[SUBTOTAL AGENESIS OF THE SACRUM ASSOCIATED WITH ENURESIS AND ENCOPRESIS].
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The anorectal function in 3 patients with bilateral and 4 patients with unilateral well-defined loss of sacral nerves after radical tumour excision was studied by clinical examination and by simultaneous registration of the following variables: volume and pressure in the rectum, pressure in the internal anal sphincter area and myoelectrical activity in the external anal sphincter. The patients with bilateral loss of sacral nerves had serious impairment of function. Constipation was their only safeguard against incontinence. The preservation of the first and second sacral nerves bilaterally was not sufficient for discrimination between different qualities of rectal contents passing the anal canal. The sensation of rectal distension was also impaired. The reflex pattern of the internal anal sphincter was, however, intact. The external anal sphincter displayed a weak spontaneous myoelectrical activity in the patients who had at least one second sacral nerve intact, and a weak increase of the activity could be induced voluntarily. The normal transient increase of myoelectrical discharge from the external anal sphincter in response to rectal distension could not, however, be elicited. In patients with total unilateral loss of the sacral nerves no significant impairment of anorectal function was noted. Total one-sided denervation implied deficient sensibility of the anal canal unilaterally, but no disturbance of sphincter function as judged from the reflex response of the internal and external anal sphincters to rectal distension.
We report on the crush preparation findings of a case of "sarcomatoid" chordoma occurring in the sacral region of a 78-yr-old Chinese male. The smears showed clumps and small cords of polygonal tumor cells containing bubbly cytoplasm and round to oval nuclei. Focally, there were also aggregates of long filamentous spindle cells and stellate bizarre cells with marked nuclear pleomorphism. Occasional tumor cells were seen in association with dense amorphous material. Histologic examination of the excised specimen showed features of the so-called "sarcomatoid" chordoma which consisted of prominent foci of mitotically inactive spindle and pleomorphic cells, in addition to the conventional chordoma areas. An osteosarcoma-like pattern of probably metaplastic nature was also seen within the tumor. Immunohistochemical study showed that most tumor cells expressed cytokeratins. Ultrastructural examination revealed the characteristic rough endoplasmic reticulum-mitochondria complexes. While there are many spindle and pleomorphic cells seen in crush preparations, the distinction from other true high-grade malignancies is important. Recognition of these "pseudoanaplastic" cytologic features also helps to expand the morphologic spectrum of chordoma.
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Sacral neoplasms constitute a wide range of pathological entities including primary and metastatic as well as benign and malignant conditions. Often these lesions are large at the time of initial diagnosis and surgical cure may be difficult. Nonetheless, surgery may be indicated for a wide range of reasons including tissue diagnosis, palliation of pain, preservation of neurological function, or attempts for curative resection. There are numerous surgical approaches to lesions of this area which require a complete understanding of the neural, pelvic, and bony anatomy. For this reason we utilize a multidisciplinary team approach when treating these lesions. This allows for the combination of expertise from areas such as general surgery, orthopedic surgery, and neurosurgery that optimizes the treatment of these patients. In this article we review the basic techniques of diagnosis and treatment of these lesions. This overview includes the relative anatomy, symptoms, diagnosis, imaging, operative indications, surgical approaches, and potential complications.
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A series of five cases of sacral aneurysmal bone cyst is presented. There were three females and two males, the youngest patient being 11 years old, the oldest 41 years old. All patients had pain of long duration with varying signs of nerve root compression. Three patients had micturitional problems. Four patients had a palpable mass which was tender. All had standard radiographs. Tomograms, computed tomography (CT) scans or MRI (magnetic resonance imaging) were obtained in some patients. In all patients a definite histologic diagnosis was established. Four lesions healed after resection (two cases) or curettage (two cases). One patient is considered to have a stable lesion which is not progressing.
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