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1.  Semilobar holoprosencephaly in a 12-month-old baby boy born to a primigravida patient with type 1 diabetes mellitus: a case report 
Holoprosencephaly is a rare spectrum of cephalic disorders resulting from a failure or incomplete division of the embryonic forebrain into distinct cerebral hemispheres. It is the most common brain malformation with an incidence of 1:250 during embryogenesis; however, owing to the associated high rates of spontaneous abortion the incidence is 1:16,000 among live deliveries. Pathogenesis of holoprosencephaly is complex and heterogeneous involving genetic abnormalities, teratogenic exposures, and syndromic associations. Among the teratogenic exposures, maternal diabetes is a well-established risk factor associated with a 200-fold increased incidence of holoprosencephaly.
Case presentation
We report a case of a delayed diagnosis of semilobar holoprosencephaly in a 12-month-old baby boy of African descent who presented to us with a history of global developmental delay, erratic sleep patterns, and poor weight gain. He was born to a type 1 diabetes mellitus mother at 39+ weeks by emergency cesarean section due to fetal distress and breech presentation. The baby weighed 2315 g and had Apgar scores of 6/10 and 8/10 at 1 and 5 minutes respectively. A physical examination done at 12 months of age revealed a small-for-age child with a developmental age of 2 months. He had normal facies but a neurological examination revealed hypotonia in all four limbs. The rest of systemic examination was unremarkable. Hematological and biochemical investigations revealed normal findings except for iron deficiency anemia. The child also underwent magnetic resonance imaging of his brain which revealed distinctive features of semilobar holoprosencephaly. He was treated for iron deficiency anemia with Hemovit syrup (ferric ammonium citrate, folic acid, pyridoxine hydrochloride, cyanocobalamin, and zinc sulfate) 10 ml thrice daily, ferrous sulfate 10 mg once daily, folic acid 1 mg once daily, and multivitamin syrup 5 ml once daily. Furthermore, nutritional and genetic counseling was offered to his parents.
In conclusion, although rare, holoprosencephaly is the commonest structural anomaly of the brain with a complex and multifactorial etiopathogenesis. It is prudent to diagnose it prenatally, classify its severity, and forge its prognosis so that parents are counseled early enough to make informed decisions especially where termination of pregnancy may be implicated.
PMCID: PMC5175312  PMID: 27998308
Holoprosencephaly; Semilobar holoprosencephaly; Structural brain malformation; Developmental delay; Case report
2.  Acute polyarthritis in a young patient caused by meningococcal and parvovirus B19 infections: a case report and review of the literature 
Meningococcal infection is a multifaceted disease including acute polyarthritis. This presentation should be known by clinicians in order to prevent delay in treatment. We report what we believe to be the first case of an association of parvovirus B19 and meningococcal polyarthritis in a young adult.
Case presentation
A 19-year-old Caucasian woman presented to our hospital with fever, intense leg pain, and a transient rash. A physical examination showed asymmetric polyarthritis and no neurological abnormalities. A parvovirus B19 polymerase chain reaction performed using a blood sample and knee fluid aspirate came back positive, but serology was negative for immunoglobulin M and positive for immunoglobulin G. A blood culture was positive for serotype C meningococcus; a polymerase chain reaction performed for Neisseria meningitidis was positive in joint fluid but negative in blood samples (performed after antibiotic treatment had begun). Our patient was treated with ceftriaxone for 15 days, associated with analgesic therapy. Hydroxychloroquine treatment was introduced 5 months after the onset of polyarthritis because of persisting inflammatory arthralgia.
To the best of our knowledge, this is the first case report of polyarthritis caused by concomitant meningococcal and parvovirus B19 infections. This unusual presentation of meningococcal disease may have resulted from the persistent parvovirus B19 infection. Our experience with this case illustrates the need for a systematic approach to the diagnosis of febrile acute polyarthritis. Only long-term follow-up will reveal if this infectious polyarthritis will evolve towards an autoimmune rheumatism.
PMCID: PMC5175313  PMID: 27998301
Meningococcemia; Polyarthritis; Parvovirus B19; Case report
3.  Primary hyperparathyroidism in pregnancy treated with cinacalcet: a case report and review of the literature 
The efficacy and safety of various modes of medical treatment for primary hyperparathyroidism in pregnancy are largely unknown.
Case presentation
We report the case of a 34-year-old white woman with primary hyperparathyroidism symptomatic for nephrolithiasis. Her serum calcium was 3.15 mmol/l and parathyroid hormone was 109.0 ng/L. Neck imaging found no pathological parathyroid tissue. Cinacalcet and cholecalciferol were started. She became pregnant 17 months later. The calcimimetic was stopped. During pregnancy, she was admitted for hydration administered intravenously two to three times per week. In her 24th week of pregnancy, cinacalcet was restarted. In her 32nd week, a cesarean section was carried out as planned.
Only three cases of primary hyperparathyroidism in women on cinacalcet therapy in pregnancy have been published in the literature. In the present case, hydration was useful in controlling serum calcium. Cinacalcet therapy helped to control serum calcium.
PMCID: PMC5175373  PMID: 27998296
Cinacalcet; Calcimimetic; Pregnancy; Primary hyperparathyroidism; Hypercalcemia; Case report
4.  Decompressive craniectomy for malignant middle cerebral artery infarction in a 16-year old boy: a case report 
Cryptogenic stroke frequently occurs in younger patients and has a high risk of recurrence. Consequently, secondary prevention is often suboptimal as there is no known risk factor to target. This case demonstrates an unexpected finding of middle cerebral artery infarction and extensive malignant transformation in a 16-year-old boy more than a day post-admission. The lack of a proven culprit lesion makes this case even more intriguing and subsequently raises questions of cryptogenic mechanisms in the context of unrelated trauma.
Case presentation
A 16-year-old white boy had been stabbed in his chest but had a Glasgow Coma Scale score of 15. Over a day later he developed sudden signs and symptoms consistent with a neurological event of unknown etiology. Computed tomography demonstrated significant cerebral edema but was equivocal in its list of differentials. A computed tomography scan of his chest demonstrated no cardiac wall or vascular injury and he was transferred to our neurosurgical unit for intracranial pressure monitoring. A computed tomography angiogram revealed an unexpected finding of malignant middle cerebral artery infarction. Failure to medically manage his intracranial pressure resulted in a decompressive craniectomy less than 12-hours postictus. Despite extensive diagnostic investigations no culprit lesion was identified and no patent foramen ovale found. Since discharge he has returned to full functional status. He was the youngest patient (mean age of 43 years) out of a 10-year institutional retrospective on decompressive craniectomies for malignant middle cerebral artery infarction (n = 40) and had the singularly best Glasgow Outcome Scale score of 5.
This case highlights the preponderance of cryptogenic stroke in younger patients and its etiological elusiveness. It further demonstrates that age is predictive in terms of survival and functional outcome in the context of malignant middle cerebral artery infarction.
PMCID: PMC5175374  PMID: 27998316
Cryptogenic; Decompressive; Craniectomy; Vasospasm; Stroke
5.  Mycobacterium tuberculosis as a cause of mandibular osteomyelitis in a young woman: a case report 
Tuberculosis is considered an emerging disease worldwide; in the last 10 years, its incidence has increased to more than 9.6 million cases of active tuberculosis. In 2014, it resulted in 1.5 million patient deaths. However, oral presentation with bone involvement occurs in less than 3% of all reported cases and rarely arouses clinical suspicion on initial presentation.
Case presentation
A 15-year-old Mexican girl who had a previous diagnosis of neurofibromatosis presented to our hospital with pain and swelling in the region of the left mandibular body since November 2011. A clinical examination revealed pain in the mandibular region, a mass of soft consistency that seemed to involve bone, and a fistula with discharge of intraoral purulent material. Additionally, tachycardia and hyperthermia were observed. The left submental and submandibular regions had a 12-cm-diameter swelling, which was well-delineated and nonerythematous. The final diagnosis was established by real-time polymerase chain reaction.
The final diagnosis of rare cases of tuberculous osteomyelitis in the jaw can be established by deoxyribonucleic acid (DNA) identification of Mycobacterium tuberculosis in the lesion. Simple and fast complementary diagnosis by real-time polymerase chain reaction is a fundamental approach to establishing early and effective pharmacological and surgical treatment.
PMCID: PMC5175378  PMID: 27998317
Tuberculosis; Osteomyelitis; Mycobacterium tuberculosis; Jaw; Case report
6.  A rare case of a double phytobezoar causing gastric and jejunum obstruction in an adult man: a case report 
Bezoars are an uncommon cause of mechanical intestinal occlusion. There are four different kinds of bezoars: phytobezoars, made of vegetables and fibers; trichobezoars, resulting from the ingestion of hair and frequently an expression of psychiatric disorders; lactobezoars, which are formed of milk curd; and pharmacobezoars, caused by drugs and medications. Symptoms are classically indistinguishable from one another and from more common causes of intestinal occlusion, so it can be difficult to establish a correct diagnosis in order to apply the correct treatment. We present a rare case of two different phytobezoars causing intestinal occlusion (gastric and jejunal). We also describe the correct techniques for making a correct and fast diagnosis of occlusion caused by phytobezoars, and the possible conservative and operative treatments.
Case presentation
We present the case of a double phytobezoar that was surgically treated with a double enterotomy. Our patient was a 68-year-old Caucasian man with a medical history of hypertension, a previous open appendectomy, and open repair of a perforated gastric ulcer. He was admitted with a 5-day history of abdominal pain located in his upper quadrants along with vomiting. After a preoperative examination, he was taken to the operating room. He was discharged in a good clinical condition 11 days after surgical intervention. A physical examination at 6 months demonstrated our patient was in good health.
Diagnosing bezoars is difficult because of their rarity. However, they must be taken into consideration in a differential diagnosis because their treatment is not always surgical. In fact, it may be conservative in many cases and a correct diagnosis will guide towards the correct therapy.
PMCID: PMC5159969  PMID: 27978851
Bezoars; Phytobezoars; Small bowel obstruction; Gastrostomy; Enterotomy
7.  Khat – a new precipitating factor for reversible cerebral vasoconstriction syndrome: a case report 
Postpartum reversible cerebral vasoconstriction syndrome is one of the rare reversible cerebral vasoconstriction syndromes. The clinical presentation is usually characterized by recurrent headache, focal neurological deficit, and reversible cerebral vasoconstriction seen on cerebral angiography.
Case presentation
We report a case of a 35-year-old Yemeni woman who presented with headache and focal neurological deficits that occurred 10 days after delivery, with segmental narrowing of cerebral arteries on angiography. She had significant clinical and radiological improvement on follow-up.
The presentation of our patient’s reversible cerebral vasoconstriction syndrome is unusual as she has two possible precipitating factors. In addition to being in the postpartum state, she also has a long history of chewing khat, a vasoactive substance commonly used by immigrants from Yemen. We hope that this case report will increase awareness among physicians about the use of this plant by immigrants from the horn of Africa and Yemen.
PMCID: PMC5160011  PMID: 27978853
Reversible cerebral vasoconstriction syndrome; Khat; Postpartum cerebral angiopathy; Call–Fleming syndrome
8.  First report on treating spontaneous infectious spondylodiscitis of lumbar spine with posterior debridement, posterior instrumentation and an injectable calcium sulfate/hydroxyapatite composite eluting gentamicin: a case report 
Spontaneous infectious spondylodiscitis is a rare, but serious disease with the risk of progressive neurological impairment. The surgical approach to spontaneous infectious spondylodiscitis is in most cases an anterior debridement and fusion, often in staged surgeries. Here we report a case of single-stage posterior debridement and posterior instrumented fusion in combination with an injectable calcium sulfate/hydroxyapatite composite eluting gentamicin.
Case presentation
A 59-year-old Caucasian man presented with a 6-week history of lumbar pain without sensory or motor disorders of his lower extremities. A magnetic resonance imaging scan of his lumbar spine in T2-weighted sequences showed a high signal of the intervertebral disc L4/L5 and in T1-weighted sequences an epidural abscess at the posterior wall of L4. Additional computed tomography imaging revealed osteolytic destruction of the base plate of L4 and the upper plate of L5. Antibiotic therapy was started with intravenous ciprofloxacin and clindamycin. We performed a posterior debridement via a minimally invasive approach, a posterior percutaneous stabilization using transpedicular screw-rod instrumentation and filled the intervertebral space with an injectable calcium sulfate/hydroxyapatite composite which elutes a high concentration of gentamicin. The patient’s lower back pain improved quickly after surgery and no recurrence of infection has been noticed during the 1-year follow-up. Computed tomography at 11 months shows complete bony fusion of L4 and L5.
An injectable calcium sulfate/hydroxyapatite composite releasing a high level of gentamicin can support the surgical treatment of spondylodiscitis in combination with posterior debridement and transpedicular screw-rod instrumentation.
PMCID: PMC5153911  PMID: 27955704
Spondylodiscitis; Surgical treatment; Posterior instrumentation; Local antibiotic; Injectable calcium sulfate/hydroxyapatite; Gentamicin; Vertebral osteomyelitis; Case report
9.  Possible failure of novel direct-acting oral anticoagulants in management of pulmonary embolism: a case report 
The relative effectiveness of vitamin K antagonists compared with novel oral anticoagulants in treating pulmonary embolism remains unclear. Recent trials comparing the efficacy of vitamin K antagonists with factor Xa inhibitors for the treatment of pulmonary emboli have been non-inferiority studies based primarily on risk reduction (such as bleeding events), rather than resolution of specific diseases such as pulmonary embolism. Consequently, there is a lack of evidence indicating which of these agents are more effective. Here, we present a case where pulmonary emboli were treated with novel oral anticoagulants followed by warfarin to discuss the potential limitations in the use of novel oral anticoagulants as prevention or treatment of thromboembolism and the continued role for warfarin in this setting.
Case presentation
A 34-year-old African American woman presented to our clinic with shortness of breath and pleuritic chest pain several months post-surgery. She was identified as having multiple bilateral pulmonary embolisms and was treated with several novel oral anticoagulants, which failed to resolve the clots. Complete resolution was achieved upon switching to warfarin.
The patient described in this report failed to respond to novel oral anticoagulant therapy, but her emboli resolved when she was treated with warfarin. This study challenges the notion that factor Xa inhibitors are better alternatives to vitamin K anticoagulants in the treatment of pulmonary emboli based on their safety profile and ease of use alone. As a result, further post-marketing investigations into the efficacy of these agents in the management of pulmonary emboli may be warranted.
PMCID: PMC5135742  PMID: 27912791
Anticoagulant therapy; Warfarin; Factor Xa inhibitor; Thrombosis; Embolism; Case report
10.  Bilateral anomalous drainage of the posterior divisions of renal veins into the azygos venous system in a 20-year-old woman: a case report 
Renal vein anomalies are relatively infrequent and generally asymptomatic. Preoperative knowledge of such variants is, however, of paramount importance in several angiographic and surgical procedures including renal venography, renal vein sampling, spermatic embolization, and renal transplantation. Inadequate knowledge and failure to recognize such anatomic variations may lead to several operative hazards including hemorrhage, nephrectomy, and even death.
Case presentation
We report a case of bilateral anomalous drainage of the posterior divisions of renal veins into the azygos venous system in a 20-year-old woman of African descent from Tanzania who presented to us with a 12-year history of recurrent anemia. She had anemia, a positive sickling test, and hemoglobin electrophoresis revealed a sickle cell trait (AS). She underwent computed tomography angiography of her chest and abdomen to rule out the presence of arteriovenous malformations. Aortography findings were normal but venography results revealed features of tortuously dilated azygos and hemiazygos veins each receiving blood from its respective posterior division of renal vein.
Although venous anomalies are relatively infrequent and generally lack a clinical significance, a thorough understanding of embryologic development and its associated errors is of immense importance in equipping angiographers and surgeons to select appropriate interventional/operative techniques, anticipate risks, and prevent intervention-related complications.
PMCID: PMC5135777  PMID: 27912798
Renal vein anomaly; Renal vein variations; Azygos venous system; Azygos vein; Hemiazygos vein; Tanzania
11.  Follicular thyroid carcinoma presenting as solitary liver metastasis: a case report 
Distant metastasis from differentiated thyroid carcinoma at presentation is rare and isolated liver metastasis on presentation is almost unknown. We report a case of primary follicular carcinoma of the thyroid with isolated liver metastasis at presentation.
Case presentation
A 65-year-old man of Kashmiri origin presented to our tertiary referral center with obstructive jaundice; he was evaluated with magnetic resonance cholangiopancreatography and positron emission tomography-computed tomography. Positron emission tomography-computed tomography documented a lesion in his liver in addition to a metabolically active thyroid nodule. Fine needle aspiration cytology of the liver lesion supplemented with immunohistochemical analysis using thyroid transcription factor 1 confirmed the lesion as being an isolated metastasis from the primary thyroid lesion (which on fine needle aspiration cytology showed follicular architecture).
To best of our knowledge, this is first reported case of primary differentiated thyroid carcinoma presenting with isolated liver metastasis manifesting as obstructive jaundice.
PMCID: PMC5135783  PMID: 27912795
Thyroid follicular carcinoma; Liver metastasis; Positron emission tomography; TTF1; Case report
12.  Fetal malformation in maternal toxoplasma and rubella co-infection in Cameroon: a case report 
There has been a recent increase in the number of newborns with brain malformations due to congenital infections, but the impact of these diseases remains largely under ascertained in middle-income and low-income countries. This case report presents a fetal anencephaly following maternal toxoplasma and rubella co-infection in a resource-limited setting and the challenges faced by the patient and the health care provider in the management of the condition.
Case presentation
A 25-year-old black Cameroonian woman of Bakossi origin, gravida3 para1010, presented with a positive rubella and toxoplasma immunoglobulin G serologic test at 21 weeks of pregnancy; she could not benefit from a fetal morphologic ultrasound partly because there was none at the site of her antenatal clinic and because there were accessibility constraints getting to the nearest referral hospital approximately 100 km away. She returned to the hospital in labor pains 14 weeks later and, upon examination, she was observed to be at almost full cervical dilatation and had a stillbirth a few minutes later; a baby boy weighing 1600 g with anencephaly.
The devastated parents of the baby were counseled and given psychological support. She was discharged from hospital 3 days later and now benefits from continual follow up as out-patient. She was advised to consult a gynecologist-obstetrician before her next pregnancy.
Much attention still has to be paid to ameliorate the health care in resource-limited settings where pregnant women generally obtain less than adequate care.
Electronic supplementary material
The online version of this article (doi:10.1186/s13256-016-1133-y) contains supplementary material, which is available to authorized users.
PMCID: PMC5135823  PMID: 27912790
Toxoplasma and rubella co-infection; Anencephaly; Resource-limited settings; Case report
13.  Osteoid osteoma of the acetabulum successfully treated with computed tomography-guided resection and ablation using a standard electrosurgical generator: a case report 
Osteoid osteoma accounts for approximately 10% of all benign bone tumors. The most common sites of osteoid osteoma are the subcortical shaft and metaphyses of long bones, but any other skeletal bone site can be involved. The acetabulum is a rare site according to past reports. This site presents challenges to optimal management because it is anatomically difficult to approach, and because its rarity leads to limited experience with therapeutic procedures. Here, we report for the first time a rare case of osteoid osteoma in the acetabulum that was successfully treated via resection of the nidus and ablation using a standard electrosurgical generator under computed tomographic guidance.
Case presentation
A 9-year-old Japanese girl presented at a clinic with left hip pain without apparent cause for 1 month. She was diagnosed as having coxitis simplex. However, her pain did not change for 1 year and she was admitted to another hospital where osteoid osteoma in her left acetabulum was suspected. She was then referred to our hospital approximately 1 year after first symptom presentation, where she presented with severe left hip pain and was completely unable to walk. Computed tomography examinations revealed a well-demarcated 5 mm mass with bone sclerosis in her left acetabulum. The mass was characterized by low intensity on T1 and high intensity on T2 magnetic resonance images. These findings were consistent with osteoid osteoma of left acetabulum. She underwent computed tomography-guided resection of nidus and ablation using a standard electrosurgical generator. A histological examination confirmed acetabular osteoid osteoma. Complete pain relief was achieved after the procedure and she experienced no complications. She could walk without any pain at the final follow-up 1 year post-treatment and no local recurrence was observed.
We successfully treated acetabulum osteoid osteoma without any symptom recurrence by computed tomography-guided resection and ablation using a standard electrosurgical generator. In addition, we preserved our patient’s sciatic nerve and triradiate cartilage. Our report provides evidence that a computed tomography-guided procedure should be considered the treatment of choice for osteoid osteoma of the acetabulum because it is a less invasive alternative to en bloc resection.
PMCID: PMC5135830  PMID: 27912788
Osteoid osteoma; CT guidance; Heat ablation; Acetabulum; Case report
14.  Radiation-induced osteosarcoma of the skull base after radiation therapy in a patient with nasopharyngeal carcinoma: a case report and review of the literature 
Radiation-induced osteosarcomas are a recognized complication of radiation therapy. Owing to the fact that it is rare, publications on radiation-induced osteosarcoma of the skull base are limited to a small series and some case reports.
Case presentation
We describe a rare case of a patient with a skull base radiation-induced osteosarcoma treated 11 years before with ionizing radiation for an undifferentiated carcinoma of the nasopharynx. The patient was treated with chemotherapy alone, but he died after the third cycle.
Radiation-induced osteosarcoma of the skull base after treatment of nasopharyngeal carcinoma is a very rare but very aggressive complication with a poor prognosis. Chemotherapy gives bad results, and regular follow-up of treated patients should be considered.
PMCID: PMC5133737  PMID: 27906102
Osteosarcoma; Skull; Radiotherapy; Nasopharynx; Chemotherapy; Pathogenesis
15.  Legionella pneumonia in the Niagara Region, Ontario, Canada: a case series 
Legionella pneumophila, a major cause of Legionnaires’ disease, accounts for 2–15 % of all community-acquired pneumonia requiring hospitalization and up to 30 % of community-acquired pneumonia requiring intensive care unit admission. Early initiation of appropriate antimicrobial therapy is a crucial step in the prevention of morbidity and mortality. However, recognition of Legionnaires’ disease continues to be challenging because of its nonspecific clinical features. We sought to describe hospitalized community-acquired Legionnaires’ disease to increase awareness of this important and potentially lethal disease.
A retrospective multicenter observational study was conducted with all patients with confirmed Legionnaires’ disease in the Niagara Region of the Province of Ontario, Canada, from June to December 2013.
From June to December 2013, there were 14 hospitalized cases of Legionnaires’ disease in the Niagara Region. Of these, 86 % (12 patients) had at least one comorbidity and 71 % (10 patients) were cigarette smokers. In our cohort, Legionnaires’ disease was diagnosed with a combination of a urinary Legionella antigen test and a Legionella real-time polymerase chain reaction assay. Delay in effective antimicrobial therapy in the treatment of Legionella infection led to clinical deterioration. The majority of patients had met systemic inflammatory response syndrome criteria with fever >38 °C (71 %), heart rate >90 beats per minute (71 %), and respiratory rate >20 breaths per minute (86 %). Eleven patients (79 %) required admission to the intensive care unit or step-down unit, and nine patients (64 %) required intubation. Clinical improvement after initiation of antimicrobials was protracted.
Legionnaires’ disease should be considered during the late spring and summer months in patients with a history of tobacco use and various comorbidities. Clinically, patients presented with severe, nonspecific, multisystem disease characterized by shortness of breath, abnormal vital signs, and laboratory derangements including hyponatremia, elevated creatine kinase, and evidence of organ dysfunction. In addition, antimicrobial therapy with newer macrolides or respiratory fluoroquinolones should be initiated for severe community-acquired pneumonia requiring intensive care unit admission, prior to laboratory confirmation of diagnosis, especially when a clinical suspicion of Legionella infection exists.
PMCID: PMC5133757  PMID: 27906087
Legionella pneumophila; Legionnaires’ disease; Community-acquired pneumonia
16.  Craniopagus parasiticus – a parasitic head protruding from temporal area of cranium: a case report 
Craniopagus parasiticus is rare with an incidence of approximately four to six cases in 10,000,000 births. In our case, the head of the parasitic twin protruded from the temporal area of the normal twin’s cranium. The parasitic twin had two deformed lower limbs, of which one was rudimentary, and long bones of the bilateral lower limbs and some pelvic bone. Dissection of the mass of the parasitic twin’s body revealed the intestine but no chest organs or abdominal organs. There was a rudimentary labium but no vaginal opening. In resource-limited countries, maternal age or nutritional factors may play a role in craniopagus parasiticus.
Case presentation
A 38-year-old multigravida (gravida V para IV) woman of Amhara ethnicity was referred from a rural health center to our hospital due to prolonged second stage of labor at 42+1 weeks. On her arrival at our hospital, an obstetrician decided to do a caesarean section because she was unable to deliver vaginally. A live baby girl weighing 4200 g was delivered. The placenta was single and normal. Her Appearance, Pulse, Grimace, Activity, and Respiration scores were 7 and 9 at 1 and 5 minutes, respectively. She appeared to be grossly normal except for the parasitic co-twin attached to her cranium. After a week of extensive counselling and investigation, a successful separation operation was done. Postoperation, she comfortably suckled on the breast and had no neurological deficit. Two weeks after separation she was discharged in a good healthy condition with an arrangement for postnatal follow up.
The causes of craniopagus parasiticus are still unknown due to a rarity of cases and a limited number of studies on it. There have been only nine to ten cases of craniopagus parasiticus, of which only three survived past birth and were documented in the literature. Genetic scientists and researchers continue to investigate this case because they might find explanations for the birth defect, and provide answers to improve the prognosis and the life chances of twins with craniopagus parasiticus.
PMCID: PMC5134060  PMID: 27906038
Craniopagus parasiticus; Parasitic twin; Rare case
17.  Complicated acute appendicitis presenting as a rapidly progressive soft tissue infection of the abdominal wall: a case report 
We report a case of a rare complication of acute appendicitis with perforation through the abdominal wall. The case points out that an intraabdominal origin should be considered in patients presenting with rapidly spreading soft tissue infections of the trunk.
Case presentation
A 58-year-old European woman presented to our hospital with a 1-week history of severe abdominal pain accompanied by rapidly spreading erythema and emphysema of the lower abdomen. On admission, the patient was in septic shock with leukocytosis and elevation of C-reactive protein. Among other diagnoses, necrotizing fasciitis was suspected. Computed tomography showed a large soft tissue infection with air-fluid levels spreading through the lower abdominal wall. During the operation, we found a perforated appendicitis breaking through the fascia and causing a rapidly progressive soft tissue infection of the abdominal wall. Appendicitis was the origin of the soft tissue infection. The abdominal wall was only secondarily involved.
Even though perforated appendicitis as an etiology of a rapidly progressive soft tissue infection of the abdominal wall is very rare, it should be considered in the differential diagnosis of abdominal wall cellulitis. The distinction between rapidly spreading subcutaneous infection with abscess formation and early onset of necrotizing fasciitis is often difficult and can be confirmed only by surgical intervention.
PMCID: PMC5134067  PMID: 27906036
Perforated appendicitis; Abscess of the abdominal wall; Case report; Soft tissue infection
18.  Massive adrenal vein aneurysm mimicking an adrenal tumor in a patient with hemophilia A: a case report and review of the literature 
Visceral venous aneurysms are exceedingly rare, and until now, there have been no reports of this phenomenon in the adrenal vasculature. This report details the first adrenal venous aneurysm reported in the literature. The aneurysm presented as an 18-cm mass that was initially suspected to be a hematoma or tumor on the basis of the complex medical history of the patient, which included hemophilia A and testicular cancer. After surgical excision, pathologic examination confirmed this mass to be a 15.9-cm adrenal vein aneurysm, the largest aneurysm of any type or location recorded in the medical literature.
Case presentation
A 58-year-old caucasian male with hemophilia A presented to the emergency room of another institution with abdominal pain, blood in the stool, and a history of diverticulosis and symptomatic hemorrhoids. A large, left-sided adrenal mass was detected by computed tomography, and because of the patient’s hemophilia A and imaging consistent with a hemorrhagic mass, a hematoma was initially suspected. The patient was transferred to our institution, monitored for further bleeding with a stable hospital course, and discharged from the hospital under close monitoring. After 7–8 weeks with no change in the size of the mass, concerns grew regarding increasing symptoms of both satiety and mass effects from the large anomaly, as well as about the patient’s complicated medical history, which also included cancer. Surgical excision was recommended because of the concerns about increasing symptoms and the possibility of a malignancy. Correction and maintenance of factor VIII levels were incorporated pre-, intra-, and postoperatively, and en bloc surgical resection was performed to minimize bleeding and provide oncologic extirpation of the mass. A bowling ball-sized mass was removed, and careful pathologic examination revealed the mass to be a venous adrenal aneurysm. After a brief hospital stay, the patient made a full recovery. Extensive review of the literature revealed 11 reports of adrenal artery aneurysms but no reported case of an adrenal aneurysm arising from the venous system.
Several case reports suggest a correlation between hemophilia and aneurysms. In patients with inherited clotting disorders such as hemophilia A, aneurysms may present in atypical fashions and should be carefully ruled out.
PMCID: PMC5134102  PMID: 27906086
Adrenal artery aneurysm; Hemophilia; Case report
19.  Esophageal perforation after perioperative transesophageal echocardiography: a case report 
Transesophageal echocardiography is widely used in cardiac surgery. Transesophageal echocardiography probe insertion and manipulation can injure the esophagus.
Case presentation
A 76-year-old Asian man was admitted to our hospital for coronary artery bypass graft revascularization surgery. A right carotid endarterectomy was successfully performed 2 days before coronary artery bypass graft revascularization surgery. After the coronary artery bypass graft revascularization surgery was done successfully, postoperative computed tomography and esophagography revealed perforation of the middle esophagus. The patient was managed with fasting, parenteral nutrition, and intravenous antibiotics.
Esophageal perforation can occur with transesophageal echocardiography probe insertion, even without difficulty or resistance, in patients with atherosclerotic disease. Appropriate imaging and vigilance for esophageal injury after a transesophageal echocardiography examination in patients with cardiovascular disease are necessary for appropriate diagnosis and treatment.
PMCID: PMC5134105  PMID: 27906089
Transesophageal echocardiography; Complications; Esophageal perforation; Postoperative period; Case report
20.  Is radical surgery of an inverted papilloma of the maxillary sinus obsolete? a case report 
Sinonasal inverted papilloma is a locally aggressive tumor arising from the Schneiderian membrane which lines the nasal cavity and paranasal sinuses. Aggressive surgical approaches, such as lateral rhinotomy, were used until recently for complete removal of the inverted papilloma. Currently, endoscopic resection is the gold standard in the treatment of inverted papilloma. However, there are situations that justify an open approach. For example there are studies that report a higher postoperative recurrence rate after endonasal endoscopic resection, particularly in the treatment of recurrent diseases. While endoscopic resection performed by an experienced surgeon is definitely a minimally invasive therapy, an open approach is not necessarily associated with functional and aesthetic disadvantages.
This case report describes the treatment of inverted papilloma by an open approach. This has been described before but the new gold standard of endoscopic resection has to be taken into account before any treatment decision is made nowadays.
Case presentation
Contrast-enhanced magnetic resonance imaging of the head and neck area was indicated in a 72-year-old white German man who presented with suspected squamous cell carcinoma of his lower lip. Magnetic resonance imaging additionally revealed a 3×2 cm2 polycyclic arranged mucosal thickening with cystic and solid contrast affine shares at the antral laterocaudal area of his right maxillary sinus, extending from his right lateral nasal wall to his maxillary sinus floor.
He received antral polypectomy with medial maxillectomy via a unilateral LeFort I osteotomy approach. His pterygoid plate was preserved. A histological examination demonstrated a tumor composed of hyperplastic squamous epithelium protruding into the stroma (surface epithelial cells grew downward into the underlying supportive tissue), thus producing a grossly convoluted cerebriform appearance. Two weeks later, the patient regained a well-formed maxilla without any restrictions. He has remained disease-free for 25 months following the surgery and surveillance was continued in our tumor clinic.
Endoscopic resection of an inverted papilloma continues to be the gold standard. However, some cases require a radical approach. This does not necessarily increase patient morbidity.
PMCID: PMC5134116  PMID: 27906054
Inverted papilloma; Endoscopic resection; Radical approach; Caldwell–Luc; Case report
21.  Pancreatic metastases from ocular malignant melanoma: the use of endoscopic ultrasound-guided fine-needle aspiration to establish a definitive cytologic diagnosis: a case report 
When encountering solid pancreatic lesions, nonpancreatic primary metastases are rare and differentiating a metastasis from a primary neoplastic lesion is challenging. The clinical presentation and radiologic features can be similar and the possibility of a pancreatic metastasis should be considered when the patient refers to a history of a different primary cancer. Endoscopic ultrasound offers a key anatomical advantage in accessing the pancreas and endoscopic ultrasound-guided fine-needle aspiration has become the gold standard method for diagnosing pancreatic lesions.
Case presentation
A 58-year-old white Hispanic woman with a history of uveal malignant melanoma, presented with abdominal pain and jaundice. On admission, laboratory tests were performed (her total bilirubin was 6.37 mg/dL with a direct fraction of 5.30 mg/dL). Cross-sectional, abdominal computed tomography with contrast, showed a low-attenuating lesion localized in the pancreatic head (measuring 4 × 3 cm) and a thinner section of the distal bile duct suspicious for compression. Our patient was scheduled for an endoscopic ultrasound-guided fine-needle aspiration to establish a diagnosis. Endoscopic ultrasound showed a solid, hypoechoic, well-defined lesion with regular contours (measuring 3.17 × 2.61 cm), localized between the head and neck of the pancreas. Endoscopic ultrasound-guided fine-needle aspiration was performed with a 22G needle and cytology confirmed the diagnosis of metastatic melanoma. Our patient subsequently underwent right orbital exenteration, followed by duodenopancreatectomy without complications. At the moment our patient is receiving adjuvant chemotherapy at an outside oncology clinic.
To the best of our knowledge, this is a very rare presentation of an ocular malignant melanoma with an isolated pancreatic metastasis causing symptomatic biliary obstruction. Endoscopic ultrasound-guided fine-needle aspiration has proven to be the best method to diagnose solid pancreatic lesions. In this particular case, cytology was essential in confirming the diagnosis and guiding the most adequate therapy, which was a pancreatic resection, ocular exenteration of the melanoma, followed by adjuvant chemotherapy.
PMCID: PMC5134225  PMID: 27906105
Endoscopic ultrasound; Endoscopic ultrasound-guided fine-needle aspiration; Melanoma; Uveal melanoma; Metastatic melanoma; Pancreatic metastasis; Case report
22.  Folie à Deux and its interaction with early life stress: a case report 
Folie à deux is a very rare psychiatric syndrome in which a psychotic symptom is transmitted from one individual to another. We present a case of folie à deux occurring during childhood, which is not an usual presentation of this syndrome. In this case, the disorder is correlated with child abuse and neglect, which possibly had a role in the development of the symptoms in our case.
Case presentation
We present a case of folie à deux between an “induced” 9-year-old black Brazilian boy and the “inducer”, his grandmother. They were found to be sharing similar auditory and visual hallucinations and delusional beliefs. The boy was neglected by his parents and was being cared for by his grandmother, who had a history of mental disorder.
The close relationship between the boy and his grandmother, the family history of first-degree psychosis, and the child abuse and neglect suffered by the boy may have altered his vulnerability to early-onset psychosis and, in this case, folie à deux.
PMCID: PMC5134243  PMID: 27906088
Case report; Early psychosis; Child abuse; Child development; Induced delusional disorder; Shared paranoid disorder; Folie à deux
23.  Primary melanoma of the small bowel revealed by gastrointestinal bleeding: a case report 
Primary melanoma of the small bowel is extremely rare. Only a limited number of cases have been described in the literature. Mostly, the small intestine is affected by metastatic tumors of other primary lesions, especially cutaneous.
Case presentation
We report the case of a 75-year-old North African woman with a small bowel melanoma. The diagnosis was made by histological examination and immunohistochemical profile matching after a segmental small bowel resection. Postoperative investigations looking for cutaneous, gastrointestinal or ocular primary lesions found no abnormalities.
The diagnosis of primary small bowel melanoma can be retained although it remains difficult to exclude the possibility of metastatic melanoma.
PMCID: PMC5134275  PMID: 27906109
Intestinal melanoma; Primary melanoma of the small bowel; Gastrointestinal bleeding
24.  Previously undiagnosed hereditary spherocytosis in a patient with jaundice and pyelonephritis: a case report 
Hereditary spherocytosis is autosomal dominant inherited extravascular hemolytic disorder and is the commonest cause of inherited hemolysis in northern Europe and the United States. The classical clinical features of hereditary spherocytosis are anemia, jaundice, and splenomegaly. However, all of these classical features are not always revealed in the case of mild hemolysis or when hemolysis is well compensated. Patients with hereditary spherocytosis may remain undiagnosed for years if their hemolysis is mild.
Case presentation
A 42-year-old Asian woman presented to our clinic with a sudden onset of high fever with shaking chills and jaundice, suggesting septicemia; however, following detailed investigation, the patient was diagnosed with pyelonephritis and accelerated hemolysis of hereditary spherocytosis due to infection.
It is important to note that transient anemia or jaundice can sometimes be the only initial presenting symptoms in cases of undiagnosed latent hereditary spherocytosis. This case also highlights the fact that physicians should consider concomitant hemolytic disease in patients in whom jaundice and infections that rarely cause jaundice coexist.
PMCID: PMC5134285  PMID: 27906107
Hereditary spherocytosis; Jaundice; Anemia; Hemolytic disease
25.  Severe thrombocytopenia in a child with typhoid fever: a case report 
Although thrombocytopenia is common in typhoid fever, its course, response to treatment, and need for specific therapies such as platelet transfusion are not well characterized.
Case presentation
We report a case of typhoid fever in a 4-year-old Asian male returned traveler, admitted with prolonged fever and found to have severe thrombocytopenia (platelets 16 × 109/L). Despite appropriate antibiotic therapy, his platelet recovery was slow, but did not lead to complications and he did not require platelet transfusion.
There is no consensus in the medical literature guiding the optimal management of severe thrombocytopenia in typhoid fever, but it may improve with conservative management, as in our case. The epidemiology and management of this condition merits further research to guide clinical practice.
PMCID: PMC5134245  PMID: 27906062
Typhoid fever; Thrombocytopenia; Prolonged fever; Case report

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