Presentation
The median age at presentation was four days. 69.44% of patients presented during the neonatal period. 77.77% of patients presented with sacrococcygeal mass followed by lower abdominal mass in 11.11%. [nepjol.info]
CASE DESCRIPTION Clinical presentation A 23-year-old female presented with pelvic pain. Magnetic resonance imaging (MRI) of the pelvis revealed a complex cystic structure anterior to the coccyx [ Figure 1 ]. [surgicalneurologyint.com]
PRESENTATION OF CASE: We present a giant, long standing Sacrococcygeal teratoma in an adult female patient which caused her significant obstructing lower gastrointestinal and urinary symptoms besides the discomfort and disfigurement of the sacral region [read.qxmd.com]
Neonatal tumors present at birth protruding from the sacral site and are usually mature or immature teratomas. [en.wikipedia.org]
RESULTS: Patients most commonly presented with pelvic pain (n 16) and/or a palpable mass (n 15). On radiology, 8 lesions were purely cystic, 14 were mixed, and 4 were solid; teratoma was suspected as a diagnosis in 8 patients. [mayoclinic.pure.elsevier.com]
Entire Body System
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Asymptomatic
SCT may be asymptomatic, cause constipation, obstruction to urinary flow, low back pain, lower extremity paresis/paresthesias, or dystocia 1,4. [eurorad.org]
Small tumors often do not cause any symptoms (asymptomatic) and can usually be removed surgically after birth without difficulty. However, larger sacrococcygeal tumors can cause a variety of complications before and after birth. [rarediseases.org]
Most cases are asymptomatic in utero and diagnosed after birth. Fetuses with this malformation are at risk for significant perinatal morbidity and mortality [1, 3, 4, 7, 9]. [obgyn.net]
Patients with SCTs can be asymptomatic or have signs of obstruction of the rectum or colon, such as constipation or frequent stools, or obstruction of the bladder neck ( 27 ). Fig. 3. Immature SCT in a male neonate. [journals.sagepub.com]
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Falling
Chemotherapy will be initiated if there is evidence of tumor recurrence based on failure of AFP levels to fall or recurrent mass accumulation. References 1 Gabra JP, Jesudason EC, McDowell HP, Pizer BL, Losty PD. [nature.com]
PMID: 25183379 ; DOI: 10.3109/15513815.2014.949934 Ndour O, Ngom G, Faye Fall A, et al. Aspects épidémiologiques, diagnostiques et thérapeutiques du tératome sacro-coccygien chez l'enfant : analyse de 11 observations. [scitemed.com]
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Collapse
During dissection of the presacral space the baby experienced complete cardiovascular collapse due to a paradoxical air embolism. [fetalcaredallas.com]
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HELLP Syndrome
Among the 26 patients continuing the pregnancy, 81% experienced obstetric complications including polyhydramnios (n 7), oligohydramnios (n 4), preterm labor (n 13), preeclampsia (n 4), gestational diabetes (n 1), HELLP syndrome (n 1), and hyperemesis [fetalcaredallas.com]
Cardiovascular
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Hypertension
On evaluation, he had pulmonary hypertension, hydrops, thrombocytopenia, hypoglycemia, respiratory failure, ascites and metabolic acidosis. [academic.oup.com]
Because of a hyperdynamic cardiovascular state, the mother develops symptoms that are similar to pre-eclampsia and may include vomiting, hypertension, peripheral edema (swelling of the hands and feet), proteinuria (protein in the urine), and pulmonary [fetus.ucsf.edu]
The neonatal period was further complicated by pulmonary hypertension and chronic lung disease requiring prolonged ventilation. [obgyn.onlinelibrary.wiley.com]
If neglected, hydrops can also be dangerous for the mother resulting in similar symptoms of swelling, hypertension, and fluid on the lungs with shortness of breath. [rarediseases.org]
In addition, the mother had mild hypertension, edema, and proteinuria. Preterm labor developed that was controlled with tocolytic agents. [fetalcaredallas.com]
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Tachycardia
At 25 weeks of gestation a type II SCT had rapid enlargement and development of polyhydramnios and placentomegaly, with associated maternal tachycardia and proteinuria suggesting impending maternal mirror syndrome. [fetalcaredallas.com]
Psychiatrical
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Claustrophobia
Fetal MR imaging was recommended to better characterize the mass; however, the patient refused due to claustrophobia. The follow-up fetal sonography at 36 weeks showed the mass had grown (10 10 6 cm) and the AFI had increased to 24 cm. Fig 1. [ajnr.org]
Neurologic
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Irritability
Tumor mass effect may also result in uterine irritability and preterm delivery because of uterine distention (Flake et al., 1986; Bond et al., 1990). [fetalcaredallas.com]
Urogenital
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Urinary Incontinence
Faecal and urinary incontinence were present in nine. [pmj.bmj.com]
They account for 50% of teratomas in children but are rarely seen in adults.[ 3 ] The clinical presentation in adults includes bowel dysfunctions, urinary incontinence, lower back pain, and/or venous engorgement of the lower limbs. [surgicalneurologyint.com]
Later complications of the mass effect and/or surgery may include neurogenic bladder, other forms of urinary incontinence, fecal incontinence, and other chronic problems resulting from accidental damage to or sacrifice of nerves and muscles within the [en.wikipedia.org]
Urinary incontinence and vesicourethral dysfunction in pediatric surgical conditions. Semin Pediatr Surg 2002; 11:91-99 [Google Scholar] [ajronline.org]
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Cervical Incompetence
The examination included a full evaluation of the fetus ( to assess fetal anatomy, size, position and state ), placenta, amniotic fluid and uterus as well as the cervix and lower uterine segment ( to look for evidence of cervical incompetence or placenta [obgyn.net]
Workup
[…] the International Society for Pediatric Neurosurgery # 32 REVIEW Apeksha Chaturvedi, Arie Franco, Abhishek Chaturvedi, Nina B Klionsky The objective of this review is to describe antenatal and postnatal imaging criteria, which allow diagnosis and aid workup [read.qxmd.com]
Treatment
Unlike Type I teratomas, prenatal treatment is not an option, so babies with types II, III and IV must be born before treatment begins. [rileychildrens.org]
Our fetal care team will also work with families to provide informed treatment plans based on the type, size, and composition of a fetal sacrococcygeal teratoma (verified by a fetal MRI). [childrenscolorado.org]
At the end of the day, our team will sit down with your family to explain your baby’s diagnosis, discuss treatment options, and answer your questions. [chop.edu]
The UC Davis Fetal Care and Treatment Center unites leaders in maternal-fetal care with renowned fetal, neonatal and pediatric surgeons to provide advanced diagnosis and treatment for complex conditions that can affect newborns. [health.ucdavis.edu]
All patients with sacrococcygeal teratomas diagnosed prenatally are seen in our office for a detailed discussion of the condition and treatment options. [surgery4children.com]
Prognosis
The diagnosis is frequently made prenatally although a delayed presentation, which adversely impacts prognosis, can be seen in patients with intra-abdominal lesions. Multiple factors make the management of SCT challenging for the pediatric surgeon. [pedsurglibrary.com]
Those presenting in older infants tend to have a higher malignant potential which those presenting in utero have a poor prognosis due to complications. Malignant change may be also commoner in males. [radiopaedia.org]
[…] factors were found to be independent predictors of a poor prognosis on multivariate analysis. [emedicine.medscape.com]
The prognosis in these cases depends on the type of problem that the baby has. When the baby is stable, a surgeon will do one or many surgeries to remove the mass. [isuog.org]
Prognosis is good but the prevalence of malignant cells increases with the child’s age. Therefore early diagnosis and resection is of utmost importance. [imagejournals.org]
Etiology
We present the intradural extension of SCT in a neonate, briefly review the currently proposed etiology regarding teratoma formation, and discuss the possible embryological basis causing this kind of teratoma. [thejns.org]
Though the exact etiology of most SCTs is unknown, the majority are thought to be sporadic. A few families with autosomal dominantly inherited presacral teratomas have been described in the literature, however. [obgyn.net]
Epidemiology
You are ( * ) If you have selected the Other category, please specify which type of user you are: * Email address: * Topic of your comment * Epidemiology data Summary and related texts Related genes Clinical signs Nomenclature and/or coding Your message [orpha.net]
This epidemiological parameter was verified in our study where both cases were reported in female subjects. Sacrococcygeal tumor can be discovered in three circumstances. [scitemed.com]
Descriptive epidemiology of teratoma in infants, Hawaii, 1986–2001. Paediatr Perinat Epidemiol. 2006;20: 54-58. Fraumeni JR, Li FP, Dalager N. Teratomas in children: epidemiologic features. J Natl Cancer Inst. 1973;1425-1430. [fetalcaredallas.com]
Prevention
A Cesarean section is recommended for babies with larger tumors to prevent dystocia and reduce the risk of tumor rupture, or bleeding. Each pregnancy is evaluated on an individual basis. [mottchildren.org]
Sometimes, maternal-fetal specialists determine that an early delivery is needed to address the tumor and prevent further danger to the heart. [rileychildrens.org]
This staged surgical process is used to prevent tumor rupture and hemorrhaging should the mother go into spontaneous labor. Treatment for sacrococcygeal teratomas at the Colorado Fetal Care Center has proven very successful. [childrenscolorado.org]
During surgery, precautions must be taken to prevent hypothermia, which is easily precipitated because of the large surface area and the vascularity of the tumour. 44 The treatment of choice for sacrococcygeal teratoma is early surgical resection with [pmj.bmj.com]
The fluid surrounds the internal organs, such as the heart, liver and lungs, and prevents them from working properly. [choc.org]