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Cureus May 2024Introduction Congenital malformation studies serve several purposes, including establishing baseline rates, monitoring changes over time, exploring the origins of these...
Introduction Congenital malformation studies serve several purposes, including establishing baseline rates, monitoring changes over time, exploring the origins of these defects, and helping in planning health services. Increasing public awareness about pediatric surgical interventions is another goal of these studies. However, the impact of congenital malformations is often underestimated in developing countries due to insufficient healthcare data and diagnostic facilities, particularly in rural areas. Families affected by the birth of a child with congenital malformations face significant stress and hardship. Methods The main aims of this study were to evaluate the clinical pattern of congenital structural malformations in our region (Uttarakhand, India), identify possibly associated factors of congenital malformations, and find out the immediate outcome of congenital malformations in enrolled participants. Results Among a total of 150 cases, 73 (48.7%) cases were inborn, whereas 77 (51.3%) cases were outborn. Investigation of congenital malformation revealed cleft lip or palate in 37 (24.7%) cases, congenital heart disease (CHD) in 33 (22%) cases, meningomyelocele (MMC) in 18 (12.0%) cases, anorectal malformation (ARM) in 11 (7.3%) cases, hypospadias in 10 (6.7%) cases, congenital talipes equinovarus (CTEV) in nine (6.0%) cases, tracheoesophageal fistula (TEF) in nine (6.0%) cases, polydactyly in seven (4.7%) cases, pelviureteric junction obstruction (PUJO) in four (2.7%) cases, duodenal atresia in three (2.0%) cases, midgut volvulus in three (2.0%) cases, umbilical sinus in two (1.3%) cases, sacrococcygeal teratoma (SCT) in one (0.7%) case, phimosis in one (0.7%) case, microtia in one (0.7%) case, and micrognathia in one (0.7%) case. Mortality was observed in 11 (7.3%) cases, whereas 105 (70%) cases were successfully discharged. Among 11 mortality cases, the cause of death was CHD in seven (63.2%) cases, TEF+CHD in two (18.1%) cases, MMC in one (9%) case, and duodenal atresia in one (9%) case. Conclusion Contrary to the common belief that advanced maternal age of greater than 35 years is a major cause, 86.6% of the congenital structural anomalies in our hospital-based study in Uttarakhand occurred in babies of mothers belonging to the age group of 18-30 years. Also, consanguineous marriage was observed in only 3.3% of cases, indicating that it may not be a major contributing factor causing congenital structural malformations in our region. External congenital anomalies are most commonly observed (60.7%), with cleft lip and cleft palate being the most common. The most frequently observed internal congenital anomaly is CHD (22%) followed by gastrointestinal (GI) (18.6%) and urinary anomalies (10.1%). Death and referral are commonly seen in CHD.
PubMed: 38883135
DOI: 10.7759/cureus.60375 -
International Journal of Surgery Case... Jul 2024Sacrococcygeal teratoma is a rare extragonadal germ cell tumor occurring at the lower end of the back. It is congenital, and can be diagnosed in utero or just after...
INTRODUCTION AND IMPORTANCE
Sacrococcygeal teratoma is a rare extragonadal germ cell tumor occurring at the lower end of the back. It is congenital, and can be diagnosed in utero or just after birth. These tumors are sporadic in nature, more common in females than in males. They can be solid, cystic or a mixture of the two in consistency. Authors present this case to share their experience because these congenital tumors are rare and a lapse in appropriate management can be devastating because of chances of recurrence and the possibility of a malignant nature of the disease.
CASE PRESENTATION
Authors in this case present a baby that was referred to their center 5 days post vaginal delivery with a huge mass on the sacrococcygeal region. MRI revealed sacrococcygeal teratoma type II. Wide local excision was done. Tumor margins were microscopically negative. The patient recovered well and was discharged. The patient did not require adjuvant treatment.
CLINICAL DISCUSSION
Routine obstetric ultrasound can diagnose sacrococcygeal teratoma with 100 % sensitivity. Fetuses with tumors <5 cm in size can be delivered vaginally. Tumors larger than that should be delivered at term through cesarean section. High risk sacrococcygeal teratomas can be delivered at 28 weeks of gestation by cesarean section followed by ex-utero intrapartum therapy. MRI is useful to assess the intrapelvic extent of the tumor and to plan management. Surgical intervention should be done after delivery.
CONCLUSION
Intrauterine diagnosis is not an indication for termination of pregnancy. Surgical resection with negative margins provides a cure. Histological examination of the tumor is mandatory. For malignant sacrococcygeal teratomas, adjuvant chemotherapy is necessary for positive tumor margin after surgical resection. Positive margins may require chemotherapy. Neoadjuvant chemotherapy may be used for tumor debulking prior to surgery if the tumor infiltrates neighboring structures or is metastatic.
PubMed: 38852569
DOI: 10.1016/j.ijscr.2024.109895 -
Journal of Clinical Medicine Apr 2024This study aims to evaluate the outcomes of fetal sacrococcygeal teratoma (SCT) submitted to prenatal interventions. We performed a systematic literature review of... (Review)
Review
This study aims to evaluate the outcomes of fetal sacrococcygeal teratoma (SCT) submitted to prenatal interventions. We performed a systematic literature review of fetal SCT patients and compared the outcomes between open fetal surgery and percutaneous intervention. In addition, we also compared the results of SCT fetuses who did not undergo any surgical intervention (NI). We identified 16 cases of open fetal surgery (OS), 48 cases of percutaneous fetal intervention (PI), and 93 NI patients. The survival rate was 56.2% in OS, 45.8% in PI ( = 0.568), and 71.0% in NI patients. The gestational age at delivery was earlier in cases where there was no survival compared to cases where the fetuses did survive across all evaluated cohorts (OS: = 0.033, PI: < 0.001, NI: < 0.001). The gestational weeks at delivery in OS and PI fetuses were more similar; however, OS tended to be performed later on in pregnancy, and the affected fetuses had more severe presented findings. In our evaluation, we determined that the presence of fetal hydrops and cardiac failure had no significant impact on survival in SCT cases. In NI patients, polyhydramnios was much higher in fetuses who did not survive compared to their surviving cohorts ( < 0.001). In conclusion, gestational age at delivery can affect the short-term prognosis of fetuses affected with sacrococcygeal teratomas. Regardless of the mode of delivery or the necessity for intervention during the fetal period, monitoring for complications, including polyhydramnios, can prevent premature delivery.
PubMed: 38731178
DOI: 10.3390/jcm13092649 -
Medicine Apr 2024Adult sacrococcygeal teratoma (SCT) is a rare disease that is not easily detected or easily missed, and its treatment is based on surgery, including transabdominal,...
INTRODUCTION
Adult sacrococcygeal teratoma (SCT) is a rare disease that is not easily detected or easily missed, and its treatment is based on surgery, including transabdominal, transsacral, or a combination of both, but there are no clear guidelines for diagnosis and treatment. We share a case of Altman type III SCT in order to provide more reference protocols for the diagnosis and treatment of adult SCT, and more importantly to increase our understanding of different types of SCT cases in adults.
PATIENT CONCERNS
Our patient was a 31-year-old adult woman who underwent complete surgical resection of a cystic mature teratoma of the right ovary 8 years ago and is currently 13 months postpartum without menstruation, usually with a feeling of anal bulge, with symptoms such as constipation.
DIAGNOSIS
We diagnosed SCT by vaginal ultrasonography, computed tomography and magnetic resonance imaging (MRI); benign tumors were considered in the results of serum tumor markers.
INTERVENTIONS
We chose the surgical approach of laparoscopic transabdominal-sacrococcygeal approach to completely remove the patient SCT and coccyx.
OUTCOMES
The location of SCT is concealed and the clinical symptoms are not obvious. Vaginal ultrasonography, CT and MRI can not only improve the diagnostic rate of SCT, but also understand the size and mass of SCT, providing an exact basis for clinicians to select the laparoscopic transabdominal-sacrococcygeal approach.
CONCLUSION
Our sharing increases the reports of rare cases of teratoma with the same histological findings in different organ tissues of the same patient at different times, whether this occurs incidentally requires more case reports and further basic research; in addition, the laparoscopic transabdominal-sacrococcygeal approach is a safe and effective surgical approach for the treatment of Altman type III SCT in adults; finally, this case reminds us that SCT may not affect pregnancy and pregnancy outcomes and provides a reference for the selection of interventions for SCT with pregnancy.
Topics: Humans; Female; Teratoma; Adult; Laparoscopy; Sacrococcygeal Region; Ovarian Neoplasms; Magnetic Resonance Imaging
PubMed: 38669424
DOI: 10.1097/MD.0000000000037887 -
Journal of Pediatric Surgery Feb 2024Serum alpha-fetoprotein (AFP) is often used as tumour marker for recurrent sacrococcygeal teratoma (SCT). We aimed to assess the normal dynamics of serum AFP levels...
BACKGROUND
Serum alpha-fetoprotein (AFP) is often used as tumour marker for recurrent sacrococcygeal teratoma (SCT). We aimed to assess the normal dynamics of serum AFP levels after initial resection and diagnostic accuracy of serum AFP levels the follow-up for recurrence in SCT.
METHODS
This retrospective study included 57 patients treated for SCT in the six pediatric surgical centers in the Netherlands from 1980 to 2018.
MAIN RESULTS
57 patients were included in the study of whom 19 children developed 20 recurrences at a median of 14.0 months after initial resection. No significant difference was found in serum AFP level dynamics between the recurrence and non-recurrence group after initial resection (p = 0.950). Serum AFP levels did not significantly increase before recurrence (p = 0.106) compared to serum AFP levels of children without recurrence at the same time. However, serum AFP levels did significantly increase in malignant recurrences (n = 7) (p = 0.03) compared to patients without recurrence. A cut-off value of 55 μg/L was found to be predictive for recurrent SCT with an Area Under the Curve (AUC) of 0.636 with sensitivity of 50% and specificity of 100%.
CONCLUSION
Dynamics of serum AFP levels are not different between patients with and without recurrence after initial resection of SCT. Serum AFP levels are not predictive for mature or immature recurrent SCT and normal AFP levels do not rule out recurrent SCT. However, serum AFP levels exceeding 55 μg/L can indicate recurrent SCT, especially malignant recurrences.
PubMed: 38418277
DOI: 10.1016/j.jpedsurg.2024.01.048 -
Journal of Indian Association of... 2024This case report describes a 4-year-old girl with an isolated neurofibroma in the sacrococcygeal region. Although initially resembling sacrococcygeal teratoma,...
This case report describes a 4-year-old girl with an isolated neurofibroma in the sacrococcygeal region. Although initially resembling sacrococcygeal teratoma, histopathology revealed a benign nerve sheath tumor. Wide local excision was performed, and the final diagnosis was plexiform neurofibroma. Diagnostic challenges in rare childhood tumors require stepwise evaluation and multidisciplinary team discussions.
PubMed: 38405243
DOI: 10.4103/jiaps.jiaps_136_23 -
Diagnostics (Basel, Switzerland) Jan 2024Immature sacrococcygeal teratoma represents a histological form with rapid tumor growth, a risk of premature birth, an enhanced rate of complications, an increased risk...
Immature sacrococcygeal teratoma represents a histological form with rapid tumor growth, a risk of premature birth, an enhanced rate of complications, an increased risk of recurrence, and a higher mortality rate than the mature type. Thus, prenatal diagnosis of immature forms would significantly improve the prognosis of these cases. To this end, we performed an extensive literature review on the diagnosis, therapeutic management, and follow-up of immature teratomas. Regarding this medical conduct, we also presented our case. In conclusion, the early identification of immature sacrococcygeal teratomas with or without other associated structural abnormalities and their correct therapeutic approach are basic principles for a favorable evolution of these cases.
PubMed: 38337762
DOI: 10.3390/diagnostics14030246 -
JNMA; Journal of the Nepal Medical... Aug 2023Sacrococcygeal teratomas are common tumours in neonates and infants, primarily affecting females. A 35-year-old primigravida presented with a large sacrococcygeal...
UNLABELLED
Sacrococcygeal teratomas are common tumours in neonates and infants, primarily affecting females. A 35-year-old primigravida presented with a large sacrococcygeal teratoma that was detected during the 30th week of gestation in the fetus. The baby was delivered via elective caesarean section at 36+3 weeks, and surgical excision of the 10x10x5 cm³ mass was performed successfully on the third day of life. Despite a surgical site infection, the patient had a favourable outcome with normal vital signs, bowel, bladder, and lower extremity functions upon discharge. Early diagnosis and prompt management of sacrococcygeal teratoma in newborns is vital for optimal outcomes, providing valuable insights and guidance to medical practitioners.
KEYWORDS
anaesthesia; case reports; neonate; teratoma.
Topics: Humans; Pregnancy; Infant, Newborn; Female; Adult; Cesarean Section; Sacrococcygeal Region; Teratoma; Spinal Neoplasms; Anesthesia
PubMed: 38289807
DOI: 10.31729/jnma.8251 -
European Journal of Pediatric Surgery :... Apr 2024Patient-reported outcome measures (PROMs) can be employed in both research and clinical care to enhance our understanding of outcomes that matter to patients. This... (Review)
Review
A Narrative Review of Patient-Reported Outcome Measures and Their Application in Recent Pediatric Surgical Research: Advancing Knowledge and Offering New Perspectives to the Field.
INTRODUCTION
Patient-reported outcome measures (PROMs) can be employed in both research and clinical care to enhance our understanding of outcomes that matter to patients. This narrative review aims to describe PROM use in recent pediatric surgical research, identify and describe psychometrically robust PROMs, providing an overview of those derived from pediatric patient input, and make recommendations for future research.
MATERIALS AND METHODS
A search was conducted to identify articles published from 2021 to August 2023 describing the availability and/or use of at least one valid or reliable PROM in children with conditions including anorectal malformations, biliary atresia, congenital diaphragmatic hernia, duodenal atresia, esophageal atresia, abdominal wall defects, Hirschsprung's disease, sacrococcygeal teratoma, and short bowel syndrome. Articles were categorized based on their objectives in applying PROMs. Psychometrically robust PROMs were identified and described.
RESULTS
Out of the 345 articles identified, 49 met the inclusion criteria. Seventeen focused on esophageal atresia and 14 on Hirschsprung's disease. Twenty-nine PROMs were identified, with 12 deemed psychometrically robust. Seven psychometrically robust PROMs were developed using patient input in the primary item generation. Most PROMs were applied to advance understanding of conditions and/or treatment and fewer were developed or psychometrically evaluated. No PROMs were assessed for their impact or incorporated into an implementation study.
CONCLUSIONS
This review reveals gaps in the application of PROMs in recent pediatric surgical research. Emphasis should be placed on the development and utilization of psychometrically robust PROMs, broadening the scope of covered diseases, conducting impact assessments, and evaluating implementation strategies.
Topics: Humans; Child; Quality of Life; Hirschsprung Disease; Patient Reported Outcome Measures; Short Bowel Syndrome
PubMed: 38272041
DOI: 10.1055/s-0043-1778108