scholarly journals Extensive Bone Lengthening for a Patient with Linear Morphea

2018 ◽  
Vol 2018 ◽  
pp. 1-5
Author(s):  
Kenichi Mishima ◽  
Hiroshi Kitoh ◽  
Masaki Matsushita ◽  
Tadashi Nagata ◽  
Yasunari Kamiya ◽  
...  
Keyword(s):  
Rare Condition ◽  
Localized Scleroderma ◽  
Bone Lengthening ◽  
Leg Length ◽  
Linear Type ◽  
Reconstruction Surgery ◽  
Frequent Form ◽  
Regenerative Ability ◽  
Clinically Significant

Localized scleroderma, also known as morphea, is a rare condition characterized by progressive sclerosis of the skin and associated atrophy of the underlying tissues. The linear type of localized scleroderma is the most frequent form in childhood, usually affecting unilateral extremities. Fibrosclerosis of the fasciae and muscles can spread across joints and impair the range of motion of the joint. Dysplastic and/or atrophic bones of the affected lower extremity can lead to clinically significant leg length discrepancy (LLD). Limb reconstruction surgery has rarely been indicated for LLD in patients with linear morphea. We report on a case of extensive bone lengthening for appreciable LLD in a pediatric patient with linear morphea. A Japanese girl with linear morphea underwent staged simultaneous lengthening of the femur and tibia twice at seven and eleven years of age using a unilateral external fixator. A healing index exceeded 100 days/cm except for the first femoral lengthening that was complicated by regenerate fracture. At the final follow-up, LLD of 38 mm remained, but she could walk independently without a brace or a crutch. Due to soft tissue tightness and poor regenerative ability in the affected limb, cautions should be taken to prevent regenerate fracture and/or malalignment of the limb.

Fetal chylous ascites may redevelop only after birth

2014 ◽  
Vol 3 (2) ◽  
Author(s):  
Ivan Peychl ◽  
Karel Harvanek ◽  
Petra Krasnicanova
Keyword(s):  
Abdominal Cavity ◽  
Chylous Ascites ◽  
Rare Condition ◽  
Good Health ◽  
Minimal Amount ◽  
Medium Chain Triglycerides ◽  
Hernia Sac ◽  
Clinically Significant ◽  
Long Chain

AbstractCongenital chylous ascites is a rare condition. We describe a case in which fetal ascites was found on a routine antenatal ultrasound, with all abnormalities resolving by 36 weeks’ gestation. No investigations or treatment for the ascites was undertaken after the baby´s birth. At the age of 3 months, when the baby underwent surgery for bilateral inguinal hernias and hydrocele, milky fluid was drained from the hernia sac. Laboratory and ultrasound investigations confirmed the presence of a significant amount of chyle in the peritoneal cavity. Congenital chylous ascites was treated by means of a diet based on medium chain triglycerides. By the age of 6 months, the fluid in the abdomen had resolved. However, it partly reappeared once a normal mixed diet was allowed. A minimal amount of fluid in the lower abdominal cavity was consistently found up to the age of 22 months. The fluid had finally disappeared by the age of 30 months when the child was generally in good health and developing normally. We conclude that congenital chylous ascites may be diagnosed prior to birth but may become more clinically significant only after birth, when its severity is enhanced by feedings of milk containing long chain fats. We recommend a thorough postnatal evaluation and follow-up in all infants diagnosed with fetal ascites, so that appropriate and timely management decisions can be made, if the chylous origin of the ascites is confirmed.

Autoimmune Pancreatitis – Diagnosis, Management and Longterm Follow-up

2014 ◽  
Vol 23 (2) ◽  
pp. 179-185 ◽  
Author(s):  
Suvadip Chatterjee ◽  
Kofi W. Oppong ◽  
John S. Scott ◽  
Dave E. Jones ◽  
Richard M. Charnley ◽  
...  
Keyword(s):  
Autoimmune Pancreatitis ◽  
Rare Condition ◽  
Team Approach ◽  
Diagnostic Challenge ◽  
Multisystem Disorder ◽  
North East ◽  
The North ◽  
Work Up ◽  
Igg4 Level

Background & Aims: Autoimmune pancreatitis (AIP) is a fibroinflammatory condition affecting the pancreas and could present as a multisystem disorder. Diagnosis and management can pose a diagnostic challenge in certain groups of patients. We report our experience of managing this condition in a tertiary pancreaticobiliary centre in the North East of England.Methods: Patients were identified from a prospectively maintained database of patients diagnosed with AIP between 2005 and 2013. Diagnosis of definite/probable AIP was based on the revised HISORt criteria. When indicated, patients were treated with steroids and relapses were treated with azathioprine. All patients have been followed up to date.Results: Twenty-two patients were diagnosed with AIP during this period. All patients had pancreatic protocol CT performed while some patients had either MR or EUS as part of the work up. Fourteen out of 22 (64%) had an elevated IgG4 level (mean: 10.9 g/L; range 3.4 - 31 g/L). Four (18%) patients underwent surgery. Extrapancreatic involvement was seen in 15 (68%) patients, with biliary involvement being the commonest. Nineteen (86%) were treated with steroids and five (23%) required further immunosuppression for treatment of relapses. The mean follow up period was 36.94 months (range 7 - 94).Conclusion: Autoimmune pancreatitis is being increasingly recognized in the British population. Extrapancreatic involvement, particularly extrahepatic biliary involvement seems to be a frequent feature.Diagnosis should be based on accepted criteria as this significantly reduces the chances of overlooking malignancy. Awareness of this relatively rare condition and a multi-disciplinary team approach will help us to diagnose and treat this condition more efiectively thereby reducing unnecessary interventions.

scholarly journals Surgical Treatment of Diffuse Idiopathic Skeletal Hyperostosis (DISH) Involving the Cervical Spine: Technical Nuances and Outcome of a Multicenter Experience

2021 ◽  
pp. 219256822098827
Author(s):  
Giorgio Lofrese ◽  
Alba Scerrati ◽  
Massimo Balsano ◽  
Roberto Bassani ◽  
Michele Cappuccio ◽  
...  
Keyword(s):  
Cervical Spine ◽  
Clinical Outcome ◽  
Waiting Times ◽  
Diffuse Idiopathic Skeletal Hyperostosis ◽  
Surgical Techniques ◽  
Rare Condition ◽  
Surgical Strategies ◽  
Bone Removal ◽  
Mild Dysphagia

Study Design: Retrospective multicenter. Objectives: diffuse idiopathic skeletal hyperostosis (DISH) involving the cervical spine is a rare condition determining disabling aero-digestive symptoms. We analyzed impact of preoperative settings and intraoperative techniques on outcome of patients undergoing surgery for DISH. Methods: Patients with DISH needing for anterior cervical osteophytectomy were collected. Swallow studies and endoscopy supported imaging in targeting bone decompression. Patients characteristics, clinico-radiological presentation, outcome and surgical strategies were recorded. Impact on clinical outcome of duration and time to surgery and different surgical techniques was evaluated through ANOVA. Results: 24 patients underwent surgery. No correlation was noted between specific spinal levels affected by DISH and severity of pre-operative dysphagia. A trend toward a full clinical improvement was noted preferring the chisel ( P = 0.12) to the burr ( P = 0.65), and whenever C2-C3 was decompressed, whether hyperostosis included that level ( P = 0.15). Use of curved chisel reduced the surgical times ( P = 0.02) and, together with the nasogastric tube, the risk of complications, while bone removal involving 3 levels or more ( P = 0.04) and shorter waiting times for surgery ( P < 0.001) positively influenced a complete swallowing recovery. Early decompressions were preferred, resulting in 66.6% of patients reporting disappearance of symptoms within 7 days. One and two recurrences respectively at clinical and radiological follow-up were registered 18-30 months after surgery. Conclusion: The “age of DISH” counts more than patients’ age with timeliness of decompression being crucial in determining clinical outcome even with a preoperative mild dysphagia. Targeted bone resections could be reasonable in elderly patients, while in younger ones more extended decompressions should be preferred.

scholarly journals Rare Etiology of Odynophagia in a Female Adolescent

2021 ◽  
pp. 352-358
Author(s):  
Anastasios Koutsoumourakis ◽  
Asterios Gagalis ◽  
Maria Fotoulaki ◽  
Maria Stafylidou
Keyword(s):  
Antiviral Treatment ◽  
Healing Process ◽  
Rare Condition ◽  
Female Adolescent ◽  
Mucosal Barrier ◽  
Esophageal Mucosa ◽  
Intact Immune System ◽  
History Of ◽  
Orolabial Herpes

Herpes esophagitis (HE) is a rare condition in immunocompetent adolescents. However, it commonly occurs as a primary infection in younger individuals. Herein, we report a 16-year-old female patient who had a history of fever for 5 days, odynophagia, and orolabial herpes infection for 7 days. Clusters of painful vesicles on an erythematous base on the lips, gingiva, and palate were observed on physical examination. Further, esophagogastroduodenoscopy revealed diffuse linear ulcerations in the distal esophagus. The patient then received the following treatment: intravenous (I.V.) acyclovir 5 mg/kg three times a day, I.V. omeprazole 40 mg two times a day, and acyclovir 5% cream four times a day. After 8 days of admission, the patient was discharged. A follow-up esophagogastroduodenoscopy was performed 7 weeks after discharge, and the results revealed that the esophageal mucosa had a normal appearance. The effect of antiviral treatment against HE remains unknown in these patients. Nevertheless, it is believed to accelerate the healing process in individuals with esophageal mucosal barrier damage. To the best of our knowledge, this case of a female adolescent with an intact immune system is the sixth case of herpes simplex esophagitis to be reported in the literature.

scholarly journals Acute otitis media in an infant complicated with osteoarthritis of temporomandibular joint and sub-periosteal abscess of the clavicle

2021 ◽  
Vol 37 (1) ◽  
Author(s):  
Kaoutar Cherrabi ◽  
Hind Cherrabi
Keyword(s):  
Septic Arthritis ◽  
Temporomandibular Joint ◽  
Nutritional Assessment ◽  
Acute Otitis Media ◽  
Clinical Signs ◽  
Rare Condition ◽  
Pediatric Emergency ◽  
Ear Infections ◽  
The Right

Abstract Background Otomastoiditis is a very frequent affection and a current complication of mal-treated benign ear infections in children. However, this a very rare case of the association of two rare complications of otomastoiditis in a newborn. On the one hand, septic arthritis of the temporomandibular joint which is a very rare condition that is difficult to diagnose, and when unrecognized or not treated accordingly, it can resolve in serious infectious complication and or definitive injury to the temporomandibular joint. On the other hand, osteomyelitis of the clavicle is also very rare, and only a few cases have been cited in the literature concerning infants. Case presentation This 46-day-old infant was brought to pediatric emergency consultation for 2 swelling inflammatory bulges, one in the right mastoid and pre-auricular regions, and another in the right basi-cervical area. The infant was hypertrophic febrile, hypotonic, and pale. He had preserved archaic reflexes. Besides, blood test showed an inflammatory syndrome, inflammatory anemia, and no other abnormalities. Upon supplementary computed tomodensitometry exam, the diagnosis of a combination of septic arthritis of the right temporomandibular joint and sub-periosteal abscess of the ipsilateral clavicle in a context of hypotrophy and malnutrition was suspected. A pus sample was obtained for bacteriological evaluation, after which the infant had a course of intravenous associated antibiotics, along with nutritional assessment and management. Surgical drainage of both collections was performed. The 6-month follow-up was satisfactory, without clinical signs of functional impact on temporomandibular joint, or acromioclavicular joint. Conclusion This work stresses the necessity of thorough clinical examination of infants even in cases of benign ear infections, as well as the importance of adapted treatment and follow-up, which could allow early diagnosis, appropriate treatment, or even prevention of severe complications that can be associated with such benign conditions.

Persistent Multispecies Actinomyces Mastitis Treated With Repeated Aspiration and Long-Term Oral Antibiotics

2021 ◽  
pp. 000313482110257
Author(s):  
Colin Jenkins ◽  
Anand Ganapathy ◽  
Crystal Fancher ◽  
Kazuhide Matsushima
Keyword(s):  
Antibiotic Therapy ◽  
Rare Condition ◽  
Breast Abscess ◽  
Amoxicillin Clavulanic Acid ◽  
Recurrent Breast ◽  
Causative Bacterium ◽  
Repeated Aspiration ◽  
Prolonged Antibiotic Therapy

Actinomycosis is an infection characterized by abscess formation, draining sinuses, and tissue fibrosis. The causative bacterium is a Gram-positive facultative anaerobe from the genus Actinomyces. Infections classically affect the cervicofacial, thoracic, or pelvic region and often require prolonged antibiotic therapy. Actinomycosis of the breast is a rare condition that may present as a recurrent breast abscess. We present a 33-year-old female with a recurrent breast abscess which grew A. radingae and A. israeli on aspirated fluid cultures. Treatment with surgical aspiration and a 6-week course of oral amoxicillin/clavulanic acid 875 mg twice daily resulted in clinical improvement. Our case demonstrates how recurrent breast abscesses caused by Actinomyces can be difficult to manage. Long-term antibiotic therapy with surgical aspiration and regular follow-up offer the best chance of clinical resolution.

Ossicular chain reconstruction: endoscopic or microscopic?

2021 ◽  
pp. 1-7
Author(s):  
E A Guneri ◽  
A Cakir Cetin
Keyword(s):  
Word Recognition ◽  
Middle Ear ◽  
Risk Index ◽  
Ossicular Chain ◽  
Recognition Score ◽  
Reconstruction Surgery ◽  
Ossicular Chain Reconstruction ◽  
Operation Duration ◽  
Air Conduction

Abstract Objective To compare the results of endoscopic and microscopic ossicular chain reconstruction surgery. Methods Patients undergoing ossicular chain reconstruction surgery via an endoscopic (n = 31) or microscopic (n = 34) technique were analysed for age, gender, Middle Ear Risk Index, ossicular chain defect, incision type, ossicular chain reconstruction surgery material, mean air conduction threshold, air–bone gap, air–bone gap gain, word recognition score, mean operation duration and mean post-operative follow up. Results Post-operative air conduction, air–bone gap and word recognition score improved significantly in both groups (within-subject p < 0.001 for air conduction and air–bone gap, and 0.026 for word recognition score); differences between groups were not significant (between-subject p = 0.192 for air conduction, 0.102 for air–bone gap, and 0.709 for word recognition score). Other parameters were similar between groups, except for incision type. However, endoscopic ossicular chain reconstruction surgery was associated with a significantly shorter operation duration (p < 0.001). Conclusion Endoscopic ossicular chain reconstruction surgery can achieve comparable surgical and audiological outcomes to those of microscopic ossicular chain reconstruction surgery in a shorter time.

scholarly journals Evaluation of a multidisciplinary lipid clinic to improve the care of individuals with severe lipid conditions: a RE-AIM framework analysis

2021 ◽  
Vol 2 (1) ◽  
Author(s):  
Laney K. Jones ◽  
Megan McMinn ◽  
David Kann ◽  
Michael Lesko ◽  
Amy C. Sturm ◽  
...  
Keyword(s):  
Primary Care ◽  
Program Effectiveness ◽  
Primary Care Providers ◽  
Care Providers ◽  
Lipid Levels ◽  
Framework Analysis ◽  
Lipid Clinic ◽  
Adherence Counseling ◽  
Clinically Significant

Abstract Background Individuals with complex dyslipidemia, or those with medication intolerance, are often difficult to manage in primary care. They require the additional attention, expertise, and adherence counseling that occurs in multidisciplinary lipid clinics (MDLCs). We conducted a program evaluation of the first year of a newly implemented MDLC utilizing the RE-AIM (reach, effectiveness, adoption, implementation, and maintenance) framework to provide empirical data not only on program effectiveness, but also on components important to local sustainability and future generalizability. Methods The purpose of the MDLC is to increase the uptake of guideline-based care for lipid conditions. Established in 2019, the MDLC provides care via a centralized clinic location within the healthcare system. Primary care providers and cardiologists were invited to refer individuals with lipid conditions. Using a pre/post-study design, we evaluated the implementation outcomes from the MDLC using the RE-AIM framework. Results In 2019, 420 referrals were made to the MDLC (reach). Referrals were made by 19% (148) of the 796 active cardiology and primary care providers, with an average of 35 patient referrals per month in 2019 (SD 12) (adoption). The MDLC saw 83 patients in 2019 (reach). Additionally, 50% (41/82) had at least one follow-up MDLC visit, and 12% (10/82) had two or more follow-up visits in 2019 (implementation). In patients seen by the MDLC, we found an improved diagnosis of specific lipid conditions (FH (familial hypercholesterolemia), hypertriglyceridemia, and dyslipidemia), increased prescribing of evidence-based therapies, high rates of medication prior authorization approvals, and significant reductions in lipid levels by lipid condition subgroup (effectiveness). Over time, the operations team decided to transition from in-person follow-up to telehealth appointments to increase capacity and sustain the clinic (maintenance). Conclusions Despite limited reach and adoption of the MDLC, we found a large intervention effect that included improved diagnosis, increased prescribing of guideline-recommended treatments, and clinically significant reduction of lipid levels. Attention to factors including solutions to decrease the large burden of unseen referrals, discussion of the appropriate number and duration of visits, and sustainability of the clinic model could aid in enhancing the success of the MDLC and improving outcomes for more patients throughout the system.

scholarly journals Prenatal findings and postnatal follow-up of a midline dural sinus malformation

2021 ◽  
Vol 10 (4) ◽  
pp. 205846012110063
Author(s):  
Hana Shabana ◽  
Johannes Leidinger ◽  
Johan Wikström ◽  
Ove Axelsson
Keyword(s):  
Magnetic Resonance Imaging ◽  
Spontaneous Regression ◽  
Rare Condition ◽  
Present Knowledge ◽  
Magnetic Resonance Imaging Examination ◽  
Dural Sinus ◽  
Resonance Imaging ◽  
Prenatal Magnetic Resonance Imaging ◽  
Dural Sinus Malformation

Dural sinus malformation is a rare condition. We describe a prenatally detected case followed by repeated ultrasound scans and a prenatal magnetic resonance imaging examination. A substantial spontaneous regression was observed, which is associated with a favorable outcome. We believe that our observations, including a long postnatal follow-up, will add to the present knowledge of prenatally detected cases, and thus improve management of the pregnancies as well as our possibilities to counsel the parents-to-be.

Lactotransferrin-Related Breast Amyloidosis: Report of a First Case

2021 ◽  
pp. 106689692110160
Author(s):  
Mingfei Yan ◽  
Mark Rodgers ◽  
Aparna Harbhajanka ◽  
Hannah Gilmore
Keyword(s):  
Breast Lesion ◽  
Hematologic Malignancy ◽  
Genetic Disorders ◽  
Rare Condition ◽  
Myoepithelial Cells ◽  
Hematological Disorders ◽  
First Case ◽  
Benign Nature ◽  
Extensive Involvement

Breast amyloidosis is a rare condition which is mostly associated with hematological disorders or hereditary genetic disorders. Imaging findings of breast amyloidosis can mimic malignancy, which often leads to biopsy or excision of the lesion. Here, we presented a case of localized lactotransferrin-related breast amyloidosis in an elderly female patient. Histologic examination revealed extensive involvement of breast lobules by amorphous amyloid materials, with attenuation of lobular structures and prominent calcifications. Positive immunostains for myoepithelial cells helped to exclude the possibility of invasive carcinoma. The patient had no hematologic malignancy besides immunoglobulin G lambda monoclonal gammopathy of undetermined significance. Mass spectrometry of the breast amyloid identified lactotransferrin and no immunoglobulin or its light chain. On follow-up, the patient showed no recurrence of the breast lesion after local excision nor showed other systematic comorbidities, indicating the benign nature of the lesion. This first report of lactotransferrin-related amyloidosis may represent a special type of localized breast amyloidosis that has no correlation with systematic disorders.

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