A 13-month-old girl presented to the emergency department with a history of intermittent constipation beginning at approximately age 9 months. The day before presentation, the child had one small bowel movement followed by intermittent straining and crying. She had no systemic symptoms.
The medical history was unremarkable. On physical examination, the child had a distended abdomen with normal bowel sounds and palpable stool. There was an anterior anal fissure. On rectal examination, the tone was normal and no stool was detected in the rectal vault.
An abdominal radiograph (Figure 1) was performed. Due to the presence of a large amount of fecal material in the proximal colon and the absence of air or fecal material in the rectum, a barium enema examination (Figure 2, see page 681) and abdominal and pelvic computerized tomography (CT) were performed (Figure 3, see page 681).
Figure 1. Supine abdominal radiograph reveals a large amount of fecal material in the proximal colon and a paucity of air or fecal material in the rectum.
Figure 2 (above). Lateral view from barium enema reveals anterior displacement of the rectosigmoid colon by a presacral soft tissue mass (arrows).
Figure 3 (right). Contrast enhanced CT scan through the pelvis reveals a rounded presacral soft tissue mass with scattered foci of calcification. Significant displacement of the rectosigmoid colon (arrows) is observed.
Constipation is a frequent complaint in children. Possible etiologies vary with age, but include functional retention or psychogenic retention, dietary factors, Hirschsprung's disease, segmental dilatation of the colon, occult spinal dysraphism, post-surgical stenosis, hypothyroidism, and an obstructing presacral mass lesion.1 Masses that may arise in the presacral region leading to constipation include neuroblastoma, sacrococcygeal teratoma, anterior meningocele, lipoma, and rectal duplication.2
In a child with prolonged unexplained constipation, appropriate imaging studies include an abdominal film to assess for fecal impaction, spinal dysraphism, or possible mass, and a barium enema to evaluate for transition zone due to Hirschsprung's disease, stenosis, or mass effect on the bowel. The differential diagnosis of this child's mass was neuroblastoma and sacrococcygeal teratoma; its solid appearance was not compatible with meningocele or rectal duplication cyst. This presacral tumor was proven pathologically to be a neuroblastoma.
Neuroblastoma is the third most common malignancy of childhood.3,4 The most frequent site of origin is abdominal sympathetic nervous tissue in the adrenal medulla or paraspinal ganglia.5 The pelvis is an unusual site of origin, occurring in only 4% to 6% of cases.6-8 Children with pelvic neuroblastomas most frequently present with a palpable abdominal mass and symptoms of urinary retention, constipation, or both.6,7
The behavior of neuroblastoma varies dramatically with some tumors demonstrating spontaneous remission while others have rapidly progressive metastatic disease.8 Stage of disease and age at diagnosis are the most important prognostic factors for survival.8 Several reports have described a more favorable outcome in patients with pelvic neuroblastoma independent of tumor stage.6,7
The pelvic location of the tumor can lead to a high incidence of morbidity due to treatment In one series,6 35% of patients with surgically resected pelvic neuroblastoma developed post-operative neurologic complications, including leg weakness, sciatic nerve palsy, and fecal incontinence with a neurologic bladder.
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