Discriminating preoperative features of pleuropulmonary blastomas (PPB) from congenital cystic adenomatoid malformations (CCAM): a retrospective, age-matched study.

BACKGROUND: The etiopathogenesis of pleuropulmonary blastoma (PPB) and its relationship to congenital cystic adenomatoid malformation (CCAM) remain controversial. Our recent analysis indicates that both the co-incidental occurrence and the outcome of PPB among CCAM patients are significant. We report here on our efforts to determine clinical and radiological features discriminating PPB from CCAM preoperatively. MATERIALS & METHODS: A retrospective analysis of all patients treated for PPB and CCAM between 1981 and 2008 at 2 tertiary academic centers under a centralized, single payer healthcare system was performed (REB#1000013239). Clinical, radiological and demographic data were analyzed. PPB patients were secondarily age matched with CCAM patients (± 10% age difference in months), and clinical, radiological and demographic variables were compared. Descriptive statistics and non-parametric analysis were used. RESULTS: A total of 10 PPB patients was identified. Median age at diagnosis was 24 months; the male to female ratio was 5:5. No PPB patients had an antenatal diagnosis (p<0.01). 9 were symptomatic, with symptoms including dyspnea (7/10), upper respiratory infection (6/10), poor weight gain (3/10), and 1 patient was asymptomatic. 5 of 10 patients had solid parts on CT, of which 4 out of 5 were diagnosed preoperatively as PPB and 1 out of 5 as CCAM. 5 of 10 were predominantly cystic of which none was diagnosed preoperatively as PPB (p=0.0476). Given that most CCAM patients are now diagnosed antenatally, only 5 PPB patients could be age-matched with CCAM patients. In the predominantly cystic PPB patients (n=5), no significant discriminating clinical and radiological features were identifiable when compared preoperatively with age-matched CCAM patients. CONCLUSION: PPB patients continue to represent a diagnostic challenge. Asymptomatic and predominantly cystic PPB remain indistinguishable from CCAM preoperatively. A high index of suspicion for PPB must be considered in any child presenting with cystic lung lesions beyond early infancy, particularly in a child with poor weight gain.

The current state of congenital tracheal stenosis.

Congenital tracheal stenosis (CTS) is an uncommon condition that has challenged pediatric surgeons for decades. Patients with CTS can present with a wide spectrum of symptoms and varying degrees of severity. In addition, a variety of techniques have been devised to repair this malformation. A review of these procedures and our suggestions for clinical standards and practice guidelines will be presented in this paper. A retrospective review of the literature on CTS from 1964 to 31 March, 2006. There is not one standard technique for the repair of CTS, as individualized approach to each patient and airway lesion is necessary to optimize patient management; nevertheless there is a consensus about segmental resection and anastomosis being best for short segment stenosis while slide tracheoplasty is most effective for the long-segment ones. Conservative management is also an option for select group of patients with careful and close follow up. Survival following surgery over the years has improved, but mortality remained high, particularly in a specific subset of patients presenting at the age less than 1 month with associated cardiac malformations. In conclusion, CTS remains a significant challenge for pediatric surgeons. Additional research is required to improve our understanding of the pathogenesis of CTS, and to develop evidence-based treatment protocols for the entire spectrum of presentation including conservative management.

Correlation between radiographic transition zone and level of aganglionosis in Hirschsprung's disease: Implications for surgical approach.

BACKGROUND/PURPOSE: The anticipated level of aganglionosis can influence the surgical approach to Hirschsprung's disease. The aim of this study was to determine the accuracy of the contrast enema in predicting this level. METHODS: Over a 6-year period (1995 through 2000), 88 patients with Hirschsprung's disease underwent surgical correction. Preoperative contrast enema findings were available for 75 of these patients and were compared with operative and pathology reports. Data were analyzed by chi(2). RESULTS: The contrast enema showed a transition zone suggestive of Hirschsprung's disease in 67 of 75 patients (89%). In 59 of 67 (88%), the pathologic and radiographic transition zones were concordant. Seven of the 8 patients with discordant studies had total colonic (n = 5) or long-segment (n = 2) disease. Contrast enema correctly predicted the level of aganglionosis in 55 of 62 (89%) patients with rectosigmoid disease but only 4 of 13 (31%) of those with long-segment or total colonic disease (P <.01). Of the patients with a radiographic transition zone in the rectosigmoid, 54 of 60 (90%) had a matching level of aganglionosis. CONCLUSIONS: In rectosigmoid Hirschsprung's disease, the location of the radiographic transition zone correlates accurately with the level of aganglionosis in 90% of cases. However, the small incidence of discordance between anticipated level of aganglionosis and operative findings should be recognized, particularly when planning a one-stage transanal pull-through.

Is laparoscopic subtotal colectomy better than open subtotal colectomy in children?

PURPOSE: The role of laparoscopic colectomy is not defined clearly. The aim of this study was to compare clinical outcomes of laparoscopic versus open subtotal colectomy in children with inflammatory bowel disease. METHODS: Eight consecutive patients undergoing laparoscopic subtotal colectomy were compared with 10 consecutive patients undergoing open subtotal colectomy. All patients were refractory to medical management on immunosuppressive regimens. Operating time, length of postoperative stay and intravenous narcotic use, time to return of intestinal function, and perioperative complications were compared between the groups. RESULTS: Operating times were significantly longer in the laparoscopic group (mean laparoscopic, 4 hours 40 minutes v mean open 2 hours 25 minutes; P <.01). There was no difference between the 2 groups in length of postoperative intravenous narcotics or hospital stay. Ileostomy output occurred earlier (mean laparoscopic, 2.5 days v mean open 3.8 days; P =.01), and there was a trend toward earlier oral intake in the laparoscopic group. A total of 6 complications occurred in 4 patients in the laparoscopic group compared with 5 complications in 5 patients in the open group. CONCLUSIONS: Perioperative clinical outcomes, including complication rates, are similar with laparoscopic and open subtotal colectomy. Laparoscopic subtotal colectomy can be performed safely in children with improved cosmesis.