A six yr. old female presents with constipation and urinary retention . On examination a presacral mass is noted. Most probable diagnosis is:
Correct Answer: Anterior sacral meningocele
Description: Ans is d i.e Anterior sacral meningocele Well all 4 options can present as presacral mass and any large pelvic mass can cause constipation and urinary retention due to mass effect. All the above lesions are rare, so we go for the most probable choice i.e. Ant. Sacral meningocele. It appears to be the most common of all, at the given age. Some books/students would argue it to be sacrococcygeal teratoma. But points against it are: Its common age of presentation is in the newborn and infants. Most of the sacrococcygeal teratomas have external component, rarely it is entirely pelvic. Anterior sacral meningocele Anterior sacral meningocele is characterised by a defect of the anterior aspect of the sacrum involving one or more segments with herniation of the meningeal sac into the extra peritoneal pelvic region. It is more common in females than in males. The symptoms appear usually in the second to third decade of life. The symptoms of anterior sacral meningocele are primarily owing to the mechanical effects of a pelvic tumor. Occasionally minor nerve defects may be associated with the condition. - Abdominal pains, constipation, urogenital (urinary retention or incontinence), gynecological (dysmenorrhea, dyspareunia) and neurological (low back pain, radiation to the legs and meningitis) symptoms may be seen. On x-ray: A unilateral sickle shape distoion of the sacral bone - The " Scimitar sacrum" - is considered pathognomonic. This sign is not present in all cases. Sacrococcygeal Teratoma Teratomas are tumors comprising more than a single cell type derived from more than one germ layer Teratomas range from benign, well-differentiated (mature) cystic lesions to those that are solid and malignant (immature). The most common location is sacrococcygeal (57%). Sacrococcygeal teratomas are the most common tumors in newborns. Most SCTs are found in babies and children, but SCTs have been repoed in adults. The tumors are subdivided and classified according to their location: Type I - tumors predominantly external with only minimal presacral involvement; Type II - tumors presenting externally but with a significant intrapelvic extension; Type III - tumors apparent externally but with predominant pelvic mass and extending into abdomen; Type IV - Presacral with no external presentation . A sacrococcygeal teratoma can he distinguished from its primary differential meningo-cele by its more abundant internal soft tissue, whereas the meningocele is more completely cystic in nature. Rectal duplication cyst Gastrointestinal duplications are rare congenital malformations that may vary greatly in presentation, size, location, and symptoms. The small intestine is the most frequent site involved(about 44%). Rectal duplication cysts are the rarest form of duplication comprising less than 5% of all gastrointestinal duplications. Presentation depends on the size and location of the duplication. Presenting signs of colonic or presacral duplications may include constipation, rectal bleeding, hematochezia, rectal prolapse, hemorrhoids, fistula-in-ano, and perirectal abscess. Classification of retrorectal/presacral masses a. Congenital d.Osseous Developmental cysts Osteoma Epidermoid cyst Osteogenic sarcoma Dermoid cyst Sacral bone cyst Teratoma Ewing's tumor Chordoma Giant cell tumor Anterior meningocele Chondromyxosarcoma Rectal duplication e.Miscellaneous Adrenal rest tumors Metastatic disease b.Inflammatory Lymphangioma Granulomas Desmoid tumor Perineal abscess Leiomyoma Pelvirectal abscess Fibrosarcoma Fistula Endothelioma Chorionic granulomas c.Neurogenic Neurofibroma Neurolemmoma Ependymoma Ganglioneuroma Neurofibrosacroma
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