Clinical differences between choledochal cysts in infancy and childhood: an analysis of 160 patients.

BACKGROUND: Choledochal cysts (CDC) can become symptomatic at any age. Clinical features of infants and older children are different and have been documented by many authors. OBJECTIVE: The aim of this report was to study the differences in clinical spectrum of CDC between the infants and children treated at our hospital during a 17-year period. MATERIAL AND METHOD: A retrospective study was conducted of 160 patients with CDC treated at Queen Sirikit National Institute of Child Health between 1996 and 2012. The patients were categorized into 2 groups based on age at clinical presentation: an infantile group (up to one year old) and a childhood group (over one year old). Clinical characteristics of patients in the infantile and childhood groups were compared with statistical analysis using the Chi-square and Fisher's exact test. RESULTS: Of the 160 patients with CDC, 48 cases (30%) were categorized in the infantile group and 112 cases (70%) in the childhood group. Over three-quarters ofthe patients in the infantile group presented with jaundice and acholic stool, and this incidence was significantly higher than in the childhood group (77% vs. 46.4%, p = 0.001 and 50% vs. 13.4%, p < 0.001). Abdominal pain was the most common symptom of patients in the childhood group, whereas it was noted in only a small number of those in the infantile group (82.1% vs. 8.3%, p < 0.001). Average amylase level in CDC content was markedly elevated in the childhood group but much lower in the infantile group (43,630.5±90,234.5 vs. 79±189.9, p < 0.001). Only type I and type IV CDC as defined by Todani's classification were found in our patients, and there were no statistical differences in incidences of type I and type IV in the two groups (79.2% vs. 67.8% and 20.8% vs. 32.2% p > 0.05). Neonates and infants with CDC had a significantly higher risk of liver cirrhosis than did the childhood group (25% vs. 8%, p < 0.001). However, surviving patients with cirrhosis in both groups were doing well at least 3 years after surgical CDC excision. CONCLUSION: Neonates and infants with CDC were more likely to present with jaundice and acholic stool, whereas older children were more likely to present with abdominal pain. Amylase level in CDC content was markedly elevated in the childhood group but at a much lower level in the infantile group. Neonates and infants with CDC tended to develop liver cirrhosis earlier and more often than older children.

Clinical outcome of patients with gastroschisis: what are the differences from the past?

OBJECTIVE: The aim of the present study was to review the experience in management of neonates with gastroschisis and analyze the differences in the clinical outcome during a 24-year period. MATERIAL AND METHOD: A retrospective study of patients with gastroschisis treated at Queen Sirikit National Institute of Child Health (QSNICH) between 1986 and 2009 was conducted. Patients' information was compared between the first period (1986-1997) and the second period (1998-2009) regarding demographic data, modes of operative procedures and results of the treatment. The statistical differences were analyzed by the Chi-square, Fisher exact and student t-test with a p-value less than 0.05 considered significant. RESULTS: During a 24-year period, 919 neonates with gastroschisis were treated at QSNICH; 342 cases (161 males and 181 females) in the first 12-year period and 577 cases (295 males and 282 females) in the second 12-year period. The incidence of gastroschisis at Rajavithi Hospital was 0.26: 1,000 live births in the first period and 1.03: 1,000 live births in the second period. Average birth weight of the patients and average maternal age in both periods were not significantly different (p > 0.05). Congenital anomalies were found in approximately 15% of the patients in each period. Regarding modes of the operative treatment, primary closure of the abdominal wall defect was attempted in 23.7% of the patients during the first period and increased to 44% in the second period. The overall survival rate in the second period was better than the first period with statistical significance (92.4% vs. 75.4%, p < 0.001). In addition, complications in the second period were less than those in the first period, except for necrotizing enterocolitis, which was more frequent in the second period. CONCLUSION: The obvious differences in patients with gastroschisis during the 24-year period were the increased incidence, increased successful primary closure of the abdominal wall defect and increased overall survival rate between 1998-2009. Improvement of the clinical outcomes reflected improved neonatal care including surgical techniques, parenteral nutrition, respiratory care and anesthetic practice.

Atresia of the jejunum and ileum: what is the difference?

BACKGROUND: Atresia of the jejunum and ileum is one of the major causes of neonatal intestinal obstruction. Most affected newborn infants present with bilious emesis and abdominal distention. Traditionally, jejunal and ileal atresia have been grouped together as jejunoileal atresia. OBJECTIVE: To elucidate the difference between jejunal and ileal atresia. MATERIAL AND METHOD: A retrospective analysis of patients diagnosed with jejunal or ileal atresia, who were treated at the Department of Surgery, Queen Sirikit National Institute of Child Health during January 1988 to December 2007, was carried out. RESULTS: There were 74 patients with jejunal atresia and 68 patients with ileal atresia. The mean birth weight and gestational age of patients with jejunal atresia were significantly lower than those with ileal atresia. Antenatal perforation occurred more frequently in ileal atresia. Postoperative course was more prolonged and mortality was higher injejunal atresia. Prolonged ileus and anastomotic dysfunction requiring long-term parenteral nutrition were the major causes of complications leading to death. CONCLUSION: There were many differences between patients with jejunal atresia and those with ileal atresia. The more compliant jejunal wall allows massive dilatation upon obstruction with subsequent loss of peristaltic activity, thus poorer outcome in comparison with ileal obstruction. We suggest that atresia of the jejunum and ileum be considered differently.

Long-Term Outcomes of Sacrococcygeal Germ Cell Tumors in Infancy and Childhood.

Purpose. The aim of this study was to evaluate long-term outcomes of sacrococcygeal germ cell tumors (SC-GCTs) over a 15-year period. Materials and Methods. A retrospective review was conducted of all pediatric patients treated for SC-GCTs at our hospital from 1998 to 2012. Results. Fifty-seven patients were treated for SC-GCTs with the most common in Altman's classification type I. Age at surgery ranged from one day to 5.6 years. Tumor resection and coccygectomy were primarily performed in about 84% of the cases. Pathology revealed mature, immature, malignant sacrococcygeal teratomas (SCTs), and endodermal sinus tumors (ESTs) in 41 (72%), 4 (77%), 6 (10.5%), and 6 (10.5%), respectively. Recurrence of discase occurred in 3 of 41 patients with mature teratomas (7.3%); 2 recurrences with mature teratomas and one recurrence with EST. Five of 6 malignant SCTs and 3 of 6 ESTs responded well to the treatment. Alpha-fetoprotein (AFP) level was elevated in both malignant teratomas and ESTs. No immediate patient death was noted in any of the 57 cases, but 4 patients with malignant tumors and distant metastasis succumbed at home within 2 years of the initial treatment. Conclusion. Benign SCTs have a significant recurrence rate of approximately 7%. Close follow-up with serial AFP level monitoring should be done for 5 years after initial tumor resection and coccygectomy. The survival rate for malignant SC-GCTs with distant metastasis was unfavorable in the present study.

Clinical outcomes of esophageal atresia: comparison between the Waterston and the Spitz classifications.

INTRODUCTION: Preoperative prognostic predictors are important for surgeons and parents to estimate the survival of patients with esophageal atresia (EA). The aim of this study was to update the clinical outcomes of EA treatment by comparing between the Waterston and the Spitz classification. MATERIALS AND METHODS: Medical records of the patients with EA treated at Queen Sirikit National Institute of Child Heath from 2003 to 2010 were reviewed. All of the patients were categorised into 3 groups of the Waterston and 3 groups of the Spitz risk factor criteria for comparing of the differences in each group and each classification. RESULTS: One hundred and thirty-two patients (81 males and 61 females) were treated for EA during the study period. Applying the Waterston classification, survival rate was 100% in group A, 91.5% in group B and 48.8% in group C. There was no statistical difference between the survival rate in group A and group B (P = 0.119) but significant difference between group B and group C (P = 0.000). Using the Spitz classification, survival rate was 97.4% in group I, 64.4% in group II and 27.3% in group III. There was obviously statistical difference of the survival rate between each group (group I vs group II, P = 0.000; group II vs group III, P = 0.041). CONCLUSION: Comparing with the prognostic predictors, the Spitz classification was more valid than the Waterston criteria. The Spitz classification is suitable to use for preoperative predictor to parental counselling and comparing of treatment outcomes of EA among paediatric tertiary care centres.

Influence of Down's syndrome on management and outcome of patients with congenital intrinsic duodenal obstruction.

PURPOSE: Children with Down's syndrome (DS) have been reported to have a high incidence of congenital heart disease (CHD) and respiratory tract infection. These anomalies and complications lead to poor outcomes, especially after treatment of congenital intrinsic duodenal obstruction (CIDO). The aim of this study was to review and compare the outcome of management of CIDO at a single tertiary institute for children in Thailand. METHODS: We reviewed the medical records of patients with CIDO, who were treated at Queen Sirikit National Institute of Child Health (Bangkok, Thailand) from 1997 to 2006. The patients were classified into 2 groups: A = CIDO with DS and B = CIDO with non-DS (NDS). Each group was then divided into 2 subgroups: A(1) = DS without CHD, A(2) = DS with CHD, B(1) = NDS without CHD, and B(2) = NDS with CHD. Comparisons of management and outcome between group A and group B, subgroup A(1) and subgroup B(1), and subgroup A(2) and subgroup B(2) were performed. Statistical differences were analyzed by the chi(2) test at a P < .05. RESULTS: A total of 227 patients (male-female = 108:119) underwent surgical correction of CIDO. Of 227 patients, 86 (37.9%) were shown to have DS (group A), and 141 (62.1%) were NDS patients (group B). Demographic information did not differ between the groups, except for the mothers' age at pregnancy and the operative procedure used for duodenoduodenostomy. Group A had a higher incidence of CHD than group B (66.3% vs 26.2%; P < .05). Early outcome regarding survival rate (SR) was not statistically different between groups (87.2% vs 92.9%; P = .15), but the rate of early postoperative complications in group A was higher than that in group B (45.3% vs 31.1%; P = .03). The overall SR of group A at long-term follow-up was worse than that in group B (68.6% vs 86.7%; P < .05). Late complications and sequelae in group A were also more frequent than in group B (34.6% vs 9.2%; P < .05). When comparing early outcome between DS and NDS patients without CHD, the SR of subgroup A(1) was lower than that of subgroup B(1) (86.5% vs 96.5%; P = .04), but the rate of early postoperative complications did not differ (41.5% vs 24.1%; P = .05). At long-term follow-up, the overall SR of subgroup A(1) was worse than that of subgroup B(1) (73% vs 95.6%; P < .05) because of a higher incidence of late death because of recurrent respiratory tract infection in the A(1) subgroup. In addition, late complications and sequelae were more common in subgroup A(1) than in subgroup B(1) (37.5% vs 8.9%; P < .05). In patients with CHD, there was no statistical difference between early and long-term results of SR or complications between subgroup A(2) and subgroup B(2). The overall SR at long-term follow-up was 63% in subgroup A(2) and 56% in subgroup B(2) (P = .5). CONCLUSIONS: Early outcome of CIDO management revealed minimal differences between DS and NDS patients. However, these differences were clearer in long-term outcomes. The DS patients had lower long-term SR and higher rates of complications and sequelae than NDS patients. It is concluded that DS negatively impacts the management and outcome of patients with CIDO.

Management of recurrent intussusception: nonoperative or operative reduction?

PURPOSE: The aim of this study was to determine how to manage children with recurrence of intussusception. METHODS: Medical records of patients treated for intussusception from 1976 to 2008 at the Queen Sirikit National Institute of Child Health were reviewed. Information on patients who developed recurrent intussusception was extracted to study patterns of recurrent attack and suitable management procedures. The statistical differences were analyzed by the χ² and the Student t test, with a P value < .05 considered significant. RESULTS: During the study period, 1340 patients were treated for 1448 episodes of intussusceptions, with an average of 40 cases per year. There were 108 episodes of recurrent intussusception in 75 patients (45 males and 30 females). The overall recurrence rate was 8%. Patient age at the first episode ranged from 3 months to 12 years (average, 14.9 months). The time interval before each recurrence ranged from 1 day to 3.2 years (average, 7.8 months). The number of recurrences ranged from 1 to 5 attacks. Recurrent intussusception occurred in 35 (15.8%) of 222 children following successful hydrostatic barium enema (BE) reduction and in 55 (11.4%) of 482 after successful pneumatic or air enema (AE) reduction. There was no statistical difference between the recurrence rates after the 2 nonoperative procedures (P = .08). Recurrent intussusception developed in 14 (3.0%) of 457 patients after operative manual reduction. Recurrence was not observed after intestinal resection for initial irreducible intussusception in 175 patients. The remaining 4 recurrent episodes occurred after spontaneous reduction. Of the 108 episodes of recurrence, BE and AE reductions were successful in 25 (96.2%) of 26 attempts and in 57 (92%) of 62 attempts, respectively. Seven patients had their first episode of intussusception treated surgically. All 7 when they recurred were successfully treated with either BE or AE reduction. Operative intervention was needed in 23 episodes of recurrent intussusception; 18 were reduced manually, and 5 required intestinal resection. Overall, 7 (9.3%) of the 75 recurrences had a pathologic lead point: colonic polyps in 4 cases and Meckel diverticulum in 3 cases. There were no deaths among the 75 patients with recurrent intussusception. CONCLUSIONS: Recurrent intussusception should be initially treated by nonoperative reduction. Laparotomy is needed in cases with failure of BE or AE reduction, in cases with suspicion of a pathologic lead point, and in selected cases with several episodes of recurrence. The treatment of recurrent intussusception, in general, should be similar to that of primary intussusception.