[Application of Fetal Magnetic Resonance Imaging in Prognosis Assessment of Fetuses With Congenital Pulmonary Cystic Diseases]. / äº§å‰ç£å ±æŒ¯åœ¨å ˆå¤©æ€§è‚ºå›Šæ€§ç–¾ç— èƒŽå„¿é¢„åŽè¯„ä¼°ä¸­çš„åº”ç”¨ç ”ç©¶.

Objective: The aim of this study is to explore the practical value of prenatal magnetic resonance imaging (MRI) in the assessment of congenital cystic lung disease in fetuses, to evaluate the relative size of the lesion and the status of lung development, and to make an attempt at utilizing the strength of MRI in post-processing to obtain assessment indicators of the size of the lesion and the status of lung development, with which predictions can be made for the prognosis that these fetuses may face after birth. We retrospectively collected and analyzed the data of fetuses diagnosed with congenital cystic lung disease. Prenatal ultrasound examination of these fetuses led to the diagnosis that they were suspected of having congenital cystic lung disease and the diagnosis was confirmed by subsequent prenatal MRI. The fetuses were followed up to track their condition at birth (postnatal respiratory distress, mechanical ventilation, etc.), whether the fetuses underwent surgical treatment, and the recovery of the fetuses after surgical treatment. The recovery of the fetuses was followed up to explore the feasibility of prenatal MRI examination to assess fetal congenital pulmonary cystic disease, and to preliminarily explore the predictive value of prenatal MRI for the prognosis of fetuses with congenital pulmonary cystic disease. Methods: MRI fetal images were collected from pregnant women who attended the West China Second University Hospital of Sichuan University between May 2018 and March 2023 and who were diagnosed with fetal congenital pulmonary cystic disease by prenatal ultrasound and subsequent MRI. Fetal MRI images of congenital cystic lung disease were post-processed to obtain the fetal lung lesion volume, the fetal affected lung volume, the healthy lung volume, and the fetal head circumference measurements. The signal intensity of both lungs and livers, the lesion volume/the affected lung volume, the lesion volume/total lung volume, the cystic volume ratio (CVR), and the bilateral lung-liver signal intensity ratio were measured. The feasibility and value of MRI post-processing acquisition indexes for evaluating the prognosis of fetuses with congenital cystic lung disease were further analyzed by combining the follow-up results obtained 6 months after the birth of the fetus. Logistic regression models were used to quantify the differences in maternal age, gestational week at the time of MRI, CVR, and bilateral lung-to-liver signal intensity ratio, and to assess whether these metrics correlate with poor prognosis. Receiver operating characteristic (ROC) curves were used to assess the value of the parameters obtained by MRI calculations alone and in combination with multiple metrics for predicting poor prognosis after birth. Results: We collected a total of 67 cases of fetuses diagnosed with congenital cystic lung disease by fetal MRI between May 2018 and March 2023, and excluded 6 cases with no normal lung tissue in the affected lungs, 11 cases of fetal induction, and 3 cases of loss of pregnancy. In the end, 47 cases of fetuses with congenital cystic lung disease were included, of which 30 cases had a good prognosis and 17 cases had a poor prognosis. The difference in the difference between the signal intensity ratios of the affected and healthy sides of the lungs and livers of the fetuses in the good prognosis group and that in the poor prognosis group was statistically significant (P<0.05), and the signal intensity ratio of the healthy side of the lungs and livers was higher than the signal intensity ratio of the affected side of the lungs and livers. Further analysis showed that CVR (odds ratio [OR]=1.058, 95% confidence interval [CI]: 1.014-1.104), and the difference between the lung-to-liver signal intensity ratios of the affected and healthy sides (OR=0.814, 95% CI: 0.700-0.947) were correlated with poor prognosis of birth in fetuses with congenital cystic lung disease. In addition, ROC curve analysis showed that the combined application of lesion volume/affected lung volume and the observed difference in the signal intensity ratio between the affected and healthy lungs and liver predicted the prognosis of children with congenital cystic lung disease more accurately than the single-parameter judgment did, with the area under the curve being 0.988, and the cut-off value being 0.33, which corresponded to a sensitivity of 100%, a specificity of 93.3%, and a 95% CI of 0.966-1.000. Conclusions: Based on the MRI of fetuses with congenital cystic lung disease, we obtained information on lesion volume, lesion volume/affected lung volume, lesion volume/total lung volume, CVR, and bilateral lung-to-liver signal intensity ratio difference, all of which showing some clinical value in predicting the poor prognosis in fetuses with congenital cystic lung disease. Furthermore, among the combined indexes, the lesion volume/affected lung volume and bilateral lung-to-liver signal intensity ratio difference are more effective predictors for the poor prognosis of fetuses with congenital cystic lung disease, and show better efficacy in predicting the poor prognosis of fetuses with congenital cystic lung disease. This provides a new and effective predictive method for further assessment of pulmonary lung development in fetuses with congenital cystic lung disease, and helps improve the assessment and prediction of the prognosis of fetuses with congenital cystic lung disease.

Coloboma Accompanying Microphthalmos With Orbital Cyst in a Mother and Child.

Microphtalmos with orbital cyst is a rare congenital abnormality of the eye and orbit that is caused by incomplete closure of the embryonic fissure. The cysts project through in a coloboma of the affected eye. It may be sporadic or genetic. Herein, the authors present a 32-year-old mother with unilateral and her 4-month-old daughter with bilateral microphtalmos and accompanying orbital cyst.

Periventricular pseudocysts of noninfectious origin: Prenatal associated findings and prognostic factors.

OBJECTIVE: The purpose of this study was to establish prognostic factors in fetuses diagnosed with periventricular pseudocysts (PVPCs) without known congenital infection, between 28 and 37 weeks of gestation. METHODS: This retrospective study included cases of fetal PVPC from 2008 to 2018. PVPCs were classified according to location, number, extension, morphology, and size. Additional findings, MRI and genetic studies were recorded. Pregnancy outcome, postnatal, or postmortem results were obtained. Images from patients with normal (Group 1) and abnormal postnatal development (Group 2) were compared for analysis of factors predictive of outcome. RESULTS: One-hundred and fifteen pseudocysts were observed in 59 patients. In 34 fetuses (57%), the PVPC was an isolated finding. Thirty-nine patients delivered live newborns, 27% opted for termination of pregnancy, and 4 patients were lost to follow-up. Eighty-four percent of the liveborns had normal development. When assessing for the influence of pseudocyst characteristics, a wide CSP, or large head circumference, neither of these affected the outcome. The presence of additional anomalies was the only positive predictor for abnormal development regradless of specific PVPC characteristics (P = .002). CONCLUSIONS: In fetuses with PVPCs, the presence of additional anomalies was the only predictor for adverse postnatal outcome. No association between cystic characteristics and adverse outcome was observed.

Congenital canaliculops with punctal agenesis: is there a possibility to establish patency?

Canaliculops is a non-inflammatory dilatation of the canaliculus which can be secondary to trauma, punctal agenesis, herpetic infections or chronic drug use. This condition can affect either canaliculi and has shown female preponderance. Treatment described so far has consisted of cyst marsupialization with no literature on the post-operative epiphora in these cases. We describe two cases of canaliculops with punctal agenesis where we have tried to establish lacrimal patency.

Tricuspid regurgitation due to blood cyst.

Cardiac blood cysts are confirmed by autopsies in stillbirths but rarely detected in adults. Although cardiac blood cysts are common in newborns, they disappear within a few months after birth. These cysts are blood-filled diverticula surrounded by endothelium. Their possible etiology is invaginations of atrial endothelium into the stroma of atrioventricular valves. Most blood cysts originate from the left side of the heart and are located on the atrioventricular valves. Complication is followed in very little cases, such as embolic stroke, valvular dysfunction, and ventricular outflow system obstruction. The blood cysts leading to complications should be removed surgically.

Congenital Dacryocystocele: A Major Review.

PURPOSE: To provide a systematic review of the literature on congenital dacryocystoceles (CDCs) and summarize their presentations, investigations, management, and outcomes. METHODS: The authors performed a PubMed search of all articles published in English on CDCs. Data captured include demographics, clinical presentations, investigations, management modalities, complications, and outcomes. Fourteen major series (10 or more than 10 cases) and 89 isolated case reports/series on CDCs with a collective patient pool of 1,063 were studied in detail. Specific emphasis was laid on addressing the controversial issues including initial conservative versus surgical management and the role of endoscopic evaluation. RESULTS: Numerous terminologies have been used to describe CDC. Congenital dacryocystoceles are rare variants of congenital nasolacrimal duct obstructions and comprise of 0.1% to 0.3% of all such cases. There is a female predilection (64.2%, 683/1,063) and the mean age at presentation is at 7 days of birth. Initial conservative treatment can be a viable option in the absence of an acute dacryocystitis or a respiratory distress. Endoscopy-assisted probing appears to have better outcomes as compared with the in-office probing. Congenital dacryocystoceles with acute dacryocystitis are preferably managed with intravenous antibiotics and an early probing under endoscopy guidance to avoid missing intranasal cysts. Marsupialization is the preferred technique in the management of intranasal cysts. Silicone intubation was rarely used and has no definitive indications. Dacryocystorhinostomy is very rarely needed in the management of CDC. CONCLUSIONS: Congenital dacryocystocele is a commonly accepted term and its use should be advocated to enhance uniformity in reporting. Endoscopic evaluation of CDC is useful in the diagnosis and treatment of associated intranasal cysts and enhances the rates of successful outcomes.

Multiple subependymal pseudocysts in neonates play a role in later attention deficit hyperactivity and autistic spectrum disorder.

BACKGROUND/PURPOSE: To assess the long-term neurodevelopmental outcome of normal-term neonates who were accidentally found to exhibit subependymal pseudocysts (SEPCs), frontal horn cysts, or choroid plexus cysts through cranial ultrasound (CUS) examination in a neonatal health examination. METHODS: In total, 5569 neonates received CUS examination as an item in a health examination during the first week of birth between 2002 and 2012. Among them, 5147 infants fulfilled the inclusion criteria. The participants were aged between 5 and 15 years at the time when the data were collected. We retrospectively collected these data and interpreted their statistical significance by using one-way analysis of variance, Chi-square test with Yate's correction and odds ratios. RESULTS: The presence of SEPCs was significantly correlated with developmental delay and developmental disability, particularly with attention deficit hyperactivity disorder (ADHD) and autistic spectrum disorder (ASD). The risk of ADHD or ASD was significantly higher in participants with multiple SEPCs, among whom the odds ratios for ADHD and ASD were 6.50 (95% confidence interval [CI] = 2.27-18.64) and 28.54 (95% CI = 5.98-136.36), respectively, higher than those for the total study population. CONCLUSION: Our data revealed multiple SEPCs in normal-term neonates as a risk factor for neurobehavioral outcome, particularly in ADHD and ASD. Simultaneously, the utility of CUS examination as a health examination item for neonates was confirmed.

Multimodality characterization of a noncommunicating congenital duodenal duplication cyst causing pyloric outflow obstruction in a young dog.

A 10-month-old German Shepherd Dog presented for evaluation of intermittent vomiting. Abdominal radiographs revealed a marked right cranial mass effect. Initial differentials included abscess/cyst or less likely neoplasia from undetermined origin. On abdominal ultrasound the mass appeared cystic and thin walled. Computed tomography revealed a large cystic lesion originating from the pyloroduodenal junction causing pyloric outflow obstruction. A noncommunicating duodenal duplication cyst was found on exploratory laparotomy and further confirmed with histopathology and immunohistochemistry. Enteric duplication cyst should be considered as a differential in young dogs with gastrointestinal signs and a cystic abdominal mass detected with different imaging modalities.

A Triplication of 6q24 and Meconium Pseudocyst: A Case Report.

With the rise in genetic screening both pre- and postnatally, new variances in genes are being recognized. Some are of unknown significance, while other known genetic expressions have obvious phenotypical expressions. Transient neonatal diabetes mellitus is a result of the duplication of chromosome 6q24, but little is known about the phenotypic expression of a triplication of chromosome 6q24. This case study presents an infant with a postnatally diagnosed triplication of chromosome 6q24, meconium pseudocyst, and multiple congenital anomalies with unknown genetic significance.

Uterine Paramesonephric Cysts in Sprague-Dawley Rats from National Toxicology Program Studies.

Congenital uterine wall cysts arising from paramesonephric (Müllerian) and mesonephric (Wolffian) ducts are typically incidental findings in most species. We used immunohistochemistry to characterize and determine the origin of uterine cysts in Sprague-Dawley (SD) rats from multigeneration studies conducted by the National Toxicology Program. Subserosal uterine cysts were observed in 20 of the 2,400 SD rats evaluated in five studies, and 10 cysts were characterized for this study. Single cysts were unilocular, fluid-filled, and occurred throughout the uterus. Microscopically, all cysts had a well-developed smooth muscle wall, lined by flattened to cuboidal, sometimes ciliated, epithelium that stained intensely positive for cytokeratin 18 and paired box protein 8 (PAX8). Most cyst epithelia displayed weak to moderate positivity for progesterone receptor (PR) and/or estrogen receptor α (ER-α), as well as were negative for GATA binding protein 3 (GATA3). Cyst lumens contained basophilic flocculent material. The cysts appeared to be developmental anomalies arising from paramesonephric tissue based on positive PAX8 and ER-α and/or PR staining. Additionally, 70% of the cysts lacked GATA3 expression. Taken together, the subserosal uterine cysts observed in adult rats in these studies most likely arose from the paramesonephric duct.

What are the prevalence, characteristics and significance of fetal lateral neck cysts detected in an early anatomical scan?

PURPOSE: This study evaluated the association of fetal lateral neck cysts (FLNC) with adverse pregnancy outcomes, in relation to specific sonographic characteristics and co-existing findings. METHODS: Pregnancies in which FLNC were detected by a single examiner in early anatomical scans (14-16 weeks) were included. Data regarding the pregnancy and its outcome were retrieved from telephone-based questionnaires, patient charts and from the examiner's reports. RESULTS: 654 cases of FLNC were detected among 9446 early anatomical scans (6.9%). Complete data regarding 219 pregnancies were available. FLNC were significantly more prevalent in males (65.2%). The prevalence of heart malformations was 3.2% [all were non-isolated cases or with abnormal nuchal translucency (NT) and/or nuchal fold (NF)]. Amniocentesis performed in 165 pregnancies was abnormal in 1.2%. Among 206 children born from this cohort, adverse medical outcomes were reported in 5.3%. The likelihood of adverse pregnancy outcomes was significantly higher in non-isolated cases and in cases with abnormal NT or NF. Sonographic characteristics such as cyst size and bilateral findings were not linked to adverse pregnancy outcomes. CONCLUSION: Isolated FLNC are benign findings which do not require additional work up. FLNC with additional sonographic abnormalities are associated with a significantly increased risk for adverse pregnancy outcomes.

Ruptured Thoracic and Abdominal Gastrointestinal Duplication Cysts Presenting With Failure to Thrive.

Gastrointestinal duplication cysts are rare congenital malformations, with esophageal and gastric duplication cysts being among the rarest. We report an 8-week-old female who presented to the emergency department with failure to thrive and was subsequently found to have multiple gastric and esophageal duplication cysts that had ruptured intrathoracically and intra-abdominally. We describe the diagnosis and management of this patient who underwent successful resection of 4 gastrointestinal duplication cysts. This report emphasizes the unexpected, and sometimes relatively benign, presentations of gastrointestinal duplication cysts. To our knowledge, this is the first reported occurrence of multiple duplication cysts that independently ruptured thoracically and abdominally.

Post Mortem Diagnosis of Blake's Pouch Cyst: A Presentation of Distended Cyst at Necropsy.

BACKGROUND: Blake's pouch cyst forms from non-permeabilization of Blake's pouch. It is difficult to visualize at necropsy as the cyst ruptures easily into the 4th ventricle during dissection. CASE REPORT: Based upon prenatal imaging, delicate dissection allowed post-mortem confirmation of the Blake's pouch cyst. CONCLUSIONS: This highlights the importance of utilizing premortem imaging to help guide the postmortem dissection and documentation of a posterior fossa cyst.

[Congenital seminal vesicle cysts in children].

The article reports two cases of seminal vesicle cysts with ipsilateral renal agenesis in children. The congenital nature of this condition, characteristic features of the clinical manifestation of seminal vesicle cysts makes it difficult to recognize this rare genitourinary disease. Using current methods of diagnosis and surgical treatment result in successful outcomes.

Minimally Invasive Approach for Treatment of Seminal Vesicle Cyst Associated with Ipsilateral Renal Agenesis.

OBJECTIVES: We reviewed the clinical presentation and approach for the treatment of symptomatic seminal vesicle cyst associated with ipsilateral renal agenesis while presenting our experience with a series of 5 patients treated with minimally invasive surgery for Zinner syndrome. MATERIALS AND METHODS: Between the years 2008 and 2016, we operated on 5 patients who presented with symptomatic seminal vesicle cyst and ipsilateral renal agenesis. Patients' charts and medical records were reviewed and compared with past published minimally invasive series. RESULTS: Four patients were treated in a laparoscopic approach and one was treated with robotic-assisted seminal vesicle cyst excision. In all cases, the cystic complex was drained and excised with marsupialization of the remaining cyst walls to prevent cystic recurrence. The mean operating time was 3:47 h and mean hospitalization time was 7 days (4-14). The mean follow-up period was 3.2 years (range 1.7-4.8 years). All patients reported a resolution of symptoms during postoperative follow-up. CONCLUSIONS: Seminal vesicle cyst with ipsilateral renal agenesis should be suspected in young male patients presenting with pelvic cystic masses. Treatment is reserved for symptomatic patients and the preferred approach is minimally invasive surgery. This approach is feasible and effective while providing advantages both for the patient and the surgeon.

Oral foregut duplication cysts: A rare and fascinating congenital lesion. Case report and review of the literature.

Oral foregut duplication cysts are extremely rare lesions with approximately 57 cases reported. They are congenital cysts, located in the anterior or ventral tongue, and occur predominantly in males. They are lined by one or more types of epithelia which is limited to gastric, intestinal or respiratory epithelium. The differential diagnosis includes lymphangioma, hemangioma, ranula, epidermoid cyst, teratoma and less likely a malignant process.