Katika Nawapun

Establishing Prenatal Surgery for Myelomeningocele in Asia: The Singapore Consensus

Objectives: The Management of Myelomeningocele Study (MOMS Trial) has inspired many fetal therapy centers (FTCs) to offer open fetal surgery for myelomeningocele (MMC). This is an initial effort to create a candidate model that can be applicable to many parts of Asia. Material and Methods: A limited selection of specialists from 4 established FTCs in Thailand, Hong Kong, India, and Singapore met for a round table discussion. Experts from Childrens Hospital of Philadelphia (CHOP) involved in the Trial moderated the session. The practice suggestions in this statement were from a targeted literature review and expert opinion. Results: A high prevalence of MMC in Asia supports an effort to adopt the procedure, but only in established FTCs with good maternal and neonatal ancillary supports. The falling incidence of MMCs may affect case volume and maintenance of skill. A regional approach was recommended. Fetal benefits have to be weighed against maternal risks, with a consideration of recent outcome data from the endoscopic approach. Responsible FTCs need standardized diagnosis and management, with their long-term outcome data available for an audit. Conclusions: It is envisaged that the information presented by this multidisciplinary team would be useful for FTCs in Asia or elsewhere that plan to establish more advanced fetal care in the future.

Three-dimensional ultrasound for prenatal assessment of conjoined twins: additional advantages?

Abstract Conjoined twins are a rare, but serious, complication of monozygotic twins. Early prenatal diagnosis of conjoined twins is increasingly made with transvaginal ultrasound and color Doppler studies. Most prenatally diagnosed conjoined twins are terminated due to the high perinatal mortality, but advancement in pediatric surgery has allowed for successful postnatal separation in a small number of cases, and some parents may consider this option over termination of pregnancy. It is important to get a detailed prenatal ultrasound for the site and extent of fusion for an accurate categorization. Three-dimensional ultrasound (3DUS) provides images that can facilitate counselling for the parents. Additional information that impacts on diagnosis, prognostication, and perinatal management of conjoined twins could be obtained from selective use of 3DUS, particularly those with atypical fetal union. Most of the proposed additional benefits of 3DUS are based on case reports. Magnetic resonance imaging (MRI) has been increasingly used for the purpose of identifying intricate organ sharing. Because of the rarity of this condition, and the heterogeneity of fetal fusion, added benefits of either 3DUS or MRI for prenatal diagnosis and perinatal management of conjoined twins have not been demonstrated by well-conducted clinical trials. This article aims to review clinical application of various 3DUS display modes in prenatal assessment of conjoined twins, focusing on their potential additional benefits, risks and misuses. 3DUS may help detecting additional findings that are not possible with 2DUS, but, it has not been scientifically shown to improve the survival rate of the twins or reduce maternal morbidity.

Current Strategy of Fetal Therapy I: Principles of In-utero Treatment, Pharmacologic Intervention, Stem Cell Transplantation and Gene Therapy

Fetal conditions with high morbidity are amenable for prenatal intervention. It is important that the selective and investigative nature of most procedures needs to be clarified with the family during counseling session. Fetal therapy is fostered by accurate prenatal diagnosis with advanced fetal imaging, and molecular genetics technology. The treatments can be categorized into medical treatment, stem cell transplantation and gene therapy, minimally invasive intervention, endoscopic surgery, and open hysterotomy approach. Scientific validation of their genuine benefits has been a subject of ongoing researches. Prenatal administrations of pharmaceutical agents, for prophylactic or therapeutic purposes, have been broadly adopted. Transplacental administration of betamethasone to enhance the function of pneumocytes type II in premature fetus has been widely practiced for decades, and it might be the most common ‘fetal therapy’ being performed. However, the optimal dosage and interval of prenatal steroids administration was validated only recently. More invasive route of fetal administration, such as transamniotic, direct intramuscular, and intravenous injection, may be required for other pharmacologic agents. In this article, the authors selected to review common fetal conditions whose proposed prenatal pharmacologic treatments have undergone scientific validations. In-utero stem cell transplantation and gene therapy remain highly experimental. Informed choice and clinical experiment need to be balanced when prenatal treatment is offered.

Undiagnosed Uterine Sarcomas Identified During Surgery for Presumed Leiomyoma at a National Tertiary Hospital in Thailand: A 10-Year Review

Objectives The aim of this study was to evaluate the rate, clinical characteristics, and survival outcomes of an undiagnosed uterine malignancy in patients who underwent surgical treatment for presumed leiomyomas. Methods Medical records of patients who underwent surgical treatment for presumed leiomyomas, from January 2004 to September 2013, were retrospectively reviewed, and the data were followed until September 2016. Demographic data, tumor characteristics, oncologic treatment, and response rate were analyzed by descriptive statistics. Kaplan-Meier method was used for survival analysis. This study includes follow-up data through December 31, 2016. Results A total of 11,258 medical records of presumed leiomyoma patients undergoing hysterectomy during the studied period were reviewed. Pathology of uterine sarcoma was found in 22 patients (0.2%), all of whom were included. Nineteen patients had leiomyosarcoma, and 3 had endometrial stromal sarcoma. Mean age of patients was 48.3 ± 6.9 years. All patients underwent total abdominal hysterectomy, with 20 patients undergoing concurrent bilateral salpingo-oophorectomy. Uterine sarcoma was classified as stage IB in 21 patients and stage IIIC in 1 patient. Fifteen patients were prescribed the following adjuvant treatment: chemotherapy in 13 patients and megestrol acetate in 2 patients. Thirteen patients had recurrent disease, and 3 patients died of their disease. The mean progression-free survival was 50.1 ± 41.3 months, and overall survival was 59.3 ± 43.0 months. Conclusions One in 512 patients who underwent hysterectomy because of presumed uterine leiomyomas had inadvertent uterine sarcomas. Even with adjuvant therapy, treatment outcome was rather poor, with almost 60% recurrence rate and median progression-free survival and overall survival of less than 5 years.

Advanced ultrasound for prenatal interventions

Ultrasound is an integral part of prenatal interventions. Doppler studies and 3-dimensional ultrasound (3DUS) are frequently used to determine whether fetal surgery is required. The operator’s experience remains crucial for reducing procedure-related morbidity. Real-time 3DUS (or 4DUS) can simultaneously display the needle tip in three orthogonal planes, providing reassurance that no fetal parts are in the path. In experienced hands, 4DUS guidance may not be more effective than B-mode, but its value for less-experienced operators remains to be determined. Recent developments in needle, shunt, and video endoscopic technologies may compliment the use of image-guided in utero procedures. Future developments of higher-dimensional transducers and image software may improve the utility of ultrasound for invasive obstetric interventions.

Time and temperature dependence of radiofrequency ablation in the human placenta

The objective of the study is to compare radiofrequency (RF) effects on fresh placentae with varying levels of sustained time (Ts) and degrees of target temperature (°t).

Model Surgical Training: Skills Acquisition in Fetoscopic Laser Photocoagulation of Monochorionic Diamniotic Twin Placenta Using Realistic Simulators

Fetoscopic laser coagulation of arterio-venous anastomoses (AVA) in a monochorionic placenta is the standard of care for twin-twin transfusion syndrome (TTTS), but is technically challenging and can lead to significant complications. Acquiring and maintaining the necessary surgical skills require consistent practice, a critical caseload, and time. Training on realistic surgical simulators can potentially shorten this steep learning curve and enables several proceduralists to acquire procedure-specific skills simultaneously. Here we describe realistic simulators designed to allow the user familiarity with the equipment and specific steps required in the surgical treatment of TTTS, including fetoscopic handling, approaches to anterior and posterior placenta, recognition of anastomoses, and efficient coagulation of vessels. We describe the skills that are especially important in conducting placental laser coagulation that the surgeon can practice on the model and apply in a clinical case. These models can be adapted easily depending on the availability of materials and require standard fetoscopy equipment. Such training systems are complementary to traditional surgical apprenticeships and can be useful aids for fetal medicine units that provide this clinical service.

Overview of Fetal Therapy

An improving ability to diagnose fetal conditions with higher accuracy prompts an attempt for a salvage treatment in utero. Fetal conditions amendable for prenatal intervention are limited to only diseases that can either kill the baby in utero or leave the baby with significant handicap. Therapeutic intervention has to be highly selective. Factors that need to be considered before offering in utero treatment include the highly investigative nature of certain procedures. For instance, at the time of writing this paper, fetal endoluminal tracheal occlusion for severe congenital diaphragmatic hernia is still under a randomized controlled investigational trial to validate its potential benefits and risks to the fetus with severe congenital diaphragmatic hernia with suboptimal growth of the residual lung tissue. There are chances of procedure-related miscarriages, preterm premature rupture of the membranes, and maternal morbidity that need to be discussed in an unbiased counseling session. The right balance between potential benefits and harms requires validation with rigid scientific methodology before the practice has become a “standard of care.” For example, laser photocoagulation of anastomosing chorionic vessels has become a standard of care in many places due to its superior perinatal survival and composite outcomes, particularly for the childhood neurodevelopmental status. With an ongoing technological development, it is foreseeable that there will be more proposals of implementing novel medical technologies to the use of fetal therapy. Currently, there are only a handful of fetal care centers, and most of the experienced ones are clustered in developed part of Europe and the USA. Dissemination of this type of service which requires years of experiences to develop surgical skill and the support by the most technological advanced instrument and setting is a real challenge that need to be addressed, discussed, and solved as a global agenda.