scholarly journals Rare Fetus-in-Fetu: Experience From a Large Tertiary Pediatric Referral Center

2021 ◽  
Vol 9 ◽  
Author(s):  
Mao Xiaowen ◽  
Cheng Lingxi ◽  
Lin Song ◽  
Pan Shengbao ◽  
Yang Xiaohong ◽  
...  
Keyword(s):  
Open Surgery ◽  
Clinical Characteristics ◽  
Ultrasound Examination ◽  
Single Case ◽  
Case Reports ◽  
Alpha Fetoprotein ◽  
Pathological Examination ◽  
Fetus In Fetu ◽  
Antenatal Ultrasound ◽  
Referral Center

Objective: Fetus-in-fetu (FIF) is an extremely rare disease, and most prior publications are single case reports. Here, we describe the clinical characteristics, imaging manifestations, and the treatment and related complications of FIF from a large tertiary pediatric referral center.Materials: After institutional review board approval, patients with a diagnosis of FIF between January 2010 and November 2019 were further selected and reexamined. We analyzed the general clinical characteristics, imaging manifestations, treatment, and prognosis of the patients.Results: A total of seven (four male and three female) patients with FIF were included in the study. All patients were diagnosed with FIF during the antenatal ultrasound examination along with an abnormal increase in alpha fetoprotein, and it was confirmed by subsequent pathological examination. The median gestation period when FIF was first diagnosed was 25 (range: 22–32) weeks. Ultrasound, computed tomography, and magnetic resonance imaging were the main pre-operative diagnostic techniques used. All patients underwent FIF resection within 1 month after birth: four patients had open surgery and three had laparoscopic surgeries (one case was converted to open surgery); only one patient developed ascites after surgery. All patients are growing up healthy and without tumor recurrence at the last follow-up. The level of alpha fetoprotein decreased to normal within 1 year (range 3-10 months) after surgery performed.Conclusion: As the size of the FIF increases, it can be found and diagnosed in antenatal ultrasound examination. Surgery is an important curative treatment for FIF and generally results in excellent long-term quality of life.

Report of new case of Pallister – Killian syndrome in fetus

2018 ◽  
Author(s):  
I.V. Novikova, E.G. Trebka, S.I. Kovalev, et all
Keyword(s):  
In Situ Hybridization ◽  
Fluorescent In Situ Hybridization ◽  
Ultrasound Examination ◽  
Pathological Examination ◽  
Second Trimester ◽  
Antenatal Ultrasound ◽  
Isochromosome 12P ◽  
Additional Chromosome ◽  
Mosaic Distribution

Pallister — Killian syndrome (PKS) is a sporadic chromosomal anomaly caused by a tissue-specific mosaic distribution of an additional isochromosome 12p. A case of second trimester fetus with PKS is presented. Antenatal ultrasound examination showed left diaphragmatic hernia. Standard cytogenetic analysis of cultured amniocytes supposed mosaic tetrasomy 12p: 46,XY/ 47,XY,+?i(12p). Fluorescent in situ hybridization (FISH) was used to confirm the nature of the additional chromosome. Pathological examination confirmed prenatal diagnosis.

Fetus in Fetu: Lessons Learned from a Large Multicenter Cohort Study

2019 ◽  
Vol 30 (04) ◽  
pp. 343-349
Author(s):  
Heba M. A. Taher ◽  
Mostafa Abdellatif ◽  
Ahmed Mohamed Kadry Wishahy ◽  
Saber Waheeb ◽  
Yasser Saadeldin ◽  
...  
Keyword(s):  
Pediatric Surgery ◽  
Single Case ◽  
Case Reports ◽  
Lessons Learned ◽  
Pathology Report ◽  
Multicenter Cohort Study ◽  
Complete Excision ◽  
Diagnostic Modality ◽  
Fetus In Fetu ◽  
Increased Risk

Abstract Introduction Fetus in fetu (FIF) is an extremely rare condition of abnormal twinning during embryogenesis. Most publications are single case reports. We describe the combined experience of four large tertiary referral centers with FIF which were not previously reported or published, and thereby draw conclusions to establish criteria for the workup, diagnosis, and management including intraoperative risk. Materials and Methods A survey was forwarded to a national pediatric surgery group which includes members from all pediatric surgery centers in the country enquiring about unpublished cases of FIF encountered over a 20-year interval. The cohort was analyzed for age of presentation, type of presentation, diagnostic workup, surgical management, and outcome. Results From 1998 to 2018, a total of 10 FIF cases were included in the study. Mean age of presentation was 4 months. Computed tomography and ultrasound were the main preoperative diagnostic modality in our cohort. Resection of the mass was curative in nine cases. Two cases in which the FIF was in direct topographic proximity to the biliary tree suffered severe intraoperative or lethal postoperative complications. Conclusion Complete excision of FIF is the treatment of choice and generally results in excellent long-term quality of life. Mortality is rare and may be associated with biliary involvement and retroperitoneal right upper quadrant location of the FIF tends to be associated with increased risk in excision, and there is also a possible association with the presence of immature elements in the pathology report.

scholarly journals Intracranial aneurysms associated with middle cerebral artery anomalies: the clinical characteristics and treatment considerations: a systematic review

2019 ◽  
Author(s):  
Kun Hou ◽  
Guichen Li ◽  
Hongping Liu ◽  
Kan Xu ◽  
Jinlu Yu
Keyword(s):  
Systematic Review ◽  
Intracranial Aneurysms ◽  
Open Surgery ◽  
Clinical Characteristics ◽  
Hemorrhagic Stroke ◽  
Case Reports ◽  
Case Series ◽  
Good Recovery ◽  
Treatment Considerations ◽  
Pubmed Search

Abstract Background: As a result of its low occurrence, most of the studies on intracranial aneurysms associated with MCA anomalies were presented as case reports or small case series. In this study, a systematic review on this specific entity was conducted. Methods: A PubMed search of the published studies was performed on April 6th, 2019 for patients who had intracranial aneurysms associated with MCA anomalies. The languages included in this study were English, Chinese, and Japanese. Results: Finally, 58 articles reporting of 67 patients including 1 case in our center were included. The identified patients (37 females, 55.2%) aged from 4 to 81 (49.85 ± 15.22) years old. Fifty (50/67, 74.6%) patients presented with hemorrhagic stroke either because of rupture of the aneurysms associated with MCA anomalies or other sources. Sixty-three aneurysms (63/67, 94.0%) were saccular, 3 (4.5%) were dissecting or fusiform, and 1 (1.5%) was pseudoaneurysm. Thirty-two (32/65, 49.2%) patients had other concurrent cerebrovascular anomalies. Fifty-six (83.6%) patients underwent open surgeries, 8 (11.9%) patients underwent endovascular treatment, and 3 (4.5%) patients were conservatively managed. Fifty-six (56/61, 91.8%) patients achieved a good recovery. Conclusions: The pathophysiological genesis of MCA anomalies associated aneurysms is still obscure to us. The inflicted patients tend to have concurrent other cerebrovascular anomalies, which denotes that congenital defect in cerebrovascular development might play a role in this process. Open surgery is, hitherto, the mainstay of treatment for this specific entity. Most of the affected patients could experience a good recovery.

Management of Primary Retroperitoneal Mucinous Tumors: A Retrospective Meta-Analysis

2017 ◽  
Vol 27 (6) ◽  
pp. 1064-1071 ◽  
Author(s):  
Benjamin Wolf ◽  
Christin Kunert ◽  
Lars-Christian Horn ◽  
Jens Einenkel
Keyword(s):  
Confidence Interval ◽  
Cox Regression ◽  
Single Case ◽  
Case Reports ◽  
Meta Analysis ◽  
Case Series ◽  
Regression Modeling ◽  
Hazard Ratio ◽  
Pathological Examination ◽  
Primary Retroperitoneal

ObjectivePrimary retroperitoneal mucinous tumors (PRMTs) are rare epithelial neoplasms arising in the retroperitoneal space whose pathogenesis is poorly understood. To date, only single-case reports and small case series on diagnosis and management of these tumors exist. The aim of this first-time meta-analysis was to provide more reliable data to inform the management of PRMTs.MethodsDatabase searches were conducted to identify case reports and case series between the years of 1975 and 2015. Two cases of patients treated at our institution were also included. Diagnostic, radiographic, surgical, and follow-up data were gathered. Regression modeling was used to identify factors aiding preoperative diagnosis and affecting treatment. Survival analysis including Cox regression modeling was performed to provide insights into treatment effects on the prognosis of female patients with PRMTs.ResultsOne hundred forty-four cases of PRMTs were included in this analysis. Eighty-nine percent occurred in women. Most of the PRMTs were found to be malignant (53%). No preoperative factors predicting tumor dignity were identified by multiple regression analysis. Five-year disease specific survival of patients with benign and borderline tumors was excellent, reaching 100%, whereas it was only 68% for patients with malignant disease. Adjuvant surgical treatments including hysterectomy, salpingoophorectomy, and lymph node dissection were not associated with a statistically significant survival advantage. However, there was a nonsignificant association of hysterectomy with improved survival (hazard ratio, 0.42; 95% confidence interval, 0.09–2.07; P = 0.285). Adjuvant chemotherapy was associated with reduced survival (hazard ratio 3.791; 95% confidence interval, 1.509–9.526; P = 0.0046).ConclusionsA reliable diagnosis of a PRMT can only be made by pathological examination of the tumor. Surgical excision is thus necessary to facilitate diagnosis while it also remains the mainstay of treatment. There is no conclusive evidence supporting the role of adjuvant surgical procedures or chemotherapy.

scholarly journals The clinical characteristics and treatment considerations for intracranial aneurysms associated with middle cerebral artery anomalies: a systematic review

2020 ◽  
Author(s):  
Kun Hou ◽  
Guichen Li ◽  
Hongping Liu ◽  
Kan Xu ◽  
Jinlu Yu
Keyword(s):  
Systematic Review ◽  
Intracranial Aneurysms ◽  
Open Surgery ◽  
Clinical Characteristics ◽  
Hemorrhagic Stroke ◽  
Case Reports ◽  
Case Series ◽  
Good Recovery ◽  
Treatment Considerations ◽  
Pubmed Search

Abstract Background: As a result of its low occurrence, most of the studies on intracranial aneurysms associated with MCA anomalies were presented as case reports or small case series. In this study, a systematic review on this specific entity was conducted.Methods: A PubMed search of the published studies was performed on April 6th, 2019 for patients who had intracranial aneurysms associated with MCA anomalies. The languages included in this study were English, Chinese, and Japanese.Results: Finally, 58 articles reporting of 67 patients including 1 case in our center were included. The identified patients (37 females, 55.2%) aged from 4 to 81 (49.85 ± 15.22) years old. Fifty (50/67, 74.6%) patients presented with hemorrhagic stroke either from the MCA anomalies associated aneurysms or other sources. Sixty-three aneurysms (63/67, 94.0%) were saccular, 3 (4.5%) were dissecting or fusiform, and 1 (1.5%) was pseudoaneurysm. Thirty-two (32/65, 49.2%) patients had other concurrent cerebrovascular anomalies. Fifty-six (83.6%) patients underwent open surgeries, 8 (11.9%) patients underwent endovascular treatment, and 3 (4.5%) patients were conservatively managed. Fifty-six (56/61, 91.8%) patients achieved a good recovery.Conclusions: The pathophysiological genesis of MCA anomalies associated aneurysms is still obscure to us. The inflicted patients tend to have other concurrent cerebrovascular anomalies, which denotes that congenital defect in cerebrovascular development might play a role in this process. Open surgery is, hitherto, the mainstay of treatment for this specific entity. Most of the affected patients could experience a good recovery after treatment.

scholarly journals Post-Infectious Guillain–Barré Syndrome Related to SARS-CoV-2 Infection: A Systematic Review

Life ◽  
2021 ◽  
Vol 11 (2) ◽  
pp. 167
Author(s):  
Pasquale Sansone ◽  
Luca Gregorio Giaccari ◽  
Caterina Aurilio ◽  
Francesco Coppolino ◽  
Valentina Esposito ◽  
...  
Keyword(s):  
Single Case ◽  
Case Reports ◽  
Case Series ◽  
Guillain Barre Syndrome ◽  
Response To Treatment ◽  
Comprehensive Overview ◽  
Fisher Syndrome ◽  
Immunomodulating Therapy ◽  
Guillain Barré Syndrome ◽  
Guillain Barré

Background. Guillain-Barré syndrome (GBS) is the most common cause of flaccid paralysis, with about 100,000 people developing the disorder every year worldwide. Recently, the incidence of GBS has increased during the severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) epidemics. We reviewed the literature to give a comprehensive overview of the demographic characteristics, clinical features, diagnostic investigations, and outcome of SARS-CoV-2-related GBS patients. Methods. Embase, MEDLINE, Google Scholar, and Cochrane Central Trials Register were systematically searched on 24 September 2020 for studies reporting on GBS secondary to COVID-19. Results. We identified 63 articles; we included 32 studies in our review. A total of 41 GBS cases with a confirmed or probable COVID-19 infection were reported: 26 of them were single case reports and 6 case series. Published studies on SARS-CoV-2-related GBS typically report a classic sensorimotor type of GBS often with a demyelinating electrophysiological subtype. Miller Fisher syndrome was reported in a quarter of the cases. In 78.1% of the cases, the response to immunomodulating therapy is favourable. The disease course is frequently severe and about one-third of the patients with SARS-CoV-2-associated GBS requires mechanical ventilation and Intensive Care Unit (ICU) admission. Rarely the outcome is poor or even fatal (10.8% of the cases). Conclusion. Clinical presentation, course, response to treatment, and outcome are similar in SARS-CoV-2-associated GBS and GBS due to other triggers.

Analysis of Clinical, Imaging, and Pathologic Features of 36 Patients with Primary Intraspinal Primitive Neuroectodermal Tumors: A Case Series and Literature Review

2021 ◽  
Author(s):  
Xuefeng Wei ◽  
Xu Zhang ◽  
Zimu Song ◽  
Feng Wang
Keyword(s):  
Literature Review ◽  
Single Case ◽  
Case Reports ◽  
Case Series ◽  
Subtotal Resection ◽  
Study Cohort ◽  
Primitive Neuroectodermal Tumors ◽  
Pathologic Features ◽  
Neuroectodermal Tumors ◽  
The Mean

Abstract Background and Study Aims Primary intraspinal primitive neuroectodermal tumors (PNETs) account for ∼0.4% of all intraspinal tumors, but information about these tumors in the medical literature is limited to single case reports. We report four cases of primary intraspinal PNETs and present a systematic literature review of the reported cases. Materials and Methods We retrospectively reviewed and analyzed the clinical data of 4 patients with primary intraspinal PNETs who underwent neurosurgical treatment at our clinic between January 2013 and January 2020, and of 32 cases reported in the literature. Results The female-to-male ratio was 2.6:1. The mean patient age was 21.42 ± 15.76 years (range: 1–60 years), and patients <36 years of age accounted for 83.30% of the study cohort. Progressive limb weakness and numbness were the chief symptoms (accounting for ∼55.6%). The mean complaint duration was 0.89 ± 0.66 months for males and 2.72 ± 3.82 months for females (p = 0.028). Epidural (41.7%) was the most common site, and thoracic (47.3%) was the most frequent location. Most PNETs were peripheral, and magnetic resonance imaging (MRI) appearance was isointense or mildly hypointense on T1-weighted images and hyperintense on T2-weighted images. Homogeneous contrast enhancement was observed. The 1-year survival rate of patients who underwent chemoradiation after total or subtotal lesion resection was better compared with patients who did not undergo chemotherapy, radiotherapy, or total or subtotal resection. The modality of treatment was associated with survival time (p = 0.007). Conclusion Primary intraspinal PNETs mainly occur in young people with a female preponderance. In patients with a rapid loss of lower limb muscle strength and large intraspinal lesions on MRI, PNETs should be considered. Surgical resection and adjuvant radio chemotherapy are key prognostic factors.

scholarly journals Fetus in fetu: two case reports from North African country

2021 ◽  
Vol 69 (1) ◽  
Author(s):  
Moutaz Ragab ◽  
Omar Nagy Abdelhakeem ◽  
Omar Mansour ◽  
Mai Gad ◽  
Hesham Anwar Hussein
Keyword(s):  
Histopathological Examination ◽  
Case Reports ◽  
Abdominal Mass ◽  
Mature Teratoma ◽  
Surgical Techniques ◽  
Rare Condition ◽  
Mass Effect ◽  
Fetus In Fetu ◽  
Vascular Connection ◽  
First Case

Abstract Background Fetus in fetu is a rare congenital anomaly. The exact etiology is unclear; one of the mostly accepted theories is the occurrence of an embryological insult occurring in a diamniotic monochorionic twin leading to asymmetrical division of the blastocyst mass. Commonly, they present in the infancy with clinical picture related to their mass effect. About 80% of cases are in the abdomen retroperitoneally. Case presentation We present two cases of this rare condition. The first case was for a 10-year-old girl that presented with anemia and abdominal mass, while the second case was for a 4-month-old boy that was diagnosed antenatally by ultrasound. Both cases had vertebrae, recognizable fetal organs, and skin coverage. Both had a distinct sac. The second case had a vascular connection with the host arising from the superior mesenteric artery. Both cases were intra-abdominal and showed normal levels of alpha-fetoprotein. Histopathological examination revealed elements from the three germ layers without any evidence of immature cells ruling out teratoma as a differential diagnosis. Conclusions Owing to its rarity, fetus in fetu requires a high degree of suspicion and meticulous surgical techniques to avoid either injury of the adjacent vital structures or bleeding from the main blood supply connection to the host. It should be differentiated from mature teratoma.

scholarly journals Mesenteric Cysts: A Retrospective Review. Its Not All That Simple

2020 ◽  
Vol 154 (Supplement_1) ◽  
pp. S117-S118
Author(s):  
M Bourgeau ◽  
V Avadhani
Keyword(s):  
Cyst Wall ◽  
Imaging Techniques ◽  
Case Reports ◽  
Lymphatic Vessels ◽  
Mucinous Cystadenoma ◽  
Final Diagnosis ◽  
Cystic Mass ◽  
Pathological Examination ◽  
Urachal Cyst ◽  
Mesenteric Cysts

Abstract Introduction/Objective Mesenteric cysts are rare intra-abdominal lesions in adults. However, with the advanced imaging techniques and laparoscopic techniques, they are more often being identified and resected when clinically significant. There is a lack of detailed information in histopathology (except as case reports) since mesentery is generally neglected in our organ-based textbooks. The aim of our study is to highlight the importance of identifying and classifying mesenteric cystic lesions; they are not all that simple. Methods We performed a retrospective search on all mesenteric cysts submitted as excisions in our electronic database from 2013-2019. We classified them as per the de Perrot (PMID: 11053936) classification with modification. Results Our search showed: A. Lymphatic origin-11 (lymphangioma-10, Lymphangioma hamartomatous-1, associated with LAM-0), B. Mesothelial origin-68 (Benign mesothelial cysts-57, multilocular mesothelial cyst-11), C. Enteric origin- 3, D. Urogenital origin (Urachal cyst, mullerian inclusion cyst)-9, E. Mature cystic teratom-2, F. Pseudocyst-12, G. Epithelial cyst (not urogenital)- 11 (a/w LAMN-3, MCN-4, Mucinous cystadenoma-4), H. Associated with carcinoma-2. Case illustration: A 61-year-old male presented with worsening dysphagia, emesis and hiccups. A CT scan showed a 21.2 cm cystic mass with at least one septation (Fig 1). The cyst was resected. On gross pathological examination, the cyst measured 18 cm in greatest dimension with a thick, rough, tan-brown capsule. Microscopic examination showed a fibrous capsule, and cyst wall composed of numerous lymphatic vessels (CD31 positive) and prominent smooth muscle proliferation (Desmin positive). Scattered lymphoid aggregates were also present throughout the cyst wall. No definite epithelial lining was identified and was suspected to have been denuded. HMB-45 immunostain was negative, ruling out association with LAM. The final diagnosis of a Lymphangiomyoma, hamartomatous was rendered. Conclusion Though most of the mesenteric cysts are benign, some of them are significantly important such as Lymphangiomyoma (esp secondary to LAM), MCN, those associated with LAMN etc. and identifying and differentiating from their mimics has distinct clinical implications.

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