Decision-Making and Challenging Surgery in a Child with a Giant Tumor of the Sacro-Gluteal Region: A Humanitarian Aid Context
Oliver Renz*, Paul Hechenleitner, Gerd Wimmer, Murat Sanal, Beatrice Häussler
Department of Pediatric Surgery, University Hospital Innsbruck, Austria
*Corresponding author: Oliver Renz, Department of Pediatric Surgery, University Hospital Innsbruck, Austria. Tel: +4351250480810; Email: oliver.renz@tirol-kliniken.at
Received Date: 02 August, 2018; Accepted Date: 06 August, 2018; Published Date: 15 August, 2018
Citation: Renz O, Hechenleitner P, Wimmer G, Sanal M, Häussler B (2018) Decision-Making and Challenging Surgery in a Child with a Giant Tumour of the Sacro-Gluteal Region: A Humanitarian Aid Context. J Anaplast Reconstr Surg: JRSA-101. DOI: 10.29011/JRSA-101. 000001
1. Abstract
Sacrococcygeal teratoma (SCT) is a common germ cell tumor in infancy and early childhood. SCTs are generally benign tumors; however, malignant elements can be present, and their frequency increases with the age of the patient. In the rare case of a nine-year-old girl from Zimbabwe with a giant sacro-gluteal tumor, appropriate decision-making was a very demanding task, especially under the circumstances of humanitarian aid. A total resection of the tumor with reconstructive surgery of soft tissue was performed. Histology showed a mature Altman Type II teratoma consisting of fully differentiated tissues from somatic sites. Post-operative care included wound treatment and physiotherapy. Due to meticulous decision-making, this case of SCT was treated successfully.
2. Keywords: Childhood; Humanitarian
Aid; Sacrococcygeal Teratoma
1. Introduction
Mature fetiform and malignant sacrococcygeal teratomas are rare tumors presenting mainly in neonates. Primary sacral tumors such as chordomas, osteoblastomas, and sacrococcygeal teratomas, presenting as large external tumor masses, are very rare in young adults [1, 2]. In our case, a nine-year-old girl from Zimbabwe presenting with a huge SCT was treated at our Department of Pediatric Surgery.
2. Case Presentation
In December 2017, our Department of Pediatric Surgery received a request
for humanitarian aid from the local non-profit relief organisation, “Lovemore -
Tirol für Zimbabwe”, to provide treatment at our hospital for a nine-year-old
girl from Zimbabwe presenting with a huge mass in the gluteal region. There was
no detailed knowledge of her medical history available, and diagnostic findings
were limited to standard blood count, X-ray image of the pelvic region, one
ultrasound investigation, and two patient photographs (Figures
1 and 6).
Generously, the chief financial officer of our
hospital guaranteed coverage of all financial costs. Following admission to our
hospital, further preoperative assessment confirmed our assumption of benign SCT
by means of alpha-fetoprotein and beta-hCG tests, serum levels of which were
within the normal range. Ultrasound examination (duplex sonography) and an MRI
scan showed mature Altman Type II SCT. Neuro-urological assessment revealed
lower urinary tract dysfunction with post-void residual urine due to tumour
compression, but without coexisting bowel dysfunction. Accurate preoperative
planning was necessary to ensure the best possible functional and cosmetic
results of this complex surgical procedure, since errors in treatment can rarely
be corrected at a later date (Figure 2).
Surgery for the complete excision of the SCT,
including removal of the coccyx, was performed. During the operation, special
attention was paid to the conservation of both ureters in the pelvis and the nerve supply to the bladder and rectum, in addition to the protection of the
sphincter muscle innervation. The main afferent presacral tumour artery was
selectively ligated. Although the tumor was adherent to the surrounding tissue, all structures could be identified and separated without damage. Despite
the remaining large wound area, primary skin closure could be achieved. Following
reconstructive pelvic floor surgery, the remaining deep tumor cavity was closed
with muscle layers and a supply of soft tissue (Figures
3, 4, and 5).
3. Discussion
Overall, the key ethical question in humanitarian relief projects remains: do we do more harm than good? A major invasive surgical procedure should offer the best chance of a cure; however, due to the risk of possible postoperative complications such as wound healing problems, neurological deficits, and urinary and fecal incontinence, which may not be treated sufficiently, in addition to the need for urgent or long-term follow-ups, this is not usually feasible in low-income developing countries. Under these circumstances, we worked out three essential key criteria for decision-making, and finally accepted the major surgical challenge.
5.1. First key criterion
There was the challenging task of ensuring the correct differential diagnosis based on poor previous diagnostic findings. According to the report, the tumour had been present since birth, with a slow growth rate, and a recent photograph showed the child to be in good overall health, both of which were encouraging. On the other hand, an increase in tumour mass implied certain risks for the patient. However, as tumor size increases over time, so does the risk of malignant transformation, and the subsequent development of metastatic disease [3, 4]. Therefore, complete surgical resection was the cornerstone of management in this setting, even though surgical resection is often an extensive procedure and can cause significant acute and long-term side effects [5, 6−10]. Taking into account all available information for the assessment, we diagnosed a mature sacrococcygeal teratoma. Finally, a histological examination confirmed a mature teratoma consisting of fully differentiated tissues from various somatic sites, with complete tumour resection in healthy tissue.
5.2. Second key criterion
A crucial factor for decision-making was that the tumor had already
reached a critical size. It was expected to be only a matter of time until the
tumor penetrated through the skin, which would most likely be followed by
purulent infection with septicemia and possible fatal consequences (Figure 6).
Another important aspect was that without treatment further tumor growth
would cause significant neurological malfunction and serious incontinence
problems [7]. Postoperative neuro-urological examination showed normal neurological
bladder function without residual urine, and there were
no postoperative bowel dysfunction problems such as incontinence or fecal
soiling. During the first postoperative week, we observed good wound healing
without any acute infection (Figure 7).
However, a spontaneous perforated and
emptying seroma hollow space in the wound area subsequently led to a delayed
healing process. This was followed by open phase-specific wound treatment during inpatient
stay, which was completed as outpatient care (Figure 8).
5.3. Third key criterion
An important decision guidance for us was the quality of life aspect.
The girl’s father had left her mother and older siblings shortly after her
birth, since disability is still considered a disgrace and cruse in Zimbabwe.
Subsequently, poverty became worse for the family. Growing up, the girl had
been increasingly socially marginalised in many different ways; and due to the
increasing tumor size, the simplest of movements and activities such as
sitting, running, and getting dressed had become more burdensome. For her, an
extraordinary girl indeed, a new life with a completely new body image is just
beginning (Figure 9).
6. Conclusion
In general, it can be said that, in pediatric surgery, there is a fine
line between doing harm and good; however, the situation is particularly
aggravated under the circumstances of humanitarian aid in patients from
low-income developing countries. For this reason, conscientious and careful
decision-making is a precondition, and more than any overblown surgical
ambition, first and foremost, is a mandatory ethical and moral obligation to
medical care.
Figure 1: The first photo of our patient from Zimbabwe.
Figure 2: With the patient in the prone position, the pre-operative tumor size is
exposed.
Figure 3: Intraoperative imaging.
Figure 4: Intraoperative imaging.
Figure 5: The removed tumor, with a size of 20 x 19 x 15 cm and a weight of 3 kg.
Figure 6: The second photo of our patient from Zimbabwe showing the risk of a
pressure ulcer (arrow).
Figure 7: One week after the surgical intervention.
Figure 8: Finally, the healing process led to a pleasing scar, with an
excellent cosmetic result.
Figure 9: Farewell photo prior to the patient returning home (with the consent of
all persons for publication).
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