar spine amyloidosis.

A 60-year-old man presented with severe abdominal pain, two episodes of massive haematemesis and chest discomfort. CT angiography showed a saccular aneurysm of the juxtaphrenic aorta with possible oesophageal erosion. Upper GI endoscopy revealed external compression of the lower oesophagus—near total luminal obstruction with impending rupture of the aortic aneurysm. Emergency aneurysmal repair by interposition grafting using 20 mm Dacron graft with oesophageal–gastric reconstruction done. Postoperative period was uneventful

Mycotic aneurysms are rare and if left untreated, can have devastating outcomes. In this case, a 72-year-old man presented to hospital with fevers, night sweats and abdominal pain. A CT scan revealed the development an infrarenal pseudoaneurysm over the course of 8 weeks, increasing from 2.8 cm to a 3.1 cm. The aneurysm was not present on a CT scan performed 6 months earlier. The patient underwent an emergency endovascular repair of the aortic aneurysm (EVAR) and was placed on broad-spectrum antibiotics. Intra-aortic blood cultures aspirated adjacent to the aneurysm and tissue biopsy confirmed tuberculosis bovis as the cause of the mycotic aneurysm. The patient had been treated with intravesical BCG for transitional cell carcinoma of the bladder several months prior. The patient was treated with an extended course of antituberculosis medication. He recovered well and was back to his baseline function within weeks.

We report a case of a 60-year-old man who struggled with frequent migraines for 12.5 years, which were refractory to all conventional therapies. Six months before initial consultation, these migraines become chronic. The patient was then advised to follow the Low Inflammatory Foods Everyday (LIFE) diet, a nutrient-dense, dark green leafy vegetable-rich, whole food plant-based diet. Within 2 months, his headache frequency declined from 18 to 24 headache days per month to 1, and he discontinued his preventive and abortive migraine medications. After 3 months, the patient had no headaches. These results far exceed the goal of migraine treatment with medication, which is to reduce migraine frequency by >50% per month. In addition, the results were durable; this patient has been migraine-free for 7.5 years. Serum beta-carotene more than tripled after the patient started the LIFE diet, consistent with its high content of dark green leafy vegetables. Weight, high-sensitivity C-reactive protein (hsCRP), complete blood count (CBC), hydration status, sodium and other electrolytes remained constant throughout the study.

A 73-year-old man presented to the emergency department with lethargy and influenza-like symptoms. Incidentally, prominent blue sclera and blue-grey skin discolouration to the periorbital skin, pinnae, neck, upper and lower limbs, hands, feet, fingernails and toenails were noted. His general practitioner (GP) had previously ceased amiodarone, believing it to be the causative agent. A literature search confirms the side effects were likely due to minocycline, which the patient had been taking for 10 years. Long-term minocycline use is associated with scleral and skin hyperpigmentation, with no apparent adverse effect on ocular structure or function. The pigmentation may reverse with cessation of minocycline, or it may be permanent. Amiodarone may also cause skin hyperpigmentation, but scleral pigmentation is not a known association. This case report explores the side effect profiles of these two drugs, and highlights the potential for confusion regarding causative agents when used concurrently.

A 4-week-old boy presented to the hospital with symptoms of diarrhoea and vomiting initially thought to be due to cow’s milk allergy. He was discharged with extensively hydrolysed formula. The patient represented with worsening of symptoms with metabolic acidosis and was screened and treated for sepsis. However, his condition deteriorated further and he developed methaemoglobinaemia. He was transferred to the high dependency unit and was given two doses of methylene blue. Further investigations were carried out, including rapid trio exome sequencing, which identified a homozygous pathogenic Peptidase D (PEPD) variant (c.978G>A, p.(Trp326*)). This was consistent with a diagnosis of prolidase deficiency.

Multiple myeloma (MM) patients live longer due to more effective treatment, and we now see previously uncommon manifestations of MM, like extramedullary disease. We present a case of a 74-year-old man known with MM that relapsed with extramedullary manifestations at different locations. One of them as a gastric plasmacytoma (GP). He was successfully treated with chemoradiotherapy (Daratumumab, Bortezomib and Dexamethasone), which resulted in clinical response for 8 months, confirmed by biopsy and histopathology. Perforation of the GP occurred, and he underwent partial gastrectomy (Billroth II gastrojejunostomy). The patient’s disease progressed again 5 months after surgery, and he did not want any additional treatment. He accepted palliative care and died 10 months after the operation. A lack of knowledge about the characteristics and treatment of extramedullary MM exists, and prospective studies to investigate incidence, prognosis and treatment for extramedullary MM are needed for improving the poor prognosis of this manifestation.

A 75-year-old man presented with a 3-week history of melaena and right upper quadrant pain. This was on a background of significant alcohol intake and a complex medical history. He was haemodynamically unstable with investigations indicating a new iron-deficiency anaemia. After resuscitation, urgent intervention was required under general anaesthesia. This involved a triple phase abdominal CT, followed by emergency oesophagogastroduodenoscopy. This revealed deep ulceration with extension to the pancreatic head and common bile duct. There was also evidence of pneumobilia on CT, secondary to a choledochoduodenal fistula. Treatment encompassed an invasive and medical approach. Following treatment, the patient was stable, with follow-up endoscopy exhibiting good duodenal mucosal healing.

Charcot’s joint is a type of neuro-arthropathy, where asymmetrical damage of the involved joint happens haphazardly, without following any described pattern. We present a rare case of Charcot’s joint involving the knee joint in an adult male following spina bifida. His knee joint was successfully fused after two surgeries. Later, he sustained a fracture through the arthrodesis after a fall, which we managed surgically. The fracture through the knee arthrodesis was managed surgically by an open reduction and internal fixation, using a 14-hole broad low contact dynamic compression locking plate with bone grafting. Union was achieved at the knee arthrodesis site in 6 months. Fracture through a fused knee requires surgical management. Re-arthrodesis was done using a stable fixation. Postoperative rehabilitation should include protected weight bearing with braces and splints until a sound bony union is achieved.

We present the case of a young male patient who presented with paralysing muscle weakness due to severe hypokalaemia and hypophosphataemia. The initial patient history evaluations could not establish the aetiology. Only after we reviewed the patient’s history did he reveal that he had been following a severe calorie-restricted regime, the human chorionic gonadotropin diet, which had ended 2 days prior to developing symptoms. This information then allowed us to diagnose severe refeeding syndrome. As a further complication, the patient developed rhabdomyolysis. After correction of serum electrolytes, symptoms resolved completely. This case emphasises the potential harm of severely calorie-restricted diets, often recommended by online ‘experts’. Furthermore, we underline the importance of thorough history taking.

Iliopsoas abscesses (IPA) are uncommon, with an associated mortality rate of up to 20%. We describe the case of a 55-year-old man war veteran who presented with an unusual cause of IPA secondary to retained foreign body (FB). His initial trauma 30 years before was a result of a blast injury with shrapnel penetration suffered after inadvertently driving over a landmine as an ambulance driver in a conflict region. A CT scan was performed, revealing a 13 mmx8 mm radio-opaque FB within the right psoas at the level of the fifth lumbar vertebra with a surrounding collection. Subsequent open surgical exploration removed two gravel fragments. Given the knowledge of a traumatic blast injury with retained FB and repeated episodes of sepsis, surgical exploration is warranted. To our knowledge, this is the first case of recurrent IPA secondary to a retained FB from a historical trauma.

We describe the case of a 32-year-old man from Cape Verde having headache and increasing visual loss. Clinical and radiological investigations disclosed a massive destructive lesion involving the anterior and central skull base, orbit and nasoethmoid region initially interpreted as a malignant small round cell tumour. Surgical biopsies were negative for neoplasm, showing an intense inflammatory infiltrate together with fungus, later characterised as Aspergillus flavus spp. The patient was immunocompetent with no evidence of congenital or acquired immunodeficiencies. Invasive fungal infections in immunocompetent patients are rare and can be a diagnostic challenge. The best diagnostic clues include the patient’s origin from tropical climates, imaging features and the identification of fungal hyphae on pathology specimens. Although a devastating disease in immunocompromised patients, craniocerebral aspergillosis in immunocompetent patients carries a better prognosis. Available literature supports the combined used of ‘conservative’ surgical resection and antifungal therapy as the best treatment option.

We present a case of a 51-year-old immunosuppressed man with underlying chronic lymphoproliferative leukaemia (CLL), who presented to us in emergency with breathlessness, hydrophobia, anxiety and restlessness. He had a history of category 3 dog bite 2 months ago and had received a full course of rabies immunoglobulin and antirabies vaccine (ARV) as per the national schedule. As there were frank clinical reports of rabies, the patient was managed according to Milwaukee regimen. The patients died within a week of the appearance of symptoms. The brain autopsy revealed Negri bodies conforming the mortality due to rabies.Immunosuppressed patients, like our patient who had CLL have low antibody formation after rabies prophylaxis. Antibody titres in immunosuppressed patients need to be measured after the 2–4 weeks of the last injection of ARV to decide whether a booster of ARV needs to be administered or not.

We describe a 9-day-old baby with coarctation of the aorta who required urgent resuscitation including intubation and cardiac compressions. Despite the commencement of prostaglandin E1 (PGE1) to reopen the ductus arteriosus via the intraosseous route, postductal saturations remained unrecordable for a further 45 min. Within 3 min of administration of PGE1 via an umbilical venous catheter (UVC), saturations were recordable at 92%. UVC access was the sentinel intervention that irrevocably altered the clinical prognosis. This baby boy has survived with excellent neurodevelopmental outcome. Clinicians are less familiar with UVCs outside of the newborn period. Our data demonstrate successful placement in neonates up to 28 days of age. We hope this case encourages clinicians to consider the UVC as first-line central venous access in collapsed neonates. In cases of suspected left heart obstruction, we argue that UVCs are the optimal route.

A 67-year-old man was referred to the renal team following an episode of acute kidney injury on a background of chronic kidney disease. He had a 9-year history of steroid-sensitive arthritis, epigastric pain and isolated submandibular gland enlargement. He was noted to have a raised eosinophil count, total serum protein and total immunoglobulin G4 (IgG4) level as well as a serum hypocomplementaemia. A renal biopsy showed a tubulointerstitial nephritis with lymphoplasmacytic infiltrates, fibrosis and IgG4-positive plasma cells on immunohistochemistry. A diagnosis of IgG4-related disease was made based on clinical presentation and pathology. Renal function improved with glucocorticoids and the patient was successfully transitioned to azathioprine as a steroid-sparing agent.

Primary neuroendocrine tumours of the kidney are rare, and their pathophysiology is uncertain; since their discovery in 1966, they have been described only a few times in the literature. We present a case of a well-differentiated neuroendocrine tumour of the kidney in an asymptomatic patient, which required a multidisciplinary approach by the hospital’s team, including precise surgical treatment and an effective radiopathological diagnosis. The patient underwent right radical nephrectomy. During follow-up, he remained asymptomatic, and no metastases or complications were identified.

Renal cell carcinoma (RCC) frequently presents with osseous metastasis, predominantly lytic and prone to pathological fracture. The metastatic lesion in the extremity presents with local swelling, pain and immobility due to pathological fracture. The solitary or oligometastatic lesions should be treated with curative intent, which can help the patient to lead a more prolonged and disability-free life. The RCCs and their metastases are hypervascular with an exuberant arterial supply. Surgery can lead to uncontrolled life-threatening haemorrhage. Preoperative transarterial embolisation reduces tumour vascularity significantly and reduces intraoperative blood loss. We present a 46-year-old male patient with solitary hypervascular metastatic recurrence of RCC with a pathological femoral fracture with an infeasible initial surgery due to profuse haemorrhage. He was successfully treated by preoperative transarterial embolisation, followed by surgical resection and implantation of a megaprosthesis. Multidisciplinary management reduces patient morbidity and mortality with successful treatment in solitary hypervascular metastasis from RCC.

Liver involvement in Graves’ disease can be seen as a part of autoimmune process or rarely, due to the direct effects of thyrotoxicosis on liver. Hyperthyroidism can also have gastrointestinal manifestations like frequent bowel movements, diarrhoea, even malabsorption with steatorrhoea. We report a 36-year-old man with hyperthyroidism, presenting with cholestatic jaundice and persistent small bowel diarrhoea. He was diagnosed to have Graves’ disease and after ruling out more common causes, the cause of cholestatic jaundice was supposed to be Graves’ disease. Considering this possibility, the patient was started on treatment with carbimazole. As patient’s thyroid function tests started improving, he showed significant clinical and biochemical improvement from liver point of view as well.

We report the case of a 10-year-old boy that presented with a palpable, painless, frontal lesion. Laboratory assessments were unremarkable and the patient was asymptomatic. Initial investigation, with a skull radiograph and unenhanced CT scan, showed a lytic midline frontal lesion involving the inner and outer tables of the skull and a large subgaleal hypodense component. MRI further depicted communication with the epidural space and contact with the superior sagittal sinus (SSS). Subsequent evaluation by Doppler ultrasound and MR angiography excluded a sinus pericranii and showed normal patency of the SSS. Surgical biopsy revealed chronic granulomatous inflammation; PCR was positive for Mycobacterium sp. One year after surgical resection and antitubercular therapy, there are no signs of recurrence. Primary calvarial involvement by tuberculosis is rare, even in developing countries. Familiarity with the expected clinical and imaging features is required to avoid diagnostic delay.

Objectives. To describe health-related behaviors or indicators associated with overall health and well-being using the Global Assessment Tool (GAT), a health behavior and psychosocial questionnaire completed annually by US Army personnel. Methods. We analyzed GAT responses from 2014 to 2018, consisting of 367 000 to 449 000 respondents per year. We used generalized estimating equations to predict the presence of each health behavior or indicator, aggregated by year and stratified on various demographics. Results. Key findings included decreases from 2014 to 2018 in risky health behaviors such as hazardous drinking (7.5% decrease) and tobacco use (7.9% decrease), dietary supplement use (5.0% to 10.6% decrease, depending on type), self-reported musculoskeletal injury (5.1% decrease), and pain interference (3.6% decrease). Physical activity, sleep, and nutritional habits largely remained consistent over time. Conclusions. In the Army, tobacco, alcohol, and risky dietary supplement usage appears to be declining, whereas lifestyle health behaviors have been stable. Whether these trends reflect responses to health education is unknown. The GAT provides useful insights into the health of the Army, which can be leveraged when developing health-related educational programs and policies. Public Health Implications. Health behaviors that have changed less over time (e.g., nutrition, sleep) may require novel approaches compared with those that changed more (e.g., dietary supplement use, drinking). (Am J Public Health. 2021;111(11):2064–2074. https://doi.org/10.2105/AJPH.2021.306456 )

Background: Social and demographic factors may influence patient treatment by physicians. This study analyzes the influence of patient sociodemographics on prescription practices among hand surgeons. Methods: We performed a retrospective analysis of all hand surgeries (N = 5278) at a single academic medical center from January 2016 to September 2018. The average morphine milligram equivalent (MME) prescribed following each surgery was calculated and then classified by age, race, sex, type of insurance, and history of substance use or chronic pain. Multivariate linear regression was used to compare MME among groups. Results: Overall, patients with a history of substance abuse were prescribed 31.2 MME more than those without ( P < .0001), and patients with a history of chronic pain were prescribed 36.7 MME more than those without ( P < .0001). After adjusting for these variables and the type of procedure performed, women were prescribed 11.2 MME less than men ( P = .0048), and Hispanics were prescribed 16.6 MME more than whites ( P = .0091) overall. Both Hispanic and black patients were also prescribed more than whites following carpal tunnel release (+19.0 and + 20.0 MME, respectively; P < .001). Patients with private insurance were prescribed 24.5 MME more than those with Medicare ( P < .0001), but 25.0 MME less than those with Medicaid ( P < .0001). There were no differences across age groups. Conclusions: Numerous sociodemographic factors influenced postoperative opioid prescription among hand surgeons at our institution. These findings highlight the importance of establishing more uniform, evidence-based guidelines for postoperative pain management, which may help minimize subjectivity and prevent the overtreatment or undertreatment of pain in certain patient populations.

Background: The Rhode Island State Legislature passed the Uniform Controlled Substances Act in 2016 to limit opioid prescriptions. We aimed to objectively evaluate its effect on opioid prescribing for hand surgery patients and also identify risk factors for prolonged opioid use. Methods: A 6-month period (January-June 2016) prior to passage of the law was compared with a period following its implementation (July-December 2017). Thumb carpometacarpal arthroplasty and distal radius fracture fixation were classified as “major surgery” and carpal tunnel and trigger finger release as “minor surgery.” Prescription Drug Monitoring Database was used to review controlled substances filled during the study periods. Results: A total of 1380 patients met our inclusion criteria, with 644 prelaw and 736 postlaw patients. Patients undergoing “major surgery” saw a significant decrease in the number of pills issued in the first postoperative prescription (41.1 vs 21.0) and a corresponding decrease in morphine milligram equivalents (MMEs) (318.6 vs 159.2 MMEs) after implementation. A 30% decrease in MMEs was also seen in those undergoing “major surgery” in the first 30 days postoperatively (544.7 vs 381.7 MMEs). Risk factors for prolonged opioid use included male sex and preoperative opioid use. Conclusions: In Rhode Island, opioid-limiting legislation resulted in a significant decrease in the number of pills and MMEs of the initial prescription and a 30% decrease in total MMEs in the 30-day postoperative period after “major hand surgery.” Additional research is needed to explore the association between legislation and clinical outcomes.

Objectives. To assess the impact of the COVID-19 pandemic on immunization services across the life course. Methods. In this retrospective study, we used Michigan immunization registry data from 2018 through September 2020 to assess the number of vaccine doses administered, number of sites providing immunization services to the Vaccines for Children population, provider location types that administer adult vaccines, and vaccination coverage for children. Results. Of 12 004 384 individual vaccine doses assessed, 48.6%, 15.6%, and 35.8% were administered to children (aged 0–8 years), adolescents (aged 9–18 years), and adults (aged 19‒105 years), respectively. Doses administered overall decreased beginning in February 2020, with peak declines observed in April 2020 (63.3%). Overall decreases in adult doses were observed in all settings except obstetrics and gynecology provider offices and pharmacies. Local health departments reported a 66.4% decrease in doses reported. For children, the total number of sites administering pediatric vaccines decreased while childhood vaccination coverage decreased 4.4% overall and 5.8% in Medicaid-enrolled children. Conclusions. The critical challenge is to return to prepandemic levels of vaccine doses administered as well as to catch up individuals for vaccinations missed. (Am J Public Health. 2021;111(11):2027–2035. https://doi.org/10.2105/AJPH.2021.306474 )

Background: Moebius syndrome is a disorder characterized by facial and abducens nerve paralysis. Patients can present a wide range of upper extremity malformations. Literature focused on orthopedic manifestations of Moebius syndrome shows variability in the prevalence and clinical presentation of upper extremity anomalies. The aim of this work is to evaluate the prevalence of upper extremity malformations in patients with Moebius syndrome, clarify its various clinical presentations, and present treatment strategies for their management. Methods: This is a retrospective, cross-sectional study including patients with Moebius syndrome and upper extremity malformations between 2012 and 2019. Data include demographic characteristics, Moebius syndrome subtype, type of malformation, affected extremity, and surgical procedures underwent. Quantitative data were recorded as mean (standard deviation [SD]), and qualitative data were expressed in terms of totals and percentages. Statistical association between Moebius syndrome subtype and development of upper extremity anomalies was evaluated using binary logistic regression. Results: Twenty-five out of 153 patients (16.3%) presented upper extremity malformations (48% male). Mean age of presentation was 9.08 ± 9.43 years. Sixty-eight percent of the malformations were unilateral. The most common presentations included Poland syndrome and simple syndactyly with 8 cases each (32%), followed by 5 cases of brachysyndactyly (20%), 3 cases of amniotic band syndrome (12%), and 1 case of cleft hand (4%). No statistical association was found between Moebius syndrome subtype and odds ratio for development of upper extremity anomalies. Thirteen patients (52%) underwent reconstructive procedures. Conclusion: Poland syndrome and syndactyly are the most common anomalies in patients with Moebius syndrome. Patients may present with a wide range of hand malformations, each patient should be carefully evaluated in order to determine whether surgical treatment is needed and to optimize rehabilitation protocols.

Background: The clinical outcomes of reoperations for recurrent neurogenic thoracic outlet syndrome (NTOS) remain undefined. Methods: From 2009 to 2019, 90 patients with recurrent NTOS underwent anatomically complete supraclavicular reoperation after previous operation(s) performed at other institutions using either supraclavicular (Prev-SC = 48), transaxillary (Prev-TA = 31), or multiple/combination (Prev-MC = 11) approaches. Prospectively maintained data were analyzed retrospectively. Results: The mean patient age was 39.9 ± 1.4 years, 72% were female, and the mean interval after previous operation was 4.1 ± 0.6 years. The mean Disabilities of the Arm, Shoulder, and Hand (QuickDASH) score was 62 ± 2, reflecting substantial preoperative disability. Residual scalene muscle was present in 100% Prev-TA, 79% Prev-SC, and 55% Prev-MC ( P < .05). Retained/residual first rib was present in 90% Prev-TA, 75% Prev-SC, and 55% Prev-MC ( P < .05). There were no differences in operative time (overall 210 ± 5 minutes), length of hospital stay (4.7 ± 0.2 days), or 30-day readmissions (7%). During follow-up of 5.6 ± 0.3 years, the improvement in QuickDASH scores was 21 ± 2 (36% ± 3%) ( P < .01) and patient-rated outcomes were excellent in 10%, good in 36%, fair in 43%, and poor in 11%. Conclusions: Anatomically complete decompression for recurrent NTOS can be safely and effectively accomplished by supraclavicular reoperation, regardless of the type of previous operation. Residual scalene muscle and retained/residual first rib are more frequently encountered after transaxillary operations than after supraclavicular or multiple/combined operations. Supraclavicular reoperation can achieve significant symptom reduction and functional improvement for approximately 90% of patients with recurrent NTOS.

To describe longitudinal, anatomical, and functional alterations caused by inflammatory and neovascular lesions of idiopathic multifocal choroiditis/punctate inner choroidopathy using adaptive optics imaging and microperimetry.Longitudinal case study using multiple imaging modalities, including spectral-domain optical coherence tomography, fluorescein angiography, indocyanine green angiography, optical coherence tomography angiography, flood illumination adaptive optics, and microperimetry.A 21-year-old myopic Asian man presented with blurred vision in the right eye. Clinical examination was notable for an isolated hypopigmented, perifoveal lesion in each eye. Multimodal imaging showed inflammatory lesions in the outer retina, retina pigment epithelium, and inner choroid lesions of both eyes. The right eye additionally exhibited active Type-2 macular neovascularization with loss of cone mosaic regularity that was associated with reduced sensitivity on microperimetry. The clinical picture was consistent with multifocal choroiditis/punctate inner choroidopathy. The patient was treated with oral steroids and three injections of intravitreal bevacizumab in the right eye. After therapy, imaging showed reestablishment of the cone mosaic on flood illumination adaptive optics and improvement in sensitivity on microperimetry.Adaptive optics imaging and microperimetry may detect biomarkers that help to characterize the nature and activity of multifocal choroiditis lesions and to help monitor response to therapy. With timely intervention, structural abnormalities in the outer retina and choroid can be treated, and anatomical improvements precede improvements in visual function.

Objective Overall trends in rates of fully alcohol-attributable mortality may mask disparities among demographic groups. We investigated overall, demographic, and geographic trends in fully alcohol-attributable mortality rates in Minnesota. Methods We obtained mortality data from Minnesota death certificates and defined fully alcohol-attributable deaths as deaths that would not occur in the absence of alcohol. We calculated age-adjusted death rates during 2000-2018 using 5-year moving averages stratified by decedents’ characteristics and geographic location. Results Chronic conditions accounted for most of the alcohol-attributable deaths in Minnesota (89% during 2014-2018). Alcohol-attributable mortality rates per 100 000 population increased from an average rate of 8.0 during 2000-2004 to 12.6 during 2014-2018. During 2000-2018, alcohol-attributable mortality rates were highest among males (vs females), adults aged 55-64 (vs other ages), and American Indian/Alaska Native people (vs other racial and ethnic groups) and lowest among people aged ≤24 years and Asian or Pacific Islander people. During 2014-2018, the alcohol-attributable mortality rate among American Indian/Alaska Native people was more than 5 times higher than the overall mortality rate in Minnesota. Conclusions Results from this study may increase awareness of racial and ethnic disparities and continuing health inequities and inform public health prevention efforts, such as those recommended by the Community Preventive Services Task Force, including regulating alcohol outlet density and increasing alcohol taxes.

Background: To determine if the “unaffected” hand in children with hemiplegic cerebral palsy (CP) is truly unaffected. Methods: We performed a retrospective review of manual dexterity as measured by the Functional Dexterity Test (FDT) in 66 children (39 boys, 27 girls, mean age: 11 years 4 months) with hemiplegic CP. Data were stratified by Manual Ability Classification System (MACS) level, birth weight, and gestational age at birth, and compared with previously published normative values. Results: The FDT speed of the less affected hand is significantly lower than typically developing (TD) children ( P < .001). The development of dexterity is significantly lower than TD children (0.009 vs. 0.036 pegs/s/year, P < .001), with a deficit that increases with age. MACS score, birth weight, and age at gestation are not predictors of dexterity. The dexterity of the less affected hand is poorly correlated with that of the more affected hand. Conclusions: Both dexterity and rate of fine motor skill acquisition in the less affected hand of children with hemiplegic CP is significantly less than that of TD children. The less affected hand should be evaluated and included in comprehensive treatment plans for these children.

Objectives. To assess cannabis and alcohol involvement among motor vehicle crash (MVC) fatalities in the United States. Methods. In this repeated cross-sectional analysis, we used data from the Fatality Analysis Reporting System from 2000 to 2018. Fatalities were cannabis-involved if an involved driver tested positive for a cannabinoid and alcohol-involved based on the highest blood alcohol concentration (BAC) of an involved driver. Multinomial mixed-effects logistic regression models assessed cannabis as a risk factor for alcohol by BAC level. Results. While trends in fatalities involving alcohol have remained stable, the percentage of fatalities involving cannabis and cannabis and alcohol increased from 9.0% in 2000 to 21.5% in 2018, and 4.8% in 2000 to 10.3% in 2018, respectively. In adjusted analyses, fatalities involving cannabis had 1.56 (95% confidence interval [CI] = 1.48, 1.65), 1.62 (95% CI = 1.52, 1.72), and 1.46 (95% CI = 1.42, 1.50) times the odds of involving BACs of 0.01% to 0.049%, 0.05% to 0.079%, and 0.08% or higher, respectively. Conclusions. The percentage of fatalities involving cannabis and coinvolving cannabis and alcohol doubled from 2000 to 2018, and cannabis was associated with alcohol coinvolvement. Further research is warranted to understand cannabis- and alcohol-involved MVC fatalities. (Am J Public Health. 2021;111(11):1976–1985. https://doi.org/10.2105/AJPH.2021.306466 )

OBJECTIVE Describe the incidence of new persistent opioid use after inguinal hernia repair as well as its associated risk factors. SUMMARY BACKGROUND DATA The development of new persistent opioid use after surgery is a common complication, however its incidence following inguinal hernia repair has not been described. Given that roughly 800,000 inguinal hernia repairs are performed annually in the United States, any incidence could have profound implications for patients. METHODS Retrospective cross-sectional study of the incidence of new persistent opioid use after inguinal hernia repair using a national database of de-identified administrative health claims of opioid-naive patients undergoing surgery from 2008-2016. RESULTS During the study period, 59,795 opioid-naive patients underwent inguinal hernia repair and met inclusion criteria. Mean (SD) age was 57.8 (16.1) years old and 55,014 (92%) patients were male. 922 (1.5%) patients continued filling opioids prescriptions for at least 3 months after surgery. The most significant risk factor for developing new persistent opioid use after surgery was filling an opioid prescription in the 30 days prior to surgery (OR 4.34, 95% CI 3.75-5.01). These prescriptions were provided by surgeons in 52% of cases and primary care physicians in 16% of cases. Other risk factors for new persistent opioid use included receiving a larger opioid prescription, having more comorbidities, having a major postoperative complication, and certain mental health disorders and pain disorders. CONCLUSION After undergoing inguinal hernia repair, 1.5% of patients developed new persistent opioid use. Filling an opioid prescription in the 30 days prior to surgery had the strongest association with this complication.

A 63-year-old man with hypertension was referred for catheter ablation of persistent atrial fibrillation. He was diagnosed with paroxysmal atrial fibrillation approximately 6 years prior. Over the previous 12 months, his atrial fibrillation had become persistent despite medication optimisation for rate control and elective cardioversion. Sinus rhythm was restored briefly. The decision was made to pursue catheter ablation and isolation of the pulmonary veins. On anaesthetic induction, the patient suffered from a hypertensive crisis. The procedure was aborted, and the patient was admitted to hospital for investigations of secondary hypertension. Ultimately, the patient was diagnosed with bilateral pheochromocytomas. This case outlines the diagnostic challenges and cardiac comorbidities associated with bilateral pheochromocytomas.

A 36-year-old man with schizophrenia, on two times per day clozapine, presented with a 2-year history of diffuse intermittent body pain.Per chart review—and on presentation—his physical examination had been consistently unremarkable, without point-tenderness elicited at any major muscle groups or focal neurological deficits. Workup for myopathy, neuropathy and supratherapeutic clozapine levels had similarly been unrevealing.Given that prior interventions had been unsuccessful in alleviating these symptoms, we queried whether clozapine might have been contributory. As a result, we adopted a previously described strategy of scheduling the bulk of patients’ medication during non-waking hours.At 1-month follow-up, the patient reported about a 50% improvement in his symptoms. At 6-month follow-up, this improvement in symptoms had been sustained.Our findings add to the limited anecdotal reports of this side effect whose true prevalence remains unknown. Timely recognition has the potential to promote adherence to therapy among patients in the maintenance phase.

Granulomatosis with polyangiitis (GPA) is a rare disorder characterised by inflammation of small-sized and medium-sized blood vessels that result in damage to various organ systems, but it most commonly affects the respiratory tract and kidneys. It is one of the few entities that can present with ocular inflammation as well as renal impairment at the same time. We describe a case of a 38-year-old man with conjunctivitis, episcleritis, anterior uveitis as a first manifestation of GPA. His presentation with red eye and anterior uveitis prompted further workup, which revealed acute renal failure (creatinine 442 mmol/L), elevated inflammatory markers (erythrocyte sedimentation rate of 85 mmol/hour and C reactive protein of 72 mg/L), and a c-antineutrophil cytoplasmic antibody titre >8. An urgent renal biopsy was performed demonstrating necrotising crescentic glomerulonephritis, which led to the final diagnosis of GPA. Treatment induction with intravenous methylprednisolone and plasmapheresis followed by an oral prednisone taper and intravenous rituximab infusions leading to resolution of all symptoms and normalisation of kidney function. This report highlights conditions that can present with both ocular inflammation and renal dysfunction with a focus on GPA and its ocular manifestations.

A 42-year-old man who had suffered from severe fatigue for 5 years was diagnosed as having chronic fatigue syndrome (CFS) and fibromyalgia. Endocrinological workup using combined anterior pituitary function tests showed that the patient had adrenocorticotropin hormone (ACTH) deficiency, with a normal pituitary MRI. Treatment with a physiologic dose of oral hydrocortisone replacement physically ameliorated his general fatigue. A secondary workup using a growth hormone-releasing peptide-2 test revealed that he also had growth hormone (GH) deficiency, and GH replacement therapy was started. His muscle pain and depression were improved by the therapy. Here, we present a rare case of combined deficiency of ACTH and GH in a middle-aged man with severe general fatigue. This case report aims to raise awareness of combined deficiency of ACTH and GH as a differential diagnosis of CFS and its mimics.

Lymphoepithelial carcinoma (LEC) of the larynx is an extremely rare tumour which, unlike its nasopharyngeal counterpart, has shown a propensity to affect elderly Caucasian men and is not commonly associated with Epstein-Barr virus. We present a 70-year-old Chinese man who complained of hoarseness and dysphagia. Nasoendoscopy revealed a left supraglottic tumour. Preoperative MRI (in particular Diffusion Weighted Imaging) showed the possibility of two distinct components within a tumour. The patient underwent total pharyngolaryngectomy and bilateral selective neck dissection. The final histology report confirmed the presence of a tumour with two distinct components: predominant LEC with a smaller conventional (keratinising) squamous cell carcinoma component. The patient recovered well after surgery and subsequently underwent adjuvant radiotherapy. Final staging was pT3 N2c M0 (AJCC stage IVA). Follow-up over 2 years revealed no tumour recurrence.

Porphyria cutanea tarda is the most common type of porphyria. It is associated with a deficiency of uroporphyrinogen decarboxylase enzyme responsible for heme synthesis. Clinical manifestations are predominantly dermatological and very rarely present with ocular involvement. Although scleral thinning in the interpalpebral area is a well-documented entity, sight-threatening corneal involvement is rarely described. We, herein report a case of a 58-year-old man who presented with ocular surface dryness, photophobia and mild redness. Slit-lamp biomicroscopy revealed corneo-scleral thinning in both eyes. The diagnosis was confirmed with a urine porphyrin test, serum iron and serum ferritin levels. We started him on conservative management after which he was lost to follow-up. He presented again after 6 years with total corneal opacification and progressive loss of vision in the right eye.