Significance of Contrast Enema in One-stage Transanal Endorectal Pull-through Operation for Hirschsprung Disease

In one-stage transanal endorectal pull-through operation (TERPT) for Hirschsprung disease, preoperative evaluation by contrast enema (CE) is important tool in aspect of planning of surgical procedure as well as diagnosis. This study was to evaluate the significance of CE for identifying the extent of aganglionic bowel. A retrospective analysis was performed in 40 patients who underwent TERPT between 2003 and 2011. The authors reviewed the CE studies and their correlation with pathologic extent of aganglionosis. Total 66 contrast enemas were performed in 40 patients. Twenty patients underwent single CE, but 20 patients required multiple CEs. In single CE group, 17 had clear radiographic transition zone, but 3 had less definite transition zone. In multiple CE group, 17 patients who had equivocal finding in first or second CE had definite radiographic transition zone, but 3 patients of this group had less definite radiographic transition zones. Overall, 34 patients (85%) had clear radiographic transition zone by single or repeated CE. One (2.9%) out of 34 patients with clear radiographic transition zone had discordance between radiographic and pathologic transition zone. In contrast 4 (66.7%) out of 6 patients with equivocal radiographic transition zone had discordance between radiographic and pathologic transition zone. Observation of clear radiographic transition zone is important in preparation of TERPT, and repeated CE is helpful to reduce the discordance between radiographic and pathologic transition zone. Awareness of the possibility of discordance is also important if radiographic transitional zone is not clear.

Meconium Obstruction in Neonates-Clinical Characteristics and Treatment

Meconium obstruction (MO) in neonates arises from highly viscid meconium and the poor motility of the premature gut. Recently the incidence of the MO in neonates has been increasing, but, the diagnosis and treatment of this disease have not yet been clarified. Between March 2004 and April 2010, 24 neonates were treated for MO at Severance Children's Hospital. Their clinical characteristics and treatment were reviewed retrospectively. Twenty neonates were diagnosed with MO and 4 neonates were diagnosed with Hirschsprung's disease (HD). The mean birth weight and gestational age of the 20 neonates with MO were 1.45+/-0.90kg and 31.1+/-4.6 weeks, respectively. Thirteen neonates (65%) diagnosed with MO weighed less than 1.5kg and 10 neonates (50%) weighed less than 1kg. Half of the neonates with MO were treated by non-operative methods and the other half were treated by operative methods. Compared with the group that weighed over 1.5kg, the group that weighed less than 1.5kg were more frequently operated upon (61.5% vs. 28.5%), and contrast enemas were performed later and more frequently. Also the group that weighed less than 1.5kg had a higher mortality rate (15.4% vs. 0%). Three of the four neonates with HD were diagnosed with long-segment aganglionosis. In conclusion, MO occurred in very low birth weight neonates more often and must be differentiated from HD. Also, MO in very low birth weight neonates should be treated with special attention due to more a complicated clinical course.

Chronic Recurrent Volvulus of the Colonic Splenic Flexure Associated with the Eventration of Left Diaphragm / 대한소화기학회지

The eventration of diaphragm is usually found incidentally on chest X-ray or sometimes presented as acute gastric volvulus. However, colonic volvulus on splenic flexure area complicated by diaphragmatic eventration is extremely rare. A 25 year old man complained of upper abdominal pain for three days. He had a history of brain injury during infant period, and had epilepsy and mental retardation. Plain chest X-ray showed left diaphragmatic eventration and marked dilatation of colon on splenic flexure area which had not been changed for last three years. Barium enema showed bird beak appearance on distal colon near the splenic flexure. Colonoscopic reduction failed. After decompression with rectal and nasogastric tubes, colonic volvulus was relieved. To prevent the recurrence of volvulus, we performed segmental resection of left colon including splenic flexure area and repaired the left diaphragmatic eventration. After the operation, the patient had no further recurrent episode of volvulus although ileus persisted.

Percutaneous Gastrostomy Tube Reinsertion after Accidental Dislodgement Using Modified Seldinger's Technique

This case report describes a baby who received a laparoscopic gastrostomy tube insertion, which was dislodged accidentally at 16(th) postoperative day. After the dislodgement, cutaneous tract rapidly closed, and reinsertion seemed to be impossible. However, gastrostomy tube was reinserted safely with fluoroscopy-guided Seldinger's technique under local anesthesia with sedation. This is the unique method of modified Seldinger's technique for reinsertion of gastrostomy tube under local anesthesia and sedation for accidentally dislodged gastrostomy tube. This method was thought to be safe, easy and useful technique for gastrostomy reinsertion after dislodgement of gastrostomy tube.

Usefulness of Tunneled Trans-saphenous IVC Catheters for Long Term Venous Access in Pediatric Patients

Central venous catheter (CVC) for long-term venous access is indispensable for various reasons including hyperalimentation, frequent blood sampling, frequent IV drug use in pediatric patients. We report clinical experience of surgical neonates in whom CVC was inserted primarily via great saphenous vein into suprarenal inferior vena cava. From March 2004 to March 2006, we performed CVC insertion via saphenous vein - contralateral side to main wound - into suprarenal inferior vena cava in surgeries of neonates. 2.7Fr or 4.2Fr, single lumen, tunneled Broviac catheters (Bard Access system, Inc, Salt Lake City, Utah) were used. Skin exit site of tunneled catheter was located in ipsilateral flank area just below edge lower rib. At the end of the procedure, location of the catheter tip was confirmed by plain radiography of abdomen. We retrospectively reviewed the admission records of the patients including nursing staff charts. Nine (50.0 %) patients were male and nine (50.0%) were female. Median gestational age was 38 weeks (range, 29-42 weeks) and median birth weight was 3,105 gm (range, 1,040-3,720 gm). Median age at catheter insertion was 38.5 days (range, 1-236 days). The purpose of CVC insertion was short-and long-term hyperalimentation in nine (50.0 %) patients. CVC insertion was performed in operation room under general anesthesia in sixteen (88.9 %) patients (in these cases, CVC insertion was performed just prior to concurrent operation) and neonatal intensive care unit (NICU) under local anesthesia with adequate sedation in two (11.2%). During the admission period (total catheter-indwelling time : 553 days), CVC functioned well without any significant side effects. Transient swelling of the ipsilateral leg (n=1, 5.6 %) and transient migration of catheter tip (n=1, 5.6 %) were noted, which did not affect function of the indwelled CVC. Mean catheter-indwelling time was 30.7days (range, 3-72 days). All catheters were removed electively except two mortality case. Complications, such as thrombosis, infection, kinking or extravasation of drugs, were not observed in our study period. Tunneled trans-great saphenous vein inferior vena cava catheters are not only comparable to cervical CVCs in terms of function and complication rates, but also very beneficial in selected patients, especially those in whom cervical approach is technically impossible or contraindicated.

A Case of Primary Intestinal Lymphangiectasia / 대한소아소화기영양학회지

Primary intestinal lymphangiectasia is a congenital lymphatic disorder in which intestinal lymphatic channels are dilated and ruptured resulting in loss of protein, lipid, and lymphocyte into the intestine or peritoneum. As a result, hypoalbuminemia, generalized edema, diarrhea are clinically manifested. We report a case of primary intestinal lymphangiectasia with generalized edema which occurred in a 7-year old boy who was treated with lipid restriction diet with medium chain triglyceride oil supplement.

Proper Treatment for Megacolon after Various Anoplasties for Anorectal Malformation

PURPOSE: The megacolon after repairing an anorectal malformation is not a rare complication, and there is much controversiy on the causes, the treatment of choice and the results after a longterm follow-up. We present 5 cases of a megacolon after the repair of an anorectal malformation, which were controlled with either a surgical resection or conservative treatment. METHODS: Five patients with a megacolon after the repair of an anorectal malformation were studied. A retrospective chart review was done and fecal continence was evaluated with an individual interview. RESULTS: All five patients initially underwent conservative treatment with laxatives and/or enemas. One Patient responded well to conservative treatment and the diameter of the bowel reduced to normal size. Another patient responded to conservative treatment after correcting the location of the anus. Three patients needed a surgical resection and one of those needed a further procedure to correct the anal location. After the surgical resection of the megacolon and/or correction of the anus (one out of the three patients), they soon reported an almost normal bowel habit. CONCLUSION: The first step in treating a megacolon after repairing an anorectal malformation was conservative treatment. However patients without an adequate response to conservative treatment are best managed with a surgical resection. The cause of the megacolon is now under investigation and the lack of adequate management after repair is one of the subjects.

The development of fetal surgery

The history of fetal surgery features an absolute dependency upon the possibility of diagnosis before birth. Powerful new imaging methods, the techniques of sampling amniotic fluid and fetal tissue, and modern molecular genetics for the prenatal diagnosis of various congenital diseases have removed the veil of secrecy from the fetus. Even though most prenatally detected congenital malformations can be managed after maternal transport, a few simple anatomic defects require fetal surgery, albeit with predictably poor results. The understanding of intrauterine physiology and pathophysiology in several congenital malformations has been worked out in animal model study, and the natural history of congenital defects revealed by prenatal observations on human fetuses. Selection criteria for intrauterine intervention have been developed. Over the last two decades, surgical techniques for open and endoscopic fetal surgery have been defined and anesthesia and tocolysis for fetal surgery improved. As we enter the 21st century, this field of surgery will surely expand.