Caudal Duplication Syndrome Systematic Review—A Need for Better Multidisciplinary Surgical Approach and Follow-Up

Background and Objectives: Caudal duplication syndrome is a rare association of anatomical anomalies describing duplication of the hindgut, spine, and uro-genital structures, leading to varied clinical presentations. The current literature focuses on case reports which describe the embryological etiology and anatomical spectrum of the condition giving little attention to the surgical preparation, the need for a well-structured follow-up program, or the transition into adult healthcare of these complex patients. No reviews have been published regarding this complex pathology. Materials and Methods: A review of caudal duplication syndrome cases was done to assess the range of the clinical malformations, timing, and types of surgical interventions. Inconsistencies in multidisciplinary care, follow-up, and risk events were described. Results: Hindgut duplication always involved the anorectal region. Anorectal malformations were evenly distributed as unilateral and bilateral. Colon duplication extended from the anal region to the transverse colon or ascending colon in most of the cases and less to terminal. In females, genital duplication was present in all cases. The follow-up period varied between 3 months and 12 years. In all adult females, the motive of presentation was related to pregnancy (complications after successful delivery, fertility evaluation) or late complications (fecalith obstruction of the end-to-side colon anastomosis, repeated UTIs with renal scarring). Conclusions: Complex malformations affecting multiple caudal organs may have a strong impact in many aspects of the long-term quality of life; therefore, patients with caudal duplication syndrome need increased awareness and joined multidisciplinary treatment.

Download Full-text

The impact of surgical intervention on renal function in cystinuria

Brazilian Journal of Nephrology ◽

10.1590/2175-8239-jbn-2018-0034 ◽

2018 ◽

Vol 40 (3) ◽

pp. 256-260 ◽

Cited By ~ 1

Author(s):

Serra Sürmeli Döven ◽

Ali Delibaş ◽

Hakan Taşkınlar ◽

Ali Naycı

Keyword(s):

Renal Function ◽

Radionuclide Imaging ◽

Surgical Intervention ◽

Renal Scarring ◽

Urinary Stones ◽

Surgical Interventions ◽

Significant Difference ◽

Before And After ◽

The Impact

ABSTRACT Introduction: Cystinuria is an autosomal recessive disorder due to intestinal and renal transport defects in cystine and dibasic amino acids, which result in recurrent urolithiasis and surgical interventions. This study aimed to assess the impact of surgical interventions on renal function by analyzing estimated glomerular filtration rates. Methods: Thirteen pediatric patients with cystinuria, who were followed-up in a single tertiary institution between 2004 and 2016, were included in the study. Medical records were reviewed to collect data on clinical presentation of patients, urine parameters, stone formation, medical treatment, surgical intervention, stone recurrence after surgical procedure, stone analysis, ultrasonography, 99m-technetium dimercaptosuccinic acid (99mTc-DMSA) radionuclide imaging results, and follow-up time. Creatinine clearances estimated by modified Schwartz (eGFR) formula before and after surgery were used to assess renal function and compared statistically. Results: Nine patients (69.2%) had renal scarring which were detected with 99mTc-DMSA radionuclide imaging. In ten patients (76.9%), open surgical intervention for stones were needed during follow-up. Significant difference was not detected between eGFR before and after surgical intervention (mean 92 versus 106, p = 0.36). Nine of the patients (69.2%) were stone free in the last ultrasonographic examination. Relapses of stone after surgery were seen in 66.6% of patients who underwent surgical intervention. Conclusions: Surgical interventions for urinary stones are commonly required in patients with cystinuria. Renal scarring is a prevalent finding in cystinuric patients. Surgical interventions have no negative impact on eGFR in patients with cystinuria according to the present study.

Download Full-text

HPV Type 6 and 18 Coinfection in a Case of Adult-Onset Laryngeal Papillomatosis: Immunization with Gardasil

Case Reports in Otolaryngology ◽

10.1155/2015/916023 ◽

2015 ◽

Vol 2015 ◽

pp. 1-4

Author(s):

Virginia Fancello ◽

Andrea Melis ◽

Andrea Fausto Piana ◽

Paolo Castiglia ◽

Andrea Cossu ◽

...

Keyword(s):

Hpv Vaccine ◽

Case Reports ◽

Therapeutic Option ◽

Adult Onset ◽

Laryngeal Papillomatosis ◽

Surgical Interventions ◽

Potential Efficacy ◽

Hpv Types ◽

Immunogenic Response

Laryngeal papillomatosis (LP) is a rare human papillomavirus (HPV) related disease that often requires multiple surgical interventions and residual impairment of voice is almost inevitable. We report the case of a patient with adult onset recurrent LP, showing moderate dysplasia and coinfection with HPV types 6 and 18. The tetravalent HPV vaccine Gardasil was prescribed off label, with the aim of triggering an immunogenic response and consequently reducing the probability of further recurrences. The patient was followed for 9 months with no sign of relapse of his LP. The postexposure use of the anti-HPV vaccine could represent a promising therapeutic agent in established LP. Unfortunately, the potential efficacy of this new therapeutic option in this situation has been suggested only by isolated case reports. Further controlled studies, with a longer follow-up and a larger sample size, are needed to assess efficacy of Gardasil in LP.

Download Full-text

Severe Head Injury linked to Subsequent Development of Malignant Brain Tumour within a Short Period- A Case Report

Medical & Clinical Research ◽

10.33140/mcr.03.02.09 ◽

2018 ◽

Vol 3 (2) ◽

Keyword(s):

Head Injury ◽

Brain Tumour ◽

Severe Head Injury ◽

Case Reports ◽

Subsequent Development ◽

Malignant Brain Tumour ◽

Tumour Development ◽

Short Period ◽

The Brain

There have been a few case reports of head injury leading to brain tumour development in the same region as the brain injury. Here we report a case where the patient suffered a severe head injury with contusion. He recovered clinically with conservative management. Follow up Computed Tomography scan of the brain a month later showed complete resolution of the lesion. He subsequently developed malignant brain tumour in the same region as the original contusion within a very short period of 15 months. Head injury patients need close follow up especially when severe. The link between severity of head injury and malignant brain tumour development needs further evaluation. Role of anti-inflammatory agents for prevention of post traumatic brain tumours needs further exploration.

Download Full-text

Mechanical thrombectomy in pediatric stroke: systematic review, individual patient data meta-analysis, and case series

Journal of Neurosurgery Pediatrics ◽

10.3171/2019.5.peds19126 ◽

2019 ◽

Vol 24 (5) ◽

pp. 558-571 ◽

Cited By ~ 3

Author(s):

Kartik Bhatia ◽

Hans Kortman ◽

Christopher Blair ◽

Geoffrey Parker ◽

David Brunacci ◽

...

Keyword(s):

Systematic Review ◽

Ischemic Stroke ◽

Acute Ischemic Stroke ◽

Mechanical Thrombectomy ◽

Case Reports ◽

Meta Analysis ◽

Case Series ◽

Neurological Outcomes

OBJECTIVEThe role of mechanical thrombectomy in pediatric acute ischemic stroke is uncertain, despite extensive evidence of benefit in adults. The existing literature consists of several recent small single-arm cohort studies, as well as multiple prior small case series and case reports. Published reports of pediatric cases have increased markedly since 2015, after the publication of the positive trials in adults. The recent AHA/ASA Scientific Statement on this issue was informed predominantly by pre-2015 case reports and identified several knowledge gaps, including how young a child may undergo thrombectomy. A repeat systematic review and meta-analysis is warranted to help guide therapeutic decisions and address gaps in knowledge.METHODSUsing PRISMA-IPD guidelines, the authors performed a systematic review of the literature from 1999 to April 2019 and individual patient data meta-analysis, with 2 independent reviewers. An additional series of 3 cases in adolescent males from one of the authors’ centers was also included. The primary outcomes were the rate of good long-term (mRS score 0–2 at final follow-up) and short-term (reduction in NIHSS score by ≥ 8 points or NIHSS score 0–1 at up to 24 hours post-thrombectomy) neurological outcomes following mechanical thrombectomy for acute ischemic stroke in patients < 18 years of age. The secondary outcome was the rate of successful angiographic recanalization (mTICI score 2b/3).RESULTSThe authors’ review yielded 113 cases of mechanical thrombectomy in 110 pediatric patients. Although complete follow-up data are not available for all patients, 87 of 96 (90.6%) had good long-term neurological outcomes (mRS score 0–2), 55 of 79 (69.6%) had good short-term neurological outcomes, and 86 of 98 (87.8%) had successful angiographic recanalization (mTICI score 2b/3). Death occurred in 2 patients and symptomatic intracranial hemorrhage in 1 patient. Sixteen published thrombectomy cases were identified in children < 5 years of age.CONCLUSIONSMechanical thrombectomy may be considered for acute ischemic stroke due to large vessel occlusion (ICA terminus, M1, basilar artery) in patients aged 1–18 years (Level C evidence; Class IIb recommendation). The existing evidence base is likely affected by selection and publication bias. A prospective multinational registry is recommended as the next investigative step.

Download Full-text

THYROID TYPE CARCINOMA ARISING IN STRUMA OVARII. SIX CLINICAL CASES AND REVIEW

Problems in oncology ◽

10.37469/0507-3758-2018-64-6-708-715 ◽

2018 ◽

Vol 64 (6) ◽

pp. 708-715

Author(s):

Natalya Severskaya ◽

Andrey Rodichev ◽

Aleksey Ilin ◽

Dmitriy Semin ◽

Pavel Isaev ◽

...

Keyword(s):

Radioiodine Therapy ◽

Clinical Course ◽

Case Reports ◽

Thyroid Tissue ◽

Follicular Carcinoma ◽

Complete Response ◽

Pelvic Surgery ◽

Ovarian Teratoma ◽

Struma Ovarii

Struma ovarii is a rare variant of the mature ovarian teratoma composed of more than 50% thyroid tissue. Thyroid type carcinoma can occur in 5% of struma ovarii. Given the rarity of this pathology, as well as the different clinical course, approaches to the treatment of this disease are controversial. The proposed approaches to treatment vary from ovarian resection to total hysterectomy with bilateral salpingo-oophorectomy and adjuvant therapy. We present here 6 case reports of thyroid type carcinoma in struma ovarii and outcome of patients treated in our clinic. All patients had pelvic surgery of different extent, followed by thyroidectomy and radioiodine therapy. The incidence of metastasis is 67% (4/6), 2 - intraperitoneal metas-tases, 2 - bone metastases. Among patients with metastases, 2 have reached a complete response, one with a good response continues treatment, one had progression. The follow-up period is 1 to 15 years (median 4 years). One patient with follicular carcinoma died of progression 8 years after diagnosis. The remaining patients are alive.

Download Full-text

Outcomes of Multidisciplinary Care for Pediatric Post-Concussive Symptoms

Neurology ◽

10.1212/01.wnl.0000581004.98313.0c ◽

2019 ◽

Vol 93 (14 Supplement 1) ◽

pp. S18.1-S18

Author(s):

Jillian O’Neil ◽

Sean Rose ◽

Ashley Davidson ◽

Kathleen Shiplett ◽

Anthony Castillo ◽

...

Keyword(s):

Aerobic Exercise ◽

Psychological Intervention ◽

Multidisciplinary Treatment ◽

Symptom Burden ◽

Multidisciplinary Care ◽

Exercise Session ◽

Symptom Improvement ◽

Final Visit ◽

Post Injury ◽

History Of

ObjectiveTo evaluate the effectiveness of a multidisciplinary treatment approach for adolescents experiencing prolonged recovery from concussion.BackgroundAlthough most youth recover from a concussion within 2–4 weeks, an estimated 14% of those injured remain symptomatic at 3 months post-injury. For those experiencing protracted recovery, the 2017 Berlin Concussion in Sport Group Consensus Statement recommends multidisciplinary collaborative care. While recent research utilizing progressive aerobic exercise among adolescents with concussion has shown promise for reduction in symptom burden, limited evidence exists for multidisciplinary care.Design/MethodsParticipants included 39 adolescents (77% female, 87% Caucasian) referred to the Nationwide Children’s Hospital Complex Concussion Clinic. All patients included had persistent (≥30 days post-injury) post-concussion symptoms (SCAT-5 symptom score ≥10). The sample ranged in age from 11-20 years (mean = 15.0, SD = 2.0) and median days since injury was 60 (range = 30–161). 31% of participants had a history of one or more previous concussions, 54% had a history of anxiety or depression, and 26% had a history of ADHD or a learning disorder. The multidisciplinary treatment included sessions with Neurology (mean number of sessions = 2.5), Neuropsychology (mean = 2.1), Physical Therapy (mean = 3.6), and Athletic Training sessions involving graded physical exercise (mean = 4.0), with an average treatment duration of 57.4 days. SCAT-5 symptom rating scales were completed at each visit.ResultsSymptom burden among participants significantly decreased between their initial visit (mean = 49.6, SD = 19.2) and final exercise session (mean = 12.8, SD = 14.1); p < 0.001. Gender did not predict symptom ratings at treatment onset, though males (mean = 5.6) had significantly lower symptom scores than females (mean = 15.0) at their final visit (p < 0.05). Demographic factors and premorbid psychological history did not predict rate of symptom improvement.ConclusionsHigh rates of premorbid psychological problems are evident in children referred for treatment of PCS. Multidisciplinary care involving graded aerobic exercise and psychological intervention shows promise, though specific factors associated with treatment response remain to be elucidated.

Download Full-text

P084 DEEP REMISSION WITH DOUBLE BIOLOGIC THERAPY: A SUCCESSFUL CASE OF COMBINATION USTEKINUMAB AND VEDOLIZUMAB FOR SEVERE REFRACTORY CROHN’S DISEASE

Inflammatory Bowel Diseases ◽

10.1093/ibd/zaa010.182 ◽

2020 ◽

Vol 26 (Supplement_1) ◽

pp. S72-S72

Author(s):

Ahmed Elmoursi ◽

Courtney Perry ◽

Terrence Barrett

Keyword(s):

Crohn’S Disease ◽

Crohn's Disease ◽

Combination Therapy ◽

Clinical Remission ◽

Biologic Therapy ◽

Case Reports ◽

Sigmoid Resection ◽

Safety And Efficacy ◽

Ileal Stricture

Abstract Background Stricturing Crohn’s disease (CD) constitutes a severe phenotype often associated with a high degree of morbidity (3). Surgical resection is first-line therapy for symptomatic strictures, but most patients relapse without subsequent medical therapy (4–5). Biologics are the mainstay for inducing and maintaining remission, but some cases are refractory despite maximum dosage of therapy. Reports of dual biological therapy (DBT) in refractory, stricturing CD are sparse, and prior case reports document only clinical remission (1). To contribute further knowledge regarding the use of DBT in stricturing CD, we present the case of a refractory CD patient who achieved deep remission with ustekinumab and vedolizumab. Case Presentation A 35 year old non-smoking, Caucasian male was referred to our clinic in 2014 for refractory CD complicated by multiple strictures. Prior to establishing care with us, he received two jejunal resections and a sigmoid resection. Previously failed therapies included azathioprine with infliximab, adalimumab, and certolizumab. He continued to progress under our care despite combination methotrexate/certolizumab, as well as methotrexate/golimumab. He underwent proctocolectomy with end ileostomy in 2015 and initiated vedolizumab q8weeks post-operatively. He reoccurred in 2018, when he presented with an ulcerated ileal stricture. He was switched from vedolizumab to ustekinumab q8weeks and placed on prednisone, but continued to progress, developing significant hematochezia requiring hospitalization and blood transfusions. Ileoscopy performed during hospital admission confirmed severe, ulcerating disease in the ileum with stricture. Ustekinumab dosing was increased to q4weeks, azathioprine was initiated, and he underwent stricturoplasty. Follow-up ileoscopy three months later revealed two ulcers in the neo- TI (Figure 1). Vedolizumab q8weeks was initiated in addition to ustekinumab q4weeks and azathioprine 125mg. After four months on this regimen the patient felt better, but follow-up ileoscopy showed two persistent ulcers in the neo-TI. Vedolizumab dosing interval was increased to q4weeks. After four months, subsequent ileoscopy demonstrated normal neo-TI (Figure 2). Histologic evaluation of biopsies confirmed deep remission of crohn’s disease. No adverse side effects have occurred with maximum doses of both ustekinumab and vedolizumab combination therapy. Discussion This case supports both the safety and efficacy of ustekinumab and vedolizumab dual biologic therapy for treatment of severe, refractory Crohn’s disease. While there are reports of DBT inducing clinical remission, this case supports efficacy for vedolizumab and ustekinumab combination therapy to induce deep histologic remission. Large practical clinical trials are needed to better investigate the safety and efficacy of DBT with vedolizumab and ustekinumab, but our case suggests this combination may be a safe and efficacious therapy for refractory CD patients.

Download Full-text

Spontaneous haemorrhage of an adrenal angiomyolipoma: case report

African Journal of Urology ◽

10.1186/s12301-019-0006-1 ◽

2019 ◽

Vol 25 (1) ◽

Author(s):

Danielle Whiting ◽

Ian Rudd ◽

Amit Goel ◽

Seshadri Sriprasad ◽

Sanjeev Madaan

Keyword(s):

Case Reports ◽

Case Presentation ◽

Open Adrenalectomy ◽

Large Size ◽

History Of ◽

Loin Pain ◽

Benign Tumours ◽

Spontaneous Haemorrhage ◽

Mesenchymal Tumours

Abstract Background Angiomyolipomas are rare mesenchymal tumours arising from the perivascular epithelioid cells consisting of variable amounts of adipose, thick-walled blood vessels and smooth muscle cells. These benign tumours commonly occur in the kidney with only a few case reports of adrenal angiomyolipomas which have the potential to reach a large size and haemorrhage. Case presentation A 45-year-old lady presented with a 3-week history of right loin pain, nausea and vomiting. A CT scan revealed a right adrenal angiomyolipoma measuring 6.3 × 6.8 cm with associated haemorrhage. The lesion was successfully treated with right open adrenalectomy, and histology confirmed the diagnosis of adrenal angiomyolipoma. The patient remained well with no evidence of recurrence at the 36-month follow-up. Conclusion Adrenal angiomyolipomas are rare benign tumours that have the ability to reach a large size and potential to bleed. Here, we report the second case of spontaneous haemorrhage in an adrenal angiomyolipoma, which was successfully treated with open adrenalectomy.

Download Full-text

Immediate Post-Extraction Short Implant Placement with Immediate Loading and without Extraction of an Impacted Maxillary Canine: Two Case Reports

Materials ◽

10.3390/ma14112757 ◽

2021 ◽

Vol 14 (11) ◽

pp. 2757

Author(s):

José Antonio Moreno-Rodríguez ◽

Julia Guerrero-Gironés ◽

Francisco Javier Rodríguez-Lozano ◽

Miguel Ramón Pecci-Lloret

Keyword(s):

Dental Implant ◽

Alveolar Bone ◽

Case Reports ◽

Young Patients ◽

Aesthetic Result ◽

First Choice ◽

Surgical Extraction ◽

Impacted Canine ◽

Short Implant

For the treatment of impacted maxillary canines, traction associated with a complete orthodontic treatment is the first choice in young patients. However, in adults, this treatment has a worse prognosis. The surgical extraction of the impacted tooth can result in a series of complications and a compromised alveolar bone integrity, which may lead to the requirement of a bone regeneration/grafting procedure to replace the canine with a dental implant. These case reports aimed to describe an alternative treatment procedure to the surgical extraction of impacted maxillary canines in adults. Following clinical and computerized tomography-scan (CT-Scan) examination, the possibility of maintaining the impacted canine in its position and replacing the temporary canine present in its place with a dental implant was planned. A short dental implant with an immediate provisional crown was placed, without contacting the impacted canine. At 3 months follow-up, a definitive metal-ceramic restoration was placed. Follow-up visits were performed periodically. The implant site showed a physiological soft tissue color and firmness, no marginal bone loss, no infection or inflammation, and an adequate aesthetic result in all follow-up visits. These results suggest that the treatment carried out is a valid option to rehabilitate with an osseointegrated short implant area where a canine is included, as long as there is a sufficient amount of the remaining bone.

Download Full-text

The Long-Term Follow-Up of Patients with Cystine Stones: A Single-Center Experience for 13 Years

Journal of Clinical Medicine ◽

10.3390/jcm10071336 ◽

2021 ◽

Vol 10 (7) ◽

pp. 1336

Author(s):

Toshifumi Takahashi ◽

Shinya Somiya ◽

Katsuhiro Ito ◽

Toru Kanno ◽

Yoshihito Higashi ◽

...

Keyword(s):

Surgical Intervention ◽

Median Number ◽

Small Sample ◽

Age At Diagnosis ◽

Surgical Interventions ◽

Significant Difference ◽

Long Term Follow Up ◽

Cystine Stones

Introduction: Cystine stone development is relatively uncommon among patients with urolithiasis, and most studies have reported only on small sample sizes and short follow-up periods. We evaluated clinical courses and treatment outcomes of patients with cystine stones with long-term follow-up at our center. Methods: We retrospectively analyzed 22 patients diagnosed with cystine stones between January 1989 and May 2019. Results: The median follow-up was 160 (range 6–340) months, and the median patient age at diagnosis was 46 (range 12–82) years. All patients underwent surgical interventions at the first visit (4 extracorporeal shockwave lithotripsy, 5 ureteroscopy, and 13 percutaneous nephrolithotripsy). The median number of stone events and surgical interventions per year was 0.45 (range 0–2.6) and 0.19 (range 0–1.3) after initial surgical intervention. The median time to stone events and surgical intervention was 2 years and 3.25 years, respectively. There was a significant difference in time to stone events and second surgical intervention when patients were divided at 50 years of age at diagnosis (p = 0.02, 0.04, respectively). Conclusions: Only age at a diagnosis under 50 was significantly associated with recurrent stone events and intervention. Adequate follow-up and treatment are needed to manage patients with cystine stones safely.

Download Full-text