Laparoscopy-assisted suction colonic biopsy and intraoperative rapid acetylcholinesterase staining during transanal pull-through for Hirschsprung's disease

Background: It is crucial to identify the exact level of transition to normal ganglion cells in instances of Hirschprung's disease. This report describes a technique for laparoscopy-assisted suction colonic biopsy during transanal pull-through. Methods: Laparoscopy-assisted suction colonic biopsy (SCBx) was used in 12 patients with Hirschsprung's disease affecting the rectosigmoid. Average age was 4.4 [plusmn] 2.1 months with a mean operative weight 6.2 [plusmn] 1.0 kg. The pull-through was performed as the primary operative procedure in 11 patients. Using a 2-team approach (laparoscopic team and transanal team), the site was chosen for transanal suction biopsy and marked externally by the laparoscopic team with a silver clip. Biopsies were processed for ganglion cells and rapid AChE technique. Results: There were no biopsy-induced perforations. Abnormal biopsies were repeated more proximally until ganglion cells were observed. Transanal pull-through was performed and an open full-thickness biopsy performed to confirm the presence of ganglion cells. All procedures were performed successfully. Conclusions: Laparoscopy-assisted SCBx can be used successfully in patients with Hirschsprung's disease affecting the rectosigmoid (80% of cases). The technique, when used with rapid AChE staining, provides accurate identification of the level of normoganglionosis.     

Pediatric extraadrenal paraganglioma

Background: Pediatric paraganglioma is rare and extraadrenal paraganglioma has not been characterized in children.Methods: The authors reviewed the medical records and pathology samples of children with extraadrenal paraganglioma treated at our institution between December 1978 and September 2000. Clinical presentation, treatment, and outcome were evaluated.Results: Eight patients (median age, 11.4 years) were identified, 4 were boys and none had a family history of paraganglioma or associated syndromes. Primary tumors arose in the retroperitoneum (n = 3), carotid body (n = 2), jugulotympanic ganglion (n = 1), cervical-paraspinal region (n = 1), and lung (n = 1). Extraadrenal paraganglioma had not been suspected at presentation in any patient. Of 5 patients who underwent gross total resection at the time of diagnosis, 4 remain disease free, 1 had microscopic residual tumor and died of disease. Three patients had initially unresectable disease, 2 are disease free after neoadjuvant therapy and delayed surgery, and 1 has persistent disease after tumor embolization and radiotherapy.Conclusions: Pediatric extraadrenal paraganglioma occurs most commonly in the retroperitoneum and head and neck, and the diagnosis usually is not suspected at the time of presentation. Surgery is the mainstay of treatment, and outcome is good after gross total resection. Neoadjuvant therapy can facilitate complete resection of initially unresectable tumors.     

Interstitial fluid pressure in breast cancer,benign breast conditions,and breast parenchyma

Background: Interstitial fluid pressure (IFP) in rodent malignant tumors is reportedly much higher than in surrounding normal tissue. We hypothesized the same may be true in human invasive breast tumors. Methods: We measured IFP in the operating room in 25 patients undergoing excision breast biopsy under local anesthetic for diagnostic purposes. Results: In patients with invasive ductal carcinomas IFP was 29 ± 3 (SE) mm Hg, compared with ?0.3 ± 0.1 mm Hg in those with normal breast parenchyma (p < 0.001), 3.6 ± 0.8 mm Hg in those with benign tumors (p < 0.003), ?0.3 ± 0.2 mm Hg in those with noninvasive carcinomas (p = 0.034), and 0.4 ± 0.4 mm Hg in those with other benign breast conditions (p = 0.002). There was a direct correlation between IFP and tumor size (R ² = 0.3977; p = 0.021). No correlation was found between IFP and nuclear grade, angiolymphatic invasion, systemic blood pressure, metastasis to lymph nodes, or estrogen and progesterone receptors. Conclusions: IFP measurements may facilitate radiographic or ultrasound localization of small or nonpalpable malignant tumors in those patients undergoing needle aspiration cytology or stereotactic core needle biopsy.     

Malignant retroperitoneal and abdominal germ cell tumors: an intergroup study

Background/Purpose: This randomized study examined survival (S) and event-free survival (EFS) rates using high-or standard-dose cisplatin-based combination chemotherapy and surgical resection for this subset of germ cell tumors. Methods: Twenty-six of 317 patients enrolled on the POG 9049/COG 8882 intergroup study for malignant germ cell tumors had abdomen or retroperitoneum as the primary site. Twenty-five of 26 were eligible for inclusion (n = 25). Patients had biopsy or resection at diagnosis and randomization to chemotherapy including etoposide, bleomycin, and either standard-dose (PEB) or high-dose cisplatin (HDPEB). In patients with initial biopsy, delayed resection was planned. Results: Median age was 26 months. There were 14 girls and 11 boys. There were 3 stage I to II, 5 stage III, and 17 stage IV patients. Surgical management included primary resection in 5, resection after chemotherapy in 13, and biopsy or partial resection in 7 patients. Overall 6-year EFS rate was 82.8% [plusmn] 10.9%, and 6-year survival rate was 87.6% [plusmn] 9.3%. By group, 6-year survival rate was 90.0% [plusmn] 11.6% for PEB and 85.7 [plusmn] 14.5% for HDPEB. Deaths include one from sepsis, one from malignant tumor progression, and one from bulky disease caused by benign components despite response of the malignant elements to chemotherapy. Conclusions: Malignant germ cell tumors arising in the abdomen and retroperitoneum have an excellent prognosis despite advanced stage in most children. Aggressive resection need not be undertaken at diagnosis, but a concerted attempt at complete surgical removal after chemotherapy is important to distinguish viable tumor from necrotic tumor or benign elements that will not benefit from further chemotherapy. J Pediatr Surg 38:315-318.     

Diagnosing Hirschsprung's disease: increasing the odds of a positive rectal biopsy result

Background/Purpose: Rectal biopsies are performed as a definitve means of diagnosing Hirschsprung's Disease (HD) in children presenting with constipation. The authors hypothesized that key features in the history, physical examination, and radiographic evaluation would allow us to avoid unnecessary rectal biopsies. Methods: A retrospective analysis was conducted on patients undergoing rectal biopsy between 1995 and 2001. Patients with HD were identified (n = 50), and a concurrent cohort of patients with idiopathic constipation (IC; n = 50) was selected. Pertinent features in patients with HD versus those with IC were cross tabulated using Pearson [Chi ]² testing (significance was P [lt ] .05). Results: Sixty percent of patients with HD and 15% of patients with IC experienced onset of symptoms in the first week of life. HD patients more frequently experienced delayed passage of meconium (P [lt ] .05), abdominal distension (P [lt ] .05), vomiting (P [lt ] .05), and transition zone on contrast enema (P [lt ] .05). All patients with HD had one or more of these significant features. In contrast, only 64% of patients with IC had one or more of these features. The classic triad of symptoms (ie, delayed passage of meconium, vomiting, and abdominal distension) was present in 18%, and one or more of these symptoms was present in 98% of HD patients. In contrast, only 60% of patients with IC had a history of delayed passage of meconium, vomiting, or abdominal distension. Conclusions: A history of delayed passage of meconium, abdominal distension, vomiting or the results of a contrast enema identified all patients with HD and excluded HD in approximately 36% of patients with idiopathic constipation. The authors have shown that key features in a patient's history, physical examination, and radiologic evaluation can differentiate between HD and IC. In a child presenting with constipation and none of the above features, it is not necessary to perform a rectal biopsy to exclude HD. J Pediatr Surg 38:412-416.     

Techniques for and outcome of liver transplantation in neonates and infants weighing up to 5 kilograms

Background: Neonates and small infants represent less than 5% of paediatric candidates for liver replacement. Most cases present under urgent conditions and receive grafts from large donors. Surgical techniques must be adapted for adequate graft preparation, vascular reconstruction, and abdominal closure. Methods: Technical aspects and outcome of 15 liver transplantations in infants weighing less than 5 kg performed at our unit were analysed retrospectively. Results: Liver transplantation was performed under urgent or highly urgent condition in 13 cases. Reduced or split liver grafts were used in all cases (median donor to recipient weight ratio, 9), including a monosegmental graft in 2 cases. In 10 cases, vascular reconstruction was done using a vascular conduit (5, 4, and 1 for artery, portal, and hepatic veins, respectively) and a delayed closure of the abdomen was necessary in 7 children. Postoperative complications were as follows: thrombosis of hepatic artery (n = 1) or portal vein (n = 1), gastrointestinal haemorrhage (n = 2), intraperitoneal bleeding (n = 1), biliary stricture (n = 2), septicaemia (n = 1). Two infants died of brain damage with a functioning graft. One child underwent retransplant for chronic rejection. Conclusions: Overall, survival rate is 60% (median follow-up, 34 months), which compares favourably with older patient groups when case mix is comparable.     

Choledochal cysts: age of presentation,symptoms, and late complications related to Todani's classification

Purpose: The aim of this study was to compare presentation, complications, diagnosis, and treatment of choledochal cysts in pediatric and adult patients. Methods: Forty-two patients were analyzed after subdivision into 3 groups: group A, less than 2 years (n = 10); group B, 2 to 16 years (n = 11); group C, greater than 16 years (n = 21). Results: The cysts were classified as extrahepatic (n = 33), intrahepatic (n = 5), and combined (n = 4). Seventy-six percent of patients presented with abdominal pain, (20 of 21 group C), and 57% with jaundice, (10 of 10 group A). Cholangiocarcinoma occurred in 6 patients, 4 of whom had previously undergone internal drainage procedures. Excision of the extrahepatic cyst was performed in 27 of 37 patients. Five patients, of whom, 4 had cholangiocarcinoma, were beyond curative treatment at the time of diagnosis. Six patients had died at the closure of this study, 5 of them had carcinoma. Conclusions: Presenting symptoms are age dependent with jaundice prevailing in children and abdominal pain in adults. In view of the high risk of cholangiocarcinoma, early resection and not internal drainage is the appropriate treatment of extrahepatic cysts. Patients who had undergone internal drainage in the past still should undergo resection of the cyst.     

The survivin:Fas ratio is predictive of recurrent disease in neuroblastoma

Background/Purpose: Several clinical and biologic features of neuroblastoma (NB) are used to predict the risk of recurrent disease. The balance between antiapoptotic and proapoptotic factors within a tumor may affect its ability to survive. Survivin is an antiapoptotic factor expressed in highly proliferative NB, whereas Fas is a proapoptotic factor that portends a favorable prognosis. The authors determined whether the ratio of survivin to Fas (S:F ratio) is predictive of recurrent disease in patients with NB. The authors previously have shown the S:F ratio is predictive of recurrent disease in pediatric renal tumors. Methods: The authors quantified the levels of 9 different apoptotic mRNA species using Rnase Protection assay (RPA, Riboquant, PharMingen, San Diego, CA). Twenty-eight primary tumor specimens were evaluated from patients with ganglioneuroma (n = 3), ganglioneuroblastoma (n = 2), and neuroblastoma (n = 23) from tumors of all clinical stages obtained at the time of diagnosis. mRNA levels were calculated as a percentage of L32 for each specimen assayed, and positive expression was assumed to be greater than 10% of L32. Results: Survivin was expressed in 90% of tumors that went on to recur and only in 27.7% of those that were cured. The S:F ratio was significantly greater in tumors that went on to recur (n = 10) compared with those from patients that were cured (n = 18) (median S:F ratio, 3.3 v 0.75; P = .0002, Wilcoxon rank-sum test). A cutoff ratio of 2.3 was highly predictive of tumor recurrence irrespective of clinical stage of disease (area under ROC curve = 0.906). Sensitivty was 80% (CI, 44.4% to 97.5%), specificty was 94.4% (CI, 72.7% to 99.9%), positive predictive value was 88.9% (CI, 51.8% to 99.7%), and negative predictive value was 89.5% (66.9% to 98.7%). Twenty-five of 28 (89.3%) tumor ratios were correct in predicting outcome. Conclusions: The survivin: Fas ratio in primary tumors may be used to predict the risk for recurrent disease in patients with NB. The S:F ratio appears to be a more sensitive predictor of recurrent disease than survivin expression alone. Determining this ratio may not only be helpful in guiding follow-up of patients with NB, but also may aid in stratifying patients for more aggressive therapeutic strategies.     

Blunt renal trauma-blessing in disguise?

Purpose: The purpose of this study was to quantify pathologic lesions of the kidney found incidentally during the workup of a blunt renal trauma. Methods: A retrospective review of the medical records of 103 patients ages 0 to 18 years with blunt renal injuries admitted to a level 1 pediatric trauma center between January 1, 1991 and December 31, 1999 was performed. All patients underwent ultrasonography and Doppler of their renal vessels. Additional investigations with computed tomography (CT) scan, cystography, or nuclear medicine functional studies were performed as indicated. Results: Coexisting urogenital lesions were identified in 13 of 103 (12.6%) patients reviewed, and 7 (54%) required surgical treatment. The majority of the patients (9 of 13, 69%) suffered minimal trauma. All patients presented with gross hematuria as their main symptom. Stenosis of the uretero-pelvic junction was the most frequent diagnosis (n = 7): 3 patients required uretero-pyeloplasty, and 3 required nephrectomy. Two heterogeneous renal masses were discovered in which the diagnosis of a malignant process could not be eliminated; elective resection and open biopsy were performed. The diagnoses of multicystic kidney and solitary cyst with complex hematoma, respectively, were confirmed on pathology. Grade III ureterovesical reflux with pyelonephritis (n = 1), polycystic kidney (n = 1), extrarenal pelvis without obstruction (n = 1), and horseshoe kidney (n = 1) were the other lesions discovered. Conclusions: Pathologic lesions of the urinary tract are uncommon; however, they may complicate an otherwise negligible renal trauma. The diagnostic and therapeutic approach to blunt renal trauma must be modified in these cases. A high index of suspicion must be maintained when a patient presents with gross hematuria with a minimal force blunt abdominal trauma.     

Epithelial ovarian tumors in children: a retrospective analysis

Background/Purpose: Epithelial tumors of the ovary account for about 15% of pediatric ovarian masses. The authors reviewed a 14-year experience with ovarian masses to understand the spectrum of pathology, presentation, and outcome of children with epithelial lesions. Methods: All ovarian masses resected or biopsied at the authors' institution from 1988 to the present were reviewed retrospectively. Patient age, presenting symptoms, operative procedures, postoperative treatment, and outcome were obtained from the medical record. Results: A total of 240 cases were identified. A total of 123 (51.2%) were nonneoplastic in nature. Of the 117 neoplastic masses, 79 (67.5%) were germ cell tumors and nineteen (16.2%) were epithelial-derived tumors. Patients with epithelial tumors had a mean age of 13.9 [plusmn] 4 years. Ten patients (52.6%) presented with abdominal pain, and 9 (47.4%) presented with an asymptomatic mass. Eleven underwent oophorectomy, 6 underwent cystectomy, and 2 had biopsy alone. Four patients (21%) had ascites, and 1 (5.3%) had a pleural effusion. The histopathologic diagnoses for the epithelial tumors included 9 serous cystadenomas (47%) and 3 mucinous cystadenomas (16%), 3 mucinous cystadenocarcinomas (16%), and 4 serous tumors of borderline malignancy (21%). Two patients (11%) had bilateral disease. Four patients (21%) underwent a subsequent laparotomy for either staging or recurrence, and 2 patients (11%) required chemotherapy. One patient (5.3%) died of ovarian adenocarcinoma. Conclusions: Epithelial tumors comprise a small but significant proportion of pediatric ovarian masses. The pediatric surgeon must understand the biologic characteristics, operative management, and follow-up treatment of these tumors, and how these differ from germ cell lesions. J Pediatr Surg 38:331-335.     

Surgical complications and outcome of pediatric liver transplantation in Hong Kong

Purpose: The aim of this study was to analyze the early and late results of pediatric liver transplantation, with particular reference to complications that required surgical or radiologic intervention. Methods: The records and code sheets of children who underwent liver transplantation in the authors' institution between September 1993 and December 2001 were reviewed. Results: Twenty-nine children (16 boys and 13 girls) underwent 31 liver transplantations (23 living donor, 8 cadaveric donor) during the study period. The ages of the children ranged from 4 months to 132 months (median, 16 months). Eighteen children had complications that required surgical or radiologic interventional procedures. Complications included, among others, hepatic vein thrombosis (n = 1, 3%), hepatic vein stenosis (n = 2, 7%), portal vein thrombosis (n = 2, 7%), biliary stricture (n = 3, 10%), bile leakage (n = 2, 7%), hepatic artery pseudoaneurysm (n = 1, 3%), jejuno-jejunostomy leakage (n = 1, 3%), graft hepatitis (n = 1, 3%), and posttransplant lymphoproliferative disorder (n = 2, 7%). In addition, 6 children (21%) suffered from intraabdominal bleeding from a variety of causes. After appropriate interventions, at a median follow-up of 38 months (range, 1 to 96 months), patient and graft survival rates were 79% and 74%, respectively. The retransplantation rate was only 7%. There was no incidence of hepatic artery thrombosis. All living donors remain alive and well. Conclusions: Complications are inevitable in pediatric liver transplantation. However, with timely recognition and active intervention, a good outcome can be achieved.     

Increased microvascular density predicts relapse in Wilms' tumor

Background/Purpose: Tumor stage and histology are the most important prognostic criteria in Wilms' tumors; however, a subset of patients remains who have favorable histology tumors and unexpectedly relapse. The authors postulated that increased microvascular density (MVD), a hallmark for angiogenesis, could identify patients at risk for relapse. Methods: A case-control study was used to compare relapse (n = 15) with nonrelapse tumors (n = 35). Tumor MVD was counted in 5 random high-powered fields (hpf) using anti-Factor VIII antibody and expressed as mean vessel count/hpf +/[minus ] SEM. MVD and clinical data were evaluated using univariate analysis and student's t test. Results: The relapse group had higher MVD than the nonrelapse group (34.9 +/[minus ] 2.9 v 22.4 +/[minus ] 2; P [lt ] .05). When evaluating the favorable histology (FH) group alone, there was higher MVD in the relapse group (32.4 +/[minus ] 2.7 v 19 +/[minus ] 1.8; P [lt ] .05). MVD was found to be the only predictor of relapse when compared with age, sex, tumor weight, and histology. Conclusions: These results suggest that increased MVD can identify Wilms' tumor patients at high risk for relapse, especially those patients with favorable histology tumors. A larger study is warranted to determine the potential utility of MVD in stratification of Wilms' tumor patients. J Pediatr Surg 38:325-330.     

Surgical management and genotype/phenotype correlations in WT1 gene[ndash ]related diseases (drash, frasier syndromes)

Background/Purpose: The WT1 gene plays a role in urogenital and gonadal development. Germline mutations of this gene have been observed in patients with Drash or Frasier syndrome (Sd). The purpose of this report is to compare phenotype and genotype of these patients. Methods: Retrospective study of 12 patients treated since 1980 for WT1 gene[ndash ]related disorders was conducted. Results: End-stage renal disease (ESRD) occurred in 9 patients, mostly because of diffuse mesangial sclerosis (DMS) or focal and segmental glomerular sclerosis (FSGS). Seven patients underwent kidney transplantation, and 2 died. Eleven tumors occurred: 8 Wilms' tumors, one soft tissue tumor, one bladder papilloma, and one gonadoblastoma. Wilms' tumors occurred at a younger age than expected. Eight patients had a 46,XY karyotype. One of these XY patients had female phenotype (Frasier syndrome); she was raised as a girl with bilateral gonadectomy. Seven XY patients had ambiguous phenotype; 4 have been raised as boys and 3 as girls. Four patients had a 46,XX karyotype; they had female genitalia and were raised as girls. WT1 gene analysis was performed in 10 patients and showed heterozygous germline mutations in exon 9 (n = 6), intron 9 (n = 1), exon 3 (n = 1), exon 4 (n = 1), or exon 7 (n = 1). Conclusions: ESRD was secondary to DMS when exon 9 was mutated, and secondary to FSGS when intron 9 was mutated. When exon 3, 4, and 7 were mutated, no nephropathy has been observed. Wilms' tumors occurred with any kind of WT1 mutation except intron 9. Abnormal sexual differentiation has been observed in all XY patients with WT1 mutation, and the most profound inversion of phenotype was observed with mutation in intron 9. Correlation between phenotype and genotype provides better understanding of the role of WT1, and can help the surgeon in the management of these patients. J Pediatr Surg 38:124-129.     

Magnetic resonance cholangiography for the diagnosis of biliary atresia

Purpose: The aim of this study was to evaluate the usefulness of magnetic resonance cholangiography (MRC) for the diagnosis of biliary atresia in infantile cholestatic jaundice. Methods: Forty-seven consecutive infants with cholestatic jaundice underwent single-shot MRC. The diagnosis of biliary atresia was made by MRC based on the nonvisualization of extrahepatic bile ducts and excluded on the basis of the complete visualization of extrahepatic bile ducts. The final diagnosis of biliary atresia (BA group, n = 23) or nonbiliary atresia (NBA group, n = 24) was established by operation or clinical follow-up until the jaundice resolved. Results: The extrahepatic bile ducts including the gallbladder, the cystic duct, the common bile duct, and the common hepatic duct were visualized in 23 of the 24 infants of the NBA group. The extrahepatic bile ducts, except the gallbladder, were not depicted in any infant of the BA group. MRC had an accuracy of 98%, sensitivity of 100% and specificity of 96%, for diagnosis of biliary atresia as the cause of infantile cholestatic jaundice. Conclusions: MRC is a very reliable noninvasive imaging modality for the diagnosis of biliary atresia. In infants with cholestatic jaundice and considered for exploratory laparotomy, MRC is recommended to avoid unnecessary surgery.     

Does transitional zone pull-through in Hirschsprung's disease imply a poor prognosis?

Purpose: Accurate frozen section interpretation of intraoperative biopsies is critical to the success of the Duhamel procedure. Errors during sampling or interpretation may result in an abnormal pull-through. The authors’ aim was to determine the incidence and outcome of transitional zone pull-through (TZPT).Methods: Ninety-six children who underwent a Duhamel procedure between January 1987 and May 2002 were followed up prospectively. The outcome of 18 children with TZPT was compared statistically with that of a cohort of 58 patients with a ganglionic pull-through.Results: The incidence of TZPT was 18.8%. Concordance rate between frozen and paraffin-section analyses was 88.5%. The incidence of enterocolitis (P = .003) and intractable constipation (P = .02) was found to be significantly higher in TZPT. There was no significant difference in continence (P = .34), rectal sensation (P = .35), and control (P = .53). Five (27.8%) TZPT patients required a revisional surgery for failure of conservative management. The incidence of stoma placement and revision was significantly less in the ganglionic group (3.4%, P = .007).Conclusions: Up to one third of patients with TZPT will require revisional surgery for intractable symptoms. Vigilance is warranted in view of the greater risk of enterocolitis. Continence is not significantly affected.     

Surgical decision making in necrotizing enterocolitis and focal intestinal perforation: predictive value of radiologic findings

Background/Purpose: Given the current controversy over the appropriate surgical management (peritoneal drainage versus exploratory laparotomy) of advanced necrotizing enterocolitis and focal intestinal perforation, the authors examined the predictive value of radiologic findings. Methods: The medical records of 80 infants undergoing exploratory laparotomy for presumed advanced necrotizing enterocolitis (NEC) or focal intestinal perforation (FIP) were reviewed. Radiologic criteria were evaluated as predictors of NEC (pneumatosis intestinalis, portal venous gas) or perforation (free air, gasless abdomen). The standard epidemiologic measures were calculated for each criterion. Results: For pneumatosis intestinalis, the sensitivity was 44% (n = 27) and specificity, 100% (n = 19). For portal venous gas, the sensitivity was 13% (n = 8) and specificity, 100% (n = 19). The sensitivity and specificity calculated for free air was 52% (n = 23) and 92% (n = 33), respectively. The sensitivity and specificity calculated for a gasless abdomen was 32% (n = 14) and 92% (n = 33), respectively. Conclusions: While demonstrating high specificity, the radiologic signs traditionally associated with NEC and FIP have unexpectedly low sensitivities. Although positive radiologic findings are of great predictive value, negative radiologic findings acquired while determining the need for and specific type of surgical intervention in suspected NEC or FIP must be interpreted with extreme caution.     

Groin exploration for nonpalpable testes: laparoscopic approach

Purpose: Diagnostic laparoscopy (DL) is the technique of choice for exploration of nonpalpable testes (NPT). Nevertheless, groin exploration is necessary to evaluate the cord and gonadal structures entering the internal ring. This retrospective analysis evaluates our hypothesis that hypoplastic cord structures entering the internal ring predicts absence of a viable testicle and a laparoscopic groin exploration in these cases can reduce the number of unnecessarily performed open groin exploration (OGE). Materials: A retrospective review was performed of 23 boys with 26 NPTs who were operated on from June 1998 to October 2000 to evaluate our protocol for NPT using DL and OGE. Results: Of 26 NPTs in 23 boys, 3 bilateral intraabdominal testis were detected (2 Fowler Stephens; 1 standard orchidopexy). Twenty cord structures entered the internal ring. Three appeared normal at DL with a viable testis followed by an orchidopexy. Seventeen were hypoplastic without patent processus. During LGE no viable testis was detected: blind-ending cords, no biopsy (n = 4); testicular regression syndromes (n = 3), early fetal regression (n = 6), no residual testicular structures (n = 4). Conclusions: This experience confirms the authors' hypothesis and criteria for LGE in all cases. The authors conclude that LGE is a helpful tool in the diagnostic workup of NPT to avoid unnecessary OGE and is a further step in the minimally invasive approach to all kinds of findings of NPT.     

Experience with thoracoscopic lobectomy in infants and children

Purpose: This study evaluates the safety and efficacy of thoracoscopic lobectomy in infants and children. Methods: From January 1995 to May 2002, 45 patients underwent video-assisted thoracoscopic lobe resection. Ages ranged from 2 days to 18 years and weights from 2.8 to 78 kg. Preoperative diagnosis included sequestration/congenital adenomatoid malformation (n = 28), severe bronchiectasis (n = 12), congenital lobar emphysema (n = 3), and malignancy (n = 2). Results: Forty-three of 45 procedures were completed thoracoscopically. Operating times ranged from 35 minutes to 210 minutes (average, 125 minutes). There were 6 upper, one middle, and 38 lower lobe resections. There was one intraoperative complication (2.4%) requiring conversion to an open thoracotomy. Chest tubes were left in 38 of 45 patients for 1 to 3 days. Hospital stay ranged from 1 to 5 days (average 2.4). Conclusions: Thoracoscopic lung resection is a safe and efficacious technique. It helps avoid the inherent morbidity of a major thoracotomy incision and is associated with the same decrease in postoperative pain, recovery, and hospital stay as seen in minimally invasive procedures. J Pediatr Surg 38:102-104.     

Long-term follow-up of patients with necrotizing enterocolitis

Background/Purpose: Despite the rise in the incidence of necrotizing enterocolitis (NEC), there is a paucity of data regarding long-term patient outcome. The authors examined functional outcome of infants with NEC (n = 103) treated at our institution between 1991 and 1995. Methods: The authors reviewed the medical records of infants who were treated both operatively and nonoperatively, n = 103. Variables examined included gestational age, birth weight, Bell stage (I through III), operations performed, and mortality rate. Telephone interviews assessed school enrollment, developmental delay, bowel function, and nutritional status. Results: Children treated operatively had a lower gestational age than those in the nonoperative group. Likewise, birth weight in the operative group was significantly lower. Sixty-three percent of patients had stage III, and the remainder had stage II disease. The telephone response rate was 61%. Mean age at follow-up was 7.5 [plusmn] 2.5 years. All children ate by mouth. Nearly all children were toilet trained. All children were less than the 50th percentile for height and weight, and the majority (83%) were enrolled in school full time. Conclusion: Infants with stage II and III NEC who are treated operatively or nonoperatively have a favorable long-term outcome. J Pediatr Surg 37:1048-1050.     

Amniotic fluid beta-endorphin: a prognostic marker for gastroschisis?

Purpose: The aim of this work was to study amniotic fluid [beta ]-endorphin as a potential predictor for postnatal morbidity in gastroschisis. Methods: Beta-endorphin was assayed in 43 amniotic fluid samples from 13 pregnant women with fetal gastroschisis undergoing diagnostic amniocentesis or therapeutic amnioinfusion and compared with 33 controls. Within the gastroschisis group, the authors investigated the relationship between postnatal morbidity and the peak value of amniotic fluid [beta ]-endorphin (AFBE). Results: Ten AFBE values in 6 cases of gastroschisis were above the upper limit of the 95% confidence interval derived from controls. Postnatal morbidity was significantly higher when peak AFBE exceeded 10 [mu ]g/L (n = 4 pregnancies) compared with below 5 [mu ]g/L (n = 9 pregnancies), as shown by mean duration of mechanical ventilation (15.2 v 3 days; P = .01), of parenteral feeding (77 v. 18.7 days; P = .04), and of hospitalization (84 v 32.2 days; P = .04). There was no statistically significant association between postnatal morbidity markers and prenatal dilation of fetal bowel. Conclusions: The most severe cases of gastroschisis are associated with high levels of AFBE. The authors speculate that this fetal hormonal response could result from stress or pain caused by prenatal bowel damage.