Cystic Dilatation of the Ventriculus Terminalis: Examining the Relevance of the Revised Operative Classification Through a Systematic Review of the Literature, 2011-2021.

The literature features limited evidence on the natural history of the cystic dilatation of the ventriculus terminalis (CDVT) and its response to treatment. The goal of this study is to ascertain which impact the revised operative classification of CDVT had on the management of patients diagnosed over the past 10 years.Ten new clinical articles presenting a total of 30 cases of CDVT were identified and included for qualitative analysis. Two take-home messages can be identified: (1) Adequate consideration should be given to designing national pathways for referral to tertiary centers with relevant expertise in the management of lesions of the conus medullaris, and (2) we suggest that type Ia should be, at least initially, treated conservatively, whereas we reckon that the signs and symptoms described in types Ib, II, and III seem to benefit, although in some patients only partially, from surgical decompression in the form of cystic fenestration, cyst-subarachnoid shunting, or both.While the level of evidence gathered in this systematic review remains low because the literature on CDVT consists only of retrospective studies based on single-center series (level of evidence 4 according to the Oxford Centre for Evidence-Based Medicine (OCEBM)), the strength of recommendation for adopting the revised operative classification of CDVT is moderate.

Resección laparoscópica de dilatación quística congénita del conducto cístico en un paciente pediátrico / Laparoscopic Resection of Congenital Cystic Dilatation of the Cystic Duct in a Pediatric Patient

Introducción: La dilatación quística congénita del conducto cístico o dilatación tipo VI de la clasificación de Todani, es una variante rara de dilatación congénita de la vía biliar. Objetivo: Explicar la metodología diagnóstica y terapéutica empleada en un caso pediátrico con esta entidad, y destacar la ventaja de su tratamiento oportuno por vía mínimamente invasiva. Presentación del caso: Paciente masculino de 6 años con sintomatología sugestiva de enfermedad vesicular, referido desde la atención secundaria por sospecha de quiste de colédoco. En el ultrasonido se detectaba una lesión ecolúcida adyacente a la vesícula biliar, sin dilatación de las vías biliares intrahepáticas. Se realizó colangiografía laparoscópica y se confirmó una dilatación quística aislada del conducto cístico, la cual se resecó por vía laparoscópica, igualmente. Conclusiones: La incidencia de la dilatación quística del conducto cístico es muy baja y se puede presentar en niños con sintomatología variable. El diagnóstico generalmente es tardío, puede sospecharse mediante la ecografía abdominal y confirmarse con la colangiografía laparoscópica, aun en ausencia de otros medios diagnósticos más modernos. Su reconocimiento y correcta clasificación permiten realizar el tratamiento quirúrgico definitivo exitosamente, de preferencia por vía laparoscópica(AU)

Introduction: Congenital cystic duct dilatation, or Todani classification type VI dilatation, is a rare variant of congenital bile duct dilatation. Objective: To explain the diagnostic and therapeutic methodology used in a pediatric case with this entity, and to highlight the advantage of its timely minimally invasive treatment. Case presentation: Six-year-old male patient with symptoms suggestive of gallbladder disease, referred from secondary care for suspicion of a common bile duct cyst. Ultrasound showed an echolucent lesion adjacent to the gallbladder, without dilatation of the intrahepatic bile ducts. Laparoscopic cholangiography was performed and confirmed an isolated cystic dilatation of the cystic duct, which was resected laparoscopically, likewise. Conclusions: The incidence of cystic dilatation of the cystic duct is very low and may present in children with variable symptomatology. Diagnosis is usually late, can be suspected by abdominal ultrasound and confirmed by laparoscopic cholangiography, even in the absence of other more modern diagnostic tools. Its recognition and correct classification permit a successful definitive surgical treatment, preferably laparoscopically(AU)

Cowper's Syringocele: A Literature Review.

Cowper's syringocele is a rare but underdiagnosed cystic dilatation of the main ducts of Cowper's gland. It is becoming more widely known in the adult population. Recent research proposes that syringoceles should be categorized according to the intraductal pressures causing ductal dilatation from mild to gross ultimately involving the gland itself. Although there may be some overlap in the clinical manifestations of different syringoceles, mildly dilated ducts are frequently asymptomatic. Moreover, moderate to gross duct dilatations can manifest as lower urinary tract symptoms (LUTS) or obstructive symptoms. A valid differential diagnosis is essential because these symptoms can be found in a wide range of severe illnesses. Syringocele can be diagnosed by ultrasonography in combination with voiding retrograde/antegrade cystourethrogram (VCUG), nevertheless, other procedures like cystourethroscopy, CT scan, and MRI scans can be helpful. Initially, conservative surveillance is advised, but if necessary, endoscopic marsupialization or surgical excision is the preferred treatment modality to address persistent problems.

Zinner Syndrome.

Zinner syndrome is a less common birth anomaly of the Wolffian duct consisting of unilateral kidney absence, ipsilateral ejaculatory duct obstruction, and seminal vesicle cyst. A failure of embryogenesis of the ureteric bud between the fourth and 13th week of gestation results in Zinner syndrome. Conservative treatment is recommended for asymptomatic patients, whereas invasive treatment is reserved for symptomatic patients and for those who have failed conservative treatment. In this case report, we describe the non-specific presentation of lower abdominal pain and dysuria, as well as episodes of hematuria and new-onset hypertension, in a male patient, who was otherwise deemed healthy, with no other previous medical or surgical history. An imaging study and laboratory investigations were performed, and the patient was detected to have left renal agenesis and hypointense/hyperintense cysts in the left seminal vesicle of the left kidney. The findings supported the diagnosis of Zinner syndrome. The patient did not present with any symptoms or findings that would suggest infertility at the time of the study. Zinner syndrome is a rare cause of painful micturition and hematuria in males and can be diagnosed using ultrasound (USS), computer tomography (CT), and magnetic resonance imaging (MRI) techniques. Zinner syndrome should be considered as a differential diagnosis in male patients with unilateral renal agenesis and cystic pelvic masses. Patients who are asymptomatic typically undergo conservative treatment and are followed up to prevent infertility. For patients with symptomatic cysts who fail to respond to conservative treatment or whose cysts are larger than 5 cm in diameter, surgical intervention is recommended (open or laparoscopic surgery and ejaculatory duct balloon dilatation).

[The new classifications of biliary tract diseases based on actual anatomy].

In order to facilitate the treatment strategies for biliary tract injury, hilar cholangiocarcinoma, bile duct tumor thrombus, cholangiocellular carcinoma and bile duct cystic dilatation, many classifications have been made, even more than 10 types for one disease. Each type is represented by numbers or English alphabet, which are not only confusing but also difficult to remember. The Academician Mengchao Wu divided the liver into five sections and four segments base on its anatomy, this classification is very direct and visual, thus had been using till now. In order to overcome those complicated problems, it is considered to develop a new classification based on actual anatomic location similar to that for liver cancer, which is easy to remember and to directly determine the treatment strategy. All kinds of classifications have their own characteristics and advantages and disadvantages. This practical classifications avoid the complexity and may be useful for clinicians.

The new classifications of biliary tract diseases based on actual anatomy / 中华外科杂志

In order to facilitate the treatment strategies for biliary tract injury, hilar cholangiocarcinoma, bile duct tumor thrombus, cholangiocellular carcinoma and bile duct cystic dilatation, many classifications have been made, even more than 10 types for one disease. Each type is represented by numbers or English alphabet, which are not only confusing but also difficult to remember. The Academician Mengchao Wu divided the liver into five sections and four segments base on its anatomy, this classification is very direct and visual, thus had been using till now. In order to overcome those complicated problems, it is considered to develop a new classification based on actual anatomic location similar to that for liver cancer, which is easy to remember and to directly determine the treatment strategy. All kinds of classifications have their own characteristics and advantages and disadvantages. This practical classifications avoid the complexity and may be useful for clinicians.

Cistomatose de glândula apócrina em onça-pintada (Panthera onca) / Apocrine cystomatosis in a jaguar (Panthera onca)

A cistomatose de glândula apócrina é uma condição rara e não-neoplásica, que tem sido relatada em cães e gatos, e caracterizada por vários grupos de glândulas sudoríparas dilatadas, cuja distribuição generalizada raramente é observada. Uma fêmea de onça-pintada melânica (Panthera onca), com idade estimada de 16 anos e mantida em cativeiro, apresentou aumento de volume abdominal. Ao exame físico foram observados vários nódulos de consistência macia e flutuante, não aderidos, com tamanho de 2 a 15cm de diâmetro, na região ventral abdominal. Outras massas de menor diâmetro (0,5-1,0cm) estavam presentes na região cervical e no dorso do animal. Estas massas foram removidas em um procedimento cirúrgico. Dentro deste cisto havia conteúdo líquido, inodoro e translúcido. Microscopicamente, observou-se entre a derme média e profunda a formação de múltiplos cistos de tamanho variável. Esses cistos eram revestidos por uma única camada de células predominantemente cubóides. Em alguns focos eram observadas raras projeções papiliformes de epitélio glandular. O citoplasma estava levemente eosinofílico e com raras imagens de decapitação do ápice. O diagnóstico de cistomatose de glândulas apócrinas foi realizado através dos achados macroscópicos e microscópicos.(AU)

Cystomatosis of the apocrine gland is a rare condition reported in dogs and cats. It is a non-neoplastic condition, characterized by several groups of dilated cystic sweat glands. Rarely a more widespread distribution can be observed. A captive female about 16 year-old melanic jaguar (Panthera onca) presented increased abdominal volume. Physical examination showed multiple floating nodular masses ranging from to 2 to 15cm in diameter located in the ventral abdomen. Other masses of smaller diameter (0.5-1.0cm) were present in the cervical region and in the back of the jaguar. These masses were surgically removed. Within this cyst had a odorless translucent content. Microscopically, between the mid and deep dermis there existed multiple cysts of different sizes, coated with a single layer of cuboidal cells. In some foci, there were rare papilliform projections of glandular epithelium. The cytoplasm was slightly eosinophilic with rare images of decapitation of the apex. The diagnostic of apocrine cystomatosis was performed through macroscopic and microscopic findings.(AU)

Planned hepatectomy for the "central type" intrahepatic and extrahepatic choledochal cysts / 中华肝胆外科杂志

Objective To retrospectively study the clinical value and the advantages in " planned hepatectomy" for the "central type" intrahepatic and extrahepatic choledochal cysts.Methods The clinical data of 7 patients with the "central type" of intrahepatic and extrahepatic choledochal cysts which were treated with "planned hepatectomy" from January 2014 through April 2017 at the Department of Biliary Tract Surgery of the Eastern Hepatobiliary Surgery Hospital,Second Military Medical University were retrospectively analyzed.Results All the patients completed radical resection of the intrahepatic and extrahepatic choledochal cysts in accordance with the " planned hepatectomy".The operations included 6 patients who were treated with percutaneous transhepatic cholangial drainage (PTCD) and 5 patients with portal vein embolization (PVE) prior to the surgical excision.Combined right liver resection was performed in 6 patients,and combined left liver resection in one patient.All the 7 patients had a history of chronic cholangitis.Liver volume tests demonstrated that the hemiliver volume to be removed (the embolized hemiliver) significantly decreased after PVE,whereas the hemilivers to be persevered were remarkably enlarged.No complication associated with PTCD and PVE occurred.The mean postoperative hospitalization was 12 days.Liver function tests suggested all the patients recovered well.No postoperative complication of bleeding,infection or liver function failure was observed,except in one patient who experienced pleural and abdominal effusion.Conclusions Combined subtotal hepatectomy may increase the risk of complications associated with the "central type" intrahepatic and extrahepatic choledochal cysts.The surgical strategy in planned hepatectomy can be used effectively to treat the "central type" of intrahepatic and extrahepatic choledochal cysts,with improved surgical safety,decrease in incidences of postoperative liver function failure and residual choledochal cysts.

Pancreaticobiliary maljunction and choledochal cysts: from embryogenesis to therapeutics aspects.

Pancreaticobiliary maljunction (PBM) and choledochal cysts (CC) are rare and little-known diseases. Several definitions have been proposed for the PBM, but the most widely accepted is an excessive length of the common pancreaticobiliary duct due to the abnormal convergence of the pancreatic and biliary ducts out of the duodenal wall. This anomaly, thought to develop during embryogenesis, is associated with a loss of regulation of the Oddi's sphincter leading to a pancreaticobiliary or biliopancreatic backflow. This reflux could be responsible, or associated with cystic dilatation of the bile ducts and biliary tract cancers, to various biliary or pancreatic events such as cholangitis or pancreatitis. For the diagnosis of PBM, magnetic resonance cholangiopancreatography has now become the gold standard as a noninvasive imaging tool. However, the main risk of PBM is the development of bile duct cancer, most often on a distended area. PBM without CC increase the occurrence of gallbladder cancer and require a preventive cholecystectomy. Surgical treatment of PBM with concomitant CC is more complex and depends on localization of the dilatation(s) as reported in the Todani's classification. This review describes the pathogenesis, embryogenesis, clinical features, investigation and management of PBM and CC.

Gran galactocele / Big Galactocele

El galactocele es la dilatación quística de un conducto durante la lactancia. Se considera la lesión mamaria más común encontrada durante esta etapa, aunque ocurre más frecuentemente después del cese de esta cuando la leche es retenida y comienza a acumularse en la mama. Se reporta el caso de un gran galactocele presentado durante la lactancia en una paciente angolana de 28 años de edad, cuya resolución se logró con tratamiento quirúrgico. El objetivo del artículo es presentar, con fines docentes, algunos aspectos de esta lesión benigna frecuente, pero que no alcanza generalmente la magnitud y dimensiones del caso presentado.

The galactocele is cystic dilatation of a duct during lactation. It is considered the most common breast lesion found during this stage; although it occurs most frequently after its cessation is when milk is retained and accumulates in the breast. A case of a large galactocele during lactation in an Angolan patient aged 28 is reported in this paper. Her solution was achieved with surgical treatment. The aim of this paper is to present, for teaching purposes, some aspects of this common benign lesion, but the size and dimensions do not usually reach thoswe of the case presented here.

Dilatação cística do úraco e uroperitônio em touros: relato de cinco casos / Cystic dilatation of the urachus and uroperitoneum in bulls: report of five cases

Descrevem-se os aspectos clínicos da dilatação cística do úraco e uroperitônio em cinco touros. Os animais apresentaram, em datas distintas, distensão abdominal e diminuição da ingestão de alimentos e água, até culminar com inapetência, cerca de duas semanas após o aparecimento dos primeiros sintomas. Ocorreu distensão abdominal bilateral progressiva, que, no início do processo, era discreta e restrita ao quadrante inferior do abdômen; com cerca de duas semanas de evolução, o abdômen assumiu forma arredondada semelhante à pera. Observou-se bruxismo, atonia ruminal e desidratação. A abdominocentese revelou a presença de líquido amarelado com concentração de ureia superior a 200mg/dL. A concentração de ureia no soro sanguíneo variou de 220 a 280mg/dL e a creatinina de 65 a 82mg/dL. A ligadura do divertículo do úraco próximo ao vértex da bexiga foi eficaz nos quatro touros operados.

The clinical findings and outcomes in five bulls with a perforation or rupture of the urachal diverticulum are described. All the bulls had a dilated round or pear-shaped abdomen, bruxism, ruminal atony, and dehidration. In all the bulls, abdominocentesis yielded a stream fluid and the serum concentrations of urea and creatinine were 220 to 280mg/dL and 65 to 82mg/dL, respectively. Peritoneal fluid concentration of urea was higher than 200mg/dL. In fours bulls, urachal diverticulums were closed next to the cranial pole of the bladders. After the surgery, the recovery was effective.

A case of infantile nephrotic syndrome / 영남의대학술지

We have experienced a case of infantile nephritic syndrome confirmed by renal biopsy in a 13-month-old female patient who showed and develop mental retardation and persistent proteinuria. She revealed mild eyelid edema, joint laxity, delayed speech development and adrenal cortical calcification on the radiologic study. Renal biopsy showed microcystic tubular change, micro-glomeruli and marked mesangial proliferation.