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Surgical Case Reports Jan 2021Teratoma is a true neoplasm and originates from the three germ cell layers and it can contain any tissue derived from these layers. The location of teratoma is variable...
BACKGROUND
Teratoma is a true neoplasm and originates from the three germ cell layers and it can contain any tissue derived from these layers. The location of teratoma is variable according to the age group. In adults, sacrococcygeal teratoma is rare and carries a low risk of malignant transformation. Surgical resection is the mainstay of treatment and is challenging due to tumor location.
CASE PRESENTATION
We are presenting a case report of a 16-year old female referred to our hospital with recurrent attacks of urine retention. Imaging study showed a large sacrococcygeal tumor. It was successfully resected by a combined laparoscopic and posterior approach without any major complication.
CONCLUSION
The combined laparoscopic and posterior approach is a safe surgical technique for resection of the large sacrococcygeal tumor. This surgical method has been published around 10 times in separated reports around the world and for first time in our region.
PubMed: 33438088
DOI: 10.1186/s40792-020-01104-4 -
Diagnostics (Basel, Switzerland) Feb 2023Fetal heart failure (FHF) is a condition of inability of the fetal heart to deliver adequate blood flow for tissue perfusion in various organs, especially the brain,... (Review)
Review
Fetal heart failure (FHF) is a condition of inability of the fetal heart to deliver adequate blood flow for tissue perfusion in various organs, especially the brain, heart, liver and kidneys. FHF is associated with inadequate cardiac output, which is commonly encountered as the final outcome of several disorders and may lead to intrauterine fetal death or severe morbidity. Fetal echocardiography plays an important role in diagnosis of FHF as well as of the underlying causes. The main findings supporting the diagnosis of FHF include various signs of cardiac dysfunction, such as cardiomegaly, poor contractility, low cardiac output, increased central venous pressures, hydropic signs, and the findings of specific underlying disorders. This review will present a summary of the pathophysiology of fetal cardiac failure and practical points in fetal echocardiography for diagnosis of FHF, focusing on essential diagnostic techniques used in daily practice for evaluation of fetal cardiac function, such as myocardial performance index, arterial and systemic venous Doppler waveforms, shortening fraction, and cardiovascular profile score (CVPs), a combination of five echocardiographic markers indicative of fetal cardiovascular health. The common causes of FHF are reviewed and updated in detail, including fetal dysrhythmia, fetal anemia (e.g., alpha-thalassemia, parvovirus B19 infection, and twin anemia-polycythemia sequence), non-anemic volume load (e.g., twin-to-twin transfusion, arteriovenous malformations, and sacrococcygeal teratoma, etc.), increased afterload (intrauterine growth restriction and outflow tract obstruction, such as critical aortic stenosis), intrinsic myocardial disease (cardiomyopathies), congenital heart defects (Ebstein anomaly, hypoplastic heart, pulmonary stenosis with intact interventricular septum, etc.) and external cardiac compression. Understanding the pathophysiology and clinical courses of various etiologies of FHF can help physicians make prenatal diagnoses and serve as a guide for counseling, surveillance and management.
PubMed: 36832267
DOI: 10.3390/diagnostics13040779 -
Pediatrics and Neonatology Feb 2018Neonatal tumors are different from tumors of the older children and knowledge gained from treating older children can not be extrapolated to neonates. Neonates have...
BACKGROUND
Neonatal tumors are different from tumors of the older children and knowledge gained from treating older children can not be extrapolated to neonates. Neonates have immature physiology and their haematopoietic and immune systems are not fully developed and the response to therapy is unpredictable. Hence it is imperative to study these tumors as separate entity. The aim of this study is to analyse this rare set of tumors in terms of their incidence, clinical features and management.
MATERIALS AND METHODS
All babies admitted in our hospital with tumors from January, 2011 to January 2016 were studied. Tumor-like conditions like haemangioma, lymphangioma and hamartomas were included. The age, sex distribution, type of tumor and management were studied.
RESULTS
A total of 51 cases were registered out of which, 29 cases were haemangiomas and lymphangiomas. Of remaining 20 cases, 5 were benign ovarian cysts, 3 were neuroblastomas, 3 were congenital fibrosarcomas, 3 were sacrococcygeal teratomas. Wilm's tumor, congenital mesoblastic nephroma, haemangioendothelioma of liver and others formed the remaining six cases.
CONCLUSION
Our study insists that the neonatal tumors are distinct subset of pediatric tumors, requiring careful selection of treatment modalities and most of the solid tumors can be successfully managed if diagnosed and treated early. Neonatal tumors are defined as tumors which are diagnosed before the first month of life. Some of them can be congenital (present at birth). Neonatal tumors are different from tumors in older children in terms of etiopathogenesis, behavior and response to therapy as well as long-term outcomes.
Topics: Female; Humans; Incidence; India; Infant; Infant, Newborn; Male; Neoplasms; Prognosis
PubMed: 28736178
DOI: 10.1016/j.pedneo.2016.12.007 -
Autopsy & Case Reports 2021The sacrococcygeal region is the most common site for the extragonadal germ cell tumors comprising seminomatous and non-seminomatous tumors. Seminomatous tumors are... (Review)
Review
The sacrococcygeal region is the most common site for the extragonadal germ cell tumors comprising seminomatous and non-seminomatous tumors. Seminomatous tumors are seminomas, and non-seminomatous tumors comprise mainly teratoma (mature and immature), yolk sac tumor (YST), embryonal carcinoma (EC), and choriocarcinoma. These tumors occur in newborns, infants, and adolescents. Other common sites for extragonadal germ cell tumors are the brain and mediastinum, although they may occur anywhere in the body. These tumors may occur in mixed as well as pure form. So, sectioning from different areas should be done before labeling them as pure germ cell tumors. YST, in its pure form, is rare and therefore should not be missed as it is chemosensitive. The patient should be thoroughly assessed clinically. Imaging also becomes necessary while evaluating swelling in the sacrococcygeal region and can aid in differentials. When the clinical and imaging suspicion of either Sacrococcygeal teratoma or other germ cell tumor is high, serum biomarkers as alfa-fetoprotein should be requested. The serum levels are necessary and should be done preoperatively, postoperatively, and during the course of chemotherapy as follow-up. However, the final diagnosis rests on the histopathological diagnosis. We report one such case of pure YST in the sacrococcygeal region in a 9-month-old female child. The imaging suggested sacrococcygeal teratoma type 4, and high alfa-fetoprotein levels were determined postoperatively.
PubMed: 34249791
DOI: 10.4322/acr.2021.287 -
Annals of Translational Medicine Oct 2016Historically, the gold standard for the treatment of congenital malformations has been planned delivery at tertiary care center with attempted post-natal repair or... (Review)
Review
Historically, the gold standard for the treatment of congenital malformations has been planned delivery at tertiary care center with attempted post-natal repair or amelioration of the lesion. Over the last few decades however, rapid advances in imaging and instrumentation technology combined with superior knowledge of fetal pathophysiology has led to the development of novel intrauterine interventions for most common fetal anomalies. Great success has already been seen the treatment of previous devastating anomalies such as myelomeningocele (MMC), congenital cystic malformations of the lung, twin-twin transfusion, and sacrococcygeal teratomas. Although still limited, these innovative techniques have unique potential to improve outcomes in the most devastating fetal anomalies.
PubMed: 27867946
DOI: 10.21037/atm.2016.10.34 -
Fetal Diagnosis and Therapy 2022Although maternal-fetal surgery to treat fetal anomalies such as spina bifida continues to grow more common, potential health disparities in the field remain relatively... (Review)
Review
BACKGROUND/PURPOSE
Although maternal-fetal surgery to treat fetal anomalies such as spina bifida continues to grow more common, potential health disparities in the field remain relatively unexamined. To address this gap, we identified maternal-fetal surgery studies with the highest level of evidence and analyzed the reporting of participant sociodemographic characteristics and representation of racial and ethnic groups.
METHODS
We conducted a systematic review of the scientific literature using biomedical databases. We selected randomized control trials (RCTs) and cohort studies with comparison groups published in English from 1990 to May 5, 2020. We included studies from across the globe that examined the efficacy of fetal surgery for twin-twin transfusion syndrome (TTTS), obstructive uropathy, congenital diaphragmatic hernia (CDH), myelomeningocele (MMC), thoracic lesions, cardiac malformations, or sacrococcygeal teratoma. We determined the frequency of reporting of age, gravidity/parity, race, ethnicity, education level, language spoken, insurance, income level, and relationship status. We identified whether sociodemographic factors were used as inclusion or exclusion criteria. We calculated the racial and ethnic group representation for studies in the USA using the participation-to-prevalence ratio (PPR).
RESULTS
We included 112 studies (10 RCTs, 102 cohort) published from 1990-1999 (8%), 2000-2009 (30%), and 2010-2020 (62%). Most studies were conducted in the USA (47%) or Europe (38%). The median sample size was 58. TTTS was the most common disease group (37% of studies), followed by MMC (23%), and CDH (21%). The most frequently reported sociodemographic variables were maternal age (33%) and gravidity/parity (20%). Race and/or ethnicity was only reported in 12% of studies. Less than 10% of studies reported any other sociodemographic variables. Sociodemographic variables were used as exclusion criteria in 13% of studies. Among studies conducted in the USA, White persons were consistently overrepresented relative to their prevalence in the US disease populations (PPR 1.32-2.11), while Black or African-American, Hispanic or Latino, Asian, American-Indian or Alaska-Native, and Native-Hawaiian or other Pacific Islander persons were consistently underrepresented (PPR 0-0.60).
CONCLUSIONS
Sociodemographic reporting quality in maternal-fetal surgery studies is poor and inhibits examination of potential health disparities. Participants enrolled in studies in the USA do not adequately represent the racial and ethnic diversity of the population across disease groups.
Topics: Black People; Ethnicity; Female; Hispanic or Latino; Humans; Maternal Age; Pregnancy; United States
PubMed: 35272297
DOI: 10.1159/000523867 -
BMC Pregnancy and Childbirth Dec 2020Sacrococcygeal teratoma is one of the most common congenital tumors in newborns and infancy. The incidence is 1 per 20,000-40,000 live births. Ultrasonography is an... (Review)
Review
BACKGROUND
Sacrococcygeal teratoma is one of the most common congenital tumors in newborns and infancy. The incidence is 1 per 20,000-40,000 live births. Ultrasonography is an optimal method for prenatal screening and diagnosis of fetal sacrococcygeal teratoma. MRI can be used to assist in the diagnosis. However, sacrococcygeal teratoma in the twin pregnancy is rare.
CASE PRESENTATION
We reported a case of one twin with sacrococcygeal teratoma in dichorionic-diamniotic twin pregnancy.One twin with sacrococcygeal teratoma was diagnosed at the second trimester by ultrasonic examination and another twin was normal. A regular and careful antenatal care was conducted by the multidisciplinary team. The parents refused to perform the fetal MRI and examine the chromosome of both twin.At 37 + 1 of gestation, planned cesarean section was performed. The healthy male co-twin (twin A) weighed 2880 g.The male twin with SCT (twin B) weighed 2900 g, complying with 6 × 3 × 3 cm cystic and solid mass in sacrococcygeal region. At four days of age twin B underwent excisional surgery of the sacrococcygeal teratoma and coccyx and discharged 7 days after surgery. The mother and both babies were followed up and are all in good health until now.
CONCLUSION(S)
Sacrococcygeal teratoma in twin pregnancy is rare. Early antenatal diagnosis is important. Once the sacrococcygeal teratoma is diagnosed, clinicians should be aware of the associated maternal and fetal complications. Expecting parents should be counseled by the multidisciplinary team about the management and prognosis of the STC twin and co-twin. Prompt surgical excision of the sacrococcygeal teratoma after birth should be suggested.
Topics: Adult; Cesarean Section; Female; Humans; Infant, Newborn; Magnetic Resonance Imaging; Male; Pregnancy; Pregnancy, Twin; Sacrococcygeal Region; Teratoma; Ultrasonography, Prenatal
PubMed: 33267783
DOI: 10.1186/s12884-020-03454-1 -
Journal of Neonatal Surgery 2013
PubMed: 26023438
DOI: No ID Found -
Surgical Case Reports Oct 2023Sacrococcygeal teratomas (SCTs) are known to cause urological complications, but urethrovaginal (UV) fistula as a complication of SCT is rare. We herein report a case of...
BACKGROUND
Sacrococcygeal teratomas (SCTs) are known to cause urological complications, but urethrovaginal (UV) fistula as a complication of SCT is rare. We herein report a case of SCT with UV fistula and hydrocolpos.
CASE PRESENTATION
A 1-day-old female neonate presented to our department with prominent swelling in the sacrococcygeal region. She was born at 37 gestational weeks via spontaneous vaginal delivery from a 39-year-old woman. The weight of the baby was 2965 g, and her Apgar scores were 4/10 (at 1 and 5 min). An MRI examination confirmed an 11 × 11 cm Altman classification typeII SCT associated with hydrocolpos, a dilated urinary bladder, and bilateral hydronephrosis. When she was 5 days, the SCT was excised totally and a coccygectomy was performed. After the operation, as her urinary output appeared unstable, a cystoscopic examination was performed on the third postoperative day. This revealed that the UV fistula was located approximately 1 cm from the urethral opening. In addition, the proximal urethra was unobstructed and connected to the bladder. The cystoscope allowed for the passage of a urinary catheter through the urethra. After 1 month of catheter placement, she was discharged from the hospital at 57 days of age. Follow-up was uneventful, with neither urinary infection nor retention.
CONCLUSIONS
SCTs are associated with not only trouble with rectal function and lower extremity movement but also urinary complications. The pathogenesis of this UV fistula is thought to be the rapid growth of the SCT that developed in the fetal period, resulting in obstruction of the urethra by the tumor and the pubic bone, which in turn caused urinary retention and the formation of a fistula as an escape route for the pressure. Because SCTs can cause a variety of complications depending on the course of the disease, careful examination and follow-up are necessary.
PubMed: 37903968
DOI: 10.1186/s40792-023-01772-y -
Journal of Indian Association of... 2020The study focuses on the clinical presentation, management, and outcomes (both short term and long term) in patients with sacrococcygeal teratoma managed over a decade...
INTRODUCTION
The study focuses on the clinical presentation, management, and outcomes (both short term and long term) in patients with sacrococcygeal teratoma managed over a decade in a tertiary care center.
MATERIALS AND METHODS
This is a prospective study on children with sacrococcygeal teratoma over 12 years data collected included antenatal diagnosis, mode of delivery, age at diagnosis, clinical presentation, physical extent of mass (including Altman classification), levels of alpha-fetoprotein, surgical approach, histopathology, clinical outcome, recurrence and long-term results including bladder-bowel dysfunction and neurological impairment. Functional results were evaluated clinically and radiologically.
RESULTS
During the study, 41 patients (male to female ratio of 1:3.1) with a median age of 36 days (1 day-11.6 years) with sacrococcygeal teratoma were managed at our center. The mean follow-up duration was 54 months (range 19-110 months). Nearly, two-thirds of the tumors were either Altman Type 1 or 2. Yolk sac tumor was present in 8 (19.5%) patients, while the rest has either mature or immature teratoma. Tumors were removed through a posterior sagittal or a chevron incision. In seven patients, abdominosacral approach was necessary. Eight patients with malignant disease received chemotherapy (neoadjuvant in 5). Overall survival was 95% at a mean follow-up of 54 months. Among the late complications, three patients had a local recurrence of tumor, and urinary dribbling was present in three patients.
CONCLUSIONS
Teratomas are the most common germ cell tumors of the sacrococcygeal region. Most of the tumors are benign, and the incidence of malignancy increases with age. The evaluation of malignancy is, therefore, necessary in these children. Excellent survival of 95% was achieved in this series. Morbidity due to associated malformation, disease recurrence, and treatment may persist in these patients; hence, proper follow-up is needed.
PubMed: 31896894
DOI: 10.4103/jiaps.JIAPS_219_18