Hirschsprung's disease is one of the most common causes of intestinal obstruction in neonates and infants. The underlying pathology of this disease is the absence of the ganglion cells in both the myenteric (Auerbach's) plexus and the submucosal (Meissner's) plexus. Since Hirschsprung's report in 1886, there have been thousands of papers on Hirschsprung's disease but the cause of the absence of the ganglion cells has not been identified. Hirschsprung's disease can be successfully treated with the Swenson, the Duhamel, and the Soave operations even though the pathogenesis is unknown. With the recent progress of molecular biology and genetics, a more detailed approach to the pathogenesis of Hirschsprung's disease can be undertaken. In addition, there have been recent developments in the surgical approach. In this review, recent advances in surgery for Hirschsprung's disease are presented.
Ganglion Cysts ; Genetics ; Hirschsprung Disease* ; Humans ; Infant ; Infant, Newborn ; Intestinal Obstruction ; Molecular Biology ; Pathology

Tailgut cysts are rare congenital lesions thought to arise from vestiges of the tailgut/postanal gut, which is a primitive gut temporarily present at the most caudal portion of the embryo. The lesions are usually multilocular cysts lined by various types of epithelium in the retrorectal space. We experienced a tailgut cyst in an 18-day-old girl initially presented as an epidermoid cyst like skin lesion in the coccygeal region. MRI showed a well-defined multiloculated cystic mass between the rectum and coccyx, measuring 12x23 mm and markedly hyperintense on T2 weighted images and hypointense on T1-weighted images. The cyst was surgically removed via a posterior sagittal approach and microscopically lined by ciliated columnar, transitional and squamous cells. Tailgut cysts are usually found in adult females, and extremely rarely in neonates. We report a tailgut cyst in a neonate with a review of the literature.
Adult ; Coccyx ; Embryonic Structures ; Epidermal Cyst ; Epithelium ; Female ; Gastrula ; Humans ; Infant, Newborn ; Rectum ; Sacrococcygeal Region ; Skin

No abstract available.
Dermatomycoses* ; Humans

Osteoporosis is defined as a pathologic condition that there occurs no change in the chemical composition of the bone, while bone resorption is abnormally increased, compared with osteogenesis, leading to a decrease in bone amount. Though many varieties of therapy have been tried, no definitely effective method has been found. Ginseng saponin is an importanat component of Korean ginseng and many studies have been performed for investigating its effectiveness. But there has been no report on saponin effects to osteoporosis. The purpose of this studies is to find out the effect of saponin in osteoporosis induced by ovariectomy in rats. For the study, 30 rats underwent sham operation(group I) and 90 rats were ovariectomized. In group ll (30 rats), only ovariectomy was performed. In group III (30 rats), 50mg/kg/day ginseng saponin was administered per oral route and group IV (30 rats) was administrated 150mg/kg/day saponin. All the rats were sacrificed at the 16th week. The sections of the femoral shaft were stained using Villanueva bone stain and the sections of the tibia were stained with hematoxylin-eosin. The femoral shaft and metaphyseal region were applied to compression device for measurements of bone consistency. The effect of saponin increased weight of the femur. The cross sectional area and cortical area of the femoral shaft were significantly higher in 50mg saponin group(group III) than osteoporosis group. But there was no significant difference in the size and number of osteoclast and the percentage of trabecular bone between saponin group(group III ,IV)and osteoporosis group. In the biomechanical test, the consistency of cortical bone was significantly increased in 50mg saponin group than osteoporosis group. In conclusion, saponin had an effect on experimental osteoporosis induced by ovariectomy in rats. As though its precise mechanism was unknown, we considered that its action mechanism was due to osteoblastic activity resulting in a correction of bone mass deficit in osteoporotic rats.
Animals ; Bone Resorption ; Female ; Femur ; Methods ; Osteoblasts ; Osteoclasts ; Osteogenesis ; Osteoporosis ; Ovariectomy ; Panax ; Rats ; Saponins ; Tibia

Angiomatoid fibrous histiocytoma is a rare soft tissue tumor that generally affects children and young adults. We report a case of angiomatoid fibrous histiocytoma in an 11-year-old boy who complained of a back mass for 3 years. Surgical excision was performed. The excised specimen showed a 4.0 x 3.6 x 3.0 cm, well circumscribed, grayish white tumor, with multicystic changes. Histological examination showed proliferation of spindle or round shaped tumor cells. There was a dense fibrous pseudocapsule with prominent chronic inflammatory cell infiltrates.
Child ; Histiocytoma, Benign Fibrous* ; Humans ; Immunohistochemistry ; Male ; Young Adult

Multilocular cyst of the kidney is a rare pathologic entity, noninherited benign renal neoplasm occurring in the both children and adults. The entities commonly were known as multilocular cyst of the kidney or multilocular cystic nephroma. The controversy over the pathogenesis of multilocular cyst-neoplasm versus developmental anomaly- is still unsettled. We report two cases of multilocular renal cyst, one in a 8-year-old child with a chief complaint of gross hematuria and another in a 44-year-old woman with epigastric discomfortness. They were preoperatively suspected multilocular cyst by ultrasonography and computed tomography. We performed a simple nephrectomy as the treatment of choice because of the difficulty in accurate diagnosis and the documented association with renal neoplasia.
Adult ; Child ; Diagnosis ; Female ; Hematuria ; Humans ; Kidney ; Kidney Neoplasms ; Nephrectomy ; Ultrasonography

PURPOSE: To evaluate CT findings of normal anatomic alteration after neck dissection. MATERIALS AND METHODS: The postoperative CT findings were retrospectively reviewed in 40 patients with neck dissection, comparing to preoperative CT. There were 28 patients with radical neck dissection and 12 patients with modified radical neck dissection or selective neck dissection. In addition to the neck dissection, 10 patients had undergone pectoralis major myocutaneous flap reconstruction, 20 patients taken laryngectomy, and 25 patients treated with radiotherapy. RESULTS: The typical CT findings of radical neck dissection were non-visualization of internal jugular vein and sternocleidomastoid muscle(28/28), ipsilateral neck flattening(26/28), and trapezius muscular atrophy(12/28). The other non-specific findings were tissue plane eftacement, subcutaneous reticular pattern, platysma muscle thickening, and adjacent soft tissue contrast enhancement which was the only evidence of previous operation in the cases of modified radical neck dissection or selective neck dissection. Reconstruction with myocutaneous flap was shown as ipsilateral bulk of fat and muscle(10/10). In cases with radiation therapy there was much increased density of submandibular gland(12/25). 15 patients showed recurrence on follow up CT scan, including regional metastasis in 10, stomal recurrence in two, and residual mass at primary site in three patients. CONCLUSION: CT is useful in evaluation of patients with neck dissection and in whom postoperative change impese diagnostic problem.
Follow-Up Studies ; Humans ; Jugular Veins ; Laryngectomy ; Myocutaneous Flap ; Neck Dissection* ; Neck* ; Neoplasm Metastasis ; Radiotherapy ; Recurrence ; Retrospective Studies ; Superficial Back Muscles ; Tomography, X-Ray Computed

A malignant granular cell tumor (MGCT) occurred in the left shoulder of a 62-year-old man. The patient underwent wide marginal excision followed by chemotherapy and radiotherapy. A metastatic tumor was identified in the axillary lymph node 22 months after the excision of the shoulder mass. The primary tumor was a poorly circumscribed mass measuring 5 5 4 cm. On cut section, it was a solid mass with yellowish tan color. Histologically, both primary and metastatic tumor consisted of polygonal cells with abundant granular cytoplasm and a vesicular nucleus with a prominent nucleolus. Two to three mitotic figures per ten high power fields at 200 were counted. Tumor cells were weakly stained with periodic acid-Schiff (PAS) preparation both before and after diastase digestion, and were positive for S-100 protein, neuron-specific enolase (NSE), and vimentin. By electron microscopy, the cytoplasm was filled with numerous autophagolysosomes containing myelin figures, mitochondria, and fragmented rough endoplasmic reticula. Basal laminae and angulated bodies were also noted. These findings suggest schwannian differentiation of this tumor.
Amylases ; Basement Membrane ; Cytoplasm ; Digestion ; Drug Therapy ; Granular Cell Tumor* ; Humans ; Immunohistochemistry ; Lymph Nodes ; Microscopy, Electron ; Middle Aged ; Mitochondria ; Myelin Sheath ; Phosphopyruvate Hydratase ; Radiotherapy ; S100 Proteins ; Shoulder* ; Triacetoneamine-N-Oxyl ; Vimentin

PURPOSE: Transanal one-stage Soave (TOS) procedure is the most recently reported procedure for Hirschsprung's disease. This study was performed to compare the surgical outcomes of TOS with those of modified Duhamel procedure. METHODS: The study populations were 17 consecutive patients who underwent TOS procedure between March, 2003 and February, 2008 (TOS group) and 19 consecutive patients who underwent modified Duhamel procedure between March, 1996 and February, 2001 (Duhamel group) by one pediatric surgeon. Age, gender, length of aganglionic segment, operating time, complications, duration of postoperative hospital stay, and functional results were retrospectively analyzed and compared between the two groups. RESULTS: There was no difference in age, gender, and length of the aganglionic segment between the two groups. The operating time (195 versus 255 minutes, P<0.05) and the duration of postoperative hospital stay (7 versus 9 days, P<0.05) were significantly shorter in the TOS group. Postoperative complications occurred in 10 of 17 in the TOS group (4 perianal excoriation, 3 anastomotic stenosis, and 3 postoperative enterocolitis) and 10 of 19 in Duhamel group (2 wound infection, 1 perianal excoriation, 1 anastomotic leakage, 1 rectal bleeding, 2 intestinal obstruction, and 3 postoperative enterocolitis). Two cases in the Duhamel group required operation to treat postoperative complication, one for anastomotic leakage and one for intestinal obstruction. There was no significant difference in the functional results between both groups. CONCLUSION: TOS shows similar functional results and postoperative complications but has the advantage of a significantly shorter operating time and postoperative hospital stay compared with modified Duhamel procedure.
Anastomotic Leak ; Constriction, Pathologic ; Hemorrhage ; Hirschsprung Disease ; Humans ; Imidazoles ; Intestinal Obstruction ; Length of Stay ; Nitro Compounds ; Postoperative Complications ; Retrospective Studies ; Wound Infection

Anorectal malformations comprise a spectrum of disease and the majority of patients have one or more abnormalities that affect other systems. In evaluating a newborn with anorectal malformation, the decision regarding the need for a colostomy and detection and management of any life threatening associated anomalies are thetwo most important considerations. Perineal inspection provides the clue to the surgical approach in about 80-90% of male and 90% of female newborn baby. The remaining patients who do not show any clinical evidence need radiologic evaluation to decide whether a colostomy should be performed. In most cases the decision to make a colostomy should not be made until the baby is 20 to 24 hours old and evaluation to rule out the presence of associated anomalies completed. A divided colostomy at the junction of the descending and sigmoid colon is recommended for anorectal malformations.
Colon, Sigmoid ; Colostomy ; Diagnosis* ; Female ; Humans ; Infant, Newborn ; Male