Problems in Survivors of Biliary Atresia -5 or More Year After surgery-

Hepatic porto-enterostomy or Kasai's original Roux-en Y portojejunostomy was done on 56 patients during the 1980-1985 period. Seventeen patients or 30 per cent had survived more than 5 years at the end of 1990, and are presently being followed up. Average survival age is 7.5 years after surgery. No survivor has clinical or laboratory jaundice. Esophageal varices were demonstrated in 8 patients (47%), and 4 had bleeding episodes. Five patients or 29 per cent had more than one episode of hospitalization for cholangitis. Growth was above 75 per centile in height in 53 per cent. Nine patients(53%) have normal liver function, showed neither varix and nor ascites. Good prognostic factors for 5 year survival were an operative age of 60 days or less, mild fibrosis of the liver, and a big fibrotic mass at the porta hepatis at the time of surgery

Defecation Patterns after Posterior SagittaI Anorectoplasty with an Objective Scoring System

Imperforate anus, the common name representing a wider spectrum of anorectal malformations in newborns is one of the major congenital lesions in pediatric surgery. Since the establishment of pediatric surgery in Seoul National University (1978), more than 500 cases of anorectal anomalies had been treated up to 1990. To evaluate the quality of life after corrective treatment, defecation patterns were studied using clinical Kelly scores in 90 patients with high type ano-rectal anomalies repaired during the 1978-1985 period. These 90 patients were treated by two surgical repair methods (26 Rehbein, 64 Pena). Not only the continence scores but also other aspects of social adaptation as well as an index of quality of life were compared with a control group. Although precise anatomical repair under direct view (Pena) will give a better defecation pattern compared to the blind type of abdomino-perineal repair, their long term adaptation is not significantly superior. This study showed that the function is not always the net result of anatomic repair. Thus, the importance of the initial corrective operation is again emphasized

Juvenile Angiodysplasia of Gut

Angiodysplasia or arteriovenous malformation of the gastrointestinal tract in younger patients is different from "classic" angiodysplasia in older patients, by age of onset, clinical presentation, location of the lesion and diverse histologic features, We report two cases of juvenile angiodysplasia of the gut. The diagnosis was suspected by radionuclide blood pool scan and was confirmed by resection and pathological examination, Both cases were girls of 5 years and 12 years of age, and the lesions were in the jejunum and ileum, Grossly, one case showed multiple petechiae and another case showed a hemorrhagic polypoid mass, Microscopically, the lesions were composed of irregularly dilated vascular channels in mucosa and submucosa with abnormal proliferation of arteries and veins in submucosa, Both cases are free of recurrence after local resection

Falciform Ligament Abscess after Omphalitis: Report of a Case

A falciform ligament abscess is a rare type of intra-abdominal abscess. A 2-yr-old male, who had omphalitis two months previously, presented with a fever and right upper quadrant abdominal pain. The ultrasound and CT scan showed an abdominal wall abscess located anterior to the liver, which was refractory to conservative management with percutaneous draninage and antibiotics. On the third recurrence, surgical exploration was performed and revealed an abscess arising from the falciform ligament; the falciform ligament was excised. A follow up ultrasound confirmed complete resolution of the abscess with no further recurrence

Neonatal Surgical Emergencies At Seoul National University Hospital: Comparison Between the Pre- and Post-Children's Hospital Period

Results of management of the neonatal surgical emergencies were analyzed and compared between 460 infants treated during the 1979-85 period (prechildrens hospital) and 471 infants treated during the 1986-92 period (post-childrens hospital). No statistical difference was noted in patients population, and average mortality rate in the pediatric surgical department which has already been functioning as an independent unit since 1978. Other surgical departments have experienced 2-3 fold increases in number of patients. Patient's age at admissionl delay in transfer} was not significantly improved. However, significant improvement in survival was noted in patients who required respiratory support such as gastroschisis, and esophageal atresia with tracheo-esophageal atresia. Changes in the general management plan are discussed

Colorectal Lymphoid Polyposis in a Child

Lymphoid polyposis is a lymphoid hyperplasia of the gastrointestinal tract that usually presents as multiple small polyps in the colon during childhood. This should be differentiated from other neoplastic or familial polyposis of the intestine. We report a case of benign lymphoid polyposis of the colon in a 17-month-old boy who presented with perianal fistula and mucosal ulceration. Colon study and rectal examinations showed multiple polyps in the sigmoid colon and rectum. Segmental resection of the sigmoid colon and rectum showed over 100 smallt 3 - 7 mrn) sessile or pedunculated polyps that were diffusely scattered through out the removed segment. The polyps consisted of mature lymphoid tissue with numerous germinal centers, that was located mostly in the lamina propria and submucosa

Separated Fetus-in-fetu In Retroperitoneum

Fetus-in-fetu is an extremely rare enigmatic condition, every case of which should be carefully examined, particularly for the distinction between it and a mature teratoma and the determination of its embryopathogenesis. This report deals with a case of fetus-in-fetu that was found in two parts. The host(autosite) was a 14month- old boy who first presented with a back mass. A large round mass, 7 X 6 X 6cm, was removed from the left upper retroperitoneum. The mass was cystic containing 200ml of yellow serous fluid and consisted of two separate masses. One part resembled a stunted dysmorphic fetus, while the other was an amorphous lump, which disclosed a sacrococcygeal bone with five vertebrae. Histologically, both masses showed largely regressed digestive, respiratory, and musculoskeletal tissue. However, the parenchymal organs were missing. Definite vertebral bones, intervertebral discs, and spinal cord remnant were found

Visceral Myopathy of Intestinal Pseudoobstruction

Intestinal pseudoobstruction is a syndrome complex caused by a variety of disorders of various etiology. It can be classified pathologically as visceral myopathy and visceral neuropathy. The sporadic form of visceral myopathy is characterized histologically by vacuolar degeneration and fibrosis of smooth muscle but differs from the familial form only by the absence of other affected family members. We studied 6 cases with intestinal pseudoobstruction classified as sporadic visceral myopathy. They were four boys and two girls, and were two neonates, two infants and two children. The duration of symptoms ranged from two days to two years. Two babies were dead from pneumonia and sepsis. Others were alleviated after surgical resection of the bowel. Both small and large intestines were found affected in autopsy cases. Histopathologic features were vacuolar degeneration of muscularis propria, disproportionate hypoplasia of outer muscle layer, abnormal muscle direction of muscularis propria, submucosal and/or interstitial fibrosis and extra muscle layering. It is presumed that a variety of histopathologic features accounts for visceral myopathy of intestinal pseudoobstruction

Gastrointestinal hemangioma in childhood: a rare cause of gastrointestinal bleeding

Gastrointestinal (GI) hemangiomas are relatively rare benign vascular tumors. The choice of an appropriate diagnostic method depends on patient age, anatomic location, and presenting symptoms. However, GI hemangiomas are not a common suspected cause of GI bleeding in children because of their rarity. Based on medical history, laboratory results, and imaging study findings, the patient could be treated with either medication or surgery. Herein, we report 3 cases of GI hemangioma found in the small bowel, rectum, and GI tract (multiple hemangiomas). Better knowledge and understanding of GI hemangioma could help reduce the delayed diagnosis rate and prevent inappropriate management. Although rare, GI hemangiomas should be considered in the differential diagnosis of GI bleeding

Additive Manufactured Zirconia-Based Bio-Ceramics for Biomedical Applications

Zirconia was established as one of the chief vital ceramic materials for its superior mechanical permanency and biocompatibility, which make it a popular material for dental and orthopedic applications. This has inspired biomedical engineers to exploit zirconia-based bioceramics for dental restorations and repair of load-bearing bone defects caused by cancer, arthritis, and trauma. Additive manufacturing (AM) is being promoted as a possible technique for mimicking the complex architecture of human tissues, and advancements reported in the recent past make it a suitable choice for clinical applications. AM is a bottom-up approach that can offer a high resolution to 3D printed zirconia-based bioceramics for implants, prostheses, and scaffold manufacturing. Substantial research has been initiated worldwide on a large scale for reformatting and optimizing zirconia bioceramics for biomedical applications to maximize the clinical potential of AM. This book chapter provides a comprehensive summary of zirconia-based bioceramics using AM techniques for biomedical applications and highlights the challenges related to AM of zirconia