scholarly journals Robotic removal of Müllerian duct remnants in pediatric patients: our experience and a review of the literature

2018 ◽  
Author(s):  
Mario Lima ◽  
Michela Maffi ◽  
Niel Di Salvo ◽  
Giovanni Ruggeri ◽  
Michele Libri ◽  
...  
Keyword(s):  
Primary Treatment ◽  
Invasive Technique ◽  
Mullerian Duct ◽  
Robot Assisted ◽  
Müllerian Duct ◽  
First Case ◽  
Disorder Of Sexual Development ◽  
Prostatic Utricle ◽  
Tract Infections

Persistent Müllerian duct syndrome is a disorder of sexual development, which features a failure of involution of Müllerian structures. An enlarged prostatic utricle is a kind of Müllerian duct remnant (MDR) with a tubular shaped structure communicating with the prostatic urethra. Treatment is aimed at relieving symptoms when present, preserve fertility and prevent neoplastic degeneration. We describe 3 cases of successful robot assisted-removal of symptomatic MDRs. The first case came to our attention for pseudo-incontinence; the other two for recurrent urinary tract infections. The patients have not presented such symptoms anymore on follow-up. We then reviewed existent literature on authors who have recently investigated the main issues concerning MDRs and have attempted a roboticassisted approach on them. Robot-assisted laparoscopy can be considered a valid, safe and effective minimally-invasive technique for the primary treatment of prostatic utricle.

A mutation inactivating the distal SF1 binding site on the human anti-Müllerian hormone promoter causes persistent Müllerian duct syndrome

2019 ◽  
Vol 28 (19) ◽  
pp. 3211-3218 ◽  
Author(s):  
Helena F Schteingart ◽  
Jean-Yves Picard ◽  
Clara Valeri ◽  
Ian Marshall ◽  
Dominique Treton ◽  
...  
Keyword(s):  
Binding Site ◽  
Mullerian Duct ◽  
Mobility Shift ◽  
Müllerian Duct ◽  
Persistent Müllerian Duct Syndrome ◽  
First Case ◽  
Disorder Of Sexual Development ◽  
Persistent Mullerian Duct Syndrome ◽  
The Difference ◽  
Duct Syndrome

AbstractThe persistent Müllerian duct syndrome (PMDS) is a 46,XY disorder of sexual development characterized by the persistence of Müllerian duct derivatives, uterus and tubes, in otherwise normally masculinized males. The condition, transmitted as a recessive autosomal trait, is usually due to mutations in either the anti-Müllerian hormone (AMH) gene or its main receptor. Many variants of these genes have been described, all targeting the coding sequences. We report the first case of PMDS due to a regulatory mutation. The AMH promoter contains two binding sites for steroidogenic factor 1 (SF1), one at −102 and the other at −228. Our patient carries a single base deletion at −225, significantly decreasing its capacity for binding SF1, as measured by the electrophoresis mobility shift assay. Furthermore, by linking the AMH promoter to the luciferase gene, we show that the transactivation capacity of the promoter is significantly decreased by the mutation, in contrast to the disruption of the −102 binding site. To explain the difference in impact we hypothesize that SF1 could partially overcome the lack of binding to the −102 binding site by interacting with a GATA4 molecule linked to a nearby response element. We show that disruption of both the −102 SF1 and the −84 GATA response elements significantly decreases the transactivation capacity of the promoter. In conclusion, we suggest that the distance between mutated SF1 sites and potentially rescuing GATA binding motifs might play a role in the development of PMDS.

scholarly journals Urinothorax Caused by Xanthogranulomatous Pyelonephritis

2018 ◽  
Vol 2018 ◽  
pp. 1-3
Author(s):  
Waiel Abusnina ◽  
Hazim Bukamur ◽  
Zeynep Koc ◽  
Fauzi Najar ◽  
Nancy Munn ◽  
...  
Keyword(s):  
Urinary Tract ◽  
Obstructive Uropathy ◽  
Xanthogranulomatous Pyelonephritis ◽  
Robot Assisted ◽  
Rare Form ◽  
X Ray ◽  
History Of ◽  
Chest X Ray ◽  
Tract Infections

Xanthogranulomatous pyelonephritis is a rare form of chronic pyelonephritis that generally afflicts middle-aged women with a history of recurrent urinary tract infections. Its pathogenesis generally involves calculus obstructive uropathy and its histopathology is characterized by replacement of the renal parenchyma with lipid filled macrophages. This often manifests as an enlarged, nonfunctioning kidney that may be complicated by abscess or fistula. This case details the first reported case of xanthogranulomatous pyelonephritis complicated by urinothorax, which resolved on follow-up chest X-ray after robot-assisted nephrectomy.

Two cases of nasopharyngeal branchial cleft cyst treated by powered instrument assisted marsupialisation

2013 ◽  
Vol 127 (6) ◽  
pp. 614-618 ◽  
Author(s):  
Y W Kim ◽  
M-J Baek ◽  
K H Jung ◽  
S K Park
Keyword(s):  
Case Report ◽  
Surgical Treatment ◽  
Literature Review ◽  
Sleep Apnoea ◽  
Invasive Technique ◽  
Branchial Cleft Cyst ◽  
First Case ◽  
Branchial Cleft ◽  
The Right

AbstractObjective:We report two extremely rare cases of symptomatic nasopharyngeal branchial cleft cyst treated by powered instrument assisted marsupialisation.Methods:Case report and literature review concerning nasopharyngeal branchial cleft cyst and surgical treatment methods.Results:The first case was a two-year-old boy with a 1 × 2 cm, cystic, oropharyngeal mass, who also had severe snoring and sleep apnoea. The second case was a 56-year-old man with right nasal obstruction and a sensation of fullness in the right ear. In both cases, we performed endoscopic marsupialisation using a powered instrument. There was no recurrence in either case over two years of follow up.Conclusion:Powered instrument marsupialisation is a simple, effective and less invasive technique for the treatment of nasopharyngeal branchial cleft cyst.

V539 ROBOT-ASSISTED LAPAROSCOPIC EXCISION OF A MULLERIAN DUCT REMANANT

2012 ◽  
Vol 187 (4S) ◽  
Author(s):  
Ying Hua Jura ◽  
Saman Shafaat Talab ◽  
Rafael Pieretti ◽  
Shahin Tabatabaei
Keyword(s):  
Mullerian Duct ◽  
Robot Assisted ◽  
Müllerian Duct ◽  
Laparoscopic Excision
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