Giant abdominal wall hernias represent a major challenge to the hernia surgeon in practice today. Of the common abdominal wall hernias, those located in the subcostal region are among the most difficult to repair, and have historically been plagued by higher recurrence rates than other locations, such as the midline. No technique has been identified as the clearly superior choice for hernias of this type.
We report a successful repair of a giant, multiply recurrent subcostal hernia with loss of domain in a 45-year-old obese Caucasian man. This was accomplished in a novel fashion, using a porcine acellular dermal matrix (Strattice™) as the floor of the repair, which was fixed to the costal margin using orthopedic bone anchors (Mitek™), then covered with a pedicled omental flap to eliminate dead space and facilitate a more rapid revascularization of the porcine acellular dermal matrix implant.
This case emphasizes the need for a thorough understanding of the challenges of the specific type of hernia defect encountered, as well as knowledge of any available techniques that may be adjunctively employed to enhance the chances of achieving a successful result.
Subcostal; Hernia; Omental flap; Porcine acellular dermal matrix; Strattice
Partial complex epilepsy with psychosensorial and psychotic symptoms remains a relatively rare condition that can sometimes be mistaken for an axis I psychiatric disorder. There is no specific treatment for this particular type of epilepsy, anti-epileptic medication being the cornerstone of therapeutic intervention with the occasional addition of neuroleptics. Lack of response to anti-epileptic agents is often a sign of poor prognosis and requires risky and sometimes invasive interventions with high morbidity for patients.
We report the case of a 21-year-old right-handed Caucasian man of French-Canadian descent who was living with his mother immediately before being hospitalized in a psychiatric setting for the first time. He seemed obsessed with developing new concepts to reach a more ‘perfect’ existence. He also claimed feeling odd sensations in his mind and in his body that could be linked to some sort of ‘evolutionary’ process resulting from spiritual uplift. He reported non-specific visual hallucinations and what sounded like auditory hallucinations and telepathic powers. The first diagnosis was a possible schizophreniform disorder and our patient was hospitalized. Shortly afterwards, an electroencephalogram showed an important subcortical epileptic activity, compatible with partial complex epilepsy with psychosensorial and psychotic symptoms. Despite a negative response to medication, symptoms proper to this type of epilepsy were substantially alleviated using a psychotherapeutical treatment intended for patients with psychotic disorders, namely integrated psychological therapy (IPT). Significant functional improvement in our patient has been achieved since then.
This case report illustrates that despite a negative response to medication, symptoms proper to this type of epilepsy could be substantially alleviated using psychotherapeutical treatment modalities. To the best of our knowledge, this is the first time such a finding has been reported in the scientific literature. This could open the way for new research themes and therapeutic interventions for such patients.
Partial complex epilepsy with psychosensorial symptoms; Integrated psychological therapy (IPT); Psychosis; Medication; Rehabilitation
Peptic ulcer disease is still the major cause of gastrointestinal perforation despite major improvements in both diagnostic and therapeutic strategies. While the diagnosis of a perforated ulcer is straightforward in typical cases, its clinical onset may be subtle because of comorbidities and/or concurrent therapies.
We report the case of a 53-year-old Caucasian man with a history of chronic myeloid leukemia on maintenance therapy (100mg/day) with imatinib who was found to have a subphrenic abscess resulting from a perforated duodenal ulcer that had been clinically overlooked. Our patient was febrile (38.5°C) with abdominal tenderness and hypoactive bowel sounds. On the abdominal plain X-ray films, a right subphrenic abscess could be seen. On contrast-enhanced multi-detector computed tomography, a huge air-fluid collection extending from the subphrenic to the subhepatic anterior space was observed. After oral administration of 500cm3 of 3 percent diluted diatrizoate meglumine, an extraluminal leakage of the water-soluble iodinated contrast media could then be appreciated as a result of a perforated duodenal ulcer. During surgery, the abscess was drained and extensive adhesiolysis had to be performed to expose the duodenal bulb where the ulcer was first identified by methylene blue administration and then sutured.
While subphrenic abscesses are well known complications of perforated gastric or duodenal ulcers, they have nowadays become rare thanks to advances in both diagnostic and therapeutic strategies for peptic ulcer disease. However, when peptic ulcer disease is not clinically suspected, the contribution of imaging may be substantial.
Peptic ulcer disease; Subphrenic abscess; Abdominal plain film; Multi-detector computed tomography
We report an interesting clinical case which could represent a new syndrome never described previously in the literature.
A 15-year-old Caucasian boy presented to our institution with recurrent respiratory infections, severe atopic dermatitis, short stature and skeletal malformations. Laboratory tests showed a high level of immunoglobulin E, hypereosinophilia with a normal white blood cell count and a low level of somatomedin C. The patient had had atopic dermatitis resistant to treatment since the age of 6 months. His height did not increase despite receiving cyclic therapy with recombinant growth hormone.
We hypothesized the presence of several diseases not confirmed by any genetic tests. Our patient could have an unknown disease. Further research is needed to identify this possible new syndrome.
Atopic dermatitis; Hypereosinophilia; Hyperimmunoglobulin E; Short stature; Skeletal malformations
Pulmonary amyloidosis is rare and is often misdiagnosed due a lack of general awareness.
In this case report we describe a 69-year-old Chinese woman who presented with a right lower lobe pulmonary nodule. After video-assisted thoracoscopic lobectomy, a histopathologic diagnosis of pulmonary nodular amyloidosis was rendered. She has done well postoperatively, showing no local recurrence or distal disease in an 8-month follow-up period.
Distinguishing parenchymal nodular amyloidosis from a malignant lung tumor is often quite difficult. In the differential diagnosis of pulmonary nodules, nodular amyloidosis should be considered to avoid unnecessary lobectomy.
Amyloidosis; Differential diagnosis; Pulmonary nodules
MRI is becoming increasingly important in the evaluation of shoulder pain, especially in the diagnosis of rotator cuff injuries and conditions that mimic them. Parsonage-Turner syndrome is a well-defined clinical entity that presents with acute-onset shoulder pain and weakness, often first recognized on magnetic resonance imaging scans.
We studied magnetic resonance imaging features of two Asian men (ages 24 and 31 years) who presented with variable-onset shoulder pain and weakness. Magnetic resonance imaging revealed increased T2-weighted signal intensity of supraspinatus and infraspinatus muscles in both patients.
Magnetic resonance imaging findings are distinctive, although nonspecific, in cases of Parsonage-Turner syndrome, and knowledge of the imaging and clinical features of this disease enable clinicians to arrive at the correct diagnosis and guide appropriate management.
Fallopian tube carcinoma is a rare gynecological malignancy with low accuracy detection preoperatively. The symptoms are unspecific and imaging can be misleading. Since it was first described in 1847, there have been only a little over 2000 case reports.
This case report describes a 66-year-old Caucasian woman who presented with progressive diffuse abdominal pain, without other symptoms. After abdominopelvic magnetic resonance imaging, she was sent to the Portuguese Oncology Institute of Oporto with the suspicion of peritoneal carcinomatosis of unknown primary tumor. Due to a pelvic palpable mass (calcified giant uterine fibroid) she was directed to the Gynecology team. Surgery was performed and a large mass in her upper abdomen was identified. The extemporary examination revealed a high-grade adenocarcinoma. During surgery a small change of color and consistency of her left fallopian tube was noted and unilateral adnexectomy was performed. After pathologic and immunohistochemistry tests, the diagnosis of fallopian tube carcinoma with peritoneal dissemination was made.
This case is very unique in the way that a small primary fallopian tube carcinoma was able to disseminate to the upper abdominal quadrant with little pelvic dissemination. The symptoms and imaging were unspecific. Although a rare occurrence, we should not forget fallopian tube carcinoma in the differential diagnosis of peritoneal carcinomatosis, even in the absence of Latzke’s triad.
Fallopian tube carcinoma; Histology; Prognostic factors; Review; Symptoms
Branches from the cervical portion of the internal carotid artery are rare. In most cases, atherosclerotic stenosis is found at the bifurcation of the internal and external carotid arteries. However, when associated with atherosclerotic carotid artery disease, the origin of the rare branches arising from the internal carotid artery can be another site of stenosis. This report describes a rare case of such tandem carotid stenosis treated by carotid endarterectomy and the importance of the possibility of stenosis at the origin of the anomalous branch from the internal carotid artery.
A 73-year-old Japanese woman presented with transient left hemiparesis and vertigo. Magnetic resonance angiography seemed to indicate two stenotic lesions distal to the right internal carotid artery in addition to the origin of the right internal carotid artery, and angiography indicated tandem stenotic lesions of the internal carotid artery. The patient was successfully treated with right carotid endarterectomy, including the distal stenotic lesion of internal carotid artery, and postoperative angiography indicated that the occipital artery arose from the internal carotid artery.
It is important to recognize rare cases of the anomalous origin of the occipital artery from the internal carotid artery and the possibility that the origin of such an anomalous occipital artery may be the cause of stenosis.
Anomalous branch; Carotid endarterectomy; Tandem stenosis
While paraneoplastic syndromes in patients with malignant and metastasizing tumors are common, they are rarely associated with skin tumors showing predominantly local growth patterns. This case report relates to a patient with giant condyloma acuminatum, also called Buschke-Löwenstein tumor, with paraneoplastic hypercalcemia, who was successfully treated with conservative treatment.
The patient in question is a 48-year-old German man with a giant periscrotal tumor. Before and during the therapy, two episodes of symptomatic hypercalcemia occurred, which were successfully treated by bisphosphonates, intravenous fluids and diuretics. No evidence of lytic bone affection was found.
Paraneoplastic hypercalcemia may occur in patients who have a Buschke-Löwenstein tumor. For patients, where surgery is not an option, established medical therapies like bisphosphonates may be useful in addition to diuretics and infusions.
Paraneoplastic hypercalcemia; Buschke-Löwenstein tumor; Giant condyloma acuminatum
Rheumatoid nodules occur in 30 percent of patients with active rheumatoid arthritis. Common sites include the buttocks or the extensor surface of the forearm, with one group documenting their presence in the thyrohyoid membrane. To the best of our knowledge, rheumatoid nodules have not been described in the thyroid bed.
We present the case of a 46-year-old Caucasian woman with active rheumatoid arthritis and Hashimoto's thyroiditis who presented with compressive neck symptoms. An ultrasound scan revealed that both lobes of her thyroid were enlarged. The right lobe measured 7.9×3.4×3.3cm and the left 8.3×3.3×3.1cm. A solitary 1.0×0.6×0.8cm nodule was seen in the right lower lobe. Her thyroid-stimulating hormone level was 4.22uU/mL (0.34 to 5.60). A total thyroidectomy was performed due to her symptoms and the possible growth of a nodule when on levothyroxine. A postoperative ultrasound scan showed no remaining thyroid tissue. The pathology revealed several small neoplasms ranging from a well-encapsulated adenoma to highly atypical follicular and papillary Hurthle cell lesions in the setting of Hashimoto’s thyroiditis. Low-dose radioactive iodine (33.4mCi) was given. Four months later, our patient complained of a feeling of fullness in her neck. A solid nodule of mixed echogenicity (5.6×3.3×2.3cm) was seen in the right level VI of the neck, and solid tissue of mixed echogenicity (2.9×2.3×1.7cm) on the left. Following repeat surgery, the pathology from the right specimen showed Hashimoto’s thyroiditis. The left specimen had areas of granuloma formation with fibrinoid necrosis and palisading histiocytes, consistent with the histology of rheumatoid nodules. No evidence of malignancy was seen. The patient continues to do well and remains disease-free.
Rheumatoid nodules have not been reported in the thyroid bed. Their pathogenesis is not clear. Postoperative release of tumor necrosis factor alpha and local vascular damage may have triggered the nodule formation in this case. Rheumatoid nodules must be kept in the differential diagnosis of an enlarging thyroid in the setting of active rheumatoid arthritis. A fine-needle aspiration biopsy may show granuloma formation and be the most cost-effective initial diagnostic step, especially if there is a concern for malignancy. Early identification of these nodules will help decrease morbidity from unnecessary interventions and result in treatment that is both timely and appropriate.
Rheumatoid nodule; Thyroid bed; Rheumatoid arthritis
Although several anatomical anomalies in the middle turbinate have been reported, they usually remain asymptomatic. However, complicated aberrant middle turbinate extending through the choana to the contralateral nasopharynx has not been reported.
A 23-year-old Korean woman presented with a 6-month history of nasal obstruction and postnasal discharge refractory to medical treatment. An endoscopic examination revealed an abnormal middle turbinate, and a pedunculated nasal mass covered with normal mucosa that originated from the right lateral nasal wall filled most of the right posterior choana and extended to the left nasopharynx across the posterior free edge of the nasal septum. Computed tomography of the paranasal sinus showed abnormal bony trabeculation from the posterior bony attachment of the right middle turbinate to the left posterior nasopharyngeal wall. In addition, right maxillary sinusitis was observed. From these findings, the lesion was suspected to be an abnormal configuration of the right middle turbinate with secondary maxillary sinusitis and was successfully treated by resection of the malformed middle turbinate and middle meatal antrostomy.
Although this case illustrates a very rare developmental anomaly of the middle turbinate, thorough knowledge of the development of and anatomical anomalies associated with the middle turbinate is of utmost importance to perform proper sinus surgery and avoid complications. Therefore, these findings should be considered by otolaryngologists, and careful examination of the nasal cavity is necessary to determine the existence of this rare anomaly.
Congenital abnormalities; Maxillary sinusitis; Nasal obstruction; Nasopharynx; Turbinates
The average time spent playing video games is increasing. Prolonged immobility associated with gaming may therefore be an important risk factor for venous thromboembolism. We report a case of deep vein thrombosis associated with prolonged playing of PlayStation® games.
A 31-year-old Caucasian man, an exterior painter, presented with a three-day history of left leg pain and swelling after playing PlayStation® games for almost eight hours a day for four consecutive days. Doppler ultrasound of the left leg confirmed extensive left leg deep venous thrombosis requiring thrombolysis and anticoagulation.
Video gaming should be considered a risk factor for venous thromboembolism. Further studies are needed to estimate the degree of risk associated with prolonged periods of playing video games, and education for preventing venous thrombosis should be provided to gamers.
Video games; Venous thromboembolism; DVT
Although dabigatran, a novel oral anti-coagulant, has been approved for the prevention of thromboembolism in patients with non-valvular atrial fibrillation, the efficacy of dabigatran for the resolution of established intra-cardiac thrombi has not been validated. Herein is describe a case in which dabigatran was effective for thrombus resolution in a patient with a left ventricular aneurysm.
A 59-year-old Japanese man with a mid-ventricular obstructive hypertrophic cardiomyopathy-associated apical aneurysm presented with a left ventricular apical thrombus (15.0mm×17.0mm). Anti-coagulation therapy with dabigatran (150mg b.i.d. with meals) was initiated. Following dabigatran administration, weekly echocardiographic examinations demonstrated gradual decreases in thrombus size. After three weeks, no thrombus was detected and no systemic thromboembolic events had occurred.
The left ventricular apical thrombus resolved after dabigatran administration. Hence, dabigatran may represent an alternative to warfarin as a therapeutic option in patients with previously detected intra-cardiac thrombus.
Dabigatran; Intra-cardiac thrombus; Mid-ventricular obstructive hypertrophic cardiomyopathy
Hyponatremia associated with rhabdomyolysis is a rare event and a correct diagnostic approach is required to rule out this or other diseases as a primary cause and to avoid other complications resulting from a lack of appropriate treatment.
A 64-year-old Caucasian woman presented to our facility with worsening fatigue, slurred speech, nausea and vomiting, and high serum levels of creatine kinase and myoglobin together with hyponatremia. Normal arterial blood gas analysis results, normal serum potassium levels, increased urine sodium levels, urine specific gravity of >1003N/m3 and low urine volume suggested an endocrine etiology. Her low cortisol and thyroid hormone serum levels suggested a pituitary disorder. A magnetic resonance imaging study showed atrophy of her pituitary gland. A more detailed study of our patient’s obstetric history revealed a post-partum hemorrhage 30 years earlier. She was diagnosed as having late-onset Sheehan’s syndrome and treated with hormone replacement therapy, which normalized her clinical picture.
This case report shows that, in hyponatremia-associated rhabdomyolysis, an endocrinological origin should always be considered. This should include Sheehan’s syndrome as it can occur with late onset.
The emergence of multiple myeloma as a second malignancy in patients with essential thrombocythemia is extremely rare. Several cases have been published so far, pointing out the impact of a cytotoxic effect during treatment of essential thrombocythemia on the development of multiple myeloma.
We report the case of a 52-year-old Caucasian man who presented to our hospital because of leukocytosis, a slightly decreased hemoglobin level and thrombocytosis. After a complete hematological work-up, essential thrombocythemia was diagnosed. The patient was included in a multicenter clinical study, treated with anagrelide and his platelet counts were maintained in the normal range for more than 3 years. A sudden drop in his hemoglobin level with normal leukocyte and platelet count occurred at the same time as a back pain. Magnetic resonance imaging of his spine revealed the existence of a pathological fracture of Th4, the collapse of the upper edge of Th7 and osteolytic lesions of multiple thoracic vertebrae. Repeated hematological examinations, including bone biopsy with immunohistochemistry, disclosed diagnosis of multiple myeloma of the non-secretory type.
To the best of our knowledge this is the first published case in which multiple myeloma developed during the treatment of essential thrombocythemia with the non-cytotoxic drug anagrelide. Our attempts to find a common origin for the coexistence of multiple myeloma and essential thrombocythemia have not confirmed the genetic basis of their appearance. Further studies are needed to determine the biological impact of this coexistence.
Angiogenesis; Essential thrombocythemia; Multiple myeloma
Paratesticular fibrous pseudotumors represent benign new growths confined to intrascrotal structures. Both pathogenesis and clinical management are little understood due to the rarity of the lesion, with less than 200 cases reported to date. Recently, paratesticular fibrous pseudotumors have been postulated to be immunoglobulin G4-related, pathogenetically. Here we report two cases of patients with paratesticular fibrous pseudotumor to highlight the clinical features of this rare disease and we report the immunohistochemical examinations to support the theory of paratesticular fibrous pseudotumor being an immunoglobulin G4-related disease.
A 28-year-old white man presented with a painless intrascrotal mass. After a clinical examination, a malignant growth was suspected. His ultrasound results revealed a well-demarcated hypoechoic lesion of 1.5cm in diameter at the spermatic cord. Our patient underwent local excision. His follow-up has been uneventful for 12 years. The second case was an 18-year-old white man who presented with a painless scrotal mass suspicious of testicular tumor. A magnetic resonance imaging scan revealed a 3cm mass at the spermatic cord with very low signal density on T2-weighted imaging and a low and inhomogeneous uptake of gadolinium contrast agent on T1-weighted, fat-suppressed imaging. Following local excision, our patient has been well for 18 months.
On histological examination, both of the lesions consisted of collagen-rich hyalinized fibrotic tissue with storiform features. There were lymphofollicular infiltrates and, sporadically, also venulitis. The immunoglobulin G4 staining (in case 2) showed an infiltrate of 10 to 15 positive cells per high-power field on average, corresponding to a proportion of 40% in evaluable hot spots. The two patients with paratesticular fibrous pseudotumor presented within a time span of 15 years. During that time, 400 patients with testicular germ cell tumors had been treated in our institution.
The specific histological features documented in our case lend support to the theory of paratesticular fibrous pseudotumor being an immunoglobulin G4-related sclerosing disorder. Paratesticular fibrous pseudotumors usually occur in young adulthood. Clinically, paratesticular fibrous pseudotumor can mimic testicular malignancy. Ultrasonographic findings are largely unspecific, however, scrotal magnetic resonance imaging may aid in discriminating the lesion from malignant tumors. Local excision, whenever technically feasible, is the preferred treatment of paratesticular fibrous pseudotumor.
Paratesticular pseudotumor; IgG4-related disease; Scrotal magnetic resonance imaging; Scrotal ultrasound; Testis-sparing surgery
A case report is a narrative that describes, for medical, scientific, or educational purposes, a medical problem experienced by one or more patients. Case reports written without guidance from reporting standards are insufficiently rigorous to guide clinical practice or to inform clinical study design.
Primary Objective. Develop, disseminate, and implement systematic reporting guidelines for case reports.
We used a three-phase consensus process consisting of (1) pre-meeting literature review and interviews to generate items for the reporting guidelines, (2) a face-to-face consensus meeting to draft the reporting guidelines, and (3) post-meeting feedback, review, and pilot testing, followed by finalization of the case report guidelines.
This consensus process involved 27 participants and resulted in a 13-item checklist—a reporting guideline for case reports. The primary items of the checklist are title, key words, abstract, introduction, patient information, clinical findings, timeline, diagnostic assessment, therapeutic interventions, follow-up and outcomes, discussion, patient perspective, and informed consent.
We believe the implementation of the CARE (CAse REport) guidelines by medical journals will improve the completeness and transparency of published case reports and that the systematic aggregation of information from case reports will inform clinical study design, provide early signals of effectiveness and harms, and improve healthcare delivery.
Case report; Case study; EQUATOR Network; Patient reports; Meaningful use; Health research reporting guidelines
Antiphospholipid syndrome is a multisystem autoimmune disease characterized by arterial and/or venous thrombosis and persistent presence of antiphospholipid antibodies. It can be a primary disease or secondary when associated with other autoimmune diseases.
We present a case of a 16-year-old Caucasian boy with a massive arterial and venous thrombosis in his lower limbs as well as in his central nervous system with clinical symptoms such as headaches and chorea. He did not present any clinical or laboratory signs of a systemic inflammatory connective tissue disease, including systemic lupus erythematosus. Based on the clinical picture and results of the diagnostic tests (positive antibodies against β2-glycoprotein and a high titre of anticardiolipin antibodies) we finally diagnosed primary antiphospholipid syndrome. During a 9-month follow up after the acute phase of the disease, he was treated with low-molecular-weight heparin. Neurological symptoms were relieved. Features of recanalization in the vessels of his lower limbs were observed. After a subsequent 6 months, because of the failure of preventive treatment – an incident of thrombosis of the vessels of his testis – treatment was modified and heparin was replaced with warfarin.
Although the preventive treatment with warfarin in our patient has continued for 1 year of follow up without new symptoms, further observation is needed.
Antiphospholipid syndrome; Arterial thrombosis; Systemic lupus erythematosus; Venous thrombosis
In the International Statistical Classification of Diseases and Related Health Problems 10 and Diagnostic and Statistical Manual of Mental Disorders IV classification systems, catatonia is classified as a subtype of schizophrenia. However, catatonia is more frequently associated with mood disorders than schizophrenia. It is also associated with organic conditions. Catatonia responds to treatment with benzodiazepines and electroconvulsive therapy rather than antipsychotics. These features support the categorization of catatonia as an independent syndrome. There is a lack of consensus regarding the definition of chronic catatonia. There are two previous case reports of effective treatment of chronic catatonia with electroconvulsive therapy.
A 29-year-old South Asian woman was admitted to hospital because of poor food intake. Her condition had progressively worsened over the past seven months. She had features of catatonia. On admission, her Bush-Francis Catatonia Rating Scale score was 24. Her symptoms resolved after the administration of 17 electroconvulsive therapies but recurred later. She was given a further four electroconvulsive therapies. She remains well on aripiprazole at a dose of 60mg a day.
Bilateral electroconvulsive therapy is effective in the treatment of chronic catatonia and should be considered as a treatment option. A relapse of symptoms can occur after the discontinuation of treatment.
Chronic catatonia; Electroconvulsive therapy; Schizophrenia
The simultaneous rupture of both distal biceps tendons is a rare clinical entity that is difficult to treat and can have poor outcomes. A variety of treatment and rehabilitation options exist and have been reported for single sided and staged bilateral repairs, but none have described an approach for acute bilateral ruptures. Repairing distal biceps tendon ruptures using a single anterior incision and a cortical suspensory button technique has become increasingly popular in recent years. We present a report of our surgical approach using an endobutton technique and rehabilitation algorithm for this unusual injury pattern.
A 43-year-old Caucasian man presented with acute onset bilateral elbow pain while lifting a large sheet of drywall off the ground. He initially felt a ‘pop’ on the right and almost immediately felt another on the left after having to quickly shift the weight. He was unable to continue working and sought medical attention. His pain was predominantly in his bilateral antecubital fossae and he had significant swelling and ecchymoses. His clinical examination demonstrated no palpable tendon, a retracted biceps muscle belly, and clear supination weakness. Magnetic resonance imaging was performed and showed bilateral distal biceps tendon ruptures with retraction on both sides. After discussion with our patient, we decided that both sides would be repaired using a single anterior incision with endobutton fixation, first his right followed by his left six weeks later.
Overall, our patient did very well and had returned to full manual work by our last follow-up at 30 months. Although he was never able to return to competitive recreational hockey and was left with mild lateral antebrachial cutaneous nerve dysesthesias on his right, he felt he was at 85% of his premorbid level of function. We describe what we believe to be, to the best of our knowledge, the first case of simultaneous bilateral distal biceps tendon ruptures successfully treated with a single-incision endobutton technique, which represents a valid option in managing this difficult problem.
Creutzfeldt-Jakob disease (CJD) is an ultimately fatal, neurodegenerative disease caused by misfolded prion protein aggregation and accumulation. The development of amyotrophic features has been described in CJD, though rarely as an early or prominent feature. Consequently, the significance of amyotrophy in prion disease etiology remains unclear.
Our patient, a healthy 70-year-old French/Algerian man, presented to our hospital following a work-related fall and was admitted with lower limb skeletal muscle atrophy and fasciculations; the fasciculations progressed to involve the trunk, upper limbs and face. Within days, he developed evidence of a progressive ascending neurological syndrome and subsequent brain involvement with supranuclear palsy of upgaze, catalepsy and death 36 days after symptom onset. Amyotrophy remained the principle feature of his disease. Dementia started to develop within 10 days of the onset of his amyotrophy. Prion disease was confirmed at postmortem.
Our observations suggest an unusual form of prion disease with prominent early involvement of anterior horn cells, ascending prion propagation in the central nervous system and a grave prognosis.
Creutzfeldt-Jakob disease; Amyotrophy; Anterior horn cells
The dorsal root ganglion is a rare manifestation of metastatic spread. We report what we believe to be the first case of metastasis of a pulmonary adenocarcinoma to the lumbar dorsal root ganglion. Only four descriptions for different primary tumors spreading to the dorsal root ganglion have been described in the literature so far.
A 70-year-old Caucasian woman with a four-month history of left-sided lumbar radiculopathy was admitted to our department under the assumption of a herniated lumbar disc. Her past medical history included a pulmonary adenocarcinoma and invasive ductal breast cancer.
Lumbar magnetic resonance imaging revealed a space-occupying mass in her left neuroforamen L3-L4 with compression of her L3 nerve root. Neurinoma was taken into account as a differential diagnosis, although not considered typical. Surgery revealed a metastasis of pulmonary adenocarcinoma to her dorsal root ganglion.
Dorsal root ganglion metastases seem to be extremely rare and can mimic primary local nerve sheath tumors. Therefore, they usually present as incidental findings. Resection should be performed strictly under intraoperative monitoring as tumor spread between the nerve fibers is commonly observed. Metastases should be taken into account in spinal nerve tumors involving the dorsal root ganglion, especially in patients harboring known malignant diseases. The low incidence means that no clear treatment advice can be given. Resection is possible under intraoperative monitoring and relieves neurological symptoms.
Malignant tumors of the small bowel are rare. Melanoma of the small intestine is in most cases metastatic from a primary skin lesion. Perforation of small bowel melanoma is an extremely rare entity. To the best of our knowledge this is the fifth case published to date.
We report a rare case of acute abdomen due to perforated metastatic small bowel melanoma in a 38-year-old Caucasian man.
In the majority of cases small bowel melanoma represents metastasis from cutaneous sites. Although rare, the possibility of abdominal metastatic melanoma presenting with the clinical picture of acute abdomen must be always considered by the operating surgeon in patients with a history of primary cutaneous malignant lesion.
Acute abdomen; Melanoma; Metastasis; Perforation
Winged scapula is defined as the prominence of the medial border of the scapula. The classic etiopathology of scapular winging are injuries to the spinal accessory or long thoracic nerves resulting respectively in trapezius and serratus anterior palsy. To the best of our knowledge, there are only few reports of scapular lesions being mistaken for winging of the scapula. We report a rare case of a large scapular osteochondroma arising from the medial border and causing a pseudowinging of the scapula.
A 17-year-old Caucasian boy came to us complaining about a winged left scapula. The patient had a complete painless range of motion, but a large hard bony swelling was palpable along the medial border of his left scapula. A grating sensation was felt when his arm was passively abducted and/or elevated causing discomfort. A lesion revealed on X-rays was diagnosed as an osteochondroma of the medial border of his scapula. After preoperative examinations, he underwent open surgery in order to remove the lesion. A histological examination confirmed the clinical diagnosis of osteochondroma. A clinical examination 3 months later showed a full and painless range of motion, the absence of the grating sensation during passive abduction and elevation and the complete disappearance of his left shoulder deformity. After 2 years of follow-up, there were no clinical or radiological signs of recurrence.
Despite its rarity osteochondroma should be considered in the differential diagnosis for any adolescent presenting with a winging of the scapula.
Adolescents; Benign tumors; Osteochondroma; Pseudowinging; Winged scapula
Vasculopathy of the large vessels commonly occurs in sickle cell disease, and as a result cerebral infarction is a well characterized complication of this condition. However, spinal infarction appears to be rare. Spinal infarct is infrequent in the non-sickle cell population as well, and accounts for only about 1 percent of all central nervous system infarcts.
In the present work, we report the case of a 19-year-old African-American man with sickle cell disease who experienced an anterior spinal infarct and subsequent quadriplegia. He was incidentally noted to be a heterozygote for factor V Leiden. We also reviewed the literature and found two previous cases of spinal cord infarction and sickle hemoglobin. Our literature search did not demonstrate that heterozygocity for factor V Leiden plays an important role in spinal cord infarction.
The paucity of cases associated with sickle hemoglobin does not allow us to postulate any particular risk factors with sickle cell disease that might predispose patients to spinal cord infarction. Our patient’s case raises the question as to whether spinal cord infarction is being missed in individuals with sickle cell disease and neurologic symptoms.