The survival of Very Low Birth Weight (VLBW) infants has been improved with the advancement of neonatal intensive care. However, the incidence of accompanying gastrointestinal complications such as necrotizing enterocolitis has also been increasing. In intestinal perforation of the newborn, enterostomy with or without intestinal resection is a common practice, but there is no clear indication when to close the enterostomy. To determine the proper timing of enterostomy closure, the medical records of 12 VLBW infants who underwent enterostomy due to intestinal perforation between Jan. 2004 and Jul. 2007 were reviewed retrospectively. Enterostomy was closed when patients were weaned from ventilator, incubator-out and gaining adequate body weight. Pre-operative distal loop contrast radiographs were obtained to confirm the distal passage and complete removal of the contrast media within 24-hours. Until patients reached oral intake, all patients received central-alimentation. The mean gestational age of patients was $26^{+2}$ wks ($24^{+1}{\sim}33^{+0}$ wks) and the mean birth weight was 827 g (490~1450 g). The mean age and the mean body weight at the time of enterostomy formation were 15days (6~38 days) and 888 g (590~1870 g). The mean body weight gain was 18 g/day (14~25 g/day) with enterostomy. Enterostomy closure was performed on the average of 90days (30~123 days) after enterostomy formation. The mean age and the mean body weight were 105 days (43~136 days) and 2487 g (2290~2970 g) at the time of enterostomy closure. The mean body weight gain was 22 g/day after enterostomy closure. Major complications were not observed. In conclusion, the growth in VLBW infants having enterostomy was possible while supporting nutrition with central-alimentation and the enterostomy can be closed safely when the patient's body weights is more than 2.3 kg.
Patients with severe Ebstein's anomaly showing in the neonatal period, represent progressive cardiac enlargement with pulmonary hypoplasia and functional pulmonary atresia with patent ductus alteriosus-dependent pulmonary circulation. Biventricular repair in these patients had been mostly unsuccessful except for Starnes' procedure that converts the anatomy to single ventricle physiology for Fontan procedure. A 4-days old male was admitted with the diagnosis of severe Ebstein's anomaly with anatomic pulmonary atresia and severe cardiac enlargement. He successfully underwent biventricular repair with vertical plication method of atrialized right ventricle, tricupid annuloplasty, transannular right ventricular outflow tract reconstrulltion, atrial septal defect patch closure with fenestration, and right atrial reduction angioplasty Postoperatively, cardiothoracic ratio was significantly reduced and mild tricuspid regurgitation was remnant in echocardiography. The patient is currently 10 months old and is fully active without restrictions.
The aim of this study is to review our clinical experience with patients with Hirschsprung's disease (HD) Medical records of 39 children who underwent definitive surgery for HD at Inha University Hospital from September 1996 to June 2008 were analyzed by age at presentation, sex, gestational age, birth weight, clinical presentation, diagnostic tools, level of aganglionosis, surgical procedures, postoperative complications, and postoperative bowel function. Twenty-five patients (64.1 %) were males and 14 (35.9 %) were females. Thirty patients (76.9 %) were diagnosed and treated in the neonatal period. The transitional zone was at the rectosigmoid region in 89.7 %. Twenty-seven patients (69 %) were treated by preliminary colostomy or ileostomy. Twenty-four patients had the Duhamel operation, 6 patients anorectal myectomy, and 9 patients had transanal endorectal pull-through (TEP). Five of 9 patients who had the TEP procedure did laparoscopic assistance. Postoperatively, seventeen patients (83 %) passed stool once or more times per day and 3 patients had stool soiling. This study demonstrated that the majority of the patients had good results. To determine which treatment is most effective comparative review by operation method would be required.
Biliary atresia (BA) is very difficult to distinguish from neonatal hepatitis (NH) and its prognosis depends on the age at the time of Kasai operation. Therefore early differentiation between these two conditions is very important. Although various clinical and laboratory tests have been reported to differentiate between them, they are still of limited value. From 1980 to 1999, forty-five infants were referred to our pediatric surgical unit for operation for suspected BA. Eight patients underwent Kasai operation immediately because of late diagnosis. These were excluded from the study. The clinical history, physical findings. radiologic and laboratory examinations of 37 cases were analyzed retrospectively. The average age of BA (n=20) was $55.1{\pm}16.7$ days, and that of NH (n=17) was $55.8{\pm}15.6$ days. The sex ratio of BA was 13:7, and that of NH was 14:3. All the patients had obstructive jaundice and acholic stool except 4 BA and 6 NH patients. Acholic stool with yellow component was more frequent in NH. Onset of jaundice was within 2 weeks after birth in 85 % of BA, and in 65% of NH. The onset of acholic stool was within 2 weeks after birth in 60 % of BA, and in 23.5 % of NH. The duration of jaundice and acholic stool of BA were $50.9{\pm}16.6$ days and $41.3{\pm}18.4$ days and those of NH were $40.1{\pm}23.1$ days and $26.6{\pm}25.4$ days respectively. The ultrasonogram and hepatobiliary scan were useful, but not a definitively reliable method for the differentiation of these two diseases. There was no difference in laboratory data. Seventeen cases had NH among 45 referred cases for Kasai operation with the clinical impression of BA, and 4 cases of 17 NH cases needed to be explored to rule out BA. In conclusion. false positive rate of clinical impression of BA was 37.8 %. and negative exploration rate was 8.9 %, Therefore. careful clinical observation for 1-2 weeks by an experienced pediatric surgeon was very important to avoid unnecessary operation to rule out NH up to the age of 8 - 10 weeks. so long as the stool had yellow component.
Background: Pulmonary atresia with intact ventricular septum is a rare congenital cardiac disease. There have been various surgical options, but there are still controversies. Material and Method: Fourteen neonates who were operated on between 1999 and 2000 were enrolled in this study. We measured Z-value of tricuspid valve by echocardiography. We performed right ventricular outflow tract transannular patch in 9 patients. Modified Blalock-Taussig shunt was placed according to the condition of patients. Mean follow up period was 46.0 months. Result: There were 2 operative mortality and 1 late mortality. Biventricular repair was possible in 7 patients. One and a half ventricular repair were done in 3 (tricuspid valve Z-value was -2, -2.5 and -3) and single ventricular repair in 2 patients (tricuspid z-value was -4.6, -4.5) The tricuspid valve Z-value for the patients who had biventricular repair and one and a half ventricular repair were -0.8$\pm$1.50 (-3.2$\∼$1.2) and -2.5$\∼$0.5 (-3$\∼$ -2) respectively. All patients who survived had fair to good right ventricular function, good left ventricular function and good clinical states. Conclusion: Neonatal surgical management of pulmonary atresia with intact ventricular septum has a good surgical outcome, z-value of tricuspid may be helpful for the determination of surgical options.
Background: Recently, open heart surgerys using homograft are progressively increasing in complex cardiac anomalies, and even though the use of homograft tissues harvested from hearts of transplant recipients and brain-death patients are allowed and their use is increasing, the supply of homograft tissue is very limited. Material and Method: The large diameter homografts are difficult to apply directly for RVOT reconstruction of small neonatal and infant hearts due to the size mismatching. Therefore, were surgically down-sized the large diameter tricuspid homograft into bicuspid conduits by means of a longitudinal incision of the oversized homograft, excision of one cusp, and oversewing of the“Bicuspid homograft”wrapped around a Hega dilator of the appropriate size. Result: 3 patients(Male 1, Female 2: tetralogy of Fallot with pulmonary atresia), ranging in age from 5 months to 4 years and ranging in weight from 5.5Kg to 12.95Kg underwent reconstruction of the RVOT with bicuspid conduits obtained by appropriate tailoring from large-diameter homografts. The mean follow-up period was 4.3 months(range, 2 to 6 months). There were no complications related to the homograft tissues. Conclusion: In the short term follow-up, the bicuspid homografts provided good competence and excellent hemodynamics although a long term follow-up is needed to assess the functions of the bicuspid homografts in RVOT. We believe this technique may be a more effective alternative than the use of synthetic conduits when the use of an appropriate-sized homograft is not possible.
Infantile hepatic hemangioendotheliomas (IHHEs) are benign vascular tumors, but can be associated with the life-threatening complications, such as congestive heart failure, disseminated intravascular coagulation, and massive bleeding. Various therapeutic options have been developed and the treatment response depends on the patient's clinical status and the nature of the lesion. In the case of a symptomatic IHHE, a non-invasive and precise diagnosis should be performed promptly before the therapeutic method is chosen. Additionally, it should be kept in mind that the residual lesions have malignant potential. We report a case of a congenital giant IHHE that was successfully reduced in size by interferon-$\alpha$ and completely removed by surgical tumor resection with a hepatic lobectomy.
In order to test the hypothesis that the pulmonic valve, when used to replace the aortic root as a pulmonary autograft, will remain a viable anatomical structure and will grow and develop normally along with the host, we performed aortic valve replacement with the pulmonary autograft in 15 neonatal piglets. The weight of the donor was 9.3 $\pm$ 0.2 kg, the recipient 9.6 $\pm$ 0.3 kg. Measured diameters of pulmonic annulus were 14 $\pm$ 0.2 mm for autograft and 14.2 $\pm$ 0.2 mm for pulmonary artery homograft. Operation was performed under cardiopulmonary bypass with deep hypothermia [20oC at low flow perfusion [70 ml/kg/min . The mean operation time was 227 $\pm$ 10 min., bypass time 152$\pm$ 7.6 min. and aortic cross clamp time 73$\pm$ 4.6 min.. 9 piglets survived more than 12 hours. One survived 12 days and died of pneumonia and the latest one survived in good condition and sacrificed at postoperative 6th week for cardiac catheterization and pathologic examination that revealed the viability and growing of the pulmonary autograft. Currently we are able to complete the operation with good preservation of cardiac function, and our postoperative care has evolved to the extent that we are now confident enough of having an acceptable percentage of long term survivors to undertake a definite study in this regard.
Pulmonary atresia with intact ventricular septum, and with VSD were uncommon congenital anomalies with high mortality in the neonatal period. Those survivals depend on an adequate interatrial communication or interventricular communication and pulmonary flow via large aortopulmonary collateral including PDA. Recently we experienced surgical correction of 2 cases pulmonary atresia with intact ventricular septum and with VSD. On case 1, 10-years old male patient was confirmed as pulmonary valvular membranous atresia with intact ventricular septum combined with large functioning PFO and mild tricuspid incompetence. So we performed total correction under the E.C.C, that was PDA ligation, RVOT reconstruction with monocusp valved outflow patch [16mm], repair of tricuspid insufficiency and closure of PFO. Post-operative hemodynamic result was good and there was no event during hospital course. On case 2, 16-years old female patient was diagnosed as pulmonary atresia with VSD and PDA. MPA was absent, remained fibrous cord like remnant and type of VSD was subaortic defect [3cm by 3cm in the size]. PDA was located at the usual site. Under the E.C.C. VSD patch closure through the right ventriculotomy, anastomosis between the right ventricular outflow tract and the pulmonary bifurcated site with the extra-cardiac Hancock valved conduit [22cm] and PDA ligation were performed.
Kim, Seung-Hwan;Kim, Seong-Min;Oh, Jung-Tak;Han, Seok-Joo;Choi, Seung-Hoon
Advances in pediatric surgery
/
v.12
no.2
/
pp.167-174
/
2006
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.
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