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Manoover Management of Sacrococcygeal Teratoma In A Very Low Birthweight Extreme Premature Infant: A Case Report

C. Holbrook, T. Tsang, J. Clibbon, A. B. Mathur

Department of Paediatric Surgery, Norfolk and Norwich Hospital, Norwich, UK

 

Correspondence

C Holbrook

Department of Paediatric Surgery

Norfolk and Norwich Hospital

Norwich NR47UY, UK

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Abstract

Sacrococcygeal teratoma diagnosed in utero is associated with a high risk of mortality and morbidity. Preterm birth is commonly associated with the condition. The management and outcomes of such infants are well described. However, in all cases, the birthweight has been greater than 1kg. We describe the successful management and outcome of an infant born at 26 + 2 weeks with very low birthweight (950g).

Keywords: sacrococcygeal teratoma, prematurity, low birthweight

 

Introduction

Sacrococcygeal teratoma (SCT) is the most common type of neonatal tumour, with a reported birth prevalence of 1 in 27000 live births [1]. Several papers in the literature describe the management and outcome of SCT in preterm infants with birthweight >1000g. In contrast, our case report describes a neonate with very low birth weight (950g) who had a SCT that was successfully excised.

Case report

SCT was diagnosed antenatally at 17 weeks gestation. Ultrasound showed a mass at the base of the spine, but not involving the spine itself. Between 19 and 25 weeks, the SCT increased in size to 10 cm in diameter and there was increasing polyhydramnios. At this stage, with no features of fetal hydrops or maternal mirror syndrome, no intervention was undertaken. A further scan was scheduled for the following week in consideration of in-utero ablation of the sacral ‘feeding’ vessels to prevent any further cardiovascular compromise. This did not take place as there was spontaneous onset of labour at 26 + 2 weeks gestation.

Delivery was by emergency caesarian section. Birth weight was 950g. The (female) infant was born with a heart rate of > 100, some shallow respirations and some active movements, and was subsequently intubated and ventilated. During delivery there was rupture of the sacral tumor with an estimated blood loss of 8-10 mls. A large vein was clamped and covered with sterile gauze. A postnatal ultrasound scan confirmed no extension of the tumour into the abdomen (Fig. 1).

Figure 1: Appearance of sacrococcygeal teratoma at time of surgery including image of excised tumour

8.4-7.1

Surgery took place at 7 hours of age. A chevron incision was made over the lesion with the apex of the incision at the sacrococcygeal junction. The upper flap was raised to reach the sacrococcygeal junction and the coccyx was excised. Medial sacral vessels were ligated and cut. Dissection was carried laterally through a plane between the tumur and the muscles. A tube was passed into the rectum to identify it, allowing the tumor to be dissected free from this structure. Following total excision of the SCT, the muscle and fascia were approximated, and the skin was closed over a small suction drain (Fig. 2).

Figure 2: Initial post operative appearance following excision of sacrococcygeal teratoma and closure of wound

8.4-7.2

Towards the end of the procedure, the infant developed bradycardia and hypotension, but was successfully resuscitated and returned to the neonatal unit, remaining intubated for 10 days. Antibiotics were given prior to the procedure and continued postoperatively for 7 days. The excised tumour was a mass measuring 10 x 9 x 4cm weighing 325g. Histology showed that the tumor contained the following tissues: cartilage, bone, glandular epithelium, blood vessels, smooth muscle, hepatic and pancreatic tissue, squamous epithelium and primitive neural tissue. A benign course was predicted as neither yolk sac tumour nor embryonal carcinoma were seen (these two features tend to be associated with malignant behaviour).

The infant made a good recovery and was discharged home on day 79 (corrected gestation 37 + 4). A developmental check at 23 months (corrected) was appropriate for age, with her height and weight following the 25th centile. Further follow up confirmed that she had full continence and mobility. At birth, her alpha-fetoprotein level was 142.000 kU/L. This level fell following excision and continued to fall following discharge. It remained at an undetectable level (< 0.1 kU/L) during six years of follow up. It was noted at the time of surgery that revision of the surgical scar would be required at a later date, due to the size of the tumor. This proved to be the case; as the infant grew, the scar appeared unsightly with the skin tethered on the left buttock. This scar was revised by plastic surgeons when the child was 3 years of age (Fig 3a and 3b).

Figure 3a and 3b: Child at age of 3 years showing the appearances of the scar prior to revision surgery

8.4-7.3a 8.4-7.3b

Discussion

Our case shows a favorable outcome in an infant with sacrococcygeal teratoma, despite prematurity and a very low birthweight. A number of case series describe the management and outcomes of infants born at a similar gestation, but birthweight has always been >1000g (Table 1), [1, 2, 3, 4, 5, 6, 7]. In our patient, birthweight was 950g, which becomes noteworthy when the size of the tumour is considered. The excised weight was 325 g, so 34% of the baby’s bodyweight was tumour.

Table 1: Case series describing the management and outcomes of infants born with sacrococcygeal teratoma

 

No.of cases

 

Gestation range/mean (weeks)

 

Birthweight range/

mean(grams)

Swamy[1]

16

32-43

2080-4400

Makin[2]

29

26-40

1200-4600

Gabra[3]

33

30-41

1500-4900

Usui [4]

83

26-41

1020-5014

Murphy [5]

12

27-41

1500-4400

Kamata [6]

13

31+3-38+2

2449-3731

Derikx [7]

173

35-40+6

2500-3900

SCT diagnosed in the neonatal period is associated with a good outcome and low overall mortality. In contrast, in cases such as ours, when SCT is diagnosed in utero (usually incidentally on screening US), mortality is high (reported at 25% in a large study of 44 cases [8]). Preterm birth is also common, with rates reported at 44.6% [8]. Mortality is associated with the onset of high output cardiac failure; SCTs are highly vascular and contain haemodynamically significant arteriovenous fistulae [9], placing excessive demands on the systemic circulation. Polyhydramnios, placentomegaly and foetal hydrops can develop, and are associated with a poor prognosis [10]. Maternal health can also be impaired if there is development of maternal ‘mirror’ syndrome, a preeclamptic condition precipitated by development of hydrops in the foetus [8, 11].

In our case, polyhydramnios did develop, but preterm delivery occurred before any further impairment in cardiac function could be seen, and the infant was born in relatively good condition. Delivery was by caesarean section; this is strongly recommended where the tumor is large (> 5cm) to avoid complications such as dystocia and tumor rupture [5]. We experienced a major complication at the time of delivery with rupture of the tumor. It was fortunate that we were able to gain control of this by clamping a large vessel. The tumor represented a significant proportion of the patient’s bodyweight, so haemorrhage would have resulted in a considerable loss of total blood volume. Mortality due to exsanguination has been reported, at delivery and also in the preoperative period [2, 4, 5, 7]. Spontaneous rupture in utero has also been reported [12]. Haemorrhage is a recognised complication of surgery [3, 4, 6, 7, 13], so early intraoperative vascular control is very important. SCT can be excised via a perineal approach, but in cases where the SCT is large and highly vascular, control of the aorta and/or ligation of the middle sacral artery via a transabdominal approach has been advocated [6].

Laparotomy can be performed, but laparoscopy has also been successfully used both to gain control of the vessels and assist with dissection of any intraabdominal component [7,14]. Staged resection can also be used, with initial devascularisation of the tumor, then subsequent later resection following correction of the hyperdynamic state [15]. Preoperative embolization has also been used [16]. In our case, there was no intraabdominal component and we excised the tumor with the perineal approach. This was successful, as though the tumor was large, it was not highly vascular. Even when vascular control is good, bleeding can be a problem due to associated coagulopathy. This may partly result from bleeding leading to consumption of clotting factors, but the aetiology is probably multifactorial. It has been suggested that the tumor may contain defective endothelium which can induce the development of disseminated intravascular coagulation, particularly if there disruption of the endothelium due to trauma during delivery [5]. In addition, congestive heart failure caused by vascular steal in large SCTs may lead to stasis and formation of clots, with consumption of clotting factors and initiation of local fibrinolysis, leading to DIC [5].

Prompt surgical intervention has therefore been recommended to prevent worsening of coagulopathy over time [5, 6]. Our infant required blood transfusion and FFP in the postoperative period, but did not show evidence of progression to DIC as the clotting profile was not deranged. Long term outcomes in this patient were also good. The tumor was excised en bloc along with the coccyx, which is recommended to avoid recurrence [3]. However, even benign teratomas have a significant recurrence rate and close follow-up with monitoring of AFP for at least 3 years is recommended [7, 13]. Our patient was followed up for over 6 years, and levels of AFP remained undetectable. Faecal incontinence, constipation, and urinary incontinence are all reported as long term sequelae of SCT [3, 13, 17, 18]; our patient did not suffer any of these. This may be related to the fact that there was no intrapelvic extension, which is associated with a higher incidence of functional problems [17]. One study of long term outcomes revealed more than 40% of patients reported their scar to be subjectively unacceptable cosmetically, particularly when there was history of an especially large tumor or diagnosis at a young age [18]. In our case, the tumor was proportionally large in relation to the patient and the scar became unsightly in appearance. Fishman et al. developed a closure technique that allows reconstruction of the buttocks with a normal contour and hidden scars [19]. Our opportunity to optimize the cosmetic appearance during wound closure may well have been hindered by the sudden deterioration of the patient towards the end of the procedure. Subsequent revision of the scar took place when the child was older, so ultimately, a good result was achieved.

Conclusion

In patients with SCT, careful monitoring and planning can improve outcomes. Surveillance in utero will allow detection of cardiac compromise and fetal intervention may be considered. Early delivery may be planned, requiring collaboration between obstetricians, neonatologists and paediatric surgeons. Since SCT is often associated with preterm birth, this sort of planning may not be possible, as in our case. When dealing with this kind of emergency, we believe that an awareness of the problems associated with SCT within a multidisciplinary team can allow a satisfactory outcome to be achieved. Close attention should be paid to suggestion of tumor rupture at delivery as this can lead to life threatening exsanguination. It may also precipitate DIC, so clotting abnormalities should be looked for and corrected prior to surgery.

US is useful prior to surgery to assess for abdominal extension. Where the tumor is large in proportion to the patient size, the excision may result in a significant loss of blood volume in the patient, precipitating hypovolaemic shock, so the anaesthetic team should be well briefed and blood products should be available for the operation. Although it is important that the patient is stabilized prior to surgery, it may not be prudent to delay too long, due to the risk of developing DIC and high output cardiac failure.

 

 

 

References

1.Swamy R, Embleton N, Hale J. Sacrococcygeal teratoma over two decades: birth prevalence, prenatal diagnosis and clinical outcomes. Prenat Diagn 2008; 28(11):1048-51.

2.Makin E, Hyett J, Ade-Ajayi N, Patel S, Nicolaides K, Davenport M. Outcome of antenatally diagnosed sacrococcygeal teratomas: single centre experience (1993-2004). J Pediatr Surg 2006; 41:388-393.

3.Gabra H, Jesudason E, McDowell H, Pizer B, Losty P. Sacrococcygeal teratoma – a 25-year experience in a UK regional centre. J Pediatr Surg 2006; 41:1513-1516.

4.Usui N, Kitano Y, Sago H, Kanamori Y, Yoneda A, Nakamura T, et al. Outcomes of prenatally diagnosed sacrococcygeal teratomas: the results of a Japanese nationwide survey. J Pediatr Surg 2012;47(3):441-7.

5.Murphy J, Blair G, Fraser G. Coagulopathy associated with large sacrococcygeal teratomas. J Pediatr Surg 1992; 27(10):1308-1310.

6.Kamata S, Imura K, Kubota A, Sawai T, Nose K, Hasegawa T, et al. Operative management for sacrococcygeal teratoma diagnosed in utero. J Pediatr Surg 2001; 36(4):545-548.

7.Derikx J, De Backer A, De Schoot, et al. Long term functional sequelae of sacrococcygeal teratoma: a national study in the Netherlands. J Pediatr Surg 2007; 42:1122-1126.

8.Benachi A, Durin L, Maurer S, Aubry M, Parat S, Herlicoviez M, et al. Prenatally diagnosed sacroccygeal teratoma: a prognostic classification. J Pediatr Surg 2006; 41:1517-1521.

9.Calenda E, Bachy B, Guyard HF. Sacroccygeal teratoma and venous shunting through a tumour: biologic evidence. Anaesth Analg 1992; 74:165-166.

10.Bond S, Harrison M, Schmidt K, Silverman N, Flake A, Slotnick N, et al. Death due to high output cardiac failure in fetal sacrococcygeal teratoma. J Pediatr Surg 1990; 25(12): 1287-1291.

11.Finamore P, Kontopoulos E, Price M, Giannina G, Smulian JC. Mirror syndrome associated with sacrococcygeal teratoma: a case report. J Reprod Med 2007; 52(3):225-227.

12.Sy E, Lee H, Ball R, Farrell J, Poder L, Nobuhara K, et al. Spontaneous rupture of fetal sacrococcygeal teratoma. Fetal Diagn Ther 2006; 21:424-427.

13.Huddart S, Mann J, Robinson K, Raafat F, Imeson J, Gornall P, et al. Sacrococcygeal teratomas: the UK Children’s Cancer Study Group’s experience. Neonatal. Pediatr Surg Int 2003; 19:47-51.

14.Lee KH, Tam YH, Chan KW, Cheung ST, Sihoe J, Yeung CK. Laparoscopic-assisted excision of sacrococcygeal teratoma in children. J Laparoendosc Adv Surg Tech A. 2008; 18(2):296-301.

15.Robertson F, Crombleholme T, Franz I, Shephard B, Bianchi D, D’Alton M. Devascularization and staged resection of giant sacrococcygeal teratoma in the premature infant. J Pediatr Surg 1995; 30(2): 309-311.

16.Lahdes-Vasama TT, Korhonen PH, Seppänen JM, Tammela OK, Iber T. Preoperative embolization of giant sacrococcygeal teratoma in a premature newborn. J Pediatr Surg 2011; 46(1):e5-8.

17.Malone PS, Spitz L, Kiely EM, Brereton R, Duffy P, Ransley P. The functional sequelae of sacrococcygeal teratoma. J Pediatr Surg 1990; 25(6):679-80.

18.Derikx J, De Backer A, Schoot L van de, Aronson D, Langen Z de, Hoonaard T van den, et al. Factors associated with recurrence and metastatic disease in sacrococcygeal teratoma: results in the Netherlands. Br J Surg 2006; 93:1543-1548.

19.Fishman SJ, Jennings RW, Johnson SM, Kim HB. Contouring buttock reconstruction after sacrococcygeal teratoma resection. J Pediatr Surg. 2004; 39(3):439-41.