Postnatal Surgical Intervention Research Articles

2- to 20-year myelomeningocele follow-up outcomes from a referral center in Southern Iran: the Shiraz experience

BackgroundThe current convention for treatment of children with myelomeningocele (MMC) is timely surgical intervention combined with long-term follow-up by a multidisciplinary specialized team. This study aims to investigate the outcomes of MMC patients treated at Namazi Hospital.MethodsAll children presenting to Namazi Hospital with myelomeningocele between May 2001 and August 2020 were eligible for this study. For those with a documented telephone number, follow-up phone surveys with the patient’s caregivers, on top of the review of the medical documents were carried out to assess mortality, morbidities, and the functional outcome of the care provided to them.ResultsA total of 125 patients were studied (62 females). All of the patients were followed up for a mean duration of 6.28 years (range 1–23 years). The majority were located in the lumbosacral area. All of the patients underwent postnatal surgical intervention for MMC in Namazi Hospital. Mean age at surgery was 9.51 days. There were statistically significant differences between urinary and bowel incontinence and presence of scoliosis, MMT grading of the lower limbs, school attendance, number of readmissions, and requirement of laminectomy at the initial surgical intervention.ConclusionsThis study is the first to characterize the long-term outcomes of MMC patients in Iran. This study illustrates that there is a great need for improved access to and coordination of care in antenatal, perioperative, and long-term stages to improve morbidity and mortality.

Imaging and outcomes of fetal adrenal hemorrhage: A systematic review.

To describe prenatal and postnatal imaging findings of fetal adrenal hemorrhage (FAH) and its associated perinatal outcomes, including frequency of postnatal surgical intervention. A systematic literature review of seven electronic databases was conducted from inception until January 2022, with 2008 articles identified reporting prenatally identified fetal adrenal masses. Studies with confirmed FAH diagnosis were included. Quality and risk assessment were evaluated. Thirty-five studies, including 102 FAH cases, were analyzed. FAH was commonly described as cystic (28/90, 31%), anechoic (25/90, 28%), or mixed echogenic (14/90, 16%) on ultrasound. Outcome data were available for 65 cases (64%) of FAH: 9% (6/65) resolved prenatally, 35% (23/65) resolved postnatally, 34% (22/65) regressed in size after birth, and 22% (14/65) persisted postnatally. Overall, 25% (16/65) of cases underwent postnatal surgical intervention. Neuroblastoma was suspected in all 16 surgical cases. Only one case (1/16, 6%) confirmed a cystic hematoma with microscopic islets of neuroblastoma in situ on pathology. Prenatal diagnosis of FAH is challenging due to the significant heterogeneity of ultrasound findings. Final pathology did not support the need for surgical intervention. Persistent postnatal FAH warrants shared decision making for further management based on the clinical presentation.

Perinatal Outcomes in Hypoplastic Left Heart Syndrome

Aim: To review our experience in fetuses with prenatally diagnosed hypoplastic left heart syndrome (HLHS).
 Materials and Method: Retrospective study of fetuses prenatally diagnosed with HLHS between 2020 and 2022 in a tertiary referral center.
 Results: 29 fetuses with HLHS were identified. 13 of all 29 cases (44.8%) had associated cardiac abnormalities and 8 cases (27.5%) had coexisting extracardiac abnormalities. Only a small minority of cases underwent invasive diagnostic testing (20%), and all of which had a normal karyotype. Most cases were delivered at term (58.6%), median gestational week at delivery was 37 (range, 34-39), and the mean standard deviation (SD) birthweight was 3099±455 grams. In addition, HLHS was more common in male than in female fetuses (69% vs 31%). Termination of pregnancy (TOP) was selected in 4 (13.7%) of the diagnosed 29 cases and performed before at ≤ 26 weeks of pregnancy. There were 3 intrauterine fetal demise (IUFD) at 23,29, and 34 weeks of gestation. Among the 29 cases of HLHS, 22 (75.8%) were live born. 3 of 22 live born infants were managed by medical procedures as they were not appropriate surgical candidates and later died at 22nd, 23rd and 25th of postnatal day. Surgery was performed in 15 cases and only one of them is are still alive. Mortality rate in the first three months of neonatal period was 96.5%. 
 
 Conclusion: Fetal echocardiography allows an accurate diagnosis of HLHS, which is made even
 in the late first trimester. Despite early prenatal detection and postnatal surgical interventions, the results from our center in last two years reflect poor outcomes for HLHS.

Postnatal surgical treatment and complications following intrauterine vesicoamniotic shunting with the SOMATEX® intrauterine shunt. A single center experience

Intrauterine vesicoamniotic shunting (VAS) using a Somatex® shunt was shown to significantly affect survival of male fetuses with megacystis in suspected lower urinary tract obstruction (LUTO) [Figure1]. Data on postnatal surgical management and complications are largely lacking. To describe the postnatal management of patients with prenatal VAS for megacystitis in suspected severe LUTO. All male newborns with previous intrauterine VAS using a Somatex® shunt treated in our institution were retrospectively analyzed. We evaluated the spectrum of urethral pathologies and postnatal surgical management, especially focusing on shunt removal. Between 2016 and 2022, 17 patients (all male) were treated postnatally in our institution after VAS for suspected severe LUTO. Five fetuses with dislocated shunts underwent re-implantation in utero. Overall, premature birth before the 38th week of gestation was observed in eight patients (8/17). Seven shunts could be removed without further anesthesia as a bedside procedure. Ten patients required surgical shunt removal under general anesthesia due to migration (59%). Laparoscopic shunt extraction was performed in 8/10 cases. Most frequently, dislocated shunts were located incorporated in the detrusor in eight cases and the removal required a bladder suture in 2/8 patients. In one case, the shunt was removed from the abdominal wall and in one case from the intestine wall [Figure2]. Posterior urethral valves were found in 8/17 patients, 6/17 patients showed a urethral atresia and one patient had urethral duplication. In two patients, we identified a high grade bilateral vesicoureteral reflux without LUTO. In our observation, more than half of the newborns with megacystis in suspected LUTO require a shunt removal surgery after early VAS using a Somatex® shunt. Urethral atresia may be found more frequently in these patients. These data should be taken into consideration for prenatal counselling of parents and planning of postnatal management.

Utility of novel echocardiographic measurements to improve prenatal diagnosis of coarctation of the aorta

Prenatal recognition of coarctation of the aorta (CoA) may improve neonatal survival and reduce morbidity. However, prenatal diagnosis of CoA remains challenging, with relatively high false-positive and false-negative rates. This study aimed to identify a novel formula based on fetal echocardiographic measures to predict prenatal identification of CoA. A retrospective comparison on the echocardiographic evaluation of 30 patients with suspected CoA between May 2016 and April 2021 was performed. The patients were divided into a postnatal surgical intervention group (n = 13) and a non-intervention group (n = 17). The measurements that showed significant differences were aortic isthmus diameter Z-score (p < 0.001), ductus arteriosus diameter/aortic isthmus diameter (p < 0.001), and distal aortic arch (DA) index (p < 0.001). In the receiver operating characteristic curves analysis, the DA index was the largest with an area under the curve of 0.941 and a cutoff value of 1.28, with a sensitivity of 85% and a specificity of 94%. Measurement of the DA index improved the diagnostic rate of fetal CoA and a DA index ≧ 1.28 indicated fetal CoA cases requiring surgical intervention.

Predictive value of fetal echocardiographic parameters in surgical strategy for Tetralogy of Fallot.

This study aimed to evaluate whether fetal echocardiographic parameters were predictive of the postnatal surgical treatment required for fetuses with Tetralogy of Fallot (TOF). The fetal echocardiographic and postnatal clinical data of all cases of prenatal TOF at Xinhua Hospital from 2016 to 2020 were reviewed. Patients were categorized based on the operation type, and cardiac parameters were compared between groups. Of the 37 fetuses assessed, the development of the pulmonary valve annulus (PVA) was significantly poorer in the transannular patch group. Patients with a prenatal PVA z-score (Schneider's method) ≥ -2.645, a PVA z-score (Lee's method) ≥ -2.805, a PVA/aortic valve annulus diameter ratio ≥.697, and a pulmonary annulus index ≥ .823 were more likely to undergo pulmonary valve-sparing surgery. There was a strong correlation between prenatal and postnatal PVA z-scores. The PVA growth potential was greater in the pulmonary valve-sparing surgery group. PVA-related parameters evaluated by fetal echocardiography can predict the type of surgical intervention required and are valuable in improving prenatal counseling in fetal cases of TOF.

Cardiopulmonary Vascular Odyssey and Haemodynamic Conundrum: A Case Report of Total Anomalous Pulmonary Venous Connection

Total anomalous pulmonary venous connection (TAPVC) is a cyanotic congenital heart disease where all the pulmonary veins drain directly or indirectly into the right atrium. Each of these types can be further classified into two types based on the pulmonary veins – obstructive and non-obstructive. This case report describes a rare case of a 2-month-old male infant with an unobstructed supra-cardiac TAPVC. The patient presented with tachypnoea, cyanosis and feeding difficulties, which prompted a thorough examination. Unobstructed supra-cardiac TAPVC was revealed by the diagnostic evaluation using two-dimensional echocardiography. Supra-cardiac TAPVC repair surgery was performed, following which post-operative evaluations indicated that the patient had satisfactory biventricular functionality. This case emphasises the relevance of early detection of TAPVC, thereby highlighting the need for meticulous and prompt postnatal care. The primary insight lies in the crucial role of prenatal diagnosis in tertiary care facilities, which allows timely planning of delivery, postnatal surgical interventions and a better prognosis of the disease. By underlining the significance of early intervention in the management of such rare congenital cardiac diseases such as TAPVC, this case report offers valuable insights into the scientific community.

Outcomes of sonographically-suspected fetal intra-abdominal cysts: Surgical intervention, conservative management and spontaneous regression.

The prenatal diagnosis of fetal intra-abdominal cysts is challenging. This study aimed to evaluate the diagnostic ability of prenatal ultrasound for fetal intra-abdominal cysts and to develop a predictive method for pre- and postnatal outcomes. We retrospectively reviewed fetuses with ultrasound-detected intra-abdominal cysts between January 2013 and January 2020. The maternal-fetal clinical characteristics and ultrasound parameters were integrated into a model of pre- or postnatal outcomes. The study enrolled 190 eligible fetuses, including 94 cases of spontaneous regression, 33 cases of conservative management and 63 cases of surgical intervention. For the 63 cases of surgical intervention, prenatal ultrasound was found to identify fetal intra-abdominal cysts with 80.00% sensitivity (95% CI: 67.03%-89.57%), 37.50% specificity (95% CI: 8.52%-75.51%), 89.80% positive predictive value (95% CI: 83.51%-93.86%), 21.43% negative predictive value (95% CI: 8.80%-43.53%) and 74.60% accuracy (95% CI: 62.06%-84.73%). The predictive model of prenatal spontaneous regression was as follows: y = -3.291 + 0.083 × gestational age + 1.252 × initial diameter, with an area under the curve (AUC) of 0.819 (95% CI: 0.739-0.899) and an optimal cut-off value of 0.74. The large cyst diameter before delivery was an independent predictor of postnatal surgical intervention (p < 0.001), with an AUC of 0.710 (95% CI: 0.625-0.794) and an optimal cut-off value of 3.35 cm. Although ultrasound has a limited ability in the accurate diagnosis of fetal abdominal cysts, a simple method of measuring the diameter can predict fetal outcomes and identify the cases that may require surgical intervention or spontaneous regression.

Predictors of postnatal surgical intervention for Antenatally detected Ureteropelvic Junction Obstruction

Predictors of postnatal surgical intervention for Antenatally detected Ureteropelvic Junction Obstruction

Impact of Fetal Echocardiography Comprehensiveness on Diagnostic Accuracy

Fetal echocardiography is a major diagnostic imaging modality for prenatal detection of critical congenital heart disease. Diagnostic accuracy is essential for appropriate planning of delivery and neonatal care. The relationship between study comprehensiveness and diagnostic error is not well understood. The aim of this study was to test the hypothesis that high fetal echocardiographic study comprehensiveness would be associated with low diagnostic error. Diagnostic errors were defined as discordant fetal and postnatal diagnoses and were further characterized by potential causes, contributors, and clinical significance. Fetal echocardiographic examinations performed at Lucile Packard Children's Hospital in which fetuses with critical congenital heart disease were anticipated to require postnatal surgical or catheter intervention in the first year of life were identified using the fetal cardiology program database. For this cohort, initial fetal echocardiographic images were reviewed and given a fetal echocardiography comprehensiveness score (FECS). Fetal diagnoses obtained from initial fetal echocardiographic images and reports were compared with postnatal diagnoses confirmed by transthoracic echocardiography and other imaging studies and/or surgery to determine diagnostic error. The relationship between FECS and diagnostic error was evaluated using multivariable logistic regression. Of the 304 initial fetal echocardiographic studies, diagnostic error (discrepant diagnosis, false negative, or false positive) occurred in 92 cases (30.3%). FECS was not associated with diagnostic error, but low FECS (≤80% complete) was associated with false negatives and procedural/conditional (P<.001) and technical (P=.005) contributors compared with high FECS (>80% complete). Cognitive factors made up the largest proportion of contributors to error. The comprehensiveness of fetal echocardiographic studies was not related to diagnostic error. The most common contributors to error were cognitive factors. Echocardiography laboratories can work to mitigate preventable cognitive error through quality improvement initiatives.

OP09.01: The prenatal ultrasound predictors for the postnatal surgical intervention in fetus with intra‐abdominal cysts

OP09.01: The prenatal ultrasound predictors for the postnatal surgical intervention in fetus with intra‐abdominal cysts

Ovarian cysts

Ovarian cysts

Fetal Non-Ovarian Abdominopelvic Cystic Lesions: A Single-Center Report

Introduction: There is a paucity of reports describing the clinical course and likely postnatal outcomes of prenatally identified simple cystic abdominopelvic lesions which are not associated with the ovary. Objective: The aim of this study was to describe the natural history and postnatal outcomes of prenatally discovered abdominopelvic cystic lesions seen at our center. Methods: This study is a retrospective review of all newborns with prenatally discovered non-ovarian simple cystic abdominal or pelvic lesions (September 2012–December 2018). Prenatal solid organ involvement, lesion size, and postnatal clinical outcomes are described. Results: Sixty-six patients with 68 cystic lesions were identified; 22 patients with 24 lesions met the defined study criteria and were included. Eleven (46%) resolved prenatally, while 5 (21%) resolved by 18 months of age. Of the 10 lesions associated with an organ, 4 (40%) resolved prenatally. Of the remaining 14 lesions not associated with a solid organ, 7 (50%) resolved prenatally. Seven lesions (29%) required postnatal surgical intervention. Larger maximum prenatal lesions tended toward postnatal surgical intervention (one-way ANOVA: p = 0.072). Conclusions: The majority of simple non-ovarian cystic abdominopelvic lesions at our center resolved in the perinatal period. Due to the low frequency of these lesions at fetal centers, a larger multicenter study based on a consistent monitoring protocol should be undertaken to better describe the resolution patterns of simple non-ovarian cystic lesions for improved prenatal counseling.

VP11.18: Can postnatal surgical intervention be predicted using the UTD classification system? A single centre experience

VP11.18: Can postnatal surgical intervention be predicted using the UTD classification system? A single centre experience

Innovative Molecular and Cellular Therapeutics in Cleft Palate Tissue Engineering.

Clefts of the lip and/or palate are the most prevalent orofacial birth defects occurring in about 1:700 live human births worldwide. Early postnatal surgical interventions are extensive and staged to bring about optimal growth and fusion of palatal shelves. Severe cleft defects pose a challenge to correct with surgery alone, resulting in complications and sequelae requiring life-long, multidisciplinary care. Advances made in materials science innovation, including scaffold-based delivery systems for precision tissue engineering, now offer new avenues for stimulating bone formation at the site of surgical correction for palatal clefts. In this study, we review the present scientific literature on key developmental events that can go awry in palate development and the common surgical practices and challenges faced in correcting cleft defects. How key osteoinductive pathways implicated in palatogenesis inform the design and optimization of constructs for cleft palate correction is discussed within the context of translation to humans. Finally, we highlight new osteogenic agents and innovative delivery systems with the potential to be adopted in engineering-based therapeutic approaches for the correction of palatal defects. Impact statement Tissue-engineered scaffolds supplemented with osteogenic growth factors have attractive, largely unexplored possibilities to modulate molecular signaling networks relevant to driving palatogenesis in the context of congenital anomalies (e.g., cleft palate). Constructs that address this need may obviate current use of autologous bone grafts, thereby avoiding donor-site morbidity and other regenerative challenges in patients afflicted with palatal clefts. Combinations of biomaterials and drug delivery of diverse regenerative cues and biologics are currently transforming strategies exploited by engineers, scientists, and clinicians for palatal cleft repair.

New surgical paradigm for open neural tube defects.

An open neural tube defect (ONTD) features an exposed, unclosed neural plate in the form of an expanded, flat, and frequently hefty neural placode. Traditional philosophy of ONTD repair aims at preserving function at any cost, which often means stuffing the entire thick and unwieldy but non-functional placode into a tight dural sac, increasing the likelihood of future tethering of the spinal cord. The same philosophy of attempting to save the whole perimetry of the placode also sometimes leads to inadvertent inclusion of parts of the squamous epithelial membrane surrounding the placode into the reconstructed product, only to form inclusion dermoid cyst causing further injury to the neural tissues. Lastly, defective closure of the caudal primary neural tube usually results in abolition of secondary and junctional neurulation, leaving a defective conus and sacral nerve roots, clinically presenting in most cases with neurogenic bladder and bowel dysfunction. Preserving this trapped but locally active sacral micturition center, isolated from suprasegmental inhibitory moderation, leads to a spastic, hyperactive, low compliance, and high-pressure bladder predisposing to upstream kidney damage, without benefits of normal bladder function. We report the post-natal surgical treatment of 8 newborn infants with ONTD, in which we resected the non-functional portion of the neural placode identified as such by direct spinal cord/placode and nerve root stimulation, as well as by transcortical evoked motor responses to check for suprasegmental corticospinal connectivity. Any part of the placode without local function or upstream connections was resected, and the small caudal spinal cord stump closed with pia-to-pia microsutures. The patients were followed for pre- and post-operative neuro-urological status and with serial magnetic resonance imaging (MRI) at 3weeks, 6months, and 2years post-repair. Follow-up period ranged from birth to 3years (mean of 24months). Of the 8 patients analyzed, 7 had a terminal and one a segmental ONTD. Pre-operative neurological level ranged from L4 to S2. Applying our new surgical paradigm, we found no neurological worsening post-operatively. All patients had a neurogenic bladder and bowel dysfunction but none had a high-pressure bladder on urodynamics studies. Early and late MRIs all showed a loose and capacious neural placode to dural sac relationship. None had an inclusion dermoid cyst. We propose a new paradigm for the surgical repair of open neural tube defects with intraoperative neuromonitoring and introduce a safe and reliable technique of placode debulking.

Fetal surgery and stem cell therapy for meningomyelocele.

To review the advance of maternal--fetal surgery, the research of stem cell transplantation and tissue engineering in prenatal management of fetal meningomyelocele (fMMC). Advance in the imaging study provides more accurate assessment of fMMC in utero. Prenatal maternal--fetal surgery in fMMC demonstrates favourable postnatal outcome. Minimally invasive fetal surgery minimizes uterine wall disruption. Endoscopic fetal surgery is performed via laparotomy-assisted or entirely percutaneous approach. The postnatal outcome for open and endoscopic fetal surgery shares no difference. Single layer closure during repair of fMMC is preferred to reduce postnatal surgical intervention. All maternal--fetal surgeries impose anesthetic and obstetric risk to pregnant woman. Ruptured of membrane and preterm delivery are common complications. Trans-amniotic stem cell therapy (TRASCET) showed potential tissue regeneration in animal models. Fetal tissue engineering with growth factors and dura substitutes with biosynthetic materials promote spinal cord regeneration. This will overcome the challenge of closure in large fMMC. Planning of the maternal--fetal surgery should adhere to ethical framework to minimize morbidity to both fetus and mother. Combination of endoscopic fetal surgery with TRASCET or tissue engineering will be a new vision to achieve to improve the outcome of prenatal intervention in fMMC.

Prenatal ultrasound diagnosis of fetal pelvic tumors. II. Fetal ovarian cysts – what does really matter? Literature review

Literature review of prenatal ultrasound and MRI-diagnosis of fetal ovarian cyst is presented. Cysts evolution, ultrasound features and related risk of complications and postnatal surgical intervention are reviewed. Authors analyzed data of publications in aspects of time of diagnosis, complications and outcomes of antenatal diagnosed ovarian cysts. Antenatal and postnatal management are discussed.

Congenital talipes equinovarus (clubfoot)

Congenital talipes equinovarus (clubfoot)

Experience in prenatal ultrasound and MRI diagnosis of 49 fetal ovarian cysts

Analysis of 49 cases of prenatally diagnosed ovarian cysts are presented. The outcomes of prenatally diagnosed ovarian cysts were studied on their antenatal ultrasound and MRI images. Outcomes analysis of fetal ovarian cysts depending on their ultrasound and MRI features are presented. Prenatal features, complications of pregnancy, cysts evolution, outcomes and postnatal surgical intervention on 49 cases of fetal ovarian cysts are presented. 1/3 of the ovarian cysts were visualized partly or completely in the abdomen, 1/3 — laterally from the bladder. Simple cysts were of less dimension compared to complex lesions. Increase in size is presumably of higher risk of complications. Spontaneous regression by the time of delivery was in 34.7%. 100% of lesions  2 cm disappeared, the rate of regression of simple and complex cysts was equal, the lowest rate of regression was seen in cysts  4 cm. The frequency of surgical treatment was in 26.5%.